In times of emergency, telemedicine, home-based treatments, and lab tests should be adopted whenever possible to help people with Fabry disease manage their condition while ensuring their safety, an observational study in Italy suggests.
The study, which focused on analyzing the clinical outcomes of Fabry patients during the COVID-19 outbreak, has found that all non-urgent treatments and clinical examinations were postponed — and telemedicine was used as much as possible for routine monitoring.
According to the researchers, these findings may be useful in creating future consensus guidelines on how to manage patients with Fabry using telemedicine and home-based treatments in a period of emergency.
The study, “Impact of COVID-19 pandemic on patients with Fabry disease: An Italian experience,” was published in the journal Molecular Genetics and Metabolism.
COVID-19 is the contagious respiratory disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). In March, the World Health Organization (WHO) declared COVID-19 a global pandemic. With 220,000 confirmed cases and 30,000 deaths in a period of two months, Italy was one of the most affected countries.
People with Fabry are among the group of individuals thought to have a higher risk of contracting COVID-19 and developing a more severe disease course.
“The rapid spread of COVID-19 combined with the consequent complete global lockdown, required a number of changes in our FD [Fabry disease] center organization to avoid unnecessary exposure of staff and patients to infection, while still continuing to provide care and support to our patients,” the researchers wrote.
Now, the investigators report the findings of an observational study that aimed to assess the impact COVID-19 had on the management and clinical outcomes of 129 Fabry patients who had been referred to the Fabry Center of Federico II University of Naples, one of the main specialized centers for the disease in the country.
Physicians contacted all patients by phone to gather information on their health status and organize their follow-up.
None of the 129 patients — 60 men and 69 women, with an average age of 47.5 years — reported any symptoms that could be associated with COVID-19, nor had direct contact with an infected individual. Given that, none of the patients had a nasal swab test to specifically identify the virus.
Only three patients included in the study had mild fever (less than 37.5 C or 99.5 F) and flu-like symptoms. These patients isolated themselves for 14 days, and no further investigations were carried out.
At the start of lockdown, a majority of patients (55%) were receiving intravenous enzyme replacement therapy (ERT) with either Replagal (agalsidase alpha) or Fabrazyme (agalsidase beta) to manage their condition. About a quarter (27.9%) were not receiving any form of treatment, and a smaller portion (12.4%) were being treated with oral Galafold (migalastat).
Those receiving Galafold (16 patients) continued treatment with no modifications or interruptions. All those receiving intravenous ERTs (71 patients) started being treated at home. Apart from eight patients who missed one infusion, all others continued receiving treatment on a regular basis. Those participating in clinical trials continued to have access to the study drug, which started to be given at home, during the pandemic.
To maintain social distancing and ensure patients’ safety, all non-urgent treatments, lab tests, and clinical examinations were postponed, while telemedicine was adopted as much as possible for routine monitoring.
“For patients needing laboratory surveillance, we limited it focusing on laboratory tests easily drawn at not a hospital-based laboratory,” the researchers wrote.
Additionally, the investigators suggested the use of urine dipsticks, so that patients could easily monitor the presence of proteins in the urine — an indication of kidney malfunction that is common in Fabry — without leaving their home.
“At present, no official indication exist on the management of FD [Fabry disease] patients during emergency and post-emergency period. Therefore, the analysis of the COVID-19 pandemic effects on medical care and health status of patients with FD will be useful to delineate consensus guidance,” the researchers concluded.
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