Reenergized and Holding on to Hope

Laura Hooley is a retired human resources director. She lives in a small New Hampshire town with her husband of 44 years. They like to travel, hike, do home renovations and spend time with family and friends. She enjoys writing, volunteering and activities that involve heights, such as parasailing or ziplining.

By Laura Hooley

In 1966, a Brazilian doctor traveled to America to train surgeons on a miraculous new heart-lung machine. As an 8-year-old with a congenital heart defect, I was a lucky recipient of his skillful ventricular septal defect repair, giving hope to my distressed parents and offering me a chance at a normal life.

Throughout childhood, I viewed heart disease as a simple annoyance that limited my activities and marking me as “different.” No bike riding, no gym class, lots of medical appointments.

After the surgery, my world expanded. I enjoyed my teen years, playing volleyball, skiing and experiencing relatively good health. I married and began a career. A moderate stroke in my 20s was another hurdle, but I recovered.

In my 40s, CHD caught up again. My cardiologist suspected I was developing pulmonary hypertension (PH). As he carefully explained the disease, I jumped right to frightening questions, such as “How much time did I have?” He answered honestly and said two medications were available.

Telling my family was devastating; my future felt tenuous.

Thank goodness for the resources of the Pulmonary Hypertension Association (PHA), which helped us understand this rare, difficult condition. Over the next decade, I would devour its expanding information about PH.

As each new medication was approved, I would press my doctor about whether it was right for me. A willing guinea pig, I tried almost every therapy. I learned to push through side effects and give the drug time to work.

PH is a slippery slope. We have good days and bad; we struggle with medications; and we fight unyielding exhaustion.

When I could no longer work, I sought a productive outlet. I volunteered to become a PHA support group leader.

Bonding with other PH patients at those first meetings is forever etched on my heart! We felt invigorated, understood, and we couldn’t wait to meet again. The group insisted on meeting monthly, and we have done that for six years now. The faces have changed, but the solidarity that holds us together and welcomes new members is a powerful force that I cherish.

In 2019, I was scheduled for an echocardiogram. Watching the screen, I noticed lots of purple. My tricuspid valve had failed, and the atrial enlargement had progressed. I clutched the steering wheel as I drove home, less worried about the ice storm than the echo report.

Further consults confirmed that surgery was essential. The tricuspid valve would be repaired, and my enlarged right atrium resized. I needed a heart lift!

The hospital was in the midst of the pandemic, so I waited four months until surgery was scheduled. Amazingly resilient nurses put my fears to rest. A few days after surgery I joked with the surgeon that I had never seen his entire face behind his mask. I left the hospital nine days later with a bonus: a tiny pacemaker implanted directly in my heart.

At age 61, I feel like a new woman. I’ve had unexpected improvement in my PH symptoms since the surgery, with better oxygen saturations and more energy.

Who knows what the future holds? But every time I have needed an intervention, there has been one right around the corner, so it’s easy to hold on to hope.

Join PHA as we celebrate Rare Disease Day 2021: Feb. 28. More than 300 million people throughout the world live with a rare disease. Many people with PH also have scleroderma, hereditary hemorrhagic telangiectasia, idiopathic pulmonary fibrosis, sickle cell disease or other rare diseases.

If you have PH and a rare disease, share your story in PHA’s Right Heart Blog.

Follow us on social media for information about how PH relates to other rare diseases on #RareDiseaseDay.