The Liver
The liver is the largest organ in the body. It is found high in the right upper abdomen, behind the ribs. It is a very complex organ and has many functions. They include:

•Storing energy in the form of sugar (glucose)
•Storing vitamins, iron, and other minerals
•Making proteins, including blood clotting factors, to keep the body healthy and help it grow
•Processing worn out red blood cells
•Making bile which is needed for food digestion
•Metabolising or breaking down many medications and alcohol
•Killing germs that enter the body through the intestine

The liver cells excrete bile into tiny tubes within the liver called bile ducts. These tubes come together like the tiny veins on a leaf. They drain the bile into the common bile duct, a larger single tube leading into the intestine. There the bile aids digestion and gives stool its brown color. The liver is a very important organ.

Treatment
At the present time there is no cure for PSC, but tablets for management of the symptoms is available. There are a number of ways to treat symptoms and the various stages of the disease. Itching, from too much bile in the bloodstream, can be controlled with drugs such as Questran. Bile is usually reabsorbed into the bloodstream from the large intestine, and goes back to the liver to be reused. Questran binds up bile in the intestine, allowing it to be eliminated with the stool instead. This helps to reduce the build-up of bile in the body. Actigall is a drug that favorably changes the make-up of bile in the liver. This, in turn, seems to reduce the amount of liver damage that occurs. Results of early medical studies seem to indicate that this drug may increase survival. Sometimes the bile ducts become infected and must be treated with antibiotics. If ulcerative colitis is also present, it is treated with the appropriate medicines. Swelling of the abdomen and feet, due to fluid retention from cirrhosis, can be treated with a salt-restricted diet and diuretics (fluid pills).

In some cases, endoscopic or surgical procedures may be used to open major blockages in bile ducts. Through an endoscope, the consultant places a tiny tube with a balloon on the end into the narrowed bile duct. The balloon is inflated to expand the duct so bile can flow through it once again. Sometimes stents (plastic tubing) can be placed in the narrowed ducts to keep them open. Often PSC progresses to a point where liver transplantation must be considered.

Although att present there is no known cure or specific treatment for PSC preliminary trials have suggested that the natural bile acid, ursodeoxycholic acid, may slow the progression of the disease possibly by increasing bile flow and reducing liver inflammation.

Symptoms such as itching can be treated with agents such as cholestyramine, rifampicin or naltrexone.Cholestyramine ( trade name Questran or Questran Light) may be prescribed to help relieve itching. It works better when taken with meals, especially breakfast, but it may take some days before the treatment is effective. Some people taking Questran experience side effects such as altered bowel habits or bloating. Keep your doctor informed - there are other treatments that may suit you better.

Many people suffer with a dry mouth and dry eyes, but this can be helped by taking lozenges and artificial tears prescribed by your doctor.
For a few people who eventually go on to get advanced cirrhosis, a liver transplant may be recommended when their quality of life has deteriorated and medical treatment can no longer control their symptoms. Because PSC usually develops slowly, transplantation can be carefully planned well ahead.

People with advanced PSC are often deficient in vitamins A, D and K and replacement fat-soluble vitamins are given. Endoscopic treatment of the bile ducts is aimed at reducing or halting the progression of the disease, usually by stretching or dilating the narrowed bile ducts. In later stages of the disease liver transplantation is often an option when their quality of life has deteriorated and medical treatment can no longer control their symptoms. The survival after liver transplantation is good, although in some patients PSC can recur in the new liver.