Despite best efforts, children living with chronic, life-threatening, and terminal illnesses experience substantial suffering. This experience of serious disease is multidimensional. It has broad-ranging implications in the child’s life as a whole and in the family as a functional unit. Suffering results from a threat to one’s physical and psychological self, from a threat to one’s relationships with others, and from a threat to one’s relationship with a transcendent source of meaning (7). Suffering is a profoundly personal experience and is endurable, and at times even fulfilling, when it becomes meaningful (8). Serious illness threatens children’s sense of personal integrity and shatters all aspects of their lives. Physical pain and other symptoms cause fear, depression, and isolation. Illness affects their daily activities, sense of well-being, physical strength and agility, and the motives and quality of their relationships. Disease crushes their sense of security, and brings fears of the unknown, rejection,
and punishment. Children also become confused by the experience of a mixed variety of emotions of anger, anxiety, sadness, loneliness, and isolation in the presence of a threatening situation (9). Children are highly vulnerable to the stress inherent to the experience of severe illness. They have an egocentric view of the world and lack a fully developed repertoire of coping mechanisms, such as problem solving or decision making, which are influenced by age-dependent behavior and cognitive abilities.

Within the physical realm, the published experience speaks best to those with malignant diseases. Recent studies by Wolfe (10) and Collins et al. (11, 12). point to the wide variety and high prevalence of symptoms from which children with life-threatening illness suffer. There appears to be significant discrepancies between the reports of parents and physicians regarding the children’s symptoms in the last month of life, with parents reporting each symptom more than the physicians. Furthermore, currently available treatments may not be successful in easing suffering associated with these symptoms that can cause significant distress. For children with nonmalignant diseases, some of the more troublesome symptoms might include gastroesophageal reflux, neuroirritability, immobility, incontinence, seizures, muscle spasms, pressure ulcers, contractures, recurrent infections, increased secretions, restlessness, sleep disturbance, and edema. There are few valid and reliable tools available to assess these symptoms and little data to substantiate the use of many of the interventions currently prescribed.

Suffering, for the parents of a child with a life-threatening illness, can also be a multidimensional experience of pain, fear, failure, despair, powerlessness, hopelessness, purposelessness, and vulnerability. Parental anxiety is due in part to the changing parent-child structure, the need to understand the illness experience, become familiar with the hospital environment, adapt to the changing relationships with their child and other family members, and negotiate with professionals about their care (13, 14). Parents must also deal not only with the immediate threat of disease on their child’s life, but also with important additional family stressors during treatment such as lifestyle changes, marital tension, financial strain, loss of self-esteem, and even loss of sleep (15). Furthermore, when confronted with the suffering and possible death of their child, parents frequently recognize their own limitations and mortality. Their perception of life, death, and the world around them is changed dramatically by the reality of the loss of their child. In addition, parents must also satisfy the emotional needs of other children in the family which many times parallel those of the seriously ill child (16). Finally, children and their families may also suffer spiritually. This may be manifested as a sense of isolation and abandonment, a sense of hopelessness and uncertainty about the meaning and ultimate purpose of life.

The American Academy of Pediatrics supports an integrated model of palliative care in which the components of palliative care are offered at diagnosis and continued throughout the course of illness, whether the outcome ends in cure or death (17). Basic principles of pediatric palliative care include the following:

These essentials do not mandate a particular structure for care delivery other than to suggest the function of an interdisciplinary team of health care and allied health care professionals to provide care coordination and to facilitate the delivery of services with the goal to minimize suffering and improve quality of life.

Much of the fragmentation of services that occurs in modern health care systems results from the lack of a coordinating entity. The loss of continuity may be addressed by providing a medical home for these children along with the services of an advanced-illness care coordinator. This may be a registered nurse whose primary responsibilities are to enhance communication across settings, facilitate the participation of the patient and family in the decision-making process, ensure that health care providers adhere to the goals and principles of palliative care, and honor the patient and family’s wishes. The coordinator may also be responsible for ensuring access to proper management of pain, and psychosocial and spiritual support. The advanced-illness care coordinator may advocate for change in the nature of the palliative care interventions according to the stage of disease and the patient and family’s expectations, values, and beliefs. In our experience, provision of a trained advanced-illness care coordinator to facilitate end-of-life communication also increases utilization of and length of stay in end-of-life services and completion of advance directives. The roles provided by a physician advocate and the integration of services facilitated by the advanced care coordinator are essential for maintaining a patient-focused, relationship-centered approach and promotion of palliative care goals (18).

Shared decision making in pediatrics, driven by communication between child, parent, and physician, defines the standard in medical ethics. Clinicians must establish an effective process to ensure that all treatment decisions are made in the best interest of the child. Understanding the illness experience from the perspective of the child and family, establishing accurate prognosis and communicating it effectively, setting reasonable goals and establishing a comprehensive advanced-illness care plan are indispensable steps in this process. Judgments that relate to withholding and/or withdrawing certain therapies, as well as judgments concerning medically inappropriate interventions, are essentially subjective but realistically indispensable (19). There is marked variability among physicians regarding the use of artificial life support, but in general, these decisions must be goal directed and in consonance with the child’s and family’s beliefs and values (20).

Withholding and withdrawing artificial life-sustaining interventions in children, particularly medically provided artificial hydration and nutrition, is often controversial. However, in patients with no hope of regaining some potentiality for function, clinicians may find insufficient justification for continued treatment if the burden of therapy is greater than the benefits to the patient and family (21, 22). Discontinuation of nonbeneficial interventions is squarely within the scope of parental decision-making authority for their child and is not inconsistent with the child’s best interests. Although children are allowed to assent rather than to consent to plans regarding their care, parents and health care providers must recognize the subjective personal nature of suffering and respect the child’s autonomy and capacity to make decisions, particularly for emancipated and mature minors (23). Whenever possible, caregivers must make an effort to invite children to participate in medical decision making and honor their end-of-life care wishes. This is particularly important for any child, regardless of age, who can understand his or her medical condition, who can communicate his or her preferences, and who is able to reach a reasonable decision and can understand its consequences (24). In addition, the principles that guide the rule of double effect and sedation of highly symptomatic patients with pain or dyspnea in adults also apply in the care of children (25). In difficult cases, institutional ethics committees can help resolve conflicts about treatment decisions, provide a forum for discussion of hospital policies, and educate the health care community about ethical concepts (26). Of note, although some alternative medicine practices in the care of seriously ill children may be justified; there are no published guidelines for the use of these practices in children (27). Parents of seriously ill children may be willing to try a variety of approaches, some of which may be potentially harmful, hoping for benefit in a desperate situation, particularly when the condition imposes a heavy burden for which mainstream therapies are insufficient (28). To best serve the interest of children, physicians must maintain a scientific perspective, provide balanced advice about therapeutic options, and establish and maintain a trusting relationship with families (29).