What is the treatment for Sheehan syndrome?

Treatment for Sheehan Syndrome

The treatment for Sheehan syndrome is lifelong hormone replacement therapy targeting all deficient pituitary hormones, with immediate priority given to corticosteroid replacement to prevent life-threatening adrenal crisis. 1, 2

Immediate Management Priorities

Corticosteroid Replacement (First Priority)

• Hydrocortisone is the cornerstone of initial treatment and must be started immediately upon diagnosis or clinical suspicion, as adrenocorticotropic hormone (ACTH) deficiency can lead to fatal adrenal crisis 3, 4
• Typical dosing: hydrocortisone 15-25 mg daily in divided doses (morning and afternoon) to mimic physiologic cortisol secretion 1, 2
• In acute presentations with imminent adrenal crisis (hypotension, severe electrolyte abnormalities, altered mental status), initiate stress-dose hydrocortisone (100 mg IV bolus followed by 50-100 mg every 6-8 hours) before awaiting confirmatory test results 4, 5

Thyroid Hormone Replacement (Second Priority)

• Levothyroxine should be initiated only AFTER corticosteroid replacement is established, as thyroid hormone can precipitate adrenal crisis in untreated adrenal insufficiency 3, 4
• Start with low doses (25-50 mcg daily) and titrate based on free T4 levels, not TSH (which will be inappropriately low in central hypothyroidism) 1, 2

Comprehensive Hormone Replacement Strategy

Sex Hormone Replacement

• Estrogen-progesterone replacement therapy in premenopausal women improves quality of life, bone density, and cardiovascular health 1, 2
• The most common early clinical clue is failure of lactation and absent menstrual resumption after delivery complicated by severe postpartum hemorrhage 2, 5

Growth Hormone Replacement

• Growth hormone (GH) deficiency is the most frequently affected hormone in Sheehan syndrome, followed by prolactin 1, 2
• GH replacement has demonstrated positive effects on body composition, bone density, cognitive function, and quality of life, though cost-benefit analysis and individual patient factors must be considered 2
• GH therapy should be considered in symptomatic patients with confirmed deficiency after stabilization on other hormone replacements 1

Diagnostic Confirmation Approach

Clinical Recognition

• High index of suspicion is crucial: any woman with a history of severe postpartum hemorrhage presenting with nonspecific symptoms (fatigue, weakness, cold intolerance, weight changes) years or decades later should be evaluated for Sheehan syndrome 3, 4, 5
• The syndrome can present acutely (rare) or insidiously over years to decades after the initial obstetric event 1, 3

Hormonal Assessment

• Prolactin response to TRH stimulation is the most sensitive screening test in patients with typical obstetric history 2
• Measure baseline levels: prolactin, IGF-1, free T4, cortisol (8 AM), FSH, LH, estradiol 1, 2
• Dynamic testing (ACTH stimulation test, insulin tolerance test) confirms pituitary hormone deficiencies 1

Imaging

• MRI of the pituitary typically shows decreased gland size (empty or partially empty sella) in established cases 3, 4
• The American College of Obstetricians and Gynecologists notes that attention to Sheehan syndrome is warranted in placenta accreta spectrum patients given the clinical scenario and potential for hypoperfusion 6

Monitoring and Long-term Management

Regular Follow-up Requirements

• Monitor free T4 levels every 6-8 weeks when adjusting levothyroxine, then every 6-12 months once stable 1
• Assess cortisol replacement adequacy clinically (blood pressure, electrolytes, symptoms) rather than by serum cortisol levels 2
• Screen for complications: anemia, pancytopenia, osteoporosis, cognitive impairment 2

Patient Education Critical Points

• Patients must understand stress-dose steroid protocols: double or triple hydrocortisone during illness, injury, or surgery 1, 4
• Provide emergency medical identification (bracelet/card) indicating adrenal insufficiency 4
• Educate on signs of adrenal crisis requiring immediate medical attention 5

Common Pitfalls to Avoid

• Never start thyroid hormone before corticosteroid replacement - this can precipitate fatal adrenal crisis 3, 4
• Do not rely on TSH levels to guide thyroid replacement in central hypothyroidism; use free T4 instead 1
• Avoid dismissing nonspecific symptoms in women with remote postpartum hemorrhage history - diagnostic delay of years to decades is common and increases morbidity 3, 4, 5
• Do not overlook the syndrome in developing countries where it remains a substantial cause of maternal morbidity and mortality despite being rare in developed nations 1, 3