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Osteoporosis in Duchenne Muscular Dystrophy

Occurrence of Osteoporosis
  • While osteoporosis occurs in boys with Duchenne muscular dystrophy (DMD) not receiving steroid treatment, the risks are increased in patients receiving steroid therapy, especially in the spine.
  • Long bone osteoporosis occurs in boys who are still actively walking, although vertebral bone osteoporosis does not appear to occur until after boys become nonambulatory. [Aparicio: 2002] .
  • Vertebral bones appear to be more vulnerable than long bones to the effects of steroid use.
  • Osteoporosis is associated with increased fracture occurrence, with some estimates as high as 44% of boys with DMD exhibiting fractures.
  • Severe pain may be associated with fractures in this population, particularly when the fractures occur in the vertebrae. Often, after a fracture in a long bone, the child with DMD does not walk again. [Larson: 2000]
  • Bone pain, independent of fractures, may be present in boys with osteoporosis and DMD.

Measuring Bone Density
The most clinically useful method to measure bone density in boys with DMD is bone densitometry, or dual energy X-ray absorptiometry (DEXA). This technique is relatively inexpensive, painless, and usually available, although it does expose the patients to X-rays. The DEXA scan is able to measure both long bone and vertebral bone density. Interpretation of DEXA is difficult in children, although the conversion of results to Z-scores, which are normalized for age, rather than size or Tanner stage, is somewhat helpful. Moreover, a fracture threshold is not known to correlate with any given Z score. A Z-score less than -1.5 is used by most investigators to define osteoporosis in this population.

Intervention
Several different therapies are used to lessen the impact of osteoporosis:
  • Calcium: Although controversial as to its effectiveness, many clinicians will implement calcium and vitamin D supplementation early on in the course of the disease. There is some evidence that dairy product consumption may be preferable to calcium ingestion. See Calcium and Vitamin D.
  • Exercise: or if not possible, standing in a specially designed stander, is often prescribed. Standing has been shown to increase bone mineral density in children with cerebral palsy, but no information is available in boys with DMD. As noted above, boys with DMD show decreased bone mineral density in their long bones even before they become nonambulatory so standing may be a less helpful treatment in the DMD population. [Caulton: 2004]
  • Bisphosphonates: Several studies have suggested that bisphosphonates may have a positive effect on DEXA Z-scores in patients with DMD; these results do not necessarily correlate with a decrease in fracture rate. These effects need to be sorted out in regards to bisphosphonates with and without steroid therapy, optimal ages to begin treatment, the intravenous vs. the oral route, and the possible occurrence of long-term complications.. Quinlivan and others recommend that routine use in boys with DMD be postponed until more information is available, but some clinicians will start treatment with bisphosphonates if fractures have occurred. [Quinlivan: 2005] [Hawker: 2005]

Resources

Information & Support

The Parent Project, the MDA, and Treat-NMD websites listed below contain detailed information about many aspects of DMD for both parents and professionals.

For Professionals

Osteoporosis and DMD (Parent Project)
Information and links to workshops, etc. from the Parent Project.

For Parents and Patients

Support

Parent Project - DMD
This website provides useful information and links to other sources regarding DMD and BMD, including studies, research, and clinical information.

Muscular Dystrophy Association
A nonprofit health agency dedicated to curing muscular dystrophy, ALS, and related diseases by funding worldwide research.

General

Information from TREAT-NMD.com
TREAT-NMD is a network for people with neuromuscular diseases and professionals working in the field. This site features Cochrane reviews on neuromuscular topics, research information, patient care topics, and more.

Services

Bone Densitometry/DEXA

See all Bone Densitometry/DEXA services providers (3) in our database.

Nutrition/Dietary

See all Nutrition/Dietary services providers (53) in our database.

Pediatric Endocrinology

See all Pediatric Endocrinology services providers (2) in our database.

For other services related to this condition, browse our Services categories or search our database.

Helpful Articles

Quinlivan R, Roper H, Davie M, Shaw NJ, McDonagh J, Bushby K.
Report of a Muscular Dystrophy Campaign funded workshop Birmingham, UK, January 16th 2004. Osteoporosis in Duchenne muscular dystrophy; its prevalence, treatment and prevention.
Neuromuscul Disord. 2005;15(1):72-9. PubMed abstract

Carter, G; Charash, L; Cwik, V; Johnsen, S;Stern, L.
Journey of Love, A Parent's Guide to Duchenne Muscular Dystrophy.
Tucson: MDA Publications; 1998. http://www.mdausa.org/publications/journey/preface.html
This guide offers parents the most comprehensive, accurate and readable compendium on Duchenne muscular dystrophy. The index will permit ready access to desired information. The list of community resources and government and voluntary agencies should help ensure that families obtain every available benefit.

Caulton JM, Ward KA, Alsop CW, Dunn G, Adams JE, Mughal MZ.
A randomised controlled trial of standing programme on bone mineral density in non-ambulant children with cerebral palsy.
Arch Dis Child. 2004;89(2):131-5. PubMed abstract / Full Text

Aparicio LF, Jurkovic M, DeLullo J.
Decreased bone density in ambulatory patients with duchenne muscular dystrophy.
J Pediatr Orthop. 2002;22(2):179-81. PubMed abstract

Larson CM, Henderson RC.
Bone mineral density and fractures in boys with Duchenne muscular dystrophy.
J Pediatr Orthop. 2000;20(1):71-4. PubMed abstract

Authors

Author: Lynne M Kerr, MD, PhD - 10/2013
Content Last Updated: 10/2013

Page Bibliography

Aparicio LF, Jurkovic M, DeLullo J.
Decreased bone density in ambulatory patients with duchenne muscular dystrophy.
J Pediatr Orthop. 2002;22(2):179-81. PubMed abstract

Caulton JM, Ward KA, Alsop CW, Dunn G, Adams JE, Mughal MZ.
A randomised controlled trial of standing programme on bone mineral density in non-ambulant children with cerebral palsy.
Arch Dis Child. 2004;89(2):131-5. PubMed abstract / Full Text

Hawker GA, Ridout R, Harris VA, Chase CC, Fielding LJ, Biggar WD.
Alendronate in the treatment of low bone mass in steroid-treated boys with Duchennes muscular dystrophy.
Arch Phys Med Rehabil. 2005;86(2):284-8. PubMed abstract

Larson CM, Henderson RC.
Bone mineral density and fractures in boys with Duchenne muscular dystrophy.
J Pediatr Orthop. 2000;20(1):71-4. PubMed abstract

Quinlivan R, Roper H, Davie M, Shaw NJ, McDonagh J, Bushby K.
Report of a Muscular Dystrophy Campaign funded workshop Birmingham, UK, January 16th 2004. Osteoporosis in Duchenne muscular dystrophy; its prevalence, treatment and prevention.
Neuromuscul Disord. 2005;15(1):72-9. PubMed abstract