Porphyria and Menstruation

Porphyria refers to a group of genetic disorders characterized by high levels of porphyrins in the blood and tissues. Several types of porphyria exist, each with different symptoms and disease progression.

Patients with acute porphyria can experience episodes of severe symptoms that may require hospitalization and be life-threatening if left untreated. The menstrual cycle may be linked to acute porphyria attacks.

About the menstrual cycle

The menstrual cycle is a natural biological cycle controlled by hormones. In the early stages of the cycle, the levels of a hormone called estrogen start to rise and trigger the ovaries to release a new egg, which potentially can be fertilized if sperm is introduced at the right time. Then, levels of another hormone, progesterone, start to rise and prepare the uterus for implanting a fertilized egg. The lining of the uterus swells in anticipation of a possible pregnancy.  If sperm does not fertilize the egg, the inner lining of the uterus starts to shed and menstruation occurs.

Estrogen, progesterone, and porphyria

Estrogen and progesterone also stimulate the enzymes that produce porphyrins. That’s why many women with porphyria experience attacks either in the middle of their cycle (when a surge in estrogen occurs during ovulation) or a few days before the beginning of menstruation (due to high levels of progesterone).

Attacks often begin with cramping and abdominal pain. Many women may mistake the porphyria attack for the “normal” pain and cramping that typically accompanies menstruation.

How doctors treat menstruation-associated porphyria attacks

Usually, not every period will involve an acute porphyria attack, but many women have two to four severe porphyria attacks a year. For those women should go to the hospital for treatment with haem arginate. It may take three or four days for the attack to subside.

For women whose attacks are more severe (or for whom every cycle can cause an acute attack) medications that prevent the increase of estrogen and progesterone may be prescribed. These medications (GnRh agonists) induce menopausal symptoms. They do not work for all patients, and they do come with side effects, including osteoporosis, so there is a risk with taking these medications long-term. Moreover, medications do not treat the underlying porphyria. However, removing a trigger for acute porphyria attacks, even short-term, may be helpful for some patients.

It is important to note that some oral contraceptives contain hormone analogs that may cause acute porphyria attacks. If you have porphyria, it’s important to talk to your gynecologist about what medications and contraceptives are safe to use.

In extreme cases when porphyria attacks are so severe that patients require frequent hospitalizations, a liver transplant may have some benefit. However, larger studies are necessary to confirm these findings.

Last updated: Aug. 11, 2020

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