Home Mechanical Ventilation in Childhood-Onset Hereditary Neuromuscular Diseases: 13 Years’ Experience at a Single Center in Korea

• DOI: 10.1371/journal.pone.0122346
• Publication Date: 2015-03-31T09:10:10Z
• Authors: Young Joo Han; June Dong Park; Bongjin Lee; Yu Hyeon Choi; Dong In Suh; Byung Chan Lim; Jong-Hee Chae
• Keywords: Neuromuscular disease

Introduction Children with hereditary neuromuscular diseases (NMDs) are at a high risk of morbidity and mortality related to respiratory failure. The use home mechanical ventilation (HMV) has saved the lives many children NMD but, due lack studies, dependable guidelines not available. We drew upon our experience compare various underlying NMDs evaluate HMV regard morbidity, proper indications timing for its use, develop policy improve quality noninvasive (NIV). Methods retrospectively analyzed medical records 57 childhood-onset in whom was initiated between January 2000 May 2013 Seoul National University Children's Hospital. degree estimated by frequency duration hospitalizations caused distress. Results most common spinal muscular atrophy (SMA, n = 33). Emergent 44% patients before confirmed diagnosis, indicators pre-HMV (e.g., extubation trials, hypoxia, hospitalizations, intensive care unit stay) were greater these than others. proportion post-HMV (range, 0.00−0.52; median, 0.01) lower that (0.02−1.00; 0.99) (P < 0.001). Eight able maintain NIV. main causes NIV failure air leakage large amount airway secretions. Conclusions application helped reduce NMD. Patients SMA type I can benefit from an early diagnosis timely HMV. choice invasive should be based on patient's age trial tolerance. Systematic follow-up provided multidisciplinary team needed.