Pathlight - Spring 2023

A publication of the Pulmonary Hypertension Association

Artist Focuses on MIND AND BODY WELLNESS

PLUS NEW GUIDELINES ADDRESS

DIAGNOSIS AND TREATMENT 5 TIPS

FOR PEDIATRIC/ADULT CARE

TRANSITION

Doctor Advances

International Collaboration

2023 // Volume 32 // No. 1

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 2023 // VOLUME 32 // NO. 1

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

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Regional Educational Workshops To Resume in May

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How New Guidelines Can Influence PH Diagnosis and Treatment Specialists conduct lung and heart tests, including an echocardiography

13 PHPN Symposium To Feature Lead Author of New PH Guidelines 14 5 Tips for Transitioning From Pediatric to Adult Care

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Find Answers

Learn Why

Get Talking

17 3 Avenues To Find Copay Assistance

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18 Artist Focuses on Mental Health After PAH Diagnosis

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.

24 Doctor's Mission Advances International PH Collaboration

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

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.

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.

16 Advocating for PH Patients in the New Congress

27 Know Your PHA Member Benefits 28 Family Shares Story To Help Other Bereaved Caregivers

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ON OUR COVER Artist Suzanne Miller focuses on mind and body wellness to manage her pulmonary hypertension. This month’s issue explores the state of PH through our article on new recommendations for diagnosis and treatment.

Cover photo: Burgundy Blue Photo.

IN EVERY ISSUE 5

Letter From the Chair

30 QuickTakes 33 Calendar This program is sponsored by Janssen Pharmaceuticals, Inc. ©2021 Actelion Pharmaceuticals US, Inc. All rights reserved. cp-201358v1 0121

34 Passages Knowledge. Understanding. Empowerment.

PHASSOCIATION.ORG 3

PUBLISHER Matt J. Granato President & CEO

BOARD OF TRUSTEES Officers

STAFF Linda Busche Editor-in-Chief Paula Maturana Graphic Designer Karen Smaalders Vice President, Communications and Marketing

CONTRIBUTORS Naya Baharona, Danielle Clement, Jeff Dunn, Courtney Durham, Angela Golden, Haley Johnson, Lauren Intrieri, Daniel Layton, Suzanne Miller, Zangi Miti, Melanie Padgett Powers, Abby Sickles, Jaeger Spratt, Sara Tyghter. Cover story photos: Burgundy Blue Photo.

CONTACT 301-565-3004 PHAssociation.org

SUPPORT LINE 800-748-7274 We’re here to support anyone with a connection to PH. (daytime, please) Submissions to Pathlight are welcome.

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

Headquarters 1629 K St., Suite 300 Washington D.C., 20006

Billing and Mailing Address 8401 Colesville Road, Suite 200 Silver Spring, MD 20910

Tony Lahnston, chair Traci Stewart, R.S.N., C.S.N, CHFN, chair elect Matt Wall, treasurer Nicole Creech, secretary Colleen Brunetti, M.Ed., C.H.C, immediate past chair Matt J. Granato, L.L.M., M.B.A, president and CEO, ex officio

SCIENTIFIC LEADERSHIP COUNCIL

Todd Bull, M.D., Scientific Leadership Council chair elect Murali Chakinala, M.D., FCCP, Scientific Leadership Council immediate past chair Colleen Connor Ramona Doyle, M.D., M.Sc. Anna R. Hemnes, M.D., Scientific Leadership Council chair Kimberly Jackson, R.N., B.S.N., PH Professional Network chair Jesse Kohler Wenninger, Esq. Mitch Koppelman, Ph.D. Michelle Ferdinand Liu, M.D., M.P.H., FAAOA Melissa Magness, M.S.N., APRN, CNP-AC, PH Professional Network chair elect Monica M. Penaranda Diane Ramirez Doug Taylor Delphine Yung, M.D, Accreditation and Registry Committee (PHCC) chair

Anna R. Hemnes, M.D., 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., chair-elect Murali M. Chakinala, M.D., FCCP, immediate past chair Kelly Chin, M.D. Vinicio A. de Jesus Perez, M.D., FCCP 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. Daniel Grinnan, M.D. Kristin B. Highland, M.D., MSCR Tim Lahm, M.D. Deborah J. Levine, M.D. Wes McConnell, M.D. Lana D. Melendres-Groves, M.D. John J. Ryan, M.D., M.B., B.Ch., B.A.O. Sandeep Sahay, M.D. Oksana A. Shlobin, M.D., FCCP Thenappan Thenappan, M.D. Nidhy Varghese, M.D. Corey E. Ventetuolo, M.D., M.S. R. James White, M.D., Ph.D. Timothy L. Williamson, M.D.

Emeriti ex officio

Distinguished Advisers

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

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. Ron Oudiz, M.D.

Trustees

Honorary Carl Hicks President & CEO Matt J. Granato, L.LM, M.B.A PH PROFESSIONAL NETWORK EXECUTIVE COMMITTEE Kimberly Jackson, R.N., B.S.N., chair Cheri Abbott, R.N., CCRP

4 PATHLIGHT

April Blakley, R.N., B.S.N. Jessie Dunne, PharmD, BCPS, BCCP Loida A. Johnson, CRNP Tisha Kivett, R.N., B.S.N. Melissa Magness, M.S.N., APRN, CNP-AC, chair-elect

LETTER from the

CHAIR Dear PHA community: As I read the wonderful articles and stories in this issue, I think about the year 2007 when we lost my niece to this disease. I decided to take an active role in the Pulmonary Hypertension Association (PHA) and quickly learned that there was a lot of work to be done regarding diagnosis and treatment.

At the time, I believe only one or two treatments were available, there was little awareness of pulmonary hypertension (PH) symptoms, and diagnosis was challenging.

I was amazed at the dedication of the medical professionals I was so fortunate to meet. We had a lot of work to do, and we had a great group of committed individuals working hard to push through the difficult challenges facing patients and caregivers in managing their PH worlds.

The spring 2023 issue of Pathlight shows how far we have come since then as well as opportunities ahead. We take a broad look at the current state of PH in the U.S. and worldwide. This issue includes an in-depth look at PH guidelines for treatment and diagnosis that were published in Europe in August 2022. The guidelines are expected to influence PH care and lead to earlier, more accurate diagnosis.

But PH care globally takes more than just research and guidelines. It takes grassroots efforts such those undertaken

by Vinicio de Jesus Perez, M.D., of Stanford University Medical Center. Dr. Perez, a member of PHA’s Scientific Leadership Council, has made it his mission to advance international PH collaboration because research and treatment access vary throughout the world. He aims to bridge the gap between existing care guidelines and the

reality of people with PH wherever they live.

Our inspiring cover story details how artist Suzanne Miller manages her PH by focusing on mind and body wellness. Suzanne tells about her journey, from acute depression over her diagnosis, the loss of her home and possessions, to therapy, PH treatment and acceptance.

In this month’s donor spotlight, we hear from the bereaved family of Joanna Grey. Since Joanna’s 2021 death, her family has been striving to keep her memory alive. With their loss still fresh, Joanna’s family is working to preserve her legacy through PHA. They want to encourage other bereaved caregivers that hope and community can be found in the darkest places.

This issue also shows that the hard work of advocating for PH patients in the new Congress continues! After the 2022 midterm election brought new legislators to Congress, PHA is educating lawmakers about PH and laying out our legislative priorities.

In the 15 years that I have been involved with PHA, the accomplishments and advancements continue. This is why I chose to join this wonderful organization. I am so proud to see, first-hand, the continued successes and advances in diagnosis and treatment for PH patients and caregivers.

A special thank you to the medical community, researchers and the dedicated staff of the Pulmonary Hypertension Association. Let’s keep that momentum rolling through 2023 and beyond!

Tony Lahnston, chair, Pulmonary Hypertension Association Board of Trustees

PHASSOCIATION.ORG 5

HEALTH MATTERS

Reach members of the PULMONARY HYPERTENSION ASSOCIATION Advertise in:

T

PHAssociation.org: Comprehensive resource for anyone affected by PH

Pathlight: PHA’s quarterly member magazine

PHA News: Biweekly patient-focused e-newsletter

PH Community Workshops: New Name, Same Great Content he Pulmonary Hypertension Association (PHA) is bringing back its free regional workshops under a new name. Previously known as PHA on the Road, the events are now called PHA PH Community Workshops.

The community workshops bring together people with pulmonary hypertension (PH), families and caregivers for education sessions on PH care and treatment. The daylong events are designed to connect people in various regions of the country, from the newly diagnosed to long-time thrivers.

If you live on the West Coast, join us May 6 in San Diego for our first community workshop this year. If you live in the Midwest or on the East Coast, join us July 29 in the Boston area.

“PH patients should attend to get a better understanding about our disease,” says Denise Clark, who has PH and has spoken at PHA on the Road. “The [regional] medical minds are in one place where you can pick their brains … as well as listen to their informative talks.”

These invaluable workshops provide information about treatment options, disease management and up-to-date clinical research. Speakers include doctors, PH experts and people with PH. The workshops also set aside time for support group meetings.

Complimentary meals are included.

The San Diego workshop features sessions on: • Understanding your medication and side effects.

• CTEPH and blood abnormalities.

• Ask the Expert Q&A. • Healthy living with PH. • Self-advocacy.

• Clinical research.

Health care professionals also are welcome to attend. Doctors, nurses, therapists and other professionals can connect with patients and families to directly learn about PH from the perspective of those affected by PH. Health care professionals also can take advantage of the opportunity to network with colleagues in the region who specialize in PH.

PHA suspended regional workshops in 2020 and 2021 because of the pandemic and resumed with PHA on the Road last fall in Louisville, Kentucky. The new name, PH Community Workshops, better represents the purpose of these educational events.

It’s not too late to register. Visit PHAssociation.org/ PHworkshop to sign up and find more information.

JOIN US! May 6 The Alexandria at Torrey Pines San Diego

July 29 Boston Marriott Burlington Burlington, Massachusetts

PHAssociation.org 6 PATHLIGHT

Contact: Advertising@PHAssociation.org

PHASSOCIATION.ORG 7

HEALTH MATTERS

HEALTH MATTERS

How New Guidelines Can Influence

PH Diagnosis and Treatment MELANIE PADGETT POWERS is a freelance health care writer and editor in the Washington, D.C., area.

BY MELANIE PADGETT POWERS

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ew European guidelines for the diagnosis and treatment of pulmonary hypertension (PH) include updates on how PH is defined, diagnosed and treated.

The guidelines, published in August 2022 in the European Heart Journal and the European Respiratory Journal, were developed by a task force of the European Society of Cardiology and the European Respiratory Society.

Guidelines in health care are recommendations — not requirements — based on the most updated evidence on how to treat and manage a particular condition or disease.

While these new guidelines are European, they could be used by health care providers around the world. In the U.S., organizations such as the American Heart Association and American College of Chest Physicians

have not changed their guidelines or the PH definition.

“I don’t think that we can fully apply everything from these guidelines to the United States, but they certainly will influence practice in the United States,” said Anna Hemnes, M.D., PHA’s Scientific Leadership Council chair and associate professor of medicine and assistant director of the Pulmonary Vascular Center at

Vanderbilt University Medical Center in Nashville, Tennessee.

“We use the guidelines as practitioners to benchmark our care for patients, but there are reasons that we may differ from guidelines in any specific patient — because a guideline that’s meant to address the vast majority of patients can’t address every single patient and their own complexity, ultimately,” Hemnes said.

The last European guidelines on PH were published in 2015, so it was time for an update, according to Marc Humbert, M.D., Ph.D., the lead author of the guidelines. Humbert is director of the French Pulmonary Hypertension Reference Center, professor of respiratory medicine at the University of Paris-Saclay in France and president of the European Respiratory Society.

“Pulmonary hypertension is a very important topic in medicine because it is fast moving, fast progressing,” Humbert said. “There have been a lot of important discoveries in the last seven years, and it is really important to regularly update the guidelines to match the current evidence.”

CHANGING THE DEFINITION OF PH

The guidelines start with a change to the definition of PH. Pulmonary hypertension is high blood pressure measured by the force of blood flowing through the lungs. It is measured by right-heart catheterization.

International and U.S. guidelines consider pulmonary artery pressure to be normal at 8-20 mmHg. In previous guidelines, it was considered abnormally high if the pressure was more than 25 mmHg at rest.

However, Humbert pointed out, the measurement from 21 to 24 mmHg was considered abnormal but wasn’t included in previous definitions. “There was a gray zone which did not define pulmonary hypertension, but everybody knew that these values were not normal,” he said.

“For many patients, these values reflect early pulmonary vascular disease, and these patients will need to see a specialist regularly to keep an eye on the evolution of their condition,” Humbert said. “This is particularly

Specialists conduct lung and heart tests, including an echocardiography.

The new guidelines recommend a three-step screening algorithm for PH. Ideally, the process would help clinicians diagnose PH earlier.

If the specialist suspects PH, they should refer the patient to a PH center for further diagnosis and evaluation.

Health care providers should refer patients to a pulmonologist, cardiologist or other specialist if they can't identify a common respiratory or cardiac reason for the patient's shortness of breath.

8 PATHLIGHT

PHASSOCIATION.ORG 9

HEALTH MATTERS

HEALTH MATTERS

the case in patients with increased risk of pulmonary hypertension such as scleroderma or when there is a past history of pulmonary embolism.

“It doesn’t mean that you need a drug, but it means that you need attention,” he continued. “We want to make it clear that the upper limit of normal is 20 mmHg. Any value above 20 mmHg is abnormal. Thanks to PHA and to other government and non-government organizations, there are many specialists of pulmonary hypertension worldwide who are available to give the best counseling to the people who have elevated pulmonary artery pressure.”

NEW WAYS TO SCREEN AND DIAGNOSE

The guidelines include a new three-step screening algorithm for PH. This is an attempt to diagnose PH earlier. The first step acknowledges that most PH patients first visit a health care provider complaining of

shortness of breath. If the clinician can’t find a common respiratory or cardiac reason for shortness of breath, the patient should be referred to a specialist, usually a pulmonologist or a cardiologist. The guidelines recommend that specialists conduct lung and heart tests, including an echocardiography, which is an ultrasound to look at the heart. If PH is suspected, the patient should be referred to a PH center for further diagnosis and evaluation.

The guidelines also present a new risk stratification for pulmonary arterial hypertension (PAH), also known as Group 1 PH. PAH is when the arteries in the lungs become narrowed, thickened or stiff. A risk stratification is a process that allows physicians to assign a level of risk to a person with a certain condition, to help determine the proper treatment.

Hemnes said it’s important for patients to know that risk stratification is commonplace in treatment, even if

different clinicians use different types of risk tables.

“We’ve gotten more evidence based in our ability to use these risk stratification tools to guide therapy in the last several years, and that’s been incorporated into these guidelines,” Hemnes said. “Exactly what tool people use may be different based on their preferences and on available data or just their read of the literature, but in general, incorporation of some sort of risk stratification tool in decisions about what therapies should be used are now considered standard of care.”

The PAH risk stratification at initial diagnosis divides patients into low, intermediate and high risk categories.

“The risk stratification table is one of the most important additions in the guidelines for the community,” Humbert said. “We really want to make sure that everybody will riskstratify Group 1 PH before treatment — because when you have a high risk of mortality at one year, there is a

Variations in PAH Severity A Healthy Pulmonary Artery

B Mild PAH

C Moderate PAH

D Severe PAH with Plexiform Lesions

Adventitia Media Intima

Adventitia Media Intima

10 PATHLIGHT

Arteries are healthy and flexible, blood flows easily through the vessels.

Thick and stiff artery walls limit blood flow and increase the resistance.

Blood flow is restricted as the artery narrows.

Advanced vascular lesions (abnormal growth) and thrombi (blood clots) form.

'This is a little different from prior guidelines — that this PH with comorbidities even exists — and I think that particularly will need to be evaluated in the context of the United States.'

Anna Hemnes, M.D., chair, PHA Scientific Leadership Council

recommendation to treat you upfront more aggressively.”

Currently, the three potential drug treatments used in the initial treatment strategy are endothelin receptor antagonists, phosphodiesterase type 5 inhibitors and prostacyclin derivatives.

“For the severe [PAH] patients who have more than 20% risk of mortality at one year, you need to start with a combination of three drugs, including a prostacyclin derivative used subcutaneously or intravenously,” Humbert explained. “But for the people who have a low or intermediate risk, we usually start with initial combination therapy of two oral drugs: one endothelin receptor antagonist

and one phosphodiesterase type 5 inhibitor.”

At follow-up visits, the risk stratification expands from three to four categories: low, intermediate-low, intermediate-high and high risk.

The goal is for the treatment to move patients into the lowrisk category. The follow-up risk stratification process has been simplified to include three mostly non-invasive measures. They include the World Health Organization’s “functional class,” which describes the severity of a person’s PH symptoms.

The other two are a six-minute walk test and a NT-proBNP test, which is taken from a blood sample.

When needed, other tests such as echocardiography and right-heart catheterization can be added to better estimate the situation, Humbert said.

“We have divided the intermediate risk into two subgroups: intermediatelow and intermediate-high risk because intermediate risk represents around two-thirds of the patients treated,” Humbert said. “You have some intermediate risk patients who are doing better than others.

Distinguishing these two categories is important at follow-up with a different treatment approach which will be more aggressive in the intermediatehigh risk.”

'Many people with Group 1 pulmonary hypertension are young, fit, and they have no comorbidities. They are the traditional patients we used to see in the 1990s. But ... with better awareness of PH, we see more and more elderly patients — age more than 65 — with cardiopulmonary comorbidities.'

Marc Humbert, M.D., Ph.D., director, French Pulmonary Hypertension Reference Center PHASSOCIATION.ORG 11

HEALTH MATTERS

HEALTH MATTERS

Functional Classes Class I

Class II

You have no symptoms during ordinary physical activity.

You are comfortable at rest, but your ordinary physical activity is somewhat limited because of breathlessness, chest pain, fatigue or dizziness.

Class III

Class IV

You usually have no symptoms at rest, but breathlessness, chest pain, fatigue or dizziness greatly limits routine activity.

CONSIDERING COMORBIDITIES

The guidelines also introduce the concept of considering comorbidities in the treatment algorithm.

Comorbidities are a person’s other health conditions or diseases.

“This has driven a lot of debate,” Humbert said. Comorbidities that the guidelines ask providers to consider, especially among elderly patients, are risk factors of left heart disease (obesity, diabetes, high blood pressure and coronary heart disease) and risk factors of lung disease (significant smoking history with alterations of the lung function). The guidelines suggest that these patients be treated with more caution because of their other conditions, starting first with monotherapy, or just one medication.

This can be followed by adding in other medications.

Comorbidities such as obesity have much higher rates in the U.S. than in Europe. In the U.S., 42% of adults 12 PATHLIGHT

You are often breathless and tired even while resting. You can’t do any physical activity without symptoms. You show signs of right heart failure. Anyone prone to fainting would be in this class.

are considered obese, according to the Centers for Disease Control and Prevention. About 23% of adults in Europe are obese, according to the World Health Organization, although that number has been climbing.

“It may not be appropriate to treat obese PAH patients with monotherapy,” Hemnes said.

The guidelines have been endorsed by the International Society for Heart and Lung Transplantation and the European Reference Network on rare respiratory diseases. The guidelines likely will be part of the discussion at the 7th World Symposium on Pulmonary Hypertension, Hemnes said. The event will take place in June 2024 in Barcelona, Spain.

The 2024 symposium will include pulmonary vascular disease experts from all over the world, including Humbert. Hemnes anticipates some revisions to the European guidelines as new literature is published before

the 7th World Symposium and as the PH community considers the implications of the European guidelines on their practices. “World Symposium proceedings are extremely important to clarify some aspects of the guidelines,” Humbert said. “They always help refine and improve the next updated guidelines.”

‘The risk stratification table is one of the most important additions in the guidelines for the community.’

PHPN Symposium To Feature Lead Author Of New PH Guidelines

P

ulmonary hypertension (PH) health professionals can meet the primary author of recently released PH guidelines at the PH Professional Network Symposium. The Pulmonary Hypertension Association (PHA) event is Sept. 28-30 in Arlington, Virginia.

Marc Humbert, M.D., Ph.D., will discuss new guidelines for diagnosing and treating PH in one of the symposium’s general sessions. The guidelines, developed by a taskforce from the European Society of Cardiology and European Respiratory Society, were published in August 2022. They include updated hemodynamic definitions of PH and pulmonary arterial hypertension (PAH) that might influence early detection strategies; updated diagnostic algorithms; and recommended testing.

Humbert is the lead author of the 2022 guidelines and director of respiratory and intensive care at the French Pulmonary Hypertension

Marc Humbert, M.D., Ph.D.

Jeffrey Lauzon, M.A., Ph.D.

Reference Centre in Paris. See Page 12 for a comprehensive article on the new guidelines.

The two-day event will include a session on wellness and mental health for pulmonary hypertension (PH) providers and patients. Joan Cain, M.S., FNP-BC, ACHPN, and Jeffrey Lauzon, M.A., Ph.D., will address patient reaction and provider fatigue related to traumatic events, as well as trauma diagnostic criteria, prevalence rates and treatment recommendations.

Their presentation also will offer tips to help patients to have control in navigating their chronic condition.

Cain is a nurse practitioner and adjunct faculty member at the Medical University of South Carolina.

She works with the palliative care department and multidisciplinary health care teams in a 700-bed hospital.

Lauzon is a licensed clinical psychologist at Aurora Health in Milwaukee, Wisconsin. He develops cardiac psychology services in his postresidency fellowship and his clinical practice (inpatient and outpatient) at St. Luke’s Medical Center heart failure and abdominal transplant clinics.

He works closely with PH patients through the heart clinic.

This year’s PHPN Symposium, “PHoundation for a Brighter Future,” will focus on how to deliver high

quality, patient-centered care through multidisciplinary collaboration.

The PHPN Symposium attracts hundreds of health care professionals to learn from renowned PH experts.

Joan Cain, M.S., FNP-BC, ACHPN

Join more than 500 health care professionals for opportunities to network with your peers, earn continuing education credit, and engage in need-to-know topics.

All health care professionals are encouraged to attend. Registration opened in March.

Find more information, including speaker bios and session information, at PHAssociation.org/ symposium.

PHASSOCIATION.ORG 13

HEALTH MATTERS

HEALTH MATTERS

5

Tips To Transition From Pediatric to Adult Care

Transitioning from pediatric to adult pulmonary hypertension care can bring new questions and challenges for young adults with PH and their families. “It’s totally natural to have a hard time letting go,” says Anna Brown, D.N.P., CPNP, of Vanderbilt University about changing care.

Brown shared transition tips at PHA 2022 International PH Conference and Scientific Sessions. Her session “The Gray Area: Navigating from Pediatric to Adult Care,” also featured tips from Meghan Cirulis, M.D., of Intermountain Healthcare, and parent and caregiver Jody Petry.

14 PATHLIGHT

Prepare early for the transition. It can be difficult for families to transition from the pediatric world, Brown says. There’s not a hard cutoff age, but the transition to adult care typically occurs between 18 to 22 years old. Speak with your provider about the best time to start your child’s care transition.

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An important step to prepare your child is to give them ownership of their disease and medications, Dr. Cirulis says. When the child is about 13, slowly shift each clinic visit to the child to help them gain ownership, Brown recommends. Your child’s care team can do this by meeting

one-on-one with the child before meeting together as a family. In the one-on-one, the care team should ask the child how they’re feeling and about their medications. You also can help the transition by giving your child more responsibility in managing medications and changing their central line.

Meet with your adult care team prior to transition.

Have open dialogue and ask questions when you and your child first meet your adult team to make sure you’re on the same page about care. Once a young adult starts an adult PH program, some doctors will speak directly to the child and refuse to talk to the

2

parents, Brown says. Schedule a routine visit with the adult care team to meet everyone in case your child has an emergency or needs hospitalization. If your child is 23 or older, the children’s hospital could refuse to admit them, Brown says.

Discuss milestones. When transitioning care, pediatric and adult physicians ideally will share a patient’s history and journey. Although your new care team can look at records, it’s helpful to have an organized synopsis of the child’s medical records in a binder or booklet, Dr. Cirulis says. It’s also important to discuss the milestones of your child’s PH journey with your child so they understand why decisions were made for their care and treatment. That will help them make their own care decisions in the

3

future, such as those about treatments that have and haven’t worked.

Be transparent with your child. It’s hard to be a teenager and manage a chronic disease at the same time. As your child grows older, tough discussions, such as those about birth control and pregnancy, can come up. It’s easier on the child if parents are transparent from the beginning, Brown says.

4

She recommends having conversations when your child is young, then have those conversations again when they’re older. Speaking to your child isn’t going to change the disease but it’s going to change your relationship with your child in a good way, Brown says.

Discuss future postpediatric care. Once your child turns 18, consider becoming your child’s power of attorney. Jody is her daughter’s power of attorney in case she becomes incapable of making decisions. Her daughter knows her mom’s power of attorney is only in case of an emergency.

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Power of attorney is different than guardianship, Dr. Cirulis says.

Guardianship gives parents full control of the child’s care after they turn 18. A medical power of attorney allows parents to make medical decisions if the child can't.

For additional resources, view PHA’s Transitioning From Pediatric to Adult Care guide.

PHASSOCIATION.ORG 15

ADVOCACY

ADVOCACY

Get Involved

Advocating for PH Patients In the New Congress

T

he 2022 mid-term election brought in a new wave of legislators to Congress. The Pulmonary Hypertension Association (PHA) remains committed to bringing forward the concerns of the PH community as Congress puts forth legislation this year.

PHA began the new year by sending welcome letters to newly elected officials, educating them about pulmonary hypertension (PH) and laying out PHA’s legislative priorities for the 118th session of Congress.

Read about our priorities: HEALTH CARE DRIVEN

BY CLINICAL JUDGMENT

Too often, insurers restrict access to physician-prescribed treatment through prior authorization requirements and step therapy policies.

PHA continues to support the Safe Step Act, which would allow doctors in some cases to bypass step therapy requirements. Those situations include when patients have tried lowertier therapies or when delaying the 16 PATHLIGHT

prescribed treatment would harm the patient.

Last year, the Safe Step Act gained significant congressional support but wasn’t brought forward for a vote.

PHA will work with other advocacy groups to reintroduce the bill and gather enough support in the House and Senate to approve the legislation.

PH RESEARCH AND AWARENESS

FINANCIAL ASSISTANCE FOR MEDICATION COPAYS

SUPPLEMENTAL OXYGEN ACCESS

Copay costs can impede PH treatment even when health insurance covers the medication and less expensive generic versions are available.

PHA supports the Help Ensure Lower Patient Copays (or H.E.L.P. Copays) Act, which was introduced in the House in late 2021. The bill would close loopholes some insurers have used to “double-dip.” That means they accept copayments from charitable assistance foundations and other third parties, but they don’t count the copayments toward out-of-pocket deductibles.

Each year PHA asks Congress to fund the National Institutes of Health to the fullest extent possible so it can allocate more money for PH research.

PHA also supports Centers for Disease Control and Infection funding for PH awareness to secure accurate and timely diagnosis.

Supplemental oxygen is critical for many in the PH community. Oxygen equipment covered by insurance often is cumbersome, and their bulk and weight make activities outside the home difficult. Liquid oxygen, typically the best option for people with high-flow needs, has become so hard to access that many clinicians no longer prescribe it.

PHA urges Congress to ensure people who need oxygen receive it in the manner that best suits their needs and improves quality of life.

Does your member of Congress know about pulmonary hypertension (PH)? Lawmakers often aren’t aware of PH. And they don’t understand its severity until people affected by the disease share their stories.

While Pulmonary Hypertension Association staff can explain how legislation would affect people with PH, personal experiences from patients, caregivers and health care providers are more compelling. That’s why it’s important to connect with your elected officials, share your experience and explain the real-life impact of proposed policies.

Use PHA’s Action Center to share your story, and look up and email your Congress members. Visit PHAssociation. org/advocate.

If you’re interested in deepening your connection with your legislators through a phone call or video meeting, email Advocacy@ PHAssociation.org, or call 301-5653004 x758.

3 Avenues to Finding Copay Assistance

S

ecuring financial assistance for pulmonary hypertension (PH) medication copayments can be challenging. The best options for financial aid differ according to each person’s health insurance coverage and prescribed treatment.

Use this roadmap to begin your search: CHARITABLE ASSISTANCE FOUNDATIONS

Nonprofit foundations are common sources of copay assistance.

Pay attention to the assistance offered and the eligibility requirements when applying for a particular grant. Some grants cover specific dollar amounts, while others ensure as much coverage as you need for the year. Other sources limit eligibility only to those with Medicare or Medicaid, while others accept applicants regardless of insurance type.

Tip: Sign up with Fund Finder to get notified as soon as a grant opens up.

Visit fundfinder.panfoundation.org or call 866-316-7263 to register for email and/or text notifications when PH grants become available.

MANUFACTURER ASSISTANCE PROGRAMS

Drug manufacturers sometimes provide coupons or discounts on medications. The discounts more frequently apply to people with commercial health insurance, typically provided through an employer.

However, some manufacturers also help uninsured or under-insured people covered by Medicare. Always check whether the maker of your medicine offers an assistance program, especially if all PH grants are closed when you need help.

Tip: Visit PHAssociation.org/help to see available manufacturer programs listed alphabetically by medication brand name.

SPECIALTY PHARMACY HARDSHIP PROGRAMS

Typically offered as a last resort, specialty pharmacies sometimes provide assistance to patients who have no other options. When contacting your specialty pharmacy, it’s important to tell them that you’ve attempted to secure funding from nonprofit grants and the manufacturer, but no assistance is available.

Tip: Contact your care team as soon as you know you might have trouble covering your copay. Many PH care teams are experienced in finding copay coverage for patients and can be a great resource.

PHA’s Treatment Access program is here to help you find financial assistance. Call 301-565-3004 x758 or email Insurance@ PHAssociation.org.

PHASSOCIATION.ORG 17

FEATURE NEWS

FEATURE NEWS

Mind and Body Wellness

Artist Focuses on Mental Health After PAH Diagnosis

ARTIST SUZANNE MILLER, 41, lost hope after her pulmonary arterial hypertension diagnosis. When she no longer could work, she became homeless and lost everything. Thanks to therapy, her pulmonary hypertension specialist, and friends who took her in, she and her dogs Kate and Leo now have a home. They live in Thousand Oaks, California, with her friend Steve and his family. Suzanne, who uses flowers and plants to make living art, enjoys gardening, painting and collecting old books for pressing flowers.

BY SUZANNE MILLER

W

hen I was 16, I visited a primary care physician who specialized in diet and nutrition. After talking with him at my appointment, I was prescribed phentermine and diethylpropion diet pills to help me lose weight. I started a regimen of pills and a strict diet. I lived in an affluent community where vanity was valued more than good sense. After I lost 30 pounds, I was a “Westlake Girl.” I was thin, tan and I fit in.

As I grew up and continued to take diet pills, my weight and moods fluctuated. While I was in college, I was prescribed Adderall to treat ADHD. The Adderall seemed to help my weight and mood swings. I was in my last semester of college, studying for my final exams when my insurance had changed, and my Adderall was no longer covered. I was desperate

18 PATHLIGHT

and went to my marijuana dealer for something to get me through my exams. He gave me methamphetamines and warned me to stop taking the meth as soon as my finals were over.

I didn’t take his advice. I became addicted to methamphetamines for seven months. After entering a rehabilitation program for young women, I beat my addiction.

Little did I know that the drugs I took for seven months potentially could damage my future health.

Though I stopped taking meth, I continued taking diet pills and Adderall on and off for 20 years.

LIVING ART

After college, I became an artist, and my medium was living walls made from succulents and colorful sustainable botanicals. The work was

long, strenuous and demanding. I worked day and night, slept little and ate only when forced by family or friends.

In January 2019, I came down with a flu that developed into pneumonia.

After my recovery, I continued to work as much as I could and eventually moved into an artist community where I felt my business could thrive. The three-story condo with a workshop on the first floor and a rooftop garden seemed very appealing.

As time went on, I found it increasingly difficult to manage the six flights of stairs within the condo. I made an appointment with the doctor to discuss my shortness of breath and lack of energy. The doctor treated me for asthma and prepared me for the possibility of stomach cancer.

PHASSOCIATION.ORG 19

FEATURE

PHA 2022 FEATURE

JOURNEY TO DIAGNOSIS

I took little stock in this diagnosis and didn’t inform my family. I continued with the breathing treatments, but they didn’t seem to help. I couldn’t work, sleep or eat, and my symptoms worsened.

In July 2019, I was supposed to attend a going-away party for a friend, but I couldn’t get out of bed.

I must have been lying there for over 24 hours when my mother found me unresponsive and blue. She called 911, and I was rushed to Community Memorial Hospital.

While I was minutes away from succumbing to this unknown illness, a doctor passing through the emergency room stopped in.

20 PATHLIGHT

Fortunately for me, this doctor was a pulmonologist and recognized the signs of pulmonary hypertension (PH). He told the emergency room doctor to order an echocardiogram of my heart. The EKG confirmed his suspicions. The hospital wasn’t equipped to treat PH so the next day I was transferred to Cottage Hospital in Santa Barbara.

There, I met my PH specialist, Jeffrey Sager, M.D. After numerous tests, I learned I had an even more rare disease, pulmonary arterial hypertension (PAH). For the next nine days, I remained in the intensive care unit, where I was treated and educated about changes I needed to make in my life because of this dreaded disease.

In the ICU, I learned about the link between stimulants and PH. Dr. Sager told me to stop taking all stimulants, including Adderall and diet pills. He suspected my seven months of meth usage combined with 20 years of Adderall and diet pills led to my PAH. It shocked me that something I had taken when I was in my early 20s was affecting me in my late 30s.

As I prepared to leave the ICU, the staff handed me a 300-page manual on PH and sent me on my way. I didn’t realize the life I had known was over. I experienced daily pain and was prescribed Veletri through a Hickman catheter plus oral Adempas and Opsumit.

PHASSOCIATION.ORG 21

FEATURE

FEATURE

My outlook on life was bleak. I was depressed, angry and felt worthless. I went to the internet for answers and went down a rabbit hole that told me I needed a double lung transplant to live longer.

That ended up not being the case, but I faced multiple complications during the first couple of months acclimating to my life with PAH. After leaving the hospital, I lost everything and became homeless. I was disabled, living out of my car and trying to manage my new diagnosis both physically and emotionally.

I continued to struggle with my mental health and could not see a light at the end of the tunnel. I thought that if this was the life I was going to live, then I did not want to live at all.

As I began seeing my doctor to care for my PAH, I also began seeing a therapist to help deal with my emotions. I discovered the Pulmonary Hypertension Association (PHA) online forums and Facebook groups, which taught me more about how I could thrive while managing with my PH. In 2022, my friend Steve and his family took me in. They gave me a safe space to mourn my old life and establish my new one. Things finally made a change for the better.

22 PATHLIGHT

ONE STEP AT A TIME

To others who recently have been diagnosed with PH, make sure you have a good medical support system. If I have learned anything, it’s that your mental wellbeing is just as important as your physical health. Receiving the care your need for your mental health after your diagnosis is just as important as your regular treatments. With a good care team, you can understand your own PH and focus on your own path.

Take time for yourself to adjust to the changes that will need to take place in your life and for those around you.

Stay connected with the pulmonary hypertension community, whether it's on Facebook, online chat forums or through the Pulmonary Hypertension Association. Know that everyone’s PAH story is different and everyone has different struggles.

Since my diagnosis, I’ve been shifting my outlook to a more positive state of mind and I’ve set small, attainable goals that have helped me through the past few years of my PAH journey.

In times like this, it feels impossible to maintain a sense of hopefulness or optimism about the future, with or without a PH diagnosis. But as uncomfortable as it may feel, I’m continually pushing myself to imagine

a better, brighter, healthier future by taking one small step at a time.

I’ve learned to see the world for what it is and appreciate what I have accomplished and what I still can do.

Although the world is suffering, it also is full of brave people. I choose to be brave, and you can too.

Research continues into the causes and development of PH, including stimulants.

Learn the connection between stimulants, like methamphetamines, and PH at PHAassociation.org/ pha-free-materialstore.

A PH diagnosis can negatively affect mental health. Depression affects about 5% of the general population but can be as high as 26% among people with PH. Learn more about PHA’s mental health resources at PHAssociation.org/patients/ living-with-ph.

PHASSOCIATION.ORG 23

FEATURE

FEATURE

Doctor’s Mission Advances International PH Collaboration

Vinicio de Jesus Perez, M.D., of Stanford University, seeks to improve understanding of PH related to infectious diseases.

O

n May 5 each year, the pulmonary hypertension (PH) community celebrates World PH Day. World PH Day recognizes that PH is a global disease, affecting people regardless of age, gender, ethnicity or background.

Like the difference in culture, climate 24 PATHLIGHT

and customs globally, the PH patient experience varies from country to country.

Vinicio de Jesus Perez, M.D., has seen this firsthand. After meeting his first PH patient in 2000 while completing his residency at Massachusetts General hospital in

Boston, Dr. de Jesus Perez sought out PH medical experts to learn more.

When he saw little literature on the disease existed, he knew his future was in expanding the knowledge and treatment of PH.

PH BEYOND THE U.S.

After completing his fellowships at the University of Colorado in Denver, Colorado, and Stanford, Dr. de Jesus Perez joined the Stanford faculty in 2010. Through his research and teachings at Stanford, he gained a better understanding of the biology of PH and how potential therapies could be developed. He also learned the role of international PH scientists and researchers.

“Most of the research effort has been led by Europe and the United States, at least when it comes to publication,” says Dr. de Jesus Perez. “However, that does not mean that other countries are not taking care of their patients or doing research.”

Research isn’t the only thing that varies from country to country.

“In the U.S., there is a heterogeneity in the drugs that are available to manage PH. But this is not the case for other countries,” says Dr. de Jesus Perez. “In some countries, patients have access to most if not all the drugs routinely prescribed in the U.S. and Europe. In other countries, patients lack access to therapies that we would consider standard, such as combination therapies, because they are not covered by the government.”

For Dr. de Jesus Perez, this is a huge problem. In recent years, he has helped develop a shared knowledge base for the international community. That knowledge base aims to bridge the gap between existing care guideline and the reality of people with PH throughout the world.

“To me, bringing together colleagues from around the world is a big priority. There is clear variability in the patient experience and in access to drugs. PH related to infectious diseases, like HIV, or related to high altitude is not something that we often do not see in the U.S. or in our clinical studies. However, infectious diseases and high altitude are leading causes in PH in the global community. But this is not what our research reflects.”

BRIDGING INTERNATIONAL GAPS

Dr. de Jesus Perez is committed to creating collaborative environments for medical professionals around the world.

In 2020, he joined Ghazwan Butrous, M.D., Ch.B., Ph.D., of the U.K., and Magdi Yacoub, O.M., F.R.S., of Egypt, to organize the first Infection and Pulmonary Vascular Disease consortium. As part of the Pulmonary Vascular Research Institute’s annual meeting, the consortium brings together clinicians and scientists from Africa, Asia, the PHASSOCIATION.ORG 25

NEWS

FEATURE

Know Your PHA Member Benefits Middle East, Europe, the U.S. and Latin America to discuss priorities in research and care for PH related to infectious diseases.

At the first meeting in 2020, 15 people attended. At the most recent meeting in 2022, about 150 medical professionals from around the world were present.

“Creating and attending the consortium made me realize my level of familiarity with the field needed to change,” says Dr. de Jesus Perez.

“When you step outside of the U.S., you realize the reality of what our colleagues are dealing with in their communities is much different than what we are used to. I really felt like I had to learn about PH from scratch by talking to my colleagues.”

'When you step outside of the U.S., you realize ... what our colleagues are dealing with in their communities is much different than what we are used to.'

MAKING AN IMPACT IN THE AMERICAS

Originally from Puerto Rico, Dr. de Jesus Perez is engaged with the Latin American PH community.

Making an impact within the Latin American community has long been one of his goals.

Through his collaboration with PH patient societies in Central and South America, Dr. de Jesus Perez has helped the organizations connect with 26 PATHLIGHT

Latin American health care leaders to discuss pediatric and adult PH needs.

Dr. de Jesus Perez also helped facilitate a regional meeting of patient societies to discuss educational opportunities for patients and scientists in Latin America. The first professional meeting took place in 2017 in Peru.

“This meeting was the first time that we brought PH experts from all of Latin America in a room together,” says Dr. de Jesus Perez. “Unlike smaller, regional meetings that were already occurring, there is now the opportunity to build combined lectures for both the pediatric and adult PH community.”

His impact has gone further than just the region. As a result of the Latin American meetings, Stanford has begun an educational collaboration with PH care centers in Latin America. This partnership will help both parties learn from each other and move towards a future of shared research and knowledge.

“I’m hopeful that in the next 20 years, there will be more representation from colleagues in Latin America, Africa, the Middle East and Asia. There is a lot of great science happening in these countries and there is great expertise,” Dr. de Jesus Perez says.

“Most importantly, I’d love to see more PH medications become available around the world. At the end of the day, that’s what we are all hoping for.”

The Pulmonary Hypertension Association is one of many organizations throughout the world committed to promoting PH awareness, research and earlier diagnosis. To learn more about World PH Day, visit PHAssociation.org/ worldphday. To learn more about international World PH Day activities, visit WorldPHDay.org.

A

re you taking advantage of your Pulmonary Hypertension Association (PHA) membership benefits? In addition to your print subscription to Pathlight magazine – published exclusively for PHA members -- you receive the following benefits:

GENERAL MEMBER BENEFITS

• In-person and online patient and caregiver support.

• Toll-free patient support Line at (800) 748-7274.

• Financial assistance and treatment-access resources.

• Discounted registration for PHA’s International PH Conference and Scientific Sessions. Eligibility for conference scholarships.

• Biweekly newsletters.

• Free copy of “Navigating Pulmonary Hypertension: A Guide for Newly Diagnosed Patients.”

PROFESSIONAL BENEFITS

• Professional development training and workshops.

• Networking opportunities.

• Free or significantly discounted registration for webinars and events.

• PHAR patient registry and the Pulmonary Hypertension Care Center network.

• Advances in Pulmonary Hypertension journal.

To continue providing these benefits, PHA raised its annual membership rates on March 1. The increase is the first in more than a decade. General member fees increased to $25 a year from $15.

Family memberships increased to $50 from $35.

Professional memberships also increased. For members of PHA’s Pulmonary Hypertension Clinicians and Researchers, the rate went up to $175 from $150. The rate for members of PHA’s Pulmonary Hypertension Professional Network rose to $115 from $95.

New prices will start on your next renewal date after March 1.

New multi-year, discounted rates are now available. Join or renew at PHAssociation.org/join.

People with PH, caregivers and family who have financial difficulties can request free general membership. Email us at memberships@phassociation.org, or call 301-565-3004 x833.

PHA members enjoy discounted conference registration, including those who participated in the PHA 2022 fashion show (seen above).

PHASSOCIATION.ORG 27

FEATURE

FEATURE

Family Shares Story To Help Other Bereaved Caregivers

Joanna had a great experience when she went to see Jonathan Van Ness’ comedy show at the Orpheum Theatre in Boston in 2019. Joanna, her husband Jeff and her sister Nicky had balcony seats, so Nicky called the box office before the show to make sure Joanna could safely and easily get to her seat. At the theater, an employee escorted them inside, invited them to join a meet-and-greet with Jonathan, and put them first in line. Joanna was thrilled when Jonathan said her hair was gorgeous and complimented the backpack for her IV pump.

diagnosed with idiopathic pulmonary arterial hypertension (PAH). Her family had heard of hypertension, but not PH. Joanna and Jeff.

I

n 2016, Joanna Grey and then-fiancé Jeff Dunn were walking through their Boston-area neighborhood when she was hit with significant chest pain. She thought she was having a panic attack, so her family convinced her to see a doctor who walked her through how to manage anxiety and stress.

Joanna, who kept active through neighborhood walks, regularly running and attending spin class, thought the advice made sense. She had been experiencing breathlessness and chest pain for about a month but pushed through as much she could.

Doctors attributed her symptoms to panic attacks. But her symptoms continued, and she suspected something was wrong. She was diagnosed with PH later that year.

Jeff and other members of Joanna’s family shared her story with the

28 PATHLIGHT

Jeff, Joanna and their kids (bottom row from left): Dawson Grey and Erin, Karly and Olivia Dunn.

Joanna and Dawson Grey.

Pulmonary Hypertension Association (PHA) as they grieve her 2021 death from complications due to transplant surgery. They want to encourage other bereaved caregivers that hope and community can be found in the darkest places.

about going to the emergency room.

They were discouraged to go and told it would be a waste of time.

JOURNEY TO DIAGNOSIS

Throughout 2016, Joanna had more episodes of breathlessness and dizzy spells. She once passed out while walking up the stairs, but her doctor said it was stress. When she told her family about fainting, they urged her to seek medical help.

Jo went back to the doctor and was placed on a heart monitor but was sent home. Later that evening, she had another episode. Jo and Jeff rushed to urgent care only to be told again that she had anxiety. With urgent care resources limited, the couple asked

LIFE AFTER DIAGNOSIS

TURNING POINT

Nov. 20, the day after Jo’s birthday, was like any normal day for her and Jeff until Joanna collapsed in the entrance of a Target. After being rushed to their local hospital in Norwood, Massachusetts, a doctor mentioned the possibility of pulmonary hypertension.

“They talked around the disease like, ‘We think it might be pulmonary hypertension,’ Jeff says. “Then a doctor came in and dropped a whole bunch of stats, price, costs and stuff like that. It was a horrifying experience.”

Joanna was transferred from Norwood to Beth Israel Hospital in Boston, where she immediately had a right heart catheterization and was

Jo tried many different medications to help with her symptoms, including sildenafil, Opsumit and Uptravi.

She switched often because the medications didn’t work as well as they had hoped. Jo experienced many of the side effects listed for each medication: “Leg pain, nausea, diarrhea, it was every day,” Jeff recalls.

Jo moved to intravenous medications like remodulin and Veletri. The switch was daunting, but Jeff took over her care and made sure everything was done properly.

“It was my way of controlling the uncontrollable,” he says.

Jo powered through her discomfort and fatigue as she continued to work full time and be a mom, wife and sister. Very rarely did Joanna share her reality as someone who was sick with an invisible illness.

“She’d go to work, not be late, be sick at work, and call me to bring her a change of clothes so she could keep

going,” Jeff says.

In 2019, after battling her employer for accommodations, Joanna’s family convinced her to leave her job. That put a whole new stress on her family.

“As much as I recognized the progression, sometimes you didn’t notice it because you were in it every day, you know? It’s much different living with someone who is sick like that,” Jeff says. “You can’t live your life thinking the worst things will happen, and it’s hard when they do.”

FINDING HOPE

Jo and her family found a huge source of hope in two of her doctors: Barbara LaVarge M.D., and Alison Witkin, M.D. Though opposites in demeanor, they were the advocates that she needed, her family says.

Dr. LaVarge suggested the family check out PHA. Before the pandemic, Joanna, Jeff and their family participated in two PHA O₂breathe walks in Boston. At the fundraising events, they met many other patients and caregivers, giving them a sense of community.

“Barbara LaVarge was just an amazing human,” Jeff says. “She was

the perfect person for Jo at the time, very calm and comforting. Her second doctor, Alison Witkin, was amazing in a different way. She was tireless and a bulldog advocate for Jo while we were figuring out her transplant.”

Jo was transferred to Dr. Witkin. when her condition started to decline.

When her surgery at Brigham and Women’s Hospital fell through, Dr. Witkin set them up on an alternative course with UPenn.

Jeff says the whole family needed those doctors. They needed someone in their corner to advocate for them and fight for Jo after so many people had dismissed her. Those doctors instilled hope in the unknown and stood up for the patients and families battling these invisible illnesses, Jeff says.

After Joanna passed from transplant complications in 2021, her family has been striving to keep her memory alive. They continue to get together, remembering the good times and their independent and sassy Jo. Her experience taught them how short life can be and how important it is to have someone in your corner as support.

With their loss still fresh, the family is brainstorming with PHA on ways to keep her legacy alive. The family encourages those who are feeling lost and hopeless to reach out to their communities, support groups or trusted health care professionals.

Learn about free bereavement resources at PHAssociation.org/ bereavement. Memorialize your loved one through a one-time gift of cash or other assets. Or consider making a recurring donation.PHAssociation.org/ donate /information.

PHASSOCIATION.ORG 29

QUICKTAKES All Nationwide PHA Support Groups Now Online

T

he Pulmonary Hypertension Association recently merged its telephone support groups with its nationwide support group program. The groups are designed for those with shared interests to connect, such as parents, young adults, bereaved families, Spanish speakers, etc. All nationwide meetings now take place through Zoom calls. Participants receive call-in numbers after registering. Learn more: PHAssociation.org/support.

PHA Names Pediatric and Innovation Grant Recipients

P

aul Critser, M.D., Ph.D., received the Pulmonary Hypertension Association Pediatric PH Research Award. Dr. Critser is an assistant professor of pediatrics at Children’s Hospital Medical Center in Cincinnati.

His project, “Pulmonary Vascular Development in BPHD Associated PH,” examines new a non-invasive MRI-based imaging tool. The project could lead to earlier diagnosis of bronchopulmonary dysplasia associated-PH, which affects pre-term infants with underdeveloped lungs.

Csaba Galambos, M.D., Ph.D., is the first recipient of PHA’s Innovation Research Award. The new grant funds research projects that address critical issues in Paul Critser, M.D., Ph.D. advancing PH research. Projects must introduce new scientific or medical concepts or examine existing problems from a new perspective. Dr. Galambos is the director of pediatric pulmonary research at Children’s Hospital Colorado and a lead investigator at the Pediatric Heart Lung Center. His research seeks to understand lung vascular development to identify new strategies for earlier diagnosis, prevention, and potential cures for infants and children with pulmonary disease and PH. Csaba Galambos, M.D., Ph.D.

30 PATHLIGHT

HOW TO REACH US TEL 301-565-3004

| FAX 301-565-3994

EMAIL PHA@PHAssociation.org WEB PHAssociation.org

Support Group Leader Advisory Board Names New Member

T

he Pulmonary Hypertension Association (PHA) welcomes Doug Taylor to its Support Group Leader Advisory Board.

Doug joins Laura Hooley, Marcie McGregor, Diane Ramirez, Nikole Nichols, Tina Stiyer, Pat Ofori, Monica Penaranda and Debra Hines.

Doug has led the MidlandsColumbia support group in South Carolina for 16 years.

Doug, who was diagnosed in 2003, celebrates each day to remind his members that every day is precious and that attitude affects state of mind. “If we make each day a holiday and celebrate, it makes our life fuller.” The advisory board consists of eight patients and one caregiver who lead support group.

Houston, Tampa Kick Off 2023 Fundraising Walks

T

he Houston-area pulmonary hypertension (PH) community’s first O2breathe walk was the Pulmonary Hypertension Association’s first fundraising event of the year. The Jan. 28 event raised $19,000.

Sandeep Sahay, M.D., of Houston Methodist Hospital, and Nidhy Varghese, M.D., Tampa Texas Children’s Hospital, welcomed everyone to the event. Patients Jordan Peoples, Barron “Coach Bear” Honea, Sarah Donoughue and Sawanda Cornett shared their PH journeys.

Team Houston Methodist, the top team, raised more $13,000, followed by Team Liquidia, and Team Texas Children’s HospitalPH Center. The top three Houston individual fundraisers were Jordan Peoples, Sawanda Cornett and Barron Honea.

Team Couch Rage was the top team, raising $7,015 in memory of PHA’s second fundraising event of Haley Willow. The top individual the year was the Tampa O2breathe fundraiser, Rick Swartz, raised Walk and 5K. The Feb. 18 event was $3,815 for Team Couch Rage. in memory of Missy Stok Rizzo, MissyFits PHighting for a Cure was who died from PH in 2018. It was the second highest fundraising Tampa’s fifth O2breathe walk. team. Her PHight is our Fight was Missy’s husband Brian Rizzo shared the third-place fundraising team.

Missy’s story and the event history.

Host an event in your area: More than 90 participants from events@PHAssociation.org. several states raised $20,530.

PHASSOCIATION.ORG 31

QUICKTAKES

CALENDAR OF EVENTS

HOW TO REACH US TEL 301-565-3004

| FAX 301-565-3994

EMAIL PHA@PHAssociation.org WEB PHAssociation.org

Community Fundraising Events PHA Events

An online classroom of free videos about pulmonary hypertension to help you live your best life with PH.

National O2breathe Events

APRIL

APRIL 15 Southern California O2breathe Walk Long Beach PHAevents.org/southerncali2023

MAY 20

AUG. 27

Dewitt Take a Breath for PH Dewitt, Michigan PHAevents.org/dewitt2023

PH in the Park Denver PHAevents.org/phinthepark2023

JULY

SEPTEMBER

JULY 20

SEPT. 9

Paddle the Gallup Ann Arbor, Michigan PHAevents.org/paddle2023

Chicago O2breathe Walk and 5K Chicago PHAevents.org/chicago2023

JULY 22

SEPT. 16

PH Community Workshop San Diego

PH Warrior Walk Claremont, New Hampshire PHAevents.org/phwarrior2023

Glowing for Kassie Lawrenceburg, Tennessee PHAevents.org/glowingforkassie2023

MAY 6

JULY 29

SEPT. 16

Detroit O2breathe Walk Detroit PHAevents.org/detroit2023

PH Community Workshop Burlington, Massachusetts

Puttin’ PHore Poots Bloomington, MN PHAevents.org/poots2023

MAY

MAY 5 World PH Day

MAY 6

AUGUST

MAY 20

Watch these newly posted videos at PHAssociation.org/Classroom: • Respiratory Infections and Pulmonary Hypertension • Journey through Pulmonary Hypertension Diagnosis and Treatment • Understanding the Side Effects of Pulmonary Hypertension Medication • Spotlight on Pulmonary Hypertension Clinical Studies and Research

32 PATHLIGHT

Greater Washington DC O2breathe Walk Alexandria, Virginia PHAevents.org/greaterwash2023

AUG. 12 Northern California O2breathe Walk San Francisco PHAevents.org/nocal2023

SEPT. 28-30 PHPN Symposium Arlington, Virginia

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

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

PHASSOCIATION.ORG 33

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. BennettSchenecker* 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 Stephen Carter-Hicks 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* Jeffery S. Hall* Tammy A.* and Dean S. Hazen Mary Jan Hicks Carl Hicks, Jr. Phyllis M. Hill* Jackie Holt Richard L. Horrocks Constance G. Ives* Terri L. Jakuboski* Stanley T. Jusinski Laura J. Kelly* James Kenney Jessie Kohler-Wenninger Terri L. Kopp* Debbie L. and Mitchell Koppelman Frank D. Koppelman* Dee* and Walter Kruger* Gloria J. Lang*

Marie* and Ronald J. Levendoski Mary Jo* and Thomas Linnen* Sally Maddox* Bonnie and Michael D. McGoon Joseph W. Mihuc* Linda Miles* Karen S. Moody Larry D. Moody Pamela R. Morris Marjorie D. Mott* Joyce L. Mowrer* Dorothy M.* and Harry J. Olson* Theresa "Terry" E. (Cavanaugh) O'Reilly* 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 Joanne Sperando 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 Carol E. Vreim Daniel R. Walsh* Andrea and Stephen L. White * deceased members

Navigating Pulmonary Hypertension:

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

Barbara G. Allen Kathy N. Anderson Adriana Araya Andrew H. Bowers John J. Bresnan John M. Butler Anise M. Callas Joyce M. Caven Alexandria Chadwick Norine M. Charlonis Brian D. Comalander Briston A. Covington Thomas Crigger Kevin DeCrane Marianthi Diacoyannis Beth J. Dixon Linda Espinosa Carmelo J. Figueroa Carl Flink Randal Garbez Debra I. Greene Carson Hamilton

Kenzella Hardridge Sally Hearn Nikki C. Hinds Nicole Hogan Williams Karen R. Hope Dorothy N. Jacobson Sandy Jarvis Regina P. Jones Cheryl Kneal Stacey Koppell Barbara Krispel Tina Lisenby Thomas Liska Lorrie Martin Sharon L. Mayer Katharine J. McDonough Mark E. McGuire Brenda F. Moore Gerald Nye Tammy Olson George Paire Liana Raday

Terry A. Reiley Johnny N. Robertson Susan Rudnicki Dorothy A. Ryan Lina G. Seale Carolyn D. Sheehy Abigail Sheridan Pauline Shields Tiffany Sites Sue Smith Ann C. St. Marie Annette Tuckey Marguerite G. Turpel Kathleen Updegrove Zanetta Vann Doris Webster Fred West Lila Wexler Irene Willmarth Martha Zlatkin

A Guide for Newly Diagnosed Patients

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.

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