What Are the Signs and Symptoms of Cystic Fibrosis?

The symptoms of CF vary from person to person and over time. Sometimes you will have few symptoms. Other times, your symptoms may become more severe.

One of the first signs of cystic fibrosis (CF) that parents may notice is that their baby's skin tastes salty when kissed or the baby doesn't pass stool when first born.

Most of the other signs and symptoms of CF develop later. They are related to how CF affects the respiratory, digestive, or reproductive systems of the body.

Cystic Fibrosis

Figure A shows the organs that cystic fibrosis can affect. Figure B shows a cross-section of a normal airway. Figure C shows an airway affected by cystic fibrosis. The widened airway is blocked by thick, sticky mucus containing blood and bacteria.

Respiratory System Signs and Symptoms

People who have CF have thick, sticky mucus that builds up in their airways. This buildup of mucus makes it easier for bacteria to grow and cause infections. Infections can block the airways and cause frequent coughing that brings up thick sputum (spit) or mucus that's sometimes bloody.

People who have CF tend to have lung infections caused by unusual germs that don't respond to standard antibiotics. For example, lung infections due to bacteria called mucoid Pseudomonas are much more common in people who have CF. An infection caused by this bacteria may be a sign of CF.

People who have CF have frequent bouts of sinusitis, an infection of the air-filled spaces behind your eyes, nose, and forehead. Frequent bouts of bronchitis (bron-KI-tis) and pneumonia (nu-MO-ne-ah) also occur. These infections can cause long-term lung damage.

As CF gets worse, you may develop more serious complications, such as pneumothorax (noo-mo-THOR-aks), or collapsed lung; or bronchiectasis (brong-ke-EK-ta-sis).

Some people who have CF also develop nasal polyps (growths in the nose) that may require surgery.

Digestive System Signs and Symptoms

Mucus that blocks tubes, or ducts, in your pancreas and prevents enzymes from reaching your intestines causes most digestive system signs and symptoms.

Without these enzymes, your intestines can't fully absorb fats and proteins. This can cause ongoing diarrhea or bulky, foul-smelling, greasy stools. Intestinal blockage also may occur, especially in newborns. Too much gas or severe constipation in the intestines may cause stomach pain and discomfort.

A hallmark of CF in children is poor weight gain and growth. These children are unable to get enough nutrients from their food due to the lack of enzymes to help absorb fats and proteins.

As CF gets worse, other complications may occur, such as:

• Pancreatitis (PAN-kre-a-TI-tis). This is a condition in which the pancreas become inflamed, which causes pain.
• Rectal prolapse. Frequent coughing or problems passing stools may cause rectal tissue from inside you to move out of your rectum.
• Liver disease due to inflamed or blocked bile ducts.
• Diabetes.
• Gallstones.

Reproductive System Signs and Symptoms

Men who have CF are infertile because they're born without a vas deferens. This is the tube that delivers sperm from the testicle to the penis.

A woman who has CF may have a hard time getting pregnant because of mucus blocking her cervix or other CF complications.

Other Signs, Symptoms, and Complications

Other signs and symptoms of CF are related to an upset of the balance of minerals in your blood.

CF causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. This can cause dehydration (a condition in which your body doesn't have enough fluids), increased heart rate, tiredness, weakness, decreased blood pressure, heat stroke, and, rarely, death.

CF also can cause clubbing and low bone density. Clubbing is the widening and rounding of the tips of your fingers and toes. It develops late in CF because your lungs aren't moving enough oxygen into your bloodstream.

Low bone density also tends to occur late in CF. It can lead to a bone-thinning disorder called osteoporosis.