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Pediatric Endocrinology

Managing Emergencies: Adrenal Crisis and Low Blood Sugar

At a Glance

Children with pituitary stalk interruption syndrome (PSIS) may develop cortisol deficiency, causing adrenal crisis and dangerously low blood sugar during illness or injury. A written sick-day plan, prescribed emergency hydrocortisone, and caregiver training help families respond quickly and seek emergency care.

The pituitary gland’s most critical job is signaling the adrenal glands to produce cortisol, often called the “stress hormone.” Cortisol is essential for maintaining blood pressure, energy levels, and blood sugar during physical stress [1].

In Pituitary Stalk Interruption Syndrome (PSIS), the signal to produce cortisol may be weak or absent, leading to Secondary Adrenal Insufficiency. While not every child with PSIS has this deficiency at diagnosis, it can develop over time as the pituitary function changes [2][3]. Important note: Stress dosing and emergency hydrocortisone are for children with a clinician-diagnosed or specifically suspected ACTH deficiency/adrenal insufficiency and must not be started or adjusted without a clinician-provided plan. If your child lacks cortisol, managing it becomes the highest priority in their care, as it is a life-threatening condition if left untreated during illness or injury [4].

Recognizing an Adrenal Crisis

An adrenal crisis occurs when the body’s demand for cortisol far outweighs its supply. This is a medical emergency. In infants and children, the signs can be subtle and easily mistaken for common illnesses like the stomach flu [1].

CRITICAL ACTION SEQUENCE FOR ADRENAL CRISIS:
If your child with adrenal insufficiency shows these Red Flags:

  1. Give the prescribed emergency IM hydrocortisone injection IMMEDIATELY without waiting for transport or laboratory testing [5].
  2. Call 911 or your local emergency number. Tell them your child has Adrenal Insufficiency and needs help.
  3. Do NOT give anything by mouth to a child who is unconscious, having a seizure, or unable to protect their airway.

Red Flags include:

  • Altered consciousness: Extreme sleepiness, inability to wake up, or appearing “out of it” [6].
  • Persistent vomiting: Ongoing inability to keep down oral medications or fluids [5].
  • Signs of shock: Cold or clammy skin, very pale appearance, or a rapid, weak pulse [7].
  • Seizures or convulsions: Often caused by a sudden drop in blood sugar [8][9].
  • Severe dehydration: Sunken eyes, no tears when crying, or a dry mouth [9].

The Danger of Severe Hypoglycemia

In children with PSIS, cortisol deficiency often goes hand-in-hand with low blood sugar (hypoglycemia). Because cortisol helps the body release stored energy, a child without enough cortisol can see their blood sugar plummet during illness [10][11].

In newborns, this might look like jitteriness, poor feeding, or blue-tinted skin (cyanosis - note: cyanosis is an emergency sign of oxygen deprivation that requires immediate assessment rather than just attributing it to low glucose) [1]. In older children, they may become shaky, sweaty, confused, or unusually irritable. Severe hypoglycemia is a medical emergency that requires immediate treatment and, often, an emergency steroid injection [4][12].

  • If the child is awake and able to swallow: fast-acting oral glucose (like juice) can be given.
  • If the child is unconscious, actively seizing, or unable to swallow: DO NOT give anything by mouth. Use prescribed glucagon or give IM hydrocortisone for known adrenal insufficiency, and call emergency services immediately.

Sick-Day Rules and “Stress Dosing”

To prevent a crisis, doctors use stress dosing (also called “sick-day rules”). When your child’s body is under physical stress, they need more hydrocortisone than their usual daily dose [6][7].

Standard triggers for stress dosing include:

  • Fever: Use the child’s individualized written action plan for the specific temperature threshold and dosing instructions [13].
  • Persistent Vomiting or Diarrhea: If your child has an ongoing inability to retain oral medicine (not just a single loose stool), they need an emergency injection immediately [5].
  • Major Injury: Significant trauma, such as a broken bone or a deep cut, requires an immediate stress dose [7].

Important: Always follow the specific, written Adrenal Insufficiency Action Plan provided by your child’s endocrinologist [6][14].

Your Emergency Toolkit

Because an adrenal crisis can happen quickly, if your child has known or suspected adrenal insufficiency and a clinician’s prescription, you must be prepared to act before medical professionals arrive.

  1. Emergency Injection Kit: You should always have an unexpired vial of injectable hydrocortisone (such as Solu-Cortef) and the training to administer it into the large muscle of the thigh [5][14]. Ensure school and caregivers are also trained. This injection can be life-saving if your child is unconscious or persistently vomiting [7].
  2. Medical Alert Identification: Your child should wear a bracelet or necklace at all times stating they have “Adrenal Insufficiency” and “Needs Stress Dose Steroids” [15]. This tells emergency responders exactly what to do if you are not there [14].
  3. Emergency Action Plan: Keep a copy of your doctor’s written instructions in your diaper bag, at school, and with any family members who care for your child [6].

When in doubt, it is always safer to follow your specific action plan to treat a potential crisis early than to manage a severe one in the hospital [6][16].

Common questions in this guide

What should I do first if my child with PSIS may be having an adrenal crisis?
If your child has clinician-diagnosed or specifically suspected adrenal insufficiency and shows crisis warning signs, give the prescribed emergency hydrocortisone injection into the muscle immediately, following your action plan, and call 911 or your local emergency number. Do not wait for transport or laboratory tests. Give nothing by mouth if your child is unconscious, having a seizure, or unable to swallow safely.
What signs could mean a child with PSIS is in adrenal crisis?
Warning signs include extreme sleepiness or inability to wake, persistent vomiting, cold or clammy skin, marked paleness, a rapid weak pulse, seizures, or severe dehydration. These symptoms can be mistaken for a stomach illness, but adrenal crisis is life-threatening and needs immediate emergency help.
What should I do if my child’s blood sugar seems dangerously low?
If your child is awake and can swallow safely, give fast-acting glucose such as juice. If the child is unconscious, actively seizing, or unable to swallow, do not give anything by mouth; use prescribed glucagon or, for known adrenal insufficiency, the prescribed emergency hydrocortisone injection, and call emergency services immediately.
When should my child use sick-day or stress-dose hydrocortisone?
Follow the individualized written plan for fever and other illness because the temperature threshold and dose vary by child. Ongoing vomiting or diarrhea that prevents oral medicine, or a major injury, may require an emergency injection or immediate stress dose as directed by the clinician. Contact the endocrine team or emergency services according to the plan.
Can adrenal insufficiency appear after a child is diagnosed with PSIS?
Yes. Not every child with PSIS has cortisol deficiency when first diagnosed, and pituitary function can change over time. The child’s endocrinology team may need to reassess cortisol or ACTH function periodically and should provide the emergency plan if deficiency is diagnosed or specifically suspected.
What should families keep ready for a PSIS adrenal emergency?
Keep an unexpired prescribed injectable hydrocortisone kit, know how to give it, and make sure school staff and other caregivers are trained. Your child should wear medical alert identification stating adrenal insufficiency and the need for stress-dose steroids, and a written action plan should be available at home, school, and with caregivers.

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References

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This page describes emergency signs and preparedness for children with PSIS and possible adrenal insufficiency for informational purposes only and does not constitute medical advice. Follow your child’s individualized endocrinology plan and call emergency services for suspected adrenal crisis.