What is Cystic Fibrosis?
Cystic Fibrosis is an inherited disease that affects the lungs and digestive system. It is caused by a defective gene, and it affects the body’s ability to move salt and water in and out of the cells. This causes the body to produce abnormally thick and sticky mucus that clogs the lungs and impedes digestion. Cystic Fibrosis is one of the most common inherited diseases in the United States, affecting 1 in every 3,500 babies born.
Signs and Symptoms of Cystic Fibrosis
The severity of symptoms can vary widely, but they usually include:
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Coughing, often with phlegm
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Wheezing and shortness of breath
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Difficulty gaining weight
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Recurring lung infections
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Poor growth
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Greasy, bulky stools
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Salty-tasting skin
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Poor appetite
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Rectal prolapse (when part of the large intestine slips out of the anus)
Causes of Cystic Fibrosis
Cystic Fibrosis is caused by a defective gene, usually inherited from both parents. The gene causes the body to produce a thick, sticky mucus that clogs the lungs and impedes digestion.
Risk Factors for Cystic Fibrosis
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Having a family history of the disease
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Being born to a parent with the defective gene
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Being of Caucasian descent
Prevention of Cystic Fibrosis
Because Cystic Fibrosis is an inherited disease, there is no known way to prevent it. However, genetic testing and screening can help identify those at risk for the disease.
Diagnosis of Cystic Fibrosis
Cystic Fibrosis is usually diagnosed in childhood, but it can also be diagnosed in adults. Diagnosis is made through a combination of physical exam, family history, genetic testing, and laboratory tests.
Treatment of Cystic Fibrosis
There is no cure for Cystic Fibrosis, but there are treatments available to help manage the symptoms and improve quality of life. These treatments include:
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Medications: Antibiotics, bronchodilators, and anti-inflammatory drugs to help reduce inflammation in the lungs and airways
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Airway clearance: Chest physical therapy and breathing exercises to help clear the lungs of mucus
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Nutrition: A high-calorie, high-fat diet to help maintain a healthy weight
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Oxygen therapy: Supplemental oxygen to help with breathing
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Surgery: Surgery to remove blockages in the lungs or to repair damage caused by the disease
Coping and Support for Cystic Fibrosis
Living with Cystic Fibrosis can be challenging, but there are a variety of ways to manage the disease. Support groups can provide emotional support, while medical professionals can provide advice on managing the disease.
Complications of Cystic Fibrosis
Complications of Cystic Fibrosis can include:
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Lung damage
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Liver disease
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Diabetes
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Poor nutrition
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Poor growth
Living with Cystic Fibrosis
Living with Cystic Fibrosis can be challenging, but it is possible to live a full and active life with the disease. It is important to stay on top of treatments and take steps to prevent infection. Eating a healthy diet, getting adequate rest, and avoiding contact with people who are sick can help to reduce the risk of complications.
