Pathlight - Spring 2020

A publication of the Pulmonary Hypertension Association

Issue 2 2020 // Volume 29 No. 2

‘Achieving the impossible’ via surrogacy

PLUS Get Moving with Exercise Videos PHA Connects: A Health and Wellness Series Finding Hope Through Loss

IN THIS ISSUE

WHAT CAUSES PAH?

HOW DOES PAH AFFECT THE HEART?

WHAT HAPPENS TO THE PULMONARY ARTERIES IN PAH? Find answers to your questions at PAHuman.com

ACTPT20014 PAHuman.com 4CB Journal Ad - Pathlight Colors: 4CP Bleed/Trim/Live - Do Not Print Bleed: 8.75"w x 11.25"h Trim: 8.5"w x 11"h Live: 8"w x 10.5"h Output @ 100% Giant Creative Strategy

HOW COMMON IS PAH?

WHAT IS PULMONARY ARTERIAL HYPERTENSION (PAH)?

2020 // Volume 29 No. 2

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Research Grows as PHA Registry Enrollment Nears 1,300

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5 PHA Classroom Videos Help You Get Off the Couch and Get Moving

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Connecting in the Time of COVID-19

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Make a Difference During Advocacy August

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Make the Most of PHA’s New Treatment Access Resources

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World PH Day Goes Social

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PH Journey: A Patient and Nurse Practitioner Connection

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My Miracle Baby

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Avenues to Adoption

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Congratulations, You’re a Caregiver. You’ve Got This.

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Finding Hope Through Loss

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Legacy of Hope: A Gift to Help Others

IN EVERY ISSUE 5

Letter From the Chair

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Calendar

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Support Group Check-In

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Passages

PAHuman is a registered trademark of Actelion Pharmaceuticals Ltd © 2018 Actelion Pharmaceuticals US, Inc. All rights reserved. ACT-01841 0218

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BOARD OF TRUSTEES Karen A. Fagan, M.D., chair

PUBLISHER

Colleen Brunetti, M.Ed., C.H.C., chair-elect

Brad A. Wong President & CEO

Roger Towle, immediate past chair

STAFF

Diane Ramirez, secretary

Alanna Coogan Designer Karen Smaalders Vice President, Communications and Marketing

CONTRIBUTORS Linda Busche, Skye Ellison, Nancy Jordan, Mike Knappen, Mira Kruger, Julia Selby, Abby Sickles and Kelley Skumautz.

CONTACT As of June 1: 8401 Colesville Road, Suite 200 Silver Spring, MD 20910

William A. Hoff, treasurer

Trustees-at-Large Cheri Abbott, R.N., CCRP Erika Berman Rosenzweig, M.D. Frank Cann Murali M. Chakinala, M.D., FCCP Colleen Connor Nicole M. Creech Ramona Doyle, M.D., M.Sc. Jessie Kohler Wenninger, Esq. Mitch Koppelman, Ph.D. Tony Lahnston Ronald J. Oudiz, M.D. Monica M. Penaranda Fran Rogers, M.S.N., CRNP Traci Stewart, R.N., M.S.N., CHFN Doug Taylor Matt Wall Melisa A. Wilson, ARNP, ACNP-BC Brad A. Wong

Phone: 301-565-3004 Fax: 301-565-3994 Support Line: 800-748-7274 We’re here to support anyone with a connection to PH. (daytime, please) PHAssociation.org PHA@PHAssociation.org Submissions to Pathlight are welcome. Please email your articles, photos and queries to Communications@ PHAssociation.org.

Emeriti ex-officio C. Gregory Elliott, M.D., FCCP, MACP Michael D. McGoon, M.D. Pat Paton, R.N. Edwin Simpson Judith Simpson, R.N., Ed.S. Honorary Carl Hicks President & CEO Brad A. Wong PH PROFESSIONAL NETWORK EXECUTIVE COMMITTEE Fran Rogers, M.S.N., CRNP, chair Cheri Abbott, R.N., CCRP, chair-elect Johnell Diwan, R.N., B.S.N. Kimberly Jackson, R.N., B.S.N. Tisha Kivett, R.N., B.S.N. Susie McDevitt, M.S.N., ACNP-BC

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SCIENTIFIC LEADERSHIP COUNCIL Ronald J. Oudiz, M.D., chair Murali M. Chakinala, M.D., FCCP, chair-elect Erika Berman Rosenzweig, M.D., immediate past chair Steven Abman, M.D. William R. Auger, M.D. Sonja Bartolome, M.D. Todd M. Bull, M.D. Kelly Chin, M.D. Lorinda Chung, M.D. Vinicio A. de Jesus Perez, M.D., FCCP, FAHA Teresa De Marco, M.D. Ankit A. Desai, M.D., FACC, FAHA Jeffrey Fineman, M.D. Robert P. Frantz, M.D. Mardi Gomberg-Maitland, M.D., M.Sc. Anna Hemnes, M.D. Steven Kawut, M.D., M.S. Tim Lahm, M.D. Deborah J. Levine, M.D. Roberto F. Machado, M.D. Lana D. Melendres-Groves, M.D. John Ryan, M.D. Jeffrey Sager, M.D., MSCE Oksana A. Shlobin, M.D., FCCP Thenappan Thenappan, M.D. Corey E. Ventetuolo, M.D., M.S. Timothy L. Williamson, M.D. Delphine Yung, M.D. Liaisons Fran Rogers, M.S.N., CRNP Cheri Abbott, R.N., CCRP Nicole Reid, R.N., B.S.N., DABAT Distinguished Advisors David B. Badesch, M.D. Bruce H. Brundage, M.D. Richard Channick, M.D. C. Gregory Elliott, M.D., FCCP, MACP Karen A. Fagan, M.D. Michael D. McGoon, M.D. Vallerie V. McLaughlin, M.D. John H. Newman, M.D.

As I began my term as chair of the Pulmonary Hypertension Association (PHA) Board of Trustees two years ago, I was energized by the recent PH International Conference and Scientific Sessions.

Since joining PHA’s Scientific Leadership Council (SLC) in 2004, I have enjoyed the educational opportunities, personal connections and examples of hope that arise from Conference. Sadly, with Conference canceled because of the pandemic, I can’t end my term in the same manner.

But I am heartened by the spirit the pulmonary hypertension (PH) community has shown in response to COVID-19. Our community has shown its (right) heart like never before, despite health concerns, isolation, personal and economic loss, and great sacrifice. To help the community cope, PHA quickly pivoted from Conference planning to virtual programming and support.

The June issue of Pathlight addresses how PHA helps our community navigate this crisis: virtual support groups, resources on PHAssociation.org and PHA Connects: COVID-19 and Your Health.

This month’s magazine also provides ample inspiration. Especially memorable is a story we had hoped to present at the Journeys Luncheon at Conference: How Allison Dsouza and nurse practitioner Elizabeth Colglazier, MS, PNP-AC, “grew up” together in PH. Our cover story is equally remarkable. Skye Ellison, whose childhood artwork graced PHA holiday cards nearly two decades ago, shares the challenges related to invitro fertilization and surrogacy to start a family.

Their stories – and those of everyone in our community – are ongoing reminders of why I’ve been involved in PHA for 16 years. I’m especially proud of my contributions to PHA’s mission and the work PHA has accomplished during my chairmanship.

Last fall, I co-presented a women’s issues session at

the PHPN Symposium. The session developed from interest in Living with PH: A Guide to Intimacy.

The guide and session show PHA’s commitment to address important topics for people with PH and the health care professionals who treat them.

Over the last two years, PHA has expanded resources for everyone. To help medical professionals maintain their continuing education, PHA added options to PHA Online University. To give clinicians more resources for patients, PHA produced several patient-focused worksheets. Access to quality care is growing with more than 80 PHAaccredited Pulmonary Hypertension Care Centers.

Many centers collect data through the 1,300-patient PHA Registry.

Caregivers have many new resources too. In the past two years, PHA: • Unveiled a Clinical Trial Finder to find PH clinical trials throughout the country.

• Expanded pediatric resources.

• Created the PH & brochure series that addresses screening, diagnosis and treatment options for people with associated diseases and conditions.

• Added more videos in the PHA Classroom series, including five new exercise videos covered in this month’s magazine.

• Developed a bereavement brochure.

It’s been a privilege to serve as your chair, and I look forward to seeing PHA grow and meet our community’s needs under new chair Colleen Brunetti. Since we can’t pass the gavel at Conference, please join me in extending a huge welcome and virtual hug to Colleen, the second PH patient to serve in this volunteer role! Her perspective will be invaluable in setting PHA’s direction and reinforcing the patient focus so important to this organization.

All my best,

Karen A. Fagan, M.D. Board of Trustees Chair, Pulmonary Hypertension Association Professor of Medicine and Pharmacology, Chief of Pulmonary and Critical Care Medicine, University of South Alabama PHASSOCIATION.ORG

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5 PHA Classroom Videos Help You Get Off the Couch and Get Moving

Research Grows as PHA Registry Enrollment Nears 1,300

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he Pulmonary Hypertension Association Registry (PHAR) includes data from nearly 1,300 patients. Researchers use the PHAR database to learn more about pulmonary hypertension (PH). PHAR data includes demographic characteristics, diagnosis and treatment information, and quality-of-care metrics.

PHAR collects data from people with pulmonary arterial hypertension (PAH), pediatric PH due to developmental lung disease and chronic thromboembolic pulmonary hypertension (CTEPH).

They need data from as many patients as possible to make meaningful discoveries about PH. At press time, PHAR included patient data from 47 adult and seven pediatric Pulmonary Hypertension Care Centers (PHCC).

Researchers use PHAR data for academic articles, abstracts and presentations. Since 2016, the PHAR Data Access/Publications and Presentations Committee has received 23 research proposals and two manuscripts. Recent abstracts using PHAR data accepted for American Thoracic Society (ATS) 2020 include: • Derivation of the Pulmonary Hypertension Association Registry Evaluation (Grinnan et al.)

• Health Disparities and Treatment Patterns in Portopulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension: Analysis of the Pulmonary Hypertension Association Registry (DuBrock et al.)

• Hospitalization in Connective Tissue Disease Associated Pulmonary Arterial Hypertension: The Pulmonary Hypertension Association Registry (Mullin et al.)

• Pulmonary Artery Compliance in Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: The Pulmonary Hypertension Association Registry (McCormick et al.)

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• Quality of Life of Patients With Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Idiopathic Pulmonary Arterial Hypertension (IPAH): The Pulmonary Hypertension Association Registry (PHAR) (Narasimmal et al.)

• Risk Prediction Models Are Associated With Patient Centered Outcomes in PAH: The Pulmonary Hypertension Association Registry (Min et al.)

Learn more at PHAssociation.org/recent-researchand-the-pha-registry.

Safeguarding Patients During Pandemic As health care facilities face challenges related to COVID-19, Pulmonary Hypertension Association (PHA)-accredited sites have reduced in-person clinic visits and now offer more telephone or video-enabled visits to maintain consistent support for patients.

To date, 80 PH centers are PHA-accredited through the PH Care Centers initiative. That includes 59 adult Centers of Comprehensive Care (CCCs), 13 adult Regional Clinical Programs (RCPs), and eight pediatric CCCs.

Although new onsite reviews have been postponed until the end of the year, new applicants continue to apply. To find a PHA-accredited site near you, visit: PHAssociation.org/phcarecenters/ accredited-centers/

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s the Pulmonary Hypertension Association (PHA) launched its landmark exercise video series in March, the world was changing.

A rapidly spreading coronavirus prompted government entities to issue stay-at-home orders, close non-essential businesses, reduce transit services and restrict travel. Health care facilities were overwhelmed with seriously ill patients with COVID-19 and insufficient personal protective equipment, ventilators and other supplies.

The pandemic changed plans for University of Rochester Medical Center physicians who helped create the PHA Classroom Exercise Series and had hoped to introduce the five videos and worksheets to their pulmonary hypertension (PH) patients in person. Dan Lachant, D.O., and Jim White, M.D., Ph.D., from the medical center’s PH program, are among the growing numbers of PH-treating

physicians who promote the benefits of exercise for many PH patients. By the time the videos were posted to PHAssociation.org/Classroom, they were treating most patients by phone or televisit.

“Now that they’re stuck at home, people are looking for things to do,” Dr. Lachant says. “This is safe to do in the comfort of your home.”

Dr. White approached PHA in 2018 about working together on videos that highlight the importance of exercise. The idea began germinating after several studies since the mid-2000s showed exercise could be beneficial for PH patients.

The studies made Dr. White feel comfortable recommending light weights, walking on a treadmill and riding a stationary bike to PH patients.

5 new videos on PHAssociation.org/Classroom: 1.

2.

3.

4.

5.

Introduction to Exercise Introduction to Pulmonary Rehabilitation Introduction to Yoga Introduction to Cardio Introduction to Strength Training

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HEALTH MATTERS

HEALTH MATTERS

of pulmonary rehabilitation exercises in the Introduction to Pulmonary Rehab video.

Dr. Lachant advises his patients to start with the Introduction to Exercise, yoga and strength training videos because they show activities patients can do at home without special equipment.

He explains that disability from PH can worsen as patients’ muscles weaken from inactivity. “If they don’t do easy things like walking, they don’t get the maximum benefit of their life-saving drugs,” Dr. Lachant says.

The 2018 World Symposium on Pulmonary Hypertension (WSPH) in France confirmed the need to educate PH patients about the benefits of exercise, he says. At the meeting, the Patients’ Perspective Task Force discussed the need to empower patients in managing their PH.1 The WSPH proceedings highlighted the role of non-pharmacologic therapies in improving health-related quality of life, including exercise programs.

“It was the first time there was agreement that exercise was beneficial to people with PH,” Dr. White says. Many physicians had been advising PH patients against exercising since the 1980s because it made them short of breath. “The idea that people needed to be packed in egg crates was slow to die.”

“Hopefully, in years to come, we can say this will lead to better outcomes for your disease.”

After PHA sent a film crew to Rochester, New York, it consulted its Scientific Leadership Council (SLC) and PH Clinicians and Researchers (PHCR) network. The groups provided input on safety messages and other considerations for the videos

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and complementary worksheets. They wanted to emphasize that exercise might not be appropriate for every patient and that patients should consult their PH doctors before beginning a workout program.

Patients must read and agree to disclaimers to watch the videos.

“Hopefully, in years to come, we can say this will lead to better outcomes for your disease,” Dr. White says.

The series begins with an Introduction to Exercise from Drs. White and Lachant. They explain the series and what happens to the heart, lungs and muscles during exercise.

The Introduction to Strength Training video demonstrates exercises to help patients strengthen their arms, shoulders and backs. Such exercises allow patients to improve their quality of life by building strength to push a vacuum cleaner or carry groceries, White says.

The Introduction to Yoga video shows several yoga poses to improve flexibility, balance, strength and focus. Caroline M. Quill, M.D, University of Rochester Medical Center, demonstrates standing and seated modifications.

In the Introduction to Cardio, Lindsay McAvoy, R.R.T, shows simple exercises using treadmills and exercise bikes. McAvoy also gives an overview

https://erj.ersjournals.com/content/erj/early/2018/10/11/13993003.01919-2018.full.pdf

1

A goal of the exercise series was to motivate and help patients understand what happens to their bodies when they exercise. The videos needed to show people with PH so patients could envision themselves exercising.

Six PH patients from the University of Rochester Medical Center volunteered to participate: Kerri Claffey, Sammy M. Jones Jr., Mychea Morehouse, Patrick B. O’Connor, Jhenna Pacelli and Elizabeth Quetchenbach. The patients range in age, body type and ethnicity and some have oxygen tanks.

“We tried to demonstrate to different ability levels,” says Mary Beth Brown, P.T., Ph.D., who has studied exercise and PH for nearly a decade, beginning with her postdoctoral research in a pulmonary critical care department at Indiana University. Early in her career, she tested a home-based exercise program for

pulmonary arterial hypertension (PAH) patients, funded by a PHA and American Thoracic Society (ATS) grant. “We had good adherence … We tried to make it fun and motivate them.”

Now director of research for the physical therapy division of the University of Washington’s Department of Rehabilitation, Mary Beth demonstrated exercises in the strength training video.

“I was excited to do what I love, which is helping people exercise through therapy.”

The videos minimize barriers to exercise. Patients can follow the videos after checking with their doctors, use supplies they have at home and exercise at their own pace at home on their own schedules.

“There’s no reason for patients not to take advantage of this important therapy,” Mary Beth says.

The PHA Classroom Exercise Series is supported by the PHA Community PH Education Program: Actelion Pharmaceuticals, United Therapeutics, Bayer, Gilead and Acceleron Pharma.

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HEALTH MATTERS

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Connecting in the Time of COVID-19 PHA Programming Tackles Coronavirus Concerns

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hen we began planning this issue, we expected to be in Anaheim, California, by the time Pathlight landed in your mailbox. Soon afterward, the Pulmonary Hypertension Association (PHA) Board of Trustees made the anguishing decision to cancel the PHA 2020 PH International Conference and Scientific Sessions because of the coronavirus pandemic.

While our priority was to keep the pulmonary hypertension (PH) community safe, we shared the PH community’s disappointment in losing the opportunity to renew friendships, make new connections and hear about advancements in PH research.

Within days, we refocused our efforts on supporting the PH community during the pandemic.

We quickly launched the online resource center COVID-19 and Pulmonary Hypertension. The site at PHAssociation.org/COVID-19 includes a regularly updated COVID-19 FAQ with information from our health care community and the Centers for Disease Control and Prevention (CDC). This section is now the most popular area of the PHA website and features tips to maintain your health, understand the risks for people with underlying health conditions and digitally connect with others in the PH community. It also includes information on PH treatment access and financial assistance.

Thanks to the support of our corporate partners, we then launched PHA Connects: COVID-19 and Your Health, a series of interactive online events that address COVID-19 concerns and topics to help you live your best life with PH during the pandemic. Our first was a Facebook Live Q&A with Dr. Ron Oudiz, M.D., chair of PHA’s Scientific Leadership Council.

Within days, the video had reached nearly 9,000 viewers. Another Facebook Live event featured a Spanish-language Q&A on COVID-19 and PH with Dr. Gustavo Heresi, M.D., of the Cleveland Clinic.

A virtual town hall featured PH experts from Texas Children’s Hospital in Houston. Elise Caroline Whalen, M.S.N., APRN, FNP-C, C.P.N., and Nidhy P. Varghese, M.D., of Texas Children’s Hospital in Houston answered questions about pediatric issues related to COVID-19.

Upcoming virtual town halls for the months ahead will include: • Managing stress and anxiety • Maximizing your energy • Starting a home exercise program • Preparing for a telemedicine appointment • The drug discovery process • Advancements in PH research • Understanding the PH drug pipeline

To learn more about PHA Connects, view videos of previous events, or sign up for a Tuesday email that announces new programming, visit the PHA Connects webpage at PHAssociation.org/PHAConnects. PHA Connects is made possible through the generosity of our corporate partners, which include Bayer, United Therapeutics, Accredo and Acceleron Pharma.

In addition to the virtual town halls, PHA offers opportunities to interact through virtual support groups. As stay-at-home orders became the norm, PHA trained support group leaders to conduct meetings via telephone or video conference. It also provided resources and support to ease the transition from in-person meetings. A schedule of virtual support group meetings is at PHAssociation.org/ PHA-Connects.

Another way to connect with others is through closed PHA Facebook groups for parents, teens, caregivers and individuals with PH. To connect with another patient or caregiver or learn more about Facebook groups, visit PHAssociation.org/support or call 800-748-7274.

PHA also offers opportunities to connect during the pandemic through social media. Our #FlauntYourMask campaign encourages people to share pictures of themselves wearing and making fabric masks. Our #PHunatHome campaign shares opportunities for free escapism through links to parks, museums, aquariums and other organizations with online programming and web cams. We also have a #PhoreverThankful campaign to support our health care professionals on the front lines of the pandemic.

Discover the resources you need at PHAssociation.org/COVID-19.

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ADVOCACY IN ACTION

ADVOCACY IN ACTION

‘If You Don’t, Who Will?’

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very August, U.S. senators and representatives leave Washington, D.C., to spend the month in their home states. They meet with constituents to find out which issues matter to the people they represent. People affected by pulmonary hypertension (PH) can make a nationwide impact during August by sharing their stories with members of Congress.

When elected officials know the story of someone in their community affected by PH, they are more likely to support laws that benefit everyone with the disease. As the advocates below demonstrated, even someone with no previous advocacy experience can help improve lives for those with PH, just by sharing their stories.

‘I Made a Difference for Nevada’ Nicole Hogan-Williams spoke with congressional offices last summer about the impact of PH on her life. She visited staff members from the offices of Rep. Susie Lee and Sen. Jacky Rosen, both Nevada Democrats. Rosen became one of the first cosponsors of S. 2546, the Safe Step Act. This important bill aims to protect people with serious health conditions from having to try the least-expensive therapies first if their physicians think more expensive therapy would be more effective. “[The staff person in Congresswoman Lee’s office] was very easy to talk to and made me feel comfortable — like I was meeting up with an old friend,” Nicole says. “I told her how devastated I was at first and how PH affected my life. I have a feeling I made a difference for Nevada and also for our local support group.

In Rosen’s office, staffer April Lalone hadn’t heard of PH. “[She] was totally unaware of the diagnosis, symptoms and life expectancy of this disease we live with every day … I am just so proud of myself for speaking up and being heard on behalf of pulmonary hypertension community.”

Nicole presented her facts on a global, local and personal scale.

“I was a bit nervous, but … I realized ... they actually do care about me, and people who have this disease. They want to help, and that in itself made me feel so good.”

‘I Realized I Have a Voice’ Thekla McGinely made her first congressional visit to the office of Rep. French Hill, R-Arizona.

“I was so nervous that I would forget what I wanted to say, but once I started talking, that changed. I was able to tell my story and talk about how important it is for PH patients to get proper care. The legislative staff person I met with had lots of great questions and was very engaged in trying to understand what life with PH is really like.”

Thekla urges others affected by PH to use Pulmonary Hypertension Association (PHA) resources to schedule meetings with members of Congress. Congressional schedules fill up fast, so now is the right time to schedule an August meeting, she emphasizes. “If you are nervous to take the first step, take a friend. The more of a voice we have, the more understanding everyone else will have. We just have to be heard.”

Holly Szmutko was diagnosed with PH in January 2019 after several misdiagnoses. Even after receiving an accurate diagnosis, Holly tried multiple therapy options before finding a combination that made her feel better instead of worse. Despite those challenges, Holly began meeting with her members of Congress within months of her diagnosis to advocate for herself and be a voice for others.

Holly considers her meeting with Rep. Peter Visclosky, D-Indiana the highlight of her advocacy efforts. As a result of her meeting, Visclosky cosponsored the Safe Step Act in the House of Representatives. “Members of Congress work for us,” she says. “It never hurts to ask. If you don’t do it, who will? It is rewarding to fight for yourself and to fight for others.”

Ready to talk with your U.S. senators or representative? PHA staff can: • Request a virtual meeting on your behalf.

• Coach you while you plan and practice what you want to say.

• Send you materials to leave behind after your meeting.

Get started at PHAssociation.wufoo.com/forms/advocacy-august-request or call 301-5653004 x749.

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of PHA’s New Treatment Access Resources

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he Pulmonary Hypertension Association (PHA) has overhauled the Insurance and Treatment Access section on PHAssociation.org. The Treatment Access section, previously known as the Online Insurance Guide, has been reorganized for easier navigation. The updated content includes: • • • •

Financial assistance resources.

Questions to consider when choosing coverage.

How to troubleshoot insurance problems.

Your oxygen therapy rights.

Everyone with pulmonary hypertension (PH) should have prompt, affordable access to treatment prescribed by their PH specialist. Visit

PHAssociation.org/Patients/Insurance to put PHA’s new treatment access resources to work for you.

No internet? No problem! Contact PHA’s Treatment Access Program at 301-565-3004 x749 with your treatment access questions. PHA staff will work to connect you with the resources you need.

Thank you to Actelion Pharmaceuticals U.S. Inc. for supporting PHA’s Treatment Access Program.

SPEAK OUT In Advocacy August To Improve Life For People with PH

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ur friends from across the pond might have said it best: “Pulmonary hypertension doesn’t care which country you live in. We’re all in this together.”

That inspirational message came in the form of a tweet from the Pulmonary Hypertension Association UK (PHA UK). The organization joined thousands of people throughout the world May 5 to mark World PH Day. This year’s events largely were virtual because of the COVID-19 pandemic, so most people used the #WorldPHDay2020 hashtag on social media.

They shared symptoms, questions and information about the frequently misdiagnosed disease, as well as photos that celebrated life with PH. The #WorldPHDay2020 hashtag had reached nearly 52,000 people through social media interactions at press time.

Here in the U.S., the Pulmonary Hypertension Association (PHA), which manages the World PH Day website, reached more than 34,500 people through its social media posts. WorldPHDay.org received nearly 2,400 page views on May 5. More than 100 U.S. news outlets helped spread the word, reaching a potential audience of more than 20 million people.

Some people used other means to connect on World PH Day. Jack Bartky, an amateur radio enthusiast from New Jersey, created a special event day to raise PH awareness. Over slightly more than 24 hours, Jack and his wife Jessica, who has PH, heard from about 400 amateur radio operators from 20 countries. Their related website has attracted 1,700 visitors from more than 30 countries.

“The on-air response to our event was great, with nearly every operator asking questions about PH and thanking us for what we were doing,” Jack says.

PHA will: • Help you schedule virtual meetings with members of Congress.

• Provide materials for you to share with congressional staff.

• Plan what to say.

Members of Congress work for us. If you don’t [ask for their support], who will? It is rewarding to fight for yourself and to fight for others.”

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Legislative schedules fill up fast.

Complete this form to start your Advocacy August visit request.

PHAssociation.wufoo.com/ forms/advocacy-august-request

Jack Bartky PHASSOCIATION.ORG

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A Patient and Nurse Practitioner Connection Allison Dsouza and Elizabeth Colglazier like to say they “grew up together in PH.” Allison, a 22-year-old nursing student at the University of California-Los Angeles (UCLA), was diagnosed with pulmonary hypertension (PH) as a senior in high school.

Elizabeth is a pediatric nurse practitioner at UCSF Benioff Children’s Hospital, where she specializes in PH. This is the story of their PH journey.

A

llison Dsouza had an “ah-ha” moment in high school biology class when her teacher said some people with heart murmurs had difficulty walking. Allison, who had become progressively short of breath since the eighth grade, could barely walk to her car or go horseback riding.

She mentioned the teacher’s comment to her parents, who took her to see her pediatrician.

Her pediatrician ran some lab tests, which revealed a high red blood cell count. He recommended further testing, including a CT scan. The scan showed that her heart was enlarged, so she underwent an echocardiogram the next day.

The cardiologist said, “If it’s what I think it is, you need to go to UCSF. And you need to go right

away.” The next morning, after giving a presentation in English class, she went to Benioff Children’s Hospital, part of University of California-San Francisco Medical Center (UCSF).

Nine Days in ICU The following day, Allison had a cardiac catheterization, which confirmed PH and nearly caused a heart attack. She also met Elizabeth, who then specialized in pediatric critical care, in the ICU. “She was poring over her textbooks and made it clear that she was going to fight PH and go to college,” Elizabeth recalls.

Allison’s PH was one of the worst cases her medical team had ever seen, Elizabeth says. Jeffrey Fineman, M.D., who directs Benioff’s pediatric pulmonary hypertension program, started triple therapy to open her pulmonary arteries: subcutaneously delivered Remodulin, tadalafil and ambrisentan.

Elizabeth Colglazier, Allison Dsouza and Jeffrey Finneman, M.D., chief of pediatric critical care, Benioff Children’s Hospital, University of California-San Francisco Medical Center.

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Discovering a Role Model As Allison applied to colleges and took Advanced Placement classes, Elizabeth took over her health care. Eventually, Elizabeth helped her transition to college and an adult PH specialist. “It was more of mentoring role,” Elizabeth says. “You don’t have that with every patient.”

After high school, Allison attended California Polytechnic State University-San Luis Obispo, where she studied biological sciences. She thought she wanted to go to medical school until the summer she shadowed Elizabeth on her job.

Instead, she decided to get a nursing degree at University of California, Los Angeles.

The biggest compliment a patient can give a health care professional is to say, “I want to do what you do professionally,” Elizabeth says. So she was flattered when Allison told her she planned to go to nursing school.

“She will be an incredible nurse and nurse practitioner,” Elizabeth says. “Parents and patients love to meet Allison because they can see that their children can go to college and live normal lives.

She is an inspiration to many.”

Allison and Elizabeth had planned to share their story at the Journeys Luncheon at PHA 2020 PH International Conference and Scientific Sessions.

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the cost and stopped feeling sorry for myself, the easier everything became.

Almost immediately after signing with the agency, we found a match with someone who lived about an hour and a half away. We were excited because sometimes it can take several months, or the surrogate lives in a different state, requiring travel and more logistical planning. The agency facilitated the first phone call, and we later met her in person.

She was perfect. I couldn’t believe how lucky I was.

MY

Surrogacy options

MIRACLE BABY Skye Ellison of Huntsville, Alabama, has been active in the pulmonary hypertension community since she was diagnosed at 11 years old. Her artwork was featured on the Pulmonary Hypertension Association’s holiday cards about 20 years ago. Today, she balances the demands of parenting with her career as program manager for a medical device company.

By Skye Ellison

I

will never forget the day when I found out my husband and I were going to have a baby. The fertility clinic called, and our surrogate’s pregnancy test had come back positive. After almost a lifetime of hearing, “You won’t be able to have children,” it felt like I had just achieved the impossible. I didn’t think I would ever experience such a magical feeling, until nine months later when I held my daughter in my arms.

As most pulmonary hypertension (PH) patients know, pregnancy isn’t an option without taking on life-threatening risks. In spite of improvements in PH treatments, the mortality rate remains very high for both infant and mother during pregnancy.

I was diagnosed with Group 1 pulmonary arterial hypertension (PAH) at 11 years old (I am 32 now).

I had the standard symptoms, such as shortness of breath, chest pain and dizziness. This was a very scary time, especially for my family, as my original prognosis was not good. After seeing a specialist and undergoing several tests, including a right heart catheterization (RHC), I was given calcium channel blockers (CCB) as treatment. I consider myself

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one of the very lucky patients who are long-term responders to CCBs. Seventeen years later, I got married and decided to start a family. I knew surrogacy was an option and wanted to start the process, but I had no idea what I was getting into. During my adolescence, my PH doctors had mentioned gestational surrogacy, where another woman carries the child in her womb.

Starting the process After researching surrogacy agencies, we found one within driving distance. Generally, you don’t need an agency if you know someone willing to be a surrogate. But in our case, we needed the agency to match us with one. After a consultation that went very well, we anxiously signed on the dotted line and tried not to dwell on how much money we’d be spending.

I had been saving for this since my first job at 16, preparing for it to cost about $100,000. It felt unfair to have to spend so much, especially when my peers made having kids look so easy. The sooner I accepted

After talking more with our agency and doing more research, I learned about many options for surrogacy.

If you want your child to be biologically related to you and the father, you must undergo in-vitro fertilization (IVF) to harvest eggs, and the intended father must do a sperm collection.

If IVF isn’t an option for you (it might not be for some PH patients), you can consider alternatives.

One option is using a donor egg paired with the intended father’s sperm to create an embryo, then implanting it in a gestational surrogate. Alternatively, a traditional surrogate could be used, where the surrogate mother is also the biological mother of the child. Her egg is fertilized using sperm from the intended father. Adoption and foster parenting are other options to consider.

These decisions are personal and not easy to make.

I chose IVF and gestational surrogacy because I wanted a child that was biologically mine. However, there were times my husband and I looked at other options more closely when we ran into obstacles.

However, I was still considered too high risk to undergo IVF. I was surprised and discouraged.

Despite several phone calls, emails and visits to my PH doctor, I couldn’t get clearance. The major risk seemed to be fluid retention as a side effect of increased estrogen levels, but I was not in heart failure.

My doctors’ biggest concern was the lack of data on how IVF can affect patients with PH. No specific guidelines exist to assist doctors in determining who is too high risk for IVF with PH, and providers must rely on their clinical judgment, which usually means erring on the side of caution. It was disheartening to learn the same community that had encouraged surrogacy for most of my life was telling me they wouldn’t support me now that I was ready.

Changing direction I started doing my own research. I closely assessed my specific case, including symptoms, medications, test results, lab results, etc. I couldn’t find a reason significant enough to stop pursuing IVF. I realized I needed to find a PH doctor who could help me. I did some digging online and found Rana Awdish, M.D., director of pulmonary hypertension at Henry Ford Hospital in Detroit, Michigan.

Consulting your medical team When I was ready to begin IVF, I set up a consultation with a fertility practice that was a part of the same health care organization as my PH specialist. The fertility specialists were concerned about how IVF would affect my health because of PH. I discussed those concerns in more detail with my PH doctor and followed the doctor’s recommendation to undergo an RHC to get an upto-date status on my heart function. I was so relieved: I had good cardiac output and no signs of heart failure. I thought I surely would be cleared for IVF treatments.

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Egg extraction Two days later I arrived at the hospital for the egg extraction. While this usually occurs at a fertility clinic, mine was in the operating room because I was high risk. First, I received an IV in a patient room.

After listening to me complain extensively about how easy men have it, my husband went upstairs to do a sperm collection, which also was time sensitive.

In the OR, I was given medication to put me in a deep sedation, or “twilight state.” I don’t remember anything after that, only waking up and wondering whether I should ask the nurse if I said anything embarrassing while I was under the drugs. I decided I would rather not know.

I stayed in the recovery room for a few hours before going home. I was pretty sedentary the next few days, mostly because of soreness and discomfort. I started to feel normal again about two weeks later.

Statistics and success Although I lived in Alabama, I decided the travel would be worth finding a doctor who supported my long-term goals. I contacted her office, and she agreed to see me. She evaluated my case and determined IVF was doable for me, given my latest RHC results and history of clinical stability. Because my right ventricle wasn’t enlarged and worked well, as well as having low pulmonary vascular resistance, she told me IVF would be a safe option for me. I found another fertility clinic near me and was able to continue with a clearance letter from Dr. Awdish. I realized how lucky I was to have found a doctor who evaluated my case in its entirety and context.

Fortunately, our surrogate had waited several months for us to be ready and I eagerly went to the second fertility clinic to begin the IVF treatments and harvest eggs.

Needling concerns To grow the eggs to the right size, one undergoes hormone injections in the belly tissue, typically over eight to 14 days. I’m not a fan of needles, so that was intimidating. Although it wasn’t very painful, the biggest challenge was overcoming the mental part of sticking myself.

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The second week was the most difficult physically.

My ovaries felt like they were the size of grapefruits, and I had a lot of bloating (I thought I looked six months pregnant). It almost felt like I had an unnatural tumor growing in my abdomen. I don’t remember any other significant side effects from the hormones, other than my husband telling me I was a lot moodier than usual. That makes sense given that my estrogen levels were almost 50 times higher than usual, so I get a pass on that one!

I went to the fertility clinic daily for ultrasounds to monitor the size of the egg follicles. Once they got to the right size, I needed another injection, this time submuscular (in the upper/outer quadrant of the butt) to start the ovulation process. My husband had to give me the shot because of the angle and force required to inject it. The shot had to be exactly 36 hours before the egg extraction procedure, which ended up at 2 a.m.

We set our alarms about an hour prior to mentally prepare, which included re-watching instructional YouTube videos and reminding ourselves why we were doing this. I remember blasting music during the injection so I could take my mind off the fact that my husband was about to use my glute as a dart board for a 21-gauge needle. It ended up not being that bad!

Over the next five days, we learned how many eggs had fertilized and become embryos. We ended up with six embryos that had made it to the frozen

state. At first, I wasn’t very happy with that number considering how many eggs were retrieved, but I learned that was pretty good statistically. Sadly, some patients don’t get any embryos to freeze.

We chose to do genetic testing on our embryos, which turned our six embryos into only two “normal” embryos. In hindsight, I wouldn’t have done the genetic testing unless there was a specific gene to test for, or if I were over 35. I highly encourage anybody considering IVF to research genetic testing.

Embryo transfer The next step was transferring the two embryos to our surrogate. Before the transfer procedure, she had to undergo hormone therapy, which can be administered by suppositories or injections. The transfer is done via catheter by taking the embryo and implanting it into the uterus. The actual procedure is pretty quick and painless, and no sedation is required. I went with our surrogate to the procedures for support. Seeing her go through it made me realize how truly blessed I was that somebody was willing to do this for me. It was so brave of her. I questioned whether I was worthy of such compassion. To know there are such people in the world made me realize I needed to raise the bar for myself.

Ultimately, it took two IVF cycles and three transfer procedures with our surrogate to achieve a pregnancy. There were a lot of highs and lows; it definitely was an emotional roller coaster. The first two transfers didn’t work and required a second IVF cycle. With the second IVF cycle, I only yielded one “transferable” embryo, which was a lot less than I expected.

A lot of the highs and lows were made even more emotional by not fully understanding the statistics and chance of success with IVF — it is a game of odds. There were times I doubted it would work, and we were going to keep spending money and taxing my body for nothing. Looking back, I realize that my husband and I were pretty lucky that we got a pregnancy as quickly as we did. That’s not always the case. Often, it can take more rounds of IVF and transfers before a pregnancy is achieved.

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Sharing my story There are so many things I wish I had known before starting this journey, and I hope PH patients considering IVF and surrogacy can learn from my experiences. I’ve listed some recommendations below: • Discuss IVF as soon as possible with your PH doctor to understand if it is a viable option and how it might affect your health. Ask whether he/she has assisted a PH patient through surrogacy before.

• Harvest eggs as early as possible in your life. Go through the IVF process before you sign with an agency or match with a surrogate. Frozen embryos have a shelf life of almost 13 years. I wish I had started this earlier in my 20s, even though I wasn’t ready to have children. You can create as many embryos as possible while you are young and transfer them to a surrogate when you’re ready. After age 35, the fertility clinics view patients as “high risk” because of age and less favorable outcomes. The sooner you harvest eggs, the better your chances.

• Understand the statistics of IVF and the low chance of success of each treatment. It isn’t guaranteed to work on the first, second or even third attempt! This can be draining emotionally, physically and financially.

• IVF and surrogacy processes are expensive.

Decide how much you can or want to invest in the process. It’s rare, but some insurance plans might cover parts of fertility treatments and surrogacy (mine did not). If you know you want to pursue this, start saving — early, early, early! Undergoing gestational surrogate with an agency and IVF can cost over $100,000.

Knowing someone willing to be a surrogate can drop these fees considerably, but I recommend using a surrogacy attorney so there’s a contract to fall back on.

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The most valuable lesson I learned is to drive your own health care. You know your body the best, better than any doctor or expert because you are physically living in it. Be fully engaged in your health care management plan. Understand your test results, lab results, etc. Ask questions and be proactive instead of waiting to be told what to do. I would encourage every patient (PH or not) to have the same mindset.

If I hadn’t advocated for myself throughout this process, it is likely that our daughter never would have been born.

I feel blessed that my husband and I were able to have our daughter. I didn’t fully understand the definition of a miracle until we had her because that is what she is. She makes all our “struggles” seem so insignificant because she is perfect in every way. We are so grateful for all the people who helped get her here and supported us on our journey. We are in complete awe of our surrogate who was willing to carry a child that wasn’t her own, the most selfless act I can think of. We hope we can teach our daughter the overwhelming amount of courage, compassion and strength it took to bring her into this world.

Lindsay, Jason, Hunter and Hailey

PH and the Paths to Parenthood

W

hen Kellie and Paul Tasto walked down the aisle in 2007, they were already thinking about adoption. Three months before their wedding, Kellie learned that she had pulmonary hypertension (PH). She was critically ill with severe right heart failure, and she was devasted to learn that PH would affect their plans to start a family.

“I knew there was a greater risk of me or the baby not making it through a pregnancy,” Kellie recalls.

“That just wasn’t a risk we were willing to take.”

Carrying a child places increased stress on the heart and lungs. In women without PH, blood volume increases about 50%. For women with PH, the change in blood volume can lead to right-heart failure in PH patients whose right hearts are already overworked from the increased pressure in their pulmonary arteries. Some medications prescribed to PH patients also can be harmful to a developing fetus.

Many women with PH pursue adoption after consulting their PH physicians about family planning. Some couples go through private agencies,

like Kellie and Paul. Others, like Lindsay and Jason Howe of Peoria, Arizona, become foster parents before adopting.

“Don’t let PH stop you from following your dreams and your heart’s desire,” Kellie says. “We ended up with the greatest blessing of our life, adopting our daughter.”

Saved by ‘grace’ Kellie, 41, was diagnosed with PH when she was 28. She and Paul adopted their daughter Grace when she was an infant. The process took about two years.

Soon after they completed their application, Kellie’s mom also was diagnosed with PH. Today, her mother still faces PH challenges, but she enjoys time with Grace and four other grandchildren.

“They’re definitely the light in her days; they keep her pushing ahead,” Kellie says.

“After we adopted and had Grace in our lives, it gave life so much purpose. It gave us so much joy. It made everything worth it.”

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PH and the placement process Kellie and Paul began researching adoption agencies four years after they were married. They chose an agency in the Minneapolis-St. Paul area after weighing several considerations. Determining factors included length of the placement process, percentage of successful placements and cost.

Initially, Kellie was concerned her PH might prevent her from adopting. The screening process requires physical exams for prospective parents, with a doctor’s clearance. Kellie’s family and PH doctors wrote notes to confirm that she was in good health and her PH had stabilized.

When social workers interviewed them during a home visit, they asked a few questions about PH to ensure Kellie was healthy enough to care for a child and had a good support system. In the questionnaire she filled out for birth mothers, she indicated that her lung condition prompted her to choose adoption.

“For someone with PH, it’s a lot to take on the responsibility of being a mom,” says Kellie, who says she is well and busy raising a 7-yearold. “Fortunately, I’ve had only a couple of hospitalizations since.”

Kellie, Grace and Kellie’s mother Kellie and Grace

Like Kellie, she needed a letter from her physician stating that she was well enough to become a foster/ adoptive parent. Her physicians from the UCLA Medical Center and her care team in Arizona supported her parenting ability.

Parenting with PH

Raising Arizonans Lindsay and Jason Howe’s two-year journey to adopt biological siblings Hailey and Hunter began about four years ago when they moved to Arizona from California.

They applied for a license to become foster and adoptive parents to give them more options. The eight-month process included training classes, interviews and home inspections.

Lindsay, 37, worried the licensing agency, the Department of Child Services and the court might view her PH negatively. “I always feared that my licensing agency would Google and see the common prognosis as ‘terminal.’ While I have had PH for over 17 years, I knew those statistics were not entirely accurate, and I didn’t want them to assume my prognosis would be what is listed on an internet search.”

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Lindsay, Hailey, Hunter and Jason

Lindsay and Hailey

In her paperwork, Lindsay described the support network to help her parent with PH: her husband, in-laws, parents, medical team, friends and neighbors. After they were approved and licensed, Lindsay’s PH didn’t come up again. “Licensing agencies work with families with all kinds of disabilities and limitations every day, and it’s best to be honest and forthright about your condition and your capabilities.”

“Foster care and adoption can be very mentally, physically and emotionally tolling for anyone, healthy or not,” Lindsay says. “It’s important that you have resources set up before you start any of these processes.”

Be realistic about what you can and can’t handle, and plan your resources for help in the short and long term, Lindsay advises. Be honest with everyone about how your PH affects your daily life, and express fears, concerns, stressors to your loved ones and your medical team. That way, they can help you throughout the entire process.

“Make sure your spouse or partner knows that when you have a ‘PH day’ that you will need some extra support,” she says. “Don’t be afraid to ask a family member or medical professional for some extra emotional support or physical help.”

Within three days of being licensed, Lindsay and Jason received a foster placement of an 18-monthold girl and her infant brother, who was born

“Don’t let PH stop you from following your dreams and your heart’s desire.” prematurely at about 30 weeks gestation. After a year of court proceedings and psychological assessments, they petitioned to adopt the children. The adoptions were finalized six months later.

“Every family-building avenue comes with its own type of stress … While it is stressful, the dream of holding a child you get to call yours can be accomplished.”

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Congratulations, You’re a Caregiver.

You’ve Got This

Caregiver Roles • • • • • • •

Confidant Emergency manager Medication helper Diet watcher Cook Cleaning person Shopper

• Chauffeur • Appointment keeper • Reporter (responsible for updating friends and family) • Listener

What makes a good caregiver?

We asked some longtime caregivers for tips to be a great caregiver.

Here is what they said:

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f someone you love has pulmonary hypertension (PH), and he or she relies on you for support, then you are a caregiver. Welcome. You are a part of the Pulmonary Hypertension Association (PHA) community.

With the COVID-19 pandemic, family members may be suddenly thrust into the caregiving role to grocery shop, pick up prescriptions and do other tasks outside the home so those with PH can avoid risk.

Many new caregivers have questions, and there are many people whose caregiving role has greatly expanded with the arrival of the coronavirus. PHA is here to help you find the answers.

What is a caregiver?

In the PH community, caregiver – or care partner or carer – refers to a family member or friend who helps or supports someone living with PH. Outside of the PH community, those terms often refer to paid professional helpers. Within the PH community, they usually do not.

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“My husband Michael often tells me that PH is harder on me than it is on him,” says Lisa HagenLaughlin. “I disagree. PH is hard on both of us.”

Anyone can be a caregiver, and many people have more than one. Often, caregivers are immediate family: spouses, children, parents, siblings. But some caregivers are friends, roommates or neighbors.

Some caregivers don’t use that term because they already have a title: mom, dad, sister, brother, husband, wife. “I didn’t refer to myself as ‘caregiver’ because I’m her husband,” says Rick Peacy, whose wife Deloris passed away from PH in 2019.

What do caregivers do?

The best answer is another question: What does the “PHer” in your life need? A PH patient with mobility issues might need help with errands, but someone else might need support at medical appointments.

Jane Northrup accompanies her daughter Nicole to doctor’s visits. “everyone should have someone with them,” she says. “Four ears are better than two.”

• Listen to your patient. PH is invisible, so the best way to know what’s going on is to ask your loved one and believe what he or she tells you.

• Learn about PH. Educate yourself as much as possible about PH. • Check PHAssociation.org for credible, up-to-date information.

• Take time for yourself. Many caregivers have jobs and other responsibilities in addition to supporting their loved ones.

Managing so much can be stressful, so find ways to continue doing things you enjoy and take breaks when needed. Depending on your health, the health of your loved one, your relationship, insurance and other factors, you might be eligible for paid temporary relief called respite care. Learn more at www.ARCHrespite.org.

• Connect with other caregivers. PHA support groups, which now are offered virtually as long as social distancing is recommended, are great places to meet other caregivers. PHA hosts a monthly telephone support group and a Facebook group for caregivers. Caregivers are active and welcome members of the PHA community.

Caregiving During a Pandemic While being a caregiver can be stressful under the best of circumstances, the COVID-19 pandemic has intensified caregivers’ stress.

Those who live with PH patients have additional responsibilities to prevent infection in the home.

Many restrict visitors, keep cleaning supplies at the front door and wear face coverings outside the home. Some immediately wash their clothes when they get home, in addition to frequently washing their hands and cleaning their living spaces.

Caregivers who live apart from patients face the stress of not being able to be physically near their loved ones, which is also difficult for those separated from those who fall ill.

Learn more about caregiving, connecting to other caregivers and PH at PHAssociation.org/caregivers.

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Through Loss

By Nancy Jordan

W

hen my husband James Jordan started experiencing symptoms of pulmonary hypertension (PH), we had no idea how sick he was. A few years after we retired to Florida from Michigan, Jim began experiencing shortness of breath. He had difficulty doing everyday tasks – even his beloved game of golf became too much for him.

He originally was diagnosed with COPD but Jim was a very active person who loved to garden, wasn’t getting better with treatment. At one of his tinker with things in the garage, play golf and pulmonary rehab sessions, a technician suggested we travel. His PH slowed him down, but it didn’t stop visit the Cleveland Clinic for a second opinion. him. He had a positive outlook and tried to live his We decided to return to Michigan for the summer life as normally as possible. He used to say, “I have and make an appointment with the Cleveland Clinic, pulmonary hypertension; it doesn’t have me!” a Pulmonary Hypertension Association (PHA)I began to take on more routine tasks that I never accredited care center. Throughout that time, Jim’s thought about before. Our daughters lived nearby, condition worsened. and we had lots of love and About six weeks after returning support from them. Our to Michigan, we finally had our “I became committed five grandchildren visited appointment at the Cleveland us frequently. Our son Clinic. What we thought was to speaking about PH lived in California, but Jim going to be a few days turned because so many people always looked forward to into an entire month of tests – his calls. We felt blessed and most of them in the intensive don’t know what it is.” hopeful. Unfortunately, the care unit. The doctors at strain was too much for Jim’s Cleveland Clinic completed a heart. He never received the lungs we had hoped and right heart catherization and diagnosed Jim with PH. prayed for.

Jim then spent weeks in the ICU to get him strong My husband passed away September 17, 2009, of enough to go through a double lung transplant. We complications from PH, at age 63. moved back to Michigan and began our routine of Before Jim passed away, I subscribed to PHA News doctors’ visits to Cleveland. In March 2009, Jim to keep us informed about medical advances and PH was approved for a transplant and placed on the research. I appreciated learning more about PH and transplant list. The waiting began. We were scared feeling like we weren’t alone in our struggle. After we and apprehensive about what our future held, but we lost Jim, my family and I began to look for ways to had hope.

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get involved with PHA and raise awareness for this often-misdiagnosed disease.

I became committed to speaking about PH because so many people don’t know what it is. Last year, I paid for a vanity license plate from Michigan that reads “CUREPH.” Many people ask me what it means.

Another way we decided to get involved was to participate in local PHA fundraising events. We learned about PHA fundraising walks from a PHA email, and we have participated in the PHA Detroit O2breathe Walk since it began in 2014. We lead the team “Jim’s PHAngels” every year in memory of Jim. Last year, I shared my story with the Detroit PH community during the walk. I am always honored to

share my story – working to make people aware is a cause very near and dear to my family.

I know there have been many strides in the past 10 years to find answers, new medications and treatments for this insidious disease. We are so grateful that PHA is working toward finding a cure to help people like Jim. That’s why it’s so important to raise money to support PHA. It takes money to fund research and PHA’s programming. By participating in the PHA Detroit O2breathe Walk, my family, friends and I help take strides toward finding a cure.

Unfortunately, it’s too late for my husband, but there is always hope.

Team Jim’s PHAngels at the 2019 Detroit O2breathe Walk.

Nancy Jordan’s Vanity Plate.

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A Gift to Help Others

By Kelley Skumautz

M

y family’s journey with the Pulmonary time, I held out hope that once her PH stabilized, Hypertension Association (PHA) began she would see her family grow and thrive and we all almost 10 years ago, when my sister Mallory would have many more years together.

Hicks was diagnosed with pulmonary hypertension Sadly, my chill and funky sister (my nickname for (PH). She had been experiencing symptoms for a her is Funky Mal) passed away Jan. 3, 2011, mere while and faced misdiagnosis after misdiagnosis. We months after her diagnosis. Though her disease were all glad when we finally had a name for the brought challenge after challenge, she always showed exhaustion and fatigue that kept her from “normal” positivity and grace. Mallory faced PH first and living and parenting. foremost with the assurance that life had given her Right away Mallory’s care team connected her to all she ever wanted – her boys, husband and home PHA’s support group near her home in Minnesota.

– and she didn’t want anyone to feel sorry for her She joined the PHA online community and met situation. patients across the country. It was wonderful to hear My family and I searched for something we could her talk about the people she do to honor Mallory and call communicated with — young attention to this rare disease. mothers like herself, patients We settled on organizing “I have seen first-hand in with her exact diagnosis and what has become several my work and in my family people who had been living community walks in her with PH for many years. She the importance of person- memory. We organized the found wisdom, compassion first walk in June – around to-person support.” and understanding while her birthday – the year she our family found vital died. information and resources We are a small family, and through PHA. PHA was a buoy for all of us during since we had come to love the patients, caregivers, difficult and turbulent times. doctors and others who experienced the peculiarities When Mallory was diagnosed, I was living in of this disease, we invited them all to attend. It was Southern California and had just started a new job. I a moving celebration of her life, and over the next made it a priority to get home to Minnesota as soon few years we raised thousands of dollars for PHA at as I could muster some time off. Mallory had gotten annual community events. very sick in a short period of time. It was a shock In my career, I’ve been fortunate to work to see how difficult moving and breathing were for several advocacy and community-driven for her when I finally got to see her in December. organizations. I have seen first-hand in my work and Coincidentally, we had the fifth largest snowstorm in my family the importance of person-to-person on record in a century when I was there; we were support. A strong bond forms over sharing common required to stay put once I got to her house. At the experiences and I have such reverence for that.

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PHA’s resources and support groups were so important to Mallory and my family throughout her illness. That’s why a few years ago I joined PHA’s Legacy of Hope Society and included PHA in my estate plans. I want families like ours to be able to rely on PHA’s programs long into the future. I think of it as giving back to an organization that meant so much to each of us and helped us connect when we felt we had nowhere else to turn.

I spoke with my lawyer when I was updating my plans, and we decided it would be best leave PHA as a beneficiary in my will. Though I continue to support PHA with charitable donations, the gift I am planning to leave PHA through my estate is so special and important to me. I know that I am giving a gift of hope and helping others who will come after me.

Five years ago, I moved back to Minnesota to live closer to the rest of my family and see my nephew grow into the smart, gifted 15-year-old he is now. Next year will mark 10 years without having Mallory to share life with. My mom, Mal’s best friend and I are planning another commemorative event. We look forward to reflecting on where journeys within and outside of the PH community have led. We believe there are many miles and smiles to go when remembering Mallory and being there for others who might benefit from how we’re sharing and staying connected.

Kelley Skumautz lives in Minneapolis, Minnesota, with her husband. She has worked for environmental and community-based nonprofits throughout her career.

To join the Legacy of Hope Society or learn more, contact PHA at (240) 485-0755 or giving@PHAssociation.org.

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Please Note: Events may be canceled or rescheduled as needed in response to the COVID-19 pandemic and public health regulations.

JULY

SEPTEMBER

Paddle the Gallup PHor PH Thursday, July 16 Ann Arbor, Michigan Janet McCallister janetmcallister@msn.com

Annual Steps for Stripes Walk Saturday, Sept. 12 Brookfield, Wisconsin Kristen Rooney Klr9773@yahoo.com

St. Louis Area Trivia Night Friday, July 24 Maryland Heights, Missouri Mira Kruger MiraK@PHAssociation.org

Detroit O2breathe Walk Saturday, Sept. 12 Shelby Charter Township, Michigan Azalea Candelaria AzaleaC@PHAssociation.org

AUGUST Sarah Smiles PHun-Raiser for PH Sunday, Aug. 16 Camillus, New York Michelle Peek mpeeker@verizion.net Colorado PH in the Park Sunday, Aug. 23 Denver Angie Gonzales ang.gonzales711@gmail.com

Puttin PHore Poots Scramble Saturday, Sept. 19 Bloomington, Minnesota Cindy Warnberg puttinphorepoots@comcast. net Philadelphia O2breathe Walk Saturday, Sept. 26 Philadelphia Mira Kruger MiraK@PHAssociation.org OCTOBER

PHun Walk4Hay Sunday, Aug. 30 St. Louis Nicole Phillips phunwalk4hay@yahoo.com

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sMiles for Katie Friday, Oct. 2 Middletown, New Jersey Deb McCarthy smilesforkatie@verizon.net

Kansas City O2breathe Walk Saturday, Oct. 3 Kansas City, Missouri Azalea Candelaria AzaleaC@PHAssociation.org Annual Georgia Fun Walk Saturday, Oct. 10 Marietta, Georgia Mary Felkel PHAGeorgiaFunWalk@gmail. com Baltimore O2breathe Walk Saturday, Oct. 10 Baltimore Mira Kruger MiraK@PHAssociation.org Cleveland O2breathe Walk Saturday, Oct. 10 Cleveland Mira Kruger MiraK@PHAssociation.org Banding For a Cure Sunday, Oct. 18 Wind Gap, Pennsylvania Rhonda Cramer rhonda.gonzalez106@gmail. com

Greater Louisville O2breathe Fun Walk Saturday, Oct. 24 Louisville, Kentucky Azalea Candelaria AzaleaC@PHAssociation.org Southern California O2breathe Walk Saturday, Oct. 31 Fountain Valley, California Azalea Candelaria AzaleaC@PHAssociation.org NOVEMBER RunJonahRun Saturday, Nov. 14 Murfreesboro, Tennessee.

Sheri Guider guiderr@bellsouth.net ZebraPHest 5K and PHun Walk Saturday, Nov. 14 Dallas Mira Kruger MiraK@PHAssociation.org Phight for Life Walk and Fundraiser Sunday, Nov. 15 Rochester, N.Y. Jenn Garigen Jenngarigen@gmail.com

Upcoming Fundraisers Central California O2breathe Walk Fall 2020 Fresno, Calif.

Events@PHAssociation.org

Community Fundraising Events National O2breathe Events

Driving Fore a Cure Fall 2020 Raleigh, N.C. Matt Wall Mattwall67@gmail.com Pulmonary Pints Fall 2020 Baltimore Katy Hayes Katyehayes@gmail.com Monrovia PHun Run/ Walk Fall 2020 Monrovia, Calif.

Events@PHAssociation.org

Contact PHA at 240-485-3810 with questions about any events. For more information, visit PHAssociation.org/ Events.

South Florida Fun Walk Fall 2020 Pompano Beach, Fla. Pat Hellyer Pathellyer79@gmail.com

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PHA’s Legacy of Hope 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:

Oregon A recent Central Oregon PHA Support Group meeting focused on preparing for medical crises. Members discussed keeping an extra supply of medication and documents to keep handy, such as the most recent summary from your doctor that lists your medications. The group discussed what to do if you’re hospitalized, what medicine and equipment to take to the hospital, and the importance of informing your primary care practitioner and your specialists.

They also discussed what to do in the ER and how to let emergency room practitioners know about your medical history and medications.

Leaders: Michele and Leslie Freeman, mfreeman2000@gmail.com

Michigan The Lansing, Michigan, PHA Support Group recently invited Dr. Majid Mughal to speak about living with PH, CTEPH and stress. He discussed how to identify stress, feelings, behaviors and physical effects. He offered ideas for dealing with stress, such as journaling, painting, needlework, coloring books and learning a new language. He also said caregivers need to take time for themselves to deal with their stress.

Leaders: Karen Sawyer, Luluidapple@gmail.com

Utah Every February for Valentine’s Day, the Salt Lake City Zebras Support Group created “I ‘Lung’ You” cards for loved ones and support teams, including physicians. “We want them to know how much we appreciate them, and this is a cute and silly way to do that,” says leader Heidi Krum. The group uses glitter, pipe cleaners, stickers, pom poms, pictures of lungs and silly cartoons. They also include business card-size information about pulmonary hypertension (PH) and resources from PHA. “It’s the meeting I most look forward to,” Heidi says.

Leaders: Heidi Krum, CTEPHeidi@gmail.com

New Jersey The Feb. 8 Somerset County Support Group meeting brought together PH patients, family members and caregivers to discuss how pulmonary hypertension (PH) has affected their lives. Leaders described the meeting as an expression of experience, love and hope in the face of odds that sometimes seem insurmountable.

Leaders: Patrick Mellin, Patrick.Mellin@gmail.com Cheryl Farmer, cyfarmer.fightph@gmail.com

Since March, PHA support group leaders have been meeting remotely to connect and support the PH community. Find a virtual meeting by visiting PHA’s support group calendar at PHAssociation.org/ supportgroups/support-group-calendar.

The great work of these leaders is made possible in part thanks to generous contributions to the PHA Peer Support program from Actelion Pharmaceuticals U.S. Inc. 34

PATHLIGHT // ISSUE 2

Laura* and Rino Aldrighetti Alice A. Arnott* Sandra A. Awood* Dauna L. Bauer* Sylvia M. Becherer* Joan F. Bennett-Schenecker* Kris L. Best Gloria G. Blodgett* Dorothy E. Bradley* R. J. Braun Roberta F. Browning* and Lee A. Broadbent Rita and Bruce Brundage Colleen Brunetti Colleen and Shawn Connor Jane P.* and Harold P. Cooper James F. Corbett* Nicole M. Creech Laura H. D’Anna Charles W. DeVier, III* Linda M. Feibel* Barbara T. Gamer Stacey Gausling* Franklin D. Gillespie* Tammy A.* and Dean S. Hazen Mary Jan Hicks Carl Hicks, Jr. Phyllis M. Hill* Jackie Holt Richard L. Horrocks Stanley T. Jusinski Laura J. Kelly* Jessie Kohler-Wenninger Terri L. Kopp* Debbie L. and Mitchell Koppelman Dee* and Walter Kruger* Gloria J. Lang* Marie and Ronald J. Levendoski Mary Jo* and Thomas Linnen* Sally Maddox* Bonnie and Michael D. McGoon Joseph W. Mihuc* Linda Miles* Karen S. Moody Larry D. Moody Pamela R. Morris Marjorie D. Mott* Joyce L. Mowrer* Dorothy M.* and Harry J. Olson* Rita and Guy Orth Patricia R. and Gerald D. Paton* Cynthia and John R. Pickles Jean D. Pitcher* Carol L. Powell* Frances A. Price Carol J. Posner* and Marc Priore Diane Ramirez James Ryan* Louise C. and Gene P.* Salvucci Judith and Edwin L. Simpson Kelley Skumautz Marcia and Jack Stibbs Helena M. Strauch* Douglas R. Taylor Frank A. Tobac* Martha and Carlos Torres Deborah J. and Roger K. Towle Carol B. Ungar Daniel R. Walsh* Andrea and Stephen L. White * deceased members

Passages

S

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

Beverly Alessandrini

Vicki L. Kennedy

Paulette Sayko

Jewel A. Arntsen

Mary Ann Key

Kathleen A. Schoenrock

Boann W. Atz

Billie J. Kincade

Gary Slimmon

Mary Bandyke

Xavier C. Lee

Elizabeth A. Smith

William L. Belke

Arlene Lespier

Jack Smith

Linda Bermes

Rosalie F. Lillie

Walker A. Smith

Joseph Brandolo

Jerry Long

Mary R. Snyder

Tobi L. Carter

Edythe M. MacMurdo

Martha Steere

Elena Culqui

Lois J. Manee

Sharon Stucker

Bessie Damon

Antonio McGlothin

Colleen Termini

Terri Devellevez

Connie Mikuski

Chuck Thompson

Faith M. Dulaney

Alberta E. Mumph

Paula A. Warren

Hilary Dyson Teachout

Cristina Nates

Rebecca J. Wright

Betty Jane Euler

Thomas W. Paxton

James D. Yarbrough

Patricia R. Feeney

Tom Petitt

Victoria Freeman

Teressa M. Powers

Stacey M. Gausling

Emily E. Pugsley

Ellen J. Henderson

Tonya Redding

John M. Huff

Jessica Robertsmoore

Jan Kane

Jacey Sargent

William Keisler

Mary M. Savoy

The accuracy of this list is very important to us. Please contact the PHA office at 301-565-3004 x746 or Passages@PHAssociation.org to have a recently deceased loved one’s name listed or to 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.

PHASSOCIATION.ORG

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Pulmonary Hypertension Association 8401 Colesville Road, Suite 200 Silver Spring, MD 20910

The Pulmonary Hypertension Association’s (PHA’s) health and wellness series, PHA Connects: COVID-19 and Your Health, educates and supports our pulmonary hypertension (PH) community during the COVID-19 pandemic. PHA Connects provides patient education, live interviews and online events that matter to your health and wellness today.

PHA is grateful to the generous sponsors of PHA Connects, including Bayer, Accredo, United Therapeutics and Acceleron Pharma.

You can learn more about our new program and sign up for emails at PHAssociation.org/ PHA-Connects.