희귀ㆍ난치성 신경근육계 질환자의 호흡부전 관리 실태 조사
A Research on the Management of Ventilatory Insufficiency in Patients with Neuromuscular Diseases
4
조회
0
다운로드
6
인용
0
참고문헌
키워드
초록
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. (J Korean Acad Rehab Med 2010; 34: 347-354)
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. (J Korean Acad Rehab Med 2010; 34: 347-354)
참고문헌 (15)
- Leith DE, "Cough. In Respiratory Defense Mechanisms. Lung biology in health and disease", pp.545~585 (1977), Marcel Dekker
- 강성웅, "말기 신경근육계 환자에서 비침습적 호흡기 관리", Annals of Rehabilitation Medicine, 28(1), pp.71~77 (2004), 대한재활의학회
- 김동현, "신경근육질환 환자에서 비침습적 호흡기 사용 경과 고찰", Annals of Rehabilitation Medicine, 31(4), pp.427~433 (2007), 대한재활의학회
- Misuri G, "Mechanism of CO2 retention in patients with neuromuscular disease", Chest, 117, pp.447~453 (2000)
- Braddom RL, "Physical medicine and rehabilitation", pp.744~746 (2007), Saunders
- Bach J, "Guide to the evaluation and management of neuromuscular disease", pp.113~116 (1999), Hanley & Belfus
- Delisa JA, "Physical medicine & rehabilitation principles and practice", pp.1853~1859 (2005), Lippincott Williams & Wilkins
- 조동희, "신경근육계 질환에서 자세에 따른 폐활량의 변화 양상 비교", Annals of Rehabilitation Medicine, 28(5), pp.454~457 (2004), 대한재활의학회
- Hopkins LC, "Management of ALS: respiratory care", Neurology, 47(2), pp.S123~125 (1996)
- Greenbaum DM, "Continuous positive airway pressure without tracheal intubation in spontaneously breathing patients", Chest, 69, pp.615~620 (1976)
- Brimacombe J, "Pressure support ventilation versus continuous positive airway pressure with the laryngeal mask airway: a randomized crossover study of anesthetized adult patients", Anesthesiology, 92, pp.1621~1623 (2000)
- Bach JR, "Standards of care in MDA clinics. Muscular Dystrophy Association", Am J Phys Med Rehabil, 79, pp.193~196 (2000)
- Bach JR, "Mouth intermittent positive pressure ventilation in the management of postpolio respiratory insufficiency", Chest, 91, pp.859~864 (1987)
- Simonds AK, "Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy", Thorax, 53, pp.949~952 (1998)
- Louwerse ES, "Amyotrophic lateral sclerosis: mortality risk during the course of the disease and prognostic factors. The Netherlands ALS Consortium", J Neurol Sci, 152, pp.S10~17 (1997)