What is CF?

 

Cystic fibrosis (CF) is a life-shortening inherited chronic disease that most critically affects the liver, lungs, sinuses, pancreas, and intestines. It disrupts the body’s salt balance, leaving too little salt and water on the outside of cells and causing the thin layer of mucus that usually keeps the lungs free of germs to become thick and sticky. This mucus is difficult to cough out, and it clogs the lungs and airways, leading to infections and damaged lungs. The disease eventually leads to disability and multisystem failure.  

In addition to breathing difficulties and serious lung infections, CF mucus affects digestion by obstructing the pancreas and stopping natural enzymes from helping the body to break down and absorb food. This can cause vitamin deficiency and malnutrition. Ultimately, lung and/or liver transplantation is often necessary as CF worsens.

Respiratory system signs and symptoms

  • • a persistent cough that produces thick spit (sputum) and mucus;
  • • repeated lung infections, typically caused by unusual germs that don't respond to standard antibiotics;
  • • wheezing and breathlessness (later in life patients often require round-the-clock oxygen therapy); and
  • • inflamed and congested nasal passages, nasal polyps and sinusitis.

Digestive signs and symptoms

The thick mucus blocks ducts that carry digestive enzymes from the pancreas to the small intestine. Without these digestive enzymes, the intestines can't fully absorb nutrients, especially fats and proteins, from food that is eaten. The result is often:

  • • bulky, foul-smelling and greasy stools;
  • • chronic diarrhea;
  • • poor weight gain and growth;
  • • intestinal blockage, particularly in newborns (meconium ileus); and
  • • severe constipation.

As CF gets worse, other problems may occur, such as:

  • • liver disease, often resulting in the need for a transplant;
  • • gallstones;
  • • pancreatitis; and
  • • diabetes.