PH Through the Years

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.

By 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.

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

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 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.

Words to live by

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 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.

L to R: Gwendolyn’s son-in-law Freddie, daughter Kennethian Green, her mother Delores Rutledge and Gwendolyn.

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.

Monica Sanchez and Alex Flipse

By 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

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 nurses knew me and would greet me by opening the main door.

L to R: Alex, Erika Prieto, Elizabeth Galindo and Monica Sanchez.

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 for the future

When I was diagnosed, there was very little social media. Facebook lets me connect with other PHers 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.

By 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.

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 voice

At PHA’s 2008 International PH Conference and Scientific Sessions in Houston, I learned about 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.

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.

Treatment and 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.

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

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.

he PH field has come a long way since I began my career, and the changes have been truly remarkable.

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.