Primary Sclerosing Cholangitis (PSC)

Digestive system diagram showing the bile duct

The image above is an intraoperative cholangiogram, which is an X-Ray of the bile ducts during a laprascopic cholecystectomy. Sandy has had several of these procedures for diagnostic purposes as well as to allow doctors to deploy stents in the ducts. Stents are temporary devices to used help relieve constricted areas in the ducts and restore normal flow.

Sandy was diagnosed with PSC in September of 2005, shortly after the initial onset of symptoms.

Primary sclerosing cholangitis (PSC) is a rare, chronic disorder of the liver in which the bile ducts outside the liver (extrahepatic bile ducts) and/or inside the liver (intrahepatic bile ducts) become inflamed, thickened (sclerotic), narrowed, and finally obstructed. This is a progressive process that can in time destroy the bile ducts and the liver.

Primary sclerosing cholangitis is also commonly called idiopathic sclerosing cholangitis ("idiopathic" means the cause is unknown) or just sclerosing cholangitis. Sclerosing means "scarring," and cholangitis comes from cholangio, referencing the biliary region. Collectively, the ducts make up the biliary tree.

What causes primary sclerosing cholangitis?
The cause of sclerosing cholangitis is not known, but multiple theories exist. One such theory is that PSC is an autoimmune disorder, and we have found this to be credible in our experience with Sandy. Some believe that PSC may be a pre-cancerous condition. This would seem to be the case with Sandy, as well.

What are the symptoms of primary sclerosing cholangitis?
PSC often triggers jaundice (yellowing), generalized pruritus (itching all over the body), upper abdominal pain, and infection.

As PSC progresses, the disease causes cirrhosis of the liver (irreversible scarring of the liver) and liver failure, leading to the consideration of liver transplantation. PSC is, in fact, one of the more common reasons for a liver transplant.

Portions of this description were borrowed from answers.com and wikipedia