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Cystic Fibrosis

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Signs and Symptoms

Signs and symptoms associated with cystic fibrosis include:

  • Frequent coughing, chronic cough and sputum production
  • Repeated or persistent chest infections such as bronchitis or pneumonia
  • Recurrent sinus infections and nasal congestion caused by nasal polyps
  • Abdominal pain or discomfort
  • In newborns, lack of stool in the first 24-48 hours after birth (meconium ileus)
  • Chronic diarrhea or bulky, foul-smelling, greasy stools
  • Weight loss or malnutrition
  • In children, failure to gain weight or grow at a normal rate (failure to thrive)
  • Decreased levels of protein in the blood, leading to accumulation of fluid, usually just under the skin (edema)

CF interferes with electrolyte and fluid balance in the body. In most individuals affected with CF, sweat contains up to five times more salt (sodium chloride) than unaffected individuals. Losing excess sodium and chloride can affect the heart rhythm and may sometimes cause shock.

In people with CF, lubricating mucus in the lungs becomes thick and sticky, providing a breeding ground for microbes, causing frequent respiratory infections. These must be aggressively treated with intravenous, oral and/or inhaled antibiotics. Many of the illnesses associated with CF are due to respiratory infections and lung complications.

Mucus plugs in the pancreas lead to blocked ducts, resulting in malabsorption because pancreatic enzymes (needed to digest food, particularly protein and fat, so that it can be absorbed) cannot travel to the intestine. This can lead to vitamin deficiencies and malnutrition. Pancreatic enzyme and vitamin A, D, E and K supplements can ease these symptoms, along with a low-fat, high-protein diet. If pancreatic damage progresses, some people with CF eventually become diabetic.

Other problems associated with CF include:

  • Gallstones
  • Pancreatic insufficiency and pancreatitis
  • Delayed growth and delayed sexual development at puberty
  • Enlargement or rounding (clubbing) of the fingertips and toes
  • Chronic liver disease and biliary cirrhosis
  • Rectal prolapse (protrusion of the rectum through the anus)
  • Infertility in men

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