WANTING MUSCLE AND  BONE HEALTH
IS SOMETHING WE CAN ALL AGREE ON.

IN THE TREATMENT OF DMD

Duchenne muscular dystrophy (DMD) is a devastating disease with
consequences throughout the body.

DMD is a rare neuromuscular disease marked by progressive muscle degeneration and weakness. While DMD is rare, the neuromuscular effects of DMD are well characterised. Beyond muscles, DMD has a damaging effect on patients’ cardiac, pulmonary, and skeletal health.1-8

Learn more about the early signs and symptoms of DMD through a range of DMD advocacy organisations.

DMD progresses over time and causes many life limitations including premature death.9-12, a

DMD is a progressive disease with new and more serious effects on mobility and independence as patients age

aAge of onset and timeline of symptoms may vary but are
based on the experiences of patients with DMD who received the latest standard of care.

Progressive deterioration of bone health is one of the many serious consequences of DMD.

Bone development is partly dependent on the action of muscles. The shape and thickness of bones is influenced by the mechanical force muscles put on them.13

This relationship is disrupted by DMD. As a result, bone fragility is a major component of disease progression. Patients with DMD can experience osteopenia and osteoporosis that lead to bone fractures and skeletal deformities.14

Managing skeletal symptoms of DMD6,11,12:

  • Standing may help maintain bone density
  • Routine physical therapy can help maintain
    joint flexibility and reduce contractures
  • Scoliosis can occur and may require surgical correction
  • Bisphosphonates can help to strengthen bone

Early intervention is critical: A delayed start of therapeutic interventions may lead to continued loss of muscle strength, increased bone fragility, the onset of contractures, reduced mobility, and shorter lifespan.12,14,16

Multiple factors contribute to
poor bone health in DMD.

Muscle weakness and immobility

The strength and growth of bones
is impacted by the tension muscles
exert on bones. As muscles
weaken, so do bones13,17

Activation of bone-destroying cells

Chemicals released in response to muscle loss in DMD stimulate the activity of osteoclasts, cells that break down bone17

Lack of
vitamin D

Lifestyle changes caused by DMD can
keep patients out of the sun and lead
to inadequate levels of vitamin D,
which is essential for healthy bones17

Poor calcium absorption

Multiple factors may contribute to the
improper calcium maintenance
observed in patients with DMD. Calcium
is central to proper bone mineralization17

Traditional corticosteroids, the standard of care in DMD, have been shown to have a negative impact
on bone health.7-8,17

Review the treatment
considerations

References: 1. Stimpson G, Raquq S, Chesshyre M, et al. Growth pattern trajectories in boys with Duchenne muscular dystrophy. Orphanet J Rare Dis. 2022;17(1):20. 2. Falzarano MS, Scotton C, Passarelli C, Ferlini A. Duchenne muscular dystrophy: from diagnosis to therapy. Molecules. 2015;20(10):18168-18184. 3. Buddhe S, Cripe L, Friedland-Little J, et al. Cardiac management of the patient with Duchenne muscular dystrophy. Pediatrics. 2018;142(Suppl 2):S72-S81. 4. Nitahara-Kasahara Y, Takeda S, Okada T. Inflammatory predisposition predicts disease phenotypes in muscular dystrophy. Inflamm Regen. 2016;36:14. 5. Finder J, Mayer OH, Sheehan D, et al. Pulmonary endpoints in Duchenne muscular dystrophy: a workshop summary. Am J Respir Crit Care Med. 2017;196(4):512-519. 6. Pedlow K, McDonough S, Lennon S, Kerr C, Bradbury I. Assisted standing for Duchenne muscular dystrophy. Cochrane Database Syst Rev. 2019;10(10):CD011550. 7. Ward LM, Hadjiyannakis S, McMillan HJ, et al. Bone Health and Osteoporosis Management of the Patient With Duchenne Muscular Dystrophy. Pediatrics. 2018; 142(Suppl 2): S34-S42. 8. Ward LM. Glucocorticoid-Induced Osteoporosis: Why Kids Are Different. Front. Endocrinol. 2020; 11:576. 9. Asher DR, Thapa K, Dharia SD, et al. Clinical development on the frontier: gene therapy for Duchenne muscular dystrophy. Expert Opin Biol Ther. 2020;20(3):263-274. 10. Tian C, Wong B, Hornung L, et al. Age-specific prevalence of osteoporosis and frequency of poor bone health indices in Duchenne muscular dystrophy. Neuromuscular Disorders. 2014; 24: 857. World Muscle Society abstract G.P.171. 11. Birnkrant DJ, Bushby K, Bann CM, et al. Diagnosis and management of Duchenne muscular dystrophy, part 3: primary care, emergency management, psychosocial care, and transitions of care across the lifespan. Lancet Neurol. 2018;17(5):445-455. 12. Birnkrant DJ, Bushby K, Bann CM, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol. 2018;17(4):347-361. 13. Berendsen AD, Olsen BR. Bone development. Bone. 2015;80:14-18. 14. Morgenroth VH, Hache LP, Clemens PR. Insights into bone health in Duchenne muscular dystrophy. Bonekey Rep. 2012;1:9. 15. Guglieri M, Bushby K, McDermott MP, et al. Developing standardized corticosteroid treatment for Duchenne muscular dystrophy. Contemp Clin Trials. 2017;58:34-39. 16. Matthews E, Brassington R, Kuntzer T, Jichi F, Manzur AY. Corticosteroids for the treatment of Duchenne muscular dystrophy. Cochrane Database Syst Rev. 016;2016(5):CD003725. 17. Buckner JL, Bowden SA, Mahan JD. Optimizing bone health in Duchenne muscular dystrophy. Int J Endocrinol. 2015;2015:928385.

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