Growing up with a CHD: The good, the bad, and the…*interesting*

By Sarah Clark

Part 1: First, the good news

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.

Just in case you missed the point of this recitation of our nice little life in suburbia, let me make it clear: I lead a normal, happy, reasonably healthy life, and as far as I can tell, my life is pretty typical of adults with my defect (the symptom-free survival rate at 30 years post-correction is over 80%, and most of us had Mustards or Sennings instead of the new and improved arterial switch procedure). I’ve had no physical complications to speak of, and I’ve accomplished most of the modest physical goals I’ve set for myself. My CHD is merely one of a laundry list of things that are notable about my life. I’ve led a generally happy life (with a few bumps in the road I’ll hit in part two), I’ve graduated bachelor’s and master’s programs with excellent grades, I studied in Scotland during my sophomore year of college, and I’m the first TGA that I know of to climb (a very small chunk of) the Great Wall of China. The most important thing I want to share is that most of those things didn’t happen in spite of my defect—in a very real way they happened because of it. Let me explain with a brief list of the benefits of being born with a congenital heart defect.

1. Immortality? What immortality? 24 hours after my mustard procedure, I managed to disconnect myself from the ventilator, coded, spent the next month in a drug-induced coma, and was expected to wake up with major brain damage. At two years old it looked like I might be developing pulmonary hypertension. Pretty much every CHD kid I knew well before the age of 4 died of their heart defect—I believe I went to my first funeral when I was about 5. I never believed I was immortal or untouchable. That’s probably a big part of the reason I’ve never touched drugs or nicotine, drink no more than one or two cups of coffee a week, and consider a glass of wine with dinner a wild night on the town. (Well, that and I'm a nerd who grew up in the 80s…)

2. Carpe Diem: I don’t know my life expectancy. Some of the first patients to have surgically corrected heart defects are now in their 80s and the picture of health. Then again, there are people younger than me with my defect who are in heart failure and on disability. All those cheeseburgers I ate on the run in my dot-com days (and am still working off) could catch up with me tomorrow (or never). For that matter, I could get hit by a bus. I save as though I’m going to be one of the first TGA centenarians (which is the plan), but I don’t put off my dreams. If I find myself in a crappy job or relationship, I start working to get out of it. If I want to see the world I save up for a plane ticket and go. If I want the triple cheese decadence special, I eat it with very little guilt (this one’s a double-edged sword, though…) In a nutshell, everyone’s life is too short to put up with nonsense, but I can’t assume I’ll have as many “later”s as other people. And if I make it to that 100th birthday, I’ll have a LOT of cool stories to tell at my party.

3. Overachiever and proud of it: I play hard, and I work hard. I always have to have some sort of goal or project going, and while I’m no prodigy, I’ve accomplished a good bit in my first 30 years. I keep striving for the next accomplishment or the next refinement, which is a good thing in general but is another one of those double-edged swords. (again, see part two for the dark side of this) As a wee kid my cardiologist Dr. Razook told me that if I set my mind to it, I could accomplish anything. I was young/naïve enough to take him at his word, and for the most part he was right.

4. I don’t wait to tell people how I feel about them. Good or bad.

5. I feel an obligation to use my talents to leave the planet better off than I found it.

6. I don’t particularly care what other people think of my choices. I do a job most people would see as the height of boredom. I ignore people who tell me I need to watch some soap opera or “fix” some sartorial flaw of my husband’s. I march to my own drummer—in childhood because I didn’t know how to fit in as a bespectacled hyper-intelligent neurotic wimp, but later on because I didn’t particularly care to fit in. As I’ve gotten older, I’m more willing to wear the right outfit or schmooze with the right people to get to my goals, but the subterfuge is still only skin deep. And I don’t hide my brains for anyone.

To boil this thousand word description of the upside of TGA to its essence, I long ago realized that life was too short to put up with bullshit. I’m driven to make the most of my life, and I also care a lot about using my talents to help others improve their own lives. For the most part these are good things, and I wouldn’t change that for anything. However, that drive, and the drive of other adult CHD survivors that I know of, is powered by a decreasing but eternally present fuel of anxiety, perfectionism, survivor’s guilt, and by memories of the long-dead friends, acquaintances, and playdates that were no less deserving of the healthy, normal, and seemingly improbable life I enjoy today. Not only did I live while other children died, I live *because* other children died, and provided the knowledge needed to save the life of a chubby neurotic drama queen.

For better and worse, all of my life’s experiences have been marked by that essential truth. I don’t know whether that will be true for your child—but all I can do is share my experiences, good and bad, and hope that you can find some use for them in parenting your child. But remember—I still wouldn't change a thing.

Next week: The Bad.


Blog Note - Sarah is one of many people who I have befriended online from this blog. I hope you enjoy her perspective and experience as much as I do! Sarah has kindly agreed to provide a three-part series for LearnAboutCHD, and we can't wait for the next installment.

If you have a story, perspective or news item you would like to share, please email learnaboutchd@blogspot.com. The more we share, the more we learn and the more we can accomplish!

New Guidelines Urge Heart Tests Before Taking ADHD Drugs

Excerpts from article by RON WINSLOW
Wall Street Journal
April 22, 2008; Page D1

Children diagnosed with attention deficit and hyperactivity disorder should have a thorough heart work-up, including an electrocardiogram, before taking stimulants such as Ritalin to treat the condition, according to a new recommendation by the American Heart Association.

The advice marks the first time a medical-guidelines body in the U.S. has urged wide use of an electrocardiogram, which charts electrical activity in the heart, to screen a presumed healthy population for abnormalities.

But there is debate among experts about the value of using the test to screen such a large pool of patients to detect a rare condition. The hope is that such a test -- in combination with a comprehensive checkup -- will help to avoid the rare cases of sudden cardiac arrest that have been linked to the widely used medicines.

AN ADHD CARDIAC CHECKLIST

A child diagnosed with ADHD should get a thorough heart checkup, including:

• Patient and family history, with special attention to palpitations, fainting or recent difficulties during exercise.

• A physical, including blood pressure and a check for abnormal heartbeats.

• An electrocardiogram to measure electrical function of the heart.

• If necessary, a consultation with a pediatric cardiologist to discuss important findings.

Source: American Heart Association

"The goal is to improve the care of children, including allowing them to have their ADHD treated and have it done safely," says Victoria Vetter, director of electrophysiology at Children's Hospital of Philadelphia and lead author of the heart association's statement.

More...

New Grants Available for Families Struggling With Child Health-Related Expenses

MINNEAPOLIS (April 10, 2008) – UnitedHealthcare Children’s Foundation (UHCCF) announced that new grants are available to help children who need critical health care treatment, services or equipment not covered or not fully covered by their parents’ health benefit plans.

UHCCF provides grants to families to help pay for child health care services such as speech therapy, physical therapy, occupational therapy sessions, prescriptions, and medical equipment such as wheelchairs, orthotics and eyeglasses.

Parents and legal guardians may apply for grants of up to $5,000 for child medical services and equipment by completing an online application at http://www.uhccf.org/. Tax-deductible donations can also be made online.

To be eligible for grants, children must be 16 years of age or younger. Families must meet economic guidelines, reside in the United States and be covered by a commercial health benefit plan. “We are taking action to improve access to health care in America and are dedicated to helping more children and families this year and beyond,” said Matt Peterson, UHCCF president. “We encourage families who need assistance paying for their child’s medical needs to visit the UnitedHealthcare Children’s Foundation Web site and apply today.”

The foundation aims to help more children by increasing awareness of the foundation through fund-raising events, partnerships, newspaper and Web advertising, and a revamped multimedia Web site, www.uhccf.org/.

About UnitedHealthcare Children’s Foundation
The UnitedHealthcare Children’s Foundation is a nonprofit 501(c)(3) organization that strives to enhance either the clinical condition or quality of life of children who have health care needs not fully covered by commercial health insurance. Foundation funding is provided by contributions from employees of UnitedHealth Group as well as individuals and corporations. While UHCCF receives contributions from UnitedHealth Group and its employees, individual and corporate donations to help provide assistance are deeply appreciated. To donate or learn more, please visit http://www.uhccf.org/.

CHD and Neurodevelopmental Risks

Risk of attention and behavior problems for kids with CHD
Children's Hospital of Philadelphia
Public Release, 7 April 2008

Schoolchildren who required surgery as infants for congenital heart disease (CHD) run a significant risk of having problems with inattention and hyperactivity, and often require remedial services in school. “These children are at risk for academic and behavior problems, and our findings reinforce how important it is to provide them with ongoing follow-up and neurodevelopmental screening,” said study leader Amanda J. Shillingford, M.D., a pediatric cardiologist at The Children’s Hospital of Philadelphia.

The researchers, who reported their findings in the April issue of Pediatrics, studied a group of 109 children, aged five to 10, who had undergone cardiac surgery for complex congenital heart disease at Children’s Hospital when they were newborns. Of that group, 53 children--nearly half of them--were receiving remedial services at school, and 15 percent were in special education classrooms.

Based on questionnaire responses from their parents and teachers, although the majority of the children with CHD scored in the normal range, the rates of high-risk scores for inattention and hyperactivity were three to four times greater than those found in the general population.

Previous studies at The Children’s Hospital of Philadelphia and other centers found that school-aged children with complex CHD tended to have normal cognitive abilities but were at risk for problems in visual and motor skills, as well as impairments in speech, language and executive functioning (executive functioning refers to capacities for attention, planning, decision-making and problem-solving).

“As survival rates have improved,” said Shillingford, “the important longer-term issue is quality of life for patients and their families as they reach school age and beyond. We hope our findings will help raise awareness among parents, teachers and physicians about the children’s risk of neurodevelopmental problems.” Shillingford added that a next step for researchers is to conduct larger, multicenter studies, with more formalized diagnostic tools, and to develop formalized follow-up protocols for these children. Such follow-up programs are currently being designed at Children’s Hospital.

New Techniques for Stenting in Adults with CHD

A pioneering paper was presented at last week’s Society of Interventioanl Radiology meeting, in Washington, DC by Dr John Moriarty, Specialist Registrar in Dublin’s Mater Hospital. The Mater is the only place in Ireland and one of a few places in Europe that places pulmonary valves in adult patients via stenting. This procedure, which is not yet licensed in the US, was examined with CT during Dr Moriarty’s presentation.

Moving beyond diagnosis and into therapy, interventional radiologists have new imaging techniques and new ways of getting into organs which, when combined, give an extremely powerful new tool that is changing the course of care for patients, in quality as well as quantity of life.

Posted in Research and Education on 29 March 2008