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The rare disease responsible for the bacterial infections that claimed the life of 19-month-old Bilqis Anindya Passa last Saturday is all too familiar to a doctor who says hundreds of babies are born with the life-threatening biliary atresia in Indonesia every year — all of them with little chance of survival because of late detection.

“Every year hundreds of babies are born with biliary atresia here and many of them eventually die,” Sjamsul Arief, head of the pediatric hepatology division at Dr. Soetomo Hospital in Surabaya, told the Jakarta Globe on Tuesday, adding that two babies died at the hospital last week due to the same condition.

Biliary atresia is a condition where the bile duct between the liver and the small intestine is blocked or absent. If untreated, the bile stays in the liver, leading to failure of the organ.

Sjamsul said that unlike Bilqis, who received over Rp 1.5 million ($167,000) from donations made through a highly-publicized Facebook donation page before the government stepped in and announced it would cover her medical bills, the majority of biliary atresia cases receive little attention.

A 10-month-old baby from Gorontalo, Ismail Daud, died last Thursday from biliary atresia. And Tempointeraktif.com recently reported that 21-month-old Eka Putra Prasetya from Clumprit village in Malang, East Java, has also been diagnosed with biliary atresia. Eka is still alive, but his father Sholihin, who works as a crew member on a ship, cannot afford the treatments at Dr. Soetomo Hospital.

Muhammad Alfan, a spokesman at Dr. Kariadi Hospital in Semarang, where Bilqis died, said a team of doctors there was now treating another biliary atresia case in a 4-year-old from Medan, North Sumatra. “She was detected with this condition when she was two months old,” he said.

It is unclear what causes biliary atresia, Sjamsul said. Usually it develops when the baby is still in the womb, but in some cases biliary atresia develops when the bile duct becomes infected during a child’s growth. The resulting inflammation could cause a blockage, preventing bile from draining away into the small intestine.

“In the worst cases, this condition often causes cirrhosis in children,” he said.

If detected early, biliary atresia can sometimes be cured with the surgical Kasai procedure which creates a channel that connects the bile duct to the intestine. However, Sjamsul said, it could only be performed on a baby less than two months old and had a 20 percent success rate.

“If the baby is older than that, cirrhosis usually has happened and a liver transplant is the only option,” he said.

Even with the liver transplant, Sjamsul said, there was only a 20 percent chance that the baby would live normally, with most transplant recipients having to undergo lifelong treatment with immunity medications to keep the body from rejecting the liver. He said that the treatment could affect a child’s normal growth.

“Without the medication, many children have to undergo a re-transplantation procedure because their bodies are rejecting the new liver,” he said.

AG Soemantri, senior hepatologist at Dr. Kariadi Hospital, said patients with biliary atresia are highly susceptible to infection from three dangerous germs: klebsiella pneumonia and acinetobacter baumannii bacteria which attack the lungs, and serratia marcescens bacteria that attacks the blood. “Forty percent of patients die of these infections,” he said. Bilqis died after an attack by all three.

Sjamsul warned parents to be extra cautious if their babies were born with a yellowish hue. He said that many parents, not understanding the warning signs, tried to treat what they thought was only a skin condition by exposing their babies to the morning sun.

He said the liver was the center of all metabolic activity, and that liver dysfunction could seriously affect a child’s growth. He stressed that parents must be extra cautious if their babies started to develop swollen stomachs because it could be a symptom of advanced biliary atresia.

 

Additional reporting by Candra Malik from Semarang, Central Java