ALS patients offer perspectives on having access to prognostic test data
Obtaining results on smartphone app elicits fear, anxiety for some people
Having access to results from prognostic tests — including neurofilament light chain (NfL) and the ALS Functional Rating Scale-Revised (ALSFRS-R) — is informational, personally relevant, and relevant to research for people with amyotrophic lateral sclerosis (ALS), a study suggests.
The perceived burden related to receiving the tests results was generally low, but a significantly greater proportion of patients getting updates on their blood levels of NfL, a marker of nerve damage, via a smartphone app did find it burdensome relative to those who received results from a neurologist in the clinic. Still, the study highlights the value of sharing information about biomarkers related to disease prognosis, as long as a patient’s individual preferences are kept in mind, the researchers said.
“This study emphasized the perceived benefit of shared information on NfL and ALSFRS-R from the patient’s perspective,” Thomas Meyer, MD, the study’s co-senior author and the director of the ALS Center at Charité University Hospital in Berlin, Germany, said in a summary emailed to ALS News Today. “These results support the patient-centered concept of sharing prognostic information on NfL and ALSFRS-R.”
The study, “Shared prognostic information in amyotrophic lateral sclerosis – systematic assessment of the patients’ perception of neurofilament light chain and the ALS functional rating scale,” was published in Neurological Research and Practice.
In ALS, there is a is progressive dysfunction and death of motor neurons, the specialized nerve cells that control voluntary movements, which leads to muscle weakness. ALSFRS-R and blood NfL levels are used as prognostic tools, meaning they’re monitored so doctors might understand how the disease is progressing.
NfL is a protein released from damaged nerve cells that’s elevated in ALS and increases as the disease advances. NfL levels also correlate with scores on the ALSFRS-R, a standardized measure of patients’ ability to perform a number of daily activities to monitor disease progression.
In recent years, new digital tools have enabled patients to have more control over managing their condition. Various smartphone applications can help track symptoms and medications, and, in some cases, allow for remote data collection. The mobile ALS App lets patients perform a digital self-assessment of the ALSFRS-R. The app recently added an NfL diagram where patients can see their NfL status after a blood test is performed in a clinic.
Where prognostic information is accessed matters
While having access to this prognostic information outside a clinical setting can help patients feel more knowledgeable and empowered, it can also make them fearful or anxious about their disease’s progression. Here, Meyer and his colleagues surveyed 149 patients as part of a multicenter observational study on perspectives about this issue.
All the patients had access to standard care where information about ALSFRS-R and NfL were shared at routine clinical visits. A total of 57 people received ALSFRS-R updates in the clinic alone and 24 of them were also informed about their NfL levels. The remaining 92 people also conducted remote self-assessments of ALSFRS-R on the ALS app and 67 of them received NfL information via the app.
More than three-quarters of the participants believed having information about NfL levels or ALSFRS-R was personally relevant, and nearly all believed the data were relevant for research (98% for NfL, 96% for ALSFRS-R). More than three-quarters said having access to results from either measure was an information gain.
Around half of the participants given information about NfL levels (remote or in-clinic; 49.5%) or who performed a remote ALSFRS-R assessment (54.3%) indicated they were likely to recommend it to another ALS patient.
Concerns about ALS App
While few patients felt that receiving NfL information was burdensome, this was felt significantly more often by those who were given the results via the app over those who were informed in the clinic by a neurologist (12% vs. 4.2%). Remote digital assessment of ALSFRS-R was perceived as burdensome by 9.8%.
A total of 40.3% of all participants met the criteria for severe fear of disease progression, with women having significantly higher fear of progression scores than men.
While there were no significant differences in fear of progression between patients with remote or clinic ALSFRS-R assessments, those who received NfL updates through the app had significantly higher fear of progression. In the remote group, 56.7% exhibited severe fear of disease progression compared with 20.8% in the clinic group.
To prevent prognostic NfL information from becoming a burden, the researchers said if patients actually want to receive that information should always be considered.
The ALS App will be updated to give patients the option of deactivating the NfL diagram. Moreover, including informational resources in the app could help patients better comprehend how to interpret their test results.
Overall, “this study underscored that patients value information about NfL and ALSFRS-R, not only for their own understanding of the disease, but also for its relevance for ALS research,” the researchers wrote. “Future studies with larger [patient groups], longer observation intervals, and the inclusion of additional prognostic factors … will contribute to further improve the concept of patient-centered information sharing in ALS.”
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