Cystic fibrosis - a disease that affects the lungs, digestive system and other organs

Cystic fibrosis - a disease that affects the lungs, digestive system and other organs

What is cystic fibrosis?

Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body.

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 secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas.

Although cystic fibrosis is progressive and requires daily care, people with CF are usually able to attend school and work.

They often have a better quality of life than people with CF had in previous decades.

Symptoms

Cystic fibrosis signs and symptoms vary, depending on the severity of the disease.

Some people may not experience symptoms until their teenage years or adulthood.

People with cystic fibrosis have a higher than normal level of salt in their sweat. Parents often can taste the salt when they kiss their children. Most of the other signs and symptoms of CF affect the respiratory system and digestive system.

Respiratory signs and symptoms

The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs.

This can cause signs and symptoms such as:

  • 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

The thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. 

The result is often:

  • Foul-smelling, greasy stools
  • Poor weight gain and growth
  • Intestinal blockage, particularly in newborns (meconium ileus)
  • Chronic or severe constipation

 

Source:

https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700