This comprehensive document details international recommendations for the diagnosis of HHT and for related AVMs, by organ. It also details recommendations for management of themulti-system manifestations of the disorder. The HHT Guidelines were developed with experts from 13 countries, using AGREE methodology for pre-meeting evidence search, retrieval and abstraction, and GRADE methodology for the recommendation development process.

The guidelines development process also involved HHT patients. HHT is a not-so-rare (1/5000) autosomal dominant disease which unfortunately remains under-diagnosed and under-treated. The development and publication of these evidence-based consensus guidelines are the first step towards improving the standard of care for HHT patients.

PLEASE NOTE THE FOLLOWING DISCLAIMER REGARDING THE CONTENT OF THIS SITE AND ANY LINKS FOUND ON THIS SITE.

The information (in particular, the medical and scientific information) contained in the HHTFI website is provided with the understanding that the HHTFI is a voluntary health organization and is not engaged in rendering medical advice or recommendations on diagnosis or treatment of the HHT syndrome. The material provided is designed for educational and informational purposes only and as a benefit and service in furtherance of the HHTFI mission. The HHT syndrome is a complex, multi-organ disorder that can only be properly diagnosed and managed by skilled and trained health care professionals. The information contained on this site should not replace necessary consultations with qualified health care professionals.

The HHT Foundation International does not endorse any particular drugs or treatments reported or discussed on this website. The manifestations and severity of the HHT syndrome vary greatly among individuals and require personalized medical management. None of the medications or courses of treatment discussed on this site should be undertaken without being discussed with your physician.

The HHTFI, its officers, directors, members, employees, agents, and representatives, both individually and collectively, make no representations with respect to the contents hereof and specifically disclaim any other warranties, including but not limited to implied or expressed warranties of merchantability or fitness for any particular usage, application, or process.

The HHTFI site contains certain links to other websites whose content might be of interest to our visitors. The HHTFI is not responsible for the material at such linked sites and presence of such a link does not constitute an endorsement by the HHTFI of the material on the linked site.

The House and Senate Labor, Health and Human Services and Education Appropriations subcommittees completed a critical early step in the appropriation process in June with the “marking up” of their spending bills.

The full Senate Appropriations Committee and the full House Appropriations Committee met their plan to approve their respective bill in mid July prior to leaving for the summer congressional recess that started August 6^th.

In working with Senator Sherrod Brown’s office and Representative Rosa DeLauro’s office we found out that the HHT language made it into both “marked up” bills however the funding request was not added. As reported by Representative DeLauro’s office, only 50% of her priorities were funded this year because of the tight budget. The specific reported language is listed below.

House FY 2008 LHHS Appropriations Bill

Hereditary Hemorrhagic Telangiectasia (HHT).-The Committee encourages CDC to establish a Hereditary Hemorrhagic Telangiectasia resource center to increase identification of people affected 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.

Hereditary Hemorrhagic Telangiectasia (HHT).-HHT is a multisystem vascular genetic disorder that affects 75,000 Americans, producing arterioveneous malformations in the brain and lung. The Committee recognizes that while HHT is largely preventable with proper intervention, twenty percent of children and adults with HHT die or become disabled due to lack of recognition by the medical community. The Committee encourages NINDS to support research that would improve the quality of life for people living with HHT.

Senate FY 2008 LHHS Appropriations Bill

Hereditary Hemorrhagic Telangiectasia.—The Committee is aware of interest in the establishment of a Hereditary Hemorrhagic Telangiectasia [HHT] National Resource Center. The Committee encourages the CDC to examine carefully proposals to establish such a center.

Positive Outcomes from HHT Legislative Advocacy

Understandably, we are disappointed that federal funding was not added into the bill at this point. Many people have worked very hard including Members of the Foundation, HHT Treatment Center of Excellence Directors, Board of Directors, and the Foundation’s staff. On a positive note, there are a couple things I would ask you to keep in mind as we continue to proceed with the HHT Legislative Initiative.

• HHT’s pursuit of federal funding is a necessary activity but gaining the funding is a long and difficult process

• HHT has benefited in several ways as a direct result of our HHT Legislative Initiative. As a result of our HHT Language being added to the House and Senate bill, HHT has benefited in the following ways:

• • The United States Government now recognizes HHT as a national health problem.

o The Center for Disease Control and Prevention (CDC) now has HHT on their radar. In our initial meeting with the CDC, HHT and the CDC agreed to participate in a two day conference to be held in November of 2007. This conference is entitled “HHT Health Initiatives for the 21^st Century”. The goal is to develop a health initiative of surveillance, detection and treatment of this deadly disease. The HHT Foundation has the disease expertise, which married with the CDC’s experience, will certainly lead to improved care for patients with this disease. The CDC has awarded the HHT Foundation a grant of $32,000 to be utilized for the conference.

The specific objectives of this conference:

• to develop a roadmap for HHT treatment and research
• to implement Clinical Guidelines and effectively disseminate information to primary care providers to aid in identifying undiagnosed individuals
• Data Base Development to establish methods for data collection related to screening, treatment, and outcomes of medical interventions

o The National Institute of Health (NIH) has HHT on their radar as well. The HHT Foundation’s researchers and clinicians write proposals to gain funding for their specific research projects. Since the HHT Language has been in the bill, these researchers have been awarded more funding for research. To follow is just a partial listing of Clinical Trails that have been funded:

There is still an opportunity to have funding added and the HHT Foundation is working side by side with a few of our HHT Treatment Center of Excellence Directors to influence this process with key individuals in the political arena. I want to be candid however; it will be difficult to have funding added at this point for FY2008. Regardless, we still have to try and will keep all informed if action is needed.

The leadership will soon convene in the conference process. This is the process of reaching a compromise in the differences between the House and Senate bills. It is important to ensure our HHT Language stays in the final bill. Strong and persistent outreach by advocates now and through this conferencing process will be critical to assure HHT language stays in the bill for FY2008.

Congressional Appropriations for Uncommon Disorders

One of the common objections that a Member of Congress or their staffer will state is that the budget for FY2008 is very tight and that is why they may not be able to help us with the HHT federal funding this year. The budget has been tight for several years and will continue to be so in the future. Similar disorders have received millions of dollars in federal funding over the years and continue to do so. Most of these disorders are less prevalent than HHT. These disorders are worthy of federal funding and so too is HHT. This disorder has been ignored too long. It is our time.

Key Congressional Committee

Our HHT Legislative Initiative is seeking an appropriation; therefore, we are still focusing our efforts on the Appropriations Committee. Since HHT is a health issue, HHT falls under the domain of the Appropriation Subcommittee for Labor, Health and Human Services, and Education (Labor HHS). This is where we will focus a great deal of our efforts. Utilizing http://www.congressmerge.com website, you can find a list of the members of the House and Senate Appropriation Committees. Please see if your Senators or Representative are on this list. You can click on their names to view a wealth of valuable information about each member.

These Representatives have the power to set aside funding for the HHT initiative. While these individuals are key to our cause, it is important to contact all your Senators and Representatives, regardless of whether they are on the committee or not, to gain their support. If they are not on one of these committees they can help us by letting their colleagues on the committee know that they support the HHT Legislative Initiative.

The objective is to get appointments or make contact with your Member of Congress (through phone, email or fax) in an effort to obtain recognition and funding for HHT. Please see the Advocacy 101 section that follows for guidelines to help you accomplish this task.

The next step in the process is for the House LHHS and Senate LHHS subcommittee to meet and “mark up the bill”. This is the process whereby the subcommittees analyze a piece of legislation, section by section, and make changes. We needed to make sure we had each of the members support in order for our legislation to make it through this process. A special plea with customized instructions and sample letters went out to all our HHT members who have LHHS Committee Members that represent them in order to make sure they recognize how important their call, visit, and/or letters are.

While every call, letter and/or visit to a member of Congress is important, there are some that are critical. There are 8 HHT Center of Excellence in the United States. They are in: New Haven, Connecticut, Salt Lake City, Utah, Portland, Oregon, St. Louis, Missouri, San Diego, CA, Philadelphia, PA, Rochester, Minnesota, and Augusta, Georgia. The Senators and House Representatives that represents these states and/or districts should have a vested interest in the well-being of these Centers and it is very important to gain their support. A special call to action went out to every HHT Center of Excellence Director to make sure they were actively involved in contacting their two Senators and House Representative to gain their support. The same plea went out to every HHT member in those states with customized instructions and sample letters.

Our HHT Language, and most importantly, our $8 million Appropriation Request made it into the House and Senate Labor, Health and Human Services, Education (LHHS) Subcommittee. We are so thankful to Representative Rosa DeLauro of Connecticut and Senator Sherrod Brown of Ohio for introducing our legislation to their respective LHHS subcommittees. The Foundation’s office put out a formal thank you to both Representative DeLauro and Senator Brown and asked that Connecticut and Ohio constituents do the same. It would be impressive if our whole membership sent a thank you as well. If you are interested in doing so, please contact the Foundation office for their addresses.

A mass email was sent from the HHT Foundation to approximately 2,000 HHT members. In this email the Foundation asked each person to send a letter to their Members of Congress. In the email the Foundation provided a variety of information to help individuals with this endeavor including: the FY 2008 HHT Legislative Language and Funding Request, a sample letter that could be customized by each HHT member and sent to their two Senators and House Representative, a website by which individuals could determine who their Members of Congress are and their contact information, and a step-by-step instruction guide to walk individuals through this legislative advocacy process.

We also provided additional resources on our HHT website under the section called Legislative Advocacy. This information can be used to help educate your Members of Congress on HHT and includes such resources as: a HHT Fact Sheet, the HHT Impact on the United States, and a listing of the HHT Impact on individual states.

If you want to view these resources you can type the following URL address into your web browser (exactly as it appears – case sensitive) or go to the HHT website under the section called Legislative Advocacy and click on the link.

HHT Language and Funding Request for FY 2008 – http://www.hht.org/docs/HHT_Congressional_Initiative_FY2008.doc

HHT Fact Sheet – http://www.hht.org/docs/HHT_Impact_on_US.pdf

HHT Impact on the United States - http://www.hht.org/docs/HHT_Impact_on_States.pdf

HHT Sample Letter – http://www.hht.org/docs/General_May_2007.doc

We further instructed members that had gained their Member of Congress’ support for HHT, to ask them to send a Support Letter to the Chairman and Ranking Member of the respective Labor Health and Human Services Education (LHHS) Subcommittee. For your Congressperson’s convenience, the Foundation provided a sample support letter that only needed to put on their letterhead, customized if they wished, signed, and mailed.

The URL address is below to view these Support Letters.

Senate Support Letter - http://www.hht.org/docs/Senate_Support_Letter_June_2007.doc

House Support Letter - http://www.hht.org/docs/House_Support_Letter_June_2007.doc

Second Annual HHT Capitol Hill Day

Capitol Hill Day was held March 7^th – 8^th. With the combined efforts of our HHT members, Board of Directors, Center of Excellence Directors, and our Foundation office staff, the goal of 60 Congressional Office visits was achieved. We had HHT members who returned for their 2^nd Capitol Hill Day and many new HHT members joined us for the first time. We were thrilled with the quantity of appointments, but more importantly, the productivity of the meetings was greatly improved. At the conclusion, we left Washington, DC with the knowledge that 60 more members of Congress were educated on HHT and that 12 Senate offices and 13 House offices made statements of varying levels of support for our Legislative Initiative!

Senate House

Ted Kennedy of MA Rosa DeLauro of CT

Christopher Dodd of CT Jim Gerlach of PA

Tom Harkin of IO Patrick Murphy of PA

Arlen Specter of PA Mark Kirk of IL

Robert Bennett of UT Dave Reichert of WA

Orrin Hatch of UT Jerry Lewis of CA

Frank Lautenberg of NJ Lois Capps of CA

Robert Menendez of NJ Stephanie Herseth of SD

Lindsey Graham of SC Jim Matheson of UT

Mary Landrieu of LA Rodney Alexander of LA

Johnny Isakson of GA Chris Cannon of UT

Tim Johnson of SD Ralph Regula of OH

Bart Gordan of TN

What’s Been Accomplished Thus Far

By Sharon Williams, Legislative Advocacy and Regional Networking Alliance Manager

January and February 2007: FY 2008 HHT Legislative Language and Funding Request was established by HHT Foundation.

The HHT Foundation is asking for an $8 million appropriation designation for HHT through the Center for Disease Control (CDC) for FY2008. This federal funding would be utilized by 8 HHT Treatment Centers of Excellence to provide surveillance; create a multi-center clinical database to collect and analyze data; support epidemiological studies; provide and manage patient interventions; train health care professionals to prevent death and disability, improve outcome, reduce costs and increase the quality of life for people living with HHT. Additionally, a National Resource Center would be established to increase HHT education, outreach, and support. With the help of this funding and through aggressive management of the HHT at risk population, it is estimated that over $6.6 billion in preventable health care costs can be saved.