(P-TA-14) Use of Patient Narrative to Adjust Frequency of Red Cell Exchange in Sickle Cell Disease

Red Cell Exchanges (RCE) are used in the medical management of many patients with sickle cell disease (SCD). SCD patients receiving chronic RCE often describe a clear pattern of initial symptomatic improvement in response to RCE, followed by a decline in the week(s) leading up to their next exchange. While hemoglobin (Hb) S goals are often used to determine the appropriate interval between RCEs, it is unclear how providers use patient narrative to adjust RCE frequency. There is little published on optimizing the frequency of RCE to improve quality of life. Increase in energy and decrease pain severity and/or frequency are the most commonly described benefits of RCE. We present a case series where patient narrative was used to adjust RCE frequency, leading to subsequent improvements in patient symptoms. 

Patient one is a woman in her 3rd decade of life with HbSS who presented after delivery of her first child. She has experienced new spasms since her pregnancy. Her exchanges were every three months, but she feels she would benefit from more frequent RCE. She reports immediate improvement with RCE, noting an increase in energy the very next day and free of spasms for two months. By the end of two months, she starts feeling more fatigued and notices an increase in the frequency and intensity of the spasms.  Her RCE frequency was increased to every 8 weeks. 

Patient two is a woman in her 4th decade of life with HbSS. Her first RCE were 4-5 months apart to help manage acute pain crises. Her RCE frequency was increased to every 12 weeks, but she still describes significant fatigue keeping her in bed for entire weeks before her next exchange. Her RCE frequency was recently increased to every 8 weeks.


Study

Design/Methods: N/A. This is a case series.

Results/Findings: In both cases, increased frequency of exchange has resulted in a downtrend of pre-procedure HbS% (Figure A). Both patients also reported increased energy and improved management of their pain, with more effectiveness of their home pain medications.

Conclusions: SCD patients who respond well to RCE report increased energy and decreased pain severity and frequency of pain crises. The timeline of these benefits and their drop off seems to be directly associated with the frequency of their RCE. It is worth noting that both patients are on medical management to increase their HbF fraction (hydroxyurea); however, there have been no changes in doses throughout the changes in their exchange frequency. This timeline of symptoms is likely unique to each patient, and further research is needed to understand the factors that influence how different patients respond to RCE to better optimize and personalize care for patients who live with SCD.