Treatment
Often, there is no specific treatment and the avoidance of precipitating factors is the best action that can be taken.
Treatment of acute hepatic porphyia (AHP):
- treatment options for AHP aim to prevent attacks or manage symptoms
- include pain management, stopping medication that could have triggered symptoms, gonadotrophin releasing hormone (GnRH) analogues for hormone-induced attacks in women, and oral or intravenous glucose for acute attacks
- haem arginate is indicated for treating acute attacks of AHP
- also used outside its marketing authorisation to prevent attacks
- liver transplant may be an option for some people with recurrent severe attacks when other treatment options have not worked
- givosiran is recommended as an option for treating acute hepatic porphyria (AHP) in adults and young people aged 12 and older, only if:
- they have clinically confirmed severe recurrent attacks (4 attacks or more within 12 months)
- is a small-interfering ribonucleic acid that suppresses delta-aminolevulinic acid synthase 1 production by the liver. This reduces the level of toxic precursors of porphyrin
Phlebotomy and / or chloroquine has been effective in porphyria cutanea tarda.
Beta-carotene is used to reduce sensitivity in erythropoietic protoporphyria.
Splenectomy may be used for intractable haemolytic anaemia.
Reference:
- Edel Y, Mamet R. Porphyria: What Is It and Who Should Be Evaluated?. Rambam Maimonides Med J. 2018;9(2):e0013. Published 2018 Apr 19. doi:10.5041/RMMJ.10333
- Karim Z, Lyoumi S, Nicolas G, Deybach JC, Gouya L, Puy H. Porphyrias: a 2015 update. Clin Res Hepatol Gastroenterol. 2015;39:412-425
- NICE (November 2021). Givosiran for treating acute hepatic porphyria
Create an account to add page annotations
Annotations allow you to add information to this page that would be handy to have on hand during a consultation. E.g. a website or number. This information will always show when you visit this page.