I haven’t written a blog post in so long, as life seems to have ran away from us lately. In October we welcomed our fourth little one (and first girl!) into our family, and the baby stage coupled with the holidays never lends itself to a lot of free time. Although things haven’t slowed down by any means, Emmett’s Fontan surgery is one that I want to document for myself as the details are easy to forget over time. Despite the memories not being pleasant ones, this surgery is such a milestone and therefore such an integral part of our lives that I wish to keep record of it. I also find it incredibly helpful to read about others’ experiences in the heart world, and hope that sharing our story can be encouraging and helpful to others behind us on this journey.
Prior to surgery, Emmett had a heart cath done in February– February 14th to be exact. We took it as a good omen that this procedure was done on Valentine’s Day. We drove down to Indy the night before and stayed with my sisters-in-law who thankfully live less than 20 minutes from Riley Hospital. We had to be there at 7:30am, and they took him back around 9am. Although this is considered routine, it is still done under general anesthesia (at least for kids). Thankfully everything went seamlessly, they coiled one collateral (which makes things easier at the time of surgery) and afterwards we talked with the cardiologist who completed the cath. He told us that the pressures and everything looked great and that Emmett is an ideal candidate for the Fontan (such a relief!). We were then able to join Emmett in post-op; he had to stay laying flat and keep his leg (where they inserted the catheter) as still as possible for 4 hours afterwards. Although there were some initial tears coming off of anesthesia, he calmed down and enjoyed popsicles, apple juice, and snacks while watching something on an iPad and throwing a ball around with myself and Taylor. He was discharged that afternoon– we luckily did not have to stay overnight– and he seemed back to his normal self by the time we got home! Although I was less nervous about the cath than the surgery, it was still a good feeling to have the heart cath behind us.
Despite having almost a month between the two events, the surgery date arrived in the blink of an eye. Leading up to it, we were quarantining in an effort to keep Emmett healthy (same as prior to the cath). If Emmett showed any illness symptoms for 4-6 weeks prior, he would be at risk of having the surgery rescheduled. As much as you don’t want surgery day to arrive, you also just want to get it over with and don’t want to have to re-do the waiting period. Although a little lonely and isolating, the quarantining thankfully paid off and we were able to proceed as planned.
Emmett’s diagnosis is Tricuspid Atresia with a hypoplastic right ventricle, ASD and VSD. The Fontan is the final of 3-staged surgeries for single ventricle patients; he had the PA band at 7 days old, then unexpectedly needed the BT shunt at just under 3 weeks old, and then the Glenn at 6 months. The Fontan surgery is usually completed between 2-4 years old with some variability based on the heart warrior’s needs and cardiologist preference. Emmett at the time of surgery was 2 years + 4.5 months. The Fontan surgery is where the surgeon disconnects the Inferior Vena Cava or IVC (which brings the deoxygenated blood from the lower half of the body back to the heart) and instead of going to the heart, reconnects it directly to the pulmonary artery going to the lungs. This essentially reroutes the returning blood, bypassing the heart. This is beneficial because in Emmett’s case his right ventricle is basically non-existent and his left ventricle has been working double time to compensate. This rerouting of the deoxygenated blood takes that workload off to relieve his left ventricle.
The day before surgery (March 11th) we headed down to Indy for Emmett’s pre-op appt. This went much quicker than last time partly because we got to skip blood work (they were able to draw labs during his heart cath the previous month) and partly because there was less waiting around between providers. He did have chest X-rays and then we met with various members of the team most notably from anesthesia and his surgeon. We went over details of what to expect, asked questions, and signed consents. Leaving the pre/post-op area that day was such a melancholy feeling, knowing we would be back there early the next morning for the big day.
The day of surgery we had to be at pre-op at 6am, where we had more paperwork to complete online, lots of people coming in and out, and of course lots of waiting. The anesthesiologist did give him some medicine to make him sleepy/out of it to make the process of him leaving and putting him to sleep easier. So once we gave him that and changed him into a hospital gown, they took him back shortly afterwards, at 7:30. This part feels like it never gets easier, the goodbye. Regardless of how many times you go through it (with Emmett, this was his 6th time going under anesthesia, and 4th heart surgery), it still feels like a part of your own heart is getting forcibly removed from your body when they take your baby away.
{{Since then, I have pondered how this surgery compared to his earlier ones and the truth is some parts were harder and some easier this time around. Going into it we knew what to expect and so there were less surprises, which makes things like seeing him with lines and wires less traumatic. Because he’s older, the physical recovery for his Glenn and Fontan also went smoother compared to when he was a newborn. However it’s a double edged sword in that because he is older and more aware, as a parent it pulls at your heartstrings in a different way. For example, when we first joined him in the CVICU, the nurse told us that he had been calling out for me prior to our arrival and that broke me in a way that is hard to put into words.}}
The waiting game while your child is in the OR is as terrible as you imagine it to be. The emotions of waiting for updates are only amplified by the lack of sleep the night before, as it’s hard to sleep when you know what the next day will bring. In the family waiting room, a nurse comes around every hour to give updates on how things are progressing. Each time she came around I caught myself subconsciously holding my breath as I prayed she would confirm that all is going well. Thankfully (a word that doesn’t seem quite big enough) the surgery itself went very smoothly, and by 12:45pm we heard news that he was extubated in the OR and already in the CVICU. By 2pm we were able to join him, and 20 minutes later he was in my arms–the best feeling in the world! The fact that he was extubated in the OR was already so surprising to us, but we were floored when we realized he was alert enough to be talking before we even arrived to his room. This was a huge difference compared to his other surgeries, and we took this as a good sign of progress to come.
When he arrived in the CVICU, Emmett had a central line in his neck, an arterial line in his wrist, 2 peripheral IV’s (one in each arm), 2 chest tubes, temporary pacing wires, and a Foley (catheter). Although he was on room air initially, he did need some extra oxygen support overnight that first night, as his sats were dipping below where they wanted him to be. The next day he was able to come off oxygen without issues. Prior to the Fontan, his sats were upper 70’s to low 80’s, but his new target post-Fontan is upper 80’s and even low 90’s, which they explained may take a bit to get there.
Sleep in the hospital, but especially in the ICU is hard to come by. The checks are frequent, the beeping and noises from machines are loud, and things like labs and chest X-rays don’t wait until a reasonable hour. That first night was especially hard for sleeping; Emmett was woken up frequently and cried each time. Thankfully he was comforted by me being there, but it did take a while for him to fall back asleep each time. Throughout his hospitalization Taylor and I took turns staying overnight with him, while the other went to Taylor’s sister’s house for a much needed night of sleep. Emmett quickly became skeptical and scared each time someone entered his room and came near him. Some of the nurses or other providers would ask him “can I….” to which he would quickly respond “no!”. That was hard, seeing his fear and yet knowing he really didn’t have a choice in the matter.
Wednesday, Post-op Day 1:
His chest X-ray that first day post-op showed his lungs were a little wet (or “fluffy” as they jokingly call it) which is common after heart surgeries. He was started on lasix and another diuretic to help him pee off the excess fluids.
As far as food went, he was able to eat and drink as much as he wanted with the caveat that he is on a low fat diet, per his surgeon’s orders. After surgeries like the Fontan, there is a risk for developing chylothorax, which is when a fatty lymphatic fluid can build up around the lungs. Some surgeons will recommend a low fat diet once the patient is already dealing with chylothorax, others (like ours) recommend it to all patients after the Fontan. This low fat diet will be something that he needs to follow until he gets the approval to stop, hopefully at the follow-up appointment with cardiology (5 weeks post-surgery). His limit is 10g of fat per day, and this includes healthy fats too such as avocado, nuts, eggs. You don’t realize how much fat is in different foods until you have to limit it!
Ordering food turned out to be very frustrating; the kitchen staff taking the orders seemed to be seeing different diet restrictions than what we were being told, so we’d try ordering what we thought he would be allowed to have only to be told constantly that he’s not able to have it. We had one of our nurses call down to clarify/make adjustments which helped somewhat and eventually a dietician made some additional changes to his orders so that they were less restrictive.
Today, Emmett had his arterial line removed (which is an IV line in an artery in his wrist that measures his BP continuously). This is a requirement before moving to step down, and a sign that he was not having any blood pressure issues. He also had his central line capped as they weren’t actively using it. He was willing to have some food today although his appetite was significantly decreased compared to normal.
Thursday, Post-op day 2:
Emmett’s overnight chest X-rays showed “marked improvement” since yesterday! They are decreasing the lasix to 3x a day and stopping the second, stronger diuretic.
Emmett has been doing so well that orders were placed to transfer to the step down unit, however it ended up not happening today due to staffing. This has happened to us in the past where we were told we would be moving to step down and it not happening, so we have learned to not get too excited simply because orders are placed.
There was also talk of removing chest tubes today but they decided to keep it one more day. He is having some, although minimal output from the chest tubes. They say that having chest tubes in can be very painful/uncomfortable and that patients feel much better once they are removed. We were hopeful that tomorrow will be the day!
Friday, Post-op day 3:
We’ve learned throughout our various times being inpatient that hospital life is full of inconveniences, moments of frustration, and instances where you are forced to simply roll with it. Exhibit A today was when Emmett and I were woken up at 7am after another night of very little sleep to be told that we were getting transferred to the step down unit in 15 minutes. So in my pjs and slippers before getting a chance to fully wake up, I scrambled to get all of our things packed up and loaded onto a cart so we could move units.
Moving to the heart step down unit was definitely a welcome change! Not only does it signify that Emmett’s making progress and no longer considered as high-needs as before, it also means more rest for him (and us) as there are less checks and interruptions (and less beeping!!)
Today his lasix was further decreased as his chest x-ray was looking really good. He is now only on Tylenol for pain. The biggest thing today was getting his chest tubes and pacing wires removed! (He did get some morphine prior to help with the pain). It was hard on him as well as us because the removal is not pleasant and Taylor and I ended up helping the nurse and NP keep him still for it. Thankfully it went fairly quickly. Later that afternoon he had another chest xray and an echo. The additional chest x-ray this afternoon was to make sure that no air entered the space where his chest tubes had been during the removal process, which is a possible complication.
A dietician came today to talk to us about the low fat diet and answer any questions that we had. She gave us some handouts of low fat meal ideas and like I mentioned earlier, helped adjust his food orders so that ordering food was a bit easier. Taylor’s parents also came to visit today and brought Violet so we were able to see her and I had a chance to nurse. The hospital was not allowing visitors under the age of 18 including siblings at that time (due to cold/flu season) so we met them down in the lobby and I stayed down there with her while Taylor’s parents took turns coming up to visit Emmett. It’s been hard being away from our other kids, but especially Violet as she’s just under 5 months and still nursing. This whole time since leaving home I’ve had to stop and pump every 3 hours or so, which is never fun but is especially inconvenient when I want to be focused on Emmett. I kept telling myself it should hopefully not last too long, which thankfully was the case. They started mentioning the “H” word (home) and told us that Emmett could potentially be discharged this weekend, which we weren’t ready to believe!
Saturday, Post-op Day 4:
Emmett is now down to lasix 2x a day, and Tylenol every 6 hours. His appetite is still not back to normal, he is eating much less than he typically would. However, this is to be expected and not a concern.
Taylor’s parents came to visit again, along with Violet and Taylor’s grandma. It was nice to be able to nurse Vi again and to see loved ones. It’s a breath of fresh air from the monotony of the hospital.
The big event today was getting Emmett’s central line removed. They gave him some versed (medication to keep him calm/sleepy) so that he wouldn’t fight it. Unfortunately, the process ended up being a little traumatic, both for him and for us. We again ended up helping to keep him still, and restraining your child while they are turning red from crying/screaming is just horrible. In addition, once the NP put a bandage on the site and turned to snipping the external parts of the sutures on his incision, we noticed that Emmett had started bleeding quite a bit from the central line site, so the NP had to remove the bandage and apply pressure to stem the bleeding. They also replaced the gauze over the chest tube sites with steri strips, and we realized that Emmett had a bad reaction to the adhesive from the bandages. His poor skin was so red and raised all over where the adhesives had been. All in all, we were so relieved when the whole ordeal was over. Emmett who hadn’t napped prior to that quickly konked out from a combination of tiredness, the versed, and the adrenaline rush.
Taylor went to the pharmacy and picked up the medications that we would be going home with which were lasix (2x a day), aspirin (increased to a full tab daily), and Tylenol and oxycodone as needed for pain management.
We tried to encourage Emmett to sit up on the side of the bed this evening with hopes of getting him up to try walking. However, he was not wanting any part of it. He cried and kept pointing to laying back down, and although we couldn’t convince him to try walking or getting into the little push car that they had, he was eventually willing to sit on our laps (alternating between mama and dada).
Sunday, Post-op Day 5:
We were thrilled to learn that Emmett would be discharged today! In addition to excitement, I was also feeling a little nervous as Emmett had still not yet been out of bed besides to sit on our laps at that point. This morning we were able to coax him up by laying a mat down on the floor and offering to play ball with him. He seemed very unsteady on his feet, like he couldn’t find his balance, and it was very nerve-racking figuring out how to support him/catch him safely while he was stumbling. (Post surgery, he is on sternum restrictions for 6 weeks until his sternum heals which means no grabbing and pulling him up from under his arms). We were told that for the most part, kids after open heart surgery will self-limit and will refrain from doing things they shouldn’t be doing simply because it hurts. However, sternum restrictions also means that when we get home, his older brothers need to be careful around him and not wrestle or rough-play, something that made us nervous with a 4 and 7 year old. Thankfully since we’ve been home, for the most part they have done a pretty good job at remembering and respecting those limitations!
The last step was going over discharge instructions and signing paperwork, including discussing medications, low fat diet instructions, physical restrictions, and follow up appointments. Emmett was scheduled for a follow up in person with our primary care provider’s office that coming week, a virtual appointment with Riley surgery’s office for the following week, and a cardiology appointment for 5 weeks out post-surgery. After that we were officially released and on our way home! We could not believe how smoothly everything went with his recovery and how quickly he was able to go home. We definitely feel incredibly blessed and in awe of our sweet boy’s strength and resilience!
