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.

15,000 people

“Glad to be here, happy to be alive.” – End of the Line, The Traveling Wilburys

15,000 people have registered for the Atlanta Heart Walk! Wow!

And my original plan was to just take a quick flight down to Atlanta. I’m glad I didn’t – not with supicious packages being found on various aircraft today. For those of you who may not know, Atlanta is home of the busiest airport in the world. I can’t imagine how crazy it is out at the airport right now.

Don’t you let my chest be hurtin’!

Another gem from the files of Dr. Wes:

The doctors and staff of Vanderbilt Heart and Vascular Institute in Nashville, Tennessee are a crazy bunch!

The Nashville Heart Walk (sponsored nationally by the American Heart Association) is coming up on October 2nd, and Vanderbilt Heart is the local sponsor. They could have put an ad in the paper, a few radio ads, and even a TV commercial or two. All that has been done before, and it works. Years of advertising have proven that.

But Nooooooo…. the Vanderbilt team created a MUSIC VIDEO!

Give them a break

I heard it again last night!

Every so often I’ll hear (or read on a blog) frustration or anger aimed at the American Heart Association. “The American Heart Association only allocates 2% of its funds (or 3%, or 1%… the number always seems to change) to Congenital Heart Defect research! This is a shame and disgrace!”

Umm… I hate to be the bearer of bad news, but that is not their job. As proof, I offer the Association’s own Mission Statement, copied from their website:

The American Heart Association is a national voluntary health agency whose mission is: “Building healthier lives, free of cardiovascular diseases and stroke.”

In fact, the American Heart Association deserves a pat on the back – even though they normally only work to eliminate cardiovascular disease and stroke, for many years they were the only Heart Advocacy group around. Because of this, their website maintains several good pages about Heart Defects (look HERE and HERE for two examples; there are other pages as well ); they offer a 64 page booklet titled If Your Child has a Congenital Heart Defect, and also has a good webpage for Adults living with a Heart Defect.

The American Heart Association controls the Legacy of Life Endowment, a national campaign to raise one million dollars for Congenital Heart research. Florida also has the American Heart Heroes, an Association program that helps sends kids with Heart Defects to Camp Boggy Creek, a camp for seriously ill children north of Orlando. Broken Hearts of the Big Bend, the great CHD Support group located in Tallahassee, works closely with the American Heart Heroes program. And you can designate a donation to the American Heart Association for CHD causes, with the assurance that is where it will go. Just write “For Congenital Heart Defect causes” or “Legacy of Life Endowment” on the memo line of your check.

The Big Red Heart plays hardball

Let’s take a short break from our usual adventures to take a quick look at what happens when you “break the rules”:

Certainly you’ve noticed that all the major news outlets seem to highlight the same health story at the same time. No, you aren’t crazy – this happens because of a News Embargo.

Here is how it works: A medical journal – lets call it the Journal of Smelly Feet (JSF) – has concluded a major study that shows that socks that are not regularly washed smell 37% worse than the average sock. So the JSF releases this study and its results to the press on a Wednesday afternoon – with the stipulation that this must not be broadcast or appear in print until 8:00 AM Eastern time Monday.

This gives interested journalists (and everyone is interested, the study is a major work) more time to read the research, gather comments from scientists involved in sock care, and write their stories. Monday at 8:00 AM, the embargo is lifted and everyone is free to publicize the story.

Despite this silly example, the embargo does serve a purpose: It allows science and medical reports to become full-fledged “News Events” rather than a few pages in an obscure journal that only a few people will read.

Everything usually works on the honor system, but what happens if someone breaks the embargo? Grabs the bull by the horns and runs with it?

Well, if the offended organization is the American Heart Association… the hammer falls!

The BIG PRESS RELEASE (and it was pretty big, a new study that found that both a stent and a surgical procedure can prevent a stroke) was embargoed until 8:30 AM; but someone at Reuters pressed the button an hour and 43 minutes too early and the story appeared on their website.

Whoops!

It wasn’t long before the jury delivered a guilty verdict and the judge spoke:

As a result of this embargo break, all Reuters reporters will be eliminated from our media distribution list, they will no longer have access to our embargoed newsroom where they can have access to our embargoed journal articles and we will not provide any interviews to any Reuters reporters for a period of 6 months. These sanctions will apply to the reporter who broke the embargo for a period of one year.

Wow! Those AHA folks don’t play.

So, this begs the question – what is Reuters going to do? Well in the “good old days” they could put their science reporters on the street, attending conferences, interviewing experts in the field and developing their own stories. In fact, the Journal of the American Medical Association (JAMA) “disciplined” a reporter who broke an embargo. The reporter contends that she did not break the embargo, she got a lead from a source and worked it into a story. Her “reward” for her hard work was a “Sucks to be you!” note from JAMA.

The problem is reality – with news organizations experiencing a decline in readership and ad sales, staffs have been cut. Most of those science reporters are gone; the few that are left are usually stuck behind their keyboards, writing whatever material they gather through press releases, email, and the fax machine.

So there is not as much research, not as much legwork, and the overall quality of medical and science reporting declines.

Close encounters of the dental kind

It has been a busy week! Things don’t stop when you go out of town… I’m just now getting really caught up from my trip to Durham! I haven’t even unpacked my laptop bag; I need to get everything out and start charging batteries!

But Monday afternoon I had to stop for a while and go see my dentist, to write the final chapter of The Tooth Chronicles*. As usual, I took my antibiotics: The American Heart Association recommends that people with certain Congenital Heart Defects (press release with basic information) take antibiotics an hour before the dental appointment.  (Here’s the full report)

We do this in an effort to avoid Endocarditis. Endocarditis is an inflammation of the lining of the heart, and it usually attacks the heart valves. And since I have had it, I am a firm believer in taking my pills! Endocarditis put me in the hospital for seven weeks, getting IV antibiotics every minute of it. Well, except for a couple of hours…

I was slurring my speech, forgetting things that just happened, and dragging a foot when I walked – and I didn’t realize it. My parents became alarmed, naturally, and mentioned it to the doctor, who looked me over and decided that I needed to have the Neurologist drop by. The Neurologist decided pretty quickly that I needed an MRI.

So I was bundled up, shipped to a different hospital – this was the late 1980’s, there wasn’t an MRI machine on every corner back then – and they ran a scan of my head. Sure enough, there it was: a silver dollar sized cyst inside my brain.

My next stop was the Operating Room. Thankfully it was not very deep and pretty straightforward – the operation started about 3:00 PM and the surgeon made it home in time for dinner. I did come out of the OR looking like Kojak, though.

After that, there was two more weeks of IV antibiotics before I was able to go home. And as often happens with Endocarditis, there was really no way for sure to tell how I had gotten the bacteria. But it can occur when a CHD survivor has dental work, so I am careful to “dose up” before I see the dentist. And it was only later that I found out that Endocarditis can lead to heart valve damage – enough to need surgery to replace the valve – and can even kill you! So if you have to see the dentist, call your cardiologist first, and get a prescription for antibiotics if you need them.

* I have an overbite; over time, my lower teeth have worn down the back of that tooth and finally exposed the root. I got the root canal and went back to my local dentist for the follow-up. He felt that it would be better to bond it rather than insert a crown.

Pulse Oximemetry: Not Recommended

A new scientific statement from the American Heart Association and American Academy of Pediatrics will not recommend using Pulse Oximetery to screen for Congenital Heart Defects (CHDs).

Despite repeated pleas from the CHD community (including a post on the Funky Heart blog) the authors of the scientific statement feel there are too many variables that can affect the test. They are not saying that the test should be abandoned completely; in fact they encourage facilities that are already performing the test to continue – and to compare results.

Pulse Oximetery is a simple, painless test that can be used to detect the presence of a Congenital Heart Defect in a newborn infant – but is it too simple? The Pulse Oximeter can detect low levels of oxygen in the blood, which is called Cyanosis and is a recognized sign of a complex heart defect. But only 25% of heart defects cause Cyanosis. And as simple as it is, the Oximeter can be misused.

If you want an accurate test, you have to standardize the the testing procedure. A child born in a hospital in Denver will naturally have a slightly lower PulseOx reading than a child born in Washington, DC – would we have to have a “Normalized PulseOx reading” for different sections of the country?

What we need is more statistical information about the accuracy of Pulse Oximeter testing, and perhaps an even better screening test. A test that is just as fast and just as inexpensive.

That’s one of the reasons that ongoing CHD research is so important.

More on the Tooth

My dentist normally takes Fridays off, so I had to wait until this morning to see him. Thankfully my local Primary Care Physician (PCP) was prescribed some pain medication, so I had a peaceful weekend. My Cardiologists in Atlanta also prescribed the antibiotics for my dental visit. (It is VERY IMPORTANT for certain CHD patients to take antibiotics before a dental appointment; click HERE to read the American Heart Association’s guidelines. Antibiotics before dental appointments can help you avoid Endocarditis!)

My dentist thinks that the tooth can be saved via Root Canal… Sort of scary to a Funky Heart with a fear of dentists and their power tools, but it has to be done. I have an appointment with a dentist who is a “root canal specialist” Thursday morning. It’s listed as a “consultation”, but I am hopeful that this dentist will just say something like “We may as well get started….” because I am ready for this problem to go away!

Getting there

Every six months or so I feel the need to mention this information again: Perhaps there are readers of Funky Heart who are new to the Congenital Heart Defect world and they don’t have their bearings yet. All of this came crashing down on them like a ton of bricks. In a few short weeks they’ve learned an entirely new language, met doctors they didn’t expect to meet, and learned what the phrase right now truly means. And you wonder if this madness will ever end.

The short answer is no. Your child will need specialized medical care all of his life. But we’re getting better at it. Today, 90% of all children who have open heart surgery survive to become adults. 75% of all children with Hypoplastic Left Heart Syndrome (HLHS) survive the three surgery procedure needed to live. That’s too low to be acceptable, but in 1980 there was no surgical option at all and the survival rate was close to 0% – a lucky few were able to get a heart transplant.

To educate yourself about your child’s heart defect, go to THIS LINK; it is excellent. The American Heart Association has some good information, and THIS ONE is probably the best.

If your Cardiac Kid is in his/her teens, it’s time to be thinking about moving them to Adult Cardiac Care. You can’t just pick an adult cardiologist out of a phone book, they aren’t trained to deal with patients who have CHD’s. You need someone who is trained to deal with Congenital Heart Defects but can see it from an adult perspective.  There aren’t that many of them (yet), so you need to go here and read the patient summary of the BETHESDA 32 REPORT. At the bottom of the page is a link to download the entire report as a .pdf file, you need to download it, print it (have plenty of paper for your printer; it is long) and read it carefully. It will give you an outline about how to move your child from a Pediatric Cardiologist to Adult Care. Also of interest is THIS REPORT (in .pdf format) from the National Heart, Lung, and Blood Institute (NHBLI). It’s only 9 pages long; print it and read it.

The ultimate goal is to enable Congenital Heart Defect survivors to live longer, better, and more fulfilling lives. Like the adults in this video produced by the Adult Congenital Heart Association (ACHA):

New Guidelines for Heart Warriors

There are new guidelines out for Adults born with Congenital Heart Defects. Written as a collaborative effort between the American Heart Association and the American College of Cardiology, the guidelines were published online just last week, and honestly I haven’t had a chance to read them yet.

Even without reading the guidelines, I’m encouraged by the fact that at least three of the writers are closely associated with the Adult Congenital Heart Association (ACHA) and are highly respected in the field of Adult Congenital Cardiology. Dr. Carole Warnes is a Professor of Medicine at The Mayo Clinic in Rochester, Minnesota; Dr. Michael Landzberg is director of the Boston Area Congenital Heart (BACH) and Pulmonary Hypertension Services; and Dr. Gary Webb is director of the Philadelphia Adult Congenital Heart Center. There may even be more ACHA – associated doctors among the writers that I’m just not familiar with; if I have missed someone, it’s unintentional and I apologize.

The guidelines are available online by clicking HERE. The overall readability is good –  there is a limited amount of “doctor-speak”, and important points are in plain English. The website says that they can be downloaded as a .pdf file. It would probably be best to download the document and read it on your computer rather than print it. The .pdf is 124 pages long!

Heart Moms and Heart Dads, you may also want to read the guidelines. After all, ninety percent (90%) of children born with a heart defect today will survive to adulthood. Even if your Cardiac Kid is still very young, you can learn a lot about the current thinking concerning Congenital Heart Defects and their treatment. In the online version, many of the footnotes (and there are over 500!) refer you to additional material that is also available online.