Around Fall of 2007 I started noticing a significant change in my ability to breath. I was having noticeable shortness of breath and had difficulty moving. Of course my first thought was that I’ve been neglecting the gym and eating irresponsibly instead of cooking healthy meals for myself.
In November my sister and I took a trip to Cancun for my birthday, and I remember thinking to myself, how did I let myself get this out of shape? Slowly over time I started getting more and more short of breath despite my efforts to exercise and eat better.
At the end of May I started to notice that my ankles were swelling. Not good. At the beginning of June I noticed that the swelling didn’t go down and actually started getting worse so I called my PCP. When the intake nurse found out that I was swelling and had shortness of breath she had me come in for a same day appointment. It normally takes 3-4 weeks to get an appointment with my PCP. Not good.
When I first got to the office they did an EKG which showed I had a high resting heart rate, but we attributed it to stress (I had just hit a pedestrian with my car a week ago… long story short- the pedestrian had a broken left ring finger and a bruised elbow. The police report came out in my favor stating the accident was her fault).
It also turned out that I had gained nearly 30 pounds in 6 months. Not normal. So over the course of the next few weeks my doctor had me take a battery of tests so we could figure out why I was swelling and had rapid weight gain. All tests and labs came back negative. Apparently it seemed I was as healthy as can be: normal liver, kidney, and spleen functions, low cholesterol, and negative on all diseases tested that could be associated with swelling. My doctor put me on a low dose diuretic to help with the swelling and we set up a follow up appointment.
Meanwhile, over the summer my work’s wellness program had an exercise group that met twice a week at lunch. Before and after each session they took our resting heart rate and blood pressure. I noticed that my resting heart rate was consistently between 110 – 115 and my blood pressure was consistently high. Not good. The director of the program recommended that I call my doctor.
I spoke with my doctor on the phone and she had me go for an echocardiogram – which is basically a sonogram of the heart. When I got to my appointment the technician said that it would take about 15 minutes and I’d be out of there in no time. In the middle of the echo she stops and says, ‘I’ll be right back I need to consult with a doctor.’ Not good. She comes back with a cardiologist and a nurse and they explain to me that I have a heart condition and they told me that my right heart was failing and my lungs were affected. This was mid to late July.
The next week I had an appointment with my PCP who told me that I had Pulmonary Hypertension. She said there are two kinds: idiopathic and secondary. Idiopathic meaning there is no known cause and secondary meaning something else is going on that is causing me to have PH. She then referred me to a cardiologist who specializes in PH at UCSF. The appointment with the cardiologist wasn’t for a few weeks, and that was being specially squeezed in. In those weeks while I was waiting I went through a whole new series of labs and testing to see if the PH was secondary.
At the end of August I saw the cardiologist who told me that I had IPAH. I was then scheduled for a right heart catheterization on September 3rd to measure the pressure in my heart. The average blood pressure in the pulmonary artery for patients with PH is greater than 25 mm Hg at rest. Mine was 99 mm Hg. At that point I was admitted right away to the Intensive Cardiac Care Unit (ICCU) and put on a medication called Flolan. Since there is no cure for PH, you can only treat the symptoms. Apparently Flolan is the most effective drug out there to reduce the pressure, but it’s also the most complicated. I would have to be on Flolan for the rest of my life and since it’s an IV/pump medication I would need to be under 24/7 care at all times. Independence lost. Not good.
Well it turned out that I wasn’t just ‘out of shape and overweight’. I had a rare, serious heart/lung disease that as been creeping up on me for who knows how long. There is no way to know how long I’ve been living with PH but I think it’s been a while since my case was extremely severe. The cardiologist told me that people with PH have a life expectancy of 2-3.5 years if symptoms are left untreated. In theory I could have been living with it for 3 years. Crazy.