In a cohort of children with sickle cell disease (SCD) and vaso-occlusive pain visits served through South Carolina's Medicaid system over a 6-year period (N 5 523), we compared the number of vaso-occlusive pain or acute chest syndrome (ACS)/pneumonia episodes, and outpatient or acute service costs in those treated or not treated with hydroxyurea (HU). HU may be an underused intervention for SCD in this practice setting, for a variety of reasons. Treatment with HU varied greatly, appears to have been administered to more severely ill children, but was associated with a reduction in vaso-occlusive pain episodes, hospitalizations, and total costs of care within the HU cohort during a 2-3 year period of active HU treatment. Those receiving care through specialized SCD clinics were less likely to have pain or acute care episodes (RR 5 0.79, P < 0.0001; RR 5 0.90, P 5 0.01). Compared with the non-HU cohort, the HU group evinced a significantly higher risk of experiencing vaso-occlusive pain episodes (RR 5 3.32, P < 0.0001) and ACS/pneumonia episodes (RR 5 2.66, P < 0.0001), and higher outpatient, inpatient/emergency, and total service costs (RR 5 1.85, 2.11, 2.10, and P < 0.0001, respectively) over time. HU is clinically effective in reducing pain episodes, hospitalizations, and total care costs, but those receiving it might be more severely ill.Erythrocytes in children with sickle cell disease (SCD) become deoxygenated, dehydrated, and crescent-shaped, and tend to aggregate or stick to blood vessel walls, blocking blood flow within limbs and organs, causing painful episodes. These patients are frequently seen in emergency departments and hospitalized for these severe pain episodes [1]. SCD poses an enormous personal burden to these young patients and is also costly to the family and third-party payer, especially for low-income children [2]. Interventions designed to control vaso-occlusive pain episodes, and avoid hospitalizations may reduce the significant personal and economic burdens of the disease [3,4].Hydroxyurea (HU), a myelosuppressive agent, which raises the levels of Hb F [5] and of hemoglobin [6][7][8], effectively decreases the rate of painful vaso-occlusive and acute chest syndrome (ACS) episodes by 50% in adults [9][10][11][12], and is generally safe and well-tolerated in children older than 5 years of age [13]. There is strong evidence that HU reduces the frequency of hospitalization in children with SCD and moderate evidence that it decreases the frequency of painful crises [14]. Given the short-term safety profile of HU in children and its established efficacy in adults, HU is commonly used, off-label, in children with multiple painful episodes (3 per year) to reduce episode frequency and acute services utilization [15][16][17], even in children as young as 9 months.Results from a multicenter study of HU in sickle-cell anemia demonstrated that adult patients treated with HU had a 44% decrease in hospitalizations compared with those taking placebo, which accounted for the majority of cost savings in ...
We have examined the effect of 2, 3-diphosphoglycerate (DPG) on the solubility of deoxy-sickle hemoglobin (deoxy-Hb S) under conditions such that concentration, pH, and osmolarity of deoxy-Hb S solutions approached physiological. The range of DPG/Hb molar ratios encompassed the extremes found for this ratio in erythrocytes from individuals with sickle cell anemia. After monomer-polyer equilibrium had been established, the phases were separated by centrifugation and assayed for concentrations of Hb and DPG. DPG had no effect on the solubility of deoxy-Hb S. Furthermore, at DPG/Hb molar ratios less than one, there was no preferential incorporation of deoxy-Hb S containing bound DPG into polymers. At DPG/Hb molar ratios greater than one, concentrations of free DPG in monomer and polymer phases were virtually identical. Thus, under the specified equilibrium conditions, DPG is not a determining factor in the polymerization of deoxy-Hb S.
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