Costs and impact of disease in adults with sickle cell disease: a pilot study.

ABSTRACT: We assessed the feasibility to estimate illness burden in adults with SCD, investigated factors associated with health-related quality of life (HRQoL), and estimated societal burden. We recruited 32 participants and collected data on fatigue, HRQoL, and work productivity and activity impairment via patient survey. Health care utilization was abstracted for the 12 months before enrollment using medical chart review. Mean age was 36.7 years; 84.4% of participants had hemoglobin SS or Sßthal0 disease, and 81.3% reported chronic pain (experiencing pain on ≥3 days per week in the past 6 months). Mean EQ-5D-3L visual analogue scale score was 63.4 and the index score was 0.79. The mean fatigue score was 57.9. Higher fatigue score was correlated with lower EQ-5D index score (correlation coefficient r = -0.35; P = .049) and Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me) scores, including pain (r = -0.47; P = .006), sleep (r = -0.38; P = .03), and emotion scores (r = -0.79; P < .0001). The number of hospitalizations was negatively correlated with HRQoL (all P < .05). Patients who reported chronic pain had significantly lower mean ASCQ-Me sleep scores (48.3 vs 57.1; P = .04) and EQ-5D index scores (0.72 vs 0.89; P = .002) than those without chronic pain. Mean estimated annual per person costs were $51 779 (median, $36 366) for total costs, $7619 ($0) for indirect costs (estimated from lost earnings of participants), and $44 160 ($31 873) for medical costs. Fatigue, SCD complications, hospitalization, and chronic pain negatively affected HRQoL. This sample experienced a high economic burden, largely from outpatient doctor visits.

Disparities in pediatric hospital use during transition to adult healthcare for young adults with childhood-onset chronic conditions.

BACKGROUND: Young adults (YA) with childhood-onset chronic conditions-particularly YA with cystic fibrosis (CF), congenital heart disease (CHD), and sickle cell disease (SCD)-continue to have pediatric hospital admissions. Factors associated with this continued pediatric hospital use remain underexplored. OBJECTIVE: To determine if pediatric hospital use by YA differed (1) across condition and (2) within each condition by sociodemographic factors. METHODS: Conducted a cross-sectional analysis of admissions for YA 22-35 years with CF, CHD, and SCD from 2016 to 2020 in the National Inpatient Sample. Admissions for YA with CF, CHD, and SCD were identified by international classification of diseases, 10th revision-clinical modification diagnosis codes. To determine if conditions or sociodemographic factors were associated with YA pediatric hospital use, we used multivariable logistic regression with separate models for the different objectives. RESULTS: YA with SCD had lower odds of pediatric hospital use compared to YA with CF. Relationships between sociodemographic factors and pediatric hospital use varied. Black YA with both CF and CHD had lower odds of pediatric hospital use than white YA with CF and CHD. For YA with SCD, despite 17,810 (6.5%) having rural residence, zero (0) had pediatric hospital use; whereas YA with CF living in a rural area had greater odds of pediatric hospital use compared to urban residents. CONCLUSION: YA with SCD used pediatric hospitals less than YA with either CF or CHD. Coupled with our findings that Black YA with CF and CHD had less pediatric hospital use, these data may reflect systematic racial differences within pediatric to adult healthcare transition programs.

The Montreal cognitive assessment as a cognitive screening tool in sickle cell disease: Associations with clinically significant cognitive domains.

Adults with sickle cell disease (SCD) are at risk for cognitive impairment, which causes significant morbidity. Guidelines support routine cognitive screening, but no screening test is validated in this population. We explored the Montreal Cognitive Assessment (MoCA) as a possible screening test in SCD. We administered the MoCA; a literacy test, the Wide Range Achievement Test, fourth edition (WRAT-4); and a health literacy test, the Shortened Test of Functional Health Literacy in Adults (S-TOFHLA) to adults with SCD and gathered clinical variables through chart review. Spearman's rho, Mann-Whitney, and Kruskal-Wallis tests and quantile regression models were used. Among our sample of 49 adults with SCD, the median MoCA score was 25.0 [interquartile range (IQR) 22.0-28.0]. Higher educational attainment was associated with MoCA scores (p = 0.001). In multivariable models, MoCA scores were associated with S-TOFHLA (p = 0.001) and WRAT-4 Reading (p = 0.002) scores, and overt stroke (p = 0.03) at the median. This pilot study adds to the limited literature of cognitive screening tests in adults with SCD and demonstrates a relationship between MoCA scores and measures of literacy and health literacy. The MoCA is a promising option for briefly screening for cognitive impairment in adults with SCD, though further study is needed to confirm its validity.

Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects.

Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a medically and socially complex, multisystem illness that affects individuals throughout the lifespan. Given improvements in care, most children with SCD survive into adulthood. However, access to adult sickle cell care is poor in many parts of the United States, resulting in increased acute care utilization, disjointed care delivery, and early mortality for patients. A dearth of nonmalignant hematology providers, the lack of a national SCD registry, and the absence of a centralized infrastructure to facilitate comparative quality assessment compounds these issues. As part of a workshop designed to train health care professionals in the skills necessary to establish clinical centers focused on the management of adults living with SCD, we defined an SCD center, elucidated required elements of a comprehensive adult SCD center, and discussed different models of care. There are also important economic impacts of these centers at an institutional and health system level. As more clinicians are trained in providing adult-focused SCD care, center designation will enhance the ability to undertake quality improvement and compare outcomes between SCD centers. Activities will include an assessment of the clinical effectiveness of expanded access to care, the implementation of SCD guidelines, and the efficacy of newly approved targeted medications. Details of this effort are provided.

The Economic Burden of End-Organ Damage Among Medicaid Patients with Sickle Cell Disease in the United States: A Population-Based Longitudinal Claims Study.

BACKGROUND: The management of sickle cell disease (SCD), an inherited, chronic, and multifaceted condition, is associated with considerable health care resource utilization (HRU) and costs, especially for Medicaid. Anemia affects most patients with SCD and correlates with end-organ damage (EOD), such as stroke, chronic kidney disease (CKD), end-stage renal disease (ESRD), and pulmonary hypertension (PH). Limited research has been conducted to quantify the economic burden of EOD among patients with SCD. OBJECTIVE: To estimate the effect of EOD on HRU and direct costs and productivity loss incurred by patients with SCD on Medicaid. METHODS: Patients with ≥ 3 nondiagnostic SCD ICD-9-CM/ICD-10-CM codes in ≤ 5 years (January 1, 2013-December 31, 2017) were identified in the MarketScan Medicaid claims database. The earliest SCD diagnosis date was the index date. Continuous enrollment at least 3 months before and 1 month after the index date were required. Patients' post-index periods were divided into 3-month intervals (referred to as "intervals"). History of stroke, CKD, ESRD, and PH were identified in patients' claims histories from January 1, 2008. Intervals within 1 year and more than 1 year after an acute stroke event were also defined. All-cause HRU, direct costs, and productivity losses were summed across intervals and stratified by EOD type. Multivariate regression models were used to estimate the effect of stroke, CKD, ESRD, and PH on annual total cost, inpatient days, and number of emergency department visits by controlling for patients' demographic characteristics and other SCD complications. RESULTS: In total, 10,784 Medicaid patients with SCD (average age: 18.5 years; female: 54.5%) contributed to 152,455 intervals. Approximately 12% of the intervals had EOD. Patients with EOD had higher all-cause health care costs and more inpatient days, emergency department visits, outpatient visits, laboratory tests, and outpatient pharmacy claims than patients without EOD. After controlling for patient characteristics, among Medicaid patients with SCD annual costs within 1 year after stroke were 4.68-fold versus patients with no EOD (more than 1 year after stroke: 2.08-fold; CKD: 2.19-fold; ESRD: 3.40-fold; PH: 2.32-fold). Adjusted mean annual costs for adult patients with SCD on Medicaid were $285,816 and $127,393 within 1 year and more than 1 year after stroke and $135,493, $209,172, and $148,174 for CKD, ESRD, and PH, respectively. Patients with multiple SCD complications had even higher costs. The mean annual time patients with SCD spent receiving health care services ranged from 56 to 62 days for those with EOD versus 21 to 25 days among those without EOD, which created additional economic burden. CONCLUSIONS: When Medicaid patients with SCD experience EOD, the economic burden is significantly increased through direct costs to the health care system and indirect costs from productivity loss to society. SCD management strategies that potentially reduce the risk of EOD offer clinical and economic value to patients and society. DISCLOSURES: Funding for this study was provided by Global Blood Therapeutics (GBT). Campbell is a consultant for GBT, Bluebird Bio, and Cyclerion and receives research funding from Novartis, GBT, and Cyclerion. Cong and Agodoa are employees of and have equity ownership in GBT. Song, Martinez, Black, Lew, Varker, and Chan are employees of IBM Watson Health, which received research funding from GBT for this study. Lanzkron receives research funding from GBT, Pfizer, Ironwood, HRSA, and NIH. A poster based on this study was presented at the 61st ASH Annual Meeting and Exposition; December 7-10, 2019; Orlando, FL.

Comparison of US Federal and Foundation Funding of Research for Sickle Cell Disease and Cystic Fibrosis and Factors Associated With Research Productivity.

Importance: Sickle cell disease (SCD) and cystic fibrosis (CF) are severe autosomal recessive disorders associated with intermittent disease exacerbations that require hospitalizations, progressive chronic organ injury, and substantial premature mortality. Research funding is a limited resource and may contribute to health care disparities, especially for rare diseases that disproportionally affect economically disadvantaged groups. Objective: To compare disease-specific funding between SCD and CF and the association between funding and research productivity. Design, Setting, and Participants: This cross-sectional study examined federal and foundation funding, publications indexed in PubMed, clinical trials registered in ClinicalTrials.gov, and new drug approvals from January 1, 2008, to December 31, 2018, in an estimated US population of approximately 90 000 individuals with SCD and approximately 30 000 individuals with CF. Main Outcomes and Measures: Federal and foundation funding, publications indexed in PubMed, clinical trial registrations, and new drug approvals. Results: From 2008 through 2018, federal funding was greater per person with CF compared with SCD (mean [SD], $2807 [$175] vs $812 [$147]; P < .001). Foundation expenditures were greater for CF than for SCD (mean [SD], $7690 [$3974] vs $102 [$13.7]; P < .001). Significantly more research articles (mean [SD], 1594 [225] vs 926 [157]; P < .001) and US Food and Drug Administration drug approvals (4 vs 1) were found for CF compared with SCD, but the total number of clinical trials was similar (mean [SD], 27.3 [6.9] vs 23.8 [6.3]; P = .22). Conclusions and Relevance: The findings show that disparities in funding between SCD and CF may be associated with decreased research productivity and novel drug development for SCD. Increased federal and foundation funding is needed for SCD and other diseases that disproportionately affect economically disadvantaged groups to address health care disparities.

Predictors of acute care utilization and acute pain treatment outcomes in adults with sickle cell disease: The role of non-hematologic characteristics and baseline chronic opioid dose.

Despite its rarity in the United States, sickle cell disease accounts for a disproportionate amount of healthcare utilization and costs. The majority of this is due to acute care for painful crises. A small subpopulation of patients accounts for most these costs due to frequent visits to emergency departments and acute care facilities. Previous investigations have found that these high utilizing patients are distinguished by both a more severe disease course and certain non-hematologic characteristics, which may include higher socioeconomic status and some psychiatric and psychological characteristics. This prospective observational cohort study was undertaken to test the ability of these characteristics to prospectively predict acute pain care outcomes, including visit frequency, total opioid doses, and pain improvement at the Johns Hopkins Sickle Cell Infusion Center (SCIC). Seventy-three participants were followed for 12 months and SCIC utilization and treatment outcomes were tabulated for 378 visits. Participants who visited the SCIC most frequently had markedly worse pain improvement despite higher within-visit opioid doses. Higher utilization was associated with indicators of greater illness severity, more aggressive treatment for sickle cell disease, higher baseline opioid doses, higher socioeconomic status, greater pain-related anxiety, and a history of psychiatric treatment. Overall, poor acute pain treatment response was associated with higher utilization and higher baseline opioid doses. The pattern of association between high utilization, poor acute care outcomes, and higher baseline opioid doses is discussed in terms of prior research and future directions.

Do Words Matter? Stigmatizing Language and the Transmission of Bias in the Medical Record.

BACKGROUND: Clinician bias contributes to healthcare disparities, and the language used to describe a patient may reflect that bias. Although medical records are an integral method of communicating about patients, no studies have evaluated patient records as a means of transmitting bias from one clinician to another. OBJECTIVE: To assess whether stigmatizing language written in a patient medical record is associated with a subsequent physician-in-training's attitudes towards the patient and clinical decision-making. DESIGN: Randomized vignette study of two chart notes employing stigmatizing versus neutral language to describe the same hypothetical patient, a 28-year-old man with sickle cell disease. PARTICIPANTS: A total of 413 physicians-in-training: medical students and residents in internal and emergency medicine programs at an urban academic medical center (54% response rate). MAIN MEASURES: Attitudes towards the hypothetical patient using the previously validated Positive Attitudes towards Sickle Cell Patients Scale (range 7-35) and pain management decisions (residents only) using two multiple-choice questions (composite range 2-7 representing intensity of pain treatment). KEY RESULTS: Exposure to the stigmatizing language note was associated with more negative attitudes towards the patient (20.6 stigmatizing vs. 25.6 neutral, p < 0.001). Furthermore, reading the stigmatizing language note was associated with less aggressive management of the patient's pain (5.56 stigmatizing vs. 6.22 neutral, p = 0.003). CONCLUSIONS: Stigmatizing language used in medical records to describe patients can influence subsequent physicians-in-training in terms of their attitudes towards the patient and their medication prescribing behavior. This is an important and overlooked pathway by which bias can be propagated from one clinician to another. Attention to the language used in medical records may help to promote patient-centered care and to reduce healthcare disparities for stigmatized populations.

Utility of the Montreal Cognitive Assessment as a Screening Test for Neurocognitive Dysfunction in Adults with Sickle Cell Disease.

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.

Effect of Free Dental Services on Individuals with Sickle Cell Disease.

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.

Impact of a dedicated infusion clinic for acute management of adults with sickle cell pain crisis.

Most adults with sickle cell disease (SCD) receive care for their acute painful episodes in an emergency department (ED) setting. The purpose of this article is to describe the impact of opening a dedicated treatment center for adults with SCD [Sickle Cell Infusion Clinic (SCIC)] on patient outcomes and on hospital discharges for SCD. Descriptive data including demographics, time to first dose of narcotic, and pain scores were collected on patients presenting to the SCIC and ED. Maryland hospital discharge data were obtained from the Maryland Health Services Cost Review Commission. Analyses were conducted using T tests, χ(2) tests, and simple generalized estimating equation regression models accounting for the clustered nature of observations, as appropriate. There were 3,874 visits to the SCIC by 361 unique patients; 85% of those visits resulted in the patient being sent home. During the same time period, there were 3,408 visits to the ED by 558 unique patients with SCD. The overall admission rate from the ED for these patients was 35.9% but decreased significantly over the time period with a rate of 20% in December 2011. There was a significant decrease in readmissions over time for the entire Baltimore Metro area with the likelihood of readmission decreasing by 7% over time. The SCIC model provides adults with SCD access to high quality care that decreases the need for hospital admission. Further research needs to be done to evaluate the cost effectiveness of this model.

Problematic hospital experiences among adult patients with sickle cell disease.

BACKGROUND: Adults with sickle cell disease (SCD) have often reported difficulties obtaining care during vaso-occlusive crisis (VOC) in qualitative studies. METHODS: We measured the experiences of 45 SCD patients who received in-hospital care for VOC using the Picker Patient Experience Questionnaire (PPE-15), and used the one sample binomial test to compare with national norms. RESULTS: Most SCD patients reported that they were insufficiently involved in decisions (86%), staff gave conflicting information (64%), it wasn't easy to find someone to discuss concerns (61%), doctors' answers to questions were not clear (58%), nurses' answers to questions were not clear (56%), doctors did not always discuss fears and anxieties (53%), and nurses did not always discuss fears and anxieties (52%). A greater percentage of SCD patients than the U.S. sample in 9 of 12 areas reported problems. CONCLUSIONS: Further research is needed to determine the consequences of and potential interventions to improve these poor experiences.

The burden of emergency department use for sickle-cell disease: an analysis of the national emergency department sample database.

It is estimated that there are 100,000 people living with sickle-cell disease (SCD) in the United States [1]. The most common manifestation of SCD is vaso-occlusive crisis, which is characterized by intermittent, unexpected episodes of excruciating pain. As these episodes often come on suddenly, much of the care for these crises occurs within emergency departments (EDs). Several studies have examined ED use and costs for certain groups of patients with SCD [2-4]. For example, in 1997, Woods et al. [2] found that 85.7% of 7,202 hospital admissions for SCD in Illinois were for patients that came through the ED, and the total charges for sickle-cell admissions in Illinois were found to be $30 million a year. A recent study of healthcare use by children with SCD demonstrated that children insured by Medicaid had higher ED utilization than those with private insurance (57% vs.45%) [5]. The purpose of our study was to provide national level estimates of ED utilization by SCD patients, which have not previously been available.

Examining the effectiveness of hydroxyurea in people with sickle cell disease.

This study investigated hydroxyurea use in people with sickle cell disease (SCD) outside of a research setting. Pharmacy data, outpatient visits, hospital admissions, and length of stay were assessed for all patients with SCD enrolled in a Medicaid managed care organization in Maryland. Three hundred and ninety (390) people with SCD were covered between the years 2001-2005. A large majority (85.9%) never had a claim for a hydroxyurea refill. Hydroxyurea users had higher admission rates than non-hydroxyurea users (5 vs. 1.5, p=.004). Patients who were in the highest tertile of refills of hydroxyurea had significantly fewer hospital admissions than patients in the lowest tertile (2.44 vs. 7.57, p=.043). Patients with the lowest hydroxyurea refill usage had significantly higher mean costs per month enrolled than those with the highest number ($4,553 vs. $2,017, p=.031). Hydroxyurea was underutilized in this patient population. Patients with more regular refills of hydroxyurea had fewer admissions to the hospital and markedly decreased costs.

A systematic review of barriers and interventions to improve appropriate use of therapies for sickle cell disease.

Clinical experts have expressed concern about underutilization of sickle cell disease (SCD) therapies, including hydroxyurea, prophylactic antibiotics, iron chelation, bone marrow transplantation, pain management during vaso-occlusive crisis, and receipt of routine ambulatory health care. We synthesized studies that identified barriers to and interventions to improve appropriate use of these therapies. Of the 48 studies included in our review, 35 identified therapeutic barriers or facilitators, and 13 evaluated interventions to improve use of therapies. Consistently identified barriers to appropriate pain management were negative provider attitudes and lack of provider knowledge. Four of 9 pain management interventions improved direct measures of pain management quality, while 5 improved indirect measures. One intervention improved receipt of routine ambulatory care. We concluded that interventions to improve pain management in SCD can be effective and should address providers' negative attitudes and knowledge and that more intervention studies are needed to improve receipt of recommended SCD therapies.

The course and correlates of high hospital utilization in sickle cell disease: Evidence from a large, urban Medicaid managed care organization.

Although most patients with sickle cell disease (SCD) are hospitalized infrequently and manage painful crises at home, a small subpopulation is frequently admitted to emergency departments and inpatient units. This small group accounts for the majority of health care expenses for patients with SCD. Using inpatient claims data from a large, urban Medicaid MCO for 5 consecutive years, this study sought to describe the course of high inpatient utilization (averaging four or more admissions enrolled per year for at least 1 year) in members with a diagnosis of SCD and a history of hospitalizations for vaso-occlusive crisis. High utilizers were compared with the other members with SCD on demographics, medical and psychiatric comorbidity, and use of other health care resources. Members who were high utilizers had more diagnostic mentions of sickle cell complications than low utilizers. However, the pattern of high inpatient utilization was likely to moderate over successive years, and return to the pattern after moderation was uncommon. Despite this, a small subpopulation engaged in exceptional levels of inpatient utilization over multiple years.

Provider barriers to hydroxyurea use in adults with sickle cell disease: a survey of the Sickle Cell Disease Adult Provider Network.

The FDA approved hydroxyurea for use in adults with sickle cell disease (SCD) in 1998. In 2002, the NHLBI published recommendations for the use of hydroxyurea in this patient population. The purpose of this study was to evaluate providers' awareness of the NHLBI recommendations regarding hydroxyurea prescribing, whether these recommendations have changed providers' practices and how these providers prescribed hydroxyurea. We chose to survey a select group of providers of care to adults with SCD, members of the Sickle Cell Adult Provider Network. The response rate was 47%. Eighty-one percent of respondents reported they had read the NHLBI recommendations on hydroxyurea use, and 76% of respondents agreed with the NHLBI recommendations. Only 45% of respondents to this survey reported that they prescribe hydroxyurea to every eligible patient. Black respondents reported a higher percentage of their eligible patients being on hydroxyurea than white respondents. Seventy-seven percent of respondents thought that hydroxyurea was effective or very effective in patients with SCD. Ninety-four percent of black versus 73% of white versus 40% of other race respondents thought that hydroxyurea was effective or very effective in SCD patients (p = 0.006). The results of this survey suggest that lack of awareness, agreement and belief in the benefits of hydroxyurea contributes to providers underprescribing hydroxyurea.

Hospitalization rates and costs of care of patients with sickle-cell anemia in the state of Maryland in the era of hydroxyurea.

The multicenter study of hydroxyurea (MSH) in sickle-cell anemia (SCA) demonstrated that patients treated with hydroxyurea (HU) had a 44% decrease in hospitalizations when compared with those taking placebo. A subsequent study looking at the cost-effectiveness of HU showed that decreased hospitalizations for painful crisis accounted for the majority of cost savings in those taking HU. The purpose of this study was to examine whether the expected decrease in hospital utilization occurred after the approval of HU in Maryland. We used data collected by the Maryland Health Services Cost Review Commission to obtain SCA discharge data for Maryland from FY1995 through FY2003. We also reviewed the inpatient and outpatient charts of all adults with SCA admitted to a large university hospital during 2003. Hospitalization rates for adults with SCA in Maryland have increased significantly since approval of HU. While the total costs of inpatient care in Maryland are estimated to have increased by 31% above inflation from 1995 to 2003, the costs of inpatient care for adult SCA patients has increased by almost 60% above inflation. By comparison, there has been no significant increase in the pediatric hospitalization rate. We found that 70% of patients in one hospital who were appropriate candidates for HU were not taking the medication. Hospital utilization among adults with SCA has increased significantly. There are likely many factors that have played a role in this increase. One factor that appears to be involved is the underutilization of HU.