The Connection Between Exocrine Pancreatic Insufficiency and Cystic Fibrosis ~ JPEEI CLINIC
Cystic fibrosis is an inherited disorder that causes body fluids to be thick and sticky instead of thin and runny. This severely impacts the lungs and digestive system.
People with cystic fibrosis have breathing problems because mucus clogs their lungs and makes them vulnerable to infections. Thick mucus also clogs the pancreas and hinders the release of digestive enzymes. About 90 percent of people with cystic fibrosis also develop exocrine pancreatic insufficiency (EPI).
Keep reading to learn more about the relationship between these two conditions.
Cystic fibrosis is caused by a defect in the CFTR gene. A mutation in this gene causes cells to make thick, sticky fluids. Most people with cystic fibrosis are diagnosed at a young age.
Cystic fibrosis is a genetic disease. If your parents have the disease or if they carry the defective gene, you’re at an increased risk for developing the disease. A person with cystic fibrosis has to inherit two mutated genes, one from each parent. If you only carry one copy of the gene, you won’t have cystic fibrosis but you’re a carrier of the disease. If two gene carriers have a child, there’s a 25 percent chance that their child will have cystic fibrosis. There’s a 50 percent chance their child will carry the gene but not have cystic fibrosis.
Cystic fibrosis is also more common in people of Northern European descent.
How are EPI and cystic fibrosis related?
EPI is a major complication of cystic fibrosis. Cystic fibrosis is the second most common cause of EPI, after chronic pancreatitis. It occurs because the thick mucus in your pancreas blocks pancreatic enzymes from entering the small intestine.
The lack of pancreatic enzymes means your digestive tract has to pass partially undigested food. Fats and proteins are especially hard for people with EPI to digest.
This partial digestion and absorption of food can lead to:
fatty and loose stools
Even if you eat a normal amount of food, cystic fibrosis can make it difficult to maintain a healthy weight.
What kinds of treatments are available for EPI?
A healthy lifestyle and balanced diet can help you manage your EPI. This means limiting alcohol intake, avoiding smoking, and eating a nutritious diet with plenty of vegetables and whole grains. Most people with cystic fibrosis can eat a standard diet where 35 to 45 percent of calories come from fat.
You should also take enzyme replacements with all of your meals and snacks to improve digestion. Supplement use may help make up for vitamins that EPI prevents your body from absorbing.
If you’re unable to maintain a healthy weight, your doctor may suggest using a feeding tube at night to prevent malnutrition from EPI.
It’s important for your doctor to monitor your pancreatic function, even if you currently don’t have decreased function because it can decline in the future. Doing so will make your condition more manageable and can lower your chances of further damage to your pancreas.
In the past, people with cystic fibrosis had very short life expectancies. Today, 80 percent of people with cystic fibrosis reach adulthood. This is because of big advancements in treatment and symptom management. So while there’s still no cure for cystic fibrosis, there is a lot of hope.