SAT-423 Case of Adrenal Insufficiency in 11 yo Male on Cortisol Pump

Abstract Disclosure: N.M. Goldenberg: None. M. Bornovali: None. Background: Oral steroids are used for treating adrenal insufficiency, but some patients are not always able to tolerate them or not able to control their symptoms. In such cases, cortisol pumps can be recommended. Clinical Case: 11 yo male with autism, EDS; autonomic dysfunction; mitochondrial dysfunction, Charcott Marie-Toot and immunodeficiency. At age 9 he was started on IVIG and required intermittent high doses of steroids. By age 10 he developed fatigue, episodes of low blood glucose and blood pressure. He was found to have low random cortisol and low ACTH stimulation test response (cortisol 4.3->10.6->4.7), ACTH was low. He was diagnosed with tertiary adrenal insufficiency and started on oral hydrocortisone. He required frequent rescue Solu-Cortef injections, emergency room trips and admissions to the hospital. He gained 50 lbs and stopped growing between age 10 and 11 and therefore his parents and the patient were looking into alternative treatment. Patient was switched to the cortisol pump with circadian rhythm cortisol delivery. He started growing and his weight stabilized. He and his parents reported improved quality of life, since the patient started sleeping well through the night and had better energy and he had much less adrenal pre-crisis, that they were able to control with small doses of steroids with pump (1-2 units) or IM injections of 25-50 mg of Solu-Cortef in more severe cases. He went into puberty at age 13 and had grown to 6 feet. He was 80th %time at age 11 and currently at age 17 is 95.7th %tile for hight. His BMI was 97th %tile at age 11 and at age 17 is at 93th %tile. He did not require any admissions to the hospital related to Adrenal insufficiency over the last 2 years. Unfortunately, he has developed other medical issues including portal hypertension, likely due to autoimmune hepatitis with hepato-splenomegaly and low platelets and therefore by November, 2024 he could not tolerate pump. He had bleeding into the injection sites and after 6 years of Cortisol Pump treatment had to find the alternatives. He was started on delayed release prednisone (Rayos) at bedtime in addition to injectable hydrocortisone in different doses through the day in circadian rhythm pattern and tolerates this regimen well. Conclusion: This case demonstrates the potential of cortisol pumps to improve the quality of life of patients with inadequately controlled adrenal insufficiency long-term. Delayed Release prednisone (Rayos) can help with management of poorly controlled AI. Presentation: Saturday, July 12, 2025