Disease and Treatment Knowledge Deficits in Patients with Mild and Moderate Hemophilia May Impact Transition Readiness

Objectives: Demonstration of age appropriate disease and treatment knowledge is important in preparing adolescents with hemophilia for transition to adult care. Our pediatric hemophilia treatment center (HTC) aimed to use quality improvement (QI) methodology to develop a tool to assess and document transition skills in order to foster medical independence in a complex healthcare environment.

Methods: A standardized transition tool questionnaire was developed for adolescent/young adult patients diagnosed with hemophilia A or B followed in the Nationwide Children's Hospital HTC. Key drivers for the project included; identifying age appropriate tool elements, development of an educational resource repository, development of communication strategies and development of educational strategies to address knowledge gaps identified through tool use. The Ohio State University adult HTC actively collaborated in tool development. A final 19 item paper tool was devised to assess the knowledge and skills of adolescent patients in the domains of fundamental hemophilia knowledge (6 items) and hemophilia treatment knowledge and skills (13 items). Each item had a potential score of 0 to 2 for a total score range of 0 to 36 points. An item score of 0 indicated an incorrect, incomplete or blank answer, 1 meant the patient could answer with assistance of family or staff and 2 represented an independently derived correct answer. Patients with hemophilia A or B aged 15-21 years who were seen for comprehensive hemophilia care were eligible to complete the tool with each comprehensive visit up to twice per year. Patients with mild and moderate disease are seen once per year while those with severe disease are seen twice per year. The tool was administered by hemophilia staff using a standard script. Once the tool was completed by the patient the hemophilia staff reviewed the answers with the patient and family and provided directed education for incorrectly answered items. The tool was scored independently by a hemophilia nurse and physician. If there was a discrepancy in scoring the answer was reviewed and the score was adjudicated. The final score results were entered into a password protected data collection instrument. Scores between patient groups were compared using a Mann-Whitney test.

Results: The tool was introduced into clinic April 5, 2016 and data was collected through June 5, 2017. During the review period there were 28 eligible patients seen for comprehensive care and 27 patients completed the tool. In 1 case the source document of a completed tool was lost so this patient was eliminated from analysis. Of the remaining 26 patients there were a total of 36 tools to evaluate, 18 patients completed the tool during 1 clinic visit, 6 completed the tool at 2 separate successive visits and 2 patients completed the tool at 3 successive clinic visits. Twenty-five patients were male, and 1 was female, 17 had hemophilia A and 9 had hemophilia B, 15 had severe hemophilia, 7 had moderate disease and 4 had mild disease. The median baseline score for patients with mild and moderate disease (n=15) was 14 points which was significantly lower (p=0.0156) than those with severe hemophilia (n=21) who had a median score of 27 points. Of the 8 patients with multiple assessments, 5 improved their scores by an average of 9 points, and 3 patients had decreases in scores by an average of 5 points.

Conclusions: Quality improvement methodology was used to successfully develop, implement and sustain use of a standardized transition tool in a pediatric HTC. The project identified knowledge and skill gaps and targeted educational opportunities in addition to fostering improved communication between staff at the pediatric and adult HTCs regarding the important topic of transition of care. The scores highlighted that patients with milder disease were at risk for less adequate knowledge about their hemophilia care than those with severe disease. Next steps include continued utilization of the tool and ongoing evaluation of scores to determine if the tool fosters sustained improvement in disease and treatment understanding as well as development of tools to span the age continuum. Ongoing collaboration between the adult and pediatric HTCs will be needed to determine if the tool increases successful transition to adult care.