Sutton's bronchoscopy

Sutton's bronchoscopy didn't happen until late this evening, so we just got our update from his nurse. The procedure went well and he is still pretty snoozy from all of the sedation. 

His ETT (the tube that is in his throat to help him breathe) was changed to a different size because it was found to be rubbing and further irritating his airway. He will get some decadron, a steroid, overnight to help decrease some of the inflammation in his airway. There was discussion of possibly extubating him tomorrow, but we expressed concern over doing that because of the trauma of the last extubation (both for Sutton and for us). His nurse will address these issues with the physicians and we will most likely keep him intubated until after surgery. He will have to be intubated during surgery, so it seems silly to extubate him when he will have to be reintubated in 48 hours. 

This does give us more hope, though, that after the debulking maybe he can breathe on his own and we can avoid a trach. Oh boy, how we would like to avoid a trach. 

Cohen's adventures

Poor Cohen has been passed around to various friends and family members all over Missouri and Illinois. And because he's so easygoing, he seems to have loved every minute of it. There is new scenery and new playmates at every stop. 

But the kid still misses home. He even tells us which toys he wants to play with on our rides back. 

Kedric and I just want to give a heartfelt thank you to all of the friends and family that have provided us with support, encouragement, kind words, prayers, food and even child care over the past two weeks or so. There is no way we would still be functional without each and every person that has helped us. Please know that, even if we fail to respond to a message or return a call, your kind sentiments are greatly appreciated and often come just when we need them the most. 

Fire drill

Some if you may not appreciate this as much as others, but one of the most daunting things about having a newborn is the significant lack of sleep. When they wake in the middle of the night to nurse, there is incentive to get out of bed and feed them. However, I have been waking to an alarm every 3 hours around the clock for the past 10 days. Couple that with the surprise nature of our situation and traveling 2 hours away from home to be with Sutton and you have one exhausted mama. Needless to say, it's very hard not to ignore my phone alarm in the middle of the night and go back to sleep. However, I am mass producing milk - about 4 feedings worth at every pumping (that may be TMI for some of you, sorry!) so I allow myself one 4 hour stretch in the middle of the night just to get a little extra sleep. 

Last night, almost exactly on the mark of hour 3 on my 4 hour block, the fire alarm goes off at our hotel!

By the time we got back to our room, I was pretty close to my 4 hour mark so I lost out on a little extra sleep last night. At least I got a little nap in on our way back home this afternoon! ;)

Our newest game plan

After reviewing the various images from Sutton's MRI with his ENT doc, we have a game plan that takes us out about a week or so - our longest yet.

They weren't exaggerating, the masses (as there are several small cysts rather than a few large ones) take up a large area in his neck and upper chest. His trachea is significantly deviated as a result and there is even some tongue displacement because it is pushing up on his jaw. According to his doc, the majority of the cysts are more solid. She says this is not necessarily better or worse than a more fluid filled cyst, but the more solid cysts do not respond very well to sclerotherapy and this is possibly why we did not have great success with it last week. The tricky part is that on the right side of his neck and around his clavicle there are cysts surrounding a bundle of nerves and two major vessels, one that runs down into his arm and the other up into his neck. These are more fluid and, because of the risk, will not be resected (removed). If they become an issue the sclerotherapy may be an appropriate treatment option. Despite all of this, Sutton really likes his pacifier!

He will have a bronchoscopy done late in the afternoon on the 5th to take a look at what happens to his airway when there is no tube holding it open. We do not know how long the cysts have been present (I had my last ultrasound at 28 weeks and it was not seen then, but it could have been difficult to see even if we had been looking for it) and it may have weakened the cartilage in his trachea. This is in preparation for a partial resection where they will remove as much of the mass in the front of his neck and chest as is safe, in the hopes that it will be enough to relieve the pressure on his trachea and jaw and allow things to fall back into the appropriate place. The partial resection is scheduled for Monday afternoon and will take several hours. 

The incision will be on his neck so he will remain intubated until it heals. After it has had some time to heal we will reassess whether a trach is still necessary. Any way you spin it, we are looking at another several weeks to a month here in St. Louis and will spend the next several years following up on an outpatient basis with his physician. 

I asked the ENT to explain what the interventional radiologist meant when he said that the condition would resolve itself by toddlerhood. She said that the condition will not go away, but it can often remain dormant without swelling as long as the individual isn't sick. When he gets sick we may see some swelling in his neck. For those of you that have children, you appreciate what a task the next several years may be in maintaining his health, especially with a 2 year old at home!

Also, yesterday they removed his umbilical line and placed a PICC line on the left side of his head. They had previously shaved some hair off in an attempt to get a peripheral IV in and they ended up having to place the umbilical line but, fortunately, they were able to place the PICC in the area they already shaved so he incurred no further hair loss. :)

Right now, we just want all of the procedures to be as safe and successful as possible. We are really hoping to avoid a trach still, but only time will tell. 

MRI results

We received a call from one of Sutton's physicians early this afternoon with the results of his MRI. Unfortunately, they were not what we were hoping to hear. His hygroma is rather large and involves major vessels. It extends into his chest just below the clavicle and is much more complex than the ENT typically sees. We have not spoken with his ENT as she is discussing surgical possibilities with her colleagues before going over them with us. She will likely have some options for us tomorrow. We do know that she has OR time available on Monday, so any surgery would probably occur then.

The good news? The ultrasounds of Sutton's heart and abdomen that the geneticists wanted came back completely normal. This gives us more hope to lean towards an unknown cause instead of a genetic cause.

Tomorrow morning we will head back to St. Louis to be with Sutton and discuss the options for him.

Meeting with the geneticists

We spent some time talking with the geneticists today. In 60% of cases of cystic hygroma there are chromosomal abnormalities. The most common include Turner's syndrome (found only in females) and trisomies 13, 18 or 21 (of which Sutton displays no traits). 

Based on our family medical history and Sutton's overall health, we're putting all of our eggs in the it-was-just-a-fluke basket. If that is the case, the docs say that it would be unlikely we would see this show up in subsequent children. 

More or less, the genetic counseling is being used to rule out any chromosomal abnormalities. As many of you know, we planned on more children, so this counseling is extremely important to us. If  we are wrong and there is a chromosomal abnormality it will change our plans dramatically. BUT, that's a big 'if' right now. And seeing as I'm only a week postpartum, future family planning isn't an immediate concern. 

The results of these tests will take 2 weeks to a month to come in, so we'll likely put this topic on the shelf until then. 

MRI

Today was the day for Sutton's MRI. We had originally anticipated an early morning MRI, but because his condition is stable he was put on the schedule for early afternoon. Then, as sometimes happens, one of the machines goes down and everything gets backed up. Fortunately, his docs pushed to have it done today when MRI wanted to reschedule for tomorrow. At 4:00 they started preparing him for his travels. They replaced all of his monitoring equipment to be MRI compatible, snuggled him in some sort of papoose to keep him still and assist in moving him around for the procedure, and gathered up respiratory therapy (RT), our nurse, and some other staff to transport him. It was quite the production. 

They gave him 2 hours worth of Fentanyl instead of taking his drip with him and the little booger fought falling asleep quite impressively! He has a bit of a temper and doesn't particularly like when he isn't swaddled, so once he got into his papoose-thingy he was quite happy and comfortable and eventually started to doze off.

It is unlikely that we will have any results or a game plan before tomorrow. After speaking with the attending, ENT, residents and fellows, our hope is that they won't find anything new from the imaging today that wasn't already seen on his ultrasound last week. If that is the case, there is a possibility that they could remove the affected lymph nodes and wait for his swelling to go down and trachea to shift back to midline. That, in fact, is our only issue right now - the deviation in his trachea. His labs are otherwise unremarkable, he is up to his goal feedings without incident and he remains on CPAP (where he does the work initiating his breaths) instead of the ventilator settings. 

Although that is our hope, the ENT made it clear that many times the affected nodes can be wrapped behind the trachea (the ultrasound would not have been able to see this) which would, at the very least, complicate any surgical removal. They have made us aware that there is the possibility of needing to place a trach in order to maintain his airway until the condition resolves itself. When Sutton had sclerotherapy (the drainage of some of the larger nodes) last week, the interventional radiologist noted that he doesn't see this in teens because its typically resolved by the time they reach toddlerhood. All things considered, we are very fortunate that this one thing is what is keeping him in the NICU. 

There are a lot of 'what ifs' swirling right now, but we are just trying to be patient and wait for all the data to come in so we have a clear picture of what lies ahead for Sutton.