Jacob's "yellow eye" from Coats' Disease
                                                                              Spring of 2005

 

 

 Coats' disease was first identified by Scottish opthalmologist George Coats in 1908. For a brief biography of Dr. Coats, here is a link:

 http://www.whonamedit.com/doctor.cfm/1926.html

In June of 2005, our 3 year old son Jacob was diagnosed with Late Stage 4 Coats' Disease. He will not regain the vision in his right eye with the treatments available today. We started this website with the hopes of providing information for parents who are just learning about Coats' Disease. We also wanted to provide a forum for parents to share their stories, to ask their questions, and get support from some of the amazing parents that post on our message board. It is our sincere hope that you find some answers to your questions here, and we would love to hear from you.

It is now April of 2010, and Jacob is doing great. He still has his Coats' eye, although it is completely covered with a cataract. Removing the cataract would only be for cosmetic purposes, so we will leave it for now. Jacob is a completely normal 7 year old boy (almost 8!). He is doing great in school, and is now finishing up 3rd grade. He is doing extremely well with his school work and his social skills. We are so proud of what and who he has become.

What is Coats' Disease?

      Coats' disease is also known as Coats' Retinitis, Coats' Syndrome, Exudative Retinitis, and Retinal Telangiectasis. There is some evidence to suggest that Coats' Disease is caused by a somatic mutation of the NDP gene. (Norrie Disease Protein). You can search this link ( http://www.sph.uth.tmc.edu/Retnet/disease.htm ) for "NDP", to find the gene loaction.  Coats’ disease is a very rare  condition where there is abnormal development in the blood vessels behind the retina. The blood rich retinal capillaries break open, leaking the serum portion of the blood into the back of eye. The leakage causes the retina to swell, and can cause partial or complete detachment of the retina. Coats' disease is seen predominantly in males, about 69 percent of the cases. It progresses gradually and affects central vision. It is almost always unilateral (affects only one eye). If caught early, some level of vision can typically be restored. If not caught until it's late stages, complete loss of vision can occur. In it's final stages, enucleation (removal of the affected eye) is a potential outcome.

For an excellent resource, packed with links to research papers (some pretty technical) , go here:

http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=300216

The 5 stages of Coats' Disease

As you read through the stages, it may help if you first open this link in another browser window. You can keep it minimized, and pull it up when you want to see a particular part of the eye.

http://www.stlukeseye.com/anatomy/AngleStructures.asp

 

  • Stage 1 (telangiectasia only):
 
 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 (telangiectasia and exudate):
 

 This stage is typically treated with either Laser Photocoagulation or Cryotherapy, depending on the extent of the leakage, and the preferences of the doctor. If the exudate is limited to one quadrant, a good percentage of vision may be restored. This stage is divided into stages 2a and 2b:

 

          In stage 2a, the chances of restoring some level of vision are pretty good, because the fovea is not yet involved. (The fovea is the center most part of the macula. This tiny area is responsible for our central, sharpest vision. A healthy fovea is critical for reading, watching television, driving, and other activities that require the ability to see detail.) 

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

  • Stage 3 (subretinal or retinal detachment).This stage is also divided into stages 3a and 3b.

 

 In stage 3 a, 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 (Total retinal detachment and glaucoma):

There is most likely no chance for recovery of vision at this stage. In cases diagnosed at this stage, the damage to the retina is severe. A large percentage of cases that are diagnosed at this stage result in enucleation (removal of the eye), due to extreme pain. Treatment with laser therapy would only be an attempt to stop the leakage, and possibly prevent the increase in eye pressure that can lead to enucleation.

 

  • Stage 5:

At this stage, total blindness is present and irreversible. If there is no pain, aggressive treatment is not required. You and your opthalmologist should discuss and agree on what treatment (if any) should be attempted.

 

 

Prognosis for visual recovery

 

As you will all soon find out, Drs. Carol and Jerry Shields are known around the world for their tireless work in the management and treatment of Coats' disease. A large percentage of the children diagnosed with Coats' disease are sent to the Wills Eye Hospital in Philadelphia, PA, to be seen by Drs. Shields. Much of the information on this page was taken from the 2000 Proctor Lecture on the Management and Classification of Coats' Disease. We thank Dr. Shields for allowing us to post some of their data here.  We thank them as well, for the care they provided for our son Jacob.  

 

Drs. Shields led a team that followed 117 patients (124 eyes) for a mean of 55 months. In this subset of Coats' patients, the following visual outcome was observed:

 

 

Poor visual outcome (20/200 or worse) was found in 0% of eyes with stage 1, 53% with stage 2, 74% with stage 3, and 100% of stages 4 and 5 Coats disease. Enucleation was ultimately necessary in 0% of stages 1 and 2, 7% of stage 3, 78% of stage 4, and 100% of stage 5 disease.

 

The 2000 Proctor Lecture can be found here:

 

http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&list_uids=11336931&cmd=Retrieve&indexed=google

 

 

What treatments are available?
 

The two most common treatments for Coats' Disease are Laser Photocoagulation and Cryotherapy. The treatment chosen depends on the stage at which the disease was diagnosed. There are a few new treatments in various stages of clinical trials, but are years away from becoming available. Some doctors are experimenting 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 Coats' 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 it causes.  

 

The National Eye Institute website now provides a link to our website when you do a search for Coats' disease. We hope that this will allow more parents the opportunity to talk to the loving parents that regularly post here

 

. Our personal dedication:

Vicki and I are not physicians, we are proud parents of Jake and his sister Emily. The content of this website is mostly our understanding of Coats' disease, and should not be taken as medical advice. You should always, always see your pediatrician or opthalmologist for matters relating to your child's vision.  We started this website with the hope that it would provide information, and mostly support, for families walking down this road with us. We welcome your feedback, and we hope you will share your story with us and with the other families that post their stories here. We have some amazing, supportive parents that post here, and we can't thank them enough for their participation and their love. On the Google Map page, you can see where some of them live. If you have suggestions or comments , please feel free to let us know by using the "Feedback" page. We look forward to hearing from you.

We never cease to be amazed the the strength, courage and spirit we have seen in our children as we learn about Coats' disease, and we dedicate this site to them. They really are our little heros.