Table 6 Recommendations for the management of AS
1. | Stress steroids during periods of physiological stress |
|---|---|
|  |    - Adrenal crisis: Hydrocortisone injection (Solu-Cortef) 100 mg/m2 (max. 100 mg) IV/IM stat with saline volume expansion, followed by 25 mg/m2 q 6 hours (max. 25 mg q 6 hours); call endocrinologist on call |
|  |    - Surgery: Hydrocortisone injection (Solu-Cortef) 50-100 mg/m2 IV (max 100 mg) pre-operatively, then 25 mg/m2 q 6 hours (max 25 mg q 6 hours); call endocrinologist on call |
|  |    - Illness or fever: 20 mg/m2/day hydrocortisone equivalent, divided BID or TID |
|  |    - Fever >38.5oC or vomiting: 30 mg/m2/day hydrocortisone equivalent, divided TID |
|  |    - Unable to tolerate orally: Hydrocortisone must be administered parenterally as Solu-Cortef, 25 mg/m2/dose q 6 hours IV or q 8 hours IM |
2. | ± Daily physiologic dose of hydrocortisone (8-10 mg/m 2 /day) |
3. | Family education |
|  |    - Stress steroid dosing |
|  |    - Emergency medical contact information in case of illness |
4. | Information card/Medic-Alert bracelet |