Case 3 History ---- The patient was the product of a term gestation (38-3/7-week and 2615 gram) to a then 21-year-old gravida 1, para 0 woman. Delivery was complicated by fetal decelerations with bloody amniotic fluid noted at rupture of membranes. The patient was initially admitted to Barnes Special Care Nursery where she was noted to be hypertonic and tachypneic with significant metabolic acidosis and was transferred to SLCH where her course was remarkable for neonatal encephalopathy with an abnormal MRI showing extensive diffusion restriction changes. A VP shunt was placed when the patient was one year of age due to severe communicating hydrocephalus. Head CT at age 3 showed profound global brain atrophy. Although she presented with severe metabolic acidosis and had an extensive workup, there has been no unifying diagnosis. She had G tube and had been in hospice since age 4. Her baseline responsiveness was movement of eyes and arms appearing to be intentional in response to touch, but she was severely developmentally delayed and unable to speak or ambulate. She had seizures 2-5 times per week as stiffening and generalized shaking. At the time of her final hospital admission this 7-year-old girl had a history of static encephalopathy (“cerebral palsy”) S/P VP shunt and G-tube with fundoplication. She had refractory seizures, thalamic storming, and intermittent respiratory obstruction with vocal cord dysfunction. One morning her home nurse noticed that the patient started having abnormal eye movements, decreased responsiveness, and increased work of breathing. Her labs and clinical history showed concern for multisystem organ failure, sepsis/bacteremia vs pneumonia and possible intestinal obstruction. Blood and urine cultures were negative. Since the patient was previously DNR/DNI a full discussion about interventions was held with the family and it was decided to discontinue the BiPap and provide comfort care. Upon discontinuation the patient's respiration declined further and she was pronounced dead.
