key: cord-274779-0emfl1e5
authors: Andrews, Jinsy A.; Berry, James D.; Baloh, Robert H.; Carberry, Nathan; Cudkowicz, Merit E.; Dedi, Brixhilda; Glass, Jonathan; Maragakis, Nicholas J.; Miller, Timothy M.; Paganoni, Sabrina; Rothstein, Jeffrey D.; Shefner, Jeremy M.; Simmons, Zachary; Weiss, Michael D.; Bedlack, Richard S.
title: Amyotrophic lateral sclerosis care and research in the United States during the COVID‐19 pandemic: Challenges and opportunities
date: 2020-06-05
journal: Muscle Nerve
DOI: 10.1002/mus.26989
sha: 
doc_id: 274779
cord_uid: 0emfl1e5

Coronavirus disease 2019 has created unprecedented challenges for amyotrophic lateral sclerosis (ALS) clinical care and research in the United States. Traditional evaluations for making an ALS diagnosis, measuring progression, and planning interventions rely on in‐person visits that may now be unsafe or impossible. Evidence‐ and experience‐based treatment options, such as multidisciplinary team care, feeding tubes, wheelchairs, home health, and hospice, have become more difficult to obtain and in some places are unavailable. In addition, the pandemic has impacted ALS clinical trials by impairing the ability to obtain measurements for trial eligibility, to monitor safety and efficacy outcomes, and to dispense study drug, as these also often rely on in‐person visits. We review opportunities for overcoming some of these challenges through telemedicine and novel measurements. These can reoptimize ALS care and research in the current setting and during future events that may limit travel and face‐to‐face interactions.

impact on amyotrophic lateral sclerosis (ALS) care and research is illustrated in a recent survey of members of the Northeast ALS (NEALS) Consortium, a network of ALS clinical centers. Most of the 133 sites surveyed between April 21, 2020 and May 1, 2020 are affiliated with academic medical centers in the United States, have multidisciplinary care teams, see large numbers of patients, and participate in a variety of ALS research studies, including clinical trials. 6 The survey itself is included in Figure S1 (see Supplementary Material online). Results from the 61 sites that responded are shown in Tables 1, 2, and 3.

The first two survey questions asked about available options for the clinical evaluation of new and return patients (Table 1) . One third of responding centers were no longer able to see new patients in-person.

More than half of the responders were able to see new patients from their same state by video visit. Smaller numbers were able to offer video visits outside their state. Only 3% of responders were unable to see any new patients. With regard to follow-up patients, again a large number of responders (47%) were unable to see these in-person.

Most could offer video visits to return patients in their same state, with smaller numbers being able see patients from other states. All responders were able to see follow-up patients in some manner.

Question 3 asked about evidence-and experience-based ALS measurements and treatments that may be challenging to obtain during the pandemic (Table 2) . Spirometry was unavailable at most sites. Many sites reported difficulty getting feeding tubes, multidisciplinary team care, wheelchairs, home health/hospice, lifts, or hospital beds.

Only 16% of responders reported being able to get everything they could for their patients before the pandemic.

Question 4 asked about available options for research participants (Table 3 ). Very few responding centers were able to enroll new participants into studies, either in-person or by phone or video. Most were unable to see participants for return in-person visits. About half the responders were able to see return participants virtually, even those residing in states beyond where the clinic was located. Twenty percent of responders were unable to see any research participants by any means during the pandemic.

Our survey results confirm that COVID-19 has created specific challenges to ALS care and research in the United States. The first of these is making/confirming new ALS diagnoses. It is not clear how this may occur at sites that are now unable to offer in-person visits. Current ALS diagnostic criteria require a physical examination showing widespread upper and lower motor neuron signs. 7, 8 Although some aspects of a neurological examination can now be performed virtually, including measures of muscle bulk and power, assessment of muscle tone and tendon reflexes still cannot. 9 This is likely to aggravate the already significant problem of diagnostic delay in ALS. 10 Unfortunately, there may be no immediate way around it because previous work has shown that preliminary ALS diagnoses by nonexperts are inaccurate about 5% of the time. 11 Even if a clinician could be certain of the findings documented by another provider, he or she may not feel comfortable breaking the news of an ALS diagnosis virtually. This idea is supported by a survey of clinicians

Options for evaluation of new and return clinic patients at NEALS sites showing they felt that telemedicine visits lacked the emotional connection associated with in-person visits. 12 The second challenge relates to monitoring disease progression for clinical care and in trials. The most commonly used measure of ALS progression in clinics and in trials, the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R), can be obtained reliably and easily over the phone or by telemedicine. 13 home health and hospice, which provide education as well as support and palliative care, are also all currently unavailable at some NEALS some sites. These restrictions can also affect research studies. It has largely been assumed that baseline ALS care being provided at different sites of a multicenter trial is similar; this assumption is currently incorrect.

The rapid expansion of telemedicine represents an exciting opportunity to improve clinical care and expand access to research opportunities for many patients (Table 4 ). At the beginning of 2020, a small but growing number of ALS centers were offering telemedicine visits to patients in their homes. 12, [19] [20] [21] Because no billing codes existed for this, it was supported by philanthropy, ALS nonprofits, or local institutions. Many states had laws restricting telemedicine visits to clinicians licensed in that state. 22 The current pandemic has catalyzed the creation of new billing codes for telemedicine in the home, 23 12 other approaches can also be successful.

These can include hybrid models in which some clinicians see patients face-to-face and others see them virtually, 29 models where the physician visit is scheduled and follow-up visits with the multidisciplinary team occur ad hoc over time, 19 and asynchronous visits using recorded video for patients without access to internet. 17 In the Veterans Affairs hospital system, one analysis demonstrated that quality of ALS care was independent of its delivery by telemedicine or in clinic. 20 Some measurements or evaluations will need to be altered to be administered via telemedicine. Although spirometry is generally considered a clinic-based assessment, it can also be done using a home spirometer and having a trained clinic staff monitor the patient as he/she performs the spirometry. 30 Surrogates for spirometry could also be explored for ALS patients.

These may include single-breath counting or vocalizing a sound; these have proven to correlate with spirometry in other study populations. 31 When it comes to using telemedicine for research, remote or vir- speech analysis can be evaluated through recordings from cellphone apps to monitor disease progression. 33 Medical surveys can be delivered to people with ALS, and quantitative data about movement can be collected. As algorithms for processing data mature, passive cell phone data could be used to inform providers about patient wellbeing. 34, 35 Wearables may paint an even more detailed picture of patient function and behavior, although with the cost of requiring the patient to wear an additional device. 35 

The COVID-19 pandemic has created an unprecedented challenges to ALS clinical care and research. The traditional face-to-face paradigm of medical decisionmaking and trial conduct have become difficult or impossible for many sites. An unexpected silver lining to these challenges is the development and maturation of a telemedicine infrastructure, providing clinicians and researchers with opportunities to fortify and even improve the way we approach ALS care and research.

Our combined experiences of clinician-patient interactions during this pandemic will provide us with new paradigms that will likely improve the efficiency of clinical care and availability of research participation.

Additional supporting information may be found online in the Supporting Information section at the end of this article. 

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