Cystic fibrosis (CF) is a serious genetic disease that causes severe damage to the respiratory and digestive systems. This damage often results from a buildup of mucus in the organs. Medical screenings, like a chest x-ray or CT scan, can help doctors diagnose and monitor cystic fibrosis. Early diagnosis and treatment are critical for improving quality of life and lengthening the expected lifespan.
The most commonly affected organs include the:
The most drastic effects of cystic fibrosis include infections, respiratory failure, and malnutrition. It’s highly recommended to get treatment for cystic fibrosis right away.
Approximately 1,000 people are diagnosed with cystic fibrosis every year in the United States. Although people with the condition require daily care, they can still lead a relatively normal life and work or attend school.
Symptoms of Cystic Fibrosis
The symptoms of cystic fibrosis can vary depending on the person and the severity of the condition. The age at which symptoms develop can also differ.
Some people with CF show symptoms in infancy, but for other children, symptoms may not begin until after puberty or even later in life. As time passes, the symptoms associated with the disease may get better or worse.
One of the first signs of cystic fibrosis is a strong salty taste to the skin. Parents of children with cystic fibrosis have mentioned tasting this saltiness when kissing their children.
Other symptoms of CF result from complications that affect:
- the lungs
- the pancreas
- the liver
- other glandular organs
How to Diagnose Cystic Fibrosis
A complete diagnostic evaluation for CF should include a sweat chloride test, a genetic test 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.
Other diagnostic tests that may be performed include:
- Immunoreactive trypsinogen (IRT) test
The immunoreactive trypsinogen (IRT) test is a standard newborn screening test that checks for abnormal levels of the protein called IRT in the blood.
A high level of IRT may be a sign of cystic fibrosis. However, further testing is required to confirm the diagnosis.
- Sweat chloride test
The sweat chloride test is the most commonly used test for diagnosing cystic fibrosis. It checks for increased levels of salt in the sweat. The test is performed by using a chemical that makes the skin sweat when triggered by a weak electric current.
Sweat is collected on a pad of paper and then analyzed. A diagnosis of cystic fibrosis is made if the sweat is saltier than normal.
- Sputum test
During a sputum test, the doctor takes a sample of mucus. The sample can confirm the presence of a lung infection. It can also show the types of germs that are present and determine which antibiotics work best to treat them.
- Chest X-ray
A chest x-ray is useful in revealing swelling in the lungs due to blockages in the respiratory passageways. Lung CT scans also show this.
A CT scan creates detailed images of the body by using a combination of x-rays taken from many different directions. CF causes the buildup of mucus in the lungs, airways, and nasal passages, which can be visualized on CT scans.
Chest CT scans can reveal both mucus and bronchiectasis (a thickening of the walls of the small compartments of the lungs called the bronchi) that may indicate infection, inflammation, and potential lung damage.
Normally, sinuses are filled with air and appear black in CT scans. In patients with CF, the sinuses can become filled completely with mucus and appear white or grey in a sinus CT.
Although there’s no cure for cystic fibrosis, there are various treatments available that may help relieve symptoms and reduce the risk of complications.
Antibiotics may be prescribed to get rid of a lung infection and to prevent another infection from occurring in the future. They’re usually given as liquids, tablets, or capsules. In more severe cases, injections or infusions of antibiotics can be given intravenously (through a vein).
Mucus-thinning medications make the mucus thinner and less sticky. They also help you to cough up the mucus so it leaves the lungs. This significantly improves lung function.
More drastic options include surgery to remove a blockage in the bowels, inserting a feeding tube to ensure the absorption of nutrients from food, and double lung transplant when medical management alone can no longer maintain lung health and physical function.
These treatments will vary depending on the severity of your CF and if there are any other medical conditions. It is best to talk to your doctor regarding any changes in cystic fibrosis treatment you may want to implement.
The outlook for people with cystic fibrosis has improved dramatically in recent years, largely due to advances in treatment and early detection. Talk to your doctor if you or someone in your care would like to learn more about cystic fibrosis symptoms and treatment.
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