Background Neurocognitive deficits have been described in school age children with sickle cell disease (SCD), even in the absence of stroke or silent infarcts. However, the age of onset and factors contributing to this problem have not been well studied. We hypothesized that in children with SCD the failure rate with Brigance screening would be higher than in the normal population. Methods We reviewed retrospectively the Brigance Preschool Screen-II test results in three year-old children with SCD. Findings were correlated with hemoglobinopathy genotype, hemoglobin level, transcranial Doppler ultrasound (TCD) velocities, and treatment with hydroxyurea, as well as with psychosocial factors. Results Eighty-eight children with SCD followed by the St. Jude Sickle Cell Center (mean age 3.5 years) had neurocognitive screening during their regular clinic visits. Forty-four (50%) children had scores below the normal cut-off value for their age (twice the national failure rate of 25%). Failures were associated with less parental education (p=0.005 for maternal and p=0.03 for paternal education levels) and with speech deficits (p=0.01), but were not associated with sickle cell genotype or hemoglobin concentration. Conclusion These preliminary data suggest that psychosocial factors may have more profound effects on early childhood development than disease-related factors in this group of young sickle cell patients. A larger prospective study with appropriate controls is warranted to validate these findings, which have implications for the etiology and prevention of neurocognitive decline in children with SCD.
Summary We previously found that neurodevelopmental deficits commonly occurred in three‐year‐olds with sickle cell disease (SCD), but clinical significance was uncertain because a comparison group was lacking. Our objective in the current study was to prospectively compare neurodevelopment in three‐year‐old children with SCD to an age‐appropriate control group. The Brigance Preschool Screen II is a neurodevelopmental screening examination which can be administered in 15–20 min. SCD patients (Group 1) were compared with community controls of similar age and ethnicity enrolled in daycare/preschool (Group 2). SCD patients who were receiving hydroxycarbamide treatment were also compared (Group 3). Two hundred forty‐five three‐year‐olds were evaluated: Group 1, 111; Group 2, 114; and Group 3, 20. The below cut‐off rate on the Brigance test was higher in Group 1 (73%) than in Group 2 (61%; P = 0·04). In multivariate analysis of Group 1 patients, only lower household income and more persons living in the home were independent predictors of this. Patients with SCD and matched controls had high rates of ‘failing’ the Brigance test. The below cut‐off rate in untreated children with SCD was associated with low household income and increased number of persons living in the home.
To report on the use of outpatient anesthesia (OPA) facilitating delivery of stereotactic body radiation therapy (SBRT) in patients with severe cognitive impairments (CI) diagnosed with inoperable early stage lung cancer. Methods and Materials: We surveyed our institutional review boardeapproved prospective lung SBRT data registry to document the feasibility of using anesthesia in CI patients and to determine their SBRT outcomes. Results: From 2004 to 2018, 8 from a total 2084 patients were identified for this analysis. The median age at treatment was 68 years (range, 44-78). Most patients were female (62.5%). CI diagnoses included Alzheimer-related dementia (3 patients), chronic schizophrenia (3 patients), severe anxiety disorder (1 patient), and severe developmental disability (1 patient). The median tumor size was 3.4 cm (range, 1.1-10.5), and 7 patients (87.5 %) had central lesions. The median follow-up time was 22.5 months. The most common (50%) SBRT schedule used was 50 Gy in 5 fractions. Intravenous propofol (10 mg/mL) was used for OPA in all cases at the time of simulation and with daily treatments. OPA was well tolerated and all patients completed SBRT as prescribed. There was one grade 5 but no other grade 3 or higher SBRT-related toxicities. One patient died with local failure and one of distant failure. Conclusions: OPA made lung SBRT feasible for patients with CIs. SBRT outcomes were in keeping with those reported in the literature. CI should not be considered a contraindication per se to SBRT delivery in patients otherwise appropriate for this modality.
Children with sickle cell disease (SCD) frequently have diminished academic attainment and are particularly vulnerable to reading dysfunction. We explored the effectiveness of a multisensory reading intervention offered during the summer to children with SCD at our institution. Subjects with reading deficits were identified through parent report, clinical findings, or school meetings. Summer reading programs utilizing Phonemic Awareness and Symbol Imagery were provided. The Lindamood–Bell Auditory Conceptualization/Phonemic Awareness Test, Third Edition (LAC‐3), and the Symbol Imagery Test were used as pre‐ and postintervention examinations to measure progress. Fifteen students (median age 9.4 years, range 6–14 years, eight females, all African American) received the Phonemic Awareness intervention, two times a week for 6 weeks. The subjects showed statistically significant gains in standard scores derived from the LAC‐3 (mean change 7.9 points, p < .001), with associated improvements in age equivalency (AE) and grade equivalency (GE). Twenty‐nine students (median age 9 years, range 6–17 years, 13 females, all African American) participated in the Symbol Imagery reading program, also two times a week for 6 weeks. These students showed significant gains in overall standard scores (mean change 9.8 points, p < .001). Although results should be interpreted with caution due to small sample sizes, we found that summer reading clinics for children with SCD improved phonological processing and symbol imagery skills, potentially leading to substantial gains in reading capability.
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