Case 5: Challenges in the Management of Pancreatogenic (Type 3c) Diabetes

Abstract

A 33-year-old female with a history of hereditary pancreatitis and type 3c diabetes was admitted to the hospital with abdominal pain and nausea. The endocrine service was consulted for inpatient management of diabetes. She experienced her first episode of pancreatitis at the age of 5 and was soon after diagnosed with hereditary pancreatitis. On genetic testing, she was found to have an R122H PRSS1 (protease serine 1) variant, which is an activating mutation in trypsinogen and the most common variant in patients with autosomal-dominant hereditary pancreatitis. She underwent a partial pancreatectomy and jejunostomy at age 14 but continued to have chronic abdominal pain and recurrent pancreatitis episodes, requiring chronic opioids for pain management. Persistent abdominal pain impeded her ability to maintain consistent follow-up. This sporadic treatment contributed to inadequate compliance with her diabetes regimen. Issues with insurance and medication cost led to inconsistent treatment with oral pancreatic enzyme replacement therapy.