Luke's Story - Birth to Trach

Luke's breathing/respiratory story begins at Luke's beginning and as you will see things can and do change on a day to day and sometimes hour to hour basis.

Day 1: When Luke was born (by c-section) he squeaked a couple of times and then was not really breathing on his own. We knew something was most likely not right with Luke so there were neonatologists standing by. They intubated Luke and after a short look at him they whisked him away and put him on a ventilator. He was on “room air” which Kevin, my husband, told me was really good for being on a ventilator. Neither of us really knew what this meant but boy oh boy we would learn. Room air means that he is not on any supplemental oxygen, which is really good.

Day 2: Luke was doing well and they thought he was ready to breath without a ventilator the day after he was born but they wanted to give him an MRI first. Since you have to be still for an MRI they put babies under and they don't have the reflexes to breath on their own so he would need the vent for the MRI and they would take if off after the MRI was done. They wanted to do an MRI because they though he had a neurological disorder and they wanted to find out more and hopefully make a diagnosis with the help of the MRI. This was a huge time of unknowns. Remember this was in the first 48 hours of our experience with Luke and we had a full intervention birth after starting with a home birth plan. At this point they were not sure why he wasn't breathing on his own, Luke was a full term plus baby (41.5 weeks) and Luke was not sucking. Kevin and I do not remember if they had noticed the lack of facial expression yet.

Day 3: The MRI was scheduled for his second night and when we came to visit our sweet Luke in the morning he was on a nasal cannula (just oxygen through the nose). By the afternoon he was put on CPAP because he wasn’t breathing well enough on his own. CPAP for a baby entails putting a large prong thing in his nose that is attached to tubing which is attached to a setup that keeps Luke's airways open a little bit all the time and thus he doesn't have have to totally close and open his airways for every breath making it a little easier to breathe. I am not sure if he wasn't keeping his oxygen levels up or if it was that he had too high of CO2 levels. I am thinking CO2 because I don't remember Luke ever being on oxygen in the NICU (expect for right after surgeries and for a few brief moments here and there until he got sick in the fall of 2009). I don't remember what PEEP (positive something about airway pressure) he started on but I remember a lot of PEEP 6 and 5 for the time he was on CPAP. A PEEP of 5 is the lowest they will go on CPAP and the next step after a PEEP of 5 is a nasal cannula. Luke was getting breastmilk through a feeding tube in his mouth at this time and doing great with it.

Day 4: We got to hold Luke for the first time. Luke's ventilator and and umbilical catheter were removed and so Luke was ours for cuddling. The results of the MRI came back to say that all neurological parts where there but he did have two old subdermal (I think) hemorrhages from in the womb and that the blood would drain from those on its own. They didn't see these hemorrhages as explaining anything going on with Luke, breathing, sucking or anything. My note from this day said that Luke is getting better in not having so much mucus so they thought that he may be swallowing and that his motor functions were getting better. When Luke was born he was hypertonic or very stiff but sometime at some point he became hypotonic meaning he had really low tone and continues to have hypotonia. (We would later find that the servre low muscle tone is the most probable cause of his breathing issues.) The plan was to put Luke on nasal cannula the next day and we all expected great things out of him. We would then move from working on breathing issues to working on sucking and swallowing. (What was little known to us then was that Luke’s breathing would be and still is a long road to travel.) Luke is doing well digesting breastmilk and if he continues he will get his IV out. Luke was a good eater then and is still a good eater or should I say digester since food starts in his stomach.

Day 5: The doctors decided to wait one more day until trying Luke on nasal cannula. Not sure of the reason.

Day 6: Luke was doing well on the CPAP and so on Luke's 6^th day they put him on the nasal cannula around two in the afternoon, I am not sure how much oxygen though. He also got his IV out because he is doing well and getting all his nutrition from breastmilk. We had now seen the neurologist and geneticist and they said that we may never find a diagnosis for Luke but they would look because with a diagnosis you can plan and know what the future may hold. We actually figured out the diagnosis after our friend Leslie, who has Moebius, heard Luke's story and said she thought he had Moebius Syndrome, but more on this later.

Day 7: In the early morning of day 6 Luke was put back on CPAP because his CO2 levels were too high. This was Luke's problem throughout the process of weaning to nasal cannula and probably still is. He doesn't have much trouble keeping the oxygen up but he has a hard time keeping his CO2 levels down to the levels they should be at. At this point we have no diagnosis and if we had one it would give us a better idea of what is doing on with Luke. The doctors had a couple of ideas why he wasn't doing well on nasal cannula (I don't remember what they were just knew this from a note I wrote back then). We will try nasal cannula tomorrow and Luke will get a couple of more chances before they decide to put a trach in so that they can send him home. I remember them telling me at some point that staying on CPAP long term is not good for the structure and health of Luke's upper airway and so if he needs help long term they would need to put a trach in.

Day 8: (February 12, 2009) We gave him a day of rest and on his 7^th day in the morning of day 8 he was put back on nasal cannula. He lasted for a couple of hours till early afternoon when his CO2 levels were too high and then was back on CPAP. The ear, nose and throat doctor (ENT) was scheduled to come the next day and talk to us about how to help Luke breath and swallow. I am sure this was disappointing at the time for us as my note for that day started with “Today was a day of ups and downs”. I don't really remember much about my feelings then. I look back and it seems like I was taking this all so well and I would say I continue to take all of Luke's medical issues and ups and downs in stride and fairly well. I attribute this to God. There is no other way I could function and continue to be a mother, wife, daughter, friend through all of this. Our Pastor and his wife came to see us 3 ½ hours away from our home this day and it was one of the high points of the day. Luke has been with us for a week now and oh what a week it has been.

The next day, a Friday, the ENT said that Luke would get one more try to move to nasal cannula and if he didn't last then a tracheotomy was the next step. He said that if Luke could not make it on nasal cannula then he would likely need to be on CPAP long term and that it was bad for the nose and throat passages to have CPAP through his nose long term. Also Luke would not be able to go home on CPAP through the nose but would through a tracheotomy. He also said that the tracheotomy tube may bypass what is causing the problem and he may be able to breathe without the help of CPAP after the tracheotomy. I was thinking this was kind of fast and shouldn't we be doing more tests and I still sometimes think this. But back then there was a lot going on and I didn't have the energy or know to explore all of these things. The ENT did mention that he would put a scope down and look at the structure of everything involved in breathing when he did the tracheotomy. I didn't know what else to do at this point and this made sense. I was just hoping and praying my son would be able to breathe long term on the the nasal cannula.

On Sunday we were told that Luke would try his last chance at the nasal cannula on Monday. However, when Monday came they derided to wait because they heard some not so great lung sounds in the morning and instead turn him down from CPAP with a PEEP of 6 to a PEEP of 5. I was disappointed but figured his chances would be even better at making it the next day.

Luke did not last on nasal cannula on his last try. This was a hard thing for us to deal with. My son would be getting a tracheotomy. And not only a tracheotomy but most likely a g-button as well. Since no one had really seen him swallow, they didn't think with his neurological issues that he may ever swallow. They also thought that he may have aspiration issues and with the upcoming tracheotomy they decided a g-button would get food where it needed to go without the tubes down his throat or nose, which he was starting to pull out on a regular basis. During the nasal cannula trial Luke turned blue in my arms, very scary, and it was just after or during a feeding so the nurse had me lean him forward thinking he was aspiration and hoping leaning him forward would make it come out his mouth. I didn't write about this in my notes from back then but I think there was some milk or something that I saw filling up his mouth and then the turning blue. Well he got an x-ray to see if any got down in his lungs and thankfully it was clear. He would get an upper GI to make sure all his digestive track was physically there and in the right place but also see if he was aspirating so that if he was aspirating they would also give him a Nissen.

When looking at my notes it looked like they would explore his swallowing better after his trach was put in. They were thinking after the trach was in that he would not need ventilation and with no ventilation and no CPAP blowing air into his nose and mouth areas creating mucus from the irritation of the air constantly blowing that they could see what was really going on with normal secretions. This was huge, two major surgeries that were planned to happen within 7-10 days of each other. We would not be taking Luke home like a normal baby, but with a trach, g-tube and all the medical issues that come with it. And at this point I thought I would be going home a few weeks after the trach was put in (which would have been the case if not for the ventilator which you will hear about later). Luke slept most of the day after the failed nasal cannula trial which was good for me as I needed to try to regroup and take in all of this.

Luke has been alive for 14 days at this point and wow he has gone through a lot. I really see how much when I write it out like this. But there were good things going on in the mist of the disappointments with his breathing. He was moving more and it seemed that once food got into his stomach his digestive system worked very well, the proof was in the many many diapers.

I am not sure when it happened but I see in a note that on February 22, 2009 I wrote that our working diagnosis for Luke is Moebius Syndrome. In Luke's 2^nd week I know we had thought that Luke had Moebius and started asking doctors, who when we asked said they suspected but didn't know for sure. Once we mentioned the possibility of Moebius Syndrome they were supportive and I think the announcement in this note comes from us finally getting the neonationlogist to say it. The ophthalmologist suspected it first but didn't say anything till we did because he didn't want to tell us something before more doctors came to the same conclusions. I know that at least one of the doctors wanted to make sure it wasn't just birth trauma and would go away with time. What got us thinking was my parents talking to Leslie Dhaseleer, who has Moebius Syndrome. My parents had of course shared some of Luke's story with their churches so people generally knew what was going on. Leslie happened to be at church that first Sunday after Luke was born. She heard about Luke and came up to talk to my parents after church. She started asking them if Luke had certain symptoms and Luke had the symptoms she asked about but the congregation was not told about these things. My parents were blown away and asked how she knew this and she told them she thought Luke could have Moebius Syndrome. My parents talked to me and we started asking questions of the doctors and found out that it had been thought of, as I mentioned before, in some of the notes but nothing said to us yet. Having Moebius Syndrome was really not we wanted to hear but now we had a diagnosis, we had a place to start looking for solutions to some of Luke's issues, and a glimpse of what the future may hold. Leslie has become a great resource and friend and gotten us involved in the Moebius Syndrome Community. I feel blessed by God to have her in our lives and that we were able to figure out what Luke had and then have a immediate contact to a community of people who had Moebius or were parents of Moebius children.

On February 23, 2010, before he was a month old, Luke had a tracheotomy (to put a trach in) and broncosopy (to put a scope down Luke's airways to check their physical status). I am not sure how long we waited in the waiting room but it wasn't a short amount of time. The doctor came and talked to us as soon as it was over. He said that his airways looked good and clear and it did not look like Luke's tongue or any other part was interfering with his ability to move air. He did say that Luke's left vocal cord may be paralyzed. Luke was so little that the doctor did not want to give a for sure on the left vocal cord paralysis but said it was a possibility.

Luke looked pretty bad when we got to seen him. They had him on sedative and pain drugs and he was still pretty out of it for awhile. He had two strings that were attached to his trachea so that if his trach fell out they would be able to open his trachea up and stabilize his airways. He had these for a week before the tissue healed enough to be more stable. At this point he had an IV in his had with a splint on it to keep the IV in and laying right. This splint was a good club and throughout the time with the IV it was tough to keep Luke from clubbing himself with it. Luke was on IV fluids for two days before they put him on milk and took the IV out. They put the IV in his head for his next surgery and I liked it there much better. He has the use of his hands it was more out of the way and less in danger of coming out. Luke is a really hard poke and so putting the IV in again is not something anyone in the NICU wanted to do.

So that is Luke's story pre-trach. I will post more of his story later.

Communication - No facial expression does not mean no expression

I will not deny it, having a baby with no facial expression can be hard.  You can't see smiles or smirks or the twitch of their face as they get upset and are ready to cry.  However, Luke has gotten pretty good at letting you know what he wants and what he likes and does not like.  Luke is not keen on new things and when he sees a toy or something he doesn't know or like he will turn away with his whole body or just keep his head turned away and ignore something.  Since he can't move his eyes outward and has to move his head to see side to side it is easy to see what he is noticing or ignoring.  Luke does a lot of flailing and kicking his legs.  I think he does this becuase he is not able to cry audibly and kicking make more noise and gets more attention than his silent crying.  When I am right there with him I can easily see him cry.  He can open and close his mouth and when he cries I think he has his frozen face.  Not that his face is mostly frozen but he will have his mouth open and frozen and the look in his eyes.  Oh his eyes.  His eyes can say so much and then sometimes when you want them to say so much he is just unreadable.  But those eyes can pierce through to your soul and you can do nothing but love him.

Luke has been learning sign language since he was around 5 months old.  He picked it up pretty fast and could sign potty/diaper and milk right away.  Sometimes he is in periods where he will sign a lot and sometimes not so much.  He signed when he had a diaper a lot at first and then didn't for a couple of months and then he did again and now does it occasionally but will still sign diaper if you ask him to.  A lot of his signs come as confirmation or a yes to questions you ask of him.  If you ask him if he needs a diaper and he does he signs diaper, if he doesn't he doesn't sign.  This works a lot of the time and is good.  He also knows suction which is a two handed sign.  His two handed signs all sort of melt together.   Love and all done look similar.  More, book (he learned this as more books and just does more) and suction look similar.  It is sometimes a guessing game and sometimes you have to put his signs in context. He also shakes his head yes and no, but mostly no.  I am trying to get him to sign yes with his hand but it is close to potty and I think they are overlapping right now.  Luke is able to put his hands up when he wants to be picked up and he points with his hand and that is helpfull.   We are still working on pointing with just a finger.

Some sign language resources are "Sign with your Baby" by Joseph Garcia and Baby Signing Time (Signing time as well for a little older kids).  There are also a lot of resources out there on the web and I hear there are some iphone apps for those of you that have an iphone.

Oh and one of the best things in his signing was at Christmas when my parents got him letter blocks that spelled his name.  My mom put them in order and said "L-U-K-E spells Luke" and Luke held his hand out and made an "L".  We were stunned and amazed.  We had been signing Luke his name and mainly the L for awhile with no response from him and this response was totally unprompted.  He may not look it in the face but he is so smart, I may be biased though.  Don't underestimate your baby. 

So bacially what I am trying to say is that just becuase your child doesn't have facial expression and may not move around as much it doesn't mean you won't be able to tell what they want or are thinking.  They will figure out their own way to get your attention and let you know what they want.

Cloth diapers

I am big into cloth diapers.  I use a mix of organic prefolds and fitted diapers (Growing Green or something like that I think) with covers.  I also use the Gro Baby system which is now been revamped as GroVia.  They are an all-in-two system so they are meant to use the cover over again but the insides snap into them.  I love the covers and use them with my prefolds and fitted diapers as well.  I also have a couple of Bum Genius Organic all in ones that are just fine.  I like using the covers over and not just tossing the whole diaper, especially after just a pee because the covers are rarely that dirty.  I throw mine over the shower in the bathroom where the diaper pail is kept.  This is a shower we do not use but I am sure you can find some place to keep them airing out for the next use.  We don't have a diaper changing area mainly because Luke is tied to a ventilator most of the time and is just not that portable.

I have one-size diapers because it seemed like a better deal to me to not have to buy three or four sets of diapers as my baby grows.  I don't know how a newborn would due with my stash of diapers as Luke was 3 1/2 months when he came home and so not quite a newborn anymore.  We planned to make or buy ones for newborns as I have heard that one-size can be hard to fit but I also have see pics of the diapers I have on newborns that seem fine.  

Try out some diapers, ask around to friends, buy some on-line and try with your new baby before you buy a bunch.  And if you do want to make a bunch before baby comes or get a deal on a whole set great!  If you don't like them there are many places on-line where you can sell or trade them for a type you do like. 

I have made some covers, diaper pail liners and some diapers and it is very easy but with Luke and his special needs and the pregnancy we thought would end at any minute, but ended up lasting 41.5 weeks I really didn't get to making them.  Recently I have made a cover and have started sewing again and it is really easy.  There are tons of patterns out there for free and for a price.  I have looked at some of both.  I would test some diapers out to get the gist of what type you like before making a bunch.  Go over to a friend's house or ask around.  All babies are different so some may not work well on your baby.  However, if you make a bunch you don't like you can always sell or trade on places like diaperswappers.com or ebay.  Diaperpin.com has a lot of reviews as well that can be helpful.  I found a great site http://fernandfaerie.com/sewing_prefolds.html and I have used old t-shirts to make prefolds and they work great.  I have a box of t-shirts waiting to be made into prefolds.   There are many places to buy fabric and I have even bought organic and hemp fabrics that I really like.

For the diaper pail liners I bought some PUL and sewed it like a pillow case.  The top I folded over and left a space for a cord to cinch it up.  It took me very little time.  Although PUL is hard to sew so I would look on-line for tips.  I wasn't really worried about how diaper pail liners would look so I just plowed through without using some of the more ascetically pleasing tips.

Washing is a big deal.  Make sure you get a good detergent.  I am using one I got at the local co-op natural foods store called Selestial Soap (http://www.selestialsoap.com/) and I realized they are made in my area so all the better as they are a local product.   I would like to try Tiny Bubbles by the Natural Baby Company that also makes GroVia diapers.  They have good reviews.  The biggest thing is to get a soap with no residue, at least that is what we needed.  Our diapers started to smell bad and I got tips like I could be using too much soap so to rinse them a lot until all the soap is out.  This helped some but then I realized our soap leaves residues and when switching to Selestial Soap the smell has gone down.  I don't know if I can get it out after the months of residue but only a few of the covers smell really bad on a regular basis.  Luke's pee can be foul smelling as he is fed a lot of veggies that normal kids may not eat because he just go right into his stomach.  This is a site that rates detergents for diaper washing http://www.pinstripesandpolkadots.com/detergentchoices.htm

When we go to doctor visits and out of the house we use Seventh Generation disposables or we use the g-diaper inserts.  GroVia makes biodegradable, compostable, flushable inserts like the g-diaper inserts you have heard of and they go right into your GroBaby/GroVia

And if you are thinking ewww my hands in the toilet to rinse poopy diapers don't worry they have sprayers you can get that attach to the water line for the toilet that you can use.

Well that is my take so far on cloth diapers.

Luke's g-tube and feeding history Part 3 with a little bit of bloating and refulx

So in December some time Luke had a couple of things start to happen that we still don't really have explanations for but they are not happening anymore and didn't happen with that much frequency even when they were happening.

First, he would wake up at night high pressuring his vent by bronchial spams (his airways opening and closing or spasming).  When his vent high pressures it is because the vent is using more pressure that we would like to put air into Luke's lungs.  This usually happens when he is out of sync with the vent or crying, or purposely trying to get our attention by pushing air back against the vent.  Luke would not really be awake during the time.  His eyes may open but he was really really trying to be asleep and he was also arching his back and seemed to be very uncomfortable.  Holding him may help for a minute or two but that was all.  This first episode lasted hours.  It was not fun to watch as a mom who could do nothing.  The next night was fine though and I was hoping this was just a fluke.

A few days after this we also noticed that Luke's button got really tight and pretty quickly, it seemed like in the space of a couple of hours and the nurse didn't notice when she was feeding him in the morning but I noticed 3 hours later.  I just watched it but noticed that there was bright yellow bile coming up from his g-tube when we burped him before the feeding.  By that night there was some dark coffee grounds looks stuff coming out from around the stoma and sometimes in the feeding tube.  This was not too good but I didn't know what to do and at that point was hoping it would go away soon and I had just eaten something weird that had come through the breast milk to Luke.  Well the next day Luke was uncomfortable and lethargic.  By the time Kevin got home I was worried but we thought we would watch him for a bit more.  By this point lots of coffee grounds were coming from around the tube.  He was looking so uncomfortable and maybe it pain, it is hard to tell with a Moebius child who has no facial expression we decided to take him in.  His button was super tight and he seemed very bloated.  We called the surgeons who put the button in who agreed with taking him to the ER.  When we got there the ER doc ordered an x-ray to look at where things were and saw that there was some pocket of something by the tube.  He called the pediatric surgeons from DeVoss and over the phone they thought that the coffee grounds was dried blood and this would irritate the stomach and was likely what was bloating it and it is hard for Luke to get rid of excess air because he has a Nissen Fundoplication which pinches the top of his stomach so things can't go back up and possibly aspirate into his lungs.  They instructed us, Kevin and I, through the ER doc to take the button out and let things drain and then put the button back in.  I like to do as much as I can myself, some people don't want to be the bad guy but I would rather have someone who knows Luke and is comfortable with him and him with them to do stuff too him.  Also a lot of docs don't seem very comfortable with Luke because he is on a vent and they assume very fragile.  Also I am pretty go to and in your face.  So the ER doc was very happy to let us do it.  He was finally sleeping at this point which we liked because he wouldn't be so wiggly.  Well when we pulled that button out there was a mini volcano of coffee grounds and bile.  Luke's stomach immediately went back to a normal size and Luke woke up and started making noise and was his normal happy, playing self.  It was like we just turned a switch.  I was ecstatic that it was that easy and wished I could have done it from home.  The docs gave us a prescription for Zantac to help calm his stomach and sent us home with Malox, I think, until we could get the Zantac the next day.

I remember talking to maybe the pediatrician and I am not sure what other specialist about the night time episodes and the tummy bloating and not really getting any answers.   The next time we were at the pulmonologist (which may have been a month or two later) I ran the night episodes, as they were still happening, by the doc and she immediately said she thought it was reflux.  They thought that the food was coming up just enough to trigger the Vagus nerve which signaled reflux and protected the airways from aspiration by having bronchial spasms.  Even with the Nissen Fundoplication they thought that reflux just a little bit up could trigger the nerve.  I have also read a lot about Nissen Fundoplication that fail.  The episodes sometimes had a lot of suctioning and sometimes not.  The suctioning seemed to go more with the face that the spasms where creating the mucus than any food getting up there so I didn't think the Nissen Fundoplication had failed.  I also started making sure he was fed sitting up or at least with his trunk up and leaving him inclined for at least 20 minutes after each feeding.  We used towels to incline his crib since the nurses fed him in there twice a day. 

These reflux episodes can be minor to severe, I think all in all there were only 2-3 really sever episodes and maybe less than 10 minor ones.  However they usually lasted for an hour or more.  It was awful to watch and not be able to do anything.  Luke couldn't stop the spasms and they would wake him up and then the uncomfortableness of  reflux just sent him to a place where no person could sleep.

We continued to take Zantac and got an appointment to see the GI docs.  I was talking to the pediatrician about the reflux episodes, they weren't stopping and he offered to prescribe Prevacid.  I was wary and didn't want to because I had heard some. bad things about it from other blenderized diet parents.  However, when we saw the GI doc (which was in May and this all started around December) she could not say exactly what it was especially because the episodes were so few and far between.  She said reflux is difficult to concretely diagnose and she wondered if Luke just had a really sensitive stomach.  With all the big episodes, but I don't think so much of the small ones, I had been trying to find anything that could have triggered reflux or any of the reactions we were getting.   I had figured out that the big episodes were linked to him accidentally getting 30+ ml more in a feeding or being fed while laying flat.  The doc thought this supported the sensitive stomach but since we had recently had a bad episode she wanted to try Prevacid for a month and then no reflux meds and see how things went.  I agreed to the Prevacid after asking many questions and being assured that getting off it would be very easy if he did not have reflux but still not too hard with reflux (I had heard stories that once you started Prevacid it would be hard to wean a child off it).  Prevacid basically stops the acid production in your stomach which is a pretty major thing to do.  But for a month I would try it.  After a month we got off all medicine and we have not had any reflux or should I say "reflux" episodes in many months.  We will see the GI doctors in a month and hopefully that will be the last time.  I am hoping we were just blowing up out of proportion a normal baby digestive system getting used to the world thing. 

I was also trying to see if the stomach bloating/tight button episodes were correlated with the reflux episodes (I am a trained research scientist).  And for the reflux episodes I never wanted to test Luke by giving him way to much food or laying him flat to feed and seeing what happened because I didn't want to cause him hours of no sleep and pain at night.  It ended up that it didn't always happen after reflux episodes at night but also a couple days before a bad reflux episode so we determined that they were not directly tied together.

The bloating episodes were still going on through all of this.  Mainly in January and February I would say.  We pulled the button out and drained things once with good success.  We never got as much out but then we never let it get as far as the first time.  One time we pulled the g-tube out to drain in and then put it back in and two hours later he was bloated again.  After a couple of times in and out where it didn't make a difference we stopped taking it out when he was bloated, thinking we would only do it as a last resort.  We also worried that by taking it in and out often he would lose enzymes and nutrients he needed in his stomach.  The bloating episodes could come on in an hour or so and go away during a feeding or another time just as fast.  Sometimes they would be all day and sometimes just a few hours.  They were usually accompanied by bright yellow bile coming out of the g-tube when we were decompressing before feeding.  We never got answers to why this was happening.  Their best guess was that the button was irritating the inside of the stomach causing things to bleed and the blood was irritating the stomach which cause bloating. 

We are not on medicine for any of these issues and we haven't had a bloating or reflux night episode in months.  We thank God for this and continue on.  During this, in the middle of January I started introducing real food through Luke's g-tube.  By late February I stepped up the food big time and he has been doing great with the food.  I stopped pumping in June and ran out of breast milk in August and Luke has had no problem on all cow's milk.  I will do a separate post about Luke's blenderized diet even though this was going to be a feeding post, I realized it is a post all unto itself.

Luke's g-tube and feeding history Part 2

So in September (Luke is now 7 months old) the valve on Luke's Bard button broke and anytime you opened the button stomach contents came spilling out.  This was not good as he was losing stomach acid and valuable enzymes.  We tried to work with it and be really quick about putting the feeding tube in and taking it out and closing it when we were done but it was not going well.  We took a fast trip down to the surgeon and got it replaced with a Mic-key button which we would be able to change out ourselves, which proved to be very beneficial in a couple of months.  The Mic-key button sticks out more from Luke but not enough to make any difference it seemed to Luke's daily lives.  Now getting the correct extensions to go from the button to the feeding syringe for the Mic-key button was very difficult.   In the hospital it seemed that almost every time we ordered new extension tubes they sent up ones for a Mic-key button and not the Bard button.  So I was thinking that extension tubes would be easy to get for the Mic-key button.

However, getting the correct connections and lengths were the tough part.  They come with strait or 90 degree ends at the button and the other end can come with and without a medicine port.  They also come in two different diameter tubes.  We used the 90 degree thin tube when we were putting just breastmilk through it but when I started putting my real food blenderized diets we needed the strait end to help it move through and the strait end (bolus) tube is a larger diameter.  All the reference numbers for these tubes are really close together and I had to do a lot of trial and error with my durable medical equipment (DME) company.  The people ordering at the DME may have no idea what your equipment really looks like so you need to give as much description as possible and if you can find a reference number somewhere that helps.  However, be ready for trial and error.

We started with the medicine port top it didn't stay on the bottom of the syringe very well and we had LOTS of messes.  Also the medicine port would just come open.  When I started ordering the strait bottom larger tubes I realized that we could get the non medicine port top which fits very snugly on the syringe.   I would recommend the tip without the medicine port to everyone.  We usually put medicine strait into his g-button with syringes that fit directly into the button.  These syringes are hard to fine but you can ask for them at your pharmacy or your DME.  We ended up with some for awhile that had a twist tip that we just cut off with a box cutter and then washed the syringes before use.

So we were smooth sailing g-tube wise for a couple of months (breathing and health wise is a different story and got crazy in here but that will be a different post).

So much to say and so much going on

I have this strong drive and passion to get Luke's story all written down and I have wanted to for awhile.  There is so much that I get stuck on where to start.  But I have by making this blog and I keep telling myself even if it takes months at least I am working on it.  There is a lot of stuff to say about Luke.  And as I am writing and thinking I have come to realized how things with Luke are relatively slow right now.  I am looking back at one thing after another going one with Luke.  If it wasn't breathing, it was his g-tube and then feeding issues, and then Luke just being a baby.   I get exhausted just thinking about all of it sometimes.  But I will get it down and it feels good to have every new portion down.  It may be a little disjointed at first but it will get better hopefully. 

And as I write I have found that the story doesn't speak loudly to Moebius Syndrome directly but all the issues Luke is dealing with are things that many Moebius Syndrome parents face.  Moebius Syndrome is a clinical diagnosis and there is no cure, you just treat and help the symptoms as they come with therapy, ventilators, trachs, g-tubes, ect...

Which makes me think of something.  One thing I have had trouble with since Luke has so many "issues" is not overreacting to normal baby behavior and issues.  As my mom says "Luke is under a microscope" and under that microscope his normal baby issues tend to be blow out of proportion many times.  So I would caution those with children with a lot of medical needs to ask around to other parents and your own parents and see if some of the things going on are just baby things and not related to the medical things going on.

Luke's g-tube and feeding history Part 1

At birth Luke didn't seem to be sucking, or swallowing, and so they put a tube down his throat to get some good nutritious breastmilk down to his tummy.  He did great on breastmilk but his breathing did not improve and becuase of the trach (and ventilator) and aspiration risk and with Moebius children having trouble swallowing and eating they put in a g-tube when Luke was about a month old.   His first g-tube was a Bard button.  The Bard button has a low profile, it is a lot thinner than the Mic-Key buttons) and we liked it.  The downside is that if something breaks on it you have to go back to the surgeon to get it fixed.  They told us this button could last from 1-3 years and we thought that Luke would possibly not need a g-tube by then.  It's a 3.5-4 hour drive down to our surgeon so we weren't really wanting this button to break.  A few weeks later he wasn't gaining as much weight as he should and so they brought a dietitian/nutritionist in to see what he needed.  Since I he was eating breastmilk they assumed that he wasn't getting enough calories and so said they wanted to add some formula to the breastmilk.  I was devastated and mad.  But I didn't know as much as I do not, or even a couple weeks later, and so I said okay since I wanted my son healthy.

So begins my battle with nutritionists.  Well this nutritionist came back 3-4 days after we started adding the formula to his milk.  During these 3-4 days I carefully watched his weights that were taken ever night.  They wanted him to gain 25-35 g every day.  Well he had been gaining 30-40 g those 3-4 days.  However the nutritionist said that he still wasn't gaining enough weight and she wanted to increase the formula to a higher calorie one.  I was freaking out and asked how she came up with that since every day he had been gaining enough weight.  She said that she hadn't taken the last two days weights into consideration but she had averaged the weights of the past 10 days which is what she does to evaluate weight gain and he still wasn't gaining enough.  Okay does anyone else see a problem with this.  She is using the past 10 days weights to determine if the formula Luke had been getting for 3-4 days was working of which she only had a few days of data.  So I pointed out to her that she can't use the past 10 days since he hasn't been getting formula for all those 10 days.  She has a before average weight gain and she has to take just the weights since the addition of formula including the ones she hadn't figured in yet to see if he was increasing.  I told her he was doing great with weight gain now and the numbers should show that.  She took her stuff and said she would be right back.  When she came back she had her statistics figured correctly and didn't think Luke needed the higher calorie formula.

About a week or so, I don't quite remember, Luke's vent settings were changed around to help him with his breathing more and then he started gaining weight too fast.  So after 14-16 days of formula addition it was removed.  However, it was only removed becuase I noticed the large weight gains and requested the nutritionist come back becuase I wanted to remove the formula.  I am not sure when she would have come back and even if she did if the formula would have been removed.

So back on track with just breastmilk we left the hospital when Luke was 3 1/2 months old.  Which since then I have realized is a minor miracle for a vent baby.  Before he left Luke had been getting some granulation tissue around his stoma site.  We were told that it is normal for the stoma to develop granulation tissue, which I think is when tissue is trying to grow to close the open hold and exposed skin in the hole created by the button.  We tried putting a split sponge under the button and having nothing under it.  On the day we left they decided to put silver nitrate on it, they just took at stick with silver nitrate on the end and touched it to the granulation tissue.  The tissue turned gray/black and then fell off and after a day or two his stoma site looked great.  We were glad it was taken care of with a pain free easy method.

Luke left the hospital with plaster casts on and his last weight without casts was a day before he left.  They also took a weight, with the casts on, the day he left.  We had to change the casts every week so we took our scale and weighed Luke while his casts were off every week.  I called in a number of the the cast weights to pulmonlogy.

When we got to our first pulmonoloy appointment Luke had just gotten a new set of casts on but they weighted him anyway becuase it is their procedure.  I did give them the no cast weight of that day and some others they did not have.  When the nutritionist came in we asked if we can eliminate one of Luke's night feedings.   We were also looking for a possible food increase since it had been over a month since he had an increase.  When the nutritionist came in she said she didn't want to increase Luke's food becuase he was gaining 50 g per day.  I was surprised but we moved on and she went out to figure out the numbers of how to eliminate one of the night feedings.  While she was out I figured did the math and he had only been gaining 25g since he left the hospital not 50 g.  Something was wrong here.  So when the nutritionist came back I asked her how she figured 50 g and she was figuring it based on cast weights.  I informed her that his casts in the hospital were very different from the ones he got out of the hospital as they had more time to work on them and that each week the casts were different and that is why I had been giving her no cast weights.  Also she was using the weight they got at the office just then which was a cast weight when the casts were fresh and wet so they weighted quite a bit more than a dry cast.  She seemed to not really care that she was using cast weights and didn't really think it was that bad of a thing to be doing.  Ahhhhh!  She did say that she wouldn't change what she was doing since 25g was still within what they wanted.  She told me that if I wanted a food increase before we came back in 6 weeks that I would have to call the office.  I told her I would be calling, and I did a couple of weeks later. 

From here things went along pretty well for the next couple of months with feeding (increasing the amount of food every so often) and the stoma site had a little granulation here or there but nothing that stayed to long.   Our next big hurdle was going to be in September when the valve on the button broke.

Fluid in the ears

So it has been determined that Luke has fluid in his ears.  Right now he is getting an antibiotic to see if it will clear it up.  We will check it in 6 weeks and if there is still fluid he will be getting ear tubes.  This is a new adventure for us, one of many.  I am not sure how I feel about this.  Luke's does not swallow well becuase of his Moebius Syndrome and always seems to have a runny nose (we suspect he may have allergies) so I understand why fluid could just build up in his ears and that the tubes could really help.  He just doesn't seem to be that affected by it and so I am cautious about this.  Ear tubes are common and seem to help a lot of kids.  I guess it is just one more thing on top of the many many "things" that go with Luke.  Feel free to post comments with advice or tips.  I am all ears ;)

Real food for real people

Real food for real people... I have chosen to feed my son, who eats by g-tube, real food blended up.  I have heard many stories of speical needs kids greatly improving when moving from formula to real food diets.  Luke hasn't know any different and is doing just great.  Its not really that hard.  The hardest part is getting the nutritionist you are working with to get on board with you.  Here are some resources I have found helpful.

Blenderized Diet group on Yahoo Groups
Super Baby Foods by Ruth Yaron (Yes this is for babies who eat by mouth and are "normal" but has great nutritional info, recipes, how to blend, store, use fruits and veggies, and how to do it all in a small amount of time.)
Homemade Blenderized Diet Handbook
http://youstartwithatube.blogspot.com - a blog I recently found that I haven't even gotten to all the info yet)
Me - I love to talk about it so feel free to contact me.

An interesting note is that while at the Moebius Syndrome Conference a nurse that used to be in Australia said it was the norm to give blenderized food to g-tube fed people in the hospital.  Awesome for Australians.

NICU and dealing with the medical community tips from my perpective

So yesterday morning I was thinking of tips for parents that find themselves with NICU (Neonatal Intensive Care Unit) world.  This is not a place I wish on anyone but if you are there here are some quick thoughts from my perspective.  And remember I had a full term, rare syndrome, respiratory compromised baby.

A lot of this can apply to all sorts of situations in dealing with the medical community.

Please know that I have had very good nurses, doctors and therapists.  There are good and not so good ones out there as there is with most things.

 
-2. Communication - People would call me that I knew and some that I didn't know and they wanted to offer help or just to talk and I really didn't want to talk to people.  It was just too much, and I am an extrovert.  There are people I just didn't call back, and that is okay.  I also had my parents or husband call people or being in charge of updating family members so I didn't have to talk to as many people.  Also a great thing to do is to get a blog, Care Page, Caring Bridge page or something similar so you can post updates and progress.  People love and care about you and your family and want to know and pray for you so this is a way to help do that.  Also, allow comments, it can pick you up (even if it make you cry, again) to know about all the people that care.

-1.  Don't be afraid to cry or show your emotions whether in the NICU (we all understand) or in private.  Get it out, grieve the loss of those things that you wanted to and thought would happen when your baby was born.  It is good for you all the way around.  This can be hard.

0.  Touch and Kangaroo care - ask to be able to hold your baby and do kangaroo care as soon as it is possible.  Since I had a term baby, kangaroo care wasn't really mentioned and I wish it was. 

1.  Read your kids chart... you can find out some great info and keep up on what the doctors think.  If you can't read it ask a nurse or the doctor to come and interpret for you (you will be able to read them fairly well by the end of your stay.)

2.  Ask, ask, ask questions.  There are not stupid questions.  You may need to hear things over or clarified.  You are probably in a state of shock, stress, ect... your brain can't process it all at once usually.

3.  If a doctor, specialist is coming to see your baby and you can't be there (maybe becuase they are coming at 6 am!)  ask the nurse to have them call you after their exam.  Or leave a note on your baby's bed to have them call you.  They have done this for me and it is a great help to hear it from the doctor directly and ask them questions directly.


4.  If you want something a certain way or to make sure tubes are laying a certain way and it just isn't happending.  Tape notes around the areas you are concerned about.

5.  Don't be afraid to ask doctors, nurses, therapists, anyone who comes near your baby if they have washed their hands and ask them to go wash if they haven't.  Usually just asking prompts them to wash if they haven't.

6.  Pump your milk... mom's this is something concrete you can do for your baby.  Most NICU's have lactation consultants and the one I was in had a pumping room or you could pump at your baby's bedside.  This can be quite a chore but is well worth it.  Breastmilk is best.  Even formula cans say that they are the next best thing to breast milk.  Also, this can be a time to get away, call on the phone, or just have a moment to yourself.

7.  Trust your instincts, if you don't think something is right then ask questions to understand, bring it to the attention of the nurses (who can really get things done for you).

8.  Nurses - find nurses you like and trust and ask them to be primary nurses for you.  Getting to know your nurse for the day or night and having a relationship with them is a great way to make sure your baby is getting the best care.  Nurses can be great or so so, work on making sure you get great ones.

9.  You can say no to things, you can said wait I want to think about this or research thing.  There are things that need to be done immediately and can't wait and just have to be done yes I know.  But there are many things that you can take a little time to think about.

10.  Nutritionists and dietitians - I have not had good relationships with them as a mom wanting only breastmilk and no additives and then feeding my tube fed baby a blenderized diet made up of all real foods.  Push them to give you clear evidence that what you are doing is not working.  Check their math or get someone who is good at statistics to check their math.  They are many times not quite right with their math and that can lead to decision you don't want.

11.  In general you have to be an advocate for your child... I know you may hear this a lot, however, the doctors and dietitians are dealing with a lot of patients and their first suggestions and courses of action are usually the generalized ones.  Your child is different and unique so you need to make sure they are aware.  Don't assume they know everything in your child's file or even the obvious stuff.  Don't be shy to remind them, remember this is your child's well-being at stake.

Okay well that's all (as if it wasn't a ton) I have for now.

A start

I have been thinking for a long time about writing a book or something to get Luke's story out there for other parents of and people with Moebius to see.  So I have found a little bit of time to set this up and over the next couple of days/weeks I should be posting Luke's story here.  Stay tuned...