This is from http://epidermolysis-bullosa.com/Understand-the-pathology/Dystrophic-EB-DEB:
The Hallopeau-Siemens (RDEB-HS) form of EB is the most severe, since it causes major disability. It manifests initially with a general eruption of blisters, however these are predominantly localised on the extremities of the limbs. It includes dental complications and mucous lesions including oral, anal and esophageal stenosis which can cause malnutrition and retard growth. Scarring of the cutaneous lesions is slow and abnormal and is accompanied by milia, terminating in the characteristic atrophic lesions that cause disability (syndactylies, contraction when flexing the limbs). During childhood, repeated blistering and long periods of pathological scarring result in a significant handicap: it becomes more and more difficult to walk and perform everyday movements. Anal erosion makes defecation painful. Death often occurs during the first three decades of life as a result of infectious complications, malnutrition with anemia, retarded growth and even secondary amyloidosis. Spinocellular carcinoma often appears on the most affected areas of skin and mucous membrane.
So, not good news. Frankly, Cody and I were quite shocked that Gunner has the most severe subtype. BUT, the way we look at it is, Gunner has had this subtype since he was given to us by God. We are just putting it on paper and finding out now. The other kiddos and adults with EB that I have seen pictures of that have RDEB-HS as well all present differently. Some genetically have the worst subtype of EB, but only have to wrap areas that have wounds, others, have the worst subtype and have to wrap completely head to toe whether there is a wound there or not. That is the weird thing about EB. You can put two kids that have the same type AND subtype, and they will not look the same or have wounds in the same areas. All EB kids and adults are different.
Cody and I want to thank EVERY single person that contributed in one way or another to help us raise the money to get these results for our Gunner. Without the generosity of so many people, it wouldn't have happened. THANK YOU!
We had a great Easter and hope you all did too! Cody pushed Gunner around in his little blue car to hunt eggs and put them in the trunk of the car. Gunner was squealing and laughing the whole time. He kept picking up the eggs, opening them and throwing them.
We went to the Colorado Children's hospital for the EB clinic on the 3rd. We learned quite a bit. The derm team said that his skin and skin care was looking good. They looked at his feet and said that for EB feet they look good. They agreed that his nutrition is something that we are going to have to really keep a close eye on. They told us to start him on pediasure as well as still offering table foods. (He is doing better and trying most things that are puree consistency of course.) They said that pediasure will is the last resort as far as getting him to gain enough weight on his own. This is hard for him since he doesn't take a whole lot of nutrition by mouth and what he does take by formula and table food, all his calories go to wound healing first and growth second. In the next visit, since he will be on pediasure for 6 months, we will see where he is at on the growth chart and may start recommending a G-tube. Right now he is in the 5th percentile. So, we are praying he gains weight! I know he looks chunky in pictures, but he is short, so that makes him fall into a low category.
They gave us some prescriptions. Two itching meds. One for daytime and one for night (because this one will make him drowsy). Itching is important to control because Gunner's skin itches all the time day and night and even while he is sleeping. Even with no nails, he still breaks open wounds just from the rubbing motion.We also got a prescription for some numbing medicine to help with Gunner's mouth blisters and ulcers. This is going to be so helpful when he can't eat because of mouth blisters. They put him on a multi-vitamin with zinc and iron as well as a prescription for extra iron to help replace all the blood that he loses from blisters and wounds. This is important because EBer's tend to be a little anemic and need blood and iron transfusions. At last check Gunner was right under the "normal" level and technically considered anemic. They also put him on Vitamin D. He needs this because Vitamin D you get from the sun and being outside. Because he can't be in the sun much (because heat causes blisters too) and if he is in the sun, most of his body is covered in bandages, EBer's don't get the adequate amount needed on their own. They also put him back on his reflux medication. Although his reflux has slowed down a lot, he still gets it from time to time. It's important to control this because the reflux burns the esophagus and is a main cause of strictures in the throat.
The PT said his hands look great, no signs of webbing or fusing yet-so that is good. They gave him some night splints to wear to keep his foot in the 90 degree angle it needs to be in. His heels are contracting up making his foot drop down all the time. When he tries to stand, his foot doesn't stand flat. Shoes would (hopefully) fix this. But his foot had never been in a shoe, until a couple days ago! We found these pair of Adidas at the mall in Aurora, and they fit great and haven't caused any blisters yet! When we would stand Gunner up on his feet before, he would whine within seconds because it hurt to put pressure on his feet. So far with his shoes, he hasn't whined! YAY!!! He will get his kid walk Tuesday, so Cody and I are praying that with shoes, splints, and his kid walk, he will take off in no time! We can't wait!
They also said that it seems Gunner has developed a food aversion with textures and that we will have to really work with him and be patient as we keep offering new foods and help him to like textures. As far as the anxiety goes that he has been having, we were told he is too young to really do anything. To keep offering comfort for Gunner during bandage changes and all times of day and reassure him that we are doing this because we have to keep him healthy. I know with time and as he gets older, he will understand.
It is a ten and a half hour drive to and from Oklahoma to Colorado. It turns into about 11 to 11 and a half with the stopping for Gunner. This is Cody and Gunner on the way to Colorado.
And on our way back... He has beef jerky in his hand. We bit off the ends so they weren't rough thinking he would be able to suck on it and taste it. He liked the taste and sucked on it for a while, but paid the price for it in blood blisters all over his tongue and mouth afterwards... :( Mommy and daddy felt so bad..
We thankfully were blessed to stay at the Ronald McDonald House again! How awesome is that?! They are all so sweet and truly care about the people there. Here is Gunner's latest picture with Ronald McDonald! Remember how I said we were going to make this a tradition? :)
Overall it was a good and informative trip. We also got to see two other families. One at the RMH and one at the clinic. Both families were sweet. They both had boys that have RDEB as well. Both were walking and eating soft things by mouth. Cody and I were so thrilled to see that. We both literally just stared at them watching them walk and play. We believe and can't wait for that day for Gunner!
I am thinking about making Gunner a Facebook page. I have been asked ever since Gunner was pretty young if he has one or if I am going to make one. At first, I wasn't as open to sharing wound pictures and other details of Gunner and his struggles, but now I am to the point where all I want to do is educate and get awareness out there about EB so we can find the cure for all EBers. I think this will be great for awareness since most people are already on Facebook. They can "like" the page and I can share links and stories and they can share and that will just start a chain of awareness. Also, I will post updates everyday on how Gunner is and what he did, whether good or bad. Not that I will stop updating the blog, because I won't. I just feel like not everyday something happens that is "blogpost" worthy, so I wait until something "big" happens to post about, then it turns into a huge post. I feel like I will be able to post smaller, simpler posts on Facebook everyday and then post the same kinds of things on his blog. I will make a post soon with a link to Gunner's page. Thank you all so much for following our Butterfly Prince's story and all the kind words and support we receive! Can't wait to join you on Facebook soon!