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.


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.”

Friday, November 22, 2013

Meet the PH Peddler: A 3000 Mile Journey

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.

Post PH Fundraiser Celebration
Today’s introduction is about a rather unique PH patient. From his medical journey to how he chooses to live his life and dedicated himself to the PH cause, Joshua Griffis brings something special to our community.

Joshua’s journey with PH officially began in 2010. For some time, he had been experiencing shortness of breath, extreme fatigue, and general weakness. He was working as a corrections officer in a jail at the time, and one day after running to break up a fight between inmates, he blacked out.

However, it was really the fatigue that first led Joshua to seek treatment. Like so many patients, he was experiencing a feeling of being extremely run down, and unable to garner his energy. The doctor he saw wanted to rule out pneumonia, and it was during the chest x-ray that aortic aneurisms were discovered.

Initially scheduled for corrective surgery, the doctor wanted an echocardiogram prior to the procedure. It was then that the extreme elevated pulmonary pressures were found. The doctor called and told him he had pulmonary hypertension – and that he had perhaps six months to live.

The cause of these aneurisms which kicked everything off is still somewhat uncertain – rare diseases such as HHT and Loeys-Dietz syndrome have been tossed around, but a solid diagnosis continues to be elusive. Whatever the underlying cause, this much is certain, Joshua still has severe pulmonary hypertension.

Instead of surgery, Joshua found himself undergoing a right heart catheter. He was immediately admitted to the hospital and started on Flolan. However, before discharge, the doctors elected to try just oral medications, starting with Adcirca.

As with so many PH meds, the side-effects were intense. Experiencing extreme headaches and heart palpitations added to the breathing challenges that were already present. Eventually, Sub-Q Remodulin was added, and finally IV Remodulin was settled on.

Fast-forward to the summer of 2012 and Joshua was presented with a unique opportunity. He applied for a job at the Pulmonary Hypertension Association and was selected, becoming the first full-time employed PH patient at PHA, and is currently the National Support Group Coordinator

I talked with Joshua about his experience there. What’s it like signing 10 – 20 condolence cards per week for patients who have passed from the disease we share? What’s it like to experience that kind of emotion, and to interact so personally with the ups and downs that come with this disease? He stresses that finding outlets are important, for him: reading, writing, and the support of close friends and family being chief among the strategies he draws on.

While Joshua is already dedicating his life to the PH cause, this past fall he took it up another notch. The Race of Our Lives Campaign has launched, and four women formed Team Phenomenal Hope to cycle 3000 miles across the nation. In his own way, Joshua is joining them.

Inspired by what the team will accomplish and experience, he has created the PH Peddler in an act of solidarity. Launched on September 9th, 2013, he too will cycle 3000 miles, spread over the months between his start date and the race itself. The plan is to cycle a certain number of miles per weekday, with weekends either off or for make-up time in case of illness or set-back. With PH, we always have to have a contingency plan.

The goal – to raise up to $50,000 towards PH research, and to engage people at the community and corporate levels in meaningful ways.  There has been a multistage roll-out, starting with social media on Facebook and Twitter and now it is time to take it to the next level and find corporations willing to join the cause.

Joshua says he feels blessed that he can do this, although it is not without challenges. As he wears a continuous infusion pump, he has to be careful to not tug the pump, open the site to infection, or even over-exert. Pacing and sometimes taking breaks mid-ride are key.

Following the PH Peddler on Facebook gives you a glimpse into this journey. Miles covered, feelings as he rides, and if you are paying attention…sometimes inspirational quotes instead of miles. Why? Because when a PH patient gets sick or travels, or overdoes it or just wakes up “off”, everything can come to a halt. The PH Peddler has had a few unexpected breaks as of late – actually, not totally unexpected, given the severity of the disease and reach of this project.

But this is Joshua – given six months to live three years ago, facing multiple critical illnesses, and experiencing things that would shut most people down, he could be sitting at home having given up. But instead of giving in, he bounces back every time, dedicates himself to the cause every day, and is impacting people around the country because of it. There is little doubt the Peddler will be back on the bike and more than make up for lost time in the future. 

Follow the Peddler on Facebook HERE
And on Twitter HERE

If you’d like to get involved in the Race of Our Lives in your own act of solidarity, there are many ways to do so, and they don’t have to involve a bike. Visit to learn more.

Wednesday, November 13, 2013

Meet Steve White

Well, it is PH Awareness Month, and truth be told I don't have a lot of pressing things to write about right now. Maybe that's because life is so full with other things... a nice problem to have. But still, I should spend the month doing something, right?

So, I thought it might be neat to bring you the story of others in the PH community who inspire me. I draw so much of my strength and drive from them... and it gets boring (and slightly self-indulgent) to write about my own experiences so much. The first is Steve's. The others are HERE.

So, without further ado, the first introduction.

Meet my friend and fellow Board of Trustees member, Reverend Steve White.

Steve is the chair-elect for the PHA Board of Trustees, and will take the helm this June at our 11th annual PHA International Conference (you are going, aren't you?)

Steve has been really important to the PH journey for many people. He lost his daughter, Christen, to the disease in 2002 and has turned their story into a way to help others (see why he is inspiring?).

More specifically and importantly, he often helps people understand and go through the grief process that is so often associated with the disease. When you have this disease, or know people who do, you loose people dear to you. A lot.

I remember when I first joined the Board, and realized how many people on there had lost someone they loved, and were carrying on the fight in their memory and honor. I often wonder if I would have the strength to do the same... it seems so raw. We have a lot to thank these people for, as they turn their grief and loss into strength and gain for our community.

You should know Christen's story too. But I should not be the one to tell it. Steve does it much better justice, and you can read all about her, and the importance of early diagnosis and pediatric research HERE.

And be sure to subscribe to the blog for more inspiring stories... I have some good ones on deck!

Sunday, November 3, 2013

Top 10 Things I am Learning by Having a Rare and Incurable Illness

10. Life comes at you fast. It can come fast, it can go fast. "Make every moment count" means more than you'll ever know until you come face to face with just how limited those moments might be.

9. The little moments can become big moments when you stop and savor.

8. You may develop a rather polarizing love/hate relationship with "big pharma" and related industry. This might require some adjusting of ideals, and you're going to have to learn to be okay with that. They're saving your life. They also hold your life in their hands (this can really piss you off when it goes badly).

7. You might have to start doing your own fundraising towards a cure. There's no really nationalized recognition month, like for some cancers (ah-hem... it's actually November for PH), and corporations aren't going to splatter their merchandise with your purple ribbon. Quite frankly, even the people closest to you might not mobilize on their own initiative (for many reasons). So be prepared to lead the charge. If not you - then who?

6. When you do those fundraisers and that awareness raising, the people you might expect to be there sometimes just... aren't. You can't let this be anything other than what it is. Again, for whatever their reasons. Holding on to that won't make your own work or journey any easier. Let it go.

5. When you do those fundraisers and that awareness raising, people you might never have expected will come bursting through and do amazing things for you. You're going to be touched to your core at the generosity of people and what their care for you and your cause will do.

4. You're going to fall in love with your community. You're going to glow at their triumphs. You're going to be shattered when they suffer. You're going to sit in your home and weep uncontrollably when the disease you share takes them away. You can't help but internalize it. If you let it, this grief will poison you. But if you use it, it will serve to push you only to work harder. But, I will be honest, this part doesn't ever get easier.

3. You are going to loose parts of your life, parts of your plans and hopes and dreams, that you can't get back. This is going to hurt. Take time to mourn those. You might have to do this more than once, and it might hit (again) when you least expect it. But don't stay too long in that black place. There is no moving forward there.

Take the time you need, and then lift your head, shake your shoulders, and resolve to move on. Replace those lost things with new things that make you smile. That make your heart sing. You might discover something new and beautiful you might otherwise have never had.

2. Take care of all of you. While you're attending to your medications and doctor's visits, the demands of your family, your daily tasks... make sure you leave room for you. Don't forget to take time to breath (mindfulness and meditation can change your life), and make sure you fulfill the side of you that loves the arts, or nature, or... whatever it is. You might have to find new ways to do it. But still do it.

1. And the top thing I have learned... you never, ever, ever have to accept the status quo. Don't like the outcome predicted for you? Fight like hell to change it. Don't like the way those involved in your care conduct themselves? Fire them (if at all possible), or go charging in and make a (polite and well documented) stink until it changes.

The outcome of all this has little to do with the disease, the doctors, the medications, or even most of the people around you. It is you. You must take charge. You must advocate. You must make the changes.

And then, no matter the outcome, there will never be a "she lost her battle" at the end. Instead, it will be a fight well fought. A life worth living. A journey and an end to be proud of. And, if you are really lucky, the world left better because of it.