What are the management instructions for patients with Congenital Adrenal Hyperplasia (CAH) undergoing a stress crisis?

Management Instructions for Patients with CAH During Adrenal Crisis

Immediate Recognition and Emergency Response

Patients with CAH experiencing signs of adrenal crisis (severe weakness, vomiting, diarrhea, abdominal pain, confusion, or hypotension) require immediate administration of injectable hydrocortisone 100 mg intramuscularly or intravenously without delay, followed by urgent transport to the emergency department. 1, 2

Critical Warning Signs Requiring Emergency Injectable Hydrocortisone:

• Persistent vomiting or diarrhea preventing oral medication absorption 2, 3
• Severe abdominal pain with inability to keep fluids down 1, 2
• Altered mental status, confusion, or extreme lethargy 1, 2
• Signs of shock: severe weakness, dizziness, rapid heart rate, or low blood pressure 1, 2
• Any situation where oral hydrocortisone cannot be absorbed 4

Patient Education on Stress Dosing Protocol

Mild to Moderate Illness (Fever, Cold, Minor Infections):

• Double or triple the usual daily hydrocortisone dose immediately when illness begins 2, 3
• Continue elevated doses until 24-48 hours after symptoms resolve 4, 1
• For children, this typically means 2-3 times their maintenance dose 5, 6
• Monitor temperature, hydration status, and ability to tolerate oral medications 5, 7

Severe Illness or Inability to Take Oral Medications:

• Administer emergency injectable hydrocortisone 100 mg IM immediately 1, 2
• Call emergency services or proceed directly to the emergency department 1, 3
• Do not wait to see if symptoms improve—gastrointestinal illness is the most common trigger for adrenal crisis 2, 5

Essential Emergency Supplies and Preparedness

Required Items All CAH Patients Must Have:

• Filled prescription for injectable hydrocortisone (100 mg) with supplies for intramuscular administration 4, 7
• Written stress dosing guidelines specific to the patient's usual doses 7
• Medical alert bracelet or necklace clearly stating "adrenal insufficiency" and "requires stress-dose corticosteroids" 4, 1
• Emergency card with crisis management instructions 3

Critical Education Components:

• Demonstration and practice of injection technique for caregivers—studies show only 50% of caregivers receive proper training despite its importance 7
• Recognition that even mild gastrointestinal upset can precipitate crisis since patients cannot absorb oral medications when they need them most 2, 3
• Understanding that upper respiratory infections and gastrointestinal illnesses are the two most common precipitating events across all ages 5

Special Considerations for Pediatric CAH Patients

Children with CAH are at particularly high risk for adrenal crisis with hypoglycemia, especially those under 12 years of age 5

Age-Specific Risks:

• More frequent illness episodes and stress dosing requirements occur during childhood compared to adulthood 5
• Adrenal crisis with probable hypoglycemia has been documented in children as young as 1.1 years 5
• Low hydrocortisone doses and high fludrocortisone doses during childhood predict more stress dosing episodes 5

Pediatric Emergency Management:

• Initial fluid bolus of 10-20 mL/kg normal saline (maximum 1,000 mL) for hypotension 2
• Hydrocortisone 100 mg IV or IM immediately 1, 2
• Monitor for hypoglycemia and treat with IV dextrose if present 5

Common Pitfalls to Avoid

Critical Errors That Increase Mortality:

• Delaying treatment while waiting for diagnostic confirmation—this can be fatal 1, 2
• Underestimating the severity of gastrointestinal symptoms—these are the most common crisis triggers 2, 5, 3
• Failing to have a filled prescription for injectable hydrocortisone at time of diagnosis—less than 40% of patients receive this initially despite its life-saving importance 7
• Not providing written stress dosing guidelines—only 70% of caregivers receive these 7
• Inadequate fluid resuscitation alongside corticosteroid administration 1

Situations Requiring Extra Vigilance:

• Any surgical procedure requires stress-dose coverage: 100 mg hydrocortisone IM before anesthesia for major surgery 8
• Major dental procedures require 100 mg hydrocortisone IM beforehand and doubling oral doses for 24 hours 8
• Pregnancy, particularly third trimester and labor, requires increased dosing 8
• Strenuous physical activity and heat exposure can precipitate crisis 3

Note: Short-term high-intensity exercise does not require stress dosing in well-controlled CAH patients, but prolonged or extreme exertion may 9

Hospital Management Protocol

Emergency Department Treatment:

• Hydrocortisone 100 mg IV bolus immediately upon arrival 1, 2
• Rapid IV fluid resuscitation with normal saline: 1 liter over first hour, followed by 2-3 additional liters at slower rate 1, 2
• Continue hydrocortisone 50-100 mg IV every 6-8 hours for first 24 hours 4, 1
• Monitor electrolytes, blood pressure, and clinical response frequently 1, 2

Transition Back to Maintenance:

• Taper stress-dose corticosteroids over 3-5 days after clinical improvement 1
• Resume oral maintenance hydrocortisone at doubled doses for 48 hours to one week depending on illness severity 4, 1
• Restart fludrocortisone when hydrocortisone dose falls below 50 mg daily, as higher doses provide adequate mineralocorticoid effect 2

Prevention Through Education

Repeated stress-related glucocorticoid dosing education is essential—studies show this reduces but does not eliminate adrenal crises 5, 3

Key Educational Messages:

• Never run out of medications or emergency injectable supplies 3, 7
• Increase doses at the first sign of illness, not after symptoms worsen 2, 3
• Seek immediate medical attention for persistent vomiting, severe illness, or any doubt about oral medication absorption 1, 2, 3
• Inform all healthcare providers about CAH diagnosis and stress-dosing requirements 4, 3

Despite optimal education and management, adrenal crises occur at a rate of 6-8 per 100 patient-years, with mortality rates of 0.5 per 100 patient-years, emphasizing the ongoing life-threatening nature of this condition 4, 3