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?

 

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!

UNSUNG CASULTY

I have had enough. I am tired, I am sick of this illness. I want OUT!

Please understand, I am not tired of my husband, I love him dearly, and wouldn’t want to be without him. Therefore I must accept this intruder into our lives, that will never leave, even when we take the welcome mat out from under his feet.

It is the Illness I am sick of. I am tired of it, I HATE it. But I am told I don’t have a right to say these things because I am not the one with it.

Actually, Yes I Am!

When I got married, I promised to love Derek, and to stay by his side in sickness and in health. And I will. But we are a partnership. When we married we put everything in OUR name. This includes this illness.

I claim it as much as Derek does, because I also live with it. Derek suffers bodily from it, but I suffer mentally from it. WE OWN IT EQUALLY.

Why am I saying this now?

Let me explain

One Friday morning Derek “crashed” about 11.00 am at work. He suddenly felt like he needed his meds. He didn’t take them, he decided to wait till his alarm went off. After all, it was only 30 minutes away.

At 11.30 it went off, but he was in the middle of something, so he doesn’t know if he took them.

30 minutes later he noticed his phone was flashing because he hadn’t recorded his meds. He said he couldn’t remember taking them, and felt “off”. The normal policy is, if you can’t remember taking your meds then take them again because even if you did take them, you probably need more. And too much won’t kill you, but not enough will.

He came home from work tired that night, but not sick. Just like had had a bad day. It got to 7.00 and I felt like I desperately needed to go to sleep. Because Derek wasn’t feeling great, we both went to bed. We also both fell asleep almost immediately.

Derek woke up Saturday with a bit of wind under his rib cage. Other than that, he felt tired, but not sick. He didn’t feel like he needed to take extra meds. We relaxed for the morning as I still wasn’t feeling great.

By the afternoon he still had the wind so we went for a walk. He considered taking extra meds, but decided that apart from this irritating wind which was causing a bit of breathlessness, he felt ok.

Sunday morning we woke and I asked him how he slept. Not good he said. He couldn’t lay on his right side, it was too painful. We decided then that we should possibly go to A&E, as now he was starting to feel more unwell (not sick, just unwell). It didn’t appear serious, it was just pain, so we felt we could take our time.

Derek went to have a shower, while I did a few things around the house. I went check how he was just as he was finishing his shower and saw him pull up in serious pain. He had reached up, and got a very sharp pain under the ribs moving up over the shoulder.

We phoned a Medical Help Line to see if we were worrying over nothing. I spoke to the nurse as by this time Derek was not feeling well at all.

The nurse asked if we had an emergency ambulance alarm. I could sense she was about to say she would call him an ambulance. We didn’t feel he was that sick so told her that we could just take him to Hospital. We actively live as close to the hospital, as the nearest Ambulance station, so it was quicker for us to drive.

We still didn’t think that Derek was that sick. He seemed a little low because of the pain, but didn’t think there was a need for extra Hydrocortisone.

We arrived at A&E at 9.49 am. We went straight to triage and spoke to the nurse.   We explained what Derek’s symptoms were, and explained that with Addison’s he could very quickly go down hill. We didn’t give Derek extra meds at this point because we didn’t want to mask his symptoms, and we felt that as soon as they spoke to him in a cubical, they would give him emergency cover.

A&E have a system (quoted from their website):

  • Triage 1: life-threatening e.g. heart attack. Treatment is immediate.
  • Triage 2: emergency e.g. chest pain, shoulder dislocation etc. We aim to treat these patients within 10 minutes.
  • Triage 3: urgent e.g. bad injuries, appendicitis etc. We aim to treat these patients within 30 minutes.
  • Triage 4: semi-urgent e.g. fractures etc. We aim to treat these patients within 60 minutes.
  • Triage 5: non-urgent e.g. minor strains or sprains which could be treated by a GP/primary healthcare organisation. We aim to treat these patients within two hours.

I will say here that what they say on their website and what is truth are completely different. When you get their they tell you they are required to make sure all people are “Treated and Streated” within 6 hours. In fact, it can take up to 3 hours just to get to see someone other than the triage nurse.

Derek presented with a life threatening medical condition, and unexplained chest pain. He was classed as Triage 3.

I let the nurse know I was not happy with this, as although he was awake, semi alert and talking, he had Addison’s and was unwell. I pointed out twice that this could turn into a crisis without notice, and was she happy having me inject him with emergency medication in front of everyone because they weren’t treating him with the correct urgency.

It didn’t make a difference. By the time we left the triage area, Derek had worsened.

After 10 minutes sitting in the waiting room Derek started going down hill. He could not keep his eyes open, he was starting to feel nauseous, he had a really bad headache, he had become sensitive to light and noise, he felt weak and was starting to lose focus of what was happening around him. Basically a number of the “signs and symptoms of an adrenal crisis”.

The Triage Nurse could see him going down hill, she even came over and asked if he wanted it slightly darker where we were sitting. He was sitting there propped up against the wall, with his legs on my knees and his eyes closed. He didn’t speak to her because of how he felt.

 

After what seemed a very long time (almost an hour I think) we finally got taken out the back and he had to try and repeat his symptoms. By now I was having to do most of the talking as Derek continued to go down hill.

 

The Dr came in a few minutes later. It was now 11.00. 1hr 10 minutes since presenting at Triage.

 

Derek was going down fast and still no offer or suggestion of emergency meds. I turned to Derek and said I thought he needed HC early as he could no longer focus, didn’t acknowledge properly what was going on, basically, he was crashing and was now in crisis.

 

I deliberately pulled his medical box out in front of the Dr and asked Derek did he want 20mg instead of the normal 5 he was due. He couldn’t decide (confusion starting to kick in). He told me later that all he could do was say yes, because anything else was too hard.  I made a drama of it to see what the Dr would do. She simply said “if you feel he needs that much, then give it to him”.

 

What? He needs a dam site more than that.

 

The Dr ordered blood work and an xray, then left. I started talking to the nurse about Adrenal Insufficiency. She admitted she had never seen anyone with it before.

 

Looking at Derek I could see he was now seriously in crisis and it was getting worse by the minute.  I pulled out my magic bullet. I had a letter, signed by one of the senior Emergency Dr’s at this exact hospital, stating what was required.

 

The letter was given to us for use outside our own Health Area. It stated what He, as an ED Doctor, would want to know in the case of an emergency.

 

This was now an emergency.

 

I gave the letter to the nurse. She stopped what she was doing (about to draw bloods). She read the letter, recognised the signature and said she would be right back, she was giving the letter to the Dr.

 

She came back in less than 5 minutes with 200mg HC IV.   YAY. But I shouldn’t have had to give them the letter.

 

The nurse then drew the bloods, Derek went for his Xray, and they did a heart trace. They also finally did his BP.

 

The nurse had also been given instructions to do a lay down, stand up BP to note any change.

 

The Xray came back clear, the heart trace came back clear, the bloods came back “normal”. I asked the Dr what the bloods actually were, she said “they were normal”. No, I wanted to know what the levels of sodium and potassium were. She said “normal”. I asked for a copy of the bloods because I knew what was normal for Derek, and what was normal for him, with Addison’s was different to that of healthy people.

 

She then told me his Creatinine was fine. I asked what it was because I wanted to compare it to previous Creatinine levels.

 

Well you could have blown me away with what she then told me. She had compared it to what it was in February 2013, and because it was similar to that, even though it was too high, she was happy because it wasn’t worse!

 

Feb 2013? Seriously? I asked her the number. She finally told me it was 115. This makes an eGFR (clearance in the kidneys) of 61.

 

I tried to tell her that that number was way off, but she wouldn’t listen to me.

 

In the last 18 months, his Createnine was 103-105, which gave him an eGFR of 67-69. To suddenly be down at 61 meant his kidneys were not working properly. It is most commonly caused by dehydration!

 

Good Practice with an Addisonian Crisis is to hydrate. Addison’s causes dehydration due to salt wasting, especially when sick.  It doesn’t matter how much water you drink, in fact drinking copious quantities of water can make you more dehydrated. He was meant to be getting fluids, but he was never given any.

 

She still wouldn’t listen to me. They couldn’t find a reason for him going into crisis, but were surprised at how good he looked 20 minutes after the 200 HC. When I say good, he was able to open his eyes and speak again. He still felt like crap.

 

They kept him in hospital over night to observe him, but were not giving him any more high dose meds. Nor did they give him fluids. He was to take 10mg HC at 5 pm, and hoped that would keep him going over night.

 

Day 2 at hospital and I arrived in time to meet with the Dr’s. I found several things of interest in this meeting:

 

  1. There WAS something on the chest. It was pneumonia (which is what we thought).
  2. He was dehydrated, even though he was drinking water. It was agreed that he SHOULD have been given fluids when he arrived at ED along with the 200mg HC IV Stat.
  3. We mentioned that last time Derek was in hospital the Dr who was training the students another day told them that the main cause of AI was TB. This Dr’s response was very immediate, and was a very curse “in 3rd world countries perhaps”. I told him that it was a Dr at that hospital that said it, he turned round and told the students in no uncertain terms that that information was incorrect.

 

Two of the students were told they should come back to talk to Derek because he is probably the rarest patient they will ever come across in their medical career.

 

You see from this that it is a shared illness. When Derek suffers, I suffer. We are in this together.

 

So yes, I should be allowed to say, I Hate this Disease, I hate what it is doing to US, I hate what it has robbed from US.

We will both live to fight another day.  I am now more determined than ever to get awareness of this illness out there.  to make sure others don’t have to go through this in our country.  Processes need to be put in place to make sure those with Chronic Illness get the appropriate help they need it, without having to “shout the roof down”.  We all need to be able to trust in our health system, that they will know what to do when we rock up to A & E.

Emergency Kit Contents

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I have been asked by several people, what is in Derek’s emergency kit.

He has several, but the main one is his Take Everywhere Kit.  He doesn’t leave home without it (even to the supermarket 5 mins away) at the moment because of earthquakes (we have had a large number recently).

I got a metal makeup box (Thin Lizy for those that know the brand) and made a kit up, sealing the top so it doesn’t come off.  I then bought him a “man bag” for Christmas so he could carry it.
His contains:
100mg Solu-Cortef
Syringes
swabs
gloves (for medics who use the kit)
20mg Hydro (small container of)
5mg Hydro (he uses this kit at work, or if we are out)
2.5 Hydro
his Warfarin (if we are out for dinner he doesn’t have to remember to take it)

Vitamin K (in case of excess bleeding because of Warfarin)
Anti Nausia tablets
Panadole
Hand Cleanser
Sterile Pad in case of injury.

Only thing he doesn’t carry daily is his Florinef, but that will go with us in a small chiller.
He then has a similar kit at work in his Emergency Incident kit under his desk, and we have a spare at home in our Emergency “Get out of Dodge quick” Survival Pack (we live in an area prone to earthquakes).

Emergency Kit

The Take Every Where Kit

Emergency Kit

Contents of his Kit