Anticipating Death in Children and Adults with Childhood Onset Severe Central Nervous System Impairment: A Case Series Review (FR480)

Abstract

•Discuss problems due to severe central nervous system impairment that can remain intractable to treatment options.•Define tipping points that can prompt anticipatory discussions, including suggested recommendations that best meet goals.•Utilize language strategies that prepare families when prognosis is not possible. Little is written about trajectories and features that precede death in those with childhood onset severe central nervous system (CNS) impairment. This session will cover this topic through a review of deaths at a long-term care facility for children and young adults with severe neurological impairment and associated complex medical care needs. Of the more than 30 deaths, the majority (70%) were anticipated and guided by goals of care. Circumstances that prompted discussions included: problems that can be attributed to the CNS and remained intractable to various interventions, including seizures, recurrent intestinal pseudo-obstruction (hypothalamus and medulla), pain (thalamus), agitation (loss of CNS inhibition), and edema (medulla); increasing symptom burden; and gradual changes in alertness and ability to participate in activities. Details that preceded unexpected deaths will also be reviewed, including several with acute ileus who died following transfer to a hospital. Details will include: the length of time from the redirection of care plans until death, symptom management at the end-of-life, results of tests, prolonged survival following discontinuation of mechanical ventilation, changes in medical nutrition and hydration, and differences in planned vs unexpected deaths. Outcomes in those who survived using the same noninvasive management strategies will be included given the lack of prognostic ability, a time when parents were prepared for their child’s potential death, and how parents responded before and after this experience. This is an important session given the lack of prognostic information, placing such individuals at significant risk for invasive testing and interventions, including surgery, at the end of life. Consideration of testable and “fixable” problems versus problems that can be due to the CNS without confirmatory tests will be discussed. This information will be used to outline “tipping points” that can prompt anticipatory discussions with parents and guardians, with suggested language offered.