Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy.
OBJECTIVE
To evaluate the effects of a new respiratory management protocol on respiratory morbidity and hospitalization rates for patients with Duchenne muscular dystrophy (DMD).


METHODS
A retrospective cohort study.


METHODS
Using a protocol in which oxyhemoglobin desaturation was prevented or reversed by the use of noninvasive intermittent positive pressure ventilation (IPPV) and assisted coughing as needed, the hospitalization rates and days for 24 protocol DMD ventilator users were compared with those of 22 nonprotocol DMD tracheostomy IPPV users.


RESULTS
The 22 conventionally managed patients were hospitalized a mean of 72.2+/-112 days when undergoing tracheostomy. This included a 16.1+/-5.4-day period of translaryngeal intubation. The 24 protocol patients were hospitalized a mean of 6.0+/-2.4 days (p<0.005) when beginning ventilator use. Over their next 126.2 patient-years of ventilator use, the 24 protocol patients had significantly lower rates of hospitalization (p<0.008) and hospitalization days (p<0.005) than had the tracheostomy IPPV users over a 167.2 patient-year period. This is true although 14 of the 24 protocol patients went on to require 24-h noninvasive IPPV for 4.5+/-3.6 years. Five of the 14 have yet to be hospitalized.


CONCLUSIONS
The use of inspiratory and expiratory aids can prolong survival while significantly decreasing the pulmonary morbidity and hospitalization rates associated with conventional resort to tracheostomy IPPV.
Bach
J R
JR
Department of Physical Medicine and Rehabilitation, Jerry Lewis Muscular Dystrophy Association Clinic, University of Medicine and Dentistry of New Jersey-New Jersey Medical School, Newark 07103, USA.
Ishikawa
Y
Y
Kim
H
H
D000293:Adolescent; D000328:Adult; D002648:Child; D015331:Cohort Studies; D003371:Cough / Q000503:physiopathology; D005069:Evaluation Studies as Topic; D005500:Follow-Up Studies; D006760:Hospitalization; D006801:Humans; D001239:Inhalation / Q000502:physiology; D007385:Intermittent Positive-Pressure Ventilation; D007442:Intubation, Intratracheal; D007902:Length of Stay; D008171:Lung Diseases / Q000209:etiology / Q000517:prevention & control*; D009136:Muscular Dystrophies / Q000150:complications*; D010100:Oxygen / Q000097:blood; D010108:Oxyhemoglobins / Q000032:analysis; D012119:Respiration / Q000502:physiology; D012138:Respiratory Therapy; D012189:Retrospective Studies; D014139:Tracheostomy; D016896:Treatment Outcome; D012122:Ventilators, Mechanical
D010108:Oxyhemoglobins; D010100:Oxygen