Porphyria Educational Services Bulletin
Vol. 2 No. 30
July 30, 2000
FOCUS: Maintaining a Healthy Liver
The liver is a vital organ for everyone, but the liver is especially
the majority of porphyria patients.
Porphyrin production takes place in the liver. Overproduction of
during porphyria attacks can and do damage the liver.
To avoid having liver problems in porphyria
you need to remember to maintain
healthy eating habits.
Fatigue, decreased appetite, nausea,
vomiting, and food intolerances are sometimes
associated with liver disease. Throughout the progression of the disease,
of ascites (increased fluid in the abdomen), hepatic encephalopathy
increased diarrhea (from medications) and edema fluid retention in the feet
may leave you feeling further fatigued and depleted.
The goal of nutritional management of liver
disease is to help you maintain or improve
your nutritional status, achieve or maintain calories, and appropriate
amounts of protein
(without contributing to hepatic encephalopathy).
It is always advisable for porphyria patients to have 60 -65% of their
in the form of carbohydrates, reduced protein, and minimal fat intake.
It may also be necessary to reduce your
sodium and fluid intake.
Another very important thing is to avoid all alcohol, inhalation of
and toxic fumes, and to avoid the use of all cytochrome P-450
Your physician may advise you to have a
registered nutritionist provide you with a diet
individually tailored to meet your nutritional needs and stay as healthy as
while coping with your porphyria.
It is essential for porphyria patients to also keep active.
The effects of a compromised liver such as
malaise, decreased appetite, fatigue, and
depression can take a toll on an individualís ability to perform
activities of daily living.
Such reduced activity can lead to a
decrease in flexibility, and endurance, and eventually
a decrease in cardiopulmonary performance resulting in an increased surgical
A daily program consisting of regular
exercise and normal activity can reduce
complications and maximize your strength, flexibility, mobility, endurance,
All of these areas are of utmost importance in feeling healthy while still
It is important for porphyria patients to be
able to care for themself.
Keeping healthy is paramount. Being active is also paramount.
Most porphyria patients have active lives.
The goal is to provide excellent care
and follow up in concurrence with your primary physicians in order to make
length and quality of life appreciable better just by following some good
healthy lifestyle management.
Eloise WIlson, [AIP] , MNS, RN