My wife gave birth to our third child on 9th March 2009. At day 5 we noticed his right eye had a wider pupil, was brown (when his left eye was blue), and the eye was red behind the pupil (like red-eye in photographs but it was red all the time). Our midwife, health-visitor, GP and an optician had all had a look by day 11, and all said that it was probably nothing to worry about, but we were referred to our local hospital in Aberdeen, Scotland. We went to the hospital on day 24, and he was diagnosed with Coats or Retinoblastoma. The following day we were sent to Birmingham specialist retinoblastoma unit where they discounted retinoblastoma, but confirmed Coats disease, which had by now already caused complete retinal detachment, and all sight loss in that eye. We showed the specialist our photos (from day 5 onwards) and from these he concluded that the Coats was very rapid indeed. He also said that Coats was very rare in a newborn baby. Unusually, whatever is going on has also affected his iris, in that it has shrunk, and exhibits ectropion uveae (where the iris edge at the pupil has turned inside out to reveal the black back of the iris which now shows as a black ring around the pupil - the pupil itself is now very large indeed. The iris is brown as a result of vascular abnormalities, according to the specialist). Is anyone out there aware of, or have any information about Coats in new-borns or with this 'dual' effect of retinal detachment and affected iris.
We do have retinal specialists in Aberdeen, and between them and the Birmingham specialists, we will start to discuss what might be done for our boy Campbell. We know he will never regain sight in his Coats (left) eye, so our efforts will be directed towards stabilising his eye such that he might be able to keep it. Note he is only 2 months old as I write this). There has been some talk of treatment / surgery to try and get the retina back in it’s correct position, but the very young age of Campbell make this ‘unchartered’ territory. Does anyone out there have any knowledge or advice that might be useful to us.
If it turns out that the best chances for Campbell are for him to be treated in the US, then we would be prepared to come over.
Any help / knowledge / advice appreciated. This has all come as a big shock to us, and we’re just desperate for information, as even the specialists over here don’t seem to have the answers.
My child was also diagnose recently and he is just 7. He just had his surgery and lucentis given. He is now recuperating well but he may need more treatment. Both my husband and I was glad that he was cared for by the most prestigious and professional team of doctors. He had his treatment done at Singapore National Eye Centre. They even have patients from as far as Middle East. My whole family is still reeling from shock at the discovery of coat's that was so foreign to us. But we have to be strong for our baby and so should you guys. NEVER GIVE UP. My prayers for your baby and your family. I can be contacted at sitilyas@hotmail.com.
It's not good and he is due to have his eye removed on Wednesday (today is Monday). The doctors are pretty sure it's Coats but cannot rule out Retinoblastoma. They say that even if it wasn't RB, eye removal is still best from a cosmetic point of view as his eye is shrinking and if left in place his skull wont develop properly (he's only 2 months old). There is no way his retina can be re-attached.
Very sad, but at least we'll be able to move forward after this and not always be worrying about the next complication waiting around the corner.
I will post more after the enucleation, as I hope I may be able to help other parents who are faced with this outcome.
Duncan and Tracy, I am sure that this was a difficult and painful decision for you. I hope that you can understand that this is probably best for Campbell. It is still difficult to grasp the concept that a child so young and innocent should have to endure this. Our thoughts and prayers are with you and with Campbell as his treatment continues. We would love to hear from you in the future, as Campbell recovers from the enucleation. Your willingness to share your story will help many other parents as they cope with similar situations. May God Bless you and watch over Campbell as he grows. We will certainly be thinking of you.
Duncan and Tracy, Have a newly diagnosed child with Coats disease I can somewhat sympathize but I am so sorry that this has happened to your new baby. My thoughts and prayers are with you as you go through this difficult time. Kathleen
An update on Campbell - sorry it's a long post but I had a lot to write!
His eye was enucleated last week and even though he is only two and a half months old, he seems none the worse for his surgery. For our consultant/surgeon, Campbell was the youngest patient he had enucleated, but he managed to fit an 18mm implant in which he was very pleased with (they try and get the largest implant in they possibly can). He is wearing a temporary shell (with an eye painted on it) while the swelling goes down, and should receive a proper prosthetic eye in about 6 weeks. He will need new prosthetics every few months as his face grows, and they may need to increase the volume of the implant in a few years by packing extra material around the 18mm implant.
Campbell was treated at Birmingham Children’s Hospital in England which is one of the two principal centres of excellence in the UK for retinoblastoma (and Coats). We had the best care and had every confidence in the knowledge of the consultants of what is a very rare disease. Both our consultant in Birmingham and out VR consultant in Aberdeen, Scotland, sought opinion on Campbell’s condition from around the world, including Will’s eye hospital and Toronto Sick Kids Hospital and in London. It seemed an unusual case because he was so young and the disease progressed so rapidly.
Does anyone know what are the chances of developing Coat’s are (e.g. 1 in ???)?
We have been told that his face/skull/eye socket will continue to grow normally, and that he should end up with little or no facial asymmetry. I have read things on the internet that possibly contradict this, so I would be really grateful to hear from anyone who has personal experience of enucleation at a very young age (less than 3 years old).
Campbell’s eye will now be tested to provide a confirmed diagnosis, and will hopefully rule out retinoblastoma (which the opthalmologists could not confidently rule out before).
For us, once we knew all the facts, enucleation was not such a hard decision. His eye was blind with a completely detached retina. The retina couldn’t be re-attached as neo-vascularisation and fibrosis was extensive, making the retina inflexible. We were worried about the vascularisation and fibrosis leading to secondary glaucoma, but in fact the opposite happened, and the pressure in his eye dropped leading to phthisis (a soft and shrinking eye). We were told that an attached retina is a requirement for normal eye growth in a young baby and, coupled with the phthisis, his eye/socket/face would simply not grow properly. Their conclusion was that eye removal would give the best cosmetic result. They also said that even if they knew 100% that Campbell did not have retinoblastoma they would still recommend taking the eye out.
Although eye removal seems (and indeed is) a drastic step to take, I can tell you that my wife and I feel great relief now that it has been done.
I’ve learned a lot about Coat’s disease over the past two months, and concluded that in many ways there is no ‘normal’ case. Campbell’s case was extremely rapid – from what we thought was a minor eye problem to eye removal in 2 months. That’s quite shocking and very stressful for all involved. Campbell’s iris was also badly affected, and even to the end we could see changes happening on a daily basis.
But this is the important bit – we, as parents, feel great relief now. Enucleation is not the end of the world. We feel that we can now move forward with Campbell, and start to get him ‘fixed’. We are no longer worrying about what the next complication is waiting around the corner. I think there are nearly 50-60,000 people in the UK with a false eye (on a proportional basis that means there could be about a quarter of a million in the US). Lots of people know other people with false eyes and they all say “you can’t tell that they are false”. I’m not saying enucleation is always the right decision – it clearly is not. But, if an eye is permanently blind, and causing all sorts of problems, it probably is the right decision.
If anyone wants to email me direct, then I give permission for the webmaster to pass on my email address.
I will post further updates as Campbell’s treatments continue. I hope this information may be helpful.
God Bless you and your family. Your strength is admiring. This story was amazing to me and I will think of it if Joshua's coats progressess rapidly. He is having his third cryo surgery on June 8th. He is 2yrs. old and more brave than most children. Again, God bless you for your strength, I can only imagine what a tough decision that was for you and your wife.
Thanks so much for sharing your story. I too have a son with Coat's disease. It was picked up on routine eye exam when he was 5. He is now 7.5 and is having is 4th surgery tomorrow. We have been told or read, I can't keep straight what I have heard or read lately but I believe the prevalence is 1 in 200,000, I am not sure if that is worldwide or in the states. We felt very lucky when he was diagnosed that it was picked up prior to any vision loss. We thought that would mean that we could prevent vision loss, however he has recently begun to loose vision. We are just trying to preserve as much vision as possible. It seems that every procedure has just as many risks as benefits. I hope this answers your question.
One in 200,000 was about the same figure that I deduced for myself in the UK. A rare disease is classed (I think) as 1 in 2000 or less so it is clear that Coats is not just rare, but very very rare.
Do you know why your son's vision has now started to deteriorate? What surgery is he having? And good luck to you and him for today (11th June).
If you paste the link below into your browser, and search for Coats' Disease, the estimated rate of incidence is 2 per 100,000 children. I saw a lot of papers that simply said that the rate was not understood.
Jassi, When you see the number of parents that post here, telling us that their child was just diagnosed, it seems as though the rate is much higher than "the experts" think it is.
Both the orpha.net info and jassi's info tally up. Also I've been told that in the UK there are about 15 cases every year. The birthrate is about 770,000/year and 770,000/15 = 51,000. Or to look at it another way, population of UK = 60,000,000. One in 50,000 means there are about 1200 UK cases. Assuming an average lifespan of 80 years, that's 15 cases per annum. So the numbers all seem to make sense at a Coats rate of 1:50,000. These numbers also tie-in with retinoblastoma - RB is about 2.7 times more common than Coats or about 1:19,000 (which tally's with the UK stats of about 40 new RB cases per annum).
The US population is about 305,000,000 so at 1:50,000 there must be 6100 cases there, or about 75 new cases each year.
I know none of this is an exact science, but the 1:50,000 does seem to be in about the right ball-park.
So all of us and our kids are in some respects pretty unlucky to have Coats. Having said that, although Coats is serious, there are many conditions that are far more serious and far more common. When my son was being treated in Birmingham Children's Hospital, vitually every child on the ward had something worse than he did. My heart goes out to all those children and parents, but also made my wife and myself realise that we were maybe the lucky ones!
He had another lasr procedure last Thursday. They said it went well but truly only time will tell. His vision loss is due to the build up of fluid in the fovea and under the retina, as I understand it. Apparently new leakages occurred very close to the fovea. I don't know if anyone else has heard this but it was recommended that we have our son tested for fascioscapular humeral muscular dystrophy(FSH). As I understand stand it 1% of children with fascioscapular humeral muscular dystrophy have Coat's disease but there are no statistics out there, that I can find that says how many children with Coats' disease have FSH. Too complicate matters our gentic test came back inconclusive, which at the time of doing the test we didn't even realize that was an option, we just thought it would be positive or negative, so now we are not even sure what to do with the information. Was this type of testing recommended to anyone else?
JMom, I really hope your son gets some positive results from your latest surgery - I'll be keeping my fingers crossed for him.
I have found a little information on fascioscapular humeral muscular dystrophy(FSH). If you paste the following link into your browser address bar, you can see it:
It states the estimated prevalence is 1:20,000. If you say that 1% of FSH sufferers have Coats that means 1:2 million have both FSH and Coats. Working back the other way from the Coats prevalence rate of 1:50,000, then in means that 1:40 of Coats sufferers will also have FSH, or 2.5%.
No one has ever told us about FSH, all I know about it is what is written in the attached link, but I would be interested to know if anyone out there has further knowledge, especially as to how it may relate to Coats.
I read about Campbell. Tears dropped from my eyes imagining the little baby who has to endure all this. But I really appreciate you for being the strong parents.
I am the mother of 3 years boy who is living with coat's in his right eye. I always used to feel guilty that I was not fast enough to notice that my son had a problem in his eye. I always felt there would have been the chance of restoring his vision if it was diagnosed earlier. But hearing about Campbell, it tought me that sometimes the earliest diagnosis also can't help.
Keep on posting the update on Campbell. My prayers are with Campell. Hope the little boy will not have to face furher complications in his life.
I thought I would post another update on how my son, Campbell, is doing. He's 4 months old now, and had his Coats eye removed back in May when he was just 2.5 months old (see previous posts).
Well, he received his first prosthetic eye last week, and he already looks a lot better (than when he was wearing a temporary shell post operation). As his face is growing rapidly we have been told that he will need new protheses every few months initially.
He will also have his non-Coats eye checked under anaesthetic every few months. Even though this eye is perfectly healthy and would be expected to stay that way, the examinations are a precaution and intended to catch anything untoward happening as early as possible.
It's weird, but having learned so much about Coats disease over the past few months, and have it totally consume our lives, we reached the end of the line very quickly, and his eye had to be removed. So you could say that Campbell doesn't have Coats anymore, just an artificial eye. Having said that, it was Coats that was the root cause and we will always have an unwanted association with this nasty and random disease.
We will move on now, but I will always be thinking of all the parents and children on this site, and hoping and praying that things all work out for the better for all of you. I'm sure I will still check the message boards for years to come, such is the connection I now have with Coats, and I'll still post the odd update about Campbell.
This site is a fantastic resource and I would like to thank Jacob's parents for the time and effort they have put into it. It really is appreciated.
Thanks also to all the people who have posted supportive messages to us. It's so nice to know that complete strangers care so much.
I now need to find a support group that can help us with advice and knowledge with respect to eye removal in infants (because Campbell's face still has a huge amount of growing to do, there could be facial growth complications, and that's what I need to learn more about now).
Many thanks to everyone, and good luck to all of you.
Duncan, It is great to hear that Campbell is doing as well as can be expected. When we look at how you and Tracy have handled the situation, it is easy to see where Campbell gets his strength. We wish you the best in the future, and your contributions to our site will not be forgotten. Please do check in once in a while, we really appreciate hearing from you. Please give Campbell a kiss for us all.
As Dave said, keep in touch on the progress of Campbell.You all have gone thru a lot in the last few months. I rememeber it is not easy, reading about all this and not knowing what is next. But we are all getting strength from each other.
