Emergency Care: Preventing and Managing Adrenal Crisis

Because children with STAR deficiency cannot produce the “stress hormone” cortisol, their bodies cannot respond naturally to illness or injury ^[1]. Without the ability to ramp up cortisol levels, a common cold or a fever can rapidly escalate into a life-threatening medical emergency known as an adrenal crisis ^[2][3].

Recognizing an Adrenal Crisis

An adrenal crisis occurs when the body’s demand for cortisol far exceeds its supply ^[1]. This is a medical emergency that requires immediate action. Signs that your child is entering a crisis include:

• Severe Lethargy: Extreme sleepiness or being difficult to wake up ^[4].
• Vomiting: Persistent vomiting or diarrhea, inability to retain medication ^[5][4].
• Altered Mental Status: Confusion, irritability, or acting unlike themselves ^[6].
• Physical Collapse: Pale skin, weak pulse, or fainting (signs of dangerously low blood pressure or shock) ^[6][7].

Important: Do not wait for severe lethargy, collapse, or shock to develop before acting.

Sick Day Rules: Oral Stress Dosing

When your child is ill but still alert and able to swallow, you must provide “stress doses” of hydrocortisone. This mimics the extra cortisol a healthy body would make during sickness ^[8][9].

You must follow a clinician-specific algorithm for your child. Do not rely on universal rules.

• Fever: Your endocrinologist will define specific thresholds and tell you when to adjust the oral dose based on age and weight ^[5].
• Dosing Frequency: These stress doses are typically divided and given every 6 to 8 hours to ensure a steady supply ^[5].
• Minor Injury: For small bumps or minor scrapes, stress dosing is usually not needed. However, for significant trauma (like a broken bone) or surgery, higher doses are required ^[1].

When to Inject: The Emergency Shot

If your child cannot take oral medication, you must bypass the stomach by giving an emergency injection of hydrocortisone (such as Solu-Cortef) ^[10][11].

You must give the prescribed emergency injection and call emergency services at the same time or immediately afterward if your child:

1. Experiences persistent vomiting or diarrhea, or an inability to swallow ^[5]. (If a dose is vomited shortly after taking it, follow your clinic’s specific protocol regarding repeating the dose versus injecting).
2. Is too weak or lethargic to swallow medication or has altered consciousness ^[6].
3. Shows signs of shock (pale, cold skin, or fainting) ^[6].
4. Experiences a major trauma, such as a serious car accident or a deep wound ^[1].

The injection is given into the large muscle of the outer thigh (intramuscularly) ^[8]. It is better to give the injection “too soon” than to wait until a crisis is full-blown. Doctors and paramedics can always monitor a child who received an unnecessary shot, but they cannot easily reverse a crisis that has gone on too long ^[4][8].

The Emergency Action Plan

Every child with STAR deficiency must have a written, clinician-completed Emergency Action Plan ^[2]. It must contain the child’s exact dose, preparation/reconstitution instructions, route, and local emergency number. This document is your “voice” in a crisis and should be provided to:

• School/Childcare: Teachers and nurses must know when to call you and when to give the injection ^[2].
• EMS/Paramedics: Show this plan immediately. Adrenal crisis treatment generally requires prompt parenteral hydrocortisone plus isotonic fluids, with glucose when hypoglycemia is present ^[2][3]. Solu-Cortef is not an alternative to fluids; they are used together.
• The Emergency Room: Triage staff need to see this plan immediately so they understand adrenal crisis is suspected ^[2].

Emergency Kit Essentials

Always keep a “Go-Bag” ready that includes ^[3][12]:

1. Your unexpired injectable hydrocortisone (Solu-Cortef) kit.
2. The correct syringes and needles for your child’s age.
3. A copy of the Emergency Action Plan.
4. Extra oral hydrocortisone tablets.
5. A medical alert bracelet or necklace worn by the child at all times ^[3].

Prevention is the best management. By acting quickly and following your endocrinologist’s specific plan at the first sign of illness, you can prevent most adrenal crises from reaching a critical stage ^[10].