Caring for and Empowering your Child

Ultimately, no one knows how to better care for your child than you do. As a parent, you have instincts, passion, and most of all love – all these things mean that you are perfectly capable of giving your child the best care possible. However, when you learn that your child has been diagnosed with a CHD, most parents can’t help but wonder, “How do I care for my child?” We all second-guess our parenting abilities from time to time, but when you have a child with extra needs, we may find ourselves second-guessing our actions even more. Here are some specific things that parents of children with a CHD should consider when providing care for their children.

Although many of the day-to-day tasks such as special feedings, looking out for signs of problems or complications, or administering medicines actually become like second nature and relatively “easy” over time, other issues within the realm of caring for our children are what cause us to doubt ourselves. One of these issues involves how to treat our children when it comes to addressing their illness. Whether your child is diagnosed with a CHD in infancy or later in childhood, you’ll have to explain to him or her things such as why they have a surgical scar, why they need to visit the doctor/hospital more frequently than some other children, why they must take medication every day, etc. Many parents think they can’t handle or shouldn’t address these issues; however, it is by addressing these issues and having these types of discussions with your child that makes your care for them even that much better.

Besides communicating with and educating your child, the main advice that numerous child psychologists and doctors will give regarding this issue is to encourage your child to become involved in their own care (when/if they are emotionally and physically able of course). This makes children feel much more confident and in control – children will most likely think, “Wow, if Mommy and Daddy trust me to take care of myself I must be doing great!” It’s a great tool to empower your child and yourself. Depending on the level of care your child requires, the doctor or other members of the medical team can give you specific instructions regarding how to incorporate your child in the caretaking process. And at the end of the day, involving your child in their own caregiving is the best way you as their parent can care for them as well.

Never Give Up

An article published by Associated Press today left me dumbfounded. It reminded me to never, ever give up hope. You've GOT to read this article in its entirety (see summary below). See if you feel the same...

British girl's heart heals itself after transplant

Excerpted from Maria Cheng, Ap Medical Writer – Tue Jul 14, 12:40 am ET

In 1994, when Hannah Clark was eight months old, she developed severe heart failure and doctors put her on a waiting list to get a new heart. But Clark's heart difficulties caused problems with her lungs as well. To avoid a risky heart/lung transplant, doctors took a chance at a new and novel idea.

Sir Magdi Yacoub of Imperial College London thought that said that if Clark's heart was given a time-out, it might be able to recover on its own. So in 1995, Yacoub and others grafted a donor heart from a 5-month-old directly onto Clark's own heart. After four and a half years, both hearts were working fine, so Yacoub and colleagues decided not to take out the extra heart.

However, the powerful anti-rejection drugs Clark was taking caused cancer, which led to chemotherapy. Even when doctors lowered the doses of drugs to suppress Clark's immune system, the cancer spread, and Clark's body eventually rejected the donor heart.

In February 2006, doctors removed Clark's donor heart. Luckily, by that time, Clark's own heart seemed to have fully recovered. Since then, Clark — now 16 years old — has started playing sports, gotten a part-time job, and plans to go back to school in September.

At the moment, doctors aren't sure how that regeneration happens. Some think there are a small number of stem cells in the heart, which may somehow be triggered in crisis situations to heal damaged tissue.

Granted, Hannah's case is a rare and miraculous one, but it does give us a window of hope for the future!

Side note - even if this were to become an option for treatment in the future, there is a serious shortage of donor organs available for such transplants. Consider becoming an organ donor today, and asking your loved ones to do the same!

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.

Growing up with CHD: Part 2

Part 2: And now the Bad (or at least challenging) Stuff

By Sarah Clark

First, my apologies for the length—as somebody once wrote, if I'd had more time to edit I would have made this piece shorter. Second, you're going to get tired of hearing this, but growing up with CHD was a relative non-issue, especially when I compare myself to people who suffered various types of abuse or neglect. However, that's not to say CHD wasn't an issue at all.

The first 10 years of my life were hard. About 3 years after my medical drama, my dad started having seizures, and it took several brain surgeries, countless drugs, and ten years of searching to find the cause and get them under control. (And he worked full time during all that!) So much happened to my family in my early life that it's hard to say what caused a given problem, and what just made it worse. However, the ones I'm going to focus on the issues that were there before my Dad got sick. I'm also glazing over some of the obvious fears you may have for your kids like body image and gym class traumas, because their effects aren't that much different or worse than any other kid's psychic bumps and bruises, and are treated in the same way. I'm a deep thinker, so I'd rather focus on the Deep stuff I faced, and that your kids might face as well.

Guilt:

The summer after my 3rd grade year, Mom and Dad went to UCLA for his big brain surgery. We shuttled between several relatives and family friends back in Oklahoma, who each gave us varying amounts of attention and affection. I could probably write a book about the experiences of that summer, but for the purposes of this article, I'm going to stick to the "Blacks". The Blacks were one of a few families my folks had kept in touch with from the CHD days. Their oldest, "Amy", had been a couple years older than me, and hers was the first funeral I attended (about 5 years prior to the summer we spent with the Blacks). She looked like something from a fairy tale with her blue eyes and blond curls. We'd played together some, but really all I remembered was that she was pale and kind of bossy. The Blacks had two younger kids whose ages roughly corresponded to me and my little brother Daniel, so we all got along well, and spent the several weeks we stayed there romping in the backyard and doing Kid Stuff. The only weird thing was the staircase.

The wall alongside the stairs to the second floor was a shrine to Amy. Every morning when I ran down to breakfast, I was torn between averting my eyes from and gazing in fascination at the dozens of photos, certificates, and plaques that lined the walls. Somehow I felt ashamed looking at them, as if I had no right to feel a slight pang of envy for the cute girl who'd gotten to be in front of so many TV cameras and meet so many interesting people in her short life. One afternoon I came back to the house when I got pooped from too much racing on my bike. I came in the back door and grabbed the first book I came across (I left books in my wake like Hansel and Gretel left breadcrumbs), and flopped down in Mr. Black's squashy armchair to read for a bit. I heard Mrs. Black's voice from the kitchen, and realized she was on the phone. Being 9 and nosy, I did the best I could to listen in. I can only assume she didn't hear me enter.

"She'd be thirteen soon…I know God wanted her because she was so special. She's probably the prettiest angel in His garden…," Mrs. Black continued, sharing her dreams for her late daughter to the person on the other end of the line. I sat in the chair, unseen, wanting to throw up. Maybe it was my carefully suppressed fear that I'd never see Dad again, or simply the fact that I was getting older, but for the first time in my life, her overheard words caused the full implications of the eternal question "Why?" to hit me like a ton of bricks. My mind whirled with rage at God, confusion at Mrs. Black's comments, and a strange dark tug at my soul that I eventually recognized as guilt. Did this mean that I was more special than Amy—which made no sense—she was angelic (when grownups were looking) even when she was alive. Or did it mean that I wasn't special enough? Had I done something bad to be born sick, or worse--was my survival some sort of bargain that made Dad sick? Had I made Amy die by praying to God to get back at her for pulling my hair? Her defect hadn't been that much worse than mine--why save a nearsighted geeky clutz with mouse-brown hair and no social skills? Why me and not Amy?

Nearly 25 years later I still have no clue on that one. If somebody here figures that one out, please let me know. It's certainly one of the strongest arguments I've come across for the non-existence of God.

Anxiety:

I've had panic attacks, generalized anxiety, and phobias of various types and degrees pretty much since the day I woke up from my mustard procedure and the ensuing complications. Call it PTSD, brain damage, damaged seratonin receptors from spending so much time on the heart-lung machine--ultimately the cause doesn't matter. Store closings, unfamiliar doctors, elevators, Bs on report cards, any of those things could and did send me into panic attacks at various points in my life. Think twice before you put ET in the DVD player for the first time—I was 4 when I saw that in theaters and I went into a full-tilt inconsolable shrieking meltdown when ET "died". In fact, the only thing I seriously regret in my life occurred because at a moment when someone I loved needed my help desperately, I had a panic attack.

It was a week or two before Christmas. Mom was out for a "meeting with Santa's elves", and Dad was reading us a bedtime story in my brother's bedroom. He finished the story, closed the book, and stood up, preparing to tell me to brush my teeth and go to my bedroom. Then his voice trailed off, and he got a real funny look in his eyes, like he was staring 100 miles into the distance. Then he collapsed into convulsions, and knocked himself unconscious on Daniel's toy piano. Daniel (who was no more than a year old) started screaming, and I swung into action. So what if I was 5? I knew what to do, 799-HELP, I had the phone in my hand, I started dialing—and I froze.

All of a sudden I couldn't breathe, my heart was pounding a million miles a minute, and I started thinking a million different things—what if Dad was dead, and I was supposed to be doing CPR? Would the paramedics be mad if I called and he was dead and I'd wasted their time? What if dad woke up, and then the paramedics would really be mad because it wasn't a real emergency? Would they say "aw, you're just a kid" and hang up? No matter how I tried, I couldn't dial that phone. I hung up and crawled into bed weeping until Dad came to from his first seizure. Dad, of course, long ago told me there was nothing to forgive. I hope one day I can figure out how to be that generous to my younger self.

Since that night 25 years ago when I learned what my panic could make me do (or not do), I've fought a ruthless pitched battle against my terror in all its forms. It's been a foe more formidable than my circulation, by far. It's not just the sweaty palms and the 3 AM catastrophizing over whether I CC'd the right people on an email or if I paid the light bill. The person I loved most in the world needed me, and I failed him. I've come to terms with that (at least intellectually), but it will always gnaw at me, and give my anxiety the most potent ammunition possible when I try to fight it down. I have beaten it down to a dull murmur, but I will always have to be on the defensive--it often sneaks up when I least expect it.

Perfectionism:

Guilt + Anxiety=Perfectionism. Good enough is never good enough for me—I had a serious depression in my early teens and one of the greatest things that fed it was that I simply lacked the energy or motivation to do homework because I was brooding over my "deformed" body, my sick father, or the heavy existential questions mentioned above-- or hiding from that brooding via compulsive reading. That of course lowered my grades, made me feel worse about myself, and the cycle continued down for several years before I pulled myself up through therapy and sheer force of will. The perfectionism remains though—it took me 24 hours after the greatest physical success of my life, climbing the first third of the Great Wall of China at Badaling, to stop berating myself for not training hard enough to conquer the whole thing and to take pride in what I *had* accomplished. I've mellowed a lot since my "emo" teen years and become more balanced as I've matured. I've learned to use that impulse for good--it's certainly helped me out in my career.

The Kid Thing:

"Sarah, It would be a very good idea if you decided not to have children. Pregnancy could be very dangerous for you."

That was NOT the reaction I'd expected when I told Dr. Razook about my first serious boyfriend! I'd expected him to grin, remind me he'd told me so when I'd worried over my scars making me unattractive in previous checkups, and generally play the proud if protective surrogate dad he'd been through my childhood. Instead he asked to talk to me without mom in the room, asked a few delicate questions about what "physical contact" we'd done ("Peter" was president of the Bible Club and Math team and I was barely 16—we were both too nerdy and too scared of eternal retribution to try very much), and then Dr. Razook hit me with that bombshell. Peter and I parted the following year, but I will always remember how he came right over that night when I called, and held me as I cried, telling me that he still thought I was just as beautiful as before. Peter had his faults, but I've held every man I've dated to that standard when I've told them my medical history and its implications. I've been married for 6 years to a man who passed that test with flying colors.

Everyone's situation is different. People with TGA can and do decide to have children, and 15 years on I realize Dr. Razook's blanket disapproval was probably mostly his extra-cautious nature, and partially his impulse to slow down a teenager who was in too big a rush to grow up. However, I've read the studies, talked to people who've had both good and bad outcomes, and I came to the same decision. I feel it would be unethical to knowingly put a child at increased genetic risk of a CHD, and considering that a pregnancy might well complicate my uncomplicated outcome, I will NOT subject my child to growing up with a chronically ill parent if at all possible. Your kids' mileage may vary. Remember that "anxiety" stuff above? I'm a devout believer in Murphy's law.

Being a "miracle"

I'm sure you say it all the time—you've probably said it to your kids, or your friends, or had others say it to you. "You're a miracle". "Her recovery was miraculous." "God blessed us with a miracle". In the sense that at least one event happened to me that could not then be fully explained by medical science, I am a "miracle". I learned the story of my early life, surgery, and (at least metaphorical) rebirth as a wee child, right alongside Cinderella, Snow White, and Sleeping Beauty. My defect was never something to be ashamed of, and my parents told me to wear my scars proudly. From early on I knew I was destined for Great Things—God had saved my life, had He not? (I was still a little young yet to ponder either the "Amy Counter-argument", or wonder Who had been in charge of creating the situation where my life needed saving in the first place)

In addition to having a rather more dramatic origin story than most, I was intelligent. Scary, precocious, read off the menu at the restaurant when I was 2 intelligent. (apparently when the waitress complimented me on my literacy, my granny whipped up my dress to show her my scars, to my parents' horror…). Between those two things, every adult in my life was CONVINCED that I was going to do something amazing with my life—and from my earliest days through 3^rd grade I complied. When maturity, hormones, and the events of the first decade of my life finally slammed into me as mentioned above, not only did I feel depressed for those reasons, my despair deepened because I wasn't living up to what I was Supposed To Do with my life, the mysterious, still-unknown reason I had been saved and made a Miracle.

When I clawed my way out of my funk in my mid-teens, I set myself to figure out what Great Work I had to accomplish to pay back the life debt of my miracle. I put myself under a lot of pressure and agony, and it was definitely a big part of the perfectionism. In recent years I finally got mature enough to throw up my hands and essentially say "God, if you exist and I have an outstanding bar tab, you're going to have to send me the bill via some sort of signal I'll understand. Otherwise I'm just going to muddle through the best I can, and try my best not to feel guilty over that." I've yet to get a bill, so I assume I'm doing fine.

As for the "miracle" thing? As a kid, I wore it with uncomfortable pride, like an Easter dress that was too itchy with starch and made me want to change back into anonymous jeans and a t-shirt. As a teen and young adult I hid from the descriptor or rejected it outright. Now? I don't even know if I still believe I'm a miracle (sorry mom), much less what I think about what that implies. I really am uncomfortable with and to an extent resent all the baggage that has come with being slapped with that 7-letter albatross. Then again, one doesn't get to pick and choose the good and bad in life. If not for the "Miracle" label…Would I be this determined, or this intelligent? Would I be wasting away in a job I hated instead of doing something I love? Would I be living such an (over)examined life?

The point to all of this:

I hope I haven't scared you too much, or convinced you that your children are doomed to a life of brooding and anxiety. Remember Part 1: I am so strong, and lead such a wonderful life in large part BECAUSE I faced these demons at an early age and came out whole on the other side. I don't mean to sound like a Pollyanna, and it is very likely that your children will need your help and possibly professional help in answering the very adult questions and fears that surround their very existence. Fortunately I had that. My mom practiced desensitization therapy long before it was in the books by gradually and carefully exposing me to my phobic triggers until they no longer set me off. Dad is a living example of how one can have a chronic medical issue and still live a productive and fulfilling life. My parents considered no topic taboo, and helped me talk through a lot of my early struggles. At crucial moments they got me to therapists who helped me sort out my thoughts and fears. My parents are the real heroes of this story—in my most honest moments I know that I'm afraid to have children because I simply could not do what they did—and what you do now. And while I feel somewhat unqualified to suggest things to you all, simply because I'm not a parent of a child with a heart defect, I'd like to wrap up this series next week with a list of Dos and Don'ts for raising a kid with a CHD. Hopefully I can talk Mom into co-writing…

Development of the Heart

A special thanks to Josie, the mother of a little girl who was has the CHD known as Transposition of the Greater Arteries (TGA), for today's post!

When you stop to think about the process of fetal development, really think about what is happening every day, it's absolutely breath-taking. The heart is one of the very first organs to form, in fact, it begins to beat often before the mother even knows that she's pregnant, within 21 days of conception. Congenital heart defects thus occur in the first days and weeks of existence; by the time the heart begins beating, most structural anomalies already exist. Depending on the particular defect, that may only be the beginning.

Although the structures of the heart exist from the earliest days, the heart itself continues normal development throughout the pregnancy. The flow of blood through the heart furthers growth and development of the various structures. When there is a structural anomaly, the blood may follow an unusual path through the heart and thus exacerbate the defect. Other defects result not from structural problems, like our daughter's, but rather from problems with the valves in between each section of the heart. Valves are critical to normal cardiac functioning because they prevent the backwards flow of blood as the heart contracts to pump blood to the body and the lungs. In order to function properly and efficiently, the flaps must be thin and pliable and should open completely and close completely. When they don't work, either because they are too small and restrict the flow of blood through the valves, or because they are weak and leaky, the development of the fetal heart may be seriously impacted. Problematic valves may prevent entire chambers of the heart from growing and developing.

But I digress. The point of this post was to explain that congenital heart abnormalities exist from the earliest days of the pregnancy. And no one knows why. No one knows what triggers the incorrect formation or when exactly development goes awry. Sometimes, although very rarely, there is a genetic component. The vast majority of congenital heart defects have an unknown cause and therefore we don't know how to prevent them. Seriously, when you stop to think about it, a normal heart is really quite amazing!