*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 the plan is to do a total of 3 heart surgeries (PA Band, Glenn/DKS, and Fontan). 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. Sunley spent her first 6 days in the NICU, went home for a while, and then spent 26 days in the hospital after her first surgery (PA Banding) at 6 weeks old. She had a one week hospitalization for dehydration at 4 months old, then went home for a month. At 5 months old she was hospitalized for low saturations for 5 days, and underwent what I’ve officially termed a “doing-something cath” (atrial septostomy). On November 6th, Sunley had her second open heart surgery: a Bi-Directional Glenn and DKS procedure. We are still inpatient and recovering from that.
First of all, PRAISE THE LORD. I am constantly just humbled and have been literally brought to my knees thinking of all the prayers said on the behalf of our little family. I won’t try and thank all of the individuals that have been there for us all year long, but I do have to say thank you a million times to my parents and to my aunt, who is keeping our older two kiddos while we deal with all of this. My aunt is filling in for my parents so they can be here with us, along with Lonnie and Tonya, who came for Sunley’s surgery. The love that believers have for each other and for my sweet baby has been so uplifting and unifying, and I am so in awe of how much love we have received through encouragement and prayer. God is constantly blessing us not only with good physical outcomes for Sunley, but with new encouraging relationships all across the world.
Whew. It’s been quite a whirlwind over here! I’m not really sure how to get all the info in here, so I’ll just give a summary of each day:
Monday, November 5th
We arrived to TCH at 8am to start the very lengthy check-in process. Sunley had to be cleared by several doctors to make sure she didn’t have a runny nose, fever, ear infection, etc. It’s been a long month getting ready for surgery, keeping her almost completely quarantined so she didn’t catch anything that would bump her surgery date. This also means that the kids couldn’t come visit, and that was the hardest part. I finally just HAD to see them, and went to Midland for a quick weekend trip at the end of October. They haven’t been to Houston (haven’t seen Sunley) since September 3rd (Labor Day weekend). We finally got a room on one of the step-up floors, room 2309, and stayed there overnight. We must have had a brand new nurse (since Sunley was obviously the easiest case on the floor), because I had to show her how to do just about everything, including put on a posie over Sunley’s pulse ox. It was no biggie since Sunley really didn’t need any nursing care, but I am hoping we get a more veteran nurse next time we’re there post op. Sunley got A LOT of snuggles from all of us (both sets of grandparents are in town for surgery). Getting ready for an early day tomorrow — Surgery is set for 7:15 am. She will go NPO after 3am, which really isn’t too bad. I requested that we stop fortifying her breastmilk bottles to see if we can gain weight post Glenn without the help of formula. Not sure how well we will sleep tonight, but I’m ready to get this over with!
Tuesday, November 6th
Grandparents came in early for one last snuggle, and we’ve been rocking her ever since. Surgery got delayed just an hour, but that’s kind of a long hour when you have a hungry baby.
When we got back to the holding area, Sunley’s whole team was ready for her, including Dr. Ayres (her cardiologist) who made sure she got put on Sunley’s surgery today, even though she had 3 patients going in. She has made us feel SO confident the whole time we’ve been here. Sunley got some last snuggles and kissies, and we gave her a light sedative to help her zone out, since she’s been starting to feel some stranger danger lately. Watching her drift off made me more relaxed, too.
We LOVE our anesthesia team! Sunley had the same anesthesiologists that she had for her PA band surgery, so it was great to see them again. (Heart kids have anesthesiologists that are specific to cardiac care. Primal Trivedi and Eugene took her back, and Eugene picked her up and put her on his shoulder and said “I’ll take of your baby” as he walked away. He’s everyone’s favorite anesthesia fellow because he’s always so kind and gentle with the babies. 8am: Sunley went back to the OR, and they said to expect an update in about an hour/hour and a half.
Unfortunately, it sounds like Dr Heinle will most likely be doing the most invasive option, the DKS procedure, along with the bidirectional Glenn, but the final decision won't be made until he gets in there to inspect the aorta and pulmonary arteries, and their valves. I'm so grateful for the team of doctors that we have, and I know that they will make the right decision. Surgery will last between 6 and 15 hours. We were previously hoping to get through the Glenn without stopping her heart, but that is no longer an option, as her atrial septum needs additional opening.
10:30 am: Our first update from Deanna - They've started the incision. She has her lines placed and is doing well. The TEE took a while because there is A LOT of debate on which option is the best option for Sunley long-term. Still leaning toward a DKS but Dr Heinle hasn't officially decided yet. Dr. Ayres and Heinle discussed both options for a really long time, but ultimately it’s just a (very) educated guess as to what will be better for her in the long run (which is why it MATTERS to which hospital you take your kids!).
Update #2 at 1pm: Officially on bypass.
Update #3 at 2ishpm:
Her heart has been stopped for a while now. They are officially doing the DKS. We are pretty bummed out that she needed that option, but we are grateful that they saw whatever they needed to make that decision, hopefully avoiding extra surgeries in the future. (She will, of course, still have a Fontan down the road). Her heart will be stopped longer than we wanted, but as soon as the DKS is completed they will restart the heart (she will still be on bypass though) and proceed with the Glenn.
Update #4 at 3 pm:
The DKS is completed (that was fast!) and Dr Heinle is beginning the bidirectional Glenn. The heart is still stopped but will be started again after the atrial septostomy is completed (I think).
Update #5 at 4pm:
Once again, they underpromised and over delivered on the time estimate: Sunley is DONE with her surgery! Her heart is beating, she is off of bypass, and her function looks great. They WILL get to close her chest! They are just about to close. Praise God!!! Now we are very anxiously awaiting the next few days of recovery to see how her heart does with this crazy new physiology.
Last update at 5.15:
She is closed and headed to her room. We should see her within an hour. Of course she is intubated, and hopefully we can extubate soon.
Overall, the surgery time from going under to being closed up was around 8-9 hours. Dr. Heinle came to talk to us after it was done, and when I asked him how long she was on bypass he didn’t even have to think about it and said “She was on bypass for 161 minutes, and had her heart was stopped for 57 minutes.” I also asked him if there were any unexpected issues during surgery, or any delay in getting her heart started again, and he said no. I’ve learned that you have to ask these things, as that information is not always offered —although I think Dr Heinle goes into more depth…or maybe I just ask too many questions ;) We are all extremely happy with the outcome, but with such an INVASIVE procedure, we are expecting a slower recovery than originally thought.
During surgery, our parents sat with us, and we hung out quite a bit with Leslie and Brian (Oliver’s parents). We sat with them during Oliver’s surgery and played board games, so it was great to get to do that all again. We also met another sweet couple who has an adorable little girl HLHS interstage. The friendships we’ve made here have been such incredible blessings!
6.30pm: Mommy starts asking to see Sunley, since there is a history of nurses/doctors forgetting that we are waiting anxiously outside the rooms. Within five minutes of asking, we got to go back to see our girl. We were happy that the surgeons were able to close her chest. She is of course intubated, but all of her vitals look good. I’m shocked that the doctors are discussing possibly extubating her tonight. I’m wondering if that’s a little soon, so we discuss it, and get to a good decision of being open to it, but not pushing the extubation. Derek and I are sleeping in the Ronald McDonald rooms tonight in the hospital to get away from the monitors and busyness of the CVICU. Sunley is in room 1803. (We were hoping to get next door to Oliver on a different floor, but oh well!)
I had a list that kept track of ALL her meds aaaaaand I accidentally deleted it. SO bummed about that. I remember that she came out with one chest tube (glad it was only one, as we were expecting two), an IJ line (Glenn line through her neck to check her new glenn pressures), two femoral art lines, one wrist art line, an IV in her arm, foley Cath, and temp probe. She was on Milrinone, Precedex, Nitrocardipine, Enalapril, Nitroprusside, Nitroglycerine, of course TPN and lipids. There may have been other drugs but I can’t remember them all. I may go in her chart sometime and look art them all.
She still looks pretty swollen in her head, and I’m sure will have some Glenn headaches as her body gets used to this drastically different physiology. But for now, she is sleeping hard and stable. We don’t care about the election.
Sunley had a great day today! She was extubated in the morning to high flow 8L at 100%, and is down now to 75%. She SAT up like a FREAKING SOLDIER less than 24 hours after her heart was stopped and chest open on a table. Everyone is AMAZED by her progress, and a couple of doctors have said that they’ve never seen a DKS/BD Glenn do this well so soon after surgery. I’m excited, but I kind of have that pessimistic feeling like I’m waiting for something to go wrong. Sorry. Can’t help it. Her Glenn pressures had a couple spikes, but none of us think they were accurate reads, and the numbers have been stable otherwise. She also got one of the fem art lines out, and did an oral feed of 30mL! She’s pretty amazing, and the prayers are definitely working! We had one of our favorite nurses today, Marty, and we are so grateful for such a great day!
Sunley stayed stable through the night. Glenn pressures are perfect, so we took out the IJ (the one in her neck). She has more pain today and won’t let herself cough, so she has some gunk building up that may need deep suction later. We ended up giving Sunley some Dilaudid and Hycet today to help her with the pain. This afternoon her blood pressures started going up higher and higher and higher. They got up to about 149/67. The whole time they were climbing, I could tell our nurse was getting nervous, and our attending was busy with an emergency so no one was coming. Also, the BP was NOT due to pain —Sunley was sleeping comfortably, and we tried every limb, a new blood pressure cord, etc. It was a REAL high BP. Momma bear was starting to come out and I finally told our nurse I would wait ten more minutes, then I’m going to find someone (I mean, how many doctors are in this building right?!) I was staying pretty calm until our attending finally came in (understandably flustered from the emergency next door) and called her a “he” (also understandable, there’s lots of babies here), and then started telling me that this is “totally normal, especially because his [HER] heart had a big change since the NORWOOD.” And Sunley didn’t have a Norwood. I got pretty upset because I felt like not only was he not concerned enough, but he clearly wasn’t even thinking about her physiology. He gave her a dose of Hydrolozine to bring her BP down and told me that everything is fine, and her BP will be back to normal range within ten minutes. FORTY minutes later, Sunley was still having super high blood pressure, like 130s systolic, and I was FRUSTRATED. The doctor ordered more Hydrolozine but never came back by…pretty sure he didn’t want to admit he was wrong. I really would have been calm had he taken me seriously, but I think the more he tried to downplay it, the more worked up I became —and the more worked up I was, the more he tried to downplay it. It was not a fun afternoon. We stayed in her room, until her blood pressure got to about 112 systolic consistently (Ideal is under 100). So it is still high, but we feel good about going to bed since she’s not in the danger zone anymore.
I found out later that high blood pressure is actually even more dangerous than I thought for a DKS-er. Such a fresh DKS can leak if the BP gets too high. Really not happy with how the attending handled it, but the important thing is that we are moving in the right direction now.
I woke up knowing that the doctors were going to totally downplay her high BP from yesterday, in hopes of moving us to the step-up floor. Derek ran into one of our favorite doctors when he was getting coffee, and mentioned the high BP and they told him that No, that’s definitely NOT normal and not ok. We had a great nurse yesterday and today, and she’s helped me feel totally validated with my concerns.
Sunley had to be NPO again last night so she could have her pacing wires removed today so she was grumpy when I got here, and of course the doctors ran late getting here to pull the wires, so that was pretty miserable. When they got here, I got to have a good talk with them about the BP concerns (BP was still in the 110s/120s) and again felt very validated in not wanting to move to step up yet.
At rounds, just like I predicted the doctors from yesterday totally downplayed to the team the whole BP thing. They said “Maybe her new normal is just in the 110s” which brings me to this tangent:
When a baby is not hitting the health standard the doctors have set for them, let’s try something to get them to that standard instead of LOWERING THE STANDARD. Tangent over.
At the end of rounds, one of the doctors said that we could move to step up, and I very calmly (not calm in my brain) said that I would feel more comfortable with 24 hours of good BP before moving. The attending basically said that they might need our bed for more critical patients, which brings me to another tangent:
Please do not try to guilt me out of a hospital room for my baby, because I am all too painfully aware of other “more critical” babies, and that’s a horrible reality that will never change. I’ve been guilted out of a room before (remember our wasted hospital week on the Neuro floor?) and it was a HUGE mistake! I really think that’s a cheap shot to just get me to agree with you. And if the brand new tower needs more rooms, lets have fundraisers and get that done, but I’m not moving my kid when my ALWAYS right mom gut says no. Tangent over.
We decided to table the discussion to later in the day. But as the day progressed, Sunley had some still-too-high BP, minor desats, her skin was mottled, hands blue and cold, and she had small spikes in her heart rate (nothing crazy, but still enough to raise concern). Overall, her body told us all day long that we were moving too fast and she needed to slow down. So that’s what we did. Before we left tonight, she was having abnormally low ST stuff (that’s the technical term) so they did an EKG which came back normal. OH! She also FINALLY pooped and it was a DOOZY. We had to do a dressing change on her art line afterwards, and change all the bedding. So happy for her though, haha!
Today, November 10th
Sunley’s BP finally stayed under 100 systolic all night long. Her heart rate is fine and she was all smiles when we came in this morning which was SO wonderful to see! She is playing with all her old toys and doing all the Sunley things, so it’s obvious that her brain function has remained 100% since having so much bypass/heart stopped. She did struggle with her last 2 feeds, so I’m anxious to see her BUN numbers, because I feel like she might be a little dehydrated. She is still getting some lasix and is her input/output is still negative even though she is all PO (besides occasional Delaudid). If she is dehydrated, I think we can easily get back on track with some IV fluids.
Her coloring is much better, except when she gets super fussy. I THINK the coloring yesterday and for a short time today is from the dreaded Glenn headaches. It seems like her entire body tenses up, which just makes it worse. Hoping those don’t last too long!
We are still waiting on the team to round on us, but I really think they will move us to step-up as soon as a room is available, and I think she is ready now. We just needed to listen to her body telling us to slow down, and that’s exactly what she needed. She will need to get her double lumen fem art line out, and get an IV put in her arm (her first one went bad quickly) so I’m bummed she’ll get at least one more poke. But getting that line out will be good, since it’s very high risk for infection.
Photo of a Glenn headache: