Prioritizing Neuromuscular Patients for COVID-19 Vaccination

Barry J. Byrne, MD, PhD

People with neuromuscular diseases should be among the first to receive COVID-19 vaccinations, following health care workers and residents and staff of long-term care facilities. The Muscular Dystrophy Association (MDA) is advocating at the national level and is leading a coalition of 20 partner organizations representing people with neuromuscular diseases that are advocating with more than 55 state and local governments, to help ensure that this happens. So far, these patients have not been on CDC’s high-priority list, and we are encouraging these state and local entities to include them in Phase 1 of their COVID vaccine administration efforts.

This advocacy is being done on behalf of over 300,000 Americans living with neuromuscular disease represented by MDA. The experiences, comorbidities, and impacts of these neuromuscular diseases should serve as qualifying factors for Phase 1 vaccination for these individuals who are over 16 years of age.

The CDC’s Advisory Committee on Immunization Practices (ACIP) has been making recommendations on the prioritization of groups for COVID vaccination. While these decisions will ultimately be up to the states, ACIPs recommendations carry a lot of weight.

ACIP’s 1C priority group currently encompasses people with serious underlying conditions. So far, ACIP has not included neuromuscular conditions such as amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA), and the muscular dystrophies in this high-priority group. Those of us who treat these conditions and the 40-odd other neuromuscular diseases know that our patients are immunocompromised and at high risk for SARS-CoV-2 infection and serious complications such as acute respiratory distress syndrome (ARDS). While I haven’t yet seen anything in the medical literature about it, I have no doubt that these patients have higher than average COVID-related mortality rates as well.

The wide range of neuromuscular conditions vary in symptom severity and patient experience, but generally implicate the peripheral nervous system, resulting in progressive muscle weakness affecting both skeletal muscles and the muscles of internal organs. Mobility issues as well as cardiac, pulmonary, and digestive complications are the hallmarks of neuromuscular diseases that include ALS, muscular dystrophies, and SMA, along with many myopathies and mitochondrial diseases, among others.

Our older patients and those with cardiorespiratory problems are at especially high risk and should be given the highest priority. Most individuals living with neuromuscular diseases experience a multisystem impact from their conditions. These diseases can weaken the pulmonary muscles and diaphragm as they progress, increasing the risk of severe pulmonary infection. Many neuromuscular patients also have underlying cardiovascular disease. Research is increasingly showing that these individuals are at high risk of acute cardiac injury during the course of their illness. These factors do not bode well for their potential COVID-related outcomes.

Individuals with neuromuscular diseases are often prescribed corticosteroids to combat muscle weakening. The CDC has stated that individuals who take corticosteroids “might be at increased risk” due to the associated weakening of the immune system. Individuals with certain neuromuscular diseases, such as myasthenia gravis and Lambert-Eaton myasthenic syndrome, require immunosuppressant treatment, leaving them more vulnerable to contracting severe COVID-19. The CDC also lists neurological conditions as a potential risk factor for severe COVID.

Neuromuscular clinical care in the United States has become much more challenging since the start of the pandemic and is expected to worsen as the virus surges in the coming months. Measuring progression and planning interventions rely on in-person visits that may now be unsafe or impossible in some cases. 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. Many patients have been relying on telehealth since the start of the COVID pandemic, and this is not ideal in diseases that must be regularly monitored and treated in multidisciplinary settings such as MDA Care Centers.

Finally, each individual neuromuscular disease is considered rare (fewer than 100,000 cases in the US). Due to this “rare” status, there is little to no research on the effects of COVID-19 on these patients. This indicates that there may be additional risk factors that increase susceptibility to severe COVID-19 infection of which we may be unaware.

For all of these reasons, the MDA asks the CDC and state and local governments to give high priority to vaccinating individuals with neuromuscular diseases against COVID-19.

Barry J. Byrne, MD, PhD, is the chief medical advisor at the Muscular Dystrophy Association, and the director of the Powell Center for Rare Disease Research and Therapy at the University of Florida Health.