Tuesday, September 30, 2008

What is Biliary Atresia ?

What is biliary atresia?

Biliary atresia is caused when the bile duct to the liver becomes inflamed. This is normally due to damage caused around the time of birth. It is not clear how or why this occurs.
The bile duct becomes blocked and means bile builds up in the liver and begins to poison it.
The child will develop jaundice. Other symptoms include yellow-coloured urine and pale stools.
Many children suffer jaundice in infancy, but concerns are usually linked to children who have the condition for longer than two weeks.
Doctors warn that the earlier it is caught, the less damage it will have done to the liver.
Operations to cure the condition are most successful in children under eight weeks old.

How is the condition diagnosed?

A battery of tests are often required to confirm a diagnosis of biliary atresia, as the symptoms can be confused with other conditions.
These can include:
A blood test to look for raised levels of bilirubin
An abdominal x-ray to look for an enlarged liver and spleen
Abdominal ultrasound to examine the liver and bile ducts
An HIDA scan to determine how well bile is flowing
A liver biopsy to determine degree of cirrhosis or to rule out other causes of jaundice
If the test results suggest biliary atresia, an operation is performed to examine the bile ducts and an x-ray is taken during the operation to determine whether the bile duct is blocked.

How is biliary atresia treated?

The condition is treated through an operation called Kasai-portoenterostomy.
This involves using a loop of bowel to form a duct to drain the bile from the liver.
The operation is named after the Japanese surgeon, Professor Morio Kasai, who developed it in 1959.
It was first introduced in the UK in the 1960s.
The operation is extremely intricate and its success depends on the skill of the surgeon, whether he or she carries out the operations fairly regularly and the support available to the child before and after surgery.

Operations can be carried out on children as young as 11 days old.
If surgery fails, the child's only other option is a liver transplant, which is a traumatic operation with varying degrees of success.

According to the Children's Liver Disease Foundation, the operation costs for a Kasai if around 50,000/- INR to 80,000/- INR and A liver transplant costs Rs.15 lacs plus the cost of anti-rejection drugs.
There are many centres who have experts in performing Kasai operations but it is recommended that you choose a center which also have liver transplant capability. In north India you have Sir Ganga Ram Hopsital (www.livertransplantindia.com) and Apollo Hospital (www.transplantliverindia.com)

This page contains basic information about biliary atresia. For more information, consult your doctor. All information on this site needs validation and is from my own experience,pls speak with your Doctor for treatment & guidance.

RELATED INTERNET LINKS
Children's Liver Disease Foundation
Biliary atresia
Biliary atresia and liver transplants

Biliary Atresia - Biliary Tree / Liver Disorder: Biliary Atresia - Defect in Children

Biliary Atresia - Biliary Tree / Liver Disorder: Biliary Atresia - Defect in Children

Monday, September 29, 2008

Biliary Atresia - Defect in Children




I am a Father who has just found out that my son Prajas (picture on left) who is 2.5 months old is having billiary atreisa. I had so far only heard of AIDS, Cancer etc. as rare diseases and have been caught completely by surprise. This disease cause the bile not to follow from the liver to the intestine thus damaging the liver. Early detection is the key and you need to have a good paed.. I have learnt this from experience, my doc could have cleary diagonsed this early but he choose not to and here we are. My son has gone through the Kasai Surgery on Sep 17, 2008 and still here is no bile flow, surgeon/doc told us about this beforehand . His abdomen has increased to 50 cm yesterday and needed aspiration. We have met up with all transplant experts and they say that the baby should in good health free of infections for a sucessful liver transplant. We are praying to god that is well. Tomorrow Sep 29, 2008 doctor has planned for CT Scan to chk where are the fluid leaking from and if any leakage then a surgery would be done again. Pls share your experience if you have know of this rare disease. In India there is limited information and I would really appreciate inputs here. Ultimately Prajas (picture on left) will need a transplant but we have to get him ready for the same. Yours prayers can work, so please do pray for him..God Bless