Sunday, September 10, 2006

A Journey of the Heart

Ethan Markus Lindberg

Our story begins just as many others do. With the joy of discovering we were having a baby. Newly married, we were eager to start a family and in October 2004 we found out we would be welcoming a new baby the following summer. At the suggestion of my wonderful OBGYN at Northwestern in Chicago, I agreed to all the prenatal testing just be sure everything was okay. My first trimester testing came back just fine. At the beginning of my second trimester I underwent another common screening, the Alphafetoprotien (AFP) Test. I received a call from Dr. Goldman, my OBGYN, soon after saying that my AFP results showed I had a 5% chance my baby would have Trisomy 18, a fatal chromosomal abnormality. She stressed that there was a 95% chance my baby would be healthy and suggested an immediate amniocentesis just to be sure. During this very upsetting conversation I was driving in the car on the way to pick up my husband and head to the airport to visit family for Christmas. For some reason, I did not feel inclined to have an immediate amniocentesis since I knew that, regardless of the outcome, I would give birth to the baby. And I just needed some time to think. After speaking with Dr. Goldman later that day, we agreed I would have a level 2 ultrasound at the hospital during my 20th week.

January 19, 2005 my husband and I sat anxiously in the ultrasound room. The perinatologist came in and began the ultrasound. To our relief he did not find any markers for Trisomy 18 and felt confident things were fine. He also told us we were having a baby boy. Our little boy was positioned in such a way that it was hard for the doctor to see his heart. After some repositioning, the doctor said that while all looked okay, he noticed a “spot” on the heart. Sure it was probably nothing, he suggested we go have lunch and he would run our baby’s pictures by a pediatric cardiologist that just happened to be in the office that day. We returned from lunch to a very worried cardiologist, Dr. Gotteiner, who wanted to do an immediate fetal echo. She diagnosed him with aortic stenosis and evolving Hypo Plastic Left Heart Syndrome, a severe congenital heart defect. Hypo Plastic Left Heart Syndrome (HLHS) occurs when the left side of the heart does not grow properly and is completely abnormal. The left side of the heart is the strongest and most important half of the heart. While there is no cure, there is a series of three palliative surgeries that allow children to live with his defect.

This was a devastating diagnosis. We listened through tears as Dr. Gotteiner explained her worry that our little boy would not make it through the pregnancy. We discussed the palliative surgeries for HLHS and asked a few questions about living with this defect. The long term outlook for HLHS is unknown. The oldest survivor is in their twenties, since it’s in the last two decades the palliative surgeries have been perfected. Children that do live into their teens are often sick and limited by their heart defect. Then she told us about a doctor at Children’s Hospital Boston, Wayne Tworetzky, who was doing experimental fetal interventions, dilating the aortic valve inutero with the hopes of increasing blood flow to the left side thus promoting growth. He’d had some success. Some children were actually born with a healthy functioning left ventricle. And others, more commonly, needed immediate surgery, but the left side of their heart was contributing to their circulation. Most children with HLHS do not have a left ventricle, forcing all the blood to be pumped entirely by the right side of the heart, something it was never designed to do.

Within 48 hours my husband Erik and I were on the phone with Dr. Tworetzky learning about this fetal intervention. In order to qualify for this intervention I needed to have an amniocentesis, which showed our little boy’s chromosomes were completely normal. We also had to travel to Boston to so Dr. Tworetzy could do a fetal echo and be sure our baby was a candidate for this procedure. We also learned the details of the fetal intervention;. a team of physicians conduct a procedure where they insert a catheter into the mother’s uterus and then into the heart of the baby. Once inside the heart they blow up a balloon and dilate the aortic valve. On February 1, 2005 we traveled to Boston and I underwent this intervention. We were the 32nd family to agree to this inutero procedure. Our fetal intervention was successful and we returned to Chicago and waited for Ethan to be born.

We elected to have Ethan born in Boston so we could have access to the world renowned surgeons and cardiologists at Children’s Hospital Boston. Ethan Markus Lindberg was born on May 20, 2005. He was pink and he cried right away. We briefly held him and then he was transferred to the Cardiac ICU. At birth Ethan’s left ventricle was not ready to be it’s own pumping chamber. Dr. Pedro Del Nido performed a modification of the Stage 1 surgery for HLHS, the Norwood, on May 26th 2005. Although Ethan has needed 2 subsequent surgeries during his first year of life, the left side of his heart is now significantly formed and contributing to his circulation. Dr. Del Nido is perfecting a surgical technique that aids the left ventricle in growth and function. Over time some children’s left ventricles have been big enough to support 50% of the body’s cardiac output. We are hopeful that Ethan might be one of those children. The road has been long, sometimes scary and has taken us far from home (in Chicago) for long periods of time. But we are committed to doing all we can for our son. We continue to forge ahead in hope that soon the left side of his heart will be strong enough and big enough to allow him the blessing of a two ventricle heart. Regardless of where his heart ends up, Ethan is better off because we have taken this road less traveled, beginning with the fetal intervention.

Had I agreed to the immediate amniocentesis on that cold December day, I would have gone through the remainder of my pregnancy assuming I would have a healthy baby boy. Something told me to wait and have the ultrasound; a decision that lead us to Boston and ultimately to a better heart for Ethan. Never doubt a mother’s intuition!

For more information contact:

Jessica Lindberg

Check out Ethan's website

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