Twenty-Six Days Post-Op from PA Banding

*For anyone who needs a recap, our little Sunley Summit has Hypoplastic Right Heart Syndrome (HRHS). Specifically, she has Double Inlet Left Ventricle (DILV) with an extremely small (almost non-existent) right ventricle and large VSD. Both the Aorta and the Pulmonary Artery are well developed and going into the left ventricle (as far as we can tell right now).  We are getting care at Texas Children’s Hospital in Houston, TX, whose heart center is ranked number one in the nation. There is no real cure for Sunley’s condition, so on June 11, 2018 she had a  PA Banding surgery, and will later receive a Glenn, and Fontan (a total of 3 heart surgeries). Our hope is that these surgeries will work well enough to avoid a heart transplant and she will lead a long, full life with only those 3 surgeries.

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It's hard to believe that the photo above was only a few weeks ago. Sunley has gained about 12 ounces in just four days!! We are so proud of her! The last few days have been spent tweaking her meds to find just the right balance. We THINK we have found it now and so…drumroll…they are sending us home TODAY! She weighs a whopping 8 pounds 1.6 ounces and is going home with only 18 days left of sternal precautions. She eats around 3 ounces every 2-3 hours, so we are about to be very exhausted at home, but for happy reasons :) We worked with OT here at the hospital, and we feed her in very specific ways, so right now only Derek and I can give her a bottle, since we’ve been trained in it.

It’s been an emotional 24 hours, as we are officially “through” the first surgery. It didn’t go perfectly, but we are happy to be going home within a month of her surgery date (June 11). We are being sent home with more meds and slightly lower saturations than we hoped for, so I hope the next surgery gets us closer to our goals. Her lungs have still not improved since surgery, and so we are desperately hoping for positive changes there over the next months. She is going home on doses of Lasix, Diuryl, Enalipril, Zantac, Nexium, and Simethicone. The Simethicone will probably be stopped soon, and the rest will continue to be tweaked as she grows. All of these are oral and not injections, which is good. I downloaded an app to give me daily reminders for her meds, as well as track my pumping with her eating, to make sure nothing gets overlooked. They are also sending us home with a pulse ox for spot checks, but not constant monitoring. They weren’t going to send us home with one, but I pretty much insisted, just for my peace of mind.

The doctors told us to treat her like a newborn as far as outings go. So, we’ll be homebound for just a little while, but should be able to resume normal outings in just a couple weeks, within reason. I can go to Target as long as she’s covered, for example. (I specifically asked about Target, because PRIORITIES.)

Her bradycardia has been brief enough that it isn’t a concern, and her echo yesterday didn’t show anything new — Not that it didn’t show anything concerning, just nothing NEW. We all feel confident that she will not have a cardiac emergency between now and the Glenn, but her main risk is catching a cold or a stomach bug, or even a fever. The doctors also told us to limit exposure to non-vaccinated kids. I am 100% NOT asking for your input with this one. We have so many friends who we love dearly that don’t vaccinate their kids, so we were sad to hear this, but we agree with our doctors. It doesn’t mean we can’t be friends, and we absolutely still want to see you and spend time with you, but it does mean we probably can’t go camping together. And honestly, how are we supposed to know which kids at Chick Fil A or at a church meeting are vaccinated and which ones aren’t? So if you are in the no-vax club, maybe just discourage your kiddos from holding or kissing on our little ones. I know that if you aren’t vaccinating, then you obviously think this is a stupid rule for us. I get that not vaccinating is an option for a lot of people, but it is no longer an option for any of us, or our extended family. A runny nose could quickly end our time with Sunley, so not only do we have to ask non-vaxxers to use caution, but anyone who is even just suffering from a minor cold or cough. I HATE how much I have to care about germs now. I hate it A LOT. I’ve always been a “rub some dirt on it, build your immune system” mom, but I don’t get to be that anymore, and I need your support and understanding. Most of all, I need you to stay away if you are sick :) There is definitely a balance of keeping away from germs while still getting out and LIVING life, but our balance is permanently different than it was before. I’m really sad about that, so please don’t pick on me for having to change our rules.

Sunley will have weekly check-ups as she did before, and we are hoping to put off her Glenn procedure until the end of October/early November. I’m not sure she’ll make it that long, but that’s what we are hoping. Please keep us all in your prayers! Pray for her lungs to improve, and for her to continue to gain weight!

Sunley's favorite thing is watching her monitors every day. If she's upset, we just turn the monitor to face her, and she usually calms down. Our nurse a couple days ago decided that this means she'll be a doctor when she grows up, so she got her a scrub cap and it was pretty much the CUTEST.

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Twenty-Two Days Post-Op from PA Banding Surgery

*For anyone who needs a recap, our little Sunley Summit has Hypoplastic Right Heart Syndrome (HRHS). Specifically, she has Double Inlet Left Ventricle (DILV) with an extremely small (almost non-existent) right ventricle and large VSD. Both the Aorta and the Pulmonary Artery are well developed and going into the left ventricle (as far as we can tell right now).  We are getting care at Texas Children’s Hospital in Houston, TX, whose heart center is ranked number one in the nation. There is no real cure for Sunley’s condition, so on June 11, 2018 she had a  PA Banding surgery, and will later receive a Glenn, and Fontan (a total of 3 heart surgeries). Our hope is that these surgeries will work well enough to avoid a heart transplant and she will lead a long, full life with only those 3 surgeries.

I really wish I could do a better job of keeping this updated, but the thing is, there can be several days in a row where we are just experimenting and seeing what her body does, so I have a hard time trying to explain that.

Since the last update, so many things have changed. First of all, Sunley is eating great! Feeding tube came out today!! Her echo didn’t show anything to explain her lack of weight gain. It did show some possible future issues with her mitral valve. There is a “ridge” near it (could cause some blockage), but also maybe a hole that could resolve the possible blockage issue. It isn’t causing any problems, just something to watch in future. Last Friday, we backed off of her diuretics a little bit more — completely came off of diuryl and just kept with lasix. Weaning off of diuretics ALWAYS causes some weight gain, but on Saturday morning, after eating well on Friday, she had a huge dip in her weight. I asked the nurse to check and make sure that they had used the same scale as the day before. When she asked the charge nurse about it, she was told that there was no way to know or track what scales they were using. In other words, we couldn’t trust ANY of the weight measurements we’d had that week. Also, even though I wrote down every feed and weighed every diaper, the nurse from the day before hadn’t recorded any of it. That was the 2nd time that had happened. So we officially made the call to get us back to a cardiac floor/pod. We were moved Sunday to EPod, which is in NICU2 specifically for heart babies. She has her own designated scale here, and the nurses have been WAY more meticulous than on the Neuro floor. I did let the cardiologists and Nurse Practitioner know that while all the nurses were very kind and helpful on floor 10, that is NOT the place for cardio babies in the future.They all agreed — in fact, as I mentioned before, most of our doctors didn’t want us going to 10—and now we are in much better hands. 

Well, when we got to EPod on Sunday, Sunley was experiencing very low sats, about 70-72. I wasn’t concerned at all, because I see those numbers often from her. The NP though was not ok with anything less than 75, and very strongly recommended putting her on 2L high flow room air to see if it would help. I agreed and immediately regretted it because Sunley HATED the cannula and was also very constipated (we had switched to 27 calorie fortifier that day). I went home that night knowing she was in for a long night, and that was extremely difficult. Before I left, the NP told me that if her sats continued to be low, they would consider putting in an IV to hydrate her or something (I can’t really remember the details of why), and I said absolutely not until I get back in the morning to discuss it. My head was spinning, because I thought we were just coming down to eat and take off wires, and instead Sunley had tape on her face again, with a cannula and way more monitoring. The NP was very patient with me that night and the next morning, explaining everything. They did not end up needing an IV, and decided that she must have been a little dehydrated from all the diuretics she’d been on. 

Luckily (more like a God-thing), our very favorite cardiologist, Dr Ayres, was the attending cardiologist for the first time since we’ve been here. She is Sunley’s official cardiologist, and also diagnosed her prenatally. I could do an entire blog post about how many times we’ve heard the doctors here RAVE about her — she is just short of worshipped at TCH, and for good reason. Anyway, when we saw her on Monday morning, she completely changed the “plan” for Sunley — took her off of all diuretics and put her on a blood pressure med (I’ll have to look up the name again) due to some squeeze/relax issues she noticed with Sunley’s single ventricle in the aforementioned echo. I have some more questions about this drug, but it does look like she will need this for the rest of her life. I haven’t totally accepted that yet, but I’ll get there :)

We removed the nasal cannula sometime on Monday, and she also had 3 huge bowel movements around the same time. She was much more comfortable after that. We changed a bunch of things happening with her on Monday, but NOW she is on 24kcal Similac Total Comfort fortifying her breastmilk, eating every 2-3 hours 65ml and up (sometimes even 80ml). She is on Zantac, her blood pressure med, and will later today probably get back on her Lasix, depending on the results from her Xray today. There is no talk of us going home yet, but I do feel like it could happen relatively soon. I have mixed feelings about that again. I was so ready to go home until we started changing everything, and now I’m nervous again.

Last night (Monday night), she did go bradycardic for just a couple seconds while she was crying. I was so shocked to hear this, because that hasn’t happened since the couple days after surgery. Actually, just as I typed that, it happened again for about 10 seconds. Hmm. I was also surprised to hear about the squeeze/relax issues with her ventricle, because I thought we were past that. Her sats now are running around 80 which is great. But I wonder how long that will last, just based on her patterns in the past. 

I also miss my big kids. I’m seeing them a lot more lately which is great, but I sure am missing a lot. I know it’s so temporary and short in the grand scheme of things, but it’s most evident with Ruger — he’s just growing so fast, and I hardly recognize him when I rock him at night. I think to myself, Who is this big kid in my arms?? He recently had a RIDICULOUS growth spurt (mostly in head circumference, unfortunately for him) and I just can’t believe how big he is. Two years old was probably my favorite age with Hadelyn, and I’m missing so much of it with my big boy. Right now, it’s looking like Sunley’s surgery will be right around the time Hadelyn starts Kindergarten. The public school that we were comfortable with here is full (I didn’t know public schools could be full), and I know that I will not be able to homeschool her while Sunley recovers from surgery, so we are very seriously considering sending the kids back to Midland when that happens. And that will be hard. No final decisions on that yet.

Here are some favorite photos from the past couple weeks. Some of them are "outdated" -- she doesn't have the head sticker or arm IV anymore. The most recent is the one with Hadelyn <3

Sixteen Days Post-Op from PA Banding Surgery

*For anyone who needs a recap, our little Sunley Summit has Hypoplastic Right Heart Syndrome (HRHS). Specifically, she has Double Inlet Left Ventricle (DILV) with an extremely small (almost non-existent) right ventricle and large VSD. Both the Aorta and the Pulmonary Artery are well developed and going into the left ventricle (as far as we can tell right now).  We are getting care at Texas Children’s Hospital in Houston, TX, whose heart center is ranked number one in the nation. There is no real cure for Sunley’s condition, so on June 11, 2018 she had a  PA Banding surgery, and will later receive a Glenn, and Fontan (a total of 3 heart surgeries). Our hope is that these surgeries will work well enough to avoid a heart transplant and she will lead a long, full life with only those 3 surgeries.

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(Photo was taken at home before surgery)

Every day, the cardiology team rounds on each patient. They state their name, age, congenital heart condition, procedures that have been done, meds, etc. Sunley’s rounds usually start with “This is Sunley Nichols, and she’s pretty confusing.” While I’m all for my kids not taking the beaten path, I’m pretty ready for Sunley’s body to start acting as it should. Yesterday morning, she had lost another ounce even though we are feeding every 3 hours around the clock with fortified breast milk at 24kcal/oz (She doesn’t tolerate above 24 very well). After observing one of her feeds, the cardiology team and I decided that she needs an NG tube for now (a feeding tube inserted through the nose). We can still continue bottle feeds, but now whatever is left in the bottle at the end of a feeding goes in the tube. So she is still using her sucking skills, still having to work for her food, but when she is just DONE we quit and put the rest in ourselves. Our hope is that this will allow her to get some weight gain, and hopefully as she gains weight she will have more energy to eat via bottle. The cardiologists are hoping that the NG tube will be brief. But I’m preparing myself for a long haul with it because nothing is going as planned.

Because she has no cardiac drugs going, and really isn’t at a huge risk right now with her heart, they moved us from the cardiac floor to the neurology floor, because they have more open beds. I’m totally fine with this, because the cardiac ICU floors are always completely full, so us moving means that they have more room for more surgeries. With that said, transitioning to a new staff, slightly different equipment, and just an overall new routine is ROUGH. The nurses are not cardiac nurses, and I found myself having to RUN THE SHOW and repeat a whole lot. I’m ok with it, but it does make me just a little irritated. I’m so happy that my kid is the one who doesn’t require cardiac nurses but I’m tired of her getting the nurses that haven’t been around long or, now, don’t have experience with heart patients. I’ve definitely had to advocate for her more on this floor and that’s pretty exhausting. After we left, there was some big to-do about it and one cardiologist actually came down and gave me the option to go back to floor 15. So, I really can’t complain because I did have the option to go back. But I told him that as long as she will get the attention she needs then I can deal with having to be a little more on top of things. And we’ve seen our doctors 3 times today already, so I know she’s not being “forgotten” down on 10. If you feel like I should’ve done differently, kindly keep that opinion to yourself please ;) We’re exhausted but doing just fine.

Right now, our only goal is to get some weight on her so she can be big and strong enough for her Glenn procedure. Her lungs are still just as “wet” as they were right after surgery, but I’m told that it is not atypical for lungs to take a few months to heal, since we waited so long for her first surgery (we waited 6 weeks for the PA Banding Surgery). It was definitely a good decision to wait — she was bigger, older, stronger, and I know that certain parts of her recovery have gone so smoothly because of how long we waited. But, like every cardiac care decision, there are pros and cons with every option, and the con is that her lungs have been overworking for a very long time and now they are tired and have fluid and need time to heal. Her respirations look great, which has the team a little confused. The characteristics of her eating habits SHOULD mean that she has fast respirations, and that’s just not the case. So, today we did an Echo and tomorrow we will have an Xray. She is on a couple of diuretics, plus potassium and salt, plus a medication to help retain salt, and also on 2 antacid meds for reflux. They will also be running some blood tests. We are all just trying to figure out why this girl isn’t hungry enough to put some weight on! She is currently about 7 lbs 7 oz. So she’s gained only 6 ounces in 2 months of life.

I’m really not sure what to pray for. Do I pray that they don’t find anything wrong in the echo, or should I just pray that they find what’s keeping her so tiny?I’m not sure what’s gonna happen, but I’m very grateful for our team of doctors here. Oh, and by the way, if you read my earlier blog posts then you know that we almost had Dr. Mery as our surgeon, but then ended up with Dr. Heinle. I was emotional over the switch (I wanted Heinle originally but then just really got along with Dr Mery, and had a hard time with all the transition), just because the switching can be really stressful. Well yesterday Dr Mery announced that he is leaving to go to Austin, where Dr. Fraser went. So, there’s yet another answered prayer that didn’t feel like answered prayer at the time. If we’d had Mery, I’d be a WRECK right now knowing that we needed a new surgeon for her next surgeries. So I’m trusting that I’ll look back and see some answered prayers happening this week that don’t feel like blessings right now. Keep the prayers coming!

On a happier note, Sunley smiles all the time now, and has started reaching for toys and even trying to roll over! I mean, I can’t LET her roll over because of sternal precautions, but I’m still super proud :) We can't wait to have her HOME again! Here's a video from the night before her surgery. I love the innocent way Hadelyn calmed her own nerves while caring for Sunley <3 My babies are all pretty amazing. Ruger's amazing too, but he rarely participates once he sees me taking a video.

Eleven Days Post-Op from PA Band

*For anyone who needs a recap, our little Sunley Summit has Hypoplastic Right Heart Syndrome (HRHS). Specifically, she has Double Inlet Left Ventricle (DILV) with an extremely small (almost non-existent) right ventricle and large VSD. Both the Aorta and the Pulmonary Artery are well developed and going into the left ventricle (as far as we can tell right now).  We are getting care at Texas Children’s Hospital in Houston, TX, whose heart center is ranked number one in the nation. There is no real cure for Sunley’s condition, so on June 11, 2018 she had a  PA Banding surgery, and will later receive a Glenn, and Fontan (a total of 3 heart surgeries). Our hope is that these surgeries will work well enough to avoid a heart transplant and she will lead a long, full life with only those 3 surgeries.

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 Since my last post, Sunley has moved to the CVICU on floor 15! Basically what that means for her is that she has officially survived her surgery, and now we are waiting to see just how well the surgery worked. Her nasal cannula and feeding tube are both GONE! She has been eating and taking meds by mouth. She has an IV in her arm, but it isn’t currently being used. So besides the leads on her torso and pulse ox on her foot, she is wire free, and we can see her WHOLE face. It’s so wonderful to see. She is eating like a champ, and for the last 2 days the doctors have let her eat as much as she asks for without limits.

Yesterday morning, Sunley had a chest Xray that was a little concerning. It showed too much blood flow to her lungs. She also has had a few times where her respirations have been too fast, and she has also had some very low saturations which have us all a little worried. After much debate, the cardiology team and we decided to let her continue eating as she was through today, and give her body a chance to resolve the issue itself. We should know today sometime with an X-ray if that is working. If not, we will go back on some more diuretics that we had weaned off. If that doesn’t work, we may be in a pickle — Usually, the next step would be to restrict feeds. But Sunley is SO behind on her weight, and weight gain is a major priority for her to even be a candidate for the Glenn procedure (which she needs to survive). Not sure what we would do if the diuretics didn’t work, because you also don’t want to stress out her lungs so much that THEY disqualify her for the Glenn. But hopefully we won’t get there.

I can’t believe it’s only been 11 days since her surgery. There are lots of people who have been in the CVICU for months and months, so I can’t complain —  I can’t wait to take her home, but I’m also very, VERY nervous about it. It’s so hard to know that we will soon be coming back to do it all over again. And next time, she has a much more invasive surgery planned. Our hope is that her lungs continue to heal, and we can wait several months before the Glenn. Being an older Glenn patient slightly reduces some very scary risks. The doctors have told us that they hope to send us home sometime next week with no monitoring equipment and no medication. I’m not sure we will get to do the no medication part, but it all sounds encouraging! No monitors sounds a bit scary to me, but I won’t miss all the alarms throughout the night. I’m pretty sick of waking up my baby while attempting to reattach her pulse ox! Right now, the only monitors she has are heart rate/rhythm, oxygen saturation, and blood pressure, and the only one thats raised concerns is her oxygen saturation, which randomly dips down form time to time. No one is really sure why, and unfortunately the extra blood flow to her lungs could mean that it will lower again. Right now, she ranges from the 60s to the low 80s — most of the time, she’s around 74. Pre-surgery, doctors said they hoped for mid 80s, then last week, they said she needed to be 75 minimum, and now they’ve said that as long as she’s in the 70s, it’s ok. That’s very hard for me to get on board with, because I’m still not over that we didn’t get to our mid 80s goal. But the fact is, we can’t change it. The surgeon technically could go back in and readjust her PA band, but the risk far outweighs the benefit in this case. Right now, it’s looking like she could be on the early side of receiving her Glenn, but we are all hoping her body decides to readjust some more and find a more stable normal.

Derek and I have been switching off evenings at home with the kids, and my Aunt Missy has kept them during the day (which makes her a SAINT). We try very hard to compartmentalize — when we are at the hospital, our whole focus is on Sunley, and at home we try not to talk about the hospital stuff much and just focus on our big 2 kiddos. But of course, it’s very draining and obviously pretty hard on all 3 of the kids. It’s possible that we alone are responsible for Uber’s current success —we use it multiple times a day because parking here is just very expensive. We’ve gotten LOTS of food from ladies at church and those have fed my kids and aunt for a LOT of meals, and me too when I’ve been home. We are daily blown away by the amount of prayers going up for Sunley. If you’re not already, please consider officially becoming an organ donor — there are SO many babies and older kids here waiting for transplants, and it just may be Sunley someday. It’s so hard to see knowing that so many decent organs end up being buried because someone just didn’t get around to making their organ donation wishes official. And in case I’m sounding too preachy, I didn’t get around to it either until I was told my daughter MIGHT need some new organs someday (Yes, unfortunately that’s plural — but hopefully will never happen).

As much as I miss home and our old routine, the hospital is actually pretty great. The rooms are pretty comfortable, Ronald McDonald has rooms for when you don’t have a sleep-in room, and most of all, you’re in this bubble of families all going through hard stuff. There aren’t any judgmental looks toward bald-headed little girls or moms crying on the elevator. Getting around in a wheelchair or lugging around an oxygen tank is perfectly normal. All of us had that gut-wrenching diagnosis moment — we all seem to have a phrase from that day that will always haunt us — but we all survived. It’s kind of a nice support group of people whose lives are on pause. Of course we all have the goal of LEAVING, but honestly it’s going to be very hard to leave every time she is discharged. We've also made some pretty strong bonds with some other families here -- one in particular -- and it will be very hard not seeing them every day when the time comes. I won't go into all of their details (because I did not ask permission to share any of it), but we have been so blessed to meet them, and I have no doubt we will be friends forever. We've already gotten our older kids together, and it's been so SO wonderful to be able to spend time together and laugh so hard that we forget for a minute where we are and why we're here.

I’ll post an update to this post below when we get the results back from her X-ray today. Pray for good news!

UPDATE: X-ray showed that she is still continuing to have too much fluid in her lungs. She is now maxed out on diuretics and still allowed to eat as much as she wants. We are praying the drugs do their job, and she continues to gain weight!