Inherited disorders: Acute porphyria and medicines
Medicines can contribute towards triggering an acute attack of porphyria in a patient with AIP, VP, HCP or ADP. There are a number of ways by which they can do this, including:- Induction of the haem pathway. Some medicines increase the activity of haem pathway enzymes or induce cytochrome p450 synthesis.
- Female sex hormones. The mechanism is unclear, but these are known to be highly porphyrinogenic.
- Adverse drug reactions. A side effect may cause sufficient physiological disturbance to trigger an acute attack (e.g. drug-induced vomiting, leading to reduced calorific intake).
Deciding whether a medicine is safe
If you are asked about choosing a 'safe' medicine for a patient with porphyria, you must start by identifying the type of porphyria they have. It's only patients with acute porphyrias that must avoid the medicines that trigger acute attacks. If you're not able to speak to the patient directly about their precise diagnosis then you may be able to ask a relative or carer: Does the patient suffer from acute porphyria and have they ever had an acute attack?Note that patients can still have a diagnosis of acute porphyria, even if they have never suffered from an acute attack.
UK Porphyria MI Service (UKPMIS) Tel. 029 2184 2251 (Monday to Friday 9am to 5pm; Saturday 9am to 12.45pm)
At present there is no consensus view about the safety of many widely-used drugs; largely because of difficulty in reconciling evidence from disparate sources. However, you have two core sources of information to help you. The BNF contains a list of medicines that are rated as unsafe in acute porphyria. There is also a list of medicines rated as safe, produced by the UK Porphyria Medicines Information Service. You should always check both lists.
If you cannot find out if a medicine is safe, or you are uncertain, then you can contact the following services for advice:
UK Porphyria MI Service (UKPMIS) Tel. 029 2184 2251 (Monday to Friday 9am to 5pm; Saturday 9am to 12.45pm)
National Acute Porphyria Service (NAPS) Tel. 029 2074 7747 (available 24/7).
Where there is no safe alternative, drug treatment for serious or life-threatening conditions should not be withheld from patients with acute porphyria.
When advising on the safety of medicines in
acute porphyria, you should point out that any risk from a medicine is
cumulative with other precipitants of an acute attack such as infection etc.
(see previous
page).
The Welsh Medicines Advice Service have produced a series of articles on prescribing in patients with porphyria;
Prescribing restrictions in non-acute cutaneous porphyria (available in Welsh)
Travel – a guide for people with acute porphyria (available in Welsh)
Acne treatment in those with acute porphyria (available in Welsh)