Optimizing Bone Health in Duchenne Muscular Dystrophy
Table 1
Screening recommendations and course of action summary for children with DMD [26, 27].
Screening
Timing
Course of action
Back pain assessment
Each visit
If present, obtain vertebral imaging
Calcium intake and vitamin D intake (diet and amount of sun exposure)
Initial and subsequent visits
Calcium and vitamin D supplementation as needed; see Table 2.
Serum 25-hydroxyvitamin D
Every 1-2 years
Vitamin D insufficiency/deficiency treatment without clinical signs of rickets. Ergocalciferol or cholecalciferol dose based on vitamin D level: 20–30 ng/mL: 1000 IU PO daily, <20 ng/mL: 2000 IU PO daily, <10 ng/mL: 4000 IU PO daily. (i) Dose may need to be higher in patients with malabsorption, chronic glucocorticoids use, dark skin pigmentation, or obesity. (ii) Serum 25-OH vitamin D level should be repeated in 3 months after giving pharmacologic doses of vitamin D. (iii) When the level is optimal, vitamin D dose should be reduced to a supplementation dose at 400–800 IU/day (or higher in chronic glucocorticoid use).
Bone turnover markers
Not formally recommended at this time
Further research is needed and may be useful in monitoring bisphosphonate therapy.
DXA scan
Obtain baseline prior to glucocorticoid use every 1-2 years thereafter
If height-adjusted lumbar BMD score <−1, should repeat DXA in 1 year. Worsening BMD and/or BMD score or the gain in BMD is less than expected, consider vertebral imaging.
Vertebral imaging (X-rays or densitometric lateral spinal imaging)
Obtain if back pain present or lumbar height-adjusted -score < −2
If vertebral fracture is present, start bisphosphonate therapy.