Steve Van Wormer's path of pediatric PH...6 years later

The following guest blog was written by Steve Van Wormer on the anniversary of his son's diagnosis with PH. Thank yoiu Steve, Lucas and Marina for all you do and for being models for so many in our community...

My PH Past:

Today marks the six-year anniversary of my son Lucas’ Pulmonary Hypertension diagnosis.  On October 28, 2006 he was only four years old when he underwent an array of cardiac testing and my wife Marina and I first heard the words, “Pulmonary Hypertension.”  As any PH parent, patient or caregiver can attest to, it’s a day one never forgets and a diagnosis that changes everything.  Lucas did have about a year of declining health and energy, but in hindsight, we consider ourselves lucky and blessed that his diagnosis was made earlier than most in our community.  What I remember most about that day, after shedding some tears of course, was making the decision to not curl up in a ball but to become a champion for our only child.

 

My PHA Present:

In January through May of this year, thanks to receiving a Tom Lantos Innovation in Community Service Award, I created a series of five Public Service Videos for PHA, with the hope of building PH Awareness.  These videos seem to be resonating not only within our community, but in the media as well.  They have generated news stories on TV & in print and have become a fixture on YouTube and facebook and at PHA events.  They’ve even played on flat screen TVs inside port-o-potties at PHA’s Woodland Crawfish Festival fundraiser… true story!

 

One of those spots, which can be found here, is a call to action to support the Robyn Barst Pediatric Research Fund, voiced by none other than Lucas himself.  As a parent, I couldn’t be prouder of his contribution to PHA.  As a member of PHA’s Board of Trustees, I couldn’t be more thankful to former board chair, Carl Hicks, who was instrumental in taking the Barst fund over its $1,000,000 goal at a recent fundraiser, triggering PHA’s first ever pediatric specific research initiative.   

 

Earlier this month, to coincide with PH Awareness Month, my creative partners and I were asked to translate some of these PSA spots into Chinese and Spanish to reach patients and medical professionals across the globe in China, Spain, Mexico and South America.  I’m so thankful that, six years after Lucas’ diagnosis, PH Awareness continues to spread across the world.

 

My PHA Future:

I’m very happy to announce another PH Project that launched a few days ago on Thanksgiving: a PHA Podcast, dedicated to raising PH Awareness.   “Empowered by Hope – The PHA Podcast” will bring dynamic stories and journeys from PH patients, caregivers and medical professionals in a series of impactful, insightful and, most importantly, hopeful conversations with members of the Pulmonary Hypertension Community.  Three episodes were released last week.  They are a series of conversations with our “Kitchen Table” Co-Founders Pat & Jerry Paton, Judy and Ed Simpson, and Dorothy Olson.  I’m sure you will enjoy listening and learning all about how our organization was formed.  A facebook page was also created (search: Empowered by Hope: The Pulmonary Hypertension Association Podcast).  I hope you can take a moment to “like” it and join in on the conversation.  I’m spending today’s six-year anniversary of Lucas’ PH diagnosis at UCLA with Lucas’ PH doctor (Dr. Juan Alejos), recording a new episode, with many more to follow.

 

In one of the podcasts, which you can subscribe to and download here, PHA Co-Founder, Judy Simpson told me what it means to be a PH Caregiver.  She said, “You do things you never thought you could do.”   Truer words have never been spoken. 

 

So many things have happened on our PHA journey over the past six years… so many things I never thought I could do. 

 

Did I ever think I would travel to Washington D.C. to visit Congressional and Senate offices to solicit support for our PH Healthcare Bill?  No.

 

Did I ever believe I would serve on a planning committee for an international Conference for 1500 patients, caregivers and medical professionals?  Certainly not.

 

Did I ever suppose I would create videos and podcasts that would be shown across the globe from Burbank to Barcelona to Beijing?   Never.

 

But I did.  And I do.  And I will continue.  In honor of my son, Lucas.  On behalf of pediatric patients like Iain, Katie Grace, Parker and Maddie, who need more research and treatment options sooner than later.  In memory of pediatric patients like Rayni, Taylor, Meaghan and Molly - who may have lost their PH battle, but will never be forgotten.

 

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.