It has been discovered that the majority of patients with Amyotrophic Lateral Sclerosis (ALS) develop respiratory difficulties in the advanced stages of the disease. This calls for them being placed under chronic ventilator support to improve the severity of symptoms.
It is with renewed hope that an emerging study has reported the fact that in spite of improved respiration, the quality of life of ALS patients is prone to deteriorating as time goes by.
The study investigated to what extent chronic ventilatory support was effective in ALS patients. It was published in the Journal of Neurology.
In an effort to gauge if the advantages of ventilatory support exhibited disparity among ALS and other patients, the researchers analysed findings from a trial that had been published earlier. The team was from the University Medical Centre of Groningen. They assessed the study’s data on quality of life of at-home versus in-hospital initiation on Non-Invasive Ventilation (NIV) which aids patients in breathing.
The trial was done in a cohort of patients with ALS; other neuromuscular disorders ranging from Diaphragm Paralysis, Myotonic Dystrophy, Limb Girdle Muscular Dystrophy, or Facioscapulohumeral to Dystrophy; or a thoracic cage problem such as Kyphoscoliosis or Obesity Hypoventilation Syndrome.
The overall quality of life was examined with a number of questionnaires that had been designed to particularly look into this parameter in patients with respiratory insufficiency, consisting of 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).
Out of the 77 patients on NIV, 30 had ALS and 47 did not have the disease. The analysis revealed that NIV notably enhanced respiration in both groups after 8 to 24 weeks. On the other hand, despite improving the quality of life in the other patients, the ALS patients exhibited a distinct pattern. After 8 weeks, only three domains of the SRI questionnaire had significantly enhanced in ALS patients, and after 24 weeks, the quality of life of these patients was seen to worsen over time.
The researchers established it was reported that patients were content with the level of care and outcome from the therapy, comfort during sleeping time and were more awake during the day.
However, they further noted that these benefits were no longer evident with time as leakage of the mask during ventilation became a burden for both the patient and caregivers. More particularly, the sound of air leaking by the mask and the ventilator kept everyone awake and became incapable of offering any comfort anymore.
It was observed with concern that one of the drawbacks of the study was centred of the fact that the number of ALS patients was significantly smaller in comparison to the number of other patients. This created a situation which made it difficult to compare the two groups effectively.
On the plus side, researchers are convinced that the results alleviated doubt on the benefit of long-term NIV in ALS patients. In conclusion, future studies should investigate the issue in larger groups of ALS patients.