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 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.

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?

 

International Travel with a Chronic Illness

leaving-on-an-aircraft

Our Next Adventure Part 1

Having managed tripping up and down the North Island of New Zealand over the last 4 years, including flying, just to see how it would go, Derek and I want to venture a little further.

The Practice

To begin our preparation for an international flight, the first thing we did was try flying to Auckland.

It required a trip to the Airport, then flying to Auckland, and driving to Hamilton.

Derek took extra medication to fly.  He took extra hydrocortisone for the drive to Hamilton from Auckland.  He then lay down for a long rest when we got to Hamilton.  He also had to rest the next day, but that is normal when we travel any kind of distance.

On the whole, the trip was good.  We listened to others’ advice, had learned what Derek could manage, and when he needed to up dose.

Flying Further

This time we decided to go to Australia.  Originally our first trip overseas was going to be to Sydney or Melbourne for a weekend for a Show. After being offered a trip to Fiji earlier this year, and the realisation that a 2 day trip would be way too hard, we thought a longer trip would be better.  Because a friend with Addison’s was heading from Hong Kong to Brisbane for medical treatment, and we had friends in Brisbane, that was going to be our destination.

We are not the first to travel with chronic illness, and we won’t be the last.  When you look around the cue of people going to the flight, you don’t know who has a chronic illness, who has spent days and weeks preparing, and who has just grabbed a ticket and headed to the airport.

I asked my cousin, who’s husband has several serious medical issues, what they do to take a trip.

Trev just sorts all his medication and I just carry it…never had any problems, I think I have only been asked once and they were good about it. I never carry hospital documents but for you guys being a first since Derek has been sick, just get your GP to write a letter out lining the diagnosis and a list of the medications on the letter. Never really had problems with insurance just be up front about it all, you may not get full cover but shop around. We don`t stress about overseas travel, there is always a hospital where you go if things don`t go to plan. Just relax and enjoy the trip. We are probably not a good example…as we are pretty relaxed about it and just roll with it…lol…isn`t that naughty, but thats just us…great place we have just come back from there.

The trip they had returned from was a trip to celebrate 25 years since “Trev” had an organ transplant.  (By the way, I am totally in favour of organ transplants.  It saves lives, including that of my cousin’s husband so please think about donating organs if the unfortunate need should arise).

Booking the ticket.

We have made the decision to travel over to Australia.

Now What?

Do we need to get permission from the Dr?

We don’t believe so because we went to see her about going to Fiji with a work trip for Derek, and she said no, because of the risk of food poisoning, the possible need for INR while there, and the short time frame.

But she said that if we wanted to travel somewhere else, like Australia or England, then yes, she would make sure we could do it.

We bought the tickets. tickets

When I booked it, I also requested a wheelchair at both ends.  That meant an alert on the ticket booking, and I had to call a number, and speak to someone.

The flight is a Partner flight, which means we are booking through one airline, but the flight is provided through another, so there was a delay while the confirmed that the wheelchair was available.

We knew from experience going places with Derek he could not stand in line long.  He gets very fatigued just waiting at the supermarket, and if there are more than 2 in a line, he has to sit while we wait.

Then there is the timing of the flight.  We could fly out at 11am.  That would mean Derek could wake up as normal, and we could take our time.  But that also meant 9-12 hours flying as we would have to fly from Wellington to Auckland, wait for 2 or more hours, then fly to Brisbane.  We would arrive at night, and it would be a very long day.

That would take more out of him.

Alternatively we could fly out from Wellington at 7am.  And then fly for 4 hours, directly to Brisbane, and land at 8am Brisbane time.

This meant a lot less travel time, but a very early morning.  Derek normally wakes at 6am, takes his first round of HC, then goes back to sleep for an hour while he waits for his meds to kick in.

This trip would mean waking him at 4am to take his first round of HC, Drive to the airport, have breakfast, then check through Customs. (hopefully we haven’t got any fines that hold us back).

So next we book an appointment with the Dr.  We need:

  • A letter for treatment protocol (if he suffers an Adrenal Crisis)
  • A letter confirming he is able to fly (because of Antiphospholipid Syndrome and risk of DVT)
  • A list of medication (it needs to be declared at every port entry and exit).
  • Any meds he may run low on before he leaves, or soon after he gets back.
  • We also need to organise an INR when we land in Australia. This is the bit we are unsure of, but the essential bit to make sure it hasn’t dropped too low while flying, which could mean a blood clot.

Then there is Travel Insurance.

Normally you just buy it when you buy your tickets.  All you need for Australia is cover so if you get waylaid, you can get a change of ticket.  But if you have extra conditions, you need to tell them about them.  Then the cost goes way up.  It went up by $150 for Derek.

But we don’t know if we have declared everything as they didn’t have the ability to declare the catastrophic event he had.  And I hadn’t declared his prostate cancer.  Oh well, looks like a phone call.

I got two quotes.  So we needed to call both companies to see how things would change.

After a long phone call to each company, Derek’s insurance was going to cost $6 extra for the prostate cancer which is in remission.

Medication

Emergency Kit

The Take Every Where Kit

We would need a list to make sure we took everything.   It currently sat in multiple areas of the house.  We also needed to make sure it all had a proper prescription labels.

When my friend Wendy travelled from Hong Kong to Australia I told her to declare, declare, declare.  If she declared everything she would be fine.

I was going to work on the same principle.  But that is not the case for Derek as he takes DHEA (Dehydroepiandrosterone).  It is classed as an anabolic steroid, and therefore restricted.  Derek needs a licence to carry it into Australia, even as a prescription medication.

And one of his other medications needs authority to carry it out of New Zealand and then back into the country.

So, we have a Dr’s note x 2, we have an emergency letter, we have an application to take his medication into Australia.

We can only carry a month’s supply in and out of New Zealand but that’s fine because we are only away 6 days.

We have to wait to hear from the Dr regarding whether he needs 1 more medication for the trip, but otherwise, we have things ticked off that we need.

Getting all the advice is key to a successful trip.  So I went seeking advice.

Travel Advice from the Experts

The Addison’s Disease Self Help Group UK has some great advice:

  • Good general advice for long distance air travel includes:
  • Remember that air travel is dehydrating so you will need to drink more fluids than usual in the air. Drink alcohol, cola drinks, coffee and tea sparingly as these dehydrate the body further. If possible, carry a large bottle of water in your hand luggage. If you forget to bring your own water, be assertive about requesting extra refreshments from the cabin crew.
  • Walk around the plane as much as possible. Try to get up out of your seat every two hours to stretch your legs and keep the blood flowing.
  • Many chemist shops now stock knee-length support socks, which can help prevent the formation of blood clots that might lead to a deep-vein thrombosis (‘stroke’).
  • Adjust your watch to the time of your destination as the flight begins, and adjust your in-flight activities to that new time zone as well. Sleep through the in-flight meals, if necessary, to get attuned to the new time zone.
  • Try to book flights that allow you to arrive at your destination in the late afternoon or early evening local time, so that you get a night’s sleep at the end of your travelling. Flights which arrive in the early morning local time will leave you tired after travelling but having to stay up all the day before you get a proper night’s sleep.

So we missed the last one.

But we are going to stay at the house of an Addisonian, so they will know that Derek will be going to have a sleep when he arrives.

So everything we can think of is ticked off.  Now to wait.

Now to wait for the actual trip.

We are excited about the trip.  We have thought of all the possible issues, and taken care of everything we can.  Derek isn’t being wrapped in bubble wrap, but we are reducing the risk as much as possible without saying, “too hard, not going.”

Next Chapter – The trip.

I am hoping this will be a very dull chapter with just the excitement of having the Sunday lunch with other addisonian’s, and enjoying visiting somewhere I have never been.  I won’t apologise if my after trip post is boring, as that would be the best trip ever.

 

 

 

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.

I have a cure for Addison’s

tui ad(Those of you that are from New Zealand will understand this ad, for others, it’s about the “Yeah Right”.  Basically, it means what is on the left, is a complete fabrication.)

I belong to several on line support groups for Adrenal Insufficiency (Addison’s Disease) to both help others with Adrenal Insufficiency (AI) and to get assistance with questions Derek and I have about AI.

One thing that annoys many who have struggled to get a true diagnosis, and the correct treatment for AI once diagnosed, is the people that post that they had AI, diagnosed by this herbalist, or that naturiopath, or some Dietitian with a Dr in front of their name and even once by a chiropractor. None of these people have access to the correct blood tests for a definitive diagnosis of either SAI or PAI. They then tell everyone how they have had this miraculous cure by neck manipulation, taking this herbal mixture, or that vitamin concoction, or eating this menu of food. They then try to convince those who have the condition, to stop their steroids, take these natural cures, and they will be cured. “Here, read this, it will fix you.”

If you truly believe you have this condition, go to the UK Addison’s Disease Self Help Group for a fantastic resource on how to get diagnosed, and treated.  Some people have difficulty finding a Dr that understands blood tests enough to correctly interpret the results, or that put their symptoms down to anorexia, depression, psychosis etc.

Here I am talking about, those who come in asking about everyone elses symptoms, so they can copy them, to make it sound like they have the condition.

I have also had people come to me personally and tell me that they heard about a natural cure for AI.

Some of the sites they refer you to look like genuine medical sites. They even claim to be by Dr’s.

And that is not to say that the “Dr’s” don’t have a Doctorate. One particular one has a Doctorate, but in fact it is in nutrition. But he has developed a great, natural cure for AI. He is not a medical practitioner, can not do blood tests (and does not recommend blood tests). He is a multimillionaire by selling “snake oil” aimed at those with “Adrenal Fatigue”.  Unfortunately people thing that Addison’s is simply Adrenal Fatigue and can be cured naturally.

Another such site was posted on one of the support groups recently. Not to encourage us to use the cure, but in share frustration that the member posting it, had yet again been told that they can stop taking steroids, and just eat natural herbs, vitamins, and other cures.

I have also heard someone was told that they could go through a IVIG (immunoglobulin exchange) and they could stop their steroids because fixing the immune system would fix the already damaged Adrenal Glands. Fixing the immune system is great, and can stop an inflammatory attack from doing further damage (it even be used during CAPS), but it will not repair the damage already done.

AI does not only last while you are suffering an attack by your immune system, it is the resultant damage. Even once the attack is over, the damage is permanent.

Sadly some people believe these sites, and can end up in Hospital, fighting for their lives.

The cure this week: Vitamins, Liquorice and Green Tea. (Warning Signs Of Adrenal Insufficiency And Natural Methods Of Treatment)

The issue I have with this one isn’t taking vitamins, it isn’t eating liquorice, or drinking green tea. All of things, in moderation, can be good for you.

The issue is people miss quote research, meaning their solutions can be dangerously wrong.

Grapefruit juice and licorice increase cortisol availability in patients with Addison’s disease: Paal Methlie1,2, Eystein E S Husebye1,3, Steinar Hustad1, Ernst A Lien1,2 and Kristian Løvås1,3

This study looked at the effect of Grapefruit Juice and Licorice on Cortisol.

Conclusion Licorice and in particular GFJ increased cortisol available to tissues in the hours following oral CA administration. Both patients and physicians should be aware of these interactions.

The most important thing about this study (which is what people use to say stop the cortisol and start eating licorice) is that it helps prolong the life of the cortisol in your body.

This sounds great, but it has been shown that over eating of such things as licorice WILL cause other medical conditions especially in Addisonians. The reason. Addisonians (especially primary) suffer from high potassium which must always be kept under control and licorice can decrease your potassium levels.  But there is a warning out there “Despite its apparent use in a few clinical scenarios, the daily consumption of licorice is never justified because its benefits are minor compared to the adverse outcomes of chronic consumption.” (Licorice abuse: time to send a warning message. Hesham R. Omar,)

The catch?   To help extend the life of cortisol in the body, you must first HAVE cortisol in the body. Addison’s Disease/Adrenal Insufficiency means you no longer naturally produce cortisol. What you have in your body is what you take via replacement therapy. With secondary AI you may actually produce a little, but not enough to live. It doesn’t mat­­ter if you don’t have a math degree, you should still know that 0 x anything will still be 0.

Are you getting the point yet?

You can’t increase the effect of something you don’t have. For this particular website to say you should stop taking steroids and start taking their “cure” is actually dangerous.

Education doesn’t always work

As said earlier, I have very recently been the victim of this myself. I spent some time explaining to someone, the problems with Adrenal Insufficiency, what it was, what Derek had to take to stay alive.

I have now been told twice by this person that natural treatments will fix Derek. I should get him off all his medication, and give him “Adrenal Support”, which I discovered when I looked up the product I was told about, was in fact animal adrenal glands with other herbs and vitamins.

Wow, if only. Unfortunately this was the “cure” from 1890’s to 1950’s when they found Hydrocortisone allowing Addison’s sufferers to actually be able to get out of bed (although some still can’t), a lucky some to work, and if lucky (and the medical profession don’t kill you first) to live a longer life.

I am sure I have said before, hydrocortisone is not a Cure for Addison’s disease. There is no cure. Hydrocortisone and fludrocortisone are what is currently used to keep the Addisonian Patient alive. The average Life expectancy is now found to be reduced on average but a lot longer than in 1942 when it was still less than 2 years and where if you had to have surgery, got an infection or even a tooth extraction, it would probably kill you.

Why, oh why, would anyone go backwards to “natural cures” that would if you were really lucky, keep you alive (but in bed and in pain) for no more than 2 years over the synthetic hydrocortisone that is available today.

If you are reading this please DO NOT tell your friend who has Adrenal Insufficiency, either primary or secondary, that you have read about a cure. You will only achieve 2 things.

  1. The loss of a good friend (if they were one to start with)
  2. Manage to make yourself look stupid.

In Dr’s we Trust?

Without really thinking, we do.  They are Dr’s, they know it all.  But putting all your faith in these special people, especially specialists, is not always a safe option.

We have been going around in circles trying to get more things sorted.  Derek’s lungs don’t appear working properly.  He gets breathless quickly, when talking, when walking, when laughing.  He is now worse than he was a year ago.

We used to walk 5.5km in an hour with no stops.  Now he walks that distance in 1 hour 20 minutes and he has to stop.

We are trying to find out why he is going down hill so have asked several medical people to investigate.

We went to a specialist Endocrinologist in June this year. One we have been to several times.   We mentioned this reduction in ability, but it was not recorded.   He spent a short time with us, discussed things, then wrote a letter to our GP.

This is an important letter as it tells Derek’s GP how he is doing currently with his health and his medication levels.

We have recently changed GP’s as our previous one has recently retired.  Therefore the new Dr needs all these specialist notes, to ensure she is treating Derek properly.

At the time of transferring, we decided to get a copy of all of Derek’s GP’s notes from his previous GP to see what had or hadn’t been noted.

What I found floored me.

We all put our faith in the medical profession getting things like medication correct..  But is that faith justified?

To put your all trust in what Specialists say (and they are supposed to be the all knowing Gods of Medicine), has again proven to be very dangerous.  Thankfully this time, we ignored what the specialist wrote, and the Dr didn’t bother reading what the specialist wrote.  Instead, the Dr asked US what medication Derek was on.

What the letter stated as Derek’s medication was  dangerously wrong.  This specialist  is the one responsible for making note of what Derek’s medication should be on a daily basis to keep him alive.

According to the latest letter to our GP (and the one that the hospital would read to confirm dosage) The letter states ….Hydrocortisone is 10 (5 at lunch, 5 at dinner)….

hc dosingThe letter should read 10mg HC in morning, 5mg at lunch and 5mg at dinner.  There is 10mg of Hydrocortisone daily missing.  It is half his daily dose to keep him alive!

If Derek took that low a dose he would not be conscious to take HC at lunch time on the 2nd day.  He takes 10mg in the morning (6am) rolls over to let it kick in, then takes another 5 before lunch time, and another 5mg at 4pm.

Derek has had blood tests done at 8am before his morning dose of HC and it was found that he had a level of  9nml/L in his system, the absolute minimum a normal person should have is 250nml/L.  It should actually be in the 500’s at that hour.  Less than that and you are probably in crisis.

Taking 5mg at lunch time, then 5 in the late afternoon would not be enough to keep him alive for more than a few of days, and those would be a major struggle to stay conscious.

Taking Hydrocortisone is not like eating extra food.  You can’t take your normal dose one day, stay in bed all day, and store it up, to use when needed another day.  It peaks at 2 hours post taking, and only lasts in the system for 4-6 hours.  Once that 4-6 hours is up you start to go down hill until you take your next dose.

Derek cannot get out of bed in the morning except to struggle to the toilet if he hasn’t taken any HC.

And the kicker is, the less HC you have in your system, the quicker you use it up as your body starts fighting to cope, so it use up what it has to function pretty quickly.

So putting on a legal document that he is only on 10mg a day when he struggles to stay out of crisis on 20mg, is a medical misadventure waiting to happen.

NEVER PUT ALL YOUR TRUST IN YOUR DR.  Always get a copy of your medical notes, and check them for accuracy.  They have a habit of not recording everything you say, interpreting it how they want so it fits the book, or just brushing it off.  Or worse, not checking their own notes, when writing to inform others of important information.

There is no excuse for getting critical details wrong when it could seriously harm someone

 

The Puzzel Pieces Don’t Fit!

Have you ever tried doing a jigsaw without the picture? You can put small groups of pieces together based on similar colour/shape/patterns, you can even put the edge together. But you end up with these small groups of pieces all over the place, but you can’t get them to make a clear picture.

You keep twisting and turning the pieces around and trying to fit them in different areas and in different ways, but they just won’t line up nicely. You think you have the right two groups together, then someone looks over your shoulder and tells you, no, that can’t go there because that piece doesn’t work. You end up with what appears to be something, but all together, make nothing.

That is what it is like with Derek. We have all the pieces of the puzzle. We have put them into small shapes, but we just can’t make the small shapes fit the big picture.

We always seem to be missing something!

Finally someone has come and put a group into the middle, that we left to the side. Suddenly it all fits!

Derek’s Addison’s is “under control” or at least as much as it will be. He takes extra medication when needed. His BP is reasonably stable most days. He is learning to cope with the extreme fatigue he suffers at times.

But that didn’t explain the bad headaches, the lack of cognitive skills which make him tired, the inability some days to do basic math when he has a Math’s and Computer science degree.

Recently I did a post called “It’s all in his Head” in which we were told that there “could” be a brain injury. Today we had it confirmed.

He had a simple neurological test. At the end of it, the Dr that was testing him said he had to go to see a Neurologist, as it was above his pay grade.

He failed the test!

The Dr deals with all types of Head Injury rehabilitation, but is not a Neurologist. He has pinpointed the damage to the Frontal Lobe somewhere. Now we have to find out where.

We also now have to “hurry up and wait” for testing to see how extensive the damage is, and put a stick in the sand (actually this one will be in cement as it won’t move) so in a couple of years time we can see if there is any improvement. It is not likely, but we also don’t want there to be any deterioration.

You think if you are told you have brain damage you would worry, but this puts all the pieces of the puzzle together so well. All the little groups are now sitting nicely in line with the brain, and the puzzle is starting to look like the whole picture.

It was with great relief that, as we walked out of the Dr’s office, we both laughed and said “You failed, Yay”.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

And the mystery continues.

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

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

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

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

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

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

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

We are none the wiser.

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

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

Adrenal medulla

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

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

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

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

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

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

A Catastrophic Event – Part 3

We pick up the story on Friday.  Things were going down-hill ………………………….

The only reason I know what was happening was because I was told by some wonderful people on a Ladies Prostate Cancer forum, that I should record everything that happened with his treatment, and after care, for future reference.  I didn’t realise then, how important it would be in saving his life.

 Friday 5th October
D
erek had an appointment at Urology at the Hospital.
His catheter was removed.  He had had diarrhea for 24 hours.

We asked the Surgeon about the rash on his back. He told rash he didn’t know about rashes and just left it at that.  We then mentioned the bad gastric wind, pain, being unable to do anything.  He said that recovery from surgery was sometimes very slow with 2 steps forward, 1 step back.  We couldn’t make him understand that there were no forward steps, each day was a step backwards.
He told Derek to stop all meds.

 Saturday, 6th

Derek woke to a lot stronger pain in abdomen. He still had very loose bowels.
I gave him Panadine twice during the day for the pain. His stomach was becoming very tight and swollen.

 Sunday 7th:

I forced Derek to walk to end of street hoping that his wind would ease.  Each time he got up to walk around he would burp.  It was relentless.  But it didn’t ease the pain.  The ball of wind was under his ribs. He felt weak and dizzy when he stood up, he would have to sit down again.  He couldn’t stand for more than a minute or two.

The pain and swelling was so intense that I phoned a medical help line to see what I should do.  They told me to take him to an out of hours medical clinic for assessment.

His stomach was distended and painful to the touch on the right side.

He was given Omeprazol 20 mg 1 bd to reduce gas and told to go on a liquid diet for 2 days.  By this time he wasn’t eating, and was starting to lose weight.  He was also told to increase fluids and take panadole.  He also had indications of a throat infection (red and swollen, similar to the rash he had on his back.

 Monday 8

Derek would only get up to try and reduce the wind.  The omeprazole would work for a short time, but not completely.  The pain was worse.  He could not stand apart from getting himself to the toilet. He was now in bed most of the time.  He wasn’t even able to sit/lay in his chair in the Lounge.

 Tuesday and Wednesday were pretty much just a worsening of previous days.

 Thursday 11 Oct

Derek started complaining of Groin pain (described as “Like a stick being shoved between his scrotem and anus.”   It was bad enough to get him taking a lot more Panadine.  He could not get comfortable, he still had a temperature.

He had now developed a strange rash around his neck.  It was very red, raised and blotchy.  It stayed for several days.

He had an INR in the morning which came in at 6.5.  He was ordered to stop Warfarin.

 Friday 12 Oct

I took Derek to the Dr again.

We were sent for blood tests.   His temperature at the Dr’s was  37.8.  We kept giving Panadol, but it would not come down.  It rose to 37.9 an hour after taking Panadol.

His INR was down to 4.5

 Saturday 14 Oct

Derek went for another INR.  We left the lab and took the lift to the ground floor, walked outside to the car, which I parked right outside the doors.  Derek stopped before he got in the car.  He noticed a strange sensation.  He lifted his sleeve to see blood streaming down his arm.  This had never happened before.  Normally when he had an INR test, he would have a couple of drops on his arm and that was it.

Derek’s temperature continued to go up and down all weekend.

His Stomach was very tight, swollen, and painful.  The pain kept increasing.  He was completely bed ridden.

 Monday 15 Oct

The pain was at 8/10.  He Vomited at 3.30pm.   He hadn’t had any sleep Sunday night due to pain, high temp etc.

 Tuesday 16 Oct

He vomited at 3.30am.  Later in the morning he vomited again twice.

He described the pain now as 9/10.  I phoned the Dr for an urgent appointment.

I managed to get him to the car but it was not a good trip to the Dr’s surgery.  Every corner I went round caused major pain.  He could barely stay conscious.  He could not hold his head up.

The Dr examined him and said it was time he went to Hospital (finally).  I told him there was no way I was driving him there.  It had been bad enough just driving him to the Surgery.

The Dr agreed and phoned for an ambulance.

He was given Morphine and fluids during ambulance journey to Hospital.  By the time he arrived at the Emergency Department he described the pain as down to 5-6/10 (on morphine).

His stomach was very swollen by now, he had dry cough, and was barely conscious.  His blood pressure was very low, but his pulse rate was up.

They took a chest xray to see if he was bleeding into the abdomen and admitted him to the Urology ward thinking that it was all to do with the surgery.  He had a partially collapsed lung and possibly pneumonia.

I left late that night as I didn’t know what was wrong, and I was very concerned.  Derek looked very unwell.  He was in a lot of pain, he wasn’t drinking.  They put him on a drip for fluids, a drip for antibiotics and were trying to settle him in for the night.

 Wednesday 17 Oct

I got to the hospital in the morning and Derek looked worse.  His blood pressure was way down, his heart rate was up, he was now on oxygen and there were people coming and going constantly.

They decided to do a CT scan.  They couldn’t find any fluid in abdominal cavity.

What they did find was a Small amount of fluid on the lungs (plural effusion) and something in the lungs, which they thought was probably from the fluids being given.

Urology couldn’t find anything wrong on that side of things so they decided to call in some other people.

The case was being referred to General Medical for consultation.

I walked past the Nurses station at one point to see a group of Dr’s talking.  Apparently they were all there discussing Derek.  The list of Dr’s were:  Urology, Heamotology, General Medical, Infectious Diseases and Cardiology.

I heard a comment from someone who asked “Where was he diagnosed with Antiphospholipid Syndrome”.  I knew immediately that this was a problem, because it meant they didn’t know anything about his blood counts for IgG or Lupus Anticoagulant.

A female Dr came into the room and I immediately recognized her voice.  I asked her if she was the one querying his diagnosis and she said yes.  I told her exactly when and where he was diagnosed.  She admitted there was nothing on his notes (which there should have been because of his surgery), and she went off to check on the diagnosis.  We never saw her again.

 Thursday 18 Oct

Derek was deteriorating.  A General Surgeon Registrar came around at 11.30 Wednesday evening.  Derek doesn’t remember much of the visit.  All he remembers is something about a Heart Murmor and being rushed off to have something done.  He couldn’t remember what.  He is now to be seen by a Cardiologists as he didn’t have a heart murmer when he was admitted on Tuesday.  His Treponin T ‘s were elevated.  He had an Echo for Heart Issues.

He was moved to CCU due to concerns with his heart and his increasing TT’s.  They removed fluid from his plural sack for testing.  They were also considering pancreatitis as a possibility.  He now had Kidney issues as well.  They were failing.

They had noticed in one of his CT’s that his adrenal glands weren’t quite right, but they dismissed them as not crucial because he had:

  • Heart Failure
  • Partial collapse of one Lung
  • Plural Effusion
  • Worsening Kidney Failure
  • High readings of his Liver enzimes
  • Splinter hemorrhages in his fingertips,

All Fluids were stopped as he was not processing them properly and he was begining to swell.

They changed his Antibiotic to vancomycin IV.  He was was taken for another scan of his heart, and an internal probe to see what they could find.

By this time he was barely conscious, not able to wake up.  If he paused to think about an answer to a question, he would lose consciousness and not know what he was trying to say when he came too again.

I was offered a chair to sleep in beside his bed in CCU.

 It was at this point that our GP phoned me to make sure I realized that he was probably not going to survive and I should be prepared for the worst.  Nobody else was willing to say it, but I knew the truth.

 Friday 19 Oct

Friday was a further few steps downhill.  He didn’t know me all the time.  He was taken for a Transesophageal echocardiogram (TOE) to see if there was an infection in the heart valves.  This came back clear so they couldn’t explain the heart failure.

When he came back from the TOE he didn’t know where he was. Disoriented. He tried to remove his IV lines as he thought he was home.  He thought he was in England, then at University.  When asked how he was feeling by one of his Drs his only answer was “I don’t have enough data for that” and he lost consciousness again.

GENERAL MEDICAL had taken over the case, but he remained on CCU.  He was also give a PARS nurse (Patient’s at Risk).  They are the ones that make the call to take people to ICU.  CCU wanted to keep him there because of his heart so the PARS nurse came in every two hours to monitor him.

They did a blood test and found that his Cortisol levels were dangerously low.  He was given IV hydrocortisone.

He showed some improvement within a couple of hours.  I was happy going home for the first time all week.

 Saturday 20 Oct

I arrived in the morning to find him slumped over in bed.  He looked terrible.  His temp hit 40 during the night.  His BP at 7.00 am was BP 84/56.

By 9.30 am it was 91/59.

He was very confused, could not sit up, going down-hill again.  He was given more HC.

 Sunday 21 Oct

I arrived to see a slight improvement. He was able to retain information

He continued to improve over the week, and was still receiving Hydrocortisone.

He was taken off the heart monitor, oxygen etc and moved from CCU to a general Cardiothoracic ward on Tuesday.

 Thursday 25 Oct

Derek was given a ACTH Stress Test on the Thursday morning.  This involved stopping the HC the night before.  They took a blood test at 8 am, then gave him some ACTH.  He started to go down-hill again.  40 minutes later they took more blood, then gave him some Hydrocortisone IV.  He improved almost immediately.

The results came back later that day confirmed Diagnosis of adrenal insufficiency.

We were also told that it looked like he had had a Catastrophic Antiphospholipid event.  He was visited by a Rhumatologist who ran some tests to see if he had Lupus.  She confirmed that he didn’t, and was referring him to another Rhumatologist at our Local Hospital.

 Friday 26 Oct

We were finally told that Derek could go home.  He would be on Hydrocortisone for the rest of his life, he had to be very careful of his APS, and he was to be moved to another ward for the weekend.  It was planned that he would have one last CT of his Adrenal Glands on the Monday morning, then discharged.  2 weeks after being admitted.

He was even allowed to go home for a couple of hours on the Sunday.

He still wasn’t well, his heart rate would not come down under 100 but they were sure it was just the damage from the CAPS event, and it would improve.

We waited for Monday’s CT……………………………………………………….