Cystic fibrosis is a life-threatening genetic disorder that causes thick, sticky mucus to build up in the lungs, digestive tract, and other areas of the body. It is one of the most common chronic lung diseases found in children and young adults.
Causes and Risk Factors
Cystic fibrosis (CF) is caused by a defective gene that causes the body to produce an abnormally thick and sticky fluid called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas, which is the organ that helps break down and absorb food.
In effect, life-threatening lung infections and serious digestion problems stem from this collection of mucus.. The disease can also affect the sweat glands and a man’s reproductive system.
Millions of Americans carry the defective CF gene, but do not have any symptoms. This is because a person with CF must inherit two defective CF genes — one from each parent.
Symptoms
Symptoms in newborns may include:
- Delayed growth
- Failure to gain weight normally during childhood
- No bowel movements in the first 24 to 48 hours of life
Symptoms related to the lungs and sinuses may include:
- Coughing or increased mucus in the sinuses or lungs
- Fatigue
- Nasal congestion
- Recurrent episodes of pneumonia
- Sinus pain or pressure
Symptoms that may be noticed later in life include:
- Infertility (in men)
- Respiratory symptoms
- Repeated inflammation of the pancreas (pancreatitis)
Treatment
An early diagnosis of CF along with a comprehensive treatment plan can improve both survival and quality of life. Follow-up visits and monitoring are very important. If possible, patients should be cared for at cystic fibrosis specialty clinics, which are found in many communities.
Treatment for lung problems includes:
- Antibiotics
- Inhaled medicines
- DNase enzyme therapy
- High concentration of salt solutions (hypertonic saline)
- Flu vaccine and pneumococcal polysaccharide vaccine (PPV) shots yearly
- Aerobic exercise
Facts about CF
- CF affects more than 30,000 kids and young adults in the United States.
- Today in the United States, about 1 of every 3,600 Caucasian children is born with CF while 1 of every 17,000 African Americans inherits the disease.
- CF is the most common, deadly, inherited disorder that affects Caucasians in the United States. It is more common among those of Northern or Central European descent.
- Most children with CF are diagnosed by age 2.
- A small number of people are not diagnosed with CF until age 18 or older. These patients usually have a milder form of the disease.
Learn more about cystic fibrosis by visiting www.cff.org
