The first pediatric PH research fund is opening its doors…

In little more than a decade and a half, we have seen incredible growth in the field of pulmonary hypertension.  We have gone from no treatments to nine, to being a disease of little notice in the medical community to one with great and growing educational structures, from hopelessness to hope.

It is now time to bring that same progress to pediatric PH.  Opening the doors for the RobynBarst Pediatric Research and Mentoring Fund is an early and essential step.

Here is an updated version of the note I sent to PHA’s Board and staff about a week ago …

On Saturday evening September 22 at about 7:00pm Pacific, Carl Hicks announced that the Robyn Barst Pediatric PH Research and Mentoring Fund reached its $1,000,000 stage 1 goal.  The doors for the first ever pediatric research fund for PH are now opened!

While the goal was reached at the at the Thirsting for a Cure event in Oregon, it was the culmination of a two year communitywide effort. We owe a great deal of thanks to a great many people…to the Cardiovascular Medical Research and Education Fund (CMREF) for their leadership grant, to Steve White and his family for their generous pledge to get us on our way, to the Linnens, Srinivasans and MacDonalds for their special generosity, to Actelion and United Therapeutics for their high-level sponsiorships for Thirsting for a Cure, to many other donors, to Carl Hicks whose impatience to get us to our goal led to the event that brought us over the top and to Actelion CEO, Shal Jacobovitz who called Carl just before the event and committed to fill any gap to get us to the $1,000,000 target for activation of the Fund.

Most of all, we owe Dr. Robyn Barst for her work in pulmonary hypertension as a clinician, a researcher and builder of the field, especially in the once lonely area of pediatric PH.  Robyn’s generous lead gifts allowed us to establish the Fund and move it from dream to reality in little more than two years.  Her vision in including a mentoring element will help ensure the development of future pediatric PH experts.  Robyn, we are grateful.

Dr. Serpil Erzurum, chair of PHA’s Research Committee recently led a meeting to plan for anticipated implementation of Barst Fund activities.  Based on that meeting, we contacted and rapidly reached agreement with the American Thoracic Society for grant management services. We will now begin to work with them to establish program guidelines based on our existing proposal, set application and review deadlines and make our first grants.

In closing, Saturday’s event was a lot of fun with a lot of wine at the Alexana Winery in Newberg, Oregon.  Mike and Bonnie McGoon, John Hess, Betty Lou Wojciechowski (and family), Mary Jan Hicks, Bruce and  Rita Brundage, Gail and Denis Hayes, Gareth Gwyn and many others joined us, including Charity Sunshine who sang for the gathering.  The Portland Metro Support Group did great work at the registration table and beyond to make the event the success that it was.  Carl got a lot of people driving happily down a long gravel road to get to this event…and more happily back.  Well done. 

The Robyn Barst Pediatric PH Research and Mentoring Fund has gone from dream to reality.  Now it’s time to leverage that reality to growth of the pediatric PH field – for the good of the kids and their families living with this disease…today and tomorrow.

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.

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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.

Busy day in Greater Washington...

Today was supposed to be a big ice storm in Washington DC...but the temperature held above freezing because we had a lot going on outside the office.

At an 8:00am breakfast in Bethesda, Maryland I had a chance to introduce Candice Abate, our new VP for Medical Services & Patient Education to PHA Board Chair, Laura D'Anna.  Laura was in town for an NIH meeting.

After that, I headed up the road to Congressman Chris Van Hollen's office in Rockville.

He is our local Member of Congress and PHA volunteer Conchita Watson had written him a letter during November Awareness Month telling him she wanted to talk to him about PH...and received an invitation in response.  The rest of our group was her friend and fellow patient, Georgia M., PHA's Advocacy and Awareness Director, Katie Kroner, our Washington rep, Gavin Lindberg and me.  It was a great meeting with Conchita and Georgia telling our Congressman what it is like to live with PH.  He was very interested in learning about the disease and we were able to give him a lot of information.   Our big issue was the reintroduction of our bill in the House and Senate (Katie and Gavin have been working with Senator Casey and Rep. Brady and others on that).  After we spoke, he told us he had read the bill and was glad to co-sponsor and would help to move it along! We will be following with his staff on that and another issue.

While that was going on, Debbie and Dr. Steve Mathai from Johns Hopkins were across the District line to do their interview on the show I blogged about yeasterday.  I was able to listen to the Kojo Nnamdi Show...and you can, too!  Once you click the link to the show's page, drag the white time line on the black bar to 24:33 to catch the start of the segment.

They were a great team, joined by Dr. Gregory Kato from NIH.  Many thanks for helping to expand awareness of this disease. 

...And what a relief we didn't have to cancel all this for ice!

Debbie, Dr. Mathai and Kojo...

First it was PHA board member Steve White, Generation Hope leader Coleen Brunetti and Yale's Dr. Terrence Trow, now PHA's own Director of Volunteer Services, Debbie Castro and Johns Hopkins physician and PHA research award grantee, Dr. Stephen Mathai are hitting the NPR airwaves.

Here are the details about tomorrow's show...

The Pulmonary Hypertension Association will be on the Kojo Nnamdi Show, a local D.C.-area National Public Radio (NPR) program, on Wednesday, February 2 at 1 p.m. ET. If you are local you can listen on the radio at 88.5, otherwise listen online at www.WAMU.org. With 700,000 listeners, the show will help us raise awareness about the disease and PHA's work with the PH community. They are dedicating the entire show to pulmonary hypertension, and the program includes a listener call-in segment, so get your questions and comments ready!


Show Details

Kojo Nnamdi Show

Wednesday, February 2

1:00 p.m. ET (will probably begin around 1:15 p.m. ET)

Listen: WAMU, 88.5 OR www.WAMU.org

Panel: PHA staff member Debbie Castro, PH specialist Dr. Stephen Mathai from Johns Hopkins, and Dr. Gregory Kato of the National Heart Lung and Blood Institute

Includes a listener call-in segment: 800-433-8850; anyone can listen and call in

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.

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 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!

Visiting friends...

Steve White is a PHA board member. He"s also an Episcopal priest just back from a trip to Israel.  Here's part of his note on his visit with our friends from PHA Israel.  I think it gives a great view of the importance of international connections.

For more on Steve's connection to PH visit his Virtual Birthday site.


As you know, I met with some of the leaders of PHA-Israel in Tel Aviv this past Wednesday. At the dinner meeting were: Aryeh Cooperman (PHA-Israel C.E.O.), Rochi Cooperman (patient), Miriam Don (patient), Joni Berg (president of PHA-Israel).

Here is a report of that meeting from my notes.

I got to the restaurant earlier than the others. The first to arrive was Miriam Don a New Zealander and a widow who was diagnosed in the U.K. about 3 years ago. She moved permanently to Israel about a year ago. She was active in the PHA in the U.K. and while there developed a celebrity cookbook for patients titled “Miriam’s Marvels”. She was able to get celebrity chefs and some members of royalty to give her recipes without salt and that are simple to fix to make the task of cooking easier for PH patients. The book is distributed by PHA-UK and Miriam would like it to be distributed more widely. If we don’t have anything like this in the USA, we might see about reproducing it here. Of course the measurements would have to be converted from metric.

Aryeh and Rochi Cooperman arrived with Joni Berg. Aryeh was born in Ireland but grew up in Israel. Rochi is a sabra (a native-born Israeli). They have four children. Joni Berg is a native South African and a lawyer who works for the Ministry of Health in Jerusalem. His wife is a PH patient and they have 2 children.


Since Aryeh came on board as the C.E.O. on a part time basis (he is also a teacher) the organization has become more productive with materials to help patients and families. They have had several social events that have built the community and have helped raise money.


Aryeh showed me a video they produced (in Hebrew with English subtitles) and gave me copies of the DVD. It is alsoon YouTube, titled Israeli Association for PH. He also showed me a DVD on living with Flolan which is designed to make patients feel hopeful and positive. The group has also worked on excellent print media and is designing a new web site which should be up soon. I will send all the materials he gave me to Christine.




Joni Berg told me how they have been successful in getting medications and services needed by PH patients included in the so-called “basket of health services” provided to each Israeli by the government.


Some of other issues we discussed:


Recruiting new patients to the organization – I suggested getting pharma companies to allow them to place a PHA brochure in shipments of meds to patients. They had not thought of this idea and said they would try it. If we have other ideas, they would like to hear them.


There is a great need for portable oxygen concentrators that could be loaned out to patients who are housebound and need to make medical appointments or even go on holiday. The units cost about $5,000 each and Aryeh said they would like to have 2 or 3 on hand.




They need a list of medications that PH patients cannot take due to drug interactions. Do we have such a list?


Everyone expressed an interest in getting more doctors involved in PHA-Israel. Any help we can give them on how to get referrals to PHA from doctors and how to involve doctors in the organization would be helpful to them.


They would like to know the conditions under which they might be able to use content from our print media and web site for use in Israel. They are especially interested in translating the Patient’s Handbook into Hebrew. Perhaps Christine could clarify this for them. They do not get Pathlight and we might consider sending copies to our international partners.




A major challenge for them concerns how to get support groups to meet since they have so few patients spread all over the country. Until they have enough patients to establish local support groups, patients have to travel long distances at great expense and funding is needed for this.


I encouraged them to come to Conference in California. Joni was in Houston last year and attested to how helpful Conference can be for patients and caregivers. However, the long flight will require oxygen for patients who have trouble breathing.


I had a real sense that these five people regard their efforts as part of a life and death struggle that touches them directly and personally, as indeed it is (Joni’s wife is a patient, Miriam and Rochi are patients). They are incredibly dedicated and creative and I hope we can continue to support them. It seemed to mean a great deal to them that a PHA Board Member from the States took the time to be with them. It was a humbling experience for me to spend an evening with these wonderful people.

Learn more about the 50 PH Associations worldwide at PHA's International Website.