It's Not Really an Emergency

So I had my first trip to the Emergency Room since being diagnosed with Pulmonary Hypertension. Four years with no emergency was a pretty good run.

I woke up very late on Sunday morning, feeling pretty crappy. I kind of dragged myself through the day, using coffee (which I had greatly cut down on) to get me through, especially the part of the day that involved a five year old's birthday party in a giant ware house filled with inflatable jump houses.

I got home from the party and attempted to keep functioning. But at about 4:30 I was standing in the kitchen talking to my husband and suddenly said, "Okay, I'm going to faint. I need to lie down".

Into the bedroom I went, and as per usual when I feel "off", I popped my pulse oximeter on my finger. I expected maybe some unusually low oxygen saturations, or a fast heart beat or something.

Nope. Perfect 02 sats. And a heart rate that was dipping down to the low 50s. Normal range is 60 - 100 for a healthy adult. PHers tend to run high. I run at a solid 70 - 80 at rest. So this was strange. And it would explain the crummy feeling.

I waited it out for a bit while laying down. Feeling pretty rough. I decided to call the on-call doc at my specialist's office. He was not comforting as, "I can not tell if you have a heart blockage from here. Go to the emergency room" Great.

Then I did something I promised myself I would never do unless I had to. I have my PH Specialist's personal phone number because he called me one day from it and I thought, "HEY! That could come in handy!" and I saved it... but I never want to abuse that.

So I did call him on a Sunday night. And he too said to go to the ER. Crap. Crap. Crap.

Our local ER is a scary place. End of story. So my husband and I made the decision to go to the hospital where my specialist is based. It is a bit of a ride, but at least there we had a prayer of someone knowing what to do with a PHer.

Drink your water kids!

When we got there they asked all the right PH related questions during intake, and I remained calm and very very happy we were there and not at the local place. Pokes, prods, pee in a cup, (complete with the walk of shame with the gown open in the back while you balance a bunch of wires hanging off you and, you know, a cup of your own urine) and several hours later, we had a diagnosis: dehydration.

Really?

I came out on a Sunday night and spent four hours in the ER because I was thirsty?

Sheesh.

And here folks is the part where we take a break and I tell you all once again in no uncertain terms YOU MUST ADVOCATE FOR YOURSELF!!

With a diagnosis of dehydration they naturally wanted to hang a bag of fluids for me. You know, the wet stuff. The stuff I could drink instead. While in the ER I had touched base with my PH nurse (okay, yeah, I have her number too). And she stressed, as she always does, do not let them overload you with fluids! She's right of course, because to do so could very quickly put me into heart faliure. Bad plan.

So, I'm negotiating with the ER doc, trying to explain this to him and promising with gusto that if he discharges me I will indeed go home and hydrate up. He was totally reasonable and great about it and left the room to get the discharge going.

But while we were having the conversation this nurse is fiddling with all my wires and stuff. I'm focused on the doc. She is behind me, as is all the equipment.

They all leave and suddenly my husband says, "Um... what is dripping into you?".

While we were having a conversation about no fluids the stupid nurse WAS ATTACHING THE FLUIDS. I flipped. I stuttered. I sputtered. And then I sent my husband into the hall to go get someone to take that freaking thing off.

A different nurse came in. And she says, in all seriousness.... wait for it... "Oh. Now we've gone and wasted a bag of fluids."

I choked just a little. Then I looked her straight in the eye and said, "Well, given the alternative, it is a risk and sacrifice I am willing to take.".

She responded with, "What, you mean CHF?" (chronic heart faliure)

Yeah, lady. That's what I meant. And congratulations. You've just become a blog post.

Okay, switching gears (sorry, this is a long one)

We left with me more or less in one piece. And while dehydration could explain the low heart rate, I wasn't totally buying it. For some time now, I have been wondering about one of my meds. Digoxin is sometimes given to PH patients for heart support. It keeps a struggling heart rate low. When I was diagnosed my heart was a huge (literally, huge) mess. But all that's been better for some time now and I was kind of hoping I might come off it, if only to be down one med.

In fact, I had a scheduled appointment for Monday where I was going to bring it up. While my blood work from the ER showed I was not suffering from Digoxin toxicity (something easy to slip into, and quite dangerous), the theory goes it may still in fact be contributing to my low heart rate.

Last week I had an echo and a stress test on a bike (tube in the mouth, plugged nose, wires attached everywhere, the works). I'm rambling here. But it's all connected, I promise.

So, appointment on Monday comes. I'm surprised with no six minute walk! YAY! Because truth be told, not only are they a pain in the ass, I was still feeling pretty icky from the ER ordeal and wasn't up to it.

When meeting with my nurse she informed me that I have been doing kind of too well on my six minute walks. They're not showing much. And the exercise test I did on the bike showed way more - so maybe we should just do those for a while. Okay!! :)

What did show up on that bike test is my heart rate maybe didn't get up as high as they would expect for someone with PH exercising, and perhaps the Digoxin is in fact holding me back too much. So no more Digoxin for me.

Echo looks great, my heart maintaining it's previous improvement. And one little note that I hadn't seen before. When your right heart is enlarged it can push on the left side and sort of compress it, and this has been the case for me. Well, that compression of sorts has gone down. I'll take it as a good thing.

Again I say, YAY!

And in closing, just because it's too bizarre not to include for fun, this hot little number is what is currently hanging outside the hospital gift shop.

Now think real hard, in what scenario would this be an appropriate gift to bring someone in the hospital?

I'll leave you to ponder. I need a drink of water.

An Insatiable Sense of Carpe Diem

I have just marked my fourth anniversary of being diagnosed with Pulmonary Hypertension. It's a day of mixed emotions.

First and foremost I am deeply deeply grateful for all that has happened over the past year. It's been an amazing year. My health, at least symptom wise, has returned to what I would consider almost normal. Yes, I get the occasional palpitation and chest pain, and even a little out of breath at the odd times, but by and large my complaints are minimal. What is most annoying really is the constant struggle with myself to remember to take my medications so many times a day (Crap! Did four hours go by already? Where the heck is my nebulizer??), and the ridiculous battles I have had to wage with my specialty pharmacy.

But all in all feel like I have really and truly made it back from all of this, and that is something I am grateful for daily. And in the back of my mind I am always acutely aware of how temporary it may be.

PH remains progressive and incurable. If I remember the horrid survival charts right, I've outlived about 40% of the patients who were diagnosed the same time I was.

In the past month the PH community has said a sad goodbye to several of our leaders. People who seemed to be defying all the odds themselves, and then slipped away. It never fails to give me a jolt of reality - that this situation is tenuous at best. And while my heart is breaking and the tears fall for phriends gone too soon, the will to fight renews with even more intensity.

But today is a day for celebration. A day to look back on all that has happened and truly count the blessings. And since I have been terribly remiss in updating this blog, here are a few of the highlights...

1) Travels... everywhere: This year I have traveled to Orlando, Cleveland, Washington D.C., Omaha, and Boston for a variety of PH events. These are such incredible opportunities.  I get to talk to other patients and medical professionals about my own journey, and I hope inspire them a little bit. To fight harder. To not take "no" for an answer. To keep the fire alive for research. And as PH is always full circle, they inspire me, and I always learn something from someone who knocks me out with what they've accomplished. I get to meet new people, and meet people I have only known online. It is making my virtual community come alive. So cool.

2) Mountains (of sand) climbed: I have realized that, for better or for worse, I have developed an insatiable sense of carpe diam. I literally have to seize each and every moment, and I do, even it if is perhaps a bit of a foolhardy decision. So this summer we were visiting my adorable new nephew on the West Coast. Oregon beaches are beautiful, and the one we were at happens to have this sand dune that is about 500 feet straight up.


Of course, I got it into my head to climb it. And I did. I had to sit and rest countless times... but oh the view... so worth it. At the top, once I was left alone for a few minutes, I began to cry. I was just so overwhelmed by the combination of the beauty and being so grateful for the chance to be up there (even though it was incredibly difficult going), and very aware that it just might be a once in a lifetime opportunity for me. Wow.

3) My baby started Kindergarten: A day that I actually stood a little too much of a chance of not seeing, that seemed so far off, but came too quickly... and it was another of those bittersweet moments. He's probably it for us - an only child as pregnancy is out of the question and adoption feels a little too overwhelming at the moment. I am so so proud of him and the sweet and spirited little boy he is becoming. And wow is it nice to have (almost) enough time to get all my work done during the day. But boy is it getting quiet around here.


4) Spread the Word: Pulmonary Hypertension E-mail Groups: This was cool... I was a recipient of a Tom Lantos Innovation in Community Service Award which basically means I was given $5,000 and the go-ahead to create this idea I dreamed up a few days before the grant application deadline. The basic idea was to create a public service announcement letting people know about all the incredible e-mail based groups that the Pulmonary Hypertension Association offers for specific demographics. My own group is Generation Hope, and it has been my lifeline. I wanted to get the word out to patients that there are groups like this for everyone. The video is complete and it debuted to over 1,000 PH allied health professionals through the PHPN Conference in September. It was then mailed out to every PH support group leader in the country and is now available on YouTube. So much bigger than I ever imagined, thanks a great deal to the vision of the production company I worked with (Co:lab through Design is Love), and all the help I got from the PH community.

5) Zumbathon!: I have held off on doing a fund raiser for a long time. Asking for money or help of any kind isn't really my thing at all. But a party... now that I can do!! This past summer I wandered into Valley Ballet, drawn by their new Zumba poster offering classes. I thought... well... maybe... just maybe I'm ready. I grew up dancing. 13 years of lessons in fact, and did plenty of dancing in college and after at clubs and such. But PH of course had brought all that to a halt. There was just no way when I was having trouble getting up one flight of stairs that I was going to be be able to dance. And really, I was pretty sure all that was over. But I was feeling so good now. Maybe...

And so I tentatively bought a class card and started going to classes. I did Zumba and a class called DanceFit Mix, and while I have to concentrate a lot on my breathing and pay close attention not to overdo it, I'm doing it. And keeping up pretty well I think.
So I kind of wanted to celebrate all of that. I also knew Zumba is crazy popular around here. So how cool would it be to integrate the fitness community into some awareness and fundraising for PH? The studio had previously hosted a Zumbathon for another cause and I approached the owner who is amazing, and she said yes, she would host!

Long story short, we had about 45 participants and between their donations, a silent auction, and a grant, the first ever Zumbathon for Pulmonary Hypertension raised about $4,500! I could not be more thrilled and am so thankful to everyone who took part, whether through dancing, donating, or prep and break down. It could not have been done without you!

So... that's my year. Crazy, right? I am constantly in awe at what is happening to me. I don't feel like I go after a lot of this. Rather it falls in my lap and if I don't give 110% to make an opportunity everything it can be, and to fight for a cure, then I don't deserve to hope for one. So... I do it. And there is so much to celebrate. People to miss terribly. So much hope to have. A little fear of the future that will probably never quite go away. But everyday I think I'm learning to deal with that. Learning to ride out the incredible ups and the crushing downs. Bring on the next adventure. Oh yes, I have a few ideas. This insatiable sense of carpe diem leaves me never quite satisfied with the status quo... but it's probably best to keep those to myself until I can see if I pull them off. ;)

Ah 1, 2, 3... let's Zumba!

Next week will be my first attempt at a fundraiser.

After a few years of resisting I finally took the plunge and started an event that I hope will raise awareness and a few bucks for Pulmonary Hypertension research and support.

Zumba is a fusion of Latin dance and hip-hop and is wildly popular in health clubs everywhere. I wanted to do something that was going to be a ton of fun for family and friends to participate in, and maybe reach some people who hadn't otherwise heard of PH, or gotten involved in the cause.

I've been incredibly blessed this year to return to one of my first loves and dance again. Thank you God and modern medicine! So it was really cool to have the opportunity to sort of celebrate and integrate the whole thing and channel it for PH.

If you can't make it, we are gratefully accepting donations and 100% of all donations go directly to PHA. We were really lucky and got some grants to cover our event costs - so 100% really means 100%. :)

Check it out! http://zumbathonforph.blogspot.com/

PH and Your Kids

I left the international PH conference last year having learned a lot. But there was one topic still on my mind that I had never heard addressed, and that I desperately wanted to know more about.

When the time came, how in heaven's name was I going to explain this condition to my son?

I thought about it a lot, requested it as a topic for the conference in 2012, but didn't want to wait that long.

And then it dawned on me... I'm an educator! I know about this whole talking to kids on their level stuff, I just couldn't put my own feelings about the disease aside well enough to mesh what I knew about kids with what I knew I will some day have to do regarding PH.

So, deep breath in, deep breath out, I tackled the topic. I wrote an article for the Pulmonary Hypertension Association. I was talking to my friend Sam about it and come to find out, she spent a lot of time talking to kids about their parent's illness when she worked as an oncology nurse. So, I sent her my article and she helped me round it out into something that could be used for all ages and stages, no matter where you are at with your illness.  You can read the full version here: Helping Your Child Cope With Your Illness

I still dread the day my son asks why I'm sick. Right now he won't notice much of anything because I'm doing really well and all my medications and oxygen have been a part of his world for as long as he can remember. To him, this is all normal (ah, childhood innocence!) But, the day will come when he will start asking tough questions, and the day will also come when I have to give him some tough answers. But, now I feel just a little bit more ready.

Heart and Soul

Having PH is like... having emotional whip lash.... or maybe it is like reading a bad Nicholas Sparks novel... whatever the case, after much sadness, I have a happy ending to my current chapter.

Not gonna lie, the last couple of weeks sucked. Within my online support group we lost two members in just a couple of weeks. With both of them their passings were so unexpected. They were around, seemed to be doing well, and then BAM! Gone.

Sometimes that is the nature of PH, and while it is a fact I usually successfully bury deep deep away from my consciousness,  loosing M. and then S., brought it screaming to light. I cried for a couple of days, especially over S. I had not met her in person, but I had the privilege of getting to know her parents this summer at Conference. They had traveled across the ocean from their home country to ours, seeking help for their daughter.

I spent so much time with them... looking into the mom's eyes, promising her there was hope. And what happened to that hope? It didn't pan out. I somehow, as irrational as it may be, felt guilty. I felt like I had offered her something false, like I was fooling myself into a false sense of security in the process. I ache for these people, who so desperately wanted to see their daughter well and now have lost her.

My team of support group co-leaders and I worried about the new members of our group too. How would they take the fact that two of our members passed so quickly? Would it scare them too much? How could we be honest and yet supportive at the same time? The truth is, you can't shield anyone from this. We did the only thing we could - gave the news, coupled with an urging to get involved towards fighting for a cure so that we don't have these losses any more.

In the midst of all of this I was preparing to go to my routine PH check-up. I was due for an echocardiogram, the first in a year, although I had a cardiac MRI six months ago. Pretty much they get a good picture of my heart every six months, and that picture is always bad. In addition, I would do my exercise test, the six minute walk, and have a check-up with the specialist. Since three appointments in a day are cake, I added a visit to the Rheumetologist later that day, you know, just for fun.

Since starting Tyvaso a year ago I have steadily improved symptom wise. This past June, I had marched in, head high, to my last cardiac MRI, so certain that my heart was finally going to show the improvement I've been dreaming about. Instead, I was crushed with the news that my hemodynamics continued to suck... big time. Heart size and functioning were still severely compromised. After fighting my way back from the black hole that news caused, I resolved "no more false hope"... no more going into these appointments with expectations that will be dashed. 

This doesn't mean I lost hope, in fact I still had it in spades, but I stopped setting myself up for disappointment by thinking that this time, this time will be different. Instead, I continued to just focus on someday.

I don't even know how to make this story short, so bear with me. The echo procedure was standard, my six minute walk stellar, and my specialist said words I had actually dared hope for, "We'll see you in six months". Prior to this I had needed to see him every three months, and had done so for three years, but my steady functioning improvement was finally enough. Echo results weren't available yet, but I was happy enough with that.

Later that day I went to my Rheumy appointment. I walked in and he says, "Hey! How are you? Saw your echo results, looks good!"

WHAAAATTTTT??? I had never ever ever heard the words "echo" and "good" in the same sentence. Quite the opposite, actually.

I made him open up the computer to my records, and as he read me my results I entered a near dream state. He was saying to me the very words I had imagined and fantasized about and finally put aside...

Echo results: "Degree of right ventricle dilation,
dysfunction, and severity of tricuspid regurgitation has all
improved" 

In addition, the right atrium is back to NORMAL, down from
severely dilated and my moderate tricuspid regurgitation is now
NORMAL!! The right ventrical still has significant dilation and
systolic function is still significantly compromised, but both have
improved

Translation: My heart is remodeling itself and repairing much of the damage of Pulmonary Hypertension. I'm not out of the woods yet, far from it. It could go back the other way. But for the very first time ever my body is showing inward signs of improvement.

I got a bit emotional right there in the office. And, since this doc has, well, no inner filter whatsoever, he turned to me and said the words that are destined to become classic in my circle of friends and family:

"Well! Ain't that the shit!"

It is, Doctor, it is indeed!

Just when I was on my knees emotionally, so unsure of where I was going to find my footing to get back to my positive outlook, God opened this window for me. There is hope in this disease process, even in the hopeless times. It may not be your time for improvement, just as so often it has not been mine. But hope is there.

Wow, this feels good. 

Blogging Day 2.0

Welcome to the new blog site! I've made the move here because my old site just didn't have all the fun bells and whistles you can use here. All the content from the old blog is here as well.

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.

Finding A Voice

Original Date: November 10, 2010 

 

So, if you know me at all, you know I’m not exactly quiet. But the truth is, I’m still pretty shy sometimes. Public speaking has never been my thing (unless you are a room full of six year olds, then I’m all good!).  But, I’m also pretty vocal and I’ve found the relative anonymity of  working online to suit my personality very well. It gives me time to think out what I want to say, to change it six times, to stress over it and change it three more times after that, and t0 finally put forth to whomever cares to read it something I really want to express.

I believe that when you have something like PH you simply can not sit around waiting for something better to happen to you, and you must take your destiny into your hands by being proactive. For some, this involves fund raising. It turns out I am not one of those people! I’ve done fund raising and quite honestly, I don’t like it too much! I admire those who do it so well, but it makes me uncomfortable to ask for help. What works for me though is writing about my experience (clearly, a lot!) and what I know (some, and a little more everyday). It has become my message to other patients and their families that each of us must find our voice, whether it is through fund raising, or networking, or writing, or art, or… well, what’s your voice for PH?

A few months ago I was approached out of the blue and asked if I would be interested in speaking at the annual meeting of the REVEAL registry (www.revealregistry.com). In short, this is a massive national study that is endeavoring to put a modern face to Pulmonary Hypertension. The hope is that the new data will drive more focused research, better treatments, and ultimately a cure.
Would I like to speak to about 130 people who are instrumental in making this study happen: doctors, nurses, respiratory therapists, project managers, etc. Um, no, I was shaking just thinking about getting up in front of all those people…. and YES YES YES! What an incredible opportunity.

The YES! won.

So, in early October, I traveled to Boston and did just that. Writing the speech was a process. I sat down one night and banged out seven pages (about 15 minutes worth of talking). Then I started my obsess, revise, obsess, revise thing. As I said to my audience that night, “What does one say to a room full of people working to make your deepest desire come true – a cure for Pulmonary Hypertension?”

The general assignments I was given was to put a face and a story to the data – “A Day in the Life of a PH Patient” kind of thing, I guess. I’m okay with that, I don’t mind telling my story. But I also wanted to tell our story, the story of the PH experience… how any doctor that isn’t a specialist often risks a PH patient’s life because they simply do not know better, how medications and the fear of the future effects a patient’s ability to cope, how there are patients out there who get to defy every odd and how they inspire me to keep fighting. Many of my PHriends  all chipped in their ideas and stories, helping me to shape what I ultimately would say.

The biggest message that I wanted medical professionals to come away with is that when they are treating PH they are treating a whole person, one with hopes and dreams and fears. I’m sure on some level they know that, but the clinical needs can trump other parts of a person’s needs during a routine appointment. I’d like to share the same idea with you, and to that end, here is a brief excerpt of what I spoke about that night. I hope it inspires you to take care of your emotional needs if you are a patient, to understand your loved ones needs if you are a caregiver. And if by chance you are a medical professional, I hope you can remember in the midst of all the incredible physical care you give us (thank you) to step back and say, “and how are you doing with all of this?”.

Having PH is like having a dark crystal ball. You know all the statistics. You know the words “degenerative” and “incurable” are ultimately pretty true. It is a little like living your life just waiting for the darkness to fall, and that is no way to be. So, in so much as is possible, I choose not to be that way.

I have a son to raise. Failure to do so without all the vivacity and energy I feel he deserves from me is simply not an option. Truth be told, if PH can be beat by pure stubbornness, then I’m your girl. I’ve been both credited and chastised for my stubborn Irish ways, but either way, I am quite sure the unstoppable fight in me takes some part in my ability to not just survive PH thus far, but to actually truly thrive with it…

In a particularly rough time a little over a year ago, a wonderful man with heart problems of his own gave me Dr. Bernie Segal’s classic, “Love, Medicine, and Miracles”. Through reading that book I came to the profound understanding that I needed to take spiritual control of my destiny with PH. I truly gave this whole thing up to God, and took the very conscious path of thinking always in positive terms, always with the belief that the cure will be found.

To say this and to live this are two different things. It has to be with every fiber of my being, or I loose a little strength and a little of my fight with each negative thought that enters my mind.
This is not to say I don’t have bad days. I am a strong patient. I am a strong woman and mother. But, a rough appointment or a scary test will still sideline me for a while. My smile and spunk in the doctor’s office, or the positive report to my parents on the phone, can turn to torrential tears when I am first alone after rough news.

I have learned to give myself permission to feel these things for a while, and then to pick myself back up, dust off, and begin again with new resolve. To deny the crappy hand I have been dealt with PH would be unhealthy, to wallow in it would be the same….

(and later)

Co-leading Generation Hope has given me a sense of purpose. Before it started, I was sitting around hating PH, but not too sure what to do about it past that. Now I feel like my ability to share my story, and my assurance that I can rest on these people when I struggle, makes me feel stronger and more powerful against the disease than ever. I hope every patient is encouraged and inspired to find their own outlets and their own ways to fight back.