Primary biliary cholangitis (PBC) and cystic fibrosis are different health disorders that can affect the liver and cause damage to it. Hepatoprotective agents effectively prevent liver damage in the PBC and cystic fibrosis patients. In this article, we will discuss the role of hepatoprotective agents in PBC and cystic fibrosis treatment.
Overview:
Primary biliary cholangitis (PBC) and Cystic fibrosis (CF)
Primary biliary cholangitis (PBC), or primary biliary cirrhosis, is an autoimmune disorder that leads to inflammation and destruction of the bile duct.
Cystic fibrosis (CF) is an hereditary disorder that leads to severe damage to the lungs, digestive system, liver, and other organs in the body. It causes blockage of small ducts in the liver and leads to CF-related liver disease. Both conditions can cause damage to the liver in the patients, which can even cause liver failure if untreated.
What is a hepatoprotector?
Hepatoprotectors are agents that help to prevent and manage liver injury caused by hepatotoxic agents. They are medications used to protect and support the liver and are often employed in various liver diseases, like Primary Biliary Cholangitis (PBC) and cystic fibrosis.
Symptoms of PBC
Individuals may experience tiredness or fatigue.
You may experience itchiness in the skin.
Individuals may experience yellowing of the skin and eyes.
You may have ankle, leg, feet, and tummy swelling.
You may have a small fatty deposition on the skin layers
Symptoms of CF
You may experience a smell or greasy stools
You may have poor weight gain and growth
Newborn babies may have an intestinal blockage
You may experience chronic or severe constipation
Role of hepatoprotective agent in PBC and cystic fibrosis
Ursodeoxycholic Acid (UDCA): Ursodeoxycholic acid (UDCA) is the primary treatment option for PBC patients. It helps to delay the process of liver damage in the early stage of PBC. It also helps treat the CF-related liver disorder by improving the flow of the bile duct, blocked by the thick and sticky mucus.
For example, the Udiliv 150mg tablet contains the active ingredient ursodeoxycholic acid. It is used to treat PBC and CF-related liver damage.
Obeticholic Acid (OCA): OCA is used in cases where UDCA alone is not effective. It has been shown to improve liver function in PBC patients and prevent the progression of reverse liver fibrosis in CF.
Vitamin supplements: Individuals with cystic fibrosis and PBC are at risk of fat‐soluble vitamin (A, D, E, and K) deficiency. Vitamin supplements can help overcome vitamin deficiency, which PBC and CF cause, and also help provide antioxidants for liver cells.
Immunosuppressive Agents: Immunosuppressive agents such as corticosteroids or azathioprine are used with UDCA to treat the primary biliary cirrhosis-autoimmune hepatitis (PBC-AIH) and a combination of elexacaftor/tezacaftor/ivacaftor is effective in treating CF in patients.
Herbal supplements: Herbal supplements like Silymarin (milk thistle) protect the liver cells from CF-related liver damage and have a strong antioxidant property to promote liver regeneration.
It is important to note that your healthcare provider will give you an appropriate hepatoprotective agent based on your previous health history and disease condition.
Side effects of hepatoprotective agents
Hepatoprotective drugs can cause some side effects, which are given below:
Pasty stool: Individuals may experience a sticky poop during bowel movement.
Diarrhea: You may experience diarrhea or loose and watery stool.
Rash: Individuals may experience a rash in the skin
Abdominal pain: Patients may experience abdominal pain during the medication.
Inform your doctor immediately if you have any allergic reactions like difficulty breathing, tight throat tightness having, hives, or swelling.
Tips for PBC and CF!
Avoid or limit your alcohol intake.
Eat a healthy diet.
Stop smoking or tobacco consumption.
Drink enough water to tackle the water loss.
Hepatoprotective agents play an important role in protecting the liver against primary biliary cholangitis and cystic fibrosis. Always consult with healthcare professionals for personalized advice and to stay updated on the latest developments in the management of these conditions.