scholarly journals High Incidence and Recurrence Rate of Venous Thromboembolic Events in Patients with Sickle Cell Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2381-2381
Author(s):  
Bart J. Biemond ◽  
Ewout R. Egner ◽  
Erfan Nur ◽  
Charlotte F.J. Van Tuijn
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Recurrence Rate ◽  
Sickle Cell ◽  
Organ Damage ◽  
Clinical Risk Factors ◽  
Cell Disease ◽  
Clinical Risk ◽  
High Incidence

Abstract Introduction: Sickle cell disease (SCD) is characterized by chronic hemolytic anemia and chronic inflammation resulting in endothelial damage, neutrophil and platelet activation, enhanced cell adhesion and coagulation activation. Due to these conditions, sickle cell disease is considered to be a procoagulant condition. However, limited studies have evaluated the incidence of thrombosis in sickle cell disease and clinical risk factors for thrombosis are not clear. Here we present a retrospective cohort study to determine the incidence of VTE in a well characterized cohort of adult sickle cell patients. The aim of the study was to assess the cumulative incidence of venous thromboembolic events in patients with SCD and to relate this complication with clinical risk factors, genotype, organ damage and laboratory parameters. Methods: All patients with SCD (HbSS, HbSC, HbSβ0 thalassemia, HbSβ+ thalassemia) of ≥ 18 years in a tertiary clinic for SCD at the Amsterdam University Medical Center in the Netherlands were eligible for the study. Patients with a history of prior VTE or VTE or VTE at first presentation were excluded. Patients were divided into a subgroups with a severe genotype (HbSS/HbSβ0 thalassemia) and a mild genotype (HbSC/HbSβ+ thalassemia). Hospital electronic records were analyzed for occurrences of deep vein thrombosis (DVT) or pulmonary embolism (PE) objectified with diagnostic tests (ultrasound, high probability ventilation/perfusion scan or CT angiography). All sickle cell related complications, organ damage and clinical risk factors at the time of the VTE were scored. General laboratory parameters were gathered from patient records in steady state condition. Results: In total 228 patients were included in the study with a mean age of 24 ± 10 years at the start of follow up years and a total follow up of 1548 patient years. Median follow up was 5 years (IQR 2-10). Twenty-one patients suffered one or more VTE episodes (9.2%), resulting in an incidence rate in the entire cohort of 13.6 VTE events per 1000 patient years (95%Cl 9.7-22.0). We recorded 8 recurrences of VTE in 5 patients (1 patient suffered two VTE recurrences, and 1 patient had three VTE recurrences) with a median time to recurrence of 3 years (IQR 1-4). Mean age at the time of first VTE was 29 years. The first VTE episodes consisted of an isolated DVT of the leg/arm in 8/21 patients, an isolated PE in 10/21 patients, and a combined DVT and PE in 3/21 patients. Of these first VTE episodes 9/21 (43%) were found to be idiopathic, 5/21 (24%) occurred during oral contraceptive use and 7/21 (33%) were provoked by recent surgical procedure/hospital admission/central venous catheter. VTE was significantly associated with a previous history of acute chest syndrome (ACS) (OR 10.6 [3.3 - 46.6] P<.001), avascular necrosis (AVN) (OR 5.6 [2.1 - 15.0] P <.001) and a ferritin level >1000 μg/L (OR 3.8 [1.4 - 10.2] P=0.023). No significant association with other forms of organ damage was found. Patients with a severe genotype had a higher incidence of VTE than patients with a mild genotype (11.4% versus 5.6%). In the severe genotype subgroup, lower median HbF levels correlated with an increased risk of VTE (P <.005). Ten patients died during follow-up at a mean age of 40 ± 16 years. Conclusion: Sickle cell patients have a remarkably high incidence rate of VTE (13.6 events per 1000 patients years) with a high recurrence rate (23.8%). This confirms the hypercoagulable state of patients with SCD. Sickle cell related complications like ACS and AVN were associated with VTE. Given the high incidence and recurrence rate, awareness for VTE in SCD patients is warranted and long term anticoagulation may be indicated. Disclosures No relevant conflicts of interest to declare.

scholarly journals Psychosocial and Clinical Risk Factors Associated with Substance Use in Observational Cohort of Patients with Sickle Cell Disease

2020 ◽  
Vol 55 (13) ◽  
pp. 2205-2212 ◽  
Author(s):  
J. Deanna Wilson ◽  
Sophie Lanzkron ◽  
Lydia H. Pecker ◽  
Shawn M. Bediako ◽  
Dingfen Han ◽  
...  
Keyword(s):  
Risk Factors ◽  
Substance Use ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Risk Factors ◽  
Cell Disease ◽  
Factors Associated ◽  
Clinical Risk ◽  
Observational Cohort

scholarly journals Genetic, laboratory and clinical risk factors in the development of overt ischemic stroke in children with sickle cell disease

2018 ◽  
Vol 40 (2) ◽  
pp. 166-181 ◽  
Author(s):  
André Rolim Belisário ◽  
Célia Maria Silva ◽  
Cibele Velloso-Rodrigues ◽  
Marcos Borato Viana
Keyword(s):  
Risk Factors ◽  
Ischemic Stroke ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Risk Factors ◽  
Cell Disease ◽  
Clinical Risk

scholarly journals Cumulative Incidences and Risk Factors for Intra and Extracranial Cerebral Arteriopathy in Sickle Cell Disease Children

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 56-57
Author(s):  
Francoise Bernaudin ◽  
Suzanne Verlhac ◽  
Cécile Arnaud ◽  
Annie Kamdem ◽  
Isabelle Hau ◽  
...  
Keyword(s):  
Risk Factors ◽  
At Risk ◽  
Risk Factor ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cumulative Incidence ◽  
Intracranial Stenosis ◽  
Cell Disease ◽  
Independent Risk Factors

In children with sickle cell disease (SCD), cerebral vasculopathy is responsible for overt strokes and silent cerebral infarcts (SCI). Transcranial Doppler (TCD) detects children at risk of strokes (intracranial time averaged mean of velocities (TAMV) ≥200cm/s). The extracranial portion of the internal carotid artery (eICA) can also be the site of stenosis or occlusion. eICA assessment requires cervical Doppler using a submandibular approach and cervical MRA (cMRA). We previously reported that eICA TAMV≥160cm/s are highly predictive of eICA stenosis and are a risk factor for SCI independently of acute and chronic anemia. However, the kinetics of eICA arteriopathy are unknown. The aim here was to evaluate and compare the cumulative incidence of intra/extracranial arteriopathy and associated risk factors in a longitudinal SCD cohort. Children born between 01/1988 and 01/ 2018, followed at our center at least until 06/2012 and up to 09/2019, annually assessed by TCD imaging and at least once by cervical Doppler were included, resulting in 493 SCD children (238F-255M) with 398 SCA (385SS,10Sb0,3SDPunjab), and 95 SC/Sb+ children (65SC,30Sb+). Alpha-genes, b-globin haplotypes, G6PD activity, CD36 expression were recorded. The average of baseline biologic parameters recorded between 1 and 3 years of age, a minimum of 3 months away from transfusion, 1 month from a painful episode, and before any intensive therapy was calculated. The median (range) follow-up of the overall cohort was 10.6 years (1.1-22.9), providing 5335 patient-years of follow-up. Six deaths occurred (5 SCA-children at 2, 4, 7, 19 & 20 years and 1 in SB+ patient at 13 years). Three SS patients had an ischemic stroke at 1.5, 3 and 4.3 years. Kaplan-Meier estimates of cumulative incidence (95%CI) are shown (Figure). In SCA-children, abnormal eICA TAMV and/or eICA stenosis were sometimes associated with abnormal intracranial TAMV and/or stenosis, but isolated eICA TAMV≥200cm/s or 160-199cm/s were observed in 19 (4.8%) and 28/398 (7.0%) patients, respectively, and isolated eICA stenoses in 33/294 (11.2%).Thus, risk factors were only analyzed in patients with isolated intra- or extracranial arteriopathy. COX regression analyses are shown (Table). For isolated intracranial TAMV≥200cm/s, multivariate analyses after introducing all significant genetic and biological risk factors retained the number of SEN b-haplotypes [HR=0.547 (95%CI:0.335-0.893); p=0.016], reticulocyte count&gt;400x109/L [HR=1.961 (95%CI:1.119-3.436); p=0.019], and WBC count&gt;20x109/L [HR=2.410 (95%CI:1.340-4.329); p=0.003] as independent risk factors. Isolated eICA TAMV≥160 cm/s were only strongly associated with the presence of tortuosities [HR=8.6 (95%CI :4.3-17.2); p&lt;0.001]. eICA tortuosities were present in 94/284 (33.1%) SCA vs 5/43 (11.6%) SC/Sb+ children (p=0.004), most often seen at the first cMRA but secondarily in 16 patients. Multivariate COX analysis retained genotype [HR/SCA vs SC/Sb+ = 3.6 (95%CI:1.4-9.4); p=0.010], low hemoglobin [HR=1.25 (95%CI:1.04-1.50); p=0.020], and high LDH [HR=1.002 (95%CI:1.001-1.002); p=0.001], as independent risk factors for eICA tortuosities. As expected, the risk of intracranial stenosis was significantly associated with isolated intracranial TAMV≥200 cm/s [HR (95%CI)=4 .255 (2.146-8.475); p&lt;0.001]. After adjustment with isolated intracranial TAMV≥200 cm/s, a-thalassemia, low hemoglobin, high WBC, MCV and LDH remained as significant, but not independent, risk factors for intracranial stenosis. The risk for eICA stenosis was only highly associated with the presence of tortuosities [HR=10.9 (95%CI:4.7-25.0); p&lt;0.001], or a history of eICA≥160cm/s [HR=15.4 (95%CI :7.5-31.2); p&lt;0.001]. This study reports eICA arteriopathy kinetics using a longitudinal cohort of SCD children systematically assessed by Doppler and cMRA. While we confirm that only SCA and not SC/Sb+ children are at risk of intra/extracranial arteriopathy, we show for the first time that extracranial arteriopathy progressively develops as early as 2 years old in SCA-children and reaches a plateau around 10 years of age, as for intracranial arteriopathy. Furthermore, eICA tortuosities, which are the risk factor for eICA arteriopathy, are themselves significantly and independently associated with the SCA genotype and the severity of hemolytic anemia. Figure Disclosures Bernaudin: BlueBirdBio: Consultancy; AddMedica: Honoraria, Other; GBT: Membership on an entity's Board of Directors or advisory committees. Verlhac:BlueBirdBio: Consultancy; AddMedica: Honoraria.

Abstract T MP110: Central Retinal Artery Occlusion in Patients with Sickle Cell Disease

Stroke ◽  
2015 ◽  
Vol 46 (suppl_1) ◽  
Author(s):  
Hashim Khan ◽  
Morad Chughtai ◽  
Ahmed A Malik ◽  
Adnan I Qureshi ◽  
Fareed K Suri
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Central Retinal Artery Occlusion ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Cell Disease ◽  
History Of ◽  
Retinal Artery

Objective: To determine the rate of and risk factors for incident central retinal artery occlusion (CRAO) among patients with sickle cell disease enrolled in a large cohort with longitudinal follow-up. Background: Sickle cell disease increases the risk of ischemic stroke among women but the risk of CRAO is not studied. Design/Methods: A total of 4085 patients from newborns to 77 years-old, were enrolled in Phase 1 of Cooperative Study of Sickle Cell Disease from 23 centers across the US. Participants underwent a baseline examination for assessment of demographics, prior medical history, lab assessments, and clinical data. Post baseline data included routine follow-up examinations, measures of organ damage, and collection of acute and chronic complications. The risk factors for CRAO were identified using Cox Proportional Hazards analysis. Results: A total of 9 (0.002%) of 4085 patients with sickle cell disease developed CRAO over a mean follow-up of 5.2 (95% CI 0.9 - 11.3) months, with an estimated incidence of 0.02 per 100 patient years. The incidence of CRAO was 0.03 per 100 patient years and 0.05 per 100 patient years in men and women, respectively. The history of previous stroke (2.6% versus 0.0%, p=0.7) and the proportion of patients diagnosed with large arterial occlusive disease (55.6% versus 7.7%, p=<0.001) was greater in those who developed CRAO compared to those who did not develop CRAO. History of exchange transfusions (2.7 % versus 22.2 %, p=0.02) and cigarette smoking (13.7 % versus 22.2 %, p =0.4) were more common among patients who developed CRAO. The hematocrit (%) (mean ± SD) was similar between patients who did or did not develop CRAO (29.5 ± 9.6 versus 27.3 ± 5.6, p=0.3). At completion of follow-up, 22.2% (n=2) patients with CRAO reported disability compared with 11.1% (n=455) patients without CRAO (p=0.7) Conclusions: The incidence of CRAO is relatively high among patients with sickle cell disease and was associated with disability.

scholarly journals Successful Transition from Paediatric to Adult Care in Sickle Cell Disease: An Experience in a Belgian Center

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 2495-2495
Author(s):  
Tracy Vandergraesen ◽  
Laurence Dedeken ◽  
Andre Efira ◽  
Phu Quoc Lê ◽  
Sophie Huybrechts ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Organ Damage ◽  
Transition System ◽  
Cell Disease ◽  
Adult Care ◽  
Walk Distance ◽  
The Us ◽  
Cardio Vascular

Abstract Introduction Despite reduction of mortality during childhood, young adults with SCD are at higher risk of morbidity and mortality after transition from paediatric to adult care, mainly in the US. To evaluate our transition system, we have studied the outcome of patients beyond the transition to adult care and looked if it was associated with an increase in vaso-occlusive crisis (VOC) and acute chest syndromes (ACS), the occurrence of progressive organ damage and changes in hydroxyurea prescription. Patients and Methods This study focus on sickle cell disease patients, born after January 1, 1980 and who have been transitioned from pediatrics (Hôpital Universitaire des Enfants Reine Fabiola, HUDERF) to adult care (CHU Brugmann), followed for minimum 2 years at HUDERF and having transitioned at least 2 years before December 2015. Demographic data, genotype, disease modify therapies, biological data, clinical data, acute clinical event, 6 minutes-walk test, cardiac echography) were collected retrospectively. Patients who underwent a hematopoietic stem cell transplantation were excluded from the analysis. Student test was used to analyse continuous variable and Wilcoxon regression for paired sample. Mc Nemar's test was used for comparison of paired nominal data and Chi-square test when data missed. Results The comparison of the data before and after transition is detailed in Table 1. Thirty eight patients (11 males) were included and made the transition at a median age of 20.1 years (range: 18.0-26.8). All patients were HbSS. The median age at diagnosis was 0.8 year (range: 0-7.95). The median duration of the follow-up before the transition was 15.95 years (range: 3.3 - 25.2) and after transition was 4.7 years (range: 2.2-5.8 years), accounting for 552.15 and 159.61 patient-years (PY) of follow-up respectively. Two patients died, 5.2 years and 0.6 year after the transition respectively. The death rate after transition was of 1.25/100 PY. Four women have delivered after a median gestation of 37.1 weeks (range: 35.9-38.7 weeks). The median new-born weight was 2.9 kg (range: 2.4-3.4 kg). The rate of disease modifying therapies remains stable after the transition. 33 patients were treated with hydroxyurea before transition compared to 35 after transition. Four patients were on chronic transfusion program before and 6 after transition. Hematological data were not statistically different at time of transition when compared to last follow-up, suggesting stable compliance to therapy but creatinine level increased significantly. VOC and ACS rate did not increased after transition as well as hospitalisation rate and days of hospitalisation. Tricuspid regurgitation velocity (TRV) increased significantly over the time but no patient developed pulmonary hypertension. The 6-minutes walk distance decreased significantly. Systolic and diastolic blood pressure increased significantly between the time of transition and the last follow-up. Regarding the educational status (8 data missing), 21 (70%) patients were able to reach graduate school and only 4 were not able to finish high school. Conclusion Our data provide useful information on the outcome during the years after transition. No increase in hospitalizations, VOC or ACS was observed. This is very different from the data provided by Blinder et al. One of the reason could be that comparing to the US situation, the Belgian Health Care System provide access and quality of healthcare even in underprivileged population. The prescription rate of HU remained unchanged and the compliance to treatment evaluated by hematological parameters remained stable. Nevertheless, changes in cardio-vascular parameters and creatinine levels suggest that despite the well-standardized follow-up for young adult patient with sickle cell disease, progressive organ damage leading to renal and cardio-vascular disease might not be prevented. However, compared to the literature, we have found less high level of TRV (9.7% vs. 24-45%), no patient with pulmonary hypertension (compared to 6-10%). In addition, our patients seems to have a better 6-minutes walk distance even in the childhood cohort (paediatrics data : 562m vs. 491m in Waltz study; adults data : 490m vs. 398m). Additional data from a larger number of patients as well as a longer period of follow-up are required to confirm the efficacy of our transition system. Disclosures No relevant conflicts of interest to declare.

Systematic Evaluation Of Chronic Organ Damage In Adult Sickle Cell Patients. A Seven-Year Follow-Up Study

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4683-4683
Author(s):  
Charlotte F.J. van Tuijn ◽  
Marein Schimmel ◽  
Eduard J. van Beers ◽  
Bart J. Biemond
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Outpatient Clinic ◽  
Organ Damage ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Avascular Osteonecrosis ◽  
Tertiary Teaching

Introduction The occurrence of organ damage in sickle cell disease (SCD) is a crucial determinant for both medical treatment and prognosis. In a previous study, we systematically analyzed the prevalence of SCD related organ damage and complications in adult sickle cell patients in a tertiary teaching hospital in the Netherlands. We now describe a seven-year follow-up of this patient cohort, to provide an insight into the course of the various forms of organ damage and SCD related complications. Methods At baseline in 2006, 110 adult patients visiting the outpatient clinic of our hospital were enrolled. All enrolled patients from the primary analysis were included for follow-up. Patients were screened for sickle cell related manifestations during visits to the outpatient clinic biannually. Various forms of sickle cell related organ damage and complications (presence of microalbuminuria, renal failure, pulmonary hypertension retinopathy, iron overload, cholelithiasis, avascular osteonecrosis, leg ulcers, acute chest syndrome, stroke, priapism and admissions for painful crises) were routinely screened according to international guidelines. Patients with genotype HbSS/HbSβ0 and HbSC/HbSβ+were grouped for further analysis. Results Of all originally included patients (N=110), nine were lost to follow-up (N=9). The mean age of the current study cohort is 37 years (IQR 27-46). Overall, 59 patients (58%) developed a new form of organ damage or new complication since baseline analysis, including eight patients who deceased (7 due to a sickle cell disease related death). In the HbSS/HbSβ0 genotype group (N=60) we found an increase in the percentage of patients who have had an Acute Chest Syndrome (29% to 47%) or have been diagnosed with avascular osteonecrosis (15% to 24%), retinopathy (23% to 34%) or pulmonary hypertension (31% to 48%). In the HbSC/HbSβ+ (N=35) group we found an increase in the occurrence of avascular osteonecrosis (9% to 14%), retinopathy (59 % to 70%) and pulmonary hypertension (9% to 19%). Furthermore, the use of hydroxycarbamide increased in both genotype groups and the frequency of admissions for painful crises remained stable for both genotype groups. Conclusion In the past period of seven years 58% of the patients in a previously well descript cohort of adult SCD patients developed a new sickle cell related complication. For some forms of organ damage or complications a substantial increase occurred dependent of a patient’s genotype. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Real-World Clinical Burden of Sickle Cell Disease in the US Community-Practice Setting: A Single-Center Experience from the Foundation for Sickle Cell Disease Research

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5856-5856
Author(s):  
Lanetta Bronté-Hall ◽  
Matthew Parkin ◽  
Courtney Green ◽  
Elsa Tchouambou ◽  
Lynn Huynh ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Real World ◽  
Research Funding ◽  
Organ Damage ◽  
Community Practice ◽  
Cell Disease ◽  
End Organ Damage ◽  
The Mean

Background Sickle cell disease (SCD) is a progressively debilitating monogenic disease characterized by unpredictable, acute, life-threatening episodes and chronic complications such as hemolytic anemia and end-organ damage. It presents with a range of severity resulting in significant morbidity, poor quality of life, and early mortality. Real-world data on treatments and clinical outcomes for patients (pts) with SCD are limited, particularly in the community clinical care setting. The objectives of this retrospective real-world study were to characterize clinical manifestations and management of pts with SCD treated at the Foundation for Sickle Cell Disease Research (FSCDR). Methods A retrospective longitudinal analysis of an electronic health records (EHR) database from the FSCDR, which captured laboratory testing, treatments, and records on outpatient, emergency, and inpatient visits, was conducted. All unique pts with SCD assigned a medical record number in the EHR (N=172) were considered. Data from external records were manually entered into the EHR to supplement the EHR database. To address limitations of a real-world database, medical records for all pts included were manually reviewed and validation was performed on 10% of the sample. Pt demographics, clinical characteristics, hydroxyurea (HU) treatment (as captured by prescriptions and usage notes), administration of red blood cell transfusions, vaso-occlusive crisis (VOC) events and acute chest syndrome (ACS) (based on physician assessment) were described. Annual VOC rates were summarized by dividing the number of events by the total follow-up duration in years. Results In total, 122 pts with SCD were included. Twenty-three pts were excluded as they were not actively seeking care and/or had no relevant clinical data from 01/01/15-07/19/19. Data for 27 pts <22 years of age were unavailable at time of analysis (results of this cohort will be included in the presentation). Among all pts, 76 (62.3%) were female and 118 (96.7%) were Black or African American. The mean age at time of analysis was 39.1 (standard deviation [SD] 12.3) years with 9 (7.4%) 22-24 year-olds, 67 (54.9%) 25-40 year-olds, 35 (28.7%) 41-55 year-olds, and 11 (9.0%) ≥ 56 year-olds. The most common genotypes were HbSS (77.0%) and HbSC (17.2%). Over a mean follow-up period of 2.9 (interquartile range: 2.0, 4.4) years, 27 (22.1%) pts were treated with HU and 66 (54.1%) pts received transfusions, of which 1 (1.5%) pt chronically received episodic transfusions (i.e., continuous monthly transfusions for ≥ 6 months). The mean total hemoglobin (Hb) was 8.3 (SD 1.7) for HbSS pts and 10.9 (SD 1.5) for HbSC pts. Among pts who never received HU or transfusions during follow-up, mean Hb was 9.4 (SD 1.7) for HbSS pts and 11.4 (SD 1.6) for HbSC pts. Among pts who ever received HU or transfusions, mean Hb was 7.7 (SD 1.3) for HbSS pts and 10.3 (SD 1.1) for HbSC pts. Eleven (9.0%) pts had ≥ 1 ACS whereas 97 (79.5%) pts had ≥ 1 VOC event. ACS and VOC events occurred mostly in pts 25-55 years of age (ACS: 9 [81.8%] pts aged 25-40 years and 2 [18.2%] pts aged 41-55 years; VOC: 54 [55.7%] pts aged 25-40 years and 29 [29.9%] pts aged 41-55 years). Annual rates of VOC are described in Figure 1. Conclusions This is one of the first studies to describe clinical characteristics and management of pts with SCD in a community practice setting. Higher Hb levels among pts who never vs ever received HU or transfusions during follow-up may be a reflection of who were selected for treatment and additional studies to take into account timing of treatment and Hb assessments and confounding factors need to be conducted. The study found high VOC rates particularly among pts aged ≤ 40 years. Lower VOC rates among older pts do not necessarily indicate less severe disease. One potential reason for lower VOC rates among older pts with SCD is that cumulative exposure to ischemia-related tissue injury and resulting end organ damage may decrease VOC-related pain over time. Further investigation to elucidate the rate of VOC decline observed in older pts is needed, including looking at underlying health and end organ damage. While limitations are inherent in real-world studies, these findings underlie the ability to and importance of studying SCD management and clinical outcomes in community care settings. This study highlights the clinical burden of SCD and possibly higher than expected unmet need in this community setting. Disclosures Bronté-Hall: bluebird bio: Research Funding. Huynh:bluebird bio: Research Funding. Puri-Sharma:bluebird bio: Employment. Chang:bluebird bio: Research Funding. Chawla:bluebird bio: Employment. Signorovitch:bluebird bio: Research Funding.

Hospital Readmission within 30 Days: A New Benchmark for Quality Care among Children with Sickle Cell Disease.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3403-3403
Author(s):  
Melissa J. Frei-Jones ◽  
Michael R. DeBaun
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
Quality Of Care ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Hospital Readmission ◽  
Cell Disease ◽  
First Admission

Abstract The National Association of Children’s Hospitals and Related Institutions (NACHRI) has established benchmarks for measuring quality of care in children that are hospitalized. Readmission within 30 days of discharge is the benchmark chosen to assess the quality of care for patients with Sickle Cell Disease (SCD). Limited data exist to determine risk factors for re-admission and whether such risk factors are modifiable. We performed a retrospective case-control study to identify risk factors for hospital readmission in children with SCD. All hospital admissions of patients with SCD for one year were reviewed. Cases were defined as children with SCD who were readmitted within 30 days of their first admission during the 12 month study period. Controls were defined as children with SCD who were not readmitted within 30 days of their first admission. A total of 30 cases and 70 controls were identified. The average time between admissions was 10.7 days with 50% readmitted within 8 days and 77% readmitted within 21 days. No difference in demographic data was found between cases and controls. The most common admission and readmission diagnosis was pain, 78% and 70%, respectively. The greatest risk factor for readmission was no follow-up appointment within 30 days after discharge in the SCD clinic (OR 7.7, 95% CI 2.4–24.4). The second highest risk factor was severity of disease, defined as patients with ≥ 3 hospitalizations in the previous 12 months versus patients with ≤ 2 hospitalizations in the previous 12 months (OR 7.3, 95% CI 2.8–18.9). A diagnosis of asthma was also a risk factor for readmission (OR 2.9, 95% CI 1.2–7.3). Patients who initially required supplemental oxygen to maintain their oxygen saturation in the normal range and were subsequently on room air for ≤ 24 hours at discharge were also more likely to be readmitted (OR 3.3, 95% CI 1.1–9.7). Steroid administration was not a risk factor for readmission (OR 1.2, 95% CT 0.5–3.2). Potential modifiable risk factors exist to decrease the rate of readmission. Specifically, strategies targeted at the modification of disease severity, aggressive management of asthma, and outpatient follow-up after hospitalization may decrease the 30 day readmission rate.

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