Medical Considerations of Long-Term Survival of Werdnig–Hoffmann Disease

@article{Bach2007MedicalCO,
  title={Medical Considerations of Long-Term Survival of Werdnig–Hoffmann Disease},
  author={John R Bach},
  journal={American Journal of Physical Medicine \& Rehabilitation},
  year={2007},
  volume={86},
  pages={349-355}
}
  • J. Bach
  • Published 1 May 2007
  • Medicine
  • American Journal of Physical Medicine & Rehabilitation
Bach JR: Medical considerations of long-term survival of Werdnig–Hoffmann disease. Am J Phys Med Rehabil 2007;86:349–355. Objectives:To report intercurrent nonrespiratory complications of unprecedented survival for Werdnig–Hoffman disease (spinal muscular atrophy type 1 [SMA 1]). Design:A retrospective chart review and caregiver questionnaire for 103 consecutively referred SMA 1 patients for whom death was prevented during infancy. Results:Overall, 15 of 63 (23.8%) respondents had severe… 
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NIV and tracheostomy can both prolong survival for SMA 1 patients, but the latter results in continuous ventilator dependence and speech does not develop.
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