Cystic fibrosis (CF) is a hereditary disease that affects the lungs and digestive system. The body produces thick and sticky mucus that can clog the lungs and obstruct the pancreas.
Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery, but in people with CF, a defective gene causes the secretions to become sticky and thick.
Instead of acting as lubricants, the secreting plug up tubes, ducts and passageways, especially in the lungs and pancreas.
Symptoms
The symptoms of CF can vary depending on the person and the severity of the condition. The age at which symptoms develop can also differ. Symptoms may appear at infancy, but for other children, symptoms may not begin until after puberty or even later in life. As time passes, the symptoms associated with the disease may get better or worsen.
One of the first signs of cystic fibrosis is a strong salty taste to the skin.
Other symptoms of cystic fibrosis result from complications that affect:
- The lungs
- The pancreas
- The liver
- Other glandular organs
Respiratory Signs and Symptoms
- A persistent cough that produces thick mucus (sputum)
- Wheezing
- Exercise intolerance
- Repeated lung infections
- Inflamed nasal passages or a stuffy nose
- Recurrent sinusitis
Digestive Signs and Symptoms
- Foul-smelling, greasy stools
- Poor weight gain and growth
- Intestinal blockage, particularly in newborns (meconium ileus)
- Chronic or severe constipation, which may include frequent straining while trying to pass stool, eventually causing part of the rectum to protrude outside the anus (rectal prolapse)
Treatment
Although there’s no cure for cystic fibrosis, there are various treatments available that may help relieve symptoms and reduce the risk of complications.
- Medications
- Antibiotics
- Mucus-thinning medications
- Nonsteroidal anti-inflammatory drugs (NSAIDs)
- Bronchodilator
- Surgical Procedures
- Chest Therapy
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