Pediatric Liver Transplantation Information for Healthcare Professionals

Chapter 3: Medical Management

Nutrition

Proper nutrition is essential to the management of progressive liver disease.  Bile salts, a primary component of bile, are necessary for the digestion of fats and absorbing fat-soluble vitamins A, D, E and K.  When bile salts are inadequate, there is poor absorption of fat-soluble vitamins which leads to vitamin deficiencies.  Stools become pale in color, foul smelling and of a large quantity.  Special fat substitutes, such as formula with medium chain triglycerides (Pregestamil) or MCT Oil, can alleviate this problem because they do not require bile for intestinal absorption.  Often vitamins A, D, E and K are given daily.

Treatment of Pre-Transplant Conditions

Other aspects of medical management in end stage liver disease include treating infections, bleeding, fluid accumulation (ascites) and pruritus (itching).  Children, who are diagnosed with biliary atresia and undergo a Kasai portoenterostomy in the first few months of life, will be managed with Bactrim to prevent cholangitis. Cholangitis is an infection of the biliary tree.  If your child develops a fever and cholangitis is the suspected cause, he will be treated with 10-14 days of IV antibiotics.  Bleeding complications, another consequence of liver disease, occurs when the liver is unable to produce clotting factors.  If your child develops prolonged bleeding time, additional vitamin K will be administered orally or via a shot. 

Some children also develop ascites or increased fluid in the abdomen.  In this case, a diuretic may be administered to increase urine output and to reduce the fluid accumulation.  Another problem that some children with end stage liver disease develop is pruritus or itching.  Several different therapies can be tried including using mild soap such as Dove.  A few children require medications such as Atarax, Benadryl or Rafampin to relieve the symptoms.  Parents may have to keep their children’s hands covered with socks or mitts to prevent scratching and skin irritation.  As mentioned above, nutrition is a very important  aspect of liver disease management. It is also important to provide emotional support to the child, siblings and extended family. The transplant team is committed to treating the whole child. 

Lab Work/Testing

Liver Function Tests

Liver function tests as well as other blood tests are commonly used to monitor the function of the liver and the biliary system in conjunction with the physical exam.  If the bilirubin level is elevated (greater than 1.0/0.2), bile flow may be obstructed.  You hear the health care team use the terms “direct” and “indirect” bilirubin.  Elevated indirect bilirubin  occurs in a newborn who has an increased production of bilirubin and a decreased ability of the liver to break it down and excrete it.  After the newborn period, an increased level of indirect bilirubin indicates damage to the liver cells.  On the other hand, elevated direct bilirubin levels reflect obstruction of bile from the liver itself.

Other blood tests include the liver function tests for the liver enzymes ALT, AST and Alkaline Phosphatase.  Elevation in any of these three tests indicates an obstruction of bile flow and/or damage to the liver cells.  Blood clotting studies, PT and PTT, will be done to determine how quickly the blood clots.  Other studies such as an untrasound, Tc-HIDA scan and liver biopsy are also used to determine liver function.

A liver biopsy involves the removal of a small piece of liver tissue to determine how well the liver

cells are functioning.  Before the biopsy your child will have lab work to determine if blood clotting is rapid.  In most cases, ultrasound guidance is used for the biopsy.  The child may not eat or drink for several hours prior to the procedure.  Medication is given to help him relax and to eliminate the pain.  A core of tissue is aspirated using a special needle.  A large pressure dressing is applied to the biopsy site at the end of the procedure.  After the procedure, your child will be kept on bed rest for six hours; a nurse will monitor blood pressure and vital signs frequently and check the dressing for signs of bleeding.  Once your child is awake, he can begin a regular diet.  Lab work will be done four hours after the biopsy and again the next morning to monitor for any signs of bleeding.  Biopsy results will be discussed with the family as soon as they are available. 

The following terms will become part of your vocabulary.  Always consult your doctor to help you understand what is “normal” for your child.

Laboratory Tests And Values

Pediatric Transplant Parent Support

The Johns Hopkins Children’s Center pediatric transplant service offers support through social workers dedicated to pediatric transplant.  The Comprehensive Transplant Center, which includes both pediatrics and adults, provides continuing education by presenting a speaker approximately every other month.  Some topics of interest include medical advances in transplantation, medications, FDA drug approval, nutrition and donor awareness.  A quarterly newsletter, Bridges, is distributed to all pediatric and adult  transplant families announcing upcoming events, committee activities as well as providing useful medical information.  Parents are invited to participate on any level, by attending a meeting and/or a social event.  

For more information please call your transplant coordinator at 410-955-5966 or link to the current transplant events calendar here.