Caring for Patients With Duchenne Muscular Dystrophy During COVID-19 Pandemic

Genetic research abstract – Duchenne muscular dystrophy
Tori Rodriguez, MA, LPC, discusses the progression of care for patients with Duchenne muscular dystrophy during the COVID-19 pandemic.

The coronavirus disease 2019 (COVID-19) pandemic resulted in widespread disruptions in care for patients with ongoing medical needs. To reduce the risk of virus exposure, many healthcare settings began offering nonurgent services via telemedicine or rescheduling in-person visits for a later date. These shifts in care have prompted concerns regarding the potential effects of treatment lags for patients with neuromuscular conditions including Duchenne muscular dystrophy (DMD).1,2

In a paper published in May 2020 in Muscle and Nerve, an expert panel of neuromuscular specialists provided consensus recommendations to guide the care of this population during the current global crisis.1 Although emerging data suggests that the course of COVID-19 may not be as severe as initially anticipated for patients with pediatric neuromuscular disorders, the panel emphasized the need for patients and their families to heed current public health guidelines regarding protective measures against the virus, including social distancing practices.1,3

Regarding ongoing management, the experts recommended that patients with DMD continue their existing corticosteroid treatment unless advised otherwise by their treating clinician. Patients and families should be informed of the risk of adrenal crisis associated with illness or steroid cessation. If patients become ill, dose adjustment may be required to prevent adrenal insufficiency. In the setting of acute illness or hospitalization, stress dose corticosteroids should be considered.4

If steroid changes are made during hospitalization, consultation with an endocrinologist is warranted. If a patient is unable to tolerate their regular steroid doses due to vomiting (a rare symptom of COVID-19) or other reasons, parenteral administration may be required, or a family member may need to deliver hydrocortisone intramuscularly.1

While patients receiving exon-skipping agents are generally advised to continue treatment, providers should discuss with patients and families the risks and benefits of receiving such infusions in healthcare settings during the pandemic. The option of home infusions should be discussed if appropriate.1

Regarding angiotensin‐converting enzyme inhibitors or angiotensin receptor blockers, which many patients with DMD receive for cardiomyopathy prophylaxis or treatment, there “has been some concern surrounding the use of these drugs due to the interplay of the SARS‐CoV‐2 virus and angiotensin‐converting enzyme 2, which is a coreceptor for the virus,” as explained in the paper.1 However, the relevant medical societies advise that patients continue these agents “in light of the known benefits to the heart and the uncertain risks of COVID‐19.” 

All decisions regarding treatment should be individualized and jointly considered between the patient, family, and providers, and no patient should be “labeled as ‘terminal’ and triaged for nontreatment simply on the basis of their disability and diagnosis,” according to the recommendations.1

To minimize the risk of virus exposure, standard monitoring practices – such as periodic blood work, cardiac MRIs, and X-rays – should be modified when possible and individualized to each patient. For some patients, it may be appropriate to delay routine monitoring or to consider options such as home-based blood draws or polysomnography.1

For patients with DMD who develop symptoms of COVID-19 and require emergency department assessment, patients and families should be instructed to “bring their home ventilatory support, including ventilators, masks, and mechanical insufflation‐exsufflation (cough assist) devices, with them and have their settings and respiratory treatment plans readily available,” the panel advised.1 Regular cough-assist treatments should continue while heeding appropriate safety measures including personal protective equipment for the treating personnel or caregivers.

The administration of supplemental oxygen without adequate ventilatory support may exacerbate hypercapnia in patients with DMD and is not advised for those with chronic respiratory insufficiency. Collaboration with pulmonologists and anesthesiologists should be considered when caring for these patients. When possible, neuromuscular blockade should be avoided if intubation is required, and depolarizing agents are contraindicated.1

Hydroxychloroquine is also contraindicated for patients with DMD and COVID-19, due to its uncertain benefits and associated risks of cardiac and skeletal muscle damage, vascular myopathy, and life-threatening arrhythmias.1

Many rehabilitative therapy services including physical, speech, and occupational therapies were halted due to the pandemic. Some of these services as well as psychological and behavioral care may be delivered via telehealth when appropriate.1,5 Patients, physicians, and therapists should jointly discuss and agree upon the urgency of these therapies and their suspension or continuation on a case-by-case basis.1

Additionally, home therapies and exercise should be considered, such as intermittent bracing, therapeutic positioning, and increased physical activity in the home and neighborhood. It should be noted, however, that during the pandemic, the “parent or caregiver has been forced to assume the sole responsibility for providing all the in-person therapy services that were previously provided by the therapists,” according to Stratton et al.2

Combined with the range of other stressors associated with the current pandemic and ongoing care of individuals with DMD, these added responsibilities may be exhausting and difficult for caregivers to manage. Providers involved in the ongoing care of patients with DMD should take this into consideration when making treatment recommendations that may increase caregiver burden.

Overall, despite various pandemic-related challenges to continuity of care for this population, with the “careful adherence to public health recommendations, careful management of acute medical issues, and use of telehealth technology, patients with neuromuscular conditions can continue high level medical and rehabilitation care to remain healthy and thrive.”2


1. Veerapandiyan A, Wagner KR, Apkon S, et al. The care of patients with Duchenne, Becker, and other muscular dystrophies in the COVID-19 pandemic. Muscle Nerve. 2020;62(1):41-45. doi:10.1002/mus.26902

2. Stratton AT, Roberts III RO, Kupfer O, Carry T, Parsons J, Apkon S. Pediatric neuromuscular disorders: Care considerations during the COVID-19 pandemic. J Pediatr Rehabil Med. 2020;13(3):405-414. doi:10.3233/PRM-200768

3. Natera-de Benito D, Aguilera-Albesa S, Costa-Comellas L, et al. COVID-19 in children with neuromuscular disorders. Published online January 2, 2021. J Neurol. doi:10.1007/s00415-020-10339-y

4. Bowden SA, Connolly AM, Kinnett K, Zeitler PS. Management of adrenal insufficiency risk after long-term systemic glucocorticoid therapy in Duchenne muscular dystrophy: clinical practice recommendations. J Neuromuscul Dis. 2019;6(1):31-41. doi:10.3233/JND-180346

5. Sobierajska-Rek A, Mański Ł, Jabłońska-Brudło J, Śledzińska K, Ucińska A, Wierzba J. Establishing a telerehabilitation program for patients with Duchenne muscular dystrophy in the COVID-19 pandemic. Published online December 21, 2020. Wien Klin Wochenschr. doi:10.1007/s00508-020-01786-8