The Scope of the Challenge

There are multiple dimensions to the effects of chronic illness on children and members of their families. The scope of the challenge of managing children and youth with rheumatic diseases goes far beyond medications and therapy. Several early studies suggested that chronic illness has a negative impact on psychosocial development, family, school life, and family finances. Studies from Sweden and the United States that focused on children with juvenile idiopathic arthritis (JIA) growing up before the availability of modern drugs showed that 30% to 50% entered adult life with active disease ; it is likely that the percentage is even higher in developing countries. Severe disability was common in this young adult population, with decreased physical functions, poor health perception, and pain. All of the psychosocial and physical problems assume greater importance when children reach adolescence or adulthood with continuing disease activity.

The current approach to early treatment of JIA, with immune modulators and intraarticular glucocorticoids, has resulted in reduced incidence of severe deformities. However, in one recent study conducted after the introduction of the current therapies, persistent disease activity and impaired functioning were still seen frequently. With the advent of newer, more efficacious therapies, achieving inactive disease and remission is a realistic goal for a significant proportion of children with JIA, and for increasing numbers of children with other rheumatic diseases (systemic lupus erythematosus [SLE], juvenile dermatomyositis, the vasculitides, etc.)

The early observations of poor outcome for children with rheumatic diseases have not been substantiated in recent studies. Even studies that emphasized the negative impacts of growing up with JIA showed that many of these patients completed college, worked full time, and raised children. Recent well-designed studies seem to indicate that JIA is not necessarily a psychosocial stressor, and families of children with JIA are, in general, resilient. Patients with JIA growing into early adulthood were shown to be able to earn a living, live independently, and have a stable spousal relationship, although patients with active disease had poor health-related quality of life in the physical component of the assessment tool. Some children do suffer disabilities, have reduced function and decreased social acceptance, and have overall adjustment problems and internalization of symptoms. For all of these reasons, a program of care for children with rheumatic diseases should be planned for the whole child and the future and should be comprehensive (i.e., family-centered, community-based, coordinated, and cost-effective) ( Boxes 11-1 , 11-2 ).

Current research suggests that planning for care of these children should be based on new concepts of disablement, should be evidence-based, and should be individualized. New concepts of the “disablement” process include four distinct constructs: (1) active pathology, (2) impairment, (3) functional limitation, and (4) disability. Each of these stages offers potential for intervention. In addition, there may be other factors, such as coping skills, access to care, economics, comorbidities, and the family’s psychosocial climate, that contribute to the disablement process and therefore are appropriate targets for intervention. A tailored program individualized for each patient, considering his or her environment, is thus essential to optimize the efforts of the team providing care and the family. The newer International Classification of Functioning, Disability and Health (ICF) recognizes external factors (physical, social, and attitudinal) that have an impact on disability and impairment. Newer tools for the evaluation of disability include items to measure body function and structure, activities and participation, and environmental and personal factors. Based on this conceptual framework, tools have been developed for use in specific conditions such as rheumatoid arthritis and osteoarthritis and for use in children and youth (ICF-CY).

Based on their well-designed study, Noll and colleagues suggested that “randomly occurring, challenging life events do not alter the child’s potential for inclusive fitness by denigrating their social status or emotional well-being.” It is important to know why some patients and families are vulnerable to functional and psychosocial disabilities and others are not. One concept to explain differences in vulnerability focuses on risk factors that tend to push children with chronic illness and their families to dysfunction and disability, and resilience factors that tend to give them more stability. The relative predominance of risk factors or resilience factors influences outcomes ( Table 11-2 ).

In planning for the care of these children, emphasis should be on the child and the family, and efforts should be aimed not only at controlling the disease and managing the current problems, but also at planning for the future. Steps should be taken to improve the resilience of patients and families through better education to cope with the vicissitudes of these diseases, and improving social and peer support for the children and their parents. Education in stress management, including family empowerment and self-management to help the child cope better with their disease and disability, is equally important.

The Team

Expertise should be the cornerstone of the care of children with rheumatic diseases. Accurate diagnosis is the first step. Rheumatic diseases may be acute and explosive in onset, or they may evolve over a period of months and years. Only one organ system may be affected, or several systems may be involved. Rheumatic disease may mimic several other inflammatory and noninflammatory diseases. It may not be possible to place an accurate diagnostic label at first, yet life-threatening complications and functional disabilities may have to be managed. Great skill and patience are required to support the families in managing their problems in the face of uncertainties in diagnosis and prognosis. Therein reside the challenges and pleasures of rheumatology.

Comprehensive treatment centers should be based in tertiary care academic centers. The treatment team ( Fig. 11-1 ) should consist of a pediatric rheumatologist, a nurse specialist, physical and occupational therapists, a social worker, and a psychologist, all working as a team with the child’s primary care physician. Consultations with an orthopedic surgeon, ophthalmologist, nutritionist, and dentist should be available when required. Team care is expensive and is not required for every child, particularly with the recent advances in medical management. However, these services should be available when needed. In the developing world, where resources and multidisciplinary tertiary care centers are scarce or inaccessible, different models of care may have to be considered ( Fig. 11-1 , Table 11-1 ). In these situations, for instance, reliance may have to be placed more heavily on less specialized teams for routine follow-up, with less frequent visits to tertiary centers. Communication, possibly assisted by telemedicine, is a central component in ensuring appropriate care in this model. Appropriate outreach training in basic rheumatology and examination techniques for community health care workers, pediatricians, and general practitioners at outreach sites can help to foster trust and facilitate effective communication. Specially trained nurses have fulfilled the contact function effectively in many pediatric rheumatology centers.

Patient and Parent Education

Children with rheumatic diseases and their parents require education on several issues. These issues are discussed in detail in publications listed at the end of this chapter. The mode of teaching has to vary to suit the needs and skills of parents. Language barriers, cultural backgrounds, and literacy issues must be kept in mind before such programs are organized. Education about the disease, medications, adverse effects of medications, and therapy programs should be provided both individually by the pediatric rheumatology team and in parent support groups. The belief system of the family should be explored, and all doubts and questions should be addressed. Information required by parents varies with the time that has passed after diagnosis, and also depends on the developmental needs of the child. Therefore, education must be individualized. Services of a translator may also be needed.

Information is readily available on the Internet, where resources are easily accessible. However, families need help to ensure that information is gathered from reliable sources, such as those listed at the end of this chapter, and they will need assistance with interpretation of the information gathered.

Counseling

A trusting relationship is the most important requirement for effective counseling. To be trusted, one must be trustworthy. Open, honest communication with the child and the parents is a major component of this relationship.

Collecting information about a child’s illness, behavior, and family dynamics may require many visits and observations. Empathic listening and careful observation is the first step, and attention must be paid to nonverbal cues.

Physicians are trained to look for and diagnose weaknesses in the person and the system. It is also important to identify strengths in the patient, family, and the community that can be utilized to counter weaknesses. This strength may lie in the extended family, an interested schoolteacher, or a loving grandparent. Some families grow in strength in the presence of adversity. Factors that contribute to successful coping should be supported and encouraged. Referral to a clinical psychologist may be needed.

Children with physical disabilities and deformities and children who look different because of a rash or steroid therapy are bound to feel different and self-conscious. In some parts of the developing world, where adherence to traditional beliefs is strong, this may lead to stigmatization; these afflictions may also be blamed on curses or bewitchment. Children and adolescents who are disabled may be left aside because they may not be considered to be productive individuals in the family. Physical disability and fluctuation in disease activity may make it difficult for these children to participate in social and family activities. Emphasizing what they can do and planning activities in which they can participate helps their sense of self-worth and morale. The involvement of siblings, friends, and classmates in helping a disabled child during activities should help create a successful experience for everyone, including the healthy children.

Adherence

Children with chronic illness soon tire of taking medicines day after day with no end in sight. They often ask why they have to do mindless exercises that do not appear to help them. Therefore, adherence with medication and therapy programs is a major issue for children with chronic diseases. The word compliance implies that the physician gives orders and the patient obeys. But the ideal situation is an informed patient who chooses to follow the treatment prescribed by a trusted physician after being convinced of the benefits and made aware of the consequences of not following the treatment. In other words, the patient chooses to adhere to the prescribed program. In pediatrics, one must consider the child, the parent, and the caregiver to ensure the treatment plan is followed. Patient and parent education and including the needs of family members in the planning process are essential. Listening to the specific needs of a child and family and incorporating them into the plan, and designing a plan that accounts for the stresses and strengths of the family and their cultural values, increases the likelihood that the plan will be followed. Factors that affect adherence and strategies to enhance adherence are listed in Table 11-2 and Boxes 11-3 and 11-4 .

Meta-analyses show that education and behavioral intervention provide not only improved adherence but better health outcomes overall. Effect sizes ranged from small to large, with particular characteristics such as age, gender, and diagnosis having a significant impact on the effectiveness of interventions. The use of electronic monitors to measure adherence can provide more objective data and enable easy data feedback to parents. Web-based interventions for adherence can also be effective.

Sibling Issues

Stress related to living with chronic illness affects every member of the family, including siblings. The role of siblings and their impact on the developmental needs of the patient may vary with the age and cognitive level of siblings, their perception of the child with chronic illness, parental attitudes, and the demands placed on unaffected siblings. Feelings may range from guilt (that siblings are responsible for the sick child in some magical way), to fear of catching the illness, to embarrassment. Siblings may resent the extra time and attention given to the affected child and perceived favoritism in matters of discipline. Realistically, parents may not be able to provide adequate time to satisfy the developmental needs of the siblings. This is a problem for single parents in particular.

Depending on the parental demands, some siblings may take an important role in the medical treatment of the affected child (such as reminding them about medications and helping with exercises); others may be protected from this role by their parents. Some siblings may take a more protective role of their brother or sister in school and outside the home.

Disease severity, parental functioning, family stress, and family support systems are some of the determinants of the effects of chronic illness on siblings. In spite of ambivalent feelings, siblings of children with arthritis function well in life and can be a great source of extra support, both at home and at school. It is important to evaluate the needs of siblings in caring for children with chronic illness and to help support their needs.

School Issues

In general, rheumatic diseases do not affect cognition, except for conditions that affect the central nervous system, such as SLE, central nervous system vasculitis, or drug side effects. Children with arthritis can and should attend regular school except in special circumstances. Parents must be advocates for their children and learn both their own rights and responsibilities and those of their children. However, the school system often must institute several adaptations to the standardized schedule. In the United States, children with chronic illness and disabilities must be educated in the “least restrictive environment.” Individualized education plans have to be formulated at the parents’ request, and there are strong “due process” requirements. Similar requirements are in place in most jurisdictions in the developed world.

Depending on the medical condition and the nature of the disability, including participation in daily and community life activities, one or more of the “related school services” may need to be provided to ensure optimal educational opportunities. Special school services provide physical adaptations in schools for handicapped access and elevators; arrange class assignments on the same floor; and provide a duplicate set of books. Transportation, school counseling, nutrition, adaptive physical education, and homebound instruction are some of the other services that may be needed.

Common concerns expressed by children and parents in relation to school, as well as suggested solutions, are given in Table 11-3 . The nurse or occupational therapist can provide an individualized checklist for parents that can be shared with the school nurse or teacher. School nurses are some of the best advocates for children with disabilities and special needs. They can work with teachers and physical education instructors to make appropriate modifications within the school. Therefore, communication between the tertiary center staff members (particularly the nurse) and the school nurse is essential. It is important to recognize that teachers and parents tend to emphasize issues related to activities of daily living as limiting school life, whereas children themselves rate peer acceptance and self-concept as more important. Therefore, these children need more help with peer support and better coping skills.

Finally, if a child is considering vocational training or postsecondary education, early planning beginning in secondary school is essential. It should include visiting prospective campuses, avoiding schedule (credit hours) overload, arrangement for regular meals, and, if the student is leaving home, locating a local pharmacy and physician.

Transition

Growing up through adolescence into young adulthood is a major task for any child. This time becomes a challenge for children with chronic illness and their parents. Adolescents with chronic diseases have to be encouraged gradually to take control of their disease management. This requires preparation of the family and child before adolescence. Preparation of these children requires attention to independent living skills and self-advocacy. Both physicians and parents have to let go of the child in a sensitive and gradual way. The parent has to trust the child, and the child must demonstrate that he or she is capable of taking care of ongoing management and needs. Adolescent support groups with professional leadership may be helpful.

Transition to adult care is a process that should start when a child reaches adolescence. This requires planning and coordination with participation by the patients, family, the pediatric service team, and the adult service team. It should be comprehensive and responsive to the needs of the patient. Some of the subjects that should be addressed individually or in group sessions are sexuality, alcohol and drug use, and vocational planning. The young person may need coaching in self-management, self-advocacy, communication, and decision making. Barriers may include changing location for college, changing from a well-known care provider to a new one, reluctance of parents to relinquish control, or gaps in insurance coverage.

Box 11-5 lists some strategies that may be useful to achieve successful transition.

Financial Issues

Children with chronic illness account for a large proportion of health care expenditures in the United States. Families of children with rheumatic diseases may spend hundreds to thousands of unreimbursed dollars out of pocket per year, not including time lost from work. In the current health care competitive environment, children with chronic illness and disabilities are particularly vulnerable. The high cost that goes with chronic illness and the pressures to cut costs may make it difficult to provide adequate and appropriate care for children with chronic illness and disabilities. In the United States and many other countries, families need to be educated about various types of health coverage and how to work with health maintenance organizations, insurance companies, community resources, and government agencies. Both parents and physicians need to work through the political process to bring about changes in financing of medical care that will ensure access to appropriate services for all children with chronic diseases.

Nutrition

Nutritional abnormalities affect a significant number of children with rheumatic diseases. Factors that contribute to these abnormalities include metabolic effects of inflammation, physical inactivity, reduced energy intake, and the effects and side effects of drugs used to treat these diseases. The fact that fasting can have an antiinflammatory effect and that rare patients develop transient arthritis caused by certain foods (e.g., gluten-containing foods) has promoted interest in the relationship between diet and arthritis. The earlier concern regarding relationship of alfalfa sprouts in SLE has generally been disproved.

Patients and parents are also influenced by the publicity for alternative and complementary medicine because scientific medicine cannot promise a cure for rheumatic diseases. Therefore, the following questions are raised often: Can specific food items aggravate or precipitate symptoms of arthritis? If so, what food items should be avoided in the diet? What are the roles of dietary supplements, special diets, and elimination diets?

The physician’s main goal should be to control inflammation as rapidly as possible and thus minimize the nutritional abnormalities. The child should be maintained on a well-balanced, healthy diet with adequate vitamins and minerals and should be encouraged to be as physically active as possible. The physician also has to counsel parents on the proper role of nutrition and educate them about fad diets and dietary supplements.

Physicians have to teach parents that at present there is no specific, evidence-based dietary recommendation for the treatment of rheumatic diseases. There are no data to recommend an elimination diet, although rare patients may exhibit altered immune response to items such as milk or gluten. Moreover, an elimination diet should be tried only under strict medical supervision, because there is a danger of precipitating malnutrition and deficiency diseases. Megavitamins and macrobiotic diets should be avoided.

Evidence-based advice should include supplemental calcium and vitamin D, particularly for children on glucocorticoid therapy, folic acid for children on methotrexate, salt restriction and potassium supplementation for children on long-term glucocorticoid therapy, and supplemental iron with or without vitamin C for children with anemia of chronic illness.

In previous decades, the main dietary focus for patients with arthritis was increasing caloric consumption. However, as new therapies have been developed, the number of patients with insufficient caloric intake has declined. Today, childhood obesity is becoming an important issue in children with arthritis. Increased body weight adds increased stress on the weight-bearing joints. An individualized weight management plan and consultation with a dietitian are indicated for some patients. Programs that include exercise along with dietary restriction are more likely to be successful. Obesity is often a family problem, and successfully controlling it requires family participation.

Children with rheumatic diseases on chronic glucocorticoid therapy are at increased risk for accelerated atherosclerosis. Therefore, the American Heart Association places children with chronic inflammatory diseases in Tier II in the algorithm for cardiovascular risk reduction. Children in this tier should be screened for additional risk factors. The screenings should include obtaining a family history of early coronary heart disease, assessment of the amount of physical activity, and measurement of blood pressure, body mass index, fasting blood sugar, and a lipid profile. On the basis of this screening, patients may have to be referred to a dietitian or other specialists for further management.