Rant: Let's start a revolution. Don't allow cortisol and ACTH testing to determine your hydrocortisone dosing.

"Treatment surveillance of chronic glucocorticoid

replacement is mainly based on clinical grounds because

no objective assessment has proven to be reliable for

monitoring replacement quality."

"Thus, in the absence of objective variables to measure

replacement quality, the doctor has to rely primarily on

clinical judgment, taking into account signs and

symptoms potentially suggestive of glucocorticoid overreplacement

or under-replacement (table). Underreplacement

bears the risk of incipient crisis and severe impairment of wellbeing."

Adrenal Insufficiency Arlt and Allilio

"Individual dose adaptation and monitoring of glucocorticoid replacement remains challenging as cortisol production is highly variable during the day and further influenced by many factors that activate stress responses like physical activity, pain, infections, psychological stress, low blood glucose, etc. Recommended daily hydrocortisone doses in primary adrenal insufficiency (PAI) are lower than estimated before ranging between 10 and 20 mg.  However, this reflects a mean need during the day and may not cover the need induced by additional stressors. Therefore, patients need to learn how to adapt their dose according to daily needs in a more flexible manner. Furthermore, comedication has to be taken into account...Some authors recommend weight-adjusted hydrocortisone dosing, thrice daily before food, leading to a reduction of intervals with excess cortisol exposure during the day and to reduced interindividual variability of cortisol profiles. This might be helpful when newly starting hydrocortisone replacement. However, other authors showed that there was no correlation of a clinical score assessing quality of replacement therapy with total or body weight-adjusted glucocorticoid dose. This demonstrates that dose finding has to be individually adapted and also requires patient education enabling the patient to correctly and autonomously adapt the hydrocortisone dose. Because of the nonphysiological cortisol profiles achieved by current replacement regimes, to date no reliable laboratory parameter exists for correct assessment of replacement quality. Even the serum cortisol day curves suggested by some authors only give a rough estimate and help to identify largely over- or underreplaced patients but are of limited value in the standard monitoring of glucocorticoid replacement. ^[4] Treatment surveillance is mainly guided by clinical judgment assessing daily performance, subjective health status and signs and symptoms of glucocorticoid over-replacement (weight gain, skin alterations) or under-replacement (fatigue, nausea, myalgia and joint stiffness). Fatigue is, however, a common complaint also under apparently optimized standard replacement conditions. Therefore, an increase in hydrocortisone should timely be reevaluated to avoid overdosing."

What is the Best Long-Term Management Strategy f0r Patients With Primary Adrenal Insufficiency? 

Quinkler and Hahner

More references for your reading pleasure:

Debono, M., Ross, R.J. & Newell-Price, J. (2009) Inadequacies of glucocorticoid replacement and improvements by physiological circadian therapy. European Journal of Endocrinology, 160, 719– 729.

Mah, P.M., Jenkins, R.C., Rostami-Hodjegan, A. et al. (2004) Weight-related dosing, timing and monitoring hydrocortisone replacement therapy in patients with adrenal insufficiency. ClinicalEndocrinology (Oxford), 61, 367–375

Arlt, W., Rosenthal, C., Hahner, S. et al. (2006) Quality of glucocorticoid replacement in adrenal insufficiency: clinical assessment vs. timed serum cortisol measurements. Clinical Endocrinology(Oxford), 64, 384–389.

So your question is, why does my doctor test my ACTH and cortisol?  The answer is simple.  He does no research and has even less comprehension of adrenal insufficiency than you know.

Testing ACTH is expensive and a waste of your blood and time once you've been diagnosed*.  Study after study concludes, "

*There are exceptions to this statement.  If you are secondary, ACTH may need to be tested.  If you are Cushing's and have had a BLA.  If you are primary and hyperpigmented, you may need your ACTH tested.  More than likely, if you're hyperpigmented and primary, you need more HC.

"ACTH cannot be used as

a criterion for glucocorticoid dose adjustment, since in

primary adrenal insufficiency it is invariably high before

the morning dose and rapidly declines with increasing

cortisol concentrations after glucocorticoid ingestion.122,124

Aiming at morning ACTH values continuously within the

normal range would, therefore, lead to chronic overreplacement.

However, in case of reappearance of skin

hyperpigmentation in primary adrenal insufficiency,

concentrations of plasma ACTH should be measured."

 Adrenal Insufficiency Arlt and Allilio

I really have no clue what you can do if your doctor recommends testing cortisol and ACTH except a) refuse b) just don't get the draw c) ask what he's basing his potential conclusions on (yes, easier said than done).  

The most important thing you can do is respect yourself.  Are you still suffering from symptoms of untreated adrenal insufficiency such as nausea, vomiting, diarrhea, fatigue, hyperpigmentation, low blood pressure?  Do you suffer from symptoms of Cushing's like weight gain, insomnia, easy to anger, purple striae, easy bruising and thin skin?  Let your symptoms guide you and your physician.  

Feel like crap and think you're on the "right" dose of HC?

1. Are you dosing your HC physiologically?
2. Does the amount of florinef you take keep your sodium consistently at 140 at 8 am, fasting after 24 hours on a normal sodium diet (2,300 mg)?
3. Is your free T4 1.3 if you're female and 1.4 if you're male?  GET YOUR RESULTS.  NORMAL is not an acceptable answer here.
4. Are you allowed to replace the hormones in which you're deficient like DHEA-S, testosterone, progesterone?

If you feel like crap and the answers to each and every question above is "yes", you need further testing and/or dosing assistance.  Get help.  If the answer is "no" to even one of these questions, work on each one in order with your physician.

In summary, doctors are wasting our blood, our money and our lives by testing cortisol and HC to determine the proper dosing since there are NO standards upon which to base their opinions EXCEPT clinical grounds!  

Let's start a revolution.  

Require your doctor to provide you with proof of why he wants to test your cortisol and/or ACTH to determine your hydrocortisone dosing.  He might fire you but it might be worth it.  You'll find someone who thinks you and your judgement is valuable.  Who knows?  You might feel better if you were allowed to trust yourself and your symptoms that YOU live with 24 hours a day, 7 days a week.

Rant: Many doctors do not know the first thing about adrenal insufficiency nor hypothyroidism. Caveat Emptor.

At the request of my new DO, I went to see an endocrinologist.  Because I want to keep this DO, he's highly recommended, I went against my instincts and saw the endo that he said he would hypothetically have his wife or daughter see.

No surprise, I walked into the waiting room to be greeted by a multitude of Type II diabetics.  Type II diabetics are the bread and butter of most endocrinologists.  For this reason, the endocrinologists know the most about...you guessed it...diabetes.

The doctor promptly met in me at the appointed time.  I was encouraged by this.  We talked for a minute and he asked to see my test results from my diagnosis.  mistake on my part.  My diagnosis was not text book depending upon what information was used to determine this.  At 30 minutes, I stimmed to 16.  That's a failure of the ACTH stim test if you use the 30 minute reading.  I stimmed higher than 18 for the 60 minute reading which this doctor declared a "pass".  I asked why a 90% failure of the adrenals was used to determine the need for replacement steroids and what would have happened to me if I had been in an accident at that time in my life where I had an obvious 80+% failure of my adrenals.  His reply, "That's just the criteria that was established a very long time ago and that's what I use!  You didn't have adrenal insufficiency! Now you have iatrogenic (that's doctor induced (yes, no shit Sherlock)) adrenal insufficiency."

I showed him my insulin tolerance test.  The baseline was high and he declared that because of that number alone, the test didn't matter.  He could not explain to me why the cortisol went down when my body was stressed with insulin nor could he explain why I went unconscious from low blood sugar.  I can tell you why and I don't have a medical degree. Cortisol is used to synthesize glycogen.  Glycogen is released when the body is stressed as in the case of an insulin tolerance test.  When the body has no glycogen stores due to long term, untreated adrenal insufficiency, it will do what it can to keep the blood sugar up, like, duh, use the cortisol to synthesize glycogen.  If there's no glycogen, the person will a) have lower and lower cortisol readings as time progresses b) go hypoglycemic.  BINGO.  I did both.  A diabetes doctor didn't understand the insulin tolerance test, sigh.

He saw my thyroid results where my TSH was 3.6, within the lab range but high if you consider 85% of the US population has a TSH of 1.5 or so.  I didn't have hypothyroidism either according to him!  I asked why the American Association of Clinical Endocrinologists recommend keeping the TSH at 1-2.  He said that I would have to use the same lab as the AACE to find a TSH of 3.6.  At this point, I collected everything I had and got up to leave.

I am healthy.  I am very healthy.  I have AI and hypothyroidism yet run ultramarathons.  I blew out my shoulder and had reconstructive surgery and can still to pull ups. I have streaked walking for five years and running for three.  I teach spin classes.  I rarely get colds.  Since I've quit drinking, nausea occurs infrequently.  He claimed he could not take me on a a patient since he did not agree with my 15 year old diagnosis.  From a visual perspective, it's quite obvious that I'm not over replaced on steroids nor on thyroid medication.  My life went from illness and struggle to good health yet this man thought my diagnoses were wrong.  On a positive note, he said he would recommend to my DO to continue to prescribe my medications to me.  Except testosterone.  I would need an OB/GYN for that.  WTF?! An endocrinologist who has no understanding of the adrenals.  60% of a woman's testosterone is produced in the adrenals.  My adrenals don't work.  Testosterone is not an OB/GYN issue in my case.  Complete and utter lack of understanding of the adrenal glands by a medical professional.

Here is what I learned from seeing an endocrinologist:

• Never give a doctor information that won't help with your current situation unless they are absolutely positively textbook failures.  For example, I should not have brought ANY medical records that were more than a year old.
• When a doctor shows his absolute, positive lack of interest in clinical symptoms paired with lack of understanding of basic endocrinology (IE not knowing the AACE guideline and, worse yet, not understanding them in the slightest), RUN.  
• When a doctor only uses only one lab range (incorrectly, I might add) to determine a diagnosis, he's neither intuitive nor caring.  He doesn't care about alleviating symptoms nor helping you feel better.  He wants to be right.
• I will try to never see an endocrinologist again unless he or she has a proven track record with AI.  I'm sure I can see an anti-aging doctor who would be tickled pink to have a healthy middle aged woman as a client.
• I came out of the office, mad and hurt.  I doubted myself and my diagnosis as a result of the things that were said to me and the misinformation that he tried to spread.  I felt horrible for those who have not studied adrenal insufficiency and hypothyroidism the way that I have.  The doubts they might have would be overwhelming.
• MOST IMPORTANTLY, I NEED TO MAKE A DIFFERENCE IN THE MEDICAL COMMUNITY.  I NEED TO FIND A WAY TO EDUCATE THEM SO THAT THEY DON'T KEEP TRYING TO MAKE US FEEL TERRIBLE ABOUT OURSELVES, OUR DIAGNOSES AND THE SYMPTOMS THAT WERE VERY REAL AND DEBILITATING.  THE SYMPTOMS THAT WE HAD AND THE AWFUL LIVES WE USED TO LIVE THAT WERE ALLEVIATED BY PROPER TREATMENT COUNT FOR SOMETHING.  ALLEVIATION OF SYMPTOMS IS AN INDICATION OF PROPER DIAGNOSIS AND MANAGEMENT OF OUR DISEASES!
• More than anything, I would love to see a doctor who listens and will talk to me.  I would like a doctor who can trust his own eyes and my current lab work.  I am healthy.  I want to stay that way.  What's so hard about working with a patient to help him or her achieve her greatest potential?  I suppose it's not cost effective to spend time with a patient, listen and discuss things only to have a healthy patient who will only see you once a year?  It's a much better business model for a doctor to be all knowing and keep your business coming in throughout the year.

Rant: Think your surgeon knows what he's doing with hydrocortisone dosing for surgery, think again

http://www.asaabstracts.com/strands/asaabstracts/abstract.htm;jsessionid=B8A9D5FA13A9A47553678ED64622BC4A?year=2015&index=16&absnum=2911

A1219 October 24, 2015 1:00:00 PM - 3:00:00 PM Room Hall B2-Area C

Survey of Pediatric Anesthesiologists Regarding the Use of Peri-operative High Dose Steroids for Children With Adrenal Insufficiency

Hardave S. Gill, M.D., Kristine Urmson, M.D., FRCPC, Jennifer O'Brien, Not Applicable University of Saskatchewan, Saskatoon, Saskatchewan, Canada

Disclosures: H.S. Gill: None. K. Urmson: None. J. O'Brien: None.

Background: Adrenal insufficiency is a disorder of the adrenal glands where they do not produce enough of certain hormones, mainly cortisol and aldosterone. Management of patients with adrenal insufficiency presenting for surgery in regards to steroid supplementation remains unclear. Congenital adrenal hyperplasia (CAH), one form of adrenal insufficiency, is a disorder involving a deficiency of an enzyme involved in the synthesis of cortisol, aldosterone, or both. Current guidelines are clear that high dose steroids are recommended for children with CAH undergoing anesthesia. High dose steroids have potential risks such as bradycardia, hypotension and asystole, increased risk of infection, blood glucose disorders, liver & gastrointestinal effects, and psychiatric syndromes. Given the risks identified, it is important to examine if current recommendations reflect clinical practice in providing optimal care for patients. Methods: Local research ethics board approval was obtained prior to study commencement. A cross-sectional survey was distributed following pretesting and pilot-testing. Invitation to participate in the survey was distributed via the Canadian Pediatric Anesthesia Society members’ email list. The initial email invitation was followed with two additional invitations to complete the survey. Responses were analyzed using standard tabulations. Results: 55% of respondents would not provide stress-dose steroids for a cystoscopy and 21% would not do so for a laparotomy, despite the Endocrine Society Clinical Guidelines on CAH. See Table 1. Discussion: Our results demonstrate variation in clinical anesthetic practice regarding stress dose steroids in children with CAH undergoing anesthesia. Even when guidelines are provided, many respondents indicated they would not follow them. Our data also highlight that the decision to provide stress dose steroids is related to the proposed procedure. Finally, given the significant variation of practice, a need for future research is identified with an eye to change current practice recommendations.