Prognosis

Cardiac and respiratory complications are seen, especially with disease progression, and if untreated DMD typically leads to death in the late teens (1).

Multidisciplinary care, particularly the initiation of corticosteroids and assisted ventilation supported by regular cardiac and respiratory monitoring, has led to increasing numbers of patients living into adulthood in recent years - however death often occurs in the mid-20s.

Life expectancy for patients with DMD has improved during the last decades, however, it is still poor, with a 2020 systematic review and meta-analysis of studies in Organisation for Economic Co-operation and Development (OECD) countries finding a median life expectancy between 21.0 and 39.6 years, provided patients received ventilatory support (4)

• similarly, a 2016 chart review of all deaths in the DMD population in North East England found that the mean age of death caused by underlying cardiac or respiratory failure was 23.9 years (5)

Reference:

• Rodger S et al. Adult care for Duchenne muscular dystrophy in the UK. J Neurol. 2015; 262(3): 629–6
• Fox H et al. Duchenne muscular dystrophy. BMJ 2020;368:l7012
• Lisak RP, Truong DD, Carroll W, Bhidayasiri R (2011). International Neurology. Wiley. p. 222. ISBN 9781444317015.
• Landfeldt E, Thompson R, Sejersen T, McMillan HJ, Kirschner J, Lochmuller H. Life expectancy at birth in Duchenne muscular dystrophy: a systematic review and meta-analysis. European Journal of Epidemiology. 2020;35:643-653.
• Van Ruiten HJA, Marini Bettolo C, Cheetham T, et al. Why are some patients with Duchenne muscular dystrophy dying young: An analysis of causes of death in North East England. European Journal of Paediatric Neurology. 2016;20(6):904-909