ALBERT

Description: Abstract 5171 Infants who are heterozygous for a number of (γδβ)0-thalassemia deletions are known to present with neonatal hemolytic anemia (Koenig et al, Am J Hematol 84:603, 2009). The breakpoints for most of these large deletions have not been identified. Molecular diagnoses of these deletions therefore can be challenging. We now report an infant girl of Irish/Scottish descent with self-limited fetal and neonatal hemolytic anemia, in whom we have defined the extent of the large deletion removing the entire β-globin gene cluster. At 32-week of gestation, fetal tachycardia prompted percutaneous umbilical cord blood sampling, which revealed a hematocrit of 14%. Umbilical cord transfusion was undertaken followed by Caesarean section delivery. Brisk hemolysis continued in the first few days of life (reticulocyte count of 22 – 24%), with no other causes of hemolysis identified. In the following weeks, the infant was transfused on three occasions. After 2-month of age, she became transfusion-independent with a stable microcytic anemia. The infant's mother also had a history of hemolytic anemia requiring transfusion in the neonatal period, and subsequently became transfusion independent with a microcytic anemia. The mother and several of her family members were extensively investigated, and shown to have a novel (γδβ)0-thalassemia deletion of over 100 kb (Pirastu et al, J Clin Invest 72:602, 1983). Multiplex ligation-dependent probe amplification (MLPA) was carried out in the genomic DNA from the present neonate. The infant was heterozygous for a large deletion spanning at least from 5′ to the HS 5 of the LCR to 3′ of the β-globin gene. Sequential gap-PCR reactions and nucleotide sequencing were done. The deletion was characterized with its 5` breakpoint at nt 5,376,341 and 3` breakpoint at nt 5,178,572 (GenBank NT_009237). The deletion measures 197,770 bp, removing the β-globin LCR, all of the β-like globin genes, and several olfactory receptor genes. A diagnostic gap-PCR test was established for detection of this deletion. This case illustrates the syndrome of neonatal hemolytic anemia caused by large deletions removing the entire β-globin gene cluster. MLPA is a useful tool to screen for these deletions. The pathophysiology of these self-limited and sometimes severe fetal and neonatal hemolytic anemias is presently not understood. We speculate that expression of α-hemoglobin stabilizing protein (AHSP) and/or the proteolytic capacity to degrade excess α-globin chains within erythroid cells might be diminished during fetal and neonatal development, accounting for increased red cell membrane damage and hemolysis in affected fetuses and neonates. Disclosures: No relevant conflicts of interest to declare.

Description: Abstract 4219 Background: Clinical characteristics associated with abdominal vein thrombosis (including hepatic, portal, and mesenteric veins; renal veins; and inferior vena cava [IVC]) overlap with characteristics associated with deep vein thrombosis (DVT) in the legs and pulmonary embolism (PE) but also possess unique attributes. These characteristics may differ between adult and pediatric patient groups. Methods: Using a standardized data-collection form, demographic and baseline characteristics were prospectively collected from consecutive consenting patients enrolled within one of seven Thrombosis and Hemostasis Centers over a seven-year period, August 2003 to June 2010. Patients with intra-abdominal venous thrombosis (defined as abdominal and renal veins, and IVC) were divided into pediatric (age

Description: Sickle cell disease (SCD) is a chronic disease for which complications include acute chest syndrome, painful vaso-occlusive crisis, and stroke. There has been increased use of chronic transfusions to prevent and treat complications associated with SCD (Blinder, 2013), however, the result is iron overload for which complications include liver failure, heart failure and death. (Niederau, 1996; Brittenham, 1994; Harmatz, 2000) To effectively prevent and treat iron overload, chelation with oral medications must be taken correctly (Kwiatkowski, 2004). Depending on the method used to measure medication compliance, adherence varies between 43% and 76%. (Raphael et al., 2009; Alvarez et al., 2009) The variability underscores the need to better investigate and improve adherence. The success of treatment plans requires collaboration among patients, families and providers to take prescribed medications. We sought to implement mobile technology to facilitate this collaboration, while at the same time providing education and engaging both patients and parents. We performed a feasibility study using an original mobile application to assess adherence with oral chelation therapy and provide a platform for education modules. Eligible patients had a diagnosis of SCD or thalassemia with a history of iron overload, were ≥5 years of age and had access to a phone or other mobile device with the iOS operating system. Devices were provided for use if needed. During the first 30 days of the study, participants were asked to self-record up to a 5-minute video of daily medication administration. They performed this with our mobile application, which we could review and track. On day 2-3 of participation, they received a generic welcome message via the application. On day 7 of participation, they received a personalized message of encouragement for consistent use, or a message prompting them to try harder for daily use. Weekly, for the remainder of the first month, they received generic text ‘pop-ups’ on the application. For months 2-3, patients were then asked to do a short photo log of daily medication administration. For months 4-6, they were asked to maintain a log and continue to record videos if they desired. In addition, during the initial 3 months, patients participated in educational modules and quizzes related to SCD/thalassemia, iron overload and chelation therapy. Data was analyzed starting after one week of participation. Eight patients (4 males and 4 females, median age 12.5 years old, range 8-19) have been consented to participate. Seven patients have SCD and 1 has thalassemia major. We have analyzed 106 data days for 5 patients; Two patients have not yet reached 1 week of participation, and one patient has been temporarily excluded due to difficulties with internet connectivity and inability to confirm entries. Compliance of recording ‘selfies’ was 84%. Average length of ‘selfie’ videos was 12.7 seconds for male participants and 74.7 seconds for females (range 3 to 300 seconds). Videos reviewed also included: messages to providers, indications of difficulties with taste, and interaction with parents while preparing medication. Three patients have returned for 30-day follow up. Mean ferritin in these patients prior to enrollment was 1948.3 ng/ml (SD 758.4) and at one month follow-up was 1589.3 (SD 147.6). This study illustrates that mobile technology in the form of an interactive application is a feasible method that can be used to increase adherence to therapy plans, such as oral chelation therapy. We were able to accurately document adherence (84%) using our newly developed mobile app and are interested to see long-term compliance following completion of the study. Interestingly, we also report longer ‘selfie’ videos for females and may argue for gender specific recommendations for improving compliance to treatment plans. Detailed review of videos also revealed information that may further assist in compliance such as identification of improper mixing. Further expansion of this study and evaluation of laboratory markers (such as ferritin) at 3 months will additionally support the use of a mobile technology based intensive training program for patients with SCD. Efforts are also underway employing a similar strategy for hydroxyurea adherence. In summary, we believe mobile technology has become an integral part of health care management and has the potential to improve care for patients with chronic illnesses. Disclosures Shah: Novartis: Speakers Bureau.

Description: Introduction: Sickle cell disease (SCD) is a complex disease for which pain is the hallmark. Pain from vaso-occlusive episodes is the number one reason for ED visits and hospital admissions. This paper reports Medicaid claims data from NC for individuals with SCD, including: 1) ED encounters and re-encounters within 7, 14 and 30 days; 2) hospitalization and re-hospitalization within 7, 14 and 30 days; and 3) ED reliance (EDR) score. Methods: We examined Medicaid claims data from for patients with a diagnosis of SCD (ICD 9 CM codes: 282.6x, ICD 10 CM codes: D57.0x, D57.1, D57.2x, D57.4x, D57.8x) in North Carolina. Data is reported for a cohort of 2,790 patients with a diagnosis of SCD, age 1 to 65+ and enrolled at least 11 months in NC Medicaid between March 1, 2016 and February 28, 2017. ED re-encounters and re-hospitalizations within 7, 14 and 30 days were identified using the time between the date of service listed on the ED or hospital claim and the next date of service in the subsequent claim. Individual ED Reliance (EDR) score was calculated as the total number of ED encounters divided by the total ambulatory visits (outpatient + ED encounters) per enrollee, (ambulatory visits reported elsewhere). Similar to Kroner et al, an EDR of 〉0.33 was considered a high score. Inpatient claims were identified using a category of service code indicating hospitalization. Results: The participants in the sample (n=2790) were majority female (57.92%), lived in metropolitan areas (77.63%) and had a mean age of 23.05 years old (SD=16.06). Of the 9,075 total ED encounters, 69.86% of the total sample had an ED encounter during the 12-month study period. There was a mean of 3.25 (SD=7.38) and median of 1 (IQR = 0 - 3) ED encounters per patient for the sample. Those who were 18-30 years old had the highest mean and median ED encounters per patient (4.98, SD= 9.34 and 2, IQR 1 to 5). The 31-45 year old group had the second most, with 4.82 (SD= 11.03) total ED encounters. The percentage of the sample with an ED re-encounter within 7, 14, and 30 days was also highest among the 18-30 year old group (29.17%, 33.98% and 40.89%) followed by those 31-45 years old (23.71%, 28.49%, and 34.80%), respectively. The 31-45 age group had the second most hospitalizations/patient and re-hospitalizations. The mean EDR was highest among 18-30 year old patients (0.35) and 46.48% of this age group had an EDR of 0.33 or greater. In the 31-45 year-old age group, the mean EDR was 0.28 and 35.18% had an EDR of 0.33 or greater. The overall sample had a mean of 1.30 (SD= 2.75) hospitalizations/patient. The 18-30 year old age group also had the highest mean total hospitalizations (2.08, SD= 3.72) and mean re-hospitalizations within 7 (0.16; SD=0.77), 14 (0.41; SD=1.68), and 30 (0.82; SD=2.79) days. The 31-45 age group had the second most hospitalizations/patient and re-hospitalizations (Table 1). Conclusions: Overall, increasing age coincided with increased ED and inpatient utilization, as well as with the period of transition from pediatric to adult SCD care. Furthermore, high EDR was most prevalent in the 18-30 age group. Our study further supports the need for increased focus on acute care utilization in the 18-45 year-old age group and considerations for improved care transition interventions. Disclosures Tanabe: NIH: Research Funding; AHRQ: Research Funding. Shah:Novartis: Consultancy, Research Funding, Speakers Bureau; Alexion: Speakers Bureau; GBT: Research Funding.

Description: Introduction: Sickle cell disease (SCD) is a complex genetic disease with a multifactorial pathophysiology including multi-cell adhesion between red blood cells, white blood cells, platelets and endothelial cells, ultimately resulting in vaso-occlusive crises (VOCs). VOCs are the hallmark of SCD and are the primary cause for hospitalization. These recurrent episodes induce severe pain, decrease quality of life, can cause life-threatening complications, and are associated with increased risk of organ damage and mortality. While the clinical burden of SCD is well-documented, less evidence exists surrounding how the severity of SCD affects patients economically. The number of VOCs and other complications not only affect the likelihood of patients being able to work on a given day, but may also affect their long-term economic prospects. This analysis aims to better understand the association between SCD disease severity and its impact on the likelihood of collecting Supplemental Security Income (SSI) and patient income. Methods: A web-based survey was administered to adult patients (≥18 years) living in the United States with a self-reported diagnosis of SCD. The outcomes of interest for this analysis were Social Security Income (SSI) receipt and self-reported total household annual income. These outcomes were stratified by SCD disease severity. Our first measure of disease severity was the number of self-reported VOCs in the prior year. Our second measure of disease severity was developed through clinical expert opinion and relied on an algorithm for dividing patients into 3 disease severity classes. Severity Class I was defined as having no VOCs requiring treatment by health care providers in the past year; Severity Class II was defined as ≥1 emergency department visit or hospital admission for a VOC, or complication in the past year without any organ damage; and Severity Class III was defined as long-term organ damage (such as stroke or renal disease). Generalized linear models (GLM) with a binomial link function were used to analyze the association between SCD disease severity and SSI collection (one model used VOC frequency; a second model used severity classes). A linear regression model was used to analyze the relationship between VOC frequency and income level, while an ordered logistic regression model was used to analyze the association between SCD disease severity classes and income level. Results: The final sample was comprised of 303 individuals who completed the survey. The average age was 34.4 years (range 18 - 72) and 221 (72.9%) were female. The probability of SSI collection among patients with SCD varied across VOC frequency in the previous year. The probability of collecting SSI for patients having 0 and ≥4 VOCs in the past year, was 12% (95% confidence interval (CI): 4% to 31%) and 47% (39% to 55%), respectively. A chi-squared p-value of 0.002 indicated a statistically significant association between a greater number of VOCs and probability of SSI collection. The probability of SSI collection among SCD patients with Severity Class II and Severity Class III SCD was 16% (7.5% to 32%), and 39% (32.9% to 45%), respectively. A chi-squared p-value of 0.03 indicated a statistically significant association between SCD disease severity class and SSI collection. The predicted mean income for patients with SCD experiencing 0 and ≥4 VOCs in the past year was $47,488 and $34,569, respectively. A linear association test p-value of 0.06 indicated weak evidence of a lower mean income in relation to number of VOCs experienced. The predicted mean income among patients with class II and class III SCD was $42,443 and $36,842, respectively. There was no evidence of association between SCD severity class and mean income (p=0.29). Conclusion: Among patients with SCD, having more VOCs was strongly associated with the probability of collecting SSI and weakly associated with lower income. Disease severity class was strongly associated with the probability of collecting SSI. Disclosures Shafrin: Precision Health Economics, part of Precision Medicine Group: Employment, Equity Ownership. Thom:Bayer AG: Consultancy; Hoffman-La Roche: Consultancy; Pfizer: Consultancy; Novartis Pharma AG: Consultancy. Gaunt:Novartis Pharma AG: Consultancy. Zhao:Precision Health Economics, part of Precision Medicine Group: Employment. Joseph:Cigna: Equity Ownership; Pfizer: Equity Ownership; Amgen: Equity Ownership; Novartis: Employment, Equity Ownership. Bhor:Novartis: Employment, Equity Ownership. Rizio:Optum: Employment. Bronté-Hall:bluebird bio: Research Funding. Shah:GBT: Research Funding; Alexion: Speakers Bureau; Novartis: Consultancy, Research Funding, Speakers Bureau.

Description: Abstract 1004 Hydroxyurea (HU) is approved for use in adults with Sickle Cell Disease (SCD) and increases the production of fetal hemoglobin (HbF). Increased HbF is associated with decreased clinical severity in adults and children with SCD, such as decreased numbers of vaso-occlusive events, transfusions, and hospitalizations. Higher HbF at initiation of HU is predictive of HbF response, but association between age of hydroxyurea initiation and HbF response has not been investigated. We hypothesize that starting hydroxyurea at an early age may improve hematological and clinical response. In order to determine if younger age at hydroxyurea initiation affects the percentage of HbF achieved with hydroxyurea, we conducted a retrospective cohort study. We identified subjects enrolled in the Duke University Medical Center Comprehensive Sickle Cell program who initiated hydroxyurea when they were less than 17.99 years of age and were prescribed hydroxyurea for at least six months. The following data were abstracted from the medical record between December 1996 and April 2011: age, hemoglobin, percentage HbF, and mean corpuscular volume (MCV) at start of HU and at maximum tolerated dose (MTD) of HU therapy. The correlation coefficient and p-values for various parameters were calculated. Seventy-three patients (41 males and 32 females) were included in the analysis. The mean age at hydroxyurea initiation was 5.5 years (1.2–14.1). The mean hydroxyurea dose at MTD was 28.6 ± 3.2 mg/kg/day. At initiation, the mean hemoglobin was 8.2 ± 1.2 g/dL, the mean MCV was 83±7.4 fl and mean HbF was 10 ± 5.7%. At MTD, the mean hemoglobin was 9.4 ± 1.1 g/dL, the mean MCV was 99 ± 11.1 fl, and the mean HbF was 21.7 ± 9.4%. As expected, at MTD, an elevated MCV was correlated with elevated fetal hemoglobin (r2= 0.19, p= 0.0001) [Table 1]. There was a statistically significant relationship between the age at HU initiation and the HbF at MTD (r2= 0.08, p= 0.015) [Figure 1] as well as the age at HU initiation and the hemoglobin at MTD (r2= 0.19, p= 0.016). The relationship between the age at starting HU and the overall change in HbF (DHbF) was not statistically significant (r2= 0.01, p= 0.41). There was not a statistically significant relationship between age at HU initiation and the MTD of HU (r2= 0.003, p= 0.61). The 6 patients started on HU at age less than 2 years (mean 1.5 ± 0.3 years) maintained a mean elevated HbF of 19.1 ± 5% at last documented follow-up with follow-up ranging from 1.4–13 year of uninterrupted hydroxyurea use. Starting hydroxyurea therapy at a younger age appears to improve HbF response as measured at MTD, although there is variability in the level of fetal hemoglobin attained. There is not an association seen with the DHbF or dose at MTD and age at hydroxyurea initiation. In summary, starting hydroxyurea at a younger age, when HbF is 〉20%, leads to persistence of HbF production and overall improvement in hematological efficacy. This was not simply the result of achieving MTD at a younger age before physiologic decline of HbF. Disclosures: Off Label Use: Hydroxyurea for complications of sickle cell disease in pediatrics. Shah:Eisai: Research Funding; Adventrx: Consultancy.

Description: Abstract 1649 INTRODUCTION: Implantable venous access devices (VADs) are used in sickle cell disease (SCD) for patients with poor venous access to facilitate chronic blood transfusions and management of acute complications. Children and adults with chronic illnesses have high rates of VAD-related complications including bloodstream infection and thrombosis. Patients with SCD may be at higher risk given the presence of functional asplenia and evidence of a hypercoaguable state. The objective of this study was to define the frequency of VAD related bloodstream infections and thrombosis in adults and children with SCD. PATIENTS AND METHODS: We performed a single institution retrospective review of VAD placement in patients with SCD. Subjects were identified through the sickle cell clinic database and the Hospital Information System. Subjects were included if they had SCD, VAD placement between December 1, 1998 to December 1, 2009 and had completed at least 12 months of follow-up. VAD-related bloodstream infection was defined by positive blood culture and VAD-related thrombosis (deep vein thrombosis, superior vena cava syndrome, and pulmonary embolism without lower extremity thrombosis) was defined by imaging. Comparisons were made between pediatric and adult sickle cell patients using Student's t-test for continuous variables and Fisher's exact test was used to compare categorical variables; p

Description: Background Adolescents and young adults (AYAs) with sickle cell disease (SCD) are at increased risk for disease complications and mortality, particularly during transition to adult care. Little is known about the disease course that might be contributing to the increase in morbidity and mortality during transition. This study provides a longitudinal description of the clinical characteristics and disease severity among AYAs with SCD during transition to adult care. Methods We conducted a longitudinal analysis of the electronic health records of 339 AYAs (ages 12-27 years) with SCD (97% black, 56% male, 69% hemoglobin SS) who received care at a comprehensive SCD center in the Southeast US between 1989 and 2015. Measures included sociodemographics, transfer, complications, comorbidities, and severity of SCD (using a modification of the pediatric SCD severity index). We used group-based trajectory modeling to identify subgroups of AYAs with distinct severity trajectories. AYAs who were in care for at least 10 years were included (n=133). We used chi-square and unpaired student t-test to explore subgroup differences among (1) AYAs who died compared to AYAs who were still alive during the study; (2) AYAs who were eligible to transfer (i.e., ≥ 19 years) (n=293) and transferred compared to AYAs who were eligible to transfer and did not transfer; and (3) AYAs with different severity trajectories. Statistical significance was set at p=0.05 and 95% CI. Results Common complications among AYAs (n=339) included vaso-occlusive crises (80%), acute chest syndrome (41%), chronic pain (35%), and cerebral infarcts (22%). Comorbidities included depression (19%) and anxiety (14%). Most AYAs who were eligible to transfer transferred (n=220, 75%) at a mean age of 19 years (SD=1.3). Fourteen AYAs died, 10 (71%) after transfer at a mean of 7.3 years (SD=3.1) from transfer. SCD Severity Trajectories (Figure 1): Group-based trajectory modeling 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%) severity trajectory groups. Most AYAs (74%) had stable severity over time, whereas 26% had increased severity with increasing age. Subgroup Differences in Care and Mortality: AYAs who died (n=14) spent less time in pediatric care (mean=4.6 years, 95%CI 3.7-5.6), however, had a higher mean number of pediatric annual visits (mean=9.3 visits; 95%CI 6.3-12.3) compared to those who were alive (n=324, mean=6 years in pediatric care, 95%CI 5.7-6.1; mean number of pediatric annual visits=5.4, 95%CI 4.8-6.1). There were no differences in demographics, SCD complications, or morbidity. Subgroup Differences in Transfer to Adult Care (Tables 1 and 2): Compared to AYAs who did not transfer, AYAs who transferred were older and lived closer to the SCD clinic. AYAs who transferred were at higher risk for SCD complications and comorbidities. They were more likely to be receiving hydroxyurea. They had higher mean annual pediatric SCD clinic visits, especially during older adolescence, and were in pediatric care for a longer duration. Subgroup Differences in Disease Severity (Tables 3 and 4). Compared to AYAs in stable severity trajectory groups (Figure 1: Groups 1, 3, and 5), AYAs in unstable disease severity trajectory groups (Figure 1: Groups 2 and 4) were more likely to be married, older, and lived closer to the SCD clinic. AYAs in unstable groups were also at higher risk for SCD complications and comorbidities. They were less likely to be receiving treatments and had less pediatric SCD clinic visits by age 18. However, they were more likely to transfer to adult care, have had more adult SCD visits overall, and remained longer in adult SCD care after transfer. Conclusions Whereas most AYAs with SCD had stable disease severity, nearly a quarter had increasing disease severity, over time. AYAs with increasing severity lived closer to the SCD clinic, were more likely to transfer to adult care, and demonstrated higher and longer use of adult SCD care compared to AYAs with stable disease severity. Genotype was not associated with disease severity trajectory groups, underscoring the importance of clinical care for AYAs, over time. Disclosures Kayle: Department of Health and Human Services, Administration for Community Living, NIDILRR Advanced Rehabilitation Research Training Health and Function Grant #90AR5019 (PI Heinemann): Other: post-doctoral fellowship; Department of Health and Human Services, Administration for Community Living, NIDILRR Advanced Rehabilitation Research Training Health and Function Grant # 90ARHF0003 (PI Heinemann): Other: Postdoctoral fellowship. Tanabe:Duke University: Employment; NIH and AHRQ: Research Funding; Alliant Health: Consultancy. Maslow:The Crohn's and Colitis Foundation and the American Gastroenterological Association, in collaboration with Pfizer, Inc: Research Funding. Holl:Before Brands, Inc: Research Funding; NIH: Research Funding; Branstad Family Foundation: Research Funding; AHRQ: Research Funding. Shah:Novartis: Research Funding, Speakers Bureau.

Description: Abstract 3221 Children with sickle cell disease (SCD) are at high risk for thrombotic stroke. Transcranial doppler ultrasound (TCD) is utilized to predict children at highest risk. Anemia and and vessel occlusion are assocated with elevated TCD velocities and risk of stroke. There is increasing evidence that a prothrombotic state contributes to the complications of SCD including stroke. We hypothesized that increased thrombin generation and platelet activation are associated with increased TCD velocities. We conducted a cross-sectional cohort study of children with SCD at Duke University Medical Center. Children had been clinically well for at least 2 weeks, had not been transfused for at least 3 months, and had TCD performed at study enrollment (n= 28) or within the prior 6 months (n=7). TCD time-averaged mean velocities (TAMV) were calculated for each patient and used for analysis. Blood was collected at the time of study entry per study protocol. Thrombin generation was measured utilizing: D-dimer; thrombin antithrombin (TAT); and calibrated automated thrombography (CAT) which evaluates phases of thrombin production including lag phase, time to peak thrombin, endogenous thrombin potential (ETP), and peak thrombin. Platelet activation was assessed by measurement of soluble glycoprotein V (sGPV). Clinical data were abstracted from the medical record including confirmation of SS genotype, use of hydroxyurea, prior sickle cell-related clinical events, and hematological data including hemoglobin, platelets and white blood cell count. SCD severity scores were calculated to classify patients as either high risk or low/moderate risk (van den Tweel et al, 2010). Linear regression analyses were conducted to assess correlation. Children with high risk severity scores were compared to children with low/moderate risk severity scores. Statistical analysis was performed using Graphpad 5.0; p

Description: Introduction. Advances in medicine in the last four decades have allowed adolescents with Sickle Cell Disease (SCD) to survive into adulthood. Consequently, there is a sizeable number of maturing young adults with SCD who require transition from pediatric to adult care. This transition can be difficult given the tumultuousness of outgrowing adolescence and entering adulthood, the burden of living with a chronic disease, and the self-advocacy and medical independence expected from patients by adult health care providers. To better understand utilization of care during this period in a comprehensive SCD center, we investigated the number of patients who transferred and successfully transitioned from pediatric outpatient hematology care to adult care. Methods. Following IRB approval, a retrospective chart review of patients with SCD aged 18-23 years in the Duke University Medical Center electronic medical record database was conducted. Included for each patient was a list of all clinic encounters at Duke through the December 2013. A total of 342 SCD patient records were retrieved, of which 88 (26%) were excluded because of missing data on outpatient SCD encounters. Transfer was defined as a clinic visit in pediatrics followed by a subsequent visit in the adult clinic. Successful transition was defined as at least 1 year of follow up care in adult hematology clinic post-transfer. Results. The 284 patient records fell into three utilization patterns: 182 whose last encounter was a pediatric visit (72%), 20 who started care in the adult clinic (8%), and 52 who transferred from pediatric to adult care (20%). Within the pediatric group, 36 patients were actively receiving care while 146 were lost to follow-up. Among patients that transferred, the average age of transfer was 18.6 years and the transfer occurred within 3 months of the last pediatric visit for 61% of patients. Although 71% of transferred patients continued care for at least 1 year post-transfer, the majority of 19-20 year old patients (61%) did not transition successfully. Discussion. Our findings show that SCD patients aged 18-23 are having difficulty transferring and successfully transitioning from pediatric to adult hematology care in the Duke Medical Center despite having a single medicine-pediatric provider. The majority of patients had their last visit in pediatric care which is consistent with Duke’s SCD clinical practice to keep 18-20 year old patients in pediatric care to better educate patients. The large number of patients who stopped care in pediatrics may be explained by care provided by non-Duke hematologists or primary care providers; relocation; or use of acute care clinics (emergency department, urgent care, and day hospital) as a substitution for regular care. As observed in this data, when transfer does occur among this younger adult group of 18-20 year olds, only a third of the patients have a successful transition. The low success rate may be accounted for by patient impression of health; avoidance of regular care; loss of insurance; and struggle with independence. Implications. The results underscore the need to not only improve successful transition rates for SCD patients but also to assist and track patients preparing to transfer to adult specialty clinics. Further research is needed to determine what is happening to patients who are lost to follow-up during their pediatric care and to determine the relationship between the amount of time to transfer and successful transition rates. Because our robust definition of a successful transition is more sensitive to identifying gaps in continuous care as compared to other success metrics based on a 50% show rate, we were able to identify 19-20 years as a critical age group for intervention. Disclosures Shah: Novartis: Speakers Bureau.