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.

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What Does YOUR Adrenal Crisis Look Like?

April is Adrenal Insufficiency (Addison’s Disease) Awareness month in many countries of the world so I thought I would look at what an Adrenal Crisis is to those that suffer with Insufficiency.

I don’t mean, what does the medical world consider a crisis, but at what point do those that suffer AI believe they are in crisis, and at what stage do they feel they need medical intervention.

Let’s start with what a medical dictionary says is a CRISIS:

According to one online medical dictionary:

crisis

 [kri´sis] (pl. cri´ses) (L.)

  1. the turning point of a disease for better or worse; especially a sudden change, usually for the better, in the course of an acute disease.
  2. a sudden paroxysmal intensification of symptoms in the course of a disease.
  3. life crisis. – addisonian crisis (adrenal crisis) the symptoms accompanying an acute onset or worsening of addison’s disease: anorexia, vomiting, abdominal pain, apathy, confusion, extreme weakness, and hypotension; if UNTREATED  these progress to shock and then death.

Alterntively the Oxford Concise Medical Dictionary (9 ed.) states:

  1. the turning point of a disease, after which the patient either improves or deteriorates.

Neither of these sources, or any other I can find, state that a medical crisis is only once the patient has entered a state of hypovolemic shock.   In fact, the first one states it only progresses to shock if a Crisis is UNTREATED.  Yet SHOCK is what the Dr’s wait for, or believe to be a crisis, in the ED when an Adrenal Insufficiency patient presents.

Professor Bruno Allolio stated in his document EXTENSIVE EXPERTISE IN ENDOCRINOLOGY – Adrenal crisis

Bruno Allolio1,2,†

Prevention of adrenal crisis

Physiological endogenous glucocorticoid secretion (normal Cortisol production) is highly flexible with rapid adjustments to unexpected needs….

In the foreseeable future (if ever), no replacement therapy will be able to fully mimic this amazing adaptive potential of a healthy HPA axis.  However, many stressors can be anticipated (e.g. elective surgery) and allow to adjust the glucocorticoid dose to the expected need, thereby preventing the occurrence of clinical deterioration and adrenal crisis.  It is important to understand herein the underlying concept.  The recommended dose increase is not intended to mimic the median cortisol increase in healthy subjects during such procedures.  Instead, it is intended to mimic the maximum cortisol increase, which may occur in euadrenal (normal functioning adrenal gland) subjects triggered during these procedures, potentially induced by some unforeseen events (e.g. postoperative bleeding).  Patients with intact adrenal function can respond immediately to such problems with an increase in adrenal cortisol output.  By contrast, in adrenal insufficiency, additional glucocorticoids would only be given when clinical deterioration becomes evident.  Until then, valuable time may have been lost to adjust the hydrocortisone dose to the increased need.  For that reason, dose adjustments aim at the upper limit of the normal variation to cover such unexpected needs.

As infections are the most frequent cause of adrenal crisis, it has been suggested that the patient doubles the hydrocortisone dose if the body temperature increases above 38.0C and triples the dose above 39. 0C.  This dose is maintained as long as the fever persists and rapidly (within 1–2 days) reduced to the standard replacement dose after recovery.  Gastroenteritis poses a particularly high risk, as glucocorticoid availability may be compromised by vomiting and diarrhoea, while the demand is clearly increased.  Thus, early parenteral hydrocortisone (100 mg subcutaneously) is strongly recommended either via self-administration or by a physician.  This dose may need to be repeated and health-care professionals should be involved early for clinical assessment.  Similarly, in severe infection (e.g. pneumonia) with altered cognition, early parenteral hydrocortisone and medical help are warranted.

It has been suggested that patients today are at a greater risk of adrenal crisis, because they have less of a ‘cushion’ of excess circulating cortisol with a standard daily dose of 20 mg hydrocortisone compared with the old-fashioned standard dose of 30 mg hydrocortisone.  However, there is no scientific evidence for such a protective ‘cushion’ effect.  Instead, chronic over-replacement may rather increase the susceptibility to infection and thereby increase the risk of adrenal crisis.

Since his crisis in 2014 from pneumonia, we have learned when Derek is suffering low cortisol vs when he is needing medical assistance.  We have become very good at getting him to stress dose BEFORE he gets too sick.  He has had 3 infections in the last 2 years.  We have given him very high doses of steroids (up to 40mg in one dose over and above the 5 or 10 he would normally take).  What this meant was ignoring the Dr’s that stated that his first clear sign to US of Low Cortisol,

Doing this, and then getting him to a Dr to find out WHY he is so low, has saved us from having to use his emergency injection or taking him to the Emergency Department for urgent medical intervention so far.

That is not to say that one day we will get it wrong, or not be able to stop a crisis from getting to the emergency state and have to call an ambulance, but we are on the winning side at the moment.

It got me thinking what is it that tells an Addisonian (I am including SAI and PAI in this term for ease of typing) what are the first symptoms they have.  What is is for THEM that says, you need a high oral dose, or you need an injection or an ambulance.

Since Derek’s diagnosis in Oct 2012 we have learned a lot about AI.  The biggest thing we have learned is that NO TWO ADDISONIAN’S ARE THE SAME.  They all have different comorbidities, they all have different symptoms, they all have different knowledge levels, and most importantly they all have different support systems in place, from nothing, to fantastic.

All these things impact on how quickly things can go from good to bad to dead.

The final piece to this puzzle is the ability and knowledge of their endocrinologist.

  1. Have they been given the right advice on updosing and stress dosing, the right knowledge on when to use the emergency injection, have they even been prescribed the emergency injection (some don’t believe in giving it).
  2. Do they have confidence in the care they will receive when they call an ambulance or go to their Emergency Department.

These last two things can will make or break an Addisonian.

So this doesn’t tell us what an adrenal crisis actually is.  And this is the hard part.  I asked some of my Addisonian friends what their signs of crisis are.

Each person I asked gave a different answer.  The symptoms were very similar, but presented in a different order, different intensity, and different clusters.

Symptom #1 Symptom #2 Symptoms #3 Symptoms #4
· Feeling really tired    & listless and
generally blah
· Dizzy &
unbalanced
·  nausea
·  Hot and Sweaty
·  raging migraine
·  high BP
·  So tired just want
to sleep
·  seizures
·  Extremely fatigued,
·  lifeless legs
·  Dizziness
·  pain in abdomen,
·  low back and legs.
·  tachycardia
·  mostly high BP,
·  then it can plummet
·  Disorientated
·  Belief that rest will
fix us.
·  severe torso
spasms
· dizzy,
·  start to stagger,
·  ose balance,
·  barely lift feet, legs
feel like heavy jelly,
·  feel shaky,
·  nauseous/dry             reach.
·  Big D,
·  hard to talk, words
just don’t come out
right.
·  Back pain,
·  torso spasms.
·  Extreme fatigue
·  Abdominal pain
·  Gas/wind
·  headache
·  Speech goes
quiet
·  confusion
·  High BP
·  Light headed
·  Wants to sleep
·  Inability to speak
·  Nausea

 

Each one of these lists shows, a sudden paroxysmal intensification of symptoms in the course of a disease.

Yet the Addison’s Disease Self Help Group in the UK, and the National Institute of Diabetes and Digestive and Kidney Diseases have different definitions of a Crisis.

Symptom ADSHG NIDDK
Adrenal crisis Warning signs include:
·    severe nausea
·    headache
·    dizziness
·    extreme weakness
·    chills or fever
·    confusion.
Symptoms of adrenal crisis include
·    sudden, severe pain in the lower back,        abdomen, or legs
·    severe vomiting and diarrhoea
·    dehydration
·    low blood pressure
·    loss of consciousness

 

All the above experience the symptoms from the ADSHG, but they all also inject BEFORE they get to the severe vomiting, diarrhoea, and low blood pressure if they can.

These symptoms are those experienced with an infection.  A gastric bug is a whole different issue.

These 5 people know, from several years of experience, when they can take in more oral meds, vs when they need to inject.

Yet if they went to hospital, they would be told that they are not in crisis as they are not vomiting, their blood pressure is not low, and they are conscious.

If you break your leg, it’s obvious by Xray (mostly) that it’s broken.  If you have a heart attack, they can show it within minutes, stroke – signs and symptoms are clear, Hypo or Hyperglycaemic they test and they know what to do.  Arm sliced open and bleeding?  Dr’s, Nurses, and EMT’s know immediately to stem the flow of blood BEFORE shock sets in.

But when it comes to Adrenal Crisis there is no “test” they can do, many in the medical profession throughout the world don’t recognise it, don’t believe the patient (or their advocate) when they present in crisis, and unfortunately still don’t know how to treat it.

Many also won’t follow written instructions the patients carry, when you give them information.  We have seen people turned away being told “you are not in crisis”, but they clearly are.

Derek was at an after-hours Dr one day as we though he had a throat infection.  He couldn’t feel much pain as the infection was on the left side of his throat, which is numb from CAPS, and he can’t swallow on that side.  During the examination the Dr said “I don’t believe you need an emergency injection.”  My immediate response was “No, he is not in crisis.  IF we thought he was in crisis we wouldn’t be at After-Hours, we would be at Hospital.”

The Dr acknowledged we obviously knew what we were doing.

Another time we said to the Emergency Room Dr that Derek’s AI was under control, we were not there for that, we were there for another reason.  The Dr was happy with that and dealt with the issue we were there for.

One time that he was dropping fast into crisis, couldn’t keep her eyes open, couldn’t answer any more than yes/no answers, was in a lot of abdominal pain, was nauseous.  Yet the Dr had no idea, even when I pulled out all Derek’s medication and said I was giving him 20mg, the Dr’s question was “Do you really think he needs that much.”  And left his cubical Um, yes, he needs a lot more than that.

They believed us quite willingly when we said he was not in crisis, but when we knew he was heading that way, they didn’t believe us.  The 20mg didn’t do anything.  After finding an old letter a senior Dr from that ED had written, and giving it to a nurse, they finally gave him 100mg and admitted him.

So what is an Adrenal Crisis?

The image here shows THREE pathways to death from adrenal crisis.

One friend spent years having seizures during adrenal crisis.  Yet she was frequently told seizures were NOT part of the adrenal crisis pathway (Far Left Pathway, bottom symptom).

Others have been told, you can only be in crisis if you are vomiting (ONLY middle pathway mentions vomiting).

 

With the 3 pathways in adrenal crisis, and you can be suffering a mixture of symptoms from each.  You do not have to follow only one pathway.  Most medical books only talk about the middle pathway, and don’t look at the two outside ones.

Derek vomited a total of 4 times during his adrenal crisis and multi organ failure yet he was sick/near death for 3 weeks.  He did not vomit at all while in hospital.

If each Adrenal Insufficient patient presents differently, how are the medical world supposed to know what is wrong.

One size does not fit all.  And THAT is a serious problem, with no easy answer.  If someone seeks medical help for anything and they have Adrenal Crisis, they need that checked first.  The Dr need to ask the patient, do you think you need/have you taken emergency medication.

Most patients know if their oral steroids aren’t working.

Do you know YOUR signs?

Does your support person?

 

A Drug Seeking Nut Job?

This story is fictional, but is based on stories of people that I have talked to.

Are you a drug seeking nut job? Lifesaving Steriods ARE NOT recreational drugs!

Are you a drug seeking nut job?
Life saving Steriods ARE NOT recreational drugs!

Sally woke suddenly.  She could feel the headache.  She knew what it meant.  She had had these before.
She reached on her night stand for her drugs.  Taking 4 tablets, she put them in her mouth, took a mouth full of water and rolled over to try and go back to sleep.
She knew that getting this headache was going to mean a bad day ahead. 
Sally looked at her clock.  It was only 3am.  If she could sleep till 7 and then take more drugs she would be fine she hoped. 
She rolled over, praying the drugs would kick in soon.  Wishing the headache would go away, and hoping the rest would not come.  Deep down she knew it would, she knew she would spend the next two days fighting it off.  There was no way Sally was going to go to THAT place again.
In a town a thousand miles from Sally, Dave woke.  He knew that feeling. That urgency.  He also knew what he had to do.  He rolled over and looked at the clock.  It was 5.10 in the morning.  Dam, hopefully he could fix this.
He leant over to his bedside cabinet to get some tablets out of his bedside container.  He took a drink.  As he sipped his water he felt his wife stir beside him.  He didn’t want to wake her because he knew it would worry her.
After he had swallowed the tablets, he gently sat up in bed.  He didn’t have much time, but he knew, if he took it easy and didn’t try to rush this, he would make it.
He stood up and, leaning on the edge of the bed he walked to the end and around the foot of the bed.  Not much further.  He would make it. 
Oh crap, he had to move faster or disaster would strike.
He got to the bathroom and sat on the toilet.  The relief of making it was great.  There had been times when he hadn’t.
He sat, thinking about the last 2 minutes.  If the tablets stayed down for an hour, just an hour, then he knew he would be ok. 
Then it hit.  That all too familiar feeling of nausea.  Time was not on his side.
Back in Sally’s bedroom she lay quiet.  Then it struck.  She looked at the clock and did a mental calculation.  3.30.  Bugger, it was only half an hour.  Not long enough.  And with that though she leaned over the side of the bed and threw up.
Deep down she knew what she had to do, but she didn’t think it was that bad.  She grabbed her phone, there should be someone in the world awake.  “If I can just talk to them” she thought, they would tell her there was nothing really wrong, and she could just stay quiet for the day.
She opened her phone, swiped her pattern, and hit the facebook icon.
“I think I need help.  Is anyone there?”
“yes, what’s wrong” came the typed response.
“I woke up with a headache half an hour ago.  I took 20mg, but I have just thrown them up.”
“Do you have an emergency injection?”  Sally was focusing hard on what the person talking to her was saying but she was having trouble reading the words properly.  She couldn’t work out who was talking, and it didn’t matter.  She was just grateful someone was there to answer her.
“No, they won’t give me one.”
“How long ago did you take the 20?”
“Abot hald an hour.”  Sally was having trouble typing.
With that Sally dropped the phone and sped to the bathroom.  She just managed to get her head in the bowl of the toilet before she threw up again.
Dave was sitting on the toilet of his ensuite wondering if he was going to need to throw up, or if he could get back to bed.
He stood up.  The room moved around him as he fell against the wall.  This was going to be a good one.
He got a bowl from the bathroom cabinet where Jane, his wife had insisted they keep one, and took it back to bed.
So far so good, the vomiting wasn’t starting, and there wasn’t the urgency of the toilet needs.
He half lay, half sat up in bed.
“Are you ok?” Jane rolled over to face him.
“Yes, I don’t feel great, but I will be ok.”  He lied.
“Have you taken any more HC?”
“Yes, I just took 10mg.”
“Well make sure you still take your waking dose in half an hour.”  And with that Jane rolled over.  She knew Dave would be OK as long as he had taken extra meds.  He hadn’t thrown up, so there was no need to panic.
She also knew she would not bet any more sleep tonight as she would be listening for all the signs.
“Sally, how are you?”  Another group member had joined the conversation.
“I’ve just thrown up again.”
“You need to go to ER.”  The new group member said it.  Sara didn’t want to read it.
“I can’t.  I know what will happen.  They will leave me waiting for hours, and will ignore me.  If I bed for steroids they will say I am drug seeking.  They always do.”
“Sally, do you have someone who can advocate for you?”
“No, I will have to get a cab and go alone.  But I can’t go.  They won’t treat me.  An idiot Dr wrote into my records that I had Munchausen’s!”
“Shit. No wonder you are scared to go.  Do you have any evidence on you that you can take, to prove you don’t have it, but that you have Addison’s?”
“I had doctor who knew my issues who wrote a letter I keep in the file !…He stated not only do I not have Munchausen’s, I have all the positive test results proving my diagnoses! I cannot tell you how many times I’ve had to deal with poorly trained er drs and nurses who simply thought the was in my head, or that I was anxious!”  Sally was remembering the letter she had got written 3 months before.  Would it help?
“Please take that, get a cab, or call an ambulance, and get to hospital.  You are going into crisis.”
 Dave came back to bed,  He had just had a second trip to the toilet, this time he had gone at both ends at once.  It was 6am and he felt very dizzy, new he would continue to throw up.
“Can you take me to hospital.  I need fluids, and to find out why I am so sick.”
Jane was already dressed.  “Where’s your bag, I need to inject you before we go”.
She got out the solu cortef and began drawing up the meds.  “Don’t worry about getting dressed, just put a dressing gown on.”
“Sit here while I get everything ready.  Do you think you will be ok with the injection? “
“Yes, just get me to hospital.”
With that Dave lay down and closed his eyes.  Jane took the injection and jabbed his leg, slowly injecting the steroids, praying it would keep him ok till he got to hospital.
Sally picked up the phone to call a cab but realised she just didn’t have the energy.  She needed to go to the bathroom again.
It seemed to be a long way this time.  The apartment was small, but she could hardly move.  Leaning against the wall she slowly made her way.
All of a sudden the walls began spinning, the floor started moving out from under her feet and she couldn’t stand.
It all seemed to be happening to someone else.  The pain was intense, it was like a vice grip was around her chest.  She started having trouble breathing.
“Dear God, please let Judith stop in on her way to work, and find me.”  All Sally could think was that Judith, her sister, was due to call in in an hour, to drop off some paperwork.
Sally lay there, wishing she had had the courage to go to ER, but knowing that there would have been no point, as they never believed her anyway.  She slowly felt herself going to sleep on the cold bathroom floor.
Sally’s life slowly slipped from her body.  It would later be found that she had had a heart attack due to extremely high levels of potassium, and dangerously low sodium.  Unfortunately, this was a very text book adrenal crisis, however, the Dr’s would fail Sally again, as the wrote the cause of death on her death certificate as “Myocardial Infarction”.   There would be no mention of the cause being an Adrenal Crisis.
Dave and Jane arrived at the emergency room.  Jane went to check Dave in and struck a nurse who clearly had no idea what an Adrenal Crisis was.    She knew what was coming.  She knew that this nurse thought she was being over dramatic.
“Please calm down ma’am, he’s not dying” was the stock standard response the Triage nurse gave her when she tried explaining the crisis.
This didn’t surprise Jane as she had heard it all before, the last one was  “modern medicine is just failing you. I’m sorry but go home and both of you get some rest “.
She knew what she had to do, and did it.  Right in the middle of ER she threw a fit.  She demanded a Dr look at him immediately, or the nurse had to sign the letter she was holding, stating she was refusing to treat her husbands adrenal crisis as a medical emergency.
She had used this letter once before, and just like that time, it had the desired effect.
Dave was taken to a treatment area, given IV fluids, and asked if he needed more steroids.
“He will need at least two bags of fluids, and another 100mg solu cortef in about 4 hours.”  Great, I will chart it came the response from the Dr.
He had never seen an adrenal crisis, but he had been told by the nurse that this woman was serious, was very knowledgeable about her husbands condition, and was not going to take no for an answer.
He had met people like this before.  People with a life threatening chronic illness.  They knew more than the Dr’s about their conditions, and they knew what treatment they needed.  He loved patients like this because he didn’t have to guess, and get it wrong.
Jane was happy.  “Finally, a Dr that listens to his patients, and gets chronic illness.”  She said this to Dave after the Dr left the room.
Dave left the hospital 8 hours later, having had anti nausea meds, 300mg Solu Cortef, and 2 bags of fluids.  He also had a script for antibiotics for the infection that was brewing (according to his blood tests).  They didn’t know where the infection was, but the Dr didn’t want to take chances.
The Dr had seen someone die once during his training because his supervisor hadn’t listened to the chronically ill patient, had disbelieved what was required, and provided a course of treatment that was wrong.  That was never going to happen again on his watch.
Both times he was in crisis and I pretty much had to throw a fit to get him treated.
________________

The people in this short story are not real.  But the stories of their treatment are.  Also the fact you can have a heart attack or stroke because of an adrenal crisis are is real.

There are also stories of people being refused steroids because they are drug seeking, but offered barbiturate pain killers, so they will hurry up and leave the ED.  They are then told they are depressed, anxious, or just nuts, if they refuse the pain medication and ask for life saving steroids instead.

For many, telling a Dr you need steroids makes you a drug seeker.  It is all to common.

Many people, mostly women, before diagnosis, are told they are neurotic, psychotic, depressed, suffering anxiety and in one case I have heard of, been sectioned.

Adrenal Insufficiency can alter the thoughts, and mental capacity of the sufferer.  They don’t always make sense, they don’t always understand what is being said.  Dr’s in ED’s don’t have time to think, they only have time to act on what they see.  And they see a drug seeking nut job, not someone who is critically ill.

­Once these labels are on your medical file, it is extremely hard to take it off.  If you live in a small town and you get the same ambulance officers each time, you will find their treatment the same.  And if you have family that also believe this, it makes it harder to get the right treatment.

There are many people who will not go to hospital when in adrenal crisis because they are so tired of being labled, and left.

I don’t have an answer for anyone.  It is a change that needs to come from the Medical side, not the patients side.

Bruno Allolio, is Professor of Medicine at the University of Würzburg comments:

“As most physicians very rarely encounter an adrenal crisis, they frequently fail to act adequately. There are numerous reports from our patients and others that physicians ignored emergency cards or failed to give parenteral hydrocortisone despite the patient presenting the emergency kit. Thus, I occasionally have given my patients a written text to be signed by any such future emergency physician. It states that the patient has communicated the presence of impending crisis, has shown the emergency card, and the emergency kit. By signature, the physician would thus attest that this evidence was presented, thereby documenting malpractice, if refusing to administer glucocorticoids. I reasoned that asking for the signature would greatly facilitate hydrocortisone administration.

As a consequence of these frequently encountered problems, the essential principle of crisis prevention can be stated as follows:

The well-informed patient (or his/her relative) guides the poorly informed health-care professional!“
If you are a Dr and reading my blog, think about how many patients have you had where you have decided the symptom they have must be anxiety or depression, because they are not presenting in the normal way.

If you have a patient present with a “pseudo seizure” how often do you decide that it means a seizure caused by a mental problem, rather than “caused by something other than an electrical misfiring of the brain, but NOT a mental issue?”

Pseudo seizures have been suffered by a number of AI patients who, because of them, are treated as if they are faking their symptoms.  They are not, there simply isn’t any medical research into why these seizures happen.

Chronically Ill patients should never be afraid to go to hospital (where they should feel safe) because of seriously poor treatment by the medical profession.

According to www.ncbi.nlm.nih.gov

Which drugs do people seek?

Benzodiazepines and opioids are the two most common classes associated with drug-seeking behaviour. Opioids commonly misused in Australia include oxycodone, fentanyl, codeine and morphine.

Psychotropic drugs producing stimulant effects, euphoria, sedation or hallucinatory effects are sometimes sought. These include the newer antipsychotics quetiapine and olanzapine, and stimulants such as dexamphetamine and methylphenidate.2 Anabolic steroids are also increasingly misused.

Anabolic steroids are NOT the steroids needed in adrenal crisis, however, as soon as you say steroids, it is assumed this is what you are asking for, and therefore, you must be drug seeking.

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).

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!

But What Cost, a Medical Misadventure

When the Medical Side is Under Control

4 years on from Derek twice having his warfarin stopped by a hospital who’s excuse is “Oh, the Dr didn’t realise the importance of bridging” and we have, with very little help from them, managed to get Derek as stable as he will ever be.

 

We have got his Adrenal Insufficiency as under control as it will ever be.  We have worked out through experiement, what dosing works for him (and it’s not what they say in the books).  We have added other replacement medication (DHEA, Vit D) which is helping.  We are learning the symptoms of low cortisol.  We should have learned, we see them frequently.  Derek updoses as he needs, which is different to stress dosing when sick, and how to stress dose (double or triple HC) when he IS sick.

He is being driven to work, although he works mostly from home at the moment.

But what is the unseen cost of the medical fraturnity almost killing him?

It’s the costs they don’t compensate you for.  It’s the cost of things he can’t do any more. The things we now need to pay to get done.  For some it is things like having to employ a builder, for others, it is having to downsize their home, pay a cleaner, pay a gardener, or just not do things any more.

Why is employing a builder a hidden cost of his injury?

Bedroom RenovationBefore Derek suffered his CAPS event we used to work together to do renovations to the house.  This included turning our downstairs bedroom, shower room, and toilet, into a Master Bedroom with ensuite.

The cost of this exercise was extremely low.  We paid just for the materials used.  We paid a very small amount to have 2 power points moved.  We worked weekends, nights, and took a few days off work.

Top Left is the entrance foyer of our home.  You can see the door to the shower room, and the door to the downstairs toilet.  The bedroom is the door to the right.

Derek built the new wall (back of new wardrobe).  He then demolished the old wardrobe, we gutted the room, insulated, relined, and created a new Master Bedroom.

This was done a year before CAPS.

We have done a couple of small outside projects, with a lot of help from others including building a garden and a garden shed  and an deck outside the dining room.

Derek designed the deck, but we had to call on friends and family to build it.  It is an amazing asset,  and again, we paid for materials, and food for workers.  The help to build these things was fantastic.  But what about the bigger projects. Something the size of the Master Bedroom.   Like a kitchen?  Or insulating the Living Room?

We are looking at selling our home in a few years and find something slightly smaller.  There is a lot of new building going on around our area and we have to have our home at the right level to get the right money.  That means insulation in the Living Room is essential, as is a new kitchen.

Once upon a time we wouldn’t have thought anything of it.  We would have just purchased the materials, contracted a kitchen manufacturer to make the cabinets and bench top and over a period of a couple of months, done the work.  But not now.  And it was too big a job to ask friends to spend 2 days building.

So we had to pay a builder.  He is a great builder, with home renovations/alterations as his specialty, but still, we had to pay.  We have had to pay him for a lot of the other “little” jobs around as well.  Things that would normally take a weekend for Derek and I to do, now either doesn’t happen, or Chris comes to our rescue, but at a price.

So, Chris came in and gutted the lounge.  He then built a new ceiling, insulated the entire lounge, relined it.  We had to get a plasterer in to paint.  We had to get a painter in to paint it.

As for the kitchen.  It is a great Kitchen, I love it.  But again, we kept Chris employed where we could would once have done much of the work.

Gutted KitchenWith the help of a friend we did remove the old kitchen.

Most of the kitchen was still in good enough shape to be used elsewhere.  It was after all, a Designer kitchen.  It just wasn’t a well designed kitchen.

Our friend agreed to remove it with her and I doing the bulk of the work including carrying the units down to her trailer, taking them to her place, and putting them in her garage, for her to have installed in her home.  Derek did the little bits.  He unscrewed units from each other, and supervised, or held things while Sian and I did the hard labour.  Thank the Lord for Sian.  She has a new kitchen and Derek didn’t end up overdoing things.

But once everything was removed the walls were a complete mess.  Patches here and there.  Holes in the wall.  No insulation,  It had to be gutted.  We also needed to install a new window as one of the windows was sitting below the height of our new bench.

 

rebuilding

Chris, working hard (instead of Derek)

Enter Chris again. It wasn’t a big room.  For someone fit and healthy, which Chris is, it was a 5 day job to put up new ceilings, new wall lining, and install the new window.  Working along with the plumber and electrician it took 2 weeks in total.  Inbetween that, we had the kitchen company come in and install the units and bench top.

Then Derek and I could do some more work.  Well I could do some more work.  Derek “supervised”.  I plastered, sanded, and painted.  Derek did bits and pieces, working as he could, taking extra HC as he needed.  It took several days, with me doing the bulk of the work.  It was hard work, but rewarding.

Once we had the units installed it was time to put up the tile spashback behind the sink, and along the bench top.  Again, Derek cut a few tiles with a motorised tile cutter, and I did the installing of them.

KitchenFinished

The Finished Kitchen

All Up, for the living room and kitchen, it cost us $10,000 for work that 5 years ago we would have done ourselves.  This was the cost of employing a builder for work that Derek was once very capable of doing, and in a good timeframe.

So what is the hidden cost of a medical misadventure.  So far in the last 12 months it has added up to $10,000 and rising.  This, you do not get compensated for.

 

I understand that others would already have to get someone in for this, but when it is something you would have previously done yourself, it makes a difference. It certainly made a difference to the cost of the Kitchen.

For others it may be something different but there will always be hidden costs that are not compensated for, or that have no real measure and that are not thought about when it comes to the cost of a Chronic, or unexpected illness.