.png)
.png){kind=icon}
What is
Cyctic Fibirosis?
Learn the facts
Cystic fibrosis is a chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product causes the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. The mucus also obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.
Frequently Asked Questions
Is cystic fibrosis fatal?
Currently, there is no cure for cystic fibrosis. However, specialized medical care, aggressive drug treatments, and therapies, along with proper CF nutrition, can lengthen and improve the quality of life for those with CF.
What is the life expectancy for people who have CF?
It is not possible to accurately predict how long a person who has CF will live. Many different factors — for example, severity of disease and age at diagnosis — can affect an individual’s health and the course of the disease. Recent research has shown that the severity of CF symptoms is based partly on the type of CF gene mutations a person has.
The CF Foundation Patient Registry collects information on the health of the more than 27,000 people treated at CF Foundation-accredited care centers. According to the most recent Patient Registry data, the median predicted age of survival for people with CF is in the early 40s. Median predicted age of survival is the age by which half of the people tracked in the Patient Registry would be expected to survive, given the ages of the patients in the Registry and the distribution of deaths in a particular year.
In the 1950s, children with CF were not expected to live long enough to attend elementary school. Today, approximately half of all people with CF in the United States are 18 years or older. The steady “aging” of the CF population reflects the remarkable progress that has been made in understanding and treating CF. Thanks to CF Foundation-supported research and care, an increasing number of people with CF are living well into adulthood and leading healthy lives, pursuing careers, getting married and having children of their own.
What is a typical day for someone with CF?
Because the severity of CF differs widely from person to person, and CF lung infections flare up from time to time, there is no “typical” day. However, each day most people with CF:
Take pancreatic enzyme supplement capsules with every meal and most snacks. Do some form of airway clearance at least once and sometimes up to four or more times a day. Take aerosolized medicines—liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer.
What treatments or therapies are available?
Since CF affects the lungs of most patients, a large part of the medical treatment is to clear mucus from the airways by using different airway clearance techniques. These techniques use vibrations to help loosen the mucus in the lungs so it can be coughed out. There are several medications that treat lung infections and can help people with cystic fibrosis breathe better.
Is gene therapy a treatment for people with CF?
When scientists found the most common gene that causes CF in 1989, there was much excitement about the possibility of developing gene therapy. Scientists are currently exploring the use of gene therapy for many diseases but have had little success. That is because it has been very hard to find a safe and reliable way to deliver healthy genes into the cells and tissues of the body.
When will there be a cure?
Because CF researchers are blazing new trails in drug development and gene therapy, experts have no way of saying for sure when a cure will be available. The “aging” of the cystic fibrosis community is largely due to the increase in innovative new treatments and specialized medical care. But a better quality of life and partially increased length of life are simply not enough. That is why the CF Foundation’s efforts to expand and strengthen the drug development pipeline of potentially life-saving new therapies while, at the same time, supporting a vital care center network.