Another Eye Appointment 27 July 2010

On Sunday I decided to use a new lens that we have spare as the one I have been using for 2 months now seems to be a bit dirty and I've been trying and trying to clean it without much success. When William was patched with the new lens in he got so excited and went absolutely nuts under the play gym! But I'm not sure if that's because his new lens is super sparkly clean or if it was because he had had a great sleep?? Anyway we went to our eye appointment today and had to tell them that William has decided to be dominant in his "bad" eye again and we're not sure if its because of the new lens. So we are back to no patching until he becomes dominant in his good eye and then we're down to only 2 hrs per day of patching. Its great news but once again the PO said we HAVE to get him to be dominant in his good eye again. They also noticed that he can alternate which eye to use which is a positive. The only thing that is a real bummer is that William has got an eye turn which may or may not have happened if he hadn't had PHPV so his chances of binocular vision are pretty much zilch. Anyway I'm not so worried about that now. The PO also said we may consider having eye alignment surgery before he turns 1 to help with peripheral vision but he said to not hold out any hope for Binocular vision. He did say though it would be quite a challenge as William has a Micro eye! I asked Dr Maslin how many cataracts he sees in Wellington and he said sometimes we have 2 a year (which they've got now with William and James) and then they may not see any for 3 years. So being from a small city there's not much chance to get really familiar with pediatric cataracts and certainly not PHPV. I have joined a Yahoo Health Group called PHPV and its great to be on this site as I have learnt a lot from other parents and am able to go into my appointments with information that I have read from this site. I've really had to research PHPV myself to understand exactly what William has - though he is a tricky one as he didn't have a stalk which is associated with PHPV, he only had a damaged lens and a hazy vitreous.

When I think back 13 weeks ago when we were told he was virtually blind in his right eye and not expected to have any vision at all to him now being dominant in that eye (even though that's not great)I feel very blessed!

Monocular Vision

I'm feeling a lot better about William's eye now. I've caught up on sleep and have been reading a few people's experiences on living with Monocular Vision http://en.wikipedia.org/wiki/Monocular_vision.

It's not as bad as I think and many people have monocular vision, its amazing the amount of people I have told about William's condition and they have gone on to say that they are blind in one eye or they know somebody who is. One lady in our Antenatal group was born blind in one eye (her mother had Toxoplasmosis during pregnancy), they never knew until she was 5 and then they began patching her. She is a beautiful lady, drives a car and lives a normal life, I never knew she was blind in her eye until we talked about William! People who have never had Binocular Vision don't even know what they are missing. I'm still a little sad because I know William won't have eye sight like me, but then how many people have eye sight like me anyway - I may not have the greatest sight either. My husband went through a lot of school thinking the blackboard looked fuzzy but just dismissing it because that is what he thought everyone else saw. It wasn't until his last few years at school he got glasses and was amazed at what he could then see! Its vital that every parent gets their children's eyes checked so they don't go through school missing out on an education!

In one respect I'm pleased that we will be getting William's eyes checked all the time because at least we'll be able to monitor his vision and act on something if we need to! In saying that I should really get Sophie's eyes checked as I just presume she has good vision. Lexie has ok vision as that has been tested in the B4 School Check they do here in New Zealand. All in all William won't really be disadvantaged at all and I'm sure he'll be able to achieve all the things he wants to in life. It makes me a little bit happier knowing that. But for now we'll get on with patching, eye appointments and having a fun time with our beautiful little boy who we love to bits!!!

Eye Alignment

Its really bugging me that William's eyes are not aligned when the patch is off. It seems to be getting worse. When he had surgery and a few weeks after his eye's were actually aligned ok but now the weeks are moving on they seem to be worse. I'm not sure if they got worse when we upped the patching time, but in any case its annoying me. I knew there wouldn't be much chance of his eyes working together but its glaringly obvious that they won't now and I think that's what is upsetting me the most. God why did William get this rare condition!!! I think its one of those days. I'm not always down about it but some days it just pisses me off that he had to get this and I can't just take my baby out without a patch on! Everyone at Kindy asks how his eye is and how am I suppose to respond?? There's not much to say apart from "we believe he can see out of it, but in any case the eyes won't work together and we'll be patching for 8 - 10 years". Grrr, I'm sure I'll feel better tomorrow after a good sleep. Actually I think sleep deprivation is the answer, last night Lexie was scared about the monkey in Toy Story 3 and didn't get back to sleep until after 12 am and the night before Sophie was up about 3 times! and William was waking up 2 times a night, so after a good sleep I'm sure I'll feel a bit better.

Bad Reaction

I've never had bad skin in my life until now! All I can think is that it must be related to all the stress I've been under since William was diagnosed with PHPV. I have been to the Dr and he also thinks its from stress. I'm finding it quite embarrassing to go out in public, my lips look like Botox gone wrong and I've got eczema all over my neck, eyelids and face! Crikey I hope it goes away before we go to Queenstown for our skiing holiday, I'm hoping Jason and I can go out on our own without the kids for a few dinners and drinks but don't want my skin to look all angry and hideous! Not a sexy look. And my lips are driving me nuts I want to peel them off!

Sophie, William and I took off to the Mall today for a little shopping while Lexie went to the movies with a friend. The weather has been so bad these school holidays so we're not getting outside much and we've got the central heating going all day, so I guess that's affecting my skin also. As we were having a treat at the mall I was looking down at William and wanted to pick him up to show the world my beautiful baby boy but then didn't want the stares that I thought may go with it. And as I looked at some other kids in the Mall I got all sad and thought "Why did my boy have to have this defect and what did I do wrong in my pregnancy?" Dr Small did say right at the beginning when we found out that it wouldn't have been anything I did its just unfortunately a freak of nature - but why William?? Anyway I didn't dwell on it long as Sophie was pinching all the icing off my muffin and I hadn't eaten anything yet. I think the good thing about all this is that William probably won't be that much affected by this whole condition as much as me. I think it hurts mothers more than anything when there is something wrong with our children as we have this overwhelming feeling of love in us and we'll do anything protect our children!

Good Eye Becoming more Dominant - Wed 7 July

William's good eye is starting to become more dominate again which is a relief. So we are now back to patching but only for an hour a day.

I have been reading another lady's blog about her son who had to have scar tissue removed this week and its just so horrible. I have also just been called by a mother who I met at Wellington Hospital whose son had a cataract removed when he was 8 weeks old and they have just found out today they have to go back to hospital next week to have scar tissue removed from his eye. I hope William doesn't get scar tissue but unfortunately its something that could definitely happen. We have another appointment on the 28th July so I will ask Dr Maslin again if he can check for scar tissue because last week when I asked Dr Maslin said don't worry about it, but now that its happened to two people I know I want to make sure that they check. We are going skiing the week following our appointment so I'm praying he doesn't have scar tissue cause if he does that means we'd have to stay here and have another operation!

Wednesday 30th June

We had a meeting with the PO today and he is quite concerned that William's operated eye is becoming dominant. It was decided that we would not patch him to encourage his good eye to become dominant again. If that doesn’t work then we might have to patch his operated eye to force the good eye to become dominant. I don’t think our PO has seen a case like this before! I guess it is good in one regard because William can obviously see out of the operated eye but we don’t want him to become dominant in that eye because William's good eye is far superior to a eye with a imitation lens! We also talked about eye alignment surgery which Dr Maslin doesn't want to do until William is about 18 months old. I hate the thought of William going for another surgery but it's something that we'll definitely have to consider. The surgery would be for cosmetic purposes only.

Wednesday 16th June

Getting the lens out and in seems to be getting easier every week, I just hope it stays this way as when William is happy and not crying his eye doesn't clamp shut so getting the lens in is far easier than when he starts getting agitated and cries.

Today we went to see the PO again and they have told us to up the patching to 3 hrs a day. Dr Maslin also said something about William having to wear bi focals when he is older - I'm just realising that there will always be something new we will find out at every appointment which can be a surprise and sometimes those surprises aren't so nice to hear. In the back of my mind I'm always thinking about Glaucoma, scar tissue and other complications that could arise and the only way we ever find out is from going to our eye appointments.

We started patching 3 hrs a day and then noticed when William was unpatched he still favoured his PHPV eye and his "good eye” (left one) didn’t move very much. I did some research and I thought he had Reverse Amblyopia – I called the PO the next day and they told us to come in on 30th June to check his eyes again. I also spoke to the Orthoptist and she explained a few more things about how eyes work and how William's eyes will probably work. I asked whether William had any chance of gaining Binocular Vision and she said that would be the Ultimate Goal and what we would be working towards, however its probably very unlikely. When I heard that I felt quite down again, I'd pretty much come to terms with William having this condition and for some reason I thought with surgery, patching for years, going to our eye appointments, etc William would gain pretty much the same sight as any other person, but I guess this isn't the case. I have read a little about Binocular Vision and babies develop this by about 4 months of age. It is very rare for young babies that have had cataract or phpv operations to gain binocular vision especially if they get Strabismus (misalignment of the eyes) which is exactly what William has got. So we will be working towards getting good sight out of the right eye so he has a spare just in case something happens to his good left eye and if we have lots of good luck and no other complications, just maybe he'll get that ultimate goal of Binocular Vision.

Thursday 27th May

The next day I noticed that William's contact wasn’t in and went into panic mode. I looked for it in his cot and for some reason I looked down on the carpet and saw it shining in the sun – thank god I found it as they are expensive little things and we didn’t have a spare. The timing of losing that lens was perfect as Jason came home to drop Lexie off from Kindy which was lucky as we had pin William down to insert the lens back in. This time it didn’t go so easily but I finally got it in after a few tries! Not so easy.

Patching is just part of the routine now and sometimes William's patch would be on for most of the day. A side effect of patching to try and get the weaker eye to work makes young babies quite tired so they sleep a lot!

I have sourced some new patches as the ones we started off with seemed to rip his skin off and makes him cry!

We really believed William was seeing more and more every day as he smiled and looked around! It made it worthwhile patching and getting those contacts in and out!

Meeting with Dr Small - Wed 26th May

I had been thinking a lot about William's eye condition and researched a bit on the net about PHPV. I had also joined a PHPV support group on Yahoo. I read a lot of posts from other mothers that had children with this condition and there seems to be varying degrees of mild to severe PHPV cases. So on Wednesday 26th May I asked to see Dr Small (the PO who did the surgery) and had a LOT of questions that I wanted to ask him. Dr Small is fantastic and takes the time to listen to me and explain what we can expect with William. I asked Dr Small about William's lens, his retina, if he had to remove a stalk (constricted blood vessels which is typical of PHPV) and if all of William's eye was developed. Dr Small said he really doesn’t believe William has PHPV at all and its more a case of Cataract and a small eye. He told us that William's natural lens in his eye had to be removed and that he may be able to have an IOL (intraocular Lens) inserted into his eye when he is a lot older. Unfortunately IOL's are not like your natural lens so you are only able to focus up close or at a distance and then you need to use glasses or a contact lens for the opposite of the IOL. After we talked about a few more things we had to put in the new lens. I had cut my finger nails for this as I wanted to put it in as I didn't want William to have to go through the eye speculum thing again. Unfortunately we both couldn’t get the old lens out but Dr Small did and he made it look so easy. It was then my turn to put it in and (I'm still amazed at this) I got it in one go - unfortunately it wasn't to be so easy all the time!

One Week After Surgery - Wednesday 5th May

The following Wednesday we went back to have the contact lens put into his eye. Out came the eye speculum again and it was horrible, William cried and cried as Dr Maslin was putting in the lens (the Drs aren't exactly gentle!). All I could think was that Dr Maslin was going to do further damage to the eye when the speculum was inserted into it, I'd hate to have that thing put in my eye. Once the contact was in they did a focus test and decided he needed a new contact lens to make him focus a little closer. I thought it was a positive that at least William was focussing. They told us we were to patch William for 2 hours a day and take out the lens once a week and clean it and they’d see us in 3 weeks with the new contact lens. The one thing I have found is that we haven't been given much information about anything, i.e. how to clean a contact lens, how to take it out, how to put it in, and is there anything we need to look out for. I know William now has a higher chance of getting glaucoma than someone without an operated eye so it would be good to know any warning signs.

I have met another lady in the waiting room who has a baby who had a similar surgery (a cataract removed) to William's, though that baby doesn't have PHPV or a Microphthalmic Eye. He is 2 months older than William and its nice to have another mother to talk to about this whole situation.

Once we started patching William didn’t seem to mind the patch but also didn’t smile at us or respond to much but after a couple of weeks we really did see some improvement! He really looked like he was focussing and following movements and when we came up close he blinked!

Post Surgery - Wednesday 28th April

We went back to hospital the next day to have William's eye checked. Dr Maslin is the Paediatric Opthamologist that would be taking over post operative care. We were shown into a room and then a few Opthamologists came in and all peered at Williams eye and chatted about it. Dr Small told us to patch his good eye straight away for an hour a day and Dr Maslin didn't think there was any point as William wouldn't be able to see anyway. When we had his contact we'd have to patch for 50% of William's waking hours. I asked how would we tell if he could see with his contact in and Dr Maslin said if he can't see he'll rock his head back and forth and if that's the case then we'd have to up the patching to 80% of his waking hours. The contact lens would be arriving in a week and that's when Dr Maslin thought it would be a good idea to patch. We patched that week just to get William used to being patched and I think it was a good thing.

Surgery Day - Tuesday April 27th

The big day arrived and I had to give William his last feed at 7.30 am as he would be put under a General Anaesthetic. We left Lexie and Sophie with Gran and drove into the hospital at 10.30 am and were shown to the Childrens Ward. We had a nurse that did all the final checks, he weighed 6.1 Kilos (massive for a 7 week old baby) and he measured 62 cms. We were so thankful that he took a dummy (mum had persevered the 2 days prior to get him to take one) as he was not allowed to feed again until after the operation. We were told he was going to be taken away at 11.30am to be prepped for the operation but 11.30 am came and went, so did 12 noon and then 12.30 pm then 1 pm and finally about 1.30 pm we were taken up to the theatre meeting room where we waited for Dr Small. He finally arrived about 2 pm and while we were waiting we had been visited by a couple of other people that would be going in to watch and learn from Dr Small. PHPV is very rare and I'm not totally sure if any other babies have been diagnosed with this condition in Welington or NZ for that matter so there were a few people that were interested in this surgery! By this stage William had slept and sucked on that dummy all day in Dad’s arms and didn’t cry once, he was being soooo good. The nurse then took him away to be put under the general. Dr Small had asked if we wanted to come up to theatre to see him before they operated and show us what they were going to do so we had to dress in some surgical gear and then we were led up to theatre. As I walked into the room there he was so peaceful lying there asleep with a sheet pulled right up to his nose. There were a dozen or so people in the room but I didn't really notice as I wanted to check he was actually alive. I asked the Anaethetist if he was while looking at the machines to make sure his heart was still beating. We were then told what they were going to do then left and told to be back in the children’s ward in an hour. We went to have some lunch in the hospital café and sat chatting trying to keep our minds off what was happening up there. Back in the Children’s ward we waited and waited (the waiting was the worst part for us) and at about 4 pm we finally got the call from the Dr. The Dr spoke to me and said the operation went better than expected and that he would be waking up soon. We were so happy and relieved that everything had gone ok and were dying to see him. The nurse came and got us and walked us up to theatre and as we were walking up there I said "I never want to come here again!" Then there he was in a nurses arms with all these other nurses looking and cooing at him. Next minute he was in my arms still quite groggy from the anaethestic and I hopped onto a bed and fed him while being wheeled back to the Children’s Ward. His eye wasn’t patched but I did notice an arrow above the operated eye! The Dr came about 6 pm but I can’t remember what he said as it was such an emotional day! We were suppose to stay in the hospital overnight but they let us go home so we left and came home very tired from the days events. He was such a good little boy and that night he slept quite well considering his tragic day - and this was only the beginning. I look back now and think the surgery was actually the easy part its the rehabilitation of working on getting sight in the right eye which is the hard part.

Wednesday 21st April

We arrived at the Wellington Hospital Outpatients Eye Department at 8.30 am to find lots of other people waiting to see the Eye Specialists as well! We sat in the waiting room and began our wait (the Hospital is going through an upgrade but unfortunately the Eye Dept is still in the old part and isn't a very welcoming place!). Finally we were seen by a Registrar who had a quick look at William's eye and then she said she needed to get her boss - we knew then it must be very serious and we were praying that it wasn't a tumor. Dr Maslin came and checked his eye and announced matter of factly "oh yes its PHPV/Microphthalmic Eye" - what was PHPV/Microphthalmic Eye???? Dr Maslin then put some drops into Williams eye and we were told to come back in 30 mins. When we came back they lay William down on a bed and proceeded to get the eye speculum into his eye so they could have a really good look. I couldn't handle this so left the room as William was crying so hard - but Dad stayed with him. Once they checked his eye they told us to wait in a room and did an ultrasound on his eye to check to see if his eye was developed at the back. We were then seen by a Dr Small who spoke to us about this condition called PHPV. I can't quite remember what he said but it went basically something like "he has PHPV which is a very rare condition and means that his eye wasn't developed completely in utero. He probably won't be able to see out of that eye. He also has a microphthalmic eye which means he has a smaller eye than the other and it will always be smaller. He will live a completely normal life though and will know no different." Oh my god on one hand I was happy it wasn't a tumor but on the other hand I just didn't know what to think. Dr Small advised that we should have surgery to give him a chance at getting some sight but the prognosis wasn't all that hopeful. The operation was scheduled for the following Tuesday 27th April.

William Mark Brown

William Mark Brown was born on Monday 8th March 2010 at 7.30 am weighing in at 9lbs 4oz at Lower Hutt Hospital. Jason and I were so thrilled that he was a happy healthy big baby boy and the first boy in our family. We have two girls Lexie (4) and Sophie(2) and they love their baby brother to bits. Everything was going so well. At William's 6 week check with the Plunkett Nurse I mentioned to the Nurse that he had a smaller eye and she advised that we needed to get to a Dr that day. We went to the Drs and they checked his eye and said he needed to be referred to the Eye Department at Wellington Hospital. The appointment was booked for the following Wednesday at 8.30 am! That's when it struck us that there could actually be something seriously wrong with William's right eye. Thoughts of Retinoblastoma went through our heads and the next 5 days were hell waiting to find out what was wrong.