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

Wednesday, July 6, 2011

EUA and Impression for Prosthetic done

I woke Joel up at 6:30 yesterday morning to try to get some food into him before he had to fast at 7. He was not impressed about waking up early and refused to eat eggs, toast or cereal, he wouldn't even have a bottle of milk. I started getting Annalise's lunch box ready and Joel was very interested in the small strawberry squares that were going in there so I gave him some and he ate 3 of them at about 7:15 so I thought that will have to do him! Annalise woke up at about 7:30 and had her cereal so of course Joel wanted some of that too so I gave him his bath while she ate then got them both ready for the day. He kept following her around, wanting everything that she had - an apple, glass of milk, juice. He was allowed to have juice and water so I gave him that but then it just got really hard to stop him from taking her food so we drove around for an hour or so, then I dropped Annalise off at my friends for the day at about 9:30.

Joel usually sleeps between 10 and 1 so I was hoping he'd sleep in the car which he did - from one end of the freeway to the other. As soon as I turned off the freeway and headed back north again he woke up and screamed all the way to the hospital. This was about 11:30. We arrived at the hospital and what a different experience it was to PMH. If you have private insurance I would definitely recommend Subiaco Private Hospital. There were 3 other kids there, not 20 like there have been at PMH. 2 were getting grommets and 1 family didn't talk to anyone so I have no idea what they were there for.

We filled out the paperwork and sat down in the lobby and drew, played toys, looked out the window for a while. Jenny Geelen arrived, she is the Ocularist making Joel's prosthetic eye. We spoke about the procedure for making the mould and how long her part of the procedure would take (about 2 minutes!). She was so lovely and so good with Joel.

We met with the Anethetist and she explained how she would be using a different type of anesthetic this time. The other 3 operations, Joel has had the mask on his face and gone to sleep that way. This time he would need intravenous anesthetic as the gas can cause low blood pressure and give a false reading of low pressure in his eye. She looked at his little fat, chubby hands and said she might have some trouble finding a vein. I told her about how at our last operation, they couldn't find a vein to put his pain relief drip in and he came out of surgery with 2 holes in both hands and one in his foot and his hands were bruised for weeks. She said she was sure she could do a good job and that wouldn't happen.

At about 1:30 Joel's name was called so I put the gown and hat on and went into theatre with him. Dr Lam, Jenny, two nurses and Tanya the Anethetist were waiting for him in there. As soon as he saw them he started freaking out. I had to lay him on the bed with his head in the head rest and sit nice and close to him but he kept trying to flip off the table and sit on me so they had to hold his legs and arms down, with one hand on his belly. He was screaming, his face was red, he was crying so much. I was just saying "it's going to be all done soon, baby boy. A quick one this time." Kept telling him how brave he was and that he's my beautiful little baby boy and mummy's here but he just kept crying and looking at me. Absolutely broke my heart. It is so hard because I am doing all this so that he can have his false eye made and help his face to grow and get him used to having it in while he's still little but while they were trying to put the needle in his hand, all I could feel was guilt. I wondered why was I doing it, why am I getting him a prosthetic. Is it for me or for him or for both of us. Why does he have to go through this. Does he really need it. Should I just wait unti he's older and he asks for one.

They told me that with this type of anesthetic he will still appear to be awake for a while after it has kicked in and that's true. He was still looking at me with his one good eye and sobbing but they said he was actually asleep. He has the most beautiful colour eye I have ever seen. It's so green and beautiful. I just wish he had two. I wish it was me going through it all and not him. They told me I could leave him and I walked out and cried.

After about half an hour I could hear him in recovery. He is really loud. The nurse came out and got me and they took me over to his little bed and he was laying on his tummy with his bum in the air. I picked him up and he was all floppy and his head kept falling back and he was crying. They said "he won't recognise you yet" but I sang Twinkle Twinkle Little Star to him and he stopped crying so I think he knew it was his mummy. He laid on me, crying on and off for about 5 minutes, then we moved to the big chairs because my back was killing me with a 30 week pregnant belly and a 13 kilo toddler hanging off me! We sat down and had cuddles for ages and the nurses brought him his milk bottle. He couldn't quite get his mouth to close on it and suck but he tried for about 10 minutes and eventually got there and started to wake up. His eye looked red and teary but not bad, just like he'd been rubbing it. After another 10 minutes he really started to wake up and saw his lunch box and grunted and pointed until I'd opened it. He ate a packet of chips and some mandarine, had a big drink of water and juice and he was raring to go. He kept trying to get down off the chair but his legs weren't working yet, he wasn't going to let that stop him though. He tried to walk over to the machines and pushed me away when I tried to help him. I showed him some chocolate and he came back to the chair though, so funny. They took his canula out and Dr Lam came to tell us how it went.

His eye is doing really well. There is no glaucoma or any pressure problems, the yellow blood should eventually fade and the cornea will become clear again. We don't need to put any more drops in for the first time since November last year which is awesome. The moulding went well and they should have a clear shell for us in the next week. Dr Lam doesn't need to see us for another 6 months which is great.

All in all, it's a good outcome. The day itself was very tiring and very emotional and I am glad it's over. He has recovered from the general really well this time, I think because it was such a short one and not an invasive surgery. He did wake a few times last night for cuddles but that's not unusual, sleeping isn't really his thing, he's got so many other things he wants to be doing. He's a very busy little man and has so many adventures he wants to get up to, just like every other normal sighted little boy <3

Thank you everyone for your messages to see how he is going, he's definitely back to his normal destructive self today xx


  1. My heart hurts for you! I am glad it is over and he seems to be recovering well. I think that I would do the same thing so that his face would grow normally. He will appreciate when he is a teenager. But that doesn't make that day any easier. I hope you don't mind me following your story on your blog. Let me know if you would like me to stop... Hope Joel continues to feel better!

  2. Thanks so much for following and posting your comments! I really appreciate it and enjoy reading what's happening with you, it's great to be able to share others experiences with all of this. xx Tracie