Pathlight - Winter 2021

A publication of the Pulmonary Hypertension Association

2021 // Volume 30 // No. 4

A TRUE CHAMPION’S FIGHT TO SURVIVE

PLUS Hope and Inspiration AMONG PEERS

PH, Pandemic and Anxiety?

TRY A HOBBY TO HELP YOU COPE

3 TIPS

TO ENSURE SEAMLESS TREATMENT ACCESS IN 2022

Life With PAH Can Be Challenging.

Learning About PAH Shouldn’t Be. Join a free, educational webinar in the comfort and safety of your home

IN THIS ISSUE 2021 // VOLUME 30 NO. 4

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Share Your PH Story or Research at PHA 2022

8

Feeling ‘So Alive’ with New Outlook

10 Finding Hope and Inspiration Among Peers

The Navigating PAH Pathways program offers free webinars created specifically for patients and caregivers, all led by a PAH Nurse and sponsored by Janssen.* 7

12 PH, Pandemic and Anxiety?

Try a Hobby to Help You Cope 16 Dialing Back Decision-Making

12

18 A True Champion’s Fight to Survive 25 450+ Clinicians Attend PHA’s First Online Symposium

Find Answers

Learn Why

Get Talking

26 Find a Virtual Support Group

10

28 Meet PHA’s New Advocacy Committee

Hear an educational Understand more about the Discover how to have more presentation from a PAH effects of PAH, the 3 key informed conversations nurse on topics that may be treatment pathways, and about PAH with your care Knowledge.

Understanding. Empowerment. important to you. treatment goals. community.

30 3 Tips To Ensure Seamless Treatment Access Next Year

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

ON OUR COVER

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.

32 Learning that Life Doesn’t Stop With Diagnosis

Cindy Champion was 18 when she contracted PH related to meth use. She’s been clean 17 years and now makes every day count. This month’s theme is “Living With PH.”

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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 Actelion, a Janssen Pharmaceutical Company, and must present information in compliance with FDA requirements applicable to Actelion, a Janssen Pharmaceutical Company.

IN EVERY ISSUE 5

Letter From the Chair

31 Quick Takes 31 Calendar This program is sponsored by Janssen Pharmaceuticals, Inc. ©2021 Actelion Pharmaceuticals US, Inc. All rights reserved. cp-201358v1 0121

Knowledge. Understanding. Empowerment.

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35 Passages

PHASSOCIATION.ORG 3

30 YEARS OF PHA

30 YEARS OF PHA

PUBLISHER

BOARD OF TRUSTEES

Matt J. Granato President & CEO

Colleen Brunetti, M.Ed., C.H.C., chair Tony Lahnston, chair-elect/treasurer Karen A. Fagan, M.D., immediate past chair Nicole Creech, secretary Matt J. Granato, LL.M., MBA, President and CEO, ex officio

STAFF Linda Busche Editor-in-Chief Karen Smaalders Vice President, Communications and Marketing

Trustees-at-Large

TEL: 301-565-3004

Cheri Abbott, R.N., CCRP Frank Cann Murali M. Chakinala, M.D., FCCP Colleen Connor Ramona Doyle, M.D., M.Sc. Anna R. Hemnes, M.D. Kimberly Jackson, R.N., B.S.N. William Jansen Mitch Koppelman, Ph.D. Michelle Ferdinand Liu, M.D., M.P.H., FAAOA Ronald J. Oudiz, M.D. Monica M. Penaranda Diane Ramirez Traci Stewart, R.N., M.S.N., CHFN Doug Taylor Matt Wall Jessie Kohler Wenninger, Esq. Melisa A. Wilson, D.N.P., APRN, ACNP-BC

FAX: 301-565-3994

Emeriti ex-officio

WEB: PHAssociation.org

C. Gregory Elliott, M.D., FCCP, MACP Michael D. McGoon, M.D. Edwin Simpson Judith Simpson, R.N., Ed.S.

CONTRIBUTORS Seker Anderson, Cindy Champion, Kathryn Downey, Mesha Guilloty, Tonya Hall, Noel Holly, Lauren Intrieri, Joyce Kossman, Mira Kruger, Zan Laughlin, Bill O’Donnell, Marco Reinoso, Karen Sawyer, Abby Sickles, Marie Smith, Jaeger Spratt, Melissa White, Ava Zechiel. Design: Mary Beth Ramsey.

CONTACT 8401 Colesville Road, Suite 200 Silver Spring, MD 20910

EMAIL: PHA@PHAssociation.org

SUPPORT LINE

SCIENTIFIC LEADERSHIP COUNCIL Murali M. Chakinala, M.D., FCCP, chair Anna R. Hemnes, M.D., chair-elect Ronald J. Oudiz, M.D., immediate past chair Steven H. Abman, M.D. William R. Auger, M.D. Eric D. Austin, M.D. MSCI Sonja Bartolome, M.D. Todd M. Bull, M.D. Kelly Chin, M.D. Vinicio A. de Jesus Perez, M.D., FCCP, FAHA, ATSF 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. Kristin B. Highland, M.D., MSCR Steven Kawut, M.D., M.S. Tim Lahm, M.D. Deborah J. Levine, M.D. Lana D. Melendres-Groves, M.D. John J. Ryan, M.D., M.B., B.Ch., B.A.O. Jeffrey S. Sager, M.D., MSCE Oksana A. Shlobin, M.D., FCCP Thenappan Thenappan, M.D. Corey E. Ventetuolo, M.D., M.S. R. James White, M.D., Ph.D. Timothy L. Williamson, M.D. Delphine Yung, M.D. Liaisons Cheri Abbott, R.N., CCRP Kimberly Jackson, R.N., B.S.N.

800-748-7274

Honorary

Distinguished Advisors

We’re here to support anyone with a connection to PH. (daytime, please)

Carl Hicks

David B. Badesch, M.D. Erika S. Berman Rosenzweig, 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.

President & CEO Matt J. Granato, LL.M., MBA

Submissions to Pathlight are welcome.

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

4 PATHLIGHT ISSUE 4

PH PROFESSIONAL NETWORK EXECUTIVE COMMITTEE Cheri Abbott, R.N., CCRP, chair Kimberly Jackson, R.N., B.S.N., chairelect Fran Rogers, M.S.N., CRNP, immediate past chair Loida A. Johnson, CRNP Tisha Kivett, R.N., B.S.N. Melissa Magness, M.S.N., APRN, CNP-AC Susie McDevitt, M.S.N., ACNP-BC

LETTER from the

CHAIR A

s Pathlight goes to press, I will have hit the 14-year milestone of living with pulmonary hypertension (PH). That fact gives me both pause and a reason to celebrate. I remember those early days of diagnosis with crystal clarity. I mostly focused on what if I didn’t make it or how much would I suffer. While those fears are normal, they aren’t healthy to dwell on.

As I began to adapt to this “new normal,” my outlook slowly began to shift. I quickly learned that I couldn’t do much about having PH, besides taking my medication and listening to my doctor. However, I had full control of my mindset and outlook; the way I chose to feed my body; and my reaction to having PH. In time, I chose to live my diagnosis out loud, always acknowledging the hard parts. I also did everything I could to channel this incredibly hard situation into something good. That meant good for my family and my mental health, and doing all I could to be an advocate and voice for my PH and rare disease community.

When you get a diagnosis like PH, your world stops for a while. If you’re newly diagnosed, know that whatever you’re feeling is normal. But also know that there is hope, as well as stories of triumph and beauty in life.

This month’s Pathlight explores inspiring stories of living — truly living — with PH. I’m so excited to share them with you: Our cover story relates the story of a true Champion.

Literally. Seventeen years ago, Cindy Champion was dying.

But she was determined to overcome her meth addiction and improve her PH prognosis. Today, the Vista, California, resident focuses on making each day count.

In “Finding Hope and Inspiration Among Peers,” Mesha Guilloty relates her PH journey that she now realizes began in her teens. Mesha was born in 1991, the same year that four women formed what today is the Pulmonary

Hypertension Association (PHA). Mesha, who lives in North Carolina with her husband and daughter, represents Generation Hope, people 18 to 39 with PH. In “Feeling ‘So Alive’ With New Outlook,” Marie Smith relates how she let go of anger and self pity after after hearing a Goo Goo Dolls song. She didn’t want to waste the time she had left by being miserable.

This issue also explores how hobbies help people manage illness, anxiety and stress. Noel Holly of Orange County, California; Marco Reinoso of Tampa, Florida; Karen Sawyer of Okemos, Michigan; and Kathryn Downey of Calgary, Alberta, Canada; discuss their creative outlets, such as gardening, guitar-playing, painting and crocheting.

Our donor profile describes how Joyce Kossman learned that life doesn’t stop with a PH diagnosis. Joyce retired from her job as an elementary school principal in Connecticut after her pulmonary arterial hypertension diagnosis. Since then, she and her husband have moved to Virginia, where she works part-time at a medical school.

In our volunteer profile, Bill O’Donnell describes “Dialing Back Decision-Making” now that his daughter Shannon is an adult. Bill shares how his caregiving role has changed in the past 20 years.

As you can see, the PH community is rich with stories of hope and thriving, not just surviving, with PH. We hope these stories of living with PH bring you inspiration and peace. Thank you for reading!

Warmly,

Colleen Brunetti, M.Ed., C.H.C. Board of Trustees Chair, Pulmonary Hypertension Association

PHASSOCIATION.ORG 5

30 YEARS OF PHA

30 YEARS OF PHA

Share Your PH Story or Research at PHA 2022

T

he Pulmonary Hypertension Association is seeking speakers for PHA 2022 International PH Conference and Scientific Sessions.

People with PH, family members, caregivers and health care professionals can apply to present breakout sessions related to: • • • • • •

Pediatric/parenting issues.

New diagnosis.

Young adulthood.

Living with PH. CTEPH. Caregiver experiences/challenges.

We also are looking for Spanishlanguage presentations on similar topics.

The PHA 2022 planning committee will review and evaluate the applications based on the following criteria:

We strive to help PH patients thrive.

We work every day to help patients with pulmonary hypertension. It’s more than a mission—it’s our promise.

• Does the presentation fit the track and topic selected?

• Is the topic relevant to the community?

• Are the solutions presented new or innovative?

• Can the solutions presented be easily duplicated or implemented by the community?

To be considered, submit your application by Dec. 23 to PHAssociation.org/pha2022/ breakout-speakers.

SCIENTIFIC SESSIONS

BAYER and the Bayer Cross are registered trademarks of Bayer.

Bayer 100 Bayer Boulevard, Whippany, NJ 07981 USA ©2017 Bayer PP-400-US-3751 August 2017

Science For A Better Life

PHA also is accepting applications for abstract and symposia proposals

Photo: Kathleen Sheffer.

for the Scientific Sessions. Abstracts should report original basic or clinical research or interesting/informative cases. Encore abstracts are welcome as long as the work doesn’t infringe upon copyrights or proprietary or personal rights of any third party.

Proposals can cover research or clinical management topics in as solo presentations, debates or roundtable/ panels. All applicants must have PHA medical memberships.

MARK YOUR CALENDARS Online registration for PHA 2022 will open in February. PHA will contact selected speakers for breakouts and Scientific Sessions in January.

The Scientific Session Committee will review and rank submissions through a predetermined scoring rubric. Apply by Dec. 23 at PHAssociation.org/pha2022/ scientific-session-speakers.

PHASSOCIATION.ORG 7

30 YEARS OF PHA

30 YEARS OF PHA

Feeling ‘So Alive’ with New Outlook

‘From that point on, I decided to be nice to people, to face others with PH without getting freaked out and to embrace the disease and stop running from it.’

BY MARIE SMITH

I MARIE SMITH was a critical care nurse for 26 years and now works in radiology.

Before her pulmonary hypertension (PH) diagnosis, Marie was a singer and songwriter for 25 years.

Originally from Nova Scotia, Canada, she lives in Harrisburg, Pennsylvania, with her cats Aslan and Grace.

had just returned from completing the Rock ’n’ Roll Montreal half marathon in 2013. I had typical vague PH symptoms: puffy abdomen and ankles and sense of fullness. I realized later that I had some symptoms in 2007 when I started doing marathons and half marathons as a race walker. After those races, I felt full or had puffy ankles, but my teammates didn’t.

While driving on the Pennsylvania Turnpike to Philadelphia for a doctor appointment, I developed sharp pain in my back and groin. I called an ambulance and was taken to Paoli Hospital, where a CT scan showed a kidney stone.

A month later, I received my health records from my doctor and read my CT. Holy cow. It was nearly two pages long with only one line about the kidney stone.

My liver was enlarged. My portal vein, a blood vessel that moves from the spleen and gastrointestinal tract to the liver, had fluid around it. My vena cava, which carries deoxygenated blood into the heart, was dilated, as was my right atrium.

I freaked out. My doctor ordered an echocardiogram and consulted cardiology. My echo numbers were very high, and I had a bad tricuspid valve, one of the main valves on the right side of the heart.

After that, I had a right heart catheterization, and my numbers were in the high to normal range. That’s when I was officially diagnosed. It was Christmas Eve 2013.

SCARED TO DEATH

I will never forget the feeling when I read my first echocardiogram results. I am a critical care nurse, so I read these things. I was well aware of the outcomes for PH. I had cared for patients who were far into their disease. To say I was scared to death is putting it lightly. I was sick to my stomach.

8 PATHLIGHT ISSUE 4

I was told I might have five years to live even though my numbers weren’t that bad. I fell into a cycle of anger, fear, nastiness to other people and crying.

I turned to substance abuse and felt angry and sorry for myself. I made my life miserable.

In 2016, I was driving to Target when I heard a new song by the Goo Goo Dolls called “So Alive.” The lyrics were so powerful that I had to pull over.

It seemed to be about deciding to live.

I realized that I was wasting any time I had left by being miserable. I was still able bodied and could work and travel.

Why was I feeling so scared and angry?

From that point on, I decided to be nice to people, to face others with PH without getting freaked out and to embrace the disease and stop running from it. Just like that, I changed.

SO ALIVE

Every day is precious to me. I know that at any moment I could become disabled or lose my ability to go for

walks. There is no room for fear and negativity.

I also became a diehard Goo Goo Dolls fan. Although I have trouble dancing and singing along, I have been to six of their concerts! If I were still stuck in anger and sadness, I would have missed all that.

After my “2016 epiphany,” I got up the nerve to join PH support groups rather than run away from them. I belong to three, including a PHA Facebook support group.

I am out with an injury right now, but I like to hike even if I am the slowest in the group and the person who struggles most. I like to go to Orange Theory, a gym that has intense cardio sessions. My cardiologist doesn’t want my heart rate over 150. I am relegated to the pool right now, but I have to be mindful that holding my breath could increase my pulmonary pressures.

I still get pangs of fear when I have to sit on the curb after taking

the garbage out or I need to catch my breath after walking into work from the parking lot. But I don’t indulge them.

Life is good in some way no matter what is going on, and I make the best of it.

DOES MARIE’S STORY resonate with you? Inspire and give hope to the PH community by sharing your PH experience.

PHAssociation.org/the-right-heart

PHASSOCIATION.ORG 9

30 YEARS OF PHA

30 YEARS OF PHA

Finding Hope and Inspiration Among Peers

BALANCING FRUSTRATION AND DETERMINATION

BY MESHA GUILLOTY

MESHA GUILLOTY WAS BORN IN 1991, the same year that four women formed what today is the Pulmonary Hypertension Association (PHA). Mesha’s pulmonary hypertension (PH) symptoms began when she was 14, but she wasn’t diagnosed until nearly a decade later.

Mesha, who lives in North Carolina with her husband Nathan and daughter Mila, represents Generation Hope, people 18 to 39 with PH. She’s working on her nursing degree and expects to graduate in 2023.

I

remember my PH symptoms as if they happened yesterday. I was at home with my dad and siblings when my dad asked me to grab him a snack. I ran down the stairs, back up and handed him the snack. I was extremely winded, tired and dizzy.

I tried to make my way to the bathroom, and the next thing I remember, I woke up on the floor. I was confused and scared. I was unsure how I ended up there and couldn’t recall how long I had been there. That was the first of many times I would lose consciousness.

My PH diagnosis journey was a rollercoaster. I remember several trips to the emergency room, urgent care and ambulance rides after passing out. I was told many different things, and I felt frustrated to keep experiencing what I now know to be syncope episodes.

My medical tests all came back normal. I realize now that my symptoms were apparent during exercise or activities that caused my heart to work harder (such as carrying books/ groceries), but not when I was at rest.

It took me about eight years to be diagnosed with 10

idiopathic PH. My case is interesting to my providers because my identical twin sister doesn’t have PH.

Since being diagnosed, I try to find beauty in everything. I once was told that everyday tasks would become impossible for me to complete. I don’t know what I’m trying to do more: prove doctors wrong or show how good God is.

Maybe a little bit of both.

I’ve learned to stand up for my health and my decisions related to that. My team at Atrium Health has done an amazing job at helping me achieve that.

Since I’ve been diagnosed, so many drugs have developed to help people with their symptoms. One I’m taking now wasn’t around when I was first diagnosed. It has changed my life. The road to getting to the optimal dose was tough, but it was worth it.

STRENGTH IN NUMBERS

Sometimes, being a young person with a rare disease is overwhelming and frustrating. Other people my age don’t experience life the same way I do.

I connected with PHA’s Generation Hope group shortly after my diagnosis. My mom found the group, and I joined after that. So many amazing people share their stories of victory and tribulations with the disease. At times it can be humbling, and it can be a wakeup call that the disease is real and life-threatening. But I’ve learned resilience and not to feel sorry for myself.

FUTURE OF HOPE

I hope PH research can continue to grow. I think I speak for everyone when I say a cure would be the best outcome for all of us. I also would like to see more education for health care providers. So many of us are misdiagnosed for so long.

It’s critical that people be diagnosed as quickly as possible. It could be the difference between life and death.

I also want other young people to know that they aren’t alone, and they don’t have to be defined by this disease.

Every day is a fight, but it’s a good fight. I wish I would’ve known that It’s important to listen to your body, speak up when something doesn’t feel right and that it’s OK to fight for what you want.

Mesha Guilloty with her husband Nathan and their daughter Mila.

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30 YEARS OF PHA

30 YEARS OF PHA

PH, PANDEMIC AND

ANXIETY?

Try a Hobby to Help You Cope

In addition to gardening, Noël likes to can fruit and vegetables from the garden, including homemade tomato sauce. She also enjoys garden tours, scrapbooking and making cards, which aren't as physically demanding as gardening. While she enjoys all her hobbies for different reasons, Noël says gardening is the most rewarding.

“Gardening helps me slow down, listen to the birds and see the fruit of my efforts.”

DEVASTATING DIAGNOSIS

N

oël Holly has been an avid gardener for most of her adult life. She started growing flowers, then branched into vegetable gardening. She maintains a community garden that she started with her neighbors and makes sure to share the bounty.

After Noël was diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH) in May 2008, she didn’t garden much for a year and a half. It took time to adjust to her medication and lifestyle changes before she regained her energy.

“It was daunting at first to start up again,” says Noël, of Orange County, California. “The poor little plot at the community garden was overgrown with weeds, but thankfully several friends helped me tackle it.”

Noël is one of several people with pulmonary hypertension (PH) who shared how hobbies help them cope with illness, anxiety and stress. Others who discussed their creative outlets are Marco Reinoso of Tampa, Florida; Karen Sawyer of Okemos, Michigan; and Kathryn Downey of Calgary, Alberta, Canada.

BLOSSOMING INTEREST

‘Gardening helps me slow down, listen to the birds and see the fruit of my efforts.’

Gardening helps Noël cope with her CTEPH by giving her a creative outlet. She no longer does “power gardening” — planting, weeding and harvesting for three or four hours — instead, she goes more slowly, working one section at a time.

Noël loves to watch her roses bloom and share bouquets and vegetables with friends. She says gardening is a good way to exercise.

Similarly, Marco’s hobbies also helped him relax and regain interest in life.

Born and raised in Quito, Ecuador, Marco moved to El Salvador in 1991. Active in sports, he played soccer once or twice a week and went mountain biking every Saturday.

Eventually, his stamina started to change. He was getting tired sooner and had constant headaches.

In 2005, after almost passing out while playing soccer on the beach, Marco went to see his doctor. A right heart catheterization showed his pulmonary pressures were over 60. The doctor told Marco he no longer could exercise.

“I was completely and utterly devastated,” he says. “It was like they amputated my legs.”

NEW DETERMINATION

One afternoon, Marco saw his guitar collecting dust in the closet. He realized he now had time to learn to play.

Within three to four months, Marco could play the guitar with ease, thanks to determination and YouTube lessons. The first songs he learned were “Hotel California” and the “Sounds of Silence.” His advice? Find a song you love, and “keep on trying until you get it.”

The guitar became his refuge from his post-diagnosis depression.

“Guitar is definitely a great relaxing hobby,” he says. “It allows my mind to go places and travel down memory lane.”

Three years after his diagnosis, Marco’s health started

‘Guitar is definitely a great relaxing hobby.

It allows my mind to go places and travel down

memory lane.’ to decline. So he moved to Florida in 2011 for treatment unavailable in South America.

In Tampa, Marco started playing guitar in his church. In El Salvador, he had organized Christmas carols at the U.S. Embassy where he worked.

Marco still plays guitar and does aerobic exercise to stay active. He says the aerobic exercises help keep his heart rate steady, and he hopes to start mountain biking again soon.

Noël Holly relaxes in in the community garden she restored after adjusting to her PH diagnosis and medications.

Opposite page: Marco Reinoso plays guitar to relax.

12 PATHLIGHT ISSUE 4

PHASSOCIATION.ORG 13

30 YEARS OF PHA

30 YEARS OF PHA

SPARKING CREATIVITY

‘Watercolor has sparked that forgotten creativity I didn't know I had’

Karen Sawyer shows some of her watercolor creations, also pictured below.

Opposite page: Kathryn Downey dressed in PH purple in Calgary, Alberta.

14 PATHLIGHT ISSUE 4

Like Marco, Karen Sawyer learned a new skill after her 2003 PAH diagnosis. She took up painting to deal with loneliness, depression and difficult days.

She found watercolor painting in March 2018 after a friend started teaching a class. Before the class, Karen had never painted and didn’t know if she could paint. Now, every Monday, she goes to an art class with four friends and spends three hours painting and learning new techniques.

Before discovering painting, Karen says she sometimes would burst into tears about her life-threatening disease.

“Painting really helps me take my mind off the stress of having this disease,” she says. “I have something to look forward to doing all week.”

Karen likes working in watercolor because she says the colors flow and blend together better than other forms of painting. Karen took one acrylic class, but she says watercolor is more forgiving and allows her to build colors and erase mistakes more easily.

Karen says she feels proud of herself as she improves her technique and ability.

“Watercolor has sparked that forgotten creativity I didn't know I had,” says Karen, leader of the Lansing, Michigan, PHA Support Group since 2017. “It’s comforting, peaceful and sometimes challenging, but it teaches you to slow down and concentrate.”

FOCUSING AWAY FROM PH

Kathryn Downey had a hobby to turn to when she was diagnosed with idiopathic PAH in March 2020 soon after the start of the pandemic. After

the initial shock of her diagnosis, crocheting gave her something to focus on that wasn’t PH-related.

Kathryn started crocheting at 8 years old when her Auntie Mitzi also taught her cross-stitch and simple crafts. She got back into crochet about seven years ago after she was laid off from her job.

Losing her job caused high anxiety and depression that led to agoraphobia. The anxiety disorder, which causes people to avoid places that could cause panic, made Kathryn housebound for four years.

During her time at home, Kathryn opened an Etsy store and made Halloween-themed toques, or knitted caps.

CROCHETING FOR A CAUSE

During the pandemic, Kathryn started crocheting lung keychains. In each right lung, Kathryn includes a piece of paper with the name of someone with PH. Whoever buys the keychain sponsors someone with PH. The buyer becomes “the lungs” of the person with PH when hiking, camping, swimming and traveling. Kathryn donates a portion of her sales to PHA Canada.

Kathryn says it’s rewarding to educate people about PH while doing something she loves.

“It’s gotten me through some really hard times,” she says. “Crocheting allowed me to express my creative side and make things that people liked.”

‘It’s gotten me through some really hard times.

Crocheting allowed me to express my creative side and make things that people liked.’

PHASSOCIATION.ORG 15

30 YEARS OF PHA

30 YEARS OF PHA

Dialing Back Decision-Making

BY BILL O’DONNELL

Bill O’Donnell is a media specialist at Babson College.

He lives in Medford, Massachusetts, with his wife Laura and their daughter Shannon, who developed pulmonary hypertension (PH) as a child. He shares how his caregiving role has changed over the past 20 years.

M

y daughter, Shannon, was diagnosed 20 years ago with primary pulmonary hypertension, now called idiopathic pulmonary arterial hypertension (PAH). She was 6.

I will never forget how my heart sank and how tough it was for the doctor to explain the seriousness of the illness. Despite the severity of Shannon’s illness, Laura and I were determined that PH wouldn’t rule our world. We insisted Shannon participate in her kindergarten graduation five days later. A few days after the ceremony, she had her first right heart catheterization.

Given that Shannon was 6, her care fell completely on my wife and me. In a matter of a few weeks, Laura and I went from having little medical knowledge to feeling like we earned an M.D. After the heart catheterization, Shannon was put on Flolan, the only drug approved at the time to treat PH. Laura and I learned how to mix and manage Flolan, care for a central line, manage a long-term illness and work with medical professionals.

RELIABLE SOURCES

In 2001, the internet was not what it is today. Information on rare diseases was hard to come by. We relied on Shannon’s care team at Boston Children’s Hospital and

Early on, we discovered the best thing we could do was advocate for Shannon. Part of an advocate’s responsibilities means volunteering for organizations like PHA and Boston Children’s Hospital. If you want something done or want to change something, you sometimes need to pitch in. Amazingly, I get more than I give when I volunteer.

PRACTICING SELF-CARE

other health care services, such as specialty pharmacies.

We received a copy of a Pulmonary Hypertension Association (PHA) publication, “Pulmonary Hypertension: A Patient’s Survival Guide.” The book proved to be worth its weight in gold.

Credible information on PH was hard to find, and just like today, not everything online is true.

We joined PHA and attended a PHA support group meeting geared toward adults. With the help of a specialty pharmacy, we established a small pediatric support group in the Boston area.

Eventually we attended PHA’s International PH Conference and Scientific Sessions. We have presented at several PHA conferences since then.

Left: Shannon at Bunker Hill Community College graduation 2017. Above: the O’Donnells at Shannon’s kindergarten graduation. Opposite: Bill, Shannon and Laura at the Hole in the Wall Gang camp.

16 PATHLIGHT ISSUE 4

‘If you want something done or want to change something, you sometimes need to pitch in.’

Caregivers must remember to make time for themselves. That doesn’t mean always doing things alone. It means doing things you receive satisfaction from. However, you often will find me watching a movie or sports by in my man cave for a few hours on weekends.

A few years after Shannon was diagnosed, she became a camper at the Hole in the Wall Gang Camp. She had an excellent experience. As soon as the opportunity came our way, my wife and I volunteered. We may leave exhausted but always rejuvenated.

The camp is an amazing place. Its staff realize parents and caregivers need different kinds of healing and offers weekend getaways where parents can spend a weekend at a resort having fun.

For many parents, the getaways are the first time away from their sick children.

This was the case for my wife and me. Fortunately, we found a friend who was willing to learn everything, including mixing medication, to care for a child on Flolan. My wife

and I continue to volunteer at these weekends. We haven’t yet met parents of a child with PH, but we find it easy to relate to parents who care for children with other serious illnesses.

NEW ROLE

For me, the hardest thing over the past 20 years has been the transition from parent-caregiver to caregiver. When Shannon was diagnosed, she didn’t have a say on whether to be admitted to the hospital or attend kindergarten graduation. My wife and I made all of the decisions.

Today the decisions are all Shannon’s. All I can do is advise, which can be hard, and be there for her. On the plus side, she can do a lot on her own, like mix remodulin, although the

three of us still take turns. The most rewarding thing is watching Shannon advocate for herself and assisting with running PHA’s young adult online support group. We now volunteer for camp as a family.

I never considered myself a caregiver and find that title difficult to own. I am just a parent, who like all parents, just does whatever I can for my child.

As a parent/caregiver, I would advise other parents to find an organization you believe in and get involved. The rewards and experiences are sure to be worth more than the effort put in. As I have, you may find that being around people in similar situations is reward enough.

PHASSOCIATION.ORG 17

30 YEARS OF PHA

Cindy Champion, 54, has overcome a 2004 pulmonary hypertension (PH) diagnosis, thyroid cancer and meth addiction. Despite the hurdles thrown her way, the Vista, California, resident maintains a positive outlook on life. Cindy, 17 years clean, focuses on enjoying life and making each day count.

BY CINDY CHAMPION

A True CHAMPION’S FIGHT TO SURVIVE 18 PATHLIGHT ISSUE 4

O

ne day in 2004, my body started to quickly fill with fluid from head to toe.

My eyeballs were swollen from fluid going to my head. I looked like the girl from “Willy Wonka and the Chocolate Factory” who swells into a blueberry.

My friend Al wanted to take me to the hospital, but I wanted to get high one more time before I went. My friend Jennifer pulled up next to us in her car and was talking to Al. She didn’t even recognize me. When she realized it was me, she told me to get in her car, and they took me to the hospital.

The health care team took two liters of fluid from me within the first hour. I was dying, and my body was shutting down.

The doctors diagnosed me with primary pulmonary hypertension and gave me six months to live. I couldn’t walk, talk or breathe on my own. The doctors said I would never speak again due to the pressure on my heart and lungs, but that was the least of my worries. I felt empty and defeated.

PHASSOCIATION.ORG 19

30 YEARS OF PHA

30 YEARS OF PHA

so they could see the results of using meth and what it does to your body.

Being in the nursing home was hard. I was on morphine every day, and I had to wear headphones at night so I wouldn’t hear the screams of people dying. I would wake up and notice some people didn’t make it through the night.

My younger sister Cathy had to give me baths because I didn’t have the strength to do it myself. I didn’t want that for me. I was the youngest one there, and it scared me. That woke me up and made me want to live.

FINDING HOPE

SAYING GOODBYE

Top left: Cindy with her niece Amaryah Becker. Top right: Cindy during a hospital stay.

Bottom right: Cindy and her dog Rosco.

Opposite page: Cindy and her sister Cathy Petit.

20 PATHLIGHT ISSUE 4

My father had PH as well, but my meth use caused my heart and lung issues. I had used meth since I was 18.

Sometimes people use drugs to escape from reality. I had to deal with things in my life that I don’t wish on anyone, and meth offered me an escape from the things that were haunting me.

I was sent to a nursing home to spend my final months. It was the worst feeling to watch my loved ones struggle to say goodbye and keep it together without breaking down in front of me. I was OK with dying, but it was harder for me to see the fear in their eyes when they saw me. I told my friends to bring their kids to my room,

While I was in the nursing home, a nursing student told me she was diagnosed with PH when she was a little girl. She gave me all the information for her doctor, Nick Kim, M.D., from University of CaliforniaSan Diego Health, and I gave it to my sister Donna.

Donna fought for me and did everything in her power to get me an appointment with Dr. Kim. It was difficult to get insurance coverage and access to specialty medicines because I was dying. She worked her butt off and dealt with frustrating situations because she knew time was of the essence. My sister Donna is the reason I’m still here today.

When Dr. Kim did my first right heart catheterization, my pressures were over 100. He didn’t think I would make it, but he was willing to try. Dr. Kim and his nurse, Sandy Lombardi, R.N., considered whether I should undergo a heart or lung transplant, or if pills and IV therapy were the best forms of treatment. Luckily, Dr. Kim got me into a clinical trial for Tracleer.

‘I’m a strong girl, and I never give up. I fight as hard as I can, so I can represent my last name, Champion. That’s what I am. I will always put up a good fight.

I’m proud of how far I have come and if I can do it, anyone can.’

PHASSOCIATION.ORG 21 21

30 YEARS OF PHA

30 YEARS OF PHA

While I was on Tracleer, Cathy would take me for walks around the nursing home and sneak me into the physical therapy room. She would help me get out of my wheelchair with my oxygen tank and have me walk up three steps to strengthen me. I would walk a little bit more each time until I couldn’t. Then she would push me

around outside, so I could get fresh air and a change of scenery.

Despite the medication, I still didn’t know if I would live or die. My ex, Jimmy, took me into his home after six months and took care of me so I didn’t have to die in the nursing home. I will never forget what Jimmy did for me.

Little by little, I noticed that I was starting to improve. Each right heart catheterization showed that my pressures were going down. In 2006, I started showing even more improvement. I no longer needed oxygen, and I could walk up hills without an issue. I never went back to the nursing home.

I was on Tracleer for many years until I started having problems with my liver and kidney. I am now on Opsumit and sildenafil. My body has taken to the medication, and I have managed to come a long way from where I once was.

Some days were tough, but the love and support of my friends and family made me work harder to get better.

My mom, my grandma, and my sisters, Donna and Cathy, came to see me almost every day. The love and support of so many people made a difference.

Dr. Kim and Sandy Lombardi are my heroes and have helped me every step of the way. I owe my life to them, and I wouldn’t be here today if it weren’t for them. They fought for me, and I will do whatever it takes to make them proud and not waste their time.

FIGHT LIKE A CHAMPION

It has been a long road, and I fought every step of the way. It was a challenge not being able to talk. I lost my voice for almost two years. Then it came back with a vengeance, just like me.

22 PATHLIGHT ISSUE 4

‘It has been a long road, and I fought every step of the way. It was a challenge not being able to talk. I lost my voice for almost two years. Then it came back with a vengeance, just like me.’

I have other health conditions as well as PH. I was diagnosed with thyroid cancer in 2013 and had my thyroid removed. In 2016, it came back in my lymph nodes, which were removed as well.

I also have a pancreatic subdivision, which means I have two pancreatic ducts. If my main one gets backed up by the extra one, I can get very sick. I survived a motorcycle accident, and in 1992, I was shot in my left leg in a home invasion. I’m lucky to still have my leg.

I’m glad I got sick because it lit a fire under my butt to live a life without meth. Sometimes we take things for granted and don’t realize how precious life really is until it’s taken away from you. I made a promise to God that if I ever made it out of the nursing home alive, I would never do meth again.

I have kept my promise to this day. I have been clean 17 years.

Each day is a blessing, even if it’s a bad day. I’m still here to enjoy each day as it comes. I want others to know that they should never give up. I’m thankful for all the chances I’ve been given in life. Now I deal with the things that haunt me through counseling. It’s one of the best decisions I have ever made.

It doesn’t hurt to get help if you need it, and boy do I need it.

I’m a strong girl, and I never give up. I fight as hard as I can, so I can represent my last name, Champion.

That’s what I am. I will always put up a good fight. I’m proud of how far I have come and if I can do it, anyone can.

PHASSOCIATION.ORG 23

HEALTH MATTERS

HEALTH MATTERS

What Is the Relationship Between Meth and PH?

M

ethamphetamine is a highly addictive derivative of amphetamine, a stimulant used to treat attention-deficit disorder, narcolepsy and other disorders.

Ramona Doyle, M.D

Meth use has been associated with diseases of the coronary arteries, heart muscle, acute bleeding of the lungs, excess fluid in the lungs, collapsed lungs and PH. Like other stimulants, meth causes blood vessels to constrict, and become permanently obstructed, forcing the heart to work harder, says Vinicio A. de Jesus Perez, M.D., FCCP, FAHA, ATSF, of Stanford University Medical Center. Dr. de Jesus Perez presented a 2020 PHA webinar on meth and PH. Amphetamines are similar to diet pills like fenfluramine, or Fen-Phen. The anti-obesity drug was withdrawn from the market because of the potential link with of PH and heart valve problems.

Even people who casually used meth in the distant path are at risk of developing PH. If you have PH, it’s important to tell your health care provider if you use or have used meth. It can help doctors identify what they need to focus on so they can take better care of you.

Vinicio A. de Jesus Perez, M.D., FCCP, FAHA, ATSF

LEARN MORE ABOUT METH USE AND PH with these Pulmonary Hypertension Association (PHA) resources: STREAM OUR WEBINARS “Ask the Expert: PH and Meth,” presented in English by Ramona Doyle, M.D., of University of San Francisco Medial Center (PHAssociation.org/ask-the-expert-ph-and-meth) and by Dr. de Jesus Perez in Spanish (PHAssociation.org/portfolio-items/pregunta-al-expertosobrehp-y-meth).).

DOWNLOAD our free “PH & Methamphetamine Use” brochure at PHAassociation.org/pha-free-materials-store.

READ RESEARCH based on PHA Registry data, “Clinical Differences and Outcomes between Methamphetamine-Associated and Idiopathic Pulmonary Arterial Hypertension.” PHAssociation.org/learn-more-aboutthe-link-between-ph-and-meth.

450+ Clinicians

Attend PHA’s First Online Symposium

T

he Pulmonary Hypertension Association (PHA) welcomed more than 450 attendees to the PH Professional Network (PHPN) Symposium Sept.

30-Oct. 3. Symposium was entirely online for the first time because of the pandemic.

Health care professionals spent four days learning about the latest advancements in pulmonary hypertension (PH) research and care while earning continuing education credits. PH professionals shared their expertise and findings in informative sessions. The Poster Hall featured 24 posters about COVID-19 and pulmonary arterial hypertension, quality of life, treatment outcomes and more.

While many sessions focused on treatment, advancements and science, others offered a personalized care perspective. For example, Hilary DuBrock, M.D., a pulmonologist at Mayo Clinic in Rochester, Minnesota, discussed ways to improve the quality of life of patients in and out of the doctor’s office. When managing patients with PH, medical therapy is only one piece of the puzzle, she said.

“I was really struck by how many patients had impaired quality of life despite meeting their other PH treatment goals,” Dr. DuBrock said.

Improving a patient’s overall health ensures people can live their best possible lives with PH, she noted.

Another session focused on navigating difficult discussions. Rana Awdish, M.D., FACP, FCCP, shared tips to master sensitive conversations among care teams, patients and families. She highlighted the importance of listening to patients’ wants and concerns and responding to emotion with empathy.

“To be efficient is never the goal. The goal is to have understanding,” she said.

to discuss legislation that could improve the lives of people with PH. Participants urged support for the Safe Step Act, NIH research funding and preserving access to charitable assistance. PHA conducted 36 meetings with the offices of 21 senators and 15 representatives.

“As health care professionals who are constantly running into obstacles with laws and insurance with our patients and their medications, it’s vital to advocate in any way we can for our patients,” said Teya Thai, PH and heart failure case manager at Nebraska Medicine. “[Advocacy Day] is one way to make a huge impact in advocacy for our PH patients.”

TRIVIA NIGHT

Symposium attendees tested their general and PH knowledge Oct. 1 during virtual PHPN Trivia Night. All funds raised for Trivia Night benefit PHA’s Conference Scholarship Fund. Fifty-two Trivia Night registrants raised $1,300 to help PH patients and their caregivers attend PHA’s 2022 International PH Conference and Scientific Sessions in Atlanta.

HEALTH CARE PROVIDERS: You can still earn up to 24 CME/CMU credit by streaming PHPN Symposium sessions. Visit PHAssociation.org/Symposium by Dec. 20 to register and gain access to this valuable content. Sessions will be available online through Jan. 4, 2022.

ADVOCATES IN ACTION

This year’s event began Sept. 30 with Advocacy Day. Nearly 50 attendees from 25 states met virtually with lawmakers 24 PATHLIGHT ISSUE 4

PHASSOCIATION.ORG 25

HEALTH MATTERS

HEALTH MATTERS

CONNECT BY PHONE OR COMPUTER

Find a Virtual Support Group

P

atient support has been a significant part of the Pulmonary Hypertension Association (PHA) since it began 30 years ago.

In 1991, Shawn Wiggins organized the first pulmonary hypertension (PH) support group meeting at her Evanston, Illinois, home. A single mother of two children, Shawn worked part-time as a nurse. Her low-key, optimistic personality drew people to her. The Chicagoland PH Support Group met a second time at Ed and Judy Simpson’s home in DeKalb, Illinois, in October 1991. Eight people attended. The group still exists, with 26 regular members.

Nationwide, more than 4,000 people attend 700 PHA Support Group meetings, which have operated remotely since the beginning of the COVID-19 pandemic.

As the pandemic continues, PHA offers several support resources the PH community can access from home. Learn about our support resources below: PHA Support Line

PHA has support lines in English and Spanish. Support Line volunteers answer questions, lend a sympathetic ear or help guide you in solving problems or issues related to living with PH. Last year, volunteers fielded nearly 1,000 calls. Call the support line: (800) 748-7274.

Telephone Support Groups

Each of our seven telephone support groups meet monthly. The telephone groups connect PH community members with those of similar experience to exchange struggles and triumphs, support and advice. A PHA volunteer facilitates introductions and conversation. The groups are: • • • • • • • 26 PATHLIGHT ISSUE 4

Patients (fourth Thursday) Caregivers (third Wednesday) CTEPH patients/caregivers (third Tuesday) Bereaved community members (second Thursday) Parents of children with PH (second Wednesday) Young adults with PH (fourth Tuesday) Grupo de apoyo telefónico en español (fourth Wednesday)

To participate, you must register each month so the support group leader knows how many attendees to expect. Register at PHAssociation.org/support.

Virtual Support Group Meetings

PHA support groups offer knowledge, support and empowerment. A meeting might feature a guest speaker discussing a topic of interest, or it could simply be a chance for members to share experiences. Because these meetings take place remotely, you can attend from anywhere by phone or computer.

PHA Facebook Groups

PHA hosts eight private Facebook groups for various segments of the PH community. PHA staff administer the groups. Volunteers monitor the groups to make sure participants follow guidelines and ensure the groups are safe for sharing sensitive information. The Facebook groups are:

• • • • • •

PHA CTEPH (chronic thromboembolic PH) PHA Teens PHA Parents of Kids with PH PHA Caregivers PHA Newly Diagnosed PHA Email PHriends.

Email PHriends

Contact an experienced, trusted fellow patient or caregiver by email to get information, swap stories or connect. Email PHriends share what they’ve learned along their PH journeys, point you to information and resources, and work with you to develop strategies to cope with PH. Email PHriends can answer single questions or be available for three-month email relationships. Request an email PHriend at PHAssociation.org/emailphriends.

• PHA Long-Term Thrivers • PHA Generation Hope: Young Adults • PHA PH Plus: Associated Conditions

VOLUNTEER for one of PHA’s support programs.

The only skills you’ll need are compassion and a desire to help other people with PH, their families and caregivers. Sign up at PHAssociation.org/volunteer.

Get Our New Brochure: ‘PH & Left Heart Disease’ During PH Awareness Month, the Pulmonary Hypertension Association released a new brochure as part of its “PH &” series.

“PH & Left Heart Disease” addresses the most common cause of PH worldwide.

In patients with known left heart disease, 30% to 60% also have PH. The signs and symptoms of PH due to left heart disease overlap with those of heart disease.

Pulmonary Hypertension

& LEFT HEART DISEASE

LEARN MORE ABOUT SYMPTOMS, DIAGNOSIS AND TREATMENT. Download the free brochure at PHAssociation.org/pha-free-materials-store.

PHASSOCIATION.ORG 27

ADVOCACY

ADVOCACY

MEET PHA’S

New Advocacy Committee The Pulmonary Hypertension Association (PHA) formed an Advocacy Committee to advise and mentor people interested in advocating for the pulmonary hypertension (PH) community. Each member of the Advocacy Committee will work with people from specific geographic regions. PHA is finalizing committee members to represent the Southwest and New York/New Jersey.

Learn about some of the committee members, and the regions they represent: MICHELLE CHANCE, M.S.N., APRN-BC Florida

Michelle is a PH nurse practitioner at AdventHealth Orlando’s advanced lung disease program.

Michelle wants to create greater awareness about PH and showcase the determination and strength of people with PH. Michelle says one of the most rewarding aspects of her PH experience has been watching patients regain their quality of life. She looks forward to continued growth as an advocate and health care provider.

JOHNELL DIWAN, B.S.N., R.N. Oregon and Washington

Johnell, a pulmonary arterial hypertension (PAH) nurse since 2006, is the PAH nurses coordinator at Legacy Health in Portland, Oregon. A member of PHA since 2006, Johnell has attended many PHA conferences.

Her favorite advocacy memory is

28 PATHLIGHT ISSUE 4

lobbying for the Tom Lantos PH Research and Education Act during a previous PH Professional Network Symposium Advocacy Day. MARK DODSON, M.D., PH.D. Alaska, Colorado, Hawaii, Idaho, Montana, Nevada, Utah, Wyoming

Dr. Dodson became fascinated with PH when he was a medical student, which was about the time when effective PH therapies became available.

He saw how medications improved patients’ quality of life and knew that he wanted to pursue a career in PH. Dr. Dodson is the medical director of the chronic thromboembolic pulmonary hypertension program at Intermountain Medical Center, a PHA-accredited PH Care Center in Murray, Utah.

KAREN FRAGALE Iowa, Kansas, Missouri, Nebraska, North Dakota, South Dakota

Karen, of Johnson, Iowa, was diagnosed with PH in 2010.

She is a co-leader of the PHA Iowa

Support Group, a PH advocate and volunteer for PHA’s patient support line. Karen connected with PHA after she was diagnosed when she was looking for a support group. About five years ago, she began sharing her story with senior staffers for her U.S. senators.

Karen’s most memorable advocacy experience was when staff from Sens.

Joni Ernst and Charles Grassley‘s offices agreed to a joint meeting.

HEATHER MAYBERRY, M.S.N, RN-BC, C.N.S, ACNS-BC, CCRN, RCIS Pennsylvania, Delaware, Maryland, District of Columbia, Virginia, and West Virginia

A clinical nurse specialist and pulmonary hypertension clinical coordinator at Lynchburg General Hospital in Virginia, Heather was named nurse of the year in 2020 by the Virginia Nurses Association.

As clinical coordinator, Heather has helped develop PH policy, management protocols and educational opportunities.

MONICA PENARANDA California

Monica, of Hacienda Heights, California, was diagnosed with PH when she was 16 and soon joined a PHA support group. Twenty-five years later, she serves on the PHA board of directors, leads a PHA support

group, raises money for PH awareness and research, and works to educate lawmakers about PH. DIANE RAMIREZ Alabama, Arkansas, Georgia, Kentucky, Louisiana, Mississippi, North Carolina, South Carolina, Tennessee

Diane, of Lexington, North Carolina, was diagnosed with PAH in 1987. A few years later, her younger brother and sister also were diagnosed. Losing family members to PAH motivated Diane to get involved with PHA. Diane, a member of PHA’s Board of Trustees, has been involved with advocacy since 2006. Her goal is to help as many patients as possible. She has met with most of North Carolina's congressional delegation in their district offices and in Washington, D.C. Diane believes advocacy is a platform for the PH community’s voices to be heard. She feels empowered to know people with PH can speak up and make a difference.

She says PH patients must be tenacious to survive, which makes them powerful advocates.

HOLLY SZMUTKO Illinois, Indiana, Michigan, Minnesota, Ohio, Wisconsin

Holly, of Valparaiso, Indiana, was diagnosed with PAH in 2018. By late 2020, she was

so sick that she needed a double lung transplant, which she received on May 5, 2021, World PH Day. Holly, a PHA support group leader, believes no one should have to fight for medication, procedures, surgeries or other health care needs. Quality care should be accessible and reasonably priced, says Holly, who has guided several people toward help with health care challenges.

MARY WHITTENHALL, N.P. Connecticut, Maine, Massachusetts, New Hampshire, Rhode Island, Vermont

Mary, of North Attleboro, Mass., is program coordinator/nurse practitioner of the Rhode Island Hospital/Brown Medicine Pulmonary Hypertension Center, a PHA-accredited PH Care Center. Mary runs the patient support group affiliated with the center and educates her colleagues about PH. A member of PHPN since 2014, Mary has advocated for her patients in many situations where their voices weren’t strong enough to be heard or they didn’t feel comfortable speaking up.

EMAIL US at Advocacy@ PHAssociation.org to contact your regional Advocacy Committee representative.

Or call 301-565-3004 x758.

PHASSOCIATION.ORG 29

ADVOCACY

3 TIPS To Ensure Seamless Treatment Access Next Year

If your insurance coverage is changing in January 2022, take these steps to prevent delayed prescription refills, prior authorization renewals or other treatment needs.

1|

Update your PH care team.

Many health insurance plans require prior authorization or step therapy for PH medication. Your health care team might need to complete new paperwork or file an appeal to ensure seamless access to your therapy. Notify your health care team right away about insurance changes so they have time now to tackle potential obstacles and reduce the chances of a delay in the new year.

While you’re speaking with your health care team, consider requesting that your last refill of the year be a 60or 90- day refill. If you are eligible, a multi-month refill will further reduce your risk of a delay in January.

2|

Update your specialty pharmacy.

Health insurance plans work with specific specialty pharmacies to deliver PH therapy. If your health insurance plan changes, notify your specialty pharmacy. Check with your specialty pharmacy to see whether an insurance change will affect how you receive your PH therapy.

Share positive experiences or service challenges through PHA’s specialty pharmacy feedback form: PHAssociation.org/specialtypharmacy-feedback. PHA will send your comments to our contacts at your pharmacy.

3|

Review your financial assistance options.

Changes in your health insurance coverage sometimes affects co-pay assistance eligibility. Eligibility criteria varies with manufacturer assistance programs, nonprofit grants and government-funded support programs. Visit PHAssociation.org/ HELP or contact PHA’s Treatment Access Program for information.

Call 301-565-3004 x758 or email Insurance@PHAssociation.org.

PROBLEMS WITH OXYGEN ACCESS? Share Your Story If your medical equipment supplier no longer delivers liquid oxygen, or you’ve experienced a delay or barrier to accessing liquid oxygen treatment, let us know.

The Pulmonary Hypertension Association is working to bring the PH community’s voice to Congress and advocate for access to liquid oxygen.

Share your experience at advocacy@PHAssociation.org, or call 301-565-3004 x758.

QUICKTAKES PHA Registry Enrollment Tops 1,700 Participation in the Pulmonary Hypertension Association Registry recently surpassed a new milestone with more than 1,700 patients.

The registry represents data from newly diagnosed patients and newly treated within six months at more than 60 PHA-accredited Pulmonary Hypertension Care Centers.

Pathlight Wins Award for 30th Anniversary Issue

T

he Pulmonary Hypertension Association won a silver Trendy award from Association Trends. The Trendy awards recognize nonprofit organizations’ outstanding communications and marketing efforts.

PHA’s award was for the winter 2020 issue of Pathlight, which launched our 30th anniversary celebrations. The issue includes stories about PHA’s early days, long-time survivors, advances in PH research and a tribute to founder Pat Paton, who died in July 2020.

NOW AVAILABLE

Advances in Pulmonary Hypertension, Issue 4

T

he latest issue of Advances in Pulmonary Hypertension addresses PAH and interstitial lung disease.

Volume 20, Issue 4, includes articles on identification, evaluation and monitoring; connective tissue and interstitial lung diseases; infants with brochopulmonary dysplasia and PH; and more. Advances, the Pulmonary Hypertension Association’s quarterly peer-reviewed clinical journal, is available for free at meridian.allenpress. com/aph.

CALENDAR OF EVENTS FEBRUARY 2022 Online registration and scholarship applications open for PHA 2022

OCTOBER 8, 2022 Georgia Fun Walk Marietta, GA PHAevents.org/Georgia

FEBRUARY 22, 2022 Tampa O₂ breathe 5K and Walk

PHA thanks its national fundraising-event sponsors for their generous support: DIAMOND

O₂breathe.org/Tampa

APRIL 9, 2022 Southern California O₂ breathe Walk Long Beach O₂breathe.org/SoCal

MAY 5, 2022 World Pulmonary Hypertension Day Online

JUNE 10-12, 2022

VIRTUAL FUNDRAISERS Visit PHAssociation.org/phafundraising-events to find a fundraising event in your area.

PLATINUM

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

SILVER

PHA 2022 International PH Conference and Scientific Sessions Atlanta 30 PATHLIGHT ISSUE 4

PHASSOCIATION.ORG 31

DONOR PROFILE

DONOR PROFILE

Learning that Life Doesn’t Stop With Diagnosis

BY JOYCE KOSSMAN

Joyce Kossman was an elementary school principal in Hamden, Connecticut, when she was diagnosed with pulmonary arterial hypertension (PAH). As her symptoms worsened, she retired and started treatment. Since then, she and her husband Jay moved to Norfolk, Virginia, where she works part time at a medical school.

A

n unexplained swollen ankle in 2009 eventually led to my PH diagnosis.

The swollen ankle caused my shoe to fit improperly, and I mentioned the pain at my annual physical. X-rays showed no damage to my ankle or foot, so my primary care doctor began to look for other reasons for the swelling. He ordered further testing, including a chest x-ray.

My doctor was concerned about the chest x-ray results. So he referred me to a pulmonologist, who sent me to a cardiologist. The cardiologist referred me to a pulmonologist who specialized in PH. Within six months of my physical, I was diagnosed with idiopathic PAH. Jay and I were stunned. We had never heard of PAH and had no idea what it meant. Since my April 2010 diagnosis, other symptoms appeared: I had extreme shortness of breath when talking, walking and doing daily activities.

After working all day, my legs felt like I was walking through quicksand, and I was completely fatigued.

My doctor gave me an outdated handbook on PAH, and it was quite distressing to see that treatment was invasive and concluded with a transplant.

My PH doctor reassured me that new treatments were available, including a nebulizer and pills, and many more options were available as needed. I started PH treatment in July 2010.

NAVIGATING A NEW FUTURE

Above: Jay and Joyce Kossman on a cruise to Italy.

Opposite page: (top right) Jay and Joyce vacationing in the Mediterranean; (bottom left) Joyce in a helicopter over Alaska.

32 PATHLIGHT ISSUE 4

We were scared for the future. We were in a crisis about how to manage our new life. Jay read an obituary in our local newspaper about someone who had died of PAH. Without telling me, he called the widower to see what to expect.

He called our friends and family to share the news without really knowing what to say. He became my coach, support system, cheerleader and researcher.

He has attended every pulmonologist and cardiologist appointment with me since my diagnosis.

We learned that life doesn’t have to stop with a PAH diagnosis. Since 2010, we have adjusted our travel habits and discovered cruise ships. Planning far

in advance is a necessity, as I must contact my health insurance company to override prescription refill dates to have enough medication to take on long trips.

I learned to navigate various airline requirements for portable oxygen concentrators with lithium batteries and get through Transportation Security Administration checkpoints with a nebulizer, spare lithium batteries and distilled water for the nebulizer.

Our most recent trip, before the pandemic, was to Japan for a 14-night cruise around the island.

FINDING COMMUNITY

When we moved back to Virginia in 2013, I was fortunate to find a PAH specialist who has helped me continue to manage my PAH. I attended pulmonary rehab classes to gain stamina and joined a local Pulmonary Hypertension Association (PHA) support group.

By listening and talking to members of the PHA support group, I discovered that many patients travel this journey without any support outside of the group. The PHA support group is like a family for many.

I enjoy sharing my successes, like how to find a portable oxygen concentrator light enough to carry without a cart and get through airports and air travel with equipment.

MANAGING PH

I have successfully managed my PAH by adhering to my doctor’s guidance and with a spouse who is with me for the ride.

I avoid being outside on hot, muggy days because of the dew point’s effect on my breathing. I watch out for wind chill and plan my days according to the weather forecast.

I enjoy my part-time work with a local medical school, where I help students refine their communication skills. I consider my work a way to show appreciation for the medical providers who have helped me thrive.

I would not be doing as well as I am today without the wonderful medical advancements PHA has supported

over the years. We still need a cure, and I know PHA is helping find one.

Jay and I donate to PHA to support research and awareness of this mostly unknown disease. Thanks to PHA, real progress has been made over the past 30 years to help PH patients like me manage daily life.

I look forward to more discoveries and treatment options as I continue my PAH journey alongside PHA. Every donation to PHA is a step in the right direction for those like me who have been diagnosed and those yet to be diagnosed.

HELP SUPPORT PHA’S MISSION to extend and improve the lives of those affected by PH. Visit PHAssociation.org/donate to make a gift.

PHASSOCIATION.ORG 33

Announcing ...

More Knowledge and Inspiration for People

Living With PAH PAH Initiative Ambassadors Karen, Lauren, and Denise

From the PAH Initiative, sponsored

by United Therapeutics

Nutrition and the impact of sodium on PAH

PHA’S LEGACY OF HOPE

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.

Laura* and Rino Aldrighetti Alice A. Arnott*

Marie and Ronald J. Levendoski

Sandra A. Awood*

Mary Jo* and Thomas Linnen*

Dauna L. Bauer*

Sally Maddox*

Sylvia M. Becherer*

Bonnie and Michael D. McGoon

Joan F. Bennett-Schenecker*

Rick Bartke Marsha Bettez Wanda Capo Sherryl B. Davis Michael L. Drap Wilda Ferrer Florence B. Fialk Joseph Florio Reginald Gamble Winifred Hitchen Billie Jo Jensen Julie H. Jones

Barbara King Marita Loffredo Walter Newton Michael R. Pascetta Claudia J. Pascetta Contillio Patricia Pilloni Nancy C. Sculthorp Loy P. Teague Avelino Torres Ryan H. Woodson Soukanth Xayarath

Kris L. Best

Joseph W. Mihuc* Linda Miles*

Gloria G. Blodgett*

Karen S. Moody

Dorothy E. Bradley*

Larry D. Moody

Mary M. Brady*

Pamela R. Morris

R. J. Braun

Marjorie D. Mott*

Roberta F. Browning* and Lee A. Broadbent

Joyce L. Mowrer* Dorothy M.* and Harry J. Olson*

Rita and Bruce Brundage Colleen Brunetti Colleen and Shawn Connor

Rita and Guy Orth

Jane P.* and Harold P. Cooper James F. Corbett*

Patricia R.* and Gerald D. Paton*

Nicole M. Creech

Cynthia and John R. Pickles

Laura H. D’Anna

Jean D. Pitcher*

Workouts designed for people with PAH

Charles W. DeVier, III*

Carol L. Powell*

Linda M. Feibel*

Frances A. Price

Barbara T. Gamer

Connecting with others

Stacey Gausling*

Carol J. Posner* and Marc Priore

Caregiver insights and resources

Tammy A.* and Dean S. Hazen

PAHInitiative.com is always being updated with helpful and encouraging information featuring perspectives and insights from PAH experts and patients like you.

Use your phone to scan here and visit PAHInitiative.com/living-with-PAH.

Sponsored by United Therapeutics Committed to Improving the Lives of Patients © 2021 United Therapeutics Corporation. All rights reserved. US/DS/0765

Diane Ramirez

Franklin D. Gillespie*

Emotional well-being

34 PATHLIGHT ISSUE 4

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:

Theresa “Terry” E. (Cavanaugh) O’Reilly*

The PAH Initiative is here to help with even more useful and encouraging content about living with PAH, including:

Self-care with PAH

Passages

Stephen Carter-Hicks

Navigating life with PAH is about more than understanding the disease—that’s just the first step!

Real patient stories and insights

30 YEARS OF PHA

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.

James Ryan*

Mary Jan Hicks

Louise C. and Gene P.* Salvucci

Carl Hicks, Jr.

Judith and Edwin L. Simpson

Phyllis M. Hill*

Kelley Skumautz

Jackie Holt

Marcia and Jack Stibbs

Richard L. Horrocks

Helena M. Strauch*

Constance G. Ives*

Douglas R. Taylor

Terri L. Jakuboski*

Frank A. Tobac*

Stanley T. Jusinski

Martha and Carlos Torres

Laura J. Kelly*

Deborah J. and Roger K. Towle

Jamses Kenney

Carol B. Ungar

Jessie Kohler-Wenninger

Daniel R. Walsh*

Terri L. Kopp*

Andrea and Stephen L. White

Debbie L. and Mitchell Koppelman

* deceased members

Frank D. Koppelman* Dee* and Walter Kruger* Gloria J. Lang*

PHASSOCIATION.ORG 35

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