Patient A
This patient was a term infant girl who had a history of transient
myeloproliferative disorder (TMD) of Down syndrome in the first couple
of weeks of life, and Atrial Septal Defect. Shortly after the TMD
resolved, she was found to have a chylous pleural and pericardial
effusion and CCLA was diagnosed by MR lymphangiogram. Sirolimus was
initiated at 3 weeks of age. The initial dose of sirolimus was 1.6
mg/m2/day via NG tube. This patient had 3 chest tubes at the beginning
of sirolimus therapy. Chest Tube #1 had initial drainage of 102 mL
noted on day 8 which decreased to 33 mL by day 13, and further minimized
until chest tube removal on day 17. Chest Tube #2 had initial output of
130 mL on day 12. Drainage was minimal by days 17-20 with eventual
removal on Day 20. Chest Tube #3 had 8 mL output on day 17 and tube was
removed the next day. Sirolimus level was supratherapeutic on days 2,
12, 15, and 16 at 24, 29, 28 and 21 ng/ml respectively. Day 12
supratherapeutic levels coincides with increased output from Chest Tube
#2 and days 15 and 16 with increased output from Chest Tube #3 (Figure
1). She was discharged home on sirolimus without chest tubes and
clinically stable almost one month later. The patient was re-admitted at
8 months of age due to acute respiratory failure secondary to human
metapneumovirus pneumonia and passed away.