The Downward Spiral - Discovering Lillian's Heart Failure

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.

Home For Good, But For How Long?

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.

Home at Last! With a Surprise Trip

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.