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.

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 Beneficial effects of adenotonsillectomy in children with sickle cell disease

2020 ◽  
Vol 6 (4) ◽  
pp. 00071-2020
Author(s):  
Ilaria Liguoro ◽  
Michele Arigliani ◽  
Bethany Singh ◽  
Lisa Van Geyzel ◽  
Subarna Chakravorty ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Arterial Oxygen Saturation ◽  
Arterial Oxygen ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Beneficial Effects ◽  
Admission Rates ◽  
The Mean ◽  
The Impact

Tonsillectomy and adenoidectomy (T&A) is frequently performed in children with sickle cell disease (SCD). Our aim was to evaluate the impact of this surgery on overnight oxygenation and rates of complications in these patients.Children with SCD who underwent T&A between 2008 and 2014 in two tertiary hospitals were retrospectively evaluated. Overnight oximetry and admission rates due to vaso-occlusive pain episodes (VOEs) and acute chest syndrome (ACS) in the year preceding and following the surgery were compared.19 patients (10 males, 53%) with a median age of 6 years (range 3.5–8) were included. A significant increase of mean overnight arterial oxygen saturation measured by pulse oximetry (SpO2) (from 93±3.6% to 95.3±2.8%, p=0.001), nadir SpO2 (from 83.0±7.1% to 88±4.1%, p=0.004) and a reduction of 3% oxygen desaturation index (from a median value of 5.7 to 1.8, p=0.003) were shown. The mean annual rate of ACS decreased from 0.6±1.22 to 0.1±0.2 events per patient-year (p=0.003), while the mean cumulative rate of hospitalisations for all causes and the incidence of VOEs were not affected.T&A improved nocturnal oxygenation and was also associated with a reduction in the incidence of ACS at 1-year follow-up after surgery.

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.

The Impact of Recurrent Acute Chest Syndrome on the Lung Function of Young Adults with Sickle Cell Disease

Lung ◽  
2010 ◽  
Vol 188 (6) ◽  
pp. 499-504 ◽  
Author(s):  
Jennifer M. Knight-Madden ◽  
Terrence S. Forrester ◽  
Norma A. Lewis ◽  
Anne Greenough
Keyword(s):  
Young Adults ◽  
Sickle Cell Disease ◽  
Lung Function ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
The Impact

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.

scholarly journals The Impact of Sickle Cell Disease on Academic Performance among Affected Students

Children ◽  
2021 ◽  
Vol 9 (1) ◽  
pp. 15
Author(s):  
Abdulaziz Alhazmi ◽  
Khalid Hakami ◽  
Faisal Abusageah ◽  
Essa Jaawna ◽  
Meshal Khawaji ◽  
...  
Keyword(s):  
Academic Performance ◽  
Sickle Cell Disease ◽  
Online Education ◽  
Sickle Cell ◽  
Demographic Data ◽  
Acute Chest Syndrome ◽  
Lower Grade ◽  
Cell Disease ◽  
Grade Point ◽  
The Impact

Background: Sickle cell disease (SCD) is a genetic disease that is highly prevalent in Jazan Province, Saudi Arabia, and is mostly characterized by many complications such as vaso-occlusive crises (VOC), acute chest syndrome (ACS) and well-documented neurological complications. These complications may affect patients’ academic performance. Methods: An observational, cross-sectional, retrospective study was conducted in Jazan Province. General and demographic data were collected and questions about academic performance of students with SCD were answered. Both t-tests and chi-square tests, along with multiple logistic regression, were used for analysis. Results: 982 participants were selected for this study with a mean age of 23 years (SD: 7). Most of the participants were female (64%). The number of participants with SCD was 339 (36%), of whom 42% were male. Students with SCD recorded lower grade point averages (GPA) and more absences compared to healthy participants. Further, about 60% of students with SCD thought they performed better than 40% of the participants without SCD during the COVID-19 pandemic when most of the educational activities were online. Conclusion: As has been previously reported, this study suggested that the academic performance of students with SCD is negatively affected compared to healthy individuals, and this is mostly due to complications associated with the disease. Further, students with SCD acknowledged better performance with online education, an option that should be considered to improve their academic performance. National studies on a larger population are required by health and education officials, and supportive online educational programs are warranted to enhance the academic performance of this population.

scholarly journals Successful Management of Sickle Cell Disease Patient with Covid-19 Infection in a Low Resource Setting: Case Study

2021 ◽  
pp. 110-114
Author(s):  
Akaba Kingsley ◽  
Edu C. Betta ◽  
Akaba Edakabasi ◽  
Essien Ofonime ◽  
Bibia Glory Philemon
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Disease Patient ◽  
Sickle Cell Disease Patient ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Management Options ◽  
Adequate Hydration ◽  
History Of ◽  
The Impact

Background: Sickle cell disease (SCD) patients have greater susceptibility to infections, they are reckoned to be vulnerable patients during the current COVID-19 pandemic. SCD patients are commonly affected by pulmonary complications such as acute chest syndrome (ACS), pulmonary embolism (PE) and pneumonia that contribute significantly to mortality risks. Aim: The study was aimed at showing the impact of SARS-COV viral pandemic on SCD patients. Presentation of Case: A 42-year-old male known sickle cell disease patient, who presented with a 5 days’ history of chest pain and difficulty in breathing with a pain score of 8/10. Pain was said to be localized and, subside on the ingestion of analgesics (Tab DF118/60mg and PCM 1000mg) with no known aggravating factor, but there was associated history of difficulty in breathing. The patient was being managed as a case of vaso-occlusive crises R/O acute chest syndrome, and was commenced on adequate hydration, oxygen saturation was between 95-85%. On examination, respiratory rate was 20 cycles per minute, pulse rate – 96 beats/minute, BP and chest examination were essentially normal. CBC showed the Packed Cell Volume of 31%, White Blood Cells 15.04 x 109/L, Neutrophils 7.51x103/µL Lymphocyte 6.50 x103/µL, Monocyte 0.76 x103/µL Eosinophils 0.20x103/µL, Basophils 0.05x103/µL, Platelet 358. The electrolytes (Na-135 mmol/L, K 3.5mmol/L, HCO3-20), urea -10 mmol/L and creatinine (88mmol/L) were normal, the chest x-ray showed cardiomegaly but the lung fields were clear. The patient was administered ceftriaxone (prophylactic antibiotics – 1 g daily).  The patient tested positive to COVID-19 and was immediately transferred to the isolation centre for proper management. He was commenced on oral medication, azithromycin, dexamethasone, ivermectine, amoxicillin/clavulanic acid, vitamin C, clexane and the analgesic was changed to paracetamol and dihydrocodeine to alternate 3 hourly with accordance to the national guidelines. In addition, he was administered subcutaneous enoxaparin due to the hypercoagulability state of SCD. The patient’s health status improved within 24hours of commencement of the above medications and remained stable all through the period of isolation and a repeat covid-19 test was done 15 days of admission using and reverse transcriptase PCR and was discharged home according to the National protocol. Conclusion: Studies and clinical trials are essential to evaluate effective diagnostic and management options for SCD patients and other high-risk conditions like diabetes hypertension, cancer patients and so on that are associated with fatal complications when infected with COVID-19 and similar diseases.

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