Archive for the ‘Registry’ Category

A Cure for Heart Defects!

November 26, 2010

“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.

REACH for the goal!

August 12, 2010

Great news: The Adult Congenital Heart Association (ACHA) announced today that the Research Empowerment in Adult Congenital Heart Disease (REACH)  initiative would receive a one million dollar federal grant! The money will be used to activate CONGENERATE – The Congenital Evaluation, Reporting, and Tracking Endeavor. CONGENRATE is an Electronic Health Record (EHR) designed for Adult Congenital Heart Defect Survivors!

REACH is a joint project between the ACHA and the Alliance for Adult Research in Congenital Cardiology. The two groups are already working together on HEART-ACHD, a two-year study of why adult CHDers “fall through the cracks” and  get away from good Cardiological care. And we aren’t in this alone: CONGENERATE will be tested at Oregon Health Sciences University, Nationwide Children’s Hospital, Cincinnati Children’s Hospital Medical Center, Children’s Hospital Boston/Brigham and Women’s Hospital, and Children’s Hospital of Wisconsin.

Whenever I travel, I take a large folder with me that contains basic health information, my diagnosis, the names of my doctors (Including my team of Cardiologists in Atlanta and that I would rather they be the first doctors contacted) EKGs, and a diagram of my heart. It comes in handy – it was with me when I went to the Emergency Room in Houston, and I have a smaller version in my back pocket when I leave the house.

If CONGENERATE had began five years ago, I wouldn’t need my folder. I’d have an ID card or an access number that could have been entered into a computer and everything you need to know about me would appear on a computer screen! CONGENERATE should be ready to roll out sometime in 2012…. and if it is a success, perhaps the next project would be a similar application for Cardiac Kids!

ACHA’s vision for every CHDer is “a life unlimited by congenital heart disease.”  Projects like CONGENERATE are small baby steps in that direction.

Clinical Trial stopped!

June 2, 2010

Pfizer has decided to stop the recruitment of patients into the EMPHASIS-HF drug trial early. That headline should raise some eyebrows, since it means one of two possible outcomes: Early results are either very good or very, very bad.

Thankfully, the results seem to be very good! According to the press release (a 5 page .pdf file) the study reached its “primary efficacy endpoint” early – in other words, the investigators proved what they were looking for. Taking Eplerenone (also known as Inspra) in conjunction with recommended Heart Failure standards of care, lowers incidence of hospitalization and/or death in patients with mild to moderate heart failure. The adverse events (things that went wrong) included a higher level of potassium in 8% of the patients taking Eplerenone and 4% had renal impairment. Renal impairment means that their kidneys stopped functioning, but that condition can occur because of heart failure also. Apparently the incidence of Renal impairment caused by the new drug is not much different from the number of kidney problems cause just by having heart failure. The Clinical Trial was a double-blind test, which lends it even more credibility, as neither the subject or the investigator knew which drug was being used – the Eplerenone or a harmless placebo. When carried out correctly, a double-blind test prevents either the subject or the investigator from being biased.

So what happens now? Don’t expect to be getting a prescription for Eplerenone, the company does not have a license to sell to human subjects. The study was a small one – only 3100 people, if it had reached full enrollment. All patients, no matter which drug they are on, will be informed of the decision to close the study early. Pfizer has asked for permission to move all consenting patients into a larger study of the drug.

Sometimes medical research comes in small increments – baby steps. But you have to walk before you can run!

Five CHD Questions: Why?

May 13, 2010

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;

Lobby Day 2010: Let’s win this…

April 22, 2010

“Let’s win this for everyone who never had a chance to get here.”Hoosiers, 1986

(LIVE UPDATES – REFRESH THIS PAGE OFTEN!)

6:26 AM: Good Morning from Lobby Day 2010, from our home base here at the Phoenix Park Hotel! I have actually been here since 5:45, but unable to get online for some reason. Then suddenly, the internet connection worked and we are in business!

I plan to liveblog throughout the day, giving updates as events warrant. If you are getting this through Facebook or through an email subscription, it might be better for you actually head over to the Funky Heart website (https://tricuspid.wordpress.com) and follow us LIVE!

The quote above is from the movie Hoosiers, about a very small (64 Students) Indiana high school Basketball Team that makes it to the state finals. As the quote says, let’s win this for everyone who never had a chance to get here – The child in the ICU at Johns Hopkins who passed away the night I was admitted in 1967. Karen McNaulty, the driving force who formed the ACHA. and my friend Jim Wong, who was here for Lobby Day 2006, 2007, and 2009, but who passed away due to Cancer earlier this year. Jim, we’ll do our best to make you proud, my friend.

6:42 am: Pace is picking up, states are being assigned to tables in ball room. SC crew will share table with Iowa, Tennessee, and Oregon. I asked for SC to be put here because I FINALLY got an internet connection and it might be bad luck to move! (No, I’m not a bit superstitious! Not at all!)

I don’t know when my lobbying assignments will take place, so I might disappear for a stretch of time. And if the internet goes out again, I might be out of luck  but we’ll deal with problems as they occur!

6:55 AM: Time for breakfast!

7:20 AM: Training session is beginning! We’re reviewing the “ask” – an “ask” is just that – what we want our legislator to do. The Congenital Heart Futures Act is law, but only words on paper until money is allocated. So the thought for today is a familiar line from the movie Jerry McGwire: “SHOW ME THE MONEY!”

Our Ask is 7.25 million dollars. 3.75 million for the Congenital Heart Defect Surveillance System, 3.5 million for pediatric CHD research. (Surveillance system for Pediatrics already exists, but does not include CHDs.)

The National Institutes of Health (NIH) does not like for Congress to say “Spend XXXX on this type of research.” They are top notch scientific minds, everything is tested and evidence based. NIH would rather be allocated total amount of funds with recommendations on how/where to spend it. They’re very good at sorting through data and finding what needs a higher level of funding.

Taking a moment to remember the warrior who are no longer with us…. and to make this a celebration of them. In the words of Paul Cardall, “Let’s Celebrate Life!”

8:09 AM: Got my Schedule: John Spratt at 10 AM, Lindsey Graham at 2:30, Jim DeMint at 4. Liveblogging is going to be a bit of a challenge this afternoon, but we’ll work it out!

I’ll have to leave about 9 to get to my meeting, need to clear up a few things before then. May be a while before I update again.

8:31 AM: Here we go! Back in a while!

8::41 AM: Slight Delay, but all good. Almost ready to catch the shuttle to the Hill.

11:07 AM: Just back from a meeting in the offices Representative John Spratt (D-SC)! His staff are well informed and seem to be on board with CHD research funding! I think he is with us!

11:41 AM: It is quiet right now, as most of the lobby teams are out. I’ve filled out my Visit Report and written a thank you note to the young lady we spoke with. Lobbying isn’t just about walking in and saying what you want – that happens all day, every day. It’s too easy to get lost in the crowd. Lobbying is really about building relationships, and a thank you card goes a long way. After all – how many people send thank you cards there days? You gotta stay in the front of people’s minds!

1:01 PM: Off on another visit, on the Senate Side this time!

4:41 PM: Had a meeting with the staff of Senator Lindsey Graham. Excellent meeting! Mr. Colin Allen remembered me from two years ago wand was very receptive to our request! He understands where we are coming from, and why we need more survellence and research. I think we can count Senator Graham as a WIN!

I hung around in a Senate office building cafe rather than return to the hotel and then have to go back, and later had a good meeting with Senator Jim DeMint’s staffer. This young man just started – he doesn’t even have business cards yet. but he warmed to the subject and before long, he had a good understanding of what we were looking for. Of course, we left our Ask Sheet and in a few moments I’ll write the visit reports.

I was happy to have my Cardiologist, Dr. Mike McConnell, with me during the visit to DeMint’s office!

5;57 pm: I’m going to close the liveblogging now, it has been a great day, I think a lot of progress has been made! Tomorrow, ACHA is presenting the Vision 2020 forum – changing the way Adult Congenital Care is done. Read tomorrow for a look at the future of CHD care !

Make it Easy!

April 21, 2010

Sung to the tune of Take it Easy, by The Eagles:

Well, I’m a standing on a corner in Washington, DC
Such a fine sight to see!
Hundreds of people showing up to lobby
Most of us with a CHD!
Come on Congress, don’t hold up progress;
We’re gonna find out today who is with us.
We can’t lose, but if we don’t win
we’ll keep coming back again and again.
So clear your schedule we’re coming in,
let’s make this easy!

30 seconds

April 17, 2010

“…when you play at this level there’s no ordinary venue.”

One Night in Bangkok, from the musical Chess, 1986

Legislative meetings can occur in an instant. I don’t mean just not lasting very long, but quicker than you can imagine. You almost have to be talking – and making your point – as you walk in the door because there is no guarantee how much time you may have.

Is this rapid pace fair? Probably not – but it is the way things are done, so if you plan to plead your case to your legislator, you had better learn how Congress functions. Your objectives are to 1) to make sure the Legislator knows that you are a constituent – Members of the House of Representatives are especially interested in hearing the concerns of the people they represent, since they are up for election every two years; 2) make an impression by telling your story; 3) make sure the Legislator knows what he can do for you (vote on legislation, fund a project, etc.); and 4) leave some resource material behind that they can refer to later. And because you don’t really know how much time you will have, you need to be prepared to do all this in 30 seconds.

Could you do it?

My 30 second story is below. I’ve moved it further down on the page so you can think about your own story, and then compare yours to mine.

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My name is Funky Heart, and I live in Funkytown, South Carolina. I was
born with a Congenital Heart Defect back in 1966 – I survived only
because I have great doctors and parents who would turn the world
upside down to give me a chance to live.

CDC’s new National Congenital Heart Surveillance System will compile
data on heart defects for researchers to sift through. Hopefully this
will lead to longer, better lives for those of us who have a heart
defect. All it needs is for Congress to fund it.

Everyone needs a hero – here’s your chance to actually be a hero: Fund the new Congenital Heart Defect initiatives.

Invest in a Cardiac Kid’s Future

March 31, 2010

You’ve seen the TV ad in which the guy says “You can pay me now or you can pay me later!” The implication is that you can pay a reasonable amount now, to protect your car, or pay a lot more later, when the car breaks down and needs major repairs.

That’s the theory behind the push for funding for the Congenital Heart Futures Act (CHFA). As mentioned yesterday, Section 425 (C) authorized the provisions of the CHFA, but does not fund it.To put it bluntly, we need some cash.

During the Lobby Day 2009 event we looked for sponsors for the Congenital Heart Futures Act and asked for twelve million dollars annually to fund it. Let’s assume that this year, our goal is fifteen million dollars per year until the Act expires in 2015 – a total of seventy-five million dollars.

$75,000,000 – that’s a lot of money.

But consider the cost of Congenital Heart Defect care. A 1994 study estimated that the cost per CHD patient under 21 years old varied from $47,500 to $73,600. And remember, that study is 16 years old! The Utah Birth Defect Network estimated a lifetime cost of $1.2 billion dollars for Congenital Cardiac care – and they were only looking at children born with certain defects ( Tetralogy of Fallot, Transposition of the Great Arteries, Single Ventricle Defects, and Truncus Arteriosus) during the study year. And a 2008 study estimates that the costs of hospitalization for adults with Congenital Heart Defects to be 3.1 billion dollars.

So let’s fund the Congenital Heart Futures Act. Activate the National Congenital Heart Disease Surveillance System. Expand CHD research. (Don’t let anyone tell you that there isn’t any Congenital Heart Defect research going on; there is. Click HERE for a list of current Clinical Trials or HERE for the U.S. National Library of Medicine. Just type “Congenital Heart Disease” in the search box).

Funding the Act could lead to improvements in all our lives, but especially for the Cardiac Kids. If we can make surgery a little easier to deal with, replace a surgery with a Catheterization procedure, or reduce the number of medications we have to take, funding the Congenital Heart Futures Act won’t be an expenditure – it will be an investment!

$75,000,000 vs. $3,100,000,000.

You can pay me now, or you can pay me later.

Section 425(C)

March 31, 2010

Here’s the text of the Congenital Heart Disease (CHD) provisions in the Patient Protection and Affordable Care Act (H.R. 3590). This link leads you to THOMAS, the website maintained by the Library of Congress that has the most current legislative information available. It is the actual text of the new law, not someone’s interpretation of What It All Means. The three sections that affect CHDers are Section 10411, Section 399-V2, and Section 425.

The new law will create a Congenital Heart Disease (I usually say “Defect”) Surveillance System – this is the “Registry” that the Adult Congenital Heart Association (ACHA) has campaigned for, including Lobby Day efforts in 2006 and 2007 before joining forces with seven other CHD groups as part of the National Congenital Heart Coalition for Lobby Day 2009. It also gives the Director of the National Institutes of Health (NIH) the power to “expand, intensify, and coordinate research and related activities of the Institute with respect to congenital heart disease…” including the causes of CHD, eliminating barriers to life long care,  and studying long-term outcomes of those with Congenital Heart Disease.

But the most important clause in the Congenital Heart Futures Act is Section 425 (C). Only 32 words long and making up less than two complete lines of text, everything hinges on 425(C):

Authorization of Appropriations- There are authorized to be appropriated to carry out the amendments made by this section such sums as may be necessary for each of fiscal years 2011 through 2015.

In other words, the Act is authorized but not yet funded. Why are we going to travel to Washington and hold yet another Lobby Day event when the bill has already been passed and is now law? Because of Section 425(C).

And that’s why we need you to join us in Washington on April 22, 2010 as we work to secure the funding that the Congenital Heart Futures Act needs to come to life. Right now, it’s just words on paper – you can help turn it into a reality! Register to attend Lobby Day 2010 and help to defeat Congenital Heart Defects! (ACHA is maintaining the registration link on their website; registering does not obligate you to join the group… but we would be happy to have you if you did!)

And we’ll have to go back next year. And the year after that. And we’ll have to come up with new legislation to take effect after 2015, when the Act expires and everything grinds to a halt.

But I’m not worried; we’ve all been here before. It takes a lot of patience and the ability to focus on the long term goal to live with a bad heart, or to be the parent of a Cardiac Kid.

We’ve got the stamina… and we’re in it to win it!

A Moral Obligation: Amy Verstappen, CC-TGA

March 21, 2010

NOTE: It’s been a busy weekend for me, I’ve been dealing with some personal issues. It is not health related; but it has been time consuming… so much that I haven’t really had time to write for the blog. So here’s a blast from the past dated October 2008:

Born with Congenitally Corrected Transposition of the Great Arteries, Amy Verstappen had a normal childhood. Although she had “a bizarre heart murmur and a weird EKG,” her life was on track until she decided to have a child of her own. “In my sixth month (of pregnancy) I had lots of shortness of breath, edema, and my Nurse-Midwife says to me `You’re looking funny and we’re worried; we think you need to go back to your cardiologist.’” The cardiologist dismissed their concerns. “`They’re just Nurse-Midwives, what do they know about the heart?’”

Amy successfully gave birth to her daughter, Lena, and was contemplating pregnancy again two years later. “Before my pregnancy I had very pristine heart function. Two years after my pregnancy, I was planning another child but I was very tired. I had been tired a long time but I had a little baby.”

Stunned to learn that her heart function had “really gone south,” Amy was also shocked to be told that she needed a heart transplant. When she asked questions about her condition, the answer was “Mrs. Verstappen, you are in denial.”

After searching for answers, Amy found a clinic where the doctors were familiar with Adult Congenital Heart Defects and were capable of giving her a correct diagnosis. The news was good: She did not need a transplant, nor was her heart enlarged. Her reversed Ventricles merely created that illusion. But one of her valves was wearing out, and she would need to have it replaced. “Instead of a transplant I had a valve repair,” Amy says. “Luckily my heart function really snapped back, and I have pretty much normal exercise function.”

Afterward, one of her doctors not so gently told Amy that was time for her to make a difference:  “After I had my surgery, (Mayo Clinic Cardiologist) Dr. Carole Warnes looked at me and said `You are incredibly lucky. Very few people with your anatomy actually get to have surgery and you have a moral obligation to speak out on behalf of them.’”

Amy’s recent close call – and that moral obligation – led her to a fledgling organization known as the Adult Congenital Heart Association (ACHA). Joining the Board of Directors in 2000, Amy stepped off the Board in 2005 to become the Association’s first full time paid President.

In the past year, The Adult Congenital Heart Association has begun to transition from only dealing with adult issues to actively working for both adults and children with heart defects. The ACHA has recently joined with several other Congenital Heart Defect organizations to form the National Congenital Heart Coalition. (NCHC)

“I’m very excited about the new legislation that we are drafting,” Amy says. Her face becomes more animated as she speaks, her passion for better Congenital Heart care reflected in every word. “We’re actively writing a bill that we hope to have turned into law. The working title right now is the Congenital Heart Futures Act. It will say – very strongly – that Congenital Heart Disease is a lifelong disease, and this country needs to make a commitment to treating people over their lifetime, not just in childhood.”

“I am 100% positive that in the long run we will have an integrated, high quality care system for all survivors,” Amy says as she looks toward the future. “Our really long term goal is to have a very unified system with research, a registry, a way to quantify outcomes, all the centers talking to each other, sharing information and building knowledge together… that’s the long term goal and I am very positive we’ll get there.”

“You’ll see the ACHA working more and more with the Pediatric community. Remember that ACHA doesn’t just involve patients and families but also health care professionals. We’re really working to get the Pediatric Cardiologists together more with the Adult Congenital Heart Defect (ACHD) doctors and clinics because we still have a huge problem in this country in that we’re not getting good transitional care.”

“We designed a new brochure for parents, very specifically talking about what kind of care their children would need,” Amy recalls. “And when I presented a pilot version of this brochure to a group of Heart Moms – all leaders of some of the advocacy groups for children – three of them told me that no one had ever told them that their child was going to need this level of care. So what I would say to Heart Moms is that your kids can do great, they can thrive, you can have a high quality, excellent, full life…but the second thing I would say is parents should talk very specifically with their Pediatric Cardiologist and ask them hard questions. How often do you expect my child to see a Cardiologist? What kind of doctors do you think they might need? We don’t know all the answers right now but there are guidelines, like the Bethesda 32 report. The bottom line is anybody who has anything complicated, has to go at least every two years and be seen at a highly specialized Congenital Heart Center. And that includes those children with a complete repair.”

Amy turns quiet as she recalls an all too familiar event: “One of the saddest things about my job is to find someone who says to me, `By the time I got back to good heart care it was too late (for me).”