|
PORPHYRIA FACTS: NUTRITION IN PORPHYRIA sent
ASSORTED TOPICS ON NUTRITION
Quite often porphyria patients when they go to the clinic or hospital while
experiencing an acute attack often will relate that they have not felt well enough
to eat or have been unable to eat.
Ironically it is the very treatment they need,high consumption of carbohydrate
containing foods.
Often it is felt that if the porphyria patient had not skipped over the consumption
of carbohydrate containing foods, that they would have aborted their own acute
attacks and not needed to present for ermgency interventional care.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++++
Alcohol
Alcohol should be avoided by all porphyria patients..
SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++
Alcohol stimulates the heme biosynthetic pathway in the liver and can itself
exacerbate porphyria.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
++++++++++++++
Medical drugs books list alcohol as being contraindicated for porphyrics.
Alcohol has other harmful effects and can lead to weight gain.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++
Artificial sweeteners
Artificial sweeteners are acceptable.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++
Beta carotene
All cutaneous porphyrias can be alleviated by avoidance of sunlight.
Treatment of erythropoietic protoporphyria involves administering large doses
of beta-carotene, which may improve tolerance to sunlight.
SOURCE:
Management of acute and cutaneous porphyrias.
Badminton MN, Elder GH.
Department of Medical Biochemistry
University of Wales College of Medicine
Cardiff, UK.
Int Journal of Clinical Practice
2002 May;56(4):272-8.
++++++++++++++
Beverages
In mild attacks the use of high calorie drinks helps bring attacks under control.
SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++
Bezoars
Bezoars, also known as trichobezoars or hairballs are common with the
intake of too much fiber.
A ball of swallowed fiber that collects in the stomach and fails to pass through
the intestines.
A barium swallow X-ray will show the mass in the stomach, sometimes a scope if
used (endoscopy) to directly visualize the bezoar.
The bezoar may need to be surgically removed (especially trichobezoars which
tend to be large).
Sometimes small bezoars can be removed through a scope placed
through the mouth and into the stomach.
Persistent vomiting that can be assoiated,can lead to dehydration.
SOURCE:
Jonathan Fanaroff, M.D.
Department of Pediatrics
Rainbow Babies and Children's Hospital
Case Western Reserve University
Cleveland, OH.
+++++++++++
Bone loss
Research studies have found that calcium supplements, if taken regularly, help
prevent osteoporosis by reducing bone loss.
Bread products and orange juice, are enriched with calcium to make
them a significant source of calcium for people whose dairy product consumption
is inadequate.
SOURCE:
Calcium
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++++
Calcium
Calcium intake in women should be at least one gram daily.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
+++++++++++++
Teeth also need calcium to remain healthy.
The bones incorporate calcium into their structure. Like other tissues in the
body, bones are continually being reabsorbed and re-formed.
SOURCE:
Calcium
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++++++
Carbohydrate
In severe attacks of porphyria administration of intravenous dextrose, at least
1,500-2,000kcal/24 hour ofcarbohydrate is required.
SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++
High carbohydrate intake ameliorates symptoms.
SOURCE:
Porphyria
Dictionary of Medical Syndromes
Fourth Edition 1997
Lippincott-Raven
++++++++++++
In many acute porphyria patients the onset of an acute attack can beaborted by
increasing carbohydrate intake.
SOURCE:
The Porphyrias
Kappas, A. et. al.
The Metabolic Basis of Inherited Disease
7th Edition 1995
+++++++++++
Remember that daily requirements run 300 mg or better of carbohydratedaily.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++
Dietary restriction of carbohydrates is a precipitating factor of AIP.
SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison’s Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++
Most acute porphyria patients suffer from nausea and vomiting during an attack
and their poor carbohydrate intake aggravates the disease process.
SOURCE:
Dr. Michael R. Moore
Porphyria Research Unit
University of Queensland
Department of Medicine
Brisbane, Australia 1999
++++++++++++
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].
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++
Many porphyics fail to realize the importance or ignore the importance of their
carbohydrate [sugar] intake.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++
It is also important to consume high carbohydrate upon retiring in order to
reduce the body fasting during the sleep.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++
It is important to eat every 2-3 hours throughout the waking portion of one's day.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++
Cholesterol
Limit dietary cholesterol.
To accomplish this goal, limit or avoid concentrated sources such as organ
meats, egg yolks and whole-milk products.
Of course, for porphyria patients, it is bestto avoid such products because often
theseproducts contain triggering elements such as steroids, sulphur, and
chemical toxins.
SOURCE:
Sheryl WIlson RD
+++++++++++
Dieting
Dieting or fasting should be avoided.
If weight loss is required the advice of a dietitian should be sought.
SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++++
Reduction of carbohydrates may precipitate acute attacks.
SOURCE:
Disorders of Porphyrins or Metals
Dwight Montgomery Bissell M.D.
# 203
pp1182-7
Cecil Textbook of Medicine
Volume 1 18th Edition
+++++++++++++
Attacks are often precipitated by low intake of carbohydrates and calories in an
attempt to lose weight.
Thus dietary counseling is very important.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++
Poor metabolism or insufficient carbohydrate is a keey factor in the acute
hepatic porphyrias.
The decrease in activity of erythrocyte aminolevulinate dehydratase observed in
diabetic ALA-D hepatic porphyria patients, may represent an additional and
useful parameter for the assessment of the severity of carbohydrate
metabolism impairment.
SOURCE:
Intternational Journal of Biochemistry and Cell Biology
1999
Mar-Apr;31(3-4):479-88
Delta aminolevulinate dehydratase (ALA-D) activity in
human and experimental diabetes mellitus.
Fernandez-Cuartero B et. al.
Department of Internal Medicine
University Complutense of Madrid,
Spain.
++++++++++++++
Acute Intermittent Porphyria (AIP) patients should avoid decreased caloric
(carbohydrate) intake.
SOURCE:
Porphyria
New Vision Medical Services
1997
++++++++++++++
Fasting
The severity and extent symptoms will vary from patient to patient and from day
to day, depending in part on internal or endogenous factors such as fasting.
SOURCE:
The Canadian Porphyria Foundation
Neepawa, Manitoba, Canada
+++++++++++
Fasting in porphyria patients must be avoided.
SOURCE:
Disorders of Porphyrins or Metals
Dwight Montgomery Bissell M.D.
# 203
pp1182-7
Cecil Textbook of Medicine
Volume 1 18th Edition
++++++++++++
Often it is felt that if the porphyria patient had not skipped over the consumption
of carbohydrate containing foods, that they would have aborted their own acute
attacks and not needed to present for ermgency interventional care.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++
Fat
Control total fat.
Limit all types of fat - saturated, polyunsaturated, trans fatty acids (trans fats)
and monounsaturated - to no more than 30 percent of your total daily calories.
Because all foods with fats contain a combination of these fats, it's important to
reduce total fat.
If your daily intake is 2,000 calories, 30percent equals 65 grams of fat.
Limit saturated fat to no more than 10 percent of total calories.
SOURCE:
Sheryl WIlson RD
+++++++++++
If there 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.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
Fiber
Fiber intake should be about 40 grams per day.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++
Foods high in soluble fiber are varied.
Such foods include rice bran, oat bran, oatmeal, beans, peas, barley,
citrus fruits, strawberries and apple pulp.
SOURCE:
Sheryl Wilson, RD
++++++++++++
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."
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++
Folic Acid
The body uses synthetic folic acid more easily than it uses natural folate.
The body absorbs almost 100 percent of the synthetic folic acid compared with
about 50 percent of the natural folate.
SOURCE:
Sheryl Wilson RD
++++++++++++
Glycemic foods - low index
Low glycemic foods can curb the appetite and help with weight management.
The glycemic response to a food varies from person to person, as well as from
meal to meal (depending on the combinations of foods eaten).
SOURCE:
Carbohydrate Confusion
Nancy Clark, MS, RD
Director of Nutrition Services
SportsMedicine Associates
Brookline MA. April 2002
++++++++++++++
Glycogen
Carbohydrates stored in the body are called glycogen.
The muscles can only store small amounts of glycogen.
If the body is not taking in enough carbohydrate then a person runs out of
glycogen.
Without the glycogen it greatly affects your stamina and performance.
Fatigue and weakness will set in.
SOURCE:
Sheryl Wilson RD
++++++++++++
Iron Deficiency
Iron deficiency can compromise heme synthesis and excerbate porphyria.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
+++++++++++
Iron is a component of heme.
Iron deficiency can compromise heme synthesis and excerbate porphyria.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
++++++++++++
Melons
Some people allergic to ragweed feel symptoms spike suddenly after eating
honeydew melon, cantaloupe or bananas.
Symptoms can also spike after drinking chamomile tea, using soap or face
powder containing chamomile, or taking herbal supplements that include it.
SOURCE:
AETNA IntellaHealth
September 5, 2002
Common Foods Worsen Symptoms
++++++++++++++
Meats
Particles of insecticides along with heavy metal toxins are stored in the liver.
Eating liver should be avoided especially from wild game and poultry.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++
Nausea
Bananas are also part of the BRAT diet, a diet many physicians and nurses
recommend for personal recovering from gastrointestinal upsets, particularly
diarrhea.
BRAT stands for the different components that make up the diet: Bananas, Rice
cereal, Applesauce, Toast.
SOURCE:
Victoria Kennedy, RN,
J. Gordon Lambert, MD, Associate Medical Director
RxRemedy
++++++++++++
Neurotoxic foods
Try to avoid foods or drinks high in caffeine, including coffee, chocolate and
sodas.
Caffeine can make neuropathy and pain worse."
SOURCE:
Mayo Health News
Mayo Clinic
Rochester, Minnesota
++++++++++++
Niacin Deficiency
In niacin (B3) deficiency the Gastrointestinal manifestations include glossitis,
cheilosis, stomatitis, nausea, vomiting, and diarrhea.
If untreated death occurs.
SOURCE:
Advanced Nutrition and Human Metabolism
Journal . Groff (251)
+++++++++++++
Parental feeding
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.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++
If a patient 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.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++
Peeling fruit
Only fruits that are not sprayed should be eaten without peeling, peel all other
fruits.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++
Porphrogenic foods
Avoid foods such as red grapes, cabbage, brussel sprouts, red plums, all which
may contain chemicals that in large amounts can stimulate heme and porphyrin
synthesis.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++
Protein
If there is any kidney impairment protein must be reduced.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++
Phytochemical supplements
It is not necessary to be taking phytochemical supplements.
If you are eating a variety of foods that are rich in these substances you will
consume enough phytochemicals.
Phytochemical-rich foods like brightly colored fruits and vegetables, and whole
grains and legumes contain much more than phytochemicals.
These foods are healthful for a variety of reasons, like being naturally low in fat
and rich in vitamins and minerals.
SOURCE:
Phytochemicals
L.B. Bobroff et. al.
Institute of Food and Agricultural Sciences
University of Florida
1998
+++++++++++
Salt intake
Salt intake need not be restricted unless it is important for controlling
hypertension.
The majority of porphyria patients do not have chronic hypertension.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++
Vitamin C
Hydrogen or electron donors, such as vitamin C, are believed to inhibit the
transformation of porphyrinogens to porphyrins.
Also, vitamin C will facilitate the conversion of the products to cytochromes and
hemoglobin, reducing the buildup of intermediate products in the above
pathway.
SOURCE:
Porphyria
Greek for "Purple Urine"
Orthomolecular Medicine
+++++++++++
Vitamin Supplementation
Chemical toxicity in normally healthy persons is a slow recovery process.
In some cases it is a very slow process and recovery may only be partial after
pyridoxine ingestion is stopped.
In a porphyria patients the damage can be even more severe and damage may
become permanent.
SOURCE:
Jeff Danniels, PhD.
Molecular Biology & Alternative Medicine
++++++++++++
Soy products
Soy is a food products that is found in many forms.
For most people soy does not cause any problems.
However acute porphyria patients as well as PCT patients, soy products should
be avoided.
Soy contains phytoestrogens. Estrogen is to beavoided by all PCT patients and
is thought to be a trigger in the acute porphyrias.
SOURCE:
Dr, Robert Johnson M.D.
+++++++++++++
Sulphites
A widely used preservative and antioxidant in foods. Often used to keep
lettuce fresh.
It is also common in packaged potato products, as well as other produce, certain
types of seafood and even in beer and wine. They are currently used in over
1,000 drug products both prescription and nonprescription.
Common label names: sulphur dioxide, potassium bisulphite, potassium
metabisulphite, sodium metabisulphite or sodium sulphite.
SOURCE:
Gray's Medical Encylopedia
+++++++++++++++
Washing fresh produce
Limit your exposure to chemical toxins.
Buy organic foods, stay away from prepared foods clean fruits and vegetables
thoroughly with asolution of food- grade hydrogen peroxide, limit use of
chemicals in your home and garden.
SOURCE:
Chemical Overload
A Little Bit Won't Hurt You - Or Will It?
Dr. Regan Golob DC
++++++++++++
Porphyrias are due to deficiencies in enzymes in the chemical pathway that
makes heme from porphyrins and other precursor substances.
This pathway of enzymes is called the heme biosynthetic pathway. Enzyme
deficiencies in the porphyrias are usually inherited.
However, the enzyme deficiencies along do not produce disease.
Additional factors determine whether or not there will be disease manifestations.
Diet is one of the additional factors that influences the manifestations of certain
types of porphyria.
The so-called acute porphyrias, which are acute intermittent porphyria,
hereditary coproporphyria and variegate porphyria are characterized by acute
attacks of abdominal pain and other symptoms.
Attacks of these diseases can be brought on by restricting intakes of
carbohydrate and energy. (Energy is measured in calories or kilocalories.)
Conversely, providing a normal or increased intake of carbohydrate and energy
is part of the prevention and treatment of attacks of porphyria. Therefore,
attention to diet is particularly important in these three diseases.
The acute porphyrias are affected by diet because the chemical pathway in the
liver that makes heme from porphyrins and other precursor substances is very
sensitive to intakes of carbohydrate and energy.
In the acute porphyrias porphyrin precursors (8- aminolevulinic acid and
porphobilinogen) and porphyrins are produced in excess amounts by the liver.
Porphyrin precusors are in excess especially during acute attacks of porphyria.
SOURCE:
Diet
Pamphlet published by
American Porphyria Foundation
++++++++++++++++++++++++++++
carbohydrate
Carbohydrates are very abundant compounds, usually an aldehyde or ketone
derivative of a polyhydric alcohol, particularly of the pentahydric and hexahydric
alcohols.
They are so named because the hydrogen and oxygen are usually in the
proportion to form water with the general formula Cn(H2O)n.
The most important carbohydrates are the starches, sugars, celluloses and
gums.
They are classified into mono, di, tri, poly and heterosaccharides.
The smallest are monosaccharides like glucose whereas polysaccharides such
as starch, cellulose or glycogen can be large and indeterminate in length.
Acute hepatic porphyria patients must be sure to have a minimum of 400 mg of
carbohydrate per 24 hours to bring an attack under control.
During remission a porphyria patient must be sure to consume at least 350 mg
minimum of carbohydrate.
++++++++++++++++++
Carbohydrate metabolism is the breakdown of starches and sugars into smaller
units that can be used by the body for energy.
+++++++++++++++++++
Nutritional and metabolic diseases
A collective term for nutritional disorders (result of poor assimilation or utilization
of food) and metabolic disorders (result of poor metabolism or inherited enzyme
abnormality).
SOURCE:
Sheryl WIlson MNS RD
++++++++++++++++++
Nutritional support
The administration of nutrients for assimilation and utilization by a patient by
means other than normal eating.
It does not include fluid therapy which normalises body fluids to restore
water-electrolyte balance.
SOURCE:
Sheryl WIlson MSN RD
+++++++++++++++++++
Nutritive value
An indication of the contribution of a food to the nutrient content of the diet.
This value depends on the quantity of a food which is digested and absorbed
and the amounts of the essential nutrients (protein, fat, carbohydrate, minerals,
vitamins) which it contains.
This value can be affected by soil and growing conditions, handling and storage,
and processing. In the acute porphyrias it is important to know the nutritive
values for carbohydrate and proteins as well as caloric value.
SOURCE:
Sheryl WIlson MSN RD
+++++++++++++++++++++
What is the action of carbohydrates on porphyrins?
Carbohydrate intake blocks ALA-synthase, which has been demonstrated in
numerous clinical and experimental studies .
However, the mechanisms by which carbohydrates modulate the components of
porphyrins and heme synthesis are highly complex and only partially elucidated
to date.
SOURCE:
Effects of diabetes mellitus
on patients with acute
intermittent porphyria
C. Andersson et. al.
Journal of Internal Medicine
Volume 245
Issue 2 Page 193 -
February 1999
++++++++++++++++++++++++++
How much carbohydrate is needed daily during acute attacks?
Carbohydrates should be administered either orally or by i.v.
so that the equivalent of 300 to 500 grams of glucose are given daily.
SOURCE:
Wintrobe's Clinical Hematology
Dr. Karl E. Anderson
"The Porphyrias
++++++++++++++++++++++++++++
What is the purpose of carbohydrate loading?
Carbohydrate loading is the specific therapy for hepatic porphyria attacks
because it represses hepatic ALA synthase and the overproduction of ALA and
PBG.
SOURCE:
Dr. Karl E. Anderson, MD
The Porphyrias
Cecil Textbook of Medicine
W.B. Saunders
++++++++++++++++++++++++++
What does the carbohydrate loading do?
Carbohydrate loading works by forcing your body into storing more glycogen.
By eating significantly more carbohydrates, you fill your muscles with excess
glycogen that can be used as fuel, delaying fatigue,and at the same time
releasing the neededcabohydrate (glycogen) to the liver to stopthe
overproduction of porphyrins.
It is the overproduction of porphyrins which cause generate porphyria attacks.
SOURCE:
Sheryl Wilson, RD
+++++++++++++++++++++++++++
Carbohydrate loading is the specific therapy for hepatic porphyria attacks
because it represses hepatic ALA synthase and the overproduction of ALA and
PBG.
SOURCE:
Dr. Karl E. Anderson, MD
The Porphyrias
Cecil Textbook of Medicine
W.B. Saunders
+++++++++++++++++++++++++++
What is carbohydrate loading?
Porphyria patients usually require hospital care when an accute
attack occurs, because the clinical course of an acute attack is difficult to
predict.
Carbohydrates should be administered either orally or by i.v. so that the
equivalent of 300 to 500 grams of glucose are given daily..
This therapy is based on the observation that a high-carbohydrate intake can
stop the overproduction of porphyrins which cause the attack.
The response to carbohydrate loading [the glucose effect] is variable and in
many cases, especially the chronic, the attack will persist.
If improvement is not observed within 24 hours iv hematin should be
initiated.
SOURCE:
Wintrobe's Clinical Hematology
Dr. Karl E. Anderson
"The Porphyrias"
+++++++++++++++++++++++++
Steps should be taken to ensure an adequate carbohydrate intake.
SOURCE:
Dr. Michael R. Moore
Porphyria Research Unit
University of Queensland
Department of Medicine
Brisbane, Australia 1999
+++++++++++++++++++++++++++
What are good carbohyrate foods to be eaten duribng an acute attack?
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.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++
What is glycogen?
Carbohydrates stored in the body are called glycogen.
The muscles can only store small amounts of glycogen.
If the body is not taking in enough carbohydrate then a person runs out of
glycogen.
Without the glycogen it greatly affects your stamina and performance.
Fatigue and weakness will set in.
SOURCE:
Sheryl Wilson RD
+++++++++++++++++++++++++
Is use of glucose during acute attacks part of the overall total nutritional
management of porphyria?
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.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++
What does glucose do for the patient?
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.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++
What things are neccesary to know regarding nutriion in acute porphyria?
Porphyria attacks are caused by the overproduction of porphyrins in the liver.
Overproduction of porphyrins occurs when a person hass too low of a caloric
intake, especially carbohydrate containing foods.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++
What is necessary dietary carbohydrates in acute porphyria?
Carbohydrates present in food comprising digestible sugars and starches
andindigestible cellulose and other dietary fibers.
The former are the major source ofenergy.
The sugars are in beet and cane sugar, fruits, honey, sweet corn, corn
syrup,milk and milk products, etc.; the starches are in cereal grains, legumes,
tubers, etc.
In patients with hepatic forms of porphyria, a person should consume at least
350 mg ofcarbohydrate per day, or the carbohydrates should make up 60-65% of
the dailyconsumption.
SOURCE:
Sheryl WIlson MSN, RD
++++++++++++++++++++++
A minimum of 300 grams of carbohydrate per 24 hours has been found to control
dietary precursors of acute attacks.
SOURCE:
The Porphyrias
D. Montgomery Bissell M.D.
+++++++++++++++++++++
When a patient has exacerbation of another medical condition, especially
conditions like influenza where vomiting and diarrhea may occur, it is necessary
to have carbohydrate intake of 400-500 grams, which often includes intravenous
infusion of glucose with necessary added electrolytes.
SOURCE:
Rafael Lumonosa M.D.
Vascular Medicine & Infusion Therapy
++++++++++++++++++++
+++++++++++++++++++++++++
Are carbs fattening?
Carbohydrates are not inherently fattening.
Excess calories are fattening.
Excess calories of carbohydrates (bread, bagels, pasta) are actually less
fattening than are excess calories of fat (butter,
mayonnaise, frying oils) because the body has to spend calories to convert
excess carbohydrates into body fat.
In comparison, the body easily converts excess calories of dietary fat into body
fat.
This means, if you are destined to be gluttonous but want to suffer the least
weight gain, you might want to indulge in (high carb) frozen yogurt instead of
(high fat) gourmet ice cream.
SOURCE:
Carbohydrate Confusion
Nancy Clark, MS, RD
Director of Nutrition Services
SportsMedicine Associates
Brookline MA. April 2002
+++++++++++++++++++++++
What are the nutritional requirements of carbohydrate?
"Many porphyics fail to realize the importance or ignore the importance of their
carbohydrate [sugar] intake.
A steady daily amount is necessary to suppress disease activity.
Remember that daily requirements run 300 mg or better of carbohydrate daily.
During an acute attack a porphyric's requirement runs 500 mg of carbohydrate
total including both consumed and iv infusion of carbohydrate.
Quite often porphyria patients when they go to the clinic or hospital while
experiencing an acute attack often will relate that they have not felt well enough
to eat or have been unable to eat.
Ironically it is the very treatment they need, high consumption of carbohydrate
containing foods.
Often it is felt that if the porphyria patient had not skipped over the consumption
of carbohydrate containing foods, that they would have aborted their own acut
eattacks and not needed to present for ermgency interventional care."
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++++++++++++++++
What does low carbohydrate intake and vomiting do during an attack?
Most patients suffer from nausea and vomiting during an attack and their poor
carbohydrate intake aggravates the disease process.
SOURCE:
Dr. Michael R. Moore
Porphyria Research Unit
University of Queensland
Department of Medicine
Brisbane, Australia 1999
+++++++++++++++++++++++++++
Are carbohydrate necessary in VP?
Extreme carbohydrate-restricted dieting or fasting should be avoided.
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
Maureen Poh-Fitzpatrick, MD
Department of Internal Medicine
Division of Dermatology
University of Tennessee
College of Medicine
++++++++++++++++++++++++++
Are there differences in the types of carbohydrates we eat?
Eating too many carbs that are refined or in the form of sugar can make it
difficult to control weight because they're quickly absorbed, sending blood sugar
levels soaring, followed by high surges of insulin.
After two or three hours, the large amounts of insulin bring the bloodsugar levels
crashing down, sometimes even below normal levels.
At this point, people feel strong urgesto look for snacks well before the next
meal.
Over many years, these powerful surges of insulin exhaust the pancreas to the
point where diabetes can develop.
SOURCE:
Weighs and Means
Controversy Surrounds Role
of Carbs in Weight-Loss Diets
Alexa Pozniak
Too Many Carbs?
++++++++++++++++++++++++++++++
Is it important to eat a high level of carbohydrates?
Sufficient levels of carbohydrate must be retained in the liver at all times.
SOURCE:
Disorders of Porphyrins or Metals
Dwight Montgomery Bissell M.D.
# 203
pp1182-7
Cecil Textbook of Medicine
Volume 1 18th Edition
+++++++++++++++++++++++++++++++
What is hypoclycemia and how do it parallel porphyria onsets?
"Hypoglycemia occurs when your body's blood sugar, or glucose, is abnormally
low.
Hypoglycemia results when your body's glucose is used up too rapidly, when
glucose is released into the bloodstream more slowly than is needed by your
body.
Hypoglycemia is relatively common in acute porphyria patients
Hypoglycemia and onset of acute porphyria attacks both happen when not
enough carbyhydrate containing food is eaten, or from a sudden increase in the
amount of exercise without an increase in food intake.
Sometimes the cause of hypoglycemia is unknown, or idiopathic
but has become realized in acute porphyria as a "supply and demand" signal for
carbohydrate intake.
People who are not diabetic and who do not have another known causes of
hypoglycemia experience hyperglycemia due to low intake of carbohydrates.
It has long been known that Hypoglycemia can occur because of an liver
disease such as porphyria.
Here is a simple formula to remember the relationship of serum blood glucose
monitoring and onset of porphyria.
Remember this fomula:
LOW BLOOD SUGAR =
LOW CARBOHYDRATE LEVEL =
TRIGGERING OF ACUTE PORPHYRIA
It is a known factor in acute porphyrias, that over production of porphyrins
begins when the body is lacking in carbohydrate.
An acute attack of porphyria can be triggered by inadequate caloric intake and
more specificaly insufficient amounts of carbohydrate.
When carbohyrate levels falls in the body, the serum blood sugar levels falls as
well, thus when blood testing is done, the results will show low end blood sugar
levels. The normal range runs 70-110 for blood sugar monitoring.
When a porphyria patients is nearing the onset of an attack due to insufficient
carbohydrate levels the body begins overproduction of porphyrins which
eventually trigger an attack.
At this same time when carbohydrate levels are low enough to trigger porphyrin
production, a porphyria patient will often experience hyperglycermia.
Blood testing will indicate blood serum levels below 70, and the symptoms of
hyperglycemia will become noticeable in the patient.
Many acute porphyria patients have found relative sucess in monitoring their
blood sugars to be a good indicator of pending porphyria activity.
When a patient feels tired, ill-feeling, perhaps overly tired without good reason, it
is time to take a blood serum glucose testing.
While a normal fasting blood serum range runs 70-110, anything below 75 for a
porphyria patient who carboloads, should be a "heads up" . If 60 or below the
porphyria patient needs to alert a caretaker and begin infusion.
SOURCE:
Dr. Robert Johnson
Internal Medicine
++++++++++++++++++++++++
Can low glycemic foods help slow the desire to eat?
Low glycemic foods can curb the appetite and help with weight management.
The glycemic response to a food varies from person to person, as well as from
meal to meal(depending on the combinations of foods eaten).
SOURCE:
Carbohydrate Confusion
Nancy Clark, MS, RD
Director of Nutrition Services
SportsMedicine Associates
Brookline MA. April 2002
++++++++++++++++++++++++
Are carbohydrates with low glycemic effect nutritionally better for a porphyric?
Foods with a low glycemic effect tend to be wholesome, fiber-rich fruits,
vegetables and whole grains that are health protective and satiating.
SOURCE:
Carbohydrate Confusion
Nancy Clark, MS, RD
Director of Nutrition Services
SportsMedicine Associates
Brookline MA. April 2002
++++++++++++++++++++++++
What does high blood sugar cause?
High blood glucose triggers the body to secrete insulin to carry the sugar out of
the blood.
Insulin can stimulate the appetite, as well as fat deposition.
SOURCE:
Carbohydrate Confusion
Nancy Clark, MS, RD
Director of Nutrition Services
SportsMedicine Associates
Brookline MA. April 2002
+++++++++++++++++++++++
Malabsorption
Malabsorption is the impaired intestinal absorption of nutrients.
Malabsorption syndromes
Malabsorption syndrome is the general term for syndromes of malnutrition due to
failure of normal intestinal absorption of nutrients.
Malnutrition
Malnutrition is the faulty or inadequate nutrition. Under nourishment.
Nutrition units are known as a calorie
A unit of measurement defined as 4.184 absolute joules or the amount of energy
it takes to raise the temperature of one gram of water from 15 to 16 degrees
Celsius (or1/100th the amount of energy needed to raise the temperature of one
gram of water at one atmosphere pressure from 0 degrees C to 100 degrees C),
food calories are actually equal to 1,000 calories (1 food calorie = 1
kilocalorie).
SOURCE:
Sheryl WIlson MNS, RD
+++++++++++++++++
PORPHYRIA FACTS: NUTRITION - FOODS THAT ARE TRIGGERS
Porphyria patients can avoid bad drugs, environmental and industrial toxins and
avoid infection, but can still trigger themselves due to porphyringenic foods.
Not all of the foods will trigger all porphyria patients, as all patients have
different sensitivities and react differently, however all patients should be aware
of such foods and the additives and preservatives which can trigger attacks.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++
Does one have to avoid foods that are porphyrinogenic?
Avoid porphyrinogenic foods. Such foods can trigger heme synthesis.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++
Can some foods trigger porphyria?
Certain vegatables such as cabbage and brussel sprouts may contain chemicals
that can stimulate heme synthesis.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
+++++++++++++++++++++++++
Can certain foods trigger a porphyria attack?
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.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++
Estrogen is to be avoided by all PCT patients and is thought to be a trigger in
the acute porphyrias.
SOURCE:
Dr, Robert Johnson M.D.
++++++++++++++++++++++++++
Can plant al;lergies be the same as food allergies?
Allergy-stimulating proteins in pollens released by plants are chemically similar
or even identical to those in some fruits and vegetables.
SOURCE:
Dr. Leonard Bielory
Asthma and Allergy Research Center
University of Medicine and Dentistry of New Jersey.
++++++++++++++++++++++++++
Tomatoes contain sulphur and should be avoided since sulphur is a trigger.
SOURCE:
Audrey Winter RD
++++++++++++++++++++++++++
Can melons cause allergic reactions?
Some people allergic to ragweed feel symptoms spike suddenly after eating
honeydew melon, cantaloupe or bananas.
Symptoms can also spike after drinking chamomile tea, using soap or face
powder containing chamomile, or taking herbal supplements that include it.
SOURCE:
AETNA IntellaHealth
September 5, 2002
Common Foods Worsen Symptoms
++++++++++++++++++++++++++++
Is it o.k. to eat red grapes and red cabbages and plums?
Avoid porphyrinogenic foods such as red grapes, red cabbage, plums
and prunes.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++
Are soy containing products safe for acute porphyrics?
Soy is a food products that is found in many forms.
For most people soy does not cause any problems.
However acute porphyria patients as well as PCT patients, soy products should
be avoided.
Soy contains phytoestrogens.
SOURCE:
Sheryl Wilson [HCP] MSN R.D.
+++++++++++++++++++++
Can some foods stimulate heme synthesuis and induce porphyria attacks?
Certain vegatables such as cabbage and brussel sprouts may contain chemicals
that can stimulate heme synthesis.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
++++++++++++++++++++++
Graefruit in any forms should be avoided as it can induce the cytochrome P-450.
Grapefruit consumption can also alter the metabolism and absorption of various
drugs.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++
********SEE SEPARATE FILE “Nutrion: Grapefruit
++++++++++++++++
Should eating organ meats be avoided?
Particles of insecticides along with heavy metal toxins are stored in the liver.
Eating liver should be avoided especially from wild game and poultry.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++++++++
Internal organs of wild game such at the liver, should be avoided.
Heavy stores of metals along with traces of insecticides may be present.
SOURCE:
Valerie Christiansen RD
+++++++++++++++++++
++++++++++++++++++++++++
Do frozen seafood products contain substanvces that need to be avoided
by porphyria patients?
Many seafood items are packaged with sulphites to keep them fresh.
It is necessary to read all package labels.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++
Purple plums and prunes can stimulate heme synthesis and must be avoided by
porphyria patients.
SOURCE:
Jocelyn Barnes RD
Nutrition & Dietary Patient Educator
+++++++++++++++++++++++
Should eating organ meats be avoided?
Particles of insecticides along with heavy metal toxins are stored in the liver.
Eating liver should be avoided especially from wild game and poultry.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++++
Red grapes are known to stimulate heme synthesis which can trigger porphyria.
The eating of red grapes should be avoided.
SOURCE:
Francine Gibbons MSN
Nutrition and Dietary Patient Education
++++++++++++++++++++++++++++
Should there be iron intake for porphyria patients?
Iron intake should be adequate to avoid iron deficiency.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
+++++++++++++++++++++++
Should porphyria patients consume pytochemical supplements?
It is not necessary to be taking phytochemical supplements.
If you are eating a variety of foods that are rich in these substances you will
consume enough phytochemicals.
Phytochemical-rich foods like brightly colored fruits and vegetables, and whole
grains and legumes contain much more than phytochemicals.
These foods are healthful for a variety of reasons, like being naturally low in fat
and rich in vitamins and minerals.
SOURCE:
Phytochemicals
L.B. Bobroff et. al.
Institute of Food and Agricultural Sciences
University of Florida
1998
+++++++++++++++++++++++
What foods stimulate heme synthesis and trigger acute attacks?
Avoid foods such as red grapes, cabbage, brussel sprouts, red plums, all which
may contain chemicals that in large amounts can stimulate heme and porphyrin
synthesis.
.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++
Which foods are neurotoxins which can make the PN worse?
"Try to avoid foods or drinks high in caffeine, including coffee, chocolate and
sodas.
Caffeine can make neuropathy and pain worse."
SOURCE:
Mayo Health News
Mayo Clinic
Rochester, Minnesota
+++++++++++++++++++++++++++++++
What affect do Nutrients have in regard to porphyria peripheral neuropathy?
While nutrients and vitamin supplements haven't been studied the way other
drugs have been as treatments for peripheral neuropathy, there are some things
you can do yourselfthat may help with symptoms.
Many of the B vitamins have been found to help withsymptoms of peripheral
neuropathy, although high levels of B6 may worsen symptoms.
The ones that are helpful are B1, B12 and folate.
Try taking a multivitamin with vitamin Ein it, and a B complex to help with
symptoms of peripheral neuropathy. Make sure the Bcomplex, sometimes called
a vitamin B stress complex, has folate in it. Vitamin B12 canalso be given by
shots into a muscle every coupleof weeks, and this probably gets morevitamin
B12 into the body than vitamin pills.
Other supplementation that has shown some effect on neuropathy include
magnesium.
SOURCE:
Mount Sinai Neuro Research Program
+++++++++++++++++++++++++++
Hepatic porphyria patients should avoid the following at all times.
Always restrict fat and proteins during active porphyria.
Avoid chamomile containg foods.
* Acute porphyria patients known to have
sensitive reactions to chamomile.
Avoid Balsam containing foods.
Citrus fruits
Cola
Chocolate
Spices including Vanilla
Tomatoes
Avoid Soy containing foods.
* Soy is a natural estrogen.
SOURCE:
Sheryl Wilson MNS RD
+++++++++++++++++++++++++++
What are porphyrinogenic foods?
Porphyrenogenic foods contain chemicals that stimulate heme synthesis.
Cabbage
Brussel sprouts
Red plums
Red grapes
Red/purple grape juice
Purple prunes
SOURCE:
Sheryl Wilson MNS RD
+++++++++++++++++++++++++
Can ragweed affect porphyria patients?
Some people allergic to ragweed feel symptoms spike suddenly after eating
honeydew melon, cantaloupe or bananas.
Symptoms can also spike after drinking chamomile tea, using soap or face
powder containing chamomile, or taking herbal supplements that include it.
SOURCE:
AETNA IntellaHealth
September 5, 2002
Common Foods Worsen Symptoms
++++++++++++++++++++++++
Are some foods to be avoided by porphyria patients with cutaneous symptoms?
If you have a skin allergy known as contact dermatitis, you might want to think
twice before you eat that tomato.
Tomatoes and other foods, as well as certain spices, are related to balsam,
which exacerbates contact dermatitis.
These same foods can be considered irritants and exacerbate other cutaneous
medical conditions including the porphyrias. .
Avoiding balsam-related foods can help improve skins conditions which
produce redness and itching.
People who gave up foods such as citrus fruits, cola, chocolate, and spices
such as vanilla, as well as tomatoes, reported a complete or significant
improvement of their symptoms.
SOURCE:
Journal of the American Academy of Dermatology
2001
"Balsams and Food That Aggravate Skin"
John F. Fowler, MD et. al.
+++++++++++++++++++++++++++
Should porphyria patients limit their intake of tomatoes?
One should also remember that tomatoes contain a high amount of sulphur, and
thatsulphur 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.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++
Should organic produce still be washed thoroughly before use?
Limit your exposure to chemical toxins.
Buy organic foods, stay away from prepared foods clean fruits
and vegetables thoroughly with asolution of food- grade hydrogen peroxide, limit
use of chemicalsin your home and garden.
SOURCE:
Chemical Overload
A Little Bit Won't Hurt You - Or Will It?
Dr. Regan Golob DC
+++++++++++++++++++++++++++
What effect does broccoli have as far as P-450 action is concerned?
Broccoli is a known inducers of P-450.
Broccoli also has a large Vitamin K content and reduces the prothrombin
time in blood coagulation.
SOURCE:
P-450 Drugs - Inhibtors
Dr. David FLockhardt
School, of Pharmacology
Univeristy of Indiana Medical School
++++++++++++++++++++++++++++
Does consumption of brussel sprouts affect blood coagulation and cause
clotting in porphyria patients?
Brussel sprouts also have a large Vitamin K content and reduces the
prothrombin time in blood coagulation (clotting factor).
Brussel sprouts also are inducers of the P-450 and help in triggering acute
attacks.
SOURCE:
Sheruyl WIlson MNS RD
+++++++++++++++++++++++++++++
What effect do brussel sprouts have as far as P-450 action is concerned?
Brussels sprouts are a known inducers of P-450.
SOURCE:
P-450 Drugs - Inhibtors
Dr. David FLockhardt
School, of Pharmacology
Univeristy of Indiana Medical School
+++++++++++++++++++++++++++++
Can certain foods be associated with migraine headaches?
Foods that have been associated with migraine in some peopleinclude foods
containing the amino acid tyramine (red wine, aged cheese, smoked fish,
chicken livers, figs, some beans), chocolates, nuts, peanut butter, fruits
(avocado, banana, citrus fruit), onions, dairy products, baked goods, meats
containing nitrates (bacon, hot dogs, salami, cured meats), foods containing
monosodium glutamate (an additive in many foods), and any processed,
fermented, pickled or marinated foods.
SOURCE:
Elaine T. Kiriakopoulos, M.D., M.Sc.,
Department of Neurology
Beth Israel Deaconess Medical Center
Harvard University
Boston, MA.
+++++++++++++++++++++++
How does caffeine effect porphyria patients?
Caffeine represents a safe and sensitive indicator of the activity of the
cytochrome P-448 system."
SOURCE:
Assessment of the cytochrome P-448 dependentliver enzyme system by a
caffeine breath test.
Wietholtz H et. al.
European Jouurnal of CLinical Pharmacology
1981;21(1):53-9
+++++++++++++++++++++++++++++
What effect does caffeine have on PN and pain?
Try to avoid foods or drinks high in caffeine, including coffee, chocolate and
sodas.
Caffeine can make neuropathy and pain worse.
SOURCE:
Mayo Health News
Mayo Clinic
Rochester, Minnesota
++++++++++++++++++++++++++++
Can nutrition cause Anxiety?
Nutritional cuases of Anxiety include deficiences in some B-complex vitamins,
calcium, magnesium, potassium, copper, iron, zinc, and essential fatty acids.
Also the excessive use of stimulants inlluding tea, coffee, colas, excess sodium,
overindulgence in alcohol, toxic overload and a generally poor intake of nutrients
usually due to a lack of interest in food.
SOURCE:
Vitamins and Minerals:
Healthy Diet & Safe Supplementation
Denise Mortimore
Element
2001
++++++++++++++++++++++++++++
Potatoes should be peeled before cooking.
In order to avoid insecticides in green vegetables and root vegetables, always
peel and rinse them properly.
SOURCE:
HEather Kieffer RD
Nutrition and DIetary Patient Educaot
+++++++++++++++++++++++++
Only fruits that are not sprayed should be eaten without peeling, peel all other
fruits.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++
Can nutrition cause depression?
Nutritional cuases of depression include deficiences in some B-complex
vitamins, calcium, magnesium, potassium, copper, iron, zinc, and essential fatty
acids.
Aso the excessive use of stimulants including tea, coffee, colas, excess sodium,
overindulgence in alcohol, toxic overload and a generally poor intake of nutrients
usually due to a lack of interest in food.
SOURCE:
Vitamins and Minerals:
Healthy Diet & Safe Supplementation
Denise Mortimore
Element
2001
+++++++++++++++++++++++++
Why should porphyria patients avoid eating liver?
Particles of insecticides along with heavy metal toxins are stored in theliver.
Eating liver should be avoided especially from wild game and poultry.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++++
The liver is a cleaning and dumping ground in the human body.
Heavy metal toxins along with particles of insecticides and other toxins are
stored in the liver.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++
Why should liver and organ products be avoided in the diet?
Heavy metal toxins are stored in the liver.
Eating liver should be avoided especially from wild game and poultry.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++
What foods should acute porphyria patients avoid due to porphyrinogenic
triggers?
Avoid foods such as red grapes, cabbage, and brussel sprouts.
Red plums,as well as prunes all which may contain chemicals that in large
amounts can stimulate heme and porphyrin synthesis.
.
SOURCE:
Dietary Concerns fopr Porphyria Patients
Learning Healthy Nutritional Styles
Brenda Wintherall MSN RD
+++++++++++++++++++++++++
Is it safe to use artificial sweetners in acute porphyria?
:
Artificial sweetners are acceptable in acute porphyria.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
++++++++++++++++++++++++++++++
Since acute porphyria is known to elevated cholesterol is it wise to ingest
fructose corn syrup?
And the fructose propels the liver into a fat-promoting mode by activating the
formation of enzymes that lead to elevated levels of "bad" cholesterol and
triglycerides.
SOURCE:
Corn syrup
Dr. William J. Whelan
Biochemistry
University of Miami
++++++++++++++++++++++
Is fructose (corn syrup) better than sucrose?
Eating fructose corn syrup results not only in lower insulin levels but also lower
leptin levels.
Because both hormones are involved in appetite control, eating lots of fructose
"could increase the likelihood of weight gain."
Such studies have prompted the American Diabetes Assn. to conclude that
"large amounts of fructose may increase blood fat levels" and that "there is no
reason to use large amounts of fructose" in place of sucrose.
SOURCE:
Corn Syrup
Patricia King
Los Angeles Times
3-24-2003
++++++++++++++++++++++
Beside the sulfite question in dextrose infusions, what have studies proven in
regard to corn syrup use (fructose)?
Animal studies and preliminary human studies have found that a high-fructose
diet leads to health problems including insulin resistance and elevated
triglyceride levels, a marker for heart disease.
Unlike glucose, fructose is almost entirely metabolized in the liver.
When fructose reaches the liver,the liver goes bananas and stops everything
else to metabolize the fructose.
SOURCE:
Corn syrup
Dr. William J. Whelan
Biochemistry
University of Miami
+++++++++++++++++++++++++++++
Fructose corn syrup is often used in soda pop and sports drinks and should be
avoided by porphyria patients.
Ingestion of high fructose corn syrup causes a close down of normal metabolism
function which needs to be avoided by acute porphyria patients.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++++++
Can a porphyria patient safely use artificial sweetners?
Artificial sweeteners are acceptable.
Remember that some may cause an increase in diarrhea during an acute attack.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++
Why is soda pop considered empty calories?
The sugar in orange soda pop is void of vitamins and minerals; that's why it's
described as "empty calories".
SOURCE:
Carbohydrate Confusion
Nancy Clark, MS, RD
Director of Nutrition Services
SportsMedicine Associates
Brookline MA. April 2002
++++++++++++++++++++++++++++
Can iron deficiency trigger porphyria?
Iron is a component of heme.
Iron deficiency can compromise heme synthesis and excerbate porphyria.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
++++++++++++++++++++++++++++
Is salt safe to use in porphyria?
Salt intake need not be restricted unless it is important for controlling
hypertension.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
++++++++++++++++++++++++++++
Do porphyria patients have to restrict their salt intake?
Salt intake need not be restricted unless it is important for controlling
hypertension.
The majority of porphyria patients do not have chronic hypertension.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++
How can one control edema during acute attacks?
Remember that during acute attacks often some of the glucose IV will contain
saline solution, while other may be only water.
Poprhyria patients who are troubled with edema need to restrict their saline
and should use the water based glucose infusions.
SOURCE:
Barbara Mattock RD
+++++++++++++++++++++++++
This is important to remember if you have problems with edema.
Then you should use the water based glucose only.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++
What does anemia mean?
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.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++
Most acute hepatic porphyria patients experience hypertension during acute
attacks and some will find that hypertension remains into remission.
For nutritionally controlling hypertension the following may be used in "cutting
back" and reducing dietary omduicers of hypertension.
Avoid:
Added salt
Alcoholic beverages
Caffeine containing foods
Cappucino
Chocolate
Coffee (with caffeine)
Soda pop (with caffeine)
Tea (wiff caffeine)
Foods containing high amount of bicarbonate sodium.
SOURCE:
Sheryl Wilson MNS RD
++++++++++++++++++++++++++++++++
Can chamomile cause allergic reactions?
Chamomile causes the most severe reactions, occasionally including
anaphylactic shock, a severe, often fatal systemic immune reaction.
"The chamomile-ragweed association has been known for a long time, because
in the plant kingdom they're in the same family,"
SOURCE:
AETNA IntellaHealth
September 5, 2002
Common Foods Worsen Symptoms
+++++++++++++++++++++++++++++
Is the consumption of soy products good for reducing high cholesterol?
Soy compounds called isoflavones act like human hormones that regulate
cholesterol levels.
However soy is a natural estrogen and therefore considered a trigger of
heme synthesis.
Porphyria patients must look to other alternatives for reducing cholesterol levels.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++
Should porphyria patients avoid soy?
Eating soy proteins can reduce your levels of total cholesterol, low-density
lipoprotein (LDL) cholesterol and triglycerides.
Eating soy may also raise your level of high-density lipoprotein (HDL)
cholesterol, the "good" cholesterol, which may protect you against heart
disease.
While soy products help lower cholesterol levels, soy products are natural
estrogen containing products and must be avoided by porphyria patients.
SOURCE:
Kaaren DeLipio MNS RD
Clinical Nutritionist
+++++++++++++++++++++++++
For normally healthy people the use of soy is advisable.
However, Estrogen must be avoided by most porphyria patients.
Because of this, be careful with soy products unless weighing the risks of high
cholesterol to the risks of triggering attacks.
Remember that high cholesterol is a secondary condition of acute porphyria.
SOURCE:
Sheryl Wilson [HCP] RD
++++++++++++++++++++
Should eating white bread be avoided for good nutrition?
White bread's reputation for being "poison" is partially because of its high
glycemic effect.
That is, 200 calories of white bread quickly digests and causes the blood
glucose (blood sugar) to elevate higher than would the same amount
of a whole grain, fiber-rich bread.
SOURCE:
Carbohydrate Confusion
Nancy Clark, MS, RD
Director of Nutrition Services
SportsMedicine Associates
Brookline MA. April 2002
+++++++++++++++++++++++++
In choosing breads to help with high carbohydrate intake is one bread better
than another?
Whole grain breads are a far better choice than white bread or highly refined
wheat bread.
Whole grains have a lower glycemic rating and are better nutritionally, and also
provide fiber.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++
What is the difference in whole grain and white bread?
The starch in whole wheat bread is accompanied by fiber and B-vitamins.
The starch in white breads has lost many health protective nutrients during the
refining process.
White bread provides muscle fuel, but fewer vitamins.
SOURCE:
Carbohydrate Confusion
Nancy Clark, MS, RD
Director of Nutrition Services
SportsMedicine Associates
Brookline MA. April 2002
+++++++++++++++++++++++++
Are dairy products good for porphyria patients?
For porphyria patients the use of dairy products is a good source of
carbohydrate.
For some people, there is an allergy to milk.
This condition requires restriction of all dairy products.
These individuals may have trouble obtaining enough calcium in their diet and
may need to take calcium supplements.
SOURCE:
Calcium
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
++++++++++++++++++++++++++
Can consuming charbroiled meats be problematic for porphyria patients?
The ingestion of charbroiled meats may induce isoenzymes, resulting in
increased clearance of drugs (i.e. theophylline).
SOURCE:
Journal of Clinical Investigation
1997 May 15;99(10):2545-2553
+++++++++++++++++++++++++
Does eating char-broiled foods cause problems for porphyrics?
Car-broiled foods are inducersof the cytochrome P-450.
SOURCE:
P-450 Drugs - Inducers
Dr. David Flockhardt
School, of Pharmacology
Univeristy of Indiana Medical School
+++++++++++++++++++++++++++++
How do porphyria patients get exposed to dioxins right in our very own
kitchens?
Dioxins cause Carcinogens.
Carcinogens cause cancer. (This is especially true of breast cancer.)
Here are some simple things to think about and avoid:
Don't freeze your plastic water bottles with water as this also releases dioxins in
the plastic.
This doctor is the manager of the Wellness Program at the hospital.
He was talking about dioxins and how bad they are for us.
Moreover we should not be heating our food in the microwave using plastic
containers.
This applies to foods that contain fat.
The combination of fat, high heat and plastics releases dioxins into the food and
ultimately into the cells of the body.
Dioxins are known to trigger porph.
Everytime you exercise, get an elevation in body temperature or diet to loose
weight....you are releasing those toxins from the fat and into the blood
stream.....and in fact exposing yourself to triggering another porph attack.
Dioxins are carcinogens and highly toxic to the cells of our bodies.
Instead, he recommends using glass, Corning Ware, or ceramic containers for
heating food.
You get the same results without the dioxins.
So such things as TV dinners, weight watchers dinners, lean cuisine dinners,
instant ramen cup of noodles, and soups,etc., should be removed from the
container and heated in something else.
Paper isn't bad but you don't know what is in the paper.
Just safer to use tempered glass, Corning Ware, etc.,
He said we might remember when some of the fast food restaurants moved away
from the foam containers to paper.
The dioxin problem is one of the reasons.
To add to this: saran wrap placed over foods as they are nuked, with the high
heat, actually drips poisonous toxins into the food, use paper towels.
Avoid any chance of dioxins.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++
Is it possible to avoid pesticides in food?
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.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++
Is use of alcohol contraindicated for porphyrics?
Medical drugs books list alcohol as being contraindicated for porphyrics.
Alcohol has other harmful effects and can lead to weight gain.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++
All alcoholic beverages should be avoided by porphyric patients.
Alcohol stimulates the heme biosynthetic pathway in the liver and can itself
exacerbate porphyria
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++
Can certain foods cause Migraines
Some nutritional cuases of Migraines or Headaches include food intolerance
such as the "nightshade" foods which include tomatoes, potatoes, bell peppers,
and eggplant.
SOURCE:
Vitamins and Minerals:
Healthy Diet & Safe Supplementation
Denise Mortimore
Element
2001
++++++++++++++++++++++++
PORPHYRIA FACTS: NUTRITION - NECESSARY SUPPLEMENTS
Nutrients and supplements thereof are essential in nutrition for porphyria
patients.
Regular laboratory testing should be undertaken to determine any deficiencies
before supplementation is begun. Then further testing should be run periodically
to monitor the levels of various nutrients.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++
Because I take a diuretic do I need to take magnesium supplements?
If you take diuretics, they will tend to deplete the body's natural magnesium
levels and you will need supplementation of magnesium.
SOURCE:
J ean Carper
Might Magnesium
Eat Smart
USA Weekend
(4) 8-30-2002
+++++++++++++++++++++++++++++++
Does magnesium have a role in supplying needed energy to the body?
Magnesium plays a role in the production and transport of energy within the
human body.
SOURCE:
Donna R. Cooper, M.D. M.P.H.
Department of Medicine
Massachusetts General Hospital
Boston, MA.
++++++++++++++++++++++++++++++
For porphyria patients who are prescribed dieuretics there can be risk of the
depletion of magnesium in the body.
Magneisum levels should be check in a simple blood serum test, and
supplmentation prescribed if necessary to maintain normal levels.
SOURCE:
Robert Johnson M.D.
+++++++++++++++++++++++++++++++
Should one take magnesium in addition to calcium?
If you take high doses of calcium you need to take magnesium supplements
because calcium depletes magnesium in the boidy."
SOURCE:
Jean Carper
Might Magnesium
Eat Smart
USA Weekend
(4) 8-30-2002
++++++++++++++++++++++++++++
What are the signs of severe magnesium deficiency?
If magnesium deficiency is severe, there can be delirium,numbness, tingling,
muscle cramping, and hallucinations.
SOURCE:
Donna R. Cooper, M.D. M.P.H.
Department of Medicine
Massachusetts General Hospital
Boston, MA.
+++++++++++++++++++++++++++
Can there be mineral loss in CEP?
Bone demineralization in CEP can be substantial.
SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++++++++++++++++
Should magnesium be taken along with calcium and vitamin D?
When using calcium supplements especially for safeguarding bone density
it is best to take magnesium unit for unit along with the calcium and 400 UIs
of Vitamin D since both the magnesium and D help the calcium have proper
absorption and metabolism.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++
What is the role of magnesium in porphyria and metabolism?
Magnesium is an essential cofactor for many enzymatic reactions, especially
those involved in energy metabolism such as the porphyrias.
SOURCE:
"Brain and CSF magnesium concentrations during magnesium deficit in animals
and humans: neurological symptoms."
Morris, M. E.
Magnesium Research
5(4): 303-13.1992.
++++++++++++++++++++++++++++
Is it common for acute porphyria patients to experience low magnesium levels?
Hypomagnesia has also been reported and may be severe enough to cause
tetany.
Porphyria patients with low magnesium levels may experience spasm, muscle
twitchings, cramps, and convulsions.
SOURCE:
Dr. Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++
What does magnesium do for muscles?
Magnesium is important for the contraction and relaxation of muscles.
SOURCE:
Donna R. Cooper, M.D. M.P.H.
Department of Medicine
Massachusetts General Hospital
Boston, MA.
+++++++++++++++++++++++++++
Does calcium play a role in enzyme reactions?
A function of calcium is the stimulation of hormone secretion, and the activation
of enzyme reactions.
SOURCE:
Calcium
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++++++++++++++++++++
What should calcium intake be in women with acute porphyria?
Calcium intake in women should be at least one gram daily.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++
How much calcium should be consumed daily?
Calcium intake in women should be at least one gram daily.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
+++++++++++++++++++++++.
How can one prevent bone loss?
Research studies have found that calcium supplements, if taken regularly, help
prevent osteoporosis by reducing bone loss.
Bread products and orange juice, are enriched with calcium to make them a
significant source of calcium for people whose dairy product consumption is
inadequate.
SOURCE:
Calcium
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++++++++++++++++++++
What is the role of potassium in electrolytes?
Potassium works along with sodium and chloride as an electrolyte.
Electrolytes are crucial to the balance of the body fluids and the control of the
acid/alkaline levels.
SOURCE:
Vitamins and Minerals: A Practical Approach
Denise Mortimore
2001 Harper Collins Publishers
++++++++++++++++++++++++++++++
Does below normal range blood serum potassium have specific consequences
in acute porphyria patients?
Below normal range potassium most often leads to mental change and confusion
in porphyria patients.
The low potassium is usually the result of naseau or diarrhea during the aute
attack.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++
What are the first signs of potassium deficiency?
The first two signs of Potassium deificiency are fatigue and muscle weakness.
Soon thereafter mental change often becomes apparent.
SOURCE:
Vitamins and Minerals: A Practical Approach
Denise Mortimore
2001 Harper Collins Publishers
+++++++++++++++++++++++++++
What are the main symptoms of Potassium deficiency?
The main symptoms of Potassium deficiency include muscle weakness, fatigue,
cardiac arrythmias, (tachycardia) mental depression, mental confusion, water
renetion, and low blood pressure.
SOURCE:
Vitamins and Minerals: A Practical Approach
Denise Mortimore
2001 Harper Collins Publishers
++++++++++++++++++++++++++
Does Potassium help with high blood pressure?
Many studies have demonstrated that highblood pressure can successfully be
treated by increasing the intake od dietary potassium.
Potassium supplementation may be especially useful in the treatment of high
blood pressure in people over 65 years of age.
Studies have shown that potassium supplementation produces a significant
reduction ui blood presure.
SOURCE:
Vitamins and Minerals: A Practical Approach
Denise Mortimore
2001 Harper Collins Publishers
++++++++++++++++++++++++++++
Can fatigue and weakness in acute porphyria be related to low potassium?
Deficiency in potassium produces faitgue and muscle weakness.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++++
How can one control fluid balance that causes fluid build-up in the body and
especilly cranial pressure?
Potassium is vital for fluid balance and the proper muscle contraction.
SOURCE:
Vitamins and Minerals: A Practical Approach
Denise Mortimore
2001 Harper Collins Publishers
++++++++++++++++++++++++++++
Is potassium an electrolyte?
Potassium is an electrolyte and needs to be checked regularly during acute
attacks and periods of nausea, vomiting and diarrhea and loss of body fluids.
SOURCE:
Robefrt Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++
Why do acute porphyria patients experience a lot of muscle contractions and
muscle weakness?
One of the reason for abnormal and painful muscle contrations in acute
porphyria patients is that potassium shortage reults in low leveles of stored
glycogen.
Exercising muscles use glycogen for energy.
SOURCE:
Vitamins and Minerals: A Practical Approach
Denise Mortimore
2001 Harper Collins Publishers
++++++++++++++++++++++++++++++++
Do PCT have deficient vitamin C levels?
Ascorbic acid (vitamin C) levels are deficient in some patients with PCT.
SOURCE:
Variable abnormalities in the porphyrias.
Hindmarsh JT.
Clinical Chimica Acta 217:29,
1993.
+++++++++++++++++++++++++++++++
PORPHYRIA FACTS: NUTRITION - CHOLESTEROL - FATS - PROTEIN
Since acute porphyria patients have elevated cholesterol levels associated with
their porphyria,what should be their cholesterol intake in dietary?
Cholesterol intake should be less than 300 milligrams per day.
Remember that in the acute porphyrias high cholesterol is commonly present.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++++
Are cholesterol levels increased in the hepatic porphyrias?
There is often hypercholesterolemia, hyperlipidemia, glucose interlerance and
increased CSF protein levels.
SOURCE:
Dr. Karl E. Anderson MD
The Porphyrias
Cecil Book of Medicine
++++++++++++++++++++++++++++++
Is high cholesterol normally associated with acute porphyrias?
Hpercholesterolemia is a airly frequent finding in patients with acute porphyria.
SOURCE:
The Porphyrias
Kappas, A. et. al.
The Metabolic Basis of Inherited Disease
7th Edition 1995
+++++++++++++++++++++++++++++
Cholesterol is usually elevated in blood serum studies of acute porphyria
patients.
Porphyr8ia patients should make every effort to reduce total cholesterol intake
levels.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++++
What is cholesterol?
A soft, waxy substance that is present in all parts of the body including the
nervous system, skin, muscle, liver, intestines, and heart.
Cholesterol is made by the body and obtained from animal products in the diet.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++++++++++++++++++
How much cholesterol intake should be part of a porphyric's daily intake?
Cholesterol intake should be less than 300 milligrams per day.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++
What percent of a porphyric's diet should consist of fat?
Total fat intake should be less than 30 percent of total calories.
Be sure to keep the saturated fats to a minimum.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++
Does the high carbohydrate intake and weight gain influence the cholesterol
level?
Being overweight promotes a high total cholesterol level.
Losing weight improves your cholesterol levels.
Set up an exercise program to lose weight using your doctor's advice.
SOURCE:
Mayo Medical Education
Mayo Clinic
Rochester, MN
++++++++++++++++++++++++++++++++
Is fat bad for porphyria?
If there is any kidney impairment protein must be reduced.
SOURCE:
Robert Johnson M.D.
Internal Medicie
++++++++++++++++++++++++++++++++
Total fat intake should be less than 30 percent of total calories.
Be sure to keep the saturated fats to a minimum.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++++++
How much protein does a porphyria patient need?
Porphyria patients will want to keep thei protein intake below the maximum
daily recommended amount of 30% of the total calories.
SOURCE:
Robert Johnson M.D.
Internl Medicine
+++++++++++++++++++++++++++++++
If a porphyria patients is having renal complications of porphyria should protein
intake be reduced?
If there is any kidney impairment protein must be reduced.
SOURCE:
Garrett Hackenberry PhD
Nutritional Sciences
++++++++++++++++++++++++++++++
Porphyria patients hould avoid all organ meats.
SOURCE:
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++++++
A porphyric will also want to avoid high intakes of protein.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++
What products contain cholesterol?
Cholesterol is found in eggs, dairy products, meat, poultry, fish, and shellfish.
Egg yolks and organ meats (liver, kidney, sweetbread, and brain) are high in
dietary cholesterol.
Fish generally contains less cholesterol than other meats, but some shellfish is
high in cholesterol content.
Foods of plant origin (vegetables, fruits, grains, cereals, nuts, andseeds) contain
no cholesterol.
Fat content is not a good measurement of cholesterol content.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++++++++++++++++++++
Is the presence of high cholesterol a common finding among acute porphyria
patients?
"Hyperchloresterolemia is a fairly frequent finding in persons with acute
porphyrias.
In the United States it has been found that elevations in lipoprotein
concentrations were reported.
In Finland there is a trend toward elevated high-density lipoprotein.
Differences may in part be dietary."
SOURCE:
New England Journal of Medicine
282:432 1970.
Dr. R.S. Lees et.al.
++++++++++++++++++++++++++
Does the intake of dietary fats increase the chances of being subject to an acute
attack.
Dietary fats may increase the excretion of porphyria precursors in AIP."
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
+++++++++++++++++++++++++++
How is the best way to lower cholesterol and fat intake?
Control total fat.
Limit all types of fat - saturated, polyunsaturated, trans fatty acids (trans fats)
and monounsaturated - to no more than 30 percent of your total daily calories.
Because all foods with fats contain a combination of these fats, it's important to
reduce total fat.
If your daily intake is 2,000 calories, 30 percent equals 65 grams of fat.
Limit saturated fat to no more than 10 percent of total calories.
Limit dietary cholesterol.
To accomplish this goal, limit or avoid concentrated sources such as organ
meats, egg yolks and whole-milk products.
Of course, for porphyria patients, it is best to avoid such products because often
these products contain triggering elements suchas steroids, sulphur, and
chemical toxins.
SOURCE:
Sheryl WIlson RD
+++++++++++++++++++++++++++++++++
In some forms of porphyria dietary fats ten to increase the amount of uinary
porphyrins.
SOURCE:
Gretch Vasaqyez, FNP
Endocrinology Division
+++++++++++++++++++++++++++
What should the fat and protein intake levels be for porphyria patients?
"Fats and proteins given to porphyria patients should not exceed basic RDA
requirements."
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
++++++++++++++++++++++++
Does consumption of almonds actually reduce cholesterol?
Eating one ounce of almonds per day reduces "bad" HDL cholesterol
by about 4 percent.
SOURCE:
Circulation Journal
2002
++++++++++++++++++++++++
How much protein is needed in a porphyria diet?
Protein intake should follow the RDA during remission.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++
Must protein be reduced with renal problems?
If their is any kidney impairment protein must be reduced.
Be sure to keep the saturated fats to a minimum.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++
How much fat should be in a porphyria diet?
Total fat intake should be less than 30 percent of total calories.
Be sure to keep the saturated fats to a minimum.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++++
Is it necessary to cut the fat and protein during acute attacks?
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.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++++++
Why is some protein in dieting necessary?
Protein tends to be more satiating than is carbohydrate.
That is, protein (and fat) lingers longer in the stomach than does carbohydrate.
Hence, having high protein (and fat) eggs & bacon for breakfast stays with you
longer than does a high carb bagel with jam.
By curbing hunger, you have fewer urges to eat and can more easily cut
calories--until you start to crave carbs and binge eat.
SOURCE:
Carbohydrate Confusion
Nancy Clark, MS, RD
Director of Nutrition Services
SportsMedicine Associates
Brookline MA. April 2002
++++++++++++++++++++++++++++
Is dieting bad for acute porphyria patients?
Attacks are often precipitated by low intake of carbohydrates and calories in an
attempt to lose weight.
Thus dietary counseling is very important.
Fats and proteins should follow the RDA recommendations.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
++++++++++++++++++++++++++
What amount of protein should there be in a porphyria diet?
Protein intake should follow the RDA during remission.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++
In dieting what makes the differences between proteins and carbohydrates?
The dieter loses water weight.
Carbs hold water in the muscles.
For each ounce of carbohydrate you stored as glycogen, your body
simultaneously stores three ounces of water.
When you deplete carbs during exercise, your body releases the water and you
experience a significant loss of weight that's mostly water, not fat.
SOURCE:
Carbohydrate Confusion
Nancy Clark, MS, RD
Director of Nutrition Services
SportsMedicine Associates
Brookline MA. April 2002
+++++++++++++++++++++++++++
PORPHYRIA FACTS: NUTRITION - ANTIOXIDANTS
What are antioxidants, what do they do, and are they safe?
Preservatives include antioxidants which prevent foods from becoming rancid,
and antimicrobials which protect foods against microrganisms.
Antioxidants work in foods containing fats and oils and prevent the oxidation
that produces rancidity that causes loss of flavor, color, nutrient value and
texture.
Antioxidants include BHA, BHT, TBHQ, PG, sulfites, tocopherols, ascorbic
acid/salts, erythorbic acid/salts, and substances with preservative properties
such as spices/herbs, essential oils, citric acid and phosphoric acid
SOURCE:
GA-O88 SHELF LIFE TECHNOLOGY FOR PROCESSED FOODS
Dorothy Kroll
July 1995
+++++++++++++++++++++++++++++
What are antioxidant vitamins and why are they important?
Our bodies are actually battlegrounds for infection and diseases.
Normal body functions, such as breathing or physical activity, and other
lifestyle habits, such as smoking, produce substances called free radicals that
attack healthy cells.
When these healthy cells are weakened, they are more susceptible to
cardiovascular disease and certain types of cancers.
Antioxidants, such as vitamins C and E, and carotenoids, which include
beta-carotene, lycopene, and lutein, help protect healthy cells from damage
caused by free radicals.
SOURCE:
Antioxidants for Optimum Health
American DIetetic Association
National Center for Nutrition & Dietetics
+++++++++++++++++++++++++++
What are antioxidant vitamins and why are they important?
Our bodies are actually battlegrounds for infection and diseases.
Normal body functions, such as breathing or physical activity, and other lifestyle
habits, such as smoking, produce substances called free radicals that attack
healthy cells.
When these healthy cells are weakened, they are more susceptible to
cardiovascular disease and certain types of cancers.
Antioxidants, such as vitamins C and E, and carotenoids, which include
beta-carotene, lycopene, and lutein, help protect healthy cells from damage
caused by free radicals.
SOURCE:
Antioxidants for Optimum Health
American DIetetic Association
National Center for Nutrition & Dietetics
++++++++++++++++++++++++++++++
PORPHYRIA FACTS: NUTRITION - FIBER
What should be known about fiber in the diet of porphyria patients?
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.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++++++
Is fiber necessary for porphyria patients?
A diet should be rich in fiber as constipation is a often a symptom of an
acute porphyric patient.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++++++
What are foods that are high in soluble fiber?
Foods high in soluble fiber are varied.
Such foods include rice bran, oat bran, oatmeal, beans, peas, barley, citrus
fruits, strawberries and apple pulp.
SOURCE:
Sheryl Wilson, RD
+++++++++++++++++++++++++++
How much fiber should porphyria patients have in their diets?
Fiber intake should be about 40 grams per day. High dietary fiber intakes
should beavoided 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.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++++
What will aid with the constipation experienced in porphyria?
Dietary fiber can sometimes be taken in addition to daily ingestion of fiber
containing foods.
SOURCE:
Robert Johnson M.D.
+++++++++++++++++++++++++++
How can one reduce the side effects of fiber intake?
Dietary fiber intake sometimes causes abdominal cramping,
diarrhea and flatulence.
These can be minimized by increasing fiber intake gradually.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++
What are bezoars associated with too much fiber in porphyria?
Bezoars, also known as trichobezoars or hairballs are common with the intake of
too much fiber.
A ball of swallowed fiber that collects in the stomach and fails to pass through
the intestines.
A barium swallow X-ray will show the mass in the stomach, sometimes a scope if
used (endoscopy) to directly visualize the bezoar.
The bezoar may need to be surgically removed (especially trichobezoars which
tend to be large).
Sometimes small bezoars can be removed through a scope placed through the
mouth and into the stomach.
Persistent vomiting that can be assoiated,can lead to dehydration.
SOURCE:
Jonathan Fanaroff, M.D.
Department of Pediatrics
Rainbow Babies and Children's Hospital
Case Western Reserve University
Cleveland, OH.
++++++++++++++++++++++++++
Is folate supplementation of any benefit?
"Folate supplementation may prove beneficial as an adjuvant therapy for a
porphyric crisis or during prodromal symptoms."
SOURCE:
Neurology
July 1993
F.J. DiMArio Jr., M.D., et.
+++++++++++++++++++++++++++++++
What types of fiber should porphyria patient eat?
Eat foods with soluble fiber.
As part of a low-fat diet, soluble fiber can help lower your total blood cholesterol
level.
SOURCE:
Mayo Medical Education
Mayo Clinic
Rochester, MN
++++++++++++++++++++++++++
Potassium levels must be maintained during acute attacks as loss of ptassium
is associated with mental changes that often occur.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++++
What does potassium have to do with carbohydrate conversion in acute
porphyria patients?
Potassium plays a vital role in the regulation and functioning of the heartbeat,
protein synthesis, nucleic acid synthesis nerve conduction, energy production,
muscle contraction and the conversion of blood sugar into glycogen.
SOURCE:
Vitamins and Minerals: A Practical Approach
Denise Mortimore
2001 Harper Collins Publishers
+++++++++++++++++++++++++
Is there any type of fish that is high in Potassium?
A salmon steak is high in potassium and has a ratio of about 4.1 potassium to
sodium.
SOURCE:
Vitamins and Minerals: A Practical Approach
Denise Mortimore
2001 Harper Collins Publishers
++++++++++++++++++++++++++++
What foods are high in Potassium?
Foods high in potassium include potatoes, avacados, and bananas.
SOURCE:
Vitamins and Minerals:
Healthy Diet & Safe Supplementation
Denise Mortimore
Element
2001
+++++++++++++++++++++++++++++
Is it possible to avoid the use of prescription drugs in treating fatigue during
acute attacks?
Extra magnesium may help with fatigue.
SOURCE:
Dr. Mildred Seeling
University of North Carolina
Might Magnesium
Eat Smart
USA Weekend
(4) 8-30-2002
++++++++++++++++++++++++++++
Does nutrition have anything to do with having insomnia?
Nutrient deficiences, especially of the B Vitamins and Magnesium can produce
insomnia.
SOURCE:
Vitamins and Minerals:
Healthy Diet & Safe Supplementation
Denise Mortimore
Element
2001
++++++++++++++++++++++++++
What does Folic Acid do?
Folic acid works with vitamins B-6 and B-12 to lower blood levels of
homocysteine.
Homocysteine is an amino acid that builds and maintains tissues.
SOURCE:
Sheryl Wilson RD
+++++++++++++++++++++++++++++
Can porphyria patients safely consume alcohol?
All alcoholic beverages should be avoided by porphyric patients.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++
How is Folate acquired in the body?
Folate occurs naturally in certain foods such as:
Citrus fruits and juices , Liver, Spinach and other dark leafy vegetables, Wheat
bran and other whole grains .
SOURCE:
Sheryl Wilson RD
+++++++++++++++++++++++
What is the normal recommended allowance of Folic Acid?
The Recommended Dietary Allowance (RDA) of Folic Acid for Adults age 19 or
older is 400 mcg/day. (mcg = micrograms)
The maximum daily intake (from supplements and fortified foods) unlikely to
pose risk of side effects for adults: 1,000 mcg/day
SOURCE:
Folic acid/folate (vitamin B-9)
Your Guide to Vitamin and Mineral Supplements
++++++++++++++++++++++++++
Is it necessary to take Folic Acid when Folate is found naturally in foods?
Often Folate is lost in food.
Storage, cooking or processing at high temperatures can destroy most of the
natural folate in foods.
SOURCE:
Sheryl Wilson RD
+++++++++++++++++++++++++++
What are the sources of Folic Acid in foods?
Food sources of Folic Acid include: Citrus juices and fruits, beans, nuts, seeds,
liver, dark green leafy vegetables and fortified grain products, such as bread,
pasta, breakfast cereals, rice.
SOURCE:
Folic acid/folate (vitamin B-9)
Your Guide to Vitamin and Mineral Supplements
+++++++++++++++++++++++++++
Does the use of Folic Acid supplements have side effects?
People who take folic acid may develop bright-yellow urine, fever, shortness of
breath, a skin rash or, very rarely, diarrhea.
Doses over 1,500 mcg/day can cause nausea, appetite loss, flatulence and
abdominal distention.
SOURCE:
Folic acid/folate (vitamin B-9)
Your Guide to Vitamin and Mineral Supplements
++++++++++++++++++++++++++++++
Which is better, Folate or Folic Acid?
The body uses synthetic folic acid more easily than it uses natural folate.
The body absorbs almost 100 percent of the synthetic folic acid compared with
about 50 percent of the natural folate.
SOURCE:
Sheryl Wilson RD
+++++++++++++++++++++++++++
Is there any validity to the use of Folic Acid in the treatment of acute porphyrias?
"Folic acid treatment is not thought to be useful in the intervention or
prevention of the acute porphyrias.
The suggestion that an unknown pteridine is a cofactor for porphobilinogen
deaminase substantiated by other researchers.
The rationale for this treatment therefore is not thought to be valid.
The reported improvement in patients was observed when folic acid was
combined with other vitamins and glucose, and glucose is regarded as an
effective treatment. I am not aware that anyone is doing research on folic acid in
porphyria, because folic acid is not thought to be required by any enzymes in
the heme biosynthetic pathway.
Folic acid and other vitamins might be beneficial if a porphyria patient has a
vitamin deficiency, due to a deficient diet, and treatment might then leadto
improvement in those symptoms due to the vitamin deficiency."
SOURCE:
Dr. Karl E. Anderson
Porphyria Specialisyt / Researcher
University of Texas Medical School
Galveston, Texas
+++++++++++++++++++++++++++
Can nutrition cause depression?
Nutritional cuases of depressioninclude deficiences in some B-complex
vitamins, calcium, magnesium, potassium,copper, iron, zinc, and essential fatty
acids.
Also the excessive use of stimulants including tea, coffee, colas, excess sodium,
overindulgence in alcohol, toxic overload and a generally poor intake of nutrients
usually due to a lack of interest in food.
SOURCE:
Vitamins and Minerals:
Healthy Diet & Safe Supplementation
Denise Mortimore
Element
2001
+++++++++++++++++++++++++++
Perepheral Neuropathy dietary needs are another list of items to remember.
While there is no one set given list of foods that are known to imporve PN, there
are a few to avoid.
(1) Alcoholic beverages
(2) Caffeine
*There is medical evidence that caffeine contributes
to neuropathy as well as to the pain in PN.
Coffee with caffeine
Cappucino
Tea with caffeine
Chocolate
Cocoa
Soda pop with caffeine
(3) Certain vitamins
Vitamin A
Vitamin D
Vitamin B-6 - (Pyridoxine)
Beneficial in the reduction of back pain i PN is added magnesium.
SOURCE:
Sheryl Wilson MNS, RD
++++++++++++++++++++++++++
What is the smart thing to remember in eating as a porphyria patient?
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
inexcess.
Food intake should be consistent, but should take into account lifestyle and
physical activity.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++++
USDA Food Value Data Base
Search online for values in the USDA National Nutrient Database for Standard
Reference, Release 15 Download ... Food Composition Products
www.nal.usda.gov/fnic/foodcomp/
============================================
www.nal.usda.gov/fnic/cgi-bin/nut_search.pl
US Dept. of Agriculture (USDA) - Nutrient Database for Standard Reference
Description: Interface allows simple searches for food ingredients by entering
keywords or the Nutrient Database Number.
|
