What is the emergency treatment protocol for adrenal crisis in a patient with known or unrecognised adrenal insufficiency?

Emergency Treatment Protocol for Adrenal Crisis

Immediately administer hydrocortisone 100 mg IV bolus without delay for diagnostic procedures, followed by aggressive fluid resuscitation with 0.9% saline 1 liter over the first hour. 1, 2, 3

Immediate Emergency Management (First Hour)

Step 1: Recognize and Act Without Delay

• Never postpone treatment to obtain diagnostic confirmation when adrenal crisis is clinically suspected, as mortality increases with delayed intervention 2, 3
• Draw blood for serum cortisol, ACTH, electrolytes (Na, K), creatinine, urea, and glucose before treatment begins, but do not wait for results 1, 2
• Look for hypotension (often <90/60 mmHg), dehydration, nausea/vomiting, abdominal pain, confusion, or altered mental status 1, 4

Step 2: Hydrocortisone Administration

• Give hydrocortisone 100 mg IV bolus immediately - this saturates 11β-hydroxysteroid dehydrogenase type 2 to provide the necessary mineralocorticoid effect 1, 2
• If IV access cannot be rapidly established, use intramuscular route as acceptable backup 2
• This single intervention is the most critical and must not be delayed 4

Step 3: Fluid Resuscitation

• Administer 0.9% isotonic saline 1 liter IV over the first hour - fluid resuscitation must occur simultaneously with hydrocortisone, as both are essential for reversing shock 1, 2, 4
• In pediatric patients, give initial normal saline bolus of 10-20 mL/kg (maximum 1,000 mL) 2

Ongoing Management (First 24-48 Hours)

Continued Glucocorticoid Therapy

• Continue hydrocortisone 200 mg per 24 hours as continuous IV infusion (preferred method based on pharmacokinetic data showing this maintains cortisol in the required range) 2, 5
• Alternative: hydrocortisone 50 mg IV or IM every 6 hours (total 200 mg/day) 1, 2, 3
• The continuous infusion is superior to intermittent boluses for maintaining appropriate cortisol concentrations during major stress 5

Continued Fluid Management

• Maintain slower isotonic saline infusion for 24-48 hours with total of 3-4 liters over this period 1, 2, 3
• Monitor hemodynamics frequently to avoid fluid overload 1, 2
• Check serum sodium, potassium, and glucose every 4-6 hours initially 4

Critical Monitoring Parameters

• Monitor for hyponatremia (present in ~90% of cases), hyperkalemia (present in ~50%), hypoglycemia (especially in children), and increased creatinine 2, 4
• Watch for hypernatremia developing with prolonged high-dose hydrocortisone beyond 48-72 hours - consider switching to methylprednisolone if this occurs 4
• Monitor blood pressure in both supine and standing positions to detect orthostatic changes 2, 4

Additional Supportive Care

• Consider ICU or high-dependency unit admission for severe cases with persistent hypotension 1, 2, 3
• Provide gastric stress ulcer prophylaxis 1, 3
• Consider low-dose heparin prophylaxis 1, 3
• Treat precipitating conditions (infections are most common) with appropriate antimicrobial therapy 1, 6

Critical Pitfalls to Avoid

• Do NOT add separate mineralocorticoid (fludrocortisone) during acute crisis - the high-dose hydrocortisone (≥50 mg/day) provides adequate mineralocorticoid activity 2
• Do NOT use dexamethasone - it lacks mineralocorticoid activity and is inadequate for primary adrenal insufficiency 2
• Do NOT attribute persistent fever solely to infection - it may be due to adrenal insufficiency itself, and steroid supplementation should not be reduced while the patient is febrile 2
• Do NOT abruptly stop high-dose therapy - always taper gradually to prevent rebound crisis 4

Transition to Maintenance Therapy

• Taper parenteral glucocorticoids over 1-3 days to oral therapy once the precipitating illness permits and patient can tolerate oral medications 1, 2, 3
• Restart fludrocortisone only when hydrocortisone dose falls below 50 mg per day, as lower doses no longer provide adequate mineralocorticoid effect 1, 2
• Double the usual oral hydrocortisone dose for 48 hours after resuming oral intake following uncomplicated recovery 2
• Transition to maintenance hydrocortisone 15-25 mg daily divided into 2-3 doses 2

Prevention of Future Crises

• Patient education is paramount but current concepts are not sufficiently effective - documented instances exist where patients are discharged with inadequate education 2, 6
• Teach patients to double or triple oral glucocorticoid doses during minor illness 2, 3
• Provide emergency supplies including injectable hydrocortisone for parenteral self-administration during severe illness or vomiting 2, 3, 6
• Ensure patient has medical alert jewelry and emergency steroid card 3, 4
• Evaluate for chronic under-replacement with fludrocortisone, low salt consumption, poor compliance, and psychiatric disorders in patients with recurrent crises 1, 2

Special Populations

Pediatric Considerations

• More frequent blood glucose monitoring is essential as children are more vulnerable to hypoglycemia 2
• Hydrocortisone dosing should be based on age and body weight 2

Obstetric Patients

• Administer hydrocortisone 100 mg IM at onset of labor 1, 2
• Continue 200 mg per 24 hours IV infusion or 50 mg IM every 6 hours until after delivery 2