First, please accept my apologies for the mass email. I thought you’d all want to know, sooner rather than later, what’s going on here with me, Philippe and Sprout, but figured I’d lose my voice if I tried to call you all.
As most of you know, I’m about 22 weeks pregnant with our first child. We went last Friday for our 20 week ultrasound, the one Philippe refers to as “the state of the union,” where they measure all the bones, check to make sure the heart, brain and other organs are present and intact, and generally do as full an assessment as possible on a little 11 oz. thing the size of a large Vidalia onion.
We were among the very small population who learned that not everything is alright. After a 2.5 hour ultrasound session, performed by 2 sonographers and then a very specialized OB/GYN, we learned that Sprout has a serious congenital heart defect. The doctor immediately went into red alert mode and by Monday morning we had had emergency amniocentesis and a consult with a pediatric cardiologist. Yesterday we had a second pediatric cardiologist look at our little dude/dudette.
Our current diagnosis is something called Truncus Arteriosus and occurs in about 1 in 10,000 live births each year. What does this mean? From the website of the American Heart Association: Truncus arteriosus is a complex malformation where only one artery arises from the heart and forms the aorta and pulmonary artery. (There should be 2 distinct arteries.) Surgery for this condition usually is required early in life. It includes closing a large ventricular septal defect within the heart, detaching the pulmonary arteries from the large common artery, and connecting the pulmonary arteries to the right ventricle with a tube graft. Children with truncus arteriosus need lifelong follow-up. The positive news seems to be that we have 4 visible and correctly proportioned heart chambers and the valve that is extent is functioning well. The large vein will be used as a native aorta and a new vein and valve will be created as the pulmonary artery. 90% of children survive this surgery and go home from the hospital about 2-4 weeks post-surgery. For those of you who like lots of information, there is a bit more and a picture (as well as a comparison to a normal heart) at http://www.americanheart.org/presenter.jhtml?identifier=1677. The Cleveland Heart Institute website is good, too.
The OB/GYN who found this must be Sprout’s guardian angel—we’ve been told that many level II ultrasounds will see 4 heart chambers and a strong heartbeat and declare the state of the union to be strong. Dr. Rudy Sabbagha and his sonographers also look for blood flow (like weather radar) and look closely at the larger arteries. About 50% of TA babies are diagnosed after birth, lowering their chances for survival.
What happens next? We continue the pregnancy as healthfully as possible. Based on current knowledge, there is no reason to believe that I would deliver pre-term or that I would need to have a C-Section. In addition to my monthly OB/GYN appointments, I’ll go every 5-6 weeks for a fetal echocardiogram so the cardiologist can keep her eye on the defect. The defect cannot improve in utero, but it can worsen.
Immediately after birth, as soon as Sprout is stable, the baby will be transferred to Chicago Children’s Memorial Hospital. Within the first three weeks of its little life, baby will have open heart surgery to do the repairs described above. Follow up surgeries will depend on how quickly the baby’s heart outgrows the graft/conduit and valve that are inserted for the fix.
We’re meeting with the cardiovascular surgeon next week and are hopeful that his outlook will be as positive as the 2 cardiologists we’ve seen.
While TA children are heart patients for the rest of their lives, once they are past surgery they can expect to live relatively normal lives, participating in recreational sports and gym with some restrictions.
For those of you who want to know “how did this happen?” no one really knows. Fewer than 1 in 1,000 babies are born with congenital heart defects. Of those, about 0.4%–2.8% are born with truncus arteriosus. Our cardiologist has said that the risk of recurrence is low, so when the time is right, we’ll take that up with our Reproductive Endocrinologist, Embryologist and a geneticist to determine if we want to risk using our remaining 12 embryos on ice (“totsicles”).
I’m feeling fine and Sprouty is kicking up a storm as I write this. We’ve had to process a lot of information and make a lot of decisions in the past 6 days and our bodies are finally feeling it, so we’re likely to be sleeping a lot this weekend. Now we’re in project management mode—establishing Sprout’s medical team, confirming insurance, etc. My goal is to keep our lives as normal as possible from now until early June. The summer will be tough, but with luck we’ll have a normal, wonderful summer in 2006 with our little Sprout playing in our backyard.
As you can imagine, the past week has been quite a rollercoaster for us. We’re extremely fortunate to have a wonderful children’s hospital in our city. We’re also surrounded by the most extraordinary group of friends and family as well as an incredible Rabbi. And, I have to say that the folks at Philippe’s office (both in NY and Chicago) as well as his clients have surprised us with their compassion and concern. We’re scared, but doing as well as possible.
I’ve started an email distro list for “Team Sprout” with which I’ll keep everyone updated throughout the pregnancy and afterwards. I’m happy to include you. I only ask that in return you keep us and Sprout in your prayers.
By the way, 2/14 is not only Valentine’s Day. It is Congenital Heart Defect Awareness Day. We’re more aware than ever.