Effect of COVID-19 on People With Cystic Fibrosis in New York City Area

Although the presence of cystic fibrosis (CF) may increase a patient’s risk for complications from COVID-19 infection, the results of a recent study conducted in the New York metropolitan area (NY) have shown a lower prevalence of SARS-CoV-2 infection among persons with CF (pwCF) compared with those in the general population. A low rate of hospitalization also was reported among patients with CF during the first wave of the COVID-19 pandemic. These findings were recently published in the Journal of Cystic Fibrosis.

A multicenter, retrospective cohort study was conducted to evaluate the following: (1) the prevalence of COVID-19 infection in pwCF in New York; (2) the clinical characteristics, disease management, and outcomes associated with COVID-19 in pwCF; (3) the delays encountered in routine outpatient CF care; and (4) the effect of COVID-19 on the mental health of pwCF during the first COVID-19 wave.

The researchers hypothesized that the prevalence of COVID-19 would be lower in pwCF than in the general NY population, based on their observation of cases of COVID-19 within their centers and early international data. The investigation included pwCF in NY between March 1, 2020, and August 31, 2020, at 6 adult and 6 pediatric CF Centers (ie, Northwell Health, Mount Sinai Beth Israel, New York Medical College, New York Presbyterian-Columbia University Irving Medical Center, New York University Langone Health, and Stony Brook University Hospital).

In all participants, COVID-19 infection was diagnosed with the use of polymerase chain reaction (PCR) or immunoglobulin G (IgG) antibody positivity during the study period. The testing approach that was used and the timing were based on clinical indication and patient accessibility. In some of the centers, in-person clinic visits ceased by March 13, 2020, resuming as early as May 15, 2020, or as late as July 28, 2020, while telehealth visits were implemented.

During the first wave of the pandemic, those patients suspected of having COVID-19 underwent PCR testing. Most of the PCR tests were administered at urgent care centers or makeshift swabbing sites during the initial wave of the pandemic. By May 2020, antibody testing became available in New York and was carried out prior to the availability of vaccination in December 2020, thus eliminating the possibility of immunity from vaccination.

Of the 12 CF centers, 6 centers (3 pediatric and 3 adult centers) routinely offered COVID-19 antibody testing to patients; the other 6 centers, however, performed antibody testing on a case-by case basis, because of the following reasons: (1) PCR testing was not readily available during the early part of the pandemic; (2) the patients did not undergo PCR testing at the time they were suspected of having COVID-19; (3) the patients exhibited symptoms that were indistinguishable from those of COVID-19; and (4) the patients reported a suspected COVID-19 exposure.

The clinical features of positive COVID-19 cases that were examined included demographic characteristics, medical comorbidities, baseline forced expiratory volume in 1 second (FEV₁), suspected source of infection, home medications, reason for testing, symptoms, treatments received, at-home or in-hospital management, and mortality.

Delay in care was evaluated between March 1, 2020, and May 31, 2020 — a period when most in-person visits were suspended. Delay in care was defined as a “composite variable including at least 1 care plan that was not performed within an expected time frame and may be routine or patient-specific.” Included in the definition of delay in care were missed opportunities in at least 1 of the following: office visit, laboratory testing (eg, microbiology and blood tests), pulmonary function testing, elective surgery or invasive procedure, diagnostic imaging, subspecialty follow-up, or postponement of lung transplant listing. Patients’ anxiety and depression were evaluated qualitatively by each CF Center’s social worker or clinician by telephone or during a clinic visit.

A total of 810 pwCF from 12 CF centers in New York were enrolled in the study. The age of the patients ranged from 5 to 61 years, with a median age of 24 years; 46% of the patients were female. The 2 most common comorbidities were pancreatic insufficiency in 96% of the patients and CF-related diabetes in 38%. The average FEV₁ was 2.2 L (69% of predicted).

In the full study cohort, the prevalence of COVID-19 infection by PCR was 1.6% (13 of 810) of the patients. This rate is lower than that in the NY general population (2.69%). Per IgG antibody screening, the prevalence of COVID-19 was 12.2% (18 of 147) among those who were tested. This antibody positivity rate is lower than the seroprevalence of the NY general population at the time estimated in the literature — which may have been as high as 24% to 44%.

Overall, 26 distinct cases of COVID-19 were detected, which included 5 patients who tested positive by both PCR and IgG antibody, 8 by COVID-19 PCR testing alone, and 13 by COVID-19 IgG antibody testing alone. In total, 8 of the13 PCR-positive patients were symptomatic, whereas 13 of the 18 IgG-positive patients were asymptomatic. 

The protective effect of cystic fibrosis transmembrane conductance regulator (CFTR) modulators is recognized as correcting the underlying protein defect that was associated with improved airway ciliary function in the pwCF, thus reducing pulmonary exacerbations and improving lung function. The fact that elexacaftor/tezacaftor/ ivacaftor (ETI) was approved by the US Food and Drug Administration in 2019 — prior to the 2020 onset of the COVID-19 pandemic — is likely responsible for  the significant reduction in pulmonary exacerbations by 51% in 2020 compared with 2019.

The researchers concluded, “The prevalence of COVID-19 among pwCF in NY during the pandemic first wave was low and most cases were managed at home. CFTR modulators may be protective. PwCF experienced delay in routine care and increased anxiety.”

The investigators added that additional studies are warranted to better understand the long-term impact of COVID-19 on pwCF as the overall population becomes vaccinated and social contact begins to return to normal.

Reference

Simonson JL, Esposito C, Frantzen T, et al. The clinical impact of the COVID-19 pandemic first wave on patients with cystic fibrosis in New York. J Cyst Fibros. Published online February 21, 2022. doi:10.1016/j.jcf.2022.02.012