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.