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PORPHYRIA FACTS: LIVER DISEASE & NUTRITION
In the hepatic porphyrias nutrition is very important.
SOURCE:
Sheryl WIlson MNS, RD (HCP)
+++++++++++
Nutrition in porphyria is intended to protect the liver so that liver disease does
not progress.
Nutrition in porphyria patients should protect the liver from overwork and enable
it to function as efficiently as possible.
SOURCE:
Sheryl WIlson MNS, RD (HCP)
+++++++++++
Proteins assist with tissue repair, and prevention of fatty infiltration and damage
to the liver cells.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++
Fatty infiltration into the liver is common in hepatic porphyria.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++
There can be fatty liver due to alcohol ingestion which is common in the PCT
form.
NASH (non-alcoholic-steato-heptitus) aka Fatty Liver is also commonin the acute
porphyrias.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++
In people with severely damaged livers, the proteins cannot be properly
metabolized and waste products (including ammonia) that are toxic to the body
can build up and affect the brain.
In this case, protein may be restricted.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++
Hepatic porphyria patient need to follow the guidelines for protein intake and not
exceed the recommended levels.
SOURCE:
Sheryl WIlson MNS, RD (HCP)
+++++++++++
The increase in carbohydrate helps in providing for an adequate reserve of
glycogen, which is the storage form of carbohydrate. Carbohydrates should be
the major source of calories for people with liver disease.
Carbohydrate intake in hepatic porphyria is not only necessary to maintake the
blood glucose levels and prevent acute attacks of porphyria due to porphyrin
overproduction, but carbohydrate intake is necessary to provide glycogen.
SOURCE:
Sheryl WIlson MNS, RD (HCP)
+++++++++++
Anemia, peripheral neuropathy, and nutritional deficiencies may be treated with
medication and vitamin supplementation.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++
Beware of the use of herbal supplements.
Most herbal supplements have not been studied in porphyria patients and many
can not be taken in compbination with other medications.
Herbal supplements also have not been FDA approved.
SOURCE:
Sheryl Wilson MNS, RD, (HCP)
+++++++++++
Ascites and edema may be worsened by salt intake because salt holds on to
water in the body.
Most people with live disease will need to have a sodium restricted diet.
The liver is involved with the metabolism of all foods.
Therefore, proteins, carbohydrates, fats, vitamins, sodium (salt), and fluids may
be affected by nutritional intake.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++
Because liver disease can affect the absorption of all types of food, weight loss,
anorexia and vitamin deficiency are major side effects.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++
It is good to remember that many porphyria patients have abnormal metabolism
of carbohydrate.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++
Prolonged protein restriction can cause deficiencies of certain amino acids.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++
The dietary recommendations may vary somewhat depending on how well the
person's liver is functioning.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++
It is important to have LFT's routinely performed periodically.
LFT's are liver funciton tests and can indicate the functioning of the liver and
progression of any liver disease.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++
It is very important to be under the care of a health professional otherwise
malnutrition can lead to serious consequences.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++
Acute porphyria patients need a high intake of carbohydrate foods.
SOURCE:
Sheryl WIlson MNS, RD (HCP)
+++++++++++
Carbohydrates should be the major source of calories in this diet.
SOURCE:
Dr. Karl E. Anderson MD
Porphyria Specialist
University of Texas Medical School
Galveston, TX
+++++++++++
Remember to avoid grapefruit intake since grapefruit in any form affects heme
synthesis and can trigger porphyria.
SOURCE:
Sheryl WIlson MNS, RD (HCP)
+++++++++++
SAMPLE MENU
breakfast
1 orange
cooked oatmeal, with milk and sugar
1 slice of whole-wheat toast
2 teaspoons of margarine
strawberry jam
coffee or tea
lunch
4 ounces of cooked lean fish, poultry, or meat
a starch item (such as potatoes)
a cooked vegetable
salad
2 slices of whole-grain bread
2 teaspoons of margarine
1 tablespoon of jelly
fresh fruit
milk
midafternoon snack
milk with graham crackers
dinner
4 ounces of cooked fish, poultry, or meat
starch item (such as potatoes)
a cooked vegetable
salad
2 whole-grain rolls
2 tablespoons of margarine
fresh fruit or dessert
8 ounces of milk
evening snack
high protein milk (milk with non-fat dry milk added)
Usually, there are no cautions against specific foods.
However, many people are unable to tolerate strongly flavored vegetables,
high-fat foods, fried foods, chocolate, nuts, and foods that have a lot of
seasoning.
If you have questions about your diet or your symptoms contact your physician
immediately.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++
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