Management of Sickle Cell Disease Complications Beyond Acute Chest Syndrome

Ogu, Ugochi Olivia; Badamosi, Nnenna U; Camacho, Pamela E; Freire, Amado X.; Adams-Graves, Patricia

doi:10.2147/jbm.s291394

Cited by 17 publications

(24 citation statements)

References 77 publications

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“…In settings where drug abuse co-exists with structural racism and poverty, the need for opioids to manage pain is often met with resistance. 36 On the other hand, opioid related disorders have been described to be higher among SCD pregnant woman than in those without SCD and significantly impact maternal sepsis and other pregnancy outcomes. 37 …”

Section: Discussionmentioning

confidence: 99%

“…Given the systematic inequity endured by the affected population, many of these novel strategies will be beyond their reach, especially in developing countries. 36 A systematic review by Sins et al. of controlled clinical trials of pharmaco-therapeutical interventions to reduce VOCs in SCD patients found that the most promising interventions to reduce VOC frequency or hospitalizations were the oral antioxidants L-glutamine and ω−3 fatty acids, and the intravenous antiadhesive agent, crizanlizumab.…”

Section: Discussionmentioning

confidence: 99%

“…highlight the burden of maternal complications in SCD and the relatively small number of clinical trials objectively evaluating strategies to improve outcome. 36 , 48 …”

Section: Discussionmentioning

confidence: 99%

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Maternal mortality among women with sickle cell disease in Jamaica over two decades (1998–2017)

McCaw-Binns

Campbell²,

Harris

et al. 2022

eClinicalMedicine

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Summary Background Sickle cell disease (SCD) affects 2.8% of Jamaican antenatal women. Between 1998–2007 their maternal mortality ratio was 7–11 times higher than women without these disorders. We aim to determine if outcomes improved between 2008 and 17 amid declining fertility and changes in referral obstetric care. Methods Maternal deaths in Jamaica's maternal mortality surveillance database (assembled since 1998) with SCD reported as underlying or associated cause of death were compared to those without known SCD, over two decades from 1998 to 2017. Social, demographic and health service variables were analysed using SPSS and EpiInfo Open. Findings Over the two decades from 1998 to 2017, 806 (74%) of the 1082 pregnancy-associated deaths documented by the Jamaican Ministry of Health and Wellness were maternal deaths. The maternal mortality ratio (MMR) did not statistically change over the two periods for women with ( p = 0.502) and without SCD ( p = 0.629). The MMR among women with and without SCD in 2008–17 was 378.1 ( n = 41) and 89.2/100,000 live births ( n = 336) respectively, an odds ratio of 4.24 (95% CI: 3.07–5.87). When deaths due to their blood disorders were excluded, risk remained elevated at 2.17 (95% CI: 1.36–3.32). There was an upward trend in direct deaths over the two decades (p [trend]=0.051). Interpretation MMRs were unchanged over two decades for Jamaicans with SCD. The high contribution to maternal mortality by women with SCD may explain some of the persistently higher mortality experience of women in the African diaspora. Multi-disciplinary evidence-based strategies need to be developed and tested which improve survival for women with SCD who want to have children. Funding No external funding was provided.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Maternal mortality among women with sickle cell disease in Jamaica over two decades (1998–2017)

McCaw-Binns

Campbell²,

Harris

et al. 2022

eClinicalMedicine

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“…Serious complications include the onset of acute chest syndrome (ACS), avascular necrosis, hemolytic anemia, silent cerebral infarction, inflammation, priapism, and stroke. Moreover, serious complications often revolve around organ damage, eventually resulting in organ failure, including renal failure [ 2 , 3 ]. Apart from the disease complications, treatment-related adverse effects also occur, especially in those who require frequent blood transfusions, most commonly presenting as TRALI [ 5 ].…”

Section: Reviewmentioning

confidence: 99%

“…Due to their deformed shape, sickle cells tend to become trapped within small vessels and deprive the tissues of blood that is rich in oxygen [ 2 ]. The onset of SCD is often accompanied by various complications, including hemolytic anemia, reduced immunity, inflammation, acute/chronic organ damage, and stroke, among others [ 3 ]. Therefore, providing effective treatment to patients impacted by SCD is critical.…”

Section: Introductionmentioning

confidence: 99%

A Systematic Review on the Management of Transfusion-Related Acute Lung Injury in Transfusion-Dependent Sickle Cell Disease

Arzoun¹,

Srinivasan²,

Adam³

et al. 2022

Cureus

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The onset of respiratory distress and acute lung injury (ALI) following a blood transfusion is known as transfusion-related acute lung injury (TRALI), although its pathophysiology remains unknown. Even though sickle cell disease (SCD) has been studied for more than a century, few therapeutic and management strategies adequately address the emergence of TRALI. TRALI, an immune-mediated transfusion response that can result in life-threatening consequences, is diagnosed based on clinical signs and symptoms. Early detection and treatment increase the chances of survival and, in most cases, result in a complete recovery. Our objective is to provide a firm grasp of the present status of SCD-related TRALI care and therapy. After exploring multiple databases, this study offers evidence-based guidelines to aid clinicians and other healthcare professionals make decisions concerning transfusion assistance for SCD and the management of transfusion-related complications. Other risk factors for acute lung injury including sepsis aspiration should be ruled out throughout the diagnostic process. Several recent studies have shown that immunotherapy or immunological targets can effectively prevent these complications. Red cell transfusions, red cell antigen matching optimization, and iron chelation can also help reduce negative consequences. It is to be noted that poor clinical outcomes can be avoided by early detection and treatment of hemolytic transfusion reactions. Finally, preventing the onset of TRALI may be the most effective therapeutic strategy for SCD patients who rely on blood transfusions for survival.

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Safety and efficacy of L-Glutamine in reducing the frequency of acute complications among patients with sickle cell disease: A randomized controlled study

Ebeid,

Aly,

Shaheen

et al. 2024

Ann Hematol

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To evaluate the safety and efficacy of L-glutamine in reducing vaso-occlusive crisis (VOC) and improving cerebral arterial blood flow in children with sickle cell disease (SCD). This is an interventional randomized controlled trial that recruited sixty SCD patients, aged 9.2 ± 3.7 years, who had at least two VOCs during the last 12 months and on a stable dose of hydroxyurea. They were randomly assigned in a 1:1 ratio to receive glutamine (0.3 gm/kg/dose/12h) orally for 24 weeks or the standard of care (SOC). All patients had VOCs in the last year > 3, those on glutamine had a higher number of VOCs and hospitalization for VOC in the last year. There was a decreasing trend in the number, severity, and hospitalization of VOC and a significantly lower cumulative number of VOCs and hospitalizations in the glutamine group than in SOC (p = 0.008, p < 0.001 respectively). Time-averaged mean maximum velocity for the glutamine group had a marginal increase in both middle cerebral arteries, all values remained normal within a normal range, and in both internal carotid arteries, values increased from abnormally low to normal ranges at week 24. Glutamine reduced the number of VOCs and severity and may have a potentially favorable impact on the cerebral arterial flow velocities.

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Management of Sickle Cell Disease Complications Beyond Acute Chest Syndrome

Cited by 17 publications

References 77 publications

Maternal mortality among women with sickle cell disease in Jamaica over two decades (1998–2017)

Maternal mortality among women with sickle cell disease in Jamaica over two decades (1998–2017)

A Systematic Review on the Management of Transfusion-Related Acute Lung Injury in Transfusion-Dependent Sickle Cell Disease

Safety and efficacy of L-Glutamine in reducing the frequency of acute complications among patients with sickle cell disease: A randomized controlled study

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