An Interview With @AffyAvo on Hereditary Angioedema♥

To commemorate HAE Awareness Day, I would like to ask you some questions regarding your experiences and to foster positive discussion in our community. For reader's clarification - HAE means Hereditary angioedema.

*Trigger warning* For those who have emetophobia (the word is mentioned, no details) or needle phobias (no graphic details)

Question (1): Since HAE is a genetic condition, did your parents also have HAE? Were you aware that you would have this condition from childhood?

No, I have a de novo mutation which is a fancy way of saying new, which is true for about 25% of people with HAE. I had never heard of the condition until I was told my C1 inhibitor function was low.

Question (2): Most times during early childhood or adolescence a person may not recognize the symptoms associated with HAE until later. Was this the same for you, how was your experience with HAE progressively?

It has definitely gotten worse overtime for me, I think due to other health problems. In hindsight, I can recognize attacks I had as a child though. For example, I went to the emergency room as a young child from a bug bite as my entire face was swollen. A hairline fracture resulted in swelling way beyond what was normal for the injury. I also lived with abnormal things that were considered to be minor that I highly suspect were attacks, like a lot of frequent vomiting that I would get over quickly and sore throats. I do have asthma and allergies, so I think that made it easy for doctors to just handwave away my symptoms.

Question (3): What kind of symptoms do you experience because of hereditary angioedema?

The main thing is it causes severe swelling, that’s the defining feature of an attack. With that comes pain and general discomfort. Depending on where the swelling is it can cause things like gastrointestinal problems, voice changes and difficulty breathing or seeing. I have completely lost my voice before because of it. There are also both prodromes and postdrome symptoms that occur before and after. I tend to get pretty severe fatigue and malaise both before and after and sometimes mood changes or a sense of doom beforehand.

Question (4): Was it difficult for your partner to learn how to best support you?

He actually learned how to give me my medications extremely well quickly. I had a lot of fear when I first got diagnosed though and his favourite phrase at that time was don’t worry about it. He learned that wasn’t helping and after a while, we would laugh when we caught him saying that. He also did have a great deal of stress with getting my IVs started, as even though he was really good they didn’t always work on the first try or sometimes would stop part way through. So he had to learn to deal with that stress and we both did much better when a subcutaneous option was available.

I think he used to brush off how bad it could be, a few years into my diagnosis we went to an educational session and the number of people with tracheostomies (a hole in the neck to the airway) or scars from previous ones was a wakeup call for him.

Question (5): You’ve been on 7 Cups since 2014. How has the community of people diagnosed with HAE grown, and the disability support community in general?

The HAE community on 7 Cups is mostly me, although I did come here based on someone with HAE who shared their referral link in an online group. Every now and then I meet someone else who has it or another type of angioedema. Off of Cups, it has grown a ton and the ‘rare’ status may be dropped as diagnosis rates improve.

The disability community has grown so much! When I first showed up we had one forum that wasn’t divided into categories beyond ‘disability’ Shortly after that guided discussions were a thing and people like you stepped up to do some disability ones (yes! @FrenchMarbles was one of the early discussion hosts!) and we got our own 24/7 group chat room shortly after that. Even with those things, many people on the site were confused about why someone with a physical illness would be here. I actually started the chronic pain and illness forums and eventually those were combined with disability and the subcommunity formed. Since then the disability subcommunity has grown and I look forward to seeing where it goes!

I’m so thankful for people like @DonaldK (who started the disability forum) and the early disability session hosts, they gave me a place to be when I came here and likely wouldn’t have stuck around without a dedicated place.

Question (6): What challenges did you face from when you were diagnosed with HAE till now?

At first, there was just a huge learning curve and a lot of fear. At that time the only medication in Canada was an IV c1 inhibitor, so my very first treatment was combined with IV training. That did NOT go well, I was pretty much unconscious by the end of it. Besides the fear of self-starting and IV, there was also the fear that if I had a throat attack and didn’t get it done quickly I could die.

Funnily enough, even when I stitched to subcutaneous injections which are WAY easier I was still freezing up at times, so I went off to more therapy to deal with that phobia which was well worth it. I wish I would have done more therapy earlier on.

Learning triggers was also very difficult, I have a lot and I suspect I will learn more with time. Being active is one so just learning to really listen to my body and that threshold took a lot of trial and error.

I also always figured I had an undiagnosed problem that made the HAE much worse, as it did worsen considerably leading up to my diagnosis. My symptoms and test results also backed that up, but everything was fairly generalized. It actually turns out I have tumours. It is a rare condition so I haven’t shared much about that on 7 Cups to keep some anonymity. When those were first found it was assumed by multiple doctors that I had very advanced cancer and no one really gave me a timeline but it wasn’t good. I was literally told to have no hope.

Sidenote - I actually came to 7 Cups that day and told some people I was dying, so to anyone who was there and didn’t hear updated info, I wasn’t lying, just misinformed! The support and love I got during that time were invaluable!

So at this point, it’s figuring out ‘normal’ again between that and issues that cascaded from that. Plus throw in COVID and connected problems like right now a healthcare system that is crumbling and processing trauma, figuring out how to interact with healthcare that has really let me down at times.

Question (7): What valuable lessons/experiences have you had living with HAE?

Advocacy! When I was first diagnosed I joined online groups about HAE. Most of them were American and I would hear horror stories of emergency room experiences, many of them in small towns. I figured here would be a different, different healthcare system, in a major city. NOPE! First emergency trip for an attack I experienced a lot of what others shared, they wanted to treat me with allergy medications. I denied multiple medications and tried a lot of doses of one that made things worse until eventually, they agreed to use my medication.

I have learned since then, to speak like an expert - as I am one! (to be fair at that time things were so new I wasn’t). Delays still happen at times when they shouldn’t with treating attacks. I do not accept drastic treatment changes from the plan my specialist has set.

That advocacy has helped me since, from asking to having certain tests done to ensuring things that should be done actually get done.

I have also learned to incorporate mental resources into my overall healthcare. With the healthcare system, many of those are outside of the public system, especially if not involving psychiatric care, but there are some, there are also some other free resources provided by the government beyond the system and work/school benefits cover some of the others.

I get to mentor med students! It’s a wonderful program that many medical schools do where people with chronic conditions teach the students how to be good doctors to people like us.

Question (8): Regarding our theme for this mental wellness month: Speaking Up And Stigma, what instances have you faced stigmatisation from individuals and society at large?

There has been a lot of issues with the assumption I am just anxious or stressed, both before and after diagnosis. There is also a lot of stigma associated with IV use. There was an internalized stigma, particularly when my symptoms weren’t severe, but the fatigue persisted. I sometimes would wonder if I was being lazy, especially when I had quit school and wasn’t working.

Question (9): Define who you are using 5 adjectives.

Stubborn, loyal, intelligent, caring

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Question (10): What interests/goals do you have if you are comfortable sharing.

I have always had an interest in science, especially medical science. : Shortly after being diagnosed I started gardening which I really enjoy. I would like to learn canning.

Question (11): What would you say to people who may be experiencing difficulties dealing with genetic conditions such as HAE?

1. It’s a steep learning curve, but so worth it to put in the effort to learn. You will be the expert - with someone who really specializes in the condition you are the expert in how it’s affecting you. When dealing with other doctors, well, there’s a good chance you will be the expert in the details of the condition itself, even if it is something that’s more common or well-known.

2. Connect with other patients and organizations, they will really help with the above and other things! If it’s so rare that those groups don’t exist, seek out broader using key terms like rare, an organ or system, etc. Sometimes you find the specific people within the broader communities.

3. Take care of your mental health! Sometimes the medical is far too draining to throw in more things to manage, and that’s ok. Just take out a bit of time to destress and as things settle seek out some of the outside mental health resources. There’s typically stress, grief and sometimes trauma involved.

4. A normal will be found! It may look very different from what you had planned. Symptoms may or may not be reduced. Things will improve though as you learn what to expect and how to work around them and you will find new normalcy.

Question (12): Is there anything else you would like to include? (resources, stories, etc)

The HAEA has some great information. If anyone has recurrent swelling episodes I would definitely encourage them to keep up with getting it investigated until diagnosed, as there is chronic histamine based swelling, MCAS, acquired angioedema (similar to HAE), etc. If people have more questions about HAE they can ask me :)

Wow, thank you SO much for your knowledge on the Hereditary Angioedema, I feel you are a wonderful educator and the way you inform others is astonishing. I've seen your work here on 7Cups and I am forever astounded by what you do for this amazing community. You inspire me to reflect on my life and realise I have to be appreciative of what I have and remember the value of being kind because you never know if someone is going through pain. I also want to thank @MynameisNicole for her hard work, especially with the interview series, a wonderful person and I cannot wait to see how we evolve as a team and a community by spreading awareness throughout the website.

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