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

W A Stewart1, K E Gordon, P R Camfield

  • 1Department of Pediatrics, Division of Neurology, Dalhousie University, IWK Health Centre, Halifax, Nova Scotia, Canada.

Pediatric Neurology
|November 13, 2001
PubMed

Insights

Krabbe disease, a rare neurological disorder, causes severe irritability in infants. Low-dose morphine effectively managed this challenging symptom in two infants, offering a potential new treatment avenue.

Area of Science:

  • Neurology
  • Pediatrics
  • Genetics

Background:

  • Krabbe disease is a rare, fatal lysosomal storage disorder.
  • It is characterized by progressive neurodegeneration and severe irritability in infants.
  • Current management options for irritability are limited and often ineffective.

Observation:

  • Two female infants presented with symptoms of Krabbe disease at 5 months of age.
  • They exhibited increasing irritability, motor control issues, and developmental regression.
  • Standard treatments for irritability, including reflux management and nitrazepam, were unsuccessful.

Findings:

  • Low-dose morphine was administered to both patients.
  • Patient 1 received 0.06 mg/kg every 6 hours, and Patient 2 received 0.1 mg/kg every 8 hours.
  • Morphine resulted in remarkable and successful control of irritability in both infants.

Implications:

  • This study suggests low-dose morphine as a viable option for managing intractable irritability in Krabbe disease.
  • Effective irritability management can significantly improve the quality of life for affected infants and their families.
  • Further research is warranted to explore the long-term efficacy and safety of morphine in Krabbe disease management.

Related Concept Videos