After ten years of seeking answers for fluctuating energy levels, my twin diagnoses of Hashimoto’s and Addison’s Diseases arrived a couple of weeks apart. Addison’s was delivered in the emergency department at around midnight on 14th June 2014 by the simple act of reading 2 numbers.
For several hours, I’d been harassing any nurse who came within earshot to obtain a copy of my Addison’s test results. They all ignored my request.
Except for the one who tried to reassure me that they would simply re-run any required tests. My words, “but it took me over an hour to locate a pathology lab in Melbourne which had the requisite hormone in stock to run this test,” were sucked out of my cubicle as she whooshed out.
A cryptic call
My urgency stemmed from a phone call received that afternoon from the pathology lab doctor who had reviewed the neglected test results.
With barely contained desperation in her voice, the lab doctor explained “I’ve been trying all afternoon to get onto your GP. I really need to speak to them about your results”. Bemused, I explained that GPs don’t often work on Saturday afternoons. “What do my test results say? Do I have Addison’s Disease?”
The lab doctor responded that she was unable to give me the test results and urgently needed to speak with my GP. Wow, that was a subtle “yes”, Addison’s is on her way.
“Are you already taking steroids?” she asked with a spark of hope in her voice. “No, should I be? Do I have Addison’s?”, I read between the lines. Given steroids are the treatment for Addison’s Disease, “yes” was firming.
Still unwilling to tell me my test results, she instructed “if you start vomiting, feel weak, or experience pain in your back or legs, you need to go straight to the emergency department. Ask them to contact us to obtain your test results.” The “yes” had become rock-hard.
Jack Nicholson in the film a Few Good Men, meme-ofied by myself.
Laughter came first, manic laughter. That was one of the top 5 stupidest conversations of my life. I had just listened to the lab doctor’s internal battle between upholding the Medical Model (under which patients are considered unqualified to be given their own test results) and Duty of Care (to protect my safety and wellbeing). Ridiculously, the Medical Model had won out – I was left feeling unprotected and unsafe because she’d kept me in the dark, assuming that I couldn’t handle the truth.
The solid truth is far easier for the mind to handle than the countless boogie-men that lurk in the darkness of unknowning – me
Dr Google to the rescue
Without access to a GP for the rest of the weekend and knowing that an ill-defined impending medical catastrophe could roll in at any moment, I did the only logical thing – I turned to Google for answers. This was the second time that I had Googled “Addison’s Disease” in my life.
The first time had been a week earlier. I had been newly diagnosed with Hashimoto’s Disease (an under-active thyroid) and started on replacement thyroid hormone – thyroxine.
My research of Hashimoto’s Disease found that it sometimes co-occurs with a disease called Addison’s. What’s that?
As I scanned the symptoms of Addison’s Disease, all of the strange changes in my body over the past 18 months were neatly articulated in a single checklist.
- Headaches: Mine had lasted for up to 5 weeks at a time and weren’t relieved with pain killers.
- Dizziness: I experienced this particularly on standing up and in hot environments.
- Fatigue: I caught colds and viruses easily, and would be couch-bound for weeks afterwards unable to move from exhaustion.
- Nausea, vomiting, diarrhoea: I had episodes of nausea and vomiting for no apparent reason. Once, I vomitted immediately after eating pancakes my partner had cooked for me for brunch. They had been delicious, really.
- Oversleeping: I slept for 14 hours per night.
- Hyperpigmentation (see exhibit A above): For 18 months, I had what looked like an all over tan plus “bruises” that didn’t go away on my elbows and knees.
- Difficulties standing up: This got so bad that I experienced chest pain on trying to stand up
- Salt cravings: I was gradually consuming more electrolyte tablets. I also craved and ate copious amounts of sugar in my body’s attempt to produce more energy.
- Appetite loss leading to weight loss (see exhibit B above): I would feel full after eating a tiny portion of protein-rich food.
It was when I almost crawled into my GP’s office from weakness and presented her with this checklist, that she had ordered the test for Addison’s Disease. She commented “you don’t look right” (I’d been telling her I didn’t feel right for 18 months, it’s interesting that it took for me to not look right before she became concerned).
During the second Googling of Addison’s Disease, I discovered that Addisonians are at risk of a potentially life-threatening state called an Adrenal Crisis. This was clearly what the lab doctor had been tip-toeing around.
With growing concern, I read that Addisonians were at the greatest risk of an adrenal crisis when untreated for an extended period of time (panic), when stressed (double panic), and especially if started on thyroxine (full scale panic). The best practice clinical guidelines for someone presenting in my condition is to skip all diagnostic testing and go straight to treatment with steroids – do not pass GO, do not collect $200 – it’s too risky to wait.
Things take a turn for the crappier
Queue sudden (but in hindsight not unsurprising) rapid deterioration in my health. Within 2 hours of receiving the phone call from the lab doctor, my body weakened. It was so weak that after standing up, my husband said that I looked like I had just finished running a marathon.
“I think I need to go to hospital”, I suggested. My husband responded with zero hesitation – I must have looked really crappy.
By the time I arrived in the emergency waiting-room (surely that’s an oxymoron), I was shaking uncontrollably from head to toe. I had to grip onto the counter to hold myself upright. When asked for my address, my brain was functioning sufficiently well to think to produce my drivers’ license (I was pretty smug about this) but only in lieu of being able to recall where I lived.
“Adrenal crisis” was all I could stammer at the check-in staff. I guess that I expected the staff to spring into immediate action on hearing those magical words, calling for a trolley-bed and an IV, STAT! I’d never before been in an emergency situation. I’d only seen it on TV.
Instead with bored non-concern, they reached over the counter and took my blood pressure. I had to concentrate hard so as not to fall over from releasing one hand from the counter.
My blood pressure was high (apparently it’s common for it to spike then tank during an adrenal crisis – caused by an initial, short-term adrenaline burst as the body tries desperately to maintain blood pressure). Unfortunately medical textbooks inaccurately state that blood pressure is only low during an adrenal crisis.
Bernard from Black Books excited to be working at Mambo Burgers after being locked out and stuck on the street over night
So with eye-roll-voice reminiscent of Bernard Black, the hospital check-in staff told me to breathe and take a seat. They seemed oblivious to how condescending it was to tell a grown woman to breathe. I suspect they thought I was having a panic-attack. They could have at least offered me a side of fries – the salt would have actually been helpful.
When my name was called and I stood up, I shook so violently all over that it took one person under each arm to help me walk into the emergency ward. Yes, walk because an angry demanding woman having a panic-attack doesn’t need a wheelchair. Yes, shook violently because no amount of fancy yogic breathing learnt over a decade of practice treats a sudden drop in blood pressure and weakness on standing up that are hallmarks of an adrenal crisis.
After what felt like a couple of hours, I finally saw a doctor. Thankfully when I barked at her, there was a look of recognition and respect in her eyes. Within 10 minutes, the emergency doctor excitedly showed me a copy of my test results (hallelujah!).
They couldn’t have been any clearer (or worse depending on your perspective). My blood cortisol level at 8am was 26 nmol/L (note that the Australian units of measurement are different to those used in the US). It should have been at least 10 times higher with the normal range being 240-620 nmol/L.
During the test I was injected with the adrenal stimulating hormone and my adrenals should have responded by pumping out a minimum of 200 extra units of cortisol. My adrenal glands managed to squeeze out a piddly extra 2 units of cortisol.
In short, my adrenal glands were totally stuffed. I marvelled at how I’d lived through a Disco Yoga class in a heated room the previous weekend.
Your order has been delayed
The emergency room doctor contacted a senior endocrinologist (hormone specialist) who ordered the standard treatment for an adrenal crisis. “Finally, some actual emergency action”, or so I thought.
Whilst they inserted a canula (a big needle that stays in your arm for hours or days) and ran saline (salty water) into my bloodstream, for some reason the emergency staff disregarded the second part of the instruction about the need for life-saving steroids.
I suspect that they didn’t believe that I was dying fast enough. The emergency doctor had commented multiple times how well I looked and talked given the circumstances. “You know that my body shakes violently every time that I stand up and that I couldn’t recall my own address?” I thought bemused.
They moved me into the short-stay ward, dismissed my husband as if he were a fly and left me alone for several hours to “sleep”. I was confused about their instruction. I called the nurse back in. “So aren’t you going to remove this thing?” I gestured to the huge uncomfortable needle sticking out of my arm, the long tubing that had already wrapped around me like a snake and the bedside machine I was connected to which emitted constant churning sounds and an intermittent beeping at precisely irritating level.
This was my first time being placed onto a drip. They had inserted the cannula into my arm without a single word of explanation. Not a whiff of informed consent to be smelt anywhere.
My question triggered in the nurse an equal mix of annoyance and surprise which came out sounding like disdain. “You’re not even half way through the first bag of saline. Why on earth would I remove the drip?” Dramatic stage-exit.
At 3am, I discovered that my status had been upgraded from “angry demanding woman having a panic-attack” to “angry demanding woman having a panic-attack and dying a bit” because I’d earned a wheelchair transfer.
Your order has been delivered
Now transferred out of the emergency department and onto a private ward next door, I was treated quite differently. The first thing they did was to follow the endocrinologist’s directions to add steroids into my drip.
During the 6 hours in the emergency department and despite telling everyone exactly what I required to stay alive, I had not received life-saving steroids. As a result, whilst I was “sleeping” alone, my blood pressure had steadily dropped to an extremely low level.
I was visited by that exact endocrinologist in the morning and boy, was he mad. Mad enough to say that he’d lodge a complaint that the emergency department hadn’t administered steroids and apparently they’d also messed up a crucial blood test that couldn’t be rerun now that treatment had commenced.
It took 4 days of lying flat (apparently being upright was unsafe), frequent monitoring (blood pressure and blood glucose tests every 4 hours) and treatment on an IV drip including steroids to raise my blood pressure to a safe enough level to be transferred onto steroid tablets.
I wonder how much shorter my stay would have been had the emergency department performed the simple, cheap task of injecting me with steroids on arrival. I also sometimes wonder how things would have turned out for my long-term health had my GP started me on steroids or consulted with a specialist months sooner. But hey, I survived. Go body.
I now take steroids daily for life in order to remain alive and I carry my own steroid injection kit for emergencies. I’ve been upgraded from a patient without the qualifications to be given my own test results to an expert in the self-management of a complex rare disease.
If you haven’t already, check out my post on What is Addison’s Disease: Explanations to Entertain, Educate and Impress a Crowd.
Look out for the upcoming Part 2 in the Addison’s Story: A Rewriting of the Fairytale Ending, and posts exploring the Treatment for Diagnosis Delay and Why Emergency Departments Don’t Treat my Emergency.
Here is a brilliant post on Living with an Addisonian that discusses the problems with current definitions of an adrenal crisis (hint: they are vague and potentially dangerous), the difference between pre-crisis and full blown crisis, and an improved definition of adrenal crisis proposed by Professor Bruno Allilio.
You may wish to read more on the Short Synacthen Test for adrenal insufficiency, guidelines for the management of adrenal crisis and a literature review of adrenal crisis. This handout on the treatment of adrenal crisis is designed for patients to give to emergency staff in the hope of providing legitimacy for their request for treatment.