primary sclerosing cholangitis psc

WHAT IS PRIMARY SCLEROSING CHOLANGITIS (PSC)?

Primary Sclerosing Cholangitis (PSC) is a somewhat rare, progressive liver disease where the bile ducts become inflamed and scarred.

In a healthy functioning liver, bile ducts carry a liquid produced by the liver called bile, to the intestines where it helps digest food. When a child is suffering from PCS, the bile ducts become narrow. This stops the smooth flow of bile out of the liver. This abnormal flow of bile and its subsequent buildup in the liver can cause chronic liver issues and over time, possibly result in serious liver damage that can lead to cirrhosis liver failure. In such cases, a child suffering from PSC may need a liver transplant.

The Importance of the liver in the human body:

The liver is an extremely important organ in the body. It produces proteins that allow blood to clot normally, transport oxygen and support the immune system. It produces a substance called bile that help in the digestion of food. It also stores extra nutrients that are used by the body from time to time. Moreover, it helps clean the bloodstream and also helps control blood sugar and cholesterol levels.

WHAT ARE THE SYMPTOMS OF PRIMARY SCLEROSING CHOLANGITIS (PSC)?

The symptoms of PSC can go unnoticed for a long period of time; however, they could include:

• This is the most common symptom of PSC.
• Itchiness on the skin
• Unexpected weight loss
• Abdominal pain
• Unexplained fever

Over time, the condition progresses to show symptoms of liver failure, such as:

• Jaundice
• Pain in the upper right abdomen
• Weakness
• Dark urine
• Confusion or disorientation
• Easy bleeding and bruising

WHAT ARE THE CAUSES OF PRIMARY SCLEROSING CHOLANGITIS (PSC)?

Doctors, researchers and scientists have still not been able to determine the exact cause of PSC. Some researchers believe it to be an autoimmune disorder (a disorder that causes the body’s immune system to attack the healthy tissues), however, it hasn’t been proven yet.

Male children with a family history of PSC and autoimmune disease are believed to at a higher risk of developing PSC.

WHAT ARE THE SIGNS AND SYMPTOMS OF PRIMARY SCLEROSING CHOLANGITIS (PSC)?

In most cases, a child may have PSC for years before he/ she develops any symptoms. If the disease is mild, there might not be any visible symptoms, however, if the disease is an advanced condition, the child may develop the following symptoms:

• Jaundice
• Itching
• Dark colored urine and pale stools – usually happens if the bile ducts are blocked.
• Tiredness and reduced energy levels.
• Loss of appetite resulting in weight loss
• Abdominal pain
• And in some rare cases, high fever.

HOW IS PRIMARY SCLEROSING CHOLANGITIS (PSC) DIAGNOSED?

The first steps towards the diagnosis of PSC in children is a physical exam and examination of medical history. The objective of a physical exam is to look for signs of liver damage and bile duct infection. While looking at the child’s medical history, the pediatric gastroenterologist will ask whether the child has had any symptoms, such as fatigue, itchiness, unexplained weight loss, or abdominal pain. This information will help the gastroenterologist assess the likelihood of a child having PSC. The doctor would also enquire whether any family member has had a history of PSC and whether the child has had inflammatory bowel disease or autoimmune disease.

If the results of the physical exam and medical history indicate the likelihood of PSC, the following tests will help the doctor confirm the diagnosis:

• Blood Tests:
  Liver blood tests over many days would be carried out to show a pattern that indicates damage of the bile ducts. These blood tests are suggestive and not diagnostic. The liver blood test will indicate whether the liver is working normally or not.
• Endoscopic Retrograde Cholangiopancreatography: Endoscopic Retrograde Cholangiopancreatography is a type of imaging test where the doctor uses endoscopy to inject dye into the bile ducts. This dye makes the bile ducts visible on an x-ray that is taken with a tiny camera at the end of the endoscope. The pediatric gastroenterologist examines the image for signs of blocked ducts. The procedure is typically carried out under the effect of a sedative to help keep the child calm.
• Percutaneous Transhepatic Cholangiography: Percutaneous Transhepatic Cholangiography is another imaging test that uses dye to make the blie ducts visible on an x-ray. The difference here is that the dye is injected into the abdomen through the skin of the stomach and the x-ray taken with a camera on the outside of the body.
• Magnetic Resonant Cholangio-pancreatogram (MRCP Scan): An MRCP Scan is a specialist MRI (Magnetic Resonance Imaging) scan of the bile ducts to show changes in the bile ducts and the damage to the bile ducts. An MRCP scan is better at diagnosing PSC.
• Liver Biopsy: If the diagnosis of PSC from the above tests is not clear, the pediatric gastroenterologist may ask for a liver biopsy that will confirm the scarring around the bile ducts and also confirm bile duct damage. The biopsy will also confirm the extent of the scarring in the liver.

WHAT, YOU AS A MOTHER, SHOULD KNOW ABOUT PRIMARY SCLEROSING CHOLANGITIS (PSC)?

PSC is a chronic condition for which there is currently no cure. The modality of treatment for PSC is supportive and is focused on managing the symptoms, improving the bile flow and the consistency of bile in the bile ducts.

Further, you should immediately contact your child’s pediatrician or pediatric gastroenterologist if your child complains of severe itching that persists. And also, when you observe your child being extremely tired for most of the time in a day. The unexplained itching and fatigue must be immediately discussed with the doctor as this could be an indication of either ulcerative colitis or Crohn’s disease.

WHAT ARE THE POSSIBLE COMPLICATIONS OF PSC?

The following are the possible complications of PSC:

• Liver Disease & Failure
  Chronic inflammation of the bile ducts in the liver can eventually lead to tissue scarring (cirrhosis), liver cell death and even loss of liver function.
• Frequent infections: The scarring of the bile ducts or blockage of the flow of bile out of the liver may lead to frequent infections in the bile ducts.
• Portal Hypertension: The portal vein is the major route for the flow of blood from the digestive system into the liver and high blood pressure in this vein is referred to as portal hypertension. It can cause the liquid from the liver to leak into the abdominal cavity (ascites), or it can diver blood from the portal vein into other veins resulting into the swelling of those veins (varices). Varices are weak veins and can bleed easily which could be life-threatening.
• Osteoporosis (thinning bones)
• Cancer of the bile duct: Children suffering from PSC are at an increased risk of developing cancer of the bile ducts or gallbladder.
• Cancer of the colon: Children suffering with PSC associated with inflammatory bowel disease are at an increased risk of colon cancer.

HOW IS PRIMARY SCLEROSING CHOLANGITIS (PSC) MANAGED?

While there is no cure for PSC, there are however ways to treat affected bile ducts and treatments to relieve symptoms.

Treatment for Narrow Bile Ducts:

Endoscopic Retrograde Cholangiopancreatography is done to open blocked bile ducts to allow the bile to travel more easily and not get accumulated in the liver.

Treatments to relieve symptoms:
The pediatric gastroenterologist may prescribe certain medications to relieve other symptoms of PSC, such as itching or antibiotics to treat the bacterial infection of the bile ducts.

Liver Transplant:

Many children with PSC might eventually need a liver transplant, however, most pediatric gastroenterologist would recommend a liver transplant only after other treatment or management options have been tried. While the time frame differs, a child with PSC will typically need a liver transplant 20 to 25 years after the first diagnosis.

WHAT ARE SOME EVERYDAY HABITS THAT HELP KEEP THE LIVER HEALTHY?

For children with PSC the following habits can help keep the liver healthy for longer:

• Eat a healthy diet
• Maintain a healthy weight
• Get vaccination for hepatitis A & B
• Take no more than the recommended dosage of any OTC or prescription medication