A Cystic Fibrosis Foundation display at the organization’s 2019 conference in Nashville, Tennessee, urges a cure for CF. Credit: Larry Luxner

Long before anyone had heard of COVID-19 or social distancing, people with cystic fibrosis (CF) knew they had to stay 6 feet away from other CF patients to avoid infecting each other.

Yet the pandemic has had surprisingly little negative effect on children with CF, according to a study cosponsored by the Bethesda, Maryland-based Cystic Fibrosis Foundation (CFF). On the other hand, it has changed—perhaps permanently—the way patients receive care, CFF officials said during the organization’s 2022 national annual meeting.

“Prior to the pandemic, there was a lot of talk at CFF, and a strategic plan to work on a future model of CF care, because we knew the needs of the community were changing,” Whitney Brown, MD, senior director of clinical affairs at CFF, said during the virtual conference. “That was already in motion, but really it was the pandemic that accelerated this concept of evolving care, and the rapid uptake of ‘telehelp’ by necessity. As a byproduct, there’s been a ton of innovation and shared learning.”

The study, carried out by the Cystic Fibrosis Registry Global Harmonization Group (which includes the CFF), appeared in the Journal of Cystic Fibrosis. It was based on 105 CF patients aged less than 18 years who developed SARS-CoV-2 infections between February 1, 2020, and August 7, 2020—before the emergence of the Delta and Omicron variants of the disease. The patients were in the US, Argentina, Brazil, Chile, France, Germany, Italy, Russia, South Africa, Spain, Sweden, Switzerland, and the UK.

It concluded that COVID-19 usually causes mild illness in pediatric CF patients without severe pre-existing lung disease—with 70% of cases managed at home. Of the 24 patients requiring hospitalization, only 6 needed supplemental oxygen during their admission and 2 received noninvasive ventilation.

By the study’s conclusion, 2 children were still recovering, while all others had made a full recovery. No deaths were attributed to COVID-19.

“We are highly encouraged by these findings on the impact of COVID-19 in children with CF,” Bruce Marshall, MD, the CFF’s chief medical officer and one of the paper’s authors, said in a press release. “However, further studies are needed to fully understand the impacts of this novel disease. We continue to urge all people living with CF to continue to take precautions to avoid exposure to COVID-19 and to work directly with their care teams to assess their personal risk during this time.”

CF Patients With Advanced Lung Disease at Increased Risk

The CFF monitors COVID-19 cases through its patient registry, which includes more than 30,000 people with CF being treated at a network of 133 care centers across the United States. Albert Faro, MD, the CFF’s vice president for clinical affairs, said the study has “allayed some concern” that COVID-19 would be devastating for people with CF.

“Multiple studies that have now been published from the data we’ve collected from this collaborative effort indicate that people with CF at greater risk for poor outcomes from COVID are those with advanced lung disease—those who go into the infection already with lower lung function as well as those who’ve had a lung transplant,” Dr. Faro explained.

“People who’ve had a lung transplant, in a sense, have a different disease process,” he said. “In addition to still having cystic fibrosis, though, they’re now immunocompromised.”

Albert Faro, MD
Albert Faro, MD. Credit: Larry Luxner

Dr. Faro, a pediatric pulmonologist who’s been with the CFF for the past 5 years, said that since the study’s conclusion, anecdotal evidence suggests that people with CF continue to fare well during the pandemic—possibly because they’re among the most prudent of all Americans when it comes to masking as well as maintaining physical distance from others.

Asked if there were any particular concerns regarding vaccines and boosters, Dr. Faro said: “We encourage all people with CF who are eligible to get vaccinated—and that includes receiving a booster.“ During the Omicron surge, trends in the CF population mirrored what was seen in the general population—that the people who got particularly sick from the variant were those who had not been vaccinated, he said.

People With CF Now Living Longer Than Ever

Since the CFF’s establishment in 1955, the average life expectancy for Americans born with CF has skyrocketed from 5 years to about 50 years. Dr. Faro said the 3 main factors accounting for this dramatic jump are improved patient care, the development of cystic fibrosis transmembrane conductance regulator (CFTR) modular therapies, and more access to lung transplants.

Unlike in past years, Dr. Faro said, a network of about 280 accredited CF care programs now offers patients access to “more than just the lung doctor, but also the dietitian, the respiratory therapist, the social worker, nurses, and now gastroenterologists and endocrinologists.”

He said the 4 FDA-approved CFTR therapies now on the market have made an enormous difference in treating CF. The therapies, all developed by Vertex Pharmaceuticals, are ivacaftor (Kalydeco®), lumacaftor/ivacaftor (Orkambi®), tezacaftor/ivacaftor (Symdeko®), and elexacaftor/tezacaftor/ivacaftor (Trikafta®).

“These are drugs that, for the very first time, address the basic cellular defect of the chloride channel in people with cystic fibrosis to now allow for the normal movement of ions and water, which then allows the mucus not to be as desiccated and dehydrated as it is in people with cystic fibrosis,” Dr. Faro said.

The third factor, better access to lung transplants, is one Dr. Faro knows well as the leader of the CFF’s Advanced Lung Disease Initiative.

“Five years ago, there wasn’t a dedicated program to try and help people who were pursuing the lung transplant journey,” he said. “People with CF and their families had expressed frustration around the transplant journey and the referral process. There was a lack of standardization of therapies and approaches to transplant.”

To remedy the situation, Dr. Faro said the CFF has put together a consortium of 15 transplant programs across the nation and is also funding an observational study of chronic lung allograft dysfunction, or CLAD, which remains the biggest obstacle to good long-term outcomes for people after transplant.