Cystic fibrosis (CF) can affect a person's quality of life and influence their life expectancy. How long someone with CF can expect to live depends on their age and the stage of their condition.
Before the 1980s, about half of the people with CF did not live into their 20s. However, over the past few decades, life expectancy for people with this condition has improved dramatically.
Thanks to advances in treatment and care, people with CF can now expect to live much longer. In fact, recent research suggests that by 2025, the number of adults living with CF will increase by approximately 75%.
Several factors — including sex, lifestyle choices, any infections, and the type of CF gene mutation that a person has — can influence life expectancy.
Some research has reported that people with CF find information on life expectancy to be useful. It may especially help with formulating a healthcare plan and dealing emotionally with the condition.
In this article, we look at average life expectancies for people with CF based on their age and other factors.
The Cystic Fibrosis Foundation Patient Registry tracks people with CF who are receiving care at specialist centers across the United States.
The Patient Registry Annual Data Report publishes the median predicted survival age of people with CF based on their year of birth. The median predicted survival age is an internationally accepted way to estimate life expectancy.
Unlike a mean average, the median uses the midpoint in a set of numbers. It more accurately reflects the age that a person with CF can expect to reach.
Based on the 2017 statistics, the median predicted survival ages are:
|Year of birth||Median predicted survival age|
The data also indicate that half of all babies born with CF in 2017 will live to be 46 or older.
Other statistics suggest that more than 50% of babies with CF born in 2018 and 50% of people with CF aged 30 or older in 2018 will likely reach at least their fifth decade of life.
It is important to note that small changes in the CF population can have significant effects on the calculations. The current figures for life expectancy at birth are estimates that can change from one year to the next.
Also, these predictions do not take into account the potential for improvements in care and treatment that may occur as people age.
It is also important to note that these figures are just averages. Some people will live longer. In fact, some people with CF are living into their 70s.
These statistics reflect current life expectancy rates in developed countries, such as the U.S. and the United Kingdom.
However, Cystic Fibrosis Worldwide suggest that in developing countries, such as El Salvador and India, life expectancy for people with CF is under 15 years of age.
When discussing life expectancy, it is also important to consider a person's quality of life. How an individual views their quality of life depends on a number of factors, including their age and general health status.
Many people with CF develop health complications as they age. Some of these can contribute to reduced quality of life and early death.
Potential complications include:
- bile duct or intestinal obstructions
- bronchiectasis, a condition that causes airway damage
- chronic infections, including bronchitis and pneumonia
- infertility, particularly in males
- nutritional deficiencies
- osteoporosis, a type of bone condition
- pneumothorax, which involves air collecting in the space between the lungs and the chest wall
- respiratory failure
Despite the possibility of these complications, some research suggests that the perception of quality of life improves as people with CF get older. The study relied on self-reported data from more than 300 adults with CF.
More specifically, the findings suggest that as people with CF age, they perceive their treatment burden differently and their "emotional functioning" increases.
This differs from person to person, however. For example, people with better lung function consistently rate their quality of life as being higher, compared with the ratings of people with poorer lung function.
In the later stages of CF, complications often cause serious problems for people. These complications typically affect the lungs, but they may also affect the:
- endocrine (hormone) system
The leading causes of death among people with CF are respiratory failure and chronic progressive pulmonary disease.
Other conditions, such as acute pancreatitis, can lead to fatal heart, lung, or kidney issues.
Several factors can influence a person's quality of life and life expectancy. These include:
As people get older, there is an increased risk of complications, some of which can be fatal.
However, other factors may have a greater influence on life expectancy, which is why some people with CF live into their 70s.
Women with CF have a poorer outlook than men with the condition. Some research suggests that this is due to the increased risk of death in women with CF-related diabetes.
Life expectancy for people with CF largely depends on the complications that they develop. Conditions associated with a lower survival rate include:
- certain infections, including Staphylococcus aureus
- diabetes, in women
- being female
- low body mass index, or BMI
- pancreatic insufficiency
- low lung function
- frequent pulmonary exacerbations, or episodes during which lung symptoms get worse
To date, researchers have discovered over 1,700 mutations in the CFTR gene that can give rise to CF. Some mutations cause more severe symptoms than others, and some may be associated with earlier death.
There are many treatment options available for CF. Often, people receive a combination of treatments, depending on their symptoms and complications.
For example, people with severe lung problems may require a lung transplant. Although this does not cure CF, receiving a transplant may improve survival and quality of life. The 5 year survival rate after a transplant is approximately 50%.
Also, the availability of newborn screening tests in all 50 states means that most people in the U.S. can receive a diagnosis and treatment at the earliest stage. In general, the earlier people begin treatment, the better their outlook will be.
Some other factors can play a role in life expectancy rates. For example, exposure to cigarette smoke or a low socioeconomic status may increase the risk of earlier death.
Although there is no cure for CF, people with the condition are now living longer than ever. Their self-reported quality of life is also much better than in previous decades.
Researchers continue to investigate the genetic mutations that cause CF and work toward potential treatments and cures.
It is important for people with CF to try to remain optimistic about the future, as scientists continue to make advances.