Once Upon a Time

What do you do when life hands you a diagnosis that turns your world upside down? 

You re-define everything.

 www.DefiningTheNewNormal.com

A few years ago, I became a certified holistic health coach. Not at all without coincidence, that was also around the time my health took a massive turn for the better. I started living the idea that taking care of yourself mind, body, and soul matters more than we could ever measure.

As I went through my schooling and began my business I was often asked, "Oh, do you help people like you?" Meaning, people who were sick.

I always said something of the following, "Well... no. You see, I live that life everyday, managing my medications and doctors and watching for and working around symptoms. It's exhausting. I spend hours a week volunteering for PHA and interacting with my community. I love that part. But I need a space away from that too. I need a job that's just for me. Away from 'sick' all the time."

People would sort of nod. Some would look at me sideways. But the conversation would end.

And there was always this nagging feeling in my head too. I mean, I was doing really really really well. And I had a pretty good idea why. I would share snippits of my healthy living philosophy with other patients as it seemed appropriate, but I pretty much just did my thing. The universe might have been requiring more from me, but I wasn't ready to hear it.

I don't know when exactly the idea of writing a book about my journey and all I had learned came to me. Perhaps it was the jokes, "You should write a book!" Perhaps it was the fact that people kept asking more and more questions about what I was doing and why. Perhaps it was just time.

So, this January I enrolled in a self-publishing class and began what would be the most interesting, excruciating, exhilarating, challenging, fun, maddening, and gratifying process. Yes, it was pretty much more than I could have imagined.

Irony is my shadow. The winter I was writing I spent 28
days intermittently bed-ridden from a variety of illnesses. I hadn't gotten that sick in years, but I sure did while writing about getting healthy!

I also kept up with tenacity at my lifestyle practices... when I could get my head off the pillow. And I had some of the best medical results of my life two weeks after the last health blow that had left me in bed for days.  Go figure. (Fingers crossed, I've been up and about a solid five months and counting now...)

The day the big box of books arrived (yesterday), I spent the evening curled up on my side begging the world to stop tilting and spinning. I had a rare hit of side-effects from my medications which left me very dizzy for a few hours. It passed, of course. And this morning as I sit here writing I'm right back where I expect to be. Irony. Always following me.

Anyway, the book... it is called "Defining The New Normal: A Guide to Becoming More Than Your Diagnosis"

It is so easy to let our disease define us. We claim it in how we speak about ourselves, "I am a PHer." or "She is a cancer patient." But, no... we are so much more than our diagnosis. I have PH, it doesn't have me. It is not who I am. Those of us who carry a diagnosis just have to fight a bit harder to create an identity of someone who can be quite well, even though they are sick (yes, it's possible!).

This book takes the reader step-by-step through so many areas of self-care that are crucial to our overall well-being. Nutrition, exercise, sleep, those big ones are in there. But there are some you might not expect too. Like the healing power of connecting to others, and of reaching out. The absolute crucial necessity of being the (humble) leader of your medical team. So much.

Every key chapter ends with a "Defining Moment". A moment when the reader takes a pause and goes through a serious of journal-like questions to apply on the most personal of levels how they can put into actions the lesson at hand.

When you're done with the book, well I hope you're a little healthier and happier for it. I hope you go back to it and read it again and again as you need to. Goodness knows, life is rather a case of "lather, rinse, repeat". I am constantly falling and getting up and brushing off... but at least I have the tools to do so, and I hope by sharing mine with you, you can too.

Here it is: http: DefiningTheNewNormal.com

No Ice Bucket Challenge for Me

It happened. I got "nominated" for the currently Internet-famous ALS Ice Bucket Challenge.

I figured I would since it is viral and all. And I wasn't sure how I would choose to respond right up until the moment it did happen. And when it did, my gut response was an immediate quite, "No, thank you."

In case you've been living under a rock, the ice bucket challenge goes like this: You stand in front of a camera, say you've been nominated for the "ALS Ice Bucket Challenge", nominate a handful of friends, then dump ice cold water over your head on camera. Skip the challenge, donate $100 to ALS instead... some good folks dump and donate.

It's more or less amusing. And the ALS Foundation has made an incredible amount of money in the last couple of weeks. I hope more people now know what ALS is, and took a few minutes to learn about the disease prior to the big ice dump. Because that was, after all, the point. And the point is a good one.

Soooo... why is it not for me? Well, purely selfishly, I despise the cold. My vaso-dialating medication makes me particularly susceptible to it. I also worry the shock of cold would make my heart jump, and that we can not have. When that happens it is uncomfortable and it scares me. It's the simple fact that I have my own rare disease to contend with. That's where my focus is.

Perhaps I have a bit of compassion fatigue. More sensitive than is probably good for me, the plights of others affects me deeply, whether I know them or not. If I reacted to every cause that touched my heart I'd be dead broke and exhausted. Perhaps I look at this whole ALS thing and I get it so deeply and personally, that I don't feel the individual need to get on the trend bandwagon.

The truth is, I don't know anyone with ALS. And not that that means those who suffer from it don't deserve our support. It's a hideous disease that takes lives. I get that. Oh, do I get that. But right now I'm watching a dear friend in the fight of her life against cancer. I watch her kid when she goes for treatment. I worry about her every day. I worry about people in my own disease community.  I worry about a lot every day.

I watched a video this morning of the man who started it all, Pete Frates. I made my son watch it as well. This is because yesterday I got a video from a friend of my own kiddo doing the challenge. When he got home I asked him if he knew what ALS was, and why would he do the challenge? He didn't have an answer to either question, and we had a long chat. Now he knows. And I'm proud my boy has a little more knowledge and compassion because of it.

Back to Pete's video... in it he goes to the FDA to plead for better treatments, faster movement on research. I've twice been in those same halls, even that same room I think, having the same conversation with the FDA. I wonder if he got anywhere with them? I wonder if I did? I wonder what the sum of each of our actions and plea for a better tomorrow come to. Right now Pete's result is pretty damn impressive. I'm happy for him. I hope the same for all of us fighting a rare disease.

So many of you have dumped icy water over your head and made a generous and heart-felt donation to a cause that has caught your attention. For that, I thank you. To be noticed, to be supported, to be cared for... that's half the battle while we wait for the cures we so need.

Always moving forward, I wonder what the coming months will look like. I imagine non-profits all over the world are wracking their brains for a way to replicate the phenomenon. The ALS Foundation has gotten a pretty impressive sum of money... and I have to tell you... it isn't enough. Research is so expensive. A good research program will blow through that new sum in a heartbeat.

If you truly have awareness, if a cause is truly something that has caught your attention, take it past the viral video stage. Take it to monthly or yearly donations. Take time to research what it is you just supported. Take time to actually get to know someone who suffers from the illness and understand their days, their wishes, their trials... not just the name of their cause.

And watch Pete's video here. Because even though I won't be dumping ice cold water over my head, I wish him and his sweet growing family nothing but a miracle and the very best.

The Long Road to Her

This blog is probably one of the most raw things I have written to date. It contains thoughts and experiences I had previously chosen to keep private (in fact much of it was written a year ago and never published) - odd for someone like me who has otherwise made a decision to live this diagnosis out loud in an attempt to change its course. But now is the time to share. My prayer for you if you are living some of these same struggles is that you find some hope in our story.
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My journey to her began at age 19. It was the year I took the job as a nanny for a little girl newly adopted from China. I clearly remember rocking her to sleep in my arms, gazing down at that gorgeous perfect little face, and having the wind sucked out of me. What if her parents had not cared enough to go and get her? What if she had stayed in a Chinese orphanage? What hell of a life would she have lived? And here she was, safe in their home, safe in my arms, the world at her feet. I vowed then that I too would make the difference in a little life, and adopt.

I knew the child I got would be a girl... Altruistically speaking, girls have a pretty tough time in much of the world and I wanted to make a difference for one. Totally selfishly, I wanted to raise a ballet dancer, like me, someone to share that with (for as long as she would tolerate it before asserting independence, of course!)

Eight years ago I had another brush with adoption that only further solidified these feelings. I was teaching special education and I had twin girls from India on my case load. They were the result of a botched late-term abortion and had survived, eventually being adopted by an American couple. Again, I found myself looking at them and thinking, "What if..."

Fast forward many years, and I have an incredible biological son. A son who is my own miracle - as we now know I shouldn't have survived the pregnancy because I probably had PH already, and we just didn't know it.

When you have PH you can't get pregnant. Or, at least, you really really shouldn't. This is something you're told pretty much upon diagnosis. When I was told, it didn't faze me. My son was an infant at the time, and pregnancy was not something I was too big of a fan of the first time around. Since I already knew I wanted to adopt, I figured that would be our route, and that was totally cool.

Except, for a long time it wasn't our route at all. I was so busy chasing a toddler and trying to get better and trying to be self-employed, and my husband always works so hard, and our lives were just full. Although we batted the idea of adoption around many times, the answer always seemed to be "Not now." And that was fine.

But then a little over a year ago my damn biological clock started ticking...and chiming... and the gong started going off... and I couldn't make it stop. I wanted that baby.

After long heart-to-hearts with my husband, it was clear he just wasn't ready for adoption. And I couldn't blame him. It's not like other times when we've disagreed and my nose is all out of joint because he doesn't see it my way (come on, you know you all get like that). This time, he had real fears about the future. How the financial burden of supporting our family is by and large on him.  And worse, he had fears about being a single parent some day, if PH took the ultimate toll. These aren't fears we face head on too often. In fact, we never had before. We've chosen to live with the positive and not think that way. But when we're choosing to discuss a very purposeful choice for the long-term future, and involve the life of a child, we kind of have to wrestle with that dark side too.

For the sake of my marriage, I vowed to force this clock away. MAKE it go away. Focus on my son - whom I love and adore and is so so so enough in all possible ways. Basically, I tried to beat my biology. And pretend I was winning. And I did okay... for a while.

Then in the spring of 2013 I attended a dance recital. And I sat there and looked at the moms around me smiling with so much pride for their daughters on stage and I just lost it. I sat in the dark auditorium and cried silently. That should have been me. PH robbed that from me.

The grief followed me in the months ahead, although I tried to keep it in. Baby showers were torture. My sister got pregnant and I cried my way through shopping for the niece whose arrival I was over the moon excited for. And finally it was clear that I was going to have to either get some serious professional help in letting this all go, or re-visit the decision.

I tentatively broached the topic with my husband. And... he said yes. Why the timing is right now, I just don't know (neither does he). But my health is stable (thriving, even), all of my doctors are 100% in support of this decision, my business is growing, things have changed... and now we're ready as a couple and as a family.

Then it was just down to deciding on how to get her. International adoption held little draw now. I wasn't that interested in having to travel overseas with PH to go and collect her. Domestic adoption sure sounded good, but the tens of thousands of dollars it costs seriously deterred us. Surrogacy was also on the table... but I ended up deciding that if we really were going to do this, I needed to stay true to my original intent all those years ago and help a child in need.

And so, we were left with the foster system. We have chosen to adopt through social services and take a child whose parents can not care for her. It does not escape me that for us to get our daughter, someone else will loose theirs.

This path is scary. In fact, I should probably be much more jittery about it than I am. The child may come to us drug exposed, abused, neglected... who knows. We could be placed with a child, only to have the arrangement fall through because the system deems reunification with the birth family or extended family is better. But the truth is, the road to her has been so very long, sometimes so very hard, and now is the time.

It is going to be fine.

I have total peace about it. A peace that surpasses understanding, and I'm pretty content with that.

We're almost done with all of our DCF licensure requirements.

Now, all we have to do is wait for her.

What a Weekend Can Mean

Well over 1,500 lives were touched this weekend in an event that, as far as I know, has no parallel. Certainly in my admittedly biased opinion, the Pulmonary Hypertension Association's International Conference is an event unlike any other.

Our Conference is unique for so many reasons, not the least of which is that it brings together patients, caregivers, industry, and health care professionals under one roof for an event that teaches, tugs at the heart, and (most importantly) inspires. And it all gets pulled off by about 40 incredible staff and one very busy hotel.

Most of the Generation Hope Advisory Board

My own highlights were many. My favorite parts are split equally between spending time with other patients, many with whom I am in frequent contact with on the Internet but rarely see or have never met, and getting a chance to hear from the premier thought leaders in our field and soak up every bit of knowledge I can from them.

Now that I've been involved with PHA for quite a few years, and had a hand in building a few resources that bring patients together, there is

Three Colleens

nothing that makes me quite so happy as to sit back and just watch the joy that comes from the connections that get made. You have to see it to truly feel it, but when you watch people embrace over a shared cause, a shared heartache, a shared sense of inspiration... well, I am rarely at a loss for words, but for that feeling, I am.

Dr. Greg Elliot

And then there are the doctors and nurses. I don't think I'll ever quite get over my awe, and certainly

will never let go of the feeling of thankfulness for what these professionals have accomplished thus far, and work so hard to still achieve. Because of course we've made amazing strides in the PH field these last few decades, but goodness knows there is still far to go.

I made it a point to track down a few of them this time around and pick their brains. The fact that a busy doctor would pause in their day (or late evening!) and spend 20 minutes with me in focused conversation, sharing their thoughts and ideas, their knowledge, and their history, means the world to me.

It inspires me too. I've got a fundraiser in mind for a targeted PH cause that is getting to be pretty important to me... And it is because key players took the time to educate me on why it is so important to them.  When inspiration strikes, it's best to get moving on making something happen.

Actually, my friend Diane Ramirez said it best in her Sunday morning keynote, "You can do something big or you can do something small. But it doesn't matter as long as you reach at least one person."

Well... she is absolutely right. And if you came away from this experience inspired like I did, let's get you moving. Even if you're not quite sure what you want to do, contact PHA (or myself), tell us you want to get more involved, and I promise you we will find you a job.

Right from the beginning of this weekend, I had this deep sense of peace I was having a hard time identifying the source for. Usually there is more of a sense of urgency, or a fair dose of stress around a to-do list, that I have around Conference (this is not necessarily a bad thing). This time, I was just so... zen. I'm still not sure, but I think it has to do with how excited I am about the incredible progress and new initiatives I know PHA is spearheading. I also know in my heart that I am literally doing everything I possibly can to move us towards a cure, and a whole lot of other people are doing that as well. And the results of this collective effort are getting to be pretty staggering. 

In some ways I could go on and on about what Conference means to me.  But I think I'll let other voices tell you instead.

As other patients told me this weekend: To me, Conference is...

"The greatest." (Rick P.)

"A life changing event that allows you to feel connected and alive through interaction with medical professionals and patients." (Michelle F.)

"Awesome-sauce." (Kayla N.)

"An experience above any other..." (Deziree R.)

"Simply amazing!" (Summer G.)

"AMAZING!" (Shelly C.)

"Encouraging" (Barbara C.)

"A lifetime amazing experience to meet new people, reunite with across the world friends, and an opportunity to celebrate that you are alive." (Anaelis P.)

"Busy... interesting... educational meeting lots of people." (Lisa H.)

"PHantastically PHun!" (Susie A.)

"Awesome and very informational" (Karen D-G.)

"Awesome and busy too." (Debra R.)

"An amazing collaboration of PHA staff, Board Members, patients, Medical Professionals, and sponsors that have created a once in a lifetime experience for every attendee." (Tiffany G.)

"A must for every PH patient and caregiver." (Deborah W.)

"Inspiring!" (Caroline R.)

"Encouraging!" (Janet M.)

"Phantastic!" (Pat Z. and Jennifer K.)

"Phabulous!" (Jennifer K.)

"A life changing experience, a moment in time never to repeat itself, a beacon of hope for the Southern most tip of Africa - Creating Awareness of PH in South Africa!!!" (Denneys S.)

"Awesome and amazing!!" (Elaine W.)

"Awesome but too short!" (Ornah L.)

"The most comfortable place in my world." (Jenn L.)

"A way to help others and a way to get a little help for myself." (Debbie C.)

"A must." (Michelle H.)

"Conference is amazingly exciting and totally exhausting but I was like a kid who was scared to nap because I might miss something." (Karen L.)

"Empowering and hope instilling!" (Vikki A.)

"An event that must be experience to be fully understood. There are no adequate words to describe the magic that happens when patients get together to compare notes, learn, and celebrate and support each other." (Mary S.)

"Where everyone understands me totally!! We meet as strangers but know in many ways what the other is feeling." (Grace W.)

"Educational and busy!" (Joellen B.)

"A conference of hope!" (Lindsey B.)

A Chat with the FDA

FDA 2012 - Round 1

Back in 2012 I visited the FDA in a bid to have PH chosen as one of several rare diseases that would be studied over the next five years under the PDUFA V mandate. This mandate basically directed the FDA to study at least 20 rare diseases in-depth, looking specifically at patient reported outcomes and view points during the clinical trial process.

While my testimony that day played a part in our selection, as usual, it was a team effort from PHA that got the job done. Amongst the 7,000 known rare diseases, we got "short listed" to 40, and then finalized as part of the 20 to be focused on. This past week, we got our first chance to talk directly to the FDA about what it is like to be a PH patient and live under the medications they approve... or don't end up approving.

Lunch at PHA

 This was an historic opportunity, to say the least. And quite a bit of fun. Many people gathered at PHA prior to the meeting to catch up and have lunch. Then we loaded on to the bus to be brought over to the FDA Campus in Silver Spring, MD.

Prior to the meeting, the FDA put out requests for people who wanted to be panelists and speak
directly at the event. I threw my hat in the ring and was honored to be one of 10 chosen. So I had four (!) minutes to present my comments. I have to say, every single panelist was just outstanding. They were honest, they were raw (some of it was hard to hear), they did an amazing job sharing what living what this disease is like and how desperately we need better medications and a cure.

Along with the panelists, they frequently opened up the room for commentary, and even integrated comments online. Overall, they did a really nice job integrating as many people's viewpoints as possible.

I left feeling we were well heard, but that perhaps this was just the first of what are hopefully many conversations. Because we had to focus on such a select set of questions, the really big picture of living with PH wasn't fully covered. I hope they want to hear more from us in the coming months (and I believe they do).

FDA - ROUND 2!

Above all, I feel like this day served one really key (although perhaps unintended) purpose. As people gave live updates of the day online, and discussed it after, the excitement was palatable. What an incredible boost for our community. You could feel the sense of morale, ownership, and excitement build as people interacted around the experience.

I am very proud of my community. The state of this disease isn't really going to change because of the FDA, or the NIH, or just about anything else. It will really change because although we are small, we sure are loud and focused... in very good ways. As long as we keep our message clear, and continue to be honest about our experiences with each other and with those who can make a difference, it is us, the patients, and PHA, who will lead the charge to our better tomorrow.

The Whole Crew

My commentary to the FDA appears below. If you'd like to add your own, it isn't too late! Open public comment is available until July here: http://www.regulations.gov/#!documentDetail;D=FDA-2012-N-0967-0592


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Hello. Before I begin, I would like to take a moment to thank the FDA for having us here today. As you well know, in a rare disease state like pulmonary hypertension, it can be a challenge to feel heard. We really appreciate you taking the time to listen.

I was diagnosed on January 2, 2008. What I thought was shortness of breath due to being deconditioned from having a baby was in fact pulmonary hypertension. From the night I went to the ER for an unrelated illness to the point of diagnosis was actually just two weeks. I was very lucky.

I currently take three medications: Adcirica, Letaris, and Tyvaso.  I am also on nocturnal oxygen. For the first couple years after diagnosis I needed digoxin, but do not any longer.

To begin,  I wanted to try one treatment at a time so that I could gauge which might be helping me and how I might be impacted by side effects. We started in the beginning with Revatio, which later was switched to Adcirica for convenience. We added Letaris about eight months after the Revatio. I felt okay on these two medications, but there were definitely moments my PH stopped me short, particularly when it came to keeping up with my son.

I caught Swine Flu when it came through in 2009 and I was unable to recover fully. It was at that point that we added Tyvaso, which has worked quite well, and it was soon after I started it that my activity levels started to come back up.

In addition to all these treatments, I have practiced integrative medicine almost from the beginning, believing that the PH medications alone are not enough. I could see how they helped a great deal, and I could also see how they would never work as well as I wanted them to if I used them as a stand-alone approach.

I take supplements which are carefully selected with the help of my naturopath and the go ahead from my PH specialist, I exercise several times a week, and I try to have a spiritual practice that keeps me in balance.  I also overhauled my diet a few years after diagnosis and am now a vegetarian. After studying nutrition for some time, I felt that a vegetarian diet was the most heart-healthy and conscientious decision I could make. I do think I fare better eating and living this way because fighting disease is a mind/body/soul effort.

My current treatments (in conjunction with lifestyle practice) control the shortness of breath and heart function quite well. Nothing has been able to touch the fatigue that comes and goes though.

The medications have definitely improved quality of life. However, they also kind of run my life because every four hours I am taking something.

My quality of life is also impacted by the specialty pharmacy system. I have personally experienced quite a few barriers to getting my medications in a timely fashion, primarily due to issues with the order and delivery process, and I know many other patients have experienced the same, some even being forced to go without treatments, or getting dangerously low on a vital medication because the system does not always work as smoothly as it should.

I have taken an active roll in addressing these issues with the help of the Pulmonary Hypertension Association. In fact, we now have an entire system in place where many of our key players in the pharmacy industry are taking a direct roll in gathering and analyzing patient feedback and working with PHA to build on best practices, as well as improve in areas where there is need.

The downsides to the treatments themselves include a nagging cough from the nebulizer and the acute nasal dilatation, which makes it a little uncomfortable to breath on a good day, and nearly impossible if I have even a simple cold. The equipment can be somewhat cumbersome.

One key area of impact for me is actually psychologically related. I understand that it is an FDA mandate that I must use two forms of birth control, get monthly pregnancy testing AND monthly pregnancy counseling because of one my medications.  I can’t stress enough the emotional toll this takes on so many women just like me. We do not want to have to re-live every month the way this disease has taken one of life’s most precious gifts. And yet we are forced to… as if we could ever forget for a minute what this disease has done not only to our lungs and heart, but to our lives and families as well.

Assuming there is no complete cure, my dream is to have treatments where the side effects don’t impact us just as badly as the disease.

I want equipment that doesn’t get us treated like potential criminals at the airport just because we carry high-caliber medical devices… or better yet, no need to carry equipment at all!

If there were more ways to titrate up and down on oral medications more carefully, that would be wonderful.

Finally, I want FDA approved medications for children with pulmonary hypertension.  This is a pretty tough disease to handle as an adult, but we do have lots of options. I would love to see so much more research and opportunity for the kids. Right now we know that the outcome of PH for children is likely worse than it is for adults, and that any treatments that might be selected are prescribed off label. Additional research and trials that will get our kids appropriate treatment options would be priceless.

Again, thank you for taking the time to hear us today, and to weigh our thoughts and ideas moving forward. I feel confident speaking for the PH community and saying we very much appreciate it.

When PH is the Least of Your Worries

I wrote this several weeks back. And then I didn't publish it. Why? Well... I was wrestling with feelings of faliure and vulnerability. I'm a health coach - I help people get and stay healthy. And I have been sick as a dog for months. If I published this, would people think I'm not good at my job? If I'm so good at this, why can't I get better and stay better? (Even my doctors are stumped, by the way). Nothing I've been dealing with is PH related. Maybe auto-immune though... we're just not sure.

And while these feelings of inadequacy were authentic, I'm also being too hard on myself. What I really preach is facing whatever you're handed head on and powering through it however you can. Always thinking outside the box. Always believing your body is capable of more than it might feel it can be. Always believing that if you look and work hard enough, you can re-gain control on some level.

So, anyway, I'm publishing this NOW. And I'm owning the fact that I am going through something that is beyond my skill set, and even the skill sets of my doctors so far. And I'm claiming the fact that I'll get better again anyway. Yeah... I can do this. Soon would be nice.

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So, let's make a deal. When you get PH (or any other rare, life-threatening, incurable, blah-blah-blah illness), you get a FREE pass. That's it! No more medical drama. You only have to deal with that one thing. Sound good?

I think so too, but alas, it is not to be.

This last... er, several months, have been a doozy. Let's highlight since November:

Auto-immune flare (I assume), followed by a series of joint issues.

Two viral infections and a bacterial infection - all at once. Recover, get a good two weeks reprieve.

A cold. Recover.

Flu-like illness: high fever, pounding head, clogged sinuses, the works. Recover, get about four good days, and then re-lapse which turns into...

A peritonsillar abscess: Walk-in clinic visit for extreme throat pain where I am then called "Boo-boo" and "Baby" by a highly inappropriate doctor. How do I attract these people?

Subsequent ER visit where, among actual real screw-ups,  I also discover the hard way I am allergic to morphine, followed by an attempt at oral antibiotics which again I discover the hard way I am allergic to, followed by new antibiotics which work but "require extra monitoring with pulmonary hypertension" - yes that was actually in the medication literature. I assume my corresponding bouts of dizziness can be attributed here.

My dentist also informs me this particular abscess is what killed George Washington. While it turns out she may be using old info, this is still illustrative of how fun such an event is.


Again, I get about four good days. And then the throat pain returns. I tried really hard to handle it, and succeeded really well in fighting it back for a few days. But, a night of violent puking thanks to something that disagreed with me put me over the edge.

Back to a new walk-in clinic. Ever see a doctor who is hearing a PH heartbeat for the first time? I have! But at first I thought he was just freaking out because he suspected an infection in my heart, courtesy of the above string of illness, which is what he said he thought it was, and that freaked me out too. And I got another fast-tracked trip to the ER.

I am fine. Just fine. (Well, except for the new case of strep throat - the only thing that came from ER visit #2). The wonky heart sounds are mine, normal for me, courtesy of PH. And I am so freaking sick of being sick. I can't figure out what's wrong, and neither can my doctors. And since I've seen no less than ten doctors and PAs of various specialties and modalities in the last month in an attempt to get well, it's safe to say this is a mystery.

I am normally really really good at staying healthy. In fact, I haven't had more than a cold since 2009. That's a darn good run, especially for PH! And I'm good at helping other people to stay healthy, and even regain health. Why I can't solve this eludes me - I'm using all my tricks and then some.

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So, I've battled several more colds since I first wrote this, and on good days I work (on bad days I've worked a lot too), I've traveled quite a bit, I'm still a mom who had to get food on the table and attend to the kiddo. And I'm still desperately working on what I'm usually good at - being healthy in spite of PH. I'm even exercising at full tilt again, after a necessary several months off. Sometimes I think I'm a weeble wobble... down, up, down, up...

Oh, and I've discovered I am a difficult patient. But I promise I don't mean to be. Maybe I get a bit snarky when ill. And maybe it is because every time I have to deal with medical professionals when I am in trouble, I have the following conversations. Here, for your enjoyment.

Question: "On a scale of 1 - 10 how bad is your pain?"
Answer: "I gave birth and it took 37 hours. My perception is skewed. Please define pain." (What I'm really saying is I have a ridiculously good pain tolerance and I am not a good judge when I am in crisis because I put up with too much).

Statement: "Oh no! You can't possibly have (insert rare illness complication here), that's really really rare,"
Answer: "Yup. And I got PH at a one in a million chance. Rare happens to me. Let's check anyway." (What I am really saying is check anyway. Because it is obvious statistics and me are not a good match.)

Statement: "Well, we're just going to hang a bag of fluids to that IV now."
Answer: "Nope. No way. It might kill me. No... Really... No. Can I have some water, please?" (What I am really saying is I know you are a fantastically wonderful and caring and skilled medical professional and you STILL don't see many PH patients and you STILL might kill me if you hang that thing to an IV. Don't.)

Seriously, this had better be it for a while.

Facing a Fear

Did you know that the fear of public speaking is often cited as the second most common fear amongst humans?

Yeah, #2 (Flying is #1). That means people are more afraid of public speaking than snakes, spiders, (or octopus if you're me), and even death.

Well, I understand that fear! Despite both a theater and teaching background, for the longest time the idea of standing up in front of a crowd and delivering a speech, which somehow has to be so much more refined and ideally powerful than teaching a lesson to six-year-olds, or reciting lines and... ahhhhhhh!!

Seriously, I have been known to shake in my stilettos before, during, and after (okay, stilettos are a poor choice for such an event). I have been actually able to feel my heart trying to escape my chest, and it isn't the PH at play.

None of that mattered though when I was approached prior to the PHA International Conference of 2012 and asked to be the keynote for the Founders' Dinner. Oh sure, all the fear flashed through my head, but how does one turn something like that down?

So, I said yes. And continued to freak out. And wrote and re-wrote and re-wrote a speech. Then my best friend edited it, and my mom gave me a brilliant idea on how to practice, and another friend snapped me back to reality and out of a potential melt-down a few days before, and another friend insisted in putting a glass of wine in my hand just prior to delivery, and... well, I didn't keel to the floor after all.

And now, public speaking doesn't bother me so much. In fact, I kind of dig it.

So, this speech is dedicated to:

Rachel - my editor, ass-kicker as needed, and best friend since we were 18.
Mom - who knew exactly the trick I would end up needing to practice this enough not to shake.
Joshua - for, as usual, making the frank matter-of-fact observation that made me believe I could do this.
PHA - for, also as usual, trusting me with a task I didn't know how I was going to pull off, but did anyway.
And you, the PH Community - who gave me the strength from the beginning, and make me want to fight harder every day.

Thank you.

What Drives Us: Making Sense of the Senseless

In 2010 I was attending my first PHA International Conference. I had been asked to sit on stage at a breakfast event, one speaker on a panel of "Next Generation of PHA Leaders", a request that left me rather surprised. At that time, I had no idea about the crazy ride I was embarking on with PHA, or the rolls I would find myself in, still to this day looking behind me thinking, "Don't you want someone else for this? It's just me here..."

Anyway, I was there because of my activity with Generation Hope. The young adult group I helped co-found. Others were there for their fundraising, advocacy, and awareness work. Near me sat a beautiful young lady named Lauren Johnson, just 17 years old.  She was a fundraiser and worked to get her story in the media. She was articulate. She was effective in her efforts. She was full of life.

She died yesterday.

Still in college. Unexpectedly. The best I can gather, after a very rapid decline nobody could see coming. I hadn't spoken to her since that day in 2010, but that doesn't really matter. Because when this happens, it is always personal. Always.

I saw the news pop up on my Facebook newsfeed. The way I most often get word of a passing (totally bizarre). I just stared at her face. Numb.

Yes, this time it is just numb. I feel like I should feel something. And I can't, really. Because if I feel anything through the ones like this - the ones where PH just suddenly strikes and takes someone who seems fine, it opens up a shit storm I don't know how to face. It starts with the knowledge this will happen again and again, and it ends knowing that it could just as easily been me.

So many times, people say to me, "You do so much!"  

My answer: She died from the disease we share. Every month it seems, I watch this happen. So, it isn't enough. Ever.

All I can say is that until I no longer have to feel (or try not to feel) the impact of person after person passing away from my disease, then my fight will never be enough.

Together though... if everyone kicks in more than they are able. Maybe these stories will no longer haunt my days.

In Lauren's own words, "It is hard, because we don't have... something everybody knows, like breast cancer, or diabetes, or tons of different diseases...we have something that nobody really knows, and we're the only ones that can fight for it... We're our own advocates."



This is the link to our video:  https://www.youtube.com/watch?v=9cLrXGxsQ2c

Our section starts about 11 minutes in.

Reflections on a Six-year Anniversary of a Disease That Gave Me a 50/50 Chance of Living Five Years

The title of this blog is complicated. But, then again, so is PH, right?

Each anniversary of my diagnosis has brought about a new wave of complex emotions. While the years are now somewhat blurred together, I remember the first year just being awe struck that a year since my earth-shaking news had already gone by. Subsequent years brought more and more hope, but my deep uncertainty for my well-being and even my life remained.

Conversely, year five was striking in just how un-striking it felt. Even though my chance of survival was said to be only 50/50 at five years and I had reached that point, I just felt how normal the year was (as in, very little medical drama) and was truly grateful for that sense of peace.

And now, here's year six. And, while a good one, this really has been a hell of a year! Truth be told, I still have a lot of sadness and uncertainty floating around. Right now it is centered on those I care for with the disease still struggling. I hate this disease and I hate the pain and anguish it causes. Even as I feel pretty good about my own future, too many people are not out of the woods. It can be paralyzing to be a part of. The pain of watching someone you care for suffer (and sometimes die... or almost die) from the disease you share is beyond words. You hurt for them, and at the same time, even though you try to fight it, you hurt for what your own future might be. This all sucks.

However, as PH is always a roller coaster, I am also struck by just what a crazy year of new milestones and celebrations this has been too. And, as always, while I give an honest nod to the hard parts, cry when I need to, and lower my head and ride through the storms when I have to, it is the celebrations I really want to stay focused on. So here goes...

I have just completed my third year on the PHA Board of Trustees and am looking forward to my next three-year term. And Generation Hope, the young adult group I helped start with PHA and some other patients, is turning four years old this month!

In 2012 I had traveled to the DC area to testify in front of the FDA in a bid to get them to choose PH as one of just of 20 rare disease states on which they would focus new patient-focused clinical trial initiatives. In April of 2013 we found out that the FDA had indeed selected us!

I also traveled to the DC area to PHA headquarters twice this year in order to meet with specialty pharmacy representatives. The goal - to implement a feedback system designed to improve patient care and medication delivery. This system is now FULLY IN PLACE and I still can't quite wrap my head around the idea that one patient who wrote a very polite but pretty pissed off letter, combined with countless phone calls and e-mails, and an incredibly dedicated organization for an incredible rare-disease community, has not only met big pharma head on, and then teamed up together, we ultimately made something that can have a really tangible difference. Now we look to the future where it could potentially become a model for other patient communities, and nothing would make me happier.

So... what else...

I completed my third 5K race (and second Warrior Dash... and signed up for a third) and my friend Heather and I raised $1800 for the PH cause.  When I was diagnosed I couldn't climb a flight of stairs without nearly passing out. What a difference a few years of the right medications, a holistic integrative approach, and a good dose of faith and miracles makes.

PHA launched the Chapters, and I attended the first NYC gala in honor of Dr. Robin Barst, a pioneer and hero in our community who was unparalleled in leading the fight for better treatments and at saving lives. Without her, none of us with PH would be where we are today and listening to her colleagues, patients, and family pay her tribute was an honor indeed.

And finally, this year I launched Willow Tree Wellness, my holistic health coaching practice. At first glance, this may not seem PH related, but to me it very much is. PH stole my teaching career, and I hated that. Mourned it in fact (Yeah, I cried when I got rid of some teaching supplies!). I left the classroom (ah-hem, that's a nice way to say I was covertly "let go" because sick teachers are inconvenient), and while I'm doing great, it is abundantly clear that a full time high-stress job with germy little kids is not in my best interest... so I went back to school and became a health coach. The irony of the sick girl helping people to find wellness does not escape me at all (in fact, it makes me laugh)... but I really cling to the fact that our bodies are meant to find wellness (which is different than total "good health" or a cure), and you can certainly always work to feel and do better. I truly believe that an integrative approach has saved me, and I know I can teach others to find the same.  Add to that I get to work with families and children as well and... life is good. Very, very good.


Oh, and just for fun, I noticed this blog has 13,000 hits as of today. Thanks for stopping by. :)

Happy six years to me. Here's to the next six... I wonder if I'll be a long-term survivor by then or if the bar for years that make up "long-term survival" will just keep going up and up as things get better and better for us. It's kind of amazing to wait and find out.