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?

First Surgery

It's Finally Here...
Although it seems like many months, not just 2 weeks, since Ryan's diagnosis, surgery day finally arrived. To make sure he was well enough for surgery, we took Ryan to his pediatrician to make sure he was not developing another sinus infection.
Having been in and out of doctor's offices more in the last week than in his entire life, Ryan was happy once he realized he did not have to have the "drops" at that visit! She did inspect his eye (and let me look through the pin-hole) and sure enough, you can see how yellow his eye is (the other eye has the normal red -- hence the "red-eye" in photos). It's like looking into an eye full of pollen (it is peak pollen season in Atlanta...) She confirmed that our surgeon is top notch and felt confident in our remaining in Atlanta for the procedure and with doing the Avastin injection, which is only recently being used for Coat's patients.

It almost didn't happen..
We were told that we'd get a call before Friday with a surgery time. When I hadn't heard by noon on Friday (although I was chained to my desk for fear I'd miss the call!) I called the hospital and was told Ryan wasn't on the already booked surgical calendar. This is not what an anxious mother wants to hear. After some begging and pleading, they added him to the end of the day - at 2:00.

Really? No food?
Now I know why they typically schedule children's surgeries first thing. It's not easy convincing a 2 (almost 3-year-old) to put down the pop tart and have a nice glass of water for breakfast. And lunch! I finally found a place to take him without food -- the car wash! Special thanks to a good friend who brought her 3 year old to hit a few golf balls with Ryan outside to help pass the time! Ryan absolutely loves to hit golf balls and Tiger Woods is his hero! We took extreme measures to lock Ryan out of the pantry -- Brian tied it shut with an actual belt. Ryan was not happy about that. Luckily, an almost 3-year-old boy believes that the pantry doors are broken.

Finally Made It!
As luck would have it, Emory's graduation was ending as we were nearing the hospital on Emory's campus with a very crabby child begging for snacks. The traffic was touch and go for a while -- we almost abandoned the car and walked! Lucky for us, they offer valet parking. We arrived on time and our luck turned when we saw a familiar face. Brian's former law clerk is now a nurse anesthetist and got on Ryan's case when she saw his name. She was wonderful and made us feel so much better, just knowing she was with him. Everything about Children's was impressive. We are so fortunate to live in a city with a hospital like Children's -- we'd never given it a second thought before now since we've only driven by it. It goes without saying that we will be recurring contributors!

The Prep.
Ryan was not happy about putting on the hospital gown and surgical cap, especially after 3 set of eyedrops (2 each time) were administered in both eyes. Uggh -- the drops! Once he took the dose of liquid anesthesia, though, he went on to la-la land and did not care what he was changed into! He was so cute that it was hard for us not to laugh and cry at the same time. Brian (you can always count on him for a good sense of humor) asked where we could get more of that stuff for bedtime! Once Ryan was back in the OR, the doctor came to tell us that he'd done an initial exam under anesthesia and found the disease to be pretty widespread, but confirmed that Ryan has a classic case of Coat's. We were his 12th surgery today - all of the prior surgeries and exams for retinoblastoma patients as young as 7 months, so as sad for Ryan as we were, and feeling like all the other scared, helpless parents, we felt fortunate to be there for Coat's. He told us the gameplan would be the avastin injection and chryotherapy.

The Surgery.
The procedure lasted about an hour, after which the doctor came to tell us that it went well and they were able to do the Avastin injection and perform chryotherapy on about 25% of the leaking blood vessels. Trying to get them all at one time is too much trauma on the eye. He had told us beforehand that sometimes the first surgery "triggers" leaking in others and so the condition can get worse before it gets better. It's a wait and see approach on both that and the effects of the avastin injection. He confirmed again that Ryan has good peripheral vision in that eye, but didn't sound optimistic about saving much central vision. Again, the goal is to save the actual eye, but saving some vision is ideal, even if it is not correctable.

The Recovery.
Well, we were warned about the post-op recovery! When he was brought back to us after surgery, Ryan was unhappy, to say the least! He was inconsolable (or as his big sis would say: "freaking out") which we understand is not unusual, but broke our hearts more than anything we've been through thus far. After about 45 minutes of flailing and holding the eye, he fell asleep such that they were able to remove his IV and we took him home. He slept the entire way home and transferred right to the couch. We hope he sleeps through the night so that his recovery is underway overnight. He hasn't opened the eye yet, but we understand he'll be pretty sore for the next day or so. The torture for him (and us) will be the 4 times daily antibiotic drops in the eye for a week. We're not above bribery! Good thing his birthday party is this weekend -- we're using that for all it's worth!

What's Next?
We take Ryan back in 2 weeks for a post-op checkup, to ensure the eye is healing properly and to assess the benefits from the avastin injection and status of the blood vessels treated with chryotherapy. He will do his second round (not sure if it will be only Chryo or another avastin shot as well) in 8 weeks. We appreciate this aggressive approach because the sooner we stop the leaking, the more vision is potentially saved. Unfortunately, this condition is likened to a hose in that you can patch a leak but it can spring a new one.

Our Continued Thanks.
We simply cannot get over how unbelievable our family and friends are. The outpouring of prayers, well wishes and support are truly what is keeping the wheels on!

Thank heaven for medicine.
Ryan will not be happy about this when he's old enough to read this, but here are photos of Ryan just after he swallowed the anesthesia medication (wearing the special snail hair cover he got for having an "in" with our wonderful nurse anesthetist!)

First Exam -- Treatment Plan

We met with Dr. Baker Hubbard today at Emory. He confirmed that Ryan has mid-staged Coat's Disease with slight retinal detachment. That it wasn't in a later stage and that his retina was still attached was good news to us. He is optimistic that it can be treated with one or both of Laser Photocoagulation and Cryotherapy. We really liked him and "team Ryan" has been busy gathering information on him -- his reputation (for his experience and bedside manner) is fantastic. We heard only positive things from a variety of sources (you know who you are and we thank you!)

The good news:
Ryan has peripheral vision in his Coat's eye. I assume he could tell by seeing where the fluid has accumulated on the back of his eye (imagine a glass of water with milk slowly being poured in and then trying to look through that glass). He estimates his vision to be about 400/20 in that eye, so unless there is no further deterioration of the vision from scar tissue after the surgeries, he should maintain that vision. I asked him whether they can drain the existing accumulation (such that his sight could be restored), but there is not yet a procedure for doing so. Perhaps some day there will be.

Avastin:
Some doctors are treating Coat's with a drug called Avastin, which is actually a drug used to treat cancer. Avastin is an FDA approved therapy designed to inhibit angiogenesis, the process by which new blood vessels develop. In cancer patients, this anti-angiogenic process starves the tumor of new blood cells. In Coat's
Disease, it is believed that Avastin would greatly inhibit the development of new blood vessels. This could potentially eliminate the need for repeated sessions of laser therapy and the scar tissue (and decreased vision) the scar tissue causes. We talked to Dr. Hubbard about Avastin and he has not yet used it in children to treat Coat's, but he uses it often to treat age-related macular degeneration, the leading cause of blindness in people over 50 years of age. He is interested in exploring it for Ryan. We'll do some research in the meantime on risks, side effects, etc.

Ryan's treatment plan:
Ryan's first surgery will take place at Children's Hospital on May 11th. He'll perform both Cryotherapy and perhaps Avastin (via an injection into the eye). A month later, he'll undergo an Exam Under Anesthesia (EUA) whereby the doctor will be able to tell how much of the leakage he was able to stop. We'll start the whole process over 2 months later, and with any luck, he'll only need the 2 cycles. He could need 3 or 4, but we're optimistic that two will be the charm! After he feels confident that the leaking has stopped, he'll see him every three months, then six months, then once a year. Unfortunately, the leakage could resume at a later date, so there's always a chance he'll have additional surgeries down the road. Then again, there's the chance that medical technology will progress such that his sight can be fully recovered.

The recovery:
We asked about recovery from the procedure. He said his eye will be pretty swollen that first day and maybe the day after and there will be a red spot on the white of his eye. He won't need to be bandaged, so that's probably a good thing from a "keeping a 3-year-old's eye bandaged" perspective. The timing is good because he'll have recovered for his 3rd birthday party (Diego theme, of course) the following Saturday.

Sigh of Relief:
We've been on a roller coaster of emotions. Relieved he doesn't have retinoblastoma, but scared and sad for the upcoming procedures and also knowing that our baby will always have to live with this and potentially lose all vision in that eye. Our family has always been so lucky in the health department and this has been a very humbling experience.

A funny story:
When we first arrived and were brought back to have Ryan's eyes dilated, Ryan flipped out (now he knows about "the drops" and does not like them!) The technician put a drop onto Ryan's hand to show him that they were no big deal. Ryan promptly rubbed his hand into Brian's right eye, so Brian's eye was dilated all day. Now he can emphathize with what Ryan's experiencing! Interestingly, the drops affect children and adults differently - this particular one lasts much longer in adults. Poor Brian.

Glasses:
In the meantime, we have to protect both eyes, so glasses and sunglasses will be part of Ryan's daily routine. We're starting with "Diego" sunglasses and work our way up...it's not easy convincing a 2-year-old to wear glasses, but he thinks sunglasses are cool.

Our story and Photos

Brian and I set up this blog for several reasons. First and foremost, we are blessed with extraordinary family and friends who have overwhelmed us with concern for Ryan and offers to assist. We want to keep everyone up to date on his battle with Coat's Disease. We are new to this, never having heard about the disease until last week. The other reason is perhaps another family will see similarities in photos of their child and seek an eye exam and diagnosis sooner. We will update this periodically to keep you informed, and perhaps another child's vision may be saved because they stumbled upon Ryan's story. This picture was taken a few weeks ago at Sea World. He was so happy, I just had to include it because it makes me smile. I've posted photos below showing the affected eye, but first, a recent family photo.



Here's our story:
We noticed in photos that our son's right eye (Ryan turns 3 on May 17th) turned out a bit in photos. We also sometimes noticed an odd "glow" in his right eye, also in photos. We brought him in for an eye exam on April 22nd. The optometrist told us that Ryan had very poor vision in his right eye and we talked about a patching schedule in an attempt to strengthen that eye. Thankfully, he proceeded to dilate his eyes (not always an easy task with a 2-year-old, but Ryan was a champ) and saw an issue with his retina. Suspecting Ryan had retinoblastoma (eye cancer) he immediately sent us to a pediatric ophthalmologist who determined instead that Ryan has Coat’s Disease (the two diseases exhibit similar symptoms).

Although Coat’s is very very rare, and really serious, next to eye cancer, we were beside ourselves with relief that he does not have to undergo chemo, radiation, etc. He’ll likely need multiple surgeries – we read that the surgery cycle is every 3 months. The goal of the surgeries is to "stop" the blood vessels from leaking into his eye. Along with surgery, he'll need eye therapy to prevent the eye from becoming "lazy" even though he will have little, if any, vision in that eye. He’ll be 3 in May, so won’t remember the initial procedures, but will always live with it because there is no “cure”. There are other potential problems associated with Coat's (such as the development of cataracts and glaucoma and some others.)

The other battle we'll face is protecting the unaffected eye. Since he'll essentially have eyesight in only one eye, he'll need to wear glasses and sports goggles to protect it. So far, Diego sunglasses are acceptable to him. We'll work our way up to glasses.

We have his first appointment on May 1st at Emory with Dr. Baker Hubbard, a retinal specialist, at the Emory Eye Clinic. We've heard wonderful things about him. The success rate for saving the eye is much better if it is caught sooner rather than later. We will not know what stage the eye is in until the appointment. If it is in its advanced stages, he could lose the eye.

I'm including some FAQ below about Coat's Disease gathered from the internet. We are very new to this and gathering as much knowledge as we can and with such a rare condition, there isn't much out there. We are trying to get all the answers and determine the best course of treatment for our little guy and keep our friends and family informed about what is going on. We're trying to keep our sense of humor, knowing we have a rough road in store for us, but thankful for the outpouring of support we've already experienced. We'll get through this.


About Coat's Disease:

What is Coats’ disease?
Coats’ disease is a progressive condition of the retinal capillaries which occurs in children and young adults -- most of those diagnosed are boys (I've read up to 81%). It is gradual in progress and affects central vision, usually in only one eye. [Ryan's is only in his right eye]

What are the causes of Coats’ disease?
At the moment there is no known cause of Coats’ disease. It is not hereditary.

What are the effects of Coats’ disease?
The main symptom of Coats’ disease is the deterioration in either central or peripheral vision.

What happens?
Coats’ disease causes capillaries in the retina to break open and leak fluid (cholesterol deposits) into the back of eye. The leakage causes the retina to swell, and can cause partial or complete detachment of the retina. It was described on one site as "pouring soap onto your retina".

Is there any treatment for Coats’ disease?
If caught early, some level of vision can be saved (although vision cannot be corrected -- simply keeping the existing vision). The treatment chosen depends on the stage at which the disease was diagnosed (see stages below) If not caught until its late stage, complete loss of vision can occur. In its final stages, enucleation (removal of the affected eye) is a potential outcome.

Surgery, usually by light (photocoagulation) or extreme cold (cryotherapy), can be used. Multiple surgeries are often performed in an attempt to stop the leaking.

What is the prognosis for Coats’ disease?
If applied early and depending on the stage of the disease, treatment may be successful in preventing progression and in some cases can improve vision but this is less effective if the retina has detached.

Stage 1: This stage is typically treated with Laser Therapy. In Stage 1, there is a high probability that some (or most) vision can be saved. Unfortunately, Coats’ Disease is rarely caught in Stage 1. Coats’ typically begins to progress at an age where the child either does not understand or can’t explain the symptoms. Onset of the disease can happen as early as 12 months.

Stage 2: This stage is typically treated with either Laser Photocoagulation or Cryotherapy, depending on the extent of the leakage.

In stage 2a, the chances of restoring some level of vision are pretty good, because the fovea is not yet involved. If the fovea has a dense yellow nodule in the center (this is the “yellow eye” seen in pictures), visual prognosis is not nearly as good.

In stage 2b, vision can still be restored if the fovea is not heavily affected.

Stage 3: (subretinal or retinal detachment). This stage is also divided into stages 3a and 3b. In stage 3a, Laser therapy or Cryotherapy can still be used. Because of the subretinal fluid, Laser treatment is not as effective as Cryotherapy.

In stage 3b, the retina is totally detached. Cryotherapy may be useful if the retinal detachment is shallow. Surgery to re-attach the retina may be required if the detachment is advanced and posterior to the lens.

Stage 4: There is most likely no chance for recovery of vision at this stage.

Stage 5: At this stage, total blindness is present and irreversible.

As you can see in the photos, Ryan's eye is already showing the yellow, so we are prepared for him to be diagnosed in a later stage.

Some Photos

Here are some photos of Ryan revealing the Coat's. At first, we dismissed it as a "red-eye" type reflection off of his "lazy eye".

The first photo was taken on his 2nd birthday in May 2008. Going back and looking through thousands (yes, thousands) of photos, we believe it began in the Spring of 2008. The Coat's appeared more as a "glow" than the "yellow" we see now.




This second photo was taken in September 2008 at the beach. Again, we dismissed this as a camera malfunction or red eye.



And now April, 2009:



We've been asked many times why it's so hard to detect and diagnose. A digital photo flash picks it up, as does an exam performed under dilation. Simply looking at him does not show the condition. We are thankful that the doctor decided to dialate his eyes; otherwise, many more months could have gone by and the conditioned worsened and his eyesight completely gone.

Blissfully Unaware


Here is a photo I took last weekend. The yellow is pretty pronounced now. We don't think he is in any pain (although he's been rubbing the eye). We know that he is about to endure many procedures, surgeries, eye drops, patching, glasses, sports goggles (his mother wanting to put him in a bubble so he doesn't get hurt) -- you name it. We're tortured parents, but he is blissfully unaware of what's to come.

Again, it is fascinating that the condition is not noticable "live" - it only shows up in digital photos when a flash is used (and not always even with a flash). Just as the red-eye effect is caused by a reflection off blood vessels in the back of a normal eye, an eye affected by Coats’ will glow yellow in photographs as light reflects off cholesterol deposits from the leaking vessels.