Surgery precautions and cutaneous porphyrias

What's UP Doc?
Written By Dr. Herbert Bonkovsky

What's UP Doc? Is a regular column where we feature a patient question along with a response from a member of the UPA Scientific Advisory Board.

I have EPP and am having a planned laparoscopic surgery next week. Should there be a filter on the light on the laparoscope? If so, would you be able to tell me what filter would need to be used? Can you also tell me what filters would be needed for the surgery lights in the operating room?

 

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 severity of your EPP or other porphyria, how long does it take to have a phototoxic reaction when exposed to light?

  • The concentration of red blood cell and plasma protoporphyrin

  • The length of the procedure

  • The intensity of the operating room or other lights used

 

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:

  • Operating room lights and laparoscope lights. These can be covered with filters that will block unsafe wavelengths of light. Recommended filters include:

  • The finger pulse/oxygen device should not be left on your finger for extended periods. These types of devices shine light unto the finger where they are attached. I’d advise they be put on periodically to take measurements and then removed.

Overall, surgery can be undertaken safely with a cutaneous porphyria if steps are taken to minimize the risk of a phototoxic reaction.

 

Reference:

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.

 

Thank you to Dr. Herbert Bonkovsky for this What's UP Doc? answer! Do you have a question for a porphyria expert? Send it to info@porphyria.org.

 

 

Check out related What’s UP Doc? Responses

Porphyria Spotlight
Urine Porphyrins for Diagnosis
Urine Porphyrins for Diagnosis

Question: I think I might have Acute Intermittent Porphyria (AIP). My doctor ordered a urine porphyrin test, my porphyrins were high but then I was told that the results aren't considered diagnostic. Why not? And what tests should my doctor order?

Porphyria and Menopause
Porphyria and Menopause

Question: How will menopause affect my porphyria? Are estrogen treatments for menopause safe?

Surgery precautions and cutaneous porphyrias
Surgery precautions and cutaneous porphyrias

Question: I am having a planned laparoscopic surgery next week.  Should there be a filter on the light on the laparoscope?  If so, would you be able to tell me what filter would need to be used?  Can you also tell me what filters would be needed for the surgery lights in the operating room?

Acute porphyria and liver tests
Acute porphyria and liver tests

Question: If you have acute porphyria, why is it important to have your liver function checked annually? What can you do to prevent issues with your liver?

Dr. Herbert Bonkovsky

Dr. Bonkovsky’s interest in the porphyrias began in medical school and was strengthened during his time studying under porphyria researcher D.P. Tschudy at the National Institutes of Health. Dr. Bonkovsky was among the first to show that hepatic porphyrin and heme synthesis is under the negative feedback regulatory control of heme itself, acting chiefly to down-regulate delta-aminolevulinic acid (ALA) synthase-1, the rate controlling enzyme for heme synthesis. This discovery was the basis for developing heme therapy for acute porphyria attacks, which is still today the treatment of choice for these life-threatening attacks. Dr. Bonkovsky was the first physician-investigator to purify heme and to administer it to a patient with severe acute intermittent porphyria.

Since that time, Dr. Bonkovsky has continued the search for new treatments for the porphyrias, administering clinical trials for potential new therapies at hospitals and medical centers in Massachusetts, Connecticut, and now at the Atrium Health--Wake Forest Baptist Medical Center in Winston-Salem, North Carolina. He is principal investigator of the Porphyria Center of Excellence at Wake Forest/NC Baptist Medical Center, which is a part of the Porphyrias Consortium of the USA, sponsored by the National Institute of Diabetes, digestive and Kidney Diseases and the National Center of Rare Diseases Research.

Dr. Bonkovsky also identified deficiency of ferrochelatase, the final enzyme in the heme synthetic pathway as the fundamental metabolic defect in EPP. Studies on the regulation of hepatic heme metabolism, especially the mechanisms and factors that regulate ALA synthase and heme oxygenase, have been the subject of a large number of studies, both in the basic research laboratory and the clinical research center during the past 50 years.

Dr. Bonkovsky has trained many medical students, residents, and sub-specialty fellows, especially in the fields of internal medicine, gastroenterology, and hepatology, as well as graduate students and junior faculty working on laboratory research.

Previous

Acute hepatic porphyria and anaesthesia
Next

Meet Padmini Pillai, PhD and UPA President's Council Chair