The Dentist and the Dragon

The Dragon

As many of my readers know, we have a good medical system in New Zealand.  It’s good, but it’s not great.

We also have a very different system when it comes to injuries and medical misadventures.  For a start, in New Zealand we don’t call them Medical Misadventure.  Oh, it’s an adventure.  For some, like Derek, it’s the ride of your life.

But it is, like a broken leg, classed as an injury.  For Derek, it’s a “Treatment” injury.  He was injured because of medical treatment.  But that’s history.  Or should that be “His Story”.

When you suffer at the hands of the medical community, and are injured because of it, you get to have an assessment by the Government’s “Independent” “Insurance” Company.

Set up in the 1970’s, it was designed to stop any form of Litigation.  Stop people having to pay crazy amounts of insurance just in case they suffer an injury.  But like anything new, the concept and the reality can be vastly different.

This great independent organization, that was there for the injured, were under some very strict Insurance type rules.  And like any good insurance company, they change the rules as often they change their pants.  So, you have an injury you don’t know if you will be adequately covered.

When you read back through my blog you will see that Derek was covered for his Treatment Injury.  This injury is his Adrenal Insufficiency.  This impacts all parts of his life.

He had to give up driving because, although there is no obvious brain damage, he can no longer multi-task (Yes!  I was lucky enough to marry a man that could multi-task).  He is also one of the most intelligent men I have ever met.  This is not me saying this, his School History (top 50 in the country) says this.  He has been assessed as being unable to focus on more than one thing, his short-term memory is now not what it was, his ability to manage his stress is nothing compared to what it was.

But we manage.  He still has his job.  That’s thanks to an amazing boss.

So, the New Zealand insurance system, known as the Accident Compensation Corporation (ACC as we all know it) is our Dragon.

His treatment injury is permanent.  It affects many aspects of his life, and if we need help because of that, we have to go into the dragon’s cave, and convince that dragon to stop breathing flames, and let us past, to get to the help we need.

To this end, he has a “Case Manager”.  This is his direct contact in the dragon’s lair.  But this Dragon is very hard on their workers.  They are always being burned to the point the run, screaming from the lair, never to go back.

But on their way out, as the scream ENOUGH, they are supposed to contact all their “clients” and let them know they have reached boiling point, and they have run.

The Dentist

So why the back story?

Earlier this year Derek when for his normal checkup with the Dentist.  She checked his teeth.  They are ok.  He needs more work the longer he is on steroids, but it could also be the fact he has “reached a certain age”.   It comes to us all eventually.

She did x-rays, and then the bad news.  “You need to have your 2 remaining wisdom teeth out.”

“OK can you do it?”  Ah, the naivety of my darling husband.  Hell NO!  No way she will do it.  She doesn’t mind the fact he is on warfarin to stop him clotting.  He can even keep his INR within his designated range.  She isn’t even worried that he has suffered CAPS.  What she is terrified about is the stress it will cause him, and the very real, and high risk of an Adrenal Crisis.

“You need to see an orthodontist.  They will do it under anesthetic, in hospital, where they can keep an eye on you, and monitor you properly.

Here’s where it gets a bit real.  Oral and Maxillofacial Surgeons cost money.  OK, not as much as in the US, but a lot for little old NZ.  And I sure as hell am NOT paying.

I don’t mind paying $1500 to have it done in the local surgery, under local anesthetic.  But an oral surgeon, anesthetist, a hospital bed for the day (or longer).  No way.  We would not have to pay that if he didn’t have AI, so no way am I paying.

Fighting the Dragon

Off to the Dragon’s Leir we go.  Putting on our bravest face.  Derek puts the words on paper, and I then writing them in a firm way.

Please Mr Dragon, can we have the money for the surgery.  We should be covered as it’s due to the treatment injury he needs the high-risk extraction.

I don’t know if it’s the words I use, but we were told “Please, who would you like to assess you.  We have Surgeons on our supplier list.  Use one of them”.

Well, this is going to be a battle then.  And the line has been drawn.  We have to use one of THEIR suppliers.  And anyone who has dealt with ACC know that their “authorized suppliers” are not there to spend ACC money, they are there to reduce that spend.

First blood to us.  The Oral and Maxillofacial Surgeon we wanted to specifically use, is on THEIR list.  We get our choice.  But this is just stage one.  The visit to the Orthodontist to get an assessment.

Does Derek need the extractions: Absolutely, and soon, there is already jaw damage being caused by the teeth.  It will soon become painful, and there is a high risk of infection.

Does it need to be done by a specialist:  Absolutely yes.  These could be done in a standard dental surgery, but if something goes wrong, the teeth break or they are difficult extractions, then it would have to stop, and then it would become an emergency extraction.

Does Derek need General Anesthetic.  Yes.  Due to his complicated situation, a General, even though stressful on the body would be safer in case something goes wrong with the extraction.

We wait for the report to come back from the Oral and Maxillofacial Surgeon.  It didn’t come.  Oh, yes it did, it went to SPAM.

We then draft a very short email to Derek’s Case Manager and forward the report and the quote.

We decide that we will be willing to split the cost.  We pay what it would cost in a normal dental surgery, ACC pay the rest.  Now we wait to see what they decide.

Again, we waited.  The first thing we were expecting was a letter from the Case Manager saying “thanks for your email, I will forward it for decision”.  It never came.  So, a week later we sent it again.  Having fought battles with this Dragon before (and won) we did our usual.

“HI, you haven’t responded to our previous email.  Please tell us what is happening, we are reattaching the documents in case they were stopped at your gateway.”  We sent the email again 6 working days later.  Then we sent one without the attachment.  Still absolutely no response.  So we had one more go.  But this time we sent a copy to their “Complaints” department.  We have learned through the 6 years so far of battling ACC, that we need to do this to make things happen.

We received a very quick response, but NOT from our case manager.   The response included “ Ann has left ACC, this is why you wouldn’t have received a response yet.  We have sent your email to someone else”.  That was it.  Still no idea who.

Apparently, “Ann” ran so fast out the door of the Dragon’s Leir, she didn’t have time to let anyone know she was going.  We were put on to someone else.

We waited until “someone else” then responded to us.

So off to the complaints office we trot.  This time it was a “Formal” complaint, not just a CC.

We want to know who our case manager is, why have we not had a decision on the dental work, why were we not told our case manager had left, oh, and why, after making the first “complain” was his right to have what is called “Driving for Independence” looked at, and why was our GP being asked whether Derek was now able to drive safely, and therefore cancel the driving provided.

Suddenly a swathe of emails from the Dragon’s Leir.

  • SORRY! We have given you TWO case managers so this never happens again.
  • SORRY! Please resend the information and we will get it to our assessment team urgently.
  • SORRY!  I hadn’t read the case file fully, and just saw that your driving was renewed 6 monthly, and that hadn’t happened so was reassessing your need.  I now understand that it is a permanent (lifelong) Driving service we provide.

I guess, if you have to be given a sword to slay a dragon, it’s great when you get the RIGHT sword.

I have mentioned before, and if anyone fighting ACC reads this again, I will repeat.  YOU HAVE RIGHTS!  And for Derek, the key right is that they MUST take his injury into account whenever dealing with him. They had also failed to properly communicate with us, along with other violations.

For someone with a broken leg it’s easy.  They simply say, here, have some crutches and come on in for an assessment.  With a Mental disability due to an accident, it’s not as easy, and the sword they are given is NOT a strong one.  They are disadvantaged as they must fight with all the might of someone fully functional, but who is missing two arms.  And the sword can be taken at any time, just because they can.

But for Derek, they can’t do that.  His covered condition is Adrenal Insufficiency (although the regularly get that wrong, and are currently calling it Adrenal Sufficiency).   But as it is written everywhere that it is the lack of the stress response hormone, it’s a “Gotcha”.  They are not allowed to cause him stress because it will make him sick, and that is not taking his injury into account.

And they have come to the party.  Not quite with the best offer, but it’s a start, they have agreed to pay for the hospital, and the anesthesiologist.  We haven’t finished, but we have time.

So now the preparation begins for the 24 January 2020 (baring anything going wrong in the meantime). Derek is scheduled for the first appointment of the day, in a private hospital, for his tooth extraction, and a day’s monitoring to ensure he doesn’t get sick.

 

 

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.

When Will the Doctors Start to Listen

I have written several times, about what happened to Derek, and why. After getting all Derek’s medical records etc, we have now sent a letter to the hospital concerned to let them know what we found out. In all their investigations of why Derek got sick, we were never once asked 1 vital question. Did he use Low Weight Molecular Heparin when they stopped his warfarin for surgery? The answer is NO, because he didn’t know he had to, because nobody told him!

It shouldn’t have taken 18 months, and a letter from us, for someone to ask such a vital question. It should have been asked before he had surgery.

Finally, after getting our letter, we got a phone call from the Hospital, and they asked about it. We pointed out that he had never before been prescribed that drug when stopping Warfarin, only when going back on it, so why would he be this time.

We have since learned about a thing called Bridging. This means that if you have a coagulation condition such as Antiphospholipid Syndrome, then if your Dr, a specialist, or anyone else, wants to take you off your Warfarin, or other Vitamin K antagonists (drugs that reduce blood clotting by inhibiting vitamin K), then you MUST have cover with LWMH (e.g. Clexaine). Before you stop you warfarin for any reason, consult your rheumatologist. We didn’t. We didn’t even have a rheumatologist looking after Derek’s APS. We do now!

What I haven’t written a lot about is the anger I feel sometimes over not so much what happened (don’t get me wrong, I am angry about that) but the way we have been treated, both when it happened, and in the ensuing 18 months.

According to a Dr I spoke to a few weeks ago, Dr’s speak a different language to us mare mortals, and in fact think differently as well. This is not new information. We have all felt it. The interesting thing was that he admitted that they “make assumptions”. These assumptions can lead to death or, in Derek’s case, near death.

When a Dr writes a script for a patient, they assume the patients knows about the script, understands the importance of the script, and therefore will know what to do with the script. It is in the Doctors psyche that, because THEY know all about the script, therefore, so does the patient. It seems beyond their understanding to actually tell the patient the “what, when, why, how long” etc. It’s on the script in code, what does anyone else need to know the info for. What made it worse in Derek’s case was that he was never actually given the script himself.  It was faxed to our local pharmacy, with instructions for the pharmacy to contact us. The pharmacy didn’t have our contact details, and they assumed that we knew about the script being sent to them.

When you have one department giving advice on the need for the script, a second person in a different department writing the script, person 3 organising where to send the script, and person 4 (yet another department)  getting the special authority because of the type of script, it is assumed by ALL that somebody else has told the patient about the existence of the script. BAD ASSUMPTION!

That is not where the poor treatment ends.

When you see your Dr, they have a set routine list of questions they ask you to try and make a diagnosis.  Some of these questions rule out illness, some rule illnesses in.  But all to often the Doctors are tired, thinking about the last patient, or the queue of patients in the waiting room, and don’t have their full attention on you.  To cut down the time you have (normally 10 miutes)  the questions asked are close ended questions requiring a yes/no answer.

Do you have pain? Yes.
Rate your pain with a number? 8.
Where is the pain? My abdomen.

It’s not often that they actually allow the patient enough time for the patient to try and explain how that pain has been in more, or any other symptoms they have had at the same time.

If you have a temperature at home, but not when you get to the Medical person, then you don’t have a temperature.   Because you can walk into the clinic/ER etc under your own steam, you are obviously ok, and not dying.

When they do examine you, they make assumptions because when they are in Medical School they have it drummed into them that “If you hear hooves, look for horses”. That is great because a lot of symptoms are straight forward.

The problem is, what if the hooves they are hearing is one lonely Zebra, in amongst the herd of horses? If they don’t listen carefully to ALL the sounds around them, they may miss that crucial different sound.

For us, that different sound was in the guise of me.

I had been advised to record everything regarding Derek’s recovery from surgery so when we were asked about his history I would pull out my phone and go day by day, blow by blow, everything that had happened. I even offered to e-mail one Dr the details.

When I read the history in the medical notes later, I discovered that half of what we had told them, had not been recorded.  They recorded what THEY thought was important.  The big things, not the little things.  But it was the little things that were the clue to the CAPS and adrenal insufficiency.

For 2 weeks I kept telling people he was not well, it was not normal, and he was worse than he himself thought.

What neither of us realised at the time was that Derek had suffered a brain injury and his cognitive skills were shot.  He thought he had a headache because he was tired, but it turns out it was because he had difficulty thinking and assessing/analysing things.

For 2 weeks they kept asking Derek the same questions. And being a male, he would say he felt fine, yes there was pain, but it was more discomfort, yes he was tired, but he wasn’t well and not sleeping so great (because of the pain for goodness sake, but he didn’t say that) so no more than he would expect.

I on the other hand, was telling them he couldn’t walk 20mtrs without needing to lay down for an hour. He was drinking water, but barely, and then only sips. He was in pain all the time (not discomfort). He would groan in pain, but didn’t tell them that.  Each time I told them something, he would play it down. If they asked him a direct question, he would stop, think, then come up with some answer that was a simplistic personal analysis of how he felt, based on what HE thought they needed to know.  Because I was not the patient, they took more notice of him than me.

Each of the medical personal we spoke to (who didn’t know Derek) would pick another of the horses running around the room and say “Oh, that’s the one I can hear”. None of them seemed to be listening to me and putting the collective sounds together to see the zebra.

I don’t know how to get it through to the medical profession (and better women than me have tried) that when a patient is unwell, they are not the best judge of themselves. And the worse they are, the worse their judgement of their own health is. You need to ask the people that know them best because they are the ones that see the subtle changes.  It’s not just a mother’s instinct about her child.  Wives also have that instinct about their husbands.

Common questions were:

Have you eaten today?   Yes.
(Truth: actually, he took two bites of something and decided he had had enough)

Have you drunk much fluid?   Yes, I am drinking all the time.
(Truth: He is taking very small sips, and it takes him all day to drink a 750ml bottle of water)

Have you got pain?   Yes, it comes and goes.
(Truth: it was there constantly, but at times it was worse than other times.  The pain included a headache which was constant, leg aches, body aches, joint aches, but mainly the abdomen.)

Again, when he was in hospital I hit the same walls. Derek was barely conscious at times, could not hold a conversation, was not drinking water properly, could not stand without assistance, and could not think clearly. Yet still they insisted on asking HIM how he felt. At times he was disoriented and didn’t even know he was in hospital, but still they asked HIM.

At one point I asked a member of the medical staff (I believe it was a nurse) if there had been any brain involvement, and I was told NO, because there was nothing written down about it. Huh? Just because nobody else had noted it, doesn’t mean it doesn’t exist!

What makes me even angrier is that the person I asked didn’t even make a note of the query. I pointed out a “droop” that didn’t used to be on the left side of Derek’s face. No mention in notes about my concerns, so nobody investigated further. The question was brushed aside.

We now know that Derek did suffer a brain injury which still causes cognitive dysfunction at times, given we now know this, how the hell was he able to answer for himself when unwell.  I had to be his advocate, and I wasn’t being listened to.

It Happens everywhere.

According to one publication in the UK in recent years, some child deaths in UK could be prevented if the Dr’s listened to the child’s mother, the person that knows the child best. When a mother’s instinct says a child is seriously ill, they should be listened to. This should apply to the Partner/Spouse of a sick adult as well.

I have become very stroppy when dealing with Dr’s now. If I don’t get an answer I am happy with, I will keep pushing. I request copies of all reports, test results, letters from consultants.

I now keep a medical file, and check the results myself.

I have learned not to trust what the Doctors and other medical staff tell me.  I check everything for myself.

An example of this happening is when Derek had been in hospital for 2 weeks  they had finally worked out what had happened to him and they felt he was ready to be discharged.  They gave him the choice of being discharged home on the Friday and going to another hospital on the Monday for a CT of his Adrenal Glands, or stay as an inpatient  until Monday, but go home on a 4 hour visit on the Sunday.

I refused to let them discharge him and send him home. I said I didn’t want him going home until he had the CT on the Monday. I still wasn’t happy with how he was. I was concerned that his resting heart rate was still too high for my liking but when I questioned it, I was told that it was fine, “it’s because he has been sick!” He seemed to be breathless extremely easily, that he just wasn’t quite right still.

It was a good call. He had a large amount of fluid around his heart which could have killed him. Again, my instincts said leave him there, even though the medical staff were insistent that he was fine.  I was proven right and they were wrong again.

In all of this, we have had 1 Doctor that listened to me rather than Derek. That is  Dr called Helen Myint. She is the one who saved Derek’s life.  She literally went from his toes to his head examining him, but more importantly, she ask ME questions and I actually felt that she listened to me.  It was this Dr that picked up the CAPS, and Adrenal Insufficiency.  Two zebra’s in a room of horses.

I just wish I had asked her about the facial drooping as well, perhaps we might have had the brain injury found when it first happened.  I have since mentioned the droopy left side of the face (especially noticeable when tired), left arm weakness, bad left foot slap which took months of physio to correct to every Dr we have seen, but it took over a year for someone to listen, and accept that yes, there was a brain injury.  Again, the Doctors didn’t listen!

My best advice is, if you think there is something wrong with someone you know well, be it child or adult, don’t worry about pissing off the Doctors, make them listen, and don’t leave until they do.  And don’t be afraid to ask for a second opinion.  You have every right, and any good doctor should value extra input.

1 Year On

I can’t believe it is 1 year today since Derek was admitted to hospital for surgery to remove his prostate.  The day his life changed forever.

We thought that his diagnosis of prostate cancer was the end of the world.  It took a lot of strength to stay calm while waiting for surgery.  But life since surgery has been so different.

That is not to say that having Adrenal Failure (Addison’s is just part of the problem, but it is more than that) is worse than Cancer, but the life we now live is far harder than we imagined it would be following the successful removal of the cancer.

What was supposed to be a simple operation, with a 2-4 week recovery has turned into a lifetime of lifesaving drugs 3 times a day, with high risk of hospital admission due to illness/shock.  I worry daily about what could cause Derek to become ill.

The trip we took a few weeks ago just showed us how close he can come to a crisis just because something unexpected happens.

We put our trust in a medical system that let us down.

Something I have not made public until now was that there was something that could have been done to reduce considerably, the risk of DIC/CAPS.

Because what happened was such a rare event we wanted to know why it happened, and if there was anything that could have/should have been done to reduce the risk of such a catastrophic event.  Apparently there was.

In our search for answers, we found references all over the internet, from hospitals outside New Zealand, that talked about “Bridging” APS patients when they withhold warfarin for a period of time, to reduce the risk of a clotting event.  This involves LWMH (clexane) administered by injection and daily monitoring of INR.

Every medical person is concerned about the risk of bleeding out, and that is great because warfarin does create a risk.  But NOBODY was concerned about the risk of clots, which is why he was on warfarin in the first place.

Our thought was, did they know about the practice of Bridging with LWMH in New Zealand.  What we found out was YES THEY DID.

I found a booklet on line from a New Zealand Hospital that stated that patients on Warfarin were to be assessed as to their risk factors of bleeding vs clotting when having surgery.

When I examined the risk factors, the document put Derek in a High Risk group.

Patients at higher risk of thromboembolism if warfarin is withheld:

(a) Patients with mechanical prosthetic heart valves

(b) Patients who have suffered an acute thrombosis within the preceding 3 months

(c) Patients with a high-risk thrombophilia on chronic anticoagulation  (Antiphospholipid IS a high-risk thrombophilia)

These patients should receive bridging anticoagulation in the peri-operative and post-operative period. This can be done in consultation with a cardiologist (a) or a haematologist (b & c).

 

 

Time Low Risk Patients High Risk Patients
Before Surgery
  • Withold warfarin therapy 4-5 days before surgery
  • The night before surgery: If INR>2, give 1-5 mg vitamin K1 IV.
  • The day of surgery: If INR ≤ 1.5, surgery can proceed. If INR > 1.5, defer surgery, or if surgery is urgent, give Prothrombinex-HT (25 – 50 units/kg) plus 150 – 300ml FFP or 10 – 15ml/kg of FFP if Prothrombinex-HT is not used.
  • Withhold Warfarin therapy 4-5 days before surgery
  • 2-3 days before surgery: start giving once daily or twice daily treatment doses of enoxaparin SC (refer to: Therapeutic Anticoagulation with LMWH) or UFH IV infusion as per protocol (without bolus dose).
  • If using enoxaparin, the last dose (maximum dose 1mg/kg) should be at ≥ 24 hours before surgery. If using UFH IV infusion, it should be discontinued 4 – 6 hours before surgery.

They did not do the 2-3 days out from surgery cover, and they never checked his INR during that time so they have no idea what was happening.  We do know his INR was around 1.2 when he was admitted to hospital.

His blood results on diagnosis were extremely high:

  • PPT  –  42.3 sec (24-32)
  • Lupus Anticoag – 85.8 sec (32-45)
  • Cardiolipin IgG  – >150  (>80 considered High)

These three tests together scream extremely high risk of clots.  He was a sitting duck.

The booklet I have quoted is from the Capital and Coast District Health Board.  This is the hospital that operated on Derek.  Their own recommended procedure wasn’t followed.

We do not blame the Urologist that performed the surgery.  It was up to Hematology to advise the Anethatist/Surgeon what should be done regarding Warfarin/Heprin withdrawal before, during and after surgery.

The hematology department, when asked about treatment for his Warfarin, SHOULD have looked at his APS results which were in the system and accessible, and assessed him as high risk, and followed their own procedures.

There is no guarantee that it would have prevented what happened, nobody can say for certain it would have prevented it.  But it certainly wouldn’t have hurt, and it would definitely have reduced considerably.

We have talked to the surgeon and commented that he wouldn’t want to operate on Derek again.  His comment was that it wouldn’t be a problem, he would be given appropriate advice on how to deal with his coagulation management.

That’s when we gave him the news that he was given the wrong advice last time, so why would it be any different this time.  He just looked at us.  We then went on to explain what we had found out.

Whether he investigates further I don’t know, but we will see.

But for the want of 3 days of LWMH, he might not be on lifelong medication now.

If anyone reading this has APS, and has to have their warfarin with-held for a period of days, letting their INR drop below 2, you must ask your Dr what they are going to do about “Bridging” and if you are unsure, take information along, force them to look at what they should be doing.

Adrenal Infarction is an extremely rare complication in APS, CAPS is an extremely rare cousin of APS, both these things can be are triggered by withholding warfarin and having surgery.  Both these things have a 50% survival rate at best.

They are rare yes, but it does happen.  We all need to make sure our Dr’s take every precaution possible to reduce the risk of it happening.

It seems, from what I have read from others that suffer from rare conditions, the patients, their careers, and their families, have to take the initiative when it comes to getting the correct care.  The more authoritative information we can provide (not from other sufferers, but from medical specialists via the internet) the more informed our own Dr’s will become, and the better the care we will receive.

There are a lot of very good medical authorities out in cyber space that post genuine information about how to treat rare diseases.  Check them out and educate yourselves before it’s too late.

We asked about cover while they stopped his warfarin, but we didn’t investigate for ourselves, so blindly accepted their decisions and didn’t question further.

This lack of info on our part also meant that we foundered for 2 weeks afterwards before Derek was sent back to hospital, and I watched him almost die in front of me while we waited for the Dr’s to realise what had happened.

I hope that this blog helps save someone else who might go through this.

Roll on the next year.