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Adrenal crises in the oncology patient

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If an adrenal crisis is suspected, please discuss urgently with the paediatric oncologist on call. It is necessary to define if this is primary adrenal insufficiency (rare) or secondary adrenal insufficiency (relatively common). Discussion with the paediatric endocrinologist may also be needed. Emergency management of adrenal crises in paediatric oncology patients is similar to that of other paediatric patients however the combination of neutropenia and immunosuppression with a sepsis induced adrenal crisis can be rapidly fatal. If treatment is recommended, the treatment schedule is outlined below.

Management of Steroid Deficient (Adrenal) Crises

Oncology patients who have recently received steroids and become septic, who undergo surgery or other stressful events are at particular risk of adrenal crisis. It can also occur in patients with brain tumours or those who undergo cranial surgery or radiotherapy due to direct effects on the pituitary.

Relative steroid potency 
Hydrocortisone  1
Prednisone  4 - 6
Methylprednisolone  6 - 8
Dexamethasone  80 - 120 

Recognising the situation

The first thing to ascertain is whether there has been prolonged or high dose steroid use recently. A high index of suspicion is required as the symptoms are generally non-specific. All children who receive regular steroids are at risk, including those who receive steroids for platelet transfusions if they require them frequently, for example, post transplant.

Non-specific symptoms
malaise 
nausea and vomiting, abdominal pain, weight loss 
fever or hypothermia 
headache 
hypotension/poor perfusion (which is poorly responsive to pressor agents). This is normally postural in the first instance. 
hypoglycaemia, hyponatraemia, hyperkalaemia is the classic triad
Crisis
acute onset of shock, metabolic acidosis, severe electrolyte disturbances. 
Symptoms seen in adrenal crisis
dehydration, shock 
GI symptoms (vomiting, diarrhoea, abdominal pain, constipation) 
syncope 
fever 
tachycardia 
hypoglycaemia - may cause seizures 
acidosis 
lethargy, weakness 

Key risks are sepsis, diarrhoea and vomiting, and non-compliance with steroids.

Children who have recently stopped steroids who present with malaise, perhaps a low temperature and hyponatraemia should be admitted.

Diagnosis

  • Biochemistry including Na, K, Cr and glucose
  • FBC
  • Cortisol and ACTH level (preferably pre 8am but if treatment is needed urgently then take level and initiate therapy without delay). While random levels are difficult to interpret, a level of <100nmol/L strongly suggests insufficiency while one >600nmol/L suggests normal function.

It is important to treat these crises early to avoid severe morbidity and mortality. Boluses of Hydrocortisone are insufficient treatment for severe adrenal crisis and allow potentially dangerous trough levels in children with severe illness. Deaths have also occurred with bolus iv hydrocortisone as the trough levels can be dangerously low, even when given 4 hourly.

  1. Admit to hospital.
  2. IV Hydrocortisone treatment, 100 mg/m2 IV infusion of 2 mg/m2/hour (50mg/m2/day). Note: This calculation is not appropriate for children weighing less than 10 kg.
  3. Treat hypotension with IV fluids boluses 0.9% NaCl as required and hypoglycaemia with IV glucose.
  4. Chart on hydrocortisone infusion chart.

Perioperative management

Children who have been on regular steroids who are undergoing surgery requiring a general anaesthetic should receive IV hydrocortisone infusion as per the moderate to severe illness guideline (30 mg/m2/day in three divided doses respectively). Normal steroid usage can resume 24-48 hours later depending on recovery.

Principles of management

  • Cortisol secretion, and thus requirement, in normal subjects increases approximately 5 to 10 fold during any physical stress such as illness, anaesthesia and all but minor surgery.
  • The treatment goal is to mimic the normal response by providing doses approximating 50-100 mg/m2/day of hydrocortisone (HC) equivalent.
  • Providing adequate glucocorticoid treatment in patients who are not able to can prevent a life threatening adrenal crisis. If in doubt, it is always safer to provide 100 mg/m2 HC to sick patients. Overtreating in a critical situation (adrenal crisis) may be life saving, whereas not treating, delaying treatment, or under-dosing can be life-threatening. Furthermore, once overt hypotension has developed, it can take a long time to restore normal cardiac function (days not hours).
  • The preferred route of administration for any child who is vomiting or severely stressed is intravenous. A continuous IV infusion is ideal, as it mimics normal secretion during severe stress, but intermittent boluses 6 x per day (4 hourly) are acceptable because of the prolonged biological effects of all the glucocorticoids.
  • Stress doses of HC (but not dexamethasone) provide full mineralocorticoid effect.
  • Stress doses of hydrocortisone can be returned very rapidly back to simple replacement treatment, as the patient recovers. "Tapering" the dose is not usually needed.
  • Don't forget to treat the underlying cause of the stress, such as infection!
  • Other steroids have no advantage over HC, but can be used for particular children. Their potency is probably more than described in textbooks.

See the Hydrocortisone Infusion Chart if >10 kg.

Management details for adrenal insufficiency

Severely unwell - unstable / crisis or unwell (vomiting, diarrhoea, drowsy), unable to tolerate oral HC

  • Start IV fluids: 10-30 ml/kg 0.9% Saline, then maintenance 0.9% Saline + 5% glucose
  • Hydrocortisone: 50 to 100 mg/m2 IV or IM bolus followed by 100 mg/m2/day until stable.
Age Dose of hydrocortisone succinate (mg)
{IM or IV}: Initial Bolus 
Hydrocortisone Infusion (50 mg HC
in 50 ml 0.9 saline) 
0 - 6 months  12.5  1 ml/h:infants 
6 mths - 5 years   25  2 ml/h: pre-schoolers 
5 - 10 years  50  3 ml/h: older children 
> 10 years  100   -

Presumed adrenal insufficient patient undergoing CNS tumour resection: Neurosurgical dose of Dexamethasome during surgery is sufficient.

Moderate illness

  • stable with fever, reduced activity. Able to tolerate oral hydrocortisone: HC 50 mg/m2/day.
    Give five times the total daily dose hydrocortisone, as 5 divided doses (breakfast, lunch, afternoon tea, dinner, bedtime).
  • Gastroenteritis: Stable with mild diarrhoea: Able to tolerate PO fluids: start oral HC 50 mg/m2/day as above or give IV/IM).
  • If already on a phase of treatment involving prednisone or dexamethasone and tolerating oral intake, continue with this form of steroid.

Mild illness (respiratory, ear infection no fever, vomiting or diarrhoea, and looks well)

  • Continue normal maintenance doses.

Elective surgical procedures

Weight (kg) Single dose HC PRE-operatively (mg) PO prior to being NPO Rate of infusion INTRA and POST operatively (ml/h = mg/h) 
(50 mg HC in 50 ml 0.9% saline) 
3 - 10  25 mg  
10 - 20  50 mg 
> 20  100mg 
  • Maintenance IVF with 0.9% saline containing 5% glucose during surgery
  • or dosing can be based on age: En route to surgery, prior to anaesthesia:
    0 - 3 yrs : HC 25 mg IV bolus, then 50 mg/m2 (~25 mg) infusion for remaining 24 hrs
    3 - 12 yrs : HC 50 mg IV bolus, then 50 mg/m2 (~50-60 mg) infusion for remaining 24 hrs
    12 yrs : HC 100 mg IV bolus, then 50 mg/m2 (~100 mg) infusion for remaining 24 hrs.

Once stable: switch to maintenance oral hydrocortisone (~10 mg/m2/day).

Hydrocortisone infusion chart

See link for pdf version of chart

References

Hahner S, Allolio B, Therapeutic management of adrenal insufficiency. Best practice and research clinical endocrinology and metabolism. 2009 23(2):167-179

Howard SC, Pui C_H. Endocrine complications in paediatric patients with acute lymphoblastic leukaemia. Blood Reviews;2002;16:225-243

Starship Clinical Guideline (Stress Steroid Management 2013)

Personal communication - Dr Craig Jefferies, 2016

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Document Control

  • Date last published: 24 April 2017
  • Document type: Clinical Guideline
  • Services responsible: National Child Cancer Network
  • Author(s): Scott Macfarlane, Craig Jefferies
  • Owner: Scott Macfarlane
  • Review frequency: 2 years