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

Monday, May 10, 2010

4 months in...

I thought I'd start this blog to help me, other people, people with PHPV, parents of PHPV kids... everyone really. It's such a rare condition/defect that no one really understands what we go through every day. The emotions, the challenges, surgeries, contact lenses, patching...Sometimes it just feels like it's never going to end. And it probably won't. We (I) will do what we (I) can for Joel now but eventually it's all going to be his responsibility to manage his PHPV when he's old enough, I just hope that between now and then I do everything I can to make sure he has the best possible outcome with his vision.

PHPV (or PFVS) results when a blood vessel at the back of the eye isn't absorbed into the system like it should. This should happen before they are born. That vessel causes a scarring in the vitreous (white part) of the eye, essentially blocking all light from getting into the eye, which results in blindness. The scarring also restricts the eye from growing, so the PHPV eye can be saller than the non affected eye. This is called micropthalmia and it was my main reason for alot of my upset when we found out about Joel. All I could think of was the teasing at school, the not looking as "normal" as everyone else. I felt so bad that I was worried about the cosmetic look of him when really I should have been more upset that he was blind. He also had a cataract on his eye, that was how we first noticed something was wrong. We noticed that under lights (like down lights) his eye would glow, like a cats eye. A big shiny silver glowing eye. Like a mirror. Very scary to look down at your baby and see that. I did all sorts of little experiments of my own, like covering his good eye to see what would happen and shining a torch into his bad eye to see the reflection. When I covered his good eye he would freak out. I knew he couldn't see out of it even before we saw the PO (Pediatric Opthamologist). I just knew he was blind in that eye. I left it for 3 days before taking him to the hospital. I just wanted to have my perfect little baby boy for that little bit longer. He was 3 and a half weeks old when he was diagnosed. Those 3 and a half weeks were the best weeks of my life. I had my perfect baby boy, my beautiful daughter and my marriage was getting better all the time. Things were just perfect.

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