Acute attacks in patients with acute hepatic porphyria (AHP) have long been associated with female hormones and the use of hormone containing contraceptives. Females with acute hepatic porphyria have traditionally been advised to avoid all forms of exogenous forms of oestrogen and progesterone due to the risk of causing an acute attack. This leaves patients in a difficult position when it comes to choosing contraception, or treating symptoms such as menorrhagia, dysmenorrhoea, menopause symptoms. There is limited research on the effect of specific hormonal contraceptives, with just one study in 2003 finding 1 in 4 AIP patients experienced an attack with contraceptives containing progesterone, oestrogen or a combination.¹

We designed a questionnaire which was sent to all female patients with a diagnosis of AHP - acute intermittent porphyria (AIP), hereditary coproporphyria (HCP), variegate porphyria (VP), in our hospital database, and distributed via the Australian Porphyria patient support group. Participation was voluntary and unidentifiable. Patients were asked their previous experience with hormonal treatments. Thirty responses were obtained (AIP 12, VP 12, HCP 6). 23 patients had used hormonal treatments. 7 patients had not used hormonal treatments, with 5/7 (71%) of this group reporting the reason for this being due to fear of side effects including a flare of porphyria.

HCP was the most common porphyria type to experience a flare with hormones (66%), and approximately 20% of VP and AIP patients. No patients flared with hormone replacement therapy (HRT), the progesterone-only pill, or progesterone implant. The progesterone-containing intrauterine device (IUD) resulted in a flare in 2/7 (28.5%), whilst 7/15 (46.6%) flared with the combined oral contraceptive pill. Drospirenone-containing pills, Yaz and Yasmin were the most likely brands to cause a flare. Levonorgestrel in the Mirena IUD and Levlen, were more likely to be tolerated, particularly by patients with VP compared to HCP. In terms of treatments that were reported to have helped porphyria symptoms, the Mirena IUD was most likely to be of benefit, likely as a result of keeping hormone levels stable. Many patients reported being unsure of whether they received any benefit from the medication.

This study, although limited by small sample size and the retrospective nature of the questionnaire, add to the literature and aid in our ability to effectively counsel patients and prescribe much-needed hormonal treatments.

Reference

1. Andersson C, Innala E, Bäckström T. Acute intermittent porphyria in women: clinical expression, use and experience of exogenous sex hormones. A population-based study in northern Sweden. Journal of Internal Medicine, 2003;254(2):176–183.