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Cystic Fibrosis

Cystic Fibrosis

Cystic fibrosis is a serious and life-threatening disorder that is usually passed down through families. As a genetic disorder, it is common for children of parents with cystic fibrosis to also get the disease. It is also the most common chronic lung disease in children, teens and young adults. Cystic fibrosis consists of a sticky and thick mucus that builds up in the digestive tract, lungs, and other parts of the body.

Causes of Cystic Fibrosis

Cystic Fibrosis is most commonly caused by a defective gene, therefore can only be passed down through blood relatives. This defective gene causes the body to produce an abnormally sticky and thick fluid, which is also called mucus. When it builds up too much, it can fill the lungs, pancreas and breathing passages. It keeps your body from breaking down and absorbing food properly as well as blocking airways. It is considered a life-threatening disease that causes severe digestion problems and can also affect a man’s reproductive system and fertility. Many people carry the cystic fibrosis gene but don’t have symptoms of the disease. This is because they don’t have both defective genes to cause side effects and symptoms of the disease.

Symptoms of Cystic Fibrosis

The symptoms of cystic fibrosis will be different depending on the person and their age. In infants, common symptoms include the failure to gain weight at a normal pace, delayed growth, no bowel movements in the first 24-48 hours following birth and salty-tasting skin. Additional symptoms of cystic fibrosis for children and adults include increased gas, abdominal pain from constipation, pale or clay-colored stools, foul smelling stools, weight loss, nausea, loss of appetite, coughing and increased mucus in the lungs, fatigue, nasal congestion, fever, shortness of breath, sinus pain, respiratory problems, repeated pancreatitis, and infertility in men.

Diagnosing Cystic Fibrosis

The first done typically done to check for cystic fibrosis is a blood test. This will look for the cystic fibrosis gene that usually causes the disease. Additional tests used for diagnostic purposes are the sweat chloride test, immunoreactive trypsinogen, CT scan, chest x-ray, fecal fat test, lung function test, secretin stimulation test, upper GI, small bowel series, trypsin and chymotrypsin in stool, or measurement of pancreatic function.

Treating Cystic Fibrosis

A variety of treatments are available for cystic fibrosis including inhaled medicines to open the airways, antibiotics for sinus or lung infections, Dnase enzyme therapy, hypertonic saline, oxygen therapy, flu vaccine, and a lung transplant in the most severe cases. There are additional treatments for nutritional or bowel problems such as changing your diet to one that is high in protein and calories, pancreatic enzymes, vitamin supplements including vitamins A, E, K and D, and other lifestyle changes.
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