Me and My Unstable Adrenal Insufficiency - Part 2 My Life With AI

 In a previous post, I gave a general overview of adrenal insufficiency, and in this post I'm going to write about my personal experiences with AI.  

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My AI Origin Story and History

Through as series of medical mistakes, misdiagnoses, and misadventures, I was put on high doses of steroids that I didn't actually need for several months in 2017.  This confused my already fragile hypothalamic-pituitatry-adrenal axis into slacking way the hell off.  I had a minor surgery for a congenital defect in my neck and had the joy of experiencing my first adrenal crisis the next day when my cortisol suppressed body couldn't cope.

My body's reaction to stress

  Adrenal crises are always scary, but this one was especially so because I didn't know what was going on.  It felt like I was going crazy and had the worst flu ever and a was in a horrible dysautonomia episode.  Luckily I had enough brain cells still firing to pitifully whisper, "adrenal issues" over and over again in the ER and my mom took over explaining my complex medical history from there.  I got the meds I needed, a 5 day hospitalization, and a shiny new diagnosis of secondary adrenal insufficiency (SAI).  I went home very weak, but stable.  Ish.      

I thought I'd be good to go after that and would wean back off steroids like I have dozens of times in the past.  No such luck.  It took me months to recover from that first adrenal crisis.  Months of weakness, gaining tons of weight, and feeling generally sick.  I found I was unable to stop the steroids, but at least through trial and error found a (relatively) stable daily basal dose of steroids.   

Some people are able to take a few pills of steroids a day and go on with life as usual.  I am not one of those people.  I've never found managing my AI easy.  I am more often too high or too low than just right in the steroids department.  

Things did get better when I learned about some unconventional treatment options from an AI support group.  In 2019 I switched from oral steroids to injectable steroids.  It helped a ton, but there was an even better option.  After exhaustive research, advocacy, and appeal letters to insurance I got an insulin pump that I could use to deliver the steroids.  I could program the pump to deliver cortisol 24/7 and at the rates of a normal cortisol day curve.  I could updose in minute increments in response to symptoms or activity.  Big meal?  Have a little updose.  Get the flu?  Easily increase my daily dose by 30%-50%.  Stressful day full of doctor appointments?  Here's a little bit of a bigger updose.  It became much easier to mimic a functional adrenal gland.  

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I got so flipping healthy that I started volunteering in vocational rehabilitation with the intention of going back to work.  Though I still found AI difficult to manage perfectly, I was doing pretty ok for a while. 

In September 2021 my (relative) stability slowly degraded for as yet undetermined reasons.  I went from going over a year without an adrenal crisis to having one every other month...then every month, then I was giving myself emergency injections a couple times a week and was in and out of the hospital.  My yo-yoing between high and low cortisol symptoms became pretty extreme.  My doctors think the AI instability is due to an infection somewhere in my body since I do get stable anytime I'm on strong antibiotics.  (I also have wonky blood tests that suggest a chronic infection.)  It could just as easily be any of my other health issues causing too much stress on my body though.  Yay medical mysteries! 

Not as fun as Dr. House made it out to be. 
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So that's where I am now.  Prophylactic antibiotics have helped cut down on the instability, but not completely.  I was just in the hospital with a crisis a month ago and am currently battling one at home.  I'm seeing lots of specialists trying to figure out various other health issues with the hope that addressing some of them will help the AI become more stable.  

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Symptoms

Most people who know me know that my "adrenal issues" cause frequent hospitalizations and make me feel sick, but I usually don't talk a lot about what the actual symptoms are.  Because I'm unstable, I have both high cortisol and low cortisol symptoms depending on the situation.

The high cortisol symptoms are slower to develop and usually less intense than low cortisol symptoms.  They are more likely to happen as I come out of an adrenal crisis and am slowly weaning back down to my usual daily dose.   

It's not fun to be on high doses of steroids.  They impact your mood, skin, weight, hunger, energy, pain, etc.  The general picture of high steroid me is a grumpy, tired, very weak, hungry person who feels like they have the flu.  

And that super sexy steroid moon face.
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It's also really hard to maintain adequate potassium levels, so I get a lot of nerve pain and other weird neuro symptoms like wonky vision and ringing in my ears.  Usually I become so weak that I literally feel and move like I'm walking through jello instead of regular old earth air.  The weakness has been a truly humbling experience.  It's wild to have to rest between each step of getting dressed, for example.  I need a lot of help to do pretty much everything when I'm that weak. 

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Occasionally, I'll have the pleasure of going a bit crazy from the steroids.  Once when I was accidentally given 6x the dose every 3 hours for a few days due to a dosing equivalent miscalculation I even needed IV calm down meds.  While terrible at the time, I now find my reenactment of The Exorcist  hilarious.   When I sheepishly told my doctor about it, he said I was lucky.  He has had patients strip down naked and run/crawl through the halls of the hospital from steroids.  I'm glad that all I get is fidgety and irritable.  In my last round of the high steroid crazies I became very offended if anyone dared to speak to me.  I'm sure I was a delight to be around.      

Now I'm thinking that like 80% of horror movies are just people overmedicated with steroids.
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 The hunger is something else too.  It's not like a fun kind of, "I'm craving cake," hunger or even the, "Hey it's about dinner time, let's eat," hunger, but this exhausting, never ending need to get out of a hypoglycemia episode where my hands are shaking, my vision is blurry, and my heart rate increases 15-20 beats per minute.  And it's not even fun to eat because steroids give me a terrible bitter taste in my mouth.  Where is the justice in that, I ask!  

In addition to these fun things, high doses of steroids are going to cause a lot of problems in my future.  I'll be much more likely to experience osteoporosis, type II diabetes, hypertension, obesity, cardiovascular disease, and frequent infections.  Lifestyle choices can help mitigate this, but even with pretty healthy habits, I'm already slipping down this road in my mid 30's.  I'm choosing to cope with this picture of my future by a combination of denial, living in the moment, and banking on medical advancements to fix me up in the future.  So just like everyone else, basically.         

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Even with a lot of high cortisol symptoms being pretty uncomfortable and unhealthy, I much prefer it to low cortisol symptoms.  These ones can come on quickly, are more extreme, and more imminently dangerous.  

The worst part is that I go a bit bonkers.  High cortisol me is grumpy and sleepy, but low cortisol me is a brain bathed in panic juice.  I can't settle down at all and nurture that Impending Sense of Doom doctors tend to actually take pretty seriously as it often precedes things like heart attacks.  Pain is bad.  Nausea sucks.  But hours or days of panic...that's the worst.  I thank my lucky stars that it's temporary and once treated, I get to go back to my regularly scheduled balanced brain chemicals.  

Actual footage of my brain chemistry during a crisis.
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As my body starts to freak out and shut down, I'll have unstable blood pressure, a very high heart rate, GI cramping, nausea, electrical heart issues, lots of trouble breathing, rapid loss of blood volume, and moderate to severe pain.  I also get...kind of dumb?  It call it adrenal dementia and basically I just sort of forget how to do things.  I'll be just as likely to lay down on the ground and stare off into space as get myself emergency meds.  Or if I do get myself emergency meds, it will take me forever to sequence the steps and I am likely to make bad dosing decisions.  The result of this is a loss of independence.  I generally need people nearby to get me help if I start drifting off into la-la land.   

I'm getting much better at catching low symptoms early and when they are more mild, which is great.  I recently got a fitness tracker which has been providing some good data and advanced warning when things start slipping.  I also have a very wonderful and attentive alert dog who will headbutt me or start aggressively licking my legs if I'm heading low.  And my parents are almost as good at my dog at recognizing low cortisol symptoms.  I have a good team supporting me.

My little hero

The sooner I can updose and get stable, the better because things usually escalate on an exponential scale once they get going.  

To give you a picture of how bad I look by the time I get to the hospital, I usually am rushed through triage and into a room no matter how crowded the ER is.  Once I even got to "cut" in line ahead of the guy who just came in on an ambulance with a heart attack.  Yikes.

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Impact

   As you can imagine, this has had a major impact on my life.  It's really pushed me into an existence of minute by minute living.  I have to be on constant alert for any change in symptoms.  Since it's rare to have AI this out of control, I don't have as much help from doctors as I'd like.  I have to make high stakes decisions without sufficient data, education, or experience.  Which I find very stressful.  And tiring.  I swear, like 8 times a day I just sigh and say, "I'm just so tired."       

  I'm limited in what I can do because I can't really be alone and I have to stay close to local healthcare resources who are familiar with my treatment protocols.  Even more than usual, I have to cancel plans last minute or not make them in the first place. I have great days and sometimes even a good week or two here and there, but most of the time I'm either recovering from or heading into an adrenal episode or some other health issue.  

I've adapted by pursing more hobbies that I can pick up and put down easily.  I'm taking online classes and continue to work a bit from home.  I miss the in-person socializing, and just doing stuff in general, but whatever I can do from bed is a better fit for me right now. 

The good news is that I don't expect things to be this difficult forever.  I continue to follow leads and chip away at my health issues.  Each thing that I can address gives me that much more stability.  I am so far from exhausting all my resources.  When I am overwhelmed wondering what the next steps should be, I remind myself that's a good thing because I still have options.  

As a charmingly forgetful little blue fish once said...

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Me and My Unstable Adrenal Insufficiency - Part 1 (The Basics)

 One of the most difficult health issues I've struggled with in the past few years is one most people don't know that much about*.  I thought it would be a good idea to write a post about my experience with unstable adrenal insufficiency (AI) so my friends/family can have a better idea of what I have going on, and for anyone who may find this helpful if they are in a similar situation.  

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This fist post is a general overview about adrenal insufficiency.  Stay tuned for part 2 where I'll talk about my personal experience with AI (which, spoiler alert, is super chaotic and abnormal.  Yay.) 

What is it? - The physiology

First, forgive me, but I need to go over some boring hormone stuff.

Hormones are chemical messengers that help the body regulate and function.  There are a bunch of different kinds and many of them work together in groups called axes.  If something goes wacky in how your body creates or responds to hormones, you're gunna have a bad time.  

In adrenal insufficiency, something in the hypothalamic-pituitatry-adrenal axis get's messed up and the body stops producing enough cortisol.   Which is a bummer.  Because you absolutely need cortisol to stay alive.  

Something can go wrong at any of these levels.   So much opportunity for hormonal mischief!  

  There are different types of adrenal insufficiency which all have similar-ish end results.  There is primary adrenal insufficiency, also known as Addison's Disease*.  This is where the adrenal glands - the cute little pyramids above your kidneys - stop producing cortisol.  The lazy bums.  Then there's secondary adrenal insufficiency where the pituitary stops sending messages to the adrenal glands to produce the cortisol.  And there's tertiary where the hypothalamus slacks off on it's job communicating to the pituitary which impacts the adrenal glands. 

   

What are the symptoms?

So you are unlucky enough to have stopped producing enough cortisol - what will that look like?

Symptoms can start suddenly, usually if there is a major trigger like surgery, or it can come on slowly over time.  

Without enough cortisol, you can enjoy entries from the delightful menu of fatigue, GI upset, POTS,  weakness, and mental health disturbances, among many other things.

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That's just the kid stuff though.  That's just the baseline of not feeling very well.  Some people go years without being properly diagnosed in this state if they are still producing some cortisol.  

The big scary stuff happens when you don't have enough cortisol to meet the demands of your body and it sets off a cascade of horrible known as an adrenal crisis.  

This is very hard on the body and a medical emergency.  If you don't get urgent treatment, you will (most likely) die.   

The symptoms of an adrenal crisis are more extreme versions of the above menu, with some extra fun thrown in.  

Symptoms might include vomiting, fever, hypoglycemia, severe pain, cognitive decline, lethargy, heart rhythm issues, trouble breathing, and, my personal favorite, the overwhelming sense of Impending Doom.   

Basically your body is going into shock and shutting down.   It's not a fun process.   In fact, I'd go as far to say it's the worst thing I've ever experienced.  And that's saying something as someone who woke up from neurosurgery without any pain meds due to an IV malfunction.  Twice.  

Adrenal crises can look like a mental health crisis, a stroke, appendicitis, hypoglycemia, drunkenness, and a lot of other things.  This confusion can lead to adrenal crises not being caught and treated in time.  In fact, if you survive your first adrenal crisis, congratulations, your life expectancy just skyrocketed!  

What is the treatment?

Anyone with adrenal insufficiency needs to replace the cortisol that their body would normally produce.  That is their base biological dose.  They need to replace this dose in a way that mimics what the body would do if it was working correctly.  In functioning bodies, cortisol is released at different rates at different times of the day known as the cortisol day curve.  So both the amount and timing of daily steroid replacement is important.   

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In addition to this daily base dose, people with AI may need to increase their dose (called updosing or sick dosing) if their body is stressed by illness, injury, or abnormally high activity.  If there is a severe emergency or adrenal crisis, they will need to get a large injection or IV dose of steroids.

So people with AI:

1. Have a basal dose they take each day.

2. May need to updose in response to illness, injury, or stress.

3. Need to carry an emergency kit with injectable steroids in case of severe illness, injury, or adrenal crisis.

Most people get their steroids through taking 1-3 pills each day.  Some people do great with this, have a fairly normal life expectancy, and are basically asymptomatic.  Them lucky ducks!

Like this guy.  A very lucky duck in the AI area.  Not so much in other areas. 
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  For everyone else with AI, figuring out the exact dose and timing of meds can be difficult.  People digest, absorb, and utilize steroid meds at different rates.  We can't test our cortisol levels at home, so we have to do this based on symptoms, past experience, and...I dunno, sometimes it feels like I'm just making it up as I go along.  Even if we could test cortisol levels at home like people can test blood sugar, that wouldn't be very helpful because the amount of cortisol your body needs at any given time varies.  Because of this, we are only ever cosplaying as an adrenal gland and can never get things quite right.  It's probably more common to be a little high or a little low in cortisol replacement than in the just right range.  

Speaking of cosplay...potential Halloween costume this year??? I'd just need a little triangle hat.
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Some people don't tolerate the pills or they need more control of their steroid dosing.  If these people happen to be privileged with excellent healthcare and are strong self-advocates, they may be able to switch to sub-cutaneous injections of steroids.  

Look at those beautiful life giving little soldiers. 
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  Some very lucky people who respond well to injections may be able to sweet-talk their doctor into prescribing them an insulin pump that they can use to get a continuous dose of steroids.  This pump is programable to more closely follow the cortisol day curve, can handle minute changes in dosing, and is as close as anyone is going to get to a bionic adrenal gland.  In my opinion it's the best option by far and I hope someday it becomes standard of care.  

My beloved pump.  Built like an old school Nokia and deliverer of the elixir of life. 

   Depending on the type of AI and various comorbid conditions, some people may need to take additional meds too.  But these are usually much easier to manage and I'm not going to go over them in this post.

In the case of an adrenal crisis, treatment becomes more complex and involved stabilizing blood volume, electrolytes, blood sugar, controlling pain, etc. in addition to providing large amounts of IV steroids.  If caught early, some people can be stabilized in the ER and then go home, but often an adrenal crisis results in a hospitalization and a long recovery.    

What is the prognosis?

   The most dangerous time to have AI is before you are diagnosed.  If you survive your first adrenal crisis, things look much better for you.  After that, it depends on how easy your case of AI is to manage, how good you are at taking care of yourself, and the quality of healthcare you have access to.  It's pooooooossible to recover from secondary (and maybe tertiary?) adrenal insufficiency, but in general, it's not something that can be cured or reversed.  You're stuck with it.  

  Some people have pretty normal lives once they get on a good treatment regimen.   Unfortunately, many (citation needed) people seem to have at least some symptoms and negative side effects from the steroids.  Things like fatigue, obesity, and type II diabetes are common.  If you go by population statistics, life expectancy is less if you have AI, but it really depends on the person.  

It's a pretty bummer disease, and it can be dangerous, but it's not a terminal diagnosis.  

*Other random notes:

  Adrenal insufficiency shouldn't be confused with "adrenal fatigue."  Adrenal fatigue is a made up thing that is not supported by current science.  It's a concept perpetuated by snake oil salesman and dodgy figures spouting dangerous health advice.   If you have AI and anyone tries to tell you to stop taking steroids and switch to supplements or magic crystals or whatever, run away as fast as you can.  

  I've over-simplified some things in this post for the sake of accessibility.  If you want to get a full picture of adrenal insufficiency, I highly recommend Congenital Adrenal Hyperplasia: A Comprehensive Guide by Peter C. Hindmarsh (Author), Kathy Geertsma (Author).   I've read it cover to cover and it's a great resource.  

  When I introduced this post, I mentioned that it's not a very well understood condition.  By that I mean that even doctors, even endocrinologists, even the best endocrinologists at major research hospitals don't really know that much about it.  Though the basics are something you can learn in intro college biology, management of difficult cases can get very complex.  If you don't respond well to taking a few pills each day, no one really knows what to do with you.  There's a lot of controversy surrounding use of injections and pumps, and a lot of dangerous advice against updosing.   

Nothing in this post is meant to be medical advice.  Please consult your doctors for that.

Thanks for reading!