Thursday, February 25, 2010


You may remember this poem that my mom wrote when Ella lost her first fingernail.

Ella’s hands are often the first thing people notice about Ella. Her face is usually in pretty good shape, and if people see boo boos on her face they just assume that she fell down. But her hands are not typical kid hands, they are dry (no matter how much lotion, oil or Vaseline we put on them), she doesn’t have fingernails, and her fingers are short. We always knew that hand webbing was a result of Ella’s type of EB, and we have been realistic to the fact that Ella’s hands would one day fuse together. The timing is what no one knows. For some kids it happens when they’re 2 or 3, for others it’s in elementary school, and still for others it happens when they’re in high school.

Many parents wrap each finger individually in an effort to stave off finger and hand fusion. We talked about it, but Joe and I decided when Ella was a baby that we wanted her to know how things felt. We wanted her to know the difference between hot and cold, smooth and bumpy, soft and rough. So we don’t wrap her fingers. Of course we’ve had the occasional accident where her finger took the brunt of a fall and we had to wrap it for a few days, but all in all we don’t wrap her fingers.

Now, we’re at a crossroads. Ella’s precious fingers are starting to contract and fuse. I’m crying just typing those words. It breaks my heart. (If I haven’t mentioned if before, I HATE EB!!!) Her precious pinky on her right hand has been getting worse over the past few months, and when we woke up last Thursday morning, the inevitable had happened and Ella’s pinky was completely fused together (like a fist, but it was only her pinky). I did what any mom would do…I freaked out. And I did what I probably shouldn’t have done…I pulled her pinky apart. We’re now wrapping her pinky as best as we can, and once that finger is healed, we’re going to have to revisit the issue of wrapping her fingers individually. I know it will be hard on her at first, but like everything else, she’ll rise above it and continue being the best 2 ½ year old daughter I could ask for.

A lot of parents don’t do hand surgery, and I completely understand why. It’s very painful and the hands always go back to their original state. And most of the adults that I know with RDEB choose not to have hand surgery and just learn to function without fingers (these are some of the most amazing people I have ever met). My fear is that we won’t do anything about Ella’s hands and one day that sweet little face will look at me and ask me why…why didn’t we at least try to fix her hands? I don’t want to face that question. I want her to spend her life knowing that we tried EVERYTHING to help her.

I also want her to be able to make her own decision on whether or not she lets her fingers fuse. However, in order to give her the opportunity to even make that decision, Joe and I have to act right now. We have to make very difficult decisions that will give her the ability to have that choice in the future.

While I want more than anything for a genius doctor to find a cure for this horrendous disease, and I want more than anything for Ella to start preschool next year without bandages, what I’m asking for right now is prayer for wisdom. One of our family mottos is that you have to deal with where you are, not where you want to be. And right now, we have a child with EB. As much as we want that to not be the case…that is the way it is. So don’t stop the prayers for healing, but please pray that Joe and I will have the wisdom to know how to handle Ella’s precious hands. That we’ll know when it’s time for surgery. That we’ll be directed to the perfect doctor who can do the surgery most effectively. That we’ll know how to wrap them to prevent more contractures and fusing. We just need wisdom.

Saturday, February 20, 2010

Parental Frustration

Being a parent has its frustrating moments. In fact, there are times it seems that the older a kid gets, the more parental frustrations there are. I would argue, however, that there are few things more frustrating than wrestling down your 2 year old and changing her wee wee diaper, then not 5 minutes later she poops. Ugh! Ella seems to prefer pooping in clean diapers, and sometimes it seems like she waits until she has a clean diaper on before taking care of business. Then, I have to bribe, threaten and wrestle her down AGAIN to change her second diaper. So frustrating! Oh well, at least she has a clean diaper now and if all goes according to schedule, I won’t have to change another poopie diaper all day!

Thursday, February 18, 2010


We have a funny kid…I don’t know if you know that or not, but she really is funny. And the older she gets the funnier she gets. Here are a few funny things she’s said the past few days.

A woman comes once a month to check in with Metti and make sure that Ella still qualifies for Medicaid. She came yesterday. Whenever Ella hears a knock on the door she thinks that it’s Grammy Janet, so when the woman came in Ella looked at her and said, “That’s not Grammy Janet! That’s an OLD LADY!”

We have been talking a lot about Ella starting preschool next year and trying to get her excited to go. Whenever we go anywhere if we see kids walking we say that they are going to school. So on Monday Ella and I were in the car and she saw some kids walking and she said, “Those kids are going to school.” “Yes” I replied. “Do you want to go to school?” To which Ella said, “Uh…no thank you.” So then I asked if she wanted daddy to go to school, and she said, “Uh…no thank you.” So I asked the next obvious question, “Do you want mommy to go to school?” And Ella replied with a very enthusiastic, “OK!!!”

Metti and I went to wrap her poor little pinky today (that’s a whole other story) and when we were wrapping she looked at it and said, “Oh gosh!” This is just proof that we have to watch everything we say around her! Luckily we don’t use swear words, otherwise we’d be in major trouble. She has also been known to use the words ‘gross’ and ‘crap.’

Ella has recently started calling us ‘mom’ and ‘dad’ instead of the standard ‘mommy’ and ‘daddy.’ I wasn’t too happy about this transition, so in an effort to beat her at her own game, when Ella would call me ‘mom’ I would respond by calling her ‘El.’ Well, my trick backfired on me. Now when I call her Ella she corrects me and says, “El!”

Saturday, February 13, 2010

There’s no place like home

Sorry it has been awhile since I posted. We’ve had a lot going on, what, with being stuck in Dallas by Snowmageddon and all. Joe, Ella and I were scheduled to leave on a flight to Texas on Friday morning (Feb 5). I heard all the news reports about a massive snowstorm due to hit on Friday morning and didn’t want a repeat of Christmas, so I decided to change our flights to Thursday night. I called the airline and explained the situation and they changed them for free (there are nice people who work for those companies). We headed out Thursday night and got to Dallas safe and sound. This is where I need to give a huge shout-out to my awesome uncle and aunt who let us stay at their house for not only the original 3 nights, but an additional 5 nights (side note: ALL of my aunts and uncles are awesome!)

Friday morning was the family viewing of my grandmother. It was weird seeing her lay there. She looked beautiful, but it was just weird…I don’t know how else to describe it. I can’t believe she’s gone. I was going through some old voicemail the other day and came across a message she left. I’m going to save it as long as I can. The hardest part was seeing my sweet grandfather. I know he misses her something awful.

Friday night was the viewing for everyone else. Joe and Ella stayed back at the house while I went with the rest of my family. Joe and I always say that the only time everyone gets together is during weddings and funerals, and this was no different. I got to reconnect with aunts, uncles and cousins that I haven’t seen in years, and met some cousins that I’ve never met. Although I wish we had seen each other under different circumstances, I am so thankful for the time to reconnect with each one of them.

Friday afternoon was the funeral. The church provided a big lunch for all family and friends that were in town, then we headed to the service. Within a few minutes Ella was chatting away and I had to take her out. How appropriate that my grandmothers namesake, Little Ella, was the one that had to be taken out for talking. To say Big Mama was a talker would be an understatement. She talked ALL THE TIME!!! In fact, she was known in the family for calling and leaving a message that was so long the answering machine would have to cut her off…then she’d call back and finish what she had to say. But as much as a talker as she was, Big Mama got very frustrated at kids who were talking in church and would get up constantly to go to the bathroom. Several years ago she came up with the brilliant idea to take bubble gum to church and if a kid sat all the way through church without getting up or talking, then she’d give them a piece of gum. Of course this was a hit among the kids and at her memorial service there were baskets of bubble gum so people could have a piece. The memorial service was beautiful and I am so glad we were able to be there to help celebrate her life.

Friday night was a great time of the family sitting around and having fun together. Big Daddy played the harmonica and each of the great grand kids got to have a turn at entertaining the masses. Ella waited until the other 3 great grandkids were gone, then she let her light shine. As shy as she is, she was very willing to get in the middle of the family circle and sing song after song. I think she liked the applause she received after each performance.

The original plan was to fly out Sunday night, but because of Snowmageddon, our Sunday night flight was cancelled. Unfortunately the next available flight was Wednesday morning. As of Monday night that flight had also been cancelled. We were rebooked on a Thursday night flight.

Since we had some time to kill we drove to Shreveport, LA to visit my paternal grandmother, Maw Maw. Ella was a baby the last time we were able to visit her, so I am so glad we had a couple of extra days to drive over to see her. Ella warmed up right away and didn’t hesitate to show Maw Maw her performing skills. She sang and danced like she has never sang or danced before…and Maw Maw loved every minute of it. Maw Maw even bought Ella some Valentine chocolate. It was just before lunch so I only let her have one piece, but before I knew it Ella had taken things to a new level and asked daddy to open another piece for her…of course he did and she finished that one faster than she did the first one. I have some great pictures of them together in the January 2010 pictures folder.

As fate would have it, Dallas received a record snowfall on Thursday, which meant, yes, our Thursday night flight was cancelled. Luckily we were able to rebook on a Friday afternoon flight. After an hour of sitting on the tarmac waiting for maintenance to clear off all the snow from the plane, we took off. We landed at BWI around 7:30 pm. Our plan was to have Joe go dig out the car while Ella and I hung out at the airport. My genius husband suggested we throw a shovel into the car so that when we got back we’d be able to dig our way out. That was the best idea of the trip! When he finally made it to the car he discovered that we had a new delay getting home…our car battery was completely dead. Yeah…this just keeps getting better. Luckily we have roadside assistance through our insurance, so Joe called to have someone come jump our car. While he was waiting he dug out the van. Meanwhile, Ella and I were living it up at the airport observation deck. They had a big toy plane, bus and train for kids to play on, and Ella was loving it. She’d see other, older kids running and jumping all over the toys and as soon as they were done she’d say, “It’s my turn!” We’d do the same thing on a much smaller scale than the big kids.

Joe came to get us a little after 9:00 pm and we headed home. We both just knew that we were going to come home to some new catastrophe…broken pipe, fallen tree, something. Thank heavens we were wrong and the house seems to be in perfect running order. The good news in all of this is that even though we were gone for a week, we didn’t miss any work since our offices were closed. The bad news is that poor Joe got really sick while we were in TX. He’s on medicine now, but it’s not helping too much. We are so glad to be home! We all slept in this morning, including Ella who slept until 10:30. Metti came over and is watching Ella so Joe and I can take care of some things around the house…shoveling, laundry, cleaning, etc. Although I am thankful for the time we spent with family, and I’m even thankful for the extra days that we got to spend with everyone, Dorothy had it right…there’s no place like home. Now, I’ve got some laundry to do.

Monday, February 1, 2010

Big Mama

This morning my family experienced a great loss. My wonderful grandmother passed away at 85 years old. She lived a long and wonderful life and is survived by 3 children, 3 in-laws, 6 grand children and 5 grand in-laws, and 4 great grand children.

My grandmothers legal name was Annie Ella Jean (Lorance) Gooch, but she was most widely known as Big Mama. Believe it or not, that is a name that she picked out herself and it described her perfectly. The picture above is of the 2 Ella’s meeting each other for the first time, Big Ella and Little Ella.

There is no possible way I could begin to tell all the things I love about Big Mama. Going to her house was always so much fun. We always lived far away, so visiting in the summer was quite a treat. The deal was that my parents got to sleep out in the guest house, and Curby and I got to sleep in cots in Big Mama and Big Daddy’s room. Curby’s cot was at the end of the bed and mine was right next to Big Mama where she would hold my hand until I fell asleep. The space between her bed and the cot was about 6 inches, so she would have to shuffle in to crawl into bed, and shuffle back out in the morning…but she never complained. She was so happy to have us visit that I don’t think she minded one bit. While we were visiting Big Mama let us have as much soda and candy as we wanted. My parents never kept either in the house, so when we got to the Bigs (that’s what we called them) Curby and I would go straight to our stools in the kitchen (each grand kid had their own stool) and we each had a nice stash of candy. And the garage had shelves of soda and we could drink as much as we wanted. And don’t even get me started on the Blue Bell Ice Cream! That’s what grandparents are all about…letting you do stuff that your parents wouldn’t dream of!

I went to college in Oklahoma, so for the first time in my life I lived close to the Bigs. I would visit about once a month and every time I came I brought with me a basket full of laundry. Big Mama would spend all Saturday morning washing my clothes for me. I’ll never forget one weekend when my brother also came out to the farm (he was living in Ft. Worth at the time) and he also brought his laundry. She did laundry ALL DAY and on Saturday night she counted how many pair of underwear she had washed. The next day at church she went around telling everyone how many pairs of underwear there were. I was mortified!

When I was pregnant she and I were talking on the phone and Big Mama was telling me a story about when she was pregnant and she was walking outside and fell “flat on her fliddle dee diddle!” She always used made up words like that, and sometimes she didn’t even know they were made up (like when she called Metamucil “Metamuchual”). I still laugh when I think about it. And she always used good old southern terms that people don’t use anymore, like “up yonder.”

As you know, Ella is named after Big Mama. Even as a kid I had always loved the name Ella, and I decided that I wanted to have a little girl named Ella someday. Big Mama is actually the one that suggested combining her name with Joe’s name to come up with Joella. In some ways Little Ella is a lot like Big Ella. She’s a talker, and anyone who knows Big Mama knows that she was a talker! And Little Ella has those big, blue eyes, just like what Big Ella had. And they are both fighters…Little Ella proved that at the beginning of her life, and Big Ella proved it at the end of hers (although we all knew it long ago). Now we just need to teach Little Ella to be as outgoing as Big Ella, then she’ll be all set. Big Mama NEVER met a stranger! She would talk to anyone about anything for any amount of time!

In August I had a gut instinct that was telling me to take Ella back to Texas. It wasn’t practical, the timing was wrong, it was going to cost a lot for plane tickets, and the weather was going to be bad for Ella’s skin, but I knew that we needed to get back down there. Something was telling me that her time was coming short. Joe has been with me long enough to know that my gut rarely steers me wrong, and this time was no different. Just a couple of months after our trip, Big Mama fell and broke her hip. She was never able to fully recover. I cherish that trip and I’m so glad that she got to see Little Ella doing so well.

There are 2 things I am most sad about…1) Ella will not remember her namesake. She’ll have the stories that we tell her, but those will never do Big Mama justice. And she’ll have the pictures of them together, but those only tell part of the story. I wish she had been able to get to know her better. 2) Big Daddy is losing his life partner. They began dating in high school and got married at 19. They were married for 66 years. The other day Big Daddy said to my mom, “My whole life I’ve prayed that I would go first. I just didn’t know how I could live without her.” That breaks my heart. I am so thankful that my Uncles, Aunts and cousins all live close enough that they’ll be able to take care of Big Daddy and make sure he’s not alone.

Big Mama was such an integral part of our family. It’s weird to think about life without her. My parents are staying at their house and I asked if I could call on the Bigs house phone. When my mom told me it has been disconnected I cried. I’m not ready to delete their number from my cell phone. I’ll miss dialing that number and hearing Big Mama on the other end. I miss her.