Psychosocial factors show a significant relationship between child behavior problems, family functioning, and cognitive performance in children with Sickle Cell Disease, marking those as important targets for intervention among this population. The purpose of this research is to address the effects of psychosocial factors impact on specific cognitive domains.

Archival data from the National Institutes of Health’s Cooperative Study of Sickle Cell Disease was used. Data was restricted to individuals aged 14 or younger (N= 2,408), with 47.8% (n = 1,152) identified as female and 52.2% (n = 1,256) as male. Black or African American (96.9%, n = 2,334) children made up the majority of the sample, with the remainder coded as “other” (2.8%, n = 68). The measures utilized included the Wechsler Intelligence Scale for Children-Revised (WISC-R), Wechsler Intelligence Scale for Children-Third Edition (WISC-III), Peabody Picture Vocabulary Test (PPVT), Achenbach Child Behavior Checklist, and Family Environment Scale (FES).

Bivariate correlations were completed with significant correlations found between FES and performance on the WISC-R/III and PPVT. Supportiveness subscale on the FES demonstrated several statistically significant correlations with WAIS FSIQ (r = .21, p = .000) as well as the Information (r = .22, p = .000), Similarities (r = .17, p = .001), Arithmetic (r = .13, p = .021), Block Design (r = .11, p = .036), Vocabulary (r = .22, p = .000), Object Assembly (r = .12, p = .033), Comprehension (r = .19, p = .000), and Digit Span (r = .13, p = .014) subscales. A statistically significant correlation was observed between the PPVT and the Supportiveness subscale (r = .34, p = .000).

Various areas of cognitive functioning are affected by family dynamics. Improvement in family functioning would benefit the cognitive functioning of children with SCD. To increase aspects of family functioning including supportiveness, early identification of children with SCD, targeted interventions, and family and/or individual therapy for caregivers are suggested.

Youth with sickle cell disease (SCD) are at increased risk of neurocognitive difficulties with and without neurological complications. Research has identified disease-related, socioeconomic, and sociodemographic risk factors as independently having significant associations with brain physiology for youth with SCD. Notably, sleep has a profound effect on youth’s neurocognitive abilities including learning, executive function, memory, attention, and processing speed. Furthermore, youth with SCD are at an increased risk for poor sleep measured by self-report questionnaires and by polysomnography (PSG). Within the SCD literature, only a few studies have examined the relationship between sleep and cognition. Of these, the majority examined individuals with SCD and comorbid sleep disorder diagnoses. The aim of this study is to identify associations between subjective sleep measures and neurocognitive outcomes in youth with SCD.

This study investigated a cohort of 24 youth with SCD (ages 9-16, 11 males, 13 females; HbSS [63%], HbSB+ [8%], HbSC [21%], HbSB0 [8%]) who received sleep questionnaires and a neuropsychological evaluation. Exclusion criteria included a history of neurological disorder (e.g., overt stroke, seizures, or moyamoya disease) or prescribed psychotropic medication. Sleep questionnaires measuring sleep disturbance (e.g., sleep onset, sleep continuity, and sleep quality) and sleep-related impairments (e.g., daytime sleepiness, sleepiness interference with concentration, and difficulty with activities of daily living skills) were collected. Executive function, working memory, processing speed, and verbal comprehension measures were assessed. Demographics and disease-related risk factors were analyzed individually from medical records.

All analyses were controlled for age, the time between neuropsychological testing and sleep questionnaires, SCD genotype, and sex. Partial correlations were conducted to explore associations with neurocognitive outcomes. Verbal comprehension was significantly correlated with sleep disturbance (r= -.673, p=.001). Multiple linear regressions revealed that sleep disturbance significantly predicts verbal comprehension (ß= -.572, p=.003). Specifically, verbal comprehension decreased by 4.4 standard points for every one-point increase in sleep disturbance. Additionally, total sleep problems significantly predicted working memory (ß=-.414, p=.044) and processing speed (ß= -.411, p= .046). Specifically, working memory decreased by 3.5 standard points while processing speed decreased by 3.3 standard points for every one-point increase in total sleep problems reported. Sleep parameters did not significantly predict executive function.

Results support the association between poor sleep and neurocognitive difficulty in youth with SCD. Three of the participants in this study received a PSG, which further demonstrates the importance of the current findings. This study serves to identify potential risk factors for neurocognitive deficits and provides potential methods for identifying youth with SCD who may need to be referred for a PSG assessment. Research should replicate these findings with increased sample sizes including utilizing PSG and investigating neurobiological effects. Findings may inform future screening tools, treatment approaches, and advanced cognitive initiatives and resources for this population.

Individuals with sickle cell disease (SCD) are at increased risk for developing impairment across cognitive domains, although the most common deficits are thought to be related to processing speed and executive functions. One of the most common ways of evaluating cognitive functioning is through the administration of intellectual tests. While lower overall intellectual functioning in individuals with SCD compared to healthy controls has been found, the specific pattern of strengths and weaknesses across indices is not well known. Anecdotally, it has been observed at our clinic that individuals with SCD are more likely to show relative or significant weaknesses in visuospatial abilities, but this has not been formally investigated. Further, based on the extant research, individuals with SCD would likely demonstrate lower working memory and processing speed indices, but, as far as we are aware, this has not been investigated either. The purpose of the present study is to examine the intellectual profiles, including areas of relative and significant strengths and weaknesses, of children and adolescents with SCD.

Participants are children and adolescents (age 6-16) with SCD who were referred for a neuropsychological evaluation at Montefiore Medical Center’s Neuropsychological Assessment Service from 2015 to 2022. These participants (N=54) were identified through a thorough review of patients seen through this service and were administered the Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V; Wechsler, 2014). Mean scores were calculated for WISC-V indices. In addition, differences were calculated between WISC-V indices (e.g., VCI-VSI, etc.), and a discrepancy analysis was conducted comparing the base rates of these differences in the present sample to the WISC-V standardization sample.

In our sample, the mean total FSIQ of our sample was 85 (SD=14.5). The following mean scores were obtained across indices: VCI, SS=90 (SD=14.5); VSI, SS=86.5 (SD=14.9); FRI, SS=90 (15.5); WMI, SS=89 (SD=15.6); and PSI, SS=82 (SD=17.4). Many of the index score discrepancy base rates were similar to the standardization sample. However, our sample had greater discrepancies between several indices compared to the standardization sample. In particular, the following base rate discrepancies between index scores emerged as being different in our sample compared to the standardization sample: VCI>VSI and VCI>PSI. Notably, a 30+ point difference VCI>VSI was found in 6% of our sample (compared to 1.6% of the standardization sample) and a 30+ point difference between VCI>PSI was found in 12% of our sample (compared to 4.6% of the standardization sample). In addition, a 10+ point difference found between VCI>PSI was found in 50% of our sample (compared to 29% of standardization sample).

In our sample, FSIQ and index scores fell approximately 0.5-1.33 SD below the standardization sample means, with the lowest index scores being PSI and VSI. Consistent with the literature, the PSI (but not WMI) emerged as an area most discrepant to other indices (particularly VCI). In line with our observations, the VSI emerged as an area of relative difficulty as compared to the VCI. These results suggest that, in addition to processing speed, visuospatial/constructional ability is an area that warrants consideration in the assessment of individuals with SCD.

Patients with sickle cell disease (SCD) with vaso-occlusive crises (VOC) often visit the emergency department (ED) for management of painful episodes. The primary objective of this pilot study was to evaluate the acceptability of a short-stay model for treatment of VOC in SCD outside of the ED in Toronto, Canada. Secondary objectives were to assess patient satisfaction of this model, barriers to its use and comparison of clinical outcomes to a historical control.

Adult SCD patients with symptoms of an uncomplicated VOC between October 2014 to July 2016 were managed according to best practice recommendations in a short-stay unit as an alternative to the local emergency room. Primary outcome of time to first analgesia, and secondary outcome of discharge rate were compared to a historical control at a local ED from 2009-2012. Satisfaction and barriers to use of the ambulatory care delivery model were assessed by patient survey.

Twenty-one visits were recorded at the short-stay unit during the study period. Average time to first opiate dose was 23.5 minutes in the short-stay unit compared to 100.3 minutes in the ED (p<0.001). Discharge rate from the short-stay unit was 84.2%. Average patient satisfaction with this model of care was high (>4/5 on Likert scale) except for geographic accessibility (85% response rate, n=18).

This study demonstrated high patient satisfaction and acceptability of a short-stay model for treatment of uncomplicated VOC in adult SCD patients in Toronto, the first of its kind in Canada.

An unusual clivus mass is reported, following investigation of memory disturbance and headaches in a patient with β thalassaemia and sickle cell disease. This lesion proved to be a site of extramedullary haematopoiesis.

Computed tomography demonstrated a 2 cm mass at the base of the sphenoid. However, magnetic resonance imaging showed little bony expansion, and the differential diagnosis included chordoma, dermoid cyst and fibrodysplasia.

Examination of the biopsy showed the presence of bone marrow demonstrating erythroid hyperplasia and small aggregates of B-cell lymphocytes. The features were considered compatible with erythroid hyperplasia associated with haemolytic anaemia.

The patient was reassured that she did not have a neoplastic lesion, and was referred back to the haematologists for further management of her sickling β thalassaemia.

Extramedullary haematopoiesis occurs outside the reticuloendothelial system in response to haemolytic anaemia. Extramedullary haematopoiesis causing a clivus mass is an unusual ENT presentation. Such haematopoiesis is occasionally seen in the calvarial skull, but this is the first report of this process occurring in the anterior skull base, to our knowledge.

Priapism is a recognized complication of sickle cell anemia (SCA). When initial conventional treatments fail, simple or exchange blood transfusion has been advocated as a secondary intervention. However, recent literature suggests this may not be an effective therapy and may have significant neurologic sequelae. This paper reviews and summarizes the effectiveness and risks of blood transfusion compared with conventional priapism therapy.

All relevant papers identified from a MEDLINE search were systematically examined for data related to the use of blood transfusion in the setting of priapism due to SCA. The effectiveness of conventional therapy was compared with transfusion therapy using the outcome of “time to detumescence” (TTD). In addition, papers documenting adverse neurologic sequela were reviewed and summarized.

Forty-two case reports were identified containing complete information with regard to patient age and TTD. The mean TTD was 8.0 days with conventional therapy (n = 16) and 10.8 days with blood transfusion therapy (n = 26). Adverse neurologic sequelae from blood transfusion therapy was described in 9 cases, with long term outcomes ranging from complete resolution to severe residual deficits.

The current literature does not support the contention that blood transfusion is an effective therapy in the treatment of priapism due to SCA, as defined by an acceleration of TTD. In fact, numerous reports suggest that serious neurologic sequelae may result from this treatment. We feel the routine use of this therapy cannot be recommended.