MANAGEMENT OF THE PORPHYRIC
PATIENT
EXPOSED TO POTENTIALLY UNSAFE DRUGS
INTRODUCTION
A patient at risk of an acute porphyric
crisis may be exposed to a porphyrinogenic drug under
one of three circumstances: firstly, where the diagnosis
of porphyria only becomes known to the doctor following
initiation of treatment; secondly where the drug has
been given inadvertently; and thirdly where a decision
has been taken to use the drug because its use is demanded
by pressing circumstances. Such patients must be regarded
as being at some degree of risk for the subsequent development
of an acute attack. The risk is greater for AIP than
for VP.
MANAGEMENT
- Where the drug has been given
inadvertently, rather than purposefully,
stop the drug immediately.
- Specific prophylactic measures
are not recommended.
- Reassure the patient that exposure
even to dangerous medication is
frequently tolerated.
- Advise the patient of the symptoms
of an impending attack (abdominal
pain, dark urine) and instruct
them to report the onset of such
symptoms without delay. (Read
Acute
symptoms of porphyria).
- Ensure that your pharmacy or
hospital is able to access haem
arginate promptly if treatment
appears necessary. Note that treatment
within 24-48 hours of onset is
usually satisfactory, which does
allow leeway for obtaining a stock
PROVIDED arrangements have been
made beforehand. Read Obtaining
haem arginate.
- In the event of such symptoms,
confirm the presence of an acute
attack by having urine tested
urgently for the presence of PBG,
and proceed to treat if necessary.
(Read Management
of the acute attack.)
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