MDA 2023: Patients typically need wheelchairs within 2 years of onset

Disease's rapid progression underscores need for better treatments

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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A person shown sitting in a wheelchair.

On average, a person with amyotrophic lateral sclerosis (ALS) will require a wheelchair to get around within two years of the initial onset of disease symptoms.

Findings underscore the condition’s rapid advance, and the need for new ALS treatments that work to slow disease progression, its researchers noted.

“Effective therapies that slow down disease progression may have the potential to reduce [the] humanistic burden of ALS and would help patients, physicians, and caregivers to plan better around these transitions and spread out the costs over time,” they wrote.

These data were shared at the Muscular Dystrophy Association (MDA) Clinical and Scientific Conference, taking place March 19-22 in Texas, in the poster, “Time From Amyotrophic Lateral Sclerosis Onset to Key Disease Milestones: Analysis of Patient Data From a Real-World Cross-Sectional Survey.”

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I have met the enemy, and surprise! It’s not my ALS this time.

Estimates of average time of ALS progression based on neurologist survey

ALS is caused by the death and dysfunction of motor neurons, the specialized nerve cells that control movement, resulting in muscle weakness that eventually progresses to paralysis. It tends to progress very quickly, though considerable variation among patients can be seen.

To better understand ALS progression given current approaches, scientists led by a team at Cytokinetics analyzed information from the Adelphi ALS Disease Specific Programme, which surveyed neurologists in the U.S. and several European countries: France, Germany, Italy, Spain, and the U.K.

The analysis included data covering 1,003 ALS patients — 379 in the U.S. and 624 in Europe — collected between July 2020 and March 2021. About two-thirds of the patients were male, and the vast majority were white.

Most patients in Europe (82.5%) were on treatment with riluzole (sold as Rilutek and other formulations), while most U.S. patients (78.1%) were being treated with riluzole and/or Radicava (edavarone), which is not available in Europe.

Among all the patients, 3.9 months was the average time from initial awareness of symptoms to a first medical visit. The average time from symptom onset to ALS diagnosis was eight months.

At about 1.5 years after disease onset, the average patient started to require a walking aid like a cane or walker. Around this time, these people also tended to experience changes in employment and require more caregiving.

On average, the time to needing a wheelchair was slightly less than 23 months after disease onset. Between 24.6 months and 28.6 months after onset, on average, patients required medical intervention to help them communicate, eat, and breathe. The average time before they needed the care of a specialized facility was slightly more than 30 months.

“Patients rapidly transitioned through various disease milestones, which demonstrates the rapid progression of ALS,” the researchers wrote in their abstract.

Comparisons of patients by where they lived showed that U.S. patients tended to be diagnosed earlier, by nearly four months on average. However, patients in the U.S. also tended to hit other disease milestones (e.g., needing a walking aid, caregiver support, or wheelchair) earlier than their European counterparts.

Because these data were based on neurologists’ reports about their patients at a single point in time, the researchers stressed that caution is needed in interpreting these differences. Findings, they noted, contained “limited information about individual patient journeys or disease history.”