Research Studies

The primary purpose of our project will be to provide education that is specific to adolescent females diagnosed with a bleeding disorder. Most of the information available in the bleeding community is gender specific to males, age specific to adults and focused on von Wilebrand Disease and not platelet dysfunctions. In order for these young girls to form a concrete knowledge base that they can build upon through their adult life, they need additional educational resources for their diagnosis and prescribed therapy as well as support.

The purpose of this pilot study is to characterize social integration of persons with congenital bleeding disorders (PWCBD), specifically adult males with hemophilia A or B. Due to early onset of bleeding symptoms, they are predisposed to acute, life-threatening bleeding, bleeding complications, risk for long-term disabling hemophilic arthropathy, and blood-borne infections. A European study has shown that adult males with hemophilia have inferior socialization as measured by work, work disability and health-related quality of life when compared to adult men in the general population. We hypothesize that a broader view of socialization components also contribute to quality of life. We also propose that the role of the spouse or significant other may have relevance for current social work goals in providing comprehensive care for adult PWCBD.

Dr. Pels joined the hemophilia treatment center at Yale University as a pediatric hematology/oncology resident in 2004 after receiving her undergraduate degree from Johns Hopkins and her MD from the University of Vermont College of Medicine. Her recently-authored poster abstract on immune thrombocytopenia was selected for presentation at the 2009 Plenary Scientific Session of the Hemophilia and Thrombosis Research Society's Annual Meeting. As an NHF-Baxter Clinical Fellow, Dr. Pels will spend two years developing advanced skills in clinical hemostasis and learning rigorous methodologies of clinical trial design, completion and monitoring. She will also continue her work on several ongoing studies under the mentorship of Dr. Diana Beardsley. Dr. Pels intends to pursue a career that includes both academics and research, including clinical work in the broad areas of hemostasis and thrombosis. She is hopeful that this work will lead to better therapies for patients with hemophilia as well as other bleeding and clotting disorders.

Dr. Boles is currently a hematology and oncology fellow with the University of North Carolina at Chapel Hill, where he received both his BS and MD. His fellowship mentor at UNC will be Dr. Nigel Key. Also involved in Dr. Boles' training will be Dr. Raj Kasthuri, a former NHF Clinical Fellowship recipient. The co-author of a number of publications, including many in the field of neuroscience, Dr. Boles has also been honored for his teaching skills. Training received during the course of the program will include rigorous laboratory research as well as career guidance in hemostasis. Dr. Boles hopes to continue his career in hematology post-fellowship and conduct research that will improve the lives of patients with hemophilia and other bleeding disorders.

Dr. Flood earned her undergraduate degree from Harvard University in 1995 and her doctorate from Tufts University in 1999. She is board certified in pediatrics as well as pediatric hematology/oncology. She has been serving as an Assistant Professor at the Medical College of Wisconsin, where she has been performing research and seeing patients since 2006.

As part of her NHF Career Development Award, Dr. Flood will study the common variants and interactions of the von Willebrand factor molecule as a means to enhance overall understanding and eventually improve upon current methods of von Willebrand disease testing. Using mouse models, she will investigate diagnostic alternatives by examining the interaction between ristocetin and its ability to bind to von Willebrand factor. As Dr. Flood states in her summary, "Improved testing for VWD will prevent patients with normal VWF function from receiving unnecessary treatment, while allowing more accurate assessment of patients with true defects in VWD."

Nurses and health care providers recommend Emergency Medical Identification (EMI) such as Medic Alert for children of all ages with hemophilia and other bleeding disorders, but most are unsure of what type should be used and where they should be located. This is because there are no guidelines on how EMI should be worn. This project will develop guidelines for the use of Emergency Medical Identification (EMI) and develop educational information for families of children with hemophilia. Guidelines will show recommended ages to wear EMI, its use with infants, and where it should be worn or if it should be carried. In addition, this project will review barriers for not wearing EMI based on our research, including perceived lack of need, sizing concerns for infants, and the fear of being different as expressed by adolescents.

This study will collect data from interviews with women who have moderate or severe hemophilia or another congenital bleeding disorder. Participants will be asked to express the impact that a congenital bleeding disorder has on the development and maintenance of identity and social relationships. It will provide women a chance to share how they feel about the impact that living with a bleeding disorder has on their lives. The results of this study will be of benefit to treatment providers and the entire community as we come to better understand the unique experiences of this segment of the bleeding disorders population.

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.

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.