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, June 30, 2010

3 days til the op

And he's still not 100%. The Anethetist is due to ring me this afternoon to see how he's doing. All I'll be able to say is that his cough is worse but his nose isn't runny anymore.

I really hope they don't leave the decision up to me.
Persistent hyperplastic primary vitreous (PHPV), also known as persistent fetal vasculature, is a rare congenital developmental malformation of the eye, caused by the failure of regression of the primary vitreous. It is divided into anterior and posterior types and is characterized by the presence of a vascular membrane located behind the lens. The condition can be of an isolated type or can occur with other ocular disorders. Most cases of PHPV are sporadic, but it can be inherited as an autosomal dominant or recessive trait.

This is an excerpt from an abstract by Barkur Shastry in a study of Clinical and Experimental Opthamology. Over the next few days and weeks I will be doing a bit of research in regard to a link and will post anything interesting I find.

There has to be a link

I've just been reading another blog about a lady with a baby with PHPV. Her boy also has a cleft lip. My mum had a cleft lip and my brother had a cleft lip and palate. This isn't the first time I have seen the cleft lip/palate link on a PHPV board, blog or forum. There has be be more to it that just a coincidence.

If the chances of getting PHPV are "random" and 1 in a million and the chances of a cleft lip, alhough proven to be genetic, are also very small, how it is possible that there are so many kids born with both? Or if they don't have both, as in Joel's case, there is a family link somewhere down the line? I don't have a cleft lip or palate, neither does my other brother, but that gene is obviously in our makeup, there just has to be more to it than coincidence.

I wish there was more information. Not that it will help, I know that it's not going to change anything, my curious mind needs to know...

Tuesday, June 29, 2010

Still sick

This morning he's woken up with a cough too. I really don't think he's going to be well enough for the op.

I haven't been patching him for the last 3 days, he hasn't worn his lens either becuase of having the drops every few hours and taking it out and putting it back in. He is REALLY freaking out again while he's patched, so I am pretty sure he can see basically nothing again. Plus I was thinking that the surgery is only days away so those few days can't hurt but now....If his op isn't for another week, that could potentially be 2 weeks without the lens in :-( I feel terrible now, I wish I just pushed through it and put the lens in and kept patching. I don't even know how he'll go with the lens in today, his eyes are all red from this damn cold. I'm really frustrated.

Rich goes to Melbourne tomorrow for 4 days. He's back on Sunday. He's probably glad to be going so he doesn't have to listen to me going on about the op, lens, patching, drops, cold, coughs....

Monday, June 28, 2010

General Anesthetic and his cold

I just spoke to the Anethetist and he said to just watch Joel for the next 72 hours and see what happens. If it goes to his chest there's no way they'll put him under a GA, so I just hope it stays in his sinuses. Well, best hope is that it goes away totally.

I remember reading, in my research before his first GA, of a little baby who had complications following his surgery because he had a chesty cold 2 weeks before he was anethetised. It's pretty nerve racking stuff! I am really nervous for him. I really hope this cold goes away soon or else I am going to make myself even more nervous knowing there's that one extra risk.

I will just keep loading him up on breastmilk, that stuff is magic ;-)

Sunday, June 27, 2010

Joel has a cold

I don't know what this means for the surgery. I know the hospital warned us last time that if he had a cold they probably wouldn't put him under a GA. I really hope it clears up by Monday. I've been researching about GA's and respiratory issues and it looks like it is very dangerous for babies so if he is still sick or if the cold is lingering I wouldn't risk it.


Thursday, June 24, 2010

Green eyed monster

I have been debating whether or not to post this. I think there's a line between being honest and giving away too much of how I'm feeling but this just keeps coming up for me and I want to get it out.

I get really jealous, sad, angry and disappointed when I see a toddler with perfect eyes. All I can think is "Joel will never look like that". It kills me, it breaks my heart. I see this gorgeous little face looking at me and having this conversation with me, whether it's at the shops, park, playground - wherever - and I just get so sad that my little boy will never have these two perfect big round eyes. It just makes me so sad that I can't even look at another child without that thought in my head.

To me he is perfect. To me he is handsome and sweet and so loving and affectionate, so the feeling is contradictory. I want to not think "Joel will never look like that". I want to just appreciate the beauty in every child. I wish I could just talk to these other kids and think "wow, they're cute." But I can't. I hope over time it goes away.

Wednesday, June 23, 2010

Date for next surgery has been booked

It's Monday 5th July.

It's a good date because Rich goes to Melbourne for work from the 1st to the 4th of July, so he will be back just in time. The pre admissions lady will call over the next week to talk about how it will all go.

On the subject of surgery, I was so pleased the other day to have a win with our private insurance. The first surgery Joel had was supposed to be covered by our fund, MBF, but when we rang to confirm they said Joel wasn't covered for anything because I didn't call to add him to our policy within 8 weeks of him being born. I let it go at the time but after thinking about it for ages and having a friend say I should follow it up, I called them. It took about half an hour and lots of being put on hold while th lady spoke to her managers but they eventually came back and said that he will be covered because we have an exisiting family policy and there is no time frame to add another child to it. Joel's PHPV is considered pre-existing so they weren't covering anything to do with his eye, which meant for the next surgery we would have one of the students or Registrars doing it because PMH is a teaching hospital.

So between now and Monday week, I will just pray for my little man. God, heal him. Guide the surgeon's hand and be with our family through this next step of our journey. Give us wisdom and peace with the decisions we make for him and make his recovery as quick and painless as You can. We know that You are watching out for him and that You love him and we just pray that You will have your hand on Joel and heal his eye and make him see. Amen.

I haven't put his lens in today. It's getting really hard taking it out and putting it in all day. He is starting to get really angry when he knows it's coming and we were getting to a stage before where he was smiling when we were putting it in or out. It feels like I put the drops in, put the lens in, he sleeps, wakes up, feeds, lens out, drops in, sleep, wake up, lens in, feed, sleep, wake up, lens out, drops in... argh. It's too much for him. I will put the lens in when Rich gets home from picking Annalise up from kindy and we will patch him tonight when we have the time to distract and play with him.

Tuesday, June 22, 2010

A really great blog for parents

I found this today, after reading through 99% of it (have to take Annalise to kindy otherwise I would read the rest!) it's great. Very technical but still, a real eye opener - pardon the pun!


Lots of amazing photos too.


Friday, June 18, 2010

More praise

Today Joel was patched and he reached out and grabbed a big fluffy teddy. He smiled while he did it too. It wasn't directly in front of him, it was to the left of his head.

I am so sure that patching him while he has the drops is the right decision.

I spoke to his Optometrist today and told him that I am sure he can see something and he told me to keep patching.

I was thinking about it too - I know the lenses are expensive and yes we could wreck it with these drops, but we can always pay for another lens, we can't pay for another eye.

The doctors may think that the mesh of scarring stops him from seeing, but we know differently. All the prayer is being answered, I just know it.

Praise God.

When he's asleep

I go in and check on him. I sit there and just watch him, watch his little chest rise and fall, watch his lips form smiles and frowns and his beautiful little eyes squint and relax.

He has no idea now, and probably never will, of the weight I feel on me with some of the decisions I make for him . I am so blessed and so honoured to be his mum. He is such a special little man. God has a huge plan for his life, I just know it. We are so amazingly lucky to have him. He is just such a lovely, sweet, affectionate and gorgeous little boy. Thank you God for giving him to us.

Thursday, June 17, 2010

My Plan

After sleeping on it, I have decided that I still want Joel to wear his lens and be patched for 2 hours a day. This is what I can come up with:

7am - wake up and drops in
8am - sleep
9am - wake
9 to 11am - lens in and patch on
11am - lens out and drops in
11:30am - sleep
2pm - wake
3pm - drops
3:30pm - sleep
5pm - wake
5:30 - 7pm - lens in and patch if the morning doesn't go to plan
7pm - drops
7:30pm - sleep

Fingers crossed.

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.

Friday, June 4, 2010

Can't believe we forgot to take it out

Rich has just left for church and I can't believe this - we forgot to take Joel's lens out! I put him to bed, then as I was saying good bye to Rich I realised his lens was still in. Rich will be home around 10pm so this is one of the first nights I actually wish Joel would wake up. We can leave it in overnight of we really have to but there's no point, he's not looking around or anything plus it will irritate his eye and it needs to be in the conditioning solution for at least 4 hours between coming out and going in.

If he wakes up in the meantime I will have a go at getting it out on my own. For the first time. I am going to have to get used to it because Rich is going to Melbourne for 4 days in a few weeks and I'll be home on my own so it's probably good if I get some practice in now.

I have heard from PMH (the hospital). We have another appointment on the 17th June. Please pray for us that he hasn't developed glaucoma and everything is fine with his eye.

Thursday, June 3, 2010

Miss Utah talks about PHPV




So yesterday Joel was patched and grabbed at a teething ring from about 10 cm away.

So happy for him

Here is the video proof!