This is one of those nights that most parents never dream of, but heart parents face repeatedly. The night before a procedure with their child to which the outcome is uncertain. How do you explain how many times in the last 3 days I have teared up while thinking of the possibilities? How do you explain that gut wrenching feeling that you get every time you think about the next few days? How do you explain to co-workers who ask if you are ok that-no, in fact, I'm barely hanging on- but I won't let my children see that. Tomorrow, we will hand Addy over to the trusted nurses, anesthesiologists, and cardiologists- and pray that they bring her back just as she is. Some people say, "oh, it's JUST a cath". It's never JUST any procedure. For a child with a single ventricle functioning heart, something can go wrong with the simplest of procedure. Sometimes the heart doesn't like being probed with catheters, and sometimes lungs don't like being ventilated with an endotracheal tube and machine. For Heath and I, we have seen the cath cases that go wrong. Yes, it is less risky than surgery. But there is also less support there and no one "expects" things to go wrong as with surgery. How often we have seen it go not as expected. We have met some wonderful people through mine and Heath's experiences as being heart nurses, and now heart parents. We know that Addy has many guardian angels watching over her, both living and passed.

We were actually semi-prepared for her cath to be next Wednesday. Then on Tuesday, Dr Doyle's nurse informed me that he was going to be out of town, but there was a cancellation this Friday....Whooosh, that's my breath being sucked out of me and my heart skipping its own beats. Of course, we will take the slot, because otherwise it means waiting until after Thanksgiving, and Addy needs this now. Then again today, Mary Beth called me. I really hate seeing the Vanderbilt number and it not being my husband on the other line (calling me while he's Sooo busy at work). She said Dr Doyle's first case tomorrow was sick, so would we like to be there at 6:30 instead of 11? Again, that annoying sick feeling in my chest of my heart pounding...of course, it would actually be better so that Addy isn't a bear by 11 am and being NPO all morning. We do whatever we have to for her, no question about it. But please people, my sanity can not take one more curveball before this procedure! Preparing for a procedure is kind of like preparing for taking a huge test. You've got it all worked out in your head where the other kids need to go, what I need to do that morning, what time we need to leave, when should I be packed by....then someone tells you that you are out of time! Where's the Xanax?

So tomorrow, we will wake our sweet baby girl up from her crib, and put her in the car at 5:30 am. She will not know where she is going or why, but her favorite Bearie Manilow will be there, along with a pacifier as a backup. We will sit and hold her, trying to remember every moment with her, before they take her from my arms. Then wait. Wait for that voice on the other end giving good news, not bad. How many times Heath and I have been the ones that have been on the other end of bad news, it is totally different when you are the recipient. I'll wait for the moment that I'll see her again, safe and sound. I'll give her as many extra kisses and hugs as I can until then.

I will try to update again tomorrow night, as well as my FB page throughout the day. With any luck, Addy will be the trooper she has always been, and we will be resting in a room on 6C 24 hours from now. For now, I pray.

We have a bug! :(

Our cath has been moved up to October 5th. Hopefully at this time the cardiologist will do something with that L-PA so that she doesn't have to work so hard to play & breath. However, Addy likes to keep us on our toes... we thought Addy was teething...well actually she is. But it looks like after 4 days of watching her, she also has some sort of respiratory virus. I have watched her sats of the last 2 days while she plays (before she really got sick) and they were only running 60-75%. This is not good for her. The minimum her sats should be is 75%, although most of the time she should be 80-85%. I spoke to her cardiologist today and we have an appointment with her PCP in the morning. She hasn't eaten or drank too well in the last few days. The problem with Addy getting a virus is much more complex than a normal child. When your heart is already working harder, you don't really need or want a virus to make it work harder. Also, when she is working harder to breath, the vessels (that are already small) going to her lungs are affected, hence she has less reserve before she gets into respiratory trouble. She has been quite much more out of breath the last few days. I truly hope that when we wake in the morning and go to the PCP, she makes me look like a fool by acting just fine. Her cardiologist and I agreed to give her through the weekend to get over her illness, and we will re-evaluate her sat & activity intolerance issue next week (if we don't wind up in the hospital before then). If we still feel that she is progressing in her out of breathness, with low sats, her date will be moved up again to the next week or so. For now, I will continue to post until I know a more definitive plan. It's really not up to us, Addy drives this boat!

New Cath Plan

We called Addy's cardiologist last week because since her MRI, she has progressively worried us more. Our Addy has never been much of a napper, but over the last month, she is rarely awake anymore! Our typical routine has been that she sleeps until about 9am usually until I wake her up. She eats breakfast and by 10:30 she is ready for a 2 hour nap. About 12:30-1:00 I wake her up and she eats lunch, then a snack about 2:30, then it's back for a nap from about 3-5. She is awake then to eat dinner and by 7 she is ready for bed again. So on average over the last month she is only up about 5 hours a day! I have had trouble even getting 3 meals & 2 snacks a day into her because she is sleeping so much. She is resting much more often while playing, sometimes she just lays down on the floor mid crawl and rests- this never used to happen. She also is much more cyanotic while drinking and gasps to catch her breath. Six weeks ago she was taking 5-6 steps on her own at a time, but since then, she has completely quit trying and just has reverted to crawling Although increases in sleeping can be attributed to growth spurts, over the last month, she hasn't even gained 1/2 pound. That is also significantly down from what she has been gaining, which is probably due to less intake and harder work.

So after calling cards last week and basically demanding a sooner appt, and taking her to the PCP in the mean time to prove that she wasn't otherwise sick, they saw her today. Basically we know that her L-PA is small (3mm) and was going to need to be augmented. However, they were hoping to wait and augment it with an early Fontan. Our cardiologist explained that a lot of times kids can have small PAs and never even be symptomatic, so there isn't really a reason to explain these changes. However, we know our child, and this isn't her. I understand that children this age wine and have a certain amt of difficultness, but we can't even take her anywhere bc she is so tired she makes everyone miserable with squealing and crying nonstop. Luckily, our cardiologist does trust our judgement and after over 1 month of this, she feels that the safest thing is to go ahead and move up her cath date. They can get pressures at the very least and make sure that everything else is ok and their is no cardiac reason for this- or if their is then hopefully discover and fix it. Although this means a sooner procedure, it actually somewhat puts us at ease. My biggest concern is that Heath and I watch our daughter slowly work harder and tire out over the next 4 months, then go into cath or surgery week because she has lost all of her reserve. We know now that she is strong, and she will do good, but I'm not going to watch her suffer and then have them tell us she's too weak for intervention 6 months down the road.

Ok, enough update about the sad and depressing stuff- now I want to update everyone on what good things she is doing! Addy is quite the little talker- so far she says uh oh, no, yes (just tonight!), mama, dada, I did it, hey there, kitty, stinky, & hello! OK, so these aren't always crystal clear, but you can definately tell what she is saying! Even though she isn't walking free-style, she is breaking speed limits in her walker, and her favorite thing to do in it is run full speed ahead! She knows where her belly button is. She still loves to eat everything that is put in front of her, and usually wants to feed herself.

She wasn't too sure about it to begin with, but once I put her in my lap, it was on like Donkey Kong!

Beloved Bearie, her security object, he stinks to high heaven, but she loves him! Even in sleep, he is nearby!

Those big blue eyes, who could say no to her! She really enjoyed her cake for her 1st Birthday!

I have talked to other heart parents and read blogs where they often cite the same things that I think and feel everyday. Every single day, I think about the future and what could be. I kiss my daughter and hug her every chance I get because who knows when their won't be a tomorrow. Every smile and new thing I want to take note of and remember. I don't ever want to take her for granted or quit being amazed at what she can do. Every picture helps me remember every moment that I can. I want Addy to know how hard Heath and I will fight for her, and when she gets older for her to be able to fight for herself. I want her to grow up to be a strong, independent woman. I want her to be able to have a family, fall in love, and have a child. I don't know if all of these things will happen, but I thank god for today, and for tomorrow, and pray for thousands of tomorrows to come. And this is why, with the fierceness of a mother bear, I will fight for her every step of the way. I am a heart mother.

HAPPY BIRTHDAY ADDY!

As many of you know, Addy had her cardiac MRI last Friday. The reasoning behind it was because they were unable to visualize the Left-PA, or vessel that feeds her lungs blood supply. We were hoping it was just because Addy was being an uncooperative patient (imagine that!) but that unfortunately is not the case. Addy's L-PA is only measuring 3mm at this time. Enough for her to get blood supply, but way smaller than it should be. This means that it is not growing as she grows. Unfortunately, this is often a problem area because of the Aorta lying on it can compress growth.

Our team of highly intelligent doctors presented her at cath conference yesterday. Cath conference is the weekly meeting where all of the cardiologists and surgeons get together and discuss (or argue) about what the best plan for complex patients who are looking at surgery. If you ever want to see what your friendly cardiologist REALLY acts like, sit in on one of these meetings! All the sudden there are so many egos in the room that you never would have even suspected from the nicest cardiologists (note I didn't say cardiac surgeons as their egos are usually already apparent).

So that plan that they have come up with is this...normally the last stage of palliation surgeries is done in the range of 2-3 years. They usually have a heart cath done about a month or so before hand to look at pressures and make sure they are ready for surgery. They have decided to move this cath for Addy up to in the next 3-6 months. At the time of the cath their are several things that can happen. The goal is to be able to go in there, take a look around, and hopefully see that the vessel is big enough and they don't have to do anything. If this is the case, Dr Bischell will surgically augment it at the time of the Fontan while he already has her chest open. The second option is that the vessel will need some sort of intervention right then in which case the first option of that would be a balloon dilation. This is commonly done but carries its own risk when you think about stretching something with a balloon sometimes things tear and that would be very bad. The second option that could happen during a cath is that if the vessel is too small to balloon, they could have to place a stent. A stent would physically hold the vessel open in that spot but would also be harder to work around during the Fontan. Dr Bischell would prefer that they not do anything to the vessel until he gets in there. That means there is also a chance that if they get in there they could decide right then to take her on and do the Fontan that day. If they are just able to do the cath and get out, the date for the Fontan would probably be pushed up until she is about 16-18 months. If she were to lose complete blood flow, it would be life threatening, hence why it is so important to do something now.

This is not really news that we wanted to hear the day before Addy's birthday, but unfortunately this is our life. Many people don't understand that having a child with a hypoplastic heart doesn't mean that they can "fix" her in 3 surgeries and she will be fine. The reality is that these kids will continue to need echos, caths, and surgeries throughout their lifetime. Many have to go in for "tune up" type surgeries because, as in Addy's case, sometimes things don't grow with the body like they should. Cardiac children also often have other multi-system dysfunction as well that takes the focus off of just the heart as a problem area. So please, although you may mean well by saying "oh well she only has one more surgery and then she'll be fixed", please don't minimize the true condition by forgetting that she may have a dozen more surgeries in her lifetime, after she is "fixed". Cardiac children endure more in the first few years than most people experience in a lifetime.

OK, now that I have updated everyone on Addy's Heart, I have more important news....TODAY IS ADDY'S FIRST BIRTHDAY!!! This day is so special to us because one year ago today we didn't know if Addy would make it to 1 week or month, much less 1 year! The last year has been a roller coaster of emotions but I wouldn't trade it for anything. Every day I am amazed by the things that Addy is learning to do, and I take no day for granted. A year ago today, I held my baby in my arms for the first time. She already had multiple IV's and was on medication to keep her alive until surgery, but she was mine! People often say "I don't know how you guys do it" Yes, we both work at the place that our child frequently visits, we have 2 other kids, and we are both in NP school full time- call us crazy. But this is our life. I wouldn't change a single part of it. When you're living something instead of seeing it from the outside, it's not an option. We didn't want our baby to have to go through so much pain throughout her life. But we are going to fight for her every step of the way, because this is our child. If it were yours, you would do it too.

Our date for Addy's cardiac MRI & bronchoscopy has been set for August 12th. We will have to be there at the ungodly hour of 6am. They will do the bronchoscopy 1st about 8am, then anesthesia will take her for the MRI from 10-12 o'clock. We will expect to be in radiology recovery for most of the day but they said if she does well she should be able to go home that afternoon. It makes me want to vomit to think about the possibilities that can happen in the 4 hours that she is away from us. We know she needs this though. As she gets more active, (i.e. closer to talking all by herself!) she becomes more out of breath and labored with her breathing. She often has a dusky color to her lips that improves as soon as I pick her up and make her settle down. How much of this is normal? Who knows.... we know she will have some degree of activity intolerance, it doesn't seem to hold her back so that's a good thing.

On the other hand, this will take place exactly one week before Addy's 1st birthday! We are planning a little party to celebrate this occasion with friends and family. One year ago, we didn't know if we would get to celebrate a first birthday with Addy. We had no clue what the future would hold. While that remains unchanged, the last year has been one wonderful worldwind as we have gotten to know our baby and rejoice in every step she takes! I'm so proud of my baby girl, she is amazing. I have realized that the fear of what will happen in the future will never go away. I just have to love her every day that she is here and know she is meant for big things. 

Hiccups along the way....

 I love my baby so much...I can't tell you how many times a day I think that to myself...I don't know what I'd do if I lost her. Unfortunately, these are things that a heart parent faces every day. However, I don't think I could possibly take more joy than when my baby girl squeals at the sight of me, breaks out in a big cheesy grin, or makes a new noise/word. I wish that I could take a picture or video of every second of every day, so that I never forget a single moment...we never know when the last might be. That's why when we have those hiccups that are expected- having half a heart and all, it is so scary.

Our last cards checkup looked good except that they were unable to get very good pictures due to Addy's unwillingness to cooperate...but it did look like she might have some left Pulmonary Artery narrowing (that goes to her lungs). Hence, we have been waiting for 2 the last 2 months for Cardiology to decide whether we were going to have a sedated Echo (by sedation team) or a cardiac MRI (intubated by cardiac anesthesiology). After a few weeks of watching sats we decided that yes, in fact, her sats DO run in the 80's and we should be ok with the Echo. However, then Heath and I started noticing something....

Over the last few months, we have noticed that Addy makes a very peculiar sound when she cries. Now we are also hearing it at night while she sleeps sometimes as well as if she gets overly excited.  Granted, my overly joyous baby doesn't truly CRY very often...although whining is a totally different story. When she cries we hear this rather loud and disturbing "eeeeee!" when she breaths in. This mommy (& daddy's) trained ears immediately cue in that this is not a normal sound (no matter what other people say!). Stridor. Not something we want to hear. When I think the word stridor this is what runs through this ICU nurses head.....stridor...laryg/tracheo/broncheomalacia....difficult intubation...airway trauma....trach...no beautiful words when talking....  OK OK, yea I know it's kind of twisted. But you can't turn off the ICU nurse in me and Heath...and we have seen that progression TOOOO many times. While this would not change how I love my sweet baby, no parent wants to go through that or have to make their child. So, we insisted on an ENT evaluation before we would allow them to decide on the Echo or MRI. Once again we are thinking in overtime of...stridor of unknown cause...sedated...unstable airway...bad situation.... Are all other heart parents brains thinking like this too or is it just our over education in the subject of possibilities that could happen?

So...we saw the ENT specialist today to ease Mommy & Daddy's fears. Although we have been told by several well meaning people that this can be a normal sound, you aren't going to convince us. Right off, Addy was not a fan of the fellow or the attending. It really PISSED her off that they dare to look in her mouth and ears. Those of you who have never actually met Addy might not know that she doesn't fit the profile of a "hypoplast" heart kid. She is bulldozer- hard headed, never tires (or doesn't stop if she does), not afraid of anything, and opinionated...just like her mommy & daddy! Needless to say, she let them know that their behavior was not going to be tolerated with loud screams- while at the same time demonstrating what we were talking about! So then they decided that they wanted to take a quick peek at her vocal cords by shoving this little camera scope up her nose....thank God we swaddled her because otherwise I'm fairly sure she would have decked them! See, when she still had that NG tube she was fairly used to having things put down her nose, then she didn't even cry. But now, I'm sure she thought that we were putting it back in or something because she was terrified! Luckily I had enough forethought to remember to make sure we had a pacifier, but more importantly "Beary Manilow". As soon as they finished and stepped back away from her she quickly calmed and shot them a dirty look while hugging Beary and clinging to me. I felt so bad for her! However, it did give us some answers....

So it appears that Addy has some extra skin that is kind of sitting up above her vocal cords that sometimes gets in the way when she cries, hence making the stridorous sound. Most likely this is an anatomical thing, not something that was a result of her previous intubations. Sometimes as kids grow, these pieces of extra tissue may grow as well, thus creating more problems. Most likely, nothing will ever have to be done, but it is good to know as it could potentially create more difficulty with accessing her vocal cords for future intubations. However, the doc would like to take a more thorough look at everything further down below the cords to make sure their is no other stenosis or narrowing that could also be causing it. So...that pretty much secures our future of a cardiac MRI. Sedation team is not going to be thrilled about sedating her with these questions and then they can bronch her at the same time as the MRI (which anesthesia will be present at). On one hand we are glad that we aren't crazy and neurotic overprotective parents that are making up imaginary things; on the other hand, I hope and pray that this is nothing that will affect her in the future.

On a lighter note, Addy is thriving in every other way! She is THE MOST active baby I have ever seen. As my mother tells me almost every day, "She's just like you!" She crawls amazingly fast, pulls up in a heartbeat, and is starting to play around with letting go of the furniture! She says "no,no, no" a lot, she has learned "uh-oh" and drops things a lot so she can say it, and seems to be repeating an assortment of other words. She is the only baby I have ever seen that actually whistles! I think it is because sometimes she purse-lip breaths to pace herself and figured out that she can make a noise when she does it. She loves playing in the swimming pool and is a true water baby. She now has 3 teeth and eats everything! At our last checkup she was almost 18 pounds, no puny heart baby by far! We recently went on vacation and she had her first trip to the beach! She didn't think much of the ocean, instead she spent more time in her baby pool on the shore! As I feel the need to photograph every event of every day, I have lots of recent pictures to post but here are just a few...please enjoy our sweet girl, we think she is awfully cute!

We just had one little pirate tooth here, but 2 more quickly followed!

Her first boat ride! The life jacket was bigger than her, she didn't smile like this for very long before it made her cranky!

Mommy & Addy!

Two peas in a pod! Maci is the best big sister!

Addy sure loves her piggy Mr Rooty! He was debating joining her for a swim!

Mrs. Independent likes to stand all by herself now!

The two drama queens, we are in trouble!

 

Rubber Ducky....you're so yummy! Water baby!

3 month post-op checkup- almost 9 months old!

I haven't written anything in awhile because things have been going so well. Addy is growing well and acting like a normal baby! She is up to 16 lb and 12 oz now! She is eating a lot of foods now but getting all of the formula in her that she needs is now a struggle again because she just doesn't want it! Unfortunately my child has taken after both of her parents in that she likes good food so she doesn't want to drink that nasty ole formula! She will drink water (or whatever I am drinking) out of a cup for the most part, but when you put formula in it she is like "no thanks!" We are now crawling....or scoot/crawling everywhere! My mother says that Addy is just like me when I was this age. I do not have a child that you can take to an event and she will will sit quietly on your lap. No, my child is flipping around in your arms, jumping up and down, turning upside down, and being a monkey!


Addy had her cardiology appointment today for her 3 month checkup following the Glenn surgery. On one hand it seems like we just had our last appointment. On the other hand it seems like it has been forever since we have seen them! Her blood pressures were a little higher and her oxygen saturations were a little lower than they like so that bought us an Echocardiogram compliments of the house just to make sure everything is ok. Clinically, Addy could not look better. She is nice and pink, warm, and active! Her echo showed that her arch is nice and wide open. The Glenn looks good and where they augmented her right pulmonary artery looks great. The only thing that is concerning that her left pulmonary artery (which looked fine at the time of the Glenn) was a little smaller than would be expected. This could mean 2 things 1.) They were just unable to visualize the area very well because it's difficult to see on a wiggly child... or 2.) this part of her pulmonary artery isn't growing along with Addy the way that it should. If this were the case, if it gets severe enough before the time of her Fontan, she will have to at some point go to the cath lab and have a balloon angioplasty done to widen it. Otherwise, they would aument it at the time of the Fontan. The only ways to tell for sure what this vessel really looks like are either by a sedated echo so that she isn't moving or by cardiac MRI (which she also has to be sedated for). A sedated echo is fairly simple, but they can only do it if her sats are above 80%. Otherwise, cardiac anesthesia will have to sedate and intubate her for a cardiac MRI...a little bigger of procedure then. So for now we are scheduling the MRI and I am going to monitor her sats (something we haven't had to do for 2 months) for the next few weeks to see if they really are running in the 70's most of the time.

I wouldn't say that I'm upset but of course I'm concerned. The concern never goes away when you have a child working off of half a heart everyday. I think it's almost like I expect something to be not quite right every time each time we go for a checkup. Addy has had a good run so far, but I know that no child is without the ups and downs associated with being a single ventricle. When Heath and I tell people about Addy, they often ask whether her heart can be repaired, and will surgery fix her. They think, oh ok, she is doing good so everything is great. If only it were that easy. How can I convey to people that even though technically 3 surgeries will "fix" her heart, that is only just to make it physically compatible with life. Over the years, she will still likely encounter these kinds of things. There will always be echos, caths, and EKGs to make sure everything is functioning just right. She will always stand the risk of going into complete heart failure and needing a transplant. There is a good chance she will need balloon angioplasty's or valve repairs, or some other kind of maintenance surgeries or caths in the future as maintenance. It's unsure at this time how much activity restriction Addy will have. Some kids run and play sports, but others just don't have the energy.

So immediately following our last appointment, Heath and I decided we were done with the feeding tube (see previous posts) Addy has done wonderful and as you can see according to her pictures, she looks perfect! She's not thin, but she's not a sumo baby (which cardiology likes to make by making tube feeding dependent children and leaving them on strict feeding regimens of high calorie formula for FOREVER!) Well, apparently Addy is only sitting at the 25% mark on the growth/height chart now. Her doctor said if she falls anymore, "we will have to consult with nutrition" First of all, last I checked anywhere between 10% -90% is considered acceptable. Second, if my child didn't have a heart defect and you looked at her, would she still be concerned? Third of all, after our last checkup when I asked about where we should go with nutrition they told me to talk to my pediatrician about it because she could handle it from now on- OK then, so butt out! Fourth, is the fact that Addy is almost 9 months old and my cardiologist is telling me that I need to make her drink more formula and eat less food...because the food isn't going to give her the calories she needs. I'm sorry...are we going in reverse aging? Last I checked as babies got older, they were supposed to eat more food! I just have a problem with the fact that as we near a year old, they still want us to shove this formula down her throat all day instead of eating like a normal child. At 1 year we switch to cow's milk, which is about 20 calories per ounce. Right now we are on 27 calories per ounce. So what are we supposed to do then- feed her formula until she's 2 years old? It infuriates me when doctors look at a chart which show's a Bell curve of what MOST children fall within and say that my perfectly nourished child is too small.  Who exactly made this chart that says you're not feeding your child enough if they don't follow this curve to a T? Ok, so what I am going to do then is a nice little calorie count for the next 2 weeks to show them that she is getting plenty of calories, and growing just fine. I know my child better than they do. Sorry, that's my rant. Those that know me well know that the way they treat feeding and nutrition is something that I feel is a big issue with taking care of heart children, even at our "top notch" facility.The pics don't look like she has any trouble eat does it?

 Baby girl loves her oatmeal in the morning!

 

Do I have any on my face? 

Recently I saw a posting on fb about a single ventricle woman that had a child. In the past it typically hasn't been recommended for women with a SV to get pregnant due to the strain that it puts on even a healthy womans body, much less half a heart. Although some have been given the OK to get pregnant medically, the risks are still huge. Who knows where we will be in 20 years with care of these patients and how far technology will have come along to help them make it safer.This brought back the memory of the day that we got the definate diagnosis of Addy's heart condition. We were 24 weeks pregnant, and 24 hours before we thought we were having a healthy baby girl. Heath and I had just clung to each others' hands as we watched an echo that we knew didn't look good. The mourning had already begun before she even told us exactly what the details were. I remember our cardiologist going over all the statistics, options, and what the future looked like (Which we both already knew better than we ever wished) I remember her telling me that the likelyhood that she would ever be able to get pregnant and have her own babies was small. Something about that has always bothered me. For one, I feel like when we are discussing whether my child is going to live past infancy to adulthood or not, I feel like adding in the second left hook that, "oh by the way, if she did, she could probably never be a mother" is just a little insensitive. At that time, what we were most concerned with was were we ever going to get to hold our baby and take her home. Now, I feel sadness at the possibility that Addy might never be able to experience the joy of carrying a baby in her body, the bond, and being a mother. Will she be angry if she can't? Will she put her own life at risk to have that experience? There is a 20% chance that a woman with CHD will have a child with it as well. This is just one of the things that as a heart mother we have to consider, that other mothers don't.

 The first time she has ever had enough hair to put a bow in it (just barely)!

Ok, so now that I have hit all he high points and blown off some steam I will keep updated on how her sats look and if we wind up doing the cardiac MRI or sedated echo. Right now both girls have a nice little cold so Addy is a bit of a beast. I'm hoping it doesn't get any worse than it already is but it still worries me. It's hard not to freak out every time she gets sick when you know that a cold could potentially put your child in the hospital, or on the ventilator. More soon to come....enjoy the new pics, I might be prejudiced but I think she 

sure is beautiful!

 Modeling her new swimming suit for the beach!

Taking baby steps, just need a little bit of hand holding still!

How time flies, already 6 months old!

It's hard to believe that our baby girl is already 6 months old! Addy is doing wonderful at home and some days it is easy to forget that she has half a heart! She has made many strides in the last month...
After successfully getting rid of our feeding tube over a month ago, we have not put it back in one time! I still have a stash of them just to ward off evil spirits but I'm not counting on having to use them. I think everyone should have a spare NGT around just in case their child is sick and won't eat! She is doing great with her feeds since switching her to Similac Sensitive formula. She had her 4 month shots last week, at 6 months...she weighed 15 lb and 4 oz! She is hanging in the 30-45% percentile for weight and height, which is perfect (anywhere between 10-90% is good). We decided that even though flu season only has about a month or two left we went ahead and tortured her with a flu shot since she is now 6 months. She didn't feel too good for a day or two but it is worth it if it keeps her from getting sick. She might get one more month of RSV prophylaxis but by then hopefully the majority of the winter sicknesses will have passed without her getting them! We don't have to to oxygen saturation checks anymore but my best guess would be that she hangs out somewhere between 80-88% most of the time.

Most days Addy drinks about 20-24 ounces. I still stress out when she has a day where she doesn't take her "goal". It is hard for me to give up the thinking that she has to take 100% of her volume every single day since for so long she had to get every drop to make her gain weight. Even though we are now feeding on demand, I still don't feel like our feedings are "normal", nor do I know if they will ever be! We are still technically giving her 27cal/oz fortified breast milk but I am starting to wean down the calories to a more normal amount so that she isn't drinking quite such a milk shake all the time. We are finally getting 6 hours of uninterrupted sleep at night! I usually give her 5-6 oz right before bed, then she wakes up about 5 am to eat again, and then goes back to bed until about 9 am....thank god she's not one of those early riser babies! She is a good baby and takes her medicines by mouth like a pro though! Her color remains great, she is nice and pink with rosy cheeks. Sometimes the only time the seriousness of her condition slaps me in the face is when she gets cold or is crying and her hands and feet turn dusky. It's then that the nurse/mommy in me starts running through possible scenarios and the mommy monitor for complications is kicked into high gear for awhile. Always she goes right back to her normal self but it still is a reminder of how fragile she is.

In other ways, Addy is as normal as can be! We are finally doing a good bit of baby/table foods and I have learned that she MUCH prefers real table food to the bland baby foods. I'm positive that it is not in my imagination that as soon as I sit down to eat she decides to scream, hence she also tries to grab every piece of food I have out of my hand! In the last 2 weeks she has discovered the whine squeal of "I'm not getting what I want", and is taking full advantage of that. She now decides when she is going to eat, and quite loudly voices when she is NOT. She is mobile, rolling over and over, as well as scooting herself around and round in circle in her crib. She eats EVERYTHING that you don't want her to, I'm decided she is part goat because she will gnaw on anything you give her a chance to! She is no longer content to just sit in her car seat when we go places...she needs to see and be involved, and for the most part the center of attention! She can't quite sit up completely by herself unassisted but she is tripoding and I think that will come any day now. She can put her own pacifier in her mouth, although I always know she did it herself when it is in her mouth upside down. All of these normal baby things are such a joy for me to be able to see in my baby that at one point I didn't know if I would ever get to bring home. Every day is a blessing.

We have gotten a lot of really positive feedback from our announcement for our plans to start a foundationt to help families of children with congenital heart disease. We have started the process to formally start the foundation and are waiting on all of our federal numbers and official paperwork. We will welcome any donations of any kind, but right now it can't be claimed as a tax deduction. We soon as we get everything in we will post more information to start working on our first fundraisers!

Officially Tube Free

So just a quick update over the last few days...since we changed her formula to Similac Sensitive Addy has become a different child. She has now been without the tube for 6 days, life is finally starting to feel normal. It is so nice not to have to fight with her to take what she will by mouth, check tube placement, bolus the rest of the feed, flush the tube-and then do it all over again in 3 or 4 hours. In between that it was always highly likely that she would pull the tube out, get it caught on something, or the tube end would pop off and one of us would wind up wearing nasty formula. Although I'm still a little paranoid she isnt' going to eat enough,she is eating 22-24 oz a day, I think today we may be tipping the scale at 26oz! For the first time since Addy was born, she is actually crying when she is hungry! You can't believe how much easier it is to wake up at 2 am to feed a baby when she is crying, not just an alarm clock going off! We haven't been concentrating much on working on baby foods this week but I think after next week if she is still doing well we will starting adding more back in once I'm sure she is taking enough fluid. She hasn't thrown up one time but their do seem to be a lot of dribble spit ups since changing formula. This is mostly probably due to the fact that she chugs 4-6 oz in about 10 minutes. She seems unphased by the spit ups though and to me the trade off for the fact that is is taking all PO is worth it. Her next RSV shot and immunizations are next week so we will check her weight then but she looks nice and well fed in the mean time. Being able to feed our baby like a "normal" baby is such a big step in being able to "normalize" our life, it feels as though a giant weight has been lifted.

Day 2 of tube wean

So, we have been without the tube since Thursday afternoon after Addy's appointment. Thursday she didn't seem to notice a difference, she got most of her fluid for that day. Friday I went to work so Daddy was left at home with Addy & Maci. Heath hurt his back this week so on second thought this was probably not the best idea to leave her with him by himself on the first day of the wean. He can pick her up but it is difficult, and he is still in a good bit of pain. He has an appointment next week to get it checked out, as well as some bone changes in his hip that showed up on unexpectedly on an xray.

Friday morning she didn't take much by mouth and by 11 am she had cried for 2 hours straight and was refusing to eat anything, bottle or food. Heath finally broke down and put the tube back in and gave her a few ounces. That seemed to satisfy her and then she fell asleep and took a nap. That afternoon she took a few more ounces but didn't eat great, by 3 or 4 that afternoon she had pulled her tube out again. This time he didn't put it back in and just let her PO what she would take. By the time I got home she was in a better mood and eating a little better. I think for Friday her total intake was about 12 to 15 oz, 3 was by tube.

So day 2 of our "wean"....just to clarify we had been letting her go sometimes without tubing the rest of her daytime feeds occasionally througout the last few weeks but had been hesitant to really "wean" until we got our clearance and cut her lasix back to once a day....anyway, starting at 1:30am she started eating today. This is the first time that I think Addy has ever woken up in the middle of the night since we brought her home to eat on her own. We have always woken her up when she would have otherwise slept. She took 3 oz at 1:30, 1oz at 5:00am, and 3oz at 9:30am. This feeds have been on her cues for the most part, when she refused the bottle we did not push it. Heath and I went to see Dave Ramsey speak today so she was with Grandma and Grandpa for the day. We have always said that it just seems like Addy doesn't like milk (especially since she will suck down pear juice from the bottle) but we have never changed her formula that we are fortifying her breast milk with. My mom finally decided she was going to do some research because she thinks it is because Addy doesn't like the Goodstart taste...it does smell like sour milk or vomit, either way it's nasty. So she found that the only formula that the ingredients started with corn syrup was the Similac Sensitive...I'm not sure what the other formula's first ingredient was but she says this one was different. So mom bought a can and tried it with her today. Honestly I dont' know if it's the formula or that her hunger drive has finally kicked in but she ate better today overall than she ever has. She took 8 more oz between when I dropped her off at 11am and 6pm. Then, when we got home she took 4.5 more oz for me at home! She took the whole thing without any coaxing or fussing or distractions! I think this is the most she has ever taken voluntarily while she is awake so I am really excited! Today was just the beginning so who knows how she will do tomorrow but I am optimistic. It is late now and she is still in their playing & fussing instead of sleeping so I will update more the next few days.

6 week Glenn clearance- Goodbye tube!

Well, we finally made it to our six week followup!! Besides an unusally long echo due to a squirmy 5 month old, it was fairly uneventful as far as appts go. Her echo looked good, no sign of restenosis of her aorta and her flow looked great. We won't see cardiology again for 3 months....that's hard to believe. More on that in a few minutes though. The last 5 months has been a roller coaster, although I feel that we have been lucky that we have had more ups than downs. We have been blessed to meet a lot of families along the way that are on similar paths as us. Some of their paths take good turns, and others don't. Every time that I forget for a moment how precious her life is, the reality that another family is dealing with reminds me of just how precious every good day we have is. There are people that feel sorry for us that Addy has been born with her heart the way that it is, but we don't want pity. We have long since accepted that this is what we have been dealt, and I wouldn't trade my sweet girl for anything. Every day with her is a blessing. She is such a joy, her laugh makes time stand still. Her sweet smile is enough to melt my heart. I have accepted with open arms that she will be spoiled rotten when she gets older because I don't know that I will ever be able to say no to her. I feel lucky for every day that I get to scoop her up in my arms and cover her with kisses as she squeals with delight.

The last few weeks have been great, I think we are finally getting to start enjoying our baby like a regular child! She has officially reached the fun stage where every day she seems to learn something new! The list of things that are new is quickly growing...She is officially ticklish. She has found her feet and loves to swing them in the air and grab hold of them. She squeals so high pitched only dogs should be able to hear it. She can now put her own pacifier in her mouth. She absolutely has to see everything going on in the room, and will completely flip herself around or crane her neck around while you're holding her just to see. She still doesn't really like the bottle, but she LOVES pears. Her favorite object is still Bearie Manilow. She is already addicted to television, her favorite thing to watch it in is her jumparoo so that she can jump up and down when she likes something. She likes the sippie cup but mostly just as a chew toy. She is endlessly fascinated with Cookie (dog) and loves to pet her. She can roll OFF her stomach, but not all the way ON it. She thinks that everyone's lap is a jumparoo and attempts to vault herself off it it when you hold her. Those are just a few things that have become apparent in the last few weeks that I love about my sweet baby girl.

So, back to our appointment today. So the biggest reason I was excited about this appointment is because we are ready to be done with this stupid feeding tube! So...the last few weeks we have changed Addy's feeding schedule. I mean, really, how ridiculous is it that at 5 months old we are still easting around the clock every 3 hours...without her telling us she is hungry? We started out by feeding her 4-5 oz 5 times a day and she has done well with this. We are at least getting 6 hours of sleep at night now, and she sleeps like a baby should! Dr Kavanaugh didn't have a problem with basically letting us push her to eat more during the day and not tubing the rest. However, she still wants us to supplement and make sure she gets all her calories and fluid every day. Personally, after doing research I have a different opinion so I'm pretty much just going to do what we feel is right. I mean, between Heath and I we should be able to know how to feed our own kid, right?! We think that Addy is telling us that she is done with this tube too, because she has pulled htis tube out at least twice but up to 4 times a day for the last week. We can barely even keep the thing in now! We have skipped giving her what she didn't take some, trying to get her to get hungry, but I think it's going to take more than just a day at a time. So here we go, no more tube!! We're going to watch how much she takes over the few days and try to actually get her to learn what hunger is. It is time to let her act like a real baby. She only gained 200 grams the last month but she grew at least 2 inches so we're not too worried about her weight at this point. My wonderful friends with normal babies the same age keep reassuring me that their babies don't take that much more than she does, and they're not force feeding them with a tube! So the math that Dr Kavanaugh gave us was that she needs 100ml/kg which would be 600ml a day. OK....so, that's what she is just about getting but we are still on 27cal/oz breast milk & formula...once again, why? If she is getting what she needs, why are we still stuffing her like a thanksgiving turkey? Does anybody else get hungry when they are essentially drinking a milkshake every 3-4 hours? So stay tuned, I'm going to try to keep everyone updated as we go through this journey. Although we love our doctors and hospital that has given our little girl a fighting chance, we are extremely disappionted with their lack of weaning protocol to help these kids get off of feeding tubes. Although we have a lot of medical knowledge, this isn't something that we deal with every day (except with our own child!) at work. We feed them through tubes and don't worry about the ensuing tube dependance that we cause when they go home. Here we go....

Our Sweet Baby Returns...3 weeks post-Glenn

I haven't written much lately because the last few weeks have been tough. Overall, I feel like Heath and I were better prepared for our journey than probably 99% of heart parents because of our congenital heart and PICU background as nurses. This was true up until we took Addy home post-Glenn. In our defense, we didn't even see our cardiologist pre-op to discuss the post-op course because "oh you guys already know the routine...no questions, right?" was what they said.

So, after our 5 day stay we took our sweet Addy home. Within 24 hours we quickly realized that this was no longer the sweet baby we handed over before surgery. She was a short tempered, clingy, incredibly moody baby who sort of resembled a pissed off smurf while she screamed for periods of 30 minutes to an hour at a time. Addy quickly became affectionately known as "dragon baby", for she looked as though she were going to spit fire at us any minute. Every time I attempt to lay her down while she sleeps, as I gently lift her away from my body I see that she is holding her breath in preparation for the neverending scream to come. She's sneaky all right- she appears to be sleeping soundly, yet she detects the slightest attempt to lay her down and is not going to have it.

On day 9 we called the cardiologist office....We asked, "Is this normal? We are shoveling Tylenol and Oxycodone into her on a regular basis but this is just not our child". Basically the answer we got was yes and there is nothing they could do but the headaches should only last about a week and she will start getting back to herself. Great, meanwhile my ears have been ringing for 4 days from a child screaming in my face. There is no worse feeling than knowing your child is hurting and being unable to console her or take her pain away. I feel helpless. We are tired, frustrated, and worried that our sweet baby is not going to return. In our defense I will say that Heath and I do not usually see these kids this far out of surgery. We take care of them while they are nice and sedated, and intubated. Then, when they come off the vent, we give them nice generous doses of Morphine until they are sent to the floor happy little campers on heavy narcotics. What we wouldn't have given for some Morphine the last 2 weeks....

2 weeks post-op, we have our follow-up appointment with Dr Kavanaugh. Yes, the screaming episodes are still going on. In addition, Addy has basically refused to eat anything by mouth since we brought her home. She starts to suck the bottle and then starts screaming bloody murder and won't take any more. Dr Kavanaugh assures us that the irritability is normal and in time it shall pass. She suggests giving it the full 6 weeks of post op recovery before we get concerned. Easy for her to say when she doesn't see how miserable she is. She also says no more Oxycodone for her....ok, can Heath and I have some? Now that the Glenn is done she should be burning a significant amount less calories than before but she only wants to go to 27 cal/oz formula right now to give her time to heal.

3 days ago- OUR SWEET BABY RETURNED TO US AND DRAGON BABY HAS LEFT THE PREMISES!!! At 3 weeks post op, the day finally came. The day when Addy finally seemed to turn the corner and start acting like herself again. Up until now Addy still would not take anything by mouth without screaming but it is slowly improving again. She now smiles constantly and is actually more of a clown than ever before! It seems that she has lots more energy than she did before, which is good except that she refuses to even nap because she doesn't want to miss a minute of anything! She has found her voice again and coos, aahs, and squeals constantly. She knows just what gets our attention and finds it funny when her antics work. She is learning to eat baby foods, even though we technically haven't been given the go ahead to start adjusting her feeds, I am. When I inquired about starting to push for PO feeds and starting more foods the cards nurse basically blew me off. We have come to the conclusion that Addy has gotten a bit spoiled with the NGT and knows that she doesn't have to eat by mouth to get a full belly. They said that now she isn't as high risk anymore, so why are we continuing to do Q3 hour feeds around the clock? What other 5 month old do you know that has to get woken up in the middle of the night to feed when they are not hungry? Tomorrow is her next RSV shot so hopefully I will be able to get a good game plan from the pediatrician but otherwise I think Momma Bear is going to have to take maters into her own hands.

The last 3 weeks have been a physical and emotional rollercoaster, for all of us. I thank God every day that Addy has done as well as she did. I read some other families blogs about their journeys and I realize that it could still be so much worse. Some days I have self pity but for the most part I think the fear is still the worst part. I know what the "worst case outcome scenarios" are, and although those are the last things I want to thing about happening, sometimes you just can't help it when the possibility is staring you in the face. I don't think there will ever be a point where we will go whole days, weeks, or months without examining every aspect of Addy's appearances and behavior for possible signs of trouble. We know only too well that she will never be completely "out of the woods", but I am going to make every attempt for her to be able to do everything as though she were complately healthy. I am looking forward to the days where there are no more NG tubes and she can eat like a regular baby. I still believe that Addy was intended for something great, otherwise why would she have been given us to take such excellent care of her? Coworkers often say to Heath and I that they just don't know how we do it. On one hand, I feel like we barely do some days. But I would say that response is mostly based on our sleep deprivation over the last 4 and a half months. Otherwise, my response is, how could we not? For every frustrating moment that I have about feeding, or medicines, or irritability- it only takes one smile from Addy to make it worth every minute. We didn't ask for Addy to be born with this, but we know that God has an ultimate plan for us all, and that we will fight to the edge of our life for her. In many ways I feel blessed to get to be Addy's mom. She is my biggest hero for what she goes through every day of her life.