I have scars on each of my wrists — one from a feeding tube and IV — and the other from a PICC line doctors used to monitor my blood. I also have a zipper-like scar down the front of my chest, along with two scars from drainage holes on either side of my ribs. These scars tell the story of my battle with chronic thromboembolic pulmonary hypertension.

It’s common for CTEPH patients to go undiagnosed for years. Doctors often treat conditions they are used to seeing first. But it only takes one specialist and one referral to make all the difference in your treatment.

Journey to diagnosis

In 2014, I had a pulmonary embolism and took Coumadin (warfarin) for six months. A year later, I had another pulmonary embolism while flying home from a work conference, which I attributed to the combination of Evista and air travel.

When I got home from the hospital, I learned online that people with a history of blood clots shouldn’t take Evista (raloxifene), which is what I took for osteoporosis. No one had warned me: not my doctor, pharmacist or anyone at the hospital. I immediately stopped taking it.

In 2019, I started having shortness of breath and chest tightness. My doctor ordered a VQ scan, which measures air and blood flow in the lungs. He probably should’ve ordered this exam after my first and second blood clot.

The results were bad. I spent the night in the hospital and in the morning, every half hour, a new doctor came into my room to evaluate my charts. I went home the next morning with no answers.

Finally, about a week later in November 2019, I was diagnosed with pulmonary hypertension and referred to Vallerie Mclaughlin, director of the pulmonary hypertension program at the University of Michigan. After evaluating my records, she referred me to Jonathan Haft, a cardiac surgeon at the University of Michigan Medical Center.

In December 2019, Haft ordered a right heart catheterization, which confirmed CTEPH. He said PTE surgery would improve my lung capacity by 85%.

The idea of the surgery terrified me. It sounded like being carved up like a Thanksgiving turkey. Haft told my husband and me to think it over, pray about it and give him a call with our decision.

Moving forward

I knew if I didn’t have the surgery right away, I would never do it. So before leaving the hospital, we set a date for January 2020. Thank God we did, because two months later, the pandemic shut down the world.

The waiting period before the surgery was absolute hell. I was scared and often held onto my husband and cried. We even made funeral arrangements in case I didn’t make it. It was hard to come to terms with that possibility.

Before the surgery, I asked Haft to pray with me. I felt the prayers of friends, family, and friends of friends from all religions. I felt incredibly supported and hopeful the surgery would go well.

A month later, Haft showed me a picture of what he removed from my lungs. It looked like a small tree trunk, the size of my thumb.

Recovery and healing

The road to recovery was rocky. I initially took Adempas (riociguat) to treat my symptoms, but I experienced five of the seven possible side effects, even on the first day of taking the medication. I had unbearable headaches, severe nausea and vomiting and constant facial redness.

After about a month, I couldn’t take it anymore. I asked my doctors to lower the dosage, but I was already on the lowest dose. I switched to tadalafil and Opsumit (macitentan) with no problems.

When the FDA approved the tadalafil-macitentan combination pill in April 2024, I switched to Opsynvi. It feels so good to take only one pill a day instead of three. Mentally, it feels way more manageable, like taking a daily aspirin.

The difference between what I couldn’t do before my surgery, how much I struggled and what I can do now is incredible. I’m part of a walking group, and we walk two to three miles, three times a week.

One exercise we do is a 45-minute walking routine. Before surgery, I had to sit multiple times to catch my breath during that routine. Now, I can do the full 45 minutes without stopping. Being able to move, exercise, do laundry and even talk on the phone without having to catch my breath is a miracle.

Connections

After my diagnosis and surgery, I needed support. I called PHA’s Patient and Caregiver Support Line and talked with a nice lady who let me ramble about everything I was going through.

Afterward, I joined the Ann Arbor support group at the University of Michigan via Zoom. The people in my group are so wonderful. They inspire me with how long they’ve been thriving with PH, and we share many tips to help each other. I am the only member with CTEPH, but we connect over issues we all face, like medications and oxygen use.

I use two liters of oxygen a night and was having trouble sleeping because of the oxygen hose around my ears. One woman in my group said she hooked her oxygen hose above her head at night, so the hose went on the front of her ears instead of around them. I’ve done that ever since.

At PHA 2024, I got to see many of my group members in person, which was marvelous. I also presented on diagnosis challenges, especially for CTEPH patients and am a part of the national virtual PHA CTEPH Support Group led by Missy Storm. Storm and I met in person last year in Florida for lunch. It was an amazing time and made me grateful to have friends nationally.

Pulmonary Hypertension Association groups are amazing for support. Those of us with PH are zebras because we’re rare, and sometimes being rare can feel overwhelming and isolating. The support groups offer knowledge, support and a community that will help you get through the pains of this condition.

It’s interesting to see how the tables turn the longer you live with this condition. I asked a million questions in the first meetings I attended. Now I offer my experience.

It’s OK to feel like crap when you’re diagnosed. It’s not fun. The most important thing to get through this journey is finding your true support person, whether that’s your significant other, a friend, family member or roommate. You need to find a team — a community that can give you confidence when you don’t have it.