If you are reading our story because your baby has been diagnosed with complex heart defects I have good news for you. The good news is that there now are more resources available than ever before. You can find online specialty message boards, journal articles, lots of websites with chd survivors with many varieties of defects. You can even email many of the top ranked surgeons and pediatric heart centers! The not-so-good news is that while access to all this information will help you make important treatment decisions about your baby, it's still not an easy road, by any means. Despite many advances in medicine, many complex defects simply cannot be "fixed". But that is where hope comes in. I hope our story will provide to you the same kind of hope that reading about other children gave to us in our hour of need.

We received our "bad news" about baby in June of 2001. I was 22 weeks along and expected only to find out the sex of the baby, and confirm what I knew to be true, that baby was perfect! Wrong. The OB left us in the exam room for a very long time. I don't know if she had ever delivered bad news before. I wonder what must have raced through her mind when she looked over the ultrasound. Eventually she did return to the little room where I sat with legs dangled over the exam table and my husband bobbed our squirmy 10 month old son on his knees. There was no good way to phrase it, I’m sure, but telling us that our baby had an "incompatible with life" condition still seems boldly absurd to me. We were told that our baby had hypoplastic left heart syndrome and we were given our options. My body, mind, and spirit were already rejecting this news. With arms firmly crossed I asked, probably with some misdirected defiance, "What about fetal surgery?"

The scene essentially repeated itself again not many days after when a maternal-fetal specialist confirmed the diagnosis. We were given our options once again; termination, comfort care, surgeries, or transplant. By then we had done some research and had a better understanding of the situation. We knew by then that "incompatible with life" was not entirely accurate. We also knew that if our baby would survive, it would require that we fight for her. We didn’t know yet exactly what we would do, although termination was out of the question entirely. The mfm offered to set us up with an appointment with a fetal cardiologist to help us sort things out.

Dr. Cuneo of the Heart Institute for Children determined that our baby did not have hlhs, but instead hrhs, or hypoplastic right heart syndrome. An extremely rare defect known as L-TGA (L-Transposition of the Great Arteries), or ventricular inversion, had fooled the mfm – he may never have even encountered the defect before. In addition to that, our baby also appeared to have Ebstein’s anomaly, a coarctation of the aorta, a few holes, and of course, the hypoplastic right ventricle. The new diagnosis did not mean that our baby was out of the woods. No. It simply meant that the right sided issues vs. the left sided issues resulted in a potentially more favorable prognosis. She would still require the same surgeries we had read about when we thought we were dealing with hlhs. We consulted with Dr Cuneo, and Dr Ilbawi, a top-rate pediatric cardio-thoracic surgeon, and researched as much as we could online. Our insurance company assigned us an excellent case manager to help us research surgeons and hospitals. We were moving inch by inch away from despair and toward the light.

Our hearts told us that we needed to fight for our baby. We did not want to risk wondering what might have happened. We did not want to know that someone was missing from our family because we were afraid of surgery. I certainly did not want our baby to go through three successive surgeries and whatever suffering that might involve. But given the fact that it was her very life that was at stake, and that there was as good a chance as any that she would survive and do well, we regarded the surgeries as very much worth it. In fact, instead of fearing the future, I began to feel a sense of relief and gratitude. I was thankful that we had an option, that we really might be bringing a baby home after all, and that she might live a long and happy life on top of it!

And so, in several months time, Celine entered the world. She was only hours old when she began to turn blue and required her first intervention, initially only some oxygen, but they gradually began adding support in anticipation of her surgery, such as an arterial line, an "ng" tube, and a ventilator. Within two days things began to deteriorate and she required a balloon catheter procedure to keep her alive. And then, in several more days, it was time for her first surgery, the Norwood. She did well, although it was extremely hard on us, her parents. I was a basket case and ill-prepared for the experience of seeing my baby after open-heart surgery. During her three week recovery period, I often had to be at home with our son. The separation from my new baby was almost unbearable. We muddled through.

When Celine came home, we weren’t quite sure what to do with her! She was technically still medically fragile, and would be up until her second surgery. She had some difficulties with feeding and we were creative about figuring out how to make sure she had more than the minimum required calories. Day by day we understood what she needed from us and life started to unwind from the tight little ball we were all wrapped up in at the time of her birth and first surgery. Her coarctation began to narrow, dangerously, two months after she came home, which required a catheterization to open it up . . . one that failed (I was heartbroken) and would require a surgical resolution. Fortunately, her surgeon was willing to wait to correct it during her second scheduled surgery.

The time for her next surgery came quickly, and again she did well, and we, the parents, did slightly better than we did the first time around. We sought out Early Intervention because Celine seemed to have some developmental delays. EI determined her primary delay was with speech. If you are unfamiliar with EI, it is a state program that provides one on one therapy in your own home. Many, many infants and young children are eligible for these services. Celine benefited from speech therapy for several months and suddenly it was time for her fontan (and hopefully final!) surgery. She was 18 months old, so her recovery was a little bit harder to bear. She did not enjoy a week restricted to her hospital bed, the near dehydration (this is a therapeutic issue required for a good recovery), the IV’s, the suture pain, the chest tube removal, the poking and prodding, yes . . . the boredom. No, of course she didn’t like it, but it was a temporary period of discomfort that ultimately saved her life!

Now she is a very normal 4 yr old. She is in preschool and has no known delays to report. Her heart function has been excellent. She had an oxygen saturation level of 100% several checkups ago. She’ll be monitored for the possibility of a pacemaker beginning sometime in the near future. And just to throw in a few details of her personality here, she wants to be a doctor, a nurse, or a rock star when she grows up. Her favorite movies at the time of writing include "The Incredibles" and "Madagascar". She has a slap-stick sense of humor and often has her parents and her three siblings laughing with/at her. We have been extremely blessed, this we know. But we had to take a chance to receive our blessing. We know not to take it for granted that tomorrow will be trouble free. I suspect that some trouble will come, sometime down the road. Whatever troubles may come (if they do), we are a family and we will weather them as a family, just as we celebrate our trouble-free times as a family. We think we will do just fine, but as we will pray for you (anyone who may be facing some very difficult decisions and circumstances regarding their baby), please pray for us!

Monica recommends: Life and Death Decisions and Hypoplastic Right Hearts. Additionally, these spiritual resources helped her get through the pregnancy with Celine, as well as the subsequent surgeries: Sisters of Life and The Divine Mercy Devotion.