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:
[kri´sis] (pl. cri´ses) (L.)
- 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.
- a sudden paroxysmal intensification of symptoms in the course of a disease.
- 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:
- 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
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.
- 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).
- 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
· Dizzy &
· Hot and Sweaty
· raging migraine
· high BP
· So tired just want
|· Extremely fatigued,
· lifeless legs
· pain in abdomen,
· low back and legs.
· mostly high BP,
· then it can plummet
· Belief that rest will
· severe torso
· 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
· Back pain,
· torso spasms.
|· Extreme fatigue
· Abdominal pain
· Speech goes
· High BP
· Light headed
· Wants to sleep
· Inability to speak
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.
|Adrenal crisis Warning signs include:
· severe nausea
· extreme weakness
· chills or fever
|Symptoms of adrenal crisis include
· sudden, severe pain in the lower back, abdomen, or legs
· severe vomiting and diarrhoea
· 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?