June 20 2006 has got to be the worst day of my life. Up until that point I was a somewhat healthy overall twenty five year old woman with a steady job a loving family and the best friends any one could ever hope for. That said, you’re asking yourself so why was that day so bad? It was the day I was officially diagnosed with Idiopathic Primary Pulmonary Hypertension. Go ahead say it three times fast - I dare you!
My journey to that diagnoses actually started in April of the previous year, but more on that later.
In July of my sophomore year of high school I spent the month living with my father, George, and my step mother, Susan. I had chosen to spend the July fourth weekend at a friend’s house and while I was there contracted a viral lung infection from her little sister. After returning to my father’s and presenting symptoms of a severe cold, my stepmother (who just happens to be a nurse among other things) suggested we see a local walk-in medical center. The doctors there prescribed an antibiotic as well as prednazone and inhalers because they said I was showing signs of being asthmatic. A full month later, and several return trips to the doctors, I returned to my mother’s home where I normally resided. All was well unless I had a cold then I would need to be on rescue inhalers. For many years we were told I had asthma and things seemed to be ok.
In November of 04 my best friend and I flew to Las Vegas for a week of r and r. I was fine the entire flight out there but I noticed a day or two into our trip that I was not breathing right, my chest felt very tight. I attributed it to things like jet lag and being in a different environment, dry heat opposed to the East coast, and the humid moist heat. Thinking nothing of it I went on with my trip and returned home and went about my daily routines. The following April again while away I had to climb a very steep hill about half way up I had to stop and catch my breath, my heart was pounding in my chest and it felt like my chest was on fire. I rested for a bit and then made the trek to the top where I got to my destination only to be smacked in the face by what seemed to me 2,000 steps, again I had to stop to rest only to have to climb more steps once inside the building. Returning from that trip I immediately scheduled a visit to my PCP who again put me on asthma meds, but changed them this time. Two months later and still no improvement, so back I went. This time I was sent for pulmonary function testing and a sleep study. The Pft results were way below normal even with treatment the sleep study came back positive for sleep apnea. I was then referred to a pulmonologist who quickly scheduled another sleep study so they can measure the pressures for a cpap machine also on that visit he did a test where I was required to walk several flights of stairs with a pulsoximeter on my finger. My O2 levels spiked dangerously low but on rest shot back up into normal ranges. The doctor then told my mother and he suspected Pulmonary Hypertension and referred me to Dr Terrence Trow of Yale's Winchester Chest Clinic. While waiting for my first appointment at Yale I was sent for a CAT scan of the chest and based on the fact that they found nodes on my mediasteinum and given my sister's battle with Hodgkin’s disease they had me undergo a biospy to rule out any type of cancer. Thankfully those results came back negative. In January of 2006 I met Dr. Trow and his team. They had already read all of my records and knew my case history quite thoroughly and I was highly impressed with that. I then under went what seemed like a never ending series of tests to determine was this primary PH or was it secondary PH? These went on for several months until June 20th of 2005 when we got our definitive answer it was primary. As a team we were then able to decide how to treat it.
Well it’s been a year and a half and I have to say I am doing pretty ok. I have my emotional days and my rough breathing days but all in all things seem well.
Debra S. Tenney, age 26