A QUALITATIVE SYSTEMATIC REVIEW OF LIVING WITH DUCHENNE MUSCULAR DYSTROPHY

Abstract

Duchenne muscular dystrophy (DMD) is one of the most severe forms of inherited muscular dystrophies, leading to limited upper limb use in its later stages. Due to medical and technological advances, patients with DMD are living longer and are reaching adolescence and adulthood. The benefits of bringing together qualitative views and studies in a systematic way include gaining a greater breadth of perspectives and a deeper understanding of health, psychological issues, and concerns from the point of view of patients, and helping the medical community and policymakers to create a better support system. This review aimed to describe the experiences of patients living with DMD in order to identify common themes across all relevant studies. The research question is: What is the lived-world experience of men with DMD? This review adopted a systematic search methodology in accordance with PRISMA guidance, and followed the SPIDER search strategy and the analytic procedure for thematic synthesis. In total, 11 studies were included in the review concerning 132 young people with DMD. In these studies, 5 main topics were identified: experiences of body powerlessness (progressive loss of body control, limited social freedom, lack of privacy); the search for identity (the meaning of becoming an adult man, feelings at the margins, maintaining normality, balancing dependence-independence); challenges in social relations (vulnerability to prejudice, risk of isolation, (in)dispensable parental support, emotional connectedness with carers, the support of others “in the same boat” with DMD); coping processes and resilience (underestimated strength, taking autonomy, toward acceptance, active self-management); and end-of-life experiences (living in the present, experiences of dying, (mis)communication with others about death and dying).

Keywords

• neuromuscular disease
• progressive illness
• male gender
• qualitative methodology
• thematic synthesis