Cystic fibrosis (CF) is a genetically caused, chronic, progressive disease of the body’s mucous glands. It affects many systems in the body but primarily interferes with the normal functioning of the respiratory and digestive systems. The thick mucus characteristic of CF accumulates in the intestines and lungs and can lead to malnutrition, poor growth, chronic respiratory infections, and breathing problems. Patients can also develop many other medical concerns. The course of the disease varies greatly from patient to patient.
What Are the Symptoms?
Most CF problems are caused by the production of mucus that is thick and sticky, rather than watery. Symptoms include frequent coughing that brings up thick sputum, or phlegm, frequent bouts of bronchitis, and pneumonia, which can lead to inflammation and permanent lung damage. Other symptoms include salty-tasting skin, dehydration, infertility (mostly in men); chronic diarrhea or bulky, foul-smelling, and greasy stools; a significant appetite but poor weight gain and growth (this is called “failure to thrive” and is due to the chronic malnutrition that results from an inability to absorb enough nutrients from food); stomach pain and discomfort caused by too much gas in the intestines. CF is also associated with the following medical problems:- Sinusitis. Swollen sinuses that become blocked with mucus can become infected.
- Bronchiectasis. Here, the bronchial tubes that carry air to the lungs become stretched out and weak, forming pockets where mucus collects and provides a breeding ground for bacteria, which can cause repeated lung infections. Untreated, bronchiectasis can lead to serious illness, including respiratory failure.
- Pancreatitis, a painful inflammation of the pancreas.
- Episodes of intestinal blockage, especially in newborns.
- Nasal polyps, which may require surgery.
- Clubbing—that is, widening and rounding of the tips of the fingers and toes that develops because the lungs are not moving enough oxygen into the bloodstream.
- Collapsed lung, also called pneumothorax.
- Rectal prolapse. As a result of frequent coughing or problems passing stools, rectal tissue can protrude through the anus.
- Liver disease due to inflammation or blocked bile ducts.
- Diabetes.
- Gallstones.
- Low bone density due to vitamin D deficiency.
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