Biologic Half-Life of Hydrocortisone.  Why is this important?

As Derek lives with Adrenal Insufficiency, we started looking into his steroid doses to work out whether he was on the best dosing schedule possible for him.

In 2016 we had an Endo appointment and asked for a Day Curve to confirm his dosing was right.  It was refused.  So we asked for 1 random cortisol blood test.  This was agreed to, more to keep us quiet than that the endocrinologist was actually looking for something.  What we didn’t tell him was what WE were looking for.

We both believed that his dosing at 3 times a day was leaving him with low cortisol in the middle of the day.  The only way to show this was to have a random cortisol taken right before his second dose of the day was due. His dosing at the time was:

6.00 am – 10mg / 12.00 noon – 7.5 mg / 4.00 pm – 5 mg

The problem with this dosing was that by 2.00 pm every day he was feeling like he wanted to sleep, and felt “blah”.  Some days he was also showing clear signs of low cortisol.

We had seen tables that said that cortisol had a Half Life of 8-12 hours, but that didn’t make sense.  We had also seen other tables that said 2 hours.  That was a big difference.  We needed to know what was going on for Derek.

1 Blood Test Tells It All

On the day we had set for the test Derek took his morning dose as usual at 6am.  We then did the things we normally do on a weekend, very little.   At 11.15 we went to the Lab for the blood draw.  We wanted it as close to his second scheduled dose of the day as possible.

When we got the results it showed what we already believed.  He was under range.  Not just under range for that time of day, but below range completely.  His cortisol was not lasting long enough in his body.  But we had been shown tables that said it had a biologic half-life of 8-12 hours, so how could he be below range in 5.5 hours?

This didn’t make sense even though we knew it was right.  So we started looking into what was meant by biologic half-life.  What we found out is very scary, very concerning, and actually very dangerous.

What did we find?

BIOLOGIC HALF-LIFE CAN BE RUBBISH.  It can be a false number, it shouldn’t be used in the way the below table indicates.

The table here is beening used by many groups/forums and on medical sites including on websites such as Endotxt.org, NCBI, and NADF so it must be right, surely.

Do NOT use this to work out the half life of your Hydrocortisone or Prednisone for dosing!

 

Here it was, the table we got shown constantly.  So Derek started looking further to try and find out where the biologic half-life came from.  The first thing he found was the definition for biological half-life:

 

“Time required by a body to process and eliminate half the amount of a substance introduced into it. Also called biological half-life, biological half time, metabolic half-life, or metabolic half time.”

A number of variations of this table appear on the Internet and use the column heading Duration of Action.  Other variations of this table simply classify the corticosteroids as short-, intermediate- or long-acting.  The same numbers apply no matter what the column is referred to as.

If this column truly is a (biologic) half-life, and we apply the rule of 5 half-lives for complete elimination, then that would mean that Hydrocortisone would be visible in the body for up to roughly 2 days (40 hrs).  Yet when Derek had a blood test before his morning dose, his cortisol was undetectable having had HC at 4pm the night before.  That was 17 hours and no detectable cortisol.  What would happen for the other 20+ hours?  It was clear there was something seriously wrong with this table.  None of this would be consistent with the title Duration of Action.

Also, if that was the case, you would only be prescribed cortisol once a day, not 3x, or more often now, 4x a day.

Where did this Table column come from?

There is no clear ownership of the table that we could find.  It is used, copied, and the copy is referenced, but tracking back to the original hasn’t been possible by us.  We do know it was created before 1980

He became very curious and decided to look further for the source of the information and came across this quote from “Principles of Endocrinology and Metabolism”,3rd edition, 2001, Chapter 78 “Corticosteroid Therapy” by Lloyd Axelrod.

This paper references the definition of:

“The commonly used glucocorticoids are classified as short-acting, intermediate-acting, and long-acting on the basis of the duration of the corticotropin (ACTH) suppression after a single dose, equivalent in anti-inflammatory activity to 50mg of prednisone.”

This is all about suppression of ACTH on high doses of prednisone, nothing to do with the amount of time you will remain within a safe cortisol range when you have Adrenal Insufficiency, yet Dr’s and patients alike use the table to justify twice a day dosing on HC.

So what are the implications of this table?

If someone uses this table to tell you that half-life is 8-12 hours for hydrocortisone they are wrong.

After looking for the original source of the table we discovered that the test was done as above, with a normal healthy person being given 50mg prednisone (approx 200mg HC).  The only thing that can be taken from the original research is that 50mg prednisone will suppress ACTH production for a period of time.  The hydrocortisone, and other drugs, were extrapolated from there (guess work based on poor knowledge).

If you had Primary Adrenal Insufficiency (Addison’s) and Hydrocortisone had a half-life of 8-12 hours, then taking HC every 6 hours would mean constant suppression of ACTH, and you would not have high ACTH after starting the steroid.  But we know this isn’t correct because many with Addison’s still have some part of their Addison’s “Tan” due to raised ACTH.  This is supported by the document below.

Professor Peter Hindmarsh is Professor of Peadiatric Endocrinology at University College London and Consultant in Peadiatric Endocrinology and Diabetes at University College London Hospitals and Great Ormond Street Hospital for Children. He is currently Divisional Clinical Director for Paediatrics at University College London Hospitals.  He also runs a website called CAHISUS.  He has written an article called GETTING CORTISOL REPLACEMENT OPTIMAL IN ADRENAL INSUFFICIENCY

The major goal of cortisol replacement in patients with adrenal insufficiency is to mimic as closely as possible, the normal pattern of cortisol production known as the circadian rhythm. The reason why we try to achieve this, is primarily to minimise side effects of over and under replacement and promote improved overall health. The two key factors are understanding the circadian profile and the pharmacology of hydrocortisone.

In this article Prof Hindmarsh talks about getting optimal dosing, and also looks at the absorption and clearance of people.  What he showed is that there is a very large variation between people. The article is well worth a read.  He also pointed out that the half life of hydrocortisone is a lot shorted than 8-12 hours, in fact, it is more like 70-90 minutes.

Another CAHISUS leaflet states this:

Hydrocortisone has a quick onset and the cortisol peaks to the highest level usually around 2 hours after being taken.  The cortisol obtained from the tablet lasts in the blood circulation between 4-6 hours.

This is a change from an old document by Prof Hindmarsh which included the old figures as above.  Things have changed, research has improved, and there is more knowledge out there.

What Does All This Mean in Steroid Dependant People?

For me?  Gobbledygook.  If you have a clear understanding of Half-Life, Clearance, and metabolism you may follow what is talked about in the studies.  Personally, it confuses the heck out of me.

I do however, understand the concept of half-life.  I first heard about it when watching a movie years ago about a child who had a certain amount of a chemical in his body at point C, and they claimed he had been given the chemical at point A.  It was pointed out that he would have drunk a gallon of the chemical to have the amount still in his system because of the half-life of the chemical.  The chemical had been very bitter and it would not be possible for the child to drink that much.  I became very interested in half-life.  I didn’t think then that it would be so important in Derek’s everyday life.

I had to ask Derek what everything he had found, and what the implications of half-life on hydrocortisone meant in layman’s terms, but even he struggled to explain it in a way that I could be easily understand. One thing he reminded me of is that while your Cortisol is going up, it is also being used.

I have also learned through this research is that even legitimate medical websites actually have misleading or wrong information.

When you are looking at a good way to dose for you, it must be an individual choice, based on how you feel between doses, whether you are willing to take multiple doses a day, and base it on signs and symptoms.  The fact that Derek felt low at the scheduled time of his second dose of the day, and this was supported by a blood test that showed low cortisol, meant we could get the Endo to agree that dosing more frequently was the right option for him.

Now that he is on a better regime of 4 times a day, he functions a little better, he has a low base level of HC, and in the last 6 months, has lost weight without trying, but not in a bad way.

I wish you all luck with this as I understand that there are many Dr’s out there who are not interested in listening to their patients on more dosing throughout the day.  One of the reasons for this is they don’t believe that you will be compliant, even though you are the one asking.

If they think you are asking for something that shouldn’t be done, then show them Prof Hindmarsh’s document above.

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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.

 

 

 

What Happens When Doctor’s Don’t Know Everything.

Over the last year one thing has become very clear.  Dr’s don’t always know everything.

We recently went to our Dr to ask about the fact that Derek was not feeling great.  After being told several times that he should not be taking extra Hydrocortisone just because he was feeling fatigued, or unwell, or had a ball of gastric wind under his ribs.

Because of this, he stopped taking the extra “stress dosses” of HC and began charting his BP instead.  After 6 weeks of readings at 4 times each day, we went to the Dr.

We found that his BP will go into a clinically Hypertensive range of 154/96 with a 68 heart rate one day and clinically Hypotensive at 94/59 HR 101 the next.  Just the effort of standing, and the change of position could have his pulse rate jump by anything from 40-60bpm (up to a 90% increase), where it should only increase by 20%.

Derek had been trying to get back to a semblance of fitness, so we organised through ACC, to have a schedule of fitness sessions with his Physiotherapist.  The physio was doing the starting assessment, and found out about the problem with his BP and would not touch him without clearance from the Dr.

Armed with the readings from the previous 6 weeks we went along to the Dr to ask if he knew what was happening and why.  The first thing he did (and all kudos to him) was to admit that Derek was the one and only “Addison’s” Suffer he had ever dealt with, and didn’t know that much about Adrenal Infarction and the complications of it.

He looked at Derek’s medical records.  The Endocrinologist had written that at some point, and probably without warning, he would need his fludrocortisone increased.  So it was decided that perhaps a change of Fludro from 0.05 to 0.1mg per day could work to stabilise it.

Home we went, armed with the hopeful knowledge that it was the fludro out of sync that was probably causing the large difference in BP.

We tracked his BP for another week.  There was definitely a change in pressure and heart rate.  It continued going higher during the day, but falling further overnight.  It had also become more erratic.

OK, so 0.1 wasn’t the answer.  So go back to 0.05 but twice a day.  We had seen on various forums where others had had some success with this option.

Another week of tracking and another change in BP (or not).  Still heading upwards during the day and downwards at night and still no real pattern for week days and weekends.

We returned to the Dr with this new information.  He agreed that the extra fludro wasn’t doing anything positive so we should go back to 0.05.

He also had the results of some blood tests.  There was nothing markedly different from last time.  Kidney’s still not at a great level, everything else seemed fine.  Potassium and Sodium all comfortably within range.  Thyroid a little wonky but he has no adrenals so it is not unexpected, and not sounding an alert.

The Dr agreed that it was time to refer him back to his Endocrinologist and to a Cardiologist.  So we now wait for an appointment.

While we wait however, we don’t just sit here doing nothing…..

And the mystery continues.

Along with his BP and HR issues, he also can’t take a fright/shock or be startled now without “crashing”.

We decided we needed to get to the bottom of it.  Thinking it was BP related we went out on Saturday to go for a drive, taking the BP monitor with us.  One of two things was going to happen.  Either nothing would happen to cause him an adrenaline rush, or someone would do something to force me to break quickly and sharply, which would give Derek a fright, and I would get an adrenaline rush.

Thankfully the latter happened.  Derek was on the phone, and didn’t see the car in front of me that started to pull into another lane, and then started driving in both lanes, blocking everyone.  I had begun to speed up as we were going 20-30 km under the speed limit.  A little late, I realised he wasn’t pulling all the way over, and I braked harder than intended.  I got a little adrenaline rush as I thought for a brief instant, that I may hit him.  I felt my heart start to beat a little faster and harder.  I got a tingly feeling around my chest.  I am sure we have all felt it.

I asked Derek how he felt.  I could tell immediately that he hadn’t had an adrenaline rush.  It was obvious that it was going to be another drop.  I continued driving for about 2 minutes, until I could find somewhere safe to pull over.

We took his BP and pulse.  It was perfectly normal.  Almost TOO normal. His pulse was in the low 60’s.  Yet we could see, and he could feel, the usual symptoms.  Headache, feeling slightly ill, couldn’t keep his eyes open, felt weak, his speech went quiet, it slowed down.

We continued home (5 minutes away) and checked his BP when we drove in the drive (before he got out of the car).  It hadn’t moved.  His pulse was the same and his BP was within 2mmgh/l of the first reading for both systolic and diastolic.

We continued checking it every 10-15 minutes for an hour.  I had to wake him to do it as he went to bed and just lay there, unable to talk, or really communicate.  Still there was no change in his BP or pulse.

We are none the wiser.

So we are going to try his glucose levels next.  The problem is, we have to wait to see what happens with a fright.  Unfortunately I can’t just make a very large sudden noise and frighten him myself, as tempting as it might be.

We have done a lot of reading on the topic of the Adrenal Medulla and epinephrine/norepinephrine.  There are definite links to these hormones and stress.  There doesn’t seem to be a lot of research into what the effect of not having an abundance of these hormones does.

Adrenal medulla

The inner part of the adrenal gland is called the adrenal medulla. The adrenal medulla produces hormones called catecholamines such as adrenaline and noradrenaline. Catecholamines play a role in the response to acute or sudden severe stress, for example during life threatening event.

Catecholamines are responsible for the palpitations (racing heart), sweatiness, widening of eyes and shakiness of the hand when faced with sudden fear or other stressful situation.

This information is all over the internet.  What we can’t find is what happens when this process doesn’t happen properly and you don’t get the palpitations, sweatiness, etc with sudden fear or stress.  Does the body skip to the next section (rest and digest), or is it part of the process fails but another part kicks in, and then is not switched off because again, the Medulla has failed it’s job.

It is very hard when you have a rare condition, one that is so rare that little research has been done.

If we could find an answer to what is happening when Derek faces the sudden stress perhaps we could find a way to reduce the impact but the Dr’s don’t know and we don’t know how to get them to investigate.  In the meantime, we are having to do it ourselves.

Dr’s don’t know what to do with Derek.  If anyone has an answer please let us know.

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.