Head tilting

Another thing we have to "contend" with is Joel's head tilt. Try this:

Look at an object or room with both eyes open and head perfectly straight.
Take note of how much you can see.

Now close or cover your left eye and look at the same object with your head straight.
Take note of how much you can see.
Now tilt your head to the right and take note of how much you can see. It's a fair bit more than you can see with your head straight.

Babies learn to do this from an early age when they have a problem with their vision. It soon becomes a habit. Because they can see more of a room or object by tilting their head, that's what they do.

Joel's head always tilts. When he's laying down he tilts it and sometimes when he is sitting up he will tilt it. I have no idea whether or not he will grow out of it or if it will lessen the more he can see.

Another unknown.

Development in vision research

http://www.sciencedaily.com/releases/2010/05/100526170248.htm

Amazing

Joel's problem with his vision has very little to do with his retina, but if they can create a retina from stem cells - what else will they be able to do...

Gives me hope.

Patching

So every day I have to patch Joel's good eye for 2 hours. The purpose of this is to strengthen the muscles of his PHPV eye and help to build the pathways to the brain for that eye. Not being able to see out of it from birth has meant that it's become lazy and turns in toward his nose. We have a few boxes of patches, they are like almond shaped bandaids that cover his entire eye. At the start I had some huge problems getting the patch off his face, I would leave a huge red mark and lots of sticky glue behind. It hurt him alot too. One trick I have found is to put the patch on the back of my hand a couple if times before putting it on Joel. This takes away a bit of the sticky and means it's easier to rip off. I will have to find a better trick as he gets older though because he will just rip it off himself if it's not stuck on properly.

He doesn't like to be patched. Because he doesn't have any useable vision in that eye, I assume all he can see is blurry shadows. He wants to be held, nursed, cuddled and talked to while he's patched so I have to be organised enough to dedicate those 2 hours to him every day. I have to find an activity and have food ready for Annalise so she's happy too. I have found the easiest time of the day to patch is from about 3-5. I can keep him awake for that long an Annalise is usually sufficiently tired from the mornings activites that she will sit and watch a movie.

Once I put the patch on I try to distract him straight away. He has a teddy bear that plays music and it's face lights up so I show him that. I assume he can see the glowing light. I don't really know. Then we walk around the house looking at things and I tell him what we're doing. It's all very exciting stuff like " oh look Joel, we're cleaning a cup". If we just sit down and stare blankly at nothing he grizzles and eventually cries. Some days the two hours goes forever. Some days it goes quickly. Every day it makes me sad to see him with his patch on but I know it's for the best.

It gets easier...

I had an email from another mum who has a child just diagnosed with PHPV (or PFVS, as they seem to know it more by that acronym in the US) and she was feeling how I felt right at the start of this journey. I just wanted to put it out there that it does get easier. I was told that by other parents with APHAKIC kids (kids without the natural lens of the eye due to surgery or defect) and I never thought I'd get there and stop crying but it does get easier. You always think about it but already, after only a few months, it doesn't have that "sting" to it like it did before. So... chin up and stay strong for your little ones xx

Today is a much better day

This morning I got the lens in fairly easily. I wrapped him up in his big sheet and put him on his play mat with his feet facing the tv so he was looking down and I could put it in much quicker. He didn't even cry :-) So proud of my little man, he's just the best.

The hospital were supposed to call me this week with his next appointment time but they haven't yet. I called yesterday and they'd gone home so I called back today but no one answered. They are so busy and understaffed. They do such an amazing job with all these kids coming through. In January they saw something like 8,000 kids. Not just for Opthamology but also for other problems like hearing and neurology. I'll try again this afternoon.

After the hospital I'll go past Ezekial again an get his Optometrist to make sure the lens is all ok.

Annalise is at Kindy today, it's only her second time but she loves it. I am so glad. I am so happy she has something that's just "hers". I seem to spend so much time with Joel she misses out. Tomorrow we are making a herb garden together so I think she'll love that.

I have noticed that Joel sleeps a lot more when she's not here, must be the quietness in the house!

I am really hoping that my writing about the ups and downs of PHPV is helping other parents and giving others an insight into this rare condition. 4 months ago I knew nothing about eyes. It's funny how quickly you can arm yourself with information when you have to.

It's in!

This is what I did:

Instead of wrapping him in a muslin wrap, I used a really big cot sheet with his arms pinned by his side, not across his chest. I sat on my knees with him between then, so my knees were roughly at his armpits, not squeezing him, just more to get to his face at the right angle. Previously I had been leaning across him, like sitting at his side or leaning over the change table. With my left hand, I lifted his lid and held his forehead at the same time and had the lens in my right hand. When I needed him to look down, I said "Ah boo!" and he looked down toward me. I slipped it up into the top lid then pulled the bottom lid down and it slotted into place. Sounds easy hey lol.

Unwrapped him then lots of cuddles.

RELIEF.

Now we can get on with our day.

Bad day

Well today is a bad day.

Every day, Joel wakes up at 7. I feed him, give him a bath, feed him again then try to put his lens in on my own until he gets tired at 8. Only two times have I been successful in doing it on my own. If I can't do it between 7 and 8, when he wakes up again at 9:30, I have another go and if I stil can't do it I drive to my MIL's place and get her to restrain him so I can put it in.

Today I tried between 7 and 8, then again for about 15 minutes from 9:30 - 9:45. I feel like I am NEVER going to be able to do it on my own :-( I ended up in tears, yelling at Annalise, angry at myself, and feeling pretty useless.

Yesterday I called another mum who has a daughter who wears a contact and asked her how she went about doing it on her own. She told me a big tip is to keep my nails really short, so short that there is absolutely no white nail showing. So yesterday, I cut off all my nice long nails. It doesn't make a difference. It's still impossible to get the lens into the eye of a screaming, wriggling 4 month old. She told me to get Annalise to distract Joel so he's looking down and that way I can slip the lens into his lid easier. All that happened was me and Annalise cracked our heads together as I was leaning over Joel to put the lens in and she was leaning over him to sing to him. It's just crap. It's so crap.

If I don't put it in, I can't patch him because he can't see without the lens. If I don't patch him, the chances of him ever seeing out of that eye are even more slim. It has to go in.

I am going to try it again and if it doesn't go in I suppose I will have to pack both kids into the car and drive to MIL's place again.

I just want to scream. Or cry.

Joel's Contact Lens

At our third or fourth hospital check up, Dr Lam looked into Joel's eye and said that he could see all the way to the back. This was AMAZING news because it meant that if Dr Lam could see in, Joel could see out. Light was getting to the back of his eye where it needed to go. I was so excited. I rang Rich to tell him and he was really happy too. This meant that we could get Joel fitted for a contact lens and start him on the road to seeing out of his left eye. We got a referral to Ezekial Optometrists, but again, had to wait a week to see them.

We went in and spoke about the lens, how it works, how you put it in, what it looks like. I was surprised at how big and thick it was. It is called a Rigid Gas Permeable lens and is like a curved round disk with 3 holes in it. It is made of a hard plastic and is almost invisible once it's in the eye. It was ordered for us and again, we waited 2 weeks for them to manufacture.

Finally the day came where we could take Joel to have some "practice" fittings of the lens. Rich took another day off work and we went back to Ezekial. Damon, Joel's Optometrist, told us it would most likely take the whole day to get the lens right. We got there at 9 and went into Damon's rooms.

He explained how to put the lens in. One of us would be the "restrainer" and the other one would be the "do-er". It was decided I would be the one to put the lens in because Rich has a weak stomach when it comes to these things and chances are I'd be putting it in more often than he would anyway.

We laid Joel down on the bench with a little folded up towel under his head.

Rich stood behind his head, while I leaned over him with the lens in my right hand.

Damon stood next to me and gave me instructions.

It was SO hard. Rich had to hold his little arms beside his head to stop him from moving his head and thrashing about his arms while I lifted his upper lid with my left hand and tried to angle the lens onto the white of his eye. Joel screamed. He squealed like a little baby pig. I just wanted to get it done, I just wanted to learn how to do this for my baby boy. After a few minutes of trying unsuccessfully to get it in, Joel's eye and the skin around it was red and he was really upset. Damon suggested we take a break and come back in a little while.

We walked around and I fed Joel and after about half an hour we went back in. This time I was determined to do it right.

We all assumed our positions again and I held his eye open and eventually got the lens in. It felt good to know that I could do it, I was just praying I'd get quicker at it. Damon had a look with one of his machines and said the lens was a bit too big for Joel and he'd have to reshape it so we went for another walk, got some lunch and after an hour, went back to Ezekial.

This time, it still took ages to get the lens in and it was my turn to try getting it out. Getting it out was far harder than getting it in. Joel screamed and screamed while Rich held him down and I clumsily poked and prodded my fingers into his eye. The lens is so slippery. Before you put it in you need to rub a conditioner over it, which is like oil so if you get any of that on your fingers, they just slide on his skin and you can't get his eye open. We got it out but then had to put it back in again to wear it home. Again it took ages. We ended up leaving at 3 in the afternoon with his lens and a stockpile of saline, conditioner and cleaner.

Every morning, his lens goes in. Every night his lens comes out. I am getting better at it every day. Some days it goes in really easily, other days it doesn't but overall, it's a lot faster than our first attempt. The hardest part is trying to do it on my own. 5 days out of 7, Rich has left for work before Joel wakes up so it's up to me to get it in. We have tried waking him up before Rich leaves but he was so upset at being disturbed that it was impossible to get him to open his eye. Most days I have to drive to my mother in laws house, or my mums house, just to get someone to restrain him so that I can fit his lens. I still try to do it every day on my own but haven't quite worked out how to be successful at it by myself every time.

Post surgery recovery

We stayed in recovery for a few hours then drove home. I stayed with Joel while Rich went to get Annalise from her Nanny's house. When Annalise saw Joel, she said "Oh Joel. You have a sore eye" and kissed him. It was so sweet. She has been so good with everything. I sometimes think about how unfair it is on her. True, when the second baby comes along your attention is divided but with Joel needing so much more time than a "normal" baby, she does miss out. She also has to see and hear Joel when we are trying to put his lens in and misses out on mummy for 2 hours a day while Joel is patched. She is such a good kid.

The day after surgery we went back to the hospital to see Dr Lam and the Registrar, Josh. They said that the surgery went well and took his patch off to have a look at his eye. I couldn't believe how good his eye looked. The cataract was gone so we could see the beautiful blue colour of his iris. We could see blood through the colour though, which is normal after surgery but it still freaked me out a bit. The surgeons told us that they had a good look at Joel's right (good) eye during surgery and that there was absolutely nothing wrong with that eye. This was a huge relief. We were able to leave his patch off and just had to put drops into his eye every hour for 2 weeks to stave off infection and keep the swelling down.

We went back home and I just layed down on the bed and prayed over my baby. I prayed for ages. I cried and spoke and asked God to heal Joel, to give him his vision and make his recovery fast and asked God to not let Joel develop glaucoma. Just give him the best possible chance to recover, to have vision and to stay healthy.

3 days later we went back to the hospital for another check up. We were told we'd have to go back twice a week for a month. Josh looked at Joel's eye and said the bleeding was lessening and his eye looked really great and that he didn't need to see us for another 2 weeks. I thanked God the whole way home :-)

After a week, photos of Joel show that it didn't even look like he'd had surgery. It was truly an amazing recovery.

The surgery

We got to the hospital early in the morning and everyone we dealt with was great. So understanding and gentle and patient (especially with me, every time they asked me a question I would cry). We met the Anethetist and Dr Lam and went through the procedure. He was having a vitrectomy (removal of some of the vitreous - white part - of his eye) and a lensectomy (removal of the lens). This would take away the scarring at the back and the cataract and membrane at the front. Without the lens, the light that goes into the eye is dispersed instead of focussed on the one spot it needs to go, the retina. Joel would never be able to see without the use of glasses or a contact lens to replace his natural lens.

Joel had to fast from 9am until his surgery at 1. This was harder for me that it was for him. All I wanted was that closeness, that bond with him. I wanted to put him on the breast and hold him and never let him go but I couldn't. I couldn't even hold him because every time I did, he would nuzzle down searching for milk. Rich had to hold him the whole time while I sat in a big red leather chair and watched. Finally the time came to hand him over, only one of us was allowed to go in so I took my baby in there. The nurse came up and asked "Is this Joel?" and again, I lost it. I told her yes, this is my baby Joel and she asked if I wanted to give him a kiss and he would see me in a few hours. I kissed him all over his little face and he smiled at me as they took him through the doors.

We went to get something to eat and waited for it to be over. After almost 3 hours we were sill waiting and I was convinced something was wrong, that they'd found something else wrong with his eye. He had already had an ultrasound of his eye - we had to restrain him while they put gel on his eyes and for 10 minutes ran the ultrasound machine over his little face - so we knew there was nothing else there but you just can't help thinking "what if". FINALLY the nurse called out "Joel's mum?" and I was allowed to see him.

He was in recovery, wrapped up in about 10 blankets with his black spiky mohawk sticking out the top. This time, I didn't cry. It was over and he was back with me and the surgery was done. I just held him while he started to wake up and look around for me. He had a big gauze patch over his eye with a plastic shield over that. He looked SO small. It took him about half an hour to recover from the GA and when he did, he was hungry! I fed him really slowly for a while then took him out to see his daddy. He was 8 weeks and 5 days old.

Emotional Affect on Parents

One thing I haven't really covered is the feelings and emotions that go along with the disgnosis of PHPV. They are so wide and varied but from talking to other parents of PHPV kids - we all feel them. We all seem to go through the same grief process and can identify with and understand without judgement how it feels to know your child has PHPV.

At first, all I could do was cry. I would be feeding my little boy and I'd look down at him and I'd cry. I'd be changing his nappy and I'd cry. I'd be laying in bed at night thinking about him and I'd cry. For about 2 weeks all I did was cry. I didn't want to go out. I didn't want to talk to anyone. I just wanted to stay home and cry.

I felt sad for him. I kept asking "why us?". I felt embarassed there was something wrong with him. I imagined his future, him being teased for having a small eye or not being able to drive or play sports. I felt angry. I felt helpless and useless. I felt guilty. Oh that mothers guilt is a killer. I kept thinking it was something I did wrong when I was pregnant. Something in my genes. I was so SO angry when I saw a pregnant woman smoking. I was so mad when I would hear that a woman who drank through her pregnancy had a healthy baby who could see out of both eyes. I did everything right during my pregnancy, EVERYTHING. I did it all by the book and yet here I was with a baby boy who could only half see the world. It made me really mad and it still does when I see a pregnant woman smoking or drinking or eating any of the foods we're not "supposed" to eat.

I found a group of other parents of kids with PHPV and they all told me it gets easier in time. And it has. Now, I don't feel guilty or sad for him. I never ever forget about his eye, it's always there in the back of my mind, but it's just become part of who he is now and who is is, is a funny, handsome, gorgeous, smily chatty little boy who brings happiness and light to everyone who meets him.

Next post will be about his surgery, the ongoing care, contact lens and patching.

And so we prayed...

We asked our whole church to pray, we asked other churches in Perth to pray. We prayed for healing and read every healing scripture we could find. We prayed for wisdom with the decision for or against surgery. We prayed for peace and God's protection over our little boy. We just prayed and prayed and prayed. Rich fasted. Other friends fasted, and we al just kept on praying. I took Joel to Sydney with me for the Colour conference and it was there, 2 days before his booked surgery date, that God gave me confirmation that surgery was the right option. Tears streamed down my face while I held my baby boy, I have never in my life cried like that before. I felt humbled, grateful, in awe, loved and covered. I knew God would protect him, I knew that whatever happened, God would make it right and guide the surgeon's hand. I cried out to Jesus. I sang JESUS JESUS at the top of my lungs and just knew in my heart that Jesus was holding my baby boy. I thank God every day for His healing and protection on my son and I thank God that His son loves my little Joel.

My baby's not "perfect"

We are waiting for our turn to see him, a man called Dr Lam. I look around the room and there's so many kids. So many kids with glasses, patches, lazy eyes, blind eyes...it makes me so sad. I cry again. I look down at my baby boy and I cry. All I can think is "don't end up like these kids. This sort of thing doesn't happen to me or my family, it happens to other people, not us". Dr Lam calls us in and we sit down, I've got my little boy in my arms and he asks why are we there. Again with the shiny, small eye story. He gets out all his instruments and has a really good look into my baby's eye. I can feel this big ball of anxiety in my chest, like I'm going to be sick, I feel like I can't breathe. The expectancy of it all, just tell me what's wrong!! I can feel the way I'm looking at Dr Lam, like a child looks at their mother when they hurt themselves, "just make it all better. Just fix it". He sits back in his chair and tells us, "what Joel has is a condition called PHPV. He has a cataract as well, and a membrane behind his lens. His eye is micropthalmic, so it's significantly smaller than the other eye". I don't say anything, my husband doesn't say anything. Joel cries because I've been holding his head straight. In my mind I have all these questions but I can't get them out. Finally I say "Can he see?". NO.

Dr Lam tells us our options. He can operate on Joel, on my precious, tiny, longed for baby boy. He can remove the scarring at the back of his eye, the cataract on the lens, he will remove his natural lens. The surgery will take about an hour and a half. I ask "will he be able to see? Will his eye grow?" He tells us that the surgery is done mostly for cosmetic purposes, the scarring is preventing his eye from growin so in theory, by removing it, the eye should grow. Also, removing the cataract means his eye won't glow like it does, it will look "normal" in photos. It's all for cosmetic purposes though, and we're told that if he gets any vision from the eye it will be a "bonus". If he can see shadows, we should consider ourselves lucky. So what are the risks? Increased risk of glaucoma, PHPV eyes are predisposed to glaucoma and surgery increases that risk 10 fold. Detaching the retina during surgery. The usual risks with general anesthetic. Having absolutely no useable vision at all after the surgery. It's a lot to take in so we go away to think about it, surgery is booked for 15th March just in case we decide to go down that road. We are to come back and meet with Dr Lam in 2 weeks. Joel is just 5 weeks old.

Waiting for a diagnosis

When we took Joel to the hospital, I explained the shiny eye to the triage nurse. She had a look and told us to wait. We were waiting for ages and there was another family there with their son who looked about 3. The dad was getting really agitated with how long it was taking to be seen and kept asking "how much longer?" and every time a doctor came out to call the next patient, the father would tsk and sigh when it wasn't their turn. All I could think was "Yeah your kid has a cough, mine is blind, wait your turn". Finally Joel's name was called and we stood up to go in and THAT father cuts in front of us and says to the doctor "I need to see you, can we talk? My son is here and we've been waiting for a really long time and he hasn't had anything to drink". The doctor then takes this other father into a different room so we are left waiting. Again. Sitting in this room looking at our sleeping baby boy, waiting for a doctor, waiting for help, waiting for someone to come and tell us what's wrong with him. The doctor comes back, apologises and asks us why we're there. Again we explain his shiny eye, the fact it's smaller than the other eye. She has a look, shines some lights into his eye, weighs him and says "I'll need to get the Pediatrician to look at him". So, we wait. An hour passes, my husband and I sitting in a tiny room looking at our beautiful sleeping baby boy, trying to guess what's wrong...cataract, cancer, blindness...what if, what if...another hour...we're not talking, just looking at each other now. I'm crying. He's angry...Pediatrician comes in. Asks us what's wrong. Again we explain his small, shiny eye. She has a look, shines some lights in his eye, covers the good eye and says "We'll have to get the Opthamologist to look at him". We ask her what does she think it is? She says "It looks like a cataract but I'm not sure". We ask if she thinks he can see and she says.... NO....I cry again. My baby boy can't see.

We ask when can we see the Opthamologist and she says we can see the Junior Eye Registrar today but he deals more with eye traumas and it's probably best if we wait to see the Senior Surgeon. We know from that suggestions that the disgnosis isn't going to be good. This was on a Sunday. I am told the Senior Surgeon's office will call me during the week to make a time, just go home and wait....

By Tuesday I haven't heard anything from them so I call to make an appointment. Thursday at 1:15. 2 days and we'll know what's wrong.

All day Tuesday and Wednesday I google and research as much as I can about problems with babies eyes. I come up with a best and worse case scenario. I tell my husband:

Best case is that it's just a cataract and they can remove that and he'll be able to see.
Worst case is retinoblastoma, cancer of the eye. They'll have to remove the eye.
In the middle is a condition called PHPV, man I really hope he doesn't have that!

I tell myself for 2 days "it's just a cataract, it's just a cataract".

Waiting is killing me, I just want to know what's wrong.

Thursday we go to the hospital to meet with the Senior Surgeon.

4 months in...

I thought I'd start this blog to help me, other people, people with PHPV, parents of PHPV kids... everyone really. It's such a rare condition/defect that no one really understands what we go through every day. The emotions, the challenges, surgeries, contact lenses, patching...Sometimes it just feels like it's never going to end. And it probably won't. We (I) will do what we (I) can for Joel now but eventually it's all going to be his responsibility to manage his PHPV when he's old enough, I just hope that between now and then I do everything I can to make sure he has the best possible outcome with his vision.

PHPV (or PFVS) results when a blood vessel at the back of the eye isn't absorbed into the system like it should. This should happen before they are born. That vessel causes a scarring in the vitreous (white part) of the eye, essentially blocking all light from getting into the eye, which results in blindness. The scarring also restricts the eye from growing, so the PHPV eye can be saller than the non affected eye. This is called micropthalmia and it was my main reason for alot of my upset when we found out about Joel. All I could think of was the teasing at school, the not looking as "normal" as everyone else. I felt so bad that I was worried about the cosmetic look of him when really I should have been more upset that he was blind. He also had a cataract on his eye, that was how we first noticed something was wrong. We noticed that under lights (like down lights) his eye would glow, like a cats eye. A big shiny silver glowing eye. Like a mirror. Very scary to look down at your baby and see that. I did all sorts of little experiments of my own, like covering his good eye to see what would happen and shining a torch into his bad eye to see the reflection. When I covered his good eye he would freak out. I knew he couldn't see out of it even before we saw the PO (Pediatric Opthamologist). I just knew he was blind in that eye. I left it for 3 days before taking him to the hospital. I just wanted to have my perfect little baby boy for that little bit longer. He was 3 and a half weeks old when he was diagnosed. Those 3 and a half weeks were the best weeks of my life. I had my perfect baby boy, my beautiful daughter and my marriage was getting better all the time. Things were just perfect.