Acute Porphyria Attacks
Porphyrias are a group of genetic disorders characterized by high levels of porphyrins in the blood and tissues. There are several types of porphyrias, each with different symptoms and disease progression.
What are acute porphyria attacks?
Acute porphyria attacks are periods of severe symptoms that may require hospitalization; they can be life-threatening if left untreated.
An acute porphyria attack often starts with severe pain in the abdomen, back, or thighs. Many patients experience nausea, vomiting, and constipation. Some people become very confused and disoriented. Although it is rare, some people have convulsions and muscle weakness.
An acute porphyria attack usually lasts one or two weeks, but leaving them untreated can be dangerous because of neurological problems they may cause, such as paralysis.
Most patients experience one, or sometimes a few, attacks in their lifetime. It is more likely for women to have multiple attacks over their lifetime because of hormonal changes.
What causes them?
Anything that stimulates the enzymes in the porphyrin pathway that leads to a rapid increase in porphyrins can cause acute porphyria attacks.
Some common triggers are alcohol, some medications, liver disease, smoking, cannabis, and hormonal changes (such as those women experience during the menstrual cycle). Some women may even require medications that stop the menstrual cycle to prevent these attacks. It is important to note that some hormonal birth control pills may not be safe for some women with porphyria.
How do doctors treat the attacks?
The first step for the management of acute porphyria attacks is to remove the trigger. In severe cases, it is important to go to the hospital so that you can receive haem arginate, a medication that slows the production of porphyrins.
If you have recurring acute porphyria attacks, you should go to the hospital when your symptoms first appear, so that treatment can begin immediately. This is especially important in patients who experience paralysis or other neurological problems as a result of acute porphyria attacks.
Last updated: Aug. 18, 2020
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