Liver Transplant in Children

What is a pediatric liver transplant?

A pediatric liver transplant is an operation performed to replace a child's diseased or malfunctioning liver with a healthy one from another person. The liver may come from a deceased organ donor or from a family member who is willing to donate a part of their liver and is a suitable candidate.

Most transplanted livers come from deceased organ donors. Organ donors are adults or children who have become critically ill or injured and have been declared brain dead. If the donor is an adult, they may have agreed to be an organ donor ahead of time. Parents or spouses can also agree to donate a relative's organs.

Your child may get a whole liver or a segment of one. If an adult liver is available and is an appropriate match for two children (or a child and an adult) on the waiting list, the donor liver can be divided into two segments, and each part transplanted. This is called a split liver transplant. If an infant or child receives a split liver, the liver segment will be the right size for the recipient even if it is only part of an adult sized liver.

A living family member or loved one may also be able to donate a section of their liver. This is called a living-donor transplant. Those who donate a portion of their livers can live healthy lives with the remaining segment. The remaining organ will even grow back to the original size of the whole liver; the liver is the only vital organ in the human body that can do that.

Liver Transplant in Children and Teenagers
Mara, Annie's Mom: It was February 13th and my daughter had just celebrated her 8th birthday. Two weeks later, they're telling me she may not even make it through the night.

Ramon, MJ's Dad: When my son MJ was born, he spent three weeks in the hospital because they couldn't control his bilirubin levels. It was a scary situation.

Lissa, Mya's Mom: When Mya was 5 weeks old, we noticed that the whites of her eyes had turned yellow. Her whole body was yellow. We rushed her to the pediatrician and they said, “We can't help you; you need to go the CHOP.”

Elizabeth Rand: The most common serious liver disorder we see at CHOP is biliary atresia. This condition accounts for roughly 40 percent of pediatric liver transplant performed throughout the world.

Kim Olthoff: Many of the children we see with biliary atresia often need a transplant in their first years of life. And when we have the conversation with some parents about liver transplantation as a viable option for their child, it can be a very hard fact for them to process.

Rachel Goldberg: Often, we can perform a procedure to help the bile flow from the liver called the Kasai procedure. And while this procedure may help a child to forgo a liver transplant until later in life, it often delays the inevitable.

Lissa, Mya's mom: Persistent GI issues left Mya unable to absorb the nutrients from what she ate. She was malnourished, tired all the time and so unbelievably thin. She had to be hospitalized several times and get a feeding tube. She missed a lot of school and couldn't do any of the things her friends were doing because she felt so sick all the time.

Ramon, MJ's Dad: MJ went into liver failure when he was 8 years old. His liver couldn't filter out the poisons in his body and it got into his blood stream and made him itch. He would itch all day and all night. It was so bad that he couldn't sleep. His body also couldn't process any foods, so he was malnourished and tired most of the day. He suffered through many hospitalizations and feeling sick for a while and by age 9, he needed a new liver.

Mara, Annie's Mom: For eight years, Annie had been a very healthy little girl and then suddenly, because of some unknown virus, she became jaundice, she couldn't walk, and we were told that she needed a life-saving liver transplant to survive.

Elizabeth Rand: Thirty or 40 years ago, these children would have died from liver failure. But now we can do a liver transplant and give children a normal quality of life.

Rachel Goldberg: Some of our patients receive care for chronic liver failure for a while and then their condition gets worse to the point that they need a new liver. While other patients come to us after a sudden infection that causes their liver to shut down and their need for a liver is urgent.

Elizabeth Rand: Whatever the circumstances, our team comes together and meets with families to walk them through the entire transplant process and to answer each and every question they have.

Lissa: Mya's had a whole new life since her transplant. That part has been absolutely phenomenal. It's amazing what a good liver can do. Before, she couldn't play sports, particularly contact sports because of the risk of a splenic rupture. I cried when they wheeled her down the hallway for her transplant surgery, but I was so confident in the transplant team. CHOP's transplant team is one of the best in the country. This is what they do, day in and day out.

Elizabeth Rand: Every member of our team is devoted to our patients and their families. We don't just treat the child, but we seek to treat the whole family. Because even though it's the child who needs the liver transplant, the rest of the family is effected too.

Rachel Goldberg: When children get listed for transplant, a computer algorithm takes the results of their evaluations such as lab work, weight and height and calculates a score that determines your priority level on the transplant waiting list. For children with lower scores there can be a long time between getting listed and getting a liver. The higher the score, the sooner a child will be transplant. But even then, you're reliant on a donor liver coming available.

Kim Olthoff: There are three potential types of donors for liver transplantation. A transplant of a whole liver from a deceased donor, a transplant of just a portion of a liver from a deceased donor, and a transplant of a piece of a liver from a living donor. The liver is an organ that regenerates and grows so when a portion of the liver is transplanted, it will continue to grow in the body over time.

Our team is one of the most experienced in the country. We do around 150 liver transplants a year of which about 20 are children. We have been working together as a team for many years. Our surgeons are extremely skilled in transplanting even the most complex cases including very sick children and newborns whose tiny bodies require the utmost precision.

Jim, Annie's Dad: There was no donor in sight and they didn't know how long it would take until we got a donor. So we asked about the option of Mara becoming a living donor and fortunately, Mara was a blood match.

Part of Annie getting sick, we didn't realize how very fortunate we were to have Children's Hospital so close to us. The professionalism, the friendliness, the comfort, the care, the knowledge, everything you can imagine, it's just such a life-changing experience.

Kim Olthoff: As families get further out from transplant, those bonds get even tighter because as these kids go back to a normal life and experience all those wonderful milestones of childhood, their families will continue to send us pictures and share those special moments with us.

Lissa: I will never forget Dr. Rand's call at 2 o'clock in the morning saying, I think this is it. We got to the hospital around 4 in the morning for Mya's liver transplant.

Ramon: When we finally got the call, a lot of emotions were swirling because we wanted him to get the liver so that he would get better, but since it was surgery, there were also risks involved.

Rachel Goldberg: There are always risks and complications with transplant, immediate and long term complications. You can't really predict who that's going to happen to, so we do our best to prepare families for any potential scenario.

Elizabeth Rand: The first few days after transplant are critical and high stress for families.

We are involved in a minute-to-minute basis in the care in the ICU. Parents are usually surprised to hear that rejection is the least of our worries because it is so easily treated.

The majority of our kids recover quickly in the hospital and return to normal health.

Ramon: The first thing I remember after his transplant was when he opened his eyes, instead of being jaundice, they were white.

Jim: Immediately after the surgery, the doctor said her color started to change. It started from her toes and worked its way up, and the jaundice immediately went away.

Rachel Goldberg: The transformation of a sick child who has liver disease always amazes me. The actual transplant itself is still a little miracle whenever you see it happen. I think what really makes an impression on you is how the children transform so quickly after transplant. Going in, they are often so sick, then after transplant, they feel better and have more energy. They are finally able to absorb the nutrition they take in, they gain weight and grow. They go back to school, get to hang out with their friends, play sports. You see them in clinic a year later and don't even recognize them. They're happy and look fantastic. It's amazing to hear them tell you how good they feel and how much their life has changed from transplant.

MJ: I feel so much better now. I haven't been to the hospital in two years, and I sleep better 'cause I'm not itching all the time. I have a scar on my stomach from my transplant surgery, but it's awesome; I love scars.

Mya: It's amazing how much my life has changed. I no longer have chronic migraines or some sort of stomach issue and I'm able to go about my daily life without pain. Not needing a feeding tube in my nose was one of the greatest things that's ever happened to me. I never ever could have dreamed my life would be this great, and I'm so incredibly thankful to the amazing staff at CHOP for giving me my life back.

Mara: Annie is doing great. She's playing soccer, she plays tennis, she loves to swim, and she loves riding her bike. She's doing everything she wants to do.

Kim Olthoff: There's nothing more rewarding than seeing a child who's critically ill, dying from liver failure, and you somehow are able to use this gift of life that another family has so graciously offered and change a child's life for the better.

Transcript Transcript

Why would a child need a liver transplant?

Children need liver transplants because they have a disease or condition that has damaged the liver or caused it to work improperly. In rare cases, genetic diseases that do not cause liver injury but do harm other organs can be corrected with liver transplants. Some children require liver transplant in order to remove large tumors that can’t be removed without taking out the whole liver.

Children with these diseases or conditions may also need a liver transplant:

Biliary atresia: a disease in which the bile ducts are obstructed, preventing bile from passing from the liver into the intestines. It's the most common reason children need a liver transplant. Children's Hospital of Philadelphia is the leading center for biliary atresia care and referrals and is part of the Biliary Atresia Research Consortium (BARC).
Alagille syndrome: a genetic disorder that often affects the liver (among other organs), causing the bile ducts to narrow or fail. CHOP is a world-renowned Alagille clinical care center.
Primary sclerosing cholangitis: a disease in which the bile ducts narrow because of inflammation and scarring.
Hepatoblastoma: a very rare cancerous tumor that can spread to other parts of the body. CHOP is one of the nation's leading pediatric oncology centers and has a multidisciplinary team experienced in treating children with hepatoblastoma.
Acute liver failure: sudden loss of liver function that occurs when a large part of the liver is damaged, generally as the result of a virus or medication.
Some genetic disorders may also result in a liver transplant:
- Alpha-1-antitrypsin deficiency: a hereditary disease that can cause hepatitis and liver failure.
- Tyrosinemia: a genetic condition associated with severe liver disease in infants.
- Wilson disease: a hereditary disorder in which copper accumulates in the liver and nervous system, causing severe liver and other organ disease, which can be cured by liver transplant.

About the liver

The liver, the largest organ in the body, is located on the right side of the abdomen, underneath the ribs and diaphragm and on top of the stomach, right kidney and intestines. It's reddish-brown, weighs about 1 1/2 to 2 pounds in a child (depending on age), and holds about 13 percent of the body's blood supply at any given moment. It receives oxygenated blood from the hepatic artery and nutrient-rich blood from the hepatic portal vein.

The liver has two main lobes, both of which are made up of thousands of lobules. These lobules are connected to small ducts, which connect with larger ducts to ultimately form the hepatic duct. The hepatic duct transports bile produced by the liver cells to the gallbladder and duodenum (the first part of the small intestine).

One of the liver's primary jobs is to filter the blood of toxins and other harmful substances, which are then excreted into bile or blood. Bile waste products enter the intestine and leave the body in the feces; the kidneys filter out other blood waste products, which leave the body in urine.

The liver carries out many other important functions, including:

Converting food into chemicals necessary for life and growth.
Making the proteins needed for normal blood clotting.
Processing medications we take into forms the body can use.
Manufacturing bile, which helps the body digest and absorb fat, vitamins A, D, E and K, and medications. Bile is stored in the gallbladder and released into the intestines as needed.
Producing cholesterol and special proteins to help carry fats through the body.
Converting excess glucose (sugar) into glycogen for storage; this glycogen can later be converted back to glucose for energy.
Regulating the level of amino acids, which form the building blocks of proteins, in the blood.
Resisting infections by producing immune factors and removing bacteria from the bloodstream.
Accommodating the large volume of blood flow from the intestine on its return to the heart and lungs.

Preparing for a pediatric liver transplant surgery

Once your child has been evaluated by the liver transplant team, and it’s determined that he needs a new liver, your child should continue to visit his primary care pediatrician for well visits and immunizations, as well as when he or she is sick. It's very important that your child be as healthy as possible for the liver transplant. Care provided by your child’s liver transplant team should not replace regular pediatrician visits.

When the liver transplant coordinator contacts to let you know a suitable liver is available, he or she will ask you about your child's health. Let the coordinator know if your child has recently had a cold, flu or fever, or been exposed to chickenpox or other infectious diseases. If there are any concerns, the liver transplant coordinator will discuss them with the transplant physicians.

Once you're contacted, you will be instructed about your arrival time at the hospital, when your child should stop eating and drinking, and given any other special instructions. The liver transplant coordinator may also be able to give you an estimated time for the transplant surgery.

When you arrive at Children’s Hospital of Philadelphia, you should proceed to the welcome desk located on the first floor of the main hospital. Between the hours of 5 p.m. and 8 a.m., you should enter through the emergency room. Once the admission process has been completed, you and your child will be escorted to an assigned room.

When you arrive on the patient unit, we will place an intravenous (IV) line through which your child will receive IV fluids. We will draw blood with IV placement. The anesthesiologist (the doctor who will help your child sleep during the surgery) and the transplant surgeon or surgical fellow will perform physical examinations to assess your child's health and review the surgical procedure. They will ask you to sign the surgical and anesthesia consent forms — standard procedures before any surgery.

You should keep in mind that sometimes families are called into the hospital for liver transplant and the donor liver is then found to be unsuitable for the child. Although this is extremely disappointing, the surgeons want to be confident the organ is perfect for your child before transplanting it.

In the operating room

Liver transplant surgery usually lasts four to eight hours, but this can vary considerably based on the child's size, whether or not they have had prior surgery, and other factors. Throughout the surgery, an operating room nurse or other member of the transplant team will update you regularly — generally at least once an hour — on the surgery's progress.

The operating room team consists of pediatric surgeons, anesthesiologists, registered nurses and technicians. The operating room staff will call for your child one hour before liver transplant surgery, so the anesthesiologist and nurses can prepare your child for the operation. They will give your child medication through the intravenous line to help him sleep. Once he's sleeping, the staff will insert additional IV lines and an arterial line, as well as a central line inserted into a large neck vein. Blood can be drawn from this line, and it may remain in place during your child's hospital stay. In addition, the team will monitor your child's:

Urine output, with a Foley catheter placed into the bladder
Vital signs:
- Blood pressure, with a blood pressure cuff
- Heart rate and breathing rate, with monitor leads placed on your child's chest
- Temperature, with a temperature probe
- Blood oxygen level, with a pulse oximeter

The pediatric surgeon will begin the operation by cleaning your child's skin with an antiseptic, then place drapes on your child to protect the area on which she is operating. The surgeon will remove the diseased liver and send it to the laboratory for examination, then sew the healthy liver in place. When all the vessels are connected, the new liver will look pink and feel soft and blood will flow into the new liver.

Next, the surgeon will connect the bile ducts. If your child is small or has had a Kasai procedure, the surgeon will connect the piece of intestine that was fashioned into a bile duct during the Kasai procedure to the bile duct of the donor liver. This allows bile to drain from the liver. Otherwise, the surgeon will connect the bile ducts to each other. Throughout the operation, your child may receive blood products, only if necessary, to replace any he may have lost.

Once the procedure is complete, your child will be transferred to the Pediatric Intensive Care Unit.

Recovery and rehabilitation

The average length of stay following transplant is approximately seven to 10 days for otherwise healthy older children, and 10 to 14 weeks for infants.

Your child's first stop after liver transplant surgery will be the Pediatric Intensive Care Unit (PICU). When your child arrives in the PICU, the nurses and physicians will ask you to wait to see your child so they can get her settled.

The PICU is a busy place with many different types of equipment, alarms and sounds. Your child may be hooked up to quite a few pieces of equipment, and you may find it all a bit overwhelming. It helps to know how all this technology is helping your child. Here are some of the things you may see attached to her in the PICU:

Monitors that check heart rate, respiratory rate, blood pressure and oxygen saturation.
An endotracheal (ET) tube, which was placed in your child's trachea (windpipe) during the operation and connected to a ventilator to help her breathe. Your child won't be able to talk, eat or drink with the ET tube in place. Doctors will remove the ET tube when your child no longer needs help breathing.
An abdominal dressing over the incision. This will be removed on the first day for an ultrasound. The incision may be closed with surgical staples or sometimes with stitches.
A nasogastric (NG) tube, which passes through the nose and into the stomach, to help drain secretions from the stomach.
Several Jackson-Pratt drains in your child's abdomen to help remove extra drainage from the surgical site.
A Foley catheter to drain urine from the bladder.
Multiple intravenous (IV) lines to provide pain relief, antibiotics and other medications and fluids.
An arterial line to monitor blood pressure and provide blood samples. It will be removed before your child is transferred from the PICU to a surgical unit.
A central line — a flexible catheter inserted in a large vein — to give IV fluids and medications and, if necessary, provide blood samples.

Some of these tubes and lines will be removed when your child is transferred out of the PICU; others may stay in longer. If you have any questions, be sure to ask your child's caregivers.

Your child will also be monitored closely with routine studies and tests, such as:

An ultrasound, performed either the day of or the day after surgery, to check blood flow in and out of the liver
Chest X-rays to assess your child's lungs
Blood tests, done daily throughout the hospital stay, primarily to check for elevated enzyme levels, which can indicate rejection or bile duct problems

Once your child is medically stable and doesn't require frequent monitoring, she'll be transferred from the PICU to a medical unit, where nurses will continue to care for her and assess her recovery.

Once your child is transferred to a room on a medical unit, the atmosphere will be more relaxed, because medical unit patients no longer need the continuous monitoring that they needed in the PICU. However, your child's physicians and nurses will continue to monitor his vital signs and progress — and you, too, can play a role in helping your child's recovery go smoothly. You can help your child with:

Walking, which helps build strength, stimulates the gastrointestinal system and encourages bowel movements. Take your child for walks around the room and hallway; you'll notice he becomes more active with each passing day.
Coughing and deep breathing exercises, which helps prevent fluid buildup in the lungs. The nurse will help your child with these exercises and may teach him to use an incentive spirometer, a plastic container that helps him take deeper breaths.
Eating, which provides the calories your child needs to heal and grow following surgery. When your child is allowed to eat, he will make a gradual transition from clear liquids to solid foods. It's important that your child eat well-balanced meals, so a nutritionist will meet with you to help you develop a good diet plan. If he can't eat enough to get adequate nutrition, your child may receive nutritional supplements through a nasogastric tube or an IV.
Relaxation and play, which is an important part of your child's physical and emotional recovery. The Child Life specialist will help your child find toys and games to play with during the recovery period.

Follow-up care for pediatric liver transplants

Once your child is discharged, he will need follow-up care so his doctors can make sure his new liver continues to stay healthy and working properly. He should also continue to receive all necessary immunizations.

In addition to making sure your child gets the necessary follow-up care, you should watch him carefully during the recovery period. His play and activities are the best gauge of how he's feeling. You should also be sure you know the symptoms of rejection and call your child's doctor immediately if you notice any.

While you may be concerned about your child's health, it's important to remember that your child is a normal child who should be given the opportunity to do all the things other children do. The only difference is that your child must continue to take antirejection medications as directed.

If your child was a CHOP patient, his care will be transferred to The Hospital of the University of Pennsylvania (HUP) or an adult medical center of his choice in adulthood.

Signs and symptoms of rejection and infection

It's important that you know the symptoms of rejection and watch your child closely for them. And because the first sign of a rejection episode may show up in the regular tests your child will undergo (and not necessarily with any outward signs and symptoms), it's also important that you ensure he gets to all his follow-up appointments.

Remember, many children experience at least one rejection episode following a liver transplant. Rejection only means that the transplant team needs to fine-tune the immunosuppressant medications your child is taking to prevent his immune system from trying to reject the liver. When a rejection episode occurs, your child's doctor may prescribe a short-term steroid treatment, then adjust the dose of antirejection medications.

Outlook for pediatric liver transplants

According to the Scientific Registry of Transplant Recipients, the one-year pediatric liver transplant patient survival rate in 2002 - 2003 was 95 percent. The graft (transplanted organ) survival rate for patients transplanted in 2009-2011 at CHOP was 97.25 percent. The graft (transplanted organ) survival rate was 94.59 percent.

Survival rates vary from hospital to hospital around the country. At Children's Hospital of Philadelphia, we're proud that our graft (transplant) survival rates meet or exceed national averages. You can find more information about specific medical centers by visiting www.srtr.org.