The Half-life of Facts

Aside

I was watching QI this morning. It’s a show where the facts are “Quite Interesting”, and generally obscure.

This morning the episode said something I was so intrigued with, that I rewound it to.
It stated that “At medical college, they usually teach that half of what medical students will learn, will be considered untrue in 10-20 years. This is termed the “half-life of facts”. That is to say that you know that half of the information will be untrue, you just don’t know which half.”

I found that to be Quite Interesting. Enough so that I decided to look the fact up, and see if there was any truth to it.

Samuel Arbesman, a mathematician at Harvard, titled his new book “The Half-life of Facts”. When talking to the Economist, he stated:

For example, in the area of medical science dealing with hepatitis and cirrhosis, two liver diseases, researchers actually measured how long it takes for half of the knowledge in these fields to be overturned. They gave a whole bunch of research papers from fifty years ago to a panel of experts and asked them which were still regarded as true and which had been refuted or no longer considered interesting. They plotted this on a graph. What they found is that there is a nice, smooth rate of decay; you can predict that every 45 years, half of this particular sort of knowledge gets outdated.”

But why am I citing an article in the Economist. Because you probably know a Dr that is still using that 50% of knowledge that is now outdated. Yet they treat you based on that knowledge.

According to Dr. Michael Gold from the Medical University of South Carolina; To paraphrase:

The half-life of medical knowledge is seven years.”

If Dr’s are not keeping up to date, they may be treating you based on what is now outdated knowledge. We have found one such Dr in the guise of the Medical Advisor of one national Advocacy group

He trained 50 years ago. Based on the half-life of facts, if he finished his training in 1970, by 2010, almost 50% of what he learned is outdated. Some of that is “knowledge” is still stating as fact which is on the groups public webiste. When we recently questioned this knowledge (which we showed with research papers to be obsolete) we were told by the Advisor “I don’t care.” (words in writing).

This attitude by the “Medical Avisor” of a major Advocacy Group for a rare condition leads to several questions.

  1. Why is he holding the position? Is it just that it has some kudos?
  2. How does his obsolete knowledge help patients today to live a better life when other Doctor use that “knowledge” to treat the patient.

Knowledge is Power, but obsolete Knowledge is DANGEROUS!

Continuing education is key to good Doctoring. And that continuing education must include reading recent research on the conditions they are treating and accepting that just because it goes against what they have been taught, doesn’t mean it is wrong, or that the Dr researching and promoting the new information is doing it “for ulterior motives”.

Next time you speak to your Dr, ask him the date of the last Research Paper was that they read? If it is before 2000, then there is a better than even chance that half that knowledge is obsolete, or will be in the next couple of years. Ask them if they have heard the term Half-life Of Facts.

Some will agree, others will get upset because they will be reminded that they are not as up to date as they should be, and others will probably refute the statement, or get angry about it. Most likely the latter, are so well past their half-life with knowledge, that they should perhaps be ignored completely.

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The Expert Patient

As far back as 2002 an article was written for the British Medical Journal encouraging patients to become “Expert Patients”.

Clin Med (Lond). 2002 May-Jun;2(3):227-9.
The expert patient: a new approach to chronic disease management for the twenty-first century.   Tattersall RL1.  Author information

Abstract
The expert patient: a new approach to chronic disease management for the twenty-first century, produced by the Department of Health, recommends the introduction of ‘user-led self management’ for chronic diseases to all areas of the NHS by 2007. The premise is that many patients are expert in managing their disease, and this could be used to encourage others to become ‘key decision makers in the treatment process’. Furthermore, these expert patients could ‘contribute their skills and insights for the further improvement of services’. It is hypothesised that self-management programmes could reduce the severity of symptoms and improve confidence, resourcefulness and self-efficacy. It is stressed that this is more than just patient education to improve compliance. Instead there should be ‘a cultural change…so that user-led self management can be fully valued and understood by healthcare professionals’. I point out that these ideas, while welcome, are not particularly new. Achieving the desired culture change will not be easy.

“Expert patient”—dream or nightmare?

BMJ 2004328 doi: https://doi.org/10.1136/bmj.328.7442.723 (Published 25 March 2004)Cite this as: BMJ 2004;328:723:

The concept of a well informed patient is welcome, but a new name is needed

Since the chief medical officer for England first introduced the term expert patient, it has been picked up and used very widely.1 During this time, the notion of the expert patient seems to have been criticised by doctors at least as much as it has been welcomed.2 If one asks lawyers, architects, social workers, or management consultants whether they prefer clients who take an interest in the issues they face and are motivated to work in partnership to achieve successful results, the answer seems obvious. So why does the idea of expert patients provoke such antipathy within the medical profession?

There is even a Training Course across England called the “The Expert Patient Program which states:

“Creative thinking is key for the Expert Patients Programme (EPP), a thriving Community Interest Company that gives people more control over their conditions through cognitive therapy courses.

It has made a particular difference to people with long-term illnesses, such as diabetes, arthritis or respiratory problems, by teaching them how to self manage and monitor their own symptoms. “It’s giving patients more control,” says Renata Drinkwater, Chief Executive (interim). “Courses like these are proven to make a difference. They boost confidence and, in some cases, can delay the onset of other conditions. They also have other benefits, like reducing the amount of times the patient needs to go into hospital or use Accident and Emergency.”

Conclusions
So, will the vision set out by Professor Donaldson and the Expert Patient’s Task Force work? The simple answer is ‘no’, unless there is a sea change in attitudes among patients and, more importantly, healthcare professionals.

What About You?

As someone with a chronic illness, would you consider yourself an “Expert Patient”?  Do you consider your Dr a Good Dr or a Bad Dr.  Is he bad because he has a history of harming patients, or a bad Dr because he doesn’t know your specific condition.

As a patient with a chronic illness you go to your Dr expecting a good level of knowledge, and that they keep their knowledge up to date.

You go to a specialist with the same expectation.

What happens though if the chronic illness you have is rare condition?  If your Dr or Specialist only has 1 patient with your condition, but over 1000 with a more common one.  Do you expect them to keep their knowledge up to date?

The reality is, if you have a rare condition, you can’t and shouldn’t expect them to be as up to date as you want, or to have the knowledge you would like, at least not when you are a new patient.  It’s different if you have had them for 2, 3, 4 or more years.

Instead, what you CAN expect, is to be listened to, to have them acknowledge that it is a learning process for you both, and that if you are able to provide relevant up to date information, they will accept it and not dismiss it because it wasn’t what they learned when they were training 20/30/40 years ago.

Yet this is also not always the case.  So instead you learn what you can about your condition, to fill the gaps and hope you can at least work around your Dr.

In truth, a patient goes to the specialist wanting help to improve their quality of life.  If you are lucky your specialist, knowing you are coming, has done a quick review of your medical notes, a quick read up of the condition.  But with that, they have decided, almost before you walk in the door, what treatments they will or will not offer.  If you ask a question they are not expecting, you take them off guard.  But they don’t want to appear like they don’t know the answer (which they probably don’t) so they either ignore the question, or give some answer they drag from the back blocks of their brain where they remember something they heard once years ago, whether right or wrong.

These are the Dr’s we hear about in the forums.  The ones that “don’t get it”.  We get frequent cries of “I’ve fired my endo.”  “My Dr is an Idiot.” “I can’t find a decent Dr who knows anything.”  And I can see why they say it.  I have heard the horror stories of medical appointments, ED/ER visits, lack of knowledge and but refusal to acknowledge it.  These Dr’s need training in your condition, but they are not getting it.  Why?  Is it lack of time?  Lack of interest?  Or maybe they don’t know where to start?

What would happen if, instead of leaving frustrated, angry, and ready to fire them, you sat down and tried talking to them as equals?

What if you opened up the consultation with a different tact.  If, instead of expecting them to know everything, or worse, you go in expecting to have a bad appointment with them knowing NOTHING,  you went in knowing they didn’t know anything, but were open to learning.

What would happen if you opened up the conversation with something like “I am struggling with my conditions, and with the general lack of knowledge and research available.   I don’t know how you Dr’s keep up to date when there are so many rare conditions out there.  I need to get a better quality of life, perhaps we could learn and work together on how to better manage my health.”

What would your Dr say?  You are not challenging their knowledge, but you are also not putting expectations on them to know it all.   If they take that bit well, perhaps go on with “Do you have any recent research I could read, or a website where I could go to get good up to date research on my condition so I can learn more about how to help myself”.

The worst that could happen is they dismiss you out of hand and go back to their personal ideas. If this happens you have two options, you can get upset and leave the consultation frustrated then go on line to rant.  Or you could ask them straight what their objection to having a knowledgeable patient is.

By now you have nothing to lose
if you have already decided to leave
and find a new Dr?

You might get a surprise.  You MIGHT get a Dr that is willing to work and learn with you.

The Expert Patient may not only need to be an expert in their condition, but also in negotiation.  A Win/Win negotiation is an art.  It requires giving the Dr something they really need (which may be stroking their ego) but also getting what you need, which is  a Dr willing to work with you.

Dr’s won’t learn, if they are not taught and quitting a Dr because he doesn’t already know simply means that the next patient will get what you have, a Dr that has neither the time or interest to learn.  You never know, you might awaken in that Specialist or Dr, a new interest in your rare condition.  Then he won’t be a Dr to run from, but a Dr to run TO.

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.

Blue September

Man Up – Give Prostate Cancer The Finger

It is a few words, with a short reach.

If you go on line and ask for “Breast Cancer Catchphrases” you get hit after hit.  The top ones were:

85 best Breast Cancer catchphrases
71 best Breast Cancer catchphrases
50 top Breast Cancer catchphrases.

Yet when you do the same search for Prostate Cancer you get

Prostate Cancer Humour
Prostate Cancer Quote of the Day
Funny Cancer T-shirts

And the images are worse.  Images of young women with Breast cancer, or “representing” breast cancer.

When you search for images of men with Prostate Cancer, they are all over 65 and the most common quote?  “Lots of men die WITH Prostate Cancer but not OF Prostate Cancer”.

According to information on the New Zealand Prostate Cancer web site:

In New Zealand, prostate cancer is the most common cancer in men, around  3,000 registrations each year and about 600 deaths from prostate cancer each year (based on the statistics from the Ministry of Health 2007 – 2009 which show an average of 3082 registrations and 602 deaths).

Men who develop prostate cancer are mostly over the age of 65. It rarely occurs in men younger than 55. About one in 13 men will develop prostate cancer before the age of 75. In very elderly men, prostate cancer often grows very slowly and may cause no symptoms.

Some men are more at risk of getting prostate cancer than others, but the most important risk factor is ageing. Men with a family history of prostate cancer have a higher risk; that is, if the father, an uncle or a brother has had prostate cancer.

Sounds good doesn’t it?  Only 1 in 13 men develop Prostate Cancer before age 75, it RARELY occurs in men younger than 55.

Just as a comparison, according to the Breast Cancer Foundation of New Zealand, Breast Cancer is the #1 Cancer for women (same as prostate cancer in men), 3,000 new cases of Breast Cancer each year (same as men), 600+ deaths in a year (same as men).  What isn’t the same, is the awareness campaigns.  The only real differences are:
Men get Breast Cancer, Women don’t get Prostate Cancer,
Women are more likely to get it over the age of 50,
men, prostate cancer is more common over the age of 65.

Fantastic.  At 50, Derek should not have to worry about Prostate Cancer.  There is no history in his family of breast or prostate cancer, no history of any other form of hormone cancer, he is only early 50’s.  Absolutely NO reason to even consider he would have it.  There is no required routine screening.  There is no “when was your last DRE” at the Dr’s office the way you are asked about your last Smear.  There is no national recall every 2 years the way there is for a Mammogram over the age of 45.

Why would there be, at 50 it is very rare to get Prostate Cancer.  And according to a survey conducted by the Cleveland Clinic:

“It turns out that taller men, and men who have bigger body mass indexes are at higher risk of high grade prostate cancer, and also are at higher risk of dying of prostate cancer,” said Dr. Klein.

Dr. Klein said previous research has shown a connection between obesity and the risk of developing high grade prostate cancer, however the relationship with the height factor has not been noted previously.

Researchers surveyed data from more than 100,000 men and found that for every ten centimetres in height, the risk of developing an aggressive prostate cancer grew by 21 percent.

Not considered taller than average at 5’10”, a healthy weight, under 55, and no family history of Prostate Cancer, Derek is safe.

He might get Prostate Cancer in his 70’s or 80’s but who cares, a lot of men do, and “they don’t die of it, they die with it”.

But all the risk factors are just that, risk factors, not a guarantee that you won’t get prostate cancer.

So why am I blogging about Prostate Cancer on a Blog about Adrenal Insufficiency.

Because, if you haven’t already read the rest of this site, you may not know that Derek has AI because of a medical botch-up when he was having surgery for the prostate cancer that according to all the risk factors, he wasn’t at risk of getting.

We didn’t actually know a lot about Prostate Cancer when Derek was in his 40’s.  So how did he get diagnosed just a couple of years later?

A mixture of things.  At 50 he had he had a problem and went to the Doctor.  He was given DRE (Digital Rectal Exam).  It is far less invasive and embarrassing than a woman having a smear, yet men won’t talk about it let alone have it.  What man wants a stranger to shove his finger up your rectum?  Well get over it guys, you expect us woman to lay there while we have a very cold speculum put up your vagina, followed by a scraping tool, to take cells.  Men simply have 1 finger press down on the prostate, and out, that’s it.  20 seconds at most.  GET OVER YOURSELVES, YOUR RECTUM ISN’T THAT PRESCIOUS!!!!!

It turns out this DRE was actually not completely normal, but Derek wasn’t told that.  He was sent for blood tests to see if there was an infection.  The Dr also requested a PSA.  We didn’t take much notice of blood tests back then, so we didn’t look at what was being asked for, and certainly didn’t worry about the results.

By 52 we had forgotten all about it.  Then he got a soft calf injury that caused a DVT.  He was diagnosed with Antiphospholipid Syndrome (APS).  That’s fine, strange but fine.

By 2011 (at the age of 53) his PSA was rising but hadn’t hit the special number of 4. (It was close)

Then, at the beginning of February of 2012, a month after turning 54 his PSA shot up.  He was fit and healthy, he had APS but that was not cancer so no worries.

In May 2012 Derek had a positive biopsy for Prostate Cancer. Thankfully, because he was having his INR monitored for his Warfarin (because of the APS) he was also having PSA every 6 months.  The Dr had seen in increasing.  So he had had Prostate cancer since BEFORE he suffered a DVT.

After the positive biopsy we looked into the options available.  His Cancer was considered MODERATELY AGGRESSIVE, which meant that, without treatment he would die OF prostate cancer, not WITH prostate cancer.

So, Not overly tall, not over weight, under 55, no family history of hormonal cancer in the family (although his father had dies of leukaemia, but that’s wasn’t a risk factor for prostate cancer) yet he had prostate cancer.

Women are told all the time that you can get breast cancer even without the risk factors.  Men are not told the same.

Why Not?  What is it that makes it so important that a woman is checked for cancer, but a man is not.  Are men any less important?  Or is it because men make the decisions, and their decision is they don’t want that initial FINGER UP TO PROSTATE CANCER?

Some people believe that it is not necessary to have PSA checks, that the chances of prostate cancer killing you is not worth the effects of invasive testing that can be wrong, the worry, the removal of a prostate (and the very high probability of erectile dysfunction) is not worth it.

Well, those people can go elsewhere to comment.  I know that Derek would be terminal now, if not dead, if we hadn’t had the testing, the biopsy, and the surgery.

SEPTEMBER IS PROSTATE CANCER AWARENESS MONTH

Even if you don’t meet ANY of the risk factors for Prostate Cancer, YOU CAN STILL GET IT?

We didn’t know this, until we did!

 

When Different Became Normal?

4 years and 9 months ago I took my husband to hospital for surgery to remove cancer.

I knew, as we walked Derek to the hospital ward for admission that things would change.  He was having his prostate out, there were implications, including not knowing if this would put his cancer in remission, or if it was just step one of a long journey.

We had no idea at that time, that things would go so horribly wrong, and that he would forever by living life on the edge of the precipice, waiting for the slightest thing to push him over the edge.

We spent the first 18 months after surgery going from medical appointment to medical appointment.  It wasn’t unusual to be told “wow, and he is alive” like the Dr’s were patting each other on the back for doing such a great job, or shocked that anyone can live through what he suffered (very few do).

Then the appointments they were making started to dry up.  So we began pushing.  Things were still not right.  There were still things that had not been acknowledge, investigated, diagnosed.

After 3 years we felt we knew mostly how the CAPS affected him.  Knowing what was wrong, what we could fix (which wasn’t a lot) and what we had to learn to live with.

We kept using the term “our new normal” when asked about how we coped with everything.  According to the many medical books out there, most people with Adrenal Insufficiency can just take their medication 2-3 times a day and have a normal life.

Except that this isn’t the case for most.

At the 3 year mark we started reading, we started downloading “stuff”, we started learning everything we could about Derek’s medical conditions.  Neither of us has read so many medical studies.  Or chased so many references to find the original source data for all the presumptions.

While learning, life carried on.  We added meds (at our insistence, not the Dr’s), we changed Dereks dosing schedule as we learned that HC didn’t last long

The we realised it.

We are coming up 3 years since Derek’s last Crisis.  We have managed chest infections, urinary tract infections, colds, throat infections, injuries (mostly minor), and frights.  All without emergency medical intervention.

We had woken up one day and we weren’t working on getting used to our “new normal”, life was again just “normal”.

When did that happen?  When did Derek having Primary Adrenal Insufficiency, Dysphagia, constant brain fog, and a frequent need to “up dose” become just NORMAL?

I had to race home earlier this year as Derek was unwell, but I didn’t panic, I didn’t get a ride with anyone, I didn’t even feel an urgent need to go and get a taxi home, I just took the bus and train, then routinely sorted out his medical appointment and treatment.

I wake every morning and wonder “is this they day I find him unresponsive”, is he going to roll over and take his morning cortisol like normal.

For 4 years I would leave home each day worried about what would happen if I got that call.  When I got it, I didn’t panic, I just told Derek what to do, and headed home.

I am never really happy in the morning until I know that he has rolled over and taken his meds.  But quite often I sleep through him doing it.  Whether or not he is awake by the time I am out of the shower is still on my mind, for a fleeting moment, then things turn to normal daily routine.

I get up, I get ready for work, I head out the door, wishing Derek a good day, and work all day.

There was a time when I had to call or txt Derek 2-3 times a day to see how he was, listening intently to his voice to see if I can get any clues on whether or not he is sick.

Now I only call when I need to speak to him about something.  I will still txt him most days, especially if I notice that he seems a little tired the night before.  But it’s not with dread of what the response will be, it’s with a genuine “how are you” as you would ask anyone who was tired.

I don’t know when it happened, but our New Normal, is now just Normal.

The misquote in the medical text which originally said “you can live a normal life span” became, for many Dr’s “You can live a normal life.”   It is a bit like the misquote from Spock, who never actually said “It’s life Jim, but not as we know it”.  It sounds great, but is an urban myth based on some small portion of words.

However, there is some truth in it for anyone who is chronically ill.  As the mother of young children, running around dropping them at different activities, sitting up until they were asleep at night, having them with me in my bed when they were sick was all very normal for the situation, but if you didn’t have children, then it wasn’t normal, it was different, it was hard work, it was tiring.

So too with the chronically ill.  For the outsider, it isn’t normal to feel tired all the time, it isn’t normal to take multiple doses of multiple medications just to function, it isn’t normal to finger prick every day before and after meals.  But for those that do it, at some point, it does become normal.

And that is where we find ourselves.  We are out the other end of the tunnel, and that big light heading towards us wasn’t a freight train on our track, it was on the adjacent track.  It shook our world when it went flying past, but it didn’t stop us in our tracks, it just caused us to take a little detour in our life.

Derek still has to take hydrocortisone, fludrocortisone, DHEA, warfarin, vitamin D, BP meds, and anything else that he needs to function, but that’s normal.

We are lucky.  Our normal is actually OK.  We’ve got this.

How did we get here?  We got educated.  We studied his conditions, and we took control of them and we lived.  We continued to do things daily.  Some days it is a struggle, but we do it, one foot in front of the other, one dose of medication after the other.  Along the way we learned what “normal” meant for Derek, in his blood tests, his BP, his fatigue levels, even his body temperature.  Knowing his “normal” and accepting it, means we can work with it.

Part of accepting the new normal was accepting what you can’t change.  After working around it for a while, it will become normal.  It’s like taking a different road when going to work.  If you take it often enough, it becomes your normal routine.

There is a prayer that many groups use about acceptance.

 

Even if you are not a person who believes in prayer, the sentiment is the same.  Acceptance of what is, courage to learn how to change what is needed to change, and the understanding that there is a difference.

 

 

 

There are days when Derek isn’t so well, but that’s ok.  We know what to do, and we do it.  The good days outweigh the bad.

Normal, in this new form, is not great, but it is good.  It is doable.  And in the words of a good friend, “He is clearly alive”

I asked Derek his take on this NORMAL

“The new normal is doing less than before, but it is something.  We do what we can, and enjoy it. Failure to accept the situation would lead to depression, and I’m not going there

It’s not what Jo signed up for. But it’s what she has also accepted.  We have all had to accept it, including the kids.  It could never be normal until we all accepted it.”

Thank you to all those who have helped us to get to this point, there are too many to mention here.

 

Doctor Shopping

According to Wikipedia “Dr Shopping” is done by patients trying to get prescriptions for pain killers or the like, from multiple Dr’s.  Dr’s believe this is also the only reason.

But it’s also a term used patients when they are dissatisfied with the care their current PCP/GP is giving them and they want either a Single Second Opinion, or you want a complete change of Dr, and they try several before they settle on one.

I recently heard about a Seinfeld episode (I didn’t watch it myself) where someone had been labeled “a difficult patient” and then “fired” by their Dr and tried to steal their medical records to change the “difficult patient” label because it was being seen by every Dr they went to.

When you Dr Shop for a better standard of care, what it is assumed is that you are looking for drugs, because, well, what else could it be?  Heaven forbid you might actually just want to get your life back on track, and have a better Quality of Life.

And once something negative is put on your medical file, anyone that reads it, will believe it, because it was written by a Dr, or Nurse, and they know you better then you do.  After all, they have seen you for at least 10 minutes.

Many patients with chronic illness have this problem.  One I heard of recently had the term “malingerer” put on their file, even though during that particular ER visit, they were admitted to hospital seriously ill for several days in adrenal crisis.

The process to have that single word removed from their file was going to be long and arduous.  And it was not guaranteed it would be removed.

These days people are realising that we all have a right to a certain standard of care, and to get that you sometimes have to shop for the right Dr for you.

Have YOU ever gone Dr shopping?

We have.  Our Dr, who we liked, retired and we had to find someone who could look after Derek’s complex situation.  We wanted someone who was willing to acknowledge what they didn’t know, and be willing to learn what they needed to, to help Derek live the most “normal” life he could.

We needed someone who would order tests if we asked, who would acknowledge tests that were not within Derek’s “normal” range, and who would suggest options, referrals, medications, to help, not hinder him.

That was hard.  Our Dr had gone on a 3 month sabbatical so we used the Locum, but found her to be a very basic, “if it’s not a cold, I don’t want to know” type Dr.  We then learned, because Derek needed to see a Dr, that ours was not coming back but had decided to retire.  We were offered the opportunity to stick with the temporary Dr in his practice or go to another Dr in the practice.  Neither of these was an option as we knew both and had found them lacking in both People skills, and willingness to work with the patient.  They would rather talk AT you than WITH you.  There was no way I would entrust Derek to their care.

We had also learned not to trust Doctors completely so finding one I was willing to trust was going to be hard.

I started thinking about this recently because of a news article I read about a new Dr’s practice opening up in a town which was suffering an extreme shortage of Dr’s.  The problem was, the Head Dr in the town said patients should NOT Dr shop.  She insisted you should stick with the Dr you had good or bad, and learn to work with them.  But there was no mention of the Dr learning to work with the patient.

When you have a chronic illness it is more important than ever that you have a Dr you trust, but also a Dr that knows the correct way to treat you.

In the social media groups, you are not supposed to Criticize doctors.  The reason for that became obvious to someone one day when their specialist told them he no longer wanted to treat them because they had heard what this particular patient had said in a closed group, about how she felt his care was.  It was also originally not allowed to recommend or “rate” your Dr.  Because of issues around recommending Medical Practitioners and litigation in some countries, it is also good practice to avoid support groups advising one Dr over another.

Now however, there a several “Recommended” lists for people to find the right specialist.  This works to a point, but you have to be able to get in to them.  You also still need a General Practitioner or Primary Care Practitioner.

This is where Dr shopping IS a good idea.  And this is what Derek and I proceeded to do.

A couple of years previous the local Medical Centre had been taken over, and renovated.  We were hearing good things about the new owner.

Since we were looking for a new GP, and the Medical Centre was, within walking distance for when Derek was feeling well, it was decided that we would check out the Doctors there.  Only I didn’t want just any Doctor.  I wanted one I could click with.

I had already been Dr shopping when looking for a family Dr years ago, when I found the one that is now retiring.  I had been to a number of Drs after we moved town, but hadn’t yet met one I “clicked” with, until that particular Dr.

This time I was doing it deliberately.  So I went in with a list of questions, and informed her immediately that I was there to see if I felt she was the right Dr for us.  Her books were closed, she was the 2nd in charge in the practice of 10 Doctors and it would be hard to get on to her books.

Some of the questions were:
1.  Are you willing to allow me full access to all test results?
2.  Are you willing to have patient led care?
3.  Are you happy being interviewed by a patient?

Derek is lucky.  The Doctor was very happy we were interviewing her.  And once we explained Derek’s medical conditions, and history, she understood that we were being careful about who we chose.

Once you find your Dr

Whoever you decide on, once you start to work together it needs to be a partnership.  It can’t be one sided, from either side.

A friend of mine has written a document for working with your Dr which is well worth a read.

Talking to your Doctor

by Des Rolph

When going to appointments be prepared. Take your medical history with you. If it is extensive, type it up making dot points, not long paragraphs. It is easier for them to read. Keeping your notes concise and to the point is key, no rambling on.

  1. Tell them that you would like to work with them in treating your problem and that you need to fully understand reasons behind treatment and what the expected outcomes should be.
  2. Ask if they have any documentation/information around their proposed treatment and your condition, or can they point you to websites that explain the condition.
  3. Initially go in with a list of symptoms and general questions rather than with your own thoughts on diagnosis. You can always do a bit of steering if they aren’t connecting with you, but things get missed if you try to lead too early. If you are not sure about anything, ask questions.
  4. Have a list of questions! Do not throw them together at the last minute, but jot them down in the weeks leading up to the appointment. Take two copies, one for the doctor, one for you to make notes. I have found some doctors write on your notes and hand them back to you.
  5. It is helpful to take someone with you to the appointment, so that they can take notes so you don’t miss anything. For some reason, having someone with you who can validate your symptoms can have a positive impact. It might be useful to have an extra copy of your list of questions for them as well.
  6. Keep a symptom diary. Jot down BP on waking and going to bed at minimum. It is helpful to have a glucose metre, and take note of your blood sugars before meals. Jot down any symptoms you experience and record your cortisone dose if you have updosed for the day. If you have pain, indicate out of 10 what level it was.
  7. Medication list – also break it down by time of day and dosages for each
  8. If you have multiple doctors and/or multiple conditions, be sure to note who prescribes each medication and/or what condition it is for.
  9. Include in your medication list, any supplements, over the counter medications and as-needed tablets, solutions, powders or remedies.

You have many things to weigh up, and make decisions about. If you have multiple issues, often one treatment can inversely affect something else, and we have to decide which is the most at risk.

Remember that they are working for you, and that the ideal relationship between you and your doctor should be open, honest, and equal. They should respect that you need to understand their decisions and why they are making them. Without understanding treatment, we can become anxious and not feel confident.

 

What Does YOUR Adrenal Crisis Look Like?

April is Adrenal Insufficiency (Addison’s Disease) Awareness month in many countries of the world so I thought I would look at what an Adrenal Crisis is to those that suffer with Insufficiency.

I don’t mean, what does the medical world consider a crisis, but at what point do those that suffer AI believe they are in crisis, and at what stage do they feel they need medical intervention.

Let’s start with what a medical dictionary says is a CRISIS:

According to one online medical dictionary:

crisis

 [kri´sis] (pl. cri´ses) (L.)

  1. the turning point of a disease for better or worse; especially a sudden change, usually for the better, in the course of an acute disease.
  2. a sudden paroxysmal intensification of symptoms in the course of a disease.
  3. life crisis. – addisonian crisis (adrenal crisis) the symptoms accompanying an acute onset or worsening of addison’s disease: anorexia, vomiting, abdominal pain, apathy, confusion, extreme weakness, and hypotension; if UNTREATED  these progress to shock and then death.

Alterntively the Oxford Concise Medical Dictionary (9 ed.) states:

  1. the turning point of a disease, after which the patient either improves or deteriorates.

Neither of these sources, or any other I can find, state that a medical crisis is only once the patient has entered a state of hypovolemic shock.   In fact, the first one states it only progresses to shock if a Crisis is UNTREATED.  Yet SHOCK is what the Dr’s wait for, or believe to be a crisis, in the ED when an Adrenal Insufficiency patient presents.

Professor Bruno Allolio stated in his document EXTENSIVE EXPERTISE IN ENDOCRINOLOGY – Adrenal crisis

Bruno Allolio1,2,†

Prevention of adrenal crisis

Physiological endogenous glucocorticoid secretion (normal Cortisol production) is highly flexible with rapid adjustments to unexpected needs….

In the foreseeable future (if ever), no replacement therapy will be able to fully mimic this amazing adaptive potential of a healthy HPA axis.  However, many stressors can be anticipated (e.g. elective surgery) and allow to adjust the glucocorticoid dose to the expected need, thereby preventing the occurrence of clinical deterioration and adrenal crisis.  It is important to understand herein the underlying concept.  The recommended dose increase is not intended to mimic the median cortisol increase in healthy subjects during such procedures.  Instead, it is intended to mimic the maximum cortisol increase, which may occur in euadrenal (normal functioning adrenal gland) subjects triggered during these procedures, potentially induced by some unforeseen events (e.g. postoperative bleeding).  Patients with intact adrenal function can respond immediately to such problems with an increase in adrenal cortisol output.  By contrast, in adrenal insufficiency, additional glucocorticoids would only be given when clinical deterioration becomes evident.  Until then, valuable time may have been lost to adjust the hydrocortisone dose to the increased need.  For that reason, dose adjustments aim at the upper limit of the normal variation to cover such unexpected needs.

As infections are the most frequent cause of adrenal crisis, it has been suggested that the patient doubles the hydrocortisone dose if the body temperature increases above 38.0C and triples the dose above 39. 0C.  This dose is maintained as long as the fever persists and rapidly (within 1–2 days) reduced to the standard replacement dose after recovery.  Gastroenteritis poses a particularly high risk, as glucocorticoid availability may be compromised by vomiting and diarrhoea, while the demand is clearly increased.  Thus, early parenteral hydrocortisone (100 mg subcutaneously) is strongly recommended either via self-administration or by a physician.  This dose may need to be repeated and health-care professionals should be involved early for clinical assessment.  Similarly, in severe infection (e.g. pneumonia) with altered cognition, early parenteral hydrocortisone and medical help are warranted.

It has been suggested that patients today are at a greater risk of adrenal crisis, because they have less of a ‘cushion’ of excess circulating cortisol with a standard daily dose of 20 mg hydrocortisone compared with the old-fashioned standard dose of 30 mg hydrocortisone.  However, there is no scientific evidence for such a protective ‘cushion’ effect.  Instead, chronic over-replacement may rather increase the susceptibility to infection and thereby increase the risk of adrenal crisis.

Since his crisis in 2014 from pneumonia, we have learned when Derek is suffering low cortisol vs when he is needing medical assistance.  We have become very good at getting him to stress dose BEFORE he gets too sick.  He has had 3 infections in the last 2 years.  We have given him very high doses of steroids (up to 40mg in one dose over and above the 5 or 10 he would normally take).  What this meant was ignoring the Dr’s that stated that his first clear sign to US of Low Cortisol,

Doing this, and then getting him to a Dr to find out WHY he is so low, has saved us from having to use his emergency injection or taking him to the Emergency Department for urgent medical intervention so far.

That is not to say that one day we will get it wrong, or not be able to stop a crisis from getting to the emergency state and have to call an ambulance, but we are on the winning side at the moment.

It got me thinking what is it that tells an Addisonian (I am including SAI and PAI in this term for ease of typing) what are the first symptoms they have.  What is is for THEM that says, you need a high oral dose, or you need an injection or an ambulance.

Since Derek’s diagnosis in Oct 2012 we have learned a lot about AI.  The biggest thing we have learned is that NO TWO ADDISONIAN’S ARE THE SAME.  They all have different comorbidities, they all have different symptoms, they all have different knowledge levels, and most importantly they all have different support systems in place, from nothing, to fantastic.

All these things impact on how quickly things can go from good to bad to dead.

The final piece to this puzzle is the ability and knowledge of their endocrinologist.

  1. Have they been given the right advice on updosing and stress dosing, the right knowledge on when to use the emergency injection, have they even been prescribed the emergency injection (some don’t believe in giving it).
  2. Do they have confidence in the care they will receive when they call an ambulance or go to their Emergency Department.

These last two things can will make or break an Addisonian.

So this doesn’t tell us what an adrenal crisis actually is.  And this is the hard part.  I asked some of my Addisonian friends what their signs of crisis are.

Each person I asked gave a different answer.  The symptoms were very similar, but presented in a different order, different intensity, and different clusters.

Symptom #1 Symptom #2 Symptoms #3 Symptoms #4
· Feeling really tired    & listless and
generally blah
· Dizzy &
unbalanced
·  nausea
·  Hot and Sweaty
·  raging migraine
·  high BP
·  So tired just want
to sleep
·  seizures
·  Extremely fatigued,
·  lifeless legs
·  Dizziness
·  pain in abdomen,
·  low back and legs.
·  tachycardia
·  mostly high BP,
·  then it can plummet
·  Disorientated
·  Belief that rest will
fix us.
·  severe torso
spasms
· dizzy,
·  start to stagger,
·  ose balance,
·  barely lift feet, legs
feel like heavy jelly,
·  feel shaky,
·  nauseous/dry             reach.
·  Big D,
·  hard to talk, words
just don’t come out
right.
·  Back pain,
·  torso spasms.
·  Extreme fatigue
·  Abdominal pain
·  Gas/wind
·  headache
·  Speech goes
quiet
·  confusion
·  High BP
·  Light headed
·  Wants to sleep
·  Inability to speak
·  Nausea

 

Each one of these lists shows, a sudden paroxysmal intensification of symptoms in the course of a disease.

Yet the Addison’s Disease Self Help Group in the UK, and the National Institute of Diabetes and Digestive and Kidney Diseases have different definitions of a Crisis.

Symptom ADSHG NIDDK
Adrenal crisis Warning signs include:
·    severe nausea
·    headache
·    dizziness
·    extreme weakness
·    chills or fever
·    confusion.
Symptoms of adrenal crisis include
·    sudden, severe pain in the lower back,        abdomen, or legs
·    severe vomiting and diarrhoea
·    dehydration
·    low blood pressure
·    loss of consciousness

 

All the above experience the symptoms from the ADSHG, but they all also inject BEFORE they get to the severe vomiting, diarrhoea, and low blood pressure if they can.

These symptoms are those experienced with an infection.  A gastric bug is a whole different issue.

These 5 people know, from several years of experience, when they can take in more oral meds, vs when they need to inject.

Yet if they went to hospital, they would be told that they are not in crisis as they are not vomiting, their blood pressure is not low, and they are conscious.

If you break your leg, it’s obvious by Xray (mostly) that it’s broken.  If you have a heart attack, they can show it within minutes, stroke – signs and symptoms are clear, Hypo or Hyperglycaemic they test and they know what to do.  Arm sliced open and bleeding?  Dr’s, Nurses, and EMT’s know immediately to stem the flow of blood BEFORE shock sets in.

But when it comes to Adrenal Crisis there is no “test” they can do, many in the medical profession throughout the world don’t recognise it, don’t believe the patient (or their advocate) when they present in crisis, and unfortunately still don’t know how to treat it.

Many also won’t follow written instructions the patients carry, when you give them information.  We have seen people turned away being told “you are not in crisis”, but they clearly are.

Derek was at an after-hours Dr one day as we though he had a throat infection.  He couldn’t feel much pain as the infection was on the left side of his throat, which is numb from CAPS, and he can’t swallow on that side.  During the examination the Dr said “I don’t believe you need an emergency injection.”  My immediate response was “No, he is not in crisis.  IF we thought he was in crisis we wouldn’t be at After-Hours, we would be at Hospital.”

The Dr acknowledged we obviously knew what we were doing.

Another time we said to the Emergency Room Dr that Derek’s AI was under control, we were not there for that, we were there for another reason.  The Dr was happy with that and dealt with the issue we were there for.

One time that he was dropping fast into crisis, couldn’t keep her eyes open, couldn’t answer any more than yes/no answers, was in a lot of abdominal pain, was nauseous.  Yet the Dr had no idea, even when I pulled out all Derek’s medication and said I was giving him 20mg, the Dr’s question was “Do you really think he needs that much.”  And left his cubical Um, yes, he needs a lot more than that.

They believed us quite willingly when we said he was not in crisis, but when we knew he was heading that way, they didn’t believe us.  The 20mg didn’t do anything.  After finding an old letter a senior Dr from that ED had written, and giving it to a nurse, they finally gave him 100mg and admitted him.

So what is an Adrenal Crisis?

The image here shows THREE pathways to death from adrenal crisis.

One friend spent years having seizures during adrenal crisis.  Yet she was frequently told seizures were NOT part of the adrenal crisis pathway (Far Left Pathway, bottom symptom).

Others have been told, you can only be in crisis if you are vomiting (ONLY middle pathway mentions vomiting).

 

With the 3 pathways in adrenal crisis, and you can be suffering a mixture of symptoms from each.  You do not have to follow only one pathway.  Most medical books only talk about the middle pathway, and don’t look at the two outside ones.

Derek vomited a total of 4 times during his adrenal crisis and multi organ failure yet he was sick/near death for 3 weeks.  He did not vomit at all while in hospital.

If each Adrenal Insufficient patient presents differently, how are the medical world supposed to know what is wrong.

One size does not fit all.  And THAT is a serious problem, with no easy answer.  If someone seeks medical help for anything and they have Adrenal Crisis, they need that checked first.  The Dr need to ask the patient, do you think you need/have you taken emergency medication.

Most patients know if their oral steroids aren’t working.

Do you know YOUR signs?

Does your support person?