#NACFC2022 – By 2040, Over 70% of CF Patients in US Will Be Adults

Advances in treatment has led to longer survival and a decline in CF births

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

Share this article:

Share article via email
An illustration shows people in virtual meeting for NACFC.

With continual advances in treatment, more people with cystic fibrosis (CF) are surviving well into adulthood and by 2040, nearly three-quarters of these patients in the U.S. will be adults.

That’s according to a study, “Cystic Fibrosis in the United States by 2040: a population landscape analysis,” presented by Elizabeth Cromwell, PhD, of the Cystic Fibrosis Foundation, at the 2022 North American Cystic Fibrosis Conference, in Philadelphia.

In the early 1990s, the expected lifespan for someone born with CF was around 30 years. Now, it’s closer to 50.

Recommended Reading
The words

High Blood-sugar Levels at Night Affect Lung Function in CF Adults

57% of today’s CF patients are adults

Today, more than half (57%) of people living with CF are 18 or older. Understanding how the demographics of the CF population are expected to change in the coming decades may aid in planning patient care.

Researchers at the CF Foundation used a simulation to predict how the size, age, and other factors of the CF population is likely to shift by the year 2040. The simulation was constructed using data from the foundation’s patient registry.

A major assumption that influences results is the expected high lung function of children younger than 6 years, Cromwell noted. In addition, the model does not directly account for how non-pulmonary complications of CF affect survival if not mediated by lung function. Also unknown is whether CF incidence follows U.S. fertility forecasts.

Results showed that, by 2040, more than 70% of the CF population will be adults, and more than one-third of these adults will have minimal problems with lung function — specifically a score of 90% or higher on percent predicted forced expiratory volume in one second (FEV1pp).

Recommended Reading
An illustration of a presenter at a conference.

Most Adults in CF Study Have Low Bone Mineral Density

The simulation also predicted that nearly 80% of children with CF will have a score of 90% or higher on FEV1pp.

“Although CF has historically been characterized as a pediatric disease, we predict continued growth of the adult CF population over the next 20 years because of longer survival and a decline in CF births,” the researchers wrote.

Cromwell said: “Changing distribution in US births may have implications for NBS [newborn screening] programs.” She also noted that the future incidence of lung transplants is challenging to forecast.

“It is likely that this growing, aging adult CF population will be more medically complex because of [non-lung] and age-related comorbidities, so coordination of care between CF care teams, subspecialists, and transplant centers will remain critical,” the team added. “The need to expand the workforce knowledgeable in the nuances of multidisciplinary adult CF care is more important than ever.”

Note: The Cystic Fibrosis News Today team is providing in-depth coverage of the 2022 North American Cystic Fibrosis Conference (NACFC) Nov. 3–5. Go here to see the latest stories from the conference.