Drugs in Porphyria

Drugs in Porphyria

 

The Welsh Medicines Information Centre (WMIC) offers a specialist advisory service on the safe use of drugs in porphyria.

The UK Porphyria Medicines Information Service (UKPMIS) is provided in conjunction with the Cardiff Porphyria Service provided by the Department of Biochemistry and Immunology, University Hospital of Wales.


 

In line with the new General Data Protection Regulations you are required to opt in to receive any future communications regarding the Porphyria Safe List.

 Opt in here  OR contact us on 029 2074 4298

Search the Porphyria list

Download a PDF copy of the 2018 Porphyria Safe List or use the search function below

2018 SAFE LIST: Drugs that are considered to be SAFE for use in the acute porphyrias
This safe list was produced jointly by the UK Porphyria Medicines Information Service (UKPMIS) and Cardiff Porphyria Service and is supported by the National Acute Porphyria Service (NAPS). We gratefully acknowledge the clinical impact of the evidence-based drug safety assessments provided on the comprehensive Drug Database for Acute Porphyria (http://www.drugs-porphyria.org) on the range of available medicines for porphyria patients.
N.B. Some drugs may be included under their group name and not their individual drug name.

This safe list is based on the best information available to us at the time of compilation and provides guidance on drugs to use first line. It is not intended to be comprehensive. Inclusion of a drug does not guarantee that it will be safe in all circumstances. For information on medication not listed and advice on the use of antiretrovirals, antineoplastics or other complex treatment scenarios please contact UKPMIS by telephone (029 2074 3877). Unfortunately we are unable to accept enquiries via e-mail. Healthcare professionals requiring clinical advice on management of acute porphyria attacks should contact NAPS on 029 2074 7747.

Key

1. The following drugs have not been assessed as safe, and should not be used:
cangrelor (Antiplatelet drugs); ciprofibrate (Fibrates); albiglutide (GLP-1 receptor
agonists), pivmecillinam, temocillin, ticarcillin (Penicillins); nalidixic acid
(Quinolones); frovatriptan (Triptans). Contact UKPMIS for further advice.
2. Intravenous doses should be used with caution.$Gliptins = DPP-4 inhibitors = alogliptin, linagliptin, saxagliptin, sitagliptin,
vildagliptin
3. Clinical safety has not been assessed for continuous use of these preparations.+GLP-1 receptor agonists = dulaglutide, exenatide, liraglutide, lixisenatide
4. Conflicting safety advice for flucloxacillin. Contact UKPMIS for further advice.#SGLT-2 inhibitors = canagliflozin, dapagliflozin, empagliflozin
5. An acute attack has been reported following yellow fever vaccine, so
caution should be observed when using any live vaccine in adults.
*SSRIs = Selective Serotonin Reuptake Inhibitor antidepressants

All topical preparations (including topical antifungals, lice treatments & eye drops) are considered safe, when applied to intact skin or mucosa.


What is 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’.


Why can drugs be problematic?

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 WMIC. You can download these from the ‘resources for patients’ and ‘resources for healthcare professionals’ sections below.


Resources for patients

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 WMIC.

Drugs considered ‘safe’ in the acute porphyrias

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

The Welsh Medicines Information Centre (WMIC) offers a specialist advisory service on the safe use of drugs in porphyria to patients and healthcare professionals in the UK. The centre contact telephone number is 029 2074 2251/029 2074 2979 and fax number is 029 2074 3879.


Resources for healthcare professionals

In conjunction with the Department of Biochemistry and Immunology, University Hospital of Wales, WMIC has recently had two articles published in the Adverse Drug Reaction Bulletin on safe pescribing 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, WMIC 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 also produce a series of ‘questions and answers’ (Q&As) on medicines-related porphyria enquiries that are available to view through the SPS website:

And finally, WMIC offers a specialist advisory service on the safe use of drugs in porphyria to patients and healthcare professionals in the UK. Medicines Information enquiries can be submitted by telephoning the centre on 029 2074 2251/029 2074 2979 or faxing the centre on 029 2074 3879.


Links to useful websites

Disclaimer: These links are provided for information only and WMIC 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 WMIC or the Department of Biochemistry and Immunology, University Hospital of Wales.

What information we need to answer porphyria enquiries

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

Patient’s 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