Charlotte's Journey Home

Just a Regular Kid, Sort Of


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Dr. Mavroudis, Surgical Consult

We met yesterday with Dr. Constantine Mavroudis, chief of pediatric cardiovascular and thoracic surgery at Children’s Memorial Hospital. We really enjoyed meeting him, though of course we wish we had no need to know him. Anyway, he confirmed the procedure that our cardiologist explained to us. The good news is that so far, Sprout does not seem to have any of the factors that further confound the repair of a Truncus Arteriosus. We’re lucky to have one valve that is insignificantly leaky and a short pulmonary trunk.

Here’s the timeline he explained to us: Once Sprout is born, the baby will be transferred from Prentice Women’s Hospital at Northwestern Memorial (where I’ll deliver), up the road to Children’s. The cardiovascular surgery team will organize and arrange for the transport, including sending a team to come get the baby. Sometime within 7-10 days of birth the surgery will occur. Dr. Mavroudis will operate with his associate Dr. Backer. Sprout will be on the heart/lung machine while they do the following: remove the pulmonary artery from the trunk, use a pulmonary homograft (from a human) to create a proper pulmonary trunk, close the ventricular septal defect (VSD) and put it all back together. Once in the pediatric ICU (PICU), Sprout will be kept asleep for 2-3 days while the pulmonary pressure decreases. Then baby will be weaned off the ventilator. Once off the ventilator, baby will leave PICU for a regular room. Total hospital stay should be 3-5 weeks.

We learned, with a sigh of some relief, that the mortality rate (based on 30 day survival of surgery) depends on the risk factor. Our risk factors put us at a 2-5% mortality risk. It’s lower than we thought.

During the surgery registered nurse practitioners will keep us informed of the progress and the doctors will talk to us afterwards. The nurse practitioners will also work with me to pump and freeze my breast milk so that Sprout can have it when s/he is ready. If it’s possible, we’ll try to feed the baby prior to the surgery, but it’s likely that the baby will be on an NG tube for feeding until after the surgery.

The conduit will probably need to be replaced for the first time between 8 months and 3 years. Follow up surgeries are dependent on the conduit (pulmonary trunk replacement) failure/lifetime.

The one new piece of information that we’re trying not to freak out about—Dr. Mavroudis told us that 10-12% of Truncus babies need heart transplants in their 20s or 30s. Of course, this is based on kids who had the repair done +20 years ago, so we’re hopeful that this procedure, and the future ones, will continue to improve. He mentioned some research going on in which they’re trying to use a child’s own cells to grow the replacement artery. If this is successful, it could mean that eventually the replacement conduit would grow with the child and no longer need to be replaced.

BTW, Dr. Mavroudis went to Jersey City HS, Rutgers and University of VA medical school. He did his pediatric and surgical training at University of California (Berkeley, I think). His credentials are excellent and he’s from NJ! He also told us that the 3 best places for this surgery are Ann Arbor, Chicago and San Francisco. So, we’re as confident as we can be that we’re in the best possible place for what we need.

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Follow up with Dr. Young, Fetal Cardiologist

We had a consult today with one of the cardiologists most likely to follow my pregnancy and then care for the baby post-operatively. She confirmed the diagnosis although she sees a slightly different variation of the anomaly than Dr. Cuneo saw. This is pretty technical stuff and has to do with where the veins and trunks are coming from. Bottom line, it affects the approach to the repair, but not the prognosis for recovery. As the fetus grows, the ultrasounds will be able to more accurately inform the doctors as to the exact location of all the parts. As Dr. Young pointed out, which fascinated me, the ultrasound is a 500% or more magnification of a heart about the size of your pinkie fingernail. Technology is amazing.

If we haven’t told you—this heart anomaly does not mean that my pregnancy is high-risk. Nor does it mean that I will necessarily have a C-section. So far, all of that looks normal and I’ll stick with my current OB unless she has a comfort-level issue.

The genetics counselor at Children’s Memorial has said that she’s not heard of the false negative with the Digeorge’s test. Phew. I have sent her the article Philippe read and am hoping she can explain it to our satisfaction and comfort level. Meanwhile, we’re still waiting for the rest of the chromosome reporting from the amnio (another 2 weeks or so), but the chromosomal anomalies most commonly associated with Truncus have been ruled out.

Meanwhile, we have a consult set with the surgeon for next Thursday, February 10. After that, our next fetal ultrasound isn’t until 3/16

And now a word from Philippe (which I’m copying from an email he sent earlier today): We had yet another great encounter with a doctor this morning – this time the doctor, Dr. Young, who will likely be our treating cardiologist moving forward. As we get more info, Ilene and I are getting to be in a better place. We’re still collecting more data: we’re planning on talking to a geneticist as soon as possible, while we have a consult with our likely pediatric cardiac surgeon lined up for next Thursday. But, in the meantime, we’re 99% certain we want Sprout to be a part of our lives. Nobody ever said it was going to be easy – we simply know it is not going to be… knowledge as a curse and as a blessing.


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20 Week Ultrasound and Diagnosis: Truncus Arteriosus

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.