Learn More About Biliary Atresia
Biliary Atresia is the leading cause of liver transplantation in children as the majority of those diagnosed end up needing a liver transplant. They will be in and out of hospitals all their lives having operations, procedures, tests and scans to monitor their disease and ultimately keep them alive.
In a Nutshell
Biliary Atresia is a rare, life-long & life threatening disease of the liver that affects about 1:16,000 newborns. The cause is unknown, not hereditary, and there is no cure. Treatments are only partially successful and often requires liver transplant.
The infants must undergo a major life-saving operation called the Kasai Procedure shortly after diagnosis, but the procedure often fails. Therefore, the majority of those children requires a liver transplant in order to survive.
Biliary Atresia Awareness & Research (BAAR) is a Non-Profit Organization founded in February 2012, and we aim to accomplish the following goals:
Increasing awareness of the symptoms by providing information to front-line health professionals such as doctors, midwives, health visitors as well as new parents, mom & tot groups or schools. The sooner a diagnosis is made, the more chance of a successful outcome, reducing the loss of infant lives.
Reducing the stigma and promoting organ donation. More children are now surviving into adulthood before requiring a transplant. Therefore, increased understanding from the general public is needed to recognize that not all cases of liver disease and liver transplantation are the result of lifestyle choices.
Initially, the proceeds will be used to further raise awareness. Excess profits from the awareness activities will be forwarded to the appropriate research centres in discussion with the BA specialist at BC Children’s Hospital, for the furthering of research into the cause and cure.
What is the symptoms of Biliary Atresia?
The first signs or symptoms of Biliary Atresia is jaundice, which causes a yellow color to the skin and to the whites of the eyes. Jaundice is caused by the liver not removing bilirubin, a yellow pigment from the blood. Ordinarily, bilirubin is taken up by the liver and released into the bile. However, blockage of the bile ducts causes bilirubin and other elements of bile to build up in the blood.
What causes Biliary Atresia?
The cause of Biliary Atresia is not known. The two types of Biliary Atresia appear to be a “fetal” form, which arises during fetal life and is present at the time of birth, and a “perinatal” form, which is more typical and does not become evident until the second to fourth week of life. Some children, particularly those with the fetal form of Biliary Atresia, often have other birth defects in the heart, spleen, or intestines.
What are the teatments for Biliary Atresia?
Kasai Procedure (hepato-portoenterostomy). If Biliary Atresia is the diagnosis, the surgeon generally goes ahead and performs an operation called the “Kasai procedure,” named after the Japanese surgeon who developed this operation. In the Kasai procedure, the bile ducts are removed and a loop of intestine is brought up to replace the bile ducts and drain the liver. As a result, bile flows from the small bile ducts straight into the intestine, bypassing the need for the larger bile ducts completely. (More about the Kasai procedure follows.)Liver transplant. If the Kasai procedure is not successful, the infant usually will need a liver transplant within the first 1 to 2 years of life. Children with the fetal form of biliary atresia are more likely to need liver transplants—and usually sooner—than infants with the typical perinatal form. The pattern of the bile ducts affected and the extent of damage can also influence how soon a child will need a liver transplant.