Adrenal insufficiency in children, emergency and acute management guidance, paediatrics (239)

Need for IM or IV hydrocortisone.

DO NOT DELAY ADMINISTRATION OF IM or IV HYDROCORTISONE WITH SUSPECTED ADRENAL CRISIS.  PATIENTS WITH SUSPECTED ADRENAL CRISIS COULD DETERIORATE RAPIDLY EVEN IF APPEAR WELL.

INITIAL MANAGEMENT OF SUSPECTED ADRENAL CRISIS

• Administer IV bolus or IM hydrocortisone
• Manage acute presentation as appropriate (treat the underlying cause)

IM or initial IV bolus hydrocortisone doses for suspected adrenal crisis

Indications:

• Acutely unwell with diarrhoea and vomiting
• Reduced responsiveness or loss of consciousness
• Hypoglycaemia or new onset seizure in known or suspected adrenal insufficiency
• Painful fracture or fractures with significant deformity
• Significant burn
• Major trauma or severe shock (eg road traffic accident, head injury with loss of consciousness

On arrival to hospital, check blood glucose as soon as possible.
If blood glucose < 3 mmol/L, give 3 ml/kg of 10% dextrose as bolus.
Recheck blood glucose in 15 min and repeat bolus if necessary.

If shock or moderate to severe dehydration, give 10 ml/kg of 0.9% sodium chloride and repeat if necessary.
Check electrolytes at presentation to inform fluid usage.

SUBSEQUENT MANAGEMENT OF SUSPECTED ADRENAL CRISIS

Following bolus of IV/IM hydrocortisone the child should be started on a hydrocortisone infusion

Hydrocortisone infusion for acute illness

To make up hydrocortisone infusion

• Add 50 mg hydrocortisone in 50 ml 0.9% sodium chloride
• Hydrocortisone infusion can run alongside 0.9% sodium chloride or 5% glucose or PlasmaLyte solutions
• Consider more concentrated infusions in those needing fluid restriction (eg 100 mg hydrocortisone in 50 ml 0.9% sodium chloride)

Maintenance fluid type

0.9% sodium chloride / 5 % glucose is usually an appropriate starting point.

Hyperkalaemia: Children with primary adrenal insufficiency can be hyperkalaemic at presentation or in an adrenal crisis because of mineralocorticoid deficiency. Emergency management of adrenal crisis with IV glucocorticoids and IV fluids (0.9% sodium chloride) will reduce potassium levels. Hyperkalaemia is potentially life-threatening and can lead to cardiac arrhythmias. Additional measures such as the use of IV calcium gluconate, nebulised salbutamol, IV insulin and glucose or IV bicarbonate and cation exchange resins should also be considered. ²³

Hyponatraemia and fluids: Sodium chloride 0.9%/5% glucose is usually a good starting point for initial fluid management if the clinical and biochemical picture suggest that the low sodium has arisen primarily because of salt wasting.

• In primary adrenal insufficiency (eg Addison’s disease, Congenital adrenal hyperplasia), mineralocorticoid deficiency will cause hyponatraemia due to renal losses.
• In secondary adrenal insufficiency (eg hypopituitarism, patients with brain tumours or post-radiotherapy, patients on long term treatment dose of glucocorticoid eg Prednisolone, Deflazacort, Dexamethasone), cortisol deficiency can lead to a lack of free water clearance which can contribute to hyponatraemia. In this latter scenario (those with secondary adrenal insufficiency), a degree of fluid restriction may be more appropriate.

Fludrocortisone is an oral mineralocorticoid used in primary adrenal insufficiency. The dose does not need adjustment in the event of a sick day episode or an adrenal crisis. If the child is unable to take oral medication then IV fluids may be required to maintain the salt and water balance depending on the clinical situation. However, the mineralocorticoid effect of hydrocortisone at stress doses is often sufficient to cover the mineralocorticoid requirement. Oral fludrocortisone should be re-commenced when able to tolerate oral medication.

Need for oral sick day dosing

Indications:

• Acute infections/childhood illnesses with fever (usually not well enough to go to school)
• Vomiting or diarrhoea (only if tolerating oral medication and fluid but low threshold for consideration for IM or IV therapy)

Oral sick day dosing based on weight.

A guide to sick day dosing that can safely be used in the emergency setting is provided below. However, the actual dose may vary depending on the strength and preparation of the available hydrocortisone medication. Rounding up to the next 0.5 mg dosing is appropriate. Sick day dosing should be given for the duration of the illness. Patients own personalized sick day dosing plans from clinical review within the last 6 months (if available) can also be used.

Patients should should be advised to ring primary medical team if no improvement after 48 hours or if further deterioration on oral sick day dosing following discharge from the hospital/A&E.

For patients on treatment dose of glucocorticoid (eg Prednisolone, Deflazacort), a simple and safe approach is for additional sick day hydrocortisone to ensure adequate plasma cortisol levels throughout the day and night, on top of usual treatment dose of glucocorticoid (eg Prednisolone, Deflazacort). This is relevant as the half-life of Deflazacort is 1.5 to 1.9 hours; and the half-life of Prednisolone is 2.0 to 4.0 hours. The treatment dose of glucocorticoid (eg Prednisolone, Deflazacort) does not need to be altered during sick day episodes.

No need for sick day dosing (ie no change to usual treatment)

Indications:

• Mild cold or runny nose with no fever.
• Minor playground bumps and bruises