Irritability in Krabbe's disease: Dramatic response to low-dose morphine

Krabbe's disease is characterized by normal neonatal development with subse quent regression and profound, medically intractable irritability. Two fema le infants presented at 5 months of age with increasing irritability, abnor mal motor control, and developmental regression. Investigations confirmed t he diagnosis of Krabbe's disease. Maximal treatment of gastroesophageal ref lux and nitrazepam 0.1 mg/kg by mouth three times daily were unsuccessful i n controlling irritability. Morphine was initiated and titrated to 0.06 mg/ kg by mouth every 6 hours in Patient 1 and 0.1 mg/kg by mouth every 8 hours in Patient 2, resulting in remarkably successful control of irritability. The diagnosis of Krabbe's disease is devastating for families and is compou nded by the marked irritability. Management is difficult, but in these two infants, irritability was successfully controlled with low-dose morphine. ( C) 2001 by Elsevier Science Inc. All rights reserved.