HHT Health Initiatives for the 21st Century HHT Health Initiatives for the 21st Century Conference Overview & Objectives Conference Overview & Objectives Mark S. Chesnutt, M.D. Mark S. Chesnutt, M.D. Director, Critical Care, Portland VA Medical Center Director, Critical Care, Portland VA Medical Center Director, HHT Center of Excellence, Dotter Interventional Institute Director, HHT Center of Excellence, Dotter Interventional Institute Associate Professor of Medicine, Pulmonary & Critical Care Medicine Associate Professor of Medicine, Pulmonary & Critical Care Medicine Oregon Health & Sciences University Oregon Health & Sciences University
HHT Health Initiatives for the 21st Century. Conference Overview & Objectives. Mark S. Chesnutt, M.D. Director, Critical Care, Portland VA Medical Center Director, HHT Center of Excellence, Dotter Interventional Institute Associate Professor of Medicine, Pulmonary & Critical Care Medicine - PowerPoint PPT Presentation
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HHT Health Initiatives for the 21st CenturyHHT Health Initiatives for the 21st Century
Director, Critical Care, Portland VA Medical CenterDirector, Critical Care, Portland VA Medical Center
Director, HHT Center of Excellence, Dotter Interventional InstituteDirector, HHT Center of Excellence, Dotter Interventional Institute
Associate Professor of Medicine, Pulmonary & Critical Care MedicineAssociate Professor of Medicine, Pulmonary & Critical Care Medicine
Oregon Health & Sciences UniversityOregon Health & Sciences University
Mark S. Chesnutt, M.D.Mark S. Chesnutt, M.D.
Director, Critical Care, Portland VA Medical CenterDirector, Critical Care, Portland VA Medical Center
Director, HHT Center of Excellence, Dotter Interventional InstituteDirector, HHT Center of Excellence, Dotter Interventional Institute
Associate Professor of Medicine, Pulmonary & Critical Care MedicineAssociate Professor of Medicine, Pulmonary & Critical Care Medicine
Oregon Health & Sciences UniversityOregon Health & Sciences University
HHT Health Initiatives for the 21st CenturyHHT Health Initiatives for the 21st Century
Purpose of the ConferencePurpose of the Conference
Bring together individuals from a broad range of disciplines to Bring together individuals from a broad range of disciplines to work to:work to:
1. increase awareness of HHT in the United States1. increase awareness of HHT in the United States
2. develop a plan to:2. develop a plan to:- improve early recognition and intervention- improve early recognition and intervention- improve clinical outcomes of persons with HHT- improve clinical outcomes of persons with HHT
Ultimate goalsUltimate goals• reduce health and economic impact of HHTreduce health and economic impact of HHT• enhance quality of life of persons with HHTenhance quality of life of persons with HHT
HHT Health Initiatives for the 21st CenturyHHT Health Initiatives for the 21st Century
Participants in this processParticipants in this process
Representatives from governmental and non-governmental organizations Representatives from governmental and non-governmental organizations with interests in patient care and researchwith interests in patient care and research
Representatives from health care groups that are likely to encounter Representatives from health care groups that are likely to encounter persons with HHT and who might be able to assist in early recognition, persons with HHT and who might be able to assist in early recognition, diagnosis and interventiondiagnosis and intervention
• Defect in development of some blood vessels Defect in development of some blood vessels (capillaries), forming:(capillaries), forming:- telangiectasestelangiectases- arteriovenous malformations or fistulas (AVM / AVF)arteriovenous malformations or fistulas (AVM / AVF)- other vascular lesionsother vascular lesions
• Wide range of clinical manifestations/presentationsWide range of clinical manifestations/presentations
HHT Health Initiatives for the 21st CenturyHHT Health Initiatives for the 21st Century
Why spend time, energy and resources on HHT?Why spend time, energy and resources on HHT?
• Prevalence in US:Prevalence in US:- ? 1:5,000-10,000- ? 1:5,000-10,000
- Up to 75,000 Americans may have HHT- Up to 75,000 Americans may have HHT
• Variable clinical presentation and health care Variable clinical presentation and health care provider awareness makes diagnosis of HHT provider awareness makes diagnosis of HHT challengingchallenging
• Most serious complications (including death in Most serious complications (including death in 10-20% of persons with HHT) can be 10-20% of persons with HHT) can be prevented by screening and treatment using prevented by screening and treatment using successful approaches and techniquessuccessful approaches and techniques
HHT Health Initiatives for the 21st CenturyHHT Health Initiatives for the 21st Century
PreventablePreventable HHT-related Healthcare Costs HHT-related Healthcare CostsEstimated costs per incidentEstimated costs per incident
(Data from 18 Years of Yale HHT Center Statistics)
HHT Health Initiatives for the 21st CenturyHHT Health Initiatives for the 21st Century
The path to Atlanta………The path to Atlanta………
• First descriptions - late 1800’s• Advances in imaging and interventional radiology - 1970’s• Birth of HHT Foundation - late 1980’s, incorporated 1991• Formation of Scientific and Medical Advisory Board - 1992• Grass roots efforts of persons & families with HHT - early 1990’s• Establishment of new HHT Centers of Excellence• Annual patient care conferences• International Scientific meetings - significant growth• Identification of genes for HHT - mid-1990’s• Analysis of impact on morbidity and mortality• Successful HHT Foundation fund raising to support research• International Guidelines Consensus Conference - 2006• NIH HHT Research Conference - 2006• Ongoing efforts to increase funding of clinical care and research• Congressional language established CDC and NIH relationships
Familial EpistaxisFamilial Epistaxis
Henry Gawen Sutton, M.D.Henry Gawen Sutton, M.D.
Sutton HG: Medical Mirror: 1864 (London) 1, 769-781Sutton HG: Medical Mirror: 1864 (London) 1, 769-781
First report suggestive of HHTFirst report suggestive of HHT
• Four patients with familial epistaxis Four patients with familial epistaxis and telangiectasias of the skin and and telangiectasias of the skin and mucus membranesmucus membranes
• Differentiation from hemophilia since Differentiation from hemophilia since bleeding occurred only from sites bleeding occurred only from sites with telangiectasiaswith telangiectasias
• Referenced articles of Babington Referenced articles of Babington and Renduand Rendu
HHT Foundation InternationalHHT Foundation InternationalMission: to find a cure for HHT while saving the lives and improving the Mission: to find a cure for HHT while saving the lives and improving the
well-being of individuals and families affected by HHT.well-being of individuals and families affected by HHT.
To achieve this mission, the HHT Foundation will:To achieve this mission, the HHT Foundation will:
Fund researchFund research to find better treatments and a cure. to find better treatments and a cure.
Educate families and physiciansEducate families and physicians about HHT so that awareness of crucial about HHT so that awareness of crucial diagnosis and available treatments prevents needless disability and death.diagnosis and available treatments prevents needless disability and death.
Provide linkages between people affected by HHT.Provide linkages between people affected by HHT.
Collaborate with multidisciplinary HHT Treatment Centers worldwideCollaborate with multidisciplinary HHT Treatment Centers worldwide while advocating for patient access to these Centers.while advocating for patient access to these Centers.
Advocate for and support those with HHTAdvocate for and support those with HHT while increasing public, private, and governmental awareness of the disorder.while increasing public, private, and governmental awareness of the disorder.
Engage the scientific and medical communityEngage the scientific and medical community so that talented individuals dedicate efforts toward advances in HHT screening, so that talented individuals dedicate efforts toward advances in HHT screening, diagnosis, treatment, and research.diagnosis, treatment, and research.
HHT Centers in North AmericaHHT Centers in North America
* Univ of California, San Diego
* Univ of Utah
* * OHSU
*Mayo Clinic
*Washington University
*Toronto*Toronto
* Univ of * Univ of PennsylvaniaPennsylvania
* Medical * Medical College of College of GeorgiaGeorgia
*Yale*Yale
HHT Foundation efforts have included creation of HHT Centers of HHT Foundation efforts have included creation of HHT Centers of Excellence and direction of HHT patients to these centersExcellence and direction of HHT patients to these centers
Patient Care ConferencesPatient Care ConferencesNational = 13National = 13 Regional = 3Regional = 3
International Scientific ConferencesInternational Scientific Conferences7 since 19967 since 1996
International diagnostic criteria consensus- 1997International diagnostic criteria consensus- 1997
Liver guidelines - 2005Liver guidelines - 2005
Other EffortsOther Efforts2 Hands-on Physician Training Programs2 Hands-on Physician Training Programs
International Clinical Guidelines ConferenceInternational Clinical Guidelines Conference
Facilitation of Genetic Testing in the USFacilitation of Genetic Testing in the US
7 research grants totaling $450,000; one leveraged a $1 7 research grants totaling $450,000; one leveraged a $1 million FDA grantmillion FDA grant
11 Young Investigator Grants11 Young Investigator Grants
HHT Language Reported by House and HHT Language Reported by House and Senate Appropriations Committee- FY 2008Senate Appropriations Committee- FY 2008
H.R. 3043, The Labor, HHS., Education and Related Agencies H.R. 3043, The Labor, HHS., Education and Related Agencies Appropriation Bill Appropriation Bill Encourages the CDC to establish an HHT resource center to increase identification of people with HHT, and increase knowledge, education and outreach of this largely preventable life-threatening condition. The Committee encourages CDC to provide information on effective evidence-based interventions and treatments to prevent premature death in the HHT population, improve outcomes and the quality of life for people living with HHT by creating a database to collect and analyze data, support epidemiology studies, provide surveillance, and train health care professionals.
S. 1710, The Labor, HHS., Education and Related Agencies S. 1710, The Labor, HHS., Education and Related Agencies Appropriation BillAppropriation BillThe Committee is aware of interest in the establishment of a Hereditary Hemorrhagic Telangiectasia National Resource Center. The Committee encourages the CDC to examine carefully proposals to establish such a center.
HHT Health Initiatives for the 21st CenturyHHT Health Initiatives for the 21st Century
The path to Atlanta………The path to Atlanta………
• First descriptions - late 1800’s• Advances in imaging and interventional radiology - 1970’s• Birth of HHT Foundation - late 1980’s, incorporated 1991• Formation of Scientific and Medical Advisory Board - 1992• Grass roots efforts of persons & families with HHT - early 1990’s• Establishment of new HHT Centers of Excellence• Annual patient care conferences• International Scientific meetings - significant growth• Identification of genes for HHT - mid-1990’s• Analysis of impact on morbidity and mortality• Successful HHT Foundation fund raising to support research• International Guidelines Consensus Conference - 2006• NIH HHT Research Conference - 2006• Ongoing efforts to increase funding of clinical care and research• Congressional language established CDC and NIH relationships
Despite efforts to date, many persons with Despite efforts to date, many persons with HHT remain undiagnosed and experience HHT remain undiagnosed and experience
significant morbidity and mortalitysignificant morbidity and mortality
HHT Health Initiatives for the 21st CenturyHHT Health Initiatives for the 21st Century
Specific Objectives of the ConferenceSpecific Objectives of the Conference
To define and prioritize strategies to:To define and prioritize strategies to:
1.1. Determine the prevalence of HHT in the United StatesDetermine the prevalence of HHT in the United States2.2. Disseminate HHT Evaluation and Treatment GuidelinesDisseminate HHT Evaluation and Treatment Guidelines3.3. Increase early detection and diagnosis of persons with HHTIncrease early detection and diagnosis of persons with HHT4.4. Form effective partnerships and collaborationsForm effective partnerships and collaborations5.5. Increase funding for care of persons with HHTIncrease funding for care of persons with HHT
HHT Health Initiatives for the 21st CenturyHHT Health Initiatives for the 21st Century
Conference agendaConference agenda
General sessionsGeneral sessionsOverview of HHT Screening and DiagnosisOverview of HHT Screening and DiagnosisHHT GuidelinesHHT GuidelinesSurveillance Approaches & StrategiesSurveillance Approaches & Strategies
WorkshopsWorkshopsSurveillance to determine prevalenceSurveillance to determine prevalenceDissemination of HHT GuidelinesDissemination of HHT GuidelinesEarly diagnosis and interventionEarly diagnosis and intervention
Workshop Summaries and Future DirectionsWorkshop Summaries and Future DirectionsRecommendations for Next steps - Group ConsensusRecommendations for Next steps - Group ConsensusPotential Funding Sources and First WinsPotential Funding Sources and First Wins
Roadmap for HHT Public Health Initiatives for the 21st CenturyRoadmap for HHT Public Health Initiatives for the 21st Century