Cystic Fibrosis Description

Cystic fibrosis (CF) is a serious medical disorder that affects the lung and causes severe nutritional deficiencies. This inherited disorder affects cells which produce sweat, saliva, digestive juices, and mucus. In normal people, these secretions are thin and slippery, but in CF patients, the defective gene causes secretions to become extremely viscous, sticky and rubbery. Instead of acting as a soft lubricant, the secretions plug up pores, ducts and essential tubes which are important for the functioning of the lungs and pancreas. The most dangerous complication of CF is lung failure. Moreover the viscid secretions block the pancreatic duct (tube) and thus causes failure of release of important enzymes for digestion and absorption of nutrients.

Cystic Fibrosis Symptoms

Individuals who have cystic fibrosis symptoms are generally sick. The symptoms of CF do vary; one child may have symptoms of the lung and the other may have symptoms of malabsorption. CF usually presents soon after birth. The infant may not grow properly, will have bulky and oily stools and develop recurrent lung infections. With time, the sweat will be very salty and the infant may develop wheezing and coughing. Other cystic fibrosis symptoms include recurrent nasal polyps and wide bluish looking fingernails (clubbing).

CF is due to a defective gene which regulates salt secretions. Once this occurs, the secretions become very sticky and viscous. The affected gene is acquired from each parent. If the child only has one gene, he or she will not be affected but will be a carrier of the disease. People who carry the gene are otherwise healthy. The biggest risk factor for CF is parent history. If you and your partner each carry the gene, your baby has a 25 percent chance of having CF. This risk is even greater if you are of European descent. The diagnosis of CF includes a sweat test and other genetic analysis. Because the disorder is inherited, other members of the family are usually checked to determine their status. Today, genetic blood testing is available for pregnant females who may want to know if the fetus is affected.

Cystic Fibrosis other causes

CF is a serious illness and causes a variety of lung infections including recurrent pneumonia, sinusitis, bronchitis and severe asthma. Coughing up blood and collapse of the lungs are also very common. Ultimately, most people die from lung complications. Because of severe diarrhea, affected patients develop severe nutritional deficiencies and are unable to absorb most vitamins.

Cystic Fibrosis Treatment

Cystic fibrosis treatment is quite demanding and requires the use of antibiotics to clear infections. Mucus-thinning drugs are available but tend to have side effects. Bronchodilators are the mainstay when one develops wheezing. Most patients need physical therapy to drain the chest and help cough up thick secretions. Other aspects of cystic fibrosis health treatment include the use of oral pancreatic enzymes to help absorb nutrients and prevent malabsorption.

Lung transplant is an option but the problem is obtaining a donor. In CF, both lungs have to be transplanted and there is a very high risk of getting the lungs infected after surgery. So far, not much progress has been towards finding a cure for cystic fibrosis; yet researchers are now studying ways to insert copies of normal genes into the cell of the respiratory tract.

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