Adrenal Insufficiency & Adrenal Crisis

Lots of people have similar questions, so i thought I’d write answers- sometimes I don’t explain it very well in conversations! It’s written so that non-patients/family can hopefully understand.

What is Adrenal Insufficiency?

Adrenal Insufficiency is a condition where the adrenal glands don’t produce any or enough of the stress hormone cortisol. Cortisol is required to stay alive and provides energy. It’s usually higher in the morning and decreases as the day goes on. The adrenal glands produce more cortisol to help combat ‘stressful’ situations the body encounters. You might have heard of ‘fight or flight’ reactions- cortisol plays a huge role in this.

Primary Adrenal Insufficiency (Addison’s Disease) is where the adrenal glands themselves don’t function. Secondary Adrenal Insufficiency is where something else is stopping the adrenal glands from functioning, for example, messages from the pituitary gland not reaching the adrenal gland telling it to produce cortisol.

Some patients take their replacement and can maintain normal lives. Others have symptoms including: nausea, pain, dizziness, fogginess, fatigue, poor memory, low blood sugar, problems sleeping, weight gain, anxiety, shaking, sweating, emotional outburtsts, irritability… In some cases of Secondary Adrenal Insufficiency, the adrenal glands can ‘wake up’ and start producing cortisol again. Each patient is different- the endocrine system is complex.

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Photo: AI United

How is it treated?

Patients see a consultant called an Endocrinologist and are prescribed replacements for cortisol, commonly known as steroids. There are a few different treatment options, depending on whether you have primary or secondary, and any other conditions which led up to diagnosis. Mine is Hydrocortisone, which I take 3 times a day: 10mg at 8.30am, 5mg at 12.30pm and 5mg at 5pm. This is my baseline dose, each dose lasts about 4-6 hours and taking them at the right times is really important. Notice how the morning one is the biggest dose? That mimics the idea that cortisol should be higher in the morning.

Stress dosing is doubling the dose- so 20mg/10mg/10mg for me. I do this when my body needs extra help to fight whatever is causing a stress on it.

Updosing is when I encounter a one off stressful event, which might not require the full stress dose. In this case, I wouldn’t double my doses, I’d keep my baseline but maybe add another 5mg in to see if that helps.

When would you stress dose/updose?

The most common things I stress dose for are infection, high temperature, injury, vomiting/diarrhoea or emotional upset. I would also need to stress dose for minor surgeries or dental work and long haul flights. Anything requiring anaesthetic or invasive procedures would require supervision at a hospital and possibly IV hydrocortisone treatment.

I would consider an updose on days where I’ve ‘done too much’ and I’m struggling to manage symptoms e.g. physical or emotional exertion. I’ve also updosed for short haul flights, minor dental work or for situations which I know I’d find stressful, to cover me just in case. It’s a bit of a guessing game- ‘normal’ people might feel stressed or anxious several times a week, but their body compensates by making cortisol. I have to try to preempt my ‘stress’ a bit so that I can take my medication- life doesn’t always give you a 20 minute warning to prepare though!

Once the ‘stress’ is over, you need to taper back down to your baseline dose (which is never pleasant). It’s best to be at your baseline as much as you can. Imagine being on a rollercoaster and constantly going up and down and how sick you’d feel. If you’re consistently adding in doses or stress dosing, that’s what it feels like. It’s also not good to have too much cortisol in your system- this can lead to Cushings Disease. It’s a tough balancing act to get right.

What’s an Adrenal Crisis?

Adrenal Crisis happens when the body hasn’t made enough cortisol and/or it hasn’t been replaced by medications. It’s life threatening and can happen quite quickly, for example, after a car accident or when someone experiences shock. Sometimes it can happen because the patient is fighting an underlying ‘stress’ they weren’t aware of e.g. infection. The symptoms of an Adrenal Crisis are:

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Photo: AI United
If in doubt, but particularly if the patient is unconscious, ring 999 stating ‘adrenal crisis’ and administer an emergency injection. You have to assemble it, mix the saline and powder, draw it up and inject into the thigh. Mine looks like this:

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Not all ambulances carry Hydrocortisone, which is why the emergency injection is important (even if it is a bit fiddly!). Patients should get themselves Red Flagged by the ambulance service and wear medical ID to alert people that they need an injection if unconscious. Once at hospital, treatment and/or observations would start, normally including IV fluids and Hydrocortisone along with other appropriate treatments depending on the patient.

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Photo: Great Ormond Street Hospital

Banner Image: AI United

Why Living with a Rare Illness is Hard.

Living with any Chronic Illness is hard. Especially for those who have more than one. Living with a rare illness is more challenging. Mine is Secondary Adrenal Insufficiency. There’s an internet Meme which says ‘where the f*** is my adrenal gland anyway?!’. Until 18 months ago, I didn’t know either.

The actual illness itself might not be as limiting as other chronic illnesses. There are definitely people who are sicker than me who have really well-known and recognised illnesses. This isn’t about symptoms, it’s about what I personally find difficult about having a rare illness.

  1. You can’t just Google it. If you Google ‘Secondary Adrenal Insufficiency’ you mostly get medical articles, which are really hard to understand, articles about Addison’s, which isn’t quite the same thing, or articles about Adrenal Fatigue. Adrenal Fatigue itself isn’t life threatening, Adrenal Insufficiency is. Big difference. If you Google a well known illness or a surgical procedure, you get step by step information, support groups, blog posts, charities, awareness campaigns, Facebook pages…etc The worst thing for me was I didn’t know if new symptoms were ‘normal’ for my illness or if they were something I should be telling a Doctor about. I went to my Doctor, she didn’t know so she Googled it too and didn’t understand either.
    2) You start to question Doctors. Once you’ve seen your GP open Google and look baffled, or she’s asked you to read a test report yourself ‘to double check’ her diagnosis, it makes you start to question them. Doctors don’t know everything (or anything) about your illness. Most people can trust their Doctor when they say ‘your blood results are fine’. I now have to follow this with ‘what were the exact numbers please?’. The endocrine system is so complex that individual tests might come back as ‘fine’, but combined they can be an indication of other issues. So if my GP doesn’t know for sure, this leads me to hours on Google reading medical articles so that I can make sure my Doctor doesn’t accidentally kill me. (NB I like my current GP, she’s really good at working with me).
    3) Everyone has an opinion as to what will ‘cure’ you. This is a common irritant amongst people with chronic illnesses anyway, but no-one knows anyone with my illness so they try to compare it to something they do know. Or they decide that if they haven’t heard of it and you can’t see it, it can’t actually be that bad and I’m just a drama queen. The worst conversation I had was with a woman in a GPAU waiting room who had major heart problems requiring complex surgeries telling me that if I exercised more and pushed myself, I would feel better, because that’s what worked for her. I felt upset because she actually has chronic health issues herself and therefore must know how irritating it is to be given unasked for advice, but she’d decided that because her’s was heart related and she didn’t understand mine, she clearly knew best.
    4) You start to risk assess your daily life. I’m quite practical about living with AI, some people avoid going out at all for fear of dying. I figure that anyone could be in a car accident and die, even if it is more dangerous for me. But I do wear a medical alert and have an alert in my car and on my bag. I’m red flagged with the local ambulance service and hospitals. I know where the nearest hospital is. If I start feeling poorly quickly, I ask myself the question ‘how long have I got before I might pass out?’ when deciding on if I need an ambulance so I can explain what dose of IV HC I need or what my protocol is. And not all ambulances carry the life saving drug I need.
    5) It’s a guessing game. Literally. Asthmatics have Peak Flow Metres, Diabetics have Blood Glucose Metres to help them manage their conditions. I have to guess what my dose should be, there aren’t enough patients with AI to justify funding a metre. And then if I deteriorate more, I go to hospital and get them to run labs. But even then it’s just educated guessing- yes I might have a numerical value, but the only way of testing to see if the dose is adequate is by doing labs again once adjustments have been made. And it can all change in the space of an hour anyway. Clearly I can’t go to hospital every time I need to change my dose, so I have to ‘guess’. And guessing with an illness which can kill you if you get it wrong is great fun!
    6) I haven’t found anyone with the exact same illness/symptoms as me. I’ve been researching for 18 months. I’ve now found 2 Facebook Support Groups with people with similar conditions, but I’ve not found anyone with the same diagnosis, symptoms and medications as me yet. People should always be seen as individual patients regardless of their illness, but because of the nature of endocrine disorders, any slight variable can rapidly change the way it’s treated. It’s really isolating.

Photo: AI United (AIU)

Treehouse Wedding- Alnwick Gardens

When we were planning our wedding for Autumn 2014, neither me or my husband wanted a ‘traditional’ wedding. After some googling, we came across the Treehouse Restaurant at Alnwick Gardens and loved the idea. We arranged to visit before booking, spoke to Wendy who organises events there, and agreed a date before leaving. It was the only venue we looked at, we loved it. It’s also not as expensive as you’d think!

The day we visited was an awful day- raining (normal for the north), windy, dark. But it was still beautiful. It might be a treehouse, but it’s fully accessible for wheelchairs and push chairs. There’s a long ramp up to a covered viewing point and then a further walkway to the Potting Shed (bar area) and an additional space which we used as a place for our guest book and put some family photos on the screens in there. If you’re feeling adventurous, there are some rope bridges you can cross (good photo opportunity).

 

 

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Covered viewing point and walkway

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Potting Shed (right)

The Treehouse is magical. We’ve been there four times in total, including our wedding day. There’s a big, log fire in the centre, and everything is made out of wood- grand wooden chairs, tables, branches decorated with fairy lights. It’s not square- it has hidden nooks and crannies. Photos don’t do it justice. Coincidentally, BBC 3’s Don’t Tell The Bride episode featuring the Treehouse aired soon after we booked it and we both commented that the camera couldn’t pick up all of its qualities.

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Organisation wise, it was a walk in the park. We live 5 hours from Alnwick, but Wendy and her team arranged everything for us- we told them what we wanted and what we’d provide and she sorted it. She also had some great advice which we followed, for example ‘don’t overdress the Treehouse or tables, it’s so pretty that simple makes everything pop more’- She was right. Last minute issues or requests, she sorted it. A man had a medical emergency when we were visiting once, she was cool as a cucumber.

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Once we’d booked we were offered a complimentary tasting of the suggested wedding menu. We were able to choose 3 dishes for each course to provide our guests with options. We opted for: chicken liver pate; cream of tarragon mushrooms on toast, warm pigeon salad for starters. For mains: supreme of chicken; roasted lamb or cheese, red onion and chive filo parcel, and for dessert: lemon tart; Bailey’s Cheesecake or Chocolate Brownie. There were also options for the Children’s menu. The food is incredible. There were three options for hire: all day, from midday-midnight and from 5pm-midnight. We opted for the latter and it was the perfect amount of time. You pay for the venue hire (discounts were offered for certain months/days) and then there’s a minimum spend. After many calculations, we worked out that for 70 guests it was cheaper to order alcohol from the Treehouse rather than pay a corkage fee and supply our own.

We arranged a meeting with Wendy the day before to confirm arrangements for the day and drop off the table plan, wedding favours, photos, guest book etc. The wedding ceremony itself takes place with everyone standing facing the raised area with lanterns on the floor making the ‘aisle’. Chairs could be provided for elderly or disabled guests. Once the ceremony was over, we had drinks out on the walkway and in the Potting Shed and Wendy’s team set up the tables, chairs and places according to our plan. My husband came up with a table map which was complicated to set up (involving jam jars, battery powered tea-lights and lemon squash!) and they followed his instructions perfectly. We hired a pianist to play and sing (Ashley Humble) during the meal and made an iPod playlist for afterwards which we gave to Wendy. There is an area by the fire which can serve as a dance floor, but it isn’t large. There was a microphone available for the speeches.

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Wendy recommended the Hogs Head for accommodation since all of our guests needed rooms. We also hired a coach (£200) to transport guests to and from the Treehouse- I’d recommend this as it made life much easier rather than trying to persuade 70 southerners that taxis aren’t common in the North. Getting a photographer who has worked there before is a must- like I said, photographs don’t do it complete justice. Andrew Davies, our photographer, was great. He listened to what we wanted, he didn’t ‘get in the way’ and he was prompt in sending us the photos.

We had lots of people say that our wedding venue was the best they’d seen and even our hard to impress family and friends were suitably stunned by the location and the food. I’m biased, but it was the best wedding I’ve been to!

Details based on Autumn 2014 Wedding. Photos used were taken by us or our guests.

10 Ways to Manage Adrenal Insufficiency.

‘You’re absorbing steroids for your asthma well is the good news. But your short synacthen test shows you have Hypoadrenalism, so you will need to keep taking Prednisolone every day and see an Endocrinologist’. And then to the nurse ‘7.5mg Prednisolone stat’.

That was my diagnosis of Secondary Hypoadrenalism or Secondary Adrenal Insufficiency. Hypo-what? End….? I couldn’t even say half of the words, let alone remember them. And back on steroids? That wasn’t the point of 2 weeks in hospital! The only thing I’d really registered was that he’d said ‘stat’, which, from my hours of Grey’s Anatomy watching, meant ‘quickly please!’.

It felt a bit like I’d been abandoned and I had to learn a lot of the stuff I know now from experience, or from reading medical journals and support groups on Facebook (a real life-line!). Now it seems pretty obvious, but it was a steep, difficult learning curve and I quickly learnt that I knew more than my GP. Here are 10 practical things I’ve learnt based on my experience which I wish my Doctor had actually said when diagnosing me. NB: this is not a replacement for your Doctor’s advice!

  1. Take your replacement (mine is Hydrocortisone, changed soon after diagnosis) at the same times every day. It’s annoying having to live by this but you will feel so much better. My doctor told me ’10/5/5mg 3 times a day’ so I was doing it at meal times, which aren’t always at the exact same time. Work out a schedule which suits you, every 4-6 hours (if on hydrocortisone) to keep in line with a normal circadian rhythm, even if it means getting up early at weekends. Keeping ahead of symptoms works better than trying to get on top of them.
  2. Cortisol is like a bank. It doesn’t replenish itself over night like lives on the X-Box. Your steroids act as a deposit into it but if you’ve been ‘robbed’, you have to make extra deposits to make up the difference in balance. Deposits could be: stress dosing, sleep, rest or, for me, carbohydrates.
  3. Follow your stress dosing Sick Day Rules but pay attention to your body. If you’ve not been given the Sick Day Rules (I wasn’t), ask for them. Everyone responds to ‘stress’ differently, so why would illness be any different? Don’t get confused though- you’ll likely feel nauseous and fatigued with an illness normally, that doesn’t necessarily mean you need to stress dose, that could just be part of the illness- adrenal insufficiency is great isn’t it ;)?
  4. The most useful thing I read was: if you feel overly sick, tired, have headaches, dizzy, have stomach ache etc take 5mg or 10mg more. An updose (one off dose) won’t do you any harm in the short term. Not dosing enough could kill you. This works well for things where you don’t need a full stress (double/triple)dose. Eg a filling at the dentist or a short haul flight- 5mg half an hour before usually covers me enough.
  5. Rapid tapers don’t always work. A lot of sick day rules say ‘return to normal/baseline dose the next day’. If I do that I crash and burn spectacularly and can’t get out of bed for a few days (read: sitting up to eat is too tiring). I find a taper of 5mg every 3 days works well for me. If on the third day I feel really rough, I’ll leave tapering another 5mg for a few more days.
  6. Not all Health Care Professionals have heard of or know how to treat AI. Or they might think they do, but not every patient fits the ‘textbook’ symptoms. So if something you’re told doesn’t make sense to you, it’s ok to ask for a second opinion (from someone more senior). A good trick is to ask doctors to repeat their advice or repeat it back to them and see if they say the same thing a second time. Chances are, if they’re unsure, their advice will change and they’ll contradict themselves.
  7. Get to know your symptoms and be your own advocate. You shouldn’t be left waiting if you go to A&E, even for triage. Remember the point above, you might have to be firm or forceful. Lab results (like random cortisol, potassium and sodium) can take an hour, and an hour is enough time to kill you if you’re having a crisis and don’t get the right treatment.
  8. Make sure you’ve covered yourself for emergencies. Buy a medical ID bracelet and write your name and something along the lines of ‘adrenal insufficiency, requires emergency injection/solu-cortef’ on it. Carry your emergency injection with you. Get yourself Red Flagged by your local ambulance service so that they prioritise ambulances carrying hydrocortisone and a quick call out. You have to do this every year and it can be done by emailing the local service.
  9. Carry extra oral hydrocortisone all the time. It’s handy for unexpected updosing but if you end up in hospital, they never have it on the wards and often don’t appreciate the time sensitivity of it. So it’s useful to have your own so you can tell your nurse ‘I’m having this now’ if you really have to (nurses tend to ‘get it’ more than doctors, so make friends with them!).
  10. Use your smartphone. Use the health app on your iPhone (the one with a heart icon) to put your medical info in as an alert. This can be accessed by paramedics even when your phone is locked. The same app can be used to record symptoms, medications and vitals (BP/HR/Blood Glucose), which comes in handy when you’re asked to keep a diary. I’ve also got a photo album on my phone called ‘Doctor’s Notes’ which has all of my letters from Consultants and protocols. If there’s a problem or someone needs information, I can just show them on my phone.

Photo: AIUnited (AIU)