Survival probabilities of patients with childhood spinal muscle atrophy.

OBJECTIVE: Medical and technological advances over the past 2 decades have resulted in improved patient care for children with spinal muscular atrophy (SMA). The objective of the present study was to describe changes in the life expectancy of pediatric patients with SMA over time and to compare these findings with previously reported survival patterns.

METHODS: Medical records of all patients diagnosed with SMA over a 16-year period (1989-2005) at Cincinnati Children's Hospital Medical Center were reviewed. Data pertaining to date of birth, type of SMA, medical and surgical interventions, pulmonary complications, and date of death were obtained.

RESULTS: Kaplan-Meier survival analyses showed a significant improvement in survival probabilities in the severest form of SMA.

CONCLUSIONS: We found a positive trend in the survival of patients with severe SMA. Although we cannot attribute this trend to any single factor, it is likely that advances in pulmonary care and aggressive nutritional support have played a significant role.