Diagnosis and management of Duchenne muscular dystrophy, part 3: primary care, emergency management, psychosocial care, and transitions of care across the lifespan

David J Birnkrant¹, Katharine Bushby², Carla M Bann³, Susan D Apkon⁴, Angela Blackwell³, Mary K Colvin⁵, Linda Cripe⁶, Adrienne R Herron⁷, Annie Kennedy⁸, Kathi Kinnett⁸, James Naprawa⁹, Garey Noritz⁶, James Poysky¹⁰, Natalie Street¹¹, Christina J Trout¹², David R Weber¹³, Leanne M Ward¹⁴; DMD Care Considerations Working Group

Affiliations

¹ Department of Pediatrics, MetroHealth Medical Center, Case Western Reserve University, Cleveland, OH, USA. Electronic address: dbirnkrant@metrohealth.org.
² John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.
³ RTI International, Research Triangle Park, NC, USA.
⁴ Department of Rehabilitation Medicine, Seattle Children's Hospital, Seattle, WA, USA.
⁵ Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA.
⁶ Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA.
⁷ Rare Disorders and Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA, USA; Oak Ridge Institute for Science and Education, Oak Ridge, TN, USA.
⁸ Parent Project Muscular Dystrophy, Hackensack, NJ, USA.
⁹ Emergency Department, University of California San Francisco Benioff Children's Hospital, Oakland, CA, USA.
¹⁰ Baylor College of Medicine, Houston, TX, USA.
¹¹ Rare Disorders and Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA, USA.
¹² Stead Family Department of Pediatrics, University of Iowa, Iowa City, IA, USA.
¹³ Division of Endocrinology and Diabetes, Golisano Children's Hospital, University of Rochester Medical Center, Rochester, NY, USA.
¹⁴ Division of Endocrinology and Metabolism, Children's Hospital of Eastern Ontario, and University of Ottawa, Ottawa, ON, Canada.

PMID: 29398641
PMCID: PMC5902408
DOI: 10.1016/S1474-4422(18)30026-7

Review

Diagnosis and management of Duchenne muscular dystrophy, part 3: primary care, emergency management, psychosocial care, and transitions of care across the lifespan

David J Birnkrant et al. Lancet Neurol. 2018 May.

. 2018 May;17(5):445-455.

doi: 10.1016/S1474-4422(18)30026-7. Epub 2018 Feb 2.

Authors

Affiliations

¹ Department of Pediatrics, MetroHealth Medical Center, Case Western Reserve University, Cleveland, OH, USA. Electronic address: dbirnkrant@metrohealth.org.
² John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.
³ RTI International, Research Triangle Park, NC, USA.
⁴ Department of Rehabilitation Medicine, Seattle Children's Hospital, Seattle, WA, USA.
⁵ Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA.
⁶ Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA.
⁷ Rare Disorders and Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA, USA; Oak Ridge Institute for Science and Education, Oak Ridge, TN, USA.
⁸ Parent Project Muscular Dystrophy, Hackensack, NJ, USA.
⁹ Emergency Department, University of California San Francisco Benioff Children's Hospital, Oakland, CA, USA.
¹⁰ Baylor College of Medicine, Houston, TX, USA.
¹¹ Rare Disorders and Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA, USA.
¹² Stead Family Department of Pediatrics, University of Iowa, Iowa City, IA, USA.
¹³ Division of Endocrinology and Diabetes, Golisano Children's Hospital, University of Rochester Medical Center, Rochester, NY, USA.
¹⁴ Division of Endocrinology and Metabolism, Children's Hospital of Eastern Ontario, and University of Ottawa, Ottawa, ON, Canada.

PMID: 29398641
PMCID: PMC5902408
DOI: 10.1016/S1474-4422(18)30026-7

Abstract

Improvements in the function, quality of life, and longevity of patients with Duchenne muscular dystrophy (DMD) have been achieved through a multidisciplinary approach to management across a range of health-care specialties. In part 3 of this update of the DMD care considerations, we focus on primary care, emergency management, psychosocial care, and transitions of care across the lifespan. Many primary care and emergency medicine clinicians are inexperienced at managing the complications of DMD. We provide a guide to the acute and chronic medical conditions that these first-line providers are likely to encounter. With prolonged survival, individuals with DMD face a unique set of challenges related to psychosocial issues and transitions of care. We discuss assessments and interventions that are designed to improve mental health and independence, functionality, and quality of life in critical domains of living, including health care, education, employment, interpersonal relationships, and intimacy.

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Figures

**Figure 1. Considerations for psychosocial care of individuals with Duchenne muscular dystrophy**
DMD=Duchenne muscular dystrophy.

**Figure 2. Components of young adulthood to be addressed during transition planning for individuals with Duchenne muscular dystrophy**
DMD=Duchenne muscular dystrophy.

See this image and copyright information in PMC

Comment in

Evidence-based care in Duchenne muscular dystrophy.
McDonald CM, Mercuri E. McDonald CM, et al. Lancet Neurol. 2018 May;17(5):389-391. doi: 10.1016/S1474-4422(18)30115-7. Lancet Neurol. 2018. PMID: 29656735 No abstract available.

References

1. Bushby K, Finkel R, Birnkrant DJ, et al. for the DMD Care Considerations Working Group Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. Lancet Neurol. 2010;9:77–93. - PubMed
1. Bushby K, Finkel R, Birnkrant DJ, et al. for the DMD Care Considerations Working Group Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurol. 2010;9:177–89. - PubMed
1. Saito T, Kawai M, Kimura E, et al. Study of Duchenne muscular dystrophy long-term survivors aged 40 years and older living in specialized institutions in Japan. Neuromuscul Disord. 2017;27:107–14. - PubMed
1. Medical Home Initiatives for Children With Special Needs Project Advisory Committee, American Academy of Pediatrics. The medical home. Pediatrics. 2002;110(1 Pt 1):184–86. - PubMed
1. Homer CJ, Klatka K, Romm D, et al. A review of the evidence for the medical home for children with special health care needs. Pediatrics. 2008;122:e922–37. - PubMed

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Diagnosis and management of Duchenne muscular dystrophy, part 3: primary care, emergency management, psychosocial care, and transitions of care across the lifespan

Affiliations

Diagnosis and management of Duchenne muscular dystrophy, part 3: primary care, emergency management, psychosocial care, and transitions of care across the lifespan

Authors

Affiliations

Abstract

Figures

Comment in

References

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Other Literature Sources

Medical