CHD Awareness Month 1 & 2

February is Congenital Heart Defect Awareness Month. I'm sure I've spammed you enough that now you know that 1 in 110 babies is born with a heart defect, making it the most common birth defect.

I didn't know that until Helena was diagnosed, so I'm guessing you didn't either.

In honor of CHD Awareness Month, I'm going to post following the Mended Little Hearts prompts - one for each day.

Today I'll cover February 1st (Diagnosis) and February 2nd (Surgery/Meds/Intervention).

Diagnosis. Oy. Well, as I've written about before, Helena's diagnosis came at our 20 week ultrasound. We went in assuming that everything was fine, and already knew she was a girl. When the ultrasound tech goes from super chatty to super quiet and spends a great deal of time on your baby's heart you know something is up. She left the room, saying that the doctor would be in shortly to go over everything with us. When the doctor came in, he immediately told us that there was something wrong with the baby's heart, but that he wasn't sure what. Luckily for us, the pediatric cardiologist was at the hospital that day and agreed with meet with us immediately. He took a look and said he wasn't sure what was going on because she was small and moving too much. When we went back four weeks later, he confirmed that she does have a TGA with pulmonary stenosis, and a VSD. We've also learned that her coronary artery is in a weird place, because why not?

What's weird about getting Helena's diagnosis is that neither My Husband nor I were shocked by it. I cried. We were upset and sad and scared, but we weirdly weren't shocked by it. I'm not sure if it's because that's just how we are - we see the hand we're dealt and we immediately figure out how to play it, or if there was something else going on. But we weren't shocked, which is weird.

I feel lucky that we did get a diagnosis so early. We were able to monitor her throughout my pregnancy and make sure she was growing and developing well. We were able to switch hospitals so that I could deliver at the hospital with the best pediatric heart team in the are. We knew. We knew she had a serious defect. We knew she wouldn't be coming home with us. We knew there was a lengthy NICU stay ahead of her. We knew it was going to be rough. We had time to come to grips with the road ahead of us, and to prepare ourselves and the boys for it. We were able to get our support network in place. We were so very lucky.

We were lucky that we had a great ultrasound tech, and a doctor who realized something was wrong. We were lucky that our amazing cardiologist was on the floor that day and that because he's amazing he agreed to take time out of his day to meet us when we didn't have an appointment. We were lucky to live 30 minutes from the best hospital for Helena. We were just so very lucky. I couldn't imagine thinking everything was fine and delivering her and wondering why she was turning blue. It was scary enough as it was, with us knowing as much as we did. I can't imagine the parents who aren't lucky enough to find out about their baby's heart defect before birth.

Surgery/Meds/Intervention. Sometimes the though of everything Helena has gone through in her little life blindsides me and I just completely lose it. No baby who's only six month old should have had to be put under and cut open. No baby should have to get scared anytime she's naked on a table with a stranger because she already knows bad things happen to her when that's the situation. No baby should ever have to suffer pain, or be scared the way she has been. And it's not over. We still have her big corrective surgery ahead of us. No adult should have to suffer through that - let alone a little baby.

Helena has already had two cardiac cath procedures, and one surgery in which they did have to open her up and break her sternum to put in a shunt. She's been intubated for weeks at a time and had IV lines put in both her little legs. 

This is Helena 2 days after her shunt surgery, so she's just over two weeks old here. I wasn't allowed to see her the day after it because I came down with a crippling stomach flu the night after her surgery.

Helena in recovery after her most recent cardica cath. The owie on her cheek is from the tubie tape.

Helena isn't actually on many meds, considering what she's got going on. She's on Lasix, a BP med, and baby aspirin for her heart. Throw in some reflux meds and that's it. The nice thing about her NG tube is that I don't have to battler her to get her to take the meds. That's one thing I'm not looking forward to when she finally does start eating orally.

When she does go back in for corrective surgery, we're looking at about a two week hospital stay if all goes well. Then another 6-8 weeks of restricted activity while her sternum heals again. 

It's amazing how quickly something becomes "normal". It's normal for us now to have to resinert her NG tube. It's normal to have to give meds four different times during the day (they can't all be given at the same time because some interact with others badly). It's normal to take her oxygen levels a couple times a day. It's normal to do daily weight checks. It's normal to constantly be checking her coloring. This is just what our life with Helena is like. This is our new normal. This is what normal is like with a Heart Warrior.

So now I'm going to ask you for money cause that's how I roll. 

https://www.crowdrise.com/Corri-roarnrunvirtualrace

Go there. Give me money so that more research can be done to figure out what causes CHD's, and to help other families who aren't are lucky as we were with our diagnosis. Go help our Heart Warriors.

Update 1.10.16

I haven't updated in awhile and I'm sure you're all just dying to know what's been going on over here. We're just so fascinating, I know.

We all survived a variety of colds over the holidays that of course caused My Little Guy to wind up with an ear infection. Helena caught them too, but since she is a fierce warrior baby she handled them fine. She likes to do this thing where I'm looking at her and for whatever reason (lighting, my own paranoia, whatever) I feel like her coloring is off so I rush over and hook her up to her oxygen monitor and it spits out... 91. Ok, sure, yeah, Mommy is crazy. Good then. This exact scenario has happened at least 4 times now.

We were supposed to get the cardiac cath back in December, but Helena was in the thick of a cold and at that point and the nurses and doctors didn't want to risk anything by doing it while she wasn't 100% well. Her pediatrician and I felt that asking for 100% health during cold and flu season was a stretch (he cleared her for the cath saying her lungs were clear and her congestion was all in her head and that even as a cardiac baby she looked better than some of his normal patients with colds), but agreed that unnecessary risk was a bad idea.

The cath was rescheduled and we all hoped that she would be healthy for it this time around.

The issue was that in addition to having her NG tube in, she's also teething so she's pretty much in a constant state of congestion. It's hard to tell if it's from a cold, or the NG, or the teething or what percentage of those three things is causing it. Even though our cardiac nurse told us the cath wasn't urgent, we still wanted to get it over with. And it was going to give them the information they needed to help figure out the timing of her corrective surgery. As a planner, I needed that information.

As it worked out, My Little Guy's ear infection wasn't better after his 10 days of drugs, so the day before Helena's cath I took him into the pediatrician and brought her in for a quick once over. My Little Guy got another round of drugs (and it's sadly looking like he might need a third - poor guy), and Helena got the all clear for the cath. It did seem to make a difference when the cath nurses called that I was able to say that she just saw her pediatrician who said it's more teething and the NG than a cold and they said the cath is on.

So we woke up at 4:45 that morning, after not much sleep and brought her in. It's a horrible experience to say the least, but it's nothing compared to what we'll go through for her corrective surgery. Having to hand someone your baby and watch them walk away, knowing they're going to do things to her that no baby should ever have to have done is heartbreaking. You're literally putting the life of your child in someone else's hands. It's a feeling I wish no one ever had to feel.

Luckily the catheter procedure was only about 2 hours, so the waiting wasn't too bad at all. The doctor came out and said everything went well, she did great, and they got good pictures and video and information. We waited a bit longer while they woke her up and extubated her and brought her back to the recovery room.

When we finally got to see her she was PISSED. She was starving (no food since late the night before per surgery instructions) and uncomfortable and scared. Some snuggles and food and loves and she was out cold. Helena spent most of her time in recovery sleeping, but did great. She did so great in fact that they told us we could go home that evening. When we had scheduled the cath they told us that with the little ones they usually keep them overnight for observation just to be safe, but they felt she was fine and sent us home. I was very happy to not have to spend the night on that horrible hospital couch-bed thing with a constant parade of nurses coming in. I did end up sleeping on our couch since Helena fell asleep in her downstairs bed and I didn't dare move her, but as I told my husband - our couch was significantly more comfortable than the hospital one.

The next day Helena was more back to her usual self. She was still a little fussy, and a little needy but she was more smiley and slept less. By the second day after the procedure she was totally herself again.

Unfortunately she's caught another cold, but she's handling it fine so far. She has a six month checkup this week any way, so she'll see her pediatrician and make sure her lungs are clear and everything but I suspect they are.

We met with her surgeon who once again went over the two surgery options for Helena. Because she not only has TGA, a VSD, and a pulmonary stenosis, but one of her coronary arteries is in a weird place, he feels that one procedure might be safer but won't know for sure until he gets in there and sees everything. That's sort of what he had said before, so that wasn't a surprise. What was interesting is that he told us that even if she has to have the surgery requiring artificial parts (and therefore more surgeries down the line to replace them as she grows), they're hoping that in the next 10 years or so they'll be able to create the parts she needs using stem cells (in all the chaos of her diagnosis we forgot to bank her cord blood, but we did bank My Little Guy's so they might even be able to use that) and then since they'd be biological they wouldn't need to be replaced as they'd grow with her. That sounded super cool.

He also said that because her oxygen levels are doing so well and she is growing, there's no need to rush into surgery. He said let's wait at least another 2-3 months and keep seeing our cardiologist and when her levels start dipping a bit, that's when we'll start thinking about scheduling her corrective surgery. It was a relief knowing that she's doing well, and there's no need to rush anything. The bigger she is the easier the surgery will be, and the easier her recovery will be. He told us that even after this big corrective surgery she should only spend like 2 weeks in the hospital which is amazing considering what they'll have to do to her. Babies heal fast.

So for now we're just doing what we're doing since it seems to be working. She's teething like crazy and putting everything in her mouth which is huge progress in getting her off the NG tube. She's still not taking a bottle, but I did find one that she tolerates in her mouth more than anything else so we've been working on that. I find it amusing that the whole deal with this new bottle is that it's supposed to be for breastfed babies who refuse any other bottles, but Helena has literally never been breastfed and yet this is the bottle she has decided to like.

We've also started playing around with solids, or "goo food" as My Boys call it. She ate half a container of carrots over two meals the other day, so that's huge progress.

I think that's it. The rest of the family is doing well. We appreciate all the care and concern and thoughts and prayers from everyone. We are very lucky to have you all in our lives.

Update 11.19.15

Things here are going well.

Helena finally kicked that horrible cold and has been putting on weight like a champ. We had a cardiologist appointment on Monday, and he was very happy to see how much weight she's gained. Her cardiac functions are all doing what they're supposed to do. Her doctor did an echo on her and said everything looks great. They were planning on discussing her at the big cardiac meeting last night, but the general thought is that we'll do a cardiac catheter procedure in December to get a better idea of what exactly is going on in her heart. Then we'll hold off on the big corrective surgery until January or February. She's doing really well and putting on weight so they want to wait as long as possible to do the surgery. It's easier for everyone involved if she's bigger and stronger.

We also saw Helena's pediatrician. That appointment basically revolved around her reflux. We felt it had gotten a lot worse, and I wanted to know why and what we can do to fix it. Turns out that in all babies, not just cardiac ones on NG tubes, reflux peaks around 4 months. Which is how old Helena is. They start moving around more, which stirs up the reflux crap in their tummies and it comes up more. Her pediatrician said because she is gaining weight so well (she went from the 5th percentile at her 2 month visit to the 25th at her 4 month - that's 4 lbs she's gained in 2 months!) it's clearly not bothering her THAT much so he's not changing anything. Her job right now is to grow as much as possible, and she's doing that well so we're not rocking the boat. What we're doing is working, so we're just going to keep at it. She also measured in at 25 inches long, which is the 69th percentile for height. This once again allows me to make my "she's tall and skinny, like her mommy" joke. *snorting* Poor baby girl also got her vaccines (cause we ain't crazy) and she qualified for the RSV antibodies shot since she's a cardiac baby. All in all it was four shots, which was rough for everyone. I hate having to restrain her and watch her poor little face when the needles go in her legs, but she needs the shots. Especially the RSV antibodies one. RSV lands perfectly healthy kids in the hospital, it could be even more dangerous for Helena.

We're also still seeing a speech therapist and a physical therapist and are in the process of switching from private therapists at the hospital to Early Intervention therapists. All Helena's doctors said they don't want her going out in the world any more than she absolutely has to during cold and flu season, and the Early Intervention (EI) people will come to the house to see her. It also makes my life a lot easier because I don't have to arrange childcare for her brothers while I take her to therapy twice a week. I can just banish them to the playroom and they'll stay out of our way.

As far as therapy goes, Helena is also doing well. Her speech therapist loves seeing us because Helena is progressing so well so quickly. Her therapist said she's done more in the 6 weeks that we've been going than some kids will do in 6 months. We've gone from complete oral aversion (screaming, coughing, gagging anything we would attempt to put anything in her mouth) to oral defensiveness (allowing us to put a bottle in her mouth, but still being a bit scared and wary of the situation). She's still not taking her feeds by mouth, but she will let me put the bottle in her mouth and she'll play with it and swallow what comes out. She's lost her instinctual sucking, so we're working on ways to get her to eat that either encourage her to figure it out again, or don't involve sucking (I've ordered sippy cups and we might transition to those super early since without the valve in you don't really have to suck on them to get milk out). She's taking 20 mls orally a couple times a day, which sounds like nothing but considering a few weeks ago she wouldn't even open her mouth for the bottle and would scream if I tried to put it in her mouth it's huge progress.

In physical therapy we're working on general strengthening and stretching. After spending a month in the hospital Helena's arms and legs were a bit weaker than they should be. And because of the surgery we couldn't do tummy time for weeks after she came home, so she was behind on that as well.

"What the crap is this nonsense Mommy?"

She tolerates it now, rather than screaming her head off whenever we tried it, which was the case before. We're also working on reaching and grabbing at toys and generally getting her whole body to be more midline. From the reflux, she wants to keep her head turned up and to the right (apparently it's A Thing for all reflux kids), so we're working on fixing that, as well as getting her arms and hands more in the middle. She's just started really reaching for her toys, so we're really encouraging that too. A lot of "normal" development stuff is stuff we need to work on, but everyone who sees her says that she's doing really really well considering everything she's been through.

She's both holding and looking at the toy. This is big in our world.

All in all things here are good. We're going to keep doing what we're doing and hope it stays that way for awhile!

Therrr-a-pee

When Yiya was doing her physical therapy, she would pronounce it "therrr-a-pee" and it's one of those things that have lived on in our family.

Helena is now in therrr-a-pee too. We're seeing both a speech therapist and a physical therapist and she's making good progress with both.

With the speech therapist Helena has gone from holding her tongue in a defensive position (who knew there was such a thing?) to not, and she will actually allow things in  her mouth without screaming and/or gagging now. She's licking her hands, her blankeys, her lovey and anything that comes near her mouth. It sounds like nothing, but it's actually a big deal for her. Just two weeks ago none of that was happening. She's actually letting me put her nee-nee (Natarelli for pacifier) in her mouth now. She's still not latching on to it, but she's tolerating it in her mouth and playing with it in there which is big progress for us. Her therapist actually said she's made more progress in two weeks than some babies make in two months, so we feel really good about that. We're still not attempting any bottle feeds, but we feel good that there is progress being made.

We also just started physical therapy (PT) per the suggestion of our speech therapist. Helena's default position is to hold her head to the right looking up and away to the right. We learned that this is very common for reflux babies, as it helps ease the pain of the reflux. The downside is that her neck muscles aren't strengthening as they should be, and she's really tight on the left side. Her speech therapist said if we start PT it will help strengthen the muscles and it can help the reflux and I'm on board to do anything to help that so we signed up. I'm actually super glad we did because it turns out Helena really needs PT. Because of all her time in the hospital, her arms and legs are weak and in weird default positions from being strapped down and hooked up to everything. She despises tummy time with a vengeance, and frankly because of her surgery we weren't able to start it right away so she's behind on her head/neck control. The therapist showed me different ways to do help her work on that, as well as stuff to get her reaching and stretching more and get some more muscle in her body. I had no idea she was so far behind, so I'm so glad we signed up for this. We're already seeing progress there too - she's reaching for toys on her playmat and putting her hands in and near her mouth.

It may seem like nothing, but to us her sleeping like this is major progress

All of this stuff has thrown us for a loop. We were so focused on the heart stuff, we never thought about how the heart stuff would impact her regular development. We never even considered needing all this therapy, but we're glad that it's available and that it's helping her so quickly. It was just very unexpected for us, and I wish someone had warned us that cardiac babies almost always have reflux and/or eating issues and that she might have motor control and strength issues from her time in the hospital.

Otherwise she's doing very well. She's finally beat the cold (it took us like 3+ weeks to beat it, of course it took her a bit longer), she's smiley and she's interacting with people more. The one downside to her getting better control of her hands is that she's becoming too good at pulling out her NG tube, so I've resorted to putting socks on her hands anytime she's in her bed. She wakes up and is so quiet we don't realize she's up and then pulls it right out. We went through two in two days, which resulted in a call to our medical supply company to order more. I need more than 4 a month with this kid! She's also gaining weight well and is over 11.5 lbs now!

The plus side is we get no-tubie photo shoots 

So things are good. We're getting her the help she needs, and she's progressing and developing well with it.

Wait and See

We had another appointment with our cardiologist today, and it went well.

The cardiac team has decided on a "wait and see" plan for Helena.

She's growing, just not as quickly as they had hoped, but she is steadily putting on weight. She is still battling the cold (for realz, all the adults in the family are still battling the damn cold), and her cardiologist said it's actually a good sign that she's handling the cold so well. She's fighting it off on her own, which is showing how strong she is, so that's good news.

They're going to let her keep growing and see what happens. She's doing well right now so they don't want to rush into anything, and they're hoping in a few more months she'll have grown into her shunt and then they can put off surgery as long as possible.

The one thing we do need to work on is getting her to eat orally again. We've been doing dips, which means dipping her pacifier into her formula and putting it in her mouth while the NG tube feed is happening so she's getting some sort of oral stimulation, but she is still refusing the bottle and she's still not latching on to the bottle or the pacifier.

I've made an appointment with a speech therapist at our local hospital (NOT the hospital she had surgery at, with the stupid speech therapist we hated) and I'm hoping she can give me some guidance as to how to get Helena to eat orally again. She was up to half her feeds by mouth before the cold knocked her out, so I'm hoping we can make her relearn how to do that. So we'll see how that goes, I'm hoping it's a better experience than the one we had with the speech therapist earlier.

Other than that we're to keep doing what we're doing and let Helena take the lead and show us what she needs.

I'd also like to thank everyone once again. It's stupid and cliche, but it's also true - you really realize who truly cares about you when something bad happens in your life and the outpouring of love and support and prayers has been amazing and we appreciate all of it. Our eyes have been opened that some relationships aren't what we thought they were, but we've also realized how strong others really are. We have a long road ahead of us, and we're so grateful that we have all the love and support that we do going forward. So once again - thank you.

Despite all her troubles, she's a happy girl

Setbacks

We haven't had the best couple of weeks here.

What we thought was Helena's reflux acting up was actually a cold that she caught from My Big Guy (he also shared it with the entire family). The symptoms of reflux and a head cold are shockingly similar, and with the dire warnings the doctors and nurses gave us about Helena catching a cold we overlooked that possibility because she wasn't in that bad of shape. Luckily, we went to the hospital to get her stitches clipped and her cardiac nurse was all like, "No, that's totally a cold!" It was just good timing that I happened to make the appointment with them because her stitches happened to start peeking out. We would have just kept thinking it was the reflux.

The good news about the cold is that it explains her aversion to eating - she can't breathe while she's eating because her nose is so stuffed up. Once the cold clears up we're going to get back to trying to get her to take more food by mouth, but at least we have a real reason as to why she stopped her oral feeds. After getting some new meds and switching to Nutramigen the reflux seems to be doing much better, we just need to get the cold under control now. All her doctors and nurses agree that there's no point in trying to make her eat by mouth while she has the cold because again, she can't breathe while doing it.

We had a visit to the pediatrician who confirmed that the cold is just a cold and there's nothing we can do but let it run its course. Her lungs are clear and she's not running a fever so meds won't help with anything.

We also saw the cardiologist and learned that the shunt seems to be doing its job a little too well. Helena has too much bloodflow going through her heart right now. They put in a big shunt assuming that she'd grow into it rather quickly, but she's not growing as quickly as anyone would like so the shunt is still too big. As a result Helena has congestive heart failure.

Now, congestive heart failure is a horribly misleading name for what's going on. Her heart isn't failing. Basically what it means is that her heart is working too hard, which is is because it has too much blood flowing through it. The cardiologist and the surgeon and the whole cardiac team from our hospital met and discussed Helena and decided not to do anything right now, but to give her a little more time to grow into her shunt. If she grows into the shunt the heart failure will go away. In the meantime we've adjusted some of her meds to deal with the symptoms of the congestive heart failure and we're going to really try to get her to eat more. Her cardiologist said her heart functions all look great, again, it's just too much bloodflow going through it.

It's sort of horrible cycle again - her body is working so hard just to be alive that she's tearing through the calories she consumes so quickly that she's not gaining weight. But if we try to give her more food she's likely to puke it up, or it might cause her body to have to work more to digest more and burn even more calories. But she needs more calories to grow. We upped the volume of food a little, but we don't want to overwhelm or overwork her.

We go back to the cardiologist in two weeks and we'll see what he has to say about her then. Hopefully by then the cold will be gone and she'll have gained some serious weight.

Despite the setbacks Helena is still happy. She loves being snuggled, especially when there are butt pats involved. She has also developed a fondness for her playmat which her brothers love since they get to lay on the floor and "play" with her.

We're just taking it one day at a time, and hoping and praying for the best. Her doctors don't seem super concerned with the situation since they're giving her more time to let her body sort of work this out on its own, which we really like. Hopefully it does just that.

Tubie Troubles

When dealing with a sick baby, there are going to be highs and lows, good days and bad, and ups and downs.

We're currently dealing with more of a "down" situation in regards to Helena's eating. Her heart is fine, her cardiologist said she's doing well, but we're having some feeding issues.

Helena was on the NG tube, and we were doing great in increasing her oral feedings. She was taking almost half her feedings by mouth and doing well with it. Then reflux hit. She had all the classic, horrible reflux symptoms - gagging, coughing, spitting up, crying when we tried to orally feed and refusing the bottle. Since she needs to gain weight (her shunt is technically too big for her - they put it in assuming she'd grow into it so she needs to grow into it!), we just sort of stopped pressing the oral feeds rather than torture her (we knew it hurt her to eat by mouth) and did pretty much all NG feedings while we figured out new meds and a new formula (she's now on Nutramigen).

The reflux seems to be getting better, but Helena is still refusing any oral feedings. She's still gaining weight because we're using the NG tube, but she really needs to learn how to eat by mouth again.

We're working on it, but it's hard because she does still cough and gag when we try to use the bottle and it's so hard seeing that happen to her. I know it's awful for her too, but I also know she needs to wean off the NG tube and the longer we let her do just NG feedings the harder it will be.

We'd really rather tackle the issue ourselves after having a terrible experience with the speech therapist in the hospital. She only saw Helena three times and was dictating how she would eat. She claimed that Helena wouldn't/couldn't eat orally but that was because Helena was asleep when this chick showed up. This chick woke her up and then tried to immediately make her eat from a bottle. Of course Helena refused! This chick then assumed that meant that she couldn't do it, and recommended we only try feeding tiny amounts by mouth. How the hell do you make recommendations for a patient you've only seen three times (while we were there a month, it's not like she didn't have ample opportunities to come by) and are attempting to feed in a not normal situation? She also never called us back, and when I requested she come see us during one of our sleepovers she didn't. Her tips and tricks seemed useless and overall it was just a terrible experience.

The problem is that because Helena really needs to gain weight, we need to make sure she's getting all her calories in each day so we HAVE to use the NG tube to feed her. Well, the NG tube runs down her nose and throat and into her tummy. It keeps the pathway open for the stomach acid to gurgle up from the reflux. The NG makes the reflux worse, but we can't take the NG tube out because she won't eat without it because of the reflux. We're stuck in a horrible reflux-y cycle.

I plan on reaching out to some of her nurses for tips, but it's frustrating and hard. It sucks to see her suffer through her feedings, and not sleep well knowing she's in pain.

So we're in a valley right now. It's hard, and it sucks but we'll work through it. We have a great team of medical professionals helping us, and I am confident we'll get her eating by mouth again, it might just take some time.