Synacthen Test Results

A couple of weeks ago, I had to repeat my Synacthen test. You can read about they whys and wherefores in my previous post about it, here. This is the third time I’ve done this test, and, this time, I was hoping for some definitive answers about whether or not I’ve got Primary or Secondary Adrenal Insufficiency, or both. The other times I took the test, they didn’t collect an ACTH sample, which is one of the ways you can rule in Secondary Adrenal Insufficiency, despite me asking them to last time. This time my endo and I both agreed that it was important to get this result.

However, I’m a tad grumpy because my bloods weren’t processed within the 30 minute window for ACTH testing, meaning they couldn’t get a result. So we still don’t know! And I might have to potentially take part of the test again, which I’m not happy about, because you have to stop steroid medications, which is horrible.

Not all of the test was pointless though. We did get my cortisol results back. My baseline result was ‘less than 10 nmol/l’ and my 30 minute response after ACTH injection was 63 nmol/l. For reference, a ‘normal’ person’s levels should have been 500 ish at baseline and increased further by 30 minutes. I’ve told many doctors many times that I might ‘look ok and sound ok, I’m just good at compensating’ and this test kind of proves it. On the day of the test, I was able to walk the short distance into the hospital and sound coherent in conversations, but with a level that low, both before and after the test, I wasn’t far off being critically ill or dying from very little shock or over exertion. Maybe I should give myself more credit that I am still able to even vaguely function, be nice to people and not be a hormonal wreck all the time even though my bloods came back like that!

The results were sent in the post to me and my GP within 48 hours of the test along with very specific instructions, which kind of also highlights the seriousness of having low cortisol. I already knew the instructions anyway, and nothing changes medication wise for me because I’m already on replacements, but it did come with a very helpful, idiot-proof letter from the professor in endocrinology managing my care. It clearly says that I have Adrenal Insufficiency and not Adrenal Suppression (where the adrenal gland might be suppressed and stand a chance at recovery) and that no one is allowed to decrease my steroid dose. Hooray! I’ve been having arguments with various people about these two points for about a year, so it felt good to have it in writing.

Even better, it very clearly states that IM or IV therapy might be required ‘to prevent risk of adrenal crisis’. This might not seem like a big thing, but sometimes I end up in hospital arguing with medics who are refusing to give me my IV, that while I might not be in full blown adrenal crisis because I’m quite clearly talking and not unconscious in a coma, could we give me the IV while I have hypoadrenal symptoms so that I don’t have to get to coma stage before they give me it. This letter might help with that. And, the icing on the cake, it says ‘the lowest dose the patient feels well on is the safest dose’, in big letters and bold. Which means the this department seems to actually care about what its patients actually *feel* like and not just being on the standard 10/5/5 dose.

So my annoyance at the ACTH test being messed up kind of got neutralised a bit by the really helpful stuff that also came in the envelope. Fingers crossed I don’t have to do the test again though!

What I learnt from watching the sunrise

After being ill with Alzheimer's for a very long time, my Grandma died this week. So it wasn't unexpected and definitely kinder for her than living the way she was, but, you know, a family member died, so it's still sad.

I found myself awake for my usual 4am time (endocrine system usually wakes me up then). We were away on holiday in Devon with a beautiful view of the sea, so I decided to get up and watch the sunrise over the sea and Wales. Like lots of people do after someone dies, it got me thinking about the impact we leave on this planet once we die. I read somewhere once that people 'die' twice- firstly when they physically die, and again when the memory of that person dies as well e.g. the people who knew them either die too or 'forget'.

But what if you don't really know anyone? Or people don't talk about you to remember you? Does that mean the impact you had was nothing?

This is where the sunrise was quite timely. It was a beautiful sunrise as well, which helped! The most important thing which happens in our lives every day consistently is that the sun rises. Without it, plants wouldn't grow and we wouldn't have oxygen. Or we'd freeze to death. Or be depressed from not having any light. You get the idea. But hardly anyone notices it, except insomniacs and people awake at 4am like me. And even fewer acknowledge it or are grateful for it. But every day it pops up, does its thing and goes down in the evening.

I guess my point is that we might think we have very little impact on other people, but, actually, we still can without realising it. Seemingly small or routine things are actually the important things. Kind words or gestures which the giver might forget usually stick around the longest with the person receiving them. Random acts of kindness do make a difference.

So our impact might not be the same as JK Rowling losing billionaire status because she gave all of her money to charity kind of impact, but to someone somewhere, something very simple can have the same impact on someone as the sun rising has on the billions of people on this planet.

What I’ve learnt from clouds

I'm on holiday by the sea in Devon. In fact, I can see and hear the sea from every window in the house we're staying in. It's been lovely! The other evening, I had a bath in this amazing bath:

I listened to the sea and watched the clouds. It was sooooo relaxing. I'm going to apologise to my friend Thor, who studies clouds, because I'm pretty sure my analogy isn't remotely meteorologically correct (I'm not even sure if that's the right spelling!).

There were grey, rain clouds and fluffy, white clouds. The fluffy white ones were still and big and there consistently and the grey, rain ones were drifting speedily by in the strong wind. Every now and then the grey ones covered the white ones and you only got glimpses of the white ones. And then sometimes the grey ones were really thick and completely obscured the white ones. But the grey ones passed over and the white ones came back again, still big and white and with the sun reflecting on them in the same place.

On my Calm app, Tamara (the woman who narrates it -we're on a first name basis 😉) says every day 'emotions pass, this too shall pass'. And it's like the clouds. The bad, nasty things are just dark clouds temporarily covering up the white, fluffy, exciting things, or the good things in life. Good things are still there in the background even when the dark clouds pass over. You have to work harder to see them, but they're still there.

Not exactly a difficult conclusion to come to, but it made me feel good at the time I was watching clouds (which I love to do anyway!). Plus, for once, I get to brag about somewhere beautiful I've been 😉

Insulin Resistance & Reactive Hypoglycaemia

I saw a different endo last week, which was mostly preparation for the synacthen test I had this week. However, we did talk about the hypos (low blood sugar episodes) I've been having, and I got diagnosed with something else; reactive hypoglycaemia.

A hypo isn't pleasant. When I have them, I feel shaky, sweaty, confused, have poor concentration, dizzy, and have a headache. Too low a blood sugar is dangerous, so needs to be treated so you don't become acutely ill.

The consultant actually diagnosed me with an 'abnormal phase 1 lag release' (whatever that is!). But that's a bit of a mouthful! I already have insulin resistance, which means my body needs a bit of persuading to make insulin, and then when it does it floods my system with it. But having reactive hypoglycaemia means that my blood sugar level gets pushed down by the excess insulin and I have a hypo, or something that feels like a hypo because my body doesn't know if it's coming or going.

Still following? If so, well done! It was at this stage of the explanation that I really wished I'd paid more attention in biology at school. Reactive hypoglycaemia also means that when I eat, my blood sugar goes up a tiny bit and then down a lot, rather than up a lot (ish) and down a bit like most people's do. I've been telling doctors this for about 18 months and they've all been telling me 'that's not right' or 'you're reading it wrong' but the professor in endocrinology I saw last week didn't flinch when I said it and knew exactly what was going on, so it can't be that weird!

This actually explains my 'I need to eat' feeling I get and why I need to eat seemingly constantly, even overnight. I eat something, insulin isn't used the way it's meant to, my blood sugar goes down and I feel hypo-ish so I need to eat to get my blood sugar to go up a bit and the cycle starts again. So really, when I feel hypo, it's because of what I've eaten a few hours ago because my body has a lag or delay. Which is a tad hard to predict!

Just to complicate things further, hypos are also really bad for Adrenal insufficiency, and hypo is one of the onset of crisis symptoms. It's been pretty entertaining trying to guess whether I need sugar, carbs or more hydrocortisone during these episodes!

My consultant's exact words were 'think and eat like a sheep- graze little and often'. Which made me laugh- a consultant with a sense of humour! Rather than 3 meals a day, I should be eating more like 6 or 7 smaller meals, made up of low or complex carbs (low GI). I've essentially been eating the right foods but the dieticians and my other endo told me to aim for 3 meals a day. So I did and it was hard. I'm hoping this might work better and I won't feel so much like I'm on a rollercoaster blood sugar wise.

I never have high blood sugar, my over producing insulin (albeit with a lag) takes care of that. I need to do some more reading about it and, from what my consultant said, it's not easy to work out and will be a lot of trial and error. But it feels like some progress after I've been hitting brick walls for almost 3 years with this.

Synacthen Test Done :)

A few people have messaged me to wish me luck or ask how the test went, which is really kind, thank you 🙂 It's all done now, which I'm pretty relieved about I have to say!

After I wrote yesterday's post, I didn't really sleep much. Hilariously, one of the symptoms of low cortisol is fatigue and exhaustion but you can't necessarily sleep when you're low on cortisol. It's a bit like jet lag, you're tired but awake because your body clock is off. The other downside is if you're cortisol deficient, your body compensates with adrenaline surges instead, which make it doubly hard to sleep. Aside from that, I did end up with stomach ache and feeling really sick so spent a pretty rough night up and down with that. Usually I'd updose on Hydrocortisone, but that wasn't an option because of the test. So I ate a lot of ready salted crisps (AI patients crave salt when struggling) and drank a lot of Coke. Don't ask me why, but those things help if Hydrocortisone isn't an option. Not the healthiest of nights, but if I'd had sickness or a bad upset stomach then I'd have had to have abandoned the test, injected and gone to hospital anyway. So another compromise.

The doctor was really nice. She read out all the bloods they were doing as well as the synacthen, and then added on a couple I asked her to. There was one moment of panic where she said 'fasting panel' and I'd eaten breakfast, but it turned out the consultant had just got a bit tick happy with the boxes. Unlike last time, I took my morning HC dose immediately after the test- the endocrine nurse last time said I wasn't allowed to because it was past my scheduled time. This time I didn't bother asking and did it anyway. This helped a lot because it meant that I'd only really missed yesterday's evening dose, although my Total Daily Doses were all different.

Today I got home, got into bed and promptly crashed out. My husband woke me up to feed me some lunch and my lunchtime tablets and then I went back to bed again until about 4.30pm. I usually say 'can you check I'm still alive every now and then' on days like this. Now, I feel a bit like I do after I come out of hospital- sitting up is ok, Facebook stalking is ok, chatting is just about ok, doing anything else is a lot of effort so it's safer not to. I doubled my evening dose because I was feeling weak and sick and dizzy despite all my sleeping, and took a bit extra just now before bed so that I'll hopefully sleep properly. That way I'm hoping a slight increase now will avoid me having to stress dose for a week or so because my body didn't catch up and I pushed through it without extra hydrocortisone rather than letting it recover. Fingers crossed anyway!

Stopping Hydrocortisone for tests

I've got my repeat synacthen test in the morning, which meant taking a reduced dose of hydrocortisone for my morning and lunchtime ones and taking none for my evening one tonight and none tomorrow morning. It's because the test measures how much cortisol you make, and taking Hydrocortisone would give it a false reading. Realistically, having been on it is going to give it a slightly skewed reading anyway, so reducing and stopping it for 24 hours before is a compromise.

I also have to stop my steroid inhalers for my asthma, which I think most people can empathise with more because everyone knows that breathing is important and not being able to breathe is scary. Truth is, most asthmatics, including me even when I was at my absolute worst, will be able to withhold their inhaler for one dose for testing without too many issues. You can't leave the house and maybe couldn't do much but it would be ok. And you've always got your reliever to fall back on. With cortisol, even if I didn't get out of bed, I wouldn't survive much longer than 24 hours without it and have to be really careful doing even basic things so as not to set off a crisis. So I'm going to explain what it feels like to stop hydrocortisone, because a reduction in cortisol is something that most people won't experience ever!

The first thing I notice, which reminds me of pre diagnosis days, is I have this overwhelming feeling that I have to lie down and put my head down. It's fatigue based, but it's an instinct more than a feeling. I *have* to put my head down. It feels like being on a boat, kind of waves. In fact, it's like being underwater and hearing voices out of the water.

I feel unwell a lot of the time anyway, but this is a whole other level. It's mainly 'weird' more than anything. I've permanently got that feeling you have before you throw up, where you feel everything kind of drop away and you get clammy and hot and shaky.

My head feels really foggy. Like just having woken up and disorientated but all the time. I can't work out if I've imagined some things or if they're real because I recall everything with the same haziness you remember dreams.

I get these waves of nausea but not actually being sick. I had a cold last week which seems to have come back a bit, which sometimes happens after I stress dose for illness if I taper too quickly. So I've got a bit of a cough and sore throat, but that could also be because I've missed my inhalers and allergy spray. When I breathe in I get pins and needles in my hands and feet. All my muscles feel achey like I've got the flu and things keep cramping. So far I've only had a slight headache and no stomach ache *touch wood*- that head and stomach ache are by far the worst symptoms I have. My skin feels like it's on fire and I want to pull it off.

I'm not happy about the test because I feel grotty but I was super happy with my endo preparing me for the test on Friday. He asked me what dose I could manage on today and if I could withhold entirely tomorrow morning. In other countries, they swap you onto a different steroid, Dex, for the test because it doesn't affect cortisol readings but they don't do that here- usually you just get told to withhold meds and get on with it, so it was nice my endo took the time to ask me how I felt about it all! It'll all be over by this time tomorrow and my normal dosing schedule will be back in place.

Why I Started ‘Out With Animals’

It’s a very strange thing when you stop working because of ill-health. The only thing I can really compare it to in any way is it must be a bit like what retirement feels like. It’s the end of an era, you’re admitting that you’re not able to function like you used to, you’re taking some time for yourself and you’re putting your working life behind you. But retirement is expected, and it’s a celebration in some ways as well. When you stop working 40 years earlier than expected like I did, you don’t get people celebrating your working achievements with you or praising your dedication and merits. I didn’t even get a leaving card or a cake. It was a bit like I died- I just ceased to exist in the working world and life carried on for everyone around me.

I get it, to everyone else – family and friends included, not just work colleagues- it’s a case  of people don’t know how to respond. It’s not like leaving for a new and exciting job role, or a change in career, or going back to studying, where you can wish someone luck in their new projects. It’s not like giving up work to go on maternity leave or to concentrate on bringing up your family where you can at least make jokes about the perils of parenthood, while also wishing luck for the future. It’s not even like taking a break because I’ve got cancer or another illness with an end date on it and I’d hopefully go back to work sometime in the future. So people didn’t know what to say, and, truth be told, it’s only now I think about it that I realise what would have helped me at the time.

I realise now that I didn’t fail because I stopped working. But I felt like that was the case because of my feelings about it and because of the way people (inadvertently) made me feel by dodging the subject or the way they spoke to me about it. There should have been something to mark the successful bits of my career.It takes a bit of getting used to, being unemployed, even if you’ve been off sick for a while beforehand. It’s completely different and still comes as a shock. It also takes a bit of getting used to admitting you’re disabled. So until Christmas, I basically was trying to find where I fitted in the world without my job and all of the things I loved about having a job which came with it. Not to mention the sudden drop in income and the change in lifestyle which has to come with it.

I decided to knit everyone I knew Christmas presents, which kept me busy. But then I got to January, where everyone always feels sad and depressed anyway and it kind of hit me. What the hell am I going to do with my life now? The way I saw it, there was two options:

  1. Give up and become a victim-like personality and exist purely as my illness and nothing else.
  2. Find myself a reason to get up every day which gave me some sense of purpose. I can’t work but I can find something to do with my life which makes it actually worth living. I’m definitely not living the way I planned my life to be, but I can’t do much about the cards I’ve been dealt, so I may as well make the game as good a one as I can. Just because I’m incapacitated doesn’t mean I’m incapable. I still have worthwhile characteristics and qualities which didn’t just vanish when I stopped working.

Number 1 is by far the easiest. If people feel sorry for you, you get attention, and who doesn’t feel better from attention? You can use your illness as an ‘excuse’, some people will let you act in whatever way you want ‘because you’re sick’. But it’s more pity than attention, and it’s completely fake. And, if you’ve got a normal lifespan with your chronic illness, other people aren’t going to put up with you acting like that forever.

Number 2 is definitely harder. Most mornings I wake up and think ‘here we go again, I feel awful, I haven’t really slept, I wanted to do X today, but I probably won’t manage. Why is the kitchen so far away to make my breakfast? Can I really be bothered with this?’ In January, it was even worse and I really struggled mentally. So I decided to do something about it.

I sat down and thought about what I’d like to do with all my ‘free’ time if I wasn’t restricted by illness:

  • volunteer
  • support a charity
  • do sports
  • play music
  • start a business
  • meet up with friends
  • read
  • study something
  • Take up a hobby I do every day
  • travel

Then I put a line through all of the ones I can’t do because of illness. Meeting up with friends with no money wasn’t going to be a sustainable thing. Plus they have jobs during the day. Reading is something I struggle with. I’d been supporting Post Pals, a charity which sends post to seriously ill children to cheer them up over Christmas with their reindeer post scheme, so I contacted them and asked if there was anything regularly I could do. They said people send their kids post all year round. I know I like receiving post when I’m feeling poorly and depressed, so I decided to find something along those lines which I could do. I could do it from home when I could manage it with my illness, which was good for me too.

However, as much as sending post to children would give me something to do every now and then, it wasn’t really the regular thing I was looking for i.e. something every single day. What else can I do every day? Knitting. I was doing a lot of knitting. I could also write blogs on my phone. The thing with me and charity work is that I don’t do charity work for the ‘praise’ I get back. I actually prefer doing charity stuff if I don’t get feedback from whoever I’m doing it for, because it makes it feel less like I’m doing it for myself and more like I’m doing it for the ‘right’ reasons (in my head anyway). But, I was pretty isolated, and I knew that whatever I was trying to do every day needed to be something which involved a two-way interaction process with another human being, so that I didn’t get stuck in a depressive spiral.

So I had knitting, Post Pals and writing as my ‘things I can do most days’ list. I’d been making a lot of animals for my nephew because I’d got bored of making conventional things like scarves. The animals were good to make because they only took me a couple of days each and they knit up in sections, meaning I can start and stop them when I needed a rest without getting lost. Except my nephew was quickly developing quite a zoo in his bedroom, so that couldn’t continue much longer. But I thought ‘if he likes animals, then other kids will too. Maybe I’ll make some for the post pals kids‘. Another thing which I realised, is that I always have some kind of stuffed toy in my hospital bag because it gives me some kind of comfort when I’m feeling vulnerable in a hospital. Now, if I like to have something pocket sized to take with me as a 30 year old, those kids from Post Pals definitely will. What if the animal was their friend? And wrote a letter to them to say hello when they got sent to them? Or, what if the animals talked about what they’d been up to before they got sent to the child, and then the child could introduce them to their world?

Basically, I came up with: knit an animal (daily habit), persuade a friend to take out an animal and take photos of it doing things (2 way interaction for me), write a story (daily habit), post the animal and story off to the post pals children (charity). My doctors, especially my psychiatrist all heartily approved of me doing this.The friend who took the first animal out for me works in comms, so suggested social media to get more people involved, and because she found the whole concept quite amusing! I was having to work hard to convince people that they wanted to take a stuffed animal out with them places and take photos of it ‘pretending’ to be on an adventure (people thought I was a bit nuts), so I set up twitter, instagram and Facebook profiles, as well as an online blog for the stories, under the name ‘Out with Animals‘.

I was quite enjoying being sent photos of the animals doing fun things (and seeing what my friends were up to) and using my brain a bit to write stories- it appealed to the creative side of me that had kind of got covered in dust since stopping music teaching. Then around Easter time, a lot of people I know had holidays planned and, instead of me thrusting an animal on them and begging them to take photos, suddenly a lot of people asked if they could take an animal with them. And I’ve had some really imaginative photos from lots of people! I started using the social media pages I set up to ‘chat’ to places the animals visited- I had a lovely chat with Monarch Airlines about Lizzy the Lizard’s travel sickness from having eaten too much Easter Chocolate, I managed to get Eric the Elephant a City of Culture Badge by tweeting them about his adventures and the Kennedy Space Centre tweeted me back about Oscar the Octopus’s attempt to be the first octopus in space. Not only were the animals getting more and more adventurous- they’ve been to places all over, from Italy to Japan, but it really was helping me and my mental health. Around the same time, another charity called Help for Dominica emailed me and asked if I could send some animals over to Dominica to help with their school education and outreach programmes following Tropical Storm Erika. That was also pretty exciting, and I’ve sent 7 out there so far.

Now I’ve constantly got a ‘list’ of animals that people have requested to take out, and I’ve also had a few random strangers who have come across my page ask to be involved either by crocheting animals or because they want an animal to take out. Groups and organisations like primary schools, Scouts, Rainbows, Hospitals, the army, a mayor, and the police have asked to be involved somehow. That’s pretty cool for an idea that came out of me sitting in my PJs knitting one day in January!

Making animals is in no way filling the massive hole that teaching and having a job left. But it’s something I can do which helps other people, gives me some kind of a sense of purpose, and stops myself from hiding away and exacerbating my illness by isolating myself. I can do it at my own pace and stop when I’m having rough patches with illness. If you follow my social media, I tend to do an update before 8am when I’m doing my whole ‘here we go again….’ monologue- that’s intentional. It’s the hardest time of day for me, and even when I’ve been in hospital, I’ve still managed to post an update. It means that I can feel like I’m contributing to life even though I’m not out of bed yet and everything is really hard work. I’ve achieved something even if I manage nothing else for the rest of the day. It’s only a small thing, but it’s quite important for me to be able to do it. It’s not the same as having a career, but I enjoy doing it, and it’s a lot better than giving up and letting my illness take over completely. If nothing else, I’m proud of myself for that!

Emergency Medicine and AI: Why We Need More Awareness

This is intended as an awareness post, not me using it as an opportunity to slag off the NHS or particular members of staff. The main problems I encountered recently in a&e were because of a lack of awareness, not because of a lack of skill. There were many people who were on my side during my recent hospital admission, and I was happy with the way senior staff eventually handled my issues. However it was probably the most horrendous admission to date and is, sadly, the way that a lot of patients with Adrenal Insufficiency are treated when accessing emergency care. Hence the need for an awareness post.

I felt ill fast, oral HC wasn’t shifting it so I rang 999. The dispatch found my red flag and said I was on a red 1 (fastest call out) but there was a high number of users so she couldn’t guarantee how long. I decided I’d better do my injection myself to be safe but the crew arrived before I’d had time to draw it up fully and administer it so were able to give it to me IV. That’s a super quick call out by the way- 4 minutes from me picking up the phone to paramedics on scene. If I’m in suspected adrenal crisis, things are supposed to move that quick. An example of the system working at its best and most efficient.

I think their speed and our joint decisions possibly might have saved my life that night, because when we got to hospital is when it started to go horribly wrong.

I was triaged incorrectly on arrival

This was the major problem. An administrative error/lack of knowledge nearly killed me. The wonderful paramedic rang ahead to resus to let them know I was coming in but resus said that because the crew had stabilised me, I could avoid resus providing I stayed that way by the time I got to hospital. Yep, we all agreed that was a good plan. The paramedic handed over- it was the best hand over I’ve ever had- but the receiving nurse hadn’t heard of adrenal insufficiency or addisons and didn’t write it down and triaged me as ‘abdo pain’ instead, which was one of my symptoms, but not the cause. This meant that instead of having immediate bloods, fluids, IV HC and pain relief, I didn’t get anything and was put low down on the priority list, assigned to a very junior doctor with very little experience.

There was a delay in bloods

It took them an hour to come and bleed me, and that hour is crucial. A suspected heart attack patient wouldn’t have to wait that long, neither should I. They also should come back asap to either rule in crisis or rule it out, but it wasn’t prioritised because my chart was wrong.

I wasn’t given pain relief for an hour and a bit.

This is bad for any patient but particularly bad for me. Pain drains cortisol fast and I already didn’t have enough on board. The doctor wouldn’t write any up for ages so the nurses couldn’t give me any.

The doctor dismissed me to the nursing staff

I complained of feeling very unwell a number of times. I kept telling my nurses I needed more IV HC but they kept coming back saying ‘the doctor says your BP is fine so he’ll come when he’s ready’. I kept saying it wasn’t and tried to explain how my BP goes up rather than down when I’m acutely ill. But the doctor refused to take me seriously and kept passing messages via the nursing staff, making judgements based on my incorrect chart and his textbook knowledge.

I had a drug interaction/sensitivity which wasn’t dealt with

One of the anti sickness IV meds made me feel really ill and I blacked out for a few seconds. The nurse immediately told the doctor but he wasn’t bothered. This should have been followed with an IV dose of HC stat to counteract the further stress to my body. but it wasn’t given despite me asking for it. I hadn’t had an anaphylaxis reaction, which is what the doctor was concerned about, but he wasn’t aware that any kind of reaction in a patient with AI causes us huge complications without appropriate treatment.

I developed a temperature which justified an IV dose

But again, my request was ignored because the doctor wasn’t aware of sick day rules.

I was refused IV HC despite clear, clinical reasons to back it up.

Which is unacceptable. I tried to get my HC refusal letter out, given to me by Addisons U.K., which basically says ‘I have made you aware that I have a life threatening condition which you have refused to administer medication for. Giving me IV HC won’t cause me any harm in the short term but could kill me if I don’t have it’. But I must have handed it over in my pack the last time and forgot to replace it- that’s completely my fault. But the letter is meant to be used as a last resort, so I shouldn’t have remotely needed to have got that far in the first place.

The doctor tried to cover up his mistakes

Some doctors find it hard to admit when they’ve made a mistake and plough on regardless. Which might work if you’re not a frequent visitor to a&e, but doesn’t when you know how everything is supposed to work. When he eventually came to see me, he didn’t take a patient history. He didn’t ask me about other medical conditions. He said he’d run a set of bloods but because I was triaged incorrectly and he didn’t come to see me before ordering them, they didn’t include ones that were appropriate for me, but for abdo pain instead. Aside from that, this admission was caused by emotional stress not infection, so that won’t show up on the blood tests he’d run. He then waved the chart at me and declared me fine and that I could go home. Basically he had his plan worked out based on my chart and I had to fit into it. I’d used my injection, meaning I no longer had one, so I said I was worried I’d decline again and wouldn’t make it back to hospital in time if I needed it. His reply was awful- I’d be fine because I had my oral meds. No. Oral meds won’t save my life. And at this point I couldn’t sit up let alone walk.

He insisted he’d followed addisons protocol and gave me a patronising lecture as to why he was right and I was wrong. Except he hadn’t followed any addisons protocol at all, which I pointed out. His reply was ‘I’m not taking responsibility for this, you only became my problem 10 minutes ago’.

Stress makes me more sick so by now I was a big mess. And feeling terrible.

I asked for the nurse in charge who went and got the most senior doctor in the department. She apologised profusely many, many times and tried to help sort a plan for that night and for the future. I liked her thinking, she was fantastic. And I got my IV HC stat. She started off backing up her colleague, which is to be expected, but once I’d explained she said she hadn’t been told the full story. In short, people were either not communicating or they were choosing what to communicate to each other.

If junior doctors are reading this (or any HCPs for that matter), I don’t expect you to know everything and I’m happy to help fill in the blanks. But you can’t and shouldn’t rely on a chart telling you everything is ‘fine’ when the nurses are coming and telling you it isn’t. Take responsibility when you mess up- yeah you’re liable but patients have a lot more respect if you say you’re unsure rather than trying to quote a protocol which you’re getting wrong anyway and definitely didn’t follow. Patients who go to hospital a lot know a lot about their illness, don’t patronise us. Don’t call me ‘a problem’. What a way to show compassion! Bite the bullet and apologise on behalf of your colleagues/whatever went wrong because a lot of healthcare is handholding- if he’d apologised I maybe wouldn’t have eaten him alive so much. Yeah it sucks, but say something like ‘I’m sorry you’ve had such a rough night’ or ‘I’m sorry you felt your care hasn’t been very good, what can I do to make it better now’ if you can’t bear to actually apologise. It’s apologising but not actually taking liability for it. And it goes a long way in improving patient experience.

I shouldn’t ever have to say the words ‘I could die if you don’t listen to me’, which I did say a lot that night. Why wait until I’m critically ill because you can’t think outside the box? Think about the bigger picture. I’m not just a bp and a set of bloods. In 19 years of having worked for the ambulance service, I was the paramedic’s second AI patient and she was genuinely gutted that her student was on his day off because she wanted him to have learnt about it. As a chronically ill, rare patient, no one is expecting you to diagnose or wave a magic wand. But you need to treat the patient, not the chart. If you can’t see beyond that and aren’t willing to learn, to be blunt, you shouldn’t be a doctor.

I’ve been incredibly lucky in that I usually have very good care at that hospital and that night was a blip. But that’s unfortunately how a lot of patients with AI or are treated a lot of the time. It’s not because the staff aren’t skilled enough to be able to manage it, it comes down to a lack of awareness of the condition and how we can deteriorate very quickly. There is nothing more terrifying than having to argue for drugs you need to stay alive when you feel like you’re going to lose consciousness at any second and are panicking that if you did pass out, the staff wouldn’t know what to administer or why. A little bit of education would go a long way in treating this disease.

I don’t like ending on a negative note, and actually I’ve had some very positive A&E experiences. Read this post to see how good emergency care for Adrenal Insufficiency can be 🙂

I have to repeat my synacthen test (again)

Here’s hoping for third time lucky…The synacthen test is the diagnostic test for adrenal insufficiency. It works out if your adrenal gland is working and/or if your pituitary gland is sending the hormone ACTH to the adrenal gland to tell it to make cortisol. I did a repeat one last year because a doctor thought I might not be cortisol deficient anymore and said I could come off steroids. The test showed he was very wrong, my adrenal gland was making only a dribble of cortisol and I definitely did need steroids. However, because they weren’t expecting those test results, they only ran the cortisol bit of the test and not the ACTH bit, despite me asking them to. And now it’s been decided that we need that result too, so I have to repeat the test, which I’m a tad grumpy about.

The test itself is easy. Blood test, injection, 2 more blood tests, done within an hour and fifteen minutes. Easy peasy. But if you’re already on treatment for AI, it means coming off steroids beforehand, which is not good since you get told at diagnosis ‘if you miss a dose, you could die’. Last time, I had to come off steroids for about 28 hours, which was horrendous and I ended up in resus soon after. This time, they’ve said to do 15 hours. It’s a bit like saying ‘we’re going to take some of the oxygen out of the room for a little while. Chances are you’ll probably be fine, you’ll just feel really crappy, you might hallucinate, be in pain, perhaps fall into a coma, but most people are alright so you will be too‘. A lot of it, I’m aware, is psychological. I didn’t die before I started treatment from low cortisol, so chances are I won’t now. Except for emotional distress or injury or illness, so please keep everything nice and calm for me and germ-free for the next couple of weeks 🙂

So, you might be thinking I have a diagnosis of AI, so why do we have to do the test again? Especially as I’ve already done two, and the results are always going to be slightly skewed because I’m on steroids.

It’s because we’re trying to work out if I’m primary or secondary AI. The treatment for AI is largely the same either way, but it might help get to the bottom of some of the other weird endocrine things which are going on. In a synacthen test, here’s what’s supposed to happen:

If you’re primary AI, your first cortisol blood test will be very low but your ACTH level will be likely very high. This is because your pituitary is constantly sending out ACTH to the adrenals desperately trying to make them work to produce cortisol and wondering why they’re being stubborn and doing nothing. When you’re given the ACTH injection, you’ll end up with more ACTH in your blood and still no cortisol. Your adrenal glands don’t work. The primary problem is with the adrenal gland.

In secondary AI, you might have a cortisol reading to start off with, but it’s likely pretty low and your ACTH will be non-existent. So ‘normal’ people at 8am should have a cortisol level of about 500 ish, give or take. Someone who is secondary AI might have a baseline reading of 200-300 or less, but it’s still nowhere near what it should be. Sometimes it might be 400 ish. The important thing with SAI, is how your adrenal gland responds to the ACTH injection. If it ‘stimulates’ in response to ACTH, e.g. roughly doubles the cortisol, it means your pituitary isn’t functioning properly and there isn’t any/enough ACTH coming from it to make the adrenal gland work. So the problem isn’t with the adrenal gland itself even though it looks that way, but because the pituitary isn’t doing its thing it’s causing a secondary problem.

I’ve done 2 synacthens now and failed both. However, my results are a bit odd which is why we really could do with that ACTH result. Last year when I did the test the cortisol results were (nmol/L):

Baseline (before injection): 18

30 minutes blood test: 54

60 minutes blood test: 65

So I had a very slight amount of cortisol production to start with. But bearing in mind a healthy person’s cortisol at that time of day is 500, that’s very low. Then it did increase a bit, although it’s still far from the ‘safe’ range which starts at 150 (ish). In other words, I shouldn’t have been walking around and talking before the test, but somehow was. I saw a consultant a few weeks ago who looked at the result and said ‘that’s your synacthen? How is that right?’. I had a really low reading to start with, and then it didn’t really move (like primary). But it did double the result technically (like you’d expect in secondary), but if my adrenals were working properly and I was actually secondary, it should have gone higher than that. You can also be both primary and secondary AI at the same time ie the adrenal glands don’t function well and the pituitary also isn’t doing its thing.

I could speculate about what my last synacthen potentially means until the cows come home but it’s a pretty pointless exercise because, knowing my luck, my endocrine system will throw a curve ball and it’ll be doing something completely different at this synacthen. A lot has changed in the past year, but I wouldn’t say no to a miraculous recovery though!! The reasons I want to have some kind of answers from it include:

– the potential of some other treatments e.g. replacement ACTH or aldosterone which might help some of my symptoms

– we might be able to see if there are any other endocrine loops which are broken and treat those

– hopefully my baseline dose will be looked at again. That’s another blog post for another time though.

– I hopefully won’t have to have repeated conversations with other doctors which include me trying to explain the differences between primary and secondary and why we’re not sure which one I have, but can they please bear both in mind while treating me ‘just in case’ it helps. Most doctors don’t understand both versions and only remember one of them or get confused, so it’ll be good to just have one to talk about!

Hormones are fascinating. Did you know the adrenal cortex is made up of 2 bits and the bit that currently doesn’t work in me is responsible for about 90 hormones? But I’m only taking medication to replace one of those. 1 out of 90. Now you can see why I don’t give people much sympathy when they blame their bad moods on hormones- if you think PMT is bad, you should try having 89 hormones missing completely! (Guess which bit of your body is responsible for PMT as well…!)

‘Let’s Reduce Your Steroid Dose’

No, let’s not. When I hear that from a doctor it makes me want to jump up and down and have a strop. Unless they’re making an informed decision, which they’re usually not. This was from an endocrine registrar at my 6 month review. Cue lecture from him about steroids being bad. It’s always the same:

  • ideally we want you to be on 10/5/5. You’re currently on a much higher dose.
  • steroids can cause you problems with your blood sugar and put you at risk of diabetes.
  • steroids make you put on weight. You’ve put on a lot of weight since your last review.
  • they also make your bones weak and you’ll end up with osteoporosis.
  • it’s not good for your heart to be on high doses of steroids.
  • you’ll have other problems with your stomach and teeth crumbling because of steroids.
  • you might get cushing symptoms (too much cortisol).

To a lot of doctors, steroids are BAD. End of. I agree, if you’re taking them to reduce inflammation or swelling or following an operation or for something temporary. Long term steroids are bad, particularly at high doses and you want to taper off them asap, otherwise they can have bad consequences for your adrenal cortex. That ship has sailed! However, there isn’t another treatment for Adrenal Insufficiency currently, which means steroids are necessary, despite side effects. We’re only replacing what our body doesn’t naturally make.

So I took a deep breath and started my well rehearsed speech as to why I am not going to reduce my steroid dose from what it currently is unless there’s a better reason than the doctor feels like it.

You should be on 10/5/5

12.5mg of Hydrocortisone is the survival dose, which means lots of doctors see 20mg (10/5/5/) as them being ‘generous’. However, 10/5/5/ doesn’t take into account different people with different weights, metabolisms, day to day activities, other illnesses… It’s how old school diabetes used to work- everyone had to take the same dose of insulin and get on with it. Except now they can change their insulin depending on what their blood sugar is doing because doctors have realised how stupid that advice was and how crap diabetics’ quality of life was like that. Like diabetes, Adrenal Insufficiency isn’t a one size fits all, but modern medicine has not caught up with this yet. So yes, I’m on a higher dose, but I can’t get out of bed on 10/5/5.

Problems with blood sugar

Well, I’m already Insulin Resistant because one of my previous endocrinologists didn’t listen to me and my ’10/5/5 isn’t enough’ speech. So to compensate for the lack of cortisol in my body and to stop myself going hypoglycaemic (and ending up in a coma), I had to eat a lot to raise my blood sugar. Like every 10 minutes eating. So now I’m insulin resistant. Steroids make your blood sugar go up, and actually, my blood sugars don’t ever go above 6, even after I’ve eaten, which is well below ‘diabetes concern’. So I’m currently more at risk of being hypo- rather than hyperglycaemic.

Steroids make you put on weight

Yes they do in a lot of people. However, I was steadily losing weight on my Low Carb High Fat regime, until my GP said the fateful words ‘let’s reduce your steroid dose by 2.5mg’ in February and then I started putting on weight again. Why? Because I started having hypos, which you have to treat with food. Plus I feel like I’m going to pass out and am hungry all the time unless I eat. Whereas before, I wasn’t snacking between meals or eating at night. Now I’m up 2 or 3 times a night feeling awful. If I take more Hydrocortisone, I go straight back to sleep and don’t need to eat. Which would you rather- should I eat more overnight or take more hydrocortisone? Because you’re saying both are bad, but I’d quite like to avoid dying. Besides, now that I’ve put on weight again, if anything, my steroid requirement will have increased rather than decreased. So decreasing it would be doubly bad.

They make your bones weak.

Also true. I already have osteopenia (pre-osteoporosis). However, do you know what else is bad for your bones? Not getting out of bed and moving. And also putting on weight. So on a slightly higher dose I can do my physio and lose weight, and eventually my overall steroid requirement will reduce and I might even be able to manage on 10/5/5. Surely that extra 2.5mg in the short term isn’t going to make that much difference to my already weakened bones? But being able to move my joints and lose weight will definitely have a positive impact. Besides, what’s the point in having slightly stronger bones in old age when I didn’t manage to get out of bed for 30 years…

It’s not good for your heart.

You could say that about many things. Alcohol and smoking aren’t good for your heart but people still do it. I’ve been adamant that there’s something weird going on with my heart for about a year, especially overnight, and how this is linked to my steroid dose, but got told a few times that I was ‘just anxious’. My sleep study showed I have an irregular heart rhythm while asleep, so not due to anxiety. When your electrolytes are ‘off’ (low cortisol), it can cause a funny heart rhythm. And I don’t get that heart rhythm if I take more steroid. So maybe my steroid dose isn’t actually high enough?

Other problems

I could get a stomach ulcer just because I get a stomach ulcer. These things happen to ‘healthy’ people too. If I’m going to get these things because I’m going to get these things because I’m taking steroids, they’re going to happen regardless of whether I’m on 10/5/5 or my current baseline.

Cushings

Has anyone tested my blood level to see if I’m remotely near cushingoid? No? In which case we’re not changing the dose ‘just in case I get cushingoid’. If my blood work suggests it, then I’ll reduce my dose, but not otherwise.

So yes, I agree that being on the lowest dose of steroid which my body needs is definitely a good thing. But I’m not decreasing it now when you don’t have any blood work to support it and I’m still very symptomatic and have a poor quality of life as it is.

I obviously didn’t say it in as sarcastic a tone as I’ve written it, but I have this argument so many times a year it’s getting tiring. This doctor was actually pretty amenable and agreed with me after I made my points. A lot aren’t though, which is very, very frustrating. I have absolutely no issue if the doctor is making reasonable, informed judgements based on my symptoms and blood test results. But most aren’t- they are adamant that 10/5/5 is *the* dose I should be on because anything over that is bad because steroids are bad. And that’s what they’re basing their decision on, which isn’t right. There’s a bigger picture here. Yes steroids are bad, but they’re also what keeps me alive and living, so actually, steroids, to me, are pretty damn good.