Abstract
Lead Author's Financial Disclosures Nothing to disclose. Study Funding None. Background/Synopsis At 26 weeks gestation, a 24-year-old pregnant woman with type 2 diabetes mellitus (T2DM) and hypertriglyceridemia (HTG) was hospitalized for her second episode of acute pancreatitis (AP) associated with triglycerides (TG) > 4000 mg/dL. Medical therapy including plasma exchange resulted in dramatic improvement of TG, and the remaining hospital course was uneventful. She was discharged with plan for twice weekly plasma exchange. However, she did not have adequate childcare or transportation to enable treatment and the tunneled catheter had to be removed due to infection before she could receive any outpatient plasma exchange. This case highlights potential barriers to management of severe HTG and the need for effective therapy. Objective/Purpose To review a clinical scenario where severe inherited lipid abnormality and socioeconomic distress complicate care. Methods Laboratory testing was performed by University of Pennsylvania Health System Cernmill Laboratory. Results A 24-year-old G6P3 woman at 26 weeks gestation with T2DM presented to the hospital with intractable abdominal pain and was found to have diabetic ketoacidosis (DKA) and severe HTG causing AP (Table 1). Her medical history was notable for one prior episode of AP at age 21, during which she was first diagnosed with HTG and T2DM and treated with gemfibrozil and insulin. Subsequent medical care was disrupted by limited financial resources and housing insecurity. Follow-up lipid testing while on this regimen did not achieve TG less than 1000 mg/dl. She was admitted to the obstetrics unit for intravenous fluids and insulin and kept nill per os (NPO) resulting in resolution of the DKA. She underwent plasma exchange resulting in TG reduction and resolution of abdominal pain and elevated pancreatic enzymes. TG levels rose to >1200 mg/dL two days after plasma exchange despite clear liquid diet. A prolonged discussion about the risk of recurrent AP to her and her pregnancy, the interventions to manage HTG-associated AP, and available resources supported the role of once- or twice-weekly plasma exchange until delivery. Unfortunately, this plan was abandoned one week after discharge from the hospital because of inadequate access to appropriate childcare, transportation costs, and difficulty with safe maintenance of her tunneled IV access catheter. Present therapy includes close monitoring, dietary counseling, and combination of gemfibrozil and high dose omega-3 fatty acid supplementation. Conclusions This case illustrates a treatment dilemma stemming from the intersection of lipid biology, pregnancy, diabetes, and social determinants of health. Our patient has features of familial (FCS) and multifactorial chylomicronemia syndrome (MCS). Proper diagnosis may inform expectations from pharmacotherapy (e.g. gemfibrozil and high dose omega-3 fatty acid supplementation). In this case, the decision was made to initiate twice-weekly plasma exchange until delivery. There are no clinical trials to directly inform clinical decision-making for this rare condition. However, HTG-associated AP during pregnancy is reportedly more severe than non-pregnancy associated AP and intensive treatment is sensible given the expectation of worsening HTG in the last trimester and the high stakes for the patient and fetus. Unfortunately, our patient's socioeconomic barriers did not allow for appropriate treatment with plasma exchange and the social safety net could not solve these problems in the time required. This case serves as a reminder that our patients may face barriers to accessing complex healthcare and further highlights the need for more effective, convenient, and reliable HTG treatments for both FCS and MCS. Nothing to disclose.
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