Spinal Muscular Atrophy Mortality Despite Novel Medications: Case Reports.
Am J Phys Med Rehabil
; 103(8): e98-e101, 2024 08 01.
Article
em En
| MEDLINE
| ID: mdl-38529620
ABSTRACT
ABSTRACT Despite new effective medications, patients with spinal muscular atrophy types 1-3 can continue to have inadequate cough flows to prevent episodes of acute respiratory failure. Ventilator unweanable intubated patients are thought to require tracheostomy tubes. As a result, potentially beneficial medications may be discontinued and patients die despite receiving these medications. Three cases are presented of medically treated, physically strengthening children, with spinal muscular atrophy type 1. All three subsequently died or underwent tracheotomy. However, there is no evidence of extubation attempts to noninvasive ventilatory support settings or optimal mechanical insufflation-exsufflation despite this option being described to be over 98% successful for extubating unweanable medically untreated children with spinal muscular atrophy 1.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Atrofias Musculares Espinais da Infância
Limite:
Humans
/
Male
/
Newborn
Idioma:
En
Ano de publicação:
2024
Tipo de documento:
Article