Patient D
This patient was a 4-week old infant born at 32 weeks at an outside
hospital due to hydrops fetalis with Trisomy 21 and bilateral chylous
pleural effusion secondary to lymphatic malformation. She was initially
treated with octreotide at the outside facility but due to significant
persistent chylous effusions even after two weeks, she was transferred
to TCH and transitioned here to sirolimus at a starting dose of 0.8
mg/m2/day PO. There was large volume of chest tube output of 550 ml on
day 0 which gradually decreased to 300 ml a week later, to less than 100
ml by day 15 and ultimately 10 ml on day 19, and chest tube removed on
day 20 (Fig 1). Levels were supratherapeutic at the time of chest tube
removal at 23 ng/ml, but no sirolimus-related toxicity noted. The
patient was transitioned to comfort care measures only due to inability
to control fluid overload, and passed away at 3.5 months of age.