What Is Cystic Fibrosis?
In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.
Symptoms of Cystic Fibrosis
People with CF can have a variety of symptoms, including:
Testing for Cystic Fibrosis (link coming soon)
How can you know for sure if you or your child has cystic fibrosis? If someone in your family has the disease, could you pass it down to your children? There are several ways to help answer these important questions.
While newborn screening is not a definitive diagnostic test for cystic fibrosis, it may lead to tests that can rule out or confirm a CF diagnosis. All states in the U.S. screen newborns for cystic fibrosis.
If a person shows symptoms of CF or if a baby has a positive newborn screen for CF, a doctor may order a sweat test. This simple, painless test is the best way to diagnose CF. It measures the concentration of salt in a person’s sweat. A high salt level indicates CF.
The second part of the test consists of cleaning the area and collecting the sweat on a piece of filter paper or gauze or in a plastic coil. Thirty minutes later, the collected sweat is sent to a hospital laboratory to measure how much chloride is in the sweat.
Clearing the Airways
Some airway clearance techniques require help from family members, friends or therapists. Adults with cystic fibrosis can do many airway clearance techniques themselves.
One technique is called “postural drainage and percussion.” People with cystic fibrosis sit, stand or lie in a position that will help free up mucus as their chest and back are pounded or clapped. Sometimes a patient will use a mechanical “vest,” or blow into a device that shakes the mucus loose, to help clear their airways.
There are several kinds of inhaled medications used to treat CF symptoms:
Antibiotics are used to fight infection-causing bacteria. Infections are common in the lungs of people with cystic fibrosis, so antibiotics are an important part of regular care. The antibiotic drug, the dosage, and the length of time to take the drug, all vary from person to person. The infection-causing bacteria can become resistant to some drugs. Antibiotics come in three different forms:
- Intravenous (IV) antibiotics – liquid medicine that goes directly into the blood through an IV catheter. An IV may require a hospital stay, but can also be done at home.
- Inhaled antibiotics – an aerosol or mist that can reach the airways directly.
Research supported by the Cystic Fibrosis Foundation has led to other respiratory treatments that are now considered “standard therapies” for many people battling cystic fibrosis.
Azithromycin, a common antibiotic, was shown to have special benefits for some people with cystic fibrosis. To study this possibility, the Foundation conducted a Phase 3 trial and found that, in some cases, the drug helped to preserve and improve lung function. The drug also reduced the number of hospital stays for many people who participated in the clinical trials.
Nutrition and Eating Right
Nutrition needs change with age—especially for people with cystic fibrosis. Dietitians at cystic fibrosis care centers offer specially tailored dietary programs for each stage of life.
Children and teens with cystic fibrosis need extra calories to grow and develop. Everyone with CF, no matter their age, need good nutrition to stay strong against lung infections and other challenges. Occasionally, patients need to take nutrition through a tube ("tube-feeding") to provide extra calories that help the body grow and stay strong.