Objective: Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy in childhood and affects 1:3500 live male births. DMD is caused by mutations (typically deletions) in the dystrophin gene on the X chromosome. Since the gene was cloned in 1986, many strategies for treatment have been proposed and tested in animal models, as well as human subjects. Duchenne muscular dystrophy (DMD) leads to progressive muscle weakness of legs and arms, and respiratory and cardiac failure. Most patients become wheelchair-bound and dependent on others for their daily activities during the second decade of life. Thus, children diagnosed with DMD may be able to reach the fourth decade of life, which is a significant improvement over the historical DMD life expectancy. In this study, we aimed to compare health and sleep quality of children diagnosed with DMD and healthy children; also, we examined the anxiety level of cases and controls’ mothers.
Method: Fifteen children diagnosed as having DMD and 22 healthy controls were recruited in the study. All case and controls were male. Sociodemographic data form was used to determine the social status of children. Pediatric health-related quality of life (HRQOL) was used for determining the health quality of children, which was filled in by both parents and children. The Pittsburgh Sleep Quality Index (PSQI) is a self-rated questionnaire which was used to investigate sleep quality of children We rated the anxiety levels of mothers with The State-Trait Anxiety Inventory (STAI) - state anxiety and trait anxiety forms.
Results: There was no significant difference between the ages of case and control groups (p=0.295). Significant difference was not found between anxiety levels of cases’ and controls’ mothers in STAI state anxiety and trait anxiety forms (p=0.630, p=0.395 respectively). Significant difference was found in HRQOL parent and child forms between cases and controls (p=0.02 and p=0.01). Difference between sleep quality of cases and controls was significant (p=0.031).
Conclusion: The main finding of our study is that patients with DMD have more difficulties in sleeping and their quality of lives is lower than healthy controls. Sleep is crucial for children and adolescents’ learning, memory processes and school performance. Research shows that poor sleep, increased sleep fragmentation, late bedtimes and early awakenings seriously affected learning capacity, school performance, and neurobehavioral functioning. Children diagnosed as having DMD may have also sleeping problems so these problems also can affect the quality of life in children. Both children of case group and their parents reported a worse HRQOL on physical functioning, which can be expected considering the progressive nature of their disease. They also reported a worse HRQOL on social functioning and emotional functioning as compared to age-related healthy individuals. Difficulties in motor functioning can affect the emotional, social functioning and may be sleep quality of children. Mothers’ anxiety levels were same with healthy controls’ mothers’ anxiety levels. This may relate to better coping abilities in mother, whose children diagnosed as DMD adapting themselves to this process.