Defining the New Normal

I’ve been interacting with PH patients for a long time – about 15 years now – so you might think I would no longer be amazed by the strength, creativity and perspective they bring to their disease. But I am. Case in point: Colleen Brunetti, the first Generation Hope member to serve on PHA’s Board of Trustees, has published an honest and thought-provoking book about her experiences living with PH, Defining the New Normal: A Guide to Becoming More than Your Diagnosis.

Writing a book is no small chore, and in addition to living with PH, Colleen is a wife, mother, certified holistic health coach, teacher and fundraiser. Yet she made the time and found the inspiration to produce this book which takes those with all forms of chronic and critical illness step-by-step through the areas of self-care that Colleen believes are crucial to patients’ overall well-being. Learn more about Colleen’s motivation to write the book and how you can get a copy of your own.

You and your Specialty Pharmacy...an important new tool for you

This guest blog marks the first announcement of an important new PHA service, launched in partnership with Caring Voice Coalition. You will be hearing much more about it in Pathlight and through other channels in coming months. As with many programs and activities at PHA, this one began with one person's story and need and grew from there to involve many others.  In this case, Colleen Brunetti was that person. Here is her story...

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 emails 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.

 View the Specialty Pharmacy Feedback Form

Yes we can...yes we did!

In 2012, the FDA began an initiative to involve patients in the drug development process.  They identified 39 potential diseases of which PAH was one.  Through their public process, the candidate diseases grew to 90.  For each of the selected 20 disease areas during the five year course of this effort, the FDA will conduct a public meeting to discusss the disease and its impact on patients' daily lives, the types of treatment benefit that matter most to patients, and patients' perpectives on the adequacy of available therapies. Besides patients, the meetings will include participation of FDA review divisions.

Today, we got the great news that PAH is one of the 20 selected diseases for participation!

After our Washington rep, Dane Christiansen notified us, here's what PHA's Advocacy and Awareness Director, Katie Kroner, wrote in her announcement...

In October, PH patient and PHA Board Member, Colleen Brunetti, represented the PH community before the Food and Drug Administration (FDA). In her testimony, Colleen argued for the inclusion of PAH in FDA’s new patient focused drug development initiative.

The goal of this initiative is to engage those living with a wide variety of diseases in giving input to the FDA about how the drug and device approval processes can be adapted to better meet patients’ needs. For example, FDA will seek input on the way they assess risk versus benefit during the approval process for new drugs and devices.

FDA selected 20 disease communities to work with over the next three years and the PAH community is one of them! Several diseases associated with PAH are also among the 20 selected, including sickle cell, HIV and idiopathic pulmonary fibrosis.

PHA plans to use this opportunity to build a long-term, collaborative relationship with FDA that will benefit the entire PH community well beyond the term of the initial project. We look forward to updating you as we learn more.


So, PHA was successful and, as always, that success emerged from the "power of one" - an individual willing to step forward and unite with other individuals in the fight against PH.

Like Colleen, you can make a difference, too.  PHA's 435 Campaign matches patients and families with your own Member of Congress.  Over time - and with PHA's help - you can develop a relationship with your Congressman or Congresswoman.  The late Speaker of the House, Tip O'Neill often said "all politics is personal".  He was so right...and it's also true that all politics is local.  Your House Member or Senators want to hear from you and they will respond.  We can only win the battles we must continue to fight if we work together.  We very much need you.   If you would like to find out more about how you can help with your Member of Congress through the 435 Campaign (or make a connection with your local media through PHA's PHAware Campaign), contact Elisabeth Williams at ElisabethW@PHAssociation.org.

I'll close this blog with Colleen's testimony. She made a difference...and so can you.  Drop Elizabeth an email today!

Colleen's Testimony

Hello, thank you for having me here today. Before I begin I would like to extend my sincerest thanks to the FDA for undertaking this tremendously important Patient-focused Drug Development initiative. My name is Colleen Brunetti and I am a pulmonary hypertension patient, looking to my coming 5-year anniversary of diagnosis.

I was diagnosed at the age of 28. I went from a young mom and wife, quickly climbing the ladder in a successful career, to suddenly facing down my own mortality with a disease that I was originally led to believe might kill me in less than five years. I went from planning a life to worrying that I might not see my baby boy reach Kindergarten.

Well, that baby boy entered first grade this fall, and I was there. And I’ve learned a lot.

When you get a diagnosis of pulmonary hypertension, or PH for short, your life changes. For some patients, it means a sudden stop of activities once enjoyed, because you quickly find you no longer have the breath or endurance to sustain them. For other patients, it is finally the answer they were seeking, after too long of searching for an explanation to their symptoms.

Because PH is often misdiagnosed, many who finally get a diagnosis are already in advanced stages, with quality of life severely impacted. Medications can help, but often with severe side effects. The only cure remains a lung and sometimes also heart transplant.

Medication can mitigate symptoms of PH and slow disease progress, and that could be dramatic, or minor, or temporary, depending on the patient.

I have responded well to therapy, although this was not always the case. But I am always acutely aware that this improvement could be temporary. I’m doing great. But PH is still PH. It’s still progressive and the ultimate treatments are still extreme

 The Pulmonary Hypertension Association has blazed a trail of research, education and support. Patients, doctors, caregivers, allied health professionals, volunteers… all coming together to put hope in action, and to spur us forward until we do indeed reach a cure.

Please select “pulmonary arterial hypertension” and “organ transplantation” as disease areas for the initial 5-year pilot program of the Patient-focused Drug Development Initiative. PHA and the PH community are dedicated to working with FDA in a meaningful and constructive way to ensure this pilot program is a success. It is not only our goal to see that the patient experience better informs regulatory decision-making for PH, but that this effort grows into a sustained campaign of close collaboration between FDA and all patient communities, which continuously improves the system for reviewing and approving innovative therapies.     

Thank you.

Colleen, PH Specialty Pharmacies and You...

Here is a guest blog from Colleen Brunetti ... co-founder of PHA's Generation Hope, blogger and PHA Board member.  As you'll see below, Colleen well represents the Power of One as it's exercised for the good of many. Soon, the Specialty Pharmacy Feedback Committee will come to reality. You'll be hearing a lot more details from PHA as that takes place...

Today I am thrilled to bring to the PH community a new initiative that, for me, has been several years in the making. But first, here’s how we got to today…

I have been diagnosed with PH for five years, and like many of you, quickly had to learn to navigate a very complex healthcare system, including the delivery and management of medications that can only be procured through a specialty pharmacy.

It wasn't very long into this experience that it became apparent that this is not always a seamless process. For a couple of years I struggled to get deliveries as promised, or I would call to order a prescription only to find out I needed a renewal (which delayed delivery), or be blindsided by company protocols that did not meet my needs as a patient and made me very concerned for the specialty medication delivery process for the patient community as a whole.

As I fought these issues out one by one, and participated in online PH communities where it was abundantly clear that I was not the only one going through challenges, it became obvious to both PHA and myself that something needed to be done.

PHA has been helping me address these concerns via the Corporate Committee, a group of representatives 

from many of the major companies who have a stake in the PH experience (specialty pharmacies, drug manufacturers, etc.). It started with a letter I wrote to the committee over a year ago expressing my concerns and frustration. The letter was well received by industry representatives and we have been assured of their cooperation and support with what I am about to share.

Through our ongoing and collaborative efforts, I am very pleased to tell you that we are in the processes of forming a Specialty Pharmacy Feedback Committee. This committee will be comprised of patients, caregivers, industry representatives, PHA, and Caring Voice Coalition. Our goal is to create a process by which patients can quickly and efficiently get major concerns with their specialty pharmacy addressed. Further, we will strive to identify both areas where improvements can be made, and also best practices that can be implemented.

This feedback process will be accomplished through an online form which will soon be available on both PHA’s and Caring Voice Coalition’s websites. Our goal, however, is not to become only a complaint department. We want to help by empowering patients to reach out to the right people within their respective provider network, and to help all parties involved understand how to avoid repeat issues in the future.  We want to help specialty pharmacies see where their procedures are not meeting patient needs, and to also see what is working well.

In addition, this form can be used for praise and accolade. Many of us have had fantastic experiences with representatives from specialty pharmacy companies and they need to hear about this too. When we start from a strength-based model, and move forward on what works, we all benefit.

You will be hearing more about this process in the coming months. In the meantime, we welcome your feedback! Feel free to leave a comment on this blog on what you think of this new initiative, ideas you have on how it can best serve your needs, and anything else on this topic that you wish to comment on.

In closing, I would like to take a moment to thank PHA, Caring Voice, and especially the industry representatives who are supporting this process. It isn’t often that we get to see how a patient is not only heard, but responded to in such a positive and pro-active way. I really appreciate that our community is being given this platform, and with such support from all involved. Thank you!

Awareness Month: Voices from the PH Community (#3)...

Just a few weeks ago, Carl Hicks spent 36 hours in the air for an extended weekend trip to Seoul, Korea.

The new Korean PH association had invited him to help infuse the same spirit of volunteerism that they had seen in PHA into their own new organization.

As an important part of his presentation, Carl had asked Colleen Brunetti, co-founder of PHA's Generation Hope group, to speak on film on what her volunteer involvement means to her.

It is a wonderful statement that was well received in Korea and has value for anyone anywhere interested in fighting back against the disease that has intruded into their lives.  So before PHA places Colleen's video on our website, here it is as an awareness month preview...



This is number 3 in an Awareness Month series on the Empowered by Hope blog.

You can make a difference, too.

Rare Disease Day... February 29

Today is Rare Disease Day.

To mark the occasion, PHA partnered with the American Thoracic Society and pulmonary fibrosis groups for a Congressional briefing on pulmonary hypertension and pulmonary fibrosis.  About 40 Congressional offices sent representatives - many because our volunteer advocates contacted them and asked. 

Dr. Susan Shurin, the Acting Director of the National Heart Lung and Blood Institute, hosted and Dr. Seril Erzurum of the Cleveland Clinic and Chair of PHA's Research Committee told the story of the development of PH research and the encouraging story of where she sees it going.  As she spoke of the 9 treatments developed across three unique pathways (endothelin, nitric oxide, prostacyclin) - with two Nobel prizes given for this work - she spoke of new targets and movement towards a cure and the importance of research in this field to other diseases as well.  Her presentation was inspiring but no more so than Nicole Cooper's who spoke movingly of her own journey with PH from diagnosis to her recent operation to remove arterial clots (CTEPH).

When I came back to the office, I read Colleen Brunetti's great blog for Rare Disease Day.  Colleen, a fine writer from the mind and heart reminds us that a disease is not rare if it is in your own body, that treatments are not a cure. 

I encourage you to read Colleen's blog, too.

Calling in the chips for awareness...

Today is PH Awareness Month Blog Day...and I can't think of a better awareness story to blog about than what PHA board member Steve White brought to pass this past week. 

Faith Middleton is a Peabody Award winning (twice) broadcast journalist on Connecticut Public Broadcasting Network.  Dialing back to May 21, 1968, she was a student at Eastern Connecticut State College, a year ahead of Steve White.  For an article she was writing, she convinced the freshman to wear a barrel as part of the Eastern State College Dream Boy contest.  The scene was that Steve was disqualified from the contest due to the fish odor in the barrel.  Faith got her story...and Steve banked a favor due.

Forty-two years later, Rev. White found the picture of that interview and wrote to Faith - who he hadn't seen since college.  He opened the letter with,

"You owe me a big favor and I'm writing to call in the chips."

Well, Faith Middleton paid her debt with style and feeling - and in a big way.  Steve spoke about his daughter, Christen, and PH.  Dr. Terrence Trow who heads up the PH Center at Yale and Colleen Brunetti, an organizer of PHA's Generation Hope young patient group were interviewed in depth...and the interview has been posted online, where you can click on the player and hear it now. 

It's a great interview with a great lesson.  Good things can happen when you make the time to ask.

Introducing Generation Hope: Colleen's guest blog

There's a new group in PHA...and we're delighted as they begin to make their voice heard.  Generation Hope has been forming in recent months as a group that...  Wait, why should I tell you when one of their leaders, Colleeen Brunetti has sent a guest blog.  That's Colleen in the picture with her son Aidan.

So how exactly does one start an introduction to a guest blog spot? Name? Colleen. Why I’m here? I am 30 years old, a mother of one, a teaching professional, and I was diagnosed two years ago with Pulmonary Hypertension. I have a lot to say on the subject! In fact, I’m pretty hard to keep quiet. But as to why I’m really here… well, Rino was kind enough to lend out his blog to a group that I am really excited to be a part of. And it is my privilege, on behalf of the group, to tell you all about us here.


Introducing Generation Hope! We are a group of PH patients in our 20s and 30s. Our road on the PH Journey has been varied – from diagnosis to treatments. Collectively, we’ve probably seen just about all of it. We come together with a distinct purpose in mind. From PHA’s (snazzy new) website, “Generation Hope is an email group for college students, young professionals and other 20- and 30-somethings living with pulmonary hypertension. The group is a safe space to vent, socialize and chat about everything from relationships to exercise to conquering all of life’s adventures with PH.”  Take a look

Personally, Generation Hope has helped me form a new life motto… the goal for me is to be “Thriving, not just surviving, with Pulmonary Hypertension”. The disease turns your world upside down. It rattles you to your core. It replaces hopes and dreams and life plans with uncertainty and fear… and while those feelings may always be there, rearing their ugly little heads on the dark days, it doesn’t have to stay that way all the time. We can take what life has hurled at us and hurl it right back with a positive spin. We can have hope. Most of all, that hope is in a cure yet to come.

There is a saying that gets bounced around the PH Community, “Hope is a verb”. Reach way back, if you will, to 7th grade English class… a verb, by definition, is an “action word”. It shows action. Now if your memory is REALLY good, you might be thinking, “Wait, so that makes hope a noun. It is an idea, not an action”. Sorry, wrong! Without action there is no hope – and hope must be put into action. So, we take a little license with the English language (apologies to Webster and friends) and state, unequivocally, that “HOPE IS A VERB”.

So, what does that action look like? Well, for me and my three co-leaders of Generation Hope it means stepping forward and spearheading the e-mail group and Generation Hope’s future direction. Those three other leaders – Brittany, Lindsay, and Sean – are also pursuing pretty amazing PH advocacy projects of their own.

For Generation Hope itself, it means we are taking our first baby steps as a group and joining in on the “Path to a Cure”: This February 19 - 26, two PH specialists and Pulmonary Hypertension Association (PHA) Scientific Leadership Council members, Dr. Ray Benza (Allegheny General Hospital) and Dr. Robert Frantz (Mayo Clinic), along with Jessica Lazar, PA (Allegheny General Hospital), are tackling the ultimate challenge together - climbing Mount Kilimanjaro, Africa's highest mountain, reaching its summit on February 26, 2010! Their goal? To find a path to a cure by raising $100,000 to support PHA's research program and patient- and family-serving programs.

We are challenging our Generation Hope members to create giving pages of their own to raise money in support of this amazing cause. Wish us luck. Please support us in donations, positive thoughts, vibes, and prayers. Tell your friends – tell them about us, tell them about PH. Keep the hope alive and in action.

Thank you.

Colleen and the Generation Hope Leadership Team