A radio pucker..

A lot's been happening internationally...

Last week, Christine Dickler - Associate Director of our International Services program - sent me a link to an interview she did with PHA-UK's radio show (hosted by Paul Pennington) on our participation in their global Pucker Up for PH Campaign.

You can listen to the interview from this link by scrolling down to "Select a Show", clicking on Episode 9 and then segment 4. After the segment starts playing, drag the sound bar to 5:25 for the introduction and, almost a minute later christine's interview.  Phew - a lot of work - but worth it...and you might be interested in the other programming, as well!

The Campaign is counting their worldwide responses now. It will take 40,000 "blue lips kisses" to break the Guiness record and we're all waiting to see the results.  All we know that, led by Puerto Rico, well over 10,000 have been sent in by the U.S....and, whatever happens, this has been a great international partnership to build awareness in PH!

And thank you to our friends in Brazil...

In my last blog, I wrote about Debbie's trip to the ABRAF Conference in Brazil.  We've since heard more about her welcome and the work of our South American PH partners. And, we were surprised - and thrilled - when she returned with an award for PHA from the Brazilian PH Association.  Our sincere thanks.  We look forward to our continuing progress together.

Our partners in Brazil...

PHA's Director of Volunteer Services, Debbie Castro, spent the Thanksgiving weekend in Sao Paolo, Brazil.

She had been invited to speak at the third annual conference of the Associação Brasileira de Amigos e Familiares de Portadores de Hipertensão Arterial Pulmonar (ABRAF), one of 50 PH associations PHA works with worldwide. 

Here is Debbies first report about her experience:

There are about 55 people here at the Third annual meeting of ABRAF.

 For a patient to get on treatment in most states of Brazil, they must take legal action and sue the state to pay for treatment. 25 of 27 states don't recognize PH in their treatment protocols.

Paula Menzes lost her mom to PH and founded the organization 4 years ago. Debbie reported that Paula's father, Paulo, was an excellent MC for the event. They both speak English, Spanish and Portuguese.  That's Debbie on the left, Paulo in the center and Paula oon the right.

We value this and all our international partnerships in the fight against PH...and we're looking forward to hearing more when Debbie gets back!

Thanksgiving...

This year, as every year for the past decade, I am thankful for so many of the amazing people - the true heroes of life - that I get to meet in my work at PHA.

Too often, the great meaning and pleasure of this work carries a bittersweet side to it.  And that is no different this year.

So, this Thanksgiving season, Im writing about two good friends, recently lost. They never met each other but there were a lot of similarities in who they were...
That's Arlene Gabbert in the picture on the left. I rememember the moment it was taken. It was in August, 2007, at a Walk for PH in Minneapolis. The day was windy and wet and people were slow to leave the park shelter for the walk. Then Arlene, a longtime PH survivor, rolled her wheelchair out and led 200 people into the rain and around the loop. It was a brightness of spirit that lit a gray day. I will never forget it.


I saw Arlene a couple of times after that and was saddened to learn earlier this month that she was in hospice. A few days later, we got the word of her passing. We'll miss you, Arlene.

Around the same time, we also learned that Anabel Sivira had passed.

When she notified her fellow staff members of Anabel's passing, Christine Dickler wrote:

Anabel, a PH patient, was the former president of the Venezuelan PH association, FUNDAVHIP, and had recently moved to Costa Rica, where she hoped to establish a new group in a region with very little support. She hoped to apply for a Lantos Grant this year to support that work. Anabel was a positive, indefatigable energy in the community and her life is an incredible reminder of why our work is so important.

This summer, when we wrote about beginning her work in Costa Rica, Anabell sent this note. I think it speaks to making connections, even if they seem small. I hope that it encourages you…

“Friend, you don’t know the peace that is knowing that I have a friend like you and with [such an] Association of patients. This gives me much confidence and motivates me to continue with my foundation. [You all] are my support. Thank you very much.”

I wish I had a picture of Anabel to include but, for now, this page from the Association she led will have to stand as her tribute.

As we give thanks for the advances we are making, we remember all those who make them possible.

 

Calling in the chips for awareness...

Today is PH Awareness Month Blog Day...and I can't think of a better awareness story to blog about than what PHA board member Steve White brought to pass this past week. 

Faith Middleton is a Peabody Award winning (twice) broadcast journalist on Connecticut Public Broadcasting Network.  Dialing back to May 21, 1968, she was a student at Eastern Connecticut State College, a year ahead of Steve White.  For an article she was writing, she convinced the freshman to wear a barrel as part of the Eastern State College Dream Boy contest.  The scene was that Steve was disqualified from the contest due to the fish odor in the barrel.  Faith got her story...and Steve banked a favor due.

Forty-two years later, Rev. White found the picture of that interview and wrote to Faith - who he hadn't seen since college.  He opened the letter with,

"You owe me a big favor and I'm writing to call in the chips."

Well, Faith Middleton paid her debt with style and feeling - and in a big way.  Steve spoke about his daughter, Christen, and PH.  Dr. Terrence Trow who heads up the PH Center at Yale and Colleen Brunetti, an organizer of PHA's Generation Hope young patient group were interviewed in depth...and the interview has been posted online, where you can click on the player and hear it now. 

It's a great interview with a great lesson.  Good things can happen when you make the time to ask.

facebook...finally

Time is always my worst enemy...

There's always too much to do and too little time to get it done.

So, ever since our staff had set up a Facebook account for me a few years ago (during an Awareness Month fundraiser), I have been avoiding it religiously.

Today, I got a note telling me that I had "100 friends awaiting your response".

Being of a generation that gets hives when we let things lie - after a 10 minute tutorial from Debbie - I went to the page and started accepting friends.  In reading back messages, I saw the following from Sarah Peek's grandmother, Bonnie Corey

I am Sarah's grandma. I want to write for Pathlight. I always meant to when she was alive and have to now that she has passed. My youngest daughter (Sarah's aunt) is 12. They celebrated their birthdays every year. She did not know how to have a birthday this year. She turned her grief into a BBQ for their birthday where she collected a jar of "Pennies" and collected 588 dollars for PH. I still look up in the sky and sometimes Sarah is there. Hopefully myself, Aunt Emily, and Sarah's grandpa will be able to join Michelle, Dave, and Nathan at this years walk on Long Island. Hope to speak with you soon.

How could I let a note like that go unanswered since September 3? 

I'm moved and I'm sold.  My next step is to talk to Emma - like Debbie, one of PHA's social networking experts - about how I can keep a Facebook presence putting in 15 minutes a day.

.

Stem cell treatments...

Sometimes there's science, sometimes there's guesswork, sometimes there's quackery...

Several months ago, in an effort to help patient understanding and navigation of the very new and untested waters of stem cell therapy, PHA’s Scientific Leadership Council produced a statement and fact sheet on this subject.

Recently, we heard from Nick Hill, M.D. who leads the PH Center at Tufts University. He wrote to make us aware of valuable new information for patients produced by the International Society for Stem Cell Research (ISSCR).  They offer three wonderful new tools: the Top 10 Things to Know About Stem Cell Treatments, the ISSCR Patient Handbook and How Science Becomes Medicine. We took these items to leadership of PHA's Scientific Leadership Council and have just posted mutual links with ISSCR to make this material available to you.

We hope they are helpful to anyone trying to understand the current state of the stem cell world..

Kicking Off Awareness Month in Style...

Here’s a guest blog from PHA staffer, Katie Kroner. Awareness Month is an organization-wide event at PHA, but if you have questions about how to get involved, Katie and her team in the Advocacy and Awareness Department are a great place to start…

The PH community has grown and evolved over the years, and so has Awareness Month, but one thing hasn’t changed. Raising PH awareness is about sharing your story. No matter who you are.

Carol Lindstrom knows the importance of PH education. When she was finally diagnosed, it was because her doctor had recently read an article about the disease and referred her for a right heart cath. This week, Carol and her daughter, Cindy Klein, kicked off Awareness Month in style—they advertised their special event and shared information about the symptoms of PH on TV and in two newspapers.
Here’s Carol and Cindy on KMTV Channel 3

As members of the PH community, we’re part of something big. Dr. Michael McGoon says, “You immediately feel, once you have identified other patients with pulmonary hypertension or other physicians that are dealing with it, that you are a member of a common family directed towards a very focused and consensual goal — to cure this thing.”

During Awareness Month, we’re telling the world our goal and asking them to lend a hand in our climb toward a cure. I hope you’ll join us!

P.S. Special events are just one way to raise awareness about PH, so the fact that Phenomenal Hope for a Cure was one of seven fundraisers that took place this past weekend tells you something about our Awareness Month momentum. From the Inaugural Patrick Garcia Memorial 5K in New Mexico, to the 3d Annual Baltimore PH Walk for Hope, to the 10th annual Stanford Race Against PH, we’re raising awareness coast-to-coast. A calendar of upcoming PH events is available at www.PHAssociation.org/Calendar

Charity's back...

The last time I blogged about Charity Tilleman-Dick was in October 2009, shortly before her double lung transplant.   Several years earlier, as a rising young opera singer living with PH, Charity gave a concert to introduce official Wahington to an understanding of PH.  Her pianist at the Kennedy Center that special night was then Secretary of State, Condoleezza Rice.  The concert generated world headlines.

Late last year, while speaking at the Cleveland Clinic's annual PH Symposium, Drs. Paul Hassoun, Omar Minai and I walked over to the hospital to visit with Charity, who was beginning her long road to recovery.   She was in bed but was anxious to speak and her brother voiced the words she was mouthing. We saw her take one of her first brief walks down the hall.

I didn't hear much about Charity for awhile - until late May of this year - when she burst into the press again.  Charity, and her spirit and extraordinary voice were back!  She was performing at the Cleveland Clinic Patient Experience Summit, and, in the second song of this clip, dedicates Gershwin's, Someone to Watch over Me to the doctors, nurses and hospital staff who had taken care of her.



Then in September, she gave an impromptu concert at the inaugural Colorado PH 5K Walk/Run and, this past Tuesday, opened the TEDMED health conference.

Yes, Charity's back...we're glad of it...and I'm sure we'll be hearing more from her!

Awareness Month is almost here...

PH Awareness Month is a special time to focus our communities on understanding PH.  It begins next week...and a lot is already happening.

Press is starting to build. Diane Ramirez from North Carolina and Vallerie McLaughlin, M.D. from the University of Michigan had a great joint interview with Dr. Radio (Sirius) from both a patient and physician perspective. It's well worth a listen.  After the interview, Diane also got word that the New York Times would like to interview her for their Patient Voices series.

If you'd like to get your own free Media Guide, Media Action Alerts and other media resources, they're all waiting for you here.

Every year more Members of Congress and their staff members learn about PH at our Congressional Luncheon.  This year it will be on Wednesday. November 17 at noon at the Library of Congress, Members Room, Jefferson Building.  If you're in the Washington, DC area, you're welcome to attend.  If not, we ask you to call your own Member's office and make a personal invitation. PHA has all the information you need  to find your Representative and make an effective connection.

More and more PHA members are involved in creating awareness by inviting their local goverments to issue Awareness Day, Week or Month Proclamantions.  Patients Doug Taylor and Leslie Polss are having so much fun with this they've created a video and cartoon showing how it's done. There's even a model proclamation for you to use as a template.

If you're into social networking, there are special programs available for you.  Blogging Day is November 16.  Find out how you can use your personal story, blog or Facebook page to build Awareness.
 
November is Awareness Month.  It's a great time to put in a little effort to make a big difference.  We hope you'll take advantage of one of the many choices and how-tos available to let your community know about pulmonary hypertension.  If you do, we all win!

Alfred Fishman, MD: an appreciation

On Friday morning, as I was getting off a plane in Raleigh-Durham, I received a note from Carol Vreim (via Dr. Steve Kawut) that Dr. Al Fishman had died in Philadelphia at 92 years of age.

It was appropriate that the note came from Dr. Vreim, who in the early 1980's worked with Dr. Fishman and others in developing the NHLBI pulmonary hypertension registry which formed a cornerstone for much of the research over the following two decades. Dr. Fishman argued that registry into existence.

Put simply, Dr. Fishman was one of the early builders of the field of pulmonary hypertension.  His work made much of today's progress possible.

Here is what three of his colleagues have written...

The modern era in pulmonary hypertension began with the NIH Registry of Primary Pulmonary Hypertension in the early 1980’s that defined the disease accurately and began to base therapy on scientific study. Many of us in the field and in PHA started our clinical careers through work on this registry.

Al Fishman began his career in the pulmonary circulation in the 1940’s as a fellow with Cournand and Richards, who won the Nobel Prize for their work defining the physiology and diseases of the pulmonary circulation through right heart catheterization. Al Fishman was a major reason for the initiation and success of the NIH registry and for developing this second generation of PH researchers and doctors. We  owe him a great debt as we stand on his shoulders and are grateful for his vision.

John H. Newman, MD
Elsa S. Hanigan Professor of Pulmonary Medicine

Vanderbilt University School of Medicine
Chair, PHA Scientific Leadership Council

Al was the grandfather of PH. To me, that says it all. His insight and dedication to the field is why we have been so successful over the past 6 decades in studying the pulmonary circulation, which had to occur before we could even attempt to develop treatments for PH. I will always be indebted to what Al has taught me, that is, how to think and expand our dreams, exploring far beyond the horizon. He truly was a legend in his own time and will remain a great figure in the field of PH. 

Robyn Barst, MD

Professor of Medicine Emerita

Columbia University School of Medicine
Past Chair, PHA Scientific Leadership Council

The sadness of this news is buffered by the recognition that he led such a productive and gratifying life, and did it in a way that was inspiring to many, including me.  He will certainly be missed.  It seemed poignant and meaningful that the news reached many of us as we were at the REVEAL Registry Investigators Meeting in Boston, hopefully carrying on one of the many threads of his career in trying to further understand PAH by means of a national database

Michael McGoon, MD

Professor of Medicine

Mayo Clinic School of Medicine

Past Chair, PHA Scientific Leadership Council

Judy Simpson and her husband Ed who were among PHA's founders added this...

Dr. Fishman was an important person in the early days of UPAPH. [PHA's original name was United Patients Association for Pulmonary Hypertension.] 

Dr. Walker Long, with Burroughs Wellcome at the time, told us that if we could get Dr. Fishman involved other physicians interested in pulmonary hypertension would join in. How right he was!

Dr. Fishman was our keynote speaker at the First International Pulmonary Hypertension Conference in Stone Mountain, Georgia, in 1994. As many of you will remember this was the largest gathering to that date of physicians interested in pulmonary hypertension.

Dr. Fishman encouraged PHA and offered helpful suggestions to us along the way. We owe him a great debt of gratitude.

I had the pleasure of spending time with Dr. Fishman at many of the PHA International and American Thoracic Conferences and, like the Simpsons, appreciated his understanding and support of PHA's efforts.   When we were a small organization, he gave us encouragement and credibility.  As we grew, he continued to share his wisdom and knowledge.

In 2004 in Miami, Dr. Fishman delivered his last plenary session lecture at a PHA International Conference.  It was titled One Hundred Years of Pulmonary Hemodynamics. Typical of who he was, he wanted the lecture to provide understanding of progress to patients..and it did.

Thank you, Dr. Fishman, for all you did to make this a vibrant field with growing hope for patients. 

Your contributions were immeasurable.  Your legacy continues.


___________________________________________________________

Dr. Fishman wrote over 250 papers during his career. I thought you might like to read one written in 2003 and titled, Primary Pulmonary Arterial Hypertension: A Look Back.

For the many who have already asked whether PHA will establish a tribute fund for Dr. Fishman, that is being discussed and we hope to make an announcement shortly.

PHA Mentors send e-mails from the heart...

Here is a guest blog from Emma Bonanomi.  Emma has been working on developing PHA's new targeted programming for newly diagnosed patients, young adults, patients with associated diseases, family members of adult patients and family members of children with PH.  The newest step is PHA's mentor program. Here's what she writes about it...

Some say letter writing is a lost art. Tell that to the patients, parents and spousal caregivers who recently signed up to spend hours at their computers each week, emailing carefully crafted hope and encouragement to people living with PH. With precisely 26 mentors poised and ready to write, there are as many pen pals on the PHA Mentor team as there are letters of the alphabet. This is an apt coincidence for a group of people who make such powerful use of words.

From Anna in Colorado, living with PH, scleroderma and lupus; to Bill in Boston, with a teenage daughter who was diagnosed with PH in kindergarten; to Colleen in New York, diagnosed with PH in 1975 and eager to answer questions about her experiences with supplementary oxygen, there’s no doubt that the PHA Mentors represent a broad range (if not the A to Z!) of backgrounds and experiences.

Yet like so many members of our growing community, each mentor has a deep commitment to helping other PH patients and caregivers that makes them more alike than different, despite their various ages, associated diseases and hometowns around the globe. This is a set of knowledgeable and caring individuals who value the solace of thoughtful language and shared experience. They are prepared to do what it takes to share their hope and courage with members of the PH community who are looking for help finding information or for a virtual shoulder to lean on.

Take Joni, a loving husband and father in Jerusalem whose wife was diagnosed with PH in 1998. On top of his commitments as a caregiver and as chairperson of PHA Israel, Joni was so dedicated to helping other PH caregivers that he stayed up until 3 a.m. Israel time to participate in a mentor webinar training. And take Liz, a PH patient from North Carolina who drove 30 miles to the nearest McDonald’s for Internet access to prepare for the Mentor Program while she was receiving treatment in Minnesota.

Why do these volunteers make such sacrifices to do what they’re doing? Joni says, “I love to help other caregivers … and hopefully help them appreciate the positive aspects of their situation." Liz says, “I am happy, positive and motivated to do all I can to help others deal with our disease." To these PHA Mentors, who provide support through words, sentences and paragraphs, hope is more than a motto. It’s the air they breathe and the driving force that keeps them striving to reach more people affected by PH.

Having exchanged quite a few emails with these mentors as we prepared to launch this program, I can let you in on a little secret: their brand of hope is contagious. But you don’t have to take my word for it. Email a PHA Mentor and find out for yourself.

Visit www.PHAssociation.org/Mentors to email a patient, parent or caregiver mentor. (Mentors are available for case-by-case questions or for email relationships up to three months in length. Free PHA website sign-in is required to view mentor profiles.)

A rare convergance of meetings...

When I was a kid, I was fascinated by rare convergences of planets.

Maybe that's why last week was so special (maybe there were other reasons, too).

On Thursday, PHA's Corporate Committee met, focusing on the major agenda item of how industry can help PHA build greater awareness of PH among the American public.

This was followed on Friday by PHA's Scientific Leadership Council meeting. Providing guidance and direction for PHA's complex array of scientific and medical programming and interest, this leadership group's agenda is always dynamic and wide-ranging.  Last week's meeting took us through discussions and decisions on everything from expansion of PHA's research program, analysis of an investigator training program, new developments related to our medical journal, and consideration of a new prostacyclin document...to the pros and cons of defining expert PH centers, recommendations to Social Security on how they handle PH disability issues, discussion of next steps for PHA's Medical Education Fund programs and the rapid evolution of our pediatric programming. As always, it was a busy and fruitful day.

But that wasn't the end of the week.  On Saturday we had much more in Towson, Maryland.  With their new Chair, Louise Durst RN, PHA's PH Resource Network Executive Committee met to plan their next steps.  This group within PHA, now composed of over 900 nurses, pharmacists, respiratory therapists and other allied health professionals has been growing rapidly.  their leaders discussed their 2011 Symposium, changes in their strategic plan and support of various PHA patient and medical programs.

Simultaneously in the same hotel, PHA's Baltimore/Washington area regional "On the Road" patient Conference took place.  Almost 250 attended a full day of programming that included sessions on current treatments, diagnosis, impact on family and relationships, eating better, traveling with PH, exercise and yoga and a whole lot more.  All the committee planners (led by Charles Burger, M.D. of Mayo-Jacksonville) and speakers were volunteers, including many nationally and internationally known physicians.

Well, I wish I had time to write about the great people I met and the stories I saw and heard but there's not much time to put our feet up. Our next regional "On the Road" Conference is next Saturday in Dallas and I'll be preceding that with a Thursday 30 City Medical Education program led by Gregory Elliott, MD in Provo, Utah.  More on that next week!

Back & forth...& worth it

Sometimes, it's worth the jet lag...

I just got back from a four day trip to Barcelona, Spain...well, maybe a little less with the time in the air.  The itinerary included a presentation to the leaders of PHA Europe, work on a global survey of PH patient and caregiver depression and anxiety issues, getting to know a new company in the field and attendance at the European Respiratory Society Conference.

The PHA Europe Annual General Assembly was a thrill for me to attend.  I remember just a few years ago that this confederation was just an idea.  Now- in Castelldefels, a beautiful beach suburb of Barcelona - it is clear how far that idea has come.  Representatives from PH associations in Austria, Belgium, Bulgaria, the Czech Republic, France, Germany, Greece, Hungary, Ireland, Israel, Italy, the Netherlands, Norway, Poland, Portugal, Slovakia, Spain, Sweden, Turkey and Switzerland were meeting to discuss ways they can better work together and help each other grow.

Many of the leaders, including PHA Europe's president, Gerry Fischer (Austria), and Vice-President, Pisana Ferrari (Italy) and Melanie Gallant-Dewarin (France) are old friends who have attended PHA Conferences and who we've worked with on many projects.

I was honored to be invited to give a presentation about PHA in the U.S.  As I spoke and heard the questions during and after, it was clear that, while we may speak different languiages and live in different places our cause is the same...and that the cooperative spirit and common purpose that has made progress possible and rapid in the U.S. is the backbone of growth in Europe, as well.

We look forward to our continuing and  growing partnership.

Out of hope...joy

I couldn't resist one more posting on Lil Long's swim.  Yesterday, PHA board member Steve White who represented us at Lil's crossing sent his story on what took place and its meaning. It's wonderfully told and well worth sharing.  Also, last night Lil sent a video (which you can see below) made by her friends Nancy and Lisa.  It's tells the adventure of the Mt. Moriah Choir in getting to the victory celebration.  They surely do know how to party in the Delta!



Dear All,



I had a great trip down south for Lil Battle Long's swim across the Mississippi River to honor her dear friend Nicky Roberts who lost her struggle with PH just three years ago.


Friday I flew from Albany, NY to Memphis and from there drove to Cleveland, Mississippi though fields white with cotton to meet up with Doug Miller, producer for the film crew, and Gareth Gwyn from Actelion. We then headed over to Lil's home in Duncan and enjoyed a great meal in one of the most interesting homes I've ever been in. It was creatively remodeled by Lil and her husband Henry Earl Long. It's almost impossible to describe what they've done with this house. Suffice it to say that the kitchen, which is like something you'd see on the Food Channel, has bright red cabinets and counter-tops made from rare African zebra-wood, and a guest bedroom has a ceiling covered with antique doors that Henry Earl got from an architectural salvage yard. The main thing about their home, though, is its warmth and openness to guests. When you spend a little time with Lil and her wonderful family you get a glimpse into how incredible her support system is and how loving and courageous they all are in facing Lil's PH. But we made it an early evening since Lil wanted to get to bed and get rested up for the next day.


In the morning, all wearing red T-shirts with "Go, Lil, Go!" printed on the front (courtesy of our friends at Actelion) we met up with the film crew and Henry Earl's step-son Justin to make our way over to a boat-launch on the river. We drove up and over a levee and down the other side to the shore of a lake that is parallel to the Mississippi River. There we met up with Lil, Henry Earl, Lil's son Battle, a newswoman from a local TV station, and a friend of Lil's who is a respiratory therapist. Justin and Henry Earl's boats were put into the lake and we headed south to a cut that gave on to the mighty river itself. Where we were is a very remote and wild area teeming with birds of all kinds (egrets, wild turkey, gulls, etc.). As we went through the cut connecting the lake and the river, enormous carp, which were disturbed by the sound of the boat motors, leapt out of the water to a height of two or three feet. Some of the fish were 10 to 15 pounders, according to Justin who grew up on the river and was a superb tour guide.


After checking out a few possible launching sites, we tied up the boats at a sandy stretch and Lil began to prepare for the swim. She was interviewed by Doug for the film first, and then she asked for some private time with me to pray and take communion and have a blessing while the others waited a dozen yards away (we Episcopalians stick together!). Finally Lil put on an inflatable floatation device, flippers, webbed gloves, and a snorkel. She also had an audio player with some Celtic music that had been some of Nicky's favorite music. She was ready to go, but she had to wait for a tug pushing about 8 or 10 barges loaded with rocks to pass by. After collecting her thoughts for a few minutes, she gently eased into the river and headed for the Arkansas side which was about a quarter of a mile away, straight across.


The two boats stayed near Lil the whole way over. At one point Lil rolled over on her back and began laughing. She told us later that she had felt something slimy -- probably a huge catfish or some other denizen -- slide along her leg and this made her laugh. Lil had predicted that the swim would take about an hour. But 27 minutes after pushing off from the Mississippi shore, she was on the Arkansas side feeling tired but triumphant. She rested in the water for about 5 or 10 minutes before getting into the boat. She said she was amazed she had done it and now knew she could do anything and that PH would not stop her. She said she felt Nicky with her all the way, as well as the presence of God, and my daughter Christen, and Doug's mother (who also died of PH seven years ago). It was a powerfully emotional moment for all of us who were there with most of us shedding a tear or two.


On the way back to the boat launch we estimated that, given the current that carried her down the river, she swam a total of about half a mile. Pretty good time for someone with PH! As we came out of the cut and into the lake, a giant carp leapt out of the water and smacked Gareth on the left shoulder hard. Justin estimated it was about a 10-pounder and we were going maybe 30 miles an hour. The fish left a slimy, smelly impression on the left sleeve of Gareth's T-shirt that I thought looked rather like Homer Simpson. We all advised Gareth that he might want to change his shirt before going to the airport.


This was followed by a party with the best fried catfish I've ever had, a wonderful church choir of young people from Jackson, MS, a local flamboyant antique blues singer who worked the crowd, and lots of Lil's family and friends. It was a very happy occasion and there was much pride in Lil's accomplishment. Lil glowed and, with a glass of champagne in one hand, gave a hug around the neck to one and all and accepted everyone’s congratulations.


What a day of honoring Nicky and all who have, and continue to struggle with PH! Lil’s swim was an act of courage and determination that shows the value of exercise for PH patients as well as the bonds of love and hope that unite everyone in the PH community. I'm confident that Lil's story in what I'm sure will be a superb film will be inspiring to many people who fight this wicked disease for along time to come.


I am full of gratitude for the opportunity to be a small part of this incredible event.


Steve White

...and as a bonus, here is the Mt. Moriah Choir performing at the celebration!

On the ramp to Awareness Month, part 2...

Awareness Month in November may seem like it's a long way off...but, here at PHA, the preparations have been long underway.

This year's theme will be Climbing Toward a Cure...in recognition of the focus brought to PH awareness by Drs.Benza and Frantz and Jessica Lazar, P.A.'s Kilimanjaro climb and the related Unity walks in communities throughout the U.S.

On Firday, PHA's 2010 Awareness Month page launched.  I hope you'll visit and consider ways you can help us all Climb to the Cure.

In the meantime, I hope you'll take a look at the video we presented just before the climbers took the stage on June 25 to tell their story - and ours - at PHA's International Conference.

On the ramp to Awareness Month, part 1...

It's been an exciting Saturday morning...

While I've been at my desk preparing for upcoming meetings in Europe and the U.S., I've also been tracking PH patient Lil Long's heroic swim across the Mississippi in memory of her late friend, Nicky Roberts who lost her own struggle against PH in 2007 (See August 27 and May 31 blogs).

All morning PHA Board Member, Steve White and Actelion's Gareth Gwyn and I have been texting back and forth.  Steve is representing PHA at Lil's swim and Gareth's company has loaned us a film crew to document Lil's amazing feat.

At 10:36am, Gareth e-mailed me the picture on the left.  That's Lil coming out of the Mississippi River after a successful swim and months of preparation and building PH awareness in her region of the country.

This comes 10 days after PH patient Christie Breault's appearance on the Today show. 

As we move toward November and Awareness Month, the question is, "What's next?"

All I know is that whatever the answer, the certain thing is that awareness is the essential first sep toward solutiions and PH is no longer living in a dark corner.  Lil, Christie and all who are building awareness, thank you for bringing the power of one - your own efforts - in partnership with and to the benefit of many.

If you're moved to be a part of building PH awareness, Awareness Month is coming!

Making Noise About PH

I speak and write a lot about the power of community.  It's what makes advances in so many directions possible in the fight against PH. 

On Friday, Margaret Beardsworth, who works on insurance issues here at PHA came in with some great news.  A partnership of patients, family members and medical professionals that began 3 years ago has been making steady advances in working with the Social Security Administration (SSA) to understand the special issues of PH patients, especially as it relates to diability issues.  Friday's victory comes out of the work of a team of physicians that has been working with the Institute of Medicine under contract to SSA.  The impact is expected to be very large for PH patients.

Here's what Margaret wrote today in her guest blog...

“YAHOO!!!!!” That was Alan Harder’s response Friday when PHA received an official report from the Institute of Medicine (IOM) committee that outlines a set of recommendations for the Social Security Administration (SSA) to use in updating their Cardiovascular (CV) Listing of Impairments.


Alan is a former SSA employee and PH caregiver. Like many others in the PH community, Alan has been working for three years to guide the SSA in the process of updating the PH-related language in their Listing of Impairments - the list of disease states that adjudicators use to determine if an applicant qualifies for Social Security Disability (SSD).


Friday’s report is a detailed set of recommendations that touches on the broad spectrum of cardiovascular disease states. It contains an entire section dedicated to PH, with language mirroring, almost word for word, the recommendations that PHA provided the IOM. You can learn more about the recommendation process from one of Rino’s past blog entries.


If 328 page report is a little heavy, you can read the report introduction instead - a 4 page PDF that gives an overview of the IOM’s work and mentions pulmonary hypertension twice.


What does this mean? We are one step closer to streamlining the SSD application process for PH patients by updating the language used to determine if a PH applicant is disabled. The Social Security Administration is still a long way from releasing completely updated cardiovascular listings, but the PH community is heading in the right direction!


A big reason PH is receiving so much attention in this process is because of the united efforts between the Scientific Leadership Council’s physicians, PH community members and activists and the government. A special thank you to all who have volunteered their time and expertise on this initiative, including Drs. Ronald Oudiz (Harbor UCLA), Robyn Barst (Columbia University emeriti), Harrison Farber (Boston U.), Vallerie McLaughlin (U. of Michigan), Gregory Elliot (University of Utah), Adaani Frost (Baylor), Michael McGoon (Mayo), Nickolas Hill (Tufts), Erika Rosenzweig (Columbia), Charles Burger (Mayo) as well as Carol Vreim, Alan Harder, Gavin Lindberg, and Jennifer Jaff.


Making noise about a rare disease like PH can be challenging, but we’ll continue to push forward, one step at a time, each time making a bigger difference.

Swimming the mighty Mississippi River...

All of us at PHA have been following PH patient Lil Long's preparations to swim the Mississippi River in memory of her friend Nicky Roberts and to build PH awareness.  The story is compelling.


Today, we got great news from Actelion that they will provide PHA with a film crew to cover the September 11 event, presenting the magnitude of the achievement and help us also use it as a platform to create a video that will offer a platform to talk about PH and exercise.  The film crew will be Glenn and Glenn Productions, the folks who did such a great job procucing our 2010 Conference Video.

Jess McKearin, who leads our special Events team, sent me a passage from Lil's journal in which she talks about her recent test swim.  I think it's well worth reading.

It broke my goggles; it tore my snorkel away from my mouth. My nose clip nearly drowned me because I couldn’t get enough air. In other words everything I thought had prepared me for over a year was useless. My heart was pounding I guess as hard a heart can pound. I swam in circles for fifteen minutes from being so disoriented I didn’t know up from down. That current is a powerful force to be reckoned with.



When I finally figured out what all I was doing wrong, I grabbed the boat (I was swimming with no life jacket or tether) and had to rethink everything I had learned.


First of all, you CANNOT keep your head under water and swim the river, thus the disorientation. Second, you have to spend money on a very good pair of goggles. You cannot do completely without them because my left eye where my goggles broke is almost swollen shut with sand! Third, keep your eyes on the prize, the other side! Fourth, if you ever get a little over halfway across, the current starts to help you instead of hinder you. It will begin to push toward the other side. And, most of all, fifth, stay calm! It’s hard to breathe when your heart in beating 90 miles an hour!


When I figured all of this out, I finally started getting somewhere. If I hadn’t spent that time going in circles, I probably could have made it three fourths across a very swollen river.


Next time I’ll be calmer. It didn’t help any that right before I went in the water the Cap’m and a media guest saw a four foot alligator gar! I really did NOT want to hear that.


When I got to the middle, I paused to look around. I absolutely could not believe that I was in the middle of that great river of ours. It gave me hope and I felt Nicky all around me. I was completely awestruck.

To see Lil speak about her test swim, click here.

She's been building  a lot of awareness through TV (the clip below talks about a swim date earlier than what is now a September 11 planned date)...

New name, same great program...

A number of years ago, I heard a story that instantly helped me understand the importance of PHA's Patient to Patient Helpline.

One night, a young woman called 800-748-7274.  She was with her father in the parking lot of her doctor's office.  They had just left the doctor's office where she had been given a diagnosis: pulmonary hypertension.  The doctor told her she had a year to live and gave her a PHA brochure. Devastated, she called the Helpline number listed there. 

The volunteer who picked up the phone was Dorothy Olson.  After listening to the young woman's story, Dorothy said,                   
                 It's not over.  I have been living with this disease for over 25 years.

...and everything changed.

Today, Debbie Castro, PHA's Director of Volunteer Services, asked our staff to start circulating some important news.

Here it is...

PHA’s Patient-to-Patient Support Line is a new name for one of our very first patient services. Formerly known as the “Helpline,” we’ve renamed it to highlight one of the biggest benefits of this service: immediate connection with another patient. Our Support Line volunteers also provide information, like the contact point of a nearby PH clinician or support group, but we really want patients and caregivers to know that a PHriend is always a phone call away.

800-748-7274

Need to talk to another patient about anything and there’s no support group nearby or the next meeting is several weeks away? Are you a caregiver that needs information and support? No internet access to our Discussion Boards or online chats? No problem—you can always dial our Support Line to talk to another patient right away.

Spirit that drives us forward...

I write a lot about the patients and family members and medical professionals who drive our common cause forward. Today, I'd like to write a few words about our PHA staff...



Early on, our hiring values evolved to include commitment to cause, a demonstrated ability to work in teams and folks who are as bright as we can find them. One of the things I've always appreciated about our staff is their ability and willingness to collapse around a problem and solve it.


Recently, two staff at the supervisory level and one program associate left PHA for personal circumstances that gave us shorter than usual time to prepare. Unfortunately, all three were in the same work area, so it put a lot of pressure on our remaining medical services team.


Not only have various staff been jumping in to fill the gaps but they have been doing so with gusto!

In a great show of spirit, they printed up badges in the picture you see, carrying the message

Medical Services

Special Forces

The Few - The Proud

So, helping Rachel Wheat and Meghan Finney, are Doreen Lucadamo, Amanda Butts, Meghan Tammaro, Suzanne Flood, Carsten Hailey, Patti Lalley, Patty Hunt, Patty Scuderi and Jessica Ritter As the badge says, they and their peers are the Few and the Proud! And we're proud of them and all the good folks here who are doing so much to change the history of this disease.


Many thanks.

Life has many paths...

Roham Zamanian is a bright and energetic young physician who I've known for the past several years.  When Dr. Ramona Doyle left the Vera Moulton Wall PH Center at Stanford University, Dr. Zamanian was the natural choice to lead the Center's Adult Pulmonary Hypertension Clinical Service.

Today, I got to know Roham in a different way...through his extraordinary life's journey 

From our PH community, I have learned that heroes are defined by the way they handle what life puts in front of them. Roham's quiet and clear sharing of his important story is heroic in my view.

When I asked his permission to share it with you, he wrote back:

I feel that I have arrived at a moment of clarity in my life – that I have found the purpose – and working in the PAH community is just what I was meant for. I’ll be honored for you to share my story.

93 medical education meetings..and more

In early August, I traveled to Cheyenne, Wyoming.

Dr. Dave Badesch, a Professor of Medicine at the University of Colorado was presenting a PHA medical education session for physicians and allied health professionals.  A leader in the field, Dr. Badesch is also in charge of the highly regarded PH Center at the U. of Colorado and has been a past Chair of PHA's Scientific Leadership Council.  He is one of the early group of physicians who began building PH research and clinical practice more than two decades ago.

The program Dr. Badesch was delivering is one of 30 being presented this year by expert PH physicians in smaller cities throughout the U.S.  Chaired by Darren Taichman, M.D., Ph.D. from the University of Pennsylvania, the 30-City program is connecting expert physicians with less experienced PH physicians in ways that will provide increased knowledge, earlier diagnosis and better treatment for PH patients.

It is one of three PHA initiated face-to-face medical education programs - PHA's Preceptorship and On-Demand programs being the other two - that will this year provide medical education in pulmonary arterial hypertension through over 90 sessions (up from 40 last year and 6 per year between 2005 and 2007). That's 90 education sessions in a disease state with between 20,000 and 30,000 diagnosed patients.

Such intensive medical education in a relatively small field is possible for several reasons.  First, PHA is blessed to have two active and engaged medical sections, one with 500 physicians, the other with 850 nurses and other allied healt professionals.  Through the interest and commitment of these medical leaders, PHA has a pool of expert educators willing to step forward and further develop this important new field of medicine. Second, our industry sponsors - Actelion and Gilead at the Platinum level and United Therapeutics and Pfizer at the Silver level - understand that co-sponsored medical education brings greater credibility to the content, with a greater sense of ownership and engagement by the participating medical educators. 

We believe this model of delivering medical education provides a win-win for everyone...most importantly the patients who are living with pulmonary hypertension.

A strange world...

In 2001, Alicia Mundy wrote Dispensing with the Truth.  It is the story of the battle over fen-phen, the drug combination that was ultimately withdrawn from the market because some patients who were taking it developed heart and lung damage, most notably pulmonary hypertension.

Many of PHA's members and some of our board members became PH patients after taking fen-phen.  Indeed, Alicia Mundy's book opens with the story of Mary Linnen, the daughter of former PHA board member Tom Linnen and his wife, Mary Jo.  As the liner notes describe Mary, she was "a healthy young woman...who took the drugs for only twenty-three days to lose weight before her wedding, and then died in the arms of her fiance a few months later".

I've been thinking a lot recently of Mary and Tom and Mary Jo and others like Candi who lost their health or their lives or family members to PH developed through fen-phen (Pondamin and Redux).

On July 13, the New York Times reported under the headline F.D.A. Review of Diet Pill Relieves Investors that the drug Qnexa had received a positive staff review of it's safety and effectiveness.  The maker's shares immediately soared 15% while two competing drugs soon due for FDA review went up 9% and 21 %. 

I understand that weight loss is the holy grail of American life but I - and all of us who are part of the PH community - have seen the collateral damage.  Qnexa contains the amphetamine phentermine -- one half the ingredients of the fen-phen combination.  The makers would say, it's the safe half. 

Maybe.

On June 15, the New York Times carried a second report that the FDA panel of expert advisors reviewing the drug on June 14 had voted 10 to 6 to recommend against approval of the drug based on safety concerns.

Now the FDA has a choice.  We'll see what they do.

Carol Vreim...

Today is Carol Vreim's last day at PHA...

She certainly been been a tremendous asset to PHA as a part-time employee for the past several years but her life and work have been intertwined with the PH community since the 1970's.

In 1973, a group of physicians with an early interest in PH met under the auspices of the World Health Organization. Their goal was to review the current state of knowledge on Primary PH (later described as idiopathic PH).  Following that review, they proposed the establishment of a multicenter collaborative study. This led to the historic and invaluable NHLBI PPH registry which enrolled 187 U.S. patients  from 1981 to 1985. Carol was in charge of that registry.  It was this information that framed much of the research that took place over the next two decades.  It also helped to develop a network of physicians who built this little known field into today's vibrant and active medical community.

Carol also was a catch point for patients during a period of great isolation.  They would call her desk at the National Heart Lung and Blood Institute and she would put them in touch with each other and, as PHA came into existence, with PHA leaders.  As a trustee of the Dauna Leigh Bauer Foundation, she kept Dauna's legacy alive, through early Foundation gifts that helped PHA establish itself in the early days.

After Dr. Vreim's retirement as Deputy Director of the Lung Division at NHLBI, we were honored to have her talents at PHA.

Thank you Dr. Vreim.  Good luck in your move to Pennsylvania. Have no doubt that we'll miss you and that we'll stay in touch!

A striking point...

On June 29, the following appeared in an article titled FDA Weighs Rare Disease Drug Regulation But Agency Nixes New Division in the publication, FDA Week.

“Experts suggest that there are roughly 200 FDA-approved treatments for the nearly 7,000 rare diseases that exist globally. So while there have been many successes, the unmet needs are huge,"

I read these words and read them again. 

Why were they so striking to me?  Do the math and consider this...

With 8 treatments for PH, 4% of the approved treatments for the 7,000 rare diseases are for PH.

While PH is a rare disease, our patients' and families' focus on putting a spotlight on this disease has brought it out of the dark corners and into the attention of our dedicated researchers and clinicians and the pharmaceutical companies considering new treatments to pursue.

We are not yet where we need to be...at this point there is not a cure.  However, these numbers  are the result of hard work toweards a clear target.  They.are one indicator that we are moving forward rapidly thanks to the dedication of our community of hope.

Conference bits – Heroes: Wendy and Bill Bockhorst

One of the greatest parts of the Conference experience for me is the connections and reconnections.

Bill and Wendy Bockhorst are among the heroes I have seen at past Conferences. I knew that I would not see Wendy this time. She passed last year. I did see Bill though and it reminded me what great people they are.

Together, they had for years organized golf tournaments and casino nights in Arizona and Wendy had been a support group leader.

Seeing Bill reminded me of Wendy’s courage and her words.

“One main reason that I get involved through special events is because my PH is genetic…if I can continue working to raise funds for and awareness of this disease, then maybe the next generation won’t have to suffer as much."

It is because of people like Wendy that the dedication on the video we create at Conference is unchanging. That video – and, indeed, the Conference – is dedicated to

“…the memory of those who have gone before, to the spirit of those who fight back today and to those yet to come.”

Wendy is on the left in the picture above.

Conference bits: on a personal note

Our PHA staff prepares detailed outlines for each of our plenary sessions. With so much going on, it’s important that our meal and other programming not run over and keep registrants from other activities. I receive and approve those timelines.

At our Friday night dinner, something seemed wrong.

I saw Amanda escorting Pat Paton, one of our founders, to the stage. Staff was also putting a chair on the stage next to the podium. This was not in the script.

The next thing I knew, Pat Paton was inviting me to the stage to sit in the chair. Her presentation was about my 10 years at PHA. While that was quite a surprise, a bigger surprise came a moment later. Pat spoke about my wife, Laura, not being able to attend past Conferences but now she was in the room. I started looking around for a speakerphone. Instead, I saw Laura starting to come up the stairs to the stage. She really was in the room!

Following Pat’s talk, her sister and PHA co-founder, Judy Simpson presented me with a very special plaque. I say special because it includes the names of all the board members I have served with during the past decade.

It was a wonderful surprise and one for which I will always be grateful.

Conference bits - A radio marathon

Before I came to Conference for the first time in 2000, I spoke with our board chair, Bonnie Dukart, about using the event to generate press and build awareness of PH. She told me there had been board concerns about such public visibility. At the time there was fear of losing jobs.

That has changed over the years, with our community understanding and embracing media awareness as a tool to put a spotlight on this disease and build momentum for change.

This year, we focused a considerable part of our press efforts on the fact that PHA, the organization representing a disease with only 20,000 to 30,000 diagnosed patients, gives an annual award to a physician carrying a prize of $50,000 for continuing clinical excellence at the physician’s PH center.

The approach worked – bigtime!

The day before we left for Conference, this year’s winner, Dr. Vallerie McLaughlin and I started off with an hour long PBS radio interview that turned out to be just a warm-up.

Last Thursday at 3:30am Pacific Time, Dr. McLaughlin and I made our way down from our rooms to a meeting space where we did 15 more radio interviews – each 10 to 15 minutes long – over the course of the next four hours. The interviews brought us into East Coast drive time and covered markets ranging from New York City to Oklahoma City, from Detroit to Colorado Springs. First reports were that we brought the PH message to 2.5 million listeners…and replays are expected to add up to 4 million more.


How can we fail when we have members of our community willing to work day and night – in this case, literally – to make a difference?

After the interviews, Val and I made our way to other meetings, including our PHA board meeting and International Leaders Summit that started soon after.