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Published on: July 29, 2014
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.
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.
Abstract:
Krabbe's disease is characterized by normal neonatal development with subsequent regression and profound, medically intractable irritability. Two female infants presented at 5 months of age with increasing irritability, abnormal motor control, and developmental regression. Investigations confirmed the diagnosis of Krabbe's disease. Maximal treatment of gastroesophageal reflux and nitrazepam 0.1 mg/kg by mouth three times daily were unsuccessful in 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 compounded by the marked irritability. Management is difficult, but in these two infants, irritability was successfully controlled with low-dose morphine.

