Paediatric Liver Transplant - Guidelines, Age, Life Expectancy

A paediatric liver transplant is a life-saving surgical procedure that involves replacing a damaged or failing liver in children with a healthy liver from a deceased or living donor. This medical intervention offers hope and a chance at a healthier life for children suffering from severe liver diseases.

Anatomy and Physiology of the Liver

The liver is a vital organ located in the upper right abdomen. It is large and reddish-brown and weighs roughly 3 pounds. There are two lobes of the liver; right and left. These lobes are further divided into lobules, which are even more small lobes. 

The liver performs over 500 functions for maintaining overall health and well-being. Below are some functions: 

1. Metabolism: It helps regulate blood glucose levels by storing excess glucose as glycogen and converting glycogen back to glucose when needed. It aslo helps in protein synthesis and lipid metabolism.

2. Detoxification: The liver aids in removing toxins, medications, and metabolic waste products from the bloodstream.

3. Bile Production: It aids in the breakdown of lipids into smaller molecules, enabling effective absorption.

A paediatric liver transplant is considered when a child's liver is no longer functioning adequately, and other treatment options have been exhausted. Here are some situations where a paediatric liver transplant may be necessary:

1. End-Stage Liver Disease: Children who have reached an advanced stage of liver disease, where the liver can no longer perform its essential functions adequately, may require a transplant. End-stage liver diseases include.

1. Biliary Atresia: This condition involves the blockage or absence of bile ducts, leading to bile accumulation and progressive liver damage.

2. Alagille Syndrome: A genetic disorder that affects various organs, including the liver, causing bile duct abnormalities and liver dysfunction.

2. Acute Liver Failure: A sudden and severe impairment of liver function, often caused by viral infections, drug toxicity, or metabolic disorders.

3. Metabolic Liver Diseases: These include conditions such as Wilson's disease, alpha-1 antitrypsin deficiency, and glycogen storage diseases, which affect the liver's ability to process and store nutrients.

4. Liver Tumours: Certain types of liver tumours, such as hepatoblastoma or hepatocellular carcinoma, may require a paediatric liver transplant as part of the treatment plan.

5. Congenital Liver Abnormalities: Congenital disorders like progressive familial intrahepatic cholestasis (PFIC), Crigler-Najjar syndrome, cystic fibrosis-related liver disease, and other rare genetic conditions can necessitate a liver transplant.

By understanding what to expect before and on the day of the transplant, families can be better prepared to navigate this transformative journey.

Before the Paediatric Liver Transplant

A set of protocols is followed before the surgery. These includes the following:

Paediatric Liver Guidelines 

The primary purpose of these guidelines is to ensure that the transition phase from childhood to adulthood remains smooth. The paediatric liver transplant guides are as follows:

1. Selection of the candidate for paediatric liver transplant and risk management. 

2. Patient consent, optimisations, and appropriate timing before the transplant.

3. Intraoperative measures and postoperative considerations for the patients.

4. Minimum age for a liver transplant.

5. Proper care and management of post-operative patients moving from intensive care units.

6. Smooth transition of paediatric liver transplant patients from hospital to home.

7. Ensuring that there are no other existing health conditions.

8. Evaluation and treatment of existing health conditions.

The child will undergo a series of consultations with the transplant team, including the transplant surgeon, hepatologist, anaesthesiologist, and other specialists. 

The doctor would also perform a pre-anaesthesia check-up before the surgery to check for any abnormalities and allergic reactions to the anaesthesia on the child.

Donor Compatibility Assessment: Compatibility tests are conducted to determine the donor's suitability and assess the liver's compatibility.

In the case of the living donor, the doctor may provide a tentative surgery date, subject to the results of the pre-anaesthesia check-up and other tests. The final date of surgery will be provided based on the tests. 

After the above protocol, the family members may expect the following before their child's liver transplant surgery:

On the Day of Paediatric Liver Transplant

The parents may expect the following for their child on the day of the liver transplant:

A dedicated medical team (anaesthesiologists, hepatologists, and critical care specialists) performs the paediatric liver transplant procedure. This procedure takes approximately 10 to 12 hours to complete based on the complexity.

The steps of the procedure are:

1. Evaluation and Pre-transplant Preparation: Before the transplant, the child undergoes a thorough evaluation to assess their overall health and suitability for the procedure. This includes blood tests, imaging studies, and other diagnostic tests. The child is also evaluated for organ compatibility and placed on the waiting list for a suitable liver donor.

2. Anaesthesia and Incision: The child is taken to the operating theatre and is given general anaesthesia to ensure a painless and comfortable treatment. A surgical cut is done in the upper right side of the abdomen to access the liver.

3. Donor Liver Retrieval: In a paediatric liver transplant, the liver can be obtained from a deceased donor or, in some cases, a living donor, typically a family member. The donor's liver is carefully retrieved, preserving its blood vessels and bile ducts.

4. Donor Liver Implantation: The diseased liver is removed from the child's body, and the healthy donor liver is positioned and connected to the child's blood vessels and bile ducts. The surgeon carefully reconnects the blood vessels to ensure proper blood flow to the new liver.

5. Biliary Reconstruction: The bile ducts of the new liver are meticulously attached to the child's bile ducts or the small intestine, allowing the flow of bile from the liver.

6. Closure and Recovery: Once the transplant is complete, the incision is closed using sutures or staples. The child is then transferred to the recovery room for close monitoring.

After a paediatric liver transplant, the initial recovery phase occurs in the hospital under close medical supervision. The child would need to stay at the hospital for 10 to 15 days before getting discharged.

Donors can resume their normal lives without long-term medications or dietary restrictions after discharge.

Recovery at Hospital

After a pediatric liver transplant, patients are taken to the wardroom for further recovery. One can expect the following during their hospital stay:

1. Monitoring Vital Signs: Healthcare professionals will continuously check vital signs such as heart rate, blood pressure, and oxygen levels to ensure a stable recovery process.

2. Pain Management: Effective pain relief methods will be implemented to reduce discomfort and enhance the patient's overall well-being during the initial recovery phase.

3. Immunosuppressive Therapy: The patient will begin taking immunosuppressant medications to prevent organ rejection. The dosage of these medications will be adjusted over time, but they do increase the risk of infection. To counteract this, prophylactic antibiotics, antifungals, and occasionally antivirals will be administered.

At-home Recovery 

When the child is discharged from the hospital, the attendants will receive a comprehensive summary detailing follow-up tests and medication schedules.

It's important for families to understand the necessary precautions, follow-up appointments, medication regimen, symptoms of potential complications, and how to contact the transplant team in an emergency.

Apart from resting well, a smooth at-home recovery involves:

1. Nearby Accommodation and Infection Prevention: Ensure the home is thoroughly cleaned with disinfectants. Stay close to the hospital, preferably in a clean place with easy access and minimal stairs, and with 24-hour transportation available.

2. Visitor Restrictions and Precautions: Limit visitors during the first few weeks and avoid contact with anyone who is sick. If the child shows signs of illness, inform the transplant team immediately. The child should wear a mask for the first three months and avoid crowded places.

3. Medicines: Adhere strictly to the prescribed medications, especially immunosuppressants, to promote recovery. Over time, the dosage of these medications may be reduced. However, a small dose of at least one immunosuppressant can be continued for life.

4. Personal Hygiene and Wound Care: Emphasise frequent handwashing and maintain oral hygiene. Keep the child's fingernails trimmed and follow wound care guidelines, changing dressings 2-3 times a week initially.

5. Diet and Nutrition: Ensure the child follows a high-protein diet to support wound healing and liver regeneration. Food should be prepared and consumed hygienically. Consider fresh fruits, vegetables, and a balanced, low-fat, high-protein diet.

6. Foods to Avoid: Avoid fried or greasy foods, leftovers, raw or undercooked eggs, overripe fruits, and expired packaged foods. Adjust the diet according to the child's blood sugar and potassium levels.

7. Activity and Exercise: Gradually reintroduce walking and routine activities. Regular exercise will improve the child's energy levels and muscle strength. A physiotherapist can guide limb exercises to reduce complications.

First Follow-up Appointment

Consistent follow-ups and hospital visits are crucial for a successful liver transplant, ensuring both donors and recipients stay healthy and address any concerns promptly.

The follow-ups of the donors and recipients are as follows:

1. For Donors

1. Following a donation, donors should schedule follow-up appointments every five to seven days for the first month.

2. From the initial month of transplantation, evaluations and further testing are required three months and a year later.

2. For Recipients

1. In accordance with the plan given upon discharge, recipients will have regular follow-ups, which can be frequent in the beginning.

2. As the recipient's health stabilises, follow-up appointments will become less frequent, usually occurring every three to six months.

The one-year survival rate after liver transplant is over 90%. It is one of the safest and most effective procedures for curing liver ailments in children. The benefits are:

1. Extended Lifespan: Liver transplant recipients generally see a substantial increase in life expectancy as the new liver restores vital functions compromised by liver failure.

2. Improved Quality of Life: Post-transplant, patients often experience significant relief from symptoms like chronic fatigue, jaundice, and abdominal pain, leading to a more comfortable and active lifestyle.

3. Reduced Risk of Mortality from Liver Disease: The risk of dying from liver disease drops significantly after the transplant, as the new liver effectively performs essential metabolic and detoxifying functions.

4. Increased Energy Levels: Many recipients report higher energy levels post-transplant, enabling them to participate in previously exhausting or impossible activities.

5. Return to Normal Activities: Patients often resume regular activities, including work, travel, and hobbies, which enhances their sense of normalcy and well-being.

Although safe, there can be some complications after the procedure. The most common risks and complications may include: 

1. Bleeding: Just like any other medical procedure, there is a risk of bleeding during or after the procedure.

2. Infections: Certain medications used during the liver transplant can cause multiple types of infections, such as fungal, bacterial, and urinary tract infections.

3. Bile Duct Complications: Complications such as leaks and blockages can occur post-liver transplant surgeries leading to infections and damage of the liver cells.

4. Rejection of the Donated Liver: In many cases, the immune system of the recipient has been known to reject the acceptance of the foreign organ, causing problems.

When is consultation needed with the doctor?

Throughout the child's recovery, it's essential to maintain open communication with the medical team and seek consultation when needed. It is recommended to contact the doctor if the child experiences any concerning symptoms. These symptoms include 

1. Fever

2. Weakness and fatigue

3. Significant change in appetite

4. Persistent pain does not reduce with time

5. Swelling and abscess at the site of the incision

Liver diseases and conditions can progress rapidly in children. Recognising the potential risks of delaying the procedure and their impact on health is important. The following can happen if the transplant is delayed:

1. Disease Progression: It increases the risk of complications and reduces the chances of a successful transplant outcome.

2. End-stage Liver Damage: It can cause end-stage liver damage or failure, progressing through stages of inflammation, fibrosis, and cirrhosis.

3. Spread of Liver Cancer: If cancer is present, it can spread to other organs, potentially resulting in fatal outcomes.

On average, the cost of a paediatric liver transplant in India is approximately between ₹ 15,00,000 to ₹ 25,00,000. There are several factors that influence the cost of a paediatric liver transplant. Some of these factors are as follows: 

1. Patient’s age

2. Type of donor

3. Doctor’s expertise 

4. Location and type of hospital

5. Pre and post-treatment medications 

6. Physiotherapy and rehabilitation 

It is advisable to consult with the medical facility and discuss the financial aspects, including insurance coverage and potential financial assistance programs available.

A paediatric liver transplant is a life-saving procedure for children with end-stage liver disease. India is renowned for providing top-notch paediatric liver transplants. 

HexaHealth offers comprehensive assistance for your better health. From consultation with the best doctors to assisting you financially, we guide families through the transplant process.

Suggested Reads