*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. 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 at 6 weeks old.
I wrote this post early this morning, but forgot to post before we left for Sunley’s appointments. Updates from the appointment are at the end :)
After 5 days and 5 nights, we were able to be discharged last Saturday morning, and we’ve been home since with a baby who FINISHES her bottles! The morning of discharge, we had a new cardiologist working our floor, Dr. Mott — Yet another fabulous doctor that we really enjoyed having. He immediately recommended Sunley be put BACK on Enalipril. This was super frustrating, after all the back and forth discussing her meds with Dr. Schlingmann, and I told Dr. Mott that I didn’t know whose opinion to go with since Sunley’s plan of care seems to just depend on which cardiologist is on duty that week. He was very sympathetic to our concerns, and after much discussion, we decided to put Sunley back on Enalipril. That drug is usually to help with blood pressure issues (which Sunley doesn’t have), but for her it is used to help her single ventricle (the left one) fully relax in between pushes. She is expected to need Enalipril for her whole life.
Later today, we have a checkup with Dr. Ayres. I plan on having a discussion with her about our frustrations with Sunley’s med switching. I don’t want her on anything if she doesn’t need it, and I need to understand why there seem to be different opinions. I think maybe that it just wasn’t clear to the cardiologist WHY Dr. Ayres had her on Enalipril, but also, it shouldn’t be up to me to explain it— that kind of stuff should be in Sunley’s records.
I also plan on asking about starting Sunley on some solids. Most people wait until 6 months old to introduce solids, but when Hadelyn was about this age, I started her on an avocado/breast milk mixture and she loved it. I’m hoping we can do that with Sunley and get her some good fats in there. Also, since I know everyone loves hearing about my breastmilk, it has started to become more fatty! Yay!! I’m sure you all wanted to know that. It hasn’t been tested or anything, but it’s visibly got more fat, and that should mean that it has more calories so that’s great. I won’t ask yet if we can stop the formula fortification, but I’m hoping we’ll get there soon. I doubt that we will ever get to breastfeed again, and I’m so thankful that we had a couple weeks of that in the beginning.
Oh, and before I forget: Sunley rolls BOTH ways now —back to tummy has been happening for a while, but now she can do tummy to back also! Momma is proud. She reaches and pulls things to her mouth, giggles, smiles — She’s hitting all the milestones you’d expect from a heart healthy 4 month old.
Our big kids get here tonight!! They’ll stay through Monday, and we can’t wait to see them! This is the longest they’ve ever been away. Derek was able to see them a couple times since they left, but I haven’t seen them in 3 weeks. That’s a lot. Thankful for Duo, and thankful that we have so many people willing to help! I’ll try and get an update on here tomorrow with the results from Sunley’s appointment today. Hoping for good news all around.
Sunley’s appointment went very well. I got all of my questions answered, all the Enalipril why’s, etc. Dr. Ayres explained to me (again! Thank goodness for her patience haha) that in her experience, Enalipril seems to help many single ventricle patients long term, even though there may not be anything clinically showing up right now that deems it necessary. Because Sunley’s heat has to overcompensate with the added workload of having only one ventricle, the Enalipril helps that ventricle with it’s function, and helps avoid enlargement of the heart. Sunley’s heart today was very slightly enlarged since the last appointment, but nothing too concerning. Dr. Ayres agrees that we can stay off of the diuretics, and we can start some avocado feeds! She also suggested that we continue to work on Sunley’s trunk control. She is right on track for a 4 month old, but Dr. Ayres said that sometimes being able to sit upright after surgery helps with the infamous Glenn headaches. Have I mentioned those here before? Ya. Not fun, and not just any headache — Visibly swollen head, feeling like your hanging upside-down, 24 hour a day headaches. Hoping they are short-lived, or better yet NONEXISTENT for our Sunleyshine.
We will have a follow up appointment in 4-6 weeks, and the cardiolgist expects for us to schedule Sunley’s Cath and surgery at the next appointment. So we are getting closer! Mixed feelings there. Dr. Ayres also mentioned that the surgeon, Dr. Heinle, may consider doing a different kind of Glenn procedure than we thought we were getting— as in, he may choose to NOT close off the pulmonary artery. We will see, jury’s still out with that.
Last but not least, our big kids arrived safely today!! So far we’ve been swimming and had a pizza picnic in the living room. Very much looking forward to some quality time these next few days!!