Evolution of life expectancy of patients with Duchenne muscular dystrophy at AFM Yolaine de Kepper centre between 1981 and 2011

Abstract Objectives Retrospective study over the last 30 years of life expectancy in patients suffering from Duchenne muscular dystrophy (DMD). Analysis of the role of ventilatory assistance and causes of death. Patients and methods One hundred and nineteen adult DMD patients were hosted during 1981...

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Bibliographic Details
Published in:Annals of physical and rehabilitation medicine 2013-09, Vol.56 (6), p.443-454
Main Authors: Kieny, P, Chollet, S, Delalande, P, Le Fort, M, Magot, A, Pereon, Y, Perrouin Verbe, B
Format: Article
Language:English
Subjects:
Adolescent
Adult
Assistance ventilatoire
Cause of Death - trends
Duchenne muscular dystrophy
Dystrophie musculaire de Duchenne
Espérance de vie
Humans
Internal Medicine
Kaplan-Meier Estimate
Life Expectancy - trends
Muscular Dystrophy, Duchenne - mortality
Muscular Dystrophy, Duchenne - therapy
Physical Medicine and Rehabilitation
Respiration, Artificial - statistics & numerical data
Retrospective Studies
Survival
Tracheotomy
Trachéotomie
Ventilatory assistance
Young Adult
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Summary:Abstract Objectives Retrospective study over the last 30 years of life expectancy in patients suffering from Duchenne muscular dystrophy (DMD). Analysis of the role of ventilatory assistance and causes of death. Patients and methods One hundred and nineteen adult DMD patients were hosted during 1981 to 2011 at AFM Yolaine de Kepper centre, Saint-Georges-sur-Loire, France. Patients’ life expectancy was calculated using Kaplan-Meier model. Results Life expectancy without or with ventilatory assistance was 22.16 and 36.23 years, respectively. Similarly, life expectancy of patients born from 1970 (mostly with ventilatory assistance) was 40.95 years old from 1970 and 25.77 years old before 1970. Causes of death changed. Cardiac origins of death have increased from 8% to 44%. Conclusion Ventilator assistance, in this study mostly through tracheotomy prolongs by more than 15 years life expectancy of DMD patients. It allows conservation of a satisfactory quality of life, and should be systematically proposed to patients.
ISSN:1877-0657
1877-0665
DOI:10.1016/j.rehab.2013.06.002