Emergencies and Urgent Care

While APS-1 is a chronic condition, it carries two specific risks that are true medical emergencies: adrenal crisis and acute hypocalcemia (critically low calcium) ^[1].

Crucial Distinction: Not every person with APS-1 will develop adrenal insufficiency or hypoparathyroidism. You only need to carry an emergency hydrocortisone kit, a medical ID bracelet, and specific sick-day action plans if your doctor has confirmed you have these organ failures and prescribed these tools. Carrying or taking emergency medicine without an individualized prescription can be harmful.

Adrenal Crisis: The “Addisonian” Emergency

If you have been diagnosed with primary adrenal insufficiency (Addison’s disease), your body cannot produce enough cortisol to handle physical stress like an infection, injury, or surgery ^[2]. An adrenal crisis occurs when your body’s demand for cortisol far exceeds its supply ^[3].

Warning Signs of Adrenal Crisis:

• Profound Weakness: A sudden, “bone-deep” exhaustion that makes it hard to stand or speak ^[4].
• Severe Gastrointestinal Distress: Nausea, repeated vomiting, or intense abdominal pain ^[5].
• Low Blood Pressure: Dizziness, fainting, or a cold, clammy feeling ^[6].
• Confusion or Fever: Unexplained high fever or a sudden change in mental clarity ^[6].

Emergency Action: Your Prescribed Sick-Day Plan

Adrenal emergencies require you to follow a clinician-approved written plan ^[7]. Generic advice is unsafe, but your plan will likely include:

1. Stress Dosing: For minor illnesses with a fever, you will temporarily increase your oral hydrocortisone dose based on your specific age, weight, and prescribed formulation ^[7].
2. The “Vomiting Rule”: A single vomit may only require repeating an oral dose (ask your prescriber). However, if you are persistently unable to keep your medication down, you must use your emergency hydrocortisone injection ^[8][9].
3. Inject and Go: Administer the intramuscular injection exactly as prescribed (the dose is weight/age-specific) and call local emergency services or go to the ER immediately ^[9][10]. Do not wait for symptoms to improve or for lab confirmation.

Acute Hypocalcemia: The Calcium Emergency

If you have hypoparathyroidism, low calcium levels (hypocalcemia) can occur. When calcium drops too low, it affects how your muscles and nerves function, and severe cases can impact the heart ^[11].

Warning Signs of Low Calcium:

• Paresthesia: Tingling or “pins and needles” around the mouth, or in the fingertips and toes ^[11].
• Muscle Cramps and Twitches: Sudden, painful spasms in the hands or feet (known as carpopedal spasm) ^[12].
• Tetany: Severe muscle stiffness where the hands or feet may “lock” into a position ^[13].
• Stridor: A high-pitched, gasping sound when breathing, caused by spasms in the voice box (laryngospasm). This is a critical emergency ^[13].
• Seizures: Very low calcium can cause the brain to misfire, leading to a seizure ^[13].

Action for Low Calcium:

Early signs like mild tingling should prompt you to call your endocrine team based on an individualized threshold they set for you. Do not repeatedly increase your calcium or calcitriol beyond your written plan, as this can severely damage your kidneys ^[14].

If you experience tetany, stridor, breathing difficulty, or a seizure, this is a medical emergency requiring intravenous (IV) calcium in a hospital ^[15]. Call emergency services immediately ^[16].

Your Emergency Toolkit (If Prescribed)

If your clinical team determines you are at risk, you should have three things ready at all times:

1. Emergency Injection Kit: An unexpired vial of hydrocortisone and supplies ^[9].
2. Medical ID: A bracelet or necklace stating your specific conditions (e.g., “Adrenal Insufficiency” and/or “Hypoparathyroidism”) ^[7].
3. Written Action Plan: A clear, one-page guide from your doctor detailing exactly how much medicine to take and when ^[10]. Ensure school, work, and caregivers are trained on this plan.