Table 7. [Prevention of Acute Attacks in COPX Heterozygotes]. - GeneReviews®

Principle	Prevention	Consideration/Other
Identifying those at risk of attacks	See Evaluation of Relatives at Risk.
Education of at-risk persons	Educate re circumstances that may trigger an acute attack (see Clinical Description).
Selection of appropriate contraception for females	Recommended method of birth control for HCP heterozygotes: IUD plus a barrier (diaphragm &/or condom) ¹	A copper-releasing IUD is theoretically the safest in women w/porphyria. A hormone-releasing IUD may also be safe because the systemic ↑ in hormone is quite small; however, little information exists on its use in women w/acute porphyria.
Suppress menses using a GnRH agonist.	Leuprolide, nafarelin, & other GnRH agonists may help those who experience monthly exacerbations. If menopausal side effects occur, they can be treated by adding low-dose estrogen. ²
Prevention of acute attacks in those undergoing surgery	Minimize preoperative fast as much as possible.
Provide intravenous glucose (10% dextrose in half-normal saline) in perioperative period.
Induce anesthesia using non-barbiturate agents that have little or no P450-inducing activity (e.g., propofol, ketamine, short-acting benzodiazepines).	Inhalation agents (isoflurane) & muscle relaxants also appear to be low-risk for triggering an attack.

Principle

Prevention

Consideration/Other

Identifying those at risk of attacks

See Evaluation of Relatives at Risk.

Education of at-risk persons

Educate re circumstances that may trigger an acute attack (see Clinical Description).

Selection of appropriate contraception for females

Recommended method of birth control for HCP heterozygotes: IUD plus a barrier (diaphragm &/or condom) ¹

A copper-releasing IUD is theoretically the safest in women w/porphyria.
A hormone-releasing IUD may also be safe because the systemic ↑ in hormone is quite small; however, little information exists on its use in women w/acute porphyria.

Suppress menses using a GnRH agonist.

Leuprolide, nafarelin, & other GnRH agonists may help those who experience monthly exacerbations.
If menopausal side effects occur, they can be treated by adding low-dose estrogen. ²

Prevention of acute attacks in those undergoing surgery

Minimize preoperative fast as much as possible.

Provide intravenous glucose (10% dextrose in half-normal saline) in perioperative period.

Induce anesthesia using non-barbiturate agents that have little or no P450-inducing activity (e.g., propofol, ketamine, short-acting benzodiazepines).

Inhalation agents (isoflurane) & muscle relaxants also appear to be low-risk for triggering an attack.

From: Hereditary Coproporphyria

GeneReviews^® [Internet].

Adam MP, Feldman J, Mirzaa GM, et al., editors.

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