Maybe this will answer some of your questions…

Beyond the millions of questions I have about the baby, others have even more. We don’t have all the answers and are learning as it comes.  I read everything from medical reports to blogs of people I have never met.  At first, we both read so much and had so many questions.  But as time went on, we have decided to take what the doctors tell us and make decisions from there.  Our research about the actual heart (think anatomy class 101) has helped to understand and have a mental picture of what is actually wrong/different than a normal heart.  Until the baby is born, we don’t know everything and only plan for what we can.  But in the meantime, please let me answer some of the questions that I have had asked of me since we broke the news.  So here goes…

What is Tetralogy of Fallot (TOF)?

TOF is a congenital heart defect. A congenital heart defect is a problem with the heart's structure that is present at birth. TOF is a rare, complex heart defect, but it is one of the more common heart defects. The difference between TOF and other heart defects is that TOF is actually 4 heart defects:

1. A hole between the lower chambers of the heart (the left and right ventricles);
2. An obstruction from the heart to the lungs (smaller than normal pulmonary valve);
3. The aorta (blood vessel) lies over over the hole in the lower chambers;
4. The muscle surrounding the lower right chamber becomes overly thickened.

Yes, a baby can have those individual defects, but you must have all 4 for the TOF diagnosis.

Here is a healthy normal heart versus TOF picture.  This might help in comparison. 

From what we can see on the fetal echocardiograms, the VSD (hole between the right and left ventricle) is very large.  It almost looks like the entire wall is missing (as in this TOF picture you can see the wall).  When the fetal echo has been switched to the color view, you should see blue (deoxygenated blood) and red (oxygenated blood) separately.  But in our fetal echos, we basically see orange everywhere.  This is because the hole is so large and the deoxygenated and oxygenated blood are mixing.

What did you do to cause this? (More politely, "How did this happen?")

I don't know. It just happens unfortunately. Truthfully, doctors don't know what causes most cases of TOF. Of all the reasons that can increase the risk of having a baby with TOF (Rubella, poor nutrition, alcoholism, over 40 years of age, and diabetes), I don't have any of those issues. And if you watch TV and see all those malpractice lawyer commercials, then you probably have noticed that TOF is listed as a birth defect for prescription drugs such as Paxil and Zoloft. [No, I have never taken either. I actually have taken very few prescription drugs in my lifetime.] And as far as we both know, neither of us have a family history of TOF. [So it's not hereditary.] I hope you know that I have researched the topic as much as I possibly can.

If you have another baby, will that baby have heart problems too?

As far as we know, the baby does not have a underlying genetic disorder. Neither Phillip nor I have a family history of congenital heart defects. So no - Emerson is perfectly healthy and there is no reason to assume another baby wouldn't be either.

Did you consider abortion?

No. Enough said. Although it wasn't directly discussed at any of our appointments, we could tell that it was almost said earlier on. We are still reminded at every doctor's appointment that the baby is at an increased risk of stillbirth.

How did the doctor's discover the problem?

Many TOF babies are not diagnosed until after birth (from what I have read, it seems that those that know before birth, have also had other complications). I am assuming because the VSD (hole in the heart) may not be large enough on the earlier ultrasounds to be seen. But during my 5 month ultrasound, the ultrasound tech and OB noticed that the umbilical cord only had 2 vessels (should have 3) which is called a Single Umbilical Artery (SUA). But sometimes due to the view, the third vessel (or second artery) is just hidden on the back side. Sometimes, a SUA is an indicator that there could possibly be other problems. Therefore, my OB wanted to confirm the SUA at my next appointment at 6 months. During the ultrasound at 6 months, the doctor had the ultrasound tech do a thorough ultrasound looking for any other possible abnormalities and confirmation of the SUA. At that appointment, the initial heart problems were noticed and my doctor even mentioned possible TOF. At that time, we were referred to the high risk fetal specialist at USA C&W. The rest...Well, you can read my blog to catch up. ;)

What are you most worried about?

Well, I can't say there is only one worry. Of course, as much as we do know, I worry about the unknown right now. But after the baby is born, a lot of our simple questions will be answered (eventually).

After that, I worry about the surgeries. We really won't know how many and when he will need them. The pediatric cardiovascular surgeon thinks that the baby will need surgery soon after birth to place a shunt on his heart that will increase the amount of blood flow to the lungs (as the pulmonary valve is extremely constricted according to the fetal echocardiograms). And then the full repair of the heart somewhere between 6 and 9 months (to fix the actual defects).

And then beyond that, I worry about his development. From everything I have read including medical reports and personal experiences with heart babies, delayed development is to be expected. Heart babies are typically on the lower percentile of the growth charts, have delayed motor skills, and need speech therapy. Emerson has been on the higher end of the growth chart from birth and did everything from crawling to walking to jumping early. So I figure if he has her genes, then maybe he was destined to do them early, but now will just do them on time. That's my hope at least. :)

So as you can see, my worries have developed into stages. I am focusing on more on the immediate, but still concerned about the future.

What is "open heart surgery"?

Let me just say this upfront...Open heart surgery is NOT just another surgery that happens in the chest cavity or even in the entire abdomen. It is NOT the same as having your appendix removed or having a broken rib. There. Said it.

Open heart surgery is any surgery where the chest is "opened" and surgery is performed on the heart. A heart-lung bypass machine is used to pump oxygenated blood to the brain and other organs while the heart is stopped. The heart is not beating while surgery is performed. [The initial surgery to insert the shunt will require a closed-heart surgery where an incision will be made through the side of the chest between 2 ribs.]

Recovery without complications for any child will take 3 to 4 weeks once at home. An infant needs even more care for the first 4 to 6 weeks. An infant needs to be held very carefully during this time as supporting their head and bottom is important. No matter age, we will be unable to lift by the arms (which would be typical at 6 to 9 months old). Activity will be limited which means that even crying for too long may cause severe exhaustion. No pushing or pulling (think rolling over, crawling, sitting up), and even lifting the arms over the head is a big no-no.

 

I hope this helps answer any questions that anyone may have. I am an open book if you want to know anything else. But unfortunately, I don't have all the answers either.  Please feel free to ask my any additional questions.  Seriously, I don’t mind!