Friday, October 31, 2014

Calm between the storms

Today has been nice and quiet. He did great on his shunt surgery yesterday and the scan this morning looks stable. He still has some stridor, but nothing like what it was and he was extubated ( breathing tube removed) so we can hear him again. No commotion in our room today, just lil Thomas and I hanging out. We are on the schedule for a decompression surgery on Monday so we are just going to enjoy some quiet cuddle time before another flurry of activity Monday and then a little rougher of a recovery after that surgery. I would have brought his superman costume if I had known we would be here for Halloween. Never has a costume been more fitting....


Thursday, October 30, 2014

Silence is deafening

We have been living with the stridor for a few weeks now, so to have him be silent is eerie, at best. With his breathing tube in, he can't even cry. He literally does not make a sound. When he is mad he still thrashes and throws his hands around and opens his mouth wide as if to yell, but only silence comes out. He turns red and it is so obvious he is agitated, but not a peep. The dichotomy of yesterday at home and today in the hospital is striking. We often couldn't hold a conversation if Thomas was in the room because the stridor was so loud and now....silence, literally! It is such an alarming experience to go through all of this with such a young little babe. With the other two boys, I wanted them to be different and unique and for my stories and experiences with them to be so special and personal that it was like our own mark on the world, brand new! With Thomas, I crave similarity. I am constantly searching Evil Mr. Google for someone, anyone, to have had the same trouble and taken the same path that Thomas is taking. We need company on this, not because we are miserable, but because we are unsure. I need path finders in front of us to say where they went right and left and show us what map to use. When I come across some of these people, I want to soak them in and have their strength and knowledge ooze into us. We could use strength as we send him off for his second shunt revision in 24 hours....good luck little boy!!! Come back screaming like hell, please

Re-shunt

The shunt revision from last night did not take. The catheter is not in an ideal position when they looked at it on CT scan today.  So they are going to go back in and re-do the revision. Less than 24 hours and surgery #2.....hopefully they can get the breathing tube out in the OR safely and he can be evaluated over the weekend. They will be watching for signs that his breathing has not improved and that will lead us to a decompression surgery on Monday. A much more complicated surgery that might not fix the stridor but will keep it from getting worse and also decrease the pressure being put on his nerves. 

He's out

He is out of surgery. It was the proximal catheter that had moved out of position. They replaced it and saw that the new one had good flow. The Doc is concerned about the Chiari though. He thinks that pressure is being put on the nerves at the base of the brainstem and that is what is causing the stridor....if that is the case then we will have another surgery this weekend to decompress. Basically remove a bone from his spine to give the brainstem some room. He doesn't want to do this since Thomas is so young, so we are going to watch him tomorrow to see if he can breath around the tube on his own and if he still has trouble on the inhale. That will determine if we need to do anything else. He is still sedated and we are in the ICU. 

Wednesday, October 29, 2014

Shunt hit the fan

Back at the hospital. Thomas is in surgery to repair a shunt malfunction. We came back in because his stridor had gotten worse. We went to the ER and it became what was the most terrifying few minutes of my life. We checked into the ER and they took us back to triage. The nurse was asking me the basic questions about Thomas while she was hooking him up to the monitors and then an alarm went off for his oxygen levels and she told me to get my stuff and she called for the emergency response team as we were hustled into another room. I spoke of the emergency response team in one of our NICU posts. It is crazy. 30 people came swarming from out of nowhere and started hooking up wires to him and manipulating him, feeling his lungs and throat. One doctor was yelling instructions and another asked if I was mom and told me to sign this paper. They grabbed a breathing thing-a-majig (scientific name) and it looked like steam was coming from it. They held it over his mouth. All while he is screaming and I can barely get glances of him between shoulders that are surrounding his bed. I hear someone explaining to me that his saturation level was 90 (out of 100) and they were giving him some medicine to relax the airway to see if that would help. A voice is still asking me questions...does he have normal temp? How much does he weigh? How long has he had the stridor? Does he have Chiari? They are finally satisfied with how he is doing and he is stable enough to get a chest and airway x-Ray. So we are whisked off to the X-Ray room. Accompanied by an MD and still hooked up to a machine because he could de-sat again. Once the films are done we are put in an ER room and this is the first chance I have had to sit and believe what just happened....this is serious. But before I can even call John and fill him in, a neurosurgeon comes in and starts asking questions. Has Thomas' temperament changed? Are his eyes sunsetting? Has head circ changed? No, nothing is out of the ordinary as far as his temperament. He starts looking at Thomas and examining him. Everything looks normal, but he doesn't flinch when the neuro puts a hand to his eyes....how have I not noticed this? I knew he wasn't focusing on us yet, but we expected that to be delayed, but I hadn't noticed the no flinch. You could tell that it struck the surgeon, he kept going back to it and trying again and my thoughts raced....he should be flinching....does he flinch at home....it's too bright in here....does that matter....should I have noticed this....has damage been occurring inside his brain that I could have prevented....then an ENT comes in. She scopes him and sees what we already knew:partial paralysis of the vocal cords. She doesn't know what is causing it but wants to keep him overnight for observation if he is cleared by neuro. We just did that, it's called a sleep study, why do I need to stay again?
Next thing I know we are off to get pictures of the shunt taken. Now Thomas is hungry and not shy to let us know. Now he's inconsolable...but I can't feed him just in case it's a shunt failure and he needs to go to surgery. After the shunt pics are taken we are back in our own room. And now he's really mad at me for not feeding him and there is no doctor in sight. LOOK AT THE FILMS!!! Everything is fine, just like last time...let me feed him. Well turns out I am the worst mom ever. Everything is not fine. His vents have increased and we need to do surgery tonight. Shunt repair surgery is pretty straightforward and they do it often here, but another brain surgery on a 3 month old is not ideal. Plus he has some croup that showed up on the x-Ray, so when they put the breathing tube in for surgery, they most likely won't be able to take it out in the OR. Which means they have to keep him sedated until the inflammation goes down. This could take days.....just hope for the best. He is in surgery now, will know more in two hours or so! They think it was the proximal catheter of his shunt that came out of place and that is causing the malfunction. It isn't completely in the ventrical and not draining effectively. If it is truly that simple that will be great!

Tuesday, October 14, 2014

Awareness

Thomas' sleep study has been scheduled for October 20. We are hoping to get some answers from it...in the meantime just gathering as much information as possible on vocal chords and the surgeries!!


Thursday, October 9, 2014

Latest with stridor

We had our follow up with ENT this week and unfortunately it wasn't the news we wanted to hear. The Doc heard the stridor and scoped Thomas and he saw the same thing that they saw two weeks ago, some movement, but he had an explanation. Thomas has the muscles to actively close his vocal chords (adduction) which is what you need to speak and make sounds, but he is not able to actively open his chords (abduction), which is what you need to breath. His chords only open about 30% of what they are supposed to. This is concerning because he may not be getting enough air. Multiple times they asked me if he turns blue or gasps or is in pain/distress ever. I feel like I am giving the wrong answer when I say no since everyone is always so surprised by it. I have never seen him turn blue or felt concerned that he wasn't breathing, but now that I have been asked, I think back to every weird noise he makes and when he yawns and wonder if I am missing something. The next step to see how much air he is getting is to do a sleep study. We go spend the night in the hospital and he is hooked up to all the monitors and they record his vitals and activity to make sure everything is normal. They also will check for apnea. The doctor said that if all of that is stable we will just wait and see if he can develop the ability to actively open those chords or if he will need intervention. The intervention may come in the form of a tracheotomy, which is slightly disheartening. I posed this dilemma to the SB young family group that I am in and it seems it is very common and many of them have gone through it, unfortunately all with different outcomes and advice. A few got the trachs and said it helped and was scary, but it is fine now. Some chose to do decompression surgery instead and were very happy with their choice and it solved the issue of the paralyzed vocal chords without a trach. A few mentioned a different doctor at children's who is an airway doc and has helped them with this issue. So, as usual, no clear cut path, but lots of options and wait and see. My concern with wait and see is damaging his vocal chords further and possibly losing function. One family was diagnosed with stridor and told to wait and see if their little one would out grow it, but he never did and they got a second opinion eventually and had surgery, but lost one vocal chord in the process due to damage. At what point do you question doctors and at what point do you trust they know what they are doing? I hate doing unnecessary surgery, but would hate even more losing a function because we were trying to avoid surgery. Also, what surgery do you pick? The more common one that is pretty straight forward but puts a hole in his throat or the more complicated one that wouldn't have outward signs and could still fix the problem? Hopefully the sleep study will have some answers. In the meantime here is a picture of my little trooper in his best known place, the hospital!!

Wednesday, October 8, 2014

Awareness month

October is Spina Bifida Awareness Month. At first I felt like I needed to be wearing the yellow ribbon and changing my profile pic on FB to 'I love someone with spina bifida', but I didn't like it. I do want people to know about spina bifida and that it does exist and if someone you know (or are about to know) is diagnosed with it, it isn't the end of the world, it's just a shift in your reality, a new normal. I definitely don't wish spina bifida on anyone, but I don't like the idea of him being a cautionary tale. "Take your folic acid or THIS WILL happen to you!!". While it is important to take your vitamins and be healthy, especially if you can't get all your nutrients in, but folic acid isn't a guarantee to avoid spina bifida and it is presented like it is. Spina bifida births did decrease when folic acid was touted as important for pregnancy, but also at that time, ultrasounds came about and the knowledge on how to look for birth defects. So the number of spina bifida births did decrease, but not necessarily all due to folic acid. In a recent year, there were around 3000 cases of spina bifida diagnosed prenatallly and only 1500 births. Many families who receive the diagnosis are advised, or choose, to terminate the pregnancy. So yes, spina bifida births are down, but folic acid isn't the only reason and when it is touted as the cure, or only prevention, of spina bifida, that is false and misleading and has lead to many nights of guilt for lots of people. Yes, take your folic acid, but not to avoid spina bifida, b/c your baby may have it anyways, take the vitamins to be as healthy as you can be for you and your baby, knowing that some of it may be out of your control. The real awareness should be given to the families who receive a diagnosis and the OB doctors who don't know a lot about spina bifida and advise termination based on misinformation. I have found many people whose OB doctors said that their baby will have a significantly poor quality of life because of the spina bifida and hydrocephalus. This isn't true. Hydrocephalus with a known cause like spina bifida isn't as debilitating. The pressure can't build on the brain since the fluid is still flowing out from the opening in the back. Also the soft spot allows for some give with the extra fluid. I completely understand the mindset in that moment of finding out your baby has spina bifida, your whole world crashed, all the images you had for your future with that little baby have been stolen from you, or so you think. At that moment when you hear your baby won't be mobile (which P. S. you can't tell anything with spina bifida for certain before birth) the only thing you can think about is how you had looked forward to walks in the park and playgrounds and sports teams. You hear that they won't be able to control their bowel and bladder and you think about sleepovers and camp outs and teenagers with diapers. You are told that developmental delays are quite possible. While all of this is running through your head like a bad dream, your OB, a trusted doctor, offers the option to terminate the pregnancy. Without being political, I see the point of view and I get it and being in that position has given me a new point of view and understanding on such a complicated issue. But, I digress, awareness month...I would like people to be aware that if they receive the diagnosis, life will change. Things will be hard, but that is true of just having kids in general. We are still so new to Thomas' spina bifida journey, but I see it differently already. He's a part of our life and we do what we need to for him and with him without thinking about it, it's not like we dwell on all his differences, we learn as we go and our life is constantly morphing. It truly is an adventure and I wouldn't trade him or change him for anything in the world, because then he wouldn't be him. That is what I am aware of and not just this month. 

Sunday, October 5, 2014

New normal of life with the Bif

This post is overdue...we had our first SB related scare. Thomas was having some stridor (high pitched wheezing when inhaling) that randomly started and then got worse over the course of a couple of days. I had remembered reading about stridor and how it can be linked to something common with spina bifida, so of course we googled it...how have we not learned to stop googling health related topics at this point??!! A call to the MM clinic and our pediatrician got us an appointment locally which was immediately followed by a trip to Cincy Children's ER. Our local ped didn't want to take any chances with his shunt.  We got down there and got right in a room, everyone we saw heard the stridor - which was a weird kind of relief, I wanted to be sent home b/c we were worrying over nothing, but I also wanted validation that we did the right thing by acting on it and coming to the hospital! We were sent for a CT scan to look at his airway and the ER Doc didn't seem concerned with what the imaging showed, she was thinking it was probably tracheomalacia, which is basically an exaggerated narrowing of the airway on inhalation, it can be caused by any number of things and is relatively common, but just to be sure they wanted us to be seen by the Ear, Nose and Throat docs. So they came in and scoped our little guy, they put a tube up his nose and then threaded it down his throat to see what was happening when we heard the sounds. I really wanted to look into the scope to see and I must have been making a weird face and stepping closer because they kept saying how it wasn't hurting him and how it was good that he was crying so they could see the whole situation - trying to reassure what they must have perceived to be a concerned mom. I wanted to correct them and say that I wasn't concerned about them hurting him and I wasn't nervous about his crying, I just want to play with your scope and see what it looks like in there, but I decided against it since it made me sound like an unsympathetic mom, so I just nodded and tried to look relieved that they weren't hurting him. They also didn't see anything of too much concern. With Thomas' Chiari Malformation (basically his brainstem pulled down into the spinal cord) sometimes it can put pressure on the vocal chords, paralyzing them and not allowing them to spread apart, causing stridor. If this were the case, we would be looking at a decompression surgery possibly. Obviously another surgery on this poor little man is not ideal especially a complicated one like a decompression surgery. But they didn't think they saw that, although the vocal chords did freeze up when the stridor was happening, they moved correctly when he was crying normally. This was good news and they thought that the stridor and intermittent spasiming of the vocal chords could be due to reflux, so they were planning on sending us home with reflux medicine after they checked in with the attending doc. Sometime later the ER doc comes back in and said they are going to admit us for the night. This came as a pretty big surprise since we were packing up to go home based on the last doctors that had been in our room. Apparently someone up the chain of command wasn't comfortable with an educated guess about reflux and wanted more tests run to make sure it wasn't a silent shunt failure or symptomatic Chiari. So we had another CT scan done of his whole shunt this time (shunt series imaging) and we were put on the schedule for an MRI in the morning. Usually they sedate the kids that can't be distracted by movies or music, but we were able to try it without sedation and I just fed him and swaddled up tight and hoped for the best. He will have enough done to him in his life that the less anesthesia and stuff like that we can do the better. He did great with the MRI and the images came back stable and no signs of shunt failure or pressure from the Chiari. On that note they sent us home with the offer to stay another night if we wanted him to be observed overnight....ummmm thanks but no thanks, we went home! Home with no real answers but also no concerns, so we'll take it, for now. This is what we are to expect when there is a slight health blip with our little Tom Tom, we need to rule out all the big stuff and be happy when it is just something normal like a cold or fever or fussiness. We will always have bags packed when heading to the hospital and also always have phone chargers with us (I may have had to borrow the nurse's phone charger so I could get through the night with a working phone....). Also as he gets older we will always have books, snacks and stuff to do. Lesson learned and new normal still taking shape!