Addressing pain, depression may aid people with Fabry disease
People with symptoms of disease may have lower quality of life
People with Fabry disease who have burning limb pain or depression are more likely to report poor quality of life, a new study shows.
Addressing these issues may help them, researchers said in “Screening for health-related quality of life and its determinants in Fabry disease: A cross-sectional multicenter study,” published in Molecular Genetics and Metabolism.
Living with a chronic disorder like Fabry disease can be stressful, and previous studies have suggested patients report worse quality of life than those without the disorder, leading an international team of scientists to try to identify factors that are independently associated with quality of life among people with the disease.
“Knowledge of the independent determinants of [health-related quality of life] could facilitate early identification of patients who need more intense psychological support and care, increased monitoring, and specifically targeted adjunctive treatment such as pain medication or antidepressants,” the scientists wrote.
The study included data on 135 people with Fabry disease who received care at centers in Switzerland or Germany. Most were female with a median age in the late 40s and most were taking enzyme replacement therapy. The researchers collected data on their clinical status as well as demographic and socioeconomic factors. Health-related life quality was assessed using the EuroQol questionnaire.
Quality of life in Fabry disease
The scientists constructed statistical models to look for strong associations between quality of life and other factors.
Patients with classic Fabry tended to report worse life quality than those with late-onset disease and it tended to be poorer in those with kidney or heart involvement, and with a history of stroke.
Burning limb pain and depression were also identified as independent factors linked to poorer life quality. Because pain and depression can often be alleviated with symptom-specific treatments (including pain meds, antidepressants, or talk therapy), providing support to help patients address these issues could help improve their quality of life, the researchers said.
Patients on enzyme replacement therapy tended to report better life quality than those not on this treatment.
These clinical factors showed statistically meaningful associations with quality of life, but demographic and socioeconomic factors didn’t show any clear associations.
“The analyses show that the [health-related life quality] of [Fabry disease] patients is mainly influenced by clinical symptoms rather than demographic or social parameters,” the researchers wrote.
Fabry disease is usually thought to affect men more severely than women, but results showed no difference in quality of life based on sex or gender. This shows the importance of providing support to female patients with Fabry disease, said the researchers, who noted the study only included patients in Europe, so the findings may not be applicable to people with Fabry disease in other parts of the world.
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