[18 years experience with mechanical ventilation in patients with Duchenne muscular dystrophy].
OBJECTIVE
To find out which patients with Duchenne muscular dystrophy are eligible for starting home mechanical ventilation and what the survival rate is.


METHODS
Retrospective.


METHODS
In 48 patients with Duchenne muscular dystrophy who were treated with home ventilation from 1987, the results were assessed in the follow-up visit in February 2005. Initially, ventilation was only given through a tracheotomy (TPPV), but after starting up a multidisciplinary neuromuscular consultation, non-invasive ventilation (NIPPV) was offered in an earlier stage of the disease. The following data were derived from the outpatient medical record: indication for ventilation, vital capacity (VC), arterial blood gas values, duration of ventilation up to February 2005, survival and causes of death.


RESULTS
15 patients died. The 5-year survival rate was 75% from the start of mechanical ventilation and 67% (18/27) of the patients were still living at home at the time of the follow-up visit. The most important causes of death were cardiomyopathy (5/15) and tracheal bleeding (3/15). The group of patients who started ventilation before 1995 (n = 17) had a significantly smaller VC than the group (n = 31) who started after the neuromuscular consultation was set up. The PaCO2 during daytime was significantly higher in the group that started ventilation before 1995 compared to the group that started later.


CONCLUSIONS
Home mechanical ventilation can be implemented effectively in patients with Duchenne dystrophy, with a 5-year survival of 75%.
Meinesz
A F
AF
Universitair Medisch Centrum Groningen, Postbus 30.001, 9700 RB Groningen, Centrum voor Thuisbeademing, afdeling Longziekten.
Bladder
G
G
Goorhuis
J F
JF
Fock
J M
JM
Staal-Schreinemachers
A L
AL
Zijlstra
J G
JG
Wijkstra
P J
PJ
D000293:Adolescent; D000328:Adult; D002423:Cause of Death; D002648:Child; D005260:Female; D006699:Home Care Services; D006801:Humans; D007385:Intermittent Positive-Pressure Ventilation / Q000379:methods*; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology / Q000628:therapy*; D009468:Neuromuscular Diseases / Q000503:physiopathology / Q000628:therapy*; D012121:Respiration, Artificial / Q000379:methods*; D012131:Respiratory Insufficiency / Q000628:therapy*; D012189:Retrospective Studies; D016019:Survival Analysis; D014797:Vital Capacity