Exocrine Pancreatic Insufficiency and Cystic Fibrosis – Is There a Connection?

Exocrine pancreatic insufficiency (EPI) is a medical condition that is marked by a deficiency of the exocrine pancreatic enzymes. This leads to an inability to digest food properly. Cystic fibrosis, on the other hand, is an inherited disorder that leads to body fluids becoming thick and sticky instead of being thin and runny as it should be. This leads to problems with the lungs and the digestive system. People who have cystic fibrosis develop breathing problems since the thick mucus clogs the lungs, making them susceptible to infections. This thick mucus also obstructs the pancreas, disrupting the release of digestive enzymes. Nearly 90 percent of people with cystic fibrosis go on to develop EPI. Read on to find out if there is a connection between exocrine pancreatic insufficiency and cystic fibrosis.

Causes & Risk Factors of Cystic Fibrosis

Cystic fibrosis is an inherited condition that is caused by a mutation or defect in the CFTR gene. A mutation in the CFTR gene leads to the cells to start producing thick and sticky fluids. A majority of people with cystic fibrosis are typically diagnosed at quite a young age. (1)

As cystic fibrosis is an inherited disease, if either of your parents has the condition, then you are also at an increased risk of developing this disease.

A person with cystic fibrosis has inherited two mutated genes, one from each parent. If you only have one copy of the mutated gene, you will not develop the disease, but you will still be a carrier of the disease.

It has also been observed that people of Northern European descent are at a higher risk of getting cystic fibrosis.

Exocrine Pancreatic Insufficiency and Cystic Fibrosis – Is There a Connection?

Exocrine pancreatic insufficiency (EPI) is known as a major complication of cystic fibrosis. Cystic fibrosis is, in fact, the second most common cause of Exocrine pancreatic insufficiency (EPI), with chronic pancreatitis being the leading cause. (2)

This happens because the thick mucus from your pancreas blocks the pancreatic enzymes from entering into the small intestine.

The lack of pancreatic enzymes means that your gastrointestinal tract has to process partially undigested food. Proteins and fats are particularly difficult for people with Exocrine pancreatic insufficiency (EPI)to digest.

The partial absorption and digestion of food can cause:

Even if you eat a healthy amount of food, but having cystic fibrosis makes it challenging to maintain a healthy weight, and it may often lead to malnutrition.

Treatments for Exocrine Pancreatic Insufficiency

While there is no cure for Exocrine pancreatic insufficiency (EPI), your doctor will work together with you to find the best possible treatment to manage and ease your symptoms.

A healthy and balanced diet and lifestyle can help you to a great extent in managing Exocrine pancreatic insufficiency (EPI). This also means that you will have to restrict your alcohol intake, quit smoking, and at the same time, consume a healthy diet that is rich in fresh vegetables, fruits, and whole grains.

A majority of people with cystic fibrosis are able to eat a regular diet in which 35 to 45 percent of the calories are derived from fats. (3)

You also need to be taking enzyme replacements along with all your meals and even snacks in order to improve and boost your digestion. The use of supplements may also help make up for the vitamins the medical condition of Exocrine pancreatic insufficiency (EPI) prevents your body from absorbing naturally.

If you are unable to achieve and maintain a healthy weight, then your doctor may also suggest the use of a feeding tube in the night to help prevent malnutrition from Exocrine pancreatic insufficiency (EPI).

It is very important that your doctor monitor the functioning of your pancreas very carefully, even if you presently do not have a decreased pancreatic function as it can easily decline in the future. Monitoring of your pancreatic functioning will help make your condition more manageable, and you will also be able to reduce the chances of causing any further damage to your pancreas.

Conclusion

Traditionally in the past, it had been observed that people with Exocrine pancreatic insufficiency (EPI) and cystic fibrosis had a very short life expectancy. Today, however, nearly 80 percent (3) of all people with cystic fibrosis successfully reach adulthood and have a good life expectancy. This is because of the many significant advancements in the treatment of the condition. So even though there is no cure for cystic fibrosis and Exocrine pancreatic insufficiency (EPI), there are still many ways of managing both the conditions effectively.

References:

  1. Zielenski, J. and Tsui, L.C., 1995. Cystic fibrosis: genotypic and phenotypic variations. Annual review of genetics, 29(1), pp.777-807.
  2. Identifyepi.com. (2019). Causes of EPI – Cystic Fibrosis, Chronic Pancreatitis & More. [online] Available at: https://www.identifyepi.com/hcp/epi-associated-conditions [Accessed 11 Nov. 2019].
  3. Clevelandclinicmeded.com. (2019). Disease Management Project – Missing Chapter. [online] Available at: http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/pulmonary/cystic-fibrosis/ [Accessed 11 Nov. 2019].

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