Data on COVID-19 and cystic fibrosis, one year on

Blog - Rebecca Cosgriff, Director of Data and Quality Improvement -

It’s been over a year since the first lockdown was announced in the UK. It’s also been a year since an international collaboration of global cystic fibrosis (CF) Registries, led by the Cystic Fibrosis Trust, began monitoring coronavirus (COVID-19) in people with cystic fibrosis. In this blog, Rebecca Cosgriff, the Trust’s Director of Data and Quality Improvement, reflects on the swift actions taken by CF Registries worldwide, what we now know, and what the future holds for studying COVID-19 and cystic fibrosis.

Coronavirus has loomed large over almost every aspect of our lives for more than 12 months. Suddenly, it wasn’t only people with CF or those immunosuppressed who had cross-infection concerns dominate their work, home and social lives; the whole world was abruptly confronted with the possibility of suffering serious health consequences resulting from a viral infection.

However, following severe consequences of previous epidemics for people with CF and a lack of real-world data on coronavirus in March 2020, we were all concerned that encountering COVID-19 could be catastrophic for anyone with cystic fibrosis. People with CF were designated as clinically extremely vulnerable to the infection and told to shield, without leaving their homes at all. The fear of not being able to venture further than their own garden, house, or even a single room meant significant anxiety about the future for much of the CF community.

It was clear that we needed to work together to advance our understanding of how COVID-19 affects people with CF, and who within the community might be at higher or lower risk, as quickly as possible. However, given that people with CF are incredibly effective at guarding against infection, no single country had sufficient data to draw these conclusions as quickly as they were needed by a worried population of people with CF, their loved ones, and their healthcare providers.

What did we do?

The UK CF Registry team quickly set up a real-time COVID-19 monitoring process, with support from the UK CF Medical Association (UKCFMA) and clinical teams all around the UK. This meant we knew about each case as it happened, and could share learnings between those responsible for providing care and add headline figures to our website for everyone to access and stay up to date.

As well as keeping a close eye on UK cases, we mobilised a global collaboration of CF Registries to monitor COVID-19 on a global scale. In April 2020, this collaboration published a paper, of the first 40 documented cases of COVID-19 in people with CF worldwide. Since then, a paper looking at COVID-19 specifically in children with CF has been published, with the analysis carried out by Trust-funded Summer Student Robbie Bain, who pivoted to this research after his original lab-based project had to stop because of the pandemic.

UK CF Registry statistician Elliot McClenaghan analysed data on 181 cases of COVID-19 from 19 countries around the world for a second study, which was published in the Journal of Cystic Fibrosis last winter. I was privileged to present these results on behalf of the collaboration at the virtual international North American CF Conference (NACFC), with content available on-demand and open to all.

We also worked with the European Cystic Fibrosis Patient Registry to publish a paper comparing outcomes of people with CF with the general population.

What do we know now?

Thanks to over 85,000 people with CF worldwide who consented to participate in a Registry, we now know that the outcomes of COVID-19 for people with CF are not as bad as we initially feared. That being said, people with CF are more likely to be admitted to hospital due to COVID-19 than people in the same age group within the general population. It also appears that those with lower lung function, or who have received a transplant, might be at higher risk of a severe infection that requires admission to hospital. The number of people with CF who have died of COVID-19 is low worldwide, but unfortunately some have still been reported. In the UK, there have sadly been two people with CF who have lost their lives to this infection.

At the beginning of the pandemic most people with CF infected with COVID-19 were hospitalised, but over the last 12 months this has reduced significantly. This might be because there is more confidence in people with CF managing symptoms at home without having to be admitted, as evidence has become available and clinical teams get more experience of the infection.

The highest number of diagnoses in any one month was in January 2021, when 82 people with CF were recorded as being diagnosed with COVID-19. Since then, the number of COVID-19 infections has dropped sharply, with 17 cases in February, 8 in March, and less than five so far in April. As of 26 April 2021, the UK has had a total number of 325 cases of COVID-19 reported among people with CF, with less than 20% being admitted to hospital. Since January 2021, as well as a drop in numbers of cases overall, we have also seen a fall in the proportion of adults aged 16 and over acquiring COVID-19. This might be due to people with CF over 16 have receiving a COVID-19 vaccination.

What does the future hold?

Right now, the UK CF Registry team is working on an analysis of over 1,500 cases of COVID-19 from 22 countries from around the world. We hope that the number of cases included means we will be able to provide clearer information about what characteristics put people with CF at higher risk of severe COVID-19 infection. People with CF from around the world are involved in this study, helping us to write the manuscript and communicate the results in a meaningful and understandable way.

We are now collecting COVID-19 vaccination data on the UK CF Registry, so that we can do future research on vaccine uptake and its effectiveness in preventing COVID-19, or severe infection, among people with cystic fibrosis. Because of the CF community’s long-standing support of Registries around the world, we can continue to monitor COVID-19 to identify risk factors, spot any long-term impacts of infection, and evaluate the effectiveness of vaccination efforts. We can use this information to support clinical decision making, guide government policy decisions, and give timely information to people with CF and their families.

Now that shielding has ended and restrictions are beginning to lift across the UK, it’s vital that people with CF take up both doses of the COVID-19 vaccine when offered, and speak to their clinical team about any questions they might have. The latest guidance on the vaccine from the UKCFMA is available here.

I want to say thank you to people with CF, their families, and their clinical teams for making all this research possible. We will continue to find out as much as we can, as quickly as we can, as we begin to look towards the light at the end of the tunnel of this pandemic. 

I would also like to thank my colleagues around the world who have supported this work without additional resource, across borders and time zones (some dialing in to our twice-monthly calls at midnight!), and even from within war zones. It has been a real honour to work with this group of dedicated professionals, determined to make a real difference to people with CF during extremely challenging times.  

To help the Trust fund vital research like this and support people with CF during the COVID-19 pandemic and beyond, please consider making a donation today.

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