HAE - Many Faces, One Family
I often talk about having an 'immune disorder'. Well I have HAE along with significant food and environmental allergies. Just what is HAE and why is it so difficult to live with and what does it have to do with allergies? HAE stands for Hereditary Angioedema. It affects about 1 in 50,000 people and in the US it is estimated to affect about 6,000 individuals. It is as it states, hereditary and it causes swelling of various parts of the body often for no apparent reason at all (though some have a 'mutation' form in about 10% of cases). While many individuals with HAE might have 'triggers', many have no reason what-so-ever to cause their 'swells' to occur. We often refer to ourselves as 'puffer fish'! LOL! (yes, we can 'joke' about ourselves!) The most common places for these swellings to occur are the face, throat, arms, legs, or abdomen. I tend to get them in my face, legs, and abdomen but mostly the abdomen (which you often can't see, but then again I can look 9 months pregnant when I'm not). (see photo before and after!) This can become life threatening if it attacks the throat.
One of the biggest triggers for most with HAE is hormones. Women are most affected by HAE because of estrogen (the female hormone). In fact it use be known as 'Hysterical Angioedema'. Conversely, the male hormone testosterone is often a treatment for it but can cause huge side effects in women as well as men. I was treated with the androgen's when I was first diagnosed and had horrible side effects. I was only on it for about a month and was moody, emotional and had significant effects on my liver. My entire life while I was on every type of hormone I had complications taking everything I took. Additionally, every time I was pregnant I was sick, very, very sick. There is a reason my girls are nearly four years apart! I was advised with my youngest to never get pregnant again. At that time we never knew what the problem was but it all makes sense now. Another trigger is soy. Soy has estrogenic properties to it and can really make things difficult for those with HAE. It is also one of the top 8 allergens. Yet, many, many foods contain soy! Talk about making it difficult to find food to eat! I can't eat at certain places - ever, because they use soybean oil just to cook with! Soy Lecithin also is used as a 'buffer' or 'binder' in many, many foods and while the FDA states that 'most with soy allergy should not be bothered by this substance' many of us with a true allergy are most definitely 'bothered' with it.
HAE is NOT an allergic reaction. It does NOT respond to allergic reaction medications like antihistamines, epi-pens, or steroids (though many physicians try to treat us with those!). It just doesn't work. Many HAE patients have died waiting for proper treatment trying to TELL the physician this and they refused to listen to them. I had one occurrence in an Emergency Room telling the physician what I had and was told "I'm not treating you for something you don't have." I was already diagnosed, I just didn't have the proper medication prescription yet and had been taken off the androgen's due to the side effects. This just isn't acceptable. Education of this disease is imperative to save lives!
Additionally, many of us with this also have significant food and environmental allergies (which is why they often can 'confuse' us as having an allergic reaction. I have some of the worst there is. One immunologist I visited asked me 'What aren't you allergic to?' He was actually being quite serious. My list of what foods I CAN eat is far shorter than what I can't eat. Eating out became a nightmare let alone shopping for food (I now pack my lunch almost every day). Our families entire way of living had to change. This was nearly five years ago but became more intense when we found out about the HAE last year. I will never forgot going to the grocery store that first day just trying to find a spaghetti sauce that I 'could' eat (from a jar) and there wasn't a single one! Not to mention the pasta! I couldn't find anything that I could buy!! I came home and just cried and cried. I became seriously depressed that year. If it wasn't for my girls getting me involved in learning to cook all over again by watching 'The Food Network' and 'The Cooking Channel' I don't know what I would have done! There are many, many things that I simply can't avoid (environmental allergies like grasses, weeds, molds, etc.) and I still have to take antihistamines every day. But, it is far better than it ever was. But why is HAE and allergies so closely together? Well, they are both on the same 'cascade' in the body!! There is a part of the immune system known as the 'Complement Cascade' and HAE is the first complement (it affects C1 Esterase Inhibitor)! Allergies come into play a little bit down the line and if the complement system is 'broken' the rest can be affected.
HAE has three 'types' as well. The types really don't matter except when it comes to treatment and how 'insurances' treat you as well as how you are diagnosed. It is quite complicated and far too long to go into this short post. The best place to get information from is the Hereditary Angioedema Association. They are fabulous at helping people with diagnosis and treatment options! I don't know what I would do without them!!
I now 'can' (yes, that's right, old fashioned canning!) all my own foods including condiments, fruits, vegetables, meats, sauces, soups, stews, you name it! I make my own spices (obviously as I sell them!), make my own soaps, lotions, creams, and eat the healthiest life I have ever lived! Even sulfites in shampoos, soaps, toothpaste, etc, I have had to get away from. If I get something in my diet that is a 'no-no' I pay for it dearly! Sometimes it can be immediate but other times it can be 2-3 days later. I just can't risk something being in food that I can't have. Food manufacturers do not have to completely 'list' all their ingredients. Spices often only have to list 'spice' and when you're allergic to many spices, this poses a problem.
HAEA! Help us find a cure!