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PubMed This is a summary of 27 peer-reviewed journal articles Updated
Endocrinology

Life After Surgery and Long-Term Management of FH-III

At a Glance

Long-term FH-III care depends on the treatment path: after removal of both adrenal glands, lifelong hydrocortisone and usually fludrocortisone plus emergency planning are needed; medication treatment requires ongoing blood pressure, renin, potassium, and kidney monitoring.

Whether you manage the condition with medication or choose surgery, managing Familial Hyperaldosteronism Type III (FH-III) is a lifelong commitment. Both paths require a “new normal” that involves consistent monitoring and a deep understanding of how your body handles hormones [1][2].

Life After Surgery: Adrenal Insufficiency

If you or your child undergoes a bilateral adrenalectomy (removal of both adrenal glands), the body’s overproduction of aldosterone stops instantly. However, the body also stops producing cortisol and other essential hormones [3]. This state is called primary adrenal insufficiency [4].

Lifelong Replacement Therapy

You must replace these missing hormones every single day. Your team will provide an individualized written regimen, as doses vary widely by age, body size, residual adrenal function, blood pressure, and electrolytes. The regimen typically includes:

  • Glucocorticoids: Most patients take hydrocortisone (amounts like 15–25 mg daily for adults are merely illustrative examples, not fixed instructions), divided into two or three doses to mimic the body’s natural rhythm [4][5].
  • Mineralocorticoids: After removal of both glands, you will usually require fludrocortisone (doses vary) to help your kidneys balance salt and water [6][7].

Preventing and Managing Adrenal Crisis

An adrenal crisis (or Addisonian crisis) is a life-threatening emergency that occurs when the body does not have enough cortisol to respond to stress [8]. It can be triggered by infection, injury, or missing a dose of medication [9][10].

Warning Signs of Crisis:

  • Extreme weakness or profound lethargy [10].
  • Severe nausea, vomiting, or abdominal pain [11].
  • Confusion or altered mental status [8].
  • Dizziness or fainting due to very low blood pressure [9].

The Emergency Protocol:

  1. Sick-Day Rules: During a minor illness or fever, this involves following an individualized written sick-day plan specifying when to use oral stress doses [12][13].
  2. Injectable Steroids: If you have severe diarrhea, confusion, collapse, or are vomiting and cannot keep pills down, do not wait to see if oral doses work. You must use an emergency hydrocortisone injection (Act-o-Vial or similar) immediately and seek emergency medical care [14][15]. Ensure you regularly check kit expiration dates.
  3. Medical Identification: Always wear a medical-alert bracelet or necklace stating “Adrenal Insufficiency—Requires Hydrocortisone” [16][17].

Creating an Emergency Action Plan

Work with your team to create a one-page emergency action plan for yourself, caregivers, school, and sports staff detailing contact numbers, sick-day rules, and injection instructions. Address practical daily-life details such as school or work travel and where backup kits will be stored.

Life on Medical Management

If you choose to manage FH-III with medications like spironolactone, your focus is on preventing long-term damage from high aldosterone and high blood pressure.

The Monitoring Schedule

Medical management is not “set and forget.”

  • Early Titration: Potassium, creatinine/eGFR, blood pressure, and renin are checked soon after starting or changing an MRA. MRAs can produce high potassium and worsening kidney function, making close early monitoring critical [18][19][20].
  • Stable Follow-Up: A fixed “every 3–6 months” schedule applies only for stable follow-up and should not replace closer early monitoring [21][22].
  • Blood Pressure: Daily home logs are essential to ensure the medication is working around the clock [23].
  • Renin Levels: Doctors measure renin as one tool to see if the medication is successfully blocking aldosterone [21][24].

When to Re-evaluate

Medical management can become less effective over time. If kidney function begins to decline or blood pressure becomes impossible to control despite multiple medications, it may be time to revisit the conversation about surgery [25][1]. Regardless of the path, regular communication with an endocrinologist experienced in rare adrenal disorders is your best tool for long-term health [26][27].

Common questions in this guide

What happens when both adrenal glands are removed for FH-III?
The excess aldosterone production stops, but the body also loses its ability to make cortisol and other essential adrenal hormones. This causes primary adrenal insufficiency, so lifelong hydrocortisone and usually fludrocortisone replacement are needed under an individualized treatment plan.
What should I do if I cannot keep my steroid medicine down?
If vomiting or severe diarrhea prevents you from keeping oral medicine down, use the prescribed emergency hydrocortisone injection immediately and seek emergency medical care. Your care team should teach you and your caregivers how to use the kit and how to check its expiration date.
What are the warning signs of an adrenal crisis?
Severe weakness, profound tiredness, vomiting, severe nausea, abdominal pain, confusion, dizziness, or fainting can signal an adrenal crisis. Because an adrenal crisis is life-threatening, follow your emergency plan and seek urgent medical care, especially if oral medication cannot be kept down.
How often should FH-III medicines and kidney function be monitored?
After starting or changing an MRA such as spironolactone, doctors usually check potassium, blood pressure, renin, and kidney-function tests closely. Once treatment is stable, visits may occur every three to six months, but your clinician may recommend a different schedule, and home blood-pressure logs may be needed daily.
When should surgery be discussed again if medication is not controlling FH-III?
Surgery may need to be reconsidered if kidney function begins to decline or blood pressure remains difficult to control despite several medicines. Discuss these changes with an endocrinologist experienced in rare adrenal disorders.
What should an adrenal-insufficiency emergency plan include?
A written plan should list sick-day steroid instructions, emergency contacts, injection steps, and when to seek urgent care. It should also address medical-alert identification, backup injection kits, and how school, work, travel, sports staff, and caregivers will respond.

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References

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This page is for informational purposes only and does not constitute medical advice about FH-III, adrenal insufficiency, or medication doses. Your endocrinology team must tailor hormone replacement, monitoring, and emergency instructions to you or your child.

Guide contents Familial hyperaldosteronism type III
Research & Literature
04 Standard Treatment Strategies for FH-III 06 Building Your Care Team and Managing Family Genetics