Hereditary Angioedema in Women

For many women, managing HAE is particularly complex because hormonal changes, such as those that occur during puberty, menstruation, pregnancy, and menopause, can influence the frequency and severity of attacks — making life transitions that are already challenging even more difficult.

Estrogen plays a major role in how HAE behaves in women. Higher levels of this hormone, whether naturally occurring or due to contraceptives or hormone therapy, can increase bradykinin, the chemical that triggers swelling in HAE.

As a result, women often experience their first attacks or worsening symptoms at times when estrogen levels rise. Research suggests that hormonal fluctuations are one of the most important factors influencing how HAE affects women.

For many girls, HAE symptoms begin or become more noticeable around puberty. The increase in estrogen that accompanies this stage of life can cause attacks to occur more frequently or be more severe.

Because of the role hormones play in attacks, immunologists and allergists who treat HAE work closely with their female patients’ primary care providers or ob-gyns, says Rita Kachru, MD, an immunologist at UCLA Health in Santa Monica.

“If we start seeing patterns of attacks occurring during ovulation or occurring during menstruation or a few days before your period starts, we want to make sure that [your] hormone levels are as stable or optimized as possible so that we can better regulate these attacks,” says Dr. Kachru.

Education and self-management training are key for teens and young women. Having a clear plan for treating attacks, avoiding known triggers, and understanding when to seek emergency care can help reduce anxiety and promote independence.

Close monitoring by both an obstetrician and an immunologist familiar with HAE is crucial. It’s important to talk about your concerns with your providers or with a mental health professional so that you can get the support you need during your pregnancy.

Labor and delivery require careful coordination between the obstetric, anesthesia, and immunology teams to discuss the plan in case of an attack, though it’s uncommon to have an attack during labor. Typically, short-term prophylactic treatment isn’t needed.

After delivery, some women may have angioedema (swelling) of the vulva. On-demand medication should be kept ready for emergency use.

Vaginal delivery is generally preferred, because cesarean section (also known as a C-section) carries a higher risk of triggering an HAE attack. For women who do need a C-section, short-term prophylaxis with plasma-derived C1 inhibitor before surgery is recommended.

Breastfeeding can increase HAE attacks, which are thought to be caused by an increase in prolactin levels. (Prolactin is one of the main hormones involved in breastfeeding.) Typically, when women stop breastfeeding, the number of attacks go down.

Hot flashes and night sweats are common symptoms of menopause, but estrogen-based hormone replacement therapy (including phytoestrogens) isn’t recommended. Vaginal estrogens are okay because the systemic dose is so low.

Treatments such as lifestyle adjustments, cognitive behavioral therapy, or nonhormonal medications are safer alternatives. As with other stages of life, the key is individualized care guided by an allergist-immunologist and gynecologist familiar with both HAE and women’s health.

Beyond the medical management of HAE, the emotional toll can be significant. Many women report feelings of isolation, anxiety about attacks, and frustration with being misunderstood by healthcare providers or family members.

• Hormonal changes throughout life — from puberty to menopause — can influence how hereditary angioedema affects women.
• Estrogen can trigger or worsen attacks, so contraception, pregnancy, and menopause management require careful coordination with providers familiar with HAE.
• Pregnancy and breastfeeding may require HAE medication changes to prevent the fetus from potential harm.
• Connecting with knowledgeable specialists and patient groups can make living with HAE more manageable and less isolating.