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 ~ >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 :•: :;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 References Alexander, M. A., & Steg, N. L. (1989). Myelomeningocele: Comprehensive 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. Pediatrics, 81(5), 736. Dorval, J. (1994). Achieving and maintaining body systems integrity and function: Clinical issues. In Preventing secondary conditions associated with spina 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. 56 Hallum, A. (1995). Disability and the transition to adulthood: Issues for the disabled child, the family, and the pediatrician. Current Problems in Pediatrics. 25(1), 12-50. Hayward, R. A., Bernard, A. M. A, Freeman, H. E., & Corey, C. R. (1991). Regular source of ambulatory care and access to health services. American Journal of Public Health, 81(4), 434-438. Health Resources and Services Administration (1992). Moving on ... Transition from child-centered to adult health care for youth with disabilities. Arlington, VA: 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 and getting medical care: Youth with special health care needs. Surgeon General’s Conference, March 13-15,1989, 3-5. Laurence, K. M. (1964). Natural history of spina bifida cystica: Detailed analysis of 407 cases. Archives of Disease in Childhood, 39,41-57. Lollar, D. J. (1994). Encouraging personal and interpersonal independence. In Preventing secondary conditions associated with spina bifida or cerebral palsy, 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 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 needs: The challenge for financing. In Growing up and getting medical care: Youth with special health care needs. Surgeon General’s Conference, March 13-15,1989, 16- 21. 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. Okinow, N. A. (1992). Advocacy and policy direction. In Transition in care for young adults with special needs, proceedings of a regional conference held on April 23 and 24, 1992 in Seattle, WA. Orem, D. E. (1995). Nursing: Concepts of practice, 4th ed. St. Louis: Mosby- 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 conditions associated with spina bifida or cerebral palsy, proceedings and recommendations of a symposium, Donald L. Lollar, Ed.D. (Ed.), 3-16. 58 Rauen, K. (Ed.) (1990). Guidelines for spina bifida health care services throughout life. Location: Spina Bifida Association of America. 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 eligibility criteria and scope of services. Rosen, D. (1995). Between two worlds: Bridging the cultures of child health and adult medicine. Journal of Adolescent Health, 17(1), 11-16. Sandler, A. (1997). Living with spina bifida: A guide for families and professionals. Raleigh, NC: University of North Carolina Press. Society for Adolescent Medicine (1995). Moving on: Transition from pediatric to adult health care, working conference held on September 9-11, 1994. Summary of 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?