Monday, November 26, 2012

A Matter of Perception

Well, PH Awareness Month is winding down and there is one more topic I wanted to address this month.

I want to talk about death. I realize this is not a comfortable topic for people in our culture. It's not a particularly comfortable topic for me either, although I would wager my take on it is a little bit different than some might expect. But I promise not to dwell on the macabre here. So bear with me.

Let's be frank for a moment. The life expectancy with PH sucks. Completely and utterly sucks. When I was diagnosed almost five years ago they told me life expectancy was about 50% at five years. That means I had a 50/50 chance of making it to... well, this January, actually! Odds are, I'm going to beat those odds. :)

New treatments keep coming, and now they've bumped up average survival to more like seven years (According to REVEAL). Yippee. I am certain I have socks in my drawer that are older than that. So, yeah, I've had to contemplate my own mortality a bit more than your average 30-something adult.

I have ways of dealing with this that I've gleaned from other survivors. Like, telling myself I'm not a statistic. Or reminding myself there is no expiration date stamped on the bottom of my foot telling me when my shelf life is up. And these things help.

But the other way I've had to deal with this is simply to come to terms with it. I was asked recently by another patient if I thought I was going to die soon. This took me completely by surprise... until they explained that their perception was that I talked about death as if it was imminent. I had to chew on that one for a while. And I came to the conclusion that I do indeed address the topic of death pretty head on. Do I intend to be here to see my six year old son graduate from high school, move on to college, someday get married... of course I do! But I've also somehow learned to live for a bright future while acknowledging the more shadowy places that may come, and preparing for them.

It's not so different than buying life insurance, really. You prepare for the worst and hope for the best. It's just that my preparations are a bit more... pressing. I know what songs I want at my funeral, I know what I will leave my son to remember me by. I know what message I want to leave behind. I have faith that I won't need these things in the near future. But, which is worse? Knowing the legacy and message you want to leave behind, and never getting the chance, or facing it and making sure it happens?

And speaking of the worst, you know what's REALLY the worst? The way we talk about those who have passed after an illness. Think about what we always say, "Oh, she lost her battle with PH." (cancer, AIDS, etc)

Say what???

Think about it. If your neighbor crosses a busy street and meets an unfortunate end, we don't say, "He lost his battle with a speeding bus."

If your grandmother lives to be a ripe old age of 97 and passes quietly in her sleep, we don't say, "She lost her battle with aging."

No, of course not. And that sounds ridiculous, doesn't it? So why in heaven's name would we take the strongest, bravest, most dedicated fighters for life, those who have major illness, and decide that once their time has come, they have lost?

I don't believe I have lost anything. Sure, PH has taken things from me. It might even take more. But, at the end of this, I have not and will not have lost a battle. Not if I fight a good fight, keep my dignity, and keep trying like hell to make a difference. No way. I will have won. My time will simply be up.

So, please, please, if that time comes, do not say I have lost my battle (I might come back and haunt you if you do!). Instead, look to the journey as just that, a journey. And celebrate that.

It is life after all. As far as I know, nobody gets out of life alive.

Tuesday, November 20, 2012

A Semi-Charmed Kind of Life (A Thanksgiving Post)

Because of PH I am thankful for (in no particular order):

1) Dr. Joseph Marakovits: a cardiologist out of Bristol Hospital who diagnosed me. Most patients go months or even years seeking answers. I wasn't even aware I was sick yet, blaming my symptoms on other more benign things. I landed in the ER for something unrelated, and after the x-ray there showed my enlarged heart, his office did my first echo cardiogram. I am certain he knew I had PH at that moment, and he fast-tracked me to the right testing, and then to the right specialist, in record time. He may have saved my life.

2) My son: I got pregnant at an "inconvenient" time. So inconvenient in fact, that I was sure that God had some sort of plan I didn't know about yet, and that's why it happened when it did. Shortly after my son turned one I found out that not only did I have a life-threatening disease, but that to get pregnant again would almost certainly kill me. Had things happened in "my time", I would not have ever had a child.

Dihann Wilcox, APRN and Dr. Raymond Foley
3) My excellent medical care: I am lucky to love and trust my doctor, nurse practitioner, and pretty much his whole office. I am confident I am getting the best care possible, that they have my back, and that they will listen when we have a difference of opinion.

A One Month's Supply
4) The advances of science and medicine:  Prior to 1995 there were exactly zero treatments for PH. Now we have nine (I take three of them), and more on the way. Without them I would be either severely disabled or dead.

5) Medical Insurance: We estimate that my medication will cost upwards of $245,000 this year alone. That's before all the doctor visits and thousands upon thousands of dollars in annual testing.

6) My friends and family: Who support me unconditionally, even though I "don't look sick", or even need a whole lot at the moment. They are still there at the drop of a hat and rally for anything.

7) My hot Irish temper: Even though it pains me to "get my Irish up", even though I despise the feeling of vulnerability it insights, and the fact that I'm likely to burst into tears right after, the fact that I can get mad sometimes does get results. I'm seeing real change in some medical related practices because I got mad enough to say "THIS ISN'T RIGHT!" (and then do something about it). And frankly I think getting mad enough at PH to fight back all that much harder can be a really healthy thing too.

8) The Pulmonary Hypertension Association: Who has created my PH community, who gives me an outlet to fight back, and who was crazy enough to put the relatively young and more than relatively outspoken patient on their Board of Trustees and take their chances with the results. I feel like I can make a difference here, and I think that helps me continue to heal just as much as anything.

Members of PHA's Generation Hope gather in NYC

9) Other PH Patients: Who give me a reason to fight harder every day. Nobody should go through what so many of them go through. Nobody should bury a child with this disease. Nobody should fear leaving their child without a parent. Nobody should suffer the way PH and even the medications make us suffer. And there is no real rest until all of this is only a thing of the past.

And so, I find that with all of the struggle and fear of this disease, it has also brought about some amazing things. Things I would not otherwise have been cognizant of.

If you were given the chance to make your life infinitely better, but had to loose something precious in exchange, would you do it?

PH has afforded some pretty damn incredible opportunities and I would be both ungrateful and stupid if I overlooked them, even for a day, and I would like to think that I've taken this rotten situation and made a difference.

Of course I wish I'd never gotten PH, But, if given the chance, would I give it all back tomorrow? Would I trade all I've been able to do and see, trade in the incredible people I've met, for my health and some sort of peace of mind? I honestly have no idea. Would you?

Sunday, November 18, 2012

You're Doing What?

It has come and gone! A group of 50 dancers and five instructors gathered on Friday, November 9th to "Shake it for PH"... and, if I may say so, we did so in style. :)

Really, it was such a blast. I can't believe how well everything went. (I even asked my husband to confess if anything had gone wrong behind the scenes that I didn't know about, and he had nothing to report).

Doing these things is always eye opening for me. I wrote earlier about how I am a rather reluctant fundraiser. All of that was happily forgotten as I watched friends, old and new, come together to support the PH cause. And there was a good handful of people I didn't know at all there too. They probably just came for the Zumba (Good! That was the idea!), but I hope they left knowing a little bit more about PH too.

I was so touched at how people came together. I think it's safe to say that through this process new friendships were formed and old ones deepened - priceless.

You can see lots of pics HERE.

Now... there's something I really want to address. This is the second annual Zumbathon. And both years I have fielded questions from PHers on what exactly I was thinking with this event, and questions on why I would want to hold an athletic event at all, and was I participating? Hard questions to answer, because the emotions behind them can run high. After all, PH more often than not robs you of even typical physical stamina, and that is hard to deal with.

So, here's why I did it: Zumba is ridiculously popular. People who Zumba likely don't come in contact with a lot of PHers. I could therefor throw an event that would draw in a community of people who had never heard of PH and would come first for the Zumba, but leave with so much more. And this theory works.

Full disclosure: I can Zumba. And I work out several times a week. Most PHers can not. This makes me both very very grateful, and more than a little guilty feeling. I have the active sick person's version of survivor's guilt, I think. I struggle with it. A lot. I simply don't want other patients to feel bad. But I don't really want to hide it either. Heck, I kind of want to celebrate. Because I know what a gift this is, and I worked very hard for it.

I really want to see the holistic health focus in the PH Community continue to grow. I remember very clearly the first day I tried to work out in my basement after I was properly medicated and starting to feel better. I lasted two minutes, and ended up on my couch desperately trying not to pass out. But once the room stopped spinning, I got up. And I did it again. And again. And again. And I changed my eating habits, and spent time in meditation, and worked with a naturopath, and just generally set my mind on the whole idea of gaining some sort of good activity level back.

It took me maybe a year of this just to feel like I could go out past my basement, or walk around my neighborhood. And when I did, I started water aerobics, and wore a floaty, and hung out with the old ladies... they were working at just my speed. And I kept going. And going.

A little over a year ago, I took a deep breath and walked into Valley Ballet (host of the Zumbathon) to check out their fitness classes. Dancing is in my blood. It was, by far, one of the hardest things taken from me when I got sick. I thought maybe I was ready to try again. It was really challenging at first, but I did it. And I'm still doing it. And I never once leave that studio without a deep sense of appreciation for the gift I have been given.

Now, maybe that can't be the story for every PHer. In fact, I'm sure it can't be. The disability that comes with PH is VERY real. I am also very aware that there is a chance it may be a part of my future. But I do believe that we, all of us, sick or not, can push ourselves further to better health. It is not easy. It isn't meant to be easy. But it took us a lifetime, or maybe a catastrophic illness, to get us where we are today, and it will take time and significant effort to work towards improvement. Giving up is easy. Not accepting the status quo, and working like hell to change it, is what makes a real difference. Besides raising money for PH, and spreading awareness, my own private little reason for doing a Zumbathon is to honor that process, and celebrate the results.

Monday, November 5, 2012

In Summary

I recently wrote about how I visited the FDA to provide public commentary on a new patient-focused initiative they are working on. As it turned out, I only had two minutes to deliver my remarks. There was so much more I wish I could have said! I thought I'd put my first much longer draft here. It kind of summarizes how I see my PH, and the bigger picture of PH, today. Good for awareness month. :)

And speaking of awareness... I am REALLY aware that the Zumbathon is in less than five days! Still time to donate! (end shameless plug):

I was diagnosed at the age of 28. I went from a young mom and wife, quickly climbing the ladder in a successful career, to suddenly facing down my own mortality with a disease that I was originally led to believe might kill me in less than five years. I went from planning a life, to worrying that I might not see my baby boy reach Kindergarten.

Well, that baby boy entered first grade this fall. And I was there. And between that scary uncertain time back then, to today, I have learned a great deal.

First, I have learned that hope is in fact a verb. While the dictionary defines it at as a noun, to me hope is a state of constant action. It is a state of fighting not only against a disease, but also for a community that has taught me so much.

When you get a diagnosis of pulmonary hypertension, or PH for short, your life instantly changes. For some patients, it means a sudden stop of activities once enjoyed, because you quickly find you no longer have the breath or endurance to sustain them. For other patients, it is finally the answer they were seeking, after many months, years even, of searching for an explanation to their symptoms of breathlessness, dizziness, edema, and fatigue. Whatever the case, the diagnosis does bring change.

Nearly ¾ of patients who finally get a diagnosis are already in advanced stages, and their quality of life and possible activity level is severely impacted. Medications can and do help this condition, but at a very dear price. Not only are they extremely expensive, but the side effects of many of them can sometimes be just as debilitating as the disease itself.

Pulmonary Hypertension is progressive disease, and that means even after you have answers, things are still changing. There is a great deal of up and down. After treatments, your breathing and functioning is likely to improve. Some patients see great improvement, like myself, while others fight to still simply survive. It’s not terribly unusual for a patient to find themselves attached to a pump delivering 24/7 medication, and an oxygen tank as well, and taking medications to combat the side effects of the PH medications.

So far, this has actually not been my personal experience.  I struggled to climb a flight of stairs when I was diagnosed. After a couple of oral medications, I remember the huge celebration I had when that changed. I called another friend with PH to share my news. Still though, I struggled, and my doctor began to feel it was time to start the IV medication option.

This was something I simply could not wrap my head around. I couldn’t imagine balancing a pump, and a line, and the side effects, and my active son. As luck, or grace as I think of it, would have it, a new inhaled medication had just come on the market and I was the first one in my clinic to try it. The results were outstanding for me. My old energy levels began to return and after many months of careful conditioning, I even returned to a physical activity level I thought was forever lost to me.

I can’t even begin to describe what a gift like this is like. I am deeply deeply grateful for my medical team, for the research that has made this possible, and for a faith and spirit I have been given that make me a fighter. The other side of this is that I am always acutely aware that it could in fact be temporary. I’m doing great. But PH is still PH. It’s still progressive. It’s still incurable. The ultimate treatments are still extreme. And they may be in my future.

In the meantime though, every moment has become even more precious, as cliché as that could sound. I look to channel this energy and drive into supporting my community, because to do anything less would hardly be right. So, I stay very active in the PH Community, doing everything I can to try to shape a future where other patients can experience what I have, and to ensure that the future of PH is as bright as possible.

We could talk for hours about the science of disease progression, and how medication can mitigate that, and how that mitigation could be dramatic, or minor, or temporary, depending on the patient. And all those things are indeed well worth considering.

But what medication simply can not touch is the emotional aspect of this disease. When I was first diagnosed, it was like looking in a dark crystal ball. It was like waiting for the darkness to fall, and that is no way to live at all. I told you at the beginning that hope is a verb. Well, it is. Hope is patients coming together to support each other, to help one another learn to navigate what can sometimes be the murky waters of medication management, or healthcare system navigation. Truly, having PH can be a full time job sometimes!

We are blessed to be led by the Pulmonary Hypertension Association, which has blazed a trail of research, education and support. There is a community there that is like nothing I have ever seen. Patients, doctors, caregivers, allied health professionals, volunteers… all coming together to put hope in action, and to spur us forward until we do indeed reach a cure.

PH changes your life in dramatic ways. The medications you need and how you’re functioning can change in a moment. But what doesn’t change is the hope we have. The scientific community has made amazing breakthroughs, and things can only get progress in positive from here.

Thursday, November 1, 2012

Volunteers Needed

Welcome to PH Awareness Month!

To kick things off, I'd like to share a video with you. I created this message to send to the South Korea Pulmonary Hypertension Conference in October.

I had been asked to attend, but that many hours in flight felt a little too daunting for my life right now. I was really bummed to say no, but thrilled that I was able to offer this up instead.

Here's the thing... we all know we're living in "tough times". From social issues, to financial markets, to government in flux, to people's personal accounts, things are shaky. Add to that increased medical field regulation, decreased corporate support, and the fact that PH is just plain old rare, and we're looking at more than a few challenges to move our cause forward here.

But these challenges are not insurmountable. We NEED everyone, every single person, who has a vested interest in seeing improved treatments and an eventual cure to PH to throw themselves into the cause somehow. Fundraising. Awareness raising. Media. Social Media. Supporting other patients. Just talking to friends and family.  To volunteer means to make a difference. And that is priceless. What are you up to this month? I'd love to hear about it.

Saturday, October 27, 2012

Back to DC

This week I traveled to Washington DC (Well, Silver Springs, MD, more specifically) to deliver comments to the FDA in support of Pulmonary Hypertension becoming a part of the new Patient Focused Drug Development Initiative. In summary, the FDA has created an initial short list of 40 rare diseases to potentially study, and PH is on there. Now we are, in essence, competing to land on the final short list of 20 diseases. My task was to deliver remarks that would hopefully support an FDA decision to choose us. In short, they are looking to study these chosen diseases more in-depth from a patient perspective. When treating a rare disease there is significant cost/benefit analysis that must be undertaken with the drugs. They can be terribly toxic to our systems, and very difficult to manage, but to many people those risks are worth the benefit of being able to, let’s say… breathe. Or live. The FDA wants to take a closer look at the patient perspective during clinical trials and drug approval. A movement I personally applaud.

The process was very interesting. I arrived at the FDA with a PHA staffer and my friend Joshua Griffis, who is involved in the project from the Association end. We met up with Dane Christensen, formally my guide through the Congressional offices when I went to try and secure sponsorship for the Tom Lantos Pulmonary HypertensionResearch and Education Act this past May, and now one of my coaches and guides through this whole FDA thing. Cheering us on from the PHA office via the live webcast was Katie Kroner, Director of Advocacy and Awareness, also a key player in prepping me for this.

The FDA provided panel discussions from their experts that helped us better understand their initial selection process and what exactly they were looking for in this round. There was to be open commentary opportunity for the public, and because of the crowd, we had to kind of scramble to be sure I was signed up to speak. Dane rocked it, got there early, and got me on the list as #8.

We were originally told that I would have five minutes to speak, and I prepped for four in order to leave a little wiggle room. Then a few days before the event, when the significant public interest in the event became more evident, there was rumor that we might be cut down to two or three minutes. So, I ended up showing up with two speeches, one that lasted four minutes and one that lasted a little over two. It wasn’t until the event was under way, and they saw how many people had signed up, that we were finally told we would indeed have only two minutes to make our case. I had to make further cuts to be sure we were well within time limits. It was shaking in your stilettos nerve wracking. (Note to self: wear more solid shoes if I'm going to do public speaking and thus shake a little!)

I’ll include my commentary at the end of the blog. I’m hoping for an audio recording, but am not sure if one will end up being available or not. Before I get into that though, I have to say, this was an incredibly humbling and eye-opening experience. PH sucks, no doubt. But out of all the 7,000 rare diseases in the USA, we sure are amongst some other pretty serious stuff.

I learned a lot and am really glad I got to listen to the testimonies of people on conditions such as Chronic Fatigue Syndrome, ALS, Muscular Dystrophies… A fathers speaking of a disease that will kill him and leave behind young children because he has no treatments available, a 10 year old boy speaking so frankly from his wheelchair about how he and his brother will soon no longer be able to feed themselves and have a dramatically short life expectancy… I can only say that while we have a hell of a fight on our hands with PH, at least we have some good weapons. Others are not yet so blessed.

Now, we wait. Hopefully the FDA will come back with their decision before the end of the year, we shall see.

(Ignore any weird font. Blogger is being persnickety)

Hello, thank you for having me here today. Before I begin I would like to extend my sincerest thanks to the FDA for undertaking this tremendously important initiative. My name is Colleen Brunetti and I am a pulmonary hypertension patient.

I was diagnosed at the age of 28. I went from planning a life to worrying that I might not see my baby boy reach Kindergarten.

Well, that baby boy entered first grade this fall, and I was there. And with the help of the Pulmonary Hypertension Association, I’ve learned a lot since those first early months.

When you get a diagnosis of pulmonary hypertension, or PH for short, your life changes. For some patients, it means a sudden stop of activities once enjoyed, because you quickly find you no longer have the breath or endurance to sustain them. For other patients, it is finally the answer they were seeking, after too long of searching for an explanation to their symptoms. For me, I also found out I had an auto-immune condition as well as pulmonary hypertension, a common scenario with PH.

Because PH is often misdiagnosed, many who finally get a diagnosis are already in advanced stages, with quality of life severely impacted. Medications can help, but often with severe side effects. The only cure remains a lung and sometimes also heart transplant.

While medication can mitigate symptoms of PH and slow disease progress, that could be dramatic, or minor, or temporary, depending on the patient.

Even when therapy is successful, PH is still PH. It’s still progressive and the ultimate treatments are still extreme

The Pulmonary Hypertension Association, has blazed a trail of research, education and support. Patients, doctors, caregivers, allied health professionals, volunteers… all coming together to put hope in action, and to spur us forward until we do indeed reach a cure.

Please select “pulmonary arterial hypertension” and “organ transplantation” as disease areas for the initial 5-year pilot program of the Patient-focused Drug Development Initiative. PHA and the PH community are dedicated to working with FDA in a meaningful and constructive way to ensure this pilot program is a success. It is not only our goal to see that the patient experience better informs regulatory decision-making for PH, but that this effort grows into a sustained campaign of close collaboration between FDA and all patient communities, which continuously improves the system for reviewing and approving innovative therapies.     

Thank you.

Sunday, October 21, 2012

Confessions of a Reluctant Fundraiser

It's here, it's here!

The 2nd annual Zumbathon for Pulmonary Hypertension!

Or, as I've affectionately dubbed it "Shake it for PH!"

It will be Friday, November 9th at the Valley Ballet in Canton, CT. Doors open at 6:30, Zumba starts at 7:00. Full information and how to register or donate is HERE.

Can I let you in on a little secret? I hate doing fundraising. I really really do. In fact, there was a time that I wrote on this blog that fundraising was not for me. I still kind of feel that way, but the drive to move us forward in this disease has trumped that. Truth be told, there is so much good coming from pushing myself out of my comfort zone, that it is really worth it. But, first, my confessions.

I hate asking for help. I am really uncomfortable saying "I need this!" I think it's mostly because I'd much rather be giving the help. It's hard for me to be on the other side. It's hard for me still to admit that PH is so freaking serious that massive action has to be taken if I, or anyone, am to survive it.

But I am indeed here. Until someone hands me a cure, I'm here, and I'm in it deep. Since PH is a rare disease, it pretty much stands to reason that nobody else is going to step up, unless those of us most directly involved in the community do it ourselves (hint-hint).

As I approach people and have to explain it for the first time it starts with,

"I'm doing a fundraiser for Pulmonary Hypertension. Have you heard of it? No? Well, PH is a rare disease of the pulmonary arteries. The hypertension is in there, not your whole body, and it backs up pressure to the heart. Which has to work too hard, then it enlarges, and can ultimately fail. It is progressive and there is no cure."

Pause. Watch them process.

Then say, "I have it."

Watch them process that.

If you want to have a little fun, lighten the mood if you will, you can always throw in, "AND, I take Viagra!" (Actually, I take Cialis, but same thing... more or less.)

With people who already know about the disease it's a little different. It's asking for help of people who already know you're sick. And here's my other dirty little secret. I don't like doing that either. I wish they'd just magically help on their own. But... life isn't like that. Not because they don't care though. Everyone is pulled in a zillion directions, with a zillion possible things to tug on heart strings, demand attention, request or require support. Let's face it, our world is kind of messed up. So it's up to me, it's up to you if it's important enough, to lead the charge.

Okay, I can do that.  And what I can DEFINITELY do is throw a party!! So, in just a couple of short weeks, we will "Shake it for PH", and we will do so in style.

Five instructors. 50+ participants. One very very fun night, for a great cause!

And... I would be so remiss not to mention that doing this makes me appreciate the goodness in people too. And that TOTALLY wins. First, PHA makes this so easy, with awesome help from their event staff. My husband will take the day off from work and, I can already tell you now, knock himself out doing whatever is needed. His direct co-workers have all donated. They don't even know me. A classmate of mine donated. He's spoken to me once. A friend from the dance studio is working so hard creating the best event t-shirts ever. My Zumba instructor is coordinating music and instructors. The owner of the dance studio is donating her space. Several friends are stepping up in big ways to secure donations, sell tickets, etc. Two friends and one sister will travel from out of state just to be by my side. All of this humbles me, and makes me so grateful. Big time.

As I've said before, if I have to be here, it's worth being here.

Tuesday, September 11, 2012

A Makeover

So, if you haven't seen it, the blog got a make-over!

Hope you like the new format with the added resources! I welcome your feedback as I try to convert this into both a regular old blog and a resource.

As it turns out, people are reading this thing! And more than one patient has contacted me and told me they found it when newly diagnosed and searching for answers. That makes every word, edit, and moment of questioning if I want to put some of this stuff out there worth it.

And... eventually, I'll get a new post up here. In the meantime, explore some of the resources. The PHA has been busy, and I borrowed from their hard work. :)

Tuesday, July 17, 2012

My Comedy of Errors

Today I had my six month work-up. For me this was to entail an echo cardiogram and a pulmonary function test (PFT) on a bike. I don't get six minute walks at this point because they don't like that I burn up the floor and walk too far. (Kidding... but they really are kind of useless with me at this juncture).

No matter how long I'm in this (working on five years - wow!), I still get the jitters the day before and of these tests. For a long time I was steeling myself for more bad news, or at least no good news. When that changed to getting good news I now keep hoping for more good news, or at least no new bad news. Kind of a never ending cycle.

So, after a restless night I dragged myself out of bed about 45 minutes late, which meant no breakfast because I wasn't supposed to eat after 7am for the PFT. Got my son off to camp and headed to the hospital.

The echo was fine. They are really no big deal and I spent the time chatting with the tech about right heart enlargement and some cool new technology I looked at during the recent PH International Conference. Usually it is my husband who embraces being the geek in our family, but I now get all sorts of interested and excited with medical technology, so the nice tech played with the 3D echo machine for me a bit too, just so I could see it. Cool stuff.

I had a few minutes to kill on a sunny terrace and then it was back in the hospital for the PFT. I was greeted by one of my favorite medical techs. He often did my six minute walks. Nicest guy, and we always have the same conversation. He asks how my "baby" is, and I politely smile and tell him my baby is now almost 6, and thanks for asking. He always looks shocked and convinced the timing is wrong. And I always have to do the math with him on how long he's been working at the hospital and how long I've been coming there... and, oh yes, it's true, my kid is almost 6. It's actually kind of endearing because he just means so well.

I get into the echo room and there's another tech, a lady that I also like. I am informed they will do the PFT together because the regular girl is out and they are "new" at this. Um... okay. I can roll with that. So it's a bit of a production getting all hooked up to the wires and such, and before I can do that, I have to clean off with some alcohol prep pads.

When asked to do so, I comply. And then I look at the female tech (the guy having high tailed it out of there in embarrassment since I had to lift my shirt... again, endearing) and I said, "This shit stinks!"

Yes, I said that. Somewhere around my second or third right heart cath I realized that things like half brazillians and painful (physically or emotionally) procedures were pretty much here to stay for me, and I lost both my dignity and my inner filter.  I actually decided to start telling medical people just how it felt when they did something to me because, "This is going to sting a bit" was constantly an understatement and sometimes you just need to say, "Oh holy hell! Stop that and give me some good drugs first." Or something like that...

Anyway, that shit DID stink. And when we looked at the package it became clear why. It was nail polish remover I had just spread all over my torso. Nail. Polish. Remover. I was not wearing nail polish anywhere but on my toes, which doesn't count in this test.

So, I got to give myself a sponge bath, because you know, that's what you do in hospitals, but isn't it supposed to be done by a cute nurse? THEN to the alcohol pads, then to the wires (or leads) being attached all over.

It's a long ordeal, and at the end, I look like this:

MISSING: The lovely nose clip so I can only breath through the tube, and also a pulse oximeter.

So I get all hooked up, hop on my sweet ride, and the test begins. Except it doesn't. First, they can't get a reading and one of the leads must be off. I feel around inside my shirt and find the loose one which at first I think is popping off because it was around the curve of my rib cage. But then we figure out the tech just never took the sticker off so it is sticking to... nothing. Easy fix.

Then they can't get a breath reading. But I promise, I was breathing! And we discover (ah-hem, I discover) that since they didn't plug my nose I was using it to breathe, silly me, so the tube wasn't picking up the air.

All of that gets straightened out and we begin. I peddle, and peddle, and peddle. And nothing. There's no resistance. It's easy. Too easy.

Now I have a little claustrophobia and a gag reflex that would kill a cat. Shoving a tube in my mouth and plugging my nose does not go over very well for me. Heaven forbid I ever need a ventilator and I'm certain I would be terrible at scuba diving. But I really try hard to power through it. And I did manage for a while as I peddled uselessly and the two techs and the doctor fussed over the machine, which clearly was not working. For nine whole minutes.

I finally got to get off the bike while they figured out what was going on.

Back on. Everything is fixed. The test begins in earnest 45 minutes after it actually began.  And it gets challenging fast. Every time I take a good breath, the belt holding all my leads to me pops apart and everything starts to fall. So I'm trying not to gag, trying not to panic (claustrophobic feeling in full effect!) and trying to gesture and make some noise so they can hook me up again. By the third time it pops off I give up and just let the darn thing hang there. So not my problem!

All of this extra distraction takes my attention away from concentrating on my breathing through the tube, the bike resistance is getting harder and harder, I cough, can't take a recovery breath, and then chest tightness and a full blown panic, like I'm drowning, takes over. I just lost my focus. I broke out in a full sweat, my eyes watered, and I spit the tube out of my mouth to draw a real breath. And gag. And almost threw up on the doctor.

The mouth tube collects spit. And I let it fly. If you ever sat next to the trombone player in middle school, you can guess what the floor below me looked like after that. Whoops. Like I said, dignity out the door.

Yup, failed that test! Well, pretty sure I did. With the false start, there was also a lot of talk of there being false results because I had been exercised before the real data could be taken. And then there was the whole spit flying gagging thing. So I may or may not have to go back and do it again. Yipee.

But, I am a good patient. Which means I smiled at everyone and told them all the little snafus were no big deal. And then I took myself out for lunch. And a large glass of wine. Because I earned it! And, well, because that's what I always do after a test.
The World's Best Turkey Burger (with goat cheese and roasted tomato)

Monday, June 25, 2012


The 10th Annual Pulmonary Hypertension International Conference is done.

I stayed an extra day to hit up Sea World. However, Tropical Storm Debby had other ideas, and I spent two days in the hotel hiding from sheets of rain and whipping wind. Not so bad though… no amount of cute dolphins could trump hanging with a friend who makes you laugh so hard your face hurts, or long talks over bottles of wine. So, it’s all good. Wouldn’t trade it, actually. Sea World can wait.

And now I’m sitting here on the plane ride home. And while Conference closed on Sunday afternoon and it is now Monday night, it is just now I can start to process it all. I kept flipping through my pictures. And suddenly, my throat closed up and I felt the tears coming. Not so cool on an airplane… finally sitting still for a few minutes, alone with my thoughts, and it’s hitting me. I’m a little dizzy with what has happened. It’s a blur, but bits and pieces are floating into focus and I’m struck, as I so often am, at the enormity of what I’ve become involved in.

Becoming a part of something that could change the course of a disease…IS changing the course of a disease. Impacting people’s lives. Meeting people who rock my little world. Becoming close to other patients… and all that comes with that. It’s a crazy thing to totally love someone (many someones) with chronic illness and, more than that, who share your illness. I find myself feeling sometimes fiercely protective of their care and wellbeing, and deeply worried when things don’t seem right, and crazy-happy for them when things go well… and most of these people I’ve only spent a few hours with, if any at all. Weird.

So here’s the Conference story… how does one describe Conference? As is so often the case with PH, I feel at a loss for words. Pretty sure I can find some though.


Starting with the Board Meeting on Thursday before Conference began. I joined the Board of Trustees just a little over a year ago, and I have yet to loose the feeling that I am walking amongst giants. For a while, I was just a little star struck (uncharacteristic for me), and now in place of that grows a sense of… I don’t know what exactly. I’m beginning to be able to call these people friends. In fact, I know I can. And it’s a group of incredible human beings. Each has dedicated themselves to the cause of fighting PH. Some as medical professionals who have made this their career. Too many who have lost loved ones and fight on in their honor. A few who still fight for their children who have the disease. Patients – even a former patient. Damn. That’s really all I can say. And it’s so crazy cool to sit in a room that becomes a giant think-tank for PHA, and then a few months later see those ideas in full swing… like the “Sometimes It’s PH” campaign we launched at Conference.

I got a chance to do a presentation to the International Leaders Summit – representatives from all over the world who fight PH in their own country. I spoke on creating patient connections. It was a cool opportunity to sit back and reflect on how PHA reaches out to the patient population and builds platforms that encourages support – very cool.

When I left Conference two years ago, the one piece I still had missing was the chance to connect and talk with other parents, moms especially, who had PH and were trying to raise kids. Well, PHA gave me an incredible opportunity and I was able to chair a patient-led panel with two great friends so we could address the topic. Wendy and Hannah have both adopted beautiful kids since their diagnosis, and we were able to talk about our experiences and give other patients hope that having this disease and a family at the same time is indeed possible. The next day I also led a support group for patients on the same topic, also a great chance to swap stories and tips, and learn from each other.

Hannah, Me, Wendy

And then there was this little assignment I had that has had me shaking in my shoes for weeks. I was blown away to be asked to be the keynote speaker for Saturday night. Me. Talk to a room of 1500 people. Crap. I so badly wanted to say no. The thought was terrifying. And yet, how could I let such an opportunity pass? So, I said yes. And it was… surreal. I spent weeks practicing my speech. I even recorded myself on my phone and listened to myself over and over again (thanks for the idea, Mom – brilliant!).

Well, I’ve done some public speaking before, with mixed results.  Some have gone well and others I’ve been horribly nervous and shaking, and it showed. Badly. I really wasn’t sure what to expect with this one. But, I climbed that stage and sat there almost deadly calm. It is a calmness I have experienced before just ahead of a large event, it’s a calmness that comes from knowing I was doing the exact right thing. Oh, and the wine beforehand probably didn’t hurt. ;)

Anyway, I got up there and delivered my speech, and the response was… well, overwhelming. People were incredibly warm and generous in their feedback afterwards. A few said things that will probably stick with me for the rest of my life.  I don’t know. I think I’ll have to let the speech speak for itself (ha – that sounds weird). I’ll post it when it’s available.

I finished off the Conference hanging out in the Kids’ Room and did a sign language lesson with them. It was really fun to connect with some of the pediatric patients and also siblings of patients. We had such a fun time. I’m a little nervous to see pictures and video from that though – I look like a nut when I’m jumping around and signing with the kiddos. But they love it, and it’s totally worth it.

And then there were all these little moments I couldn’t possibly capture right. A minute or two connecting with someone I’ve known only on-line. Someone saying something incredibly sweet. The feeling caught in my throat as I looked at a patient I knew was failing and struggling. Laughing so hard I lost my breath. The hugs of those who have become dear to me. Eye contact and smiles when someone just “gets it”. And the tears that well up when that happens too.  Because, truly, there’s nothing quite like being with those who understand on a profoundly deep and impossible to describe level what it is to have Pulmonary Hypertension.

Yeah… so, that’s Conference. 

My Generation Hope Patient Advisory Board cohorts: Joshua, Me, Brit, Sean

Wednesday, May 30, 2012

For Good

PH is always a one-two punch. Up. Down. Fly. Crash. A great appointment? Flying! A bad one... seismic crash. An incredible time connecting with other people who really get you (see posts below). Fly. Another patient dies... a child, like Molly Dunne, or someone just your age, as happened again this week,  and it's the crash again. A dear friend in dire health straights... and you freeze, just waiting and praying for it to be okay. And the events always seem to come together. Up. Down. Crash.

Last year I sat in a funeral for a baby girl, the daughter of a friend. There was absolutely nothing in that moment that could have been said to me to bring peace or make the situation okay, at least I didn't think so. How do you justify tragedy?

Well, the priest somehow managed to find just the right words. He spoke of our tears that day, how they were blessed. He said we have a choice in life. We can open our hearts and love, and risk our hearts being broken. Or, we can keep our hearts closed, and the price for that is a shriveled and cold heart. Each of us sat there that day totally broken-hearted, the tears flowing. And yet, it was because of the choice we had made to love the family who was saying goodbye to their baby girl. And so, because we opened our hearts, those tears were blessed. We had done the right thing, as painful as it was.

In that moment, I did feel peace. The pain of what I was witnessing didn't leave, but somehow, somehow... it was going to be okay, because in the human experience, here we were with open and broken hearts, loving someone who needed it.

I could not help but to relate all this to having Pulmonary Hypertension too. Being so involved in the PH community means I see and experience a lot of heart break. A lot. More than I feel like I can handle sometimes. And yet, I've chosen to open my heart. And if I have to be here, it is a blessed place to be. To make a difference. To have people coming into my life and change it in ways I wouldn't have imagined. I never knew I could so unconditionally love people I had never met, or only spent a handful of hours with, and yet... here we are.

I am totally obsessed with the song "For Good" from Wicked, have been for a long time, but it's been the kind of couple of weeks where music really grabs you (well, grabs me, anyway) and helps you through a rough patch.

The song speaks of how people come into our lives for a reason, how they help us, and we help them in return, and all of this results in changes in our world, big changes, for good.

I guess that sums up the PH experience right now. Alone, we are limited. Together, we can change each other for good.

I can't find a good original clip of the song online, but Glee did an amazing version:

And they lyrics...

(Elphaba) I'm limited Just look at me - I'm limited
And just look at you  
You can do all I couldn't do, Glinda  
So now it's up to you  
For both of us - now it's up to you...

(Glinda): I've heard it said
That people come into our lives for a reason  
Bringing something we must learn
 And we are led  
To those who help us most to grow
If we let them  
And we help them in return  
Well, I don't know if I believe that's true  
But I know I'm who I am today
 Because I knew you...
Like a comet pulled from orbit  
As it passes a sun  
Like a stream that meets a boulder  
Halfway through the wood  
Who can say if I've been changed for the better?
But because I knew you I have been changed for good

(Elphaba): It well may be  
That we will never meet again
 In this lifetime  
So let me say before we part  
So much of me
  Is made from what I learned from you
You'll be with me  
Like a handprint on my heart  
And now whatever way our stories end
I know you have re-written mine
By being my friend...
Like a ship blown from its mooring  
By a wind off the sea
  Like a seed dropped by a skybird
  In a distant wood  
Who can say if I've been changed for the better? 
 But because I knew you

(Glinda): Because I knew you

(Both): I have been changed for good

(Elphaba): And just to clear the air
I ask forgiveness
For the things I've done you blame me for

(Glinda): But then, I guess we know  
There's blame to share

(Both): And none of it seems to matter anymore

(Glinda): Like a comet pulled from orbit
As it passes a sun
 Like a stream that meets a boulder
 Halfway through the wood

(Elphaba): Like a ship blown from its mooring  
By a wind off the sea
Like a seed dropped by a bird in the wood

(Both): Who can say if I've been Changed for the better?
 I do believe I have been Changed for the better

(Glinda): And because I knew you...

(Elphaba): Because I knew you...

(Both): Because I knew you... I have been changed for good...

Copied from

Thursday, May 24, 2012

What is Healthy?

So I think I am in a period of transition. I recently went back to school at the Institute for Integrative Nutrition to become a health coach, and it's causing some shifts.

That's right. The sick girl is going to tell other people how to get healthy. Go figure! But no, really, it's an idea I'm very excited about. What I most want to do is support families who have children with special needs that benefit from a special diet. In short, there is a great deal of evidence that conditions such as ADD/ADHD and autism may be improved by dietary changes and intervention and I am totally fascinated by that.

Throughout the journey, I've been promised by the school that my life will be revolutionized as well. And it's already happening. Even before I started my studies I had been contemplating changes to our diet. For me, I want to eat much more plant based. For my son, I want to feed him in the healthiest way possible, and that means questioning some of the nutrition "facts" we've been raised with (milk does not always do a body good, folks!). For my husband this means the poor guy is kind of along for the ride as I do all the shopping and cooking... but I want this to be a good thing for him too, both for his health and for his happiness (the man loves him some hamburgers!). So, I can't just make sweeping changes in the house without an uprising. And that's really okay. Slow is better anyway.

Here's the hard part of the conversation: I also want to support my husband to be as healthy as possible because there's a lot on him as our primary financial provider and, let's face it, the one who may at some point solely care for our son. That is not a pleasant conversation. But it's in the back of my mind, always.

And then, there's me. What does it mean to be healthy when you are sick? I have two different thoughts on this, kind of like a devil on one shoulder and an angel on the other.

The little devil reminds me that I was raised on tofu and sprouts and home made yogurt (nasty stuff!) and, well, I got PH anyway! There is a limit to what diet can do and prevent. So screw it, raise a glass, pass the nachos!

And then there's a much more practical side that reminds me that to best fight PH, the rest of me has to be in the best shape possible. Thankfully, I can exercise right now and I love it, so that's good. And I really do love healthy food, unusual foods, and to cook. Eating very healthy is a huge job. There are political and social consequences to consider with the food industry, and I want to vote with my dollar with what I buy. There's the planning and prep. There's making a green smoothy one day and really liking it, and trying something new the next day and gagging and spitting it out. And some days I'm going to have to remind myself that this is worth it - that it will support me to be in top fighting shape for this PH battle.

You hear people say that if you have your health, you have everything? Or you have something go wrong and they say, "Well at least you have your health!". Well, by any definition, I don't have my health. And yet... maybe I do. Because I can still move around and make wise choices and make a decided difference in my outcome. So, screw that quote, because there's more than one way to look at this big picture.

Oh, and if I could loose these damn extra pounds, that would be nice too. Because I am certain that as stubborn as my body has been, if I could shake them, it would be so much better for my heart. And a healthy heart is the ultimate goal here.

Sunday, May 20, 2012

Getting Out of My Own Way

Just an observation... after a pretty intense week, with the incredible Generation Hope meet-up, followed by the devastation caused by the death of a little seven year old patient, Molly, and and a fair amount of fury at another smack-down with my specialty pharmacy company (who failed to deliver medication as scheduled AGAIN), I had reached my limit. I was starting to get a little irrational, as evidenced by my very very short fuse with all things annoying. My Irish temper was rearing its head and I could not decide between curling up in a ball and crying, or hitting something.

And then, I stepped away from it all for the weekend.

It wasn't exactly intentional to do so, in fact it had all been scheduled out before this shit hit the fan, but I took a break from all things PH (except my meds, of course!) After the Saturday run-around with my son, consisting of t-ball practice and a friend's birthday party, I threw my hubby a surprise birthday party on Saturday afternoon and spent today (Sunday) building a beautiful memorial garden for a dear friend who lost her baby girl last fall.

It's just dawned on me that this is the first time in a long time I haven't been focused on advocating for PH. Don't get me wrong, I love what I do. I am so incredibly honored that I even get to do it at all. But when I am advocating for PH, fighting for better care for patients and myself, reading about all the pain this disease causes and trying to help where I can... well, it can wear you down.

I'm not complaining. But I do think I'm having an epiphany of sorts. It's true - you can only care for others if you are caring for yourself.

It just felt good to dedicate a weekend to something else. Maybe I should do that intentionally a little more often.

Tuesday, May 15, 2012

Chasing Dreams, Part Two: This is Where it all Comes Together

In my last post I talked about a whirlwind trip to DC, but the weekend wasn't done yet. Now it was time for the Generation Hope Meet and Greet in NYC.

Joshua and I rented a car and grabbed PHA Staffer Debbie and her sister, fellow patient, Alex. After a great dinner (and fro-yo!) in Silver Spring we were off to NYC... starting at 9pm.

We drove through the night, laughing harder than I have in a really long time, tears streaming down our faces kind of laughing, and arrived in Newark NJ in the middle of the night, tumbling into bed close to 3am.

Then it was up and at 'em again the next morning. I could not have started my morning more perfectly... a knock on my door, and there stood Braden, a fellow patient who had come in to meet up with us. Braden is special to me. A little while back she had contacted me out of the blue because of this blog. She found it in the hospital while being diagnosed. She said it gave her hope. The fact that she reached out to let me know that made this blog all worth it. I started it to make a difference. To educate people, to tell my story, to let them know the crazy ups and downs, and maybe share a laugh about some of it (because if I don't laugh at the absurdity of this crazy disease, and the situations I have come into because of it, I just might loose it). It was incredibly gratifying to know it did make a difference for even one person. In fact, that communication has kept me writing.

 Braden rocks. I mean, I knew I liked her online persona before, but in person it was abundantly clear in about 20 seconds, she and I were going to be fast friends.

Stumbling our way through the Newark to NYC transportation system, we met up with the rest of the Generation Hope crew who had come into the city. All told, 11 patients and three caregivers that day. So cool to see people's eyes light up and to hear the squeals as connections were made with online friends now standing there in person.

Meeting for the first time

 We started at Ellen's Stardust Diner near Times Square with, what else, singing wait staff! From there it was a wander through an outdoor market and into Times Square, ending at a coffee shop on a corner.

Happy Birthday Song!

Here We Are!

 A little later on, it was down to just me, Joshua, Braden, and my very dear friend Wendy (Wendy and I met online three years ago - the first mom/PH patient I was able to connect with, and we've stayed fast friends ever since, even visiting a few times a year. It's been amazing.)

We all talked together quite a bit, and then after a while the conversation sort of broke off. Joshua and I were chatting, and Wendy and Braden began to exchange stories... finding they had so much in common. I could hear the excitement in their voices. I could hear the connections being made. After watching everyone together all day, and especially then, in that moment, it hit me. This is what we started Generation Hope for. This is what it was all about. I watched them hug their goodbyes... and I completely choked up. All of this. These past four years, from feeling so isolated and lonely and frightened out of my mind, to tentatively reaching out, to having crazy cool opportunities to reach out, to seeing all this come together... this... this is what I was meant to be here for. This is why we did what we did with Generation Hope. I honestly don't think I will ever ever forget that day, and especially that moment.

Wendy, Braden, Me, Joshua

We ended our night with seeing RENT! off Broadway... a blog post for another time as that is a show that has had very profound meaning to me since diagnosis.

Can't wait for Conference in Orlando in just over a month!

Monday, May 14, 2012

Chasing Dreams, Part One: Washington, DC

So... the weekend. Everyone is asking me about my weekend. And I've been trying to articulate it, but I can't seem to do it justice. Doesn't mean I won't try though... this blog needs an update anyway.

I have just returned from a marathon four days on the road chasing dreams and seeing some come to fruition. It's a long story, so for today let's just start with Thursday and Friday. Here's what happened...

I got up at 5am on Thursday morning and headed to the train station - bound for Washington, DC. My goal was to visit Capitol Hill and lobby for the Tom Lantos Research and Education Act of 2011.

Take a minute (and two seconds) to view this video and see why it was so important for me to do this... (direct link:

So, I arrived in DC mid-afternoon and hopped a commuter rail to Silver Spring, MD to head to the Pulmonary Hypertension Association where I had a lot of fun seeing staff and holding a few planning meetings for the upcoming 10th Annual International PH Conference. Dinner with one wonderful staffer capped off the evening. If you haven't had a chance to meet some of the PHA staff, you really should. Quite simply, some of the most wonderful people I know.

Friday it was off to Capitol Hill. I met up with the lobbyist, Dane, who is working with PHA and we were headed to the office buildings of the House and Senate.

Outside Senator Blumenthal's Office
It was an interesting experience. We had appointments with two out of three of my representatives. Well, representatives for my representatives. They send their health guys to meet with constituents.

I told my story and Dane covered the legalities of what the bill entails. Everyone was very receptive and nice, but there is much follow-up to do. It's so incredibly easy to contact your representatives and ask them to co-sign this bill. Without co-signers, we don't have a bill. With enough co-signers we are practically guaranteed this bill. Please join us! Simply visit this link and use the directions and even a letter PHA has provided: HERE

We were there on official business and so it was very cool to see the underground transportation systems between the House and the Senate. My only gripe is that security took away my snacks! Apparently, you can't bring food or drink into the Congress building... nuts, seeds, and dried fruit are dangerous stuff (well, the nuts I guess can be, but really??). Oh well... it just made for one very hungry afternoon as there was still much to do that day!

Leaving Capitol Hill shortly before noon, it was back to Silver Spring via the train for probably one of the coolest experience of my life. Fellow PH patient, my good friend Joshua, was waiting for me. Joshua and is one of my closest PH friends and he is one of the people who gets me through. You need that when you're going through this.  It helps that we share a lot of other things in common (our obsession with research on PH, and more simple things... like fabulous food, and mutual childhood crushes on Donny Osmond!) And as I was to find out, we get along in person pretty well too. Actually, we squabble a bit, and he throws little pieces of paper at me and teases me relentlessly... it's kind of like having a brother around. He cracks me up, and I have immense respect for the way he has faced this disease.

 Anyway, we were to go visit United Therapeutics (UT), whose office buildings for their company Lung, LLC just happen to be just around the corner from PHA.

If you're not familiar with United Therapeutics, I suggest you become so. They are an amazing company. They make Remodulin in all its forms, as well as Adcirica, and these two drugs keep a lot of PH patients alive, myself and Joshua included. The company was founded by a woman named Martine Rothblatt. Her daughter has PH and in the early 1990s there were very limited treatments for the disease, and certainly not enough of them. United Therapeutics exists to meet the needs of rare lung diseases, and the research and medications they've come up with since have improved and saved so many lives. I love the history of the company - a mother changing the world for her daughter's condition, and helping so many others along the way.

So, we had this tour scheduled and we really didn't know what to expect.... maybe a walk through some cubicles and a meet and greet with a few staff or something? Oh no. Not at all.

What can I say... they rolled out the red carpet for us. We were met by Clare Miller, the Sales Training and Development Manager, and she introduced us to Patrick. Patrick is a Senior Vice President of Manufacturing with United Therapeutics. 

We got a behind the scenes tour on how Remodulin is made. From this white powder to IV, Sub-Q, or inhaled form (with pill forms and an implantable pump in the works!), this one little medication changed my life. "Little" is a ridiculous understatement, actually. The production, and the impact, is hardly "little". Revolutionary is more like it.

The production process is extremely complex, and the sterile environment it has to be produced in was amazing to see. We couldn't actually go in to any of the production rooms of course, but many had windows where we could peek in behind the scenes.

When you see what goes into this massive production for a drug that really serves a relatively small population, you begin to glimpse why it is so expensive to get and so highly regulated.

From the production tour we headed over to the new buildings - crazy cool place! They take such good care of their staff (light therapy rooms, Japanese tea room, hammocks, and all!). We sat down with Larry Sommerville, Senior VP of Sales and Marketing, and were also pleasantly surprised to be joined by Trish Dixon, Associate Director of Marketing. At this point I needed a Tyvaso break and as I took the nebulizer treatment, Trish walked by and saw it... she couldn't resist popping in and we're so glad she did!

Larry had prepared a series of slides for us, telling us all about United Therapeutics and the companies they hold. Each with a goal of a better today and tomorrow for patients with rare lung diseases. And then they peppered us with questions. What was it like to be a patient? What were our experiences? What did we want them to know? What did we wish could come next or be done differently? I think it's okay to speak for Joshua here and say we both felt immensely gratified at the personal interest they took in us, and in the way they listened.

Larry, Me, Trish, Joshua

From there it was lunch at 3pm (Finally! I was really missing that trail mix!) and back to PHA for phase two of our adventure... details to come soon.

Wednesday, February 29, 2012

Rare Disease Day

Well, today is Rare Disease Day.  I actually hate math - but I love statistics. So here are some numbers, just for fun. And how I feel about them.

According to the Rare Disease Day website, a rare disease in the USA is one that effects less than 200,000 people in the population at any given time. It's unclear how many people in the USA are effected by Pulmonary Hypertension, and it is believed to be vastly under-diagnosed... perhaps so much so that it isn't as rare as it now seems? Whatever the case, my particular form is somewhere in the 1 to 2 in a million.

The website further goes on to say, "The lack of scientific knowledge and quality information on the disease often results in a delay in diagnosis. Also the need for appropriate quality health care engenders inequalities and difficulties in access to treatment and care. This often results in heavy social and financial burdens on patients."

I'd say so! It can take someone with Pulmonary Hypertension anywhere from several months to several years to get a diagnosis. By that time, the disease may be seriously advanced. Why? Well, PH symptoms like breathlessness and leg swelling mimic so many other conditions. Too many patients are told they have asthma or need to loose some weight. In fact, it wouldn't surprise me if most patients were told one of those two things at first.

I obviously also spend a whole lot of time with doctors and in hospitals. If I charged someone for every time I heard either, "You're too young to be here!" or "Oh...PH... so, what, do you like take an inhaler or steroids or something for that?", or if I collected a fee for every time someone doing a medical procedure wanted to hang a bag of fluids (and risk putting me in immediate heart faliure)...well,  I could pay my medications outright.

The misunderstanding about the disease by the general medical community is frightening.  I hate to do it, but I go into every medical situation with guns drawn. I watch every move. Ask every question. Corner every medical professional who says they know all about PH, and then asks the questions about my treatments that let me know they have no clue, and give them an ear full (okay, that part is kind of fun). It's exhausting though.

Rino Aldrighett, President of the PH Association, often quotes some stats that knock my socks off. He says,  "Of the approximately 7,000 identified rare diseases in our nation, there are about 400 approved treatments for about 200 of those diseases. In 1996, the first treatment for PH was approved by the FDA, in 2001, the second and third.  Today we have nine…with more in clinical trials.  So, this small disease, with 20,000 – 30,000 diagnosed patients in the U.S. is exceeded in treatments by only two of the 7,000 rare diseases…and all of those treatments have come in the past decade and a half."

Nine treatments. More to come. This is fantastic. But my medications cost over $200,000 a year.  I am not a cheap date! And they don't cure - they just slow down the progress.

I'd personally say we don't know how long people with PH survive. The stats say 50% at five years. Those are old though, before several of the medications we now have on the market. I'd like to think it's a little longer. But for many, some I have been close to, it has not been longer.

Hanging on to all these numbers gives me a sense of control over this whole damn roller coaster ride. What numbers can't do is calm me when I look at my son and wonder if I'll see him graduate. Or make my heart stop pounding out of my chest if I get too cocky going up the stairs with an arm full of whatever. Or make me any happier about taking medications four times a day. Or stop the tears as we say goodbye to yet another who lost their battle too soon... always wondering, is that going to be me?

Numbers can't ever make me like wearing oxygen at night. Oh, I hate that stupid tube. It gets tangled in my hair. It leaves deep grooves in my face that take hours to fade the next morning. It chokes me if I roll over too many times. If it falls out during the night I spend the next day feeling like I've been hit by a truck. Oxygen deprivation can do that to you.

 In truth, it is the most outward sign of what I have to deal with, and I really hate that too. When they first delivered that big tank (wow, four years ago!), the guy asked me to try on the tubing. I told him flat out no. I know it surprised him, but I just didn't want to. Okay, I did anyway, but I was still saying no in my head. I still instinctively tear it off if I hear my son coming in. He's seen it enough times. But there's something about it I just don't want him to have to face the truth of. Nobody but my husband has really seen it on.

Until now. I took some self portraits recently, ones I didn't intend to share. I felt like if I could face the images maybe I could start accepting this part. Or something like that. I don't know exactly. I just know I was trying to process it some more. I know this stupid tube is my big hang-up. I know it's all in my head. And I know it's good for me to wear it.

I also feel like people look at me during the day and think I'm doing so well, the whole PH thing can't be that bad, right? Wrong. It's an invisible illness. I could wake up tomorrow and start a downhill slide very quickly. It happens all the time and I've watched it. So, here, to me, this is the reality of my PH.

I will fight every day. I will stay positive almost every day. I will defy every odd and do everything they told me wasn't possible with PH. I will not stop until PH is no longer a rare disease - it is a disease of the past. And I will not stop until I can get this stupid tube out of my nose.