Monday, August 26, 2013

Life After Death

When you lose a child to a heart defect, do you dare to try again to have another child? What are the odds of having a second child with the same heart defect? With 35 different identified heart defects, what is the possibility of a sibling contracting one of the other kinds of heart defects? Is it worth the risk?

These are the questions that families must ask themselves when they have a child born with a congenital heart defect. My husband and I decided, before we married, that we wanted two children. Joey was born in 1991 with a healthy heart; Alex was born in 1994 with hypoplastic left heart syndrome (HLHS). The doctors told us to take Alex home to love him for what little time he had left. We said that wasn't an option for us. Instead, the doctors performed lifesaving surgery on Alex. After Alex's first open-heart surgery our son's surgeon recommended we have another child. We told the doctor we only wanted two children. His advice didn't change. We chose not to get pregnant again. Alex just turned 19 years old on August 11, 2013.

What if we had decided to have another child?

That is the situation Jayson and Crystal Darak found themselves facing after their first child died of HLHS. What is the recurrence rate of having another child with a congenital heart defect? How does a couple prepare themselves for possibly having another child with the same or a different kind of heart defect?

Over the last 19 years I've talked to parents about this very issue over and over again. Some choose to have genetic counseling to try to determine if there is a genetic component involved with the heart defect their child had. Some have thrown caution to the wind and have told me that if they had another child with a heart defect, they knew they could handle it; they had survived it before. I've even known families who have intentionally adopted a baby or child with a heart defect. The reactions have been varied and have had various results.

I've had people ask me if I know of any families who have had more than one child with a congenital heart defect. I've had people ask me if I've ever known of a family having more than one child with HLHS. The answer to both questions is "yes." I've even known of one mother who lost a child to HLHS and then over a decade later gave birth to another child with HLHS. The second child survived. The mother couldn't believe it could happen a second time.

It can happen a second time, but the odds are very small. With about 1 in 100 children being born with a heart defect, while they (CHDs as a group) are considered THE most common birth defect, it's still uncommon to have a baby born with a heart defect. So what are the odds of having a second child with a heart defect?

According to an article in the Journal of Pregnancy in 2011, the odds of having a child with a heart defect, if you've already had one child with a heart defect, are higher than professionals previously thought. According to that article "Total recurrence rate of CHD was 3.98%, 4.06% in single familial risk, 2.9% in double, and 5% in multiple risk. It was 3.5% in case of one previously affected child; 4.5% with 2 children; 5.2% with the mother alone affected and 7.5% with father alone affected and 3.5% with a single distant relative." ( One has to be careful when looking at the literature and overgeneralizing. I found one article in Circulation, a journal published by the American Heart Association, but the study was conducted entirely in Denmark. It's relatively easier for researchers to gather data like this from countries who keep medical records on all of their citizens due to socialized medicine, but can we necessarily rest assured that those data correspond to citizens in the United States? Oddly enough, the statistics described in the Danish article ( actually corresponded to another article I found published by British doctors who investigated recurrence rates based on referral rates for fetal echocardiography after a family had one child with a CHD (

What about HLHS specifically? I found one article specifically addressing this issue. It reported an 8% recurrence rate (, which oddly enough, actually relates perfectly to the Danish and British studies. But while this number is scary, it also means that there is a 92% chance that a family with a child with HLHS will have a heart-healthy second child.

Is it worth the risk? For the Darak family, it appears it was. The news article about the Daraks ( was a joyful one for me to read. Fetal echocardiography reported that their second child had a beautiful, perfect heart. This actually corresponds to my own experience. While I haven't actually gathered data and done a scientific analysis, of the heart families I have met in person, at the hospital, over the Internet (thanks to Facebook, AOL chatrooms and Yahoo groups), most families have only one child with a congenital heart defect.

Each family has to decide for itself whether or not risking having a second child with a congenital heart defect is a good idea. Each family knows its support systems, what medical assistance is available, the quality of the insurance available to the family and whether or not a family can emotionally handle whatever the result may be. For the family in the news article cited above, getting pregnant a second time meant having a second chance at parenthood. It meant being the parents of two children. It means valuing life over death.

Monday, August 19, 2013

Congenital Heart Defect Radio Show

Are you ready for some exciting news? Last week I (Anna Jaworski) received a phone call from an executive producer of a radio show asking me if I would like to have my own radio talk show! He had found my website and saw that I was passionate about congenital heart defects and he felt this was a topic that deserved its own, regular radio show!

Within two days I had put together a proposal for a radio show that would feature segments of interest to the Congenital Heart Defect Community (CHD community). I am thankful that I have so many Facebook friends who are members of the heart world. So many wonderful people stepped up and suggested topics that interested them. I was thrilled to see that all of the suggested topics fit into one (or more) of the segments I proposed for the radio show. I felt I had a lot of support from the CHD community when the executive producer called me back to say that the network approved the proposal!

I requested references so I could speak to other radio hosts. I'm hopeful they will share some information with me and help me to start this show off on the right foot. This is such an important topic to have on the radio. We have so many issues that need to be addressed globally. Our audience needs to feel united. I'm afraid that we have been fragmented for far too long.

Now we go into the preproduction phase of the radio show. I have a lot to learn but this radio network, VoiceAmerica, believes in us. This isn't my radio show -- it's our radio show! The executive producer even told me that he believed that finding out about the CHD world and then offering us a chance to share information globally was a mitzvah - a good deed. The network feels good about having an opportunity to give something as vital as communication to a world that so desperately needs communication, love and support. While talking to Josh Bernstein and Brandy Jackson (the executive producer and general manager, respectively), I felt a flood of compassion and enthusiasm for our project that gave me the confidence to take on this challenge. I've been on radio shows and even a couple of television shows for small segments but I've never hosted a regular talk show before. I'm extremely excited and humbled by this opportunity to help bring awareness and advocacy to our very important cause.

How can we spread awareness of congenital heart defects -- the number one birth defect? We can spread awareness in so many ways -- blogging, setting up Facebook pages, websites and using other social media to let people see us, hear us and associate a face, a person, a story to the cause. We need to talk about the radio show, generate excitement and show support for one another.

For too many years we've felt alone as we've dealt with surgeries and uncertain futures. For too many years many of us wondered if anyone cared. I distinctly remember being in the hospital with Alex as he was recovering from his first open-heart surgery. I looked out his hospital room window on a busy hospital parking lot and watched people coming and going. Everyone seemed in such a hurry as they scurried about. I meanwhile stroked my son's head and kissed him knowing all the while that nobody seemed to care that there was a boy in that building fighting for his life. It was an extremely lonely feeling. I wondered how people could just go about their daily lives unaware of the drama unfolding in rooms around me. Babies were being given a chance for life. Some of them made it; others didn't. It was a heartbreaking environment and I felt like I was fighting the world alone just for another breath, a smile and the whisper of hope for a future for my son.

This radio show gives us all a chance to reach out to one another. We can share our stories, we can educate each other about topics of importance in the congenital heart defect world. We can let other people who don't even realize they've been touched by CHDs know we're out here! We can provide hope, encouragement and support to people all over the world.

Thank you, VoiceAmerica! You have indeed done a mitzvah and I, for one, feel blessed by this opportunity. Let us hope that this is just the beginning of something special for us all.

Monday, August 12, 2013

Hospital Controversy

The heart world is up in arms due to controversy surrounding a hospital in Kentucky. No question about it, there's something amiss at the University of Kentucky Hospital. But what does this have to do with the heart world?

The University of Kentucky Hospital is not well known for its treatment of congenital heart defects but the issue of wrong doing -- especially the allegation that unnecessary heart procedures were conducted -- causes fear and concern in us all. Even if a heart child doesn't receive treatment at a particular facility, many of us have friends around the globe and the concern is that something of this magnitude could affect one of our friends.

But there is a bigger issue at hand here and that is a question I am frequently asked:  Knowing what you know now, would you still take your child to a hospital which is not nationally rated for its treatment of congenital heart defects?

This issue is usually addressed most by parents who have found out in utero that their baby will be born with a severe  congenital heart defect. Where should they go for treatment? Is there one "best" facility? What about family support? How long will they be away from home? What about follow-up treatment?

This is actually a HUGE issue! There are people who say that parents should only take their children to facilities like Boston Children's Hospital, Children's Hospital of Philadelphia or Mott Children's Hospital. Christus Santa Rosa or University Hospital (in San Antonio), where my son had his surgeries, are never on the lists for "best hospitals for congenital heart defect care." Knowing what I know now about the delicacy of the surgeries my son endured, would I still take my son to a lesser-known hospital?

Actions speak louder than words. My son had his first and second surgery at University Hospital in San Antonio with the brilliant surgeon John Calhoon, M.D. in 1994 and 1995, respectively. His third surgery came 16 years later, after many years of research, and that surgery was done by Dr. John Calhoon at Christus Santa Rosa Hospital in San Antonio (which is where the children's heart surgeries are now performed). Why didn't I take my son to Boston, Philly or Michigan?

"Continuity of care" was the expression we used on the insurance forms when we asked for my son's surgery to be performed by the same surgeon. He also had the same anesthesiologist he had had 16 years before. How many surgeons have some of the same members of their surgical team after 16 years of working together? What does that say about the care team? Who knew my son's heart better than the team that had worked on him 16 years prior?

Unfortunately, whenever there are controversies in the news, like the one in Kentucky, it is common for heart parents to second guess their decisions. Are they foolish for simply taking their child to the closest facility for care or to a facility that they believed would provide the best care even if they weren't nationally ranked? Should they be fighting for their child to go to the "best" facility? What facility is the best facility anyway?

Unlike parents whose children need tubes in their ears or some other kind of simple procedure, our children face life-and-death situations. There are so many issues we parents have to consider when we are trying to decide where to take our children for care. Sadly, one of the issues is: if our child doesn't make it through this procedure, are we close enough to family for them to be able to get to the hospital to say 'good-bye'? It seems melodramatic, but it's not. Unfortunately, this is an issue far too many friends of mine have had to face. It's not just about where our children receive care; finding the "right" hospital means finding a place where the parents can receive support as well. What happens to the family if the unthinkable occurs? How will being far away from family and friends complicate an already seemingly impossible situation?

My hope is that the problems associated with the University of Kentucky Hospital are all administration-based problems and not actually problems with the surgeons themselves. My hope is that the care any of my friends' children may have received at any facility was appropriate and that no child or family suffered needlessly.

Heart families need to have confidence in the hospitals they take their children to for care. Hospitals need to make sure that they are truly serving their communities and that they follow the Hippocratic Oath:  Do No Harm.

Monday, August 5, 2013

Transferring Responsibility

Watching our children grow up can be challenging. It's scary to see them make mistakes but a good parent knows mistakes are part of growing up. Being the parent of a child with a congenital heart defect means watching our children make mistakes is even more challenging since some mistakes could be so costly. Not wanting to be a "helicopter parent" means letting go and transferring responsibility of care -- even if that means mistakes might be made.

I believed in letting natural consequences serve as "punishment" when my children made mistakes growing up as often as I could, but most of the mistakes my children made were minor. They weren't life-threatening. What would happen if I totally gave responsibility to Alex for his medical care? Would he be as vigilant as I was? Would all of the years of learning about his heart defect, his medications, possible side-effects . . . would it all be for nothing?

Training our heart children early is essential. Alex knew the name of his heart defect and the name of his surgery before he even really knew what they meant. He knew his medications. From an early age, I had him help me with his medicines. It was never up for discussion. He needed his medicines and I was lucky that he was very compliant in taking them, even when he didn't like them. I just acted quite matter-of-fact about them. Since both his father and I took different medications as well, I don't think Alex really gave them much thought. Plus, since Alex was 2 months old with his first surgery and 9 months old when he had his second surgery, he's taken medications every day of his life (that he remembers). Since I've written books for the heart world and given many speeches at a variety of heart events, Alex knew his story. But would Alex carry that with him and be the responsible adult I needed him to be?

When Alex came home from his first year of college I noticed that he had more medication left than he should have. He informed me that he hadn't remembered to take his medicine everyday but that he was good "about 80% of the time." Eighty percent of the time! I told him that he would have to tell his cardiologist that when he had his visit.

Since Alex is now 18, he could go to his cardiology appointments alone. It didn't surprise me when he decided he would do his appointments without me. He went to his first appointment -- a stress test -- and did very well. This was the first time for him to take a stress test and I was a little concerned about how he would do but, according to Alex, his cardiologist was pleased with how well he did.

Next came his echocardiogram. Alex has had so many "echoes" that I didn't worry about him going for this test. It's not uncommon for him to be so relaxed during this test that he dozes off. I had hoped he would have our favorite echo tech for the test, but he had someone new. He said it was uneventful.

Finally Alex went for his meeting with his cardiologist. Again, he wanted to go alone. Transferring responsibility is tough. I wanted to be at that meeting! I wanted to hear the doctor tell Alex he'd done a good job. I wanted to see the test results myself. I was used to recording and keeping track of everything but I let him go alone and I even left town! I spent the day with my sister out of town celebrating my birthday.

While I was out with my sister I received a call from the hospital. My heart raced when I recognized the number. Was Alex okay?

The person calling said she was scheduling Alex for his follow-up appointment in six months. She then went on to tell me that her son requested she call me because he didn't have his calendar with him. She immediately started praising Alex. She told me how Alex's doctor had raved about what a great patient he was and how he wished more of his patients were like Alex. I felt so proud of my son. According to this nurse, Alex had had a stellar appointment and Alex's doctor was very pleased with his progress.


When I got home I asked Alex how his appointment went. He gave me the same details the nurse had given me and then he gave me the print outs his doctor had shared with him to put in his files. I told him what the nurse had said and he smiled. Then I asked him if he had told his doctor that he hadn't remembered his medications everyday. He said, "yes" and then told me that his doctor told him that if he forgot his medications in the evening, he should take them first thing in the morning.

Transferring responsibility can be difficult but it is so gratifying when it all works out well. I won't always be around Alex to make sure he does what he's supposed to do. I need to know that he can take care of himself. He showed me this summer that he could. I'm just thankful for the fact that Alex has so many people watching over him and for the fact that he wants to be healthy. The best thing that came out of this was seeing that all of the years of training had paid off. Alex could handle his exams and his appointment because he'd already done it over a hundred times before. He is growing in confidence and capability and I'm learning to let go and even to relax a little bit.