SAVANNAH, GEORGIA — , results of a retrospective study showed.
The findings, along with those of another study by the same group, suggest that remote monitoring of patients with ALS is a feasible option for both maximizing quality of life and minimizing cost and disruption.
Both studies were presented at the American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) 2024.
“What we’re trying to do is look for screening tools that we can use when these patients are in the community to see if a specific score transition is associated with a high probability of needing an intervention that would require bringing them in to do gold standard tests,” said study investigator Tefani Perera, MD, a neurology resident at the University of Calgary, in Alberta, Canada.
Optimizing Quality of Life
Tailoring in-person care is particularly important for patients with ALS who often face significant challenges with mobility, Perera said. However, most multidisciplinary ALS clinics schedule in-person follow-ups at regular intervals rather than “as needed.
“These are very long clinic days where they are assessed for one thing after another, even if they don’t need it. So maybe we can actually select for what they need to be assessed for at each specific visit? Life expectancy is not that long for these patients, so we want to make sure their quality of life is optimized.”
For the BiPAP study, the investigators used the Pooled Resource Open-Access ALS Clinical Trials database to identify patients with ALS with two or more respiratory assessments on the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R).
The ALSFRS-R is a 12-item questionnaire, which includes three respiratory sub-scores for respiratory insufficiency (RiS), dyspnea (DyS) and orthopnea (OS).
Patients with a baseline RiS sub-score of 4 — meaning no need for BiPAP — were included in the study (n = 3838), with the primary outcome being a drop in RiS sub-score indicating the need for BiPAP.
The median time from baseline to transition to BiPAP was 563 days, with 3.4% of patients reaching this outcome by 3 months.
Results showed the probability of needing BiPAP was significantly associated with baseline DyS and OS scores (P < .0001). Among patients with baseline DyS scores of 3, 2, and 1, the percentages of patients needing BiPAP within 3 months were 5.5%, 8.7%, and 20.1%, respectively. In addition, in patients with baseline OS scores of 3, 2, and 1, the percentages of patients needing BiPAP within 3 months were 9.1%, 12.7%, and 24.2%, respectively.
Regardless of the baseline score, any drop in either of these sub-scores over the study period was also associated with an increased likelihood of requiring BiPAP within 3 months, with a DyS transition from 3 to 2 and an OS transition from 4 to 3 being most notable.
These scores could be used to trigger gold standard assessments for BiPAP, such as nocturnal oximetry, overnight polysomnography, daytime hypercapnia, and forced and slow vital capacities, Perera said. On the other hand, the scores could also help patients and clinicians avoid unnecessary visits.
“When the dyspnea and orthopnea scores are high, they might not need this intervention until 2 years later, so do we even need to bring them in to do these tests or see a respirologist when they don’t actually need it?”
The group’s second study was a systematic review of 26 papers on ALS remote assessment devices and methods, including accelerometers (15.4%), telenursing protocols (3.8%), speech collection apps (26.9%), questionnaires (15.4%), multifactorial sensors (15.4%), and respiratory function monitors (19.2%). Domains of symptoms monitored included speech (12 studies), motor (11 studies), respiratory (11 studies), cardiac (three studies), and bulbar, psychiatric, and autonomic (one study each).
The researchers characterized various remote tools as having potential and concluded that a multidomain approach to symptom monitoring is achievable. They also noted that the majority of studies assessing adherence and patient feedback indicated a favorable response to patient monitoring.
“I work in a resource-rich center, where we have these huge multidisciplinary clinics, and we have the capacity to bring patients back every 3 months, but outside these big centers, in resource-limited settings, to have an ability to track remotely and bring patients in when they really need it is very important,” said Perera.