A GUIDE FOR PEOPLE
WITH PORPHYRIA
http://www.uq.edu.au/porphyria/PORGUIDE3.htm
Thursday, 10 October 2002
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Index
WHAT IS PORPHYRIA ? ........................................................................................................................ 1
ACUTE INTERMITTENT PORPHYRIA (AIP): ..................................................................................... 2
VARIEGATE PORPHYRIA (VP) and HEREDITARY COPROPORPHYRIA (HC): ............................. 3
PLUMBOPORPHYRIA (PP): ............................................................................................................... 3
PORPHYRIA CUTANEA TARDA (PCT): ............................................................................................. 3
ERYTHROPOIETIC PROTOPORPHYRIA (EPP): .............................................................................. 3
CONGENITAL PORPHYRIA (CP) ....................................................................................................... 3
THE ACUTE ATTACK ............................................................................................................................. 4
What is an acute attack?:..................................................................................................................... 4
What may bring on an acute attack:? .................................................................................................. 4
What can I do to avoid developing an acute attack?: .......................................................................... 4
THE SKIN IN PORPHYRIA.................................................................................................................. 5
Skin care: ......................................................................................................................................... 5
WHAT ELSE CAN BE DONE FOR PORPHYRIA? ................................................................................. 6
WHAT ABOUT MY CHILDREN? ............................................................................................................. 6
THE PORPHYRIAS: ................................................................................................................................ 7
A MEDICAL GUIDE ............................................................................................................................. 7
Features of the Acute Attack. ........................................................................................................... 7
Precipitating Factors......................................................................................................................... 8
The Drug Lists. ................................................................................................................................. 8
EVALUATION OF DRUGS FOR PORPHYRIC PATIENTS .................................................................... 9
Drug List ............................................................................................................................................. 10
1. GASTROINTESTINAL SYSTEM ................................................................................................... 10
2. CARDIOVASCULAR SYSTEM ...................................................................................................... 10
Diuretics ......................................................................................................................................... 10
Anti-hypertensive Agents ............................................................................................................... 11
Calcium Channel Blockers ............................................................................................................. 11
Anticoagulants ................................................................................................................................ 11
3. RESPIRATORY SYSTEM: ............................................................................................................ 12
Antihistamines ................................................................................................................................ 12
4. CENTRAL NERVOUS SYSTEM: .................................................................................................. 13
Hypnotics, Sedatives and Anxiolytics: ............................................................................................ 13
Tranquillisers:Anti-Emetics: ............................................................................................................ 13
Psychoanaleptics: .......................................................................................................................... 14
Anticonvulsants: ............................................................................................................................. 14
Analgesics: NON-NARCOTIC: ....................................................................................................... 14
Analgesics: NARCOTICS: .............................................................................................................. 15
Migraine: ......................................................................................................................................... 15
Appetite Suppressants: .................................................................................................................. 15
5. INFECTION: ................................................................................................................................... 16
6. ENDOCRINE SYSTEM:................................................................................................................. 16
Hormone Preparations: .................................................................................................................. 16
Anti-Diabetic Agents: ...................................................................................................................... 17
7. OBSTETRICS, GYNAECOLOGY AND RENAL DISEASE: .......................................................... 17
8. MALIGNANT DISEASE: and IMMUNOSUPPRESSION: .............................................................. 17
9. NUTRITION AND BLOOD: ............................................................................................................ 17
10. MUSCULO-SKELETAL and JOINT DISEASE: ........................................................................... 18
(a) Non-Steroidal Anti-Inflammatory Agents: ................................................................................. 18
(b) Corticosteroids: ......................................................................................................................... 18
(c) Specific Anti-Rheumatic Agents: ............................................................................................... 18
(d) Anti-Gout Agents:...................................................................................................................... 18
(e) Muscle Relaxants and Anti-Spasmodics: ................................................................................. 19
11. THE EYE: ..................................................................................................................................... 19
12. THE EAR, NOSE AND OROPHARYNX: ..................................................................................... 19
13. THE SKIN: ................................................................................................................................... 19
14. VACCINES - NONE PROVEN UNSAFE ..................................................................................... 19
15. ANAESTHESIA: ........................................................................................................................... 20
ACUTE PORPHYRIA -DIAGNOSIS: ..................................................................................................... 21
Screening of Families ........................................................................................................................ 21
A Preliminary Test for the Presence of Porphobilinogen in the Urine ............................................... 21
MANAGEMENT OF THE ACUTE ATTACK: ......................................................................................... 21
Symptomatic Therapy ........................................................................................................................ 22
Pain: ............................................................................................................................................... 22
Nausea, Vomiting and Constipation: .............................................................................................. 22
Tachycardia and Hypertension: ..................................................................................................... 22
Convulsions: ................................................................................................................................... 23
Neuropathy: .................................................................................................................................... 23
Fluid and Electrolyte Balance: ........................................................................................................ 23
SPECIFIC THERAPY OF THE ACUTE ATTACK: SPECIFIC THERAPY ......................................... 24
Haematin Therapy: ( Haem Arginate ) ........................................................................................... 24
PREVENTION OF ATTACKS: ........................................................................................................... 24
Menstruation: .................................................................................................................................. 25
Pregnancy and Acute Porphyria: ................................................................................................... 25
Anaesthetics: .................................................................................................................................. 25
Photosensitivity: ............................................................................................................................. 26
Prophylaxis and Treatment of Malaria. .......................................................................................... 26
DRUG TREATMENTS IN ACUTE PORPHYRIA .................................................................................. 28
TESTING FOR PORPHYRIA ................................................................................................................ 30
FURTHER READING ............................................................................................................................ 30
APPENDIX I – ALPHABETICALLY DRUG LISTING ............................................................................ 31
A GUIDE FOR PEOPLE WITH PORPHYRIA
WHAT IS PORPHYRIA ?
Porphyria is a fairly uncommon condition. It is not one condition, but a group of several related
diseases. Most of these are inherited but some may be acquired. People with porphyria may develop
skin problems or a condition known as the acute attack.
In all the porphyria, the basic dilemma is that excessive amounts of porphyrins and their precursors
accumulate in the body. It is under-diagnosed. Many sufferers are completely asymptomatic. All living
things, including healthy people produce porphyrins. In porphyria, there is an atypical accumulation of
porphyrins as the result of enzyme defects; this results in illness.
Our bodies convert two simple substances, 5-aminolaevulinate (ALA) and porphobilinogen (PBG)
known as porphyrin precursors, into more complicated substances called porphyrins. These are then
converted from one type of porphyrin to the next to form haem, aka heme. Haem is a vital substance
in our bodies.
Protoporphyrin together with Iron are the building blocks necessary to make haem. Each step on the
pathway is completed by a special protein known as an enzyme. In each type of porphyria, a specific
enzyme is deficient, and this is why porphyrins accumulate.
As shown, each of the eight types of porphyria is associated with a deficiency of one of these
enzymes.
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ACUTE INTERMITTENT PORPHYRIA (AIP):
AIP is an inherited condition. It is passed on from generation to generation. This means that it may be
passed on from parents to their children.
This occurs so that half of an affected parent's children are likely to be affected. For example, if you
have six children, the chances are that three of them will have porphyria too. Boys and girls stand an
equal chance of being affected.
Once the condition has entered a family, there is nothing that anyone can do about it. You cannot be
blamed for having porphyria or for passing it on to your children! People with AIP are at risk of
developing the acute attack, and you should now read the section The Acute Attack in this booklet.
If you have AIP, your skin will not be affected (unlike the other forms of porphyria) so the section The
Skin in Porphyria is not relevant to you.
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VARIEGATE PORPHYRIA (VP) and HEREDITARY COPROPORPHYRIA (HC):
Like AIP, VP and HC are always inherited conditions. People with VP and HC are prone to skin
problems, and to the acute attack. You should now read the following sections in this booklet: The
Skin in Porphyria and The Acute Attack.
PLUMBOPORPHYRIA (PP):
PP is an extremely rare condition and in the few cases described is similar to AIP.
PORPHYRIA CUTANEA TARDA (PCT):
Very rarely, Porphyria Cutanea Tarda (PCT) is inherited from one's parents in the manner described
for AIP and VP.
Most people with PCT did not inherit the disorder and will not pass it on to their children. Here, PCT is
secondary to another condition. It may arise in some people because of taking too much alcohol.
Commonly, such people have additionally an excess of iron in their bodies. It may also follow the use
of some drugs, such as the oestrogen used in the contraceptive pill or for relief of symptoms of the
menopause and after exposure to certain chemicals.
It may also develop in people with kidney failure treated with haemodialysis. People with PCT
commonly develop skin problems, and should now read the section in this booklet The Skin in
Porphyria. They will never suffer an acute attack. Drugs do not hold the same danger for them as they
do for people with AIP or VP, (though they should avoid alcohol).
ERYTHROPOIETIC PROTOPORPHYRIA (EPP):
This is a less common form of porphyria. Like AIP and VP, it is an inherited disorder that is passed on
from parents to their children. People with EPP suffer skin problems, and you should read the section
The Skin in Porphyria. As in PCT, people with EPP do not develop acute attacks nor are drugs of
concern. However the liver may become involved in later years.
CONGENITAL PORPHYRIA (CP)
This is the rarest of the porphyria. It is primarily a skin condition and uniquely is inherited as a
recessive condition. Both parents are asymptomatic carriers.
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THE ACUTE ATTACK
People with AIP HC or VP are always at risk of an acute attack of porphyria. This may be very
dangerous, and they should read this section carefully and make sure they understand how they can
prevent such an attack. Those with PCT, EPP and CP are not at risk, and this section does not apply
to them.
What is an acute attack?:
The acute attack takes place when the levels of the porphyrin precursors become very much raised for
one or other reason. One can think of this as an overloading of the body with porphyrins and their
precursors.
During such an attack, the affected person may experience abdominal pain, cramps, constipation,
nausea or vomiting. They may also show marked anxiety or disturbed behaviour. Such attacks can be
bad enough to require admission to hospital, and the most severe cases may go on to weakness and
paralysis.
People have even died of such an attack. Fortunately, a fatal outcome has become rare as modern
hospitals now have the facilities to treat such complications. This emphasises the need for people
experiencing an acute attack to be admitted to an experienced hospital. It is more common nowadays
for people with AIP or VP to develop milder forms of the acute attack with not much more than a
feeling of being unwell, some pain in the stomach and, perhaps, nausea.
If you are experiencing such problems, it is important that you immediately stop any medication you
may be taking and consult your doctor. Yet everyone has some of these symptoms at one time or
another and you cannot blame everything on your porphyria!
What may bring on an acute attack:?
Acute attacks may follow the use of many drugs. Porphyric people are unable to handle these drugs in
the normal way and their bodies respond to them by overproducing porphyrins. This is the commonest
cause of the acute attack.
However, attacks can also be precipitated by alcohol, by an infection and even by dieting. Smoking
has been shown to worsen attacks.
What can I do to avoid developing an acute attack?:
You must understand that there are many medicines that can aggravate your porphyria, possibly
resulting in an acute attack. Therefore, you must never take any medicine or remedy without checking
that it is safe for porphyrics. This includes drugs given to you by a doctor, pharmacist or dentist, as
well as those you can buy without prescription.
Always consult our list (which you will find in this booklet) before you take the medicine given to you.
Note that this includes tonics, herbal remedies and even the contraceptive pill, which has been a
major factor in the development of acute attacks.
If you ever need an operation, you must tell the surgeon and anaesthetist that you have porphyria, as
some anaesthetic drugs in common use are very dangerous for porphyrics. Safer alternatives can be
used. It is desirable to wear a Medic-Alert disc or carry a similar form of identification, so that doctors
will know you have porphyria in the event of an accident.
Finally, it is wise to eat regular meals and not to go without food for long periods, or to embark on
'crash' diets. Other than this, there is no special diet that needs to be followed. If you wish to lose
weight, discuss your diet with your doctor beforehand. It is also best to avoid alcoholic drinks and to
stop (or never start) smoking.
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THE SKIN IN PORPHYRIA
People with VP, HC, CP and PCT may have a sensitive skin. They complain that it is easily damaged
and that even the slightest knock can cause the skin to break.
Often these damaged areas take a long time to heal. Sunlight is necessary to cause the skin to
become fragile and people with porphyria find that the only parts of their bodies that are affected are
those that are exposed to light, particularly their hands, faces, necks, legs and feet.
They develop blisters and open sores. In time their skins become thin, dark, scarred and often rather
hairy, particularly on the face.
EPP and CP are somewhat different to the other porphyria. Firstly, the skin may already be affected
as a very young child or even in infancy, whereas the other porphyria usually only become obvious
later in life. Secondly, such people often find that they react to sunlight rapidly, developing a sensation
of burning or stinging shortly after going into the sun. This is unlike VP, HC and PCT, where the
damage takes much longer to develop. Affected people learn to avoid too much light because of this
discomfort.
Skin care:
If you have skin problems because of porphyria you should look after your skin as follows:1. Avoid sunlight as much as possible. This means remaining indoors during the sunniest part of the
day, and only going out in the early morning or late afternoon. Unfortunately, sunbathing is OUT if
you have skin problems. This is definitely the worst thing a porphyric with skin problems can do.
If you have a job that requires you to be in the sun a lot, you may have to consider changing your
occupation. Protect sun-exposed areas by wearing long sleeves, gloves and a hat whenever you
go out. These should be made of cotton, which screens out the sun better than nylon. No cream or
medication is as effective in protecting your skin.
The only sunscreen creams that can help are the opaque zinc or titanium oxide creams, which are
unfortunately thick and greasy. Ask your pharmacist for such a cream.
Note that the usual suntan lotions will not protect your skin. Though they prevent sunburn in normal
people, they do not keep out the light which damages a porphyric's skin.
2. Protect your skin from injury: Use a silicone barrier cream and rubber gloves to protect your hands
when washing clothes or dishes. Try not to knock your hands; a porphyric's skin is fragile and
easily broken. For instance, wear heavy leather workman's gloves when working on your motor
car.
When your skin is blistered or broken, avoid scratching and keep it clean with water and a mild
soap. Avoid strong antiseptics. You can avoid permanent scarring if you look after such sores
properly.
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WHAT ELSE CAN BE DONE FOR PORPHYRIA?
Unfortunately, there now is no cure for porphyria. Still, there is a lot that you and your doctor can do to
make it less severe. With a little care, your symptoms probably will be mild and you can live a normal
life, as many porphyrics do.
First, you must get an accurate diagnosis so that you can be absolutely certain you have porphyria
and if so, what type. Speak to your doctor in this regard. Patients with PCT can be helped by avoiding
alcohol or any other known cause of the condition.
If the skin remains bad, relief can be obtained by venesection. This means having 500 ml. of blood
removed at regular intervals - usually fortnightly - for about eight weeks. Your doctor will arrange this if
necessary. People with EPP also must ensure that they have regular medical check-ups as their
condition can eventually affect the liver. Regular examinations and blood tests will detect this at an
early stage.
WHAT ABOUT MY CHILDREN?
If you have PCT, your children will almost certainly be free of porphyria. If you have AIP, HC, VP or
EPP, each child has a 50:50 chance of being affected. If they are affected, this is not a terrible thing!
They will probably not be any more seriously affected than you - they may be milder. In fact, more
than 50% of porphyrics do not show signs of the condition at all, though they may be positive on
testing - these are known as latent cases.
With sensible precautions, your children can live to a normal age, marry and have children
themselves. Young children tend not to show signs of their porphyria till after puberty - that is, till after
they reach sexual maturity. Laboratory tests usually do not even pick it up till then.
An acute attack has developed on only very rare occasions in childhood - even before the tests are
positive. Therefore, it is wise to make your children take all the same precautions you do, to minimise
this risk.
DON'T ALLOW THEM TO HAVE ANY MEDICINES THAT ARE NOT SAFE IN PORPHYRIA AND
DO WARN THE DOCTORS BEFORE THEY HAVE ANY ANAESTHETICS.
We suggest having your children tested every two years from the age of 12 until they turn 20 years. If
the tests are still negative, then they will very likely be free of symptoms of porphyria after that.
Unfortunately, a few people are silent cases - though their tests are negative, they carry the defective
gene, and porphyria can be precipitated in them if they are exposed to the 'dangerous' medicines on
our list.
Therefore, the wisest suggestion is that no member of a porphyric family should take any such drug
unless essential. It must be emphasised that it is essential to have your children adequately tested in
a specialist laboratory.
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THE PORPHYRIAS:
A MEDICAL GUIDE
The Porphyrias are a heterogeneous group of either inherited or acquired disorders of haem
biosynthesis. In these diseases, specific abnormalities of enzymes in the biosynthetic pathway cause
generalised clinical abnormalities. They are classified, as shown below, into acute and non-acute
porphyria.
ACUTE
PORPHYRIA
NON-ACUTE
PORPHYRIA
ACUTE INTERMITTENT
PORPHYRIA
(Swedish Porphyria)
VARIEGATE PORPHYRIA
(South African Genetic Porphyria)
HEREDITARY
COPROPORPHYRIA
(Coproporphyria)
PLUMBOPORPHYRIA
(ALA Dehydratase deficiency)
PORPHYRIA CUTANEA TARDA
(Cutaneous Hepatic Porphyria:
Symptomatic Porphyria)
ERYTHROPOIETIC
PROTOPORPHYRIA
(Erythrohepatic Porphyria)
CONGENITAL PORPHYRIA
(Gunther's disease:
Erythropoietic Porphyria)
The effects of drugs are most important in the acute porphyria, which are examples of 'Toxico-genetic
diseases'. Patients with the acute form of these disorders are at risk of developing life-threatening
attacks of porphyria on exposure to certain commonly prescribed drugs.
'Toxico-genetic diseases' - are diseases, genetically acquired, which show an idiosyncratic reaction to
drugs. All the acute porphyria are inherited as mendelian autosomal dominants, and each may be
linked to lowered activity of one of the enzymes of the haem biosynthetic pathway: in Acute
Intermittent Porphyria – a decrease in porphobilinogen deaminase: in Variegate Porphyria - a
decrease in protoporphyrinogen oxidase, and in Hereditary Coproporphyria – a decrease in
coproporphyrinogen oxidase.
Features of the Acute Attack.
Attacks of Acute porphyria vary in their clinical presentation. Severe abdominal pain, vomiting and
constipation, with tachycardia and hypertension, are the commonest presenting features. Peripheral
neuropathy may develop and lead to fatal respiratory paralysis.
Tachycardia and hypertension are usually present, and hypertensive encephalopathy may develop.
Besides hypertension, severe postural hypotension, resulting in syncope may occur. Hypertension
may persist to some extent between attacks. Other manifestations of autonomic dysfunction, such as
profuse sweating, pallor and pyrexia may also occur.
Severe hyponatraemia, due to inappropriate secretion of antidiuretic hormone, complicates some
attacks and sometimes presents as convulsions or deterioration in the conscious level. Another
feature of involvement of the Central Nervous System is mental disturbance including agitation, mania,
depression, auditory and visual hallucinations, and schizophrenic-like behaviour.
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Grand-mal convulsions are not uncommon at the height of an attack and may persist between attacks.
In Variegate Porphyria, and in Hereditary Coproporphyria, there may also be skin involvement, with
development of solar photosensitivity.
Precipitating Factors
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.
All porphyrics, however, are at risk of developing an attack if exposed to various precipitating factors.
Drugs are the most common precipitating agents. Other factors that may trigger attacks, include
alcohol ingestion, reduced caloric intake, due to fasting or dieting, and infection. We have also noted
that smoking can cause more frequent attacks.
Hormones are also important. Attacks are more common in females and, rarely, occur before puberty
or after the menopause. Pregnancy and oral contraceptives may also precipitate attacks. Some
women experience regular attacks, commencing in the week prior to the onset of menstruation.
Although most of the drugs incriminated as porphyrinogenic are lipophilic and inducers of the hepatic
mixed function oxidase system, it is impossible to reliably predict from chemical structure whether a
drug will be safe for use in the porphyric patient.
The Drug Lists.
Lists in Table 1 (A) and (B) give information, alphabetically, on all drugs about which some information
is known.
It should be borne in mind that such Lists are far from encyclopaedic, that new drugs are constantly
being introduced to the pharmacopoeia, and that any form of combined preparation
must be viewed with suspicion, since little is known about metabolic interactions in these diseases.
The ultimate aim of investigative programs in the porphyria is to identify as many as possible of the
cases latent for these diseases, using sensitive enzyme-screening tests, and by a program of
education, especially on drug usage, transform these potentially fatal conditions to mere genetic
curiosities.
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EVALUATION OF DRUGS FOR PORPHYRIC PATIENTS
This Table is organised under the Therapeutic Headings given in The British National Formulary. The
drugs are categorised, as follows:
A. Those in the 'unsafe group' which have been
reported by three or more workers in the field
to be associated with clinical exacerbations of
porphyria or, in the 'safe group', those
considered by three or more authorities to be
harmless to porphyric patients on the basis of
their clinical experience.
B. Those in the 'unsafe group' which have been
reported by two or less workers in the field to
be associated with clinical exacerbations of
porphyria or, in the 'safe group', those
considered by two or fewer authorities to be
harmless to porphyric patients on the basis of
their clinical experience.
In many instances, the clinical experience with drugs in the above two groups will have been
corroborated by experimental data. There are two further headings under which the drugs are listed:
C. Those which have been evaluated only in
animals, with experimentally-produced
porphyria.
D. Those which have been evaluated only in cell
culture systems
No clinical experience has been reported with the drugs in these latter two groups.
Finally, those drugs for which conflicting data is available have been listed and classified where
possible as:
S. Probably safe.
U. Probably unsafe.
NB. While very great care has been taken in the compilation of this table and the drug information is
given in the belief that it is correct at the time of publication, all information contained herein and
opinions expressed must be taken as information and opinions given for general guidance only.
The authors hereby disclaim for themselves, The Porphyria Charitable Trust, the Porphyrias
Service, the University of Queensland and Queensland Health, all responsibility for any misstatement or for the consequences to any person of any person acting in reliance on any
statement or opinion contained herein.
Medical Practitioners and patients must make their own decisions in the circumstances of the
particular case about therapy appropriate in any case of acute porphyria.
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Drug List
1. GASTROINTESTINAL SYSTEM
UNSAFE
Aluminium OH
Hyoscine Butylbromide
Mebeverine HCl
Sulphasalazine
C
A
D
C
THOUGHT TO BE SAFE
Atropine
Cisapride
Danthron
Dicyclomine HCl
Diphenoxylate HCl
Domperidone
Famotidine
Liquorice
Loperamide
Magnesium Sulphate
Metopimazine
Pirenzepine
Propantheline
Senna
Sorbitol
Trimebutine Maleate
A
B
A
B
B
C
B
B
D
B
D
B
B
A
A
D
CONTENTIOUS
Cimetidine
Metoclopramide
Omeprazole
Ranitidine
U
U
S
U
CONTENTIOUS
Disopyramide
Fenofibrate
Prazosin
S
SD
S
CONTENTIOUS
Bendrofluazide
Chlorothiazide
Cyclopenthiazide
Mersalyl
Trichlormethiazide
S
S
U
S
S
2. CARDIOVASCULAR SYSTEM
UNSAFE
Amiodarone HCl
Ethamsylate
Oxypentifylline
Simvastatin
D
C
D
C
THOUGHT TO BE SAFE
Adrenaline
Aminocaproic Acid
Atropine
Buflomedil HCl
Clofibrate
Digoxin
Dipyridamole
Glyceryl Trinitrate
Heptaminol HCl
Naftidrofuryl oxalate
Probucol
Procainamide HCl
Quinidine
Tranexamic Acid
Trimetazidine HCl
B
B
A
D
D
A
D
B
D
D
D
D
C
B
D
THOUGHT TO BE SAFE
Acetazolamide
Amiloride
Bumetanide
Ethacrynic Acid
Tienilic Acid
D
B
B
B
D
Diuretics
UNSAFE
Frusemide
Hydrochlorothiazide
Spironolactone
A
B
C
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Anti-hypertensive Agents
UNSAFE
Alpha Methyl DOPA
Captopril
Clonidine
Enalapril
Hydralazine
Lisinopril
Phenoxybenzamine
A
D
D
B
B
B
D
THOUGHT TO BE SAFE
Atenolol
Diazoxide
Guanethidine
Guanfacine HCl
Labetalol
Mecamylamine
Metipropanolol
Metoprolol Tartrate
Propanolol
Reserpine
Timolol Maleate
Tolazoline
CONTENTIOUS
A
D
A
D
B
C
B
B
A
A
D
A
Calcium Channel Blockers
UNSAFE
Bepridil HCl
Nifedipine
Prenylamine
Verapamil
THOUGHT TO BE SAFE
CONTENTIOUS
Diltiazem
D
B
D
B
U
Anticoagulants
UNSAFE
THOUGHT TO BE SAFE
Heparin
Warfarin Na
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CONTENTIOUS
B
B
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3. RESPIRATORY SYSTEM:
UNSAFE
Allyloxy 3-methylbenzamide
Astemizole
Bemegride
Guaiphenesin
Nikethamide
Pentylenetetrazol
Theophylline
C
D
B
C
A
B
B
THOUGHT TO BE SAFE
Beclomethasone dipropionate
Ketotifen
Mequitazine
Pseudoephedrine HCl
Salbutamol
B
D
B
B
B
CONTENTIOUS
THOUGHT TO BE SAFE
Chlorpheniramine
Trimeprazine tartrate
Tripelennamine
A
D
B
Antihistamines
UNSAFE
Clemastine
Dimenhydrinate
Diphenhydramine
Flunarizine HCl
Terfenadine
B
B
B
D
B
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CONTENTIOUS
Promethazine
U
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4. CENTRAL NERVOUS SYSTEM:
Hypnotics, Sedatives and Anxiolytics:
UNSAFE
Alprazolam
Amylobarbitone
Apronalide
Carbromal
Carisoprodol
Chlordiazepoxide
Chlormezanone
Clotiazepam
Diazepam
Dichloralphenazone
Ethchlorvynol
Ethinamate
Flunitrazepam
Flurazepam
Glutethimide
Hexapropymate
Loprazolam
Loxapine
Meprobamate
Methyprylone
Nitrazepam
Prazepam
Quinalbarbitone
Sulpiride
Sultopride
Tetrazepam
Thioridazine
D
A
B
B
A
B
B
D
A
A
B
C
B
A
A
D
D
D
A
A
B
D
B
C
D
D
B
THOUGHT TO BE SAFE
Benzhexol HCl
Chloral hydrate
Chlorpromazine
Droperidol
Lofepramine
Methylphenidate
Pericyazine
Piracetam
Prochlorperazine
Promazine
Temazepam
Triazolam
Trifluoperazine
D
A
A
B
B
B
D
D
B
A
B
C
B
THOUGHT TO BE SAFE
Chlorpromazine
Cyclizine
Domperidone
Meclozine
Prochlorperazine
A
B
C
A
B
CONTENTIOUS
Bromazepam
Chlormethiazole
Clobazam
Clonazepam
Clorazepate
Estazolam
Haloperidol
Lorazepam
Methotrimeprazine
Midazolam
Oxazepam
Promethazine
S
S
U
U
U
DS
U
S
S
DS
U
U
CONTENTIOUS
Metoclopramide
Promethazine
U
U
Tranquillisers:Anti-Emetics:
UNSAFE
Betahistine HCl
Cinnarizine
Dimenhydrinate
Dixyrazine
Hydroxyzine
Isometheptene-mucate
D
C
B
C
B
C
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 13
Psychoanaleptics:
UNSAFE
Amineptine HCl
Dothiepin HCl
Flupenthixol
Iproniazid
Maprotiline HCl
Mianserin HCl
Pargyline
Phenelzine
Tranylcypromine
Trazodone HCl
Veralipride
Viloxazine HCl
Zuclopenthixol
D
C
C
B
C
C
C
C
C
C
D
C
C
THOUGHT TO BE SAFE
Fluoxetine HCl
Fluvoxamine
maleate
Lithium salts
Lofepramine
Methylphenidate
Minaprine HCl
Pipothiazine
palmitate
B
B
B
B
D
D
CONTENTIOUS
Amitriptyline
Carpipramine
Clomipramine HCl
Imipramine
Metapramine HCl
Nortriptyline
Trimipramine
U
U
DU
U
DU
U
U
CONTENTIOUS
Chlormethiazole
Clonazepam
Na Valproate
Valpromide
S
U
U
DU
CONTENTIOUS
Indomethacin
Mefenamic acid
Nicergoline
S
U
DU
D
Anticonvulsants:
UNSAFE
Barbiturates
Carbamazepine
Ethosuximide
Diazepam
Ethotoin
Hydantoins
Methsuximide
Oxazolidinediones
Paramethadione
Phenobarbitone
Phensuximide
Phenytoin
Primidone
Progabide
Succinimides
Sulthiame
Troxidone
A
A
A
A
B
A
A
B
A
A
A
A
A
D
A
B
B
THOUGHT TO BE SAFE
Bromides
Magnesium-sulphate
Paraldehyde
A
B
B
Analgesics: NON-NARCOTIC:
UNSAFE
Amidopyrine
Antipyrine
Floctafenine
C
C
D
THOUGHT TO BE SAFE
Aspirin
Co-codamol
Codeine
Diflunisal
Fenoprofen
Glafenine
Ibuprofen
Naproxen
Nefopam HCl
Paracetamol
A GUIDE FOR PEOPLE WITH PORPHYRIA
A
B
B
B
C
C
B
B
D
A
(V1.3)
Page 14
Analgesics: NARCOTICS:
UNSAFE
Dextropropoxyphene
Oxycodone
Pentazocine
Phenacetin
Tilidate
B
C
A
C
B
THOUGHT TO BE SAFE
Buprenorphine
Diamorphine
Droperidol
Meptazinol
Methadone
Morphine
Pethidine
B
B
A
B
A
A
A
THOUGHT TO BE SAFE
Pizotifen
Prochlorperazine
B
B
THOUGHT TO BE SAFE
Methylcellulose
B
CONTENTIOUS
Dextromoramide
S
Migraine:
UNSAFE
Clonidine HCl
Dihydroergotamine-Mesylate
Ergotamine Tartrate
Isometheptene Mucate
Lysuride Maleate
Orphenadrine
B
A
A
B
C
C
CONTENTIOUS
Appetite Suppressants:
UNSAFE
Fenfluramine
Dexfenfluramine
Diethylpropion
Methamphetamine
C
D
C
B
A GUIDE FOR PEOPLE WITH PORPHYRIA
CONTENTIOUS
(V1.3)
Page 15
5. INFECTION:
UNSAFE
Colistin
Chloramphenicol
Co-trimoxazole
Cycloserine
Dapsone
Econazole Nitrate
Erythromycin
Flucloxacillin
Flumequine
Griseofulvin
Ketoconazole
Miconazole
Nalidixic acid
Natamycin
Novobiocin
Pipemidic acid
Pivampicillin
Pyrazinamide
Rifampicin
Sulphonamides
Tinidazole
Trimethoprim
Vibramycin
B
A
B
B
A
B
A
A
D
A
D
D
B
B
B
D
B
A
B
A
D
B
B
THOUGHT TO BE SAFE
Acyclovir
Aminoglycosides
Amoxycillin
Amphotericin D
Ampicillin
Ciprofloxacin
Clavulanic Acid
Flucytosine
Gentamycin
Hexamine
Josamycin
Mefloquine HCl
Minocycline HCl
Netilmycin
Norfloxacin
Ofloxacin
Oxolinic acid
Pefloxacin
Penicillin
Primaquine
Quinine
Sodium Fusidate
Streptomycin
Talampicillin
Ticarcillin
Vancomycin
Zidovudine
D
B
B
D
A
B
D
D
C
C
D
D
D
D
D
D
D
D
A
B
B
B
A
B
B
B
B
CONTENTIOUS
Cephalosporins
Chloroquine
Isoniazid
Mebendazole
Metronidazole
Nitrofurantoin
Pyrimethamine
Tetracyclines
U
S
U
S
S
U
S
S
CONTENTIOUS
Androgens
Benzylthiouracil
Corticosteroids
Cyproterone Acetate
Ethinyl Oestradiol
Nandrolone
Prednisolone
Progestogens
U
DS
S
S
S
U
S
U
6. ENDOCRINE SYSTEM:
Hormone Preparations:
UNSAFE
Bromocriptine
Danazol
Dydrogesterone
Metyrapone
Oral Contraceptives
Stanozolol
D
B
C
D
A
C
THOUGHT TO BE SAFE
Buserelin
Carbimazole
Clomiphene Citrate
Corticotrophin (ACTH)
Dexamethasone
Follicle Stimulating Hormone
Glucagon
Goserelin
Methyluracil
Propylthiouracil
Thiouracil
Thyroxine
A GUIDE FOR PEOPLE WITH PORPHYRIA
A
B
B
B
B
B
D
B
B
B
B
B
(V1.3)
Page 16
Anti-Diabetic Agents:
UNSAFE
Sulphonylureas
Glipizide
A
B
THOUGHT TO BE SAFE
Biguanides
Insulin
Metformin
Phenformin
CONTENTIOUS
C
A
C
C
7. OBSTETRICS, GYNAECOLOGY AND RENAL DISEASE:
UNSAFE
Ergometrine Maleate
Hyoscine Butyl Bromide
Oral Contraceptives
A
A
A
THOUGHT TO BE SAFE
Dinoprost
Oxytocin
Propantheline
C
B
B
CONTENTIOUS
Mifepristone
Oestrogens
U
U
CONTENTIOUS
Cyproterone-Acetate
Ethinyl Oestradiol
Melphalan
Vinblastine
Vincristine
S
S
S
UD
UD
8. MALIGNANT DISEASE: and IMMUNOSUPPRESSION:
UNSAFE
Aminoglutethimide
Busulphan
Chlorambucil
Cyclophosphamide
Cyclosporin
Megestrol
Mercaptopurine
Methotrexate
Tamoxifen
B
C
C
C
C
C
B
C
B
THOUGHT TO BE SAFE
Actinomycin D
Azathioprine
Cisplatin
Doxorubicin HCl
Etoposide
Zidovudine
C
C
D
C
B
B
THOUGHT TO BE SAFE
Alpha tocopheryl acetate
Ascorbic Acid
Vitamins
Folic Acid
Fructose
Glucose
Haem Arginate
Iron Preparations
Pyridoxine HCl
Sodium Calcium Edetate
(EDTA)
Sulbutiamine
A
A
B
B
A
A
A
A
A
A
A
D
9. NUTRITION AND BLOOD:
UNSAFE
Ethanol
A
A GUIDE FOR PEOPLE WITH PORPHYRIA
CONTENTIOUS
(V1.3)
Page 17
10. MUSCULO-SKELETAL and JOINT DISEASE:
(a) Non-Steroidal Anti-Inflammatory Agents:
UNSAFE
Amidopyrine
Azapropazone
Benoxaprofen
Clometacin
Dichloralphenazone
Diclofenac Na
Dipyrone
Flufenamic Acid
Oxyphenbutazone
Pipebuzone
Piroxicam
Propyphenazone
A
C
C
D
A
A
A
B
A
D
D
A
THOUGHT TO BE SAFE
Alclofenac
Aspitin
Codeine PO4
Dihydrocodeine
Fenoprofen
Flurbiprofen
Ibuprofen
Ketoprofen
Naproxen Na
Nifumic Acid
Paracetamol
Sulindac
Tiaprofenic acid
C
A
A
A
C
B
B
C
C
D
B
C
B
CONTENTIOUS
Indomethacin
Mefenamic acid
Phenylbutazone
S
U
U
CONTENTIOUS
Hydrocortisone
Prednisolone
S
S
CONTENTIOUS
Chloroquine
S
CONTENTIOUS
Probenecid
U
(b) Corticosteroids:
UNSAFE
THOUGHT TO BE SAFE
Dexamethasone
B
(c) Specific Anti-Rheumatic Agents:
UNSAFE
Auranofin
Gold-(Sodium
Aurothiomalate)
B
THOUGHT TO BE SAFE
Penicillamine
B
THOUGHT TO BE SAFE
Allopurinol
Colchicine
C
B
C
(d) Anti-Gout Agents:
UNSAFE
Benzbromarone
Piroxicam
Sulphinpyrazone
D
B
D
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 18
(e) Muscle Relaxants and Anti-Spasmodics:
UNSAFE
Baclofen
Carisoprodol
Chlormezanone
Chlorzoxazone
Diazepam
Dipyrone
Drotaverine
Hyoscine Butylbromide
Mephenesin
Orphenadrine
Oxanamide
D
A
B
B
A
A
A
A
D
B
C
THOUGHT TO BE SAFE
Domperidone
Neostigmine
Parapenzolate Br
Propantheline Br
Suxamethonium
Tubocurarine
C
A
D
B
A
B
THOUGHT TO BE SAFE
Acetazolamide
Amethocaine HCl
Atropine
Dexamethasone
Gentamicin
Guanethidine
Oxybuprocaine
Timolol Maleate
Zinc sulphate
B
C
A
B
A
C
C
D
A
CONTENTIOUS
Metoclopramide
Pancuronium
U
S
CONTENTIOUS
Prednisolone
Proxymetacaine
Tetracyclines
S
S
S
11. THE EYE:
UNSAFE
Chloramphenicol
Cocaine
Hyoscine Butylbromide
Mercuric oxide
Oxyphenbutazone
Sulphacetamide
A
B
A
B
B
C
12. THE EAR, NOSE AND OROPHARYNX:
UNSAFE
Oxymetazoline
THOUGHT TO BE SAFE
CONTENTIOUS
B
13. THE SKIN:
UNSAFE
Crystal violet
Econazole nitrate
Griseofulvin
Ketoconazole
Miconazole
B
B
A
D
D
THOUGHT TO BE SAFE
Canthaxanthin
ß Carotene
Resorcinol
Zinc Preparations (Topical)
A
A
B
B
CONTENTIOUS
Hydrocortisone
Butyrate
S
Note - in sections 11, 12 and 13, substances that are neither absorbed nor metabolised are unlikely to
be harmful.
14. VACCINES - NONE PROVEN UNSAFE
UNSAFE
THOUGHT TO BE SAFE
A GUIDE FOR PEOPLE WITH PORPHYRIA
CONTENTIOUS
(V1.3)
Page 19
15. ANAESTHESIA:
UNSAFE
Alcuronium
Alphaxalone -Alphadolone
Barbiturates
Carticaine HCl
Chloroform
Cocaine
Enflurane
Etidocaine
Etomidate
Fluroxene
Isoflurane
Lignocaine
Mepivacaine
Methohexitone
Prilocaine
Thiopentone Na
C
B
A
D
B
B
C
C
C
B
C
C
D
C
B
A
THOUGHT TO BE SAFE
Amethocaine HCl
Bupivacaine
Butacaine SO4
Cyclopropane
Diethyl ether
Droperidol
Fentanyl
Flumazenil
Meptazinol
Midazolam
Minaxolone
Nitrous oxide
Propofol
Procaine
Suxamethonium
Tetracaine
Tubocurarine
A GUIDE FOR PEOPLE WITH PORPHYRIA
C
C
D
C
A
C
C
D
B
D
C
A
B
B
C
C
C
(V1.3)
CONTENTIOUS
Haloperidol
Halothane
Ketamine
Pancuronum Br
Propanidid
Proxymetacaine
U
U
S
S
U
S
Page 20
ACUTE PORPHYRIA -DIAGNOSIS:
Acute porphyria should be considered in any patient presenting with unexplained abdominal pain,
mental dysfunction or peripheral neuropathy. A further clue to the diagnosis is discolouration of the
urine.
During an attack, the urine is a dark reddish brown and this becomes more pronounced if it is left
standing. A simple bedside test can confirm the diagnosis. Quantitative studies of the different
porphyrins and precursors in the urine and faeces should be performed later by a specialist laboratory
to identify the particular type of acute porphyria.
Successful treatment of an acute attack of Porphyria depends largely on, early diagnosis, removal of
precipitating factors, and provision of intensive supportive therapy. On first diagnosing an attack, a
careful search should be made for any precipitating factors, and if possible, these should be removed.
The patient's current drug therapy should be scrutinised and a search made for any underlying
infection. When appropriate, a pregnancy test should be performed.
Screening of Families
Latent cases in affected families may be diagnosed, either by measurement of porphyrins and their
precursors in urine, faeces and blood, or by measurement of the activities of the enzymes of the Haem
biosynthetic pathway.
Where such screening is required, most Analytical Laboratories, such as our own, prefer to receive
samples of urine, stool and heparinized blood. Prophylaxis is extremely important. In particular, Drugs
listed in the 'Unsafe Groupings' in Tables 1 and 2, should be avoided.
Patients should also be counselled on the dangers of alcohol, smoking and dieting.
A Preliminary Test for the Presence of Porphobilinogen in the Urine
Equal volumes of Urine and Ehrlich's reagent (An acidic solution of p-dimethyl aminobenzaldehyde)
are mixed in a tube. If the solution takes a pink colouration, this indicates the presence of
porphobilinogen or urobilinogen.
The presence of porphobilinogen may be confirmed by the addition of about 2 volumes of chloroform
to the solution and shaking thoroughly. When the mixture is allowed to stand and separate the pink
colouration should have remained in the upper aqueous layer.
If it moves to the lower chloroform layer the colouration is due to urobilinogen and not
porphobilinogen. When urobilinogen concentrations are high it may be necessary to repeat the
extraction.
MANAGEMENT OF THE ACUTE ATTACK:
Specific therapies are few: these include the use of high carbohydrate intake and haematin infusion.
Initially, steps should be taken to ensure an adequate carbohydrate intake. Most patients suffer from
nausea and vomiting during an attack and their poor carbohydrate intake aggravates the disease
process.
This cycle must be broken. In mild attacks, this is achieved by ensuring an adequate oral intake of
Glucose Polymer drinks, such as Caloreen (Roussel) or Hycal (Beecham Products). In patients
experiencing more severe attacks, the constant slow infusion of Carbohydrate solution via a fine bore
Teflon nasogastric tube, is helpful.
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 21
If the symptoms are not controlled, an infusion of Haem Arginate should be considered.(See below).
This treatment is still on trial and may be obtained from the appropriate drug company on a named
patient basis.
Symptomatic Therapy
Pain:
This is a feature of most attacks. When mild, it may be adequately controlled with aspirin, paracetamol
or dihydrocodeine. For more severe pain, pethidine (meperidine), morphine or diamorphine may be
required.
Buprenorphine, which may be administered either sublingually or intramuscularly, is also useful. More
constant pain relief may be achieved by the continuous intravenous infusion of analgesics.
There is a danger of addiction in patients experiencing frequent attacks, who require large amounts of
narcotic analgesics, and every attempt should be made to withdraw all narcotic drugs between
attacks.
In a few unfortunate patients, the pain is refractory to even very large doses of narcotic analgesics,
and signs of respiratory and cardiovascular system depression appear before pain relief is obtained.
Many of our patients report that the only time the pain goes away is when they are asleep. This
observation may be used to advantage by encouraging sleep for several hours by combining
chlorpromazine or promazine with the analgesics and leaving the patient undisturbed in a darkened
room.
Some patients continue to complain of chronic abdominal pain, unaccompanied by any other
symptoms between attacks. This can be very difficult to manage, and the risk of narcotic addiction in
these patients is high. Although, sometimes, a psychological overlay may be a factor - in others, the
pain is clearly genuine and presumably a manifestation of residual neurological damage.
Nausea, Vomiting and Constipation:
These are frequent symptoms and may be controlled with chlorpromazine, promazine or
prochlorperazine. As the narcotic analgesics used in controlling the pain often aggravate the nausea
and vomiting, it is usually helpful to administer antiemetics with or shortly before the analgesics.
Besides their antiemetic effects, chlorpromazine and promazine control the agitation and other
psychiatric manifestations of the attack. In our experience, some porphyria patients develop
extrapyramidal side-effects with phenothiazines, necessitating substitution with cyclizine
hydrochloride. Constipation, where it occurs, may be severe - to the point of obstipation - and
Neostigmine is beneficial in these circumstances.
Tachycardia and Hypertension:
These are present in most attacks. They are thought to be the result of sympathetic overactivity and
should be controlled with propranolol. The dose can be titrated against its effect on the Cardiovascular
System. Frequently very large doses are required.
The pulse and blood pressure should be closely monitored, as they tend to be labile and hypertensive
encephalopathy may develop. Postural hypotension, leading to syncope, may occur when a patient
sits upright, even when the patient has been hypertensive in the supine position.
When postural hypotension does occur, the supine blood pressure should still be adequately
controlled with propranolol, taking care when moving the patient. Paroxysmal cardiac arrhythmias,
sometimes leading to collapse, may also occur. These may be precipitated by the patient suddenly
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 22
sitting upright. Whenever there is evidence of cardiovascular instability, continual ECG monitoring
should be performed and full resuscitative facilities kept at hand.
Convulsions:
Convulsions are not infrequent at the peak of an attack. Their onset may be a sign of hyponatraemia,
due to inappropriate antidiuretic hormone secretion, and plasma osmolality, and electrolyte values
should be checked. If hyponatraemia is the underlying cause, it should be corrected by restricting fluid
intake to not more than 700 ml.
The onset of convulsions may also be a sign of hypertensive encephalopathy, and the blood pressure
should be checked. Convulsions, occurring during the attack, usually disappear as the attack resolves
and, therefore, therapy should be aimed at treating the underlying disease process.
Some patients continue to experience convulsions while in remission. This presents a therapeutic
dilemma. Phenobarbitone, primidone, phenytoin and carbamazepine all increase cellular haem
utilisation by inducing the synthesis of hepatic monoxygenases, and are contraindicated.
The benzodiazepines and sodium valproate are not inducers of the monoxygenases and, although
they are porphyrinogenic in experimental models of porphyria, there is limited evidence that they are
porphyrinogenic in man. Status epilepticus, in our own experience, has been treated successfully with
intravenous diazepam.
Seizure prophylaxis can be undertaken as a calculated risk with clonazepam or sodium valproate if
this is essential, although sporadic clinical reports of porphyr- inogenicity do exist. Sodium bromide
and magnesium sulphate are safe, but generally outmoded anticonvulsants.
Neuropathy:
All patients should be examined for evidence of developing peripheral neuropathy. This may progress
rapidly, leading to quadriplegia and bulbar and ventilatory paralysis. The latter is heralded by
weakening of the voice.
When signs of peripheral neuropathy are present, the expiratory peak flow-rate should be monitored. If
there is any reduction in this rate, the blood gases should be checked, and the patient nursed in an
Intensive Care Unit with facilities for assisted ventilation.
Even in patients in whom there is widespread paralysis requiring assisted ventilation for many months,
good functional recovery can still be expected. Attention should be given to splinting of the joints and
appropriate physiotherapy in the paralysed patient.
Fluid and Electrolyte Balance:
Various disturbances of fluid and electrolyte balance are seen during the acute attack. Dehydration
may occur, owing to persistent vomiting. Hyponatraemia, secondary to inappropriate antidiuretic
hormone secretion, may also occur, sometimes first becoming apparent after commencing
intravenous fluids.
The hyponatraemia can usually be controlled by restricting fluid intake. To maintain adequate
carbohydrate intake while restricting fluid intake, it may be necessary to use higher concentrations of
glucose, administered via a central venous line.
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 23
SPECIFIC THERAPY OF THE ACUTE ATTACK: SPECIFIC THERAPY
Haematin Therapy: ( Haem Arginate )
So far we have concentrated on treating the acute attack by correcting any factors that may have
precipitated it, and providing adequate supportive therapy while the attack spontaneously resolves. It
is also possible to treat the underlying disease process more directly by administering the end
product of the deranged pathway as intravenous haematin.
In the liver, it is thought to supplement the depleted intracellular 'free haem pool', thus repressing the
activity of the initial and rate-controlling enzyme of haem biosynthesis, ALA synthase, and reducing
the overproduction of porphyrins and precursors formed prior to the enzyme block.
In an acute attack of porphyria, the intravenous administration of haematin consistently reduces both
the plasma concentration and the urinary excretion of porphyrin precursors. Its effect may be
supplemented with inhibitors of the haem degradative enzyme, Haem oxygenase, such as Tin
protoporphyrin. The clinical response to the therapy is more difficult to assess.
In a disease characterised by spontaneous relapses and remissions, it is difficult to be sure whether
improvement is the result of therapy or just the natural course of the disease. Our current impression
is that haematin does curtail the clinical attack, and we frequently employ it.
No major side-effects have been reported with haematin when used in the standard doses. Phlebitis,
around the injection site, occurs in a few patients. This can be prevented by injecting the solution into
a large peripheral vein or via a central venous line, or by administering the haematin, with human
albumin solution, to which it will bind. During haematin therapy, there is a mild disturbance of
coagulation, with prolongation of the prothrombin and partial thromboplastin times, and a slight
reduction in the platelet count.
This reverts to normal on completion of the haematin course, and has only very rarely resulted in
haemorrhagic complications. As mentioned above coagulation indices and the platelet count should
be monitored during therapy and haematin should not be used with anticoagulant therapy.
Transient acute renal failure has been reported in one patient who received a bolus intravenous
injection of 1000 mg of haematin. Yet, no renal complications have occurred with the standard
recommended dosages, and even patients with renal insufficiency appear to tolerate haematin well,
although it is probably wise to reduce the dosage slightly as an added precaution.
A commercially available haematin preparations is: NORMOSANG ( Haem Arginate - Leiras)
PREVENTION OF ATTACKS:
Patients, who have experienced a clinical attack of porphyria, should be carefully counselled
concerning the avoidance of precipitating factors. They should be encouraged to maintain a regular
diet and to abstain completely from alcohol and to stop smoking.
In addition, they should be warned about the dangers of certain drugs and given a Reference Booklet,
showing which drugs are safe and which are unsafe to take (see Tables 1 and 2).
It is also important to ensure that the patient's General Practitioner is fully informed about the disease
and given advice about management. Patients should be reminded to tell any Medical Attendant that
they suffer from porphyria.
As an added precaution, they should wear a bracelet or necklace, indicating that they have porphyria,
to prevent the administration of dangerous drugs or anaesthetics if an accident or other emergency.
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 24
Menstruation:
Some women experience regular attacks in the week prior to the onset of menstruation. These are
assumed to be initiated by the hormonal fluctuations, and various attempts have been made to
prevent them.
Sometimes, merely increasing the carbohydrate intake at the appropriate time of
the month is found to be helpful. Interfering with the hormonal fluctuations has produced varying
results.
Some patients have been reported to benefit from the suppression of ovulation, using various oral
contraceptive hormone preparations. However, in our own experience, also that of several other
Centres, the contraceptive pill has usually precipitated attacks.
Attacks have been successfully prevented by administering haematin, prophylactically, just prior to the
time in the month when the attack usually starts and through the long term use of synthetic LHRH
analogues.
Pregnancy and Acute Porphyria:
Pregnancy may precipitate acute porphyria, with attacks being most common in early pregnancy and
during the puerperium. The first attack often occurs during pregnancy. In patients with the genetic trait,
who have normal porphyrin excretion, who have never experienced a clinical attack, pregnancy is,
usually, uneventful.
If vomiting is a problem in early pregnancy, the patient should be admitted to the hospital at an early
stage and dextrose administered, intravenously, to prevent the reduced dietary intake from inducing
an attack. Dextrose should also be administered, intravenously, during labour. Patients who have had
clinical attacks of porphyria, and have increased urinary excretion of porphyrins and precursors are
likely to experience attacks during pregnancy.
We advise such patients not to consider pregnancy until they have been free of symptomatic attacks
for at least 18 months.
When attacks do occur during pregnancy, they should be treated as already described. But, as there
is insufficient information about the effect of Haematin on the foetus, this therapy should be avoided
unless the condition of the mother demands it. Most attacks of porphyria during pregnancy settle with
adequate supportive therapy, resulting in a successful outcome for both mother and child. We only,
rarely, consider therapeutic termination of pregnancy in patients with acute porphyria and then, only in
very severe attacks, occurring in early pregnancy.
Besides the possible risks of pregnancy, many patients question the advisability of bearing children
likely to inherit a genetic disorder. It should be explained to the patient that, although each child will
have a 50 per cent chance of inheriting the trait, the majority of porphyrics remain clinically latent
throughout life.
When affected individuals decide to delay or avoid pregnancy, they should be warned about the
dangers of oral contraceptives, and advised about other forms of contraception.
Anaesthetics:
Provided appropriate precautions are taken, most patients with acute porphyria can tolerate surgery
and general anaesthesia. However, patients experiencing frequent attacks of porphyria will be at risk
of developing complications, and the indications for surgery should be carefully examined. Care must
always be taken in selecting safe anaesthetic agents.
Atropine and morphine may be used as premedication. Intravenous propofol and ketamine have been
found to be safe alternatives to thiopentone as anaesthetic-inducing agents. Cyclopropane and ether
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 25
are safe inhalation agents with respect to the porphyrias, but they suffer the disadvantages of being
potentially explosive and inducing post-operative vomiting.
Nitrous oxide, used with intravenous narcotics, and muscle relaxants, may be a more acceptable
alternative. Suxamethonium and D-tubocurarine can be used as muscle relaxants and diamorphine,
morphine, pethidine or fentanyl are suitable narcotics for controlling post-operative pain.
In some situations, epidural anaesthesia may be preferable to general anaesthesia, in which case
bupivacaine is the local anaesthetic of choice. To prevent an attack being induced by fasting, an
intravenous infusion of dextrose should be commenced prior to surgery, and continued until the
patient can eat properly.
Photosensitivity:
Photosensitivity may be found in Variegate porphyria and Hereditary Coproporphyria. The occurrence
of skin lesions depends on the degree of porphyrin overproduction and the amount of exposure to
sunlight.
Skin lesions should be treated by removing any inducing factors, such as drugs, alcohol, or
inadequate diet, that may increase the porphyrin overproduction. There is no specific treatment for the
skin photosensitivity occurring in Variegate porphyria and Hereditary coproporphyria, although Betacarotene treatment has been suggested of benefit.
Barrier creams may be used. Avoidance of excess sunlight is advised. The dermatological features
often subside after the acute attack, as the amount of circulating porphyrin is reduced.
Prophylaxis and Treatment of Malaria.
The steady encroachment of chloroquine-resistant malaria and increased travel to malarious regions
has resulted in a pressing need to re-evaluate the prophylactic strategy for people visiting or residing
in areas known to harbour the disease. Many commonly used antimalarials are known to be
porphyrinogenic.
Chloroquine has been the most widely used prophylactic and therapeutic agent
for decades, however most authorities describe its use in porphyrics as 'contentious'. Added to this is
the problem of widespread chloroquine resistance, so that chloroquine per se should not be regarded
as adequately protective against malaria.
The other agents commonly prescribed for prophylaxis, dapsone and sulphadoxine, are definitely
contraindicated in porphyrics. Combined preparations as 'Maloprim' and 'Fansidar' should not be used,
as they contain one or other of these agents.
Pyrimethamine is probably safe, but it should have little place in the prophylaxis of malaria because of
relative inefficiency. 'Daraclor', contains both chloroquine and pyrimethamine which are suspect not
only for safety in porphyrics, but also for their efficacy in resistant malaria.
Finally, there are three remaining drugs primaquine, mefloquine and proguanil. These are thought to
be safe, mefloquine and proguanil on cell culture tests and primaquine on human experience. What
then, should the porphyric person contemplating a visit to a malarial area be advised to do? An
alternative to the use of chloroquine is to consider the prophylactic use of quinine, which is of proven
safety in porphyrics.
Though widely used as a prophylactic in the past it has the disadvantages of requiring a twice daily
administration (compared with once weekly for chloroquine) and of hazardous side-effects, when
taken long-term. Yet, it retains efficacy against chloroquine-resistant Falciparum malaria.
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 26
A third strategy, the one that is probably safest to suggest now, is to avoid chemoprophylaxis
altogether and adopt the following steps:-
1. Direct prophylactic measures against the vector instead of the parasite, thus reducing the risk of
an infected bite. This has been shown to reduce the risk of contracting malaria considerably and
entails:
i) visiting malarial areas in the dry rather than the wet season;
ii) staying in towns rather than the bush;
iii) covering up with long sleeves and trousers at sundown;
iv) liberal use of insect repellents, both on the person and in the environment;
v) the use of mosquito coils; (vi) proper use of mosquito netting.
2. The visitor should be advised to carry a course of quinine sulphate tablets at all times and be
instructed on the possible symptoms of malaria, particularly pyrexia, backache, nausea and
headache. A course of quinine should be commenced at the first sign of an illness and medical
support sought as quickly as possible.
Such an approach is favoured by some authorities as a general strategy against chloroquine
resistant malaria and hence, if applied to porphyrics, as outlined, does not represent a major
departure from current thinking.
What of treatment of established malaria in porphyric patients? The course here is clear: They
should receive quinine sulphate alone; this will be efficacious against chloroquine resistant
malaria and is of proven safety in porphyria.
Finally, where malaria, other than Falciparum, is suspected, primaquine may safely be
employed to eradicate the exo-erythrocytic cycle of these parasites.
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 27
TABLE 3:
DRUG TREATMENTS IN ACUTE PORPHYRIA
CONDITION
DRUG CATEGORY
DRUG
SPECIFIC
TREATMENTS
HAEM ARGINATE
GLUCOSE
ABDOMINAL PAIN
Analgesics
ASPIRIN
BUPRENORPHINE
DIAMORPHINE
DIHYDROCODEINE
MORPHINE
PARACETAMOL
PETHIDINE
VOMITING
Anti-Emetics
CHLORPROMAZINE
CYCLIZINE
PROCHLORPERAZINE
PROMAZINE
HYPERTENSION AND
TACHYCARDIA
Anti-Hypertensives
ATENOLOL
GUANETHIDINE
LABETALOL
MECAMYLAMINE
PROPRANOLOL
NEUROSIS,
PSYCHOSIS AND
SEIZURES
Sedatives,
Tranquillisers
and Anticonvulsants
CHLORPROMAZINE
CLONAZEPAM
LORAZEPAM
PROCHLORPERAZINE
PROMAZINE
TRIFLUOPERAZINE
CONSTIPATION
Laxatives and
Anti-Cholinesterases
DANTHRON
NEOSTIGMINE
SENNA
CONDITION
DRUG CATEGORY
DRUG
ALLERGIC
REACTIONS
Anti-histamines
CHLORPHENIRAMINE
TRIPELENNAMINE
ANAESTHESIA
Anaesthetics
ATROPINE
BUPIVACAINE
CYCLOPROPANE
DROPERIDOL
ETHER
FENTANYL
KETAMINE
NITROUS OXIDE
Other Clinical Features:
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 28
CONDITION
DRUG CATEGORY
DRUG
PRILOCAINE
PROCAINE
PROPOFOL
SUXAMETHONIUM
TUBOCURARINE
ARTHRITIC AND
RHEUMATIC
CONDITIONS
Anti-Inflammatories
ALLOPURINOL
ASPIRIN
COLCHICINE
FLURBIPROFEN
INDOMETHACIN
NAPROXEN
PENICILLAMINE
CANCER
Chemotherapeutic
Agents
ACTINOMYCIN D
CISPLATIN
CYPROTERONE-ACETATE
DOXORUBICIN
MELPHALAN
VINCRISTINE SO4
CARDIOVASCULAR
Anti-Arrhythmic
Drugs
and Diuretics
CONDITIONS
AMILORIDE
ATROPINE
BUMETANIDE
CYCLOPENTHIAZIDE
DIGOXIN
DISOPYRAMIDE
DIABETES
Hypoglycaemics
INSULIN
INFECTION
Antibiotics
AMOXYCILLIN
GENTAMICIN
HEXAMINE
PENICILLINS
MALARIA
Anti-Malarials
PRIMAQUINE
PYRIMETHAMINE
QUININE
ORGAN TRANSPLANT
Immunosuppressives
AZATHIOPRINE
PREDNISOLONE
THYROID DISEASE
Anti-Thyroid Drugs
METHYL URACIL
PROPYLTHIOURACIL
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 29
TESTING FOR PORPHYRIA
SAMPLES:
1. URINE
200 ml. ALIQUOT FROM 24-HOUR URINE COLLECTION
(with no added preservative)
OR SPOT SAMPLE OF AT LEAST 100 ml. URINE
2. BLOOD:
10 ml. WHOLE BLOOD IN LITHIUM HEPARIN CONTAINER
3. FAECES:
5g FAECAL SAMPLE (in sealed container)
TO BE SENT (with a short case history) TO THE:PORPHYRIAS SERVICE
NRCET
PO Box 594
Archerfield 4108
Queensland, AUSTRALIA
FURTHER READING
Should the reader require further information on this subject, the undernoted books will provide a
valuable addition to the information in this booklet
1. DISORDERS OF PORPHYRIN METABOLISM
Michael R. Moore, Kenneth E.L. McColl,
Claude Rimington and Abraham Goldberg
PLENUM PRESS, NEW YORK (1987).
2. PORPHYRIA - CLINICS IN DERMATOLOGY 3
Eds. P.B. Disler and M.R. Moore
LIPPINCOTT, PHILADELPHIA (1985).
3. CLINICS IN HAEMATOLOGY 9. THE PORPHYRIAS
Eds. A. Goldberg and M.R. Moore
SAUNDERS, LONDON (1980).
4. A CENTURY OF PORPHYRIA
Ed. M.R. Moore
Molecular Aspects of Medicine 2 (1990)
5. BIOSYNTHESIS OF HEME AND CHLOROPHYLLS
Ed. H.A. Dailey
McGRAW-HILL, NEW YORK (1990)
A GUIDE FOR PEOPLE WITH PORPHYRIA
(V1.3)
Page 30
APPENDIX I – ALPHABETICALLY DRUG LISTING
System/Area
NUTRITION AND BLOOD
THE EYE
CARDIOVASCULAR SYSTEM
Sub Area
Diuretics
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
INFECTION
CARDIOVASCULAR SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
ANAESTHESIA
MUSCULO-SKELETAL and JOINT DISEASE
RESPIRATORY SYSTEM
CARDIOVASCULAR SYSTEM
NUTRITION AND BLOOD
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
GASTROINTESTINAL SYSTEM
ANAESTHESIA
THE EYE
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
INFECTION
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
INFECTION
INFECTION
INFECTION
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
NUTRITION AND BLOOD
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
A GUIDE FOR PEOPLE WITH PORPHYRIA
Non-Steroidal Anti-Inflammatory Agents
Anti-Gout Agents
Anti-hypertensive Agents
Hypnotics, Sedatives and Anxiolytics
Analgesics: NON-NARCOTIC
Non-Steroidal Anti-Inflammatory Agents
Diuretics
Psychoanaleptics
Psychoanaleptics
Hypnotics, Sedatives and Anxiolytics
Hormone Preparations
Analgesics: NON-NARCOTIC
Hypnotics, Sedatives and Anxiolytics
Analgesics: NON-NARCOTIC
Non-Steroidal Anti-Inflammatory Agents
Drug
Useability
Test
(Edta)
Acetazolamide
Acetazolamide
Acetylcholine
Actinomycin D
Acyclovir
Adenosine Monophosphate
Adrenaline
Alclofenac
Alcuronium
Allopurinol
Allyloxy 3-Methylbenzamide
Alpha Methyl Dopa
Alpha Tocopherylacetate
Alphaxalone - Alphadolone
Alprazolam
Aluminium OH
Aluminium Preparations
Amethocaine HCL
Amethocaine HCL
Amidopyrine
Amidopyrine
Amiloride
Amineptine HCL
Aminocaproic Acid
Aminoglutethimide
Aminoglycosides
Aminophylline
Amiodarone HCL
Amitriptyline
Amoxycillin
Amphetamines
Amphotericin D
Ampicillin
Amylobarbitone
Androgens
Antipyrine
Apronalide
Ascorbic Acid
Aspirin
Aspitin
Safe
Safe
Safe
Safe
Safe
Safe
Safe
Safe
Safe
Unsafe
Safe
Unsafe
Unsafe
Safe
Unsafe
Unsafe
Unsafe
Unsafe
Safe
Safe
Unsafe
Unsafe
Safe
Unsafe
Safe
Unsafe
Safe
Unsafe
Unsafe
Contentious
Safe
Unsafe
Safe
Safe
Unsafe
Contentious
Unsafe
Unsafe
Safe
Safe
Safe
A
B
D
Comments
C
D
B
C
C
B
C
A
A
B
D
C
C
C
C
A
B
D
B
B
B
D
U
B
Note 2
D
A
A
U
C
B
A
A
A
Page 31
System/Area
RESPIRATORY SYSTEM
CARDIOVASCULAR SYSTEM
CARDIOVASCULAR SYSTEM
GASTROINTESTINAL SYSTEM
THE EYE
MUSCULO-SKELETAL and JOINT DISEASE
Sub Area
Anti-hypertensive Agents
Specific Anti-Rheumatic Agents
MUSCULO-SKELETAL and JOINT DISEASE
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
MUSCULO-SKELETAL and JOINT DISEASE
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
RESPIRATORY SYSTEM
RESPIRATORY SYSTEM
CARDIOVASCULAR SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
MUSCULO-SKELETAL and JOINT DISEASE
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
CARDIOVASCULAR SYSTEM
Non-Steroidal Anti-Inflammatory Agents
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
CARDIOVASCULAR SYSTEM
CARDIOVASCULAR SYSTEM
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
ANAESTHESIA
Tranquillisers:Anti-Emetics
Anti-Diabetic Agents
Hypnotics, Sedatives and Anxiolytics
Anticonvulsants
Hormone Preparations
THE SKIN
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
CENTRAL NERVOUS SYSTEM
A GUIDE FOR PEOPLE WITH PORPHYRIA
Muscle Relaxants and Anti-Spasmodics
Anticonvulsants
Diuretics
Non-Steroidal Anti-Inflammatory Agents
Anti-Gout Agents
Hypnotics, Sedatives and Anxiolytics
Hormone Preparations
Calcium Channel Blockers
Diuretics
Analgesics: NARCOTICS
Hormone Preparations
Anti-hypertensive Agents
Anticonvulsants
Hormone Preparations
Hypnotics, Sedatives and Anxiolytics
Hypnotics, Sedatives and Anxiolytics
Muscle Relaxants and Anti-Spasmodics
Psychoanaleptics
Drug
Useability
Test
Astemizole
Atenolol
Atropine
Atropine
Atropine
Auranofin
Aurothiomalate
Unsafe
Safe
Safe
Safe
Safe
Unsafe
Unsafe
D
A
A
A
A
B
Azapropazone
Azathioprine
Baclofen
Barbiturates
Barbiturates
Beclomethasone Dipropionate
Bemegride
Bendrofluazide
Benoxaprofen
Benzbromarone
Benzhexol HCL
Benzylthiouracil
Bepridil HCL
Beta-Carotene
Betahistine HCL
Biguanides
Bromazepam
Bromides
Bromocriptine
Buflomedil HCL
Bumetanide
Bupivacaine
Buprenorphine
Buserelin
Busulphan
Butacaine SO4
Butylscopolamine
Unsafe
Safe
Unsafe
Unsafe
Unsafe
Safe
Unsafe
Contentious
Unsafe
Unsafe
Safe
Contentious
Unsafe
Safe
Unsafe
Safe
Contentious
Safe
Unsafe
Safe
Safe
Safe
Safe
Safe
Unsafe
Safe
Unsafe
C
C
D
A
A
B
B
S
C
D
D
DS
D
Canthaxanthin
Captopril
Carbamazepine
Carbimazole
Carbromal
Carisoprodol
Carisoprodol
Carpipramine
Carpipramine HCL
Safe
Unsafe
Unsafe
Safe
Unsafe
Unsafe
Unsafe
Contentious
Safe
A
D
A
B
B
A
A
U
Comments
Associated with acute
attacks of porphyria.
D
C
S
A
D
D
B
C
B
A
C
D
Associated with acute
attacks of porphyria.
Note 1
Page 32
System/Area
Sub Area
ANAESTHESIA
INFECTION
CENTRAL NERVOUS SYSTEM
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
INFECTION
THE EYE
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
ANAESTHESIA
INFECTION
MUSCULO-SKELETAL and JOINT DISEASE
CARDIOVASCULAR SYSTEM
RESPIRATORY SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
Hypnotics, Sedatives and Anxiolytics
Hypnotics, Sedatives and Anxiolytics
Anticonvulsants
Hypnotics, Sedatives and Anxiolytics
Hypnotics, Sedatives and Anxiolytics
Muscle Relaxants and Anti-Spasmodics
Specific Anti-Rheumatic Agents
Diuretics
Antihistamines
Hypnotics, Sedatives and Anxiolytics
Tranquillisers:Anti-Emetics
MUSCULO-SKELETAL and JOINT DISEASE
GASTROINTESTINAL SYSTEM
CENTRAL NERVOUS SYSTEM
INFECTION
GASTROINTESTINAL SYSTEM
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
INFECTION
RESPIRATORY SYSTEM
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
Muscle Relaxants and Anti-Spasmodics
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
Hormone Preparations
Psychoanaleptics
Anticonvulsants
Hypnotics, Sedatives and Anxiolytics
Anti-hypertensive Agents
Migraine
Hypnotics, Sedatives and Anxiolytics
Hypnotics, Sedatives and Anxiolytics
ANAESTHESIA
A GUIDE FOR PEOPLE WITH PORPHYRIA
Tranquillisers:Anti-Emetics
Antihistamines
Hypnotics, Sedatives and Anxiolytics
Non-Steroidal Anti-Inflammatory Agents
Drug
Useability
Test
Carticaine HCL
Cefuroxime
Cephalexin
Cephalosporins
Cephradine
Chloral Hydrate
Chlorambucil
Chloramphenicol
Chloramphenicol
Chlordiazepoxide
Chlormethiazole
Chlormethiazole
Chlormezanone
Chlormezanone
Chloroform
Chloroquine
Chloroquine
Chlorothiazide
Chlorpheniramine
Chlorpromazine
Chlorpromazine
Chlorpropamide
Unsafe
Unsafe
Unsafe
Contentious
Unsafe
Safe
Unsafe
Unsafe
Unsafe
Unsafe
Contentious
Contentious
Unsafe
Unsafe
Unsafe
Contentious
Contentious
Contentious
Safe
Safe
Safe
Unsafe
D
Chlorzoxazone
Cimetidine
Cinnarizine
Ciprofloxacin
Cisapride
Cisplatin
Clavulanic Acid
Clemastine
Clobazam
Clofibrate
Clometacin
Clomiphene
Clomiphene Citrate
Clomipramine HCL
Clonazepam
Clonazepam
Clonidine
Clonidine HCL
Clorazepate
Clotiazepam
Cloxacillin
Cocaine
Unsafe
Contentious
Unsafe
Safe
Safe
Safe
Safe
Unsafe
Contentious
Safe
Unsafe
Safe
Safe
Contentious
Contentious
Contentious
Unsafe
Unsafe
Contentious
Unsafe
Safe
Unsafe
B
U
C
B
B
D
D
B
U
D
D
Comments
Note 2
Note 2
U
Note 2
A
C
A
A
B
S
S
B
B
B
S
S
S
A
A
A
Associated with acute
attacks of porphyria.
B
DU
U
U
D
B
U
D
B
Page 33
System/Area
THE EYE
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
MUSCULO-SKELETAL and JOINT DISEASE
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
ENDOCRINE SYSTEM
INFECTION
THE SKIN
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
ANAESTHESIA
INFECTION
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
ENDOCRINE SYSTEM
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
ENDOCRINE SYSTEM
GASTROINTESTINAL SYSTEM
INFECTION
THE EYE
MUSCULO-SKELETAL and JOINT DISEASE
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
MUSCULO-SKELETAL and JOINT DISEASE
GASTROINTESTINAL SYSTEM
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
A GUIDE FOR PEOPLE WITH PORPHYRIA
Sub Area
Analgesics: NON-NARCOTIC
Analgesics: NON-NARCOTIC
Non-Steroidal Anti-Inflammatory Agents
Anti-Gout Agents
Hormone Preparations
Hormone Preparations
Tranquillisers:Anti-Emetics
Diuretics
Hormone Preparations
Hormone Preparations
Corticosteroids
Hormone Preparations
Appetite Suppressants
Analgesics: NARCOTICS
Analgesics: NARCOTICS
Analgesics: NARCOTICS
Anticonvulsants
Hypnotics, Sedatives and Anxiolytics
Muscle Relaxants and Anti-Spasmodics
Anti-hypertensive Agents
Hypnotics, Sedatives and Anxiolytics
Non-Steroidal Anti-Inflammatory Agents
Non-Steroidal Anti-Inflammatory Agents
Appetite Suppressants
Analgesics: NON-NARCOTIC
Drug
Useability
Test
Cocaine
Co-Codamol
Codeine
Codeine PO4
Colchicine
Colistin
Corticosteroids
Corticotrophin (ACTH)
Co-Trimoxazole
Coumarins
Crystal Violet
Cyclizine
Cyclopenthiazide
Cyclophosphamide
Cyclopropane
Cycloserine
Cyclosporin
Cyproterone Acetate
Cyproterone-Acetate
Danazol
Danthron
Dapsone
Desferrioxamine
Dexamethasone
Dexamethasone
Dexamethasone
Dexfenfluramine
Dextromoramide
Dextropropoxyphene
Dextrose
Diamorphine
Diazepam
Diazepam
Diazepam
Diazoxide
Dichloralphenazone
Dichloralphenazone
Diclofenac Na
Dicyclomine HCL
Dienoestrol
Diethyl Ether
Diethylpropion
Diflunisal
Digoxin
Dihydralazine
Unsafe
Safe
Safe
Safe
Safe
Unsafe
Contentious
Safe
Unsafe
Safe
Unsafe
Safe
Contentious
Unsafe
Safe
Unsafe
Unsafe
Contentious
Contentious
Unsafe
Safe
Unsafe
Safe
Safe
Safe
Safe
Unsafe
Contentious
Unsafe
Safe
Safe
Unsafe
Unsafe
Unsafe
Safe
Unsafe
Unsafe
Unsafe
Safe
Unsafe
Safe
Unsafe
Safe
Safe
Unsafe
B
B
B
A
D
B
S
B
B
Comments
B
B
U
C
C
B
C
S
S
B
A
A
B
B
B
D
S
B
B
A
A
A
D
A
A
A
B
A
C
B
A
Page 34
System/Area
Sub Area
Drug
Useability
Test
MUSCULO-SKELETAL and JOINT DISEASE
Non-Steroidal Anti-Inflammatory Agents
Dihydrocodeine
Dihydroergotamine
Safe
Unsafe
A
Dihydroergotamine-Mesylate
Diltiazem
Dimenhydrinate
Dimenhydrinate
Dimercaprol
Dimethicone
Dinoprost
Diphenhydramine
Diphenoxylate HCL
Dipyridamole
Dipyrone
Dipyrone
Disopyramide
Dixyrazine
Domperidone
Domperidone
Domperidone
Dothiepin HCL
Doxorubicin HCL
Doxycycline
Droperidol
Droperidol
Droperidol
Drotaverine
Dydrogesterone
Econazole Nitrate
Econazole Nitrate
Enalapril
Enflurane
Ergometrine Maleate
Ergot Compounds
Unsafe
Contentious
Unsafe
Unsafe
Safe
Safe
Safe
Unsafe
Safe
Safe
Unsafe
Unsafe
Contentious
Unsafe
Safe
Safe
Safe
Unsafe
Safe
Unsafe
Safe
Safe
Safe
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
A
U
B
B
Ergotamine Tartrate
Erythromycin
Estazolam
Estramustine
Unsafe
Unsafe
Contentious
Unsafe
A
A
DS
Ethacrynic Acid
Ethambutol
Ethamsylate
Ethanol
Ethchlorvynol
Safe
Safe
Unsafe
Unsafe
Unsafe
B
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
RESPIRATORY SYSTEM
CENTRAL NERVOUS SYSTEM
OBSTETRICS, GYNAECOLOGY AND RENAL DISEASE
RESPIRATORY SYSTEM
GASTROINTESTINAL SYSTEM
CARDIOVASCULAR SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
MUSCULO-SKELETAL and JOINT DISEASE
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
GASTROINTESTINAL SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
ENDOCRINE SYSTEM
INFECTION
THE SKIN
CARDIOVASCULAR SYSTEM
ANAESTHESIA
OBSTETRICS, GYNAECOLOGY AND RENAL DISEASE
CENTRAL NERVOUS SYSTEM
INFECTION
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
CARDIOVASCULAR SYSTEM
NUTRITION AND BLOOD
CENTRAL NERVOUS SYSTEM
A GUIDE FOR PEOPLE WITH PORPHYRIA
Migraine
Calcium Channel Blockers
Antihistamines
Tranquillisers:Anti-Emetics
Antihistamines
Muscle Relaxants and Anti-Spasmodics
Non-Steroidal Anti-Inflammatory Agents
Tranquillisers:Anti-Emetics
Muscle Relaxants and Anti-Spasmodics
Tranquillisers:Anti-Emetics
Psychoanaleptics
Analgesics: NARCOTICS
Hypnotics, Sedatives and Anxiolytics
Muscle Relaxants and Anti-Spasmodics
Hormone Preparations
Anti-hypertensive Agents
Migraine
Hypnotics, Sedatives and Anxiolytics
Diuretics
Hypnotics, Sedatives and Anxiolytics
Comments
Associated with acute
attacks of porphyria.
C
B
B
D
A
A
S
C
C
C
C
C
C
C
A
B
A
C
B
B
B
C
A
Associated with acute
attacks of porphyria.
Associated with acute
attacks of porphyria.
C
A
B
Page 35
System/Area
Sub Area
Drug
Useability
Test
CENTRAL NERVOUS SYSTEM
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
ENDOCRINE SYSTEM
Hypnotics, Sedatives and Anxiolytics
Anticonvulsants
Anticonvulsants
Unsafe
Contentious
Contentious
Unsafe
Safe
Unsafe
Unsafe
Unsafe
Unsafe
Safe
Safe
Safe
Safe
Unsafe
Contentious
Safe
Safe
Safe
Unsafe
Unsafe
Safe
Unsafe
Safe
Unsafe
Unsafe
Unsafe
Safe
Unsafe
Unsafe
Safe
Unsafe
Safe
Safe
Safe
Safe
Unsafe
Safe
Safe
Safe
Safe
Unsafe
C
S
S
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
ANAESTHESIA
ANAESTHESIA
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
Ethinamate
Ethinyl Oestradiol
Ethinyl Oestradiol
Ethionamide
Ethoheptazine Citrate
Ethosuximide
Ethotoin
Etidocaine
Etomidate
Etoposide
F.S.H.
Famotidine
Fenbrufen
Fenfluramine
Fenofibrate
Fenoprofen
Fenoprofen
Fentanyl
Floctafenine
Flucloxacillin
Flucytosine
Flufenamic Acid
Flumazenil
Flumequine
Flunarizine HCL
Flunitrazepam
Fluoxetine HCL
Flupenthixol
Flurazepam
Flurbiprofen
Fluroxene
Fluvoxamine Maleate
Folic Acid
Follicle Stimulating Hormone
Fructose
Frusemide
Fusidic Acid
Gentamicin
Gentamycin
Glafenine
Glibenclamide
Glipizide
Glucagon
Glucose
Unsafe
Safe
Safe
B
D
A
Hormone Preparations
GASTROINTESTINAL SYSTEM
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
INFECTION
INFECTION
MUSCULO-SKELETAL and JOINT DISEASE
ANAESTHESIA
INFECTION
RESPIRATORY SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
NUTRITION AND BLOOD
ENDOCRINE SYSTEM
NUTRITION AND BLOOD
CARDIOVASCULAR SYSTEM
THE EYE
INFECTION
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
ENDOCRINE SYSTEM
NUTRITION AND BLOOD
A GUIDE FOR PEOPLE WITH PORPHYRIA
Appetite Suppressants
Analgesics: NON-NARCOTIC
Non-Steroidal Anti-Inflammatory Agents
Analgesics: NON-NARCOTIC
Non-Steroidal Anti-Inflammatory Agents
Antihistamines
Hypnotics, Sedatives and Anxiolytics
Psychoanaleptics
Psychoanaleptics
Hypnotics, Sedatives and Anxiolytics
Non-Steroidal Anti-Inflammatory Agents
Psychoanaleptics
Hormone Preparations
Diuretics
Analgesics: NON-NARCOTIC
Anti-Diabetic Agents
Hormone Preparations
Comments
A
B
C
C
B
B
C
SD
C
C
C
D
A
D
B
D
D
D
B
B
C
A
B
B
B
B
B
A
A
A
C
C
Associated with acute
attacks of porphyria.
Page 36
System/Area
Sub Area
Drug
Useability
Test
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
ENDOCRINE SYSTEM
Hypnotics, Sedatives and Anxiolytics
Glutethimide
Glyceryl Trinitrate
Gold-(Sodium Aurothiomalate)
Goserelin
Gramicidin
Griseofulvin
Griseofulvin
Guaiphenesin
Guanethidine
Guanethidine
Guanfacine HCL
Haem Arginate
Haloperidol
Haloperidol
Halothane
Heparin
Heptaminol HCL
Hexamine
Hexapropymate
Hydantoins
Hydralazine
Hydrochlorothiazide
Hydrocortisone
Hydrocortisone Butyrate
Hydroxyzine
Hyoscine
Hyoscine Butylbromide
Hyoscine Butylbromide
Hyoscine Butylbromide
Hyoscine Butylbromide
Ibuprofen
Ibuprofen
Imipramine
Indomethacin
Indomethacin
Insulin
Iproniazid
Iron
Iron Preparations
Isoflurane
Isometheptenemucate
Isometheptene-Mucate
Isoniazid
Josamycin
Kebuzone
Unsafe
Safe
Unsafe
Safe
Unsafe
Unsafe
Unsafe
Unsafe
Safe
Safe
Safe
Safe
Contentious
Contentious
Contentious
Safe
Safe
Safe
Unsafe
Unsafe
Unsafe
Unsafe
Contentious
Contentious
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Safe
Safe
Contentious
Contentious
Contentious
Safe
Unsafe
Safe
Safe
Unsafe
Unsafe
Unsafe
Contentious
Safe
Unsafe
A
B
C
B
INFECTION
THE SKIN
RESPIRATORY SYSTEM
THE EYE
CARDIOVASCULAR SYSTEM
CARDIOVASCULAR SYSTEM
NUTRITION AND BLOOD
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
ANAESTHESIA
CARDIOVASCULAR SYSTEM
CARDIOVASCULAR SYSTEM
INFECTION
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
CARDIOVASCULAR SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
THE SKIN
CENTRAL NERVOUS SYSTEM
OBSTETRICS, GYNAECOLOGY AND RENAL DISEASE
GASTROINTESTINAL SYSTEM
THE EYE
MUSCULO-SKELETAL and JOINT DISEASE
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
NUTRITION AND BLOOD
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
INFECTION
INFECTION
A GUIDE FOR PEOPLE WITH PORPHYRIA
Specific Anti-Rheumatic Agents
Hormone Preparations
Anti-hypertensive Agents
Anti-hypertensive Agents
Hypnotics, Sedatives and Anxiolytics
Anticoagulants
Hypnotics, Sedatives and Anxiolytics
Anticonvulsants
Anti-hypertensive Agents
Diuretics
Corticosteroids
Tranquillisers:Anti-Emetics
Muscle Relaxants and Anti-Spasmodics
Analgesics: NON-NARCOTIC
Non-Steroidal Anti-Inflammatory Agents
Psychoanaleptics
Analgesics: NON-NARCOTIC
Non-Steroidal Anti-Inflammatory Agents
Anti-Diabetic Agents
Psychoanaleptics
Migraine
Tranquillisers:Anti-Emetics
Comments
A
A
C
C
A
D
A
U
U
U
B
D
C
D
A
B
B
S
S
B
A
A
A
A
B
B
U
S
S
A
B
A
C
B
C
U
D
Page 37
System/Area
ANAESTHESIA
INFECTION
THE SKIN
MUSCULO-SKELETAL and JOINT DISEASE
RESPIRATORY SYSTEM
CARDIOVASCULAR SYSTEM
ANAESTHESIA
GASTROINTESTINAL SYSTEM
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
GASTROINTESTINAL SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
GASTROINTESTINAL SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
INFECTION
GASTROINTESTINAL SYSTEM
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
INFECTION
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
MUSCULO-SKELETAL and JOINT DISEASE
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
ANAESTHESIA
A GUIDE FOR PEOPLE WITH PORPHYRIA
Sub Area
Non-Steroidal Anti-Inflammatory Agents
Anti-hypertensive Agents
Anti-hypertensive Agents
Psychoanaleptics
Hypnotics, Sedatives and Anxiolytics
Psychoanaleptics
Hypnotics, Sedatives and Anxiolytics
Hypnotics, Sedatives and Anxiolytics
Hypnotics, Sedatives and Anxiolytics
Migraine
Anticonvulsants
Psychoanaleptics
Anti-hypertensive Agents
Tranquillisers:Anti-Emetics
Analgesics: NON-NARCOTIC
Non-Steroidal Anti-Inflammatory Agents
Muscle Relaxants and Anti-Spasmodics
Hypnotics, Sedatives and Anxiolytics
Drug
Useability
Test
Ketamine
Ketoconazole
Ketoconazole
Ketoprofen
Ketotifen
L.H.R.H.
Labetalol
Levonorgestrol
Contentious
Unsafe
Unsafe
Safe
Safe
Safe
Safe
Unsafe
S
D
D
C
D
Lignocaine
Liquorice
Lisinopril
Lithium Salts
Lofepramine
Lofepramine
Loperamide
Loprazolam
Lorazepam
Loxapine
Lynestrenol
Unsafe
Safe
Unsafe
Safe
Safe
Safe
Safe
Unsafe
Contentious
Unsafe
Unsafe
C
B
B
B
B
B
D
D
S
D
Lysuride Maleate
Magnesium Sulphate
Magnesium-Sulphate
Maprotiline HCL
Mebendazole
Mebeverine HCL
Mecamylamine
Mecillinam
Unsafe
Safe
Safe
Unsafe
Contentious
Unsafe
Safe
Unsafe
C
B
B
C
S
D
C
Meclofenoxate HCL
Meclozine
Medroxyprogesterone
Safe
Safe
Unsafe
Mefenamic Acid
Mefenamic Acid
Mefloquine HCL
Megestrol
Melphalan
Mephenesin
Mephenytoin
Contentious
Contentious
Safe
Unsafe
Contentious
Unsafe
Unsafe
U
U
D
C
S
D
Mepivacaine
Meprobamate
Meptazinol
Unsafe
Unsafe
Safe
D
A
B
Comments
B
Associated with acute
attacks of porphyria.
Associated with acute
attacks of porphyria.
Associated with acute
attacks of porphyria.
A
Associated with acute
attacks of porphyria.
Associated with acute
attacks of porphyria.
Page 38
System/Area
Sub Area
Drug
Useability
Test
CENTRAL NERVOUS SYSTEM
RESPIRATORY SYSTEM
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
THE EYE
Analgesics: NARCOTICS
Diuretics
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
ANAESTHESIA
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
CENTRAL NERVOUS SYSTEM
Psychoanaleptics
Anti-Diabetic Agents
Analgesics: NARCOTICS
Appetite Suppressants
CENTRAL NERVOUS SYSTEM
Anticonvulsants
Safe
Safe
Unsafe
Unsafe
Unsafe
Contentious
Unsafe
Contentious
Safe
Safe
Unsafe
Unsafe
Unsafe
Contentious
Unsafe
Unsafe
Unsafe
B
B
B
B
CARDIOVASCULAR SYSTEM
Meptazinol
Mequitazine
Mercaptopurine
Mercuric Oxide
Mercury Compounds
Mersalyl
Mestranol
Metapramine HCL
Metformin
Methadone
Methamphetamine
Methohexitone
Methotrexate
Methotrimeprazine
Methoxyflurane
Methsuximide
Methyl Dopa
Methyl Sulphonal
Unsafe
Methylcellulose
Methylphenidate
Methylphenidate
Methyluracil
Methyprylone
Methysergide
Metipropanolol
Metoclopramide
Metoclopramide
Metoclopramide
Metopimazine
Metoprolol
Metoprolol Tartrate
Metronidazole
Metyrapone
Mianserin HCL
Miconazole
Miconazole
Midazolam
Midazolam
Mifepristone
Minaprine HCL
Minaxolone
Minocycline HCL
Minoxidil
Safe
Safe
Safe
Safe
Unsafe
Unsafe
Safe
Contentious
Contentious
Contentious
Safe
Safe
Safe
Contentious
Unsafe
Unsafe
Unsafe
Unsafe
Safe
Contentious
Contentious
Safe
Safe
Safe
Unsafe
Hypnotics, Sedatives and Anxiolytics
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
Appetite Suppressants
Hypnotics, Sedatives and Anxiolytics
Psychoanaleptics
Hormone Preparations
Hypnotics, Sedatives and Anxiolytics
CARDIOVASCULAR SYSTEM
GASTROINTESTINAL SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
CENTRAL NERVOUS SYSTEM
GASTROINTESTINAL SYSTEM
Anti-hypertensive Agents
CARDIOVASCULAR SYSTEM
INFECTION
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
INFECTION
THE SKIN
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
OBSTETRICS, GYNAECOLOGY AND RENAL DISEASE
CENTRAL NERVOUS SYSTEM
ANAESTHESIA
INFECTION
Anti-hypertensive Agents
A GUIDE FOR PEOPLE WITH PORPHYRIA
Muscle Relaxants and Anti-Spasmodics
Tranquillisers:Anti-Emetics
Hormone Preparations
Psychoanaleptics
Hypnotics, Sedatives and Anxiolytics
Psychoanaleptics
Comments
S
DU
C
A
B
C
C
S
A
Associated with acute
attacks of porphyria.
Associated with acute
attacks of porphyria.
B
B
D
B
A
B
U
U
U
D
B
S
D
C
D
D
D
DS
U
D
C
D
Page 39
System/Area
Sub Area
Drug
Useability
Test
CENTRAL NERVOUS SYSTEM
Analgesics: NARCOTICS
Morphine
Nadolol
Nadrolone
Safe
Safe
Unsafe
A
Naftidrofuryloxalate
Nalidixic Acid
Nandrolone
Naproxen
Naproxen Na
Natamycin
Nefopam HCL
Neostigmine
Netilmycin
Nicergoline
Nifedipine
Nifumic Acid
Nikethamide
Nitrazepam
Nitrofurantoin
Nitrous Oxide
Nordazepam
Norethisterone
Safe
Unsafe
Contentious
Safe
Safe
Unsafe
Safe
Safe
Safe
Contentious
Unsafe
Safe
Unsafe
Unsafe
Contentious
Safe
Unsafe
Unsafe
D
B
U
B
C
B
D
A
D
DU
B
D
A
B
U
A
Norethynodrel
Norfloxacin
Nortriptyline
Novobiocin
Oestrogens
Ofloxacin
Omeprazole
Oral Contraceptives
Oral Contraceptives
Orphenadrine
Orphenadrine
Oxanamide
Oxazepam
Oxazolidinediones
Oxolinic Acid
Oxybuprocaine
Oxybutynin HCL
Oxycodone
Oxymetazoline
Oxypentifylline
Oxyphenbutazone
Oxyphenbutazone
Unsafe
Safe
Contentious
Unsafe
Contentious
Safe
Contentious
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Contentious
Unsafe
Safe
Safe
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
CARDIOVASCULAR SYSTEM
INFECTION
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
INFECTION
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
INFECTION
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
RESPIRATORY SYSTEM
CENTRAL NERVOUS SYSTEM
INFECTION
ANAESTHESIA
INFECTION
CENTRAL NERVOUS SYSTEM
INFECTION
OBSTETRICS, GYNAECOLOGY AND RENAL DISEASE
INFECTION
GASTROINTESTINAL SYSTEM
OBSTETRICS, GYNAECOLOGY AND RENAL DISEASE
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
MUSCULO-SKELETAL and JOINT DISEASE
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
INFECTION
THE EYE
CENTRAL NERVOUS SYSTEM
THE EAR, NOSE AND OROPHARYNX
CARDIOVASCULAR SYSTEM
THE EYE
MUSCULO-SKELETAL and JOINT DISEASE
A GUIDE FOR PEOPLE WITH PORPHYRIA
Hormone Preparations
Analgesics: NON-NARCOTIC
Non-Steroidal Anti-Inflammatory Agents
Analgesics: NON-NARCOTIC
Muscle Relaxants and Anti-Spasmodics
Analgesics: NON-NARCOTIC
Calcium Channel Blockers
Non-Steroidal Anti-Inflammatory Agents
Hypnotics, Sedatives and Anxiolytics
Psychoanaleptics
Hormone Preparations
Migraine
Muscle Relaxants and Anti-Spasmodics
Muscle Relaxants and Anti-Spasmodics
Hypnotics, Sedatives and Anxiolytics
Anticonvulsants
Analgesics: NARCOTICS
Non-Steroidal Anti-Inflammatory Agents
Comments
Associated with acute
attacks of porphyria.
Associated with acute
attacks of porphyria.
D
U
B
U
D
S
A
A
C
B
C
U
B
D
C
C
B
D
B
A
Page 40
System/Area
OBSTETRICS, GYNAECOLOGY AND RENAL DISEASE
MUSCULO-SKELETAL and JOINT DISEASE
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
CENTRAL NERVOUS SYSTEM
INFECTION
MUSCULO-SKELETAL and JOINT DISEASE
INFECTION
CENTRAL NERVOUS SYSTEM
Sub Area
Muscle Relaxants and Anti-Spasmodics
Analgesics: NON-NARCOTIC
Non-Steroidal Anti-Inflammatory Agents
Anticonvulsants
Anticonvulsants
Muscle Relaxants and Anti-Spasmodics
Psychoanaleptics
Specific Anti-Rheumatic Agents
Analgesics: NARCOTICS
RESPIRATORY SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
Hypnotics, Sedatives and Anxiolytics
Analgesics: NARCOTICS
Analgesics: NARCOTICS
Psychoanaleptics
Anti-Diabetic Agents
Anticonvulsants
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
Anti-hypertensive Agents
Anticonvulsants
MUSCULO-SKELETAL and JOINT DISEASE
Non-Steroidal Anti-Inflammatory Agents
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
INFECTION
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
Anticonvulsants
Non-Steroidal Anti-Inflammatory Agents
Psychoanaleptics
Hypnotics, Sedatives and Anxiolytics
GASTROINTESTINAL SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
MUSCULO-SKELETAL and JOINT DISEASE
INFECTION
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
A GUIDE FOR PEOPLE WITH PORPHYRIA
Anti-Gout Agents
Non-Steroidal Anti-Inflammatory Agents
Migraine
Hypnotics, Sedatives and Anxiolytics
Drug
Useability
Oxytetracycline
Oxytocin
Pancuronium
Pancuronium Bromide
Pancuronum Br
Paracetamol
Paracetamol
Paraldehyde
Paramethadione
Parapenzolate Br
Pargyline
Pefloxacin
Penicillamine
Penicillin
Pentazocine
Pentolinium
Pentylenetetrazol
Perhexiline
Pericyazine
Pethidine
Phenacetin
Phenelzine
Phenformin
Phenobarbitone
Phenoperidine
Phenoxybenzamine
Phensuximide
Phentolamine Mesylate
Phenylbutazone
Phenylhydrazine
Phenytoin
Pipebuzone
Pipemidic Acid
Pipothiazine Palmitate
Piracetam
Pirbuterol
Pirenzepine
Piritramide
Piroxicam
Piroxicam
Pivampicillin
Pivmecillinam
Unsafe
Safe
Contentious
Safe
Contentious
Safe
Safe
Safe
Unsafe
Safe
Unsafe
Safe
Safe
Safe
Unsafe
Safe
Unsafe
Unsafe
Safe
Safe
Unsafe
Unsafe
Safe
Unsafe
Safe
Unsafe
Unsafe
Safe
Contentious
Unsafe
Unsafe
Unsafe
Unsafe
Safe
Safe
Safe
Safe
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Pizotifen
Prazepam
Safe
Unsafe
Test
Comments
B
S
Note 1
S
A
B
B
A
D
C
D
B
A
A
B
D
A
C
C
C
A
D
A
U
A
D
D
D
D
B
C
D
B
Associated with acute
attacks of porphyria.
B
D
Page 41
System/Area
CARDIOVASCULAR SYSTEM
THE EYE
MUSCULO-SKELETAL and JOINT DISEASE
ENDOCRINE SYSTEM
CARDIOVASCULAR SYSTEM
ANAESTHESIA
INFECTION
CENTRAL NERVOUS SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
CARDIOVASCULAR SYSTEM
CARDIOVASCULAR SYSTEM
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
Sub Area
Corticosteroids
Hormone Preparations
Calcium Channel Blockers
Anticonvulsants
Anti-Gout Agents
Hypnotics, Sedatives and Anxiolytics
Migraine
Tranquillisers:Anti-Emetics
Anticonvulsants
ENDOCRINE SYSTEM
Hormone Preparations
CENTRAL NERVOUS SYSTEM
RESPIRATORY SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
ANAESTHESIA
CARDIOVASCULAR SYSTEM
GASTROINTESTINAL SYSTEM
OBSTETRICS, GYNAECOLOGY AND RENAL DISEASE
MUSCULO-SKELETAL and JOINT DISEASE
ANAESTHESIA
Hypnotics, Sedatives and Anxiolytics
Antihistamines
Hypnotics, Sedatives and Anxiolytics
Tranquillisers:Anti-Emetics
ENDOCRINE SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
ANAESTHESIA
THE EYE
RESPIRATORY SYSTEM
INFECTION
NUTRITION AND BLOOD
INFECTION
Hormone Preparations
Non-Steroidal Anti-Inflammatory Agents
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
INFECTION
GASTROINTESTINAL SYSTEM
CARDIOVASCULAR SYSTEM
Hypnotics, Sedatives and Anxiolytics
A GUIDE FOR PEOPLE WITH PORPHYRIA
Anti-hypertensive Agents
Muscle Relaxants and Anti-Spasmodics
Anti-hypertensive Agents
Drug
Useability
Test
Prazosin
Prednisolone
Prednisolone
Prednisolone
Prenylamine
Prilocaine
Primaquine
Primidone
Probenecid
Probucol
Procainamide HCL
Procaine
Prochlorperazine
Prochlorperazine
Prochlorperazine
Progabide
Progesterone
Contentious
Contentious
Contentious
Contentious
Unsafe
Unsafe
Safe
Unsafe
Contentious
Safe
Safe
Safe
Safe
Safe
Safe
Unsafe
Unsafe
S
S
S
S
D
B
B
A
B
D
D
B
B
B
B
D
Progestogens
Proguanil HCL
Promazine
Promethazine
Promethazine
Promethazine
Propanidid
Propanolol
Propantheline
Propantheline
Propantheline Br
Propofol
Propranolol
Propylthiouracil
Propyphenazone
Proxymetacaine
Proxymetacaine
Pseudoephedrine HCL
Pyrazinamide
Pyridoxine HCL
Pyrimethamine
Pyrrocaine
Quinalbarbitone
Quinidine
Quinine
Ranitidine
Reserpine
Contentious
Safe
Safe
Contentious
Contentious
Contentious
Contentious
Safe
Safe
Safe
Safe
Safe
Safe
Safe
Unsafe
Contentious
Contentious
Safe
Unsafe
Safe
Contentious
Unsafe
Unsafe
Safe
Safe
Contentious
Safe
U
Comments
Associated with acute
attacks of porphyria.
A
U
U
U
U
A
B
B
B
B
B
A
S
S
B
A
A
S
B
C
B
U
A
Page 42
System/Area
Sub Area
THE SKIN
INFECTION
RESPIRATORY SYSTEM
GASTROINTESTINAL SYSTEM
CARDIOVASCULAR SYSTEM
NUTRITION AND BLOOD
INFECTION
CENTRAL NERVOUS SYSTEM
GASTROINTESTINAL SYSTEM
CARDIOVASCULAR SYSTEM
THE SKIN
ENDOCRINE SYSTEM
INFECTION
CENTRAL NERVOUS SYSTEM
NUTRITION AND BLOOD
Anticonvulsants
MUSCULO-SKELETAL and JOINT DISEASE
THE EYE
Non-Steroidal Anti-Inflammatory Agents
GASTROINTESTINAL SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
INFECTION
ENDOCRINE SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
ANAESTHESIA
MUSCULO-SKELETAL and JOINT DISEASE
INFECTION
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
CENTRAL NERVOUS SYSTEM
RESPIRATORY SYSTEM
ANAESTHESIA
INFECTION
THE EYE
A GUIDE FOR PEOPLE WITH PORPHYRIA
Diuretics
Hormone Preparations
Anticonvulsants
Anti-Gout Agents
Anti-Diabetic Agents
Hypnotics, Sedatives and Anxiolytics
Anticonvulsants
Hypnotics, Sedatives and Anxiolytics
Muscle Relaxants and Anti-Spasmodics
Hypnotics, Sedatives and Anxiolytics
Antihistamines
Drug
Useability
Test
Resorcinol
Rifampicin
Salbutamol
Senna
Simvastatin
Sodium Aurothiomalate
Sodium Bromide
Sodium Calcium Edetate
Sodium Fusidate
Sodium Oxybate
Sodium Valproate
Sorbitol
Spironolactone
ß Carotene
Stanozolol
Streptomycin
Succinimides
Sulbutiamine
Sulfadoxine
Sulindac
Sulphacetamide
Sulphadiazine
Safe
Unsafe
Safe
Safe
Unsafe
Unsafe
Safe
Safe
Safe
Unsafe
Unsafe
Safe
Unsafe
Safe
Unsafe
Safe
Unsafe
Safe
Safe
Safe
Unsafe
Unsafe
B
B
B
A
C
Sulphadimidine
Unsafe
Sulphadoxine
Unsafe
Sulphamethoxazole
Unsafe
Sulphasalazine
Sulphinpyrazone
Sulphonamides
Sulphonylureas
Sulpiride
Sulthiame
Sultopride
Suxamethonium
Suxamethonium
Talampicillin
Tamoxifen
Temazepam
Terfenadine
Tetracaine
Tetracyclines
Tetracyclines
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Unsafe
Safe
Safe
Safe
Unsafe
Safe
Unsafe
Safe
Contentious
Contentious
Comments
A
B
U
A
C
A
C
A
A
D
Note 2
C
C
Associated with acute
attacks of porphyria.
Associated with acute
attacks of porphyria.
Associated with acute
attacks of porphyria.
Associated with acute
attacks of porphyria.
C
U
A
A
C
B
D
C
A
B
B
B
B
C
S
S
Page 43
System/Area
Sub Area
Drug
Useability
Test
CENTRAL NERVOUS SYSTEM
RESPIRATORY SYSTEM
ANAESTHESIA
CENTRAL NERVOUS SYSTEM
ENDOCRINE SYSTEM
ENDOCRINE SYSTEM
MUSCULO-SKELETAL and JOINT DISEASE
INFECTION
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
THE EYE
CARDIOVASCULAR SYSTEM
INFECTION
Hypnotics, Sedatives and Anxiolytics
Tetrazepam
Theophylline
Thiopentone Na
Thioridazine
Thiouracil
Thyroxine
Tiaprofenic Acid
Ticarcillin
Tienilic Acid
Tilidate
Timolol Maleate
Timolol Maleate
Tinidazole
Tolazamide
Unsafe
Unsafe
Unsafe
Unsafe
Safe
Safe
Safe
Safe
Safe
Unsafe
Safe
Safe
Unsafe
Unsafe
D
B
A
B
B
B
B
B
D
B
D
D
D
Tolazoline
Tolbutamide
Safe
Unsafe
A
Tranexamic Acid
Tranylcypromine
Trazodone HCL
Triacetyloleandomycin
Triamterene
Triazolam
Trichlormethiazide
Trifluoperazine
Trimebutine Maleate
Trimeprazine Tartrate
Trimetazidine HCL
Trimethoprim
Trimipramine
Tripelennamine
Troxidone
Tubocurarine
Tubocurarine
Valproate
Valpromide
Vancomycin
Veralipride
Verapamil
Vibramycin
Viloxazine HCL
Vinblastine
Vincristine
Vitamins
Safe
Unsafe
Unsafe
Safe
Safe
Safe
Contentious
Safe
Safe
Safe
Safe
Unsafe
Contentious
Safe
Unsafe
Safe
Safe
Unsafe
Contentious
Safe
Unsafe
Unsafe
Unsafe
Unsafe
Contentious
Contentious
Safe
B
C
C
CARDIOVASCULAR SYSTEM
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
CENTRAL NERVOUS SYSTEM
Hypnotics, Sedatives and Anxiolytics
Hormone Preparations
Hormone Preparations
Non-Steroidal Anti-Inflammatory Agents
Diuretics
Analgesics: NARCOTICS
Anti-hypertensive Agents
Anti-hypertensive Agents
Psychoanaleptics
Psychoanaleptics
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
CENTRAL NERVOUS SYSTEM
GASTROINTESTINAL SYSTEM
RESPIRATORY SYSTEM
CARDIOVASCULAR SYSTEM
INFECTION
CENTRAL NERVOUS SYSTEM
RESPIRATORY SYSTEM
CENTRAL NERVOUS SYSTEM
ANAESTHESIA
MUSCULO-SKELETAL and JOINT DISEASE
Hypnotics, Sedatives and Anxiolytics
Diuretics
Hypnotics, Sedatives and Anxiolytics
CENTRAL NERVOUS SYSTEM
INFECTION
CENTRAL NERVOUS SYSTEM
CARDIOVASCULAR SYSTEM
INFECTION
CENTRAL NERVOUS SYSTEM
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
NUTRITION AND BLOOD
Anticonvulsants
A GUIDE FOR PEOPLE WITH PORPHYRIA
Antihistamines
Psychoanaleptics
Antihistamines
Anticonvulsants
Muscle Relaxants and Anti-Spasmodics
Psychoanaleptics
Calcium Channel Blockers
Psychoanaleptics
Comments
Associated with acute
attacks of porphyria.
Associated with acute
attacks of porphyria.
C
S
B
D
D
D
B
U
B
B
C
B
DU
B
D
B
B
C
UD
UD
B
Page 44
System/Area
Sub Area
Drug
Useability
Test
CARDIOVASCULAR SYSTEM
INFECTION
MALIGNANT DISEASE: and IMMUNOSUPPRESSION
THE SKIN
THE EYE
CENTRAL NERVOUS SYSTEM
Anticoagulants
Warfarin Na
Zidovudine
Zidovudine
Zinc Preparations (Topical)
Zinc Sulphate
Zuclopenthixol
Safe
Safe
Safe
Safe
Safe
Unsafe
B
B
B
B
A
C
A GUIDE FOR PEOPLE WITH PORPHYRIA
Psychoanaleptics
Comments
Page 45