Chronic Respiratory Support May Decrease Quality of Life in ALS Patients, Study Suggests

Most patients with amyotrophic lateral sclerosis (ALS) develop respiratory problems in the advanced stage of their disease and are put on chronic ventilator support to improve their symptoms. Now a new study shows that despite improved respiration, the patients’ quality of life may worsen over time.

The study, “Is Chronic Ventilatory Support Really Effective In Patients With Amyotrophic Lateral Sclerosis?” was published in the Journal of Neurology.

To understand whether the benefits of ventilatory support were different among ALS and other patients, the team from the University Medical Center of Groningen in the Netherlands analyzed the data from a previously published trial on the quality of life of home versus in-hospital initiation of non-invasive ventilation (NIV, a mask that helps the patient breathe).

The trial was conducted in a group of patients with either ALS; a neuromuscular disorder such as diaphragm paralysis, myotonic dystrophy, limb girdle muscular dystrophy, or facioscapulohumeral dystrophy; or a thoracic cage problem such as kyphoscoliosis or obesity hypoventilation syndrome.

The quality of life was evaluated with several questionnaires set up to specifically address this parameter in patients with respiratory insufficiency, including the Short Form 36 (SF-36); the Severe Respiratory Insufficiency (SRI); the Maugeri Respiratory Failure (MRF-28); and the Hospital Anxiety and Depression Scale (HADS).

Of the 77 patients on NIV, 30 had ALS and 47 did not. The analysis revealed that NIV significantly improved respiration in both groups after two and six months. However, although it also improved quality of life in the other patients, the ALS patients showed a different pattern. After two months, only three domains of the SRI questionnaire had significantly improved in ALS patients, and after six months, the quality of life of these patients was even worse.

“In general, we are told [the patients] are satisfied with the level of care and result of the therapy, comfort during sleep and more awake during daytime,” the authors wrote. “However … these benefits disappear as leakage of the mask during ventilation becomes a burden for both the patient and relatives. The sound of air leaking by the mask and the ventilator wakes everybody, and does not provide any comfort anymore.”

One of the limitations of the study was that the number of ALS patients was smaller than the number of other patients, which makes it difficult to compare the two groups. Still, researchers believe these results shed doubt on the benefit of long-term NIV in ALS patients and that future studies should address this issue in larger groups of ALS patients.