Frequently Asked Questions (FAQ)
- Can you recommend a short article describing Primary Sclerosing Cholangitis (PSC)?
- Can you recommend a good article describing what abnormal liver test results mean?
- Can you recommend an inspiring book?
We highly recommend Chris Klug's book: To the Edge and Back: My Story from Organ transplant Survivor to Olympic Snowboarder.
- How is PSC diagnosed?
- How do I join a PSC support group?
- How do I calculate a "Mayo Risk Score"?
- How do I calculate a "Model for End-Stage Liver Disease (MELD) Score"?
A MELD score calculator and associated documentation is available at the United Network for Organ Sharing (UNOS) website. This calculation is typically used for patients older that 12 years of age, and is employed for liver allocation. The numerical score (which ranges from 6 to 40) is based on total bilirubin, international normalized ratio (INR), and serum creatinine. It attempts to quantify a patient's risk of dying while waiting for a liver transplant. The higher the score, the higher the risk of death. Please also visit our Model for End-Stage Liver Disease (MELD) Score Calculator to view the formula used to calculate MELD. An alternative calculation called PELD (Pediatric End-Stage Liver Disease) is used for patients younger than 12 and is based on bilirubin, INR, albumin, growth failure, and age when listed for transplant.
- How do I calculate my medication dose in terms of mg per kg per day (mg/kg/d)?
To calculate your medication dose in terms of mg per kg per day (mg/kg/d) please visit our Medication Dose Calculator web page. You will need to know your weight in pounds (lb), and your daily dose of medication in mg.
- Is there a genetic basis to PSC?
Recent research suggests that there may be a genetic component to PSC. Please see these articles for discussions of known genetic risk factors:
Karlsen TH, Franke A, Melum E, Kaser A, Hov JR, Balschun T, Lie BA, Bergquist A, Schramm C, Weismuller TJ, Gotthardt D, Rust C, Philipp EE, Fritz T, Henckaerts L, Weersma R, Stokkers P, Ponsioen CY, Wijmenga C, Sterneck M, Nothnagel M, et al. Genome-wide association analysis in primary sclerosing cholangitis. Gastroenterology 138: 1102-1111 (2010).
Karlsen TH, Schrumpf E, Boberg KM. Genetic epidemiology of primary sclerosing cholangitis. World J. Gastroenterol. 13: 5421-5431 (2007).
The latter paper notes that major genetic risk factor(s) for PSC may reside within the AH8.1 ancestral haplotype of the major histocompatibility complex. Thus, "A HLA association in PSC was first identified for HLA-B8 (i.e. HLA-B*0801) and DR3 (i.e. DRB1*0301)[24,50]. Later studies have verified that PSC associations exist also for the other alleles of the AH8.1 (the HLA-A1 allele, the HLA-C7 allele, the major histocompatibility complex class I chain-related A (MICA) *008/5.1 allele[53,54], and the tumour necrosis factor alpha (TNFalpha) promoter -308 A allele[55,56]). This haplotype is associated with a wide range of autoimmune diseases[57,58]." (Karlsen et al., 2007).
- Where can I find recent guidelines for the diagnosis and management of PSC?
- What is a cholangitis attack, and what are the symptoms?
Cholangitis is an inflammation of the bile duct system that is usually related to a bacterial infection. It may occur as a result of blockage and/or inflammation of the bile ducts. Bile is normally sterile but can become infected with bacteria when obstruction of the bile ducts occurs. Symptoms of cholangitis can include any combination of the following:
If a fever and chills occur, then it is likely that a bacterial infection is present, and medical attention should be sought immediately. Antibiotics can be administered to control the infection. For further information on this potentially life-threatening complication of primary sclerosing cholangitis, please consult the following articles:
- pain in the right upper quarter of the abdomen (pain may also occur in the chest, in the upper back, or the right shoulder)
- tenderness in the upper abdomen
- nausea and vomiting
- jaundice - yellowing of the skin and eyes
- low blood pressure
- decreased level of alertness
- pale stools
- dark urine
- What is pruritus?
Pruritus means the desire to itch. Intense itching is often associated with primary sclerosing cholangitis and other cholestatic liver diseases. The Cleveland Clinic has published an excellent article entitled "Pruritus" which describes the various types and causes of itching. A comprehensive article on pruritus is as follows: "Pruritus" (American Family Physician). The latter article describes some of the medications used to treat pruritus associated with cholestasis. These medications include:
- What are some of the common medications used to treat PSC?
- What is ursodiol?
Ursodiol (also known as ursodeoxycholic acid, and sometimes abbreviated "URSO" or "UDCA") is a bile acid originally identified in bear bile. The name originates from the name of the bear family, Ursidae. Ursodiol was first used in Western medicine to dissolve cholesterol gallstones, but has more recently been used to treat a number of cholestatic liver diseases, including primary biliary cirrhosis (PBC), and liver disease associated with cystic fibrosis. It is thought to protect liver cells against certain toxic bile acids that accumulate in the liver in chronic cholestatic liver diseases. In PBC, ursodiol treatment has been shown to improve liver biochemistry and delay disease progression and time to liver transplantation. In primary sclerosing cholangitis (PSC), high-dose ursodiol has been shown to improve liver biochemistry, but there are conflicting reports on whether it delays disease progression and prolongs time to liver transplantation. There is growing evidence that ursodiol may protect against colon cancer and cholangiocarcinoma. Trade names for ursodiol include "Actigall" (typically available as 300 mg capsules) and "URSO" (typically available as 250 mg tablets) or "URSO-Forte" (available as 500 mg tablets). Side effects of this medication are described in an article from MedSafe: "Actigall: Ursodiol (Ursodeoxycholic acid)". Please note that in some patients ursodiol can increase itching! For a detailed article on ursodiol, please consult: "Ursodiol for Hepatobiliary Disorders" (Annals of Internal Medicine).
- Where can I find information on how to let family and friends donate funds to promote research, education and support for love-ones afflicted with PSC?
PSC Partners Seeking a Cure (www.pscpartners.org)
Don and Ricky Safer
Ricky Safer e-mail: firstname.lastname@example.org
Last Update: 09/27/10