Some people living with Inherited Metabolic Disorders may be recommended for a liver transplant by their metabolic team. Here, we explain exactly what a liver transplant entails and when it is recommended for patients living with Inherited Metabolic Disorders.
What does the liver do?
The liver is a vital organ which carries out over 500 different functions. It is a very tough organ and can continue to function when it is partially damaged. The liver fights infections, controls levels of cholesterol in your body, and processes and gets rid of harmful toxins in the body.
What is a liver transplant?
A liver transplant, sometimes referred to as an orthotopic liver transplantation [OLT], is an effective therapeutic option available for individuals who are born with some Inherited Metabolic Disorders. This procedure aims to remove, or partially remove, a diseased liver and replace it with a healthy donor human liver. This can significantly improve the quality of life of patients who suffer from severe disease manifestations and/or life-threatening metabolic decompensations despite medical/dietary management.
A liver transplant is treated as a serious operation, and surgical advances have enabled the development of immunosuppressants which are taken preoperatively to prevent graft or transplant failure and to increase success rate. The drugs most commonly given to prevent organ rejection are cyclosporine, tacrolimus, sirolimus, azathioprine, mycophenolate and prednisolone.
Why do people need liver transplants?
There are many factors as to why people may need a liver transplant. One of these may be due to an inherited metabolic condition where the liver is unable to repair itself. Recent advances in surgical management and development have made liver transplantation a viable option for an increasing number of metabolic conditions. Some of the Inherited Metabolic Disorders that have been cured by liver transplants are:
- Tyrosinemia type 1
- Wilson disease
- Alpha-1 Antitrypsin Deficiency
- Acute Intermittent Porphyria
- Maple Syrup Urine Disease
- Urea Cycle Disorders
- Glycogen Storage Disease Types Ia, Ib, III, IV
- Crigler Najjar Syndrome
- Primary Hyperoxaluria
- Progressive Familial Intrahepatic Cholestasis (PFIC)
Some Inherited Metabolic Disorders may require or benefit from a liver transplant as part of the treatment. However, it is not a cure and will require further management of the condition. These conditions include:
- Familial Hypercholesterolaemia
- Erythropoietic Protoporphyria (EPP)
- Methylmalonic Acidaemia
- Propionic Acidaemia
- Some mitochondrial disorders
What happens when I am being considered for the liver transplantation list?
When you and your doctor begin to consider liver transplantation as a possible treatment option, you will need to undergo a number of tests to determine if a transplant is possible and if it’s the best option for you. You will also have to answer regular questions about your health, including how you are feeling and your symptoms. Many of these tests will be familiar from when you were first diagnosed with a liver condition and some of the tests will need to be repeated throughout the assessment period. These tests may include:
- Blood tests
- Chest x-ray
- Lung function test
- Electrocardiogram [ECG]
- Kidney function test
- Ultrasound scan
- Liver biopsy
How long will I be on the waiting list?
It was announced in 2017 that the waiting time for a liver transplant was 135 days. However, priority for surgery will always be given to those who are in urgent need of surgery. Since April 2018, a new National Liver Offering Scheme has been introduced by NHS Blood and Transplant (NHSBT). This scheme matches livers on a national rather than a regional basis, with the aim of increasing the numbers of life-years gained by the transplanted livers.
The characteristics such as age and sex of the organ donor and the patient who will receive the new liver are analysed by a computer to find the patient who is the best match. This is called the Transplant Benefit Score (TBS), and ensures the liver goes to the patient who will benefit most from it.
Where can you get a liver transplant?
Currently, there are between 650- 800 liver transplants performed per year. There are seven hospitals in the UK with adult liver transplant units:
- London – Royal Free Hospital and King’s College Hospital
- Birmingham – Queen Elizabeth Hospital (adults)
- Leeds – St James’s University Hospital
- Newcastle – Freeman Hospital
- Cambridge – Addenbrooke’s Hospital
- Edinburgh Royal Infirmary
There are also three children’s liver transplant units:
- London King’s College Hospital Paediatric Liver Centre
- Birmingham Children’s Hospital
- Leeds General Infirmary Children’s Liver Unit
From here, you or your child will be assessed, which involves speaking with specialists to discuss whether this is the right treatment option and the benefits and risks, and to answer any questions that you may have. They will also carry out the necessary tests to determine whether a transplant is possible and if it is the best treatment, and your specialist will be able to discuss looking after yourself whilst waiting for a transplant and the care and follow up needed after your transplant.
A number of helpful resources about liver transplants can be downloaded from the British Liver Trust website: https://www.britishlivertrust.org.uk/liver-information/liver-transplantation/
If you would like any further advice on liver transplants, please contact us on firstname.lastname@example.org