Fibrosing Colonopathy in Cystic Fibrosis
Cystic fibrosis (CF) can cause different complications in the body that may be mild to severe. They can also vary from person to person. Some of these complications affect the gut and organs in the abdomen.
Fibrosing colonopathy (FC) is one complication that can occur with CF. It is a rare condition related to the gut. Because it is rare, it can be hard to find information about FC. Learning about the condition and how it affects the body can help you recognize its signs and get the care you need.1,2
What is fibrosing colonopathy?
Fibrosing colonopathy is a health problem that affects the colon, which is also known as the large bowel or intestine. This is where the final stages of food digestion occurs and waste is escorted out of the body.1
FC causes the colon to swell, shorten, and scar. It most affects people with CF. It is also more common in children than adults.1,2
Fibrosis happens because of long-term injury or damage to a tissue or organ. This repeated damage causes the body to keep trying to repair itself. The body releases chemicals to try to heal the tissue or organ. Over time, this causes fibrous, hard scars to form. This fibrous tissue changes the function of the tissue or organ that has been injured or damaged.3,4
How is fibrosing colonopathy caused?
The exact cause of fibrosing colonopathy is not well known. In CF, fibrosing colonopathy happens as a complication from taking pancreatic enzyme therapy (PERT) at high doses. Most people with CF need to take extra enzymes to make up for what their pancreas can’t do. Some people with class IV or V CF mutations take PERT.2
Researchers think that PERT breaks down the lining of the colon. Once the lining of the colon has been broken, the body tries to repair itself by releasing chemicals. This starts the process of fibrosis and scarring.2
High-dose formulas of PERT are no longer on the market for people to use. This means that the number of people with FC has gone down over time.2
What are the symptoms of FC?
Most people with fibrosing colonopathy have a bloated abdomen and abdominal pain. The pain can be very severe. Other symptoms of FC may include:2
Can fibrosing colonopathy be prevented?
High doses of pancreatic enzymes have been linked to fibrosing colonopathy. However, FC still occurs when enzyme doses are within safe levels. Because FC is a rare complication, doctors are still trying to figure out if it can be prevented.2
What to do if you think you have FC
If you think you or your child may have FC, call your doctor. Your doctor and your CF team need to know about any symptoms you or your child may be experiencing. Track your symptoms and tell your doctor.
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