HSC R & D Division Cochrane Fellowship: Prevention and treatment of steroid induced osteoporosis in Duchenne muscular dystrophy

HSC R & D Division Cochrane Fellowship

Dr Jenny Bell, ICL Research Fellow based at the Centre for Infection and Immunity, School of Medicine, Dentistry and Biomedical Sciences, has been awarded a two year Health Research Board/HSC R&D Division Cochrane Fellowship, in order to work on a review of Prevention and treatment of steroid induced osteoporosis in Duchenne muscular dystrophy.

Duchenne Muscular Dystrophy (DMD) is the second most prevalent, genetic neuromuscular disease in Northern Ireland (Hicks et al., 1996). In the UK it affects about 1 in 3500 of boys born. Most boys are independently mobile in the early years but by the age of ten a wheelchair may be required. Rapid deterioration of muscle function leads to an untimely death of DMD patients in late teens and early twenties. There is no cure for DMD as yet.

Steroid therapy has slowed the progression of DMD by improving muscle strength.  Unfortunately one side effect of steroids is the thinning of bones (secondary osteoporosis) which leads to increased risk of fractures, often long bone or vertebral. Vertebral fractures are very painful. Further mediation is required for pain relief and the patient’s mobility is limited. Patients receiving steroids are advised to take Calcium and Vitamin D supplements to improve bone health. However, greater improvement of bone health has been reported when bisphosphonate therapy is combined with steroids in the treatment of DMD patients.

Regionally, the quality of life and longevity experienced by DMD patients is poor due to wide variations in the standard of care. Life expectancy for those with DMD in the UK is half that of those in Denmark.  We are part of Improving Children’s Lives; an interdisciplinary child-focused research initiative with an aim to improve outcomes for the health and well being of children and young people. We feel it is important to review if preventions and treatments, such as bisphosphonates, calcium and vitamin D for steroid induced osteoporosis in DMD reduce the risk of vertebral fractures.  This Cochrane review will take place over the next two years and involves a multidisciplinary team of experts in Northern Ireland and the US.


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