Cystic fibrosis patients are living much longer: Good news for patients revealed in new study
A new study from Epic Research, a health analytics firm based in Verona, Wisconsin, detected some encouraging news for those who suffer from cystic fibrosis (CF).
In 2008, the average life expectancy among CF patients was 26 years — 65% lower than those without the disease.
As of 2022, the life expectancy is 66 years — or just 12% lower than the non-CF population, the study found.
“This is an 82% reduction of the gap in life expectancy between patients with CF and those without,” the study said.
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“These findings suggest that the advancements in cystic fibrosis treatment and therapies are associated with a substantial increase in life expectancy for patients diagnosed with cystic fibrosis,” the authors wrote in a discussion of the findings.
The reasons for the dramatic improvement include better diagnoses, treatments and comprehensive care programs for cystic fibrosis, said Jackie Gerhart, chief medical officer at Epic Research and a family medicine physician.
“A diagnosis can now be made earlier due to advanced diagnostic testing, and comprehensive care centers have allowed for individualized therapy for complications of cystic fibrosis and for complications of general aging in cystic fibrosis patients,” she told Fox News Digital.
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“There are also new therapeutic options that not only help treat cystic fibrosis, but also treat infections that CF patients are more susceptible to, such as pneumonia,” she said.
In the study, investigators looked at the median age of death between 2008 and 2022 for 3,420 people with cystic fibrosis and 4.8 million people without the disease.
The life expectancy remained “fairly consistent” for the latter group, according to a discussion of the study findings, with a median of 75 years in both 2008 and 2022.
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Cystic fibrosis is a “progressive, genetic disease that affects the lungs, pancreas and other organs,” according to the Cystic Fibrosis Foundation.
People with the disease have a genetic mutation that creates an abnormal protein, which causes a buildup of mucus in various organs.
“New therapies called modulators help improve the symptoms of patients.”
When this mucus accumulates in the lungs, it makes it difficult to breathe and also traps germs that can lead to infections, inflammation and respiratory failure, CFF states on its website.
The disease can also hinder the functioning of the pancreas — leading to malnutrition and stunted growth — and the liver, which can cause liver disease.
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“New therapies called modulators help improve the symptoms of patients with this abnormal protein,” said Gerhart. “The most common cause for deaths in cystic fibrosis patients is respiratory failure, so many therapies are targeted to decrease that.”
With earlier diagnosis and new genetic advances for both diagnosis and treatment, the life expectancy for cystic fibrosis will continue to rise, Gerhart expects.
“There are now multiple genetic screening tests available for newborns to help diagnose cystic fibrosis,” she told Fox News Digital.
“Patients who are diagnosed should consider a comprehensive care program and take precautions against infections, including immunizations when appropriate.”
In the U.S., there are some 40,000 children and adults living with cystic fibrosis.
Common symptoms of the disease include persistent coughing with phlegm, salty-tasting skin, frequent lung and sinus infections, wheezing or shortness of breath, stunted growth, trouble gaining weight, difficulty with bowel movements, the presence of nasal polyps and rectal prolapse, according to CFF.
In men, the disease can cause infertility.
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