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

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.

Neurodevelopmental, behavioral, and emotional symptoms common in Duchenne muscular dystrophy

“…The management of the neurodevelopmental, behavioral, and emotional symptoms in patients with DMD is an integral component of comprehensive care for this multisystemic disorder that affects the physical, social, and emotional well‐being of patients and families. The need for management of these symptoms was highlighted in the 2018 update of DMD care considerations …”

Health profile of a cohort of adults with Duchenne muscular dystrophy

“…These changes create additional stresses on the affected individuals and their families. The updated Care Considerations on DMD includes a new section on transitions of care across the lifespan to emphasize the importance of, and need for, coordination of healthcare transition …”

Parent–child communication and timing of interventions are challenges in the Duchenne muscular dystrophy care

“…In addition, they clearly expressed the need for professional support at the psychological level, for themselves as well as for their sons, and in the difficult task of discussing the DMD diagnosis with their sons. This indicates, as also pointed out in the international guidelines , that multidisciplinary teams caring for boys with DMD should include such competence and have a holistic approach including the whole family.…”

“…The challenging transition for adolescents with special healthcare needs is a topic that has been increasingly focused during the recent years. Since boys with DMD often have increasing healthcare needs and physical reliance on others for daily activity at the ages when adolescents generally have desire for more independence, the transition process may for them be especially challenging . As expressed by one of the participants in our study, this aspect of DMD is a heavy burden also from the parental point of view.…”