This month, Allison King, MD, MPH, PhD, advises on how a patient with sickle cell disease (SCD) undergoing bariatric surgery should be managed.
A 33-year-old female presents with SCD. Past hemoglobin electrophoresis shows double heterozygosity for Hb S and Hb C: HGB F 3.2 Hb S = 46.7% Hb C = 50.1%. Baseline hemoglobin is in the 10s. She has relatively mild disease clinically, with mild-mod pain crises once every one to two years, none in the last few years. The patient attributes this to having moved to Hawaii. (She experienced more pain crises previously, when she lived at elevation.) Her most recent significant hospitalization was in 2009 when she had pneumonia, Hb of 5 g/dL with fevers, HAs, and required 4-5U PRBCs. She has been transfused ~10 times in her lifetime.
She is going to have bariatric surgery with a Roux en Y procedure. There are limited data published on how to manage these patients through such surgery, and we feel she should have exchange transfusion prior to surgery, though this is not available at our center or on the island of Hawaii. We do not have great experience with partial exchange. Please provide recommendations on how this patient should be managed.
EXPERTS MAKE THE CALL
Unless this patient has a history of stroke/acute chest/asthma, I would not exchange the patient. If she does have stroke/acute chest/asthma, we would do a manual exchange: pull off 10 cc/kg and give back 5 cc/kg of packed red blood cells. If you would like our protocol for manual exchange, please let me know.
Editor’s note: The doctor asking the question responded to Dr. King, asking for additional help over email. Here is that exchange:
“Dr. King, thank you so much for your response. The only article regarding surgery in patients with SCD I could find was from 1998 (Neumayr L, et al. Surgery in patients with hemoglobin SC disease. Am J Hematol. 1998;57:101-8.). It found that patients undergoing abdominal surgery had high rates of sickle cell crises if they were not transfused, but I recognize the study was small and the article is old. An article in UpToDate only included a small paragraph that recommends partial exchange in the pre-op setting to keep Hgb S <30 percent, so it’s not clear. We just don’t have a large population with this disorder.”
Dr. King then shared a sickle cell order set, developed by the sickle cell team (comprising mostly nurses and nurse practitioners) at St. Louis Children’s Hospital – Washington University, with the doctor who asked the question. View the order set.
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