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PORPHYRIA FACTS:
TREATMENT OVERVIEW
It has been stated that about 9-10% of the world’s population are carriers of
porphyria.
It has also been stated that only 1 % of the population are active porphyrics,
having any exacerbation of their disease.
The mostr severe forms of the porphyrias are those of the acute hepatic forms
which have a variety of symptoms manifesting from time to time.
It is for those suffering the acute forms that the following is directed.
Timeliness of treatments
"The porphyrias are uncommon disorders of haem biosynthesis and their
effective management requires prompt and accurate diagnosis."
SOURCE:
Front line tests for the investigation of suspected porphyria.
A C Deacon, G H Elder.
Journal of Clinical Pathology.
July 2001
v54 i7 p500
++++++++++++
Management of acute attacks
"Managing patients with acute intermittent porphyria involves removing the
precipitating factors, increasing carbohydrate intake, controlling pain, and
administering medications."
SOURCE:
AACN Clinical Issues
Critical Care Nursing
1994 Feb;5(1):36-41
Caring for patients with acute intermittent porphyria.
Shively BD, et.aL
+++++++++++.
Carbohydrates and parenteral nutrition
"Adequate calories are necessary and parenteral nutrition with carbohydrates
may be necessary.
Attacks may also require therapy for hypertension, pain and epileptic seizures."
SOURCE:
Journal of Photodermatololy, Photoimmunology and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D. Department of Medicine,
University Hospital
Helsinki, Finland.
++++++++
The first line of treatment
The first line of treatment has been the use of glucose infusion. In the 1980's
medical researchers discovered that the use of common glucose provided
enough carbohydrate to the liver wherby it would suppress the overproduction of
porphyrins and bring an acute attack to completion.
In some cases however porphyric attacks continue to persist even after 48 hours
of glucose infusion. Abbott Laboratories produced the pharmaceutical product
Panhematin to be used to treat those not responding fully to glucose infusion
therapy. Hematin, as it is known, unfortunately can not be used by those with a
history of phlebitis or other coagulation factors.
Panhematin
"Panhematin is not recommended for those AIP patients whih have a previous
history of DVT, PE, or phbletis. Surveys have rvealed that about 37% of all
porphyria patients have a history of previously coagulation problems and
therefore are not candidates for the use of the Panhematin."
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995
Yutaka Horie et. al.
+++++++++++++
Problems of hematin
"Hematin is not without side effects.Such side effects include thrombophletis
and renal toxicity."
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
++++++++++++
Glucose remains first choice of treatment
"Administration of carbohydrate is the first choice of therapy. If it does not
respond after 48 hours of glucose administration or if neurologic manifestations
develop or progress, treatment by hematin is indicated to suppress the ALA
synthetase activity [over production of porphyrins in the liver.]"
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al
+++++++++++
The glucose effect
"Glucose is most commonly used therapy in the intervention of acute porphyria
attacks. The "glucose effect" reverses or aborts acute porphyric attacks by
reducing the rate of porphyrin synthesis using normal endogenous energy
metabolism."
SOURCE:
"Use of glucose in acute porphyria"
Harris, A.L. et. al.
Journal of Family Medicine
++++++++++++
Action of carbohydrate
"Carbohydrate loadings represses hepatic ALA synthase and therefore the
reason that glucose in used as treatment during acute attacks."
SOURCE:
"The Porphyrias" Karl E. Anderson M.D.
HEPATOLOGY: A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
+++++++++++
"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
++++++++++
Glucose therapy
"Glucose can and does diminish the excess or overproduction of heme
precursors in the liver.
By using preventive glucose you can prevent an attack or by using the glucose
as Intevention therapy you can bring about the recovery from anyone one of the
acute hepatic porphyrias [AIP, VP or HCP]
Glucose therapy is simple. For those who have only occasional episodes
he can be iv accessed through a Hickman.
For those who regular run preventive glucose or are chronic porphyrics, and
need regular intervention care, the place of a PICC or a PORT is most desirable.
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 attacks and not needed to present for ermgency interventional
care.
Preventive therapy is most cost effective, as well as most importantly a healthy
move.
Preventing acute attacks, a porphyric reduces the risks of renal failure, liver
failure, scarring of the liver resulting in cirrhosis or hepatic cancer.
Moreover safe guarding onesself from the possibility of respiratory failure and
death is essential by use of preventive therapy."
SOURCE:
"Stopping Porphyrin Overproductionand the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
Respiratory failure
++++++++++
"The effective management of the acute relapse in acute hepatic porphyrias is
clinically important in order to avoid serious consequences such as a respiratory
failure."
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++++
Vomiting
"Where vomiting precludes an adequate oral intake of carbohydrate, it is
imperative to admit the patient for intravenous therapy. "
SOURCE:
Porphyria Information Centre
MRC/UCT Liver Research Centre
University of Cape Town S.A.
+++++++++++
Hyponatraemia and seizures
"Fluid overload may lead to symptomatic hyponatraemia, which often manifests
with seizures.
Thus serum electrolytes should be monitored regularly"
SOURCE:
Porphyria Information Centre
MRC/UCT Liver Research Centre
University of Cape Town S.A.
+++++++++++
Seizure activity needs medical care
"Seek medical care right away if you notice any unusual tingling, weakness, or
pain in your hands or feet.
Early diagnosis and treatment offer the best chance for controlling your
symptoms and preventing further damage to your peripheral nerves.
If your symptoms are interfering with your sleep or you feel depressed, your
doctor may be able to suggest treatments that can help"
SOURCE:
Mayo Health News
The Mayo Clinic
Rochester, MInnesota
Causes of seizures
"Severe hyponatraemia, due to inappropriate secretion of antidiuretic hormone,
complicates some attacks and sometimes presents as convulsions or
deterioration in the conscious level."
SOURCE:
Porphyria Research Unit
Department of Medicine
Brisbane, Queensland
1999
++++++++
"Gabapentin is widely approved as add-on therapy for epilepsy treatment for
partial seizures with and without secondary generalization..
Gabapentin at 900 or 1,800 mg/day is effective and safe as monotherapy
forpatients with newly diagnosed partial epilepsy."
SOURCE:
A double-blind trial of gabapentin monotherapy for newly diagnosed partial
seizures.
International Gabapentin Monotherapy Study Group 945-77.
Chadwick DW, et,. al.
Walton Centre for Neurology and Neurosurgery,
Liverpool, UK.
Neurology
1998 Nov;51(5):1282-8
+++++++++++
"Assessment of melatonin production in patients with acute intermittent porphyria
(AIP) was undertaken.
Tests susbjects were both with and without known epileptic seizures.
The test was to answer whether melatonin may have anti-convulsive or
pro-convulsive effects in AIP.
The AIP patients with epileptic seizures had a significantly lower urinary
excretion of melatonin, compared with their AIP relatives without epilepsy.
It is felt that this may be indicative that melatonin has a protective effect on
seizures."
SOURCE:
Epileptic Disorders
2000 Dec;2(4):203-8
Melatonin and epileptic seizures in patients with AIP
Bylesjo I, et. al.
Department of Medicine,
Skelleftea Hospital,
Skelleftea, Sweden.
++++++++++++
Seizure activiity must be monitored and treated during acute attacks.
The use of Gabapentin has proven both safe and beneficial.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++
"Acute intermittent porphyria is an autosomal dominant inborn error of heme
biosynthesis.
The diagnosis of acute porphyria is rare before puberty.
Its association with epilepsy induces difficulties in diagnosis and seizure
treatment.
Most antiepileptic drugs are unsafe in porphyria patients.
Adjustment of antiepileptic treatment is necessary.
SOURCE:
"Acute intermittent porphyrian associatedwith epilepsy in a child: diagnostic and
therapeutic difficulties."
Chaix, Y., et. al.
1997
Archives of Pediatrics
(10): 971-4.
++++++++++
Electrolytes need monitoring during acute attacks.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++
Electrolyte imbalances should be monitored during acute attacks.
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
Management of fluid and electrolyte imbalances, particularly hyponatremia and
hypomagnesemia, is critical during attacks.
SOURCE:
Maureen Poh-Fitzpatrick, MD
Department of Internal Medicine
Division of Dermatology
University of Tennessee
College of Medicine
++++++++++++
There must be management of fluid intake/output and elctrolyte imbalances,
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
+++++++++++++
Prophylaxis if the basic approach to AIP treatment
Because of the irreversible and basically untreatable nature of acute attacks of
porphyria, prophylaxis is the primary approach.
SOURCE:
Hereditary Hepatic Porphyrias
J. Katz et. al.
"Anesthesia and Uncommon DIsease:
Pathophysiologic and Clinical Correlations"
1973
W.D. Saunders Co. Philadelphia
++++++++++++
FIRST LINE TREATMENT
To date there is no cure for any of the porphyrias.
The infusion of IV glucose remains the first choice of treatment to control
acute episodes of porphyria.
Disorders of Porphyrin Metabolism
A Goldberg et. al.
Plenum Medical Book Company
New York
+++++++++++++
IV glucose infusion therapy is based on the observation that a high-carbohydrate
intake can stop the overproduction of porphyrins which cause the attack.
SOURCE:
Wintrobe's Clinical Hematology
Dr. Karl E. Anderson
"The Porphyrias
++++++++++++
Specific treatment should be instituted as soon as possible. COnditions can
worsen with delayed treatment.
SOURCE:
Management of acute and cutaneous porphyrias.
Elder GH. et. al.
Department of Medical Biochemistry
University of Wales College of Medicine
Cardiff, UK.
International Journal of Clinical Practice
2002 May;56(4):272-8
++++++++++++
Glucose suppresses the synthesis of ALA synthetase and is beneficial in acute
attacks.
SOURCE:
Acute Intermittent Porphyria
J.W. Stevens et. al.
Anesthesia & Analgesia
Volume 82 No. 2
February 1996
++++++++++++
Patients in acute attacks should have regular monitoring of pulse, blood
pressure and respiratory rate.
SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
++++++++++++
Treatment of the acute porphyrias is to encourage a high carbohydrate
intake.
SOURCE:
Porphyrias
Professional Guide to Disease
5th Edition
Springhouse Corporation 1995
+++++++++++++
Administration of carbohydrate is the first choice of therapy.
If it does not respond after 48 hours of glucoseadministration or if neurologic
manifestations develop or progress, treatment by hematin is indicated to
suppress the ALA synthetase activity [over production of porphyrins in the liver.]
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
++++++++++++
The disorders of AIP, HCP and VP are all treated intitially with the administration
of glucose and carbohydrta.e
The therapy has as its basis the ability of glucose to decrease porphyrin
biosynthesis in the liver.
SOURCE:
The porphyrias.
Kappas A, Sassa S, Galbraith RA.
The Metabolic Basis of Inherited Diseases,
Scriver CR, Beud AL, Sly WS, Valle D eds
6th edn.
New York:McGraw-Hill,
1989; 1305 65.
++++++++++++++
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
+++++++++++++
Treatment for acute attacks of porphyria includes a high carbohydrate intake.
SOURCE:
Porphyrias
Professional Guide to Disease
5th Edition
Springhouse Corporation 1995
+++++++++++++
Administration of carbohydrate is the first choice of therapy.
If it does not respond after 48 hours of glucoseadministration or if neurologic
manifestations develop or progress, treatment by hematin is indicated to
suppress the ALA synthetase activity [over production of porphyrins in the liver.]
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++++++++
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
+++++++++++++
Attacks of acute porphyria may be treated with intravenous glucose infusions,
glucose injections, and special high glucose drinks.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++
Clinical management of acute porphyria includes a high carbohydrate intake of
intravenous glucose.
SOURCE:
The Porphyrias
Kappas, A. et. al.
The Metabolic Basis of Inherited Disease
7th Edition 1995
+++++++++++++
Glucose given intravenously during acute attacks can be beneficial.
SOURCE:
Merck Manual
1996
+++++++++++
Early use of carbohydrate [dextrose] is mandatory because porphyria damage
must be avoided.
If this does not bring an attack under control then hematin should be employed.
Clinical remission hinges on early employment of this intervention."
SOURCE:
Dr. Claus Pierach M.D.
"The Porphyrias"
Conn's Current Therapy
*Dr. Pierach is the former chair of the APF medical/
scientific advisory board.
++++++++++++
Glucose can and does diminish the excess or overproduction of heme
precursors in the liver.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++
Treatment is based on infusing glucose, thereby slowing porphyrin precursor
excretion, and the resultant increased levels of cytotoxic metabolites.
SOURCE:
Prognosis of acute porphyria
Kauppinen R, Mustajoki P.
Medicine. 1992;71:1-13
+++++++++++++
Administration of carbohydrate is the first choice of therapy.
If it does not respond after 48 hours of glucose administration or if neurologic
manifestations develop or progress, treatment by hjematin is indicated to
suppress the ALA synthetase activity [over production of porphyrins in the liver.]"
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++++++
Specific treatment with iv infusion preparations should be instituted as soon as
possible following confirmation of increased excretion of porphobilinogen in the
urine in the acute porphyrias.
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.
+++++++++++++
Most acute attacks, if correctly recognized, settle with supportive treatment of
dextrose infusion and high carbohydrate intake.
SOURCE:
Stein, J. A.; & Tschudy, D. P. :
Acute intermittent porphyria:
a clinical and biochemical study...
Medicine
49: 1-16,
1970.
+++++++++++++
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.
Infusion of glucose and high oral ingestion of carbohydrates is essential.
SOURCE:
Intternational Journal of
Biochemistry and Cell Biology
1999
Mar-Apr;31(3-4):479-88
+++++++++++
Glucose reduces the activity of hepatic aminolevulinic acid synthase, the
rate-controlling enzyme of hepatic heme synthesis.
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
+++++++++++++
Determination of urinary porphobilinogen, urinary and faecal total porphyrins,
and total porphyrins in erythrocytes and plasma must be made in order to
achieve a correctand complete diagnosis.
After determination is completed immediateinfusion of glucose should begin."
SOURCE:
Journal of Clinical Pathology
2001 Jul;54(7):500-7
ACP Best Practice No 165:
Front line tests for the investigation of suspected porphyria.
Deacon AC, Elder GH.
Department of Clinical Biochemistry
King's College Hospital
Denmark Hill, London UK.
++++++++++++++
Glucose therapy is simple.
For those who have only occasional episodesone can be iv accessed through
a Hickman.
For those who regular run preventive glucose or are chronic porphyrics, and
need regular interventioncare, the place of a PICC or a PORT is most desirable.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++
Dextrose 10% IV is most commonly used during acute attacks.
SOURCE:
Porphyria
Greek for "Purple Urine"
Orthomolecular Medicine
++++++++++++
Specific therapies are few for acute porphyria but includes the use of high
carbohydrate intake.
SOURCE:
Dr. Michael R. Moore
Porphyria Research Unit
University of Queensland
Department of Medicine
Brisbane, Australia 1999
++++++++++++++
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
+++++++++++++
Glucose therapy is simple. For those who have only occasional episodes
it can be iv accessed through a Hickman.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++
Patients should be admitted to the hospital and IV administration of
carbohydrate initiated with dextrose to provide a minimum of 400
grams of carbohydrates.
SOURCE:
The Porphyrias
Kappas, A. et. al.
The Metabolic Basis of Inherited Disease
7th Edition 1995
++++++++++++++
Effective management requires prompt and accurate diagnosis.
Determination of urinary porphobilinogen, urinary and faecal total porphyrins,
and total porphyrins in erythrocytes and plasma must be made in order to
achieve a correct and complete diagnosis.
AFter determination is completed immediate infusion of glucose should begin."
SOURCE:
Journal of Clinical Pathology
2001 Jul;54(7):500-7
ACP Best Practice No 165:
Front line tests for the investigation of suspected porphyria.
Deacon AC, Elder GH.
Department of Clinical Biochemistry
King's College Hospital
Denmark Hill, London UK.
+++++++++++++
Initial treatment of acute porphyria patients with IV Infusion of glucose and high
oral ingestion of carbohydrates is essential.
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.
++++++++++++++++
Glucose can and does diminish the excess or overproduction of heme
precursors in the liver.
Glucose infusion will most generally bring acute attacks under control.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++
Attacks of acute porphyria may be treated with intravenous glucose infusions,
glucose injections, and special high glucose drinks.
Suck glucose drinks include HyCal which is manufacturered
by Beecham.
It must be understood that even today these treatments continue to help with
acute symptoms, but offer no lasting cure.
To date there is no cure for any of the porphyrias.
SOURCE:
Disorders of Porphyrin Metabolism
A Goldberg et. al.
Plenum Medical Book Company
New York
1987
++++++++++++
Treatment of the acute porphyrias is by glucose loading.
SOURCE:
Hepatic Porphyrias
Dr. Joseph R. Bloomer, MD
Diseases of the Liver and Biliary System
Ninth Edition 1993
Blackwell Scientific Publications
++++++++++++
During an acute attack a porphyric's requirement runs 500 mg of carbohydrate
total including both consumed and iv infusion of carbohydrate.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++
Glucose given intravenously is most frequently recommended to suppress the
biosynthetic pathway for heme.
SOURCE:
"The Porphyrias"
Diseases of the LIver and Biliary System
Dr. Joseph R. Bloomer, 1993
*Dr. Bloomer is a member of the APF
Medical Advisory Board.
++++++++++++
Glucose is most commonly used therapy in the intervention of acute porphyria
attacks.
The "glucose effect" reverses or aborts acute porphyric attacks by reducing the
rate of porphyrin synthesis using normal endogenous energy metabolism."
SOURCE:
"Use of glucose in acute porphyria"
Harris, A.L. et. al.
Journal of Family Medicine
++++++++++++
Infusion of glucose and high oral ingestion of carbohydrates is most beneficial in
bringing acugte attacks of porphyria under control.
SOURCE:
Delta aminolevulinate dehydratase (ALA-D) activity inhuman and experimental
diabetes mellitus.
Fernandez-Cuartero B et. al.
Department of Internal Medicine
University Complutense of Madrid,
Spain.
++++++++++++
For acute attacks triggered by drugs the use of high carbohydrate intake
and the intravenous infusion is the first line of treatment.
SOURCE:
Clinical Biochemistry
June 1989 22(3): pages181-8
Authors: Moore Michael R, and McColl KE
University Department of Medicine,
Western Infirmary, Glasgow, Scotland
++++++++++++
Effective management requires prompt and accurate diagnosis.
Determination of urinary porphobilinogen, urinary and faecal total porphyrins,
and total porphyrins in erythrocytes and plasma must be made in order to
achieve a correct and complete diagnosis.
AFter determination is completed immediate infusion of glucose should begin."
SOURCE:
Journal of Clinical Pathology
2001 Jul;54(7):500-7
ACP Best Practice No 165:
Front line tests for the investigation of suspected porphyria.
Deacon AC, Elder GH.
Department of Clinical Biochemistry
King's College Hospital
Denmark Hill, London UK.
++++++++++++++
An acute patient can be treated with large doses of intravenous glucose,
followed by injections of hematin.
SOURCE:
Diagnostic dilemmas in acute intermittent porphyria. A case report. Periasamy V,
al Shubaili A, Girsh Y.
Department of Neurology
Ibn Sina Hospital
Safat, Kuwait.
Medical Principles & Practice
2002 Apr-Jun;11(2):108-11
++++++++++++
Administration of carbohydrate is the first choice of therapy.
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++++
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
++++++++++++
With acute abdominal-neurological porphyria syndrome in the remission phase
treated with high carbohydrate intake (approximately 500 g/24 h), mainly in form
of intravenous glucose infusions the biochemical response with a
decrease of metabolites of porphyrin biosynthesis was highly significant,
accompanied by clinical improvement of patients.
SOURCE:
The "glucose effect" in acute hepatic porphyrias and in experimental porphyria.
Doss M, Verspohl F.
+++++++++++++
Infusions of glucose repress or inhibit hepatic ALQ sythase.
SOURCE:
Hepatic Porphyrias
Dr. Joseph R. Bloomer, MD
Diseases of the Liver and Biliary System
Ninth Edition 1993
Blackwell Scientific Publications
++++++++++++
Intravenous administration of glucose which is a pure form of carbohydrate,
is the standard treatment of acute attacks of porphyria.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
+++++++++++
The "glucose effect" has been investigated in vivo in humans in 32 courses
of hereditary acute hepatic porphyrias (acute intermittent porphyria, variegate
porphyria, coproporphyria and porphobilinogen synthase defect
porphyria).
SOURCE:
"Glucose effect" and rate limiting function of uroporphyrinogen synthase on
porphyrin metabolism in hepatocyte culture: relationship with human acute
hepatic porphyrias.
Doss M, et. al.
+++++++++++
Many porphyics fail to realize the importance or ignore the importance of their
carbohydrate [sugar] intake.
A steady daily amount is necessary to suppressdisease activity.
This is the basis of regular preventive glucose infusion.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++
MANAGING ACUTE ATTACKS
Management of acute attacks
"Managing patients with acute intermittent porphyria involves removing
the precipitating factors, increasing carbohydrate (glucose) intake, controlling
pain, and administering medications."
SOURCE:
AACN Clinical Issues
Clinical Care Nursing
1994 Feb;5(1):36-41
Caring for patients with acute intermittent porphyria.
Shively BD, et.aL.
+++++++++++
Carbohydrates and parenteral nutrition
"Adequate calories are necessary and parenteral nutrition with
carbohydrates may be necessary.
Attacks may also require therapy for hypertension, pain and epileptic
seizures."
SOURCE:
Journal of Photodermatololy, Photoimmunology and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D. Department of Medicine,
University Hospital
Helsinki, Finland.
++++++++++++
The first line of treatment
The first line of treatment has been the use of GLUCOSE infusion. In the
1980's medical researchers discovered that the use of common glucose
provided enough carbohydrate to the liver wherby it would suppress the
overproduction of porphyrins and bring an acute attack to completion.
In some cases however porphyric attacks continue to persist even after 48
hours of glucose infusion.
Abbott Laboratories produced the pharmaceutical
product Panhematin to be used to treat those not responding fully to glucose
> infusion therapy.
Hematin, as it is known, unfortunately can not be used by those with a history of
phlebitis or other coagulation factors.
***Today Panhematin is manufactured by Ovation Laboratories and is
significantly higher in costs.
Panhematin
"Panhematin is not recommended for those AIP patients whih have a previous
history of DVT, PE, or phbletis. Surveys have rvealed that about 37% of
all porphyria patients have a history of previously coagulation problems and
therefore are not candidates for the use of the Panhematin."
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995
Yutaka Horie et. al.
++++++++++
Problems of hematin
"Hematin is not without side effects.Such side effects include> thrombophletis
and renal toxicity."
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++++
Glucose remains first choice of treatment
>"Administration of carbohydrate is the first choice of therapy.
If it does not respond after 48 hours of glucose administration or if neurologic
manifestations develop or progress, treatment by hematin is indicated to
suppress the ALA synthetase activity [over production of porphyrins in the liver.]"
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al
+++++++++++
The glucose effect
"Glucose is most commonly used therapy in the intervention of acute
porphyria attacks.
The "glucose effect" reverses or aborts acute porphyric attacks by
reducing the rate of porphyrin synthesis using normal endogenous energy
metabolism."
SOURCE:
"Use of glucose in acute porphyria"
Harris, A.L. et. al.
Journal of Family Medicine
+++++++++++++
Action of carbohydrate
"Carbohydrate loadings represses hepatic ALA synthase and therefore the
reason that glucose in used as treatment during acute attacks."
SOURCE:
"The Porphyrias" Karl E. Anderson M.D.
HEPATOLOGY: A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
+++++++++++++
"Carbohydrate loading (glucose) 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 (University of Texas Medical School - Galveston)
The Porphyrias
Cecil Textbook of Medicine
W.B. Saunders
++++++++++
GLUCOSE THERAPY
"Glucose can and does diminish the excess or overproduction of heme
precursors in the liver.
By using preventive glucose you can prevent an attack or by using the
glucose as Intevention therapy you can bring about the recovery from anyone
one of the acute hepatic porphyrias [AIP, VP or HCP]
GLUCOSE therapy is simple.
For those who have only occasional episodes he can be iv accessed through a
Hickman.
For those who regular run preventive glucose or are chronic porphyrics, and
need regular intervention care, the placement of a PICC or a PORT is most
desirable.
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
attacks and not needed to present for ermgency
interventional care.
Preventive therapy is most cost effective, as well as most importantly a
healthy move.
Preventing acute attacks, a porphyric reduces the risks of renal
failure, liver failure, scarring of the liver resulting in cirrhosis or hepatic cancer.
Moreover safe guarding onesself from the possibility of respiratory
failure and death is essential by use of preventive therapy."
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
Respiratory failure
+++++++++++++
Vomiting
Where vomiting precludes an adequate oral intake of carbohydrate, it is
imperative to admit the patient for intravenous glucose therapy.
SOURCE:
Porphyria Information Centre
MRC/UCT Liver Research Centre
University of Cape Town S.A.
++++++++++++++++
Zofran has been found is be highly effective in controlling nausea and vomiting
during acute epsidoes.
SOURCE:
RObert Johnson MD
Internal Medicine
++++++++++++++
Electrolytes need monitoring
Electrolyte imbalances should be monitored during acute attacks.
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
+++++++++++
Electrolytes need to be checked during acute episodes to avoid loss
of potassium and subsequent hyponatremia.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++
Management of fluid and electrolyte imbalances, particularly hyponatremia
and hypomagnesemia, is critical during attacks along with the administeration
of glucose.
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
+++++++++++
Management of fluid and electrolyte imbalances during acute attacks is
essential.
SOURCE:
Maureen Poh-Fitzpatrick, MD
Department of Internal Medicine
Division of Dermatology
University of Tennessee
College of Medicine
+++++++++++++
Because of the irreversible and basically untreatable nature of acute
attacks of porphyria, prophylaxis is the primary approach. Glucose must be
administered for 48 hours after which time if attack has not gone into
remission heme arginate may be administered."
SOURCE:
Hereditary Hepatic Porphyrias
J. Katz et. al.
"Anesthesia and Uncommon DIsease:
Pathophysiologic and Clinical Correlations"
1973
W.D. Saunders Co. Philadelphia
+++++++++++++++
The effect of glucose on drug-promoted induction of porphyrin synthesis was
studied in chick embryo liver cell culture with and without the addition of
exogenous delta-aminolaevulinic acid (ALA).
Induction of ALA synthase was abolished by glucose.
Less than 10% of porphobilinogen is converted into protoporphyrin.
Protoporphyrin synthesis cannot be enhanced by high ALA concentrations.
The conversion of exogenous ALA into porphyrinsdecreases with increasing
concentrations of ALA from 0.1 to 2.0 mmol/l, whereas porphobilinogen
accumulates,thus reflecting the rate limiting function of uroporphyrinogen
synthase, which is not nfluenced by glucose.
This needle-eye-like function of uroporphyrinogen synthase within the porphyrin
biosynthetic chain explains the urinary increase of ALA and porphobilinogen in
the acute phase of variegate and coproporphyria, similar to that in acute
intermittent porphyria.
The "glucose effect" was also investigated in vivo in humans in 32 courses
of hereditary acute hepatic porphyrias (acute intermittent porphyria, variegate
porphyria, coproporphyria and porphobilinogen synthase defect porphyria).
Patients were treated with high carbohydrate intake (approximately 500 g/24 h),
mainly in the form of glucose infusions.
There was a resulting consistent and highly significant decrease of porphyrin
biosynthesis metabolites, accompanied by clinical improvement in most of the
patients.
SOURCE:
"Glucose effect" and rate limiting function of uroporphyrinogen synthase on
porphyrin metabolism in hepatocyte culture: relationship with human acute
hepatic porphyrias.
Doss M, Sixel-Dietrich F, Verspohl F
++++++++++++++
Infusion protocol
Infusions. Immediately start IV with D15w - 15% Dextrose in water, (or
D10w-10% Dextrose in water).
Flow rates: 300ml per hour - 1st liter. 250ml per hour - 2nd liter. 200ml per
hour-subsequent liters.
D50 - 50 cc's bolos can be given to supplement. Duration 36 to 72 hours -
occasionally longer.
APF recommends: Hospitalization is often necessary for acute attacks.
Medicine for pain, nausea and vomiting along with close observation are
required.
Initial treatment of acute attacks consists of stopping harmful drugs the patient
may be taking and providing a high intake of glucose, 300 grams or more per
day.
Glucose can be given either in the form of an oral carbohydrate or by
intravenous infusion. Intravenous infusion is better in moderate or severe
attacks, or for patients who are unable to ingest enough carbohydrate orally.
Pain, anxiety, and emotional symptoms should be treated with safe drugs.
Attacks with muscle weakness occasionally require respiratory support, but this
is unusual unless an attack is brought on by prolonged administration of harmful
drugs.
After recovering from an attack, a patient should continue to eat regularly,
because there is good evidence that skipping meals or fasting is harmful.
SOURCE:
American Porphyria Foundation
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++
TREATMENT - GLUCOSE
Fructose
A 6 carbon sugar (hexose) abundant in plants.
Fructose has its reducing group (carbonyl) at C2 and thus is a ketose, in
contrast to glucose that has its carbonyl at C1 and thus an aldose.
Sucrose, common table sugar, is the nonreducing disaccharide formed by an
alpha linkage from C1 of glucose to C2 of fructose (latter in furanose form).
Fructose is a component of polysaccharides such as inulin, levan.
Glucose
D glucose, a monosaccharide (hexose), C6H12O6, found in certain foodstuffs,
especially fruits and in the normal blood of all animals. It is the end product of
carbohydrate metabolism and is the chief source of energy for living organisms,
its utilisation being controlled by insulin. Excess glucose is converted to
glycogen and stored in the liver and muscles for use as needed and, beyond
that, is converted to fat and stored as adipose tissue. Glucose appears in the
urine in diabetes mellitus. In patients with an acute hepatic porphyria, glucose is
used as both intervention and preventive therapy. The use of glucose stops the
overproduction of porphyrins in the heme pathway and liver.
Glucose effect
The ability of the sugar glucose to block sugar metabolism by keeping the genes
which make the enzymes involved in the early steps of sugar metabolism from
making those enzymes.
Hexose
Hexose is a nonsaccharide form of glucose.
Levolose -
Levolose is a form of fructose.
Maintenance treatment
Treatment given for a period of months or years to maintain remission. In
porphyria this is often referred to as preventive treatment.
Sucrose
Sucrose, common table sugar, is the nonreducing disaccharide formed by an
alpha linkage from C1 of glucose to C2 of fructose (latter in furanose
form).
*********************************************
HEMATIN aka PANHEMATIN
Description
What is hematin?
Hematin is a preparation of heme marketed for treatment and prevention of
acute porphyrias in the United States.
++++++++++++++++
What is heme?
Heme is the all-important iron-binding molecule essential for the proper function
of many proteins, including hemoglobin (oxygen-transport), cytochrome c
(energy production) and cytochrome P-450 (detoxification).
SOURCE:
J. Alexander Bralley et. al.
MetaMetrix
Testing for Toxic Metals
+++++++++++++++++++++++++++++++++++++
Action
What does heme do?
Heme is synthesized in every eukaryotic and prokaryotic cell and it carries out
many important biologic functions.
It is the prosthetic group of many hemeproteins.
SOURCE:
Molecular genetics of acute intermittentporphyria in Finland
Sami Mustajoki
Division of Endocrinology,
Department of Medicine,
University of Helsinki &
Department of Human Molecular Genetics,
National Public Health Institute, Finland
++++++++++++++++++++++++++++++++
What is the role of heme?
Heme is most noted for the part that it plays in thehemoglobin, which is the
body's oxygen delivery system.
Hepatic [liver] heme is necessary for the manufacture of microsomal cytochrome
P-450.
The P-450 system is critical in the detoxification of drugs and chemicals.
Chemicals and drugs that adversely effectcytochrome P-450 production are also
known as porphyrinogenic.
SOURCE:
Elsevier
North Holland Biomedical Press
Amsterdam, The Netherlands
1979
+++++++++++++++++++++++++++++
Biochemical facts
What are some of the basic facts involving heme and porphyria?
Evaluation of cutaneous heme metabolism shows that: 1) the heme biosynthetic
pathway is present in epidermal and endothelial cells.
There followed anaccumulation of porphyrin, the functional assay for
ferrochelatase.
2) Porphyrin and radiation-induced alteration of the heme biosynthetic pathway
in epidermal cells is mainly caused by affecting the ferrochelatase in these cells
rather than ALA synthase.
3) Porphyrin metabolism in the skin of can be altered by the Soret band of
radiation."
SOURCE:
Dr. Henry Lim
Department of Dermatoogy
New York University
School of Medicine
"Cutaneous Heme Metabolism and the Porphyrias"
++++++++++++++++++++++++++++++
Hematin description
What is hematin?
Hemin (Panhematin) is an enzyme inhibitor derived from processed red blood
cells and an iron containing metalloporphyrin.
Panhematin was previously known as hematin, a term used to describe the
chemical reaction product of hemin and sodium carbonate solution.
Panhematin has anticoagulant effect and may cause thrombophlebitis at the
infusion site.
Must be reconstituted from lyophilized powder.
Reconstitute with human serum albumin 25% (132 mL of 25% HSA to 1 vial of
hemin [313 mg heme]) and infuse into large vein to reduce risk of
thrombophlebitis. The mixture must be immediately used and must have been
completely mixed.
Some doctors advise the misxture to have human albumin added which reduces
the chances of blood clots.
SOURCE:
Robert Johnson M.D.
Internal Medcine
++++++++++++++++++++++++++++++++
Hemin (Panhematin) is an enzyme inhibitor derived from processed red blood
cells and an iron containing metalloporphyrin.
Panhematin was previously known as hematin, a term used to describe the
chemical reaction product of hemin and sodium carbonate solution.
Panhematin has anticoagulant effect and may cause thrombophlebitis at the
infusion site.
>
Must be reconstituted from lyophilized powder.
Reconstitute with human serum albumin 25% (132 mL of 25% HSA to 1 vial of
hemin [313 mg heme]) and infuse into large vein to reduce risk of
thrombophlebitis. The mixture must be immediately used and must have been
completely mixed.
Some doctors advise the misxture to have human albumin added which reduces
the chances of blood clots.
SOURCE:
Robert Johnson
Internal Medcine
+++++++++++++++++++++
Hematin studies have been carried out in five cases of hepatic porphyria,
including three of acute intermittent porphyria, one of variegate porphyria, and
one of porphyria cutanea tarda in clinical remission.
SOURCE:
Effects of hematin in hepatic porphyria. Further studies.
Dhar GJ, Bossenmaier I, Petryka ZJ, Cardinal R, Watson CJ.
Annals of Internal Medicine.
1975 Jul;83(1):20-30.
++++++++++++
Heme Arginate
What is heme arginate?
Heme arginate, which is marketed in some other countries, is another
preparation of heme for intravenous administration.
Heme arginate is an investigational drug in the United States.
It has fewer side effects (especially phlebitis) than hematin.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++++++++++++++++++
Heme, Hematin and Heme Arginate
How can you distinguish heme, hemin and hematin from one another?
If iron (II), or ferrous iron, is added the product is heme.
If iron (III), or ferric iron, is added the product is
a.. hemin if the counterion is chloride.
b.. hematin if the counterion is hydroxide.
Porphyrinogens are more reduced than the corresponding porphyrins, so
oxidation of a porphyrinogen (pronounce) produces the corresponding
porphyrin.
SOURCE:
University of Utah Medical School
Biochemistry Department
++++++++++++++++++++++++++++++
If iron (II), or ferrous iron, is added the product is heme.
If iron (III), or ferric iron, is added the product is
a.. hemin if the counterion is chloride.
b.. hematin if the counterion is hydroxide.
Porphyrinogens are more reduced than the corresponding porphyrins, so
oxidation of a porphyrinogen (pronounce) produces the corresponding
porphyrin.
SOURCE:
University of Utah Medical School
Biochemistry Department
+++++++++++++++++++++++++++++++++++++
Panhematin
Hematin became available in 1971.
SOURCE:
Mortality in patients with acute intermittent porphyria requiring hospitalization: a
United States case series.
Bossenmaier IC, Pierach CA, Bloomer JR. et. al.
Watson Laboratory
University of Minnesota
Minneapolis,
+++++++++++++++++++++
Hematin was the first drug approved by the FDA for rare diseases under the
Orphan Drug Act.
For many years the drug wasavailable from Abbott Laboratories in Chicago
under the trade name Panhematin®.
The patent and manufactrurerof the drug has been sold toa private firm and
each vialruns around $1,900.
SOURCE:
Use of Hematin
The Porphyrias
United Medical Services
2003
++++++++++++++++++++++++++
For those who use Penhematin which wasformerly manufactured by Abbott
Laboratories,it is now available from Ovation Pharmaceuticals.
Panhematin® is still the brand name and can be ordered by calling
1-888-514-5204.
Panhematin now carries a price tag of$1,970 per vial. Often more than onevial
is used per acute attack.
SOURCEL:
Robert Johnson MD
Internal Medicine
+++++++++++++++++
Hematin is made from human blood.
Products made from human blood may contain infectious agents, such as
viruses, that can cause disease.
The risk that such products will transmit an infectious agent has been reduced
by screening blood donors for prior exposure to certain viruses, by testing for the
presence of certain current virus infections, and by inactivating certain viruses.
Despite these measures, such products can still potentially transmit disease.
SOURCE:
Renae Livingston MNS
Neurology Department
+++++++++++
Clinical severity of acute porphyria was associated with a pronounced increase
in activity of this enzyme and high urinary excretion of A.L.A.
Haematin therapy resulted in clinical improvement associated with a reduction in
the activity of A.L.A. synthase and reduction in urinary excretion of A.L.A.
SOURCE:
Haematin therapy and leucocyte delta-aminolevulinic-acid-synthase activity in
prolonged attack of acute porphyria.
McColl KE, Thompson GT, Moore MR, Goldberg A.
Lancet.
1979 Jan 20;1(8108):133-4.
++++++++++++++++
Hematin acts to limit the hepatic and/or marrow synthesis of porphyrin.
This action is likely due to the inhibition of [delta]- aminolevulinic acid
synthetase, the enzyme which limits the rate of the porphyrin/heme biosynthetic
pathway.
The exact mechanism by which hematin produces symptomatic improvement in
patients with acute episodes of the hepatic porphyrias has not been elucidated.
SOURCE:
James Wetterson
Infusion Therapy
+++++++++++
If a patient does not respond after 48 hours of glucose administration or if
neurologic manifestations develop or progress, treatment by hematin is indicated
to suppress the ALA synthetase activity [over production of porphyrins in the
liver.]"
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++++++
The brand name for Hemin is Panhemation.
Hemin for injection is an enzyme inhibitor derived from processed red blood
cells.
Hemin for injection was known previously as hematin.
The term hematin has been used to describe the chemical reaction product of
hemin and sodium carbonate solution.
Hemin is an iron containing metalloporphyrin.
Panhematin is a sterile, lyophilized powder suitable for intravenous
administration after reconstitution.
Each dispensing vial of Panhematin contains the equivalent of 313 mg hemin,
215 mg sodium carbonate and 300 mg of sorbitol.
The pH may have been adjusted with hydrochloric acid; the product contains no
preservatives.
When mixed as directed with Sterile Water for Injection, USP, each 43 ml
provides the equivalent of approximately 301 mg hematin (7 mg/ml).
SOURCE:
Panhematin
Abbott Laboratories
Chicago, Illinois, USA
++++++++++++++++++++++++++++
Hematin infusion is intended to limit the rate of porphyria/heme biosynthesis
possibly by inhibiting the enzyme [delta]-aminolevulinic acid synthetase.
SOURCE:
James Wetterson
Infusion Therapy
+++++++++++
Panhematin is a sterile, lyophilized powder suitable for intravenous
administration after reconstitution.
Am J Med Genet.
1996 Nov 11;65(4):269-73.
+++++++++++++++
A large arm vein or a central venous catheter should be utilized for the
administration of hemin for injection to avoid the possibility of developing bloods
clots.
SOURCE:
Renae Livingston MNS
Neurology Department
+++++++++++
In two cases of acute intermittent porphyria (in relapse), a marked lowering
effect on serum and urine porphobilinogen and delta-aminolevulinic acic was
observed after the infusion of hematin.
There was a prompt and gratifying clinical improvement.
SOURCE:
Effects of hematin in hepatic porphyria. Further studies.
Dhar GJ, Bossenmaier I, Petryka ZJ, Cardinal R, Watson CJ.
Annals of Internal Medicine.
1975 Jul;83(1):20-30.
++++++++++++
Hematin has exhibited transient, mild anticoagulant effects during clinical
studies.
SOURCE:
James Wetterson
Infusion Therapy
+++++++++++
Phlebitis with or without leucocytosis and with or without mild pyrexia has
occurred after administration of hematin through small arm veins.
SOURCE::
Robert Johnson MD
Internal Medicine
+++++++++++++
The use of hematin infusion in VP is generally reserved until 48 hours of
glucose have proven ineffective.
SOURCE:
James Wetterson
Infusion Therapy
+++++++++++
Hematin therapy is frequently associated with phlebitis or thrombophletis.
Side effects are found in 50% of patients using hematin.
SOURCE:
The Porphyrias
Kappas, A. et. al.
The Metabolic Basis of Inherited Disease
7th Edition 1995
++++++++++++++
Frequent treatments with hematin lead to a reduced therapeutic effect.
SOURCE:
The Porphyrias
Kappas, A. et. al.
The Metabolic Basis of Inherited Disease
7th Edition 1995
+++++++++++++
It has been found that some patients have exhibited prolonged prothrombin
time and partial thromboplastin time, after infusio of hematin.
SOURCE:
Renae Livingston MNS
Neurology Department
+++++++++++
Although clinical improvements occurred in the case of variegate porphyria after
the infusion of hematin, the results were inconclusive.
SOURCE:
Effects of hematin in hepatic porphyria. Further studies.
Dhar GJ, Bossenmaier I, Petryka ZJ, Cardinal R, Watson CJ.
Annals of Internal Medicine.
1975 Jul;83(1):20-30.
++++++++++++
Hematin is a very unstable solution.
SOURCE:
The Porphyrias
Kappas, A. et. al.
The Metabolic Basis of Inherited Disease
7th Edition 1995
+++++++++++++
Hematin is not without side effects.
Such side effects include thrombophletis and renal toxicity.
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
++++++++++++++
Heme therapy is seldom indicated unless the diagnosis of acute porphyria is
proven by a marked increase in urine PBG.
*How heme therapy should be used to prevent attacks is not well established.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++
The extent and duration of the hypocoagulable state induced by hematin
infusion has not been established.
SOURCE:
James Wetterson
Infusion Therapy
+++++++++++
The use of Panhematin may carry a risk of renal damage.
SOURCE:
Use of hematin in the
acute porphyrias.
Dhar et al.,
1978
++++++++++++
Porphyria studies have shown that when heme therapy is given late in an
attack, the overall benefits may not occur.
When hematin is given early, the heme therapy almost always normalized
ordinary porphyrin and porphyrin precursor values, bringing an acute attacks
under control.
SOURCE:
Findings of Use of Hematin
The Porphyrias
United Medical Services
1997
++++++++++++
There has been clinical evidence of a 10% fall in hematocrit following infusion
of hematin.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++
In a porphyria patient in chemical remission but with longstanding
psychoneurosis, no significant effects were noted with the infusion of hematin.
SOURCE:
Effects of hematin in hepatic porphyria. Further studies.
Dhar GJ, Bossenmaier I, Petryka ZJ, Cardinal R, Watson CJ.
Annals of Internal Medicine.
1975 Jul;83(1):20-30.
++++++++++++
The use of Panhematin in PCT has not proven beneficial.
SOURCE::
Robert Johnson MD
Internal Medicine
+++++++++++++
No beneficial effects of the infusion of hematin have been observed in
porphyria cutanea tarda.
SOURCE:
Effects of hematin in hepatic porphyria. Further studies.
Dhar GJ, Bossenmaier I, Petryka ZJ, Cardinal R, Watson CJ.
Annals of Internal Medicine.
1975 Jul;83(1):20-30.
++++++++++++
Clinical benefit from hematin infusion depends on prompt administration.
SOURCE::
Robert Johnson MD
Internal Medicine
+++++++++++++
PN effects
Attacks of porphyria may progress to a point where irreversible neuronal
damage has occurred.
Hematin infusion therapy is intended to prevent an attack from reaching the
critical stage of neuronal degeneration.
SOURCE::
Robert Johnson MD
Internal Medicine
+++++++++++++
Panhematin will have no effect on recovery of peripheral neuropathy.
Nerve regeneration is the rate-limiting factor to improvement of established
neuropathy.
SOURCE:
Acute Intermittent Porhyria
Anne LeMaistre, M.D.
1995
TMC
++++++++++++
Hematin infusion is not effective in repairing neuronal damage.
SOURCE:
Renae Livingston MNS
Neurology Department
+++++++++++
Reversible shutdown of the kidneys has occurred with administration of
excessive doses of hematin.
SOURCE:
Roger Blaylock
Renal Dialysis & Infusion Therapy
+++++++++++
Since reconstituted hematin infusion is not transparent.
Any undissolved particulate matter is difficult to see when inspected visually.
Terminal filtration through a sterile 0.45 micron or smaller filter is recommended.
SOURCE:
James Wetterson
Infusion Therapy
+++++++++++
Following hematin treatment, symptoms generally return.
In some cases remission of porphyria is thought to be prolonged.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++
There has been reported coagulopathy occurring during hematin therapy.
SOURCE:
James Wetterson
Infusion Therapy
+++++++++++
Hematin infusion should only be used by physicians experienced in the
management of porphyrias in hospitals where the recommended clinical and
laboratory diagnostic and monitoring techniques are available.
SOURCE:
Roger Blaylock
Renal Dialysis & Infusion Therapy
+++++++++++
Hematin therapy for the acute porphyrias is not curative.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++
Heme therapy
What is heme therapy?
Intravenous heme therapy is more potent in suppressing disease activity.
It can be started after a trial of glucose therapy. However, the response to heme
therapy is best if started early in an attack.
Heme must be administered by vein.
Panhematin®, manufactured by Abbott
Laboratories, is the only commercially available heme therapy for treatment
and prevention of acute porphyric attacks in the United States.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++++++++++++++++
Heme, the end biosynthetic product, is the most effective therapy after glucose
infusion for restoration of porphyrin biosynthesis to normal.
Heme is usually infused at 3 mg/kg daily for 4 days.
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.
+++++++++++++++++++++++++
Etiology of Hematin
What year did hematin become available?
Hematin became available in 1971.
SOURCE:
Mortality in patients with acute intermittent porphyria requiring hospitalization: a
United States case series.
Bossenmaier IC, Pierach CA, Bloomer JR. et. al.
Watson Laboratory
University of Minnesota
Minneapolis,
American Journal of Medical Genetics.
1996 Nov 11;65(4):269-73.
+++++++++++++++++++++++++++++++
Hematin was first documented as used in 1971.
SOURCE:
Findings of Use of Hematin
The Porphyrias
United Medical Services
1997
+++++++++++++++++++++++++++++++
There have been no clinical controlledstudies of hematin.
However based upon the clinically chartedresults, clinicians and researchers
alikefelt that the reported results were impressive enough to support a medical
protocol of the early use of heme therapy for porphyria
attacks.
SOURCE:
Findings of Use of Hematin
The Porphyrias
United Medical Services
1997
+++++++++++++++++++++
Hematin was first documented as used in 1971.
SOURCE:
Findings of Use of Hematin
The Porphyrias
United Medical Services
1997
++++++++++++++++++++
The brand name for Hemin is Panhemation.
Hemin for injection is an enzyme inhibitor derived from processed red blood
cells.
Hemin for injection was known previously as hematin.
The term hematin has been used to describe the chemical reaction product of
hemin and sodium carbonate solution.
Hemin is an iron containing metalloporphyrin.
Panhematin is a sterile, lyophilized powder suitable for intravenous
administration after reconstitution.
Each dispensing vial of Panhematin contains the equivalent of 313 mg hemin,
215 mg sodium carbonate and 300 mg of sorbitol. The pH may have been
adjusted with hydrochloric acid; the product contains no preservatives.
When mixed as directed with Sterile Water for Injection, USP, each 43 ml
provides the equivalent of approximately 301 mg hematin (7 mg/ml).
SOURCE:
Panhematin
Abbott Laboratories
Chicago, Illinois, USA
+++++Begining in 2003 Abbott Laboratories no longer manufacturered
Panhematin and sole manufacture of Panhemagtin was taken over by
Ovation Pharmaceuticals.
++++++++++++++++++++++++++++++++++
Panhematin has exhibited transient, anticoagulant effects during clinical
studies.
Concurrent anticoagulant therapy should be avoided when using Panhematin.
The extent and duration of the hypocoagulable state induced by Panhematin
has not been established.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++
For those who use Penhematin which was formerly manufactured by Abbott
Laboratories, it is now available from Ovation Pharmaceuticals.
Panhematin® is still the brand name and can be ordered by calling
1-888-514-5204.
Panhematin now carries a price tag of $1,970 per vial or more. Often more than
onevial is used per acute attack.
Robert Johnson M.D.
2003
++++++++++++++++++++++++++++++++++
Hematin was the first drug approved by the FDA for rare diseases under the
Orphan Drug Act.
For many years the drug was available from Abbott Laboratories
in Chicago under the trade name Panhematin®.
The patent and manufactrurer of the drug has been sold to a private firm and
each vial runs around $1,960.
SOURCE:
Use of Hematin
The Porphyrias
United Medical Services
2003
+++++++++++++++++++++++++++
Benefits
Do acute porphyria patients benefit from having hematin treatments?
Many patients have received hematin since 1971.
Some patients have benefited clinically.
If a patient does not respond to iv glucose over a 48 hour period, the use of
hematin should begin.
If hematin is prolonged in starting it has shown to be less beneficial.
SOURCE:
Findings of Use of Hematin
The Porphyrias
United Medical Services
1997
++++++++++++++++++++++++++++++++++
The exact mechanism by which hematinbrings forth a symptomatic
improvementin porphyria patients with acute attacks has not been elucidated.
Researchers speculate that it may well be the combined therapy of hematin and
the glucose that brings forth any benefits.
Hematin alone has not proven anymoreeffective than glucose alone, and has
numerous side effects.
Coagulations problems are known factor in the use of hematin.
About 10% of acute porphyria patients have received benefit from the use of
refined hematin that bypasses coagulationproblems with being mixed with
albumin.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++
What is the benefit of using Panhematin?
Clinical benefit from Panhematin depends on prompt administration.
Attacks of porphyria may progress to a point where irreversible neuronal
damage has occurred.
Panhematin therapy is > intended to prevent an attack from reaching the critical
stage of neuronal degeneration.
It for this reason that Panhematin is to be started at the end of 48 hours of
gluose infusion iif an acute attack has not begun to subside.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++++
Why is hematin used in the treatment of porphyria?
The scientific basis for administering heme therapy is to correct heme deficiency
in the liver and thereby repress production of porphyrin precursors which trigger
the accummulation/overproduction of porphyrins which cause attacks
to occur.
SOURCE:
Findings of Use of Hematin
The Porphyrias
United Medical Services
1997
+++++++++++++++++++++++++++++
Some neurological symptoms have improved weeks to months
in acute porphyria patients although there was little or often no response noted
during the time of treatment.
Clinicians tend to believe that such improvement is
due to the fact the patient is in remission rather than
to the use of panhematin.
There is no clinical evidence to neurological change due to use of panhematin
SOURCE:
Robert Johnson M.D.
++++++++++++++++++++++
When a patient fails to respond after 48 hours of intravenous glucose feeding
the use of hematin should begin immediately.
SOURCE:
Mustajoki P & Nordmann Y.
Early administration of heme arginate
for acute porphyric attacks.
Mustajoki P & Nordmann Y.
Archives of Internal Medicine
1993; 153: 2004 8.
++++++++++++++++++++
Clinical benefit from Panhematin depends on prompt administration.
Attacks of porphyria may progress to a point where irreversible
neuronal damage has occurred.
Panhematin therapy is intended to prevent an attack from reaching the critical
stage of neuronal degeneration.
It for this reason that Panhematin is to be started at the end of 48 hours of
gluose infusion iif an acute attack has not begun to subside.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++++
Intravenous heme therapy is more potent in suppressing disease activity.
It can be started after a trial of glucose therapy. However, the response to heme
therapy is best if started early in an attack.
Heme must be administered by vein.
Panhematin®, manufactured by Abbott Laboratories, is the only commercially
available heme therapy for treatment and prevention of acute porphyric attacks
in the United States.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
++++++++++++++++++++++++++++++++
Is heme therapy recommended, and how effective is it?
Heme therapy is seldom indicated unless the diagnosis of acute porphyria is
proven by a marked increase in urine PBG.
*How heme therapy should be used to prevent attacks is not well established.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
++++++++++++++++++++++++++++
In patients who do not have coagulopathies or are not on anticoagulant therapy
the use of hematin can be considered.
SOURCE:
Acute Intermittent Porhyria
Anne LeMaistre, M.D.
1995
TMC
++++++++++++++++++++++++++
What is the reason for the use of Panhematin?
Panhematin infusion therapy is intended to limit the rate of
porphyria/heme biosynthesis possibly by inhibiting the enzyme
[delta]-aminolevulinic acid synthetase.
For this reason, drugs such as estrogens, barbituric acid derivatives and steroid
metabolites which increase the activity of [delta]-aminolevulinic acid synthetase
should be avoided.
Use of Panhematin should come only after 48 hours of glucose infusion has
failed to bring an attack under control.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++
The scientific basis for administering heme therapy is to correct heme deficiency
in the liver andthereby repress production of porphyrin precursors which
triggerthe accummulation/overproduction of porphyrins which cause attacks to
occur.
SOURCE:
Findings of Use of Hematin
The Porphyrias
United Medical Services
1997
+++++++++++++++++++++
Renal Problems
WIth high doses renal toxcity or renal failure will occur.
SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Montgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
+++++++++++++++++++++++++
Hematin is not without side effects.
Such side effects include thrombophletis and renal toxicity.
Treatment for Porphyria
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
++++++++++++++++++++++++
Has the use of hematin in treatment of acute porphyria proven beneficial?
The exact mechanism by which hematin brings forth a symptomatic
improvementin porphyria patients with acute attacks has not been elucidated.
Researchers speculate that it may well be the combined therapy of hematin and
the glucose that brings forth any benefits.
Hematin alone has not proven anymore effective than glucose alone, and has
numerous side effects.
Coagulations problems are known factor in the use of hematin.
About 10% of acute porphyria patients have received benefit from the use of
refined hematin that bypasses coagulation problems with being mixed with
albumin.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++
Hematin at high doses can cause renal toxicity.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
+++++++++++++++++++++++
When should hematin be considered for intervening in acute porphyria?
When a patient fails to respond after 48 hours of intravenous glucose feeding
the use of hematin should begin immediately.
SOURCE:
Mustajoki P & Nordmann Y.
Early administration of heme arginate for acute porphyric attacks.
Mustajoki P & Nordmann Y.
Archives of Internal Medicine
1993; 153: 2004 8.
+++++++++++++++++++++++++++++++
Does Panhematin cure porphyria?
Panhematin therapy in acute hepatic porphyrias is not curative.
In fact panhematin is problematic for the majority of acute porphyrics in that
many porphyria patients have a history of coagulation problemsand therefore
are not suitable to receive Panhematin which by itself is known for side effects
which include coagulation problems as well as renal failure.
SOURCE:
Robert Johnson M.D.
Internal Medcine
++++++++++++++++++++++++++++++++
Problems with Hematin
Can coagulopathy occur in patients receiving panhematin?
Coagulopathy has been clinically cited in patients receiving intravenous
treatment of hematin.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++++
Panhematin® is less likely to produce phlebitis if it is mixed with human albumin
before it is given.
(Directions for preparing Panhematin® in this manner can be obtained from
porphyria specialists.)
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++++++++++++++++++
The side effects of using hematin include chemical phlebitis at the
site of the infusion and a transient anticoagulant effect.
WIth high doses renal toxcity or renal failure will occur.
SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Montgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
+++++++++++++++++++++++
Treatment by infusion of hematin has side effects and is complex
inadministration.
SOURCE:
Dr. David Lambeth
Biochemistry
Medical School
University of North Dakota
+++++++++++++++++++++
There is Instability of hematin used in the treatment of acute hepatic porphyria.
SOURCE:
Instability of Hematin
Goetsch CA, Bissell DM
New England Journal of Medicine
1986 Jul 24;315(4):235-8
+++++++++++++++++++++
The side effects of using hematin include chemical phlebitis at the
site of the infusion and a transient anticoagulant effect.
SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Montgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
++++++++++++++++++++++
WIth the use of Hematin there can be reversible renal shutdown which has
occurred with administration of hematin in porphyria patients.
One should always read the "Precautions" regarding Hematin beforeprocesing to
have the pharmaceutical administered to them.
SOURCE:
Regina Jacobsen MSN RN
IV Infusion Specialist
++++++++++++++++++++++++++
Permanent implanted PICCs or PORTS are the best for administering
Panhematin.
If one is not in place a large arm vein or a central venous catheter should be
utilized for the administration of hemin for injection to avoid the possibility of
phlebitis.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++
What is the biochemical process of hematin in providing temporary relief in
acute porphyria?
Heme acts to limit the hepatic and / or marrow sythesis of porphyrins.
This biochemical action is due to the inhibition of ALA which is the enzyme
which limits the rate of the porphins and heme biosynthetic pathway.
Hematin is only a stop-gap measure and has proven beneficial to only about
10% of the acute porphyria community.
The regular form of hematin has coagulopathy and phlebitis concerns.
Both forms of hematin administration run the risk of renal failure.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++++
Do acute porphyria patients experience negative side effects from having
hematin treatments?
Coagulation problems occur often usually do to faulty mixture of the solution and
proper/timely administration of the hematin.
Phlebitis often occurs.
SOURCE:
Findings of Use of Hematin
The Porphyrias
United Medical Services
1997
+++++++++++++++++++++++++++++
Is Panhematin of any value if one does not have coagulation problems which
causes them to be contraindicated for the use of the substance?
If started early in the attack , panhematin may have benefit through the
lowering of porphyrin precursor excretion in patients without coagulation
factors."
SOURCE:
Acute Intermittent Porhyria
Anne LeMaistre, M.D.
1995
TMC
+++++++++++++++++++++++++++++
What is the reason for the use of Panhematin?
Panhematin infusion therapy is intended to limit the rate of porphyria/heme
biosynthesis possibly by inhibiting the enzyme [delta]-aminolevulinic acid
synthetase.
For this reason, drugs such as estrogens, barbituric acid derivatives and steroid
metabolites which increase the activity of [delta]-aminolevulinic acid synthetase
should be avoided.
Use of Panhematin should come only after 48 hours of glucose infusion has
failed to bring an attack under control.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++++++
Have there been controlled studies of Hematin?
There have been no clinical controlled studies of hematin.
However based upon the clinically charted results, clinicians and researchers
alike felt that the reported results were impressive enough to support a medical
protocol of the early use of heme therapy for porphyria attacks.
SOURCE:
Findings of Use of Hematin
The Porphyrias
United Medical Services
1997
+++++++++++++++++++++++++++++++
Is the administration of hematin complex?
Treatment by infusion of hematin has side effects and is complex in
administration.
SOURCE:
Reginald Vejaski M.D.
Vascular Medicine
++++++++++++++++++++++++++++
Pre-administration testing
Why is porphyria testing done before Panhematin is administered in a hospital?
Tests for Diagnosis and Monitoring of Therapy:
Before Panhematin infusion therapy is begun, the presence of acute porphyria
must be diagnosed using the following criteria:
1.. Presence of clinical symptoms.
2.. Positive Watson-Schwartz or Hoesch test. (A negative Watson-Schwartz or
Hoesch test indicates a porphyric attack is highly unlikely.
3.. When in doubt quantitative measures of [delta]-aminolevulinic acid and
porphobilinogen in serum or urine may aid in diagnosis.)
SOURCE:
Robert Johnson M.D.
Internal Medicine
*Medical Procedure Handbook
Mosby 2002
++++++++++++++++++++++++++++
Normal hematin administration protocol
What is the normal protocol for heme administration?
Heme, the end biosynthetic product, is the most effective therapy after glucose
infusion for restoration of porphyrin biosynthesis to normal.
Heme is usually infused at 3 mg/kg daily for 4 days.
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.
+++++++++++++++++++++++++++++
When should hematin be used in those porphyrics that can tolerate hematin?
If an acute attack does not respond in 48 hours to carbohydrate iv infusion, or if
neurologic manifestations progress, treatment with hematin is then indicated.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1997
Lippincott Co.
+++++++++++++++++++++++++++++++
If an acute attack does not respond in 48 hours to carbohydrate iv infusion, or if
neurologic manifestations progress, treatment with hematin is then indicated.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
+++++++++++++++++++++++++++++
Can Panhematin be administered by the porphyria patuients themselves
like preventive or interventive glucose infusions?
Panhematin infusions should only be administered byphysicians that are highly
esperience in the management ofacute porphyrias and in hospitals where the
recommendedclinical and laboratory diagnostic and monitoring techniques are
available.
SOURCE:
Pedro S.Sequlda M.D.
Vascular Medicine
Infusion Therapy
+++++++++++++++++++++++++++++++
What have clinical studies shown in regard to phlebitis when using Panhematin?
Panhematin has exhibited transient, anticoagulant effects during clinical
studies.
Concurrent anticoagulant therapy should be avoided when using Panhematin.
The extent and duration of the hypocoagulable state induced by Panhematin
has not been established.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++
Panhematin® is less likely to produce phlebitis if it is mixed with human albumin
before it is given.
(Directions for preparing Panhematin® in this manner can be obtained from
porphyria specialists.)
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++++++++++++++
"If started early in the attack , panhematin may have benefit through the
lowering of porphyrin precursor excretion in patients without coagulation
factors."
SOURCE:
Acute Intermittent Porhyria
Anne LeMaistre, M.D.
1995
TMC
+++++++++++++++++++++++++++
In patients who do not have coagulopathies or are not on anticoagulant therapy
the use of hematin can be considered.
SOURCE:
Acute Intermittent Porhyria
Anne LeMaistre, M.D.
1995
TMC
++++++++++++++++++++++++++
Hematin therapy for the inherited porphyrias has not been fully developed.
Many who undertake such therapy have adverse reactions.
Such reactions include deep vein thrombosis, phlebitis and coagulopathy."
SOURCE:
Dr. A,. Kappas MD
"The Porphyrias"
The Metabolic Basis of Inherited Disease"
McGraw-Hill
++++++++++++++++++++++++++++
What pharmacology studies shown in regard to the use of Panhematin?
Most aspects of human pharmacokinetics have not be defined in relation to the
use of Panhematin.
SOURCE:
Richard Menninger PhD RPm
Pharmacology & Toxicology
++++++++++++++++++++++++++++
What pharmacology studies shown in regard to the use of Panhematin?
Most aspects of human pharmacokinetics have not be defined in relation to the
use of Panhematin.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++
Panhematin solution used for infusions is made from human blood.
Products made from human blood may contain infectious agents, such as
viruses,that can cause disease.
The risk that such products will transmit an infectious agent has been reduced
by screening blood donors for prior exposure to certain
viruses, by testing for the presence of certain current virus infections, and by
inactivating certain viruses.
Despite these measures, such products can still potentially transmit disease.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++
Consistency of quality
Is Hematin always of consistent quality?
There is Instability of hematin used in the treatment of acute hepatic porphyria.
SOURCE:
Instability of Hematin
Goetsch CA, Bissell DM
New England Journal of Medicine
1986 Jul 24;315(4):235-8
++++++++++++++++++++++++++++
Can panhematin be used by those who have had bloodclots?
In patients who do not have coagulopathies or are not on anticoagulant therapy
the use of hematin can be considered.
SOURCE:
Acute Intermittent Porhyria
Anne LeMaistre, M.D.
1995
Texas Medical College
+++++++++++++++++++++++++++
Phlebitis with or without leucocytosis and with or without mild pyrexia has
occurred after administration of hematin through small arm veins.
There have been reports of coagulopathy occurring in patients receiving
hematin therapy.
When this occurs there can be prolonged prothrombin time and partial
thromboplastin time as well as thrombocytopenia.
In addition there may be hypofibrinogenemia, an elevation of fibrin split products
and a 10% fall in hematocrit.
SOURCE:
Regina Jacobsen MSN RN
IV Infusion Specialist
+++++++++++++++++++++++++++++
Does hematin carry risks with it's use?
Further studies are needed to work out solutions to the use of hematin in
porphyria patients.
Presently a large number of patients have had problems with coagulation.
SOURCE:
Watson CJ. et. al.
Effects of hematin in hepatic porphyria.
Further studies.
Ann Intern Med.
1975 Jul;83(1):20-30.
+++++++++++++++++++++++++++
What are the signs of coagulopathy in porphyria patients receiving hematin?
Patients that present with coagulopathydue to administration of hematin oftenwill
present with prolonged prothrombin time and partial thromboplastin time.
Many will present with thromocytopenia.
In addition many patient receiving hematin infusion will exhibit
hypofibrinogenemia. They can have a
fibrin split.
Hematrocit often falls 10% in levels.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++
Panhematin can be used by patients who do not have coagulopathies.
No one on Coumadin/Wayfarin therapy is a candidate for panhmatin infusion.
SOURCE:
Hector Gonsalves M.D.
Vascular Medicine & Infusion Therapy
++++++++++++++++++++++++++
Does Panhematin cure porphyria?
Panhematin therapy in acute hepatic porphyrias is not curative.
In fact panhematin is problematic for the majority of acute porphyrics in that
many porphyria patients have a history of coagulation problems and therefore
are not suitable to receive Panhematin which by itself is known for side effects
which include coagulation problems as well as renal failure.
SOURCE:
Robert Johnson M.D.
Internal Medcine
++++++++++++++++++++++++++++
Can Panhematin be administered by the porphyria patients themselves like
preventive or interventive glucose infusions?
Panhematin infusions should only be administered byphysicians that are highly
esperience in the management ofacute porphyrias and in hospitals where the
recommendedclinical and laboratory
diagnostic and monitoring techniques are available.
SOURCE:
Chrissy Wilkinson RN
Infusion Specialist
+++++++++++++++++++++++++++++
Side effects of hematin consist of chemical phlebitis.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
+++++++++++++++++++++++++++
Can blood clots occur with the use of hematin?
Coagulation problems arise in the use of hematin in the treatment of hepatic
porphyrias.
SOURCE:
Instability of Hematin
Goetsch CA, Bissell DM
New England Journal of Medicine
1986 Jul 24;315(4):235-8
+++++++++++++++++++++++++++++
Coagulopathy has been clinically cited in patients receiving intravenous
treatment of hematin.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++++
Coagulation problems occur often usually do to faulty mixture of the solution and
proper/timely administration of the hematin.
Phlebitis often occurs.
SOURCE:
Findings of Use of Hematin
The Porphyrias
United Medical Services
1997
++++++++++++++++++++++++++++++
Coagulation problems arise in the use of hematin in the treatment of
hepaticporphyrias.
SOURCE:
Instability of Hematin
Goetsch CA, Bissell DM
New England Journal of Medicine
1986 Jul 24;315(4):235-8
+++++++++++++++++++++++++++
Side effects of hematin consist of chemical phlebitis.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
+++++++++++++++++++++++++++
Hematin therapy for the inherited porphyrias has not been fully developed.
Many who undertake such therapy have adverse reactions.
Such reactions include deep vein thrombosis, phlebitis and coagulopathy."
Dr. A,. Kappas MD
"The Porphyrias"
The Metabolic Basis of Inherited Disease"
McGraw-Hill
++++++++++++++++++++++++++
What are some of the problems with hematin use?
Hematin therapy for the inherited porphyrias has not been fully developed.
Many who undertake such therapy have adverse reactions.
Such reactions include deep vein thrombosis, phlebitis and coagulopathy.
Dr. A,. Kappas MD
"The Porphyrias"
The Metabolic Basis of Inherited Disease"
McGraw-Hill
+++++++++++++++++++++++++
Can renal chemical phlebitis occur with the use of hematin?
"The side effects of using hematin include chemical phlebitis at the site of the
infusion and a transient anticoagulant effect.
WIth high doses renal toxcity or renal failure will occur."
SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Montgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
++++++++++++++++++++++++++++++++
What are the signs of coagulopathy in porphyria patients receiving hematin?
Patients that present with coagulopathy due to administration of hematin often
will present with prolonged prothrombin time and partial thromboplastin time.
Many will present with thromocytopenia.
In addition many patient receiving hematin infusion will exhibit
hypofibrinogenemia. They can have a fibrin split.
Hematrocit often falls 10% in levels.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++
Does the use of Panhematin help in nerve repair?:
Panhematin infusion is not effective in repairing neuronal damage.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++
In a study of urinary aluminum and zinc chelation as well as the use of heme in
the treatment of acute porphyric patients , the study concludes that the
effectiveness of hematin injections during relases has yet to be proven.
Until there is a proper randomized trial, management of of acute attacks will be
based on personal experience rather than on questionable data."
Hematin Use in Porphyric Relapses
J.I. Suarez, MD
1998, June
American Academy of Neurology
+++++++++++++++++++++++++++++
Following intravenous administration of panhematin there can be an
increased in urobiligen.
The increase is usally in porportion to the amount of panhemain received.
In persons who are jaundice, the urobiligen remain unchanged.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++
How long do Panhematin infusions last?
After discontinuation of Penhematin insions, the porphyria symptoms return.
In a small percentage of cases remission is prolonged.
A much higher success rate is associated with glucose infusion which can also
be used preventively avoiding acute attacks.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++++
Bilirubin metabolites are also excreted in the urine for up to 24 hours following
panhematin infusions.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++++
Are hematin injections effective?
In a study of urinary aluminum and zinc chelation, as well as the use of heme
in the treatment of acute porphyric patients , the study concludes that the
effectiveness of hematin injections during relases has yet to be proven.
Until there is a proper randomized trial, management of of acute attacks will be
based on personal experience rather than on questionable data.
SOURCE:
Hematin Use in Porphyric Relapses
J.I. Suarez, MD
1998, June
American Academy of Neurology
++++++++++++++++++++++++++
Does elimination of bilirubin increase with the use of panhematin infusions?
Bilirubin metabolites are also excreted in the urine for up to 24 hours following
panhematin infusions.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++
Doe urobilingen increase in acute porphyrics when they receive panhematin?
Following intravenous administrationof panhematin there can be an increased in
urobiligen.
The increase is usally in porportion to the amount of panhemain received.
In persons who are jaundice, the urobiligen remain unchanged.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++++
After discontinuation of Penhematin insions, the porphyria symptoms
return.
In a small percentage of cases remission is prolonged.
A much higher success rate is associated with glucose infusion which can also
be used preventively avoiding acute attacks.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++
Nerve damage and use of Hematin
Panhematin infusion is not effective in repairing neuronal damage.
SOURCE:
Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++
Does panhematin use help with the neurological problems associated with acute
attacks?
Some neurological symptomshave improved weeks to monthsin acute porphyria
patients although there was little or often no response noted during the time of
treatment.
Clinicians tend to believe that such improvement is due to the fact the patient
is in remission rather than to the use of panhematin.
There is no clinical evidence to neurological change due to use of panhematin
SOURCE:
Elisabeth Sturveysant M.D.
Neurology
Chemical Studies
+++++++++++++++++++++++++
Does EPP have a reduction of heme activity?
Reduction in activity of heme synthase of ferrochelatase, a mitochondrial
enzyme and the final step in the heme synthesis pathway; to 10 to 25% of
normal levels has been found in EPP patients.
SOURCE::
Fatal liver failure in protoporphyria:
Bonkovsky, H.L. et. al
Gastroenterology 90: 191-201, 1986.
++++++++++++++++++++++++++++++
Hematin & CEP
Heme therapy is benefiial in CEP.
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
++++++++++++++++++++++++++
Heme acts to limit the hepatic and / or marrow sythesis of
porphyrins.
This biochemical action is due to the inhibition of ALA which is the enzyme
which limits the rate of the porphins and heme biosynthetic pathway.
Hematin is only a stop-gap measure and has proven beneficial to only
about 10% of the acute porphyria community.
The regular form of hematin has coagulopathy and phlebitis concerns.
Both forms of hematin administration run the risk of renal failure.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++++++
Renal toxcity or failure
Can renal toxcity occur with the use of hematin?
"The side effects of using hematin include chemical phlebitis at the site of the
infusion and a transient anticoagulant effect.
WIth high doses renal toxcity or renal failure will occur."
SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Montgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
++++++++++++++++++++++++
"Hematin is not without side effects.
Such side effects include thrombophletis and renal toxicity."
Treatment for Porphyria
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++++++++++++++++
Hematin at high doses can cause renal toxicity.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
+++++++++++++++++++++++++++
Renal shutdown has occurred with the administration of panhematin in acute
porphyria patients.
Acute porphyria patients with any history of renal disease should avoid the use
of hematin.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++++
The side effects of using hematin include chemical phlebitis at the site of the
infusion and a transient anticoagulant effect.
WIth high doses renal toxcity or renal failure will occur."
SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr.Dwight Montgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
++++++++++++++++++++++++++
Renal shutdown has been ] observed where excessive hematin has been
administered in a single infusion.
SOURCE:
Victor Krejcik M.D.
Nephrology
+++++++++++++++++++++++++++
Since panhematin contains blood is it safe from disease?
Panhematin solution used for infusions is made from human blood.
Products made from human blood may contain infectious agents, such as
viruses,that can cause disease.
The risk that such products will transmit an infectious agent has been reduced
by screening blood donors for prior exposure to certain viruses, by testing for the
presence of certain current virus infections, and by inactivating certain viruses.
Despite these measures, such products can still potentially transmit disease.
SOURCE:
Sylvan Hectora M.D.
Hematology
+++++++++++++++++++++++++++
Is the use of hematin well established?
Hematin therapy is still evolving and should be undertaken with full recognition
of adverse consequences, especially phlebitis and coagulopathy.
The iv dosage is up to 4 mg/kg once or twice daily of hematin.
SOURCE:
Dr. M.M. McGovern et al
"Inherited Porphyrias"
Principles and Practices of Medical Genetics
3rd Edition
1997
++++++++++++++++++++++++++++++
Hematin therapy for the inherited porphyrias has not been fully developed.
Many who undertake such therapy have adverse reactions.
Such reactions include deep vein thrombosis, phlebitis and coagulopathy.
SOURCE:
Dr. A,. Kappas MD
"The Porphyrias"
The Metabolic Basis of Inherited Disease"
McGraw-Hill
+++++++++++++++++++++++++
Further studies are needed to work out solutions to the use of hematin in
porphyria patients.
Presently a large number of patients have had problems with coagulation.
SOURCE:
Watson CJ. et. al.
Effects of hematin in hepatic porphyria.
Further studies.
Annals of Internal Medicine
1975 Jul;83(1):20-30.
++++++++++++++++++++++++++++++
What are the ingredients of hematin?
HEMATIN contains dessicated defatted liver concentrate, ascorbic acid,
cellulose, iron aspartate, niacin, pyridoxine HCL, vegetable stearine, copper
aspartate, Vitamin B-12 as hydroxocobalamin, folic acid, magnesium stearate,
cellulose coating.
SOURCE:
David Liers
Bringing Balance to Life Naturally
Health Products
Tubac AZ
++++++++++++++++++++
HEMATIN is a unique formulation of vitamins, minerals, and cofactors needed by
the body in order to ensure the proper absorption and utilization of iron, and the
efficient and adequate production of red blood cells,
The product includes iron, copper, Vitamin C, Folic Acid, Niacin, Vitamin B-6,
Vitamin B-12, and dessicated liver.
SOURCE:
David Liers
Bringing Balance to Life Naturally
Health Products
Tubac AZ
++++++++++++++++
"Hematin therapy is still evolving and should be undertaken with full recognition
of adverse consequences, especially phlebitis and coagulopathy.
The iv dosage is up to 4 mg/kg once or twice daily of hematin."
SOURCE:
Dr. M.M. McGovern et al
"Inherited Porphyrias"
Principles and Practices of Medical Genetics
3rd Edition
1997
+++++++++++++++++++++++ ++++
Panhematin, previously known as hematin, is a term used to describe the
chemical reaction product of hemin and sodium carbonate solution.
SOURCE:
Reginald Perkins PhD
Pharmacology & Toxicology
++++++++++++++++++
Phlebitis with or without leukocytosis or mild pyrexia has occurred after
administration of Panhemaatin through small arm veins.
When using Panhematin it is best to use a large arm vein or a central venous
catheter.
with terminal filtration through a sterile 0.45 um or smaller filter.
Panhematin, previously known as hematin, is a term used to describe the
chemical reaction product of hemin and sodium carbonate solution.
SOURCE:
Reginald Perkins PhD
Pharmacology & Toxicology
++++++++++++++++++
Hemin (Panhematin) is a heme analogue for treatment of acute attacks.
Panhematin is an
enzyme inhibitor derived from processed red blood cells.
Panhematin is an iron-containing metalloporphyrin.
SOURCE:
Reginald Perkins PhD
Pharmacology & Toxicology
++++++++++++++++++
Panhematin may prevent a porphyria attack from causing neuronal
degeneration.
Panhematin is not effective to repair neuronal damage.
Panhematin identified in asymptomatic and reversible renal shutdown, oliguria,
and increased nitrogen retention.
Panhematin, previously known as hematin, is a term used to describe the
chemical reaction product of hemin and sodium carbonate solution.
SOURCE:
Reginald Perkins PhD
Pharmacology & Toxicology
++++++++++++++++++
Panhematin is a Heme analogues.
Panhematin is an iron-containing metalloporphyrins reduce hepatic and marrow
synthesis of porphyrin by inhibiting d-ALA synthetase, the rate-limiting enzyme in
the porphyrin biosynthetic pathway.
Clinical symptoms (eg, pain, hypertension, tachycardia, mental status changes,
neuropathy) may be controlled.
SOURCE:
Reginald Perkins PhD
Pharmacology & Toxicology
++++++++++++++++++
Often there is coagulopathy after Panhematin therapy, with prolonged PT and
aPTT, thrombocytopenia, mild hypofibrinogenemia, mild elevation of fibrin split
products, and a 10% fall in hematocrit.
Panhematin, previously known as hematin, is a term used to describe the
chemical reaction product of hemin and sodium carbonate solution.
SOURCE:
Reginald Perkins PhD
Pharmacology & Toxicology
++++++++++++++++++
HEME ARGINATE
Heme Arginate
What is heme arginate?
Heme arginate, which is marketed in some other countries, is another
preparation of heme for intravenous administration.
Heme arginate is an investigational drug in the United States.
It has fewer side effects (especially phlebitis) than hematin.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++++++++++++++++++
Drug name
Normasang is the pharmaceutical name for Heme Arginagte.
SOURCE:
Robert JOhnson MD
Internal Medicine
+++++++++++++
Heme arginate is produced under the brand name Normosang by Leiras Medica
of Finland.
SOURCE:
Reginald Perkins PhD
Pharmacology & Toxicology
++++++++++++++++++
Heme arginate is not approved for use in the United States.
SOURCE:
Robert JOhnson MD
Internal Medicine
+++++++++++++
Heme arginate may have a lower incidence of thrombophlebitis than hemin
(Panhematin).
SOURCE:
Reginald Perkins PhD
Pharmacology & Toxicology
++++++++++++++++++
Heme arginate improves cytochrome P-450-mediated drug metabolism.
SOURCE:
Reginald Perkins PhD
Pharmacology & Toxicology
++++++++++++++++++
The treatment of the acute crises of the hepatic porphyries consists in making up
the cellular heme deficit by infusions of hème-arginate.
The fukll pharmaceutical name for heme arginate is known as Normosang.
More than 700 crises were thus treated in 10 years, with a significant reduction
of the average duration of hospitalization (3,5 days against 21 days before
1986).
SOURCE:
Dr. Jean-Charles Deybach, Pu-PH
French Porphyria Center
++++++++++++++
Heme, Hematin and Heme Arginate
How can you distinguish heme, hemin and hematin from one another?
If iron (II), or ferrous iron, is added the product is heme.
If iron (III), or ferric iron, is added the product is
a.. hemin if the counterion is chloride.
b.. hematin if the counterion is hydroxide.
Porphyrinogens are more reduced than the corresponding porphyrins, so
oxidation of a porphyrinogen (pronounce) produces the corresponding
porphyrin.
SOURCE:
University of Utah Medical School
Biochemistry Department
++++++++++++++++++++++++++++++
If iron (II), or ferrous iron, is added the product is heme.
If iron (III), or ferric iron, is added the product is
a.. hemin if the counterion is chloride.
b.. hematin if the counterion is hydroxide.
Porphyrinogens are more reduced than the corresponding porphyrins, so
oxidation of a porphyrinogen (pronounce) produces the corresponding
porphyrin.
SOURCE:
University of Utah Medical School
Biochemistry Department
+++++++++++++++++++++++++++++++++++++
SUPPLEMENTAL TREATMENT FOR ATTACKS
To date there is no cure for any of the porphyrias.
The infusion of IV glucose remains the basic treatment for the acute porphyrias.
Disorders of Porphyrin Metabolism
A Goldberg et. al.
Plenum Medical Book Company
New York
+++++++++++
Medicine for pain, nausea and vomiting along with close observation
are required during an acute attack.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++
Pain can be quite severe during acute attacks and must be treated immediately.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++
Severe pain is often treated with narcotic analgesics.
Pain should be treated only under a doctor's supervision.
Some patients have reported benefits from drug-free approaches to pain
management.
Such pain management therapies include hypnosis,physical therapy, heat
therapy, TENS and accupuncture."
SOURCE:
Disorders of Porphyrin Metabolism
A Goldberg et. al.
Plenum Medical Book Company
New York
1987
+++++++++++
Patients in acute attacks should have regular monitoring of pulse, blood
pressure and respiratory rate.
SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++
Zofran has been found to be quite effective in controlling nausea and vomiting
associated with acute episodes of porphyria.
SOURCE:
Robert Johnson MD
Internl Medicine
++++++++++
Pain, anxiety, and emotional symptoms of acute attacks should be treated with
safe drugs.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++
Initial treatment of AIP attacks consists of stopping harmful drugs the patient
may be taking and providing a high intake of glucose, 350 grams or more per
day.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
45-8 6:1999
++++++++++
If potassium levels have fallen supplemental potassium can be infused along
with the iv infusion of glucose.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++
Often porphyria patients experience hypertension and/or tachycardia during
the onset of acute episodes.
The use of Propranolol has been found to be beneificial to control the cardiac
aspects of acute attacks.
SOURCE:
Robert Johnson MD
Internl Medicine
+++++++++
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.
++++++++++
Carbohydrate loading may be used for mild attacks and can be given orally as
sucrose, glucose polymers, or carbohydrate-rich foods. If oral intake is poorly
tolerated glucose may be administered intrvenously for at least 300 grams per
day.
SOURCE:
The Porphyrias
Dr. Karl E. Anderon MD
Cecil Textbook of Medicine
W.B. Saunders 1996
+++++++++++++
During the acute attacks, supportive therapy is required including narcotic
analgesics, tranquilizers such as chlorpromazine, antinauseants, rehydration,
sodium and magnesium replacement, high carbohydrate diets and intravenous
therapy with high concentrations of glucose.
SOURCE:
The Canadian Porphyria Foundation Inc.
Neepawa, Manitoba, Canada
+++++++++++
In addition to iv infusion of glucose porphyria patients can find it beneficial to
suck glucose drinks which include HyCal which is manufacturered
by Beecham.
SOURCE:
Disorders of Porphyrin Metabolism
A Goldberg et. al.
Plenum Medical Book Company
New York
1987
+++++++++++
Cyclobenzaphrine has been found to be useful in control the muscle spasms
experienced during acute episodes of porphyria.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++
Glucose can be given either in the form of an oral carbohydrate or by
intravenous infusion. Intravenous infusion is better in moderate or severe
attacks, or for patients who are unable to ingest enough carbohydrate orally.
SOURCE:
"Stopping Porphyrin Overproduction and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++
In case of acute attack, try drinking beverages containing glucose or fructose as
well as lots of water (1-2 cups per hour minimum for several hours), and eating a
quick meal of carbohydrates.
In cases of respiratory paralysis or seizure, which can be life-threatening,
doctors should consider immediate injections of glucose."
SOURCE:
Evaluating Porphyrin Metabolism
Dr. Grace Ziem, MD
1996
+++++++++++
The glucose levels in the hippocampus of rats fall significantly during complex
cognitive tasks.
Researchers measured the brain levels of glucose while the animals ran mazes.
The glucose levels in older rats, those more than 2 years old, dipped nearly 50
percent in the hippocampus and needed a half-hour to replenish.
The rats' decisions about which way to run apparently were impaired by their
brains' inability to retrieve glucose from nearby cells.
Younger rats, those 3 months old, had only a 12 percent decline in glucose
levels and recovered more quickly.
When the older rats were given glucose supplements before running the maze,
they didn't show the same sharp drop in brain sugar levels and did the job as
well as younger rats.
Lest you think the human brain has little in common with rats, previous
experiments showed that both college students and people with
Alzheimer's disease do better on memory tasks after drinking sugary lemonade
than when drinking saccharine-sweetened beverages.
It does caution that sugar's effect on memory is fleeting.
However too much sugar, like too much stress, also can impair memory."
SOURCE:
Glucose Improves Memory
Dr. Paul Gold,Neuroscience, University of Illinois
Journal of Gerontology and Neurobiology of Learning and Memory
+++++++++++
PROGNOSIS IN THE PORPHYRIAS
Porphyria arises because of a flaw in the body's heme-making machinery.
The body produces heme and other porphyrins in a series of eight coordinated
stages, each catalyzed by a separate enzyme. Iron is added at the end to make
heme.
In porphyria, one of the steps does not occur, leading to a backlog of the
intermediate compounds produced earlier in the sequence. The body has not
evolved to dispose of these intermediates efficiently, so it dumps them, often in
the skin.
The intermediates do not damage the skin directly, but many of them cause
trouble indirectly.
Metal-free porphyrins (as well as metalloporphyrins containing metals that do not
interact with the porphyrin ring) can become excited when they absorb light at
certain wavelengths; their electrons jump into higher-energy orbitals.
The molecules can then transmit their excitation to other molecules having the
right kind of bonds, especially oxygen, to produce reactive singlet oxygen and
other highly reactive and destructive molecules known as free radicals.
Metal-free porphyrins, in other words, are not the agents, but rather the brokers,
of destruction.
They catalyze the production of toxic forms of oxygen
SOURCE:
Scientific American
New Light on Medicine
Nick Lane
December 16, 2002
++++++++++++
The onset of acute porphyria is insidious, but once symptoms occur, the
complaints tend to run an undulating course throughout the remainder of
the patient's life.
SOURCE:
Acute intermittent porphyria in a
children's psychiatric hospital.
Boon, F. F. and C. Ellis
J Am Acad Child Adolesc Psychiatry
1989; 28(4): 606-9.
+++++++++++
Patients should wear a Medic-Alert bracelet or necklace which would provide a
warning against administration of dangerous drugs including anaesthetics in an
emergency situation.
SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++
Many people, probably the majority of those who have the propensity for
porphyria, go through their entire lives with the inherited gene defects
and never know they have this disease.
They are called asymptomatic carriers and may be considered to have latent
disease.
SOURCE:
The Canadian Porphyria Foundation Inc.
Neepawa, Manitoba, Canada
++++++++++++++++
It is a good idea to carry in your wallet a card that summarizes your chronic
medical conditions, the medicines you are taking, and your allergies and drug
sensitivities.
SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester MN
+++++++++++
Even with modern treatment and prevention, some patients still have repeated
attacks.
However, progressive deterioration and death from paralysis in the "acute"
porphyrias has greatly dropped in the last few years.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
++++++++++++++
The porphyrias are life-long intermittent illnesses, but with good long-term
management, the affected person can expect long problem-free periods.
SOURCE:
Medline
U.S. National Library of Medicine,
8600 Rockville Pike,
Bethesda, MD 20894
+++++++++++++++
You should keep a medical information card as up-to-date as possible.
Carry it with you at all times
SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester MN
+++++++++++
Subscribe to Medic Alert which will provide you with the card
along with around-the-clock telephone contact service which can anable
healthcare workers to know your history in case you are in an accident
and are dealing with an unfamiliar clinic, ER, or ambulance service.
SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester MN
+++++++++++
Neurologic deficits may require months to several years to clear.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
+++++++++++
The porphyrias are life-long intermittent illnesses, but with good long-term
management, the affected person can expect long problem-free periods.
SOURCE:
Medline
U.S. National Library of Medicine,
8600 Rockville Pike,
Bethesda, MD 20894
+++++++++++++++
Clinical remission of an attack hinges on early employment of intervention.
SOURCE:
The Porphyrias
Pierach, Dr. Claus A.
Conn's Current Therapy
1998
++++++++++
It must be understood that even today with various treatment options and as
new treatments continue to help with acute symptoms, none offer any lasting
cure.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++
Attacks may last days or months.
SOURCE:
Porphyria
Dictionary of Medical Syndromes
Fourth Edition 1997
Lippincott-Raven
++++++++++++++
Unlike some genetic diseases in which all individuals who inherit an abnormal
gene become ill, the severity of porphyria varies considerably.
Such variability is due to certain "additional factors" other than the gene itself.
Consequently, risks of severe medical difficulties or even death in the "acute"
porphyrias are often diminished when affected individuals are well informed of
their diagnosis and adopt suggested precautionary measures.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
++++++++++
The porphyrias are life-long intermittent illnesses, but with good long-term
management, the affected person can expect long problem-free periods.
SOURCE:
Medline
U.S. National Library of Medicine,
8600 Rockville Pike,
Bethesda, MD 20894
+++++++++++++
Long-term management:
Avoid all alcohol
Avoid drugs which may precipitate an attack
High carbohydrate diet
Avoid sunlight
Sunscreen (if not contraindicated)
Avoid skin trauma
Preventive glucose infusion p.r.n.
SOURCE:
Medline
U.S. National Library of Medicine,
8600 Rockville Pike,
Bethesda, MD 20894
+++++++++++++
To date there is no cure for any of the porphyrias.
SOURCE:
Disorders of Porphyrin Metabolism
A Goldberg et. al.
Plenum Medical Book Company
New York
1987
++++++++++++
There are no cures for any of the types of porphyria.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++
It must be understood that even today these treatments continue to help with
acute symptoms, but offer no lasting cure.
SOURCE:
Disorders of Porphyrin Metabolism
A Goldberg et. al.
Plenum Medical Book Company
New York
1987
+++++++++++++
Porphyria may be considered to be acute with the sudden onset of serious life
threatening symptoms, or it can be chronic with only minimally bothersome
intermittent problems that develop gradually over months and persist for years.
SOURCE:
The Canadian Porphyria Foundation Inc.
Neepawa, Manitoba, Canada
+++++++++++++
Acute porphyria often have rapid onset of multiple and profound neurovisceral
dysfunctions that can permanently incapacitating.
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
++++++++++++++
Disfigurement of some cutaenous porphyria patients may result.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++
With improved diagnosis and treatment options in the porphyrias, many
patients have increased lifespans; consequently, issues of long-term quality of
life are coming to the forefront.
Mental health concerns are among these issues.
SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry
University of California, San Diego
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
++++++++++++
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 menstrual
hormone cycles along with external or exogenous changes including exposure to
stress, drugs, sunlight, alcohol and even fasting.
SOURCE:
The Canadian Porphyria Foundation
Neepawa, Manitoba, Canada
+++++++++++++
Exposure to sunlight can cause reddening, pain, sensations of heat, blistering
and edema in the skin.
These lesions heal slowly, often with scarring or pigment changes and can be
disfiguring.
SOURCE:
Medline
U.S. National Library of Medicine,
8600 Rockville Pike,
Bethesda, MD 20894
++++++++++++
Remission is an batement of signs and symptoms of a medical condition.
In acute porphyria it means that you are "between acute attacks".
SOURCE:
Mental Health Resource
Mayo Foundation for Medical Education and Research
Rochester, Minnesota
2003
+++++++++++
In most porphyria patients today there is a resulting consistent and highly
significant decrease of porphyrin biosynthesis metabolites, accompanied by
clinical improvement in most of the patients.
SOURCE:
"Glucose effect" and rate limiting function of uroporphyrinogen synthase on
porphyrin metabolism in hepatocyte culture: relationship with human acute
hepatic porphyrias.
Doss M, et. al.
+++++++++++
Patients with the cutaneous photosensitivity symptoms have difficulty
performing manual labor and many daily activities.
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
++++++++++++
With improved diagnosis and treatment options in the porphyrias, many
patients have increased lifespans; consequently, issues of long-term quality of
life are coming to the forefront.
Mental health concerns are among these issues.
SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry
University of California, San Diego
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
+++++++++++++
Gene therapy is a novel strategy for treating disease that is based on
modifying the expression of an individual's genes toward a specific
therapeutic goal.
SOURCE:
Medical Handbook
United Heath Systems
1997
++++++++++++++++
Porphyrin metabolism in the skin of protoporphyric mice has been altered
by the Soret band of radiation.
SOURCE:
Cutaneous Heme Metabolism;
Porphyrias; Photodermatoses
Dr. Henry Lim M.D.
New York University
School of Medicine
Department of Dermatology
550 First Avenue, New York, NY 10016
(212) 263-7300
++++++++++++
People with porphyria should be able to lead full active enjoyable lives with a
minimum of limitations or difficulties.
SOURCE:
The Canadian Porphyria Foundation Inc.
Neepawa, Manitoba, Canada
+++++++++++++
MORTALITY IN PORPHYRIA
In severe cases of psychosis associated with mental change in acute attacks of
porphyria there can sometimes be suicidal thoughts.
There is a high prevalence of suicide among porphyria patients.
Often during periods of psychosis in acute porphyria, emotion is exhibited in an
abnormal manner.
SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++++++
Many porphyria diagnosis's get missed and the patients suffer and died.
SOURCE:
The Porphyrias
Medicine
71: 1, 1992
+++++++++++
Although the course of porphyria is variable, mortality from these disorders today
is rare.
SOURCE:
Childhood porphyrias.
Ahmed I.
Department of Dermatology,
Mayo Clinic, Rochester, Minn
Mayo Clin Proc. 2002 Aug;77(8):825-36
+++++++++++
The main long-term complications of AIP are polyneuropathy, hepatocellular
carcinoma (HCC), and renal insufficiency.
SOURCE:
Beneficial Effect of Diabetes on
Acute Intermittent Porphyria
Folke Lithner, MD, PHD
Department of Internal Medicine
University Hospital, Umea, Sweden
++++++++++++++
Mortality in acute porphyria stem from , hepatocellular carcinoma (HCC), renal
insufficiency, respiratory paralysis, and polyneuropathy.
SOURCE:
Diabetes Care
2002; 25:797-798
+++++++++++
The death rate for acute
porphyria is highest in
the third decade.
SOURCE:
Porphyria
Dictionary of Medical Syndromes
Fourth Edition 1997
Lippincott-Raven
+++++++++
AIP is a life-threatening medical emergency. Death can occur wihou warning.
SOURCE:
Treatment of acute porphyria.
Elder GH, Hift RJ.
Department of Medical Biochemistry
University of Wales College of Medicine,
Cardiff Wales
Hosp Med. 2001 Jul;62(7):422-5.
+++++++++++
It is particular true for the acute porphyrias to have over-lapping features which
can present with life-threatening acute neurovisceral attacks that require
immediate medical intervention.
SOURCE:
Diagnosis and treatment of the acute porphyrias: an interdisciplinary challenge.
Poblete Gutierrez P, et. al.
Department of Dermatology and Allergology
University Clinic of the RWTH Aachen
Aachen, Germany
Skin, Pharmacology &
Applied Skin Physiology
2001 Nov-Dec;14(6):393-400
++++++++++++
Death usually does not occur unless the disease is not recognized.
SOURCE:
Dr. Karl E. Anderson
The Porphyrias
Hepatology: A Textbook of Liver Disease
Volume 1 Third Edition
1996 W.B. Saunders Company
+++++++++
Acute porphyria often have rapid onset of multiple and profound neurovisceral
dysfunctions that can be fatal.
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
+++++++++++
Motor weakness may progress to respiratoryparalysis and death.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
++++++++++++
Respiratory paralysis occurs in about 16%
of acute porphyria attacks.
Oxygen levels needs to be monitored and
life support administered.
Respiratory paralysis in acute porphyria has
been the leading cause of death of acute
porphyria patients."
The Porphyrias
Scientific American
April 1994
+++++++++
A few people have repeated or intermittent attacks
of symptoms separated by long intervals or remissions
between illnesses.
Unfortunately some patients can become very
sick and on rare occasions patients have died.
SOURCE:
The Canadian Porphyria Foundation Inc.
Neepawa, Manitoba, Canada
++++++++++
Death may occur when diagnosis and treatment are delayed.
SOURCE:
Dr, Robert J. Desnick
The Porphyrias
Harrison's Principles of Internal Medicine
Volume 2 13th Edition 1994
McGraw-Hill Inc.
+++++++++
Motor weakness may progress to respiratory
paralysis and death.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
+++++++++
13.3% of AIP patients die even though they have received treatment, but it had
been administered too late due to a delay in diagnosis. "
SOURCE:
Porphyric crisis: experience of 30 episodes (AIP)
Medicina
Buenas Aires
999;59(1):23-7
Morales Ortega X, et. al.
Departmento of Medicine
Occidente Hospital San Juan de Dios,
University of Chile, Santiago, Chile.
+++++++++
Sudden death due to cardiac arrhythmia may occur.
SOURCE:
Dr. Karl E. Anderson
The Porphyrias
Hepatology: A Textbook of Liver Disease
Volume 1 Third Edition
1996 W.B. Saunders Company
+++++++++
Rapid onset of multiple and profound neurovisceral
dysfunctions that can be fatal or permanently incapacitating. "
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
+++++++++++++++
Death may occur when diagnosis and treatment are delayed.
SOURCE:
Dr, Robert J. Desnick
The Porphyrias
Harrison's Principles of Internal Medicine
Volume 2 13th Edition 1994
McGraw-Hill Inc.
+++++++++++++
The porphyrias are emotionally distressful and potentially lethal disorders that
are evidenced prominently with neurologic and dermatologic expressions.
SOURCE:
NORD
++++++++++
It should be emphasised that most subjects who have inherited one of these
diseases will enjoy normal health and go through life without any knowledge of
his or her disorder or ever experiencing an acute attack.
Such, is the latent phase of the disease.
SOURCE:
Dr. Michael R. Moore
Porphyria Research Unit
University of Queensland
Department of Medicine
Brisbane, Australia 1999
+++++++++++
Onset of multiple and profound neurovisceral dysfunctions that can be fatal or
permanently incapacitating.
SOURCE:
Maureen Poh-Fitzpatrick, MD
Department of Internal Medicine
Division of Dermatology
University of Tennessee
College of Medicine
++++++++++++
Among patients with advanced neuropathy (paralytic) manifestations,
approximately 50% die.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
++++++++++
The most important thing about AIP is to diagnose the disease at the onset.
If not, many severe complications may occur.
Even a lethal outcome in AIP is possible. "
SOURCE:
Vojnosanit Pregl
2001 Jan-Feb;58(1):95-9
Acute intermittent porphyria as a problem in
differential diagnosis
Preradovic M, et. al.
++++++++++
Acute porphyrias may be life-threatening.
SOURCE:
Management of acute and cutaneous porphyrias.
nternational Journal Clinical Practrice
2002 May;56(4):272-8
Badminton MN, Elder GH.
Department of Medical Biochemistry,
University of Wales College of Medicine,
Cardiff, UK.
+++++++++
Porphyria patients do suffer and porphyria patients do die.
SOURCE:
Medicine 71: 1, 1992.
++++++++++
The porphyrias can be potentially lethal disorders.
SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++
Life-threatening respiratory paralysis can be experienced in any of the acute
porphyrias.
SOURCE:
Porphyria Resources
United Medical Services
+++++++++++
Attacks of porphyria can be fatal.
SOURCE:
Porphyria
Dictionary of Medical Syndromes
Fourth Edition 1997
Lippincott-Raven
++++++++++
Acute porphyria is a condition potentially treatable but can be fatal.
SOURCE:
Acute intermittent porphyria as a cause of acute respiratory failure.
Oomman A, Gurtoo A.
Department of Medicine,
JIPMER, Pondicherry.
Journal of Indian Medicine Association
2002 Jan;100(1):44, 46
+++++++++++
Fatal; AIP can occur with abdominal pain and constipation, followed with
subsequently developed status epilepticus, acute respiratory failure and
quadriparesis.
SOURCE:
Acute intermittent porphyria as a cause of acute respiratory failure.
Oomman A, Gurtoo A.
Department of Medicine,
JIPMER, Pondicherry.
Journal of Indian Medicine Association
2002 Jan;100(1):44, 46
+++++++++
The porphyrias are emotionally distressful and potentially lethal disorders that
are evidenced prominently with neurologic and dermatologic expressions.
SOURCE:
NORD
++++++++++
Acute intermittent porphyria (AIP) is a genetic disorder in which patients
may have life threatening attacks of neurologic dysfunction.
A study examined the prognosis during the past 50 years of patients in the
United States who required hospitalization for porphyric attacks.
The cumulative survival was determined for 136 patients with
AIP who were hospitalized for porphyric attacks between 1940 and 1988.
Diagnosis was established on the basis of clinical symptoms, in
combination with increased urinary excretion of porphobilinogen.
The patient group had an average age of 32 years (range 9 to 75) at
diagnosis and consisted of 43 males and 93 females.
At follow-up, 19 males (44%) and 31 females (33%) were decreased.
The standardized mortality ratio for the 136 patients, compared to an
age-matched hypothetical population experiencing USA 1970 Census Death
Rates was 3.2, with a 95% confidence interval of 2.4-4.0.
Most deaths occurred during the initial porphyric attack (20% of deaths)
or a subsequent attack (38% of deaths).
Suicide was also common (five deaths).
Comparison was made between 50patients who were diagnosed before
1971, the year in which hematin
therapy became available, and 86patients who were diagnosed afterward.
There was improved survival in the latter group, particularly after 10 years
from the time of diagnosis, but this did not reach statistical significance.
In conclusion, the proportionate increase in mortality due to symptomatic AIP
was three-fold compared to the general population during the past 50 years.
SOURCE:
Mortality in patients with acute intermittent porphyria requiring hospitalization:
a United States case series.
Pierach CA, Bloomer JR. et. al.
Watson Laboratory
University of Minnesota
Minneapolis, MN
++++++++++++
The effective management of the acute relapse in acute hepatic porphyrias is
clinically important in order to avoid serious consequences such as
a respiratory failure.
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++
The major cause of increased mortality is the porphyric attack itself.
SOURCE:
Mortality in patients with acute intermittent porphyria
SOURCE:
Am J Med Genet.
1996 Nov 11;65(4):269-73.
++++++++++++
The cumulative survival was determined for 136 patients with AIP who were
hospitalized for porphyric attacks between 1940 and 1988.
SOURCE:
Mortality in patients with acute intermittent porphyria requiring hospitalization: a
United States case series.
Pierach CA, Bloomer JR. et. al.
Watson Laboratory,
University of Minnesota,
Minneapolis MN
American Journal of Medical Genetics.
1996 Nov 11;65(4):269-73.
+++++++++++++++++
Diagnosis of AIP is established on the basis of clinical symptoms, in combination
with increased urinary excretion of porphobilinogen.
SOURCE:
Mortality in patients with acute intermittent porphyria requiring hospitalization: a
United States case series.
Pierach CA, Bloomer JR. et. al.
Watson Laboratory,
University of Minnesota,
Minneapolis MN
American Journal of Medical Genetics.
1996 Nov 11;65(4):269-73.
+++++++++++++++++
Most deaths from AIP occurr during the initial porphyric attack (20% of deaths)
or a subsequent attack (38% of deaths).
Suicide is also common in AIP .
SOURCE:
Mortality in patients with acute intermittent porphyria requiring hospitalization: a
United States case series.
Pierach CA, Bloomer JR. et. al.
Watson Laboratory,
University of Minnesota,
Minneapolis MN
American Journal of Medical Genetics.
1996 Nov 11;65(4):269-73.
+++++++++++++++++
The proportionate increase in mortality due to symptomatic AIP is three-fold
compared to the general population during the past 50 years.
The major cause of the increased mortality was the porphyric attack itself.
SOURCE:
Mortality in patients with acute intermittent porphyria requiring hospitalization: a
United States case series.
Pierach CA, Bloomer JR. et. al.
Watson Laboratory,
University of Minnesota,
Minneapolis MN
American Journal of Medical Genetics. 1
996 Nov 11;65(4):269-73.
+++++++++++++++++
The hereditary disorder acute intermittent porphyria is potentially fatal.
SOURCE:
Acute intermittent porphyria treated by testosterone implant.
Savage MW, et. al.
University of Manchester
Department of Medicine and Endocrinology
Hope Hospital, Salford, UK.
Postgraduate Medicine Journal
1992 Jun;68(800):479-81
+++++++++++
The hereditary disorder acute intermittent porphyria is potentially fatal.
SOURCE:
Acute intermittent porphyria treated by testosterone implant.
Savage MW, et. al.
University of Manchester
Department of Medicine and Endocrinology
Hope Hospital, Salford, UK.
Postgraduate Medicine Journal
1992 Jun;68(800):479-81
+++++++++++
The hereditary disorder acute intermittent porphyria is potentially fatal.
SOURCE:
Acute intermittent porphyria treated by testosterone implant.
Savage MW, et. al.
University of Manchester
Department of Medicine and Endocrinology
Hope Hospital, Salford, UK.
Postgraduate Medicine Journal
1992 Jun;68(800):479-81
+++++++++++
Compassion and the Art of Medicine
The humanities can play an important role in maintaining empathy and in
cultivating the compassion that students have on entry into medical school.
At Baylor College of Medicine, our department sponsors a series of lectures by
physicians, artists, and patients on the subject of "compassion and the art of
medicine."
The purpose is to encourage medical students and health care professionals to
become more compassionate and more effective health care providers by
applying the principles of medical humanism to everyday clinical practice.
We invite speakers who are able to articulate their experiences
of illness and suffering. Too often, students are exposed to patients who function
as specimens under glass, and they seldom have the opportunity to spend an
hour listening to patients reflect on the meaning of their illness and the quality of
care they have received.
Speakers include patients who describe examples of compassion or the lack
thereof during an illness.
One example is Sue Baier,[13] who wrote of her experiences with
Guillain-BarrüAae syndrome in Bed Number Ten. Baierdescribes the experience
of being ignored as a person (nursesand physicians would talk to each other as
though she were not present) and of being "handled" as an inanimate object
becauseshe was unable to move any muscles except to blink. She was
fully alert, however. Because she was paralyzed, attendants didnot realize that
her sensory system was fully intact and that she experienced considerable
discomfort from a rough venipunctureor from lying on a wrinkled bed sheet.
Another speaker, author and surgeon Richard Selzer,[14] in his
book Raising the Dead, describes his Legionnaires' disease, involving 23 days
of coma and then death. The amazing part of the story (if it can be believed) is
that 10 minutes after being pronounced dead (which occurred after his
electroencephalogram was flat for 5 minutes), he recovered.
A brain deprived of adequate oxygen that long usually suffers permanent
damage, yet his account shows his writing skills are as sharp as before the
illness.
The other focus of our series is the art of medicine.
In an effort to help students hone their "people skills," we invite writers, artists,
and performing artists to read to us from their writings, to present slides of their
visual art, or to give dramatic performances on various clinical themes.
Some are physicians and others draw from their experiences as patients, many
describing problems that make physicians uncomfortable, such as drug
dependency, alcoholism, and near death.
Originally, we focused on nationally prominent speakers to articulate these
themes. Our experience has shown, however, that the responsiveness of
students is usually determined more by the genuineness of the speakers than
by their prominence.
Speakers who draw from their own illness or experience are the ones who have
the greatest impact and potential to educate and motivate our students.
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