The Expert Patient

As far back as 2002 an article was written for the British Medical Journal encouraging patients to become “Expert Patients”.

Clin Med (Lond). 2002 May-Jun;2(3):227-9.
The expert patient: a new approach to chronic disease management for the twenty-first century.   Tattersall RL1.  Author information

Abstract
The expert patient: a new approach to chronic disease management for the twenty-first century, produced by the Department of Health, recommends the introduction of ‘user-led self management’ for chronic diseases to all areas of the NHS by 2007. The premise is that many patients are expert in managing their disease, and this could be used to encourage others to become ‘key decision makers in the treatment process’. Furthermore, these expert patients could ‘contribute their skills and insights for the further improvement of services’. It is hypothesised that self-management programmes could reduce the severity of symptoms and improve confidence, resourcefulness and self-efficacy. It is stressed that this is more than just patient education to improve compliance. Instead there should be ‘a cultural change…so that user-led self management can be fully valued and understood by healthcare professionals’. I point out that these ideas, while welcome, are not particularly new. Achieving the desired culture change will not be easy.

“Expert patient”—dream or nightmare?

BMJ 2004328 doi: https://doi.org/10.1136/bmj.328.7442.723 (Published 25 March 2004)Cite this as: BMJ 2004;328:723:

The concept of a well informed patient is welcome, but a new name is needed

Since the chief medical officer for England first introduced the term expert patient, it has been picked up and used very widely.1 During this time, the notion of the expert patient seems to have been criticised by doctors at least as much as it has been welcomed.2 If one asks lawyers, architects, social workers, or management consultants whether they prefer clients who take an interest in the issues they face and are motivated to work in partnership to achieve successful results, the answer seems obvious. So why does the idea of expert patients provoke such antipathy within the medical profession?

There is even a Training Course across England called the “The Expert Patient Program which states:

“Creative thinking is key for the Expert Patients Programme (EPP), a thriving Community Interest Company that gives people more control over their conditions through cognitive therapy courses.

It has made a particular difference to people with long-term illnesses, such as diabetes, arthritis or respiratory problems, by teaching them how to self manage and monitor their own symptoms. “It’s giving patients more control,” says Renata Drinkwater, Chief Executive (interim). “Courses like these are proven to make a difference. They boost confidence and, in some cases, can delay the onset of other conditions. They also have other benefits, like reducing the amount of times the patient needs to go into hospital or use Accident and Emergency.”

Conclusions
So, will the vision set out by Professor Donaldson and the Expert Patient’s Task Force work? The simple answer is ‘no’, unless there is a sea change in attitudes among patients and, more importantly, healthcare professionals.

What About You?

As someone with a chronic illness, would you consider yourself an “Expert Patient”?  Do you consider your Dr a Good Dr or a Bad Dr.  Is he bad because he has a history of harming patients, or a bad Dr because he doesn’t know your specific condition.

As a patient with a chronic illness you go to your Dr expecting a good level of knowledge, and that they keep their knowledge up to date.

You go to a specialist with the same expectation.

What happens though if the chronic illness you have is rare condition?  If your Dr or Specialist only has 1 patient with your condition, but over 1000 with a more common one.  Do you expect them to keep their knowledge up to date?

The reality is, if you have a rare condition, you can’t and shouldn’t expect them to be as up to date as you want, or to have the knowledge you would like, at least not when you are a new patient.  It’s different if you have had them for 2, 3, 4 or more years.

Instead, what you CAN expect, is to be listened to, to have them acknowledge that it is a learning process for you both, and that if you are able to provide relevant up to date information, they will accept it and not dismiss it because it wasn’t what they learned when they were training 20/30/40 years ago.

Yet this is also not always the case.  So instead you learn what you can about your condition, to fill the gaps and hope you can at least work around your Dr.

In truth, a patient goes to the specialist wanting help to improve their quality of life.  If you are lucky your specialist, knowing you are coming, has done a quick review of your medical notes, a quick read up of the condition.  But with that, they have decided, almost before you walk in the door, what treatments they will or will not offer.  If you ask a question they are not expecting, you take them off guard.  But they don’t want to appear like they don’t know the answer (which they probably don’t) so they either ignore the question, or give some answer they drag from the back blocks of their brain where they remember something they heard once years ago, whether right or wrong.

These are the Dr’s we hear about in the forums.  The ones that “don’t get it”.  We get frequent cries of “I’ve fired my endo.”  “My Dr is an Idiot.” “I can’t find a decent Dr who knows anything.”  And I can see why they say it.  I have heard the horror stories of medical appointments, ED/ER visits, lack of knowledge and but refusal to acknowledge it.  These Dr’s need training in your condition, but they are not getting it.  Why?  Is it lack of time?  Lack of interest?  Or maybe they don’t know where to start?

What would happen if, instead of leaving frustrated, angry, and ready to fire them, you sat down and tried talking to them as equals?

What if you opened up the consultation with a different tact.  If, instead of expecting them to know everything, or worse, you go in expecting to have a bad appointment with them knowing NOTHING,  you went in knowing they didn’t know anything, but were open to learning.

What would happen if you opened up the conversation with something like “I am struggling with my conditions, and with the general lack of knowledge and research available.   I don’t know how you Dr’s keep up to date when there are so many rare conditions out there.  I need to get a better quality of life, perhaps we could learn and work together on how to better manage my health.”

What would your Dr say?  You are not challenging their knowledge, but you are also not putting expectations on them to know it all.   If they take that bit well, perhaps go on with “Do you have any recent research I could read, or a website where I could go to get good up to date research on my condition so I can learn more about how to help myself”.

The worst that could happen is they dismiss you out of hand and go back to their personal ideas. If this happens you have two options, you can get upset and leave the consultation frustrated then go on line to rant.  Or you could ask them straight what their objection to having a knowledgeable patient is.

By now you have nothing to lose
if you have already decided to leave
and find a new Dr?

You might get a surprise.  You MIGHT get a Dr that is willing to work and learn with you.

The Expert Patient may not only need to be an expert in their condition, but also in negotiation.  A Win/Win negotiation is an art.  It requires giving the Dr something they really need (which may be stroking their ego) but also getting what you need, which is  a Dr willing to work with you.

Dr’s won’t learn, if they are not taught and quitting a Dr because he doesn’t already know simply means that the next patient will get what you have, a Dr that has neither the time or interest to learn.  You never know, you might awaken in that Specialist or Dr, a new interest in your rare condition.  Then he won’t be a Dr to run from, but a Dr to run TO.

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Doctor Shopping

According to Wikipedia “Dr Shopping” is done by patients trying to get prescriptions for pain killers or the like, from multiple Dr’s.  Dr’s believe this is also the only reason.

But it’s also a term used patients when they are dissatisfied with the care their current PCP/GP is giving them and they want either a Single Second Opinion, or you want a complete change of Dr, and they try several before they settle on one.

I recently heard about a Seinfeld episode (I didn’t watch it myself) where someone had been labeled “a difficult patient” and then “fired” by their Dr and tried to steal their medical records to change the “difficult patient” label because it was being seen by every Dr they went to.

When you Dr Shop for a better standard of care, what it is assumed is that you are looking for drugs, because, well, what else could it be?  Heaven forbid you might actually just want to get your life back on track, and have a better Quality of Life.

And once something negative is put on your medical file, anyone that reads it, will believe it, because it was written by a Dr, or Nurse, and they know you better then you do.  After all, they have seen you for at least 10 minutes.

Many patients with chronic illness have this problem.  One I heard of recently had the term “malingerer” put on their file, even though during that particular ER visit, they were admitted to hospital seriously ill for several days in adrenal crisis.

The process to have that single word removed from their file was going to be long and arduous.  And it was not guaranteed it would be removed.

These days people are realising that we all have a right to a certain standard of care, and to get that you sometimes have to shop for the right Dr for you.

Have YOU ever gone Dr shopping?

We have.  Our Dr, who we liked, retired and we had to find someone who could look after Derek’s complex situation.  We wanted someone who was willing to acknowledge what they didn’t know, and be willing to learn what they needed to, to help Derek live the most “normal” life he could.

We needed someone who would order tests if we asked, who would acknowledge tests that were not within Derek’s “normal” range, and who would suggest options, referrals, medications, to help, not hinder him.

That was hard.  Our Dr had gone on a 3 month sabbatical so we used the Locum, but found her to be a very basic, “if it’s not a cold, I don’t want to know” type Dr.  We then learned, because Derek needed to see a Dr, that ours was not coming back but had decided to retire.  We were offered the opportunity to stick with the temporary Dr in his practice or go to another Dr in the practice.  Neither of these was an option as we knew both and had found them lacking in both People skills, and willingness to work with the patient.  They would rather talk AT you than WITH you.  There was no way I would entrust Derek to their care.

We had also learned not to trust Doctors completely so finding one I was willing to trust was going to be hard.

I started thinking about this recently because of a news article I read about a new Dr’s practice opening up in a town which was suffering an extreme shortage of Dr’s.  The problem was, the Head Dr in the town said patients should NOT Dr shop.  She insisted you should stick with the Dr you had good or bad, and learn to work with them.  But there was no mention of the Dr learning to work with the patient.

When you have a chronic illness it is more important than ever that you have a Dr you trust, but also a Dr that knows the correct way to treat you.

In the social media groups, you are not supposed to Criticize doctors.  The reason for that became obvious to someone one day when their specialist told them he no longer wanted to treat them because they had heard what this particular patient had said in a closed group, about how she felt his care was.  It was also originally not allowed to recommend or “rate” your Dr.  Because of issues around recommending Medical Practitioners and litigation in some countries, it is also good practice to avoid support groups advising one Dr over another.

Now however, there a several “Recommended” lists for people to find the right specialist.  This works to a point, but you have to be able to get in to them.  You also still need a General Practitioner or Primary Care Practitioner.

This is where Dr shopping IS a good idea.  And this is what Derek and I proceeded to do.

A couple of years previous the local Medical Centre had been taken over, and renovated.  We were hearing good things about the new owner.

Since we were looking for a new GP, and the Medical Centre was, within walking distance for when Derek was feeling well, it was decided that we would check out the Doctors there.  Only I didn’t want just any Doctor.  I wanted one I could click with.

I had already been Dr shopping when looking for a family Dr years ago, when I found the one that is now retiring.  I had been to a number of Drs after we moved town, but hadn’t yet met one I “clicked” with, until that particular Dr.

This time I was doing it deliberately.  So I went in with a list of questions, and informed her immediately that I was there to see if I felt she was the right Dr for us.  Her books were closed, she was the 2nd in charge in the practice of 10 Doctors and it would be hard to get on to her books.

Some of the questions were:
1.  Are you willing to allow me full access to all test results?
2.  Are you willing to have patient led care?
3.  Are you happy being interviewed by a patient?

Derek is lucky.  The Doctor was very happy we were interviewing her.  And once we explained Derek’s medical conditions, and history, she understood that we were being careful about who we chose.

Once you find your Dr

Whoever you decide on, once you start to work together it needs to be a partnership.  It can’t be one sided, from either side.

A friend of mine has written a document for working with your Dr which is well worth a read.

Talking to your Doctor

by Des Rolph

When going to appointments be prepared. Take your medical history with you. If it is extensive, type it up making dot points, not long paragraphs. It is easier for them to read. Keeping your notes concise and to the point is key, no rambling on.

  1. Tell them that you would like to work with them in treating your problem and that you need to fully understand reasons behind treatment and what the expected outcomes should be.
  2. Ask if they have any documentation/information around their proposed treatment and your condition, or can they point you to websites that explain the condition.
  3. Initially go in with a list of symptoms and general questions rather than with your own thoughts on diagnosis. You can always do a bit of steering if they aren’t connecting with you, but things get missed if you try to lead too early. If you are not sure about anything, ask questions.
  4. Have a list of questions! Do not throw them together at the last minute, but jot them down in the weeks leading up to the appointment. Take two copies, one for the doctor, one for you to make notes. I have found some doctors write on your notes and hand them back to you.
  5. It is helpful to take someone with you to the appointment, so that they can take notes so you don’t miss anything. For some reason, having someone with you who can validate your symptoms can have a positive impact. It might be useful to have an extra copy of your list of questions for them as well.
  6. Keep a symptom diary. Jot down BP on waking and going to bed at minimum. It is helpful to have a glucose metre, and take note of your blood sugars before meals. Jot down any symptoms you experience and record your cortisone dose if you have updosed for the day. If you have pain, indicate out of 10 what level it was.
  7. Medication list – also break it down by time of day and dosages for each
  8. If you have multiple doctors and/or multiple conditions, be sure to note who prescribes each medication and/or what condition it is for.
  9. Include in your medication list, any supplements, over the counter medications and as-needed tablets, solutions, powders or remedies.

You have many things to weigh up, and make decisions about. If you have multiple issues, often one treatment can inversely affect something else, and we have to decide which is the most at risk.

Remember that they are working for you, and that the ideal relationship between you and your doctor should be open, honest, and equal. They should respect that you need to understand their decisions and why they are making them. Without understanding treatment, we can become anxious and not feel confident.

 

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.

The Domino Effect

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

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

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

des-place

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

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

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

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

giftsforall

All the lovely gifts provided for the Adrenal Insufficiency Sufferers.

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

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

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

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

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

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

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

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

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

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

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

Back to our story.

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

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

And so it begins

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

The Domino’s are all in a line.

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

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

Des’ recollection:

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

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

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

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

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

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

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

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

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

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

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

 

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

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

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

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

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

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

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

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

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

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

Des found her phone headed off to call an ambulance.

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

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

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

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

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

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

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

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

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

Her recollection of this part of the event:

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

michelle

Michelle being stabalised before being taken off in the ambulance.

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

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

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

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

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

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

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

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

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

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

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

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

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

The treatment received were exact opposites of each other.

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

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

Things of Note from the Day of the Inaugural Crisis Meeting

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

How did this end:

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

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

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

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

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

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

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

Why did we not hesitate to inject?

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

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

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

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

Steroids are not evil if needed, they save lives.

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

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

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

 

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!

To Get Bitter or To Get Better?

bitterorbetter

It’s Your Choice.

We are determined that for Derek, we will get Better.  To get Bitter would mean bad health, constant hospital visits, and probably the end of our marriage.

I have been reading back over the medical records of Derek’s Hospital stay, starting with his Diagnosis of Cancer in May 2012.

I have been reading everything with new eyes.  Not the eyes of someone investigating what went wrong.  Not the eyes of someone needing to prove failures in the system but with the eyes of someone who is reading for the first time, what actually happened to Derek.

It is bringing back interesting memories of sitting by his bed being told he is going to die, being told he was the 2nd worst off patient on the cardiac ward and they didn’t know why. As a side note, the worst patient was rushed to ICU an hour after Derek had been told that by the most Senior Dr on the ward.  Four years on it is finally safe to relive what happened without anger, fear, or distress.

Now it is what it is.  We have travelled the road of the mourning process you travel when you suffer a serious loss.  We have both come out the other side.  If you are able to, looking back to see how far you have come can be a good thing.  It’s something everyone should do when they have a major crisis in their lives.  But it must be “safe” to do it.  And for Derek and me now, it is safe.

Over the first 3.5 years I have read the medical notes trying to find specific things.  I missed a lot of what the nurses said in their daily reports.  I missed the real struggle Derek went through, how sick he was, and how bad he still was when he finally left hospital.

He will never be the man he was in 2011.  We have mourned that loss.  Now we are working on what is left. Learning what we can improve, and how we can improve it.  And just as importantly, learning to live with what can’t be improved by learning to work around it.

The biggest challenge was, and still is, trying to make the medical staff understand that we KNOW he won’t get better, but if we don’t know what is wrong, how we can work either with it, or around it.  There is a belief with some professionals that, if it can’t be fixed, you don’t need to know how bad it is, or sometimes even what it is.

The best example for Derek is that I began complaining while Derek was in hospital in 2012 that he had new issues with swallowing.  I told every medical professional we spoke to.   I became a cracked record, repeating the same thing over and over.  But it wasn’t until I got angry with the medical fraternity as a whole in 2015, that they finally did a swallow examination and discovered that he does indeed have dysphagia.  It is reported as mild, but also acknowledged that it will be worse when fatigued, or sick.  The problem is, one of his main symptoms is fatigue.  So we had to know.

It also turned out that the reason he would get throat infections and not know, was because he has no feeling on the left side of his throat.  Now that we do know, we have worked out what foods he can eat when he is feeling good, vs what foods he can’t eat when feeling tired.  It’s not about who’s at fault, or should they have done the investigations earlier.  It’s about keeping Derek safe because when he gets tired he chokes, and now we know why, we can work around it.

bitter or better

Derek and I came to that point where we had a choice.

We went for a second opinion in 2015.  We had ideas of what else we could do to try to improve his QOL.  We also wanted to try and keep him out of hospital.  He has come close a couple of times since 2014, but we have managed to keep him out.

How?  By talking to other sufferers of the condition, following the latest research, and learning to listen to his body.

By being proactive in his care, not leaving it to the medical world to fix or forget.

For 2 years we became like a stuck record on a lot of his symptoms.  The common question was BUT WHAT IS CAUSING IT?  Why did he always choke, why did he get so tired when taking the recommended replacement steroid doses, why did he get sick every time he suffered a fight or flight reaction (an adrenaline rush).  Why was he ending up in Hospital every time he got sick.

We know why he chokes now, we know that the text book answer for replacement steroids 3 times per day (an idea from 1973 which most Endo’s still follow) is wrong.  We know that when you get a fright you get an adrenaline rush.  But it also causes a call on cortisol to bring your body back from the fight or flight.  This is not something the Endo’s tell you when talking about “stress dosing for illness injury”.  We learned by trial and error.  We learned the difference between UP dosing, which is taking a little extra steroid for a one off situation and STRESS Dosing which is in the books and requires 2 or 3 times your daily dose for several days because of serious illness, injury, or stress like a family death.

So we have made changes to his dosing.  We have changed his eating, we have moved to 4 times a day with hydrocortisone, although we are considering 5.  The latest research out of England says maybe 6x/day and no more than 4 hrs apart would be ideal.  We have included DHEA, which is something most Endo’s don’t even consider for men, yet it is the most prevalent pre-hormone produced by the adrenals.

And we are getting on with life.  Medical appointments are now just annual routine follow ups where we tell the Dr’s what Derek is are currently doing, and how it is working.  We tell them what he has tried that hasn’t worked.

Otherwise he is at the GP’s because he is sick.  Sometimes we don’t know what is wrong, we just know that things are not right.

He still has problems.  As I write this he is sick and has been for 5 weeks, with what was for me a simple cold.  It lasted 5 days for me but for him it became a full on chest infection which he still hasn’t recovered from.

If Derek was working in the Office, he would have worked from home for most of that time, but because he was already working from home all week, he has been able to work for most of that time.  If he wasn’t able to work from home, he would have had to take sick leave for 2-3 weeks.

He has to take extra cortisol just so we can go out at night.  This is something not in the books.  It has been learned from trial and error.  The trial being taking it, the error being not taking it and ending up sick for days afterwards.  We have learned that Derek needs to rest the day after we go out, to recover.

He is not the man he was, but looking back on how sick he was 4 years ago and the major insult his body received, we realise how far he has come.

In those 4 years we have both changed and we both understand a lot more about both the medical condition and the lack of knowledge by the medical profession, for what is after all, considered a rare condition.

We have also learned the truth about the bogus condition that attracts all the “natural health professionals” that promote snake oil for Adrenal Fatigue.

We have learned people that inform us that Derek can stop steroids if he takes their snake oil, that the only way to shut them down is to shut them up with scientific fact.

Like anyone with a Chronic Illness Derek has good days, and bad days.  He has days when he can’t get out of bed, he has days when he can do things around the house.  And that’s fine, because after 4 years, that is what we know is the life of the Chronically Ill.

It doesn’t matter the illness.  If you learn about it, you can learn to live with it, and go from being Bitter to being Better.

Please note when I say BETTER, I don’t mean WELL.  I mean learning what your limits are, and learning to manage them so you can be the best your Chronic illness allows you to be.

For Want of an Emergency Injection!

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

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

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

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

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

Her Dr had previously said:

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

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

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

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

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

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

medical

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

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

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

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

 

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

 

aiunited

Enter a caption

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