scholarly journals Suspected Severe Malaria in a Sudanese Patient Affected by Sickle Cell Disease Who Was Treated with Hydroxyurea

Pathogens ◽  
2021 ◽  
Vol 10 (8) ◽  
pp. 985
Author(s):  
Giulia Chiopris ◽  
Stefano Maccario ◽  
Tawaddud Hassan Eisa Artaiga ◽  
Abdalrhman Ibrahim Mohamed ◽  
Manuela Valenti ◽  
...  
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Severe Malaria ◽  
Sickle Cell ◽  
Signs And Symptoms ◽  
Infectious Complications ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Routine Administration ◽  
Sub Saharan

Sickle cell disease (SCD) is the most common genetic disease in sub-Saharan Africa. The signs and symptoms of SCD usually begin in early childhood. Characteristic features of this disorder include anaemia, repeated infections, and periodic episodes of pain. Malaria is one of the infections that can occur in patients with SCD in endemic countries. Many guidelines recommend antimalarial chemoprophylaxis in these patients, although the debate on which drug should be used is still ongoing. Hydroxyurea (HU), which is considered a safe and effective treatment for both children and adults with SCD, seems to affect the incidence and severity of malaria, although these impacts have yet to be fully demonstrated. We report a case of an eight-and-a-half-year-old Sudanese boy with SCD treated with HU admitted for suspected severe malaria who showed a recrudescence after first-line treatment. Although he had undergone splenectomy and thus belonged to a category of patients at high risk for infectious complications, he was not receiving any malaria chemoprophylaxis. This case emphasises the importance of the routine administration of malaria prophylaxis to children with SCD living in endemic areas, even when they are treated with HU, and especially if they are at high risk for infectious complications because they have undergone splenectomy. There is an urgent need for further research to evaluate the most appropriate regimen and its optimal duration.

scholarly journals Sickle Cell Anemia Presenting with Vaso-Occlusive Pain: Should We Screen for COVID-19?

2021 ◽  
pp. 1-4
Author(s):  
Mohammad Ali ◽  
Lina Okar ◽  
Nabil E. Omar ◽  
Jabeed Parengal ◽  
Ashraf Soliman ◽  
...  
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Signs And Symptoms ◽  
Risk Groups ◽  
Position Statement ◽  
Interesting Case ◽  
International Federation ◽  
Cell Disease ◽  
The World

Despite the widespread of coronavirus disease-19 (CO­VID-19) infection around the world, there are very scarce reported literature about the care of patients with a known diagnosis of hemoglobin disorders such as sickle cell disease (SCD) or thalassemia and confirmed COVID-19 infection. Thalassemia International Federation issued a position statement to include patients with thalassemia and SCD among the high-risk groups of patients. Here, we present an interesting case of a 42-year-old patient know to have SCD presenting with Vaso-occlusive (VOC) pain episode in the absence of COVID-19 signs and symptoms, who tested positive for COVID-19 infection and had a smooth recovery. This case highlights the importance of screening SCD patients presenting with VOC-related events even in the absence of COVID-19 signs and symptoms.

Living with sickle cell disease: voices from sub-Saharan Africa

2021 ◽  
Author(s):  
Arafa Said Salim ◽  
Emmy Mwita ◽  
Joseph Sarfo Antwi ◽  
Olamide Agunkejoye ◽  
Paul Mdliva
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Sub Saharan

Abstract 65: Anemia-related Heart Failure in Sub-saharan African Sickle Cell Disease Patients

2017 ◽  
Vol 121 (suppl_1) ◽  
Author(s):  
Adebayo C Atanda ◽  
Yahya Aliyu ◽  
Oluwafunmilayo Atanda ◽  
Aliyu Babadoko ◽  
Aisha Suleiman ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Hemoglobin Level ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Jet Velocity ◽  
Sub Saharan

Introduction: Anemia has been implicated in heart failure. Existing literatures, involving predominantly African-Americans, suggests that Sickle Cell Disease (SCD) maybe linked to various cardiovascular complications including pulmonary hypertension and left venticular dysfunction. Peculiarly, our study involves exclusively Sub-Saharan population. Method: We conducted a cross sectional observational study of 208 hydroxyurea-naive consecutive SCD patients aged 10-52 years at steady state and 94 healthy non-matched controls who were studied in an out patient clinic in Sub-Saharan Africa. SCD patients were required to have electrophoretic or liquid chromatography documentation of major sickling phenotypes. Control group was required to have non-sickling phenotypes. Cardiac measurements were performed with TransThoracic Echo according to American Society of Echocardiography guidelines. Hemoglobin level was also obtained. Results: Hemoglobin level in SCD group (8.5+/- 1.5) was significant (P<0.001) compared to control (13.8+/- 1.7). Although SCD group had significantly higher values of left ventricular (LV) size, there was no qualitative evidence of LV dysfunction. SCD group had higher values of Ejection Fraction but not statistically significant. There was no evidence of LV wall stiffening to impair proper filling in SCD group, with the ratio of early to late ventricular filling velocities, E/A ratio elevated (1.7+/-0.4 compared to 1.6+/- 0.4; P=0.010). Right ventricular systolic pressure was determined using the formula of 4x Tricuspid Reugurgitant jet (TRV) square as an indirect measurement of Pulmonary arterial systolic pressure. SCD patients had significantly higher mean±SD values for tricuspid regurgitant jet velocity than did the controls (2.1±0.6 vs. 1.8±0.5; p= 0.001). Within the SCD group, there was no clear pattern of worsening diastolic function with increased TRV. Furthermore, E/A had a significant positive relationship with jet velocity in bivariate analysis (R=0.20; P=0.013). Conclusions: We were unable to demonstrate existence of anemia-associated left ventricular dysfunction in Sub-Saharan African with SCD. Further studies is required to highlight the reason behind this finding.

scholarly journals Advancing Healthcare Outcomes for Sickle Cell Disease in Nigeria Using Mobile Health Tools

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2173-2173
Author(s):  
Arwa Fraiwan ◽  
Muhammad Noman Hasan ◽  
Ran An ◽  
Amy J. Rezac ◽  
Nicholas J. Kocmich ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Mobile Health ◽  
Sub Saharan Africa ◽  
Diagnosis And Treatment ◽  
Test Results ◽  
Cell Disease ◽  
The World ◽  
Equity Ownership ◽  
Sub Saharan

Nigeria leads the world in the number of cases of sickle cell disease (SCD). An estimated 150,000 babies are born annually in Nigeria with SCD, a heredity disorder, and 70-90% die before age 5. Only a small portion of affected infants and children in sub Saharan Africa (SSA) reach adolescence. Over 650 children die per day in sub-Saharan Africa from SCD. These dismal statistics are in sharp contrast to outcomes in high-income countries (HICs) where more than 90% of SCD patients reach adulthood. The World Health Organization (WHO) estimates that 70% of deaths could be prevented with a low cost diagnostic and treatment plan. Meaningful preventive care and treatment cannot be implemented without a structured plan for early diagnosis and patient tracking.Early diagnosis requires improved access to parents and guardians of children with SCD, and gaining this access remains a challenge in most of SSA. In 2015, Nigeria's Kano state government, with support from foreign partners, established a community-based program for newborn registration. This platform provides unique access to newborn babies in one of Nigeria's most populous cities, but still lacks a functioning patient testing, tracking, and monitoring system, which we plan to address in our ongoing study. This study will introduce mobile health in a low-income country with low literacy rate and hopefully accustom that segment of the population to more varied mobile health applications that will ultimately improve their health in the long run. Our current operational platform in Kano, Nigeria provides access to a large population with a high prevalence of SCD. We have previously completed pilot testing of 315 subjects for SCD using our microchip electrophoresis test. We are planning to test up to 4,500 additional subjects less than 5 years of age at Murtala Muhammed Specialist Hospital. The hospital staff includes 97 physicians and 415 nurses and outpatient clinics serve about 30,000 patients monthly. The maternity department has a 200-bed capacity and the antenatal clinic performs about 1,000 deliveries and serves an average of 3,000 mothers monthly. Enrollment is planned to start on September 15, 2019 and medical staff are currently being trained to run the tests. Our study is registered in the United States National Library of Medicine's ClinicalTrials.gov (Identifier: NCT03948516). Our technology is uniquely paired with an automatic reader and an Electronic Medical Record (EMR) and patient management solution to record POC test results, register new cases, and track patients for follow-up (Fig. 1). The reader enables automated interpretation of test results, local and remote test data storage, and includes geolocation (Global Positioning System) (Fig. 2). The system will generate reports for all cases of SCD, track hospital visits, appointments, lab tests, and will have mobile and dashboard applications for tracking patients and samples. The application will be installed on mobile devices provided to users. The proposed system will be compliant with the existing privacy standards to handle medical data (e.g., HIPAA in the US and GDPR in the EU). All communications between the parties will be secured via end-to-end encryption as a safeguard. We anticipate that our project will increase the rates of screening, diagnosis and timely treatment of SCD in Kano State of Nigeria. The project's broader impact will likely be the ability to track and monitor screening, disease detection, diagnosis and treatment, which can be scaled up to the whole nation of Nigeria, then to sub-Saharan Africa. The data obtained and analyzed will be the first of their kind and will be used to inform the design of programs to improve access to, and availability of, effective care for this underserved populations. The importance of increased access to diagnosis and treatment should not be underestimated - it is crucial for realizing effective management of people with SCD. The impact can be enhanced by complementing diagnosis and patient tracking with education for the families so they can provide or seek the necessary preventative treatment. Identification of the location of the patients in need would help identify the areas where family, parent, caregiver education should be provided. Disclosures Fraiwan: Hemex Health, Inc.: Equity Ownership, Patents & Royalties. Hasan:Hemex Health, Inc.: Equity Ownership, Patents & Royalties. An:Hemex Health, Inc.: Patents & Royalties. Thota:Hemex Health, Inc.: Employment. Gurkan:Hemex Health, Inc.: Consultancy, Employment, Equity Ownership, Patents & Royalties, Research Funding.

scholarly journals Management of chronic respiratory complications in children and adolescents with sickle cell disease

2020 ◽  
Vol 29 (157) ◽  
pp. 200054
Author(s):  
Michele Arigliani ◽  
Atul Gupta
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Current Knowledge ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Respiratory Complications ◽  
Progressive Decline ◽  
Blood Disorder ◽  
Life Threatening ◽  
Sub Saharan

Sickle cell disease (SCD) is a life-threatening hereditary blood disorder that affects millions of people worldwide, especially in sub-Saharan Africa. This condition has a multi-organ involvement and highly vascularised organs, such as the lungs, are particularly affected. Chronic respiratory complications of SCD involve pulmonary vascular, parenchymal and airways alterations. A progressive decline of lung function often begins in childhood. Asthma, sleep-disordered breathing and chronic hypoxaemia are common and associated with increased morbidity. Pulmonary hypertension is a serious complication, more common in adults than in children. Although there is a growing attention towards respiratory care of patients with SCD, evidence regarding the prognostic meaning and optimal management of pulmonary issues in children with this condition is limited.This narrative review presents state-of-the-art evidence regarding the epidemiology, pathophysiology and therapeutic options for chronic respiratory complications commonly seen in paediatric patients with SCD. Furthermore, it highlights the gaps in the current knowledge and indicates future directions for studies that aim to improve our understanding of chronic respiratory complications in children with SCD.

Epidemiology and treatment of relative anemia in children with sickle cell disease in sub-Saharan Africa

2016 ◽  
Vol 9 (11) ◽  
pp. 1031-1042 ◽  
Author(s):  
Halima Bello-Manga ◽  
Michael R. DeBaun ◽  
Adetola A. Kassim
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Sub Saharan

French-Brazilian Survey On Pregnancy in Sickle Cell Disease A Study of the International Sickle Cell Disease Observatory

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3215-3215
Author(s):  
Jean-Antoine Ribeil ◽  
Patrícia Santos Ressende Cardoso ◽  
Aurelie Stanislas ◽  
Vanessa Maria Fenelon Costa ◽  
Benjamin Deloison ◽  
...  
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Caesarean Section ◽  
Pregnant Women ◽  
Sickle Cell ◽  
Infectious Complications ◽  
Transfusion Reaction ◽  
Cell Disease ◽  
Foetal Death ◽  
At Home

Abstract Abstract 3215 Introduction: The International Sickle Cell Disease Observatory (ISCDO) is an international group, established in 2011, including representatives from countries where sickle cell disease (SCD) is highly prevalent, in order to collect and share information of SCD patient's to improve patients care and quality of life, to define common guidelines, to develop advanced targeted approaches and transfer innovative practices worldwide. One of the first ISCDO study is a survey of pregnancy in SCD in France and Brazil. Context: Pregnancy in SCD has been associated with complications and adverse outcomes with an increased incidence of vaso-occlusive, infectious, obstetrical and neonatal complications. Recently, in Paris (France) and Belo Horizonte (BH) (Brazil), integrated care sickle-obstetric units were created, associating sickle cell haematologist, obstetrician and infectious disease specialists, experienced in the care of these high risk pregnancies. Our aim is to compare in two different geographic institutions the prognostic and evolution of SCD in pregnant women with the prospective goal to build up a clinical score in order to better determine appropriate treatment. Methods: We conducted a retrospective study on 253 pregnancies (120 Paris, 133 BH) characterized by 147 Hb SS, 91 Hb SC, 14 Hb SBeta, 2 Hb SD hemoglobinopathy. An e-crf was developed, to screen: the pre-pregnancy, the ante-partum rates of SCD-specific and infectious complications. We compared the obstetrical and the newborns health parameters and complications, the rate of Caesarean section, the perinatal and the maternal mortality in both countries. Results and Discussion: In both populations, 60% of women had a maternal age between 21–30 years old (yo). However, in Brazil there was a higher rate of young pregnant women (14–20 yo) (4% Paris; 20% BH) while in France, patients were older (>31 yo) (36% Paris; 18% BH). In the history of SCD women followed in Paris we noticed that: -Most of these patients had a severe form of SCD with 53% who had experienced an acute chest syndrome and 9% with a symptomatic cerebral vasculopathy, several infectious complications with 26% of pyelonephritis, -A high level of obstetrical complications with 35% of miscarriage and 10% of intrauterine foetal death. The patients followed in Paris during their pregnancy, were treated according to the French guidelines published in 2009. According to these guidelines 67% of patients were transfused and 17% patients were not transfused because of a post-transfusion reaction history. Caesarean section was performed in most cases in both populations (79% in Paris with 23% performed in emergency; 66% in BH). In both populations, there was 1 materno-foetal death. Furthermore, in BH, 15 perinatal deaths and 7 patient deaths were observed. In the Paris' group, there was no other perinatal death and 1 maternal death following a post-transfusional reaction after delivery. The key difference between the 2 study groups concerns the foetal/neonatal morbidity and mortality. These results lead us to compare the 2 health care structures to try to find out the medical guidelines to significantly reduce the frequency of these severe clinical events. In Paris, we introduce oxygenotherapy at home during pregnancy (2l/min) in patients who were transfused because of severe SCD symptomatology (33 patients) and who could not anymore be transfused because of a severe post-transfusion reaction history (11 patients). For these subgroups of patients, we found that 40% of them didn't experience any VOC complications, or preeclampsia. The introduction of oxygenotherapy at home during pregnancy might have a positive impact in reducing the occurrence of a number life threatening complications in these high risk pregnant woman especially when they cannot be appropriately transfused. This study is the first initial step of an international effort by the ISCDO to optimise the treatment of SCD pregnant women, to harmonize the guidelines in different countries and develop new methods of diagnosis and treatment. By improving care and the sharing knowledge of these pregnancies, we would like to increase worldwide access to the development of directed family cord blood banks in families with SCD and the access to hematopoietic stem cell transplant and other innovative therapies in developing and emerging countries where SCD is highly prevalent. Disclosures: No relevant conflicts of interest to declare.

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