Playing: You will be in my heart by Phil Collins

Surgery Tales

ALERT!
Before you scroll down, please be forewarned that this page includes several pictures of my son's wounds and blisters. These are not pretty pictures. I am finally putting them up on his site to give family, friends, and people in general an idea of how brutal EB is to my son and to every child afflicted with this dreadful condition.
Thank you for understanding.

It is incomprehensible to me sometimes when I really sit down and think about it, all the pain and heartache Nicky has been through in his short life. Certainly a million times more than my 4 decades on this planet. Who can forget his painful few days after his birth, the colic, the endless sores, the falls, the blood, the baths... but what has to be more heart wrenching than anything, has to be the surgeries he had to go through. Surgeries that were heart wrenching to decide to do and live through, but that ultimately made a *HUGE* difference in his health and quality of life.

His first surgery was the most traumatic. It was performed at St. Luke’s Medical Center in Phoenix shortly before his 2nd birthday in November 1998 on his right hand. The anesthesiologist truly did not “grasp” the concept of fragility in EB (no matter how much I explained it to him), and that is what made this such a horrible procedure to recover from. The surgery itself went fine, all it consisted was in taking “off” all the bad skin on his entire hand. Painful? Oh boy. Especially since he could not eat for a week because of the “nice” job the anesthesiologist did damaging his throat. Nicky was left in much worse shape than ever before. He threw up blood for several days, and did not sleep for close to a week as a result from it.

I tended Nicky's hand every other day or so by bandaging the fingers separately, keeping space between digits so they wouldn't contract.  I also gently massaged my son's hand to stimulate feeling (once they were healed enough). 
The result was that Nicky's fingers looked strong and straight when they finally healed, and I knew he had benefited from my constant post-surgery attention.
It wasn't easy, though, as I had nobody to help me keeping Nicky distracted and calm. He was only two years old at the time and kept fidgeting and screaming, it was hell on earth for both of us, all I did was cry during these bandage changes, I could not bear him going through this much pain, and knowing I caused it (even though it was so he could have a hand) it was truly horrible to deal with.
As usual, I was always alone, and still am, caring and tending to Nicky's wounds. Sometime I wish there was someone there-anyone-to talk to him, hug him and tell him it's going to be OK as I concentrate on the wound at hand.

As wonderful and caring the hand surgeon was, he just could not comprehend my paranoia in making sure the hand healed straight, he kept wanting to wrap it in a fist.... aarrgh. I knew that if it was wrapped that way, it would heal that way too. It took a little over two months for the whole hand to be back to normal and I’ve been wrapping it nicely and straight ever since. It is straight to this day, and I know it is the wrapping and massaging, there is no doubt in my mind. Nicky’s hands web so easily that I know for a fact that if I was not bandaging each finger this way, his hands would be completely fused by now. It is important for me to note here that even as Nicky's fingers are straight, anyone seeing the hand has to take a step back-they do not and will never look "normal". By normal, I mean the skin having all the lines, nooks and crannies, fingerprints, palm lines (the lines that palm readers read) nails and such. Nicky's fingers do not look that way at all-they just look like someone simply covered his bones with red skin. Not pretty, but... FUNCTIONAL. That is the most important part.

His second surgery took place in November 1999 with the same hand surgeon on his left hand. This time he knew I know my stuff about EB, and pretty much let me “run” the show.
This surgery was also performed at St. Luke’s Medical Center in Phoenix, and the anesthesiologist proved himself to be a very caring man, who listened to me fully and completely. Well, at least he learned from surgery #1 :-) The Drs let me in the surgery room and let me help them put an IV on him and everything else. They let me pick the products to use and asked for advice. It was a very good experience all in all.

The decision to have the surgeries in the first place was not an easy one, but Nicky’s hands web and contract so fast I felt that if I waited too long the fingers would become irretrievable, and, unfortunately, after much research, I found that to be quite true. I have had one person tell me that she was shocked that I would put Nicky through hand surgeries at all and that I would let the Drs skin his hands-she told me that she rather see her son's hands web before she put him through this hell. This statement, of course, hurt me a great deal, made me feel like a bad and horrible monster of a mom, but then, after much thought, I realize I was doing something bad, but that was ultimately in the best interest of my son. Having hand surgery performed is like giving him another owie-but, unlike other owies, he comes out with a hand from this one. He was in a lot of pain, yes, it was hell, I agree, he pretty much lived on codeine for a while there. Thanks to the bandaging now he has hands and he will have hands so he can have quality of life. In pain for a month, but a PRODUCTIVE pain, and he will have hands for years to come. A means to an actual end. In this computer era, and knowing he cannot do much as far as living an 'outside' life, his hands, to me, are of outmost importance. We, as parents, have to do many things that are painful for our children, but must to extend health. Give him shots, do dental surgeries etc.

An EB parent must always give its child pain to extend health. Should I not give Nicky a bath because it is painful for him? Should I not pop blisters because they are painful to pop? I am not thrilled to do any of these things, but I do them, because it is in the very best interest of my child.

Here is a quick explanation of what happens to the hand and why it needs to be retrieved ASAP:
The process of scarring can lead to joint contractures. A contracture is a permanent shortening or contraction of the skin, tendons, connective tissue and, among other things, one or more muscles which causes a curvature because of the fixation of one or more joints. This presents the patient with a restriction in the movements of arms and legs causing muscle atrophy and tendon shortening. Webbing causes the fusion to progress, acquired syndactally manifests itself. In this situation the fingers and toes are fused and the bone tissue and tendon substance of the fingers and toes dissolve. Because of this the hands and feet end up as `encapsulated stubs' that do not or hardly function. So... having said this, you can understand there was not much waiting around to do. 

Here is a quick explanation of the state of Nicky’s hands before going into surgery both times:

The right hand was the one that was operated on first. Most of the hand was completely encroaching the palm and there was some webbing between the index finger and the middle finger, to about half way (first knuckle). The thumb was also webbing inside the palm. Considering Nicky is right handed, it is not hard to comprehend how badly this hand was at this point if you consider that he would not use it at ALL. He could not even move the fingers.

His hand is doing quite well and the only thing I can see that has gotten worse a little is the fact that there is some curling (contracting) of the middle and ring finger, I have been able to somewhat revert the process, a slow endeavor which will enable me to delay another procedure for years.

The left hand is the one that was operated on second. This hand was in pretty bad shape as well. The fingers were encroaching the palm, and although I had been able to keep the webbing to a minimum, he still would not use this hand. Because I was working full time and had another job on the side I was not able to devote my full attention to the proper healing of this hand. Because it was not in a cast the hand did not heal straight and it became impossible to keep up with and wrap like the other one. Because of this I basically gave up and within a year's time the hand was unusable. After much research on the subject I was finally able to get Nicky a state of the art hand surgeon (one that does EB kids often) and this hand was re-worked on in August 2002. More to come on the outcome of that surgery.

The third and fourth surgery took place in March 2000, one was a surgery to open up his throat (it was closed to 1mm-a child his age should have a passage equal to 12 mm), and the second surgery was to place the Gtube in his stomach. The fifth and sixth surgeries were performed simultaneously in June 2000, one had the purpose to further dilatate his throat (to 8mm at this point) and the other one was to put his permanent tube in.

The decision to have these procedures done-and where-laid heavily on my mind for almost a year. At first I felt he really needed his throat dilatated because he simply could not swallow well-or much, and it was getting worse. From other moms I heard good and bad stories about the procedure. When it was done well (most of the good stories came from the surgery being done at Stanford) the child came out in such good shape that he/she was able to eat in a matter of hours. The bad stories (all done anywhere BUT Stanford) told of tales of children coming out in such bad shape they could not eat for a week, the child was blistered up everywhere, and then there was no improvement in the end. So, it is to no surprise I wanted to take no chances but having the “experts” at Stanford do it.

At first I wanted to just have his throat dilatated and then if he would not gain weight, I would put a g-tube, but from what I heard from other parents, the children did eat better after the dilatation, but all of a sudden they wanted nothing to do with eating Pediasure or caloric foods as such-they wanted to try foods such as vegetables and soup, hence their weight would not improve.

Well, needless to say Nicky needed to gain weight badly-he hadn’t gained any weight at all in over a year, and still wore clothes for 18 months olds at 3.5 years of age. He needed nutrition and weight gain ASAP, and that was of outmost importance for his optimal health. It was at this time, around December 1999, that I started talking to other parents that had the procedure done, and all of the kids that had a g-tube placed gained anywhere between 7-10 lb. within the first 3 months... well, that was the answer I was looking for. By having the dilatation and the peg placement at the same time I would ensure growth and eating at the same time. I felt it was a win-win situation.

As I started telling friends and family about my decision of putting a gastronomy tube on Nicky I encountered such negativity it really angered me.

“He looks fine”, most people said. 
Fine if all 3.5 year olds still wore 18 month clothing. Nicky could swim in 2 year old clothes, how can he look “fine”? I have a lot of friends who’s 1 year olds weighed more than Nicky!!

“He does not look THAT skinny”, others said. 
Okay. Now imagine him without all those layers of gauze.

“Maybe he was just meant to be small regardless of the EB” some others said. 
I am the smallest member of my family and I still weighed several pounds more than Nicky at his age, and I was 3 inches taller-this without considering the fact that he is a boy, and Nicky’s dad is a BIG guy at 6+ feet, and he would be considered “thin” at 180 lbs. Sorry, it just can't be. Genetically this is simply an impossibility.

“Well, you cannot compare him to other kids his age” one said. 
But I have to. By seeing how big his peers are I can tell how big he “should” be, hence take precautions. EB kids need more calories than any other child, because the calories go to skin repair first-and then growth. This means they can suffer from growth retardation if not enough calories are ingested. By making sure he gets enough calories through his g-tube, I can ensure he has enough calories for skin repair AND for growth. He needs to be big and strong to live with and fight EB.

The throat dilatation is something he will always have to do, however often it will depend, perhaps every 6 months, or every year, whenever he will need it done. Because his throat was in such bad shape, I had to take him back to Stanford in June 2000 to dilatate it further. The surgery itself went extremely well due to the fact that the Dr really knows his stuff about EB. Kudos to Dr. Castillo, such a nice man. Nicky was eating and drinking right away, I was crying just to see him eat so well... such a little thing I can *never* take for granted.

Unfortunately in August he started throwing up blood again which lead to further restriction, and the reason why another dilatation took place in November 2000, and then another one in March 2001... and then another one in June and November 2001. The never ending cycle of a scarred esophagus. The last one took place in July 2002 and at this point it looks like thanks to Zantac it might become a once-a-year surgery instead once every few months! Hurray!

The g-tube insertion was traumatic for me, I was really worried, but it went real well, thanks to the Stanford Drs more than anything, simply because they know how to care for an EB child. Afterwards I was in shock for about a week, trying to deal with the fact that I had to do this to him... ugh. But, Nicky needs it, and I did what I had to do to ensure Nicky’s optimal health. Within the first 2 weeks he gained 3 lbs.!!!
I am a very proud mommy, because Nicky has tolerated the g-tube so well, and his growth was astounding. In June 2000 his temporary tube was replaced by a permanent tube called a Mic-Key (see below) and in early October the pediatrician replaced it with a new one. It was replaced again in February and in June 2001 and will always be replaced every few months for years to come.
More to come...

Nicky's permanent g-button, called the Mic-Key

To read more about how Nicky is living with EB, please Click here.
To see more clinical pictures of Nicky's wounds, please Click here.

This is life for a child with Recessive Dystrophic Epidermolysis Bullosa. We need your help to end the suffering of a disorder that does NOT get better with age... but gets worse, until it takes the lives of our children. Please help us spread awareness by putting this Ribbon on your website. We need a cure, and we need it now! Knowledge is power! Please help our cause! Nicky's EB Info World: http://www.ebinfoworld.com THANK YOU!

Read the words of this song playing in the background and you will know just how "perfect" it is...

You will be in my Heart
By Phil Collins

Come stop your crying, it will be all right
Just take my hand, hold it tight
I will protect you from all around you
I will be here, don't you cry
For one so small you seem so strong
My arms will hold you, keep you safe and warm
This bond between us can't be broken
I will be here, don't you cry

'Cause you'll be in my heart
Yes, you'll be in my heart
From this day on
Now and forever more
You'll be in my heart
No matter what they say
You'll be here in my heart, always

Why can't they understand the way we feel
They just don't trust what they can't explain
I know we're different but, deep inside us
We're not that different at all

And you'll be in my heart
Yes, you'll be in my heart
From this day on
Now and forever more

Don't listen to them'
Cause what do they know
We need each other to have, to hold
They'll see in time, I know
When destiny calls you, you must be strong
I may not be with you but you've got to hold on
They'll see in time, I know
We'll show them together

'Cause you'll be in my heart
Yes, you'll be in my heart
From this day on
Now and forever more
Oh, you'll be in my heart
No matter what they say
You'll be here in my heart, always, always

Some may own castles on the banks of the Rhine,
and hire an orchestra each morning at nine.
But richer than I they will never be...
I had a mom who made Web pages for me!

~Nicky's Realm on the Web~
[Nicky's Journal] [Nicky's Story] [Nicky and Epidermolysis Bullosa] [Surgery Tales] [Picture Gallery] [Halloween Gallery] [Recent Photos] [1st Birthday] [2nd Birthday] [3rd Birthday] [4th Birthday] [5th Birthday] [6th Birthday] [7th Birthday] [8th Birthday] [9th Birthday] [DisneyWorld 2000] [Nicky's Wish] [Nicky's Toy Zone] [Nicky's Web Rings and Awards]

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  Last Updated: July 02, 2006
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