Introduction
Sickle Cell Disease (SCD) is a group of inherited blood disorders caused by a mutation in the beta subunit of hemoglobin (HbS). SCD will hereafter be referred to as Sickle Cell Anemia (SCA) as this is the term our patients and their families prefer. There are approximately 5000 Canadians living with SCA (1). The mutated B subunit causes polymerization of hemoglobin leading to sickling of red blood cells. Sickle cells are prone to destruction and clog blood vessels resulting in ischemia (2, 3). SCA is associated with anemia and vaso-occlusive pain crises (VOCs) that can lead to complications involving the bones, lungs, abdomen, brain, eyes and reproductive system (4). Life-threatening events are also common and include acute chest syndrome (ACS), ischemic stroke and infection (4).
Pediatric SCA patient education can: improve knowledge, decrease hospitalization, improve medication possession ratio, lead to better SCA related functioning, and lower pain impact (5-10). However, there is a lack of information with features desirable to adolescents on the internet written at an appropriate reading level (11). In particular, there is a lack of information about management and prevention of emergencies like ACS (11). There is a need to develop age-appropriate educational materials for patients with SCA and their families.
Furthermore, educational materials are an important component of transition to adult care and should be provided to patients starting from a young age. Acute care visits and rehospitalizations are frequent in those with SCA 18 -30 years old (10). Patients are vulnerable at this time and equipping youth with knowledge and self-efficacy of illness management is critical.