Symptoms of depression significantly affect the quality of life of people with Friedreich’s ataxia (FA), a study has found.
The study, “Health-related quality of life and depressive symptoms in Friedreich ataxia,” was published in the journal Quality of Life Research.
Friedreich’s ataxia (FA) is an inherited, progressive disease that affects nerves and muscles, causing loss of control of body movements (ataxia). It typically begins in childhood or adolescence, eventually leading to significant functional loss, wheelchair dependence, and reduced quality of life.
Little research has been conducted on the health-related quality of life issues in people with FA.
“Previous studies are scarce, but they have consistently reported that health-related quality of life (HRQOL) was perceived to be significantly worse for individuals with [FA] in practically all dimensions,” the authors wrote.
Moreover, most studies have focused on the severity of the disease as the main determining factor of HRQOL. However, emotional factors such as depression, which influences HRQOL in FA patients, have not been assessed.
To address this knowledge gap, researchers recruited 62 people with FA to determine how depression and other clinical variables affect their perceived HRQOL.
The severity of FA was assessed with the Scale for the Assessment and Rating of Ataxia (SARA), using scores from zero to 40, with higher scores reflecting more severe disease.
HRQOL was measured using the SF-36 Health Survey — a self-reported, quality-of-life assessment. It analyzes eight factors (dimensions) that influence HRQOL, including physical functioning, role physical (limitations in routine activities due to physical health problems), bodily pain, general health perception, vitality, social functioning, role emotional (limitations on routine activities due to emotional problems), and mental health. For each subscale, higher scores reflect better self-reported health.
Depressive symptoms were evaluated using the Beck Depression Inventory-II (BDI-II), which is a self-rating questionnaire. Scores range from zero to 63, with 0–13 classified as minimal depressive symptoms, 14–19 as mild, 20–28 as moderate, and 29–63 as severe.
Of the 62 patients, 28 were men and 34 were women, who ranged from 19 to 79 years old. The onset of FA ranged from two to 44 years old, and disease duration from one to 47 years. Patients in this study showed a wide range of disease severity, as SARA scores ranged from 6 to 37.
The results from the SF-36 survey found that HRQOL is significantly reduced in all eight dimensions in FA patients compared with the general population.
As expected, FA patients scored low in physical functioning, role physical, and general health. Additionally, a significant number of patients had low scores in the mental health, social functioning, and role emotional categories, which indicated that FA patients believe that emotional problems limit their social and daily activities.
Role emotional was found to be negatively associated with duration of the disease, which suggests that the longer a person has FA, the more emotional problems interfere with daily activities.
The depression survey generated a mean BDI score of 15.32, with 50% of patients reporting minimal depressive symptoms, 23% reporting mild symptoms, 23% reporting moderate symptoms, and 5% scoring in the severe range.
The BDI-II scores related to depression negatively influenced nearly all SF-36 dimensions. As expected, depression significantly affected mental health. In addition, the analysis showed that social functioning, vitality, role physical, and bodily pain were also associated with depression.
Overall, depression was found to influence problems in daily activities caused by physical health, perceived pain, health status, and feeling of tiredness. In addition, depression affected how an FA patient perceived the limitations of their social and daily activities due to emotional problems.
“In other neurological disorders, it is also common to find a reduced HRQOL,” the researchers noted. “However, if the results are compared with those generally reported by studies on Parkinson’s disease or multiple sclerosis, the magnitude of the reduction in HRQOL and the amplitude of it appears to be greater ” in FA.
“The poor quality of life experienced by patients with Friedreich ataxia is only partially related to demographic or disease variables, with depressive symptomatology being the most relevant variable for predicting quality of life,” the authors said. “Therefore, the recognition and adequate treatment of depression are essential to improve the subjective experience of quality of life of Friedreich ataxia patients.”