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.

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International Travel with a Chronic Illness – Part 2

Australian Triip – Day 1-3

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

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

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

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

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

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

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

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

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

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

We were ready for our trip.

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

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

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

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

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

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

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

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

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

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

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

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

Fast Track to a Holiday

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

“I don’t know.   Who?”

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

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

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

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

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

Let the Games Begin

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

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

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

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

des-place

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

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

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

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

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

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

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

Read about “The Domino Effect” here.

the Domino Effect

The Domino Effect

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

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

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

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

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

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

The Dr came out to reception “Derek?”

We got up and followed her into her consulting rooms.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Medical Incompetence is International

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

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

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

“So what is this number?”

“I have no idea.”

“Date of birth.  Is it ######”

“No.”

“Phone Number:  Is it ########”

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

“Dale ####”.

“That’s not Dereks form.”

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

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

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

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

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

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

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

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

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

jubblyelainejo

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

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

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

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

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

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

 

 

 

 

 

 

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

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

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

After saying a gre

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

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

A Presumptive Diagnosis

Aside

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

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

“WELL IN MY EXPERIENCE”

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

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

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

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

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

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

 Let me give you an example.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

On the morning of 17 Oct he had a CT.

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

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

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

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

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

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

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

“In my experience”

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

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

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

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

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

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

International Travel with a Chronic Illness

leaving-on-an-aircraft

Our Next Adventure Part 1

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

The Practice

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

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

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

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

Flying Further

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

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

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

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

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

Booking the ticket.

We have made the decision to travel over to Australia.

Now What?

Do we need to get permission from the Dr?

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

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

We bought the tickets. tickets

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

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

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

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

That would take more out of him.

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

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

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

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

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

Then there is Travel Insurance.

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

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

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

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

Medication

Emergency Kit

The Take Every Where Kit

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

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

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

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

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

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

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

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

Travel Advice from the Experts

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

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

So we missed the last one.

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

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

Now to wait for the actual trip.

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

Next Chapter – The trip.

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

 

 

 

WHY DR’S CAN GET IT WRONG!

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

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

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

So why is this important to my readers?

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

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

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

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

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

Why this book in particular?

Williams Textbook of Endocrinology claims, in their own words:

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

This is THE book that Endocrinologists go to for information.

But what happens when this book has failings?

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

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

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

What led me here?

Quite simply, a comment from Derek.

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

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

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

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

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

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

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

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

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

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

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

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

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

Here is where it affects those with AI

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

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

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

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

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

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

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

Let me show you.

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

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

Adrenal Steroid Replacement

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

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

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

However……….

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

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

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

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

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

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

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

Then we have Table 15-21

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

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

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

So Why the difference in Maintenance Treatment?

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

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

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

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

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

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

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

And then there’s the Emergency Injection

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

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

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

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

Changes over the years.

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

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

Added:

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

Added:

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

Added:

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

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

Added:

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

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

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

So where to from here?

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

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

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.