Liver Transplant FAQs

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Can my child have a healthy lifestyle after the Kasai?

By William Berquist, MD

I have a 9-month-old daughter who has biliary atresia. My question is why do some children survive with a Kasai and some cannot? My daughter looks so healthy. Yes, she is jaundiced but otherwise she is healthy. Her bilirubin is high, but it doesn't affect her. Is there anything I can do so my daughter can live a healthy lifestyle without being sick?

Biliary atresia is a complex disease that often involves the bile ducts inside the liver as well as outside the liver. The earlier a Kasai procedure is done, the more likely there will be a good response. A successful Kasai will result in loss of jaundice. There may be complications related to severe liver damage such as fluid retention, bleeding and infections. The best lifestyle after the Kasai would be to have your child take the prescribed medications and follow the diet recommended for children with liver disease. It will be important to obtain laboratory tests or imaging to follow the liver function and to have your child seen regularly by your health provider.

When will your child need a transplant?

by Jeffery Punch, MD

Timing for transplantation is very important, but unfortunately there are no absolutes to use as guides. Like the TV show "The Price is Right" where contestants try to guess as close as possible to the price without going over, one would like to wait as long as possible before doing a liver transplant, without waiting too long.

One must always keep in mind that a liver transplant involves removing the child’s own liver. From that moment on the child will be completely dependent on the donor liver. It’s too late to decide that child was actually better off with their original liver. Unlike kidney failure and heart failure, there is no artificial means of support if the new liver doesn’t do its job. While scientists are trying to develop an artificial liver, to date there is no system that performs the complex functions of a human liver reliably.

In general, if a child can live much longer (a year or more) without a transplant and still have as good a chance at survival, then a transplant would be too early. On the other hand, one waited too long if something happens which decreases the chance of survival after the transplant. Unfortunately, there is no accurate way to predict when such events will take place. As a child’s liver disease gets worse and worse it becomes easier to predict what will happen, but there is still no way to be accurate.

Some children will have good quality of life for many years with "compensated" liver disease, while others will not. For example in the case of biliary atresia, some children need a transplant within the first year of life, some not until elementary school age, and some not until adolescence or even into adulthood.

Compensated liver disease is when the disease exists but there are either few symptoms, or the symptoms are mild and stable. Decompensation is when a new and dire set of symptoms appears, especially if these new symptoms cannot be effectively treated. For example, a child with liver disease may develop ascites which can be effectively treated with a diuretic (water pill). The child is then said to have compensated liver disease. If after months to years the ascites suddenly will not go away even if diuretics are used, this would be decompensation. Patients are referred for transplant when they begin to show signs of decompensation.

Not every physician will agree on who is compensated and who isn’t. Like the terms "mild" and "severe," the terms compensated or decompensated are not the same in everyone’s eyes. Your physician should answer this question individually for your child, based upon his or her own experience and medical judgment.

Certain signs portend a bad prognosis, meaning that death from liver failure is likely within a year. These signs include encephalopathy, spontaneous bacterial peritonitis (SBP), and profound coagulopathy.

Encephalopathy is when the liver can no longer clear the wastes in the blood. These wastes build up and cause first a sort of sleepiness, then more profound lethargy and eventually coma.

SBP is when fluid called "ascites" collects in the abdomen, and this fluid gets spontaneously infected. It is thought that the infection may come through the walls of the bowels. The symptoms usually include abdominal pain and fever, but not always. Sometimes SBP will cause encephalopathy and this will be the only symptom.

Coagulopathy is when the blood fails to clot normally. The liver synthesizes the proteins in blood which cause blood to clot. When the liver isn’t making enough of the protein "factors," the patient has coagulopathy. Coagulopathy is measured by the pro-time (PT). When the PT is longer than normal (usually about 10 seconds is normal), the patient has coagulopathy.

In general, if the child’s overall health is not getting any worse, it is usually better to wait. But if they are not growing, or if they are having trouble with encephalopathy or ascites, and they are certain to continue to get sicker, they should be transplanted as soon as possible. It is always better to transplant before the child becomes very ill and needs an urgent graft. Urgent transplants always have worse success rates.

Some patients require transplantation even if their liver is functioning well. Examples include patients with tumors that cannot be removed without removing the entire liver, and children with certain diseases of metabolism due to inherited diseases. In these cases, the term "compensation" doesn’t really apply to their problem and their circumstance require unique considerations.

Fortunately, most children get the livers they need before they become critically ill. Living donor liver transplants (LDLT) continue to play an important role in solving the problem of timing. LDLT can be scheduled before the child becomes so sick their chances for a successful transplant are diminished. However, LDLT is not always the best option. As of 1998, the overall results for LDLT appears to be at least as good, if not better, for babies and small children compared to grafts from deceased donors (cadaveric grafts). But for larger children (over 40 pounds), the results for cadaveric grafts are at least as good if not better. For children who are nearing adult size (75+ pounds), the results for living donor grafts still looks to be inferior compared to cadaveric grafts. Different programs and doctors have different opinions regarding the issue of LDLT and the state of the art is constantly changing.

Dr. Punch is an assistant professor of surgery at the University of Michigan and member of the C.L.A.S.S. Scientific Advisory Committee.

Liver Transplantation in Children

by Dr. Esquivel

The first attempt at a human liver transplant was performed in a child with biliary atresia more than 30 years ago, but it was not until the discovery, clinical testing and adoption of cyclosporine in the late 1970's that liver transplantation began to receive acceptance worldwide. Better techniques in the donor and recipient operations, improved solutions for organ preservation and the introduction of potent immunosuppressant agents are other factors contributing to the progress of hepatic transplantation.

In children, the limiting factor is the shortage of donors. The current supply of organs is insufficient to meet the need and this problem is so serious that innovative techniques, such as the use of cutdown livers and living donor liver transplantation, have become routine procedures in large transplant centers. Considering these circumstances, the selection of patients and timing for liver transplantation is of utmost importance to ensure a successful outcome and prevent organ waste.

The goals in liver transplantation are to prolong life and to improve the quality of life. The ideal timing for transplantation depends a great deal on the underlying liver disease, the availability of other forms of therapy, the organ supply, and the expected outcome with liver transplantation. As an example, children with biliary atresia and progressive liver deterioration resulting in serious complications such as bleeding, muscle wasting, or the onset of rickets should undergo transplantation as soon as a suitable organ becomes available. On the other hand, the proper timing for a child with well compensated cirrhosis becomes more difficult to assess. In this situation, attention must be paid to subtle signs of liver failure such as a drop in the serum albumin, and increase in the prothrombin time or the development of ascites.

Diagnostic Indications

There are numerous indications for liver transplantation in children (chart); however, biliary atresia accounts for at least 50% of pediatric patients undergoing liver transplantation. The second most common reason for transplantation is a conglomerate of liver diseases which belong to inborn errors of the metabolism with alpha-1-antitrypsin deficiency being the most common among this group. Others are tyrosinemia, Wilson's disease and many others.

Contraindications

There are many contraindications or reasons why transplantation would not be performed and these are: (1) AIDS; (2) cancer outside the liver; (3) infection outside the liver; (4) technically not feasible and (5) other medical problems such as heart disease, lung failure, or epilepsy which would interfere with the success of the transplant.

Comments

Statistical formulas created by combining risk factors have been utilized as predictors of outcome. Although such formulas may be useful for the retrospective assessment of large volumes of data, they are often of little value on a daily basis. The child's condition must be assessed individually by a group of specialized physicians, including a transplant surgeon, pediatric hepatologist, and other consultants if needed. The consequences of chronic liver disease in children are devastating. Children with advanced liver disease should be referred promptly to a transplant center before they develop irreversible sequelae. Fortunately, liver transplantation is successfully performed in many medical center worldwide.

Dr. Esquivel is Professor of Surgery and Director of the Liver Transplant Program at Lucille Packard Children's Hospital at Stanford University and member of the C.L.A.S.S. Scientific Advisory Committee.

Development of young children following liver transplantation.

by Dr. Wayman

Liver transplantation has saved the lives of many young children in the last decade and has been welcomed by families and the medical community alike; but it is a highly technical medical intervention requiring lengthy hospitalizations and frequent procedures. Because of this, many parents have questions about the long term developmental effects of liver disease and transplantation. To answer these questions, the developmental specialist on the liver transplant team at Stanford University Medical Center is conducting a study that examines the developmental outcome of children with biliary atresia.

This study has focused on children transplanted before the age of two. This age group was selected because this is a time when many new skills emerge that could potentially be affected by liver disease and transplantation. Forty children were assessed with the Bayley Scales of Infant Development prior to transplantation and then 3, 12, and 24 months following transplantation. The scores from these assessments were then averaged to see if there were any general developmental trends. We found that prior to transplant, the children showed moderate delays in cognitive development and significant delays in gross motor development.

Three months after transplant the effects of surgery were evident and the developmental level of functioning dropped significantly for both mental and motor skill. However, the first few months following transplantation is a time of rapid recovery so that by the one year anniversary date, developmental functioning had returned to pre-transplant levels.

At 1 year post transplant, cognitive skills showed little change but gross motor skills tended to "catch up" to the level of cognitive functioning.

At 2 years post transplant, children continue to demonstrate some delays in both the cognitive and gross motor areas of development. Information for children 3 and 4 years post transplant is sketchy but it appears that the recovery process continues and the children continue to close the gap between developmental scores and their chronological age. Given the seriousness of the disease prior to transplant and invasiveness of the transplant surgery itself, these finds suggest that these children have a remarkable resiliency and have the potential to recover many developmental skills.

These findings raised another question: What specific developmental behaviors or skills are most affected by liver disease and transplantation?

It appears that major motor milestones and communication skills tend to lag behind other areas of development. For many children, the emergence of sitting, crawling, and walking was delayed by 3-6 months. One factor which may account for this is limitation of movement prior to transplant. This is a time when the liver can be enlarged or ascites may be present, therefore, these young children are reluctant to spend time on their tummy, sit upright or rotate their trunk—all positions or patterns of movement that are important prerequisites for strengthening the trunk muscles that support the child in walking. Furthermore, the transplant surgery severs all abdominal muscles which exacerbates the child’s already weakened trunk muscles. Following transplant, the children bounce back and begin to regain lost gross motor skills. However, we have found that intensive intervention prior to transplant and in the 3 months immediately following transplantation helps to support the child in the recovery of gross motor skill. This therapeutic intervention assures that as gross motor skills reemerge they do so with good quality of movements and normal patterns of development.

The other skill that tends to lag behind is expressive language. Expressive language is the child’s ability to verbalize his or her thoughts, needs and request. The children in our group appeared to understand the communicative attempts of adults and other children, but verbalization such as first words and 2 and 3 word phrases tended to emerge 4-6 months later than other developmental skills, after transplantation, most children seem to naturally "catch up" while others needed a little help with speech and language therapy. Children who had not caught up at approximately 1 year post transplant were referred to language therapy with good results.

The developmental picture for those children transplanted at age 3 and older appears to be somewhat different since language and gross motor skills are already in place at the time of transplant. Similar to the infants and toddlers, transplantation interrupts the developmental process, but the older children tend to recover their developmental skills more quickly with less impact on future skill development.

Many developmental questions remain, and I am sure this pioneering group of young children will continue to give us answers. My work with these children and their families has been intensely rewarding, and I want to tank them for their willingness to share information with me and other families so that together we can determine the best information and therapeutic interventions for helping future transplant patients.

Ms. Wayman is a Developmental Specialist on the liver transplant team at Lucile Packard Children’s Hospital at Stanford University Medical Center

Diagnostic indications for hepatic transplantation in children.

Chronic cholestatic liver disease

  • Extrahepatic biliary atresia
  • Arteriohepatic dysplasia (Alagille syndrome)
  • Neonatal hepatitis
  • Primary sclerosing cholangitis
  • Caroli's disease
  • Lanerhan's cell histiocytosis (histiocytosis X)

Inborn errors of metabolism

  • Alpha-1-antitrypsin deficiency
  • Tyrosinemia
  • Wilson's disease

Miscellaneous metabolic or genetic liver disease

  • Glycogen storage disease, type 1
  • Glycogen storage disease, type 4
  • Type 1 hyperoxaluria
  • Crigler-Najjar syndrome
  • Ornithine transcarbamylase deficiency
  • Familial hypercholesterolemia
  • Protein C deficiency
  • Niemann-Pick and other lipidosis
  • Gaucher's disease
  • Erythropoietic protoporphyria
  • Hemophilia A
  • Hemophilia B
  • Urea cycle enzyme deficiency

Hepatocellular injury

  • Viral hepatitis
  • Autoimmune hepatitis
  • Drug-induced liver failure
  • Amanita mushroom poisoning

Mass occupying lesions

  • Hepatoblastoma
  • Hepatocellular carcinoma (hepatoma)
  • Polycystic liver disease
  • Hepatic adenomatosis
  • Hemangioendothelioma

Miscellaneous

  • Cystic fibrosis
  • Congenital hepatic fibrosis

Living-related Liver Transplantation at UCSF

by Dr. Rosenthal

Although liver transplantation has become a highly successful and routine procedure for children with end-stage liver disease, access to cadaveric donor organs continues to be a major problem for children awaiting transplant. Innovative surgical techniques have been developed to address this serious problem. Reduced size or cutdown and split liver transplants, in which only a portion of a liver is utilized for the transplant, have been successfully incorporated into many transplant programs. Out of these techniques evolved living-related liver transplantation. In this procedure, part of a parent or relative’s liver, usually the left lateral or left lobe, is utilized for transplantation. Living-related liver transplantation has the potential of significantly improving the outcome for children awaiting liver transplantation. At the University of California, San Francisco Medical Center, living-related liver transplantation has been incorporated as an accepted and routine therapeutic option for all potential pediatric liver transplant recipients, and all families are presented with this alternative.

From May of 1992 to December of 1995, eighty-one potential pediatric recipients and 164 potential living donors were evaluated at UCSF. Biliary atresia was the most frequent diagnosis requiring transplantation in this group. Other indications included metabolic liver diseases, cholestatic liver disease, hepatitis, and fulminant liver failure. Candidate ages ranged from less than 1 year to adolescence. One hundred sixty-four potential living donors were evaluated. Of the 164 potential donors, 145 (88%) were interested in pursuing living-related liver transplantation. All were parents except for 2 uncles, 5 aunts and 2 grandparents.

The living-related liver transplant donor evaluation begins with a thorough history and ABO blood typing. If the donor is found acceptable at this point, additional laboratory work is ordered to be sure the donor is healthy and has no unsuspected liver disease. A chest x-ray, electrocardiogram and pulmonary function tests to assess the heart and lungs are also obtained. If the donor passes these tests, an abdominal CT scan and hepatic angiogram to evaluate the anatomy of the proposed donor organ is performed. This entire evaluation is performed by a physician not involved with the liver transplant program to insure that the best interests of the potential donor are being considered.

Seventy-six of the 145 potential donors (52%) were found to be acceptable in our program, and 69 (48%) were excluded. Reasons for exclusion included ABO blood group incompatibility in 16 (11%), medical contra-indications in 30 (21%), and for social reasons in 23 (16%). Medical contra-indications for potential donors included hepatitis, heart disease, hypertension, diabetes, substance abuse and obesity.

Of the 81 potential pediatric recipients, 20 received living-related liver transplants (25%), 31 received cadaveric transplants (38%), 3 expired awaiting a donor organ (4%), and 27 (33%) remain listed awaiting a donor organ. Of these 27, five have completed the living-related transplant evaluation.

The average length of stay for living-related liver transplant donors is one week, and donor survival is 100%. Living-related liver transplant donor complications have been few and include one case of biliary leak corrected by endoscopy, and one case of chronic gastritis which was responsive to medical therapy.

Immunosuppression following liver transplantation is identical in our living-related and cadaveric transplant recipients. The incidence of rejection is comparable in the living-related and cadaveric liver transplant groups.

At the University of California, San Francisco Medical Center, survival following liver transplantation in children is over 92% at one year post transplant. Living-related liver transplantation is an effective therapeutic option that should be considered for all children requiring liver transplantation.

Dr. Rosenthal is Professor of Pediatrics and Surgery, University of California, San Francisco Medical Center and member of the C.L.A.S.S. Scientific Advisory Committee.

Development of the Pediatric Liver Transplant Specific Scale

by Ross Andelman, Md and Philip Rosenthal

With the generous support of the 1998 Elizabeth Azzara Research Grant from C.L.A.S.S., we developed two questionnaires, one for parents and one for children over eight years of age, to measure the quality of life of children and adolescents after liver transplantation. We then used this questionnaire, the Pediatric Liver Transplant Specific Scale (PLTSS) to assess the quality of life of those youth between 6 and 18 years of age, cared for in the UCSF liver transplant program.

We defined quality of life as the subjective appraisal of one’s life and of pertinent life dimensions, including physical, emotional, and social well being, as well as role performance and environmental adequacy. Given the subjective nature of this area, we sought to develop these questionnaires directly from the perspectives of the children with liver transplants and their parents. To do this, we conducted focused discussion groups with adolescents with transplants and their parents. The ideas generated in the focus groups provided the basis for the items in the PLTSS.

There was consensus between parents and youth participating in the focus groups in some areas but considerable divergence as well. Both parents and youth expressed concern about the youth’s participation in school and extracurricular events, about developing peer relationships, about the youth feeling ostracized and excluded, and about the side effects of medications. Transplant recipients endorsed the emotional burden of the transplant, including anxiety over laboratory results, anger about having to have a transplant, and fear of graft rejection. Parents focused on emotional developmental issues — becoming independent and building self-esteem, and on normalizing social interactions. After initial analysis, the PLTSS consisted of 27 items with seven sub-scales, including Invulnerability, Peer Relations, Peace of Mind, Positive Outlook, Empathic Attention, and Medication Issues.

We combined the PLTSS with generic quality of life questionnaires to broadly and comprehensively assess the quality of life of children with liver transplants. This ‘battery’ approach allowed comparison of these youth, and children without transplants, as well as more in-depth examination of the impact of liver transplantation. Questionnaire packets were completed by 48 (84% response rate) of the parents asked and 34 (87% response rate) of the children asked.

We also administered the PLTSS to a comparison group of 58 pairs of children and parents.

On the PLTSS, parents of children with liver transplants generally rated their children’s quality of life as no different than that of children without transplants. Parents in the liver transplant group did report that, relative to the comparison group, their children felt more vulnerable and had missed out on more social events. On the other hand, children with liver transplants rated their overall quality of life as good, but worse than the comparison group. Children with liver transplants described themselves as more vulnerable and less hopeful than the comparison group. Youth with transplants also felt less positive about the sensitivity of adults in their lives than did the comparison youth.

Other measures used in the battery of questionnaires provided a more complex picture. On one generic measure of life satisfaction, youth with liver transplants generally reported high levels of satisfaction though less satisfaction with themselves and with their friends than the control group. On the other hand, parents rated their children with liver transplants as having greater emotional and behavioral strengths than a national comparison group.

In the final analysis, parent and child reports provided a different picture of the relative quality of life of children with liver transplants. The difference between parent and child scores could reflect parental hopefulness, a projection of parental concern for their children, the loneliness of these youth, and the youth’s inability to share all of their emotional concerns with their parents. On a more fundamental level, these differences suggested the need to obtain information from both parents and youth.

This initial investigation provides evidence of the importance of assessing the quality of life (QOL) of children following liver transplantation and the need for further research in this area. The study also provided initial support for further use of the PLTSS to assess youth following liver transplantation. The prominent themes uncovered in this study — fear of graft rejection, the impact of immuno-suppression, and the empathic attention of adults – suggest that some of these youth could benefit from formal psycho-social assessment and interventions.

The purpose of this study was the development of an instrument to assess the QOL of children following liver transplant. QOL is a broad index of outcome that challenges the clinician to look beyond the more traditional but limited outcome measures of morbidity and functional status. For the pediatric hepatologist and the families they treat, a better understanding of the correlates of QOL for children after liver transplant may result in the development of medical interventions with fewer side effects and more attention to family support and education to limit anxiety and fear.

The UCLA Pediatric Liver Transplant Experience Utilizing In-Situ Splitting Of The Cadaveric Liver

by John A. Goss, MD, and Ronald W. Busuttil, MD PhD

C.L.A.S.S. Notes, Fall 1997 -- For close to 15 years orthotopic liver transplantation (OLT) has been established as the definitive therapy for patients with end-stage liver disease. During this evolutionary period, improved patient and graft survivals have been achieved and thus a natural expansion of the list of indications for the procedure has occurred.

The full potential for liver replacement is far from being realized, however, due to the widening disparity between the number of potential recipients and a constant donor supply. This is clearly illustrated when considering that 7,279 patients were listed for hepatic transplantation in 1995 while only 3,922 donor livers were available. The donor shortage is particularly critical for children and small adults.

Several novel approaches have been developed in an attempt to alleviate the organ shortage in children. These have primarily involved reduced-size allografts and living-related liver donation. The former, however, does not increase the scarce donor organ resource and the latter still has unresolved concerns about the safety of the living-donor.

Split liver transplantation, in which an adult cadaveric liver is divided into two functioning allografts, not only overcomes the drawbacks of living-donor grafts but also increases the total number of donor organs.

The concept of ex-vivo split-liver transplantation was introduced clinically in 1988 by Dr. Pichlmayr. While early reports described the feasibility of this technique, patient and graft survival rates were disappointing and associated with biliary complications, intra-abdominal hemorrhage, and primary nonfunction of the right graft.

A modification of the ex-vivo technique is in-situ splitting of the liver. In-situ splitting of the liver is an extension of the techniques established for living-donor procurement and is applied to the heart-beating cadaveric donor. Dr. Rogiers, et al, initially described the in-situ splitting of the cadaveric donor liver and reported lower rates of biliary complications, intra-abdominal hemorrhage, and nonfunction of the right-sided liver allografts as compared to other series utilizing the ex-vivo split liver techniques.

We first attempted in-situ split liver transplantation at UCLA in 1992, prior to our establishment of a living-donor liver transplant program. Our experience was not favorable. However, after successfully establishing a living-donor liver transplant program we once again began a split liver transplant program in July, 1996, using the in-situ methods which had been established in the living-donor procurement. Since that time we have selected 27 livers from hemodynamically stable donors with a median age and weight of 17 years (range 6 - 36 years) and 58 kg (range 24 - 76 kg), respectively for in-situ splitting. This splitting technique has been performed without technical complication and resulted in 27 right trisegmental and 27 left lateral segment grafts. These grafts have resulted in 49 transplants performed in 45 patients (22 adults and 23 children) with an age range of 4 months to 62 years and a weight range of 4.1 - 77 kg respectively.

The most common etiology of end-stage liver disease in the pediatric population was congenital biliary atresia.

At the time of transplantation, seven children were confined to the intensive care unit (United Network for Organ Sharing [UNOS] status 1), six patients were hospitalized (UNOS status 2), and 11 pediatric patients were awaiting liver transplantation at home (UNOS status 3).

Of the 23 children undergoing in-situ split liver transplantation 22 are currently alive. The overall 6-month and 1-year actuarial patient survival rates were 96% and 96%, respectively; the 6-month and 1-year actuarial graft survival rates were 86% and 86%, respectively. Three of the pediatric recipients had to be retransplanted, 1 for hepatic artery thrombosis, 1 for primary nonfunction, and 1 for biopsy proven antibody-mediated allograft rejection.

In-situ liver splitting has virtually eliminated the waiting time for small infants on our waiting list and has reduced our need to resort to living-donor liver transplantation.

The average UNOS waiting period for liver transplantation in a child less than 1 year of age and 1-5 years of age outside UCLA is 197 and 128 days respectively. At UCLA these waiting times have been decreased to 24 days and 30 days respectively, since initiating our in-situ split liver transplant program. Only 3 living-donor liver transplants have been performed during this same time period (1 case for antibody-mediated rejection, 1 case for fulminant hepatic failure, and 1 case of biliary atresia with upper gastrointestinal bleeding).

With these data in mind, in-situ splitting of the adult cadaveric liver has become our technique of choice for pediatric transplantation. It has enabled us to transplant children prior to them becoming critically ill and allowed us to avoid hepatectomy on a family member.

Dr. Goss is a transplant surgeon and assistant professor of surgery at the Dumont-UCLA Transplant Center and member of the CLASS Scientific Advisory Committee.

Dr. Busuttil is Director, Dumont-UCLA Transplant Center and member of the CLASS Scientific Advisory Committee.

Coping With the Stressors of Transplant

by Betty Kim-Liu, MSW

Caring for a child with an illness can be overwhelming and seem unmanageable at times. You may have experienced a range of emotions when your child was first diagnosed with a serious illness. Now that your child has been recommended for a possible liver transplant, this information may likely create additional concerns and anxiety.

The waiting period for the donor liver, whether it is a few weeks or several months, can seem extremely wearisome and can create a sense of fear. When your child is on the waiting list for only a few weeks, you may think you didn't have enough time to prepare. On the other hand, if your child has waited for several months, your concerns and anxiety may increase as your child's illness progresses.

Often, worrying can stem from a lack of knowledge or not knowing what to anticipate. You are not alone. These feelings and concerns are appropriate for what you are experiencing, given your child's illness.

There are things you can do to help redirect your worrying into something productive. You can refocus some of your anxiety and strengthen your ability to cope with the following tips:

1. Get to know your team.

The interdisciplinary liver transplant team usually consists of the Pediatric Gastroenterologist, Surgeon, Nurse Coordinator and Social Worker. You may also interact with a Child Life Specialist, Dietitian, Psychologist, Chaplain, Physical Therapist, Pharmacist, and Nursing Staff. Talk with the members of your liver transplant team and identify each of their roles. Each person on the team has distinctive knowledge and information, which will be helpful throughout your child's transplant process.

2. Get to know your transplant center.

Request a tour of the medical facility to help address questions you or your child may have in preparing for future hospitalizations. What do the rooms and intensive care units look like? Where will my child have his/her exams? Where can I get something to eat? Where can family members sleep? Where can we take a shower? Find out about resources available at the hospital and the neighboring area. It is often helpful to contact the Pediatric Liver Transplant Social Worker prior to your initial admission to the hospital/transplant center or evaluation process to help you identify these resources.

3. Educate yourself about your child's illness.

Learn about the functions of the liver. What caused your child's liver failure (if known)? What are the symptoms to look out for? What are the medical or surgical options to help your child as his/her condition progresses?

4. Learn about the transplantation procedures.

What are the pre- and post-transplantation procedures? What kinds of tests will be involved? How does my child get on the waiting list? How long is the wait? What is a PELD score? How will we be contacted? How long will the hospitalization be? How often will we go to clinic? How many medications will my child be taking? Who do we contact in case of an emergency? Your transplant team will welcome your questions and are there as a resource to you.

5. Get organized. Develop a "care notebook" as a reference/resource guide.

If possible, request information in writing. Have a list of all the contact people at your transplant center in one place or sort all your business cards in a sleeve. Take notes and write down questions for the team. There is a great deal of information and much to learn. It can be easy to get overwhelmed. It is simple to overlook or fail to remember some of the details. Keep a calendar of appointments, lab schedules, and dates when procedures were performed. Keep copies of all medical consents for test/procedures.

6. Get support.

Emotional support is vital during this time. Your family and friends can be excellent support givers and may also be able to handle day-to-day activities, such as childcare, house sitting, getting mail, etc. Your employers may also be supportive during this difficult period and may be able to grant you FMLA Leave (Family Medical Leave Act) to be with your child or for visitation. You may also consider contacting other parents who have experienced something similar. Ask your social worker for information on support groups or resources such as C.L.A.S.S.

Explore areas where you and your family may need more assistance such as help regarding disciplining your child, school issues, appropriate social activities, sibling support or financial issues. Research community agencies or resources, which may be helpful. Parents may also face marital and financial problems. Therefore, it is important to consider asking for and accepting assistance during this time.

7. Take care of yourself.

Make sure you eat well, sleep regularly and remember to take some time for yourself. Good physical and emotional care during this time will help you care for your child more effectively. This will also permit you to fully enjoy your child and your family. If you notice you are having difficulty coping, you may consider talking with a professional about your physical and emotional health during this period.

No matter what your situation is, support for your child and getting support for yourself is essential. Enjoy your child and your family every moment. Facing a serious illness together can be enriching as well as stressful and allows families to appreciate how precious and valuable life is.

Betty Kim-Liu, MSW, is the Clinical Social Worker for the Pediatric Liver Transplant and Pediatric Small Bowel Transplant Team at Mattel Children's Hospital at UCLA Medical Center.

What kind of training is needed to become a transplant surgeon?

by Jeffrey D. Punch, MD

I’m writing a paper for my careers class and I would like to know what training is required to become a transplant surgeon.

Becoming a doctor in the U.S. generally implies graduating from a 4-year college followed by 4 years of medical school. During the third year of college students take a standardized test called the "MCAT" (Medical College Admission Test) and apply to medical schools. After graduating from college, usually with a bachelor’s degree, medical school begins.

Medical school is 4 years long and generally begins with classroom teaching in the "basic sciences": anatomy, physiology (how the body works), biochemistry, microbiology (the study of infectious diseases), pharmacology (the study of medicines and drugs), neuroscience, and pathology (the study of human disease).

The later years in medical school involve clinical rotations where the student observes doctors taking care of patients and learns physical examination and basic procedures. These rotations include internal medicine, surgery, pediatrics, obstetrics and gynecology, psychiatry, neurology, and may include specialties like radiology, emergency medicine, ophthalmology, etc.

After graduation from medical school the student is granted an MD degree and the title "doctor." The training is not over yet, though.

After medical school comes internship, which last for one year. Internship may be an integrated part of a training program in a particular discipline like general surgery or internal medicine, or it may be a "rotating internship" which includes training in several areas of medicine like pediatrics, obstetrics, internal medicine, emergency medicine, etc. Rotating internships are usually required before training programs in anesthesiology, radiology, pathology, ophthalmology, and other specialties.

Internal medicine is a residency that is 3 years long and usually includes an integrated first year internship. To become a specialist in a field of internal medicine, such as cardiology (heart disease), nephrology (kidney disease), Gastroenterology (diseases of the gut), etc., one does a fellowship that is 2-3 years long after completion of a residency in internal medicine. Some specialists also become "subspecialists" by doing additional training in specific areas such as "transplant cardiology," (the care of heart transplant patients), or hepatology (the care of patients with liver diseases or liver transplants).

Pediatrics is similar to internal medicine in that the residency takes 3 years. Specialists in pediatrics also do additional training in pediatric cardiology, pediatric nephrology, etc.

Surgical training begins with a residency that is 5-6 years long and usually includes an integrated internship the first year. Surgical residencies include neurosurgery (brain and nerve surgery), orthopedics (bone surgery), ophthalmology (surgery on the eyes), otolaryngology (surgery on the ears, nose, and throat) and general surgery (all other parts of the body not covered by the surgical specialties listed above).

Some surgical specialties require a full 5 year training in general surgery prior to their 2-3 year long fellowship training. Examples of this kind of training include plastic surgery, transplantation (usually kidney, liver, and pancreas), thoracic surgery (surgery on the chest, heart and lungs, including transplants), pediatric surgery, and trauma surgery.

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

Is it possible to use an ABO incompatible liver graft from a living donor?

by Jeffrey D. Punch, MD

I’ve heard that it’s possible to use ABO incompatible organs for liver transplantation. My child is blood type O. Which blood types will be compatible with his? I am blood type A, will I be able to give him part of my liver?

The only "ABO compatible" donor for a person with blood type O is another type O.

ABO compatibility is a very important factor for heart and kidney transplants. Virtually 100% of ABO incompatible kidneys will be rejected within minutes. ABO compatibility is less important for liver grafts, but ABO compatible liver grafts are more likely to work than ABO incompatible grafts.

Since we always want to give each patient the best possible chance to survive, we always use ABO compatible liver grafts unless the only available liver is ABO incompatible and the patient is likely to die if they don’t get this liver. Only in the case of a very serious emergency would a liver from a donor with blood type A be used for a type O recipient.

Although an ABO compatible graft is always preferred over an ABO incompatible graft, there are hundreds of people alive today with ABO incompatible liver grafts. But, to date, I am not aware of any instances where an ABO incompatible liver transplant has been done from a living donor.

ABO Blood Type Compatibility

Blood type Can receive a liver from: Can donate a liver to:
O O O, A, B, AB
A A, O A, AB
B B, O B, AB
AB O, A, B, AB AB

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

Is it possible for a child to have a different blood type than either of the parents?

by Jeffrey D. Punch, MD

My husband and I just completed the ABO blood typing for a possible living-related liver transplant for our child. We were shocked to learn that while we are both blood type B, our child has type O blood. How is this possible?

To understand this, you need to understand blood type genetics. There are four basic blood types, in order of frequency from most common to rarest they are: O, A, B, and AB. Blood type is determined by the "alleles" that we inherit from our parents. Alleles are possible types of a particular gene, in this case the blood type gene. There are three basic blood type alleles: A, B, and O. We all have two alleles, one inherited from each parent. The possible combinations of the three alleles are:

  • OO
  • AO
  • BO
  • AB
  • AA
  • BB

Blood types A and B are called "codominant" alleles, while O is "recessive." A codominant allele is apparent even if only one is present; a recessive allele is apparent only if two recessive alleles are present. Since blood type O is recessive, it is not apparent if the person inherits an A or B allele along with it.

So, the possible allele combinations result in a particular blood type in this way:

  • OO = blood type O
  • AO = blood type A
  • BO = blood type B
  • AB = blood type AB
  • AA = blood type A
  • BB = blood type B

You can see that a person with blood type B may have a B and an O allele, or they may have two B alleles. If both parents are blood type B and both have a B and a recessive O, then their children will either be BB (if each parent passed on the B allele), BO (if one parent passed on B and the other parent passed on O), or OO (if both parents passed on the O allele). If the child is BB or BO, they have blood type B. If the child is OO, he or she will have blood type O.

When you understand blood type genetics, you can see that it is not at all unusual for two parents with blood type B (or blood type A) to have children with blood type O.

Parents' Blood Types Possible Children Impossible Children
A & A A, O B, AB
A & B A, B, AB, O none
A & AB A, B, AB O
A & O A, O B, AB
B & B B, O A, AB
B & AB A, B, AB O
B & O B, O A, AB
AB & AB A, B, AB O
AB & O A, B AB, O
O & O O A, B, AB

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

How does living donor liver transplantation (LDLT) compare to living donor kidney transplantation (LDKT)?

by Jeffrey D. Punch, MD

How does living donor liver transplantation (LDLT) compare to living donor kidney transplantation (LDKT) in terms of difficulty, chances of success for the recipient, compatibility issues, level of risk and recovery time for the donor, etc.? How many living donor liver transplants have been performed to date?

Removing a section of a volunteer donor's liver is a probably a riskier undertaking compared to removing a kidney. However, since the procedure is relatively new in comparison, there are insufficient data to quantify the risk.

To date there have been more than 60,000 reported live kidney donations, and there have been about 20 reported donor moralities, for an estimated risk of ~0.03%. Comparable data for living donation of the liver are not available, but the risk is believed to be on the order of 0.5-1.0%.

Generally the same ABO compatibility issues are present for liver donors as for kidney donors, but the number of antigen matches with the recipient does not seem to matter as much as it does for kidney recipients.

Regarding the chance of success, the graft survival rate for living donor kidney transplants is far superior to the graft survival rate for living donor liver transplants. Recovery time for the donor should be roughly similar for liver and kidney donors. The liver grows back to normal size after a segment is removed, and in this sense it is unique among solid organs. The remaining kidney of someone that has a kidney removed generally enlarges and functions at about 80% of what someone with two normal kidneys would.

The 1997 UNOS Annual Report lists the number of liver transplants from living donors through 1996 in Table 1. The peak was 60 in 1994. From 1989 to 1996 there were ~250 living donor liver transplants and about 24,000 liver transplants from cadaver donors.
-Jeffrey Punch, MD

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

Is there a list of do's and don'ts for transplant patients?

by Jeffrey D. Punch, MD

My son was born with biliary atresia and was transplanted almost 4 years ago. He is now 4 1/2 years old and doing great. Are there any foods which he should not have? I've heard that he is not allowed unpasteurized food, but is there anything else I should not give him? Is there a list somewhere of the "do's and don'ts" of foods, activities, animals etc. that could help guide us? He's starting Kindergarten this year so we know he will be exposed to a lot so if you have any info it would be helpful.

You need to pose these questions to whomever is following him from a transplant point of view. There are no generally agreed upon rules. Each patient is somewhat unique. For example, sometimes the spleen will remain somewhat enlarged, other times it does not. Sometimes the bones have been thinned by steroid use, sometimes not. I generally don't place any diet restrictions on patients unless they have high blood pressure, in which case they need a low salt diet. I don't restrict activity as long as the spleen is small and overall health is good (which it usually is). Pets aren't a big problem but some sorts of infections can be caught from cat and bird feces, so transplant recipients generally shouldn't be involved in the cage cleaning sorts of activities, but they can have a cat if they want. Again, each patient needs to have individualized advice- some patients are far more immunosuppressed than others.

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

What is Epstein-Barr Virus (EBV)?

by Jeffrey D. Punch, MD

I was a living donor for my son and I tested positive for EBV. So far, our son has tested negative and sometimes borderline positive for EBV. He had IV ganciclovir for 100 days post-transplant and is now on oral acyclovir four times a day for a year. How long do we need to be concerned that he may get full blown EBV, and what will that mean to him medically if he does?

EBV is the short name for Epstein-Barr Virus infection, also called Mononucleosis, "Mono," and the "kissing disease." This virus is a member of the family of Herpes viruses that includes Herpes Simplex, which causes "cold sores" on the lips and mouth as well as genital Herpes; Herpes Zoster which causes Chicken Pox and Shingles; and CMV or cytomegalovirus, a virus that commonly infects post-transplant patients.

About 85% of adult Americans will test "positive" for EBV antibodies, which means they have been exposed to the disease in the past. All of the diseases in the Herpes family share the common element that the infection can occur multiple times. This process is called "reactivation." It happens because the body doesn’t really eliminate the virus; rather the virus becomes dormant in cells, only to reemerge at a later date. Since the virus is not eliminated, organs from anyone who has ever been exposed to EBV can transmit the virus to the recipient.

So to answer your question truthfully, the concern about EBV persists indefinitely. About one half to two thirds of EBV infections are in the first year and the rest occur after the first year.

At present, the major concern regarding EBV infection is that it may later develop into lymphoproliferative disease (LPD), which is a malignant condition (cancer). However, the virus also may be responsible for chronic fatigue in transplant and healthy patients, and occasionally it can cause hepatitis and damage the liver.

In four recent studies that looked at EBV infection in liver recipients, the incidence of EBV infection was between 4 and 20%.

The major risk factor that seemed to increase the chance of getting EBV was having treatment for rejection with immunosuppressive antibodies, such as OKT3 and ATGAM. Also, the younger groups seem to have the highest risk. Finally, there seems to be more EBV infection in children treated with tacrolimus (Prograf) compared to cyclosporine; however, most patients who were treated with tacrolimus had a higher dosage than is currently in use. This may explain the higher incidence of EBV infection.

It is unclear whether prophylactic treatment with anti-viral drugs, such as ganciclovir and acyclovir, decreases the risks of getting an EBV infection or not.

Warning signs of EBV infection include an unexplained fever, lethargy and fatigue, and swelling of lymph node tissue. Lymph nodes are normal collection of immune cells that fight infections. The lymph node "lumps" are most noticeable in the groin area, under the arm pit, and in the neck. Enlargement of these nodes happens normally to fight infection. For example, the nodes in the neck under the jaw will swell when one has a sore throat. If the swelling does not resolve when the sore throat does, this is cause for concern.

Diagnosis of an EBV infection can be difficult. The most commonly used method of diagnosing EBV is by monitoring for the presence of an antibody reaction to the virus. However, this test may not be positive early in an infection, and it may remain positive long after eradication of the virus.

In many cases it appears that EBV infection can be controlled with anti-viral therapy and a concomitant reduction in immunosuppression. But because it is hard to tell who has EBV and who doesn’t, it is hard to measure for sure the effectiveness of the anti-viral therapy.

Between one and two thirds of patients who develop EBV infection will develop lymphoproliferative disease (LPD). Treatment for LPD is not very effective and it is fatal in between 40% and 80% of cases. The thing about LPD is that most people don’t think of it as an infection but as a situation where the patient is or has been "over-immunosuppressed." The best way to prevent LPD may be to avoid "over-immunosuppression."

Refs:

Ho et al. The frequency of Epstein-Barr virus infection and associated lymphoproliferative syndrome after transplantation and its manifestations in children. Transplantation (1988 Apr) 45(4):719-27

Cox et al. An increased incidence of Epstein-Barr virus infection and lymphoproliferative disorder in young children on FK506 after liver transplantation. Transplantation (1995 Feb 27) 59(4):524-9

Newell et al. Posttransplant lymphoproliferative disease in pediatric liver transplantation. Interplay between primary Epstein-Barr virus infection and immunosuppression. Transplantation (1996 Aug 15) 62(3):370-5

Langnas et al. Epstein-Barr virus hepatitis after liver transplantation. Am J Gastroenterol (1994 Jul) 89(7):1066-70

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

Can doctors be certain a donor liver is good?

by Jeffrey D. Punch, MD

Can doctors be certain that a donor liver is "good" and how can they be sure?

It is not possible to be certain that a liver will be a good one. However, it is possible in many cases to tell when a liver will be a bad one. By a bad liver, I mean that it will not function immediately in the recipient. If the liver donor has a high serum sodium level, or if the liver has fatty infiltration, it is known that the likelihood that the liver will work well following transplantation is much lower. For this reason if the sodium is high enough or if there is too much fat in the donor liver, it is often not transplanted. The other factor that is known to be associated with poor liver function is the appearance of the donor liver. In other words, the subjective impression of the donor liver by an experienced transplant surgeon is also important. Livers that "don't look good" have rounded edges instead of sharp, distinct ones. They also may have a swollen appearance and/or an abnormal color. Even when none of these factors are present there is a chance that the liver will not work in the recipient. This is called primary non-function. It occurs after about 5% of liver transplants overall. In these cases, an immediate and emergent re-transplant will be required.

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

Are patients with liver cancer eligible for liver transplantation?

by Jeffrey D. Punch, MD

My grandson, age 3, was diagnosed with hepatoblastoma April 1999. The liver is the primary and only location of the cancer. He has undergone chemo and had a liver resection removing 70% of his liver. In January 2000, a small area was detected which had begun to grow again.

Some doctors have said he is eligible for a liver transplant, others have said he is not. Our angel has a mild genetic syndrome (Rubinstein-Taybi) which mainly is evident in slow growth. It is a very livable syndrome. What we are wondering: is he being discriminated against receiving a liver transplant because of this syndrome?

It really isn't possible to pass judgment regarding a particular case without seeing the patient for a complete evaluation. I can say with certainty that hepatoblastoma is a well recognized indication for liver transplantation. The fact that it has "reappeared" is something of a negative prognostic sign, but many surgeons would not consider that an absolute barrier to proceeding with a transplant.

I am not familiar with the Rubinstein-Taybi syndrome. If the issue is mild mental retardation, that should not preclude him from benefiting from a transplant. If the mental retardation is profound, the issue becomes more difficult, but it becomes a judgment call. The description of the child sounds as if this shouldn't be an issue, but it isn't possible to say this without meeting him. If the issue is that the syndrome is associated with other anomalies that either complicate the operation (such as cardiac problems) or that limit his life span otherwise, the decision to proceed with a transplant becomes very complex.

As always, a second opinion is always a good idea if there are doubts about the medical decision making process.

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

What are my chances of living if I have a liver transplant?

by Jeffrey D. Punch, MD

National statistics for children that have had liver transplants show that about 85% survive one year. After the first year, survival is about 98-99% each year. These numbers only apply to a group of people, not to one person. If we have a hundred people in a room and they all get a transplant, we can predict accurately that 85 of them will live for at least a year. Unfortunately, we can't tell ahead of time who will live and who will not. Keep in mind that liver transplants are only done for serious conditions that offer worse survival odds than a transplant.

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

How long is the life expectancy of children with liver transplants?

by Jeffrey D. Punch, MD

I have a 2½-year-old son who received a liver transplant a little more than 18 months ago. How long is the life expectancy of children with liver transplants? I don’t feel as though I’ve ever received a straight answer on this question.

This is a commonly asked question, but there is no legitimate way to answer it authoritatively since the success of liver transplantation is too recent and the results continue to improve. The majority of children that survive the first year after a transplant are probably destined to live into adulthood. Some will undoubtedly die of complications related to the transplant though, and others will surely live into middle age and longer.

The term "life expectancy" is really a misnomer. When people think of a life expectancy of say, 75 years, they think of a bunch of people living into their 70’s and then dying. It doesn’t work like that at all. For every person that dies 50 years early at age 25, two people must live to 100 in order to reach an average life span of 75 years. Knowing the "average" life span of a particular group says very little about how long an individual within that group will live.

Today’s methods of maintaining long term graft survival are imperfect, and the methods themselves produce harm. The secret to normalizing life expectancy will be "graft tolerance": the ability to fool the immune system into not attacking the transplanted organ without using chronic immunosuppressive medications. This could be just around the corner (in the next five years), or it might never happen. Science cannot be predicted very well either.

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

What kinds of special precautions are needed to protect against infections after a transplant?

by Jeffrey D. Punch, MD

We realize that after our daughter’s liver transplant her immune system will need to be suppressed to prevent rejection of her new liver. But we worry about how vulnerable this will leave her to the threat infectious diseases. What kinds of special precautions will we need to take to protect her?

The body has multiple methods of defending against infection: antibodies, lymphocytes, neutrophils, macrophages, "natural killer cells" and more. Some defend primarily against viruses, others work better against bacteria, parasites or fungi.

To the body, a transplanted organ is probably more like a virus than a bacteria — it is a normal cell that has weird things on its surface. For this reason our immunosuppression strategies (cyclosporine and tacrolimus) tend to mostly affect the type of immunity that keeps us from getting viruses. That is why transplanted patients are very susceptible to viruses. To a lesser extent there is also some increased susceptibility to bacterial infections.

Precautions against infection must be individualized for each patient. Different patients are on different amounts of immunosuppression. Different transplant centers have different immunosuppression systems. Different regions of the country can have different infection risks. Each patient must ask their own transplant team for their advice. The following comments apply to transplant patients in general.

Highest Risk

The more immunosuppression a patient has received the more precautions are necessary. Since the chance of rejection is highest during the first 3 months after a transplant, higher doses of immunosuppression are used, and therefore the risk of infection is highest during this time period.

When patients have an episode of graft rejection it is necessary to increase the immunosuppression and this will often markedly increase the risk of infection. Antibody drugs such as ATGAM, OKT3 and Zenapax are given either at the time of a transplant in order to prevent rejection or to treat established rejection. These drugs must be given intravenously. They are very powerful immunosuppressants and markedly increase the risk of infections for months after they are given.

Viral Infections

As noted, the most common infections after a transplant are viral infections. Patients who are on immunosuppression are always better off staying away from anyone that is ill, especially if that illness is a viral disease. On the other hand, there is usually no point to trying to accomplish this within a household because by the time the recipient’s sibling is ill, the recipient has already been exposed.

Viral infections can be new infections such as cold, chicken pox, flu, and intestinal viruses, or they can be reactivation of viruses the patient has already had that were dormant until immunosuppression allowed them to re-emerge. This is especially true for the first few months after a transplant.

A hard issue is daycare. Clearly all young children in daycare have more infections than children who stay at home all the time. Again, transplant centers are going to vary, and advice is going to vary depending on the level of immunosuppression the child has received. Daycare is usually permissible, but some centers will advise that the patient not go to daycare during the particularly vulnerable period immediately after the transplant.

Bacterial Infections

Bacterial infections such as pneumonia and urinary infections are also fairly common after a transplant and the majority can be easily treated with antibiotics.

In general, pets are allowed, but again one should check with one’s own physicians. It is usually recommended that transplant recipients should not come in contact with the feces of cats because of the theoretical risk of encountering a dangerous parasite called "Toxoplasma" that can be carried by cats. This means that a cat in the house is okay, but someone else should change the kitty litter. Dogs are usually okay, but allowing the dog to "kiss" should be discouraged. Birds are also known to carry parasites and it is usually recommend that recipients not clean bird cages or hold the bird.

When it has been many years since a transplant special precautions are often not necessary for most transplant recipients, but this is not true in all cases.

Chicken Pox

Chicken pox is a special case. All transplant patients are checked for the antibody to chicken pox (which is caused by the virus varicella zoster) prior to transplantation. If the patient is immune to chicken pox, the likelihood of having a problem later on is much less since this virus generally causes chicken pox only once. However, reactivation is possible, but very rare, when severely immunosuppressed. Also, the virus can reactivate later in life as shingles, a viral infection in the nerves. This infection can lead to chronic pain even after the viral infection has ceased.

A new chicken pox infection is a very serious matter for a patient on immunosuppression because the virus can cause more than the usual rash in these patients. It can cause a viral pneumonia or hepatitis, and these problems can be life threatening.

A vaccine is available for chicken pox and many transplant centers are starting to vaccinate children who will some day need a transplant in order to generate immunity and prevent future infections. The vaccine is a live virus though, so it cannot be given once the child is on immunosuppression. If a transplant recipient who is not immune to chicken pox is exposed to someone infectious for chicken pox, many centers recommend an injection of varicella zoster immune globulin. This immune globulin is an antibody against the virus and will prevent infection, but it must be given within 72 hours of exposure to be effective.

If a child on immunosuppression comes down with chicken pox it is usually recommended that they receive an antiviral medication called "acyclovir" intravenously until the virus clears as evidenced by the crusting over of the sores.

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

What are the signs and symptoms of rejection?

by Jeffrey D. Punch, MD

Our 14-year-old daughter received her new liver earlier this year. She hasn’t had any rejection episodes yet, but I know it is common and I worry constantly about it. What exactly are the signs and symptoms of rejection?

Here is a list of signs and symptoms that may indicate liver graft rejection:

  • Fever greater than 100°
  • Fatigue or excess sleepiness
  • "Crankiness"
  • Headache
  • Abdominal swelling, tenderness, or pain
  • Decreased appetite
  • Jaundice (yellow skin or eyes)
  • Dark (brown) urine
  • Itching

None of these symptoms are specific for rejection; but they are important enough that when they occur, they should prompt a call to your transplant center. Most centers have transplant nurse specialists who take such calls and decide whether the situation warrants further investigation or should be observed for the time being.

It is very important to realize that rejection of transplanted organs is quite variable. Some patients will feel perfectly well, only to discover that their graft is being attacked by their immune system. In fact, it is more likely than not that there will be minimal or no symptoms of rejection.

Since rejection may have no symptoms at all, the standard strategy for post-transplant care is to regularly run blood tests that may be early indicators of liver graft rejection. In the beginning, these tests are run daily. For the first month or so after a liver transplant the tests are run at least weekly. Gradually the interval between measurement is increased as the months and years pass.

The common blood tests include bilirubin, AST (also called SGOT), ALT (also called SGPT), GGT, alkaline phosphatase, and LDH. These lab tests are often grouped together and called "liver function tests" or "LFT’s."

In truth, LFT’s are not measures of liver function per se, but are indicators of liver "well being." The tests are not perfect, however. LFT’s can be extremely abnormal despite the fact that the liver is perfectly fine. They can also read normal even though the liver is barely working! Interpretation of these tests, therefore, requires some expertise in liver transplantation as well as detailed knowledge of the patient’s previous laboratory studies and medical history.

When rejection is suspected it can be confirmed by a liver biopsy. In some instances a biopsy is not needed because rejection is strongly suspected. In other situations, a biopsy is critical.

The chief problem that must be differentiated from rejection is infection. Since the treatment of rejection (increased immunosuppression) can make an infection worse, it may be important to confirm the diagnosis of rejection with a biopsy prior to proceeding with treatment.

Another important thing to know is that most rejection happens in the first year after a transplant. This is particularly true in the case of liver transplant rejection. In fact, most liver graft rejection happens in the first three months after the transplant. As long as the immunosuppression drugs are continued and taken properly, the risk of rejection of the liver is very low after the first year.

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

What is the current status of living donor liver transplants, is it the preferred method for infants, or the second choice?

by Jeffrey D. Punch, MD

The status of living donor liver transplantation is still controversial in this country. Those in favor of living-related liver transplantation point to slightly higher patient survival rates in published reports compared to average cadaver liver transplantation. (But this issue hasn't been studied very well).

Advocates also claim an advantage in transplanting patients while they are still stable, since while waiting a year or more for a cadaver liver the patient may become sicker, making the transplant riskier. Also, using living donors increases the total supply of livers.

The critics of living related transplantation point to the risk to the donor (there has been at least one death of a donor in Europe, and one in the United States), and the higher rate of reoperation and other surgical complications in living donor compared to cadaver donor liver transplants. Also, most children that are referred early for liver transplant tolerate the wait for a cadaveric organ fairly well.

Dr. Punch is a transplant surgeon at the University of Michigan and a member of the C.L.A.S.S. Scientific Advisory Committee.

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