Remote Assessments a Win-Win for ALS Patients and Clinics?

SAVANNAH, GEORGIA — Remote monitoring of respiratory scores in patients with amyotrophic lateral sclerosis (ALS) helps predict the best timing for the introduction of bilevel positive airway pressure (BiPAP), 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 on October 16 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, Calgary, 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 told Medscape Medical News. 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 orthopnia (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 

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 orthopnia 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 Perrera.

Best of Both Worlds

Ileana Howard, MD, physiatrist and professor of rehabilitation medicine at the University of Washington and medical co-director of the ALS Center of Excellence at VA Puget Sound in Seattle, agreed.

“One of the biggest challenges in ALS care today is ensuring equitable access to high quality care and supports, and telehealth was adopted by the VA early on as a means of doing that,” she told Medscape Medical News. “Remote monitoring technology is a really key development to help improve that type of care.”

However, she added that it should not be a question of one type of care vs the other. “The ideal care is when we have access to providing both face-to-face and virtual care for our patients so that we can meet their needs and preferences for care,” she said.

“Sometimes, in my experience, patients don’t understand why it’s important to go to an ALS specialty center. In those cases, I’ve been able to make initial contact with those individuals through telehealth and be able to provide education, which, in turn, often results in them making the decision to come to the specialty center once they understand what resources we have to offer.” 

Also commenting on the research, Ghazala Hayat, MD, also endorsed a mixed approach.

“Telehealth is a very good tool that we should use interspersed with in-person visits,” said Hayat, director of the multidisciplinary ALS clinic at St. Louis University School of Medicine, St. Louis, Missouri, and professor of neurology and director of neuromuscular and clinical neurophysiology.

“I think the first few visits should always be in person — you need to connect with the patient,” she told Medscape Medical News. “But then, once they feel comfortable, remote monitoring is a very good idea, especially later in the disease process, when it becomes really difficult for the family to bring the patient in.” 

The authors reported no relevant disclosures. Howard reported no disclosures. Hayat reported serving as a speaker and in advisory roles for argenx, Alexion, and MTPA. The study was funded by Amylyx Pharmaceuticals.