Clinical Benefit and Costs’ Evaluation of Erythocytapheresis Compared to Manual Exchange Transfusion for Children with Sickle Cell Disease: A Single Center Experience

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4091-4091
Author(s):  
Laurence Dedeken ◽  
Phu Quoc Lê ◽  
Laurence Rozen ◽  
Hanane El Kenz ◽  
Sophie Huybrechts ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Adverse Events ◽  
Sickle Cell ◽  
Median Duration ◽  
Exchange Transfusion ◽  
Biological Data ◽  
Acute Chest Syndrome ◽  
Continuous Control ◽  
Cell Disease ◽  
Previously Treated

Abstract Chronic transfusion in sickle cell disease (SCD) remains the gold standard therapy for primary and secondary stroke prevention and is indicated for patients with recurrent severe vaso occlusive crisis (VOC) or acute chest syndrome (ACS). Automatized apheresis (AA) has several advantages compared to manual exchange transfusion (MET): shorter procedure, continuous control of fluid balance, etc. The aim of our study was to assess the safety and efficacy of AA in SCD patients previously treated with MET at Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium and to evaluate the change of the costs related to transfusion and chelation overtime. From January 2012, the AA program for SCD patients started in our institution. Patients on chronic transfusion program (CTP) and previously treated with MET were eligible to switch to AA if sufficient peripheral venous access to allow AA without the use of central venous line and if weight ≥ 30kg. On CTP, target HbS was <30% in case of stroke risk and <50% for other indications. Data on biological values, duration of the procedure, intervals between procedures as well as adverse events were recorded for the last 6 months on MET and compared to the data on AA. The overall costs of last year on MET, 1st year and 2nd year on AA were analysed. The cost of packed red blood cell (RBC), one-day care facility, apheresis kit and chelation were recorded. For patients on AA for less than 2 years, costs for the 2nd year were extrapolated taking into account the data from the 6 previous months. Data were collected for this analysis until June 2014. Friedman test was used to compare treatment across years and Dunn's Multiple Comparison Test to compare each year of treatment among them. Ten patients switched from MET to AA at a median age of 11.8 years (range, 9.6-16.8y). The median duration of MET before start of AA was 1.9 years (range 0.5-4.4y). The median duration of AA was 1.7 years (range 1-2.4y). Four patients are on AA for > 2 years, 4 ≥ 20 months and 2 > than 12 months. Indications for CTP were overt stroke (2), pulmonary hypertension (2), recurrent VOC/ACS (5) and poorly tolerated severe anemia (1). All patients remained stable without any SCD related event, except one child who presented seizures without evidence of new stroke and for whom anticonvulsivant therapy was resumed. HbS level remained in the target values for all patients despite a slightly but significantly higher value on AA. The ferritin level and the duration of the procedure decreased significantly (Table 1). The 2 patients on iron chelation stopped it after 10 and 1 AA procedures. Interval between 2 AA was significantly longer than on MET (P<0.0001). On 181 procedures, 9 adverse events (4.9%) required medical intervention: transient hypotension (1), symptomatic hypocalcemia (2), transient headache (2), fever (1), nausea-vomiting (1) and abdominal pain (2). On AA, the requirement of packed RBC was significantly higher than on MET. During the 1st year, costs of AA were significantly higher than MET (132937€ vs.107560€; P=0.01). Nevertheless, during the 2nd year of treatment, the costs of AA were not significantly different from those on MET (102965€ vs. 107560€). Indeed chelation could be stopped in patients previously treated. AA is useful and safe for SCD patients requiring exchange transfusion program. It is less time consuming for nurses and patients, improves iron overload and interval between 2 procedures is significantly reduced. Despite higher costs related to the increase packed RBC requirement, the costs of AA and MET in the Belgian Health Care System are the same as chelation could be stopped in previously treated patients. Abstract 4091. Table 1.Changes in age, weight, biological data and procedure parameters on MET and on AA On METOn AAP value 1st year 2nd yearMedianRangeMedianRangeMedianRangeMedian age (years)11,89,7-16,812,810,8-17,713,311,8-18,6<0,0001Median weight (kg)45,530,4-66,349,933,8-72,05336,1-76,0<0,0001Median height (cm)153,5138-178161143-180165145-182<0,0001Hb (g/L)9,958,6-10,89,958,8-10,6109,2-11,7NSHb S (%)33,525-424028,5-424631-480,0002Ferritin (µg/L)666182-151225552-8111489-622<0,001Duration of procedure (min)245195-36087,375,5-1269164-1540,0002Interval between procedures (d)2821-293428-35,54228-42<0,0001Packed RBC requirement (ml/kg)18,315,1-2032,227,4-36,13026,8-36<0,0001Packed RBC requirement (unit)39,515-796749-12065,538-137<0,0001 Disclosures No relevant conflicts of interest to declare.

Adverse Events in Sickle Cell Patients with Nocturnal Hypoxia.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3793-3793
Author(s):  
Andrew D. Campbell ◽  
Megumi Okumura ◽  
Ndidi Unaka ◽  
Sally Hutchinson ◽  
Onyinye Onyekwere
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Adverse Events ◽  
Sickle Cell ◽  
Case Series ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Obstructive Sleep ◽  
The Relationship

Abstract The relationship between hemoglobin dexoygenation and sickling is well known. However, the relationship between hypoxia and severity of disease in sickle cell patients has not been well established. Recently, nocturnal hypoxemia has been associated with higher incidence of CNS events including strokes, and elevated TCDs. We present our case series on 13 patients(12 SS, 1 SC) with sickle cell disease (SCD) who have nocturnal hypoxia. Approximately 75 patients were screened at the University of Michigan Sickle Cell clinic for nocturnal hypoxia either by symptoms of obstructive sleep apnea or by longitudinal baseline clinic 02 saturations (02 Sat &lt;92%). Of the 13 hypoxic pts, median baseline O2 Sat 90%(n=13, mean 90) and the median nocturnal O2sat (Nctnl 02 sat) 84%(n=13, mean 80%) with 10/13 with moderate-severe nocturnal hypoxia (O2sats&lt;85%)based on sleep studies. Multiple adverse events noted in the cohort were pulmonary hypertension (PHTN TRJV&gt;2.5, n=9, median 2.74/mean 2.74,) frequent pain episodes(&gt;3visits to ER or hospitalizations/year, n=7, with 5 pts &gt;5/year ), recurrent acute chest syndrome( ≥ 3 episodes, n= 10), CNS events (n=3 silent infarcts, vascular stenosis), priapism( (n=4, among 6 males ). Also reported were possible causes of the underlying hypoxia including obstructive sleep apnea(OSA)(n=7 of 11 pts), asthma(n= 10 of 13 pts), and chronic lung disease( n=8). In conclusion, the persistence of nocturnal hypoxia in pediatric sickle cell disease could possibly contribute to the development of severe complications of sickle cell disease. Treatment of underlying hypoxia (ie nighttime oxygen, maximize asthma treatment, T&A for OSA)may help prevent complications and lead to the improvement clinical symptoms. Further, chronic nocturnal hypoxia may complicate pulmonary disease and accelerated the development of PHTN. More studies are needed to clarify the mechanism of hypoxia in SCD. Table I. Clinical &Demographic Data of 13 SCD Patients with Nocturnal Hypoxia. Age:(6–22y/o, mean 15) Sex: M=6 F=7 Clinical: Total Mild Mod Sev. Genotype: SS=12, SC=1 Mean Range Nctnl Hypoxia(&lt;%) 13 3 5(&lt;85) 5(&lt;80) Baseline O2 Sat(%) 90 +3.0 86–97 Obstr Sleep Apnea 7 3 3 1 Nctnl 02 sat (%) 80+8.4 59–87 Pulm HTN 9 4 4 1 Total #Apneic Events(11) 65.6+80 6–256 Rest. Lung Ds. 8 2 5 1 Obstr. Apneic Events(7) 27+68.5 0–221 # of Episodes &lt;3 3–4 &gt;4 Hypopneic Events(9) 32.5+38 0–132 ACS 2 5 5 TRJet Velocity 2.74+.42 2–3.5 Severe Pain Crises/yr 1 2 5

Red Blood Cell Exchange Transfusion, An Underutilized Effective and Reliable Therapeutic Modality in Sickle Cell Disease, Reduces Number of Admissions and Length of Hospital Stay

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4822-4822
Author(s):  
Aref Agheli ◽  
Kirshma Khemani ◽  
Madhumati Kalavar ◽  
William Steier ◽  
Zili He
Keyword(s):  
Sickle Cell Disease ◽  
Hospital Stay ◽  
Sickle Cell ◽  
Blood Viscosity ◽  
Exchange Transfusion ◽  
Hemoglobin Level ◽  
Therapeutic Modality ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
One Year

Abstract Background: The pathophysiology of sickle cell disease (SCD) is based on increased blood viscosity due to abnormal red blood cells (RBCs), which causes SCD complications, such as chronic hemolytic anemia, vaso-occlusive crisis with tissue hypoxemia, and organ dysfunction. Effective treatment of Sickle Cell Anemia is to reduce the blood concentration of Hemoglobin S (Hb S) RBCs. Exchange transfusion (ET) remains an effective but possibly underutilized therapy for the management of various acute and chronic complications of SCD such as acute chest syndrome, thromboembolic stroke, splenic and hepatic infarction, right upper quadrant syndrome, multi-organ failure syndrome, or in preparation for surgery by reducing HbS to less than 30%. RBC ET quickly replaces abnormal RBCs with normal RBCs, thus improving oxygen transport while reducing overall blood viscosity. Methodology: To determine the effectiveness of ET in SCD in reducing the total number of admissions and total in-hospital Length of Stay (LOS) in patients, admitted with any of acute complications of SCD, we retrospectively reviewed the medical records of 38 patients between June 15, 2007 and June 15, 2008. The eligibility criteria were age above 18 years old and admission to the hospital for any SCD complication. Nineteen patients had ET with Hb A containing RBCs, generally with an average packed RBC exchange volume of 70–80 ml/kg patient’s weight. Nineteen patients were treated with conventional managements. Three patients were excluded from the ET group because of prolonged LOS due to non-SCD- related complications. Four patients in the non-Exchange group signed against medical advice on the first day of admission and were excluded from analysis. Results: Sixteen (42.1%) patients were male and 22 (57.9%) patients were female. Their ages ranged from 19 to 67 years old, mean (SD) 30.2 (10.8). Of eligible patients, 19 (50%) patients received at least one therapeutic ET during the one year period of the study. In an independent-Samples T test analysis, the mean (SD) LOS were 7.5 (0.6) and 4.2 (0.6) days for the groups without ET and with ET respectively (95% CI = −5.2 to 1.5, p=.0011) (Figure 1). In this small studied group, this resulted average 3.3 days shorter in-hospital stay in ET group, could have saved 62 in-hospital days in the group who received conventional treatments. This number could have been easily much greater, since our hospital’s electronically stored data revealed that during year 2007, there had been 278 SCD admissions in all age groups. There was no mortality in the ET group, nor were any transfusion-related complications reported. In another analysis of one year follow up data, the number of admissions for the patients who never received ET ranged from 1 to 14, mean (SD) 1.7 (2.3) times in year 2007, while in patients who received at least one ET, the number of following admissions ranged from 0 to 2, mean (SD) 0.7 (1.3) times during the next year (95% CI = 0.16 to 1.7, p= .020). Hemoglobin level of patients in conventional treatment group on the day of discharge ranged from 6.5 to 10.7, mean (SD) 8.9 (1.9) and in ET group it ranged from 8.4 to 12.4, mean (SD) 10.2 (1.2) gr/dl (p= .045). Conclusion: Patients with SCD are frequently admitted to hospital for vaso-occlusive crisis and other complications. Exchange transfusion is a reliable, safe, and effective therapeutic modality in SCD patients, in particular during a catastrophic event. ET can significantly reduce the number of hospital admissions and in-hospital stay days in these patients. In addition, patients managed with ET have a better hemoglobin level on discharge. Figure Figure

scholarly journals Comparison between Automated Erythrocytopharesis (AECP) and Manual Exchange Transfusion (M-Ex)) in Reducing Hb-S and in Recovery of Acute Chest Syndrome and Other Acute Presentations of Sickle Cell Disease Patients

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4928-4928
Author(s):  
Said Yousuf Ahmed ◽  
Sameh M. Saleh ◽  
Mohamed Shefan Hameed ◽  
Ahmed M. Ragheb ◽  
Telal M. Abbas ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Pulmonary Artery ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Hemoglobin S ◽  
Artery Thrombosis ◽  
Hb S

Abstract Background: Sickle Cell Disease represents a national health problem in Saudi Arabia with close to 150 thousands of the population are afflicted with the disease. Patients are frequently admitted with life threatening complications like Acute chest syndrome (ACS), Hyperhemolysis (HH), pneumonia, thromboembolism including pulmonary Artery Thrombosis (PAT) or severe protracted painful vasoocclusive crises (VOC). Rapid lowering of Hemoglobin S helps in reducing sickling and in alleviating such complications and allowing rapid recovery. Exchange transfusion is the fastest way to remove pathogenetic sickling red blood cells and reducing Hb S level to a safe level. It can be done manually (Mex) or via Automated Erythrocytopharesis machine (AECP). In this study we compared manual exchange to AECP in achieving the targeted lowering of Hb S and in accelerating clinical recovery. Patients and Methods: Patients included are sickle cell disease patients (HbSS, HbSC, Hb S/thal) admitted to the ER of a central Hospital. Indications of exchange were: acute chest syndrome, acute severe painful vaso-occlusive crises refractory to standard ER protocol of analgesia, stroke, priapism, Hyperhemolysis, and acute pulmonary embolism. P value of significance was calculated using student t-test comparing between median Hb S achieved after manual exchange vs AECP. To assess the rapidity of reversal of desaturation in acute chest syndrome patients, the cumulative incidence of reversal of desaturation and normalization of Oxygen saturation on room air were plotted against time at 0 time of the start of exchange, 2 hours,4 , 12, 24, 48 and 72 hours/discharge (D/C) Results: Table 1 shows clinic-biological characteristics of patients who underwent exchange transfusion. A total of 230 patients-admissions were registered between Dec 1. 2017 to July 27, 2018 for painful VOC to ER; 51 (32%) had clinical indications for exchange (ACS 25, Stroke / fits 1, priapism 1, pulmonary artery thrombosis 1, Hyperhemolysis with VOC (n:7) , VOC with HLH (n:1), and the remaining with "refractory" painful VOC with or without infection. One patient died immediately at the time of arrival to ER before starting any standard resuscitative measures . Exchange transfusion was indicated and done for 53 (23%); 12 (22,6%) AECP and the remaining (77.4%) had Manual Exchange. The median Hb S after manual exchange was 44 % (range 31-74%) which was unsatisfactory and way higher than the targeted level while Automated ECP reached down satisfactorily to a median of 31%(range 8%-50%) ; 67% of whom achieved it with only one session. No mortalities or major procedure related complication reported with manual or automated ECP. Procedurally, 3 patients needed 2 automated sessions and 1 patient used 2 kits for one session. Manual exchange could not achieve the target Hemoglobin S level below or around 30% due to logistic and technical difficulties and sometimes patients' refusal while Automated ECP reached to a mean Hb s level of 28%( range 8%-50%) and nearly two thirds (67%) reached to as low as 31% Hb S level with only one session of Automated ECP and was associated with rapid improvement of the oxygenation within the first 2 hours of the procedure. Conclusions: Erythrocytopharesis (Automated RBC exchange) is effective, quick and safe procedure that is life saving for many patients with ACS and is associated with less difficulties and complications if compared with the manual exchange. Because SCA is a national problem in Saudi Arabia and acute chest syndrome and other acute major complications comprehensively kill SCD patients, Automated Erythocytopharesis should be available nation-wide like dialysis machines at all large hospitals in all cities and should be distributed according to the prevalence of SCA in the area or location. Disclosures No relevant conflicts of interest to declare.

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.

Erythrapheresis Improves Blood Viscosity Compared to Manuel Procedure in Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 2449-2449 ◽  
Author(s):  
Ait Abdallah Nassim ◽  
Connes Philippe ◽  
Di Liberto Gaetana ◽  
Offredo Lucile ◽  
Ranque Brigitte ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Blood Viscosity ◽  
Exchange Transfusion ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Blood Exchange ◽  
Genotype 2 ◽  
Blood Exchange Transfusion ◽  
The Impact

Abstract Blood transfusion is a cornerstone of the treatment in sickle cell disease (SCD). Guidelines and clinical trials indicate their use in several acute and chronic SCD manifestations such as cerebral vasculopathy prevention and acute chest syndrome. Two methods of blood exchange transfusion are available for physicians and patients: manual exchange transfusion (MET) and erythrapheresis, i.e. an automated exchange transfusion (AET). MET consists in a phlebotomy followed by a transfusion while erythrapheresis corresponds to the replacement of only sickle RBCs by healthy RBCs by controlling hematocrit. To our knowledge, no study has compared the impact of these two methods on blood viscosity and the ratio of hematocrit to blood viscosity (HVR); i.e., an index of red blood cell oxygen transport effectiveness (Alexy et al, 2006). Herein we aimed to compare those two procedures in term of biological parameters and blood viscosity, in order to offer new physiological parameters to guide the therapeutic management of SCD patients. This prospective, monocenter, observational study included sickle cell patients, ≥18 years old, treated by Blood Exchange Transfusion (BET) in our university hospital's Adult Sickle-Cell Referral Center. The primary end point was the change in blood viscosity during the BET procedure. Secondary end-points included the change in HVR, blood viscosity and HVR at the end of the procedure. Blood viscosity was measured after full oxygenation of the blood, at native hematocrit and at a shear rate of 225 s-1using a cone/plate viscometer (Brookfield DVII+ with CPE40 spindle, Brookfield Engineering Labs, Natick, MA, USA) (Baskurt et al, 2009). This study was approved by the local Institutional Review Board. All patients gave their signed informed consent for the genetic studies in accordance with the Declaration of Helsinki. All data were rendered anonymous to protect patients' privacy and confidentiality. Twelve patients in AET group and 31 patients in MET group were included. Thirty-nine patients had a SS genotype, three patients had a S-β0 thalassemia genotype (2 AET, 1 MET) and one had a S-β+thalassemia genotype (AET). The proportion of hydroxyurea-treated patients was not different between the two groups (20/31 in MET group and 5/12 in the AET group; p=0.17). The BET indication was cerebral vasculopathy in 11/12 and vaso-occlusive crisis in 1/12 in the AET group. BET indications in the MET group were: frequent vaso-occlusive crisis (10/31), severe organ dysfunction or organ transplant (12/31), provisory hydroxyurea interruption due to pregnancy, breastfeeding, paternity desire (4/31) and leg ulcers (3/31). Differences between groups before BET were only a higher percentage of HbF in the MET group and a higher percentage of HbA in the AET group. Both AET and MET procedures decreased HbS level, leucocytes and platelets counts, and increased HbA level (p ranging from < 0.01 to < 0.001). The decrease in HbS (p < 0.001), HbF (p < 0.05), HbA2 (p < 0.05), leucocytes (p < 0.001) and platelets (p < 0.001) levels was higher in the AET than in the MET condition. MET caused a significant rise in hematocrit and hemoglobin (p < 0.001). In contrast, AET did not change hematocrit and induced a slight increase in hemoglobin (p < 0.05). The percentage of change in hemoglobin and hematocrit was higher in the MET than in the AET condition (p < 0.01 and p < 0.05, respectively). The median blood viscosity after AET was significantly lower (3.77 cP [3.78-4.25]) compared to before (4.47 cP [3.88-5.22 ]; (p=0.0001)), whereas there was no difference before (4 cP [3.7-4.25]) and after (4.15 cP [3.73-4.88]) BET in the MET group (p=0.11). The percentage of variation in blood viscosity between AET and MET was significantly different (p < 0.01). Both AET and MET significantly increased HVR (p < 0.001 and p < 0.01, respectively) and the percentage of variation was not significantly different between the two procedures (p = 0.138). Conclusion: Automated exchange transfusion and not manual exchange transfusion improved blood viscosity. This might be due to the control of hematocrit allowed by the automated procedure, while decreasing the HbS percentage. Automated exchange transfusion should be preferred in acute and chronic SCD manifestations to improve blood rheology. Disclosures No relevant conflicts of interest to declare.

The Outcome Of Preoperative Transfusion Guideline On Sickle Cell Disease Patients At King Fahd Hospital-Jeddah (Ksa)

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S111-S111
Author(s):  
S M Felemban
Keyword(s):  
Postoperative Complications ◽  
Sickle Cell Disease ◽  
Hospital Stay ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Length Of Hospital Stay ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Postoperative Fever ◽  
Significant Difference

Abstract Introduction/Objective We have developed a local hospital transfusion guidelines to reduce and prevent the perioperative and the postoperative complications. This study was conducted to evaluate the outcome of practice for preoperative transfusion therapy in SCD patients in our institution. Methods A retrospective review of SCD patients undergoing surgery at King Fahd Hospital, Jeddah, Saudi Arabia was conducted between April 2005 and May 2010. The medical records were reviewed to define the perioperative risks and the postoperative complications in relation to the type of transfusion modality selected. Results The medical record of 75 sickle cell disease patients in whom the hematologists were consulted for their preoperative assessment. Preoperatively, 25.3 % had complete exchange transfusion (CETX), 17.3 % had partial exchange transfusion (PETX), 26.7 % had simple top up transfusion (STX) and 30.7 % had no transfusion (NTX). The postoperative complications were 20% vaso-occlusive crises (VOC), 2.7% acute chest syndrome (ACS), and 16% had fever. The mean duration for the hospital stay was 7.36 with SD of 5.83. There was no significant difference in the outcome between all types of transfusion modalities, the P value was (p 0.245), (p 0.282), (p 0.133), (p 0.220) for postoperative fever, VOC, ACS, and the length of hospital stay, respectively. However, The correlation was highly significant between the post-transfusion haemoglobin (Hb) level and the occurrence of postoperative fever (p 0.01) and VOC (p 0.03). Interestingly, SCD patients who received hydroxyurea were observed to have less postoperative complication like fever, and the result was highly significant (P&lt;0.01), while those who received prophylactic heparin in the postoperative period were found to have a reduced length of hospital stay (p&lt;0.01) and vaso-occlusive crises (p &lt;0.01). Conclusion The guidelines for preoperative transfusion in SCD patients was effective in reducing the postoperative morbidity and mortality. However, the selection of the optimum regimen for different surgical types in sickle cell disease patients and the surgical situations where preoperative transfusion is needed were all established in this guidelines.

scholarly journals Prediction of adverse outcomes in children with sickle cell anemia: a study of the Dallas Newborn Cohort

Blood ◽  
2008 ◽  
Vol 111 (2) ◽  
pp. 544-548 ◽  
Author(s):  
Charles T. Quinn ◽  
Nancy J. Lee ◽  
Elizabeth P. Shull ◽  
Naveed Ahmad ◽  
Zora R. Rogers ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Adverse Events ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Adverse Outcomes ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Clinical Predictors ◽  
Very Young Children

The Cooperative Study of Sickle Cell Disease reported that dactylitis, severe anemia, and leukocytosis in very young children with sickle cell disease (SCD) increased the risk of later adverse outcomes, including death, stroke, frequent pain, and recurrent acute chest syndrome. This model has not been validated in other cohorts. We evaluated its performance in the Dallas Newborn Cohort, a newborn inception cohort of children with SCD. We studied 168 children (55% male, 97% sickle cell anemia) with a mean follow-up of 7.1 years who provided 1188 patient-years of observation. Of the 23 (13.7%) subjects who experienced adverse events, 2 (1.2%) died, 14 (8.3%) had a stroke, 4 (2.4%) had frequent pain, and 3 (1.8%) had recurrent acute chest syndrome. No relationship existed between early clinical predictors and later adverse outcomes, with the possible exception of leukocyte count. Most subjects who experienced adverse events were predicted to be at low risk for those events. No subject who was predicted to be at high risk actually experienced an adverse outcome. The sensitivity of the model did not rise above 20% until specificity fell below 60%. We suggest that this model not be used as a criterion to initiate early interventions for SCD.

Hemoglobin Target and Transfusion Modality for Adult Patients With Sickle Cell Disease Acute Chest Syndrome

2020 ◽  
pp. 088506662097877
Author(s):  
Joseph L. Simonson ◽  
Juliana A. Rosentsveyg ◽  
Noah G. Schwartz ◽  
Abhinav Agrawal ◽  
Seth Koenig ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Tertiary Care ◽  
Hospital Length ◽  
Hospital Length Of Stay ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Icu Transfer ◽  
Median Hospital

Background: Despite the importance of transfusion in treating sickle cell disease acute chest syndrome, the target hemoglobin and optimal modality for transfusion remain unknown. Objectives: To compare hospital length of stay (LOS) in intensive care unit (ICU) patients with acute chest syndrome transfused to hemoglobin ≥ 8 g/dL versus patients transfused to hemoglobin < 8 g/dL; and to compare hospital LOS in acute chest syndrome patients treated with and without exchange transfusion. Methods: We performed a retrospective cohort study of all acute chest syndrome patients treated in the medical ICU at 2 tertiary care hospitals between January 2011 and August 2016 ( n = 82). We compared median hospital LOS in patients transfused to hemoglobin ≥ 8 g/dL by the time of ICU transfer to the medical floor versus patients transfused to hemoglobin < 8 g/dL as well as patients who received exchange transfusion versus no exchange transfusion using Wilcoxon rank-sum tests. We modeled the association between hospital LOS and hemoglobin at ICU transfer to the medical floor using multivariable log-linear regression. Results: Median hospital LOS was about half as long for patients transfused to hemoglobin ≥ 8 g/dL versus hemoglobin < 8 g/dL (8.0 versus 16.5 days, P = 0.008). There was no difference in LOS for patients treated with and without exchange transfusion. On average, a 1 g/dL increase in hemoglobin was associated with a 19.5% decrease (95% CI 10.8-28.2%) in LOS, controlling for possible confounding factors. Conclusions: Transfusion to a hemoglobin target ≥ 8 g/dL is associated with decreased hospital LOS in patients with acute chest syndrome. There was no difference in LOS between patients who received exchange transfusion and those who did not.

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