Understanding Changes in Life Expectancy

People with cystic fibrosis continue to live longer and healthier lives, and the Patient Registry data support this general trend. To understand what this means for our community, however, it is important to understand how these numbers are calculated and what they represent.

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Summary
  • The latest CF Foundation Patient Registry data show steady gains in survival for people with cystic fibrosis.
  • Because CF is a rare disease, however, small changes in the population can have a noticeable impact on survival calculations. 
  • Life expectancy and median age of death can be helpful to track the progress made for the general CF population, the numbers do not predict an individual will live.

CF Foundation Patient Registry

The Registry was created in 1966 to track the health of people with cystic fibrosis who receive care at CF Foundation-accredited care centers and agree to share their data to inform continued quality improvement in treatment and specialized care. Each year, the CF Foundation analyzes these data and shares this information with the CF community through the Patient Registry Annual Data Report.

Based on 2019 Registry data, the life expectancy of people with CF who are born between 2015 and 2019 is predicted to be 46 years. Data also show that of the babies who are born in 2019, half are predicted to live to be 48 years or older.

Graph showing survival statistics from 2015 through 2019
Survival statistics for the years 2015 through 2019.

To understand what this means for our community, it is important to understand how these numbers are calculated and what they represent. 

Median predicted age of survival refers to a median (the mid-point of a number set), while life expectancy refers to an average -- but the terms are often used interchangeably. It is a calculation that looks at the CF patient population, as captured in the Registry, at each age in a single year. This methodology is an internationally accepted calculation for determining life expectancy.

How Long Are People With CF Living Today?

The latest CF Foundation Patient Registry data show steady gains in survival for people with CF. Because CF is a rare disease, however, small changes in the population can have a noticeable impact on survival calculations. Statisticians and clinicians caution against putting too much emphasis on data collected in a single year, which is why it is important to keep in mind that life expectancy at birth is a calculation that can fluctuate from year to year and may decrease in the future.

Data also show that of the deaths reported in the Registry in 2019, known as the median age of death, half occurred before the age of 32 -- meaning too many people with CF miss the opportunity to finish school, begin careers, start families, and reach other milestones.

For those living with the disease, many still face significant challenges, including frequent hospitalizations, complications such as CF-related diabetes and depression, and time-consuming treatment plans that can take 2 to 3 hours a day.

Percentage of people with CF 12 years and older who reported depression—24%—or anxiety—21%
Many people with CF report mental health challenges.

So, we still have a long way to go -- particularly for those who have mutations that result in faster and more severe disease progression, who do not yet have disease-modifying treatments approved to address their mutation, or who cannot benefit from modulator therapy due to advanced disease.

What Does the Registry Data Mean for Me?

Although life expectancy and median age of death can be helpful to track the progress made for the general population with CF followed by the Registry, these numbers do not predict how long you, as an individual, will live.

As statistical calculations for the general population of people with CF, neither of these figures take into consideration your personal characteristics, such as your mutation, how severe your disease is, or how you respond to different CF treatments as part of your daily CF care.

Statistics are impersonal. They are about 'people with CF,' but not necessarily me. Linda Bowman, adult with CF

These calculations also don't reflect the potential benefits of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies or the continuous improvements in specialized CF care.

For this reason, you should talk to your CF care team if you have questions specific to your personal health.

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Topics
Care Centers | Patient Registry
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Downloads
2019 Patient Registry Highlights Download (PDF)
2020 Patient Registry Annual Data Report Download (PDF)
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