Parental Reports of Health Care Practices

for Adolescents with Down Syndrome
Thesis Nurs. 1997 W323p

c.2
Wasson, Deborah A.

Deborah A. Wasson

Parental reports of
health care practices
1997.

Submitted in Partial Fulfillment of the Requirements
for the Master of Science in Nursing Degree

Approved by:

Alice Conway, RN, Pitt).
Committee Chairperson of
Edinboro University of Pennsylvania

Date

/j Judith Schilling,tRNP, Ph.D. 7
y
Committee Member of
Edinboro University of Pennsylvania

Date

fcynthia Legin-'Bucell, PhD.
Committee Member of
Edinboro University of Pennsylvania

Apn
Date
,r r

Parental Reports of Health Care Practices
for Adolescents with Down Syndrome

by
Deborah A. Wasson

Submitted in Partial Fulfillment of the Requirements
For the Master of Science in Nursing Degree

Abstract

Parental Reports of Health Care Practices for Adolescents with Down Syndrome

Literature indicates that the majority of primary care providers are not

meeting the health care needs of their adolescent patients. A survey of parents of

adolescents with Down syndrome was conducted to determine the parents’ perception
of the extent to which primary care providers are addressing the unique needs of their

adolescent patients with Down syndrome. The tool utilized was a researcherdeveloped survey that assessed the frequency of performance of multiple health care

screenings and education as recommended by Guidelines for Adolescent Preventative
Services, the American Academy of Pediatrics, and the Down Syndrome Medical

Interest Group. The sample consisted of the parents of seven female and six male

adolescents with a mean age of 15.77 years. The results from this study indicated that
primary care providers were not addressing the unique health care needs of their

adolescent patients with Down syndrome.

Acknowledgements
I would like to take this opportunity to express my sincere appreciation to all

those people who helped to make this thesis possible. I would like to thank the
members of my thesis committee: Dr. Alice Conway, Dr. Judy Schilling, and Dr.
Cynthia Legin-Bucell for their time, energy, and direction. I would like to thank Dr.

Len Leshin, MD, FAAP, for his expert advice on my survey. I would also like to

thank the members of the northwestern Pennsylvania Association of Retarded
Citizens (ARC) parent support group who helped to pilot my survey. And last, but not

least, I would like to thank my children, Samantha, Jessica, Megan, Charles, and

Cassandra, for their unending support, patience, and inspiration throughout this long
process. My deepest appreciation to each and every one of you.

iii

Table of Contents
Title

Page

Abstract

Acknowledgements

111

List of Tables

List of Figures

Chapter I - Introduction

Background of the Problem

Research Question

Theoretical Framework

Statement of the Problem

Statement of Purpose

Assumptions

Limitations

Definition of Terms

Summary

Chapter II - Review of Literature

Risks in Adolescence

Substance Use

Sexual Activity

Health-Risk Issues

Current Health Care

Title

Page
11

Down syndrome

Routine Health Care

Cardiac

Respiratory

Growth

Endocrine

Atlantoaxial Instability

Depression

Hearing

Eye/Vision

Dental

Reproductive

Primary Care

Summary

22
26

Chapter III - Methodology
Research Design

Instrumentation

Pilot Study

Sample, Setting, and Procedure

Protection of Human Rights

Data Analysis

Title

Page

Summary

Chapter IV - Results

Sample

Demographics

Location and Frequency of Health Care

Routine Screening

Down Syndrome Complications

Adolescent Issues

Safety Issues

Sexual Issues

Social/School Issues

Additional Comments

Summary

V. Summary, Conclusions, and Recommendations

Summary of Research and Discussion

50
50

Demographics

Routine Screening

Down Syndrome Complications

Adolescent Issues

Safety Issues

Sexual Issues

Title

Page

Social/School Issues

Overall Satisfaction

Conclusions

Recommendations

Summary

References

Appendixes

A. Selection of Guidelines for Adolescent Preventive Services (GAPS)

Recommendations
B. Survey for Parents of Adolescents with Down Syndrome

C. Introduction Letter to Down Syndrome Parent Support Group

D. Introduction Letter to Down Syndrome Listserver

E. Edinboro University IRB Exempt Review

F. Survey Data

vii

List of Tables

Page

Table
1. Parents’ Ages

2. Parents Educational Levels

3. Annual Household Income

4. Ages of Adolescents with Down Syndrome

5. Mean Household Demographics

viii

List of Figures

Figure

Page

1. Routine Screening

2. Down Syndrome Complications

3. Adolescent Issues

4. Safety Issues

5. Sexual Issues

6. Social/School Issues

Chapter I

Introduction
This chapter provides a brief overview of adolescence and Down syndrome

and the need for primary care during this period. 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 definition of terms are also provided.

Background of the Problem
Adolescence is a time of rapid growth and development. This period is
difficult and often complicated by current trends in society, especially the trend
toward fewer opportunities for adolescents to have contact with supportive adults

(Millstein, Nightingale, Petersen, Mortimer, & Hamburg, 1993). These, coupled with

unhealthy behaviors such as sedentary life-style and poor nutritional habits along with

availability of harmful activities or substances—alcohol, drugs, weapons, unsafe
vehicle use, and unsafe sexual practices—place the adolescent at risk for morbidity

and mortality (Igra & Millstein, 1993).
During adolescence, the addition of a developmental disability such as Down
syndrome creates increased challenges that need to be met (Newacheck, 1989). Down
syndrome is the most common identifiable cause of mental retardation and accounts

for almost one-third of all cases (Trumble, 1993). The adolescent with Down
syndrome must deal with numerous medical conditions in addition to all the typical

problems of adolescence.

Primary care directed at risk behaviors can reduce morbidity and mortality

during adolescence (Igra & Millstein, 1993). Primary care providers are in a unique
position to direct the primary and secondary prevention services needed for today’s

adolescents (Millstein et al., 1993). However, although over 70% of adolescents are
seen by a physician yearly, their unique health care needs are not always being
addressed (Igra & Millstein, 1993).

Research Question
Review of the literature suggests that the health care needs of adolescents are

not being fully met by primary care providers. This study seeks to determine the
extent to which primary care providers are addressing the health care needs of

adolescents with Down syndrome as perceived by the adolescents’ parents.
Theoretical Framework

Dorothea Orem has outlined her self-care deficit theory of nursing in Nursing:

Concepts of Practice (1995). Orem describes her theory as based on the belief that
adult persons have developed the capabilities necessary to meet their own needs (self-

care) and the needs of their dependents (dependent-care) for functioning and
development. The ability to engage in self-care and dependent-care is termed self-

care agency and dependent-care agency. The total requirement for regulation of
functioning and development is termed therapeutic self-care demand. It is the
difference between the therapeutic self-care demand and the self-care or dependent
care agency that concerns the primary care provider. This is the area in which the

patient requires assistance to fulfill his/her needs for support and promotion of normal

functioning, maintenance of normal growth and development, prevention or cure of

3
potentially disabling processes, prevention or compensation for resulting disability,

and promotion of well-being. The primary care provider assists the person in meeting
his/her self-care requisites in one or more of several ways:

1. Acting for or doing for another.

2. Guiding and directing.
3. Providing physical or psychological support.

4. Providing and maintaining an environment that supports development.

5. Teaching.
For the adolescent with Down syndrome, the primary care provider is required

to adjust and adapt the health care orientation (Orem, 1995). In this case, the goal of
health care is to create an environment that will "support life, facilitate integrated
functioning, and contribute to present and future normalcy in daily living" (Orem,

1995, p. 139). The primary care provider needs to address several health care

requirements:
1. Continuous health care 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 genetic defect on

general health, growth and development, and functioning.

3. Continuous diagnosis to determine the effects of the genetic defect on medical
conditions.
4. Specific protection against complications or extension of present impairments into

more disabling limitations.

5. Health maintenance and promotion as well as specific protection from actual or
possible effects of the genetic defect on general health, growth and development, and

functioning.
6. Assistance to the patient and family in assuming appropriate roles in continuing
health care, including self-care.

7. Assisting the patient in meeting universal and developmental components of self-

care adjusted to this stage of development.
Statement of the Problem

Adolescent patients' unique health care needs are not being met by health care
providers (Igra & Millstein, 1993). This stage of life is a trying time for the

adolescent and caregiver under the best of circumstances. Adding a developmental
disability creates increased challenges that need to be met. The adolescent with Down
syndrome must deal with all the typical problems of adolescence, work to establish a
personal identity, find some private space, and pursue his/her own interests (Trumble,

1993). The role of the primary care provider should be to assist the adolescent and

caregiver in meeting the universal and developmental components of self-care, while
at the same time considering the potential or actual health deviations of the individual

(Orem, 1995). Unfortunately, review of literature suggests the majority of primary
care providers are not providing this type of holistic care for their adolescent patients
at this time (Igra & Millstein, 1993).
Statement of Purpose

The purpose of this research study was to determine the extent to which

primary care providers were addressing the unique needs of their adolescent patients
with Down syndrome. This was determined through a survey of parents of
adolescents with Down syndrome.

Assumptions
The assumptions of this study were:

1 Adolescents with Down syndrome have unique care needs due to both their stage

of life and their medical diagnosis.
2. Parents of adolescents with Down syndrome are aware of the health care needs of
their children.

3. Parents are aware of health care provided by primary care providers for their

adolescents with Down syndrome.
Limitations
The limitations of this study were:

1. Convenience sampling method was utilized which may effect the generalizability
of this study.
2. Sample for study was obtained from an Internet Listserver and a single Down

syndrome parent support group and may effect the generalizability of this study.
3. No previous study was found on the extent of health care provided to adolescents
with Down syndrome.

Definition of Terms
The terms utilized in this study were:
1. Adolescence is the period of life between 11 and 21 years of age (Sifuentes, 1996).

2. Down syndrome is a genetic disorder caused by trisomy 21 and characterized by

mild-to-severe mental retardation (Thomas, 1989).
3 Primary care is the comprehensive health care that patients receive from the same

health care provider over a longitudinal period of time (Noble, 1996).
4. Primary care provider is a health care professional (physician, nurse practitioner, or
physician assistant) providing primary care to patients.

Summary

Adolescents have unique developmental needs that need to be addressed by

the primary care provider in order to decrease morbidity and mortality in this
population. Currently, many primary care providers are not meeting the needs of their

adolescent patients (Igra & Millstein, 1993).
Down syndrome is a common congenital condition that results in varying
degrees of mental retardation and increased risk for various medical conditions. The

primary care provider needs to adjust care to account for the degree of retardation of

the individual patient, as well as for any concurrent medical conditions.
Dorothea Orem's self-care deficit theory of nursing was utilized as the

framework for this study. Orem states that the primary care provider should adjust
and adapt the health care orientation to meet the needs of the adolescent with Down
syndrome. The primary care provider also needs to adjust the universal and
developmental components of self-care.

It was the purpose of this study to determine the extent to which primary care

providers were addressing the unique needs of their adolescent patients with Down

7
syndrome, as perceived by the adolescents1 parents. This information was determined
through a survey of parents of adolescents with Down syndrome. Assumptions,

limitations, and terms utilized in this study have been provided.

8
Chapter II
Review of Literature

This chapter provides a review of current literature on adolescence and Down

syndrome. The purpose of this chapter is to provide the reader with a selective
overview of the population to be studied and some of their specific health care issues.

Risks in Adolescence
Adolescence is a stage of life between the ages of 11 and 21 and is
characterized by rapid growth and development (Sifuentes, 1996). The individual

begins this period as a child and ends it as an adult. According to Igra and Millstein

(1993), adolescence is a high-risk period. Studies by Richert, Jay, and Gottlieb (1990)

and Key, Marsh, and Darden (1995) have found that adolescents represent a
medically underserved portion of the population, and over the last 30 years are the

only group who have not experienced improvement in their overall health.
Adolescents today face many risks including substance abuse, early sexual activity,
sexually transmitted diseases (STDs), early parenthood, injuries, homicide,

depression, and suicide (Key et al., 1995). These factors lead to increased morbidity

and mortality in this population. It is the purpose of this section to explore some of
these factors.

Substance Use. Richert et al. (1990) found that the susceptibility to substance

use and abuse occurs more frequently in those adolescents who demonstrate low self-

esteem, feelings of alienation, high need for social approval and immediate
gratification, family distress, and inadequate coping and communication skills. They

further found that alcohol is the most frequently used substance, with many
adolescents reporting serious pressures to drink by age 15 years.

Sexual Activity. Adolescent sexual activity is considered a social morbidity of

the 1990s and, to reduce the impact of early sexual activity, this issue needs to be
brought up in early adolescence and discussed frequently after that (Spencer, Fife, &
Rabinovich, 1995). Richert et al. (1990) found that over 12 million adolescents are

currently sexually active with the average age of first coitus being 16 years. This
sexual activity may lead to STDs or pregnancy. According to Richert et al.,

adolescents account for almost one-third of all abortions performed. They also stated
that sexually transmitted chlamydia infection affects 3 to 4 million individuals

annually and is most common in the 15 to 19 year old population. The
recommendation from Richert et al. was for adolescents to understand that the only
absolute preventive method for pregnancy or STDs is sexual abstinence. Spencer et

al. (1995) found that it is unlikely that once an adolescent is sexually active he/she
will stop having sex. However, they believe the primary care provider can provide

discrete guidance from a respected authority figure to encourage responsible sexual
behavior. Richert et al. noted that if the adolescent will not abstain, he/she should be

provided with preventive methods of protection.
Health-Risk Issues. According to Richert et al. (1990), the primary care
provider should assess the risk status of the adolescent in a nonjudgmental fashion

and encourage the adolescent in health promotion. Key et al. (1995) wrote that health
assessment and screening for adolescents should include addressing health-risk issues

such as depression, suicide, homicide, and injuries.

Richert et al. (1990) encouraged the primary care provider to help adolescent
patients avoid risk-taking behaviors. To accomplish this, Richert et al. recommended

avoiding fear tactics that may inadvertently challenge the adolescent to try the
behavior. They felt a more effective strategy is to build self-esteem, which helps to

minimize peer influence.
Current Health Care. Adolescents represent a medically under-served portion

of the population and over the last 30 years are the only group who have not

experienced improvement in their overall health (Richert et al., 1990; Key et al.,
1995). According to Igra and Millstein (1993), much of adolescent morbidity and

mortality can be attributed to preventable risk factors including sedentary life-style,
poor nutritional habits, substance abuse, early sexual activity, STDs, early

parenthood, injuries, homicide, depression, and suicide.
According to Wasson et al. (1995), early detection and anticipatory guidance

is critical to reduce adolescent morbidity and mortality. Igra and Millstein (1993)

wrote that while over 70% of adolescents are seen by a physician yearly, their unique

health care needs were not being addressed. They found that two-thirds of visits by
adolescents to physicians included no counseling advice of any type. They

recommended this be corrected because adolescents view health care providers as
credible and important sources of health education and failure to provide health

education could be interpreted by the adolescent as silent approval for potentially
health-damaging behaviors.

To counteract adolescent morbidity and mortality, Elster and Kuznets (1994)

recommended a greater number of services directed to the primary and secondary
prevention of major health threats facing today's adolescents. They developed

Guidelines for Adolescent Preventive Services (GAPS) designed to provide a
framework for health assessment and screening for adolescents.

Igra and Millstein (1993) have found the use of reminder systems for primary

care providers, such as age-specific preventive services checklists attached to patient
charts, to be an effective means to facilitate adherence to prevention guidelines.
GAPS is one such reminder system and has been developed to direct primary care

providers in how to provide preventive services to their adolescent patients (Elster &
Kuznets, 1994). Through GAPS, Elster and Kuznets recommended adolescents have

yearly contact with their primary care provider. This allows the opportunity to

reinforce health promotion, identify early stages of health risk behaviors, provide

immunizations, and develop a good working relationship with the adolescent.
Although the GAPS recommendation was for annual visits, unless a problem is
detected complete physical examinations are only recommended once during each
stage of adolescence or three times during this period. Several of the GAPS

recommendations are provided in Appendix A.

Down syndrome
Down syndrome is a congenital condition marked by a collection of

commonly recognized characteristics and was first described in 1865 by Dr. John
Langdon Down (Cooley & Graham, 1991). Down syndrome is found in all cultures,

12
ethnic groups, socioeconomic levels, and geographic regions. It occurs when there is
extra chromosome 21 material, and despite prenatal testing the incidence of Down
syndrome has not decreased significantly (Trumble, 1993). According to Magill

(1989), although Down syndrome is associated with some degree of mental
retardation, it is now accepted that infants and children with Down syndrome follow

the normal stages of development but at a slower pace.
Several recent developments have improved the prospects for a healthy,
productive life for individuals bom with Down syndrome (Cooley & Graham, 1991).

First and foremost, almost all babies bom with Down syndrome today are being
raised within a family. As recently as the 1970s, institutional placement at birth was

commonly recommended. Cooley and Graham noted that access to quality health care
has also improved dramatically with the federal Child Abuse Amendments of 1984
having a major impact. These amendments require that disabled infants under all
circumstances must be provided with nutrition, hydration, medication, and any
medically indicated treatment. Thus health care cannot be withheld simply because

the individual has Down syndrome. The improved medical care received has led to an
increased life expectancy for the estimated 7,000 to 12,000 individuals with Down
syndrome bom in the United States each year (Caputo, Wagner, Reynolds, Guo, &

Goel, 1989). In 1929 according to Magill (1989), the life expectancy of an infant with

Down syndrome was 9 years; by 1947, it had increased to 15 years. A recent study by
Baird and Saadovnick (1987) found that more than 70% of individuals bom with

Down syndrome survive to age 30 years.

Individuals with Down syndrome have increased risk for numerous
conditions, which can have an adverse impact on their health and development
(Cooley & Graham, 1991). Most of these conditions can be treated and complications

minimized if diagnosed early. The American Academy of Pediatrics Committee on
Genetics (1994) recommended these conditions be addressed when providing health

care to this population. They advise that primary care providers must be aware of the
Down syndrome associated conditions so as to be aggressive in their identification

and treatment.

Routine Health Care. Several individuals and groups have developed
guidelines for caring for individuals with Down syndrome (Cooley & Graham, 1991;

Trumble, 1993; American Academy of Pediatrics Committee on Genetics, 1994;
Cohen, 1996). These guidelines aim to assist primary care practitioners in providing

quality medical care to individuals with Down syndrome and are designed to cover

health care from birth to death.

It is generally accepted that an individual with Down syndrome requires the
same preventive health care and screenings as any other individual (Cooley &
Graham, 1991; Cohen, 1996). In addition, Cooley and Graham (1991) feel the

primary care provider must assist the family and individual with Down syndrome to

deal with issues of aging, medical problems associated with Down syndrome,
problems of reproductive health, living arrangements, employment, guardianship or

estate-planning, as well as routine health screening and promotion. According to the
American Academy of Pediatrics Committee on Genetics (1994), in addition to

assessing and treating the medical conditions associated with Down syndrome, the
primary care provider needs to provide anticipatory guidance for the adolescent and

caregiver for:

1. Issues related to transition into adulthood.
2. Appropriateness of school placement with emphasis on adequate vocational

training.

3. Sexuality and socialization, including the need for and degree of supervision, as
well as the need for contraception.

4. Group homes, workshop settings, and other community-supported employment.

5. Intrafamilial relationships, financial planning, and guardianship.
6. Transfer to adult medical care, if appropriate or desired.

Cardiac. Congenital heart disease affects 50% of children with Down

syndrome (American Academy of Pediatrics Committee on Genetics, 1994). Recent
studies have sought to determine cardiac anomalies among older individuals with

Down syndrome. Goldhaber, Brown, and Sutton (1987) utilized Doppler

echocardiography to study asymptomatic non-institutionalized adults with Down
syndrome who had an average age of 26 years. Among this population, 57% were

found to have mitral valve prolapse and 11% were found to have aortic regurgitation.
This study concluded that mitral valve prolapse and aortic regurgitation seem to occur
in adulthood rather than childhood.

Another study by Geggel, O’Brien, and Feingold (1993) looked at 35 patients
with Down syndrome, aged 12 years or older, who had no congenital heart disease

15
based on previous physical examinations. For patients aged 12 to 18, they found no

incidence of aortic regurgitation. They did, however, find that 58% had mitral valve

prolapse. The Down Syndrome Medical Interest Group recommends assessment for
mitral valve prolapse and aortic regurgitation with an echocardiogram for individuals

without congenital heart disease once at 18 years of age (Cohen, 1996).

Respiratory. Cooley and Graham (1991) noted that children with Down
syndrome have an increased susceptibility to respiratory tract infections, acute and

chronic airway obstructions, sleep apnea, and cor pulmonale. According to Marcus,
Keens, Bautista, von Pechmann, and Ward (1991), obstructive sleep apnea syndrome

(OSAS) consists of complete and partial sleep apnea, hypoventilation, and arterial

oxygen desaturation, and is a common manifestation in children with Down
syndrome. Their study of children with Down syndrome found that when assessed

overnight, 63% had obstructive sleep apnea, 81% had hypoventilation, and 56% had
arterial oxygen desaturation. This compared with only 13% of control subjects having

obstructive sleep apnea and none having hypoventilation or arterial oxygen
desaturation when assessed overnight. The Down Syndrome Medical Interest Group
recommends review of respiratory system by history and physical exam at each
yearly exam (Cohen, 1996). Snoring, unusual sleeping positions, fatigability during

the day, reappearance of napping in an older child, or behavior change could indicate
an obstructive airway problem and require further investigation.

Growth. Cooley and Graham (1991) reported that individuals with Down
syndrome are consistently shorter than their peers at any age are and are expected to

16
follow a modified growth curve. Therefore, the Down Syndrome Medical Interest
Group recommends primary care providers utilize specially constructed growth charts

for their patients with Down syndrome (Cohen, 1996). Cronk et al. (1988) found

evidence suggesting that the growth rate is less severely reduced during the 3 to 10
year age range than in infancy and adolescence. They also found that the adolescent

growth spurt occurs in children with Down syndrome, but may be less than that of
other children. Cronk et al. (1988) found that children with Down syndrome also have

a tendency to be overweight beginning in early childhood. They have constructed
growth charts for children with Down syndrome from age 1 month to 18 years. These
charts are based on 4,650 observations of 730 children and provide a clearer analysis
of the growth pattern of the child with Down syndrome. They recommend these

charts to be used in conjunction with those from the National Center for Health
Statistics to provide better assessment of the child’s growth pattern.
Endocrine. Hypothyroidism is more common in children with Down

syndrome with a prevalence of about 15% (Cooley & Graham, 1991). According to
Trumble (1993), most cases of hypothyroidism in Down syndrome develop during
adolescence and may be difficult to screen for because many of the signs and
symptoms—dry skin, constipation, developmental delay, and hypotonia-are similar to

the inherent features of Down syndrome. Therefore, it is recommended to obtain
annual T3, T4, and TSH levels (Cohen, 1996; Cooley & Graham, 1991).
Atlantoaxial Instability. According to Cooley and Graham (1991), individuals

with Down syndrome generally have low muscle tone and ligamentous laxity, which

increases the risk of joint subluxations and dislocations. The most serious
manifestation of ligamentous laxity involves the cervical spine and is termed
atlantoaxial instability. Atlantoaxial instability is the increased mobility of the
articulation of the first and second cervical vertebrae (American Academy of

Pediatrics Committee on Sports Medicine and Fitness, 1995). It affects approximately
15% of adolescents with Down syndrome and is generally asymptomatic. The
instability is generally due to excess laxity of the transverse ligaments that hold the

odontoid process close to the anterior arch of the atlas (Pueschel, Scola, & Pezzullo,
1992).
Instability has been noted to vary over time with either an increased or
decreased laxity, so Pueschel, Scola, and Pezzullo (1992) recommend repeat
radiographs every 5 years. The Down Syndrome Medical Interest Group recommends

periodic reevaluation at 12 years, 18 years and once in adulthood for those individuals
with normal findings (Cohen, 1996). They recommend that children with borderline

or abnormal findings should be reevaluated after one year with further evaluation for

any significant neurological changes.

Depression. Individuals with Down syndrome frequently suffer from
depression, but it often goes undiagnosed due to the difficulty in assessing individuals
with mental retardation (Cohen, 1996). According to the Down Syndrome Medical

Interest Group, typical signs of depression in individuals with Down syndrome are
skill and memory losses, significant activity slow downs, hallucinatory-like self talk,

and more extreme withdrawal (Cohen, 1996).

18
Hearing. A study of patients with Down syndrome by Balkany, Downs, Jafek,

and Krajicek (1979) found 78% of patients examined had significant hearing loss. Of
these, 83 /o were conductive, usually related to middle ear effusions, and 17% were
sensorineural. Of all subjects tested, 64% had binaural hearing loss. According to the

American Academy of Pediatrics Committee on Genetics (1994), hearing loss may
begin to develop during the second decade in individuals with Down syndrome;

therefore, they recommend an annual audiology evaluation.
Eye/Vision. Individuals with Down syndrome are noted to have numerous eye

disorders at a higher rate than the general population. Caputo et al. (1989) conducted

a review of the ocular features of Down syndrome in a non-institutionalized
population of patients referred to an ophthalmic clinic. They found refractive

problems to be common with astigmatism in 22% of the patients, myopia in 22%, and
hyperopia in 21% of the patients. Fifty-seven percent of the patients had strabismus,

and 29% had nystagmus. Other findings included excessive tearing in 15%, spoke
wheel optic nerve in 12%, cataracts in 11%, and glaucoma in 5%. Roizen, Mets and
Biondis (1994) looked at a general population of individuals with Down syndrome.

They also found increased incidence of optic disorders, but often at a lower level.
Roizen et al. found strabismus in 27% of the patients, myopia in 22%, hyperopia in

13%, nystagmus in 20%, cataracts in 5%, and glaucoma in 1% of the patients.
Because the development of significant visual loss from these causes can be avoided
if treated early, early evaluation for ophthalmic disorders and annual or biennial
follow-ups are recommended (Roizen et al., 1994; American Academy of Pediatrics

19
Committee on Genetics, 1994; Cohen, 1996).
Dental Children with Down syndrome are predisposed to dental abnormalities
(Cooley & Graham, 1991). Cooley and Graham (1991) noted an increased frequency
of delays and alterations in the sequence of tooth eruption, and missing teeth

occurring in about 50% of individuals. Barnett, Press, Friedman, and Sonnenberg
(1986) found that patients with Down syndrome have an increased susceptibility and
earlier onset of periodontal diseases which affects the supporting structures of the
teeth. Their study found that 92% of Down syndrome subjects over the age of 16 had

some bone loss. The bone loss was first seen at about age 16. Barnett et al. also noted

a lower overall prevalence of dental caries in patients with Down syndrome when
compared to a control group. The Down Syndrome Medical Interest Group

recommends twice yearly dental examinations (Cohen, 1996).
Reproductive. Sexual maturation and development in adolescents with Down

syndrome follows the same pattern as their peers (Cooley & Graham, 1991). Williams
(1983) found that the onset of puberty, with its potential for sexual activity and

reproduction, can be especially difficult for parents of the child with Down syndrome.

Mims (1996) recommended that the primary care provider, who is less emotionally
involved with the adolescent than the parents, provide support and guidance in this

area. Williams recommended all sex education be individualized to fit the child’s
developmental level, physical maturation, and level of social skills.

Although males with Down syndrome usually have normal secondary sexual
characteristic development, the penis and scrotum may be small and the testes may be

histologically abnormal with markedly decreased spermatogenesis (Cooley &
Graham, 1991; Sheridan et al., 1989). Since attainment of a full erection is often

difficult and ejaculation is not always possible, many males with Down syndrome are

infertile (Trumble, 1993). However, Sheridan et al. reported one documented case of

a child being fathered by a man with Down syndrome. The father was a 29-year-old
man with trisomy 21. Paternity was confirmed through DNA analysis from chorionic

villi sampling of the fetus and blood sampling of the parents. Therefore, fertility must
be assumed in all males with Down syndrome unless proven otherwise.
Females with Down syndrome experience menarche and menses very much
like their peers (Cooley & Graham, 1991). Evaluation of women with Down

syndrome have found that ovulation occurs in 40-70% of menstrual cycles (Cooley &
Graham, 1991) and fertility should be presumed (Trumble, 1993). Pregnancy has

been documented in females with Down syndrome in at least 30 cases (Cooley &

Graham, 1991). According to Bovicelli, Orsini, Rizzo, Montacuti, and Bacchetta
(1982) the progress of the pregnancy is generally uneventful and maternal
complications are rare. However, they note labor and delivery are generally difficult

due to malpresentation, abnormalities in the shape and size of the pelvis, and lack of
cooperation. Obstetric operations are often required, and of 21 reported deliveries, 7
were cesarean sections, 2 were breech extractions, and 1 was a forceps delivery.

Bovicelli et al. also reported that the infants are at increased risk for mental

retardation. Of the known 30 cases of Down syndrome pregnancies, 11 (35%)
resulted in a normal child, 10 (35%) resulted in a child with Down syndrome, 6

(20%) resulted in a child with a malformation and/or mental retardation, and 3 (10%)
resulted in abortion or stillbirth. The high incidence of mental retardation may be

explained by either the child developing in a genetically unbalanced maternal
environment or the transmission of abnormal genotypes. Of the 30 known cases of
pregnancies to females with Down syndrome, 15 cases have unknown paternity. In 7

cases the father was closely related to the mother, and in the remaining 8 cases, the

father was also mentally retarded.
Primary Care

Little information is available on utilization of primary care services by

adolescents with disabilities. An analysis of data from the 1984 National Health
Interview Survey found that disabled adolescents utilize health care services three
times as much as their non-disabled peers (Newacheck, 1989). While their disability

leads to increased contact with health care providers, little information is available on

the quality or content of care provided (Newacheck, 1989; Hedberg, Byrd, Klein,

Auinger, & Weitzman, 1996).

Lack of health insurance is associated with reduced use of health care services
(Newacheck, 1989). Hedberg et al. (1996) reported an attempt to counteract poor

access to primary care due to lack of health insurance by the introduction of
community health centers. They noted that the availability of high-quality and
comprehensive health care in the community increases availability and utilization of
primary health care by adolescents.

Chicoine, McGuire, Hebein, and Gilly (1995) believed that the health care of

adolescents with Down syndrome is also better served by community-oriented
primary care centers specifically designed to meet their needs. They described one
such center, the Lutheran General Adult Down Syndrome Clinic in Chicago, opened
in January of 1992. The center was designed to provide comprehensive medical care

with an emphasis on prevention to adults and adolescents with Down syndrome aged
11 and older. The center provided complete primary care with specialty referrals as
required. Chicoine et al. reported that the impact of the center has been positive in
improving the quality of life for the patients seen. The greatest improvement was
shown in analysis of 48 patients who presented with diminishing intellectual,

functional, and/or social capabilities. Alzheimer's disease, which has a high incidence
in the Down syndrome population, was suspected as the cause. Further examination

by primary care providers trained to care for individuals with Down syndrome

indicated that 46 of the 48 patients had a reversible disease such as depression or
hypothyroidism. Appropriate treatment resulted in significant improvement of

symptoms and quality of life for the patients and families or caregivers.
Summary

This chapter has provided a review of the literature for adolescence and Down
syndrome. Adolescence is a period with several factors leading to morbidity and
mortality. Adolescents currently are medically underserved and, over the last 30
years, are the only group who have not experienced improvement in their overall

health. Early detection and anticipatory guidance by primary care providers is critical
to reduce adolescent morbidity and mortality.

Several areas constitute high risk for morbidity and mortality during
adolescence. Sexual activity is a major area of concern. Issues about sexuality need to
be brought up in early adolescence and discussed frequently after that. Substance use

and abuse is another high-risk area. Alcohol is currently the most frequently used

substance by adolescents.
The use of reminder systems has been found to be an effective way to

facilitate adherence to prevention guidelines. GAPS has been developed to direct
primary care providers in how to provide preventive services to their adolescent

patients and makes several recommendations (Elster & Kuznets, 1994). These
recommendations include yearly contact between adolescents and their primary care

provider.

Down syndrome is a congenital condition caused by extra chromosome 21
material and marked by a collection of commonly recognized physical features and

numerous medical conditions. Prenatal testing to detect Down syndrome is increasing
but has not reduced the birth incidence. Down syndrome is always associated with

some degree of mental retardation but it is now accepted that infants and children
with Down syndrome follow the normal stages of development, but at a slower pace.
Several recent developments such as family living arrangements and
improved health care have brightened the prospects for a healthy, productive life for

individuals bom with Down syndrome. Several groups have developed medical care

guidelines for individuals with Down syndrome. Those with Down syndrome require

the same preventive health care and screenings as any other individual. In addition,

24
the primary care provider must address the numerous medical conditions that affect
individuals with Down syndrome. Congenital heart disease affects 50% of children

with Down syndrome. There is also evidence of mitral valve prolapse and aortic
regurgitation developing in adolescence and adulthood. Children with Down

syndrome have an increased susceptibility to respiratory tract infections, acute and

chronic airway obstructions, sleep apnea, and cor pulmonale. Individuals with Down
syndrome are consistently shorter and heavier than their peers and are expected to

follow a modified growth curve. There are specially constructed growth charts for
children with Down syndrome, which should be utilized to monitor the patient’s
growth. Acquired hypothyroidism is more common in children with Down syndrome

with most cases developing during adolescence. Individuals with Down syndrome
generally have low muscle tone and ligamentous laxity that increases the risk of joint
subluxations and dislocations; the most serious of which involves the cervical spine

and is termed atlantoaxial instability. Individuals with Down syndrome often suffer

from depression, have a high prevalence of hearing loss, numerous eye disorders, and
dental abnormalities.
Sexual maturation and development in adolescents with Down syndrome
follow the same pattern as their peers and sexuality issues must be addressed in this
population. Sex education needs to be individualized to fit the child's developmental

level, physical maturation, and level of social skills.

Primary care is designed to help reduce the impact of the major health threats
facing today's adolescents. Adolescents with Down syndrome require the same care

in addition to health care directed towards their various medical problems. Little
information is available concerning primary care of adolescents with disabilities. The

information that is available indicates health care services are being utilized, but does
not indicate the quality or content of services provided.

Chapter III
Methodology

This chapter describes the methodology utilized to determine the extent to
which primary care providers are providing the recommended health care for their

adolescent patients with Down syndrome. Included in this chapter are the research
design, sample and setting, and procedure utilized for this study.

Research Design

This study utilized a non-experimental survey research design. The goal of
this study was to gather information regarding the health care provided to adolescents
with Down syndrome as reported by their parents.
Instrumentation

A survey was utilized as the research tool (see 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 contained a letter from the researcher describing

the purpose of the study and instructions on completing the survey (see Appendix C
and Appendix D). The second section elicited demographic information concerning
the respondent’s relationship to the adolescent with Down syndrome, number of
parents living in the household, mother's and father s ages, mother s and father s
education levels, household income range, and state or country of residence as well as

the child’s age and sex. Also included in this section were two questions concerning
the frequency and location of the child's routine health care visits. Question 1
concerned the location where the child received his/her primary care with the

availability of responses of:
1. Does Not Receive Health Care.

2. Community Health Clinic.
3. Group Practice.

4. Private Health Care Practitioner Practice.

5. Down Syndrome Clinic.

6. Other.

Question number 2 utilized a scale for frequency of visits, allowing responses
of:
1. No Routine Visits.

2. Visits Every Three Months.
3. Visits Every Six Months.
4. Visits Every One Year.
5. Visits Every Two Years.

The final section of the survey contained 29 questions. Of these, 25 questions
concerned health care services as recommended by Guidelines for Adolescent

Preventative Services (GAPS), the American Academy of Pediatrics, and the Down
Syndrome Medical Interest Group. The frequency these services had been performed

were determined and compared to recommended frequencies. These 25 questions
utilized a five point Likert-type scale and allowed for responses of:
1. Has Never Mentioned It.
2. Never Between The Ages Of 11 And 21 Years.

28
3. Once Between The Ages Of 11 And 21 Years.
4. More Than Once Between The Ages Of 11 and 21 Years But Less Than Once A

Year.
5. Once A Year Between The Ages Of 11 And 21 Years.
6. More Than Once A Year Between The Ages Of 11 And 21 Years.

Question 28 determined the level of satisfaction with the primary care

provider with five possible responses from Very Satisfied to Very Dissatisfied. The
final 3 questions were open-ended questions to elicit any additional information the
respondents wished to add. The survey was reviewed by a Fellow of the American

Academy of Pediatrics for content validity and a representative of the English

Department at Edinboro University for readability.
Pilot Study. The survey was piloted by four members of a northwestern

Pennsylvania Association of Retarded Citizens (ARC) parent support group. Two
questions were clarified after the pilot study. Options of Mom, Dad, Step-mom, and
Step-dad were added to elicit parents residing in the household. Question number 2
was clarified with the addition of the phrase physicals, not sick visits to define
Routine Visits. All participants in the pilot study completed the questionnaire in less

than 15 minutes.

Sample, Setting, and Procedure
The targeted convenience sample was parents of children with Down
syndrome between the ages of 11 and 21 residing in the United States who read

English. This study utilized two groups for convenience sampling. The developed

survey was placed on an Internet Listserver designed for parents of children with
Down syndrome. The survey was placed on the Internet Listserver on three separate

occasions over the course of seven days. The setting was in whatever location the
parents had access to the Internet. The second sample was obtained through a Down
syndrome parent support group located in northwestern Pennsylvania. A

representative from this group was given copies of the survey to distribute to

members of the group who met study criteria. This sample was given approximately
three weeks to complete the survey. The surveys were returned to the researcher in

self-addressed, stamped envelopes provided by the researcher. The setting for this

group was in whatever location they chose to complete the survey.
Protection of Human Rights
An introduction accompanied the survey to allow the parents to know the

purpose of the survey. The completion and return of the survey to the researcher was
deemed as informed consent. All data remained confidential and anonymous. The

researcher kept the returned surveys in a locked safe, no names were required,
identifying information on responses returned via e-mail was removed and destroyed,

and the data was reported as aggregate data. The study was reviewed by the Edinboro

University Internal Review Board (IRB) and given an exempt review prior to being

instituted (see Appendix E).

Data Analysis
The study consisted of 13 surveys returned by March 31, 1997 from the target

sample that met the research criteria. Data on the frequency each specific health care

need were addressed by the primary care provider was compared to recommended
frequencies. These data were analyzed through descriptive analysis with description

of the parents perception of the frequency primary care providers addressed
individual health care issues. This information was compared to recommended
frequencies as put forth by GAPS, the American Academy of Pediatrics, and the

Down Syndrome Medical Interest Group.

Summary
The goal of this study was to determine the degree to which primary care

providers are meeting the needs of their adolescent patients with Down syndrome.
Analysis of data obtained through survey of parents of adolescents with Down

syndrome was utilized to determine the frequency targeted health care is being
addressed by primary care providers as reported by the parents.

Chapter IV

Results
This chapter presents the results obtained from a survey of parents of
adolescents with Down syndrome (see Appendix F). The results were analyzed with

descriptive analysis and were utilized to determine the extent to which primary care
providers are providing recommended health care for their adolescent patients with
Down syndrome.

Sample

This study utilized two samples; one obtained from an Internet Listserver
designed for parents of children with Down syndrome, and the other from a Down

syndrome parent support group located in northwestern Pennsylvania. The survey
was placed on the Internet Listserver on three separate occasions: March 2, 1997,

March 6, 1997, and March 9, 1997. On March 2, 1997, this Listserver had 557
subscribers in 22 countries. Of the 557 subscribers, 458 were from the United States.

Eight surveys were returned via e-mail. Of these eight, two were unable to be
included in analysis due to not meeting the criteria for this sample. One of the surveys

not included was from the parent of a 24 year-old with Down syndrome. The other

survey not included was from a Canadian parent of a 6 year-old with Down
syndrome.

Twenty copies of the survey were delivered to a representative of a
northwestern Pennsylvania Down syndrome parent support group on March 5, 1997.

Seven completed surveys were returned and were included in the analysis. Thus a

32
total of 13 surveys were utilized in the analysis.
Demographics

Of the 13 returned surveys, 11 (84.6%) were completed by mothers and 2
(15.4%) were completed by fathers. There were 12 (92.3%) mothers, 10 (76.9%)
fathers, and 1 (7.7%) stepfather residing in the households. One (7.7%) survey did not

indicate which parents were residing in the household.

Mothers’ ages ranged from 30-59 years. Fathers’ ages ranged from 30-60 and

over years. See Table 1 for reported parents’ ages.

Table 1
Parents’ Ages

Mother

Father

Range

30-39 years

40-49 years

50-59 years

60 years and over

■£ = 13 surveys were relumed; one survey did nor indicate age of mother or father

33
Mothers’ reported years of education ranged from 12-18 years. Fathers’
reported years of education ranged from 10-19 years. Respondents’ reports of

parents’ educational levels are provided in Table 2.

Table 2
Parents’ Educational Levels

Mother

Father

Highest Grade Completed

,t indicate educational level of
IL - 13 surveys were returned; one survey did no

Mother or father

34
Household annual income ranged from under $20,000 to the $80,000-$99,000

range. The complete breakdown of respondents’ reported household incomes is
provided in Table 3.

Table 3
Annual Household Income

Range

under $20,000

$20,000-$34,000

$35,000-$49,000

$50,000-$64,000

$65,000-$79,000

$80,000-$99,000

13 surveys were returned

The adolescents with Down syndrome had an age range of 13-19 years (see
Table 4). Table 5 contains the mean household demographics of the respondents in

regard to mother’s age, father’s age, mother’s educational level, father’s educational

level, household annual income, and age of the adolescent with Down syndrome.

Table 4
Ages of Adolescents with Down Syndrome

Age

nl = 13 surveys were returned

The surveys came from various locations throughout the continental United

States, with one (7.7%) survey each from California, Florida, Michigan, New Jersey,
and Washington. Pennsylvania had most of the representation with eight (61.5%) of

the returned surveys.

Table 5

Mean Household Demographics

Mother’s Age

40-49 years

Father’s Age

40-49 years

Mother's Education

14.33 years

Father's Education

13.67 years

Annual Income

$50,000-564,000

Child's Age

15.77 years

na = 13 surveys were returned; one survey did not indicate age of mother or father

Location and Frequency of Health Care
Five (38.5%) of the adolescents were reported as receiving their care from
group medical practices and eight (61.5%) from private medical practices. Routine

visits were recommended once a year during adolescence. Three (23.1 /o) of the

adolescents had no routine visits with their primary care provider, two (15.4%) had

routine visits every six months, and eight (61.5%) had routine visits once a year. The

percentage of adolescents receiving routine health care at less than the recommended
frequency is provided in Figure 1.

Routine Screening
100.00%
£ c
o o

80.00%

-S

60.00%

S E

40.00%

EO 8<D

20.00% --f

y
c
CD d)

□ routine visits

□ dental
□ vision
□ hearing
■ blood pressure

0.00%

Figure 1, Percentage of adolescents with Down syndrome receiving routine

screenings below the recommended frequency.

Routine Screening
Dental examinations were recommended more than once a year during

adolescence. Primary care providers had never mentioned dental examinations for

five (38.5%) of the adolescents. Dental examinations were never mentioned between
the ages of 11 years and 21 years for one (7.7%) adolescent. Dental examinations

were mentioned once a year between the ages of 11 years and 21 years for three

(23.1%) adolescents, and mentioned more than once a year between the ages of 11
years and 21 years for four (30.8%) adolescents. (See Figure 1).
Vision examinations were recommended yearly. Six (46.2%) of the

adolescents had never had their primary care provider perform or recommend a vision
exam. One (7.7%) adolescent had a vision exam performed or recommended more

than once between the ages of 11 years and 21 years, but less than once a year. Five

(38.5 /o) adolescents had a vision exam performed or recommended once a year

between the ages of 11 years and 21 years. One (7.7%) adolescent had a vision exam

performed or recommended more than once a year between the ages of 11 years and
21 years. (See Figure 1).

Hearing exams were recommended yearly. Primary care provider performance
or recommendation of hearing exams was reported by six (46.2%) parents as never

being mentioned. Two (15.4%) parents reported hearing exams as never being

mentioned between the ages of 11 years and 21 years. One (7.7%) parent reported
hearing exams as being performed or recommended more than once between the ages
of 11 years and 21 years, but less than once a year. Three (23.1%) parents reported

hearing exams as being performed or recommended once a year between the ages of
11 years and 21 years. One (7.7%) parent did not answer the frequency of hearing

exams. (See Figure 1).
Blood pressure was recommended to be taken once a year. Blood pressure
was reported as being assessed by primary care providers more than once between the

ages of 11 years and 21 years, but less than once a year by five (38.5%) respondents.
Blood pressure was reported as being assessed once a year between the ages of 11

years and 21 years by three (23.1%) respondents. Blood pressure was reported as

being assessed more than once a year between the ages of 11 years and 21 years by

five (38.5%) respondents. (See Figure 1).

Down Syndrome Complications
Exam to screen for atlantoaxial instability was recommended to be performed

more than once during adolescence, but less than once a year. Primary care provider
screening for atlantoaxial instability had never been mentioned to five (38.5%) of the
adolescents. Screening for atlantoaxial instability had never been performed or
recommended between the ages of 11 years and 21 years for two (15.4%)

adolescents. Atlantoaxial instability screening had been performed or recommended
once between the ages of 11 years and 21 years for six (46.2%) of the adolescents.

(See Figure 2).

Down Syndrome Complications
100.00%

o o

80.00% --

S) s
2 E
5I
E 8

60.00% --

° '5

40.00% --

20.00% -0.00%

□ atlantoaxial
□ hypothyroid
□ cardiac
□ sleep apnea
■ growth

Figure 2. Percentage of adolescents with Down syndrome receiving screenings for
complications of Down syndrome below the recommended frequency.

Thyroid screening was recommended to be performed yearly. Performance or

40
recommendation of thyroid screening by primary care providers was never mentioned

to two (15.4%) respondents. Thyroid screening was performed or recommended once
between the ages of 11 years and 21 years for two (15.4%) respondents. Thyroid

screening was performed or recommended more than once between the ages of 11

years and 21 years, but less than once a year for three (23.1%). Thyroid screening was
performed or recommended once a year between the ages of 11 years and 21 years for
six (46.2%) respondents. (See Figure 2).

Echocardiogram studies for assessment of mitral valve prolapse and aortic
regurgitation was recommended once at age 18 for asymptomatic individuals.
Primary care providers never mentioned echocardiogram study for six (46.2%)

adolescents. The ages of these six adolescents were two at 13 years and one each at

14 years, 15 years, 16 years, and 18 years. It had never been recommended or

performed between the ages of 11 years and 21 years for three (23.1%) adolescents.
The ages of these three adolescents were 13 years, 17 years, and 19 years. It had been

recommended or performed once between the ages of 11 years and 21 years for one

(7.7%) adolescent aged 14 years. Echocardiogram had been recommended or

performed more than once between the ages of 11 years and 21 years, but less than
once a year for three (37.5%) adolescents. These three adolescents were aged 16

years, 18 years, and 19 years. (See Figure 2).
Assessment for sleep apnea was recommended yearly. Primary care providers'
assessment of sleep apnea was reported as never mentioned for seven (53.8%)

adolescents. It was never assessed between the ages of 11 years and 21 years for two

(15.4%) adolescents. Sleep apnea was assessed more than once between the ages of
11 years and 21 years, but less than once a year for two (15.4%) adolescents. It was

assessed once a year between the ages of 11 years and 21 years for one (7.7%)

adolescent. One (7.7/o) survey did not indicate the frequency of assessment of sleep
apnea. (See Figure 2).
Plotting of height and weight on adjusted Down syndrome growth charts was

recommended yearly. Primary care providers had never plotted height and weight on

an adjusted Down syndrome growth chart for eight (61.5%) adolescents. Height and
weight had never been plotted on an adjusted Down syndrome growth chart between
the ages of 11 years and 21 years for one (7.7%) adolescent. Height and weight had

been plotted on an adjusted Down syndrome growth chart once between the ages of
11 years and 21 years for one (7.7%) adolescent. Plotting of height and weight on an

adjusted Down syndrome growth chart was performed more than once between the
ages of 11 years and 21 years, but less than once a year for one (7.7%) adolescent.
Height and weight were plotted on an adjusted Down syndrome growth charts once a
year between the ages of 11 years and 21 years for one (7.7%) adolescent. And,

height and weight were plotted on an adjusted Down syndrome growth charts more
than once a year between the ages of 11 years and 21 years for one (7.7%) adolescent

(See Figure 2).
Adolescent Issues
Discussion of healthy diet was recommended at each yearly visit. Primary
care providers were reported as never discussing healthy diet with five (38.5%)

adolescents. Healthy diet was discussed once between the ages of 11 years and 21

years with two (15.4%) adolescents. Healthy diet was discussed more than once
between the ages of 11 years and 21 years, but less than once a year with two (15.4%)

adolescents Healthy diet was discussed once a year between the ages of 11 years and
21 years with three (23.1%) adolescents. Healthy diet was discussed more than once a

year between the ages of 11 years and 21 years with one (7.7%) adolescent. (See

Figure 3)

Adolescent Issues
100.00%

£ c
o o

-g
O) c
a>

S E
o

8o>

CL *-

80.00%

□ diet

60.00% --

40.00% -20.00% -0.00%

■BZ

□ weight control
□ exercise
□ physical changes

Ilf

Figure 3. Percentage of adolescents with Down syndrome receiving anticipatory

guidance for adolescent issues below the recommended frequency.

Discussion of healthy weight control was recommended at each yearly visit.
Healthy weight control was never discussed with seven (53 8%) adolescents. Healthy

weight control was

discussed once between the ages of 11 years and 21 years with

one (12.5%) adolescent. Healthy weight control was discussed more than once

43
.

between the ages of 11 years and 21 years, but less than once a year with three

(23.1%) adolescents. Healthy weight control was discussed once a year between the
ages of 11 years and 21 years with two (15.4%) adolescents. (See Figure 3).
Discussion of exercise was recommended at each yearly visit. Primary care

providers never mentioned exercise to six (46.2%) adolescents. Exercise was

mentioned once between the ages of 11 years and 21 years to one (7.7%) adolescent.
Exercise was mentioned more than once between the ages of 11 years and 21 years,

but less than once a year to three (23.1%) adolescents. Exercise was mentioned once a
year between the ages of 11 years and 21 years to three (23.1%) adolescents. (See

Figure 3).

Discussion of expected physical changes during adolescence is recommended
at each yearly visit. Primary care providers never mentioned expected physical

changes during adolescence to six (46.2%) of the respondents. Expected physical
changes during adolescence were discussed once between the ages of 11 years and 21

years with one (7.7%) respondent. Expected physical changes during adolescence
were discussed more than once between the ages of 11 years and 21 years, but less

than once a year with three (23.1%) respondents. Expected physical changes during
adolescence were discussed once a year between the ages of 11 years and 21 years

with one (7.7%) respondent. Expected physical changes during adolescence were
discussed more than once a year between the ages of 11 years and 21 years with two
(15.4%) respondents. (See Figure 3)

44
Safety Issues
Discussion of safety issues was recommended at each yearly visit. Primary care

providers were reported as never discussing safety issues with 11 (84.6%)
adolescents. Safety issues were discussed more than once between the ages of 11
years and 21 years, but less than once a year with one (7.7%) adolescent. Safety

issues were discussed once a year between the ages of 11 years and 21 years with one
(7.7%) adolescent. (See Figure 4).

Safety Issues
100.00%

$ c
o o

80.00%

X, «

60.00%

40.00% --

s (V3

20.00% —

Q. *-

0.00%

1_ :

-li

□ seatbelts, helmets, guns

□ alcohol, tobacco, drugs

□ abuse prevention
-----------------------------------------------------------

■

■-

„

Figure 4. Percentage of adolescents with Down syndrome receiving anticipatory

guidance for safety issues below the recommended frequency.

Discussion of (he use oftobacco, alcohol, and drugs was recommended at

each yearly visit. Primary care providers had never mentioned the use of tobacco,
alcohol, and drugs to 12 (92,3%) ofthe adolescents. The use of tobacco, alcohol, and

drugs was discussed once between the ages of 11 years and 21 years with one (7.7%)
adolescent. (See Figure 4).

Discussion regarding prevention of emotional, physical, and sexual abuse was
recommended at each yearly visit. Prevention of emotional, physical, and sexual

abuse discussions by primary care providers were never mentioned to 12 (92.3%) of
the respondents. Prevention of emotional, physical, and sexual abuse was mentioned

more than once between the ages of 11 years and 21 years, but less than once a year
to one (7.7%) respondent. (See Figure 4.)

Sexual Issues
Discussion of sexual issues was recommended at each yearly visit. Primary

care providers did not mention expected sexual interest to 12 (92.3%) of the
adolescents. Expected sexual interest was discussed more than once a year between
the ages of 11 years and 21 years for one (7.7%) adolescent. (See Figure 5).
Discussion of sexual behavior was recommended at each yearly visit. Primary

care providers never mentioned sexual behavior with 11 (84.6%) of the adolescents.
Sexual behavior was discussed once between the ages of 11 years and 21 years with

one (7.7%) adolescent. Sexual behavior was discussed more than once a year between

the ages of 11 years and 21 years with one (7.7%) adolescent. (See Figure 5).
Discussion of birth control was recommended at each yearly visit. Primary

care providers never mentioned birth control to 10 (76.9%) of the respondents. Birth
control was discussed once between the ages of 11 years and 21 years with one

(7.7%) respondent. Birth control was

discussed more than once between the ages of

11 years and 21 years, but less than once a year with two (15.4%) respondents.
Primary care providers have never discussed sexually transmitted diseases with any

(100%) of the respondents. (See Figure 5).

Sexual Issues
100.00%

$ c
o o

80.00%

0J 5

g> 5
5o £o

2 E
v.

U
0)

□ sexual interest

60.00% -H
40.00% -H

□ sexual behavior

□ birth control
□ STDs

20.00% -H
0.00%

Figure 5, Percentage of adolescents with Down syndrome receiving anticipatory

guidance for sexual issues below the recommended frequency.

Social/School Issues
Assessment for depression was recommended yearly. Assessment for

depression by primary care providers with questioning about signs and symptoms has

never occurred for 10 (76.9%) adolescents. Assessment for depression had occurred
once between the ages of 11 years and 21 years for two (12.5%) adolescents.
Assessment for depression had occurred more than once between the ages of 11 years

and 21 years, but less than once a year for one (7.7%) adolescent. (See Figure 6).
Discussion of expected social development was recommended at each yearly

visit. Expected social development discussion by primary care providers had never
occurred for !0 (76.9%) respondents. Expected social development was mentioned

once between the ages of 11 years and 21 years to one (7.7%) respondent. Expected

social development was mentioned more than once between the ages of 11 years and

21 years, but less than once a year to one (7.7%) respondent. Expected social
development was mentioned more than once a year between the ages of 11 years and
21 years to one (7.7%) respondent. (See Figure 6).

Social/School Issues
100.00%

£ c
o o

80.00% --

60.00% --

fI
O> c
(J
V<D

O
o
<D

Q. '»

n
si

40.00% -■

20.00% -0.00%

□ depression

■ social development
□ school problems

□ vocational training
■ adult transition

Figure^ Percentage of adolescents with Down syndrome receiving anticipator
guidance for social/school issues below the recommended frequency.

Discussion of actual or potential school problems was recommended yearly.
Primary

care providers had never discussed actual or potential school problems with

11 (84.6%) respondents. Actual or potential school problems were discussed once

between the ages of 11 years and 21 years with one (7.7%) respondent. Actual or
potential school problems were discussed more than once between the ages of 11

years and 21 years, but less than once a year with one (7.7%) respondent. (See
Figure 6).

Discussion of vocation training was recommended yearly. Primary care
providers had never discussed vocational training with 11 (84.6%) respondents.

Vocational training was discussed once between the ages of 11 years and 21 years
with one (7.7%) respondent. Vocational training was discussed more than once

between the ages of 11 years and 21 years, but less than once a year with one (7.7%)
respondent. (See Figure 6).

Discussion of preparation for transition into adulthood was recommended

yearly. Primary care provider discussions regarding preparation for transition into
adulthood had never occurred for 11 (84.6%) of the respondents. Preparation for
transition into adulthood was mentioned once between the ages of 11 years and 21
years to two (15.4%) of the respondents. (See Figure 6).
Additional Comments

Three (23.1%) of the respondents reported being very satisfied with the care

provided by their adolescents primary care provider’s. Six (46.2%) respondents

reported being satisfied, two (15.4%) reported being neutral, and two (15.4%)
reported being dissatisfied with the care provided by their adolescents’ primary care

providers.
Several surveys had responses to the final three open ended questions (see

49
Appendix F). Responses generally indicated parents felt that primary care providers

did not address the unique issues of adolescents with Down syndrome unless
prompted by the parents. Responses also indicated that parents wish to discuss all

issues, including sensitive ones with their adolescent’s primary care provider and did
not feel any issues should be taboo due to the diagnosis of Down syndrome.

Summary
This chapter presented the results from the survey of parents of adolescents
with Down syndrome. These results were interpreted through descriptive analysis and

the percentage of each response was provided. Analysis of responses to the three open

ended questions and additional comments was also provided.

50
Chapter V
Summary, Conclusions, and Recommendations

This chapter provides a summary of results from the survey of parents of
adolescents with Down syndrome. These results were utilized to determine to what

extent primary care providers are addressing the unique needs of their adolescent

patients with Down syndrome. Conclusions and recommendations based on these
results are also provided.
Summary of Research and Discussion

This section provides a summary of findings from this research project. These

findings were compared to recommendations of frequencies of services by primary
care providers.

Demographics. All 13 responses included in this study were received from
areas within the continental United States. Survey responses indicated that the

majority of respondents had both parents in place within the household. Mothers had

a mean age in the 40-49 year range and a mean of 14.33 years of education. Fathers
had a mean age in the 40-49 year range and a mean of 13.67 years of education. The
mean household annual income of the respondents was in the $50,000-$64,000 range.
The age range of the adolescents in this survey was 13 to 19, with a mean age of

15.77 years. These demographics along with the completed surveys being obtained

from Down syndrome support groups indicated that the sample consisted of stable,
educated, affluent, and interested families.
Individuals in this study would bo among those adolescents with Down

syndrome most likely to receive optimum health care. It can be inferred that if this

group of adolescents with Down syndrome were not receiving health care at the
recommended frequencies, then adolescents with Down syndrome in less educated,

less affluent, and less involved families would not receive health care at the
recommended frequencies either. And, in fact, the latter group would probably
receive less adequate health care, especially when considering parental indications on
the returned surveys that the parents often must direct the primary care provider in

recommended assessments.

Routine Screening. Comparisons were made between the survey results and
recommendations by Guidelines for Adolescent Preventative Services (GAPS), the

American Academy of Pediatrics, and the Down Syndrome Medical Interest Group.
Recommendations for frequency of routine health care visits are for once a year

(Elster & Kuznets, 1994). Responses indicated that this area is being well addressed,
with 10 (76.9%) of the respondents reporting visits at least once a year. This

corresponds with the findings of Igra and Millstein (1993) who wrote that 70% of
adolescents were seen by a physician yearly.

Blood pressure screening was an area generally being met at the

recommended frequency of once a year (Elster & Kuznets, 1994). Other routine

screenings for dental, vision, and hearing are recommended at least yearly (American
Academy of Pediatrics Committee on Genetics, 1994; Cohen, 1996), but results
indicated that primary care providers were not doing well in these areas.

Approximately half of the respondents indicated that these areas had never been

52
addressed by their primary care provider during their children's adolescence.
Down Syndrome Complications, Screenings for issues specific to Down
syndrome were also falling short of the recommendations. Hypothyroidism has a

prevalence of about 15% (Cooley & Graham, 1991) in individuals with Down
syndrome, and most cases develop during adolescence (Trumble, 1993). Therefore,

the recommendation is for annual T3, T4, and TSH levels (Cohen, 1996; Cooley &
Graham, 1991). Responses indicated that six (46.2%) of the adolescents were being

tested annually, but seven (53.8%) of the adolescents in this survey were being tested
at less than the recommended frequency.

Atlantoaxial instability is another condition of high prevalence in the Down
syndrome population. Recommendation is for screening twice during adolescence
(Cohen, 1996). All respondents reported less than the recommended frequency for

screening, and seven (53.8%) respondents reported their primary care provider never
having addressed this issue during their children’s adolescence.
Research indicates a high incidence of new onset mitral valve prolapse during

adolescence (Goldhaber et al., 1987; Geggel et al., 1993). This has led to
recommendations for assessment with echocardiogram once at age 18 years (Cohen,
1996). Responses indicated that only four (30.8%) of the adolescents had been
evaluated with echocardiogram during adolescence. Those adolescents evaluated

were aged 14 years, 16 years, 18 years, and 19 years. Two (15.4%) adolescents aged
18 years or older had never been evaluated with echocardiogram during their

adolescence.

Sleep apnea is another area of concern with the Down syndrome population

and recommendation is for yearly evaluation (Marcus et al., 1991; Cohen, 1996).
Only one (7.7%) respondent reported annual assessment, and nine (69.2%)

respondents reported no assessments for sleep apnea during adolescence.
Individuals with Down syndrome follow a modified growth curve and
specialized growth charts have been developed to assess height and weight (Cronk et

al., 1988). The Down Syndrome Medical Interest Group recommends the specialized
growth charts be utilized when assessing growth of children with Down syndrome,

and height and weight should be assessed annually (Cohen, 1996; Elster & Kuznets,

1994). Nine (69.2%) of the respondents reported never having height and weight
plotted on adjusted growth charts during adolescence, and only two (15.4%) reported

it at or above the recommended frequency of annually.
Adolescent Issues. Issues that should be addressed with all adolescents are

also not being addressed at the recommended frequencies by primary care providers

for their adolescent patients with Down syndrome. Discussion of healthy diet, healthy
weight control, exercise, and expected physical changes during adolescence is

recommended to be included at each annual visit (Elster & Kuznets, 1994).
Responses indicated that five (38.5%) of the respondents had never had their primary

care provider discuss healthy diet with them or their adolescent. Seven (53.8%)

respondents had never had their primary care provider discuss healthy weight control
with them or their adolescent. Six (46.2%) respondents had never had their primary

care provider discuss exercise with them or their adolescent. Six (46.2%) respondents

had never had their primary care provider discuss expected physical changes during

adolescence.

Safety Issues. Health guidance concerning safety; use of tobacco, alcohol, and
drugs, and prevention of emotional, physical, and sexual abuse is also recommended
to be given at each annual visit (Elster & Kuznets, 1994). Again, responses indicated

that this was not occurring. Eleven (84.6%) respondents reported never having had
safety issues addressed by their primary care provider. Twelve (92.3%) respondents
reported never having had their primary care provider discuss the use of tobacco,

alcohol, and drugs, or the prevention of emotional, physical, and sexual abuse with
them or their adolescent.

Sexual Issues. Sexual development in adolescents with Down syndrome

follows the same pattern as their non-Down syndrome peers (Cooley & Graham,
1991). Therefore, this area cannot be ignored, and the primary care provider is
recommended to give guidance to the adolescent and parents (Mims, 1996; Elster &
Kuznets, 1994). Responses indicated that this area is severely under-addressed by
primary care providers. Twelve (92.3%) of the respondents reported never having had
their primary care provider discuss expected sexual interest with them or their

adolescent. Eleven (84.6%) of the respondents reported never having had their

primary care provider discuss sexual behavior with them or their adolescent. Ten

(76.9%) respondents reported never having had birth control discussed by their
primary care provider.

No respondent reported their primary care provider having had discussed

55
sexually transmitted diseases with them or their child. This at a time when sexually
transmitted chlamydia infection affects 3 to 4 million individuals annually and is most

common during mid to late adolescence (Richert et al., 1990),
Social/School Issues. Social and school issues discussions are recommended
at each annual visit (American Academy of Pediatrics Committee on Genetics, 1994;

Elster & Kuznets, 1994). This is another area being poorly addressed by primary care

providers. Ten (76.9%) respondents reported no assessment by their primary care
provider for signs and symptoms of depression. Ten (76.9%) respondents reported

never having had their primary care provider discuss expected social development

with them or their adolescent. Eleven (84.6%) of the respondents reported never
having had their primary care provider address issues regarding potential or actual
school problems, vocational training, or transition into adulthood.
Overall Satisfaction. The respondents of this study were generally satisfied

with their primary care provider. Responses to the open ended questions indicated

that for many issues the parents are the ones to initiate discussions or screenings. This
parent-driven provision of services should serve an educated, highly motivated
population. However, this study indicated that even with educated, affluent, involved

families, health care needs of adolescents with Down syndrome were not being met
by their primary care providers. This corresponds with writings by Igra and Millstein

(1993) that adolescents' unique health care needs were not being addressed and twothirds of visits by adolescents to physicians provided no counseling advice of any
type.

The results of this study indicated that primary care providers are not meeting
the needs of their adolescent patients with Down syndrome. According to Dorothea

Orem (1995), the primary care provider should assist all adolescent patients by

performing those functions the patients are unable to perform for themselves, guiding
and directing the patients in healthy behaviors, assist the patients’ parents in providing
and maintaining an environment that supports development, and providing education
to the patients in areas of knowledge deficits. In addition, Orem indicates the need for

continuous assessment for the complications frequently associated with Down

syndrome. To meet the recommendations of Orem, the primary care provider must
perform the recommended screenings and provide the recommended anticipatory
guidance at the recommended frequencies.

Conclusions
This study indicated that primary care providers do not meet published

guidelines in addressing all areas of health care needs of adolescents with Down
syndrome except frequency of routine visits and blood pressure screening and, in fact,

are severely deficient in anticipatory guidance. This study does however have several
limitations, as indicated in Chapter I, effecting conclusions that can be drawn.

Additionally, the sample size of 13 returned surveys may affect generalizability of
this study.

Recommendations
This study .ndreated that primaty care providers are not currently meeting the

needs of their adolescent patients with Down syndrome. Further information is

required to determine what changes need to be instituted to increase the health care
received by this population. Some recommendations to gain that information are:

1. Repeat this study with a larger sample size.

2. Repeat this study with a more diverse sample.
3. Study a sample of parents of adolescents without Down syndrome for comparison.
4. Study primary care providers regarding their perceptions of appropriate care for

their adolescent patients with and without Down syndrome.
5. Study primary care providers to determine their knowledge level regarding
adolescent issues.

6. Study primary care providers to determine their knowledge level regarding

complications frequently associated with Down syndrome.

If the first four recommended studies would confirm the inadequacy of care
provided by primary care providers, then the fifth and sixth recommended studies

could be performed to determine if knowledge deficit by primary care providers is the
cause. If, in fact, knowledge deficit were the cause, education of primary care
providers in the health care needs of their adolescent patients with and without Down

syndrome would be indicated.
Summary

This chapter provided a summary of this research project. Data indicated that
primary care providers were not addressing the health care needs of their patients

with Down syndrome at the recommended frequencies. Discussion of implications of
these findings and recommendations for further studies were provided.

References
American Academy of Pediatrics Committee on Genetics (1994). Health
supervision for children with Down syndrome. Pediatrics, 93, 855-859.
American Academy of Pediatrics Committee on Sports Medicine and Fitness
(1995). Atlantoaxial instability in Down syndrome: Subject review. Pediatrics, 96,

151-154.

Baird, P. A., & Saadovnick, A. D. (1987). Life expectancy in Down
syndrome. The Journal of Pediatrics, 110, 849-854.

Balkany, T. J., Downs, M. P., Jafek, B. W., & Krajicek, M. J. (1979). Hearing
loss in Down's syndrome. Clinical Pediatrics, 18, 116-118.

Barnett, M. L., Press, K. P., Friedman, D., & Sonnenberg, E. M. (1986). The

prevalence of periodontitis and dental caries in a Down's syndrome population.
Journal of Periodontology, 57, 288-293.

Bovicelli, L., Orsini, L. F., Rizzo, N., Montacuti, V., & Bacchetta, M. (1982).
Reproduction in Down syndrome. Obstetrics & Gynecology, 59, 13S-17S.
Caputo, A. R., Wagner, R. S., Reynolds, D. R., Guo, S., & Goel, A. K. (1989).
Down syndrome: Clinical review of ocular features. Clinical Pediatrics, 28, 355-358.

Chicoine, B., McGuire, D., Hebein, S., & Gilly, D. (1995). Use of the

community-oriented primary care model for a special-needs population: A clinic for
adults with Down syndrome. American Journal of Public Health, 85, 869-870

Cohen, W. I. (1996). Health care guidelines for individuals with Down
syndrome. Reprinted from Down Syndrome Quarterly, volume 1, number 2, June,

1996. Available: http://www.denison.edu/dsq/health96.html.
Cooley, W. C.,& Graham, J. M. (1991). Common syndromes and

management issues for primary care physicians: Down syndrome — an update and

review for the primary pediatrician. Clinical Pediatrics, 30, 233-253.

Cronk, C., Crocker, A. C., Pueschel, S. M., Shea, A. M., Zackai, E., Pickens,
G., & Reed, R. B. (1988). Growth charts for children with Down syndrome: 1 month

to 18 years of age. Pediatrics, 81, 102-110.

Elster, A. B., & Kuznets, N. J. (1994). Guidelines for Adolescent Preventive
Services. Baltimore: Williams and Wilkins.

Geggel, R. L., OBrien, J. E., & Feingold, M. (1993). Development of valve
dysfunction in adolescents and young adults with Down syndrome and no known

congenital heart disease. The Journal of Pediatrics, 122, 821-823.

Goldhaber, S. Z., Brown, W. D., & St. John Sutton, M G. (1987). High

frequency of mitral valve prolapse and aortic regurgitation among asymptomatic
adults with Down's syndrome. JAMA, 258, 1793-1795.

Hedberg, V. A., Byrd, R. S., Klein, J. D., Auinger, P., & Weitzman, W.
(1996). The role of community health centers in providing preventive care to

adolescents. Archives of Pediatric/Adolescent Medicine, 150, 603-608.
Igra, V., & Millstein, S. G. (1993). Current status and approaches to

improving preventive services for adolescents. JAMA, 269, 1408-1412.
Key, J. D., Marsh, L. D., & Darden, P. M. (1995). Adolescent medicine in
pediatric practice: A survey of practice and training. The American Journal of the

Medical Sciences. 309. 83-87.

Magill, C. J. (1989). Down syndrome: Update for the primary care
practitioner. Journal of the American Academy of Physician Assistants. 2, 5-13.

Marcus, C. L., Keens, T. G., Bautista, D. B., von Pechmann, W. S., & Ward,
S. L. D. (1991). Obstructive sleep apnea in children with Down syndrome. Pediatrics,
88, 132-139.

Millstein, S. G., Nightingale, E. O., Petersen, A. C., Mortimer, A. M., &

Hamburg, D. A. (1993). Promoting the healthy development of adolescents. JAMA,
269, 1413-1415.

Mims, J. (1996). Sexuality and related issues in the preadolescent and
adolescent female with epilepsy. Journal of Neuroscience Nursing, 28, 102-106.

Newacheck, P. W. (1989). Adolescents with special health needs: Prevalence,
severity, and access to health services. Pediatrics, 84, 872-881.

Noble, J. E. (1996). Primary care: Introduction. In C. D. Berkowitz (Ed.),
Pediatrics: A primary care approach (pp. 1-3). Philadelphia: W. B. Saunders

Company.

Orem, D. E. (1995). Nursing: Concepts of Practice (5th ed.). St. Louis, MO:
Mosby-Year Book, Inc.
Pueschel, S. M., Scola, F. H„ & Pezzullo, J. C. (1992). A longitudinal study of

atlanto-dens relationships in asymptomatic individuals with Down syndrome.

Pediatrics, 89, 1194-1198.
Richert, V. I, Jay, M. S„ & Gottlieb, A. A. (1990). Adolescent wellness:

61
Facilitating compliance in social morbidities. Medical Clinics of North America. 74.
1135-1148.

Roizen, N. J., Mets, M. B., & Biondis, T. A. (1994). Ophthalmic disorders in

children with Down syndrome. Developmental Medicine and Child Neurology, 36,
594-600.

Sheridan, R_, Llerena, J. Jr., Matkins, S., Debenham, P., Cawood, A., &

Bobrow, M. (1989). Fertility in a male with trisomy 21. Journal of Medical Genetics,
26, 294-298.
Sifuentes, M. (1996). Talking to adolescents. In C. D. Berkowitz (Ed.),
Pediatrics: A primary care approach (pp. 10-12). Philadelphia: W. B. Saunders
Company.

Spencer, C. H., Fife, R. Z., & Rabinovich, C. E. (1995). The school
experience of children with arthritis. Pediatric Clinics of North America, 42, 1285-

1298.
Thomas, C. L. (Ed.). (1989). Taber's cyclopedic medical dictionary (16th ed.).

Philadelphia: F. A. Davis Company.
Trumble, S. (1993). How to treat people with Down syndrome: Some tips for
family physicians. Available: http://www.nas.com/downsyn/trumble.html.
Wasson, J. H., Kairys, S. W., Nelson, E. C., Kalishman, N., Baribeau, P., &

Wasson, E. (1995). Adolescent health and social problems. Archives of Family
Medicine, 4, 51-56.

Williams, J. K. (1983, January/February). Reproductive decisions:

Adolescents with Down syndrome. Pediatric Nursing. 9. 43-44, 58.

Appendixes

64
Appendix A

Selection of
Guidelines for Adolescent Preventive Services

(GAPS)
Recommendations

1 From ages 11 to 21, all adolescents should have an annual preventive services visit.
2. Preventive services should be age and developmentally appropriate, and should be

sensitive to individual and sociocultural differences.
3. Parents or other adult caregivers should receive health guidance at least once

during their child's early adolescence, middle adolescence, and late adolescence.
4. All adolescents should receive health guidance annually to promote a better
understanding of their physical growth, psychosocial and psychosexual development,
and the importance of becoming actively involved in decisions regarding their health

care.
5. All adolescents should receive health guidance annually to promote the reduction

of injuries.
6. All adolescents should receive health guidance annually about dietary habits,
including the benefits of a healthy diet, and ways to achieve a healthy diet and safe

weight management.

7. All adolescents should receive health guidance annually about the benefits of
exercise and should be encouraged to engage in safe exercise on a regular basis.

8. All adolescents should receive health guidance annually regarding responsible

65
sexual behaviors, including abstinence and latex condoms to prevent STDs including

HIV infection. Appropriate methods of birth control should be made available, as

should instructions on how to use them effectively.

9. All adolescents should receive health guidance annually to promote avoidance of
tobacco, alcohol and other abusable substances.

10. All adolescents should be screened annually for hypertension according to the
protocol developed by the National Heart, Lung, and Blood Institute Second Task

Force on Blood Pressure Control in Children.

11. Selected adolescents should be screened to determine their risk of developing

hyperlipidemia and adult coronary heart disease, following the protocol developed by
the Expert Panel on Blood Cholesterol Levels in Children and Adolescents.

12. All adolescents should be screened annually for eating disorders and obesity by

determining weight and stature, and by asking about body image and dieting patterns.

13 . All adolescents should be asked annually about their use of tobacco products
including cigarettes and smokeless tobacco.
14. All adolescents should be asked annually about their use of alcohol and other

abusable substances, and about their use of over-the-counter or prescription drugs for

non-medical purposes.

15. All adolescents should be asked annually about involvement in sexual behaviors
that may result in unintended pregnancy and STDs, including HIV infection.
16. Sexually active adolescents should be screened for STDs.
17. Adolescents at risk for HIV infection should be offered confidential HIV

screening with the ELISA and confirmatory test.
18. Female adolescents who are sexually active, or any female 18 or older, should be

screened annually for cervical cancer by use of a Pap test.
19. All adolescents should be asked annually about behaviors or emotions that

indicate recurrent or severe depression or risk of suicide.
20. All adolescents should be asked annually about a history of emotional, physical,

and sexual abuse.
21. All adolescents should be asked annually about learning or school problems.

22. All adolescents should receive prophylactic immunizations according to the
guidelines established by the federally convened Advisory Committee on
Immunization Practices.

67
Appendix B

Survey for Parents of Adolescents with Down Syndrome

Relationship of person completing survey:

Parents living in household:

(mom)

(dad)

(step-mom)

(step-dad)

(mom)

(dad)

(step-dad)

(step-mom)

Mother's age: (under 30)

(30-39)

(40-49)

(50-59)

(60 and over)

Father's age:

(30-39)

(40-49)

(50-59)

(60 and over)

(under 30)

Mother's highest grade completed:
Father's highest grade completed:

(under $20,000)

($20,000-$34,000)

($35,000-$49,000)

($50,000-$64,000)

($65,000-$79,000)

($80,000-$99,000)

Household annual income:

($100,000 or over)
State or Country of Residence:

Age of child with Down syndrome:

Sex of child with Down syndrome:

Where does your child receive his/her primary health care?
Adoes not receive primary health care
Bcommunity health clinic
Cgroup medical practice
Dprivate health care provider practice
EDown syndrome clinic
Fother

What is the usual frequency of your child’s routine visits (physicals, not sick
visits) to your health care provider (physician, nurse practitioner, physician
assistant, clinic)?
Ano routine visits
Bvisits every 3 months
Cvisits every 6 months
Dvisits once a year
Evisits every 2 years

How often has your health care provider performed or recommended a dental
exam for your child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
once between the ages of 11 years and 21 years
CDmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
more than once a year between the ages of 11 years and 21 years
F-

How often has your health care provider performed or recommended a vision
exam for your child?
has never mentioned it
Anever between the ages of 11 years and 21 years
Bonce between the ages of 11 years and 21 years
Cmore than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore
than once a year between the ages of 11 years and 21 years
F-

How often has your health care provider performed or recommended a
hearing exam for your child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
Conce between the ages of 11 years and 21 years
Dmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
Fmore than once a year between the ages of 11 years and 21 years

How often has your health care provider performed or recommended a exam
for cervical spine (neck) instability (atlantoaxial instability)?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
once between the ages of 11 years and 21 years
CDmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
Fmore than once a year between the ages of 11 years and 21 years

How often has your health care provider performed or recommended thyroid
gland tests such as T3, T4, and/or TSH for your child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
once between the ages of 11 years and 21 years
Cmore than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore than once a year between the ages of 11 years and 21 years
F-

How often has your health care provider performed or recommended a
echocardiogram study of the heart for your child?
has never mentioned it
Anever between the ages of 11 years and 21 years
Bonce between the ages of 11 years and 21 years
Cmore than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore
than once a year between the ages of 11 years and 21 years
F-

70
9.

How often has your health care provider ask about snoring, unusual sleep
positions, fatigability during the day, reappearance of napping, or other
symptoms of sleep apnea?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
Conce between the ages of 11 years and 21 years
Dmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
Fmore than once a year between the ages of 11 years and 21 years

10.

How often has your health care provider checked blood pressure on your
child?
Ahas never checked it
Bnever between the ages of 11 years and 21 years
Conce between the ages of 11 years and 21 years
Dmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
Fmore than once a year between the ages of 11 years and 21 years

11.

How often has your health care provider plotted your child’s height and
weight on an adjusted Down syndrome growth chart?
Ahas never plotted it
Bnever between the ages of 11 years and 21 years
once between the ages of 11 years and 21 years
Cmore than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore
than once a year between the ages of 11 years and 21 years
F-

12.

How often has your health care provider discussed healthy diet with you
and/or your child?
has never mentioned it
Anever between the ages of 11 years and 21 years
Bonce between the ages of 11 years and 21 years
Cmore than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore
than once a year between the ages of 11 years and 21 years
F-

71
13.

How often has your health care provider discussed healthy weight control
with you and/or your child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
Conce between the ages of 11 years and 21 years
Dmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
Fmore than once a year between the ages of 11 years and 21 years

14.

How often has your health care provider discussed exercise with you and/or
your child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
once between the ages of 11 years and 21 years
CDmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
Fmore than once a year between the ages of 11 years and 21 years

15.

How often has your health care provider discussed expected physical changes
during adolescence with you and/or your child?
Ahas never mentioned it
never between the ages of 11 years and 21 years
Bonce between the ages of 11 years and 21 years
Cmore than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore than once a year between the ages of 11 years and 21 years
F-

16.

How often has your health care provider discussed safety issues such as use of
seatbelts and helmets, and avoidance of guns with you and/or your child?
has never mentioned it
Anever between the ages of 11 years and 21 years
Bonce between the ages of 11 years and 21 years
Cmore than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore
than once a year between the ages of 11 years and 21 years
F-

72
17.

How often has your health care provider discussed use of tobacco, alcohol,
and drugs with you and/or your child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
Conce between the ages of 11 years and 21 years
Dmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
Fmore than once a year between the ages of 11 years and 21 years

18.

How often has your health care provider discussed prevention of emotional,
physical, and sexual abuse with you and/or your child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
once between the ages of 11 years and 21 years
CDmore than once between the ages of 11 years and 21 years, but less
than once a year
once a year between the ages of 11 years and 21 years
EFmore than once a year between the ages of 11 years and 21 years

19.

How often has your health care provider discussed your adolescent's expected
sexual interest with you and/or your child?
has never mentioned it
Anever between the ages of 11 years and 21 years
Bonce between the ages of 11 years and 21 years
Cmore than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore than once a year between the ages of 11 years and 21 years
F-

20.

How often has your health care provider discussed sexual behavior with you
and/or your child?
has never mentioned it
Anever between the ages of 11 years and 21 years
Bonce between the ages of 11 years and 21 years
Cmore than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore
than once a year between the ages of 11 years and 21 years
F-

73
21.

How often has your health care provider discussed birth control with you
and/or your child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
Conce between the ages of 11 years and 21 years
Dmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
Fmore than once a year between the ages of 11 years and 21 years

22.

How often has your health care provider discussed sexually transmitted
diseases with you and/or you child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
Conce between the ages of 11 years and 21 years
Dmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
Fmore than once a year between the ages of 11 years and 21 years

23.

How often has your health care provider asked about signs and symptoms of
depression such as skill and memory loss, significant activity slow down, or
extreme withdrawal in your child?
has never mentioned it
Anever between the ages of 11 years and 21 years
Bonce between the ages of 11 years and 21 years
Cmore
than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore than once a year between the ages of 11 years and 21 years
F-

24.

How often has your health care provider discussed expected social
development with you and/or your child?
has never mentioned it
Anever
between the ages of 11 years and 21 years
Bonce between the ages of 11 years and 21 years
Cmore
than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore
than once a year between the ages of 11 years and 21 years
F-

74
25.

How often has your health care provider discussed actual or potential school
problems with you and/or your child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
Conce between the ages of 11 years and 21 years
Dmore than once between the ages of 11 years and 21 years, but less
than once a year
Eonce a year between the ages of 11 years and 21 years
Fmore than once a year between the ages of 11 years and 21 years

26.

How often has your health care provider discussed vocational training with
you and/or your child?
Ahas never mentioned it
Bnever between the ages of 11 years and 21 years
Conce between the ages of 11 years and 21 years
Dmore than once between the ages of 11 years and 21 years, but less
than once a year
once a year between the ages of 11 years and 21 years
Emore than once a year between the ages of 11 years and 21 years
F-

27.

How often has your health care provider discussed preparation for transition
into adulthood such as job opportunities and/or living arrangements with you
and/or your child?
has never mentioned it
Anever between the ages of 11 years and 21 years
Bonce between the ages of 11 years and 21 years
Cmore than once between the ages of 11 years and 21 years, but less
Dthan once a year
once a year between the ages of 11 years and 21 years
Emore
than once a year between the ages of 11 years and 21 years
F-

28.

How satisfied are you with the care provided by your health care provider for
your child with Down syndrome?
very satisfied
Asatisfied
Bneutral
Cdissatisfied
Dvery dissatisfied
E-

75
29.

Are there any other adolescent issues your health care provider has discussed
with you and/or your child?

30.

Are there any other adolescent issues you wish your health care provider
would have discussed with you and/or your child?

31.

Are there any adolescent issues you do NOT wish your health care provider to
discuss with you and/or your child?

76
Appendix C

Introduction Letter to Down Syndrome Parent Support Group

Hello. My name is Debbie Wasson. I am the mother of a sixteen-year-old daughter,
Samantha, with Down syndrome. I am also a graduate nursing student at Edinboro
University of Pennsylvania. For my graduate thesis, I am attempting to determine the
extent to which health care providers (physicians, nurse practitioners, physician
assistants, clinics) are addressing the special needs of adolescents with Down
syndrome. To obtain this information, I am asking for your help. If you are the
parent of an adolescent (between the ages of 11 years and 21 years old) with Down
syndrome I would appreciate you taking the time to complete the following survey.
The survey contains 31 questions and should take about 15 minutes to complete.
Please indicate your answers to the questions by circling the most correct answer.
Return the survey to me by March 21, 1997 privately with the self-addressed,
stamped envelope provided for your convenience. Any additional comments at the
end of the survey are also appreciated. All information will be kept strictly
anonymous and will be presented as group data so that no individual respondent will
be identified. My ultimate goal is to improve the health care provided for all our
children. Thank you for your time and help. If you have any questions, please feel
free to contact me at my e-mail address (dwass@GREMLAN.ORG) or Edinboro
University (814-732-2900).
Debbie Wasson
Graduate Assistant
Department of Nursing
Edinboro University
Edinboro, PA 16444
Phone. 814-732-2900
dwass@GREML AN. ORG

Appendix D
Introduction Letter to Down Syndrome Listserver
Hello. My name is Debbie Wasson. Most of you know me as mom to Samantha (16,
DS). I am also a graduate nursing student at Edinboro University of Pennsylvania.
For my graduate thesis, I am attempting to determine the extent to which health care
providers (physicians, nurse practitioners, physician assistants, clinics) are addressing
the special needs of adolescents with Down syndrome. To obtain this information, I
am asking for your help. If you are the parent of an adolescent (between the ages of
11 years and 21 years old) with Down syndrome I would appreciate you taking the
time to complete the following survey. The survey contains 31 questions and should
take about 15 minutes to complete. Please indicate your answers to the questions by
deleting all the unwanted answers and return the survey to me privately by E-mail or
regular mail. Please return completed surveys by March 10, 1997. Any additional
comments at the end of the survey are also appreciated. Headers with identifying
information will be removed and destroyed to prevent identification of respondents.
All information will be kept strictly confidential and will be presented as group data
so that no individual respondent will be identified. My ultimate goal is to improve
the health care provided for all our children. Thank you for your time and help. If
you have any questions, please feel free to contact me at my e-mail address
(dwass@GREMLAN.ORG) or Edinboro University (814-732-2900).

Debbie Wasson
Graduate Assistant
Department of Nursing
Edinboro University
Edinboro, PA 16444
Phone: 814-732-2900
dwass@GREMLAN.ORG

Appendix E
Edinboro University IRB Exempt Review

::^;z
r.

yI y

L-L^^^C

Appendix F
Survey Data

Relationship of person completing survey:

Mother -— 11 (84.6%)

2 (15.4%)
Parents living in household

Mother —- 12 (92.3%)
Father —- 10 (76.9%)

Step-father

Mother’s age

30-39

(7.7%)

2 (15.4%)

7 (53.8%)
3 (23.1%)

Not answered
Father’s age

Mother’s highest grade completed

(7.7%)

30-39

3 (23.1%)

40-49

4 (30.8%)

50-59

4 (30.8%)

60 and over

(7.7%)

Not answered

(7.7%)

3 (23.1%)

(7.7%)

3 (23.1%)
2 (15.4%)

Not an

Father’s highest grade completed

(7.7%)

Not answered

(7.7%)

-5 (38.5%)
(7.7%)

1
14

2 (15.4%)

(7.7%)

Household annual income

State of Country of Residence

(7.7%)

Not answered

(7.7%)

■under $20,000

(7.7%)

$20,000-$34,000

(7.7%)

$35,000-$49,000

3 (23.1%)

$50,000-$64,000

3 (23.1%)

$65,000-$79,000

2 (15.4%)

$80,000-$99,000

3 (23.1%)

California

(7.7%)

Florida

(7.7%)

Michigan

(7.7%)

New Jersey

(7.7%)

Pennsylvania
Washington

8 (61.5%)
1

(7.7%)

81
Age of child with Down syndrome-

Sex of child with Down syndrome

3 (23.1%)

2 (15.4%)

Female

7 (53.8%)

Male

6 (46.2%)

(7.7%)

1. Where does your child receive his/her primary health care?

C - group medical practice

5 (38.5%)

D - private health care provider practice

8 (61.5%)

2. What is the usual frequency of your child's routine visits (physicals, not sick visits)
to your health care provider (physician, nurse practitioner, physician assistant,

clinic)?
A - no routine visits

3 (23.1%)

C - visits every 6 months

2 (15.4%)

D - visits once a year

8 (61.5%)

3. How often has your health care provider performed or recommended a dental exam

for your child?
A - has never mentioned it

5 (38.5%)

B - never between the ages of 11 years and 21 years-

(7.7%)

82
E - once a year between the ages of 11 years and 21 years

3 (23.1%)

F - more than once a year between the ages of 11 years and 21 years
4 (30.8%)
4. How often has your health care provider performed or recommended a vision exam

for your child?
A - has never mentioned it

6 (46.2%)

D - more than once between the ages of 11 years and 21 years, but less than
once a year

E - once a year between the ages of 11 years and 21 years

5 (38.5%)

(7.7%)

F - more than once a year between the ages of 11 years and 21 years
1

(7.7%)

5. How often has your health care provider performed or recommended a hearing
exam for your child?

A - has never mentioned it

6 (46.2%)

B - never between the ages of 11 years and 21 years-

2 (15.4%)

D - more than once between the ages of 11 years and 21 years, but less than

(7.7%)

once a year

E - once a year between the ages of 11 years and 21 years

3 (23.1%)

not answered

(7.7%)

6. How often has your health care provider performed or recommended a exam for
cervical spine (neck) instability (atlantoaxial instability)?

A - has never mentioned it

5 (38.5%)

83
B - never between the ages of 11 years and 21 years-

2 (15.4%)

C - once between the ages of 11 years and 21 years-

6 (46.2%)

7. How often has your health care provider performed or recommended thyroid gland
tests such as T3, T4, and/or TSH for your child?

A - has never mentioned it

2 (15.4%)

C - once between the ages of 11 years and 21 years-

2 (15.4%)

D - more than once between the ages of 11 years and 21 years, but less than
once a year

3 (23.1%)

E - once a year between the ages of 11 years and 21 years

6 (46.2%)

8. How often has your health care provider performed or recommended a
echocardiogram study of the heart for your child?
A - has never mentioned it

6 (46.2%)

B - never between the ages of 11 years and 21 years-

3 (23.1%)

C - once between the ages of 11 years and 21 years-

(7.7%)

D - more than once between the ages of 11 years and 21 years, but less than
once a year

3 (23.1%)

9. How often has your health care provider ask about snoring, unusual sleep positions,

fatigability during the day, reappearance of napping, or other symptoms of sleep
apnea?

A - has never mentioned it

7 (53.8%)

B - never between the ages of 11 years and 21 years-

2 (15.4%)

D - more than once between the ages of 11 years and 21 years, but less than

once a year

2 (15.4%)

E - once a year between the ages of 11 years and 21 years

(7.7%)

not answered

(7.7%)

10. How often has your health care provider checked blood pressure on your child?

D - more than once between the ages of 11 years and 21 years, but less than

once a year

5 (38.5%)

E - once a year between the ages of 11 years and 21 years

3 (23.1%)

F - more than once a year between the ages of 11 years and 21 years

5 (38.5%)
11. How often has your health care provider plotted your child’s height and weight on
an adjusted Down syndrome growth chart?

A - has never plotted it

8 (61.5%)

B - never between the ages of 11 years and 21 years-

(7.7%)

C - once between the ages of 11 years and 21 years-

(7.7%)

D - more than once between the ages of 11 years and 21 years, but less than
once a year

(7.7%)

E - once a year between the ages of 11 years and 21 years

(7.7%)

F - more than once a year between the ages of 11 years and 21 years
1

(7.7%)

12. How often has your health care provider discussed healthy diet with you and/or
your child?

A - has never mentioned it

5 (38.5%)

85
C - once between the ages of 11 years and 21 years-

2 (15.4%)

D - more than once between the ages of 11 years and 21 years, but less than

once a year

2 (15.4%)

E - once a year between the ages of 11 years and 21 years

3 (23.1%)

F - more than once a year between the ages of 11 years and 21 years
—I

(7.7%)

13. How often has your health care provider discussed healthy weight control with

you and/or your child?
A - has never mentioned it

7 (53.8%)

C - once between the ages of 11 years and 21 years-

(7.7%)

D - more than once between the ages of 11 years and 21 years, but less than
once a year

3 (23.1%)

E - once a year between the ages of 11 years and 21 years

2 (15.4%)

14. How often has your health care provider discussed exercise with you and/or your

child?

A - has never mentioned it

6 (46.2%)

C - once between the ages of 11 years and 21 years-

(7.7%)

D - more than once between the ages of 11 years and 21 years, but less than

once a year

3 (23.1%)

E - once a year between the ages of 11 years and 21 years

3 (23.1%)

15. How often has your health care provider discussed expected physical changes

during adolescence with you and/or your child?

A - has never mentioned it

6 (46.2%)

C - once between the ages of 11 years and 21 years-

(7.7%)

D - more than once between the ages of 11 years and 21 years, but less than
once a year
E - once a year between the ages of 11 years and 21 years

3 (23.1%)

(7.7%)

F - more than once a year between the ages of 11 years and 21 years
2 (15.4%)

16. How often has your health care provider discussed safety issues such as use of
seatbelts and helmets, and avoidance of guns with you and/or your child?

A - has never mentioned it

11 (84.6%)

D - more than once between the ages of 11 years and 21 years, but less than

once a year

(7.7%)

E - once a year between the ages of 11 years and 21 years -— 1

(7.7%)

17. How often has your health care provider discussed use of tobacco, alcohol, and

drugs with you and/or your child?

A - has never mentioned it
C - once between the ages of 11 years and 21 years-

12 (92.3%)
1

(7.7%)

18. How often has your health care provider discussed prevention of emotional,

physical, and sexual abuse with you and/or your child?

A - has never mentioned it

12 (92.3%)

D - more than once between the ages of 11 years and 21 years, but less than
once a year —-

1 (7.7%)

19. How often has your health care provider discussed your adolescent's expected

sexual interest with you and/or your child?
A - has never mentioned it

12 (92.3%)

F - more than once a year between the ages of 11 years and 21 years

1 (7.7%)

20. How often has your health care provider discussed sexual behavior with you
and/or your child?

A - has never mentioned it

C - once between the ages of 11 years and 21 years-

11 (84.6%)
1

(7.7%)

F - more than once a year between the ages of 11 years and 21 years

(7.7%)
21. How often has your health care provider discussed birth control with you and/or

your child?

A - has never mentioned it

C - once between the ages of 11 years and 21 years-

10 (76.9%)

(7.7%)

D - more than once between the ages of 11 years and 21 years, but less than
once a year

22. How often has your health care provider discussed sexually transmitted diseases
with you and/or you child?

A - has never mentioned it

23. How often has your health care provider asked about signs and symptoms of
depression such as skill and memory loss, significant activity slow down, or extreme

88
withdrawal in your child?

A - has never mentioned it
C - once between the ages of 11 years and 21 years-

10 (76.9%)
2 (15.4%)

D - more than once between the ages of 11 years and 21 years, but less than
once a year

(7.7%)

24. How often has your health care provider discussed expected social development

with you and/or your child?

A - has never mentioned it

C - once between the ages of 11 years and 21 years-

10 (76.9%)

(7.7%)

D - more than once between the ages of 11 years and 21 years, but less than

once a year

(7.7%)

F - more than once a year between the ages of 11 years and 21 years
1

(7.7%)

25. How often has your health care provider discussed actual or potential school

problems with you and/or your child?

A - has never mentioned it
C - once between the ages of 11 years and 21 years-

11 (84.6%)
1

(7.7%)

D - more than once between the ages of 11 years and 21 years, but less than

once a year

(7.7%)

26. How often has your health care provider discussed vocational training with you

and/or your child?
A - has never mentioned it

11 (84.6%)

C - once between the ages of 11 years and 21 years'

(7.7%)

D - more than once between the ages of 11 years and 21 years, but less than
once a year

(7.7%)

27. How often has your health care provider discussed preparation for transition into
adulthood such as job opportunities and/or living arrangements with you and/or your

child?

A - has never mentioned it

C - once between the ages of 11 years and 21 years-

11 (84.6%)

2 (15.4%)

28. How satisfied are you with the care provided by your health care provider for

your child with Down syndrome?

A - very satisfied

3 (23.1%)

B - satisfied

6 (46.2%)

C - neutral

2 (15.4%)

D - dissatisfied

2 (15.4%)

29. Are there any other adolescent issues your health care provider has discussed with
you and/or your child?

A. The doctor is there strictly for physical health concerns.

B. Not that I recall.

C. No.
D. No.

E. In fairness, I must mention that some of the previous issues have been

discussed, or exams have been performed, at my mention. It does seem that,

generally speaking, it is the parent's duty to inform the health care provider,

rather than the other way around when a Down syndrome child is involved.

F. No.
G. No.

H. My health care provider is very aware that the Barber Center Family
Support Services discuss job opportunities, CBI (community based
instruction), living arrangements, sexual issues, behavioral issues and there is

no need for dual services in these areas.
I. No.

J. No.
30. Are there any other adolescent issues you wish your health care provider would

have discussed with you and/or your child?
A. He will respond but not initiate. In retrospect, I feel his office should be

more pro-active in dealing with ongoing issues.

B. Not that I can think of. I am pretty assertive about asking about things I am

concerned about.

C. Many of the above questions, "esp” (especially) about sex and sex
education.
D. Would have been nice to have "recd" (received) a pamphlet or booklet

around the time of menstrual onset directly related to a girl with "DS" (Down
syndrome) and how to explain menstruation and how to take care of it. Also

booklet on birth control and self-esteem.

E. No.

F. No.
G. Haven't thought about it.

H. No.
31. Are there any adolescent issues you do NOT wish your health care provider to

discuss with you and/or your child?
A. The mother is a nurse-midwife. Everything is discussible.
B. No.

C. No.
D. No.
E. No.

F. No.

G.No.
Additional comments included on returned surveys:

A. I completed this survey using my experience with my family practice
"md’s" (medical doctors). Being in the medical field, my husband & I use our
own knowledge to assist our family. We are able to use specialists as needed

due to our "free choice" medical insurance (even though we have high

deductibles). My son has had x-rays for his neck only because I insisted on it.

He has his thyroid checked only due to my asking for it.
B. I sincerely hope that you, and others like you, can improve the health care
provided to Down syndrome children. So many doctors and nurses are totally

92
ignorant of the special concerns of a Down syndrome child. It took me many
years to educate myself about these issues.