Coronavirus and Cystic Fibrosis; What Do We Know?

Note: The COVID-19 pandemic caused by the novel SARS-CoV-2 virus will be referred to as Coronavirus in this article.

The Coronavirus pandemic has been a concerning time for everyone. But it’s been particularly concerning for people with cystic fibrosis (CF) and their families, given the potential increased risks of severe infections for patients with underlying lung diseases. When the pandemic began, we were unsure if people with CF would become very unwell if infected with Coronavirus. Now, nearly 3 months since the pandemic arrived in Australia, we have some evidence regarding the risk of Coronavirus to people with CF. And the good news is it doesn’t appear to affect people with CF more severely.

Evidence to date

So far, the best evidence to guide us regarding the effects of Coronavirus comes from a report recently published in the Journal of Cystic Fibrosis¹. The national CF registries of 8 countries, including Australia and several countries with high rates of Coronavirus infections such as the United Kingdom and United States of America, reported all identified cases up to 13^th April, 2020. They identified 40 people with CF who had been infected with Coronavirus. They were mainly adults, with the youngest being 15 years’ old. Within that group there was a wide range of CF lung disease severity, with some patients having severe disease and significantly reduced lung function (i.e FEV₁ <40%) before being infected. Eleven patients had previously undergone a lung transplant. All of the 40 patients survived: 13 needed oxygen treatment and only 1 needed to be placed on a ventilator. One of the people with CF was pregnant, and she recovered and delivered a healthy baby. The overall rate of Coronavirus infection in the CF population was calculated to be lower than that of the general population in the 8 countries involved. Around the same time as the multi-national report was published, there was a smaller study regarding Coronavirus infection in the United Kingdom, France, Italy, Germany and Spain². It also concluded the infections were rarer in people with CF when compared to the overall population. The authors of both studies concluded firstly, that the rates of Coronavirus appeared to be lower than in the general population. They both proposed that one of the potential reasons why people with CF had lower rates of infection was that, along with their families, they understood the importance of isolating and protecting themselves from cross-infection.  Secondly, the authors concluded that in infected cases there was no evidence to suggest that people with CF had a more severe illness. In other words, people with CF who were infected appeared to have the same prospect of recovery and the same risk of becoming very unwell as those without CF.

 Limitations

There are limitations to these reports. For instance, infections can often be asymptomatic (in other words people who have Coronavirus show no symptoms) and so the true rate of cases in people with CF may be higher than was documented. However, if this is the case, the missed cases are likely mild and not of concern. Also, there has been very limited reporting regarding children. There is one report³ of a 1-month-old boy with CF in Italy, who contracted Coronavirus from his grandfather, but was asymptomatic and did not have to go to hospital. Again, if children were being infected and becoming very unwell, they probably would not have been missed in the reports.

 What is AREST CF Doing?

Members of the AREST CF community will be interested to know what the AREST CF team have been doing in response to the COVID-19 pandemic. Some clinicians involved in AREST CF have been busy working as part of the CF teams at Perth Children’s Hospital and the Royal Children’s Hospital to adapt to delivering CF care remotely. From a research perspective, members of the team at Telethon Kids Institute (led by Associate Prof. Anthony Kicic) are developing experiments to better understand how Coronavirus infects lung tissue in the laboratory. The CF team at the Royal Children’s Hospital are also planning to study the effects of moving all CF clinics to remote telehealth clinics. Given the risks of cross infection, this may be beneficial to CF care moving forward.

 Summary

The evidence generated so far tells us that people with CF are not at increased risk of either contracting Coronavirus, nor of having a more severe illness. We are constantly learning more about this virus and its effects, and so maintaining appropriate social distancing measures (as guided by health officials and your CF team) is still important. In addition, the effects of the pandemic and social distancing on normal CF care have not been studied. It is important to try to continue all the normal aspects of CF care (such as taking medications, doing airway clearance and exercise, and maintaining regular contact with your CF team) as much as is safe and possible. If you have any questions or concerns, a member of your CF team is the best point of contact.

1. https://www.sciencedirect.com/science/article/pii/S1569199320301259

2. https://www.thelancet.com/pdfs/journals/lanres/PIIS2213-2600(20)30177-6.pdf

3. https://www.cysticfibrosisjournal.com/article/S1569-1993(20)30096-5/pdf