Funded by Foundation

The purpose of this project is to request support for the development of a computer-based training (CBT) module designed to teach the care and treatment of patients with bleeding disorders to nursing staff in a large, multi-campus pediatric healthcare institution. The module will be designed to exist within an intranet environment accessed by all members of the healthcare institution. Core modules will include a description of each common bleeding disorder, a basic review of processes surrounding the multidisciplinary care of bleeding disorder patients, the involvement of other hospital departments in managing a patient with a bleeding treatment plan (e.g., blood bank, coagulation lab, hemophilia treatment center staff) and medications/factor products routinely used to treat them. The result, a custom-made bleeding disorder CBT, would be available at any time to any staff member on any of the institution's campuses being assigned to care for bleeding disorder patients, thus facilitating nursing education and patient care.

Despite the fact that adults with hemophilia fit the profile of a population at high risk for experiencing depression, not to mention the significant impact of depression on their overall well-being, there are almost no studies in the literature to document the incidence of depression for these patients. This study is designed to document the prevalence of depression in adults with hemophilia at our treatment center and to evaluate risk factors for depression in these patients as well as patients from Region VIII who have completed the Centers for Disease Control and Prevention's Quality of Life questionnaire. Evaluation of the information collected is expected to show that a significant percentage of adults with hemophilia are experiencing symptoms of depression. The Arizona Hemophilia and Thrombosis Center plans to use this information to apply for future funding to support the development of a depression care management plan, which will be incorporated into standard care.

Our goal is to establish an outreach clinic in an area that will serve the Amish bleeding disorder community. After the completion of our formal project, we plan to continue to offer our services to the community to provide ongoing education, screening, and testing for individuals with bleeding symptoms or with a family history of a bleeding disorder. Furthermore, we plan to extend our clinical support to the other Amish communities located in downstate Illinois.

Paul Monahan is Translational and Clinical Development Lead for Hematology Gene Therapy at Spark Therapeutics. Prior to 2015, Dr. Monahan was Professor of Pediatrics, Hematology/Oncology at the University of North Carolina at Chapel Hill, where he spent more than 20 years as an Investigator in the UNC Gene Therapy Center and treating children with bleeding disorders as an Attending Physician in the Harold Roberts Hemophilia Diagnostic and Treatment Center. He served on several clinical medical and scientific foundations and committees including the Board of Directors of the Hemostasis and Thrombosis Research Society. For ten years he served on the Medical and Scientific Advisory Committee of the National Hemophilia Foundation and as the Region IV Director for the US Hemophilia Treatment Center Network (CDC and MCHB). His basic science laboratory maintained a research focus on gene therapy for hemophilia as well as animal models for the study of hemophilic bone and joint disease, inhibitors in hemophilia B, and novel therapies from 1996 through 2016. In 2013 the National Hemophilia Foundation awarded Monahan the NHF Leadership in Research Award. Prior to joining Spark in Spring 2018, he performed preclinical research development and coordinated clinical trial initiation of hemophilia B and hemophilia A gene therapy trials in a collaborative partnership with Asklepios Biopharmaceuticals and as Medical Lead at Baxalta/Shire.

Per Dr. Kempton, the JGP assisted her in determining the direction of her future research. As a result of her JGP project, Dr. Kempton was published in ATVB and published several abstracts.