The Sunday Mail
. . . what is biliary atresia?
Many would not know what biliary atresia is and there might be several others facing the same fate but with little, if any, knowledge of what the disease is. Biliary atresia is a life-threatening condition in infants in which the bile ducts inside or outside the liver do not have normal openings. Bile ducts in the liver, also known as hepatic ducts, are tubes that carry bile from the liver to the gall bladder for storage and to the small intestine for future use in digestion.
With biliary atresia, bile is trapped and by so doing damages the liver, which leads to scarring, loss of liver tissue and cirrhosis. Cirrhosis, which is synonymous with heavy alcoholics, is a chronic liver condition resulting from scar tissue and cell damage that makes it hard for the liver to remove toxins from the blood stream.
The result is that the toxins build up and slowly impair the liver and without treatment, the liver eventually fails thus necessitating a liver transplant.
The condition is very rare and affects about one out of 18 000 to 20 000 infants and it is more common in females than in males. Causation is multiple though not scientifically proven.
Biliary atresia is caused by an event in the womb or around the time of birth, largely triggered by immune system problems such as when the immune system attacks the liver or bile ducts for unknown reasons, a genetic mutation which might be a permanent change in the gene’s structure or a problem during liver and bile duct development in the womb.
First symptom is jaundice — that is when the whites of the eye turn yellow. This is mainly because of the liver’s failure to remove bilirubin (the end product of haemoglobin when it breaks down). Bilirubin is absorbed by the liver, processed and released in bile but now with blocked bile ducts, it builds up in the blood causing dark urine, gray or white stools and marked stunted growth.
No single test can diagnose biliary atresia and as such, a series of tests are required. Blood can be collected to check for liver damage, abdominal x-rays, liver scans, liver biopsy and diagnostic surgery can all be done. Biliary atresia can be treated with surgery called the Kasai procedure or a liver transplant.
The Kasai procedure — named after the surgeon who first performed it — is when the surgeon removes the damaged bile ducts and brings up a loop of intestine to replace them, resulting in bile flowing straight to the small intestine. This can restore bile flow, correcting problems caused by biliary atresia.
However, liver transplantation is the definitive treatment for this condition. Survival after surgery has increased dramatically and infants with biliary atresia can now survive. New methods now make it possible to transplant a portion of an adult’s liver into an infant and this commonly known as the reduced-size or split-liver transplant.
Healthy liver tissue grows quickly and after transplant, a regimen of medication is used to prevent the immune system from collapsing. A high calorie diet is required because biliary atresia leads to a faster metabolism. Vitamin supplements are needed because medication used to keep the body from rejecting the new liver can affect calcium and magnesium levels. Forward Nyanyiwa is a medical practitioner at Chitungwiza Central Hospital and writes in his personal capacity
