I apologize about the lack of posts, I find these posts extremely time consuming to write but worth the effort for cohesion and clarity in the future. For the record, we are still not up to date with this post, but I'm getting closer. The line at the top about being up to date soon will change or disappear once I am actually caught up, which I really, really hope will be before the new year.
I ended my last post with information we got on October 13th about noticing decreased cardiac function, arrhythmia and valve leakage. We were sent home on an EKG halter monitor, which we returned after having Lillian wear it for a day. We didn't hear much back from this, so it looks like the arrhythmia wasn't much of an issue. Lillian remained home with us, spending some more time with Annabelle.
This time at home was going to be short lived however. On October 21st, Kathryn noticed that Lillian's appetite was way down, to the point where she only ate about half of what we would normally expect her to eat. Knowing that this is the first sign of heart failure, we emailed Lillian's cardiologist, Matthew Files about it late in the day, feeling that it was serious, but not serious enough to take her to the ER. Matt called us back at 8 AM the next morning telling us to bring Lillian in as soon as possible, with the understanding that she was very likely going to be admitted. The plan was to evaluate her and do some tests and see how she was doing.
The first tests of an ECHO and X-ray didn't show anything to be majorly concerned about, but Lillian was still showing clinical signs of distress. Our suspicion was that her aortic arch was narrowing, but we simply couldn't tell via ECHO. If it was a narrowing, my impression at the time was that we would have two options: either doing the second surgery (the Glenn) early or by adding another surgery for a total of four, but we needed to wait for the cardiac catheterization to tell us more. Still waiting for a room to clear up so we could be admitted while waiting to do the cath, Lillian had some blood drawn so they could run some labs that could give more answers.
The results of the labs were quite surprising, and indicated (contrary to the ECHO and X-ray) that Lillian was in severe cardiac distress. Specifically, one of her hormones, BNP, was far higher than normal. BNP, or brain natriuretic peptide, is a hormone secreted by the ventricles (or pumping chambers) of the heart in response to cardiac stress. In a non cardiac patient, we would expect the BNP level to be under 100pg/mL, and ideally under 200pg/mL in a heart patient, but it can be higher without too much cause for concern. Lillian's BNP was 1700pg/mL, indicating severe cardiac distress needing immediate attention. This was enough to move the cath from Monday as planned to an emergency procedure on Saturday, as that was what she required. The cath doctor talking to me, Dr. Agustin Rubio, said that he would have done the procedure that night if he had the required support staff, but that it was best to wait til the morning when they could come in. With the plan to do the cath in the morning, Lillian simply slept the night out as an inpatient once more.
With Lillian quickly back from her second hospital stay it was time to start getting on with our lives. Lillian was unperturbed by returning home, Annabelle was positively thrilled at the prospect of having her sister back. It is really amazing to see how much Annabelle loves her sister, always wanting to interact with her in some way, whether trying to tickle her or trying to get Lillian to do something.
Even with Lillian home, we still had to return to the hospital rather frequently for checkups to make sure that her heart was still functioning properly. The first of these appointments was on September 2nd, only a couple days after Lillian had been discharged again. This initial visit held discouraging news, Lillian had lost 4 ounces and her heart was swelling; a serious issue for anyone, but especially for a post open heart surgery infant. If the swelling was still up in a week, we would have to do a cardiac catheterization, a procedure where catheters are inserted into the major arteries that allows us a very good look at her heart, but the procedure isn't without risk. In the mean time, having the heart swelling meant more blood to the lungs rather than the body, which was the entire point of the first surgery. Regardless, all we could do at this point was wait.
I'm going to take a little detour at this point and do a brief overview of the medicines we were giving Lillian at the time, as I think its rather interesting myself and may be useful to others who find themselves in this situation later. From left to right: Captopril (1mg 3x daily) to help her heart, Lasix (.4mg 2x daily) a diuretic, a gas medicine (.3mg, as needed), Keppra (.5mg 2x daily) for seizure prevention, Naistatin (1mg ~4x daily) for the thrush, and some water to flush. Not pictured, asprin (1/4th pill, 20.25mg 1x daily) a pro-biotic (half a capsule 2x daily), topical naistatin, morphine (which she's off of now) and tylenol.
On September 9th, we got the news that Lillian's heart swelling had gone down, something Kathryn and I were thrilled to hear as it meant that she was not going to need the procedure just yet. Lillian also gained 6 ounces so solid progress overall.
At this point I'm going to switch modes a bit as September was relatively quiet by Lillian's standards. So, I'm just going to post a couple of cute pictures during the time period to recap instead.
Annabelle's first photobomb
On October 13th, we got some news from a doctor appointment that one of Lillian's heart valves was leaking and her general cardiac function was down, likely moving up the schedule to the next surgery. Lillian also had a mild arrhythmia, requiring a halter monitor to watch her heart function at home. The bad thing about this is the halter monitor has to be worn for 24 hours. The good part is that the mesh top they give you gives you the perfect costume for a European rave.
Ready to party
Lillian was always happy to be home, and we were thrilled to have her there.
With Kathryn as a mom, it shouldn't be a surprise that the first thing that Lillian got to do after getting home and waking up was to take a bath. Annabelle was pretty happy to have her sister home too, but it had been a long day for Lillian, and soon she was sound asleep. Katie and I had to give Lillian her medicine for the first time, which was an interesting if ultimately uneventful thing.
The next day, Annabelle really got down to business with Lillian. Her sister was finally home, it was time to play!
Of course, Lillian might have been a little too bundled to play with Annabelle, but Annabelle was going to play with her. We took both girls out for a while, which to our surprise was more tiring for them than for us.
The next few days were interesting and exciting for the girls as they adjusted to their new roles, but relatively uninteresting in the long run. Annabelle continued to love playing with her sister and talking about her, Lillian just got used to being out of the hospital. We also had some visitors, most notably, her cousin Robert.
Robert was always ready to strike a pose, Lillian was less so, preferring instead to make a face. Overall, everything was good at home, good until August 29th that is. The 29th was Lillian's welcome home party, but during the day we noticed that she was spitting up more than normal and had what looked to be bad thrush. Only when she became completely lethargic though did we really think something was up. At the advice of Seattle Children's, we brought her back into the hospital to be evaluated. They determined that in addition to having thrush and a yeast infection as we suspected (both brought about by a wide spectrum antibiotic we gave her earlier to combat a suspected infection), Lillian was also pretty dehydrated. This doesn't sound like a huge deal, but was actually the biggest deal of all, as when a HLHS baby with Norwood circulation gets dehydrated, they stop sending blood through their shunt to the lungs. This obviously isn't ideal, and was probably the reason why Lillian was so lethargic. They did have some problems getting an IV in while in the ER due to her small veins, taking 4 tries to get an IV in to give her fluids and draw labs. Even though the labs looked normal, they wanted to keep her overnight, so she was readmitted, not even a week after she had left.
Our overnight stay soon turned into two days overnight, as the thrush infection looked pretty bad. We had started to suspect that the thrush was the actual cause of the dehydration, so we had to make sure it was gone. But we were able to take her home again on the 31st, so a relatively short stay after all.
With Lillian on the floor, most of our hospital stay was behind us. Now we just had a short period on the floor to make sure she was stable and to get us used to caring for her for the first time and getting used to her special needs. Lillian though, was clearly ready to be on her way out, as she was more alert every day.
Since she was finally stable enough to move, it was time to do the MRI that we had postponed so long to check on her brain bleeding. I was really viewing this test with apprehension, as the brain bleeding to me was almost a bigger issue than Lillian's heart. Her heart issue was serious yes, but it can be fixed and managed with relative ease. A mental issue though would be much harder to deal with, likely requiring a life time of special mental care. This MRI would tell us if Lillian would have to deal with that on top of her heart condition. A lesser worry too was that for the MRI to be useful, the patient has to be completely still for the procedure, a state that is almost impossible to get with young children. Thus, having an MRI involves being put on some sedative, and most infants are intubated on sedatives as a precaution. Since Lillian took a long time to come off the ventilator the first time, we were hoping to avoid the same thing again as a consequence of something as routine as an MRI.
Luckily for us, the radiologists happened to come at a time (on August 19th) when Lillian was dead asleep and completely still. They were able to do the procedure without having to intubate or give any sedative. The results from the MRI were also great, the bleeding had gone down and likely would not be an issue in the future. In all, exactly the results we were looking for.
The next day on the 20th, we had to remove Lillian's PIV line due to that clogging as well. We were hoping to remove the PICC line first as that goes in farther, but we had to leave it in for a little while longer. In the mean time, we were focusing on teaching her how to eat, a reflex we had suppressed since birth. Lillian struggled with this at first, she simply wasn't used to having anything in her mouth. She continued to struggle on the 21st, but was enjoying the visitors that she had.
Annabelle was thrilled to spend time with her sister
Lillian's little cousin Robert also came to visit, but she was tired at this point.
On August 22nd, we finally took out all of the medicine and continuous drips, leaving the line in a couple more days just to be safe. But seeing the pump rack finally leaving the room for good was a relieving sight.
Gone for good!
Lillian also had her great grandma and great aunt come to visit.
Since Lillian had done fine without any of the IV medications, it was finally time to take out the PICC line on the 23rd, leaving her feeding tube as the only tube in her, which doesn't even count since it goes through her nose. She had gone from 19 tubes (yes, I am counting all IV pump lines individually even though they consolidated before entering) to zero tubes, a massive milestone, even if it is only a mental one.
White patch is the last reminder of the lines.
We were starting to near the end of our journey at the hospital! There were only a few major milestones left, including removing Lillian's stitches and getting her back to oral feeding. The later is important, but is also something that we could do at home, so only the stitches remained. Kathryn and I thought it looked like it might need a little more time to heal, so we were surprised to hear that they were ready to be removed on the 24th, and removed they were.
Now all that was left was to make absolutely sure she was ready to go home by doing a car seat test and teach us how to place a feeding tube. We did both on the 24th. Lillian did great in her car seat test, and I did surprisingly well in my first time placing a feeding tube. If you ever find yourself in a position where you have to do this, it really isn't as bad as you would expect.
During the time since she had been on the floor, we had been trying to wean Lillian off of Morphine, which was proving difficult given that she had been on it for over half her life for this point. The day of the 25th was especially difficult, as Lillian was having a hard time with the wean and was extremely fussy. The 25th was also the first day she got to wear clothes, so you can imagine that this combination led to a less than happy child.
Finally, the time to go home had come! Lillian was stable, off all IV medicines, repaired, closed and stitched up and clothed. It was time to go home just after 4:00 PM on August 25th.
We finally had a good couple milestones under our belts! Lillian's swelling had gone down, she was off ECMO, and her chest was finally closed. But we weren't ready to leave the CICU yet, she still was on a ventilator, as well as medicines like Milrinone that can only be given in the CICU.
Our next step after closing her chest was to trial taking her off Lasix so we could remove the clogged PICC line in her arm before it caused any significant damage to her blood vessels. Lillian was also suffering from intermittent arrhythmia, a relatively minor issue, but just another thing that we had to deal with. Her medical team suspected that it might be an electrolyte issue, so we began labs on that to see. In the mean time, Lillian was more and more awake, in part realizing what was going on around her. She was on a ton of pain killers and intubated, but she was still awake enough to scream, but couldn't do it out loud because of the ventilator. Seeing her silent screaming was more difficult than seeing her immediately after surgery, but an extra dose of Morphine helped her feel better quickly.
By midday on the 12th, Lillian's arrhythmia was gone, but was back by midnight. One of the nurses suggested that the cause might be one of the central PICC lines being in too far and actually sticking into the walls of her ventricle, irritating it, which would certainly cause arrhythmia. My impression of PICC lines so far is that they go to the heart, not actually in it, but this line may have been different. On top of the central line possibly being in to far, the arm line was now completely blocked and leaking, so it needed to be pulled. Ultimately, the planned MRI on the 13th to reassess her brain bleeding had to be postponed due to the arrhythmia and low blood pressure; and we instead went to look at getting a new line in to replace the clogged PICC line. Here is where there can be a major difference, as the first nurse could not get one in in 30 minutes, but another one got it in in less than 5 minutes.
Only 15 tubes left!
An added benefit of removing the clotted line was that we could also remove her central drainage tube, which you can see gone with the white bandage above. She still had the side drainage tube for the pneumothorax, but the chest one was the main drainage tube, meaning that most of her chest bleeding had gone down. We could also restart the Lasix through the new line and lower 2 of the medicines keeping her in the CICU. And the best news of the day (still the 13th for the record) was that her fevers were down, indicating that she likely either did not have an infection or had already fought it off.
On the 14th, the cultures confirmed that there really was not an infection, so the fever and white blood cell counts were likely just an immune response to the procedures they were doing after the surgery. We were revealed to find that out as we had been fearing a bad infection. We were also able to turn down her Dopamine and the ventilator, ultimately putting her on pressure support (to keep her lungs from collapsing, but not actually breathing for her) with the latter early on the 15th. This went well, as the trial off on the 15th went well enough for us to extubate her and take her off the ventilator.
On the 16th, Lillian's recovery was really becoming apparent; not only did we take out the last drainage tube, but we were able to turn down the Milrinone again and hold her at long last. This meant that we could move to a less intense section of the ICU, and was also the day that I returned to work. Kathryn had the chance that day to take what is still one of my favorite pictures of Lillian:
We also turned off the Milrinone that night, which she tolerated well. This was the last thing keeping us in the ICU, so we were now able to go to the floor when there were spots available. Since there weren't though, they left in her arterial line, as that allowed us to continuously monitor her blood pressure. Even though this posed a slightly higher risk of infection, we were alright with leaving it in considering how recent the Milrinone shutoff was. We also took her from 8 IV pumps to 3, containing only flushes and foods (lipids/TPN). With this switch, all medicines were now oral instead of over IV pumps.
Alas, our move to the floor on the 18th appeared to not be happening again. We did decide to take out the arterial line at this point, which was dramatic as something wasn't properly closed before removing it and blood began shooting everywhere until the nurse was able to clamp the line. With it out successfully, we also started her on Captopril, a drug which lowers blood pressure to help her heart be more efficient; and doubled her food intake. We also finally had a bed open up on the floor, finally taking us out of the ICU around 9 on August 18th. We had been there since July 23rd. Here's a last little collection of pictures from the CICU.
Our time in the ICU was very stressful, but it was made so much easier by the never ending support of tons of friends and family members. We especially want to single out the staff at Children's NICU and CICU, who have been nothing short of wonderful, giving us hope and pep talks when we needed it (Julie specifically), always ready to sit and answer all of my stupid questions, and doing a phenomenal job of caring, treating, and fixing Lillian. We would not have survived without you!
Lillian's first real day in the CICU was on August 5th, as she came there out of surgery some time after 11:30 PM on the day before. Since she was post op and on ECMO, she was extremely fragile. The first day after surgery was immediately eventful, as even though she had been on the ECMO circuit for barely 12 hours, she had already developed a clot within the circuit and we needed to change the ECMO machine before the clot got into her system. Since she was in a small shared room with 3 ECMO machines in it (2 for her, one for the baby girl next to her), 2 ventilators, massive racks of IV pumps and 4 nurses, I had to leave, but you can see in the picture I snapped first just how crowded it was. It was so crowded I couldn't even get the ECMO machines in the picture, but they're at the bottom.
Before they had changed the ECMO machine, they had the Heparin (an anti-coagulant) levels in the lines fairly high to prevent them from clotting. This clearly didn't work, but we had to balance the risk of the clot against something else, as they had found some bleeding in her brain. This is extremely dangerous as blood in the brain ventricles (note that brain ventricles are completely different from heart ventricles that Lillian does have an issue with) can push against the brain tissue, causing brain damage and even death if it gets bad enough. We have to carefully balance that though, as using too little Heparin can cause the machine to clot as it did once before. Plus she would be at risk of a stroke and her heart has to work harder to push the blood, giving it less rest, completely counteracting even putting her on ECMO. If we over do the Heparin, we increase the bleeding on the brain, which could cause the aforementioned brain damage or death. It's an incredibly tight line to walk. However, despite having these complications, we still saw some hope from Lillian, as she was already stirring despite being on full bypass and hopped up on tons of pain medicine. Once they finished with the ECMO circuit, we finally saw Lillian's eyes open a bit.
Especially with how fragile she was after surgery, it was great seeing signs of life from her.
And despite the fact that she was functionally unconscious with all the medicine, Lillian's natural reflexes were in full view, as she worked on an imaginary pacifier.
One of the side effects of changing the ECMO machine was that Lillian had to have a flood of Heparin to prevent the lines from clogging during the switch. As you might expect, this caused the bleeding in her brain to get worse. This we unfortunately just had to deal with as not changing the clogged ECMO machine would have been fatal eventually.
Lillian's second day in the CICU was not as eventful as the first, but had its own ups and downs regardless. On the good side, we could see that Lillian's blood pressure had started to pulse on its own (the atrial lines she has allow for continuous blood pressure monitoring as opposed to having to use a cuff), which was a sign that her heart was getting stronger and starting to beat on it's own initiative, something that it had not been doing yesterday. To compare, immediately after the surgery, her heart was so tired that her blood pressure line was flat when it should have been pulsing similar to a heart beat or oxygen levels. On the bad side, it looked like Lillian had a minor seizure. It was small and we don't think it did any damage, but Neurology still wanted to start her on a anti seizure medicine just in case it was the start of a trend.
There was more good news though, as Lillian's chest bleeding had gone down, and she reacted well to a scale down in ECMO, exactly the type of thing we needed to take her off of ECMO.
On August 7th, we were ready to try to take Lillian off of ECMO. This started with a trial phase where the circuits were clamped shut, but she was still connected so if she still needed it, it would be quick to put her back on it. The trial was about 2 hours, after which they decided Lillian was doing well enough off it to take her off ECMO entirely, which was a major step in her recovery. Once they took her off of it entirely, the surgeons told us that her heart looked good and there was no bleeding as a side effect of de-cannulating her.
On August 8th, Lillian's condition took a turn for the worse even without ECMO as she started developing a fever (at 38.4c). This is normally the first sign of infection, which is especially worrisome given that Lillian still had her chest open and was still in the immediate post operation time period. Whether it was from an actual infection though or just a standard immune response from having them change things around in her chest, we weren't sure. The next day, August 9th, the fever got worse (39c or 102.2f) and her white blood cell counts were up. Since we now had two signs of infection (but no sure confirmation from early labs) we began treatment with a wide spectrum antibiotic and ran much more extensive labs to confirm any possible infection.
Later in the evening on the 9th, we were able to get Lillian's fever down to 38c, allowing us to start using Lasix again, which was important as she needed Lasix to get rid of fluids, which in turn would allow us to close her chest. However, we decided the next day that switching the PICC line from her arm to her leg was more important than closing her chest, as the PICC line in her arm looked like it was clogging, which would have severely damaged one of her vessels to the point of making her next surgery much more difficult. In the end though, after waiting most of the day for the PICC line, it was delayed due to an emergency with another patient.
Changing course from a daily recap for just a minute, I took these pictures to try to emphasize how many IVs she had going in. Some had been removed already (especially the ones relating to ECMO), but there will still 10 or so, all consolidating to the PICC line eventually.
On August 11th, we were able to get a new PICC line in her leg after taking her to Interventional Radiology to have it placed. Although a trip to Interventional Radiology is not normally required to place a PICC line as it can normally be done at the bedside, Lillian's vessels were extremely thin, requiring the more advanced placement. This timing was very good as her other PICC line in her arm had in fact clogged at this point. Even with the clog, we had to leave it in for the Lasix as that requires a separate line as it does not interact with other medicines well. More importantly than the PICC line though was that they were finally able to close Lillian's chest after having it open for 7 days. Having a chest left open that long is not normal, and was putting Lillian at increased risk for infection. Unfortunately, it looked like the closure also created a pneumothorax (basically a small air pocket) in her chest. This required a new drainage tube in her side, but was a relatively minor issue.
