Cystic Fibrosis Information (Pictures)
Cystic fibrosis (CF) is a life-threatening disorder that causes severe lung damage and nutritional deficiencies.
Sometimes called mucoviscidosis, cystic fibrosis is a generalized dysfunction of the exocrine glands that affects multiple organ systems. Transmitted as an autosomal recessive trait, it's the most common fatal genetic disease of white children.
Cystic fibrosis is a chronic disease; however, recent improvements in treatments have increased the average life expectancy from age 16 to age 28 and older.
Incidence of cystic fibrosis is highest (1 in 2,000 live births) in Whites of northern European ancestry and lowest in Blacks (1 in 17,000 live births), Native Americans, and people of Asian ancestry. The disease occurs equally in both sexes.
Signs and symptoms
The first signs and symptoms of cystic fibrosis generally include any of the following:
In advanced stages of cystic fibrosis, the person will experience any of the above signs and symptoms, as well as those listed below.
If people with CF do not have proper treatment, they will continue to have oily bowel movements, abdominal pain, and problems putting on weight. Constipation is also a frequent symptom. Occasionally the gut becomes completely blocked, resulting in extreme stomach pain.
The Cystic Fibrosis Foundation sets the following standards for a definitive diagnosis:
The aim of treatment is to help the child lead as normal a life as possible. Specific treatment depends on the organ systems involved.
Aerosol therapy includes intermittent nebulizer treatments before postural drainage to loosen secretions. Dornase alfa, a genetically engineered pulmonary enzyme given by aerosol nebulizer, helps thin airway mucus, improving lung function and reducing the risk of pulmonary infection.
A patient with pulmonary infection will need mucopurulent secretions loosened and removed, using an intermittent nebulizer and postural drainage to relieve obstruction. Use of a mist tent is controversial because mist particles may become trapped in the esophagus and stomach and never even reach the lungs. Broadspectrum antimicrobials help combat infection. Oxygen therapy is used as needed.
Recently, some patients have undergone lung transplantation to reduce the effects of the disease. Also, clinical trials of aerosol gene therapy show promise in reducing pulmonary symptoms.
There is no way to prevent cystic fibrosis. Screening of family members of a cystic fibrosis patient may detect the cystic fibrosis gene in between 60 and 90% of carriers, depending on the test used.
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