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.

Your Choice (My Answer)

Yesterday I asked Funky Heart! readers the question Given a choice, would you devise a cure for Congenital Heart Defects, or prevent them? To make it a little bit more difficult I added a stipulation: Your choice would have no effect on the other group – developing a cure will not prevent new cases from occurring; preventing heart defects will not cure those who already have them.

The question generated a lot of debate and discussion (as I hoped it would). But what would I choose to do? The answer might surprise you: I’d choose to prevent Congenital Heart Defects.

Preventing all future heart defects would isolate those of us who already have them… the need for Pediatric Cardiologists would drop as would the need for surgeons with CHD repair skills. You can assume there would be the need for a number of surgeons as CHDers could easily need heart operations later in life, but perhaps not as many as before. The number of Cardiologists would also decline, slowly – there would be a need to serve the current number of patients but that would drop as the patients begin to age and no new cases occur.

Older Heart Warriors would have to increase their efforts to inform and educate younger patients and their families. Not being boastful, but this would be the perfect job for an organization that I am a member of, the Adult Congenital Heart Association (ACHA). We’re already working toward a similar goal: adolescents with a heart defect tend to fall away from good cardiac care as they reach adulthood. They no longer visit their Pediatric Cardiologist; nor have they found an Adult Congenital Cardiologist. A good percentage of them may not even realize that they need an Adult Congenital Cardiologist.

The ACHA is working to prevent this from happening, to makes sure CHDers get good care all of their lives. Currently the Bethesda 32  report outlines care guidelines for the Adult Congenital Heart Defect patient and the ACHA’s Vision 2020 Project is a program designed to provide a seamless transition path – from Pediatric Cardiologist to well-trained, competent Adult Congenital Care Cardiologist without a break in care.(Bethesda 32 may be a name you are not familiar with. Every year, experts from the American College of Cardiology meet in Bethesda, Maryland to devise proper care guidelines for a segment of cardiology. The 32nd time they met (the year 2000), they devised guidelines for the care of adults born with a Congenital Heart Defect. You may not be familiar with the name, but the Bethesda 32 Report is very important to the CHD Community. You can read a good summary by CLICKING HERE.)

In the fictitious world we’ve created – one in which all new CHDs can be prevented – older Heart Warriors would have to step forward. We’re the voice of experience, we’ve literally been there and done that. And in this brave new world, we’re a lot more important. Now, we’re going to have to be teachers – teaching the younger generation how to take care of themselves, what to do and what to avoid. And we’re going to have to help each other out, too… the number of Adult Congenital Cardiologists will also begin to drop. After all, in this world where CHDs can be prevented, why specialize in a field that in time, will no longer exist?

This will cause problems, but I believe they can be overcome. We’re all in this fight together, and as Benjamin Franklin said, “We must all hang together or surely we will hang separately.”

A Cure for the Funky Heart?!?!

I’ve got Google Alerts searching for the appearance of certain Heart Defect words and phrases across the internet, and they deliver new information to my computer every night. The information is new to me, but not always new.

So imagine my surprise when I read this report from a 1981 edition of the Scandinavian Cardiovascular Journal. Obviously, the internet didn’t exist in 1981 (or exist in the form that we know today) so this couldn’t have been put on line then; apparently another organization recently uploaded it – and Google Alerts “hit” on the phrase Tricuspid Atresia.

In eight patients from 1976 until 1980, tricuspid atresia (TA) was corrected with valved xenograft conduits…

SAY WHAT?!?!?

This is unreal – in this trial, eight patients with Tricuspid Atresia were given a conduit that ran from the Right Atrium to the Pulmonary Artery, or a conduit that connected the Right Atrium to the Right Ventricle and “jumped” the missing Tricuspid Valve. This is the first time I have heard of this… but it sounds as if it might work.

All patients suffered from transient right-heart failure postoperatively and eventually developed normalized cardiac function throughout the first two months after operation.

Holy cow, it did work! All eight went through a passing phase of right side heart failure that quickly stabilized, and in two months their hearts began to function normally!

X-ray examination showed normalization of the heart size in the majority of the patients, and in those with conduits between the right atrium and the right ventricle a considerable enlargement of the right ventricular chamber together with normalization of right ventricular contractility had developed. Arterial oxygen saturation, haemoglobin and haematocrit values had normalized in all patients.

“Normalization” is a word I like to read – especially when pertaining to Heart Defect, and for damn sure when it’s MY defect! There was one adverse outcome – one patient died of “intractable right ventricular failure, septicaemia and intravascular coagulation” – in layman’s terms,  there was blood poisoning, runaway blood clotting and the Right Ventricle failed for an undetermined reason.

Two patients with valved conduits between the right atrium and the right ventricle showed a normal unrestricted level of activity without medication, while patients with valved conduits between the right atrium and the main pulmonary artery were digitalized with an almost normal level of activity. Early repair with valved conduits of patients with TA is advocated.

But despite the success of this very limited trial, I can not find any information about followup trials. Today’s Tricuspid Atresia patients do not have this treatment option. Which begs the question:

What happened?

The obvious answer to why this isn’t being used today is “Something went wrong.” – but what? I’d certainly like to know. With 2009 technology, this trial could very well be worth repeating.

What’s the goal?

Duncan Cross is at it again; this time he’s got a huge bone to pick with the Crohn’s and Colitis Foundation of America (CCFA).  The nation’s health care system is being renovated, everything is on the table, and practically everyone in Washington DC is putting their two cents in. The only group not fighting to get into the debate is the International Association of Airship Navigators.

So what’s Duncan upset about? The CCFA wants their members to support a Senate bill that will promote research and establish awareness programs.  So while everyone else is redefining health care as we know it, the CCFA apparently wants taxpayers to pay for those little  purple rubber “awareness bracelets”!

No wonder Duncan is upset. He’s got another post worth mentioning about advocacy organizations that funnel all their time and effort (and fund raising) into finding a cure.

For a lot of Chronic Illnesses, a cure may be a long time off. I don’t think there will ever be a “cure” for Congenital Heart Defects (CHD’s); Wikipedia lists 23 separate defects and also states that the list is incomplete. (When I count them up I usually get 35 CHDs, not counting any “blends” – one defect superimposed on top of another.)

So while all these advocacy groups are running around shouting “Donate to us! Help us find a cure! We need your help to find a cure!” at the top of their lungs, a lot of them are forgetting one of the most important things they could do:

Helping those who suffer from that illness to live their lives to the fullest.

That’s one thing I like so much about my group, the Adult Congenital heart Association (ACHA). Yes, we’re all about the technology and the research, but our mission statement makes it clear that one of our goals is “to improve the quality of life and extend the lives of congenital heart defect survivors.” And that’s right there on our website’s front page for everyone to see. Eliza’s doing her part to keep us all going by organizing the ACHA Bolder Boulder team. The national office is promoting our “Don’t Get Lost” program to return CHDers to care who were told that they were “fixed” (a common occurrence). They have also recently developed Vision 2020, our initiative to make sure that everyone with a CHD has good health care – from their Pediatric Cardiologists through the transition to an Adult CHD doctor. We’re not just looking for a cure – we recognize that part of the “cure” is 1) better medical care and 2) doing your part to take care of yourself.

So take a good close look at your support group. What are their goals? What are they doing? Trying to find a cure is great, and hopefully one day someone will figure it out. But until then, is your group trying to help you make it day to day?

If not… it might be time to look somewhere else.

Good Guys wear Scrubs!

To paraphrase Samuel L. Jackson’s classic line from Snakes on a Plane (and clean up his language, children read this blog!) “Enough is enough! I have had it with Heart Defects in our kids!”

But don’t worry.

The good guys are coming. And they are bringing lots of weapons with them.

A conference held in July brought a group of medical experts together to study new and improved methods of Interventional Cardiology to repair Congenital Heart Defects. Interventional Cardiology is not heart surgery, but rather entering the body through a one inch incision and performing a cardiac repair with miniature surgical tools mounted on the end of a catheter. Typically after a catheter the patient is home in a few days and returns to active life in less than two weeks. These doctors are currently discussing and testing heart valve replacement by catheter rather than open heart surgery. You want heroes, I got 800 of them in Vegas… and they ain’t there to play the tables.

Meanwhile, researchers in Pittsburgh are studying Zebrafish to find the gene that causes Aortic Arch malformations. They’re studying Zebrafish in other ways, too. Not because he’s cute, but because a Zebrafish can regenerate his eyes, his spine, his fins, and his heart.  That crazy little fish can practically rebuild himself! Both Duke and Children’s Hospital Boston have studied the Zebrafish.

The researchers at the Medical College of Wisconsin have discovered that mammals born with defective hearts are missing two genes. Who knows where this research will take us? A pill or an injection that can prevent heart defects?

And starting tomorrow morning (September 8, 2008) there is going to be a conference at Indiana University dealing with the subject “Heart Failure in the Young”. But don’t let the title fool you: They don’t plan to just address the issue, they plan to attack Heart Failure in the Young! They are going to study how to repair heart tissue, how to stop Heart Failure before it starts, and development at the fetal level that could lead to Heart Failure. And they are serious about this: The National Heart, Lung and Blood Institute granted $11.5 million dollars towards studying Children’s Heart Failure. Smart docs, an aggressive attitude, a major facility, and big bucks… and it’s all happening Back Home Again in Indiana!

We’ll beat Congenital Heart Defects yet!

What if there was a cure?

What if…

What if a CHD could be cured? Not “repaired” surgically, but completely and totally cured. That is one of the long range promises of Stem Cells.

You’ve heard about Stem Cells, I’m sure. You’ve certainly heard the great debate, mainly centered on Embryonic Stem Cells. An Embryonic Stem Cell, it is believed, has the potential to cure almost anything.

But where do we get those Embryonic Stem Cells? From stillborn babies? Certainly. But what if there aren’t enough Embryonic Stem Cells available from that source? Do we clone? Do we (gulp) collect eggs, fertilize, remove the stem cells and then abort later? Each answered question only seems to generate more questions.

I’m not trying to get political here. It’s not my intention to make CHDs a Conservative/Liberal, Democrat or Republican debate. By definition, a Congenital Heart Defect manifests itself before you are born, long before you’ve decided on your political affiliation. This post is my answer to a question someone asked me about a month ago — Wouldn’t you like to be “normal”? Since I don’t THINK they weren’t questioning my mental facilities, I assume they were asking if I would like to be heart healthy.

Who wouldn’t? And there is really only one or two ways to make that happen: Transplant or Stem Cells. Since a transplant obligates you to a lifetime of high powered anti-rejection drugs, I’m going to eliminate that as a “cure”.

And here’s a surprise for you: Using Adult Stem Cells, we’re halfway there already. This study showed that Adult Stem Cells taken from bone marrow can be injected into the heart. Heart function improves and none of the study group showed any ill effects. Another study done in England was also to test growth factor drugs to make the body produce more stem cells, rather than removing them from the bone marrow.

Amazing stuff. If I understand the procedure correctly, the test subject — in this case, me — is taken down to the Operating Room and some of my bone marrow would be removed. The marrow would be spun in a centrifuge, the stem cells separated and drawn off. Three days later, we go back to the OR. My heart is stopped (I am not sure why the heart has to be stopped, but it seems that it has to), the stem cells injected into my heart, and then the heart is restarted by a defibrillator. Within 72 hours my heart would be growing new heart muscle.

Right now this is being tested to improve heart function for those of us with Congenstive Heart Failure. It’s not a cure– yet. But no one though you could successfully correct a CHD through a surgical procedure. Alfred Blalock, Helen Taussig, and Viven Thomas proved them wrong.

If there was a cure for Tricuspid Atresia, would I take it? Probably not. If I hadn’t been born with a heart defect, I would never had met the great doctors and nurses I now count among my friends. I would have never developed the personality that I have. I’m sure it rubs people the wrong way sometimes, but it must be pretty good: no one has beat me up since grade school! And I would have never met the great people who make up the Adult Congenital Heart Association.

A cure? Wonderful! But as for me, I am who I am.