Decision making

Abstract

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. 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 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 are interested in any treatments, surgeries, or technology that will keep Carmen at home with her family and alive as long as possible. Is it reasonable for these families to come to differing conclusions regarding goals of care, given the same diagnosis? Is it reasonable for a health care provider to promote a plan of care supporting a child's quality of life based on the family's perception, even at risk of that life being briefer? Is this decision wrong if other families in the same situation choose to "do everything" to extend the child's life? What role, if any, do the values of health care providers play in the decision-making process? If there is a conflict of values between families and health care providers, or among the providers themselves, how should these be resolved? Health care decision making is evolving from the historically paternalistic (Todres et al., 1977, 2000; Todres, 1992) style to inclusive, family-centered paradigms (King, Rosenbaum, and King, 1997; Cuttini et al., 1999; AAP COHC, 2003) ideally built on informed consent and mutual participation (Szasz and Hollender, 1956; Curley, 1988). Decision making is, at its core, a shared experience and a shared responsibility of the child (as willing and able), the family, and the health care team. The child's primary care physician or medical home should be an integral part of this team, particularly when the child is likely to receive care in the community. This physician will be intimately familiar with existing community resources and can serve as the core of information and service coordination. While families still appropriately depend on and desire health care providers to educate and advise them, the expectation is that the family's informed and considered values will direct the ultimate choices (Meert et al., 2008). This is not because families "own" their children, but because children are raised in the context of the family; thus, families are considered the best surrogate decision makers for their minor children. Each child and family possesses a unique set of values, knowledge, and cultural expectations regarding health, health care, and disability. It is the job of the health care team to elicit these values and preconceptions as they relate to the child's health care situation, to provide education, and to discuss options, making recommendations based on the family's priorities and on the knowledge and experience of the team. Further, the provision of support and respect for the child and the family is a critical element of creating a functional decision-making team. It is also important to remember that the family lives with the outcome of the choice forever; to a lesser extent, so does the health care provider. While advance care planning is not easy and may even be painful, the ultimate goals are improved care outcomes and greater family satisfaction. Studies of families of children who have special health care needs indicate that there are substantial benefits to engaging in a thoughtful and timely process of advance care planning (Wharton et al., 1996; Hammes et al., 2005). Parents in these studies reported that the process of advance care planning • ensured their child's and family's best care; • provided time and information to make the best decisions; • helped them communicate the desired care outcomes; • provided peace of mind; and even • prompted them to complete their own advance directives. Given the same set of medical facts, the "right" advance care plan can vary from patient to patient. In addition to the illness-related factors already discussed, other key factors in health care decision making for children include • the age, capacity, and maturity of the child; • the family's values, experiences, concerns, beliefs, and priorities, including cultural and religious perspectives; • perceptions of mutual respect and acknowledgement of emotions; • practical factors, including finances and available services; • pre-existing and illness-influenced family dynamics. © 2011 by The Johns Hopkins University Press. All rights reserved.