Background & Aims
Sickle Cell Disease is a disorder of the red blood cells and involving the haemoglobin. Sickle Cell
Disease is a combination of series of haemoglobinopathies with each patient exhibiting different
variants of the clinical manifestations from this; variants from this disease are obtained from
mutations that cause abnormalities in the red blood cells (Figueiredo et al (2020).
(Khamees et al.,2021) provide a global estimate of, approximately 3.2 million individuals living
with sickle cell disease, some sharing the same variants while others have different ones. These
variants or haemoglobin categories include: HbSS, HbSC, HbSB (Thalassaemia) and HbSE,
amongst others.
The study seeks to analyze the need for a paradigm shift in SCD treatments and health outcomes
Methods
A total of 55 “Sickle cell winning warriors” (i.e., SCD patients) participated in the study. 85% of
them were young adults and adults ranging in age from 23 – 45 years. They were recruited from
support groups in Nigeria and South Africa, and counselling sessions at a non-profit organization
in Nigeria. Questionnaires were passed on the groups. Others were recruited through phone calls
from social media correspondence from across the globe. 87% were participants from Nigeria,
another 8% from South Africa
Results
Based on surveying people with lived experience of SCD, most expressed a need for further
research on variants of the condition. Furthermore, many expressed experiencing tangible adverse
impacts of receiving treatments that are not tailored to their specific variant. The findings
underscore the urgent need for increased attention to create specific health outcomes for each
variant as side effects from treatments and medications that are not compatible with the SCD
variant further puts the patient at higher risk.
Conclusions
Sickle cell disease (SCD) has spread to a lot of countries with Nigeria having the the highest
prevalence globally. However, the prevalence of each variant aligns with the peculiarities of each
geographical location. Which implies that SCD and the complications in Nigeria differs from other
parts of the world. SCD is similar to cancer and participants from SA opine that HbSS and HbSE
are the prevailing variants while few in Nigeria ascertain HbSS and HbSC as the most common
variants. There are different types of cancer and each is treated differently. Just as breast cancer is
treated differently from prostate cancer, so should treatment for each SCD variant be different
References
Figueiredo, C.V.B., Santiago, R.P., da Guarda, C.C., Oliveira, R.M., Fiuza, L.M., Yahouédéhou,
S.C.M.A., Carvalho, S.P., Neres, J.S.D.S., Oliveira, A.M.D.J., Fonseca, C.A. and
Nascimento, V.M.L., 2021. Priapism in sickle cell disease: Associations between NOS3 and
EDN1 genetic polymorphisms and laboratory biomarkers. Plos one, 16(2), p.e0246067.
Khamees, I., Ata, F., Choudry, H., Soliman, A.T., De Sanctis, V. and Yassin, M.A., 2021.
Manifestations of HbSE sickle cell disease: a systematic review. Journal of Translational
Medicine, 19(1), p.262.
Masiello, D., Heeney, M.M., Adewoye, A.H., Eung, S.H., Luo, H.Y., Steinberg, M.H. and Chui,
D.H., 2007. Hemoglobin SE disease—a concise review. American journal of
hematology, 82(7), pp.643-649.
Uwan, S.E., 2019.. All Rise!: The Sickle Cell Community vs.The Medical Establishment.
Independently Published
Presenting Author
Oluwafemi Ajayi
Poster Authors
Kehinde Ajayi
Mtech
Unisa
Lead Author
Topics
- Access to Care