Tuesday, April 28, 2009

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.

16 comments:

  1. Amy and Brian --

    What a beautiful job you have done trying to help others catch this disease at an early stage. We're so glad that you were observant enough to take Ryan to have his eyes checked and that the prognosis is so good. We'll keep him in our prayers.

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  2. Mrs. Amy and Brian you know we love Ryan and Riley so much we just cant help ourselves.God knows best for Ryan and he will be just fine. Just believe and be calm your little angel will be ok. We Love you~

    Ryan's Teachers
    Sandy, Freda and Clareesa

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  3. Give our nephew a hug and kisses from his Uncle Mike and Aunt Jen today. We are praying for a successful outcome. Love you guys.

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  4. Thank you for your insight! We just found out that our son has Coat's disease and are looking to have his first Cryotherpy procedure done ASAP. It was actually the eye exam at school that detected something was wrong with his eye. Looking back at photos of him, we now notice the yellow glow in his left eye. He is seven years old and the photos of the yellow eye did not show up till about a year ago so hopefully it has not progressed too much. Like your son, he is totally unaffected by it. Thanks God!!!

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  5. Our son was diagnosed with Coats 10 years ago, when he was six. Today he has very little vision left in his left eye. But it has not affected his life in any way. He is a star athlete and scholar. He good eye seems to have developed amazing depth perception and peripheral vision. Don't worry, God takes care of his children!

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  6. Thank you for your information. Photos of my year old grandson show a pronounced glow but the doctor didn't think there was a problem when he looked into his eyes. However, he didn't dilate his eyes. Is that important part of correct detection? Also, did his eyes ever show red in photographs with sometimes a glow instead?

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  7. Layer's Disorder is a unusual eye condition, producing full or partially loss of sight, recognized by irregular development of blood stream vessels regarding the retina.

    Cataracts for Dogs

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  8. Hi, I saw your blog and was curious about your son's improvement. My daughter has recently been diagnosed with Coat's disease and is now undergoing laser and Cryo therapy.

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  9. Amy and Brian-

    I'm not sure if you are still actively involved in this blog so you may not see this comment, but I came across it on the Internet and felt compelled to comment. Believe it or not my name is Ryan and I have coats' disease in my right eye. Just like your son the doctors originally thought I had cancer and my dad still becomes emotional when he tells me he first realized the doctors were sending me to the oncology department of our hospital. Thankfully, however it was not cancer and my eye was able to be saved. This was in 1991 or so when I was only 18 months old. I am now 22 years old and can tell you that I have lived a completely normal life. I have very limited vision in my right eye, mostly just peripheral but it has not greatly affected me. My greatest challenge growing up was cosmetic in nature in that I was self-conscious of my eye looking different because it did not have the same chance as my left eye to fully develop. I have learned that I was making a bigger deal of it than other people and most never notice anything. Other than that just minor inconveniences such as having to get a doctors clearance for my drivers permit and not being able to watch 3D movies. I also was never very good at baseball but this has become somewhat of a self-depricating joke of mine. I have learned to accept whatever minor limitations I have and realized that I am extremely lucky and my life has not been greatly affected by a disease that for whatever reason I went through two decades ago. I have always tried to make the best of my disease and have become more open about it with everyone. The good news is my left eye is very strong and I joke with my dad that I can see better out of one eye than he can with two. I hope my experience gives you comfort for your Ryan in knowing that he will still be able to lead a perfectly normal and full life. I hope that he and your entire family are doing well. Take care.
    -Ryan

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  11. Dear Mrs. Amy, Sorry to know about ur son, my daughter Saanvi is also diagnosed with Coats' disease when she was about to turn 2 in 2012. Please tell us the current situation of Ryan from 2010 till now. Please give me your contact number & Email-ID on babyycare@gmail.com

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  12. I really enjoy reading and also appreciate your work.

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  13. The blog was nice and I loved the story of Ryan who fight with coat’s disease, the blog was really informative.
    Serrapeptase

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  14. Thank you for sharing this information! I wish Ryan all the best in his future! I'm a 40 year old male who had the sudden onset of a circular "shadow" in my right eye. After two weeks of it, I went to see an optometrist. He dilated my eyes and performed his exam. He recommended seeing a retinal specialist as soon as possible. I went the next day, had my eyes dilated again, and they took several pictures, injected a fluorescent dye into my vein, and then took more pictures. I was diagnosed with Coat's disease. I understand that this affect mostly very young males, but I have had great vision all of my life. Again, I'm now 40 and am looking into treatment options. The doctor told me that sometimes this resolves itself (the leaking), but that I would probably always have that shadow there. In a couple of weeks, I have a follow-up appointment. I'd also like to know how Ryan is doing since it have been a couple of years.
    Dave

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  15. Wow, I am a young mom myself and I have one baby boy and he has had eye problems himself. Your baby boy has a long road ahead of him. But he is such a cutie! He looks like a really happy boy. My boy has a retinal occlusion that they are now starting work on. He is going to a retinal disorder treatment center in Tucson, AZ they have made me feel more confident for sure. Your post has really helped me calm down today, thank you.

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  16. My daughters eye looks very much like this as we have just noticed going through pics of her, I googled yellow glare in eyes on photos and noticed your page, will be taking her to gp tomorrow after reading, thank you gor shating and good luck to you all x

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