Blog · June 30, 2013

Nobody Knows Your Child Like You Do!

I was deep into reading a wonderful book wrote by a local mother whose daughter had brain cancer and something she said hit me like a ton of bricks. And not in a good way. Without admittedly doing any research on her own, or talking with other parents in similar situations or consulting with any other Doctor, she put her daughter’s life in the hands of just one Doctor. When he turned out to be wrong, the unthinkable happened, and only a few months later her daughter died in her arms.

Perhaps is the rarity of EB, or the fact that I am yet to find another patient who is like Nicky (similar.. yes, like him, no) or perhaps the conflicting, often absurd advice I have gotten from the Medical Community, the fact remains that long ago I decided I could never rely on Doctors’ recommendations or advice and had to make the best decisions upon personal research.
Doctors mean well, I know, but when I sit in a room full of interns and a Doctor is asking me a million questions, which turns into me teaching THEM about EB and I *still* have to pay a co-pay, things can be irritating. Why bother?


Nicky a few months after his gtube placement. He had gained 10lb and looked amazing!!!

One of the most grueling decisions I have ever made regarding Nicky’s care was the placement of his gtube. Doctors could not even advise me on this one. I was on my own. The Pediatrician was concerned for sure, Nicky had only gained 3lb in over 2 years. At 9 months he weighed 18lb and by the time he was 3 years old he only weighed 21lb! But the weight was only a minuscule part of the equation. His height growth had fallen below the charts and his head circumference was falling behind as well, FAST. There was no time to wait around, I had to make a decision.
Nicky had a hard time eating. On a typical day he took at least an hour to finish one of his bottles because apparently his throat was compromised and strictures prevented fluid from going down fast. He would wake up every morning with his pillow drenched in saliva. He was still wearing a bib to catch all the saliva he spit during the day. I knew he needed a throat dilatation for SURE, but I was also pondering the g-tube. What made that decision more important than ever was the constant news of teenagers with EB dying of either kidney or heart failure, caused by malnutrition. These teenagers did not have gtubes and spent grueling hours eating, and obviously not enough. I was told that particularly during puberty, if the patient was not going to get enough calories, the internal organs would suffer greatly. Other parents who put the gtube in told me what a blessing it was and how their children finally gained all the weight they needed to prevent illness and allow for wound healing. As I was told by Dr. McGuire at Stanford, in EB children, calories go to wound healing first, then head/brain growth, then height, finally weight. “A skinny child is not necessary malnourished unless his height growth is compromised” he told me once.

Finally, in January of 2000 I requested both a throat dilatation and a g-tube placement, to be done at the same time. The surgery, which was done in March, went well, his throat had a 1mm stricture, no wonder he had trouble swallowing! Within 3 months Nicky gained 10lb! It was an absolute miracle…

Did I get negativity thrown at me because of this? Indeed. Just imagine what could have been said to me, and consider it said. I was called every kind of ‘awful’ mother you can think of, people said Nicky looked ‘fine’ and did not need need a ‘hole’ in his stomach, and the list goes on. Whatever. I am the one that knows him like no-one else.
This also goes with the bandaging. I lost count of the times I was told I was ‘over-bandaging’ him. Doctors told me this, onlookers who know nothing about EB told me this, or other EB parents. The most irritating one was from a man with a few RDEB kids which were barely bandaged, and, sadly for me to see and say, barely moved. They just ‘sat there’, with vasts amount of skin exposed and looked like statues. I never told him he should bandage them more to allow them to be more free in movement,  perhaps not so afraid to get hurt, I mean, if he felt that was best for his sons, who am I to tell him otherwise? I kept my feelings to myself, but he surely let me have it-indirectly of course. Not only to me, but to a couple of other moms who bandaged like I did. According to him we were ‘killing’ our children with the “over-bandaging” as he called it! In the meantime, our kids walked and moved while his children did not. Hmmm…


Nicky holding Connor on Xmas Day with his legs super-extra bandaged. Hey, it worked!

I know my son like no-one else. I know what he can tolerate and what he cannot. I know what’s important to him and what is not. I know how his skin blisters and how much damage he can inflict on himself with scratching. When he was 7 years old, for example, I could not get both of his legs-the part between the ankle and the knee-to heal. They had been an open wound for almost 2 years. He would scratch them to death and were not going to heal no matter what. One day when his legs were starting to heal, to my horror, he had scratched them all open again. It was a time when Connor was a baby and I was not getting any sleep to begin with, and this threw me into a fizzy. I don’t remember being this MAD, ever. I needed to find a way to stop him from hurting himself. As I re-wrapped him, I covered the legs, just the part between the ankles and the knees-with two rolls of webril and two rolls of kerlix each! Oh yeah, he was absolutely overly, overly wrapped, but there was not going to be a flipping way he was going to be able to scratch anything. Within 4 months, his legs were completely healed. I kept them wrapped this way for a little while, until the itching subsided, and then I still used a roll of webril for protection and they rarely get a wound.

Padding for Nicky works. He himself gets mad at me now that he is 16 and tells me if I did not wrap ‘enough’. To hell with the ‘skin needs to breathe’ BS line we’ve been fed all this time. To us, that is not the answer. For someone else, maybe, but not for us.

So, moral of the story? Follow your gut, do your own research, do what is good for YOU and YOUR unique child. And… never take a Doctor’s word as gospel. They don’t know your child like you do.

Hugs and Blessings!