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, June 17, 2010

Not the news we were hoping for

We had our appointment at PMH today. It was for 1:15 and I am used to waiting a while before we are seen but this time we weren't called until just after 2. We had a new Senior Registrar check Joel out and she couldn't find a red reflex in his eye. She was looking for a while and Joel was getting a bit upset. We laid him down and she looked in and after a while she said there was a huge air bubble behind his contact lens. I had a look and told her that it's actually the lens she's looking at. The "bubble" is the raised rounded top of his lens because he has such a high prescription. She agreed but still said she couldn't see much because his eye is "too small" and his pupil wasn't dilated. So, we had to get the dilation drops put in. She also told us she would have to get the Senior Surgeon to look as she hadn't seen a PHPV eye before. I was happy with that because the Senior Surgeon is Dr Lam, the one who did Joel's op. We had the drops put in and the "drop lady" as everyone calls her couldn't believe how good Joel was with it, he didn't even flinch. My brave boy. The drops make his pupil go really big, almost as big as the coloured part of the eye. They take about half an hour to work so we sat down and Joel had a little sleep until the drop lady came back and lifted his eyelid to see how big the pupil was. He woke up and screamed, what a shocking way to be woken up.

Finally the pupil was dialted so we went in and Dr Lam had a look but it took ages. Lots of fussing around and shining lights. That's when I knew it wasn't good.

What has happened is a mesh of scarring has formed across the eye where his natural lens should be (he had his lens removed during his first op). Dr Lam said it is highly unlikely Joel has been seeing anything, even with the contact lens. I asked about him grabbing the teething ring the other day and he said he may have just caught some light through a hole in the mesh across his eye at the time I held the ring up to him. Dr Lam is pretty convinced he can't see. I am not. I believe he can, even if it is just that tiny little bit of light that is getting through one of the holes. I absolutely refuse to give up hope for him. They say he can't see, I say he can. I love him and live with him and see him every day and I KNOW my little baby boy, I know he will smile at me with his patch on even when I don't make a sound in front of him. He may not be able to see far or clearly or colours or whatever, but he can see something.

So what next. He needs to have another operation to remove this scarring that has built up over his eye. They are just calling the op "polishing" the eye. Apparently Dr Lam hardly ever sees this happen. Lucky us.

How am I feeling? Sad. Angry. Disappointed. Why us. Why Joel. Will it ever end.

I knew he would have to have another operation, just not this soon and not for this reason. I thought he would have it a year down the track and more of a follow up to his previous operation, not for something completely new. I really wasn't expecting this news today.

His eye is also inflamed so we are back on the drops. Dr Lam said it's probably a pointless exercise to put his lens in every day if he can't see. The pharmacist also told me that with the particular drops he has, they can damage the lens and at $1450 a lens, it's probably not advisable. We also have to take the lens out to put the drops in and leave it out for an hour afterwards. I am a bit stuck because I KNOW he can see something with that lens in. What if I leave it out for the whole time he's on these drops, are all the hours of patching we've done in the last few months a waste of time? I know that when I don't patch his eye it starts to turn out towards his ears or in towards his nose. It is really frustrating and I feel stuck between a rock and a hard place.

His operation will be in 2 - 3 weeks time, enough time to let the drops work as they can't operate while his eye is inflamed but it's soon enough to prevent too much more scarring from happening.

My thoughts now - will the scarring just come back. How many times can they go in and polish it off. What if it just keeps coming back until they can't operate any more and his eye just dies. So many more "what ifs".

On one hand I am really sad for him. He's not even 6 months old and he will have had 2 surgeries. Why did he have to be born with this in the first place. What crossed my mind today was "damn you PHPV". Just damn it. It's so unfair. It really will never end.

On the other hand I am grateful he could even have the surgery, I don't regret having the first surgery even though that is 100% what has caused this new condition to arise. I still think we are doing the best for him even though as I type I am crying and my heart is breaking for my little man.

We'll get there. It's not over til it's over.


  1. Oh Tracie, I'm so sorry. This is such a hard road you are both traveling. Much love and healing thoughts, Vic xx

  2. Oh Tracie, I am really feeling for you and how hard these decisions must be to make when they can't give you a definite "do this and this will happen, do that and that will happen". And I can totally understand how you can feel torn between feeling upset and angry that he has got this condition yet grateful that he is able to have the surgeries...its totally understandable. I know there is nothing I can say to change anything or make things any better but you are doing such an amazing job being his mummy and you DO know whats best for him. Trust your heart, gut feeling, motherly instincts and you will do the very best for him. Its going to be a long hard road and all you can do is deal with one thing at a time and know its ok to feel everything you are feeling. He WILL be ok no matter what happens... after all he has you for a mummy how could he not be!! Luv ya and am always here for you and thinking of you all! xxxxx

  3. Hang in there Tracie...thinking of you and you family and wishing for a positive outcome for litle joel xxx Big hugs missy!! Be blessed you have him here with you, and trust your instincts. You are a wonderful mother xx

  4. Oh Tracie I so feel for you!! And I just know what you are going through with this damn PHPV bloody thing!!! I was crying this morning with some friends over William's eye after seeing their normal eyed babies and then I came home and read your post and started crying again! I have to keep reminding myself that we are the lucky ones because we get to patch our little guys whereas the little girl I saw in hospital on Wed had her eye shut and it was like that for good so she has no chance to develop vision in the other eye. But anyway you keep telling yourself these things but its still damn hard! At least they can operate and remove the scarring and then you can get on with developing the vision again! I think the hardest thing is not knowing whats going to happen!! Hang in there and give him lots of hugs and kisses - my mum says "at least you have him - I would do anything to have my boys back and have this condition". (My mum lost my 2 brothers at ages almost 3 and 17).
    Cheers Kirstie

  5. Don't feel bad about any decisions that can be made, Tracie. Nothing is your fault, no fault can be laid. There are so many options available nowadays, so grasp them when you can and don't be afraid for his future if they don't turn out as planned. It will all be fine. I promise.

    My eye receives light..I can see shadow movement in it only when the good eye is closed, but when it's open nothing registers on that side because the good eye sends all the signals.

    Do what you need to do, but don't feel bad about it for him and don't beat yourselves up. Trust me. He won't know the challenging paths you will have travelled for him because whatever vision he has will be normal foor him. By age 2, I'd had 5 operations on my eye and still nothing came of it. And I truly don't remember mch until the ops I had after 5 years.

    My eye issues were and still are normal for me. It's amazing how resilient little bubbas can be.