Cystic fibrosis in the time of corona: the impact of the coronavirus pandemic on cystic fibrosis patients

As the coronavirus’ impact became grimly apparent in the healthcare sector, the economy, and the social fabrics of our communities, individuals with pre-existing conditions began to bear the brunt of this pandemic. High-risk groups have been facing a bleak outlook brought upon by an overstretched medical sector and a reeling economy. However, early fears of some high-risk groups soon came to pass in light of more medical research. Cystic Fibrosis (CF) is one such condition.

Early fears fueled by a cautious approach

According to the CDC, individuals with cancer, chronic kidney disease, COPD, obesity, serious heart conditions, sickle cell disease, diabetes, and those with compromised immune systems are at a higher risk for a COVID-19 infection. Meanwhile, those with asthma, hypertension, CF, etc. are warned about a possible higher risk for an infection. The real impact of COVID-19 virus on different groups has become more evident with the global expansion of the virus. For CF, early fears dwindled as the coronavirus gripped European countries like Italy and France, which have considerably more CF cases than China due to the higher genetic prevalence of the disease-causing gene in European countries.

The panic that spread across the CF community was not unfounded. CF patients are significantly more vulnerable to viral respiratory infections than the public, as witnessed during the 2009 H1N1 influenza pandemic. However, this heightened risk is mitigated with the coronavirus family, as CF patients are generally unaffected by CoV viruses. Data from the UK, Italy, and France suggests that CF patients are less likely to suffer a COVID-19 infection, and often exhibit mild symptoms without alterations to the course of the CF condition. Yet, concerns remain that novel COVID-19 strains may be more harmful to CF patients since certain Coronavirus species have linked to exacerbating CF symptoms. This worry must be addressed by careful monitoring of CF patients in COVID-19 hotspots to determine if certain coronavirus species have a detrimental impact on CF patients. In light of sparse academic information on the effects of the SARS-CoV-2 virus on CF patients, especially within the pediatric population, the advice has remained to follow strict public health procedures.

Behind the numbers: the reasons for low COVID-19 morbidity and mortality in CF patients

There are many reasons for the small number of COVID-19 cases in CF patients. Several cellular processes and characteristics conventional in CF patients have been hypothesized as the cause of the protective effect CF has against coronavirus. However, all remain speculative due to the small cohort of patients, shallow understanding of the SARS-CoV-2 virus, and the interactions between this virus and CF patients.

Another possible protective agent might be CFTR modulator therapy, commonly received by CF patients to counteract the damaging effects of the defected CFTR gene, the causative gene defect for CF. CFTR modulator therapy is an umbrella term encompassing several medical treatments that aid in repairing cellular function, improving airway hydration, lowering oxidative stress, and reducing the production of cellular products that worsen the CF symptoms, such as the NLRP3 inflammasome. This hypothesis rests on the positive impact of CFTR modulator therapy on smokers with lung disease. In simple terms, CFTR modulator therapy reduces the respiratory inflammation associated with CF and lung disease, promoting a better prognosis.

Other reasons for the CF community’s better prospects against the coronavirus include patient’s life-long adherence to public health procedures that protect against respiratory infections, such as using face masks, practicing effective hand disinfection, and staying five feet away from other CF patients.

Overall, an exciting opportunity is on the rise to use the protection enjoyed so far by CF patients against COVID-19 to accelerate meaningful research into new therapeutics and disease mitigation strategies against respiratory illnesses such as COVID-19 that can be applied to the general population. Increased attention to therapeutic methods and medicines used for CF can have favorable outcomes for the community. These include lower drug prices and better drug availability—issues that have strained CF patients and their families during the COVID-19 pandemic. While significant research attention is being diverted to the coronavirus, CF patients may benefit from research into related therapeutics.

On the same boat: the virus that challenges all

While the morbidity and mortality rates for CF patients have been better than the public, other strains continue to plague this resilient community. The ‘high-risk’ mentality and fear of the unknown risks posed by COVID-19 towards CF patients have kept the patients from hospitals and clinics, giving way to e-visits by health professionals. While online consultation can mitigate these risks, the quality of care would certainly be lowered due to the absence of PFT’s. It must be noted that all CF patients do not have the facilities required for online visitation, and for those on the severe end of the spectrum, lack of medical attention can be fatal.

Another issue is the diversion of funds and research attention from promising gene therapy and new therapeutics specific to CF patients in favor of COVID related treatments.

The impact of reduced hospital visits and in-person treatment on CF patient’s health is yet to be determined. It must be considered to comprehensively assess the effectiveness of the therapeutic strategies set for CF patients during the COVID-19 pandemic.

References

Centers for Disease Control and Prevention. 2020. Coronavirus Disease 2019 (COVID-19). [online]

Bell SC, Mall MA, Gutierrez H, et al. The future of cystic fibrosis care: a global perspective. Lancet Respir Med. 2020;8(1):65-124. doi:10.1016/S2213-2600(19)30337-6

Impact of COVID-19 on people with cystic fibrosis. Colombo, Carla et al. The Lancet Respiratory Medicine, Volume 8, Issue 5, e35 – e36

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Peckham, D., McDermott, M.F., Savic, S. et al. COVID-19 meets Cystic Fibrosis: for better or worse?. Genes Immun (2020). https://doi.org/10.1038/s41435-020-0103-y

Jarosz-Griffiths, H. H., Scambler, T., Wong, C. H., Lara-Reyna, S., Holbrook, J., Martinon, F., … & Clifton, I. (2020). Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis. Elife, 9, e54556.

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