Children account for 10 % to 18 % of all liver transplants performed across the globe. In the majority of the cases, children receive their transplant before they turn 5 years of age.
Liver transplantation is the best available treatment for pediatric liver diseases causing acute liver failure or progressing to end-stage liver disease (ESLD). The prognosis for end-stage liver disease is assessed with the help of tools such as Child-Pugh score, MELD Score (> 12 yrs), PELD Score (<12yrs). Evaluation is done as rapidly as possible because the success of the surgical intervention measure such as hepatoportoenterostomy, the Kasai procedure tends to decrease progressively at older ages. Orthotropic liver transplant (OLT) should be considered at an early stage especially in patients who have no chances of clearing of jaundice by 3 months. OLT is also recognized as the primary treatment for biliary atresia and may be permitted only for patients > 120 days of age having an enlarged hard liver and decompensated cirrhosis. Other indicators for liver transplant include how day-to-day activities have been limited, the number of days spent in hospitalization, and growth retardation caused due to underlying liver disease. Liver transplantation is the best course of action to improve life expectancy and/or quality of life.
In addition to the patient evaluation measures for liver transplantation detailed out in the previous section, the pre-transplant evaluation in a pediatric liver transplant includes the following issues:
Pretransplantation Immunizations: In most cases, live-virus vaccines are usually contraindicated in those patients with severe immunosuppression after liver transplant as there’s an inherent risk of dissemination occurring secondary to immunosuppression. Therefore, it is recommended to complete all normal immunizations before transplant for the patient as well as each member of his/her immediate family. These include – BCG, DPT + Hib, Mumps, Rubella, Hepatitis B, Measles, MMR. In addition, It’s suggested that even optional vaccines such as Hepatitis A, Typhoid, chickenpox, influenza rotavirus and pneumococcal vaccines that could compromise the patient's immunity are administered.
The vaccination program may need to be expedited which is why the actual schedule may vary as compared to the normal recommendations. The goal is to complete all rounds of live vaccination prior to transplant. Once the live vaccinations are completed, the liver transplant operation is pushed back for at least two to three weeks. In case of acute liver failures, considering there’s an urgent need for a liver transplant, the transplant team might not wait and go ahead with the surgery as is. That said, killed vaccines such as Tetanus, Hepatitis B vaccines are administered if the need arises. Some patients may also need certain types of interventional radiology procedures before the transplant such as drainage of bile leaks and insertion of central venous lines for emergency vascular access.
Successful liver transplantation in a child depends on the quality of the medical staff employed. Pediatric liver transplants usually need top-notch expertise and the skills of doctors who are thoroughly trained and experienced in pediatric critical care and transplantation. This is because children have tiny blood vessels in them which are difficult to attach. Moreover, unlike adults, children need specialized post-operative care as well.
Pre-transplantation management includes managing specific hepatic complications effectively and efficiently while the patient waits for the liver transplant. These include dealing with conditions such as portal hypertension, oesophageal varices, ascites, hypoproteinemia, encephalopathy etc.
Nutritional support:Providing adequate Nutritional support is also an important clinical challenge with end-stage liver disease. Successful liver transplantation mandates the need for aggressive nutritional rehabilitation both pre- and post-transplant which is why the dietician from the transplant team remains closely involved in the nutritional care of these children. As per research and studies, it has been revealed that optimization of nutritional status in pediatric patients at liver transplant is an important prognostic factor in survival i.e. better outcomes are witnessed in patients with good nutritional status. Not only that, but a good nutritional status also translates to fewer infections and a reduction in surgical complications.
The patient needs to be put on a high-calorie diet (150- 200 percent calories along with good protein intake) with two times the Recommended Dietary Allowance (RDA) of multivitamins.
In patients with cholestasis, nutritional rehabilitation in the form of supplementation with fat-soluble vitamins such as vitamin A, D, E, K is executed. In that regard, cholestasis patients are also recommended to use Medium-Chain Triglyceride (MCT) oil commonly extracted from coconut oil for cooking. If a child is unable to intake food orally, then tube feed supplementation is done, which could be used either for overnight feeds or during the day – as per the requirements.
In the case of babies with severe malnutrition, measures are taken to improve their overall nutrition and weight. Despite all efforts, sometimes even good calorie intake may not be enough to achieve an improvement in weight. In such a scenario, the doctor and the transplant team may decide to go ahead with the liver transplant surgery even at a low weight. Therefore, the decision of timing of liver transplantation will need to be individualized on a patient to patient basis.
The number of blood and blood product units required in case of a pediatric liver transplant is comparatively much lesser as compared to adults. On an average, for a pediatric liver transplant, 4-6 units each of packed cells, Fresh frozen plasma (FFP), and 1-2 units of platelet apheresis need to be arranged.
The use of split livers from deceased donors and partial grafts from donors has resulted in favorable graft viability. This procedure involves splitting a single deceased (cadaveric) donor liver into two separate parts – the right and left portions – and thereon implanting it into two recipients at the same time. Usually, the right lobe is implanted in adults and the left /left lateral lobe is implanted in children. As a result of this procedure, the donor pool has increased. In fact, even livers from ABO Incompatible donors can be used in children as the body’s immunity in young children is not fully developed thus reducing the chances of rejection. Further, in the case of ABO-incompatible liver transplants, usually a few sessions of plasmapheresis – a process to filter the blood and remove harmful antibodies – are required to be carried out in a patient couple of weeks before the transplant. This also leads to an increase in the overall cost of the transplant
Another alternative for increasing the donor pool is through the swap donor process. Swap donor is an option which is used when similar blood group donors are not available. In such a scenario, the donors of 2 different patients suffering from a similar problem donate to each other. Also referred to as a liver swap, the procedure basically entails two liver recipients essentially “swapping” willing donors with each other. Despite being medically eligible to donate, sometimes donors can have an incompatible blood type or antigens to his or her intended recipient. By agreeing to essentially exchange/swap recipients—giving the liver to an unknown, but the compatible individual—the donors can provide two patients with healthy livers wherein previously such a transplant wouldn’t have been possible.
Short answer – Yes. Pediatric liver transplants require a great amount of expertise and experience from a skilled team of surgeons because of the blood vessels and bile duct present in a child –especially children who weigh less than 10 kg – is very small. Additionally, because the majority of pediatric patients fall in the post-Kasai category (post biliary atresia surgery), chances of adhesions causing tissues and organs to stick together are more, thus making it all much tougher to operate for surgeons.
Anaesthetic care required in children tends to be different as children have comparatively lesser lung volumes. Moreover, the odds of intraoperative bleeding due to the adhesions made inside are usually a lot more which requires to be managed while also ensuring volume overload is avoided. Because of the narrow margins involved and low margins for errors as compared to adults, ideally a highly experienced anaesthetist expert is called in for such surgeries.
Immediately after the transplant, the child is usually transferred to the ICU where he is put on a ventilator and observed for 24-48 hours. Pediatric patients have to be observed by a team of specialized pediatric intensivists and nurses who are especially trained and experienced in pediatric intensive care. Moreover, as compared to adult patients, pediatric patients require additional care and support such as prolonged ventilation and ICU stay. For confirmation of viral infections, specific antibody tests are also carried out. In addition, several pediatric patients undergo a Roux-en-Y Choledochojejunostomy for biliary reconstruction in liver transplantation which is why feeding is delayed until usually the third-day post-operation. It needs to be mentioned that a child’s need for analgesia or painkillers is also relatively higher owing to a lower pain threshold. Pediatric patients also require undergoing regular chest physiotherapy, without which there’s a risk of the lungs developing collapse consolidation leading to pneumonia. Last but not least, physiotherapy in small babies and children requires specially-trained pediatric experts.
We try to make the child/baby feel comfortable and at ease by playing peaceful and calming music, providing a TV with child-friendly channels along with toys which can easily be cleaned with the help of sterilium. It needs to be mentioned that stuffed toys or toys with moving parts are avoided.
Immunosuppression: Following a liver transplant, the patient requires lifelong immunosuppression. There are 3 main drugs involved including Calcineurin inhibitors such as tacrolimus, Antimetabolites such as mycophenolate mofetil and Corticosteroids. Steroids are the first to be discontinued followed by mycophenolate mofetil. It’s been shown that nearly 50 percent of patients are no longer on steroids at 1 year after transplant. Thereafter, the patient needs to take a single immunosuppressive drug, usually tacrolimus, which needs to be taken twice daily. The child’s family receives instructions regarding proper nutrition and the continuation of immunosuppression and other medications. The child’s caretaker must also make sure that blood tests are done regularly in order to monitor the functioning of the liver & kidney as well as to analyze if the immunosuppressive drug levels are as advised by the doctor. After the first couple of years, the frequency of testing may be reduced to once every 3-4 months.
Thanks to medical advances, patient survival after pediatric liver transplantation has drastically improved over time. Patient survival rates in children after liver transplant is approximately 93 percent at one year, 87 percent at five years and 81 percent at 10 years. In most cases, children experience no significant issues related to mortality after this. Children who survive liver transplant go on to live normal, fulfilling lives and are able to lead a normal lifestyle despite the need for continuous monitoring of immunosuppressive drug levels. They are able to do all activities which their peers are able to do such as indulging in normal school sports, activities, etc. As a matter of fact, more than 50 percent of pediatric patients survive past 25 years.
Most children are able to resume schooling 3 months after the transplant and resume sporting activities 3-6 months post-transplant. Health-related quality of life usually has shown gradual and steady improvement over time. As a matter of fact, many patients who were operated when they were a child/adolescent have gone on to become parents of their own children later on. There are also several examples of children who have gone on to accomplish many sporting feats.
That said, regular follow-ups with the doctor and the transplant team are the key to ensuring successful outcomes. These follow-ups visits are important as the doctor monitors the functioning of the organ while also gauging if there are any side effects of immunosuppression. Sometimes, adolescents may defer or refuse to follow their normal medication routine and in such a scenario, counselling from their caregivers and professional psychotherapists is the key to the prevention of non-compliance.