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Objectives: Sickle cell disease (SCD) is associated with high healthcare utilization rates and poor outcomes in a subset of patients, although the underlying factors that predict this phenotype are poorly understood. Prior studies suggest that comorbid avascular necrosis (AVN) contributes to high healthcare utilization. We sought to clarify whether AVN independently predicts acute care utilization in adults with SCD and to identify characteristics of those with AVN that predict higher utilization., Methods: We reviewed the medical records of 87 patients with SCD with symptomatic AVN and compared acute care utilization and clinical characteristics with 87 sex- and age-matched patients with SCD without symptomatic AVN. Patients with ≥2 years of follow-up were included. Outcomes were compared using bivariate analysis and multivariate regression., Results: Our study included 1381 follow-up years, with a median of 7 years per patient. The AVN cohort had greater median rates of urgent care visits (3.2/year vs 1.3/year; P = 0.0155), admissions (1.3/year vs 0.4/year; P = 0.0002), and admission days (5.1 days/year vs 1.8 days/year; P = 0.0007). History of high utilization (odds ratio [OR] 4.28; P = 0.001), acute chest syndrome (OR 3.12; P = 0.005), pneumonia (OR 3.20; P = 0.023), hydroxyurea therapy (OR 2.23; P = 0.0136), and long-term transfusion (OR 2.33; P = 0.014) were associated with AVN. In a median regression model, AVN, acute chest syndrome, and pneumonia were independently associated with greater urgent care visits and admissions., Conclusions: Symptomatic AVN was found to be an independent risk factor for acute care utilization in patients with SCD. Because this is a potentially modifiable factor, further studies are urgently needed to determine whether AVN prevention/early treatment interventions will alter utilization and improve outcomes for patients with SCD.

Sickle cell disease (SCD) is a heterogeneous inherited disorder of hemoglobin that causes chronic hemolytic anemia, vaso-occlusion, and endothelial dysfunction. These physiologic derangements often lead to multiorgan damage in infancy and throughout childhood. The most common types of SCD are homozygous hemoglobin S (HbSS disease), hemoglobin SC disease, and sickle β thalassemia. HbSS disease and sickle β(0) thalassemia often are referred to as sickle cell anemia because they have similar severity. Screening and preventive measures, including infection prophylaxis and vaccination, have significantly improved outcomes for children with SCD. Evidence-based therapies, such as hydroxyurea and transfusion, play an important role in preventing progression of select complications. Many chronic complications develop insidiously and require multidisciplinary care for effective treatment. Primary care physicians, as well as physicians in many other disciplines, may care for these patients and should be familiar with the potential acute and chronic complications of this disease. This review addresses healthcare maintenance guidelines, common complications, and recommendations for management of pediatric patients with SCD.

Objectives: Neurocognitive dysfunction is an important complication of sickle cell disease (SCD), but little is published on the utility of screening tests for cognitive impairment in people with the disease. The purpose of this study was to evaluate the Montreal Cognitive Assessment (MoCA) as a screening tool and identify predictors of MoCA performance in adults with sickle cell disease., Methods: We conducted a retrospective, cross-sectional study of the first 100 adult patients with SCD who completed the MoCA as part of routine clinical care at the Johns Hopkins Sickle Cell Center for Adults. We abstracted demographic, laboratory, and clinical data from each participant's electronic medical record up to the date that the MoCA was administered. The factorial validity of each MoCA domain was analyzed using standard psychometric statistics. We evaluated the abstracted data for associations with the composite MoCA score and looked for independent predictors of performance using multivariable regressions., Results: Components of the MoCA performed well in psychometric analyses and identified deficits in executive function that were described in other studies. Forty-six percent of participants fell below the cutoff for mild cognitive impairment. Increased education was an independent predictor of increased MoCA score (3.1, 95% confidence interval [CI] 1.5-4.7), whereas cerebrovascular accidents and chronic kidney disease were independent predictors of decreased score (-3.3, 95% CI -5.7 to -0.97 and -3.2, 95% CI -6.2 to -0.11, respectively). When analysis was restricted to patients with SCA, increased education (3.7, 95% CI 2.2-5.2) and a history of hydroxyurea therapy (2.0, 95% CI -0.022 to 4.0) were independent predictors of a higher score, whereas chronic kidney disease (-3.3, 95% CI -6.4 to -0.24) and increased aspartate transaminase (-0.045, 95% CI -0.089 to -0.0010) were independent predictors of a decreased score., Conclusions: The MoCA showed promise by identifying important cognitive deficits and associations with chronic complications and therapy., Competing Interests: The remaining authors have no financial relationships to disclose and no conflicts of interest to report.

Objectives: Poor oral health can have a negative impact on overall health. This is especially concerning for individuals with sickle cell disease (SCD), an inherited blood disorder that affects hemoglobin and can lead to an increased risk of infection and hyperalgesia. Because the majority of individuals with SCD have Medicaid insurance and no dental coverage, we provided free basic dental care to individuals with SCD to determine whether it decreased overall healthcare utilization., Methods: Through a contract with a private dental office, we provided free basic dental care (eg, cleanings, fillings, x-rays) to individuals with SCD. We reviewed medical records for the 12 months before and after their initial dental visit to determine whether there were any changes in acute care visits (defined as a visit to the emergency department, sickle cell infusion center, or visits to both in the same day), hospitalizations, and total days hospitalized. We conducted a negative binomial regression to determine any differences in the pre-post periods., Results: In our multivariable analysis, there was a statistically significant decrease in hospital admissions. In addition, there was a significant decrease in total days hospitalized if dental work was completed, but an increase in days hospitalized in men., Conclusions: Providing dental care to individuals with SCD who did not have dental insurance did not greatly alter acute care visits. A larger sample size may be necessary to observe an effect.

Objectives: Transcranial Doppler ultrasound (TCD) effectively identifies children with sickle cell anemia (SCA) who are at increased risk of stroke. We evaluated a low-cost quality improvement (QI) intervention to increase the proportion of children screened by TCD., Methods: We measured the proportion of children with SCA receiving appropriate TCD screening for increased cerebral blood velocity, a marker of stroke risk, for time periods before (April 1, 2009-July 1, 2010) and after (October 1, 2010-January 1, 2012) the implementation of a QI program. We sent eligible families personalized reminder letters, information on screening, and a refrigerator magnet imprinted with the recommended date of TCD screening., Results: Only 54% (60/112) of children (median age 8 years, range 2.2-16.7 years) had a TCD in the baseline period compared with 79% (87/110; P = 0.0001) after implementation of the QI initiative. The odds of appropriate TCD screening decreased with older age in the baseline period (odds ratio 0.86, 95% confidence interval 0.78-0.94/year; P = 0.001), but not after implementation. Neither predicted travel time by car (median 20 minutes, range 2-164) nor distance traveled (median 9.7 mi, range 0.4-132) was significantly associated with appropriate TCD screening before or after QI implementation. The number needed to treat was four., Conclusions: We demonstrated the feasibility and effectiveness of a low-cost QI intervention to increase TCD screening. This approach was more successful than other related intervention models and is easily implemented by smaller sickle cell programs without full-time personnel.

Objective: The purpose of this study was to determine the comfort levels of primary care providers in caring for individuals with sickle cell disease (SCD) and determine factors that improved or lessened provider comfort., Methods: We surveyed providers at the annual Johns Hopkins Community Physicians retreat. The survey consisted of 19 questions and measured comfort levels in four domains: providing ambulatory care to individuals with SCD, managing SCD comorbidities, managing SCD-specific issues, and managing chronic pain. We conducted bivariate analyses to identify any demographic or practice characteristics associated with comfort levels. Multivariable analyses were conducted to identify independent correlates of physician comfort., Results: The majority of respondents lacked confidence with each of the four aspects of caring for individuals with SCD. Having treated patients with SCD and using knowledge from residency were both independently associated with increased confidence when providing ambulatory care and managing SCD-specific issues in multivariable analyses., Conclusions: The delivery of high-quality care to adults with SCD in primary care may be limited because of a lack of provider comfort in providing that care. Because provider reliance on knowledge gained from residency significantly affected the management of patients with SCD, it is essential that continuing medical education on SCD is readily available to ensure that providers are using current information and knowledge. In addition, as comfort increases with the number of patients with SCD in a provider's panel, it may be beneficial to identify a subset of primary care providers interested in SCD and refer patients to those providers.