Cystic Fibrosis & the Liver
Cystic fibrosis is an inherited condition that causes mucus to build up throughout the body. It is a chronic and usually fatal disease that can affect multiple organs, including the liver, where it may produce cirrhosis.
- Cystic fibrosis is a progressive disease that causes cells to produce a thick and sticky mucus. This results in complications in multiple organs, such as the lungs and liver.
- In the liver, this mucus can block the bile duct, causing inflammation and scarring. In severe cases, this scarring can become permanent, a condition called cirrhosis.
- While there are no treatments that can prevent or cure cystic fibrosis, there are ways to manage and reduce symptoms, including medications and nutritional changes.
Cystic fibrosis is an inherited genetic condition. Both parents must possess at least one copy of the defective CF gene to pass it on to their child. Children of parents with one defective CF gene each have a 25 percent chance of inheriting cystic fibrosis.
Cystic fibrosis can lead to liver disease by causing mucus to build up and block bile ducts in the liver. This prevents bile from leaving the liver, which causes inflammation and produces scarring (fibrosis). As a result, the liver cannot function properly.
Cystic fibrosis affects organs and systems throughout the body. This means it can produce a variety of symptoms. In the respiratory, digestive, and reproductive systems, these include:
- Chronic lung infections
- Coughing up blood
- Difficulty breathing
- Male infertility
- Respiratory failure
Cystic fibrosis can also cause liver disease. Common symptoms of this include:
- Blocked bile ducts
- Fluid in the abdomen (ascites)
- Jaundice (yellowing of skin and eyes)
- High blood pressure
- Internal bleeding
The majority of people with cystic fibrosis will experience only mild liver problems. However, 5 to 10 percent of the cystic fibrosis population will develop major liver complications.
Most newborns are now tested for cystic fibrosis. This may involve:
- Blood Test: Looks for high levels of IRT, a chemical produced by the pancreas.
- Genetic Test: Looks for mutated CF genes. This test can confirm a blood or sweat test.
- Sweat Test: Looks for high levels of chloride, which is a common sign of cystic fibrosis.
Because cystic fibrosis can affect the liver, the following are some common ways to diagnose liver disease in people with cystic fibrosis:
- Blood Tests: These will measure levels of bilirubin, albumin, and other substances to determine how well the liver is functioning.
- Endoscopy: This uses a long and narrow tube with a camera attached to check for enlarged blood vessels (varices) that can lead to internal bleeding.
- Imaging Tests: These will allow doctors to form an image of the liver so that they can better detect signs of disease. They may include a computerized tomography (CT) scan, an ultrasound, and magnetic resonance imaging (MRI).
- Liver Biopsy: This is a surgical procedure that removes a tiny portion of liver tissue so that doctors can examine it under a microscope to determine what is wrong.
There is no cure for either cystic fibrosis or cystic fibrosis-related liver disease. However, there are treatments that can help manage their symptoms. For liver disease, these include:
- Endoscopic Surgery: This uses a long and narrow tube to repair broken blood vessels.
- Dietary Changes: This includes reducing or eliminating alcohol consumption and taking vitamin supplements.
- Medications: This includes drugs to help reduce blood pressure and ursodeoxycholic acid (UDCA), a naturally occurring substance that can displace toxic bile acids.
- Liver Transplantation: In cases of severe liver damage, a liver transplantation may be necessary. This removes the entire liver and replaces it with a healthy donor liver.
Neither cystic fibrosis nor cystic fibrosis-related liver disease can be reversed, but both can be managed through treatment and ongoing evaluations. The majority of people with cystic fibrosis will only experience minor liver problems that will require careful monitoring, while a minority will require larger treatments, such as a liver transplantation. In both cases, complications from liver disease can be reduced.
However, cystic fibrosis is a progressive disease that can affect multiple organs at once. When liver disease is combined with lung failure or another complication, it can be much more difficult to manage. Because of this, people with cystic fibrosis often do not live past their 30s.
Our center has a long history of working closely with a range of cystic fibrosis specialists to create personalized and comprehensive treatment plans for patients with both cystic fibrosis and liver disease.
Call us at (877) LIVER MD/ (877) 548-3763 or use our online form to schedule an appointment.