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. ;)