I remember....

So, I totally suck at updating this blog and the story associated with it! I finally have some time to elaborate on my story, but, I thought I would start by telling you what I have been up to.

First, I found a website dedicated to those parents, caregivers and sufferers of Epidermolysis Bullosa and I have spent a significant amount of time there gaining all kinds of knowledge and hopefully making some helpful suggestions to others. In doing this and meeting others with the same disease it became very important to me to go to the Patient Care Conference in Denver, Co. last week. That was about the best decision I have made in a very long time. My mom ended up coming with me and we spent two solid days in seminars and panel discussions and checking out new bandages and meeting all kinds of people. It was by far one of the best experiences of my life. While we were there I got a chance to hear Dr. Wagner speak about the bone marrow transplant clinical trial that they are working on as a cure for EB. He was very specific about the risks and those that are candidates (which I am not). They have done two successful transplants so far and were working on the third while I was in Denver.

Here's where the story takes a very sad and tragic turn ... The third patient was a baby by the name of Sarah Mooreland. Sarah was 9 months old and was in the beginning of the testing that is done before the actual transplant. Sweet baby Sarah died on Friday before she was able to get the transplant. They are unsure at this time exactly what killed her, whether it was complications from the testing or complications from the disease or something else. I will think of Sarah and pray for her family daily, for it is families like hers that make medical breakthroughs, treatments and cures possible for the rest of us. I never had the pleasure of meeting baby Sarah or her family, but we are bound by the ties of this terrible disease and I am eternally greatful to them on a very personal level. Please stop reading and say a prayer for the Mooreland family.

So, since my last post I have spent a significant amount of time at the doctor as well. I was having a very hard time healing even after a 2 week dose of steroids. I started seeing a new dermatologist and I really like her. She really take the time to understand me and research the disease and that alone makes her a hero in my book. At my first visit they did blood work and took swabs of my sores. The swabs came back as MRSA - if you know me you know I have been MRSA paranoid for MONTHS - and of course my fears became reality. I have just finished a month long dose of antibiotics with another round of steroids and although the infection has cleared up - the sore unfortunately have not. So, I just keep on hoping and praying that one day I will be blister free again.

Ok - now back to the story.

When I was very young I started having problems swallowing (3 or 4) - my mom drug me around to every doctor she could find, until finally she found Dr. Rivkin. Dr. Rivkin gave my parents more information about EB in the first appointment than they had ever known. Over the next couple years Dr. Rivkin would do several dilliations of my esophagus. After a while the dillitations were cause more problems that they were doing good. It was time to make some tough decisions. Dr. Rivkin suggested replacing my esophagus with a piece of my large intestine. My parents called the DeBRA nurse who strongly suggested that they NOT do the surgery and just do a g-tube instead (basically a feeding tube and me never eat or swallow anything again). Thankfully, my parents decided to do the surgery - as I sit here and remember what it was like before the surgery the g-tube would not have worked for me anyway - I couldn't swallow water - or spit! I would literally have liquid get caught in my esophagus and have to throw up to get it back out. I would go days with something lodged in my esophagus, I even tried to stand on my head and jump up and down to get it to move (seriously! its ok to laugh - but desperate times called for desperate measures - it didn't work!).

Before I entered the hospital to have the first surgery I was 9 years old and weighed 42lbs, my esophagus was only 3mm wide. I had to enter the hospital a couple of days before my surgery for some prep work, basically they had to "clean" me out. The very cool thing was that my best friend at the time checked into the hospital at the same time that I did - she just had her tonsils out. She was there the day before her surgery too (oh how times have changed). So, after our parents left from visiting hours we donned our slippers and ran the halls of the hospital - which by the way didn't have a childrens ward. For those of you who where at Windy Hill Hospital back in the early 80's - SORRY! We had the most fun that night, riding the elevator and checking out every single floor. I got to visit Carrie after her surgery the next day and then I got transferred to another hospital (no it wasn't for bad behavior!). Windy Hill Hospital decided that they didn't have the capacity to handle such a small child with such a big surgery - so they took me to Northside Hospital.

That first surgery took 11 hours. 11 hours.................. I cannot imagine sitting by while my child is in surgery for 11 hours! Who can perform such a risky surgery for 11 hours. I mean, when do the doctors go to the bathroom - I just don't understand how that works. Do they take a lunch break? Do their feet hurt from standing for so long? Do they just stop and grab a diet coke? I mean 11 HOURS - I think the only thing that I have done for 11 straight hours is sleep!

My mom says that I was never in the hospital for more than 2 weeks - in my 9 year old head it was more like 2 months. I spent a couple days in critical care, I spent a couple more days in ICU and then was moved to a private room for the remainder of my stay. I remember the nurses were wonderful. I remember being very hungry and not allowing anyone to eat in my room (I was not allowed to eat or drink anything until the surgery sites were healed - so the whole time is was in the hospital). I remember turning the TV off everytime a food commercial came on. I remember that when they came to change my feeding tube bag the nurses would ask me what I want to eat - anything that I said they would write on the bag - pizza and potato chips. I remember telling a phlabodamist (sp?) that he couldn't take my blood, so he went down to the nurses station and told the nurses that he needed them to hold me down and they told him no! They told him he would have to wait until my parents got there and then I would let him take my blood (see I was smart even then!). I remember after I could eat I wouldn't eat what they sent me, the dietician came up and told me that she would personally make me anything I wanted to eat - and she did! I remember that after my parents would leave from visiting hours the nurses would come in and play cards or board games with me. And I remember that before I checked out of the hospital the nurses gave my brother and I a wheel chair and let us go wild on the floor. We had so much fun that day!

I keep saying my first surgery because I had 2 other following the first to fix some complications from the first. All of the "things I remember" could have been from any of those 3 hospital stays.

I want to end this post by thanking all the nurses out there who have not lost thier compassion and who can relate to a kid who is scared and alone and who gave me some very fond memories of what truely was a very difficult time. I don't remember any of their names and never had a chance to speak to any of them after I left the hospital - but, hopefully, somehow they will hear this thank you!

I also want to urge other parents of EB patients to consider this option if there are no others. Please don't let your children die of malnurtition because the doctors think its too risky. It worked for me, it worked for Jamie and it has worked for several other EB adults. I'm not by any means saying it is right for everyone - but I think it is an option for some and I got the distinct impression at the conference that the doctors think it too risky and are possibly not offering it as an option.

Please feel free to comment - I know that I rambled a lot and I am not going back to proof read - so please forgive the mistakes and misspelled words.

good night to everyone - sleep well.
Janel