Palliative Care for Infants, Children, and Adolescents: A Practical Handbook

6. Decision Making

139 Key points discussed in this chapter include the following: • The “right decision” for a given child and family will evolve over time; decision making is a process that accommodates ever-changing facts and perceptions rather than a one-time event. • Avoidance of advance care planning usually results in less-than-optimal care and long-term outcomes for the patient and family. • The child, nuclear and extended family, and health care team are all integral members of the decision-making team. Managing a life-limiting or life-threatening condition in partnership with an affected child and family inevitably requires making choices that vary in complexity, immediacy, and perceived significance; rarely is there a single “right” answer. Through clinical vignettes and focused summary of the literature , this chapter identifies factors involved in decision making for children living with life-threatening conditions and offers practical suggestions to optimize the process. Three families are each informed that their infant has spinal muscular atrophy type I. The diagnosis was made when upper respiratory illnesses resulted in hospitalization for each child. The infants, Abigail, Bailey, and Carmen, had each been a little “floppy,” but nothing serious was initially suspected. After neurologic, pulmonary, and genetic consultations, each family is referred to palliative care services for the purpose of engaging in advance care planning, including a discussion of goals of care and their associated treatment options. Decision Making Robert J. Graham, M.D., Marcia Levetown, M.D., and Margaret Comeau, M.H.A. The heart has reasons that reason cannot know. —Blaise Pascal 6 140 the cycle of care Given the inevitability of death during childhood for the child diagnosed with spinal muscular atrophy, regardless of medical interventions, the following topics should be addressed over time with these families and others whose children have similarly fatal conditions: 1. The name of the condition, how and why it occurs (when known), whether and how it is heritable, reversible, or treatable (should be discussed in the initial conversations). 2. The range of usual life expectancy. 3. The anticipated illness trajectory, probable symptoms, associated quality-of-life challenges for the child and family, and likely mechanisms of death. 4. Types of disease-directed treatment available, associated short- and long-term benefits (certain and hoped for), and known and potential burdens. In the case of spinal muscular atrophy, this discussion must include the pros and cons of various forms of assisted ventilation and artificial nutrition and hydration. 5. Discussion of illness complications, including their mechanisms, preventive measures, and outcomes. In the case of spinal muscular atrophy, this includes a discussion of aspiration pneumonitis. 6. Discussion of means to prevent and ameliorate the child’s discomfort and suffering, as well as maximize the quality of life for the child and family. In the case of spinal muscular atrophy, this includes managing fever, weight loss, and dyspnea, as well as helping families connect with similarly affected families and community-based supports. 7. Discussion of the impact of the illness on the family and potential means to enhance their quality of life. Abigail’s parents discuss her prospects with their extended family, minister, and other families affected by spinal muscular atrophy that they “meet” online. They decide to pursue a gastrostomy tube during this admission and discuss plans for nocturnal and nap-time BiPAP when needed. They are, however, hesitant about the idea of a tracheostomy tube, stating that they want Abigail to be free of a breathing machine when awake to help her be as “normal” as possible. Bailey’s family feels that their son would suffer unnecessarily by undergoing invasive procedures. If he will never be able to hold his head up or suck his thumb and “death is inevitable,” they do not want machines to prolong his life. After discussing their son’s diagnosis and their own decision making 141 philosophy with their primary pediatrician, they elect to go home with hospice once he recovers from his acute respiratory decompensation. They do not want a feeding tube, “even the one in the nose,” and do not want an oxygen saturation monitor at home. They have no interest in initiating mechanical ventilation. Carmen’s family discusses their realistic options with their social worker and family. They request the placement of a gastrostomy tube now and ask if she can be at home with a breathing tube (i.e., tracheostomy tube) and ventilator when she gets weaker. They...