Three Wishes for Sickle Cell Disease: Results from a Multi-Stakeholder Vision-Casting Project in Tennessee

Matthew P Smeltzer (1), Taylor P Hodges (2), Jamie Whartenby (3), Jane S Hankins (4), Robert Davis (3), William O Cooper (5)

About the authors:

1 Division of Epidemiology, Biostatistics, and Environmental Health, School of Public Health, The University of Memphis, Memphis, TN

2 Department of Art, The University of Memphis, Memphis, TN

3 Center for Biomedical Informatics, The University of Tennessee Health Science Center, Memphis, TN

4 Department of Hematology, St. Jude Children’s Research Hospital, Memphis, TN

5 Departments of Pediatrics and Health Policy, Vanderbilt University, Nashville, TN

Abstract

Although sickle cell disease (SCD) is one of the most common genetic disorders in the US, disparities in research and funding persist. To better understand stakeholder priorities, we conducted a virtual vision-casting session utilizing a graphic recorder and content analysis. Stakeholders responded to the question: “If you had three magic wishes for SCD in TN, what would they be?”. Wishes for SCD centered around information and data, care and policy, and community. Better patient-centered information about treatments and modernization of data were high priorities. Stakeholders identified a need for heath equity, starting with lifetime continuity of care and access to curative treatment for all persons with SCD. Key points concerning the community included better patient inclusion in research, increased awareness, and greater public knowledge. SCD patients expressed a desire for honesty, transparency, compassion, and trust. Key areas to address in SCD include better data coordination, more influence on health policy, broader access to care and more community awareness, with the ultimate goal of improving the lives of persons with SCD. Using data to improve care and address health disparities will require researchers listening to stakeholders and understanding multiple perspectives to form unified goals.

Introduction

Although sickle cell disease (SCD) is one of the most common genetic disorders in the US, disparities in research and funding for SCD persist (1;2). Over 100,000 people are affected in the US, the vast majority are people of color and more than half are insured by Medicaid (3). Newborn screening for SCD has been universal for more than 30 years, yet, unlike cancer or cystic fibrosis, there is no national registry. Data, when available, are often fragmented and non-uniform across states and regions.

To address these issues, the Centers for Disease Control and Prevention is supporting efforts to establish state level surveillance programs in 9 US states through the Sickle Cell Data Collection (SCDC) program (4;5). The Tennessee (TN) statewide SCD surveillance program (TN-SCDC) began capacity-building in 2019 and the first aggregate data reports are scheduled for 2021. TN-SCDC is governed by an executive committee and a state-wide steering committee comprised of researchers, clinicians, public health practitioners, patients, patient advocates, third-party payers, and healthcare administrators (Appendix 1). As the state program moves from capacity building into data reporting, we convened a meeting of stakeholders to develop a clear vision to put data into action.

Methods

In February 2021, the TN-SCDC steering committee conducted a virtual vision-casting session utilizing a graphic recorder and content analysis (6). Each stakeholder was asked to respond to the question: “If you had three magic wishes for SCD in TN, what would they be?”. We shared the question with the committee 48 hours before the session. Results were shared verbally and via comments during a 90-minute session facilitated by two executive committee members. Stakeholders were encouraged to discuss verbally, but could also provide comments using the meeting chat function. Input received via email before the meeting was shared ver¬bally by the facilitators during the discussion. Utilizing the graphic recorder, the group built consensus around the key points.

Results

The wishes for SCD centered around three categories including disease information and data, care and policy, and community (Figure 1). Better information about treatments for patients and care providers, especially acute care providers, was desired. Modernization of data, making it more extensive, integrated, and accessible was a high priority for stakeholders. Ideally, these data could help improve processes in a continuous feedback loop geared to identify and measure gaps in care and access and provide action plan in a “plug and play” model. Patients and patient advocates were particularly interested in ensuring all types of healthcare providers understood and utilized up-to-date information on appropriate treatment of SCD and its many complications. All stakeholders expressed a desire to have SCD as a higher priority among legislators and healthcare providers. They also identified a need for heath equity, starting with lifetime continuity of care and access to curative treatment for all persons with SCD.

Figure 1. Three Wishes for Sickle Cell Disease in Tennessee

Figure 1

Key points raised concerning the community included better patient inclusion in research, increased awareness, and greater public knowledge about SCD. Participants desire patient education with relevant information that is more specific to age in message and format, for example, focusing on the transition period from pediatric to adult care. Cutting across the three categories identified, SCD patients expressed a desire for honesty, transparency, compassion, and trust.

Discussion

Stakeholders identified key areas to address with the TN-SCDC initiative, including better data coordination, more influence on health policy, broader access to care and more community awareness, with the ultimate goal of improving the lives of persons with SCD. Using data to improve care and address health disparities in SCD will require researchers listening to stakeholders and understanding multiple perspectives to form unified goals.

Acknowledgements

The authors would like to thank all members of the Tennessee Sickle Cell Data Collection Consortium and the Centers for Disease Control and Prevention.

Contributors: Conception and design of the study: MS, WC. Acquisition of data: TH. Analysis and interpretation data: MS, JW, JH, RD, WC. Drafting the article: MS, JW, JH, RD, WC. Revisions and final approval of the article: all authors.

Competing interests and financial disclosures: JSH receives research funding from Global Blood Therapeutics, and consultancy fees from Global Blood Therapeutics, bluebird bio, and Vindico Medical Education. MPS, JSH, WOC, receive funding from National Heart, Lung, and Blood Institute (NHLBI) award U01HL133996. MPS, WOC, BD, JW, and JSH received funding from Centers for Disease Control and Prevention (CDC) award NU58DD000019-01 during the conduct of this study. No other disclosures were reported.

Funding/Support and Role of Funder/Sponsor: This work was supported by a United States Federal Government cooperative agreement from the National Heart Lung and Blood Institute (U01-HL133996) and Centers for Disease Control and Prevention (CDC) award NU58DD000019-01.

Patient content: Not applicable.

Ethics approval: Not applicable.

References

(1) Hassell KL. Population estimates of sickle cell disease in the U.S. Am J Prev Med. 2010; 38:S512-21. doi:10.1016/j.amepre.2009.12.022.

(2) Farooq F, Mogayzel PJ, Lanzkron S, Haywood C, Strouse JJ. Compa-rison of US Federal and Foundation Funding of Research for Sickle Cell Disease and Cystic Fibrosis and Factors Associated With Research Productivity. JAMA Netw Open. 2020; 3:e201737. doi:10.1001/jamanetworkopen.2020.1737.

(3) Wilson-Frederick SH, Hulihan M, Anderson KK. Prevalence of sickle cell disease among Medicaid beneficiaries in 2012. Centers for Medicare & Medicaid Services Office of Minority Health. Published June 2019. Available at: https://www.cms.gov/About-CMS/Agency-information/OMH/Downloads/Data-Highlight-16-Sickle-Cell-Disease.pdf. (Accessed on 31/03/2021).

(4) Sickle Cell Data Collection (SCDC) Program. Centers for Disease Control and Prevention, U.S. Department of Health and Human Services; 2021. Available from: https://www.cdc.gov/ncbddd/hemoglobinopathies/scdc.html.

(5) Aluc A, Zhou M, Paulukonis ST, Snyder AB, Wong D, Hulihan MM. Using surveillance to determine the number of individuals with sickle cell disease in California and Georgia, 2005–2016. Pediatr Hematol Oncol. 2020; 37:747-51. doi:10.1080/08880018.2020.1779886.

(6) Krippendorff K. Content Analysis: An Introduction to Its Methodology, 4th Edition, SAGE Publications. Los Angeles CA, 2018.


Appendix 1. Tennessee Sickle Cell Data Collection Consortium 2021 Membership

Appendix 1