Moving Beyond the 2018 Minimum International Care Considerations for Osteoporosis Management in Duchenne Muscular Dystrophy (DMD): Meeting Report from the 3rd International Muscle-Bone Interactions Meeting 7th and 14th November 2022
Journal of Neuromuscular Diseases, vol. 11, pp. 233–252
Abstract
Individuals living with Duchenne muscular dystrophy (DMD) are at significant risk of bone fragility due to osteoporosis, with the most potent drivers of fragility fractures in this context stemming from the aggressive myopathy and long term oral glucocorticoid therapy. Young people with DMD have a high fracture burden, with reported total and vertebral fracture rates that are four [1, 2] and 535 times [1] higher than those of healthy growing boys, respectively. Vertebral fractures can occur as early as six months following daily glucocorticoid initiation [3]. Up to 75% of young people with DMD sustain at least one fracture after eight years of glucocorticoid therapy [4]. Fractures in DMD can lead to devastating outcomes, including steeper rates of functional decline, premature and permanent loss of ambulation, chronic pain, and even death from fat embolism syndrome or adrenal crisis following long bone fractures [2, 5–8]. The potential for serious consequences and medical complications linked to fractures has driven efforts to develop effective guidelines for timely bone health surveillance and treatment with more recent efforts to develop fracture prevention strategies. To guide clinicians in the management of DMD and its related co-morbidities (including skeletal health), the first internationally-endorsed, minimum standards of care were published in 2010 under the moniker “Clinical Care Considerations” [9, 10]. This document recommends that osteoporosis monitoring include spine x-rays if back pain or kyphosis is present, followed by initiation of intravenous bisphosphonate therapy if vertebral fractures are identified [10]. In the years following the inaugural 2010 Clinical Care Considerations, studies were published showing that vertebral fractures, a key manifestation of bone fragility among children and adults living with glucorticoid-treated chronic conditions, were frequently asymptomatic, necessitating routine surveillance for early detection [3, 11]. It was also better appreciated that even a single long bone fracture can signal osteoporosis in a persistently high-risk setting such as DMD, and prompt initiation of bone protection therapy is important. With this new knowledge, the latest international, minimum standards of clinical care for DMD published in 2018, known as Care Considerations [12–14], recommended routine, standardized spine imaging for early detection of vertebral fractures, combined with more timely bone-targeted (bisphosphonate) intervention in the presence of vertebral or low trauma long bone fractures [12]. At the same time, the ever-changing therapeutic landscape for the treatment of the underlying condition calls for ongoing examination of the intimate relationship between muscle and bone development in DMD, including the effect of different DMD treatment approaches on the skeletal and endocrine systems. The overall goal of such focus is to harvest discussions about optimal management that will foster bone strength and prevent fractures in this high-risk setting across all underlying disease-targeted treatment paradigms for people with DMD.
Authors 19
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Children's Hospital of Eastern Ontario
Affiliation as printed
Children’s Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada
Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada
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Birmingham Women’s and Children’s NHS Foundation Trust
Affiliation as printed
Birmingham Women’s and Children’s NHS Foundation Trust, Birmingham, United Kingdom
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Nationwide Children's Hospital · The Ohio State University
Affiliation as printed
Nationwide Children’s Hospital, Ohio State University, Columbus, OH, USA
Nationwide Children's Hospital, Ohio State University, Columbus, OH, USA
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Parent Project Muscular Dystrophy
Affiliation as printed
Parent Project Muscular Dystrophy, Washington, DC, USA
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Affiliation as printed
Department of Pharmaceutical Sciences, School of Pharmacy and Pharmaceutical Sciences Binghamton University, State University of New York, Binghamton, NY, USA
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Children's Hospital of Eastern Ontario
Affiliation as printed
Children’s Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada
Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada
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Roslin Institute · University of Edinburgh
Affiliation as printed
Roslin institute, University of Edinburgh, Edinburgh, United Kingdom
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Affiliation as printed
Duchenne UK, London, United Kingdom
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Children's Hospital of Eastern Ontario
Affiliation as printed
Children’s Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada
Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada
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The University of Queensland · Children's Health Queensland Hospital and Health Service
Affiliation as printed
Child Health Research Centre, The University of Queensland, Brisbane, QLD, Australia
Department of Endocrinology and Diabetes, Queensland Children’s Hospital, Brisbane, QLD, Australia
Faculty of Medicine, The University of Queensland, Brisbane, QLD, Australia
Department of Endocrinology and Diabetes, Queensland Children's Hospital, Brisbane, QLD, Australia
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Erik Harmen Niks Aachen
Leiden University Medical Center
Affiliation as printed
Leiden University Medical Center, Leiden, The Netherlands
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Shriners Hospitals for Children - Canada
Affiliation as printed
Shriners Hospital for Children, Montreal, QC, Canada
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Parent Project Muscular Dystrophy
Affiliation as printed
Parent Project Muscular Dystrophy, Washington, DC, USA
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Affiliation as printed
Duchenne Parent Project, Veenendaal, The Netherlands
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Children's Hospital of Philadelphia · University of Pennsylvania
Affiliation as printed
The Children’s Hospital of Philadelphia and The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
The Children's Hospital of Philadelphia and The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
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University of Massachusetts Chan Medical School
Affiliation as printed
Department of Pediatrics and Neurology, University of Massachusetts Chan Medical School, Worcester, MA, USA
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Affiliation as printed
John Walton Muscular Dystrophy Research Centre, Institute of Translational and Clinical Research, Newcastle University and Newcastle Hospitals NHS Foundation Trust, Newcastle, United Kingdom
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University of Ottawa · Children's Hospital of Eastern Ontario
Affiliation as printed
Children’s Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada
Department of Pediatrics, Division of Pediatric Endocrinology, University of Ottawa, Children’s Hospital of Eastern Ontario, Ottawa, ON, Canada
Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada
Department of Pediatrics, Division of Pediatric Endocrinology, University of Ottawa, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada
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Royal Hospital for Children · University of Glasgow
Affiliation as printed
Department of Human Nutrition, University of Glasgow, Glasgow, United Kingdom
Department of Paediatric Endocrinology, Royal Hospital for Children, Glasgow, United Kingdom
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