Knowledge of and attitudes toward heel prick screening for sickle cell disease in Saint Lucia.

OBJECTIVES: In the Caribbean country of Saint Lucia, umbilical-cord-blood screening for sickle cell disease (SCD) was the testing method that health care workers (HCWs) on the maternity wards of the hospitals preferred until the new heel prick (HP) testing method was introduced in the country in 2014. This SCD study sought to assess HCWs' knowledge of and attitude toward HP screening and also determine new mothers' favorability toward HP screening. METHODS: A total of 70 HCWs and 132 new mothers answered survey questionnaires in three hospitals. In addition, four focus group discussions were held, two with HCWs and two with the mothers. RESULTS: Among the HCWs interviewed, 85.7% of them had knowledge of the HP test. However, only 25.7% had attended training sessions on the procedure. Among the HCWs, 64.3% of them felt the HP test should be mandatory, 27.1% said it should not be mandatory, and 8.6% did not know if it should be mandatory. In their focus groups, the HCWs said they believed the mothers would accept the HP method. For their part, 22.0% of the mothers said they had heard about the HP test, and 63.6% reported knowing the reason why the baby would be tested. Further, 83.3% indicated that the test would be beneficial for the baby. In addition, 88.6% of the mothers said that more information on the HP test was needed. In their focus group discussions, the mothers said they were generally not concerned about the pain the heel prick method might cause the baby. CONCLUSIONS: The HCWs' knowledge of the HP screening method was high. The mothers trust HCWs, and the mothers would accept the HP procedure irrespective of their knowledge of the test and any discomfort associated with this screening method.

Knowledge of and attitudes toward heel prick screening for sickle cell disease in Saint Lucia / Conocimiento y actitudes hacia la prueba del talón para la detección de la anemia de células falciformes en Santa Lucía / Conhecimento e atitudes em relação ao “teste do pezinho” para triagem de doença falciforme em Santa Lúcia

ABSTRACT Objectives In the Caribbean country of Saint Lucia, umbilical-cord-blood screening for sickle cell disease (SCD) was the testing method that health care workers (HCWs) on the maternity wards of the hospitals preferred until the new heel prick (HP) testing method was introduced in the country in 2014. This SCD study sought to assess HCWs’ knowledge of and attitude toward HP screening and also determine new mothers’ favorability toward HP screening. Methods A total of 70 HCWs and 132 new mothers answered survey questionnaires in three hospitals. In addition, four focus group discussions were held, two with HCWs and two with the mothers. Results Among the HCWs interviewed, 85.7% of them had knowledge of the HP test. However, only 25.7% had attended training sessions on the procedure. Among the HCWs, 64.3% of them felt the HP test should be mandatory, 27.1% said it should not be mandatory, and 8.6% did not know if it should be mandatory. In their focus groups, the HCWs said they believed the mothers would accept the HP method. For their part, 22.0% of the mothers said they had heard about the HP test, and 63.6% reported knowing the reason why the baby would be tested. Further, 83.3% indicated that the test would be beneficial for the baby. In addition, 88.6% of the mothers said that more information on the HP test was needed. In their focus group discussions, the mothers said they were generally not concerned about the pain the heel prick method might cause the baby. Conclusions The HCWs’ knowledge of the HP screening method was high. The mothers trust HCWs, and the mothers would accept the HP procedure irrespective of their knowledge of the test and any discomfort associated with this screening method.

RESUMEN Objetivo En el país caribeño de Santa Lucía, el hemocribado del cordón umbilical para la detección de la anemia de células falciformes o drepanocitosis era el método de tamizaje preferido por el personal de salud en las salas de maternidad de los hospitales hasta que se introdujo la nueva prueba del talón en el 2014. Este estudio sobre la anemia de células falciformes tuvo por objetivo evaluar el conocimiento y las actitudes del personal de salud hacia la prueba del talón, así como determinar si las madres tenían una actitud favorable hacia este método de tamizaje. Método Un total de 70 trabajadores de salud y 132 madres participaron en los cuestionarios realizados en tres hospitales. Además, se celebraron cuatro grupos de debate: dos con el personal de salud y dos con las madres. Resultados Del personal de salud entrevistado, 85,7% tenía conocimientos acerca de la prueba del talón. Sin embargo, únicamente 25,7% había asistido a sesiones de capacitación sobre el procedimiento. Además, 64,3% del personal de salud opinaba que la prueba debía ser obligatoria, 27,1% afirmaba que no debía ser obligatoria y 8,6% manifestó que no lo sabía. En los grupos de debate, el personal de salud manifestó su creencia de que las madres aceptarían este método de tamizaje. Por su parte, 22,0% de las madres afirmaron conocer la prueba del talón y 63,6% manifestó conocer los motivos por los que se sometería al bebé a dicha prueba. Asimismo, 83,3% de las madres señaló que la prueba sería beneficiosa para el bebé. Además, 88,6% de las madres indicó que era necesaria más información sobre la prueba del talón. En sus grupos de debate, las madres expresaron que, por lo general, no les preocupaba el dolor que pudiese suponer para el bebé la prueba del pinchazo en el talón. Conclusiones El personal de salud tiene un nivel alto de conocimiento acerca de la prueba del talón. Las madres confían en el personal de salud, y aceptarían este procedimiento independientemente de sus conocimientos sobre la prueba y cualquier malestar asociado a este método de tamizaje.

RESUMO Objetivos No país caribenho de Santa Lúcia, o exame de sangue de cordão umbilical para detecção da doença falciforme (DF) era o método de triagem preferido pelos profissionais da saúde atuantes em maternidades, até a introdução do “teste do pezinho” (TP) no país em 2014. Este estudo sobre DF buscou avaliar o conhecimento e atitudes de profissionais da saúde em relação à triagem pelo TP e também determinar a favorabilidade das novas mães em direção à triagem de TP. Métodos Setenta profissionais da saúde e 132 novas mães responderam a questionários de pesquisa em três hospitais. Além disso, foram realizadas quatro discussões com grupos focais: dois de profissionais da saúde e dois de mães. Resultados Entre os profissionais da saúde entrevistados, 85,7% tinham conhecimento acerca do TP. Porém, somente 25,7% haviam comparecido às sessões de capacitação sobre o procedimento. Entre os profissionais da saúde, 64,3% opinaram que o TP deveria ser obrigatório, 27,1% disseram que não deveria ser obrigatório e 8,6% não sabiam se deveria ser obrigatório. Em seus grupos focais, os profissionais da saúde disseram acreditar que as mães aceitariam o método do TP. Por sua vez, 22,0% das mães disseram ter ouvido falar sobre o TP, e 63,6% relataram saber o motivo pelo qual o exame seria realizado; ademais, 83,3% indicaram que o exame seria benéfico para o bebê. Além disso, 88,6% das mães disseram que precisavam de maiores informações sobre o TP. Em seus grupos focais, as mães disseram que, em geral, não estavam preocupadas com dor que o teste do pezinho poderia causar ao bebê. Conclusões Os profissionais da saúde apresentaram alto nível de conhecimento acerca do método de triagem pelo TP. As mães confiam nos profissionais da saúde, e aceitariam o procedimento de TP independentemente de seu conhecimento acerca do teste e de qualquer desconforto associado a este método de triagem.

Hydroxyurea and a cGMP-amplifying agent have immediate benefits on acute vaso-occlusive events in sickle cell disease mice.

Inhibition of leukocyte adhesion to the vascular endothelium represents a novel and important approach for decreasing sickle cell disease (SCD) vaso-occlusion. Using a humanized SCD-mouse-model of tumor necrosis factor-α-induced acute vaso-occlusion, we herein present data demonstrating that short-term administration of either hydroxyurea or the phosphodiesterase 9 (PDE9) inhibitor, BAY73-6691, significantly altered leukocyte recruitment to the microvasculature. Notably, the administration of both agents led to marked improvements in leukocyte rolling and adhesion and decreased heterotypic red blood cell-leukocyte interactions, coupled with prolonged animal survival. Mechanistically, these rheologic benefits were associated with decreased endothelial adhesion molecule expression, as well as diminished leukocyte Mac-1-integrin activation and cyclic guanosine monophosphate (cGMP)-signaling, leading to reduced leukocyte recruitment. Our findings indicate that hydroxyurea has immediate beneficial effects on the microvasculature in acute sickle-cell crises that are independent of the drug's fetal hemoglobin-elevating properties and probably involve the formation of intravascular nitric oxide. In addition, inhibition of PDE9, an enzyme highly expressed in hematopoietic cells, amplified the cGMP-elevating effects of hydroxyurea and may represent a promising and more tissue-specific adjuvant therapy for this disease.

Possible links between sickle cell crisis and pentavalent antimony.

For over 60 years, pentavalent antimony (Sb(v)) has been the first-line treatment of leishmaniasis. Sickle cell anemia is a disease caused by a defect in red blood cells, which among other things can cause vasooclusive crisis. We report the case of a 6-year-old child with leishmaniasis who during treatment with meglumine antimoniate developed a sickle cell crisis (SCC). No previous reports describing the relationship between antimonial drugs and sickle cell disease were found. Reviews of both the pathophysiology of SCC and the mechanism of action of Sb(v) revealed that a common pathway (glutathione) may have resulted in the SCC. ChemoText, a novel database created to predict chemical-protein-disease interactions, was used to perform a more expansive and systematic review that was able to support the association between glutathione, Sb(v), and SCC. Although suggestive evidence to support the hypothesis, additional research at the bench would be needed to prove Sb(v) caused the SCC.