About 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.

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.