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

When it all started

As I told you in my last post, I have Recessive Dystrophic Epidermolysis Bullosa. It is hereditary and many people could be a carrier and not know. In my case my family has Dominant Dystropic EB, unbeknowst to anyone, my mother is an EB carrier - therefore I got the recessive type - which is much worse than the dominant type. Again - visit http://www.debra.org/ for more information.

so - let the story begin:

I was born on June 10th in Ohio. When I was born I had no skin on the inside of my left foot, no toenails and many blisters and sores.

Even though this disease is hereditary, I recently found out that my family didn't have a diagnosis for it until I was born. So, imagine their suprise when I came out the mess that I was. I have seen pictures of myself in an incubator and seen pictures with blisters on my face and I still have the scar on my foot as a reminder of how fragile I was when I was born.

My mother has told me stories about how difficult it was to care for me as a baby. If you picked me up under the arms I blistered where you touched me. She had to sew very soft pieces of fabric to the backs of the buttons and snaps on my baby clothes to keep them from rubbing blisters. Diaper changes were a challenge, etc. etc.

Now, being the mother of a 20 month old, I have a new appreciation for what my mother and father went through trying to take care of me. The other day I turned around and didn't realize that MacK was right behind me and I knocked her down. She didn't get hurt but I still felt so terrible. I can't imagine the pain and guilt that my parents and family felt when they caused sores and blisters simply from touching me.

I can remember having clothing or bandages stick to the sores that I had. Pulling the material off would have caused more sores and blistering, so I would have to soak in the tub until the skin would release the material. I also remember mom using baby oil to get it released without causing too much damage.

To this day there are few doctors that know much if anything about this disease - so you can image how difficult it was for my parents to find compitant doctors to care for me. My mom took me to so many doctors that I became scared of men.

I can't really remember when I first started having problems swallowing, but that problem is what led us to the man that saved my life (or at least the quality of it), Dr. Rivken. He put me on prednisone to help control my outbreaks and dilated my esophagus so that I could swallow better, and eventually replaced my esophagus with a piece of my large intestine.

Needless to say that is a very long story that I will save for next time.

Its Saturday and as soon as my crazy baby girl wakes up from her nap we are going to play in the pool!

I hope you have a wonderful weekend!
Janel

Let me introduce myself

Hi! I'm Janel Waters, a 33 year old mother of 1.

I am a firm believer that you never know what is going on with someone else, so don't judge them! It will suprise many that know me to know that I have a hereditary skin disease called Epidermolysis Bullosa. This devistating disease causes me to have very large and painful blisters which turn into large painful open wounds.

I have downplayed this disease and its effect on my life for many years now. I have had friends for years who knew nothing about it. 95% of the people that I used to work with and my customers had no idea that I have lived with this disease every day since I was born.

Well - though this blog I am "coming out". I will give you a no holds barred look at my life since birth - the good, the bad and mostly the ugly. At some point in the future I may even post some pictures of the sores that I have just so that you can get an idea of what I am talking about.

Before I get started with my story, I invite you to do a little research on this disease, feel free to go to www.debra.org. I have recessive dystrophic epidermolysis bullosa (RDEB).

I will begin my story at a later date.

for tonight - goodnight and God Bless
Janel