What's UP Doc? Is a monthly column where we feature a patient question along with a response from a member of the UPA Scientific Advisory Board.
Do you have a question you would like to ask a Porphyria Expert? Send us an email at firstname.lastname@example.org. We’d love to hear from you!
This question was about EPP, but the information also applies to other types of light-sensitive porphyria (CEP, VP, HCP, PCT).
The risk of having a reaction to the different types of light used during surgery depends on several factors, including:
The more severe your EPP and the longer the procedure, the higher the likelihood of a reaction. While you are unlikely to have a reaction during a short procedure or from a laparoscope, the safest approach is to take steps to minimize exposure to unsafe light (wavelengths 400-470nm). Lights to consider are:
Overall, surgery can be undertaken safely with a cutaneous porphyria if steps are taken to minimize the risk of a phototoxic reaction.
Successful Liver Transplantation for Liver Failure With Erythropoietic Protoporphyria by Covering the Operating Theater Lights With Polyimide Film: A Case Report in Transplantation Proceedings, Volume 52, Issue 2, 2020, Pages 625-629. https://doi.org/10.1016/j.transproceed.2019.12.004.
The diagnosis and treatment of rare conditions like EPP and XLP can be confusing for doctors and patients alike. Join Dr. Amy Dickey as she walks us through the recommendations from the new consensus guidelines for diagnosing and managing protoporphyrias.
Liver dysfunction is a rare but very real possibility for people with EPP and XLP. These guidelines provide recommendations for the diagnosis, monitoring and treatment of liver-related dysfunction in protoporphyrias (EPP and XLP).
This research describes some important features of EPP including prodromal (warning) symptoms, a priming effect from previous light exposure, and lack of visible symptoms among many patients.
Question: Are there guidelines for managing erythropoietic protoporphyria (EPP) and x-linked porphyria (XLP)?