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.
Ruby is such a beautiful baby! What a smile! You have been given a challenge with Ruby, but like yin and yang, it sounds like you have also been given so much love, between Ruby, your other children and you as parents. I pray for you and your family and hope the surgery is a one off success!
Ruby is gorgeous!
Being a mom of a child who also had open heart surgery. I can feel a bit with you.
I was so greatful that we live in a time where surgery is possible with limited risks.
I remember I had a dream where I had to put my son in a taxi and there where people with him saying he would be ok.
I could not come with him and that frightend mefor a sec, but suddenly came at peace with it. When I woke up I realised I had to be strong, not to worry so much and trust that doctors know their scills. (ofcourse worrying is a right we have as parents hi,hi)
And everything went more than ok. He recovered so fast!
I am sure your little girl will too! She looks determined all ready as little as she is.
Good luck to you all!!
Love from Holland, Léonie
Thanks for explaining the TOF! I very probably won’t meet you at the Wallmart ever, but I might come across another parent with a baby with this defect. My reaction can be more sensitive I hope, thanks to your story. I’ll pray for Ruby, that she’ll be fine, that she’ll need only one operation, and that you all may enjoy her beautiful smile for many years to come!
Prayers are coming from Michigan for that sweet girl! What an adorable little fighter. Keep fighting, Ruby!!!!
You are doing an amazing job with this little baby! I have friends as well as family with TOF. They had this surgery successfully when they were tee tiny like Ruby. My 18yo nephew had a followup surgery when he was 17 with a fantastic prognosis for a long healthy life. I see the same in Ruby’s future. Sending love and prayers for good health.
Every time I read your posts about Ruby my heart goes out to you both. As a mum, I know how scared you can be for your children. Just wanted to say that I’m thinking about you and have everything crossed for her. She’s gorgeous!!
Dawn xx
I’m so glad she is doing so well for her condition. She is adorable! I will be keeping her in my prayers! Hugs : )
Thanks for explaining. I am glad she’s smiling and happy despite the heart problems.
Big, giant hugs to you and your whole family. She’s gorgeous and has such a special glow about her. You are in our thoughts and prayers. Stay strong momma xoxoxo
You are inspiring and so is that bundle of joy – Ruby! I am so happy to hear that despite everything that could go wrong, she is doing relatively well. I will continue to pray for your family!
I’m glad to hear the prognosis is good! I’ll keep praying. I also understand how therapeutic it is to write everything out. Every time I’ve explained my mom’s breast cancer prognosis it’s made it seem like a smaller problem.
Ruby is adorable!
I think it’s great that you are so aware and knowledgeable about TOF. Not every parent is up to the challenge of understanding and Ruby is very lucky. I’ll be praying for your family!
TOF is something I hold close to my heart. I am 27 years and I have TOF. I was 7 months old when I had my first surgery and had another one when I was 22. I know without a doubt the Lord has been faithful and has seen me through all of this. I have two beautiful girls that he has given me and I am so thankful for the advanced medical treatments available these days. I will be praying for your sweet Ruby and for you as a mom. Keep the faith because God is the ultimate physician and He will have His hands on her heart. She is absolutely beautiful!
So sweet! We’ll be putting in some prayers for Ruby.
To those I was unable to respond to, I just wanted to say thank you! Such kindness:).
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I sincerely pray for your baby! May God clear all the abnormalities in the least possible way! And wishing a very happy and prosperous long life for Ruby! May God be with your family always. Believe in his miracles!
Amen! What a beautiful way to say it.
I was just going through some “Favorites” in my browser that I had forgotten about. Yours was from 2011, so imagine my surprise that you have an (almost) 3-month-old with a CHD. We just celebrated my daughter’s 8th birthday on August 29th. She was born with TGA (Transpostion of the Great Arteries), and she just had her annual check-up with the Pediatric Cardiologist. Two thumbs up!
When Rachel was in the hospital…and throughout all of these past 8 years….we use F.R.O.G. as her motto (Fully Rely On God). Every so often God sends a frog our way….maybe a book at the library, a t-shirt on the store clothes rack, or a real frog out by the pond. Our entire family accepts these as the reminder that God is there to hold us up. He wants you to know that He is here for you as well.
You are in my prayers. I will be watching your blog closely now. From one Heart Mom to another, God bless all of you.
God bless you and your family. Sending prays for Ruby! Thank you for sharing.
God Bless that little Ruby! Also, blessings for your whole family – it’s tough to deal with the day-to-day of an ill baby.
My son, who is now 9, developed myocarditis when he was 6 days old and we had tests upon tests, worries, stress, etc. so I know a little of how you may be feeling and my heart goes out to you!
We’ll be praying for Ruby’s operation and for a quick recovery and that the doctors are focused and Ruby is a strong girl.
I’ve just had my second baby – a girl this time -(yes, after 9 years!!) who is just over 1 month old.
Again, much love to you all and kisses to that gorgeous little girl!
Beck Jones
http://www.mrsjonesvintage.com
Oh my goodness, Ruby is so sweet! That smile makes me melt. I’ve been praying since I saw the first post about her heart condition. Blessings and what wonderful children the others are for helping out.
My daughter has three holes in her heart. A larger VSD and two smaller ones. She is two months old and gaining weight is challenge for us as well. She was put on lasix and captipril (blood pressure med) at three weeks old due to showing signs of heart failure. I am SHOCKED that your baby is not on any meds. Both of these meds would make her little body and heart not have to work so hard to eat, would help her gain weight, stop the sweating and make her more comfortable. The lasix will draw the water off her lungs and heart. I urge you to ask about them both. I can’t imagine her not on them right now. I also get asked if how old she is ALL OF THE TIME! I say her age and they ask what her birth weight was. I hope all goes well for Ruby!
Wow. I’ve just come around your blog for the first time since a very long time ago, and read about Ruby’s upcoming heart surgery. My son Ernest (just 6 months old) has had openheartsurgery when he was 2.5 months old. He was born with Down Syndrome and a large AVSD. Giving him away for that surgery was without a doubt the most difficult I’ve had to do in my entire life, but now I’m sure that it has literally saved his life. Since his surgery, he has finally started to gain weight and lenght (although now, at 6 months old, he’s only 59cm and 5kg400g – I’m not good at American measures…).
I wish you all the very best with the operation, which I’m sure Ruby will come through in the way Ernest has.
I am so sorry to hear about your struggle. We have a son (he’s 4) with a serious heart condition as well. He has Ebstein’s and I can relate to many of the things you are going through. I can say though, that he was on Lasix for about a year and it was the most benign of all his med (the one I felt most nervous about him being on at least). We are always praying for babies with heart conditions around the world and their families (especially after we go in for a full cardio appointment) and we will be thinking of you as you go in for surgery.
Wow!!! Your other kids sound amazing also.
Jackie
I just found your blog..and so I am kinda catching up