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

Sunday, July 31, 2011

We've had the prosthetic for four days

And I can't believe how easily we can get it in and out. I am really pleased. We had his first fitting on Thursday at 3, left the eye in over night, went back to Geelen on Friday at 9, took it out and made adjustments then put it back in. By the afternoon of Friday though, his eye was very red so we took it out (with a little sucker thing that attaches to the eye and makes taking it out quite simple) and left it out all day Saturday. His eye was very red and swollen shut on Saturday and he didn't sleep very well at all. I was expecting irritation so I wasn't too concerned, after all if I had a large acrylic eye in my socket I wouldn't expect my body to accept it straight away. We put the eye back in on Sunday and he wore it all day yesterday with no problems. Took it out last night, he slept well, then back in this morning and he's been fine.

I am really really pleased with how it is going so far. I am so relieved that putting it in and out isn't half as difficult as the RGP contact lens. The edges of the prosthetic are very thick, so there is more to hold on to when inserting it. With the contact, it would flip out of my fingers because of the thin edges and it took us a good three months to get used to putting it in and out, by which stage Joel had gone totally blind in that eye and it was no longer useful.

This morning I said to him "come and put your eye in Joel" and he pointed to his eye then ran off laughing and played peek a boo for a while around the door. We let him have a little bit of fun before doing it, then Rich picked him up and sat with him on his lap on the lounge. As I came towards him with the eye, he started crying and screwed his face up and thrashing around. It's hard to have to restrain him to put it in and I feel really guilty for being the one to cause him discomfort when I put the eye in. Rich held his forehead and his hands and I lifted up the top lid, slid the eye in, pulled the bottom lid down and it falls into place. All up, putting the eye in takes about 8 seconds but it feels like longer when my little baby is crying. As soon as it's in we give him cuddles, show him toys and distract him and he stops crying pretty much straight away. He will rub the eye for a minute or so but after that he just carries on as usual.

Once we knew we could do the eye quite quickly, we asked Annalise if she wanted to watch. She was facinated but once we took it out she said "Oh no, mummy! Now Joel can't see!" So innocent. We asked her if she wanted to hold the eye and she sat down on the mat (no way was I letting her hold it on the hard floor! If we had to pay to replace it, Joel wouldn't be getting one for a very very long time!). She turned it over in her hands, put it on the mat, showed her Barbies and ponies and asked if she could keep it in her room. I told her no, but that she could help me clean it and put it back into it's box until we needed it again so she helped to wash it with saline and dry it with the paper towel. When I was putting her to bed that night, she said she wanted and eye that she could "pick out". I told her she didn't need one because when she was born, there was nothing wrong with her eyes but she said "when I was a baby, I had an eye like Joel but I'm a big girl now so I don't need one". They know how to tug at your heartstrings, kids. She asked if the new baby will have to go to hospital and have a new eye and I said that I really hope not, I hope she has beautiful big eyes like her sister.

We keep the eye in a little plastic container with some paper towel on the bottom to stop it flipping over and rolling around. We also clean it with saline and use a conditioner from Bausch and Lomb before we put it in to Joel's eye. This helps to keep it lubricated and slippery. So far, it has been one of the easiest experiences we've had with Joel and for that I am so grateful.

Thursday, July 28, 2011

Joel has his first prosthetic eye

At 3:30 this afternoon, we went to see the Geelen's to get Joel's first painted shell. It was all pretty straight forward: Rich sat in the chair with Joel on his lap and held him while Paul put the eye in. In expert hands, it took about 10 seconds to go in. Joel rubbed it a lot, it was still a bit cold but once it warmed up he was fine. After he'd stopped rubbing it and I got a chance to look at his little face, I cried. He just looked so normal. I hate using that term, "normal", because hey, who is! But he just looked like he would have if none of this had have happened to him. There are still a few adjustments to be made to the eye; the pupil is quite large and the eye looks down and to the right. But, it is an amazing improvement to the blood stained natural eye that lays beneath the prosthetic. Once the few changes are made to it, I really doubt anyone will know there is anything different about him. The eye makes his eye appear so much larger, basically as large as his right eye.

I have uploaded a few pictures to the blog, it was really hard to get one of him looking straight on towards the camera. The eye does look a bit off centre and the colour is perhaps a tiny bit too green in some lights but we are just so happy to finally be at this stage. It felt like we would never get here with all the surgeries, then the bleeding and waiting for his eye to settle down to a point where a prosthetic was even an option. We have to go back tomorrow at 9am and Paul will take the eye out and make the adjustments, unfortunatley Rich can't come to this one so I'm on my ownbut judging by the amazing way Joel handled it today, and the care that Paul and Jenny have for their smallest clients, I think we'll be fine. He has gone to sleep with his eye in tonight and hardly seemed to even notice it while we were at home. He was playing and wrestling with Annalise like he usually does. Nothing stops him!

Oh and we found out that the chair that Rich loved so much in the Geelen's office is 96 years old! There's a pic of joel and Rich sitting in it to the right --->

Thursday, July 21, 2011

First fitting for painted shell

Yesterday we had our first fitting of Joel's painted shell with Paul Geelen from Artificial Eye Services. It went SO well, so much better than I expected and a lot less stressful on myself, Rich and Joel than I was thinking it would be.

When we arrived, Paul had an unpainted, white shell for us to try in Joel's eye. It looks like a very small ping pong ball, sliced in half horizontally. Before Paul had even put it in Joel's eye he said that he could tell it was going to be too bulky, so he disappeared for about 5 minutes to do some shaping of the eye, then came back and had me sit in an old fashioned style chair that Rich LOVED. It was brown leather and looked like it came from a 1950's doctor's surgery. I sat with Joel on my lap and Paul very calmly tried to insert the eye. Joel was quite comfortable with the whole process but the eye was still a bit too bulky at the top so it wouldn't go in properly. Paul took it away again and made some changes and we had a chance to see other artifical painted eyes and they looked so real. There was a pile of them on a bench next to some paints and they looked like all these little eyes staring back, some with blood vessels to make them look even more natural. Paul bought Joel's eye back and again we tried to put it in without restraining him but he'd clued on by this stage and resisted, so Rich held him as it was hard to hold him against my belly without him kicking and hurting me. The eye went in and Joel rubbed and rubbed it for ages. We just distracted him with all the toys and things in the office and he calmed down realy quickly. Paul said it was very cold when it first goes in and takes a while to warm up. The eye was white acrylic with green lines drawn on so that Paul could see where the centre of the eye is, which would enable him to paint the pupil on in the right spot. A few photos were taken of Joel with the eye in and some more of his non affected eye so that his prosthetic will look as natural as possible. Paul was able to get a good idea of where the centre was so he took it out and will paint it this week. We go back next Thursday at 3:30 to try out Joel's first painted shell! We're really excited. Even though the white eye looked like something from a scary movie, his eye was bigger and I can just imagine how much this will benefit him in the long run. I am really excited for him and so happy that I perservered with PMH to get them to write the letter to Geelen so we could go ahead and get it made.

The actual proceudre of putting the eye in and out is very similar to how we used to put in his contact lense, except the eye is bigger and thicker. Paul has a little suction tool to take it out too, something we didn't get with the contact although I can tell that will make things a bit easier because taking out the lense was our biggest problem. The artificial eye goes up under the top eyelid then the bottom lid is gently pulled down and the eye slots into place. I am not expecting that we'll be experts at it for a long time but it is good to have had that experience with the contact so we know what to expect.

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

Monday, July 4, 2011

Getting ready for the EUA

Examination Under Anesthesia

It's tomorrow. Joel will need to fast from 7am until 1pm from solid food but he can have clear fluids until 11am. I think he'll be ok. it's his fourth general anesthetic but at least this time it's not invasive surgery.

Dr Lam will take the pressure of his eye and have a good look at it. The blood that's in there has dried to a yellow colour behind the cornea so his eye probably looks the worst it's ever looked from that perspective, but it's not swollen so it's as wide open as it's ever been which is good.

The Geelens will take an impression of his eye to make the mould for his prosthetic while he's under the general anesthetic. Hopefully we are home by late evening.

Please pray for it all to go well!