There's no cure yet for primary biliary cholangitis, (previously known as primary biliary cirrhosis), a long-term liver disease. But there are ways to slow its progress and help ease your symptoms.
Medications
The main drug used to treat the disease is ursodeoxycholic acid, or UDCA. Your doctor might also call it ursodiol. It’s a natural bile acid that helps move bile out of your liver and into your small intestine.
UDCA will probably be the first treatment your doctor suggests. It can do a lot of good if you start it early on. If your doctor prescribes it, you may have to take it every day for the rest of your life. It slows liver damage and may make it less likely that you’ll need a transplant. You might notice side effects like weight gain, diarrhea, and hair loss.
If UDCA alone doesn’t help, or if you can't handle its side effects, your doctor may recommend obeticholic acid (Ocaliva). You might take it alone or along with UDCA. It boosts bile flow and eases how much bile acid your liver makes. Side effects might include itchy skin, belly pain, achy joints, and a sore throat.
Doctors are researching other drugs to see if they can help. Some, like colchicine and methotrexate, suppress your immune system. Other studies suggest that fenofibrate or prednisone might help. Your doctor can help you decide what’s best for you.
Treating Symptoms
There are ways to help ease the discomfort.
- Itchy skin: This common symptom may also be the most annoying. It can be mild or so severe that it bothers you day and night. If it isn’t too bad, try over-the-counter antihistamines that contain diphenhydramine. They can make you sleepy, so you might try them if itching keeps you awake at night. If they don't help, your doctor can prescribe something else like cholestyramine (Locholest, Prevalite, Questran), naltrexone (Revia, Vivtrol), rifampin (Rifadin, Rimactane), and sertraline (Zoloft).
- Dry eyes and mouth: Start with over-the-counter artificial tears. If they don't work, see your eye doctor for prescription drops. You can also find saliva substitutes to help with a dry mouth. Prescription options are available if they don’t do the trick. You can also suck on hard candy or chew gum to help with dry mouth.
- Fatigue: PBC can make you tired all the time. But your daily habits, other medical conditions, and even drugs you take can make it worse. Work with your doctor to take care of those issues, and you might ease your fatigue. A drug called modafinil (Provigil) might help.
Liver Transplant
Treatments usually keep primary biliary cholangitis from getting worse. If they don’t, your liver could start to fail. If that happens, your doctor will discuss a liver transplant. In this surgery, your liver is removed and replaced with a healthy, donated liver. PBC can return even after a liver transplant. But it might take years.
Lifestyle Changes
Medication is the main way to treat primary biliary cholangitis. Your doctor will work with you to find the best drug treatment plan. But you can also make simple changes in your daily life that will help ease your symptoms.
Eat a healthy diet
What you put in your mouth matters when you have primary biliary cholangitis. Your body can’t absorb vitamins and minerals the way it’s supposed to, so you may not be getting the nutrients you need. The disease can change your metabolism, too, so you may be eating less because you just don’t feel hungry.
Your doctor can suggest a healthy meal plan. It's smart to eat a balanced diet rich in fresh fruits, vegetables, and whole grains. If you still can’t get enough nutrients and calories, your doctor may prescribe a liquid supplement. These nutritional drinks give you the vitamins you need.
Other diet tips to consider:
- Watch your salt. Choose low-salt and no-salt foods when possible. Too much salt can cause swelling.
- Go low-fat. Watch the fat in your diet, especially if you aren't at your ideal weight or have fatty liver.
- Drink up.Water, that is. If swelling is a problem, ask your doctor if you should drink less.
- Cut back on booze. If you drink alcohol, you should stop or at least cut back. It's your liver's job to process what you drink. Drinking can make your already damaged liver work even harder.
- Skip raw shellfish. It can be home to bacteria that could cause a serious infection. Primary biliary cholangitis affects your immune system, so you’re more likely to get this kind of infection.
Other lifestyle changes
These steps can also help you feel better:
- Get some kind of exercise every day. Walking and swimming are good ways to strengthen your bones and fight osteoporosis, which often comes with PBC. Plus even a light workout can boost your mood and make you feel less tired.
- Take care of your skin. It may get itchy or look darker. Good skin care will help.
- Ease your stress. It can make many symptoms worse, including fatigue.
- Go to the dentist. The dry mouth that comes with PBC can raise your odds of problems like tooth decay. Your dentist can catch issues early on.
- Stop smoking. If you smoke, quit. Studies show that it can trigger the disease or make it worse.
Possible Complications
If your PBC isn't treated, or if it gets worse and causes liver damage, there's a chance you could have other serious problems. That's why your doctor will keep a close eye on you. Some of those issues include:
- Portal hypertension. Your portal vein carries blood from your stomach, intestines, spleen, gallbladder, and pancreas to your liver. Scar tissue from cholangitis blocks normal circulation and can boost pressure in your portal vein. That leads to problems like swelling, an enlarged spleen and blood vessels, and toxin buildup.
- Toxic substance buildup. When your liver can't remove toxic substances from your blood, they could build up in your brain. You might notice problems with memory and concentration. It may also cause confusion, sleep problems, and personality shifts. It can become so serious that you wind up in a coma. This condition is called hepatic encephalopathy.
- Enlarged veins. When blood can’t flow freely through your portal vein, it can back up elsewhere, especially your stomach and esophagus. That leads to more pressure in your veins, which can trigger bleeding and enlarged blood vessels, called varices. This is serious, and you'd need to see a doctor right away.
- Enlarged spleen. Portal hypertension can fill your spleen with white blood cells and platelets. That means there are fewer of them elsewhere in your blood where your body needs them.
- Swelling. When your liver fails, fluid often builds up in your ankles, feet, legs, and belly. If there’s too much, you could get a serious infection called bacterial peritonitis. You’ll need to treat it right away.
- Weak bones. Your bones could become thin, weak, and easier to break. You could get osteoporosis. Your doctor will prescribe calcium and vitamin D to help keep your bones strong and prevent problems.
- Gallstones and bile duct stones. When bile backs up and can't flow through the ducts, it can harden and turn into stones. You could also get stones if bile can't flow easily to and from your gallbladder. They’re often painful and can lead to infections.
- Problems with fat absorption. When bile can't move around the way it should, it can be hard for your body to absorb fat. You might not get enough of vitamins A, D, E, and K -- the ones that dissolve in fat. You might also have loose, greasy bowel movements because of fat buildup in your stool. Your doctor might suggest you take these vitamins as replacement therapy.
- Liver cancer. The damage that comes with cholangitis could make you more likely to get it. Treatments work best if you find tumors early, so your doctor will check for signs of liver cancer every 6-12 months. They might use blood tests, ultrasound, or both.
- Other diseases. You also may be more likely to get conditions related to your immune system, like thyroid problems, limited scleroderma (CREST syndrome), Sjogren’s syndrome, and rheumatoid arthritis.
