Browsing by Subject "Sickle cell anemia"

Hydroxyurea, a mainstay of sickle cell management in the developed world, offers a range of potential benefits to children with sickle cell disease. There is strong evidence that hydroxyurea induces production of fetal hemoglobin (HbF) in red blood cells, which is generally associated with reduced morbidity and fewer incidents of clinical events in sickle cell patients. Based on literature from in vitro investigations, it has also been suggested that hydroxyurea may confer some protection against malaria parasitemia by inhibiting proliferation of the malaria-causing parasite - Plasmodium falciparum.

The purpose of this study was to examine the effects hydroxyurea use on P. falciparum infection, parasite density, HbF and morbidity among children with sickle cell disease living in a malaria endemic setting. We analyzed baseline data of 95 children (aged 1 – 10 years) enrolled in the EPiTOMISE clinical trial (Enhancing Preventive Therapy of Malaria in children with Sickle cell anemia in East Africa) between January 2018 and September 2018.

Our analyses showed no significant difference in the prevalence of P. falciparum infection between hydroxyurea users and non-hydroxyurea users, prevalence ratio [95% CI] = 1.14 [0.76, 1.71]. Among infected children, median (IQR) log parasites densities were also similar between hydroxyurea users, -0.96 (-1.67, 0.41) and hydroxyurea non-users, -0.12 (-1.32, 3.48), p-value=0.146.

We did observe substantial hematological benefits among hydroxyurea users including an approximate 1 unit increase in median hemoglobin concentration and a 2.7-fold increase in median percentage HbF. However, this observation did not translate to any meaningful difference in the prevalence of morbidity events.

In agreement with the few studies on hydroxyurea use in malaria endemic settings, these results suggest that there may be no added risk of P. falciparum infection to sickle cell patients who routinely use hydroxyurea. Furthermore, our results reflect that hydroxyurea use is associated with increased HbF and a better hematological profile in this population. There is need for more research on hydroxyurea use in sub-Saharan Africa to help resolve any existing concerns and conflicting data in the current body of literature.

Background: Sickle cell disease (SCD) is a debilitating genetic blood disorder that seriously impacts the quality of life of affected individuals and their families. With 85% of cases occurring in sub-Saharan Africa, it is essential to identify the barriers and facilitators of optimal outcomes for people with SCD in this setting. This study focuses on understanding the relationship between support systems and disease outcomes for SCD patients and their families in Cameroon and South Africa.

Methods: This mixed-methods study utilizes surveys and semi-structured interviews to assess the experiences of 29 SCD patients and 28 caregivers of people with SCD across three cities in two African countries: Cape Town, South Africa; Yaoundé, Cameroon; and Limbe, Cameroon.

Results: Patients in Cameroon had less treatment options, a higher frequency of pain crises, and a higher incidence of malaria than patients in South Africa. Social support networks in Cameroon consisted of both family and friends and provided emotional, financial, and physical assistance during pain crises and hospital admissions. In South Africa, patients relied on a strong medical support system and social support primarily from close family members; they were also diagnosed later in life than those in Cameroon.

Conclusions: The strength of medical support systems influences the reliance of SCD patients and their caregivers on social support systems. In Cameroon the health care system does not adequately address all factors of SCD treatment and social networks of family and friends are used to complement the care received. In South Africa, strong medical and social support systems positively affect SCD disease burden for patients and their caregivers. SCD awareness campaigns are necessary to reduce the incidence of SCD and create stronger social support networks through increased community understanding and decreased stigma.