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)
|
