Steady as he goes

Henry had a nice, and rather uneventful week. As we waited for our care conference with several doctors and nurses on Friday, we only made some small changes to his dexmedetomidine and continued to condense his feeds. We had requested the conference so that we could all understand, at a high level, what challenges remain for Henry before we can go home. 

Our first step is to see him show consistent weight gain. Because he is still on a fairly high dose of the milrinone to help his heart, we need to know he is at least capable of gaining weight with that much support. Even if he only goes up 10-20 grams (about half an ounce) each day, it will show he is capable of gaining weight. Only then will we begin weaning him from the milrinone. The hope is that his heart will be able to keep up and he will continue to gain weight. He did have an echo this week which showed that while the leakage in his tricuspid valve has improved since before surgery, the function of the right ventricle has not yet improved, and may be slightly worse. It is not terrible, and they have seen kids be successful with the same amount of disfunction, but it is something we will be watching closely as he is weaned from the milrinone.

After failing to come off of the dex last weekend, we have weaned him much more slowly, by only .01 or .02 micrograms (per kilo per hour) each day. At our current rate, we hope to be off of the dex by then end of this week. As his does is lowered each day, Henry gets pretty cranky for a few hours in the afternoon, but he does get some oxycodone to help him through it. He is usually less fidgety by night and is able to make it through the night without additional drugs. at other times when he is fussy, he can still be consoled the old fashioned way - he enjoys being held!

As far as feeds go, we have worked down so the amount he was getting over three hours (about three ounces) is ran over an hour. Henry seems to be tolerating it well, and we have just begun working with a bottle more regularly. It seems he got rather lazy during the six-plus weeks when he was getting continuous tube feeds, but we think he still knows the mechanics. The bolus feeds will help as the more time there is between feeds, the more he will feel hungry.

So while we wait for Henry to grow and be weaned off of the dex, if his next echo mid-week looks good, we will start to wean the milrinone. Optimistically, the doctors think we night be coming home within 4-6 weeks. Again, that is a best-case scenario, but we are hopeful that we are all home sometime in June.

Moving Forward

In the week since Henry's surgery, we have had some good days and some bad days. Overall, he is doing really well. We have been thrilled to see our little boy acting more and more like himself. We feel like we are finally moving forward, but know we still have a long way to go. Over the coming days and weeks, we will be working on three main things: dialing in the appropriate dosage for his heart and blood pressure medication, teaching him to take his feeds by bottles, and weaning him from his sedatives.

Henry will get an echo on Tuesday, which will help us figure out how to start moving him off of the IV heart medicine (milrinone) and on to the oral medication (enalapril). We have already started shifting his feeds from a continuous drip to bolus feeds. Giving his stomach some time off in between feeds will hopefully make him feel hungry, and want to take a bottle. So far, the main thing we have tried is to wean him from his IV sedative, and it has not been as easy as we would have liked.

On Friday, the doctors decided that Henry was ready to come off of his dexmedetomidine, an anti-anxiety medicine he has been on since he was intubated on St. Patrick's Day. However, Henry did not feel the same way. About an hour after his IV was turned off, he got very restless and started making lots of moaning sounds, and by the time the night shift started, he was gagging and throwing up. Both of us were frightened, as we were not sure what was happening with our little boy, and did not leave until after midnight and Henry had settled down.

Saturday was Jason's 34th birthday. Henry gave Jason a picture he made of balloons with prints of his hands and feet. But Henry was still not himself. After attempting to give Henry some oral narcotics (morphine and oxycodone), the doctors decided to turn back on the dex and to wean him down more slowly. Wow! We had a whole new baby within an hour. 

Henry still was fussy over the rest of the weekend and also making moaning sounds, but he had stretches where he was alert and happy. Sunday night, he was able to make it through the night without needing extra doses of narcotics to help him! He can again be soothed for long stretches by either some patting, music playing, looking at his mobile, or his favorite - being held.

On Monday, Henry was way more playful! He would coo and caa and smile and giggle! It was such a blessing to see that beautiful smile again and to hear him laugh. He is still showing signs of withdrawal as he is weaned off of the dex, but is more happy times than sad.

Tomorrow, Henry will have the echo to see how his heart is doing after the Glenn surgery and aorta patching. We have been warned to not expect any drastic changes and are nervous, to say the least, on what the results will be. 

Thank you all for your thoughts and prayers and well wishes for our son!

Post-Op

Henry greeting us this morning

Henry is doing very well. He was extubated around midnight last night. We are still weaning off all of the meds needed during and immediately after surgery, but his vitals so far are great!

Immediately after surgery

Two Down...

Henry is out of surgery and is doing well. We were told late Monday that we were going to be the first surgery today. They took him to the OR a little after 7am, and made the first incision around 9. A little after 11 am he was successfully off of heart-lung bypass. He has a good rhythm and his valve leakage and heart function is where we saw it right before surgery, which is the best outcome we were hoping for.

This surgery was a lot more stressful on Paige and I. The debate the doctors had between transplant vs. Glenn mainly came down to whether or not his heart was strong enough to start back up OK after being stopped for surgery. While that was still a risk when he had his Norwood, this felt a lot different. I think the biggest difference was that we had started to get to know Henry. We had seen his personality, held him, played with him.

We stayed up at the hospital all day Monday and through the night, taking turns holding him until we were to tired to do much more than sit and pat him. If all is truly well, he will be extubated later his evening and we will be one step closer. Henry has a lot more hurdles to clear before he comes home, but this feels like the biggest one.

Thank you everyone for your thoughts and prayers today and as we journey onward.

Henry resting before his Glenn

An Anxious Weekend

This weekend has had Jason and myself on edge as we anxiously await for Tuesday to get here. At this point they have stopped Henry's breastmilk feeds. His vitals have been very near the border of where everyone is comfortable. The oxygen levels around his kidneys and intestines were trending quite low Friday night so we paused his feeds and upped his milrinone. We waited to see his blood pressure stabilize and make sure it wasn't too low before they started the feeds again. Overnight, he had a bit of throw up, so they paused the feeds again.

Henry's surgeon, the rest of the ICU team (and Jason and I) agree that it is not worth the risk. He is being switched to IV nutrients (TPN and lipids) until after his surgery. We are very close now, and no one wants to rock the boat.

And so, we are hanging out with Henry, trying to not get too anxious at the numbers at each assessment. Henry is doing fine, but I think he is as ready for Tuesday to get here as we are!

In the meantime, here is some more information about the surgery on Tuesday.

This is a great diagram I found from an article in the journal Nature. You can click on the picture to go the full figure and legend from the article. It goes into excellent detail about the surgical procedures.

Right now, Henry is at image B and the post-Glenn procedure is image C.

After the surgery, they will try to get Henry extubated as quickly as possible, once he has woken up. This is because the ventilator will actually be counteracting his new physiology. After the surgery, the blood from the upper part of Henry's body (from the head to about level with the heart) will be passively draining into his lungs. The vent would be giving positive air pressure preventing that from happening.

The biggest concern is to see that his heart function returns to at least its current level, once they restart his heart in surgery. The risk is that it will not be strong enough to pump on its own so he can come off of the bypass machine. We feel confident that if the surgeons thought there was little chance of coming off of the bypass, they would not do this procedure - but the risk is there.

Back in December, Children's Mercy had a Kansas City station air "Inside Pediatrics". The series follows a set of families and their children's journey through various medical conditions. For the first three parts, one baby is followed that has hypoplastic left heart syndrome just like Henry. Unlike Henry though, this boy was able to go home for some time before his Glenn. The journey in the videos follows him through the Glenn procedure, which is what Henry will have on Tuesday. For those interested, here is the link: http://m.kmbc.com/news/inside-pediatrics-premieres-dec-17th-on-kmbctv/30119040

There are several doctors and nurses in this series we have run across in our stay here at Children's Mercy. Fair warning, this is a real tear-jerker.

We are not expecting to be able to go home as quickly as this little boy did. Even when Henry is fully past the main risks of the surgery, he will have been on a ventilator and a cocktail of opiates & benzodiazepines. He is going to need some time to re-learn how to breath and eat, and we will have to begin the struggle of weaning him off of the drugs.
We still have a long road to travel to get home, but we do feel this is the right choice for Henry. Thank you, everyone, for your support and continued thoughts and prayers for us and Henry. 

Stage II

This morning Paige & I had our "care conference" with 10 doctors and nurses to hear what the full cardiology department at Children's Mercy felt was the best course of action for Henry. Henry's surgeon came right out and told us that they think the second surgery for HLHS - the Glenn procedure - is the best choice for him, and that they will do it within the week. This was wonderful news, but it took Paige and I almost entirely off-guard. First, we thought if Glenn was still on the table, we would be told that Henry would need to wait for some undefined period of time before he would be ready for it - essentially, "wait and see". The other reason it came as a surprise was that we had fully braced ourselves for the transplant option.

That is the main reason we have not updated everyone for over a week. Last Wednesday, we made an extra trip to Kansas City to have an introductory meeting with the Heart Transplant team. Henry's status had not degraded, but there was still a lot of concern over whether or not his heart was strong enough to do the Glenn. As we spoke with the transplant team, it was becoming more obvious to us that the doctors were leaning that way. We have spent the past week grappling with whether or not we wanted to hold out for the Glenn or if we felt we were ready to open the Pandora's box that is heart transplantation.

Thankfully, we don't have to make that decision just now. Henry is set to have surgery next Tuesday, the 14th. This is excellent news, but it really just tells us which obstacle course we will be running next. There is still a lot of concern that Henry's heart is not functioning as well as they think it should be, even with all of the support he is getting. Part of the surgery will involve stopping the heart for 15-20 minutes or so, and this is riskier than it sounds given his function. We believe the surgeon would not commit to this unless he truly felt Henry can pull through, but we will have to wait and see.

We will pass along some more information in a few days about Henry and what lies ahead for him and us. For now, we are going to enjoy a day with good news.

Glenn

Henry will have the second surgery, the Glenn procedure, sometime within the next week. We will get more info up later. There will still be a lot of hurdles, but for today, this is good news!