What is the treatment plan for Addison's disease?

Treatment of Addison's Disease

Addison's disease requires lifelong dual hormone replacement with oral hydrocortisone 15-25 mg daily (divided into 2-3 doses) plus fludrocortisone 50-200 μg once daily, with the first hydrocortisone dose taken immediately upon waking and stress-dose adjustments mandatory during illness or surgery to prevent fatal adrenal crisis. 1

Glucocorticoid Replacement Strategy

Hydrocortisone is the first-line glucocorticoid, administered in divided doses to approximate the natural cortisol rhythm 1:

• Starting dose: 15-25 mg daily divided into 2-3 doses, with the first dose immediately after waking and the last dose at least 6 hours before bedtime 1
• Dosing schedule: Give the largest portion in the morning (e.g., 10 mg upon waking, 5 mg at lunch, 5 mg late afternoon) to mimic physiological cortisol secretion 1
• Pediatric dosing: 6-10 mg/m² of body surface area daily 1
• Use the lowest effective dose to minimize long-term metabolic complications while maintaining patient well-being 1, 2

The FDA-approved alternative is cortisone acetate 10-37.5 mg daily in divided doses, though hydrocortisone is preferred 3. Synthetic glucocorticoids like prednisone or dexamethasone have undesirable metabolic long-term effects and are less suitable as first-line treatment 2.

Mineralocorticoid Replacement

Fludrocortisone 50-200 μg (0.05-0.2 mg) once daily is essential for aldosterone replacement 1, 3:

• Standard starting dose: 100 μg (0.1 mg) daily 3
• Children and younger adults typically require higher doses within this range 1
• If hypertension develops, reduce the dose but never discontinue completely 1, 4
• Monitor using: blood pressure, serum electrolytes (sodium/potassium), and assessment of salt cravings—not plasma renin activity 5, 4
• Patients should consume salt and salty foods freely and avoid licorice and grapefruit juice 1

Stress Dosing Protocols

Minor Illness or Stress

Double or triple the oral glucocorticoid dose during fever, infection, or minor illness 1, 6:

• Continue increased dosing until recovery
• No adjustment to fludrocortisone is needed 1

Major Surgery

Follow this algorithm based on surgical severity 5, 1:

Major surgery with long recovery:

• 100 mg hydrocortisone IM immediately before anesthesia 5
• Continue 100 mg IM every 6 hours until able to eat and drink 5
• Then double oral dose for 48+ hours, then taper to normal 5

Major surgery with rapid recovery:

• 100 mg hydrocortisone IM before anesthesia 5
• Continue 100 mg IM every 6 hours for 24-48 hours 5
• Then double oral dose for 24-48 hours, then taper 5

Minor surgery and major dental procedures:

• 100 mg hydrocortisone IM before anesthesia 5
• Double oral dose for 24 hours, then return to normal 5

Simple dental procedures:

• Extra morning dose 1 hour prior 5
• Double oral dose for 24 hours 5

Pregnancy and Delivery

• Small dose adjustments may be needed during third trimester based on blood pressure and electrolytes 5, 1
• At onset of labor: 100 mg hydrocortisone IM, repeat every 6 hours if necessary 5, 1
• Double oral dose for 24-48 hours postpartum 5, 1

Exercise

• Regular, accustomed activity requires no adjustment 5, 1
• For unaccustomed intense or prolonged exercise (e.g., marathon): take extra 5 mg hydrocortisone before the event plus additional fluid and salt 5, 1

Adrenal Crisis Management

Adrenal crisis is a life-threatening emergency requiring immediate treatment without waiting for diagnostic confirmation 6:

Immediate actions:

• Hydrocortisone 100 mg IV bolus immediately 1, 6
• 0.9% saline 1 L over first hour, then 3-4 L over 24-48 hours until hemodynamic improvement 1, 6
• Continue hydrocortisone 100 mg IV/IM every 6-8 hours until recovery 1, 6
• Identify and treat the precipitating cause (infection, trauma, GI illness) 5, 6

Common precipitants include vomiting/diarrhea (preventing medication absorption), infections, surgical procedures, injuries, and treatment non-compliance 5. The frequency is 6-8 crises per 100 patient-years 5.

Monitoring and Follow-up

Annual assessment is mandatory 1, 4:

• Measure weight, blood pressure, and serum electrolytes (sodium, potassium) 1, 4
• Assess general health, energy levels, morning nausea, and symptom timing patterns 4
• Screen thyroid function periodically (autoimmune hypothyroidism is common) 4
• Bone mineral density every 3-5 years to detect glucocorticoid-induced osteoporosis 1, 4

Important monitoring caveats:

• Serum cortisol levels are NOT useful for monitoring adequacy—hydrocortisone produces highly variable peaks and troughs 4
• ACTH levels remain elevated regardless of replacement adequacy and do not guide dosing 4

Essential Patient Education

Every patient must receive comprehensive education 1, 4, 6:

• Wear medical alert identification (bracelet/necklace) and carry emergency steroid card 1, 4, 6
• Carry emergency injectable hydrocortisone at all times 4, 6
• Understand when to double/triple doses during illness 1, 6
• Recognize signs of under-replacement (fatigue, nausea, hypotension) and over-replacement (weight gain, hypertension, hyperglycemia) 4
• Know that even mild stomach upset requires increased dosing since medication absorption is impaired when it's needed most 5