A liver transplant, also known as an orthotopic liver transplant, is the process of surgically replacing an unhealthy liver with a complete or partial healthy liver from a donor.

The liver is required for many critical metabolic functions, including the production of bile for digestion, and many proteins and enzymes. It is also crucial for the removal of degraded metabolic products.

While the liver can repair itself in response to injury, repeated injuries can permanently damage it and lead to liver failure. That is when a liver transplant becomes the only option to save the life of the patient.

Liver transplant and FAP

FAP (familial amyloid polyneuropathy) is a progressive genetic disease caused by a mutation in the TTR gene, which leads to the accumulation of abnormal protein deposits in the peripheral nervous system and other tissues. The TTR protein is mainly produced in the liver. Therefore, performing a liver transplant can greatly slow  the progression of FAP by preventing the formation of defective TTR protein.

When should a liver transplant be performed?

Based on the widely used Coutinho staging system, FAP is classified into three stages, stage 0 to stage 3, depending on the severity and progression of symptoms.

Patients in stage 1 of the disease are best suited for a liver transplant. Those at early stage 2 may also be eligible, depending on the presence of other symptoms and extent of nerve cell damage.

Performing a liver transplant at the later stages of the disease might not be very beneficial, as the already-deposited amyloids in the nervous system or heart might still continue to form protein aggregates despite the presence of normal TTR protein after liver transplantation.

How is the operation performed?

Once the clinician determines that the patient is eligible for surgery, tests are performed to ensure that all other vital parameters are intact. The patient then goes to the operating room and is administered general anesthesia.

During the operation, the surgeon makes an incision across the abdomen toward the chest. He or she then removes the damaged liver and replaces it with a complete or partial healthy liver. The blood vessels and bile ducts are reconnected and the abdominal incision is closed with sutures.

The patient is kept under observation in an intensive care unit (ICU) for a few days before shifting to a regular ward. If all outcomes are as expected, the patient is discharged within two weeks after the operation. In the event of a partial healthy liver transplant, the transplanted liver can grow to full size over the course of the year.

Are there any complications following the procedure?

A liver transplant comes with its own set of potential complications. These include clotting in the blood vessel that supplies the liver with oxygenated blood from the heart, a condition known as hepatic artery occlusion, or clotting in the vein that brings blood from the digestive organs to the liver, known as portal vein thrombosis. There are also complications associated with the leakage of bile from the newly transplanted liver or constriction of the bile duct.

Often, the recipient’s immune system considers the newly transplanted organ as foreign and rejects it. Therefore, medicines that lower the function of the immune system (immunosuppressants) are administered.

What are the outcomes for FAP patients following a liver transplant?

A liver transplant cannot treat amyloids that have already formed in the body. However, it can slow  the formation of new ones. Therefore, the function of affected nerves might not improve, but further disturbances are greatly reduced.

The outcome of a liver transplant is usually good for FAP patients provided it is done at stage 1 or early stage 2 of the disease. The exact outcome depends on the type of mutation in the TTR gene as well as nutrition, the age of the patient, the severity of the symptoms, and the presence of amyloids in the heart.

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FAP News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website

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