Document Type

Abstract

Publication Date

2-11-2026

Academic Year

2025-2026

Comments

Presented at the 2026 Scholarly Inquiry (SI) Research Project Symposium.

Abstract

Introduction/Purpose: Despite the availability of disease-modifying therapies (DMT), adults with sickle cell disease (SCD) experience a multitude of adverse health effects that impact quality of life (QOL), with pain being the most common complication. There are few studies that objectively assess the relationship between DMT use and QOL in adults. This study aims to understand whether the currently used tools are sensitive to the use of DMT.

Methods: Data included 141 adult patients from three institutions in a national sickle cell disease registry (GRNDaD). A validated questionnaire, specific for patients with SCD (ASCQ-Me), was used to assess QOL in 5 domains: emotional, social, sleep, stiffness, and pain. Statistical analysis included t-tests and logistic regression.

Results/Conclusions: In a model that controlled for sex, age, absolute neutrophil count (ANC), and hemoglobin (hgb), SCD patients with SS or S beta 0 thalassemia genotypes on hydroxyurea (HU) had significantly better social functioning impact scores compared to those not on HU (OR = 1.09, 95% CI [1.01, 1.18], p = 0.02). In the same model, increasing age was associated with lower social functioning impact scores. Additionally, patients on crizanlizumab experienced significantly better emotional impact scores (OR 2 = 1.07, 95% CI [1.00, 1.16], p = 0.043). The results shown indicate that DMT positively impacts QOL in social and emotional domains, however in the other domains there was no significant difference in scores. Further testing in larger populations is needed to see if these tools can be used in the clinical setting to assess response to therapy.

Language

English

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