#apaperaday: Social difficulties and care burden of adult Duchenne muscular dystrophy in Japan: a questionnaire survey based on the Japanese Registry of Muscular Dystrophy (Remudy)
In today’s #apaperaday, Prof. Aartsma-Rus reads and comments on the paper titled: Social difficulties and care burden of adult Duchenne muscular dystrophy in Japan: a questionnaire survey based on the Japanese Registry of Muscular Dystrophy (Remudy)
Today’s pick is from @ojrarediseases by Mori-Yoshimura et al on the social difficulties and care burden of adult Duchenne patients in Japan, using the Remudy database. DOI: 10.1186/s13023-024-03087-z
Duchenne leads to progressive loss of muscle function, with disease milestones including loss of ambulation (around age 10) and the need for assisted ventilation (around age 20). With good care, life expectancy has increased from 19 years to 31 years in Japan.
Currently, the majority of registered Duchenne patients in Japan are adults (52%). These patients require specific care. Authors introduce that for many patients there are also cognitive and behavior components, which increase the care burden.
There are few studies on how adult patients in Japan cope & who helps with care. Here authors performed a study in the Remudy registry of Japanese patients and 234 participants of 367 eligible ones responded to the survey, 62 patients and 172 representatives on patient behalf.
The patients involved were on average 27 years old (20-48 years); 82% were on a ventilator. Loss of ambulation happened at age 10 and wheelchair use started at age 9.5. 18% of patients had developmental delays or autism or both.
Problematic behavior was reported for 34 patients (cutting class, domestic violence, and in 1 case self-mutilation/suicide attempt ????). Most patients completed their high school (67%), 14% university, and 3% a doctoral degree, while 8% were still in university.
74% had attended a special school for disabled children. 31% experienced bullying due to their physical condition ????- this happened even in special needs schools (so by other children with special needs). Authors outline more attention needs to be made to inclusivity at schools.
31% of patients had worked and 20% were still working. Reasons for not working or stopping work were physical need requirements that could not be met (e.g., help by assistant during work hours). Care was provided by family members, mainly mothers.
Unpaid care was provided for 15.5 hours per day (!). Not surprisingly, the household income for Duchenne families was lower than the average for Japan, where families lived on a single income but had higher expenses due to the Duchenne care/needs.
In 11% of cases, the Duchenne patient’s income contributed to the household income. Authors discuss that the Japanese care system needs to be overhauled, as currently families mainly provide care throughout life. This works now, but likely will not in the future:
- Demographic changes: mothers become mothers at an older age, and there are fewer siblings and Duchenne patients live longer. Thus older parents will need to care longer – likely this is not tenable.
- More Japanese families become double income and living expenses increase.
However, due to tradition, it is mainly the families, especially the mothers, who provide the care for the patients, which is incompatible with work (15.5 hours of care per day). Authors outline that traditional values also prevent families from asking for help or resources.
