In the 1940s, most babies born with CF died in infancy.
Babies born with CF today can expect to live into their 50s and 60s.
Continuing therapeutic advances are expected to further improve their quality of life and extend their lifespan.
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Even as recently as the 1980s, few people with CF survived to adulthood.
The outlook is even better for babies born with CF today.
Why men with CF tend to survive longer than women is not fully understood.
Estrogen worsens mucus dehydration in the lungs and impairs the immune systems ability to clear common lunginfections likePseudomonas aeruginosa.
Women with CF may also experience higher levels of lung inflammation.
The F508del mutation causes the CFTR protein to fold incorrectly.
These therapies are likely to extend the lifespan of people with those specific mutations.
Infections
Lung infections worsen lung function in CF and contribute to early mortality.
Common respiratory infectionssuch as those caused byPseudomonas aeruginosaandBurkholderia cepaciacan cause severe lung damage in people with CF.
CFTR protein-targeting therapies, sometimes called modulators, help fix flaws in the CFTR protein.
ENaC is overexpressed in CF, and its excess movement of sodium into lung cells worsens mucus dehydration.
Although incredibly promising, these therapies are still in various phases of testing and development.
Frequently Asked Questions
Cystic fibrosis increases susceptibility to lung infections.
Chronic lung infections and inflammation cause a progressive decline in lung function and, eventually, respiratory failure.
Cystic fibrosis also affects the digestive system.
Cystic fibrosis impairs fertility.
Most men with cystic fibrosis are infertile, but can conceive with assisted reproductive technology.
Women with cystic fibrosis can have children, but may have decreased fertility.
Cystic fibrosis is a serious, life-threatening disease that significantly shortens a persons lifespan.
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