Liver Transplant in Children
Information for parents
About the liver
The liver is the largest solid organ in the human body. It 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 in color. It receives oxygenated blood from the hepatic artery and nutrient-rich blood from the 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.
- 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.
A liver transplant is an operation performed to replace a child’s diseased or malfunctioning liver with a healthy 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 his or her liver and is a suitable candidate. In Pakistan due to scarcity of brain dead donors; the liver from living donor is the only chance for transplantation in sick children. Usually parents and close family members are the suitable donors. Their generous act of altruism can safe child’s life. Those who donate a portion of their livers can live healthy lives with the remaining liver. The donor will donate only left lateral segment of the liver (which is 15-20% of your liver) if donating for an infant and left lobe of the liver (which is 30-40% of the liver) if donating for a child. 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 has the ability to grow.
Infants and children need liver transplants when a disease or condition 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 for large tumors that can’t be removed without taking out the whole liver.
Common diseases in children that may 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.
- Alagille syndrome: a genetic disorder that often affects the liver (among other organs), causing the bile ducts to narrow or fail.
- 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.
- Progressive familial intra-hepatic cholestasis (PFIC): a rare genetic disorder that runs in families and often affects the liver, causing the failure of bile excretion form the liver and eventually leads to liver failure.
- 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. If it’s occupying most of the liver and can’t be removed without removing the whole liver.
- 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.
Preparing for 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.
Recipient Exclusion Criteria (contraindications to transplant):
- Severe, irreversible, medical illness that limits short term life expectancy
- Severe Pulmonary Hypertension (Mean PA pressure > 50mmHg)
- Sepsis or uncontrollable infection
- Severe, uncontrolled Psychiatric illness
- Uncorrectable congenital abnormalities which are severe and life threatening
Relative Exclusion Criteria:
- Advanced malnutrition
- Active Infection
- Inability to comply with medications and conform to follow up regimens
The Assessment Process
It is a stepwise assessment of the recipient and the donor. The purpose of stepwise assessment is to avoid un-necessary investigations. Assessment starts from recipient once he/ she has clear indications of liver transplantation. The timing of transplant is crucial; so objective assessment process is mandated.
Objectives of liver transplant assessment:
- Right recipient selection
- Right donor selection
- Better outcome
Stepwise Recipient Assessment:
Patients are examined by hepatologists and liver transplant surgeons. After initial assessment if a patient is a candidate for liver transplant, detailed briefings are given to the patient and their family regarding the risks and benefits of the transplant operation.
Patients are given time to think and plan transplantation. They are also provided with educational material. Once the patient and family has made final decision for liver transplantation, the detailed assessment process is started that includes following:
- Ultrasound of the abdomen
- Liver dynamic CT abdomen, and Chest CT to rule out/stage HCC, determine extent of liver disease
- MRI and CT brain in selected cases
- Echocardiogram/stress echo including PA pressure
- Coronary angiogram (selected cases )
- Cardiologist comments
- Pulmonary function tests
- Pulmonologist evaluation
- Renal function tests
- Routine urine analysis
- Urine culture
- Nephrologists evaluation
- Consultation & Evaluation
Stepwise donor evaluation:
Once it is assured that patient is fulfilling the liver transplant criteria and is fit enough to undergo major surgery, the donor workup starts. We accept donors from within the family (close blood relatives and non-close blood relatives) according to the following criteria:
Living Donor selection criteria:
- A blood related person or a spouse who shares compatible blood group with the patient.
- Donors must be healthy individuals with no major health problem.
- Must be in good physical and mental health
- Must be between the ages of 18 and 50 years
- Voluntarily, altruistic donation
- Have a body mass index (BMI) less than 35
- Must have a compatible blood type with the recipient
- Must be free from the following:
- Significant organ diseases (i.e., Heart, Lung & Kidney disease, etc.)
- Ongoing malignancy (cancer)
- Active or chronic infections
- Active substance abuse
Laboratory work-up the living donor:
The purpose of the stepwise evaluation of the donor is to avoid un-necessary investigations. If the donor is found to be unsuitable at any step, the other investigations should be abandoned. Before starting the investigations of the donor, detailed history and physical examination including height, weight, BMI is recorded. Steps of donor evaluation are as follows:
Special Considerations and/or Issues in Donor Evaluation:
Usually a physician outside of the transplant team, who will help the donor understand the process, procedure, risks and benefits of live organ donation. The donor advocate will protect and promote the interests and well-being of the donor. Donors can opt to withdraw at any time during the assessment process. The reasons for withdrawal will be kept confidential.
Our program may perform liver biopsy on potential donors based on clinical findings that suggest some degree of concern regarding histological status of the liver, i.e., elevated AST/ALT, presence of steatosis on imaging studies and so on.
Cost of Donor Evaluation:
Financial considerations of the donor evaluation and hepatectomy are important to consider because the process is complex and expensive and some of donors has “failed donor evaluations,” i.e., potential donors who undergo testing and are rejected for donation. Living donation is not possible for all the donors due to medical and technical reasons.
After complete assessment of the recipient and the donor, the case is presented in Transplant Multidisciplinary Meeting (MDM).
Transplant Multidisciplinary Meeting (MDM) Guidelines:
- a) Objective: Discussion of all patients under consideration for transplantation.
- Consultant Hepatologist
- Consultant Liver Transplant Surgeon(s)
- Consultant Liver Transplant Anesthetist(s)
- Consultant Intensivist(s)
- Transplant Coordinator Team (including MDT Co-ordinator). The Co-ordinator will also act as a social worker
- Physicians in training
Nurses responsible for care of patient
- b) Agenda in brief: For each patient:
- Short/salient summary of each patient based on evaluation proforma and listing meeting outcome.
- Discuss suitability for transplantation
- Discuss necessity/appropriateness of further investigations
- Define action plan (outcome) – listing/ time-frame – record outcome – Database
- c) Presentation at MDM: List of all patients for discussion to be circulated to attendees 24hrs prior to meeting (responsibility of MDT Co-ordinator). All names are forwarded 24 hours before the meeting.
Give short summary of patient’s history: indications for transplantation, presentation, referral source, suspected/confirmed diagnoses, and investigations +/- treatments to date. Summarise all available relevant transplant assessment related investigations.
- d) Discussion:
- Rationalisation for transplantation and absolute/relative contraindications:
- Exploration of other treatment options/further investigations
- Recommendation of treatment(s) (e.g. abstinence programs etc)
- e) Define action plan:
- If accepted for listing, discuss priority/type of graft/immunosuppressant regimen.
- If not listed, define reasons/future candidacy/ follow up plan.
- If patient to be re-discussed, define issues for re-discussion
- Record outcome – with review date if appropriate
- f) Updates: Updates will only be discussed on the basis that they require a new decision from the Transplant MDM.
- g) Acute Liver Failure: Patients with acute liver failure approaching or meeting listing criteria for urgent transplantation can be discussed ad-hoc and listed upon agreement between Intensivist/Hepatologist/consultant transplant surgeons. We follow King’s College criteria for liver transplant in acute liver failure.
- e) MDM- Responsibilities of Transplant Co-ordinator:
- Preparation of MDM
- Circulate list to all attendees 24hrs prior to the meeting
- Record attendance and generate quarterly report for “Chair”
- Ensure outcomes are recorded – check database & written record
- Contribute to MDM discussion – additional information including influential factors e.g. social issues, previous experience of patient
After approval from transplant MDM both the donor and the recipient are presented to the hospital Evaluation Committee for final approval. Hospital evaluation committee is a legal requirement of Human Organs and Tissues Act, 2009 (No.F.9 (1)/2009-Legis).
- g) The Hospital Evaluation Committee:
- Ensures that no organ or tissue is retrieved from non-related living donors without the prior approval of the Evaluation Committee
- Determine propriety of removal of a human organ from any living person using brain death protocol to be formulated; and
- Determine fitness or otherwise for transplantation of a human organ into any other body.
The hospital Evaluation Committee either approves the case (for close blood relatives) or forwards it to Human Organ Tissue Authority (for non-close blood relatives).
Documentary evidence of relation is established by relevant national identity card, birth certificate and marriage certificate.
In case of non-close blood relatives the evaluation committee evaluate for the following:
- There is no commercial transaction between the recipient and the donor. No payment of money or money’s worth as referred to in HOTA act, has been made to the donor or promised to be made to the donor or any other person. In this connection, the Evaluation Committee shall take into consideration:
- An explanation of the link between recipient and donor and the circumstances which led to the offer being made.
- Documentary evidence of the link e.g. proof of relationship (NADRA Family Relationship Certificate-FRC).
- Reason why the donor wishes to donate.
- There is no middleman / tout involved.
- The donor is not a drug addict or active substance abuse
- The next of kin of the proposed donor gives permission on stamp paper in case of non-close blood relatives.
After assessment of the recipient and the donor the surgical team designs surgical plan
For the living donor liver transplantation.
After this process is complete, both the recipient and the donor are informed about the process of liver transplantation in detail and informed consent is obtained separately from the donor and the recipient and the date of surgery is decided and following plan is given all the risks and benefits of the transplant to the donor and to the recipient are explained in detail. Recipient, donor and the family is provided with the opportunity to enquire any other information regarding the whole process.
The recipient operation will take 6-8 hours. The recipient will stay 4-6 days in the ICU followed by another 2 weeks in the hospital. The recipient average hospital stay should be around 12-15 days. Donor’s total hospital stay should be 7-8 days. The recipient will be given prophylactic antibiotics, analgesia, low molecular weight heparin for DVT prophylaxis and immunosuppressive drugs (including Tacrolimus, Mycophenolate Mofetil and steroids), anti-fungal medication and Omeprazole.
Liver transplant co-coordinator will accompany the patient and their family throughout the process to facilitate them at each and every step.
In the operating room
Liver transplant surgery usually lasts six 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, a transplant coordinator or a member of the transplant team will update you regularly — generally at least once an hour — on the surgery’s progress.
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 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 Liver Intensive Care Unit.
Recovery and rehabilitation
The average length of stay following transplant is approximately 10-12 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 Liver Intensive Care Unit (LICU). When your child arrives in the LICU, the nurses and physicians will ask you to wait to see your child so they can get her settled.
The LICU 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 LICU:
- 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.
- A nasogastric (NG) tube, which passes through the nose and into the stomach, to help drain secretions from the stomach.
- Surgical 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 LICU; 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 LICU to a transplant floor, where nurses will continue to care for her and assess her recovery.
Once your child is transferred to a room, the atmosphere will be more relaxed, because now the patients no longer need the continuous monitoring that they needed in the LICU. 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 nurse specialist will help your child find toys and games to play with during the recovery period.
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.
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.
The one-year pediatric liver transplant patient survival is 90-95 percent.