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Cystic Fibrosis SymptomsCystic fibrosis is a chronic (lifelong) disease that requires constant monitoring and medical treatment. Because there are over 1,300 mutations of the gene that causes cystic fibrosis, a person's symptoms depend on the specific gene mutation inherited. For some people the symptoms may be mild and for others the symptoms may be severe. The defective gene alters the body's ability to move salt (sodium chloride) in and out of cells. As a result, mucus secreted by the body's cavities and passages that lead outside the body (such as the respiratory and digestive systems) becomes thick and sticky. Instead of flowing normally and acting as a protective lubricant, the mucus obstructs many of the body's organs and passages. Salt also builds up in the sweat. Common SymptomsCommon cystic fibrosis symptoms include: - Coughing, shortness of breath, or wheezing
- Diarrhea
- Fatigue
- Poor growth
- Recurrent sinusitis, bronchitis, or pneumonia
- Salty-tasting skin
- Stools that are foul smelling, pale colored, or greasy
- Thick sputum
- Weight loss or failure to gain weight normally despite an increased appetite
Symptom Breakdown by OrganCystic fibrosis interferes with the normal operation of many organs throughout the body. The symptoms typically develop during the first year of life. The specific organs affected and the intensity of the symptoms vary among people. The primary organs affected include the: LungsThe mucus in the lungs frequently builds up and blocks its small air passages. The nutrients in the mucus provide additional food for bacteria, which then multiply rapidly. The air passages in the lungs become inflamed and people develop chronic lung infections. Some people develop respiratory failure, which is the most common cause of death in people with cystic fibrosis. Air sacs in the lungs may swell and rupture. When this occurs, air enters the chest cavity surrounding the lungs (pneumothorax) and may cause the lungs to collapse. Some people with chronic respiratory problems develop an enlargement or rounding (clubbing) of their fingertips and toes due to the lungs' inability to provide the bloodstream with sufficient oxygen. PancreasThe pancreas normally secretes pancreatic juices into the intestine and insulin into the bloodstream. The small intestine requires pancreatic juices to digest fats, proteins, carbohydrates, and fat-soluble vitamins (A, D, E, and K). In cystic fibrosis, mucus builds up in and blocks the pancreatic ducts (tubes), preventing the pancreatic juices from reaching the small intestine. Without the pancreatic juices, the small intestine cannot digest food. Children and adults become malnourished, and children are unable to receive the nutrition they need to grow normally. Some people may eventually develop pancreatitis (inflammation of the pancreas) or insulin-dependent diabetes. IntestineIntestinal symptoms often develop at birth. Newborns with cystic fibrosis may have an abnormal thickening of their first stool, which may obstruct their intestine (meconium ileus). When this occurs, the newborns may not have a bowel movement in first 24 to 48 hours of life. Surgery is required if medications are unable to break up the blockage. Intestinal blockages may also develop in children and adults. Occasionally, one part of the intestine slides into another part (intussusception). When this occurs, the blood flow to the area becomes reduced and gangrene (tissue decay or death) may develop. Surgery is often needed to correct the condition. On rare occasions, constipation may cause the rectum to protrude through the anus (rectal prolapse), which also requires surgery. Sweat glandsPeople with cystic fibrosis lose large amounts of salt during perspiration. The salt absorption in their sweat glands becomes impaired, and their sweat becomes extremely salty. This loss of salt upsets the blood's mineral balance and may cause the body to overheat. SinusesSome people with cystic fibrosis develop nasal polyps, which are benign (noncancerous) tumors in the lining of the nose or sinuses. If the polyps obstruct the flow of air, they may have to be removed surgically. Reproductive organsAlthough cystic fibrosis does not affect a person's sexual performance, it does affect fertility. Most men with cystic fibrosis become infertile due to an absent or undeveloped vas deferens (ducts that transport semen). Some women develop thickened cervical mucus or menstrual irregularities, which may interfere with conception. LiverThickened bile secreted by the liver may block the liver's bile ducts. The liver may become inflamed and unable to remove toxins from the blood. Chronic inflammation may result in permanent scarring of the liver (cirrhosis). GallbladderThe gallbladder may fill with thick bile and not function well. Although some people with cystic fibrosis develop gallstones, few develop symptoms and surgery is rarely needed. HeartChronic respiratory problems may affect the right side of the heart, which pumps blood through the lungs. The strain of pumping blood through diseased lungs weakens the right side of the heart and may eventually cause it to fail (cor pulmonale).
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