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

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!”

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.”

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 9^th, 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 http://phassociation.org/RaceOfOurLives to learn more.

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!

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.

Because I Fly by the Seat of my Pants...

So... usually around this year I am in the early stages of planning my annual Zumbathon.

But this year my life is insane. Why? Well, because I got the bright idea to rent office space for my holistic health coaching practice and now I need to fill it with clients. And that is taking over my life.

So the Zumbathon is on hold, and it's all good... because yesterday I had a brilliant (if I do say so myself) fundraising idea.

In short: There's a national event going on where some racing bikers are doing the Race of our Lives and dedicating their efforts to PH.

And there are events starting to spring up across the country in solidarity, and to contribute to the cause. The cause, of course, is PH. And all proceeds go directly to PHA - the best non-profit around (and yeah, I do say so myself on that one!)

So, now I am one of them! I, along with my dear friend Heather, plan to dedicate an event we were already doing to the same cause.

And it's all crazy. And super fun.

And if you want to know more (and of course donate - pretty please?) go here: http://www.firstgiving.com/fundraiser/colleen-brunetti/RaceofOurLivesVirtualFundraiser

                                                                       Thank you!

If we could...

I think I'm a tad late to the party, as I'd seen this video posted a few times and hadn't taken the time to watch it myself (who has four + minutes these days?)

But... I finally did. And it is so worth posting here.

Why? Because I live parts of this every day. And if I'm not living it, someone I love is. I was a bit shaken at how many of the scenarios I am intimately familiar with... or have considered, or will have to consider.

One thing you can't escape about this disease is the humanity of it all. And I feel that those on the clinical side miss that sometimes. I don't blame them, actually. It is exhausting as a patient. I can only imagine that as a professional dealing with us you must shut yourself off from crisis after crisis at some point, and just respond to the routine with precision, maybe even a script.

But the person on the other end of that script and protocol? That's me.

So I really want to sit every medical professional, care provider, and industry person down who has treated me like one more on a list for the day and remind them... it's me. Whatever you say... I am going to hear terrible news that is going to wreck me for a week, or wonderful news that is going to keep me floating for days. What you say is going to matter. A lot.

Whatever it is, I'm going to be in front of you with a straight face, jot some notes, ask some questions, and then I am going to go into the parking lot or hang up the phone, and really feel what just happened, and over the next several days, I am going to re-live it, and maybe wish I had reacted differently.

I have my major check-up coming up this week. Just a half day at the hospital for a round of tests - not much compared to what some go through. But I can tell you right now - I probably won't sleep much the night before. I never do. Anxiety? Anticipation? What will it be this time? More good news (gosh, I hope so), or bad news I wasn't expecting? What will the following days and weeks and months look like? Because everything that happens in those few hours will set the tone for what I experience next.

Then and Now

Sometimes it is hard to remember my world before I had a growing list of friends waiting for lung transplants because the disease we share took too much of a toll.

What was life like before I (willingly) dedicated hours to fighting different fronts of this disease, the personal and the ones that impact my community? What was life like before I had to take medication four times a day, constantly having a near-miss on a dose as hours speed by, as only they can when you're on a tight medication schedule (Four hours gone by already? Crap!).

Was there a time when exhaustion wasn't the big thing I simply can't overcome on some days?

I'm not sure I miss those days, exactly. Life is still oddly charming. I say "oddly" because... well, this whole journey is in fact crazy.  I guess with a different response, I could loose sight of the many charms and blessings that still come in spades. But, I'm sitting here tonight with so much on my mind, most of it pretty stressful and PH related, and realized I barely remember life "before". Before PH.

I don't think I'm the same person now - for better or for worse. A shorter fuse sometimes, maybe. My tolerance for bullshit is remarkably lower. And a much deeper sense of peace on other things. Because, let me tell you, "2 - 5 year survival rate" puts things in rather quick perspective... you learn to let the little things slide. And the big things? Well, those feel really big.

Wellness

I think about the concept of "wellness" a lot. Part of it is probably because I am a certified health coach now, and so helping people to a place of wellness is what I do.

The irony of this does not escape me. I have one of the most deadly lung diseases on the books, and I teach wellness. Actually, I think having PH gives me a pretty unique platform to do this from. Because, against all odds, I'd call myself a pretty healthy person. First, I was supposed to be dead. But that didn't happen. Then I was supposed to get progressively disabled. And that happened for a short while, but it has reversed. Then the meds were supposedly going to stop working someday. And that hasn't happened.

In fact, in the past couple of years I went from being critically ill to probably the strongest I've ever been. Why?

This is the funny part - everyone I know can tell me why. And the reasons fall into a few distinct categories.

The medical folks: "Your medications are working! Yay for modern science!"

The religious folks: "God has heard our prayers! Yay for miracles!"

The alternative folks: "Medication could never do this! Yay for integrative approaches!" (fill in your favorite cure-all: fish oil, kale, meditation, etc.)

And I sit here listening to all these opinions (one rarely takes into account the next), smiling and nodding, and I just keep thinking... what if, just what if, the approach to wellness is not a single road? What if the definition of wellness, and the definition of health (meaning an absence of sick) are two different things?  Can you be well even though you are sick?

I am not cured by any means. No - PH is still very much a part of me. I am, in fact, still very very sick. I fully realize that all this progress I've made may reverse and all the dark predictions of what this disease can do, what it does to my friends, may happen to me. I suppose the fear of that drives me too.

All I knew when I began this journey is that the status quo of having PH was not going to work for me. I rejected the premise of illness overtaking my life. Idealistic? Absolutely. Stupid? Could have been. But it worked. 

What I knew for sure is that there were medications that I could take, and they would buy me time. But the financial and physical prices would be, are in fact, very high. With no cure yet in sight, this simply was not good enough to be the stand-alone approach. I came to know that if I was going to get well, I had to get out of the PH box as soon as possible.

And that led to a twisty-crazy-ever-changing path to wellness that I am on. I take supplements, I practice mind-body work, I exercise, I drink my kale smoothies, I see my alternative practitioner, I see my doctor, and I take my meds. And perhaps most importantly, I fight like hell. Take the fight out of the halls of the hospitals and into the world. Awareness raising, advocacy, patient support. Striving for altruism is key too. You can not fight this for your own sake alone.

So, no, it isn't just the medication, or the prayer, or the alternative work. A path to wellness is a deeply personal thing. I don't think what I'm doing will necessarily work for the next person. But I do believe we all have the potential to find a personal level of wellness, no matter what the physical condition and prognosis is. And that is a journey well worth taking.

A Tribute

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

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

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

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

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

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

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

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

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

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

Truly, she is something to aspire to.

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

Win-win!

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

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

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

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

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

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

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

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

We got it.

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

You can read more details on Rino's blog HERE

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

Let's get this thing done.

Big News!

 Two pieces of news.

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

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

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

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

Happy Anniversary to Me

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

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

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

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

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

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

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

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