“I take each day as it comes with an optimistic approach to my CTEPH.”

By Joyce Sandberg

We moved to Port St. Lucie, Florida, in 2014 after living in Fort Lauderdale for 30 years. Once we settled in, I bought an adult tricycle to try to remain active on a daily basis. Each day I would ride a minimum of 30 minutes to one hour. Sometime in 2015, I found myself feeling short of breath prior to riding my bike. I took my time, persevered and continued with my exercise. I decided to check out the breathing difficulty with a local pulmonologist. I previously had a history with Cleveland Clinic in Weston, Florida, and had a suspicious lower lobe removed from my left lung, which proved benign.

My pulmonologist ordered a breathing test, which resulted in a mild chronic obstructive pulmonary disease (COPD) finding. He told me to use Ventolin prior to riding my bike and to continue as usual. One evening, while at a hotel in Fort Lauderdale, I found that I could not lie in bed, as I was having trouble breathing. I sat and slept in a chair. En route home the next day, I called my pulmo and had to wait two days for an appointment. (I should have gone to the emergency room.)

My doctor looked at my ankles and said, “You are in danger and must go immediately to the ER.” The hospital was across the street from the doctor’s office. I drove to the ER and left my car. My oxygen saturation was 70, and I was given oxygen immediately. I called my husband, and the testing began. I had a saddle embolism on both lungs and survived the night after close heart monitoring. An assigned cardiologist came in and said, “I don’t think you had a heart attack. I believe the right heart has had an excessive strain due to the embolisms.” She stated that the unprovoked nature demanded that I take Eliquis for life as a safety net. I was initially given Heparin and switched over to Eliquis. I was released four days later with oxygen 24/7 and Lisinopril.

Follow ups with both pulmo and cardio doctors proved the clots to be absorbed, but I was still short of breath. No one could tell me why. After many questions, my pulmo suggested I follow up with a specialist from Cleveland Clinic. I began seeing him, and he asked if I [had been] given a right heart catheter, as well as a ventilation-perfusion (VQ) scan. I replied, “No.”

He told me that was the gold standard for diagnosing pulmonary hypertension (PH), which he suspected I had. After both tests were completed, he confirmed I had chronic thromboembolic pulmonary hypertension (CTEPH). I was given Adempas, Eliquis, oxygen 24/7 and Tudorza Pressair. At that time, I was told that I was not a candidate for pulmonary thromboendarterectomy (PTE) surgery as my residual clots were distal in location. Sometime later I was told that balloon pulmonary angioplasties (BPAs) were not on my horizon either.

My exercise was nonexistent as my sats would plummet to 75 and lower once I started walking. The doctor told me to up the oxygen to higher liters to compensate. It helped a little but not much. I began using a power chair at home and an electric scooter when accompanied by my husband. ( I could not put it together myself.) I got creative to remain independent. I’d power chair to my car with my small oxygen tank in hand and a backup tank in the car and drive to a food store that had electric carts. I’d ask anyone in the parking lot to ask a clerk to bring me an electric cart, and it works to this day.

Last year my echo cardio showed mild results; this year, however, they were severe, which led my pulmonary specialist to add another drug, Uptravi. I began titration about five weeks ago and reached a plateau of 600 micrometers until now. I will once again try 800. The last time I had horrible side effects and landed in the ER while visiting my son on Christmas Eve. I’ll give it another go to see if I can handle it.

I hope the Uptravi proves to be the magic bullet to keep this disease from progressing so rapidly. I am 78 years young and had a mother who passed away two months shy of 100 years. She had asthma and was active with few prescriptions for asthma, two inhalers and one tablet per day. I thought I would inherit her longevity. So much for planning ahead. I take each day as it comes with an optimistic approach to my CTEPH.

link to the Right heart blog