P123: A SYSTEMATIC REVIEW OF THE BARRIERS TO UPTAKE AND ADHERENCE TO HYDROXYCARBAMIDE FROM THE PERSPECTIVE OF CHILDREN AND ADULTS WITH SICKLE CELL DISEASE, PARENTS AND CLINICIANS.
Authors: C Clark
Journal: HemaSphere (2022)

Aim: The aim of this study was to identify the reasons for the poor uptake and adherence to hydroxycarbamide from the perspective of people with sickle cell disease, parents of children with sickle cell disease and clinicians of people with sickle cell disease. Background: Children and adults with sickle cell disease have health related complications which impact on their health, quality of life and life expectancy. Hydroxycarbamide has been demonstrated to offer clinical benefits with minimal toxicities but uptake and adherence to hydroxycarbamide remains poor. Method: This study was a systematic review. A search strategy covered several databases including Academic Search Complete, Medline, CINAHL Complete, SocIndex, PubMed, Psych Info, British Nursing Index, Cochrane and Science Direct. An inclusion and exclusion criteria was applied and 9 studies were then critically appraised using validated appraisal tools. Data was extracted, themes identified and results and findings synthesised. Results: Four key themes and further subthemes were identified. Safety of hydroxycarbamide, subthemes: side effects, long term side effects, still experimental Knowledge and Understanding, subthemes: knowledge of hydroxycarbamide, knowledge of sickle cell disease, perceived effectiveness of hydroxycarbamide, perception of disease severity, perception of therapeutic effect in the community Clinician/healthcare, subthemes: clinician choice, clinician relationship trust in medical system Burden of taking, subthemes: remembering to take, aversion to taking pills, clinic reviews (missed work, school), number of blood tests, obtaining prescriptions/refills, costs, transport Conclusions: Whilst scientific evidence suggests that the benefits of hydroxycarbamide far outweigh the risks, the participants in the nine included studies report barriers that effect its utilisation. Whilst many of the concerns have been reported previously this systematic review confirms that these barriers still exist. Clinicians and healthcare professionals must explore barriers with their colleagues, patients and families and need to make provisions to help reduce the burden of treatment with hydroxycarbamide. Continuing discussions and education throughout childhood, transition and into adulthood will enable patients and families to make informed decisions. Further qualitative studies would enhance the findings of this research. References of Included Studies 1. Haywood, C., Beach, M.C., Bediako, S., Caroll, C.P., Lattimer, L., Jarrett, D. and Lanzkron, S, American Journal Haematology 2011; 68:1 2. Oyeku, S.O., Driscoll, C., Cohen, H.W., Pashankar, F., Mullen, C., Giardina, P.J., Velazco, N., Racine, A.D. and Green, N.S, Pediatric Blood Cancer, 2013; 60:4 3. Bekele, E., Thornburg, C.D., Brandow, A.M., Sharma, M., Smaldone, A.M., Jin, Z. and Green, N.S, 2014: 61:9 4. Creary, S., Zickmund, S., Ross, D., Krishnamurti, L. and Bogen, D.L, Biomedical Research Notes, Pediatric Blood Cancer, 2015; 8:372 5. Vaidyanathan, A. Dissertation.University of Pittsburgh, http://d-scholarship.pitt.edu/26082/, 2015; 6. Badawy, S.M., Thompson, A.A., Lais, J.S. and Liem, R.I, European Journal of Haematology, 2017;98:6 7. Bakshi, N., Sinha, C.B., Ross, D., Khemani, K., Loewenstein, G. and Krishnamurti, L, PLOS ONE, 2017; doi: 10.1371/journal.pone.0178413 8. Cevher, F., Ünal, S., Küҫükkavruk, Ş.P. and Tunҫtan, B, Turkiye Klinikleri Journal of Medical Science, 2018; 38:1 9. Sinha, C.B., Bakshi, N., Ross, D. and Krishnamurti, L, PLOS ONE, 2018; doi:10.1371/journal.pone.0199375 Key References 1. Hankins, J.S., Ware, R.E., Rogers, Z.R., Wynn, L.W., Lane, P.A., Scott, P and Wang, W.C, Blood, 2005; 106 2. Hankins, J.S., Aygun, B., Nottage, K., Thornburg, C., Smeltzer, M.P., Ware, R.E., and Wang, W.C, Medicine, 2014; 93: 28 3. Nevitt, S.J., Jones, A.P., and Howard, J. Hydroxyurea (hydroxycarbamide) for sickle cell disease. (Review) Cochrane Database of Systematic Reviews, 2017; 4 4. Public Health England (PHE) SCD in childhood; standards and guidelines for clinical care, 2010; 2 5. Qureshi, A., Kaya, B., Pancham, S., Keenan, R., Anderson, J., Akanni, M. and Howard, J. (on behalf of British Society of Haematology - BSH), British Journal of Haematology, 2018; 181 6. Wang, W.C., Ware, R.E., Miller, S.T., Iyer, R.V., Casella, J.F., Minniti, C.P., Rana, S., Thornburg, C.D., Rogers, Z.R., Kalpatthi, R.V., Barredo, J.C., Brown, R.C., Sarnaik, S.A., Howard, T.H., Wynn, L.W., Kutlar, A., Armstrong, F.D., Files, B.A., Goldsmith, J.C., Waclawiw, M.A., Myron, A., Huang, X. and Thompson, B.W, The Lancet, 2011; 377:9778)