Current challenges in the management of patients with sickle cell disease – A report of the Italian experience

Russo, Giovanna; Franceschi, Lucia De; Colombatti, Raffaella; Rigano, Paolo; Perrotta, Silverio; Voi, Vincenzo; Palazzi, Giovanni; Fidone, Carmelo; Quota, Alessandra; Graziadei, Giovanna; Pietrangelo, Antonello; Pinto, Valeria; Ruffo, Giovan Battista; Sorrentino, Francesco; Venturelli, Donatella; Casale, Maddalena; Ferrara, Francesca; Sainati, Laura; Cappellini, Maria Domenica; Piga, Antonio; Maggio, Aurelio; Forni, Gian Luca

doi:10.1186/s13023-019-1099-0

Cited by 32 publications

(30 citation statements)

References 92 publications

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“…Nevertheless, interplay of genetic and environmental factors contributes to overall clinical outcomes in a substantial burden of human diseases 12‐17 . Sickle cell disease (SCD) disproportionately affects Black/African Americans in the United States as well as forebearers from sub‐Saharan Africa, the Western Hemisphere (South America, the Caribbean, and Central America), and some Mediterranean countries 18‐20 . Increasing evidence suggests that COVID‐19 pneumonia can cause acute chest syndrome (ACS), a potentially life‐threatening complication in SCD 21‐23 .…”

Section: Introductionmentioning

confidence: 99%

Sickle cell trait and the potential risk of severe coronavirus disease 2019—A mini‐review

Kehinde

Osundiji

2020

European J of Haematology

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Following the emergence of Coronavirus Disease 2019 (COVID-19) in Wuhan (China) around December 2019, 1 the infection has become a pandemic, decimating over three hundred thousand globally. 2 The severity of COVID-19 cases reported hitherto has varied greatly from asymptomatic to severe pneumonia and thromboembolism, accompanied by overt respiratory failure with subsequent mortality. 3-5 As the COVID-19 pandemic continues to evolve, potential risk factors that may predispose individuals to fatal outcomes are increasingly becoming topical. There are ongoing epidemiological investigations and discussions of potentially at-risk populations and ethnicities. 6,7 An improved understanding of the risk factors as well as clinical course for severe COVID-19 may shed some light on novel personalized approaches to optimize clinical care and outcomes. Emerging trends in the United States suggest possibly higher mortality rates of COVID-19 among African Americans, 8,9 although epidemiological study data with adequate denominator 10 across board is pending. There is no doubt that socioeconomic determinants of health play crucial roles in health inequities and disease trajectory. 11 Nevertheless, interplay of genetic and environmental factors contributes to overall clinical outcomes in a substantial burden of human diseases. 12-17 Sickle cell disease (SCD) disproportionately affects Black/ African Americans in the United States as well as forebearers from sub-Saharan Africa, the Western Hemisphere (South America, the Caribbean, and Central America), and some Mediterranean countries. 18-20 Increasing evidence suggests that COVID-19 pneumonia can cause acute chest syndrome (ACS), a potentially life-threatening complication in SCD. 21-23 These observations raise questions on the potential contributions of SCD-related complications, for instance

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Section: Introductionmentioning

confidence: 99%

Sickle cell trait and the potential risk of severe coronavirus disease 2019—A mini‐review

Kehinde

Osundiji

2020

European J of Haematology

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“…The lack of reliable data in most countries makes it difficult to estimate the actual number of affected people worldwide [7][8][9][10][11][12]. There are over 300,000 affected births each year, with a high frequency of patients with SCD across most of sub-Saharan Africa, Saudi Arabia, and India, as well as in descendants from the Caribbean, Central America, South America, and Mediterranean countries [13].…”

Section: A Prevalence That Is Difficult To Estimate Despite the Evolumentioning

confidence: 99%

Descriptive analysis of sickle cell patients living in France: The PHEDRE cross-sectional study

et al. 2021

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Background Sickle cell disease (SCD) induces chronic haemolytic anaemia and intermittent vaso-occlusion that results in tissue ischaemia causing acute, severe pain episodes that can lead to frequent hospitalizations. These consequences can have repercussions on family, social, school and/or professional life. Here, we present some of the results of the PHEDRE study (Pharmacodépendance Et DREpanocytose—drug dependence and sickle-cell disease), which is the largest study of patients with SCD in France. This paper intends to describe characteristics of the French SCD population. We also aimed to assess the impact of the disease on the lives of patients using objective and subjective variables. Methods The PHEDRE study was a national multicentric observational study. Adults, adolescents and children with a confirmed SCD diagnosis were included in the study by their referring doctor. Then, they were interviewed by phone about their socioeconomic status, about the impact of the disease on their lives and about their analgesic and psychoactive drug use. Results The study population consisted of 872 patients (28% were minors). Seventy-two percent of adults were active, and all minors were in school. Many patients presented criteria of severe SCD. Seventy-five percent were homozygous SS, 15% were double heterozygotes SC and 8% were heterozygotes Sβthal, 87% received specific treatment, 58% were hospitalized at least once for vaso-occlusive crisis in the past 12 months, and the number of analgesic drugs taken averaged 3.8. Seventy-five percent of patients reported academic or professional consequences related to their SCD, and 52% reported social consequences. Conclusions The impact of SCD on patients’ lives can be significant, nevertheless their social integration seems to be maintained. We highlighted respect of recommendations regarding analgesic treatments and only a few patients used tobacco, alcohol or cannabis. Trial registration Clinical Trials, NCT02580565; https://clinicaltrials.gov/ Registered 16 October 2015.

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“…SCD can result in severe complications including chronic pain, end-organ dysfunction, stroke, life-long suffering, poor quality of life, and early mortality. Treatments include medications to reduce pain and to 3 prevent complications (e.g., hydroxyurea), and blood transfusions, as well as a bone marrow transplant in patients who have a related donor [3,4 ].…”

Section: Introductionmentioning

confidence: 99%

Red Blood Cell Exchange in children with Sickle Cell Disease in French Guiana

Elenga¹,

Vantilcke²,

Martin³

et al. 2020

Preprint

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The aim of our study was to describe our experience using a Spectra Optia® automated apheresis system in children with sickle cell disease (SCD). We used automated red blood cell exchange (RCE) to treat acute and chronic complications in 75 children with SCD who had a median age of 10 years [7-13]. We analysed 649 exchange sessions. Peripheral venous access was limited in a number of the children, thus requiring a femoral central double‐lumen venous catheter (CVC). We recommend the use of heparin locking, with 500 units in each lumen of a CVC. This method was well tolerated, with few complications during the procedures. For preoperative prevention, all of the patients had achieved a post-RCE HbS level of <30% since this is a mandatory condition imposed by the anaesthesiologist. With a post-RCE Hb level of approximately 10-11 g/dL, a blood exchange volume of ≥32 mL/kg, and an interval between each RCE procedure of ≤30 days, it was able to maintain the residual HbS level below 30%. Despite a target pre‐exchange HbS level of 47%, we did not encounter a single stroke recurrence. Erythrocytapheresis is useful and safe for children with SCD.

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Current challenges in the management of patients with sickle cell disease – A report of the Italian experience

Cited by 32 publications

References 92 publications

Sickle cell trait and the potential risk of severe coronavirus disease 2019—A mini‐review

Sickle cell trait and the potential risk of severe coronavirus disease 2019—A mini‐review

Descriptive analysis of sickle cell patients living in France: The PHEDRE cross-sectional study

Red Blood Cell Exchange in children with Sickle Cell Disease in French Guiana

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