Cystic Fibrosis
What is Cystic Fibrosis?
Cystic fibrosis (CF) is an inherited condition. This condition affects different parts of the body, particularly the lungs and digestive system.In a healthy person, there is a constant flow of mucus over the surfaces of the air passages in the lungs. This removes debris and bacteria. Cystic fibrosis occurs when a defective gene causes the body to produce abnormally thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. CF is the most common inherited disease in white people, affecting about 1 in every 2,500 children born.
It is much rarer in people of African or Asian descent. About one in five babies with CF are diagnosed at birth. People with CF are at risk of bacterial chest infections. About half of people with CF have repeated chest infections and pneumonia. . Symptoms may vary from person to person because there are more than 1,000 mutations of the CF gene.
Some common symptoms include very salty-tasting skin, persistent coughing, at times with phlegm; wheezing or shortness of breath; an excessive appetite but poor weight gain; and greasy, bulky stools or constipation, abdominal pain. Some other problems associated with CF can include: small growths in the nose, increased roundness of finger and toe nails, an enlarged liver and spleen, diabetes, infertility in men, fertility problems in women.
Cystic Fibrosis Symptoms
One of the first signs of cystic fibrosis is an excessively salty taste to the skin. People with cystic fibrosis tend to have higher than normal amounts of salt in their sweat. Parents often can taste the salt when they kiss their child. Most of the other signs and symptoms of cystic fibrosis affect the respiratory system or the digestive system.Respiratory signs and symptoms
The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause:
- Persistent cough
- Wheezing
- Repeated lung infections
- Repeated sinus infections
The thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these digestive enzymes, your intestines can't fully absorb the nutrients in the food you eat. This can cause:
- Foul-smelling, greasy stools
- Poor weight gain and growth
- Distended abdomen from constipation
- Intestinal blockage, particularly in newborns
