Related Experiment Video
Updated: Aug 9, 2025
05:23
Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
19.6K
Most adults with severe HbSC disease are not treated with hydroxyurea
William Kwesi Ghunney1,2, Eugenia Vicky Asare1,2, John Benaiah Ayete-Nyampong3
1Ghana Institute of Clinical Genetics, Korle-Bu, Accra, Ghana.
Blood Advances
|February 17, 2023
Summary
Sickle cell hemoglobin SC (HbSC) disease affects many adults, with 10% meeting severe disease criteria and eligible for hydroxyurea. However, less than 1% receive this treatment, indicating a gap in care for HbSC patients.
Area of Science:
- Hematology
- Genetics
- Public Health
Background:
- Sickle cell hemoglobin SC (HbSC) disease is the second most common sickle cell disease (SCD) genotype globally.
- Hydroxyurea is not routinely recommended for HbSC due to perceived risks, unlike in sickle cell anemia (HbSS).
- Ghana has a high prevalence of HbSC, with 40% of SCD clinic patients affected.
Purpose of the Study:
- To determine the proportion of adults with HbSC meeting criteria for severe disease.
- To assess eligibility for hydroxyurea therapy in adults with HbSC.
- To compare severe disease prevalence and hydroxyurea prescription rates between HbSC and HbSS.
Main Methods:
- Retrospective descriptive cohort study of adults (≥18 years) with HbSC visiting a Ghanaian clinic in 2019.
- Matched comparison group of individuals with HbSS based on age and sex.
- Severe disease defined by pain episodes, acute chest syndrome, and chronic anemia impacting daily life.
Main Results:
- 10.0% (64/639) of adults with HbSC met severe disease criteria and were eligible for hydroxyurea.
- 24.1% (154/639) of adults with HbSS met severe disease criteria.
- Less than 1% of severe HbSC patients and 3% of severe HbSS patients were prescribed hydroxyurea.
Conclusions:
- A significant proportion of adults with HbSC disease experience severe manifestations, qualifying them for hydroxyurea therapy.
- There is a substantial gap in hydroxyurea prescription for eligible HbSC patients in this tertiary care setting.
- Further research and clinical guidelines are needed to optimize HbSC management.
Related Concept Videos
01:21Rheumatic Heart Disease III: Medical Management
24
Rheumatic heart disease (RHD) management can be divided into two main strategies: prevention and long-term management.Primary PreventionPrimary prevention focuses on timely diagnosis and management of group A streptococcal pharyngitis to prevent acute rheumatic fever. The most widely used antibiotic for treating this condition is intramuscular benzathine penicillin G.Acute Rheumatic Fever TreatmentThe primary treatment goal for a patient diagnosed with acute rheumatic fever is to suppress the...
24
01:29Cardiomyopathy V: Interprofessional Care
22
Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
22
01:30Heart Failure V: Medical Management
19
Medical Management of Acute Decompensated Heart Failure (ADHF)The primary goals of therapy for patients hospitalized with acute decompensated heart failure (ADHF) include:Relieving symptomsOptimizing volume statusSupporting oxygenation and ventilationMaintaining cardiac output (CO) and end-organ perfusionIdentifying and addressing the cause of ADHFPreventing complicationsProviding patient education on factors precipitating HF exacerbationPlanning for dischargeOngoing monitoring and assessment...
19
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy
21
Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
21
01:19Multipotency of Hematopoietic Stem Cells
3.2K
The hematopoietic stem cells or HSCs are multipotent, meaning they can differentiate and give rise to all blood and immune cells. HSCs are maintained in the quiescent stage until an external stimulus initiates their differentiation. The multipotent HSCs exist as two heterogeneous populations, long-term repopulating cells (LTRC) and short-term repopulating cells (STRC). The two HSC populations have different surface markers or receptors and are classified based on quiescence and long-term...
3.2K
01:29Venous Thrombosis III: Interprofessional Care
15
Venous thrombosis requires effective prevention and treatment strategies to improve patient outcomes and reduce potential complications.Prevention StrategiesHealthcare providers must prioritize preventing venous thromboembolism (VTE) for all adult patients upon admission. Interventions depend on bleeding and thrombosis risk, medical history, current medications, diagnoses, planned procedures, and patient preferences. Patients on bed rest should change positions every two hours and, if not...
15