Despite concerns that patients with Fabry disease might be at heightened risk of severe symptoms from SARS-CoV-2, early data find that most patients experience mild or moderate symptoms.
Patients with Fabry disease (FD) who contract COVID-19 should continue their FD therapy, and most of those patients are likely to face mild to moderate courses of the infection, according to a recent report.
The report, published in the Clinical Kidney Journal, offers early insights into how the pandemic is affecting patients with the rare disease and how physicians treating those patients should move forward.
The study is based on real-world data from 22 patients with FD who were known to have contracted SARS-CoV-2. Of those, 2 patients went on to experience critical complications from the infection. Both patients were over the age of 55 and had renal or cardiac disease.
Corresponding author Dawn A. Laney, MS, of Emory University, and colleagues explained that many clinicians who treat patients with FD worried that their patients might face higher risk from COVID-19, either due to the infection itself or due to interactions between FD therapies and COVID-19 treatments.
“The pathogenesis and target organ injury of COVID-19 are common to or partly overlap with those of FD,” the authors wrote, “raising theoretical concerns that the pre-existing FD pathology may serve as a ‘first hit’ that increases patients’ risk for serious complications when infected by COVID-19.”
The existing data, although limited, suggest that patients with advanced FD, including those who underwent kidney or cardiac transplant, those using immunosuppressive medications, and those with severe cardiovascular complications, are at highest risk of severe COVID-19.
As with the general population, the investigators said the best approach for clinicians is to help patients with FD take precautions to limit their risk of contracting the virus in the first place. The study was published before COVID-19 vaccinations became widely available, and thus the impact of the vaccine was not captured in the data set.
If a patient with FD does contract the virus, the authors wrote that the patient should continue to undergo full FD therapy. They said there is no evidence that renin-angiotensin system blocker has an effect on COVID-19, nor is there any known impact of the infection upon the effectiveness of enzyme replacement or chaperone therapy.
“Continued specific therapy for FD during the pandemic is endorsed, even if it requires visits to a medical center every 2 weeks for [enzyme replacement therapy] administration, since its benefits outweigh the perceived risk of developing COVID-19 in secure healthcare settings,” they noted.
Similarly, the investigators said patients who experience severe or critical COVID-19 symptoms should have access to the full range of therapies used against the virus, as well as organ support, if needed. They noted that the only therapies used to treat COVID-19 that are directly contraindicated for patients with FD are chloroquine and hydroxychloroquine. Still, they said that each COVID-19 medication should be reviewed carefully for safety concerns before administering it to patients with FD. If the patient had prior heart conditions, they said, it is especially important that the patient’s FD physician be included in any prescribing decisions.
The investigators said FD physicians should also keep careful tabs on local epidemiological conditions in order to assess patient risk.
The authors cautioned that their recommendations are based on a small amount of evidence, and thus can only be considered hypothetical at this point. They called for a registry to be set up to track COVID-19 in patients with FD.
“Such a registry would allow scientists and clinicians to explore the impact of genetic variations that have been associated with more severe COVID-19 in the general population,” they said.
Laney DA, Germain DP, Oliveira JP, et al. Fabry disease and COVID-19: international expert recommendations for management based on real-world experience. Clin Kidney J. 2020;13(6):913-925. doi:10.1093/ckj/sfaa227