Multicenter COMPACT study of COMplications in Patients with sickle cell disease And utilization of iron Chelation Therapy

Jordan, Lanetta; Adams-Graves, Patricia; Kanter-Washko, Julie; O'Neal, Patricia; Sasané, Medha; Vekeman, Francis; Bieri, Christine; Magestro, Matthew; Marcellari, Andrea; Duh, Mei Sheng

doi:10.1185/03007995.2014.998815

Cited by 9 publications

(8 citation statements)

References 24 publications

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“…The majority of patients with SCD experience sickle cell pain crises, also known as vaso-occlusive crises (VOC), and as many as 30% of patients experience chronic pain. 6 , 7 VOCs, the hallmark of SCD, are severe painful events that result in frequent emergency department (ED) visits and hospitalizations. 8 They are also associated with increased mortality and diminished health-related quality of life (HRQL).…”

Section: Introductionmentioning

confidence: 99%

<p>Patient-Reported Outcomes and Economic Burden of Adults with Sickle Cell Disease in the United States: A Systematic Review</p>

Lee

Vania

Bhor

et al. 2020

IJGM

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Purpose: To systematically estimate the patient-reported outcomes (PROs) and economic burden of sickle cell disease (SCD) among adults in the United States (US). Patients and Methods: Two systematic literature reviews (SLRs), one each for the PROs and economic topics, were performed using MEDLINE and Embase to identify observational studies of adults with SCD. Included studies were published between 2007 and 2018 and evaluated health-related quality of life (HRQL), function, healthcare resource utilization (HCRU), or costs. Given the high degree of clinical and methodological heterogeneity, findings were summarized qualitatively. Results: The SLRs identified 7 studies evaluating the PROs and 15 studies evaluating the economic burden meeting the pre-specified selection criteria. The PRO evidence showed the prevalence of depression and anxiety to be 21-33% and 7-36%, respectively, in adults with SCD. The mean SF-36 physical summary scores ranged from 33.6 to 59.0 and from 46.3 to 61.5 for the mental summary scores. Overall HRQL for adults with SCD was poor and significantly worse in those with opioid use. Adult SCD patients were found to have varying rates of emergency department (ED) utilization (0.3-3.5 annual ED visits), hospitalizations (0.5-27.9 per patient per year), and/or readmission (12-41%). Key factors associated with significant HCRU were age, dental infection, and SCD-related complications. SCD specialized care settings and SCD intensive management strategy were reported to significantly decrease the number of hospitalizations. Conclusion: This systematic evidence synthesis found that disease burden measured by PROs and economic burden of SCD on adults in the US are substantial despite the availability of approved SCD treatments during 2007-2018. The use of hydroxyurea, optimal management with opioids, and employing intensive treatment strategies may help decrease the overall burden to patients and healthcare systems. Published data on costs associated with SCD are limited and highlight the need for more economic studies to characterize the full burden of the disease.

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Section: Introductionmentioning

confidence: 99%

<p>Patient-Reported Outcomes and Economic Burden of Adults with Sickle Cell Disease in the United States: A Systematic Review</p>

Lee

Vania

Bhor

et al. 2020

IJGM

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“…A case report of two patients describes an association between iron depletion and ameliorating symptoms, decreased mean corpuscular volume and upregulation of fetal hemoglobin [11] and discusses the potential to maintain a low iron state which is often not seen in SCD. While there has been some positive results with antiplatelet or anticoagulant strategies in attenuating VOC [1], focused chelation therapy, especially with patients with regular transfusions, has decreased the incidence and intensity of VOC [12]. Identification of patients with iron mediated hypercoagulability may broaden indications for chelation therapy.…”

Section: Discussionmentioning

confidence: 94%

Sickle cell disease is associated with iron mediated hypercoagulability

Shah

Welsby

Fielder

et al. 2015

J Thromb Thrombolysis

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Sickle cell disease (SCD) is associated with a significant hypercoagulable state and several hemostatic anomalies have been identified in this disease state. Of interest, SCD patients can become iron overloaded after transfusion, and iron can enhance fibrinogen as a substrate for thrombin, resulting in thrombi that commence coagulation quickly and form rapidly. We hypothesized that SCD patients would display hypercoagulable plasma coagulation kinetics and an iron enhancement of coagulation. After obtaining IRB approval, we assessed coagulation kinetics and iron enhancement with viscoelastic methods in archived, citrated plasma obtained from ambulatory or hospitalized SCD patients (n = 20). All SCD patients had plasmatic hypercoagulability, and 65 % were positive for iron enhancement of coagulation. In conclusion, continuing investigation correlating such viscoelastic data with clinical symptoms may provide insight into the role played by iron in the setting of SCD, including complications such as vaso-occlusive crisis.

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“…There is substantial evidence that these agents improve the clinical outcome in thalassaemia . As it is extremely difficult, if not impossible, to separate the complications of iron overload from those of SCD, there are minimal and conflicting data suggesting chelation improves SCD outcomes (Jordan et al, 2015;Vekeman et al, 2016;Vitrano et al, 2016). The most important differences between these agents relate to the route of administration, the half-life and the toxicities.…”

Section: Treatment Of Iron Overloadmentioning

confidence: 99%

How we manage iron overload in sickle cell patients

Coates

Wood

2017

Br J Haematol

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Summary Blood transfusion plays a prominent role in the management of patients with sickle cell disease (SCD), but causes significant iron overload. As transfusions are used to treat the severe complications of SCD, it remains difficult to distinguish whether organ damage is a consequence of iron overload or is due to the complications treated by transfusion. Better management has resulted in increased survival, but prolonged exposure to iron puts SCD patients at greater risk for iron-related complications that should be treated. The success of chelation therapy is dominated by patient adherence to prescribed treatment; thus, adjustment of drug regimens to increase adherence to treatment is critical. This review will discuss the current biology of iron homeostasis in patients with SCD and how this informs our clinical approach to treatment. We will present the clinical approach to treatment of iron overload at our centre using serial assessment of organ iron by magnetic resonance imaging.

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Multicenter COMPACT study of COMplications in Patients with sickle cell disease And utilization of iron Chelation Therapy

Cited by 9 publications

References 24 publications

<p>Patient-Reported Outcomes and Economic Burden of Adults with Sickle Cell Disease in the United States: A Systematic Review</p>

<p>Patient-Reported Outcomes and Economic Burden of Adults with Sickle Cell Disease in the United States: A Systematic Review</p>

Sickle cell disease is associated with iron mediated hypercoagulability

How we manage iron overload in sickle cell patients

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