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

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

 

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Now you know it, forget it!

That is the Mantra of the Lecturers to Medical Student’s after being told about rare diseases.

I was talking to a Medical Student (just completed her 5th year) while I was with someone at the Emergency Department of a hospital recently.

We began talking about Chronic Illnesses and the fact that the families of those with Chronic Illness know more than many Dr’s about their particular condition.

She asked me about Derek’s illnesses…. “do you mind me asking what he has?”

No, I did not mind her asking. I am all for education.

I told her Antiphospholipid Syndrome. “Oh” she says, “I have heard of that”.

To say I was very surprised would be accurate.

I then asked her how she had heard about it.

Apparently, they were told about it in a Gynecology Lecture, had to learn about it in relation to pregnancy, and had a question on it in their 5th yr Exam.

They were then promptly told to forget about it, as they would never meet anyone with it.

She was not aware that Men could get it, or that it was something that can and does occur outside pregnancy!

Given that I know at least 4 women with it, and Derek has it, it isn’t that rare.

She knew nothing about the Catastrophic variant  or even that such a beast existed.

This is not the first time I have heard this about rare conditions.

Every time we query a Junior Dr or fully trained Nurse they say the same thing about Addison’s Disease/Adrenal Insufficiency. “We are told it exists, what the symptoms are, then told “but don’t worry, it is so rare you will never meet anyone with it”.  They are then shocked to hear about Derek.

If he is in hospital, the Medical Students are told to go see him, they will never meet anyone like him again!

No, they won’t. Because if they have a patient with either of Derek’s conditions, the patient will probably die before they are diagnosed.

Then there are those patients who suspect they may have a rare chronic illness is who are frequently (almost always) told “No, you can’t have that, it is too rare!”

Another useful thing all Medical Students are taught is: “if you hear hooves, look for horses, not Zebras.”

Just because a condition is rare does not mean it doesn’t exist.  It also does not mean that a patient is imagining things.

So what does this mean?  It simply means you look for Zebra’s if the Horses aren’t in the room.

How can you rely on the Medical Fraternity, when they so often get it wrong.

That is not to say they should know everything. What they should be doing is listening to their patients. Don’t rule out things just because they are Rare, or not “normally” found in that sex.

Listen carefully to your patients. If what you think is wrong comes back negative, give your patient the benefit of the doubt. Do the basic test to see if it IS  a “Zebra in the Room”. If you want to be arrogant, do the test to prove your patient wrong.

What is the worst that can happen?  The Patient is right, and you have to apologize.

Heaven forbid a Dr should find out they are not GOD!

I will take care that they suffer no hurt or damage.

The Hippocratic Oath is an ethical code attributed to the ancient Greek physician Hippocrates, adopted as a guide to conduct by the medical profession throughout the ages and still used in the graduation ceremonies of many medical schools.

The Editors of Encyclopædia Britannica Last Updated 11-9-2014

hippocraticoathThe part of the oath that people still quote is:

“With regard to healing the sick, I will devise and order for them the best diet, according to my judgment and means; and I will take care that they suffer no hurt or damage.”

It has been turned into a simple line “FIRST DO NO HARM”.

Breaking the Oath

Our belief is that Dr’s do their best to keep us well, make us well, or treat our ills. But even if they can’t, they will not do anything that could cause us harm.

We trust they will do “everything within their knowledge and skills” to follow these rules.

We have seen what happens when they don’t.  Read A Catastrophic Event

What if they have the knowledge, they have the information, they have the people trained to deal with an illness, to ensure they “do no harm”, but they fail in the very basics of actually utilising the knowledge and skills at their disposal, and they do harm?

In an previous post I talked about the fact Wellington’s Capital & Coast District Health Board (CCDHB), failed us by not “bridging” Derek with Clexane when the stopped his warfarin for surgery. While investigating what happened we found out thay actually had documents that had a plan written down for people with Thrombocytopenia (low Platelet count) and a blood clotting disorder.

This plan required that if they ever stopped his warfarin, he should be put on Clexane, to prevent clotting. Unfortunately we didn’t know this.

Why didn’t we know?  Because our knowledge was based on previous behaviours by CCDHB.

CCDHB Did Harm Twice

CCDHB’s Guidelines for Therapeutic Anticoagulation Management (TAM) states that Warfarin needs to be stopped for 5 days for invasive procedures. During this time you must be bridged with Clexane. Once the invasive procedure is completed, as long as you are not bleeding, you are then started back on Warfarin. You are kept on Clexane from stopping warfarin, to the point at which your INR is at a therapeutic level for you.

They don’t specifically mention a prostate biopsy in their TAM document, but they do mention procedures such as gastroscopy’s, endoscopies etc. The idea being that if it is “invasive”, i.e. taking a biopsy, where there is a risk of bleeding, Stop Warfarin, Bridge with Clexane, Restart Warfarin, stop Clexane.

We have now discovered that when Derek had his Biopsy in May 2012 CCDHB did not follow their own written procedures. They stopped Derek’s Warfarin. There was no Bridging Plan.

CCDHB’s Plan for Biopsy:

Stop Warfarin for 5 days before biopsy.
Have Biopsy.
2-3 Days after Biopsy, restart Warfarin, if bleeding has stopped.

Nowhere in the medical notes, is there any reference to Clexane.  Hence we were not surprised when Derek was told to again stop warfarin for surgery.

But Wait…. There’s More.

An important piece of information we have recently found out about Warfarin.

We always believed that the ONLY time Derek was put on Warfarin and Clexane at the same time was when he first suffered his DVT and it was to try and speed up the dissolving of the DVT. WRONG!

The reason you use clexane when starting on warfarin is because, when you first start warfarin, it actually works in reverse. Until you are therapeutic, the warfarin increases coagulation of your blood, until your body adjusts, which takes around 3 days but should always be checked with INR testing.   So, to prevent something going wrong i.e. a thrombotic storm, you are put on Clexane to keep you safe, i.e. Clexane is used to take care that they suffer no hurt or damage.

The key to all this is a thing called Thrombocytopenia. This is the term used when your Platelet count is chronically low. Derek’s Platelet count sits between 105 and 120 (Normal Range is 150-400).

Like many things, Derek wanted to know more about Platelet Counts, warfarin, Sepsis and CAPS, so started looking at medical sites and looking at his blood results (we keep the all). In doing this we discovered that, apart from Antiphospholipid Syndrome, there is another reason for low Platelets. It can be an indicator of Disseminated Intravascular Coagulation (DIVC). When you have APS, and you suffer DIVC, it is considered CAPS.

One thing that does NOT cause low Platelet Counts is Sepsis.

Back to the medical records. This time, we went to the Hutt Valley DHB records of when Derek presented at Hospital the day after Biopsy.

Remember, CCDHB did NOT bridge Derek for his Biopsy.

In Derek’s HVDHB medical records for the admission on the day after his biopsy (he was still not on any form of anticoagulation) it clearly states “Bloods Sent”, which means they had drawn blood to do a blood workup. This is routine practice in ED for diagnosing when someone presents as ill, rather than an accident.

One of the most basic blood tests done is a Compete Blood Count (CBC). This includes your platelet count. It also shows if you are fighting an infection of any type. It won’t tell you WHAT is wrong, but will tell you if SOMETHING is wrong.

When you suffer Sepsis, your Platelet count increases (150-400 is normal). So you would expect to see a CBC Platelet count of over 300. With Derek you would at least expect to see him IN range if he had Sepsis. In Oct 2012, when the Infectious Disease Registrar was contacted, he tried to convince people Derek had Sepsis. His platelet count of 125 would indicate otherwise, and at the time, Derek was suffering CAPS.  Inflammatory markers were up, but platelet count was down.

When looking at HVDHB’s notes What we found instead of the blood results was the next note on the HVDHB file for that day stating “Blood and Pro not sent to the lab. We can only presume that by this time it was decided to transfer him to CCDHB and as they were passing his care to another DHB they didn’t need the Bloods processed, and so destroyed them.

Derek was put in a Patient Transfer Ambulance and taken to CCDHB (different District Health Board, so different process etc).

After reading that the bloods were not done at HVDHB we had a look at the CCDHB admission records. The only mention of Bloods in the CCDHB’s notes wasFollow up Hutt Bloods. They never took any bloods of their own, ever.

They only took bloods for cultures. Because bloods for cultures are put in a special bottle, they cannot be used for a general CBC.  There is also no indication in his file, that they ever followed up with Hutt because if they had, they would have seen that Hutt destroyed their bloods.

CCDHB simply did cultures. And what, you ask, did the cultures show? NOTHING.

Based on no evidence, and having failed to do a basic blood panel, there was an ASSUMPTION of Sepsis. Not a PRESUMPTION. To be a presumption, you must base your belief on the weight of the evidence available. As there was no evidence of sepsis, and no bloods taken, they erroneously presumed Sepsis.

When he was re-admitted a week later because he was still very ill, they assumed again, based on no evidence, and no positive cultures, that it was still sepsis, and threw higher dose antibiotics at him.

The better presumption based on weight of evidence being Cessation of Warfarin, NO Bridging, an Invasive Procedure, followed by starting of warfarin again with no cover of clexane, would be a Mild CAPS event.

We have now had 2 separate, independent Dr’s suggest the same thing. To prove, or disprove either theory, all they needed to do was a CBC. However, this was something that was never done. Therefore a diagnosis must be based on weight of evidence, which leads to CAPS.

Not only did CCDHB try to kill Derek during Surgery, but also during his Biopsy. This is not acceptable practice and clearly fails the Oath of taking care that they suffer no hurt or damage”

It seems that the more we look into Derek’s medical care, the worse we realise his care has been.

Our goal at present is to  make the medical profession be held accountable for their lack of care and concern.  Ignorance is no excuse for almost killing Derek, not once, but TWICE.

Worst-Case Scenario

What if I had an accident, not bad enough to require an ambulance, but that required me to go to Hospital?

Since Derek became ill I have had a “worst-case scenario” played over and over in my head.

For two years, I have imagined what would happen if I became incapacitated. For some reason, I couldn’t drive. What sort of impact would that have on the fact that I was the only driver in the house.

I have played it over in my mind many times, thinking “what would I do”, “how would I deal with the problems”. “What if I had to get Derek or Bevan to a medical appointment, or I had to do grocery shopping”.  “What are all the problems I would have to deal with?”

Who could I call.

My worst-case scenario? To break my right leg at home.

 Actual Event

Stepping down off the back lawn onto the steps. I felt my ankle roll to the outside. My mind immediately went to the thought of, “NO, This can’t happen”. So I tried to correct. But to do that I had to put my left foot down on the step. Great, I will be fine.

Imagine my shock when I realised too late that the step I was aiming for wasn’t there. I didn’t stop with both feet on the same level.

I stopped when I was layed out flat on the ground, having landed very heavily on my left knee. It hurt, but not as much as my right foot. That was screaming.

But over riding it all was the thought, “Crap, Derek is going to get a headache, need to go to bed, and I think I should probably go to A&E”.

I lay there for a few minutes, running everything through my mind, tears running down my face. I wasn’t sure if I was crying because of the pain, or because my worst-case scenario might be coming true.

While there, I worked out that I have hurt my left knee. I knew that because I could feel the blood, and I could feel the hole in my jeans. I still hadn’t risked moving my right foot. It was still yellowing for attention, and I was refusing to give it.

The reaslisation that I was the only one with a Drivers Licence, or that could Drive dawned on everyone. Offers of “I’ll call and ambulance”, “Do you want me to call an ambulance?” and other phrases including the word ambulance were used.

No, this was not bad enough for an ambulance. I felt sure I could get to my bed, rest it, and then see what was happening.

All this time I was pumping adrenaline like crazy.

This is a good example of how fast Derek reacts to an emergency. He walked over to me at his normal speed, and just stood there asking if I was ok. Not able to actually react in a normal way. I could tell that, because all he did apart from asking me if I was ok, was to call Bevan, who was already running to my aid from inside the house.

I finally managed to sit up and reassess the situation. My foot hurt, my mind jumped to “crap, broken it”, then moved slightly more slowly to “nah, it’s just going to be a bad sprain”. Ice, rest, and then a trip to A&E to get it strapped, and find some crutches for a couple of days.

I worked out how to stand up, with assistance. I then tried weight bearing. Great, I can stand on my foot.

1 step, 2 steps, that was it. No more. I was NOT able to stand on my own two feet.

Somehow, in that short time, Bevan had come up, assessed the situation, realised I needed help, and went and found my mother-in-law’s walking stick. Well done that son. I hadn’t even noticed he had left before he was back.

Bevan and our Boarder helped me inside (with me sliding down the stairs from the top of our back yard) to my bed, I was given ice.

As I lay there I realised that, even though I knew my eldest son who lived at the other end of town needed to go to bed at a reasonable hour, and that I was about to spend 5-6 hours in A&E, I sucked in my pride, accepted it as a fait accompli, I phoned him and told him the situation. He said he would turn off his dinner (which was almost cooked) and come get me.

No, I wasn’t having that, I wasn’t going to inconvenience him that much for a sprained ankle.

I took pain killers and lay there for an hour waiting for him. At first it was fine, I didn’t mind waiting as I knew it was just a sprain. I had time to go through each plan of how to get to work the next day as spraining my ankle wold mean not being able to drive for 24-48 hours.   I would need crutches but that was it.

By the time my eldest arrived to take me to hospital I was desperate. I couldn’t walk, I was in a lot of pain, even with pain meds,  and shock was setting in.

Derek on the other hand, was laying there, he looked worse than flat but not as bad as he has been some times. He took panadol, and just deal with the headache as best he could.

I worked my way to the car and got in the passenger seat. My instructions to my son was, on arrival at hospital, get me a wheelchair. I was NOT going to try and walk.

We checked in, we could see it would be a 4 hour wait at least. What was a girl to do. Post on facebook of course, where my daughter read about it.

At some point my daughter and her boyfriend (a paramedic in training) turned up. This was a great opportunity to send Derek home. He had taken extra meds, but still looked worse than me.

Finally I am taken out the back and seen, very quickly, by a Dr. Quick exam, “yes, think we might get an x-ray”.

1 found of x-rays and a 5 minute wait later and I get the good news. “Yes, you have sprained the outside of your right foot. But I need another x-ray of it. A slightly different one. That spot you say is the extremely painful, is where you broke it! But there may be another break as well”

Boom!

There it was!

Worst-case scenario now a Reality!

I am cast. Both figuratively and literally.

Oh, and I have to get Bevan to a Medical Appointment tomorrow a long way from home!

Boom! There it is again.

For the next two weeks I will probably be in a plaster case, at which time I have to see an Orthopaedics Specialist to see what they want to do. Even if taken out of the cast and put in a moon boot, I will not be able to drive for another couple of weeks.

Up to 4 weeks of not having a driver in the house.

Yup. My worst scenario is now playing out in real life.