Luke started kindergarten this week. It has gone well so far. He is still getting used to things and there is a lot for him to take in. He has been doing his morning work that involves coloring apples with letters or numbers and writing out the alphabet (not clearly but he made the letters to the best of his abilities). His favorite time is music time when they play songs on a big screen and sign, dance, count, say the alphabet, and chant.
Kevin built a new cart for him to push his vent around in. So far it works really well. Luke is still working on the steering part though. Luke walks so well that I do find it sad at times that he is once again tied to the ventilator. I feel that this cart is helpful but it isn't just walking by himself. For us, this cart is great and allows us to move around without hauling over 50 lbs of equipment or having to pull a wagon behind Luke. We haven't tried a playground yet but it has been raining a LOT.
The leaves are beautiful reds mainly with oranges, yellows, and maroons mixed in. It is relaxing and calming to look out on the beautiful fall trees. Fall is my favorite time of the year and I am enjoying it. Up until about a week ago it was beautiful outside so we got to enjoy quite a bit of it.
Hannah is doing well and is dressing herself in matching clothes more and more which makes her look even cuter than she already is. She wants to be running the show, to have all the ideas, and to be in control of all she does. She is one to do the opposite of what you suggest even if you suggest her favorite thing. She can turn into a sad girl at the drop of the hat and everything comes to a halt. However, she can also be super sweet with "Thanks yous" "Great idea mom" and "Sure mom". She is still really into drawing and coloring and really good at it. She is naming her babies now and she takes them all over like a regular mom would. It is really cute to watch her. She is also still in the book mode and loves to have lots of books read to her.
Luke has been playing with the kitchen set more and likes to put his head in the dishwasher section and look up. It is very amusing. Translucent letters are probably his favorite thing to play with. They are lines and curves of different colors. They have instructions on how to make letters but we end up making elevators, bedrooms, and people as well as letters. The other day we were playing with wooden blocks and we ended up pretending by making some people ride in a car to the playground and then go down the slide we made with steps (essential oil shipping tubes make great tunnel slides). Hannah just put her people down the slide but Luke stepped his up all the steps and then down the slide.
Hannah is getting too big and has been banned from our bed while both Kevin and I are in it. Usually she wants in our bed in the middle of the night and she has been easy to rock for a minute and put back into her bed so far. I am waiting for the morning that I wake up without having had gotten up to put her back.
We are still waiting for the final report from CAMP and last week I turned a corner and pulled myself out of the pit of stress waiting for that and have been doing much better since. Getting Luke ready and to school has also been a bit stressful but he is there and we are moving forward on that front.
We have been back to school, playgroups, and small group which has been great for my spirit as I am getting back into time with friends face to face. :)
Tuesday, October 7, 2014
Monday, September 29, 2014
Still in limbo...
So after getting home we realized that we have a lot of decisions to make based on Luke's care. We are still waiting for the final report from CAMP and can't really do anything until then. I am a person who likes to make a decision, good, bad, or indifferent, and then deal with the consequences. I don't like being in limbo.
What are some of the decisions we need to make? Our current pulmonologist doesn't seem to be willing to work with CAMP on vent settings and we are taking this opportunity to find someone closer to home, we think. We were at the current pulmonolgist becuase we were going to do the pacemakers through them but now will do them through CAMP as we like their design better. We generally get along fine with the current pulmonologist and the nutritionist from their office and I actually agree on how I am feeding Luke. However, they are 4 hours away! and not near any of our parents. Then, do we decide to switch to a new vent? This depends on if we are going to try for pacemakers in the next year or less? Which depends on how thing are going with the new settings and what the final report from CAMP says in follow up and what they think? We would have to switch medical supply companies to get a different type of vent and if we do that I don't want to go through all the process with our current medical supply company to get some of the new equipment that we will need. Although I really like my ordering people and the respiratory therapist at our current company. An one piece of equipment is looking like it will be a fight to get it covered by insurance. And then there is school we are trying to get Luke to...and we are adding a vent to all the other things that are different about him... And it goes on and on...
I am tired, stressed, and have shut down a bit. I pray that the final report comes tomorrow and we can start really making some informed decisions. I feel like I haven't been there as much as I should for the kids. I have been on the phone or e-mailing about this a lot this past week and I just want to be done. Please pray for wisdom, discernment, patience, and sleep.
On a fun note, we took a spur of the moment trip to the UP wilderness to relax and destress a bit. I feel the most relaxed surrounded by God's creation and it was good. Although, I must say that it is not as relaxing with two small children who just didn't appreciate the beauty of waterfalls, brightly colored trees, and being able to see 3 great lakes in the matter of an hour as much as I wanted them to. I think they did have a good time and enjoyed doing something else for a day. For me even the drive was beautiful and it was nice to get away. I have been wanting to make that day trip for a long time as I missed the water and the UP, there is just something that draws me in about the UP.
So what are the kids up to these days?
Luke loves to play 100 Floors and Doors on his iPad while he is being fed. He still likes letters and playing around with them and spelling different words. He also likes having you draw things for him. He has been into having me tell him stories about subject he chooses lately instead of dictating stories to me. He still likes to read a lot and spends a lot of time babbling on his iPad. He likes to go outside for walks, although he would like to be doing the riding and not the walking.
Luke has doing okay with wearing the vent all day. Kevin is making him a new cart for it tomorrow. Right now it is on a scotter but he isn't great about pushing it around. He is walking really well by himself when we follow behind with the vent. And he does walk away from the vent at least once a day forgetting that he has to take it with him. We are hoping the cart will help him be able to get around more as he can walk with it.
Luke is looking more and more like a little boy every day. I think he is beautiful and I tell him. I am not sure what he thinks of that. I love the haircut Kevin gave him in prep for our time at CAMP and the sleep study and it does help make him more boy and less toddler. He has also been communicating more with his iPad and with more comments and not questions. Although, he sometimes refuses to communicate or lately when we don't have the iPad and I ask yes or no questions he will give me both answers. This very frustrating and I haven't figured out what will motivate him to stop doing it yet. But I have to remember that even under all that medical equipment he is still just a 5 year old boy. He can be sweet like honey and a stubborn and a mule but he is so lovable and he still lets me give him lots of kisses.
Hannah is a smart, energetic, and a great pretend player. She is so fun to just watch play, when she is in the playing mode. She also tends to wine and cry at small things and want us to help her go potty when 2 hours earlier she did the whole thing all by herself, throwing a fit when we tell her she can do it. She is attached to her green and blue blankie and is upset when it needs to be washed. She loves to play with her babies, she has many as somehow all of the ones from when I was a kid plus the ones she has got out at the same time. She takes them to the pool to swim, to the store, feeds them, puts them to sleep, changes their diapers, and more.
She is still in the "Why" phase which can be exhausting. She loves to cuddle and snuggle and give hugs and kisses still which is awesome becuase sometimes Luke will let me kiss him but no hugs he says. I have to get Luke hugs when by carrying him up and down the stairs a lot of the time.
Hannah is wearing an eye patch to hopefully help her right eye get stronger as glasses are still not correcting it entirely and they are a really strong prescription. Luke's eyes seemed to have plateaued and neither of them cares when they have filthy glasses.
Kevin and I are hanging in there trying to get things done and do whats best for the kids. My desk is such a disaster and I just don't have the energy to clean it up and thankfully Hannah has gotten the vibe to stay away as she tends to color on anything that is paper. We are exhausted a lot of time time and have lists of things that are so long. But we find time to love on our kids and smile and laugh together. I could use a little more time to do all of these and I am hoping that after making some decisions about Luke's care and getting him off to school next week that there will be more time.
What are some of the decisions we need to make? Our current pulmonologist doesn't seem to be willing to work with CAMP on vent settings and we are taking this opportunity to find someone closer to home, we think. We were at the current pulmonolgist becuase we were going to do the pacemakers through them but now will do them through CAMP as we like their design better. We generally get along fine with the current pulmonologist and the nutritionist from their office and I actually agree on how I am feeding Luke. However, they are 4 hours away! and not near any of our parents. Then, do we decide to switch to a new vent? This depends on if we are going to try for pacemakers in the next year or less? Which depends on how thing are going with the new settings and what the final report from CAMP says in follow up and what they think? We would have to switch medical supply companies to get a different type of vent and if we do that I don't want to go through all the process with our current medical supply company to get some of the new equipment that we will need. Although I really like my ordering people and the respiratory therapist at our current company. An one piece of equipment is looking like it will be a fight to get it covered by insurance. And then there is school we are trying to get Luke to...and we are adding a vent to all the other things that are different about him... And it goes on and on...
I am tired, stressed, and have shut down a bit. I pray that the final report comes tomorrow and we can start really making some informed decisions. I feel like I haven't been there as much as I should for the kids. I have been on the phone or e-mailing about this a lot this past week and I just want to be done. Please pray for wisdom, discernment, patience, and sleep.
On a fun note, we took a spur of the moment trip to the UP wilderness to relax and destress a bit. I feel the most relaxed surrounded by God's creation and it was good. Although, I must say that it is not as relaxing with two small children who just didn't appreciate the beauty of waterfalls, brightly colored trees, and being able to see 3 great lakes in the matter of an hour as much as I wanted them to. I think they did have a good time and enjoyed doing something else for a day. For me even the drive was beautiful and it was nice to get away. I have been wanting to make that day trip for a long time as I missed the water and the UP, there is just something that draws me in about the UP.
So what are the kids up to these days?
Luke loves to play 100 Floors and Doors on his iPad while he is being fed. He still likes letters and playing around with them and spelling different words. He also likes having you draw things for him. He has been into having me tell him stories about subject he chooses lately instead of dictating stories to me. He still likes to read a lot and spends a lot of time babbling on his iPad. He likes to go outside for walks, although he would like to be doing the riding and not the walking.
Luke has doing okay with wearing the vent all day. Kevin is making him a new cart for it tomorrow. Right now it is on a scotter but he isn't great about pushing it around. He is walking really well by himself when we follow behind with the vent. And he does walk away from the vent at least once a day forgetting that he has to take it with him. We are hoping the cart will help him be able to get around more as he can walk with it.
Luke is looking more and more like a little boy every day. I think he is beautiful and I tell him. I am not sure what he thinks of that. I love the haircut Kevin gave him in prep for our time at CAMP and the sleep study and it does help make him more boy and less toddler. He has also been communicating more with his iPad and with more comments and not questions. Although, he sometimes refuses to communicate or lately when we don't have the iPad and I ask yes or no questions he will give me both answers. This very frustrating and I haven't figured out what will motivate him to stop doing it yet. But I have to remember that even under all that medical equipment he is still just a 5 year old boy. He can be sweet like honey and a stubborn and a mule but he is so lovable and he still lets me give him lots of kisses.
Hannah is a smart, energetic, and a great pretend player. She is so fun to just watch play, when she is in the playing mode. She also tends to wine and cry at small things and want us to help her go potty when 2 hours earlier she did the whole thing all by herself, throwing a fit when we tell her she can do it. She is attached to her green and blue blankie and is upset when it needs to be washed. She loves to play with her babies, she has many as somehow all of the ones from when I was a kid plus the ones she has got out at the same time. She takes them to the pool to swim, to the store, feeds them, puts them to sleep, changes their diapers, and more.
She is still in the "Why" phase which can be exhausting. She loves to cuddle and snuggle and give hugs and kisses still which is awesome becuase sometimes Luke will let me kiss him but no hugs he says. I have to get Luke hugs when by carrying him up and down the stairs a lot of the time.
Hannah is wearing an eye patch to hopefully help her right eye get stronger as glasses are still not correcting it entirely and they are a really strong prescription. Luke's eyes seemed to have plateaued and neither of them cares when they have filthy glasses.
Kevin and I are hanging in there trying to get things done and do whats best for the kids. My desk is such a disaster and I just don't have the energy to clean it up and thankfully Hannah has gotten the vibe to stay away as she tends to color on anything that is paper. We are exhausted a lot of time time and have lists of things that are so long. But we find time to love on our kids and smile and laugh together. I could use a little more time to do all of these and I am hoping that after making some decisions about Luke's care and getting him off to school next week that there will be more time.
Saturday, September 20, 2014
Life changing time at CAMP
So this past week we went to the Center for Autonomic Medicine in Pediatrics (CAMP) headed up by Dr. Debra Weese-Mayer. This team was awesome! These guys know their research and love data, my type of people.
In summary: Luke was at the edge of respiratory failure at his current breath rate (60-90 bpm) and CAMP got him off the edge and he is now on the ventilator 24/7 but breathing at normal rates (12-25 bpm) all the time!!! We are in the process of getting new monitors and a new ventilator and getting creative about how to get the vent to go with Luke as he is mobile and loves to explore.
At length:
CAMP specializes in CCHS and ROHHAD, two other hypoventilation syndromes. Luke has alveolar hypoventilation, neurological in origin just as his other issues are, but they still took him on and we are VERY grateful. When Luke arrived he was breathing really fast and the CAMP team thought he was at the edge of a cliff and could have diaphragmatic fatigue and shut down at any point. They also thought from some pupil studies that he was scared a lot becuase of this fast breathing and never really being able to slow down and catch up. On Tuesday they did studies that found that he does have an intact drive and responds to O2 and CO2 as a person should. There may be a little something off as his behavioral responses were not always clear but the physiological ones they were measuring were clear that his chemoresponsiveness was intact. His unclear behavioral responses were than when they tested him watching his show on the iPad and then no watching his show, he was didn't show nearly the behavioral responses to the tests (they tests distress you and your behavior should show that) when he was watching a show on the iPad as when he wasn't.
When Luke fell asleep off the vent he is still breathing but it is very dangerous. He is working so hard that he is not only pulling air in from his trach but also from his mouth and causes his mouth to move and is causing obstructions becuase the negative pressure he causes when pulling air in is so strong that it pulls his trachea closed. He breaths really fast and has a trach so it is not very noticeable but it is happening. Not good. Now he is on the vent 24/7 so I don't have to make sure I always have a vent incase he falls asleep becuase he will always have one on.
So basically his body wants to keep its O2 and CO2 levels at a good place and it will do anything to do that. In his case he was breathing REALLY fast. We are not sure why his body is unable to take big deep slow breaths on its own to keep his stats where they should be. There is just so much we don't know about the neuromuscular system and the effects of Moebius Syndrome. So we don't really know why his body can't keep his stats where they should be with normal breathing. We just know they can't and at CAMP they played with his vent settings a lot to figure out the best settings for him.
When they put him on the vent, maybe Monday afternoon (they had to get good data first to figure out a baseline and compare) and he was breathing something like 20 bpm it was amazing to me! He had not breathed that slow awake EVER! The implications of this could be huge. Think about what he could do and how he could develop if he isn't putting all of his energy into breathing just to survive. I am really excited to see what he will do in the next few weeks and months.
Luke's heart rate variability, which you want to be variable, got more and more variable and towards normal as the week progressed. Remember he started the week with no ventilation and breathing over 60 bpm and then on Monday afternoon he was put on the vent and started breathing a normal breath rate for the first time ever. The other things that was off and started becoming normal throughout the week was his pupils. They measure pupil size, constriction velocity, retraction velocity, and the amount of change. At the beginning of the week he has bigger than normal pupils that were slow to constrict and dilate. Each day they measurements normalized and that as well as the heart rate variability increases shows us that the ventilation is good for him. Yeah!!
They found that his regional blood flow to the front of his brain was good indicating that he has nice cerebral regulation so this means that O2 levels are being kept at good levels for neurocognative functions. Sometimes I felt that she didn't think he was normal cognitively as she kept telling us she was helping his brain and development in ways like he would become normal cognitively and we know he already is. I am hoping that this helps with the autistic issues we are seeing with socialization, sensory issues, and repetitive behaviors (although we found that 10% of Moebius people have repetitive behaviors with no autism at the last Moebius conference). I am also thinking that his gross and fine motor skills may take off as he is not spending so much energy breathing so his body can concentrate on developing other things.
The neurophsycologist fell in love with him and wrote a note to Dr. Weese-Mayer about how much she loved him. Parents are usually not welcome for testing as we tend to answer for our children so I was not present for his time with this doctor. However, Kevin stayed to help interpret Luke's signs and responses as he is good about only giving information when it is needed. ;) We don't have the results from this test back yet but will next week. I am very excited to see as she and Dr. Weese-Mayer thinks that she will be underestimating Luke based on the tests and what will happen now that he is getting support for his breathing all the time.
Luke's iron has been an issue for awhile and no one has given us any answers. We had them order an iron blood test when they were doing other blood work and there were some things that may need to be addressed by a hematologist. There are lots of things on my list and one is to talk to our primary care about it and a possible recommendation to a hematologist. However, maybe this will get better with better ventilation as well.
Luke did NOT like all the stickers and belts placed all over his chest, face, and head every morning and night and then taken off in the morning for a good bath. His skin is not looking good and he will be getting a good oiling up every day until he is healed.
Another good things about this is that Luke has some great physical endurance. He went on laps around the hospital in his walker, while the team followed behind with his vent and monitors on a cart and someone to record his readings as we went. He did a lot of exercise even when he was already tired and did great!!! This is awesome news and the tiredness he had before was most likely because he couldn't do all that physical activity while trying so hard to breathe.
The poor body didn't sleep very much Sunday night or Monday night for his sleep study. On Tuesday instead of messing with him a lot for his sleep study they just let him sleep which he did okay at. On Wednesday and Thursday nights they mainly just tweaked his settings and he slept better each night. They wanted to do more but becuase he didn't sleep well they just wanted to make sure he got some. They said he may not ever sleep well as in the literature with Moebius (and I know by talking to people with Moebius) that people with Moebius do not have good sleep patterns, mostly becuase the parts of the brain that are affected by Moebius are right near the sleep center of the brain. Dr. Weese-Mayer did say that when they got ROHHAD patients ventilated correctly and taken care of their sleep issues went away. I am hoping that happens with Luke as well.
Most people have Moebius plus, like Luke and not just a strait 6th and 7th cranial nerves not functioning. These nerves are in the brain stem and there is so much that goes on there that whatever caused the 6th and 7th cranial nerves to not word correctly usually hits more stuff as well. Everyone who has Moebius is a little different and there is a lot we just don't know.
One of their theories for Luke (and probably others) is that when his body is stressed becuase breathing is such hard work then the autonomic nervous system starts having problems and not functioning normally. The autonomic nervous system has a few levels and controls the functions of the body that we don't have to think about, i.e. breathing, heart functions, digestion. Luke's heart is looking better and I am hoping that his digestive system gets better and his poops will normalize as well by taking all the stress off of the body and supporting him with ventilation 24/7. We have been struggling with poops for awhile and things have been getting better but a little bit of stress usually throws it way off. I think this is the first long car ride and trip to the hospital that we have had without bad poops. Yeah!
So on to his vent settings now. He has different ones for awake and asleep and will have a CO2 monitor as well as an O2 monitor. He may even get different settings for awake active and awake calm. We changed his settings from a SIMV mode to assist control mode. On the SIMV mode it was supporting the breaths he was taking and making sure he was taking a certain amount. If he took a little breath this would count and delay the big supported breath the vent would give him as the minimum. CAMP found that when this big supported breath was delayed by his little, not very helpful, breaths that he would have uneven breathing and pauses in breath that were creating times where his oxygen saturation was dropping. This was not good at all. So on the assist control mode he gets his mandatory breaths but every breath he takes the vent will give him as a big supported breath. I may not be explaining this well but basically he will not be allowed to have pauses between good breaths that can cause his oxygen to lower. They also changed his inhalation times and some of the other settings as to how they are giving him breaths. He will be getting 0.5 L of oxygen at night. They said this is becuase when ventilation him without oxygen at night they could keep either his CO2 or O2 levels in the right range and so they got his CO2 leves right and then are giving him oxygen to keep those up.
He also got a new trach. He does not have a flex tend, which extended the trach beyond his chin, but has one that is flush with his neck and also it is cuffed. The cuff is inflated just a bit at night to prevent air leaks. They tried inflating it during the day but he didn't like it and was doing find without it inflated. Uninflated will also allow him to make noise around the cuff. They said his trach could be bigger but right now they want him to be able to make noise.
They did find right ventricular hypertrophy which means that the wall of his right ventricular in his heart was thicker. This is due to stress and a episode of low oxygen where the heart and to push extra hard to get enough blood and oxygen into the lung. Dr. Weese-Mayer said it is reversible and she expects it to reverse with this good ventilation where there will be no hypoxic (low oxygen) episodes.
So, we originally went to CAMP thinking we would be getting diaphragm pacemakers to replace Luke's vent. We decided against putting pacemakers in at this time for a number of reasons. First, the right ventricular hypertrophy can be dangerous when going into surgery. Second, Luke can have a lot of secretions and the negative pressure of the pacemaker is more than what he currently has and there is a high risk that he could aspirate his secretions. Hopefully with better ventilation and time his secretions will be less and this risk will diminish. Third, Luke does have intact drive to breathe and so he has something to lose if there is nerve damage or other complications to the process. People with things like CCHS do not have a drive to breathe in many ways and thus are going to be on ventilation for the rest of their lives for sure. Luke has that drive and since Moebius gets better with time and with proper ventilation he may get better and grow out of the need for ventilation. And fourth, these new ventilation settings are a lot different. We want to see what he will do with this and what and how much things will improve for him with these new settings. So we are not saying no forever to pacemakers, just no for right now. Luke's nerves and diaphragm respond in the correct ways that the pacemakers are possible. We did talk to the surgeon and would be ready to go when the time is right.
With all these changes we are also looking to up the amount of nursing we get and have nurses during the day to help me transport Luke and his equipment. I am very unsure about this and having more people in the house all the time but I said I would give it an honest try and have an open mind.
My mind is going a million miles an hour trying to figure out how to keep Luke and our family active with the added equipment and tubing connections. As Dr.Weese-Mayer says, take it one day at a time and just try stuff. She thinks I am pessimistic and I just wanted to know how to function with the vent and a mobile child as I will have to do it right away. We are still collecting info on what type of cart to make for Luke to be able to push his own equipment around. Kevin is a great designer and builder and I think has a lot of ideas already. My mom has sent us links to pictures of other kids things as well.
This next week will be busy getting the right equipment, figuring out how to move things around with Luke, and figuring out how to do school. We can do it, I of course want to know all the answer right now. I am learning patience again. I must say, even just getting to Kevin's parents house and moving Luke around here was not as hard as I thought.
We did take a walk around Chicago and ride some glass elevators with Luke after we checked out. He loved being out and looking at all the really tall buildings. He didn't really care much about the glass part of the elevator but liked the numbers. Us adults enjoyed the glass elevator ride though. Luke did like being close to glass railings and looking down 10+ floors though. :)
There were a couple of times that I got to walk around Chicago. I walked Michigan Ave. and many streets around there. It was very nice to be able to see Lake Michigan and walk part of the lake walk one day as I feel much more comfortable when I am close to a Great Lake or a large body of water.
And you may be wondering, "Where is Hannah?" She has been with grandma and grandpa Wright living it up in Grand Rapids. She has been to the museum, planetarium, Meijer Gardens, the pool, shopping, and more. She has been having lots of fun and we will get to see her beautiful face and get big hugs tomorrow.
We will be getting a full written report next week from Dr. Weese-Mayer and probably more details and things that I have forgotten already in trying to process it all. Luke is just waking up from the best night sleep that he has had in quite awhile!
In summary: Luke was at the edge of respiratory failure at his current breath rate (60-90 bpm) and CAMP got him off the edge and he is now on the ventilator 24/7 but breathing at normal rates (12-25 bpm) all the time!!! We are in the process of getting new monitors and a new ventilator and getting creative about how to get the vent to go with Luke as he is mobile and loves to explore.
At length:
CAMP specializes in CCHS and ROHHAD, two other hypoventilation syndromes. Luke has alveolar hypoventilation, neurological in origin just as his other issues are, but they still took him on and we are VERY grateful. When Luke arrived he was breathing really fast and the CAMP team thought he was at the edge of a cliff and could have diaphragmatic fatigue and shut down at any point. They also thought from some pupil studies that he was scared a lot becuase of this fast breathing and never really being able to slow down and catch up. On Tuesday they did studies that found that he does have an intact drive and responds to O2 and CO2 as a person should. There may be a little something off as his behavioral responses were not always clear but the physiological ones they were measuring were clear that his chemoresponsiveness was intact. His unclear behavioral responses were than when they tested him watching his show on the iPad and then no watching his show, he was didn't show nearly the behavioral responses to the tests (they tests distress you and your behavior should show that) when he was watching a show on the iPad as when he wasn't.
When Luke fell asleep off the vent he is still breathing but it is very dangerous. He is working so hard that he is not only pulling air in from his trach but also from his mouth and causes his mouth to move and is causing obstructions becuase the negative pressure he causes when pulling air in is so strong that it pulls his trachea closed. He breaths really fast and has a trach so it is not very noticeable but it is happening. Not good. Now he is on the vent 24/7 so I don't have to make sure I always have a vent incase he falls asleep becuase he will always have one on.
So basically his body wants to keep its O2 and CO2 levels at a good place and it will do anything to do that. In his case he was breathing REALLY fast. We are not sure why his body is unable to take big deep slow breaths on its own to keep his stats where they should be. There is just so much we don't know about the neuromuscular system and the effects of Moebius Syndrome. So we don't really know why his body can't keep his stats where they should be with normal breathing. We just know they can't and at CAMP they played with his vent settings a lot to figure out the best settings for him.
When they put him on the vent, maybe Monday afternoon (they had to get good data first to figure out a baseline and compare) and he was breathing something like 20 bpm it was amazing to me! He had not breathed that slow awake EVER! The implications of this could be huge. Think about what he could do and how he could develop if he isn't putting all of his energy into breathing just to survive. I am really excited to see what he will do in the next few weeks and months.
Luke's heart rate variability, which you want to be variable, got more and more variable and towards normal as the week progressed. Remember he started the week with no ventilation and breathing over 60 bpm and then on Monday afternoon he was put on the vent and started breathing a normal breath rate for the first time ever. The other things that was off and started becoming normal throughout the week was his pupils. They measure pupil size, constriction velocity, retraction velocity, and the amount of change. At the beginning of the week he has bigger than normal pupils that were slow to constrict and dilate. Each day they measurements normalized and that as well as the heart rate variability increases shows us that the ventilation is good for him. Yeah!!
They found that his regional blood flow to the front of his brain was good indicating that he has nice cerebral regulation so this means that O2 levels are being kept at good levels for neurocognative functions. Sometimes I felt that she didn't think he was normal cognitively as she kept telling us she was helping his brain and development in ways like he would become normal cognitively and we know he already is. I am hoping that this helps with the autistic issues we are seeing with socialization, sensory issues, and repetitive behaviors (although we found that 10% of Moebius people have repetitive behaviors with no autism at the last Moebius conference). I am also thinking that his gross and fine motor skills may take off as he is not spending so much energy breathing so his body can concentrate on developing other things.
The neurophsycologist fell in love with him and wrote a note to Dr. Weese-Mayer about how much she loved him. Parents are usually not welcome for testing as we tend to answer for our children so I was not present for his time with this doctor. However, Kevin stayed to help interpret Luke's signs and responses as he is good about only giving information when it is needed. ;) We don't have the results from this test back yet but will next week. I am very excited to see as she and Dr. Weese-Mayer thinks that she will be underestimating Luke based on the tests and what will happen now that he is getting support for his breathing all the time.
Luke's iron has been an issue for awhile and no one has given us any answers. We had them order an iron blood test when they were doing other blood work and there were some things that may need to be addressed by a hematologist. There are lots of things on my list and one is to talk to our primary care about it and a possible recommendation to a hematologist. However, maybe this will get better with better ventilation as well.
Luke did NOT like all the stickers and belts placed all over his chest, face, and head every morning and night and then taken off in the morning for a good bath. His skin is not looking good and he will be getting a good oiling up every day until he is healed.
Another good things about this is that Luke has some great physical endurance. He went on laps around the hospital in his walker, while the team followed behind with his vent and monitors on a cart and someone to record his readings as we went. He did a lot of exercise even when he was already tired and did great!!! This is awesome news and the tiredness he had before was most likely because he couldn't do all that physical activity while trying so hard to breathe.
The poor body didn't sleep very much Sunday night or Monday night for his sleep study. On Tuesday instead of messing with him a lot for his sleep study they just let him sleep which he did okay at. On Wednesday and Thursday nights they mainly just tweaked his settings and he slept better each night. They wanted to do more but becuase he didn't sleep well they just wanted to make sure he got some. They said he may not ever sleep well as in the literature with Moebius (and I know by talking to people with Moebius) that people with Moebius do not have good sleep patterns, mostly becuase the parts of the brain that are affected by Moebius are right near the sleep center of the brain. Dr. Weese-Mayer did say that when they got ROHHAD patients ventilated correctly and taken care of their sleep issues went away. I am hoping that happens with Luke as well.
Most people have Moebius plus, like Luke and not just a strait 6th and 7th cranial nerves not functioning. These nerves are in the brain stem and there is so much that goes on there that whatever caused the 6th and 7th cranial nerves to not word correctly usually hits more stuff as well. Everyone who has Moebius is a little different and there is a lot we just don't know.
One of their theories for Luke (and probably others) is that when his body is stressed becuase breathing is such hard work then the autonomic nervous system starts having problems and not functioning normally. The autonomic nervous system has a few levels and controls the functions of the body that we don't have to think about, i.e. breathing, heart functions, digestion. Luke's heart is looking better and I am hoping that his digestive system gets better and his poops will normalize as well by taking all the stress off of the body and supporting him with ventilation 24/7. We have been struggling with poops for awhile and things have been getting better but a little bit of stress usually throws it way off. I think this is the first long car ride and trip to the hospital that we have had without bad poops. Yeah!
So on to his vent settings now. He has different ones for awake and asleep and will have a CO2 monitor as well as an O2 monitor. He may even get different settings for awake active and awake calm. We changed his settings from a SIMV mode to assist control mode. On the SIMV mode it was supporting the breaths he was taking and making sure he was taking a certain amount. If he took a little breath this would count and delay the big supported breath the vent would give him as the minimum. CAMP found that when this big supported breath was delayed by his little, not very helpful, breaths that he would have uneven breathing and pauses in breath that were creating times where his oxygen saturation was dropping. This was not good at all. So on the assist control mode he gets his mandatory breaths but every breath he takes the vent will give him as a big supported breath. I may not be explaining this well but basically he will not be allowed to have pauses between good breaths that can cause his oxygen to lower. They also changed his inhalation times and some of the other settings as to how they are giving him breaths. He will be getting 0.5 L of oxygen at night. They said this is becuase when ventilation him without oxygen at night they could keep either his CO2 or O2 levels in the right range and so they got his CO2 leves right and then are giving him oxygen to keep those up.
He also got a new trach. He does not have a flex tend, which extended the trach beyond his chin, but has one that is flush with his neck and also it is cuffed. The cuff is inflated just a bit at night to prevent air leaks. They tried inflating it during the day but he didn't like it and was doing find without it inflated. Uninflated will also allow him to make noise around the cuff. They said his trach could be bigger but right now they want him to be able to make noise.
They did find right ventricular hypertrophy which means that the wall of his right ventricular in his heart was thicker. This is due to stress and a episode of low oxygen where the heart and to push extra hard to get enough blood and oxygen into the lung. Dr. Weese-Mayer said it is reversible and she expects it to reverse with this good ventilation where there will be no hypoxic (low oxygen) episodes.
So, we originally went to CAMP thinking we would be getting diaphragm pacemakers to replace Luke's vent. We decided against putting pacemakers in at this time for a number of reasons. First, the right ventricular hypertrophy can be dangerous when going into surgery. Second, Luke can have a lot of secretions and the negative pressure of the pacemaker is more than what he currently has and there is a high risk that he could aspirate his secretions. Hopefully with better ventilation and time his secretions will be less and this risk will diminish. Third, Luke does have intact drive to breathe and so he has something to lose if there is nerve damage or other complications to the process. People with things like CCHS do not have a drive to breathe in many ways and thus are going to be on ventilation for the rest of their lives for sure. Luke has that drive and since Moebius gets better with time and with proper ventilation he may get better and grow out of the need for ventilation. And fourth, these new ventilation settings are a lot different. We want to see what he will do with this and what and how much things will improve for him with these new settings. So we are not saying no forever to pacemakers, just no for right now. Luke's nerves and diaphragm respond in the correct ways that the pacemakers are possible. We did talk to the surgeon and would be ready to go when the time is right.
With all these changes we are also looking to up the amount of nursing we get and have nurses during the day to help me transport Luke and his equipment. I am very unsure about this and having more people in the house all the time but I said I would give it an honest try and have an open mind.
My mind is going a million miles an hour trying to figure out how to keep Luke and our family active with the added equipment and tubing connections. As Dr.Weese-Mayer says, take it one day at a time and just try stuff. She thinks I am pessimistic and I just wanted to know how to function with the vent and a mobile child as I will have to do it right away. We are still collecting info on what type of cart to make for Luke to be able to push his own equipment around. Kevin is a great designer and builder and I think has a lot of ideas already. My mom has sent us links to pictures of other kids things as well.
This next week will be busy getting the right equipment, figuring out how to move things around with Luke, and figuring out how to do school. We can do it, I of course want to know all the answer right now. I am learning patience again. I must say, even just getting to Kevin's parents house and moving Luke around here was not as hard as I thought.
We did take a walk around Chicago and ride some glass elevators with Luke after we checked out. He loved being out and looking at all the really tall buildings. He didn't really care much about the glass part of the elevator but liked the numbers. Us adults enjoyed the glass elevator ride though. Luke did like being close to glass railings and looking down 10+ floors though. :)
There were a couple of times that I got to walk around Chicago. I walked Michigan Ave. and many streets around there. It was very nice to be able to see Lake Michigan and walk part of the lake walk one day as I feel much more comfortable when I am close to a Great Lake or a large body of water.
And you may be wondering, "Where is Hannah?" She has been with grandma and grandpa Wright living it up in Grand Rapids. She has been to the museum, planetarium, Meijer Gardens, the pool, shopping, and more. She has been having lots of fun and we will get to see her beautiful face and get big hugs tomorrow.
We will be getting a full written report next week from Dr. Weese-Mayer and probably more details and things that I have forgotten already in trying to process it all. Luke is just waking up from the best night sleep that he has had in quite awhile!
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