Use of IV Hydration Instead of Exchange Transfusion for Prophylactic Management of Pain Crisis in Homozygous (SS) Sickle Cell Disease Helps Maintain Hgb Levels and Prevents Further Escalation of Iron Overload
Authors: Nagadia Urja , Brahmbhatt Niravkumar Kamleshbhai , Ghous Ghulam , Cherian Verghese 
Published: December 2023

Introduction: Indications for exchange transfusion in the management of sickle cell disease and its complications are over time becoming limited. Currently accepted indications include acute chest syndrome and cerebrovascular accidents only. However, there is often consideration of using exchange transfusions for management of sickle cell pain crisis episodes resistant to opioid-based pain management. This is, however, of questionable efficacy due to concerns of iron overload as well as alloimmunization. The excessive utilization of blood products also presents a significant challenge. Therefore, it is important to explore alternatives to exchange transfusion in the management of pain crisis in sickle cell disease.

Method: This study aimed at investigating the effects of transitioning patients with homozygous SS disease from routine exchange transfusions to IV hydration protocols. The main objective was to examine the changes in Hgb levels and the status of iron overload in these patients. A cohort of 16 patients with a Ferritin level above 1000 ng/ml, who were already receiving regular exchange transfusions, were included in the study. Their Hgb levels and Ferritin levels were measured periodically. This group was then given IV hydration twice a week, regardless of whether they were experiencing pain issues. We then compared the Hgb and Ferritin levels during a 6-month period of active exchange transfusions and 6-month period of being on IV hydration protocols. Both periods were a year apart, allowing for adequate homeostatic changes to take place. Additionally, we compared the number of simple transfusions during both these periods.

Result: The mean ferritin level in patients getting exchange transfusions was 6097 ng/mL with the mean Hgb level maintained at 7.48 g/dL. During the initial study period, there were 14 exchange transfusion sessions involving 96 units of packed red blood cells (PRBCs). Additionally, 8 units of simple transfusion units were administered during the same period. Interestingly, as this cohort transitioned away from exchange transfusions, the number of simple transfusions increased to 30. Only one exchange transfusion occurred during the second 6-month observation period. During this time, ferritin level stopped increasing, and the mean was measured at 6076 ng/ml. The Hgb levels did show a slight decrease, with the mean being 6.90 g/dL, but the difference was not statistically significant.

Conclusion: The findings of this study suggest that the use of IV hydration protocol is effective in managing iron overload issues and maintaining an adequate Hgb level in patients who were previously undergoing routine exchange transfusions to prevent or manage pain crisis episodes. The results indicate that IV hydration should be initiated when ferritin levels increase beyond 1000 ng/ml, rather than waiting for more advanced stages. Moreover, the study shows that even when IV hydration is started at a later stage of iron overload, it still appears to limit further iron accumulation, as evidenced by the stabilization of ferritin levels. Overall, the use of IV hydration as an alternative to routine exchange transfusions holds promise in managing pain crisis and iron overload in sickle cell disease.