Chronic Illness, Disability, and End-of-Life Care

Chapter 36

Chronic Illness, Disability, and End-of-Life Care

Marilyn J. Hockenberry

Perspectives on the Care of Children and Families Living with or Dying From Chronic or Complex Diseases

Scope of the Problem

Advances in medical and nursing care such as the increasing viability of extremely preterm infants, the portability of life-sustaining technology (e.g., total parental nutrition, ventilatory support), and life-extending treatments for children with conditions that previously would have led to an early death (e.g., malignancies, genetic conditions) (Burke and Alverson, 2010) have led to an exponential rise in the prevalence of children with complex and chronic diseases. These children have complex conditions involving several organ systems and require multiple specialists, technologic supports, and community services to help them function to their healthiest potential. The complexity, high level of skill required to meet their daily health care needs, and continuous nature and potential volatility of the condition sets this group apart from the broader population of children with special health care needs (Harrigan, Ratliffe, Patrinos, et al., 2002; Rehm and Bradley, 2005). A range of terms such as medically complex, technology dependent, and multiply handicapped have been used to describe this vulnerable population of children (Carnevale, Rehm, Kirk, et al., 2008; Cohen, Friedman, Nicholas, et al., 2008; Harrigan, Ratliffe, Patrinos, et al., 2002; Miles, Holditch-Davis, Burchinal, et al., 1999; O’Brien and Wegner, 2002; Watson, Townsley, and Abbott, 2002). Frequent and prolonged hospitalizations; complex and multisystem health and developmental needs; and reliance on technology and care that cross hospital, clinic, and home settings are the key characteristics that all of these terms seek to signify about the children they are used to represent (Harrigan, Ratliffe, Patrinos, et al., 2002).

The nature and severity of childhood chronic and complex conditions is widely heterogeneous. However, it is the health and developmental consequences of these diagnoses such as ongoing functional impairment; neurodevelopmental disability; dependence on medical technology; and the need for ongoing skilled, supportive care from health care providers and family members that render these children and families particularly vulnerable. The impact of chronic and complex illness in children is wide ranging. Although many authors have described the rise in prevalence that has come about because of advances in medical care (Cohen, Friedman, Nicholas, et al., 2008; Council on Children with Disabilities, 2005; Haffner and Schurman, 2001; Mentro, 2003), accurate estimates of the numbers of affected families are not known (Carnevale, Rehm, Kirk, et al., 2008). These conditions present most families with additional tasks, responsibilities, and concerns (Ray, 2002). A child’s activity level and developmental opportunities can be affected. Days can be lost from school. Children with complex chronic conditions may be at increased risk for behavior or emotional problems. Parents may lose days from work, experience financial strain, and be challenged both emotionally and physically as they cope with care of the child.

Siblings are also affected by having a “different” brother or sister and may simultaneously feel guilt and anger or jealousy toward their ill sibling. Clinicians need to know that siblings of children with chronic illnesses are at risk for negative psychologic effects (Sharpe and Rossiter, 2002). Parents need encouragement and assistance in understanding the reactions of siblings to having a chronically ill family member (e.g., behavioral regression, anxiety, withdrawal, apathy). In addition, secondary losses such as the ability to participate in extracurricular activities or social events occur because of routines imposed by the affected child’s chronic condition.

Trends in Care

Developmental Focus

Focusing on the child’s developmental level rather than chronologic age or diagnosis emphasizes the child’s abilities and strengths rather than disabilities. Attention is directed to normalizing experiences, adapting the environment, and promoting coping skills. Nurses often are in vital positions to redirect attention from the pathologic model with its focus on weaknesses and problems to the developmental model to meet the unique needs of the child and family.

A developmental focus also considers family development. The life cycle of the family unit reflects both the changing ages and needs of family members and changing external demands. A family member’s serious illness can cause significant stress or crisis at any stage of the family life cycle. Just as with individual development, family development may be interrupted or even regress to an earlier level of functioning. Nurses can use the concept of family development to plan meaningful interventions and evaluate care.

Family-Centered Care

Children’s physical and emotional health and their cognitive and social functioning are strongly influenced by how well their families function (Schor, 2003). The importance of family-centered care—a philosophy that considers the family as the constant in the child’s life—is especially evident in the care of children with special needs (see also Family-Centered Care, Chapter 26). As parents learn about the child’s health care needs, they often become experts in delivering care. Health care providers, including nurses, are adjuncts to the child’s care and need to form partnerships with parents. Effective communication and negotiation between parents and nurses are essential to forming trusting and effective partnerships and finding the best ways to meet the needs of the child and family (Corlett and Twycross, 2006). Collaborative relationships are characterized by communication, dialog, active listening, awareness, and acceptance of others’ differences (Schor, 2003).

Family–Health Care Provider Communication.

The disclosure of a serious chronic or complex condition in a child is one of the most stressful aspects of communication between families and health care professionals. Often parents have suspected for some time that something is wrong with their child and believe that their concerns were minimized or ignored by health care professionals (Smaldone and Ritholz, 2011; Thomlinson, 2002; Whitehead and Gosling, 2003). After a diagnosis is made, numerous studies have shown that parents are not always satisfied with the way in which information is given. Factors that influence parent dissatisfaction with communication include disrespectful attitudes, breaking bad news in an insensitive manner, withholding information, and changing a treatment course without preparing the child and family (Hsiao, Evan, and Zeltzer, 2007). Conversely parents report satisfaction when they perceive health care providers to be available, demonstrate competence, and engage the child and parent in care decision making (Hsiao, Evan, and Zeltzer, 2007). Similar factors are important in communication of changes in the child’s condition throughout the course of the illness.

Providing information to families with a chronically ill child should be a process of repeated discussions to allow the family to process the information and their reactions to that information and to ask for clarification and further information. Nurses play an important role in ensuring that families’ needs are met during discussions related to the child’s diagnosis, condition, and treatment. This requires assessing the amount of information with which the family is comfortable, how much they understand of the information already given to them, and how they are coping with the information both cognitively and emotionally. Nurses should ensure that the appropriate health care professionals address any concerns or further questions that families may have.

Establishing Therapeutic Relationships.

Another important aspect of family-centered care of children with chronic and complex conditions is establishing a therapeutic relationship with the child and family, which has been shown to predict improved health-related outcomes (Denboba, McPherson, Kenney, et al., 2006). Families, most often the mother, take on enormous responsibility in providing technical care and symptom management of their child’s condition outside the health care institution (O’Brien and Wegner, 2002; Raina, O’Donnell, Rosenbaum, et al., 2005; Swallow and Jacoby, 2001). To build successful therapeutic relationships with families, it is necessary for nurses to recognize parents’ expertise with regard to their child’s condition and needs. Care conferences, especially multidisciplinary meetings that include the family and key health professionals, provide an opportunity for sharing ideas and expressing feelings or concerns. Health care environments for children with serious illnesses are fraught with obstacles that serve as barriers to successful therapeutic relationships with families. For example, the complex, multidisciplinary care required is often characterized by fragmented, noncohesive approaches to care. Continuity of care can be a challenge in acute care hospitals today, and this makes it difficult to establish relationships and understand communication styles.

Individual discussions, especially with the case manager, primary nurse, clinical nurse specialist, or nurse practitioner, help establish a consistent and flexible care plan that can prevent conflicts or deal with these conflicts before they disrupt care. In family-centered care the goal is to maintain the integrity of the leadership role and support the family during times of crisis or stress.

The Role of Culture in Family-Centered Care.

Issues of culture, ethnicity, and race affect access to services, use, and follow-through with referrals and recommendations (Coker, Rodriguez, and Flores, 2010; van Dyck, Kogan, McPherson, et al., 2004; Wise, Wampler, Chavkin, et al., 2002; Wood, Smith, Romero, et al., 2002; Zuvekas and Taliaferro, 2003). For some ethnic and minority populations, cultural understandings of illness, the structure of family life, social roles for individuals with disabilities, and other factors related to the perception of children may differ from those of mainstream American culture. These factors may affect family needs and choices regarding the care of their child with special needs.

Although culture cannot completely explain how an individual will think and act, understanding cultural perspectives can help the nurse anticipate and understand why families may make certain decisions. Cultural attributes such as values and beliefs regarding illness or chronic condition and its causation, social roles for people who are ill or disabled, family structure, the role of children, childrearing practices, self-versus-group orientation, spirituality, and time orientation also affect a family’s response to illness or chronic condition in a child (Carnevale, Alexander, Davis, et al., 2006; Carter, 2002; Marshall, Olsen, Mandleco, et al., 2003; Rehm, 1999; Sterling and Peterson, 2003).

When parents are informed of their child’s chronic illness, interpreters familiar with both culture and language should be used. Children, family members, and friends of the family should not be used as translators because their presence may prevent parents from openly discussing the issues. When working with people of cultural backgrounds different from their own, nurses must listen carefully with an initial goal of understanding and articulating the family’s perspective. The ability to interpret the mainstream medical culture to the family is also important. Furthermore, every effort is made to incorporate traditional cultural beliefs of a family into treatment plans. It is important to keep in mind that “cultural norms” may not always apply to every family from a shared background. Nurses who assess the unique needs of each family, listen, and keep themselves open to novel ways of meeting the individual needs of the child and family will likely be successful in establishing a therapeutic relationship. Developing a care plan in conjunction with the family, considering their preferences and priorities, is an important first step in formulating a plan that best meets the family’s needs, no matter what their cultural background (Ahmann, 1994; Coker, Rodriguez, and Flores, 2010; Ochieng, 2003).

Shared Decision Making

Shared decision making among the child, family, and health care team can result from open, honest, culturally sensitive communication and the establishment of a therapeutic relationship among the family and health care providers. In a shared decision-making model, the health care professionals provide honest, clear information regarding diagnosis, prognosis, treatment options, and risk-benefit assessment. The patient and family then share information with the health care team regarding important family values, acceptable levels of discomfort or inconvenience, and the ability to comply with treatments being recommended (Charles, Gafni, and Whelan, 1997; Kon, 2010). This process allows them to discuss all options in terms of the risks and benefits to the child and family, the prognosis or expected course of the illness, and the impact on the family’s resources (Box 36-1). Together the parents and health care team can make decisions that are best for the family and child at the time the decision is made (Kon, 2010).


Normalization refers to the efforts that family members make to create a normal family life, their perceptions of the consequences of these efforts, and the meanings they attribute to their management efforts (Knafl, Darney, Gallo, et al., 2010). For chronically ill children such efforts may include attending school, pursuing hobbies and recreational interests, and achieving employment and a level of independence. For their families it may entail adapting the family routine to accommodate the ill or disabled child’s health and physical needs (McDougal, 2002).

Children with chronic and complex conditions and their families face numerous challenges in achieving normalization. Families move between the “normal” of living with the experience of chronic childhood illness and the “normal” of the healthy outside world; they often redefine “normal” based on their particular experiences, needs, and circumstances (Deatrick, Knafl, Murphy-Moore, 1999; Gantt, 2002; Nelson, 2002). Normalization may be an important mediator of illness-related stressors (e.g., treatment demands, uncertainty) on family outcomes.

Nurses can help families normalize their lives by assessing their everyday life, social support systems, coping strategies, family cohesiveness, and family and community resources. Interventions could include encouraging families to reduce stress through delegation of care and family tasks, identifying ways to incorporate care into current routines, structuring the home environment to encourage the child’s engagement in age-appropriate activities, and ensuring that families have access to appropriate community support services (Jokinen, 2004; Shepard and Mahon, 2000). Being supportive of the child’s illness and treatment and actively including the family in all aspects of care improves their self-esteem and promotes further development (Shepard and Mahon, 2000).

Home care represents the return to a system and set of priorities in which family values are as important in the care of a child with a chronic health problem as they are in the care of other children. Home care seeks to achieve goals that are consistent with the developmental model (Stein, 1985):

With appropriate training and support, families provide complex procedures and treatments in the home. Parents are challenged to retain a homelike setting among monitors, ventilators, and other sophisticated equipment. Throughout this text home care is discussed as appropriate for specific conditions. The process of transition from hospital to home is elaborated on in Chapters 20 and 21.

Paralleling normalization and home care is the process of mainstreaming, or integrating children with disabilities into regular classrooms. Just as the home is the natural environment for children, so school must also be included as an essential component of children’s overall physical, intellectual, and social development. Children who attend school have the advantages of learning and socializing with a wide group of peers. There is an increased focus on individualization as plans are made to meet the academic needs of these children along with those of the rest of the students.

A variety of supplemental programs have been designed in the school system to accommodate special needs, both at school age and younger, through early intervention, which consists of any sustained and systematic effort to assist children from birth to age 3 years who have disabilities and are developmentally vulnerable. This change and increasing opportunities for normalization for children with disabilities in large part have resulted from the passage of (1) the Education for All Handicapped Children Act of 1975 (Public Law 94-142) and its 1990 amendments (Public Law 101-476), which changed the name of the act to the Individuals with Disabilities Education Act (IDEA); (2) the Education of the Handicapped Act Amendments of 1986 (Public Law 99-457), which directs states to develop and implement statewide comprehensive, coordinated, multidisciplinary interagency programs of early intervention services for infants and toddlers with disabilities and support services for their families; and (3) the Americans with Disabilities Act of 1990. Nurses can provide parents with information about these laws and in some cases may participate in the development of individualized educational programs (IEPs) or individualized family service plans (IFSPs) for children with disabilities.

Managed Care

Managed care programs have become the major form of health care provision in the United States (Jackson, 2000). This model of care has brought both opportunities and challenges with respect to the care of children with chronic and complex conditions. Although managed care may promote continuity and coordination of care for children and families with private insurance, it has had an unfavorable impact on relatively resource-limited families caring for children with complex chronic illnesses (Huffman, Brat, Chamberlain, et al., 2010). Children rely on adults for access to health care and follow-up with treatment regimens, making it necessary to manage the child’s care in the context of the family (McPherson, Weissman, Strickland, et al., 2004; van Dyck, Kogan, McPherson, et al., 2004).

The Family of the Child with a Chronic or Complex Condition

A major goal in working with the family of a child with chronic or complex illness is to support the family’s coping and promote their optimal functioning throughout the child’s life. Long-term, comprehensive, family-centered approaches extend beyond supporting the child and family during the critical periods of diagnosis and hospitalization. Rather, comprehensive care involves forming parent-professional partnerships that can support a family’s adaptation across the trajectory of the illness to the many changes that may be necessary in day-to-day life, determining expectations of and for the child, and providing a long-term perspective (Box 36-2).

The impact of a child’s medical or developmental condition is often experienced over time, initially as a crisis at the time of diagnosis, which may occur at birth, after a long period of diagnostic testing, or immediately after a tragic injury. The impact may also be felt before the diagnosis is made, when parents are aware that something is wrong with their child but before medical confirmation (Thomlinson, 2002; Whitehead and Gosling, 2003).

The diagnosis and initial discharge home are critical times for parents (Coffey, 2006). Several factors can make it particularly difficult, including a long duration of uncertainty in the diagnostic process, negative perceptions of chronic illness, insufficient information, and lack of mutual trust between parents and their child’s health care team (Garwick, Patterson, Bennett, et al., 1995; Monterosso, Kristjanson, Aoun, et al., 2007; Nuutila and Salanterä, 2006). Parental feelings of shock, helplessness, isolation, fear, and depression are common (Coffey, 2006; Nuutila and Salanterä, 2006). Throughout the first year parents struggle to accept the child’s diagnosis, care, and uncertainty of the future (Coffey, 2006). Providing explicit and uncomplicated information to parents in an empathic way (Nuutila and Salanterä, 2006); assessing the family’s daily routine, living conditions, background knowledge, skills and abilities, and coping behaviors; and evaluating the family’s understanding of the information can encourage optimal support at the time of diagnosis and initial discharge home. It is also necessary to reassess parents’ needs for information and support on a routine basis (Nuutila and Salanterä, 2006).

Other critical times include the exacerbation of the child’s physical symptoms, which increases parental care. These crises often involve medical intervention and rehospitalization. Frequently the child does not return to his or her precrisis level of functioning, and parents and family must adapt to new care needs and schedules. Instability may also follow transition points on the illness trajectory. For example, changes in caregivers and significant chronologic age milestones can increase parental stress, and advocating for the child during these times is essential. Supporting parents, respecting their stress and emotions, and acknowledging their role as team members in the care of their child are important aspects of nursing care (Coffey, 2006; Nuutila and Salanterä, 2006).

Impact of the Child’s Chronic Illness

Each member of a family who has a child with a chronic or complex illness is affected by the experience (Sullivan-Bolyai, Sadler, Knafl, et al., 2003). The effects on the parents and their responses may be so intense that they directly influence the other members’ reactions and the child’s own coping.


In addition to the stress of grieving for the loss of a perfect child, parents are affected by whether or not they receive positive feedback from interactions with their child. Many parents feel satisfaction and fulfillment from the parenting role. For others parenting may be a series of unrewarding experiences that contribute to feelings of inadequacy and failure (Box 36-3). These responses may be most evident in parents who are responsible for the child’s care. For example, parents may become preoccupied with their ability to carry out certain procedures, overlooking the child’s personal comfort and satisfaction or failing to offer praise for anything less than perfect cooperation or performance. They may pursue a frustrating activity until they achieve “success”—long after the child has become irritable and uncooperative. As a result parents can become caught in a pattern of interaction that is mutually unrewarding and minimally productive. This situation may become exacerbated by disagreements or lack of support from other family members and judgment from caregivers and others in the community. For these parents several strategies may be helpful, including education regarding what can reasonably be expected of their child, assistance in identifying the child’s strengths, praise for a parental job well done, and respite care so parents can renew their energies.

Parental Roles.

Parenting a child with a complex chronic condition requires much more than raising a typical child. In addition to attending to the routine aspects of parenting, parents of chronically ill children take on the added responsibility of performing complex technical care and symptom management, advocating for their child, and seeking and coordinating health and social services for their ill or disabled child (Kirk, Glendinning, and Callery, 2005). These added responsibilities must then be balanced with the needs of other family members, extended family and friends, and personal health and obligations to minimize consequences to the overall functioning of the family (Coffey, 2006; Ray, 2002). Enormous demands may be placed on parental time, energy, and financial resources.

Often one parent or partner remains at home to manage existing family responsibilities while the other remains with the ill child. The partner who is not included in the caregiving activities may feel neglected because all of the attention is directed toward the child and resentful that he or she is not sufficiently informed to be competent in the care. Without active participation in the child’s care, the parent has little appreciation of the time and energy involved in performing these activities. When this partner does attempt to participate, the other parent may criticize the less skillful efforts. As a result, communication and support for one another may be adversely affected.

The nurse can help parents avoid role conflicts by providing anticipatory guidance early in the process. Teaching should address stressors often identified as having an impact on the marriage, including (1) the burden of care at home assumed by primarily one parent, (2) the financial burden, (3) the fear of the child dying, (4) pressure from relatives, (5) the hereditary nature of the disease (if applicable), and (6) fear of pregnancy. Other causes of tension may center on the inconveniences associated with care such as long waits for an appointment, lack of parking near care facilities, or lack of overnight accommodations. Certainly these last stressors are within health professionals’ domain to minimize, if not eliminate.

Mother-Father Differences.

Mothers and fathers in the same family often adjust and cope differently as parents of a child with a complex condition. Some mothers experience a peaks-and-valleys periodic crisis pattern, whereas most fathers tend to experience a steady, gradual recovery. Some research suggests that mothers of children with certain conditions may be more susceptible to psychologic distress and fatigue than fathers (Tong, Kandala, Haig, et al., 2002). Mothers are most often the primary caregiver and are more likely than fathers to give up their jobs to care for their children, often resulting in social isolation (Coffey, 2006). Mothers often have greater needs for social support and positive appraisal of the situation, whereas fathers are more likely to use self-controlling behaviors to cope (Goldbeck, 2001; Mastroyannopoulou, Stallard, Lewis, et al., 1997).

Fathers of children with disabilities struggle with issues that may be distinct from those of the mothers (Swallow, Macfadyen, Santacroce, et al., 2012). Fathers may think that their role of protector is challenged because they do not know how to help and cannot protect their family from the seemingly overwhelming recurring problems. With today’s increased emphasis on fathers’ involvement in the lives of their children, this loss is felt more profoundly than in the past. The extensive stresses in the family can leave fathers feeling depressed, weak, guilty, powerless, isolated, embarrassed, and angry. However, fearful that they will lose control or be viewed as weak or ineffectual, they often hide their feelings and display an outward confidence that may lead others to believe that everything is fine. They worry about what the future holds for their children, their ability to manage the increasing financial burden, and the daily disruptions of the entire family (Davies, Gudmundsdottir, Worden, et al., 2004; Swallow, Macfadyen, Santacroce, et al., 2012). Some fathers use work as an escape to dull the pain. In addition to withdrawal, common coping strategies are problem oriented and include praying, getting information, looking at options, and weighing choices (Mastroyannopoulou, Stallard, Lewis, et al., 1997).


Results of studies on how siblings are affected by having a brother or sister with a complex condition are unclear (Anderson and Davis, 2011; Barlow and Ellard, 2006). Generally evidence shows a negative effect on siblings of children with chronic illnesses compared with siblings of healthy children (Gold, Treadwell, Weissman, et al., 2011). Siblings of children with chronic illnesses report psychosocial problems more often than their peers (Gold, Treadwell, Weissman, et al., 2011; Rossiter and Sharpe, 2001). A number of factors increase the risk of negative effects for siblings of ill children. Responsibility for caregiving, differential treatment by parents, and limitations in family resources and recreational time are often the experiences of siblings of ill or disabled children (Lobato and Kao, 2002) (Box 36-4).

Box 36-4

Supporting Siblings of Children with Special Needs

Promote Healthy Sibling Relationships

• Value each child individually and avoid comparisons. Remind each child of his or her positive qualities and contribution to other family members.

• Help siblings see the differences and similarities between themselves and the child with special needs. Create a climate in which children can achieve successes without feeling guilty.

• Teach siblings ways to interact with the child.

• Seek to be fair in terms of discipline, attention, and resources; require the affected child to do as much for himself or herself as possible.

• Let siblings settle their own differences; intervene only to prevent siblings from hurting one another.

• Legitimize reasonable anger. Even children with special needs behave badly sometimes.

• Respect a sibling’s reluctance to be with or to include the child with special needs in activities.

Help Siblings Cope

• Listen to siblings to let them know that their thoughts and suggestions are valued.

• Praise siblings when they have been patient, have sacrificed, or have been particularly helpful. Do not expect them to always act in this manner.

• Acknowledge the personal strengths that siblings have and their ability to cope with stress successfully.

• Provide age-appropriate information about the child’s condition and update it when appropriate.

• Let teachers know what is happening so they can be understanding and helpful.

• Recognize special stress times for siblings and plan to minimize negative effects.

• Schedule special time with siblings; have a friend or family member substitute when parent is unavailable.

• Encourage siblings to join or help establish a sibling support group.

• Use the services of professionals when needed. If parent thinks that such a service is necessary, it should be provided in as vigorous a manner as a service for the child with special needs.

Involve Siblings

Data from Powell T, Ogle P: Brothers and sisters—a special part of exceptional families, Baltimore, 1985, Paul H Brooks; Spokane Washington Deaconess Medical Center, Pediatric Oncology Unit: Tips for dealing with siblings, Candlelighters Childhood Cancer Found Q Newslett 11(3,4):7, 1987; and Carlson J, Leviton A, Mueller M: Services to siblings: an important component of family-centered practice, ACCH Advocate 1(1):53–56, 1993.

An important factor in sibling adjustment and coping is information and knowledge regarding their brother’s or sister’s illness or complex condition. What siblings piece together or overhear is often much worse than the truth. Often they imagine gruesome things regarding the experiences related to the illness, treatment, and hospitalization (Shepard and Mahon, 2000). Latino siblings have reported less accurate information about their siblings’ condition than non-Latino siblings (Lobato, Kao, and Plante, 2005). Parents are usually in the best position to impart information, although they are often overwhelmed with the medical crisis at hand (Fleitas, 2000). Nurses can encourage parents to talk with the siblings about how they perceive their sick brother or sister and to be accepting of the siblings’ feelings. Nurses can be ideal educators and counselors of siblings during the course of their brother’s or sister’s illness (Shepard and Mahon, 2000).

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Sep 16, 2016 | Posted by in NURSING | Comments Off on Chronic Illness, Disability, and End-of-Life Care

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