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Objective Patients with sickle cell disease (SCD) often seek care in the emergency department (ED) for pain associated with vaso-occlusive crises (VOC). Research has shown that negative provider attitudes serve as a barrier to care in this patient population. Our aim was to validate a survey that measures attitudes towards patients with SCD among ED providers (nurses and physicians) and to compare differences in attitude scores between provider types. Methods We administered the General Perceptions about Sickle Cell Disease Patients Scale, previously validated amongst internal medicine providers, and the Medical Condition Regard Scale (MCRS) to ED nurses and physicians from two emergency departments. Results 215 surveys were returned (63% response rate, 200 with attitude items completed). Three subscales were identified: Negative attitudes, Uneasiness with care, and Positive attitudes. Cronbach alphas exceeded 0.81 for each subscale. Mean (SD) scores for the Negative, Uneasiness, and Positive subscales and MCRS were 61.5 (20.3), 66.1 (17.1), 41.2 (17.8), and 42.2 (8.9) respectively. Compared to physicians, nurses had significantly higher mean Negative attitude scores and lower Uneasiness scores. Conclusions A slightly modified version of the General Perceptions about Sickle Cell Disease Patients Scale appears to be a valid measure of ED provider attitudes towards patients with SCD. Amongst ED providers, this scale identified a dimension not observed in research with the original instrument amongst internal medicine providers. Practice Implications Provider attitudes influence patient-provider interactions and quality of care. The scale we present here has major clinical implications, particularly for advanced practice nurses, who can use the scale not only to assess providers’ attitudes towards patients with SCD, but also determine the effectiveness of tailored interventions to improve those attitudes.

Background. Sickle cell disease (SCD) is a chronic blood disorder in which mortality has increased for adolescents and young adults (AYA). Procedure. A longitudinal analysis of medical records was conducted to describe the clinical course among AYAs (ages 12–27 years) during transition to adult care. Measures included sociodemographic, complications, SCD severity (modified pediatric SCD severity index), comorbidities, and transfer. Group-based trajectory modeling (GBTM) to identify subgroups with distinct severity trajectories and chi-square and unpaired student t-test to explore subgroup differences were used. Results. Overall, 339 AYAs (97% black, 56% male, 69% hemoglobin SS) had 10848 clinic, 3840 hospital, and 3152 emergency department visits. Complications included vaso-occlusive crises (80%) and acute chest syndrome (41%). Comorbidities included depression (19%) and anxiety (14%). Most AYAs transferred to adult care (n=220) at 19 years. Fourteen AYAs died, 10 within seven years from transfer. GBTM identified both stable and increasing severity trajectory groups: stable-low (n=31, 23%), stable-medium (n= 61, 46%), stable-high (n=6, 5%), low-increasing (n=13,10%), and medium-increasing (n= 22,17%). AYAs with increasing severity (25%) were older, lived closer to the clinic, and had higher risk for SCD complications and comorbidities. They had less pediatric clinic visits, however, they were more likely to transfer and remain longer in adult SCD care. Conclusions: Whereas most AYAs had stable severity, nearly a quarter had increasing severity, over time. AYAs with increasing severity had more complications, were more likely to transfer to adult care, and demonstrated higher and longer adult SCD care utilization compared to AYAs with stable severity.

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The majority of surveyed physicians consider infants at gestational age less than or equal to 25 weeks gestation or 800 g at birth as non-viable, and therefore would not attempt their resuscitation. Factors influencing threshold of viability in developing countries need to be addressed and explored further.

Purpose Sickle cell disease (SCD) is associated with high acute healthcare utilization. The purpose of this study was to examine whether Medicaid expansion in California increased Medicaid enrollment, increased hydroxyurea prescriptions filled, and decreased acute healthcare utilization in SCD. Methods Individuals with SCD (≤65 years and enrolled in Medicaid for ≥6 total calendar months any year between 2011 and 2016) were identified in a multisource database maintained by the California Sickle Cell Data Collection Program. We describe trends and changes in Medicaid enrollment, hydroxyurea prescriptions filled, and emergency department (ED) visits and hospital admissions before (2011‐2013) and after (2014‐2016) Medicaid expansion in California. Results The cohort included 3635 individuals. Enrollment was highest in 2014 and lowest in 2016 with a 2.8% annual decease postexpansion. Although <20% of the cohort had a hydroxyurea prescription filled, the percentage increased by 5.2% annually after 2014. The ED visit rate was highest in 2014 and decreased slightly in 2016, decreasing by 1.1% annually postexpansion. Hospital admission rates were similar during the pre‐ and postexpansion periods. Young adults and adults had higher ED and hospital admission rates than children and adolescents. Conclusions Medicaid expansion does not appear to have improved enrollment or acute healthcare utilization among individuals with SCD in California. Future studies should explore whether individuals with SCD transitioned to other insurance plans or became uninsured postexpansion, the underlying reasons for low hydroxyurea utilization, and the lack of effect on hospital admissions despite a modest effect on ED visits.

Individuals with sickle cell disease (SCD) experience neurocognitive decline, low medication adherence, increased unemployment, and difficulty with instrumental activities of daily living (IADL). The relationship between self-perceived cognitive difficulties and IADLs, including employment, school enrollment, independence, engagement in leisure activities, and medication adherence is unknown. We hypothesized that self-reported difficulties across neurocognitive areas would predict lower IADL skills. Adolescent and adult participants of the multi-site Sickle Cell Disease Implementation Consortium (SCDIC) (n = 2436) completed patient-reported outcome (PRO) measures of attention, executive functioning, processing speed, learning, and comprehension. Cognitive symptoms were analyzed as predictors in multivariable modeling. Outcome variables included 1) an IADL composite that consisted of employment, participation in school, reliance on others, and leisure pursuits, and 2) hydroxyurea adherence. Participants reported cognitive difficulty across areas of attention (55%), executive functioning (51%), processing speed (57%), and reading comprehension (65%). Executive dysfunction (p < 0.001) and sometimes or often experiencing learning difficulties (p < 0.001 and p = 0.04) and poor comprehension (p = 0.000 and p = 0.001), controlled for age (p < 0.001), pain (p < 0.001), and Jane S. Hankins and Jerlym Porter share co-authorship.

Objective To determine if there are distinct developmental trajectories of medical responsibility in youth with spina bifida (SB) across ages 8–17 years and to identify condition-related, parental, and family systems predictors of membership in these trajectory groups. Methods Participants were 140 youth with SB and their parents who participated in four waves of a longitudinal study across 6 years (ages 8–15 years at Time 1). Multi-method (questionnaires and observed family interactions) and multi-respondent assessments were conducted during home visits. Results Findings revealed that there were two distinct developmental trajectories that characterized this sample, with one being labeled “high increasing” (two thirds of the sample) and one labeled “low increasing” (one third of the sample). Most predictor variables were significantly associated with trajectory group membership, with the exception of ethnicity, SES, and measures of conflict. When all significant univariate predictors were included in the same model, only intelligence quotient (IQ), family stress, and gender were retained as significant. Conclusions Most youth exhibited relatively rapid increases in responsibility over the course of late childhood and adolescence, but there was a smaller portion of the sample that did not exhibit this type of developmental trajectory. The magnitude of the IQ effect on group differentiation appeared to attenuate the effects of most other predictors. It will be important for clinicians working with youth with SB to recognize that the transfer of medical responsibility from parent to child cannot be expected to unfold in the same manner for all families of youth with SB.