Sickle Cell Disease

Piel, Frédéric B.; Steinberg, Martin H.; Rees, David C.

doi:10.1056/nejmra1510865

Cited by 1,007 publications

(1,019 citation statements)

References 97 publications

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“…These were recently reviewed in a few publications [67][68][69]. The major systems affected and their management will be summarized below.…”

Section: Organ Damage/failurementioning

confidence: 99%

Sickle cell disease: Classification of clinical complications and approaches to preventive and therapeutic management

Ballas

2018

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“…These were recently reviewed in a few publications [67][68][69]. The major systems affected and their management will be summarized below.…”

Section: Organ Damage/failurementioning

confidence: 99%

Sickle cell disease: Classification of clinical complications and approaches to preventive and therapeutic management

Ballas

2018

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“…56,57 Currently, HSCT is the only approach with proven curative potential in SCD. However, because of the potential for transplantation-related morbidity and mortality and particularly in the setting of comorbidities, HSCT is less often considered as a treatment option.…”

Section: Benefits and Limitations Of Dual Sot And Hsct For Scdmentioning

confidence: 99%

Toward dual hematopoietic stem-cell transplantation and solid-organ transplantation for sickle-cell disease

Hosoya

Levine

Abt³

et al. 2018

Blood Advances

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Sickle-cell disease (SCD) leads to recurrent vaso-occlusive crises, chronic end-organ damage, and resultant physical, psychological, and social disabilities. Although hematopoietic stem-cell transplantation (HSCT) is potentially curative for SCD, this procedure is associated with well-recognized morbidity and mortality and thus is ideally offered only to patients at high risk of significant complications. However, it is difficult to identify patients at high risk before significant complications have occurred, and once patients experience significant organ damage, they are considered poor candidates for HSCT. In turn, patients who have experienced long-term organ toxicity from SCD such as renal or liver failure may be candidates for solid-organ transplantation (SOT); however, the transplanted organs are at risk of damage by the original disease. Thus, dual HSCT and organ transplantation could simultaneously replace the failing organ and eliminate the underlying disease process. Advances in HSCT conditioning such as reduced-intensity regimens and alternative donor selection may expand both the feasibility of and potential donor pool for transplantation. This review summarizes the current state of HSCT and organ transplantation in SCD and discusses future directions and the clinical feasibility of dual HSCT/SOT.

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“…The complex cascade of events leading to painful episodes culminates in HgbS polymerization and vaso-occlusion and is the primary reason for admission to the hospital [5]. Because most clinicians have used the term "crisis" to describe these acute episodes, and chronic pain to describe such complications as ischemic extremity ulcers or avascular necrosis, there is the perception that most sickle cell patients have pain free periods.…”

Section: Acute On Chronic Painmentioning

confidence: 99%

Navigating the Pain, Psychosocial and Racial Dynamics of Hospitalized Patients with Sickle Cell Disease

Mitchell¹

2018

Arch Med

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Hospital readmissions are receiving more attention because of the implications on cost and quality of care. Many researchers and clinicians believe that a substantial number of readmissions are preventable as evidenced by the significant reductions achieved after the Hospital Readmission Reduction Program mandated by the Affordable Care Act. While sickle cell disease is not currently one of the diseases designated for inclusion in the Hospital Readmission Reduction Program, some of the lessons learned addressing hospital structural characteristics and processes of care can be applied to this population.Acute worsening of chronic pain is the predominant clinical presentation of patients with sickle cell disease to the hospital. Pain and the measurement of pain is subjective and there are no clearly defined objective clinical criteria or biomarkers to validate or refute the presence of acute sickle cell pain crisis. The current epidemic of nonmedical opioid use and medical opioid abuse combined with racial unrest and governmental oversight in the US worsens a wellknown atmosphere of mistrust on the part of both clinicians and patients with sickle cell disease. These dynamics force the need for a paradigm change in management of these patients.

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Sickle Cell Disease

Cited by 1,007 publications

References 97 publications

Sickle cell disease: Classification of clinical complications and approaches to preventive and therapeutic management

Sickle cell disease: Classification of clinical complications and approaches to preventive and therapeutic management

Toward dual hematopoietic stem-cell transplantation and solid-organ transplantation for sickle-cell disease

Navigating the Pain, Psychosocial and Racial Dynamics of Hospitalized Patients with Sickle Cell Disease

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