Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body. Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery.
Antibiotics to treat and prevent lung infections. Anti-inflammatory medications to lessen swelling in the airways in your lungs. Mucus-thinning drugs, such as hypertonic saline, to help you cough up the mucus, which can improve lung function.
Causes. Cystic fibrosis is an inherited disease caused by mutations in a genes called the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The CFTR gene provides instructions for the CFTR protein.
Since CF is a genetic disease, the only way to prevent or cure it would be with gene therapy at an early age. Ideally, gene therapy could repair or replace the defective gene.
There's no cure for cystic fibrosis, but a range of treatments can help control the symptoms, prevent or reduce complications, and make the condition easier to live with.
Today, the average life span for people with CF who live to adulthood is about 44 years. Death is most often caused by lung complications.
Cystic fibrosis is a genetic disease. People with CF have inherited. two copies of the defective CF gene. Each chromosome carries hundreds of genes.
CF causes thick mucus that clogs certain organs, such as the lungs, pancreas, and intestines. This may cause malnutrition, poor growth, frequent respiratory infections, breathing problems, and chronic lung disease.
Most people are diagnosed with CF at birth with newborn screening, or before 2 years of age. A doctor who sees the symptoms of CF will order a sweat test or a genetic test to confirm the diagnosis. A sweat test is the most common test used to diagnose CF.
How do I know if I am a carrier of cystic fibrosis? Carrier testing is available through a simple blood test. There are over 1,000 mutations that have been found to cause CF. Carrier screening can be done for the most common of these, and will identify about 85 to 90 percent of carriers in the Caucasian population.
What Are the Symptoms of Cystic Fibrosis?
Oct 23, 2020
Cystic fibrosis is a common genetic disease within the white population in the United States. The disease occurs in 1 in 2,500 to 3,500 white newborns. Cystic fibrosis is less common in other ethnic groups, affecting about 1 in 17,000 African Americans and 1 in 31,000 Asian Americans.
Males account for slightly more than 50 percent of all cases of cystic fibrosis (CF) but generally have better outcomes than females until about age 20.
While advancements in research have vastly improved the quality of life and life expectancy of people with CF, most will need to treat the condition for their entire lives. Currently, there's no cure for CF, but researchers are working toward one.
Ireland not only has the highest incidence of cystic fibrosis in the world, but also the largest proportion of families with more than one child suffering from condition.
What kind of limitations does someone with CF have? People with CF can live very full, normal lives. There are no limitations to their exercise, diet, or activities. However, due to the different lung infections that they can get, they should not meet or talk with other patients with CF in-person.
What is CF belly? A large percentage of CF people have insufficient pancreatic enzymes because the pancreas is inflamed and blocked just like the lungs. Many patients are prone to late gastric emptying, GERD, SIBO, DIOS, and slow gut transit. These conditions can mask each other. This just piles onto the poop problem.
Cystic fibrosis tends to get worse over time and can be fatal if it leads to a serious infection or the lungs stop working properly. But people with cystic fibrosis are now living for longer because of advancements in treatment. Currently, about half of people with cystic fibrosis will live past the age of 40.
At 86, Marlene Pryson may be one of the oldest individuals living with cystic fibrosis. During her long life, she has dedicated many years of service to helping CF families as a CF clinic coordinator and family liaison.