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THESIS HURS 1998 F17711
c.2
Ferraro, Mary Schwab.
Healthcare needs
assessment of adults
1998.
Healthcare Needs Assessment of Adults with Spina Bifida
by
Mary Schwab Ferraro
Submitted in Partial Fulfillment of the Requirements
For the Master of Science in Nursing Degree
Approved by:
Alice Conway, Ph.D., R£><
Committee Chairperson
Edinboro University of Pennsylvania
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Schilinig’I’h^CWAv/
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Edinboro l^i^ersity of Pennsylvania
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/pallet Geisel, Ph.D., RN
Committee Member
Edinboro University of Pennsylvania
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Date
Ac.
Healthcare Needs Assessment of Adults with Spina Bifida
Abstract
Literature indicates that secondary conditions are preventable in adults with spina
bifida provided they receive appropriate healthcare (Rauen & Aubert, 1992). A telephone
survey about adults with spina bifida was conducted to determine the reported frequency
with which they received primary and specialty care, the incidence of secondary
conditions and surgical interventions they had experienced since turning the age of 21,
and their level of satisfaction with their primary care provider’s ability to care for their
spina bifida. The tool utilized was a researcher-developed survey and assessed if
frequency of care impacted on the incidence of secondary conditions, surgical
interventions, and level of satisfaction with spina bifida care.
The sample consisted of 30 male and 16 female adults with spina bifida with a
mean age of 29.87. The results from this study indicated that adults with spina bifida
received more consistent and frequent care if they were seen in an adult spina bifida clinic
and that these patients appear to have a lowered incidence of decubitus ulcers, behavioral
issues, osteomyelitis, orthopaedic and urological surgical procedures, and reactive
surgical procedures. This research also found that adults who received healthcare through
a spina bifida clinic had a higher level of satisfaction with their primary care provider’s
ability to care for their spina bifida. Because of a limited sample size, not all results were
statistically significant. It is recommended that further research in this area utilize a
larger sample population and a prospective study approach in order to more accurately
determine the incidence of secondary conditions.
ii
Acknowledgments
I would like to acknowledge all those people who have helped to make this thesis
possible. I would like to thank the members of my thesis committee: Dr. Alice Conway,
Dr. Janet Geisel, and Dr. Judith Schilling for their time, energy and direction. I would
like to thank Tim Cooney, MS, for his advice on the statistical analysis of my study. I
would also like to thank the folks at Shriners Hospitals For Children, Erie: Pat Dylewski,
MSN, RN, Dr. Marguerite Jurkovic and all of my co-workers for their understanding and
patience. I would like to thank my family and Jeff, for their unending love and support.
But most of all, I would like to thank my daughter, Alaina, for without her, I never would
have made it.
iii
Table of Contents
Title
Page
Abstract
11
Acknowledgements
iii
List of Tables
vii
List of Figures
viii
Chapter I : Introduction
1
Background of the Problem
1
Theoretical Framework
3
Research Question
5
Assumptions
5
Limitations
6
Definitions of Terms
6
Summary
7
Chapter II : Review of Literature
9
Spina Bifida
9
Incidence and Etiology
9
Prolonged Survival Rates
11
Secondary Conditions
11
Factors Influencing Coordinated Care
12
Healthcare Delivery Models
12
Transitioning Services
16
iv
Funding
14
Summary
17
Chapter III : Methodology
18
Hypothesis
18
Operational Definitions
19
Research Design
20
Instrumentation
20
Pilot Study
21
Sample, Setting, and Procedure
22
Protection of Human Rights
22
Data Analysis
23
Summary
23
Chapter IV : Results
25
Sample
25
Demographics
26
Frequency of Healthcare Visits
27
Secondary Conditions
34
Surgical Interventions
Satisfaction of Spina Bifida Care
43
Future Attendance at an Adult Clinic
44
Additional Comments
44
Summary
45
v
Chapter V: Discussion
46
Summary of Research and Discussion
46
Demographics
46
Frequency of Healthcare Visits
47
Secondary Conditions
49
Surgical Interventions
51
Satisfaction of Spina Bifida Care
52
Conclusions
53
Recommendations
53
Summary
55
References
56
Appendixes
60
A. Priority Ranking
61
B. Selection of Guidelines For Spina Bifida Care Services
Throughout Life. Recommendations for the Adult Years
from the Spina Bifida Association of America
C. Introductory Script
62
63
D. Shriners Hospitals for Children, Erie Permission to
Access Previous Patient Records
65
E. Telephone Survey for the Adult with Spina Bifida
F. Survey Data
vi
List of Tables
Table
Page
1. Payment Methods
26
2. No PCP Visit Past Year
28
3. No Neuro Visit Past Year
28
4. No Ortho Visit Past Year
29
5. No Uro Visit Past Year
30
6. Surgical Classifications
41
7. Surgical Priority
42
vii
List of Figures
Figure
Page
1. Annual Primary Care Provider Visits
31
2. Annual Neurologic Visits
32
3. Annual Orthopedic Visits
33
4. Annual Urologic Visits
34
5. Behavioral Issue Incidence
35
6. Incidence of Change in Brace Needs
36
7. Incidence of Obesity
37
8. Incidence of Osteomyelitis
37
9. Incidence of Urinary Tract Infections
38
10. Incidence of Ambulatory Changes
39
11. Incidence of Decubitus Ulcers
40
12. Total Incidence of Secondary Conditions
40
13. Surgical Intervention Classification
14. Surgical Priority
viii
1
Chapter I
Introduction
This chapter provides an overview of spina bifida and the healthcare management
difficulties experienced by adults who suffer from this congenital disorder. Dorothea
Orem s (1995) self-care deficit theory of nursing is utilized as the theoretical framework
for this study and is described. Assumptions, limitations, and definitions of terms are
also provided.
Background of the Problem
Children born with chronic conditions are living longer today than in the past; it
is estimated that 85% of these children survive into adulthood (Farrow, 1992). These
figures are also true for children born with spina bifida (McLone, 1989). According to
McLone, at least 85% of children bom with myelomeningocele, a severe form of spina
bifida, can be expected to reach adulthood. The pediatric healthcare system has long
recognized that a multidisciplinary approach to the management of these children’s
special healthcare needs is most effective; unfortunately, in most instances, when these
children reach adulthood they must forfeit this model of care (Bronheim, 1989). It is at
this point that adults with spina bifida realize a higher risk for developing secondary
conditions that threaten their health, functioning and well-being (Rauen & Aubert, 1992).
Spina bifida is a conjgenital disorder that occurs within the first 4 weeks of fetal
development (Sandler, 1997). It is marked by bony anomalies of the spinal column.
When a malformation of the spinal cord and its covering (meninges) accompanies die
defect, it is called a myelomeningocele (Rauen, 1990). For the purpose of this study, the
2
term spina bifida will be used in reference to individuals that also have some degree of
myelomeningocele. Individuals with spina bifida also present with other neurological
conditions, primarily hydrocephalus, Chiari II malformation and tethered cord (Rauen,
1990). Sensory, motor, autonomic, and cognitive impairments result, the severity of
which depends on the level of defect and the extent to which the spinal cord and brain are
compromised (Sandler, 1997).
The incidence of spina bifida in the United States is approximately one out of
1,000 live births (Rauen, 1990). There are enclaves where the incidence is higher, for
example, the region extending from New England down to Georgia (Sandler, 1997). The
precise etiology of spina bifida is uncertain, however research has shown that vitamin
deficiencies, folic acid deficiencies, and environmental pollutants are likely causative
agents (Rauen & Aubert 1992).
There is an overwhelming consensus that children with spina bifida are best cared
for by a multidisciplinary healthcare team. According to Alexander and Steg (1989), the
team should consist of neurosurgery, orthopedics, urology, pediatrics and physical
medicine; nursing, physical therapy, occupational therapy, speech therapy, social work
and education should also be involved. Other key members might include a dietitian,
orthotist, psychologist/psychiatrist, endocrinologist and opthamologist (Rauen, 1990).
When the child leaves the realm of pediatric healthcare, however, this comprehensive
approach to healthcare management is generally not apparent (Bronheim, 1989).
According to Bronheim (1989),
3
While the interdisciplinary team approach to care has made an
appearance in the adult health care system (e.g. cardiac care clinics,
diabetes centers, etc.), it is not the model available in most specialty
departments. Many physicians in the adult care system are unfamiliar
with or even uncomfortable with this model of care” (p.6).
Physician attitudes and practices, ineffective healthcare delivery models,
inadequacies of insurance coverage, and lack of preparation of the adolescent and their
family for transitioning into the adult healthcare arena are a few factors that have
contributed to a lack of coordinated care (Koop, 1989). An unfortunate result of
inadequate healthcare for the adult with spina bifida is the development of secondary
conditions (morbidity) and subsequent loss of function (Rauen & Aubert, 1992). These
secondary conditions include obesity, pressure ulcers, urinary tract infections, kidney
failure, depression, chemical addiction, and loss of ambulation. Late deterioration is
common in patients with myelodysplasia; most, possibly all, of this deterioration is
preventable or correctable; it is not the natural course of the disease (McLone, 1989).
Theoretical Framework
Dorothea Orem explains her self-care deficit theory of nursing in Nursing:
Concepts of Practice (1995). She bases her theory of nursing on the belief that mature
individuals perform actions to meet the requirements for maintaining life, functioning,
development and well-being (self-care). The ability to engage in self-care is termed selfcare agency. Self-care requisite is an understanding of the actions necessary in the
regulation of human functioning and development. Therapeutic self-care demand is the
4
sum total of measures necessary to meet all of an individual’s known self-care requisites.
When therapeutic self-care demand exceeds an individual’s self-care requisites or
capabilities, self-care deficit ensues. The role of the primary care provider is to engage in
a deliberate course of action, dictated by the individual’s therapeutic self-care demands,
in order to maintain human functioning and development within the norms compatible
with life, health and well-being.
According to Orem (1995), the primary care provider’s focus must match the
healthcare requirements of the individual. Healthcare directed towards patients with
genetic and developmental defects would include:
1. Continuous healthcare to achieve the adjustments and adaptations the patient
needs for support of life processes and integrated functioning.
2. Continuous health evaluation to determine the effects of the defect on general
health, growth and development, and functioning.
3. Continuous diagnosis to determine the effects of the defect on medical
conditions.
4. Specific protection against complications or extension of present impairments
into more disabling limitations.
5. Rehabilitation of the patient as indicated.
6. Health maintenance and promotion as well as specific protection from actual or
possible effects of the defect on general health, growth and development, and
functioning.
5
Assisting the patient in assuming an appropriate role in continuing healthcare,
including self-care.
Primary care providers are obligated, according to Orem’s theory, to recognize
and chart a course of action to effectively meet the therapeutic self-care demands of
adults with spina bifida. In adults with spina bifida, this would include promoting not
only the routine health maintenance and prevention activities associated with maintaining
general health and growth and development but also those healthcare activities aimed at
preventing and treating secondary conditions associated with their spina bifida. By
assessing the healthcare services adults with spina bifida are currently receiving in the
community, nurse practitioners will be better able to identify their patients’ self-care
deficits, meet their therapeutic self-care demands and integrate routine health promotion
and education activities with disease specific prevention activities.
Research Question
Adults with spina bifida are at an increased risk for developing secondary
conditions (Patrick, Richardson, Starks & Rose, 1994). This increased risk for secondary
conditions can be attributed to a lack of coordinated healthcare (Rauen & Aubert, 1992).
Increased incidence of secondary conditions often translates into a loss of function and
independence for the adult with spina bifida (Lollar, 1994). For the purposes of this
study the following question is asked: Is there a relationship between coordinated care
and the incidence of secondary conditions?
Assumptions
The assumptions of this study were identified as follows:
6
1. Adults with spina bifida have unique healthcare needs associated with their
defect.
2. Adults with spina bifida are more apt to develop secondary conditions than
adults without disability.
3. The respondents will understand and answer the survey questions honestly.
4. Not all adults with spina bifida are able to verbally respond to questioning; if a
caretaker is needed to respond, their responses will be equivalent.
Limitations
The limitations of this study were identified as follows:
1. The sample for study was obtained from a listing of patients who had been
served at one pediatric orthopaedic facility in northwestern Pennsylvania and will effect
the generalizability of this study.
2. Different levels of morbidity may be related to the various levels of spinal
defect rather than the level of care the patient is receiving.
Definition of Terms
The terms utilized in this study were as follows:
1. Adulthood is the period of life after 21 years of age (Council on Child and
Adolescent Health, 1988).
2. Spina bifida is a condition of the spinal column where one or more dorsal or
posterior bony elements fail to fuse or unite (Rauen, 1990).
7
3. Myelomeningocele is a neural tube defect where there is a malformation of the
posterior elements of the vertebrae, protrusion of the meninges, and an external dural sac
which contains the neural elements (Alexander and Steg, 1989).
4. Secondary conditions are those further disabling conditions associated with, or
resulting from, the primary disability of spina bifida (Lollar, 1994).
5. Coordinated care is defined as attending a spina bifida clinic or annual visits
to a primary care provider, neurosurgeon/neurologist, orthopaedic surgeon,
and urologist.
Summary
Children born with spina bifida have a better chance of surviving until adulthood
than ever before (Rauen & Aubert, 1992). This is due to major strides made in managing
their unique healthcare needs over the last several decades (Bronheim, 1989). Experts
agree that a comprehensive, multidisciplinary approach to meeting their healthcare needs
has been very effective (Bronheim, 1989). Unfortunately, once children with spina bifida
leave the pediatric healthcare system and enter the adult healthcare arena, they frequently
find that this comprehensive model of managing their healthcare no longer exists; they
are left to manage their own healthcare or rely on their primary care provider to do so
(Bronheim, 1989). As a result, adults with spina bifida are more likely to develop
secondary conditions (Rauen & Aubert, 1992). These secondary conditions threaten their
ability to function independently (Lollar, 1994). If put in the context of Dorothea Orem’s
theory of self-care (1995), they are no longer able to engage in self-care agency. Orem
8
states that the primary care provider needs to adjust and adapt the health care orientation
to meet the therapeutic self-care demands of the adult with spina bifida.
The purpose of this study was to examine if the healthcare needs of adults with
spina bifida were being met and by whom. Assumptions, limitations, and terms utilized
in this study have been provided.
9
Chapter II
Review of Literature
This chapter provides a review of current literature on spina bifida and the
healthcare difficulties experienced by adults who suffer from this congenital disorder.
The purpose of this chapter is to provide the reader with a selective overview of the
population to be studied, some of their specific healthcare issues, and problems associated
with obtaining appropriate healthcare.
Spina Bifida
Spina bifida is a term that means a split or divided spine and may be an open or
closed defect (Sandler, 1997). Closed spina bifida, or spina bifida occulta, is a relatively
common condition in which there is a malformation of the bones of the spine but the
defect does not usually involve the spinal cord which is intact within the body tissues
(Sandler). It is generally thought that most people with spina bifida occulta do not suffer
from any physiological sequellae (Sandler).
Myelomeningocele, commonly referred to as spina bifida, involves not only bony
malformations of the posterior elements of the spine but also presents with a protrusion of
the meninges and an externalized dural sac which contains neural elements of the spinal
cord (Alexander & Steg, 1989). It is associated with neurological impairment at the level
of the defect and below (Rauen & Aubert, 1992).
Incidence and Etiology. The highest incidence of spina bifida worldwide can be
found in China where between 10 and 20 babies are bom with spina bifida per 1000 live
births (Sandler, 1997). The British Isles, primarily Ireland and Wales, have an incidence
10
of 3 to 6 per 1000 live births (Alexander & Steg, 1989). In the United States, the average
incidence is approximately 1 per 1000 live births with a higher incidence found along the
mountainous region extending from New England down to Georgia (Sandler, 1997).
According to Sandler (1997) “populations that move to these areas seem to have
incidence rates similar to those of more indigenous populations, whereas groups that
move to other areas have lower incidence rates” (p. 28). There is also evidence indicating
there was an epidemic outbreak of spina bifida in the United Kingdom in the 1870s and
one in the United States in the 1930s. Both of these time frames coincided with poverty
and social upheaval suggesting that both environmental factors and nutrition played a
significant role (Sandler, 1997).
Milunsky et al. (1989) reported that multivitamin /folic acid supplementation in
early pregnancy reduced the prevalence of spina bifida. They prospectively interviewed
22,776 women, who were undergoing amniocentesis, about their use of multivitamins.
Neural tube defects, of which spina bifida is a type, were three times more likely among
those women who were not taking vitamins or who took vitamins after the sixth week of
pregnancy than among those who took folic acid during the first six weeks. This was
supported by a more recent study which concluded that supplemental folic acid in the diet
of pregnant women was found to be preventative for spina bifida (Medical Research
Council Vitamin Study Research Group, 1991). In this randomized double-blind
preventative trial, 1817 women at high risk of having a pregnancy with a neural tube
defect, because of a previous affected pregnancy, were randomly assigned to either a
folic acid supplementation group, other vitamin supplementation group, folic acid and
11
other vitamin supplementation group, or a no supplementation group. Results of the
study showed a 79% protective factor for women who received folic acid
supplementation; the other vitamins showed no protective effect.
Prolonged Survival Rates. Laurence’s (1964) investigation of 407 children bom
with spina bifida between 1947 and 1956 revealed only a 29% survival rate until the age
of 12 for those newborn infants with myelomeningocele. Since that time, effective
surgical procedures have been developed to successfully close the back defect and
alleviate the often fatal complication of hydrocephalus. McLone et al. (1985) studied two
separate cohorts of 100 myelomeningocele patients. The overall mortality rate for the
initial cohort of 100 patients followed for 8 to 12 years after closure of the back was 15%.
Survival curves for the second cohort of 100 children were identical to the first. The
studies documented that 2% of the children did not survive to leave the hospital, despite
back closure and shunting; 10% died by the end of their third year; and a total of 14%
died by the end of 5 years. Death was unusual after 48 months, when the mortality
curves leveled off. Today, it is projected that at least 85% of individuals bom with spina
bifida can be expected to reach adulthood (McLone, 1989).
Secondary Conditions. Because of neurological compromise, individuals with
this disease present with musculoskeletal dysfunction, sensory deficits, bowel and
bladder impairment and some degree of sexual dysfunction; many also have learning
disabilities (Rauen & Aubert, 1992). While no hallmark study could be found in the
literature detailing all possible secondary conditions in adults with spina bifida, Farley
(1996) presented data collected by the Arkans;;as Spinal Cord Commission (ASCC) on
12
many secondary conditions. In 1993, the ASCC surveyed all known Arkansas residents
with spina bifida to determine the type and prevalence of secondary disabilities. ASCC
managers interviewed 380 individuals/families out of a possible 419 for a 91%
completion rate of known cases. The study determined that, in individuals over the age
of 20 years, 83.1% experienced pressure sores, 51.7% experienced muscle atrophy,
o9.5 /o experienced urinary tract infections, 43.5% experienced leg fractures, 12.4%
experienced cord tethering, 34.7% experienced bladder or kidney stones, and 9.8%
experienced osteoporosis. Other secondary conditions listed in the literature for which no
incidence data were found are obesity, renal insufficiency, hypertension, and depression
(Sandler, 1997). Virtually all of these conditions are preventable (Rauen, 1990; McLone,
1989; Sandler, 1997). However, it is only through close follow-up by trained healthcare
providers that these conditions can be anticipated and diagnosed early (McLone, 1989).
Without planned and coordinated care, secondary conditions ensue (Rauen and Aubert,
1992). These secondary conditions threaten the ability of the adult with spina bifida to
function independently (Lollar, 1994).
Factors Influencing Coordinated Care
There is debate in the literature concerning how to provide the best healthcare for
the ever-increasing numbers of adults with diseases of childhood onset (Rosen, 1995).
For adults with spina bifida, annual visits to a primary care provider, neurosurgeon,
orthopaedic surgeon, and urologist are among the recommendations made by the Spina
Bifida Association of America (Rauen, 1990). Hie general consensus is that adolescents,
when developmentally ready, should receive their healthcare in adult-orientcd settings
13
(Blum et al, 1993). According to Rouen (1995), “it iSMlve to Msume that providers of
adult health care are adept at delivering ongoing care of young adults with chronic
conditions and there is much to be learned from the pediatric experience” (p. 14).
Healthcare DelivgryModels, The Health Resources and Services Administration
has proposed a few models of care that provide transition from the realm of pediatric
care to the adult healthcare system. The Interdisciplinary/Multidisciplinary Team Model
features a care coordinator who provides the patient and family with comprehensive and
coordinated care by organizing the interdisciplinary/multidisciplinary team; this team
consists of, but is not limited to primary care providers, specialist physicians, nurses,
therapists, psychologists and psychiatrists, social workers, nutritionists, educators,
vocational rehabilitation and prevocational counselors, genetic counselors and
interpreters (Health Resources and Services Administration [HRSA], 1992). These teams
strengthen communication among providers and help to assure access to healthcare
services (HRSA, 1992). Unfortunately, while theoretically plausible, this model has very
little practical application at the present time for adults with spina bifida due to the very
low number of adult specialty clinics throughout the United States (Dorval, 1994).
The Generic Services Model is one in which a primary care provider provides the
care coordination with specialty medical care provided by specialists (HRS A, 1992). The
Family Practice Model is similar to the Generic Services model but treats the individual
in the context of the family allowing for continuity of care from childhood through
adulthood. In this model, the family practitioner is a generalist but, through wide
experience, referral to specialists is kept to a minimum; developmental issues are
14
considered the essential framework for medical care and “adult problems” such as weight
control, STDs and AIDS, substance abuse, contraception and prenatal care are also
considered within this context (HRS A, 1992). These two models can successfully meet
individuals healthcare needs to the extent that the primary care provider is well versed in
the problems associated with spina bifida. Lack of training is prevalent in the adult
healthcare sector where physicians are unfamiliar with the chronic conditions that affect
their disabled patients (HRS A, 1992). Few communities have physicians interested in
following adults with developmental or physical disabilities (Hallum, 1995). Kaufman et
al. (1994) conducted a retrospective telephone survey of 87 pediatric myelomeningocele
patients who had been treated at a multidisciplinary central nervous system malfunction
clinic. The clinic had disbanded 3 years prior to the survey and the researchers wanted to
determine the effects the clinic closure had on the healthcare of its former patients. They
determined that a significant problem with coordinated follow-up care existed: 66% of
the patients reported no pediatric follow-up, 66% reported no neurological follow-up,
50% reported no orthopaedic follow-up, and 66% reported no urological follow-up. They
found that the age of the patient had a highly significant correlation with the absence of
follow-up: 92% of subjects 16 years or older reported no neurological follow-up, 78%
reported no orthopaedic follow-up, and 60% reported no urological follow-up.
The Disease Specific Model has pediatric specialists continuing to provide care in
collaboration with their adult specialist counterparts. This model often lacks
comprehensive primary and preventative care (HRS A, 1992).
15
Transitioning Services. Failure to provide anticipatory guidance, or transitioning,
for the adolescent with spina bifida as they approach adulthood is also a concern (Blum et
al., 1993). There is no arbitrary age or developmental milestone at which transitioning
efforts should begin (Rosen, 1995). According to Rosen (1995),
Young people will be best prepared for the adult health care environment
if they are able to communicate independently with the health care
providers and can advocate forcefully on their own behalf. They should
have already assumed substantial responsibility for their own management and
have demonstrated themselves effective in their own care (p. 14-15).
The majority of health professionals, however, are either unaware or unsure of the issues;
this systemic inertia is fueled by insufficient information about transition options and
outcomes (Blum et al., 1993).
In 1991 the Center for Youth with Disabilities completed a national survey of
state agencies that impact on youth with disabilities and their families and found that
/o
of states reported that they provided some individual transition planning (Okinow, 1992).
In their summary of conference recommendations from Moving On. Transition From
Pediatric to Adult Health Care” held September 9-11, 1994, the Society For Adolescent
Medicine (1995) recommended:
1. Structure service delivery models to be responsive to the needs and
experiences of young people with disabilities and their families rather than to
accommodate health care professionals.
16
2. Design transition services to be developmentally appropriate addressing not
only the unique medical concerns but also the social, emotional, vocational, sexual, and
general health concerns of young people as well. Additionally, such services must
address lifestyle and risk behaviors.
3. Empower young people to take responsibility for their own health care by
providing them with the skills and knowledge needed for self-advocacy.
4. Identify, develop, and test models of transition services that define “best
practices” and evaluate the process, service mix, role of various providers, as well as the
costs and outcomes.
5. Expand research that evaluates the quality of care for young people with
chronic conditions documenting who needs and receives services, and the impact of
transition services on recipients and their families.
Funding. In addition to chaotic delivery of services, the adult with spina bifida
faces the challenge of funding the healthcare they require. At the age of 21, all 50 states
cease to offer care previously covered under Title V funding (Reiss & Dearholt, 1997).
In the late 1980s, 20% of disabled young adults, ages 19-24, were without any form of
health insurance protection at all; thousands more were insured but without adequate
coverage for the services they required (McManus, 1989). Kaufman et al. (1994) stated
that local government programs reimbursed at a lower rate for the same diagnosis in
adults than in children. Hayward et al. (1991) demonstrated that the uninsured were more
likely than the insured to lack a regular source of ambulatory care, 31.2% vs. 14.8%
respectively. They also reported that lacking a regular source of ambulatory care was a
17
risk factor for not receiving recommended medical care: 56% of respondents who had a
tegular source of ambulatory care reported having a PAP smear within the past year as
compared to 46% of respondents who lacked a regular source of ambulatory care, and
59% of respondents who had a regular source of ambulatory care reported having a breast
examination by a physician within the past year as compared to 31% of respondents who
lacked a regular source of ambulatory care.
Summary
This chapter has provided a review of the literature concerning spina bifida and
the difficulties experienced by adults who suffer from this congenital disorder. Presented
were the secondary conditions that can arise if comprehensive, coordinated healthcare is
lacking for these individuals. This review of literature noted that a lack of coordinated
care has resulted from a variety of factors.
18
Chapter III
Methodology
This chapter describes the methodology utilized to determine the frequency of
cooidinated care visits made by an adult with spina bifida to a primary care provider,
neurosurgeon/neurologist, orthopedic surgeon, and urologist, the incidence of secondary
conditions and surgical interventions in the adult with spina bifida, and the level of
satisfaction that exists with the primary care provider’s ability to care for the adult with
spina bifida. It also describes the methodology utilized determine if coordinated care is
related to the incidence of secondary conditions. Included in this chapter are the
hypothesis, operational definitions, research design, instrumentation, sample, setting, and
procedure, protection of human rights, and data analysis to be utilized for this study.
Additional information was collected that looked at the frequency of routine visits to a
primary care provider, neurosurgeon/neurologist, orthopaedic surgeon, and urologist, the
incidence of surgical interventions, and satisfaction with the primary care provider s
ability to care for the adult with spina bifida.
Hypothesis
There is an inverse relationship between the frequency of coordinated care and the
incidence of secondary conditions in the adult with spina bifida.
Operational Definitions
The operational definitions used in this study are identified as follows:
19
1. Primary care provider is either a medical doctor, osteopathic physician, or
nurse practitioner who provides routine health screenings and assessments, diagnostic
evaluations, treatment of treatable conditions within the scope of their practice and
monitoring of ongoing health conditions with appropriate referrals to specialists.
2. Secondary conditions are conditions that are causally related to the disabling
condition and that can be either a pathology, an impairment, a functional limitation, or an
additional disability. These disabilities include pressure ulcers, urinary tract infections,
kidney failure, loss of a kidney, high blood pressure, obesity, fractures, changes in brace
needs, behavior issues, changes in ability to walk, and changes in bowel funciton.
3. Surgical procedures were procedurally classified as either orthopaedic,
urological, neurological, or other.
4. Surgical procedures were also independently prioritized as either reactive
interventions, proactive interventions, or unrelated interventions. A board certified
orthopaedic surgeon, working in a pediatric orthopaedic facility, determined the priority
ranking of each surgical intervention based on the information available through the
survey (Appendix A). Reactive surgical interventions were defined as those
interventions which were performed as a result of a secondary condition and assumed less
frequent routine care. Proactive surgical interventions were defined as those
interventions which were performed in order to prevent a secondary condition and
assumed more frequent routine care.
Unrelated interventions were defined as those
interventions which would have taken place regardless of second^ conditions or
frequency of routine care.
20
5. Coordinated care is care that is received through a spina bifida clinic or annual
visits to a primary care provider, neurosurgeon/neurologist, orthopaedic surgeon, and
urologist.
Research Design
This study utilized a non experimental survey research design. It was a
descriptive correlational study comparing routine visits by adults with spina bifida to a
primary care provider, neurosurgeon, orthopedic surgeon, and urologist with the
incidence of secondary conditions, surgical interventions, and satisfaction with the
primary care provider’s ability to care for the adult with spina bifida.
Instrumentation
A telephone survey was utilized as the research tool (Appendix B) and was based
on the SBAA Guidelines for Spina Bifida Health Care Services Throughout Life (Rauen,
1990) (Appendix B). Due to lack of previous research in this area, the tool was
researcher-designed. The survey consisted of three sections. The first section elicited
demographic information concerning the respondent and their relationship to the adult
with spina bifida, either self or caretaker, the adult’s sex and age, current payment method
for healthcare services, approximate distance from Erie, Pennsylvania, and history of
hydocephalus.
The second section contained open-ended questions concerning the adult’s
frequency of routine healthcare visits with a primary care provider,
neuro surgeon/neurologist, orthopaedic surgeon, and urologist,:. If the respondent denied a
routine visit by the adult in the past year to either a primary earn provider, neurosurgeon,
21
orthopaedic surgeon, or urologist, their reason(s) were elicited. This section ended with
an open-ended question and determined if the adult received healthcare from any other
provider(s).
The third section contained specific and open-ended questions regarding the
adult s incidence of secondary conditions and surgical procedures. Question 19
determined the level of satisfaction with the primary care provider’s ability to take care of
the adult’s spina bifida. There were five possible responses from Excellent to Not at All.
Question 20 determined interest in attending an adult spina bifida specialty clinic in Erie,
Pennsylvania; possible responses were Yes, No, Unsure. Question 21 was open-ended
and intended to elicit any additional questions or concerns the respondent wished to add.
The survey was reviewed by the Research Committee at Shriners Hospitals For
Children, Erie for content validity. It was also reviewed by the Director of Research at
Hamot Medical Center in Erie, Pennsylvania and a representative of the Educational
Services Department at Edinboro University of Pennsylvania for content validity and
readability.
Pilot Study
The survey was piloted with a 24 year old female adult with spina bifida. The
decision was made not to employ Likert-type
scales, but rather, to allow for open-ended
responses to many of the survey questions. The participant completed the survey in less
than 10 minutes.
22
Sample, Setting, and Procedure
The convenience sample was adults with spina bifida over the age of 21. The
adult with spina bifida, or their caretaker in the event the adult was unable to respond,
served as the respondent to the survey; all understood and spoke English. The sample
was obtained from a listing of patients who had been served at one pediatric orthopaedic
facility in northwestern Pennsylvania and were 21 years of age or older. A telephone
survey was conducted in the first quarter of 1998. Telephone appointments for interviews
were made with the respondents at their convenience. The setting for this study was
whatever location they chose to respond to the survey. The procedure for this study was
for the researcher to read the introductory script to the respondent (Appendix C). If they
orally agreed to participate, the researcher proceeded with the survey.
Protection of Human Rights
A verbal introduction to the telephone survey was made to allow the respondents
to know the purpose of the survey (Appendix C). Agreement to participate in the survey
was deemed as informed consent. All data remained confidential, numbers were assigned
to the surveys and no names appeared. The researcher kept the list of identifying
information in a locked file cabinet and the data was reported as aggregate data. The
study was deemed exempt from a review by the Stainers Hospitals For Children Internal
Review Board (1RB) by representatives of the Research Committee at Stainers Hospitals
For Children, Erie (Appendix D).
23
Data Analysis
The first section was descriptively analyzed and trends were identified. The
second section looked at frequency of annual visits with a primary care provider,
neurosurgeon/neurologisl, orthopaedic surgeon, and urologist. These data were content
analyzed to determine if they met the criteria for coordinated care. Those subjects who
met the criteria for coordinated care were compared to those who did not. Subjects who
met the criteria for coordinated care reported receiving healthcare through a spina bifida
clinic. A Chi-square analysis, utilizing a Fischer’s Exact Test, was performed. This
analysis compared the annual visits between the identified spina bifida clinic group and
the non-clinic group.
The final section was content analyzed looking for themes. A Chi-square analysis
was attempted comparing the incidence of secondary conditions and surgical
interventions, and the satisfaction rating of the primary care provider’s ability to care for
the adult with spina bifida.
Summary
This chapter has identified the hypothesis, operational definitions, research
design, instrumentation, pilot study, sarniple, setting, and procedure, and protection of
human rights that were utilized for this study. It has identified that a descriptive
correlational study was utilized comparing routine visits by adults with spina bifida to a
primary care provider, neurosurgeon/neurologist, orthopaedic surgeon, turd urologist with
the incidence of secondary conditions, surgical interventions, and satisfaction with the
primary care provider's ability to care for the adult with spin, bifida. The hypothesis of
24
this study was that there is an inverse relationship between the frequency of routine health
care and the incidence of secondary conditions in the adult with spina bifida.
25
Chapter IV
Results
This Chapter presents the results obtained from a telephone survey about adults
with spina bifida (Appendix E). The results were descriptively analyzed and a Chi-square
analysis was performed on specific questions to determine if there was a statistically
significant difference between two identified subgroups in the sample population.
Sample
This study utilized patients that had once been seen and/or treated for spina bifida
at a small pediatric, orthopaedic hospital in northwestern Pennsylvania and over the age
of 21. Names and telephone numbers were obtained from the medical records of these
former patients.
The sample population numbered 96 and the investigator was able to contact 46
subjects (47.92%). All of those contacted were willing to participate in the survey and
were included in the research results.
The investigator initiated telephone contacts February 1, 1998 and completed
telephone contacts March 27, 1998. If the adult with spina bifida had relocated, either
their new telephone number was obtained or a family member was provided with a toll-
free number that the adult with spina bifida was instructed to use Monday-Friday, Sam4pm EST. In instances when a telephone number was incorrect or no longer in service,
the investigator utilized various person-locator sites on the internet in an effort to locate
26
the individual. These included Four-11 (www.fourli.oom) and Bigfoot
(www.bigfoot.com).
Demographics
Of the 46 completed telephone surveys, 29 (63%) were completed by the adult
with spina bifida and 17 (37%) were completed by the caretaker of the adult with spina
bifida. There were 30 (65%) male adults with spina bifida and 16 (35%) female adults
with spina bifida. The subjects ranged in age from 22 to 44 with a mean age of 29.87
(s.d.= 5.99).
Table 1 identifies the reported payment method for healthcare services. Private
insurance was defined as insurance provided by either an employer or paid for out of
pocket and public insurance was defined as insurance that was provided by either state or
federal programs.
Table 1
Payment Methods
n
(%)
private insurance
17
(37%)
public insurance
21
(45.7%)
2
( 4.3%)
6
(13%)
Type
no insurance
combination
n = 46 surveys were completed.
27
Of the sample population, 11 (23.9%) of ,he adults with spin, biMa lived wi,hi„
30 miles of Erie, Pennsylvania, 1 (2.2%) hved between 31 and 50 miles from Erie,
Pennsylvania, 9 (19.6%) lived between 51 and loo miles from Erie, Pennsylvania and 25
(54.3%) lived over 100 miles from Erie, Pennsylvania.
Because a large number of adults with spina bifida have a history of
hydrocephalus, respondents were asked if the subject had a history of hydrocephalus.
Twenty seven (58.7%) did have a history of hydrocephalus and 19 (41.3%) did not.
Frequency of Healthcare Visits
Fourteen (30.4%) of the adults with spina bifida reported, on an average, not
making a routine visit to a primary care provider annually; 32 (69.6%) of the adults with
spina bifida reported, on an average, making one or more routine visits annually. When
asked about the number of routine visits made to a primary care provider in the last year,
14 (30.4%) of the adults with spina bifida reportedly made no routine visit and 32
(69.6%) made one or more routine visits. Of the 14 who had not made a routine visit to a
primary care provider within the past year, most said they believed they didn t need to see
one (Table 2).
Twenty-eight (60.9%) of the adults with spina bifida reported not making a
routine visit to a neurosurgeon/neurologist annually; 18 (39.1%) reported making, on an
average, one or more routine visits mutually. When queried about the number of routine
visits made to a neurosurgeon/neurologist in the past year, 33 (7!.7%) of the adults with
spina bifida made no visits and 13 (28.3%) made one or more visits. Of the 33 who did
28
not make a routine visit to a neurosurgeon/neurologist within the past year, the majority
stated they didn’t feel it was necessary (Table 3).
Table 2
No Primary Care Provider Visit Past Year
Reason
no insurance
1
no provider
2
not necessary
n
n
11
14 subjects who did not see a primary care provider in last year.
Table 3
No Neurologic Visit Past Year
n
Reason
no insurance
not necessary
n
30
33 subjects who did not see a neurosurgeon/neurologist m past year.
Thirty-two (69.6%) of the adults with spina bifida reported not making a routine
visit to an orthopaedic surgeon annually; 14 (30.4%) reported making, on an average, one
or more routine visits annually. Thirty-six (78.3%) of the adults with spin, bifida
29
reported not making a routine visit to
an orthopaedic surgeon in the past year and 10
(21.7%) made one or more routine visits. Of the 36 who did not make a routine visit to
an orthopaedic surgeon within the past year, most felt it was unnecessary (Table 4).
Table 4
No Orthopaedic Visit Past Year
Reason
n
no referral
5
no provider
1
not necessary
30
n = 36 subjects who did not see an orthopaedic surgeon in last year.
Twenty (43.5%) of the adults with spina bifida were reported not making a routine
visit to a urologist annually; 26 (56.5%) reported making one or more routine visits
annually. Twenty-five (54.3%) of the adults with spina bifida reported not making a
routine visit to a urologist in the past year and 21 (45.7%) made one or more routine
visits. Of the 25 who did not make a routine visit to a urologist within the past year, 22
(88%) felt it wasn’t necessary (Table 5).
The respondents were asked which other providers routinely provided care to the
adult with spina bifida. Nine (19.6%) of the adults with spina bifida reported receiving
routine care through a spina bifida clinic.
A Chi-square analysis, utilizing a Fisher’s Exact Test, was performed. This
analysis compared the frequency of visits to a primary care provrder,
30
Table 5
No Urologic Visit Past Year
Reason
no referral
not necessary
n
3
22
n = 25 subjects who did not see a urologist in last year.
neurosurgeon/neurologist, orthopaedic surgeon, and urologist between the subgroup of
adults with spina bifida who received routine health care through a spina bifida clinic and
the subgroup of adults with spina bifida who did not receive routine health care through a
spina bifida clinic.
When comparing the frequency of the average number of annual visits made to a
primary care provider, there was a statistically significant (p = <0.05) difference between
spina bifida clinic patients and non clinic patients (Figure 1). All of the spina bifida clinic
patients saw a primary care provider an average of one or more times a year as compared
to 23 (62.2%) of the non clinic patients (Figure 1).
The difference in the frequency of routine visits within the past year between the two
subgroups was also statistically significant (p.-< 0.05). AU of the spina bifida clinic
patients saw a primary care provider within the past year while 23 (62.2%) of the non
clinic patients did.
31
.X?
100Y< 0Non clinic Patients
90-
J Spina Bifida Clinic Patients
807060OT
C
0
ra
CL
504030 -■
2010-
o+
___ A
None
One or more
Annual Neurologic Visits
p = < 0.05 significance compared spina bifida clinic patients with non clinic patients
Figure 2. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and annual routine neurologic care utilization.
There was a statistically significant difference (p.=< 0.05) between the two
subgroups in the frequency of reported average number of annual visits to an orthopaedic
surgeon. Seven (77.8%) of the spina bifida clinic patients saw an orthopaedic surgeon an
average of one or more times a year as compared to 7 (18.9%) of the non clinic patients
(Figure 3).
Although the frequency rates dropped in both subgroups, there was a statistically
significant difference (p.=< 0.05) in the frequency of visits to an orthopaedic surgeon
32
90T
0Non clinic Patients
■^Ping.^da Clinic Patients
80
70-
g 60<2
50' :
.2
40 <
a-
30-i
20-i
10-
o-r
None
One
or
more
Annual Orthopaedic Visits
p = < 0.05 significance compared spina bifida patients with non clinic patients
Figure 3. Comparative data between percentage of spinal bifida clinic patients (n = 9)
and non clinic patients (n = 37) and annual routine orthopaedic care utilization.
within the past year between the two subgroups. Five (55.6%) of the spina bifida clinic
patients were reported as visiting an orthopaedic surgeon in the past year as compared to
5 (13.5%) of the non clinic patients.
Finally, comparison frequency data of the reported average number of annual
visits made to a urologist was
also statistically significant (p.=< 0.05) between the two
subgroups. All (100%) of the spina bifida clinic patients saw a urologist an average o
one or more times a year as compared to 17 (45.9%) of the non clinic patients (Figure 4).
However, there was no statistically significant difference (p.=.O59) between the
two subgroups when comparing the frequency of repotted routine visits to a urologist
33
g
w
c
CL
100f
90- ® Non clinic Patients
* Spina Bifida Clinic Patients
80- >:•:
:;4: x:x
x ;:x;y
>:•;« x-x-x^x—v.-.- 70605040302010-
oNone
One
or
more
Annual Urologic Visits
p = < 0.05 significance compared spina bifida clinic patients with non clinic patients
Figure 4. Comparative data between percentage of spinal bifida clinic patients (n = 9)
and non clinic patients (n = 37) and annual routine urologic care utilization.
within the past year. Seven (77.8%) of the spina bifida clinic patients saw a urologist
within the past year as compared to 14 (37.8%) of the non clinic patients.
Secondary Conditions
The survey addressed incidence of secondary conditions in the adult with spina
bifida. These results determined that 36 (78.3 %) had a history of urinary tract
>f decubitus ulcers, 15 (32.6%) were obese, 15 (32.6
infections, 29 (63%) had a history oi
12 (26.1%) had changes in their brace needs,
%) had a change in their ambulatory status,
id 10 (21.7%) had a history of
12 (26.1%) reported having behavioral issues, am
osteomyelitis.
A Chi-square analysis, utilizing a Fisher’s Exact Test, was performed comparing
lhe incidence rates of the above secondary conditions between the subgroup of spina
34
bifida clinic patients and non chnie patients. Wre was no
difference (^.409) between the two subgroups and theiueidence of behaviors! issues
One (11.1%) of the spina bifida clinic patients reported having suffered with behavioral
issues as compared to 11 (29.7%) of the non clinic patients (Figure 5).
90-'
® Non clinic Patients
80-
■Spina Bifida Clinic Patients
70 -
g
w
c
60-
50 J
0)
40- i
CL
30- i
.■
20- i
10-
o-f4
No
Yes
Behavioral Issue Incidence
P = 0.409 significance compared spina bifida clinic patients with non clinic patients
Figure 5. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of behavioral issues.
There was no statistically significant difference (p.=-211) between the two
subgroups and the incidence of changes in brace needs. Four (44.4%) of the spina bifida
clinic patients had a reported change brace needs as compared to 8 (21.6%) of the
clinic patients (Figure 6).
There was no statistically significant difference (i>.=.«5) between the two
subgroups and the incidence of obesity.
Four (44.4%) of the spina bifida clinic patients
35
were reported to be obese as compared to 11 (29.7%) of the non
clinic patients (Figure
7).
® Non clinic Patients
80 Y
Spina Bifida Clinic Patients
70 J
60-
g
50-
w
c
40-
ro
30-
0)
£L
20-
I
10-
oNo
Yes
Incidence of Change in Brace Needs
P = .211 significance compared spina bifida clinic patients with non clinic patients
Figure 6. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of change in brace needs.
There was no statistically significant difference (p.=.659) between the two
subgroups and the incidence of osteomyelitis. One (11.1%) of the spina bifida clinic
patients were reported to have a history of osteomyelitis as compared to 9 (24.3%) of the
non clinic patients (Figure 8).
There was no statistically significant difference (p.=1.0) between th. two
subgroups and the incidence of urinary tract infections. Seven (77.S%) of th p
bifida clinic patients reportedly had a hlstory of urinary tract infections as compared to
29 (78.4%) of the non clinic patients (Figure 9).
36
80-f
0Non clinic Patients
70 -;
E§pina Bifida Clinic Patients
60-
? 50tn
c
o
co
q.
4030 <.•
20
‘
:
10-
o£
Incidence of Obesity
2
.445 significance compared spina bifida clinic patients with non clinic patients
Figure?. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of obesity.
□ Non clinic Patients
■ Spina Bifida Clinic Patients
908070-
■
■II
60OT
C
o
Q.
5040-
30 d
I
20- i
10- :
0-r
I
8
No
Yes
Incidence of Osteomyelitis
2 = .659 significance compared spina bifida clinic patients with non clinic patients
Eigure_8. Comparative data between percentage of spina bifida clinic patients (n 9) and
non clinic patients (n = 37) and incidence of osteomyelitis.
37
80
70-j
® Non clinic Patients
■ Spina Bifida Clinic Patients
60-i
g 50w
1 40’
£
302010<
I
o4
No
Yes
Incidence of Urinary Tract Infections
E = 1.00 significance spina bifida clinic patients with non clinic patients
Figure 9. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of urinary tract infections.
There was no statistically significant difference (p =.455) between the two
subgroups and the incidence of ambulatory changes. Four (44.4%) of the spina bifida
clinic patients had reported ambulatory changes as compared to 11 (29.7%) of the non
clinic patients (Figure 10).
There was no statistically significant difference (p.=.7O7) between the two
subgroups and the incidence of decubitus ulcers. Five (55.6%) of the spina bifida clinic
patients were reported to have a history of decubitus ulcers as compared to 24 (64.9%) of
non clinic patients (Figure 11).
There was no statistically difference (p= 1.0) between the two subgroups and the
total reported incidence of secondary conditions (Figure 12). Due to concerns regarding
38
th. content validity of reported brace changes, this category was exduded. Tout
incidence of secondary conditions was reported as a sum total of either a positive or
negative history for specific secondary conditions listed and reported and no, nuandfied
per number of occurrences. This refuted this study’s hypothesis.
80-
0 Non clinic Patients
70-
* Spina Bifida Clinic Patients
60-
? 50w
I 40‘
Q.
30 :
20*:
1004
No
Yes
Incidence of Ambulatory Changes
2 = .445 significance compared spina bifida clinic patients with non clinic patients
Figure 10. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of ambulatory changes.
Surgical Interventions
There were 51 reported surgical interventions by rhe sample population. For
descriptive purposes, they were procedurally classified as either orthopaedic, urological,
neurological, or other. There was a total of 28 (54.9%) orthopaedic procedures, 12
(23.5%) urological procedures, 3 (5.9%) neurological procedures, and 8 (15.7%) other
39
nNon clinic Patients
70
■Spina Bifida Clinic Patients
6050-
F
£
Jc 40^
.2
CD
30-
CL
20-
10-
■II
0
No
- J
Yes
Incidence of Decubitus Ulcers
p = .707 significance compared spina bifida clinic patients with non clinic patients
Figure 11. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of decubitus ulcers.
100-
999897-i
w
c
.2
co
Q.
0Non clinic
Patients
I
|
■Spina
Bifida
Clinic
96-:
959493-
Mid
■
92- i
91 +
Total Incidence of Secondary Conditions
£=1.0 significance compared spina bifida clinic patients with non clinic patie ts
Eigure_12. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and total incidence
of secondary conditions.
40
procedures (Table 6). Ota procedures Included things !Kh as
fatty abdominal tumor, and meniscus repair following a sledding injury.
Table 6
Surgical Classifications.
Reason
n
Orthopaedic
28
Urological
12
Neurological
3
Other
8
n = 51 surgical interventions.
The reported surgical interventions were also, but separately, prioritized as either
reactive interventions, proactive interventions, or unrelated interventions. There was a
total of 37 (72.5%) reactive interventions, 6 (11.8%) proactive interventions, and 8
(15.7%) unrelated interventions (Table 7).
Due to a limited sample size, a Chi-square test was not able to be performed
comparing the incidence of either the different surgical classifications or the different
prioritized surgical interventions between the subgroups of spina bifida clinic patie
and non-clinic patients. Although not statistically significant, when comparine the
subgroups, the spina bifida clinic group demonstrated a 44.4% rate of orthopaedic
procedures as compared to a 57.1 % rate in the non clinic group. Likewise, the spina
41
Table 7
Surgical Priority.
Reason
n
reactive
37
proactive
6
unrelated
8
n = 51 surgical interventions.
bifida clinic group showed a 11.1% rate of urological procedures as compared to a 26.2%
rate in the non clinic group (Figure 13).
60
0 Non clinic Patients
■Spina Bifida Clinic Patients
50-
40-
£
o>
co
o.
z
3020-
10-
[ •
0
Ortho
Neuro
Other
Surgical Intervention, Classification
g = .128 significance compared spina bifida clinic patients with non clinic patients
Figure 13. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and types of surgical interventions.
42
Again, despite its statistical insignificance, the comparison between the two
subgroups demonstrated a 55.6% rate in reactive interventions in the spina bifida clinic
group and a 76.2% rate in the non clinic group (Figure 14).
8070-
I
60-
0 Non clinic Patients
I
■
■Spina Bifida Clinic Patients
500
40-
CQ
Q.
3020-
10-
! s', ;
I I I
-
oReactive
Proactive
Unrelated
Surgical Priority
p = .129 significance compared spina bifida clinic patients with non clinic patients
Figure 14. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and prioritized surgical interventions.
Satisfaction of Spina Bifida Care
Respondents were asked to rate how well the primary care provider takes care of
the adult with spina bifida. Fifteen (32.6%) of the respondents stated their primary care
provider was excellent, 5 (10.9%) stated they were good, 12 (26.1%) said they were
adequate, 2 (4.3%) said they were poor, and 12 (26.1%) said they did not take care of
their spina bifida at all.
A Chi-square analysis was performed comparing the satisfaction ratings of the
primary care provider’s ability to take care of the adult with spina bifida and the two
43
subgroups. Again, because of a limited sample size, the analysis violated assumptions in
the Chi-square test. Of note, however, 7 (77.8%) of the spina bifida clinic group rated
their primary care provider as excellent in taking care of their spina bifida as compared
to 8 (21.6%) of the non clinic group. None of the spina bifida clinic patients rated their
primary care provider less than adequate yet 14 of 37 (37.8%) of the non clinic group
patients rated their primary care provider as either poor or offering no care at all.
Future Attendance at an Adult Clinic
The respondents were asked if the adult with spina bifida would attend, if
available, an Erie-based adult multidisciplinary spina bifida clinic for routine health care.
Seventeen (37%) said they would attend, 19 (41.3%) stated they would not attend, and
10 (21.7%) stated they were unsure if they would attend.
Additional Comments
The respondents were asked if they had any other questions or comments. Five
(10.9%) of the respondents had questions regarding the current symptoms the adult with
spina bifida was experiencing, 2 (4.3%) asked if spina bifida was indeed an accurate
diagnosis of the adult with spina bifida, and 2 (4.3%) had general information questions.
Summary
This chapter presented the results from a telephone survey conducted with adults
with spina bifida or their caretaker. These results were interpreted through descriptive
analysis with the percentage of responses provided. Specific questions were also
subjected to Chi-square analysis and comparative data was provided for two identified
subgroups of the sample population. Due to a limited sample size, this study did not
44
support the hypothesis that there is an inverse relationship between the frequency of
health care and the incidence of secondary conditions in the adult with spina bifida.
45
Chapter V
Discussion
This chapter provides a summary of results which were obtained from a
telephone survey about adults with spina bifida (Appendix F). These results were
utilized to determine the frequency of routine primary and specialty care the adult with
spina bifida received and whether or not it was related to the incidence of secondary
conditions, surgical interventions, and satisfaction with the primary care provider’s
ability to care for the adult with spina bifida. Conclusions and recommendations based
on these results are also provided.
Summary of Research and Discussion
This section provides a summary of findings from this study. These findings
were compared to the recommended guidelines for spina bifida health care services for
the adult years.
Demographics. Information was provided by the adult with spina bifida when
possible but in the event they were not able to respond, the caretaker was surveyed. The
sample population represented a slightly higher percentage of males than females,
perhaps due to the difficulty of tracking individuals longitudinally by name and
telephone number alone. Women generally change their name when they marry and if
they relocate, this makes follow-up even more difficult.
The subjects ranged in age from 22 to 44 years with a mean age of 29.87. The
majority of the population had some form of insurance.
Twenty one (45.7%) of the subjects lived within a 100 mile radius of Erie,
Pennsylvania. This was perhaps an influencing factor for the 37% of respondents who
46
indicated an interest in attending an adult spina bifida specialty clinic in Erie,
Pennsylvania.
Approximately half of the population had a history of hydrocephalus. When
comparing the number of patients who received routine care through a spina bifida clinic
with non clinic patients, it was statistically significant that all of the spina bifida clinic
patients had a history of hydrocephalus as compared to the 48.6% of the non clinic
patients. It can be inferred that having a history of hydrocephalus was an influencing
factor in seeking routine primary and specialty care.
Frequency of Healthcare Visits. The majority of adults with spina bifida in this
study received routine health care by a primary care provider most often; this is not
surprising in light of managed care. They tended to see a urologist more often than a
neurosurgeon/neurologist and they saw an orthopaedic surgeon least often. For those
who had not seen a primary care provider, neurosurgeon/neurologist, orthopaedic
surgeon, or urologist within the past year, the overwhelming reason was that they did not
feel it was necessary.
The data indicated that the adult with spina bifida is falling far short of the
recommendations for adults in the Guidelines For Spina Bifida Healthcare Services
Throughout Life. Recommendations for frequency of visits to a primary care provider,
neurosurgeon, orthopaedic surgeon, and urologist are for once a year (Rauen, 1990).
When asked about other providers who routinely cared for the health care needs
of the adult with spina bifida, a trend emerged. Nine (19.6%) of the population reported
receiving care through a spina bifida clinic. For the purposes of comparative analysis,
47
these subjects were identified and grouped as spina bifida clinic patients and the
remainder of the sample population was identified and grouped as non clinic patients.
When comparing the frequency of health care visits between the two groups,
there were statistically significant differences. All (100%) of the spina bifida clinic
group saw their primary care provider annually as compared to 62.2% of the non clinic
group. All (100%) of the spina bifida clinic group reported that they saw a
neurosurgeon/neurologist and urologist annually as compared to 24.3% and 45.9% of the
non clinic group, respectively. Seven (77.8%) of the spina bifida clinic group reported
seeing an orthopaedic surgeon on an annual basis as compared to 18.9% of the non clinic
group. These data indicated that adults with spina bifida who receive health care through
a spina bifida clinic are much more likely to receive recommended health care. This
disparity in follow up was also found by Kaufman et al. (1994). They demonstrated that
66% of former clinic patients without ongoing clinic care had no neurosurgical follow-up
as compared with 22% of former clinic patients who were receiving ongoing clinic care.
Rates for orthopaedic follow up between the disbanded clinic patients without ongoing
clinic care and those with ongoing clinic care were 50% and 25%, respectively. Rates
for urological follow up between the former clinic patients without ongoing clinic care
and those with ongoing clinic care were 45% and 30%, respectively. Hayward et al.
(1991) also was able to demonstrate that a regular source of ambulatory care impacted on
the receipt of medical care: 56% of women, 20 years or older, who had a regular source
of ambulatory care reported having had a Pap smear within the past year as compared
with 46% of women who had no regular source of ambulatory care. Similarly, for
women 40 years of older, 59% who had a regular source of ambulatory care reported
48
having had a breast exam by a physician within the past year as compared with 31% who
had no regular source of ambulatory care.
Secondary Conditions. The most frequently reported secondary conditions in this
study were urinary tract infections (78.3%), pressure ulcers (63.0%), obesity (32.6%),
changes in ability to walk (32.6%), change in brace needs (26.1%), behavioral issues
(26.1%), and osteomyelitis (21.7%). Farley (1996) found that pressure sores (83.1%),
bums (59.3%), muscle atrophy (51.7%), scoliosis (51.2%), and leg fractures (43.5%)
were among the leaders in secondary conditions in his study. The incidence of urinary
tract infections in his study was significantly lower (39.5%), but these were qualified by
having a febrile component to them.
A comparative analysis between the two identified subgroups and the incidence
of specific secondary conditions yielded no statistically significant differences. The
sample size limited a relevant comparison. It appears though, that routine care through a
spina bifida clinic may influence the incidence of behavior issues: 11.1% of spina bifida
clinic patients reported a history of behavior issues compared to 29.7% of non clinic
patients. This may reflect a higher locus-of-control in the clinic patient group.
The spina bifida clinic group reported a 44.4% incidence rate of change in brace
needs as compared to 21.6% of the non clinic group. It can be inferred that clinic
patients were more apt to proactively seek orthotic care than the non clinic patients, or
that clinic patients were more apt to still be wearing orthoses. This question was found
to be lacking in content validity.
The incidence of obesity was slightly higher in the spina bifida clinic group
(44.4%) than the non clinic group (29.7%). While obesity was defined as 20 pounds
49
over the subject s ideal weight, the researcher did not take into consideration the
respondent’s knowledge of ‘ideal weight’.
The incidence of osteomyelitis was slightly higher in the non clinic group
(24.3%) than the spina bifida clinic group (11.1%). This may reflect the tendency for a
higher incidence of pressure ulcers in the non-clinic group (64.4%) as compared to the
spina bifida clinic group (55.6%) developing from pressure ulcers.
There was a higher incidence of ambulatory changes in the spina bifida clinic
group (44.4%) than the non clinic group (29.7%). This, however, may have been
influenced by the number of subjects who were ambulatory at the age of 21; this was not
asked.
The incidence of urinary tract infections was almost identical in the two groups.
This may help to explain why urologists were the second most visited health care
provider in this study.
The total reported incidence of secondary conditions, excluding brace changes, in
the spina bifida clinic group was 100% and 94.6% in the non clinic group. Total
incidence of secondary conditions was reported as a sum total of either a positive or
negative history for all the specific secondary conditions listed and reported and not
quantified per number of occurrences. It would have been beneficial, in providing a
more accurate indication of the relationship between frequency of healthcare and
incidence of secondary conditions, to quantify number of occurrences of secondary in the
two subgroups.
Surgical Interventions. Fifty-one surgical interventions were reported; some
subjects had more than one intervention. For descriptive purposes, the surgical
50
interventions were procedurally classified as either orthopaedic, urological, neurological,
or other. Orthopaedic interventions were most frequent (54.9%) and urological
interventions occurred second most frequently (23.5%). What is interesting to note is
that while orthopaedic interventions ranked highest in frequency, visits to orthopaedic
surgeons ranked lowest in frequency.
Comparisons were made between the two subgroups and the incidence of
different surgical interventions. The incidence of orthopaedic procedures was higher in
the non clinic group than the spina bifida clinic group, 57.1% and 44.4% respectively.
The incidence of urological interventions was also higher in the non clinic group (26.2%)
as compared to clinic group (11.1%). Perhaps, due to a limited sample size, the results
were not statistically significant.
In reviewing the types of surgical interventions in each classification, it became
evident that certain procedures may be influenced by the frequency of routine health
care. A board certified orthopaedic surgeon, working in a pediatric orthopaedic facility,
was asked to rank each of the surgical interventions as either proactive, reactive, or
unrelated. His rankings were based on data obtained in the survey (Appendix E).
Definitions for these rankings appear in Chapter III.
A comparison was made between the two subgroups and the incidence of
proactive, reactive, and unrelated interventions. The sample size was limited and the
results were not statistically significant. A point of interest, however, was that there was
a 76.2% rate of reactive surgeries in the non clinic group as compared with a 55.6% rate
in the spina bifida clinic group. This suggests that frequency of routine care may impact
on the incidence of reactive surgical interventions. This relationship was demonstrated
51
by Kaufman et al. (1994) who found that 9 of 39 (23.1%) former clinic patients without
on-going clinic care experienced surgical decubitus repair as compared to 1 of 100 (1%)
former clinic patients who were receiving on-going clinic care.
Satisfaction of Spina Bifida Care. Despite a small sample size, which impacted
on statistical significance, a definite trend emerged in how satisfied the respondents were
with the ability of the primary care provider to care for the adult with spina bifida. None
of the spina bifida clinic group ranked their primary care provider less than adequate as
compared to 37.8% of the non-clinic group. One can infer that satisfaction increases
with frequency of care.
When considered in light of Dorothea Orem’s theory of self-care (1995), the
results of this study indicated that adults with spina bifida were not aware of the self-care
requisites entailed in managing their health. It appeared that attendance at a spina bifida
clinic provided a greater opportunity for individuals to meet their therapeutic self-care
demands. In the absence of a spina bifida clinic, it was evident that the primary care
provider was most frequently called upon to deliver care in an effort to promote health.
According to Orem (1995), the primary care provider should be responsible for
providing health care in an effort to achieve the adjustments and adaptations necessary
for continued support of life and integrated functioning. The primary care provider
needs to incorporate a continuous assessment of health in light of the adult’s spina bifida
and incorporate recommended specialty care in an effort to prevent secondary conditions.
Conclusions
This study indicated that adults with spina bifida are not receiving healthcare as
frequently as recommended. However, it was demonstrated that adults who attended a
52
spina bifida clinic received health care more frequently and come closer to meeting these
recommendations. Due to a limited sample size, this study was not able to significantly
demonstrate a relationship between frequency of health care and the incidence of
secondary conditions. In addition to this, the limitations described in Chapter I effected
the conclusions that could be drawn: the sample for study was obtained from a listing of
patients who had been served at one pediatric orthopaedic facility in northwestern
Pennsylvania, different levels of morbidity may be related to the various levels of spinal
defect rather than the level of care the patient is received, and not all adults with spina
bifida were able to verbally respond to questioning and therefore their caretaker may
have served as respondent for this survey.
Recommendations
This study indicated that adults with spina bifida are more likely to receive
recommended health care through a spina bifida clinic. Further research is necessary to
demonstrate that the recommended frequency of health care results in a lower incidence
of secondary conditions. Some recommendations for further research are:
1. Repeat this study with a larger sample size.
2. Repeat this study but take a prospective approach versus subjects self reports.
3. Repeat this study with a population of adults with spina bifida who receive
health care through a spina bifida clinic.
4. Study how the general aging process affect the incidence of secondary
conditions.
5. Study primary care providers regarding their perceptions of appropriate care
for adults with spina bifida.
53
6. Study primary care providers to determine their knowledge level regarding
secondary conditions in adults with spina bifida.
7. Study adults with spina bifida to determine their knowledge level regarding
secondary conditions of spina bifida.
8. Study how access to care issues may impact on adults with'spina bifida
seeking healthcare.
If the first three recommended studies indicated that frequency of healthcare was
inversely related to the incidence of secondary conditions, despite the general aging
process, then the fourth, fifth, and sixth recommended studies could be performed to
determine if knowledge deficit in the primary care provider or adult with spina bifida
served as a barrier. If this were the case, then education of primary care providers in the
health care needs of their adult patients with spina bifida would be indicated as well as
education of the adult with spina bifida. If, as determined by the seventh study, that
access to care affected the frequency of care sought by the adult with spina bifida, then
advocating for change at the governmental level would be indicated.
Summary
This chapter provided a summary of this research project. Data indicated that
adults with spina bifida do not receive the recommended healthcare at the recommended
frequency. It demonstrated, however, that adults in a spina bifida clinic have a higher
incidence of receiving annual health care at the recommended frequency. Due to a
limited sample size, this study did not support the hypothesis that there is an inverse
relationship between the frequency of healthcare and the incidence of secondary
54
conditions in the adult with spina bifida. Discussions of these findings and
recommendations for further study were provided.
55
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treatment. Archives of Physical Medicine and Rehabilitation, 70, 627-641.
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D. P., & Slap, G. B. (1993). Transition from child-centered to adult health-care systems
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Council on Child and Adolescent Health (1988). Age limits of pediatrics.
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Dorval, J. (1994). Achieving and maintaining body systems integrity and
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bifida or cerebral palsy, proceedings and recommendations of a symposium, Donald L.
Lollar, Ed.D. (Ed.), 65-77.
Farley, T. L. (1996). Secondary disabilities in Arkansas with spina bifida.
Unpublished data presented at Independence: The ultimate frontier. The Spina Bifida
Association of America Annual Conference held June 19-21,1996 in Phoenix, AZ.
Farrow, J. A. (1992). Introductory remarks. In Transitions in care for young
adults with special health needs, proceedings of a regional conference held on April 23
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Hallum, A. (1995). Disability and the transition to adulthood: Issues for the
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12-50.
Hayward, R. A., Bernard, A. M. A, Freeman, H. E., & Corey, C. R. (1991).
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Health Resources and Services Administration (1992). Moving on ... Transition
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National Center for Education in Maternal and Child Health,.
Kaufman, B. A., Terbrock, A., Winters, N., Ito, J., Klosterman, A., & Park, T. S.
(1994). Disbanding a multidisciplinary clinic: Effects on the health care of
myelomeningocele patients. Pediatric Neurosurgery, 21, 36-43.
Koop, C. E. (1989). Surgeon General’s introductory remarks. In Growing up
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Laurence, K. M. (1964). Natural history of spina bifida cystica: Detailed
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Lollar, D. J. (1994). Encouraging personal and interpersonal independence. In
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proceedings and recommendations of a symposium, Donald L. Lollar, Ed.D. (Ed.), 1725.
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MRC Vitamin Study Research Group (1991). Prevention of neural tube defects:
Results of the Medical Research Council vitamin study. The Lancet, 338(8760) 131137.
McLone, D. G. (1989). Spina bifida today: Problems adults face. Seminars in
Neurology, 9(3), 169-175.
McLone, D. G., Dias, L., Kaplan, W. E„ & Sommers, M. W. (1985). Concepts in
the management of spina bifida. Concepts in Pediatric Neurosurgery. 5, 97-106.
McManus, P. (1989). Adolescents and young adults with special health care
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Milunsky, A., Jick, H., Jick, S. S., Bruell, C. L., MacLaughlin, D. S., Rothman,
K. J., & Willett, W. (1989). Multivitamin/folic acid supplementation in early pregnancy
reduces the prevalence of neural tube defects. JAMA, 262(20), 2847-2852.
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Year Book, Inc.
Patrick, D. L., Richardson, M., Starks, H. E., & Rose, M. A. (1994). A
framework for promoting the health of people with disabilities. In Preventing secondary
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recommendations of a symposium, Donald L. Lollar, Ed.D. (Ed.), 3-16.
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Rauen, K. (Ed.) (1990). Guidelines for spina bifida health care services
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Rauen, K., & Aubert, E. J. (1992). A brighter future for adults who have
myelomeningocele - One form of spina bifida. Orthopaedic Nursing, 11(3), 16-26.
Reiss, J. G., & Dearholt, D. (1997). Directory of state Title V CSHCN programs
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Rosen, D. (1995). Between two worlds: Bridging the cultures of child health and
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Sandler, A. (1997). Living with spina bifida: A guide for families and
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conference recommendations. Journal of Adolescent Health, 17(1), 6-9.
59
Appendices
60
Appendix A
Dear Doctor,
Priority Ranking
I need to classify certain surgical procedures as part of my research project on spina
bifida. I have asked the survey participants what surgical procedures they have had since
turning the age of 21. Below is a listing of those surgical procedures; I’d like you to
classify them as:
1: proactive, i.e., those procedures that are preventative in nature and are performed
generally to prevent complications secondary to spina bifida and more likely in patients
with appropriate follow-up
2: reactive, i.e., those procedures that are generally performed as a result of a secondary
complication and more likely in patients with inappropriate follow-up
3: non-related, i.e., those procedures that would have taken place irregardless of the
patient’s myelo status and appropriate/inappropriate follow-up would have no bearing
1. Removal of bone (partial) as a result of infection/osteomyelitis
2. Removal of infected axillary nodes; result of infection/trauma from crutches
3. Debridement/closure of pressure ulcers
4. Artificial bladder sphincter
5. Release of tethered cord
6. C-section
7. Leg amputation
8. Renal lithotomy/lithotripsy
9. Renal stent insertion
10. Fractured femur pinning (?cause of fx)
11. Drainage of abdominal abscess
12. External urinary diversion/revision of diversion
13. Toe amputation
14. Hydrocele repair
15. Cystoscope
16. Foot reconstruction
17. Fatty tissue removed from bowel
18. Tom meniscus repair
19. Spinal fusion
20. Shunt Repair
ank^mi for your time,
rypmro, MSN (candidate)
Z
•2I
T
3
*2-
z
3
2
I
2-
-3_
2^
61
Appendix B
Selection from
Recommendations for the Adult Years
Guidelines For Spina Bifida Health Care Services Throughout Life
(Rauen, 1990)
Recommendations for the Adult Years
1.
Annual check-ups and episodic care by primary care providers and
provision of care coordination with specialty disciplines.
2.
Annual assessment for shunt function and presence of tethered cord,
hydomyelia, and Chiari malformation by a neurosurgeon and appropriate follow-up care.
3.
Annual evaluation with an orthopedic specialist for orthotic equipment,
mobility equipment, degenerative problems, and appropriate follow-up care.
4.
Evaluation annually by a urologist for a renal ultrasound and assessment
of urinary status with appropriate follow-up care.
62
Appendix C
Introductory Script
Hello, my name is Mary Ferraro and I am a Nursing Supervisor at Shriners
Hospital For Children in Erie, Pennsylvania. I am also a graduate student at Edinboro
University. As part of the requirements for graduation I am conducting a survey on how
adults with spina bifida are getting their healthcare needs met. I would appreciate you
taking ten minutes of time to answer the following questions. Only I will know who was
called. All information will be kept confidential. No one person will be identified. In
no way does your participation or non-participation affect your healthcare.
Are you willing to participate? If yes, let’s continue on with the questions.”
If the respondents question the meaning of the secondary conditions listed in this
tool, the following explanations will be provided:
“Pressure sores are blisters or open areas on your skin that were caused by either
pressure or rubbing of that area.”
“Urinary tract infections are when bacteria grows too much in your urine and can
cause you to either run a fever or have bad smelling urine, or both. Usually the doctor
will prescribe medicine for several days to help you get rid of them.”
Kidney failure is when your kidneys no longer make urine and you become very
ill. You would be put in the hospital if this occurred and perhaps be on dialysis.”
“Loss of a kidney is when the doctor has to surgically take out one of your
kidneys.”
63
“High blood pressure is when your blood pressure reading is over 140/80 on a
regular basis; sometimes the doctor may put you on medication to treat your high blood
pressure.”
“Obesity is being 20 pounds over what your ideal weight should be.”
“Fractures are when your bones are broken.”
“Change in brace needs means that your braces are not fitting you well or they are
in bad shape and need repaired.”
“Behavior issues are emotions or feelings that seem to prevent you from enjoying
life. They may also cause you to use drugs or alcohol.”
“Changes in ability to walk means that you can’t get around the way you used to,
for example if you used to walk with crutches most places and now you only use them
around the house and use a wheelchair most other places, you have had a change in your
ability to walk.”
“Changes in bowel function means that your bowels patterns have changed; either
you have developed diarrhea or constipation and this is not normal for you.”
u
«____
!
Shriners
Hospitals
64
Appendix D
r-1
tf
GDanncacpceDo
Erie
Hospital
July 25, 1997
1645 West 8th Street
Erie, Pennsylvania 16505
Tel. 814-875-8700
Fax 814-875-8756
Richard W. Brzuz
Administrator
James 0. Sanders, M.D.
Chief of Staff
To Whom It May Concern:
Mary Ferraro has been working with the Research Committee of Shriners
Hospitals for Children-Erie in order to develop a needs assessment tool in an
effort to query adults with myelodysplasia about their current level of functioning
and health care seeking behaviors. She has permission to access our patient data
base and select subjects from our active/inactive myelodysplasia patient list.
Karl F Frankovifch, M.D.
Emeritus Chief of Staff
Sincerely yours,
OFFICERS OF
LOCAL BOARD
OF GOVERNORS
William D. Bessor
Chairman
Robert F Thompson
Vice Chairman
James H. Richardson, Jr.
Treasurer
Richard A. Henderson
Secretary
AFFILIATED TEMPLES
Al Koran
Damascus
Ismailia
Jaffa
Mocha
Moslem
Osiris
Rameses
Syria
Tadmor
Zem Zem
Zenobia
James OfSanders,
Chief'of Staff
JOS:dct
65
Appendix E
DEMOGRAPHIC INFORMATION
Patient Number.
1. Information provided by
self iQ
caretaker 2Q
2. Sex
3. Age
4. Current Payment Method for Healthcare Services
private insurance
public insurance
self-pay
none
combination
iQ
20
30
4Q
sQ
0-30 miles
31-50 miles
51-100 miles
101 or more
iQ
2Q
3Q
40
5. Approximate Distance From Erie
6. History of Hydocephalus
yes !□
no 2Q
GENERAL HEALTHCARE INFORMATION
7. On an average, how many routine visits, not sick visits or emergencies, do you usually
make to a primary care provider (family doctor, clinic doctor, nurse practitioner, etc.) in a
year?
none 1Q
one or more 2Q
66
8. How many routine visits, not sick visits or emergencies, have you made to a primary
care provider (family doctor, clinic doctor, nurse practitioner, etc.) in the last year?
none iQJ
one or more 2
9. If you have not made a routine visit to a primary care provider in the last year, why
not?
distance
no insurance
no provider
transportation
don't think I need it
1Q
20
3Q
4Q
5Q
10. On an average, how many routine visits, not sick visits or emergencies, do you
usually make to a neurosurgeon/neurologist in a year?
none 1Q
one or more 2|^J
11. How many routine visits, not sick calls or emergencies, have you made to a
neurosurgeon/neurologist in the past year?
none 10
one or more 2Q
12. If you haven't made a routine visit to a neurosurgeon/neurologist in the past year,
why not?
distance
no insurance
no referral
no provider
transportation
don't think I need it
1Q
20
3Q
sQ
6Q
67
13. On an average, how many routine visits, not sick visits or emergencies, do you
usually make to an orthopedic surgeon in a year?
none iQ
one or more 2Q
14. How many routine visits, not sick calls or emergencies, have you made to an
orthopedic surgeon in the past year?
none iQ
one or more 20
15. If you haven't made a routine visit to an orthopedic surgeon in the past year, why
not?
distance
no insurance
no referral
no provider
transportation
don't think I need it
2Q
3 [J
4Q
sQ
60
16. On an average, how many routine visits, not sick visits or emergencies, do you
usually make to a urologist in a year?
none iQ
one or more 2Q
17. How many routine visits, not sick calls or emergencies, have you made to a urologist
in the past year?
__ _________________________________
none 1Q
one or more 2Q
68
18. If you haven't made a routine visit to a urologist in the past year, why not?
distance
no insurance
no referral
no provider
transportation
don't think I need it
1Q
20
30
40
sQ
60
19. What other providers take care of your healthcare needs on a regular basis?
20. Have you experienced any of the following conditions since turning twenty one years
old?
pressure ulcers
urinary tract infections
kidney failure
loss of a kidney Q
high blood pressure
obesity
fractures
change in brace needs
behavior issues Q
changes in ability to walk Q
changes in bowel function
Other conditions not mentioned
21. What surgical procedures have you experienced since turning 21 years of age?
22. How well does your primary care provider take care of your spina bifida?
excellent
good
adequate
poor
not at all
1Q
2Q
40
5Q
69
23. If an adult specialty clinic was (established in Erie, Pa. where an orthopedist,
urologist, neurosurgeon, and primary/ care provider could see you regularly on the same
day, would you attend?
yes iQ
no 2Q
unsure 3Q
24. Are there any questions or concerns that I can discuss with you before we hang up?
70
Appendix F
Survey Data
1.
Information provided by
self
caretaker
29 (63%)
17 (37%)
2.
Sex
male
female
30 (65.2%
16(34,8%)
3.
Age
22-44
Mean 29.87
4.
Current payment method for health care
services
private insurance
public insurance
none
combination
17 (37.0%)
21 (45.7%)
2 (4,3%)
6(13%)
5.
Approximate distance from Erie
0-30 miles
31-50 miles
51-100 miles
101 or more miles
11 (23.9%)
1 (2.2%)
9 (19.6%)
25 (54.3%)
6.
History of hydrocephalus
Yes
No
27 (58.7%)
19(41.3%)
7.
On an average, how many routine visits,
not sick visits or emergencies, do you
usually make to a primary care provider
(family doctor, clinic doctor, nurse
practitioner, etc.) in a year?
None
One or more
14 (30.4%)
32 (69.6%)
None
One or more
14 (30.4%)
32 (69.6%)
8.
How many routine visits, not sick visits
or emergencies, have you made to a
primary care provider (family doctor,
clinic doctor, nurse practitioner, etc.)
in the last year?
71
9.
10.
11.
12.
13.
14.
15.
If you have not made a routine visit to a
primary care provider in the last year,
why not?
On an average, how many routine visits,
not sick visits or emergencies, do you
usually make to a neurosurgeon/
neurologist in a year?
How many routine visits, not sick visits
or emergencies, have you made to a
neurosurgeon/neurologist in the past
year?
If you haven’t made a routine visit to
a neurosurgeon/neurologist in the past
year, why not?
On an average, how many routine visits,
not sick visits or emergencies, do you
usually make to an orthopaedic surgeon
in a year?
How many routine visits, not sick calls
or emergencies, have you made to an
orthopaedic surgeon in the past year? ..
N/A
No insurance
No provider
Unnecessary
32 (69.6%)
1 (2.2%)
2 (4.3%)
11 (23.9%)
None
One or more
28 (60.9%)
18(39.1%)
None
One or more
33 (71.7%)
13 (28.3%)
N/A
No referral
Unnecessary
13(28.3%)
3 (6.5%)
30 (65.2%)
None
One or more
32 (69.6%)
14(30.4%)
None
One or more
36 (78.3%)
10(21.7%)
N/A
No referral
No provider
Unnecessary
10(21.7%)
5 (10.9%)
1 (2.2%)
30 (65.2%
If you haven’t made a routine visit to
an orthopaedic surgeon in the past year,
why not?
72
16.
17.
18.
19.
20.
On an average, how many routine visits,
not sick visits or emergencies, do you
usually make to a urologist in a year? ..
How many routine visits, not sick calls
or emergencies, of you made to a
urologist in the past year?
If you haven’t made a routine visit to
a urologist in the past year, why not? ..
What other providers take care of your
health care needs on a regular basis? ...
Have you experienced any of the
following conditions since turning
21 years old?
None
One or more
20 (43.5%)
26 (56.5%)
None
One or more
25 (54.3%)
21 (45.7%)
N/A
No referral
Unnecessary
21 (45.7%)
3 (6.5%)
22 (47.8%)
Spina bifida clinic
Gynecologist
PT/OT/Orthotist
Nursing/assistive
personnel
Psych services
Eye doctor
Other
9(19.6%)
4 (8.7%)
3 (6.5%)
Pressure ulcers
Urinary tract
infections
Kidney failure
Loss of a kidney
High blood pressure
Obesity
Fractures
Change in brace
needs
Behavior issues
Changes in ability
to walk
Changes in bowel
function
29 (63.0%)
5 (10.9%)
2 (4.3%)
3 (6.5%)
6(13.0%)
36 (78.3%)
2 (4.3%)
1 (2.2%)
7 (15.2%)
15 (32.6%)
8(17.4%)
12(26.1%)
12(26.1%)
15 (32.6%)
8 (17.4%)
73
Other conditions not mentioned
21.
What surgical procedures have you
experienced since turning 21 years
of age?
Osteomyelitis
Scoliosis
Lower extremity
change
Bladder function
change
Upper extremity
change
Tethered cord
Other renal
Back pain
New onset
hydrocephalus
Thrombus
Other
Removal of bone 2°
to infection
Removal of infected
axillary nodes
Debridement/
closure of pressure
ulcer
Artificial bladder
sphincter
Release of tethered
cord
C-section
Leg amputation
Renal lithotomy/
lithotripsy
Renal stent
insertion
Fractured femur
pinning
Drainage of
abdominal abscess
External urinary
diversion/revision
10(21.7%)
4 (8.7%)
3 (6.5%)
1 (2.2%)
1 (2.2%)
1 (2,2%)
2 (4.3%)
3 (6.5%)
1 (2,2%)
1 (2,2%)
3 (6.5%)
2 (3.92%)
1 (1.96%)
15 (29.4%)
1 (1.96%)
2 (3.92%)
1 (1.96%)
3 (5.88%)
2 (3.92%)
1 (1.96%)
1 (1.96%)
1 (1.96%)
5 (9.8%)
74
22.
23.
24.
How well does your primary care
provider take care of your spina
bifida?
If an adult specialty clinic was
established in Erie, Pa, where an
orthopaedist, urologist, neurosurgeon,
and primary care provider could see
you regularly on the same day, would
you attend?
Are there any questions or concerns
that I can discuss with you before we
hang up?
Toe amputation
Hydrocele repair
Cystoscope
Foot reconstruction
Fatty tissue removed
bowel
Tom meniscus
repair
Spinal fusion
Shunt repair
1 (1.96%
1 (1.96%
1 (1.96%
2 (3.92%
Excellent
Good
Adequate
Poor
Not at all
15 (32.6%)
5 (10.9%)
12(26.1%)
2 (4.3%)
12(26.1%)
Yes
No
Unsure
17(37%)
19(41.3%)
10(21.7%)
1 (1.96%)
1 (1.96%)
1 (1.96%)
2 (3.92%)
Vocational/insurance
1 (2.2%)
questions
Do I have spina
2 (4.3%)
bifida?
Questions re: current
symptoms
5 (10.9%)
Questions re: general
2 (4.3%'
information
Is there a support
1 (2,2%'
group?
c.2
Ferraro, Mary Schwab.
Healthcare needs
assessment of adults
1998.
Healthcare Needs Assessment of Adults with Spina Bifida
by
Mary Schwab Ferraro
Submitted in Partial Fulfillment of the Requirements
For the Master of Science in Nursing Degree
Approved by:
Alice Conway, Ph.D., R£><
Committee Chairperson
Edinboro University of Pennsylvania
~
>7/9 r
Date
Schilinig’I’h^CWAv/
Date
z Committee Member
Edinboro l^i^ersity of Pennsylvania
/
A
/pallet Geisel, Ph.D., RN
Committee Member
Edinboro University of Pennsylvania
7hh±
Date
Ac.
Healthcare Needs Assessment of Adults with Spina Bifida
Abstract
Literature indicates that secondary conditions are preventable in adults with spina
bifida provided they receive appropriate healthcare (Rauen & Aubert, 1992). A telephone
survey about adults with spina bifida was conducted to determine the reported frequency
with which they received primary and specialty care, the incidence of secondary
conditions and surgical interventions they had experienced since turning the age of 21,
and their level of satisfaction with their primary care provider’s ability to care for their
spina bifida. The tool utilized was a researcher-developed survey and assessed if
frequency of care impacted on the incidence of secondary conditions, surgical
interventions, and level of satisfaction with spina bifida care.
The sample consisted of 30 male and 16 female adults with spina bifida with a
mean age of 29.87. The results from this study indicated that adults with spina bifida
received more consistent and frequent care if they were seen in an adult spina bifida clinic
and that these patients appear to have a lowered incidence of decubitus ulcers, behavioral
issues, osteomyelitis, orthopaedic and urological surgical procedures, and reactive
surgical procedures. This research also found that adults who received healthcare through
a spina bifida clinic had a higher level of satisfaction with their primary care provider’s
ability to care for their spina bifida. Because of a limited sample size, not all results were
statistically significant. It is recommended that further research in this area utilize a
larger sample population and a prospective study approach in order to more accurately
determine the incidence of secondary conditions.
ii
Acknowledgments
I would like to acknowledge all those people who have helped to make this thesis
possible. I would like to thank the members of my thesis committee: Dr. Alice Conway,
Dr. Janet Geisel, and Dr. Judith Schilling for their time, energy and direction. I would
like to thank Tim Cooney, MS, for his advice on the statistical analysis of my study. I
would also like to thank the folks at Shriners Hospitals For Children, Erie: Pat Dylewski,
MSN, RN, Dr. Marguerite Jurkovic and all of my co-workers for their understanding and
patience. I would like to thank my family and Jeff, for their unending love and support.
But most of all, I would like to thank my daughter, Alaina, for without her, I never would
have made it.
iii
Table of Contents
Title
Page
Abstract
11
Acknowledgements
iii
List of Tables
vii
List of Figures
viii
Chapter I : Introduction
1
Background of the Problem
1
Theoretical Framework
3
Research Question
5
Assumptions
5
Limitations
6
Definitions of Terms
6
Summary
7
Chapter II : Review of Literature
9
Spina Bifida
9
Incidence and Etiology
9
Prolonged Survival Rates
11
Secondary Conditions
11
Factors Influencing Coordinated Care
12
Healthcare Delivery Models
12
Transitioning Services
16
iv
Funding
14
Summary
17
Chapter III : Methodology
18
Hypothesis
18
Operational Definitions
19
Research Design
20
Instrumentation
20
Pilot Study
21
Sample, Setting, and Procedure
22
Protection of Human Rights
22
Data Analysis
23
Summary
23
Chapter IV : Results
25
Sample
25
Demographics
26
Frequency of Healthcare Visits
27
Secondary Conditions
34
Surgical Interventions
Satisfaction of Spina Bifida Care
43
Future Attendance at an Adult Clinic
44
Additional Comments
44
Summary
45
v
Chapter V: Discussion
46
Summary of Research and Discussion
46
Demographics
46
Frequency of Healthcare Visits
47
Secondary Conditions
49
Surgical Interventions
51
Satisfaction of Spina Bifida Care
52
Conclusions
53
Recommendations
53
Summary
55
References
56
Appendixes
60
A. Priority Ranking
61
B. Selection of Guidelines For Spina Bifida Care Services
Throughout Life. Recommendations for the Adult Years
from the Spina Bifida Association of America
C. Introductory Script
62
63
D. Shriners Hospitals for Children, Erie Permission to
Access Previous Patient Records
65
E. Telephone Survey for the Adult with Spina Bifida
F. Survey Data
vi
List of Tables
Table
Page
1. Payment Methods
26
2. No PCP Visit Past Year
28
3. No Neuro Visit Past Year
28
4. No Ortho Visit Past Year
29
5. No Uro Visit Past Year
30
6. Surgical Classifications
41
7. Surgical Priority
42
vii
List of Figures
Figure
Page
1. Annual Primary Care Provider Visits
31
2. Annual Neurologic Visits
32
3. Annual Orthopedic Visits
33
4. Annual Urologic Visits
34
5. Behavioral Issue Incidence
35
6. Incidence of Change in Brace Needs
36
7. Incidence of Obesity
37
8. Incidence of Osteomyelitis
37
9. Incidence of Urinary Tract Infections
38
10. Incidence of Ambulatory Changes
39
11. Incidence of Decubitus Ulcers
40
12. Total Incidence of Secondary Conditions
40
13. Surgical Intervention Classification
14. Surgical Priority
viii
1
Chapter I
Introduction
This chapter provides an overview of spina bifida and the healthcare management
difficulties experienced by adults who suffer from this congenital disorder. Dorothea
Orem s (1995) self-care deficit theory of nursing is utilized as the theoretical framework
for this study and is described. Assumptions, limitations, and definitions of terms are
also provided.
Background of the Problem
Children born with chronic conditions are living longer today than in the past; it
is estimated that 85% of these children survive into adulthood (Farrow, 1992). These
figures are also true for children born with spina bifida (McLone, 1989). According to
McLone, at least 85% of children bom with myelomeningocele, a severe form of spina
bifida, can be expected to reach adulthood. The pediatric healthcare system has long
recognized that a multidisciplinary approach to the management of these children’s
special healthcare needs is most effective; unfortunately, in most instances, when these
children reach adulthood they must forfeit this model of care (Bronheim, 1989). It is at
this point that adults with spina bifida realize a higher risk for developing secondary
conditions that threaten their health, functioning and well-being (Rauen & Aubert, 1992).
Spina bifida is a conjgenital disorder that occurs within the first 4 weeks of fetal
development (Sandler, 1997). It is marked by bony anomalies of the spinal column.
When a malformation of the spinal cord and its covering (meninges) accompanies die
defect, it is called a myelomeningocele (Rauen, 1990). For the purpose of this study, the
2
term spina bifida will be used in reference to individuals that also have some degree of
myelomeningocele. Individuals with spina bifida also present with other neurological
conditions, primarily hydrocephalus, Chiari II malformation and tethered cord (Rauen,
1990). Sensory, motor, autonomic, and cognitive impairments result, the severity of
which depends on the level of defect and the extent to which the spinal cord and brain are
compromised (Sandler, 1997).
The incidence of spina bifida in the United States is approximately one out of
1,000 live births (Rauen, 1990). There are enclaves where the incidence is higher, for
example, the region extending from New England down to Georgia (Sandler, 1997). The
precise etiology of spina bifida is uncertain, however research has shown that vitamin
deficiencies, folic acid deficiencies, and environmental pollutants are likely causative
agents (Rauen & Aubert 1992).
There is an overwhelming consensus that children with spina bifida are best cared
for by a multidisciplinary healthcare team. According to Alexander and Steg (1989), the
team should consist of neurosurgery, orthopedics, urology, pediatrics and physical
medicine; nursing, physical therapy, occupational therapy, speech therapy, social work
and education should also be involved. Other key members might include a dietitian,
orthotist, psychologist/psychiatrist, endocrinologist and opthamologist (Rauen, 1990).
When the child leaves the realm of pediatric healthcare, however, this comprehensive
approach to healthcare management is generally not apparent (Bronheim, 1989).
According to Bronheim (1989),
3
While the interdisciplinary team approach to care has made an
appearance in the adult health care system (e.g. cardiac care clinics,
diabetes centers, etc.), it is not the model available in most specialty
departments. Many physicians in the adult care system are unfamiliar
with or even uncomfortable with this model of care” (p.6).
Physician attitudes and practices, ineffective healthcare delivery models,
inadequacies of insurance coverage, and lack of preparation of the adolescent and their
family for transitioning into the adult healthcare arena are a few factors that have
contributed to a lack of coordinated care (Koop, 1989). An unfortunate result of
inadequate healthcare for the adult with spina bifida is the development of secondary
conditions (morbidity) and subsequent loss of function (Rauen & Aubert, 1992). These
secondary conditions include obesity, pressure ulcers, urinary tract infections, kidney
failure, depression, chemical addiction, and loss of ambulation. Late deterioration is
common in patients with myelodysplasia; most, possibly all, of this deterioration is
preventable or correctable; it is not the natural course of the disease (McLone, 1989).
Theoretical Framework
Dorothea Orem explains her self-care deficit theory of nursing in Nursing:
Concepts of Practice (1995). She bases her theory of nursing on the belief that mature
individuals perform actions to meet the requirements for maintaining life, functioning,
development and well-being (self-care). The ability to engage in self-care is termed selfcare agency. Self-care requisite is an understanding of the actions necessary in the
regulation of human functioning and development. Therapeutic self-care demand is the
4
sum total of measures necessary to meet all of an individual’s known self-care requisites.
When therapeutic self-care demand exceeds an individual’s self-care requisites or
capabilities, self-care deficit ensues. The role of the primary care provider is to engage in
a deliberate course of action, dictated by the individual’s therapeutic self-care demands,
in order to maintain human functioning and development within the norms compatible
with life, health and well-being.
According to Orem (1995), the primary care provider’s focus must match the
healthcare requirements of the individual. Healthcare directed towards patients with
genetic and developmental defects would include:
1. Continuous healthcare to achieve the adjustments and adaptations the patient
needs for support of life processes and integrated functioning.
2. Continuous health evaluation to determine the effects of the defect on general
health, growth and development, and functioning.
3. Continuous diagnosis to determine the effects of the defect on medical
conditions.
4. Specific protection against complications or extension of present impairments
into more disabling limitations.
5. Rehabilitation of the patient as indicated.
6. Health maintenance and promotion as well as specific protection from actual or
possible effects of the defect on general health, growth and development, and
functioning.
5
Assisting the patient in assuming an appropriate role in continuing healthcare,
including self-care.
Primary care providers are obligated, according to Orem’s theory, to recognize
and chart a course of action to effectively meet the therapeutic self-care demands of
adults with spina bifida. In adults with spina bifida, this would include promoting not
only the routine health maintenance and prevention activities associated with maintaining
general health and growth and development but also those healthcare activities aimed at
preventing and treating secondary conditions associated with their spina bifida. By
assessing the healthcare services adults with spina bifida are currently receiving in the
community, nurse practitioners will be better able to identify their patients’ self-care
deficits, meet their therapeutic self-care demands and integrate routine health promotion
and education activities with disease specific prevention activities.
Research Question
Adults with spina bifida are at an increased risk for developing secondary
conditions (Patrick, Richardson, Starks & Rose, 1994). This increased risk for secondary
conditions can be attributed to a lack of coordinated healthcare (Rauen & Aubert, 1992).
Increased incidence of secondary conditions often translates into a loss of function and
independence for the adult with spina bifida (Lollar, 1994). For the purposes of this
study the following question is asked: Is there a relationship between coordinated care
and the incidence of secondary conditions?
Assumptions
The assumptions of this study were identified as follows:
6
1. Adults with spina bifida have unique healthcare needs associated with their
defect.
2. Adults with spina bifida are more apt to develop secondary conditions than
adults without disability.
3. The respondents will understand and answer the survey questions honestly.
4. Not all adults with spina bifida are able to verbally respond to questioning; if a
caretaker is needed to respond, their responses will be equivalent.
Limitations
The limitations of this study were identified as follows:
1. The sample for study was obtained from a listing of patients who had been
served at one pediatric orthopaedic facility in northwestern Pennsylvania and will effect
the generalizability of this study.
2. Different levels of morbidity may be related to the various levels of spinal
defect rather than the level of care the patient is receiving.
Definition of Terms
The terms utilized in this study were as follows:
1. Adulthood is the period of life after 21 years of age (Council on Child and
Adolescent Health, 1988).
2. Spina bifida is a condition of the spinal column where one or more dorsal or
posterior bony elements fail to fuse or unite (Rauen, 1990).
7
3. Myelomeningocele is a neural tube defect where there is a malformation of the
posterior elements of the vertebrae, protrusion of the meninges, and an external dural sac
which contains the neural elements (Alexander and Steg, 1989).
4. Secondary conditions are those further disabling conditions associated with, or
resulting from, the primary disability of spina bifida (Lollar, 1994).
5. Coordinated care is defined as attending a spina bifida clinic or annual visits
to a primary care provider, neurosurgeon/neurologist, orthopaedic surgeon,
and urologist.
Summary
Children born with spina bifida have a better chance of surviving until adulthood
than ever before (Rauen & Aubert, 1992). This is due to major strides made in managing
their unique healthcare needs over the last several decades (Bronheim, 1989). Experts
agree that a comprehensive, multidisciplinary approach to meeting their healthcare needs
has been very effective (Bronheim, 1989). Unfortunately, once children with spina bifida
leave the pediatric healthcare system and enter the adult healthcare arena, they frequently
find that this comprehensive model of managing their healthcare no longer exists; they
are left to manage their own healthcare or rely on their primary care provider to do so
(Bronheim, 1989). As a result, adults with spina bifida are more likely to develop
secondary conditions (Rauen & Aubert, 1992). These secondary conditions threaten their
ability to function independently (Lollar, 1994). If put in the context of Dorothea Orem’s
theory of self-care (1995), they are no longer able to engage in self-care agency. Orem
8
states that the primary care provider needs to adjust and adapt the health care orientation
to meet the therapeutic self-care demands of the adult with spina bifida.
The purpose of this study was to examine if the healthcare needs of adults with
spina bifida were being met and by whom. Assumptions, limitations, and terms utilized
in this study have been provided.
9
Chapter II
Review of Literature
This chapter provides a review of current literature on spina bifida and the
healthcare difficulties experienced by adults who suffer from this congenital disorder.
The purpose of this chapter is to provide the reader with a selective overview of the
population to be studied, some of their specific healthcare issues, and problems associated
with obtaining appropriate healthcare.
Spina Bifida
Spina bifida is a term that means a split or divided spine and may be an open or
closed defect (Sandler, 1997). Closed spina bifida, or spina bifida occulta, is a relatively
common condition in which there is a malformation of the bones of the spine but the
defect does not usually involve the spinal cord which is intact within the body tissues
(Sandler). It is generally thought that most people with spina bifida occulta do not suffer
from any physiological sequellae (Sandler).
Myelomeningocele, commonly referred to as spina bifida, involves not only bony
malformations of the posterior elements of the spine but also presents with a protrusion of
the meninges and an externalized dural sac which contains neural elements of the spinal
cord (Alexander & Steg, 1989). It is associated with neurological impairment at the level
of the defect and below (Rauen & Aubert, 1992).
Incidence and Etiology. The highest incidence of spina bifida worldwide can be
found in China where between 10 and 20 babies are bom with spina bifida per 1000 live
births (Sandler, 1997). The British Isles, primarily Ireland and Wales, have an incidence
10
of 3 to 6 per 1000 live births (Alexander & Steg, 1989). In the United States, the average
incidence is approximately 1 per 1000 live births with a higher incidence found along the
mountainous region extending from New England down to Georgia (Sandler, 1997).
According to Sandler (1997) “populations that move to these areas seem to have
incidence rates similar to those of more indigenous populations, whereas groups that
move to other areas have lower incidence rates” (p. 28). There is also evidence indicating
there was an epidemic outbreak of spina bifida in the United Kingdom in the 1870s and
one in the United States in the 1930s. Both of these time frames coincided with poverty
and social upheaval suggesting that both environmental factors and nutrition played a
significant role (Sandler, 1997).
Milunsky et al. (1989) reported that multivitamin /folic acid supplementation in
early pregnancy reduced the prevalence of spina bifida. They prospectively interviewed
22,776 women, who were undergoing amniocentesis, about their use of multivitamins.
Neural tube defects, of which spina bifida is a type, were three times more likely among
those women who were not taking vitamins or who took vitamins after the sixth week of
pregnancy than among those who took folic acid during the first six weeks. This was
supported by a more recent study which concluded that supplemental folic acid in the diet
of pregnant women was found to be preventative for spina bifida (Medical Research
Council Vitamin Study Research Group, 1991). In this randomized double-blind
preventative trial, 1817 women at high risk of having a pregnancy with a neural tube
defect, because of a previous affected pregnancy, were randomly assigned to either a
folic acid supplementation group, other vitamin supplementation group, folic acid and
11
other vitamin supplementation group, or a no supplementation group. Results of the
study showed a 79% protective factor for women who received folic acid
supplementation; the other vitamins showed no protective effect.
Prolonged Survival Rates. Laurence’s (1964) investigation of 407 children bom
with spina bifida between 1947 and 1956 revealed only a 29% survival rate until the age
of 12 for those newborn infants with myelomeningocele. Since that time, effective
surgical procedures have been developed to successfully close the back defect and
alleviate the often fatal complication of hydrocephalus. McLone et al. (1985) studied two
separate cohorts of 100 myelomeningocele patients. The overall mortality rate for the
initial cohort of 100 patients followed for 8 to 12 years after closure of the back was 15%.
Survival curves for the second cohort of 100 children were identical to the first. The
studies documented that 2% of the children did not survive to leave the hospital, despite
back closure and shunting; 10% died by the end of their third year; and a total of 14%
died by the end of 5 years. Death was unusual after 48 months, when the mortality
curves leveled off. Today, it is projected that at least 85% of individuals bom with spina
bifida can be expected to reach adulthood (McLone, 1989).
Secondary Conditions. Because of neurological compromise, individuals with
this disease present with musculoskeletal dysfunction, sensory deficits, bowel and
bladder impairment and some degree of sexual dysfunction; many also have learning
disabilities (Rauen & Aubert, 1992). While no hallmark study could be found in the
literature detailing all possible secondary conditions in adults with spina bifida, Farley
(1996) presented data collected by the Arkans;;as Spinal Cord Commission (ASCC) on
12
many secondary conditions. In 1993, the ASCC surveyed all known Arkansas residents
with spina bifida to determine the type and prevalence of secondary disabilities. ASCC
managers interviewed 380 individuals/families out of a possible 419 for a 91%
completion rate of known cases. The study determined that, in individuals over the age
of 20 years, 83.1% experienced pressure sores, 51.7% experienced muscle atrophy,
o9.5 /o experienced urinary tract infections, 43.5% experienced leg fractures, 12.4%
experienced cord tethering, 34.7% experienced bladder or kidney stones, and 9.8%
experienced osteoporosis. Other secondary conditions listed in the literature for which no
incidence data were found are obesity, renal insufficiency, hypertension, and depression
(Sandler, 1997). Virtually all of these conditions are preventable (Rauen, 1990; McLone,
1989; Sandler, 1997). However, it is only through close follow-up by trained healthcare
providers that these conditions can be anticipated and diagnosed early (McLone, 1989).
Without planned and coordinated care, secondary conditions ensue (Rauen and Aubert,
1992). These secondary conditions threaten the ability of the adult with spina bifida to
function independently (Lollar, 1994).
Factors Influencing Coordinated Care
There is debate in the literature concerning how to provide the best healthcare for
the ever-increasing numbers of adults with diseases of childhood onset (Rosen, 1995).
For adults with spina bifida, annual visits to a primary care provider, neurosurgeon,
orthopaedic surgeon, and urologist are among the recommendations made by the Spina
Bifida Association of America (Rauen, 1990). Hie general consensus is that adolescents,
when developmentally ready, should receive their healthcare in adult-orientcd settings
13
(Blum et al, 1993). According to Rouen (1995), “it iSMlve to Msume that providers of
adult health care are adept at delivering ongoing care of young adults with chronic
conditions and there is much to be learned from the pediatric experience” (p. 14).
Healthcare DelivgryModels, The Health Resources and Services Administration
has proposed a few models of care that provide transition from the realm of pediatric
care to the adult healthcare system. The Interdisciplinary/Multidisciplinary Team Model
features a care coordinator who provides the patient and family with comprehensive and
coordinated care by organizing the interdisciplinary/multidisciplinary team; this team
consists of, but is not limited to primary care providers, specialist physicians, nurses,
therapists, psychologists and psychiatrists, social workers, nutritionists, educators,
vocational rehabilitation and prevocational counselors, genetic counselors and
interpreters (Health Resources and Services Administration [HRSA], 1992). These teams
strengthen communication among providers and help to assure access to healthcare
services (HRSA, 1992). Unfortunately, while theoretically plausible, this model has very
little practical application at the present time for adults with spina bifida due to the very
low number of adult specialty clinics throughout the United States (Dorval, 1994).
The Generic Services Model is one in which a primary care provider provides the
care coordination with specialty medical care provided by specialists (HRS A, 1992). The
Family Practice Model is similar to the Generic Services model but treats the individual
in the context of the family allowing for continuity of care from childhood through
adulthood. In this model, the family practitioner is a generalist but, through wide
experience, referral to specialists is kept to a minimum; developmental issues are
14
considered the essential framework for medical care and “adult problems” such as weight
control, STDs and AIDS, substance abuse, contraception and prenatal care are also
considered within this context (HRS A, 1992). These two models can successfully meet
individuals healthcare needs to the extent that the primary care provider is well versed in
the problems associated with spina bifida. Lack of training is prevalent in the adult
healthcare sector where physicians are unfamiliar with the chronic conditions that affect
their disabled patients (HRS A, 1992). Few communities have physicians interested in
following adults with developmental or physical disabilities (Hallum, 1995). Kaufman et
al. (1994) conducted a retrospective telephone survey of 87 pediatric myelomeningocele
patients who had been treated at a multidisciplinary central nervous system malfunction
clinic. The clinic had disbanded 3 years prior to the survey and the researchers wanted to
determine the effects the clinic closure had on the healthcare of its former patients. They
determined that a significant problem with coordinated follow-up care existed: 66% of
the patients reported no pediatric follow-up, 66% reported no neurological follow-up,
50% reported no orthopaedic follow-up, and 66% reported no urological follow-up. They
found that the age of the patient had a highly significant correlation with the absence of
follow-up: 92% of subjects 16 years or older reported no neurological follow-up, 78%
reported no orthopaedic follow-up, and 60% reported no urological follow-up.
The Disease Specific Model has pediatric specialists continuing to provide care in
collaboration with their adult specialist counterparts. This model often lacks
comprehensive primary and preventative care (HRS A, 1992).
15
Transitioning Services. Failure to provide anticipatory guidance, or transitioning,
for the adolescent with spina bifida as they approach adulthood is also a concern (Blum et
al., 1993). There is no arbitrary age or developmental milestone at which transitioning
efforts should begin (Rosen, 1995). According to Rosen (1995),
Young people will be best prepared for the adult health care environment
if they are able to communicate independently with the health care
providers and can advocate forcefully on their own behalf. They should
have already assumed substantial responsibility for their own management and
have demonstrated themselves effective in their own care (p. 14-15).
The majority of health professionals, however, are either unaware or unsure of the issues;
this systemic inertia is fueled by insufficient information about transition options and
outcomes (Blum et al., 1993).
In 1991 the Center for Youth with Disabilities completed a national survey of
state agencies that impact on youth with disabilities and their families and found that
/o
of states reported that they provided some individual transition planning (Okinow, 1992).
In their summary of conference recommendations from Moving On. Transition From
Pediatric to Adult Health Care” held September 9-11, 1994, the Society For Adolescent
Medicine (1995) recommended:
1. Structure service delivery models to be responsive to the needs and
experiences of young people with disabilities and their families rather than to
accommodate health care professionals.
16
2. Design transition services to be developmentally appropriate addressing not
only the unique medical concerns but also the social, emotional, vocational, sexual, and
general health concerns of young people as well. Additionally, such services must
address lifestyle and risk behaviors.
3. Empower young people to take responsibility for their own health care by
providing them with the skills and knowledge needed for self-advocacy.
4. Identify, develop, and test models of transition services that define “best
practices” and evaluate the process, service mix, role of various providers, as well as the
costs and outcomes.
5. Expand research that evaluates the quality of care for young people with
chronic conditions documenting who needs and receives services, and the impact of
transition services on recipients and their families.
Funding. In addition to chaotic delivery of services, the adult with spina bifida
faces the challenge of funding the healthcare they require. At the age of 21, all 50 states
cease to offer care previously covered under Title V funding (Reiss & Dearholt, 1997).
In the late 1980s, 20% of disabled young adults, ages 19-24, were without any form of
health insurance protection at all; thousands more were insured but without adequate
coverage for the services they required (McManus, 1989). Kaufman et al. (1994) stated
that local government programs reimbursed at a lower rate for the same diagnosis in
adults than in children. Hayward et al. (1991) demonstrated that the uninsured were more
likely than the insured to lack a regular source of ambulatory care, 31.2% vs. 14.8%
respectively. They also reported that lacking a regular source of ambulatory care was a
17
risk factor for not receiving recommended medical care: 56% of respondents who had a
tegular source of ambulatory care reported having a PAP smear within the past year as
compared to 46% of respondents who lacked a regular source of ambulatory care, and
59% of respondents who had a regular source of ambulatory care reported having a breast
examination by a physician within the past year as compared to 31% of respondents who
lacked a regular source of ambulatory care.
Summary
This chapter has provided a review of the literature concerning spina bifida and
the difficulties experienced by adults who suffer from this congenital disorder. Presented
were the secondary conditions that can arise if comprehensive, coordinated healthcare is
lacking for these individuals. This review of literature noted that a lack of coordinated
care has resulted from a variety of factors.
18
Chapter III
Methodology
This chapter describes the methodology utilized to determine the frequency of
cooidinated care visits made by an adult with spina bifida to a primary care provider,
neurosurgeon/neurologist, orthopedic surgeon, and urologist, the incidence of secondary
conditions and surgical interventions in the adult with spina bifida, and the level of
satisfaction that exists with the primary care provider’s ability to care for the adult with
spina bifida. It also describes the methodology utilized determine if coordinated care is
related to the incidence of secondary conditions. Included in this chapter are the
hypothesis, operational definitions, research design, instrumentation, sample, setting, and
procedure, protection of human rights, and data analysis to be utilized for this study.
Additional information was collected that looked at the frequency of routine visits to a
primary care provider, neurosurgeon/neurologist, orthopaedic surgeon, and urologist, the
incidence of surgical interventions, and satisfaction with the primary care provider s
ability to care for the adult with spina bifida.
Hypothesis
There is an inverse relationship between the frequency of coordinated care and the
incidence of secondary conditions in the adult with spina bifida.
Operational Definitions
The operational definitions used in this study are identified as follows:
19
1. Primary care provider is either a medical doctor, osteopathic physician, or
nurse practitioner who provides routine health screenings and assessments, diagnostic
evaluations, treatment of treatable conditions within the scope of their practice and
monitoring of ongoing health conditions with appropriate referrals to specialists.
2. Secondary conditions are conditions that are causally related to the disabling
condition and that can be either a pathology, an impairment, a functional limitation, or an
additional disability. These disabilities include pressure ulcers, urinary tract infections,
kidney failure, loss of a kidney, high blood pressure, obesity, fractures, changes in brace
needs, behavior issues, changes in ability to walk, and changes in bowel funciton.
3. Surgical procedures were procedurally classified as either orthopaedic,
urological, neurological, or other.
4. Surgical procedures were also independently prioritized as either reactive
interventions, proactive interventions, or unrelated interventions. A board certified
orthopaedic surgeon, working in a pediatric orthopaedic facility, determined the priority
ranking of each surgical intervention based on the information available through the
survey (Appendix A). Reactive surgical interventions were defined as those
interventions which were performed as a result of a secondary condition and assumed less
frequent routine care. Proactive surgical interventions were defined as those
interventions which were performed in order to prevent a secondary condition and
assumed more frequent routine care.
Unrelated interventions were defined as those
interventions which would have taken place regardless of second^ conditions or
frequency of routine care.
20
5. Coordinated care is care that is received through a spina bifida clinic or annual
visits to a primary care provider, neurosurgeon/neurologist, orthopaedic surgeon, and
urologist.
Research Design
This study utilized a non experimental survey research design. It was a
descriptive correlational study comparing routine visits by adults with spina bifida to a
primary care provider, neurosurgeon, orthopedic surgeon, and urologist with the
incidence of secondary conditions, surgical interventions, and satisfaction with the
primary care provider’s ability to care for the adult with spina bifida.
Instrumentation
A telephone survey was utilized as the research tool (Appendix B) and was based
on the SBAA Guidelines for Spina Bifida Health Care Services Throughout Life (Rauen,
1990) (Appendix B). Due to lack of previous research in this area, the tool was
researcher-designed. The survey consisted of three sections. The first section elicited
demographic information concerning the respondent and their relationship to the adult
with spina bifida, either self or caretaker, the adult’s sex and age, current payment method
for healthcare services, approximate distance from Erie, Pennsylvania, and history of
hydocephalus.
The second section contained open-ended questions concerning the adult’s
frequency of routine healthcare visits with a primary care provider,
neuro surgeon/neurologist, orthopaedic surgeon, and urologist,:. If the respondent denied a
routine visit by the adult in the past year to either a primary earn provider, neurosurgeon,
21
orthopaedic surgeon, or urologist, their reason(s) were elicited. This section ended with
an open-ended question and determined if the adult received healthcare from any other
provider(s).
The third section contained specific and open-ended questions regarding the
adult s incidence of secondary conditions and surgical procedures. Question 19
determined the level of satisfaction with the primary care provider’s ability to take care of
the adult’s spina bifida. There were five possible responses from Excellent to Not at All.
Question 20 determined interest in attending an adult spina bifida specialty clinic in Erie,
Pennsylvania; possible responses were Yes, No, Unsure. Question 21 was open-ended
and intended to elicit any additional questions or concerns the respondent wished to add.
The survey was reviewed by the Research Committee at Shriners Hospitals For
Children, Erie for content validity. It was also reviewed by the Director of Research at
Hamot Medical Center in Erie, Pennsylvania and a representative of the Educational
Services Department at Edinboro University of Pennsylvania for content validity and
readability.
Pilot Study
The survey was piloted with a 24 year old female adult with spina bifida. The
decision was made not to employ Likert-type
scales, but rather, to allow for open-ended
responses to many of the survey questions. The participant completed the survey in less
than 10 minutes.
22
Sample, Setting, and Procedure
The convenience sample was adults with spina bifida over the age of 21. The
adult with spina bifida, or their caretaker in the event the adult was unable to respond,
served as the respondent to the survey; all understood and spoke English. The sample
was obtained from a listing of patients who had been served at one pediatric orthopaedic
facility in northwestern Pennsylvania and were 21 years of age or older. A telephone
survey was conducted in the first quarter of 1998. Telephone appointments for interviews
were made with the respondents at their convenience. The setting for this study was
whatever location they chose to respond to the survey. The procedure for this study was
for the researcher to read the introductory script to the respondent (Appendix C). If they
orally agreed to participate, the researcher proceeded with the survey.
Protection of Human Rights
A verbal introduction to the telephone survey was made to allow the respondents
to know the purpose of the survey (Appendix C). Agreement to participate in the survey
was deemed as informed consent. All data remained confidential, numbers were assigned
to the surveys and no names appeared. The researcher kept the list of identifying
information in a locked file cabinet and the data was reported as aggregate data. The
study was deemed exempt from a review by the Stainers Hospitals For Children Internal
Review Board (1RB) by representatives of the Research Committee at Stainers Hospitals
For Children, Erie (Appendix D).
23
Data Analysis
The first section was descriptively analyzed and trends were identified. The
second section looked at frequency of annual visits with a primary care provider,
neurosurgeon/neurologisl, orthopaedic surgeon, and urologist. These data were content
analyzed to determine if they met the criteria for coordinated care. Those subjects who
met the criteria for coordinated care were compared to those who did not. Subjects who
met the criteria for coordinated care reported receiving healthcare through a spina bifida
clinic. A Chi-square analysis, utilizing a Fischer’s Exact Test, was performed. This
analysis compared the annual visits between the identified spina bifida clinic group and
the non-clinic group.
The final section was content analyzed looking for themes. A Chi-square analysis
was attempted comparing the incidence of secondary conditions and surgical
interventions, and the satisfaction rating of the primary care provider’s ability to care for
the adult with spina bifida.
Summary
This chapter has identified the hypothesis, operational definitions, research
design, instrumentation, pilot study, sarniple, setting, and procedure, and protection of
human rights that were utilized for this study. It has identified that a descriptive
correlational study was utilized comparing routine visits by adults with spina bifida to a
primary care provider, neurosurgeon/neurologist, orthopaedic surgeon, turd urologist with
the incidence of secondary conditions, surgical interventions, and satisfaction with the
primary care provider's ability to care for the adult with spin, bifida. The hypothesis of
24
this study was that there is an inverse relationship between the frequency of routine health
care and the incidence of secondary conditions in the adult with spina bifida.
25
Chapter IV
Results
This Chapter presents the results obtained from a telephone survey about adults
with spina bifida (Appendix E). The results were descriptively analyzed and a Chi-square
analysis was performed on specific questions to determine if there was a statistically
significant difference between two identified subgroups in the sample population.
Sample
This study utilized patients that had once been seen and/or treated for spina bifida
at a small pediatric, orthopaedic hospital in northwestern Pennsylvania and over the age
of 21. Names and telephone numbers were obtained from the medical records of these
former patients.
The sample population numbered 96 and the investigator was able to contact 46
subjects (47.92%). All of those contacted were willing to participate in the survey and
were included in the research results.
The investigator initiated telephone contacts February 1, 1998 and completed
telephone contacts March 27, 1998. If the adult with spina bifida had relocated, either
their new telephone number was obtained or a family member was provided with a toll-
free number that the adult with spina bifida was instructed to use Monday-Friday, Sam4pm EST. In instances when a telephone number was incorrect or no longer in service,
the investigator utilized various person-locator sites on the internet in an effort to locate
26
the individual. These included Four-11 (www.fourli.oom) and Bigfoot
(www.bigfoot.com).
Demographics
Of the 46 completed telephone surveys, 29 (63%) were completed by the adult
with spina bifida and 17 (37%) were completed by the caretaker of the adult with spina
bifida. There were 30 (65%) male adults with spina bifida and 16 (35%) female adults
with spina bifida. The subjects ranged in age from 22 to 44 with a mean age of 29.87
(s.d.= 5.99).
Table 1 identifies the reported payment method for healthcare services. Private
insurance was defined as insurance provided by either an employer or paid for out of
pocket and public insurance was defined as insurance that was provided by either state or
federal programs.
Table 1
Payment Methods
n
(%)
private insurance
17
(37%)
public insurance
21
(45.7%)
2
( 4.3%)
6
(13%)
Type
no insurance
combination
n = 46 surveys were completed.
27
Of the sample population, 11 (23.9%) of ,he adults with spin, biMa lived wi,hi„
30 miles of Erie, Pennsylvania, 1 (2.2%) hved between 31 and 50 miles from Erie,
Pennsylvania, 9 (19.6%) lived between 51 and loo miles from Erie, Pennsylvania and 25
(54.3%) lived over 100 miles from Erie, Pennsylvania.
Because a large number of adults with spina bifida have a history of
hydrocephalus, respondents were asked if the subject had a history of hydrocephalus.
Twenty seven (58.7%) did have a history of hydrocephalus and 19 (41.3%) did not.
Frequency of Healthcare Visits
Fourteen (30.4%) of the adults with spina bifida reported, on an average, not
making a routine visit to a primary care provider annually; 32 (69.6%) of the adults with
spina bifida reported, on an average, making one or more routine visits annually. When
asked about the number of routine visits made to a primary care provider in the last year,
14 (30.4%) of the adults with spina bifida reportedly made no routine visit and 32
(69.6%) made one or more routine visits. Of the 14 who had not made a routine visit to a
primary care provider within the past year, most said they believed they didn t need to see
one (Table 2).
Twenty-eight (60.9%) of the adults with spina bifida reported not making a
routine visit to a neurosurgeon/neurologist annually; 18 (39.1%) reported making, on an
average, one or more routine visits mutually. When queried about the number of routine
visits made to a neurosurgeon/neurologist in the past year, 33 (7!.7%) of the adults with
spina bifida made no visits and 13 (28.3%) made one or more visits. Of the 33 who did
28
not make a routine visit to a neurosurgeon/neurologist within the past year, the majority
stated they didn’t feel it was necessary (Table 3).
Table 2
No Primary Care Provider Visit Past Year
Reason
no insurance
1
no provider
2
not necessary
n
n
11
14 subjects who did not see a primary care provider in last year.
Table 3
No Neurologic Visit Past Year
n
Reason
no insurance
not necessary
n
30
33 subjects who did not see a neurosurgeon/neurologist m past year.
Thirty-two (69.6%) of the adults with spina bifida reported not making a routine
visit to an orthopaedic surgeon annually; 14 (30.4%) reported making, on an average, one
or more routine visits annually. Thirty-six (78.3%) of the adults with spin, bifida
29
reported not making a routine visit to
an orthopaedic surgeon in the past year and 10
(21.7%) made one or more routine visits. Of the 36 who did not make a routine visit to
an orthopaedic surgeon within the past year, most felt it was unnecessary (Table 4).
Table 4
No Orthopaedic Visit Past Year
Reason
n
no referral
5
no provider
1
not necessary
30
n = 36 subjects who did not see an orthopaedic surgeon in last year.
Twenty (43.5%) of the adults with spina bifida were reported not making a routine
visit to a urologist annually; 26 (56.5%) reported making one or more routine visits
annually. Twenty-five (54.3%) of the adults with spina bifida reported not making a
routine visit to a urologist in the past year and 21 (45.7%) made one or more routine
visits. Of the 25 who did not make a routine visit to a urologist within the past year, 22
(88%) felt it wasn’t necessary (Table 5).
The respondents were asked which other providers routinely provided care to the
adult with spina bifida. Nine (19.6%) of the adults with spina bifida reported receiving
routine care through a spina bifida clinic.
A Chi-square analysis, utilizing a Fisher’s Exact Test, was performed. This
analysis compared the frequency of visits to a primary care provrder,
30
Table 5
No Urologic Visit Past Year
Reason
no referral
not necessary
n
3
22
n = 25 subjects who did not see a urologist in last year.
neurosurgeon/neurologist, orthopaedic surgeon, and urologist between the subgroup of
adults with spina bifida who received routine health care through a spina bifida clinic and
the subgroup of adults with spina bifida who did not receive routine health care through a
spina bifida clinic.
When comparing the frequency of the average number of annual visits made to a
primary care provider, there was a statistically significant (p = <0.05) difference between
spina bifida clinic patients and non clinic patients (Figure 1). All of the spina bifida clinic
patients saw a primary care provider an average of one or more times a year as compared
to 23 (62.2%) of the non clinic patients (Figure 1).
The difference in the frequency of routine visits within the past year between the two
subgroups was also statistically significant (p.-< 0.05). AU of the spina bifida clinic
patients saw a primary care provider within the past year while 23 (62.2%) of the non
clinic patients did.
31
.X?
100Y< 0Non clinic Patients
90-
J Spina Bifida Clinic Patients
807060OT
C
0
ra
CL
504030 -■
2010-
o+
___ A
None
One or more
Annual Neurologic Visits
p = < 0.05 significance compared spina bifida clinic patients with non clinic patients
Figure 2. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and annual routine neurologic care utilization.
There was a statistically significant difference (p.=< 0.05) between the two
subgroups in the frequency of reported average number of annual visits to an orthopaedic
surgeon. Seven (77.8%) of the spina bifida clinic patients saw an orthopaedic surgeon an
average of one or more times a year as compared to 7 (18.9%) of the non clinic patients
(Figure 3).
Although the frequency rates dropped in both subgroups, there was a statistically
significant difference (p.=< 0.05) in the frequency of visits to an orthopaedic surgeon
32
90T
0Non clinic Patients
■^Ping.^da Clinic Patients
80
70-
g 60<2
50' :
.2
40 <
a-
30-i
20-i
10-
o-r
None
One
or
more
Annual Orthopaedic Visits
p = < 0.05 significance compared spina bifida patients with non clinic patients
Figure 3. Comparative data between percentage of spinal bifida clinic patients (n = 9)
and non clinic patients (n = 37) and annual routine orthopaedic care utilization.
within the past year between the two subgroups. Five (55.6%) of the spina bifida clinic
patients were reported as visiting an orthopaedic surgeon in the past year as compared to
5 (13.5%) of the non clinic patients.
Finally, comparison frequency data of the reported average number of annual
visits made to a urologist was
also statistically significant (p.=< 0.05) between the two
subgroups. All (100%) of the spina bifida clinic patients saw a urologist an average o
one or more times a year as compared to 17 (45.9%) of the non clinic patients (Figure 4).
However, there was no statistically significant difference (p.=.O59) between the
two subgroups when comparing the frequency of repotted routine visits to a urologist
33
g
w
c
CL
100f
90- ® Non clinic Patients
* Spina Bifida Clinic Patients
80- >:•:
:;4: x:x
x ;:x;y
>:•;« x-x-x^x—v.-.- 70605040302010-
oNone
One
or
more
Annual Urologic Visits
p = < 0.05 significance compared spina bifida clinic patients with non clinic patients
Figure 4. Comparative data between percentage of spinal bifida clinic patients (n = 9)
and non clinic patients (n = 37) and annual routine urologic care utilization.
within the past year. Seven (77.8%) of the spina bifida clinic patients saw a urologist
within the past year as compared to 14 (37.8%) of the non clinic patients.
Secondary Conditions
The survey addressed incidence of secondary conditions in the adult with spina
bifida. These results determined that 36 (78.3 %) had a history of urinary tract
>f decubitus ulcers, 15 (32.6%) were obese, 15 (32.6
infections, 29 (63%) had a history oi
12 (26.1%) had changes in their brace needs,
%) had a change in their ambulatory status,
id 10 (21.7%) had a history of
12 (26.1%) reported having behavioral issues, am
osteomyelitis.
A Chi-square analysis, utilizing a Fisher’s Exact Test, was performed comparing
lhe incidence rates of the above secondary conditions between the subgroup of spina
34
bifida clinic patients and non chnie patients. Wre was no
difference (^.409) between the two subgroups and theiueidence of behaviors! issues
One (11.1%) of the spina bifida clinic patients reported having suffered with behavioral
issues as compared to 11 (29.7%) of the non clinic patients (Figure 5).
90-'
® Non clinic Patients
80-
■Spina Bifida Clinic Patients
70 -
g
w
c
60-
50 J
0)
40- i
CL
30- i
.■
20- i
10-
o-f4
No
Yes
Behavioral Issue Incidence
P = 0.409 significance compared spina bifida clinic patients with non clinic patients
Figure 5. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of behavioral issues.
There was no statistically significant difference (p.=-211) between the two
subgroups and the incidence of changes in brace needs. Four (44.4%) of the spina bifida
clinic patients had a reported change brace needs as compared to 8 (21.6%) of the
clinic patients (Figure 6).
There was no statistically significant difference (i>.=.«5) between the two
subgroups and the incidence of obesity.
Four (44.4%) of the spina bifida clinic patients
35
were reported to be obese as compared to 11 (29.7%) of the non
clinic patients (Figure
7).
® Non clinic Patients
80 Y
Spina Bifida Clinic Patients
70 J
60-
g
50-
w
c
40-
ro
30-
0)
£L
20-
I
10-
oNo
Yes
Incidence of Change in Brace Needs
P = .211 significance compared spina bifida clinic patients with non clinic patients
Figure 6. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of change in brace needs.
There was no statistically significant difference (p.=.659) between the two
subgroups and the incidence of osteomyelitis. One (11.1%) of the spina bifida clinic
patients were reported to have a history of osteomyelitis as compared to 9 (24.3%) of the
non clinic patients (Figure 8).
There was no statistically significant difference (p.=1.0) between th. two
subgroups and the incidence of urinary tract infections. Seven (77.S%) of th p
bifida clinic patients reportedly had a hlstory of urinary tract infections as compared to
29 (78.4%) of the non clinic patients (Figure 9).
36
80-f
0Non clinic Patients
70 -;
E§pina Bifida Clinic Patients
60-
? 50tn
c
o
co
q.
4030 <.•
20
‘
:
10-
o£
Incidence of Obesity
2
.445 significance compared spina bifida clinic patients with non clinic patients
Figure?. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of obesity.
□ Non clinic Patients
■ Spina Bifida Clinic Patients
908070-
■
■II
60OT
C
o
Q.
5040-
30 d
I
20- i
10- :
0-r
I
8
No
Yes
Incidence of Osteomyelitis
2 = .659 significance compared spina bifida clinic patients with non clinic patients
Eigure_8. Comparative data between percentage of spina bifida clinic patients (n 9) and
non clinic patients (n = 37) and incidence of osteomyelitis.
37
80
70-j
® Non clinic Patients
■ Spina Bifida Clinic Patients
60-i
g 50w
1 40’
£
302010<
I
o4
No
Yes
Incidence of Urinary Tract Infections
E = 1.00 significance spina bifida clinic patients with non clinic patients
Figure 9. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of urinary tract infections.
There was no statistically significant difference (p =.455) between the two
subgroups and the incidence of ambulatory changes. Four (44.4%) of the spina bifida
clinic patients had reported ambulatory changes as compared to 11 (29.7%) of the non
clinic patients (Figure 10).
There was no statistically significant difference (p.=.7O7) between the two
subgroups and the incidence of decubitus ulcers. Five (55.6%) of the spina bifida clinic
patients were reported to have a history of decubitus ulcers as compared to 24 (64.9%) of
non clinic patients (Figure 11).
There was no statistically difference (p= 1.0) between the two subgroups and the
total reported incidence of secondary conditions (Figure 12). Due to concerns regarding
38
th. content validity of reported brace changes, this category was exduded. Tout
incidence of secondary conditions was reported as a sum total of either a positive or
negative history for specific secondary conditions listed and reported and no, nuandfied
per number of occurrences. This refuted this study’s hypothesis.
80-
0 Non clinic Patients
70-
* Spina Bifida Clinic Patients
60-
? 50w
I 40‘
Q.
30 :
20*:
1004
No
Yes
Incidence of Ambulatory Changes
2 = .445 significance compared spina bifida clinic patients with non clinic patients
Figure 10. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of ambulatory changes.
Surgical Interventions
There were 51 reported surgical interventions by rhe sample population. For
descriptive purposes, they were procedurally classified as either orthopaedic, urological,
neurological, or other. There was a total of 28 (54.9%) orthopaedic procedures, 12
(23.5%) urological procedures, 3 (5.9%) neurological procedures, and 8 (15.7%) other
39
nNon clinic Patients
70
■Spina Bifida Clinic Patients
6050-
F
£
Jc 40^
.2
CD
30-
CL
20-
10-
■II
0
No
- J
Yes
Incidence of Decubitus Ulcers
p = .707 significance compared spina bifida clinic patients with non clinic patients
Figure 11. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and incidence of decubitus ulcers.
100-
999897-i
w
c
.2
co
Q.
0Non clinic
Patients
I
|
■Spina
Bifida
Clinic
96-:
959493-
Mid
■
92- i
91 +
Total Incidence of Secondary Conditions
£=1.0 significance compared spina bifida clinic patients with non clinic patie ts
Eigure_12. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and total incidence
of secondary conditions.
40
procedures (Table 6). Ota procedures Included things !Kh as
fatty abdominal tumor, and meniscus repair following a sledding injury.
Table 6
Surgical Classifications.
Reason
n
Orthopaedic
28
Urological
12
Neurological
3
Other
8
n = 51 surgical interventions.
The reported surgical interventions were also, but separately, prioritized as either
reactive interventions, proactive interventions, or unrelated interventions. There was a
total of 37 (72.5%) reactive interventions, 6 (11.8%) proactive interventions, and 8
(15.7%) unrelated interventions (Table 7).
Due to a limited sample size, a Chi-square test was not able to be performed
comparing the incidence of either the different surgical classifications or the different
prioritized surgical interventions between the subgroups of spina bifida clinic patie
and non-clinic patients. Although not statistically significant, when comparine the
subgroups, the spina bifida clinic group demonstrated a 44.4% rate of orthopaedic
procedures as compared to a 57.1 % rate in the non clinic group. Likewise, the spina
41
Table 7
Surgical Priority.
Reason
n
reactive
37
proactive
6
unrelated
8
n = 51 surgical interventions.
bifida clinic group showed a 11.1% rate of urological procedures as compared to a 26.2%
rate in the non clinic group (Figure 13).
60
0 Non clinic Patients
■Spina Bifida Clinic Patients
50-
40-
£
o>
co
o.
z
3020-
10-
[ •
0
Ortho
Neuro
Other
Surgical Intervention, Classification
g = .128 significance compared spina bifida clinic patients with non clinic patients
Figure 13. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and types of surgical interventions.
42
Again, despite its statistical insignificance, the comparison between the two
subgroups demonstrated a 55.6% rate in reactive interventions in the spina bifida clinic
group and a 76.2% rate in the non clinic group (Figure 14).
8070-
I
60-
0 Non clinic Patients
I
■
■Spina Bifida Clinic Patients
500
40-
CQ
Q.
3020-
10-
! s', ;
I I I
-
oReactive
Proactive
Unrelated
Surgical Priority
p = .129 significance compared spina bifida clinic patients with non clinic patients
Figure 14. Comparative data between percentage of spina bifida clinic patients (n = 9)
and non clinic patients (n = 37) and prioritized surgical interventions.
Satisfaction of Spina Bifida Care
Respondents were asked to rate how well the primary care provider takes care of
the adult with spina bifida. Fifteen (32.6%) of the respondents stated their primary care
provider was excellent, 5 (10.9%) stated they were good, 12 (26.1%) said they were
adequate, 2 (4.3%) said they were poor, and 12 (26.1%) said they did not take care of
their spina bifida at all.
A Chi-square analysis was performed comparing the satisfaction ratings of the
primary care provider’s ability to take care of the adult with spina bifida and the two
43
subgroups. Again, because of a limited sample size, the analysis violated assumptions in
the Chi-square test. Of note, however, 7 (77.8%) of the spina bifida clinic group rated
their primary care provider as excellent in taking care of their spina bifida as compared
to 8 (21.6%) of the non clinic group. None of the spina bifida clinic patients rated their
primary care provider less than adequate yet 14 of 37 (37.8%) of the non clinic group
patients rated their primary care provider as either poor or offering no care at all.
Future Attendance at an Adult Clinic
The respondents were asked if the adult with spina bifida would attend, if
available, an Erie-based adult multidisciplinary spina bifida clinic for routine health care.
Seventeen (37%) said they would attend, 19 (41.3%) stated they would not attend, and
10 (21.7%) stated they were unsure if they would attend.
Additional Comments
The respondents were asked if they had any other questions or comments. Five
(10.9%) of the respondents had questions regarding the current symptoms the adult with
spina bifida was experiencing, 2 (4.3%) asked if spina bifida was indeed an accurate
diagnosis of the adult with spina bifida, and 2 (4.3%) had general information questions.
Summary
This chapter presented the results from a telephone survey conducted with adults
with spina bifida or their caretaker. These results were interpreted through descriptive
analysis with the percentage of responses provided. Specific questions were also
subjected to Chi-square analysis and comparative data was provided for two identified
subgroups of the sample population. Due to a limited sample size, this study did not
44
support the hypothesis that there is an inverse relationship between the frequency of
health care and the incidence of secondary conditions in the adult with spina bifida.
45
Chapter V
Discussion
This chapter provides a summary of results which were obtained from a
telephone survey about adults with spina bifida (Appendix F). These results were
utilized to determine the frequency of routine primary and specialty care the adult with
spina bifida received and whether or not it was related to the incidence of secondary
conditions, surgical interventions, and satisfaction with the primary care provider’s
ability to care for the adult with spina bifida. Conclusions and recommendations based
on these results are also provided.
Summary of Research and Discussion
This section provides a summary of findings from this study. These findings
were compared to the recommended guidelines for spina bifida health care services for
the adult years.
Demographics. Information was provided by the adult with spina bifida when
possible but in the event they were not able to respond, the caretaker was surveyed. The
sample population represented a slightly higher percentage of males than females,
perhaps due to the difficulty of tracking individuals longitudinally by name and
telephone number alone. Women generally change their name when they marry and if
they relocate, this makes follow-up even more difficult.
The subjects ranged in age from 22 to 44 years with a mean age of 29.87. The
majority of the population had some form of insurance.
Twenty one (45.7%) of the subjects lived within a 100 mile radius of Erie,
Pennsylvania. This was perhaps an influencing factor for the 37% of respondents who
46
indicated an interest in attending an adult spina bifida specialty clinic in Erie,
Pennsylvania.
Approximately half of the population had a history of hydrocephalus. When
comparing the number of patients who received routine care through a spina bifida clinic
with non clinic patients, it was statistically significant that all of the spina bifida clinic
patients had a history of hydrocephalus as compared to the 48.6% of the non clinic
patients. It can be inferred that having a history of hydrocephalus was an influencing
factor in seeking routine primary and specialty care.
Frequency of Healthcare Visits. The majority of adults with spina bifida in this
study received routine health care by a primary care provider most often; this is not
surprising in light of managed care. They tended to see a urologist more often than a
neurosurgeon/neurologist and they saw an orthopaedic surgeon least often. For those
who had not seen a primary care provider, neurosurgeon/neurologist, orthopaedic
surgeon, or urologist within the past year, the overwhelming reason was that they did not
feel it was necessary.
The data indicated that the adult with spina bifida is falling far short of the
recommendations for adults in the Guidelines For Spina Bifida Healthcare Services
Throughout Life. Recommendations for frequency of visits to a primary care provider,
neurosurgeon, orthopaedic surgeon, and urologist are for once a year (Rauen, 1990).
When asked about other providers who routinely cared for the health care needs
of the adult with spina bifida, a trend emerged. Nine (19.6%) of the population reported
receiving care through a spina bifida clinic. For the purposes of comparative analysis,
47
these subjects were identified and grouped as spina bifida clinic patients and the
remainder of the sample population was identified and grouped as non clinic patients.
When comparing the frequency of health care visits between the two groups,
there were statistically significant differences. All (100%) of the spina bifida clinic
group saw their primary care provider annually as compared to 62.2% of the non clinic
group. All (100%) of the spina bifida clinic group reported that they saw a
neurosurgeon/neurologist and urologist annually as compared to 24.3% and 45.9% of the
non clinic group, respectively. Seven (77.8%) of the spina bifida clinic group reported
seeing an orthopaedic surgeon on an annual basis as compared to 18.9% of the non clinic
group. These data indicated that adults with spina bifida who receive health care through
a spina bifida clinic are much more likely to receive recommended health care. This
disparity in follow up was also found by Kaufman et al. (1994). They demonstrated that
66% of former clinic patients without ongoing clinic care had no neurosurgical follow-up
as compared with 22% of former clinic patients who were receiving ongoing clinic care.
Rates for orthopaedic follow up between the disbanded clinic patients without ongoing
clinic care and those with ongoing clinic care were 50% and 25%, respectively. Rates
for urological follow up between the former clinic patients without ongoing clinic care
and those with ongoing clinic care were 45% and 30%, respectively. Hayward et al.
(1991) also was able to demonstrate that a regular source of ambulatory care impacted on
the receipt of medical care: 56% of women, 20 years or older, who had a regular source
of ambulatory care reported having had a Pap smear within the past year as compared
with 46% of women who had no regular source of ambulatory care. Similarly, for
women 40 years of older, 59% who had a regular source of ambulatory care reported
48
having had a breast exam by a physician within the past year as compared with 31% who
had no regular source of ambulatory care.
Secondary Conditions. The most frequently reported secondary conditions in this
study were urinary tract infections (78.3%), pressure ulcers (63.0%), obesity (32.6%),
changes in ability to walk (32.6%), change in brace needs (26.1%), behavioral issues
(26.1%), and osteomyelitis (21.7%). Farley (1996) found that pressure sores (83.1%),
bums (59.3%), muscle atrophy (51.7%), scoliosis (51.2%), and leg fractures (43.5%)
were among the leaders in secondary conditions in his study. The incidence of urinary
tract infections in his study was significantly lower (39.5%), but these were qualified by
having a febrile component to them.
A comparative analysis between the two identified subgroups and the incidence
of specific secondary conditions yielded no statistically significant differences. The
sample size limited a relevant comparison. It appears though, that routine care through a
spina bifida clinic may influence the incidence of behavior issues: 11.1% of spina bifida
clinic patients reported a history of behavior issues compared to 29.7% of non clinic
patients. This may reflect a higher locus-of-control in the clinic patient group.
The spina bifida clinic group reported a 44.4% incidence rate of change in brace
needs as compared to 21.6% of the non clinic group. It can be inferred that clinic
patients were more apt to proactively seek orthotic care than the non clinic patients, or
that clinic patients were more apt to still be wearing orthoses. This question was found
to be lacking in content validity.
The incidence of obesity was slightly higher in the spina bifida clinic group
(44.4%) than the non clinic group (29.7%). While obesity was defined as 20 pounds
49
over the subject s ideal weight, the researcher did not take into consideration the
respondent’s knowledge of ‘ideal weight’.
The incidence of osteomyelitis was slightly higher in the non clinic group
(24.3%) than the spina bifida clinic group (11.1%). This may reflect the tendency for a
higher incidence of pressure ulcers in the non-clinic group (64.4%) as compared to the
spina bifida clinic group (55.6%) developing from pressure ulcers.
There was a higher incidence of ambulatory changes in the spina bifida clinic
group (44.4%) than the non clinic group (29.7%). This, however, may have been
influenced by the number of subjects who were ambulatory at the age of 21; this was not
asked.
The incidence of urinary tract infections was almost identical in the two groups.
This may help to explain why urologists were the second most visited health care
provider in this study.
The total reported incidence of secondary conditions, excluding brace changes, in
the spina bifida clinic group was 100% and 94.6% in the non clinic group. Total
incidence of secondary conditions was reported as a sum total of either a positive or
negative history for all the specific secondary conditions listed and reported and not
quantified per number of occurrences. It would have been beneficial, in providing a
more accurate indication of the relationship between frequency of healthcare and
incidence of secondary conditions, to quantify number of occurrences of secondary in the
two subgroups.
Surgical Interventions. Fifty-one surgical interventions were reported; some
subjects had more than one intervention. For descriptive purposes, the surgical
50
interventions were procedurally classified as either orthopaedic, urological, neurological,
or other. Orthopaedic interventions were most frequent (54.9%) and urological
interventions occurred second most frequently (23.5%). What is interesting to note is
that while orthopaedic interventions ranked highest in frequency, visits to orthopaedic
surgeons ranked lowest in frequency.
Comparisons were made between the two subgroups and the incidence of
different surgical interventions. The incidence of orthopaedic procedures was higher in
the non clinic group than the spina bifida clinic group, 57.1% and 44.4% respectively.
The incidence of urological interventions was also higher in the non clinic group (26.2%)
as compared to clinic group (11.1%). Perhaps, due to a limited sample size, the results
were not statistically significant.
In reviewing the types of surgical interventions in each classification, it became
evident that certain procedures may be influenced by the frequency of routine health
care. A board certified orthopaedic surgeon, working in a pediatric orthopaedic facility,
was asked to rank each of the surgical interventions as either proactive, reactive, or
unrelated. His rankings were based on data obtained in the survey (Appendix E).
Definitions for these rankings appear in Chapter III.
A comparison was made between the two subgroups and the incidence of
proactive, reactive, and unrelated interventions. The sample size was limited and the
results were not statistically significant. A point of interest, however, was that there was
a 76.2% rate of reactive surgeries in the non clinic group as compared with a 55.6% rate
in the spina bifida clinic group. This suggests that frequency of routine care may impact
on the incidence of reactive surgical interventions. This relationship was demonstrated
51
by Kaufman et al. (1994) who found that 9 of 39 (23.1%) former clinic patients without
on-going clinic care experienced surgical decubitus repair as compared to 1 of 100 (1%)
former clinic patients who were receiving on-going clinic care.
Satisfaction of Spina Bifida Care. Despite a small sample size, which impacted
on statistical significance, a definite trend emerged in how satisfied the respondents were
with the ability of the primary care provider to care for the adult with spina bifida. None
of the spina bifida clinic group ranked their primary care provider less than adequate as
compared to 37.8% of the non-clinic group. One can infer that satisfaction increases
with frequency of care.
When considered in light of Dorothea Orem’s theory of self-care (1995), the
results of this study indicated that adults with spina bifida were not aware of the self-care
requisites entailed in managing their health. It appeared that attendance at a spina bifida
clinic provided a greater opportunity for individuals to meet their therapeutic self-care
demands. In the absence of a spina bifida clinic, it was evident that the primary care
provider was most frequently called upon to deliver care in an effort to promote health.
According to Orem (1995), the primary care provider should be responsible for
providing health care in an effort to achieve the adjustments and adaptations necessary
for continued support of life and integrated functioning. The primary care provider
needs to incorporate a continuous assessment of health in light of the adult’s spina bifida
and incorporate recommended specialty care in an effort to prevent secondary conditions.
Conclusions
This study indicated that adults with spina bifida are not receiving healthcare as
frequently as recommended. However, it was demonstrated that adults who attended a
52
spina bifida clinic received health care more frequently and come closer to meeting these
recommendations. Due to a limited sample size, this study was not able to significantly
demonstrate a relationship between frequency of health care and the incidence of
secondary conditions. In addition to this, the limitations described in Chapter I effected
the conclusions that could be drawn: the sample for study was obtained from a listing of
patients who had been served at one pediatric orthopaedic facility in northwestern
Pennsylvania, different levels of morbidity may be related to the various levels of spinal
defect rather than the level of care the patient is received, and not all adults with spina
bifida were able to verbally respond to questioning and therefore their caretaker may
have served as respondent for this survey.
Recommendations
This study indicated that adults with spina bifida are more likely to receive
recommended health care through a spina bifida clinic. Further research is necessary to
demonstrate that the recommended frequency of health care results in a lower incidence
of secondary conditions. Some recommendations for further research are:
1. Repeat this study with a larger sample size.
2. Repeat this study but take a prospective approach versus subjects self reports.
3. Repeat this study with a population of adults with spina bifida who receive
health care through a spina bifida clinic.
4. Study how the general aging process affect the incidence of secondary
conditions.
5. Study primary care providers regarding their perceptions of appropriate care
for adults with spina bifida.
53
6. Study primary care providers to determine their knowledge level regarding
secondary conditions in adults with spina bifida.
7. Study adults with spina bifida to determine their knowledge level regarding
secondary conditions of spina bifida.
8. Study how access to care issues may impact on adults with'spina bifida
seeking healthcare.
If the first three recommended studies indicated that frequency of healthcare was
inversely related to the incidence of secondary conditions, despite the general aging
process, then the fourth, fifth, and sixth recommended studies could be performed to
determine if knowledge deficit in the primary care provider or adult with spina bifida
served as a barrier. If this were the case, then education of primary care providers in the
health care needs of their adult patients with spina bifida would be indicated as well as
education of the adult with spina bifida. If, as determined by the seventh study, that
access to care affected the frequency of care sought by the adult with spina bifida, then
advocating for change at the governmental level would be indicated.
Summary
This chapter provided a summary of this research project. Data indicated that
adults with spina bifida do not receive the recommended healthcare at the recommended
frequency. It demonstrated, however, that adults in a spina bifida clinic have a higher
incidence of receiving annual health care at the recommended frequency. Due to a
limited sample size, this study did not support the hypothesis that there is an inverse
relationship between the frequency of healthcare and the incidence of secondary
54
conditions in the adult with spina bifida. Discussions of these findings and
recommendations for further study were provided.
55
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treatment. Archives of Physical Medicine and Rehabilitation, 70, 627-641.
Blum, R. W., Garell, D., Hodgman, C. H., Jorissen, T. W., Okinow, N. A., Orr,
D. P., & Slap, G. B. (1993). Transition from child-centered to adult health-care systems
for adolescents with chronic conditions. Journal of Adolescent Health, 14, 570-576.
Bronheim, S. M. (1989). Background issue brief. In Growing up and getting
medical care: Youth with special health care needs. Surgeon General’s Conference,
March 13-15, 1989, 6-8.
Council on Child and Adolescent Health (1988). Age limits of pediatrics.
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Dorval, J. (1994). Achieving and maintaining body systems integrity and
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bifida or cerebral palsy, proceedings and recommendations of a symposium, Donald L.
Lollar, Ed.D. (Ed.), 65-77.
Farley, T. L. (1996). Secondary disabilities in Arkansas with spina bifida.
Unpublished data presented at Independence: The ultimate frontier. The Spina Bifida
Association of America Annual Conference held June 19-21,1996 in Phoenix, AZ.
Farrow, J. A. (1992). Introductory remarks. In Transitions in care for young
adults with special health needs, proceedings of a regional conference held on April 23
and 24, 1992 in Seattle, WA.
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Hallum, A. (1995). Disability and the transition to adulthood: Issues for the
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12-50.
Hayward, R. A., Bernard, A. M. A, Freeman, H. E., & Corey, C. R. (1991).
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Health Resources and Services Administration (1992). Moving on ... Transition
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Kaufman, B. A., Terbrock, A., Winters, N., Ito, J., Klosterman, A., & Park, T. S.
(1994). Disbanding a multidisciplinary clinic: Effects on the health care of
myelomeningocele patients. Pediatric Neurosurgery, 21, 36-43.
Koop, C. E. (1989). Surgeon General’s introductory remarks. In Growing up
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Laurence, K. M. (1964). Natural history of spina bifida cystica: Detailed
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Lollar, D. J. (1994). Encouraging personal and interpersonal independence. In
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proceedings and recommendations of a symposium, Donald L. Lollar, Ed.D. (Ed.), 1725.
57
MRC Vitamin Study Research Group (1991). Prevention of neural tube defects:
Results of the Medical Research Council vitamin study. The Lancet, 338(8760) 131137.
McLone, D. G. (1989). Spina bifida today: Problems adults face. Seminars in
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McLone, D. G., Dias, L., Kaplan, W. E„ & Sommers, M. W. (1985). Concepts in
the management of spina bifida. Concepts in Pediatric Neurosurgery. 5, 97-106.
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58
Rauen, K. (Ed.) (1990). Guidelines for spina bifida health care services
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59
Appendices
60
Appendix A
Dear Doctor,
Priority Ranking
I need to classify certain surgical procedures as part of my research project on spina
bifida. I have asked the survey participants what surgical procedures they have had since
turning the age of 21. Below is a listing of those surgical procedures; I’d like you to
classify them as:
1: proactive, i.e., those procedures that are preventative in nature and are performed
generally to prevent complications secondary to spina bifida and more likely in patients
with appropriate follow-up
2: reactive, i.e., those procedures that are generally performed as a result of a secondary
complication and more likely in patients with inappropriate follow-up
3: non-related, i.e., those procedures that would have taken place irregardless of the
patient’s myelo status and appropriate/inappropriate follow-up would have no bearing
1. Removal of bone (partial) as a result of infection/osteomyelitis
2. Removal of infected axillary nodes; result of infection/trauma from crutches
3. Debridement/closure of pressure ulcers
4. Artificial bladder sphincter
5. Release of tethered cord
6. C-section
7. Leg amputation
8. Renal lithotomy/lithotripsy
9. Renal stent insertion
10. Fractured femur pinning (?cause of fx)
11. Drainage of abdominal abscess
12. External urinary diversion/revision of diversion
13. Toe amputation
14. Hydrocele repair
15. Cystoscope
16. Foot reconstruction
17. Fatty tissue removed from bowel
18. Tom meniscus repair
19. Spinal fusion
20. Shunt Repair
ank^mi for your time,
rypmro, MSN (candidate)
Z
•2I
T
3
*2-
z
3
2
I
2-
-3_
2^
61
Appendix B
Selection from
Recommendations for the Adult Years
Guidelines For Spina Bifida Health Care Services Throughout Life
(Rauen, 1990)
Recommendations for the Adult Years
1.
Annual check-ups and episodic care by primary care providers and
provision of care coordination with specialty disciplines.
2.
Annual assessment for shunt function and presence of tethered cord,
hydomyelia, and Chiari malformation by a neurosurgeon and appropriate follow-up care.
3.
Annual evaluation with an orthopedic specialist for orthotic equipment,
mobility equipment, degenerative problems, and appropriate follow-up care.
4.
Evaluation annually by a urologist for a renal ultrasound and assessment
of urinary status with appropriate follow-up care.
62
Appendix C
Introductory Script
Hello, my name is Mary Ferraro and I am a Nursing Supervisor at Shriners
Hospital For Children in Erie, Pennsylvania. I am also a graduate student at Edinboro
University. As part of the requirements for graduation I am conducting a survey on how
adults with spina bifida are getting their healthcare needs met. I would appreciate you
taking ten minutes of time to answer the following questions. Only I will know who was
called. All information will be kept confidential. No one person will be identified. In
no way does your participation or non-participation affect your healthcare.
Are you willing to participate? If yes, let’s continue on with the questions.”
If the respondents question the meaning of the secondary conditions listed in this
tool, the following explanations will be provided:
“Pressure sores are blisters or open areas on your skin that were caused by either
pressure or rubbing of that area.”
“Urinary tract infections are when bacteria grows too much in your urine and can
cause you to either run a fever or have bad smelling urine, or both. Usually the doctor
will prescribe medicine for several days to help you get rid of them.”
Kidney failure is when your kidneys no longer make urine and you become very
ill. You would be put in the hospital if this occurred and perhaps be on dialysis.”
“Loss of a kidney is when the doctor has to surgically take out one of your
kidneys.”
63
“High blood pressure is when your blood pressure reading is over 140/80 on a
regular basis; sometimes the doctor may put you on medication to treat your high blood
pressure.”
“Obesity is being 20 pounds over what your ideal weight should be.”
“Fractures are when your bones are broken.”
“Change in brace needs means that your braces are not fitting you well or they are
in bad shape and need repaired.”
“Behavior issues are emotions or feelings that seem to prevent you from enjoying
life. They may also cause you to use drugs or alcohol.”
“Changes in ability to walk means that you can’t get around the way you used to,
for example if you used to walk with crutches most places and now you only use them
around the house and use a wheelchair most other places, you have had a change in your
ability to walk.”
“Changes in bowel function means that your bowels patterns have changed; either
you have developed diarrhea or constipation and this is not normal for you.”
u
«____
!
Shriners
Hospitals
64
Appendix D
r-1
tf
GDanncacpceDo
Erie
Hospital
July 25, 1997
1645 West 8th Street
Erie, Pennsylvania 16505
Tel. 814-875-8700
Fax 814-875-8756
Richard W. Brzuz
Administrator
James 0. Sanders, M.D.
Chief of Staff
To Whom It May Concern:
Mary Ferraro has been working with the Research Committee of Shriners
Hospitals for Children-Erie in order to develop a needs assessment tool in an
effort to query adults with myelodysplasia about their current level of functioning
and health care seeking behaviors. She has permission to access our patient data
base and select subjects from our active/inactive myelodysplasia patient list.
Karl F Frankovifch, M.D.
Emeritus Chief of Staff
Sincerely yours,
OFFICERS OF
LOCAL BOARD
OF GOVERNORS
William D. Bessor
Chairman
Robert F Thompson
Vice Chairman
James H. Richardson, Jr.
Treasurer
Richard A. Henderson
Secretary
AFFILIATED TEMPLES
Al Koran
Damascus
Ismailia
Jaffa
Mocha
Moslem
Osiris
Rameses
Syria
Tadmor
Zem Zem
Zenobia
James OfSanders,
Chief'of Staff
JOS:dct
65
Appendix E
DEMOGRAPHIC INFORMATION
Patient Number.
1. Information provided by
self iQ
caretaker 2Q
2. Sex
3. Age
4. Current Payment Method for Healthcare Services
private insurance
public insurance
self-pay
none
combination
iQ
20
30
4Q
sQ
0-30 miles
31-50 miles
51-100 miles
101 or more
iQ
2Q
3Q
40
5. Approximate Distance From Erie
6. History of Hydocephalus
yes !□
no 2Q
GENERAL HEALTHCARE INFORMATION
7. On an average, how many routine visits, not sick visits or emergencies, do you usually
make to a primary care provider (family doctor, clinic doctor, nurse practitioner, etc.) in a
year?
none 1Q
one or more 2Q
66
8. How many routine visits, not sick visits or emergencies, have you made to a primary
care provider (family doctor, clinic doctor, nurse practitioner, etc.) in the last year?
none iQJ
one or more 2
9. If you have not made a routine visit to a primary care provider in the last year, why
not?
distance
no insurance
no provider
transportation
don't think I need it
1Q
20
3Q
4Q
5Q
10. On an average, how many routine visits, not sick visits or emergencies, do you
usually make to a neurosurgeon/neurologist in a year?
none 1Q
one or more 2|^J
11. How many routine visits, not sick calls or emergencies, have you made to a
neurosurgeon/neurologist in the past year?
none 10
one or more 2Q
12. If you haven't made a routine visit to a neurosurgeon/neurologist in the past year,
why not?
distance
no insurance
no referral
no provider
transportation
don't think I need it
1Q
20
3Q
sQ
6Q
67
13. On an average, how many routine visits, not sick visits or emergencies, do you
usually make to an orthopedic surgeon in a year?
none iQ
one or more 2Q
14. How many routine visits, not sick calls or emergencies, have you made to an
orthopedic surgeon in the past year?
none iQ
one or more 20
15. If you haven't made a routine visit to an orthopedic surgeon in the past year, why
not?
distance
no insurance
no referral
no provider
transportation
don't think I need it
2Q
3 [J
4Q
sQ
60
16. On an average, how many routine visits, not sick visits or emergencies, do you
usually make to a urologist in a year?
none iQ
one or more 2Q
17. How many routine visits, not sick calls or emergencies, have you made to a urologist
in the past year?
__ _________________________________
none 1Q
one or more 2Q
68
18. If you haven't made a routine visit to a urologist in the past year, why not?
distance
no insurance
no referral
no provider
transportation
don't think I need it
1Q
20
30
40
sQ
60
19. What other providers take care of your healthcare needs on a regular basis?
20. Have you experienced any of the following conditions since turning twenty one years
old?
pressure ulcers
urinary tract infections
kidney failure
loss of a kidney Q
high blood pressure
obesity
fractures
change in brace needs
behavior issues Q
changes in ability to walk Q
changes in bowel function
Other conditions not mentioned
21. What surgical procedures have you experienced since turning 21 years of age?
22. How well does your primary care provider take care of your spina bifida?
excellent
good
adequate
poor
not at all
1Q
2Q
40
5Q
69
23. If an adult specialty clinic was (established in Erie, Pa. where an orthopedist,
urologist, neurosurgeon, and primary/ care provider could see you regularly on the same
day, would you attend?
yes iQ
no 2Q
unsure 3Q
24. Are there any questions or concerns that I can discuss with you before we hang up?
70
Appendix F
Survey Data
1.
Information provided by
self
caretaker
29 (63%)
17 (37%)
2.
Sex
male
female
30 (65.2%
16(34,8%)
3.
Age
22-44
Mean 29.87
4.
Current payment method for health care
services
private insurance
public insurance
none
combination
17 (37.0%)
21 (45.7%)
2 (4,3%)
6(13%)
5.
Approximate distance from Erie
0-30 miles
31-50 miles
51-100 miles
101 or more miles
11 (23.9%)
1 (2.2%)
9 (19.6%)
25 (54.3%)
6.
History of hydrocephalus
Yes
No
27 (58.7%)
19(41.3%)
7.
On an average, how many routine visits,
not sick visits or emergencies, do you
usually make to a primary care provider
(family doctor, clinic doctor, nurse
practitioner, etc.) in a year?
None
One or more
14 (30.4%)
32 (69.6%)
None
One or more
14 (30.4%)
32 (69.6%)
8.
How many routine visits, not sick visits
or emergencies, have you made to a
primary care provider (family doctor,
clinic doctor, nurse practitioner, etc.)
in the last year?
71
9.
10.
11.
12.
13.
14.
15.
If you have not made a routine visit to a
primary care provider in the last year,
why not?
On an average, how many routine visits,
not sick visits or emergencies, do you
usually make to a neurosurgeon/
neurologist in a year?
How many routine visits, not sick visits
or emergencies, have you made to a
neurosurgeon/neurologist in the past
year?
If you haven’t made a routine visit to
a neurosurgeon/neurologist in the past
year, why not?
On an average, how many routine visits,
not sick visits or emergencies, do you
usually make to an orthopaedic surgeon
in a year?
How many routine visits, not sick calls
or emergencies, have you made to an
orthopaedic surgeon in the past year? ..
N/A
No insurance
No provider
Unnecessary
32 (69.6%)
1 (2.2%)
2 (4.3%)
11 (23.9%)
None
One or more
28 (60.9%)
18(39.1%)
None
One or more
33 (71.7%)
13 (28.3%)
N/A
No referral
Unnecessary
13(28.3%)
3 (6.5%)
30 (65.2%)
None
One or more
32 (69.6%)
14(30.4%)
None
One or more
36 (78.3%)
10(21.7%)
N/A
No referral
No provider
Unnecessary
10(21.7%)
5 (10.9%)
1 (2.2%)
30 (65.2%
If you haven’t made a routine visit to
an orthopaedic surgeon in the past year,
why not?
72
16.
17.
18.
19.
20.
On an average, how many routine visits,
not sick visits or emergencies, do you
usually make to a urologist in a year? ..
How many routine visits, not sick calls
or emergencies, of you made to a
urologist in the past year?
If you haven’t made a routine visit to
a urologist in the past year, why not? ..
What other providers take care of your
health care needs on a regular basis? ...
Have you experienced any of the
following conditions since turning
21 years old?
None
One or more
20 (43.5%)
26 (56.5%)
None
One or more
25 (54.3%)
21 (45.7%)
N/A
No referral
Unnecessary
21 (45.7%)
3 (6.5%)
22 (47.8%)
Spina bifida clinic
Gynecologist
PT/OT/Orthotist
Nursing/assistive
personnel
Psych services
Eye doctor
Other
9(19.6%)
4 (8.7%)
3 (6.5%)
Pressure ulcers
Urinary tract
infections
Kidney failure
Loss of a kidney
High blood pressure
Obesity
Fractures
Change in brace
needs
Behavior issues
Changes in ability
to walk
Changes in bowel
function
29 (63.0%)
5 (10.9%)
2 (4.3%)
3 (6.5%)
6(13.0%)
36 (78.3%)
2 (4.3%)
1 (2.2%)
7 (15.2%)
15 (32.6%)
8(17.4%)
12(26.1%)
12(26.1%)
15 (32.6%)
8 (17.4%)
73
Other conditions not mentioned
21.
What surgical procedures have you
experienced since turning 21 years
of age?
Osteomyelitis
Scoliosis
Lower extremity
change
Bladder function
change
Upper extremity
change
Tethered cord
Other renal
Back pain
New onset
hydrocephalus
Thrombus
Other
Removal of bone 2°
to infection
Removal of infected
axillary nodes
Debridement/
closure of pressure
ulcer
Artificial bladder
sphincter
Release of tethered
cord
C-section
Leg amputation
Renal lithotomy/
lithotripsy
Renal stent
insertion
Fractured femur
pinning
Drainage of
abdominal abscess
External urinary
diversion/revision
10(21.7%)
4 (8.7%)
3 (6.5%)
1 (2.2%)
1 (2.2%)
1 (2,2%)
2 (4.3%)
3 (6.5%)
1 (2,2%)
1 (2,2%)
3 (6.5%)
2 (3.92%)
1 (1.96%)
15 (29.4%)
1 (1.96%)
2 (3.92%)
1 (1.96%)
3 (5.88%)
2 (3.92%)
1 (1.96%)
1 (1.96%)
1 (1.96%)
5 (9.8%)
74
22.
23.
24.
How well does your primary care
provider take care of your spina
bifida?
If an adult specialty clinic was
established in Erie, Pa, where an
orthopaedist, urologist, neurosurgeon,
and primary care provider could see
you regularly on the same day, would
you attend?
Are there any questions or concerns
that I can discuss with you before we
hang up?
Toe amputation
Hydrocele repair
Cystoscope
Foot reconstruction
Fatty tissue removed
bowel
Tom meniscus
repair
Spinal fusion
Shunt repair
1 (1.96%
1 (1.96%
1 (1.96%
2 (3.92%
Excellent
Good
Adequate
Poor
Not at all
15 (32.6%)
5 (10.9%)
12(26.1%)
2 (4.3%)
12(26.1%)
Yes
No
Unsure
17(37%)
19(41.3%)
10(21.7%)
1 (1.96%)
1 (1.96%)
1 (1.96%)
2 (3.92%)
Vocational/insurance
1 (2.2%)
questions
Do I have spina
2 (4.3%)
bifida?
Questions re: current
symptoms
5 (10.9%)
Questions re: general
2 (4.3%'
information
Is there a support
1 (2,2%'
group?