Variability in sickle cell knowledge by sickle cell status

Area of Science:

• Genetics and Hereditary Diseases
• Public Health and Epidemiology
• Medical Education

Background:

• Sickle cell disease (SCD) and sickle cell trait (SCT) require comprehensive knowledge for informed decision-making and support within affected families.
• Understanding of SCD and SCT varies among family members based on their disease status.
• Current educational approaches may not adequately reach all family members, particularly those without the trait or disease.

Purpose of the Study:

• To assess variations in sickle cell-related knowledge across different disease statuses within families affected by SCD.
• To identify specific knowledge gaps, particularly concerning sickle cell trait and inheritance patterns.
• To inform the development of more effective, family-centered educational strategies.

Main Methods:

• A cross-sectional study involving 179 participants from 84 families affected by SCD.
• Data collection through online surveys and telephone interviews.
• Statistical analysis using generalized linear models with generalized estimating equations to compare knowledge scores based on sickle cell status.

Main Results:

• Participants with negative or unknown sickle cell status demonstrated significantly lower knowledge scores compared to those with SCD or SCT.
• A notable deficit in understanding sickle cell trait and autosomal recessive inheritance patterns was observed across participants.
• Overall performance on the Sickle Cell Knowledge Scale indicated widespread knowledge gaps, especially regarding SCT.

Conclusions:

• Findings highlight the need for family-centered, rather than solely patient-centered, educational interventions for sickle cell conditions.
• Educational efforts should specifically target individuals with sickle cell trait and those with negative or unknown status.
• Addressing knowledge gaps in sickle cell trait and inheritance patterns is essential for comprehensive sickle cell disease education.