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. 

Good news are the best news

Great news today! Jameson had his first eye doctor appointment and we were given the all clear. No major anomalies or issues that the doctor was able to see. its beyond amazing to get news like this when sometimes it can feel like you're always getting the short stick.

As far as the light sensitivity goes, it sounds like something he will hopefully grow out of...and in the meantime many cool baby sunglasses need to be purchased.

Next weeks agenda will be getting Jameson's helmet and getting him adjusted to that. We've also started signing with him daily, just starting with a handful or two and will work more in as we go along. Its been a fun process especially thinking about him signing back to us someday. Thanks again everyone for the support, we are so grateful!!

Helmet time!

Tomorrow is the big day! Jameson is getting fitted for his cranial helmet. Believe me, when I first heard of this helmet I was like no way we want to avoid that at all costs. But unfortunately after 4 long months of weekly physical therapy for little man and not enough progress on head/neck control, we've come to terms with the helmet being our best option.  Not only is it meant to change the look of his head cosmetically, making it less flat on the one side, but will prevent any changes is jaw and facial structure in the future.

The problem really seemed to start with our long hospital stay, where the pediatric wing's crib only opened one direction. That lead to Jameson always being faced one direction which we didn't think much of while there since our minds were on a lot of other things. By the time we really noticed his head preference to the right it almost was too late. With his torticollis, even the physical therapy did little to help. So here we are heading towards the end of 2013 and ready to start our helmet journey for the next 4-6 months. We've already ordered lots of cute stickers and we're definitely making the most of it!

Hope everyone's doing well, thanks for checking up on us!

Where we are today

As most of you know by now, Jameson was diagnosed with Cornelia de Lange syndrome officially on 12/5/2013.  When they first mentioned this disorder to us they took blood samples in order to see if Jameson had the most common gene mutation called NIPBL. We got word in June that he did not have that exact gene mutation. There are two others they want to test him for right away, SMC1A and SMC3. Those only account for another 6-10% of cases, so we still don't necessarily have a lot of answers.

We've been contacted by the Children's hospital of Philadelphia who has the largest Cornelia de Lange syndrome department in the world and they want to put Jameson in a research study for children with CDLS that didn't test positive for the genetic side of it. All of us really want to make a trip to Philadelphia in the upcoming year so Jameson can see their specialists and hopefully get even more information to help our doctors here. I will say however that the doctors and therapists we've dealt with in central Iowa have been amazing. Jameson has seen numerous specialists so far and they've all been very helpful and they work together seamlessly to where it really feels like you have a team behind you. 

As far as the future goes, we have a long ways to go. Jameson is being fitted for a cranial helmet this week to help with his plagiocephaly and torticollis. We also see eye doctors next week to work with him on his sensitivity to light. We also still have many more specialists to see including a GI doctor to work on his tube feeding and gastrointestinal issues....a neurologist so we can get an MRI done and look at some brain issues they've seen on ultrasounds...and we're still continually working with our ENT Dr Young on his hearing and airway issues. I've recently invested in some sign language books so that our family can prepare to communicate with Jameson if it does come down to him being completely or partially deaf. 

All in all, we still have a long journey ahead full of exciting adventures and setbacks I'm sure. Either way we invite everyone to join us along the way and help us support this little angel is every way possible!

The adventure so far...

Much like the famous Alice in Wonderland classic, we too have gone on a crazy adventure with our Jameson. I decided to put all of our thoughts and feelings into written words and share them with our friends and family. This way everyone can know exactly what is going on with Jameson currently and what adventures we still have to go on because there will be many! Thank you all for the support and love through this journey!