Forever is a Really Long Time

It’s been a while since I last posted, mostly because there’s been nothing new to report, but also because it’s taken us a while to process the things that have been worth reporting. Josh had some complications following his surgery last June, which I’ll get into another time. For now I want to talk about our newest battle…

One of the risks associated with Aphakia (no lenses) is increased intra-ocular pressure, which left untreated can lead to glaucoma. There are a couple of different types of glaucoma but basically the high pressure in the eye damages the optic nerve and the eye sight deteriorates as a result. We always knew it was a risk and at all of Josh’s routine eye check ups the doctor tests the pressure in each eye. It’s always been normal, until November, when it was elevated enough in his right eye to cause concern. Our PO prescribed an eye drop to be used once a day to bring the pressure back down, and we had a few visits very close together to keep monitoring it. Fortunately it went down, but we were advised to continue using the drops. We had another appointment yesterday, and the pressure was slightly elevated again. We had been away and forgot to take the drops with us so we’d missed a few doses. The fact that the pressure was elevated again told us two things – One, that the drops are very effective, and two, that Josh will need to use them indefinitely. There will be times, like last week, when we can do a controlled break from them, just to make sure he still needs them. But for the rest of his life Josh will face this risk, and he will always need something to control it. Forever. It was a stark reminder that this isn’t going to go away, and it isn’t going to get any better. 

We are fortunate that the drops are working for now. There are other ways to manage elevated eye pressure, and some of them are a lot less pleasant, so we’re hoping the drops continue to be enough for quite some time. We’re also considering trying contact lenses again. They will help him to better develop his peripheral vision. Because his glasses are so thick they tend to distort what he sees out the sides, and he turns his head to see, rather than moving his eyes. With contacts there is less distortion, and we still have a limited window of opportunity to help him develop his eyesight. Unfortunately we’d need to take the contact out every night in order to put the drops in so it’s a decision we really have to think about. 

For now, Josh continues to be oblivious to it all. He takes the eye drops in his stride (with a lot of help from daily rewards!) and wears his glasses without complaint. He also treats them very carefully, for which we are very lucky and grateful! It’s easy to forget that our little man is facing such enormous hurdles, but most days we take our cues from him, and just get on with it. Because, as Josh says, forever is a really long time. 

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Deja Vu

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I’ve just come back from Sydney, where Josh is recovering after his second lensectomy (removal of the lens). I wrote about why we needed to do this procedure so soon after the first one in this post, and even though we knew what to expect this time, we were still quite anxious. Until now the left eye has been his “good eye” – the lens was out of place, but not as much as it had been in the right, and he used that eye whenever he was looking at things up close. He hated wearing the patch over the left eye as it severely limited what he could see. The lens was gradually shifting away from the centre of his eye, so it was only a matter of time before the vision deteriorated, but it felt cruel somehow, to be taking away his best form of sight…

Again we found ourselves at the Sydney Children’s Hospital at Westmead. There’s something about that place that makes me want to run screaming in the opposite direction. The nursing staff are brilliant but our poor jet lagged boy was kept waiting until 4pm, 2 hours after he was scheduled for surgery, making an incredibly difficult day all the more challenging. He had nothing for breakfast but a banana, and I had woken him up early to go to the doctor before going out to the hospital. It was a long and stressful day, but he behaved so well, and took it all in his stride. He constantly amazes me with how resilient and patient he can be with all that he has to cope with.

The procedure itself was textbook. The anaesthetist had given him an extra drug to help avoid what they call “emergence delirium” – the mother of all tantrums that can occur as kids come round after a general anaesthetic. He was in recovery, asleep, for a long time before they’d let me anywhere near him, and it was tough. He woke up crying, in pain, and trying to tear the protective shield off his eye. We spent about an hour truing to calm him down and keep him from rubbing his eye. His tears were bloody, and he kept drifting in and out of consciousness. We had to hold his arms and it took a couple of attempts to get medicine and fluid into him. At one stage the nurse was squirting little drops of painkiller into his mouth with a syringe and in between doses I was shoving a lemonade ice block in behind it. It took lots of coaxing and cuddling, and the undivided attention of four adults but eventually he was lucid enough that we could explain to him that he needed to  put the patch on before they’d let us leave, and he suddenly became very compliant! He threw up twice on me in the car, and held my hand the whole way home but the relief that it was all over was immense.

Two days later and Josh’s eye is bruised and swollen, and he is refusing to wear his glasses, but he’s pretty much back to his usual self. He still has one surgery to go but it’s not as urgent yet, so we’ll delay it as long as we possibly can. 

He has one more visit with the doctor and then hopefully he and his dad can come home. Once the eye has completely healed he’ll be prescribed a new contact lens and we’ll begin the long process of getting the prescription right, whether it’s contacts or glasses, or a combination of the two, and hopefully improving his eyesight.

Thanks, as always for all the support xx

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Round Two

We’ve had an interesting time since I last updated… Josh is now refusing to patch at all, and we’ve given up trying to get the contact lens in. We did put it in last week but it fell out at some point. I found it the following day after sweeping the floor, rehydrated it and cleaned it, but I’m not keen to put it in again. We were lucky to make it this far without losing one, but it didn’t help that Josh didn’t seem to notice that it wasn’t there anymore! 

Since one eye is short-sighted and the other far-sighted, it takes a lot of effort for the poor guy to see. He is good at self-regulating, and we’ve noticed that he’ll often close one eye depending on what he’s doing. Ideally he should be using both eyes to see everything so this isn’t a long term solution. It’s also completely exhausting, and Josh has reverted to daytime naps (and temper tantrums!) because it’s all too hard for him.

So, we’ve decided to bring the second surgery forward, and instead of doing it in August, we’ve scheduled it for June. As in three weeks from today. Since it’s so soon after the first procedure we can’t all go. On the upside we don’t need to spend as much time away, but it will mean one of us going to Sydney with Josh and the other staying in HK with Charlie. We will probably swap halfway, it’ll be tough, but we’re hoping that the separation will be the hardest part of all of it.

Josh is not yet in kindergarten and we’ve already noticed a few delays in his development, so we’re anxious to give him the best chance we can, as soon as we can. There’ll be no more room for excuses, and once both eyes are on the same level, the hard work will really begin. 

Keep all your fingers crossed for us next month, and I’ll post again on the other side.

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A Rough Patch

I’ve been wanting to write a post about patching for a while now. Josh has amblyopia (a weaker eye on the right) and we have been putting a patch on the left eye since his initial diagnosis, with the aim of strengthening the weaker eye. He has always tolerated the patch fairly well. Ortopad make some pretty cool patches for little ones and Josh has always had fun choosing which patch he wants to wear. The need for patching was increased with the surgery, as the whole point of it was to build the vision in his right eye. Unfortunately with all the changes and the addition of the contact lens, he has started fighting us every day, and we are having to work a lot harder (and bribe more!) to keep the patch on.

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I’ve noticed that a lot of people comment on the patch when Josh is wearing it, and this often makes him uncomfortable, especially when he’s not that comfortable wearing it to begin with. He’s at an age where he’s much more aware of the fact that he’s a little bit different and he doesn’t quite understand why he needs the patch or the contact lens or constant visits to the eye doctor. I read this fantastic post on Little Four Eyes today, and it explains the need for patching, and how best to respond to kids wearing them, better than I possibly could. So please take a read if you have time, it’ll help if you see my little guy and want to know what to say to make his day a little easier.

What not to say to young kids who wearing an eye patch | little four eyes.

Contacts

I was so completely terrified about the whole contact lens thing, so I was more than happy to have a couple of weeks wait between the surgery and the lens arriving. Josh recovered beautifully from the surgery, with very little redness or swelling, and we even managed to integrate the antibiotic/anti-inflammatory drops into our routine three times a day without too much drama.

We had a narrow window of opportunity to get back to Hong Kong while Dave was with us, so the pressure was on to get the contact lens and try it out for a few days before we got the go-ahead from the doctors to go home. I had to chase the eye clinic to get us an appointment and, with a couple of days to go before our flight, we headed out to Westmead and picked up the lens. I wear contact lenses from time to time and we talked about it a lot with Josh beforehand, and I showed him one of mine and let him play with it. He even pretended to put it into my eye. He was pretty excited about it, but we were still really anxious.

I had a brief tutorial on lens care and insertion with the orthoptists while Josh played in the waiting room. When we brought him into the room he was immediately tense and uncooperative and it took three of us holding him down and wrestling for 20 minutes to get the lens in. It was almost impossible to get his eye open and it felt like he was trying to retract his eyeballs as far back into his head as possible! Once it was in he barely noticed it and we were sent home with assurances that we would have lots of support while we remained in Sydney. We also knew we would have to take the lens out before our flight…

One of the potential issues with this type of lens is the fit. It’s custom made based on the measurements taken while Josh was under anaesthetic, but while you’re asleep your eye muscles are relaxed so it’s difficult to get a completely accurate measurement. We had to keep the lens in for a few days and look for any signs of poor fit or irritation. He still had stitches in so infection was also a risk. For 48 hours we were anxiously watching his eyes, checking to see if the lens was still in every hour, watching him anytime he rubbed his eyes, it was nerve-wracking! An incorrect fit would have meant another two weeks in Sydney while we waited for a new one, but thankfully Josh was fine, and so was the lens. We even started to notice him noticing things, like leaves on trees and texture on walls – things he’d never seen clearly before.

We had our final visit with the PO the day before we were due to fly home, and while his vision was still really poor (20/200 or 6/60) she was really happy with it all: a huge relief! She did ask me to take the lens out while we were there, just to make sure I could. Then we tried to put it back in and after twenty minutes of screaming and thrashing about I decided to leave it out. The doctor was confident in my technique, we were simply having a compliance issue, a big one, just as I had feared. He has glasses with a ridiculously thick lens on the right aphakic side that he can wear when the contact is out so that’s how he travelled home. Once we were back in Hong Kong, in our comfort zone, with some fairly substantial bribes on hand, it was a lot easier to convince Josh to stay calm and let us try putting the lens in. We also waited until he decided he was ready and when we did it successfully for the first time without fuss, there were high 5’s all round, and just a little bit of shock from all of us that we managed it! We have to take it out weekly for cleaning, and anytime he’s swimming or on a plane (because of the dry air) but so far that’s proven to be far easier than we anticipated. We only have one lens, so I’m expecting to lose it any day now, but apart from that it’s all feeling pretty manageable.

Now we wait to see if any of it has made a difference to his sight…

The Post-Op Debrief

I’ve never felt more grown up than I did on Tuesday. Watching a your child go through something like surgery is never easy, no matter how simple the procedure, and with Josh’s op there were so many unknowns. In trying to improve his eyesight we could very easily have made it worse. Making the decision to go ahead with Josh’s surgery was ours alone to make, and all we could do was hope that we had made the right one. I wanted to write down all the details of Josh’s procedure in the hope that it might help parents who have may have to go through it one day. 

Josh had to fast from 7am so we woke him at 6 to make sure he had time to eat a decent breakfast. He was pretty excited as he’d watched us pack his new toys and favourite pyjamas into his bag the night before. We were told to be at the hospital at 12, for a 1pm procedure. We arrived and went through the mountains of paperwork and then the waiting began. It seems endless, and there are constantly people coming to see you, running checks and asking the same questions. The surgeon arrived just before 1 and although we were supposed to be first, they called up another patient ahead of Josh…

We’ve come to learn that waiting longer than you want to is all part of the game. We were lucky that there were some really nice families waiting with us, some who had been there before too many times to count. Josh was really well behaved given the wait, but he was tired and starting to get impatient by about 2 o’clock. We both gave him lots of cuddles and convinced him to sit on Dave’s lap and have a rest. Within minutes he was asleep…

ImageHe stayed asleep until they came to get him and the anaesthetist put the mask on his little face so gently that he was completely unaware of what was happening. It was such a contrast to his last experience with anaesthetic, and it meant that he woke up calmly and gently too.

We were told the whole thing would only take 45 minutes and were sent to a recovery ward on the other side of the hospital. We checked on Charlie who was outside with his grandparents, and went back in to wait. The waiting is the hardest part. It’s unbearable, and unavoidable. After an hour and a half someone came and found us and said that Josh was actually back over where we had left him so we dashed across, afraid that he had woken up and we weren’t there.

He was slowly drifting in and out of consciousness with a large patch over his eye. We didn’t get to see the doctors straight away but the nurses told us everything had gone really well. A huge sigh of relief! I sat with him a while listening to him breathing, but by that stage Charlie’s patience had run out, and he was hungry and tired. I had to make the tough decision to leave before Josh was awake, but Dave stayed with him and reported back.Image

Within a couple of hours he was up and devouring a bowl of spaghetti and toast. He was pretty heavily medicated so there was no pain, and there really hasn’t been any since. The following morning he saw an ophthalmologist at the hospital’s eye clinic and there was minimal swelling and very little bleeding so he was allowed to come home. We have to put in eye drops every few hours and keep the eye clean. No swimming or sandpits for a few weeks, and no “rough play” until the stitches dissolve (not an easy ask for a 3 year old!), but other than that it’s business as usual. 

We have another follow-up appointment tomorrow, where he’ll hopefully have a vision assessment for his new prescription, and then we wait for the lenses to be fitted… We’ll be back to do the other eye later in the year.

We’ve been overwhelmed by all the love and support, and want to say a big thanks to those who sent messages this week. We still have a long way to go but we are over the first hurdle!

 

A Fake What…?

Since I last posted we have done a lot of research. Lots of googling, making phone calls, emailing, trawling through forums, and generally making a nuisance of ourselves with the doctors involved in Joshie’s procedure. We’ve been heartened by what we’ve read and what we’ve been told, and are going into tomorrow’s surgery safe in the knowledge that the new plan is the best plan. 

So, tomorrow afternoon, under a general anaesthetic, surgeons will remove the lens from J’s right eye, and the capsule that holds the lens (a Lensectomy). It’s a fairly straightforward procedure now that they’re not putting in an IOL, and as much as I was disappointed about that, I have to admit I’m a little relieved. He’ll wake up with a dressing over his eye, and we’ve been told he may even be able to come home tomorrow night.

Aphakia is a new term in our everyday vocabulary, but it’s just a word used to describe an eye without a lens. Bilateral Aphakia is when both lenses are missing, and unilateral or mono aphakia mean just one eye. The risks associated with Aphakia are numerous (but we’re trying not to think about those). Infection and Glaucoma are the biggest and J will have several doses of eye drops and daily trips to the Ophthalmologist for at least this week to monitor his condition.

Once the swelling goes down, in about two weeks, he’ll be fitted for a contact lens and the fun and games will begin! For now he’s actually excited about going to the hospital. He’s been promised a collection of new “rescue vehicles” that we bought as a reward/bribe at the toy store last week. He’s completely forgotten the trauma of his last visit to the day surgery, unlike his parents, and he’s ready for his “new eyes.”

It feels like we’re on the cusp of something pretty momentous, and all day I’ve had this feeling that this was the last day of life as we’ve known it. It sounds dramatic I know, but there are big changes and challenges ahead, and right now I’m just trying to be as excited as Josh is. But first I have to cook him sausages and hot chips for dinner – tonight my big brave boy gets whatever he wants!

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Plan B

As Josh’s condition is so rare, the treatment options are limited, and so is the information about them. We talked to a lot of people and did a lot of research on our own to get our heads around what we needed to do. Our PO presented surgery as an option, but she didn’t push us one way or the other. Surgery went against all my natural instincts as a mother, I hated the idea of it, and really wanted to take the more appealing “wait and see” approach. But the more we researched, the more we realised that surgery was our only option.

So the wheels were set in motion for Josh to undergo a lensectomy. This involves removing the lens completely. It’s a common procedure, usually performed in people with cataracts. In a standard lensectomy, an artificial lens is placed into the sac that holds the lens, and there’s no more to it. In Josh’s case, the sac is unstable so it would never be as straightforward as all that. The option we were presented with was contact lenses. An eye without a lens is “Aphakic” and there are special contacts made for just this purpose. I imagined that trying to put a contact lens in a toddler is like trying to walk a cat on a lead. After a little more research we learned that Aphakic contact lenses cost around $250 USD each, and they’re not the kind of thing you can easily pick up at your local optician or drugstore. Factoring in how often he will lose or damage a lens, and the struggle we’d have taking them out and cleaning them and then trying to put them back in, this was not a favourable option.

Needless to say we were more than a little apprehensive. Then we came across an online community for parents just like us, with kids dealing with eye conditions. After a year of no one really knowing what we were talking about, and constantly having to explain ourselves, we found others who knew exactly what we meant. Little Four Eyes is the name of the site, and finding it felt like someone holding out their hand to help us up. Finally, we found people speaking the same language, and what they were saying was that Intraocular implants were the way to go. An IOL is an artificial lens, but it’s attached to the eye itself, either with stitches or a clip. There are a couple of different types of lenses, and different ways of attaching them, but they’re permanent, and, if this type of surgery was a success, it would open up  a world of possibilities for our boy. We had a lengthy discussion with the PO, determined that this was how the surgery was going to go. She agreed and we planned accordingly.

I was still anxious about the surgery after this decision was made, but ultimately knew it was the right thing to do, and I really felt like we had done our homework. The surgery was booked, and we were committed.

In order to correctly fit the IOL, Josh needed to have an examination under a general anaesthetic, where the surgeon would measure his eye. We planned to include a minor procedure called an “Oblique Recession” on his left eye. His left eye “drifts” up a little when he’s using it, and this surgery would adjust the muscle so that the eye stays where it is supposed to. Today was the day.

It was day surgery, but he needed to fast from 7.30am. We arrived at the hospital at midday, and were told the surgery was scheduled for 3pm. We were advised that they had pulled the pin on the muscle procedure, but couldn’t tell us why. It was already shaping up to be a long day, and by 2.30, after 4 hours in the waiting room, the cracks were starting to appear. At 3, our PO arrived, she commented on Josh’s glasses, saying “I didn’t know he had glasses,” despite the fact that she had prescribed them – it was not a good start… By 4pm when they finally came to take us through to theatre, Josh had had enough, and he put up quite a fight with the anaesthetist. Sitting there holding him as he kicked and screamed and held his breath, I wondered what on Earth we had done. 45 minutes later and they were done. The team of surgeons, our PO, a registrar and the Professor overseeing it all, came to talk to us. The news wasn’t good.

They had found when they measured his eye, that it was small and flat, not a good fit for an IOL. Not only that, they simply don’t make them to the strength that Josh would need. So we are back to the contact lenses, right where we didn’t want to be. It was a disappointing setback, and the road ahead seems longer than it was 24 hours ago, but our boy has bounced back today and all he cares about is when we’re going to the playground! You can’t really ask for more than that.

From the Beginning

Just over a year ago, our little boy Joshua was diagnosed with a very rare eye condition known as Ectopia Lentis et Pupillae. His pupils hadn’t developed in the right shape, size and position and the muscles holding the lenses in place were dodgy, meaning the lenses were dislocated, or also not in the right place. He was also suffering from a whole lot of opias – myopia and amblyopia to be precise. Confused yet? So were we in the beginning. 

He was prescribed glasses and eye drops and daily patching to try and give him the best chance of normal eyesight. The Paediatric Ophthalmologist mentioned surgery at that very first visit, and we imagined it was years away, and something we “may” have to consider. Now 14 months later here we are, about to fly to Sydney for not one but two surgical procedures.

This blog is for family and friends, and friends we haven’t met yet, who want to join us on our journey to help save our little boy’s eyesight. For posts from the early days after diagnosis see http://typhoonsandtantrums.blogspot.hk/search/label/ELeP. Any future posts about Josh’s eyes and this condition will be posted here.

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