Abstract TP402: Sickle Cell Disease in Pediatric Stroke - Inpatient Resource Utilization in the Decade Following STOP

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
J. Michael Taylor ◽  
Paul Horn ◽  
Heidi Sucharew ◽  
Todd A Abruzzo ◽  
Jane Khoury
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Care Utilization ◽  
Cell Disease ◽  
Common Procedure ◽  
Transfusion Therapy ◽  
Stroke Population ◽  
Clinical Classification Software ◽  
Inpatient Database

Background: Sickle cell disease (SCD) is an important risk factor for stroke in children. Natural history studies demonstrate that greater than 10% of hemoglobin SS patients suffered ischemic stroke prior to age 20 years. In 1998, the Stroke Prevention Trial in Sickle Cell Anemia (STOP) successfully demonstrated the role for routine transfusion therapy in reducing stroke in at risk SCD patients. Fullerton and colleagues then found that first time stroke in SCD decreased in Californian children in the 2 years following STOP. We investigated the stroke rate and health care utilization of children with SCD for two calendar years in the decade following publication of the STOP trial using a national inpatient database. Methods: The 2000 and 2009 Kids’ Inpatient Database (KID) were used for analysis. SCD and stroke cases were identified by ICD-9 codes 282.6x, 430, 431, 432.9, 434.X1, 434.9, 435.9. We queried the KID procedural clinical classification software for utilization of services pertinent to SCD and stroke; transfusion, MRI, and cerebral angio. Results: In 2000, SCD was a discharge diagnosis in 34,294 children and 158 (0.46%) children had SCD and stroke. By 2009, discharges with SCD rose to 37,082 children with 212 (0.57%) children carrying both diagnoses. In 2000 and 2009, AIS is the most common stroke type at 83%, males account for 53% of stroke and black race was reported by 92% of SCD and stroke subjects. Procedure utilization is higher in the SCD and stroke population than in SCD without stroke (Figure 1). Blood transfusion is the most common procedure in both study years, significantly higher in stroke subjects. Conclusion: For pediatric inpatients with SCD, blood transfusion and diagnostic cerebrovascular procedures were significantly more common in the cohort with comorbid stroke. In the decade after STOP, children hospitalized with SCD and stroke represented less than 0.6% of the total inpatient SCD population.

Retinal microvascular analysis using Fundus Fluorescein Angiography in Egyptian sickle cell disease patients

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Nayera H El Sherif ◽  
Mahmoud A Kenny ◽  
Waheed S Elhalfawy
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Fluorescein Angiography ◽  
Sickle Cell ◽  
Large Sample Size ◽  
Cell Disease ◽  
Fundus Fluorescein Angiography ◽  
Transfusion Therapy ◽  
Hydroxyurea Treatment ◽  
Trans Cranial Doppler

Abstract Background Sickle cell disease can affect retina of eye via vaso-occulsive changes that occur in micro-vessels of retina which could be analysed by using Fundus Fluorescein Angiography. Aim To analyze macular microvascular alternation in patients with SCD by Fundus Fluorescein Angiography (FFA) and to assess the role of potentially contributory Clinico-pathological factors including Trans-Cranial Doppler, genotypes, hydroxyurea, transfusion therapy and finally iron overload state on the development of macular alterations. Method This was across-sectional study which included 30 Sickle cell disease patients randomly recruited from the Paediatric Haematology clinic, children Hospital, Ain Shams University, Cairo, Egypt. Complete blood count (CBC), Trans-Cranial Doppler (TCD) and Fundus Fluorescein Angiography. Results In our study, there were 30 patients with mean age (14.1± 4.02), 5 patients had abnormal/conditional Trans-Cranial, 15 patients had Vaso-occlusive crises, 11 patients were on regular simple blood transfusion; all 30 studied sickle cell disease patients had normal Fundus Fluorescein Angiography and eye examination and only one patient hadabnormal visual acuity;A 29 years oldgirl who had five attacks of cerebral strokes last year, on regular simple blood transfusion and Hydroxyurea treatment with abnormal TCD and recurrent Vaso-occlusive crises in last two years, Although her vision is hand movement yet Fundus Fluorescein Angiography was normal. Conclusion we didn’t find any Retinal microvascular alternation in our studied SCD patients using Fundus Fluorescein Angiography, we related our results to the fact that our studied SCD patients were young and all our studied patients were on hydroxyurea therapy with fair compliance, further studies using large sample size are warranted in order to illustrate the utility of Fundus Fluorescein Angiography (FFA) as a tool for better detection of sickle retinopathy.

scholarly journals How I treat and manage strokes in sickle cell disease

Blood ◽  
2015 ◽  
Vol 125 (22) ◽  
pp. 3401-3410 ◽  
Author(s):  
Adetola A. Kassim ◽  
Najibah A. Galadanci ◽  
Sumit Pruthi ◽  
Michael R. DeBaun
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Management Strategies ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Hematopoietic Stem ◽  
Neuro Imaging

Abstract Neurologic complications are a major cause of morbidity and mortality in sickle cell disease (SCD). In children with sickle cell anemia, routine use of transcranial Doppler screening, coupled with regular blood transfusion therapy, has decreased the prevalence of overt stroke from ∼11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with SCD and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurologic deficit includes evaluation by a multidisciplinary team (a hematologist, neurologist, neuroradiologist, and transfusion medicine specialist); prompt neuro-imaging and an initial blood transfusion (simple followed immediately by an exchange transfusion or only exchange transfusion) is recommended if the hemoglobin is >4 gm/dL and <10 gm/dL. Standard therapy for secondary prevention of strokes and silent cerebral infarcts includes regular blood transfusion therapy and in selected cases, hematopoietic stem cell transplantation. A critical component of the medical care following an infarct is cognitive and physical rehabilitation. We will discuss our strategy of acute and long-term management of strokes in SCD.

Chronic Blood Transfusion Therapy Practices to Treat Strokes in Children with Sickle Cell Disease

2005 ◽  
Vol 17 (7) ◽  
pp. 277-282 ◽  
Author(s):  
Terianne Lindsey ◽  
Nutrena Watts-Tate ◽  
Elaine Southwood ◽  
Julie Routhieaux ◽  
Janice Beatty ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Cell Disease ◽  
Transfusion Therapy

scholarly journals Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease

Blood ◽  
2011 ◽  
Vol 117 (3) ◽  
pp. 772-779 ◽  
Author(s):  
Monica L. Hulbert ◽  
Robert C. McKinstry ◽  
JoAnne L. Lacey ◽  
Christopher J. Moran ◽  
Julie A. Panepinto ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Stroke Prevention ◽  
Secondary Stroke Prevention ◽  
Cell Disease ◽  
Cerebral Infarcts ◽  
Eligibility Criteria ◽  
Transfusion Therapy

Abstract Children with sickle cell disease (SCD) and strokes receive blood transfusion therapy for secondary stroke prevention; despite this, approximately 20% experience second overt strokes. Given this rate of second overt strokes and the clinical significance of silent cerebral infarcts, we tested the hypothesis that silent cerebral infarcts occur among children with SCD being transfused for secondary stroke prevention. A prospective cohort enrolled children with SCD and overt strokes at 7 academic centers. Magnetic resonance imaging and magnetic resonance angiography of the brain were scheduled approximately every 1 to 2 years; studies were reviewed by a panel of neuroradiologists. Eligibility criteria included regularly scheduled blood transfusion therapy. Forty children were included; mean pretransfusion hemoglobin S concentration was 29%. Progressive cerebral infarcts occurred in 45% (18 of 40 children) while receiving chronic blood transfusion therapy; 7 had second overt strokes and 11 had new silent cerebral infarcts. Worsening cerebral vasculopathy was associated with new cerebral infarction (overt or silent; relative risk = 12.7; 95% confidence interval, 2.65-60.5, P = .001). Children with SCD and overt strokes receiving regular blood transfusion therapy experience silent cerebral infarcts at a higher rate than previously recognized. Additional therapies are needed for secondary stroke prevention in children with SCD.

Occupational History for Forty Adults with Sickle Cell Disease Compared with Healthy Siblings

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4937-4937
Author(s):  
Modupe Idowu ◽  
Solomon Badejoko ◽  
Paul Rowan ◽  
Harinder S. Juneja
Keyword(s):  
Sickle Cell Disease ◽  
Job Performance ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Care Utilization ◽  
Future Research ◽  
Cell Disease ◽  
Occupational History ◽  
Transfusion Therapy ◽  
Psychosocial Support System

Abstract Introduction: Despite high rates of unemployment and disability in adults with sickle cell disease (SCD), these issues remain understudied. Since SCD mostly affect people of African descent, the potential risk factors for poor job performance and unemployment in adults with SCD are a complex combination of disease, demographic, and socio-economic variables. Recurrent acute vaso-occlusive crises and organ dysfunction related to SCD can limit employment options and interfere with job retention. Potential associates of unemployment and poor job performance in adults with SCD include health-related (pain frequency and intensity, health-care utilization) and psychosocial (support system, coping mechanism). The goal of this study is to compare patients’ rates of unemployment and disability with those of their unaffected siblings. We compare patients with siblings so as to adjust for some socioeconomic variables. Patients and Methods: Patients on chronic transfusion therapy and those with additional disabling conditions were excluded. Surveys were administered during routine clinic visits. Along with demographic data, the survey also assessed use of hydroxyurea, employment status, length of employment, annual income, and job satisfaction. Moreover, patients were asked to provide the same occupational data for a sibling without SCD and whose age was within five years of the patient’s age. Clinical data including number of hospitalizations in the recent year were also gathered from the patient’s medical record. Results: A cohort of 40 adult SCD patients (28 hemoglobin SS, 8 hemoglobin SC, 1 Sβ0 and 3 Sβ+; age: median = 29, range 19-56 years; sex: 20 males, 20 females) completed surveys concerning occupational history for themselves and a sibling. Of the patients, twenty-three out of forty (57.5%) were on hydroxyurea therapy and 24/40 (60%) had 3 or more hospitalizations in the previous one year. Ten out of forty SCD patients (25%) are currently employed, compared to 26/40 (65%) of their siblings (p < .001; all tests reported are McNemar’s Exact (binomial) test). Five out of forty SCD patients (12.5%) have been employed for more than 2 years as compared to 20/40 (50%) of their siblings (p <.001). Twenty-six (65%) of SCD patients are on disability, while this is true for only four (10%) of the siblings (p< .001). It is important to note that 20 out of 26 (77%) SCD patients who are currently on disability have previously had a job. Fifteen of the twenty-six (58%) SCD patients on disability reported that they missed more than 1 day per week of work due to their SCD. Of the unemployed patients, twenty-three out of 30 (77%) wished they had a job. Conclusions: In this sample, patients with SCD have notably lower rates of current employment and sustained employment when compared with similar-age siblings without SCD.Despite the fact that 32/40 (80%) of the SCD patients have previously had a job, only 25% are currently employed. Our future research studies will focus on development of specific interventions to improve job accommodation of SCD patients. Disclosures No relevant conflicts of interest to declare.

Blood transfusion therapy is feasible in a clinical trial setting in children with sickle cell disease and silent cerebral infarcts

2008 ◽  
Vol 50 (3) ◽  
pp. 599-602 ◽  
Author(s):  
Allison A. King ◽  
Michael Noetzel ◽  
Desirée A. White ◽  
Robert C. McKinstry ◽  
Michael R. DeBaun
Keyword(s):  
Clinical Trial ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Cell Disease ◽  
Cerebral Infarcts ◽  
Transfusion Therapy ◽  
Clinical Trial Setting ◽  
Trial Setting

scholarly journals Stroke in patients with sickle cell disease: clinical and neurological aspects

2008 ◽  
Vol 66 (1) ◽  
pp. 30-33 ◽  
Author(s):  
Carolina Camargo de Oliveira ◽  
Sylvia Maria Ciasca ◽  
M. Valeriana L. Moura-Ribeiro
Keyword(s):  
Ischemic Stroke ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Cerebral Hemispheres ◽  
Cerebrovascular Event ◽  
Subcortical Structure ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Neuropsychological Impairments

The aim of this study was to characterize a group of patients (n=8) with sickle cell disease (SCD) and ischemic stroke concerning the clinical, neurological, imaging and progressive aspects. Data were collected from records and completed with an interview of patients and their parents. In this study there were 8 patients with ages ranging from 10 to 23 years old; SCD diagnosis was given between one and two years of age with clinical features of fatigue and anemia. The stroke was ischemic in all individuals and the first cerebrovascular event occurred before 6 years of age; 3 patients had recurrence of stroke despite prophylactic blood transfusion therapy and both cerebral hemispheres were affected in 4 patients. Clinical and neurological current features observed were: acute pain crises, sialorrhea, mouth breathing, motor, and neuropsychological impairments resulting from cortical-subcortical structure lesions.

Encephaloduroarteriosynangiosis (EDAS) For Patients With Sickle Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 999-999
Author(s):  
Jeffrey D. Lebensburger ◽  
Christina J. Bemrich-Stolz ◽  
Christoph Griessenauer ◽  
Lee Hilliard ◽  
Thomas H. Howard ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Stroke Prevention ◽  
Case Series ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Indirect Revascularization

Abstract Introduction Regular blood transfusion therapy is the standard care for secondary prevention of strokes in sickle cell disease (SCD). Despite regular blood transfusion therapy approximately 45% of the children with strokes will have progressive neurological disease (overt strokes or new silent cerebral infarcts) with an incidence of overt strokes of 3.2 events/100 patient-years (95% confidence interval, 1.3-6.5) (Hulbert, Blood 2011). Limited additional therapeutic options exist for these patients. Encephaloduroarteriosynangiosis (EDAS) is a neurosurgical procedure to improve cerebral blood flow by transposing scalp arteries onto the surface of the brain. Five previously published series reported a total of 41 EDAS or indirect revascularization procedures on patients with SCD aged 3-22 yrs. Three of 41 patients (7%) had a stroke at 24 hours, 5 days and three weeks following EDAS/indirect revascularization. Additionally, one patient developed TIA 12 months later, two patients developed intracranial hemorrhage, and one patient died from a pulmonary embolus during an episode of acute chest syndrome 48 months post-EDAS. To date, the incidence of complications and efficacy of EDAS procedure in stroke prevention has not been well established. Objectives To examine the incidence of overt stroke pre and post-EDAS for patients on chronic transfusion. Methods We studied a pediatric cohort with history of HbSS and SB0 thalassemia on chronic transfusion for CNS injury who underwent EDAS at the University of Alabama at Birmingham. The incidence of overt stroke pre- and post-EDAS was reviewed. All pre-transfusion hemoglobin and percent Hemoglobin S levels were recorded from the time of their first recorded abnormal MRI. To determine the acute complications of EDAS, we reviewed the peri-operative hospital records at the time of EDAS, post-EDAS emergency room visits and chronic transfusion clinic visits post EDAS. Results A total of 13 patients on chronic transfusion for secondary stroke prevention underwent 17 EDAS procedures for recurrent stroke, progressive vascular disease, or neurologic change including psychosis and decline in neuropsychometric scores. The mean time to EDAS was 80 months (median 56) from their first abnormal MRI in the medical records. The patients’ mean pre-transfusion hemoglobin level was 9.4 g/dL and mean HbS was 29.5%. All participants (n=13) maintained a mean pre-transfusion HbS < 40%; 62% (8/13) participants maintained a mean HbS <30% (two patients with elevated HbS were transitioned to hydroxyurea as part of a clinical trial). Prior to EDAS, three patients had a new overt stroke during 81 patient years.(3.7 strokes per 100 pt yrs) One of 17 EDAS procedures was complicated by an acute stroke one month after the procedure. No additional strokes occurred in these patients during 34 patient years.(3 strokes per 100 pt yrs) One child developed a chronic subdural hematoma one month post-EDAS requiring burr hole drainage; this patient had a complete recovery. Conclusion This case series represents the largest cohort of EDAS procedures for children with SCD, and in combination with the literature, suggests that patients with progressive CNS disease may benefit from EDAS. A multicenter retrospective case series should be completed to identify risk factors for progression status post an EDAS procedure followed by a clinical trial to determine the effectiveness of the procedure versus regular blood transfusion therapy. Disclosures: No relevant conflicts of interest to declare.

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