National Retinoblastoma Strategy

This may not be of interest to people just checking in on Gracie, but if you happen to be concerned about retinoblastoma this will be extremely nice to know. Canada is the first country in the world to have published a multi disciplinary, multi institutional guideline for care of retinoblastoma throughout the nation. Sorry, as a proud Canadian I couldn't resist bragging about that. It was published in this months Canadian Journal of Ophthalmology and it is absolutely EXCELLENT. It is useful because RB is such a rare cancer that most facilities really don't know how to treat it best and as such children are getting diagnosed much too late and are not receiving the best care available. By creating these standards for care it should help to inform ALL opthalmologists and hospitals of what they need to be doing to treat RB as well as clarifying what criteria indicate that a child really needs to be referred to a more specialized center. By doing this it makes it absolutely clear that there is absolutely no excuse for doctors/facilities with limited RB experience to try and treat patients whose cases are way above their heads just for prides sake, which yes really does happen. It's a very long read so I've only read about half of it so far but I'm really impressed with it. Apparently Kenya is also trying to make a similar national strategy for care. Hopefully the other countries in the world will realize the need for just such a publication. When caring for kids who already have so much to deal with the care needs to be focused on what is in the best interest for the child and not what will bring the most money into the hospital. More collaboration and less competition would be nice if these kids are really going to get the best care possible.

Here is a link to the document if anyone is interested in reading this. You'll definitely want to read it if you are affected by RB. http://article.pubs.nrc-cnrc.gc.ca/RPAS/rpv?hm=HInit&calyLang=eng&journal=cjo&volume=44&afpf=i09-194.pdf

Grace has another brother!!

OK- so this picture is not about her baby brother but is from her 4 year check up. Everything looks really good but because of the chemo treatments she was behind on her shots and had to get 6 shots. She was not at all impressed when she saw the tray of needles and less impressed when I held her down and let the nurse stick her six times. All was well once she found her prize and sticker. She slept the entire afternoon while Stephanie and Gracie's new little brother got some one on one time!

This is Gracie modeling the dress that Stephanie made her. Gracie will wear it at my sister's wedding in December! She is pretty excited and extremely cute!

Here is Gracie's new brother at one day old. He looks pretty relaxed doesn't he? Gracie's hair is so funny. Usually Stephanie uses lots of gel to keep it from getting out of control but Gracie was gel-less this day. Her glasses are crooked in the picture but that is par for the course with a 4 year old. In fact it has been the standard for my glasses since I was 9 years old despite my mom's insistence that I not play basketball, football, wrestling, etc, etc with them on. A few months ago, I remembered her advice and took them off the play softball but when the game was over someone had stepped on them and they were all mangled up anyway.

Gracie is a huge help at home. She takes care of her new brother so well. She knows exactly what to do if he starts to cry at all. She just says, "Mom, the new born baby is hungry and needs you really really bad! Please come and feed him!"

The baby's eyes have been examined with dilation and they look "perfect." He will also have an exam under anesthesia and genetic testing in early December when Gracie returns for her next appointment. Oh the joys of having a child with a genetic disease.

Life has been crazy in our home with school and work, but we have been richly blessed. We continue to thank you all for your concern, your love and your support.

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