Weekend in ClarksVegas

The girls and I planned to head to Clarksville on Friday the 15th for some time with Grams and Granddaddy. We were QUITE excited. Then, about lunchtime, I got a text that Amy’s ears were hurting again. AAARRRGGGHHH!!! Needless to say, I was QUITE upset. We took her to the dr after we got off work, and thank goodness, there was no infection. We came out with some flonase. So, now, Amy has nose spray and allergy pills. I was less than thrilled at the prospect of giving her nose spray, but she has been SO GOOD about it.

After we were done there, we finally got on the road. We stopped and got supper at McDonald’s on the way. One potty stop later, we got the Clarksville about 9pm without any major drama, which is always a plus. We unloaded, and hung out for a few minutes before turning in for the night. We were all worn out!

The next morning, it was COLD!!!! Thankfully, I had packed some jeans and jackets for us. The girls, Grams, and I wanted to go out to a few stores. (Well, Grams and I wanted to go, we just took the girls with us. They were less than thrilled) Elizabeth needed some shorts, and we were able to find several pair for her. They are quite cute! She’s wearing a pair to school today, actually. We also picked up a few other things.

After we were done, we went home for lunch. Then, we headed up to Cheatam Dam. None of us had been there in YEARS, and the girls had never been. It was too cold to be out of the car for very long, but we enjoyed driving around and looking. It was different stuff.

It was VERY windy, and the water was very choppy. Obviously, some people were not deterred by this...

It was neat to see how much deeper the water was on one side of the dam vs the other.

By the way, they mean this...

After we were done, we drove over to Ashland City, just because we could. Then we headed back home.


We hung out for a while, and then we went to Cici’s for supper. No trip to Grams and Granddaddy’s house is complete without this. Shrek 3 was playing, so the kids were occupied while we ate. ;) After that, we wandered around Lowe’s for a while. I do love Lowes. It’s one of my favorite stores. Elizabeth likes it, too. She likes to look at the kitchens and the appliances. Like mother, like daughter.



Then we headed back to the house. The girls played legos, and Mama and I played Skipbo. We also taught Elizabeth how to play. We had worn Daddy out, so he just rested. Heheh :) Then, we turned in.

The next morning, we headed to Erin. I like to take the girls to my grandparent’s home place, and Daddy had some boards that I wanted to build a storage unit for Amy’s room. We played over there for quite a while and had a good time.

 this was fascinating to the kiddos. ;)

 You can see my great-grandparents house in this picture. Sometimes, we got to run through the field to their house.

As children, we were never allowed to touch the cistern.

 I'm a rebel in my old age, though.

 This is the tree where all the DRY FLIES were. ;)



Then, we headed back home. We really had SUCH a good time. We did have to stop at Taco Bell and get beans for Amy on the way, though. You know that girl and her beans!!!

Unfortunately, all this country fun took its toll on Elizabeth. She went to school yesterday, but I had to go get her. Her allergies were all kinds of messed up. Her teacher called me and said, “Something is WRONG with Elizabeth.” She stayed at school about 45 minutes before calling me to come and get her. Three doses of Benadryl and a good night of sleep later, she is back to her old self.

I.C.U. ( as in "I see you") :)

So, yesterday was the day we had been waiting for (with a combination of hope and dread) since January.


Amy and I actually left Thursday night. Our appt was 730 on Friday, and I just had no desire to fight Memphis traffic, (plus I had a free night from hotels.com) so we went down early. I found a hotel about two minutes from the hotel. We found out on Wednesday that we could have stayed at the FedEx house, but we already had reservations, so we just went ahead. From the pics I saw of the FedEx house, I might have never been able to get Amy to leave, so it was probably a good thing.

The hotel we stay at was probably the smallest hotel I have even stayed in…maybe 30 rooms? It was very trendy. My only problem was no vending machines. No quite so cool when you want to give your kid midnight snack because you know they won’t be able to eat breakfast the next morning, but we survived.

Neither of us slept very well. I just got a king bed because I knew Amy would sleep with me. She told me, “ You are on the Mama side and I am on the Daddy side. I will be Daddy.” Then she rolled over and pretended to snore like a buzz saw. HAHAHAHA! She also woke me up at three am by barking in my ear. Ah, adventures….

Friday morning, we got up and got ready and headed to LeBohnner. I could see it just fine, but had a difficult time finding parking. Then, we had to navigate the hospital maze to find the waiting room. When we finally made it, we were only about 15 minutes early instead of the 30 I had thought. It was fine, though. We waited out there until about 8, and then they took us back to pre-op. On the way, we stopped in Bunny Lane for Amy to pick out a toy to be hers and she chose a puppy that rolls on the ground and giggles. Everyone there was WONDERFUL. So nice and patient. However, no amount of wonderful could make up for the HELL of putting drops in Amy’s eyes. We finally ended up with me lying on her and two nurses putting them in. Three times. For a total of seven drops in each eye. :( After that she was pretty gun shy, and even testing her pulse was met with resistance, but we made it. She finally calmed down, and then a patient life specialist (Lauren) came to see us. She was SO SWEET. She talked to Amy about the anesthesia (but never called it that) and how they would do it, and that she could have popsicles after she opened her eyes. Amy really liked her, and so did I. She brought Amy some pictures to color. Amy was pretty much exhausted at this point (about 915). I tried to get her to just go to sleep, but of course she would have nothing to do with that. Lauren came and took us to the playroom about 930, and we waited there until 10 when they came to get her for the procedure. (Only 15 minutes late, I think that’s pretty good.)

Amy took this picture. :)

Waiting Room

All ready!

In the playroom

I got to walk with her for a bit, and then she went with the nurses and I went back to the waiting room. She was bouncing and happy as she walked with them, so I felt very good about it.


I went down and got a snack, and made a few phone calls. I sat in the waiting room and read for a while. There was a lady on a couch beside me. She had her shoes off and her nasty crusty feet sticking out from under a blanket and her head covered up. She was snoring so loud that she eventually woke herself up. When she finally roused up, her mom yells across the room, “They done took her (sleeping nasty foot woman’s baby) back there.” SNFW went back to sleep. OH. MY. Is all I have to say about that.

Around 1130, they took me to a little room to talk to the dr. It was Dr I. We talked for quite a while. He said that he still thinks she has Malattia Levantinse (Doyne’s honeycomb macular dystrophy), but we will not be 100% until the blood work comes back (they drew blood while she was sleeping, thank goodness). If it is NOT this, they will test for other genetic disorders. At any rate, these tests will serve as a baseline for her in the future. If it IS this, she will be the youngest recorded case in the world. Yeah. :( Still no for sure treatment plan; and treatment may not even be needed. They will both be examined again in six months, and tests repeated in two years. If there is no change, we will maintain. If there is a change, we will go from there. Yeah, I know frustrates me, too, but I TRULY trust Dr. I and what he is telling us. There is not a “quick fix” for this. You cannot take a pill for it, nor have surgery to fix it. We are at a definite disadvantage in that we have no access to Jason’s paternal relatives, as they are all deceased, and the tests that Jason had as a child were not sophisticated enough to see this. The fact that Dr I keeps saying over and over “I can’t believe they can see as well as they can,” both comforts and concerns me. The ONLY immediate vision problem that Amy had is her night vision is slightly impaired. Well, you know what, so is mine, so that may not even be related to this.

After our meeting, he told me Amy still had a while longer. I went back out to the waiting room. He came back out again a bit later. He had mentioned donating some of Amy’s blood sample to a DNA bank, and needed to get my consent for that. Basically, I said that they can do whatever they want with the blood sample (already drawn) and compare that to her test results in any way they want, and it will be in no way associated with Amy, unless they find something that could help her. Of course, I did it. If it does not help her, hopefully it will help some other little child.

Finally, about 1200, they came and got me. Amy was out. She was very distressed when I got back to recovery, thrashing and talking out of her head. The nurse repeatedly assured me that it was TOTALLY normal. She finally did calm down and fell into a good sleep. We ended up having to wake her up with promises of popsicles. :) They took out her IV (such a tiny little straw like thing! Nothing like a grown up IV. It didn’t even hurt her when they took it out) She went potty, and ate her popsicle, and we got her dressed. I went and got the car, and the nurse brought her down in the trademark red wagon. We loaded her up, and we were on our way!

She did fine the rest of the way (and we had to stop for beans at Taco Bell on the way home). She was distressed last night b/c she had the little heart monitor things on her and we had to soak in the tub to get them off. By bed time, she was VERY tired. She slept well, and seems to be totally back to normal this morning.

So, the answer we got is that we still don’t have an answer. We are a step closer, but not there yet. As we were driving down there, I was really thinking about all we have to thankful for. I have a friend with a daughter Amy’s age who has already survived leukemia. Another friend who just donated part of her liver to save her infant son’s life. We have to take fish oil tablets at dinner. Amy has sight, and is in no immediate danger of losing it. In fact, her vision is better than mine. God placed us where we are for a reason, and we will ALL be fine. If a cure is needed, there WILL be one, in time. Until then, we will keep our faith in the Giver of Sight, the Giver of LIFE.

New Sink!!!

So, we needed a new sink, b/c ours leaked...a lot. And if you get a new sink, you need a new counter. We had been saying for YEARS we were going to get new counters.  

NEKKID CABINET!

 EEEWWWW!!!!! This is why you dont' look under the sink. :(

Jason had to go buy a new jigsaw, b/c the one he had was broken. While he was gone, the girls and I laid out the templates for the sink and faucet. When he got back, we started with the drilling/cutting.

Jason did the rest of the drilling, b/c this is what happened when I tried.

Yeah!!!

Cabinet Guts!!!

MY EYE!!! Complete with sawdust.

Jason and his new jigsaw.

heheh Purple toolbox. :)

This is what happens when you drop a countertop on your leg. :(

YOU HAVE TO POUND REALLY FAST TO GET THIS PART TO WORK!!!!

Jason did all the plumbing. :)

Isn't it pretty!!!!
(Yes, we put the other handle back on!)

Eyeball update...

AT THIS POINT, ELIZABETH AUBREY WILES IS CLEARCLEARCLEARCLEARCLEARCLEARCLEARCLEARCLEARCLEAR!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!


Her right eye is lazy, may need to wear a patch for a few hours each day. Teh dr cannot guarantee that she will never have the problem, but the fact that both J and A had it at such a young age is a VERY GOOD sign for E!!!!

Praise GOD!!!!! Miracle #1 has occured!!! Keep praying for 2 and 3!!!

Just one problem....a boy tried to kiss her in the waiting room. UHOH!!!! ;)

Super Speller!

Elizabeth won the spelling bee at her school a while back. She beat ALL the kids at her school-2nd thru 5th grade. We were a bit proud. ;)



Last weekend, she competed in the regional spelling at Union University. There were 37 kids there from all over West TN, grades 3-8. She was the ONLY 3rd grader. We kept hearing people make comments when they read the program “There’s a THIRD GRADER here!” I so wanted to say “THAT’S MY BABY!!!”

There was a good crowd! Elizabeth did so well. She beat about 20 of the kids, and came in 16th. I think that is AMAZING. When some of the kids would miss a word, you would hear them crying. I felt so sorry for them. :( When Elizabeth missed her word (kahuna!) she bounced back to us with a grin on her face. I didn’t have to worry about her! HAHAH!

After wards, we went to Red Robin to celebrate. She even got some free ice cream because it was a special day. :) Aunt Nancy gave her some special pills, so she even got to eat it.

Needless to say, we are VERY PROUD OF ELIZABETH!!!!
(I dunno what's going on with these pics. They won't play right. But, they are all here..some more than once)

I knew they were odd…

(As I TRY to explain this, please keep in mind I am NOT a science person.)



So, we went to Memphis again yesterday. There is absolutely no doubt in my mind that God has placed us where we are supposed to be.



We saw Dr. Allessandro Iannaccone. He was awesome. His nurse, Mindy, was amazing as well. We had both of them all to ourselves all afternoon. They did a thorough exam on both Jason and Amy. Dr I (get it? Dr I) was amazed that either of them could see as well as they can, given the extent of retinal damage they both have. Amy’s is comparable to Jason’s, which is not good considering Jason is 32, and Amy is 4. It was SO helpful to have Jason there, though. The dr could compare the two of them, and it really helped with the diagnosis. We did a complete medical family tree, and several test on both Jason and Amy. Jason laughed and said it wasn’t fair…he went to AMY’s appointment and JASON got stuck with the needle. Dr. I felt confident from the beginning that the issue was genetic, and by the time we left, we were 99% sure of it. He also feels confident that, after exams and testing on both of them, he knows what the problem is. This is amazing to me. The way he explained it, we have about 200 genes. Each gene has two parts, mom and dad. When we got there, we were looking at 400 options. When we left, he felt confident we were down to ONE. Based on the location of the damage (behind the retina, rather than in front), the damage is very indicative of dominate drusen. It is also called honeycomb macular dystrophy.

Doyne's honeycomb retinal dystrophy

This disorder has symptoms quite similar to those of AMD: drusen on the macula and at the edge of the optic nerve head, macular scarring, and neovascularization in late stages, with progressive loss of central vision. Symptoms typically arise during the fourth or fifth decade of life.



Doyne's disease has an autosomal dominant pattern of inheritance. The responsible gene has been mapped, cloned and sequenced. Based on sequence similarities, it has been given the name EGF-containing fibrillin-like extracellular matrix protein, abbreviated as EFEMP1. The protein, whose function is not yet known, is found behind the retinal pigment epithelium (RPE).



I’m sure that helped you as much as it did me. :)



So, what does this mean? Well, we have to keep in mind that the diagnosis is NOT 100% yet. Jason will have another test on March 31, and Amy will have more in April. She will have to be sedated for the tests, so we are not thrilled about that, but the test is just too intense for a 4 year old to handle while awake. Elizabeth will also go to be screened on March 31. The reason we have to wait is that Dr. I also practices in Italy, and will be headed there in the next week or so. In the mean time, we have vitamin supplements, including DHA for Amy and Lutein for Jason.



In the event we do receive and official diagnosis of Doyne’s, we will proceed with genetic testing. This will lead to possible stem cell therapy. I have no idea what this will involve, but I would assume they would take my “good” cells and give them to Amy, possibly Jason, and Elizabeth (if necessary, PRAYFULLY she has skirted this issue. She has been seeing an eye specialist for 5 years for other issues and this has never been mentioned.) I don’t want to begin to think about how they will take my cells, or give them to my family.



So, that’s where we are. I have absolutely no doubt in my mind that GOD placed us with Dr. I. We are going to be ok. Amy is not going to go blind from this. Jason is not going to go blind from this. They may both end up with decreased vision one day, but they will still be able to function. God is good, and He will see us through this.

These eyes....

Well, I had all these great plans for my first 2011 post. About what a great year it is, how it's started off SO much better than 2010, etc. And it has started out better, for the most part.
Elizabeth won the spelling bee at her school. As in, beat the entire school. She will compete regionally in March. :)
Jason is recovering nicely from his surgery on Christmas Eve. He is back to work and things are back to normal.
we are the "guinea pigs" for a new curriculum at church. One of our members has written a year long study and we are testing it out for him, and for Lifeway. We will read then entire Bible over the course of a year, and study it together as a church family. Changes are already visible, and we are all enjoying the study.

This leads us to Miss Amy Hope. We went for a basic eye check up the first week of the year. The dr. noticed something on her retina. he thought it was probably nothing, but wanted us to check on it to be sure. So, off we went to a retina specialist in Jackson. He said yes, there is definitely something in there, I think it's Stargardts, but I don't know for sure. Then we heard the sentence no parent wants to hear: Do you want to go to Vanderbilt or Lebohnner? After picking my jaw up off the floor, I decided on LeBohnner. He recommend a "Dr. A", a pediatric opthamologist, for us. After the doc left the room, the nurse said, "I shouldn't tell you this, but I have to. Dr. A has a horrible bedside manner. I used to work for Dr. Byrd, and many of our patients came to us as a result of not liking Dr. A. This is her name and number, if you decide to see her, tell her I said for her to see you." I thanked her, and went to make the appt with Dr. A, planning to discuss this with Jason when I got home. when I got to the appt desk, we discovered that Dr. A wouldn't even see Amy until APRIL. So, I said what about Vandy? One of the receptionists said, "Isn't there a Dr. Byrd in Memphis?" I jumped in and said "Oh, I've heard great things about her! Let's try that!" we saw her today, a week after we made the appointment. There is no doubt in my mind we were supposed to see this woman.
Anyway, she found something in Amy's eyes. A lot of something. She called it "retinal dystrophy". to be frank, it ain't good. We are going to see a dr. at Lebohnner specializing in Pediatric Hereditary Retinal Dystrophy on Thursday. I have been warned by Dr. Byrd that more than likely there will be another visit for testing, as it will have to be done under sedation. We also have to bring Jason with us, as the dr. may well want to examine him to get a better idea about Jason. I'm glad he will be with us.
I have read a bit about this, and I believe the dystrpohy is a generic term. I'm seeing a lot of "Cone Disease" and "Bests Disease" but I really don't know enough to know anything. At this point, I think I am going to STOP investigating, b/c I dont' like what I am reading, and dont' want to totally freak out about it. I had my breakdown on Sam Cooper Blvd this morning; now I have to assume "Taking care of business mode".

I would greatly appreciate your prayers for us. I dont' know what the outcome of all this will be. I know that it is all in God's hands. I know that this is happening for a reason. But, I would just assume it was not happening to my baby.