Hereditary Spherocytosis

Research & News

As leaders in the diagnosis and treatment of hereditary spherocytosis, Dr. Buchanan and his staff at Children’s have contributed numerous professional articles on the disease and related disorders of the blood to prestigious medical publications. These scientific articles are recognized by their peers and are read worldwide. A shortened bibliography follows.

The physicians and staff in the Center for Cancer and Blood Disorders at Children’s believe that research helps provide a better understanding of medical conditions and improves the care that they give. Therefore, the staff at Children’s is actively involved in conducting research projects involving hereditary spherocytosis (HS) and participates in research on a national level with other experts.

The Hematology Clinical Research team at Children’s includes five specialists who are focused on coordinating research in sickle cell anemia, hemophilia, thrombosis and general hematology (such as immune thrombocytopenia, hereditary spherocytosis and neutropenia). Additionally, two clinical research nurses are dedicated full-time to hematology studies.

These research teams involved with blood diseases and cancer are instrumental members of the multi-pronged medical care team. Many of the clinical trials are sponsored by the National Institutes of Health (NIH) and the Children’s’ Oncology Group, among others. Institutional Review Board (IRB) approval is maintained for all research, with informed consent documents and treatment and outcome recording for all patients enrolled. The Food and Drug Administration, the sponsors and the IRB may review all records to assure the quality and accuracy of the research documentation. The team in the Center for Cancer and Blood Disorders is currently responsible for data collection of approximately 195 active research studies.

For instance, Dr. Shelley Crary’s team is interested in the long-term outcomes of spleen removal for HS. There are few current studies looking at the long-term risks and benefits of splenectomy. Her group also is conducting a longitudinal study on the impact of HS on patient and family’s daily lives that will collect clinical and laboratory information as well as use questionnaires to determine the patient and family’s quality of life.

Research

Vascular complications after splenectomy for hematological disorders, Blood, review by Shelley Crary, M.D., and George Buchanan, M.D.

Laboratory markers of thrombosis risk in children with hereditary spherocytosis, Pediatric Blood Cancer, Shelley Crary, M.D., George Buchanan, M.D., Sarah Troendle, M.D., Leah Adix, BS, CCRP

Prospective Assessment of Risk Factors for Thrombosis in Persons with Hereditary Spherocytosis, Pediatric Blood and Cancer 2010

Splenic infarction due to concomitant hereditary spherocytosis and sickle cell trait, George Buchanan, M.D., et al, Journal of Pediatric Surgery

News

Children’s Medical Center ranked among Top 10 pediatric hospitals, U.S.News & World Report