Porphyria Educational Services Bulletin
Vol. 2 No. 29
July 16, 2000
FOCUS: Liver Transplantation and
While liver transplantation is not an everyday occurence for porphyria
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
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
moren 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
secondary disease along with some types of porphyria]; and most often with
Protoporphyria type porphyria.
So where does one get a liver
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
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
The donor is carefully evaluated by the team to
make sure no harm will come to the donor or recipient.
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
A porphyria patient who has a liver transplant
has a good chance to lead a normal, healthy [in terms of a porphyric], active,
In order to be sure that the liver transplant
will work, a porphyria patient must
closely follow their physicianís recommendations. This will include
immunosuppressive medications and monitoring your blood tests regularly.
How does a porphyria patient ontain a liver
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
who help with your family and provide you with support, make up the rest of
After the initial consultation comes the
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
The Cardiac evaluation may include: ECG,
Echocardiogram, and a
Stress Test, and/or a Cardiac Angiogram.
There will also be a full Pulmonary Evaluation
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 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.