Liver transplant

Overview

Liver transplantation, first performed in 1963, provides an opportunity for a longer, more active life for people in the final stages of liver disease. Advances in surgical techniques and new medications that prevent the body from rejecting the transplanted organ have greatly improved success rates.

The largest organ in the body, the liver performs many complicated functions, including processing proteins, fats and carbohydrates and breaking down toxic substances such as drugs and alcohol. In addition, the liver makes the chemical components that help blood to clot. If the liver fails, the body loses the ability to clot blood as well as to process nutrients needed for life.

The liver also excretes a yellow digestive juice called bile, which may accumulate if the liver is not working properly. The eyes may become "jaundiced" or yellow, or the skin may itch from the accumulated bile. Some medications help treat the symptoms of liver failure, but there are no drugs that cure liver failure.

There are hundreds of liver illnesses that may result in end-stage liver disease, but 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.

If your child's liver begins to fail, he or she may be eligible for a liver transplant that could involve a relatively new procedure called a "living donor" transplant. In living donor liver transplantation, a piece of liver is removed from a living donor and transplanted into a recipient. Because the liver can regenerate itself, the piece of liver that remains in the donor quickly grows back to its original size. Transplant livers may also come from someone who has died, called a cadaveric donor.

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

Evaluation

Most liver transplant patients at UCSF are referred to the program by a primary care provider or a specialist. When a referral is made, a transplant-program patient navigator will call you to schedule an appointment.

Preliminary 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 disease. Not everyone who is evaluated for a liver transplant actually needs one. Your initial appointment will help determine your child's treatment options.

The preliminary evaluation can be scheduled for a full day, from about 8 a.m. to 4 p.m, or spaced over two days. The evaluation day can be very tiring. The following tips will help you prepare for this first appointment:

• If possible, bring family members or close friends to help you understand and remember the significant amount of information you will be receiving about the transplantation process.
• Please bring any medications your child 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. Whenever possible, we will use results from tests your child has already completed. However, testing may include:

• Blood tests to help determine how well your child's liver is functioning and to assess kidney function and to check for previous infections
• A chest X-ray to help detect infection in your child's lungs and assess the status of your child's bones
• A CT scan or MRI to obtain more detailed pictures of your child's liver
• An echocardiogram (ultrasound of the heart) to assess the structure and function of your child's heart
• An electrocardiogram (ECG) to help identify any changes in your child's heart rhythm
• An ultrasound to view the blood flow to and from your child's liver and to locate any abnormal masses in the liver

A liver specialist, called a hepatologist, and a surgeon will also evaluate your child. 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. Our transplant nurse and nurse practitioner will also meet with you to talk about the transplant and get to know your child and family.

You can discuss your test results with your care providers, who will answer any questions you have. Many parents find it helpful to write down their questions before the appointment.

You will also meet with:

• A transplant social worker to talk about resources your family will need during the transplant journey
• A registered dietitian to help optimize your child's nutrition
• A transplant psychologist to talk about how your child, and you, are doing during the transplant process and what our team can do to support your mental health
• A financial counselor to review your insurance information.

Waiting for a transplant

Once the evaluation is complete, the transplant team meets to discuss each case and to decide whether to add your child to the national waiting list for available livers. Once your child is on the waiting list, you will be notified and your child will undergo further testing.

Parents with children on the deceased donor waiting list will receive instructions about how to contact the team and keep the team informed about changing health conditions. Since a donor liver may become available at any time of the day or night, you must carry your phone with you at all times and keep it turned on. The wait for a new liver is generally a few months, though this can vary depending on your child and his or her health condition.

When a donor liver becomes available, careful testing is performed to ensure that the organs are not damaged in any way. Then, they are matched to a transplant candidate of compatible size and blood type. In some cases, the team may conclude that the donor liver is not satisfactory. If this occurs, the transplant will be canceled. If a cancellation occurs, remember that it is in your child's best long-term interest to wait for the liver that will be best for him or her.

Living donor liver transplantation

Transplant livers may come from a deceased donor or from a living donor. A living donor is often someone in the family or a close family friend, but it can also be someone who is not related to you. Living donor liver transplantation allows surgeons to perform the transplant without the sometimes-lengthy wait for a deceased donor liver. Both donor and recipient livers grow and regenerate after the transplant. During the transplant evaluation, we will discuss living donor transplantation with you.

Donor safety is of primary concern throughout the process. Donors must be in good health, have a blood type that is compatible with the recipient and be motivated to donate by altruistic reasons. If live donation is suitable for your child, a donor evaluation can be started after your child's testing is complete. If the transplant team determines the donation would work, a surgery date is scheduled for both your child and your child's donor. This whole process usually takes a few weeks to months, again varying based on how urgent your child’s need for transplant is.

Procedure

Your child's surgery may take from five to twelve 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 needed, a new one will not be transplanted.

After surgery, your child will go directly to the pediatric intensive care unit (PICU), usually for two to four days. During surgery, a breathing tube will be inserted to help him or her breathe. In most cases the tube is removed at the end of the surgery. Many monitoring lines will be attached; these, too, will be removed as your child becomes more stable. When your child is ready to leave the PICU, he or she will be cared for on the fifth floor of the hospital.

Recovery

Everyone recuperates from liver transplantation differently. Depending on your child's condition, he or she will likely be hospitalized for two to five weeks following the transplant. Most children from out of town stay in the San Francisco Bay Area for six to eight weeks after the transplant and then are referred back to their local primary care doctor and referring physician. Our social workers will assist you with free temporary housing if you live far away from the hospital.

Once your child has returned home, we work with your primary doctors to ensure that your child receives optimal care, both for the liver transplant as well as issues related to normal childhood growth and development.

Laboratory blood tests are obtained twice a week when your child first leaves the hospital after transplant; the frequency of blood tests is gradually reduced over time. Our team will communicate with you about test results and any adjustments in your child's medications or care. You will also be able to contact our team's 24-hour emergency number for guidance if any emergencies arise.

Potential complications

Complications can occur with any kind of surgery, and 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 problems, such as rejection of the new liver, may also occur.

Some possible transplant complications and medication side effects include:

• Hemorrhage (bleeding). One function of the liver is to make chemical components used to help blood clot, called 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. We expect that the new liver will start working very quickly to help prevent any excessive bleeding, but your child may need to return to surgery to control the bleeding, particularly if it occurs within the first 48 hours after transplant.
• Thrombosis. If a blood clot (thrombosis) 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 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 must take special immunosuppressive medication for the rest of his or her life. Rejection can be diagnosed early by monitoring blood tests, and sometimes by doing a liver biopsy, after liver transplant. Although rejection is common, with early diagnosis and treatment the situation can be controlled in almost all cases.

Immunosuppressive medications

These drugs decrease your child's likelihood of rejection, which is an immune system reaction against the new liver. We anticipate that your child will need to take immunosuppressive 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. Our goal is always to have your child take the least amount of immunosuppression they need to prevent rejection.

The medications have side effects, which are usually dose-related. Most people experience the most side effects in the beginning when medication dosages are high. As the dosage is lowered, these effects will 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.