Tuesday, November 16, 2010

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.

Sunday, November 14, 2010

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.

If You Have to Be Here, It's Worth Being HERE

Original Date: November 5, 2010

Wow, so here we are at the one year (and 3 days) anniversary of this blog! Yes, the time for PH Awareness Month has rolled around again. The time of year when I resolve to write here more than once every couple of months.

I want to start out on a positive note because this year has been, by and large, very positive PH health wise. There have been ups and downs, and a lot of it has been chronicled here over the months, but really, I can’t complain too much.

There have been two huge highlights for me this past year. I think I’ll start with just one for this post.
The first was attending the International Conference in June. What an experience. To sit amongst over 1,000 people who actually get what I’m going through is amazing. The Pulmonary Hypertension Association rocks. They have this way of bringing together patients, caregivers and doctors onto this level playing field I suspect you don’t see too often in medical communities.

I sat in on sessions led by doctors, and some led by patients. I had breakfast with doctors who could answer my questions. I had some questions that couldn’t be answered, but that’s okay. I got to participate in several research projects by visiting the research room and lending my data, and feedback, and many vials of blood.

Most importantly, I got to meet so many patients I have only known online. I was able to channel all the work I’ve been doing over the past year or so and help mentor and lead groups of patients too. That was pretty cool. I was the welcome person for the young adult meet and greet room on the first day.That kicked off meeting this wonderful couple who had traveled all the way from Israel just to find help and resources for their daughter. PH is rare all over the world.

The really cool role I was asked to play was to sit on a panel of “The Next Generation of PHA Leaders”. It was really an honor and a surprise to be asked. I sat up on stage at breakfast one day with a few other go-getters. Two of my co-panelists were fundraiser extrodinaires, and one just happened to have participated in a half-ironman with his PH! Me… I talked about this blog, and co-leading Generation Hope.

There were two particularly poignant events for me. The first was finally meeting patients who just defy my every understanding and expectation of Pulmonary Hypertension. There were some there who are true athletes, and others living lives that the statistics just say aren’t possible. Screw the statistics, I’ve met those people, and am working myself like crazy to become and stay one of them.

The second was listening to Carl Hicks speak. Carl was the outgoing chairman of the board this year. His beautiful daughter Meaghan passed away from complications after her transplant. To hear Carl speak, to see a father wipe away tears for his daughter, but strengthen his voice and his resolve even as he struggled, was both heart-wrenching and inspiring. I cried every time that man got up to the podium. His emotions were raw, and his reason for fighting, for still fighting after his loss, was so powerful. I am beginning to realize many of our PHA leaders are in his shoes, having lost loved ones to this disease but carrying on their memory and their fight long after the loved ones have moved on to finally breathe easy.

We’re really blessed in this community. If we have to have this absolutely shitty hand dealt to us, then I at least don’t want it to be anything else and I don’t want to be involved anywhere else. The advocacy of our community has brought us from zero PH therapies in 1994 to eight different approved medications now. The average survival rate of around six years is still absolute crap, but it is also triple what it used to be. There are eight more drugs in the FDA pipeline that show a lot of promise, and dozens of other research studies going on.

Our doctors are real people who see their patients as real people. When I ran into my docor at a conference last year,  he greeted me with a warm hug. I had the privilege of meeting who I consider to be one of our nation’s premier PH specialists a few months ago (more on that later!) and when I e-mailed him to thank him for his time, my response a day later was signed with his first name, no DOCTOR! formality needed.  My nurse and I exchange technical questions and e-mails, followed by “and how are your kids?” I admire these men and women who have dedicated their professions and their lives to saving us. The work they are doing is inspiring and I am deeply grateful.

The Pulmonary Hypertension Association is the heart and soul of it all. They listen to their members like you wouldn’t believe. They inspire research, raise unheard of funds, and even help move legislation through Washington. No small feat in the current political climate, but we’re doing it! (Want to help? Click HERE). Most of all, they provide a place of support and refuge for patients. And not just patients in general. They’ve managed to reach out to all these little sub-groups and create communities within the larger PH community. They gear up for and then sponsor conferences around the country, and then awareness month.

Well, awareness month is here. Wear your periwinkle of Fridays. Tell a friend about Pulmonary Hypertension. Tell them about me if you want. But just get the word out there.


Original Date: October 30, 2010

I am such a mess of emotions right now. From angry to humbled and heartsick, it’s been a hell of a ride these last few days.

I’ve spent the last week battling my specialty pharmacy company over a medication delivery. The specialty pharmacy is on my hit list right about now.

Long story short, I followed up on a medication delivery no less than four times, which then never showed up. Once I called customer service and just about set the rep’s ear on fire, I was ASSURED and PROMISED that my medication would be arriving today. Bullshit. None, nada, and I have run out (well, they think I’ve run out, I’ve kept a secret stash from the last time they screwed up, but shhhhhhh!)

I’m annoyed on one level and was (I’ll explain the past tense in a moment) livid on another. See, the thing with this company is that they require that you sign for your delivery of medication. Incidentally, the other specialty pharmacy who handles PH meds does not.

This is problematic on many levels. First of all, I’m not home bound. I’m blessed to feel well enough to live the life I’ve chosen, and that means some mornings I work and others I am out and about with my son. Not everyone with PH gets this choice. My medication allows me to do that.  Staying home for a four hour window for a delivery is a huge inconvenience.

This time though, it got personal. To stay home on Friday for the  medicine delivery that never came I had to miss my son’s preschool Halloween event. It’s a little silly to be upset about something small like that, but the truth is my moments are precious. I believe I will beat this thing  and I will live. But the odds are completely stacked against me. The odds say I’ll be lucky and unlikely to see him out of grade school.  I don’t get to have another kid and do it again, because childbirth is deadly.  So, to miss even a second, when I’ve committed to be there, becomes gut-wrenching for me. I don’t think I even realized how important these moments had become until I was denied one by someone’s stupidity.
I was also going to have to miss his soccer game this morning, but my dear friend and neighbor agreed to be on lookout for the package that still never came so I could go cheer him on. I am really blessed by the friends and family around me.

So, yesterday and this morning I was ready to roll heads. How dare some pharmacy company screw with my life? I got a little Momma Bear, I guess. Or, as my dad says, I really “got my Irish up”.
Then, I log on to facebook. And I find out that a girl whose story I have followed since my diagnosis has died from complications from her PH. She was 21. And now what? I can’t exactly stay mad over an inconvenience caused by my pharmacy company and ultimately my illness when someone else has died because those medications stopped working for her and her transplant attempt failed. Suddenly, my problem seems pretty minuscule in comparison.

So, as I sit here still wiping tears for Becky and her family, I’m reminded even more acutely than I was yesterday how precious every second is. How even as I felt foolish yesterday for being angry at missing some Halloween pictures of a bunch of four-year olds, I was somewhat justified. If we don’t find a cure for this blasted disease, the sadness Becky’s family feels at this moment, is the sadness all our families face, and that’s really hard to swallow.

Go hug someone you love.

Crap Isn't So Bad, I Guess

August 5, 2010 

Once again, this woefully under-updated blog stalks my mind until I sit down to write. Thankfully, I have work I am supposed to be doing and wish to procrastinate, so now seems like a good time to give in.

Let’s see… a really cool thing. Okay, freaking amazing thing.

I climb hills. No big deal to the average person, but may as well be Kilimanjaro to a lot of PH patients (more on that another day!). May as well have been Kilimanjaro to me not so long ago.
So, when I last left this blog I was having another Cardiac MRI, but hadn’t got the results back yet. Well I got them, and they were CRAP. I mean rotten. I mean my heart is huge, my hemodynamics suck, and I think medical professionals might turn just a little pale when they see it. I, on the other hand, just get nauseous at the sight of those pictures.

So, I sit in my doctor’s office talking to the nurse practitioner, a woman I have grown to love and admire these past couple of years. I depend on her for straight answers and a soft voice of reason. I’m arguing with her about the results, but really, I’m arguing with myself. I just can’t believe they are so bad, when I feel pretty darn good.

I left the office, got in the car, and cried my eyes out. Then I drove home and cried some more. I may have screamed just a little. Which is weird, because I don’t do that. But the thoughts “I am going to die” did cross my mind. And I had to fight them back with everything in me, and call on everything above me to help that.

See, this past winter, I felt like CRAP. More crappy than I ever have felt before, more crappy than I would admit to anyone else, and (let’s be honest) more crappy than I would even let myself acknowledge. But, if you look back at pictures from this winter and see the pale puffy me, and as I remember how dead on my feet I was feeling a lot of the time, I have to (hate to) admit it.

But then I did a lot of good for me things, like kept up with my acupuncture and holistic treatments, started Tyvaso (see post below), and started exercising. Here’s the amazing part – not just exercising, but like actually working out. Doing the Wii Fit. Jogging. Yes – JOGGING. Not a lot – but a couple of minutes. When I started, that couple of minutes put me on the couch totally dizzy and out of breath, heart pounding. But now that I’ve been at it for a while it is actually getting easy.

I was heart broken that the test results didn’t reflect all that.

So, how do I put together how I feel and how I function with how my tests look? I can’t – but I do attribute it to a higher power (God is very good to me!), the incredible support I receive,  and a whole lot of tenacity on my part too. I don’t think the medical professionals can actually explain all this either. And I’ve decided it doesn’t matter… because look what I can do…
This is the hill I was walking with a friend the first time I ever felt PH symptoms, about three years ago. I was pushing my infant son in a stroller. We got to the top and turned around, and suddenly it happened. I handed off the stroller and knelt to the ground, gasping and heart pounding. My friend said I turned gray. I had no idea what was wrong, but was soon to find out I had severe Pulmonary Hypertension.

A few weeks ago, I went out and climbed that hill again, this time symptom free. The test results may not look any better, but I am  living, walking, breathing proof that there are miracles and the tests only tell you so much.

I leave you with that for now… but I’ve recently returned from the International Pulmonary Hypertension Conference in Orange County, CA, and I really aught to update on that too… which means I’ll have to find another time I really want to procrastinate. :) Until then… I think I’ll go for a walk.

MRI Mishaps and Malarky

Original Date: June 15, 2010

First, let me just say I am grateful for modern medicine. As much as I hate to be subjected to it, I am here (at least in part) because of it. That being said…

It was another adventure in the wonders of a state hospital this morning. Today was my Cardiac MRI, a not all that commonly done procedure to track how my PH is treating me. Traditionally, they’ve been using Echocardiograms and Right Heart Catheters (RHC) with the disease. Both have their respective benefits and drawbacks, and now this MRI is more accurate than an Echo and less invasive (although maybe not as telling in some ways) as a RHC.

Anywho… I arrived 10 minutes early only to be told they were running late. Since this same procedure ran two hours late last year, I was prepared with a book and my new iPhone toy. However, being the dutiful patient I am, I read the sign encouraging me to use the Ladies room before the procedure and endeavored to do so. In order to do this, I had to be let into the lab, just enough to catch a glimpse of the convict on the MRI table and his security guard from the prison standing watch.

Wait… I’m early and am going to be made late by this guy running late from prison? Seriously? Don’t get me wrong, I’m enough of a humanitarian to be okay with health care for all (and this post isn’t about that) but I’ve got things to do, places to go! Come on, now!

Okay, so I wait out my time and go in to get started. I am informed of the procedures they are going to do, which are wrong. Yup, wrong. I don’t even know what exactly it is they are going to do and I still know they’re wrong. They wanted to subject me to intravenous dye I did not need for a test that was not ordered by my doctor.

Oh, there seems to be some confusion…” says the tech. (Ya think?)

Right. Well, it’s Tuesday, so my nurse is in. Call her. Your papers are wrong.” was my reply.
They were wrong, I was right, we proceed with the test.

I change into the high fashion hospital gown, although for this test I am allowed the dignity of my top (no bra) and bottoms, jeans today for me (this gets important later). Better yet, I even get to keep on my shoes. If you’ve read my previous posts, you know why this is important to me. Also, hospital floors are gross.

Into the tube I go. Thankfully I am not terribly claustrophobic, although for those of you who have not experienced such a test, let me just tell you that you lay on this table too narrow for humans (and I’m kind of a smallish medium girl, I don’t know how the big guy from the big house fit on it before me!). Hooked to a variety of monitors, you are slid into the tube, with about a six inch clearance to your face, and given a “Holy Shit, I’m Panicking, Get Me Outta Here” ball, technically called a “squeeze ball” which is your primary connection to the outside world for the next 30 – 45 minutes.  No big deal, actually, when you consider some of the other fun tests that are possible.

I lay in the tube listening to the beeps and whirs of the machine, muffled some by the headphones through which they pipe music to distract you. We begin the test, the first half of which is fairly uneventful. Then the voice of the tech comes to me through the machine…

Tech: “Is there a metal button on your jeans?

Me: “Yesss…

Tech: “They seem to be in the way. Do you think you can shimmy out of them down to your knees?

Me” “Ummm… yes…

Wait, I’m in the plastic equivalent of a connoli shell. Remember the six inches above my face? It’s also above the rest of me… and you want me to shimmy??? Well, I did it somehow, and we move on.
A majority of this test involves big breaths in, big breaths out, and then holding it just longer than is humanly possible or comfortable, but you do it anyway, and you do it on cue.

As this is going on, the music continues to pipe through my headphones. The soundtrack to my adventure? “No Air”. For those of you who struggle for your breath with PH, you get the irony. For those of you without, plug your nose for a while and breathe through a straw. It’s a pretty accurate simulation of the feeling. Then, play the lyrics over and over in your head. “No air… no air… how am I supposed to breathe with no air?”

Good times all around. The test is done, no harm done. Results in a week. Despite the relatively benign events, this is still a stressful morning. How do I deal? Like any good patient… I took myself shopping for a friend’s birthday at Crate and Barrel and then out to lunch, including a glass of wine. It’s not all bad!

I sign off with my same message from last time. I feel great. I really do. I defy the odds, and am deeply grateful and awestruck by that. But until my phriends stop suffering, until I can wake up and not think about the oxygen tube in my nose or the list of meds I need to take and when, until prognosis is no longer on my mind, it will not be enough. The hope and faith in a cure continues.

Outdated Updates

Original Date: May 23, 2010 

I’ve had the gnawing thought in the back of my mind that I really aught to update this blog. It appears people are actually still reading it (hello!) and it has been sorely neglected since February (eek!), and my last post wasn’t exactly me as my usual cheery self. Score one for PH.

Well, I’m back, and PH has had its moment, so let’s turn the tables, shall we?
The truth is, I haven’t updated in part because I feel freaking fantastic. Or is it PHreaking PHantastic? Whatever! I started Tyvaso and, as usual, the anticipation was much much worse than the reality. In fact, there’s something rather wonderful going on.

I’m walking actual miles. I’m working out, planting gardens, painting walls. Wait… this is the life I actually remember before getting sick. All of it. It really is. I haven’t had a symptom in months (okay, a bit of chest pain, but let’s not talk about that). On my last six minute walk I surpassed the expected distance for a healthy person, and certainly beat my own previous record by a nice couple of laps.
But it gets better… I’m sure Tyvaso has something, a lot of somethings, to do with all this. But, I’ve also been seeing a naturapath and she’s pretty amazing. She started working on my inflammation levels, which previously were at numbers no one had really seen before… probably thanks to an autoimmune disease which is probably causing or aggravating the PH. There are a lot of probablies in this field of medicine! Well, those numbers, and all my bad numbers in blood work, are drastically better. And I can feel it!

That’s all the good, very very very good, news. The bad news, or same old news, is that as far as we know, my heart still looks and functions like crap. And that scares the doctors (you can see it in their eyes) and so it still scares me. So, I go in for another Cardiac MRI in a few weeks. Here’s hoping that something, anything starts to look better inside. Because as nice as it is to feel this great, that could all change in quite literally a heartbeat if something doesn’t get better. And I am acutely aware of that every day.

The other thing is, most of my PH friends still suffer a lot. More than any of us, myself included, can fully imagine. I’ve lost several phriends in these past months. Many of them got something else and PH made it impossible to survive. Most of them were around my age. I’ve found this to be an occupational hazard of connecting to the community of people who understand what I go through and live what I live (and so much more). People die. A lot. And until that changes, even if I continue to do well, I’ll still be here making a huge stink about causes and cures and hope.

The international PH conference is just a few weeks away! I’ll be speaking on a patient panel and hosting a get-together for the support group I co-lead online. Here’s hoping there is some fantastic news in breakthroughs and emerging research that will keep us all going.


Original Date: February 5, 2010 
At PHA  they have a section called “Journeys” (http://www.phassociation.org/Page.aspx?pid=500). It is a place where patients, families, and caregivers tell their stories. I’ve always found the name to be quite fitting. After all, from the second you get your diagnosis it is a life-long (and life-changing) journey. We all travel it differently, but in other ways end up in very much the same place… hospitals, doctors, tests, more tests, medication, and more and more medication.

Well, the next step in my journey is here, and (surprise, surprise) it was the result of a one-day stint in the hospital, a very busy but very attentive doctor, a crappy test (see former posts on Right Heart Catheters), and now… more medication.

The time has come for the next step – where PH inevitably seems to lead – what we’ve been doing isn’t working as well as we might hope, so let’s try something new, something more. I am about to embark into the world of Tyvaso – an inhaled medication taken 3 to 4 times a day. We’re adding this to my other therapies, currently all pills, bringing the grand total of medications up to somewhere in the $200,000 per year mark and a total of nine doses of therapy a day. Oh, how my insurance company must love me!!

Tyvaso is new to the market as of this past September, and I’ll be the first in my clinic to go on it. In some ways it isn’t such a big deal. An inhaled medication is easier than a continuous IV one in many ways. And the number of treatments and time it takes for each treatment is about half that of an older inhaled med that is available. I’ve been feeling like crap for the last month, signs of PH I never had cropping up at the most inconvenient times (all times to feel your PH are inconvenient). So, I know it is time, and I’m pretty much at peace with that.

Yet, I just spent about a week being pretty messed up about all this – it is very hard to accept that PH is doing just what they say it will. Then I feel like some sort of hypocrite because I preach the positive all the time… but I’m still scared silly sometimes. And really angry, and really confused.  It reminds me of Hebrew Block Logic – a decidedly non-Western way of thinking. This was a concept I was introduced to in college that has always stuck with me. My professor, Dr. Marv Wilson, put it this way:

“Concepts were expressed in self-contained units or blocks of thought. These blocks did not necessarily fit together in any obviously rational or harmonious pattern, particularly when one block represented the human perspective on truth and the other represented the divine. This way of thinking created a propensity for paradox, antimony, or apparent contradiction, as one block stood in tension — and often illogical relation — to the other. Hence, polarity of thought or dialectic often characterized block logic….Consideration of certain forms of block logic may give one the impression that divine sovereignty and human responsibility were incompatible. The Hebrews, however, sense no violation of their freedom as they accomplish God’s purposes”

He later goes on to call this whole thing a “dynamic tension“. How fitting.

Miraculously, I found this quote online when trying to find a source for Block Logic – and it just fits so darn perfectly! Never mind the source – I know nothing about it or anything else they say, but I’ll post it here in effort to be a responsible writer and so I don’t have to go digging through dusty boxes for the actual text – which all these years later still hangs in the back of my mind. Good to know my education stuck!: http://theologylog.8.forumer.com/a/hebrew-block-logic_post8.html

So… I’ll tap into my Jewish roots here. I can be 100% full of faith in a cure and 100% human in how I feel about the whole danged thing happening to me. My human responsibility is to move forward with the treatments I need while I wait for the divine sovereignty of a cure. Okay. That works. This past week I found myself asking for “the peace that surpasses understanding” (Philippians 4:7) and I think I just found it.

By the way – speaking of Journeys – have you heard about the big one in the PH world yet? Please check it out and support me while I support the cause: http://www.firstgiving.com

Sometimes Someone Else Says it Better...

Original Date: January 28, 2010 

Walk with me…..
Walk in my shoes
for one single day.
Then you’ll see why
I need to pray.
Come live in my home
for a week or two
and then remember
I am just like you.
I didn’t ask for the things I was given
I didn’t choose this road I have taken
Walk a mile with me hand in hand
Then perhaps you will understand.
I’m not really complaining
about the stress in my life,
I know that we all have
some toil and some strife.
But walk with me, when you think
I am wrong, walk with me
and you’ll start to belong.
Embrace my sorrows,
like they are your own,
And then you will know me
And see I have grown.
The journey I take
is different from yours
My life took one of those
unexpected detours,
But this road that I travel
is not really so long,
If the people who watch me
will join in my song.
Listen to my footsteps
and watch how I dance
And then you will know me
and give me a chance.
Take heart and remember
It can happen to you,
who knows where my pathway
will cross over to you?
So speak to me softly
if you can’t understand
Remember I once stood
right there where you stand.
And walk with me gently
when the day is at end.
And then I will know
I can call you my friend.

copyright 1999
Sally Meyer

Guest Blogger!

Original Date: January 14, 2010 

This week I was honored with the task of being a guest blogger for the Pulmonary Hypertension Association’s president. Many thanks to Rino and PHA for making this possible.


Just Kidding!

Original Date: November 29, 2009
Um… the post below is now not quite the last post for this month. Oh well. :)
THANK YOU to my friend Will, who wrote and published an article in a local paper… and on his blog, where you can read it in full!


And Here We Are...

Original Date: November 28, 2009 
Wow. So this is it. The final post for PH Awareness month. Will I keep writing after this? I’m really not sure. It’s been an interesting trip, to say the least. I’m glad I put things out there, that much I know. I found it somewhat therapeutic… and a little exhausting. It’s hard to face your fears head on and put them into words. I hope people have learned a lot about PH. I’m not sure what else I have to say, but if something comes to me, I’ll be back. In the meantime, a little closure for this month…

A reader asked me about the title of the blog – and an explanation seems fitting at this juncture.
Sea glass is one of my favorite things. We used to live just around the corner from a beach in Massachusetts. I would often walk along the shore in the off-season and collect sea glass. It was calming, relaxing, often just me and the waves. When the time came to pick a name for some internet moniker, sea glass seemed fitting just because I like it so much. The more I thought about it though, the more I realized sea glass is also something of a metaphor for me. When you see it, tiny pieces tossed smooth by the waves, it looks nothing like the original form it once had – probably a bottle of some sort. But, it is still beautiful and unique. Although a piece of something, it is whole in its own right. It is kind of like having PH (or going through any major trial, I would guess)…I’m still the same person I was before I got sick. I’m on a hell of a ride – being tossed around by things I can’t control, there are major changes to my world, but I’m still me. Just different in some ways. Hence, the title of this blog.

It is weird to write about all this intense stuff. I wonder what people think. Those that see me often might suspect I have a flair for the dramatic, given my choice of words and the stories I have told on this blog. I, like so many others, “don’t look sick”. Of course, my insides tell a much different story, but you wouldn’t know it to look at me. I hope most times I don’t act sick either. People don’t know unless I tell them. For me, it is more about looking down the road and what they say is to come and wanting desperately to avoid it. It is also about knowing where this journey has already ended up for so many (see previous posts about medication and life expectancies) and wanting to take up the fight on their behalf as well. Both reasons are why I am here writing this.

I am floored by the support I have received. At the risk of slipping into the cliché – touched beyond words (but I somehow have still found a few to write…). This blog has brought some amazing stories to life. People I’ve never met, but who got the link from family, have reached out to me. I’ve gotten gifts of support in the mail at the perfect time, heard from distant relatives I haven’t seen since I was a young child, and this must be only the beginning.

Someone at PHA recently mentioned to me that they think that Pulmonary Hypertension is almost as prevalent as Multiple Sclerosis (MS), but before you met me, had you heard of it? Probably not. Had you heard of MS? Probably! The Center for Disease Control (CDC) still isn’t sure if PH is an incredibly rare disease, or still drastically under-diagnosed.

For those of you who take the time to read, thank you. If you passed on the link, thank you-thank you! I don’t necessarily know who has done it, but I know many people must have since the number of hits have been so great! (That 1,000 mark is just around the corner!)

Please, please keep spreading the word about PH until no one any longer has to be very sick before being diagnosed. Keep spreading the word until there is adequate funding and research towards a cure. We’re getting there – the people working on it, from fund raising to the labs, deserve immeasurable thanks and respect, but until we actually arrive at the day where we can say, “Oh yes, I had this disease, and it was life changing, but I’m all better now!” we haven’t come far enough.

A Lesson In Medications

November 21, 2009
Hello, my name is Colleen and I take Viagra.

To survive Pulmonary Hypertension (PH) you have to take the aforementioned shitload of drugs. (see first post in this blog)

Prior to 1995 there was nothing to be done for a PH patient except tell them to get their affairs in order and possibly get on a list for a double heart and lung transplant (which in and of itself is a kind of disease… with an average 5 year survival rate post-transplant.) Starting with the advent of Flolan in 1995, things slowly began to change.

I’ve sat down to write this installment several times, and I get stuck when I get here. PH is complicated. Treatments, by default, are also complicated. Could I explain it so the average person can understand it? Yeah… if they would stick through reading the pages it would take to do.

I will try not to do that, but bear with me while I try to condense! I think it is important that people understand the treatments and how they fall short. We’re lucky to have many to choose from, but none offer a cure and all come with a high financial and physical cost.

First – the prostanoids (a substance PH patients don’t make enough of, which, according to phassociation.org, is used to “help many PH patients by dilating blood vessels, reducing clotting, slowing down the growth of smooth muscle cells, and improving cardiac output”)

In 1995 we had the advent of Flolan. Flolan is administered via central line in the chest. A tube is hooked to a pump the patient carries and the medicine must be kept on ice. There is an 8 hour version that doesn’t need ice. Side effects are very severe, including: pain, stomach upset, and risk of infection from the line. It can take up to an hour a day just to keep the site clean and dry for regular routine like showering. And at approximately $100,000 a year, it doesn’t come cheap. However, it saves lives and those that I know who are on it say all the risks are worth it.

Then there is Remodulin. This can also be administered via central line, but a pic line in the arm and an under the skin catheter called Sub-Q is also frequently used. The Sub-Q method is extremely painful for many people and they usually have to be on one or more severe pain killers just to tolerate it. Remodulin has a lot of the same fun side effects as Flolan and costs about $75,000 a year.
This past September, Remodulin was released in an inhaled form called Tyvaso -  initial reactions from my friends in the community have been very positive! You take it three times a day and treatment time is only  a few minutes each cycle. I’m excited about this one.

A pill form of Remodulin is also well in the works, with clinical trials going on now.
Next, the other inhaled PH med, Ventavis. Ventavis must be inhaled 6 – 9 times a day, with each treatment lasting up to 10 minutes. New advances with Ventavis are decreasing treatment times. Side effects are mostly things like coughing and headache, but it is generally well tolerated. Patient compliance taking Ventavis is something of an issue because of the pure time involved. I personally would have a pretty hard time finding an hour’s worth of 10 minutes segments in my day and keeping my preschooler quiet while I breath into a machine. (I don’t know the financial cost)
The next category of drugs is the Endothelin Antagonists (PH patients make too much Endothelin, causing the vessels in the lungs to constrict and narrow).

These are Tracleer and Letairis. Both are in pill form and both cost about $50,000 a year. Tracleer is a twice a day pill and Letairis once a day. Side effects usually aren’t too bad – mostly flushing and stuffy noses, headache, and some patients experience swelling in their extremities. However, both run a high risk of liver faliure and so a monthly Liver Function Test (LFT) must be done, as well as a monthly pregnancy test because the medications are highly harmful to a fetus.

(As a side note – pregnancy isn’t really an option for the PH patient anyway. Maternal mortality rates are around 50%.)

Finally, we get to the meds we PHers are famous for – the Phosphodisterase (PDE-5) Inhibitors, known to the common man as Viagra! (and others). These help relax the blood vessels and arteries and improve blood flow. If you know anything about Viagra, then you can see how this makes sense. Interestingly, intense athletes have also begun to use Viagra as a performance enhancer because it helps them breathe more efficiently. The two common forms are: Revatio (Viagra’s cousin) and as of this fall, Adcirca (Cialis’ cousin).

So, yes, I take Viagra. Lots and lots and lots of Viagra. Side effects are generally mild and well tolerated and include things such as: headaches, flushing (I turn quite rosy sometimes!), nasal congestion, and really interesting dinnertime conversations when someone notices you popping a pill. You have to take it three times a day and it costs around $10,000 a year. Adcirica is only once a day and costs a little less, but so far I haven’t been thrilled with the side effects I am hearing about (mostly severe headache).

Let me rant for a moment. For the sake of example: Drugstore.com charges $149 for 10 50 mg pills of Viagra (a total of 500 mg). Revatio (the same exact stuff as Viagra, but packaged and marketed for PH) spread out in the 20 mg doses would end up costing about $231 for the same 500 mg. Why is it that the cost of my drug is $82 more dollars to help me stay alive for eight days (taking the prescribed 60 mg a day) while the old guy who wants to just get it on 10 times pays so much less????
I digress…

So, what you may have noticed, is that there are three primary categories of medication. Each targets a known pathway of PH. Many American patients are on more than one therapy (which is not an option in some countries), with a brave few on one from each category. There aren’t many studies about the efficacy of more than one therapy, but in theory it makes perfect sense since each category of drug has a different job in the body. However, what we don’t know is the potential impact of so many high-caliber medications in the system at once. As a friend pointed out though, we do know the impact of no drugs in the system (death) and even with two the outcome is still sub-standard in both life expectancy and life quality.

Survival rates of PH without treatment is at 2.8 years. With treatment, well, they just don’t know. Five years seems about average, seven to eight is considered long-term.

One PH medication that doesn’t get a great deal of attention is Calcium Channel Blockers (CCB). CCBs work really well on only about 6 – 10% of the PH population. Side effects are again fairly mild and the medication is often very effective for a long time for the lucky few who respond to it.
Many patients are also on Digoxin for heart support and Coumadin because Phers blood tends to clot more easily – raising the risk of stroke, etc. For those of you not in the know, Coumadin also has a common name – rat poison. Yup, same stuff. I haven’t been in a hurry to get on it, although I do understand the benefits and risks of not. I’m taking my chances.

Finally, many people are on oxygen therapy. Some wear it 24/7, while others (like me) need it only at night. Phers tend to have oxygen levels desaturate in their sleep more than most.

For those of you keeping count at home, I take: Letairis, Revatio, and Digoxin. Tyvaso seems to perhaps be on my horizon. I wear oxygen at night. I am very grateful my insurance company covers all but $42/month of the $7,000/month it is costing to keep me alive.

What I really want to impress is that medications are incredibly costly and many have debilitating or at least life-changing side effects. We’ve made phenomenal gains since 1995, but are nowhere near close enough to where we need to be.

We need medications that don’t make us as disabled (or more disabled) than the disease itself. We need ones that don’t put us in the poor house or wreck havoc on other organs.
Most of all, we need a cure.
(Post sources: www.phassociation.org, www.phcentral.org)

PH Blogging Day

November 18, 2009 

So today is PH Blogging Day – a day set aside by the Pulmonary Hypertension Association to encourage as many people as possible to take to the highways and byways of cyberland and spread the word about PH.

If you’ve followed this blog through its short life, you already know what it feels like to have PH. You know how it is diagnosed and what it does. You know one way to not just survive, but to thrive in spite of PH.

Do you e-mail? Do you have a facebook or myspace page? Twitter? If you do, I would love it if you could take a minute to tell all of your friends and family about Pulmonary Hypertension.
Tell them my story, if you want. You can also hand out my contact information and I can share more if they’re curious.

Send them a link to www.phassociation.org.

Or, send them a link to PHAs Blogspot: http://phawareness.blogspot.com/

Or, send them here to this blog: http://phandthenewnormal.blogspot.com

Whatever you do, spread the word. With awareness comes the saving of lives. Hopefully, funding and research dollars will also continue to be allocated our way so that the cure that has to be just on the horizon shows up.

Thank you for your support.


Original Date: November 12, 2009 

I knew I was in trouble the day a cardiologist told me the best case scenario he was hoping for was to find a hole in my heart that we could repair. He told me there was a worst case scenario too… but we weren’t going to talk about it unless we got there. If the best thing is a hole in the heart, what’s the worst? Well, as it turned out, Pulmonary Hypertension of course. We got there.
It is difficult to describe to someone who hasn’t “Been There” what it is like to lay in a hospital bed, have a doctor look you in the eye, and rattle off the name of some life-altering and life-threatening disease you have never heard of and apply it to you.
Where do you go from there? I imagine people have a huge range of responses. I only know my own. I freaked. Looking back on the months after diagnosis, it was pretty much me in a dark cave. Scouring the internet for any information I could find (a stupid idea – most of it is woefully outdated and false and will scare you senseless), finding sources that finally explained something updated and accurate, pacing the house late into the middle of the night just trying to wrap my mind around the whole damn thing. Crying. A lot.
During the day you probably wouldn’t have seen much of this from me. Fake it until you make it. I was still working, I still had a child to care for, a family and friends to respond to. People who say “As long as you have your health you have everything” haven’t actually lived through the blow of loosing your health and coming through on the other side still intact. I am very grateful there is more to life than just health, or I’d be screwed.
I have a wonderful support system, but for a long time none of it helped. I just had to deal on my terms in my own way. But, it really comes to a point where you must make a choice for yourself. Either keep living your life in a state of shock and grief, or pick yourself up, brush off, and start living again. There is no in between in my case. It was live, truly live, or face death head on feeling like I was… well, dieing. That doesn’t seem like such a good option, certainly not the mindset that would keep me strong for the fight of my life.
I’ve tried to explain to people how once you have a disease like this you never ever for a single second get to forget it. They often look confused – if it such a part of your everyday don’t you just get used to it? Not really. I remember every time I pop a pill – five for the PH and a huge handful of vitamins and supplements for a bunch of other reasons – all PH related. I remember every time I look in the back of the car at a load of groceries and try to gauge how heavy each bag is and which I should carry together so I don’t arrive in the house breathless and dizzy. I remember every night as I shove a tube of oxygen up my nose, and many times as I fall asleep and feel my heart palpitate with each breath. You never get to forget.
But, you can’t stop living either. And you have to find ways to do that. Some days you need more ways to focus on living over the disease than others. So, I’ve developed an arsenal.
Some are serious – like totally giving this whole thing up to God. I believe I will be healed. I don’t know how. I don’t know when. But I WILL be. It is actually that simple. In the meantime though, I still have to get by. I have to live the human side while I wait for the divine. This fight could get a hell of a lot harder before it gets better.
As much as I believe in the power of prayer, I also believe in the power of positive thinking. If I believe I will be healed, if I truly believe this is going to end up okay, my attitude is going to be better. And if the mind/body connection is half as strong as I suspect it to be, just keeping a positive mindset will keep me stronger.
The support system of friends and family I have gets me really far. I have lots of phriends in the PH Community who get the struggle first hand, who offer advice and encouragement on treatments, and sometimes a “Yeah, I know that sucks” is enough too. I have healthy friends and family who are amazing. Always checking in, lending a hand when needed. I don’t know how many people are praying for me – but I do know that over 450 people have read this blog and that’s a lot of positive vibes and well wishes. I believe in the power of that too!
Then there’s the more light hearted side. I only go to appointments wearing cute shoes. Cute shoes make me happy. If I don’t have to have an exercise test that day, you can bet they’re going to be cute and hot and with a minimum of three inch heels. If I do have an exercise test, even my sneakers are ones that make me smile.
I only take my meds by washing them down with water from a wine glass. I don’t know why – but nice stemware makes me happy too. So if I use it when I’m doing something I don’t want to do – like take $7,000 a month in drugs – it makes it a little easier.
Lately I’ve been moving to a more altruistic lifeline – reaching out and helping other patients. I am thrilled to have been asked to sit on a Patient Advisory Board for the Pulmonary Hypertension Association (PHA). We’re going to work together to reach other young people who have so much of their lives ahead of them as they also navigate PH. I’ve also joined “Generation Hope” – another PHA organization that consists of a private e-mail group where we can support other young patients. How great is the name of that group? I hope to do some writing for the quarterly newsletter that PHA puts out nationally. I want to turn all this PH knowledge I have bumping around in my head into something that helps others.
Oh… and I’m getting a tattoo… something to remind me of my battle, but also of the victory that will come. Stay tuned on that one! I just put it in writing, on the Internet no less, so I’d better follow through!

Just the Facts, Ma'am!

Original date: November 5, 2009

Okay boys and girls, all you faithful blog readers, get ready for a lesson in PH…
First of all, with the heart there is left side and right side involvement. This is about right side involvement – the kind I am most familiar with.

PH is known as “the other high blood pressure” – a totally misleading term. It is indeed high blood pressure , but treating it is no where as simple as popping a pill, watching your sodium, and loosing some weight. The blood pressure problem is within the pulmonary artery of the lungs. Simply stated, the heart has to work really hard against this extra pressure. It tends to enlarge (as mine has) and eventually it can lose its ability to pump (which I’m hoping mine won’t!).

PH knows no age, race, or gender. ANYONE can get it. However, it primarily strikes women in their childbearing years. It is not unusual for symptoms to emerge with or just after pregnancy, as was the case with me. Pregnancy doesn’t cause it, but if you’re going to have it anyway, something about pregnancy seems to kickstart it a bit.

Symptoms of PH include quite a list. I have experienced the following common symptoms:
Chest pain, shortness of breath on “easy” exertion like climbing a flight of stairs or carrying groceries, low energy/fatigue, dizziness and fainting, and heart palpitations.

In addition, a patient might have: swollen ankles and legs and bluish lips and skin.
This list, unfortunately, looks like a whole lot of other symptoms lists for other diseases. That is, in part, why so many people go through many doctor visits, misdiagnosis, and even years of suffering before a correct diagnosis is given. At that point, for many, the disease is at an advanced state and treatment has to be swift and intense. (More on that later)

There are some preliminary screenings for PH. It might be suspected from a chest x-ray showing the enlarged heart and/or an abnormal EKG (that’s me again!). An echo cardiogram gives estimates of pressures and you can measure cardiac output and heart size as well… but diagnosis is best done via a Right Heart Catheter (RHC).  Oh yeah, those are as fun as they sound. A thin tube is inserted into the body via the groin or the neck and then they move it around and take a look at your pulmonary system. The best part – the patient is awake for the whole thing. I have seen the inside of my heart up on a screen while laying on a gurney in a freeeeeeezzzzziinnnnnggg room, being worked on by a tech who resembled Harry Connick Jr. (That wasn’t so bad!). Valium is a beautiful thing in these situations, but not always offered. After two such procedures, one drugged and one not, I assure you, offered or not, it will be politely requested demanded for the next round!

Once there is a definitive diagnosis, the fun really begins. First, you have to just wrap your head around the fact that you have an “incurrable and often fatal disease”. I think that is another post for another time…

You have to look for and/or rule out all possible causes, such as: autoimmune disorders (such as Scleroderma/CREST or Lupus – two common culprits), an Atrial Septal Defect (an ASD, a birth defect of the heart – a “hole”), and blood clots in the lungs (Pulmonary Embolism). It can also be caused by other underlying health issues like liver disease and drug use – specifically diet drugs like Phen-phen and “recreational drugs” like cocain. (Yes, they really call them recreational – it took me a while to figure out what I was being asked when they screened me. And the answer was “NO!”).

These tests are not a lot of fun. You give up vials and vials of blood. Screening for blood clots in the lungs involves a procedure where you are actually radioactive for a while after (a fact not mentioned to me before the test was just about in progress!) This also means that while driving home, you may be pulled over for suspected terrorist activity since you are emmitting radioactive waves for a while. That didn’t happen to me, but I kind of wish it had. It would have been fun to explain!

“Oh, no, officer, I’m not carrying illegal chemical explosives… that’s me your nifty little machine caught.  I’m radioactive, you see…”

Back to the topic at hand, I have NONE of the underlying causes. This makes me a lucky one or two in a million who have what is often referred to as Primary or Idiopathic Pulmonary Arterial Hypertension. Yup, one in a million. How do you like those odds? I knew I should have played the lottery more…

After diagnosis, comes the treatment regime, but if you’ve stuck with me this far, you’ve probably read enough for one day! Thank you.

Treatments will come some other time in the near future… next post will be a little bit more about what it is really like living with PH. I hope you stay tuned.

(In the interest of proper citations practice, I have to give credit to www.phassociation.org for the fact in this post. This stuff is all in my head now, but most of it came at the beginning from the really good folks there)

The First Post (Pulmonary Hypertension Awareness Month)

Original Date: November 2, 2009

Well, here I am blogging. I’ve kicked around the idea of doing this now and then. Kind of wondered what the point was – sure, my thoughts are interesting to me (or I wouldn’t be thinking them, right?) but to others?? I haven’t quite caught on to the whole “my life is an open book on facebook/youtube/myspace/twitter” thing… Although I am, of course, a facebook addict, I mostly share pictures of my insanely cute kid and keep up with my friends (and many of their insanely cute kids).

My dad has bugged me to be a writer for as long as I can remember (Hi, Dad!). Okay, so maybe I’m finally stepping up. Or, maybe, I finally have something that is so important to say that I think the world needs to hear it. Yeah… it is probably more of the latter (No offense, Dad).

But here we are – November 2009. It is Pulmonary Hypertension (PH) Awareness Month. My second such month in a line of what is looking like many to come.

I started “going public” with my disease last year, so there’s a good chance many of you even taking the time to read this (thank you, by the way) know my original story and know a little about PH. I’m actually not going to dwell on that part much, at least for now.

In short, PH is “a simplified name for a complex health problem” (www.phassociation.org). It is progressive and currently incurable. It causes chronic high blood pressure in my lungs and it effects my heart in a big way. So big, in fact, that the Grinch and his heart have nothing on me – mine grew a storybook three sizes a while ago, and it doesn’t like it! Average survival rate, with treatment, is 50% at five years. I don’t know about you, but those odds (while improved from even a few years ago) sound pretty crappy to me. Thank God I’m not a statistic.

So, what does PH mean to a real person, not as a medical diagnosis?

In the day-to-day this means I take a shit load of drugs (Pardon the profanity -I decided this is my blog, my voice, and I say “shit” sometimes!). It means I have to sleep with an oxygen tube up my nose. I also think this is shitty. It means I spend more time at doctors than I care to think about. It means that I can’t carry the groceries, prod a toddler along, and block the dog from escaping all at once because if I do I will probably pass out. It means I used to kick box and dance my heart out and I can’t anymore. And that breaks my heart. It means I look at my son and pray in a way I didn’t think possible that I will live to see him grow up. It means I can’t talk about that part any more because as brave as I am (almost) every day, thinking about him like that makes me start to cry immediately.

PH means I look at my phriends (others in the PH community) and stand in awe of their fights. I know I have it easy compared to some. Many of them are attached 24/7 to pumps that deposit medication directly into their bodies through a tube in their chest. Mere minutes off these medications could be life threatening. The side effects of the medication can be incredibly debilitating – but I’m told even intense physical pain, nausea/diarrhea , flushing, rashes, and line infections beat feeling like you can’t take another breath or get out of bed. And I believe them. It means waiting desperately for a phone call that donor heart and lungs have been found, but knowing that after transplant a whole different fight begins. I also can’t help but wonder if this is my future too. PH is, after all, a progressive disease, and I have no idea how I would handle some of that. Some of them are parents of PH patients – I can’t even imagine what that must be like, but they are some incredibly strong people.

PH means some good things too. It means I’ve found out I’m stronger than I knew. It means I used to believe in miracles, but now I’ve lived them. It means I’ve found a network of friends who fight the same fight I do and I am so deeply grateful for them. It means my friends and family I had around before I was sick  have rallied around me more than ever, and I feel their support and love daily. It means I’ve renewed and deepened my faith in God, and also made a few concessions towards modern medicine that I might not have, had I not gotten sick. It means I look towards the future with a sense of insecurity I hate, but that is balanced with unshakable faith. It means that cliche of “when you get sick every moment becomes more precious” is actually true.

I’m not really sure what else I’m going to write about. I think I’ll take it as it comes. If anyone reading this wants to hear more about something, I’m open to inspiration. Also, I would love it if you would leave a “I was here!” comment so I know, well, that you were here! :)
Take Care