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." (http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3184425/). 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 (http://circ.ahajournals.org/content/120/4/295.full) 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 (http://www.ncbi.nlm.nih.gov/pubmed/12957444).

What about HLHS specifically? I found one article specifically addressing this issue. It reported an 8% recurrence rate (http://www.ncbi.nlm.nih.gov/pubmed/17936159), 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 (http://www.wilsontimes.com/News/Feature/Story/24391504----HEART-LOOKS-BEAUTIFUL-) 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.