If you're new to PH, or want to learn more about it, I recently participated in a interview on Connecticut Public Radio, which can be heard here: http://www.yourpublicmedia.org/node/8019
So, today is PH Blogging Day 2010. The day when we take to the internet and raise a ruckus about PH Awareness. I usually like to just write when inspiration strikes, or when I have something to kind of work through. But today is the day, so I need to post something! So... here's my story of diagnosis.
I was diagnosed on January 2, 2008 and I am looking towards my third anniversary of diagnosis.
15 months prior to my diagnosis, I had given birth to my son, Aidan. And so, my story begins much like it does for many women, my PH came out with my pregnancy… but not at first. At first I thought I needed to “loose the baby weight”. I gave little thought to the day I was out walking and climbed a big hill, where at the top I handed off my infant son’s stroller to a friend, as I knelt to the ground turning ashen grey, with a pounding heart and a cold sweat. I foolishly thought I had better get my rear in gear and see a personal trainer or something. This baby weight thing seemed harder to deal with than I had anticipated.
I was so busy being a mom and juggling a part-time teaching job and moving into our first home, that I failed to attach significance to the fact that I was breathless after a small set of stairs at work, or that I would be in the middle of a sentence in front of my class, and simply run out of air.
But then I came down with pleurisy, three times in one winter. Toughing it out, a round of natural remedies, and a round of antibiotics did nothing to stop the re-occurrences of this lung infection. By the third round, I was spent. One evening, I climbed my basement stairs and suddenly the heart pounding returned. I knelt onto the kitchen floor, certain I was going to pass out. After catching my breath, we took a trip to the Emergency Room. And then my journey really began.
In the Emergency Room, they found no initial significance to my symptoms. I didn’t tell them about the hill, or the stairs, or the breathless sentences because at that time I didn’t even realize it must all be connected. After a chest x-ray and an EKG, I was sent home.
The next day was Monday. I received a call at work, on my voicemail of all things.
“Hello, this is so and so from Bristol Hospital. Um, the radiologist read your chest x-ray and you have an enlarged heart. It’s probably no big deal, but you should see a cardiologist just in case. Here’s a phone number.”
Holy crap. In my limited imagination of what could be wrong, it seemed impossible that an enlarged heart could be “no big deal”. I dutifully visited the man I would later credit as perhaps saving my life. Dr. Joseph Marakovitz, a cardiologist in a small city in a small hospital saw me first. He did a series of tests. I remember my first echocardiogram. His tech chatted away with me, we had things in common, we laughed a bit. Then she stopped laughing. And she stared hard at the screen. And she didn’t talk much for the rest of the test. And I just knew. I didn’t know what I knew, but I knew it was bad.
Dr. Marakovitz met with my husband and myself to go over results. He told us that the best-case scenario was a hole in my heart. He also told us there was a worst-case scenario, but we were not going to talk about it because it would scare me too much, and if we got there I was headed straight for a specialist. He sent us home with an order for a Right Heart Catheter and instructions to hope, of all things, for a hole in my heart.
Within such a very short time, the cath was completed. I took in what I could from my post-procedure haze. In my head I can still see the words “Pulmonary Hypertension” scrawled atop my medical chart, as Dr. Marakovitz wrote it out and leaned over my hospital bed to show me the name of a disease I was about to become intimately familiar with.
A few days later, after I had some time to process it all, and to read all the wrong and alarming websites, I called him back. I asked him to go over my numbers again. As he did, with a systolic pressure of 90, a diastolic pressure of 38, and a mean pulmonary artery pressure of 55, the true impact of the situation began to sink in. I began to cry on the phone, and I swear that good doctor may have cried too. I just remember him saying, “I am so sorry this happened to YOU”.
To this day, and now knowing what I do about how long it can take for PH to be diagnosed, I think of Dr Marakovitz as something of a gift in all of this. How he knew to go straight for the diagnosis if PH, and to get there so quickly, I’ll never know. But I am so grateful he did.
I was, as promised, sent directly to Dr. Raymond Foley, at the UCONN Medical Center in Connecticut. Dr Foley, his nurse practitioner Diahann, and the rest of his staff have become my mainstay. I know I can turn to them with anything, and I often do. Dihann in particular fields a whole lot of questions from me, and I’m often running in one unusual direction or another with my treatment, all of which she answers to with patients, understanding, and support.
Dr. Foley is perhaps the kindest person I’ve ever met. We sometimes don’t see eye to eye on some of my care, but his respect for me and his ability to work with me as his patient, to guide my medical treatment with his expertise, but also allow me to be the one who ultimately knows my body best is something I wouldn’t trade for the world. I think this is part of what makes him such a great doctor, and certainly the right doctor for me.
I am dutiful with my PH medications. Prior to diagnosis I practically had to be force-fed Tylenol for a headache. Natural remedies and healthy holistic living were kind of “my thing”. To divert from that, and become so intricately medically managed was a huge change for me, and something I still struggle with sometimes.
I started with Digoxin and Revatio, adding Letairis about eight months later.
I let Letairis sit on my counter a full 24 hours before I started it. I just kept looking at it, thinking, “Okay, these aren’t the big guns yet, but we’re moving on here. This is for real.” Images of liver function failure, whatever that looks like, and endless blood draws stretched before me and I hesitated to begin.
Its kind of a funny story, but the Red Cross had asked me years ago never to return to give blood – I was just too hard of a draw to handle. I didn’t know how I was going to repeat those tests month after month. I finally got up the courage and took the first pill. Not so bad, although I still give the woman who draws my stubborn blood work heart palpitations of her own, we move on.
Through all this, I found my own ways of dealing with the PH. I spent the first few months after diagnosis in what I refer to as “my cave”. I paced the house late into the night. I cried constantly. Little things that never used to bother me could throw me into a frenzy, for I had nothing left to fight with. To say one is devastated by such a diagnosis is truly an understatement.
I found a really good group of people on the Pulmonary Hypertension Association website, and they educated me and pulled me along in ways that were so helpful, but still things were missing.
I learned to put on that brave face, to tell my story to friends and family and assure them I was handling it all fine, and in some ways I was, but in others, I very much was not.
Through all this, most of my life felt like little changed. I had quit my job, but for reasons beyond PH, although the diagnosis certainly played a factor. I still kept up after my son. I still worked from home. I was even able to do some water aerobics.
Yet, I still felt lost and somewhat alone. Where were the other people like me with this disease? The young mothers? The professionals? The people still able to live most of their lives as they did pre-diagnosis? Did they not exist? Would I be able to keep up my own life or was I destined to find this “new normal” everyone was talking about, and would that new normal really be my new nightmare?
That is where I stood a couple of short years ago. A lot has changed since then. My functioning has improved drastically, but I must take medication five times a day, to the tune of $200,000 a year, for that to happen. And it could all stop working. I could start to go downhill tomorrow. In no time at all I could have a permanent tube in my chest, pumping in medication that would knock me out with side effects. I don't focus on that, but I can't forget it either.
This concept of "new normal" is something I struggle with. It is meant to be a way of adjusting your attitude, of accepting the way things are for what they are. It is meant to be encouraging, that life as you know it might be changing and you have to adjust and accept. But the thing is, I don't accept the way PH is. I hate it and I fight it every day. I kind of hate the phrase "Welcome to your new normal!" too, because nothing with PH ever stays the same, none of it is normal, and it sure as hell isn't something I would welcome.
So, I am refusing to accept this "new normal". Instead, I'm trying to create a better one every day. It's exhausting, but worth it.