Hereditary angioedema (HAE) is a rare genetic condition characterized by acute episodes of swelling in various areas of the body, including the face, limbs, trunk, or genitalia. The swelling can also affect internal organs, such as the gastrointestinal and respiratory tracts.
How pregnancy affects HEA
Women are more predisposed to hereditary angioedema attacks than men; this is thought to be due to higher levels of estrogen in their body.
In pregnancy, estrogen levels are even higher, so the disease may get worse. A temporary increase in the number of episodes may also occur during breastfeeding, possibly due to higher levels of the hormone prolactin. The interruption of breastfeeding might decrease angioedema episodes by leading to a decline in prolactin levels.
Care during pregnancy and beyond
Close monitoring during pregnancy, labor, delivery, and breastfeeding should be conducted, as these events may alter the course of hereditary angioedema. Each woman’s body can react differently to these events and during each individual pregnancy.
Delivery should always occur in a hospital setting and the patient should be closely monitored by different specialists to provide a multidisciplinary approach. Monitoring should be continued for at least 72 hours after delivery.
Based on the patient’s medical history, the doctor will decide whether a preventative treatment, such as plasma-derived C1-INH, should be considered before delivery.
The doctor can then prescribe a personalized treatment with C1-INH to continue at home and train the patient how to use it to reduce the severity and duration of the attacks.
Treatment of HAE during pregnancy and breastfeeding
The pharmacological management of hereditary angioedema during pregnancy becomes very restricted because some medicines are contraindicated or have no benefits during pregnancy.
Attenuated androgens such as Danocrine (danazol), Winstrol (stanozolol), Oxandrine (oxandrolone), Kalbitor (ecallantide), Firazyr (icatibant) and the recombinant human C1-INH Ruconest are not approved for use in pregnancy or lactation.
Some treatments like antifibrinolytics and androgens can be excreted into breast milk and cause potential adverse effects in babies, making the management of hereditary angioedema a challenge during breastfeeding.
Treatments approved for use during pregnancy and lactation are:
- Plasma-derived human CI inhibitor concentrate (pdhC1INH): Berinert and Cinryze are indicated as a first-line treatment during pregnancy and lactation.
- Antifibrinolytics like tranexamic acid are indicated during pregnancy, but not during lactation.
- Virally inactivated fresh frozen plasma is indicated during pregnancy and lactation whenever other treatments are not available.
Will my baby also have HAE?
Hereditary angioedema is a genetic condition caused by mutations in the SERPING1 (types 1 and 2) or F12 genes (type 3). The disease is inherited in an autosomal dominant pattern, meaning that one faulty copy of the gene is sufficient to cause the disease. So the baby of a parent who has the mutation has a 50 percent risk of also having the disease.
It is important to be diagnosed as soon as possible. However, it is usually not recommended to screen babies immediately after birth as this might lead to false results. To have reliable and early results, a laboratory diagnosis should be performed after the baby’s first birthday by measuring the quantity and activity of the C1-INH protein. In ambiguous cases, a genetic test can also be performed to check for mutations in the SERPING1 or F12 genes, but this is rarely necessary.
Angioedema News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.