Development and evaluation of a sickle cell assessment instrument

The current ability to predict very young children with sickle cell disease who are likely to have severe complications later in life permits accurate tailoring of therapy to match disease-related risks. A valid and reliable instrument is essential to accurately assess these children prior to referring them to therapies that are not without risk. This study was designed to develop a Sickle Cell Disease Assessment Instrument with two domains-high-risk identification and disease severity classification-and to evaluate instrument validity and reliability. Instrument development involved identification and definition of critical attributes, assignment of numerical values to critical attributes, and development of a scoring system. Content validity was measured using a panel of five experts in the field of sickle cell disease. Registered nurses using the equivalence approach determined interrater reliability and using test-retest design determined stability. Nurses are often the first persons to identify sickle cell patients that need additional intervention. This instrument will allow them to accurately and objectively assess their patients for high-risk indicators and disease severity classification.