Pathlight - Autum 2024

A publication of the Pulmonary Hypertension Association

2024 // Volume 34 // No. 3

WHAT TO KNOW ABOUT TRANSPLANTATION PLUS

HOW ADVOCACY HELPS PATIENTS AND FAMILIES

VOLUNTEER WELCOMES PHA 2024 ATTENDEES

-8WAYS

TO PARTICIPATE IN RESEARCH AT PHA 2024

TRANSPLANT RECIPIENT

Celebrates Post-PH Life

IN THIS ISSUE 2024 // VOLUME 34 // NO. 3

14

Life With PAH Can Be Challenging.

Learning About PAH Shouldn’t Be.

6

6

Get Social at PHA 2024

8

Volunteer Welcomes PHA 2024 Attendees

10 8 Ways You Can Participate in PH Research at PHA 2024

Join a free, educational webinar in the comfort and safety of your home

13 Interact With Researchers at Poster Hall and Lightning Rounds

The Navigating PAH Pathways program offers free webinars created specifically for patients and caregivers, all led by a PAH Practitioner and sponsored by Janssen.*

14 Community Workshop Inspires Education, Hope and Support 18 Headed to PHA 2024 on a New High 26 What to Know About Transplantation 30 How Advocacy Helps Patients and Families

Find Answers

Learn Why

Get Talking

Hear an educational presentation from a PAH Practitioner on topics that may be important to you.

Understand more about PAH, the 3 key treatment pathways, and tools to help you track your PAH

Discover how to have more informed conversations about PAH with your care community.

31 Advocating for PH-Related Legislative Reforms

ON OUR COVER

Webinars are available at various dates and times so you can register for one that best fits your schedule.

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Register today at PAHregister.com or call 1-888-370-8111 Your PAH healthcare team should always be your main source of information. This program is not intended to provide medical advice or address specific medical concerns.

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PHA board member Diane Ramirez is thriving with new lungs and a heart. After 36 years with pulmonary hypertension, she’s getting used to post-transplant life. Cover story photos by Hinson Photography.

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All webinars are viewed on a computer; internet access required.

PAH=pulmonary arterial hypertension. * The speaker is a paid consultant presenting on behalf of Janssen Pharmaceuticals, Inc., and must present information in compliance with FDA requirements applicable to Janssen Pharmaceuticals, Inc.

IN EVERY ISSUE 5

Letter From the Chair

32 Quick Takes 34 Calendar 35 Passages This program is sponsored by Janssen Pharmaceuticals, Inc. ©2022 Actelion Pharmaceuticals US, Inc. All rights reserved. cp-201358v2 02/22

PHASSOCIATION.ORG 3

LETTER from the

PUBLISHER

BOARD OF TRUSTEES

Matt J. Granato (he/him) President & CEO

Officers Tony Lahnston, chair Traci Stewart, RSN, CSN, CHFN, chair elect Nicole Creech, treasurer Colleen Connor, secretary Colleen Brunetti, MEd, CHC, immediate past chair Matt J. Granato, LLM, MBA, president and CEO, ex officio

STAFF Linda Busche (she/her) Editor-in-Chief Karen Smaalders (she/her) Vice President, Communications and Marketing

CONTRIBUTORS

Trustees

Grace Beal, Mia Chiarelli, Jenny Errico, Angela Golden, Haley Johnson, Daniel Layton, Renee Mestayer, Kassandra Olgers, Melanie Padgett Powers, Diane Ramirez, Michelle Reller, Marina Salazar, Abby Sickles, Sarah Smith, Jaeger Spratt. Cover story images: Hinson Photography.

Todd Bull, MD, Scientific Leadership Council chair elect Murali Chakinala, MD, FCCP, Scientific Leadership Council immediate past chair Ramona Doyle, MD, MSc Anna R. Hemnes, MD, Scientific Leadership Council chair Kimberly Jackson, RN, BSN, PH Professional Network chair Mitch Koppelman, PhD Michelle Ferdinand Liu, MD, MPH, FAAOA Mike Lentz Melissa Magness, MSN, APRN, CNP-AC, PH Professional Network chair elect Mike Naple Monica M. Penaranda Diane Ramirez Doug Taylor Matt Wall Delphine Yung, MD, Accreditation and Registry Committee chair

Mary Beth Ramsey (she/her) Graphic Design

CONTACT 301-565-3004 PHAssociation.org

SUPPORT LINE 800-748-7274 We’re here to support anyone with a connection to PH. (Daytime, please.)

Submissions to Pathlight are welcome.

Please email your articles, photos and queries to Communications@ PHAssociation.org.

HEADQUARTERS 1121 14th St. NW, Suite 150 Washington, D.C., 20005

Emeriti ex officio C. Gregory Elliott, MD, FCCP, MACP Michael D. McGoon, MD Edwin Simpson Judith Simpson, RN, EdS Honorary Carl Hicks PH PROFESSIONAL NETWORK EXECUTIVE COMMITTEE Kimberly Jackson, RN, BSN, chair Cheri Abbott, RN, CCRP April Blakley, RN, BSN Jessie Dunne, PharmD, BCPS, BCCP Loida A. Johnson, CRNP Tisha Kivett, RN, BSN Melissa Magness, MSN, APRN, CNP-AC, chair-elect

SCIENTIFIC LEADERSHIP COUNCIL Anna R. Hemnes, MD, chair Todd M. Bull, MD, chair-elect Murali M. Chakinala, MD, FCCP, immediate past chair Eric D. Austin, MD, MSCI Sonja Bartolome, MD Vinicio A. de Jesus Perez, MD, FCCP Teresa De Marco, MD Ankit A. Desai, MD, FACC, FAHA Jeffrey Fineman, MD Robert P. Frantz, MD Mardi Gomberg-Maitland, MD, MSc Daniel Grinnan, MD Gustavo A. Heresi, MD, MSci Kristin B. Highland, MD, MSCR Nicholas Kolaitis, MD, MAS Matthew R. Lammi, MD, MSCR Peter Leary M.D., PhD Deborah J. Levine, MD Wes McConnell, MD Lana D. Melendres-Groves, MD Josanna Rodriguez-Lopez, MD John J. Ryan, MD, MB, BCh, BAO Sandeep Sahay, MD Thenappan Thenappan, MD Anjali Vaidya, MD, FACC, FASE, FACP Nidhy Varghese, MD Corey E. Ventetuolo, MD, MS R. James White, MD, PhD Distinguished Advisers David B. Badesch, MD Erika S. Berman Rosenzweig, MD Bruce H. Brundage, MD Richard Channick, MD C. Gregory Elliott, MD, FCCP, MACP Karen A. Fagan, MD Michael D. McGoon, MD Vallerie V. McLaughlin, MD Ron Oudiz, MD

CHAIR Dear PHA Community, For those of you coming to PHA 2024 International PH Conference and Scientific Sessions, welcome to Indianapolis.

For those of you who can’t make it, you are with us in spirit.

We will share messages of hope and encouragement from the conference, as well as information about advancements in PH treatment, diagnosis and understanding.

This is the 30th anniversary of PHA conferences. Our first, in 1994 outside of Atlanta, attracted 200 people from the United States, Canada, the United Kingdom and Australia. The attendees included 68 people with PH and 32 health care professionals. The rest were family members, friends and caregivers.

In many ways, some things haven’t changed. Many people with PH had never met anyone else with the same condition.

Just like today’s attendees, they were looking for support, connections and answers about their disease. Some of them participated in our first Research Room, to help scientists better understand their disease and develop treatments.

The first conference gave health care professionals an opportunity to meet people with PH. It also allowed professionals to share information. These aspects of PHA conferences haven’t changed at all.

In other ways, PHA conferences have changed significantly. On the face, PHA 2024 is much larger. This year we expect people from 50 countries. In addition to attending education sessions, attendees can interact with peers at several social activities and meet with more than 20 companies in our exhibit hall.

On another level, the event reflects meaningful changes.

PHA is now 34 years old with more than 90 accredited PH Care Centers throughout the country. We have more than 200 support groups. We offer a website, newsletters and scores of publications to deepen the community’s understanding of PH and promote connections within the community.

Our scientific understanding has matured as well. In 1994, there were no treatments for PH. As we look forward to PHA 2024, there are 16 FDA-approved treatments

for pulmonary arterial hypertension and many others in development. Today’s attendees overwhelmingly were diagnosed at an earlier stage in their disease progression than the 1994 attendees. There is more widespread, authoritative information about PH; improved treatment and care; and widespread opportunities to connect with others.

As I reflect on the changes since that first PHA conference, I look back on my past two years as chair of PHA’s Board of Trustees. PHA has been an important part of my life and my personal mission for 16 years since my niece, Marchelle, passed away in September 2007.

I wanted to keep Marchelle’s memory alive through my efforts and contributions to PHA, its patients and caregivers, its medical community and its board of trustees. Over time, I realized that I had an equally important goal. Knowing that Marchelle was a wonderful and giving person, I think she would be proud see everything I have done as a board member for all the wonderful people in this community.

PHA CEO Matt Granato and his team have accomplished a lot during my time as the chair. They understand the priorities of PHA and remain hyper-focused on the mission and continuous improvement. I truly enjoyed collaborating with them. I have thoroughly enjoyed providing the team with the tools they need to succeed and providing guidance and support for Matt and the rest of this amazing organization.

I hope that in some small way, I have been able to improve the lives of people affected by pulmonary hypertension.

Thank you for allowing me to serve. I can feel Marchelle smiling down on me.

All my best,

Tony Lahnston Chair, Pulmonary Hypertension Association Board of Trustees

PHASSOCIATION.ORG 5

PHA 2024

PHA 2024

Get Social AT PHA 2024 Connect with old confidantes and meet new friends at PHA 2024 International PH Conference and Scientific Sessions. The Aug. 15-18 conference in Indianapolis features several longstanding social activities and new events.

A

fter attending educational sessions during the day, join us for an evening of fun.

Here’s how you can turn your experience into a memorable vacation with family and friends from the pulmonary hypertension community.

EXHIBIT HALL OPENING RECEPTION OUTSTANDING MEMBER AWARDS

Celebrate heroes who don’t necessarily wear capes. Don’t miss the Outstanding Member Awards dinner and ceremony Aug. 17 at 5 p.m. EDT. The awards recognize extraordinary commitment to the PHA community through caregiving, volunteerism, advocacy, awareness, fundraising, promoting quality patient care and advancing PH research. Enjoy dinner while cheering those who go above and beyond to make a difference in the PHA community. Afterward, join us for the Saturday Night Party and Fashion Show.

SATURDAY NIGHT PARTY AND FASHION SHOW

What better way to celebrate all the friendships, connections and triumphs of the weekend, than a one-of-akind party? Join the fun 6:30 p.m.

EDT Aug. 17 and cheer on fashion 6 PATHLIGHT

show participants. Discover ways to transform oxygen cannulas, mobility devices and medication pumps into symbols of strength and hope. The Saturday Night Party will feature a DJ, dancing, games and a ton of fun.

This exciting, vibrant party is not to be missed.

Join PHA staff, sponsors and exhibitors for the exhibit hall opening reception Aug. 15 at 6:30 p.m. EDT. This is your chance to get a head start on networking with peers and industry leaders. Stop by the exhibit booths to learn about new PH updates and innovations. All registered attendees are encouraged to attend, and light refreshments will be served.

INTERNATIONAL FORUM AND RECEPTION

Non-U.S. PH associations’ leaders will convene for a panel discussion on global patient challenges and how those organizations support patients and caregivers to address the challengers. After the panel discussion, join PHA staff for a reception to build connections with PH associations around the world. The event begins at 7:30 p.m. EDT Aug. 15.

CONFERENCE OPENING LUNCH

Kick off an exciting weekend of learning and connecting with a boxed lunch, followed by the opening session.

The patient and caregiver lunch begins at noon in the same room as the keynote address. The health care professionals lunch is from 11:45 to 12:45. The keynote, featuring Mandy B. Anderson, will begin at 1 p.m. Anderson, who has cystic fibrosis, is a certified executive coach, author and co-founder of Rayma Team. She will address: • How to process disappointment.

• How to persevere when hope seems lost.

• Strategies for becoming the person who can handle what you’re hoping for.

JOURNEYS LUNCH

A tradition since PHA’s first conference in 1994, the Journeys Lunch highlights the experiences patients and their care teams share. Join us at noon Aug. 17 to hear personal and medical journeys from three patient-health care professional pairs. The pairs will discuss how together they determined

Journeys Lunch PHA 2022

the most effective treatment plans, built partnerships and planned for patients’ futures.

EARLY CAREER RECEPTION

New to PHA 2024 is a reception for early career professionals on Aug. 15 at 8 p.m. EDT. This reception in the lobby bar is an informal opportunity for clinicians and researchers who are new to PHA to network and meet new colleagues in the PH community. This casual, fun reception is open to PH professionals with no more than five years’ experience.

LGBTQ+ MIXER

Celebrate the spirit of diversity at this inclusive event. All attendees are welcome, regardless of whether you identify as LGBTQ+.

Mix and mingle in the JW Marriott bar over drinks, light fare and great conversation. Join us 9-11 p.m. Friday, Aug. 16, to show that PHA values and respects all members of the PH community.

COMMUNITY SERVICE PROJECT

As PHA 2024 comes to a close, come together with others who have PH, caregivers, health care professionals

and children to give back to this year’s host city. Work side-by-side to stuff bears for pediatric patients at Riley Children’s Health. Participants will design custom t-shirts for the stuffed animals and write cards with heartfelt messages. After building the stuffed animals, participants can move to a wrapping station to add cellophane and bows. The event begins at 8 a.m.

EDT Sunday, Aug.18.

CLOSING BREAKFAST

David Ranalli will close PHA 2024 on Sunday, Aug. 18 at 9:45 a.m. EDT, bringing his magic show, “Deception,” to Indianapolis. Ranalli is a two-time TEDx speaker and has performed at high-end conferences around the world. His creative magic displays a variety of jaw-dropping illusions and secret techniques.

Don’t miss these opportunities to make lasting connections.

See the agenda at PHAssociation.org/PHA 2024.

PHASSOCIATION.ORG 7

PHA 2024

PHA 2024

Volunteer Welcomes PHA 2024 Attendees

I MICHELLE RELLER, 54, is a member of the Welcome Subcommittee for PHA 2024 International PH Conference and Scientific Sessions. A longtime Pulmonary Hypertension Association support group leader, Reller currently leads the Kentuckiana PH Warriors group. Diagnosed with pulmonary hypertension in 2009, Reller has dealt with many illnesses since she was 10, when she had a pituitary brain tumor and her pituitary gland removed.

Reller lives in Huntingburg, Indiana.

BY MICHELLE RELLER

have attended most PHA conferences since being diagnosed, including PHA 2012 in Orlando, Florida, PHA 2014 in Indianapolis, PHA 2016 in Dallas and PHA 2018 in Orlando. I recently had the opportunity to serve on the Welcome Subcommittee for PHA 2024, which is Aug. 15-18, in my home state.

I am honored to help make this a great conference for new patients, as well as those returning. I am also honored to remember those who have lost their battle with PH through the Wall of Hope.

My favorite part about PHA conferences are the people.

When you meet so many others with the same problem and see what they have to face on a daily basis, it puts life in place and makes me want to PHight even harder. Reuniting with people you see only at PHA conferences and the friendships are priceless.

As a member of the Welcome Subcommittee, I helped create the Wall of Hope and Remembrance, recruit local volunteers, plan the Fashion Show and pitch in on other tasks that create a positive patient experience. In Indianapolis, the Welcome Subcommittee will be available at the Wall of Hope and Remembrance.

Being on the subcommittee is just one way I give back to the PH community. I became a PHA support group leader so I could talk with others who have the same issues I was having. My family understands, but I have been through so much that it’s nice to have a support network of people in the same situation.

Welcome 8 PATHLIGHT

SHARED EXPERIENCE

What motivated me the most to become a support group leader was that I lived in a rural community and no one knew what pulmonary hypertension was. I wanted someone else to relate to and talk to about what I was going through.

My first meeting was a flop. It was in Jasper, Indiana, and no one showed up, other than my mom, the doctor, the pharmaceutical representative and me. I was very discouraged and wanted to quit. The doctor and pharma rep urged me to stick with it. I am glad I did.

The doctor suggested that I hold the meeting in a meeting room in his office building. I did have a fair turnout there, including a family with hereditable PH. Some people came for a meeting or two and then didn’t come back.

Later, I formed a PHA support group in Evansville with the family from the previous group and a woman with PH and her husband. After a while, PHA asked me to help form a Louisville group because the leader had passed away.

I have since dissolved the Evansville group because the woman with PH passed away and the family sometimes travels to the Louisville group. I now lead only one group: the Kentuckiana PH Warriors. We usually have 10-12 in attendance, including friends and caregivers.

Since I have become a leader, I have had four or five members pass away, which is something we do not like to hear. We are glad to help all we can.

DIAGNOSIS AND DISCOVERY

My own PH journey began about 15 years ago. I had been feeling short of breath for a while and couldn’t walk up three steps without stopping and clutching my chest because it hurt so badly. I would catch my breath and go on, but I ended up going to a cardiologist. The cardiologist said that I had some fluid around the heart and with time it would be OK.

Find Support From a PHA Peer Mentor

The pain and shortness of breath did not stop, so I returned to the doctor’s office. The cardiologist was no longer there, so his replacement saw me. He happened to be from Vanderbilt University Medical Center in Nashville, a PHAaccredited PH Care Center.

He did not do any testing. After looking at previous records, he said that I needed to see a specialist and fast. He referred me to a doctor at Ascension St. Vincent in Evansville, Indiana.

That doctor did a right heart catheterization and told me to come straight to his office after the test. He said I had pulmonary hypertension and I had two options: I could take the medications and hope that they worked, or he would give me two years to live.

Wow, what a blow. I of course opted for the medications and am glad I did. I am now a 15-year survivor and a PHA support group leader.

I am most proud that I stuck with this journey and that I can help others as well as myself by volunteering. My inspiration is Roger Johnson, the doctor who diagnosed me.

He put me in contact with PHA and has supported me along the way.

Anyone wishing to start a PHA support group, do not quit. You would be giving up on yourself as well as those who need you to keep them informed and encouraged.

I look forward to seeing many of you in Indianapolis and hearing your stories. I hope everyone who comes to PHA 2024 leaves with a positive outlook, a renewed spirit and contacts for help from PHA. Don’t give up. We all are PHighting the same battle.

Learn more about activities, exhibitors and education sessions at PHAssociation.org/ PHA2024.

T

he Pulmonary Hypertension Association has revamped its peer support program. Formerly known as PHA PHriends, our new PHA Peer Mentor Program connects patients with patients and caregivers with caregivers.

Our volunteer peer mentors understand what you’re going through and are trained to listen to your challenges and concerns, provide practical information, and share experiences and resources. Learn more at PHAssociation.org/phmentors.

PHASSOCIATION.ORG 9

PHA 2024

Research Room

8 Ways You Can Participate in PH Research at PHA 2024

10 PATHLIGHT

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s PHA 2024 International PH Conference and Scientific Sessions approaches, consider participating in one of eight Research Room studies. Patient and family participation help pulmonary hypertension researchers further their studies to advance understanding and treatment of PH. Natural History Study of Novel Biomarkers in Pulmonary Arterial Hypertension

1

Researchers from the National Institutes of Health are recruiting participants for a study on how the PH disease process changes over time. The researchers hope the results will lead to earlier diagnosis and uncover new treatment pathways. Participants must be at least 18 years old, have Group 1 PH and be able to provide informed written consent. Learn more onsite about the study and enrollment eligibility.

Pilot Study of the Effect of Spironolactone Therapy on Exercise Capacity and Endothelial Dysfunction in PAH

2

National Institute of Health researchers seek participants for a study on an investigational drug. They are studying whether the drug improves disease symptoms. Participants

can learn more about the study in the Research Room and fill out questionnaires if they are interested in enrolling.

Eligibility: Participants must have Group 1 PH, be at least 18, and be able to provide informed written consent.

Participants must not be pregnant.

People with cirrhosis and portal hypertension and people with active infection can’t participate.

3

Characterizing Roles and Perspectives of Caregivers in PH

Hilary DuBrock, pulmonologist and researcher at Mayo Clinic in Rochester, Minnesota, is leading a study to improve understanding of caregiving in PH. Caregivers assist with medicine and transportation and provide emotional or other forms of support to people who need help taking care of themselves.

The researchers will interview people with PH about their perspectives of caregiving. They hope to identify ways to best support caregivers.

Participants must be at least 18, have a self-reported PH diagnosis and have at least one caregiver over 18.

4

Sexual Health Assessment in Patients with PH

By incorporating discussions about sexual health into PAH care management, health care providers can address patients’ needs beyond their medical conditions. This study,

conducted by clinical research coordinator Meredith Kaplan and pulmonologist Ioana Preston, uses validated surveys to start these conversations between providers, patients and their partners. Participants must be at least 18, have a historical PH diagnosis, know their PH group/ subgroup and approximate diagnosis date, and be able to provide written informed consent.

5

MObile Health InterVEntion in PAH (MOVE-PAH)

Evan Brittain, cardiologist and professor of medicine at Vanderbilt University, is leading a clinical study to evaluate how increasing daily step counts can affect quality of life and six-minute walk distance for people with PAH. Eligible participants will

receive new Fitbit devices, which they must wear for about 28 weeks.

Participants also must complete a series of questionnaires and perform three remote six-minute walk tests.

Qualifications: Participants must be at least 18. They must have idiopathic, heritable or PAH associated with simple congenital heart disease, functional class I-III. Forced vital capacity must be greater than 65% with no or minimal interstitial lung disease.

6

ACTIVITY MONITORING IN PH

Brittain also is leading a study to understand if daily activity level (measured in steps) can show who is more or less likely to get sick over time. Researchers will explain the study on site and assess eligibility.

Those eligible to participate will

receive a Fitbit to track movement and heart rate.

The study will last three years.

Participants must wear the device for four 12-week periods. There will be a baseline monitoring period, followed by monitoring periods once a year for three years. There will be no in-person study visits.

Participants must be at least 18 years with a formal clinical PH diagnosis.

RISK AND RESILIENCE IN PAH AND GENETICALLY SUSCEPTIBLE INDIVIDUALS (RARE-PAH)

7

Physicians Anna Hemnes, Eric Austin and Brittain, and registered nurse Kelly Burke, of Vanderbilt University, are studying why some people with the BMPR2 gene develop PAH and PHASSOCIATION.ORG 11

PHA 2024

Interact With Researchers at Poster Hall and Lightning Rounds

D Patient and family participation help pulmonary hypertension researchers further their studies to advance understanding and treatment of PH.

others don’t. The researchers want to know how those genes influence development of PAH and resilience against developing PAH. They will examine gene changes, hormonal and blood abnormalities, and how they work together. Ideally, the study would improve understanding of the disease process and could lead to improved therapy.

The study will include three remote visits over three years.

The study is open to people with heritable PAH, functional class I-III. They must be taking a stable PAHspecific medication for three months before enrollment. Family members can participate if they are unaffected mutation carriers or are healthy with a known or unknown heritable gene mutation, normal pulmonary pressure and RV function. Participants must be at least 13 and no older than 80.

Eligible participants will undergo blood draws with the on-site phlebotomy team in the Research Room.

12 PATHLIGHT

8

GENOMICS OF PULMONARY VASCULAR DISEASE

Many people with PH have changes in their mitochondria, the “batteries” that power cells. Micheala Aldred, director of pulmonary research at Indiana University, is leading a study to understand how those changes occur. The researchers have preliminary evidence that variation in DNA (genetic material) might contribute to those changes.

The researchers plan to collect blood samples to measure the function of the mitochondria and try to identify genetic variants that could increase the risk of developing PH. Eligible participants will undergo a blood draw with the on-site phlebotomy team in the Research Room.

The study is open to people with all types of PH (Groups 1-5) who are at least 18. Caregivers and others in support roles can participate as control subjects.

PHA has supported PH research since its first Research Room in 1994. The Research Room helps researchers collect data and blood samples to further their studies.

Thanks to people with PH and their families who participated in early Research Room studies, scientists identified the gene that causes heritable PAH. To sign up for Research Room studies, visit PHAssociation. org/PHA2024. Questions about participating? Email us at PHCC@PHAssociation.org.

iscover what PH clinicians and researchers are studying at PHA 2024 International PH Conference and Scientific Sessions, Aug. 15-18, in Indianapolis.

The Poster Hall opens at 4 p.m. Thursday, Aug. 15, with an interactive session by poster authors. Attendees can ask questions about authors’ research and clinical projects. Afterward, authors of the six highest-scoring abstracts will present their work in Lightning Round presentations. The Lightning Rounds will include time for Q&A. This year’s Lightning Round presentations are: • “In vivo modeling of arteriovenous malformations and HHTmimicry due to prospective PAH therapies,” presented by representatives of the Massachusetts General Hospital and Harvard Medical School.

• “Right heart catheterization practice patterns in pulmonary hypertension diagnosis in the United States: A cross-sectional analysis,” by Francisco Soto, MD, MS, University of Tennessee Medical Center.

• “Elevated right ventricular systolic pressure is an independent predictor of mortality in pediatric pulmonary vein stenosis,” by Daiji Takajo, MD, MPH, Cincinnati Children’s Hospital Medical Center.

• “PAH one-year survival prediction using integrative non-invasive lab and MRI variables,” by Max Cartwright, BA, The Ohio State University.

• “From breaths to beats: Pioneering PAH monitoring with end-tidal CO₂,” by Navneet Kaur, MD, University of Southern California Keck School of Medicine.

• “TRPV4 contributes to angiotensin II-induced right ventricular hypercontractility in isolated hearts of type 3 pulmonary hypertensive mice,” by Andrew Behrmann, BS, from the University of Missouri School of Medicine.

Health care professionals can earn continuing education credit for attending the unopposed research Poster Hall session and Lightning Round presentations. The Poster Hall will be open to all attendees on Friday and Saturday.

Learn more about the Scientific Sessions, Poster Hall and Lightning Rounds at PHAssociation.org/PHA2024.

PHASSOCIATION.ORG 13

HEALTH MATTERS

HEALTH MATTERS

Community Workshop Inspires Education, Hope and Support

C

laire Champion wanted to better help her patients.

Over a year ago, Champion transitioned from being an ICU nurse to a nurse practitioner at Texas Children’s Hospital. To help build community, better understand her patients’ needs, and network with other professionals; she attended the PHA Connects: PH Community workshop.

“I think the first thing any parent does when they find out their child has pulmonary hypertension is start googling and freaking themselves out, so the nice thing about the workshop and the PHA website is the information is so credible,” Champion

Photos: Clockwise from top left: Workshop session; workshop co-chairs Sandeep Sahay, MD, and Nidhy Varghese, MD; presenter Claire Champion, MSN; and attendees.

Opposite page: Barron “Coach Bear” Honea.

14 PATHLIGHT

said. “It provides knowledge that brings comfort rather than fear.”

Champion was among the 90 people who attended the April 6 workshop, which brought together patients, caregivers and health care professionals at the Westin Houston Medical Center. The day was filled with information where attendees learned the latest advancements in PH research and disease management from regional experts.

Champion is also the new leader for the pediatric support group at Texas Children’s Hospital, so the workshop allowed her to connect one on one with the parents and children she serves.

“Hearing their specific needs made me realize what we can improve on,” Champion said. “It sounded like palliative care is a big thing that we need to have in our clinic and just making sure patients are getting the actual benefits from their team members.”

Champion said the workshop provided great insight and educational resources for attendees, especially for families with teenagers who learned about adult medicine options and steps to make the transition between pediatric and adult care as seamless as possible.

“Hearing from adult patients who love their providers brought a lot of comfort for the pediatric families that have been with us a long time,” Champion said. “They got to see and hear firsthand what adult care looks

like and make connections with other providers and patients.”

The workshop also offered support group conversations for caregivers, patients and parents of children with PH to share experiences in a safe space. Free breakfast and lunch were included and allowed opportunities for connection and networking.

INSPIRATION

“If you look around this room today, you will realize that you are sitting amongst angels,” said keynote speaker

Barron “Coach Bear” Honea, who was diagnosed with PH in 2020 and was the face of PHA’s fall campaign in 2023.

Coach Bear reflected on the patients, caregivers and health care professionals who share hope for new treatments and eventually a cure for PH. Honea moved many attendees to tears as he expressed gratitude for his wife and caregiver Darla as well as his health care team at Houston Methodist Hospital. Honea credits the team — who attended the session — with saving his life.

PHASSOCIATION.ORG 15

HEALTH MATTERS

Top left: Corey Cartham, MD; Nidhy Varghese, MD; Rozie Fombin, RN; Claire Champion, MSN; Raysa Morales Demori, MD; Elise Whalen, MSN; Michael McDowell Jr., DO; Fadel E. Ruiz, MD. Top right: Ryan Torres, Sophia Esteves, Debra Hines-Bruce, Gary Bruce, Shirley Craig, Claire Champion, MSN; and PHA's Abby Sickles.

Below: Nidhy Varghese (center) with a pediatric patient.

16 PATHLIGHT

FUNDRAISING

The following day offered a physically and emotionally moving experience when the PH community met in Helix Park for the second Houston O₂breathe Walk. The event raised $22,525, surpassing the organizers’ fundraising goals.

Sawanda Cornett attended the walk with her family and twin sister.

Cornett has been involved with PHA and the PH community since her diagnosis 20 years ago by fundraising, participating in clinical trials and attending events. At the walk, Cornett donated hats she had made with the PH purple ribbon to raise awareness.

Cornett was one of several participants who shared their story to spread hope to others.

“My twin doesn’t have PH and I do, and people asked why God picked on me,” Cornett said. “And I say, God didn’t pick on me, he picked me. He picked me to be this force of light and gave me a platform to speak to others on this journey.”

Brandon Cuellar, who attended the walk with his wife and two

children, also shared his 20-month-old daughter’s diagnosis story.

“Our miracle daughter, Hayden, had a routine ear-tube surgery at 6 months old,” Cuellar said. “After though, she had difficulty waking up from the anesthesia because of low blood oxygen. We rushed her to Texas Children’s Hospital where she was diagnosed with congenital heart disease and pulmonary hypertension.

We know this is something we’ll be fighting for a long time. There have been excruciating moments, tough moments, but also a lot of rewarding moments, so I just invite everyone to stay positive, keep the faith and keep fighting.”

The next dual PHA Connects: PH Community Workshop and O₂breathe Walk will be Oct. 26-27 in Phoenix.

Don’t miss the opportunity to advance your knowledge and connect with members of the local PH community. Register at PHAssociation.org/workshop.

PHASSOCIATION.ORG 17

COVER STORY

Transplant Recipient Celebrates Post-PH Life

W

hen Diane Ramirez went to PHA 2022, she worried it might be her last Pulmonary Hypertension Association conference.

Her heart was failing, her oxygen needs were increasing, and she was taking the maximum doses of her PH medications.

“I had days where I wasn’t doing anything, wearing 10-12 liters of oxygen, and I was still short of breath,” says Ramirez, 61, of Lexington, North Carolina. “I couldn’t take a shower without oxygen, and I had to sit to dry myself off and catch my breath. It was very scary.”

Six months later, Ramirez underwent heart and lung transplant. As she recovers, she’s planning her trip to Indianapolis for PHA 2024 International PH Conference and Scientific Sessions. She’ll be oxygen-free, and significantly, PHfree, after 36 years.

“I think about like some of this stuff I went through as a PH patient, and I’m shocked that I was able to survive as long as I did,” says Ramirez, a member of the Pulmonary Hypertension Association Board of Trustees. “There were some really rough times. And I then I think about how good I feel now.”

Headed to PHA 2024 on a

New High 18 PATHLIGHT

‘I think about like some of this stuff I went through as a PH patient, and I’m shocked that I was able to survive as long as I did.’

THE MESS PH CREATED

In spring 2022, Ramirez felt sicker and weaker than she’d been since her 1987 diagnosis.

Between March and June, she had three cardiac catheterizations. The surgeon “saw the mess that the PH had created,” and said something like, “Enjoy life and do everything you can,” Ramirez recalls. “It was his way of saying, ‘The end is near.’

I knew at that point that something drastic had to be done.”

Ramirez, who’s attended six PHA conferences, went to Atlanta with a portable oxygen concentrator. She hadn’t seen many of her PHA friends since 2018, before the pandemic. Everyone wanted to know what was going on with her health.

After she came home from PHA 2022, her doctor, Terry Fortin at Duke University Medical Center, advised her to consider a referral for lung transplantation.

PHASSOCIATION.ORG 19

COVER STORY

‘We helped each other through a very unusual time. You’re waiting for this massive surgery so you can continue living a different life ... We were all kind of just living, working out and waiting.’

THE HEART HAS TO GO

After two days of testing and consulting with the transplant committee, transplant doctor Jamie Todd at Duke confirmed Ramirez needed a new heart as well as lungs.

Wear and tear from 36 years of PH had enlarged the pulmonary artery connecting the heart to the lungs too much to be repaired.

“My artery was so big that it had stretched the connection,” Ramirez says.

“So the whole thing (heart) had to go.”

The realization shook her. “It went from one thing in my mind to something so much bigger,” Ramirez says. “I had 1,000 questions and couldn’t formulate one sentence because I was overwhelmed, confused and a little angry at the same time.”

Initially, Ramirez wasn’t sure she wanted to go through with the evaluation. But somewhere in the “seven days of non-stop testing,” she realized she was no longer saying, “If I do this.” She was having conversations like, “When this happens,” What do we have to do?” and “Where do we go?”

The evaluation was intense: meetings with doctors and a social worker, X-rays, blood work, a psychiatric review and more.

The social worker explained that Duke transplant patients must live within 30 minutes of the hospital the entire time they’re in rehab, which had to be at Duke. Ramirez received fundraising recommendations to help pay for temporary housing and other costs not covered by insurance and a list of companies offering short-term medical rentals. Insurance covered 100% of the $1.5 million-plus cost of the transplant surgery and hospital stay. But it didn’t cover copays for rehab and many transplant clinic visits.

After the evaluation, Ramirez signed a contract and committed to following strict protocols to get listed for transplantation.

“There’s so much that you have to be prepared to tackle. Immediately.

Because time is of the essence.”

‘UNUSUAL TIME’

Back in Lexington, Ramirez and her husband Dale discussed living arrangements and financial concerns.

Dale talked to his employer so he could work from the company’s Durham office.

Within a month, the couple moved two miles from Duke University Hospital to a fully furnished apartment

with utilities, internet and cable. After moving in, Ramirez started cardiopulmonary rehab classes for transplant patients. Her exercise class met two hours a day, five days a week. The participants became close.

“We helped each other through a very unusual time. You’re waiting for this massive surgery so you can continue living a different life, which you don’t know what it’s going to be.

We were all kind of just living, working out and waiting.

She and her caregivers took Zoom classes that covered “everything that you should prepare yourself for” and “everything you don’t want to hear.”

The classes addressed medicine, food, exercise, recovery, differences between ICU and step-down, discharge, followup care and complications.

“It’s scary stuff,” Ramirez says.

“When you have a heart and lung transplant, there are complications related to the heart part that are completely separate from lung. There’s so much that can go wrong.”

Ultimately, Diane and Dale stayed in Durham 11 months: four and a half months on the waiting list, and six and a half months for rehab, surgery and recovery.

PHASSOCIATION.ORG 21

COVER STORY

THE WAIT

One of the hardest parts of waiting for organ transplants is experiencing what’s known as a dry run, Ramirez says.

“They’re traumatic. They call you and say we have organs for you. You get there and they do all this testing while they’re testing organs from the donor.”

For her first dry run, Ramirez was at the hospital for five hours. As they were wheeling her into the operating room, she saw a man with a cooler who gestured that the surgery was a no-go.

The second time, Ramirez was sedated in the operating room and had started transplant medication. After a few hours, she woke up to find nurses disconnecting tubes. “I thought, ‘Oh no. Clearly, they wouldn’t be taking any tubes away if I’d had the transplant.”

Ramirez stayed in the ICU about 16 hours to wean off anti-rejection medication, prednisone, heparin, antibiotics, fentanyl and propofol.

Throughout the day, medical personnel explained they were shutting off or reducing a medication. Finally, they said she could go home.

“I held it together in ICU,” Ramirez said. “But when we walked into the apartment, I just sobbed.”

The next morning, Ramirez said she felt like she had been hit by a truck.

She was too physically and emotionally exhausted to go to rehab.

‘LET’S DO THIS’

On Sept. 13, 2023, Ramirez received a third call. When lung transplant surgeon John Haney confirmed the transplant would happen, “The last thing I said in the operating room was, ‘Let’s do this,’” Ramirez recalls.

COVER STORY

Ramirez woke up two days later.

The surgeon left her chest open to make sure everything was working properly and closed it the night after surgery. On the second day, she was extubated. “When I woke up, I didn’t know what day it was. I was really confused.”

By that afternoon, Ramirez was marching in place, connected to IVs, machines, pumps and chest tubes. Five days after surgery, she was out of ICU. “It was a quick turn-around for me.”

GOLDEN CHILD

Ramirez’ recovery went so smoothly that word got around. “You’re the transplant golden child. We’ve all heard about you,” one doctor told her.

Another said, “We just don’t have heart and lung stats like that.”

Despite her smooth recovery, Ramirez felt like she wasn’t getting enough air. Although her oxygen saturation was 100%, Ramirez had a hard time breathing, a common feeling among transplant recipients.

The nurse gave her a liter of oxygen for comfort.

“It was completely psychological,” Ramirez says. “There’s a delay with your brain connecting to the fact that you’re actually breathing well and that it can let go of how you used to breathe.”

TEMPORARY SETBACKS

About nine days later, Ramirez’s oxygen levels dropped low enough that she needed 4 liters of oxygen. Doctors performed a bronchoscopy and found a large mucus plug that caused the lungs to collapse at the bottom. When

they removed the plug, her new lungs expanded.

Afterward, Ramirez felt like she could breathe in a way she never could before. “I was ready to walk down the hall. I wanted to do a marathon. I didn’t need oxygen again after that.”

Ramirez stayed in the hospital for 14 days. She was readmitted 10 days later when she got a bad infection and fluid had to be drained from around her heart. Even then, she felt better than she did before the transplant.

Over the next month in the hospital, Ramirez had three “really gnarly” emergency surgeries. But she was walking a mile a day with a rollator the last week, despite the tubes and drains connected to her heart and lungs.

After she was discharged, she got another infection, but she still went to the gym every day with a PICC line for IV antibiotics.

“Exercise is really important,” Ramirez says. “You lose muscle mass during surgery.”

In the six months before transplant surgery, Ramirez had been walking and riding a bike five days a week.

After surgery, her legs were weak, and she felt like her muscles “were gone.”

CAREGIVING

One of the requirements for transplant is to have a caregiving team in place to be listed. “There’s no way you go through it alone,” Ramirez says.

Ramirez enlisted the support of her sister-in-law Tammy and her best

COVER STORY

friend, Nicole Creech, whom she met many years ago through PHA. Creech, who has PH and sickle cell, traveled from Lexington, Kentucky, to Duke as soon as Diane’s husband Dale called.

Creech, treasurer of PHA’s Board of Trustees, came to the hospital every day for the first 10 days after the transplant. Creech and Dale took turns accompanying Ramirez on her hallway walks, taking notes and asking questions when Ramirez met with doctors, and relieving each other to rest or shower.

“She got to know all the nurses.

They knew she was here for me.”

In the first few days, Dale slept in a recliner in the hospital room and wouldn’t leave his wife’s side.

“You can’t take that kind of stuff for granted,” Ramirez says.

‘I’m getting used to not having limitations, ... with pulmonary hypertension, I was used to working within limitations. Now I wonder what else I can do.’

LIFE BEYOND PH Above: Besties Nicole Creech and Diane Ramirez after transplant surgery.

Below: Dale and Diane Ramirez at discharge.

24 PATHLIGHT

While Ramirez no longer needs PH medications, she now takes 33 pills a day, including 10 mg of prednisone, 8 mg of Prograf (tacrolimus) and 2,000 mg of Cellcept (mycophenolate) to prevent her body from rejecting the new organs.

The high doses of prednisone caused diabetes, which Ramirez says is common for many people after transplant. Sometimes the diabetes goes away, but some people need treatment for high glucose the rest of their lives.

Unlike her PH medications, the anti-rejection medications are generally more affordable, with better insurance coverage. The anti-rejection medications fall under Tier 2 in the insurance formulary, compared to Tier 5 for most PH drugs.

“With PH drugs, you’re talking about $300,000 a year and your copay is 33%. I haven’t come across anything that expensive [for the transplant drugs].”

Since transplant, Ramirez says her life has changed completely. But she will always be part of the PH community.

“I may not have PH anymore, but I’m still here,” she says. “I still do advocacy and awareness for PH, and I have no intention of stopping. The illness hasn’t changed, and I have lost a brother and two sisters to pulmonary hypertension.”

Nearly a year after surgery, Ramirez sometimes is surprised by what her body can do.

“I knew I was going to be better. I just didn’t think I would be so much better so soon.”

With PH, she was never sure she could do certain activities and how much she could push herself without being wiped out the next day. Now, she participates in hourlong exercise classes.

“It’s a different way of thinking. I’m getting used to not having limitations.

And with pulmonary hypertension, I was used to working within limitations. Now I wonder what else I can do.”

PHASSOCIATION.ORG 25

HEALTH MATTERS

S WHAT TO KNOW ABOUT

Transplantation

BY MELANIE PADGETT POWERS

26 PATHLIGHT

uccessful lung transplants can dramatically improve quality of life for people with pulmonary hypertension and extend their lives by several years.

Not everyone with PH will need one, but for some people, a double-lung transplant may be the next life-giving step.

“Lung transplant becomes an option for people with PAH or other advanced pulmonary diseases when they have failed maximal medical management and their disease is progressing,” says Jamie L. Todd, a transplant pulmonologist at Duke Health in North Carolina.

Progression could mean poorer right heart catheterization measurements and decreasing sixminute walk distance. In addition, the person could have worsened symptoms and a declining quality of life, Todd says.

For people with PH, lung transplant generally means a doublelung transplant because the disease affects both lungs. The average survival rate after double-lung transplant is about seven years, according to the National, Heart, Lung and Blood Institute. The first year is the most critical. Data from 2020 show that 90% of lung transplant recipients will be alive one year later, while 61% will be alive at five years post-transplant, and 33% will be alive at 10 years.1 Getting a heart and lung transplant for pulmonary arterial hypertension is less common because studies have shown that a lung transplant by itself often is sufficient. New lungs allow the previously struggling heart to improve.

Beyond surviving, lung transplant recipients often are thriving. Quality of life can improve dramatically with higher energy levels, better mental health and the ability to do everyday activities, exercise and travel. After transplant, people no longer are tethered to pumps and catheters or supplemental oxygen.

“Most of my patients tell me by six months post-transplant, they’re starting to come into their bodies and feel some glimmers of their old selves, and that by a year post-transplant, they feel fully recovered,” Todd says.

TRANSPLANT CANDIDATES

The International Society for Heart and Lung Transplantation recommends people on IV or subcutaneous prostacyclin therapy or those with worsening PAH be referred to a transplant team.

The earlier a person with PH is referred to a transplant center to learn about the process, the better, transplant pulmonologists say. When referred early, patients have more time to consider what a transplant means, ask questions, get medical evaluations and address barriers to transplant — even if they never need the surgery.

“It’s better to have all the information earlier rather than when you’re in a situation of medical duress,” says Matthew R. Pipeling, a transplant pulmonologist at Duke Health in North Carolina.

Ideally, lung transplants should take place before other organs are damaged. For example, PAH can cause kidney and liver disease.

“We’re also looking to get involved in a person’s care when they’re still functional,” Todd says. “If the disease

has gotten so bad that they can’t get out of the hospital or can’t get out of bed, then transplant becomes a much higher-risk surgery.”

Medical teams at transplant centers should decide whether someone is a good candidate for transplant, these transplant pulmonologists say. If a PH

‘Most of my patients tell me by six months post-transplant, they’re starting to come into their bodies and feel some glimmers of their old selves.’

JAMIE L. TODD, MD

doctor says a patient isn’t a candidate for transplant, the patient can ask for a referral to a transplant center, even if their PH is relatively stable.

“Being referred for transplant does not mean that you are getting a transplant and does not mean that you need a transplant right now,” says Nicholas Kolaitis, a transplant pulmonologist at University of California-San Francisco. “Being referred for transplant and meeting with the transplant team should be viewed as an educational opportunity to learn about transplant and whether it’s something you might want in the future if you actually need it … The worst thing that happens is a patient is referred to transplant too late.”

Transplant pulmonologists and centers base transplant candidacy on several medical, surgical and social factors. A medical evaluation looks at other health conditions, kidney or liver PHASSOCIATION.ORG 27

HEALTH MATTERS

damage, or heart disease. A surgical evaluation examines whether a person is fit enough to survive surgery.

Candidates must have a social support system to help them recover from surgery and perform everyday tasks such as dressing, organizing medications, grocery shopping and traveling to pulmonary rehabilitation and doctor appointments.

REJECTION AND INFECTION

Risks after lung transplant surgery fall into two areas: rejection and infection.

“Transplant is not perfect,” Kolaitis says. The way that I view transplant is it’s trading one disease for another.”

Todd tells her patients that for the first six months after transplant their full-time job is to get better. That means going to all pulmonary rehab appointments, improving nutrition, learning the new medication regimen

HEALTH MATTERS

and working with the transplant pulmonology team to deal with side effects.

There are three primary types of organ rejection. About 30% of lung transplant recipients will experience “acute rejection” in the first year after transplant, Kolaitis says. In those cases, most people get better after taking high doses of steroids.

“Antibody-mediated rejection” is rare and is treated with chemotherapytype drugs. Chronic rejection” can happen months or years after transplant. There are medications to treat chronic rejection, but they don’t work in everyone. If lungs begin to fail and don’t respond to treatment, it’s rare for someone to go back on the list for a second transplant.

However, the biggest risk after transplant — and the most common cause of death within the first year — is

‘It’s better to have all the information earlier rather than when you’re in a situation of medical duress.’

infection. Transplant recipients must take medications to suppress their immune system for the rest of their lives. After transplant, the immune system sees the new lungs as an invading virus to attack, which could lead to the body rejecting the lungs. Medications that suppress the immune system reduce that risk.

Because of those medications, transplant recipients will always be immunosuppressed. If they get sick, they are at higher risk of getting very sick. So, it’s critical to try to avoid catching illnesses, especially respiratory viruses like COVID-19, flu and RSV. Recommendations on how to avoid illness vary by transplant center. Plus, each person will need to make their own decisions on how they want to live their life and the risks they are willing to take, Pipeling says.

Transplant pulmonologists might recommend wearing a mask in large gatherings or avoiding crowds as much as possible. Family and friends should stay away when they are sick.

Some transplant centers recommend no alcohol, while others recommend one drink only a couple times a year. Alcohol can interact poorly with rejection medications, and even non-alcoholic beer has trace amounts of alcohol.

Transplant recipients shouldn’t eat uncooked fish (raw oysters or sushi), raw or undercooked eggs, or unpasteurized milk and cheese because of the risk of food poisoning. For the same reason, they must be careful eating at potlucks or buffets, where the food may have been out too long and/ or not washed properly. Also, many times gardeners are asked to give up their hobby because of the disease risk from fungus in soil.

system, the United Network of Organ Sharing changed the way lungs are distributed.

Now the “urgency” system gives a score to each person based on the severity of their disease and likely success of transplant surgery. The score is based on several medical tests.

While the newer system is an improvement, there are still some inequities for people with PH, Kolaitis says. That’s because the measurements that define “urgency” don’t favor those with PH. For

example, people with PH aren’t affected much by measurements such as lung size or carbon dioxide level. Those measurements are more relevant in diseases of the lung tissue, such as chronic obstructive pulmonary disease, not diseases of the lung vessels like PH. After the 2005 changes, every lung disease reflected on the transplant list — except PH — saw fewer people dying while still on the list. While UNOS has made revisions since then to try to address this problem, people with PH are still

getting lung transplants later in their disease, when they are very sick, Kolaitis says.

“The fact that patients with pulmonary hypertension need to be sick enough to overcome the disadvantage of the organ allocation means that we’re transplanting patients with pulmonary hypertension later than we should be, which leads to poor outcomes after transplantation,” he says. “So this needs to be revised from an advocacy standpoint.”

MATTHEW R. PIPELING, MD

Lung transplant recipients will see their transplant pulmonologist for the rest of their lives, more often in the first year. “Transplant is a major commitment and a life-altering event,” Todd says.

But the impact on one’s health and quality of life can be phenomenal. “I’ve certainly seen people go back to work or volunteer in a role that’s meaningful to them,” she says. “We’ve had people go back to college and get degrees, travel, including international travel, again. I don’t think there’s much that people have said they want to do that they can’t do after transplant.”

1 Valapour M, Lehr CJ, Skeans MA, et al. OPTN/

SRTR 2020 Annual Data Report: Lung. Am J Transplant. 2022; 22(suppl 2):438-518.

Lung Transplant Inequities

O Nicholas Kolaitis, MD

28 PATHLIGHT

ne to 3% of approximately 5,000 lung transplants each year around the world are for pulmonary hypertension, according to Nicholas Kolaitis, a transplant pulmonologist at University of California-San Francisco. That’s about 50 to 150 lung transplants worldwide for PH each year.

Historically, new lungs were matched with recipients in the U.S. based on a person’s time on the lung transplant wait list. But in 2005, in an effort to create a fairer

PHASSOCIATION.ORG 29

ADVOCACY

ADVOCACY

United in Seeking PH-Related Legislative Reforms

How Advocacy Helps Patients and Families

BY MARINA SALAZAR

I MARINA SALAZAR is a licensed clinical social worker at the University of California-San Francisco Medical Center’s PHAaccredited pulmonary hypertension program.

Salazar, who leads the PHA Support Group at UCSF, participated in the Pulmonary Hypertension Association’s May advocacy campaign to mark World Pulmonary Hypertension Day.

30 PATHLIGHT

have long enjoyed working with patients and families navigating chronic illness. It’s a privilege to offer support and be part of their journey. My favorite parts of my job are getting to know our fabulous patients and families, facilitating our support group and collaborating with my incredible colleagues.

Advocacy is critical for improving patient care and access to treatment, as well as ensuring patient needs are being met. Health care is a complex system to navigate, and anything we can do to make it easier and amplify our patients’ voices is important. Chronic illness is hard enough to manage — we must seek opportunities to minimize stress on our patients and families whenever possible.

Medical professionals have a unique perspective on the complex needs of pulmonary hypertension patients and their families. We see the challenges our patients face every day. We can offer specific insight to legislators on what we’ve learned, with the goal of creating meaningful change. Advocacy helps us maintain motivation and hope as medical professionals that there is always room for growth and improvement in the policies that regulate our health care system.

It’s easy to forget that oxygen, in addition to medication, is a treatment for PH. It can reduce symptoms of pulmonary hypertension, improve patients’ quality of life and allow them to engage in

meaningful activities. Quality of life is critical for mental health and helping patients navigate the emotional challenges associated with chronic illness. Without oxygen, many of our patients couldn’t safely work, exercise, go on outings or spend time with loved ones.

Easier access to oxygen would reduce the stress and anxiety that often occurs when patients struggle to get the treatment they need and deserve. It would ensure that all patients can access the recommended amount and type of oxygen, regardless of their income level or insurance coverage.

We want to give patients the best chance at treating and slowing the progression of their condition, and oxygen is part of that equation for many folks.

Learn more about PHA advocacy priorities.

Sign up for our Advocacy Action Alert newsletter: PHAssociation.org/ Patients/News-and-information/ Email-subscriptions.

A

lthough Renee Mestayer of New Iberia, Louisiana currently doesn’t need high-flow supplementary oxygen, she participated in a recent PHA advocacy campaign.

“I’ve needed oxygen on and off over the 25 years I’ve been dealing with this disease,” says Mestayer, who was diagnosed in 1998. “It’s reassuring to know that when I do need it, there is legislation that could make it easier to get.”

Mestayer is among the pulmonary hypertension advocates who met with 33 lawmakers’ offices to seek support for Pulmonary Hypertension Association legislative priorities. The advocacy campaign began May 5 in connection with World PH Day and ran throughout May. The virtual advocacy meetings focused on the Supplemental Oxygen Access Reform Act, introduced this year in the House of Representatives and the Senate. Advocates also asked for support of the Safe Step Act and the HELP Copays Act. The Safe Step Act would create reasonable exceptions to insurance companies’ policies that require patients to first fail on a cheaper drug before covering a more expensive one. The HELP Copays Act would ensure payments made on behalf of patients by third parties count toward deductibles or out-of-pocket maximums.

The SOAR Act, which proposes to remove supplemental oxygen from Medicare’s competitive bidding process, would make oxygen and related equipment more affordable and accessible. By mid-June, the bill had gained 11 co-sponsors in the House since PHA’s advocacy campaign began in May. “It’s important to make our representatives aware of what we as patients are going through day to day,” Mestayer says. “If we don’t tell them, then they will have no idea of the challenges that we are facing.”

“The SOAR Act is incredibly important as there is no generic for oxygen,” says Patti Sakoman, who participated in the advocacy campaign with her son and caregiver Josh Franson. Sakoman needed continuousflow oxygen for a year and a half before using oxygen only at night.

In their call with lawmakers, Sakoman explained that she struggled with having enough oxygen tanks to get safely to and from doctor appointments, even with Franson’s help.

A day after the call, they learned that their representative, Deborah Ross (D-N.C.) had co-sponsored the bill.

In addition to people with PH and caregivers, health care professionals also participated in the campaign.

“Oxygen is a necessary part of treatment for almost all pulmonary

Renee Mestayer

hypertension patients,” says Kassandra Olgers, a physician assistant Western Virginia University Medicine. “If patients do not have access to more portable forms, their livelihood is affected, which in turn leads to reduced quality of life and depression.

“If we had fewer barriers to more portable forms of oxygen for all PH patients, they would have improvement in their day to day life. Supplemental oxygen is a burden to carry — literally and figuratively — and we should do everything in our power to help patients.”

Learn more about PHA’s advocacy work, including our PHA 2024 Advocacy Challenge.

PHAssociation.org/advocacy PHASSOCIATION.ORG 31

QUICKTAKES

HOW TO REACH US TEL 301-565-3004

WEB PHAssociation.org

We’re Moving!

PHA Leaders Explore Care Access, Partnership Opportunities

T

P

he Pulmonary Hypertension Association is moving to a new office in Washington, D.C. Now that our team is working from home all over the country, we no longer need as much space.

The smaller office will save us money, leaving more funds to support the PHA community.

Our new address is 1121 14th St. NW, Suite 150, Washington, D.C., 20005.

Legislative Briefing Focuses on Patient Experiences

C

olleen Connor, a Pulmonary Hypertension Association board member, was among the PHA advocates who urged improved access to supplemental oxygen in a June 5 legislation briefing on Capitol Hill. The advocates also called for support for the Safe Step and HELP Copays acts.

“Imagine every time you leave your home, you need to plan how much time you will be outside your home and your activity level, so you know how much supplemental oxygen to sustain you,” Connor told congressional staffers. “You are restricted by the monthly allocation you receive, so you are forced to make tough choices ... Even doing that, I can only leave my house 14 hours a week.”

Heather Mayberry, a clinical nurse specialist and PH clinical coordinator at Centra Lynchburg General Hospital, and actor/singer Stephen CarterHicks, a PHA volunteer, also spoke at the event.

Pictured L-R: Stephen Carter-Hicks, Heather Mayberry, Matt Granato, Colleen Connor.

32 PATHLIGHT

| FAX 301-565-3994

EMAIL PHA@PHAssociation.org

ulmonary Hypertension Association leaders met with industry and corporate partners during the American Thoracic Society Conference. At the conference, PHA President and CEO Matt Granato was appointed to the ATS Health Equity and Diversity Committee. During the event, Granato and other PHA leaders attended sessions on diversity and inclusion. The sessions highlighted the need to reach out to disadvantaged populations to ensure all patients receive the care they need.

PHA leaders also discussed drug development and partnership opportunities with representatives from Aerovate Therapeutics, Bayer, CorVista Health, CVS Specialty, Gossamer Bio, Halo Biosciences, Johnson & Johnson, Liquidia, Merck, Oxiwear, Respira Therapeutics, United Therapeutics and VisionHealth.

MARK YOUR CALENDAR

Philanthropist Donates Major Gift in Memory of Artist Wife

Open Enrollment Season Is Approaching

T

Remember these important dates to change or update your 2025 health care plan.

Medicare: Oct. 1-Dec. 7, 2024.

Medicare Advantage transition period and original Medicare late enrollment: Jan. 1-March 31, 2025.

Health insurance marketplace: Nov. 1-Dec. 15, 2024. Late enrollment cut-off is Jan. 15, 2025, or later in some states.

he estate of Reinhold Vogel donated $486,000 to the Pulmonary Hypertension Association. The gift is in memory of Vogel’s late wife, Mary Iafolla Vogel, who died in 2005 from pulmonary hypertension. Mary Vogel, originally of Yonkers, was an artist, interior decorator and teacher. She taught at Hunter and Elizabeth Seton colleges. Reinhold Vogel died in 2022 at age 91. He was a founder of Federal Scientific Corp., which designed equipment to track long-range missiles. He later was director of digital systems for Loral Electronics Systems, which developed radar equipment

PHA Names Pediatric Research Award Recipient

A

Photo credit: Jennifer Turiano/Hearst Media CT

for F15 and F16 fighters. The Vogels married in New York in 1953 and spent much of their lives in Greenwich, Connecticut.

imee Layton, director of the pediatric cardiopulmonary exercise laboratory at Columbia University, received PHA’s most recent Pediatric PH Research Award. The grant will allow Layton to design a tele-rehabilitation program that allows patients with chronic illnesses to exercise from home under the guidance of health care professionals. Her project will provide data on how becoming physically active with peers can affect fear and anxiety.

PHASSOCIATION.ORG 33

PATHLIGHT

CALENDAR OF

EVENTS

Passages Since the Pulmonary Hypertension Association (PHA) began publishing Pathlight, “Passages” has provided a place to memorialize people with pulmonary hypertension who pass away. PHA extends its sympathy to the families and friends of these individuals and rededicates itself to its mission in their memory.

■ PHA Events ■ National O2breathe Events ■ Community Fundraising Events

AUGUST

SEPTEMBER

AUG. 25 PH in the Park Denver PHAssociation.donordrive. com/event/phinthepark

SEPT. 8 Northern Californinia O₂breathe Walk San Francisco PHAssociation.donordrive. com/event/northernca24 SEPT. 21 Puttin PHore Poots Edina, Minnesota PHAssociation.donordrive. com/event/puttin24 Chicago Stride O₂breathe Walk Rolling Meadows, Illinois PHAssociation.donordrive. com/event/chicago24

SEPT. 22 Columbus O₂breathe Walk Gahanna, Ohio PHAssociation.donordrive. com/event/columbus24

OCTOBER

OCT. 5 Boston O₂breathe Walk Quincy, Massachusetts PHAssociation.donordrive. com/event/boston24 OCT. 19 Philadelphia O₂breathe Walk PHAssociation.donordrive. com/event/Philly24 OCT. 26 PHA Connects: PH Community Workshop Phoenix PHAssociation.org/ phworkshop OCT. 27 Phoenix O₂breathe Walk Scottsdale, Arizona PHAssociation.donordrive. com/event/phoenix24

NOVEMBER

PH AWARENESS MONTH NOV. 2 Monrovia, California, O₂breathe Walk PHAssociation.donordrive. com/event/monrovia24 NOV. 2 El Paso, Texas, O₂breathe Walk PHAssociation.donordrive. com/event/elpaso24

Contact us at 240-485-0762 or events@PHAssociation.org.

Louis Lowell Debra McClellan Ann McEvoy Vanda L. McLean Amy Y. Melius Barbara A. Micco Ann C. Murphy Mary Ellen O'Rourke Roger Poole Kris Rocca Karen Whittle Cathy Wilson Ludmila Wren Jennifer L. Wright

See our calendar at PHAssociation.org/ events.

Visit PHAssociation.org/pha-fundraising-events to find a fundraising event in your area.

QUESTIONS?

Eleanor Austin Lucy Bertucci David Boissonneault John B. Boyd Donna Clark Hattie M. Clay Margaret Crump Caroline Dawson Danielle Del Pinto Judy Dotson Jeannie A. Durnar Willow J. Eggleston Barbara Haft Lori Kirby

NOV. 9 Dallas Zebra PHest O₂breathe Walk Plano, Texas PHAssociation.donordrive. com/event/zebraphest24

VIRTUAL FUNDRAISERS

34 PATHLIGHT

PHA’S LEGACY OF HOPE

The accuracy of this list is important to us. Please contact the PHA office at 301-565-3004 x746 or Passages@PHAssociation.org to share the name of your recently deceased loved one or report an error or omission.

Your donations in memory of others, in honor of others and in support of our mission mean so much to the entire PH community, and we thank you.

To honor those who have included PHA in their estate plans or whose legacies have been realized, PHA created the Legacy of Hope Society. PHA is pleased to recognize the following members: Bonnie and Michael D. McGoon Laura* and Rino Aldrighetti* Kay McMillin Alice A. Arnott* Joseph W. Mihuc* Sandra A. Awood* Linda Miles* Dauna L. Bauer* Karen S. Moody Sylvia M. Becherer* Larry D. Moody Joan F. Bennett-Schenecker* Pamela R. Morris Kris L. Best Marjorie D. Mott* Gloria G. Blodgett* Joyce L. Mowrer* Dorothy E. Bradley* Pamela and Timothy O’Connor Mary M. Brady* Helina Ofori R. J. Braun Dorothy M.* and Harry J. Olson* Roberta F. Browning* and Lee A. Broadbent Theresa "Terry" E. (Cavanaugh) O'Reilly* Rita and Bruce Brundage Rita and Guy Orth Colleen Brunetti Patricia R.* and Gerald D. Paton* John and Mary Ann Butler Cynthia and John R. Pickles Stephen Carter-Hicks Jean D. Pitcher* Colleen and Shawn Connor Carol L. Powell* Jane P.* and Harold P. Cooper Frances A. Price James F. Corbett* Carol J. Posner* and Marc Priore Nicole M. Creech Susan Tess Rae* Laura H. D'Anna Diane Ramirez Charles W. DeVier, III* Cyndy D. Reynolds* Linda M. Feibel* Dorothy A. Ryan* Barbara T. Gamer James Ryan* Stacey Gausling* Louise C.* and Gene P.* Salvucci Franklin D. Gillespie* Judith and Edwin L. Simpson Jeffery S. Hall* Kelley Skumautz Jeanette Hatch* Laverne Smith* Tammy A.* and Dean S. Hazen Debhra Ann Henderson* Joanne Sperando Marcia and Jack Stibbs Carl Hicks, Jr. Helena M. Strauch* Phyllis M. Hill* Douglas R. Taylor Jackie Holt Frank A. Tobac* Richard L. Horrocks Constance G. Ives* Martha and Carlos Torres Deborah J. and Roger K. Towle Terri L. Jakuboski* Carol B. Ungar Stanley T. Jusinski Laura J. Kelly* Reinhold Vogel* Carol E. Vreim James Kenney Daniel R. Walsh* Jessie Kohler-Wenninger Sharon K. Wendt* Terri L. Kopp* Debbie L. and Mitchell Koppelman Shirley G. Weaver* Frank D. Koppelman* Andrea and Stephen L. White * deceased Dee* and Walter Kruger* Gloria J. Lang* Marie* and Ronald J. Levendoski Mary Jo* and Thomas Linnen* Sally Maddox* Sherry Mannel

PHASSOCIATION.ORG 35

PULMONARY HYPERTENSION ASSOCIATION 1121 14TH ST. NW, SUITE 150 WASHINGTON, DC, 20005

10 YEARS

OF EXPERT PH CARE Pulmonary Hypertension Association accreditation establishes medical centers as an authority in pulmonary hypertension care. The PH Care Center network aims to: • • • • • •

Increase access to care.

Facilitate earlier diagnosis.

Improve patient outcomes.

Raise quality of care.

Promote PH care standards.

Enhance professional expertise.

• Expand patient registry.

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