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On another typical call night at the hospital, I open up the emergency department (ED) board and recognize a familiar name: “Emma, 4-year-old female in respiratory distress.” A moment later, my pager buzzes: “Please call back regarding a consultation and potential admission.” Emma, well-known to our institution, is a 4-year-old girl with methylmalonic acidemia who is tracheostomy-dependent, ventilator-dependent, and gastrostomy tube–dependent, and nights like tonight are all too familiar for her and her mother. Emma’s story began not here with us, but rather, 4 years ago and 50 miles away in a community hospital ED, as 2 frightened first-time parents stood in the waiting room holding their four-day-old infant girl. All had been well during the pregnancy and nursery stay, but by her third day of life, Emma had become more and more difficult to rouse to feed. Her breathing had begun to appear labored, and her body seemed limp. This family, who had never had occasion to visit their local ED, found themselves suddenly surrounded by a team of physicians and nurses with worried expressions. The team had worked earnestly to stabilize her, first treating her glucose level of 24 mg/dL and bicarbonate level of 5 mmol/L and ultimately intubating her when her breathing became too rapid to support oxygenation. After she failed to improve despite the initial resuscitation, the ED physicians had consulted the in-house pediatric hospitalist, who had requested an ammonia level, which returned at 480 μg/dL. The hospitalist had recommended changing the fluids to include dextrose 10%, increasing the rate to suppress catabolism, and finally transferring Emma to the quaternary care pediatric …
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