Diagnostic Years

Diagnostic years are absolutely exhausting! 

A diagnostic year is when new symptoms pop up, or a change is made, which begins a cascading effect. Other, stable, illnesses flare-up, long-suppressed symptoms make an appearance, and my schedule is filled with appointments trying to figure everything out. It is necessary, it is incredible to be able to get the appointments I need, and it is entirely draining. 

In September 2022, a medication change was made. Within weeks, my body swelling was entirely unmanageable, my intestines began bleeding severely, so my diet had to change drastically (potatoes, eggs, and fluids only). We also found that my thyroid was way out of normal, and had to change doses of medication for that. 

I also gained 30lbs within 3 months - some due to swelling, some potentially due to my thyroid, but there's still no answer for the remaining weight gain. (Stress? Age?)

In February 2023, I developed tinnitus and sudden hearing loss in my left ear. I also developed some moderately severe dizziness/vertigo, some nausea, and increased migraines. I was put on steroids for potential Labyrinthitis, but the tinnitus and hearing loss remained. 

In the summer of 2023, my eyes worsened. I had developed Geographic Atrophy (worsening Macular Degeneration), then suddenly developed double vision, added dizziness, and eye strain as a product of the double vision. It was also discovered that have the very beginnings of Cataracts.  

So all of last year we were: 

• trying to get my thyroid back to within normal limits

• trying to find the source of the intestinal bleeding

• trying to find out why I developed tinnitus and hearing loss

• trying to figure out why I developed double vision and how to try and correct it

• trying to figure out why my weight hasn't normalized

On top of all of that, I went through an episode of Depression after losing my soul-dog Decker, among other additional emotional stressors. 

We were also trying to figure out our little mini-aussie Comet - who has been having medical issues. Lots of physio appointments for him, diagnostic tests, and physiotherapy exercises at home. He has an appointment with a neurologist next month. 

Safe to say - 2023 was an excessively draining year. 

Now here's what we've figured out so far: 

• My thyroid is finally back to within normal limits (January 2024 bloodwork was the first normal thyroid result since early 2022). 

• Still no explanation for the intestinal bleeding, but it had improved by December 2023, after a year of potatoes and 5 months after re-adding the medication that had been removed in 2022. 

• The Macular Degeneration has created a scotoma (blind spot) right at my fovea (central focus point) in my left eye. So I developed what's called Dragged Fovea Diplopia Syndrome. Essentially, my left eye is displacing the central image, causing double vision. My eyes are trying desperately to connect the two images, causing strabismus. The double vision cannot be corrected, but there is a potential that it will subside when I lose all of my central vision in my left eye. 

• I have developed a nystagmus, likely from inner ear damage, causing uncontrolled eye movements and additional vertigo/dizziness. The likely suspect is the Labyrinthitis in 2023. I will be starting vestibular physiotherapy to try and correct it, although because it's been over a year, the doctor isn't overly optimistic. 

• It looks like I might have the beginnings of something called Otosclerosis in my left ear (based on a CT scan report). I am still waiting to see an ENT to find out for sure, to discover if I am a candidate for surgery, or if I will simply use a conductive hearing aid for the time being. I will need additional scans in a few months and will need to keep monitoring my tinnitus and hearing loss. 

I still have a lot of appointments coming up, but it seems like the investigations are almost at an end. 

So now my body feels like I ran a marathon every day for the last year. My body is depleted and drained and I am perpetually exhausted. 

Diagnostic years are like being stuck in suspended animation. So now that we have figured out everything that we can, my body can finally relax. So as much as I want to do things like work out, go for more walks, enjoy the nicer weather, do more things at home, my body is telling me to sleep. 

I feel like I could be in and out of sleep for a week or more. 

Sometimes You Just Have to Laugh About It

I've been struggling lately with some very annoying (and often mildly-moderately painful) symptoms since coming off of a medication. 

I am off of this medication not because it doesn't work, but because there is a question as to WHY it's working, and trying to find the root cause of the symptoms. And part of trying to find the root cause of a set of symptoms requires actually being symptomatic as we are investigating. So if I take the medication, then the symptoms aren't apparent, which makes it hard to investigate. 

So I am currently, and willingly (mostly), symptomatic - to help find out what is really wrong with me. (Goddamn chronic illness is so extra complicated!!!) 

So - I am off of this medication. 

This means that my symptoms are much more pronounced AND much more frequent. One of the many symptoms that I get is swelling. Some of this swelling looks like bloating, but has an entirely different quality to it. Some doctors say that it feels like marshmallow skin (like the Staypuft Marshmallow Man!), some say it's like I've turned into a water balloon that's been overfilled, but there is one very important consensus - it is not normal. It is not normal for regular run-of-the-mill bloating. It is not normal for swelling related to hormones. It is not normal for extra gas in the intestines. It is not normal for leaky blood vessels that are leaking water into my tissues. It is not normal for swelling related to Diabetes (which I do not have, but have been tested for in the past, just in case). Doctors and Specialists are all stumped. 

But one very clear response is that when it happens in my abdomen specifically, I often look pregnant!! 

So this weekend, when this happened, a friend took a picture of me standing as if I am giving a pregnancy announcement, by a sculpture we imagined to be a fertility idol. We laughed about how funny it would be, showing this photo, and claiming that some sculpture created this miraculous conception (which is impossible because I no longer have the organs necessary for such a thing). 

So here is my faux pregnancy photo - making fun of my crazy body and laughing about these insane symptoms, instead of feeling too stressed, overwhelmed, or angry. 

Sometimes you just have to laugh about it - because what else are you going to do? 

Swelling Disease Update

Lots of updates happening lately.

Here is the current update of the swelling disease/disorder that I have. 

Since the end of 2010, I have had unexplained swelling. At the time, I was on Remicade for my Crohn's, I had an allergic reaction to the Flu shot (because it was cultured in Gentimycin), and I was having drastic swelling episodes. In December I was told to avoid eating much of anything (and to stick close to the ER) over the holidays until I could see an Allergist in the new year. So, in 2011 I saw an Allergist (no common food allergies), I was removed off of Remicade, placed on continuous anti-histamines, and then also saw an Internal Medicine Specialist. 

At that point, Remicade was obviously a suspect, but they also did bloodwork for C1-Esterase Inhibitor Deficiency, which came back normal at the time. 

Once removed off of Remicade, the outward swelling improved, but did not go away entirely. 

In 2012, a whole ton of medical complications happened, including the emergence of the Ankylosing Spondylitis, and my inability to work. For much of 2011 and 2012, then until 2017, I began having these insane cyclic vomiting fits, once a month coinciding with my menstrual cycle. I would suddenly start vomiting - and then you could set a clock to it. It would be every 20 minutes for HOURS. The longest was 10 hours straight, the shortest was 6 hours. 

With my history and everything I have already dealt with, I rarely sought emergency medical services during these puking fits. I mean, really, it was just vomiting for a few hours and then it would settle down. Often, by hour 4, I would *think* about going in and I would make a mental plan to go in if it lasted another 4. I figured it was simply my body's way of handling my period (which, it was, but was also something more). I had categorized these episodes as just part of my regular life - so it took until 2014 to be 'flagged' as a serious symptom that should not be occurring. 

That year, I saw an Internal Medicine specialist at an Urgent Assessment Clinic - he got the ball rolling on several fronts. I was seen by a Dermatologist, another Internist, an Internist/Hematologist, and finally a Hematologist. At that time I was losing weight constantly (my thyroid was incredibly overactive and we didn't know), I was experiencing tremors, I was using a cane/walker constantly (and then a wheelchair for a few years), continued having the vomiting fits, AND having swelling episodes. So I was always losing weight but then swelling everywhere - internally and externally, and had multiple reactions to medications. 

Once I saw the Hematologists, the general consensus was that I had Hereditary Angioedema - a genetic defect that caused swelling in my tissues. The initial thought was HAE III - which is often associated with female hormone cycles, and still a C1-Esterase Inhibitor Deficiency. Bloodwork came back variable - sometimes it was really abnormal, sometimes it was within normal limits. My body, again, was confusing. 

With the episodes being so clearly linked to my menstrual cycle, I was placed on Lupron - which prevented me from having my period monthly. My vomiting fits were reduced, but always returned, accompanied by breakthrough bleeding. I was also still experiencing outer swelling, internal throat/chest swelling, and bladder swelling, with various triggers (some predictable, some not). 

In 2017, I had a full Hysterectomy & Bilateral Oophorectomy. It is now 2023 and I have not had a 6-10 hour vomiting fit since the removal of those organs (which were removed for other reasons as well). 

The body-swelling, however, continued, relentlessly. My Hematologist tried me on a few different medications, and then eventually placed me on prophylactic C1-Esterase Inhibitor plasma. 

This was phenomenal. I have said before that it was a miracle drug for me. The body-swelling reduced significantly - to the point where I hadn't even realized how much swelling I was experiencing every single day. Parts of my body that I had dismissed as general fat or a product of other medications were suddenly not puffy! I still had episodes where I needed further IV treatment, but it changed SO many aspects of my life. So the agreement was some form of HAE. 

Last year, we finally got access to genetic testing to confirm a diagnosis officially. She had originally thought HAE III, but then her thinking shifted to HAE I or II instead. This genetic testing checks for some of the mutations associated with type I or II - so that bloodwork was ordered. 

It came back negative. 

She had always felt like my symptoms were a bit atypical for HAE, so she wasn't all that surprised. (My body's symptoms have always been a little atypical for everything - it's a weird one and doesn't like to fit into a neat little textbook-defined box). The variable bloodwork, the frequency and type of swelling, and the obvious beneficial response to the C1-Esterase Inhibitor plasma were confounding. 

Due to all of this, she wanted to try taking me off of prophylaxis in the fall of 2022. 

My body-swelling has been volatile. My body doesn't feel like mine. I am perpetually swollen, feeling like I live in a fat-suit, my clothing doesn't fit properly, and I have zero control over it. Some days I can go from a relatively flat stomach to looking 6 months pregnant within 3 hours, or I can put on a bra in the morning and, very literally, bursting out of it only an hour later. It is incredibly frustrating. 

She wants to start from scratch - she is concerned that HAE might not be the correct diagnosis and wants to try and figure out exactly what this is. She is not ruling out HAE III, but she wants to investigate to see if there is a different immunological condition or Complement System malfunction that better explains how my body behaves. It is obvious that the plasma helps, and she wants to find out exactly why it helps. It *could* be that my C1-INH isn't functioning properly, but it could also be the anti-inflammatory properties in the plasma, or some kind of rare allergy that could be settled by that medication. Either way - for some reason, my blood vessels are leaking water into my tissues - all the time. 

And none of us are truly confident that we know why. 

So we start investigating again. 

We may come to the conclusion that it is HAE III and that the plasma really is the best medication for it, and if that's the case she'll put me right back on it. If we come to a different conclusion, however, then there may be an even more effective treatment! 

Tomorrow I see a new Internist to discuss possible options and tests - trying to discover what kind of immune system malfunction my body inherently exhibits.

Did my immune system simply not develop properly? Is the communication within my body so inhibited that none of my systems will ever function well? Is there a hiccup within my Complement System that is creating all of these issues? Do I have a genetic mutation for HAE that cannot currently be tested? Or do I have some rare or yet-to-be discovered disease that has caused my immune system to attack various organs and mimic all these different diseases? 

Who knows?! 

Stay tuned! 

The Lighter Side of: Weight Fluctuations

Weight can fluctuate for an entire list of reasons...
Changes in eating habits
Medication
Hormonal Changes
Illness
Changes in activity
Stress
Symptoms (like vomiting, swelling, bloating)

This type of drastic change is more frustrating than anything. Large weight fluctuations can be unpredictable, uncontrollable, and annoying.
With medications like Prednisone or other corticosteroids, weight gain is almost inevitable (mostly water weight). With a disease like Hereditary Angioedema, swelling can cause fluctuations in one's size from day to day or even hour to hour depending on the location of the swelling. Hormonal changes can cause severe weight loss or gain, thyroid problems can do the same, and significant increases in stress levels can reduce or increase appetite.
The most frustrating part is the lack of control. For people with various illnesses and on various medications or supplements, no matter how well you eat or how much you exercise, there is only so much that you can control.
Not only that, but trying to find clothing that fits at all stages is difficult and can become a financial burden.

The Lighter Side of weight fluctuation is that it gives me an excuse to go clothing shopping every now and again.
These days, you can find clothing that is very versatile. I have pieces of clothing that fit me at nearly any size. I also have 'Prednisone Pants' and a closet full of sizes from 0-14... clothing I had to buy during weight changes but only wore temporarily. They are all in good shape and are all classic pieces, so when my weight does fluctuate I at least have a few staple options.

There is another positive side - Facebook Auctions.
You can find auctions everywhere online these days. My favourite auctions to watch are the Fb low-starting-bid auctions. I have been able to find a great deal of clothing for under $10, all in excellent used condition and even some still with the tags attached.
I used to be nervous about wearing the clothing of others, but the pieces I have bought have been in 'like new' shape or with the tags on. You quickly wash the clothing before wear and, voilà, good as new!
I would love to organize a clothing drive for people who have undergone severe weight changes due to illness.
There are so many of these auctions all the time online - people wanting to rid themselves of excess stuff. Clothing barely used or entirely unused. I wish I had the energy to run something like this - but maybe one of my readers would be willing to get something like this going?

Essentially, I was thinking of doing a clothing drive to collect like-new or new with or without tags level of clothing, separate them into their respective sizes, and then people can submit applications to receive a bag of some staple clothing in their current size. Obviously it would be limited, so only a certain number of applications could be fulfilled, but I think it would be a wonderful option for people who have undergone drastic body changes and require some clothing in a new size for the short-term. Then they could always donate back those items once their weight returns to normal.
It would be much better than having to fork out money for an entirely new wardrobe if those weight changes only last for 3-6 months or even a year or two (like courses of Prednisone).
It could be as straight-forward as having a drop-off bin location over a period of a month (or however long), then organizers wash and separate the clothing into sizes, receive and organize applications, and fill the applications as they come in.

It would be a much larger undertaking than a simple online fundraiser, but I keep thinking about how much money I have had to spend over the years for severe weight changes and not fitting any of my wardrobe for several months. It would have been so much nicer if online auctions were popular at that time. My weight changes were so common and so bad that our family wound up foregoing vacations because of how much money was being spent on medication, treatments, doctor's appointments in different cities, and clothing to fit me all year round.

With online auctions, there is an easier way!!

HAE Day

Today was World HAE Day!

Hereditary Angioedema (HAE) is a disease that causes severe swelling episodes. The most common areas of swelling include limbs, face, intestinal tract, and airway. These swelling episodes can be triggered by any minor trauma, or by absolutely nothing at all.

So what does that mean?

Any bump, hit, injury, bruise, or break can cause swelling.
Any emotion that increases adrenaline: sadness, anger, excitement, elation, frustration, nervousness, or fright can cause swelling.
Swelling can also be induced from NOTHING AT ALL.

If you swell in your airway, it can cause an obstruction, mimicking anaphylaxis, but not resolvable with epinephrine. In fact, using an EpiPen during a throat swell could actually make things worse.

You can swell anywhere.
I mean - anywhere.

Anywhere on the skin, head to toe, and anywhere on the inside! Intestines are a common spot, bladder, pancreas, esophagus, trachea, vocal cords, muscles, kidneys, tongue, reproductive system.
Anywhere.

To celebrate HAE day, my body wanted to remind me what an intestinal swell felt like. How kind of it.
I suffered a partial blockage on Sunday evening, which primarily affects my Crohn's Disease. After the blockage was resolved, however, the pain and nausea only increased. That partial blockage is a type of trauma. So my body decided that it was time for an intestinal swell (the first major one since I was put on prophylactic blood products twice a week).
How timely of it to happen the weekend before HAE Day!

An intestinal swell is impossible to see on the outside. You cannot see the unbearable cramping, the pain, the level of nausea, the desperation to vomit, the near loss of consciousness solely from pain.
Swelling that is putting pressure on other internal organs, closing up the space in the intestine itself - limiting the area where food can pass through.
With swelling and already established inflammation, this means I could feel everything moving through my intestines. It burned like hot coals scraping through.

After 6 hours of pure agony, I knew for certain that it WAS an intestinal swell.
That is the other issue with internal swelling - especially when you have multiple illnesses. It could be indigestion. It could just be my Crohn's. It could be food poisoning. It could be the flu that is going around. It could be that I ate something, unwittingly, that I am allergic to. It could be a million different things... so I usually wait until I have violently vomited in three separate sessions before assuming it is a swelling episode.

So we call an ambulance and get me to the hospital, where I am treated for an HAE swell, for dehydration, and for one of two viral infections that have been going around *just in case*.
The issue was taken care of, but that does not mean that I suddenly feel like a million bucks!

Swelling episodes take a lot out of you. The amount of energy that the body expends to vomit, alone, is staggering. My body is fighting several of my own organs daily, trying to fix my back while simultaneously breaking it down, causing inflammation in various sections of my body, and then fighting with a missing link (the missing protein in my blood: C1 Esterase Inhibitor)! So my body swells up. Now it has to work 5x as hard to digest food, to vomit, to move, to stay awake, all the while fighting me every single step of the way.
I pulled some intercostal muscles (in between the ribs) from vomiting.
My eyes are bloodshot.
My esophagus and throat are raw from the acid and the force of vomiting.
My stomach is sore. (I sure got an ab workout though!)
My back is sore.
My legs, my arms, my eyes are sore.
My head is pounding.
My mind is fried.
My energy is so much lower.

This happened on Sunday and only TODAY (Thursday) am I finally seeing the depth of after-effects.
My intestines are bleeding.
My intestines took such a beating from this episode - they are so torn up and raw from everything that happened - that they will be bleeding for days.
I have medications to help with this, of course, but this is the level of trauma that one swelling episode can cause.

From a mushroom that kinda got stuck while being digested.

A MUSHROOM.

Hereditary Angioedema is so much more than just external swelling. It is true that many of the episodes affecting the extremities are not painful. Some people with HAE never experience painful swelling.
Unfortunately, swelling of the internal organs is not only painful, but can be extremely dangerous. It can even be fatal.

Projectile Style

It still amazes me how much one day can affect my body. How much one THING can cause such a chain reaction.

On Saturday, a friend had a table at a craft market and I wanted to support her. Another friend and I decided to go.
I wasn't sure how big the market would be, so I used my wheelchair so that I wasn't severely stressing my inflamed joints or affecting my energy levels.
After the market, I headed on home, cuddled with the dogs and played cards with our neighbour kids.
Later on, neither of us wanted to cook, so we went for a short pub-date.

This is where things went wrong.
But I didn't find out until the next day.

On Sunday I felt awful. It was understandable - Saturday was an eventful day, so it was normal for me to be down and out.
By 7pm, I started feeling a lot worse.
My skin got all clammy, sweaty, and I suddenly got goosebumps all over my body. The upper gastric pain started, the nausea crept up, and I knew I would be vomiting. I went into the bedroom and tried to fall asleep, but the pain and nausea kept me up. When I hit the washroom, I knew what the problem was.
I had had a few mushrooms.
Mushrooms can cause blockages, however rare. If they are not fully cooked, they absorb water and expand in the intestines.

I have had partial blockages before, so I knew it would resolve, but that didn't make it any less painful. This one was also worse than previous episodes.

I knew I was going to vomit.
I needed to vomit.
I wanted to vomit.
It took 2 hours of severe pain, horrible nausea, and being hunched over a bucket while in the washroom before my body finally expelled everything...
Projectile style!
Everything I had eaten Saturday night AND all day Sunday. So so gross.

15 minutes later, I didn't feel any better.
That's a bad sign.
I tried to take medication to help, I tried to relax, and I ensured the blockage resolved, but I still did not feel any better.
Another two hours were spent rocking back and forth wanting so desperately to vomit everything out. Then I had another vomiting fit - now just liquid.

This is the crucial moment. If this was it, then I could stay home. BUT - if I had one more vomiting fit, that would indicate an intestinal HAE attack.
So, when the third fit happened around midnight, and I STILL didn't feel any better, I knew I would need my IV treatment.
I could not fathom the drive to the hospital, sitting in a chair in the waiting room as I am having these fits while also stuck in the washroom, needing a commode, barely conscious, shaky, dizzy, and in so much pain.
So we decided to call an ambulance. They could start an IV on the way there to help settle things down, I would not necessarily have to wait in a chair, and things would get started more quickly.
So that's what happened.
I had another vomiting fit in the ambulance, and immediately afterwards they started my IV - within 10 minutes of driving.
It still took several hours in the ER, but the nurses and my doctor were phenomenal. I received fluids (I had lost just under 2000cc), anti-nauseates, pain relief, and then my Berinert. Then, just for good measure, they gave me a medication just in case I had contracted Influenza B or even Enterovirus. Both have been going around.
Personally, it was too coincidental for me, so I doubt any contagion, but I am always appreciative of extra caution.

Today I am super sore.
My throat, stomach, and intestines are just raw. Raw and torn up by acid. So today is a liquid diet and soft food kind of day. Oh, and lots and lots of rest, to prevent a relapse of HAE (which is common after such a major episode).

F*׿ing mushrooms.

An Unknown Symptom

It turns out that I have had a simple symptom for YEARS that I never knew was a symptom.

Over the years, with any singing competition or performance, for whatever reason I would often wake up and not be able to control my voice as much. I used to describe it as 'my voice just isn't working today'. It was almost as if I had developed a cold overnight that went away the day after any big singing gig. All of a sudden, songs that used to come so easily to me, seemed so much more difficult. I had less control, less power, less range, and had a much tougher time breathing properly in order to pull off phrases or trills like I normally would. My chest voice would suffer, my falsetto would sound much smaller, and I simply could not achieve the openness I am used to on a regular basis.
At first I simply believed it was my nerves getting to me - and it is entirely possible that some instances were primarily nerves. Not every single performance though. Not the performances from the comfort of my own home. Not performances I have done time and time again.

Now we know why!
Not only that, but it is a relatively easy fix!

It should have struck me a couple of years ago what it really was. It is partially nerves, but not in the same way we think about nerves.
One day, I thought maybe my HAE was acting up. Maybe my nerves are actually affecting the physical openness of my throat because of swelling from Hereditary Angioedema.
So, one day, I decided to do a home performance on a day where I did my regular prophylactic injection.
Bam.
Total openness. No issues controlling my voice.
The next time I did it the day before my medication.
Awful time with breathing properly and singing with an open voice.

This past weekend I was honoured to sing for the Crohn's Colitis Canada Fun Money Casino Night.
That morning I could feel a slight twinge in my throat and a little bit of tightening. I practiced and did some vocal warmups but nothing helped. So I made the executive decision to do an extra prophylactic treatment that afternoon before the performance.

That was the best my voice has been in ages. Entirely open, easy to control, my regular range, MY VOICE! My voice was normal. I had no surprises, no tightening, no issues breathing or finishing phrases. I was so happy with how I was doing that I did NOT want to stop. I kept on going and going and going until the rest of my body just could not physically take it anymore. I wanted to keep singing for hours more!
If my joints and general energy hadn't kiboshed it, my voice could have lasted for another hour. It was pretty hoarse afterwards and the following day, but not tight.

Then I remembered all of my failed performances - all of the mistakes. I thought of all of the times in University when I felt like I just could not open up my voice anymore, or when I ran out of breath so much faster than normal.
Obviously, like anyone else, some of those mistakes are entirely being too nervous and being unable to focus (like forgetting words, missing a cue, singing the second verse first), but I also wonder if some of the other odd mistakes could have been prevented by a simple injection.

Really interesting!
It is even more interesting to look back on all possible swelling episodes: intestinal, pancreatic, bladder, legs, arms, abdomen, face. Crazy to think of how much could have potentially been explained by this strange blood disorder that I have.

Stepping Off the Scale

Although it is fairly normal to have a scale in the home, when you have diseases in the digestive system, ones that increase water weight, or cause swelling, having a scale is kind of a moot point.
Of course it's nice to know an approximate weight, but there isn't much of a point putting faith in something that changes drastically.
When I was younger, my weight could jump 10-15 pounds within a 16hr period. At first we thought it was just the prednisone, but in hindsight it was the Hereditary Angioedema causing swelling and causing havoc in my weight.

There is no stability in weight with chronic diarrhea and swelling episodes.
So why focus on a scale?

Another reason why chronic illness can be a blessing in disguise. Why focus on something that you can't even know for sure is correct?

Allergies

In past posts I have mentioned being allergic or intolerant to various medications. I wanted to explain a bit about what that means and what the difference is between an allergy and an intolerance.

The definition of an Allergy:

a damaging immune response by the body to a substance, especially pollen, fur, a particular food, or dust, or medication, to which it has become hypersensitive.

The definition of an Intolerance:

abnormal sensitivity or allergy to a food, drug, etc.

Since that may not be clear, an allergy is an immune response and is usually measured by anaphylaxis, rash/hives, difficulty breathing, or swelling. There are many ways allergies can present, but there is a focus on the most prevalent.
An intolerance is an unusual adverse reaction that deems the patient unable to eat or take a specific substance.
For example, an intolerance to Advil could be a severe stomach bleed. It may not be considered an allergy, but if a patient is susceptible to this reaction several times over then they are advised not to take that medication.

An allergy is considered more severe and/or life-threatening, while an intolerance can be milder. With that said, an allergic reaction is not necessarily life-threatening and an intolerance can be life-threatening. It simply depends on the situation.
Good and confused now?
Great!

Sometimes, if a patient has an intolerance to medication or even an allergy to medication that is considered to be a mild reaction, if an infection or issue is especially severe, they may be asked to take it (with an antihistamine).

I have several allergies and intolerances.
They started within the first few months of being diagnosed with Ulcerative Colitis.

I was placed on Pentasa first. It is a 5ASA medication (related to Aspirin) and a common medication for inflammatory bowel diseases.
Within the first 3 doses I became severely ill and was hospitalized with severe pancreatitis.

The next was Salofalk - another 5ASA - same reaction.

Then we tried Immuran.
Hospitalized with severe Pancreatitis again.

6MP. Same story.

So we resorted to Prednisone on and off for several years along with Methotrexate.
Nothing could control the UC, which is why I wound up with an emergency surgery to remove my large bowel.

Over the years I have had some pretty major (and some strange) reactions, including the pancreatitis. With the Pentasa, the pancreatitis reaction was initially called an intolerance. Once I had several hospitalizations for pancreatitis as a reaction to various medications, it is now deemed an allergy.

Ampicillin - full body rash/hives
Gentimycin - full body rash/hives (one dose)
Erythromycin - full body rash/hives (one dose)
Biaxin - full body rash/hives
Cymbalta - pancreatitis

Remicade - severe swelling. This occurred before I was diagnosed with Hereditary Angioedema. Now that I have that diagnosis, we have found that biologics severely exacerbate my HAE and causes severe swelling. This particular reaction would be an intolerance, because it is not an immune response (Benadryl or epinephrine will not reduce this reaction or help). We have tried to use benadryl several times with biologics with no improvement on the swelling.

Simponi - instantaneous throat swell

Enbrel - body swelling

Humira - body swelling

Cosentyx - body and moderate-severe throat swelling

Cimzia - body and moderate-severe throat swelling

Toradol (related to Advil/Aleve etc...) - severe stomach bleed.
Oxycodone - severe stomach bleed. (These two in particular account for one of THE most frightening situations I have ever been in. Writhing in pain with 7 nurses in the room trying to get things under control and prevent me from dying).
Percocet - stomach bleed.

The Hereditary Angioedema confounds the issue of allergic reactions. A throat swell reaction could be either an allergy or an triggered HAE attack. The problem of not knowing is that a shot of epinephrine could actually make an HAE attack worsen. If an antihistamine is given and does not work, then it is a clear sign that it is an HAE attack... but when your throat is swelling up to the point where your breathing is affected, do you really want to wait to find out which is which?

I also have major reactions to both Caffeine and Chlorine, and a severe intolerance to Star Anise (and Jägermeister).

The reactions to both caffeine and chlorine is improved by taking an antihistamine. They both result in some hives in small doses and a throat swell in large doses. So I stay entirely away from caffeine (unless it is in one of my medications that I cannot avoid, in which case I chase it with an antihistamine). Chlorine is nearly impossible to avoid (unless I decide to bathe in lake water all year-round or in expensive filtered bottled water). I am also a water-baby and love to swim - AND swimming helps reduce my joint pain - so any time I want to go swimming I simply take an antihistamine and limit my swimming time to 20 minutes or less, and only go once every few months. 

With Star Anise and Jäger I have had instant vomiting reactions with blood. Even in accidental exposures and several times over.

With so many odd reactions, strange allergies, a disease that gets triggered by entire classes of medications, and various intolerances, no wonder it has been difficult finding medications that work!

Plus, even though I do carry an EpiPen for my allergies, I also know that using it could cause a severe throat swell due to HAE. Luckily I have never been in a position where I needed to use it... but it sits there in my purse as always.

Now with this new adverse reaction of migraines to prednisone/cortisone, I am pretty worried that another anti-inflammatory medication may be cause for concern.
With all of these allergies it also makes natural products even more elusive. We often do not know all of the ingredients, or may not recognize the familial ingredients. For instance, star anise is related to fennel. Aspirin is acetyl-salicylic-acid (ASA), which originates from Willow Bark - so anything with willow bark or salicylic acid (or any derivative) is a no-go. This means many natural remedies, many skin-care lines, and over-the-counter medication. Caffeine is also in many skin-care products and is found in various foods and natural products.

It is an interesting labyrinth navigating with so very many substances to watch for.

Being Dramatic

I was diagnosed with Ankylosing Spondylitis in 2013. Around eight months earlier is when I first started experiencing symptoms.
My hips were in pain, my shoulder blades felt like they were grinding metal on metal, and I was having more and more of a tough time playing volleyball. I was experiencing a severe increase in back spasms and a general reduction in my ability to be active.
So I started seeing a chiropractor on a regular basis. I saw an acupuncturist and had her do that as well as cupping. My muscles were in constant contraction and my hips were out of place, so I started wearing an SI belt. I began physiotherapy as well.
I was trying my hardest to get things sorted out and wound up averaging approximately 10 appointments per week, many early in the morning before I went to work.

After several months of this regimen, I knew it was something bigger. I eventually saw a rheumatologist who thought it might be a type of arthritis.

So, when I was at work one day, wearing my SI belt and limping badly, someone asked me what the doctors think that it is.
I explained that it was possible I developed some sort of arthritic condition.
My colleagues stated that I was being dramatic. They said that there was no way I had arthritis, that I was exaggerating and that I was too young. I was being a drama queen.

I can see where they were coming from.
Young girl, mid-twenties, highly active (volleyball, softball, gym) who all-of-a sudden develops joint pain and says it might be arthritis? Even I would be skeptical.

They were right. I did not have generic arthritis. I had something worse. I developed an autoimmune arthritis - a spondyloarthropy. (This is not to be confused with osteoarthritis or rheumatoid arthritis - which can be even more severe). Instead of the pain coming from bone on bone friction and experiencing pain and swelling (which is incredibly painful on its own), I was experiencing the effects of my body battling its own joints.
My own immune system is attacking my own joints - causing pain, swelling, inflammation, but also breaking down the joint matter and causing lesions and erosions. Then, my defense system kicks in and starts rebuilding that matter in the wrong places. So instead of rebuilding my joint, it builds a 'wall' in between my bones and joints - fusing them together.
A vicious cycle of inflammation and erosion, following by reinforcing defense systems where they should not be placed.

I have been fortunate with the slow progression of the disease. So far the erosion is in my SI joints, I either have herniated or degenerated discs, and my upper spine has begun to curl inward, creating a permanent hunch (that no amount of yoga can reverse - but there is no harm in trying).

There are so many civil wars in my body that it is difficult to understand what all of my systems are doing. All this internal fighting creates constant pain. Constant pain. So much so that the combination of the new disease with Crohn's Disease, severe pelvic pain, the Hereditary Angioedema that was found shortly after the AS, Hashimoto's, tachycardia, migraines, and the general fatigue, lack of energy, and ridiculous recuperation times, all took me out of the workforce (begrudgingly).

Dinner Date

Last night I had a really tough time sleeping through the entire night.
After I attempted to get back to sleep, only to find myself staring - wide-eyed - at the ceiling, I decided to grab my sketch book and do a bit of sketching and writing until I felt sleepy enough to go back to bed.
Yeah, that didn't happen.
Once Rj woke up, I realized I was going to be staying awake.

So I suggested, since we were both wide awake, that maybe we use some of our gift cards and go on a day date.
So that's what we did.

There was only one problem - and luckily it was a small problem. I guess it was too soon after my Cimzia injection to do anything exciting. Shortly after we got to the restaurant, my throat became a bit tight. Not concerningly tight, but enough for my voice to change.
We quickly made sure that there were no surprise ingredients in our food that could induce a reaction (there wasn't), and then tried to take it really easy.

Now the question lies in whether or not I should continue with the medication.
This wasn't a dental appointment or an actively exciting outing. This included Rj driving to the restaurant and ordering food. I didn't have an alcoholic drink or anything either.
The excitement of going out for food - even after taking Benadryl and Prednisone with the injection - was enough to nearly induce a throat swell. What that means is that biologics are most likely less of an allergy and more of an intolerance from the Angioedemic illness itself. 
It did not progress, thankfully, so I was able to get home and take what I required to settle my system down.
Now for the next week or two I will have to be exceptionally careful so as not to induce another minor episode (or worse).

Was it worth it?
Always. Dinner was great, it was a nice date, we went to a restaurant I had not been to before, and we had gift cards so we did not really have to spend anything above tip.
Great afternoon. .

Went home to sleep and woke up about half an hour ago (just before 10pm). May have a rough sleep again tonight.
Oops.

Answered Questions

My recent 'Ask Me Anything' post got a bit of engagement.

My post:

"Ask me anything about Hereditary Angioedema".

The questions I have received so far:

• "I have HAE as well what meds are you on and are you on prophylaxis?"

• "How many times approximately do you swell a month?"

• "What places are your usual swell spots?"

• "Are there any foods that can reduce swelling episodes?"

Before I answer these questions, you are welcome to click here for a generalized and brief overview of what Hereditary Angioedema actually is. Of course, it explains only the very basics of this complicated disease.
The summary of it is that this is a Hereditary blood disorder. People with HAE are either missing a critical component in their blood, or that component is somehow not functioning properly or at all. There are three types of HAE, defined only because of bloodwork and the common triggers of attacks - the symptoms of all three types are virtually identical in nature. Although each person's experience is just a little bit different.
This disorder in the blood will result in swelling episodes. They have been defined as similar in a sense to hives, but underneath the skin. So it resembles an allergic reaction and the swelling can occur anywhere in the body, including organs, face, tongue, throat, extremities, abdomen, even the eyes, etc... Anywhere.

So to answer the questions:

1. "I have HAE as well what are you on for meds and are you on prophylaxis?"

I do take prophylactic injections of Berinert 2x a week, and then I receive IV treatments on top of my prophylaxis if I have any severe swelling. Severe swelling would include anything in the throat or face, any severe organ swelling, and any severe acute swelling that cannot be improved by sub-cutaneous injection. This treatment plan has dramatically improved my overall disease management.

2. "How many times approximately do you swell a month?"

Now, on the above treatment plan, I have minor swelling in my arms, legs, abdomen, and breasts, a few times a week. I am now only suffering severe swelling once every couple of months - the type requiring a trip to the ER for IV treatment.

3."What places are your usual swell spots?"

My usual areas of swelling are my arms, legs, abdomen, and breasts for my externally visible swells, and my throat, bladder, intestines, and occasionally (now) my pancreas for internal/organ swelling. My face swells once in a while, but I tend not to notice as much. Since prophylaxis, I do not get the deforming facial swelling (and even only had that a couple of times). Every so often I will notice a change in my face shape - and I think that if my face wasn't so small to begin with I would notice it much more.

Before prophylactic medication, my external swelling episodes could be so severe that my clothing would begin ripping at the seams because of how puffy I got and how much my body would expand.

4. "Are there any foods that can reduce swelling episodes?"

Sorry - as far as I know, the answer to that question is no.
If you are experiencing a swell in a particular organ, there are probably foods to avoid (like avoiding alcohol during a bladder swell, avoiding carbs during a pancreatic swell, and avoiding foods that don't digest well during an intestinal swell).
What I do find is that things like caffeine - anything that can induce a stress response - can be a strong trigger for episodes, so I would suggest avoiding those kinds of foods.
Unfortunately, because there is something missing in the blood, I am not aware of any foods that can mimic that vital component. Even the medications available are blood products to replace that component.

If any more questions roll in about HAE, I will add them to this post.
Thank you so much for your questions!!

Medication for HAE

We got married this year!! September 22, 2018. Beautifully perfect day, with no real problems other than the health complications we expected.
Perfection.
Really.

We are finally going through all of our engagement and wedding photos. Like most girls, I can often be my own worst critic and usually abhor specific pictures because of an odd angle, a few extra pounds, or a smile that's just a little off.

What really struck me, though, is the evident contrast between engagement photos and wedding photos.

When we got our engagement photos done, I was not yet on any medication to help steady my swelling episodes from Hereditary Angioedema. Essentially, I was perpetually swollen, everywhere. Shortly thereafter, I started on an HAE medication that has worked wonders for me.
So now, looking back at those engagement photos, it has proven to be difficult to love them. I am extra critical because I don't feel as though I even look like myself that much. Luckily for me, it kind of just looks like I lost a few pounds before the Big Day (hooray!). That's not the case though.
The photo on the left, in the super cute yellow dress, actually happens to be one of my favourites from the engagement shoot. My super puffy knees are less noticeable, and the blissful smile on my face brings more focus to the true happiness from being engaged to the man that I love. With that said, even my face shape is different. My arms are pretty puffy. It actually looks like I may have Prednisone swelling.
Overall, I simply look puffed out.

Which I was.

Fast-forward to the rehearsal - the puffiness has been reduced substantially. There isn't as much swelling concealing the fact that I have knees. My arms seem much more toned, my face shape has returned to its usual oval shape, and I am less self-conscious about my abdomen.
This medication has done wonders for the outward symptoms of this complicated illness.
Incredible the difference though - when you look closely.

The one very crucial point about both of these photos: I am HAPPY. I am smiling. I am enjoying my life and our relationship immensely.
And I love being married.

Treatment Day

It's treatment day!!!
Which means a painful needle to the gut.
So why am I excited?

It is amazing how someone can become accustomed to painful medical tasks. 15 years ago I was terrified of any needle - even bloodwork needles within the children's system.
Now look at me.
Injections into the gut three times a week plus any new medications to try.
Subcutaneous injections have become habit; routine.

This medication specifically has vastly improved the symptoms of my Hereditary Angioedema. Instead of being frightened to do anything at all around the house, should it send me to the Emergency Room, I am more free to just contentedly exist, with only huge stressors, big events, or allergic reactions, that trigger the awful throat swelling episodes. I no longer get 10-hour-long intestinal swelling episodes on a monthly basis, my clothing actually FITS because I am not swelling drastically throughout the day, and I can wear my rings without worrying about finger swelling!!
Of course, I still have minor swelling every day, and I have to be really careful about what I do and how stressed or excited I get at any given time, but now that I have the medication to counteract those swells, I am much less afraid on a daily basis.

So am I excited to give myself a needle?
No. I'm not. It hurt, it burns, I'm getting little pitted scars all over my abdomen, and I need to ensure there is always a comfortable layer of fat on my abdomen to do these injections. Jabbing a sharp metal object under my stomach skin is not exactly my idea of fun.
No. My excitement comes purely from the results that I get from this treatment. I always look forward to treatment day because this treatment has had a significantly positive effect on my daily life.

It may not be fun. It might even be downright gross or unnerving to some. It's not a fun process. But it helps. A lot.

The Cosentyx & Dentist Problem

For some reason, the first week after my Cosentyx injection, my body seems to be hypersensitive.
It is almost like my body is intolerant while the medication is fully absorbed into my body. Of course, it shouldn't take a week for it to absorb, so maybe my body is just pissed off for a week afterwards. Or maybe it has to do with something completely different.
All I know is that Cosentyx Week is touch-and-go. I always get that warning throat tickle for the entire week, sometimes longer. So, during that week, I try to avoid any other triggers of Hereditary Angioedema swelling.

Unfortunately for me, I have a lot of HAE triggers. Excitement, nervousness, physical activity, stress, dental work, etc.... the list goes on and on.
While my swells are contained, for the most part, to my insides and extremities, because of a miracle medication I take, sometimes I still get severe swelling that can be scary.
It can get really scary.

Yesterday I had a dentist appointment.
I had already postponed appointments several times this year, and I have a TON of things going on in the very near future, so it was kinda my last real chance to go to the dentist before the wedding.
I was still nervous to go. (Which, confounding enough, likely exacerbated the situation).
I got through it okay, but by the time I got home, my throat tickle was worse. Within half an hour my voice had changed and was hoarse. I quickly did an extra sub-cutaneous injection (this can often take care of impending swells).
Ryan was with me for all of this, so I had extra monitoring. (He is pretty great isn't he?)
We allowed the medication to work for 1.5 hours... but I got worse. Quickly.
By the time we reached the ER, my breath was 'catching' in my chest. It feels like a kink in a 'hose', I had audible shortness of breath, and it was too laborious to talk.

They got be in immediately, did a quick assessment, and started mixing the medication that I brought with me for IV treatment.
Then, again, halfway through the IV injection, my breathing was already slower. It continued to get better and, within minutes, there was no 'catching', way less hoarseness, my lightheadedness had faded, and the only thing left was the slight 'tickle' in my throat.

After these episodes it always amazes me how quickly this medication works. Of course, after an episode like this, my throat is always sore. It hurts - a lot. The back of my tongue hurts, my throat, my chest, my esophagus - it is all really sore.

They say that sub-cutaneous prophylactic treatment works longer, but that we only absorb 60-75% of the medication. With IV you absorb 95%+. It makes a difference! A big difference.
Of course I still do my prophylactic injection today - which is good because there is a risk of rebound.

While I do my best to stay calm and avoid making things worse, it can be really scary to have that much trouble.
I am lucky because I have warnings. Those warnings help me get treatment in time so that my throat does not become completely obstructed.
I am lucky that I had company to help monitor how quickly things progress.
I am lucky that I am already on medication, as this episode could have been so much worse if I wasn't already taking something.
I am lucky to live in Canada and have access to treatments like these.
I am lucky I no longer live all alone.
I am lucky to be feeling so much better after such a bad episode.
I am lucky for a lot of things.

Swelling from Excitement

With Hereditary Angioedema, any extreme emotion can trigger a major episode.
Anger, frustration, anxiety, panic attacks, being frightened, excessively happy, excited - pretty much anything.
Really, to avoid a lot of episodes, ya kinda have to try and stay neutral. Alllllll the time.
Basically it wants me to be a robot.
And then even if I AM neutral, sometimes I get swelling anyways. Usually it is from some injury or a flare-up of another illness, but either way, it has a complete mind of its own.
Overall my swelling is much more controlled due to a pretty incredible medication. It is a blood product, so I am grateful to anyone who donates blood, I am directly benefitting from those generous people.

I can look at it in many different ways ... but there are two particular ways that come to mind:

A) I could look at these episodes as punishment for being happy. That I am not 'supposed' to get excited or really feel anything. On one hand it just SUCKS to be careful not to have too much fun just so I don't have a severe episode that could put me in the hospital.

B) I could look at these episodes as signs. That these episodes are just flagging me to prove how these moments are important or memorable. On this other hand, it reminds to appreciate what caused the episode. That it obviously means a great deal to me.

Sometimes I don't think I am nervous about something but then I swell - so it points out that I am clearly a little nervous.
It can be a warning.
Most of all, if I swell because of a situation that was positive, it is PROOF that I genuinely loved that moment.

It proves genuine happiness in various situations.

That is how I want to try and look at it.

Dinosaur

Okay I have to give major props to my friend Carmen.

After reading my previous blog about my current pukey situation probably due to excitement triggering my HAE - she said that Hereditary Angioedema sounds like a DINOSAUR!!

I totally love it. It's so perfect!!

It's a beast
It is fascinating
It is rare
It can seem super cool/intriguing
And yet... if you're not careful... it will
EAT. YOU. ALIVE.

See?
If I hadn't gotten super sick tonight and written about it, I wouldn't have this new description for an illness I have very little control over.

Singing

Well..
I am not able to do much, but every now and then, every few months, I get an opportunity to sing.
As long as I have people available to help me with the equipment, I can manage to sit and do some singing.
Of course, it always wrecks me for days-weeks afterwards, but it is what I love to do so it's worth it to me.

Tomorrow I get to sing outdoors on a stage at EV Junction.
I have my parents and Rj coming with me to carry my equipment and set it up on the stage before I work with the cords and sound system.
I am debating whether or not to bring my fully-backed computer chair, to help reduce the pain in my shoulder blades, but I have yet to use it for singing. I am not sure that this particular opportunity is the time to try it out - I have really been looking forward to this and I want to avoid being extra nervous.
Plus, we haven't taken the wheels off of the chair and I'd hate to be rolling around while I am trying to play...
I am nervous enough as it is.

Yesterday my throat was really sore... and not necessarily 'cold' type of sore/scratchy, but more like an injury; like I have been jabbed in the neck. It feels bruised. Then after practicing a bit today, which means that my throat might be sore tomorrow, which may make it more difficult to sing.
I always bring my xylitol melts to help with dry mouth, then chloraseptic lozenges if my throat hurts too much... plus a medication if my throat starts to tighten from the excitement.
Gotta be prepared. ;)

No matter what - no matter the risk and the pain and the nervousness - I cannot WAIT to sing tomorrow.... and because of the type of atmosphere and who has asked me to sing, I get to play some of FAVOURITE tunes and ones I don't play often. Love it.

I can't do much.
I may have difficulty walking.
I may sometimes even have difficulty breathing.
But singing -- singing will always be mine, it will always be worth it, it will always uplift me in every way possible. And that is more important to my psychological and mental health and well-being than anything else.

Love and music.
That's what it's all about.

Consequences

I tend to get self-conscious a lot about my illness. Sometimes I feel like I am being too cautious, other times I feel like I push myself too hard. Sometimes people believe me, and sometimes they don't (although I am getting a bit better at ignoring this, because if it is just a random individual who doesn't believe me, then it doesn't matter).

Yesterday I had the opportunity to do something very cool. It is an opportunity that I get maybe once a year, and nearly always results in me pushing myself too hard. This time was similar, but even more so.
Often, when I get this feeling like maybe I am being too cautious, I will go out on a limb and try to accomplish something that I am pretty positive I might be able to do if I just push a little more. That maybe pushing myself once in a while like this is a positive thing and won't be as bad afterwards as I think it will.
Well - I guess being cautious is a good thing.
It's like when I haven't done a couple flights of stairs in a long time, so then I start to think that maybe I'm fine and I won't have any trouble. And then I try and I can barely keep my composure halfway through them.

Yesterday I pushed myself. Hard. I pushed myself farther than I have in years. You know what I learned? I cannot push myself that hard anymore. I mean, I had a lot of fun, but there were several opportunities to make it easier on myself. I did not have to try certain things, I could have taken more breaks, I did not have to expend SO much energy -> I still would have been present, had a blast, and would have accomplished what I was there to accomplish.
But noooooo, I had to try and show off. I had to try and prove that there are still things I can do that I used to do in my early 20s.
Nope.
I tried. I tried and I kind of failed, miserably. Don't get me wrong here, I still would have gone, I just would have taken it a lot easier. Turns out that my boundaries are slowly, but surely, closing in on me.

Yesterday early morning I took my preventative HAE injection to try and handle the day.
After vomiting last night and suffering horribly today, I spoke to my nurse, and I am to give myself another injection today. If that does not stave off this severe intestinal swelling episode, I will be off to emergency later tonight, as instructed.

Was it worth it?
Yes, in a way.
I still would have gone and had fun, but I should have taken more breaks and been easier on myself. I still would have pushed my boundaries, but I blew right past them. I pretended to have no boundaries. I pretended to have no limitations. And that was my ultimate mistake. Now I get to suffer the consequences.