Liver Transplant

Signs and Symptoms

The signs and symptoms of liver problems include:

• yellow skin and eyes
• very pale yellow/gray or white stools
• dark tea colored urine
• nausea
• vomiting
• decreased appetite
• growth delay or failure
• abdominal swelling
• easy bruising or bleeding
• frequent nose bleeds
• constant itching
• changes in sleep patterns or mental status
• fatigue or lack of energy.

Although there are hundreds of liver illnesses that may result in end stage or terminal liver disease, biliary atresia, a birth defect in which the bile ducts fail to develop or develop abnormally, is the most common cause for children's liver transplants at our hospital.

Other liver diseases in children are:

• Obstructive biliary tract disease
  1. Progressive familial intrahepatic cholestasis (Byler syndrome)
  2. Alagille syndrome
  3. Nonsyndromic bile duct paucity (loss of bile ducts)
  4. Neonatal hepatitis
  5. Sclerosing cholangitis
• Metabolic Diseases
  1. Alpha 1 antitrypsin deficiency
  2. Tyrosinemia
  3. Galactosemia
  4. Urea cycle defects
  5. Organic acidemia
  6. Glycogen storage diseases
  7. Wilson's disease
  8. Neonatal hemochromotosis
  9. Hyperlipoproteinemia
  10. Crigler-Najjar syndrome type 1
• Sudden hepatitis, often caused by poisoning
• Chronic hepatitis/cirrhosis
• Tumors
  1. Hepatoblastoma
  2. Hepatocellular carcinoma
  3. Hemangioendothelioma
  4. Sarcoma
• Miscellaneous
  1. Cystic fibrosis
  2. Caroli disease
  3. Congenital hepatic fibrosis
  4. Cirrhosis for prolonged total parenteral nutrition (TPN)

Evaluation

A preliminary evaluation is the first step in helping you and the transplant team determine whether transplantation is an appropriate treatment for your child. It also enables the transplant team to assess the medical factors related to your child's liver failure.

The appointment will take a full day, from approximately 8 a.m. to 4 p.m., and can be very tiring. The following tips will help you prepare for this first appointment:

• If possible, have family members or close friends accompany you to help understand the significant amount of information you will be receiving about the transplantation process.
• Because your child will be undergoing many tests, don't let him or her eat or drink anything after midnight of the day before your appointment. Plan to bring a snack.
• Please bring any medications he or she is taking, your health insurance information and your child's medical records if you have them.

As part of the evaluation, a series of tests will be conducted, including:

• Blood Tests to help determine how well your child's liver is functioning, and to assess kidney function.
• Ultrasound to view the blood flow to and from your child's liver, and locate any abnormal masses in the liver. A probe will be moved over your child's liver so that its image can be reflected and reviewed on a screen.
• Chest X-ray to help detect infection in your child's lungs and assess the status of your child's bones.
• Electrocardiogram to help identify any changes in your child's heart rhythm.
• Pulmonary Function to measure your child's lung capacity. Your child will be asked to breathe into a machine, and blood will be drawn to determine how well oxygen is being absorbed from his or her lungs.

A liver specialist, called a hepatologist, and a surgeon also evaluates every patient. The hepatologist will do a full exam, review your child's health history and discuss what it means to be on the transplant waiting list. The wait for a new liver can be up to three years, and tests may need to be repeated prior to the transplant.

You can discuss your test results with the hepatologist and surgeon and both will answer any questions. Many parents find it helpful to write down questions before the appointment.

You will meet with the financial counselor to review your insurance information.

Once the evaluation is complete, the transplant team meets to discuss each case and to decide whether to add the your child to the waiting list. Once on the waiting list, you will be notified and your child will undergo further testing at your local doctor's office. Parents with children on the cadaveric waiting list will receive instructions about getting a pager and informing the team about changing health conditions.

If a liver transplant isn't in your child's best interest, a transplant team member will call to discuss other options.

Before your child's operation, a social worker will talk to you to about his or her adjustment after the surgery. Individual counseling also is available during your hospital stay. If necessary, a social worker can arrange follow-up services and answer questions about disability.

Treatment

Liver transplantation may be a treatment option for children with end stage liver disease. Transplant livers may come from a cadaveric donor who has died, or from a living donor. To receive a cadaveric donor, the patient is put on a national waiting list until a donor becomes available. This can occur at any time, day or night. The wait is generally two to three years.

A live donor is usually someone in the family or a close family friend. Living-donor liver transplantation allows physicians to do the transplantation without the sometimes lengthy wait for a cadaveric liver. Both donor and recipient livers grow and remodel after the transplant. During the transplant evaluation, live donation is discussed with the parent. Donor safety is of primary concern throughout the process. Donors must be in good health, be of a compatible blood type to the recipient and be motivated to donate from altruistic reasons. If this is a feasible option, a donor evaluation will be started after all the recipient's testing is completed. If the transplant team determines the donation would work, a surgery date is scheduled for both the donor and recipient. This process usually takes up to six months.

Your child's surgery may last from 4 to 12 hours depending on his or her condition.

During surgery, your child's old liver and gallbladder will be removed and replaced with the donor liver. Since a gallbladder is no longer required, a new one will not be transplanted.

After surgery, your child will go directly to the intensive care unit (ICU), usually for one or two days. Immediately after surgery, a breathing tube will be inserted to help him or her breathe. In most cases the tube can be removed within 24 hours after surgery. Many monitoring lines also will be attached; these, too, will be removed as your child becomes more stable. When your child is ready to leave the ICU, he or she will be cared for on either the 6th or 7th floor of  the Long wing of the hospital.

Everyone recuperates from liver transplantation differently. Depending on your child's condition, he or she will be hospitalized for two to eight weeks following the transplant.

In general, most children stay within the San Francisco Bay Area for two to six weeks after transplant and then are referred back to their primary provider and referring physician. Our social workers assist families with housing and there is a San Francisco-based Ronald McDonald House. Once the child has returned home, we work with your primary doctors to ensure that your child receives optimum care regarding their liver transplant as well as issues related to normal growth and development.

Laboratory blood tests are obtained twice a week following transplantation; the frequency of blood tests is gradually reduced over time. You will be asked to call in test results to the transplant office. You will then be notified about any adjustments in your child's medications.

Complications can occur with any surgery. Patients undergoing organ transplants may face additional complications. The life-threatening disease that created the need for your child's transplant may affect the functioning of other body systems. Other risks, such as rejection, also may occur.

Some possible transplant complications and medication side effects include:

• Hemorrhage: One function of the liver is to manufacture clotting factors. When a liver fails, the ability to produce clotting factors is impaired. To correct this problem, your child will receive blood products before and after surgery. It is expected that your child's new liver will start working very quickly to help prevent any excessive bleeding, but it is possible that he or she may be returned to surgery to control the bleeding, particularly if it occurs within the first 48 hours after transplant.
• Thrombosis: If a blood clot forms in a vessel leading to or from your child's liver, this may injure the new liver. Your child will receive special anticoagulation medication to prevent this. This is a serious complication that may require a second transplant.
• Rejection: Your child's defense system --  the immune system -- protects him or her from invading organisms. Unfortunately, it also views the new liver as foreign and will try to destroy it in an attempt to protect your child. This is known as rejection. To prevent this from occurring, your child will be given special immunosuppressive medication that must be taken for the rest of his or her life. Rejection can be diagnosed early by performing weekly liver biopsies during the first few weeks after liver transplant. Although rejection is common, with early diagnosis and treatment the situation can be controlled in more than 95 percent of cases.

Immunosuppressive medications help to prevent and treat rejection. These drugs decrease your child's resistance to foreign bodies, such as the new liver. Your child will need to take these medications for the rest of his or her life or the liver will be rejected. Immediately after surgery, the dosages will be high since the probability of rejection is greatest at this time. Dosages will be lowered quickly to smaller amounts if there are no signs of rejection.

The medications have side effects, which are usually dose-related. Most people experience the highest level of side effects in the beginning when medication dosages are high. As the dosage is lowered, these effects will probably lessen. Side effects may occur in some patients and not in others.

The medications your child will take for rejection also impair his or her ability to fight off infections. Your child will be given medication to help prevent infections but you also will need to use caution and avoid contact with people with infections, especially during the first three to six months after transplant.

In the future, alternative therapies such as artificial liver assist devices, techniques of cellular transplantation, and genetic modification of hepatocytes may become viable approaches to managing children with end stage liver disease.