Sorry this post is a little late. Lillian had her sedated ECHO last week, something we had been trying for almost two weeks to get. The results of this are good; her aortic arch is wide open and there's no sign of the blockage returning. We do see that the SANO shunt is narrowing, showing that Lillian is outgrowing it. At some point it might get too narrow; in that situation we could either do a cath to balloon it or do another surgery to replace it entirely with a bigger one. Obviously we would probably do the cath option first.
Other than the ECHO it was a relatively quiet week. Lillian is continuing to fight eating, and is down to at most 20 to 40 mL by mouth a day. The rest we have to gravity bolus. It's disappointing to lose ground on this as this was one of our target areas to work on while waiting for transplant, but at least Lillian is still relatively stable.
Lillian was put on oxygen earlier today. Those of you in the medical field could probably tell that by the nasal cannula that she's sporting above, something that she now has to have 24/7. Katie and I are mixed about this. We knew that eventually she would start the declining as she continues to out grow her heart, and this is one of the first management techniques that we can use. At the same time, we've grown accustom to being able to give her as close to a normal life as possible, something we are immensely grateful for. Lillian is hitting all of the normal developmental milestones, including sitting up and grabbing. She's generally happy and social, so even though she's in the hospital 24/7, she has a reasonable approximation of a normal life aside from the daily evals and shots and whatnot. The oxygen makes it harder to do these things, and is just one more thing to carry around. However, it has vastly improved her color, and we're hoping it will help us regain some lost ground on eating since she's functionally lost that entirely over the last couple weeks.
To briefly touch on the other options that we have for management, we could increase her Milrinone (she is at .5, Children's will go up to 1.0 on the floor and higher in the CICU), but any change in dose of Milrinone requires 48 hours observation in the CICU. At some point though, even that probably wouldn't mean anything, so we could do a second surgery to tide her over. That could mean either replacing and enlarging the Sano shunt or doing a Glenn procedure (which if she wasn't getting a transplant would be the normal next step anyways). We would probably replace the Sano if we did do another surgery though, a Glenn would make the transplant operation itself much harder and more risky. If it really came down to it, we've been told we could place her on a Berlin heart. This is an external heart that does all the work of the heart. We know of another baby who was on a Berlin for 8 months so that certainly gives us time. Obviously, we would prefer to avoid as many of these as possible, but we will do what is necessary for Lillian. Only time will tell if this is even necessary, as a new heart will render all this obsolete. But that is an unknown. I'm frequently asked if we have any sort of a time frame for a new heart, but we simply don't have anything frankly. We will simply get a call one night and that will be it. They tell us the wait is normally 6 months (even though it has seemed much shorter lately), but even after 3 months on the list I have to tell myself every day that "we only have 6 more months of this", as I don't want to pass up arbitrary deadlines and deal with that disappointment.
So for now, we stay on the oxygen. Lillian has a sedated ECHO tomorrow in the cath lab. She isn't getting a cath (as much as I would like her to considering she'll be there regardless), but the only people willing to sedate an infant on Milrinone are the cardiac anesthesiologists in the cath lab. Since they can't leave their posts, we have to go to them. I don't know when we'll get the results from that, but they're almost guaranteed to show decreased function. Hopefully, they won't also show the blockage returning.
On a different note, we met a 2 year old transplant recipient the other day. She's an inpatient but looks like a smaller shyer version of Annabelle, and I assumed at first she was a sibling. She's there because labs didn't detect any Cyclosporine in her blood, which is kind of a big deal, but she looks fantastic. Unfortunately, she now has symptoms of a viral respiratory issue, even though all labs for that are negative. Since she was playing with Annabelle the day before that happened, Annabelle is banned till Wednesday, much to Lillian's disappointment. According to Kathryn, she was extremely bored today, and misses Annabelle. It's hard to describe how much Lillian's face lights up when she sees Annabelle, but it's sufficient to say they're fond of each other.
To counteract this kind of a downer post, here's a video of Lillian laughing while being tickled yesterday. Even though she is declining, as you can see in the video, she still looks remarkably good. Kathryn and I have to remind ourselves frequently that we are extremely lucky to have her in the condition she's in, where we can hold her and play with her. Many families don't even get that.
Today was also the 90th day that we've been listed.
The best little girls ever! Lillian had a much better day today than she did yesterday. She kept more food down, she was in a better mood and she had more energy. I didn't mention this early, but she was extremely lethargic yesterday, barely able to stay up for over an hour without passing out, which is extremely unlike her. Today, she was back to her normal self, although she still wasn't interested in eating her later feeds.
We also found out that Lillian almost got a heart this weekend. I'm not entirely sure how to feel about it as we really want a heart for Lillian, but this also would have been a mismatched heart. This heart was offered and rejected by the child at the top of the list (more accurately by the doctors for the child at the top of list), and was offered to (and accepted by) another child on the list in California. We found out from our heart doctor that if this child had rejected the heart, it would have come to Lillian next. This means that Lillian is now basically 2nd on the national list (at least as far as I understand it) and will hopefully get a heart soon.
As a closing note, another CHD mother made a slideshow for a bunch of HLHS kids, and Lillian was one of the babies that she asked to include. I've included the video below.
Some interesting things have been happening today and the last few as well. Lillian has been losing interest in eating lately; now she likes to fight us every time we try to feed her. And today after eating 20mLs we had to bolus the rest and we tried doing a drawback on the feeding tube to get any air out her stomach and instead we got some bright yellow liquid, which is not what we were expecting. The doctors don't seem too worried about it at the moment, but Andrew and I have noticed that she seems extremely lethargic today and is much fussier than normal. We're a little worried because these mirror the initial heart failure symptoms. And no one can explain the yellow liquid in her stomach. Normally any formula residuals don't change color so we don't think it is that, and it isn't nec either as that would produce a black or red liquid. We'll just have to wait and see for now.
Sorry about the lack of updates again, hopefully the mobile app means I can post a little more often. Anyways, Lillian is still waiting for her transplant but doing relatively well considering. We are remind daily at Children's that no matter how dire your situation there is always someone worse off. It's not an encouraging thought but it teaches us to be grateful for the health Lillian does have. We're always thankful that Lillian is relatively normal in terms of her development so that once we get a heart we can hopefully move on with our lives. She has been sitting up on her own, something the nurses tell us kids generally don't do in the hospital. I'm a little worried about the next stage of crawling/walking with the PICC line in her leg, but she's done well so far.
Lillian has been going up and down a bit recently in her health, but relatively minorly. She lost six ounces about a week and a half ago, almost enough to send her back to the CICU to get her Milrinone increased, but we've avoided that scenario for now. I'm grateful for that too as an increase in Milrinone does mean that we're starting the decline as she outgrows her heart. The weight loss was mainly related to Lillian's frequent emisis and lack of digestion but we've been able to counter that so far by increasing her calories in certain feeds and checking her residuals to try and reduce overloading her and making her spit up. I am worried about the lack of digestion, but it's not unexpected either. As she gets bigger she will begin to start outgrowing her heart and a consequence of that is less blood to the stomach and thus poor digestion. We still have many options to manage her heart failure and keep her running while waiting for a heart including putting her back on continuous feeds or TPN, increasing Milrinone or even putting her on a Berlin heart if need be. We would just like to avoid doing them as long as possible as they either set us back in terms of development (learning to eat again) or substantially reduces her quality of life (the Berlin heart requires you to be in the CICU and likely paralyzed).
But again, she's doing ok at the moment, and even though we're a couple more lost ounces from the CICU, we've avoided it for now and she would be back fairly quickly if she went.
