Spending more time outdoors, engaging in daily activities and living in a community that accepts patients and their families all tend to improve the lives of boys with Duchenne muscular dystrophy (DMD), a European analysis finds.
The analysis,“Predictors of Health-Related Quality of Life in boys with Duchenne muscular dystrophy from six European countries,” appeared in the Journal of Neurology and looked at separate studies involving 321 boys with DMD in the Czech Republic, Bulgaria, Denmark, Germany, Hungary and the United Kingdom.
DMD is a severe genetic neuromuscular disease characterized by progressive muscle weakness that leads to reduced mobility in early teenage years. Although many of the functional symptoms are untreatable, advanced therapies for the respiratory and cardiac symptoms now allow patients to reach adulthood.
Over time, DMD has evolved from a pediatric disease to become also an adult condition. However, the factors that affect overall health-related quality of life (HRQoL) in boys with DMD are unclear.
Researchers gathered data on the boys — aged 8 to 17 — from CARE-DMD, via self-reported questionnaires that covered socio-demographic classification, disease and treatment, environment and daily life participation.
The researchers found that guaranteed, regular visits to specialized clinics improved overall HRQoL, according to the tool KIDSCREEN 10 index, though household income could be an obstacle. Additionally, away-from-home activities and positive feedback by local communities towards patients and their families is key to boosting overall HRQoL for boys with DMD. These results confirm previous observations in large European surveys on children and adolescents from the general population.
Additionally, older boys enjoyed a higher overall HRQoL, according to the generic PedsQL as well as disease-specific HRQoL. Although it may seem contradictory, this could be because older kids with DMD may adapt more easily than younger ones to the different stages of DMD, authors explained.
Further analysis showed some discrepancies in disease-specific HRQoL assessment from one country to the next. For example, German DMD patients had a better quality of life than their British counterparts — perhaps because average per-capita income in Germany is considerably higher than in Britain.
In addition to encouraging out-of-home activities for boys with DVD, the study also supports initiatives aimed at informing the public about the disease.
“Our results may be relevant for clinical practice and planning interventions for this population [boys with DMD], but should be confirmed by future research,” the authors wrote. “Future research may consider influences of further psychosocial factors (e.g. the family climate) to provide more information on how to support the overall HRQoL in boys with DMD.”