Spinal muscular atrophy type 1: Prolongation of survival by noninvasive respiratory aids

John R. Bach, Kavita Gupta, Michael Reyna, Alice Hon

Research output: Contribution to journalReview articlepeer-review

34 Scopus citations

Abstract

Previous reports on spinal muscular atrophy type 1 (SMA-1) underestimate potential survival because of failure to optimally use noninvasive respiratory muscle aids including mechanically assisted coughing and noninvasive ventilation (NIV) at full support settings. We report our center's experience in prolonging survival for these patients. We focus on early initiation of nasal noninvasive ventilation, mechanically assisted coughing, and pulse oximetry monitoring during acute respiratory tract infections to guide use of assistive technologies. Seventeen SMA-1 patients with ventilation via tracheostomy are living, with a mean age of 78.2 (range 65-179) months. Ten died at a mean age of 61.6 (range 16-270) months of age. Twenty five of 27 were not able to regain autonomous breathing ability after the tracheostomy. None of the 21 who had not developed the ability to verbalize before undergoing tracheotomy did so after tracheotomy. Six patients had comprehendible speech at the time of tracheotomy and retained some ability to vocalize subsequently. Seventy-two SMA-1 patients using noninvasive ventilation are alive at mean age 86.1 (range 13-196) months; 13 died at 52.3 (range 13-111) months. Sixty seven of the 75 could communicate verbally. The noninvasive ventilation patients had significantly more hospitalizations than tracheostomy patients until age 3 (P < 0.001) but not thereafter. SMA-1 survival past adolescence is possible using both noninvasive ventilation and tracheostomy ventilation. The tracheostomy-ventilated patients had greater levels of ventilator dependence and reduced verbal abilities.

Original languageEnglish (US)
Pages (from-to)151-161
Number of pages11
JournalPediatric Asthma, Allergy and Immunology
Volume22
Issue number4
DOIs
StatePublished - Dec 1 2009

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Immunology and Allergy
  • Pulmonary and Respiratory Medicine

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