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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
Summary
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.