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.

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Dehydration in Adrenal Crisis

When your sodium is normal, but you are dehydrated.

 


Dehydration does cause the serum creatinine to rise and the estimated glomerular filtration rate (eGFR) will, accordingly, fall. The degree of change is generally proportional to the degree of dehydration. Severe dehydration can actually cause acute kidney injury and may lead to a need for dialysis therapy.
National Kidney Foundation.


Water, water, every where,

And all the boards did shrink;

Water, water, every where,

Nor any drop to drink.

The Rime of the Ancient Mariner (text of 1834) Samuel-Taylor-Coleridge


Within the support groups there is always talk about dehydration being a sign of crisis, or impending crisis (or pre-crisis if that’s what you prefer to call it).  To decide if you are in crisis, your electrolytes are checked.  The standard sign is low sodium of <135 and/or high potassium of >5.2.  The problem is when you don’t fit this criteria.

I hear many times, people believe they are in crisis, they feel horrible, they feel sick, they feel dehydrated, but when they present at ED they have their bloods taken, and are told they are “normal”, they aren’t dehydrated and therefore they can’t be in crisis.

What is being tested is sodium and potassium.  I don’t often hear talk about creatinine.

Yet creatinine is the better teller of dehydration.  Derek has had 3 crisis since diagnosis.  The first two I didn’t get the blood test results for.

The 3rd one, because it was 18 months after diagnosis, I knew Derek’s normal blood levels.  I was recording them on OneNote so I had them with me wherever I was, whenever bloods were taken.  So I asked the Dr what Derek’s blood results were.

She insisted they were “all fine”.  I kept pushing requesting very firmly that she told me the levels.  She kept saying she couldn’t remember them, but they were fine.  After pushing more and explaining that I knew what his normal was, so I would like to compare them, she finally told me that one was creatinine.  She remembered this number because it was high, which meant his kidney function was low.

When I pointed out that it was changed and higher than his normal level she actually argued with me that it was better than the one she compared it to.  I asked the date of that one.  It was compared to when he was in hospital suffering  CAPS and his worst and most life threatening Adrenal Crisis, before diagnosis.  She wasn’t interested.

She was a Resident, which means she was learning how to be a Dr, she was a first year resident, so some basic knowledge should still have been in her brain.

Although at that time I knew Derek’s numbers, what I didn’t know was what those numbers meant.

After some more toing and froing we finally got Derek some SoluCortef, but still no fluid because his sodium had come back in the higher end of normal.  I have since learned that his potassium was just above range and higher than normal for him.

The next day when the General Medical Consultant did ward rounds, his first comment was “So, pneumonia and adrenal crisis, and you are dehydrated.”

He had looked very quickly at a vein in Derek’s neck to confirm, but he was also looking at the blood results.

After I got home I started investigating what it was, when sodium was normal, that told him Derek was dehydrated, and why drinking water hadn’t helped.

Here’s what I found out.

Why Sodium and Water may not help. A very simple explanation.

Although Sodium can be a sign of dehydration, when you are on fludrocortisone sodium can be kept within range, but it won’t stop the kidneys from functioning below par.

When creatinine is higher, and therefore eGFR is lower than YOUR normal, it is a commonly known indicator of dehydration.  The lower the eGFR number the more dehydrated you are.

But why don’t sodium and fluids help?

If you are really suffering low cortisol then that same low cortisol stops your body absorbing fluids so you not only need sodium, but you need cortisol, to keep you from becoming dehydrated.  This is where Blood Pressure comes in.  When you are first dehydrated, your body kicks in with a lot of other system mechanisms, to keep your blood volume up, hence you don’t suffer low BP immediately.  It is the dehydration that determines when your blood pressure lowers.

The reason HC works to lower your BP is because it helps your body absorb the fluids it needs to increase blood volume, and thereby reduce the other mechanisms from activating, to try and balance things out.  This won’t necessarily stop the crisis, but it will help you long enough to get more help.

So if you are told you can’t be dehydrated because your sodium isn’t low, ask what your creatinine (or eGFR) is.  If they don’t know, ask them to find out.  If they still argue, ask them what anti-diuretic hormone does, and the effect of that when you have low cortisol.    It is one of a number of the mechanisms in your body to keep your blood volume up when dehydrated.

Generally, a high serum creatinine level means that your kidneys aren’t working well. Your creatinine level may temporarily increase if you’re dehydrated, have a low blood volume, eat a large amount of meat or take certain medications. – Mayo Clinic Website.

 

Back to Derek

When Derek is dehydrated he slips from Stage 2 to Stage 3 CKD.  We have to monitor it regularly.  IF you don’t know what your normal is perhaps you should find out when you are feeling healthy.  This could save you a lot of medical issues when you are sick.  And check out my post on “know your own normal” because sometimes, it too can save you.

One thing I remember is when Derek was in hospital in crisis before diagnosis, after 3 days they had to stop the fluids as his body was swelling up, and wasn’t absorbing the fluids.  His kidney’s were failing, his blood pressure had started going down, and fluids were not working as he was just absorbing into tissue, his blood volume was going down.

A number of people have asked why they get fluids and fee better, before they are in crisis.  these people still appear to have a very small amount of cortisol production.  It may be that it’s enough to help absorb the fluids.  Derek has no production, so at the time his body had nothing, including aldosterone, to help.

 

Chinese Whispers and Medical Research

 


Reminder, we are an AI patient and wife, not medical scientists, or medical Doctors.  This is based on our own observations and experience even though we do discuss legitimate research here.


The origin of the term “Chinese Whispers” isn’t clear according to the wonderful Internet.  It has also been called “Russian Scandal” or in the US “Telephone”.  We all know what it is and have probably played it.  You say a phrase or sentence, whispered into some’s ear.  They then, without confirming what was said, repeat that phrase to the next person.  This continues until everyone has heard the phrase once.

The challenge is to pass the message through a number of people without it becoming misheard and altered.  The enjoyment of the game is that regardless how careful you are, the final message heard invariably is not what was originally said.   It is used to show how gossip can affect outcomes, how we feel, what we believe, who we trust.  The message being, if it isn’t from the horses mouth, then is it correct.

But were you aware that it can also happen with the written word.  Someone writes something, a person looks at it, takes from it what THEY are looking for, and ignores something that although the author thought was relevant, the reader felt was of no significance.

A good recent example would be from a New Zealand Senior School Exam question.

The exam asked pupils to write an essay on the following question: “Julius Caesar once said, ‘Events of importance are the result of trivial causes’. With reference to the causes and consequences of a historical event you have studied this year, analyse the extent to which you agree or disagree with Caesar.”(Otago Daily Times)

The students sitting the exam had a serious issue with the word Trivial.  Apparently many of them didn’t actually know what the word meant.  I wasn’t so much concerned with the question (or the word Trivial) but with the quote itself.  It is a 1980’s rewrite of the original and its context has slightly changed with its reinterpretation. 

10 years ago, I would have taken what was written in research as correct, accurate and as it’s peer reviewed, good methodology.  But in fact, it’s not always.  In reading research for Adrenal Insufficiency, Derek and I have discovered this happens in referencing and “quoting” research as well.

But why is that important?

It doesn’t seem important at all, in fact some could say it’s trivial.  But it is those trivial little re wordings’, the leaving out of part of a statement of the not reading the entire document and therefore missing an apparently trivial piece of information, that can have a big impact on how people are treated, and how their Dr’s will, or won’t listen to them.

One example we are looking at currently is dosing.  What is better?  Twice a day? Thrice a day?  Four times a day?  Maybe even five?

Derek is currently researching the origins of the belief that 15-25mg HC is all that is required.  In doing this, we have come across a number of discussions around how many times a day to dose.  What we have found is inconsistencies, misquotes, and lack of acknowledgement of some of the results from an original paper that is quoted.

The Original Paper:


Ekman, Bertil & Bachrach-Lindstrom, Margareta & Lindström, Torbjörn & Wahlberg, Jeanette & Blomgren, Johan & Arnqvist, Hans. (2012). A randomized, double-blind, crossover study comparing two- and four-dose hydrocortisone regimen with regard to quality of life, cortisol and ACTH profiles in patients with primary adrenal insufficiency. Clinical endocrinology. 77. 18-25. 10.1111/j.1365-2265.2012.04352.x.
https://www.ncbi.nlm.nih.gov/pubmed/22288685

Results:

The four-dose regimen gave a higher serum cortisol before tablet intake in the morning (P = 0·027) and a higher 24-h cortisol (AUC) (P < 0·0001) compared with the two-dose period. In contrast, a lower median plasma ACTH in the morning before tablet intake (P = 0·003) and a lower 24-h ln (ACTH(AUC) ) were found during the four-dose period. The patients preferred the four-dose regimen (P = 0·03), and the HRQoL scores tended to be higher (high score indicates better HRQoL) for the four-dose period. In summary, a four-dose regimen gives increased availability of cortisol and an enhanced effect with a less elevated ACTH in the morning in comparison with a two-dose regimen but the effect on HRQoL remains inconclusive.


The final statement is rather subjective.  The patients FELT it improved their QoL, but statistical analysis of the questionnaires could not support how the real life patients perceived it.  Is that a fault in the questionnaire rather than the actual results?

Articles (peer reviewed and published) citing the above reference and “quoting” the conclusion:

Paper 1


Amir-Hossein Rahvar, Christian S. Haas, Sven Danneberg, and Birgit Harbeck, “Increased Cardiovascular Risk in Patients with Adrenal Insufficiency: A Short Review,” BioMed Research International, vol. 2017, Article ID 3691913, 5 pages, 2017. https://doi.org/10.1155/2017/3691913.

https://www.hindawi.com/journals/bmri/2017/3691913/

Quote

Nonetheless, all regimens used so far fail to exactly mirror the physiological circadian rhythm, thereby having a negative impact on the metabolic system. However, other approaches like using a four-dose regimen were not able to show significant changes in quality of life, body weight, blood pressure, or glucose levels compared to a two-dose regimen [15*,16].


This report focused on the results of a small group over 8 weeks with 2 different dosing regimes.  What they didn’t mention was the significant variation + of the:  Free urine cortisol nmol/24-h 2 doses (337 ± 173) vs 4 doses (330 ± 93).  Although the mean was close, the variation was very wide and over time, could this have a higher impact on other health outcomes.  Also the preference to four doses a day was significantly higher than twice a day dosing preference for quality of life.  Without the full document, and the figures, we could believe the above statement in it’s entirety.

Paper 2


Stefan R. Bornstein  Bruno Allolio  Wiebke Arlt  Andreas Barthel  Andrew Don-Wauchope Gary D. Hammer  Eystein S. Husebye  Deborah P. Merke  M. Hassan Murad Constantine A. Stratakis  David J. Torpy. (2016). Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. The Journal of Clinical Endocrinology & Metabolism, Volume 101, Issue 2, 1 February 2016, Pages 364–389, https://doi.org/10.1210/jc.2015-1710

https://academic.oup.com/jcem/article/101/2/364/2810222

Quote

One double-blind, randomized, crossover study evaluating two-dose vs four-dose hydrocortisone treatments (98) concluded that cortisol pharmacokinetics were more physiological on the four-dose regimen; surprisingly, participating patients preferred this regimen.


The preference was a statistically valid conclusion, yet this group were surprised at the result.

They appear to have got the information right yet although the document notes that HRQoL scores tended to be higher indicating a better HRQoL with four doses a day they still only recommend 2-3 doses a day, with a rider that “high frequency regimes and size-based dosing may be beneficial in individual cases”.   They do recommend 3-4 doses a day for children.

Derek and I use this paper a lot the good work in it.  You just have to know if using it for your Dr, the parts to point highlight.  If you are in the US and you see a lot of non US researchers listed don’t worry as NADF do approve and reproduce this document for use within America so it is valid there as well.

Paper 3


Jitske Tiemensma, Cornelie D Andela, Ad A Kaptein, Johannes A Romijn, Roos C van der Mast, Nienke R Biermasz, Alberto M Pereira. (2014). Psychological morbidity and impaired quality of life in patients with stable treatment for primary adrenal insufficiency: cross-sectional study and review of the literature

in European Journal of Endocrinology. https://doi.org/10.1530/EJE-14-0023

https://eje.bioscientifica.com/view/journals/eje/171/2/171.xml

Quote

QoL did not differ between patients on a four-dose regimen and patients on a two-dose regimen, but patients on a four-dose regimen tended to report better QoL .


This one has me a little confused.  One sentence which states QoL did not differ, AND reported better QoL?

Paper 4


Frédéric Castinetti, Laurence Guignat, Claire Bouvattier, Dinane Samara-Boustani, Yves Reznik. (2017). Group 4: Replacement therapy for adrenal insufficiency. Annales d’Endocrinologie. 78. 525-534. 10.1016/j.ando.2017.10.007

https://www.em-consulte.com/en/article/1184799

Quote (invalid cite?)

Hydrocortisone must be delivered in 2 or 3 doses per day, the first and highest dose on waking and the last (in the case of a 3 dose regimen) 4 to 6 hours before going to bed. An international cohort study of 1245 patients with primary adrenal insufficiency (84%) or secondary adrenal insufficiency (16%) underlined that the majority of patients were on a 2 or 3 dose regimen (42% and 32%) while other regimens were less common (one dose, 10%, other regimens, 17%) [2]. Neither of the two most common therapeutic regimens were shown to be better, but the number of comparative studies is quite small [21, 32, 33]


One paper references the original research to justify 2 or 3 doses a day, yet the original document had no mention of 3 doses a day.

Paper 5


Forss M, Batcheller G, Skrtic S, Johannsson G. (2012). Current practice of glucocorticoid replacement therapy and patient-perceived health outcomes in adrenal insufficiency – a worldwide patient survey. BMC Endocrine Disorders 2012 12:8. https://doi.org/10.1186/1472-6823-12-8

https://bmcendocrdisord.biomedcentral.com/articles/10.1186/1472-6823-12-8

Quote

The results from this survey are in line with a recently published clinical study [24] which showed that a majority of the patients preferred the four-daily dosing regimen to twice daily when comparing equal doses of hydrocortisone given either twice daily or four times daily. The reasons reported were less fatigue, more alertness during the day, less headache and a feeling that the treatment effect was less varying during the day. The patients had complaints after the study that a four-dose regimen may be difficult to manage in the long run [24].


This final document quotes things correctly and picked up on the information buried within the report.  It looks like they may have actually read the document properly.

My take on this. 

If your Dr had only read the original document, he would have no problem if you wanted to move to 4 doses a day, especially when you aren’t increasing your dose, but spreading it out. It was also noted that on 4 doses a day, there was the potential to reduce your overall daily dose.   Yet if they read some of the other more recent documents they would say there is no difference, so why change?

Chinese Whispers?  Or just misinterpretation?  This is just ONE example Derek and I have looked at.  There are many more out there.  And these changes in wording, or missing wording, can actually have an impact on a persons’ health and quality of life.

Next time a Dr tells you that “according to X research, Y is the case” ask them if that is the original research, or someone’s interpretation right or wrong, of that research.  Then check that he has read the original research or just the abstract?   The answer to the latter question will probably be no, because in a busy practice, Doctors don’t have time to read all the research.  It is up to the well-educated/informed patient to politely assist them by highlighting the significant parts.  That is, if the Doctor is happy to work as part of a team, with your best health outcomes in mind.

 

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.

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.

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.