Addisonian crisis

Warning

Objectives

Guide the safe management of acute adrenal insufficiency ('Addisonian crisis') on operations

Scope

Management of adrenal insufficiency/ Addisonian crisis

Audience

Clinical decision makers at Roles 1 - 3

Initial Assessment & Management

Presentation and history

Adrenal crisis represents acute adrenal insufficiency, typically precipitated by infection, injury, surgery, or cessation of long-term steroid therapy. In Role 1 environments, it is likely to be indistinguishable from septic shock.  Identification in a role 1 environment will be a consequence of known adrenal insufficiency, or a medical history including long term steroid use; Look for Steroid alert card, medical alert bracelet or necklace with the words 'adrenal insufficiency' or similar.  In a patient with such a history, there should be a very high index of suspicion for adrenal insufficiency, and 100mg hydrocortisone should be administered alongside initial resuscitation. 

Features of a crisis:

The patient will present with hypotension or shock, refractory to typical sepsis resuscitation. The history may include recent steroid withdrawal or missed doses of maintenance hydrocortisone, or malabsorption precipitated by GI illness. Preceding symptoms might include low grade fever, weakness, confusion/delirium, nausea/vomiting, abdominal pain, hypoglycaemia, or unexplained collapse.
Hypotension or hypovolaemic shock, refractory to treatment Headache
Delirium Low grade fever
Acute abdominal pain Muscle weakness
Vomiting Hypoglycaemia

IN role 1, treatment will be as for sepsis: 

- Administer high-flow oxygen
- Establish IV/IO access
- Begin fluid resuscitation with initial resuscitation of up to 30mL/kg 0.9% sodium chloride, reassess haemodynamics and continue clinically guided resuscitation. 
- Reassess haemodynamics after each litre; continue further boluses guided by perfusion response and evacuation time. Patients suffering with mineralocorticoid deficiency will be profoundly volume deplete due to salt wasting. 
- Sepsis cannot be excluded at this stage in the patient care pathway, so its reasonable to commence broad-spectrum antibiotics as per microbiology guidance. 
Empirical steroid replacement if known or suspected adrenal insufficiency, or if history makes the diagnosis highly likely:
- Hydrocortisone 100 mg IV or IM immediately 
- Then Hydrocortisone 50–100 mg IV or IM every 6 hours until able to take orally
- Once enteral route is tolerated: Hydrocortisone 50 mg PO every 6 hours, with tapering to maintenance dose at higher level of care
All patients should be evacuated for further stabilisation and diagnosis. 
Continue IV fluids and hydrocortisone during transfer
IF prolonged stay at role 1 and oral agents are available (not in 370/501), hydrocortisone is really the steroid of choice.  If prednisolone or dexamethasone were available, there is minimal mineralocorticoid activity, so a switch to these might precipitate further salt wasting and hypotension. 

Advanced Assessment & Management

Features of chronic adrenal insufficiency (Addison’s):

  • Fatigue
  • Hyperpigmentation of gums, scars, hands
  • Gastrointestinal symptoms: N+V, weight loss
  • Muscular weakness and cramps
  • Postural hypotension
  • Headache
  • Loss of axillary or pubic hair in women
  • Anxiety or depression

Investigation:

  • Low Sodium in 75% of patients
  • High potassium in 35% of patients
  • Hypoglycaemia
  • Metabolic acidosis

Management

Immediate fluid replacement with IV 0/9% saline

Immediate IV Hydrocortisone: 100mg 'stat'. 50 - 100 mg 6 hourly until eating and drinking.

At R2/R3 Check initial morning cortisol. Cut-offs vary depending on assay.

  • Over 300 nmol/l normal
  • <100 nmol/l likely adrenal insufficiency
  • 100-300 nmol/l Arrange ACTH stimulation test (SynACTHen 250mcg IM, measure cortisol after 30min)

Prolonged Casualty Care

Maintain adequate hydration

IV or IM hydrocortisone dosing can be continued until oral hydrocortisone becomes available; tablets are not available at the time of writing in the 300, 500, or 370 modules.  If locally available, the patient can be converted to oral hydrocortisone as soon as eating and drinking: 'doubled dose' normally 20mg on waking, 10mg at 2pm. Adjust depending on clinical picture. Maintenance dose normally 10mg on waking and 5mg at 2 pm.

 

There is scant evidence for continuing IV/IM hydrocortisone beyond 5 days rather than switching to oral tablets.  If situation mandates ongoing intermittent parenteral hydrocortisone dosing, then when stable, eating and drinking, the dose can be reduced to 25mg QDS IM/IV, with ongoing liaison with the physician cadre via Pando and expedited evacuation. 

Paediatric Considerations

Up to date guidance for adrenal insufficiency in children can be found at the following link: https://www.bsped.org.uk/clinical-resources/bsped-adrenal-insufficiency-consensus-guidelines/.

Adrenal crisis in children may present with an acutely unwell child with tachycardia, hypotension, hypoglycaemia, hyponatraemia, hyperkalaemia, or altered consciousness not attributable to another illness.  They should be treated immediately with glucocorticoids and IV fluids as follows:

Emergency Management of Paediatric Adrenal Crisis In Role 1
Intramuscular (IM) hydrocortisone doses or initial IV dose
Age IM hydrocortisone Dose Indications
Less than 1 year 25mg
  • Acutely unwell and unable to get IV access
  • Acutely unwell with diarrhoea and vomiting and unable to tolerate oral treatment
  • Reduced responsiveness or loss of consciousness.
  • Hypoglycaemic or new onset seizure in known or suspected adrenal insufficiency.
  • Fracture / significant burn
1 to 5 years 50mg
6 years and over 100mg

 

Emergency Management of Paediatric Adrenal Crisis in Role 2/3
Children (>28 days)* Hydrocortisone dose and frequency
Severe illness
  1. Age based doses given IM or IV (25mg < 1year, 50mg 1 to 5 years, 100mg for 6 years and over - subsequent doses as in 2 below) or
  2. 2mg/kg (max 100mg) IV bolus initially then bolus dose 6 hourly
Stable and improving 1mg/kg (max 50mg) IV 6 hourly
   
Neonates (<28 days) Hydrocortisone dose and frequency
Severe illness 4mg/kg IV initially 6 hourly
(*can consider giving 4 hourly or as an infusion (see “Major surgery”)
Stable and improving 2mg/kg IV 6 hourly
(can consider giving 4 hourly or as an infusion (see “Major surgery”)
   

 

Fluid type and volume
Blood Glucose < 3mmol/L 2ml/kg of 10% dextrose as IV bolus
Recheck blood glucose after 15 minutes and repeat bolus if necessary.
Shock or moderate to severe dehydration Give 10ml/kg of 0.9% sodium chloride as a bolus and repeat if necessary
Check electrolytes immediately at presentation to inform fluid usage (see "Fluid and electrolyte management")
Maintenance fluids type and amount 0.9% sodium chloride / 5% dextrose is usually an appropriate starting point: 100ml/kg/day for 1st 10kg, 50ml/kg/day for 2nd 10kg, 20mls/kg/day >20kg