Overview of Pediatric Transplantation
Dr Anurag Shrimal
Consultant Abdominal Organ Transplantation and HPB Surgery
Liver transplantation is a lifesaving treatment option for children with serious liver disorder. Liver disease affects newborns, toddlers and teenagers and has many adverse effects; not only on liver function, but also on the child’s growth, development and intellectual achievement.
What are the symptoms and signs of Liver Failure in Kids?
The symptoms of liver disease in the young ones are not very specific. Many a times a lot of the time is spent in establishing the root cause of the problem based on the symptoms. After multiple diagnostic tests a cause of liver ailment needs to be established before liver transplant could be considered.
Liver disease symptoms and findings that may indicate the need for a liver transplant include:
- Prolonged neo-natal jaundice in infants
- Abnormal stool color – pale or clay-colored or tarry black or dark urine
- Bulging tummy (ascites)
- If your child is not playful, not eating well or itching and not growing compared to other kids of her age
- If your child is having recurring high fever and infections
- Unacceptable quality of life (itching, poor growth, failure to thrive)
- Recurrent cholangitis
- Encephalopathy (elevated ammonia levels in the blood)
- Abnormal liver function tests high levels of enzymes, billirubin
- Enlarged spleen
Liver Diseases Leading to Liver Transplantation
Though many diseases affect the liver, not all liver diseases are treated with a transplant. A liver transplant is recommended for children who have serious liver dysfunction and will not be able to live without having the liver replaced. A thorough evaluation process will help to decide whether or not a child’s disease requires a liver transplant.
Chronic End-stage Liver Disease
- Biliary Atresia – After failed attempt at Surgery – is the most common cause for pediatric liver transplant
- Autoimmune Hepatitis/Sclerosing Hepatitis/Overlap syndrome
Liver-specific genetic /metabolic diseases
Causing Chronic Liver Injury / Failure
- Alpha-1-antitrypsin deficiency
- Familial cholestatic syndromes eg PFIC1 2 or 3
- Allagille syndrome
- Cystic fibrosis liver disease
Metabolic Liver Defects Leading to other Organ Damage
- Urea cycle disorders
- OTC deficiency
- Maple syrup urine disease (MSUD)
- Crigler –Najjar syndrome
- Glycogen storage disease
- Acute liver failure
- Indeterminate (non-A, non-B, non-C hepatitis)
- Viral hepatitis
- Hepatic Drug toxicity (acetomenophin, NSAID’s, many others)
Genetic /Metabolic Diseases:
- Wilsons disease, fatty acid oxidation defects, some mitochondrial disorders
- Autoimmune Hepatitis
- Neonatal Hemochromatosis
Primary Unresectable Liver Tumors
- Embryonal sarcoma
- Hepatocellular Carcinoma
The Liver Transplant Process
Liver transplantation is a lifelong commitment for the child’s health. For understanding the process can be divided into following phases.
Comprehensive Evaluation Process
Once a liver transplant is considered to be a treatment option, a timely detailed evaluation of a child by a multi-disciplinary team for transplant is a necessity. The child’s evaluation include:
• Identify the risks and benefits of possible transplantation
• Identify the need for and timing of transplantation
• Identify technical (surgical) issues and appropriate donor sources
• Establish a pre-transplant management plan
• Provide a fully informative and frank family discussion to help prepare you and your child for the daunting but potentially life-saving road ahead
The evaluation usually takes 3 to 4 days, which may be inpatient or outpatient based on your child’s medical condition. A detailed history, physical exam and tests are carried out in all cases. Tests include:
• Blood tests to evaluate organ function, exposures and immunity to infection
• Blood typing
• Chest x-ray
• Abdominal ultrasound
Some children may require other special tests decided at the time, such as endoscopy, liver biopsy, etc.
You and your child will also meet with many transplant team members and specialists including:
• Transplant Surgeon
• Pediatric Hepatologist
• Pediatric Liver Transplant Anesthetist
• Pediatric Nutritionist
• Pediatric Physical therapist
• Other medical specialists on case by case basis such as pediatric cardiologist, endocrinologist, medical genetics etc.
Evaluation of Donor
In our country organ donation is still in infancy and the chances that a child get a deceased donor liver transplantation is very slim. Most of the time one of the parent comes forward to donate a small part of his/her liver for a living donor liver transplant for their child. Donor Evaluation takes two to three working days and include thorough assessment to eliminate risk to the donor as well as to the recipient.
Liver Transplant Surgery
After the thorough evaluation and preparation, we are now set for the Liver Transplant Surgery.
Types of Pediatric Liver Transplant
- Deceased Donor Liver Transplant (DDLT)
- Whole Organ Transplant from a pediatric brain dead donor
- Split Liver Transplant from selected adult brain dead donor
Living Donor Liver Transplant (LDLT)
- Left Lateral Lobe Liver Transplant
- Hyper reduced left lateral lobe liver transplant / Mono segment liver transplant
- Left Lobe Liver Transplant
Your child’s surgery may take from four to 12 hours depending on his or her condition. During surgery, your child’s old liver and gallbladder will be removed and replaced with the donor liver. Since a gallbladder is no longer needed, a new one will not be transplanted.
After surgery, your child will go directly to the intensive care unit (ICU), usually for one to two days. During surgery, a breathing tube will be inserted to help him or her breathe. In most cases the tube can be removed within 24 hours after surgery. Many monitoring lines will be attached; these, too, will be removed as your child becomes more stable. When your child is ready to leave the ICU, he or she will be cared for in a single room with a dedicated nurse of the hospital.
Liver Transplant Recovery
Everyone recuperates from liver transplantation differently. Depending on your child’s condition, he or she will be hospitalized for two to four weeks following the transplant. It is generally recommended that you stay in the same city where your child gets transplanted for 2-3 months.
Once your child has returned home, a regular follow is needed to ensure optimum functioning of the transplanted liver and a normal growth and development of the child. .
Laboratory blood tests are obtained twice a week following transplantation; the frequency of blood tests is gradually reduced over time. You will be asked to email in test results to the transplant office. You will then be notified about any adjustments in your child’s medications.
These drugs decrease your child’s resistance to foreign bodies, such as the new liver. Your child will need to take these medications for the rest of his or her life or the liver will be rejected. Immediately after surgery, the dosages will be high since the probability of rejection is greatest at this time. Dosages will be lowered quickly to smaller amounts if there are no signs of rejection.
The medications have side effects, which are usually dose-related. Most people experience the most side effects in the beginning when medication dosages are high. As the dosage is lowered, these effects will probably lessen. Side effects may occur in some patients and not in others.
The medications your child will take for rejection also impair his or her ability to fight off infections. Your child will be given medication to help prevent infections but you also will need to use caution and avoid contact with people with infections, especially during the first three to six months after transplant.
Dr Anurag Shrimal, Consultant Abdominal Organ Transplantation and HPB Surgery
After completing his MBBS and Masters in General Surgery at Seth G S Medical College & KEM Hospital, Mumbai and Surgical Oncology training at Tata Memorial Centre, Mumbai. He received American Society of Transplant Surgeons accredited Fellowship in Liver & Kidney Transplant at the Duke University Medical Center, Durham, NC, USA. He is trained in Liver Transplant, Kidney Transplant, Pancreas Transplant, Intestine Transplant and Multi-visceral Transplant during his tenure at Duke University.