Caring for Chronically Ill Children

The prevalence of childhood chronic illness has risen steadily for the last three decades. Sophisticated technological interventions, mechanical devises that substitute for immature or non-functioning physiology and the growing rate of acquired chronic illnesses join together to create a population of children and families who deal with illness on a day-to-day basis. The intricacies of maintaining both a parent and a “nurse” role with children causes strain in many families. Nurses knowledgeable about caring for this population provide a stabilizing influence and an ongoing support system for children and families who need to be vigilant with even the most complex therapeutic regimens.

Estimates are that as many as 31 percent of children have a chronic childhood illness with the severity ranging from mild to moderate asthma to ventilator dependent, total care (McMillan, DeAngelis, Feigin, and Warshaw, 1999). Severe chronic illnesses affect approximately 2 to 4 percent (1 – 2 million) children; this group of children is considered medically fragile and may be technology dependent. The main goals of health care are to prevent and diminish the physiological effects and the psychosocial impact of the chronic disease. Because development and physiological growth are inextricably interdependent, preserving the development, fostering the achievement of developmental milestones, and building social skills are as important as maintaining the physiological function.

A chronic illness is defined as one that lasts longer than 3 months, requires medical intervention, interferes with activities of living and may impact normal growth and development. The most common chronic illnesses in children fall into two groups, 1) allergic disorders such as asthma, eczema, hay fever and 2) neuro/seizures, neuromuscular (cerebral palsy). Children suffer from a wide variety of illnesses, unlike adults who tend to have common chronic conditions, i.e., diabetes, osteoarthritis, congestive heart disease, malignancy, hypertension, and a very few rare diseases. Similarly, childhood pathophysiology manifests itself differently according to the child’s gestational and chronological age, growth pattern, etc. Caring for children with chronic illness requires the nurse to have a broad understanding of pediatric anatomy, physiology, and development.

Chronic illness occurs in children as a result of prematurity, congenital anomalies, or, may be acquired because of infection, lifestyle or familial tendencies. Continuous advances in medical technology allow practitioners to save many children who would not have survived just a few years ago.

Despite advances in obstetrics, the rate of premature births in the United States continues to climb. Approximately eleven percent of births are classified as premature, i.e., occurring before 37 weeks gestation (Hall 2000). Even with the use of Surfactant, premature infants are likely to have respiratory problems with subsequent chronic lung disease. Similarly, premature birth accounts for approximately fifty percent of neurologic disabilities, including cerebral palsy. Gastrointestinal problems also occur frequently in premature babies leaving them with an ongoing need for feeding tubes and special diets. Premature birth remains a frequent cause of chronic illness in children necessitating specialty medical care and continual intervention by parents and health care professionals.

Congenital anomalies cover a broad spectrum of pediatric diagnoses and subsequent illness. Genetic defects, though not especially common, often involve multiple systems and may result in severe developmental delay and mental retardation. Other congenital anomalies, such as heart or renal disease may require complicated, costly interventions that ultimately compromise function and, ultimately, may be fatal.

The largest group of children with chronic illness is composed of those with an acquired illness. These typically are caused by infection, familial tendencies, and lifestyle. Dramatic increases in the incidence of asthma and obesity with its many pathologic manifestations leave thousands, if not millions of children with ongoing special health care needs. Cancer, another acquired illness in children, is becoming more treatable and, though often not fatal, is frequently painful and requires expensive treatments over an extended period of time. Although HIV-AIDS can be treated in utero when the mother takes AZT, many children are born HIV positive and face the potential for having full-blown AIDS with all of the implications for continual treatment.

Chronic illness has the potential to impact a child in many ways. Normal growth and development, physical appearance, and psychosocial skills may all be adversely affected because of a chronic condition.

All aspects of development are linked to physical growth. A popular theory is that children have critical times to master developmental milestones. If, because of a disease process, the critical time passes before the task is achieved, the milestone may never be accomplished or may be significantly delayed. Also, therapeutic intervention is needed to assist the child with the milestone because the activities that occur normally during childhood and allow healthy children to reach milestones frequently are not available to the child with chronic illness. Specialists in physical, oral-motor, and fine motor therapy are needed to simulate activities that allow children with chronic illness to gain these skills. A good example is sucking and swallowing that we take for granted in healthy infants. Infants who begin life on mechanical ventilation do not have adequate opportunities to master sucking and swallowing. Oral-motor therapy is usually necessary to enable these children to coordinate swallowing food and breathing without aspirating. The course of therapy may take several months during which time the children receive their nutrition via parenteral or enteral tubes.

School plays a vital role in the growth, development, and maturation of children. Unfortunately, children with chronic illness miss significant school days because they are not physically well enough to attend or are absent in order to receive necessary treatment. School not only prepares children intellectually for adulthood, it also provides the environment for learning social skills, peer interaction, making friends, etc. Children with chronic illnesses have to make up for those lost interactions and may rely on nurses and other health care professionals to help them learn about interpersonal interactions. The good news is that a review of the literature done in the Netherlands by Boekaerts (1999) found that although children with chronic illnesses miss more school, they did not exhibit lower school performance; the exception was children with epilepsy. Studies of this nature in the US will provide health professionals, teachers, and families with important information about how the school experience can be enhanced for children with chronic illnesses.

Adolescents, whose developmental task is to establish an identity separate from their parents and consistent with their future goals, frequently experience difficulty because a chronic illness has been incorporated into their identity and makes them more dependent on parents than most of their peers. The natural tendency for teenage rebellion may manifest as a refusal to comply with a required therapeutic regimen. Adolescents with diabetes are notorious for abandoning the prescribed regimen to enjoy typical teenage activities, i.e., pizza and cola after a football game, candy and other high calorie snacks while studying with friends, etc. The insulin pump permits adolescents greater freedom, but attention to hemoglobin A1c and blood sugars is still critical. Nurses can play a key role to help adolescents maintain good control of their diabetes while having fun, happy teenage experiences.

Haluska, Jessee, and Nagy (2002) studied adolescents with cancer to identify their sources of social support and to compare if it differed greatly from their healthy peers. Their findings indicated that adolescents with cancer expressed satisfaction with social support systems that included both family members and peers; further, they described their systems as strong and comparable to their peers. Because peer interaction is so important to adolescent development, it is important that nurses and other health care professionals encourage contact with these social networks even when the teen is hospitalized (Haluska, Jessee, and Nagy, 2002).

Self-care provides children with the opportunity to maintain some control over their illness and to achieve independence from constant parental input. Asthma and diabetes are two chronic illnesses that lend themselves very well to self-care management by children (Cohn and Brouhard, 2000). Nurses, as teachers who understand how to teach using appropriate techniques according to the child’s developmental level, can assume a lead role in promoting self-care and childhood independence.

Researchers at SUNY Medical University hypothesized that children with chronic illness would be hospitalized more often for severe acute illnesses (Dosa, Boeing, and Kanter, 2001). They found that during a 1year period, 45 percent of unscheduled ICU admissions were the result of acute illnesses in children with chronic conditions. Further, the researchers judged that more than half of the admissions were preventable but occurred because of physical and social environmental factors and decisions made by families. Good family education to promote understanding and help families make informed decisions is the cornerstone of good nursing care.

Caring for healthy children puts a strain on most families; add to that, caring for a child with special health care needs and most families experience periodic, sometimes frequent episodes of being overwhelmed (Melnyk, Moldenhouer, Feinstein, Small, 2001). The emotional aspects of care, including guilt, fear, anger, resentment is with family members every day. The parents are the ones that must perform treatments or cajole the child into self-care activities. Beyond caring for the child, parents must maintain employment, care for other children and perhaps parents, and negotiate a health care system that often baffles even health care professionals. Some parents report a sense of chronic sorrow about the loss of normalcy for their child.

Negotiating the health care system poses special challenges for families. They report feeling lost in a maze of ever-changing health care providers and want to establish a sustained relationship to reduce stress associated with change and uncertainty (Swallow and Jacoby, 2001). Similarly, families want satisfactory alliances with health care professionals and they want those alliances to be built on mutual respect and good communication. Parental coping and competence are enhanced when such relationships exist (Swallow and Jacoby, 2001).

Siblings of children with chronic illnesses often feel short-changed because so much attention is spent on the ill child. They mourn the loss of what they envision would be a normal sibling relationship. Nurses should be sensitive to the issues siblings encounter and consider their well-being in the overall plan of care.

Marital relationships seem to be either strengthened or to disintegrate with the burden of chronic illness. Some couples grow closer and appreciate life together more. Some families experience strain because the demands of the chronically ill child seem to supersede consideration for other family members. Chronic fatigue is a common complaint and inability to make family plans with any certainty interferes with family outings and parental time together without children. Families often report feeling isolated and are not invited to social gatherings because friends are unsure of how to plan activities around the constraints imposed by the child’s chronic illness.

The financial concerns of families are significant. A severely premature infant who remains in the intensive care unit may exhaust the health insurance policy limit in the first few months of life. Families are then forced to tap into public programs for ongoing funding. Fortunately, the availability of both funding and programs has increased over the last decades. Medicaid programs in most states provide some financial support and employer insurance policies are less reticent to cover services to provide home and community based care for children with special health care needs. Voluntary agencies such as Shriner’s hospitals, Easter Seals, etc. also provide services for families who have no insurance or need extended care beyond the limits of their health insurance policy. Still families constantly grapple with insurance companies to get authorization for payment.

Special funding programs such as the Ryan White legislation and the Katie Beckett waiver have provided much needed financial support for children with catastrophic illnesses. Ryan White provides communities with money to procure both medications and services for children with HIV-AIDS. The Katie Beckett waiver, now subsumed under a Medicaid Home and Community waiver program, was initially implemented to allow children to receive Medicaid benefits and, therefore home and community based services, by declaring the child a family of one and not including parental income to determine eligibility. This waiver allowed many children, particularly those who were ventilator dependent, who had been hospitalized for months and even years, to be discharged home and receive appropriate home care nursing services and equipment.

The 1996 passage of the Personal Responsibility and Work Opportunity Reconciliation Act changed welfare policy in the United States. As this legislation replaced Aid to Families with Dependent Children (AFDC), parents of children with chronic conditions struggled to comply with the new regulations that eliminated cash benefits and limited the time frame for securing employment with benefits. Smith, Romano, Wood, et al. found that families experienced substantial difficulty because of child care issues, availability of insurance coverage, and frequent absence from work because of the child’s medical needs (2002).

The passage of the Family Medical Leave Act relieves the fear that an employer will fire a parent who must be absent to care for a chronically ill child. This act allows an employee to take up to 12 weeks of unpaid leave a year to care for a family member who is ill.

Nurses have a significant role with families. Nurses serve as the organizing influence of the interdisciplinary team and plan of care. They are the communication link for the families and often times are the knowledgeable “interpreter” of complex medical information and treatment plans. Nurses build parental knowledge and confidence through clear communication and education. Parents who feel confident and competent about their ability to care for the child are usually more astute consumers of health care resources.

There is no question that sophisticated medical technology impacts on society at large. The ability to aid the survival of extremely premature infants along with new treatments for previously fatal childhood illness provides both blessings and challenges to the health care system. New methods for financing children’s health care are constantly being sought. In many states, money from tobacco lawsuits is used to fund children’s programs. Some debate whether the allocation of funds to children with severe and potentially fatal conditions is a good use of scarce resources when some children are denied basic health services because they lack funding. Resource allocation will be a continuing topic of political candidates, policy makers, insurers, and health care professionals for many years.

Children with chronic illness pose special challenges to the educational system. The passage of P.L. 94-142 provided that all handicapped children were entitled to a free, appropriate public education. Then P.L. 99-457 expanded that requirement for states to provide early intervention services to children from birth to three years of age and to provide special education and related services to all children three to five years old. Services to be included are: family education, counseling, speech and hearing therapy, occupational and physical therapy, case management, and other services to enable the child to benefit from early intervention service. While these services are reasonable expectations for families with a special needs child, the requirements tax an already overburdened, under-funded, and overcrowded public education system. However, some school districts were able to receive state and federal funding so they could hire nurses to provide services that are required by children with chronic illness. Teachers were understandably frightened to care for children with tracheotomies, receiving medications, and even on mechanical ventilation. Consequently, the presence of nurses provided a stabilizing influence to a tenuous situation.

For the most part, the health care service delivery system specializes in providing short term, acute care. The challenges of providing a continuum of services to children 0 – 18 require careful analysis of existing systems. How can we evolve part of the delivery system to provide competent, sensitive, effective and efficient care to children with a broad spectrum of health care and developmental needs? Who will serve as the coordinator of this service delivery sector? Physicians, nurses and other health care providers who are experts in the care of chronically ill children must treat these children. Providers must be sensitive to growth and development needs and be able to provide family education. Someone must coordinate the care so that both simple and complex services are provided efficiently and effectively.

Some specific attributes that the health delivery system must have are: child-family centered focus, coordinated and continuous service system, competent providers, comprehensive services provided by providers knowledgeable about childhood illness. In addition, the services must be accessible, accountable, and affordable.

A service delivery system for children with chronic illness is a perfect opportunity for nurses to use their bio-psycho-social expertise. Nurses are able to understand the technical aspects of care and can explain procedures and protocols to parents in a clear, simplified language. Moreover, nurses understand how to organize the care so that time is used efficiently and children are spared unnecessary pain and inconvenience. Nurses are advised to sensitize themselves to the needs of this growing population of children and families so that they can serve as experts and advocates.

As the number of children and adolescents with chronic illnesses continues to increase, nurses with their knowledge of pathophysiology, teaching methods, growth and development, are logical health professionals to assume a prominent role with this population in the health delivery system. Nurses can monitor the system to assure that services are appropriate and meet the requirements for specialized expertise. Additionally, nurses are the logical choice to determine if families’ needs for good, clear communication are being met.

As patient advocates, nurses may be willing to serve on policy committees to help design aspects of the health delivery system so it will be sensitive to the needs of children with chronic illnesses and their families. As advocates, nurses can educate members of the community, i.e., school personnel, physician office workers, insurance personnel, about the specialized needs of these children and adolescents.

Children with chronic illnesses and their families face many challenges as they cope with an ongoing health care deficit. Becoming sensitive to the problems faced by these children and families so that care can be organized to effectively and efficiently serve them, is a necessary step to improve the overall health care system for children and families. Nurses must be willing to offer their expertise to assist in the restructure of services.

Boekaerts, M. (1999). Stress, coping, and adjustment in children with a chronic disease: A review of the literature. Disability and Rehabilitation, 21, 311-327.

Cohn, R. C. and Brouhard, B. H. (2000). Technical skills in chronically ill children. Clinical Pediatrics, 39, 347-349.

Dosa, N.P., Boeing, N. M., Kanter, R. K. (2001). Excess risk of severe acute illness in children with chronic health conditions. Pediatrics, 107, 499-504.

Haluska, H. B., Jessee, P. O., Nagy, M. C. (2002). Sources of social support: Adolescents with cancer. Oncology Nursing Foundation, 29, 1317-1324.

McMillan, J.A., DeAngelis, C. D., Feigin, R. D., Warshaw, J. B., (1999). Oski’s Pediatrics Principles and Practice. Philadelphia: Lippincott, Williams, Wilkins.

Melnyk, B. M., Molderhouer, Z., Feinstein, N. F., Small, L. (2001). Coping in parents of children who are chronically ill: Strategies for assessment and intervention. Pediatric Nursing, 27, 548-558.

Smith, L.A., Romero, D., Wood, P. R., Wampler, N. S., Chavkin, W., Wise, P, H. (2002). Employment barriers among welfare recipients and applicants with chronically ill children. American Journal of Public Health, 92, 1453-1457.

Swallow, V.M. and Jacoby, A. (2001). Mothers evolving relationships with doctors and nurses during the chronic illness trajectory. Journal of Advanced Nursing, 36, 6-12.