You've Had a Birthday Shout HOORAY!

The post title is the name of a song kids sing in primary for our church. So really it'll mean more to you if you know the song. Gracie had a birthday as of late. This is her blowing out the candles on her cake that she requested. She first asked for a castle cake and I had a pattern I was going to use, but then when she was talking on the phone to my sister she started describing a cake that is nothing like the cake in the pattern. So I did my best to create what she wanted. This was the result. James looked at it and said "Have you seen Monsters Inc lateley?" Yes, it does kind of look like a monster doesn't it? But she said it was EXACTLY what she wanted. And unlike usual cakes that look good, this one actually tasted good. It's our families favourite chocolate/strawberry/pudding cake made to look like a castle/monster.

She LOVES to help.

So excited to get her present from grandma. It'll help her pass the time during the countless hours that she spends waiting in doctors offices and hospitals. Oh yes, she and her sister had just finished playing pioneer in their pioneer dresses that they love so much.

Longer Hair

This picture was supposed to be the last one as the post is really about how well Gracie's hair is growing. But I wanted to include this one for a realism factor. Usually we only put in pictures where Gracie's eyes are both looking in the same direction, here is one where they aren't just so you can see what it looks like. Her prosthetic is most limited in motion to look up and outwards. As a result when it's most obvious to us that her right eye isn't real is when she's looking up at us. James actually came home with a really funny story. He was talking to a lady and something just wasn't quite right with her eyes. Given that we have a daughter with a prosthetic eye that is the first thing he thought of. So through the entire conversation he just kept staring at her eyes and wondering (trying not to make it too obvious). He wasn't trying to be rude, it's just something you notice more now that you see it every day in your daughter. But then she looked up and both eyes looked up, making it very clear that it wasn't prosthetic she probably just had lazy eye. However there are times when James and I find ourselves having to remind ourselves which one of Gracie's eyes is fake. It's just such an amazing prosthetic. We're so happy with our ocularist and the work he does. Now if we can only work on insurance and their ridiculously inadequate coverage of a prosthetic. Ah, the joys of working a system.

Yesterday I realized that if I leave some bangs out Gracie can wear little tiny piggy tails. Her hair was already dried when I put them in so the bangs don't look their best, but still it's showing real progress in the hair growth department. Before we know it she'll be back to pony tails. Oh yes, she's not wearing glasses because she left them on the floor for our dog to eat them. This picture was taken right before we left to go pick out her new glasses. Stayed tuned for a post of her modeling her new glasses. Once again, they're pink.

This is Gracie playing around before church on the first day that we realized we could pull her hair back in an elastic. Her hair is so thick and so curly that it takes a lot of gel to hold it in place when it's this short or else she just looks like a fluff ball. James loves that look, he thinks it's hilarious. Gracie looks in the mirror in the morning and says "Mom my hair is CRAZY!" But when it's done she just looks super cute.

A Call For Educational Reform in America!

Our daughter in second grade came home with an English test which had been graded. One of the questions desperately needs to be amended. It reads:

When I run, my _________________ falls off.

a. sneaker

b. eye

c. story

They graded it as if "a" was the only answer. James looked at it and started to laugh. He showed it to me and we both agreed that this definitely wasn't as straight forward of an answer as the teacher supposed. We believe that the answer a. or b. would be an acceptable one. Given an eye would more correctly fall out than off, but more or less it's the same thing. Ah, some of the things you get a kick out of when you have a monocular child. We were just discussing a primary song that they sing in our church called My Heavenly Father Loves Me. The choir just sang it in General Conference this morning and it's one of my very favourites. It's all about the beautiful world the Lord has given us and our wonderful bodies. The opening line of the second verse states "He gave me my eyes that I might see the colour of butterfly wings". We once again laughed and taught Gracie that it's okay if she sings "eye" rather than "eyes" but then there isn't enough room to add "Mr. Craig gave me my other one". Yes, we're full of one eyed jokes in our family. Don't worry we deliver them in a way Gracie finds hilarious. I'm not sure that they'd always be so funny. I guess like all things it depends on who says them and the spirit in which they're delivered. If she wasn't having part in the fun we'd all stop.

On another note Gracie had her 14th EUA yesterday. Just her dad took her this time. All looks really well. Dr. G. wishes that the larger tumor in the left eye looked more flat than it does, but we may not get that. This may be as good as it gets. He couldn't tell the difference between yesterdays pictures and last months pictures. In his words "We're nearing the end" of the laser treatments. Things actually look so good that she doesn't need to go back for another appointment until December! It seems that Gracie recovered from her anesthetic better than she ever has before and by the time James was called back to the recovery room she was stating that she was ready to go.

When Gracie goes for her next EUA she'll be going with her new baby brother! Yes, we're having another baby. James and I have both been genetically cleared for the RB1 mutation that Gracie has (which is the cause of her RB) but experience has shown geneticists that even when the parents don't test positive for the mutation there is a 5-7% chance of having another child with RB. It's due to a mosaic form of the mutation in which not all, or even most of your genes carry the mutation but more than one do. We know for certain that either the egg which created Gracie or the sperm which created Gracie had this mutation. So there is a small possibility that James or I carry more than one affected egg or sperm. For this risk our new baby has to undergo testing for RB until we can determine that he is negative for the same mutation Gracie has. We had the option to perform amniocentesis and run the genetic tests in the prenatal period but we decided not to take even the small risk that this posed for the fetus. When we explained this to Gracie's geneticist she said that this was the same decision that most families come to who decide that they wouldn't terminate the pregnancy regardless of the outcome. We will be drawing blood on this baby at the December appointment to send to Toronto for the genetic testing. Toronto has the absolute best RB testing lab in the world. In the first couple of days after the baby is born he's seeing our pediatric opthalmologist here just in the clinic to rule out any tumors that would be an immediate threat. At the December appointment in Houston (when his genetic tests will be drawn) he will also undergo an EUA so that Dr. G can 100% confirm that there are no tumors on the retinas. After that we should have his genetic tests back in a couple months and he won't be required to do any further testing for RB. Of course any child should routinely see an optometrist trained to do pediatric exams including retinal exams with a dilated pupil, this isn't only looking for sporadic cases of RB but also for a numerous host of other eye conditions where early detection is critical. And oh yes, although James and I have this 5-7% risk cousins and aunts and uncles are still in the clear. So don't worry you don't need to all run out and test your kids.

Stephanie

Some pics for Gracie

Here is Gracie in the rain storm. We had not had rain all year and so when it came Gracie and her little brother headed out to dance in the rain!!
Gracie loves to help her mom bake. Especially when the baking includes chocolate!

In other news, we are so excited for Noah and his family (they are friends in Boston). We have been very worried and been praying constantly for them over the last two days. They had done an MRI and were concerned about cancer in Noah's brain. However, we just got word that the tests are not indicative of cancer. Hooray!! We are so excited and reminded once again of the miracles from faith and prayers.

Lucky #13

Gracie had her 13th EUA on Friday August 28th. Ah, a full year of going to Houston at least every four weeks. Well I'm excited to report that everything went well. The right socket looks excellent. There are no new tumors and the two tumors in the left eye continue to die. Dr. G figures he'll only laser the tumor one or two more times and then we'll just continue with observation, no more treatments (unless necessary). So the excellent news is that we can now extend the time between EUA's to every 6 weeks. It might not seem so exciting to everyone else, but this is thrilling to us as it cuts out one trip to Houston every 3 months! Remember it's a three hour drive one way so each time we go it takes our entire day. For the EUA we had an anesthesiologist who we've never had before. After the exam she came to talk to me in the recovery room. She told me how happy she was to take care of Gracie mentioning that "That's one sweet little baby girl you've got" I had to answer with "Thank you, she is isn't she?" I'm so glad I'm her mom - I must have the best job in the whole world. We're grateful for all the blessings in our life, whether big or small.

Oh yes, yesterday morning I was watching Gracie as she was playing with a small teddy bear which only has one eye (thanks to our dog). Gracie was talking to it and said "Oh look, your eye is broken!" To which I replied "Should we get it a fake eye just like yours?" Her answer was "Yes, because fake eyes are really cool!" Obviously this prosthetic hasn't set her back a bit.

Stephanie

Diagnosis Day

Gracie and her siblings

Gracie with her bear "Diagnosis Day" cake. Her hair gets a little crazy after a hard day of playing, but she's still cute as can be!

Today is the one year anniversary of Gracie being diagnosed with bilateral retinoblastoma. Really this month has been full of notable days, and we've remembered each and every one of them (although not with an actual party, more just a passing mention). On August 9 I first saw the white mass in Gracie's eye, on August 18 we first saw the opthalmologist and heard the word cancer mentioned as a possibility, August 19 she had her first CT scan which indicated it most likely was cancer. But it wasn't until August 25 when we were back in Austin and saw Dr. O that the diagnosis of bilateral retinoblastoma was confirmed. No one even thought of it being bilateral until that day and we continued to hold out hope that it was something else even in the right eye until it was 100% confirmed otherwise. But this isn't a sad day for our family today. Don't get me wrong a year ago we were completely devastated by the news we received today. But today we consider this a day to celebrate. Had we never known about Gracie's tumor's we could never treat them. Because of today we knew what we were dealing with and we had a plan of attack. Without diagnosis day we might not have her here with us today, and if we did we'd be fighting an uphill battle. So we rejoice in the many blessings that are ours today and for the blessing of having each of our children in our lives today. We are so grateful for the many miracles which have been manifest in our lives over the last year. God has been so good to us. We celebrated today with an ice cream cake from Baskin Robbin's which Gracie got to pick out with her older sisters help. It was a fun night. I look forward to celebrating many August 25th's as well as May 29th's. They are a celebration of life for our family.

Stephanie

Routine Check Up

Gracie met with her Austin oncologist Dr. L. yesterday as she needs to get scheduled for her routine hearing tests, kidney tests (GFR) and MRI. She needs the kidney and hearing tests to monitor how well they're functioning after chemo as the drugs she was on are known to impair them. The MRI is just something she is required to have every six months until she's six or seven because there is a very small risk for her developing trilateral retinoblastoma until then. Trilateral retinoblastoma is when tumors start growing in the pineal gland (in the brain). They don't spread there, rather it is just another site (like the retina) where the tumors can spontaneously grow. My theory is that trilateral rb is the reason why they say the survival rate of rb is 95-97% if contained to the eyes. Originally I was wondering why the survival wouldn't be 100% if it was contained to the eye, but then I remembered trilateral rb. In such a case you could remove both eyes at birth and still die of rb if it started to grow in the pineal gland as it's extremely difficult to treat. Thank heavens trilateral rb is extremely rare and we just check most kids to be ultra cautious.

The good thing about going to the Austin cancer clinic is that Gracie got to see her best friend Claudia, the child life specialist there. If you remember she is absolutely fantastic with Gracie. Of course we love Dr. L. too, but Claudia's whole job is to try to make the kids experience as good as possible. She's absolutely amazing at what she does. Remember she's the one who hooked our other kids up with Super Sibs and applied to Make A Wish for Gracie. Anyway, Claudia gave Gracie some more beads of courage to add to her collection. She has now started her third string! Claudia also mentioned that they'd like to throw Gracie an end of treatment party. This is something that they do for all children when they finish treatment, but I didn't think Gracie would get one here because she didn't actually do her treatment here. Although she did spend a lot of time at the clinic due to all of the transfusions she required (9 in total). So sometime in the next few weeks we'll go back to have her end of treatment party. A cake is made and donated by the wonderful "A Taste of Bountiful Bakery" and Gracie gets to choose exactly what she wants. So far she is requesting a strawberry Belle cake with pink icing. Interesting. Our family is really excited as all the kids are invited, we'll even pull them out of school if necessary. We're so grateful for the wonderful care we receive here.

Stephanie

EUA #12

Yesterday Gracie and James headed off to Houston for her routine EUA. For the first time ever I stayed home as we had 3 sick kids here that needed their mom too. This trip was a little exciting though because we were told to be there at 1130 for a 1:30 OR time. I received a phone call at 11am asking if Grace was coming. When I said yes the lady on the phone asked what time we'd been told to arrive. Apparently she was really supposed to be there at 1030 for a 1230 OR time. James had left a little late (even for the 1130 arrival time) so now we were really running behind. You can only speed up a 3 hour drive so much. The staff all know us well though so they were all quite understanding of the miscommunication. We've been going there for almost a year now and we've never been late before. Wouldn't you know it that this was the day that they were actually ahead of schedule (the last two EUA's they've been over 3 hours behind schedule). By the time James and Gracie arrived people were working like crazy to get them in as soon as possible. How nice not to have to wait! But hopefully it won't happen like that again.

The EUA went well. No new growth. Dr. G still wants to keep lasering the one tumor every 4 weeks as he's not yet 100% convinced that it's completely dead. We totally support him in that decision as we certainly don't want the tumor to start growing again and be set back several months in treatment. After the EUA Gracie had a surgeon come in and remove her port. So now she's port free!! We're glad she was able to have it when she needed it, but now that she's done with chemo let's get that thing out of there. The fewer foreign objects one has in their body the better. Now if she has a fever she's just like every other child, with the port in a fever required a trip to the ER. We've been very blessed that she's only had one fever in the 11 months that she's had the port.

We consider ourselves so fortunate that Gracie's left eye has such a good prognosis and that her vision is still excellent in that eye. So many bilateral RB kids end up with either both eyes removed or the remaining eye has such limited vision after treatment that they're legally blind any way. How blessed we are that there was such disparity in Gracie's left and right eye. We are quite certain that although Gracie will only have monocular vision that the acuity she has will always be good, even though the field of vision is somewhat limited. The Lord really has been taking such good care of us, not only through the past year, but through our entire lives. Life couldn't be better.

Stephanie

Day 2 The Eye Is Complete

Here is the eye all painted, it still needs another layer of whatever the eye is made of and some buffing and polishing until it is complete. Prosthetic eyes are made of plastic polymer, they used to be made of glass until WWII. The glass they used to make them was supplied from Germany so obviously was no longer available at the time. Some doctors? (I don't remember if that's the profession or not) and dental technicians (I know I remember that one right) got together and developed this plastic polymer for making prosthetic eyes out of. It turns out this material is lighter, safer and more durable so obviously they never returned to making glass eyes after the war ended. The blood vessels of the eye were the most amazing part for me. See the red thread, the kind you can buy at any fabric store. This is what Gracie's vessels in her right eye are. He cut a small piece off and frayed it until it was very very thin and then put them onto the eye. Once those were on he used paint to finish up the sclera as no one's sclera is truly white.

Gracie and her ocularist once the eye is totally complete. Her prosthetic is her right eye, his is his left. I had to demonstrate that I was able to put it in myself before I was able to take it home. Plus I got a little lesson on prosthetic eye care. Basically I just leave it alone unless it comes out. If it comes out I clean it with baby shampoo as it leaves no residue and pop that puppy back in there. She has improved so much with letting me put her eye in. She didn't even really fight when I took my turn and put it in.

A close up of Gracie modeling her eye. Oh, and the eye was lighter yesterday. He said they always have to make the eye lighter to begin with and then they darken it as required. If the eye is too light it's easy to darken it, but if it's too dark you basically have to start again from scratch. I think it's a pretty good match in the end. The eye looks great, but I wish the movement was better. The ocularist says that will only improve over the years. She still has swelling around the eye that could last for another year, as well as she grows into the implant better (remember they put in an adult sized implant so that they wouldn't have to repeat the surgery as she grows older) the prosthesis will suction to the implant more and allow better movement.

Day 1 of Making An Eye

Gracie had her first of two days meeting with the ocularist today in order to make her new eye. She was such a trooper! I was amazed at how well she did, and she was pretty proud of herself too. We took a few pictures to kind of chronicle the process.

Obviously her conformer had to be removed before he could start making a mold of her eye socket. Here is a picture of her without it in. It might not be as noticeable in the picture as in real life but her eye lids look much more sunken without the conformer in. That thing does an excellent job of giving the eye lids some shape.

I learned that there are a couple of different ways to make a mold of the eye socket. One commonly used is to inject a putty type stuff into the socket and let it harden. It's kind of like getting a mold of your teeth done. This is what they have video of on YouTube. Our ocularist feels that that method is quite traumatic on children, often they do this while they're still under anesthesia. He prefers to use a different method. It is much more pain staking as he makes a mold with wax by hand. He had to put it in and out of Gracie's eye at least 20 times to make sure he had it just right. It's his belief that you actually get a better fit with a child this way. Once he had the exterior mold done then he put some gel inside it and took a mold of the interior of the socket. The gel sat in her eye for about two minutes. When set it is the consistency of a boiled egg white. This was the part she liked the least as it feels pretty cold in the socket. But despite that she did her job well, she tolerated him playing with her eye for over two hours and the cold gel in her eye for two minutes. Way to go Gracie!!

Here's Gracie modeling the mold in her eye. She kept calling it her bubble gum eye as it's just the right colour so the sheet of wax really did look like a sheet of bubble gum. The hardest part of making an eye is getting a proper fit. He spent two hours making a mold and only about 30 minutes painting the iris (if that).

I thought that he painted the iris onto the eye itself. But no, the iris is a separate disc that he painted today and then inserts into the eye as it's being cast. So here is Gracie's iris being painted. Oh, in case any of you were wondering you are required to have a bachelor's degree preferably in the sciences in order to be accepted to apprentice to become an ocularist. Then the apprenticeship is 5 years of on the job training. And yes, I did ask permission before taking all these pictures of him working. He didn't mind a bit.

A close up of Gracie's iris. The iris is painted on a little round disk. The pupil is separate from that disk. It's a little suction cup looking thing with a black pupil in the center. The two (iris and pupil) are obviously joined together while the eye is being made, and the clear little handle which you see on the front of the eye is removed sometime during the process too. The black handle in the back is not attached to the iris, it's just there so he can handle it better.

The iris/pupil and Gracie. In the pictures it appears that the iris is a little too light to me. I didn't notice that until I got home. We'll have to see how it all turns out tomorrow. Tomorrow is when the sclera (white of the eye) gets painted and the final fitting gets done. AND we get to take home a new eye. Today was the hardest day by far for the process, or so we've been told, so tomorrow should be a piece of cake!