THE SPECIALIST: Dr. Ronen Arnon
An associate professor of pediatrics and surgery at Mount Sinai, Dr. Ronen Arnon is a transplant hepatologist who specializes in treating children with liver problems. This column is the second in a series for National Donate Life Month.
WHO’S AT RISK
Along with the kidney and bone marrow, the liver is an organ that can now be transplanted from living donors as well as deceased ones, and sick children are a group that greatly benefits.
“There are many diseases and congenital abnormalities that can lead to liver failure in children, including newborns,” says Arnon. “And because the liver regenerates and grows very fast, it’s now possible for a parent to give 20% of her liver to a small child, and then be recovered in just one to two months.”
The liver is essential for daily life — a person can only live one or two days once the liver fails entirely. “The liver is the factory that secretes bile, detoxifies medications and deals with almost all of the body’s metabolism — of fat, carbohydrates and proteins,” says Arnon. “Because there is no equivalent of dialysis that can replace liver function, transplant is the only option for many patients.”
There are many pathways that can lead to pediatric liver disease and eventually liver failure. “Some children have congenital structural abnormalities like biliary atresia that interfere with their liver function and can eventually require a transplant,” says Arnon. “Other common causes of liver disease in children include medication overdose, the metabolic disease called fatty liver, tumors in the liver, and infectious hepatitis, which can be inherited from the mother.”
Some groups are considered at especially high risk of underlying causes that can lead to liver failure. “People from Eastern Europe and the former USSR have a higher rate of hepatitis C, while hepatitis B is more common in Asia. And because New York has a big immigrant population from both of these regions, we see a lot of children with these diseases,” says Arnon. “Children at risk of becoming overweight or obese are also at high risk of developing liver disease, because fat buildup causes the liver to become progressively inflamed and scarred.”
SIGNS AND SYMPTOMS
Parents should keep their eyes peeled for certain red flags of liver malfunction, like jaundice. “The most common thing that happens is that the child can turn yellow,” says Arnon. “Often, the whites of the eyes are the first thing to turn yellow.”
Patients can experience a wide spectrum of symptoms. “Some patients just don’t feel well — they have fatigue and other fairly vague symptoms,” says Arnon. “The stools can become paler, the urine can become darker, some people experience bleeding, and small babies can have a low level of glucose.” During a physical exam, the doctor may find an enlarged liver or spleen simply by touch.
Getting a full diagnosis is always the first step. “First we do a physical exam, followed by blood work, an ultrasound to examine the liver and spleen, and a few other specific tests,” says Arnon. “Sometimes we will need to a do a liver biopsy to make a complete diagnosis.”
The course of treatment depends on the diagnosis and the underlying cause. “In the case of biliary atresia — the single most common congenital disease that affects the liver — all patients will need some form of surgical correction,” says Arnon. “If that alone isn’t enough, then the next step is liver transplant.”
In some cases, treating the underlying cause can reverse the resulting liver disease. “Metabolic diseases like fatty liver disease can respond to changes in diet, losing weight and doing more exercise,” says Arnon. “For patients with hepatitis B and C, there are specific treatments that can offer the possibility of a cure.”
Because there is no equivalent of dialysis that can replace liver function, transplant is the only option for many patients.
For some children, liver failure isn’t the result of a progressively worsening liver disease — it can happen suddenly. “Some children’s livers will recover from an acute liver failure event, but other children will need a transplant,” says Arnon. “About 50% of the time we don’t know why the child developed acute liver failure. In many cases it’s the result of drug toxicity, whether the child is able to get into a bottle of Tylenol, for example, or the parents accidentally gave too high a dose.”
The decision that a child is a candidate for liver transplantation is made after careful evaluation. “Then we decide if the child is going on the national waiting list for a deceased donor’s organ or if there is a possible living donor,” says Arnon. “Usually, it’s a parent or relative giving a portion of their liver. The liver transplanted into the child also grows with the child.” About 10% of liver transplants in kids are from living donors.
Transplant patients must take immunosuppression medications for the rest of their lives, and some children will end up needing another liver transplant. “Overall, children have a relatively good prognosis after liver transplant,” says Arnon. “Thanks to the gift of organ donation, hundreds of children who need organs receive them and are able to go on to live healthy and productive lives.”
One positive development over the past few years has helped more children get the transplant they need. “In the past, both children and adults used to die on the waiting list for a liver,” says Arnon. “But recently, we have increased the number of potential grafts in two ways: by using a living related donor, and by pursuing different surgeries that can take one liver and give part to an adult and the smaller part to a child.”
QUESTIONS FOR YOUR DOCTOR
If you have concerns about your child’s liver health or jaundice that aren’t being resolved, don’t be shy about asking the pediatrician, “Can you refer us to a specialist?” For many thousands of patients, the key question is, “What can I do to help prevent my child’s fatty liver disease from progressing?” And a good thing for all parents to ask is, “What is the proper amount of Tylenol or other drugs I should give my child?”
“Overexposure to medical toxicity is still a tragically common cause of liver disease,” says Arnon. “Ask the doctor exactly how much medication you should be giving your child.”
WHAT YOU CAN DO
Get informed. Arnon recommends making the National Institutes of Health site (nih.gov/health/wellness) your first stop. For more information about pediatric liver transplant, including patient stories and videos, check Mount Sinai (mountsinai.org/patient-care/service-areas/organ-transplants/programs-and-services/pediatric-liver).
Help kids maintain a healthy weight. “By preventing obesity in children, you can help prevent them from needing a liver transplant down the road,” says Arnon. “Fatty liver may lead to cirrhosis of the liver, and we think that will be the main indication for liver transplant in the future.”
Get immunized. Immunization of both mothers and children is key to fighting hepatitis A and B, which can cause liver failure.
Be an organ donor. There are still child patients who die while on the waiting list for an organ. “Being an organ donor helps save children’s lives,” says Arnon.