Drugs in Porphyria

Porphyria is a rare genetic disorder that can be passed through families. The porphyrias are a group of disorders of the “haem biosynthesis pathway”.

Briefly…

Haemoglobin, which carries oxygen around the body in the blood, is made in the liver. Haemoglobin is formed as a result of a series of chemical reactions called the “haem biosynthesis pathway”. Each stage of the pathway is controlled by a specific enzyme. All porphyrias result from partial deficiency of one of the enzymes of the haem biosynthesis pathway.

This can lead to a build-up of chemicals which cannot be processed efficiently by the defective enzyme. This can sometimes cause acute attacks of porphyria.

Acute attacks are often provoked by drugs, alcohol, and hormonal changes. Infections and stressful situations may also precipitate an acute attack. Rarely an acute attack may be life-threatening.

For more detailed information see the sections below ‘resources for patients’ and ‘resources for healthcare professionals’

Many drugs, particularly those that induce cytochrome P450s, have long been recognised as important precipitants of acute attacks in those who inherit one of the acute porphyrias.

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.

A list of drugs considered safe in the acute porphyrias has been produced jointly by the Department of Biochemistry and Immunology, University Hospital of Wales and the WMAS. You can download these from the ‘resources for patients’ and ‘resources for healthcare professionals’ sections below.

A list of drugs considered safe in the acute porphyrias has been produced jointly by the Department of Biochemistry and Immunology, University Hospital of Wales and WMAS.

European Porphyria Initiative is an informative website on porphyria that includes a section for patients and their families/carers.

The Welsh Medicines Advice Service (WMAS) offers a specialist advisory service on the safe use of drugs in porphyria to patients and healthcare professionals in the UK. The service contact telephone number is 029 2184 2251

In conjunction with the Department of Biochemistry and Immunology, University Hospital of Wales, WMAS has published two articles in the Adverse Drug Reaction Bulletin on safe prescribing in the acute porphyrias:

• Lockett CD and Badminton MN.  Safe prescribing in the autosomal dominant acute porphyrias: a practical approach: Part I. Adverse Drug Reaction Bulletin.  August 2015; No. 293: pp. 1131 – 1134
• Lockett CD and Badminton MN.  Safe prescribing in the autosomal dominant acute porphyrias: a practical approach: Part II. Adverse Drug Reaction Bulletin.  October 2015; No. 294: pp. 1135 – 1138

And again in conjunction with the Department of Biochemistry and Immunology, University Hospital of Wales, WMAS has produced an information booklet about porphyria for healthcare professionals and a list of drugs considered safe in the acute porphyrias. You can download these from here:

• WMIC Porphyria Bulletin 2008
• Drugs considered ‘safe’ in the acute porphyrias
• Porphyria categories safe list

WMAS also produce a series of articles on medicines-related porphyria enquiries:

• What are the restrictions on prescribing for patients with non-acute porphyria?
• What advice should be given to patients with porphyria who intend to travel?
• Are topical medicines safe in people with porphyria?
• How should haem arginate (human hemin) be administered in the management of acute porphyrias?
• What is the most suitable treatment for acne in a patient with acute porphyria?

And finally, WMAS offers a specialist advisory service on the safe use of drugs in porphyria to patients and healthcare professionals in the UK. Medicines Advice enquiries can be submitted by telephoning the centre on 029 2184 2251

These links are provided for information only and WMAS is not responsible for the content of these sites.
Views expressed on these sites are the views of the site owners and do not necessarily represent the views of WMAS or the Department of Biochemistry and Immunology, University Hospital of Wales.

European Porphyria Initiative
Norwegian Porphyria Centre (NAPOS) in collaboration with the Swedish Porphyria Centre
Porphyria Service – Cardiff
The British and Irish Porphyria Network (BIPNET)

In order for us to answer a porphyria enquiry, please provide us with the following information where possible:

Patient details
Name of patient
Sex of patient
Age of patient

Type of porphyria
Acute Intermittent Porphyria (AIP)
Hereditary Coproporphyria (HCP)
Variegate Porphyria (VP)
Erythropoietic Protoporphyria (EPP)
Porphyria Cutanea Tarda (PCT)
Congenital Erythropoietic Porphyria
Unknown

Further information
Current status of illness i.e. active or latent
Any history of drug induced attacks
Condition being treated