Friday, April 11, 2014

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.

Monday, April 7, 2014

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.



Saturday, April 5, 2014

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.

Thursday, January 2, 2014

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.

Wednesday, December 11, 2013

Big News. Really, Really Big News!

Drum roll, please... after years of hard work, the Pulmonary Hypertension Association and Caring Voice Coalition are rolling out something that will address a challenge that has been at the forefront of patient concerns for far too long.


Introducing: The Specialty Pharmacy Feedback Form.


What is this thing and where did it come from? Well, for me, it started back in October of 2010. At that time, I was in a deep battle with my specialty pharmacy company. They were having serious issues with getting my life-sustaining medication to me as promised. And then there was the pivotal moment I will probably never forget.


At the time, I had to sign for delivery for my medications. When the meds didn't show up one day and I had to wait at home again the next day,  I missed an event at my son's preschool. I was livid. I was on the phone with a department manager (having long since given up on working with the call center reps) and sputtering out my frustration. I'm usually articulate - I was too upset to be at the moment.


Then the manager said, "I understand your frustration, I'm a mom too." And in that moment I knew how much she didn't get it, and clarity returned.  I replied something to the effect of, "Yes, but you are pretty well promised you will see your children grow up. I'm not promised that because with this disease I don't know if I'll live long enough! This mistake made me miss a moment in his life, and I can't get that back."


Then I hung up the phone and sobbed. To express that fear aloud was more painful than anything else I had to deal with regarding PH or the pharmacy company. It still is.


Well, we eventually got the delivery issues straightened out, and while I still lived in slight unease as I had completely ceased to trust the company, things seemed okay.


Then a new mess surfaced. I started a new medication and had an adverse reaction. The way you track an adverse reaction is simple: get the lot number and report it to the manufacturer, which is exactly what was requested by the drug maker. But as it turned out, the specialty pharmacy's distribution protocol at the time was ineffective in that they did not track such things. In other words, I had no way of reporting my adverse reaction to the manufacturer, and thus there was no way to track a potentially dangerous situation for other patients.


To be fair, tracking lot numbers at the point of pharmacy distribution is not an FDA requirement. But I would argue it should be considered best practice and done anyway - especially when the medication in question has the power to save someone's life or quickly kill them if something goes wrong. And as I knew the pharmacy's competitors were tracking lot numbers, I saw it as industry standard that absolutely should have been practiced.


Every time I tried to talk to someone to deal with this issue, I got vastly conflicting information. In short, I felt I was either being lied to (probably not the case), or literally no one had a clue what they were talking about... although I do believe they thought they did and had good intentions, there was clear disconnect between information I was being given, and what was actually happening.


It would take me pages to explain what this particular battle entailed. In short, I ended up on the phone with everyone as high up in the company as I could stalk, my doctor's office wrote a strongly worded letter of protest, I filed complaints with HR for my husband's company urging them to drop this specialty pharmacy from their insurance plan, contacted the biomedical company that distributes the drug and complained, and so on.


Still, I felt I was getting nowhere. The misinformation persisted, and I never did get to report my adverse reaction in an effective way.


And then the next pivotal moment. There I was sputtering on the phone again - this time with people like corporate pharmacy managers and the head of global patient safety for a drug company. And I was repeatedly told, "You have my phone number, you call me if you have more issues." And I finally replied, "That's all well and good, but what is the next patient with problems going to do? They don't have your number.  And I don't want your number. I want your company to do their job."


And I knew - even if I somehow got my own issues straightened out, odds it would help anyone else were slim. And the idea that other patients were going through this same mess was unacceptable to me.


As luck would have it, this was around the time of a PH related conference in Boston in 2011. I was in attendance, along with the PHA president, Rino Aldrighetti. I told him what was going on, and that I was having trouble getting a certain key person to return my call. He picked up his cell phone and made the call himself - and he lit that person's voicemail on fire. My jaw hit the ground, as I had never heard a sharp word from him before, and this was an entirely new side of the PHA leader.


Rino then asked me to begin to track the time I was spending on these issues, and to write a letter to the Corporate Committee for PHA and express my concerns. This is a committee made up of representatives from many of the corporations involved with PH care, including drug companies and the specialty pharmacies that distribute their medications. I did so, and what became known as THE LETTER went out. I guess it caused a stir... or so I am told.


We have continued to do hard work on this issue in in the two years since. I have flown to PHA headquarters twice and, along with PHA staff, a doctor, and members of the Board of Trustees (dialed in by phone) met directly with leaders from one of the pharmacies. Countless e-mails and phone calls have gone on.


A Specialty Pharmacy Advisory Board has been launched, comprised of: a patient (me), a caregiver, representatives from specialty pharmacies, representatives from drug companies, The Pulmonary Hypertension Association, and The Caring Voice Coalition. We've discussed in detail the issues at hand, and I have been forwarding individual patient concerns to those directly involved for months.


We see issues and trends. We see areas of strength. And now, we want to hear from YOU.


Please, please, use this form to offer feedback to the specialty pharmacies. When you have an issue, be it small or large, report it. When you have a praise or accolade, we sure want to hear about that too. I continue to believe that change is best made when we build on strengths.


Your comments will be accessed regularly by both PHA and the specialty pharmacy for whom it is intended, and all entries will be tracked carefully for trends and areas of ongoing concern. I have worked really closely with these people over the last several months. I am entirely confident that those on the ground care a great deal about these issues and are making sure changes are made. The Advisory Board will continue to meet and discuss as well.


And if anything, I want you to remember... PH has dealt us a really lousy hand. But we are never victims unless we allow ourselves to be.


Three years ago as I sobbed in my driveway after slamming off my phone, I never would have dreamed that such progress and change could happen. But it has.


And now you have to use it to make it work.


It is here: The Specialty Pharmacy Feedback Form












Thursday, December 5, 2013

Meet Justin: When Giving Back Also Inspires


The final installment for 2013 for PHers who inspire... the previous editions are HERE
Justin is a recent high school graduate with a story to tell. This past June, I was lucky to hear that story first hand.  It is a story of incredible ups and downs, of defying the odds, of uncertain times, and of triumphs.


Born in Colorado, Justin is one of triplets, and he also has Down syndrome. At birth, the family learned he had pulmonary hypertension. 
Surgery
As can be common with Down syndrome, Justin underwent open-heart
surgery at just four months old. Because of complications, he spent a subsequent five weeks in ICU.



By age 2 ½, Justin was on oxygen constantly. Doctors told his family that the elevation in Colorado was too much for him, and that the family must re-locate somewhere closer to sea level. They chose Connecticut, within distance of Dr. Robyn Barst at Columbia in New York City, and that is when Justin’s journey with pulmonary hypertension really began.


Dr. Barst was the preeminent physician addressing pulmonary hypertension at the time, for the general patient population, but especially with pediatrics.  Justin and his family went through a lot as they tried to fight the disease, and Dr. Barst was with them every step of the way.


Justin has had two pace makers and been on many PH medications. For a long time, Flolan saved his life. Being a child with a backpack that carries a pump attached to an IV into your heart is not easy by any stretch, but Justin persevered for 12 years. The family tried him on Remodulin too, although that was not as successful. Then, on a second attempt with Flolan, Justin hit a critical point. The drug is extremely powerful, and it can also be extremely toxic. For Justin, this meant a severe drop in platelets, which led to lung bleeds, amongst other multiple complications.


And then, the miracle came for Justin. An inhaled medication called Tyvaso had just hit the market, and it was decided that Justin should try it. Tyvaso, combined with Adcirca, turned things around. Justin’s health started to improve. He competes in the Special Olympics – and medals. He graduated high school in  June 2013, and is now in a transition academy. His parents tell me they hope that someday he will attend college.


So, when to everyone’s happy surprise, Justin reached his senior year in high school and the time came to choose a senior project, Justin chose “Giving Back to the Organizations that have Shaped My Life”. He chose the Pulmonary Hypertension Association and The Special Olympics.



Fundraising
Justin worked tirelessly on his project. Two bake sales, and a pasta dinner, were all smashing successes. Through his efforts he was not only able to raise money for causes so important to him (an impressive $3,000 in total), but he was also able to spread a great deal of awareness as well.


I didn’t get this story from an adult well versed in medications and PH. Justin told it himself. As I sat in an auditorium at Shepaug Valley High School, I don’t think I ever stopped smiling or shaking my head in wonder. Justin is a remarkable person. He had clearly put so much into his project and presentation, and presented it with a level of articulation more seasoned speakers can lack. And I would be remiss if I didn’t mention that he gives the best hugs too.
 
He told his whole medical story with such fineness. His pride in his accomplishments beamed through, as well they should. Justin has defied every odd. He works to give back to the communities that supported him in his fight. We can all learn a thing or two from this remarkable young man.
Justin presents me with the check for PHA

Justin's Family: Sister Lindsey, Justin, Dad Russ, Mom Lori, and Sister Jenni



In closing, the funds Justin raised for the Pulmonary Hypertension Association will go to the Robyn Barst Pediatric Research Fund. As Justin said, “ Because of Dr. Barst and her incredible work I am alive and able to graduate! Thanks to Dr. Barst, I and thousands of others with PH are living longer and better lives. She is gone but her work will live on forever!”

Wednesday, November 27, 2013

Meet Diane Ramirez

For PH Awareness Month 2013 I'm bringing you stories of people in the PH community going the distance. Each in their own way, they have found a path that takes control around the disease and makes our community a better place.  You can read the other installments HERE




“I went through the hell that everyone is still going through today.”

Diane and me at "The Kitchen Table"
where PHA was first brought to life.

Diane Ramirez is a name many in the PH community may recognize, and with good reason.  As a 26-year survivor of pulmonary hypertension, she is perhaps one of the longest living patients we have in our community. And what she has done in her time with the disease is nothing short of amazing. It is my pleasure to bring you her story.

It began, likely, around 1984. Diane had asthma from early childhood, but around this time, her shortness of breath and difficulty with breathing began to increase. For three years, she searched for answers. Changing medication wasn’t helping, in fact it was making some things worse. She went from being told that her asthma was worsening, to that she had a mitral valve proplapse, to being referred to a therapist and being put on meds because the symptoms were supposedly all in her head.

As Diane put it, “I went through the hell that everyone is still going through today.” And she’s right… even so many years later, patients still struggle on average 2.8 years searching for a diagnosis.

It wasn’t until 1987, at the age of 24, when she passed out going up a flight of steps, that answers began to emerge. When she came to, she was in the ER. Still in her sharp business suit, surrounded by doctors who were as confused as she was. A medical history, blood work, x-rays, CT scan, and finally a cardiac cath finally yielded the answer – Pulmonary Hypertension. The diagnosis came as a relief of sorts, finally an answer to the issues that had plagued her, and validating her concerns. However, at the time, there was nothing they could do for her but put her on blood thinners and begin the process of being worked up for a transplant.

In addition to no medications, there were also really no other patients to be found. PHA didn’t exist yet. Flolan trials had not yet begun. And the people taking care of her weren’t PH specialists. They didn’t really exist the way we know them now yet either. Instead, she was under the care of the transplant team at Yale.

“His story is a part of my story.”

Finally, Diane “met” another patient. It was her own brother, Ariel Ramirez, diagnosed at age 18 in 1992. It turned out their particular form of PH was familial. Even at that time, there were only about 190 diagnosed patients in the country, mostly women, and Ariel had struggled to get a diagnosis as well. There was much loneliness in this rare disease, and the two bonded over their shared experiences. Ariel had a wonderful sense of humor, with a contagious laugh, and he was incredibly brave. During a trial to try to transition him from IV to oral meds, he suffered a massive heart attack and passed away at the age of 36. His efforts and his ultimate sacrifice are a part of PH history.

Staying the Course

Throughout this, Diane had her own journey with treatment. She had originally met with doctors in Providence, RI who were starting the Flolan trials, but she did not qualify because she lived too far away. In addition, the idea of IV meds was so overwhelming, that it was almost a relief not to go on them.

By the time Flolan was FDA approved, she had started to stabilize, and opted to stay off the only treatment for PH at the time. This might sound crazy to some… but the idea of a permanent IV in the chest, with all it entails, never mind it was such a new medication at the time, was too much.

Diane credits her ability to persevere with doing everything she could to stay healthy: eating well, keeping the house clean of irritants, wearing oxygen, and exercising. She’s also quick to point out that there is absolutely no reason for people to forgo medicine today. There are so many options out there.

Still, in 2001 she had started to go into heart failure, but remained, as she says, “mule headed” about IV medications. She happened to visit Duke University during a vacation to North Carolina and learned that clinical trials for Tracleer had begun. She relocated to North Carolina to start a new life for herself, a life that would include this new drug Tracleer.

At this point she was on oxygen 24 hours a day. The first months were difficult. She slept a lot, with very low blood pressure. And then, six months into it, something changed. She was at the mall with her sister and turned from window shopping to see her sister crying. Confused, she asked what was wrong… and was told she had just walked the entire mall without getting short of breath or needing to sit and rest, and she hadn’t even noticed. Slowly but surely, things were turning around, and it was truly a blessing.

A little while later, Diane started Ventavis as a precautionary measure. She did that for two years, before embarking on the Tyvaso trial and now has been on Tyvaso for five years. She’s tried Revatio and Adcirca as well, but they have not proved to be a good match with side effects. Still, she thinks she might try again sometime.

PHA

Around 2002 -2003, a family member gifted Diane with a computer. She was so excited to have access to the Internet from her home. She found PHA online and the first patient she ever chatted with was Alex Flipse. They exchanged phone numbers, and started calling. She began to meet more patients and eventually found the local Piedmont support group, with Cindy Pickles, who still leads the group today and also sits on the PHA Board of Trustees.

Diane’s first PHA Conference was in Texas in 2006. That was it for her – she was in! She says, “It was like somebody threw me the football, I caught it and started running… and I’m still running. Meeting all the patients was overwhelming, hearing the doctors talk about the research… it was really motivating, and meant so much.”

Diane snags not one, but two,
North Carolina Congressmen during
a recent advocacy visit to
Capitol Hill.
Diane also heard Carl Hicks speak that year, and learned from him that “Hope is a Verb.” She spent time with Katie Kroner, PHA Director of Advocacy and Awareness, and learned about the 435 Campaign. Katie also gave her the confidence to begin advocating for PH specific legislation. Today, Diane leads the way nationally on advocacy work, and is supporting patients who are mobilizing and reaching out to their representatives, specifically now for the Pulmonary Hypertension Research and Diagnosis Act of 2013.
Her first up close and personal experience with PHA was a wonderful thing. Of the experience she remembers,“Talking to Katie and hearing Carl… and realizing I needed to take action. I can’t wait for somebody else to fight my battles. This is mine.”

Well, battle she has. In 2011 Diane also joined the PHA Board of Trustees, where she has continued to lead the way in advocacy work, and is helping to meet the needs of patients, particularly long term survivors who need their own unique spin on support. She says, “It’s so cool to see stuff coming to fruition and take off, and know that I was a part of that.”

I asked what gives Diane her motivation today. She laughs deeply and says,

“To be a long term survivor with this illness, and to be a part of the group of people who started with nothing, to being a part of PHA today, you have to be a little “touched”, a little fragmented part of your brain that helps you get through it. It is so challenging, so extremely overwhelming… it has been an incredible upheaval, and major change, and extreme positives… one end of the spectrum to the other… you have to have a little insanity in your life to make it through.”


With Diane, you should be prepared to laugh with her from your core… and maybe cry from there once in a great while too.  She’s right. This is a crazy, crazy journey. I for one am very glad she is on it with us.

Her advice to long-term survivors is, quite simply, “Never give up.”

To the newly diagnosed, she says the same, but adds, “Get involved. Now that you’re diagnosed with PH, this is your battle. Get involved with it. Be a part of the fight.”