scholarly journals A Novel Sickle Cell Outreach Program Improves Access to TCD Screening, Vaccines and Hydroxyurea in a Medically Underserved Area

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4696-4696
Author(s):  
Emily Riehm Meier ◽  
Leticia Brown ◽  
Kisha Hampton ◽  
Ellen Bloom ◽  
DeAuntae Lawson ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Care Coordination ◽  
Screening Tests ◽  
Outreach Program ◽  
Underserved Area ◽  
Lake County ◽  
Vaccination Rates ◽  
Pediatric Hematology ◽  
Outreach Clinic ◽  
Multi Disciplinary Team

Background: The Sickle Care Coordination, OutReach, Education Program (SCORE) was created in 2017 to improve access to hematology care and care coordination services for children with sickle cell disease (SCD) in an urban community in Lake County, Indiana. Although Lake County has the second highest prevalence of SCD in Indiana, it lacks a pediatric hematologist, and the closest pediatric hematology practice is 65 miles away. Newborn screening data from 2009-2017 showed that only 25% of children in this underserved area received Transcranial Doppler (TCD) and only 25% received the 23 valent pneumococcal vaccine, compared with 70% for both in other regions of Indiana. The goal of the SCORE clinic is to increase TCD screening and vaccination rates as well as increase the number of children who are prescribed hydroxyurea (HU) at maximum tolerated dosing (MTD). Methods: Quarterly SCORE outreach clinics were established in Lake County, Indiana in August 2017, which is 145 miles Northwest of our center. A multi-disciplinary team (social worker, sickle cell educators, nursing staff) accompanies the pediatric hematologist and physician assistant to clinic. Vaccines are administered in SCORE clinic and laboratory tests are drawn on site. Same day TCD screening was established in May 2018. The state's electronic vaccine record was queried to determine which SCD-specific vaccines the children needed prior to each clinic. Education about SCD, HU, and needed screening tests like TCD was provided by members of the multi-disciplinary team. Results: One hundred thirty-one visits have been made to the 10 SCORE outreach clinics that have been held since August 2017. Forty children have received hematology care in the SCORE clinic; 35 (87.5%) children have had at least 2 visits. Twenty-two children were eligible for HU; sixteen (72.7%) were already taking HU at the time of their first visit to SCORE outreach clinic (mean dose prior to first SCORE visit: 18.6 + 5.9 mg/kg/day). Mean HU dose increased significantly after being followed by the SCORE clinic team (26.5 mg/kg/day, p=0.00002). The remaining 6 children initiated HU after receiving care in the SCORE outreach clinic. Nineteen children were eligible for TCD screening since their first SCORE clinic visit, and fourteen (73.7%) children completed the screening. One hundred twenty-one vaccines have been given in clinic to date: 100% of children who were eligible for the 23 valent pneumococcal, hepatitis A and B vaccines have received them. Seasonal influenza vaccine was given to 76.9% of eligible children. Forty-six of the 54 (85.2%) Menveo doses that were due were given, and 80% of Bexsero doses that were due were given. Conclusion: The SCORE program has increased access to pediatric hematology care for children with SCD in an urban, underserved community in Indiana. In the first 2 years of the program, TCD screening rates and pneumococcal vaccination rates have increased to the level seen in other regions of the state. Children who received care in the SCORE outreach clinic had a significant increase in their HU dosing. Outreach clinics can increase access to specialty care in underserved areas. Disclosures Meier: CVS Caremark: Consultancy. OffLabel Disclosure: Hydroxyurea prescription in children less than 2 years of age.

scholarly journals Neutrophil gelatinase–associated lipocalin as a biomarker of nephropathy in sickle cell disease

2021 ◽  
Author(s):  
Rajaa Marouf ◽  
Adekunle D. Adekile ◽  
Hadeel El-Muzaini ◽  
Rasha Abdulla ◽  
Olusegun A. Mojiminiyi
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Roc Curve ◽  
Screening Tests ◽  
Cell Disease ◽  
Urine Ngal ◽  
Neutrophil Gelatinase Associated Lipocalin ◽  
Plasma Ngal ◽  
Neutrophil Gelatinase

AbstractSickle cell nephropathy (SCN) develops via altered hemodynamics and acute kidney injury, but conventional screening tests remain normal until advanced stages. Early diagnostic biomarkers are needed so that preventive measures can be taken. This study evaluates the role of neutrophil gelatinase–associated lipocalin (NGAL) as a biomarker of SCN in steady state and vaso-occlusive crisis (VOC). In this case-control study, 74 sickle cell disease (SCD) patients (37 in steady state and 37 in VOC) and 53 control subjects had hematological and biochemical measurements including plasma and urine NGAL. Univariate and logistic regression analyses were used to find the associations between variables. The receiver operating characteristic (ROC) curve was used to determine the diagnostic performance characteristics of plasma and urine NGAL for detection of VOC. Plasma and urine NGAL, urine microalbumin:creatinine ratio, and urine protein:creatinine ratio were significantly higher in VOC. Microalbuminuria was present in 17.1% steady state and 32.0% VOC patients. Microalbuminuria showed significant correlations with age, plasma NGAL, WBC, and hemolytic parameters. Area under the ROC curve for plasma NGAL was 0.69 (95%CI = 0.567–0.813; p = 0.006) and 0.86 (95%CI = 0.756–0.954; p < 0.001) for urine NGAL. Urine NGAL cut-off value of 12.0 ng/mL had 95% sensitivity and 65% specificity. These results confirm the presence of nephropathy during VOC and suggest that plasma and urine NGAL would be useful in the identification of SCN. Urine NGAL should be used as the screening biomarker, and patients with VOC and urine NGAL > 12.0 ng/mL should be selected for aggressive management to prevent progression of renal damage.

scholarly journals Pediatric Hematologist/Oncologists' Beliefs about Hematopoietic Stem Cell Transplant for Children with Sickle Cell Disease: A Sickle Cell Transplant Advocacy and Research Alliance (STAR) Study

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2164-2164
Author(s):  
Emily Riehm Meier ◽  
Allistair Abraham ◽  
Alexander I. Ngwube ◽  
Isaac Janson ◽  
Gregory M.T. Guilcher ◽  
...  
Keyword(s):  
Stem Cell ◽  
Sickle Cell ◽  
Thalassemia Major ◽  
Cell Transplant ◽  
Cell Disease ◽  
Asymptomatic Child ◽  
Hematopoietic Stem ◽  
Curative Therapy ◽  
Pediatric Hematology ◽  
Asymptomatic Children

Background Hematopoietic stem cell transplantation (HSCT) provides a curative therapy for children severely affected by sickle cell disease (SCD). Rejection-free survival following a matched sibling donor (MSD) HSCT for these children is very high. As safer approaches are developed, using reduced intensity conditioning and improved graft versus host disease prophylaxis, there is rationale for extending MSD HSCT to less severely affected children to spare them increasing morbidity and early mortality in adulthood. Providers' perceptions may contribute to the slow adoption of MSD HSCT for less severe SCD. In this study we assess providers' perceptions about MSD HSCT for children with variable SCD severity and determine the influence of provider characteristics on HSCT attitudes. Methods Pediatric Hematologists/Oncologists (PHO) were eligible to participate in this IRB exempt study. An e-survey was distributed to all STAR members and ASH members who self-identified as PHO and posted on the American Society of Pediatric Hematology/Oncology Clinical Forum listserv. Analysis was performed to describe participant demographics and the proportion of participants who practice exclusively Pediatric Hematology with a focus on SCD (PSCD) vs. general PHO; we evaluated correlations between these characteristics and likelihood of HSCT referral for each scenario. Results Of the 203 respondents, 59% were female, and 69% were between the ages of 30 and 49 years. Spearman's rank correlation analysis did not reveal any significant relationship between respondent age and likelihood to refer to HSCT for any of the survey scenarios. Two-thirds self-identified as Caucasian, 19% Asian, 7% African American and 3% Hispanic. 35% of respondents practiced general PHO, 20% PSCD, 20% Pediatric Hematology, 15% Pediatric HSCT, and 4% Hemostasis. The majority (75%) of respondents were very or somewhat likely to order HLA typing for a child with HbSS/HbSβ⁰thalassemia who had full siblings, regardless of disease severity. Only 48% would refer a child with HbSS/HbSβ⁰thalassemia who had an HLA-MSD but never admitted to the hospital; referral likelihood differed significantly by practice focus [PHO: 23% very or somewhat likely vs. 54% of PSCD (p=0.002)]. Conversely, 99% of respondents were very or somewhat likely to refer a child who suffered an overt stroke, while 85% and 84% were very or somewhat likely to refer a child who had an abnormal TCD or silent infarct, respectively. 49% of respondents were very or somewhat likely to refer a child with HbSS/HbSβ⁰thalassemia if they had a history of suboptimal adherence to hydroxyurea; referral likelihood again differed by practice focus [PHO: 35% very or somewhat likely vs. 64% PSCD (p=0.021)]. Concern about transplant related mortality (TRM) was the predominant reason for not referring. For children with severe clinical phenotypes of HbSC and HbSβ+thalassemia, 78% of respondents were very or somewhat likely to refer, compared to only 23% who would refer asymptomatic children with SCD variants. PHO and PSCD did not differ in the likelihood to refer either group. 87% of respondents were very or somewhat likely to refer a child with β-thalassemia major for MSD HSCT, regardless of disease severity; no statistically significant difference was found between PHO and PSCD in referral likelihood. A significantly higher proportion of respondents would refer an asymptomatic child with β-thalassemia major (87%) than those who were very or somewhat likely to refer an asymptomatic child with HbSS/HbSβ⁰thalassemia (47%, p<0.00001) or other SCD variants (23%, p= 0.0005). Discussion HSCT is a curative therapy for people with SCD. There was almost complete agreement that a HSCT referral should be made for children with HbSS/HbSβ⁰thalassemia and cerebral vasculopathies who have an MSD, but attitudes varied for almost every other clinical scenario posed. PSCD providers were more likely to refer asymptomatic patients and those with questionable adherence to hydroxyurea compared to general PHOs. TRM was the most common reason for not referring an asymptomatic child for MSD HSCT. Yet, TRM is low in the setting of pediatric MSD HSCT for SCD. Additional education about the decreased quality of life in aging people with SCD and their lack of improvement in life expectancy is needed so that physician perception of HSCT changes and the number of young asymptomatic children with SCD referred for MSD HSCT increases. Disclosures Meier: CVS Caremark: Consultancy. Guilcher:Jazz Pharmaceuticals: Other: ASH 2017 meeting attendance.

Influenza vaccination rates and hospitalizations among Medicaid enrollees with and without sickle cell disease, 2009–2015

2021 ◽  
Author(s):  
Amanda B. Payne ◽  
Thomas V. Adamkiewicz ◽  
Scott D. Grosse ◽  
Andrea Steffens ◽  
David K. Shay ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Influenza Vaccination ◽  
Sickle Cell ◽  
Cell Disease ◽  
Vaccination Rates

scholarly journals Sickle Cell Anaemia Prevalence among Newborns in the Brazilian Amazon-Savanna Transition Region

2019 ◽  
Vol 16 (9) ◽  
pp. 1638
Author(s):  
Rayane Cristina Souza ◽  
Pedro Agnel Dias Miranda Neto ◽  
Jessflan Rafael Nascimento Santos ◽  
Sílvio Gomes Monteiro ◽  
Maria Cláudia Gonçalves ◽  
...  
Keyword(s):  
Public Health ◽  
Transition Region ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Sickle Cell Trait ◽  
Public Health Problem ◽  
The State ◽  
Screening Tests ◽  
Distribution Analysis ◽  
Important Public Health Problem

Sickle cell anaemia is one of the most common hemoglobinopathies worldwide and an important public health problem in Brazil. This study evaluated the prevalence of sickle cell anaemia and its traits in newborns from the Amazon-Savanna Transition Region in the state of Maranhão, Brazil. A cross-sectional study was carried out, based on data from neonatal screening tests performed in 2013–2015 in Maranhão. The Hardy-Weinberg theorem was applied to analyse the frequency of expected homozygotes based on HbSS phenotype. A spatial-temporal distribution analysis was performed to delimit the regions with the greatest number of newborn cases with sickle cell anaemia. Of 283,003 newborns, 162 were found to have sickle cell anaemia, while 10,794 had a sickle cell trait, with a prevalence of 0.05% and 3.8%, respectively. The prevalence of expected homozygotes was higher in the North Region and in the state capital of Maranhão. This study may contribute to existing social and public health actions or the creation of new strategies for sickle cell disease in endemic areas in Brazil to improve the quality of life.

Understanding Provider Barriers to Hydroxyurea Use for Pediatric Sickle Cell Disease

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 255-255
Author(s):  
Suzette O. Oyeku ◽  
Nancy S. Green ◽  
Farzana Pashankar ◽  
Patricia Giardina ◽  
Craig A. Mullen ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Young Children ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Phase Iii ◽  
Evidence Based ◽  
Cell Disease ◽  
Pediatric Hematology ◽  
Clinical Indications ◽  
Provider Barriers

Abstract Abstract 255 Despite proven efficacy in clinical trials, hydroxyurea (HU) has not been uniformly adopted into the care of children with sickle cell disease (SCD). In 2008, the NIH Consensus Development Conference on Hydroxyurea Treatment for Sickle Cell Disease postulated that barriers to HU use may occur at the provider level. Limited evidence exists on barriers to the use of HU, and prior studies have largely focused on use in adults. HU use is rapidly expanding to different indications, to use in patients with less common hemoglobin genotypes and to young children. Initial data from the Pediatric Hydroxyurea Phase III Trial (BABY HUG) in very young children (ages 9–18 months) is also now becoming available. To better understand current provider barriers to effective translation of efficacy trial results into “real-world” clinical care of children with SCD, we surveyed pediatric hematology providers within several regional consortiums of pediatric hematology programs in the eastern US. The objectives of our study were to: 1) describe practice patterns related to HU use among providers of children and adolescents with SCD; 2) identify provider level barriers to HU use among SCD children; and 3) solicit provider recommendations to overcome the perceived barriers. A close-ended, self-administered web-based survey was sent to 230 pediatric hematology providers in June 2010. Provider demographics, practice characteristics, clinical indications to prescribe HU, concerns related to HU use and suggestions to improve HU use were assessed by this survey. Forty-two percent (N=97) of 230 surveys were completed by hematologists (84%), nurse practitioners (12%) and physician assistants (3.7%). The number of SCD patients in provider practices ranged from 2 to 1,200 patients. 57% of respondents were female. 42% of respondents were in practice for more than 20 years. The majority (72%) of providers were white. Many providers (83%) were somewhat/very familiar with the NHLBI guidelines about HU use in SCD. Among those surveyed, the most frequent indications to start HU were: 1) history of 3 painful episodes, 2) acute chest syndrome, 3) chronic pain use requiring narcotics, 4) priapism and 5) symptomatic anemia. A majority of providers (82%) reported using HU in children ages 3–5 years of age, with 41% of providers indicated using HU in children less than 3 years of age. Fewer than half of providers (28%) prescribe HU to patients with Hgb SC or other Hgb S variants. Only 74% of providers attempted to titrate HU to maximal tolerated dose. This goal dose ranged from 20 to 40mg/kg/day among our respondents. Major provider concerns about HU in children are: 1) patient compliance with taking HU, 2) compliance with attending drug monitoring visits, 3) compliance with taking contraception, 4) effects of HU on fertility and 5) long term side effects. Almost 50% of clinicians were concerned about the age of the patient when starting HU: 48.5% of clinicians considered patients less than 1 years of age too young to start HU, while 40% of clinicians felt patients' ages 1–2 years were too young. Some providers (39%) had concerns about the efficacy of HU in patients with Hgb SC, while 24.1% were concerned about efficacy in patients with Hgb S variants. Providers' suggestions to improve HU use included: 1) developing updated evidence based practice guidelines for HU use (89%), 2) developing culturally appropriate patient educational materials about HU (84%), 3) extending FDA approval for HU to children (80%), and 4) developing a national registry of patients on HU to monitor clinical outcomes and adverse events (74%). Our survey highlights that HU use varies among pediatric providers with respect to: 1) the broader clinical indications for HU use, 2) optimal maximal tolerated dose of HU, 3) appropriate lower age limit to prescribe HU, and 4) sickle cell genotype in which to use HU. Updated national evidence- based guidelines to assist clinicians in using HU in pediatric sickle cell care are indicated given the efficacy of HU for SCD over a wide range of indications, the logistical limits and tempo of clinical studies, the paucity of other widely available treatments, and persistent barriers to HU use at the provider level. Additional studies are warranted to examine alternative indications for HU, HU use in younger ages, optimum dosing, potential impact on fertility, teratogenicity and possible carcinogenicity, and use of HU for other sickle cell genotypes. Disclosures: Off Label Use: Hydroxyurea has not been FDA approved for use in children and adolescents with sickle cell disease, the topic of the submitted abstract.

Pneumococcal Vaccination Rates in Children With Sickle Cell Disease

2014 ◽  
Vol 20 (6) ◽  
pp. 587-590 ◽  
Author(s):  
Alecia C. Nero ◽  
Kwei Akuete ◽  
Sarah Leasure Reeves ◽  
Kevin J. Dombkowski
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pneumococcal Vaccination ◽  
Cell Disease ◽  
Vaccination Rates

scholarly journals Pediatric Hematology Providers on Referral for Transplant Evaluation for Sickle Cell Disease

2014 ◽  
Vol 36 (7) ◽  
pp. 566-571 ◽  
Author(s):  
Bethany Mikles ◽  
Monica Bhatia ◽  
Suzette O. Oyeku ◽  
Zhezhen Jin ◽  
Nancy S. Green
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Pediatric Hematology ◽  
Transplant Evaluation

scholarly journals Validation and Feasibility of a Point of Care Screening Test for Sickle Cell Disease in a Resource Constrained Setting — a New Frontier

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2229-2229 ◽  
Author(s):  
Christopher Mwaniki Wanjiku ◽  
Festus Njuguna ◽  
Fredrick Chite Asirwa ◽  
Cyrus Njuguna ◽  
Chris Roberson ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Screening Test ◽  
Point Of Care ◽  
Screening Tests ◽  
Cell Disease ◽  
Positive Screen ◽  
Sub Saharan ◽  
Study Participants ◽  
Lost To Follow Up

Abstract Introduction and Objectives Sickle cell disease (SCD) is a neglected tropical disease disproportionally affecting malaria endemic regions of sub-Saharan Africa. Estimated to affect up to 3% of the population in some areas, 50-90% of children affected with SCD die before age five due to lack of diagnosis and preventative care. Early screening and intervention improves survival, but is not the standard of care throughout much of the continent. Kenya, like several other sub-Saharan African countries, has piloted newborn screening (NBS) for SCD, but NBS programs based on the infrastructure and approaches in Western countries have proved challenging to replicate in resource constrained environments (RCEs). As many as 50% of patients who have screened positive for SCD in programs throughout Africa are lost to follow-up. The recent development of affordable, point of care (POC) screening tests have provided an alternative approach to testing for SCD in RCEs. This study aimed to determine the validity and feasibility of utilizing a POC SCD screening test, HemoTypeSC™ (HTSC) in a RCE, to determine the prevalence of SCD in western Kenya, and to determine the rate of attendance at follow-up clinic after a positive POC screening test for SCD. Methodology Asymptomatic patients from birth through five years of age were approached for study enrollment in the pediatric vaccine clinic of Homabay County Referral Hospital (HCRH). 700 study participants were screened at HCRH over a 6 month period using HTSC. Isoelectric focusing (IEF) was run on a sample collected contemporaneously with the sample collected for testing with HTSC . At least one parent of each study participant was consented and counselled regarding SCD. Participants with a positive screen for either SCD or Sickle Cell Trait (SCT) were asked to return for confirmatory testing with Hb Electrophoresis (HBE). The HTSC screening results were compared to IEF and HBE results to determine the specificity and sensitivity, respectively. Samples yielding discordant results between HTSC or IEF and HBE were referred for molecular genotyping. Study participants with confirmed SCD were scheduled to attend a follow-up clinic. Participants unreachable by phone after at least ten attempts were declared lost to follow up. Results The median age of participants was 14 months (IQR: 5,30), 387(55.3%) were male. 18 patients had discordant results between screening tests and HBE and are pending molecular testing. 214 (30.6%) subjects screened positive for SCT or SCD on HTSC . 155 of these patients returned for HBE testing. The sensitivity of HTSC from the 155 receiving HBE testing was 92.1% for SCD and 95.0% for SCT. By comparison, the sensitivity of the concurrent IEF testing was 90.2% for SCD and 90.0% for SCT. The specificity of HTSC was 95.0% for SCD and 89.1% for SCT (as compared to IEF specificity of 92.0% and 89.1% for SCD and SCT, respectively). 9.6% of the subjects were found to have SCD, 20% had SCT. Of those with a positive screen for SCT or SCD, 191 (88%) were successfully contacted. 40 (78.4%) of those subjects confirmed to have SCD attended a follow-up clinical appointment. These data are summarized in Table 1. The majority of participants contacted who did not present for HBE testing or attend follow-up clinic cited socioeconomic factors as the main impediment. Discussion and Conclusions HTSC was found to be a feasible, valid POC screening test for SCD. The test was easy to perform and interpret, and facilitated prompt delivery of results. Education and counseling about SCD at the time of a positive screen led to a high rate of attendance at follow-up clinic. Further investigation is required to determine the long-term effect of POC testing on survival and quality of life outcomes. The prevalence of SCD in this cohort was among the highest rates reported in the literature. Though consanguinity was not documented as part of this study, it likely contributed to the high Hb SS frequency. These results support the notion that there is substantial regional variability in the frequency of Hb SS compared to national estimates extrapolated from Hb S frequency and highlight the criticality of more systematic, broad-based screening and treatment programs in targeted sub-Saharan African locations. Figure. Figure. Disclosures Wanjiku: Silverlake Research Cooperation: Research Funding.

scholarly journals Acute Chest Syndrome in Children with Sickle Cell Disease in Italy: Results of a National Survey from the Italian Association of Pediatric Hematology Oncology (AIEOP)

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2301-2301
Author(s):  
Vania Munaretto ◽  
Raffaella Colombatti ◽  
Serena Ilaria Tripodi ◽  
Corti Paola ◽  
Simone Cesaro ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Italian Association ◽  
Pediatric Hematology ◽  
Frequent Event ◽  
Regional Hospitals

Background: Acute Chest Syndrome (ACS) is the second cause of hospitalization in Sickle Cell Disease (SCD), burdened by significant morbidity and mortality. The guidelines regarding management of ACS are sometimes difficult to follow in the real world and the prevention and treatment strategies of ACS are often applied in an uneven manner in the various settings (community care, regional hospitals, reference university centers). Moreover, epidemiology, clinical phenotype and outcomes as well as risk factors could vary in different populations according to ethnicity, genotype or health care system organization. Aims and Methods: A retrospective multicenter observational study was conducted to investigate the epidemiology of ACS and to the evaluate the diagnostic and therapeutic pathways of ACS in children with SCD (age 0-18 years) in the 2013-2018 period, after the publication of the Italian Association of Pediatric Hematology Oncology (AIEOP) Guidelines for the Management of SCD in Childhood in Italy in 2012. Results: 126 children were recruited and 122 included in the analysis, with 208 evaluable episodes of ACS (range: 1-6 episodes /patient) from 11 AIEOP Centers. 73 M, 49 F. Mean age was 10.9 years. 85% patients were of African origin, 92% were HbSS/SB°; mean age at diagnosis of SCD of the entire cohort was 25,3 months (range 0-16,8). 44.2% of patients had more than one episode of ACS during the study period; 37% had had a previous episode before 2013. 58% had comorbidities, mostly respiratory (asthma or allergy). 75% of the patients underwent disease modifying treatment during study period (73% hydroxyurea, 2% chronic transfusion). The seasonality of ACS episodes was important in our country: 75% of episodes occured between October and March. 95% of ACS episodes were secondary to a Vaso-Occlusive Crisis. 76% of the admissions occurred in SCD reference centers, 24% in regional hospitals, but 30% later required transfer to reference centers for worsening of clinical conditions or need of exchange transfusion. The mean length of hospitalization was 9.6 days (range 1-46); one patient died of pneumococcal sepsis; 6 episodes required transfer to the Intensive Care Unit, mechanical ventilation was required in one episode. A good adherence to the AIEOP Guidelines was documented for some aspects: 99% of the patients were hospitalized, 98% performed chest X-ray for the diagnosis of ACS and in 99% antibiotic therapy was started. Others aspects were less satisfactory and in need of improvement: incentive spirometry was only performed in 19% of admissions; oxygen therapy was performed only in 75% of patients even if SatO2 was<95%; transfer to reference centers was not always timely. During 75% of ACS episodes a simple red cell transfusion was required for Hb>8g/dl, while in 16% an exchange transfusion was performed for severe respiratory distress (of these 71% were performed in patients transfered from regional hospitals); 38% required inhaled bronchodilators, 6% steroids. A preliminay evaluation of risk factors for recurrent ACS showed that in our cohort allergy to inhaled allergens (p 0.02) and enuresis (p 0.01) were associated with increased prevalence of recurrent ACS; patients with asthma/wheezing also presented more recurrent ACS compared to patients wihout them (23% vs 13%) but this data did not reach statistical significance. Conclusion: This study represents the first analysis in Italy of ACS, which is confirmed as a frequent event in our cohort, with a significant proportion of patients who experience recurrent ACS. Steps need to be undertaken to improve management of ACS and adherence to the AIEOP guidelines at a national level: stimulate the application of early preventive measures that are still under-utilized, increase the appropriateness of multidisciplinary specialist approach (transfusion specialist, acute care physicians, pneumologists, hematologists) strengthen the dissemination of information through training events for all the Hospitals of the network. Disclosures Colombatti: AddMedica: Consultancy; Global Blood Therapeutics: Consultancy; Novartis: Consultancy.

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