Opening a door to PH pediatric research

On November 7, Bob Gray in PHA's Development Department and I traveled to New York for a small but important celebration...an event that we hope will someday soon mark a turning point in pediatric pulmonary hypertension research.

On the train up from Washington, DC, I found myself thinking back to 1999 when Jack Stibbs - the father of young Emily (now a college student) - had come to PHA wanting to raise funds for pediatric PH research.  Dr. Bruce Brundage had to tell him that there was no specific pediatric PH research underway at the time and precious little in PH period.  He told Jack the goal needed to be pulmonary hypertension more generally.  That answer was always a target for change.

Last year, Dr. Robyn Barst, one of the pioneer pulmonary hypertension researchers and clinicians - and the pediatric cardiologist who founded the PH Center at Columbia - made a generous contribution to begin a pediatric research fund...one which PHA named in her honor. 

Our plan is to begin to advertise for grant applications when the Robyn Barst Pediatric Research Fund reaches $1,000,000.

The purpose of our luncheon in Manhattan was to receive an extraordinarily generous $500,000 contribution to add to the Fund.  It was given in Dr. Barst's honor from the Cardiovascular Medical Research and Education Fund (CMREF).

Pictured from left to right in the photo above are Dr. Stuart Rich who among other contributions led the early research on calcium channel blockers for PH, PHA's Bob Gray, Dr. John Newman whose many contributions to the field include a leading role in research resulting in discovery of the PH gene, CMREF board member Michael Fishbein (Mr. Fishbein is a Philadelphia attorney who coordinated much of the fen phen litigation), Robyn's husband Dr. Sam Barst and Dr. Robyn Barst, PHA board member Rev. Steve White who is also a significant contributor to the Barst Fund, Dr. Erika Berman Rosenzweig who succeeded Dr. Barst as Director of the PH Center at Columbia Presbyterian Hospital in New York City and me.

CMREF's gift has moved us to just about $800,000, much closer to the day when we can begin making grants from this important new Fund.

On December 16, PHA will be running the ad below in a pulmonary hypertension insert in over 485,000 copies of USA Today in Boston, Chicago, Dallas, Nashville, St. Louis and Baltimore/Washington, DC.  Expected readership is over 1.2 million.


Readers are encouraged to visit a new web page at
www.PHAssociation.org/BarstFund
We hope you will too!

A quarter century ago, PH research was just beginning.  Today the interest and advances are significant and growing.  It's time for pediatric PH research to have the opportunity to catch up.  PHA is proud to help make that possible.

I hope you'll take a look at the 8 page USA Today insert on PH.  It includes the Barst Fund ad and a lot more.

   

The Lantos Grants: now it's YOUR turn...

Our advances at PHA have always come as we have learned from each other.

PHA's annual Tom Lantos Innovation in Community Service Awards are a great example of that truth.

Up to ten creative ideas will be funded in 2012 with funding of up to $5,000 for each, thanks to an annual grant from Gilead.

If you've got a good idea that can help the PH community, read the guidelines and YOU can apply NOW!  The deadline is January 6.

•  In 2011, Colleen Brunetti's idea was to create a YouTube video discussing the value of PH e-mail and discussion groups.  With other patients, Melanie Kozak, Raye Bohn, Misty Lewis and Carol Morrison  as the on-screen talent and PH dad Steve Van Wormer as the narrator, Colleen's vision was funded through a Lantos grant and became reality.  It's now available online both as a value for the PH community and a best practice for others to build on.  You (and the rest of the world) can watch it now!
• Donna Lain and her daughter, Andrea, had an idea to to provide support for children diagnosed with pulmonary hypertension...Kari Bags named in honor of Donna's daughter, Kari Beth (9/9/1982 – 5/27/2003). The bags provide educational material about pulmonary hypertension and are filled with age appropriate toys, games, and stress reliever items. Kari Bags allow kids to have stuff to play with when they are required to be in the hospital for procedures and tests or admitted for infections or line problems. The bags are produced for three age groups and distributed through PH Centers and pediatric PH support groups.  So far, the new program has distributed over 150 Kari bags and were featured at this year's PHPN Symposium.
• In 2010, the first year of the Lantos Grant program, Donna Caterini received funding to fulfill her idea of building PH awareness through a clever "Huff & Puff" poster, featuring the Big Bad Wolf.  The 11x17 posters have been a big hit...distributed through support groups and available for free to any in our community who want to place them in local libraries or other public places.

And the Lantos grant program isn't restricted to the U.S.  It's available to the PH community throughout the world.  Read the story of the Spanish PH group, ANHP.  That's a picture of their meeting to educate psychologists about the needs of PH patients at the top of this page.

Now it's your turn!  If you have an idea you'd like to bring to reality for the good of the community, the Lantos grants are for you.  The application is not complex and the benfits for all who live with PH are great. 

We look forward to receiving your proposal.

An awareness month journey...

As I write this, we are only 10 days into Awareness Month...and it is already shaping up to be an extraordinary journey.

I began the month a bit early (October 28) with a 3 day trip to Frankfurt, Germany where I was honored to address the German PH Association - PHeV - on the occasion of their fifteenth anniversary.  Their founder, Bruno Kopp, is a PH patient in a family with 15 PH patients.  He is a man who said, "enough", and in 1996 decided to do something about this disease.  Over 300 patients and family members attended the conference and, besides Bruno, it was great to see so many old friends, including Gunter Timm (who acted as my translator) and his son, Drs. Olschewski, Grünig  and Ghofrani and PHA Europe president Gerry Fischer.  The event was a strong reminder of how vibrant the global PH community has become.

The day after getting back, Dr. Rich Channick and I journeyed to New Jersey where we met with Novartis medical leadership to help them understand PHA programs and activities as they anticipate entering this market.

When I got back to the office I saw an amazing growth in the ways our community is using social networking to build awareness of PH.  Weekly Facebook prompts began going out from our office with the first being, Pulmonary hypertension feels like...  Emma Bonanomi summarized this week's 83 responses as... 

ranging from the serious (“running up and down stairs for at least 10 minutes on an empty stomach with no sleep”), to the lighthearted (“the best dang excuse to play video games I've ever had :D”) to the inspirational (“my number one reason to fight harder”).

PHA's Facebook friending (and reach) has been growing at an amazing pace this month - at 4,981 this afternoon - on pace to be over 5,000 in the next day or two.

On another communication front, our web staff - Diane Greenhalgh and Kathy Frix - added a great new tool to PHA's website.  In the upper right hand corner of every page on http://www.phassociation.org/ is a select language box, allowing translation into any of 52 languages.

City, County and State PH Awareness Month proclamations have been flowing in rapidly (Elisabeth Williams reports 33 and growing) and South Carolina PHA member Doug Taylor even got his Member of Congress to discuss PH Awareness Month and PHA in the Congressional Record.

The American Thoracic Society has partnered with PHA for a PH Week (it began November 6) within that society of pulmonologists and launched a website to educate ATS membership.  As part of this effort, PHA is hosting a webinar on November 10 with 5 leading physicians presenting on Improving Patient Outcomes and Early Diagnosis.  Several days after the live event (over 170 registered!), it can be viewed on both PHA Classroom and PHA Online University.

Last Sunday, Jess McKearin and I were in Palo Alto, California for the Stanford Race Against PH.  It was their 11th Annual begun by the Ewing family and now grown to an event that includes over 1,500 participants.  Board members Harry Rozakis and Rita Orth were there to join with Drs. Zamanian, Krasnow and Feinstein and Kristi Kerivan and their great team from the Wall Center at Stanford. This year PHA launched our new Six Minute Marathon as part of the Race to provide more understanding of what PH patients go through with this disease.  It was a great success and we'll be rolling out the Six Minute Marathon at other events in 2012.

While Jess and I were in Palo Alto, Adrienne Dern, our Senior VP, was speaking at the patient program at the Cleveland Clinic PH Summit and our Medical Services staff. Rebecca Kurikeshu and Caitlin Flewellen were staffing a well-trafficked exhibit at the American College of Rheumatology meeting in Chicago.  Debbie Castro was on the road, too, for events in Phoenix and Fort Wayne.

The day after Palo Alto, Bob Gray and I Amtraked to New York where we had a wonderful luncheon to celebrate a major gift to the Robyn Barst Pediatric Research Fund.  We'll be telling more of that story very soon.  Tomorrow it's on to Memphis for an American Thoracic Society Patient Advisory Roundtable board meeting.

As I'm typing this, Megan Mallory brought in a hot off the presses copy of our 2011 annual report and that will be going out in the mail to members in the next few days.  I think it gives a great outline of all that our community is doing to change the history of this disease.

We've still got a long way to go on our the Awareness Month 2011 journey. Check PHA's News Feed to see what's new with Awareness Month...and PHA's Awareness Month pages to see how you can help! 

Let me breathe...

There is so much happening during PH Awareness Month.

...And it's happening all around the world.  Today, I received a Facebook friend request from a young man in South Korea.  When I took a look at his page, I was amazed to see a promotion of PHA's upcoming (Nov. 10) webinar - Improving Patient Outcomes and Early Diagnosis: An Examination of the REVEAL Registry - right at the top!  The presenters will include:

• C. Gregory Elliot, MD, Intermountain Health Center (Facilitator)
• Vallerie McLaughlin, MD, University of Michigan
• Lynn Brown, MD, PhD, Intermountain Health Center
• Richard Channick, MD, Massachusetts General Hospital
• David Badesch, MD, University of Colorado at Denver

Also today, Megan Mallory, who leads our Publications Department, got a lovely note from the Co-Leader of the Toronto Chapter of PHA Canada.  Ruth Dolan, who had attended a webinar on Expression of Well Being led by Megan and Janet Stone, wrote:

I thoroughly enjoyed this webinar and do look forward to attending more in the future. I mentioned during that session that I would be participating in an experience at our local library called "Get a Life- Human book Festival" and I said I would get back to you about the experience so you might choose to share it with other PH Friends.

It was an amazing experience and for me an opportunity to share my PH Journey with my youngest daughter Emily, with those who came to the library and "took me off the shelf" to "read" me.

My "Book" was called "Let Me Breathe". just like the PH Song written by my brother, J. Paul Adams for his niece and dedicated to her and all PH patients [see below]

There were about 7 human books at our local library branch and there were four libraries through out Simcoe county participating, one for the four Saturday's in October. For me it was yet another opportunity to make people aware of PH in a very personal way.

About 30 min. was allowed for each reader but we always seemed to spend at least 40 -45 min. together; as my reading sessions had a break between each one I was able to spend the extra time with the readers. My first reader was an author and a very caring listener. She has since asked me if I would like to write my story to be included in a book of other women's stories....very tempting.


I took our PH Canada and Toronto PH brochures along to give to my "readers' so they had something to take away and do further research about PH. The local media was also at the even and interviewed some of the "Books".

Although it was a "small" audience with which to share our PH Journey it was one that I would gladly do again because of the personal nature of the sharing and I think and the impact it had on the "reader". I feel that just as when I read a great book I love to share it with friends, I also feel that my readers will hopefully share the experience of reading "Let Me Breathe" with their friends and thus making even more people aware of Pulmonary Hypertension.

On another note:

Our Toronto PH Fundraiser and awareness campaign is taking place this Saturday. We have the Let Me Breathe Vegas Night and are also using that occasion to gather real and virtual hand prints that will go on a large banner or several I hope , to our capital in Ottawa on the 29th of November. Feel free to add your hand print to our efforts by using the link below from our Canadian Website.
http://www.phacanada.ca/index.php?cID=975


All the best to you and to all our American PH Friends. I am looking forward to seeing you again in Orlando in 2012

Here is Let Me Breathe, written by J Paul Adams, Ruth's brother...



It can also be found on the PHA Toronto website.

First Media in Awareness Month

I just watched Patty Kaiser's interview on St. Louis TV station KSDK.  Meet Me St Louis host Julie Tristan filmed and edited Patty, using live and archived footage that ranged from the Blue Lips campaign to  2011 PHA Dinner and Wine Tasting Fundraiser coming up on Saturday November 12th. It was a great start to Awareness Month. Watch the Video

After her interview, Patty wrote:
Patty is not alone in getting awareness month going...

Even through I am terrified of sitting in front of a camera and being interviewed, I am so passionate about PH awareness, and getting the message, out that I am willing to "put myself out there" so others may not have to go as long as I did before finally being diagnosed. 

Alex Filipse hit a proclamation trifecta with proclamations from Takoma Park (the city where she lives), Montgomery County (the county where she lives) and Maryland itself (the state where she lives...and proclamations are starting to roll in from around the country.

Gerri Brown suggested...

Here's an idea that we may want to try on PH Awareness day or Month - change your last name to one of your meds.

Now, she's Gerry Viagra-Brown on Facebook and we're seeing the idea move like wildfire through the community, raising questions and curiosity that are giving a great opportunity to talk about PH.
This year's Awareness Month theme - The Power of One - is leading to benefits for many!

Welcome to Awareness Month!

The Power of One

Awareness Month is here! To kick it off, here’s a post from Katie Kroner, PHA’s Director of Advocacy and Awareness. As you’ll see, there are Awareness Month activities going on around the world. I hope you’ll add your voice to the conversation! 

This year’s Awareness Month theme, The Power of One, reminds me of lots of remarkable things about the PH community. We’ve worked together to end isolation, growing from a community of a few to a community of thousands. Our advocacy has brought PH to the attention of the medical and pharmaceutical communities, taking us from zero treatments to nine—more than only two of the 7,000 identified orphan diseases. Most of all, The Power of One reminds me of the everyday heroes — each of you – without which we could not be where we are.

The first National PH Week was held November 2-8, 1997. PHA’s historical records note some important milestones during those early Awareness Weeks including growth of the support group network to 35 groups in 1998. (We now have 235.) Since 1997, PH Week has become PH Awareness Month and reaches across the country and around the word but its individual people, growing ideas into successes, that still make the PH community what it is. Here are just a few of the big ideas that will make Awareness Month 2011 a huge success:

·         Each year, the PH community requests Awareness Month Proclamations from their local elected officials, but when Doug Taylor issued a challenge for some state (any state!) to beat South Carolina’s record of 11 proclamations in one year, he started something big. On Facebook alone, 98 people have responded to Doug’s challenge. Perry Mamigonian has inspired Support Group Leaders across California to take on the challenge, but proclamations are also coming in from Maryland, New Jersey, Tennessee, Nebraska…you get the picture.

·         November boasts nearly twenty special event fundraisers aimed at supporting PH research and PHA programs. Organizers took their unique ideas and passions and turned them into community endeavors. Nicole Cooper is turning her first-time event vision into a reality with the Vision of Hope Gala in Maryland on November 12. Other first time events include a Zumbathon and chili cook-off. Many organizers have multiplied their impact by connecting with the media to promote their event and raise awareness of PH in their communities.

·         On November 1, more than 400 people used Facebook and other online tools to spread the word about pulmonary hypertension worldwide. One caregiver from Germany wrote to say that he has posted a PH web banner on his software blog, which receives about 2,000 visitors a day. That’s the power of one to raise awareness about pulmonary hypertension.

Individually, we are powerful. Together, we are unstoppable. How will you put your awareness raising power to work this November? Learn more and join our fight against PH!

PHPN...a building block to a better future

The more that's going on...the less time there is to write about it. 

Since the Friday before last, PHA has had meetings for our Scientific Leadership Council, medical journal Editorial Committee, Research Committee, PH Professional Network (PHPN) Executive Committee, and PHA's Board of Trustees and its various committees.   I'll try to get time to blog about some of these meetings but I'll focus today on the largest of the meetings, the 2011 PHPN Symposium.

The Symposium is a three day annual meeting that takes place every two years.  Planned by PHA's allied health group, PH Professional Network, the event is an opportunity for education and networking in this rapidly growing field.  In recent years, the event has also supported the growing strength of this community of PH nurses, pharmacists, respiratory therapists, NPs, PAs, social workers and others who support the treatment of PH patients.

The Symposium is always held in the Washington, DC area where we begin with Advocacy Day.  Almost 100 of this year's record 465 Symposium registrants participated.  Not only did they have an important and worthwhile experience talking to their elected representatives on Capitol Hill about the Pulmonary Hypertension Research and Education Act but they bring that experience back to their medical centers and patients.

This year a special target for Symposium was increasing the number of abstracts and posters presented.  This is a shared learning opportunity and one for which PHPN leadership wanted to build the skills needed.  In order to do this, they knew they had to teach.  They created a new section of the PHA website on submitting an abstract for a poster presentation.  One of their founding members, Lisa Wheeler, research coordinator at the Vanderbilt University PH Center recorded a webinar, guidelines and samples were posted and mentoring was offered.  A Symposium that had no abstracts two events ago and very few at the last Symposium grew to 40 at this year's event.  The value of information exchange was huge not only for this meeting but is now a skill that exists within the community and will grow and be used at future symposia and other medical meetings for the benefit of medical knowledge in the field.

The educational program is always rich, This year's Symposium - chaired by Melisa Wilson, APRN, BC - with 60 expert presenters   delivering an information-filled program was no exception.  A real highlight was a plenary session entitled A New Dimension of Hope: Virtual Catheterization Lab presented by Russel Hirsch, MD, Cincinnati Children's Hospital and James Tarver, MD, Orlando Heart Center.  Rather than slides, Dr. Hirsh used a specially created animation of a heart catheterization.  It was a wonderful teaching opportunity.  Motivation is also a powerful Symposium element and this year's keynoter, Jessica Lazar MPA, PA-C , was a huge hit, telling her story of the Kilimanjaro Climb she and Drs. Benza and Frantz did for PH awareness. 

PHA had all the sessions filmed and they will soon appear on PHA Online University, enriching teaching opportunities for medical professionals around the world.

Since the first Symposium in 2003, I have watched these meetings grow from 60 attendees to, this year, well over 400.  They have become not only a cornerstone of medical education for allied health professionals in the field but they have also sparked stronger collaboration that is now taking place every day online and face-to-face.  Seeing the level of commitment and compassion, I have no doubt that PHPN has become an essential building block, leading the way to a better future for patients and their families.

Making a difference...

In recent years, our friend Hall Skara has been building PHA Norway into an effective voice for pulmonary hypertension patients in his country.  It's a great effort in a nation where there are not many diagnosed patients.

On September 25, PHA Norway held their national conference.  the event generated good  press coverage and and the 11:30 minute TV interview below.  Many thanks Hall for subtitling the interview in English!



I really enjoy blogging about PHA Norway.  They are a great example about how even a small community of patients, family members and medical professionals can make a difference.

Learn more about how PHA Norway got started and about the international PH community.

Risk to reality...

Today, I did something that astonished me.

I signed a licensing agreement that will help PHA bring medical education to India in an important new way.

Why this was astonishing goes back to 2000...

On the second day of PHA's Fourth International Conference - June 24, 2000 - in the atrium of the Wyndham Hotel, Dr. Bruce Brundage, Craig Mears (then of Gentiva) and I met to talk about the possibility of publishing a medical journal.

Bruce had the reasonable concern that any publication not be "a throwaway" for the doctors who would receive it.  I saw the impact it might have but wasn't sure whether there was enough content to sustain a medical journal over the long term.  Craig was willing to talk to his company about the initial funding.

It was a different time then. There were only about 100 treating physicians in the U.S. - and they were seeing about 3,000 patients.  Only one complex and still relatively new treatment had been approved by the FDA at that point, not the nine we have today.

Several months after the Conference, Dr. Brundage (then Chair of PHA's Scientific Leadership Council) convened a telephone conference to talk about the possibility.

The group understood the potential value and decided to move forward.  Dr. Tapson at Duke was invited to become the first editor and we were off and running.  Advances in Pulmonary Hypertension: the Official Journal of the Pulmonary Hypertension Association published it's first issue in 2002.  It was a big name for a then small organization.

We were taking a huge risk for a potential large benefit. At the time of the Chicago meeting, PHA was an organization that just a year earlier had a total annual budget of $137,000.  Now we were taking on the responsibility for a new project that would cost over a quarter of a million dollars per year.  Some might say we were crazy.

As it turned out, the risk more than paid off.  Advances has published regularly in the ten years since then, reaching over 40,000 cardiologists, pulmonologists and rheumatologists four times each year, providing leadership and education from world recognized experts.  The field has grown considerably since Advances began publishing and I'm confident in saying that this journal has played an important role in that growth.

Now back to India... About 10 percent of the publication's distribution is sent to physicians in 63 nations outside the U.S. and international interest has been growing.   Over the past year, PHA has received several unsolicited requests to license international distribution and translations of Advances.

The editorial committee, led by Dr. Erika Berman Rosenzweig of Columbia University has carefully worked out a template agreement for international licensing that protects the integrity of content and allows, with PHA's editiorial review and approval, the addition of some additional content relevant to the particular nation.  Leadership of PHA's Scientific Leadership Council has approved the template.

Today, I signed our first agreement.  An Indian edition of Advances will soon be published. 

May it be as valuable to Indian physicians and their patients as Advances has been in the United States...and may it be the first of many such agreements.

People make change...

We usually write about end points...the things that happened.  The reality is that the outcomes and results we celebrate are most often built upon long and hard work.  Today, my blog is about a year's long process that, in truth, began quite a bit before the 2006 event with which I open and will no doubt continue long after this month's victory with which I close...
In 2006, Carl Hicks (then PHA board chair-elect), Dr. Ramona Doyle (then at the Vera Moulton Wall PH Center at Stanford, Congressman Tom Lantos (who has since passed), our Washington representative Gavin Lindberg, myself and others went to the Department of Health and Human Services to meet with their leadership and discuss our concerns about the then new PH transplant standards.  The new rules moved PH patients lower on the priority list...based on interpretations of information that our medical leadership and PHA disputed.

HHS was engaged and the meeting led to follow ups with the United Network for Organ Sharing (UNOS).   UNOS is the private, non-profit organization that manages the nation's organ transplant system under contract with the federal government.

PHA's medical leadership worked hard to get it right and to build some appropriate flexibility into the system for PH patients.  Their interest led to increased activity within the influential International Society of Heart and Lung Transplant (ISHLT) and, for the past several years, these doctors have been building a strong and active assembly within ISHLT and continuing to engage UNOS.

In early-August, PHA circulated the following message to over 600 physicians who are members of the PHA medical group, PH Clinicians and Researchers.  (The LAS mentioned in the note refers to the Lung Allocation Score.)

Physicians PHA works with, who are also involved with the REVEAL Registry, gave a presentation at the ISHLT in 2009. Following that, UNOS invited them to speak to the thoracic council about their results on the LAS score. Dr. Ray Benza was then asked to be on the thoracic board and began to advocate changes in the LAS for PH patients. Dr. Benza reports that the Thoracic Board - particularly its current chair, Mark Barr - was very receptive. UNOS’ new policy on submitting lung allocation score exception requests for candidates diagnosed with PH appears below.

Dr. Benza has asked us to circulate this to the PH medical community as quickly as possible since it may impact current and future patients awaiting transplant.
PHA thanks and congratulates the physicians who have been building a rapidly growing and effective PH section within ISHLT, including Drs. Benza, Frantz and Park and others and Dr. Benza for his work on the UNOS thoracic board.

The change is partly described by UNOS, as follows:

Lung transplant candidates diagnosed with pulmonary hypertension (PH) and who meet the following criteria may qualify for an increase in their Lung Allocation Score (LAS):

1. Patient is deteriorating on optimal therapy, and

2. Patient has a right atrial pressure greater than 15 mm Hg or a cardiac index less than 1.8 L/min/m2.

We hope this decision will increase options for more patients living with PH and needing to consider the transplant option. 

People make change.  We benefit from their persistence and thank them for their dedication.

New Resource...

One of the things that is most important at PHA is finding better ways to communicate valuable information to the patients, family members and medical professionals who are the reason for our work.


An example of that goal has just been released for caregivers…

Resources for Family and Friends is a free information packet designed especially for family members and friends of PH patients. It offers a range of opportunities to help caregivers plan their next steps, find important resources and connect with other caregivers.

It follows the also free Envelope of Hope packet which we created almost two years ago for patients…and which we are constantly upgrading.

Both packets can now be ordered online.

Medical professionals are able to help patients and caregivers get these packets by giving out referral postcards which can be ordered in quantity at no cost. The packets are particularly valuable for new patients and their families.

I’m particularly pleased that PHA is now focusing hard on building new ways to support PH caregivers.

Lian Latham, a nurse who has treated many PH patients, says it well.

“The families of patients are the true backbone support of PAH. They stand by the patients every day, providing solace and care to those who are in need. Often, they sacrifice themselves in many ways to make sure their loved ones get what they need. They are like the battlefield medics for PAH.”

PHA is glad to be able to help.  Like PH patients, caregivers are not alone with this disease.

The story of a book...

July 25 will be an important day for PHA.  We will be publishing the new fourth edition of the Patients' Survival Guide.  Watching the work of our volunteer writers and artists, the medical reviewers, led by Dr. Ron Oudiz, and PHA staff, led by Patti Lalley and Amanda Butts, I've been awed by the work that has gone into a major revision and addition to this 300 page patient and family treasure.

In considering about how to celebrate this accomplishment, I can think of no better way than turning to the words of the book's creator (and author of it's first three editions) - Gail Boyer Hayes - who also wrote PHA's history from its founding through 2000 (and, in the case of the Survival Guide, a little bit beyond).

Gail continues to write - novels now - and we thank her for what she began and, as you can see,others jumped in to make possible.  She is another example of the difference a single person, willing to act, can make.

Today, PHA's Survival Guide, has been translated by teams of medical professionals and others from English into Japanese, Chinese, Korean, Spanish, Farsi and other languages.

We hope you enjoy the new fourth edition!

_____________________________________________________________

FIRST PATIENT’S SURVIVAL GUIDE  PUBLISHED

Pulmonary Hypertension: A Patient’s Survival Guide

The Internet age was just dawning and most PH patients still did not have access to it. Even those who did lacked the sort of in-depth, accurate information they needed to cope with their illness and make intelligent decisions about which treatment options to discuss with their doctors. Both PH and its treatments were exceedingly complex. It is quite impossible for a patient with such an illness to remember everything a doctor tells him or her during an office visit, and equally impossible for a doctor to be comprehensive in the time allotted.

PHA decided, therefore, that patients needed a comprehensive reference book they could turn to for reassurance and for guidance.

PPH patient and writer/editor Gail Boyer Hayes (Seattle, WA) wrote the 123-page first edition of



Gail Boyer Hayes

 Pulmonary Hypertension: A Patient’s Survival Guide, which PHA published in 1998. Before she was diagnosed with PPH at the age of 40, Gail had been working as a lawyer in California, Colorado, and Washington, DC. And before that, she had been a book reviewer, magazine and newspaper reporter, short-story writer, and television talk show hostess. For the six years following her decision to write the Survival Guide, the Guide and its updates consumed nearly all of her free time.

The medical consultant was the distinguished Dr. Bruce Brundage. Andrea Rich (the wife of Dr. Stuart Rich) did the medical illustrations. Other PH patients, doctors, nurses, and family members reviewed the book prior to publication to make sure it answered the right questions and explained things in language a layperson could understand.

Chapters included: What is PH?; So How Do I Know It’s Really PH?; What Causes PH?; Treating PH; Tell Me Doc, How Long Do I Have?; Children and PH; Living with PH, and Tedious Paperwork and Legal Matters. Cost of the 123-page Guide to PHA members was $10.00.

Gail was concerned that the Guide not be slanted to curry the favor of any commercial interest. Therefore, she did not turn over the copyright to PHA until she was assured of this. (It turned out not to be an issue; PHA also wanted a Guide that patients could trust.)

The Patient’s Survival Guide was an immediate hit and flew out the door. Olsten provided PHA with $1,500 to include a copy in the new patient packets. Many doctors bought multiple copies to give out to their patients.



Barbara Smith



PH patient Barbara Smith and her husband Vern, residents of Odessa, Florida, who had built up a chain of plumbing, cable, air conditioning, and electric businesses, mailed the Survival Guides and paid for postage out of their own pocket. Barbara was already familiar with PPH before she was diagnosed in 1995, because both her sister Rachel, and her daughter Angela, had died of the disease. When Angela died, she was pregnant with her third child. After Barbara became too ill to handle the mailings herself, the Smiths continued to pay for them. For at least 5 years they also mailed out membership packets, new patient packets, PH pins and cards, and other materials, and paid the postage on them.

Few patients realized the generosity that made their low-cost orders possible. Barbara once said, “I couldn’t figure out why I was still alive after losing my daughter and my sister. The only reason I can think of is to help other people.” Barbara survived until October 2005.

Slow Walker...

For the past few weeks, Meghan Tammaro, who manages PHA's international work, has been in touch with Gloria Huanghuan, a PH patient in China.  Gloria is organizing a new PH association there - iSEEK - which she hopes will complement the work being done by her doctor, who founded PHA China.

As you watch the video (begins about 10 seconds in in Chinese with English subtitles), you are watching Gloria and the story of her experiences with PH and in trying to form a non-profit organization to help patients and their families.  What you will see is a segment of a longer film, titled Slow Walker, that she is making to help build awareness of PH and other rare diseases.  The film is applying for the Sundance Documentary Film Program.  We wish this talented young woman much success in sharing her story worldwide.

We also wish China a speedy path in sorting out out its laws and regulations for rare diseases and making it easier for NGO's to register.  What Gloria is doing can only help its citizens with rare diseases such as PH as they are being helped in 51 other nations around the world.

The meaning of events...

The First Annual Power for PH PHun Walk for a Cure from PHAssociation on Vimeo.


Last month, Jill Glenn called to say she was going to The First Annual Power for PH PHun Walk in Edison, N.J.

Jill is president of Glenn and Glenn Productions - producers of our Kilimanjaro Climb (2nd video on linked  page) and Lil Long videos - and a good friend and member of PHA.  Besides walking and raising funds for PH in her own community, Jill asked if we thought it would be ok if she and her team made a pro bono video of the event.

I know the quality of Jill's work and was excited.  Glenn and Glenn are great listeners and their fiilms go beyond pretty shots to capture the meaning of what our community does.

So, the video is ready and here it is.  I know how I feel about it...Jill and Doug and their team have taken the story of one event and presented a picture of why people do what they do across our entire community.   What do you think?

Global partnerships...greater strength and knowledge for us all

Several months ago, PHA announced the U.S. and international recipients of PHA's 2011 Tom Lantos Innovation in Community Service Awards.  PH Israel was one of the awardees.  Here is a note from Dr. Yosef  Gotlieb to PHA's Senior vice president, Adrienne Dern, on the outcome of their project.

Dear Adrienne,


It is with pleasure that I am writing to let you know that the conference sponsored by the Israel Pulmonary Hypertension Association on Thursday, June 16th was a resounding success.

Nearly a hundred participants came to the meeting on "Pulmonary Hypertension: Profile of the Disease" to hear Israel's top experts lecture on the various aspects of PH:   symptomatology, typology, diagnostics, therapies, and research horizons for treatment and diagnosis. The number of those in attendance was high despite a doctor's strike which required many senior physicians to substitute for younger doctors in the clinics and wards. Despite this, the auditorium was packed and we found ourselves having to add chairs throughout the first two sessions.

A panel on the complexity of care included a family physician, a mental health and support group worker, the director of a pulmonary rehabilitation unit at a children's hospital. a medical technology professional, and myself representing the patient's perspective. A vigorous dialogue ensued between pulmonary specialists and primary care physicians. The session ended with the promise of continuing exchange and engagement; as this was a major goal of the conference, we were very pleased to see this take place. We, the organizers and the participants who provided feedback, left the conference with the feeling that that the Israel PH community had been significantly expanded and strengthened by the conference and that there would be continuing impact on the target group we had sought to influence, namely, the primary care community.

Of those who attended the event, which was conducted in a decorous setting at a seafront Tel Aviv hotel, most were clinicians (physicians and nurses); a number of professors and heads of departments were also present. We have documented the meeting (some of the initial photos are attached) in stills and video and look forward to sharing our experience with other PH associations. We have already begun discussing possibilities for the next event to be held in what we hope will be a continuing series of meetings focused on professional and patient education.
On behalf of the Israel Pulmonary Hypertension Associationand myself, I want to reiterate how grateful we are to the sponsors of Tom Lantos Innovation in Community Service Awards and to Representative Lantos' family for enabling us to hold the event. Further, I wish to express again our gratitude to you and the Pulmonary Hypertension Association for facilitating our receipt of the funding necessary to implement this project.

We are much inspired and encouraged by the Pulmonary Hypertension Association. May you and the Association go from strength to strength.

Truly yours,

Yosef

Yosef Gotlieb, PhD

There's a lot more to read about the gvibrant and growing global PH community in the International section of PHA's website!

Ten Years Ago Today ...

I met Bill and Laura O'Donnell and their daughter Shannon six or seven years ago when they volunteered to help staff a PHA table at an event at Tufts Medical School.  Over the years our paths have crossed at PHA conferences and various Boston events and I've followed the family's activities through their support group, telephone support line, mentoring and other leadership and volunteer activities.

Yesterday, Bill posted an essay to the PHA support group leader list serv marking the tenth anniversary of Shanon's diagnosis with pulmonary hypertension.  He titled it Ten Years Ago Today. I found it inspiring and, with Bill's permission, am sharing it with you.

Godspeed and thank you to the O'Donnell's.
_________________________________________________
For many Americans 2001 will always be memorable year. This goes for me as well. Most Americans will remember 2001 because of the tragic events of September 11. The day that makes 2001 memorable for me is May 31. May 31 is the day the world stood still and Shannon was diagnosed with Primary Pulmonary Hypertension. It is a day that changed life for Shannon, Laura and I forever.

Shortly after diagnosis, Shannon started on a continuous intravenous medication called Flolan. I believe Flolan saved Shannon’s life. In 2001 it was the only medication approved by the FDA for the treatment of PH. Today there are over ten medications. Flolan has to be kept cold. It only has a half life of three minutes which meant any interruption could cause a PH episode. About two years ago she switched to Remodulin, which has a half life of 3 hours making it safer. It used the same pump and does not need to be kept cold. In April 2011 she switched from her original pump, CADD, to a Chrono 5 pump. The Chrono 5 pump is much smaller and lighter pump. She is also on several oral medications. Doctors believe combination therapy may be the best treatment available. I still hope and pray for a cure other than a double lung transplant.

How do you represent ten years? To some ten years is a decade. To others it is a half score. To me ten years is represented by three thousand two hundred eighty five mixes of life saving medicine. (8 years of Flolan, 2 years of Remodulin) This includes 2080 AA batteries, 3285 cassettes, 6570 needles and syringes, 32,850 alcohol wipes, and 328,500 ml of diluent which is equivalent to 164 two liter bottles of tonic or 87 gallons of milk. The average mix takes about twenty minutes which means 65700 minutes or 1095 hours or 46 days have been spent just mixing medicine. This does not include other time required for Shannon’s care.

During the past ten years, we have welcomed one member into our immediate family, Matthew, and many into our extended family. Sadly we have also said good-bye to several family members including Anna, Alice, Anita and Marion (four out of five of Shannon’s grandmothers), Grandfather (Ray), Uncle Tommy, Aunt Barbara and Uncle Jim.

Over the past ten years we have personally witnessed the best in humanity. On a large scale Shannon was granted a Make-A-Wish trip. She trained dolphins and fed Shamu. We stayed a special resort called Give Kids the World which was founded specifically as a place to stay for kids fulfilling their wish to the Orlando area of Florida. Shannon has also been blessed to be a camper at The Hole in the Wall Gang Camp, THITWGC, founded by Mr. Paul Newman. Paul’s vision was to provide a place where seriously ill children could be kids and raise a little hell. This summer will be Shannon’s eighth and final year. She is already talking about being a counselor.

We have attended countless shows through the generosity of others. These include performances by the Boston Ballet and the play Wicked, which Shannon loved so much she downloaded the sound track the very next day. We also attended several sporting events and met several Boston sports stars including Jason Varitek, Josh Beckett, Tim Wakefield, Big Papi (David Ortiz), and Raymond Borque.

I have attended more concerts in the past few years than the rest of my life combined. It started out with The Cheetah Girls with Ali & AJ. Next was the Jonas Brothers, my ears are still ringing from the sound 18,000 girls screaming at the top of their lungs in the Garden. This includes Shannon; you would never know she has a lung disease if you heard her scream. Just to change things up we attended a DropKick Murphys concert after Shannon filmed a public service announcement for Children’s Hospital with Ken Casey and Scruffy of the band. Honor Society was next. Most recently Shannon and I attended a Ke$ha concert at Babson College which was a standing room concert. We arrived early and wound up in the second row. We lasted two songs before I made Shannon and her friend move to the back. Although I joke about the screaming girls at the Jonas Brothers and all but being crushed at Ke$ha I will cherish the memories forever! At times I even feel sorry for dads who never have these experiences with their daughters.

This outpouring of generosity to us only makes us want to give back to the community. We have shown our appreciation and support to Children’s Hospital by raising money through its Miles for Miracles program, allowing Shannon’s image and words to be used to promote the hospital and perhaps most importantly donating blood, something everyone should do, to help patients in need. The staff of THITWGC knows all they have to do is call and if we are available we will be there to help out doing whatever is needed. Sometimes this is just being at camp on opening day to support parents who are dropping off a child for the first time and have not come to realize what a special place camp is.

Another organization that we have come really to appreciate is the Pulmonary Hypertension Association, PHA. PHA seeks a cure and prevention of PH. It provides hope to PH patients and their families. PH has given us hope with dealing with Shannon’s illness. In return Laura and I assist the organization by being mentors, help line volunteers, parent advisory members and assisting with the bi annual conference.

In thinking about this ten year anniversary I started to hum Thanks for the Memories. The song Bob Hope would sing at the end of his shows. Although I do not feel the end is near. Here is my version.


Thanks for the Memories, 10 years

Thanks for the memories

It’s been ten long years

We’ve stood and faced our fears

We were newbies

Now were oldies

We’ve even shed some tears

How terrifying it’s been!



Thanks for the Alphabet

Test like EKG’s

RN’s and MD’s

The CHB (Children’s Hospital Boston)

The PHA (Pulmonary Hypertension Association)

The THITWGC (The Hole in the Wall Gang Camp)

Especially U!



We’ve had our ups and our downs

Sometimes we’ve even gone round and round

Thanks to you we’ve never hit the ground

We shall not rest

For a cure is best



Here’s to the future

Days without mixes

And concentrator hisses

Celebrations

Graduations

Docs with all the fixes

How wonderful it will be!


As we celebrate this tenth anniversary I want to thank you for whatever you may have done to make Shannon, Laura and my life more bearable. Even if this was just to say a prayer or wish good thoughts on us. Please take a moment today to thank God for all of the blessing He has bestowed on us. Tomorrow I ask that you once again keep us in your prayers as we head to driving a car, high school graduation, college and many, many more years. And of course do not be afraid to ask Him to provide a cure.



BillfromBoston

Dragonfly Heart Camp

It's been awhile since my last blog.  There's so much to write about during recent travels to the American Thoracic Society and NHLBI's Patient Interest Organizations meeting...but none of that is my subject today.

Instead I want to tell you about Dragonfly Heart Camp. 

Located on the Eastern Shore of Maryland, it's for kids who have received a heart and /or lung transplant, or are diagnosed with Pulmonary Hypertension.

Like many great ideas, the camp was organized out of one person seeing a need and being driven to meet it.  Congratulations and thank you to Rhonda Cataldo for bringing this important idea to reality and to Brian Hanna, a PH pediatrician at Children's Hospital of Philadelphia and a good friend of PHA, who leads the medical program.

That's Dr. Hanna in the picture to the right, wearing a medication pump on his head so a camper can swim.  How cool is that!

Here's a Dragonfly video you won't regret watching.

Oh, and, if you're as moved as I was, here are some upcoming events.

PH Professional Network...another step forward

PH Resource Network was launched as PHA's first medical membership group at the 2000 PHA International Pulmonary Hypertension Conference.  As we went through that first meeting in Chicago, we knew what our greatest hope was for the group.  It was that it would grow as a collaborative center for what we anticipated would be a growing medical field...a field that would develop with strong educational opportunities and effective clinical networking for the benefit of patients.

Has that happened?

Well, since 2000 the number of medical professionals working in the field has grown 60-fold.  PH resource Network has grown from primarily RNs to an increasing number of other allied health professionals, PAs, NPs, pharmacists, respiratory therapists and others.  Well ver 900 members so far!

To recognize the broadening of their membership and the development of programming across the range of professional interests, last month PH Resource Network changed their name to PH Professional Network (PHPN).

Today, they posted four new videos on their website to encourage all allied health professionals in the field.  Here's one...feel free to watch the rest.

Rev. White Comes to Wahington...

This week, PHA Board member Steve White came to Washington to help get the PH Research and Education Act introduced with a good number of original sponsors.  This is his story.  If you'd like to to what you can to help advance our common cause through contacting your member of Congress (in your own community) or creating media awareness, contact Elisabeth Williams, PHA's Grass Roots Campaign Associate.

________________________________________________________________

I don’t know about you, but whenever I hear the First Amendment of the Constitution mentioned on the news I usually think of freedom of religion, freedom of speech, a free press, and our right to assemble. But it is when I exercise the last clause of the First Amendment that I am most aware of how unique our form of government is and of how proud I am to be an American. I am referring to our right to petition the government for redress of grievances.


I took full advantage of this right on Monday, April 4 when I joined our friends Gavin Lindberg, Katie Kroner, and Rino Aldrighetti on Capitol Hill to visit the offices of my senators and congressman from Massachusetts and other members of the Massachusetts congressional delegation to seek their support for the Tom Lantos Pulmonary Hypertension Research and Education Act of 2011 which will soon be introduced by Rep. Kevin Brady (R-TX) and Senator Bob Casey (D-PA).

It was the perfect day to be in Washington. It was warm and sunny and the cherry blossoms and flowers were at their peak. And the reception we got from various congressional staffers was just as warm. Some of you may have met Sara Mabry, Sen. Casey’s legislative aide, at the Congressional Luncheon. She is fully committed to helping us get our bill passed and is working closely with the senator and with Mr. Brady’s office to get the bill introduced within the next week or so.

My congressman, Rep. John Olver, has been a long-time supporter of our efforts and his staffer assured us that he will be an original co-sponsor this time as well. When we visited the office of Rep. Jim McGovern (D-MA) the congressman himself came out and said “What’s up?” Gavin summed up our plea for him to be an original co-sponsor in less than 25 words and dropped the name of Tom Lantos whom he knew had been a close friend of McGovern’s. At that Mr. McGovern exclaimed “The answer is yes!”

Other staffers we met listened attentively as we told them what PH is and why this bill is so important. I told them about my daughter Christen and how she had lived with PH for some time before she was finally diagnosed, in spite of many visits to the doctor for shortness of breath and other symptoms that I now know are classic signs of PH. We explained how the bill will help educate doctors and the general public about PH so that the disease can be diagnosed as early as possible and thus prolong life. At the end of every meeting we asked the staffer to urge his or her boss to become a co-sponsor of our bill. And I reminded them that I’m from a huge French-Canadian family spread all over Massachusetts that really wants this bill passed. In fact, I think Gavin had the impression that I had more cousins at the end of the day than I had in the morning! It was an exhilarating day and a successful one.

Each of us has the right to do what I did on Capitol Hill. We all have the right to ask our government to help us solve problems that none of us can solve alone. Who else but the government would do anything to educate the public about a disease like PH? Who else but the government would focus research efforts on a disease that is unlikely to make big fortunes for drug companies?

So if you happen to be in Washington, or if you can make a special trip there like I did, drop in on your representatives. You’ll be surprised at what a warm reception you’ll get. Tell them your own PH story. Ask them to support our bill to fight PH. You’ll make a big difference and you’ll feel the pride in America that I felt when I visited Capitol Hill on April 4.

Eating bugs in Texas...

Last weekend, I was in Texas for the Woodlands CrawPHish Festival.  It's a great event that started out in 2008 under my all-time favorite event name - the Cure PH Bug Boil.

But there's more to the history.  In 1999, Jack Stibbs connected with PHA and said he and his family wanted to organize a golf tournament for PH research.  It would be PHA's first large-scale event.

Jack and his wife Marcia's daughter, Emily, was diagnosed with PH two years earlier, when she was five years old.  I met Jack and Marcia that year when they and I both attended our first board meeting.  That was a dozen years, 10 golf tournaments, a few galas and 3 bug boils/crawPHish festivals ago.  In the intervening years, Emily has grown up to be a lovely young woman who is now attending college and the events that this amazing family, including son Jake, and their devoted and driven team of volunteers have produced have generated over $1,800,000 for pulmonary hypertension research!

Jack will be the first to tell you that a true backbone of all these events is Matacha Saul.  Matacha began work with Jack as his legal assistant three months before their first event and was immediately (and willingly) drafted into working on that first golf tournament.  Today, she is the firm's office administrator, marketing coordinator and Jack's legal assistant.  Somehow, with all that, she is able to keep these amazing events going and growing.  At PHA's 2010 International Pulmonary Hypertension Conference, she received the Julie Hendry Memorial Award for her extraordinary efforts over the years. This year's effort - the CrawPHish Festival - drew over 3,500 happy bug-eaters, up from 2,000 last year!

Here's Matacha telling us why she does what she does. We are so very grateful.

Oh, and when this video is over, you might want to take a look at the other short video interviews I did with some other great folks (love that FlipCam!)...