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Cystic fibrosis (CF)
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Cystic fibrosis (CF)

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Cystic fibrosis (CF) - a life-threatening disorder that causes severe lung damage and nutritional deficiencies - used to be a genetic mystery, and most people with the disease didn't live beyond their teens. But researchers have made progress in unraveling the genetic basis of CF, which has led to earlier detection. In addition, improved and more consistent treatments now allow people with CF to live into their 30s and 40s and even beyond, and to have fuller, more comfortable lives.

Complications 

The most frequent complications of CF are chronic respiratory infections, including pneumonia, bronchitis and bronchiectasis — an abnormal dilation of the walls of the bronchial tubes that makes it more difficult to clear your airways. Asthma can result from chronic inflammation of the bronchial lining.

Respiratory infections are common because thick mucus blocks the airways and provides a perfect breeding ground for bacteria. The most common infective agent in people with CF is Pseudomonas aeruginosa — a bacterium that can cause increased inflammation of the respiratory tract. Although antibiotics can decrease the frequency and severity of attacks, the bacteria are never completely eradicated from the airways and the lungs. On the other hand, P. aeruginosa rarely causes pulmonary infections in healthy people and isn't considered contagious.

People with CF may also develop bleeding from the lungs (hemoptysis), respiratory failure or collapsed lung (pneumothorax) — a condition in which lung air leaks into the chest cavity through a small hole that forms in the lung's outer layer. Lung disease eventually may cause the right ventricle of the heart to fail. Ultimately, complications from lung problems may prove fatal for many people with CF.

In addition, cystic fibrosis makes you prone to chronic diarrhea and severe nutritional deficiencies. That's because thick secretions obstruct the ducts in your pancreas, preventing enzymes that digest fats and proteins from reaching your intestines. These secretions also prevent your body from absorbing the fat-soluble vitamins A, D, E and K.

CF affects the pancreas and because the pancreas controls the level of sugar in your blood, about 7 percent of people with CF may develop type 1 diabetes. In addition, the bile duct, the duct that carries bile from your liver and gallbladder to your small intestine, may become blocked and inflamed, leading to liver problems such as cirrhosis.

CF also affects the reproductive system. Because thick secretions often block the tube connecting the testes and prostate gland (vas deferens), many men with CF are infertile. But certain fertility methods and surgical procedures may sometimes make it possible for men with CF to become fathers.

Although women with CF may be less fertile than other women are, it's possible for them to conceive and to have successful pregnancies. Still, pregnancy can exacerbate the symptoms of CF, so be sure to discuss the possible risks with your doctor. Using oral contraceptives also can sometimes aggravate certain symptoms of CF. It's best to talk to your doctor about your birth control options.

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