Basic PSC Facts
Primary Sclerosing Cholangitis (PSC) is a chronic bile duct disease that damages the bile ducts inside and outside the liver. Bile ducts carry bile from the liver to the intestines. With PSC, bile ducts are inflamed, and the inflammation leads to scarring and narrowing of the affected ducts. Eventually, blockages may occur. As the scarring blocks more and more ducts, bile becomes trapped in the liver. This damages the liver and can result in fibrosis and cirrhosis of the liver and liver failure. Patients may eventually require a liver transplant.
PSC is a rare disease that predominantly affects 30-40 year-old men. However, PSC also occurs in children of any age, women, and the elderly. PSC affects about twice as many men as women. About 43 percent do not have any symptoms when diagnosed. It is estimated that nearly 21 per 100,000 men and 6 per 100,000 women have the disease. PSC is often found in people with inflammatory bowel disease (IBD), most often ulcerative colitis (UC) and sometimes Crohn’s disease. Over 75 percent of PSC patients have ulcerative colitis. PSC is also associated with other autoimmune diseases.
Although no one knows the causes of PSC, a great deal of research is underway to understand the disease better. We know that PSC may have genetic, autoimmune, and environmental origins. It is important to remember that no two PSC patients are alike and that PSC isn’t contagious. It can’t be transmitted through kissing, sexual activity, touching, or blood transfusion. There may be a genetic predisposition to PSC, but most children of PSCers are healthy and unaffected.
There are medications and procedures that can help relieve some aspects of the disease and symptoms, but the only definitive treatment is a liver transplant. Still, many PSCers live long and productive lives with the disease, and they may never need a transplant.
There are currently no proven treatments that change the disease’s course or slow its progression, but there are medications and procedures that help relieve some PSC symptoms. For example, an inserted stent can widen a blocked bile duct, and some medications help with the itching that commonly occurs in PSC patients. Although the only definitive treatment is a liver transplant, many PSCers live long and productive lives with the disease, and they may never need a transplant.
Complications of PSC can include bacterial cholangitis, biliary stone disease, dominant stricture (especially in the common bile duct), and cholangiocarcinoma (cancer of the bile ducts). In advanced PSC, patients may experience hepatic encephalopathy, varices, ascites, bleeding disorders, jaundice, and other problems.
You can find more information on this website. Download our brochures, which you may find useful to explain the disease to physicians and family. Our brochures, Living with PSC, Diagnosing PSC, PSC Medications, and Pediatric PSC are all available by clicking here.
General PSC Frequently Asked Questions are available by clicking here
Common PSC Medications
Ursodeoxycholic acid(Ursodiol, Urso Forte, UDCA, URSO, Actigall, Ursofalk)
Urso is a popular medication used for patients diagnosed with PSC. In addition, some anti-inflammatory properties have been noticed. Urso helps to fight toxic bile acids that build up in the liver cells. In the United States, Urso is available under different trade names such as Actigall (typically 300 mg. capsules) and URSO (typically 250 mg tablets). Urso Forte is available in a 500 mg tablet and, along with Urso 250, is the only FDA approved treatment for patients with PBC (Primary Biliary Cholangitis), a disease similar to PSC. Another variation of Urso is available in Australia under the name Ursofalk (250 mg capsules). Ursofalk is also available in a liquid formulation for pediatric patients.
Ursodiol is typically prescribed in typical dosages of 15-30 mg/kg a day. The benefit of high-dose ursodiol is questionable, and a recent study suggests that high doses may actually be detrimental. For this reason, most hepatologists prescribing ursodiol today will uses doses in the range of 15 to 20 mg/kg a day. Another unproven, but suggested benefit of ursodiol may be reduction in risk of colon cancer and cholangiocarcinoma.
Rifampin is an antibiotic mostly used for the treatment of tuberculosis. However, because it activates receptors in the gut and liver, it has also been shown to help relieve pruritus (itching). Urso and Rifampin generally work well together. However, Rifampin may cause changes in other medications you are taking and alter the way they react in your system. Make sure your doctor is aware of all other medications you are taking along with Rifampin, in order to avoid any possible conflicts.
Cholestyramine is used to treat high cholesterol and also has been proven effective in combating pruritus (itching) due to bile blockages. Cholestyramine is not absorbed in the bloodstream; it remains inside the gut until excretion. Cholestyramine and Urso cannot be taken together. The general recommendation is to stagger doses of the two medications at least four hours apart.
PSC Partners Seeking a Cure believes that medical professionals should be the primary source of information for treating your PSC. Check with your doctor before making changes to your health routine or taking any alternative or herbal products.
It is important to remember that a product labeled as “natural” does not necessarily mean it is safe for the PSC patient. Supplements and herbs, despite being “natural,” can be toxic to the liver. The production and distribution of these supplements are not regulated as carefully as the production of prescription medications. In the U.S., the Food and Drug Administration does not test or regulate these products. “Natural” products can be sold with little testing and with no proof of efficacy. Sometimes the herb or supplement itself can cause liver damage. In other cases, impurities or toxins introduced during the preparation of the product may be toxic to the liver.
Common Symptoms of PSC
Although many patients report few, if any, symptoms, some of the symptoms of PSC include the following:
- Pruritus or intense itching: particularly on soles of hands or feet, though it can occur anywhere, including in eyes and mouth.
- Fatigue: Feeling run down, unable to get enough sleep, flu-like exhaustion that does not go away with sleep.
- Depression: Feelings of despair or hopelessness.
- Pain: Felt in the right side or in the middle of the abdomen towards the rib cage. This is often termed right upper-quadrant (RUQ) pain. The pain may extend to the shoulder blade area, may be of any intensity, and may last for an indefinite period of time.
- Jaundice: Yellowing of eyes and skin caused by excess bilirubin that the liver cannot process. May be accompanied by dark urine.
- Chills and fever: May be signs of a bacterial infection in the bile ducts, a condition called cholangitis. A cholangitis attack requires immediate medical attention and antibiotics.
PSC patients may report other symptoms, including variceal bleeding (spontaneous bleeding in the veins around the esophagus), ascites (fluid in the belly), hepatic encephalopathy ( “HE” or “mental fog”), night sweats, nausea, vitamin deficiency, stool changes, weight loss, clotting problems, and nail clubbing.
Vitamin deficiency and PSC
It’s recommended that patients with cholestatic liver diseases like primary biliary cirrhosis and primary sclerosing cholangitis get checked for fat-soluble vitamin deficiencies such as vitamin D and A. Vitamin supplements can be taken to compensate for deficiencies. But you have to be very careful not to over-dose.
There is information on credible websites (Mayo, WrongDiagnosis, NIDDK, ALF, etc.) that discusses PSC symptoms. The problem is that many of the symptoms listed are accompanied by others (especially if you have Crohn’s or ulcerative colitis), making it difficult to tell what’s going on.
Just as an example, let’s say I’m experiencing fatigue, stiffness/pain in my back and neck. It comes and goes, but seems to be getting worse. Did I just overdo some activity (like baling hay!), is it a PSC flare, or am I experiencing ankylosing spondylitis (one of the possible extra-intestinal complications of ulcerative colitis)? Or is it just age (please, no!)?
Also, it’s not uncommon at all for PSCers to have multiple autoimmune diseases, further complicating things (e.g., I have UC, PSC, asthma, eczema, arthritis).
Ascites and portal hypertension
Ascites (for people with liver disease) happens as a by-product of portal hypertension, (increased pressure in the vein system that carries blood from stomach, spleen & intestines to the liver) and cirrhosis. If you aren’t in stage 3 or 4 and don’t have cirrhosis, you most probably don’t have ascites.
Besides doing the wave test (actually rolling from one side to the other and watching the water move in waves) or pushing on one side of your stomach and checking if your belly moves in waves, you can check your albumin level. Normal is 3.5 – 5.0; if your albumin falls lower than 3.5 you can get ascites and edema. Albumin is a protein that keeps fluid/water in your blood, if the level gets too low, fluid leaks out of your blood and into your system: ascites. Without the lower than normal albumin, I don’t think you can get ascites and a lower albumin doesn’t happen until cirrhosis. At least that’s how it was explained to us.
Tips for travelers with PSC
All of your prescriptions should be in the original, labeled bottles. Consider asking your hepatologist for a recommendation of a hepatologist in the locale where you’ll be visiting. Take along contact information for your medical team as well as information on your medical history, perhaps a summary from a physician or related papers.
A medic alert bracelet or “dog tag” with pertinent data is recommended. Take along a copy or summary of your records. Depending on your condition, you may choose to travel only where advanced health care is readily available; or at least be able to arrange for fast transport to an area that does have reputable medical facilities (medical evacuation insurance). Visit www.medjetassist.com for more information.
You may also want to consider travelers insurance for expensive trips. Just do a Google search; there are many companies offering inexpensive travel insurance. Medical evacuation insurance is recommended for some parts of the world.
Note: Southwest Airlines offers a generous ticket refund policy: “Unused, fully refundable tickets that do not carry restrictions may be applied toward future travel or refunded within one year from the date of issue.” Thanks to PSCer Melanie S. for that information.
The Crohn’s and Colitis Foundation’s website has an excellent section on traveling with IBD, which would be useful to PSC patients. Click here to visit that site.
There are tips from the TSA on traveling and airport security. Click here for the website.