Sickle cell disease is a genetic disorder causing red blood cells to form crescent-like shapes. These can stick together, inhibiting blood flow and causing severe pain crises.

This project team formed the initial phase of a long-term endeavor to: 1) understand the separate and joint contributions of biological and nonbiological factors to phenotypic variability in sickle cell disease over the life-course; and 2) improve outcomes for people worldwide with sickle cell disease (SCD).

Faculty and students from the US (Duke), Jamaica, Cameroon and South Africa focused on tasks pertinent to their areas of expertise and interest, simultaneously integrating their parts into the whole. The team utilized existing clinical, social, environmental, genetic and phenotypic data from a sampling frame of approximately 1,036 children and 1,480 adults with homozygous SCD (HbSS) in the four countries.

This project aimed to reconcile patient datasets from the study sites, characterize relevant contextual dimensions of the four countries and conduct a proof-of concept study to evaluate and refine the preliminary conceptual framework.

Team members contributed to a new holistic model and new set of criteria to assess the role of the possible multifactorial sources of SCD heterogeneity; hypotheses, preliminary data and manuscripts for future externally funded grants; and recommendations for attending to challenges and areas of need in relation to integrative global health research and clinical practice in SCD.

One study looked at SCD in Cape Town, South Africa, where there is a low prevalence and high availability of treatment as compared to Yaoundé, Cameroon, where there is high SCD prevalence but fewer treatment resources. Researchers collected 57 total surveys and interviews; interviewed parents of pediatric SCD patients and adult SCD patients at local hospitals. Preliminary observations of the data included: many SCD patients in South Africa are immigrants or refugees from other African countries; the cost of treatment in Cameroon is a major source of struggle for SCD families; and nearly all the patients receive Hydroxyurea in SA whereas almost none do in Cameroon.

Another study explored how accessibility affects treatment for SCD and malaria in the hospital and village clinic spaces in Cameroon. The methodology included semi-structured interviews with health care workers and participant observation. One finding was that SCD is rarely seen or rarely recognized in the villages by healthcare workers because it can seem like anemia caused by more common diseases, such as malaria.

Timing

August 2015 – July 2016

Team Outputs

Charmaine D. M. Royal, Michael Babyak, Nirmish Shah, Shantanu Srivatsa, Kearsley A. Stewart, Paula Tanabe, Ambroise Wonkam, Monika Asnani. 2021. "Sickle cell disease is a global prototype for integrative research and healthcare." Advanced Genetics 2(1).

Paula Tanabe, Understanding the Intersection of Stigma and Self-Management in Sickle Cell Disease ($88,568 grant awarded from the National Institutes of Health and National Institute of Nursing Research, 2017)

Dominique Bulgin, Paula Tanabe, Monika Asnani, Charmaine D. M. Royal. 2018. “Twelve tips for teaching a comprehensive disease-focused course with a global perspective: A sickle cell disease example.” Medical Teacher, DOI: 10.1080/0142159X.2017.1420151.

A Comparison of the Social and Medical Support Systems with Health Outcomes for Families Affected by Sickle Cell Disease in Cameroon and South Africa (poster by Brittney Wittenbrink)

Accessibility of Treatment in Cameroon for Malaria and Sickle Cell Disease (poster by Carolyn Peterseim)

Reflections

Madelaine Katz

This Team in the News

Meet the Winners of the 2020 Bass Connections Student Research Awards

2015 Summer Research Series: Health as a Human Right in Cameroon

Brittney Wittenbrink: My Bass Connections Pathway

Experiential Learning at DGHI - Brittney Wittenbrink - #myDGHIsummer

Bass Connections is a great way to work toward the same goals with people in different disciplines from all over the world. I’ve already learned so much from the collaborations and experiences made possible by this project. —Brittney Wittenbrink

See earlier related team, Integrative Global Health Research on Sickle Cell Disease (2014-2015).