Out Of The Box, Into Our Own Unique Box

Ever since Nicky has started having trouble with the g-tube we had to change our whole routine. While some are small things, the biggest change has been how much weaker he’s become, so, instead of him coming to the couch to change bandages, I go to him. He spends his day at the computer chair, so I grab the bandages and I sit next to him while I change the arm, leg, or whatever.

He’s weak because he’s lost so much weight, but also because moving is painful. He’s been moving less and less over the years. When you don’t want to move much, it affects your joints and your muscles. The fact that his feet are mega-sensitive and he can’t really walk on them doesn’t help any. When he does walk he walks (with the help of his walker) on his tippy-toes, which are extremely well padded. An “out of the box” decision he made long ago. It seems our life is full of “out of the box” decisions.

When Nicky was born I craved information like oxygen. And I found little to none. This was in 1996, the Internet was in its infancy, there was no facebook and not too many people had email yet. I wanted to find out how I could deal with what nature had denied my son, something everyone else took for granted. I couldn’t pick him up, nor tightly hug my son. I needed some help, some hope.

In the beginning I was angry at the unfairness of my child’s suffering, at the very thought of his mortality, at the loss of a normal family life, at the rest of the world for going on normally. When Nicky was a baby I would go to sleep hoping to wake up in the morning to find that this was all just a bad dream, or at the very least an episode of the Twilight Zone. I felt the weight of the world on my shoulders. I could do my best to make sure the wounds healed, but beyond that, I couldn’t do much. My priority was putting out fires, then and now.

It’s hard to realize that every person dealing with Nicky was (and is) a rookie because no one, not even the professionals in the NICU had ever dealt directly with EB. For a long time I prayed for anything, even just a set of words I could Google (or whatever search engine was available back then, I forget), a even just a clue to the right questions to ask to the correct person. It took a while to get comfortable with discarding advice that didn’t work and find our own solutions to our own unique situation.

This is why I find it interesting how every now and again there is someone hell bent in telling me they know Nicky’s skin more than I do. Right! This could be from Doctors, Nurses and, sadly, a lot from other EB families. I have gotten judged like this often, and it hurts, because the reason we do what we do is because we have exhausted all the other options, always at Nicky’s expense and pain. We had to find our own “box” of things that work for us. Every EB family/patient has their own box. Trust me.

One thing we have been told countless times is that we wrap too much. If only! We don’t like wrapping, we don’t want to wrap, but we saw what horrible things happen when we don’t. Nicky’s genetic mutation is apparently different than most. Look, I understand some patients do better without bandages. I wish that was Nicky. I want that to be Nicky, I do not enjoy spending time EVERY SINGLE DAY changing bandages, but the reality is, we do what we have to. End of story. Obviously our “box” includes a ton of bandages. It is what it is.

For example, people often ask me why Nicky decided against doing the Bone Marrow Transplant. I even took Nicky to Minnesota so he could talk to Dr. Wagner and Dr. Tolar himself. We didn’t know if this was a good option for Nicky. We knew this was another instance of “will this fit in our box”? We came up with a ton of questions for them and even had to wait a year before the insurance company approved a simple “counsel”, and we even had to pay for the trip.
At the time (2010) this procedure, done without a “sibling match” had just started and the results were not great. A little girl had just died, another suffered major complications, yet another had to do it twice and it never took. A BMT is not a walk in the park either, it would be painful, costly, and take us away from home for many months… even years. One of Nicky’s EB friends from camp was one of the first to try it in 2010 and developed GVHD (Graft Versus Host Disease). He died 18 months later.
Dr. Wagner told us explicitly that the BMTs for EB were still in the beginning stages back then and overall they worked better on “little kids”, not teenagers, and even less for older patients.
My job was just to take Nicky to Minnesota so he could make a decision. He was 14 years old at the time, old enough to make this type of important decision about his life. This is one of the reasons, among many (I fully explained our whole decision process in the book), that he decided not to do this then, but we had also decided to, as we also said back then, not to “close the door”, just to keep it ajar and wait and see if the procedure became safer, better and easier, let alone with better odds of success. There have been quite a few since then that have successful. They have not cured the condition, mind you, just improved it a bit, which is still exciting.

Nicky is still not convinced this is for him, however. I doubt he will change his mind. He’s now in his 20s, so the chances of the procedure working as well as it did/does for little kids are quite small. It’s a big risk as well, one he’s not willing to take. There are other studies out there, trials that are not risky at all, and we’re waiting for those to come to fruition. I believed, and I still do, that if Nicky can just hang on long enough, science might outrun his disease.

Love & Light,