【The story of coordinator】Pediatric specialty hospital “National Center for Child Health and Development Hospital (NCCHD)”　Living donor liver transplant was performed for congenital biliary atresia, and the baby has gotten better

Parents decided that the only way for their baby to survive is to receive treatment in Japan

The baby became weaker and weaker due to poor prognosis after surgery

EAJ received a call from parents from Europe about their baby just at the age of 4 months suffering from congenital biliary atresia. The baby was diagnosed with congenital biliary atresia at the age of two months in an Asian country where his father was working. The baby underwent the Kasai procedure, which directly connects the liver and intestines. However, after the surgery, the baby became infected with cytomegalovirus, and even after treatment, the baby became weaker and weaker by the day and hardly drank milk. In addition, the parents had difficulty communicating with the local hospital’s doctors regarding the treatment plan, leading to conflicts with the doctors, and their distrust grew suspecting that the necessary measures were not being taken. As its result, the parents decided to discontinue treatment in this country and they contacted EAJ because they were considering receiving proper medical treatment in Japan and considering a liver transplant if necessary.

EAJ responded that living donor liver transplantation is possible in Japan

EAJ contacted Dr. Kasahara (current director of the hospital) of the Organ Transplant Center at the National Center for Child Health and Development(NCCHD), which is Japan’s largest children’s hospital and has a track record of performing more than half of the liver transplants for children in Japan. When EAJ informed the doctor about the baby’s medical condition, fortunately the baby had reached a weight that would allow a transplant. So EAJ decided to request detailed information about the liver function of both the baby and the parents who were potential donors. After examining the information sent by the parents, it was determined that if the conditions were right, a liver transplant would be possible. Since the baby’s liver was small, it was better for the donor liver to be smaller as well, so the petite mother was the first candidate to be the donor. Transplants are performed on a certain day every week, and it takes two weeks for both the baby and the donor to be examined, so it was decided that the baby and the parents would come to the hospital two weeks before the desired surgery date.

“We will bring your kid back home in good health”

The parents decided to come to Japan and take the test. Although there was no change in the parents’ decision to come to Japan as the extremely dangerous situation was continuing, they still had some questions. Patients with biliary atresia usually have white stools, but the stool was a normal color. So, the parents wondered if the diagnosis was correct in the first place, and normally, the transplant is supposed to be done after the child has grown up to about 5 years old, but why the transplant was done so soon? The doctor carefully explained that he would not recommend a transplant until the diagnosis was confirmed through tests and the necessity of the transplant was confirmed, along with a message telling, “We will bring your kid back home in good health.” This short message has alleviated the anxiety of parents.

Preparing to come to Japan on the assumption that transplant would be performed

The parents also prepared and transferred money for the transplant, and EAJ prepared a long-term visa for them with the assumption that the transplant would be performed. The parents will be staying at McDonald House, a facility adjacent to the hospital where families of children undergoing medical treatment can stay. While preparing to come to Japan, the parents shared the current test results with Japanese doctors. But it became clear that it would be difficult to wait until he was 5 years old for a transplant because the values indicating liver dysfunction were increasing. Although the decision to transplant would be made after arriving in Japan, it was not possible to transplant suddenly. So, everyone involved was prepared in case transplant became indispensable. Preparations have progressed, including obtaining a birth certificate from the donor’s home country that shows the relationship with the donor and confirming religious considerations.

Partial transplantation of mother’s liver

Arrived in Japan within 3 weeks from inquiry

Preparations progressed at a rapid pace, and just three weeks after we EAJ was first contacted, the parents and child came to Japan as a family. After conducting tests and communicating with the local doctor who performed the surgery, Dr. Kasahara decided that it was necessary to perform transplant instead to wait until the baby grew larger. However, the transplant itself was scheduled to be performed about a month and a half later than planned, so the baby had to be discharged from the hospital. As the baby needed special milk, EAJ arranged for it to be delivered to the place of stay. The baby developed a fever the day before the surgery, and if the cause is due to the bile duct, the doctor can perform the transplant as planned, but if it is due to another illness such as pneumonia, the doctor will have to postpone the transplant. EAJ watched the situation in suspense until just before the transplant.

The transplant was successful and progress was good

One and a half months after arriving in Japan, the transplant surgery was performed. The mother was admitted to the hospital the day before, and the surgery started the next morning. The doctor removed a quarter of the mother’s liver, then cuts it in half again and transplants it into the child’s liver. At the same time, the mother’s intestinal blood vessels were also transplanted. The surgery lasted 10 hours, starting at 9 a.m. in the operating room and ending at 7 p.m. During the surgery, the father looked at the baby’s liver, which had been removed, and found that part of it was uneven due to cirrhosis. The surgery was successful, and after the surgery, the doctor informed the parents that the short intestine that had been connected in the previous Kasai procedure had been reconnected with a longer one. The parents were also told that the most critical period for a transplant is the first week after surgery, when there is a risk of rejection, and that once this period is overcome, the risk will be reduced.

The day after the surgery, the father was able to see the baby awake and the mother was able to get up from bed and walk. The mother was discharged from the hospital after a week, and the baby was cared in the ICU. Two weeks after the transplant, ascites and pleural effusion had almost disappeared, and liver function was nearing normal. At the doctor’s examination, the parents and all of EAJ people involved were relieved to hear that the baby would be able to move from the ICU to the general ward in a few days.

The mother and child wanted to stay in Japan for about a year to see how things would be going, so EAJ assisted them with administrative procedures such as changing their status of residence. A week after returning to the general ward, the concentration of the intravenous drip was diluted, the amount of milk was increased, and the baby was able to remove the oxygen mask during the day. The baby’s muscles had weakened from lying down on the bed all the time, so a physical therapist came to the baby and started rehabilitation by playing with toys and light physical exercise. Gradually, the baby was able to move his arms and neck freely and was able to sit again, and the interpreter reported that the baby was doing well every time she went to the hospital.

Discharged about 2 months after surgery

It was decided that the baby would be discharged from the hospital 2 months and 10 days after the transplant surgery. After being discharged from the hospital, blood tests and ultrasound tests will be performed once every two weeks to monitor progress of recovery. It was a little while later, the parents told the doctor that there were not many doctors in their hometown who were familiar with liver transplants after the parent returned their home country. So, the parents asked the doctor to write a detailed explanation of the treatment and medicines to be used in case the medical condition of their baby worsens, and EAJ translated it during the discharge procedure. After being discharged from the hospital, the patient visits the outpatient clinic about once a month to receive prescriptions for immunosuppressive drugs and monitor progress of recovery. When discharged from the hospital, the baby was prescribed 10 types of medication, and though the types and doses are gradually reduced, the last immunosuppressant medication must be continued to be taken at a certain time even after he goes to school. While the parents were advised to avoid crowds for six months, they were also told that if they raise their child like normal children and do not let their child stay at home too much, the child will grow up to be strong. After the mother and the baby were discharged from the hospital, they had to stay in Japan for a long time. So, EAJ introduced a babysitting company to the mother because she was alone, and EAJ also helped her with interpreting when she needed to go to a clinic in case she felt unwell during her daily life, and helped her with shopping, continued to provide support such as assistance with administrative procedure. Since the mother will be staying in Japan for a while, she has been very proactive about caring for her child in Japan, including purchasing Japanese language learning materials and starting to study.

For and after returning to home country

Recovered enough to be able to run around

Six months after the surgery, the baby was able to gradually reduce his medication use. Since the mother could not stay in Japan forever, she said, “I want to get sick at least once while I’m in Japan,” which impressed us particularly. In fact, the mother and child were infected with various diseases and were scratched by dogs several times after that, and each time they learned how to deal with it. In the summer, the child and mother attended swimming lessons and seemed to be recovering well.

One year after coming to Japan, the staff heard that the baby, who was weak when they first met, was already standing up and running around, and asked the mother to take a photo. The mother sent EAJ a photo of her baby running away with a cell phone snatched from the mother. It was a photo of a very energetic-looking child with round, pink cheeks looking at the camera with a slightly mischievous look in his eyes, and all of EAJ people involved remembered being moved and exclaiming, “The baby has become so energetic!!”

In preparation for their return to home country, EAJ was busy making arrangement, such as checking with pharmaceutical companies for immunosuppressants available in their home country, and scheduling a plan to receive blood test results to be forwarded by the parent from their home country and to instruct them remotely to increase or decrease the amount of medication prescribed in Japan after returning their home country. In addition to medicines related to transplant, the parents desired to bring home a variety of other medicines for as long as possible, as Japanese medicines are safer, EAJ also prepared the necessary documents so that these medicines may not be confiscated by customs. After the final medical examination, the parents and child returned to their home country.

Follow-up after returning to their home country

After returning to their home country, regular remote monitoring continued. Six months after returning to their home country, they came back to Japan as they were planning to spend the year-end and New Year holidays. At that time, the parents informed EAJ that their child had white stool. and in the worst-case scenario, the child might have been admitted to the hospital for tests and surgery. But after an outpatient visit, it was diagnosed that there was no serious problem, and the child was able to spend the Japanese New Year with his family. While staying in Japan, they also visited a dentist and a dermatologist.

The next visit to Japan was a year and a half later, and there were no problems. EAJ received a technical advice letter from the doctor of NCCHD which explains local doctors in the parents’ home country as to how to take echocardiograms in areas that are difficult to see because of overlapping with the portal vein and coronary artery due to growth of liver.　The Japanese doctor said it would be okay to see the child again in two years. Unfortunately, since the outbreak of the coronavirus pandemic, it has been difficult for their child to come to Japan, but EAJ occasionally gets in touch with the patents and they tell us that their child is doing well and is attending school.