I Love a Tubie!

This is the motto of a website I stumbled upon the other day. It is called Feeding Tube Awareness. I especially love their Facebook page!
It is good for me to see all the mom's dealing with feeding tubes for a million different reasons. I don't live near anyone I know that has a child with a feeding tube. It is so frustrating some times because there is so much I don't know. I've already learned tons just by reading what others do. It has helped me realize I need to be more proactive with my insurance. I also enjoy that they discuss eating problem, suggesting foods and ways to encourage eating.
I don't know if there are any others affected by Glycogen Storage Disease, but I hope to find out soon.

Awesome tube accessory!

I really wish I had found these about 9 months ago!
When Evan had his NG tube I always hated the tape on his face, I think besides the tube in his nose the tape made people stare just as much. These cute little "stickers" made from special medical tape would have made the experience a little more whimsical!
I'm currently working on trying to make my Sweetie Pie a band for his belly to secure his Gtube and extension better.  If I can't get it right, I may just have to get some of these for his tummy!

The website has lots of things that help medical visits/procedures/stays a little more enjoyable for little ones.
Kid's Hope Chest

"Do not fear your destiny"

Last week I read a beautiful email. I wanted to share something it read.

It was written by a man from Brazil named Ismael Portilho. I do not know him personally but he posts often on a email list I belong to for Glycogen Storage Diseases.
He has Type II, known as Pompe Disease.  Pompe was recently made a little more famous because it was the topic of the movie Extraordinary Measures a couple of years ago.  This disease, while in the same "family" as my Sweetie Pie's, has its own myriad of symptoms and side affects that are not at all the same as what Evan experiences.
The subject of dealing with disease and how it affects life, and our feelings was being discussed, also how family members deal with it as well. Mr. Portilho was kind enough to share his father's kind and wise words that he gave to him to help him face the disease he'd been diagnosed with.


' "Do not fear your destiny", he said, "If you'll be bound to a bed or if you'll be cured, be happy for the life you'll have. Do not tie your happiness to things that are not on your hands. These are God's realm and the world's capricious designs. Do whatever you can to be happy, and rest easy on your work." '

I found these gentle words of this father to be so moving and touching that I was brought to tears. These
are words that I believe anyone could benefit from in any trial in their life.
I pray that as time goes on I can provide that comfort and wisdom to my child when he comes to me with tears in his eyes and the questions of "Why?" on  his lips. That I can give him the strength and presence of mind to go on and live when he may want to give up.

For now, I needed that encouragement. I'm so glad that Mr. Portilho shared this private moment. Many parents of children with autosomal recessive genetic diseases or other genetically passed diseases live with some sort of guilt for passing a burden on to their child. I personally haven't felt that guilt. I do have other feelings that I have not yet been ready to or able to form into words. For now I am just coping. I feel that fog lifting though as I get better at not fearing my nor Evan's destiny.
 

Over-night cornstarch

Well it's been about a week and I am loving the overnight cornstarch.

My Sweetie Pie is sleeping through the night!!! He has never done this before in his whole life.  I'm so happy for him and us, I can sleep in my own bed again!

My hubby and I are taking turns giving his feedings at night and that helps with the sleep. We are able to each get about a 5 hour block of uninterrupted sleep. I will admit though, that have this little worry in the back of my mind that I will sleep through the alarm and back up alarm and it makes for a light sleep. I've also gotten up and had everything prepared before I realized I was an hour early. *sigh* Better early than never, I guess.

Evan's blood sugar are seeming to do better in the morning now too. Before, he was having a hard time in the morning when we would transistion from pump to cornstarch. He would always drop really fast- as much as 20-25 "points" in a 1/2 hour. I thought it was only the pump causing this but it seems that he just has a pattern of low blood sugar first thing in the morning and so I made some adjustments and he is maintaining well.


 So with this hurdle crossed, now on to eating food- with his mouth. And figuring out this crazy diet!

Halleujah!!!

Tommorrow night Evan will sleep without his feeding pump!

I am really so excited for many reason.

I'm hoping it will mean that he will sleep through the night. He hasn't really ever done that since he was born. The pump made it more difficult because he would get caught up in it and that would disturb him.

It will be safer. I don't have to worry about him opening up the ports and it leaking everywhere in the night. It has happened a few times and luckily we caught it in time to boost his blood sugar back up before it was too severe of a problem.

Yes, we will still have to get up a couple times in the to do a feeding of formula and cornstarch but it is so much more worth it to me knowing there is one less thing to worry about.

So here is to waking up to mulitple alarms and hoping I'm still happy that I fought for this change.

We will see in a week how I still feel about it :)

Speaking of alarms, there goes one right now...

Upon diagnosis, Evan was given a nasogastric tube. This is a small tube that is threaded through the nose and into the stomach. I imagine they are very irritating. It was a nightmare because he pulled it out all the time and it was connected to a 24hour continuous drip feeding pump. Yea, try that with a baby who wants to crawl and walk- NIGHTMARE!!!
 

We would have to replace it every time it came out. It was traumatic for him and us.  I think in some ways it was traumatic to anyone having to look at him too. Little kids always wanted to know what it was and were so concerned and careful.

We are so happy his belly isn't this distended any more :)

In August of 2010,  my sweetie pie had a gastrostomy. This procedure was necessary because he refused anything orally at the time (except breastfeeding). Breastmilk was not the best food choice for him. With this device placed we were also able to get away from that feeding pump during the day and begin his cornstarch treatments.



In this procedure, the surgeons move the stomach up to the abdominial wall and stitch there. His stomach will stay attached there, forever. If I understood correctly it is done by passing a laproscopic tube through the esophagus and attaching it 'blindly' from the inside. I believe, this is the "easy" way. Evan's procedure was a little different. His liver at that time took up a majority of his abdominal cavity. His stomach was being blocked by the liver and intestines. He had to have a little extra planning done to ensure that no damage was done to those organs. Some children needing this tube have to have a lot more rearranging  done to their organs, we were fortunate not to have this complication.

The stomach is stitched permanently so that should it when it gets pulled out or needs to be changed, it can be in the right place and not slip into the abdominal cavity causing major complications. A hole is placed, called a stoma, to provide a place for a catheter or "button". This device his held in place by a small water balloon. It can deflate over time needs to be replaced when it has worn out or it has become too small as he will grow.  There is some daily care involved with keeping in clean and in place but over time it becomes easier to handle/ deal with.

When we no longer need it, it about 10-12 years I'm guessing, We can just take it out and it should close up on it's own.

A friend of mine from Jr High/ High School, that I have become reaquainted with through facebook, reached out to me when she found out that Evan would need a feeding tube. Her daughter had one for years, for other reasons. She is a a miracle baby that survived birth at 25weeks and her story is amazing. You can read her story here.

This little girl recently got her gtube removed. All appeared to be closing well until one day her parents noticed a strange wet spot on her shirt. The stoma appears not to have closed all the way on her and she will be need a small surgery to repair it. I think that with Evan having his for so long he will most likely have to use this procedure as well.

Positive Steps

My sweetie pie has been making some positive steps in his eating habits.
Im so excited to see him actually exhibit hunger, he hasnt really done that since being placed on a feeding tube in June. Somtimes he wont let me out of the kitchen!
He isn't eating full meals but hes eating more while he grazes. One night he ate 3 chicken nuggets in a row. Chicken nuggets, let alone 3 had otherwise been unheard of.
He is also experimenting with more textures. At Christmas I made him some sugar free pudding with rice milk. He ate a few bites cautiously. Previously this texture would have made him gag and vomit.
I also have been encouraging him to drink his cornstarch. He's been drinking about an ounce of it and then refusing at which point I put the other two oz through his tube.
All of these things are making me hopeful that he will soon be able to get off of all his formula during the day.

He also has been enjoying carrying a carrot around to gnaw on.