MON-502 Use of Continuous Subcutaneous Hydrocortisone Infusion Through Insulin Pump for Management of Primary Adrenal Insufficiency Complicated by Malabsorption and Frequent Hypoglycemia

Abstract A. Javed: None. A. Saif: None. M. Aguasvivas: None. P. Schroeder: None. P. Sack: None. Introduction: Continuous subcutaneous hydrocortisone infusion (CSHI) by insulin pump has been reported to lead to better physiologic replacement with lower requirements of daily hydrocortisone (HCT) and allows the patient to deliver boluses in acute illness. The use of insulin pump and continuous glucose monitor (CGM) are currently off label in patients with primary adrenal insufficiency poorly controlled on oral steroid therapy. Case: A 59-year-old female with Addison’s disease, primary hypothyroidism, primary ovarian failure, and severe gastrointestinal conditions including exocrine pancreatic insufficiency, gastroparesis, irritable bowel syndrome and acid reflux presented to our clinic after reading about off-label use of an insulin pump for continuous HCT delivery. She was frustrated with her steroid replacement regimen. She was taking sublingual HCT 1.75 mg at 12 and 2:30 am, and 2.5 mg at 7 am, 12 pm, 4 pm and 8:30 pm. In addition, she took sodium tablets 150 mg 4x daily and fludrocortisone 0.05 mg daily. She had tried higher doses of steroid and had major weight gain. Our first attempt to obtain the insulin pump for her was denied due to off label use. We tried a longer acting HCT regimen of 4 mg 3x daily with 5 mg of hydrocortisone acetate at 3 AM and fludrocortisone 0.05 mg daily. Historically she always had symptoms of low cortisol between 3-6 AM. She had hypoglycemic symptoms at night with weakness which she attributed to adrenal crisis. On several occasions her husband had given her a stress dose of HCT 100 mg intramuscularly with improvement in her symptoms. Her adrenal crisis symptoms and frequent HCT dosing were considered to be related to malabsorption and perhaps rapid metabolism of the HCT. She continued to have nocturnal symptoms and weight gain with the later HCT regimen. After repeated efforts, her insurance company approved the use of OmniPod5 with hydrocortisone (100 mg/ml). She required higher doses overnight: 0.75, 1.4, 1.6 and 1.3 mg/h at 12, 2, 4 and 6 am, respectively, and 0.5 mg/hr during the day (17.2 mg/day of basal HCT). She tried a CGM not integrated with the pump to track the overnight glucose. Based on the collected data, she bolused 0.5-1 mg of HCT if she had confirmed hypoglycemia less than 55mg/dL. The pancrelipase dose was increased, which resulted in less hypoglycemia. Lab test after 2 months on CSHI showed serial salivary cortisol throughout the day: 0.29, 1, 0.5 and 0.2 mcg/dL with lower levels at night and morning cortisol of 9.7 mcg/dL with suppressed ACTH 5.2 pg/mL. The physiologic replacement, which bypasses the gastrointestinal tract, prevents frequent crises. She is now able to sleep through the night with significant improvement in quality of life. Conclusion: CSHI in patients who are unable to tolerate conventional oral therapy is a viable option to replicate physiological circadian steroid pattern. In this case, concomitant use of CGM prevented nocturnal hypoglycemia. Monday, June 3, 2024