Moebius Syndrome and Our Family: pulmonlogy

So we had a big day yesterday visiting doctors. We ended up doing a lot of extra things. The day originally had two appointments and when his g-tube site was looking really bad and not getting better by normal methods I added a trip to the surgeon to take a look at it. We also had an order to get some blood for some allergy testing. I really don't like taking Luke to get blood drawn. He is a really hard draw, it usually involves at least 3 pokes and Luke in tears and me close to tears. I had put it off for months and finally decided since we were going down to do it at the lab that I like the best, probably because they deal with a LOT of children. So we are up to 4 places to stop now. And then on stop 2 we got a script for AFOs, braces for Luke's feet, that we needed to get molds taken of his feet to make which I decided to schedule since we were down there. Wow it was a big day. Thankfully Spectrum Health and DeVoss have consolidated many of their specialties and we only had two actually locations to go to.

So here's how it went:

Pedatric Seurgeos - stop 1

Luke has some nanstly looking granulation tisse around his g-tube site that just won't go away. The doc decided to try to cut some off and cautarize it with silver nitrate. They don't have nerve endings so this doesn't hurt. It is just hard to hold him down espeically when he doesn't like people looking at him and touching him when he thinks it is going to hurt. He did so well though that the doc cleaned the whole site up and it looks great! Onward....

So now I have to go through this building and two hospitals and into another office building (without going outside which is very nice in the winter). However, since we didn't come in time to park at the other building where we would be spending the next 3+ hours I was walking the kids and Kevin was moving the car. I don't like to be far away from the car in case we need extra, clothes, diapers, oxygen... So I had Hannah in the Boba carrier on my front, a large tool bag stuffed with kids thing over my shoulder and I was pushing the double stroller with Luke in the front and the vent sitting in the 2nd seat with the suction hanging off the back and the oxygen, pulse ox and my purse shoved in the bottom. I am sure we were a site to see. But we made it with no incident and got to our next appointment at orthopedics....

Orthodicts - stop 2

When we were at neruodevelopment last they wanted to make sure someone looked at his hips since he wasn't walking yet to make sure they were okay. Our PT said he just needed to gain more strength in his hips. Since we were at ortho we asked the doc and she got his hips x-rayed and showed us that everything was doing well and since he has neuromuscular issues that it is probably just that and he needs some more time. She did prescribe AFOs which are braces to help him with stability in his ankles. When his ankles are more stable he will be able to work on getting his muscles strength and balancing in his upper legs, hips and core in order to stand better and walk.

Luke's clubbed feet are being corrected by the Ponsetti method and doing well. His left one is still way worse than the right. The right is I think about normal now. They left one has just the amount of range of motion that they want to see. Ponsetti (or his group because he has passed away) has changed their recommendations to keep the braces on at night until he is 4 years old and not just 3 years. Also, our doctor is changing practices and so we will get another doctor next time we go. Fortunately the new doctor does almost exclusively clubbed feet. Just the doctor for us.

I must say the new offices are great. They have couches!!!! and more room for our moving NICU to get in. We were comfortable and there we weren't moving the stroller around every time someone needed to move to a new place in the room.

Blood work - attempt #1

Kevin and I were not too hungry and so we decided to get the bloodwork done before his 1:00 p.m. appointment. Well I guess a lot of people thought this because we were there for 45 minutes and finally next in line and it was time to go to pulmonology. Pulmonology will not wait and since we were first after lunch were ready for us. Fortunalty the lab said they would put us back to the top of the cue when we came back.

Oh and during this time I had to feed Hannah. Looking around the room there were two empty seats and they had people leaning over the shared arm rests from other seats. Not a good place to nurse. So I decided to go to the pulmonlogy waiting room which is huge and was fairly empty. When I walked in and told the room coordination why I was there she offered me an empty office. Bonus! Hannah is VERY easily distracted while nursing and thus far had not nursed well in the car when we stopped to feed or change Luke because she was too busy watching Luke and Kevin. So I was happy for a quite, distraction free place to nurse.

Pulmonary - Stop 3

We had a good visit to pulmonlogy. We got a blood pressure for Luke for the first time in over a year I think. Luke HATES to have his blood pressure taken. He sees the machine and goes nuts. This kid is strong. He is flails so much the machine does not read and if it does get a reading he is usually so worked up that it is not accurate. He was sitting on my lap facing me which I thought was good. The nurse was going to annouce what she was doing and I shhed her and said to just slip it on. Kevin was great and brought Hannah over and we had Luke point out the ducks on Hannah's feet and her nose and all sorts of things. He made a little fuss when it squeezed but mainly we were good to go. Luke is growing well and now down on the 40% for weight and around the 11% for height which puts his height to weight ratio at a much better place, maybe around the 50%, I didn't look real close. But he used to be 98% or more. Yikes!

We are just keeping doing what we are doing lung wise for Luke. We will need to see a cardiologist to clear him for jaw surgery this summer and if his heart looks good we can think about weaning some of his oxygen at night. Right now he sits at 98-100% almost all night on the 0.3L of oxygen he is on. The doc is hesitant right now because they saw a dip to 86% oxygen which is not good and she said there are studies that show dips in oxygen being very hard on the heart. So I understood that and we will have to see.

Blood work - attempt #2 Stop 4

So after pulmonlogy we got right into the lab for blood work. Luke is a super hard poke and he really hadn't had a nap and it was after 2 p.m. and after all the visits I saw that this could not be good. Hannah was asleep in the carrier at this point and stayed asleep through the whole thing. Me moving around, leaning down, singing, all of it. We have found that Luke's feet are the best place to get blood from him but they always have to check arms first. Kevin was holding Luke his lab and another lady and I were working on holding him still. The lady who was taking blood we have seen before. She found what for Luke is a good vein in his right arm and as she was going in he jerked and we lost it. She dug around in that arm and tried the other and then went for the foot. She said next time she will only have to try one arm. Luke is super upset. I am trying to sing "Wheels on the Bus" the only acceptable song and the song that must be sung when something is going on that Luke does not like. He requests it if he knows something is coming. But we got it done. Allergy testing, CBC and blood gas. I forgot to call yesterday with the results and so I will have to do that today.

AFO fitting - Stop 5

Since we don't go down much and I didn't want an extra trip I squeezed our AFO fitting at 4 p.m. Luke was soooo not happy about it but the therapist doing the molds was great and fast. We will have to go back in a few weeks to pick them up and make sure they fit correctly.

The car ride home was pretty uneventful. Luke did not sleep, although he must have been exhausted, but he just sat and nicely looked out the window and was just a wonderful car rider. Hannah on the other had started to loose it. Her bedtime is around 7:30 p.m. and we were past that and she hadn't had any really good naps so she was really fussy. She was so tired and just wanted to sleep in her bed I think. Poor girl. She was also awake for most of the car ride back. Normally she is a good car sleeper but I think she was just too overtired to sleep.

We are recovering and Luke just played and played yesterday, I had a lot to do and a major poop incident from Hannah and so didn't have much time to play with him (and the time I did sit down to play he wanted to watch Signing Time). He was just fine playing by himself. I think he just wanted some time away from people after all the doctor visits.

So there you have it. A very long day but I getting a lot done.

So I decided to take my 2 month old and my 2 1/2 year old on a state tour stopping to see doctors in Ann Arbor and Grand Rapids this past Wednesday. My mom has already said "I told you so" in that this was not a good idea. I in my logical, not dealing with small children in cars thought this would save us 4-5 hours of driving if we did it in two appointments. I did not think about pouring rain, a very windy day, getting 3 1/2 hours of sleep becuase my baby didn't want to go to sleep, and what would happen when shutting a teething 2 month old and active 2 1/2 year old in a car most of the day would be like. We made it and we are home. My mom went with me and at one point only an hour from home we were not sure if we were going to make it. But we did...I was sick the next day and my husband had to stay home from work to take care of the kids to top it all off. So kudos to my mom for driving through crazy weather, construction, by accidents, a crying infant and with a Luke that needed suctioning often (mostly due to the weather and dry car air) and by the end was getting upset and pulling on his trach so I was turned around in my seat suctioning him.

So why did we go on this crazy adventure? We went to a consultation with a doctor about jaw surgery for Luke. Another Moebius girl we know in our state used this doctor for jaw surgery and our ENT (we share most of our doctors) recommended him. I really like him and his resident and intern. They were friendly informative and seemed full of action. They are gathering some information from some of our other doctors and will be getting back to us with a full recommendation soon. Luke did pretty good and the doctor got a good look in his mouth while he had his hands in it so we only had to hold Luke down for a very short time for a better look. Luke does not like medical professionals in the office setting. He does not care if they are only listening to his lungs or if they are trying to get blood. It is all defenses up for it all. And his mouth is one of the worst places you can try to look.

Then we went to see our pulmonologist, for this first time since sleep study results from July were back. She did not have full results from the October sleep study, only prelimiaries so we will hear more when they come back. However, I was thinking that Luke was on the vent at night just for sleep apenea (possibly denial on my part) but our pulmonologist was clear that he still was not breathing adequately at night to be without the vent. She said that during the day he does good because a different part of his brain (I can't remember which part) can help with breathing during awake time. When Luke goes to sleep this part shuts off and leaves the breathing to the pons and medela or one or the other. These two parts are not normal in Luke's brain and have been show in the literature to be abnormal in Moebius people. She said that his breathing problems are in his brain and that there really isn't much we can do but wait and hope he grows out of them. WOW, it really hit me that I am looking at Luke on the vent for sleeping long term, but not necessarily forever, and that the trach will be in long term as well. I do believe God could work a miralcle and things could change shortly but we forge on ahead with long term ventilation for Luke.

It makes me sad as I don't want my baby boy hooked to machines when he sleep and have to breathe through his neck and all the extra care that goes with that. I want him to be as normal as possible. I am working on really coming to terms with that but I am not sure I am there yet.

Luke however, is forging ahead. He is now pulling himself to stand against furniture. He walks along the furniture as well. He is crawling fast all over and is trying to stand on his own. He did have more stitches due to an accident with his walker, gravity and his glasses but seems no worse for the wear.

Hannah is 2 months old and doing great. She is teething (my family teeths early) and so has become a fussier baby. She is sleeping through the night though, once she gets asleep. She is smiling a lot and babbles and I think I have even heard her giggle. She is generally a happy baby and loves to watch Luke do whatever Luke is doing. She was baptized a couple of weeks ago and all sorts of family came to see her and Luke. We had a great time.

Here are some not so current pics, but more current than the previous ones...