Adolescent Health Care: A Practical Guide

Chapter 82

Chronic Illness in the Adolescent

Susan M. Coupey

With the extraordinary technological advances in medicine that have occurred in the last few decades, the prevalence of chronic conditions in adolescents has increased dramatically. Many more children with conditions such as congenital heart disease, renal failure, cystic fibrosis, and even congenital human immunodeficiency virus (HIV) infection, survive into their teens and beyond (Table 82.1). Despite the availability of excellent biomedical treatment for most common chronic diseases, optimal management of illness in adolescents is not limited to biomedical prescription alone. Developmental, psychosocial, and family factors all feature prominently in the ongoing care of adolescents with chronic diseases. Chronic conditions, by definition, have no cure and therefore must be endured and managed on a daily basis. Having such a condition is a continuing source of stress for the adolescent and his or her family and can contribute to maladaptive coping and dysfunction. On the other hand, the experience of successfully managing a chronic illness also may foster accelerated maturation and coping skills in adolescents and their families. Helping adolescents manage the chronic illness well and reach their full developmental potential can be very rewarding for the clinician. This chapter emphasizes developmental and behavioral issues in management of chronically ill or hospitalized adolescents. It is beyond the scope of this book to discuss medical management of all the specific chronic conditions that can affect adolescents. The references at the end of this chapter address a number of the major chronic illnesses in this age-group.

TABLE 82.1
Prevalence of Selected Chronic Conditions in Adolescents Aged 10–17 Years

Condition

Cases per 1,000 U.S. Adolescents

From Westbrook LE, Stein RE. Epidemiology of chronic health conditions in adolescents. Adolesc Med 1994;5:197–209, with permission. Based on Newacheck PW, McManus MA, Fox HB. Prevalence and impact of chronic illness among adolescents. Am J Dis Child 1991;145:1367–1373.

Impairments

 

 Musculoskeletal impairments

20.9

 Speech defects

18.9

 Deafness and hearing loss

17.0

 Blindness and visual impairments

16.0

Diseases

 

 Asthma

46.8

 Heart disease

17.4

 Arthritis

 8.7

 Epilepsy and seizures

 3.3

 Diabetes mellitus

 1.5

 Sickle cell disease

 0.9

Definition and Prevalence

Stein et al. (1993; 1997) developed a noncategorical, or generic, approach to defining and measuring chronic health conditions in children, rather than using a list of diagnoses. Three definitional concepts provide the framework, all of which must be present for a child or adolescent to be classified as having a chronic health condition:

  1. Disorder on a biological, psychological, or cognitive basis
  2. Duration or expected duration of at least 12 months
  3. Consequences of the disorder:
  4. Functional limitations compared with healthy peers in the same age-group
  5. Reliance on compensatory mechanisms or assistance, such as medications, special diet, medical technology, assisting device, or personal assistance
  6. Need for medical care or related services, psychological services, or educational services over and above the usual for the child's age

Applying this definition and using data from the 1994 National Health Interview Survey Disability Supplement for Children aged 0 through 17 years, Stein and Silver (1999) estimated that 10.3 million children (14.8%) had chronic conditions. In 2001, the Maternal and Child Health Bureau of the National Institutes of Health (NIH) conducted the National Survey of Children with Special Health Care Needs

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using a different but similar definition of “children with special health care needs,” namely:

“…those who have or are at increased risk for a chronic physical, developmental, behavioral, or emotional condition and who also require health and related services of a type or amount beyond that required by children generally.”

The 2001 survey found that 12.8% of children younger than 18 years or approximately 9.4 million children in the United States had special health care needs. Both sets of analyses found that children with chronic health conditions or special health care needs were disproportionately older and male. The 2001 survey found that among adolescents aged 12 through 17, 15.8% have special health care needs. Asthma and other chronic respiratory tract conditions and musculoskeletal disorders account for most of the physical disabilities. Mental health disorders, including developmental, behavioral, and emotional problems, also are a leading cause of disability in this age-group.

Interaction of Adolescent Development and Chronic Illness

Early adolescence is a period of accelerated physical growth and pubertal development, and in middle and later adolescence, acceleration in cognitive and psychosocial development predominates. The interaction of chronic illness with these different developmental streams is complex and bidirectional; the illness may affect the development and/or the development may affect the illness. For example, some chronic diseases such as cystic fibrosis or sickle cell disease can cause delayed puberty, but for other chronic diseases such as diabetes mellitus, normal puberty can cause exacerbation of the disease. Similarly, disabling chronic conditions that impede peer interaction such as spina bifida can delay psychosocial development; conversely, normal psychosocial development that includes increasing independence from parents with experimentation and risk taking can lead to poor medication adherence and exacerbation of illnesses such as asthma or chronic renal failure.

Specific psychosocial areas, notably achieving independence and family relationships, have been found to be most vulnerable to dysfunction in adolescents with chronic health conditions. Wolman et al. (1994) examined emotional health in adolescents with and without a chronic illness. They found that although adolescents with chronic conditions do less well than adolescents without chronic conditions, the illness is not the most influential factor in emotional well-being. Family connectedness is of fundamental importance for adolescents' emotional well-being. Bennett (1994) reviewed 60 studies of depressive symptoms among children and adolescents with chronic medical problems. Although the findings indicate that children and adolescents with a chronic medical problem are at slightly elevated risk of having depressive symptoms, most were not clinically depressed. Mintzer et al. (2005) looked at post-traumatic stress disorder (PTSD) in >100 adolescents who had an organ transplantation (kidney, liver, or heart) a mean of 7 years previously and found that, although the majority (70%) had no symptoms, 16% met criteria for a diagnosis of PTSD. The type of transplant did not predict PTSD but adolescents with acute onset of organ failure precipitating the need for transplant were more likely to have PTSD than those having transplantations resulting from chronic or congenital conditions. Most studies indicate that the presence of a chronic physical illness, even with its attendant problems and stresses, does not necessarily lead to emotional dysfunction. Adolescents with a chronic condition, who are also disabled, however, are at increased risk for psychosocial and emotional dysfunction.

Another approach put forward by Rutter (1985) and reiterated by Patterson and Blum (1996) is to evaluate the total burden of risk and protective factors borne by each adolescent and to view chronic physical illness as one of several important risk factors for psychosocial and emotional dysfunction. Risk factors other than chronic illness include the following:

  • Male sex
  • Psychiatric illness and/or criminality in a parent
  • Severe family discord
  • Low socioeconomic status

Protective factors include the following:

  • Positive temperament
  • Above-average intelligence
  • Social competence
  • A supportive relationship with at least one parent
  • Family closeness
  • Adequate rule setting

Using this approach, clinicians can target adolescents with multiple risk factors, in addition to the chronic illness, for enhanced intervention to prevent dysfunction.

Specific Developmental Risks

Independence–Dependence

The medical requirements of managing a chronic illness and/or the perception of vulnerability of the ill adolescent can impede the movement toward independence that usually occurs during early and middle adolescence. Because chronic illness may prolong dependence on parents and others, including physicians, the adolescent may become compliant and child-like or noncompliant and rebellious.

Body Image

As stressed in earlier chapters, adolescents are highly concerned with and are self-conscious about their developing bodies. Delayed puberty or visible markers of the illness may interfere with the development of a healthy body image. This abnormal body image may lead to the following:

  • Lowered self-image and self-esteem
  • Segregation from peers
  • Increased absences from school and other activities
  • Increased anxieties over sexuality, sexual attractiveness, sexual function, and sexual relations
  • Disordered eating or eating disorders
  • Depression, anger, or both

Peer Group

Chronic illness may limit teenagers' activities, not only because of physical, mental, or sensory disabilities but also even without overt disability, because of illness-related fatigue, frequent medical appointments,

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and hospitalizations. Chronically ill adolescents may be rejected by peers or have fantasies of such rejection. These problems may lead to fear of peer involvement and social isolation.

Identity

The adolescent with a chronic illness often has difficulties consolidating a mature identity. Concerns about future vocation, financial resources, separation from parents, marriage, and reproduction may all lead to identity problems.

Modifying Factors

Age at Onset

The stage of development during which the chronic illness appears may have considerable bearing on the psychological impact of the illness on the adolescent.

  1. Preadolescence: Chronic illness or disability that originates at birth or in early childhood may lead early on to altered parental expectations. Lowered parentally perceived potential of the developing adolescent may foster reduced self-expectations in the teen. These misconceptions have major implications for the setting and achievement of future goals for the adolescent.
  2. Early adolescence: Because the early adolescent has yet to separate from his or her parents, there may be little struggle for independence or the parents may become overprotective and resist normal and reasonable independence overtures by their child. Chronic illness diagnosed in early adolescence may be particularly likely to provoke deep concerns about body integrity and body image. Such concerns can set the stage for dysfunctional coping, and if not resolved, eating disorders may develop later on. Gross et al. (2000) assessed the prevalence of eating disorder symptoms in a sample of young women with physical disabilities and found that 8% had a sufficient number of symptoms to suggest clinical disorder. Neumark-Sztainer et al. (1998) in a survey of a representative population of high school students in Connecticut, found that prevalence rates of disordered eating (using laxatives, diet pills, or vomiting to control weight) were twice as likely to be reported by boys with chronic illness than healthy boys and 1.6 times more likely to be reported by girls with chronic illness than healthy girls. Eating disorders are also reported with some frequency in adolescents with diabetes mellitus and inflammatory bowel disease, diseases that often have their onset in early adolescence.
  3. Middle adolescence: This age may be the most devastating time for a chronic illness to strike. During this phase, the adolescent is intensely involved with separation, peers, and sexual development. A chronic illness may thwart the adolescent's progress in these areas, in addition to conflicting with the high energy levels and feelings of omnipotence typical of middle adolescence. Poor adherence to medical therapy is a frequent problem during this period, as is depression, sexual acting out, and substance use.
  4. Late adolescence: Chronic illness with an onset in late adolescence usually causes less upheaval. At this stage, the teenager should have already gained self-confidence and a secure identity. Concerns are focused on how the disease may disrupt vocational and educational plans, as well as future serious relationships, parenthood, and the prospects for living independently.

Nature of the Illness

Perrin et al. (1993) list 12 dimensions, other than age at onset, for describing chronic illness that potentially may have an impact on the adolescent's adjustment:

1.  Duration

2.  Cognition

3.  Limitation of age-appropriate activities

4.  Emotional/social

5.  Visibility

6.  Sensory functioning

7.  Expected survival

8.  Communication

9.  Mobility

10.      Course

11.      Physiological functioning

12.      Uncertainty

For example, a highly visible disease such as psoriasis may cause more emotional disruption than a life-threatening malignancy such as Hodgkin disease. However, as is true for all of these dimensions, there is a complex relationship between visible deformities and adjustment. Sheerin et al. (1995) evaluated psychosocial adjustment in children with facial port-wine stains compared with adjustment in children with prominent ears. The children with disfiguring facial birthmarks were found to be as well as or better adjusted than nondisfigured peers, whereas those with prominent ears were less well adjusted. To explain this counterintuitive finding, the authors suggest that having a clearly abnormal disfigurement, such as a port-wine stain, elicits uniformity of both opinion and support from family, whereas having a “deformity” such as prominent ears, which is merely an exaggeration of normal, is not as likely to elicit support and may explain the poorer mental health in that marginally disfigured group.

An exacerbating and remitting course of illness with the accompanying uncertainty and lack of control over when the symptoms will strike, seems to be more likely to be associated with emotional problems. In reviewing studies among children and adolescents with chronic medical problems, Bennett (1994) found that certain disorders with these characteristics (i.e., asthma, recurrent abdominal pain, and sickle cell anemia) are associated with a greater risk for depression than other disorders that are more predictable (i.e., cancer, cystic fibrosis, and diabetes mellitus). Ireys et al. (1994) studied young adults (mean age, 21.9 years) with chronic illness and found that hearing and speech problems, unpredictability of symptoms, and restricted activity days were illness characteristics that had significant adverse effects on mental health status. Diseases that interfere with cognition, sensory function, or communication are particularly likely to be associated with poor adjustment. Howe et al. (1993) found that adolescents with brain-based conditions had more behavioral problems, less autonomous functioning, and poorer school achievement than those without brain dysfunction.

Coping with Chronic Illness

To cope with difficulties and frustrations of chronic illness, the adolescent usually adopts one or more of the following coping mechanisms:

  • Insightful acceptance: Unusual in adolescents, particularly during early and middle adolescence.
  • Denial: A common coping strategy used by adolescents. Some forms of denial may be adaptive and helpful, but more often, this strategy leads to poor adherence

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behaviors such as missed appointments and forgotten medications.

  • Regression: Also common in chronically ill adolescents. With regression, the teenager becomes abnormally dependent on parents and other adults and exhibits inappropriate child-like behavior.
  • Projection: This coping mechanism allows feelings of rage, frustration, or guilt to be transferred onto parents or health care providers. Projection is often observed on adolescent inpatient units, where anger is transferred to members of the staff.
  • Displacement: A similar coping mechanism to projection, but the anger is usually transferred to an object or an activity. Displacement is observed frequently on adolescent units and is typified by behavior such as throwing of objects.
  • Acting out: Similar to displacement but less constructive. In frustration, the adolescent exhibits out-of-control behavior, necessitating disciplinary management.
  • Compensation: A highly useful coping strategy in which the patient alters usual activities in response to the restrictions of the disease. For example, an adolescent who formerly achieved self-esteem through dancing switches to performing music for fulfillment.
  • Intellectualization: This coping mechanism separates the realities of the disease from the emotional impact. Teenagers who become highly involved in the technical aspects of their disease use intellectualization. Although intellectualization can be positive, there needs to be some period during which the adolescent acknowledges his or her emotional concerns.

Over time, the adolescent with a chronic illness or disability likely uses many of the listed coping mechanisms, often using different mechanisms to cope with different situations. Most adolescents who come from psychologically healthy and supportive families can cope amazingly well with a multitude of stressors and are even able to use these situations as emotional growth experiences. It is not uncommon to observe regressed behavior in an adolescent during a period of acute distress, with a rapid regaining of maturational status almost immediately afterward (e.g., when a bone marrow aspiration needle is removed).

With maturation and experience in dealing with chronic illness and its treatment, many adolescents are able to use cognitive reframing of situations to render them less threatening and stressful. Such adolescents also develop an array of coping strategies that have worked for them and learn to reuse those successful strategies instead of continuing to try unsuccessful ones. Exposure to multiple stresses of a similar nature permits stress inoculation and the ability to respond more rapidly and often more appropriately to familiar challenges.

For those adolescents who cannot consistently meet the challenges of chronic illness, breakdown of coping may be manifested behaviorally by poor adherence to treatment recommendations, increased risk-taking behaviors, or overall withdrawal from developmental tasks as a manifestation of depression.

Risk Behaviors

Risk taking, particularly in the areas of sexuality and substance use, contributes substantially to morbidity in healthy adolescents. When this behavior interacts with chronic illness, however, negative health consequences are often exacerbated. It is ironic that risk taking among chronically ill teenagers is probably directly related to improved management, which most often results in normal or near-normal growth, pubertal development, and fertility; better nutritional status; normal energy levels; and age-appropriate opportunities for social interaction that can, and often do, include risk-taking behaviors.

Sexuality

For adolescents with a chronic illness, the health risks of sexual activity may be exacerbated by the illness itself, the medications used to treat it, or by a maladaptive emotional response to illness. However, studies show that many adolescents with chronic illness and disability are sexually active and most have concerns about their sexual attractiveness, normalcy of their reproductive system and sexual response, fertility, safety of contraception use, and genetic aspects of their disease.

TABLE 82.2
Prevalence of Sexual Activity among Chronically Ill Adolescents

Study Population

Sample Size

Age Range (yr)

Sexually Active (%)

a Suris J, Resnick MD, Cassuto N, et al. Sexual behavior of adolescents with chronic disease and disability. J Adolesc Health1996;19:124–131.
b Alderman EM, Lauby JL, Coupey SM. Problem behaviors in inner-city adolescents with chronic illness. J Dev Behav Pediatr1995;16:339–344.
c Britto MT, Garrett JM, Dugliss MAJ, et al. Risky behavior in teens with cystic fibrosis or sickle cell disease: a multicenter study.Pediatrics 1998;101:250–256.

School-based, Minnesota, various diseasesa

     

 Males—visible conditions

148

13–18

49

 Females—visible conditions

308

13–18

42

Inner-city, New York, various diseasesb

217

14–17

33

Tertiary care centers, North Carolina, sickle cell diseasec

205

12–19

51

Tertiary care centers, North Carolina, cystic fibrosisc

116

12–19

28

  1. Prevalence of sexual activity: Table 82.2 shows prevalence of sexual activity among chronically ill adolescents.

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Suris et al. (1996) conducted a statewide survey of Minnesota high school students and showed that for both boys and girls, there was no difference in age at first intercourse between those with chronic illness and those without, and no effect of visibility of illness on age at first intercourse. Cheng and Udry (2002) in an analysis of physically disabled adolescents (mainly with musculoskeletal disorders) interviewed in the National Longitudinal Study of Adolescent Health found that by age 16, physically disabled teens were at least as sexually active as their nondisabled peers with 39% and 42% of severely disabled boys and girls, respectively, reporting sexual intercourse versus 37% and 34% of nondisabled boys and girls, respectively. In a study of inner-city adolescents with various chronic diseases (e.g., asthma, diabetes mellitus, cancer, sickle cell disease, and others) and their healthy friends aged 14 through 17 years, Alderman and Lauby (1995) found that approximately one third of both the chronically ill group and the healthy group were sexually active, with a mean age at first intercourse in the 13th year. However, these investigators also found that the interaction between gender and sexual debut was significantly different; boys without chronic illness initiated intercourse at a younger age (12.9 years) than boys with illness (13.4 years), and the reverse was true for girls, those with chronic illness initiated intercourse at a younger age (13.8 years) than their healthy friends (15.3 years). Britto et al. (1998) surveyed adolescents with cystic fibrosis and sickle cell disease from five pediatric tertiary care centers in North Carolina and compared their sexual behavior with that of controls matched for age, race, and gender. Fewer teens with cystic fibrosis (28%) and sickle cell disease (51%) than matched controls (46% and 76%, respectively) reported sexual activity. Also, the age at first intercourse for girls was older in those with chronic illness compared with matched controls, 15.7 versus 14.6 years and 14.8 versus 13.9 years for those with cystic fibrosis and sickle cell disease, respectively. The same was true for boys, only the ages at first intercourse were approximately 1 year younger in all groups.

As the study by Britto et al. shows, sexual debut may be delayed in adolescents with illnesses that delay puberty such as cystic fibrosis and sickle cell disease. Onset of sexual activity may also be delayed for adolescents with mobility limitations and brain-based conditions that result in fewer opportunities for peer interaction. Sexual assault is also a salient issue, particularly for cognitively impaired adolescent girls who are uniquely vulnerable to exploitation.

  1. Fertility: Some chronic conditions are associated with impaired fertility related to the disease itself or to the treatment. For example, males with cystic fibrosis are infertile, but females are fertile and many have children. In a large study of survivors of childhood cancer, Byrne et al. (1987) demonstrated that compared with sibling controls, radiation therapy below the diaphragm had the greatest adverse effect on fertility in females. It is important to remember, however, that most chronic illnesses do not impair fertility, and particularly for sexually active girls, effective contraception is extremely important. Girls with chronic illness should be counseled about the necessity of carefully planning pregnancies to minimize teratogenicity from medications and treatments and to ensure the best outcome for mother and fetus. Chapter 42 reviews contraceptive options in teens with various chronic illnesses and Table 82.3 outlines contraceptive options for selected chronic diseases.
  2. Sexually transmitted diseases: Girls who are immunosuppressed either due to the disease (e.g., HIV) or the treatment (e.g., organ transplant, lupus) are at risk for a prolonged and more complicated course if infected with a sexually transmitted disease. These girls should receive detailed instruction and frequent reinforcement regarding barrier contraceptive use. Genital infection with human papillomavirus (HPV) is particularly virulent in girls who are immunocompromised, and they are at increased risk of developing cervical cancer. In the future, with widespread use of HPV vaccines, this risk may be considerably mitigated.

Substance Use

Substance use by adolescents with chronic illness can contribute significantly to morbidity and mortality. Alcohol, illicit drugs, or over-the-counter herbal preparations may interact with prescribed medications to cause deleterious effects. Certain specific chronic conditions, notably heart disease, put affected adolescents at particular risk of death from even one-time use of stimulants such as cocaine, amphetamines, or “Ecstasy” (methylenedioxymethamphetamine [MDMA]).

Prevalence of Substance Use

Westbrook et al. (1991) compared substance use in three different groups of adolescents, aged 13 to 19 years. The groups included 34 patients with idiopathic epilepsy, 32 with various chronic diseases other than epilepsy, and 50 with no chronic illness. Fewer adolescents with epilepsy were daily smokers (9%) than adolescents who had other chronic diseases (19%) or were healthy (30%). Approximately 15% of the teens in each group reported drinking alcohol once a week or more, and 20% in each group reported that they used marijuana, with less than half using it once a week or more. The study by Britto et al. (1998) in North Carolina also found that fewer teens with cystic fibrosis versus matched controls smoked regularly (2.6% versus 29.6%), were binge drinkers (18.0% versus 35.3%), or tried marijuana (9.7% versus 29.4%), respectively. Tyc et al. (2006) reviewed smoking rates in children and adolescents with chronic illness. They reported that some of these adolescents had smoking rates at least comparable to those of their healthy peers. The article also reviewed suggested interventions for smoking cessation in youth with a chronic illness. Although the proportion of adolescents with chronic illness using substances may be less than that of their healthy peers, the absolute numbers are still quite high, and the risk for health complications are greater. Screening by history for substance use, and anticipatory guidance and counseling regarding specific substances and their effect on the adolescent's illness are mandatory.

Management of Chronic Illness in the Adolescent

General Principles

Optimal care of the adolescent's medical condition is of primary importance. However, high-quality treatment often

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cannot be achieved without consideration and exploration of the adolescent's mental health, developmental progress, and family relationships. Simply prescribing treatment is not enough. The adolescent must cooperate with the health care team, believe that adhering to a complex regimen is better than the alternative, and have family support and assistance in carrying out the treatment plan. Helpful principles for management include the following:

TABLE 82.3
Choice of Contraceptives for Girls with Selected Chronic Conditions

Condition

Special Considerations

Suggested Contraceptives

OCP, oral contraceptive pill; DMPA, depot medroxyprogesterone acetate; IUD, intrauterine device; HPV, human papillomavirus; STD, sexually transmitted disease.

Diabetes mellitus

Low-dose OCPs do not affect glucose tolerance. Estrogen-dominant pills promote a favorable lipid profile. Obesity is often a problem in type 2 diabetes.

Second- or third-generation combination OCPs, patch, or ring plus male latex condom. Avoid DMPA in girls with type 2 diabetes.

Heart disease

OCPs, patch, and ring contraindicated with pulmonary vascular disease, thromboembolic disease, and cerebrovascular disease.

Progestin-only injectable or implantable methods plus male latex condom. Consider progestin-releasing IUD (Mirena). May use OCPs if no contraindications.

Immunosuppression

STDs, especially viral pathogens such as HPV, are more virulent and hard to treat.

Male latex condoms preferred. Consider female condom. May use hormonal methods as well if no contraindications.

Mental retardation

Compliance is an issue unless parent will administer pills. Barrier methods difficult to use.

Long-acting injectable or implantable methods are preferred.

Seizure disorder

OCPs, patch, and ring have lowered efficacy with some seizure medications but not with valproic acid.

DMPA or combination OCPs plus male latex condom. Progestin-only OCPs are contraindicated.

Sickle cell disease

Hormonal methods reduce frequency of vasoocclusive crises. OCPs, patch, and ring contraindicated with history of stroke.

DMPA with male latex condoms is preferred. OCPs, patch, or ring may be used if no contraindications.

Spina bifida

May have latex allergy. OCPs have lowered efficacy with some seizure medications. OCPs, patch, and ring may have higher thrombosis risk in immobilized patients. DMPA may exacerbate osteopenia in immobilized patients.

Polyurethane condoms (male or female) plus hormonal methods if no contraindications. DMPA is not advised for those who are wheelchair bound. Consider progestin-releasing IUD (Mirena–reduced uterine bleeding, no osteopenia).

  1. Educate: Inform the adolescent about the nature of the disease and the limitations of treatment. The educational process should be directed at both patient and family and should use language that is easy to understand. For illnesses diagnosed early in life, update adolescents as they become more cognitively mature and capable of understanding their condition better.
  2. Respond to emotion: Listen carefully, give permission, and allow time for the adolescent and the family to express feelings, fears, and hopes.
  3. Involve the family: Family support and guidance are crucial. Help families to allow their adolescents increased autonomy and independence over time. Families must be coached to avoid the “overs”—overprotection, overanxiety, and overattention.
  4. Involve the patient: The more an adolescent is involved with his or her own care, the greater the chance for improved compliance and a greater sense of self-determination.
  5. Use a multidisciplinary team: Many health care professionals are usually necessary to optimally manage adolescents with chronic illness, including physicians, psychologists, social workers, nurses, occupational therapists, physical therapists, and nutritionists. Respect for the skills of these individuals, as well as communication in interdisciplinary conferences, can improve the care of the adolescent.
  6. Provide continuity of care: The chronically ill adolescent needs an advocate whom he or she can trust. At least one member of the health care team, preferably a primary care clinician, should maintain an ongoing relationship with the patient and family. This clinician's role is to coordinate care among various specialty disciplines, monitor adolescent development, provide support and anticipatory guidance, and treat intercurrent illnesses.
  7. Provide comprehensive ambulatory services: Such services would include psychological, educational, speech and hearing, social work, and other special services. It may be difficult for families to obtain insurance reimbursement for such necessary services, although they are cost-effective. Liptak et al. (1998) showed that

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comprehensive ambulatory service decreased number of inpatient hospitalizations, length of stay, hospital costs, and readmissions for chronically ill children.

  1. Referral to peer and disease support groups: Participation in such groups allows for increased expression of concerns and anxieties by both patients and families and sharing of information among families with similar problems. A number of support group agencies are listed at the end of this chapter.
  2. Consider self-help techniques: Training the adolescent in various cognitive-behavioral techniques to manage stress and pain can enhance feelings of self-control and reduce disease- and treatment-related stress. Examples of these strategies include hypnosis, relaxation and distraction techniques, guided imagery, and thought stopping.
  3. Limit setting: If compliance or behavior is a problem, limits must be set and the entire health care team and the parents must agree and enforce the limits. Avoidance of splitting and pitting one member of the team against another is crucial.
  4. Inpatient care: When necessary, inpatient hospital care is best handled in an environment conducive to the developmental needs of the adolescent.

Health Care Transition from Pediatric to Adult Services for Young Adults with Chronic Illness

Approximately half a million youth with special health care needs will turn 18 each year in the United States and most will need to be transitioned into the adult health care system. An appropriately timed transition to adult-oriented health care allows youth to optimize their independence and to more successfully assume adult roles and functioning. Indeed, the Healthy People 2010 initiative has established a goal that “All youth with special health care needs will receive the services necessary to make transitions to all aspects of adult life, including adult health care, work, and independence.” A joint consensus statement issued in 2002 by leading pediatric and adult health care societies recommends that a written health care transition plan for adolescents with special health care needs be created by age 14 and that affordable, continuous health insurance coverage be ensured throughout adolescence and adulthood. However, there are several barriers to effecting a successful health care transition for chronically ill youth and their families. Such barriers include the following:

  • Lack of adequately trained or accessible adult-oriented physicians to manage chronic childhood conditions such as congenital heart disease or cystic fibrosis
  • Loss of health insurance coverage when the young adult is no longer covered on the parents' policy
  • Reluctance of patients, families, and pediatric subspecialists to terminate a long-standing relationship

The Society for Adolescent Medicine in its 2003 position paper outlined several principles of successful transition services. Such services should have the following features:

  • Be appropriate for both chronological age and developmental attainment
  • Address common concerns of young people such as growth and development, sexuality, mental health disorders, and substance use
  • Facilitate self-reliance and autonomy, and increase personal responsibility
  • Be flexible enough to meet the needs of a wide range of young people
  • Have a designated professional who, along with the patient and family, takes responsibility for the process

Scal and Ireland (2005) evaluated the adequacy of transition services in an analysis of parent reports from the 2001 National Survey of Children with Special Health Care Needs. These investigators found that overall, 50% of youth and parents had discussed transition issues with the adolescent's doctor but only one in six (16%) had developed a plan. The strongest predictors of the adequacy of health care transition services were a high-quality parent–provider interaction, a greater number of needed services, female gender, and older age. This study provides us with a first look at how we are doing as a nation in providing optimal care for our adolescents and young adults with chronic illness and indicates that we have much room left for improvement.

Resources

http://www.pacer.org/links/national/disability.htm. PACER Center (Parent Advocacy Coalition for Educational Rights): This site has up-to-date links and addresses for more than 160 specific disability resources including organizations and associations such as Autism Society of America, National Fragile X Foundation, American Diabetes Association, Cystic Fibrosis Foundation, March of Dimes Birth Defects Foundation, Candlelighters Childhood Cancer Foundation, and many others. Resources are listed for 14 different categories of disability including: Attention Deficit Disorder, Autism Spectrum Disorders, Cognitive and Developmental Disabilities, Deaf Blind, Deaf and Hard of Hearing, Emotional and Behavioral Disorders, Genetic Disorders, Health Impairments, Learning Disabilities, Neurological Disorders, Physical Disabilities, Speech or Language Impairments, Traumatic Brain Injury, and Visually Impaired.

Web Sites

http://www.nichcy.org. National Dissemination Center for Children with Disabilities. Information on disabilities in children and youth, special education, and research.

http://www.depts.washington.edu/healthtr/. Adolescent Health Transition Project at the Center on Human Development and Disability at the University of Washington, Seattle. This site contains extensive high quality information for health care providers and educators, parents and families, and teens and young adults.

http://www.hrtw.org. Healthy and Ready to Work National Center. This Center is part of the Division of Services for Children with Special Health Care Needs of the MCHB. It is filled with information for professionals, parents, and youth.

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http://www.youngwomenshealth.org. This site is sponsored by the Children's Hospital Boston. There is excellent information for girls, parents, and professionals on contraception and sexual health as well as on chronic gynecologic conditions including reproductive consequences for cancer survivors, poly cystic ovary syndrome, endometriosis, congenital anomalies of the female genital tract, and ovarian failure.

References and Additional Readings

Alderman EM. Reproductive health for girls with chronic illness. In: SM Coupey, ed. Primary care of adolescent girls. Philadelphia: Hanley & Belfus, 2000:127–135.

Alderman EM, Lauby JL, Coupey SM. Problem behaviors in inner-city adolescents with chronic illness. J Dev Behav Pediatr 1995;16:339.

American Academy of Pediatrics, American Academy of Family Physicians, American College of Physicians, American Society of Internal Medicine. A consensus statement on health care transitions for young adults with special health care needs. Pediatrics 2002;110:1304.

American Academy of Pediatrics, Committee on Children With Disabilities. General principles in the care of children and adolescents with genetic disorders and other chronic health conditions. Pediatrics 1997;99:643.

Bennett DS. Depression among children with chronic medical problems: a meta-analysis. J Pediatr Psychol 1994;19:149.

Blum RW. Sexual health contraceptive needs of adolescents with chronic conditions. Arch Pediatr Adolesc Med 1997; 151:290.

Britto MT, Garrett JM, Dugliss MAJ, et al. Risky behavior in teens with cystic fibrosis or sickle cell disease: a multicenter study. Pediatrics 1998;101:250.

Byrne J, Mulvihill JJ, Myers MH, et al. Effects of treatment on fertility in long-term survivors of childhood or adolescent cancer. N Engl J Med. 1987;317:1315.

Cheng MM, Udry JR. Sexual behaviors of physically disabled adolescents in the United States. J Adolesc Health 2002;31:48.

Desguin BW, Holt IJ, McCarthy SM. Comprehensive care of the child with a chronic condition. II. Primary care management. Curr Probl Pediatr 1994;24:230.

Freed GL, Hudson EJ. Transitioning children with chronic diseases to adult care: current knowledge, practices, and directions. J Pediatr 2006;148:824.

Garwick AE, Millar HEC. Promoting resilience in youth with chronic conditions and their families. Rockville, MD: U.S. Department of Health and Human Services, Maternal and Child Health Bureau, Health Resources and Service Administration, Public Health Service, April 1996.

Hauser ST, DiPlacido J, Jacobson AM, et al. Family coping with an adolescent's chronic illness: an approach and three studies. J Adolesc 1993;16:305.

Holden EW, Chmielewski D, Nelson CC, et al. Controlling for general and disease specific effects in child and family adjustment to chronic childhood illness. J Pediatr Psychol1997;22:15.

Howe GW, Feinstein C, Reiss D, et al. Adolescent Adjustment to chronic physical disorders. I. Comparing neurological and non-neurological conditions. J Child Psychol Psychiatry. 1993;34:1153.

Ireys HT, Werthamer-Larsson LA, Kolodner KB, et al. Mental health of young adults with chronic illness: the mediating effect of perceived impact. J Pediatr Psychol 1994;19:205.

Kyngas HA, Kroll T, Duffy ME. Compliance in adolescents with chronic diseases: a review. J Adolesc Health 2000;26:379.

Liptak GS, Burns CM, Davidson PW, et al. Effects of providing comprehensive ambulatory services to children with chronic conditions. Arch Pediatr Adolesc Med 1998;152:1003.

Maternal and Child Health Bureau. Achieving success for all children and youth with special health care needs: a 10-year action plan to accompany Healthy People 2010. Rockville, MD: Maternal and Child Health Bureau, 2001.

McPherson M, Arango P, Fox H, et al. A new definition of children with special health care needs. Pediatrics 1998; 102:137.

Mintzer LL, Stuber ML, Seacord D, et al. Traumatic stress symptoms in adolescent organ transplant recipients. Pediatrics 2005;115:1640.

National Survey of Children with Special Health Care Needs Chartbook 2001. Prevalence of children with special health care needs. http://mchb.hrsa.gov/chscn/pages/prevalence.htm. 2001.

Neal W, Coupey SM. Biopsychosocial aspects of chronic illness. Adolesc Med 1996;7:427.

Neumark-Sztainer D, Story M, Falkner NH, et al. Disordered eating among adolescents with chronic illness and disability. Arch Pediatr Adolesc Med 1998;152:871.

Newacheck PW, Strickland B, Shonkoff JP, et al. An epidemiologic profile of children with special health care needs. Pediatrics 1998;102:117.

Patterson J, Blum RW. Risk and resilience among children and youth with disabilities. Arch Pediatr Adolesc Med 1996;150: 692.

Perrin EC, Newacheck P, Pless IB, et al. Issues involved in the definition of chronic health conditions. Pediatrics. 1993;91:787.

Rosen DS, Blum RW, Britto M, et al. Transition to adult health care for adolescents and young adults with chronic conditions: position paper of the Society for Adolescent Medicine. J Adolesc Health 2003;33:309.

Rutter M. Resilience in the face of adversity: protective factors and resistance to psychiatric disorder. Br J Psychiatry 1985;147:598.

Saetermoe CL, Farruggia SP, Lopez C. Differential parental communication with adolescents who are disabled and their healthy siblings. J Adolesc Health 1999;24:427.

Scal P, Ireland M. Addressing transition to adult health care for adolescents with special health care needs. Pediatrics 2005;115:1607.

Sheerin D, MacLeod M, Kusumakar V. Psychosocial adjustment in children with port-wine stains and prominent ears. J Am Acad Child Adolesc Psychiatry 1995;34:1637.

Stein RE, Bauman LJ, Westbrook LE, et al. Framework for identifying children who have chronic conditions: the case for a new definition. J Pediatr 1993;122:342.

Stein RE, Silver EJ. Operationalizing a conceptually based noncategorical definition: a first look at U.S. children with chronic conditions. Arch Pediatr Adolesc Med 1999;153:68.

Stein RE, Westbrook LE, Bauman LJ. The questionnaire for identifying children with chronic conditions: a measure based on a noncategorical approach. Pediatrics 1997;99:513.

Stuber ML. Psychiatric sequelae in seriously ill children and their families. Psychiatr Clin North Am 1996;19:481.

Suris J, Resnick MD, Cassuto N, et al. Sexual behavior of adolescents with chronic disease and disability. J Adolesc Health 1996;19:124.

Tyc VL, Throckmorton-Belzer L. Smoking rates and the state of smoking interventions for children and adolescents with chronic illness. Pediatrics 2006;118:471.

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Valencia LS, Cromer BA. Sexual activity and other high risk behaviors in adolescents with chronic illness: a review. J Pediatr Adolesc Gynecol 2000;13:53.

Westbrook LE, Stein RE. Epidemiology of chronic health conditions in adolescents. Adolesc Med 1994;5:197.

Wolman C, Resnick MD, Harris LJ, et al. Emotional well-being among adolescents with and without chronic conditions. J Adolesc Health 1994;15:199.

References for Specific Conditions

Asthma

Angsten JM. Use of complementary and alternative medicine in the treatment of asthma. Adolesc Med 2000;11:535.

CDC. Self-reported asthma among high school students–United States, 2003. MMWR 2005;54:765.

Howenstine MS, Eigen H. Medical care of the adolescent with asthma. Adolesc Med 2000;11:501.

Morikawa A, Mochizuki H, Shigeta M, et al. Age-related changes in bronchial hyperreactivity during the adolescent period. J Asthma 1994;31:445.

Reznik M, Sharif I, Ozuah PO. Classifying asthma severity: prospective symptom diary or retrospective symptom recall? J Adolesc Health 2005;36:537.

Rodriguez MA, Winkleby MA, Ahn D, et al. Identification of population subgroups of children and adolescents with high asthma prevalence. Arch Pediatr Adolesc Med2002;156:269.

Vila G, Nollet-Clemencon C, Vera M, et al. Prevalence of DSM-IV disorders in children and adolescents with asthma versus diabetes. Can J Psychiatry 1999;44:562.

Zbikowski SM, Klesges RC, Robinson LA. Risk factors for smoking among adolescents with asthma. J Adolesc Health 2002;30:279.

Cancer

American Academy of Pediatrics. Guidelines for pediatric cancer centers. Pediatrics 2004;113:1833.

Bahadur G, Whelan J, Ralph D. Gaining consent to freeze spermatozoa from adolescents with cancer: legal, ethical, and practical aspects. Hum Reprod 2001;16:188.

Bassal M, Mertens AC, Taylor L, et al. Risk of selected subsequent carcinomas in survivors of childhood cancer: a report from the childhood cancer survivor study. J Clin Oncol2006;24:476.

Bleyer WA. Cancer in older adolescents and young adults: epidemiology, diagnosis, treatment, survival, and importance of clinical trials. Med Pediatr Oncol 2002;38:1.

Grant J, Cranston A, Horsman J, et al. Health status and health-related quality of life in adolescent survivors of cancer in childhood. J Adolesc Health 2006;38:504.

Green DM, Zevon MA, Reese PA, et al. Factors that influence the further survival of patients who survive for five years after the diagnosis of cancer in childhood or adolescence.Med Pediatr Oncol 1994;22:91.

Grundy R, Gosden RG, Hewitt M, et al. Fertility preservation for children treated for cancer: scientific advances and research dilemmas. Arch Dis Child 2001;84:355.

Hudson MM, Mertens AC, Yasui Y, et al. Health status of adult long-term survivors of childhood cancer. JAMA 2003; 290:1583.

Kazak AE. Evidence-based interventions for survivors of childhood cancer and their families. J Pediatr Psychol 2005; 30:29.

Klopfenstein KJ. Adolescents, cancer, and hospice. Adolesc Med 1999;10:437.

Nicholson HS, Byrne J. Fertility and pregnancy after treatment for cancer during childhood or adolescence. Cancer 1993;71:3392.

Sarafoglou K, Boulad F, Gillio A, et al. Gonadal function after bone marrow transplantation for acute leukemia during childhood. J Pediatr 1997;130:210.

Shankar S, Robison L, Jenney MEM, et al. Health-related quality of life in young survivors of childhood cancer using the Minneapolis-Manchester quality of life–youth form.Pediatrics 2005;115:435.

Von Essen L, Enskar K, Kreuger A, et al. Self-esteem, depression, and anxiety among Swedish children and adolescents on and of cancer treatment. Acta Pediatr 2000;89:229.

Wu X, Chen VW, Steele B, et al. Cancer incidence in adolescents and young adults in the United States, 1992–1997. J Adolesc Health 2003;32:405.

Cystic Fibrosis

Bartholomew LK, Parcel GS, Swank PR, et al. Measuring self-efficacy expectations for the self-management of cystic fibrosis. Chest 1993;103:1524.

Birnkrant DJ, Hen J, Stern RC. The adolescent with cystic fibrosis. Adolesc Med 1994;5:249.

Britto MT, Garrett JM, Konrad TR, et al. Comparison of physical activity in adolescents with cystic fibrosis versus age-matched controls. Pediatr Pulmonol 2000;30:86.

Graetz BW, Shute RH, Sawyer MG. An Australian study of adolescents with cystic fibrosis: perceived supportive and nonsupportive behaviors from families and friends and psychological adjustment. J Adolesc Health 2000;26:64.

Nasr SZ. Cystic fibrosis in adolescents and young adults. Adolesc Med 2000;11:589.

Sawyer SM, Rosier MJ, Phelan PD, et al. The self-image of adolescents with cystic fibrosis. J Adolesc Health 1995;16: 204.

Thompson RJ, Gustafson KE, George LK, et al. Change over a 12-month period in the psychological adjustment of children and adolescents with cystic fibrosis. J Pediatr Psychol1994; 19:189.

Zindani GN, Streetman DD, Streetman DS. Adherence to treatment in children and adolescent patients with cystic fibrosis. J Adolesc Health 2006;38:13.

Diabetes Mellitus

Daviss WB, Coon H, Whitehead P, et al. Predicting diabetic control form competence, adherence, adjustment, and psychopathology. J Am Acad Child Adolesc Psychiatry1995;34:1629.

Draznin MB, Patel DR. Diabetes mellitus and sports. Adolesc Med 1998;9:457.

Farrell SP, Hains AA, Davies WH, et al. The impact of cognitive distortions, stress, and adherence on metabolic control in youths with type 1 diabetes. J Adolesc Health2004;34:461.

Fagot-Campagna A, Pettitt DJ, Engelgau MM, et al. Type 2 diabetes among North American children and adolescents: an epidemiologic review and a public health perspective. J Pediatr 2000;136:664.

Hoare P, Mann H. Self-esteem and behavioral adjustment in children with epilepsy and children with diabetes. J Psychosom Res 1994;38:859.

Jacobson AM, Hauser ST, Lavori P, et al. Family environment and glycemic control: a four-year prospective study of children and adolescents with insulin-dependent diabetes mellitus. Psychosom Med 1994;56:401.

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Lawrence JM, Standiford DA, Loots B, et al. Prevalence and correlates of depressed mood among youth with diabetes: the SEARCH for diabetes in youth study. Pediatrics2006;117:1348.

Mellin AE, Neumark-Sztainer D, Patterson J. Unhealthy weight management behavior among adolescent girls with type 1 diabetes mellitus: the role of familial eating patterns and weight-related concerns. J Adolesc Health 2004;35:278.

Pettitt DD, Jones KL, Arslanian SA. Type 2 diabetes mellitus in minority children and adolescents: and emerging problem. Endocrinol Metab Clin North Am 1999;28:709.

Pollock M, Kovacs M, Charron-Prochownik D. Eating disorders and maladaptive dietary/insulin management among youths with childhood-onset insulin-dependent diabetes mellitus. J Am Acad Child Adolesc Psychiatry 1995;34:291.

Sperling MA. Diabetes in adolescence. Adolesc Med 1994;5:87.

Svoren BM, Butler D, Levine B, et al. Reducing acute adverse outcomes in youths with type 1 diabetes: a randomized controlled trial. Pediatrics 2003;112:914.

Weissberg-Benchell J, Glasgow AM, Tynan WD, et al. Adolescent diabetes management and mismanagement. Diabetes Care 1995;18:77.

Disabled Adolescent

Apple DF Jr, Anson CA, Hunter JD, et al. Spinal cord injury in youth. Clin Pediatr 1995;34:90.

Balogh R, Bretherton K, Whibley S, et al. Sexual abuse in children and adolescents with intellectual disability. J Intellect Disabil Res 2001;45:194.

Clark MW. The physically challenged athlete. Adolesc Med 1998; 9:491.

Greydanus DE, Rimsza ME, Newhouse PA. Adolescent sexuality and disability. Adolesc Med 2002;13:223.

Hallum A. Disability and the transition to adulthood: issues for the disabled child, the family, and the pediatrician. Curr Probl Pediatr 1995;25:12.

Langley JD, Stanton WR, McGee RO, et al. Disability in late adolescence. I. Introduction, methods, and overview. Disabil Rehabil 1995;17:35.

Saetermoe CL, Farruggia SP, Lopez C. Differential parental communication with adolescents who are disabled and their healthy siblings. J Adolesc Health 1999;24:427.

Taniguchi MH, Schlosser GA. Adolescent spinal cord injury: considerations for post-acute management. Adolesc Med 1994;5:327.

Epilepsy

Hoare P, Mann H. Self-esteem and behavioral adjustment in children with epilepsy and children with diabetes. J Psychosom Res 1994;38:859.

Raty LKA, Larsson BMW, Soderfeldt BA, et al. Health-related quality of life in youth: a comparison between adolescents and young adults with uncomplicated epilepsy and healthy controls. J Adolesc Health 2003;33:252.

Westbrook LE, Silver EJ, Coupey SM, et al. Social characteristics of adolescents with idiopathic epilepsy: a comparison to chronically ill and non-chronically ill peers. J Epilepsy1991; 4:87.

Heart Disease

Cetta F, Graham LC, Lichtenberg RC. Piercing and tattooing in patients with congenital heart disease: patient and physician perspectives. J Adolesc Health 1999;24:160.

Doroshow RW. The adolescent with simple or corrected congenital heart disease. Adolesc Med 2001;12:1.

Gillum RF. Epidemiology of congenital heart disease in the United States. Am Heart J 1994;127:919.

Mendelson MA. Gynecologic and obstetric issues in the adolescent with heart disease. Adolesc Med 2001;12:163.

Inflammatory Bowel Disease

Engstrom I. Inflammatory bowel disease in children and adolescents: mental health and family functioning. J Pediatr Gastroenterol Nutr 1999;28:S28.

Griffiths AM. Inflammatory bowel disease. Adolesc Med 1995;6:351.

Lindberg E, Lindquist B, Holmquist L, et al. Inflammatory bowel disease in children and adolescents in Sweden, 1984–1995. J Pediatr Gastroenterol Nutr 2000;30:259.

Renal Disease

Cameron JS. Lupus nephritis in childhood and adolescence. Pediatr Nephrol 1994;8:230.

Furth SL, Hwang W, Yang C, et al. Relation between pediatric experience and treatment recommendations for children and adolescents with kidney failure. JAMA 2001;285:1027.

Hogg RJ, Furth S, Lemley KV, et al. National kidney foundation's kidney disease outcomes quality initiative clinical practice guidelines for chronic kidney disease in children and adolescents: evaluation, classification, and stratification. Pediatrics 2003;111:1416.

Manifica S, Dazord A, Cochat P, et al. Quality of life of children and adolescents after kidney or liver transplantation: child, parents and caregiver's point of view. Pediatr Transplant 2003;7:228.

Watson AR, Shooter M. Transitioning adolescents from pediatric to adult dialysis units. Adv Perit Dial 1996;12:176.

Rheumatological Conditions

Adam V, St-Pierre Y, Fautrel B, et al. What is the impact of adolescent arthritis and rheumatism? Evidence from a national sample of Canadians. J Rheumatol 2005;32:354.

Bentas W, Karch H, Huppertz HI. Lyme arthritis in children and adolescents: outcome 12 months after initiation of antibiotic therapy. J Rheumatol 2000;27:2025.

Britto MT, Rosenthal SL, Taylor J, et al. Improving rheumatologists' screening for alcohol use and sexual activity. Arch Pediatr Adolesc Med 2000;154:478.

Cuneo KM, Schiaffino KM. Adolescent self-perceptions of adjustment to childhood arthritis: the influence of disease activity, family resources, and parent adjustment. J Adolesc Health 2002;31:363.

Hollister JR. Medical treatment of adolescents with rheumatic disease. Adolesc Med 1998;9:163.

Sathananthan R, David J. The adolescent with rheumatic disease. Arch Dis Child 1997;77:355.

Shaller J. Diagnosis and management of rheumatic diseases in adolescence. Adolesc Med 1998;9:1.

Silva CA, Hallak J, Pasqualotto FF, et al. Gonadal function in male adolescents and young males with juvenile onset systemic lupus erythematosus. J Rheumatol 2002;29:2000.

White PH. Psychosocial aspects of rheumatic disease in childhood and adolescence. Adolesc Med 1998;9:171.

White PH. Transition to adulthood. Curr Opin Rheumatol 1999; 11:408.

Sickle Cell Anemia

Athale UH, Chintu C. The effect of sickle cell anaemia on adolescents and their growth and development: lessons from the sickle cell anaemia clinic. J Trop Pediatr 1994;40:246.

Britto MT, Garrett JM, Dugliss MA, et al. Preventive services received by adolescents with cystic fibrosis and sickle cell disease. Arch Pediatr Adolesc Med 1999;153:27.

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Kell RS, Kliewer W, Erickson MT. Psychological adjustment of adolescents with sickle cell disease: relations with demographic, medical, and family competence variables. J Pediatr Psychol 1998;23:301.

Lemanek KL, Steiner SM, Grossman NJ. Too little, too late: primary vs. secondary interventions for adolescents with sickle cell disease. Adolesc Med 1999;10:385.

Mantadakis E, Cavender D, Joe RN. Prevalence of priapism in children and adolescents with sickle cell anemia. J Pediatr Hem Oncol 1999;21:518.

Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood 1998;91:288.

Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease–life expectancy and risk factors for early death. N Engl J Med 1994;330:1639.

Yang YM, Cepeda M, Price C, et al. Depression in children and adolescents with sickle-cell disease. Arch Pediatr Adolesc Med 1994;148:457.

Spina Bifida

Appleton PL, Minchom PE, Ellis NC, et al. The self-concept of young people with spina bifida: a population-based study. Dev Med Child Neurol 1994;36:198.

Ammerman RT, Kane VR, Slomka GT, et al. Psychiatric symptomatology and family functioning in children and adolescents with spina bifida. J Clin Psychol Med Settings 1998; 5:449.

Blum RW, Resnick M, Nelson R, et al. Family and peer issues among adolescents with spina bifida and cerebral palsy. Pediatrics 1991;88:280.

Coakley RM, Holmbeck GN, Friedman D, et al. A longitudinal study of pubertal timing, parent-child conflict, and cohesion in families of young adolescents with spina bifida. J Pediatr Psychol 2002;27:461.

Gross SM, Ireys HT, Kinsman SL. Young women with physical disabilities: risk factors for symptoms of eating disorders. J Dev Behav Pediatr 2000;21:87.

McDonnell GV, McCann JP. Issues of medical management in adults with spina bifida. Childs Nerv Syst 2000;16:222.

Sandler AD, Worley G, Leroy EC, et al. Sexual knowledge and experience among young men with spina bifida. Eur J Pediatr Surg 1994;4(Suppl 1):36.

Silber TJ, Sheer C. Eating disorders in adolescent girls with spina bifida. J Adolescent Health 1996;18:139.

Wolman C, Basco DE. Factors influencing self-esteem and self-consciousness in adolescents with spina bifida. J Adolesc Health 1994;15:543.