Researchers found that the use of mobility devices is associated with a poorer quality of life in children with Friedreich’s ataxia (FA).
The study, “Impact of Mobility Device Use on Quality of Life in Children With Friedreich Ataxia,” was published in the Journal of Child Neurology.
Friedreich’s ataxia is a neurodegenerative disease mainly characterized by slow progressive ataxia — or lack of voluntary movement coordination — and muscle weakness, among other symptoms.
Previous studies demonstrated that patients had a lower quality of life when compared to people without the disease. They also found the clinical severity of this disease had a stronger correlation to lower quality of life than with biochemical markers of severity.
One of the main ways to manage the progressive ataxia and muscle weakness is the use of mobility devices. But the impact on the quality of life, especially in newly diagnosed children, has yet to be fully understood.
Now, a team of researchers assessed the impact of mobility devices on the quality of life in children and teens with FA.
Using data from 111 pediatric patients with genetically confirmed Friedreich’s ataxia, they analyzed a series of parameters, including age of disease onset, first symptoms, type of mobility device used (if applicable), and health-related quality of life, using the Pediatric Quality of Life Inventory (PedsQL) module. This integrates both generic core scales and disease-specific modules in children and adolescents ages 2 to 18.
Mobility devices were used by 47 out of the total 111 (42%) young patients whose data was studied. Of these, 43 used a wheelchair, three used only a walker, and one used only a cane.
They found that patients who used a mobility device had significantly lower pediatric quality of life scores, as well as emotional, social, and academic scores when compared to those who didn’t require such devices. Overall, the use of a mobility device affected all domains of quality of life.
They also assessed how transition to or between mobility devices may affect the life of these pediatric patients.
Researchers found that during the transition period to the use of a mobility device, psychosocial support was fundamental and led to a stable emotional quality of life in pediatric patients.
These results suggest that understanding the course of quality of life associated with the use of a mobility device would be useful for counseling and anticipatory care, and that additional research is needed to “define the unique physical and emotional needs of this population.”
Overall, “focus on promoting adaptation, support, and understanding of their new limitations should be a priority for supporting children with Friedreich’s ataxia and their families,” the authors concluded.