Pediatric Liver Transplantation Information for Healthcare Professionals

Chapter 11: Transplantation And The Immediate Post-Operative Period

The day has finally come for your child’s surgery.   Some families have expressed amazement when the call comes.  Once you receive the call from the coordinator, make plans to come to the hospital as quickly and safely as possible.  The coordinator will ask you several questions regarding your child’s current health: does he have a cold, fever or rash or has he recently been exposed to a communicable disease (i.e. chicken pox)?  Children who currently have a virus or the flu may not be a good candidate for transplantation because the immunosuppressant medications used in the post-transplantation period to prevent rejection may prolong recovery from these ordinary illnesses.  The coordinator will also tell you when to come to the hospital. 

Once you get the call, do not give your child anything else to eat or drink.  When you arrive at the hospital, go directly to the emergency room for surgery prep.  Blood work will be obtained, an IV will be started and a bowel prep will be administered to clean out the large bowel before surgery.  Some of the medicine will be given through the mouth, and the remainder will be given as an enema through the rectum.  Your child will also receive IV antibiotics. 

Although it is rare, it is possible you could arrive at the hospital and find out the surgery has been canceled.  Although many tests are done on the donor, it is only after the liver has been examined that the transplant surgeon can determine whether the donor organ is acceptable.

One parent may accompany the child into the operating room and stay until the child is asleep.  A cover gown, mask and show covers will be provided for the parent to go into the operating room.  The surgery is approximately 8 to 12 hours in length.  During the surgery, one of the OR nurses or surgeons will come out to speak with you and provide you with updates on your child’s condition.

Two surgical teams will perform the transplant surgery; one team will remove your child’s native liver, while the second team will prepare the donor liver for placement.  An incision is made in the upper part of the child’s abdomen, from right to left, occasionally with a vertical extension up to the breast bone.  If your child has had a previous procedure, the Kasai procedure for example, the same incision is usually used.  After the diseased liver is removed and sent to the laboratory for examination, the healthy liver is sewn in place by the transplant surgeons.  Four major vessels are connected; the suprahepatic vena cava, infra hepatic vena cava, hepatic artery and portal vein.  Once these procedures are complete, the new liver will look pink and feel firm.  Next the bile drainage is established with the child’s intestines.  If your child has had a Kasai procedure, the biliary connection will be similar.  The most common pediatric biliary connection is called a Roux-en-Y.  A small plastic tube called a stent will be placed internally across the suture line between the intestine and the bile duct.  This prevents the area from closing off and blocking bile flow while the healing takes place.  The stent will pass out through your child’s stool within a few months after the transplant.  Occasionally, your child’s bile duct and the bile duct of the transplanted liver will be large enough to connect the two ducts directly together.  A t-tube will be inserted into the bile duct connection and passed through the skin in the abdomen.  This allows the bile duct to heal and the bile is collected into a bag on the outside of your body.  Before discharge, the tube will be tied off, and you will be taught how to change the dressing and care for the skin around the insertion site.  Four months after transplant, a study (t-tube cholangiogram) will be performed through this tube.  If there are no problems, the bile duct tube is removed at that time.

All of the new structures are carefully inspected prior to closing the abdomen.  Two or three soft silastic drains are placed around the liver to remove excessive drainage that occurs after surgery.  The wound is then closed with staples.

Pediatric Intensive Care Unit

After surgery your child will be taken to the Pediatric Intensive Care Unit (PICU).  The sights and sounds of the unit will be unfamiliar to you, so the nurses and doctors will explain everything that is happening to your child in the PICU.  It will take approximately an hour to connect equipment and “settle” your child into the unit.  Once everything is complete, you may visit.

Visitors to the PICU are limited to two people at a time.  You may call the unit at any time to ask about your child’s condition.  To maintain privacy and confidentially, the PICU staff will not release information about your child to anyone but the parents.  Because of the extensive equipment and the one-on-one nursing care, parents are not allowed to sleep in the child’s room.  Please leave a phone number with the nursing staff so they can contact you if there is a change in your child’s condition overnight.  Sometimes a visit may not be possible because a doctor is performing a sterile procedure, or the staff is making rounds.  Always check at the front desk before entering the unit.

Over the first 24 hours, your child will be in protective isolation.  Everyone entering the room must wear a gown, mask and gloves.  On the second post-operative day, and throughout the hospital stay, good hand washing should be adequate to avoid an infection.

Your child will return from the operating room with a breathing tube in his mouth (endotracheal tube).  The breathing tube is connected to a ventilator to assist your child’s breathing.  While the breathing tube is in place, your child will not be able to speak or cry.  If he is awake, he can be given a communication board or paper and pencil to communicate his needs.  The tube is usually removed on the second or third day after surgery.  However, children who have an underlying problem with their heart or lungs may need the breathing tube for a few more days.  After the breathing tube is removed, most children need extra oxygen for a few days.  It will be provided by either a face mask or a nasal canula.

Your child will have a large abdominal dressing with two abdominal drains.  The nurse will carefully monitor how much fluid comes out of the drain.  Your child will also return from the operating room with multiple intravenous lines to provide medications, fluids and blood products as well as to monitor laboratory values.  If your child becomes very active when he wakes from the surgery, he may need to be restrained to protect the IV sites.  A nasogastric tube (NG), which passes from the mouth or nose into the stomach, will be inserted while your child is asleep in the operating room.  It will be left in place several days to drain secretions from the stomach and to prevent vomiting.  It may also be used to give medications.  Once your child has active bowel  sounds or passes stool, the NG tube will be removed.  A catheter, or a tube to drain urine, will be inserted into your child’s bladder while he is asleep in the operating room.  The nurses will measure your child’s urine output to monitor his kidney function.

Initially, your child will not receive anything to eat or drink (NPO).  All of his nutritional needs will be met through the IVs.  Once your child is able to eat again, he will be offered clear liquids and then an age-appropriate diet.  Some children require supplemental feedings which can be provided via an NG tube as formula or through the IV line as TPN (total parenteral nutrition).

Complications

Many parents ask about pain medication.  Pain medication is provided to the children to keep them as comfortable as possible.  However, it is very important to assess neurologic function, so children are not given medication to make them sleep all of the time.

Biliary Leaks

Biliary leaks or obstructions account for 10 percent of complications in the pediatric transplant population.  Children with reduced size liver grafts are at a greater risk for developing a biliary leak or stricture.  The complication is related to inadequate blood supply to the biliary anastomosis (surgical connection) or a technical difficulty because of the small structures.  Prompt treatment is required; the child will require reexploration in the operating room.  Obstruction of the biliary tree is usually a late finding.  It can occur as long as a year after surgery.  Usually the child will have symptoms of cholangitis, such as a mild fever or deteriorating liver function.  Obstructions are managed with transhepatic stenting.  This procedure is performed in the interventional radiology suite (CVDL) and does not require a second surgical procedure. 

Hepatic Artery Thrombosis

Hepatic artery thrombosis is a serious complication that occurs in 10 to 20 percent of pediatric liver transplant recipients.  The hepatic artery, which is attached to the new liver’s artery, narrows or a blood clot forms.  Usually the child will develop acute graft failure or formation of an intraphpatic abscess or sepsis.  Correction requires surgical intervention, and in some cases. Retransplantation.  Routine studies to assess blood flow to the liver will be obtained in the immediate post-operative period to determine if blood flow to the liver is a problem.  Additionally, cultures of the fluid from the abdominal drains will assist in early detection of infection.

Rejection

Rejection is the most common post-transplant complication: more than 90 percent of the children experience it at least once.  Don’t panic if this happens to your child!  The first episode of rejection usually occurs about seven to ten days after transplant.  However, even after months at home, your child may experience rejection.  How will you know your child is rejecting his liver?   The classic signs include a fever (temperature greater than 38.5C or 101F, elevation of the AST and ALT liver enzymes, dark urine or clay colored stools, loss of appetite, jaundice and/or pain over the graft.  Often changes in lab values are the only sign of a rejection episode.  Usually a liver biopsy is performed by the pediatric gastroenterologist to confirm the diagnosis.  If rejection is confirmed, most children receive large doses of steroids, one of the medications that provides immunosuppression.  If the liver enzymes return to normal after the initial steroid doses, the doctors will prescribe a 10-day course of oral steroids.  Occasionally children need a stronger medicine to help fight the rejection episode.

Infection

The same medications that prevent rejection also decrease the body’s ability to fight infection.  As a result, care must be taken to limit your child’s exposure to infections such as colds or the flu.  You must be alert to signs of infection in your child, including a fever (temperature greater than 101F, a sore throat, a cough that last longer than 24 hours, rashes and diarrhea. 

Common viral infections include CMV (cytomegalovirus), herpes and fungal infections such as candida.  Some of the common sites for CMV infection include the new liver, the gastrointestinal tract (GI) or the lungs.  Typical symptoms include night sweats, low grade fever and diarrhea.  A first time exposure occurs in many children who are CMV negative at the time of transplantation because they may receive a CMV positive donor organ.  All children who are seronegative for the CMV virus and receive a seropositive organ, will receive Intravenous Immunoglobulin (IVIG) or Cytogam on an eight-week schedule to prevent CMV infection.  Regardless, many children will develop CMV.  Treatment involves administration of Ganciclovir IV for four weeks and adjustment in other immunosuppressant medications.  Viral shedding may persist for several weeks to months after the initial infection occurs.  Good hand washing will help prevent further spread of this virus.

The herpes virus is another common viral infection, which appears as an itchy, small blistery, red rash on the skin or in the mouth.  Treatment involves the administration of Acyclovir by mouth or IV.

The most common fungal infection that occurs in an immunosuppressed child is candida or thrush.  This fungus often grows in the mouths of children on antibiotics, such as Bactrim, because the antibiotics eliminate bacteria that is normally present in the body.  The primary symptom of thrush is an outbreak of white spots on the tongue, gums and inside the lips and cheeks.  Your child may also develop a fever, cough or digestive problems.  Oral nystatin is prescribed to prevent or treat the thrush.  If a fungal diaper rash occurs, mycostatin cream may be applied to the affected area.

After transplantation your child is at risk for a number of bacterial infections.  Initially they will receive antibiotics such as Bactrim to prevent an infection and routine cultures will be taken several times a week in the immediate post-operative period.  If an organism is identified, the appropriate antibiotic will be ordered to fight the infection.

Post-Transplant Lymphoproliferative Disorders (PTLD)

Lymphocytes are white blood cells that are part of your immune system and play a key role in attacking foreign invaders such as bacteria and viruses.  The lymphocytes are divided into B cells and T cells. (For more detail, see the section labeled the Immune System.)  The immunosuppressant medications (Cyclosporine or Prograf) your child takes suppress the activity of the T cells and prevent rejection.  The medications are not specific to the organ but in fact suppress the entire immune system.  Normal regulation or control of B cell production and T lymphocytes is altered when a child takes immunosuppressant medications.  As a result of this imbalance the B cells can multiply rapidly. 

When a person has a virus, the normal immune response is to produce more B cells to fight the infection.  The Epstein-Barr Virus (EBV) is a virus that directly attacks B cells and causes an overproduction of B cells.  In a healthy person the EBV infection is called mononucleosis, and the patient recovers from the virus by controlling the B cells that are activated. In a transplant patient, the normal T cell control over the infected B cells is compromised by the immunosuppression, and these B cells may grow uninhibited.  This is lymphoproliferative disorder.

When the donor and recipient are an EBV mismatch (in other words, the child is negative for the virus and the donor is positive), your child will be given an antiviral called Acyclovir to prevent this problem.  However, if the EBV virus develops, the amount of immunosuppression medication is usually reduced to allow the immune system to recover and fight the overproduction of B cells.  The usual signs and symptoms include fever, malaise, lymph node enlargement and liver and spleen enlargement.

Transfer To The Transplant Unit

Children are transferred to the transplant unit once they are stable, usually after a week, but possibly after just a few days.  It is important to remember that every child has a slightly different course in and out of the intensive care unit, so predicting length of stay is difficult.

Some things that you can do to help your child during the recovery period:

• Encourage your child to walk around the room; this will help him build strength.
• Participate in coughing and deep breathing exercises; use the incentive spirometer or blow up a glove placed on a large syringe.
• Encourage your child to eat.  The body requires many calories to heal after surgery.  A nutritionist will meet with you to help plan the appropriate diet.  If your child has difficulty taking an oral diet after transplantation, an occupational therapist will be consulted.
• Play is an important part of your child’s recovery.  A child life therapist will help you find appropriate toys to assist your child in adjusting to the hospitalization.