David and Clara Bartley Jordan Epistaxis Research Grant Award
Development of a Standardized Epistaxis Severity Score (ESS) in HHT in Order to Validate Therapy Efficacy
Dr. Jeff Hoag
Johns Hopkins University School of Medicine and Drexel University School of Medicine
This study is awarded to Dr. Jeffrey Hoag of Drexel University School of Medicine and will develop a uniform epistaxis severity scoring system. This system will be used to assess the effectiveness of specific treatments for HHT-related epistaxis. HHT-Epistaxis Severity Scores will help to validate current treatment algorithms, and serve as a benchmark for the development of future medical and surgical treatment strategies. The study will involve a population of 245 participants with a varying range of epistaxis severity which will provide an adequate representation of the population of patients with HHT-related epistaxis.
Specific Aim 1: The study will try to determine the factors associated with epistaxis in HHT. A standardized list of patient perceived important variables combined with provider perceived important variables will provide a comprehensive list of factors associated with HHT-related epistaxis. This list will be developed with patient care providers from HHT Centers of Excellence and Patient Focus Groups through the support of the HHT Foundation International.
Specific Aim 2: (Epistaxis Severity Score) This study will develop a uniform epistaxis severity scoring system.
Specific Aim 3: To validate the HHT-Epistaxis Severity Score through correlation with standardized quality of life (QOL) assessments and statistical models.
Specific Aim 4: (Assessment of Epistaxis Treatment) To use the HHT-Epistaxis Severity Score to assess the effectiveness of specific treatments for HHT-related epistaxis. HHT-Epistaxis Severity Scores will help to validate current treatment algorithms, and serve as a benchmark for the development of future medical and surgical treatment strategies.
Outcome: Dr. Hoag presented his research at the HHT Scientific Meeting held in Santander, Spain in 2009 and published his findings in the April 2010 edition of Laryngoscope. The Epistaxis Severity Scoring Tool developed by Dr. Hoag is used by ENT's at HHT Treatment Centers around the world to determine severity of nosebleeds and improvements realized from laryngology treatments, including the 2011 NOSE Study.
ESS Published Article - Laryngoscope 120: April 2010
This study was made possible through a generous donation from David and Clara Barley Jordan and many other HHT Foundation members who made donations to the HHT Epistaxis Fund.
Mayo Clinic Team Leverages $50K HHT Foundation Research Award to $1 Million
Dr. Karen Swanson, D.O., Director of the Mayo Clinic HHT Center
Dr. Swanson has been approved for funding from the Office of Orphan Products Development within the Food and Drug Administration (FDA) for an interferon protocol. Dr. Swanson's budget is now $1 million over three years. The study, entitled "Interferon-2B Treatment in Hereditary Hemorrhagic Telangiectasia," was directed towards determining whether interferon may provide a safe and effective treatment for HHT. A certain type of interferon has already been shown to have anti-angiogenic properties and is currently used in the treatment of infantile hemangiomas and liver hemangiomas. Both of these are diseases of abnormal blood vessel formation similar to the teleangiectases and AVMs that occur in HHT. To everyone who donated generously to the HHT Foundation so that we could provide that initial $50,000, without which the larger grant would never have been approved, we offer our wholehearted thanks on behalf of all of those with HHT.