Disease Progression Has Significant Effect on Quality of Life of ALS Patients, Study Says

In addition to symptoms of depression, disease progression is one of the strongest influences on health-related quality of life in amyotrophic lateral sclerosis (ALS) patients, a study reports.

According to the study, titled Disease progression impacts health-related quality of life in amyotrophic lateral sclerosis,” slower disease progression is linked to higher levels of emotional well-being in these patients. It was published in the Journal of the Neurological Sciences.

ALS is a progressive neurological disease that destroys the nerve cells, leading to an inability to perform day-to-day functions. The rate of disease progression and deterioration of physical activity vary considerably from patient to patient.

Factors such as impaired physical function and mental disorders such as anxiety and depression, which have a higher incidence in ALS patients than in the general population, can all have a negative effect on the health-related quality of life of these patients.

Researchers believe that faster disease progression can significantly influence a patient’s quality of life, but the relationship between the two was not entirely clear. So a team at the University of Jena in Germany designed a study to test this hypothesis.

A total of 161 ALS patients, recruited between May 2013 and December 2017, answered the long version of the ALS Assessment Questionnaire (ALSAQ-40), which measures health-related quality of life.

The ALSAQ-40 assesses five domains: physical mobility, activities of daily living/independence, eating and drinking, communication, and emotional well-being. It also determines the presence of depressive symptoms, hopelessness, dysphagia (difficulty swallowing), and pain.

Researchers used the revised ALS Functional Rating Scale (ALSFRS-R) to quantify physical impairment.

Most patients were in stages 2 and 3 of the disease. Factors such as age, sex, and ALS subtype did not differ between disease stages. Patients with more advanced disease — the higher stages — had more physical impairment and a lower quality of life.

Researchers found that physical impairment, depression (reported in more than two-thirds of the patients), pain, hopelessness, and progression rate were the factors that more strongly influenced health-related quality of life in ALS patients.

“The present study confirms that physical function and psychological factors are important predictors of health-related [quality of life] in ALS. Here, physical ability and depression had the strongest influence,” the authors wrote.

“While substantial evidence for depression being a core predictor of health-related QoL in ALS already exists, the present study has revealed that the progression rate is also a relevant and independent predictor of health-related QoL in ALS,” they added.

Emotional well-being, activities of daily living/independence, and communication and eating were the subdomains most strongly influenced by the progression rate.

Further studies that evaluate the changes over time in disease progression, emotional well-being, and health-related quality of life would help to better understand the mechanisms behind these associations, according to the investigators.