J Korean Acad Rehabil Med.  2010 Jun;34(3):347-354.

A Research on the Management of Ventilatory Insufficiency in Patients with Neuromuscular Diseases

• Kim DH
• Kang SW
• Choi W
• Moon JH
• Baek JH
• Choi SH
• Park JH
• Shin YB
• Seo JH

Abstract

OBJECTIVE
To investigate the real condition of pulmonary rehabilitation for patients with advanced neuromuscular diseases (NMDs) on mechanical ventilation in Korea. METHOD: In order to estimate current state of pulmonary rehabilitative management, chart review and pulmonary function evaluation were conducted in a total of 267 NMD patients who had applied mechanical home ventilator in our center from March 2001 to December 2008.
RESULTS
Total 267 patients were included: 95 with Duchenne muscular dystrophy, 69 with other types of myopathy, 83 with amyotrophic lateral sclerosis (ALS), 20 with spinal muscular atrophy. Among them, 18 who were previously intubated and 17 patients who had undergone tracheostomy were switched into volume-limited non-invasive ventilation (NIV). At the time of hospital discharge, 234 patients were applied NIV successfully. Twenty other patients who once used continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) ventilators inappropriately were switched into volume-limited NIV. However, 20 patients who had successfully applied NIV first underwent tracheostomy due to exacerbation of underlying disease.
CONCLUSION
Adequate pulmonary management is the only promising method to prevent lethal complications, and to prolong life span of advanced NMD patients. We assume that more NMD patients can improve their quality of life and prolong their life through proper pulmonary rehabilitation including regular pulmonary function check-ups and ventilatory state monitoring as well as early NIV application.

Keyword

Neuromuscular disease; Non-invasive ventilation; Pulmonary rehabilitation; Mechanical ventilation

MeSH Terms

Amyotrophic Lateral Sclerosis
Continuous Positive Airway Pressure
Humans
Korea
Muscular Atrophy, Spinal
Muscular Diseases
Muscular Dystrophy, Duchenne
Neuromuscular Diseases
Noninvasive Ventilation
Quality of Life
Respiration, Artificial
Tracheostomy
Ventilators, Mechanical