Pain Burden in the CASiRe International Cohort of Sickle Cell Patients: United States and Ghana

Author:

Zempsky William T1,Yanaros Mary2ORCID,Sayeem Mohammed2,Boruchov Donna1,Piccone Connie M3,Manwani Deepa4,Strunk Crawford5,Tartaglione Immacolata6,Colombatti Raffaella7,Akatue Sophia8,Oteng Bianca8,Owda Ahmed8,Bamfo Rose8,Wilson Samuel8,Rivers Angela9,Farooq Fatimah8,Urbonya Rebekah8,Boatemaa Gifty Dankwah10,Rao Sudha11,Inusa Baba12,Antwi-Boasiako Charles1013,Segbefia Catherine11,Sey Fredericka13,Andemariam Biree14,Asare Eugenia Vicky13,Campbell Andrew D815

Affiliation:

1. Department of Pediatrics, University of Connecticut School of Medicine, Connecticut Children’s Medical Center , Hartford, Connecticut, USA

2. University of Connecticut School of Medicine , Farmington, Connecticut, USA

3. Pediatric Hematology/Oncology, Rainbow Babies and Children's Hospital , Cleveland, Ohio, USA

4. Department of Pediatrics, Albert Einstein College of Medicine, Children's Hospital at Montefiore , Bronx, New York, USA

5. ProMedica Russell J. Ebeid Children’s Hospital , Toledo, Ohio, USA

6. Department of Woman, Child and Generalized and Special Surgery, University of Campania “L. Vanvitelli,” Naples, Italy

7. Department of Women’s and Children’s Health, University of Padua , Padua, Italy

8. Division of Pediatric Hematology/Oncology, University of Michigan , Ann Arbor, Michigan, USA

9. UCSF Benioff Children's Hospital Oakland , Oakland, California, USA

10. Department of Physiology, University of Ghana Medical School , Accra, Ghana

11. Department of Child Health, University of Ghana Medical School , Accra, Ghana

12. Department of Pediatrics, Paediatric Haematology, Guys and St Thomas Hospital , London, UK

13. Ghana Institute of Clinical Genetics, Korle Bu Teaching Hospital , Accra, Ghana

14. New England Sickle Cell Institute, University of Connecticut Health Center , Farmington, Connecticut, USA

15. Center for Cancer and Blood Disorders, Children’s National Medical Center , Washington, DC, USA

Abstract

Abstract Objectives Sickle Cell Disease (SCD) is a genetic blood disorder affecting over 1 million people globally. The aim of this analysis is to explore the pain burden of patients with SCD in two countries: the United States and Ghana. Methods The Consortium for the Advancement of Sickle Cell Research (CASiRe) was created to better understand the clinical severity of patients with SCD worldwide. Data regarding gender, SCD genotype, prior medical diagnoses, and validated pain burden measures were analyzed from the CASiRe database. The Sickle Cell Pain Burden Interview (SCPBI) was used to assess pain burden, the impact of pain on physical, emotional, and social function. Results Most subjects identified as Black/African American (n = 298, 97.0%). Patient ages ranged from 6 to 73 years. 35.9% resided in the United States, 64.1% resided in Ghana, 40.9% were men, and 58.7% were women. The mean SCPBI score for US SCD patients was 6.53(±5.89) vs 4.04(±5.10) for Ghanaian patients, P <0.001. Pain burden was higher in US men vs Ghanaian men (6.74(±5.68) vs 3.54(±4.46), P = .003) and in US women vs Ghanaian women (6.37 ± 6.06 vs 4.44(±5.54), P = .032). Pain burden was higher in US patients than Ghanaian patients for both the Hb SC/SBeta+ genotype (5.40(±5.29) vs 2.82(±4.86), P = .054) and Hb SS/SBeta0 genotype (6.79(±6.01) vs 4.49(±5.13), P = .003). Pain burden was significantly higher in SCD patients with comorbid conditions independent of geographic origin including stroke, cholecystectomy, gallstones, depression, and headache. Discussion US patients with SCD have a higher pain burden than Ghanaian patients. Further studies should investigate underlying contributors to pain burden in these populations and further explore the etiology of geographic differences in pain.

Funder

Global Blood Therapeutics

Grifols

Novartis

Pfizer

CRISPR/Vertex, Forma Therapeutics, Global Blood Therapeutics

NovoNordisk

Publisher

Oxford University Press (OUP)

Subject

Anesthesiology and Pain Medicine,Neurology (clinical),General Medicine

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