A Cure for Heart Defects!

“I believe that this nation should commit itself to achieving the goal, before the decade is out, of landing a man on the moon and returning him safely to Earth.”
– President John F. Kennedy, May 25, 1961

 

On September 29, 2010, Fran Visco of the National Breast Cancer Coalition appeared in a video posted to YouTube. It contains a powerful message.

“We can’t count on hope as a strategy to end breast cancer. I’m giving up hope, because hope is not a solution. Hope will not end breast cancer; we need a plan, a goal… a deadline.”

What a statement… and what a shift in thinking! Think about what she is saying: HOPE CAN NOT CURE OUR DISEASE. Hope is a good and pure thing, a friend we hold close in our most desperate moments. Take note that she did not say “I am giving up on hope,” because she’s not. Hope is what keeps us going when the world crashes down around us. But we need to realize that hope alone can not fix defective hearts.

A popular phrase that you may have heard is “We will cure (chronic illness) in our lifetimes.” The problem is, all generations living with the chronic illness use that phrase as a rallying cry. Time moves on, the older generation passes away, but the phrase is still being used. Eventually, the urgency disappears. There is no real urgency for beating the disease… just an empty promise that it will happen “in our lifetimes.”

In the same vein, “Awareness” is not an endpoint for the Heart Defect community. What good is awareness if it does not bring people to take action? We are winning – the number of deaths caused by Heart Defects is dropping. The American Heart Association estimates that in 2005, 3,500 people died from Congenital Heart Defects. That’s the most current statistics that they have available, so the number may even be lower today. But that’s not acceptable. That’s still 3,500 of our friends, our families, our sons, and our daughters – dying each and every year.  Being “aware” of a Heart Defect isn’t enough, so perhaps it is time to redefine ourselves, and rethink everything we’re about, everything we are fighting for.

It’s time to declare war on Congenital Heart Defects. All out, bare knuckle, unrestricted mortal combat. Like Miss Visco said, there needs to be a plan, a goal, and a deadline.

THE PLAN:

POINT ONE: To find new medical and surgical options to increase the survivability of Congenital Heart Disease;
POINT TWO: To research the occurrences, causes, and possible prevention of Congenital Heart Disease;
POINT THREE: The reduction and elimination of mortality and disability associated with Congenital Heart Disease.

THE GOAL: To eradicate Congenital Heart Disease.

THE DEADLINE: November 29, 2024 – the 80th anniversary of the first Blalock-Taussig shunt.

So how are we going to do it?

I can’t answer that. I am not a doctor or a scientist; I have Bachelor of Science degrees in History and Political Science.  And to add to the challenge, we are going to have to do this all by ourselves. If we’ve proven one thing, it’s the fact that Congenital Heart Disease is an invisible disability and without a unified approach, we have no voice. For several years the Adult Congenital Heart Association (ACHA) traveled to Washington DC to lobby for a federal level registry of Congenital Heart Defects. Only after we united with seven other nationally recognized Heart Defect Support groups and formed the National Congenital Heart Coalition were we able to get legislation introduced in Congress and eventually inserted into the Patient Protection and Affordable Care Act.

So lets put our heads together again. It will be up to us to not only do the fund-raising, but to be the first ones to give – give our money, give our effort, and give our time. We have to gather the resources, fund the research, educate the public and lead the charge. If we don’t support our own cause, why should anyone else support it?

In 1961, President John Kennedy challenged the nation to put a man on the moon before the end of the decade. Back then, we could barely get a rocket off the ground, much less think about going that far. Along the way we won some major battles and we lost some good people, but in July 1969 the Apollo 11 crew landed safely on the moon and came home.

We have to be willing to put everything we have on the table and draw that last card. It could all blow up in our face…but we could also hit the jackpot!

Let’s rise to the challenge. This is our Apollo program, our impossible dream.

Let’s go to the moon.

Grunt Work

I was in Philadelphia last April, helping out at the offices of the Adult Congenital Heart Association (ACHA) as they prepared for Lobby Day 2010.  On Lobby Day ACHA members – joined by members of the Children’s Heart Foundation and Mended Little Hearts – gathered in Washington, DC to visit the US Capitol. Our goal was not a sight-seeing trip, but to visit our legislators and convince them to fund Congenital Heart Defect provisions of the new healthcare law.

Knowing the ACHA office would be swamped with work (we had a total of four people in the office at that time) I volunteered to come to Philadelphia a few days early and help out any way I could. Unusually enough, “any way I could” turned out to be…

…. copying, sorting, and stapling.

Wow – that was a classy assignment there, Steve. They must not have needed that much help, after all!

Hardly. The ACHA staff was still setting up appointments with the legislators or their staff when I arrived. 48 hours until we left for DC; 72 hours to Lobby Day, and things hadn’t fallen into place yet. When we weren’t making appointments we were on the phone with our Advocacy Director working out final details. There was also a small crisis with the host hotel that wasn’t resolved until the last moment.

Making legislative appointments for 100+ attendees, smoothing things out with the hotel, working with the Advocacy Director to make sure things were just right… I don’t know how to do any of those things. So I was copying, and folding, and sorting, and stapling – so that the people who do know how to do those things didn’t have to worry about it. Or stop what they are doing to do it.

It’s “grunt work” – the repetitious, mindless type of job that no one wants to do. Everyone there could have done it, but they had skills that I don’t have, so I did the job. My best guess is that I handled 500+ separate sheets of paper. If you attended Lobby Day 2010, your informational sheets were assembled by me. (Don’t worry, I washed my hands!)

This is what advocacy for any worthwhile cause is – the willingness to do the little tasks, the grunt work, the behind the scenes activities that makes everything flow smoothly.

Second Hand Heroes

We’re not your classic heroes. We’re the other guys. – Mystery Men (1999)

The other day I discovered a Facebook based Heart Defect group engaged in a pointless mental exercise: They were listing celebrities that are connected to the CHD Community in some way that they feel should become a “Congenital Heart Defect representative”. Once that happened, “awareness” would be raised, money for research would come pouring in, and we’d all be saved.

Oh good grief.

I’ve seen this happen before; the group concentrates its efforts on recruiting that one big megastar and ignores the people who are actually doing the job. Because true advocacy for an issue is difficult work. You don’t just stage a few events, look good for the camera, and recite the proper sound bites. It takes time, and the job is detail oriented grunt work. The i has to be dotted and the t crossed correctly and that is your job. Long hours, very little sleep, and the “pay” is a pittance, if anything. You do this because you believe.

And by the way – if all you are doing is “raising awareness”, then you are not helping. That young mother doesn’t know Heart Defects is the most common birth defect… and she doesn’t care. What she does know is that her child is very sick and the doctors are throwing words at her she doesn’t understand.  She wants someone who can explain this to her in simple English and give her some hint of the future. And even if the future is dire, she wants to talk to someone who will tell her the truth, and gently let her know that sometimes its OK to say goodbye.

I’m honored to know people like this and count them as friends; I’ve also shared some of their stories on Funky Heart! over the past two years. You probably haven’t heard of these people anywhere else. They don’t mind; because they aren’t seeking personal glory.

The most “famous”  Heart Warrior I know might be Heather, a CHDer who saw an ad in a magazine for a TV show seeking women with heart disease. From the wording of the ad Heather realized they were looking for people with Acquired Heart Disease and ignoring CHDers. Heather got in contact with the show producers and gave them a piece of her mind – and as a result, appeared on the TV show How to Look Good Naked!

Then there is George, a retired doctor with Tetralogy of Fallot who spoke at Lobby Day 2010. I always thought George was kind of reserved, but I found out he just waits until the proper moment. When he got through speaking we were ready to tear the walls down! “One day, Congenital Heart Defect survivors are going to live to be eighty, ninety, and even one hundred years old,” he said. George is also doing his part to keep Heart Warriors going – During Lobby Day he took a young adult/older teenager under his wing. Near the end of the day George told him “Remember what we’ve done here. One day this will be your job.”

Karen T. Chavez and Kim Rooks serve as Co-Directors of the Broken Hearts of the Big Bend. Heart Defect Awareness isn’t always the answer, there are times when what is needed is Education and Support. If you have a Cardiac Kid in the Tallahassee (Florida) area, Broken Hearts members can walk you through what may come next – and be right there with you, lending a helping hand when you need it. Why? Because we’ve all been there.

Colorado’s Amanda Adams has a Cardiac Kid; when he was born she could find very little information about his defect and almost no support. Frustrated, she decided that no mother should ever be in such a situation… and began the support group Hypoplastic Right Hearts.

Amy Basken is into everything – right now, she’s on the Advocacy Staff of both the Adult Congenital Heart Association and the Children’s Heart Foundation; serving as the National Advocacy Coordinator for Mended Little Hearts; and serves as Chairperson of the National Congenital Heart Coalition. She does all this “because I am a mom of a kid with a heart defect.”

A former teacher, Amy Verstappen‘s health deteriorated after the birth of her daughter. Unfortunately she had some doctors we were not familiar with Congenital Heart Defects in Adults and they almost sent Amy for a heart transplant! Thankfully Amy found Cardiologist Dr. Carol Warnes. Dr. Warnes saw the X-ray, knew what she was looking at, and got Amy what she really needed – a valve replacement. After the surgery this same Cardiologist challenged Amy to do something about the adults with CHD who were not getting good care. Her journey has taken Amy out of the classroom to serve as President of the Adult Congenital Heart Association.

And not to be forgotten is the late Jim Wong, a PhD who lived and worked on the West Coast. Jim’s training was in Chemistry, but when it came to learning about heart defects he just wanted to know as much as possible… and he never hesitated to share what he had learned.  He often traveled to Washington to serve on a patient advisory committee for the National Institutes of Health, and was at every ACHA event I attended until he was too ill to attend.

Fifty-four years old, Jim was one of the older CHD survivors, and grew up in the days when CHDers were X-rayed, scanned, and fluoroscoped as much as possible. A lot of older survivors pass away after a bout with cancer, not because of their heart. There is currently no medical evidence to prove this, but one theory contends that absorbing all that radiation while they were young leaves older Heart Warriors more susceptible to cancer.

Jim Wong died of cancer earlier this year, perhaps as a result of all those x-rays. I like to think he died the way he lived – helping others learn about defective hearts.

What is a Health Registry?

Before Adventures of a Funky Heart! began I took part in Lobby Day 2007, sponsored by the Adult Congenital Heart Association (ACHA). This was my very first Congenital Heart Defect (CHD) Advocacy effort, traveling to Washington DC to meet with other adult CHD Survivors and to lobby Congress for the passage of an National Adult Congenital Heart Defect Registry. Now, three (almost four) years later, we have one – but it exists only on paper.

So ACHA members (along with a few other CHD Advocacy groups who have joined the fight) keep pounding the pavement in Washington, doing the grunt work needed to obtain funding for the Congenital Heart Futures Act and the registry it contains. But even though we’ve been doing this for nearly four years, the question is often asked: What exactly is a registry? And I’ve even gotten some strange comments, such as the person who insisted that they were not going to turn over their personal information, no matter what. So let’s try to clear up some misconceptions.

A Health Registry can best be described as an Excel spreadsheet: a database of facts and numbers. Fill the sheet with data, apply the correct sorting formulas, and you can learn a lot of information. Obviously, the more data you have, the bigger the pool of information you have to make the calculations and the more accurate they are. (My favorite college football team, for example, has won two games this year and lost none. That limited information points to a undefeated season and a National Championship. Even though I am pretty certain that won’t happen, that’s what the statistics predict.)

No one has to give any identifying information to be part of the registry. That would make it nearly impossible to keep track of anyone, especially women; ladies tend to take the last name of their husband when they marry. I’m not who I say I am either – most of you know me as Steve, but that’s not my legal name. I don’t sign an official document with Steve and it is not the name on my ID or my credit card. You could overreact and have a separate entry for every variation of a name, but that would give you too much information and dilute the results. It would be much easier to give everyone who is eligible to enter information into the registry a code number that will follow them for their lifetime.

The registry would be limited to adults at first. Why? it doesn’t sound fair, but we’ve been there and done that – what better group to use to get results right away. We’ve been through the childhood surgeries, the medications (some of which don’t exist anymore, because we proved they don’t work) and we’ve made it. Get the data from a couple of hundred adult CHD survivors into the registry, and you can begin to see some preliminary trends. And each addition makes the data more accurate.

So once we have this registry up and running, what can we learn? All kinds of useful information! The Centers for Disease Control (CDC) currently produces a work known as the Atlas of Heart Disease Hospitalizations Among Medicare Beneficiaries. This is a good example of a Health Registry, and the data is overwhelming. You can order a printed copy, download it as a .pdf file, and even view a series of interactive maps. (If you look up the South Carolina map, you’ll find that White men aged 65 or older with Heart Failure were hospitalized most often in the Northeastern part of the state – the border counties from Chesterfield County to Horry County. Data that detailed is priceless.)

What else could it tell us? Dr. Wes recently published results gleaned from an ICD Registry. Click the link and read what the registry revealed – the amount of information is staggering. Even though the plan is to gather information from adults with a heart defect at first, the registry is not exclusive and will benefit the entire CHD Community.

Choose a side

I received two invitations via Facebook to “Like” a political party this week. I turned them both down.

I wholeheartedly believe in our system of government and I have voted in every election since I turned eighteen. I’ll vote for the candidate before I vote for the party, but I usually vote *COUGH*. (That’s a nice way of saying “I ain’t telling!”) But when I have the opportunity to discuss Congenital Heart Defects (CHDs) with government officials, I try not to get involved in partisan politics. The Funky Heart doesn’t support one political party over another.

In its early stages, a heart develops as a long straight tube – almost like a drinking straw. In a few days it is viable and actually starts to beat! Later, it rolls over to form a U shape and the U begins to grow together, forming the organ we have all come to know. If you are going to have a Congenital Heart Defect, this is when it begins. 124 out of 125 times the heart forms perfectly, but that one time – 1 out of 125 – there is an imperfection that leads to a heart defect. And all this occurs before your mother even knows she is pregnant. By the time your future mom goes to the doctor for the official confirmation, you could already have a Congenital Heart Defect.

That’s the reason I try to keep the Funky Heart politically neutral. Our government – from the federal government on down – is highly partisan right now. If you identify with THIS party,  you may not be able to build a relationship with members of THAT party. Both sides can have good ideas, and the voters can change the power alignment during any election.  So I feel that it is best to set my personal preferences aside and be able to work with members of any political party.

Newborn children obviously haven’t chosen a political affiliation. Both Republicans and Democrats have heart defects.  So do Independents, Libertarians, and even Green Party supporters. Even those people who choose not to vote at all are affected by them. So if you ask me to choose sides, that’s fine.

You can just count me with the people affected by a Heart Defect.

Perspective

I’ve worn my Kryptonian at Heart T-Shirt several times, but I have noticed that it has gotten different reactions from different people. In case you forgot, fellow CHDer Jason saw this shirt at a comic convention and just had to have one. When I saw it, I had to get one too.

The interesting thing about this shirt is the reaction you get. I wore it one day while hanging out with a group of heart-healthy friends.

Kryptonian at Heart

There were a couple of smirks and a little laughter, and one friend asked me “Superman fan, huh?” ( I prefer Batman. With no superpowers, Batman’s primary weapon is his brain. I have one of those, too!)

But I also wore it to an informal reception held just after Lobby Day. This reception took place the night after our Lobby Day effort and was a both a celebration of the day’s events and a preview of Vision 2020. A lot of the attendees were leaving in the morning, so it was also an unofficial good-bye party.

The reaction of my fellow CHDers was amazing. “I need a shirt like that!” More than one person said. Several people asked me where I got it – no problem remembering that; the name of the company is printed on the sleeve. I even got a couple of “threats” to literally peel my shirt off of my body! (At least, I think that they were joking!)

They took a look at Superman’s S shield hiding behind the human ribs and saw what I saw. Although all our stories are slightly different, we’ve all been cut on, knocked down, knocked out, too sick to move, poked, prodded, and connected to just about every monitor that there is, yet somehow we managed to fight back. They saw not a comic book hero, but themselves.

My buddies back home still think I’m a Superman fan. That’s fine, they are great people and I couldn’t make it without their friendship. But the Cardiac Kids and the Heart Warriors that I know see that shirt and they see their own heart – the heart of a true hero.

George

My friend George spoke at the recent Lobby Day event in Washington DC. I wish all of you had been there to hear him.

Like many of us there, George has a Congenital Heart Defect (CHD). His defect is Tetralogy of Fallot (ToF) and he is a retired Pathologist from out West. That’s right – a retired Pathologist. I’m not going to insult him by listing his true age, but George worked a full career with a major heart defect.

He’s quiet and unassuming, probably the result of his years in the medical field. Pathology is a very scientific branch of Medicine; attention to detail is a must and you do not jump to conclusions. So when George stood up to speak, I wasn’t expecting what came next.

Long story short: George tore the house down! He stated that he had recently celebrated a birthday and then he said;

“I firmly believe that one day those of us with heart defects will routinely live to be eighty, ninety, and even one hundred years old.”

That did it for me right there. I’m 43 years old, but I’m 42 years, 7 months past my “expected” life expectancy. And here’s a respected member of the CHD community, a retired scientist, saying that he thinks that one day we’ll live a good long time? And might even hit the century mark? And all we have to do to start down this path is obtain funding for the Congenital Heart Futures Act? Right then, I was ready to walk down to the Capitol (Three blocks away), climb the dome, and rip that statue right off the roof!

George also has a very dry wit; he can tell a joke with such a deadpan expression that you don’t even realize he’s joking. But his humor can bite, as he proved when he recalled an exchange from his younger days.

“`When I attended medical school they taught me that people like you didn’t make it,’ a doctor told me. I didn’t know what to make of that so I didn’t say anything.”

People like you? Wait a minute… that includes me!

“A few moments later the doctor asked me `Have you thought about which medical school you would like to attend?’ I answered, `Not the one that you attended.'”

You tell ’em, George!

Five CHD Questions: Why?

Why are Congenital Heart Defects (CHDs) the least understood and the least funded of all the birth defects?

Despite being the birth defect with the highest rate of incidence, Congenital Heart Defects receive the least funding for research. One would think that this would be the target of massive funding, intense awareness campaigns, and major research allocations, but that is not so. In fact, it is one of the lowest.

CHDs is an “invisible disability” – we hide in plain sight. Other disabilities and illnesses have obvious signs, but the average CHDer can usually blend into a crowd with little trouble. Most of the time this is a good thing; I personally don’t prefer to stand out. One thing I write about on this blog and occasionally mention in a presentation is look at me – see how normal I am! But that also works against us as we can become lost in the shuffle.

When I was born there was no such thing as pre-natal testing; I was born on a Tuesday but it was Friday before my mother realized that there was probably something seriously wrong with me. Even today, a Fetal Echocardiogram is not standard procedure: an expectant mother normally receives this test only if her family has a history of heart defects or other factors come into play.

Despite what you may hear or read on the internet, CHDs are more survivable today than at any time in the past. A minimum of 90% of all children in the United States who undergo heart surgery survive to adulthood. And every year, the odds tilt more and more in our favor. There are currently about one million adults living with a Heart Defect and about 800,000 children.

With almost two million people living with a CHD, we’ve become our own little world. Survivors represent every gender, every race, and every age group. This diversity has led to advocacy groups of all types, such as the Adult Congenital Heart Association (ACHA), Hypoplastic Right Hearts (HRH), and Broken Hearts of the Big Bend. Every Survivor/Advocacy group serves a segment of our community and each one fills a need.

The members of the Congenital Heart Defect community are many and varied. But the one thing all segments of the CHD family needs is more awareness and better research. Because the goal of every Survivor/Advocacy group is to cease operations  because there is no need for their services anymore.

We’ll get there one day;

Birth of a CHD Advocate

I’ve written about Amy Verstappen’s entry into the world of Congenital Heart Defect (CHD) Advocacy before, but her story is so powerful that you have to hear it firsthand for the full impact. Amy was kind enough to take a few moments on Lobby Day and tell her story once again.

Thank you!

Thank you for being a friend
Traveled down the road and back again
Your heart is true you’re a pal and a confidant

– Thank You for Being a Friend, Andrew Gold (1978)

A very big THANK YOU to the people who helped make Lobby Day 2010 such a wonderful experience:

Amy Verstappen, President of the Adult Congenital Heart Association (ACHA), and her husband Richard; my wonderful hosts and tour guides;

Anna, Fatima, and Nicole; all members of the ACHA Office Staff who welcomed me and put me to work;

Amy Basken; who works for several Congenital Heart Defect (CHD) organizations, serves as ACHA’s Advocacy Director, and coordinated most of our Lobby Day activities;

Molly Nicholson of the American College of Cardiology; for her work in helping this bill become a law and also for her friendship;

Paula Miller, a great friend and fellow heart warrior who accompanied me on two of my legislative visits;

Dr. Mike McConnell, my Cardiologist at Emory University in Atlanta, and my partner on my third legislative visit;

And especially to all 150+ CHDers and Advocates who brought their message to Capitol Hill!