A Drug Seeking Nut Job?

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

According to www.ncbi.nlm.nih.gov

Which drugs do people seek?

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

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

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

International Travel with a Chronic Illness – 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”.

The Domino Effect

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

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

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

des-place

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

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

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

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

giftsforall

All the lovely gifts provided for the Adrenal Insufficiency Sufferers.

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

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

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

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

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

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

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

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

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

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

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

Back to our story.

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

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

And so it begins

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

The Domino’s are all in a line.

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

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

Des’ recollection:

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

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

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

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

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

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

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

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

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

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

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

 

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

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

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

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

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

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

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

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

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

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

Des found her phone headed off to call an ambulance.

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

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

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

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

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

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

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

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

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

Her recollection of this part of the event:

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

michelle

Michelle being stabalised before being taken off in the ambulance.

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

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

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

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

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

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

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

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

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

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

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

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

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

The treatment received were exact opposites of each other.

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

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

Things of Note from the Day of the Inaugural Crisis Meeting

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

How did this end:

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

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

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

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

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

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

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

Why did we not hesitate to inject?

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

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

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

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

Steroids are not evil if needed, they save lives.

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

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

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

 

A Presumptive Diagnosis

Aside

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

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

“WELL IN MY EXPERIENCE”

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

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

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

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

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

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

 Let me give you an example.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

On the morning of 17 Oct he had a CT.

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

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

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

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

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

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

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

“In my experience”

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

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

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

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

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

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

International Travel with a Chronic Illness

leaving-on-an-aircraft

Our Next Adventure Part 1

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

The Practice

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

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

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

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

Flying Further

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

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

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

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

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

Booking the ticket.

We have made the decision to travel over to Australia.

Now What?

Do we need to get permission from the Dr?

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

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

We bought the tickets. tickets

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

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

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

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

That would take more out of him.

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

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

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

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

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

Then there is Travel Insurance.

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

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

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

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

Medication

Emergency Kit

The Take Every Where Kit

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

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

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

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

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

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

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

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

Travel Advice from the Experts

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

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

So we missed the last one.

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

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

Now to wait for the actual trip.

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

Next Chapter – The trip.

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

 

 

 

WHY DR’S CAN GET IT WRONG!

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

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

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

So why is this important to my readers?

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

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

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

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

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

Why this book in particular?

Williams Textbook of Endocrinology claims, in their own words:

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

This is THE book that Endocrinologists go to for information.

But what happens when this book has failings?

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

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

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

What led me here?

Quite simply, a comment from Derek.

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

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

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

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

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

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

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

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

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

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

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

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

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

Here is where it affects those with AI

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

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

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

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

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

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

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

Let me show you.

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

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

Adrenal Steroid Replacement

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

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

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

However……….

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

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

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

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

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

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

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

Then we have Table 15-21

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

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

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

So Why the difference in Maintenance Treatment?

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

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

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

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

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

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

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

And then there’s the Emergency Injection

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

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

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

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

Changes over the years.

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

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

Added:

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

Added:

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

Added:

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

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

Added:

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

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

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

So where to from here?

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

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

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.

But What Cost, a Medical Misadventure

When the Medical Side is Under Control

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

 

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

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

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

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

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

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

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

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

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

This was done a year before CAPS.

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

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

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

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

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

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

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

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

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

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

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

 

rebuilding

Chris, working hard (instead of Derek)

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

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

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

KitchenFinished

The Finished Kitchen

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

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

 

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

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

 

 

 

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/

 

Did you Really Just Say That?

In the last couple of weeks I have been compiling a list of comments from Medical Practitioners to Addison’s disease patients.  I have called it “Did you really just say that”.
Unfortunately, although these are specific to Addison’s disease, it is not just Addison’s patients that this happens to.
I am sure many with Chronic Illness have heard similar comments about their conditions.

did you really just say that 

Please feel free to download it and read it.