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