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PORPHYRIA FACTS: NUTRITION DEFICIENCES & IV FEEDINGS
The porphyrias can cause a lot of deficiencies due to problems of absorption
and metabolism, and loss of nutrients due to vomiting and diarrhea which are
often associated with the acute attack.
It is important that all porphyria patients have regular laboratory testing to check
for deficiences. Electrolytes are especially important to monitor.
SOURCE:
Robert Johnson M.D.
Internal Medicine
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Parenteral nutrition
A method of delivering nutrition or other substances directly into a vein. Fluids
given usually include salt (saline), glucose, amino acids, electrolytes, vitamins
and medications.
In severe acute attacks of hepatic porphyria parenteral nutrition is used when
vomiting or inability to consume carbohydrates takes place.
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Vitamin deficiency
Can lack of vitamins cause PN?
Nerve damage with or without porphyria can happen with certain vitamin
deficiences.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++
Certain vitamin deficiencies can damage peripheral nerves.
Treating the underlying condition can relieve some cases of peripheral
neuropathy.
SOURCE:
Sheryl Wilson, MSN RD (HCP)
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Intake of glucose drinks including HyCal which is manufacturered by Beecham,
goes a long way toward meeting the minimum daily carbohydrate intake of 350
grams.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
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How does a porphyria patient know how much nutritional value is in a bag of
dextrose?
5% DW at 150 ml per hour = 7.5 grams of glucose per hour
5% DW at 150 ml per hour = 56 calories per hour
For a 24 hour period ( 4 -1,000 bags back-to-back) = 180 grams of glucose.
For a 24 hour period (4 - 1,000 bags back-to-back = 1344 calories
*To bring AIP into remission a total of 400 grams of carbohydrate is needed.
An additional 220 grams of carbohydrate must be consumed.
If DW 10 is being used one would realize 15 grams per hour. which would = 360
grams by infusion with 40 remaining to be consumed.
Formula:
7.5 grams of carbohydrate = 56 calories
SOURCE:
"Me and AIP"
Porphyria Educational Services
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What is parental feeding?
If a patients is unable to consume orally an nutrients the patients may undergo
parental feeding.
This is a total intravenous feeding which is important to administer glucose and
other nutrients
Such a feeding is used if an acute attack is severe or prolonged, sufficient
glucose can be given by vein to meet the total energy requirements of a patient.
This is best accomplished by a catheter that is inserted into a large central vein.
Additional nutrients, including vitamins, minerals, amino acids and fat can be
given in the required amounts to maintain all requirements. Provision of total
nutritional needs in this manner by vein is commonly called "total parenteral
nutrition".
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
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Can parental feeding be added to the iv glucose infusions during acute attacks?
Corrective nutrition may be added to or 'piggy-backed" during intravenous
administration."
SOURCE:
Disorders of Porphyrins or Metals
Dwight Montgomery Bissell M.D.
# 203
pp1182-7
Cecil Textbook of Medicine
Volume 1 18th Edition
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Is it necessary to eat while having infusions during an acute attack?
Adequate calories are necessary and parenteral nutrition with carbohydrates
may be necessary in adition to glucose or heme infusions.
SOURCE:
Photodermatology, Photoimmunology and Photomedicine
1998 Apr;14(2):48-51
Management of the acute porphyrias.
Kauppinen R.
Department of Medicine,
University Hospital of Helsinki,
Finland.
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When are parental feedings used in acute attacks?
Parental 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
++++++++++++++++++++++++++++++
Are parental feedings used in acute porphyria treatment?
If a patients is unable to consume orally an nutrients the patients may undergo
parental feeding.
This is a total intravenous feeding which is important to administer glucose and
other nutrients
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++++
How much liquid should one consume daily?
It is recommended to consume 6-8 (8oz) glasses of water along with other
beverages high in glucose per day.
(Gatorade, rice water, Pedialite and sport drinks are excellent sources of
beverages high in glucose)
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++
Can a person suffer from dehydration during an acute attack?
Dehydration may occur, owing to persistent vomiting.
SOURCE:
Dr. Michael R. Moore
Porphyria Research Unit
University of Queensland
Department of Medicine
Brisbane, Australia 1999
++++++++++++++++++++++++
Does fluid intake need to be checked in porphyria patients?
Management of fluid and electrolyte imbalances, particularly hyponatremia and
hypomagnesemia, is critical during attacks."
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
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Must water be consumed daily by porphyria patients?
Six (8oz) glasses of water should be consumed by porphyria patients daily.
The water is in addition to other beverages high in glucose per day.
SOURCE:
Robert JOhnson M.D.
Internal Medicine
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During acute attacks is it necessary to continue eating?
Steps should be taken to ensure the patient has adequate nutrition for acute
porphyria patients.
SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
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Should food intake be scaterred out throughout the day or eaten at only three
main times?
Daily caloric energy intake in porphyric patients should be distributed
consistently throughout the day, with the highest amount of carbohydrate intake
being consumed upon waking after a period of fast [overnight sleep].
It is important to eat every 2-3 hours throughout the waking portion of one's day.
It is also important to consume high carbohydrate upon retiring in order to
reduce the body fasting during the sleep.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
.++++++++++++++++++++++++
Having a carbohydrate snack in the middle of the night keeps the carbohydrate
level in the blood from droppins and triggering overproduction of porphyrins
which cause acute attacks.
Also a middle of the night snack will keep blood sugar levels from fallling and
causing hypoglycemic reactions.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++
Is it important to eat several small meals around the clock to prevent attacks?
Nutrition is important in preventingacute attacks.
A porphyria patient should always ingest carbohydrate daily and such
carbohydrate intake should be appropriately disturbed in 2-3 hour intrevals
during the waking hours of the day.
SOURCE:
Robert TL, Varella L, Meguid MM.
Nutrition management of acute intermittent porphyria.
Nutrition 1994; 10: 551-55.
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Do it make a difference what types of carbohydrates that a porphyria patient
eats?
It is not only the amount of carbohydrates you eat, it's also the type that's
important
Not all carbohydrates are the same.
SOURCE:
Weighs and Means Controversy Surrounds Role of Carbs in Weight-Loss Diets
Alexa Pozniak
Too Many Carbs?
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Must a patient keep a high level of caloric intake?
Adequate calories are necessary and parenteral nutrition with carbohydrates
may be necessary.
SOURCE:
Management of the acute porphyrias.
Kauppinen R.
Department of Medicine
University Hospital of Helsinki, Finland.
Photodermatology Photoimmunology & Photomedicine
1998 Apr;14(2):48-51
+++++++++++++++++++++++++++
How many grams of carbohydrate does one need when in an acute attack?
During an acute attack your must have an intake of a minimum of 400 - 500
grams of carbohydrate.
This is includes intravenous glucose as well as consumed carbohydrate.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++++
To maintain adequate carbohydrate intake while restricting fluid intake, it may be
necessary to use higher concentrations of glucose, administered via a central
venous line.
SOURCE:
Dr. Michael R. Moore
Porphyria Research Unit
University of Queensland
Department of Medicine
Brisbane, Australia 1999
+++++++++++++++++++++++++++
How should one eat during bad attacks?
Nutritional management of acute attacks of porphyria through Intravenous
administration ofglucose (a pure form of carbohydrate) is part of the standard
treatment of acute attacks of porphyria. Glucose is given by vein because the
stomach and intestine usually do not function properly during an attack, and
material taken by mouth is not properly propelledthrough these organs.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++++++++++++
What percentage of one's food intake should be carbohydrate during times of
remission?
:
Carbohydrate intake during period of remission should be 55 to 60 percent of
total energy intake.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++++
Is good nutrition essential for porphyria patients?
Attacks of acute intermittent porphyria can be prevented by maintaining good
nutrition.
SOURCE:
Merck Manual
1996
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What is Ricelyte?
Ricelyte is the brand name of a prescription drugs used for treating acute
porphyria patients who have been unable to consume enough energy due to
vomiting.
SOURCE:
Dr. Robert Johnson M.D.
Retired Clinician
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