A publication of the Pulmonary Hypertension Association
In Celebration of PHA’s 30th Anniversary
Issue 4 2020 // Volume 29 No. 4
PLUS Flolan at 25 Pat Paton: Visionary for Patients Behind the Biomarkers
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PATHLIGHT // ISSUE 4
IN THIS ISSUE 2020 // Volume 29 No. 4
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Awareness Month: Tools to Discover PH
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Advocating During the Pandemic
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The Show Goes On
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Behind the Biomarkers
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Beating the Odds: Living Life to the Fullest
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Appreciation From an Ocean Away
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PH Through the Years
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Flolan at 25
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Pat Paton: Visionary for Patients
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Checking the Bucket List
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Family Affair: 30 Years of Volunteering
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Emergency Prep: Expect the Unexpected
On our cover: Diane Ramirez, longtime PHA volunteer, was diagnosed with pulmonary hypertension in 1987.
IN EVERY ISSUE 5
Letter From the Chair
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Quick Takes
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Passages
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BOARD OF TRUSTEES Colleen Brunetti, M.Ed., C.H.C., chair
PUBLISHER Brad A. Wong President & CEO
STAFF Linda Busche Editor-in-Chief Alanna Coogan Designer Karen Smaalders Vice President, Communications and Marketing
CONTRIBUTORS Said Boularouk, Gwendolyn Brown, Alex Flipse, Donna Head, Lauren Intrieri, Katherine Kroner, Mira Kruger, Annie McCall, Terry McGraw, Tim McGraw, Diane Ramirez, Ivan Robbins, M.D., Deborah Shapiro, Abby Sickles, Jill Zajac
CONTACT 8401 Colesville Road, Suite 200 Silver Spring, MD 20910 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.
Tony Lahnston, chair-elect Karen A. Fagan, M.D., immediate past chair Tony Lahnston, treasurer Nicole Creech, secretary Trustees-at-Large 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. William Hoff Kimberly Jackson, R.N., B.S.N. William Jansen Jessie Kohler Wenninger, Esq. Mitch Koppelman, Ph.D. Ronald J. Oudiz, M.D. Monica M. Penaranda Traci Stewart, R.N., M.S.N., CHFN Doug Taylor Matt Wall Melisa A. Wilson, D.N.P., APRN, ACNP-BC Brad A. Wong Emeriti ex-officio C. Gregory Elliott, M.D., FCCP, MACP Michael D. McGoon, M.D. Edwin Simpson Judith Simpson, R.N., Ed.S. Honorary Carl Hicks President & CEO Brad A. Wong 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
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. Nicole Reid, R.N., B.S.N., DABAT Distinguished Advisors 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.
Dear PHA Community, At the Pulmonary Hypertension Association (PHA), we have long seen ourselves “empowered by hope.” To me, being empowered means finding and using tools to make a difference. And hope means wanting something to happen and thinking it will be possible. Our hope at PHA is a better life for the PH community and a cure for PH. We use that hope, and our belief that it is possible, to empower us. Through that empowerment we support one another, fundraise, contribute to research, educate, and so much more. As PHA approaches its 30th anniversary, this edition of Pathlight focuses on “Lighting the Way Forward.” To do that, it helps to look back, as well as to look ahead. I remember when I was first diagnosed almost 13 years ago. I would look to the long-term survivors with so much awe, wondering how they did it. I hoped that someday I could be like them – defying the odds and leading the path forward. You’ll hear from some of these early heroes in this month’s cover story, “PH Through the Years.” People like Gwendolyn Brown, Alex Flipse and Diane Ramirez, who were diagnosed in the 1980s and 90s, have lived through and benefited from breakthrough developments of lifesaving treatments. In “Flolan at 25,” you’ll hear from Jeanette
Morrill and Joanne Volpe, who were diagnosed before treatments existed – and were brave pioneers trying the very first. We also take some time to honor one of our founders, Pat Paton. Pat sadly passed away this year. Her vision – and that of a core group of family and other patients who saw what PHA could be – led to the organization we know today. Pat and the founders didn’t just light the way forward. They blazed the whole early trail. Pat is truly missed, but we remember her with honor and learn from the example she set. These stories inspire us. As we look to learn from one another, those in the medical profession are pushing forward as well. In “Beyond the Biomarkers,” Jane Leopold, M.D., discusses a study that could upend current PH classifications based on cause. The science is exciting, and it’s getting better every day. Finally, as we close out this wild and unprecedented year, I want to take a moment to thank you for being a part of PHA. Things have been difficult in the world in so many ways. But through it all, PHA remains steadfast and here to help, and you are a part of that. For that, I am truly grateful. Warmly,
Colleen Brunetti, M.Ed., C.H.C. Board of Trustees Chair, Pulmonary Hypertension Association
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Announcing
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Learn more at PAHInitiative.com
Sponsored by United Therapeutics Committed to Improving the Lives of Patients
The PAH Initiative aims to improve the lives of adults living with PAH, connecting you with information and inspiration as you work with your healthcare provider to feel better and do more. © 2020 United Therapeutics Corporation. All rights reserved. US/DS/0448.
Awareness Month
Tools to Discover PH
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ach November, the Pulmonary Hypertension Association (PHA) provides tools to help people living with pulmonary hypertension (PH), their loved ones and health care professionals raise awareness of PH among the public, health care providers, those at risk for PH and those who are new to the PH community. This year’s campaign provided tools to help people “Discover PH.” The highlight of the 2020 campaign was an interactive PH Awareness Month Calendar. It worked like a simple computer game: Users clicked on the date to discover facts, tools and rewards to educate people about PH risk factors, symptoms and resources. Each day, the calendar revealed new information and downloadable resources to discover and share, similar to a virtual Advent calendar. Instead of chocolate, participants could download phone and desktop backgrounds, coloring pages for children, social media graphics and other information to promote PH awareness. The site offered many ways to take part in PH Awareness Month. As in previous years, it featured a toolkit with social media graphics, sample posts,
profile and cover images, customizable news releases, and fliers for health care professionals to print and post in their offices and clinics. Awareness Month activities also featured a Day of Action Nov. 10 and CTEPH Awareness Day Nov. 11. The Day of Action aimed to educate Congress about the devastating health and lifestyle effects of PH. PHA asked the community to share stories to help lawmakers understand the importance of funding research and ensuring accessible, affordable health care. On CTEPH Awareness Day, PHA drew attention to chronic thromboembolic pulmonary hypertension (CTEPH), a form of PH caused by blood clots in the lungs that harden over time and impede blood flow. It offered Q&As in English and Spanish with doctors and patients. Weekly Facebook Live events also addressed PH, connective tissue disease, and methamphetamine use in a four-week “Ask the Experts” series. Recordings of the Facebook Live Q&As are available at PHAssociation.org/AwarenessMonth, where you also can find the calendar, toolkit and other information.
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Thank You for Giving Tuesday The Pulmonary Hypertension Association (PHA) received $101,410 on Giving Tuesday, a global day of giving. Thanks to the generosity of the pulmonary hypertension (PH) community, donations were up 45% from Giving Tuesday 2019. The donations marked the largest amount PHA has raised on a Giving Tuesday. The record amount was possible thanks to 321 donors and a $25,000 match. Thanks to everyone who supported PHA’s Giving Tuesday campaign. You help PHA extend and improve the lives of those affected by PH.
Ensure Timely Prescription Refills If your insurance coverage is changing in January 2021, follow these steps to make sure your first medication refill of the new year arrives on time: Update your pulmonary hypertension (PH) care team. Many health insurance plans include prior authorization or step therapy requirements for PH medication. Your health care team might need to complete new paperwork or file an appeal to give you seamless access to your therapy. Alert your health care team to your insurance change to allow them to tackle obstacles now and reduce the chances of delays in the new year. Ask your health care team to prescribe a 60- or 90-day supply for your last refill of this year. Multi-month refills further reduce the risk of delays for those who are eligible.
Update your specialty pharmacy. Your new insurance plan might work with different specialty pharmacies to deliver PH therapy than your current plan. It’s important to notify your specialty pharmacies that you’re changing health insurance plans. Review your financial assistance options. Changes in your health insurance coverage sometimes can change which copay assistance you’re eligible for. Manufacturer assistance programs, nonprofit grants and government-funded support programs have different eligibility criteria. Contact PHA’s Treatment Access Program at PHAssociation.org/HELP for information. Call 301-565-3004 x749 or email us at Insurance@ PHAssociation.org.
PHA thanks Janssen Pharmaceuticals Inc. for its support of PHA’s Treatment Access Program.
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ADVOCACY IN ACTION
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he Pulmonary Hypertension Association (PHA) is the leading voice to Congress and federal agencies on the needs of people with pulmonary hypertension (PH) during the COVID-19 crisis and beyond. Over the past six months, PHA has advocated to: • End surprise medical billing. • Provide robust health insurance coverage for telemedicine and COVID-19 testing and treatment. • Expand paid sick leave benefits. • Enhance access to charitable assistance. PH advocates have been essential to raising congressional awareness and making progress on these issues. This year, PH advocates have sent more than 1,500 email messages to Congress. Others made personal connections in August with legislative staff through phone calls or video conferences. They help educated them about what life with PH is really like and how Congress can help. Become part of PHA’s advocacy network. Visit PHAssociation.org/Advocate or call 301-565-3004 x749.
Holly Szmutko, met virtually this summer with Katherine Hitchcock from the office of Sen. Todd Young (R-Ind.). She is pictured with Rep. Pete Visclosky (D-Ind.) in 2019.
PHA is grateful to our National 2020 PHA O2breathe sponsors for their generous support: Platinum Sponsor Janssen Pharmaceuticals Inc., Gold Sponsor Bayer, and Silver Sponsors CVS Specialty and United Therapeutics.
For up to date information on PHA 2021 Fundraising Events, please visit our website at PHAssociation.org/ pha-fundraising-events/. Contact PHA at Events@PHAssociation.org or 240-485-3810 with questions about PHASSOCIATION.ORG any events.
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PHashionistas Walk the Runway for Summer PHling
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he pandemic put a halt to in-person gatherings, but it didn’t stop the pulmonary hypertension (PH) community from celebrating virtually for the Pulmonary Hypertension Association (PHA) Summer PHling. Hosted by PH advocate and former Broadway performer Stephen Carter-Hicks, the Aug. 1 event included an online version of the PHA Conference fashion show and recognition of many PHA volunteers. Stephen started the event by detailing his pulmonary arterial hypertension (PAH) journey. He recalled feeling winded on stage during a performance of “Cats.” After a cardiologist confirmed PH, Stephen connected with PHA. “When I was diagnosed, I didn’t know how PH would take away things I value, like my career,” Stephen said. “When I found PHA, it literally saved my life. I got connected with my local support group and got involved with fundraising events. I had a way to fight back. I’m so grateful they are here for all of us today.” The fashion show featured 24 PH patients who proudly flaunted or cleverly concealed oxygen tanks, pumps and mobility devices. Fashions included an array of periwinkle, zebra stripes, sparkly dresses, stylish hats and superhero costumes. Because of the COVID-19 pandemic, this is the first year the fashion show didn’t take place on stage in front of a cheering crowd at the PHA International
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PH Conference and Scientific Sessions. It also was the first year face masks made an appearance on the PHA (virtual) runway. Stephen sported a hat adorned with his mask collection. He said his favorite mask reads, “Raise my voice.” After the show, PHA recognized dozens of advocates, fundraisers and support group leaders who contribute to the organization. Some volunteers shared videos of why volunteering was important to them, including support group leader Betsie Miklos. Betsie was diagnosed with PAH in 2010. She heard about PHA shortly after her diagnosis when a nurse gave her a copy of PHA’s Survival Guide. Leading the Northern Virginia Support Group was Betsie’s first volunteer experience with PHA. “I love leading the support group and watching people change after coming to meetings,” she said. Other volunteers who submitted videos included PH cardiologist Anjali Vaidya, M.D., of Temple University and PHA Board of Trustees secretary Nicole Creech.
PHA thanks Stephen and all the volunteers who contributed to Summer PHling, as well as throughout the year. PHA looks forward to seeing everyone at PHA 2022 in Atlanta.
Kelly and Riley Wiegele Aubrey and “Chopper” Rich Hozjan
Stephen Carter-Hicks Alexis Trotter
Patti Smardz
Eric Piburn
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HEALTH MATTERS
Searching for Clues Within PH Classifications
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bout 25 years ago, cardiologist Jane Leopold, M.D., noticed how the structure and function of blood vessels differed in various diseases. In particular, the differences associated with pulmonary hypertension (PH) couldn’t be explained clinically and scientifically by what happened with other blood vessels. She also observed that people with PH differed from her typical cardiac patients with chest pain and heart attacks. Those with some forms of PH were younger, sicker and had more physical limitations. They also seemed to be hospitalized more frequently with heart failure, shortness of breath and other issues. Since then, PH research has led to advancements in PH diagnosis and treatment. But researchers still need to better understand the disease to identify and develop the right drugs to make people feel better ‘Patients never fall neatly into a single category, and those within each group are really different from each other. Let’s find out what the new groups should be, based on blood samples and clinical testing.’ — Jane Leopold, M.D.
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and live longer, says Dr. Leopold, director of the Women’s Interventional Cardiology Health Initiative at Brigham and Women’s Hospital in Boston. “What we have is still not good enough,” she says. “We can’t predict who will develop PH, what medicines the patients will respond to or how sick they will get.” X Redefining PH classifications Dr. Leopold is among the investigators of a pulmonary vascular disease (PVD) omics study funded by the National Institutes of Health, National Heart, Lung, and Blood Institute and Pulmonary Hypertension Association (PHA). The study, “Redefining Pulmonary Hypertension through Pulmonary Vascular Disease Phenomics (PVDOMICS),” aims to identify biomarkers to improve PH diagnosis and treatment. The study will help Dr. Leopold and her colleagues find subgroups of patients that cluster together within the five PH groups based on underlying cause. Their findings could upend the current PH classifications. Investigators believe they will find more alike people within the subgroups than within their “assigned” groups, based on disease severity, life expectancy and response to medication. They also want to learn whether people stay in the same
HEALTH MATTERS
subgroup or shift among them. “Patients never fall neatly into a single category, and those within each group are really different from each other. Let’s find out what the new groups should be, based on blood samples and clinical testing.” X Patient involvement The study follows 1,200 people who have PH or risk developing PH, as well as a few healthy individuals. Investigators track data from clinical histories, physical exams, blood samples, right heart catheterization and other heart and lung tests. “For the first time, can compare patients with PAH and those with CTEPH or other types of PH.”
‘We need to get people to come into the study with really different backgrounds and experiences to find out what’s going on with the disease.’
In late 2019, investigators enrolled the last patients in Phase I. They began Phase II in early 2020 with about 400 people. When COVID-19 was a declared a public health emergency, investigators had to switch from inperson clinical and blood tests to phone follow-ups.
Soon after, they learned that they received more funding to follow a subset of seriously ill people with Group 2 and 3 PH. “There’s going to be a lot interesting information from this study,” Dr. Leopold says. “We’re just starting to look at the first data.” Ideally, Dr. Leopold would love to see the study expand throughout the United States. “We need to get people to come into the study with really different backgrounds and experiences to find out what’s going on with the disease,” she says. Dr. Leopold hopes her work to better understand, identify and treat PH will improve the patient journey and decrease the times patients are misdiagnosed. The research wouldn’t have been possible without PHA and people with PH enrolled in the study, Dr. Leopold says. “We could not have done this without you.”
Find out more about current clinical trials in PH through PHA’s Clinical Trial Finder: PHAssociation.org/ClinicalTrials.
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Living Life to the Fullest Donna Head, 59, first noticed her pulmonary hypertension (PH) symptoms in 1988 on a visit to Hawaii to see her brother’s new baby. At the time, her own son, Nathan, was just 3. Today, she lives along the Alabama coast and loves to read, crochet, cook and work with her church.
By Donna Head
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hile visiting Diamond Head volcano, I had trouble going up the walkway. After making it to the top, I saw stairs going straight up and said, “I can’t do this.” My brother helped me get up, huffing and puffing. I finally got to see the view. Over the next three years, I had problems with my heart racing. I worked as a nurse in a doctor’s office, where everyone thought it was stress. I went to a pulmonary doctor, who thought I had asthma and put me on inhalers. A few months later I passed out at work. I went to a cardiologist on a Wednesday morning that I will never forget. They put me on a treadmill, and my heart rate rose to 180 beats per minute in three minutes. They performed a right heart catheterization the next morning and admitted me to the hospital. Later that day, my best friend and brother came by and brought thoughtful gifts. The next morning,
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Donna and her son Nathan
Donna and family
after a pulmonary function test, my doctor said I had primary pulmonary hypertension, now known as pulmonary arterial hypertension (PAH). He was crying, and my mom was crying as he said, “You are dying, and it won’t be much longer.” Reality check Reality check My first question was, “I can’t have any more children?” I then asked, “How do we fix it?” He said there were no medications at this time, but a heart/ lung transplant would save me. We went home, and it didn’t hit me. Five days later a man from my church came by, and the look on his face told it all. I finally cried. The next week, I got an appointment at UAB Hospital in Birmingham, Alabama. They scheduled a heart/lung transplant evaluation for January 1992. The next morning, I checked into the hospital and was admitted for a rough week of transplant evaluation. On the last day, a right heart catheterization showed my pulmonary arterial pressures had dropped in half. I no longer needed an immediate transplant. Finding love Finding love I lived life to the fullest and kept working. In June 1992, I met a wonderful man, Milton, and we started dating. I had trouble getting up my nerve
to tell him about my diagnosis. One night, after a movie, I finally told him that I had a rare heart condition. I said the doctors thought I had about a year to live. He held my face in his hands and said we would get through it. He said he would make that the best year of my life. We went dancing, sat on the porch swing, talked for hours, went to concerts and walked. He even went to Birmingham with me to see my doctor. On Dec. 23, Milton proposed. I jumped in his arms and said “yes.” Nathan had helped him pick out the ring and kept the secret. The next morning, I asked Milton whether he was sure because we might not have much time together because of my PH. He said he would take a week if that’s all he could get with me. One year later, we were married. We still are. Moving forward Moving forward My doctors have tried every new medication that comes out for PH. I found Dr. Ben DeBoisblanc, whose personality perfectly matches mine. He has saved my life more times than I can count. I started Tracleer as soon as it came out. Next, we tried subcutaneous remodulin. I tried Viagra when it was in trial stage. I was put on Tyvaso, then Orenitram, then Uptravi. One of my nurses suggested that I start a
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Donna and her great nephew Harrison
Donna and her son Nathan
Pulmonary Hypertension Association (PHA) support group. After I found a spot to meet, the nurses sent fliers to their PH patients, and we had our first meeting. It was wonderful to meet other PH patients. I have been lucky to have stayed off IV medications 30 years after diagnosis. I recently went back on Tyvaso and need oxygen all the time. I have been so blessed, and PHA has helped me so much over the years. Looking back Looking back When I was diagnosed, there were no medications for PH. My doctors wanted a right heart catheterization every six weeks. They wanted to know if the calcium channel blockers stopped working so I could have a heart/lung transplant. This went on for about eight years. There wasn’t much I couldn’t do in the first 10 years of PH. Eventually, my doctor suggested that I quit working to live longer. That was rough because I wanted to be a nurse my whole life. But my son will tell you it was worth it in the long run to have me home with him. He played drums in the high school band, and I worked in the concession stands every week. I saw him graduate
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from high school and start college at the University of Alabama. full life AA full life About six months ago, I was having trouble getting from the shower to the bed. When I went to the doctor my right lung was full of fluid. I was in right heart failure.
‘I’m so happy I didn’t give up all those years ago.’ I am now on oxygen 24/7 awaiting insurance to approve a medication. Although I have stayed off IV therapy, I will do whatever it takes to get better. I want to live, see my son get married, and have grand babies. My husband and I are still very much in love, and we have two Boston terriers. I am so happy I didn’t give up all those years ago. I have had a very full good life.
Appreciation From an Ocean Away By Said Boularouk
Fifteen years after learning he had pulmonary hypertension (PH), Said Boularouk wrote to the Pulmonary Hypertension Association (PHA) from his home in France. Here is his story:
I
was a member of your association from 2005 until 2008. I had just been diagnosed with pulmonary hypertension. At the time, I was living in Algeria, hopeless. I was dying in silence because the French consulate refused to grant me a visa to leave Algeria for France, where my cardiologist of many years practiced. PHA was a lifeline of information. In 2006, I learned about your conference, the PHA International PH Conference and Scientific Sessions. I was thrilled when I was able to attend! I learned so much about PH and met many people who were able to help me on my journey. In 2007, Italy granted me a single-day visa, which was enough time for me to travel to France and see the PH specialist I needed. This doctor, whom I have known since I was 9 years old, was furious about the bureaucratic delay because it meant more damage to my heart. You see, France had been refusing my visa requests since 1992 because of the civil war in Algeria. Even though I met all the criteria on paper, and even though my father worked in France and my grandfathered served for the French in World War II, I was still denied. Finally I was able to make it there. After that, life changed rapidly for the better. I
received a transplant in 2010. I became a French citizen in 2016. I earned my Ph.D. in information technology in 2018. I have been an engineer since 1996 when I studied in Algeria, and I am an inventor. I created an invisible piano, a kind of sensor, that tests the ability of blind people to read spatial data using music. I even published a book about assistive technology in visual deficiencies in 2019. Because of you, I am alive; I progress; I am grateful.
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When Gwendolyn Brown, Alex Flipse and Diane Ramirez were diagnosed with pulmonary hypertension (PH) in the 1980s and 1990s, little was known about the disease. Life expectancy was all too short, with limited treatment options. Specialized care didn’t exist. They were scared, depressed, exhausted. But as research and treatment evolved, they found strength, resilience and their voices. They also found a community of other people affected by PH, connected by a new organization, the Pulmonary Hypertension Association. In this month’s cover story, they recall their challenges, triumphs and hopes for the future.
Gwendolyn R. Brown Cleveland, Ohio
I have lived with pulmonary arterial hypertension (PAH) for more than 40 years. I have survived a heart attack, three strokes and 11 mini strokes. Today I lead PHA’s Cleveland Community Support Group and am a minister at Zion Pentecostal Church of Christ. When I first showed signs of PH, I was misdiagnosed and treated for asthma for three years. I got worse, passed out, and my body started swelling up. I couldn’t go up a flight of stairs or do any activities around the house.
1987: Dorothy Olson, Teresa Knazik, Shirley Brown and Pat Paton connect with a goal to help PH patients.
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1990: First issue of Pathlight published.
On July 10, 1982, I had a heart attack. I was 25 with a 4-year-old daughter. After a lung biopsy, I was diagnosed with primary pulmonary hypertension (PPH). The doctor told me that I had PH, a very rare disease that had no cure. He said I should go home and get my life in order because I would not live to see 28 years old. Living with PH in 1982 was very challenging. I didn’t understand why I had this disease. The only treatments available were channel blockers, blood thinners and water pills. I did what my doctors asked of me. They told me to lose weight, and I did. New drug New drug By 1998, my illness took a toll on me. I was on daily oxygen, and my daughter Kennie had become my caregiver. I felt like there was nothing else my doctors could do. Then I got a call from Victor Tapson, M.D., at
1991: Dorothy, Teresa, Pat and Judy Simpson meet in Florida to organize United Patients Association for Pulmonary Hypertension (kitchen table meeting).
L to R: Gwendolyn’s son-in-law and daughter Freddie and Kennethian Green, her mother Delores Rutledge and Gwendolyn.
Duke University Medical Center to try a new drug called Flolan. I responded well. It brought my pressures down, and I no longer needed oxygen. I was excited to be a part of a new treatment for PH.
still take. In 2004, I became a support group leader. My group has brought me more joy than I ever could have imagined. Living with PAH for this long has allowed me to see how PAH research has grown. Now, it doesn’t take as long to be diagnosed, and there are more pulmonary doctors and clinical trials. I am amazed how far we have come. Sometimes I sit and cry of pure happiness that I lived this long and got to witness it all.
Words tolive livebyby Words to My faith and family kept me hopeful. I knew God had a better plan for me. I told myself repeatedly that this wasn’t going to be the end. Since 1998, I’ve believed, “I have PAH, but PAH doesn’t have me.” It’s something that I live by to this day. After my family and I moved to Cleveland in 2003, I met my amazing pulmonologist Robert Schilz, M.D., at the University Hospital Cleveland Medical Center. He put me on a trial for Remodulin, which I
1991: First PH support group meeting organized.
Alex Flipse Temecula, California
When I was a young mom with my third daughter on the way, I felt fatigued. I thought it was because
1992: UPAPH files as a not-for-profit organization. Scientific Advisory Board (SAB) created.
1994: First International PH Conference in Stone Mountain, Georgia, with the first Research Room. Support groups now in eight states.
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L to R: Alex, Erika Prieto, Elizabeth Galindo and Monica Sanchez.
Monica Sanchez and Alex
I was running after two toddlers while six months pregnant. After my daughter was born, I needed more sleep. A couple years later, I started passing out. I was diagnosed with idiopathic pulmonary arterial hypertension in 1998 when there was only one FDAapproved treatment. I was told I had two years to live. I started Flolan the day of my first right heart catheterization. I was told that if I hadn’t started Flolan, I wouldn’t have made it two more months. In the beginning, I was incredibly worried about mixing Flolan wrong. No matter how clean I kept my Hickman line, I had two or three infections a year for four years. Waves of exhaustion and major depression left me practically bedridden. My kids had to take care of themselves and each other. I was basically a single mom because my then-husband was working six hours away and came home every few weeks. Living in a small town in Texas at the time also made having PH difficult. I had to teach the emergency nurses, doctors and the emergency medical services team about my condition. The
1995: FDA approves Flolan. UPAPH creates support line. Julie Paton Hendry becomes first full-time UPAPH volunteer.
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nurses knew me and would greet me by opening the main door. Hidden strength Hidden strength I was on Flolan for four years before Tracleer was approved. A few years after I was weaned off Flolan, I started working again. It was wonderful. Following my divorce in 2008, I moved to Washington, D.C., to live with my sister. In the 22 years since my diagnosis, I have found my voice and a strength in myself that I never knew existed. PH gave me the freedom to speak out, raise awareness, lobby on Capitol Hill and run several support groups. I have volunteered at the PHA office in Silver Spring, Maryland, many times over the years and attended nine PHA conferences and events. Hope forthe thefuture future Hope for When I was diagnosed, there was very little social media. Facebook lets me connect with other PHers
1997: UPAPH formally becomes Pulmonary Hypertension Association. First PH/PPH Awareness Week. PHA launches PHAssociation.org.
1998: Pulmonary Hypertension Resource Network created. First Pulmonary Hypertension: A Patient’s Survival Guide published.
and share memes to raise awareness. Having an invisible disease and other rare conditions is difficult. People don’t know how sick you really are, and it doesn’t seem to get better as the years go by. I am hopeful for a cure. With PHA on our side, we will find cures for PH. I used to think we needed a single cure, but we are all so different. Our symptoms manifest differently, and the side effects of our treatments affect us differently. I have had the privilege of living past my expiration date many times over. I never thought I would see my children graduate from high school, and I never dreamed I would hold grandchildren. I didn’t think I would make it to 30 years old. But here I am.
Diane Ramirez Lexington, North Carolina I was diagnosed with PAH in 1987 when it was called PPH. There were fewer than 200 people diagnosed in the U.S. at that time. I was given a life expectancy of two to three years without a transplant and told to get my affairs in order. Receiving a PAH diagnosis at 24 years old was extremely challenging. My work, school and social life had to change. There were no treatments for PH. I was put on oxygen to help with shortness of breath, heart medicine and a blood thinner as a precaution. I was terrified, and I barely understood what was happening.
1999: PHA hires first employee Rino Aldrighetti as part-time executive director and opens Silver Spring, Maryland, office. PHA creates Research Fund. PH Resource Network becomes official part of PHA.
Finding gratitude Finding gratitude So much has changed since then. Today I have a better understanding of PH and what it entails. I am happy and grateful to be alive. I didn’t have much gratitude or happiness when I was diagnosed. I felt alone and scared most of the time. I connected with other patients online in 2001. Later that year I had my first appointment with a PH specialist. Today, I take three medications approved by the FDA to treat PH. I receive treatment at Duke University Medical Center, a PH Care Center. I wanted and needed support with this illness when I was diagnosed. I found out about PH before PHA and support groups existed. Today I am a support group leader for PHA’s Piedmont Area North Carolina group, and I try to help patients as much as I can. I have participated in clinical trials to help with research. Finding my Finding myvoice voice At PHA’s 2008 International PH Conference and Scientific Sessions in Houston, I learned about
2000: PHA grants first research award, AHA Post-Doctoral Fellowship.
2002: SAB changes name to Scientific Leadership Council. First issue of Advances in PH published.
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advocating for myself. A couple of people helped me understand the importance of my voice. Later that summer, I set up my first meeting with a member of Congress. There were five patients in that meeting, and we all felt heard. It was incredible. I have advocated on behalf of PHA for PH awareness, research and education for 12 years, and I have no intention of stopping. Meeting members of Congress locally and in Washington, D.C., and speaking up for myself and others is part of what keeps me alive today. I served on the PHA Board of Trustees for 10 years. My hope is that I can be an example to other PH patients to use their voices and be heard. After living with this illness for over 33 years, I learned that my voice counts, what I go through matters, and what I do makes a difference. I learned to enjoy all the moments life has to offer: to laugh, dance when I can, call a friend and be a friend. PH has taught me how to persevere. PHA has given me the opportunity to speak up and to learn valuable lessons. This illness has taught me how to give when I think there is nothing left to give. It has taught me to never give up.
2003: PH Resource Network (now PH Professional Network) hosts first Symposium.
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2005: PHA launches regional medical education programming.
Physician Perspective: Evolution of PH Care Ivan Robbins, M.D., of Vanderbilt University Medical Center in Nashville, shares how pulmonary hypertension (PH) treatment and research have evolved over the course of his career.
I went to medical school and did my residency in Cleveland at Metrohealth Medical Center. At the end of my internship in internal medicine, I knew that I wanted to go into pulmonary/critical care. I went to Vanderbilt University Medical Center for my training from 1994 to 1997 and have remained on the faculty for my entire career. The first PH patient I remember was a young woman with idiopathic pulmonary arterial hypertension (IPAH) who was in the intensive care unit. We gave her inhaled nitric oxide, a pulmonary vasodilator, and her pulmonary artery pressure decreased in half. I thought this was truly amazing. We treated her with calcium channel blockers for many years. When I started taking care of PH patients 26 years ago, there were no PH-specific therapies. The only options for treatment were calcium channel blockers or lung transplant. During my fellowship, intravenous epoprostenol was approved to treat IPAH and then other forms of PAH.
2006: NHLBI funds two PH Centers of Excellence, providing nearly $25 million for new PH research.
2014: PHA launches accreditation program for Pulmonary Hypertension Care Centers.
Overall, there has been a great increase in awareness of PAH and the availability of multiple treatments for this condition. I became involved in PHA when I was a fellow and have attended every biennial meeting since the second meeting at Stone Mountain, Georgia, in 1996. PHA’s support has helped me and my patients by increasing PH awareness in the medical community and promoting PH research. Through PHA, I have gotten to know many other health care professionals who have devoted themselves to the care of patients with PH. I have made many good friends along the way. There is a special comradery that comes from caring for patients with a rare disease. Although we have come a long way, lung transplant remains the only curative treatment for PAH. Other than calcium channel blockers, transplant was the earliest effective treatment for PAH. In fact, our oldest living lung-transplant patient is a woman with heritable PAH whose surgery was 29 years ago. Looking forward Looking forward
Treatment and Treatment andresearch research I was fortunate to be involved in initial studies that led to approval of the first oral therapy for PAH. Since then, over 10 therapies have been approved for treatment of PAH, alone or in combination. There are now well-recognized guidelines for evaluation and treatment of PAH, which are readily available to any clinician.
2016: Brad Wong becomes PHA’s second president & CEO. PHA celebrates 25th anniversary.
I am hopeful for the future. New treatments for PAH are evaluated every year. As our understanding of the pathogenesis of this disease grows, I am confident that eventually we will find a cure. Soon we will be able to target specific therapies to individual patients based on their genetic make-up [See Biomarkers, Page 12]. Investigators are working to regenerate organs, which could overcome many drawbacks of lung transplant, such as life-long immunosuppression drugs. The PH field has come a long way since I began my career, and the changes have been truly remarkable.
2017: Massachusetts is first state to pass legislation that calls for increased PH treatment/care.
2019: PHA Registry enrolls 1,000th patient.
2021: PHA celebrates 30th anniversary.
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First PH Treatment Offered Second Chance at Life
J
eannette Morrill initially thought she would never do anything useful or meaningful after her pulmonary hypertension (PH) diagnosis in 1976. Then 23, she was told she had six months to live. Doctors told her parents to make her as comfortable as possible. But Jeannette wanted to make the most of the time she had left. “I decided that I would make sure I did something every day,” she recalls. “It didn’t matter how I was feeling. Even on my worst days, I would at least make sure I got up, got dressed and got out of my bed.” In 1987, she and her husband David adopted two sons, Brian and Ian. She found a doctor who told her, “If you are willing to try, I will do everything I can to help you survive until, perhaps, some new medication, comes along.” In 1995, a medication did come along. The Food and Drug Administration approved Flolan, or epoprostenol. It has transformed Jeannette’s life
and the lives of thousands of other people with PH since then. “It was a complete game changer,” says Murali Chakinala, M.D, chair of the Pulmonary Hypertension Association’s Scientific Leadership Council. “It provided the first real hope that PH patients had.” Targeted therapy Targeted therapy Before Flolan, there was no targeted therapy for PH. Physicians prescribed vasodilators, blood pressure medication and other drugs that worked on arteries throughout the whole body, rather than arteries in just the lungs. For many patients, a lung transplant was their only hope. Flolan was different. Administered through a catheter placed surgically into a large vein in the chest, it targets arteries in the lungs. It relaxes and dilates narrowed blood vessels, increasing blood supply to the lungs and reducing the workload of the heart. A small battery-powered pump keeps the medication flowing continuously into the body. “People literally were able to get out of bed and start living again,” Dr. Chakinala says. No longer longer couch-bound No couch-bound In 1992-93, JoAnn Volpe was the head secretary of a Connecticut high school. At 44, she had no energy. She huffed and puffed going to and from the parking lot. She passed out trying to shower. Her daughter worried so much about her that she didn’t want to go away to college. Eventually, JoAnn was diagnosed with PH and had to leave the job she loved. “I think they thought I was going to drop dead on their premises,” she says.
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Jeannette Morrill
After her diagnosis, a doctor at Hartford Hospital In 1997, Jeannette was referred to Robyn Barst, referred her to the University of Pittsburgh Medical M.D., who told her about Flolan. After many tests, Center, where she was evaluated for a heart and lung including a heart catheterization, Dr. Barst told her transplant. During her two-week stay, a heart doctor she was a good candidate for Flolan. told her about a clinical trial for a new drug, Flolan. In April 1997, a Groshong catheter was inserted JoAnn decided to join the trial if it meant she might in her right chest, and Jeannette began using the not need a transplant. CADD pump for constant infusion. She stayed on She returned to Connecticut with a central line and Flolan until September 2006. the ability to properly mix and store her medication. “I was so happy to find that there was something “I thought I was too old to learn how to do it,” she else that would help me,” she says. recalls. Twenty-six years later, she still receives IV therapy, Game changer Game changer but she’s since switched to Remodulin, which is premixed and doesn’t have to be refrigerated. The drug The therapy was equally remarkable for health eliminated the need for a transplant. care professionals. “You now had therapy that made “What a difference,” she says of the drug that saved people feel better, that turned PH into a treatable her life. “I can breathe without condition,” Dr. Chakinala says. problems. I only use oxygen at When Jeannette started taking night. I used to be couch-bound.” ‘It was a complete game Flolan, she found that she could changer. It provided the do more than just get up and get New opportunities dressed. New opportunities first real hope that PH “I started challenging myself. I patients had.’ Jeannette was practically couchstarted walking from one telephone bound as well. Like JoAnn, she had — Murali Chakinala, M.D. pole to another until I was walking to quit her job teaching physical a mile on a regular basis.” education. But after she started Her shortness of breath gradually taking the calcium channel blocker Nifedipine, decreased. She got a cart and started golfing almost which controls blood pressure, she felt well enough every day. She taught her nieces and nephews how to to return to teaching. She became a classroom play four-square, badminton, basketball and soccer. teacher, instructing fifth graders on a variety of “I was able to play with them without having to subjects. worry about fainting in front of them,” she says. Eventually, the Nifedipine lost its effectiveness. Although she had to stop teaching to protect her “I was like I went back in time,” Jeannette says. “I immune system, Jeannette tried to get back to the couldn’t do stairs. I got very short of breath doing many things she had lost before Flolan. anything that required physical exertion.” “Did I ever think I would get back to normal, like PHASSOCIATION.ORG
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Joann Volpe and family
before PAH? No, but I was going to try very hard to balance activity, rest, family, household chores.” Critical careelement element Critical care Twenty-five years later, epoprostenol and treprostinil remain the most potent, effective PH treatments, Dr. Chakinala says.
‘What a difference. I can breathe without problems. I only use oxygen at night. I used to be couch-bound.’ — JoAnn Volpe “We still rely on them for the sickest and most compromised patients,” he says, noting that scientists and researchers continue to develop more convenient delivery methods with less risks. “That class of drugs is not going away. It’s such a critical part of our patients’ care management.’ Meanwhile, Jeannette continues trying to live well with PAH, accepting what she can’t change and working to change what she can.
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Today, Jeannette and David live in Old Orchard Beach, Maine, where she leads a PHA support group, golfs and spends time with her 7-year-old grandson son, Kai, her “joy.” “Flolan really saved me a second time from death,” she says. “I think of all the other patients it has helped and feel very blessed.”
Pat Paton: A Visionary for Patients
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atricia Rae Berg appeared a bit shy when she was growing up in Logansport, Indiana, but by high school, she already showed the leadership qualities that set her apart. “She was a determined person,” Judy Simpson says of Pat Paton, her 15-month younger sibling with whom she founded what became the Pulmonary Hypertension Association (PHA). “That’s why she survived as long as she did.” Pat’s journey with pulmonary hypertension (PH) began in the late 1970s, about 10 years before her 1987 diagnosis. At the time, she her husband Jerry were raising their daughter Julie and son Mike in Zionsville, Indiana, an affluent Indianapolis suburb. Pat left her nursing career to manage two Dairy Queen restaurants that had been in Jerry’s family. They supported the high school students who worked for them with college scholarships. They participated in civic and community organizations. Pat was known for her innovative approach. When Dairy Queen sent franchisees whole candy bars to break up and mix with ice cream to make Blizzards, Pat triple-wrapped the chocolate in plastic and drove her car over them to break them more easily. “She ran everything in Zionsville,” Judy says. “When she and Jerry moved to Florida, she ran everything there, too. Wherever she went, she took charge.” new challenge AA new challenge Cardiologist Michael McGoon, M.D., met Pat and Jerry when they came to Mayo Clinic in Minnesota after Pat’s diagnosis. Pat was stunned and understandably fearful, Dr. McGoon recalls. “There was no question that the diagnosis was correct and her degree of PH was severe.”
But Pat and her family didn’t see her six-month to two-year prognosis as “an immutable act of fate.” They saw it as a challenge to be addressed head-on. Judy recalls one of Pat’s questions for the Mayo Clinic doctors. She asked what it would be like if she died of PH. When they told her she probably would die from right heart failure, she didn’t want to drive any more. She was afraid she might injure someone if her heart gave out. But after 18 months, Judy’s husband, Ed Simpson, asked Pat, “When are you going to start thinking about living, not dying?” Standing out Standing out In those early visits to Mayo, Pat and Dr. McGoon developed what would become a lifetime friendship.
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Judy, Dorothy and Pat
Pat and Dorothy
“She would emphasize her wishes and intentions by jabbing her finger at me, often tapping my chest,” Dr. McGoon says. “This gesture became a mainstay of our conversations for decades. It unquestionably made a lot of decision-making relatively easy. “I remember once at some event, probably a PHA Conference. The speaker asked Pat to stand from her place in the audience to be recognized, and the petite but feisty lady hollered out, ‘I already am!’ ” The early years also marked Pat’s desire to meet other people with PH. It took two years to find one – a friend of one of Judy’s nursing students. They met in DeKalb, Illinois, at Judy and Ed Simpson’s house. Judy, Ed and Jerry could see how important the meeting was to Pat. She had found a kindred spirit. Dynamic force Dynamic force When Pat and Jerry moved to Florida for warmer weather and sea-level altitude to ease her breathing, she kept looking for other people with PH. After someone referred her to Teresa Knazik and Dorothy Olson, they decided to meet. Judy and Ed flew to Florida to join them. That 1991 gathering, known as the “kitchen table” meeting, set plans into motion for the United Patient’s Association for Pulmonary Hypertension (UPAPH), now PHA. “Pat’s part was so important,” Judy says. “She took on the responsibility of contacting every patient until the list got into the hundreds. She was the dynamic force behind all of us.” Judy describes Pat as smart, sympathetic and empathetic. “But she knew how to push your buttons to make you move,” Judy says. Pat saw the organization founded by four women 28
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around her kitchen table grow to become one of the largest rare disease associations in the U.S. and inspire the founding and growth of more than 80 international partner associations, says Rino Aldrighetti, who was hired as PHA’s first professional, part-time employee in 1999 and became its full-time executive director in 2001. “Pat had a vision for PHA, and I am so glad she lived to see it come to fruition,” says Bruce Brundage, M.D., a Bend, Oregon, cardiologist who served on PHA’s Board of Trustees.
‘Pat was the organizer, the person who stood out of the spotlight and did the roll-up-your-sleeves work of getting programs started and keeping them moving forward.’ — Rino Aldrighetti Setting the for growth Setting thegroundwork groundwork for growth Rino says many nonprofits ultimately fail because the founders have a hard time opening the door to new leaders. Thanks to Pat and Judy, that wasn’t the case when Rino came to PHA. “Pat said to me, ‘Always find ways to involve more people. We are a family, a community, and everyone has a right to lead or follow, as they choose.’ ” Before PHA had full-time employees, Pat set the groundwork for PHA’s growth, from the telephone support line to the regional coordinator program and so much more. “Pat was the organizer, the person who stood out of
the spotlight and did the roll-up-your-sleeves work of getting programs started and keeping them moving forward,” Rino says, describing her as the “quiet one” among PHA’s early leaders. “That’s not to say she didn’t have clearly voiced and sometimes fierce opinions,” he says. Passion for Passion forpatients patients Greg Elliott, M.D., and Dr. Brundage, remember meeting the Patons, Simpsons and other founders in 1994 at the first PH International Conference in Stone Mountain, Georgia. Dr. Elliott was gathering DNA samples from people with what was then known as primary pulmonary hypertension (PPH), in what became the first Research Room. “When we arrived at Stone Mountain, it was a complete joy to meet the Patons and the Simpsons. They were just as gracious as can be, and they remained that way every time we were together,” recalls Dr. Elliott, medical director, graduate medical education, Intermountain Healthcare, Utah, and an emeritus member of the PHA Board of Trustees. “I will always remember Pat fondly for her humility, honesty and passion for helping others.” That was evident after Conference in 2006, Rino recalls. The board was feeling good when it met afterward. Attendance had taken a large jump. For the first time, Conference had included a full scientific program for medical professionals. PHA had secured sponsorship for the Scientific Sessions from the National Heart, Lung and Blood Institute, Centers for Disease Control and Prevention and the Office of Rare Diseases.
PHA also had granted about $50,000 in patient scholarships, which the board believed to be the largest among health nonprofits. Before they could celebrate, Rino said Pat reminded them: “This Conference was great for all who could afford to come. Our job is to think about those who couldn’t. We have to expand the scholarship program a lot.” Pat’s quiet challenge made all the difference, he says. By the next Conference, PHA had quadrupled the scholarship fund to $200,000. “While she never sought credit, Pat’s focus on patients made all the difference,” Rino says. Building aalegacy Building legacy Conference was exhausting for Pat, but she loved what she was doing, Judy says. “Pat felt she had been gifted. Even though she wouldn’t have chosen to have PH, she felt that was her purpose.” In addition to her PH, Pat had severe, painful osteoporosis. “Keeping herself occupied with PHA helped control the pain,” Judy says. “She was a fighter and a warrior.” Dr. Brundage got to know Pat and Jerry well when he served on the board. He considers Pat, Jerry, Judy and Ed the four cornerstones of PHA. “Unfailingly, they were at every meeting — board, committee or biennial — always leading and always contributing,” Dr. Brundage says. “They were family, and they made you part of the family too.’ He describes Pat as a person of few words. “But when she spoke, you had better listen because it was going to be meaningful.”
Pat Paton Leadership Award To honor Pat’s legacy, PHA established the Patricia “Pat” Paton Leadership Award, which recognizes Pat’s community-based leadership style and her belief that “from the actions of one emerge possibilities for many.” To donate toward the award, which will recognize PHA’s volunteer leaders, visit PHAssociation.org/PatPaton.
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Checking the
Bucket List Annie McCall, 72, of Victoria, Australia (Oz), was diagnosed with pulmonary hypertension (PH) in 2014. She is a retired sheep farmer and district nurse. Her passions include softball, travel, gem hunting and her family. This is her story. By Annie McCall
I
made a bucket list in my 40s of the places I wished to travel. I crossed Africa off the top of my list when I went with my daughter and a bunch of friends. It was everything that I dreamed of since seeing the film “Hatari!” when I was 12. I crossed more items off my list when my cousin Angie and I visited the Greek Islands and toured Bulgaria by car. My late husband and I holidayed in Bali, China, Singapore, Malaysia and Java because going from West Australia to Asia was more affordable than other destinations. When I was a farmer in my 20s, it was my dream to go caravanning across Australia when I retired. When I met my partner Danny in my late 40s, it was his dream too, and his hobby of gem hunting became mine. Each year, Danny and I camp in our van and
go north during the winter to dig for gems all around Oz. We have found zircons, opals, garnets, citrine, topaz, aventurine, quartz and more. All of my trips and activities are arranged around my sports. I play softball two or three times a year with a team 300 km (186 miles) away. I have flown from Queensland to play softball and left Danny digging for gems. Play Ball Ball Play I’ve played softball most of my life. I started a team of farmer wives when I lived in Esperance. My team played for 10 years before we won gold. I discovered masters softball in the early 2000s. My two daughters, Chelle and Cindy, are over 35 and occasionally play on my team. We played in Sydney and New Zealand for the World Masters and in Tasmania for the Oz masters. My weekly softball game is 75 kilometers (47 miles) away. Chelle and my grandkids play with me, so I make a day of it. It’s a lovely catch-up with them. I also play tennis three times a week with an over-50s group in the nearest town, Mount Beauty. Every year Cindy and I meet in Melbourne at the Australian Open to see the final matches. It costs more than an overseas holiday, but it’s worth it. Unheard-of Diagnosis Unheard-of Diagnosis In 2014, I played in the Victorian Masters Softball in June with no problems. When we started our local season in October, I was suddenly breathless. By November, I could not walk or go up inclines without stopping for breath. I had two occasions of extreme breathlessness in the 1990s. One was when I climbed Mount Augustus in Western Australia with my uncle Geoff. The other time was after white water rafting on the Zambezi
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River in Africa, and we had to climb out of a canyon. After those instances, I continued to play sports, dig for gems, garden and travel. I had no problems visiting the Peruvian Alps and Galapagos Islands in 2013. When I visited my general practitioner about my breathlessness, he said there was nothing wrong with my lungs. I thought it might be my heart. I went to a new GP for an echocardiogram and the scan showed mild PH. I was 67. The GP told me it was nothing serious, but the internet told me I’d be dead in three years — or seven years with medication. By this time, I had contacted the Pulmonary Hypertension Association Australia (PHAA). The support group recommended that I ask my GP for a referral to a PH specialist in Melbourne, seven hours away by public transport. I made an appointment at The Alfred hospital in Melbourne. My right heart catheterization, lung function test and ventilation-perfusion scan showed severe idiopathic pulmonary arterial hypertension (IPAH). I commenced Opsumit and warfarin in September 2015.
flushing or headache, I take a Panadol and go to tennis as planned. It makes the side effects pass quicker. PH has not changed my life too much. But my overnight visits to Melbourne every three months have taken a toll. I am lucky that Angel Flight, free transportation for medical treatment, now flies from Mount Beauty to Melbourne in one hour. Since my diagnosis, more meds have been added, making PAH a chronic disease. I no longer regard myself as having a fatal disease. I consider myself very lucky to take oral trepostinil after participating in a clinical trial because that medication isn’t available in Oz. I continued to travel. In 2017, Danny and I took a cruise and toured Vietnam, Cambodia and Singapore. It was a fantastic experience. In 2018, my daughter, Cindy, and I took my son to Uluru in central Oz. I also glamped across the top end of Arnhem Land in northern Oz and traveled to Fiji with Danny. Then 2020 happened. I had wanted to go through my bucket list items more quickly because of my diagnosis, but the pandemic stopped all that. I had organized a trip with my sister-in-law in September to cruise Galapagos one more time with my PH specialist’s OK. But I may have to be happy that I’ve done it once. Hopefully sport will start soon, and I can visit my family in Queensland again. I’m really happy with the trips that we have already done, and I’m glad we took the opportunity to do them before we got too old. Just do it, I say!
Returning totoSport and Travel Returning Sport and Travel After a few weeks, my exercise capability returned, and I played tennis three times a week. Exercise makes me feel better. If I wake up with nausea,
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30 Years of Volunteering
Left to right: Mary Clare, Tom, Terry and Annie
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hen Terry McGraw was diagnosed Making aa difference Making difference with primary idiopathic pulmonary hypertension (now IPAH) in January 1990, Terry slowly became more involved with PHA as she had never heard of the disease. time went on and the association grew. At first, she “During the first few years after my diagnosis, my started participating on the patient forum on PHA’s husband and I focused on doing all we could to learn website. It helped her connect with other patients about the disease and get answers,” Terry says. and exchange advice. As her kids got older, she Her husband Tom remembers being terrified. He began attending support group meetings and started worried that Terry wouldn’t be around for their two fundraising for the PHA Boston O2breathe Walk. daughters. “The fear of them Through her participation, she not having Terry hit hard,” he learned how vital PHA is to the ‘The fight is not over, and says. But Terry fought, and PH community. Tom began to plan and fight “Because our disease is so rare, I know PHA has the right on her behalf. we need a louder voice, and PHA priorities.’ Terry and Tom are among is a strong advocate that projects the families who have lived — Tom McGraw, caregiver that louder voice — so much with pulmonary hypertension louder than we could muster on (PH) for many years – in our own,” Terry says. their case, decades. At the time, there wasn’t much Tom was slower to get involved. “At first, I was information available about PH, and it took the more focused on Terry and our kids, but as time McGraws about five years to learn about then passed, I realized that PHA was an important ally to fledgling Pulmonary Hypertension Association us.” (PHA). Tom, Terry and their daughters, Mary Clare and Annie, started the fundraising team “Tipperogues for Terry” for the Boston O2breathe Walk. Tom and
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Career Focus Longtime Commitment to PH Treatment
Mary Clare also got involved with the PHA Greater Boston Support Group, eventually becoming co-leaders with Wendy Berry, who has PH. “This disease stinks, but it can be managed,” Tom says. “Over the past 30 years, I have seen firsthand what determination can do when combined with support from family, friends and committed and caring professionals.” Looking back, Terry recalls the looks on the doctors’ and nurses’ faces when she was diagnosed. They told her: “We’re sorry; you have a tough one.” Thirty years later, she says she has many wonderful memories because of great doctors and nurses, friends and family, fellow patients and PHA staff who helped her fight. “I am living proof that you can survive, and PHA was a big part of my journey.”
Join the McGraw family in fighting PH. Visit www.PHAssociation.org/Donate to support PHA’s mission to extend and improve the lives of those affected by PH.
Stuart Rich, M.D., was a cardiology fellow at the University of Chicago in 1978 when he began treating pulmonary hypertension (PH). “My first patient was a young woman who was admitted to the hospital for right ventricular failure,” Dr. Rich recalls. “My chief suggested it would make a good career focus.” Now a cardiologist at Bluhm Cardiovascular Institute at Northwestern Memorial Hospital, Dr. Rich has seen many changes with PH treatment and awareness. He’s also had many memorable experiences. “One of my patients was a teenage girl whom I treated with Flolan,” Dr. Rich remembers. “It was her prom, and her mother managed to tape her CADD pump to her thigh under her dress so that she could wear the dress she wanted without revealing the pump. It was such a great story that she ended up on the cover of People magazine.” Dr. Rich learned of the Pulmonary Hypertension Association (PHA) in 1996 at its second International PH Conference and Scientific Sessions in Atlanta. As PHA grew as an organization, Dr. Rich became involved in many capacities. For the past 15 years, he has helped secure support for the Pulmonary Hypertension Association Registry (PHAR), Pulmonary Hypertension Care Centers (PHCC) and a potential new clinical trial network. Dr. Rich has been an annual donor to PHA for more than 20 years. “Patient support is an essential part of the care of [PH] patients,” he says. “PHA has filled a very important niche for patients to know about their disease and to not feel alone in the world.”
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EMERGENCY PREP Expect the Unexpected
H
urricanes, flooding, wildfires and excessive air pollution in the summer and fall were reminders of the importance of preparing for potential emergencies. Emergency preparedness is especially critical for people with pulmonary hypertension (PH). Your emergency plan will be unique, according to the severity of your PH, other medical conditions, location, support systems and resources. The Pulmonary Hypertension Association (PHA) offers worksheets in English and Spanish to help you create a plan to prepare for emergencies. Here are a few excerpts to get started. Find the worksheets at PHAssociation.org/emergencyprep.
• Stock up on necessities, especially drinking water, food, first aid kits, flashlights and batteries. • Keep a supply of extra distilled water for inhaled therapy devices or CPAP machines. • Ask your PH care team if you can get extra medication and/or supplies (cassettes, syringes, etc.). Some insurance companies will allow early refills for emergencies or disasters. • If you use oxygen, ask your oxygen company to have your tanks filled so you don’t run short. Also ask about extra batteries.
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outage. Work with your health provider to complete/ submit paperwork to ensure your power stays on in an outage. • If you have a central IV line (catheter), keep your cleaning and dressing supplies with you including gloves, mask, alcohol pads, dressings and tape. • Be sure you have a safe place to go and a way to travel if you need to evacuate. If you are driving, obtain a full tank of gas as soon as you learn of an impending storm. • Sign up for emergency alerts.
• Keep a list of all your medications with you at all times. Infusion patients should state that your infused medication should never be interrupted or discontinued.
• Contact your specialty pharmacy if you’re running low on medication, need to change your medication shipment address or have other therapy-access concerns.
• Keep with you a list of numbers for your specialty pharmacy, PH doctor/clinic and emergency contacts.
• Keep your insurance cards with you when you leave home.
• Consider investing in a generator, and learn how to use it. Power outages are dangerous for those on infusion medication and those who use oxygen.
• If you are a member of a PHA support group, exchange numbers with the leader and other members to keep in touch during unexpected events.
• Contact your power company about your health condition so it lists you as high priority in a power
• Discuss and share your emergency plan with your PH care team.
PATHLIGHT // ISSUE 4
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:
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* Mary M. Brady* 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 Terri L. Jakuboski 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* Theresa “Terry” E. (Cavanaugh) O’Reilly* 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
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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.
Carroll Abadie
John E. Hughes
Jim Bakey
Alice A. Hunt
Robert Bastiaanse
Dennis Jackson
Laverne Baum
Agnes V. Jones
Damon Bradtmueller
Betty H. Jorgensen
Glenda S. Breen
William E. Judd
Leonard Burke
Joe Kellams
Jane M. Clark
Kelley M. Kilday
John Connorton
Sandy Kim
Neeoma Coston
Bill Kirtley
Donna M. Dohoney
Shelli Kohan
Lois A. Epifanio
John B. Lee
Louise Evi
Renee Martin
Elise Fischer
Judith Massa
Josephine Fulton
Myrna A. Matheson
Jacqueline M. Godfrey
Joe K. Matthews
Gail M. Hadel Ryan A. Hamby Wilburn D. Hatton Darel L. Hilkin Heidi B. Holub
Grace Mcdonald Scott Mooi Jane Motta Margaret R. Novak Patricia A. Page
Mary P. Partida Gómez Colleen E. Pawelczyk Jeanette E. Pecorelli Jean D. Perkins Phyllis A. Persson Carol K. Primas Lauren RedmonVanderford Keith Saari Paulette Stafford Bonnie Tauss John Terhune Elaine Tobin Patricia A. Tucker Colleen Twarog Tito Villa-Chauvin Marion L. Wagner Darlene Walker Joyce W. Weller Brenda Woolley Christina Zloza
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. PHASSOCIATION.ORG
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Pulmonary Hypertension Association 8401 Colesville Road, Suite 200 Silver Spring, MD 20910
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1991-2021
