Marilyn H. Gaston

Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial

Children with sickle cell anemia have an increased susceptibility to bacterial infections, especially to those caused by Streptococcus pneumoniae. We therefore conducted a multicenter, randomized, double-blind, placebo-controlled clinical trial to test whether the regular, daily administration of oral penicillin would reduce the incidence of documented septicemia due to S.pneumoniae in children with sickle cell anemia who were under the age of three years at the time of entry. The children were randomly assigned to receive either 125 mg of penicillin V potassium (105 children) or placebo (110 children) twice daily. The trial was terminated 8 months early, after an average of 15 months of follow-up, when an 84 percent reduction in the incidence of infection was observed in the group treated with penicillin, as compared with the group given placebo (13 of 110 patients vs. 2 of 105; P = 0.0025), with no deaths from pneumococcal septicemia occurring in the penicillin group but three deaths from the infection occurring in the placebo group. On the basis of these results, we conclude that children should be screened in the neonatal period for sickle cell hemoglobinopathy and that those with sickle cell anemia should receive prophylactic therapy with oral penicillin by four months of age to decrease the morbidity and mortality associated with pneumococcal septicemia.

Recruitment in the Cooperative Study of Sickle Cell Disease (CSSCD).

The Cooperative Study of Sickle Cell Disease (CSSCD) is a multiinstitutional investigation of the natural history of clinical course of sickle cell disease from birth through adulthood. The study is not a trial; rather, it involves data collection at 23 institutions in a uniform, standardized fashion on 3800 patients. Recruitment aspects that were addressed include issues related to recruitment of different age groups, ranging from newborns to pregnant women to patients over 50 years of age; the need to include mildly affected patients to ensure that the study would not reflect only a severe hospital-based population; recruitment from rural populations; and the need to screen and enter a newborn population at birth. The recruitment goal of entering 3200 patients, including 2100 patients with SS hemoglobinopathy, over a 24-month period was accomplished after 27 months.

Sickle Cell Disease

Sickle cell disease (SCD) is a group of inherited disorders caused by mutations in HBB, which encodes haemoglobin subunit β. The incidence is estimated to be between 300,000 and 400,000 neonates globally each year, the majority in sub-Saharan Africa. Haemoglobin molecules that include mutant sickle β-globin subunits can polymerize; erythrocytes that contain mostly haemoglobin polymers assume a sickled form and are prone to haemolysis. Other pathophysiological mechanisms that contribute to the SCD phenotype are vaso-occlusion and activation of the immune system. SCD is characterized by a remarkable phenotypic complexity. Common acute complications are acute pain events, acute chest syndrome and stroke; chronic complications (including chronic kidney disease) can damage all organs. Hydroxycarbamide, blood transfusions and haematopoietic stem cell transplantation can reduce the severity of the disease. Early diagnosis is crucial to improve survival, and universal newborn screening programmes have been implemented in some countries but are challenging in low-income, high-burden settings.

Paradoxes in Obesity With Mid-Life African American Women

PURPOSE To examine the relationship among socioeconomic status, psychological factors (ie, health locus of control, health satisfaction), and health behaviors (ie, physical exercise, eating patterns) in a volunteer sample of mid-life African American women residing within Washington, DC. METHODS The studys sample included 351 African American women who participated in the 12-week Prime Time Sister Circles (PTSC) intervention and completed the initial self-report survey. Of the women completing the baseline survey, clinical assessments were taken on 277 participants. RESULTS The majority (64.4%) of the women were either obese or extremely obese based upon their body mass index (BMI). Fewer than one-third of the women were satisfied with their weight. Despite these outcomes, most (61.8%) of the sample rated their health, in comparison to other African American women, as either good or very good. Further, more than one-half of the women were somewhat to very satisfied with their health knowledge, attitudes, behaviors, and their physical, emotional, and spiritual health. There was no significant relationship between obesity level and socioeconomic status, as measured by education, personal income, and total household income. Additionally, there were no significant differences in weight or eating behaviors by education, personal income, and total household income. The findings revealed a significant difference in the number of days the women reported engaging in physical exercise by personal income (p < .01) and total household income (p = .05), surprisingly, with the women in the lower-income categories reporting engaging in more days of physical exercise than women in the higher-income categories. CONCLUSIONS This study documents the paradox of obesity in mid-life African American women related to socioeconomic status inasmuch as there were no differences observed in the prevalence of obesity by socioeconomic status indicators. The obese women of lower socioeconomic status reported exercising more than obese women of high socioeconomic status. Paradoxically, most of the obese women were dissatisfied with their body weight, shape, and physical health, but more than 50% were satisfied with their health attitudes and behaviors and 61.8% rated their health favorably. The paradoxes of obesity and African American women across various income levels are important to understand for interventions to success.