Posts Tagged ‘NIH’

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.

… but there is still a lot to be done

April 13, 2009

In my last post, I wrote about the amazing advances in Congenital Cardiac Surgery and how Heart Defect mortality rates have dropped 38% from 1979 to 1997. But there is still one defect we don’t have a good answer for yet: Hypoplastic Left Heart Syndrome, or HLHS.

Hypoplastic Left Heart Syndrome is not a singular defect, and could have any number of variations. But all of them feature a small (or nonexistent) Left Ventricle and a small Ascending Aorta.

If you look at a cutaway view of a normal heart, you will notice right away that while they are roughly the same size, the Right Ventricle has a larger volume than the Left Ventricle. The Right Ventricle has a smaller pumping muscle: a larger one isn’t necessary because the Right Ventricle only pumps blood to the lungs and back. But the Left Ventricle features a large, thick pumping muscle. When it contracts, the blood is really going places: out into the Aorta, and from there all over the body.

So if the Right Ventricle is content to just drive around the block, the Left Ventricle is at the airport boarding a flight to London. But in a heart with HLHS, the left Ventricle and its pumping muscle are tiny and the Aorta is barely functional. After all, the  root word for hypoplastic means “underdeveloped”. Because of this, a right sided heart defect (like Tricuspid Atresia, which is what I have) is more survivable than a left sided heart defect. 95% of children with HLHS who receive no treatment die within one week.

Even with surgery,  in the mid 1980’s only 28% of HLHS patients survived. (See the 5th Paragraph of the above link.) Until the late 1980’s an HLHS repair involved only two surgeries – The Children’s Hospital of Philadelphia (CHOP) didn’t begin to use the intermediate operation until 1989. (The entire link is informative, but page down to the section labeled “Discussion” for a look at how the three surgery procedure developed.) Current survival rates for the three stage surgical procedure are roughly 75%, with almost no data for long term survival.

This is completely unacceptable.

So what can we do about it?

1) Pass the Congenital Heart Futures Act. The Congenital Heart Futures Act, currently under review by two Congressional committees, will authorize more National Institutes of Health funding for Congenital Heart Defect (CHD) Research. Research is already going on – this January 2008 report from the National Institutes of Health (NIH) states that families with a Bicuspid Aortic Valve in their medical background are more likely to have an infant born with HLHS – but more funding means more and better tools, and more people trying to find a solution.

The Act will also create a CHD Patient Registry, maintained in one location and accessible to physicians. A properly administered registry will assemble a massive amount of data for study. The Centers for Disease Control will also develop educational programs concerning Congenital Heart Defects and their effects on patients and their families.

2) Identify Major Surgical Hubs. You aren’t going to allow a 200 bed community hospital to attempt the three surgery repair needed for HLHS. And it’s not that they are not careful, caring people… they do not have the experience. Identify the large national centers that perform many difficult medical procedures and create regional pipelines that move patients to these hospitals as quickly as possible.

3) Test new surgical theories. Gone are the days when new surgical procedures were developed through the “try it and see” method. With today’s faster computers, surgery can be simulated. A surgeon can “practice” on a computer before performing the actual operation, which give him the chance to anticipate any problems that may occur. By creating a computer simulation of an HLHS heart (multiple variations of HLHS can be programmed in, as can other defects) and using it to test new surgical theories, surgeons can explore “what if…?” theories without actually harming a patient.

4) Create a HLHS-only Registry. As a subset of the National CHD Registry, create a registry dedicated to gathering data only from patients with Hypoplastic Left Heart Syndrome. Small databases already exist and have been valuable in research: for example, researchers have used a HLHS database to  analyze the surgical approaches to HLHS to determine which ones work better. Research and better surgical procedures reduced HLHS deaths in California nearly 50% between 1990 and 2004. But such studies draw on limited databases for their information. Create a national database, and you open even more avenues for study.

5) Determine if HLHS has a genetic or an environmental origin. As noted in the January 2008 NIH report, families with an occurrence of a certain heart defect are more likely to have HLHS occur in the family later. That points to a genetic cause. But there is also evidence of an environmental factor – a “cluster” of twice as many HLHS cases than would be normally expected in a certain section of  Baltimore, Maryland. We need to devote the time and resources needed to determine what exactly causes HLHS: Is it a genetic predisposition?  Or is the environment the trigger? If it is genetic, can we learn how to prevent it? If it is environmental, what is the cause, and can we eliminate it? Or perhaps certain environmental conditions cause the genetic changes that eventually lead to HLHS.

These are just some of the things that we could do to improve Congenital Heart Defect survivability in general, and HLHS survival in particular. Quite often, we have to “think outside the box” to see the problem from an entirely different angle, and then perhaps we could find the answer.

Because every heart deserves to live a lifetime.

The Realities of the Situation

March 22, 2009

This may come as shocking news to some, but not everyone thinks that the Congenital Heart Futures Act is wonderful. They can find no good reason for it.

These guys, for example, think the Congenital Heart Futures act is “egregious”. A complete waste of time and money. And in a way, they have a point. If you are not affected by a Congenital Heart Defect or are not familiar with the costs involved, it very well may not make sense to you. And with the Stimulus plan and the bank bailouts, yet another expenditure for a disease may not seem like the best use of resources.

That’s why you need to be familiar with the facts. Estimates of the number of people living with a Heart Defect in the United States range from 2.1 million (the number I use) to 3.6 million. Two million, one hundred thousand people fighting a chronic disease generate an enormous amount of health costs. These costs fall both on private insurers AND on Medicare/Medicaid… so like it or not, every taxpayer gets to share our medical bills.

And when you consider those 2.1 million people grow an average of 5% a year due to medical advances allowing us to live longer lives, those costs are going nowhere but up.

As a personal example, I take eleven different medications a day. You probably just rocked back in your chair and thought “Eleven? Per DAY?!?!” – but that’s not the highest amount that I know of. I know other CHD Survivors who take more, and some who take less. (Mind you, we aren’t even considering the cost of surgeries and hospitalization, just medication.) But if the research and the registry created by the Congenital Heart Futures Act can eliminate one medication from a Survivor’s daily routine, the cost savings would be significant.

And what’s the cost of the Congenital Heart Futures Act? At Lobby Day 2009, it was estimated that the cost of the bill would be slightly less than twenty million dollars per year. $20,000,000 – a drop in the bucket!

But here is a dirty little secret you never anticipated: We have to fight other heart organizations, too!

According to the Wall Street Journal,

The group, however, wants more federal-research funding to go toward heart diseases, which cause more deaths in the U.S. than any other disease. The patient-advocacy group said heart disease is projected to rise by 16% each decade, and deaths from stroke are expected to double from 2000 to 2032.

The group said it worries that without comprehensive changes, reductions in heart-disease deaths over the past few decades may be erased.

The “group” that wants more federal funding is the Big Red Heart. And the Big Red Heart naturally feels that it is the best group to receive and allocate the extra funds.

The Journal article notes that the National Institutes of Health allocates $25 billion a year for medical research, but only 4% of that goes to heart studies.  So roughly a billion dollars a year, if my math is correct. So the “pie” of federal funding is a billion dollars each year, and if you are at the table when the pie is served, you get a  slice. And who doesn’t love pie?

Well, we aren’t at the table – yet. But since everyone loves pie, nobody is planning to share. “MY pie! Go bake your own!” Well, we would if we could, but this is where the pie is served, and we don’t want that much, anyway. But we’ve got to be at the table with a plate and a fork when the pie is served, or we go hungry.

And that is why your efforts, at the local level, are going to be so important.

Fix the Problem!

November 4, 2008

UPDATE, 6:12 AM Eastern Time: Katie is on my mind again… Preliminary reports suggest that the surgery may not have helped her that much, if at all. Get over to her website ASAP and encourage her parents.


Great news from the National Institutes of Health (NIH) and the Adult Congenital Heart Association (ACHA)… The NIH’s Partners in Research program will fund the ACHA’s first Adult Congenital Heart Defect research study! Titled the “Health Education and Access Research Trial in Adult Congenital Heart Disease”, (HEART-ACHD for short) the study will attempt to learn why those of us with Congenital Heart Defects often “fall through the cracks” after childhood and no longer receive good cardiac care.

This is a two year study, and ten of the largest Adult Congenital Cardiac Care centers are involved. Heart Moms and Heart Dads may not be very interested in this results of this study, but it is more important than you think. Imagine that moving from Pediatric Cardiology to Adult Congenital Cardiac Care is like crossing a river…. but the bridge has a huge hole in the middle of it. Some of us, by luck or design, are able to walk around the edge of the hole without falling in and continue with good heart care. Others stumble, and fall in the river. If we’re lucky, we catch something as we fall and are able to pull ourselves back onto the bridge, while others aren’t so fortunate.

Lifelong cardiac care is so important, especially to someone with a Congenital Heart Defect. As you age, not only do you have the problems you were born with, but also the effects of age began to set in. We’re susceptible to Cholesterol problems and clogged arteries, just as everyone else is – but with our bad hearts, the problem may manifest itself in a different form. So the transition from our “kiddie doctor” to an Adult Physician is critical, and right now there are too many ways to get lost in the shuffle… fall through the hole in the bridge, so to speak. Hopefully this study will give a “repair crew” something to work with as they repair the bridge and make the road to lifelong medical care safe to travel!

Throwing my Readers Some Bonz!

October 5, 2008

This post may be a bit unfocused, as I really don’t have any ideas about what to write about today. My weekend has been full of “normal” activities, if you can call counting small pieces of candy normal. I bought a large glass jar with an airtight lid and a five pound container of Bonz – small (two centimeter long) pieces of candy shaped like little bones. They come in five different colors: red, white, blue, yellow, and orange. For a Halloween festival prize, I’ve sorted out all the orange Bonz, counted them, and filled the jar. Now if some lucky child can correctly guess the number of Bonz in the jar, they win the jar!

A much easier question to answer is “What kind of candy does Steve eat?” At least until the end of the year, the answer is going to be “Bonz!” I’m tempted to buy another airtight jar and use the red, white, and blue Bonz to make a 4th of July “Guess the Number” prize. With a good seal they should stay fresh until then.

As you can see, the life of someone with a severe heart defect can be shockingly boring. And I like it that way! The trick is to learn all you can about your defect, and your body. The second one comes only from experience, and could change at any time. But once you figure yourself out, you’ll have a good idea when something is “just irritating”, “normal for me” or just plain wrong. And then you can deal with it.

I think the first time that I realized that something was wrong with me was in the early 1970’s. I was less than eight years old, maybe less than six. We owned a white station wagon – make and model, I have no idea – and we had some kind of a dinner at the church. It was pretty much over, all of our dishes were loaded, daddy was sitting in the drivers seat, and I was playing in the back. I planned to crawl over the seat, but I slipped and went face first into the metal bar that you pressed if you needed to lower the back seat for more room.

WHAM! I literally saw stars.

Daddy looked back at me. “You’ve busted your lip,” he said. “Come here so I can clean you up.”

He was wiping my face with a handkerchief when I glanced up into the rear view mirror. I couldn’t see the cut (it must not have been bad, because I don’t have a scar) but I could see my bloody mouth.

My blood actually looked blue. Holy cow! Even when you are Cyanotic, you blood isn’t blue… a darker shade of red, perhaps, but not completely blue. But that is what it looked like to me. Remember, I was “knee high to a grasshopper” (Interpretation for non-Southerners: I was really young!) but that is the way I remember it.

I bring this up because I dreamed about that car last night. I dreamed that my brother had wrecked his car and had been given an old white station wagon as a loaner until his car was fixed. He came by the house with it, angry that he had been assigned such an old set of wheels. My mother commented that we used to have a station wagon just like that, way back in the day, and my father said “I actually think that’s it!”All my medication causes me to have some… interesting dreams!

And finally, be sure to check the blogroll for a new resource. I’m calling it “Medical Publications” but it links to, which is part of the National Institutes of Health (NIH). If you receive government funding for your medical research, it must be published on and be accessable to anyone. So go to the homepage, type your search term into the search box, and you’ll be presented with a list of available works on the subject. They will be very technical and full of “med-speak”, but if nothing else, you can see where major research is being done and contact them if you need further assistance!