A blog about having a child with PHPV or PFVS

A blog about having a child with PHPV or PFVS

Persistent Hyperplastic Primary Vitreous

also known as

Persistent Fetal Vasculature Syndrome

and micropthalmia (small eye)

Our experiences with 3 surgeries, 2 EUA's, patching, contact lenses, scleral shells, prosthetic eyes, emotions, places to get support, links to other sites and general info on vision impairment. I really hope my blog helps and educates and I would love to hear from you with any questions you have, or even if you just need to talk to someone who has "been there, done that".


Perth, Western Australia

Thursday, September 30, 2010

Private appointment and latest with infection

I called Dr Lam's surgery yesterday to get in to see him privately so I don't have to to go through the public hospital system any more. The earliest appointment I could get is the 14th January 2011. That's the day after Joel's first birthday.

I feel like NOTHING is happening. No one has looked at his eye for a month, I have no direction for where it's all going. I don't know if we'll end up back using the lens, getting a conformer, a coloured contact. I just have no idea what the next step will be.

As for his eye, the infection is clearing up. We are still using drops and I'll just keep using them to make sure nothing comes back. I have also noticed his eye is starting to shake a bit. I'll be glad to go back and see someone at the hospital before I see Dr Lam privately, I know that's an appointment I'll be able to get.

Saturday, September 25, 2010

Bad eye day

So yesterday he didn't open his eye for the whole day, it was swollen and red. I put more Chlorsig in, every two hours (Chlorsig is used to treat conjunctivitis amongst other things. It used to be available on prescription only but it's now over the counter). This morning the eye looked a tiny bit better so I thought I would see how it was by the afternoon. We went to a birthday party and by the end of that it was looking pretty bad still so off we went to the hospital again. We were in and out fairly quickly. He has more Chlorsig drops plus Amoxil antibiotic. It is slightly infected. It looks like it's giving him some pain, he had Panadol and Nurofen yesterday. Pics to the right show what it looks like. Poor little guy.

Tuesday, September 21, 2010


I've been thinking about it and there is one thing I regret doing and wish that we hadn't have done. The third surgery. The one to drain the blood from the eye. When I look back at photos of him from a day or two before the surgery, the blood was beginning to drain on it's own accord anyway. I wish we never agreed to the doctors going in and draining it for him. The third surgery was the one that caused bleeding behind his eye and has stopped him from seeing. There are pics on the right side of the blog showing the hyphema (blood in the eyeball) the day before the surgery and you can see his pupil and it was small and round. After the third op it's shaped like the number 8 and it takes up most of his iris. If we didn't do that operation, maybe the blood would have gone away on it's own accord, maybe he'd have no blood at the back of his eye, maybe his pupil would be a normal shape.

Maybe he'd be able to see.

Monday, September 20, 2010

Annalise is asking questions

The last two days, Annalise has said things about her little brother's eye. Yesterday she pointed to him and said "his eye is all sore, what's wrong with his eye?" and then this morning in the bath she said "poor Joel, his eye is sore". She said "can he see?" and I told her that no, he can't just yet but if we keep asking Jesus, one day he will be able to see. What else do you say to that? Breaks my heart. His eye is gradually looking worse and worse. It's not helping that he's got conjunctivitis but the iris isn't going a green colour like I thought it would after we stopped using the drops. It's stayed a really dark, almost black colour.

What am I going to tell Joel when he starts asking about his own eye? I think about it alot, what questions he will ask, how will I answer them?

Thursday, September 16, 2010

Joel is sick

He has an ear infection, cold and the worst part - conjunctivitis. His good eye is all gunky and gross and his bad eye is much smaller and redder than it usually is. It's so unfair. I just feel so bad for the poor little guy, he can't catch a break.

He's slept a fair bit today but has been up most nights. I knew he was sick on Tuesday so I tried to get him into his usual doctor but couldn't, so I ended up paying $60 to see a private GP who gave him a quick check over and said there was nothing wrong. We were up from 11pm- 3am that night so I got him into his regualar doctor yesterday and sure enough, an ear infection. He's got antibiotics for that, then today I saw his gunky eye so back for more drops for his eyes. I really hope it clears up soon, it's horrible watching him not being able to see properly out of his good eye.

I've uploaded some pics of him this morning, before the conjunctivits really set it. He's got a little bruise on his forehead from playing with the "big kids".

Thursday, September 9, 2010

The latest from today's appointment

The white "stuff" in his eye that looks like spider web is scarring but it's not on the iris as I thought, it's actually on the cornea. That means it's not on the inside of his eye, it's on the outside. The most likely cause of it is the incision from the second surgery. This is pretty good news, considering. The registrar (again we didn't see Dr Lam, we saw a new registrar called Johnaton, he was really very good) said that more than likely it won't grow across his eye, and if anything it should fade over time. Woohoo. Good news again because this means that it won't grow across the pupil, further obscuring any vision he could possibly get from his eye.

His pressure is 25 which is ok. I asked again about a conformer or some way of dealing with his eye being small but was again told it's too early and that he's been through enough and in a year or so if his eye is "cosmetically unacceptable" they will do something about it.

He still has no red reflex so the blood is still at the back of his eye. KEEP PRAYING.

All in all, I am taking it as good news. His eye is no better, but it's no worse so that's great.

We don't need to go back for another 4 months but I am actually going to book in privately to see Dr Lam for our next appointment. I don't want to see Registrars any more.

I should add that we aren't using Atropt drops anymore either. It will be interesting to see what colour his eye goes. I haven't properly seen his iris for months because the drops kept the pupil so big. Speaking of the pupil, it still looks like the number 8, like two pupils.

Wednesday, September 8, 2010

Off to our appointment today

We have another appointment at the hospital today, the one I made to check out what the white mass is in his eye. His eye looked amazing yesterday, it looked bigger and the iris was green like his other eye. Usually the iris is dark brown or black. It was as if there was nothing wrong.

Thursday, September 2, 2010

So far,,,

I know there are a few new people reading the blog so to save you going back and reading all the posts looking for Joel's history, this is what's happened.

Diagnosed at 4 weeks.
Lensectomy (removal of the lens of the eye) and vitrectomy (removal of some of the white of the eye) at 2 months and 5 days old.

We patched and put a RGP contact lens in his eye for 3 months.

At almost 5 months old the surgeon found scar tissue in his eye (caused by the first op). Another operation to remove that scarring at 5 months old.

2 weeks later, his eye was full of blood (complication from the second surgery). Another operation at 5 months 2 weeks old to drain that blood.

2 weeks after third surgery we were told he has blood at the back of his eye now (most likely caused by the third operation). During the second operation, part of his iris was pulled off, causing quite a large bleed. There is no way to drain that blood, which is stopping any light from getting to the back of his eye, so he can't see.

Last week I noticed the scar tissue has come back in his eye. The surgeon will not operate on him anymore as it will affect his good eye. We have an appointment in a week to discuss what happens next.

I have been asked if we regret having surgery. Yes and no. Yes because it has had no effect on his eye, except to make it worse by giving him another problem to deal with (scar tissue and blood behind the eye) and no because his cataract looked quite bad before and at least that's gone. We did what we thought was best at the time. He did have some vision in that eye for 3 months so if it wasn't for the blood in his eye caused by the second surgery, he would still have that vision. But then, he may have lost that because of the scar tissue coming back a second time.

It may sound all doom and gloom but it never is, not with God. This scripture is one that Rich found and I'm believing it's true for my baby boy.

John 9:1

As he went along, he saw a man blind from birth. His disciples asked him, "Rabbi, who sinned, this man or his parents, that he was born blind?"

"Neither this man nor his parents sinned," said Jesus, "but this happened so that the work of God might be displayed in his life. As long as it is day, we must do the work of Him who sent me. Night is coming, when no one can work. While I am in the world, I am the light of the world."

Wednesday, September 1, 2010

White lines are changing

The lines now look more like a piece of spiders web. Like a little clump of it on the edge if his iris. I am fairly certain it's more scar tissue. Given he's not having any more operations, I guess it's just something that is going to have to stay there. It looks to be about 3mm long and about 3mm wide. My guess is the two lines have meshed together to form this mass. Now I wonder if it will just continue to form and grow across his whole eye.

Next appointment

I've got antoher appointment with Dr Lam next Thursday, 9th September. I can see the white lines in his eye, to me they are getting more noticeable. Rich can't see them, neither can my mum but I can. I know they're there I just want to know what they are. I also know there's no more operations for him so if it is something bad, we will just have to deal with it as best we can. If it's just a funny little thing going on with his eye then great. My feeling is that it's not something that's just going to go away. It looks like whatever it is, it's there to stay.

I was thinking that maybe it was something to do with where they pulled part of the iris away. It is hard to tell if it's on the cornea or not. Just wait and see. Again.