As Ruby gets older, I am thinking more and more of her upcoming open heart surgery. I can honestly say that we have felt an immense amount of peace and comfort and have been able to have a pretty normal life with her, meaning we aren’t always scared about what could happen. We are able to enjoy her and not worry all the time. And I firmly believe that we owe that to the many prayers said in behalf of her and our family. If one of your prayers has been said for us, I thank you from the bottom of my heart!
I thought I would write up this post to answer a few frequently asked questions about her heart defect. If you want to read the first post where I talked about it, you can read that HERE.
What is Tetralogy of Fallot?
Fallot is the man’s last name who found out about the heart condition in the 1800’s. Tetralogy is a set of four related abnormalities occurring together (or more, in Ruby’s case, they told us there are six things that are abnormal). You will see Tetralogy of Fallot abbreviated as TOF.
Basically it is a hole in the heart that is causing other areas of the heart to become abnormal. The hole is called a VSD, or a ventricular septal defect, or a hole in the septum that divides the right and left chambers. The hole is why a heart murmur is heard when listening to the heart. Ruby’s heart murmur is very loud and is getting louder the older she gets, because there is more fluid in her body.
Here is a picture of a heart with TOF:
There is also narrowing of the pulmonary valve, thickening of the right ventricle (or hypertrophy), and an overriding aorta which is what allows un-oxygenated blood to mix with oxygenated blood and flow into the body .
Somewhere I have written down the other things that are wrong with Ruby’s heart, but for now, I want to say that we are extremely lucky that she is what they call a Pink Tet. That means that her heart is able to pump enough oxygenated blood through her body. Those who are unable to do this are called Blue Tet babies, and they have a much rougher time, often requiring surgery for a temporary fix before the full repair is done. Pink Tet babies can still end up being a Blue Tet baby however.
What makes this hole in the heart different than the many others babies born with holes in their hearts, who require no surgery to fix them?
Many times we tell people about the hole in her heart and we hear over and over how they have one, or someone they know has one, and sometimes that the hole resolved itself and they were fine or sometimes they still have a hole but no other effects from it. I myself have a small heart murmur. But it is because of the SIZE of the hole and WHERE it is in the heart and the EFFECTS on other portions of the heart that mean it must be operated on to fix. It is rare that you will learn of someone living into old age with unrepaired TOF. And the older they get with unrepaired TOF, the greater the risk of heart failure.
What does that mean for Ruby now?
Ruby’s heart beat is very fast and her breathing is very fast. She breaths about 10 breaths per minute faster than normal babies. Which means over the course of 24 hours she breaths over 14,000 times more than normal. She burns more calories because of this and tires a bit more easily, especially while eating.
She has a hard time gaining weight and every ounce is a celebration :). We go in to her normal pediatrician weekly for weight checks and to check her oxygen levels. Her oxygen is always in the 90’s (%) which is great! If her oxygen levels start to drop, then they will schedule the surgery. If she isn’t gaining weight then we have to look into a higher calorie diet, or moving up the surgery. She nurses and also gets about 3-4 oz. of formula a day. There were a few weeks in the beginning where she didn’t gain at all, and a week a couple weeks ago that she lost weight, but her average weight gain is 5-6 ounces/week which we are thrilled with.
We get the question “was she a preemie?” all the time. As in multiple times a week. She is almost 3 months old and barely 9 lbs., so people also assume she is around a month old or younger. I had one lady ask me if she was just a couple days old when she was 2 months old. So in a way it is super fun to have her stay tiny for so long, if only there wasn’t a medical reason behind it! I’ve actually stopped explaining to everyone what is going on because one trip to Walmart can mean explaining it 5 times. I don’t mind explaining it but I also want to feel normal sometimes. I know people mean well and it’s not offensive to us :).
We see the cardiologist about once a month right now. They have done an EKG both times we were there, two echocardiograms, and a chest x-ray. They will have to do a sedated echocardiogram before her surgery. They also do genetic testing on them to determine if they have other things that may go along with TOF, like DiGeorge Syndrome. They do not think she is showing any signs of the things they test for, so that is why they are waiting to do the testing at the time of surgery (less pokes while she is awake!).
Her heart murmur is becoming louder the older she becomes. If you put your ear up to her chest you can hear it very, very well. You can also feel the constant motion if you put your hand on her chest, rather than a thump thump of a normal heartbeat. She has started to become more sweaty while she eats, which is a sign of heart failure with TOF. In her case, since she is still gaining weight and her oxygen levels are great, they talked about putting her on Lasix medicine to control fluid (still need to find more info on this but I was just told that this week).
Since she is at risk for having a spell (turning blue and not breathing well), she isn’t supposed to cry hard or cry for a long time. This can be quite stressful because babies, well, cry! Driving can be especially nerve-wracking when she starts to cry harder. I’ll just pull over if she’s getting too upset.
My kids have been amazing helping me out. If I’m showering or doing something else and can’t hold her, and she starts to cry, they are always there within seconds to try and cheer her up. They have been so great. They call to each other, “Make sure her lips aren’t turning blue!” If she does have a Tet Spell, then we’re supposed to try and calm her down and push her knees to her chest, forcing the blockage open with the stronger flow of blood. If that doesn’t work we are supposed to call 911. So as you can probably guess, she is getting lots of attention and doesn’t cry for long!
But honestly, other than the weight gain, rapid heartbeat and breathing, and now sweating during feeds, you would never know anything was wrong with her. We realize what a blessing that is.
Did they know about it before she was born?
Often they will see TOF on the 20 week ultrasound. I did have two ultrasounds while pregnant, but they never saw it. However, I am really glad we didn’t know before hand. I think I would have been completely worried and stressed the remainder of my pregnancy and I’m happy that wasn’t the case. I also am glad she did so well after birth. A lot of stories I read say that if they know the baby has it before birth, they just plan for a NICU stay even if the baby is doing well, like Ruby. Not sure if that would have happened or not, as every doctor and hospital handle things differently, but I am glad we didn’t have any of the worry and stress during pregnancy and immediately after birth.
When is her surgery scheduled for and how many will she have to have?
Her surgery will be between 4-6 months old. She will be 3 months old September 5th. So we are getting there. As long as she is gaining and her oxygen levels are good, then it will be around 6 months. So we’re hoping for that.
They told us the average number of surgeries she’ll need is 3. BUT, the last echocardiogram showed better results and there is a possibility that she will only need one! So we are praying and hoping for that! If they have to work on the pulmonary valve, then when she’s older they will have to put in an artificial valve (early childhood) and then another one in her teenage years or early adulthood, since she’ll outgrow that valve. But if the work they do on the pulmonary valve is minimal, then only one surgery is possible! Which initially we didn’t think was a possibility so I was thrilled when the echo showed more optimistic results last time.