Caring for sufferers with Duchenne muscular dystrophy in the course of the COVID-19 pandemic

The 2019 coronavirus disease (COVID-19) pandemic resulted in widespread disruption in the care of patients with ongoing medical needs. To reduce the risk of virus exposure, many health care facilities provide non-urgent telemedicine services or reschedule personal visits for a later date. These shifts in care have raised concerns about the potential impact of treatment delays in patients with neuromuscular disorders including Duchenne muscular dystrophy (DMD) 1,2

In an article published in Muscle and Nerve in May 2020, an expert panel of neuromuscular specialists made consensus recommendations to steer care for this population during the current global crisis.1 Although emerging data suggests that the course of COVID-19 may not be so The panel emphasized the need for patients and their families to adhere to current public health guidelines regarding protective measures against the virus, including social distancing practices.1,3

In terms of ongoing management, the experts recommended patients with DMD to continue their existing corticosteroid treatment unless their attending physician recommends otherwise. Patients and families should be made aware of the risk of adrenal crisis associated with illness or steroid withdrawal. When patients get sick, a dose adjustment may be needed to prevent adrenal insufficiency. In acute illness or hospitalization, stress-dose corticosteroids should be considered. 4

Consultation with an endocrinologist is required if steroid changes are made during hospitalization. If a patient cannot tolerate their regular steroid doses because of vomiting (a rare symptom of COVID-19) or other reasons, parenteral administration may be required or a family member may need to administer hydrocortisone intramuscularly

While patients receiving exon skipping agents are generally recommended to continue treatment, providers should discuss with patients and families the healthcare risks and benefits of such an infusion during the pandemic. The option of home infusions should be discussed as appropriate

With regard to angiotensin converting enzyme inhibitors or angiotensin receptor blockers, which many patients with DMD receive for the prophylaxis or treatment of cardiomyopathies, there have been some concerns about their use due to the interaction of the SARS-CoV-2 virus and angiotensin of these drugs converting enzyme 2, which is a co-receptor for the virus, “as explained in the publication.1 However, relevant medical societies advise patients to continue using these agents,” given the known benefits to the heart and the uncertain risks of COVID -19. ”

All treatment decisions should be individualized and considered jointly between the patient, the family and the care provider, and no patient should be “marked terminal” as recommended and only checked for non-treatment based on their disability and diagnosis ”. 1

To minimize the risk of virus exposure, standard monitoring practices such as regular blood tests, cardiac MRIs, and X-rays should be modified whenever possible and tailored to each patient. Some patients may need to delay routine monitoring or consider options such as home blood tests or polysomnography

For patients with DMD who develop symptoms of COVID-19 and require an emergency room assessment, patients and families should be instructed to “bring and bring their home ventilation equipment, including ventilators, masks, and mechanical insufflation-exsufflation (cough assist) equipment Have your settings and breathing treatment plans ready, ”advised the panel.1 Regular cough treatments should be continued while appropriate safety measures, including personal protective equipment, are observed for the treating staff or caregivers.

Administration of supplemental oxygen without adequate ventilation support can worsen hypercapnia in patients with DMD and is not recommended for patients with chronic respiratory failure. Cooperation with pulmonologists and anesthetists should be considered when caring for these patients. If possible, neuromuscular block should be avoided when intubation is required and depolarizers are contraindicated. 1

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

Many rehabilitative therapy services, including physical therapy, speech therapy, and occupational therapy, were suspended due to the pandemic. Some of these services, as well as psychological and behavioral care, may be provided via telemedicine, if necessary.1,5 Patients, doctors and therapists should discuss the urgency of these therapies and whether they should be suspended or continued on a case-by-case basis

In addition, home therapies and exercise should be considered, such as: B. intermittent tension, therapeutic positioning and increased physical activity at home and in the neighborhood. It should be noted, however, that during the pandemic, the “parent or caregiver was forced to assume sole responsibility for providing any personal therapy services previously provided by therapists,” according to Stratton et al.2

Combined with the number of other stressors associated with the current pandemic and ongoing care for people with DMD, these additional responsibilities can be stressful and difficult for caregivers to manage. Providers involved in the ongoing care of patients with DMD should consider this when making treatment recommendations that may increase the burden on caregivers.

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

References

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

2. Stratton AT, Roberts III RO, Copper O, Carry T, Parsons J, Apkon S. Neuromuscular Disorders in Children: 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 the risk of adrenal insufficiency after long-term systemic glucocorticoid therapy for Duchenne muscular dystrophy: recommendations for clinical practice. 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. Establish a telerehabilitation program for patients with Duchenne muscular dystrophy in the COVID-19 pandemic. Published online on December 21, 2020. Wien Klin Wochenschr. doi: 10.1007 / s00508-020-01786-8