Summing UP is a new feature where we share the latest porphyria research in easy-to-understand summaries that have been reviewed and approved by the UPA's Scientific Advisory Board of porphyria experts.
Full article: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7810766/
Published February 2021 in BJA Education
Surgery and the use on anesthesia require special considerations for patients with acute hepatic porphyrias (AHP- includes AIP, VP and HCP). This article provides an overview* of precautions for the period before, during and after an operation (the ”perioperative period”) for patients with AHP.
*Note: please refer to the full article for a fuller understanding and when making care decisions.
Surgery can be safely undertaken in patients with porphyria, provided care is taken to avoid porphyria triggers.
Acute porphyria attacks can be triggered by a number of environmental factors that may be present before, during or after a procedure requiring anesthetic. These triggers include:
Patients who have active, or recently active, porphyria are most likely to develop an acute attack and it is recommended that a porphyria specialist be involved in the management of their care during the perioperative period.
Patients who have been diagnosed with acute porphyria but do not have a history of attacks (for example, diagnosed through family genetic testing) have a low risk of developing an acute attack, provided appropriate precautions are followed. If urgent anesthesia is required and there isn’t time to test a patient with a family history of porphyria, they should be treated with the same precautions as if they are affected and have definitive testing arranged after surgery.
Medication principles and precautions
Fasting principles and precautions
Prolonged fasting has been known to trigger attacks. The following precautions are recommended:
Stress and pain principles and precautions
Perioperative stress should addressed with adequate premedication and pain control. Nausea and vomiting should also be addressed.
Following a procedure, porphyria symptoms may be masked by pain medications or look similar to operation-related complications. Patients with a history of porphyria are generally able to recognize attacks, however the possibility of complications or infection should be ruled out.
Most patients with AHP have normal pregnancies without clinical issues relating to porphyria. Acute attacks which have occurred during pregnancy have been safely treated with Haem Arginate (heme) without adverse outcomes.
During labor and delivery the following precautions are advised:
Acute porphyria is very rare before puberty. For children who are asymptomatic and have a known diagnosis of porphyria because of family testing, avoiding unsafe medication is recommended.
Cardiopulmonary bypass is used in some surgeries where a machine temporarily takes over the function of the heart and lungs during surgery, and maintains the circulation of blood and oxygen to the body. There is a theoretical risk that the stress caused by this procedure could cause a porphyria attack, however there are several reports of successful cardiopulmonary bypass surgery for patients with AHP, which suggest that it is safe as long as the general precautions described above are followed.
People with a porphyria diagnosis are generally able to recognize if an acute attack is starting, but in the case of surgery, other causes for symptoms, such as post-operative pain or pregnancy-related complications should be ruled out.
If you do begin to experience an attack in the perioperative period, the general measures for treating an attack should be followed, including:
Anesthesia can safely be given to patients with a diagnosis of AHP providing appropriate precautions are taken and only medicines on the safe list are administered perioperatively. If you have a history of acute porphyria attacks, it is advised that a porphyria specialist be consulted prior to your operation.
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?
This article provides an overview of the different types of pain in acute hepatic porphyria (AIP, VP, HCP, ADP) and how they may be treated.
Question: I have AIP. I haven’t had a porphyria attack for several years and I don’t have a porphyria specialist. Is there anything I should ask my doctor to check for during my annual check-up?
Question: I heard a doctor in a presentation say that people with porphyria should “look after their kidneys.” How do you do that?