Review Article

Optimizing Bone Health in Duchenne Muscular Dystrophy

Table 1

Screening recommendations and course of action summary for children with DMD [26, 27].

ScreeningTimingCourse of action

Back pain assessmentEach visitIf present, obtain vertebral imaging

Calcium intake and vitamin D intake (diet and amount of sun exposure)Initial and subsequent visitsCalcium and vitamin D supplementation as needed; see Table 2.

Serum 25-hydroxyvitamin DEvery 1-2 yearsVitamin 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 markersNot formally recommended at this timeFurther research is needed and may be useful in monitoring bisphosphonate therapy.

DXA scanObtain baseline prior to glucocorticoid use every 1-2 years thereafterIf 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 < −2If vertebral fracture is present, start bisphosphonate therapy.