Development and Implementation of Bone Health Protocol (BHP) in Duchenne Muscular Dystrophy (DMD)

source: American Academy of Neurology

year: 2017

authors: Alexandra Otto, Karen Loechner, Sumit Verma

Objective:
To Develop/Implement BHP for DMD.

Background:
Vitamin D (25OHD) deficiency, reduced bone mineral density (BMD) scores (dual-energy x-ray absorptiometry-DXA), and vertebral compression fractures (VFs) are common in DMD.

Design/Methods:
We retrospectively reviewed electronic medical records of 148 DMD boys for 25OHD, DXA scan frequency and scores, VFs and vitamin D therapies. Prospectively, use of the BHP in 2016 included obtaining 25OHD levels at 6 month intervals and DXA combined with spine radiographs (for those with spinal Z-score < −2.0 to assess for VFs) prior to initiation of steroids and, thereafter, annually. Intervention included treatment with vitamin D2 (ergocalciferol) 50,000 IU /week for 8 weeks for vitamin D deficiency/insufficiency and maintenance vitamin D3 (cholecalciferol) 1,000–2000 IU/day once therapeutic levels obtained. Dietary calcium supplementation augmented.

Results:
At baseline, 72% (107) DMD boys had one or more 25OHD levels available with 65% (70) having insufficient (<30 ng/ml) or deficient (<20 ng/ml) levels. 20% (30) boys had one or more DXA scans with 60% (18) having spine DXA Z-scores < −2.0 and 13% (4) having VFs. 25OHD deficiency/insufficiency correlated with low DXA Z scores. 63% (93) were on vitamin D supplementation [cholecalciferol 55% (51), calcitriol 45% (42) and ergocalciferol 6% (6)]. BHP implementation led to increased testing of 25OHD levels, DXA scans and spine films and more uniform treatment of 25OHD deficiency by providers (3 boys with deficient/insufficient 25OHD are now replete).

Conclusions:
Although preliminary, we find that a BHP will, indeed, improve surveillance for vitamin D deficiency, decreased BMD and VFs in DMD boys. Using a uniform treatment approach we will assess effects of vitamin D/dietary calcium supplementation on the above measures. The inclusion of bisphosphonate treatment in subpopulations of boys with DMD is anticipated.