Abstract

Abstract Background Conventional management of adrenal insufficiency (AI) includes lifelong oral glucocorticoid replacement therapy. Currently, no glucocorticoid regimens adequately mimic the circadian rhythm of cortisol secretion. In some patients, this causes persistence of severe symptoms. Continuous subcutaneous hydrocortisone infusion (CSHI) is an alternative delivery method using an insulin pump. Previous studies have confirmed CSHI's potential to re-establish the diurnal cortisol profile and reduce total daily dose (TDD) of hydrocortisone. To date no reports are available regarding the long-term usage (>2 years) of CSHI in adults. Methods We report the use of CSHI in 27 patients seen at the Scottsdale Endocrinology Institute from 05/2012 to present. CSHI inclusion criteria were persistence of severe symptoms, high steroid dosage, or gastrointestinal intolerance, malabsorption or bleeding in willing and able patients. Individual basal rates were set using a novel method to re-establish patients’ diurnal cortisol profiles. Patients were educated on pump basics including bolusing and temporary basal rates, allowing them to control their dosing during symptomatic and stressful periods and procedures, and using subcutaneous shots during pump failures. Quality of life (QOL) was assessed using the AddiQOL questionnaire periodically. Data was gathered from patient charts and surveys. We report patient preference of CSHI over oral therapy, and changes in QOL, TDD of hydrocortisone, and crises during pump therapy. Results In our patient sample, there were 2 males and 25 females, 9 had primary AI, 15 had secondary AI, and 3 had steroid-induced AI. Ages ranged from 26 to 78 years. 1 patient had 1 year of CSHI, 19 patients had 2-5 years of CSHI, and 7 patients had >5 years of CSHI. After at least 2 years of CSHI, 24/26 patients preferred CSHI over oral therapy, 25/26 patients continued to report improved symptoms and QOL, and average TDD reduction of hydrocortisone was 22 mg (2.5-55.8). 4 patients previously deemed disabled returned to a productive lifestyle. 2 patients underwent successful pregnancies without untoward events. Only 2 patients were hospitalized due to near adrenal crises. 4 chose to discontinue CSHI, 2 for a high out of pocket expense, and 2 for local skin reactions at infusion sites. Conclusion Our method of calculating basal rates and implementing CSHI was associated with improvement in symptoms and QOL and reduced TDD of hydrocortisone and hospitalizations over treatment duration. We expect this to reduce cumulative side effects of steroids and overall cost of treatment of these patients. Autonomy of dose regulation offered by pump therapy improves patient's ability to handle stressful situations and symptoms, including sleep quality and quantity. In summary, we found CSHI to be a safe and effective method to treat patients with AI experiencing problems with oral treatment over an extended period of time. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m., Saturday, June 11, 2022 1:12 p.m. - 1:17 p.m.

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