The journey continues...post-op Day 5

Well, we have made it to post-op day 5 of the Fontan. It seems like ages ago that we were anticipating this surgery, and now we are just living the aftermath. It's amazing how much lower my stress level is at this point. I know that most people think that continuing to be in the hospital should be the most stressful thing. For us, waiting on the other side of those double doors, not knowing how things are going, and waiting to hear a real voice and see symmetrical breath sounds post-extubation are the most stress creating. Now we are just living it. It is just a careful dance of coordination for Heath and I to continue working a few days here and there, making sure our other children are taken care of, and making sure someone is there for Addy. Yes, it is exhausting, but as long as I watch this girl sitting up by herself in bed and watching her Dora while singing "we did it, we did it" and "oh no, Swiper the Fox", it doesn't seem so bad. Yes, it really does suck sleeping on the pull out couch, my bones and joints ache so much in the morning I swear I've aged 20 years overnight. The hospital food court choices get old very quickly, and our diet is nothing but a whim of a thought. But all those negative things pale in comparison to the joy we have with how well she has done. The defining moment came last night that we knew our baby girl was starting to feel better. She was sitting up in bed and I asked her, "Are you OK" and she says "Yea, I ok!" I will admit, I became a little weepy for a moment, but you just can't imagine how sweet those words were to hear. It means slowly but surely, our baby girl will be back to herself.

She made is out of oxygen yesterday and with good coughing is slowly clearing up the pulmonary edema and junk in her lungs. Her appetite has been great but she continues to vomit liquids (all but the 1.5 cal Pediasure that is so rich it's like a milkshake), probably because she hasn't pooped since Tuesday (i know, TMI). They are finally scheduling a bowel regimen so the hope is that her ileus will resolve with this and she will be able to tolerate fluids. The biggest negative is that her chest tubes both continue to drain a LOT. I'm trying not to dwell on this because this is the nature of the Fontan game, and I should be thankful that everything else is going so well. However, I can't keep those nursing thoughts out of my head of the "what ifs". It is ONLY day 5, and many are still in the ICU or on oxygen at this point so I should just quit my whining. But in the back of my mind, I keep thinking that we have had such a good course (quite unexpected and problem free), is this going to be our big downfall? Is this going to be what stops us in our tracks? Is this the other shoe dropping that we have been waiting for 2 years?

As always, I must continue to document our journey in pictures. I think it's as much cathartic for me as anything, so please enjoy! We would like to say thank you for all of the calls, texts, and visits we have received from everyone. The support helps makes this hellatious journey just a little easier at times.

This is right after we got to see her- Post op day 0, she still looks like our girl even with all the wires and tubes!

"Dare", he's always there!

The next morning, Post-op Day 1, she puffed up quite a bit overnight!

Finally extubated later on Day 1, she was much happier to have that tube off and a few wires gone!

Post-op Day 3 we made it to the floor, she still hasn't smiled or played yet and is requiring a lot of pain meds

She finally found her smile on Day 4! This was after she had her first bath since surgery and was feeling a little goofy! Pain is finally being managed a little better!

Kisses for Papa, he helped get her all clean and got plenty of smiles from her!

Daddy got kisses too, still feeling good at this point of the day!

My favorite contemplative picture, she is watching Dora and saying "Hmm"

Post Op Day 1- What a day!

Well, yesterday was a long day but we are happy to say we are on the other side of open heart surgery #3. They took Addy back about 8am yesterday. We were thankful that they tanked her up on Versed so it made a very scary moment for us much easier to smile through. Versed is a good thing....it hit her like a ton of bricks! All the sudden she was holding my Kindle closer and closer to her face, and then she was asking "where my Kindle go, where my Kindle go?" while she held it in her hands. She went easily with the OR team and we began our wait.

 To anyone that has never had to sit in the surgery waiting room...it is hell. A colorful assortment of families inhabit this area throughout the day as they wait for their offspring to various procdures or operations. Some of these families are wondering if they will see their child again...others use it as an opportunity to have a hillbilly family reunion- this phenomenon seems to be most commonly witnessed when the child is having a relatively simple procedure such as tubes in ears or an umbilical hernia repair that brings in family members from all branches of the tree. So...now that you have an accurate picture of what we're dealing with here, we waited...and waited...and waited. Dr B. finished up about 2:30 and came out to tell us exactly what we needed to hear. Everything was picture perfect in surgery, no unexpected complications. Her anatomy and pressures were the best we could hope for and hopefully this will decrease the risk of post-op complications.

We didn't actually get to see her until almost 6:00 last night, due to some breakdowns in communication, but the positive note is that it didn't have anything to do with how she was actually doing. The downside is that after placing the Broviac, and replacing it, the line just would not draw- thus we were unable to confirm that it was a good line in place. Throughout the night she was initially a little behind with fluid management but once all of that was regulated her urine picked up, lactate dropped, and gases were perfect.

This morning, she has started waking up with a vengeance! She had her foley taken out and we have started diuretics to start the drying out process. Her chest tubes are still putting out a good but reasonable amount. One of her ART lines has been taken out and the other ART and IJ central lines will probaby be taken out tomorrow. They were able to secure a new double lumen PICC line to serve as access until she goes home since the Broviac didn't work out. This is great because she was still sedated and never knew otherwise. The CV surgery NP finally was able come and pull her LA and Broviac lines so that means we are officially ready for extubation! This is good because she has been blowing through Dilaudid and Versed like they're candy all day- she wants that tube out! She has opened her eyes and shook her head no to a few questions, but mostly she just cries and cries whenever she wakes up. She will grab Bearie and hold him and has mouthed "Mama" a few times. Even as hard as it is for her to look at me and cry with a scared look in her eyes, it's all worth it. I'll take these tough parts over many more things we could be facing....    going to extubate, to be continued....



It's now 10:30 at night and the last 6 hours have been a blur. Addy extubated to nasal cannula and within an hour was on room air! She has had the O2 back on some just because she is still desaturating when she gets ticked and holds her breath but for the most part her sats are >92% ,which is better than we have ever seen! She is definately not a happy camper but I am overjoyed to be able to say that Addy is still the same girl we sent to surgery! The first few hours she mostly slept off all the sedation and occasionally woke up distressed. After 3 hours she began demanding "Addy drink, Addy drink" and "momma hold Addy". She chugged down 3 glasses of fluids in about 30 seconds a piece and just couldn't satisfy her thirst. Finally they gave the OK to advance her diet and she got her favorite food in the world "appleshaush". She ate half a container, demanded to be held (we of course complied), let out the world's biggest belch, and promptly fell asleep! She even slept through both of her breathing treatments and never moved! They are anticipating removing her IJ central line and other ART line later tonight (when she is already awake if they are smart). We thankfully have a sleep room tonight so I am finishing this up to try to get at least 2 solid hours of sleep. The exhaustion has truly set in tonight to the point of feeling sick but it doesn't matter. I will go days on end without sleep if she needs me there. Luckily I think we have had great nurses so far so I trust they will take good are of her and won't hesitate to call me back if she needs me- so that's the only reason she is out of my sight for now. I just want to say how touched we have been by the outpouring of love and support from friends, coworkers, family, and complete strangers. We are truly blessed and feel that God continues to bless us every step of the way with not only one amazing little girl but a great amount of love from so many people!

Night before the Fontan

Today was a long day. We had pre-op appointments starting at 9 am that thankfully we were done with by 1:30. As far as pre-ops go, I don't think our timing was too bad. The only sour spot of the day was the crummy phlebotomist (who had previously been fired from ever taking her vital signs due to poor technique and attitude) that took 3 sticks (and a piss poor attitude) to get her labs drawn. Otherwise, she laid still like a big girl for her echo for the first time since she was a baby! She only got a little freaked a few times but Toy Story was on so she was able to be quickly distracted. Our meetings with Dr. B (surgeon) and K (cardiology) went great and they are happy with the shape she is in prior to surgery. Dr B. says that hopefully he will be in and out and she will fly through it the same as the last 2. We are psyched that he is going to put a Broviac line in while in surgery at our request. Let me explain why this excites us. For her previous surgeries, Addy (and most pts) went to the floor with their RA (right atrium) central line. The protocol has now changed and now they pull this line and then put a whole new PICC line in these kids when it comes time to go to the floor. Well, with Addy's history of making it out of the ICU in record time, we weren't psyched about another invasive procedure and risk of infection so soon after surgery. This way, if he puts the Broviac in while in surgery, she can keep it for as long as she needs the IV diuretics and doesn't have to undergo another procedure that she'll have to be traumatized and sedated for. Hopefully if this goes well this could be a new thing that they do for more of the kids. Yay for less sedation and traumatizing procedures!

Grandma has been hard at work the last few days making Addy special hospital gowns and tele-pack pouches for her during her stay. We have our Dora goody bag of new toys to occupy her once she gets to feeling better, and plenty of snacks to keep us awake while we stand guard at her bedside. The kids are staying with my parents tonight, it was pretty tough not to lose it as Hunter (big brother) broke down crying before he left because is worried about her. Maci understands Addy is going to the hospital to get her heart fixed, but not quite the intensity of it. We plan to let them visit after she is extubated, but just don't think that sitting in a hospital for 12 hours and waiting is fair to them. 
 
Addy had her chlorahexadine scrub bath tonight and is all clean and ready for surgery. Tonight I laid my baby girl down in her bed and kissed her for about the thousandth time today. The tears in my eyes and heart threatened to overflow as I wonder if it will be the last time I put her to bed. She smiled at me that gorgeous smile and said "night night Mama" as I closed the door to her room. Two years ago, we didn't know if we would bring our new baby home- and we thought that was hard. Now she is no longer a baby but she walks, talks, blows kisses, gives high fives and knuckles, asks for Dora, sings ABCs, listens to her heart, and about a million other things.....and I'm scared to death that her heart won't restart on that table tomorrow. You know, I think that any other kind of surgery would be easier to take than knowing that a surgeon is going to have to cut open your child's chest and stop their heart....and know that only his skill and Gods hands are what will restart it again and bring our child back to us just as she is. I know all of the heart parents out there would agree that as they grow and develop their personality it becomes oh so much even harder to hand them over. I'm afraid that this may be the first time that she will cry as they take her away from us. I'm not sure yet if she will need happy medicine yet, but I'm not opposed to it if her mood appears to be clingy in the morning. Since I know that at her age she eventually won't remember going to surgery, I think the happy medicine is really more to prevent the trauma of having to watch your child cry and be taken from your arms. After 2 years, I always think I'm so used to this that it doesn't even phase me anymore. But really, every single day it scares me to death. Please pray for our family tonight as we prepare our warrior for her last big battle.

We have just 10 days until Addy's final (planned) surgery. How time has flown! It seems like we were just celebrating her 1st birthday, and she just turned 2 last weekend! Our family has been so busy, it seems like every time we blink another month goes by! Hunter and Maci have started school back and are finally back in the routine of things. Heath graduated last month from his Nurse Practitioner program, a great accomplishment and relief to us all! Now it is my turn, as I have started the same program to complete in a year. Most people think that we're crazy but we're optimistic that if we've done it once, the second time should be a piece of cake!

Heath and the kids at his pinning ceremony

Heath and his Mom

Me and Heath- Addy is bored

Nana and the kids at pinning

Addy turned 2 this past weekend! We just had a small cake and party at the house with just us to celebrate. We were unsure of so many things when Addy was born, each birthday seems like a huge milestone that I am so thankful we get to celebrate. Although it is slightly overshadowed by the worry we have for the upcoming weeks, it helps to truly be thankful and recognize how special each birthday is. 

Grandma and Grandpa bought Addy a new wagon for our hospital visit! We are very excited to have something to haul all our stuff around with!

Addy says "I could get used to this!"

Happy Birthday to Addy!

Blowing out the candles on her Dora cake with Daddy!

Yay, she did it!

Mmm, Addy loves cake!

Addy's surgery is planned for September 5th. It has snuck up on us, but in many ways we are just ready to be on the other side of this surgery. I am already trying to prepare the things that we will need to make the hospitalization with a 2 year old easier. I don't get paid time off as an unbenefited employee, and Heath doesn't have much accrued- so we anticipate just working around Addy being in the hospital as much as we can. My parents will be a huge help as always, and Heath's mom will come help us out for a few days as well. We will do as we always have- the best we can with what we have! This past week everyone except Heath has had a nice little summer cold, Addy included. This worries us because as most heart parents know, we have planned our life around this surgery and can't afford for it to be postponed! We made a visit to the pediatrician on Thursday and went ahead and had her put on steroids so that she could improve faster. We are confident that by another week and a half she will be all better and ready for the fight of her life! Otherwise, Addy is doing great! She is talking up a storm, and the things she says can really surprise you sometimes! We are anxious to get past this step to see if her activity tolerance increases. It is anticipated that if she continues having breathing problems after this surgery she will probably need her adenoids taken out. Fun, Fun.... Well, I guess that's all of the updates for now. Hopefully the 12 people who read this blog will keep us in their prayers in the upcoming weeks! I will try to keep updating during her surgery for anyone who follows!

Addy never smiles for the camera, so of course when I finally catch her in a smile she has a mouth full of Cheetoes! A true "Cheese" moment!

She couldn't possibly go up the stairs, the slide side is much more fun! This is her favorite toy right now!

Addy's driving her boat! "Choo Choo" she says!

Results of our big day of testing!

We arrived home about an hour ago and Addy is almost back to herself. She did great through the procedures and is now appropriately crabby from anesthesia and overstimulation. The medical team was on their A-game today and everyone was great! I like to think that every patient receives such great care and has such great attention paid to any detail, but whether that is true or it is because they all know Heath and I- I'm glad they do! She is a little hoarse from the bronchoscopy but they have given her some steroids to help with the swelling of her airway. The good news we received today is that the pulmonologist said Addy's lungs look GREAT! This is a big relief, this means he saw no hypoplasia or compression of the lungs, and no signs of institial lung disease apart from expected chronic changes because of her cardiac disease! They did see a LOT of edema and redness in her airway, some subglottic swelling, and adenoids that were nearly occluding her nasal airways. We have an impedence probe on Monday to decide if reflux is one of the perpetrators in these problems, although I am not convinced that it is. We are also starting Flonase to try to shrink her adenoids and reduce some nasopharynx swelling. The thinking is that her adenoids/allergy problems are causing more mouth breathing and decreased airway clearance --> hence hyperventilation -->  leading to air trapping/hyperinflation of her lungs and desaturations. We are THRILLED that her lungs look great as we feel that her airway issues are very manageable and less likely to cause major complications at the time of her Fontan. While we were there today, her cardiologist decided to go ahead and sneak in an appointment for an update as well. She is overall thrilled from a cardiac standpoint of how Addy is doing. They did a cardiac reconstruction using the contrasted CT today to take a look at her heart, but unfortunately they weren't done with the reconstruction yet. We hope to have those results by Wednesday but we are not expecting any surprises. Dr Kavanaugh has put in the request to schedule her final surgery, and we are expecting it to be the first or second week of September. We are trying to work around several things such as Heath graduating with his Pediatric NP degree and taking boards in August, and me (hopefully) starting the program in August. She was 22.6 pounds today, although she feels much heavier when we are carrying her around! Hopefully next week will provide a new set of information as we work to slowly piece together the mystery of Addy's breathing difficulty. She wouldn't be mine and Heath's child if she did everything as expected, but that is what makes her so special! It doesn't seem like it has been so long that we should be discussing when her final surgery will be! She is growing up so fast and is rapidly becoming a little girl instead of a baby! We were so proud of how well she did today, she didn't cry or freak out one time while people were doing the necessary things such as blood pressures, EKGs, and assessments! Here are a few pictures of the last day or so. I will try to update as we find out more information. We would like to thank everyone for the prayers and support!

 The night before any procedure or surgery, I always feel the need to take a few new pictures of Addy girl. I guess it's because you really never know what tomorrow will bring and I want to remember every day I can along the way. Here she is playing in the "baff", which is a favorite activity of hers!

Ha-Ha-Ha, this is great fun!

"Where's Addy?! I can't see you!" She loves to play peek-a-boo!

On the way to the hospital this morning she was subdued, she takes after her mommy and is not a fan of being woken up at 5 am! I did manage however to get one of those beautiful smiles out of her though! Of course she has her special blanket and "Bearie Manilow" for the day, our essentials for any hospital visit!

Love that smile!

 As a reward, Daddy had to make a trip to the hospital gift shop for a surprise! Here she is playing with her new Elmo and Grover puppets and her cow! Every time she goes into the hospital we get her a stuffed animal for being such a brave warrior. I think by the time she is 5 we will have every animal on the planet but she is worth it! She is so brave and special, we would buy her the world if we could for what she goes through!

Kiss Kiss Elmo & Grover!

Aww, she loves her Sesame Street friends!

A Day To Remember

Today marks the 2 year anniversary of when our families whole world changed. At the time, it felt like the worst thing to ever happen in my life. During a routine ultrasound when we were 24 weeks pregnant, the doctors discovered Addy's heart defect. I don't think I will ever forget the feelings that I had in those next few moments and days. I wrote in a journal to help me cope with what we were going through. Looking back it seems like it was so long ago, and I realize just how blessed I am.These are some of the words I wrote that day....

"When the ultrasound was over, she asked us about screenings we had chosen or if I had any history of  diabetes or hypothyroidism. Immediately, Heath and I knew something wasn’t right, that was a big red flag. When she left out of the room to show the pictures to the doctor, we had a sick feeling, we knew something was wrong.... When Dr Boehm came in, he sat down in front of us. That’s never a good sign. He said the words “We think your baby has a complex heart defect”.....those are the words I will never forget, or the feeling of suffocation that followed immediately after....he didn’t just say those words. I looked at Heath and he had the same look on his face. He said “it looks like a corrected transposition…and a double outlet right ventricle…” and then he said the one thing in the world that we dreaded most. He said “… and it looks like there is a small hypoplastic left ventricle”.....and that was what would start our journey that would lead us to where we are today"

 The next day we had her diagnosis confirmed by fetal echo with Dr Soslow and Kavanaugh. It would turn out that Addy had a hypoplastic right ventricle, double inlet left ventricle, tricuspid atresia, corrected transposition, and a small aorta/outflow obstruction. Most of the rest of the story is history but has changed our entire lives. Remembering those initial weeks after diagnosis, Heath and I were a wreck. Our knowledge as pediatric cardiac nurses was damning. I will admit that we hesitated to buy much of anything before she was born because of our overwhelming fear that we wouldn't be bringing her home. As a nurse, you often don't remember the happy endings. You remember the sad outcomes of precious children that fight for their lives for months and months before their bodies can finally take no more. That is what we anticipated for the remaining 16 weeks of pregnancy. Thus far, our course has been far better than expected, although not without its own bumps along the way.   




Today, I am grateful for every single day that we are blessed to have Addy. She is truly an exceptional child, and I'm not just saying that because she's mine! She is 20 months old and not quite 23 pounds. She's petite, but unless you looked closely, you would never know she has a special heart. She is so smart, her vocabulary matches that of most 3 year-olds I have seen. She is stubborn and independent, which I think will only help her in her lifelong journey. She has a smile that melts my heart, and is an incredibly happy child. There is nothing that she can't do (besides walk very far) and she makes every effort to do things just like her big sister Maci. Her favorite activities are playing with her pet "chicks" and all the other farm animals. I do not take a single day for granted, and give her as many kisses and hugs as possible. We are anticipating her final Fontan surgery coming up in August, pending we are able to identify what her pulmonary issues are that keep causing her problems. My heart rate rises every time I think about August. A part of me (and Heath) has not quite fully accepted that we are going to be blessed to have Addy for a long time. I hope that after the Fontan the feeling of waiting for the other shoe to drop will subside and we will just be able to watch her grow into a little girl. Once again, our nursing knowledge is a curse and a blessing. I never imagined 2 years ago that we would be where we are today. I could not have asked for more. 

She loves her Cookie!

Add caption

Such a cutie!

 

 

How Time Flies!

I absolutely did not realize that it had been so long since I had updated Addy's blog. I guess that we have just been so busy with enjoying every minute with our sweet girl that almost 6 months has flown by! Things here at home have been pretty good except for the loss of our Great Pyrenees, Hank this week. He loved cars, and that ended sadly but now he is buried up on the hill and will always be part of the farm. I was in a car accident a few weeks ago where my car was totaled, but thanks to seat belts had no major injuries. Maci is now wearing glasses and working her way through the rigorous Kindergarden curriculum much better! Hunter is doing well and just got a clean bill of health from his yearly St. Jude checkup. Heath is currently in clinicals for school so between that and work he is occupied pretty much 6 days a week. I'm still working 2 jobs as well and doing my school online as well. All of our farm animals otherwise are doing well, all the animals are ready for spring to come along with green grass!

Let's see, Addy is doing amazing in terms of normal baby stuff. She runs everywhere she goes, until she gets too out of breath. She has an exceptional vocabulary for 17 months if I do say so! Her favorite activity is watching Elmo on Mommy's Kindle Fire! She is saying 2 word phrases now and lets you know exactly what she wants! She had a sedated echo in January that looked completely perfect except for that pesky Left Pulmonary Artery that is still 3 mm. However, the vessel past that small part is growing nicely so that means that there is still good blood flow and it should make it until the Fontan. Dr Kavanaugh said that Addy's hemodynamics (the pressures and measurements of her heart and vessels) are perfect and probably the best she has EVER seen, and her ejection fraction (function) is nearly normal for even a whole heart! Our only concern is that she continues to have a large amount of activity intolerance. It is true that she should get a little more tired than normal children, but in absence of any lung problems, she shouldn't get "out of breath". Even our cardiologist admits that while playing and walking around the room, her work of breathing is significantly harder than most. She has absolutely no explanation for why Addy is absolutely huffing and puffing after approximately 20ft of walking. We have already had an airway workup that came out clear so our next step is a pulmonary consult to see if there is problem with her lungs. This will be this Thursday and hopefully give us some additional answers. While we don't want something to be wrong, if there is, we want to know about it before the Fontan when she is intubated and we completely change her pulmonary flow and pressures. Otherwise, the plan is to do another sedated MRI (pentobarbitol for the sedated Echo in January was a NIGHTMAERE, so we are doing MRI instead with ketamine/propofol) in April or May and if at all possible she will have her Fontan around August when she turns 2. She is still growing slow and steady, not as fast as I would like, but she is still hanging between the 25-50% percentile on the growth chart. The good thing is that she absolutely loves food, so she gets to eat whatever she wants as long as it has lots of calories! Luckily, she also loves her fruits and vegetables, and eats those way better than Maci does! On a sour note she will be losing her Tn Care this month, so all of the extra bills that our insurance doesn't cover will now fall back on us, I imagine that we will be in medical debt the rest of our lives so I am working on coming to peace with how the whole government system works and who qualifies for assistance. I think those are the highlights for right now. I am posting some new pictures of her so everyone can see how much she has grown. She has turned into a toddler right under our noses!

Addy loves watching all the animals from the front porch! She knows all their names- goats, sheet, geese, and chickens!

Just a normal day of mischief for Addy! Looks like she's getting ready to swing from the pig's leash!

"Oh Elmo, how I love thee!" She loved this balloon so much I couldn't bear to throw it away so we just hung it up!

Fun times playing in her room!

Addy's first Christmas at home, her first ever was spent at the hospital!...does anyone see an Elmo theme yet?

Addy & "E-la-la" (Eli), she thinks it is her most important job to feed him his bottle!

Playing outside on the rocks!

Checking Peggy's gums for gingivitis! Peggy is as gentle as a lamb, lets her do whatever she wants to her!

OK mom, I'm done walking, come get me!

This little table set is just her size! She was calling "keeety" (kitty) over to sit with her!

Inside chilling with her Boost drink, Cookie, & Rooty! She looks like she runs the show!

These were just a few of the highlights over the last few months. I will try to keep up better with the postings, especially as we get closer to our final surgery!