Aside from some inflammatory myopathies and very few genetic disorders, there are no therapies that make most patients with myopathies stronger. Consequently, the management of these patients can be frustrating for patients and their families as well as the clinicians taking care of them. Treatment of these patients must involve a comprehensive approach focused on limiting the secondary effects of skeletal muscle weakness, managing comorbidities associated with specific diseases, and, most importantly, optimizing patients’ functional abilities and quality of life in terms of their ability to accomplish activities of daily living. While the approach to each patient differs depending on their disease, certain common themes can be addressed in each patient. This review highlights an approach centered on four conceptual themes (“the Four S’s”): Strength therapies, Supportive care, Symptomatic therapies, and pSychological support.
Although relatively few well-designed studies have been done that highlight conservative management of patients with various myopathies, an emerging literature helps guide the clinician in certain key areas, especially in relation to cardiac and pulmonary management of these patients.
While disease-altering therapies have proven elusive for many muscle diseases, a multimodal approach to the conservative and supportive care of these patients can markedly improve their quality of life. Pharmacologic treatment options for specific myopathies will not be addressed in this article but are covered elsewhere in this issue of CONTINUUM.
Supplemental Digital Content is available in the text.
Address correspondence to Dr John T. Kissel, The Ohio State University Wexner Medical Center, Department of Neurology, 395 W. 12th Avenue, 7th Floor, Columbus, OH 43210, firstname.lastname@example.org.
Relationship Disclosure: Ms King has received partial salary support from an NIH-funded multiclinic trial comparing steroid doses in Duchenne muscular dystrophy. Dr Kissel has received personal compensation for activities with Alexion Pharmaceuticals and Cytokinetics.
Unlabeled Use of Products/Investigational Use Disclosure: Ms King and Dr Kissel discuss the unlabeled use of corticosteroids in the treatment of Duchenne muscular dystrophy.
Supplemental digital content: Videos accompanying this article are cited in the text as Supplemental Digital Content. Videos may be accessed by clicking on links provided in the HTML, PDF, and iPad versions of this article; the URLs are provided in the print version. Video legends begin on page 1670.