More than 30,000 children and adults in the United States have CF (70,000 worldwide).
People with CF
inherit two copies of a defective gene -- one copy from each parent -- that produces a faulty protein, causing a buildup of thick mucus in the lungs,
pancreas, and other organs.
When mucus clogs the lungs, it can become very difficult for a person to breathe. The thick mucus also allows for
germs to thrive and multiply, which can result in infections and inflammation and often leads to severe lung damage and respiratory failure.
In the pancreas, the buildup of mucus prevents the release of digestive
enzymes that help the body break down food and absorb important
nutrients. People with CF often have malnutrition and poor growth.
The thick mucus can also block the bile duct in the liver and, in some people with CF, can cause liver disease.
In men, CF can affect their ability to have children. However, CF does not affect
sexual development in either men or women.
Babies with CF grow, develop, and do what other babies do. Children with CF go to
school, play sports, and get their driver's licenses. People with CF can go to
college, pursue successful careers, and
have families of their own.
Today, because of improved medical treatments and care, more than half of people with CF are age 18 or older. Many people with CF can expect to live healthy, fulfilling lives into their 30s, 40s, and beyond.
Read the Foundation's Patient Registry Reports.
Watch CF clinicians discuss:
- What CF is
- How CF is diagnosed
- Newborn screening
The most common symptoms are:
- Very salty-tasting skin
- Persistent coughing, at times with phlegm
- Frequent lung infections, such as pneumonia or bronchitis
- Wheezing or shortness of breath
- Poor growth or poor weight gain in spite of a good appetite
- Frequent greasy, bulky stools or difficulty in bowel movements
- Nasal polyps
- Chronic sinus infections
- Clubbing or enlargement of the fingertips and toes
-
Rectal prolapse
-
Male infertility
Learn more about CF -- from diagnosis to living with the disease as an adult -- in "An Introduction to Cystic Fibrosis: For Patients and Their Families," or watch the video series.
A complete diagnostic evaluation for CF should include a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
Most children are now screened for CF at birth through newborn screening and the majority are diagnosed by age 2. However, some people with CF are diagnosed as adults. A doctor who sees the symptoms of CF will order a sweat test and a genetic test to confirm the diagnosis.
Most people with CF are diagnosed quickly, although in some cases the diagnosis is not clear and subsequent tests may be recommended.
“I grew up wondering why I felt sick every day. As doctors suggested unlikely diseases, such as hormonal disorders, kidney disease, lupus, and depression, I felt I was further from an answer. Then, my ENT suggested CF, a disease I had never heard of. As he described what he knew about CF, it matched all of my symptoms and promised the answer I had been looking for my whole life.” -- Katie K., an adult with CF, from the CF Community Blog
Read the Foundation's guidelines for diagnosing CF.
To stay healthy, people of all ages with CF must follow a regular treatment routine. In addition to a nutrition and fitness plan, there are medications that help clear the thick, sticky mucus from airways and reduce inflammation, antibiotics to treat CF lung infections, and emerging therapies that target the defective cystic fibrosis transmembrane conductance regulator (CFTR) protein in CF to improve different symptoms of the disease.
Watch a brief video on how clinical research has made a difference in the lives of people with CF.
People with CF do best when care is comprehensive, coordinated, and done in partnership with their CF care teams. The CF Foundation funds and accredits more than 130 care centers around the country to ensure people with CF can receive high-quality, specialized care.
The health of people with CF continues to improve because of this approach. Data from the Patient Registry show steady progress in key measures associated with survival, such as improved lung function and nutritional status and decreased presence of harmful lung bacteria.
Key to the progress in the health and well-being of people with CF are the highly specialized, multidisciplinary teams of health care professionals at each center who work closely with people with CF and their families to customize care based on each individual's unique needs.
“After my son cultured positive for an infection, all I could think about was a possible hospital admission, if this was the start of his decline, and everything I knew my son was afraid to miss out on. When the results came back with good news, my son expressed his relief, to which our doctor replied, 'Me too.' Those words conveyed the feeling that we were partners who ultimately want the same thing: good news and good health." -- Mother of a teenager with CF