New Ventilator! Trilogy 100 vs. LTV 1150

Luke got a new vent last week, finally!!!  We are very excited about it and think it is great.  So far Luke is doing very well on the new vent.

So here's the deal, Luke has been on an LTV1150 since he came out of the hospital at 3 1/2 months old.  We had not serious thoughts of changing vents until we were at a special clinic for hypoventilaton and they suggested changing to the Trilogy.  When we talked to our regular pulmonolgoist they were all for it but said we needed to wait till the "sick" season was over.  To get a new vent we have to be admitted to the hospital for 1-2 nights to make sure Luke does well on the new vent.  Every vent ventilates a little bit different so you have to make sure that whoever is changing can tolerate the new ventilation.  Since there is a ventilator involved you have to be admitted to the pediatric intensive care unit (PICU).  From late fall through early spring the PICU can be pretty busy and thus since we would be there voluntarily and no sick while changing a vent we would get kicked out if they needed a bed.  So to decrease the likelihood of making all the plans for a hospital visit 3 hours from home we waited till May.

You may be asking, "Why would you want a new vent?  What are the benefits?"  I created a video to show you why I love Luke's new Trilogy 100 ventilator.  It shows many of the differences between the two vents.

Please feel free to ask questions and share your Trilogy and LTV stories in the comments below!

Enjoy!

We have a kindergartener!

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. :)

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.

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!

Walking, walking and my energetic one

Luke has been taking steps all byhimself!!!  He does not walk around by himself yet but is walking really well with help.  I only have to hold one of his hands.  He has his good and bad days.  Today was not a great day with walking, he fell a bit and seemed to just want to crawl.  He is doing awesome in his walker at church.  So well that he is now confident enough to run over people's feet so we have to watch him a little closer.  Saturday Luke even tried to take a step towards me, although I happened to only be a step or two away.  This is the first time I have seen him do that.  All his previous walking independently has been done at our request.

Luke in general is doing awesome.  He is exploring more.  We were at my grandparent's house and he was everywhere.  He is exploring more at church.  Today he walked his walker up to the raised area, crawled up the stairs and sat at the top looking at everything.  We have screens on both sides of the front for everyone to see and it was really neat to watch him discover those and discover that they were showing the same thing :)

Hannah likes to put her hands over her face and ask "Where's Hannah?" and then uncovers and says "Boo" or just laughs.  When we were at the psychologist getting evaluated for Autism the physiologist asked Luke to play peek a boo and he was interested in it so so.  He has played it with us in the past but not often but I think after that visit and seeing how much attention Hannah gets he decided it was a good game to play.  He plays it now and will even point at the cloth over his eyes to point out that he is hiding.  Hannah enjoys Luke hiding though which is really fun.

Luke is on the Autism Disorder Spectrum but not glaringly.  We have played around with this idea but not done anything official about it until now.  And I must say I had a hard time with the actual diagnosis.  Luke is still Luke the same little boy before and after and we had considered this and knew he at least had a Sensory Processing Disorder.  But it took me a day or two to get out of my sadness about this.  I don't want Luke to have more challenges in life.  I wish he played pretend with Hannah and communicated with me better.  We are working on things with him through school, therapists, and at home.  It is hard work and to know that it may always be hard work was a little overwhelming to me those first few days.  But we keep on and Luke is just so cute.  He has been giving the greatest hugs lately and letting me give him kisses all over his face.  He let me put on his essential oil mix and do a very tickley Vita Flex technique to his feet when I do it.  He is interacting more with us and I am looking towards the progress now.  He is doing awesome in oral therapy and moving right up the difficulty levels with his jaw exercises.  I can't wait till he gets to preschool this year as he is going to be in a mainstream classroom but with his special education teachers as well as the mainstream ones so it will be a good transition.  I look forward to all the interaction with many kids who don't have social issues.  He is doing well at playgroup still this summer and we have started going to reading time at the library.  Oh and did I mention that the hugs and kisses are great!

Hannah is our commentator on her own and our lives.  She loves Lukey and wants him to wake up from his nap to come play and always likes to be where he is and know what he is doing.  They are sharing okay.  They often share things that the other one does not want at all or at least not at the time, but the effort is there.

Hannah is just a happy go luck little girl most of time.  She loves to be silly and will do things and then say "silly Hannah" or silly whoever she thinks was silly.  She runs and prances around the house.  She is just getting more into books.  As a true child of this generation she is very good at working an iPod/iPhone ect... and can play music, look at "Hannah pictures", take pictures, play games, and get into the programs you would rather she not be into.  She likes playing with dolls and pretend food (and real food too).  Her eating habits leave much to be desired but it is all a phase, isn't it?  She is a happy healthy soon to be 2 year old.

Hannah has a very good vocabulary and talks a lot, at home.  She is breaking out of her shell a little more with other people and when we are out and about, which is nice that other people can hear the fun things she says.  She has her whinny moments and is not a morning person.  However, she seems to be the only one in our family that can tolerate heat and she didn't seem to mind the heat wave a couple of weeks ago too much.  The rest of us were miserable.  She is cute and cuddly and a lot of fun.

My parents have moved 3 hours away which means that we have lost our only other caretakers for Luke.  I haven't really felt this too much but it has only been a month or so.  I do miss them as I love spending time with them and they are busy and we don't travel well due to Luke still needing someone to be awake with him 24/7.

In other news my grandmother is failing.  She has lived a long, good life.  We went to see her this past Saturday and it was really nice to enjoy her company, perhaps for the last time :(  Luke practiced walking for grandma and Hannah flitted around talking and being her little joyful self.  While grandma was napping my grandpa played with the kids and it was awesome to watch the interactions.  The love that flowed both ways was really special to watch.  I am glad we made the trip and the memories.

Life goes on as I try to keep the house somewhat clean, play enough with the kids, and keep everyone fed good food.  I am working to try to work out more and was successful for a week or two.  The heat that came with allergies or colds or something not fun lead to lack of sleep and getting off track.  I do walk more with the kids now that it is summer which is nice.  We are still trying to sell the house and move into town where walking is much easier and just out the door.  My goal is to have prayer/devotional time and workout time every morning.  I am trying to go to bed earlier since most nights I am not productive but I have been lazy and have been reading too much (I think I am addicted to books, I have had to set some hard boundaries for myself that I am still working to stay in).

Another goal is also to have good blocks of focused playtime with the kids everyday.  This sounds easy but when there are a thousand things to do and places to go sometimes we don't get that on the floor zooming cars around time that I need just as much as they do.  I am doing fairly well at this and I can see positive changes in the kids.  They are less clingy, they are more apt to play with toys by themselves nicely (which is huge for Luke and a break for me with Hannah), have a better attitude throughout the day, ect...

So I sat down to write a bit and ended up with all this.  That's another goal to reach for, blogging once a week. :)

Hope for a possibly trach free Luke

It seems I update only when I have nights with no nurses.  Probably it is the only time I feel I can squeeze it in.  I should probably be working on my meal plan and shopping list right now but there are still hours to do that (more about that later).

So wow, it has been awhile.  I though at one point I would do weekly updates, even to just get myself to record what is going on in my kids lives once in awhile.  So maybe this could be the start.

Luke:
On March 6-7 we went down to Mott's Children's Hospital to get a sleep study with their team and to see their Pulmonology team about their thoughts on Luke and the possibility of getting a phrenic nerve pacemaker.  What is that you ask?  Basically it is a pacemaker that stimulates the diaphragm and thus make the lungs inhale and exhale.  It acts as a ventilator internally.  This would be awesome for us as the vent and hopefully his trach would go away.

So how did it go.   Well we got to a room and it was huge!!!!  This was awesome as usually we don't have enough room for our stuff and to move.  Since we would need to be in the room with Luke this was great.  Luke explored by walking around his crib (yep we had the rails way up) until we got him tied down with all the electrodes and things.  He fought getting it all on in true Luke, what I am supposed to have low muscle tone but can push a grown man away, fashion.  We finally got him hooked up and it took him forever to fall asleep.  And then he was leaking which we had been worrying about (and found out that he was just fine with the leak) and would not keep his CO2 monitor on.  He was so greasy from the stuff they used to put the electrodes on that we had a tough time getting the stickers to stick.  We finally cleaned off the largest portion of his forhead we could and hoped for the best.  Shortly after this my shift was over and I got to get some sleep.

Luke started with no oxygen, no vent, nothing.  His CO2 started climbing and between 45 and 50 we put him on the vent.  I was hoping that the CO2 climb was due to his maddness about getting the electrodes on and that he would fall asleep and it would go down but it did not :(  He was on the vent but no oxygen for the rest of the night.  I got back to a ready for the day Luke and we pack muled Luke's stuff (with a wagon too) back to our hotel room so that I could get a bit more sleep before our first appointment.

We saw the jaw surgery resident who said things were looking good.  We saw the dentist who said things were looking good and she was able to get in with much much less fuss that last time.  Good job Luke and dentist lady!

We then went to pulmonolgy.  I was pretty nervous.  Luke has had the same pulmonlogy team from his 2nd month of life.  The doctor and I have learned about each other and were doing well.  I didn't love them but had no qualms with them either (expect for the nutritionist which I am sure I have mentioned before).  I was nervous that they would say he wasn't a pacemaker candidate, that they would say he needed a vent for life. Yeah I know kids amaze us but hearing it still wouldn't be fun.  And then there was a new nutritionist to talk to and most likely get in a heated conversation with due to our "unconventional", we like to feed our kid real food, ways.  And I had not had that much sleep.

However, things went great!!!!!!!!!!!!

In these visits Kevin handles Luke and I handle the talking and taking notes.  I forget things if I have to handle Luke too.  And Luke needs a lot of watching as he is bored and nervous and on an exam table that he likes to lean and look over the edge but doesn't get that he can fall.

The pulmonologist came with with the neurologist that read and analyzed the sleep study.  Yes I said that right the neurologist came too!  The pulmonlogist gave us the results (the neurologist seemed the quiet like to be analyzing data on her own type) and they were positive.  Luke is a candidate for the pacemaker.  They would like to wait a year to do it so that his airway gets bigger and stronger.  The pacemaker stimulates the diaphram and the pressure that it creates as it pulls down can cause the trachea to suck in and become to small for sufficient air to go through.  This would make it necessary for Luke to always have a trach as a trach would hold the airway open.  This was a shock to me and I hope and pray that this will not happen to Luke.  As much as I didn't want to wait a year, I tell myself that waiting a year will help Luke get the trach out.  I am not thinking much about it never coming out.  When I am having a rough day with Luke I think about him being trach free in 2 years.  Yes just 2 years!!!

They confirmed Luke has hypoventilation and central sleep apnea.  They said it is unlikely that he has congentital central hypoventilation syndrome (CCHS) because of how his hypoventilation presented.  Luke's hypoventilation is not severe enough and his CO2 is lower than in CCHS.  Also CCHS patients don't respond like Luke did to interventions.  However, either way it doesn't change the management of Luke's breathing.

Then the nutritionist came in and I had my paper with Luke's diet ready to give her.  I was pretty nervous and I think there was tension as Kevin knew this could be very frustrating to me and I knew it as well.  She asked what Luke ate and I handed her the paper and told her he eats a blenderized diet and was waiting for the backlash.  And then she said that it was great that I was doing a blenderized diet and they were working to get more of their patients on them as there are benefits to reflux and all sorts of things.  I am glad I was sitting down.  Wow!  Awesome!  She asked if I wanted her to run the numbers of my food, not demanded, and I said sure because they hadn't been run in awhile.  I even e-mailed her the info I had on blenderized diets and our experience and some links that she could pass on to others.  Awesome visit!

So all in all I am very happy to be with the team at UofM.  We are still pursing getting an opinion from a hypoventilation expert in Chicago.  They don't have an opening till February.   

So how is Luke otherwise.  He still likes school.  There have been changes and some increased loudness with some other kids that have caused Luke to be more agitated and upset at school but he still likes to go.  He is progressing well.  He is walking with his walker and with us holding his hands better and I am hoping that he will walk by himself this year.  He is also been working on standing up by himself and has been doing it for up to 10 seconds well.  He loves to get the praise of others when he does.

Luke still is enthralled with books and will spend a lot of time reading.  As for toys he probably likes the cars the best.  The parking garage, road mat and cars are his favorite toys to play with.  He does still play with blocks and likes to play with all the furniture in the dollhouse.  He is noticing little spots or marks or pieces of dust on the floor, wall, anything.  So I guess his eyes are doing okay.  We pulled up to the library the other day and I was just going to drop some books off and take the kids to the park.  Oh no, we had to go in.  Luke was signing library as soon as the building came in view and was pointing to the building.  I asked if he wanted to go in or to the park and he wanted to go into the library.  So did Hannah as she likes to play with the toys they have.

Luke has an awesome new wheelchair. We are really excited for it as it sits him up nice and makes him not look so much a baby in a stroller.  He has the ability to move the wheels himself and had moved them a couple of times but is happy to have you push him.  Getting him into the wheelchair for fitting was not a good experience.  It was a hard 5 minutes as he was bent on pulling his trach out to tell us how unhappy he was.  I was almost in tears.  I realized afterwards that he needed to be introduced in the right situation.  We tried to put him in it when he wouldn't have been put in a stroller.  It was at the end of the school day and he usually walks with his walker to the door.  Also I was there and a new person, the guy who ordered and fit the chair for us.  So a couple of days later when we were going for a walk I left Hannah in her seat and took him out and put him in it.  There were some tense moments and him trying to pull the trach but he settled down after a couple of minutes.  It was a beautiful day and with Hannah on my back we went into the library and then for a walk.  The next time I put him in was when were getting out for some air on our trip to Chicago for a wedding and he didn't fuss a bit.  I guess I just need to get smarter about how I introduce things.  I would probably be unhappy too if someone came and interrupted my schedule to put me in a new kind of chair and someone I didn't know came to look at my feet and my legs and how they fit into it.  Even if the wheeles had cool lights on them.  Which they don't anymore as we had to get bigger wheels because I am not one to stay on pavement with Luke.

Luke is also getting more adventurous outside.  The other day he was crawling in the grass which he wouldn't do last summer. And our grass is wet and can be picky.  He was all over and was going down slopes both crawling and scooting down on his but.  He was a typical dirty boy when he was done.  I pointed out a small spider on the shed wall and he was tapping behind it to make it move.  He was pointing out stray garbage in the yard and crawling over and pointing right to it so that I would clean it up.  He crawled over to investigate a tree and got stuck as it was near a slope and asked for help to get out.  He was the explorer of the day.


Hannah:
Wow Hannah is a bundle of energy.  She is running, climbing stairs and other things, she can navigate an iPhone, likes to eat, likes to help me clean, likes to help dad cook, and does not like clothes.  Her favorite thing to do is color and write with crayons, markers, pens, whatever she can get a hold of.  And she needs some direction about what is appropriate to write on.

Hannah loves to go outside.  We got her some rain boots that are really cute and she wore them for a couple of days strait (except for sleeping).  Our backyard is a swamp and so there were plenty of puddles to splash in.  She loves to swing and for as much running as she does in general she will spend most of her time at the park in a swing.

She likes tools and was all involved in "helping" Kevin take screws out of wood to burn as he was cleaning out the shed.  She is interested in everything.  One of her other favorite things is to look at "Hannah pictures" on our iPhones.  If we have them out she wants to see pictures and videos.  She has her own iPod, my old one, but it isn't compatible with the videos I take on my phone so I can't transfer them.

She is not fond of clothes and especially dresses.  She looks adorable but they go on with a fight and this morning she was trying to pull one off her before church.  She is starting to pick out and put on her own clothes although this usually end up with some interesting outfits worn in some interesting ways.  Right now I really just make her put clothes on when we go out.  She has even been taking her pjs off.  Thankfully she likes to keep her diapers on.  She is becoming interested in the potty and likes to tell you when she farted.

She sleeps like a dream though.  I sometimes take her sleep for granted and I really shouldn't.  Days with her can be rough.  She is whiny as she knows what she wants, when she wants it.  She does not understand that changing shoes every five minutes and getting this and that for her will make it take longer to get out the door which causes more distress as anytime she sees that we are going out she wants her coat on (in her memory she has not know life without a coat) and to go out NOW.

Thankfully she does talk well (and a lot) so that even if you can't give it to her you usually know what she wants.  The is quite the commentator of everything going on especially what is going on with Luke.  She is working on figuring out how to feed him herself, much to my dismay as it creates quite a mess.

But wow is she cute.  And she can be very cuddly and sweet.  She is a typical toddler and we love her.

Me:
So what am I up to, besides kids?
I am still a Thirty-One consultant and I really like having all the great bags around to use.  It makes life with Luke and kids in general easier.

I recently discovered Norwex, a company that sells microfiber cloths that you use to clean.  You can clean with just the cloth and water (I tested it with raw chicken and yes its true) which I think is very "green" and awesome as it is easier.  We are using them a lot and I can't wait till I get the stuff from the party I had.  I like them so much I am thinking about becoming a consultant for Norwex too (if you are interested in it let me know ;) as I love to talk about what I love).

I have been thinking about essential oils for awhile now but have been overwhelmed at all the knowledge and info there is out there but have finally decided to take the plunge.  We have been using a few that my aunt sent me.  Thanks a bunch Aunt Sue!  But I have dove in with Young Living and am getting my first set this week.  I have been reading up on what they can do and their references in the Bible.  Lately I have been using Lavender to help Luke calm down and sleep (I am waiting for my Peace and Calm) and it has cut down his fighting sleep time at least in half and it is helping get him back to sleep in the night as well.  Yeah!!  Another Moebius mom has used them with her trached, and possibly some vent time, son and had great results keeping him healthy this winter.  I can't wait for Theives and what it can do for my peace of mind with germs and Luke.  I have used some Theives products (from my awesome aunt) and loved them and just feel better all around when I smell them but haven't used them in ways yet that my scientific mind can quantify.

Oh and I am also working on keeping our family (other than Luke who eats great, one plus about eating by g-tube, you can eat all the healthy stuff without tasting it and you don't miss the sweets) eating healthier.  And also having meals planned so that there isn't the "what's for dinner" dance and we end up eating something soso.  It is taking a bit of work.  I haven't got a system down yet and I like to plan for a month so that I can get as much as possible on my 10% off day at my local co-op natural and organic foods store (The Grain Train).  I have finally decided to dive full force into Kevin's wiki he made for us to keep all our recipes that we keep a copy on dropbox.  I am using Ziplist for my grocery list as it organizes things by category.  There are others out there and I don't keep my recipes on there because for some reason I don't like my recipes on someone elses' server in their format..  I know I am weird, I have tried it and it is just not for me.  Some of the other apps like Evernote and Springpad and Cozi are just slow and I am not one that likes to wait on an on-line based app.  I have recently heard about another order by mail co-op group that I am going to check out as well.

I can't wait for my farm share, not in season till the middle of June, as I will have fresh veggies all the time that I won't have to go to the store for.  We have found a new closer to home farm, Bluestem Farm, that I love.  They have meat shares and I love their farming practices.  They just started in the area and have a little one too.

We are still trying to sell our house.  I have high hopes that it will sell this spring.  I would love to be moved by the time my parents move July 1st and with God's help it can happen.  I am confident that he has a plan for us I just don't want to wait any longer for it.

Okay so that was a lot and here are the pictures that I am sure you were waiting for...
(there are more but my computer won't upload at this time)

An update, finally!

So I haven't posted a real update on Luke and our family in quite a while.  So here it goes...

Luke continues to thrive at school and at home.  A couple of weeks ago, around the time of his birthday, he just started doing things, good things developmentally, behaviorally that he hadn't before.  He started exploring the house.  He has been mobile but we didn't have to baby proof the house till Hannah got mobile because Luke just wasn't interested in getting into all the cupboards or what was down the hall there.  Well now he is.  He has been in cupboards, will go exploring under the dinning room table, play with the baby gate, crawl into his own room to play, go up the stairs by himself (we have to really watch him and Hannah now), ect...  It has been awesome!  Plus he has been really working, I mean like 20 minutes at a time, at standing up by himself.  He is doing a pretty good job working at it too.  We are pretty excited about this, obviously.

In December Hannah started to want to hug Luke and at first Luke didn't know what to do with her.  Luke has just learned to hug with his arms and not just put his head up against the person for a hug and a kiss.  Well it didn't take more than a day for Luke to go from head butting her to hug back to hugging her with his arms.  Yesterday, Hannah came up and said "hug" to Luke but he didn't really respond so she moved away. Just as she was moving away he put his arms out to hug her.  I got her back and they hugged.  Very cute.

 

Health wise Luke struggled with an infection in his post site from his jaw surgery that just wouldn't go away.  We called and got a January 18th surgery date which fortunately got moved up to January 4th.  We found out on Wednesday and had surgery on Friday, it was pretty fast but well worth the running around to make everything work.  We decided to admit Luke overnight since he hasn't had many surgeries and it took him over 24 hours to really come out of his funk from the first jaw surgery.  Normally this is an outpatient surgery.  The bonus was that we would be seeing a new pulmonologist while in the hospital.  Mott's Children's Hospital deals with quite a few patients with hypoventilation, which Luke has, and they are getting a pacemaker program, which we want Luke to have (a diaphram pacemaker that would work in place of his vent).  The pulmonlogist ended up seeing us while in pre-op which made pre-op even more of a circus and Luke even more upset but he got through it and his trach stayed in, thank you God!  After surgery Luke just wanted to to go, well before surgery Luke wanted to go.  However, it got so bad that evening and night that I was in tears on the phone with Kevin (who was trying to get some sleep before he was relieving me).  He just kept looking at me through the crib bars with those eyes like "take me home mama".  After a late night feeding and benedryl dose he finally fell asleep.  He looked like a little chipmunk with puffed up cheeks the next day but was feeling better.  The couldn't get us out till around 2 p.m. and we are on the priority list much to our dismay.  We finally got released and Luke made a speedy recover at home.  We kept him out of school for a week just to make sure he was 100% before going back to school.

One day post surgery #2:

Here are some before and after surgery pictures.

 

(this is not a great pictures but goes with the side view above)

Then the flu was going around so we kept him home for another week and broke down and got him and Hannah the flu shot!  He was back in school and doing well until this week.  He came down with a runny nose, lots of drool and a fever on Monday and still all this ickyness and it is Friday early morning.  I am praying that tomorrow will be a day on the way up out of the sickness pit.  However, Hannah had a low level fever when I went in to comfort her tonight :(

In the weeks before Christmas Hannah had her first big sickness.  She has a virus so bad she got a rash!  And then she got bronchitis   Poor baby.  She was feeling well enough to enjoy presents and the festivities though.  Although the glow stick was not distraction enough for me to hold onto a real candle during Christmas Eve service.  I may join the kids with glow sticks for the next few years.

January and February have been rough in terms of nursing.  We had many many nights without nursing and my sleep has been not that great.  Last week we had our first week with full nursing in a long while and it was great.  I forgot how productive I could be and how well my brain works on decent sleep.  I have been trying to adopt some of the principals in the Sanity Manifesto of my last post.  I realized that I need more routine in my life to get things done and to be more present with the kids because I know there are times in my day to get chores and other things done.  I have tried scheduling chores before and liked it and decided to go for it again.  I pick a chore that is ~30 minutes (some are more some less) for every weekday.  I leave Saturday for organizing something or a cleaning project that doesn't need done every week.  And I did it.  This week I got through Monday and Luke got sick and I got a little sick.  However, I did catch up and do the floors tonight :)  I felt and feel great about it.  My goal is to get up earlier, after going to bed earlier, and have devotional time, then workout before I need to be going for the day.  It happened once so far and I felt great that day.  My trick is getting to bed on time.

So back to nursing.  Well in the mist of the nights of no nursing my current nursing company got a call from Medicaid saying they were not going to pay them as much, by a lot per hour.  There was a mistake in the system and they were changing it reflect the fee schedule for the service.  My company was all up in arms and said if it couldn't be resolved so that they would get more money they would drop me.  This involved almost 2 weeks of being on the phone, and not playing with my kids, something I really don't like, to finally figure out what was going on.  I found out Medicaid was right there is a fee schedule for private duty nursing and they were getting paid way above it. I am not surprised by Medicaid's actions.  However, this is the second time my company has been surprised by Medicaid.  Ahhhh!  So while working to find out what was going on I was also trying to see if some other nursing company could possibly take me.  And my 3 day a week nurse was leaving to move down state.  Well one of the two nursing companies said they could work on hiring people and it was doable.  Awesome.  Their billing department, who I called to make sure my insurance would work (Luke has been on the same insurance since he started with the current nursing company but they hadn't looked anything up to make sure they would get paid and what they were getting paid and I was not making that mistake again if I could help it), helped me to figure out what was going on with Medicaid.  Because you can talk to all sorts of Medicaid people but the billers won't talk to you unless you are providing the service.  So frustrating! I do have the name and e-mail of the lady that finally helped me.  So tomorrow is the official start of our new nursing company!!!!  This new company couldn't staff me 4 years ago and I am grateful that the old company could and we got out of the hospital.  I am excited for this new company they had many private duty cases like Luke's.  This means that we will get full coverage more often when things like the last month and a half happen.

On to better topics... Hannah is also developing in leaps and bounds.  She is talking, lots of talking.  It is very cute and she says a lot of things, even puts words together.  I haven't counted all the things yet but she is pretty vocal and like to learn new words.  She is signing almost as much as she is talking and I think there are some words she just signs but she signs and talks most of the words she knows.  She is running and climbing and getting into things we never thought she would or could get into.  Typical toddler.  We were spoiled with Luke, but he seems to be picking up the pace.  This week Hannah really got into her baby, which is a cute toddler looking doll that my parents got her for Christmas.  She calls anyone that is under 10 a baby I think, and she loves babies under 1 the best.  She also likes cars and building with blocks.  She has been getting creative and enjoying playing with play doh, coloring, and painting.  

 

We got the kids a dollhouse for Christmas and they both really like it and all the little furniture pieces and people.  Luke's favorite were the chairs till he got a kitchen with doors that open for his birthday.  We were hoping this would help Luke with his imaginative play and it seems to be doing well (Hannah has no problems in that area).  Hannah's favorite is the baby and putting people to sleep.  They both really like putting people to sleep.  This dollhouse comes apart and you can rearrange the rooms themselves and not just the furniture which is pretty nifty and Hannah finds almost as much fun as I do. ;)

Oh, Luke's newest interest is in measuring tools and trying to measure stuff with them.  It started with the play square he has on his tool bench.  (He got it last year for his birthday and is just this past few weeks interested in it) and then we brought him a yard stick which he loves.  I work with him to measure the dollhouse furniture but he is more interested in measuring peoples arms.

Luke's favorite book right now is one about snakes.  It does have a page with a ruler on it, which of course is his favorite page.  Hannah's favorite book is "Yummy, yucky" and she likes to say "yucky" and "no, no, no" when we come to the page about crayons being yucky because she has been found eating too many crayons and mommy saying "no, no, no".   You never realize the things you say and the weird noises you make as you mumble to yourself until you have a toddler. :)

We had a great 10 ft tree this Christmas, well from the end of November till mid January.  Everyone loved it.  Kevin took the initiative and cut it out of the back yard.  I would not have thought to bother with it but I am so glad Kevin did.  It was well worth it.  I loved to just look at it lit up and so did the kids.  It was beautiful.

Luke has a sleep study and visit with the new pulmonology team in March that I am looking forward to (and considering keeping at home out of the way of most germs until then) hear they have to say about him.  Hopefully I will update you on that visit soon after it happens.

 



Oh yeah, our house is STILL for sale.  If you know of anyone who wants a great move in ready vacation home by great ski hills, snowmobiling trails, Lake Michigan and other large inland lakes please let us know.

Prayer requests:
That the house would sell soon
Luke to no longer have central sleep apnea and hypoventilation
Luke to be a candidate for a diaphragm pacemaker till the above happens.
Luke to be healthy for the March sleep study.

Praises:
Luke is doing tons of new things!
We have a house to keep us warm and dry.
Kevin got a job promotion!
Selling Thirty-One bags is going well both for a way to get out of the house and a way to make a little extra money.
Hannah is doing lots of new things!
Having cute kids and a great husband!