Global geographic differences in healthcare utilization for sickle cell disease pain crises in the CASiRe cohort

Crawford Strunk; Immacolata Tartaglione; Connie M Piccone; Raffaella Colombatti; Biree Andemariam; Deepa Manwani; Ashya Smith; Haikel Haile; Esther Kim; Samuel Wilson; Eugenia Vicky Asare; Angela Rivers; Fatimah Farooq; Rebekah Urbonya; Donna Boruchov; Gifty Dankwah Boatemaa; Silverio Perrotta; Ivy Ekem; Laura Sainati; Sudha Rao; William Zempsky; Fredericka Sey; Charles Antwi-Boasiako; Catherine Segbefia; Baba Inusa; Andrew D Campbell

doi:10.1016/j.bcmd.2021.102612

Global geographic differences in healthcare utilization for sickle cell disease pain crises in the CASiRe cohort

Blood Cells Mol Dis. 2021 Dec:92:102612. doi: 10.1016/j.bcmd.2021.102612. Epub 2021 Sep 21.

Authors

Crawford Strunk¹, Immacolata Tartaglione², Connie M Piccone³, Raffaella Colombatti⁴, Biree Andemariam⁵, Deepa Manwani⁶, Ashya Smith⁷, Haikel Haile⁷, Esther Kim⁷, Samuel Wilson⁷, Eugenia Vicky Asare⁸, Angela Rivers⁹, Fatimah Farooq⁷, Rebekah Urbonya⁷, Donna Boruchov¹⁰, Gifty Dankwah Boatemaa¹¹, Silverio Perrotta², Ivy Ekem⁸, Laura Sainati⁴, Sudha Rao¹⁰, William Zempsky¹², Fredericka Sey⁸, Charles Antwi-Boasiako¹³, Catherine Segbefia¹⁴, Baba Inusa¹⁵, Andrew D Campbell¹⁶

Affiliations

¹ Department of Pediatric Hematology/Oncology, ProMedica Russell J. Ebeid Children's Hospital, Toledo, OH, USA. Electronic address: Crawford.Strunk@ProMedica.org.
² Department of Women, Child and General and Specialized Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy.
³ Pediatric Hematology, Carle Foundation Hospital, Urbana, IL, USA.
⁴ Clinic of Pediatric Hematology Oncology, Department of Women's and Child Health, Azienda Ospedaliera-Università di Padova, Padova, Italy.
⁵ New England Sickle Cell Institute, Division of Hematology-Oncology, Neag Comprehensive Cancer Center, UCONN Health, University of Connecticut, Farmington, CT, USA.
⁶ Department of Pediatrics, Albert Einstein College of Medicine, Children's Hospital at Montefiore, Bronx, NY, USA.
⁷ Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA.
⁸ Ghana Institute of Clinical Genetics, Korle Bu Teaching Hospital, Accra, Ghana.
⁹ UCSF Benioff Children's Hospital Oakland, Oakland, CA, USA.
¹⁰ Department of Child Health, Korle Bu Teaching Hospital, Accra, Ghana.
¹¹ Department of Physiology, University of Ghana Medical School, University of Ghana, Accra, Ghana.
¹² Division of Pain and Palliative Care, Department of Pediatrics, University of Connecticut School of Medicine, Connecticut Children's Medical Center, Hartford, CT, USA.
¹³ Department of Physiology, University of Ghana Medical School, University of Ghana, Accra, Ghana; Ghana Institute of Clinical Genetics, Korle Bu Teaching Hospital, Accra, Ghana.
¹⁴ Department of Child Health, Korle Bu Teaching Hospital, Accra, Ghana; Department of Child Health, University of Ghana Medical School, University of Ghana, Accra, Ghana.
¹⁵ Department of Pediatric Haematology, Evelina Children's Hospital, Guy's and St. Thomas NHS Trust, London, UK.
¹⁶ Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA; Department of Pediatrics, Divison of Hematology, Children's National Hospital, George Washington University School of Medicine Health Sciences, Washington, DC, USA.

Abstract

Background: Sickle cell disease (SCD) is characterized by frequent, unpredictable pain episodes and other vaso-occlusive crises (VOCs) leading to significant healthcare utilization. VOC frequency is often an endpoint in clinical trials investigating novel therapies for this devastating disease.

Procedure: The Consortium for the Advancement of Sickle Cell Research (CASiRe) is an international collaboration investigating clinical severity in SCD using a validated questionnaire and medical chart review standardized across four countries (United States, United Kingdom, Italy and Ghana).

Results: This study, focused on pain crisis incidence and healthcare utilization, included 868 patients, equally represented according to age and gender. HgbSS was the most common genotype. Patients from Ghana used the Emergency Room/Day Hospital for pain more frequently (annualized mean 2.01) than patients from other regions (annualized mean 1.56 U.S.; 1.09 U.K.; 0.02 Italy), while U.K. patients were hospitalized for pain more often (annualized mean: U.K. 2.98) than patients in other regions (annualized mean 1.98 U.S.; 1.18 Ghana; Italy 0.54). Italy's hospitalization rate for pain (annualized mean: 0.57) was nearly 20 times greater than its emergency room/day hospital only visits for pain (annualized mean: 0.03). When categorized by genotype and age, similar results were seen.

Conclusions: Geographic differences in pain crisis frequency and healthcare utilization may correlate with variable organization of healthcare systems among countries and should be considered regarding trial design, endpoints, and analysis of results when investigating novel agents for clinical benefit.

Keywords: Complications; Geographic differences; Health resources utilization; Pain crisis; Sickle cell disease.

Publication types

Research Support, N.I.H., Extramural

MeSH terms

Adolescent
Adult
Anemia, Sickle Cell / complications*
Anemia, Sickle Cell / epidemiology
Anemia, Sickle Cell / therapy
Female
Ghana / epidemiology
Humans
Italy / epidemiology
Male
Pain / epidemiology
Pain Management*
Patient Acceptance of Health Care*
United Kingdom / epidemiology
United States / epidemiology
Young Adult

Grants and funding

T37 MD001425/MD/NIMHD NIH HHS/United States