Saturday, August 1, 2015

Goodness me..

 It has been FOR EVER since I wrote on Gunner's blog. A wonderful woman in the EB community helped me to set up a Facebook page for Gunner, and it has been great. I havent updated it in almost a year. Something about Facebook, (maybe it's just me) I felt like every post I made people felt I was looking for attention or something. Especially in the times that my posts would be not so cheerful. So then I changed to only updating on my personal facebook page, which I still do, but not near as much.  Everyone on my personal page is amazing, I just feel like I shove our "EB life" in their face. I know most enjoy reading updates, good and bad. But I can't help but feel deep down that I can't express the real reality. Maybe it is because Facebook automatically puts your posts in peoples faces and with the blog they can choose to click on the link that makes me feel better.

Regardless, I like to write. It's how I feel I can most express myself. I felt like I haven't been able to fully do that in a while and I miss it. So, I will be updating Gunner's blog now. Here I can openly exress myself, and that is what I need. I hope everyone is OK with that. :)

I don't even know where to start. Gunner is 3 1/2 years old now. Time FLIES. He also has a little sister! Yes! Her name is Zoey Mae King and she is a week shy of 8 months old. She was born December 8th, 2014. Her and Gunner's birthdays are only 6 days apart! Here is a current picture of my little darlings.
                                                        Gunner Ray 3 1/2 years old
                                                              Zoey Mae 8 months old
                                                                They melt my heart
They are the light of my life. We didn't go into much detail about it, but Zoey was born with a moderate to severe airway defect called Laryngomalacia or LM. It is where her airway is floppy because of some extra skin over her trachea. Thankfully, it is usually outgrown by age 2. She is already (thanks to God) pretty much outgrown it. She had so many feeding issues when she was born all the way up to 6 1/2 months old along with reflux and belly issues. It was a bumpy start but this girl is eating so much these days, and she can pack it away! She was born at 37 weeks gestation, I was in the hospital twice with pre-term labor, but she is a healthy little lady who definitely keeps me on my toes and likes to give me a run for my money! LOL. Due to her feeding issues at the start, she didn't gain much weight at all, plus she was born premature. She is finally gaining but is still only in the 10th percentile for weight. Her nick name around the family is "Lil Bit". Partly because she is so petite (she is 8 months and in size 2 diapers and 3-6 month clothes), but also because of her lil attitude/spunk. Her papa always says she's "a little bit hungry, then a little bit not, then a little bit happy, then a little bit mad etc etc" haha. It definitely suits her well!

Now for my Gunner. Gunner is so wise beyond his years. He is seriously the strongest, most caring, funny, sarcastic, happiest little man I know. I definitely don't say those words lightly. The hell that EB has put him through in just these short three and a half years he has been on this Earth would have made me give up such a long time ago, but not him, Every single day he continues to limp out of bed with a smile on his face and ask me, "What's for breakfast mom? I'm hungry. What are we doing today?' It makes me happy, but sad at the same time that he can wake up in such a good mood despite the condition he may be in that day, but sad at the same time because the pain and torture of this disease is just the normal and he can shrug it off as just part of the day. 
You see, EB is a progressive disease. You may think that 3 1/2 years really isn't that long of a time for EB to really do anything. But EB is brutal, it doesn't care. That is 3 1/2 years of repeated wounds and blisters over the same areas of the body. Opening and closing, forming scars and opening them right back up. Gunner requires daily oxycodone within an hour of him being awake for the day. Most days, he requires a second dose late afternoon/early evening. His wounds are bigger, drain more, are harder to heal, get infected right off the bat, and require more dressings than ever before. If I said bath time was a nightmare before, it haunts me now. Gunner has so much anxiety that he screams from the top of his lungs to stop the minute you calmly tell him it is bath time. (And that is even with him on versed before baths for anxiety). The things he says to me during the whole process breaks me down emotionally, but that is how he handles his pain. He doesn't complain about it. He gets angry and takes it out on me or whoever. And rightfully so. I try so hard to hold my composure but it is getting more and more impossible. I hold in my emotions about it in a lot more than I should, I talk to Cody about it ofcourse and I always feel better when I do, but there are some things I don't like to bother him with.
Gunner's hands are almost non-existent. He is 3 1/2 with almost no hands.I am trying my hardest to contine to wrap his hands to save what I can of his thumb webspace but it's almost impossible. His hands are still fusing and still curling. On both hands he only has his webspace between his thumb and pointer finger and it is really narrow itself. Less than the width of a pencil. I often hear him get frustrated when he can't pick up or play with his big monster trucks that he has. Or when he drops something hes so proud of carrying because he has to 'hold" things with two fists. He often asks Cody and I "Why are my hands squished? My hands are locked can you get the key?" It is the most heartbreaking thing to see your child staring at his hands and you see the realization on his face that they won't do what he needs or wants them to do. Our last clinic visit to Colorado was the beginning of July. The PT there at the clinic suggested that we make a consultation appointment with a hand surgeon there close to the hospital for a thumb and pointer finger release so he can at least have that grasp. I bet your wondering why not the whole hand. For one, hand release surgery has a lot of involvement. Not only are they sepearating that skin that was webbed for so long, but tey also have to straighten those fingers that have been curled down for years. It is extremely painful and a long process., Often people who have the surgery request to have the first dressing change done under anesthesia because the pain is too much to bear. Keep in mind this is coming from people who deal with pain from wounds like 3rd degree burns daily. Also, the hand surgeries often times need to be repeated. So they get their fingers straightened (or as straight as the surgeon could get them) theyve done the dressing change the cast is off, their hand is straight. They either don't have much improved function of their fingers because they are still tight, or they have improved function but their fingers are curling down again. The "fixed" fingers are still EB fingers. They still get wounds are more scars and more damage. Over time they curl again. Which is the main reason most surgeons won't do the whole hand. So, hand surgery is painful, time consuming and often times has to be repeated down the road. We figure we will at least see what the surgeon recommends and then go from there. We saw a hand splint specialist about a year ago when we first started noticing his fingers curling badly, but they didnt help what so ever. They only made more blisters and wounds.The compression gloves were way to tight of an opening to get on him without causing shearing to his skin, and even if we found some we could get on, his fingers were so curled they wouldn't go into the finger slots, which was the whole reason for the gloves anyway. We were told this before a long time ago that his hands would fuse, especially having RDEB-HS, it was and is my worst fear. And it is happening.  
Some good news: Gunner is walking! He has been walking for an entire year now! He is still unable to wear shoes, while we have found some that will fit on him (his foot makes the right angle like he has an ankle now due to him being able to bear weight on his feet) every pair rubs blisters. So, it is always hit and miss on if he will be able to wear shoes that day or not. And most often than not, he isn't able to. So since he isn't able to wear shoes most days, means he cant walk anywhere but inside the house or inside somewhere that has tile or carpet. We still have to carry him when he is outside or if the ground is rocky and wet etc. Even when he does walk, he doesn't have just a whole lot of endurance on his little feet. He always ends up wanting to be carried or put in the buggy about half way through the trip. Not to mention when we are at a public place with a lot of people, even on the rare chance he is able to walk there, you wouldn't believe the amount of people who don't pay attention and bump in to him and knock him down (which is obviously a HUGE deal blood everywhere, skin missing, etc). So,we applied for and received two handicap parking placards for when we do find somewhere he is able to walk, he can be close to the door. So that brings me to my next happy news:
Gunner will be getting a power chair! He isn't seen by PT weekly anymore, but we do see her monthly or so for check-ins. Due to all the reasons above (and the fact he will be starting school! Yes! more on that soon!) we decided he needs a power chair. Let me tell ya, this kiddo is so smart. It comes natural to him! He maneuvers that thing like a pro! We have been able to test out a couple different types of chairs and we found the one that best suits him. Now we need to submit to insurance and I am praying that they approve it and FAST! 
 While we trialed one of the chairs for two weeks, we let Gunner ride it around the neighborhood
We also went to the aquarium around more people and he trialed it there too :) 

Gunner starts the pre-school 3 year old program in 19 days! Yes! We have found a school that is absolutely amazing. It is small enough that they will be able to oversee Gunner a little more and able to help him with all he needs. His two teachers are amazing. We have already been there twice to speak with them and they have willingly looked up EB and all that it entails on their own to learn more about how to help Gunner. (Even other teachers in the school have as well!) He will only go half a day to start out so that he can get used to it and we can figure out how long he can handle it. Plus, we will do is tube feedings at home in the mornings and at lunch time when we pick him up. (Gunner got a g-tube put in back in August of last year due to not gaining a lot of weight and not being able to eat much by mouth due to blisters and wounds in his mouth and esophagus which cause pain and narrowing in his throat. We have been having to travel back and forth to Colorado every 4 months or so to get his throat dilated under general anesthesia to open up his esophagus so that he can eat better.) He gets all his primary nutrition through his g-tube and eats his favorites/soft foods by mouth still. The school is so flexible with all we need them to do and is already cooperating with us on Gunner's schedule. They know it will be likely that he will miss more than the alotted "sick days" for a typical child and understand we travel to and from Colorado often. 

We have a video that the Colorado clinic gave to us a while back that was made by the EB clinic staff that explains to classmates and teachers about EB. It is really helpful. It tells the kids proper ways of how to treat their friend with EB. Which is just like anyone else, but be easy ofcourse. :) We also have some pamphlets we will be sending to the school for the teachers to send home with the other kids explaining EB if their kids come home with questions, etc. The school also has a special "Meet my family" acitivity that they do for every kiddo where the kid can show off things about themselves and their family, the teachers said we would probably be first so that the kids can learn about Gun right off the bat. They are really going above and beyond getting special supplies that they may need to help Gunner and protect Gunner in the classroom. (Like things to help him learn to write even those his hands are fused, things to keep shelves and toy sides not so sharp to where he can bump into them, etc). We are obviously very nervous, but please join us in prayer that it all goes well and smooth and Gunner just loves it! Thank you! He is so smart, and Cody and I feel like if we didn't let him go and we didn't give public school a good honest shot, that we would be hindering him. He thrives being around people and I just know he will do amazing. 
Because dressing changes are getting to be so long and so traumatic for Gunner and now that we have Zoey to take care of as well, Cody and I have been weighing the options of getting a nurse in to our home to do dressing changes. I think we have finally come to the conclusion that we are wanting one. We are waiting to be set up for an evaluation to see if he will qualify (which with one observation of dressing changes and meds I know they will qualify him) and once that is done they will send it to insurance for them to approve or deny. I'm praying this is smooth, and we can get one in soon. This would take so much stress off of Cody and I, us be able to tend to Zoey more promptly, and most importantly, easier on Gunner in the sense that mommy and daddy aren't "doing this to him" as he says now. We can tell he is starting to resent us in a way and it breaks my heart. I am praying that this is the right decision and it goes well. It was not an easy thing for Cody or I one to decide to do. I mean we have been doing dressings on our own for 3 1/2 years. We have a specific way we do everything and Gun is used to that too. So any change in routine is also traumatic for him. I'm just praying this is the right thing for us all.

Last summer, Debra of America (one of the bigger foundations that helps EB families) put together a patient care conference in Nashville, TN. It was held at the Gaylord Opryland Hotel. That place is amazing! It is like a little town in the inside. There were trees, waterfalls, even a river with a boat in it that we rode through all indoors and climate controlled, so absolutely perfect for those with EB. We got to meet so many other EB families and us as parents got to meet the people we had been talking to and getting support from on the internet since Gunner was born. Nothing could replace that feeling. Gunner got to see and play with kiddos around his age that were just like him. We were able to hear speakers from all kinds of categories that all related to EB. Nutrition, hand surgeries, anxiety, diet, clinical trials. They did a family trip on an old showboat, we didn't even have to leave the hotel. I can't describe the feeling of happiness you get when you can walk around in public and your son doesn't get stared down or you don't get stopped every two seconds to be asked "what's wrong or what happened" to your child. We would walk to the halls of the hotel to the conference rooms and all around and we would see the backings of EB dressings everywhere. It would make me grin knowing that that could have came from anyone in that buidling! We also got to see a special screening of a documentary made by a young lady with EB who passed away before her film got finished. They finished it and it is now on iTunes for purchase. Search "Butterfly Girl". You won't regret it. Such an amazing time we had. Debra puts on the PCC every two years. The next one is in Dallas in 2016. Which is even closer than the one last year from us. We are most definitely going! We can't wait to see everyone. It will also be held at a different Gaylord hotel there in Dallas. We already can't wait!!
I feel like this isn't much as far as filling everyone in on the last two years. Especially if you didn't see the few posts that were on Gunner's Facebook page. Overall, Gunner is good. We can obviously tell that EB is starting to try and take its toll on him, but we are fighting it with all we've got. I feel like I have touched on all the "major" things that have been going on, but if I have left anything out or anyone has any questions, please feel free to ask away. Trust me, we always feel so much better educating someone rather than just letting them wonder. Thank you all for being patient, and welcome back!!