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Porphyria Educational Services Bulletin
Vol. 2 No. 29                                                 July 23, 2000
FOCUS:  Liver Transplantation and Porphyria
While liver transplantation is not an everyday occurence for porphyria patients,
it may be implemented in some porphyria patients who face liver failure.
Your physician may recommend a liver transplant when all other treatment options fail.
The reason for a liver transplant  is to replace your diseased liver with a healthy liver.
Ideally, after a transplant you will be free from the threat of liver failure due to porphyria.

After having a liver transplant you should be able to  lead a fairly normal life as long
as the transplant functions. However, a transplant will not be a cure for the disease porphyria.
There are many diseases which have  indications for liver transplant. The long
list of diseases include: Hepatitis B [which is common with PCT type porphyria];    
Autoimmune Hepatitis, Hepatocellular Carcinoma [which can often be associated with
than one type of porphyria];      Drug induced liver failure [which happens especially
with the use of P-450 drugs]; Hemochromatosis [which is often found associated as a
secondary disease along with some types of porphyria]; and most often with Protoporphyria type porphyria.
So where does one get a liver for transplant?
There are three options for liver transplantation: cadaver donor transplantation, living donor
transplantation, and auxiliary transplantation.
Cadaver donor: The donor liver is obtained from a person who is diagnosed as brain dead whose family
volunteers to donate the organ for transplantation. People who receive cadaver donors wait on the
national computer list (UNOS Waiting List) until a suitable donor becomes available. The waiting times
Living donor:
A healthy family member, usually a parent, sibling, or child, or someone emotionally
close to you, such as a spouse, volunteers to donate part of their liver for transplantation.
The donor is carefully evaluated by the team to make sure no harm will come to the donor or recipient.
Auxiliary transplantation:
Part of the liver of a healthy adult donor (living or cadaver) is transplanted into the recipient.
The patientís diseased liver remains intact until the auxiliary piece regenerates and assumes function.
The diseased liver may then be removed.
So how successful is a liver transplant?
The average success rate of a liver transplant at one and five years is 92 percent and 88 percent respectively.
A porphyria patient who has a liver transplant has a good chance to lead a normal, healthy [in terms of a porphyric], active, life.
In order to be sure that the liver transplant will work, a porphyria patient must
closely follow their  physicianís recommendations. This will include taking
immunosuppressive medications and monitoring your blood tests regularly.
How does a porphyria patient ontain a liver transplant?
All persons seeking to be a liver recipient must have an Initial Consultation.
The patient will meet with members of the transplant team to review  past medical
and social history to ascertain appropriate medical treatment.
Who makes up the Transplant Team?
Hepatobiliary Surgeons who are trained in the field of transplantation are foremost.
Hepatologists, Transplant Coordinators who are registered nurses and assist in the
evaluation, education and post-operative follow-up; and  specialized social workers
who help with your family and provide you with support, make up the rest of the
Transplant Team.
After the initial consultation comes the presurgery testing.
Such testing includes: Blood Tests, Radiology, [chest x-ray, ultrasound, CT Scan,
or./and MRI, as well as endoscopy.
Other tests that may be utilized include: ERCP [ a scope similar to the endoscopy ]
Liver Angiogram Liver Biopsy,  and Cardiac Evaluation.
The Cardiac evaluation may include: ECG, Echocardiogram,  and a
Stress Test,  and/or a Cardiac Angiogram.
There will also be a full Pulmonary Evaluation [lungs].
When your evaluation is completed, the transplant team will determine, based on the results,
whether a transplant is necessary and if you are physically able to undergo the procedure.
The waiting period varies depending on the type of transplant you require. living,  your
blood type, how sick your liver is, and your height and weight.