Evolution of Cystic Fibrosis Life Expectancy

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A diagnosis of cystic fibrosis has greatly changed in the past three decades. Children who are born with the disease today have a completely different prognosis from those born 30 years ago. While the diagnosis was possible through a sweat test, there were no methods of treatment and the mucus would rapidly accumulate in the lungs and digestive tracts of CF patients. There were also no effective antibiotics for the treatment of infections, and the digestive and pancreatic manifestations of the disease frequently resulted in delayed growth and malnutrition.

“In 1962, the predicted median survival for CF patients was about 10 years, with few surviving into their teenage years,” according to the National Institutes of Health regarding past CF treatment and life expectancy. “NIH researchers discovered that the underlying problem in patients with CF was a defect in salt transport through their cell membranes. The CF transmembrane conductance regulator (CFTR) gene, which is defective in people with CF, was discovered in 1989. Carrier detection and prenatal diagnosis of CF were developed for families with a history of the disease. Mouse models of the disease were developed in the early 1990s, but unlike humans, mice without CFTR did not exhibit typical symptoms of CF, including the lung and liver disease that cause the premature mortality seen in humans.”

Research achievements have resulted in the quadruplication of median life expectancy in the U. S., but in countries where medicine is not so advanced life expectancy remains much lower. In all U.S. states, newborn screening for CF is performed even when there are no symptoms. In addition, there are already antibiotics able to fight infections, other medications designed to slow the progression of lung disease, and new mechanical chest physical therapy devices that loosen the mucus and make it easier for patients to clear their lungs. Lung transplant has also become an alternative for the treatment of CF patients with severe lung damage.

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Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

5 comments

  1. Deb Haupert says:

    FAMILY HISTORY NEED NOT BE PRESENT TO HAVE A CHILD WITH CYSTIC FIBROIS OR A CHILD WHO IS A CF CARRIER. ALL WOMEN SHOULD BE TESTED PRECONCEPTUALLY. NEWBORN SCREENING DOES NOT DIGANOSIS CF NOR DOES IT IDENTIFY ALL CARRIERS.

    • Eva Z. says:

      I totally agree. The Newborn Screening is TOO LITTLE TOO LATE PROGRAM !!!!!. The Insurance Companies would save everbody a lot of money, heartache and would prevent this horrible disability by making EVERY PREGNANT WOMAN TO TEST FOR CF !!!!! We didn’t have ANY CF HISTORY in our families. ..we had no idea our child was going to be born with CF …I love her to death but if I knew about her disease while I was pregnant I WOULD NEVER LET HER TO BE BORN. I tried to talk to some politicians to change those rules…Unfortunately they are too stupid to understand. ..wait till their loved one will be born with CF…hopefully this will change. .. (??)

  2. linda sanders says:

    The bible says life begins at conception. My neice had c.f. I can’t begin to imagine what life would been without her.

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