Comparison of automated red cell exchange transfusion and simple transfusion for the treatment of children with sickle cell disease acute chest syndrome

Saylors, Robert L.; Watkins, Benjamin; Saccente, Suzanne; Tang, Xinyu

doi:10.1002/pbc.24744

Cited by 30 publications

(31 citation statements)

References 17 publications

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“…28 Retrospective comparisons of RCE and simple transfusion have been inconclusive, with 1 study in adults showing no significant differences in severity or patients' outcome between RCE and simple transfusion, but a study in children comparing simple transfusion, up-front RCE, or simple transfusion followed by RCE showed that outcomes did not differ between simple transfusion and RCE despite the RCE group having more severe features of ACS. 29 This study supports the recommendations from evidence-based reviews that not all patients with ACS will require transfusion and that the necessity and type of transfusion will depend on clinical severity. In adult patients, an arterial blood gas performed on room air may be useful in guiding treatment decisions and a partial pressure of oxygen ,9.0 kPa (65 mm Hg) has been recommended as an indication for transfusion, 25 but transfusion should not be delayed in rapidly deteriorating patients with a lesser degree of hypoxia.…”

Section: 12supporting

confidence: 83%

Sickle cell disease: when and how to transfuse

2016

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Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome, comes from observational data. Several important randomized controlled trials have shown the efficacy of transfusion in primary and secondary stroke prevention in patients with SCD but, outside these areas, clinical practice lacks a clear evidence base. Evidence for the role of long-term transfusion in the prevention of the non-neurologic chronic complications of SCD comes from analysis of secondary outcomes of these randomized trials and from observational data. In view of the paucity of data, the risks and benefits of transfusion should be fully discussed with patients/families before a long-term transfusion program is commenced. Evidence is only available for the role of preoperative transfusion or for prophylactic transfusion through pregnancy in certain situations, and the role of transfusions outside these situations is discussed. Questions about when and how to transfuse in SCD remain and will need further randomized trials to provide answers. Learning Objectives• To describe the advantages and disadvantages of simple, manual exchange and automated exchange transfusion • To discuss when transfusion should be used to treat the acute and chronic complications of sickle cell disease • To discuss the role of preoperative transfusion and of transfusion during pregnancy in decreasing sickle-related complications

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Section: 12supporting

confidence: 83%

Sickle cell disease: when and how to transfuse

2016

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“…[15] Currently, erythrocyte transfusion remains the only therapy available that rapidly reduces the percentage of sickled erythrocytes to impact the severity of illness in hospitalized patients. [16,17] Since supportive care interventions (e.g. incentive spirometry) reduce secondary complications in hospitalized patients that result in longer LOS,[18,19] interventions that optimize these supportive care therapies may also be another way to reduce LOS, ICU admissions, and erythrocyte transfusions in children with SCD until novel interventions are developed and tested.…”

Section: Discussionmentioning

confidence: 99%

Hydroxyurea use in Children with Sickle Cell Disease: Do Severely Affected Patients Use It and Does It Impact Hospitalization Outcomes?

Creary

Chisolm

Koch

et al. 2016

Pediatr Blood Cancer

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Background Expert guidelines recommend that hydroxyurea (HU) be offered to all children with Hemoglobin SS and Sβ0 sickle cell disease (SCD) and be considered for children with clinically severe Hemoglobin SC or Sβ+. This study aims to determine the rate of HU use in hospitalized children, if HU is differentially used in children with clinically severe SCD, and if HU users have shorter lengths of stay (LOS), fewer intensive care unit (ICU) admissions, and fewer inpatient transfusions compared to non-users. Procedure Using the Pediatric Health Information System, we performed a retrospective analysis of children ages 2–18 years with SCD discharged between January 1, 2011–September 30, 2014. We defined patients as having clinically severe SCD if they had a recent ICU admission or ≥3 admissions in the preceding year. Results Of the 2,665 unique children identified, approximately 80% had an inpatient code indicating HU use. Significantly more (p<0.001) non-users (30.1%) had a recent ICU admission compared to HU users (18.7%). More non-users (33.9%) had a history of ≥3 admissions compared to HU users (21.5%) (p<0.001). After applying propensity score weighting, the groups did not differ in their LOS, prevalence of ICU admissions, or prevalence of transfusions. Conclusion HU use is high among hospitalized children with SCD. However, HU is not utilized by many children with clinically severe SCD. These results support that HU be considered in children with SCD to prevent hospitalization rather than as a treatment to improve hospitalization outcomes.

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“…Retrospective studies have shown some success in demonstrating the safety and efficacy of RBC exchange for the treatment of acute chest syndrome . Limited evidence suggests that RBC exchange could be beneficial to patients with acute and recurrent episodes of priapism who fail other therapies .…”

Section: Indications For Rbc Exchangementioning

confidence: 99%

“…Patients with higher hemoglobin concentrations, such as those with hemoglobin SC disease, may still require RBC exchange, because simple transfusion may exacerbate disease by increasing intravascular viscosity or by causing volume overload . Retrospective studies have indicated that both RBC exchange and simple transfusion are equally effective in treating acute chest syndrome in both children and adults, although the choice of modality in those studies may have been biased by patient presentation . However, the degree of reduction in sickled RBCs is preferentially much greater with RBC exchange.…”

Section: Rbc Exchange Treatment Considerationsmentioning

confidence: 99%

“…15 Retrospective studies have shown some success in demonstrating the safety and efficacy of RBC exchange for the treatment of acute chest syndrome. 1,16,17 Limited evidence suggests that RBC exchange could be beneficial to patients with acute and recurrent episodes of priapism who fail other therapies. 18 Case reports also suggest that RBC exchange potentially could be lifesaving in patients who have multiorgan failure and intrahepatic cholestasis.…”

Section: Sickle Cell Diseasementioning

confidence: 99%

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Vascular access for red blood cell exchange

2018

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Red blood cell exchange is the process of removing red blood cells from a patient and replacing them with donated blood using either automated or manual techniques. Red blood cell exchange is a well‐recognized and effective therapy for many red blood cell‐related diseases, especially sickle cell disease. However, decisions regarding the best methods for vascular access are not intuitive and must account for the patient's clinical condition, complication risks, and lifestyle, especially in the context of long‐term vascular access. In this review, we discuss the recognized indications for red blood cell exchange, considerations for the selection of exchange modality and vascular access, and recommendations for the appropriate care and prevention of risks associated with vascular access.

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Comparison of automated red cell exchange transfusion and simple transfusion for the treatment of children with sickle cell disease acute chest syndrome

Cited by 30 publications

References 17 publications

Sickle cell disease: when and how to transfuse

Sickle cell disease: when and how to transfuse

Hydroxyurea use in Children with Sickle Cell Disease: Do Severely Affected Patients Use It and Does It Impact Hospitalization Outcomes?

Vascular access for red blood cell exchange

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