A Win-Win Situation

Dear Heart Friends:

This last week "Heart to Heart with Anna" dealt with Sports and Boys with Critical Congenital Heart Defects. We had two excellent dads of sons with hypoplastic left heart syndrome (HLHS) and an adult with HLHS as our Guests. During the Q & A session at the end of the show another HLHS adult and his father called in. It was a terrific show.

It seems that the news is full of discussion about people with congenital heart defects. There was the inspiring story of Hampton goalie Cameron Raidna set to return to the ice after heart surgery, the tearful story of a mom encouraging heart screening for athletes after her son's sudden death, the joyful story of a Briarcrest athlete whosurvived a potentially deadly heart defect who just graduated but the story that has really caught my attention is about an athlete who goes to college just down the road from me at Baylor University. 

It all started with an article about Isaiah Austin when a medical condition ended his dream of being a draft prospect for the National Basketball Association (NBA). I was heartbroken to read that he was diagnosed with Marfan's Syndrome and that his aorta was to weak to continue his dream. He seemed so talented an he had already overcome so much already! During his last season with the Baylor Bears he revealed that he had a prosthetic right eye after multiple operations couldn't repair a detached retina. The eye had been damaged when a previous injury was aggravated doing a routine dunk before a middle school game. 

After the news of his career-ending medical condition came out, he was very forthright and encouraging of his teammates instead of wallowing in his own disappointment. What totally delighted me was this story I read last night about what the NBA did to Isaiah Austin. They honored him during the draft when Commission Adam Silver announced between the 15th and 16th draft picks that the NBA picked Isaiah Austin.  

It was a dream-come-true for Isaiah Austin and it's a win for the NBA, too. After finishing the next two years of school at Baylor, he can start working for the NBA or who knows? Maybe he'll coach for Baylor someday. What appears to be a certainty is that this young man will use his God-given talents to help others. He spoke in such an inspiring way in a recent interview that I have no doubt we'll be hearing from this young man again in the future.

It's not everyday you see a win-win situation like the one created when the NBA picked Isaiah Austin. It's not everyday that people are made aware of the danger of congenital heart defects. It's not everyday that people learn about a syndrome that affects 1 in 5000 people. This week marks some special events in one man's life but who knows who he'll inspire and whose life he just might save by making people aware of congenital heart defects and Marfan Syndrome. God Bless you, Isaiah Austin!

Skipping a Beat

What is the number one cardiac arrhythmia? What heart condition affects over 3 million people in Europe alone? What is the fastest growing technique in cardiology?

The answer may surprise you. It isn't about cloning, stem cells or any of the in utero procedures you might think it would be. The condition isn't something that people talk about, yet 5% of the population over 75 suffer from it. That's 5% of all adults. The percentage of adults with congenital heart defects (CHDs) who suffer from this condition is 15% -- three times that of the population at large.

What is it, you may wonder? It's atrial arrhythmias or atrial fibrillation. It's commonly called Afib or AF in the heart world. This arrhythmia is characterized by a rate or rhythm problem with the heart's beat. According to the National Heart, Lung and Blood Institute, "During an arrhythmia, the heart can beat too fast, too slow, or with an irregular rhythm. AF occurs if rapid, disorganized electrical signals cause the heart's two upper chambers -- called the atria -- to fibrillate. The term "fibrillate" means to contract very fast and irregularly."

Why is this important and why should people in the heart world care about this situation? The reason is that people with undiagnosed Afib are at a much greater risk for stroke and heart failure than those who know they have Afib and are under a doctor's care for treatment of Afib.

This is especially important for the aging congenital heart defect population. According to Circulation, those born with CHDs have a much greater likelihood of developing Afib as they age. In fact, certain heart defects such as: Ebstein anaomaly, transposition complex (transposition of the great vessels also known as transposition of the great arteries), univentricular heart (which include but are not limited to: hypoplastic left heart syndrome, single ventricle, double-inlet left ventricle, double-inlet right ventricle, mitral or tricuspid atresia or single ventricle heterotaxy syndrome), and atrial septal defects have an even higher percentage of Afib associated with them as survivors of these congenital heart defects age.

Last week we talked about the concern of losing adults with congenital heart defects to follow-up care and this is one of the reasons why maintaining regular cardiology visits is so important. When people are born with congenital heart defects, they may have arrhythmias for all or most of their lives. If they are like my son, they don't feel these arrhythmias as being unique, special or worrisome. It's the way their heart has always beat. Because of that, unless they are suddenly experiencing fainting, an usually fast or slow heartbeat (for them) or sudden weakness, they may not even know they are suffering from Afib at all. For many people, they have no symptoms. I have watched my son's electrocardiogram being done on him and have watched the arrhythmias being documented on paper while my son tells his cardiologist that everything is fine. This is what is the most concerning part about Afib. For many there are no symptoms. For some, that can be life-threatening.

What's really exciting is that according to News Medical, atrial fibrillation ablation is one of the fastest growing cardiology techniques. According to this article, there are two different ways to treat Afib. One way is with anti-arrhythmia drugs (AAD) and the other way to treat the problem is through catheter ablation of atrial fibrillation. However, I know of another technique that is being used -- and it's one that I hope will save my son from future problems with Afib. The other procedure I know about is called the Maze Procedure. According to WebMD, the Maze Procedure is a surgical treatment for Afib and is also known as surgical ablation. My son had this procedure while having a Fontan revision. Since his chest was open already and he had known Afib issues, the surgeon decided to do a modified Maze Procedure on his heart to stave off future arrhythmias.

I think it's exciting to note that doctors are learning ways to prevent arrhythmias through catheterization techniques because these techniques usually result in shorter hospital stays, less chance for infection and thus less complications. It's gratifying to see that many of our children with congenital heart defects are being followed into adulthood and that doctors are trying to find ways to keep our children healthy into and throughout their adulthood. Preventing life-threatening arrhythmias is one of the ways doctors can ensure our adults with CHDs continue to live quality lives.

According to the News Medical article mentioned, "It is estimated that more than 10,000 atrial fibrillation ablation procedures are performed annually in Europe and the number is increasing exponentially since over the last years availability of more sophisticated techniques and equipment has produced a marked increase in the number of centers performing atrial fibrillation ablation. Three dimensional mapping systems, robotic techniques, new energy sources and new and more reliable catheters are easing the procedure and improving efficacy and safety." Catheter ablation plus surgical ablation are two invasive ways of saving adults' lives from fatal or near-fatal arrhythmias. Parents in the heart world know that our lives can change in a heartbeat. It's nice to know that doctors can make sure that our children's heartbeats can continue to stay regular despite their funky hearts.

Adults with Congenital Heart Defects Lost to Care?

Once upon a time, there were more babies alive with congenital heart defects than adults because babies and children often passed away before they became adults. In 2008 there was a special report published in Circulation, the journal of the American Heart Association, that stated that adults with congenital heart defects outnumbered the number of babies with congenital heart defects. Apparently, that milestone was reached as early as 2000. An article in Heart: An International Peer-Reviewed Journal for Health Professionals and Researchers in All Areas of Cardiology had an articled entitled, "Loss to Follow-up of Adults with Repaired Congenital Heart Disease" by Christopher Wren and John J O'Sullivan. It stated that by 2000 adults with CHDs outnumbered babies with CHDs for the first time ever. The article addressed the concern doctors had for the increasing population of adults with congenital heart defects and stated that the loss to follow-up care began when survivors were still children.

It is readily apparent that our infants and young children need follow-up care, especially since those children with critical congenital heart defects often have staged surgeries that last for the first years of a child's life. What happens to children after they have completed their staged surgeries? What kinds of concerns do parents have as their children with congenital heart defects age? What happens when a parent's insurance has capped out?

When parents have an infant born with a congenital heart defect they are immediately thrust into a position where it is common for them to go into shock. It is also not uncommon for parents of children to experience post traumatic stress disorder (PTSD). Some parents even find out while the mother is pregnant that the baby will be born with a less-than-perfect heart. This requires parents to make decisions that parents didn't have to make before ultrasounds helped detect problems with unborn babies' hearts. Now parents don't only have to decide where their children should have surgery and who the surgeon should be; now parents are having to decide whether or not their should abort their unborn children. In the past I've actually had fathers call me to ask how much it cost to have a child with a heart defect. With each child's care being so different, of course I couldn't give a figure but I can't help but wonder if that consideration is going to be even more prevalent now that we know that an increasing percentage of our children will make it to adulthood.

The articles I mentioned above along with the 32nd Bethesda Conference: “Care of the Adult With Congenital Heart Disease” address issues of concern from the CHD community at large about a growing population of adults -- adults with congenital heart defects. We cannot afford to lose these special individuals to follow-up care. We need doctors who are trained to deal with adults who don't suffer from acquired heart disease but rather who were born with congenital heart defects and who may appear different from their "typical" patients. There is nothing typical about our adults with CHDs, at least not the ones with critical CHDs (or CCHDs).

Many of our adults were pioneers who helped cardio-thoracic surgeons and pediatric cardiologists to develop new techniques. They were the guinea pigs who tried new medications to see if they would work well enough to allow growth and aging. Because of the brave parents who handed their children over to medical professionals and begged them to do whatever it took to give their children a chance for life, now these professionals know what works and what doesn't work. In adults with CCHDs, that might very well mean that a young surgeon will see an adult who had a surgical technique to save his/her life that was discontinued before the surgeon even entered medical school. This means that doctors today will see an even great variety of adults with CHDs who have had a number of life-savings techniques that aren't even utilized today.

What does this mean for adults with CHDs? I believe the most important thing for adults with CCHDs to know is that they must continue to see their primary care physician and cardiologist on a regular basis. It is only through doctors amassing more data on what happens to these individuals as they age that we can look for trends and patterns. It's heartbreaking to think of an adult with CHDs being brought into the emergency room with something (like a stroke or heart attack) that could have been prevented had they not been lost to follow-up care.

Heart moms and dads, it's imperative that you don't believe that your child is "fixed" if they've had open-heart surgery. Even if the child has had a simple "hole in the heart" repaired, if the operation required the surgeon to open the child's chest, complications from the open-heart surgery may not be seen until years, possibly decades later. The problem is, since so many adults with CHDs are lost to follow-up care, we simply don't know what a "normal" course is for this special population.

Parents, please work with your child's doctors to work on transitioning our children from child to adult with CHDs and from pediatric cardiologist to a cardiologist who specializes in the care of adults with CHDs. If our aging population works together, our adults with CHDs have a much better chance of living the quality of life we parents dreamed they could live when we first handed our babies over to the care of a surgeon.

Special Guests for Heart to Heart with Anna!

Dear Heart Friends:

It is with great joy that I am able to announce two very special Guests for the only talk radio show for the congenital heart defect (CHD) community available every week!

Tuesday, June 10th our topic will be: Failure to Thrive: Oral Aversions and Nutrition. Since so many of our children with CHDs suffer from the diagnosis of "Failure to Thrive" and often have oral aversions or food preferences that makes providing our CHD children with proper nutrition, this is a show you won't want to miss!

I'm pleased and excited to announce that one of our Guests for the show will be Kristi King! Thanks to Callie Rickard, I read a terrific article on the Texas Children's Blog by Kristi King. I loved how she talked about clean eating and healthy living. For our failure-to-thrive children, nutrition is even more complicated than trying to eat "clean." "Healthy living" for our families is not like it is for other families but I know that Kristi King is going to be able to share some insights with our CHD families about how to help our children to make the most out of their nutritional intake.

The other Guest I'm excited about is not currently scheduled for a particular day yet, but I hope to have him scheduled soon! Yesterday I went to a book signing at Books-a-Million for a brand new book called Special Heart by Bret Baier. Bret Baier, as many people know, is the Host of Special Report with Bret Baier on Fox News.  Bret Baier is on a book tour for this book right now and I strongly encourage my heart friends to take the time to buy this book, read it, and if you're lucky enough to be in a town Bret Baier is visiting, to go to the book signing.

"Heart to Heart with Anna" is now on Tuesdays from 11:00 to noon Central Daylight Savings Time on BlogTalkRadio. You can learn more about the show by visiting the "Heart to Heart with Anna" website. If you are interested in being a Guest, please click on "Be On The Show" and fill out the form with your information.