Why would a baby need a feeding tube?

Thought I'd dedicate a blog entry to feeding tubes, what they're all about and why Jameson needs one...

People occasionally ask me questions like, "so how long is he going to need that?" "why can't he eat normally?" etc, and my response is usually pretty simple...I'm not sure. That can be difficult for people to understand I think...they don't quite understand why a baby wouldn't just take a bottle in their mouth and grow like normal. Unfortunately, it's not that way for everyone. The act of feeding consists of three movements inside the mouth...suck, swallow, breathe, suck, swallow, breathe. Seems easy enough, but when one or more of those things doesn't work correctly it can be extremely difficult. For Jameson, it was the breathing. An infant's airway is obviously very small, maybe as wide as a nickel...Jameson's was about half of that. This was due to a lot of anatomical features including an underdeveloped jaw and laryngomalacia. (The upper larynx is so soft and unsteady that it collapses onto itself when he breathes.) This is where that squeak noise comes from when he breathes, it's called stridor.
After a couple weeks of trying to bottle feed with him and one incident of him not breathing and turning blue he was admitted to the hospital where he stayed for two months and got his feeding tube. There are a few different kinds of tubes out there, Jameson has a G tube which is a small tube that goes directly into his stomach. On the outside of his stomach is what is called a button. It's a covering for the tube that you can open and close as needed. Jameson has what is called a Bard button, which is the smallest and most discrete. When closed you can't even see it under his clothes.

Jameson eats through his tube every 3 hours for about 50 minutes. He takes only about 3 1/2 oz of food which is not much but all his small stomach can take. When we first were told about the tube, we were optimistic thinking he would only need it for 6 months while he grew...but it didn't take long to realize it wouldn't be that simple. Not only does he need to learn how to use his mouth for eating, seeing as how he never truly learned it, but we have to work with him on what it is to even be hungry. Obviously when you're eating every 3 hours for an hour at a time, you don't get the same hunger cues as a normal person would get. All of that needs to be worked on with his speech therapist in 2014 before answers on when he can get off the feeding tube can even be thought about.

I hope this answered a few questions people had on why, how, and what the feeding tube is all about. We are all more than willing to talk to anyone about it or teach someone that wants to learn. Thanks to everyone again for the support and love!

Updates before end of year

Hope everyone had a great holidays! Thought I'd share some updates with our friends and family before the end of the year. On Christmas Eve Jameson's nurse Kelly, who stops by once a week to check his weight and vitals, weighed our little man in at 13 lbs 3 oz!! This was great news as he's been stuck in the 12 lb range for a while now so we were excited for this bump. Anyone who has seen Jameson would definitely say he looks healthy and chubby like any other baby even though technically he has never been on a growth chart. He's consistently been at <1% for all measurements since he was born, but he's proportionate which is what the doctors seem to care about. When we got his official CDLS diagnosis, we found out they actually have their own growth chart and Jameson is at the 95th percentile! Greatest present you could ask for!!!

In other news, we are officially scheduled for our helmet on new years eve. I'll take Jameson down to Hanger Prosthetics around 1 PM and we'll be getting the final fitting and taking the helmet home with us. From there, it'll be quite an adjustment at first. One hour on, one hour off and so on until he is wearing it 23 hours a day. It will definitely be hard to watch him struggle with it in the beginning, but I know the more vigilant we are about wearing it, the less time he'll have to wear it in the long run. And its not like we get a second chance to do this, babies heads will only be soft and pliable enough for the first year to be able to do this adjustment, so this is our only shot at it. Make sure to send us prayers for strength so we can help little man get through the first couple weeks!

Wishing everyone a wonderful new years and will give more updates and pictures when we get our helmet. :)

What is CDLS?

Cornelia de Lange syndrome is a genetic disorder present from birth. It is caused by gene mutations. The proteins produced by these specific genes play important roles in directing development before birth. Within cells, these proteins help regulate the structure and organization of chromosomes and are involved in the repair of damaged DNA. They also regulate the activity of certain genes in developing limbs, face, and other parts of the body. 

 About 60% of people with CDLS have a gene mutation called NIPBL, these can be some of the most severe cases. Jameson tested negative for NIPBL while in the hospital. We've been told that about 20-25% of cases are from an unknown genetic source. The main reason for this is because CDLS is not something inherited, it is a random genetic occurrence that happens early on during pregnancy. 

As far as living with CDLS, there is such a large range of severity. Some children and adults have difficulty with everyday tasks and have major health concerns while others have slight delay and function quite normally. Most all have communication delays, especially with the prevalence of hearing issues. Jameson also has common traits of CDLS such as his facial features, lots of hair, GERD which is just severe reflux, difficulty gaining weight, and feeding problems. We don't know all the answers as far as developmental delays yet but we hope for the best. Autistic tendencies can be common but with his amazing eye contact we're hopeful that it will be on the less severe side. 

We have all the faith in the world that Jameson will get to live a happy and full life much like any other child. We appreciate your support with following our journey and kind words of encouragement. As he gets bigger and stronger I hope to be able to bring him out into the world more so people can meet him. I know how difficult it can be understanding something like this from the outside. None of our family ever expected or knew how to deal with this. To think this time last year when I was 4 months pregnant that we would be taking this little angel to every kind of doctor and specialist there was, we would've never believed it. And yet, when God gives you something like this you find strength and understanding you didn't even know you had.

Love to everyone and thanks for being an amazing group of family and friends.