Much to be thankful about: Surgery #4

Fourth Surgery
Ryan's 4th surgery was today. This time, we got really good news. Dr. Hubbard sees a very big difference between the amount of exhudiate (yellow stuff leaking from the vessels) on and under the retina. This means that his body is absorbing some of it. Also, the ones he's treated in the past have not sprung new leaks. And the best news -- he performed cryo on the final quadrant. So, now, all of the leaking vessels have been treated, which means his vision won't get worse and could even get a bit better as his body absorbs more of the exhudiate. More than likely, though, it will stay the same as it is now. We go back to the pediatric opthamologist in January, so he'll have a more accurate screening of his vision in that eye then.

What's Next? Ryan will go back in February for an exam under anesthesia. That means that he'll still need the dreaded drops, the anesthesia and IV, but won't have to undergo the invasive procedure and the recovery (more eyedrops). I'm not sure how many exams under anesthesia he'll need after that for monitoring, but probably every few months before they can be more spread out. His eye will likely turn out a bit more, so he'll need surgery in an attempt at straigntening it.

Thank you.
How appropriate that this surgery (and result) fell on Thanksgiving week! Without the outpouring of love and support from you -- our family, friends (new and old), neighbors, colleagues and even strangers who have reached out -- this would have been a much more difficult few months. We cannot thank you all enough for your support and prayers. I know they helped. We've connected with some wonderful families as a result of this, and for the new families who are stumbling across this blog after the initial diagnosis, we know how scary it is, but take comfort in knowing how resilient children are, and that you will see the other side of this soon. See below -- Ryan's not much worse for the wear!

Happy Thanksgiving!!

I just had to include a (not so good scan of) Ryan's recent school photo:

Third Surgery - August 24th

Whew! What a summer! It's taken a while to post this because it's been such a busy month! Our 5-year-old started kindergarten and we've had lots of fun trips to the lake with friends and guests coming into town. Between that, work and everything else, the weeks are flying by.

3rd Surgery. Ryan's 3rd surgery on the 24th went really well. His surgeon reported that he treated more vessels and the ones already treated remain treated (no additional leaks in those). We were thrilled to hear that he really sees progress this time and wants to wait 3 months before doing another round (instead of 2). That will give some time for this latest avastin injection to take effect and for some of the exhudiate (the yellow garbage causing his vision loss) to be absorbed by the body.

On November 23rd, he will examine him again under anesthesia and perform cryotherapy to any remaining leaking vessels, and there is a chance (fingers crossed!) it will be the last time he needs the cryotherapy. After that, he'll have an exam under anesthesia every 3 months until he feels confident that there is no more leaking. Then, once a year, he'll be examined under anesthesia. The problem with this disease is that the leakage can recur at a later date, so he'll always need to be monitored.

Glasses? Well, let's say we're trying. Our sweet, sweet friends who are eye doctors sent Ryan 3 pair of very nice glasses. He likes them for a few minutes until the novelty wears off, then they end up on the floor, on the playground at school, or missing for a few days. It's difficult to convince a 3 year old to wear glasses when they don't actually improve his vision. His are for protection purposes only. I think the closer he gets to 4, the easier it will be and even this week, he's getting better about keeping them on (a little bribery goes a long way!)
In any event, he looks adorable in them. Here is a photo:

And seconds later, without them, sneaking out into the garage to play:

And last, a photo of our daughter on her first day of kindergarten. It's been an adjustment for Ryan, too, since he's so used to having his sister in the next classroom. They are growing up!

Second Surgery -- June 29th

Round 2:
Well, we made it through another one. In a lot of ways it was harder than the first and in other ways, easier. First, a surgery at 6:00 a.m. beats one at 2:00 p.m. from a no-eating perspective. We rolled Ryan out of bed and into the car at 5:30 this morning. He did not flip out as we feared when we got there -- maybe he doesn't remember, but he was fine until the nurse came in with the dreaded drops. We're regulars now, so we know the parking deck, the procedure and the nurses (who are fantastic, we might add!). Peanut now accompanies Ryan to surgery. Here he is with Peanut this morning (who also had dilation drops, compliments of Ryan):

Interestingly, he wasn't nearly as "loopy" as he was the first time after the liquid anesthesia. It seemed to take longer for him to start slurring his words and when they took him away, he was saying (somewhat coherently) that he wanted to go to the beach...

The result.
The procedure went well. Dr. Hubbard said the vessels that he'd zapped the first time look much better (less enflamed). The better news was that the untreated ones looked less "angry and engorged" leading us to believe the avastin is doing its thing. He did see more exhudiate (the yellow leakage that causes the vision loss)than last time under the retina but he said that was common (it gets worse before it gets better). He said that he'd like to do another round in 2 months, and would likely need to do a total of 2-3 more. So, hopefully by the end of the year, we'll be almost finished with the surgeries. After those are complete, he'll have several exams under anesthesia so that he can verify the lack of leakage, and treat any as necessary.

The best news.
The best news is Ryan woke up from the anesthesia much better this time. The first time, he woke up in the recovery room without us. This time, he was wheeled back to us before he woke up, so we were there when he did, and didn't flip out nearly as much as the first time. He really only cried when they removed the IV. We put him in the car and were home by 11 a.m. He slept on the couch until about 2:30, then woke up asking to eat, so we are thrilled with his recovery so far. He's watching Diego with a handfull of chocolate chip cookies - sitting at the table as if he did not just have surgery! His eye is much less swollen than it was the first time. Other than being a bit red and still quite dilated, you wouldn't know he had surgery 6 hours ago. We were really anxious about this recovery, so we're really pleased. We hope tonight goes as well. Now, 7 days of dreaded drops (4x a day) and we're home free until the end of August...

Ryan and his "lunch":


Some Medical Information:
Brian asked the doc why he treats the vessels with cryotherapy instead of laser treatments. He said the lasers don't work with all of the leakage currently present in the eye. Later on, once the body (hopefully) absorbs some of that leakage, they can treat some of the remaining vessels with the laser.


Thanks again for all your notes, prayers and well wishes.

How Common is Coat's?

What are the odds?

Since not a single person we've told has ever heard of this disease or knows someone who has it, we've been looking for a statistic. I'm not sure why -- perhaps to torture ourselves a bit more.

I found this site which lists various rare diseases and their prevalence:
http://www.orpha.net/orphacom/cahiers/docs/GB/Prevalence_of_rare_diseases_by_decreasing_prevalence_or_cases.pdf

Coat's is listed as 2 in 100,000.

If the US population is about 305,000,000, at 1:50,000 there are about 75 new cases each year. Not having been a math major, this may not be an exact calculation, but shows you how uncommon this is.

Yellow Eye in Photos

When Ryan was initially diagnosed with retinoblastoma, rather than Coat's, we went through thousands of photos, trying to pinpoint when the disease first presented. Online sites lead me to believe that children are born with the disease, but present at different times and at different stages. Some cases are detected even in newborns -- others, not until age 10 or later. Recently, I went looking into some old photos, and realized I'd missed a bunch of photos I'd taken of Ryan between ages 1 and 2. We'd been somewhat comforted by thinking that this developed within the last year (sometime after his 2nd birthday, since the pediatrician did not see it at his 2-year-old check-up in May of 2008). I was stunned to see how many earlier photos show the disease. I'm posting some here for several reasons. Some were taken in different rooms (but on the same day) with different lighting and as you'll see, it it shows up in some, and not others. We've tried to comfort ourselves (because it's impossibly hard not to blame ourselves for not taking him to see an eye doctor sooner) by the inconsistencies in photos. The yellow eye bothered us, but when we saw it in maybe 10 of 100 photos, we dismissed it is a camera malfunction. Of course, now that we know, it couldn't be more obvious. For example, these two were taken on easter morning, 2008, taken one after another as you can see by the outfit and location of the photo(if you click on the photo, it will show you a close up, but you have to hit your back browser instead of closing the photo, or you'll close the blog):


And, these were taken on the same day: Christmas, 2007:



I think it's also helpful to see photos of Ryan's eyes as a baby (about 9 months in this photo). It was not present in any photos taken before his first birthday.

I also think it's helpful to see the "normal" red eye reflex -- this photo was taken when he was about 16 months old.

And this one at 18 months:

Also, since we are fortunate to have Nashville's best photographer as our sister, her equipment and flash are so advanced that they "mimic" natural light. It would not have shown up in any of her photos. For example:

First Post-Op Appt

The appointment. Ryan saw Dr. Hubbard today. Ryan and Peanut (his stuffed animal buddy who now accompanies us to each appointment) both had their eyes dilated. I wish I could say he takes the drops easier (with all the experience he now has) but not so much! Dr. Hubbard examined him and was pleased with his recovery. He does not typically do an in-office follow-up (he would just see him at the next scheduled surgery) but since he did the avastin injection, wanted to make sure he was healing well and had no negative reactions. So far, so good, on that front. He does think there is a reduction in the exudiation, but it will be much easier for him to tell when he does the next Exam Under Anesthesia on June 29th. At the same time, he'll do another round of chryotherapy on another set of vessels, and examine the ones that were treated last time. He'll do another avastin injection as well. It's likely that we'll do this at least 3 more times over the next year.

More Concerns.
Since Ryan's surgery, I've been doing what everyone says not to do -- search the internet for anything I can find on this disease. I still can't find statistics on the number of kids diagnosed with Coat's each year. It's that rare. What I have found is a very good site containing message boards with posts from families all over the world who are battling Coat's in some stage or form. From what I read, Ryan's case could be much better and it could be much worse. Sometimes it's good to be average... What scares me now is, even though we've come to terms (well, we have good days and bad days) on the fact that he will only see from his left eye and peripherally out of his right eye, are the secondary issues that he may face due to the disease, such as formation of cataracts, glaucoma, and atrophy of the eye. I read one frantic post from a mom that her daughter's eye was "dying" after her treatments which made my heart break for her. Of course, we want Ryan to appear as "normal" as possible, despite having such severe vision loss. I asked Dr. Hubbard about the risk of atrophy and he said in Ryan's case, the biggest risk for Ryan is Strabismus, a vision condition in which a person can not align both eyes simultaneously, so the eye turns out (commonly known as "lazy eye"). I asked about the dreaded patching of his non-Coat's eye (to strengthen the Coat's eye), but he said often the patching has little, if no, effect on affected eye, and surgery is often warranted instead to straighten the eye. Unfortunately, success rate for that surgery is limited when vision loss has already occurred, so we'll watch his eye closely and Dr. Hubbard will work in tandem with our pediatric opthamologist to determine the best plan for keeping the eye looking forward.

Also, since the unaffected eye will compensate for the loss of vision in the other eye, he will suffer some loss of depth perception; however, given his age, he will likely compensate for any depth perception loss better than he would if this condition presented later in his life. We'll just have to wait and see and hope that he can continue playing golf! Speaking of golf, when I put Ryan in the car after his appointment, he said: "mommy, I like Dr. Hubbard and Tiger Woods". He picked two good heroes!

Next surgery.
We'll post again after Ryan's next surgery scheduled for June 29th. In the meantime, we're spending a week at the beach and I'm told that Ryan can play in the water, sand, etc. like any other boy without an eye issue, which was a nice thing to hear.

The Recovery

Kids are amazing.
Within 24 hours of Ryan's surgery, the kid was grooving to American Idol. Kids are so resilient! He slept through the night following surgery, and woke up pretty angry and upset. He kept holding the eye and telling us that it hurt. After the tylenol kicked in, he wanted to go watch tv so he spent a good hour in front of the tv before he opened either eye. It was probably scary for him. Once open, the eye looked MUCH better than we'd anticipated. It was a bit swollen and red in the outside corner, but we were expecting him to resemble Rocky. He wasn't thrilled, not having eaten for 36 hours, so breakfast was grapes, a pop tart and fruit snacks. Whatever the patient wants, right? We waited a while before putting the first set of drops in and that wasn't very fun. He screamed and cried, so we were traumatized by the thought of doing that 4 times a day for a week. He slept all night the following night, but woke up in a similar manner -- in pain and angry, holding the eye. We repeated the previous days' events and by noon, he was ready to go back to school. He misses his friends and teachers, and they gave him a warm welcome. 48 hours after surgery, he was back at school chasing his friends (and girlfriend) around the playground.

The drops.
Brian always comes up with the ideas. Before his surgery, Ryan was given this sweet little yarn doll that he named "Peanut". Brian told Ryan to put drops in Peanut's eye (with the cap on) and sure enough, that did it. Now, before each eyedrop administration, Ryan puts the drops in Peanut's eyes, then offers himself up for drops. He still squeezes his eyes shut so tightly that it requires holding them open (and being quick!), but we've come a long way since those first ones. Hopefully soon he'll be administering them himself (well, that's a bit ambitious).

The party.
Ryan was back to 100% for his 3rd birthday party. His eye was a tad bit red, 5 days after surgery, but you really have to look closely to notice. He had the best time --even though it poured down rain! The jumpy turned into a slip n' slide which made the kids (not so much the parents) very happy! It was a celebration for us that we survived the first chapter of this and also that we have such an amazing kid! Some photos from the party are below. We all needed some smiles after the last two weeks!

Next Steps.
Ryan goes back for an office visit with Dr. Hubbard on May 27th. We're not sure what he'll be able to see as far as positive results of the first surgery in the office, but I imagine he'll track his recovery and we'll discuss next steps. He's scheduled for round 2 of surgery on June 29th. Now that round 1 is behind us, at least we know what to expect, we hope that round 2 won't be so difficult (assuming we can get him in the hospital doors now that he's on to us!). The search for glasses has started. That's our next hurdle!




Is this a happy face, or what?