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Pediatric Sickle Cell Disease in Sudan: Complications and Management
Meysaa Talha1, Bashier Osman2, Safa Abdalla2
1Clinical Pharmacy Program, Faculty of Pharmacy, University of Khartoum, Khartoum, Sudan.
Insights
Sickle cell disease (SCD) management in Sudanese children largely aligns with global standards, though vaccination and prophylactic penicillin use require improvement. This study highlights key interventions like hydroxyurea and blood transfusions effectively reducing SCD complications.
Area of Science:
- Hematology
- Pediatric Medicine
- Public Health
Background:
- Sickle cell disease (SCD) is a severe genetic disorder characterized by abnormal hemoglobin (HbS) polymerization under hypoxia, leading to significant complications.
- Current management strategies for SCD encompass symptomatic, supportive, and preventive approaches.
Purpose of the Study:
- To evaluate the current management practices for pediatric sickle cell disease patients at Gaafar Ibnauf Referral Hospital in Khartoum, Sudan.
- To assess the effectiveness of implemented interventions and identify areas for improvement in SCD care.
Main Methods:
- A descriptive, retrospective, hospital-based study was conducted.
- Data were collected from medical files of pediatric SCD patients treated between April and July 2018.
- Statistical analysis included descriptive statistics and the chi-square test, with P < 0.05 considered significant.
Main Results:
- The study included 207 pediatric patients, predominantly females (53.1%) from low socioeconomic backgrounds, with limited health insurance coverage (4.3%).
- Common complications included vaso-occlusive crisis (33.3%), infections (13.5%), and neurological issues (10.6%).
- Key treatments involved folic acid (100%), hydroxyurea (73.9%), and blood transfusions (69.6%), which significantly reduced fever and vaso-occlusive crises. Immunization rates with pneumococcal vaccine (PPSV23) were 41.1%, and prophylactic penicillin was given to 15%.
Conclusions:
- The SCD management protocol at Gaafar Ibnauf Children's Hospital aligns with international standards, incorporating effective preventive and symptomatic therapies.
- While treatments like hydroxyurea and blood transfusions show positive outcomes, there are deficiencies in immunization and prophylactic penicillin administration, indicating a need for enhanced preventive strategies.
Background:
Sickle cell disease (SCD) is a life-threatening genetic disorder due to the formation of sickle hemoglobin molecule (HbS) that polymerizes in hypoxic conditions leading to SCD-related complications. Different approaches have been used in the management of SCD including symptomatic management, supportive management, and preventive management.
Objectives:
To assess the management of SCD in pediatric patients in Gaafar Ibnauf Referral Hospital in Khartoum locality, Sudan.
Method:
A descriptive, retrospective, hospital-based study was conducted in Gaafar Ibnauf Hospital using a data collection sheet. The study included all medical files of pediatric patients with SCD attending the hospital during the period from the first of April 2018 to the first of July 2018. The data were analyzed using descriptive statistics and the chi-square test. P < 0.05 was considered statistically significant.
Results:
Out of 207 pediatric patients, 53.1% were females (mean age of 7.5 ± 3.1 years), with a 1.1 : 1 female:male ratio and low socioeconomic status. Only 4.3% of participants had health insurance. The Messeryia tribe in western Sudan had the highest prevalence of the disease among the Sudanese tribes (11.1%). Vaso-occlusive crisis (33.3%), infections (13.5%), and neurological complications (10.6%) were the most frequent complications reported during routine visits. After initiation of management, only 3.4% of pediatric patients had hemolytic crises, and 1.4% of the anemic patients had splenomegaly. 100% of patients received folic acid, 73.9% used hydroxyurea, and 69.6% underwent blood transfusion for the management of SCD. Prophylactic penicillin was prescribed for 15% of patients, and 41.1% were immunized with pneumococcal vaccine (PPSV23). Most patients had been scheduled for planned follow-up visits every 3-6 months (93.2%). Hydroxyurea and blood transfusion significantly reduced fever and vaso-occlusive crisis.
Conclusion:
The SCD treatment protocol in Gaafar Ibnauf Children's Hospital, involving preventive and symptomatic therapy, is consistent with the internationally implemented protocols for SCD management. However, immunization and prophylactic penicillin approaches are deficient.
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