Cystic fibrosis (CF)

Cystic fibrosis (CF) is a condition passed down in families that causes damage to the lungs, digestive system and other organs in the body.

CF affects the cells that make mucus, sweat and digestive juices. These fluids, also called secretions, are usually thin and slippery to protect the body's internal tubes and ducts and make them smooth pathways. But in people with CF, a changed gene causes the secretions to become sticky and thick. The secretions plug up pathways, especially in the lungs and pancreas.

CF gets worse over time and needs daily care, but people with CF usually can attend school and work. They often have a better quality of life than people with CF had in past decades. Better screening and treatments mean that people with CF now may live into their mid- to late 50s or longer, and some are being diagnosed later in life.

Symptoms

In the U.S., because of newborn screening, cystic fibrosis can be diagnosed within the first month of life, before symptoms develop. But people born before newborn screening became available may not be diagnosed until the symptoms of CF show up.

CF symptoms vary, depending on which organs are affected and how severe the condition is. Even in the same person, symptoms may worsen or get better at different times. Some people may not have symptoms until their teenage years or adulthood.

People who are not diagnosed until adulthood usually have milder symptoms and are more likely to have symptoms that aren't typical. These may include repeated bouts of an inflamed pancreas called pancreatitis, infertility and repeated bouts of pneumonia.

People with CF have a higher than usual level of salt in their sweat. Parents often can taste the salt when they kiss their children. Most of the other symptoms of CF affect the respiratory system and digestive system.

Respiratory symptoms

In cystic fibrosis, the lungs are most commonly affected. The thick and sticky mucus that happens with CF clogs the tubes that carry air in and out of the lungs. This can cause symptoms such as:

• A cough that won't go away and brings up thick mucus.

• A squeaking sound when breathing called wheezing.

• Limited ability to do physical activity before tiring.

• Repeated lung infections.

• Irritated and swollen nasal passages or a stuffy nose.

• Repeated sinus infections.

Digestive symptoms

The thick mucus caused by cystic fibrosis can block tubes that carry digestive enzymes from the pancreas to the small intestine. Without these digestive enzymes, the intestines can't completely take in and use the nutrients in food. The result is often:

• Foul-smelling, greasy stools.

• Poor weight gain and growth.

• Blocked intestines, which is more likely to happen in newborns.

• Ongoing or severe constipation. Straining often while trying to pass stool can cause part of the rectum to stick out of the anus. This is called a rectal prolapse.

When to see a doctor

If you or your child has symptoms of cystic fibrosis — or if someone in your family has CF — talk with your healthcare professional about testing for the condition. Make an appointment with a doctor who has skills and experience in treating CF.

CF requires regular follow-up with your healthcare professional, at least every three months. Call your healthcare professional if you have new or worsening symptoms, such as more mucus than usual or a change in the mucus color, lack of energy, weight loss, or severe constipation.

Get medical care right away if you're coughing up blood, have chest pain or trouble breathing, or have severe stomach pain and bloating.

Call 911 or your local emergency number or go to the emergency department at a hospital if:

• You're having a hard time catching your breath or talking.

• Your lips or fingernails turn blue or gray.

• Others notice that you're not mentally alert.