The very wise Kami asked for a quick tutorial on the heart problems we are facing, so here is a VERY layman’s description on babygirl’s condition, including some of the medical terms in bold, for those of you who like to google things. It is complicated, but try to hang with me.
Her cardiovascular system is severely malformed. This apparently happened just a few days after implantation. No one knows why, but it is thought maybe genetic factors combined with environmental factors. It is not related to her conception (IVF) or her status as a twin. And the other twin is completely unaffected. First, the heart organ itself is malformed. The right ventricle, which pumps blood to the lungs to get oxygen, is extremely small and basically nonfunctional (hypoplastic right heart). Before birth they thought she might not have one at all but it turns out she does. But it doesn’t work, for various reasons. So, she has no way to get her blood oxygenated, so her body can’t get the oxygen it needs. So, she is a single ventricle patient. This is life threatening. Actually, it cannot support life; without corrective surgery she would surely die.
Also, the heart has two upper chambers, the right and left atriums (atria). Each is uniquely formed and has different functions, not just one happening to be on the left and one on the right. But hers formed as two “lefts” — so the one on the right side is a second “left” atrium, if that makes sense. I can’t explain exactly why this is bad, but it is. This is called left atrial isomerism.
In addition, the blood vessels in her cardiovascular (CV) system are seriously weird. Major vessels are in the wrong place, go the wrong way, connect to the wrong place, etc. etc. This is, among other things, interrupted inferior vena cava. She also has pulmonary stenosis, which I can’t explain but wikipedia can: a valvular heart disease in which outflow of blood from the right ventricle is obstructed at the level of the pulmonic valve.
But wait, there’s more. Her heart points right, not left, and is on the right side of her body. This in itself is not dangerous. Many people have dextrocardia and live just fine.
She has heterotaxy: a rare congenital defect in which the major visceral organs are distributed abnormally within the chest and abdomen (copied from wikipedia). Her bowels are folded in an incorrect way (malrotation of the bowels), her stomach is more on the left than the right where it belongs. The appendix, which I think is supposed to be on the lower right side, was on the opposite — upper left? I forget. She had an operation to repack the bowels in a safer formation (the other way can cause dangerous or even fatal problems (volvulus), that is how Bee Gee member Maurice Gibb died at age 50-something) and at the same time they removed her appendix. It was healthy but appendicitis later in life would be problematic for her.
Associated with the isomerism is polysplenia — having many little bitty spleens instead of just one, which may or may not be functional. Hers appear to be functional, for now at least. If it (they) fail, she will need to go on prophylactic antibiotics for life.
There are other issues but these ar the main ones and the ones I can explain best, if only in “mom terms.” Oh, and did I mention she was on a ventilator for so long that she is basically unable to bottle feed? So she has a feeding tube (a g-tube, specifically a Mic-key button. We work with her on bottle feeding but it is slow going. She does have a good sucking reflex and loves her pacifier.
So, the corrective heasrt surgery she needs to survive is called the “fontan” series. First, as a newborn (in her case, at 3 1/2 days old) a BT Shunt is inserted to direct some blood to the lungs to pick up oxygen. This is merely a stop-gap measure to keep her alive until she is big enough to have the real repairs done. The shunt is a synthetic tube thingy. It doesn’t grow with her and is a tenuous but effective help for a small baby. Second is the “Glenn,” which is the one we are awaiting now. In the Glenn, the surgeon basically cuts and reconnects some arteries to send blood directly to the lungs without needing the heart to pump it there. It is mainly the blood coming in from the upper half of the body, but with our girl’s unusual blood vessel formation, it will actually reroute about 75% of her blood (this is a good thing). At this time the surgeon will also go in and make some other repairs inside her heart and to some other arteries, but the Glenn is the main thing. When she is two years old, the Fontan procedure will do the same thing for the blood coming in from the lower part of her body. (After the blood is oxygenated in her lungs, it comes back to her heart and the ventricle that she does have will pump it out to the body.)
So there you have it. This is NOT expert medical opinion or explanation, just a brief outline of our problems as I understand them. I thought learning the reproductive system so I could understand my IF treatment was difficult. This is a million times more complex. And the docs tell me that on a scale of 0-100, with 0 being a normal heart system and 100 being the most complexly deformed anatomy, we have about a 95. There’s no hope of me becoming as knowledgeable in this as I was for my own IF problems, so I just try to learn a little more each time they tell me things, to ask a question or two each day, and to know I have excellent docs who will give my girl the care she needs. They tell me when the fontan is done, she’ll lead a pretty normal life. She won’t be an athletic type, but we’ve been told not to raise her as “a cripple” (their word not mine) but as a regular kid who just may not be able to particpate in gym class sometimes.
One web site I like is http://tricuspid.wordpress.com/ — blog of an adult CHD patient. Great info, inspiring story. Otherwise, I do not spend much time on web sites bc it is too confusing. And frankly, her particular condition is very rare. I have been told her “cardiac anatomy is unique.” So there isn’t a lot out there that may pertain to her well.
I have learned that CHD are the most common birth defect, affecting 8 or 9 out of every 1000 births — or 1 in 112 to 1 in 125. Many are easily treatable or don’t need treatment. Some, obviously, not so much.
Her Glenn has been rescheduled for Tuesday, Dec. 29. There was hope we could await that here at home, but she is not stable enough to do so, so she’ll be in the CV ICU (cardio vascular intensive care unit) until then and during the recovery period (1-2, maybe 3, weeks). I am so sad about this, not just that we can’t have her back, or have her back over Christmas, but that she is unwell enough to need to be there at all.
I am grateful to have babies to spend Christmas with, unlike the horrible Christmases past, which featured BFNs, chemicals, etc. But oh, this is hard.