What to Know About Mucus in Cystic Fibrosis

As a result of a genetic mutation to the cystic fibrosis transmembrane conductance regulator (CFTR) gene, mucus and other bodily fluids are thicker and stickier in people with CF.

This thickness occurs because the CFTR gene mutation affects the way salt and water move into and out of cells. Changes in the CFTR gene affect cells that produce sweat, mucus, and digestive enzymes.

CF mucus may clog the lungs, pancreas, liver, and intestines.

When CF mucus clogs the lungs, breathing problems and infections may become a concern.

Enzymes necessary for digestion may not reach the gut when the pancreas is affected by thick CF mucus. When the intestines are also affected by CF mucus, it can make it difficult to absorb enough nutrients.

Typically, when mucus covers the tiny hairlike structures called cilia in the lungs, it is swept up to the nose and mouth so it can exit the body. Thicker CF mucus sticks to the cilia, though. This interferes with the typical process and makes it more difficult to clear mucus out of the lungs.

Extra mucus in the lungs can lead to blockages, infections, and damage to structures inside and outside the lungs.

Symptoms of CF can include:

• salty skin
• frequent wheezing and coughing that may include mucus
• nasal polyps
• greasy, foul-smelling stools
• shortness of breath
• chronic lung infections, bronchitis, and pneumonia

There is no cure for CF, but medicines and therapies can be useful in helping to thin the mucus and reduce related complications.

Some of the drugs that may reduce CF mucus and related symptoms include:

• antibiotics to treat or prevent lung infections
• mucus-thinning medications
• nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation in the airways
• bronchodilators to relax muscles and increase airflow
• CFTR modulators

Surgical treatment options may include:

• bowel surgeries to relieve blockages
• placement of a feeding tube
• double lung transplant

To help break up mucus in the chest and make it easier to cough up, you may have chest physical therapy several times a day with or without mechanical devices.

A doctor may also suggest oxygen therapy or ventilator support to help improve the amount of air in your body.

The predicted median survival age for a child born in the United States with CF was 48.4 years in 2019, and this number is expected to continue to climb.

The life expectancy for those with CF has grown in recent years thanks to the development of new drugs and therapy techniques. Additionally, newborn screening and prenatal testing have allowed for earlier interventions and family education.

What are the three major symptoms of cystic fibrosis?

Individuals with CF may have salty skin. They can experience frequent lung infections and cough up mucus. They may also have greasy and foul-smelling stools.

What does cystic fibrosis stool look like?

People with CF may experience constipation or have large, greasy stools. These stools can have a foul smell and may float because of the large amount of gas they contain.

When will you know if your baby has cystic fibrosis?

All babies in the United States are tested for CF as part of their newborn screening. You can also test your developing baby for CF as early as 10 weeks into your pregnancy.

The thick, sticky mucus that builds up in the lungs and intestines of people with CF can make it hard to breathe and properly digest food. You may also experience frequent lung infections if you have CF.

Advances in medical treatments are increasing the life expectancy of people with CF. A combination of medications and therapies may be helpful in thinning out the mucus associated with CF so you can live a full, productive life. It’s important to discuss your treatment plan with your doctor since every individual’s needs are different.