Canadian Report Shows Promising Trends in CF Health

Lindsey Shapiro, PhD avatar

by Lindsey Shapiro, PhD |

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Cystic Fibrosis Canada’s 2020 annual data report from the Canadian Cystic Fibrosis Registry has been released, and it shows several improvements in the health of people with cystic fibrosis (CF), and a limited impact of the COVID-19 pandemic on health outcomes.

“The Canadian Cystic Fibrosis Registry has made a remarkable impact on our understanding and treatment of the disease, and its importance continues. The data helps us to track trends in the Canadian CF community and understand the progress and impact that has been made to advance health outcomes,” Kelly Grover, president and CEO of CF Canada, said in a press release.

The report indicates that 4,332 Canadians were living with CF in 2020; 62% of them were adults. In total, 87 new diagnoses were made in 2020 — 64 of these new diagnoses were made through newborn screening. The median age of survival in 2020 was 55.4, showing a substantial improvement from 20 years ago, when the median age of survival was 35.5 years.

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CF patients attended more than 18,000 virtual or in-person clinic visits and spent more than 17,100 days in the hospital during 2020, which was fewer than in the previous year. The report suggests that this decrease in hospital stays might be linked to the COVID-19 pandemic and the fact that some people started treatment with the therapy Trikafta (elexacaftor/tezacaftor/ivacaftor).

In fact, according to an accompanying trend report, increased access to Trikafta, a triple-combination CFTR modulator therapy developed by Vertex Pharmaceuticals, was one of the trends observed in the 2020 data.

Although available only through clinical trials or special access programs in 2020, 28 children and 169 adults were documented to be on Trikafta that year. Since Trikafta was introduced part-way through the year, it is too soon to tell how it is affecting patients, CF Canada noted.

Trikafta since has been approved in Canada and is publicly funded for anyone who meets the eligibility criteria. It is likely that future reports will show a significant increase in Trikafta use among CF patients.

“For many Canadians with CF, Trikafta is transformative, and every [eligible] patient … should be given the opportunity to find out if it works for them,” John Wallenburg, PhD, chief scientific officer at CF Canada, said in the trend report.

Another trend revealed that the body weight of CF patients has changed significantly over the past 25 years. While the number of underweight CF patients has decreased markedly, the number of individuals who are overweight or obese has increased. These findings could be related to the use of some treatments, such as Trikafta, which are associated with weight gain.

An increase in CF-related diabetes, which is sometimes associated with weight loss, also was observed in 2020, occurring in 34% of the adult patients.

A decrease in lung transplants during 2020 was reported. During the year, 21 CF patients received a lung transplant, compared to 59 in 2018, and 53 in 2019.

CF Canada noted that the COVID-19 pandemic likely played a role in this reduction because organ donations decreased significantly and transplant programs were suspended.

Access to Trikafta also may have contributed to the decline. Several people who were on the transplant list were removed after beginning Trikafta treatment. Broader access to Trikafta in coming years may further decrease the need for lung transplants in the CF community.

“The decrease in lung transplants for people who were on modulator therapies is an early sign of hope for our community. For many, a diagnosis of cystic fibrosis would almost certainly mean a lung transplant in the future, but this is changing,” Grover said in the trend report.

Overall, the COVID-19 pandemic did not appear to negatively influence health outcomes in Canadian CF patients.

“We are relieved to see in the data, that the COVID-19 pandemic doesn’t appear to have had significant immediate effects on health outcomes, including the estimated median age of survival for a Canadian born with cystic fibrosis,” Wallenburg said. “Still, the pandemic had an impact on the CF community as we saw the rise of virtual care and monitored a decrease in hospitalizations and lung transplants.”

The Canadian Cystic Fibrosis Registry includes data from participating individuals at 42 accredited CF clinics throughout Canada. A similar annual report is generated each year.

“We deeply value the information provided by the Canadian Cystic Fibrosis Registry and know how fortunate we are to have it. The Registry has been instrumental for Canadian cystic fibrosis research and care and has also been a tremendous tool for our advocacy efforts,” Grover said.

The full annual report, which includes a comprehensive analysis of all health-related outcomes in Canadian CF patients during 2020, is available here.