What is the recommended weaning schedule for patients on long-term oral hydrocortisone (cortisol replacement therapy)?

Hydrocortisone Weaning Schedule for Long-Term Oral Therapy

For patients on long-term oral hydrocortisone (cortisol replacement therapy), a gradual tapering approach is essential to prevent adrenal crisis, with weaning typically occurring over months rather than days or weeks.

General Principles of Hydrocortisone Weaning

The weaning process for patients on long-term oral hydrocortisone must be carefully managed to avoid adrenal insufficiency. According to guidelines, the following monitoring schedule is recommended:

• Monthly monitoring for the first 6 months
• Every 3 months for the next 6 months
• Every 6 months for 1 year thereafter 1

Laboratory Monitoring During Weaning

• Morning serum cortisol (drawn at approximately 8 AM) is the gold standard initial test
• ACTH level should be measured simultaneously with morning cortisol
• Interpretation thresholds:
  • ≥300 nmol/L (≥10.8 μg/dL): Adrenal insufficiency excluded
  • <110 nmol/L (<4 μg/dL): Adrenal insufficiency likely
  • 110-300 nmol/L: Further testing required 1

Recommended Weaning Schedule

1. Initial Assessment: Determine baseline morning cortisol and ACTH levels before beginning weaning

2. Gradual Dose Reduction:

   • Decrease the initial dose in small decrements at appropriate time intervals
   • Continue decreasing until reaching the lowest dosage that maintains adequate clinical response 2
   • Typical decrements are 2.5-5 mg every 1-4 weeks depending on duration of prior therapy

3. Final Weaning Phase:

   • When reaching 5 mg per day of hydrocortisone, consider substitution therapy
   • Evaluate adrenocortical response using morning cortisol measurements 3
   • Continue hydrocortisone until ACTH and endogenous cortisol levels return to normal values

4. Complete Withdrawal:

   • If adrenal function tests show recovery, hydrocortisone can be withdrawn
   • If tests show persistent suppression, continue hydrocortisone at physiologic replacement doses (15-25 mg daily in divided doses) 1

Special Considerations During Weaning

Stress Dosing During Weaning Period

Patients undergoing steroid weaning should follow stress dosing protocols:

• Minor illness/stress: Double or triple the usual daily dose
• Moderate stress: Hydrocortisone 50-75 mg/day in divided doses
• Severe stress: Hydrocortisone 100 mg IV immediately followed by 100-300 mg/day as continuous infusion or divided doses every 6 hours 1

Surgery Requirements During Weaning

For patients requiring surgery while on weaning schedule:

• Major surgery: 100 mg hydrocortisone IM just before anesthesia, followed by 100 mg IM every 6 hours until able to eat and drink. Then double oral dose for 48+ hours, then taper to normal dose 4

• Minor surgery: 100 mg hydrocortisone IM just before anesthesia, then double oral dose for 24 hours 4

Signs of Adrenal Insufficiency to Monitor

Watch for symptoms including:

• Fatigue
• Weakness
• Dizziness
• Nausea
• Weight loss
• Hypotension 1

Pitfalls and Caveats

1. Never stop hydrocortisone abruptly - this can precipitate adrenal crisis

2. Individual variation - Patients who received high-dose or long-term steroids are at higher risk of adrenal suppression and may require slower weaning 1

3. Stress coverage - Patients should be educated about stress dosing during illness or surgery even during the weaning process 4

4. Patient education - All patients should:

   • Recognize early symptoms of adrenal crisis
   • Wear medical alert identification
   • Carry a steroid alert card
   • Have an emergency hydrocortisone injection kit 1

5. Monitoring challenges - There is no established biomarker of cortisol activity, making clinical monitoring essential 5

By following these guidelines, the risk of adrenal crisis during hydrocortisone weaning can be minimized while allowing for recovery of the hypothalamic-pituitary-adrenal axis when possible.