CF Survival Better in France Where Transplants Are More Available

Rate of lung transplants higher in France than Canada, Australia, New Zealand

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

Share this article:

Share article via email
cystic fibrosis survival rate | Cystic Fibrosis News Today | illustration of doctors looking at a tablet

The average lifespan for someone with cystic fibrosis (CF) is markedly longer in France than in Canada or Australia, a new study reports.

Programs that help facilitate access to lung transplants in France may in part explain the difference, according to researchers.

“For multiple reasons, which will need to be further explored, CF care results in better prognosis in France as compared with Canada and Australia,” the scientists wrote.

The study, “Beyond borders: cystic fibrosis survival between Australia, Canada, France and New Zealand,” was published in Thorax.

Survival outcomes for people with CF vary from place to place and understanding the reasons for these differences can help inform better policies and care. For example, a study from the 1980s that compared outcomes in Toronto and Boston helped demonstrate that a high-fat diet can improve survival outcomes for CF patients.

Recommended Reading
CF lung transplant | Cystic Fibrosis News Today | image of child and adult

Organ Rejection a Key Difference in Childhood Transplant Survival Rates

Access to healthcare is an important factor in determining clinical outcomes and may be a factor that differences in different places. A recent study found CF survival outcomes were better on average in Canada than the U.S., for example. Differences in insurance likely contributed: Canada has a single-payer healthcare system, whereas the U.S. operates mainly on a private insurance model.

Indeed, the findings suggested outcomes were comparable for Canadian CF patients and for U.S. patients with private insurance, but worse for U.S. residents who were uninsured or on government-funded insurance (Medicare or Medicaid).

In this study, scientists compared clinical and survival outcomes for CF patients in four countries: Australia, Canada, France, and New Zealand. All have a single-payer healthcare system. The scientists hypothesized that there would be “no statistically significant difference in survival between the countries.”

Using national registries, the scientists identified data for nearly 15,000 people with CF: 3,537 in Australia, 4,434 in Canada, 6,411 in France, and 460 in New Zealand. The data were collected between 2015 and 2019 before the availability of Trikafta, a therapy that’s now widely approved, they noted.

Demographic data were generally comparable across the four groups. Only 13.8% of patients in Canada were diagnosed via newborn screening compared to about half of the patients in the other three countries.

The rate of lung transplants was highest in France (16.5%) and lowest in New Zealand (6.7%). In France, but none of the other countries, the majority of deaths occurred after a lung transplant.

“These data suggest that a significant proportion of individuals in need of transplant in France were able to receive this life-saving therapy,” the researchers wrote, noting that a national emergency lung transplant program called HELT, implemented in France in 2007, may account in part for these differences.

“The HELT programme is unique to France and although medical need influences prioritisation on the transplant list in Canada, Australia and New Zealand, the process is less formalised when compared with the system adopted in France,” they wrote.

Despite having the highest proportion of underweight patients (a predictor of worse outcomes in CF), France had the higher median lifespan (65.9 years) compared to Australia (53.3 years), Canada (55.4 years), and New Zealand (54.8 years).

In statistical models adjusted for age at diagnosis, newborn screening, transplant status, measures of lung function, and other clinical variables, the risk of death was roughly twice as high in Canada or Australia compared to France.

“Using contemporary CF data, our study identified a survival gap between people living in high-income countries with similar healthcare systems. France had the highest recorded median age of survival of the four countries, surpassing 60 years in the contemporary time window between 2015 and 2019,” the researchers concluded, noting the better outcomes in France “may be due to differences in availability of transplant for those who need it most,” though other differences may play a role between countries. For example, the research team noted that while all four have single-payer healthcare systems, specifics on access to medications differ by country.

A Conversation With Rare Disease Advocates

Your CF Community

Visit the Cystic Fibrosis News Today forums to connect with others in the CF community.