Kristy F. Woods

Predicting the Effectiveness of Hydroxyurea in Individual Sickle Cell Anemia Patients

The study described in this paper was undertaken to develop the ability to predict the response of sickle-cell patients to hydroxyurea (HU) therapy. We analyzed the effect of HU on the values of 23 parameters of 83 patients. A Students t-test was used to confirm (Rodgers GP, Dover GJ, Noguchi CT, Schechter AN, Nienhuis AW. Hematologic responses of patients with sickle cell disease to treatment with hydroxyurea, N Engl J Med 1990;322;1037-44) at the 0. 001 level that treatment with HU increases the proportion of fetal hemoglobin (HbF), and the average corpuscular volume (MCV) of the red blood cells. Correlation analysis failed to establish a statistically significant relationship between any of the 23 parameters and the HbF response. Linear regression analysis also failed to predict a patients response to HU. On the other hand, artificial neural network (ANN) pattern-recognition analysis of the 23 parameters predicts, with 86.6% accuracy, those patients that respond positively to HU and those that do not. Furthermore, we have found that the values of only 10 of the 23 parameters (listed in the body of this paper) are sufficient to train ANNs to predict which patients will respond to HU.

Sickle cell telemedicine and standard clinical encounters: A comparison of patient satisfaction

OBJECTIVES To determine patient satisfaction with telemedicine encounters among adults with sickle cell disease and compare their scores with SCD patients who have standard medical encounters (as controls). METHODS Adults patients were recruited from a list of participants in sickle cell telemedicine clinics and prospectively at the time of clinic encounter. Patients were assigned to telemedicine or standard encounter groups. Demographic and pertinent clinical data were obtained for all subjects, and the Client Satisfaction Questionnaire (CSQ-8) was administered. Patients were also asked for open-ended comments regarding their satisfaction with the service. Their responses were recorded verbatim. RESULTS Patients with telemedicine (n = 60) and standard encounters (n = 60) were comparable in gender, genotype, education, employment, and mean number of sickle cell disease-related complications. Patients in the telemedicine group were younger (p< 0.005), more likely to have Medicaid insurance (p = 0.009), and more likely be taking hydroxyurea (p = 0.003) than patients in the control encounter group. Mean CSQ scores for the telemedicine group were high (total: 28.82+/-3.06), and there was no difference for any item between encounter groups (p = 0.389). Patients in the standard encounter group were more likely to provide positive open-ended comments regarding the encounter (95% vs. 70%; p = 0.001). Negative comments were generally in the area of confidentiality. CONCLUSIONS While some patients expressed concern about confidentiality with telemedicine, the benefits of improved access and continuity of care were recognized, and overall satisfaction with telemedicine was high. These findings support the use of telemedicine as an acceptable health care delivery option for rural, underserved populations with sickle cell disease.

Sickle cell disease telemedicine network for rural outreach

The Medical College of Georgia (MCG) Sickle Cell Center in Augusta, Georgia, USA, provides consultation and (in some cases) primary-care services to more than 1000 patients with sickle cell disease (SCD). Three SCD telemedicine clinic sites were established in rural areas of middle and southern Georgia, based on clinical need; a fourth site had been proposed. Over a 36-month study period, 77 telemedicine SCD clinics were held. There was a mean of 6.1 (SD 2.5) encounters per clinic and 466 total encounters among 128 SCD patients. By using telemedicine, the productivity of the MCG adult sickle cell clinic increased from 1413 to 1889 encounters a year, with an increase in rural outreach activity from 271 to 745 encounters a year. This was accomplished with the addition of a single physician assistant during the last 12 months of the study period; otherwise provider staffing was unchanged. A formal cost-benefit analysis now needs to be carried out.

Exercise in Sickle Cell Anemia: Effect on Inflammatory and Vasoactive Mediators

The aim of this study was to determine the response of inflammatory and vasoactive mediators to 3 consecutive days of exercise in African-American women with and without sickle cell anemia (SCA). Circulating inflammatory mediators [C-reactive protein (CRP), interleukin-6 (IL-6), tumor necrosis factor alpha (TNFalpha)] were measured before, and vasoactive mediators [endothelin-1 (ET-1), nitric oxide metabolites (NOx)] before and after each exercise bout in ten subjects with SCA and ten controls. Exercise did not affect ET-1, IL-6 or CRP concentrations (p >.05). TNFalpha was higher in SCA than controls (p < or = .0005) at all times; however, the response pattern was similar for the groups: no change from day 1 to day 2, but a decrease from day 2 to day 3 (p < or = .05). NOx increased significantly after exercise (p < or = .0001) but returned to baseline by 24 h afterward. On the 3rd day, NOx increased after exercise in SCA but not in the controls (p < or = .05). In conclusion, exercise did not cause a harmful inflammatory response in these individuals with SCA. However, NOx increased after exercise on all 3 days in SCA but appeared attenuated after 2 days in controls.

Hepatic findings of patients with sickle cell disease include a unique pattern of fibrosis in the perisinusoidal and pericentral vein areas

Hepatic findings of patients with sickle cell disease include a unique pattern of fibrosis in the perisinusoidal and pericentral vein areas