A Tribute

I know this blog is supposed to be about PH. But this post isn't. Well... in a way, it is.

This post is about my grandmother. Helen Marie O'Connor. Who is nearing her 93rd birthday, and who routinely scares the crap out of us, falling ill, making us think this might be "it", and then bouncing back, spunky as ever. She may have done this over a dozen times to date.

My grandmother has lived "with us", well near us anyway, for over 12 years. My grandfather passed away when I was 17, and my parents moved Grandma from her home in New York to be near us, around the time of my wedding, when I was just 21. So, really, much of my life she has just been here.

Here's a secret. I never told her about my illness. I barred my family from doing so as well. I was so afraid I would break her heart. She loves us grand-kids, loves her great grandsons (my son and nephew), fiercely. "So what's new?", she'll always ask. "You're my best pet", she often used to say. I just couldn't imagine responding to that with, "Grandma, I'm sick... and it isn't good." I couldn't stand the thought of giving her sadness when I could protect her. Now that so long has gone by and I am doing better, how would you start the conversation? I don't regret hiding it. Truly, I don't. But clearly, she is so strong. I suppose she could have withstood it.

I spent some time with her recently. In the nursing home where she had resided for nearly a decade. She has outlived not only 93 years of world events, but also my grandfather's cancer and emphysema and death, followed by her own breast cancer, and back surgery, and multiple strokes. Through it all, she somehow thrives.

Sometimes I can't help but think I get my sense of fight from her. I have her pale blue eyes, her stature (but never her tiny waist), her spunk, and her sharp tongue. Oh hell... we even had the same cup size at one point. Go ahead and giggle. I do. But that woman kicked breast cancer in the ass over a decade ago. Just like I intend to kick my own illness. So whatever she has, I'll take it.

This recent weekend visit was different. On this weekend I saw what my parents meant by "Grandma isn't doing so well." with my own eyes. On this weekend, instead of her greeting us up and in her chair, wig in place, eyebrows carefully dawn in, she was in bed, disoriented. I held her frail body upright. My relatively strong arms wrapped around her, holding her up so she could take a sip of much needed water. I changed her shirt... after that sip of water, or a leaky cup, or a faulty straw, or  her inability to drink, whatever it was, soaked her shirt to the skin, necessitating a clean one. She didn't have the strength to do it herself.

As I struggled to change her, to lift her meticulously kept clothes over a body bent and broken, as I caught sight of her body, marred by cancer and age, with the prosthetic boob shoved in her bra... the one she takes pride in placing, even as she is prone to joke about it and make my dad blush... I was just... struck.

Struck by the fragility of life. Struck by the fact that I, her granddaughter, could hold this old precious woman in my arms and offer her comfort... even as she cursed out the nurse who is "a dope" (a fact on which I concur), or her roommate who is "off her rack" (also true). While I giggled at her sharp tongue, and admonished her to be nice, I couldn't help but wish that her spark would always be with me as well.

I see so much of myself in her. Beyond our physical similarities, and beyond our shared middle name. I see her spunk, her will to live, her wish to maintain her dignity though some nice clothes and a little make-up, no matter how crappy she feels. I don't know what her final moments will look like. I hope I am there to hold her hand through it, or just before it...whatever it is I can do with the physical distance that separates us now that I live in a different state. And I wish the same for me. Dignity in the face of illness. Strength in the face of whatever comes. Surrounded by those I love. Enough spunk to put the people around me in their place when they are just plain "dopey".

Truly, she is something to aspire to.

Update: On May 4th, surrounded by family, my dad holding her hand, we said goodbye. Miss you everyday, Grandma. We love you.

Win-win!

I travel a lot. Like, a lot-a lot.

Sometimes, it gets pretty tiring. But then the following happens, and I remember why I keep leaving home for days at a time, juggling childcare, forgoing sleep, and being very grateful for one supportive husband!

These past few weeks the PH community has gotten a double dose of excellent news.

First, the PHA Specialty Pharmacy Advisory Board is about to launch and if you missed it, you can read about that HERE.

As an extension of that, I was just at PHA headquarters in Silver Spring, MD a few weeks ago to meet with one of the specialty pharmacies about their protocols. Long story short, one big giant specialty pharmacy merged with another big giant specialty pharmacy (or to be more specific, their respective parent companies merged) and the result has been... well, one big giant mess for several patients. After a particularly messy January, we called these folks in to PHA to talk to us about their merger plans, and to go over concerns, and to seek resolution.

I came away feeling pretty good. We got some answers. There were some answers we didn't get too - although I suppose that is to be expected. What I do know is that many of the individuals "on the ground" in these companies do have our best interest at heart. And changes are going to be made for the better. We need to hear about those good folks too, by the way. I really believe improvement is best made when you build on strengths.

What I got most from these events is a sense of feeling empowered. See, we are "just the patients" and it can feel like the big giant pharmacies and insurance companies have all the power. But that isn't entirely true. We are still in fact the customers. And since we are the kind of customers who can get all pesky critically ill when our meds are screwed with, we have some extra clout. So I feel empowered, because after a couple of years of speaking up on my own, and this past year speaking up with PHA in full force next to me - we have REALLY TRULY made a difference.  The specialty pharmacies are listening and vowing change for the better.  I believe they will. And we are going to make sure that keeps happening.

Secondly, this past fall I had traveled to Silver Spring, MD again to visit with the FDA during open commentary on a patient-focused drug development initiative. Long story short, we were amongst what turned out to be 90 disease states each advocating for the FDA to choose us as one of the just 20 they would study in detail for the next few years.

We got it.

Pulmonary Hypertension has been chosen. This is huge! Anything we can do to raise awareness is a good thing. Anything we can do on the national stage is even better!

You can read more details on Rino's blog HERE

These are awesome steps in the right direction as we work towards awareness and ultimately a cure. But the fight is far far from over. I fully expect to be on a plane again in the near future, or perhaps writing one of my rather strongly worded letters that apparently I am getting known for (blush)... Well, fine, whatever. If the fight is there, I still believe that those of us hoping for a cure have no right to hope unless we all take the front lines in our own way. It doesn't have to be policy changing or on the national stage. Far from it. But I will say this again and again - everyone has their story, everyone has their gifts, and that is what we all need to be using to move us forward. Right now I feel like I'm getting a happy ending to some of that effort - but there is still much to come.

Let's get this thing done.

Big News!

 Two pieces of news.

The first, personal. I got results back from a cardiac MRI today and my right ventricular function is normal. This means that the right side of my heart, the side effected by PH that used to be terribly compromised to a dangerous level is now functioning as expected for a healthy woman my age.

 Since diagnosis, it has never ever been normal.  I am a very happy camper. It means I have responded as well to the meds and my holistic methods as I feel I have. Sometimes I feel better than the medical evidence suggests I should - so this is nice to have them line up. My mom says it is also because I insist on good wine and great shoes. I don't think she's wrong. What it really means is I insist on doing the little things that make me happy... and I think that helps me heal too. Whatever the secret, it's working and I'll take it!

The second - for the community. Big big announcement out of PHA this week, and one I have been anxious to roll out since I have been working on it informally for a few years and much more formally for about a year. You can read about it HERE

The cool thing about fighting battles is that sometimes you win. Today feels like a win.

Happy Anniversary to Me

Here it is. My five year anniversary of being diagnosed with Pulmonary Hypertension.

It is a milestone, to be sure. When I was diagnosed, the expectation was that PH patients had a 50/50 chance of living five years. I have actually truly beaten the odds (Now, why can't I do the same with the lottery? But I digress...)

I feel like I ought to write something profound here. Some deep reflection on the meaning of life, or illness, or the battle at hand.

But I actually don't have anything to say on that at the moment. In fact, what struck me most today was how normal the day seemed.

And so, that is what I am celebrating. Normalcy.

I named this blog "PH and the New Normal" because I wanted to re-define what the PH experience was to be. I hope I am doing well at that. But now I find it comes full circle. By all counts, just like today, the rest of my life is pretty "normal". Oh sure, there's nothing normal about $250,000 a year in medication, or the number of times I visit doctors, or the procedures I am subjected to, or the number of friends I've said goodbye to as they passed away from the disease I share. Those things are decidedly not normal.

But what is normal is I wake up every day, I still get to hold my son, my husband still brings me flowers, my friends and family are amazing.

So never mind a "new normal". I am almost even overlooking today's five year milestone. Instead, I am just deeply content and grateful that, while life is nothing like I had planned, today just felt normal.

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.