Red blood cell transfusion therapy for sickle cell patients with frequent painful events

Hilliard, Lee; Kulkarni, Varsha; Sen, Bisakha; Caldwell, Cathy; Bemrich-Stolz, Christina J.; Howard, Thomas H.; Brandow, Amanda M.; Waite, Emily; Lebensburger, Jeffrey D.

doi:10.1002/pbc.27423

Cited by 15 publications

(10 citation statements)

References 20 publications

(28 reference statements)

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“…The total WBC count observed in this study was significantly higher among subjects with SCA than the controls. Same observation was made by Antwi-Baffour and colleagues in 2019 [27], Hilliard and colleagues in 2018 [28] and Kusfa and colleagues in 2017 [29].…”

Section: Resultssupporting

confidence: 64%

Evaluation of Changes in Haematological Parameters of Sickle Cell Anaemia Subjects in Rivers and Bayelsa States

Mbeera

Akwuebu

Ezimah³

et al. 2021

IBRR

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Aim: The aim of this study was to evaluate changes in some haematological parameters in sickle cell anaemia subjects in Rivers State. Study Design: This study is a cross-sectional observational study. Place and Duration of Study: University of Port Harcourt Teaching Hospital, Rivers State, and the Federal Medical Centre, Yenagoa, Bayelsa State, between the months of February and August, 2020. Methodology: A total of four hundred and fifty (450) subjects with age range of 1-50 years were randomly selected. There are about 200 registered patients (adults and children alike) at the sickle cell clinics of the University of Port Harcourt Teaching Hospital, and the Federal Medical Centre, Yenagoa, with an average of 4 new patients per month. The sample size was obtained using a prevalence of sickle cell anaemia of 2% and the sample size was calculated using Cochran sample size formula. Five milliliters (5ml) of venous blood sample was withdrawn from the peripheral vein in the upper limb of subjects using a standard venipuncture technique. The sample was rocked gently to mix and kept at room temperature and the haematological parameters were analyzed within 4 hours of samples collection. The haematological parameters: total white blood cell count (WBC), red blood cell count (RBC), Haemoglobin concentration (Hb), Haematocrit, mean cell volume (MCV), mean cell haemoglobin (MCH), mean cell haemoglobin concentration (MCHC), red cell distribution width (RDW-CV), Platelet count (PLT), MPV, Neutrophils, Lymphocyte, Monocyte, Eosinophils, and Basophils) were analyzed using Mindray BC-6800 auto Haematology analyzer system. Data management and statistical analyses were conducted using Statistical Analyses System SAS 9.4 (SAS Institute, Cary, North Carolina, USA) and p values less than .05 were considered statistically significant. Results: The results showed the mean comparison of haematological parameters in sickle cell anaemia and control subjects. The mean comparison of Haemoglobin F was significantly reduced statistically (p<.05) in vaso-occlusive crises (VOC) condition than steady state compared with the control group. There was increase trend of haematological parameters showing statistically significant difference across the subject conditions compared with the control. There were exceptions in few cases especially in lymphocytes which was not significant (p>.05) in the steady state and vaso-occlusive crisis compared with the control. Similarly, Neutrophils was not significant (p>.05) in the steady state and vaso-occlusive crisis compared with the control. Furthermore, Basophils was more significant (p<.05) in the steady state than in the vaso-occlusive crisis and control groups. Similarly, absolute eosinophil was less significant statistically (p<.05) in the steady state and vaso-occlusive crisis than in the control group. Conclusion: This study has shown that there are changes in haemtological parameters between SCA subjects and control subjects and the VOC and steady state sickle cell anaemia (SCA) subjects.

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Section: Resultssupporting

confidence: 64%

Evaluation of Changes in Haematological Parameters of Sickle Cell Anaemia Subjects in Rivers and Bayelsa States

Mbeera

Akwuebu

Ezimah³

et al. 2021

IBRR

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“…Additionally, although adherence to disease-modifying treatments is associated with fewer hospitalizations (Badawy et al, 2017;Hilliard et al, 2018), children with SCD remain high healthcare utilizers, with more frequent hospital contacts to maintain adequate clinical care.…”

mentioning

confidence: 99%

Brief Screening Measures Identify Risk for Psychological Difficulties Among Children with Sickle Cell Disease

Hood

Reife²,

King

et al. 2019

J Clin Psychol Med Settings

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Children with sickle cell disease (SCD) experience disproportionately high rates of psychological problems. Our goal was to examine the clinical utility of psychological screening measures to identify children with such problems in medical settings. Caregivers completed screening measures assessing social-emotional problems, ADHD symptoms, executive dysfunction, and health-related quality of life (HRQOL) for children with SCD (receiving either chronic blood transfusion or hydroxyurea) and their siblings. Our findings demonstrated that screening measures identified clinically elevated symptoms in children with SCD that had not been previously reported. Scores for siblings were for the most part in the normal range. The number of days hospitalized (but not cerebral infarct status) predicted higher scores, emphasizing the challenges associated with SCD complications. Overall, our findings support the notion that screening measures reduce the need for reliance on medical provider judgment for psychological referrals and increase equitability in access to services. Early identification resulting in early intervention has contributed substantially to improved psychological functioning in many contexts, and it is thus likely that such improvements would also be achieved in this uniquely vulnerable population.

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“…This approach can also lead to the utilization of ektacytometry as a tool for understanding patient response to therapies. For example, blood transfusions are a standard treatment for SCD patients 43 . A previous work has proposed reducing the %S population to less than 30% is beneficial for moderating symptoms in SCD patients 44 .…”

Section: Discussionmentioning

confidence: 99%

Characterizing bulk rigidity of rigid red blood cell populations in sickle-cell disease patients

Gutiérrez

Shamoun

Seu

et al. 2021

Sci Rep

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In this work, we utilized a parameterization model of ektacytometry to quantify the bulk rigidity of the rigid red blood cell (RBC) population in sickle cell disease (SCD) patients. Current ektacytometry techniques implement laser diffraction viscometry to estimate the RBC deformability in a whole blood sample. However, the diffraction measurement is an average of all cells present in the measured sample. By coupling an existing parameterization model of ektacytometry to an artificially rigid RBC model, we formulated an innovative system for estimating the average rigidity of the rigid RBC population in SCD blood. We demonstrated that this method could more accurately determine the bulk stiffness of the rigid RBC populations. This information could potentially help develop the ektacytometry technique as a tool for assessing disease severity in SCD patients, offering novel insights into the disease pathology and treatment.

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Red blood cell transfusion therapy for sickle cell patients with frequent painful events

Abstract: Patients with frequent pain episodes may benefit from one year of transfusion therapy.

Cited by 15 publications

References 20 publications

Evaluation of Changes in Haematological Parameters of Sickle Cell Anaemia Subjects in Rivers and Bayelsa States

Evaluation of Changes in Haematological Parameters of Sickle Cell Anaemia Subjects in Rivers and Bayelsa States

Brief Screening Measures Identify Risk for Psychological Difficulties Among Children with Sickle Cell Disease

Characterizing bulk rigidity of rigid red blood cell populations in sickle-cell disease patients

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