Welcome to the 21st Century

Joe and I have always been a day late and a dollar short when it comes to new and improved technologies. Case in point, we still use the 19 inch TV that my parents bought me my sophomore year of college. We just aren’t that into having the latest and greatest in technology gadgets. The closest we’ve come is when 2 years ago I got a new cell phone and when we were at the store getting it activated two teenage girls completely freaked out because I had the “pink chocolate.” I didn’t know that my new phone was even named the “chocolate.” I got it simply because it was pink.

So after several years of being out of the loop, Joe and I broke down last night and bought an ipod. Yes, this is the first ipod for both of us. It started because I wanted to get a little CD player for Ella’s room. She loves music (as you all know) and has figured out how to turn the CD player on in Joe’s office. If you put her on the floor and say “music” Ella will crawl over to the CD player and start pushing the buttons for it to turn on. So we thought she’d like having her own player. Well, they didn’t have any basic CD players, so we decided that it was time to join the rest of the crowd and get an ipod. I really wanted a pink one, but I couldn’t do that to Joe. I could just imagine him at the gym working out with a hot pink ipod. So we got green instead. Last night I downloaded a bunch of Ella’s CD’s and we bought some music from iTunes. We even got the speakers to go with the ipod so that we can all listen together. Joe is definitely the most excited about this new purchase. I just hope he doesn’t bring up the new TV conversation again. I just can’t bring myself to get a new big screen TV when the 19 inch we have works perfectly fine. Just because everyone else has HD doesn’t mean we have to.

Teeth!

Ella is in the process of getting 2 more teeth. Both are on top and both are causing pain. She’s had a really stuffy nose, which is usually taken care of with a little saline spray, and drool has been pouring out of her! The other night she had been asleep for about 2 hours when she woke up crying. It was obvious by the cry that something was hurting. Given our situation, we immediately go into panic mode. Sometimes it's still tough to know what is normal baby stuff and what is EB related. Nothing would calm our sweet girl down. She was wiggling and crying and sticking her fingers in her mouth. We gave her some Tylenol, rubbed some numbing stuff on her gums, and turned on the radio. Within a few minutes she was back asleep and doing fine. After talking to a couple other moms (one with a 14 month old and one with a 15 month old, both getting teeth) I found out that we are not alone. Apparently their children had also been waking up in the middle of the night with teething pain. Luckily last night it was better and Ella slept all night.

I do think it’s funny that Ella’s bottom teeth are coming in slower than her top ones. She has the two middle ones on the bottom, but that’s it. And she isn’t showing signs that the others on the bottom are going to poke through anytime soon. After these two come in, that will give her 6 on top. I know that eventually she’ll have all her teeth.

Anniversary

Today marks the 1 year anniversary on bringing our precious Ella home from the hospital. One year ago Joe and I had spent the night in the guest room of the hospital as a “trial run” of having Ella home. We got little to no sleep and were completely exhausted! The day seemed to drag on forever. Every hour passed with a slow tick-tock. Finally, she was dressed, strapped into her car seat, and we were off.

It seems so long ago, and looking at the pictures, Ella seems so little. At the time, the 34 days she spent in the NICU felt like an eternity! Now it feels like 34 days passes just in the time it takes to blink. From what I hear, the next 18 years will pass just as quickly.

In other Ella news, Ella has a snow globe with Elvis and President Nixon (thanks Aunt Darla). Every morning when she wakes up she reaches for her snow globe. Joe shakes it for her and she watches the snow fall. I am so proud that she loves her Elvis snow globe. And when I handed it to her this morning to play with, she looked at me and said thank you.

Random Thoughts

1) Since Sesame Street is Ella’s new favorite show, I thought this would be cute to post. So please take the Sesame Street Personality Quiz and comment on which character you are. I’m Ernie. I think it was question 1 that got me. I can’t help it, I like making up songs. It keeps things interesting and keeps others entertained.

http://www.blogthings.com/thesesamestreetpersonalityquiz/

2) Some of the gauze that we use on Ella comes in this box with a weird coating on the outside. Every time I open it, it makes the sound of nails on a chalkboard. I hate it! It makes me cringe just thinking about it! It reminds me of that annoying kid from Junior High that thought it was so funny to scrape his finger nails all the way down the chalk board! I wonder what ever happened to him.

3) We have started brushing Ella’s teeth and she loves it. She watches me put the tooth paste on the plastic finger cover (the kind for babies) and she opens her mouth in anticipation. She even sticks out her lower jaw to help me out. When I brush the top ones she tilts her head all the way back. And she knows that when I’m done, she gets to hold the plastic finger cover and chew on it!

4) Ella has learned her first sentence. Well, it’s actually just 2 words, but we’ll count it as a sentence. She is very polite and has learned to say “Thank you.” Moat of the time she even says it at the right time, like if we give her a toy or are about to give her food. Sometimes she just says it out of the blue for no reason at all.

A new EB baby

A baby has been born in Orlando, FL with EB. I don't know any details, but please lift up your prayers for this family and their precious baby boy. The first few days are the most scary for the parents and have the biggest learning curve. This family needs prayers of comfort and knowledge to make the best decisions for their son.

13 months old today!

Ella is getting smarter by the minute! This weekend she learned 2 new words. We took her to BJ’s (a wholesale food store) and were looking at a new Elmo video. Being the mom that I am, and wanting to instill a sense of independence in Ella, I held up all 4 Elmo videos and let her choose which one she wanted. She chose Elmo sings Country (that’s my girl). I handed her the video to look at and hold on to while we were in the store and she started saying “Mo” over and over again. She knows her buddy Elmo. She always waves at him when Elmo’s world starts, and she waves at Mr. Noodle. He is definitely her favorite Sesame Street character, so I am very happy that she has learned to say his name. This will go well with “Cookie” for Cookie Monster, which also happens to sound a lot like doggie, so it could be possible that she is just calling Cookie Monster doggie.

Last night we had a wonderful dinner at the home of our friends Casey and Christy. Little Ginny is just a couple of months younger than Ella, so we like getting them together to play. While we were there we were giving Ella strawberries, her favorite, and when she was done with one she would say “more.” It was so cute. She would draw out the “M” sound and was having a bit of trouble with the “R” (which is a hard letter to say), but we knew what she meant.

Our smart baby has also recently figured out how to work puzzles. She has a few of those wooden puzzles with pictures on them and she loves taking the pieces out and putting them back on. She doesn’t quite grasp the concept of fitting it into the slot, but she knows it’s supposed to go on there and she usually gets it in the same general area of the matching picture. Sometimes after she puts it back she’ll clap for herself. She has also learned several of her important body parts including nose, mouth, eyes and tummy.

As you can see, Ella is doing so well. She is doing better with her eating adult food, but that’s probably our biggest obstacle right now. She eats a lot of oatmeal and baby food, and she loves cheerios, strawberries and cheese, but I want her to start eating more adult food. She really has a hard time chewing meat. I guess I just have to wait it out and she’ll figure it out when she’s ready. All in all, she's a sweet and happy little girl!

Happy Birthday Daddy!

Ella asked me to put a special post telling her favorite Daddy in the world Happy Birthday! Joe is an incredible dad to Ella, and I couldn’t ask for a better, more supportive husband. Everyday I am reminded how lucky I am to have been blessed with Joe as a life-mate. Joe makes me laugh every day. And every day he says or does something that is completely unexpected. Needless to say, this keeps life around our house very interesting.

I realized that put in the situation we are in, there are a lot of men who would bury themselves in their work and completely disconnect from their family. I feel so lucky to have a teammate. Someone who hugs me when I’m sad, takes over when I’m at my breaking point, but who also speaks candidly when I’m being unreasonable. And I am so blessed to have a partner who takes an active role in Ella’s care, rather than just leaving me to handle it alone.

And I don’t need to mention what a wonderful daddy Joe is to our precious Ella. He loves her more than anything, and it shows. I know that he would do anything for her. They love playing together and Joe loves it when Ella cries because he walked out of the room. She is her daddy’s girl, and I wouldn’t have it any other way.

Happy Birthday Joey! Dancerella and I love you so much!

Thanks to the Eagles of MD!!!

We would like to take this post to send a huge shout out and thanks to the Fraternal Order of Eagles in Maryland. When Ella was born the Eagles (Brunswick Eagles #1136) joined forces with Jenni Benson (the greatest BF anyone could hope for) to create and market the wonderful CD made in Ella’s honor. Paul Armstrong and Angie Armstrong worked hard with Jenni to raise money for the project, and the Eagles donated the funds needed to produce the CD so that any money raised through sales would go directly to Ella and her medical needs. Needless to say, the money received from the sale of the CD has been a huge help to our family. There are items Ella needs that are not necessarily “medical” but are medically necessary for Ella. Such items include special cloth diapers that don’t give her boo boos, very soft sheets, a table that we use to change her bandages, little covers to protect her neck in the car seat, and much more.

On Sunday June 22, Joe, Ella and I had the privilege of thanking this group in person at their state meeting. We were welcomed with open arms. Ella didn’t even pull her shy routine, which says a lot! The Eagles sang Happy Birthday to Ella and went above and beyond by giving her a special birthday gift donation, which was promptly deposited into her college fund. We are SO thankful to this wonderful group for all of their help and support. You can find out more about the Eagles organization on their website, http://www.foe.com/.

Happy 4th of July!!!

Ok, so the post is a bit late to celebrate our Nation’s independence. Oh well, better late than never, right.

Thursday my wonderful office closed at 1:00pm, so I was able to head home early for the 3 day weekend. Unfortunately, closing early meant that we had to take Ella to get some more blood drawn. This time it was for regular testing that a lot of kids get, but that didn’t make us feel any better about it. The last time in Cincinnati it was torture and Ella screamed the entire time. This time, they brought in the veteran blood guy and he had to pull out the big guns for Ella. They have this cool red light that they put under the baby’s hand and wrist to see exactly where the veins are. This way they only have to stick the kid once. So he did the red light, which distracted Ella enough that she only slightly squealed during the initial prick. Then she was so interested in the blood being drawn that she didn’t really cry the rest of the time. Joe and I kept singing songs to her and I think that helped too. We were so glad when the blood draw was done and thank heavens there wasn’t any skin trauma in the process. Ella was a champ!

We got her blood drawn at Children’s Hospital in Washington, DC, so we were also able to see June, the wound care nurse who took care of Ella while she was there. June did a great job not only caring for Ella’s wounds when she was born, but she trained Joe and me on how to handle things once we got home. She was one of the few who had actually worked with EB kids before, so she knew the disease and exactly how to handle it. We also got to see Sharon, our Social Worker while we were in the NICU. Sharon was a wonderful advocate for Ella and was very good about getting us answers we needed. It was great seeing both of these women again.

The 4th was a wonderfully relaxing day. We didn’t do anything or go anywhere besides running a few errands. Joe did go to the store and bought us a couple of lobsters for dinner. Ella kept calling them doggies and laughing. She’d never seen anything quite like that before. They might have been funny to her but they tasted great to us!

Saturday was another relaxing day. It started off with Joe reading the Declaration of Independence to us (it was a day late but just as meaningful). Unfortunately Ella wasn’t willing to sit still and listen. While her lack of interest frustrated Joe, he is hopeful that next year she’ll be more enthused. In the afternoon we had a cookout with one of Joe’s law school friends, Colleen, and went swimming in her pool. Surprisingly Ella fell asleep on the way home, wet bandages and all.

Sunday Ella wore her special 4th of July outfit from Grammy Janet (pictures will come soon). As a treat we took Ella to Barnes and Noble and got her a special puppy book that has fur you can actually feel. After that we took her to PetSmart to see real doggies. They have a play room for the dogs with a glass wall so people can look in and see them playing. Ella stood at the glass wall with her hands and face pressed against it and she laughed the entire time! One of the dogs kept coming over to her and licking the glass, which of course sent Ella into a giggle fest! She had such a great time watching the doggies. When she’s older and we have a bigger house we will definitely have to get this girl a dog!

Confused

Our sweet baby girl is a little confused. She knows her doggie, she sleeps with it every night and that is the first thing she asks for when I pick her up in the morning. However, since “daddy” sounds an awful lot like “doggie” Ella has started calling Joe doggie too. We know the difference because if Joe walks in the door and she says doggie, she really means daddy. If it’s the morning or bedtime and she asks for doggie, that means her real doggie. Hopefully she’ll soon learn that daddy is daddy and doggie is doggie, then we won’t have the confusion issues. Until then, it’s really funny to hear her call Joe doggie.

DNA Results

While we were in Cincinnati we had all of our blood drawn for genetic testing. This is important for a couple of reasons. First, it would tell us exactly what type of EB Ella has and give us an idea of what her future may be. Second, it would tell us the likelihood of future children having EB.

There are only 3 ways a person can have EB. 1) They inherited it from one parent (dominant). Typically this form starts more severe and lessens as the child gets older. 2) Both parents carry the gene (recessive). The important thing to know about recessive EB is that in order for a child to have it, both parents must have the gene. If one parent has the recessive EB gene, but the other parent does not, then the kid will not have EB. Typically this form starts mild and gets worse as the kid grows up. 3) Spontaneous mutation. This only happens about 2% of the time.

So on Thursday we received a call that the results of Ella’s analysis were in. You may recall that when she was first born her skin biopsy was diagnosed as being an extremely rare form that she would likely grow out of. Unfortunately, her blood results have come back that the skin biopsy was wrong. Ella has been diagnosed as having Recessive Dystrophic EB (RDEB).

This news was a hard pill to swallow. It means that our precious little girl may only get more severe with time. Unfortunately many of the patients with RDEB don’t make it into their 30’s and usually develop an aggressive form of skin cancer along the way. There are also adults who have RDEB and lead wonderful and productive lives, are married and even have kids. There is no way to determine which Ella will be. Time will tell. The lab is still analyzing my blood and Joe’s blood to determine exactly who carries which mutation of EB (she has 2 mutations, one in each gene).

And to answer the question I know is going through your head, there is a 25% chance that future kids will have EB. Some may look at that and see hope that there is a 75% chance the kid won’t have it. But for Joe and me, taking that risk would be like playing roulette with a baby’s life. It’s not worth the gamble, and it’s not fair to the baby should he or she have to go through what Ella has gone through. There are a few options for us to expand our family including adoption and Preimplantation genetic diagnosis, but for now we are focusing on Ella and giving her the best care possible.

On a positive note, Ella is still the sweet baby we know and love. She is growing more and more, and has recently learned the word “WOAH!” It was pretty funny because until recently she would only eat adult food if we put it in her mouth. She didn’t quite grasp the concept of picking up something and putting it in her own mouth. We went to church today and in the nursery she saw all of these other kids picking up cheerios and eating them. Apparently something clicked in her head and she started picking up cheerios and eating them. She is also testing out standing on her own. She is cruising around the furniture like a pro, but is just starting to test the waters away from the couch. She laughs all the time and has the most wonderful personality. We love her so much!

For more positive news, there is a lot of research being done on RDEB. We are hopeful that as time progresses there will be more and more advances to help all people with EB. You can read about the research being done on www.ebkids.org.

Thanks for all of your prayers. While we are saddened by this diagnosis, we will continue to fight and live life one day at a time.