International Travel with a Chronic Illness – Part 2

Australian Triip – Day 1-3

I had just done all the preparation for our Trip to Australia.

I sat on the train on my way home from work going through things in my head.  Everything that I had done to make sure it the trip went smoothly was ticked off.  Then it came to me.  Derek took DHEA.  It is a steroid.  But what type of steroid?  I grabbed my phone and looked it up.

Well of course it would be an Anabolic steroid, and prohibited for import into Australia.

Great, this meant going on line and filling in an application to import a prohibitied substance.  It also meant a letter from Derek’s Dr confirming it was prescribed for a medical condition.  When I got home that night and pulled out the laptop, found the link to the Australian Customs website and hit apply.

I was amazed that I had a response, with a form, arrive in my inbox within 2 hours.  This meant I could take it to our Dr’s visit the next night, and have the application off by Friday.  I was cutting it fine.  It was supposed to take 20 days to get it approved, and I had 18 working days before we flew out.

We went on the Thursday night to the Dr and discussed everything we needed:

  1. A list of medication for NZ Customs
  2. A letter for Australian Customs regarding the DHEA
  3. Did we need to get an INR while in Australia.
  4. Did we need to carry Clexane incase Derek’s INR dropped too low.

After the discussion the Dr felt that, as it was only a 4 hour flight, and we were in Australia, then an INR check would not be necessary, and if something happened, they could give him clexane anyway.

We then did an audit of all his medication and put enough in containers for a months supply of everything.  If something went wrong and we had to deal with it over there, we wanted enough medication to last for the month at triple dosing, not just a week of normal dosing.

The last thing Derek had to do was go out on the Friday morning and get an INR.  It came back in the afternoon.  It was 2.9.   His preferred level is 3.2.  It wasn’t considered low, was lower than we prefer.  It could be easily managed.

We were ready for our trip.

The night before we flew out Derek took extra hydrocortisone.  We set the alarm for 4am, and I put all the bags in the car.  All we would have to do was get up, get dressed and go.

I woke up before the alarm.  In fact, I woke up half an hour before the alarm, so I went and emptied the dishwasher, and tidied up the kitchen.

At 3:50 I was dressed ready to go and I woke Derek.  All he had to do was put his cloths on, grab his normal daily bag, and get out the door.  He took 20mg of HC (normal morning dose is 10) and we left.

He closed his eyes when we got in the car, and rested.  I drove to our son’s house to pick him up.  Derek was starting to come round as we got to the city so was awake (a very loose description) when I pulled up outside Colin’s home to pick him up.  He was taking the car to his place from the airport.

I told him we would stop at the 5 minute drop off, we would unload, and he could go.

As I took all the bags out of the car I looked around.  There was a baggage trolley outside which I grabbed to put our bags on so Derek didn’t have to carry anything.

As we entered the Departures Checkin I looked around.  Directly in front of Virgin Airlines Check In counter was a wheelchair.  Great, they had it.  Derek needed it.  We went up to check in “Hi, we are here to check in, and is there a wheelchair for Derek?”

“Yes.” The checkin operator looked over to the chair I had seen.  “It’s over there.  Give me your tickets and passport and I will start doing that while you go get it.”

“You can’t take that!”  I stopped to see another check in operator looking directly at me as I was reaching gfor the wheelchair.

“It’s OK.  It’s for my husband”  I pointed to him, leaning against the counter 2 check-ins down.

“Oh, that’s ok.  I thought you were from the Qantas Flight.”  She had looked to where I was pointing.

We finished the check in process, then piled our carryon bags on Derek’s lap and went for breakfast.

Fast Track to a Holiday

I wasn’t sure what would happen when we attempted to go through the Departures Gate.  It was a lot easier than I had though it would be.

As I pushed Derek through the barrier for passengers only I noticed another woman pushing her husband.

We both took the long cattle track as it wasn’t made clear that we could take a short cut.  As we arrived at the security scanning area I unloaded everything to go through the x-ray machine.  I then pushed Derek to the Security Gate.  He was asked if he could get out and walk through.  He did, but the wheelchair was a little too close and it set the alarm off.  He then had to stand and be checked with their hand held metal detector.  He was clear.  I pushed the chair through the side entrance, then went through the scanner myself.  I too was clear.

We piled Derek up with everything again, and moved through to the duty free zone.

A sales member came and help us with our purchases after which we went to the gate to wait.  I grabbed a couple of bottles of water, put some electrolytes in Derek’s and settled him with our bags.

Right beside the gate was a coffee shop.  Yay, finally we could have a coffee.

I cued up with everyone to get my early morning coffee while we waited for boarding.  I looked up to see a Virgin Airlines staff member talking to Derek.  She was organising to get him on board.  We only had time to drink half the cup of coffee before we were called to board before everyone else.

I pushed Derek and the staff member took my carry on bag.  We were assisted the whole way.  They settled us in our seats and then turned to the rest of the passengers.

The flight itself was very uneventful.  An hour after taking off he took another 10mg HC (his normal morning dose).   About 2.5 hours into the flight Derek decided he needed to go for a walk, to stretch his legs.  The Business Class Steward saw him get up and offered him the use of the business class toilet, rather than walking to the back of the plane.

As the flight was preparing to land they again approached Derek and informed us that we would have to wait until all other passengers were off before we could disembark as they had to clear the way for the wheelchair, which would be waiting at the door to the aircraft.

After everyone was off Derek got up, and almost fell over.  He couldn’t stand up straight.  His hips hurt, his legs hurt, he was extremely tired (even though he had taken more HC on the flight).  Looking at him, I was thankful I had organised the wheelchair.

Once we disembarked, I was even more thankful.  There was a 600mtr walk from the gate to the immigration hall.  There was no way Derek could have walked that far, and then cued with 300 others (a second flight had just landed).

The beauty of the wheelchair didn’t stop there.  I didn’t have to push Derek at all.  A staff member pushed him.  He also took us through the priority system.  He helped us with our passport clearance, helped us through luggage pick up, and most importantly, helped us through declaring Derek’s drugs.

It was a doddle.  We ticked we had things to declare, they asked us what, we said drugs and food, we handed over the letter from Customs allowing us to import DHEA and we were told “Customs have already cleared you, of you go”.

With that the gentleman pushing Derek lead the way out into the arrivals concourse, took us to a seat, unloaded Derek and left us.

As we were going through baggage claim I had noticed a family (one of the first off the aircraft) waiting for their bags.

I left Derek to sit and rest on a soft seat while I went to pick up a micro sim for my old phone (to give me data while on holiday).  It took a good 15 minutes.

“Guess who I saw only just come out of the arrivals gate.” Derek asked as I arrived back.

“I don’t know.   Who?”

“The guy that was sitting next to us.”  He was also one of the first off the aircraft.  I knew we got through quickly, but not that quickly.

Next job was to get the car I had booked and get on our way up to the Sunshine Coast.

So far everything was going exactly to plan.  This was a good thing.

After Derek had had a rest we went and got the car.  It was a short walk, and he needed to stretch his legs.

We found the car, put our bags in the boot and got in the car.  We were going to stay with Des Rolph and Wendy Lau at Des’ Beach House.  Derek put the address in the phone, and set the system up to navigate.

Let the Games Begin

The trip up went well.  We arrived at the house just after 11.30.  Although I had never met Des or Wendy in person, I had skyped with Wendy a number of times, and had chatted on facebook to Des.

We arrived to discover that Des and Michelle Dalton (another facebook friend) had gone to the supermarket, but Wendy and Chris (Des’ husband) were home to greet us.

It was like meeting up with old friends.  We felt at home with them immediately.  And it was no different when Des and Michelle arrived back.

Lots of chat, some food, a little wine, and what to Derek and I was a late night.  Derek kept double dosing his HC all evening, we had a lovely dinner, and then excused ourselves to go to bed.

des-place

Michelle, Des, Wendy, Jo and Derek. Chris is the photographer.

I woke very early the next morning.  My body was on NZ time and Queensland is 3 hours behind.

I lay in bed watching video’s on my tablet while I waited for Derek to wake.  He finally woke up and took his first dose of HC for the day.  He then rolled over and went back to sleep.

As he slowly woke up he made a great announcement.  “I forgot to take my warfarin last night.”

Bugger.  Friday his INR was 2.9, flying decreased INR, he didn’t take Warfarin last night.

This all equals low INR.  We needed to get it checked quickly, but it was Sunday and we were in a foreign country.

We spoke to Des about what we could do.  There was a lunch function at the house with 6 other Adrenal Insufficient patients and we weren’t going to miss this for the world.  It was exciting to finally get to meet others with AI, and to chat to the spouses to compare notes.

Read about “The Domino Effect” here.

the Domino Effect

The Domino Effect

We had breakfast and started preparing some of the food for lunch (I say we, but mostly Michelle).

Once it was open, Des phoned the local 7 day medical centre to see if we could go in there and organise a script for an INR at thelocal Pathlab.

We were told they took walk-in’s so we could turn up at any time.  We decided we were going to leave immediately.  That way we could be back in time for the lunch.

When we checked in at the medical centre I asked if they were able to do plasma INR or if it was just a coagucheck system.

“It’s coagucheck, but that’s fine, its really accurate.”  Came the response.

I was doubtful, as I had heard through the APS forums that, for certain APS patients, it wasn’t that accurate, in fact it was dangerously wrong.

The Dr came out to reception “Derek?”

We got up and followed her into her consulting rooms.

I looked at Derek, who was still extremely tired.  “Derek has Antiphospholipid Syndrome and is on Warfarin.  How much do you know about Antiphospholipid Syndrome?”

“I gives you an increased risk of a blood clot” the Dr said with confidence.  However, she didn’t ask if Derek had ever actually HAD a blood clot.

“It doesn’t just give you an increased risk.  Derek has had clots.  He has actually suffered Catastrophic APS.”

She looked like she didn’t really know what it was, so I began to explain.  Derek chimed in as he could but he was struggling.

We then explained that he had a very high risk of having too low an INR and we needed it checked.

“We can do that here with the coagucheck machine.”

“I don’t believe that is very accurate.  We need a plasma INR.”

“Oh, these machines are very accurate, we have never had a problem with them.”

“They are accurate in atrial fibrillation, and in some patients with certain APS, but Derek has low Platelets as well, so for him it is not accurate.”

“Well let’s check it and see what it is.”

I was beginning to get the “I’m a Dr and I know better than you” feeling.  Not an unfamiliar feeling these days.

We went out the back and had the test.  It came out at 4.3.  this mean a very high risk of bleeding.

“Well that’s not possible.”  I was looking at the result, and then at the Dr.

“INR can be quite unstable when traveling.  It can be caused by heat, by long travel. Lets go back to the office and discuss it.”

Research has shown that Coagucheck INR is not recommended for patients like Derek who has High IGG and low platelets, as they tend to give a higher INR than it actually is.

Derek and I endeavored to explain that with APS flying lowered INR and therefore that result was most likely wrong, but like many Dr’s she was determined she was right.  We also got the feeling, when she mentioned Clexane, she though it would increase INR, where it does not have an impact in INR but does affect coagulation.  It should be used to reduce risk of clots, not reduce INR as was indicated by the Dr.

After some conversation, the Dr was determined we should head straight to the local hospital to have an INR as it was too high and could be dangerous.  She also advised not taking warfarin that night.

I agreed we would take a letter for taking to the Hospital, as long as she also wrote a script for an INR sat the local PathLab on Monday morning.

When we arrived back at the house we told Wendy, Des and Michelle what had happened and that there was no way we were going to the hospital before the lunch.

Wendy said she that her Dr friend was a Heamatologist and she would contact him to see what he thought.  I was still unbelieving of the high INR, and was still concerned that in fact, it would be extremely low.

After a very hectic time over lunch (explained here) Wendy finally got hold of the Dr for us.  I found it strange to be standing on a balcony on the Sunshine Coast, talking to a Chinese Dr in Hong Kong.

He agreed with me that there was no way Derek’s INR was 4.3, and that waiting till Monday would be safe, Derek should just take his Warfarin as normal that night.

Given Derek’s stress levels were very high I wanted nothing to do with Hospitals that day so we would go to the pathlab the next morning before heading south.

The next day we were due to leave.  Everyone in the house was extremely tired, and needed an early night so we went to bed by 8pm (11 NZ time) and slept.

The next morning we woke to pack our bags, get ready, and head to the Path Lab.  It was only 20 minutes down the road.  After we had the blood taken we were heading to Bribie Island to meet up with another friend.  This time someone I met through the Coro St MIRC group I had joined 20 years earlier.

I was excited to be meeting her so was wanting to get the test done.  We had to also organise for them to call us with the results as we would’t be going back to that particular medical centre again.

Medical Incompetence is International

We arrived at the Pathlab and handed over the form for the blood test.  As I handed it over I asked if there was any chance we could have the results phoned directly to us, as we were travelling.

“We will see what we can do.  Is this the correct Medicare Number?”

“What, we are from New Zealand, we don’t have Medicare.”

“So what is this number?”

“I have no idea.”

“Date of birth.  Is it ######”

“No.”

“Phone Number:  Is it ########”

“How did they have a phone number, I have never given them a phone number.  What is the name on that form?”  Derek and I looked at each other.  We couldn’t believe what was happening.

“Dale ####”.

“That’s not Dereks form.”

After a little more discussion we headed out the door to the medical centre to get it sorted.

On the way over we looked at the letter we were supposed to present to the hospital.  It also had the wrong information, but the right medical issues.

Wow, a major mistake.  If the other patient had had to go to hospital, he may have been given clexane because his INR wasn’t at the right level.  It could have killed him.  But worse, if Derek’s INR was wrong, he could also have been incorrectly treated.

We got back to the medical centre to explain what had happened and request a new form.

The Receptionist went to speak to a Dr.  She came back a few minutes later.  “The Dr discovered her mistake yesterday after you left so she faxed the correct details to the hospital.”

That was great, except there were two public hospitals, she had no idea which we would have gone to.

We got the correct Pathlab request and went back for the blood draw.  Derek was treated by a Kiwi phlebotomist.

We then headed to Bribie Island to see Jubbly (Jacqualine Russell).

It was a 1.5 hour drive so we let Jubbly knew we would be late.  We were also meeting Elaine Miles, another person I had known for 20 years without meeting.  I was excited.  Yesterday was Derek’s day, today was mine.

jubblyelainejo

We finally arrived just on 12.00 noon.  As we got out of the car to walk to where we were meeting them Derek asked if we had the chargers, tablets, and cables for our technology.

Bugger, no, and my phone was running out of battery.

I went back to the car to check, sure enough, in our haste to get to the Pathlab, and the stress of the previous day, we had left them all behind.

I would have to think about what we could do.  But that was later.  Right now we were focusing on Jubbly and Elaine.joexercisejoderek

After chatting for a short time we headed to the fish and chip shop to get lunch.  All the way Jubbly kept poking me, just to make sure I was real.  It was like on the Sunshine coast, like meeting a long lost friend.walking

We had a great time.  We talked, and talked, we laughed, we ate fish and chips by the beach, we walked along the boardwalk.jubblyjoselfie

 

 

 

 

 

 

After the stress of the previous day, it was great to relax.  Derek was starting to show his tiredness, but he also looked like he had started to relax a bit.

Then we had to organise getting the stuff we had left behind.

This meant we had to drive the 1.5 hours back north before we drove south.  So far this holiday had not gone exactly to plan.  It was a major learning curve and we were only at day 3.

After saying a gre

at goodbye to Elaine and Jubbly we got in the car and headed back north.

Watch out for the next leg of our “holiday”.

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The Domino Effect

How do you get 7 Adrenal Insufficiency Patients
in the same room at once?

Some may find the video included here upsetting.  I make no apology.  Deb herself wants awareness of this.

One of the main parts of Derek and my trip to Australia was to meet Des Rolph and Wendy Lau.  Wendy had come over for a second time from Hong Kong as she is struggling with her health, and the hospital she attends in Hong Kong, The Princess Margaret Hospital, is very slowly killing her with dangerous treatment, 3rd world conditions, and a serious lack of knowledge. (Read about her experiences here).

des-place

Hey, Hey, the Gangs all here. Michelle, Des and Wendy,  Jo & Derek

As we were going to be staying at Des’ house on the Sunshine Coast, they decided it was a good time to have a get together.  Derek had never met anyone else with Adrenal Insufficiency.  This was the perfect time to fix that.

There were going to be 7 people with Adrenal Insufficiency in the same room at the same time, and it wasn’t for a conference, it was for a get together and a lunch.   It was also to be the inaugural meeting of the Adrenal Insufficiency Australia & New Zealand Association (AIANZA).

It was all very exciting.  It was a lunch meeting.   There were crowns, and gifts for all.

giftsforall

All the lovely gifts provided for the Adrenal Insufficiency Sufferers.

Derek and I had arrived on the Saturday morning, having flown into Brisbane, and driven straight up.

On the Sunday morning we had something to do so after breakfast we went out for a short time. (explained in my second trip post).

When we got home we found all preparations well advanced.  Michelle was cooking and preparing food, Des was doing a lovely job of putting makeup on Wendy, and Derek and I just had to get ready.

I barely had time to start getting ready, and let the girls know the result of our excursion when the next guest arrived.

It was Anne and her husband Keith.  Anne has only been diagnosed for coming up a year.  She has Primary Adrenal Insufficiency, but is well controlled.  She has learned quickly, to up dose, to stress dose and to listen to her body.  However, Anne does not have an emergency injection kit as she is one of the many (too many) patients who have been told “you live close enough to a hospital, and ambulances have solu-cortef, so you don’t need it”.  To date Anne has never needed her kit, but she is about to travel outside Australia on holiday, and one of the aims of the day was to explain to Anne the importance of getting an emergency kit.

Last to arrive was Deb Salmon Brown.  Deb has had Addison’s for 8 years, but it has not been well controlled, in the main, because of poor endocrinologist, and (we now believe) because of poor absorption of her oral hydrocortisone.

Deb has a history of bad treatment by medical professionals which means she puts off going to the Dr, and will treat herself at home.  This is quite common amongst those with Adrenal Insufficiency as it is a little known, and even less understood disease, even amongst endocrinologist (those that are supposed to know all about it).

I am going to use two terms here from now on:

Pre-Crisis: When you can feel your body crashing, you have early signs of crisis which will include fatigue to a point where you have to sit or lay down and maybe nausea, weakness, brain fog, sometimes slurred speech, back pain, leg and hip pain and the biggy for many, HIGH BLOOD PRESSURE.

Crisis: Depending on the person concerned, you will have some, or all of the normal signs and symptoms of a “True Adrenal Crisis”, which will include unbalanced sodium and potassium, an unconscious appearance (although some can still hear you in this state), and the clear signs and symptoms of hypovolemic shock.  This state includes a dangerous drop in BP which can, and does cause other problems such as stroke, heart attack etc.

It is my belief (and other are in agreement) that everyone should be treated as a medical emergency at the Pre-Crisis stage.  This blog post will show you why.

Back to our story.

Debs looked ok to me when she arrived because I didn’t know her.  Des tells me she was feeling dizzy and sweating, but it was a hot day and she had just driven.

She was happy to meet others who “got it”.  She had also driven herself up from her place to Des’ home.  This was something Deb, Des, Michelle, and Ann (our last guest to be introduced) can do easily.  Derek can not drive.

And so it begins

There were 6 AI Patients Sitting in a room
6 AI patients sitting in a room
But if 1 AI patient should accidentally swoon
There are 5 AI patients laying in a room.

The Domino’s are all in a line.

Deb is sitting on the couch, Des is talking to her.  The rest of us are getting things ready for the day. Some of what happens next is how I witnessed it, some is taken from accounts from Des, some is taken directly from video captured.

My recollection of events is, as we are all chatting, Des and Deb went and sat on the couch and I heard Deb say she wasn’t feeling well.  She appeared to be becoming upset.  I heard her say she felt sick.

Des’ recollection:

When she arrived she was already shaky and sweating. Within just a few minutes she told me that she was starting to feel unwell and then she collapsed. I asked her if she wanted to come and lie down on my bed but she couldn’t move and fell into the sofa. It all happened very quickly.

Before I knew what was happening Deb had collapsed onto the couch and was shaking uncontrollably.  Des was calling for an emergency injection and we all kicked into action.

I looked around but didn’t know which bag was Debs.

Wendy was acting as well.  She was grabbing her solu-cortef and saline solution.  Wendy was also shaking.

I took the solu-cortef off Wendy and popped the lid on the vial.  As I did that, Wendy opened the Saline bottle.  I then found a syringe and needle being handed to be.

I put the needle on the end of the syringe and knew I had to draw up the saline solution, but I had two problems.  When practicing with the solu-cortef at home, it was an act o vile so it was all pre-measured in the container and I didn’t have to draw up anything, I simply mixed it and drew all the solution.

This was different.  I asked how much fluid I needed and was told 20mls.  Great, except I didn’t have my glasses on.

I grabbed the nearest pair I could find.  I don’t know who’s they were, but they worked.  I could see clearly where I had to draw the fluid up to.  I then had to insert through the top of the solu-cortef, inject the saline, mix the powder until clear, re draw up the now mixed liquid, change the needle (it was blunt and bent at this point) and then inject a stranger with medication that was going to help her.

I went to the couch and, standing behind it, I injected Deb in the arm.

Deb’s recollection of this part of the day was:

I arrived feeling fatigued and also with “nervous but excited” stress. Hugged everyone, said hello, handed out gifts then sat down with cup of tea.
Derek launched into his medical history which was a bit overwhelming for me and I started to feel generally unwell. Hot & clammy, nauseous, pain in tummy and a bit shaky. Started feeling very light headed.
Des walked past, I grabbed her arm, looked at her and said I’m going to pass out, then I did!! 5 secs max.

 

We then put a O2 monitor on her hand, found a BP cuff.  We couldn’t get an oxygen level from Debs finger so I made a comment that they looked so nice they were possibly fake, and therefore blocking the signal (they looked beautiful, well manicured and painted).

We tried her toe with that monitor, then Derek suggested we try his.  This one worked.  Her O2 was fine, we then took her BP and it was quite high, around 176/102.

Slowly, after what seemed like an age, Deb began to come around.  I looked around the room and Wendy, Des and Michelle were all shaking but they were also busy doing what needed to be done, taking care of their own and each others needs, while also taking care of Deb.   Derek was now just sitting watching.  I can’t remember, but I think Ann was helping out.

I turned to Wendy and asked her to hold her hands out in front of her.  They were shaking badly.  Des told her to do a subcutaneous injection.  Des then did her own bolus due to the stress in the room.

I told Derek to up dose.  I looked at Michelle and handed her the tablets.

Everyone was stress dosing.  Everyone could feel the tension and stress in the room.

Once Debs had come round she told us where she had an emergency letter, and where her emergency injection kit was. We began to relax a little, and settle Debs down when it began again.  She also informed me that she heard my comment about her lovely nails, and they were real.  This confirmed that, like most, Debs hearing is the last thing to go when collapsing.

“She’s going again”.  I heard the call from someone.  I turned to see Deb collapsed on the couch again.

Wendy said Deb had wanted one of these attacks filmed, so Derek, while sitting in his chair, got his phone out and began to record the video.  Debs as asked that this be made public to show what an adrenal pre-crisis can look like as her’s are not text-book.

Knowing that Deb was aware during the first attack, I asked her if she was capable of indicating to me if she wanted another injection.  She managed to grunt confirmation.

Des found her phone headed off to call an ambulance.

As the second episode began, Wendy asked Derek, was feeling calm, could he film the collapse this time.  He was tired from doing things during the first attack to support me and what I needed.

Deb had wanted these episodes filmed because she was not believed by the medical profession and she was desperate for answers.

She has since given permission for these videos to be made public to try to help people.

I moved away to draw up a second injection.  This time it was a quick process as we had Deb’s acto vile.  While I was drawing up the injection Ann moved over to the couch to be with Deb.

Everyone was again doing what needed to be done.  There was no panic in the room, just calm decisions as to who was going to do what.

Des tried to call Simon (Deb’s husband) and Chris (Des’ husband) who  were playing golf together while Michelle stayed on the phone with the ambulance service.

Anne and I stayed with Deb then once the violent shaking stopped and Debs started coming round again I went outside to wait for the ambulance.

It took 15 minutes for the ambulance to arrive and as I was standing in the street I could hear from the house that Deb was again going into a violent shaking episode.  This time they decided not to give her an injection as 200 should have been enough.

Deb was clearly distressed by now because she knew that the treatment she would get from the ambulance service and the hospital was going to be substandard.

Her recollection of this part of the event:

I was aware what was happening most of the time, aware I was going down hill fast but really couldn’t respond. Required enormous effort to grunt to you I needed more HC. I knew I did and was terrified I wouldn’t get it.
When I continued to pass out and seize when ambos were there I knew I would need a calmative to stop them and just quiet my system down because it was so hyper sensitive. So was glad when Simon arrived and filled them in.
I have now written on my Ambo Directive that Midazolam needs to be given after HC if seizures don’t settle.
The overwhelming emotion the whole time is FEAR. Fear that I won’t get enough HC, fear that ambos won’t know what to do, fear of going to hospital, fear of how I’m going to be treated.
Afterwards, it leaves me TOTALLY exhausted!! All that physical energy chewing up cortisol leaves me feeling like I’ve run a marathon or two!!

Debs had more attacks in the 15 minutes it took for the ambulance to arrive.

By the time the ambulance had arrived Deb was having another attack, there was an air of “something” in the room.  The ambulance was carrying 2 paramedics.  Just as they were getting out of the ambulance a car pulled up.  Another Paramedic (an advanced paramedic) arrived with another person.

I looked at the 4th person.  He had a Life Flight uniform on, and the word Doctor on his back but he was just riding along, and not on duty.

We all stood back as Des explained Deb’s history, and what had happened.  You could hear the fatigue in her voice.   Wendy was also starting to show signs of heading down herself.

Michelle sat in a corner on a chair.  She was clearly suffering.  Des, Wendy and Michelle were all shaking.  Derek was standing slightly back with Ann and Keith.

As I reached for Michelle’s HC bottle, which she had asked for, I looked at him.  “Are you OK?”  “I have a headache” he was looking at me, but he was talking slightly slower than normal.  You could see he was heading down hill.   I told him to take 20mg of HC and sit down.  I know Des and Wendy had been up-dosing and I had already given Michelle some of her HC.

Once I got Derek to take his 20 I looked at Michelle.  “Do you want some more as well?”

“Yes please”.  She was talking clearly but a little softer, slower.  She knew what she needed.   I handed her the tablet bottle and went to get another drink.

By this time Des had also noticed that Michelle had started going down hill and had given her an electrolyte drink to go with the first high dose of HC tablets.  Michelle sat there shaking so much she could hardly drink.  Des  started getting a subcutaneous injection ready.  As I turned to Michelle I heard the call.  “Michelle’s gone”.

In less than two minutes she had gone from talking, feeling unwell and needing tablets to needing something stronger.   She couldn’t speak, couldn’t answer questions, couldn’t move. Her eyes were closed.  She was in Pre-Crisis.  If we didn’t act fast she would be in a full blown crisis, otherwise presenting as hypovolemic shock.

I moved back to Michelle and called to the paramedics “We have another one down.”

“Just do what you would normally do.” Came the response from the Advanced Paramedic.

“We normally inject then call an ambulance” I said as I watched Des reach for Michelle’s emergency kit.

I looked up to see if Des was capable of giving Michelle her injection.  Des began to open the box the act o vile comes in.  Des’ hands were shaking so much she couldn’t open the box.  I moved towards her and reached for the solu-cortef and I took it off her.  She then began to open the needle and syringe packages.  Again, too much shaking.

I took everything off her, opened the solu-cortef, drew up the injection and went to Michelle.  She was now completely out of it.

As all this was happening I heard the Advanced Paramedic calling for a second ambulance. One of the two paramedics working on Deb then turned his attention to Des.  He did her blood pressure, which was very high, even for her.   He then put leads on Michelle to check her other readings, including a heart trace.

Simon had arrived and this point.  The paramedics had got Deb stable enough to take her to hospital.  Simon was going to go with Deb because Des needed to go with Michelle.

Neither of these crisis went according to the book.  They were both suffering from HIGH BP, neither vomited until AFTER the injection.  Deb was wheeled to the ambulance in a chair.

I looked around the room.  Everyone had been updosing for the lunch, and then taking a lot extra now.

Michelle had gone down hill to full pre-crisis, and was about to be ambulanced to hospital.  Derek laid himself down on the lounger that was off to the side to try to get rid of the really bad headache he had suddenly developed.  Des had been upping her dose through her adrenal pump, and injected subcutaneously.  Wendy was subcutaneously taking extra steroid (she was already on a high dose through her pump) and Ann was preparing lunch but had also taken extra steroids.  The Domino’s were very procariously balanced and more could tumble at any minute.

I turned to the senior paramedic and said “Next meeting, we will book a bus so they can all go at once.”

His response was “We don’t normally do a bus, but let us know in advance and we will see what we can do.”  followed by a laugh.  He knew this was a rare event, but one that could easily happen again.

We still did not have the seventh Adrenal Insufficient patient in the room.

As the first team was preparing to leave the second ambulance crew arrived.  3 paramedics came in.  They had a quick hand off from the first team and the first team, along with Deb and Simon, left for the hospital.

They moved Michelle to the couch and suddenly she became ill, saying she needed to vomit, which she promptly did.

michelle

Michelle being stabalised before being taken off in the ambulance.

At this point everyone was on very high doses of steroid, and struggling.  I looked around, wondering who was going to be next.

Des was going to the hospital with Michelle so I went outside with her and the paramedics, to get Michelle in the ambulance.

As we were talking to the senior paramedic I asked which hospital Michelle would go to.

“I was just wondering that.  I was trying to decide if we should divide and conquer, or keep them together for support.”

“Keep them together so we can keep an eye on Deb.” I responded.  “They all need support.”

“That’s what I was thinking” he said.  He then got on the phone to see which hospital Deb was being taken to.

“Your friend is going to Nambour Hospital.  Noosa refused to take her.  But we have to take Michelle to Noosa.  The Dr at Noosa is great, they will take care of her.”

Divide and conquer, or high your mistakes.  I saw the ambulance off, with Des and Michelle and went back inside.

Wendy was laying down, Derek was laying down, Anne was in the kitchen preparing lunch.

Derek had just taken his BP.   It was the highest I had ever seen it at 170/106.  He didn’t have the shaking the others had, but I put that down to the fact he hadn’t had an adrenaline rush first as his medulla is also destroyed.  That didn’t stop his body going into a stress response, it simply meant it happened quietly, hidden, and slowly.  He had time to take oral HC to reduce the effects.

Each of the 6 Addisonian’s were suffering in their own way.

Then the 7th arrived.  Renee, her fiancée and two of their children arrived just after the ambulances had left.  I invited them in to Des’ home and made sure they all knew each other.  We were now down to 4 Addisonian’s in the room.

Wendy was in touch with the two patients taken off to hospital so over the next couple of hours we had reports come in from both Deb and Michelle about their treatment.

The treatment received were exact opposites of each other.

Deb’s Experience at
Nambour General Hospital
Michelle’s Experience at
Noosa Hospital
Debbie arrived in ED at Nambour
She was attended by a nurse.
They checked her history.
She waited for several hours.
She was discharged.
No bloods.
No exam.
No extra fluids.
NOTHING!
Discharge within 3 hours.
Handed over by Paramedics with the information “You have two very experienced patients here, listen to them.”
Dr asked what they needed.
Extra Solu-cortef.
Another bag of fluids
CBC’s, electrolytes and several other bloods.
Full examination.
A cute Dr (always an added bonus).
Observation for several hours to ensure she was stable before finally being released.

Anne produced lunch just after Renee and her family arrived so they stayed for lunch as we had a “debrief” of the mornings events.  The were not able to stay long as the had somewhere else to be.  Renee and family were leaving just as Deb and Simon arrived back from the Hospital.

Things of Note from the Day of the Inaugural Crisis Meeting

  1. Once Deb went down, the stress in the house went up.
  2. ALL the Adrenal Insufficiency patients in the house suffered HIGH BP.
  3. Everyone had up-dosed/stress dosed before the event, but because of the stress of witnessing a pre-crisis, it was not enough.
  4. Everyone stress dosed trying to prevent a pre-crisis.
  5. Treatment received by those in pre-crisis was dependant on several things.
    1. Previous history with the hospital you go to.
    2. Attitude of the Dr’s treating you.
    3. Knowledge of your condition.
    4. Having a strong, knowledgable advocate.
  6. You can go down quickly so everyone needs an emergency injection kit.

How did this end:

Firstly, we never did get 7 Adrenal Insufficiency patients in the same room, at the same time.

A week after the meeting, having video of the crisis Deb had, Des contacted her General Practitioner and organised an appointment for Deb.  Deb is currently in hospital receiving the treatment she has been desperate to get for the last 8 years.

Michelle is off having a weekend with her cake decorating ladies.

Des is very tired, but keeping busy helping others while managing her own conditions.  (Something she has only been able to do since getting an adrenal pump).

Wendy is visiting Deb in hospital, and trying to relax while waiting to return again to the substandard care she receives in Hong Kong (while hoping to be able to move to a new hospital).

Derek and I are home now, and Derek is showing signs of the trip being a little too much for him.  He is still up-dosing.

Ann was tired for a day or two, but has come back well.  We are still waiting to hear if she has managed to get an emergency injection kit.

Why did we not hesitate to inject?

Some may think we were too quick to inject 100mg (200mg in Deb’s case) of hydrocortisone.  That Deb was not in a full crisis (vomiting uncontrollably, diarreha, critically low blood pressure, or a coma.)

Here is a very brief description of what can happen if you don’t or can’t inject immediately you realise you are in Pre-Crisis.

Brenda Berry:  My daughter, Katie was diagnosed at 19, 6 years ago after many trips to the ER dismissed her symptoms. After all, she looked healthy and “tan” all while vomiting. It took a violent adrenal failure and near death to FINALLY be diagnosed. The first four years and 7 endocrinologists resulted in 45+ hospital stays due to crises. Every endocrinologist had her on too low of dosing, wrong timing and all dismissed the need for an emergency injection kit. She was told not to be dramatic, she didn’t live in a 3rd world country and lived near ERs. She finally met an RN in an ER one night who also was an Addison’s patient. She told Katie about her dosing to mimic the circadian rhythm and dosing up to bedtime, with hydrocortisone, florinef and prednisone being the bedtime dose. No mention of an injection kit. It really wasn’t on her mind as it had been dismissed so often. Katie tried this and it seemed to be a life changer.
Her periods were her only real crisis triggers that occasionally still sent her to the ER. She was in the process of meeting with specialists to try to stop her periods. Sadly, before that could happen on 1/4/2016 her period came late in the evening and triggered a crisis with vomiting. She tried to manage on her own with hydration and anti nausea meds.
It’s thought that she must have had several strokes rendering her unable to ask for help and by 6:39 am we heard a crash in her room. She’d collapsed in her bathroom from cardiac arrest. She died. 35 minutes later she was revived in a trauma ER. After a month in a coma and a month in PVS she is now in her 10th month in a recovery sub acute center with permanent anoxic brain damage. She can not speak, still has a trach, is incontinent, has some comprehension but with child like judgement for her own safety. She has limited use of her hands, can barely write a few misspelled words. She was brilliant, studying to become a Neuro Ultrasound Specialist. She is 25. How will we, her aging parents care for her?
If she’d had the emergency injection and we were trained how to administer it she may have saved her own life let alone the EMTs could have tried as well. It’s not even carried on board ambulances yet they have overdose reversals, bee sting, peanut allergy and a multitude of treatments. Her life will now forever be painfully difficult and only a mockery of what really living should be.

If you think someone, or yourself, is heading into an Adrenal Crisis, even if your BP is HIGH, inject.  When someone has severe pain, or a major asthma attack, 400mg is the minimum dose given.  It will not cause harm if it is needed.  It will cause euphoria if not needed.

Steroids are not evil if needed, they save lives.

If you go to hospital in Australia or New Zealand, you are triaged.  A True Adrenal Crisis should be a  Cat 1, a pre-crisis should always be a Cat 2 (but it is not currently recognised).

Most who are still conscious when they arrive at hospital are triaged at a minimum level of 3.  We have now witnessed how quickly you can go from feeling unwell and thinking you need help, to being in a life threatening state.  Left more than 10 minutes waiting and they could well be doing CPR in the waiting room.

The Australasian Triage Scale
Triage Category Description Maximum Clinically Appropriate Triage Time
1 Immediately life-threatening, Immediate simultaneous triage and treatment
2 Imminently life-threatening, or important time-critical 10 minutes
3 Potentially life-threatening, potential adverse outcomes from delay > 30 min, or severe discomfort or distress 30 minutes
4 Potentially serious, or potential adverse outcomes from delay > 60 min, or significant complexity or severity, or discomfort or distress 60 minutes
5 Less urgent, or dealing with administrative issues only 120 minutes

 

A Presumptive Diagnosis

Aside

The marrying of Evidence-based medicine and Experience-based medicine.

Have you ever sat in a Dr’s surgery, told the Dr everything about your signs and symptoms, and then you hear those fated word:

“WELL IN MY EXPERIENCE”

Many years ago, we trusted Dr’s to use their experience to know what was wrong with us.  These days, we can now have blood tests, scans, x-rays and more, to back up that experience.

Some Dr’s are single (they only use Evidence, or they only use Experience.  Some Dr’s have successfully married the two.  Others are in a relationship, but is very shaky and they have to work hard to keep the relationship on an even keel.

If your Dr is a “in my experience” type Dr you need to remind them that actually Doc, it’s not just about your experience, it’s also about your skills to interpret results of blood tests, scans, images, or any other investigation, to build a full picture.  Its about your ability to keep an open mind, your realisation that not every blocked nose is a cold, that every high BP is from poor diet and lack of exercise.

You might have had Jane Doe in here with a blocked nose that was hay fever last week.  But I don’t get hay fever, my nose is not normally blocked like this, and it is giving me a major headache.

If ever a Dr says to you “Well in my experience” and they have never met you before, then there experience could mean diddlysquat, if it not balanced with correct interpretation from clinical investigations, blood tests, x-rays, scans, or what ever else needs to be done before a confirmed diagnosis is made..

Dr’s must learn to park their experience at the door when they first come into the room to see a new patient because their experience, however vast, can mean a missed diagnosis, a wrong presumption, the brushing aside of what appears a “minor abnormality” which can be a major cause, or the vital clue to the true condition.

 Let me give you an example.

As those who have read my blog know, my husband Derek has Antiphospholipid Syndrome.  It means his blood is more susceptible to clotting.  To prevent this happening he takes Warfarin.

In May 2012 the Dr’s stopped his warfarin for 5 days without any anticoagulation cover.  We, at this time, trusted Dr’s to know what they were doing.

The day of the biopsy he felt “unwell”, the day after his biopsy he was seriously sick.

Enter the Presumptive Dr.  Experience based diagnosis and treatment began.  In the experience of all the Drs we dealt with, on balance of presumption, he had Sepsis.

Immediately treating that sepsis was the right option.  However, they still had to make sure it was sepsis, not just use their experience and accept it.

After giving the high dose anitbiotics, what they did was ignore the facts as presented.  When we spoke to each Dr we told them:

  • Derek had not had warfarin for 5 days (and was still not taking it)
  • Derek had a blood clotting disorder which, without warfarin, meant he had a high risk of clotting
  • He felt unwell at the time of the biopsy

The Evidence Based Medicine did not support a diagnosis of sepsis, especially when you add the signs and symptoms together.   The clinical evidence showed no sign of infection based on a number of blood tests.  The blood tests only showed his body was fighting inflammation which is a classic sigh of an APS flair, or CAPS.  They could not identify any bacteria or any of the many signs, only an indication of inflammation.

Yet because sepsis was the obvious diagnosis based on experience, no other investigation even was done when the evidence didn’t support it.  It was put down to just one of those things.

In Sept 2012 the same thing happened.  Between 2 and 16 Oct we visited many Drs, telling them all the same story.  He had stopped warfarin for 5 days, then had prostate surgery, and  had been unwell the entire time.

All that was heard was Warfarin and prostate surgery.  Clearly he had some form of infection, firstly a UTI, then a reaction to antibiotics, then a throat infection, another reaction to medication, a kidney infection……. Infection, infection, infection.  Never did he have a blood test. All the diagnoses were experience based presumptions, without evidence.  2 tests were done during the week, one throat swab and one swab of a rash.  Both these came back clear.  No one listened to the real symptoms and none of the Dr’s put all the symptoms together.  Just because there was symptoms 1 yesterday, didn’t mean it should be ignore when symptom 2 happened.  In the 2 weeks from 2 to 16 October, Derek only had 1 blood test.  Nobody queried his history of cessation of warfarin and the history of blood clots.  We told of a history of his INR going high above range, then a bleeding event in the previous 2 weeks.  All were ignored because those we spoke to had no experience with those, so they couldn’t comment.

This is where experience based medication falls down.  To use experience, the Dr must first have experience.  Because it was rare, they did not.

On 16 Oct Derek was finally admitted to hospital  He had had blood tests on the Friday which when finally looked at 4 days later, and were all way out of range.

He was rushed to hospital via ambulance, but still his own Dr, knowing his history, told the Ambulance officers that it was suspected sepsis.

On the morning of 17 Oct he had a CT.

This CT showed a small pocket of pneumonia and an accumulation of fluid around the lungs.

What was also mentioned on the CT report was what “has the appearance of a clot, and the left adrenal gland appears enlarged”.

These two findings were ignored as insignificant compared to the fluid around the lungs, and the presumption that he again had Sepsis.

For 6 days they ignored the history of APS, cessation of warfarin, and the findings on the CT and instead treated him (unsuccessfully) for sepsis.  No matter the treatment, his body continued shutting down.

It wasn’t until a junior Dr visited Derek.  Having left all presumptions at the door, she started from scratch.  She asked questions, studied his body, all the time taking note.  She then went and read the reports.  Including the report from 17 Oct.  It was only then, because a Dr threw all presumptions of the previous 2 weeks, out the window, that the true catastrophe revealed itself.

I am not saying that immediate treatment for sepsis wasn’t a good idea, but when they didn’t find any evidence of it, then they should have kept looking.

Yes, treat the easiest condition first, but consider other options, including, if all else proves negative, that zebra hiding in the dark corner.

“In my experience”

If the Dr says this to you and you have never met him/her before, point out that any experience they had before facing you, although useful, needs to be successfully married to evidence, and they need to have experience of using that evidence.

What you are looking for from them is their training and knowledge in interpreting investigations, with an open mind to the possibility that they have never experienced YOUR particular circumstances before.

Experience is great once they know what is wrong, but making a presumption based more on their experience than on fact, can leave many people remaining undiagnosed, misdiagnosed, untreated or feeling sicker than before.  When it happens time and time again, some patients just feel like giving up.

It can be hard to be taken seriously when you have to go from Dr to Dr because you KNOW there is something wrong, and all you get is Presumptive diagnoses, the treatment for which, is clearly not working.

It is hard to advocate for yourself, but if you are able to, when the Dr seems to be diagnosing you based on HIS/HER experience, not yours, ask them what they are basing their diagnosis on.  Get them to explain how they came to that decision.  Ask what evidence they have.

It can be hard, but if asked in the right way, and if necessary asking the same question in several different ways, it can make the Dr rethink their own ideas.  Sometimes however, the Experience and the Evidence are divorced. They will never live happily together with the Dr, and at this time, you need to find another Dr.

Low Cortisol or an Adrenal Crisis – Learning the difference

(Low Cortisol or an Adrenal Crisis – PDF of this blog.)

CHECK OUT THE NEW PERSPECTIVE ON THIS POST HERE

Many with Addison’s Disease, especially in the early years after diagnosis, don’t have a clear understanding of what an Adrenal Crisis is­.

Some are very sick and believe it can be treated at home, others are low on cortisol and believe they urgently need Emergency Department treatments.  It can be very hard to tell the difference at times.

Then we have the Dr’s view, based on books, not on experience, which is, don’t know so won’t treat.

THE FACTS:

  1. Low Cortisol WILL lead to an Adrenal Crisis, if not dealt with;
  2. Adrenal Crisis DOES need Emergency Medical Treatment;
  3. Adrenal Crisis WILL lead to Death (or worse) if not treated urgently and correctly;
  4. Dr’s DO cause Adrenal Crisis’ because they don’t know, or don’t listen to their patients.

I am guessing the first thing you are asking is “What is worse than Death?”

Quite simply it’s Permanent Brain Damage, Permanent Heart Damage, Permanent disability.  Severe damage to other organs, and in many cases if you survive, PTSD.

An Adrenal Crisis can come on rapidly, or it can come on because you haven’t controlled the symptoms of low cortisol.  It may be because you have an infection and don’t know. There are many reasons for cortisol being used up quickly in your body.  Some of them obvious, some not so.

If you suffer a Genuine Adrenal Crisis and don’t get appropriate treatment fast, the ultimate outcome will be death.

Those with Adrenal Insufficiency and just as importantly, those who live with someone with Adrenal Insufficiency (Addison’s Disease) need to learn the difference in symptoms. And it isn’t something you can learn from a book because we are not all modelled on a book.  We are all human, with different functioning ability in our body.

It is also hard because there is a point when symptoms of low cortisol and an impending adrenal crisis meet.

And it is these symptoms that cause confusion.  If you can treat them by taking extra cortisol, then it is low cortisol.  If you can’t resolve the symptoms with oral medication, then it is the beginnings of a crisis.

Low Cortisol or Adrenal Crisis

From Low Cortisol to Adrenal Crisis

Click the image to see how the symptoms can present, and how they meet as you move from low cortisol to crisis. Most of these symptoms are not “in the book” as an adrenal crisis, and are not considered by many Dr’s as important enough to treat by bumping (updosing).

We all hear that if you have a major trauma, or vomit several times, or have a high temperature, inject.  If you have to inject, go to hospital.

But what about those symptoms in Blue above?  Should you be taking extra HC if you feel all or any of these symptoms.  Some of them occur every day for some people.   Other’s never experience them.

It’s always a guessing game.  Do you Up Dose, Stress Dose, or Inject and race to hospital.  What are these options?

Updosing:

To Updose is what Derek does when we are going out.  He will also do it if he is doing something more than normal around the house.  Perhaps he is going to help me do a little gardening or we are going to a Show, or out to Dinner.  Or if he begins to feel a certain sort of tired.  And here is the other problem.  How do we know if he is just tired because he has done more than normal, or tired because he is low on cortisol.

There is no hard and fast rule.  He had to learn to listen to his body.  We know that when he is tired, he gets sore hips or starts yawning in a certain way.  These are his first symptoms of low cortisol but it doesn’t mean he has to up dose.  It may just mean he needs to sit and rest.

The biggest clue for Derek is if he can’t keep his eyes open, he feels weak, or he gets a mild gas he can’t shift.  When this happens he will take an extra 2.5-5 mg of Hydrocortisone.  He will then lay down to rest and the symptoms will normally resolve themselves.

If he suffers more painful gas under the ribs, has gas that doesn’t ease on burping, has a headache and feels “blah”, he will “Stress dose”.  He will take 10-20 mg Hydrocortisone in one hit.  He will also then try to get to a Dr within 24 hours to find out why he is that low.

I has taken almost 4 years to learn the difference between all his symptoms, and how to respond to each.

When new to Adrenal Insufficiency we asked each of Derek’s treating Dr’s, including his Endocrinologist, if the symptoms he had were AI related.    The immediate response was no because it isn’t in the “book” as a symptom.

There is a belief within the medical community that If it’s not “in the book”, then it can’t be a symptom! In fact, you will not find any mention in medical books, about the concept of Updosing for minor stressors, or needing to take an extra 5 mg if you are going out somewhere.  Yet those that do updose, have less need to Stress Dose or inject.  That’s not to say the don’t do the latter two, they just appear to manage their illness a little better.

A good example of the need to updose to prevent the need to stress dose occurred recently.  We went to a quiz evening at a pub.  It takes 2 hours, and technically is a lot of stress free fun.  Derek has been twice now.  The first time he took 5 mg HC (2.5 mg more than normal) before going.  The second night he didn’t.

By the end of the second night you could see he was suffering from low cortisol.  He found it hard to move, was very stiff and sore in the hips, he was burping more than he should, his speech was quiet, and not “normal”, he was finding it hard to think clearly and he just wanted to go to sleep.  By the time we got home it was all he could do to get into bed.

According to the books, and many Dr’s, there is no need to take extra for these types of activities.

The next morning he woke and was very sluggish.  He found getting out of bed very hard and was not sure he would be able to work, but knew he had to.  He had to push himself all day.

The result of not taking an extra 2.5 mg for the non stressful quiz, was several days of being sluggish, with no energy and feeling ill . That 2.5 mg, although small in relative terms of his daily dose, would have been enough to make it a fun night, and be able to function the next day.  Not taking it meant he had to take things very easy work wise on Friday, and do nothing Saturday.  If it had been earlier in the week, but the end of the week he would have been stress dosing rather than updosing.

Stress Dosing

Stress dosing is something that IS in the books.  But it is not normally recommended until after you need it.  The problem with that is, sometimes it is too late.

When Derek gets really bad gas that won’t shift, we know it is a good sign that something is wrong.  After listening to the Dr’s, and ignoring it, and Derek ending up in Crisis in Hospital, we now take note of it.

This is when he will Stress Dose.  He will take 10-20 mg in one hit.  We will also either get an urgent appointment with his Dr, or I will take him to an after hours medical center, to get him checked over.  He normally has an infection.  He then continues with stress dosing for several days while most likely on antibiotics.

This gas, which the Dr’s have told him is NOT a symptom of an impending crisis, was the ONLY symptom he had before his last crisis.  It’s not in the books, therefore it isn’t real, but they can’t explain why ignoring it leads to a crisis.

When Derek went into Crisis in 2014 his Endocrinologist said he didn’t understand why the crisis happened.  Simply, we followed his advice and did as he said.  We don’t make that mistake now.

Once you get to this point, your meds may stop working because your stomach becomes upset, and you stop absorbing your them.  You are now on the steep slippery slope to full blown crisis.

Adrenal Crisis

If you have a full blown adrenal crisis, INJECT.  Don’t wait until you fit the medical definition of dangerously low blood pressure, unconsciousness, or constant vomiting.
Derek vomited for 1 day when he had his worst crisis, but he was in crisis for a week before they worked out what was wrong.

The Medical books that describe Adrenal Crisis also describe Hypovolmeic shock.

Hypovolemic shock
Hypovolemic shock is an emergency condition in which severe blood or fluid loss makes the heart unable to pump enough blood to the body. This type of shock can cause many organs to stop working.Causes
The amount of circulating blood in your body may drop when you lose too many other body fluids. This can be due to:
• Burns
• Diarrhea
• Excessive perspiration
VomitingSymptoms
Symptoms may include:
• Anxiety or agitation
• Cool, clammy skin
• Confusion
• 
Decreased or no urine output
• General weakness

• Pale skin color (pallor)
Rapid breathing
Sweating, moist skin
• UnconsciousnessThe greater and more rapid the blood loss, the more severe the symptoms of shock.
• Low blood pressure
• Low body temperature
• Rapid pulse, often weak and thread
Acute adrenal crisis
Acute adrenal crisis is a life-threatening condition that occurs when there is not enough cortisol. This is a hormone produced by the adrenal glands.Causes of adrenal crisis include:
• 
Dehydration
• Diarrhea
• 
Infection and other physical stress
• Injury to the adrenal or pituitary gland
• Stopping treatment too suddenly with glucocorticoid medicines
• Surgery
• Trauma (eg Burns)Symptoms
Symptoms and signs of adrenal crisis can include any of the following:
• Abdominal pain or flank pain
• Confusion, loss of consciousness, or coma
• Dehydration
• Dizziness or light-headedness
• Fatigue, severe weakness
• Headache
• High fever
• Loss of appetite
• Low blood pressure
• Nausea, vomiting
• Rapid heart rate
• Rapid respiratory rate
• Slow, sluggish movement
• Unusual and excessive sweating on face or palms

Dr’s treat hypovolemic shock as an incredibly serious recognised condition that must by given the highest priority.  Yet Adrenal Crisis, which can have the same symptoms, and same outcome of Death, is frequently ignored.

Many Dr’s, especially in Emergency Departments, have read a book once, a long time ago, about Adrenal Insufficiency.  The problem is, that book takes a long time to become medical education material.  The studies must be done, published, then peer reviewed. It then has to be used when writing “the book” (which can take years), then the book must be published (which can take another couple of years).  Once that has happened, it must be considered, and then accepted, as a good teaching tool.

By the time this has happened, there has already been better research, which is being peer reviewed, and published.  That research then has to wait years for the next “book” to be produced or updated.

The information is already old before it goes in the Book.  And Emergency Department Dr’s don’t read all the latest research when it comes out, if they did, they wouldn’t have time to work, so they skip over the specialist stuff that they were told in medical school they would never encounter.

Because many Dr’s don’t have the latest information, as the patient, you must have it. Join support groups so you can learn what is reliable, and what is old.  Good support groups keep abreast of all new information and medical knowledge.  Despite what Dr’s try and tell you, Dr Google can be your friend if used properly.

I have been in an emergency department and seen Dr’s use Google to find out about Derek’s conditions.  Yet some of these same Dr’s repost around the Internet, that great sign below that we have all seen.

If you have the unfortunate pleasure of attending a hospital because you are in an Adrenal Crisis you may be lucky enough to be treated by a Dr that has heard of Adrenal Insufficiency.  Unfortunately many do not get that lucky.  They suffer at the hands of Drs who do not understand a crisis.  Yet they understand shock.  For this reason, you must know the difference between the three dosing styles.

UPDOSE – STRESS DOSE – INJECT

Learn your body’s signs and symptoms, and which option is needed.  And don’t be afraid to use it.

REMEMBER:
Too much one day won’t kill you.
Too little in one day will.

If you’ve updosed, tried drinking something to increase your salt/electrolytes and you still have symptoms, then Stress Dose.  If you take 10-20 mg and after an hour you still have the symptoms (especially the overlapping symptoms) then it is probably time to inject seek medical help.  If you vomit twice and can’t keep medication down, inject and seek help urgently.

IT IS BETTER TO PREVENT A CRISIS,
THAN TREAT A CRISIS.

Dr Google has good research. Use it

Does your Dr have this somewhere?

Sometimes "Google" and the internet have better medical knowledge than your Dr. Don't be afraid to use it, just make sure you use legitimate medical sites.

Show him this one instead!

For Want of an Emergency Injection!

Some people wonder why I keep going on about Adrenal Insufficiency, and key trying to raise awareness. The reason is, knowledge can save lives.

Recently I wrote a booklet called “Did you really just say that”. It is a compilation of quotes from Medical professionals to Addison’s patients and was written because of a discussion in a closed forum about what Dr’s had said to various patients about Adrenal Insufficiency.

The reality of what has been said by medical professionals (some who should know a lot more about the condition) and why we all have a problem with it, has been hit home to us all in a way none of us thought possible.

On 4th of January a beautiful young lady called Katie (24) had a common virus. This was something any normal person would shake off. She had started to feel unwell so went to bed. At some point during the night she was found by her parent unconscious and not breathing after they heard her fall..

They called an ambulance. They could not give her an emergency injection at home as they didn’t have one. The Ambulance service could not give her an emergency injection, they didn’t carry one.

Her Dr had previously said:

“It’s not a big deal, you should just take your hydrocortisone and you should be fine, don’t be over dramatic with the injection, you live near ER’s it’s not that necessary.”

Derek was also told after diagnosis that in New Zealand we were never that far from a hospital, so would never need an emergency injection. We ignored the Endocrinologist that said that and always make sure he has his on him. And this proves us right to do so.

Those with adrenal insufficiency are always told “don’t take extra hydrocortisone unless you have a temperature, are vomiting or are injured.”

With Adrenal Insufficiency,
when you go down,
you can go down fast!
Really fast!!

Kate was a college junior. After struggling for a while, in 2015 she began to improve and was able to return to college and start “living” again.

Then one day at the beginning of this year her mother announced on her facebook page”

medical

“On Sunday Katie became just slightly nauseous, a possible stomach bug. She said she was managing, no vomiting yet. Sometime through the night she must have become very ill. With Addison’s Disease the electrolytes can plummet dangerously low very suddenly At 6:30am Dave and I heard a loud crash. She had collapsed in her bathroom. It took a minute, two? to get into her room as her door was locked. She was not breathing. Dave began CPR, EMT’s were summoned and arrived in 2 minutes. Thank God we live close to the station. Thank God we were home. She was rushed to the trauma unit, unresponsive, but stabilized medically. We were told she had suffered cardiac arrest and later informed of multiple strokes. She was moved to the critical care unit and placed on hypothermic cooling to save brain function.  Monday, Tuesday and Wednesday she remained on full life support. She is fighting. Yesterday she responded to me warming her feet and nodded her head when I asked if she wanted a warmed blanket. She was on so many means of support that her room had to be specially cooled due to the heat of the machines.

…… 36 days on:   “[we both felt] bullied by this pompous, arrogant man who has no right to be a Dr. So much for taking a life threatening disease seriously. And so this continued from one [hospital] Endo to the next. Not one ever treated this disease with the knowledge or respect or seriousness that it deserves. Katie was so under and over medicated that she was hospitalized more than 45 times the first 4 years after diagnosis.

For the past year she had it more under control without using an Endo, her GP was managing her better than anyone before.

So as I sat in our friend’s home yesterday, the three of us crying as [my daughter] lies in a subacute care home, we wondered; would she be our beautiful vivacious Katie right now had she not been undereducated and intimidated by her Dr’s?
For now I will have to speak up for [my daughter] and all of the Addison’s patients because she can’t speak and likely never will. Her time spent in a PVS condition has been painful, muscles contracting, infections, and unable to communicate or even swallow. We are in a cloudy state of day to day confusion with no real prediction of her future. We are for now, just day to day.”

 

For those that don’t know, a PVC condition is a Persistent Vegetative State. A coma like state that she is not likely to come out of. She suffered a heart attack and multiple strokes.

I was going to post this originally on Rare Disease Awareness Day which was 29 February. But that day we were notified of a 13 yr old boy who had Addison’s, who had also got a virus. He went into crisis before his parents realised what was happening. He too suffered irreparable brain and heart damage. He passed away 5 later.

Addison’s Disease Kills! And you never know when you may get a virus that will kill you, or how fast it will hit.

Quite often even though an Addisonian is admitted to hospital in Crisis, if they pass away, Crisis is not put down as the cause of death. It will be the heart attack, stroke, pneumonia, flu, or some other thing. Even if the Crisis caused the CVA/MI, or the pneumonia could have been survived if not for the AI. Hence many don’t realise that Addison’s Kills.

Am I angry/frustrated that this can happen in todays medical world? YES

Do I over react about the way Derek and others are treated by some in the medical profession regarding Adrenal Insufficiency? YES

Do I believe Wellington Hospital (CCDHB) should take more responsibility for their lack of communication that gave Derek this Life Threatening medical condition? ASBOLUTELY.

Do I believe that more medical staff need to be aware of this condition, and what to do? YES, especially ED staff.

Do Derek and I live in fear that he will again end up in hospital with multi-organ failure due to an Adrenal Crisis? OF COURSE. But we will not let that rule out lives and dictate what we do.

Having said that, we will take precautions against things like stomach bugs, the flu etc. So don’t be offended if you turn up on our door step with “only a common cold” or “just a little tummy upset” and expect to be welcomed with open arms. This little tummy upset that you are exposing my husband to could potentially kill him. That is not to say we will turn you away, but we will keep our distance.

Derek has gone into crisis in front of medical staff even after telling them we thought he would. When asked by his Endo later why he went into crisis, we thought about it. It was because we listened to his Endo about something we thought was an early sign of extremely low cortisol and impending crisis which the Endo was sure wasn’t. In fact, the Endo was adamant that it was not a sign of low cortisol. So Derek didn’t take extra cortisol until it was too late. He went from “just not feeling right, with this one symptom to crisis within an hour. Most of that time was spent in ED trying to get help.  We know better now.

The medical staff at the hospital ED department didn’t recognise the crisis when it happened. Why? Because a Crisis is never Text Book. Each Addisonian will react differently in crisis.  You will not get ALL the symptoms, only some of them. One of the key symptoms the medical staff look for is a decrease in BP to <90/50. The fact is, many AI sufferers have an Increase in BP to start because they have swallowed down so many steroids trying to avoid it. They will check your temperature but they won’t consider it high unless it is over 37.8 (Addison’s causes low body temp, many sitting around 36.0). They don’t tell you that in the medical books though. What medical staff look for is the signs and symptoms as written in the medical books. Unfortunately when you are that sick, you are being shipped off to ICU because you are in shock and your body is shutting down. Personally, I don’t want to see Derek like that again, and will therefore always insist he is treated before he gets to that point.

Two things help keep Derek and others stay out of hospital. One is self-education, and the other is a good support network.

Being told “just take these pills and you will live a normal life. Oh, and learn how to inject, but only after you have vomited 3 times” is not education. In fact, after you have vomited 2 times, you are probably verging on unconscious and incapable (unable to behave rationally or manage one’s affairs). You very seldom have the ability to give yourself an injection and very often don’t believe you need it because by then you brain is not telling you the truth. You are not going to be able to call an ambulance. You must be proactive and seek help before you get to that point. And those around you must make themselves aware of the symptoms so they can also help you.

We are lucky, we have the emergency injection, and have never needed it, although we almost had to use it in the emergency department, but they finally gave Derek the life saving injection he needed and were then shocked to see how quickly it worked.

But for the want of a US$10 injection Katie may not be in the PVC she is now in. Her family may not be sitting by her bed daily watching her in pain.  There is no knowing if giving this beautiful spirit her injection when she was “just feeling unwell” could have saved her from this. But it sure as hell would have given her a fighting chance!

Drs need to understand that what they say, can and does, have a big impact on their patients lives. Yet they never accept when they are wrong (or hardly ever).

 

aiunited

Enter a caption

For more on Katie and her battle, please read
http://aiunited.org/katies-story-adrenalcrisis/

 

Did you Really Just Say That?

In the last couple of weeks I have been compiling a list of comments from Medical Practitioners to Addison’s disease patients.  I have called it “Did you really just say that”.
Unfortunately, although these are specific to Addison’s disease, it is not just Addison’s patients that this happens to.
I am sure many with Chronic Illness have heard similar comments about their conditions.

did you really just say that 

Please feel free to download it and read it.

Now you know it, forget it!

That is the Mantra of the Lecturers to Medical Student’s after being told about rare diseases.

I was talking to a Medical Student (just completed her 5th year) while I was with someone at the Emergency Department of a hospital recently.

We began talking about Chronic Illnesses and the fact that the families of those with Chronic Illness know more than many Dr’s about their particular condition.

She asked me about Derek’s illnesses…. “do you mind me asking what he has?”

No, I did not mind her asking. I am all for education.

I told her Antiphospholipid Syndrome. “Oh” she says, “I have heard of that”.

To say I was very surprised would be accurate.

I then asked her how she had heard about it.

Apparently, they were told about it in a Gynecology Lecture, had to learn about it in relation to pregnancy, and had a question on it in their 5th yr Exam.

They were then promptly told to forget about it, as they would never meet anyone with it.

She was not aware that Men could get it, or that it was something that can and does occur outside pregnancy!

Given that I know at least 4 women with it, and Derek has it, it isn’t that rare.

She knew nothing about the Catastrophic variant  or even that such a beast existed.

This is not the first time I have heard this about rare conditions.

Every time we query a Junior Dr or fully trained Nurse they say the same thing about Addison’s Disease/Adrenal Insufficiency. “We are told it exists, what the symptoms are, then told “but don’t worry, it is so rare you will never meet anyone with it”.  They are then shocked to hear about Derek.

If he is in hospital, the Medical Students are told to go see him, they will never meet anyone like him again!

No, they won’t. Because if they have a patient with either of Derek’s conditions, the patient will probably die before they are diagnosed.

Then there are those patients who suspect they may have a rare chronic illness is who are frequently (almost always) told “No, you can’t have that, it is too rare!”

Another useful thing all Medical Students are taught is: “if you hear hooves, look for horses, not Zebras.”

Just because a condition is rare does not mean it doesn’t exist.  It also does not mean that a patient is imagining things.

So what does this mean?  It simply means you look for Zebra’s if the Horses aren’t in the room.

How can you rely on the Medical Fraternity, when they so often get it wrong.

That is not to say they should know everything. What they should be doing is listening to their patients. Don’t rule out things just because they are Rare, or not “normally” found in that sex.

Listen carefully to your patients. If what you think is wrong comes back negative, give your patient the benefit of the doubt. Do the basic test to see if it IS  a “Zebra in the Room”. If you want to be arrogant, do the test to prove your patient wrong.

What is the worst that can happen?  The Patient is right, and you have to apologize.

Heaven forbid a Dr should find out they are not GOD!