The Tallest Kid in the Room

There's a great new blog out there that I'd love to point our readers to... Adventures of a Funky Heart. The blog is written by Steve, a 41-year old survivor of Tricuspid Atresia. He's allowed us to reprint a portion of a recent entry here. Enjoy, and be sure to check out Adventures of a Funky Heart!

July 28, 2008 by Steve

They always have Highlights for Children. It’s a common staple of any Pediatrician’s office, but it never seemed that they made the magazine available for home delivery. I always wanted a subscription to Highlights, because I enjoyed all the puzzle pages. But that was then. Times have changed, and now I’d rather hae a subscription to a good baseball magazine. Most children aren’t that interested in baseball, so I need to bring my own.

I walk into the doctor’s office and I can feel the eyes boring into my back. I can’t see them, but I know that everyone is looking at me. When someone my age comes into a pediatrician’s office without a child, everyone stares. What’s he doing here? If they are wondering now, just wait until the nurse checks me in and tells me to have a seat.

Usually all of the adult chairs are occupied. It’s impolite to stand in the waiting room – there’s no rule against it, but I’ve never seen it done unless every seat is taken – so I sit in one of the children’s chairs. That’s an adventure unto itself. You sit down as you normally do, but you just keep going down until your bottom hits the chair with a bone jarring THUMP and your knees are level with your eyes. Now I look stupid, so I stretch out my legs. Not only am I blocking the walkway, but now I really look stupid, so I pull my legs back in. Hopefully this won’t be as bad as the time that I had pneumonia: my doctor admitted me to the hospital, and after a 4 hour wait in the ER I learned the only available bed was on the Children’s wing. It was a loooooooooong way from my rear end to the child sized toilet, and I was too far down to stand up! I literally had to roll off the seat and then get to my feet. But there was no room in the inn, you take what you can get, and beggars can’t be choosers.

Until I was 30 years old I saw my local pediatrician for non-emergencies. It works the same way for kids with heart problems. “Adult” Cardiologists can’t deal with us – they are trained to deal with heart attacks, clogged arteries, and all the problems that your heart develops as you age. They usually don’t have experience dealing with Congenital Heart Defects (CHD). A friend of mine – also a Cardiologist – once said that if you chose to specialize in Adult Cardiology, you received about two hours of training in CHDs. Basically just enough to know that they exist. So no matter our age, Cardiac Kids are still patients of a children’s doctor.
But now there’s a new specialty, known as an Adult Congenital Heart Defect doctor. Adult Congenital Defect care is not taught at any medical school (yet); many of the ACHD docs are really Pediatric Cardiologists. Since their patients stay with them practically all their life, these “children’s doctors” found themselves dealing with questions about work, pregnancy, dating, insurance… questions that Adult Cardiologists usually have to answer. And so the field of ACHD Cardiology was born.

You won’t find one on every corner, but ACHD care can be found. Usually, you’ll need to go to a major medical center. I live in rural South Carolina; the ACHD centers in the South include Duke University, Emory University, and the University of Alabama at Birmingham (UAB). Other major centers include The Mayo Clinic, Stanford, the Philadelphia Adult Congenital Heart Center and Children’s Hospital of Boston. There are other clinics, of course, but those are the ones that spring immediately to mind. And now, the waiting rooms have people who are more in my own age range.

I miss the visits to the Pediatric Cardiologist, though. While I’m sitting there trying to get comfortable, absorbing all the stares, the kids don’t pay a bit of attention to me. Kids are kids, they are usually congregated in the play area, getting along famously and having a great time. For once, they aren’t being left behind on the playground – everyone is moving at about the same pace.

The parents are the ones sitting there with the worried look on their face, with good reason. I always hope that one of the parents will speak to me, perhaps ask if my child is sick. If they do, I’ll tell them that no, I’m the one with the heart defect. My parents were in the same boat you were. I’m 41 now, and still going strong. And hopefully someone will come to believe that with good medical care and a little good fortune, their child will do well.

Research Yeilds Answers on Aortic Arch Defect Causes

By The Pittsburgh Tribune-Review
Tuesday, July 8, 2008

Research led by University of Pittsburgh scientists is the first to describe how two common congenital circulatory problems form, the university announced today.

The team found that a gene called unc45a plays a critical and previously unknown role in the formation of aortic arch vessels. The vessels contribute to several of the body's major arteries and often develop improperly, causing a wide range of vascular defects.

The team also found that arteriovenous malformations, or AVMs, happen when an artery fuses with a vein, diverting blood flow, and result from genetic and physiological factors. Previously doctors had believed its origins were solely genetic.

The research is published in the journal Developmental Biology. Scientists at Georgetown University Medical Center and the National Institutes of Health also participated.

First Hearts United Conference held for HLHS families

PRLog (Press Release) Bloomington, MN, USA. Jun 23, 2008 -- The non-profit organization Hypoplastic Right Hearts held its first-ever medical and educational conference, Hearts United 2008, in Bloomington, MN June 18-21, 2008. Families with children born with hypoplastic right heart syndrome (HRHS) from around the US and Australia gathered to meet in person for the first time, and some families had never met another child in person with the same diagnosis.

The members of Hypoplastic Right Hearts planned and fundraised for almost 3 years to make this special event happen.

Five years ago when Amanda Adams of Colorado had a baby boy born with a complex, life-threatening congenital heart disease (CHD) where the right ventricle of the heart does not develop properly, she felt all alone as her newborn son faced multiple open heart surgeries to have a chance at survival. Consequently, Adams founded and became president of the online non-profit group, Hypoplastic Right Hearts (www.hypoplasticrighthearts.org). Hypoplastic Right Hearts provides emotional support for parents, primary caregivers, survivors and their spouses, and bereaved parents of children with CHDs that result in a hypoplastic right ventricle. What started with a handful of “heart moms” as a Yahoo! Group in 2003 has now grown into a worldwide membership of over 450 families.

For more information about Hypoplastic Right Hearts group and Hypoplastic Right Heart Syndrome: http://www.hypoplasticrighthearts.org

Long-term Care of CHDs

As a parent of a child with Transposition of the Greater Arteries (TGA), I will never forget the moment when the surgeon sat down with me after her open-heart surgery and told me "She's fixed." When I asked what I could expect from then on out he told me "She'll be able to live a normal, active life" and "she'd only need a check-up once a year or so." What he didn't tell me is that she was NOT fixed. She will always need to be watched closely. It is likely that she will face complications down the road.

Sure, it's every pediatric cardiac surgeon's dream to tell a parent that a child is "fixed." It doesn't happen often. Some of the more severe defects require many surgeries, none of which are a "fix," but are aimed at extending the lifespan until a fix or alternative treatment can be found. But is it really fair for a surgeon to sugar-coat the truth? No, it isn't.

That's why I was upset when I found out that my daughter's defect is considered severe, requiring life-long care. I had entered into a world where TGA seemed like a minor defect. The truth was like a slap in the face that woke me up from my complacency.

The truth about Congenital Heart Defects is that many DO require life-long care and follow up - not with an adult cardiologist (that is often not specialized in CHD-care), but with a specialist in Adult Congenital Heart Defects. The Adult Congenital Heart Association is working to spread the message, as are many CHD specialists across the world.

For an example, check out today's (17 June) news article from KSPR News in Springfield, Missouri:

Heart Defects need medical attention through adulthood
By KSPR News (visit the link for video footage)

Fifty years ago babies born with congenital heart defects weren't expected to live into adulthood. But thanks to advances in medicine, most babies with heart defects now live long, full lives.

"What it's called is tetralogy of fallot. It's one of the most common occurring complex defects."

Kyra's heart had four abnormalities. The pulmonic valve was narrowed, and there was a hole in the septum, or wall between the right and left ventricles. Also, a main artery was in the wrong place. These issues caused her right ventricle to get thick because of all the extra pumping it had to do. At age 4, Kyra had surgery. She was considered cured.

"Then I started developing symptoms. Shortness of breath, electrical conduction problems, arrhythmias."

It turns out she needed another valve replacement. The repaired valve started to leak. Dr. Keith Oken says Kyra was a victim of a common misconception.

"Which is that she was cured with the surgery she had. That is rarely the case."

Kyra needs continuous, life-time care. But there are not many cardiologists trained to manage cases like hers. That's because until recently most people with congenital heart defects did not survive into adulthood. So to make sure Kyra stays healthy, she sees her regular doctor and experts at mayo clinic. Plus she's doing whatever she can to keep her heart strong...

Doctor Oken says people with adult congenital heart defects need to continue seeing their doctors... And to see a heart defect specialist.

Stereoscopic Glasses May Aid Beating-Heart Surgery

Source: iTNews, 10 June 2008

In a recent study, the three-dimensional glasses have been used in a successful operation on pigs with a common form of congenital heart disease. The newly-tested stereoscopic glasses enable depth perception by splitting computer images in two, and cocking them at slightly different angles to provide surgeons with ultrasound images of the beating heart as a hologram.

Researchers expect that clinical trials of beating-heart surgery with the patching system could begin in children with ASDs this year.

Social Security Benefits for Adults with CHD

Source: Cavey|Barrott Attorneys at Law Blog

Social Security Disability Benefits are paid for congenital heart disease. The Social Security Administration uses Listing 4.06 to evaluate symptomatic congenital heart disease. You show the results of specific medical testing and meet very stringent medical findings to qualify for listing.

More often than not, Social Security disability benefits for congenital heart disease will be awarded based on vocational criteria.

Growing up with CHD, Part 3

Growing up with CHD: A Mother-Daughter chat


By Sarah Clark and Connie Walker


Hey everyone! I apologize for the delay on this post. this week has been a bit crazy in the Clark and Walker households, with me and my husband closing on our first house next friday and my folks packing up for a family visit in Virginia. Thanks again for your patience! After the draining experience that writing (and probably reading!) the last week's column turned out to be, we're going to dial things back a notch. Mom and I were talking on the phone the night before my second column posted, and liked my idea of co-writing the final article in this series. In fact, we're going to change things up a bit, and this article will be written as a interview with me asking and Mom answering questions about how my CHD impacted our family, what we're really proud of, and what, if we had it to again, we might do differently or wish someone had told us.

Also, what's next for my involvement with the CHD blog? Well, As you see, Kim has bestowed on me the honor of co-authoring this blog, and while she's certainly more of an expert on raising a kid with CHD, I'm looking forward to adding my two cents from the other side of the fence, so to speak. After my life settles back down from our move and a few work commitments I'd kind of like to put my librarian hat on for an article or two and share some tips on doing medical research. When I was young the issue for average folks was finding any information at all. Now thanks to the Internet, we're all drowning in data on any medical subject you can think of, of quality varying wildly from expert to quackery. I'll help you figure out where to search, what terms to use, how to effectively use your local public and university libraries, and how to evaluate what you find and discuss it intelligently and assertively with your kid's medical team.

Last, I want to share my contact info and mention a resource I've found useful. As I mentioned, I didn't really have any CHD role models as I grew up, came to terms with some very strange and heavy stuff, and became the productive and upstanding grownup (?) that I am today. Again thanks to the Internet, that no longer needs to be the case no matter where you live. While I spend far more time on Lifehacker, LOLcats and Writing-oriented blogs and forums than in the heart defect corner of the net, I'm a proud and slightly goofy member of ACHA-this association lobbies for the needs and interests of adults with congenital heart defects, sponsors research and education in ACHD issues, hosts a bi-annual convention for adults with CHDs, family, friends, and physicians, and provides a message board where teens and adults with weird plumbing can share their victories, support each other through tough times, or (mostly) be silly! You can drop me a private message there (I'm on as OKLibrarian), or simply zap me an email at oklibrarianATgmail.com. If you're looking for the parental perspective, you can reach my mom and dad at connie52ATcox.net and cwalker71ATcox.net respectively. (Thanks again for volunteering to be spammed, Mom & Dad!)

Now for part 3:

Sarah: I know I've said this before, but why not let the whole internet know? I got very lucky to have you and Dad as parents. As I've mentioned, you helped me and Daniel survive some very...interesting times during our childhood, and we've somehow managed to both become mature, upstanding adults with spouses, mortgages and careers. What do you think were the most important things you and/or dad did that helped us get to adulthood with good mental and physical health?

Connie: We loved you. That simple--and that complicated. I'd like to believe it would have been the same regardless of health concerns or birth order, but who can say for sure. Life happens as it happens.

Whatever was happening in our lives, it was important to me that home meant safety--a retreat and refuge. We valued you two for who you were--not who we wanted you to be. We paid close attention to your interests and talents, and did our best to nurture them in every way we could. We also did our best to turn every perceived "failure" into a learning experience. By the time you were born, we had learned that resilience was the most important tool for a successful life. We made it our highest responsibility to make sure both of you had that tool!

Sarah: On the flip side--if you had it to do all over again, is there anything you would have done differently, or wished you'd known about in advance about raising a kid with CHD, or just parenting in general? (let's take not bringing me to see ET as read. *smirk*)

Mom: You've been very kind not to mention my worry-wart nature. It's my lifelong "defect" (can you tell I take strong exception to that word and what it implies...). When that worrying nature was applied to my deepest love--my family--I probably added unnecessary burdens to your lives. I wish I could have been braver. But as I've told you before, it could have been much worse! Without sharing my fears with you & Daniel, and forcing you both to prove to me you would be smart and safe, I would probably just have locked you in the house forever. It is a generalized fear...applied in its turn to my beautiful, first born, "blue" baby, crossing the street alone, riding the bike to a friend's house, dating, driving, calls from a deserted train station in Rome at 2 a.m. on Christmas Eve...you know, silly stuff like that. (And on the topic of ET, if it's still traumatic, I'll be happy to make you another ET costume and let you parade up and down the neighborhood, like we did all those years ago!)

(Note: The author would like to commend her Mom for her very savvy desensitization of her daughter's ET-Phobia, and also remind her that she had specifically requested a phone call on Christmas Eve, and it was the only phone booth available by the time we left midnight mass @ the Vatican! The only people around anyway were a policeman, my backpacking buddies and a very polite transvestite panhandler...)

Sarah: My younger brother Daniel is the coolest guy on the planet, and hasn't gotten nearly enough attention in the first two parts of our story. Among other gifts, he has more musical talent in his little toe than I will ever hope to possess, and plays keyboards part-time with local jazz and funk bands in addition to his career as an Industrial Engineer. However, he and I are very different people-he is a logical, laid-back guy, quiet where I am outgoing, mellow where I am melodramatic. How did you handle raising two children, both very gifted in their own ways but with two very different personalities and needs, and how did you try to make sure that your "normal" kid didn't feel like he was shortchanged?

Mom: See answer to question # 1! Truthfully, he probably did feel shortchanged from time to time. Probably you did too. Sibling rivalry is what it is. But disrespect, in action or word, was not allowed between the two of you (at least not when I knew about it). Do you remember when one of you would say, "that's not fair...you love ____ more than you love me!" I always answered "yep, you must be right". That always shut off the pity party, because you both knew that was silly talk. We valued you both, and expected you to value each other. That mutual love, respect, and support of each other is still my strongest desire for the two of you. Different is good!

Sarah: I often tell friends that I had a idyllic 80's sitcom suburban childhood "with a few twists"- Often I don't think they believe me until they've seen enough of us all interacting to realize it isn't some weird passive-aggressive act! How did you and Dad find a balance between acknowledging the medical challenges I (and later Dad) had to deal with growing up with giving me and Daniel as "normal" and stable a childhood as possible? Also, do you think everything we went through made you and dad a stronger couple, and us a stronger family?

Mom: Well, your dad and I are at least as different from each other as you and Daniel are! I think it is extremely important to have both parents involved in a child's life. I know it isn't always possible. In fact, for a good portion of your childhood, dad was too sick to be as actively involved as he had dreamed of being. It took all of his energy just to keep on working, so we would be provided for. That makes us sad when we look back on the childrearing years. But those kinds of regrets don't really serve any purpose except to keep people from making the most of the circumstances they find themselves in. We just did the best we could with each day that was given to us.

To answer your question, reference my overprotective, worrying nature. One day, after listening to my many plans for keeping you safe from any danger, harm, hurt or substandard educational experiences, your dad, in exasperation, said, "Connie, we don't live in Mr. Rogers' Neighborhood!" He had hit the nail on the head. That was exactly where I WANTED to live. And I told him so. But it also made me laugh at myself. I knew you (and Daniel) couldn't live there forever, even if we could afford a house in the Neighborhood of Make-Believe. And so it went, with every "crisis"--potential, undeniable, or conjured up--we both spoke up about our fears, opinions and reasons, communicated (sometimes loudly) and came up with a solution we could both live with. The one thing we always knew was that we both loved both of you, and each other, very much.

Sarah: I'm not a parent yet- some days I barely have it together enough to take care of our cat! :-) Because of that, I'm guessing that I'm missing some stuff you think is important for other parents to keep in mind. Is there anything you'd like to say that I didn't ask you about?

Mom: Becoming active in a support group was probably the one thing we did that saved our marriage, family, and perhaps even your life. Lifeline, the support group at Children's Hospital in Oklahoma City, was the saving grace when you were first born. I dealt with guilt (what had I done wrong), anger (what kind of God would let these things happen to innocent children), fear (if she doesn't live, I can not bear to live either), and overwhelming love (surely everyone knows this is the most beautiful, marvelous child who was ever born). I worried about every sneeze, about helping you gain enough weight for the surgery, about how you were progressing compared to others your age. Some of that was heart, and some of that was first baby. The worries were real. But the focus of Lifeline (named by a family who called their child's scar her lifeline) was hope, support, education and friendship. I never thought of you, or any of those other wonderful children we grew to love, as defective in any way. You all were perfectly, wonderfully made.

Your correction eventually had an excellent result, but there were problems from the post surgery complications that we had to deal with. It is possible we might have defined you in terms of those problems, except for what I call "God's beautiful gift wrapped in a very ugly package". You had a respiratory arrest after the correction at 18 months. It was my blinding light on the Damascus road. All my anger at God about why--why you--why precious little children--why child abuse--all those hard things I saw at Children's Hospital--fell away when I saw them holding the paddles over your tiny, wired body. I knew in that instant that you never really belonged to me. You belonged to God. Regardless of my plans, actions or thoughts, I had no power of life or death. You were in my heart for as long as I had you, and for as long as I lived. My precious privilege, but not my possession. My child, but also God's child. And so, that very minute, I repented of that anger. I gave you back to God, and starting praying for wisdom and courage to raise you and support you in the life YOU were meant to lead. By the time Daniel came along, I knew what to do. I gave him back the day he was born! And I prayed for wisdom and courage to raise him and support him in the life he was meant to lead.

That is my advice to parents, CHD or any other. The only power you have is the power of love. See your children as what they truly are--God's children. Pray for wisdom and courage to raise them and support them in the lives they are meant to lead. It is that simple--and that complicated.

~~~~~~~~~~~~

Thanks Mom, and I don't think I can really improve on her ending.

So, Is there a moral to my mini-autobiography? I don't know. You and your kids will have to visit and revisit this issue occasionally through their lives, no matter how "perfect" their outcome is. Aside from less than ideal physical stamina, and a few very minor hearing and motor control glitches created by my brain damage, I'm normal in pretty much every way you could define. And yet, every so often I've had to mull over these issues in my life, come to understand them in a deeper, more mature way, and make peace with them for another few years. It last happened around the time Kevin and I got married, this time it seems to be related to turning 30 and buying our first house, and it'll probably happen when/if we have kids and again when I retire. I suspect it's the same for anybody who's had traumatic experiences in their lives, i.e. 100% of the human population.

I started writing these posts for those of you, like Kim, whose kids have passed the crisis, and who are wondering "what now?" about things like self image and dating and all the soft psychological stuff that you can't really ask your cardiologist about. But I have to be honest and say that I was helped at least as much as anyone here by telling my story. Without realizing I was doing it, I gave myself permission to let go of a lot of my lingering guilt about being born as I was, yanking out that stupid vent tube when I was 18 months old, panicking that night when I was standing by the telephone, and all the other big regrets of my early life. I can't speak for all people with CHD any more than I can speak for all redheaded short chubby Librarians who watch too much Food Network. But in my case, having parents who encouraged us to share our feelings even when they were ugly or scary, who taught us resilience, and who encouraged us to achieve as much as our talents allowed helped me and my brother become strong, successful adults who work every day in our own ways to make the world better. And that, so I'm told, is the goal of every parent. I hope that hearing my story and Mom's will help in some way as you parent your kids. If you'd like to know more, we're no more than an email or comment away.

Thanks for reading,
Sarah