QOTW – Will genital swelling that some HAE patients experience cause issues down the road?

Oct 4, 2015
“I have a son with HAE type 1. He is in his early teens and has had episodes where his testes will swell. We do have on demand treatment, which allowed the swelling to resolve over 36 hours. He is worried that the swelling may cause future problems with normal sexual function down the road. What advice do you have for him?”

Dr. C: We would like to thank Leigh Farrar from the HAEA for forwarding this question from one of our readers. It is hard enough being a teenager and dealing with issues of sexuality–this is a frightening problem for patients. We do know that cutaneous swelling is the most common site for HAE attacks. It has been reported that the genital areas can be involved in up to 2/3rds of patients. Triggers can be trauma, activities such as horse riding and sexual intercourse. As is the case for most attacks, there is often no clear trigger. Untreated the swelling will resolve over approximately 3 days. We always advise patients to use your on demand treatment at the first sign of swelling to reduce the severity and duration of the attack. In response to our reader’s question, I have not found any reports of permanent damage or sexual dysfunction resulting from attacks of the genital area. Bruce and Marc, any thoughts here?

Dr. R: Genital swelling is a fairly common symptom of HAE.  In my opinion, it’s not discussed much because it can be very embarrassing for people to talk about with their physician, but it’s clearly a very troubling and disabling problem.  During the attacks, this swelling can be extremely uncomfortable or painful and can interfere with sexual and urinary function for both men and women.  This highlights the importance of treating attacks early with an effective acute medication to minimize both the amount of angioedema and the time it takes for the symptoms to fully resolve.  So far, there’s no evidence that recurrent HAE swelling episodes have any chronic or long-term effects on sexual function or fertility for either genders.  So it’s really the important matter of treating attacks early and effectively to prevent these functional issues DURING the attacks rather than any concern about long-term cumulative effects of the swelling episodes.  One other point that I’d bring up related to sexual health in HAE, is that long-term androgen therapy can cause adverse effects related to sexual function and fertility in both women and men.  So this is another issue to be aware of if patients choose to use androgens for long-term prophylaxis.

Dr. C: Thank you for reminding us about the need to consider side effects in the long-term management of HAE. We had discussed the need for monitoring and liver function abnormalities with androgens during our June 1 QOTW, archived in the notes section of our Facebook page. Marc brings up another important point regarding its impact on men and women in this context. Bruce what are your thoughts here?

Dr. Z: I certainly understand your son’s concern, but agree with Sandra and Marc that these attacks will not cause any lasting damage. It is essential that all HAE patients have access to effective on-demand treatment. In addition, I’m optimistic that prophylactic options will improve in the future and that the need for frequent on-demand treatments will be decreased. In the meanwhile, your son should treat these attacks as early as possible. This is another example where self-administration of on-demand treatment has particular value.

Dr. C:  Thank you, Bruce and Marc. We hope that continued careful gathering of information and analysis will lead to more refined answers in the future. We look forward to our next ‘Question of the Week’.

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