Still a Single Road at Times

Aside

In 2013, my second post was very short.  It was entitled “As single road at times”.

My posts back then were very short.  They were a lot about how I was feeling, how we were learning to live with this condition strange new called Primary Adrenal Insufficiency (Addison’s Disease).

It was May 2013 that I began to Blog.  In the last 6 years a lot of things have changed.  But a lot have stayed the same, we have just learned to accept them and adjust to them.

Going to live performances, etc alone has become a new normal.  I buy 2 tickets, and find someone to join me if Derek isn’t up to it.  This could be a big show, or one that our children are performing in.  Adjusting things so that we can go together has also become a “thing”.  Whether that is by buying a matinée performance ticket instead of a night time one, or making sure he has a full day of rest, including a sleep, we will do what we need to, to keep going.

In May last year Derek had a set back.  It was serious but it wasn’t obvious.  A few blood tests went haywire for no obvious reason, he became a lot more tired than normal, and other subtle differences occurred.  Despite this we carried on with life.  We continued planning our big adventure overseas.

We did the trip.  It was extremely tiring on Derek.  We did a lot of walking with him using a walking stick constantly.  We would walk, stop for coffee, walk, stop for a cold drink, walk, stop for a few photos, walk, stop for an ice cream (they make great ice cream in Europe).  You get the picture.

Derek has been more tired than usual since getting home.  It’s now 6 months since the trip and he is still a lot more tired than he was before May last year.  So we have made more adjustments.

We have learned from that trip that you have to choose the fight.  So now, if we are going out and we know it will be stressful, or very tiring on Derek, we use a wheelchair for him.    After borrowing one for an evening we knew would be too hard on him without, he finally agreed that he really did need it.  But not all the time, just for the harder events.  It means that sometimes he can go out, and we can enjoy ourselves together.   I still go out on my own because he is not up to it.  Or we cancel our plans because I don’t want to go alone.

What we thought was just residual tired from the trip appears to not be that at all.  After having more “crazy blood tests” we began to wonder.  We have now looked at his bloods for the last 12 months, what ever is wrong, has been wrong since his various blood levels went haywire in April/May last year.  But that’s ok, there is nothing we can do except keep an eye on his levels so that is what we do.

The reason we know things have not be right since May is that we keep track of all his blood tests.  We don’t need to go the Doctor to ask, we can see.  We know the symptoms, we know the signs and we can see his “normal” is changing and not for the better.

Having said that, in the words of a good friend who writes an amazing blog, he is clearly alive and will remain so.

Advertisements

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.

A Presumptive Diagnosis

Aside

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

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

“WELL IN MY EXPERIENCE”

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

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

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

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

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

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

 Let me give you an example.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

On the morning of 17 Oct he had a CT.

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

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

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

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

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

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

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

“In my experience”

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

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

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

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

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

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

Know Your Own Numbers

Aside

Every few months we get a question from someone on a forum as to what a normal BP or Temperature, or blood result is.

Everyone will jump in with one of two answers:

  1. The books say…….
  2. Mine is……

Both of these are valid responses. However, the book “Normal” is simply what the middle of the Bell Curve is.

Most “normal” numbers are taken from a group (be it large or small) and are based on a Bell Curve of averages.

 

Bell Curve

In this image, all areas are “Normal” according to a “range” but where you sit will differ to the person sitting next to you.

The first thing Derek and I learned when he was diagnosed with AI is that there is no Strict Normal.  Most people will sit within a range, but they will not have identical numbers.

When you are sick, the first thing they check is BP, Temperature, Heart Rate and Oxygen Saturation and, if you are really lucky, they will do a few bloods.

That is great, but what if you normally sit in the High, and you are feeling off and now sitting in the Low end of the range. Although it might not be screaming “Warning… Out of Range for the BOOK”, if you know your own numbers, you can scream “Warning… Out of Range for ME!”

When Derek had pneumonia in 2014 he went into crisis before he showed real signs of being unwell. For him Adrenal Crisis (or low cortisol) can be his first sign of being unwell.

In 2015 when he got pneumonia (yes he has had it every year since 2012) we knew his “Normal” and once his temp showed to be out of HIS normal range we sought treatment. The problem is, for him normal is 35.9 so once his Temp hit and stayed at 37.3 (considered perfectly “normal”) we knew he had some sort of infection so sought help. We kept him out of hospital because the temp was one of the first signs. We knew HIS “normal” so were able to run with that, and get treatment underway before he became too sick.

The same thing happened this year with a throat infection. Because we know what his “normal” is, we could see that some things were off so he was already taking a bump in Hydrocortisone.

The problem was, he has no feeling on one side of his throat so by the time he had a sore throat he already had a throat infection. But, it wasn’t’ crazy out of control because again, he knew his normal readings and was already taking HC to fight what ever it was he was fighting.

How do we know his “normal”. Quite simple, we did regular checks of his BP over a 3 months period. And by regular I mean 3 times a day every day. And we charted it. We also did a 10 day Basil Temperature Check. We keep all his blood tests. I know it sounds crazy but what that means is we knew when:

  • he was over prescribed his Fludrocortisone when he first started it;
  • the fludrocortisone was becoming less affective, and therefore it needed to be increased;
  • he is low on cortisol rather than just tired;
  • he is low on salt and needs to get some more into him;
  • he has an infection that needs treatment;

Why is knowing YOUR numbers important?

If you have a chronic illness, YOU, not the Dr’s know when you need to seek help, and when you can manage it at home.

If you rock up to the hospital with a BP of 90/60 according to the books it is not a problem (maybe you need to drink a little more), but if your normal BP is 135/85, then 90/60 is a big difference and there is probably something wrong. Also, you have probably already tried drinking water. For some reason many Dr’s seem to think that you are an idiot because you haven’t been to medical school and they have (especially the young, newly trained or still training ones). If you can then turn to them and say “I know my normal, I have tried correcting this myself, I need help” then perhaps they will listen to you.

We had to do that once. A Dr tried to tell us that a creatinine level of 125 was “normal” for Derek because he had that level once before (yes, 3 months after kidney failure, which had been 18 months previous). We knew that for him normal was 110-115. She ignored us, and didn’t treat the dehydration (the 125 was a text book sign). He was admitted to hospital in Crisis and the next day a senior Dr came in, took a look at his bloods and said “put fluids up, he is dehydrated”. The same resident did not bother comparing a chest xray from when he was well, to the one she had taken that night. Had that occurred, she would have seen he also had the beginnings of pneumonia. But because she didn’t know HIS normal, she missed everything.

I wrote a complaint, not because she didn’t know his normal, but because she wouldn’t listen to us when we tried to tell her his normal.

Taking time when well, to find your normal may possibly save you a lot of stress when you are not “normal” for you.

And Another Cure Emerges.

Aside

Again, the Adrenal Insufficiency Community have been notified of another Cure (actually the same “cure” just by another Quack.

I am not going to post a link to the site, but I have written an open letter to the person that perports to have a cure.  If you read the site, you can tell he has simply gathered other peoples opinions, wrapped it up in pretty fonts, and spat it back out.

My open Letter  Please feel free to share this around.   I will post if he ever replies (which we all know he won’t).

“An open letter to Josh Axe

I am loath to call a quack a Dr even if you managed to pass your thesis. What was it on by the way as it was clearly not on Adrenal Insufficiency.

I have been given, by someone concerned about my husband’s health, a copy of the information you post on your website, regarding Adrenal Insufficiency, and your belief you have the cure.

Having read it, I have serious concerns.

  1. You are not a medical practitioner, but feel you have the right to give dangerously inaccurate medical advice.
  2. You clearly have NO understanding of Adrenal Insufficiency.

Let me help you out.

You state “One type, referred to as Addison’s Disease, is caused by illnesses of the adrenal glands themselves, including adrenal cancer, infections or bleeding (called “primary adrenal insufficiency“).” I have to ask why, in your page, this linked to Secondary, not Primary insufficiency?

The Adrenal Glands are not “Ill”. They have been attacked by an Autoimmune response. This occurs over a period of years in some people. With a healthy diet (such as you suggest) you may be able to prolong the final outcome of death due Adrenal Crisis, but you can not stop the destruction of the gland and the inability of the adrenal glands to produce cortisol. Even experts in the field do not know what causes the autoimmune response that kills the Adrenal Cortex, how do you have the gall to pretend you do.

Once diagnosed, a person normally has less than 90% of BOTH glands not functioning. Without replacement steroids FOR LIFE you will die.

You state: “If you visit a doctor about adrenal insufficiency (including an endocrinologist who specializes in hormonal diseases), she or he might recommend you take medications to help speed up the healing process and reverse cortisol deficiency symptoms. Taking glucocorticoid medications like prednisone or hydrocortisone are the most common treatments, and the dosage of these depends on your level of stress, any present infections, medical history and injuries. The primary reason for taking hormonal replacement medications is to bring your cortisol and other hormone levels up to normal as quickly as possible.”

Whilst on the surface this appears correct, your conclusion is erroneous, and dangerous. Steroids are not given to “begin the healing process”. They are given because without them, you WILL die. They are not a quick, short term, or temporary fix. They are life saving and permanent. The only remotely true parts of your statement are “bring your cortisol” …”levels up to normal”.  It is done as quickly as possible because if they give you 1 mg a day, it would not keep you alive. The minimum dose for an adult with Adrenal Insufficiency either Primary OR Secondary is around 20mg/day (very few survive on less, and get out of bed).

You are correct that the dose varies depending on what stress is in your life, but what you don’t say is that that stress can include an infection, a cold, a broken toe, or a serious fright because you were witness to an accident (you don’t actually have to be in the accident). All of these “stress’s” are going on around you at all times, and you are not able to avoid them. The healthy person will produce more cortisol in their body. Those with genuine AI will not. No amount of healthy eating, meditation, vitamins, will remove these stressors from your body, and if you do not receive adequate steroid cover during these times, you can die. This is called “stress dosing”. Again I will tell you, normal people do this without knowing. You do not make your adrenal glands tired by producing more when you need it. That would be like saying that you will make your heart tired and cause it to stop beating, by working out at the gym or playing sport.  Would you tell an insulin dependant diabetic that they don’t need their insulin because they just need the right nutrition. I hope not.

You promote Licorice as something to take to assist in this miraculous recovery.  You have clearly copied this of someone elses website as they took one very small part of a study and spouted this drivil as well.   Licorice will increase the time that cortisol stays in the body, however, you need cortisol in the body to start, and once you have what you call severe adrenal insufficiency, which you are normally diagnosed while in ICU by real medical professionals (not someone with a Doctorate in Chiropractic) trying to save their lives, there is no option but to take REPLACEMENT steroids. This is not touted as a cure, but you are replacing the steroids you do not produce yourself. THEY ARE ESSENTIAL IF YOU WANT TO LIVE.

I do not have a Medical Degree, I also do not have a piece of paper telling me I know about nutrition. What I do have, is a husband with Severe Adrenal Insufficiency. This means that for the rest of his life, he must take steroids and that no matter what his diet is like (and we have already ensured he eats correctly, he will never be able to stop taking steroids because his adrenal glands will never recover.

What I am sick to death of is people like you, pretending to be Medical Professional’s, and spouting your drivel, for others to constantly ram down our throats, about how he can get off his steroids, all he has to do is change his diet. He would be dead in 3 days without steroids, and after the first 12 hours, he would not be able to eat your lovely food suggestions, or take your vitamins etc, as he would be so unwell, all he would be doing is vomiting, and suffering constant diarrhea everywhere.  I know, because I have seen it when he goes into crisis from something as simple as a common cold.

What you put on your website is dangerous and has the potential to kill people. We have lost enough people in the AI community who are miss treated by medical professionals without people like you jumping in to an area you have NO knowledge about, no authority to lecture about, no qualification in and selling your snake oil cures.

If someone who has studied Endocrine disorders as part of the Medical Degree, does not understand completely, the dysfunction of the HPA Axis, how do you, someone who has no real medical training, feel you have the right to spout your cure that will take “Up to 24 months for severe adrenal insufficiency” on a publicly available website.

Your site is tantamount to attempted murder and placing a disclaimer, in light blue on a dark blue background so you think you have covered your ass legally does not take away your gross negligence.

In conclusion, Adrenal Insufficiency is a genuine, life threatening medical condition. It is not a condition to be taken lightly as you do, it is not a condition to be treated with contempt, as you do, and it is not a condition that you are qualified to comment on. It is so far out of your scope of practice that you are going to be reported to the FDA if your information is not taken down.

P.S. There is a bet on in the Adrenal Insufficiency community that this letter will be ignored by you, as you avoid anyone who points out your incompetence. Feel free to prove us all wrong by acknowledging this email.”

 I doubt very much I will ever receive a response to this.  It will be trashed before it makes it to his personal inbox.