Research Into Digestive System Issues

Pancreatic Insufficiency

The pancreas is attached to the small intestine behind your stomach and is crucial for proper digestion. Enzymes made by the pancreas are very important for getting nutrients, calories and vitamins into our bodies; they are the heart of proper digestion and absorption. In people with CF, sticky mucus blocks ducts in the pancreas and prevents enzymes from reaching the small intestine to digest food. This problem, called “pancreatic insufficiency,” affects about 90 percent of people with CF.

For more information on how the pancreas is affected in CF, read about the digestive tract.

Research Underway

We're funding research to develop a non-porcine enzyme replacement therapy with improved activity. This will offer an alternative to people with CF with pancreatic insufficiency who need to take enzymes.

We have also helped organize a group of gastrointestinal (GI) specialists to focus on the treatment and research of GI issues in CF. Called the Developing Innovative Gastroenterology Specialty Training program, or DIGEST, this group of GI doctors is developing best practices and gaining an improved understanding of abdominal symptoms to lead to better treatments.

The GALAXY trial, the largest-ever study of GI symptoms in CF, was designed and conducted by DIGEST doctors to gauge which symptoms affect people with CF the most. Researchers are using these results to inform and prioritize future studies, including research to address:

Adult malnutrition
Constipation
Small intestinal bacterial overgrowth
The use of proton pump inhibitors in CF

In this video, Meghana N. Sathe, M.D., associate professor of pediatrics and co-director of the pediatric CF center at the University of Texas Southwestern Medical Center/Children's Health, explains why people with CF can have problems with nutrition and gastrointestinal (GI) issues and discusses the research underway.

Current Treatments

Various medications are currently available to help relieve the various symptoms associated with pancreatic insufficiency.

Cystic Fibrosis-Associated Liver Disease

People with CF also can experience problems in the liver because bile, a fluid that helps the body absorb fat, becomes abnormal and thick, blocking small bile ducts in the liver. The ducts become inflamed, which leads to scarring. Consequently, approximately 10 percent of people with CF develop a condition called CF-associated liver disease (CFLD).

For more information on how the liver is affected in CF, read about the digestive tract.

Research Underway

Because CFLD is difficult to identify in its early stages, doctors need better methods to diagnose it in time to minimize liver damage. The Cystic Fibrosis Liver Disease Network, an innovative collaboration between the CF Foundation and the National Institute of Diabetes and Digestive and Kidney Diseases, is conducting several studies investigating the best methods of screening for the presence of liver disease:

In the PUSH study, researchers are using ultrasound to see if they can identify biomarkers that would help them detect cirrhosis. If they are successful, scientists would be able to better understand who may be at risk of developing cirrhosis and identify potential ways to treat it.
The ELASTIC study is investigating a noninvasive method to identify scarring in the liver.
Another study is using MRI as a tool to identify individuals with CF who have advanced liver disease.

Current Treatments

People with CFLD do not have many treatment options, but a multidisciplinary approach that includes the CF care center team is recommended in clinical care guidelines. The team will determine the causes of specific liver abnormalities and treat them accordingly. This treatment can include nutritional therapy and a medication that thins the bile.

Colorectal Cancer

Colorectal cancer is a cancer that begins in the colon or rectum. Adults with CF have a 5-10 times greater risk of colorectal cancer than the general population and a 25-30 times greater risk after an organ transplant.

Although we do not know for certain why people with CF have a greater risk of developing colorectal cancer, it is thought to be associated with the CFTR gene mutation. In general, transplant recipients have an increased risk for all types of cancer because the medications they must take to prevent rejection weaken the immune system.

Research Underway

Research is being conducted to understand the mechanism — or the underlying process — that causes this higher risk. In the meantime, the Foundation convened a multidisciplinary team of experts to develop colorectal cancer screening recommendations.

The Foundation also plans to conduct research to find ways to provide caregivers and people with CF more information to help inform their discussions about colorectal cancer screening.