s National CHD Awareness Week! This year we will be posting entries daily, once again - 7 entries for 7 days.“I am 100% positive that in the long run we will have an integrated, high quality care system for all survivors,” Amy says in the interview. “Our really long term goal is to have a very unified system with research, a registry, a way to quantify outcomes, all the centers talking to each other, sharing information and building knowledge together...”.
Ms. Verstappen is determined to educate parents on the long-term needs of CHD patients. She tells Steve that she “...presented a pilot version of [a new brochure designed by ACHA specifically for parents] to a group of Heart Moms - all leaders of some of the advocacy groups for children - three of them told me that no one had ever told them that their child was going to need this level of care.” Amy goes on to describe some of the questions that parents of children with a heart defect should be asking.
I can tell you, as one of those moms who didn't know or understand the long term implications (I have a child with Transposition of the Greater Arteries who I was told was "fixed!"), that the information provided is valuable and too important not to take a moment to read.
I highly encourage you to take a minute and check out the latest entry at Adventures of a Funky Heart! Not only is it a great personal story of one of the movers and shakers of the CHD community, but it is also a great source of information for parents, teens and adults alike!
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.
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 3rd 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…
I’m probably not very different from some of the younger parents who follow this blog. I’m a 31 year old living in Northeast Oklahoma with my husband of 6 years and our cat, Tawnya. We’re moving into our first house in about a month, and we’re looking forward to filling up our new bookshelves, setting up a whole-house wireless network, and starting a proper vegetable garden. I’m a librarian at a local university, and I spend my days keeping up with overdue notices, teaching information literacy classes, and helping our online students get the research help they need for their classes. I was born with Transposition of the Great Arteries, VSD, and PDA, all corrected by the time I was 18 months old. My husband Kevin is a software developer. We walk a few times a week (when we’re being good), and attempt to eat a reasonably healthy diet. We hang out with friends, spend too much time online, and could really stand to clean out the bedroom closet this weekend. All in all, we’re your typical middle class DINK couple, though perhaps a little geekier than most.
When my mom had Jameson, I was very sad. My Aunt had to lie in bed with me sometimes at night and talk to me about it. My Aunt and uncle were watching me and my sister while my parents were gone. When I came to see Jameson at the hospital, I was sad when I saw the stuff they had on her. But I was very happy to see my parents and Jameson. 
On September 4, 2006, my daughter Sadie entered this world with a bang - 17 minutes after we arrived at the hospital. She came out screaming, but immediately turned blue. Six hours later, she was diagnosed with Transposition of the Greater Arteries, ASD and VSD (holes in the heart) and pulmonary hypertension. During her first days, the actions of complete strangers were what kept her alive. So today I'd like to share a personal 7 for 7...
Misty's Story. My CHD story begins on New Year’s Day, 2005 when I found out I was pregnant with my 3rd child. My pregnancy was pretty normal. All of my ultrasounds were fine and there was no cause for concern, so we thought. Sydney was born on August 24, 2005. Her APGAR scores were 9 and 9. Everything looked good until the pediatrician came in and checked her out and heard a murmur that was “slightly louder than what we like to hear” and called in a pediatric cardiologist, Dr. Albrecht. At 4:30 pm that afternoon, Dr. Albrecht came into my room with a nurse. I was there by myself taking a nap and waiting for my newest little girl to be able to come to my room and get to hold her. I’ll never forget what he said. “Your daughter has a problem with one of the valves in her heart and I have called a transport team from UVA Medical Center to come and pick her up and transport her there. She will have surgery tomorrow morning.” Sydney has Aortic Stenosis (AS). Dr. Albrecht advised me that her Aortic Valve was a bi-cuspid, instead of a tri-cuspid, valve and was barely functioning and letting very little blood out of the valve to the Aorta. After he went over everything, he sent the nurse to bring Sydney to my room so I could say my “hello’s and goodbye’s”. My husband called his parents to go pick up our other 2 children and bring them to the hospital to meet their new little sister before they took her an hour away to prepare her for the surgery the following morning.
The next morning my husband called to let me know that they were getting ready to take her back and that she had so many tubes and wires coming out of her that the only place that you could touch her was on her head. At that point, he started to break down, again, and I began falling apart, again. Two hours into her procedure, we received the news that everything went well, she had had two blood transfusions, and she was going to be ok.
When I was finally released from the hospital and able to go see my daughter two days after her birth, I found her in her little bed with an intubation tube and many tubes and wires attached to her. Since that day was my birthday, Steve’s parents brought our two older children up to the hospital to see their sister. I didn’t want them to see her with all of the tubes and wires, but someone brought them back before I could say anything. What was amazing was that when she heard them start talking, her heart rate stabilized, her respiratory rate normalized and her blood pressure returned to a normal state. It was incredible.
Today my daughter looks like a normal 23 month old, but she is not a normal little girl. There are things that she will have to go through that ‘normal kids’ won’t have to do. Besides the future surgery(ies) to open and eventually replace her aortic valve, she won’t be able to do some sports that she may want to do and she will, most likely, have to take medicine every day of her life. Her heart will never be normal.
Joshua's Story. Joshua is an active 4 year old. He was born in June of 2003 with hypoplastic left heart syndrome, a rare and fatal defect, unless treated. Basically, he was missing his left ventricle and his aorta was very narrow. Parents of children with this defect are given three options: they can take their child home to die, they can try for a heart transplant in hopes that a heart will become available soon enough, or their child can undergo three surgeries that will reroute the blood so that the child can survive. Jodi and Mark, Joshua's parents, opted for the surgeries.
Christy D' s Story. My husband were excited when we found out I was pregnant with my second child. Then our world came crumbling down at our 21 week ultrasound; Steve and I were told our little baby girl had a severe and life threatening heart condition. Instead of joy, we were expecting, our hearts were filled with pain, fear and grief. The baby was diagnosed with Tricuspid Valve Atresia (TA)---meaning her tricuspid valve did not form. Without her tricuspid value, her blood was unable to flow into her right ventricle, enabling growth; meaning she has a 3-chambered heart.
Karen's Story. As I lay on the exam table and the ultra sound tech squeezes some warm gel on my expanding belly, I look over at my two daughters who are 4 and 7 and squeeze my husband’s hand. We are all eager to find out the sex of our newest addition to the family. With two girls already, we were eager to find out if Daddy will get a little boy. The baby is uncooperative. The tech is unable to tell the sex. Our excitement elevates. The tech steps out to get the doctor. Soon the doctor comes back. He looks at the screen for a long time. I start to get a sick feeling in my stomach. I squeeze my husband’s hand harder. The sweat is building between our tightly gripped hands. His panic is beginning to flow to me threw his hand as though our bodies were connected. The doctor speaks…. “This is not good. This is bad.” I truly feel like I am going to faint. I look over at my girls who have started to look at a Dr Seuss book and have forgotten about what we are there for. I think to myself that they understand “This is bad.” Get them out of here. I don’t want them to see me cry. Oh my God! Oh my God! The tech takes my daughters out of the room to the waiting room. The doctor tells us that the baby is missing part of its heart. Missing half it’s heart! What does this mean? Is the baby going to be able to live?
Harlie is now 16 months old and has just started to crawl. She has a 20-word vocabulary in sign language. She breathes through a tube in her throat, called a trach. She eats through a tube that was surgically placed in her stomach. She’s had seven surgeries and has spent over four months in hospitals and she’s just getting started.
This post is a little personal, but something I was inclined to write after the last few weeks of complete submersion in the CHD community. I found that the very same things that have changed so drastically in my outlooks and life are shared by many families with CHD. Here are the top seven things that came to mind as I thought of how our lives have changed forever.
