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NUTRITIONAL INFO FOR PORPHYRICS

Nutritional Guidelines For People With Porphyria

AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD

The following dietary guidelines are recommended for patients with the acute hepatic inherited porphyrias or those with the newly described acquired acute toxic porphyria.

Energy intake should be prescribed at a level to maintain a desirable body weight.  A desirable weight is that of your BMR [basic metabolism rate] If you are dieting you are NOT to go more than 10% below your BMR. Carbohydrate intake during period of remission should be 55 to 60 percent of total energy intake. During an acute attack your must have an intake of a minimum of 400 - 500 grams of carbohydrate.  This is includes intravenous glucose as well as consumed carbohydrate. During an attack the main focus is carbohydrate intake, low fat, and minimal protein which will be combined with carbohydrate focused foods such as nonfat skim milk, oatmeal, oat bran or other carbohydrate foods, with the protein being secondary, and not a meat or egg entree which contain no carbohydrate at all.

Protein intake should follow the RDA during remission.  During attacks as previously mentioned. If their is any kidney impairment protein must be reduced. Total fat intake should be less than 30 percent of total calories.
Be sure to keep the saturated fats to a minimum. Cholesterol intake should be less than 300 milligrams per day. Artificial sweeteners are acceptable. Remember that some may cause an increase in diarrhea during an acute attack. Salt intake need not be restricted unless it is important for controlling hypertension. The majority of porphyria patients do not have chronic hypertension and therefore salt restriction will not be addressed here. Remember that during acute attacks often some of the glucose IV will contain saline solution, while other may be only water. This is important to remember if you have problems with edema.  Then you should use the water based glucose only.

Intakes of vitamins and minerals should meet the RDAs. Calcium intake in women should be at least one gram daily. Iron intake should be adequate to avoid iron deficiency. Women with heavy menstrual blood loss and patients who have had frequent blood drawings due to illness and hospitalization may require greater intakes of iron. (Iron
is a component of heme. Iron deficiency can compromise heme synthesis and may exacerbate porphyria. Therefore, iron deficiency should be avoided in porphyria. Early iron deficiency occurs before there is anemia. Anemia means having a low blood count. Also an early iron deficiency can be detected by tests such as serum iron and iron-binding capacity, and serum ferritin.

All alcoholic beverages should be avoided by porphyric patients. Alcohol stimulates the heme biosynthetic pathway in the liver and can itself exacerbate porphyria. Medical drugs books list alcohol as being contraindicated for porphyrics. Alcohol has other harmful effects and can lead to weight gain.

Fiber intake should be about 40 grams per day. High dietary fiber intakes should be avoided in patients with upper gastrointestinal problems. The reason for this is because sometimes excess fiber can accumulate in the form of "bezoars." Also the increasing of dietary fiber intake sometimes causes abdominal cramping, diarrhea and flatulence. These can be minimized by increasing fiber intake gradually.

Foods contain many natural chemicals that can stimulate the heme biosynthetic pathway. Although none have been definitely linked to attacks of porphyria, the possibility that these chemicals might contribute should be kept in mind especially when attacks of porphyria recur in the absence of a definite inciting factor. These foods are considered porphyrinogenic.

Some of the dietary factors that might have an adverse effect on porphyria include charcoal-broiled meats (which contain chemicals similar to those found in cigarette smoke), certain vegetables (such as cabbage and brussel sprouts. and red grapes which may contain chemicals that in large amounts can stimulate heme and porphyrin synthesis. A porphyric will also want to avoid high intakes of protein. Most likely,  none of these foods need to be completely avoided in porphyria.

One should also remember that tomatoes contain a high amount of sulphur, and that sulphur is contraindicated in porphyrics. Tomatoes can be eaten, but in moderation. It should also be noted that they may be contraindicated due to aggravation of cutaneous symptoms of porphyria.

The bottom line in dietary considerations in acute hepatic porphyrias is that it important to consume a well-balanced diet and not to consume any particular type of food in excess.

Food intake should be consistent, but should take into account lifestyle and physical activity.

Daily caloric energy intake in porphyric patients should be distributed consistently throughout the day, with the highest amount of carbohydrate intake  being consumed upon waking after a period of fast [overnight sleep].

It is important to eat every 2-3 hours throughout the waking portion of one's day.  It is also important to consume high carbohydrate upon retiring in order to reduce the body fasting during the sleep.

Nutritional management of acute attacks of porphyria Intravenous administration of glucose (a pure form of carbohydrate) is part of the standard treatment of acute attacks of porphyria. Glucose is given by vein because the stomach and intestine usually do not function properly during an attack, and material taken by mouth is not properly propelled through these organs.

Glucose and other carbohydrates can repress the pathway for synthesis of heme in the liver. As a result, the overproduction of porphyrin precursors
and porphyrins is repressed by carbohydrate administration.

Heme therapy (intravenous administration of hematin or heme arginate)
has a similar but much more potent effect, and probably leads to more rapid improvement. However due to coagulation problems with heme, most clinician orders the use of glucose for up to 36 hours after which
they consider hematin.

If a patients is unable to consume orally an nutrients the patients may
undergo parental feeding.  This is a total intravenous feeding which is
important to administer glucose and other nutrients.

Such a feeding is used if an acute attack is severe or prolonged,
sufficient glucose can be given by vein to meet the total energy requirements of a patient. This is best accomplished by a catheter that is inserted into a large central vein.

Additional nutrients, including vitamins, minerals, amino acids and fat can be given in the required amounts to maintain all requirements. Provision of
total nutritional needs in this manner by vein is commonly called "total
parenteral nutrition".

Fasting or dieting is not permitted in people who have porphyria,
Fasting or extreme dieting can provoke an acute attack.
Most porphyrics who experience repeated attacks find weight a problem.
The extra intake of sugar or glucose that is required to cure an acute
attack may lead to a problem of overweight. Dieting should be avoided.

A diet should be rich in fiber as constipation is a often a symptom  of an
acute porphyric  patient.

Particles of insecticides along with heavy metal toxins are stored in the
liver. Eating liver should be avoided especially from wild game and
poultry.

In order to avoid insecticides in green vegetables and root vegetables,
always peel and rinse them properly. Potatoes should be peeled before
cooking.

Only fruits that are not sprayed should be eaten without peeling, peel all
other fruits.

It is recommended to consume 6-8 (8oz) glasses of water along with other
beverages high in glucose per day. (Gatorade, rice water, Pedialite and
sport drinks are excellent sources of beverages high in glucose)


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