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.

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?

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.

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.