P Kieny1, S Chollet, P Delalande, M Le Fort, A Magot, Y Pereon, B Perrouin Verbe. 1. Service de MPR neurologique, hôpital Saint-Jacques, CHU de Nantes, 85, rue Saint-Jacques, 44093 Nantes cedex 1, France; Centre AFM Yolaine de Kepper, 49170 Saint-Georges-Sur-Loire, France; Centre de référence des maladies neuromusculaires rares Nantes Angers, CHU de Nantes, 44093 Nantes cedex 1, France. Electronic address: pierre.kieny@orange.fr.
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.
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 DMDpatients 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 DMDpatients. It allows conservation of a satisfactory quality of life, and should be systematically proposed to patients.
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