P0529 - Challenges for Children Living with Complex Chronic Disease: It Takes a Village

Introduction: Children with complex, chronic disease face hospital readmissions, considerations for advanced technologies and fractured care coordination. This case highlights the impactful role of palliative medicine in general pediatrics.
Case

Discussion: 16 mo female infant with congenital Zika virus infection required admission for RSV bronchiolitis and aspiration pneumonia. PMH: severe microcephaly with skull collapse, hydrocephaly, periventricular calcifications, chorioretinal scars; 3 brief admissions due to irritability and seizures. G-tube and tracheostomy considered due to severe failure to thrive, neurologic impairment and upper airway compromise. The infant’s care involved 14 subspecialties and her pediatrician. Palliative medicine was consulted at 18 months for assistance in defining goals of care. Biological mother lost custody. Infant cared for by godparent. Mother remained involved but often difficult to contact, limiting ability to have meaningful care discussions. Mother was distrustful of the medical team, had frequent outbursts then would disappear.
Palliative Care Team advocated for key subspecialties and family meeting to elucidate expectations of technologic support with worsening neurologic status and identify goals for care. Medical team pessimistic about trach and G-tube benefits. Mother and godmother acknowledged their understanding of the devastating neurologic status but expressed wish to treat her as a normal child.
Infant’s respiratory status improved with fluid limitation and anticholinergic medication; enteral feeds poorly tolerated. Team advised family that artificial support and resuscitation unlikely to meet care goals. Mother disappeared prior to obtaining consent for hospice care. Godmother wished infant discharged home with intravenous fluids, the only mode of hydration tolerated. Pediatrician provided constant reassurance; Palliative Medicine provided support/advice about symptom management. 42 days following discharge, infant became cyanotic with irregular breathing. Godmother expressed comfort in “letting her go.” Palliative medicine physician arrived at home and determined infant actively dying. Extremely agitated biological mother appeared suddenly. Physician calmly counseled that infant was dying. Death occurred moments after mother embraced daughter. EMS and police notified since DNR not signed. Physician advised that death was expected.

Discussion: This case highlights the pivotal role of the primary pediatrician, emphasizes challenges of coordinating sub-specialties, and accentuates importance of timely, frank medical discussions. Critical care specialists were hesitant to suggest tracheostomy yet opined it might minimize episodic respiratory failure. Hospitalists were frustrated by delays in gastrostomy placement, yet this was fortuitous since the infant had severe feeding intolerance. Family dynamics perplexed the medical team. Mother’s erratic demeanor generated anxiety. Our “village” involved the pediatrician who was impactful in garnering maternal trust; special immunology “ Zika Team”; social workers; and a consultant palliative care service able to circumnavigate care from the clinic, hospital, home and communicate directly with prehospital providers. Collaboration is indispensable, requiring continual adaptation due to challenges with palliative care access and paucity of robust pediatric hospice resources.