Still a Single Road at Times

Aside

In 2013, my second post was very short.  It was entitled “As single road at times”.

My posts back then were very short.  They were a lot about how I was feeling, how we were learning to live with this condition strange new called Primary Adrenal Insufficiency (Addison’s Disease).

It was May 2013 that I began to Blog.  In the last 6 years a lot of things have changed.  But a lot have stayed the same, we have just learned to accept them and adjust to them.

Going to live performances, etc alone has become a new normal.  I buy 2 tickets, and find someone to join me if Derek isn’t up to it.  This could be a big show, or one that our children are performing in.  Adjusting things so that we can go together has also become a “thing”.  Whether that is by buying a matinée performance ticket instead of a night time one, or making sure he has a full day of rest, including a sleep, we will do what we need to, to keep going.

In May last year Derek had a set back.  It was serious but it wasn’t obvious.  A few blood tests went haywire for no obvious reason, he became a lot more tired than normal, and other subtle differences occurred.  Despite this we carried on with life.  We continued planning our big adventure overseas.

We did the trip.  It was extremely tiring on Derek.  We did a lot of walking with him using a walking stick constantly.  We would walk, stop for coffee, walk, stop for a cold drink, walk, stop for a few photos, walk, stop for an ice cream (they make great ice cream in Europe).  You get the picture.

Derek has been more tired than usual since getting home.  It’s now 6 months since the trip and he is still a lot more tired than he was before May last year.  So we have made more adjustments.

We have learned from that trip that you have to choose the fight.  So now, if we are going out and we know it will be stressful, or very tiring on Derek, we use a wheelchair for him.    After borrowing one for an evening we knew would be too hard on him without, he finally agreed that he really did need it.  But not all the time, just for the harder events.  It means that sometimes he can go out, and we can enjoy ourselves together.   I still go out on my own because he is not up to it.  Or we cancel our plans because I don’t want to go alone.

What we thought was just residual tired from the trip appears to not be that at all.  After having more “crazy blood tests” we began to wonder.  We have now looked at his bloods for the last 12 months, what ever is wrong, has been wrong since his various blood levels went haywire in April/May last year.  But that’s ok, there is nothing we can do except keep an eye on his levels so that is what we do.

The reason we know things have not be right since May is that we keep track of all his blood tests.  We don’t need to go the Doctor to ask, we can see.  We know the symptoms, we know the signs and we can see his “normal” is changing and not for the better.

Having said that, in the words of a good friend who writes an amazing blog, he is clearly alive and will remain so.

Advertisements

Biologic Half-Life of Hydrocortisone.  Why is this important?

As Derek lives with Adrenal Insufficiency, we started looking into his steroid doses to work out whether he was on the best dosing schedule possible for him.

In 2016 we had an Endo appointment and asked for a Day Curve to confirm his dosing was right.  It was refused.  So we asked for 1 random cortisol blood test.  This was agreed to, more to keep us quiet than that the endocrinologist was actually looking for something.  What we didn’t tell him was what WE were looking for.

We both believed that his dosing at 3 times a day was leaving him with low cortisol in the middle of the day.  The only way to show this was to have a random cortisol taken right before his second dose of the day was due. His dosing at the time was:

6.00 am – 10mg / 12.00 noon – 7.5 mg / 4.00 pm – 5 mg

The problem with this dosing was that by 2.00 pm every day he was feeling like he wanted to sleep, and felt “blah”.  Some days he was also showing clear signs of low cortisol.

We had seen tables that said that cortisol had a Half Life of 8-12 hours, but that didn’t make sense.  We had also seen other tables that said 2 hours.  That was a big difference.  We needed to know what was going on for Derek.

1 Blood Test Tells It All

On the day we had set for the test Derek took his morning dose as usual at 6am.  We then did the things we normally do on a weekend, very little.   At 11.15 we went to the Lab for the blood draw.  We wanted it as close to his second scheduled dose of the day as possible.

When we got the results it showed what we already believed.  He was under range.  Not just under range for that time of day, but below range completely.  His cortisol was not lasting long enough in his body.  But we had been shown tables that said it had a biologic half-life of 8-12 hours, so how could he be below range in 5.5 hours?

This didn’t make sense even though we knew it was right.  So we started looking into what was meant by biologic half-life.  What we found out is very scary, very concerning, and actually very dangerous.

What did we find?

BIOLOGIC HALF-LIFE CAN BE RUBBISH.  It can be a false number, it shouldn’t be used in the way the below table indicates.

The table here is beening used by many groups/forums and on medical sites including on websites such as Endotxt.org, NCBI, and NADF so it must be right, surely.

Do NOT use this to work out the half life of your Hydrocortisone or Prednisone for dosing!

 

Here it was, the table we got shown constantly.  So Derek started looking further to try and find out where the biologic half-life came from.  The first thing he found was the definition for biological half-life:

 

“Time required by a body to process and eliminate half the amount of a substance introduced into it. Also called biological half-life, biological half time, metabolic half-life, or metabolic half time.”

A number of variations of this table appear on the Internet and use the column heading Duration of Action.  Other variations of this table simply classify the corticosteroids as short-, intermediate- or long-acting.  The same numbers apply no matter what the column is referred to as.

If this column truly is a (biologic) half-life, and we apply the rule of 5 half-lives for complete elimination, then that would mean that Hydrocortisone would be visible in the body for up to roughly 2 days (40 hrs).  Yet when Derek had a blood test before his morning dose, his cortisol was undetectable having had HC at 4pm the night before.  That was 17 hours and no detectable cortisol.  What would happen for the other 20+ hours?  It was clear there was something seriously wrong with this table.  None of this would be consistent with the title Duration of Action.

Also, if that was the case, you would only be prescribed cortisol once a day, not 3x, or more often now, 4x a day.

Where did this Table column come from?

There is no clear ownership of the table that we could find.  It is used, copied, and the copy is referenced, but tracking back to the original hasn’t been possible by us.  We do know it was created before 1980

He became very curious and decided to look further for the source of the information and came across this quote from “Principles of Endocrinology and Metabolism”,3rd edition, 2001, Chapter 78 “Corticosteroid Therapy” by Lloyd Axelrod.

This paper references the definition of:

“The commonly used glucocorticoids are classified as short-acting, intermediate-acting, and long-acting on the basis of the duration of the corticotropin (ACTH) suppression after a single dose, equivalent in anti-inflammatory activity to 50mg of prednisone.”

This is all about suppression of ACTH on high doses of prednisone, nothing to do with the amount of time you will remain within a safe cortisol range when you have Adrenal Insufficiency, yet Dr’s and patients alike use the table to justify twice a day dosing on HC.

So what are the implications of this table?

If someone uses this table to tell you that half-life is 8-12 hours for hydrocortisone they are wrong.

After looking for the original source of the table we discovered that the test was done as above, with a normal healthy person being given 50mg prednisone (approx 200mg HC).  The only thing that can be taken from the original research is that 50mg prednisone will suppress ACTH production for a period of time.  The hydrocortisone, and other drugs, were extrapolated from there (guess work based on poor knowledge).

If you had Primary Adrenal Insufficiency (Addison’s) and Hydrocortisone had a half-life of 8-12 hours, then taking HC every 6 hours would mean constant suppression of ACTH, and you would not have high ACTH after starting the steroid.  But we know this isn’t correct because many with Addison’s still have some part of their Addison’s “Tan” due to raised ACTH.  This is supported by the document below.

Professor Peter Hindmarsh is Professor of Peadiatric Endocrinology at University College London and Consultant in Peadiatric Endocrinology and Diabetes at University College London Hospitals and Great Ormond Street Hospital for Children. He is currently Divisional Clinical Director for Paediatrics at University College London Hospitals.  He also runs a website called CAHISUS.  He has written an article called GETTING CORTISOL REPLACEMENT OPTIMAL IN ADRENAL INSUFFICIENCY

The major goal of cortisol replacement in patients with adrenal insufficiency is to mimic as closely as possible, the normal pattern of cortisol production known as the circadian rhythm. The reason why we try to achieve this, is primarily to minimise side effects of over and under replacement and promote improved overall health. The two key factors are understanding the circadian profile and the pharmacology of hydrocortisone.

In this article Prof Hindmarsh talks about getting optimal dosing, and also looks at the absorption and clearance of people.  What he showed is that there is a very large variation between people. The article is well worth a read.  He also pointed out that the half life of hydrocortisone is a lot shorted than 8-12 hours, in fact, it is more like 70-90 minutes.

Another CAHISUS leaflet states this:

Hydrocortisone has a quick onset and the cortisol peaks to the highest level usually around 2 hours after being taken.  The cortisol obtained from the tablet lasts in the blood circulation between 4-6 hours.

This is a change from an old document by Prof Hindmarsh which included the old figures as above.  Things have changed, research has improved, and there is more knowledge out there.

What Does All This Mean in Steroid Dependant People?

For me?  Gobbledygook.  If you have a clear understanding of Half-Life, Clearance, and metabolism you may follow what is talked about in the studies.  Personally, it confuses the heck out of me.

I do however, understand the concept of half-life.  I first heard about it when watching a movie years ago about a child who had a certain amount of a chemical in his body at point C, and they claimed he had been given the chemical at point A.  It was pointed out that he would have drunk a gallon of the chemical to have the amount still in his system because of the half-life of the chemical.  The chemical had been very bitter and it would not be possible for the child to drink that much.  I became very interested in half-life.  I didn’t think then that it would be so important in Derek’s everyday life.

I had to ask Derek what everything he had found, and what the implications of half-life on hydrocortisone meant in layman’s terms, but even he struggled to explain it in a way that I could be easily understand. One thing he reminded me of is that while your Cortisol is going up, it is also being used.

I have also learned through this research is that even legitimate medical websites actually have misleading or wrong information.

When you are looking at a good way to dose for you, it must be an individual choice, based on how you feel between doses, whether you are willing to take multiple doses a day, and base it on signs and symptoms.  The fact that Derek felt low at the scheduled time of his second dose of the day, and this was supported by a blood test that showed low cortisol, meant we could get the Endo to agree that dosing more frequently was the right option for him.

Now that he is on a better regime of 4 times a day, he functions a little better, he has a low base level of HC, and in the last 6 months, has lost weight without trying, but not in a bad way.

I wish you all luck with this as I understand that there are many Dr’s out there who are not interested in listening to their patients on more dosing throughout the day.  One of the reasons for this is they don’t believe that you will be compliant, even though you are the one asking.

If they think you are asking for something that shouldn’t be done, then show them Prof Hindmarsh’s document above.

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.

International Travel with a Chronic Illness

leaving-on-an-aircraft

Our Next Adventure Part 1

Having managed tripping up and down the North Island of New Zealand over the last 4 years, including flying, just to see how it would go, Derek and I want to venture a little further.

The Practice

To begin our preparation for an international flight, the first thing we did was try flying to Auckland.

It required a trip to the Airport, then flying to Auckland, and driving to Hamilton.

Derek took extra medication to fly.  He took extra hydrocortisone for the drive to Hamilton from Auckland.  He then lay down for a long rest when we got to Hamilton.  He also had to rest the next day, but that is normal when we travel any kind of distance.

On the whole, the trip was good.  We listened to others’ advice, had learned what Derek could manage, and when he needed to up dose.

Flying Further

This time we decided to go to Australia.  Originally our first trip overseas was going to be to Sydney or Melbourne for a weekend for a Show. After being offered a trip to Fiji earlier this year, and the realisation that a 2 day trip would be way too hard, we thought a longer trip would be better.  Because a friend with Addison’s was heading from Hong Kong to Brisbane for medical treatment, and we had friends in Brisbane, that was going to be our destination.

We are not the first to travel with chronic illness, and we won’t be the last.  When you look around the cue of people going to the flight, you don’t know who has a chronic illness, who has spent days and weeks preparing, and who has just grabbed a ticket and headed to the airport.

I asked my cousin, who’s husband has several serious medical issues, what they do to take a trip.

Trev just sorts all his medication and I just carry it…never had any problems, I think I have only been asked once and they were good about it. I never carry hospital documents but for you guys being a first since Derek has been sick, just get your GP to write a letter out lining the diagnosis and a list of the medications on the letter. Never really had problems with insurance just be up front about it all, you may not get full cover but shop around. We don`t stress about overseas travel, there is always a hospital where you go if things don`t go to plan. Just relax and enjoy the trip. We are probably not a good example…as we are pretty relaxed about it and just roll with it…lol…isn`t that naughty, but thats just us…great place we have just come back from there.

The trip they had returned from was a trip to celebrate 25 years since “Trev” had an organ transplant.  (By the way, I am totally in favour of organ transplants.  It saves lives, including that of my cousin’s husband so please think about donating organs if the unfortunate need should arise).

Booking the ticket.

We have made the decision to travel over to Australia.

Now What?

Do we need to get permission from the Dr?

We don’t believe so because we went to see her about going to Fiji with a work trip for Derek, and she said no, because of the risk of food poisoning, the possible need for INR while there, and the short time frame.

But she said that if we wanted to travel somewhere else, like Australia or England, then yes, she would make sure we could do it.

We bought the tickets. tickets

When I booked it, I also requested a wheelchair at both ends.  That meant an alert on the ticket booking, and I had to call a number, and speak to someone.

The flight is a Partner flight, which means we are booking through one airline, but the flight is provided through another, so there was a delay while the confirmed that the wheelchair was available.

We knew from experience going places with Derek he could not stand in line long.  He gets very fatigued just waiting at the supermarket, and if there are more than 2 in a line, he has to sit while we wait.

Then there is the timing of the flight.  We could fly out at 11am.  That would mean Derek could wake up as normal, and we could take our time.  But that also meant 9-12 hours flying as we would have to fly from Wellington to Auckland, wait for 2 or more hours, then fly to Brisbane.  We would arrive at night, and it would be a very long day.

That would take more out of him.

Alternatively we could fly out from Wellington at 7am.  And then fly for 4 hours, directly to Brisbane, and land at 8am Brisbane time.

This meant a lot less travel time, but a very early morning.  Derek normally wakes at 6am, takes his first round of HC, then goes back to sleep for an hour while he waits for his meds to kick in.

This trip would mean waking him at 4am to take his first round of HC, Drive to the airport, have breakfast, then check through Customs. (hopefully we haven’t got any fines that hold us back).

So next we book an appointment with the Dr.  We need:

  • A letter for treatment protocol (if he suffers an Adrenal Crisis)
  • A letter confirming he is able to fly (because of Antiphospholipid Syndrome and risk of DVT)
  • A list of medication (it needs to be declared at every port entry and exit).
  • Any meds he may run low on before he leaves, or soon after he gets back.
  • We also need to organise an INR when we land in Australia. This is the bit we are unsure of, but the essential bit to make sure it hasn’t dropped too low while flying, which could mean a blood clot.

Then there is Travel Insurance.

Normally you just buy it when you buy your tickets.  All you need for Australia is cover so if you get waylaid, you can get a change of ticket.  But if you have extra conditions, you need to tell them about them.  Then the cost goes way up.  It went up by $150 for Derek.

But we don’t know if we have declared everything as they didn’t have the ability to declare the catastrophic event he had.  And I hadn’t declared his prostate cancer.  Oh well, looks like a phone call.

I got two quotes.  So we needed to call both companies to see how things would change.

After a long phone call to each company, Derek’s insurance was going to cost $6 extra for the prostate cancer which is in remission.

Medication

Emergency Kit

The Take Every Where Kit

We would need a list to make sure we took everything.   It currently sat in multiple areas of the house.  We also needed to make sure it all had a proper prescription labels.

When my friend Wendy travelled from Hong Kong to Australia I told her to declare, declare, declare.  If she declared everything she would be fine.

I was going to work on the same principle.  But that is not the case for Derek as he takes DHEA (Dehydroepiandrosterone).  It is classed as an anabolic steroid, and therefore restricted.  Derek needs a licence to carry it into Australia, even as a prescription medication.

And one of his other medications needs authority to carry it out of New Zealand and then back into the country.

So, we have a Dr’s note x 2, we have an emergency letter, we have an application to take his medication into Australia.

We can only carry a month’s supply in and out of New Zealand but that’s fine because we are only away 6 days.

We have to wait to hear from the Dr regarding whether he needs 1 more medication for the trip, but otherwise, we have things ticked off that we need.

Getting all the advice is key to a successful trip.  So I went seeking advice.

Travel Advice from the Experts

The Addison’s Disease Self Help Group UK has some great advice:

  • Good general advice for long distance air travel includes:
  • Remember that air travel is dehydrating so you will need to drink more fluids than usual in the air. Drink alcohol, cola drinks, coffee and tea sparingly as these dehydrate the body further. If possible, carry a large bottle of water in your hand luggage. If you forget to bring your own water, be assertive about requesting extra refreshments from the cabin crew.
  • Walk around the plane as much as possible. Try to get up out of your seat every two hours to stretch your legs and keep the blood flowing.
  • Many chemist shops now stock knee-length support socks, which can help prevent the formation of blood clots that might lead to a deep-vein thrombosis (‘stroke’).
  • Adjust your watch to the time of your destination as the flight begins, and adjust your in-flight activities to that new time zone as well. Sleep through the in-flight meals, if necessary, to get attuned to the new time zone.
  • Try to book flights that allow you to arrive at your destination in the late afternoon or early evening local time, so that you get a night’s sleep at the end of your travelling. Flights which arrive in the early morning local time will leave you tired after travelling but having to stay up all the day before you get a proper night’s sleep.

So we missed the last one.

But we are going to stay at the house of an Addisonian, so they will know that Derek will be going to have a sleep when he arrives.

So everything we can think of is ticked off.  Now to wait.

Now to wait for the actual trip.

We are excited about the trip.  We have thought of all the possible issues, and taken care of everything we can.  Derek isn’t being wrapped in bubble wrap, but we are reducing the risk as much as possible without saying, “too hard, not going.”

Next Chapter – The trip.

I am hoping this will be a very dull chapter with just the excitement of having the Sunday lunch with other addisonian’s, and enjoying visiting somewhere I have never been.  I won’t apologise if my after trip post is boring, as that would be the best trip ever.

 

 

 

WHY DR’S CAN GET IT WRONG!

I am a desk top publisher.  I have used various desk top publishing tools from Aldus Pagemaker in the early 80’s  to CS InDesign of the 2010 plus era.

I have created everything from A4 single page leaflets, to a 600 page, multi chapter Scientific document.

I understand more than some, how these larger documents are put together.  When you have 2000 pages of information, written by 90 different contributors, across 5 different countries, and multiple regions and states within those countries this book must be constructed in a particular way.

So why is this important to my readers?

In my last blog, I briefly mentioned medical books can and will have out outdated study data when published.

In this blog post I am going to expand on that, and explain why we must all be aware of the implications of this.

When a large Cohort of people have input into a Text book on a particular topic, you will get differing opinions.  When that book looks not just at a Broad topic, but then drills down into each sub-topic, it must be created by Section and Chapter.

Each Chapter is co-authored, then all chapters are put together at a publishing office to be produced in book form.  This can, and does, lead to conflicting information in the book, if not carefully checked by someone who understands the different chapters.

In this case I am talking about Williams Textbook of Endocrinology.

Why this book in particular?

Williams Textbook of Endocrinology claims, in their own words:

“In this new edition we have strived to maintain Robert Williams’ original 1950 mandate to publish “a condensed and authoritative discussion of the management of clinical endocrinopathies based upon the application of fundamental information obtained from chemical and physiological investigation.”……..
With these goals in mind, we have once again assembled a team of outstanding authorities in the field who each contribute their unique expertise in the synthesis of current knowledge for each area.”

This is THE book that Endocrinologists go to for information.

But what happens when this book has failings?

Well, we in the Forums see the results of that every day.

I am only going to look at two chapters.  I do not know the authors of these chapters, and I am not calling in to question their ability to read, and interpret their referenced material, or their knowledge of the Endocrine system.

What I am calling into question is the ability of such an important publication, to make sure that their information is up to date, accurate, and most importantly, consistent across chapters.

What led me here?

Quite simply, a comment from Derek.

We don’t go randomly into anything where his Adrenal Insufficiency, Antiphospholipid Syndrome, his Dysphagia, or any other conditions are concerned.  We read, we research, we look at multiple published and peer reviewed studies on the subject.

In doing this, we have been able to stablise Derek’s condition and get him the best Quality of Life possible for him personally.  This is not as good as some, but not as bad as others.  This is because his limitations are different to everyone else.  There are no two people with exactly the same concomitant conditions and therefore, there is no one size fits all solution to treatment.

We have found a number of things when it comes to Adrenal Insufficiency.

  • A lot of “studies” are in fact people that have read other studies, and are giving their opinion on them by pulling them together and writing their interpretation.
  • There is not a large amount of new information out there.
  • Some of the new studies are looking at medical files, relying on Dr’s writing accurate notes (which many with AI have found to be inaccurate) and very few are actually talking to a large cohort of patients.
  • Many tests on cortisol replacement are conducted on healthy subjects (or on a very small cohort (10-50) of AI sufferers, then applied to those with AI trying to mimic what happens in healthy people, not what happens in the chronically ill.

While researching something one night Derek found a snapshot of Chapter 15 from Williams Textbook of Endocrinology.  He started reading it and found it so interesting that he was talking about getting hold of a copy of the whole book.  It was over $200 NZ for an eCopy.  Unfortunately, while reading it he came to the end of the available snapshot before he could finish what was being said.

He asked me if I had ever found reference to, or knew of this book.  I hadn’t, so I started searching for it.  I found the whole book, available for a limited time (the website would allow you to read it or X number of days), via an eBook.  So I started looking up the information he was wanting.

What happened is that I found a different chapter to him, and found what I thought was the information he was searching for.

The problem with that was, the information I found, was different to what he was sure he had read.  But how could that be.  Surely, when talking about the same condition, the recommended treatment would be consistent across all chapters.

I am not talking about just the same subject, I am talking about the exact same condition of Adrenal Insufficiency, or your body being unable to produce it’s own Cortisol.

In this case, Long-Term Replacement Therapy vs Adrenal Steroid Replacement.  They were different names, for the same information, that is Cortisol replacement for Adrenal Insufficiency (both primary and secondary).

OK you say, different names, nothing wrong there, the information will still be the same.

But it wasn’t!  What I found, contridicted what Derek had found.  But we were looking at the same edition of the same book, just different chapters.

There were two different theories on adequate timing and amount of replacement.

Here is where it affects those with AI

Most of those in the Forums, are taking either 2 or 3 doses of Hydrocortisone a day.

For many, this is what they were told when they started Hydrocortisone.  Some of these people have been on the same dosing regime for over 50 years.  It has never changed, it has never been considered they could change.

And this is still what they are being told.  The amount and dosing schedule you are given, is dependant on the Dr that diagnoses you and his knowledge, or where he reads about the recommended dosing schedule.

Depending on which chapter the Dr reads the “Gold Standard” is either 2 doses a day or 3 doses a day.  The amount will vary from 15mg/day as adequate, 20mg/day, and no more, or some are given a range from 15-25mg/day which they can work within.

The 1 consistency is that 30mg/day is considered too much for the average person with AI.

So where are these Dr’s getting their information, that it can vary so much.

Most likely it is all coming from the same book.  But it will depend on which Chapter of Williams Textbook of Endocrinology the Dr is reading.  And also, whether he is reading the text, or looking at the Tables.

Let me show you.

WILLIAMS TEXTBOOK OF ENDOCRINOLOGY, 13TH EDITION Copyright © 2016 by Elsevier, Inc.

CHAPTER 8 Pituitary Physiology and Diagnostic Evaluation
SECTION II Hypothalamus and Pituitary

Adrenal Steroid Replacement

Hydrocortisone is widely used for glucocorticoid replacement. The normal secretory rate of cortisol is 15 to 20 mg/ day, which is the recommended total daily dose. As plasma circulating half-life of cortisol is less than 2 hours, three times daily dosing of a total daily requirement of 10 to 20 mg (5-10 mg in the morning, 2.5-5 mg at noon, and 2.5-5 mg in the evening) is recommended.

Reference:  Howlett TA. An assessment of optimal hydrocortisone replacement therapy. Clin Endocrinol (Oxf). 1997;46:263-268.

Where is the problem.  It recommends 3 times a day replacement.

However……….

CHAPTER 15 The Adrenal Cortex
SECTION IV Adrenal Cortex and Endocrine Hypertension

The aim of long-term therapy is to give replacement doses of hydrocortisone to mimic the normal cortisol secretion rate (Table 15-21). ………

Reference:  Howlett TA. An assessment of optimal hydrocortisone replacement therapy. Clin Endocrinol (Oxf). 1997;46:263-268.
Doses are usually given on awakening, with a smaller dose at 6 PM, but some patients feel better with three-times-a day dosing. In cases of primary adrenal failure, cortisol day curves with simultaneous ACTH measurements are advocated to provide some insight into the adequacy of replacement therapy.

Note the paragraph above a day curve is advocated.  In the text book they then give a reference for this belief.

Reference:  Arlt W, Rosenthal C, Hahner S, et al. Quality of glucocorticoid replacement in adrenal insufficiency: clinical assessment vs. timed serum cortisol measurements. Clin Endocrinol (Oxf). 2006;64:384-389.

CONCLUSIONS:
Our results suggest that serum cortisol day curves are of limited value in the monitoring of glucocorticoid replacement. Bone mineral density in AI is generally normal and does not require routine follow-up.

The conclusion of the Study referenced  is that it is NOT required, yet the text book advocates it.  Either the reference needs to be removed, or the advocating of the test should be reconsidered.

Then we have Table 15-21

Table 15-21-1
HC Dosing of 15mg am and 10mg pm, or 20mg am, and 5mg pm to a max 25 mg HC per day

So you can see above, depending on where the Dr gets his information i.e., which chapter, depends on the dosing amount and schedule you are given.

One recommends 3 x day but only up to 20mg max, one says “2, but some feel better on 3”.  The table (the easiest thing to look at when you are scanning 1800+ pages of information) states 2 x daily.

So Why the difference in Maintenance Treatment?

Simple, the articles are in two different chapters of the same book, but written or reviewed by 2 different groups, The first was by 1 person from Queensland and 1 from the States (Chapter 8) and the second by 2 people from England (Chapter 15).  It is hard, but not impossible, to ensure the treatment is consistent, however, the distance, and disjointed way such a large publication is created can lead to different opinions and the inconsistencies.

If I was a Dr dealing with a patient with a Pituitary issue, I would possibly read SECTION II Hypothalamus and Pituitary; Adrenal Steroid Replacement.

Having read that, I would not then feel a need to go to SECTION IV Adrenal Cortex and Endocrine Hypertension; Long-Term Replacement Therapy to see that the replacement there was different.  And if I did, what would I do?  2 or 3 doses a day?  I certainly wouldn’t follow the references to read and make a decision for myself.  I have 15 other patients to see that day.  I will take the first answer I come across.

After all, this is the Expert Go To document to see what is needed.  These specialists have already done the hard work.  As the Dr, I have to read what I need to read.

But where does that leave the patient.  Normally on a dose that doesn’t necessarily work for them, and in most cases, without any real cortisol cover for many hours of the day.

If you take a large dose in the morning, the half life is still the same, <2 hours.  By the end of 4 hours, you are still having a dip.

The other problem is, much of the reference material is at least 15 years old, having been written in the 1990s.  There is more recent research but instead of removing the old research, the just add to it, to increase the confusion for anyone who actually looks deeper into it.

And then there’s the Emergency Injection

Now a quick one about carrying your emergency shot.  This same document, under Section IV; Adrenal Cortex and Endocrine Hypertension it very clearly states :

Parenteral preparations of hydrocortisone for self-administration may be required for patients living far from hospitals and those planning vacations.

Hence we have had death, and permanent impairment of patients who “live close to a hospital” who are being refused their emergency injection.

Many are refused their emergency injection based on that 1 paragraph, out of a book of 1900 pages.

Changes over the years.

After seeing all of this, I decided to see what had changed in the last 13 years, in the treatment of Adrenal Insufficiency.  I have managed to access  a copy of the 10th Edition, published in 2003.  In comparing word for word, what is said about Long-Term Replacement Therapy there have been changes is wording, but not meaning, of a few paragraphs, and 4 paragraphs added near the end.

The changes in wording is of no importance as it didn’t change the context.  However, in at least 13 years (I couldn’t get an earlier copy on line) these are the actual changes to treatment:

Added:

Progesterone is a mineralocorticoid antagonist, and the rising levels across pregnancy may necessitate an increased dose of fludrocortisone.

Added:

Patients should receive regular education regarding the requirements of stress-related glucocorticoid dose adjustment, which should involve the patient’s partner and family as well. Parenteral preparations of hydrocortisone for self-administration may be required for patients living far from hospitals and those planning vacations.  (Italicised has not changed.)

Added:

However, patients with adrenal insufficiency on current steroid replacement regimens have significantly impaired health-related subjective health status irrespective of the origin of disease or concomitant disease.

Very last paragraph as if added as an after- thought as reference was published at the end of 2014

Added:

Delayed-release hydrocortisone preparations, such as Plenadren, that more closely replicate normal circadian cortisol concentrations, have recently been licensed and approved; early clinical trials show improved quality of life in both primary and central hypoadrenalism compared to conventional twice- or thrice-daily hydrocortisone administration.

Very little has changed, including the table stating twice daily dosing in the Adrenal Cortex section of the endocrine Text Book.  That is 13 years and we know that one of those statements is wrong.  ALL those suffering AI SHOULD BE GIVEN THE EMERGENCY INJECTION!

This is Critical to the survival of an Adrenal Insufficiency patient.

So where to from here?

A well worded letter to the people that wrote SECTION IV Adrenal Cortex and Endocrine Hypertension; Long-Term Replacement Therapy with a request that they urgently update their information, and issue everyone with that update, to ensure the safety of patients with Adrenal Insufficiency is paramount, and that assumptions of competence on the part of their over worked, time poor medical practitioners, is corrected to take into account a of lack of knowledge by the Endocrinologist who, never having had an AI patient before, just treat one.

Also Endocrinologists must be given consistent information no matter where in their Bible they look (or where on the Internet if needs must).

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/