Archive for the ‘VSD’ Category

Hearts on Ice

June 17, 2010

I’ve written before that I have had a bout with Endocarditis. That’s when I was introduced to the cooling blanket. Trust me, you don’t wanna meet the cooling blanket.

I had been feeling run down for several days but just couldn’t put my finger on it. It had been really busy lately and I figured I was just tired. It had been go, go go! for several days and my thinking was that my gas tank was just empty. But a quick glance in a mirror scared me – I was a lot bluer that I normally I. And I mean a lot bluer – my entire face seemed to be grey.

Down at the hospital they my temperature was still normal, so they did all the usual blood work. The tests came back funny – something was going on, but they weren’t sure what. So I’d be staying for a little while. It took them almost two days to figure things out, and only after they called in the state’s top Infective Disease doctor. I’ve never been easy to figure out – even he admitted I was “quite a challenge.” But he figured out that I had Endocarditis. And the next night, I spiked a fever.

Fevers (not just for me, but in general) seem to hit at night, I do not know why. The first and best clue that I am getting a fever is I suddenly get cold, and that is what happened this time. They ran some blood work and took my temperature. The temp must have really been something – it seemed everyone took a half step backward when they saw the readout. That’s when the doctor said “Let’s get this young man a cooling blanket.” A moment later the nurse came in with a large blanket and they kicked me out of bed long enough to set it up. It looked liked your standard electric blanket – but they did hook it into a fairly large box they placed at the foot of my bed.

Turned out that a cooling blanket has little tubes in it, and these tubes connect to a circulating pump (the box) that circulates very cold water through it. After they got the cooling blanket going, they turned the air conditioner on wide open and gave me orders not to get out of bed. Ok, I thought, this thing is going to chill me down a little. Just got to tough it out.

The only – and I do mean only – reason they call it a blanket is to fool the patient! It just kept getting colder and colder, and the air conditioner wasn’t helping. I didn’t sleep very much that night, obviously!

Cold has been used medically for quite some time, since lowering the body temperature also slows body functions. Every few years we hear a news story about someone who fell into a frozen lake and was in the cold water for a long time. When they come out it seems there can be no way that they survived. The water’s too cold, they’ve been in there too long, and/or they’ve been submerged. But somehow they are revived! The cold slows down their body systems enough that they can survive.

Here is a link to the April 20, 1953 issue of Life Magazine with an article on what seems to be an early cooling blanket. Young Maryann Ferrante had a Ventricular Septal Defect (VSD) present since birth, but doctors of the time had no good repair options. The Heart/Lung Bypass Machine was a recent invention and not very reliable. The only other option was to cut into the heart where the surgeon thought it might be, find it, and repair it – all the while with blood gushing out of the wound and blocking any view of the VSD. Nothing good could come from that.

So surgeons at Hahnemann University Hospital in Philadelphia came up with another plan. They would use a cooling blanket to chill Maryann down to 76 degrees before the operation. The lower temperature would cause her body to slow down enough to clamp the blood vessels and open up the heart. They would still have to work quickly, but the operative field would be clear enough to find the hole and do the repair. The operation was a success, as the article mentions her follow-up visit to the hospital.

Maryann was put to sleep before she went under the cooling blanket. If I had know what was coming, I would have told my doctors to knock me out, too!

Five CHD Questions: When?

May 12, 2010

When are Congenital Heart Defect (CHD) survivors at their most vulnerable?

There are two schools of thinking in the CHD world. For lack of a better term, we’ll call these two philosophies “Old School” and “New School”.

The Old School is what Cardiologists used to tell CHDers and their parents: Now that the child has his/her surgical correction(s), they are “fixed”! Certainly, they may be a step slower, not be able to do as much, can’t play as hard as the other kids, but their defective heart has been repaired and they are set for life. All is well that ends well!

New School is the reality: As time passed as the early survivors began to grow into adults, they started having problems related to their deformed hearts. Finally it was realized that just maybe that original repair wasn’t permanent, and some of the old problems were coming back. So now, New School Cardiologists tell their patients that while they are repaired, a CHDer needs lifelong care by a specialized Cardiologist to make sure that those problems stay under control.

Be warned, the Old School of thought still exists, despite all the evidence to the contrary. Old School thinking exists on several levels – not only does it include the fallacy of “once repaired, totally repaired”, there is also a segment of the population who think Heart Defect = Death Sentence. Such thinking is toxic and you do not need this in your life: The best thing you can do if you should run into someone with an Old School mindset is to RUN!

The most dangerous time for a CHDer is when he/she begins to transition away from their Pediatric Cardiologist to an Adult Cardiologist. Most Adult Cardiologist deal with Acquired Heart Disease, not Adult Congenital Heart Defects (ACHD). Acquired Heart Disease are heart problems caused by diet, clogged arteries, hardening of the arteries, high cholesterol, and the natural aging of the heart. Someone with a Congenital Heart Defect will not have these problems, or they will manifest themselves differently in our bodies. So we need someone experienced with caring for Adult Congenital Cardiac patients – an ACHD doctor.

The problem is, there are currently very few ACHD doctors available. In an interview recently posted on Adventures of a Funky Heart!, Dr. Michael McConnell gave an example: There are about fifty Pediatric Cardiology practicing in the city of Atlanta, Georgia, but only two part time ACHD Doctors.

ACHD doctors can be found, though it may take some searching. The Adult Congenital Heart Association (ACHA) offers an ACHD Clinic directory for the United States and Canada on their website. (ACHA Membership is free). ACHA is also pursuing Vision 2020, a proposed blueprint for Adult Cardiological Care in the 21st Century.

Vision 2020 builds on the Bethesda 32 Report produced by the American College of Cardiology. Bethesda 32 was produced in 2000 and addresses the issue of Adult Cardiological Care. (The name is derived from the 32nd meeting of the American College of Cardiology’s yearly focus group held in Bethesda, Maryland.  The group studies a different issue each year.)

The Bethesda 32 Report divides the known heart defects into three groups – “Mild”, “Moderate”, and “Severe” and recommends the proper level of care needed for each group. While someone with a “mild” heart defect – a very small unrepaired Atrial Septal Defect (ASD), for example – can usually be cared for by the general medical community with yearly ACHD checkups, someone with a “Severe” defect (my Tricuspid Atresia, for example) needs to be seen in at an ACHD Clinic regularly. Severe CHD patients should also consult with their home clinic before undergoing most medical procedures, getting pregnant, etc.

There is a good outline of the Bethesda 32 Report HERE; you can read the report online by clicking THIS LINK. Click “Print a PDF” from that page to print out a copy of the Report. Be prepared; it is over 60 pages long.

Having a copy (or several) of Bethesda 32 can come in handy when/if your insurance company balks at providing coverage for an out-of-network doctor. The report can be used to show established medical guidelines saying that you need to be seen by a specialist.

The profile of the “average” Heart Defect Survivor is changing, as there are currently more adult survivors than there are children. Congenital Cardiac Care is slowly changing to reflect this.

Did you cause your child’s Heart Defect?

April 9, 2010

There has been some recent news coverage that contends that you just might have, if you were obese when you became pregnant.  But just in case you need to be reminded, News organizations make money by selling their product. There are lots of ways to do this, but two of them are really effective: 1) Make something so appealing that you just have to know more; and 2) Scare you so bad that you tune in/buy a copy to learn how to protect yourself.

How many Heart Moms look at their child in an ICU unit and ask themselves “What if…?” I’d wager that the answer to that question is all of them.

So you need to take health news on TV and in magazines with a grain of salt. A big grain of salt, really. The first thing I would recommend is that you drop by Gary Schwitzer’s Health News Review Blog. Gary has been in the Health Reporting field for 30 years, and he’ll tell you if the latest news report is wrong, and how it is wrong. This is a great resource to check out all those “We’re doomed!” news stories for yourself. Gary can teach you how to look at those news stories with a critical eye and not be scared to death.

Don’t take everything you read or hear at face value; if possible, try to find the original research. Read the information for yourself; then make an informed decision.

Did you cause your child’s Heart Defect? No one can tell you for sure. The new report that is stirring so much concern says that there is a better chance of having a child with a Heart Defect, but obviously it is not a certainty.

The heart begins as a straight tube, much like a drinking straw. As the heart develops it actually starts to beat in this configuration! Later, it will twist and fold over onto itself, forming a loop that will begin to grow together and form the organ that we are all familiar with.

All this happens before you even think that you might be pregnant. By the time you see the doctor to confirm it, the heart has formed. If you are destined to be a Heart Mom, it has already been decided.

Now this doesn’t mean that you can ignore all of your doctor’s advice! But think about it – millions of women have been like my mom – she did everything right, tried to follow their  doctor’s instructions to the letter, and still  I was born with a Heart Defect. Other women had every vice known to man, and their children were born healthy. So what causes Heart Defects?

I think that the main cause is a combination of genetics and environment. Research funded by the March of Dimes has found several genes that interact to help the heart form. Of special interest is the GATA4 gene – something it does helps the heart split into the left and right sides and form the Septum.  Figure out what is happening there, and maybe you can stop ASDs and VSDs!

The National Heart Lung and Blood Institute (NHLBI) has also determined that genetics play a part in the development of Hypoplastic Left Heart Syndrome (HLHS). A study of the family medical history of HLHS patients found that their ancestors also had a specific heart problem: a Bicuspid Aortic Valve. So apparently there is a genetic cause for HLHS – now the challenge is to find it.

Environment also contributes to heart defects. A section of Baltimore, Maryland contains a “cluster” where the incidence of HLHS is twice as high as the national average. What is going on there? What makes that area so different that it could trigger a Heart Defect?

Nationally, the incidence of Heart Defects is 1 out of every 125 newborns. But in the state of Wisconsin, the rate is 1 out of every 74 newborns. Wisconsin isn’t taking this lying down – they’ve created the Wisconsin Pediatric Cardiac Registry and are studying the data that it has generated, looking for clusters and what might be in the area that could create a Heart Defect hot spot.

What is the interaction between genetic factors and environment? What happens that triggers the formation of a heart defect? No one is certain, and the scary answer is that different factors contribute to different heart defects.  But if one day we could answer those questions, then maybe we could slam the door on Congenital Heart Defects.

Because every heart deserves to live a lifetime.

Devil in the Slot

March 2, 2010

BONUS: 100 Karma Points to the first person who can leave a comment telling me where the title of this post originated!

You have a heart defect.

No, I’m not talking about the 1 in 125 of us who live with a Congenital Heart Defect, I’m talking about YOU. Mr. or Mrs. Average. You were born with a heart defect. Two of them, actually.

Obviously, you don’t need to breathe while you are still in your mother’s womb. You can’t breathe, unless you can somehow magically breathe fluid. So your lungs are “turned off” and you have two small defects that allow the heart to function but blood to bypass the lungs and pick up its oxygen from your mother. When you are born and you take your first breath, the body sends a signal to those two defects to shut down and for the lungs to take over.

(Pretty cool, huh?)

One of these small defects is called the foramen ovale and it is really a very small Atrial Septal Defect. In fact, it isn’t really a hole in the septum, it is two overlapping flaps. When you begin to breathe and blood begins to flow properly, these two flaps will eventually seal closed and everything will be great. In about 30% of people, however, it doesn’t close. When that happens, it is called a patent foramen ovale, sometimes called a PFO (“patent” means “stuck open”).

Many times this doesn’t cause much of a problem; sometimes it is even undetectable unless the patient coughs while having an ultrasound.

Now here is the problem: when a person has a PFO, or when it is slow to close, occasionally a small “pouch” forms in the wall of the Left Atrium. Doctors at the University of California at Irvine recently found that pouch while doing autopsy research, and it could be the source of several problems.

That little pouch is nice and quiet; any blood that gets in there settles down – it’s no longer part of the normal blood flow, and it is pretty calm in there. While the heart is pumping and blood is churning all around it, it’s a nice quiet little neighborhood… almost a gated community. (This Link has a good explanation and has a nice drawing of the pouch about halfway through the article. )

That’s not good. Blood can settle in the pouch and form a clot. And since it is on the left side of the heart, the clot skips the lungs (which not only add oxygen to the blood, but they also act as scrub brushes, too!) and then it is off on a tour of the body. Round and round she goes, where she stops nobody knows. But if it stops in the wrong place….

STROKE!

So if the doc tells us we have a PFO, we should get it closed, right? Perhaps, and perhaps not. The medical evidence isn’t in yet. We know what might happen, what could happen – but how many times is the pouch really the cause of a stroke? We don’t know yet. A clinical study (the RESPECT trial) is being conducted right now to determine if a PFO causes more strokes. At least one doctor isn’t waiting to find out – she’s full steam ahead. The Food and Drug Administration is saying wait a minute, slow down, catch your breath… let’s see what the evidence says. Then we can decide. (You really need to read what Isis has to say about PFOs, she gets the point across quite well, in simple English and with a lot of humor, too!)

So, is there really a devil hiding in that “slot” that could be in your heart? And if there is, what are the chances of him coming out? No one knows… yet.

The Heart of a Warrior: A Funky Heart Interview

June 16, 2009

My friend Eliza recently took time to answer my questions about growing up with a heart defect, exercise, and participating in the Bolder Boulder 10K road race.

Born with Pulmonary Atresia with a Ventricular Septal Defect (VSD), Eliza underwent four heart operations as a child.  “I was never the most athletic kid on the block,” she tells me, “but my parents did encourage me to try lots of things.  Even though P. E. (Physical Education) was never my best or favorite class, I’m very glad that my parents followed my cardiologist’s suggestions and never kept me out of it.”  Eliza participated in ballet for eight years and was on the softball team for a year in middle school.

Her biggest challenge in 4th and 5th grade was trying to keep up when her school participated in the President’s Physical Fitness Challenge. The Challenge exposed her weaknesses: “I remember being reduced to tears when I didn’t understand how I was so bad at sit-ups and the mile run. It never occurred to me that it was because of my heart and the surgeries I had for it.”  Luckily she had a great P.E. teacher  in elementary school who emphasized that the important thing was to get outside, move around, and have fun.  “(She) told me that if I wasn’t dirty when her class was over, then I wasn’t having enough fun.  I’ll always remember that.”

Eliza also had another Physical Education teacher in high school who “taught me basic physiology, anatomy and the benefits of exercise.  Without those two gym teachers’ encouragement and knowledge, I certainly wouldn’t be as healthy as I am today.”

After graduating from both high school and college, Eliza took internships in a big city and started on a path that would lead her to the Adult Congenital Heart Association (ACHA). Describing those days as “a very dark time,” she tried to deal with adulthood, work, finding friends and doctors in a new city, and the realization that her heart wasn’t permanently fixed, all while being hundreds of miles from home. “I thought that I was entering a whole new, exciting world, but I ended up feeling very alone on so many levels.”

After a false alarm with her heart and without any guidance from her doctor at home, she finally found an Adult Congenital Heart Defect (ACHD) Cardiologist. A month or so after her appointment, she received a postcard in the mail from her new doctor with information about the Adult Congenital Heart Association.  She was curious to find out how other adults with congenital heart defects managed their health and how that worked in their lives.

Returning to Colorado, Eliza got involved with the group that would eventually become the Denver-Metro Area chapter of the ACHA. Little did anyone, especially Eliza, know what would come next!

Boulder, Colorado is the home of the Bolder Boulder, a 10-kilometer (6.2 mile) road race through the streets of downtown Boulder. Described as a “citizen’s race” because the majority of participants are not professional runners, the race is organized in staggered starting groups called “waves” that allow people of many fitness levels to compete at their own pace. The minimum requirement is to be able to walk 6.2 miles in two hours.  The event concludes at the University of Colorado’s Folsom Field with a Memorial Day celebration after the citizen racers take their seats in the stands to watch the professionals runners compete in their own 10k event.

“The atmosphere is so much fun!  Besides the huge number of racers, (53,000 participated in the 2009 race) there are radio personalities, belly dancers, celebrity impersonators, rock bands, people with their sprinklers on, Slip & Slides, and neighbors sitting in lawn chairs cheering on all of the racers as they pass.”

And that’s just the bystanders. A good portion of the runners let their sense of humor come out – there is no telling what you’ll see along the course. “There are people who dress up in funny costumes in all of the waves,” Eliza continues.  “There’s a guy in a gorilla suit who is usually in the very first wave.  I’ve seen people in frog costumes playing leapfrog in the walking waves. This year I saw a group of girls dressed like an 1980’s band and another group who was trying to do the race as a 3-legged race.  They have water and Gatorade for the racers at every kilometer and race officials all over to make sure everyone has a safe and really fun time.”

Eliza had been walking the Bolder Boulder course since she was a child, usually with her mother. “The first time I did the Bolder Boulder I was probably ten years old. It was my mom’s idea – she wanted to walk it and thought it would be a fun thing to do with me, even though she is the least athletic person in my family. I walked the race with her on and off for years until I decided to do it for my own health in 2006. “

“That year I invited family, friends and a few people from the local Adult Congenital Heart Defect (ACHD) group.  We thought, ‘Man, it would be great if we could do this and officially raise awareness for the ACHA!’”

ACHA president Amy Verstappen, Eliza and the national staff worked together to create the legal documents needed to make it an official event. The ACHA’s Bolder Boulder team was off and running!

“Given the economic climate this year, I am very proud that we raised over $1,300 even though it’s significantly less than what we’ve raised in previous years.  In 2008, we raised over $3,000 for the ACHA.  This year we had a record number of ACHA racers – 24!.”  (That number includes both Congenital Heart Defect (CHD) Survivors and supporters.)

A handful of ACHA members and supporters have come to Colorado from out of state to participate in the festivities. This year, there were people from California, Ohio, New York, Illinois, Pennsylvania, and South Carolina.

While those numbers are impressive, they’re not the essence of the event for Eliza. “Since I decided to do it (participate in the Bolder Boulder) for my own health, it’s been a positive goal that’s kept me exercising regularly,” she says. From her enthusiasm when she talks about it, you can tell Eliza is happiest about getting other CHD survivors to be active. “I’ve been thrilled to find out that our participation not only inspires our racers, but also people across the country and other ACHDers who aren’t quite able to do a 10k to figure out how to incorporate some exercise into their lives. I love that something that started out as a personal goal for me has mushroomed into an avenue and an inspiration for so many in the ACHA to be as healthy as they can be!”

As Adventures of a Funky Heart! readers know, I often ask interview subjects, “Do you have any wisdom or advice for young parents of Cardiac Kids?” Eliza didn’t disappoint:

“Medical interventions like surgery and caths (Catherizations) can help our hearts to function more normally, but that willingness to get out of the house, to get up when we fall down, to actually move around and exercise itself is what strengthens even hearts like ours and gives us the confidence, mental and emotional fortitude to manage our heart health as we get older.”

“For everyone, but especially for kids with CHD, exercise isn’t just about moving your body and making is stronger.  If the adults around you have the right attitude, it’s about having the self-confidence to try another way when you reach a roadblock.  It’s about learning to maintain and trying to top your own personal best. It can be about learning to be part of a team.  It’s about kids (teens and adults too) who have known what it’s like to feel incredibly physically weak and vulnerable, finding ways to feel physically powerful, able, strong and independent.”

As far as specific advice, Eliza says, “Anytime they are excited about doing something physically active, go with it! As long their cardiologist thinks it’s healthy for them, let them do the mile run – even if they end up being the last person who finishes it. It’s even more important for us to learn how to handle minor scrapes & bruises than most people because we’ll face so many physical challenges in our lives.  Resist the urge to run and pick your kids up immediately when they fall.  We need to learn that resilience. Let them climb trees. Let them fall and scrape their knees. ”

Eliza reminds us of a very hopeful statistic: “At least ninety percent of children born with congenital heart defects today will be adults someday. Always assume that your child will be in that ninety percent and treat them accordingly. Don’t forget to daydream about what you hope to do with your child and the kind of person that you hope they will become.”

ENDNOTE: Eliza was recently accepted into graduate school to study health promotion, partially inspired by her love of enabling CHD Survivors to challenge the Memorial Day race over the last few years.  She’ll be leaving the Denver area, but I’m hopeful that no matter where life takes her, she’ll always find her way home just in time to take on the Bolder Boulder!

I hope you’ve enjoyed meeting my friend;

The back of Eliza's 2008 Bolder Boulder shirt

The back of Eliza's 2008 Bolder Boulder shirt