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): http://www.firstgiving.com/phassociation/zumbathonforph

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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.

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.

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.

FDA PRESENTATION 
(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.

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.

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. :)

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)

Conference

The 10^th 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.

Highlights…

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