Living with Hope: My PAH Journey and the Power of Perseverance

My Husband Ron and I married in 1997. I didn’t know then, but the shortness of breath and fatigue I had been feeling for the previous year were symptoms of an incurable degenerative disease called pulmonary arterial hypertension (PAH), a type of pulmonary hypertension (PH). The arteries in my lungs had narrowed, making it harder for my heart to push enough blood through them, causing increased blood pressure in my lungs.

By our first anniversary, I was barely able to walk. I felt as though I could not breathe. The Heart and Lung specialists in Calgary diagnosed me with severe Pulmonary Hypertension and an enlarged heart and told me I had between two weeks to two years to live. I was only 27 years old. There was only one hope: we would get good results from a nitrous oxide test, showing that medication would be an effective treatment. The nitric oxide brought down my lung pressure substantially.

Many people were praying for me —more than I will ever know. I took a medication that only 6% of people with pulmonary hypertension can take. It worked effectively for several years, and I outlived the two years predicted at my diagnosis.

Ron and I moved back to my hometown of Edmonton, where I met the fantastic team of doctors, nurses and other support people who have kept me alive. Cheers to my healthcare team! About five years after diagnosis, Ron and I decided to grow our family. I was told that pregnancy with pulmonary hypertension was definitely out of the question as there was a risk of my dying during or even after giving birth. I could also pass the disease genetically to my children, so we chose adoption. We adopted our son in 2003 from Haiti and our daughter in 2007 also from Haiti.

Around this time, my medication stopped working, but I was given a second chance. I went on an IV medication that I wore on a pump around my waist. A central line catheter entered my heart, and the medication entered my pulmonary arteries. I was on that medication (epoprostenol sodium) for 18 years. In 2020, I became a candidate for a new medication called Uptravi, which was a pill form of epoprostenol sodium. I was taken off my pump and put on a pill. I gradually increased it until I got to the maximum dose. The side effects are tough to deal with sometimes, but my quality of life is excellent.

I feel better right now than I have in a long time. The long-range plan is to have a lung transplant eventually. We’ll see what God has planned. Hopefully, new medications will continue to be developed, and people with Pulmonary Hypertension will live longer.