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!    

  

  

Fifteen Years

I posted this on facebook on the actual day and wanted to post here too.  Fifteen years...quite a landmark!  

15 years ago today I got a chiropractic adjustment on my neck that went very wrong and I see it as the turning point of when I officially became sick. Though I was born with Ehlers Danlos Syndrome, I didn't know it at the time, and still managed a pretty normal life going to school, playing soccer, traveling, and dreaming without many of the limitations I have today. I had a very different life envisioned for myself.

Because of that neck adjustment, I had a stroke and tore some ligaments in my neck. I was lucky that I had a good neuro recovery from the stroke. I learned to walk again pretty quickly and got used to reading at a slower pace. But since that day, I've never been healthy.

I developed POTS and learned to live with a resting heart rate in the 90s-110s. It's exhausting. The chronic pain I'd had since I was 9 years old became a much bigger issue. I've had spinal cord injuries because of the instability in my neck, developed some seriously strange allergies, stopped absorbing many nutrients, had SO many huge surgeries... the list goes on. Literally for pages! More recently I've been struggling with unstable adrenal insufficiency and CSF leaks. It's been extra rough since 2016. Each piece of the puzzle that's figured out takes months or years of doctors appointments, tests, and lots of "are you sure it's not just that you're a weak woman?"

One thing I can say for myself is I never stopped living. Yes, I need to rest a lot, and I have to do things differently, and I'm pretty uncomfortable most of the time. But with lots of hard work, support, and an increasingly amazing healthcare team, I do have a life. While the last 15 years hasn't been what I'd expected, I've done some amazing things, met so many awesome people, and most of the time I'm pretty happy.

I made it through college on the slow path, even studying abroad (after two previous failed attempts). I went to grad school on the other side of the country and became an OT. I lived in NYC, and even though I had to move back home, it was everything I thought it would be while I was there. I got over a needle phobia and faced my fear of public speaking to raise funds and awareness for causes important to me. I've lobbied congress for better healthcare and helped create local support networks for people with chronic illness. When I couldn't find accommodating work, I started my own OT practice so I could continue in the career I loved. I found so many tiny ways to celebrate life and go on little adventures when I can't manage the big ones. I have so much time to devote to hobbies and lifelong learning. I've found over and over again resilience as challenges piled up. I have a closeness with my family and friends that grew from my extraordinary circumstances and my dependency on others. I have a lot of love in my life and truly know how lucky that makes me.

It's not the life I expected or wanted, but I think little me would be proud of what I've done in the past 15 years. I think I've done pretty well with the cards I've been dealt. So many opportunities have come out of adversity and I am very much grateful for that. I wake up each morning happy to be alive and excited about the day. I know that alone makes me one lucky duck.

To everyone who had been here with me, I truly can't thank you enough. It takes a village and I have an exemplary one.

Just like everyone else, I'm not sure what the future holds, but I'm always hopeful and always looking forward to it.

So happy "sickiversary" to me! It's been a crazy 15 years full of the best and worst times of my life, adventure, adversity, joy, and perseverance. Thanks for sharing the journey with me.

I'll be adding some photos with captions in the next couple of days. More good times then bad!