Trying to be 'normal'

Thanks to our happy checkup at Moorfields on Thursday, I was able to take Aaron to a Halloween party with our NCT friends. It was so nice to do something 'normal' and spend an afternoon just watching the babies play and having nice food and coffee (being able to finish a hot beverage is such a luxury). I did have to give Aaron his drops at one point but one of our friends kindly helped me hold him and even sang to him whilst I tried to aim the tiny dropper at Aaron's squished up squirming face! We've definitely survived the last week with lots of help from friends, everything from prayers sent from afar to wonderful people giving us wine, flowers, chocolates, homemade goodies. Our families have also been great, calling, texting and Face Timing frequently to check on us!

Aaron continues to be a trooper through it all and his little eyes are tracking and following everything. He still tends to look down a lot but the orthoptist thought this was more due to his squint than the glaucoma so I'm not sure whether we'll see a change in that until we get through the December exam. He also is still sensitive to light sometimes, we can't bath him with the overhead light on (it is rather bright) and when he lays on his back he keeps his eyes shut. But he opens them a lot more than he used to.

His physical development seems to be carrying on as it should (I think??), he's crawling backwards, doing pushups and starting to bang around more in the jumperoo vs. just playing with the toys. The two areas where he's struggling are sleeping and eating. The sleeping is a nightmare (no pun intended) - he's napping more in the day now, which is good (and isn't enough that it should affect nights), but he won't settle after his night feeds and can scream for up to two hours before going back down. Last night Adam had to bring him downstairs at 4 am and the little monkey fell asleep on the couch.

Eating is improving a bit in that he's drinking his milk feeds properly again but feeding him solids is a bit of work. Before the surgery he would eat off a spoon without so much messing about, now he wants to lick what's on his hands, his bib, etc. and he is less interested in the spoon. A complete clothing change is usually necessary afterwards. However, I don't know that all this has anything to do with the glaucoma - I think he's just being a baby!

Our little pumpkin

Crawling under the kitchen table this morning

Good checkup at Moorfields today

We had a really positive meeting with our doctor at Moorfields today regarding the status of Aaron's congenital glaucoma! Adam and I had thought the cloudiness was clearing in Aaron's corneas but were worried we were being too optimistic; however, the doctor confirmed our diagnosis. We couldn't have asked for better news at this stage, super happy with these results after the roller coaster of emotions the past week.

We must have looked like something the cat dragged in when we turned up for our appointment at the Richard Desmond Children's Eye Centre, as Aaron was up and down all night last night. Not sure what was keeping him up but Adam and I had to pull every trick in the book to get Aaron back to sleep, including Ewan the Sheep (had to dig him out of the closet) and the bloody hairdryer as well - three times (I'm guessing we won't be getting a Christmas card from the people next door this year). He kept trying to rub his eyes so I think it's fair to say that us pinning him down didn't exactly soothe him back to sleep.

Our visit to Moorfields started with a new gentleman calling for us instead of our usual doctor but he was quick to explain that he was working closely with our doctor and would be part of Team Aaron - the more the merrier! He knew everything about our case and went through all of Aaron's prescriptions with us, answered questions and had a look at Aaron's eyes (Aaron was peering out from the folds of my shirt as he'd just woken from a nap but was very keen to be part of the action). The new doctor liked what he saw in terms of the cloudiness being reduced and was kind enough to take a special look at Aaron's left eye, which we managed to get pears in during breakfast this morning and I was sure it was going to blind him worse than the glaucoma (he's fine).

The new doctor also told us why Aaron's probably rubbing his eyes - the sutures are starting to dissolve. It's totally normal and there are enough sutures that restless babies can't undo hours of surgical precision with one fell swoop.

Our usual doctor joined us in the room at that point and echoed the happy diagnosis, the corneas were clearing which means the 360 degree trabeculectomy is working! He explained that our next steps for treating the congenital glaucoma would include weaning Aaron from the drops he is on (an antibiotic and a pressure reducer) followed by an in-depth eye exam, which must be done under general anaesthesia, where they will look at Aaron's pupils, optic nerve, the healing from the sutures and the pressure in the eye.

If the pressure is down once the drops have been discontinued, we will then see the orthoptist about any necessary patching, glasses, etc.

If the pressure is still high, they will put Aaron back on some form of pressure-reducing drops and wait until he's a bit bigger before performing another surgery that will involve a tube implant to help with the drainage. The doctor told us that if the pressure is high and the optic nerve is in good shape, we'll wait a bit longer as it's better if Aaron has a chance to grow before getting the implant. However, if the optic nerve is not so great they will do the surgery quicker.

It's worth noting that in no case will we be riding off into the sunset with no further visits regardless of the December outcome (I sort of knew that but I asked anyway just in case). Aaron will need monitored for a long time (forever?) to make sure the pressure stays down.

All of the news today was positive but we were particularly excited to hear that we don't have to pin Aaron down every time he rubs his eyes. Here's to hopefully a good night's sleep!

Improvement?!

Our little patient's red eyes are starting to look better 4-5 days on from the 360 degree trabeculectomy. Over the weekend Aaron's eyes were dark, blood red and now he looks a bit like he's been swimming in chlorine without goggles. In his irises you can see little flecks in his eyes like most people have, hopefully because the cloudiness in his corneas is clearing? (Our unofficial observations, the poor kid must be sick of us staring at him like a specimen)

The ibuprofen seems to have really helped as he was engaged in play most of yesterday, crawling backwards, chewing and just watching the world around him. Lots of squeals and giggles towards evening and we managed to get him down at 7 pm in his cot, so almost his normal bedtime routine. No bath as we'd had to give him one in the morning (strawberries for breakfast ended up everywhere) but he got his usual songs and stories, which knocked him out till midnight and then he fed and slept until 05:30!!

A banner night for Aaron on any given day, let alone less than a week after major surgery. I don't know if it's been his congenital glaucoma that's made him a sometimes night owl so far or it's just his nature but either way we were ecstatic to get more than two hours of consecutive sleep. Happy days. :)

Up and down

Another long night here with Aaron waking every 1-1.5 hours. His cries sound upset vs grizzly so for the time being we have stopped any sleep training and are picking him up when he cries.

This morning around 7:30 the cries turned to little coos and I was excited to see a big smile when I lifted him over my head. However, he kept his eyes snapped shut tight like he used to do. I changed him and brought him downstairs for both sets of drops, which he yelled through. He kept yelling after the drops so I put him in the jumperoo, where he played for a few minutes with his eyes partially open. He didn't seem sensitive to the light, it just looked like his eyes were sore and he was protective of them.

Playtime was short lived, he took an impromptu nap that ended as quickly as it started and then was ready for business! He spent an hour or so on his foam mat playing with his toys and creeping around like a little lizard. His eyes were open - the redness is going down just a bit - and he seemed alert and happy. So nice to see him happy and seeming comfortable!

He was back in upset mode by mid morning when a lady from Moorfields called to check on him - she could hear him down the phone. She wanted to know how we were managing Aaron's pain and recommended adding ibuprofen alongside the paracetamol we were already giving him. She said he would be suffering for potentially 7-10 days post surgery given the magnitude of the operation and that he had both eyes done. It seems his behaviour on Saturday where he was so chilled and happy most of the day was a fluke - maybe he was over excited with his new vision, who knows, but it coloured Adam's and my expectations and we were feeling thrown by the unhappy camper we saw on Sunday. It was good to hear Aaron was doing ok although it's not great to know he's suffering.

Lots of naps today and another good stretch of playtime with his eyes open, and of course I'm following the advice on his pain meds. Fingers crossed he continues to improve and that we have nipped this congenital glaucoma in the bud. We go back to the clinic at Moorfields Richard Desmond Children's Eye Centre on Thursday, really hoping for good news in terms of the eye pressure being down, cloudiness disappearing and overall healthy healing of Aaron's precious eyes.

Full of beans again

The second day following the 360 degree trabeculectomy

Last night was slightly better. Aaron got up at 11 pm and 3 am and then was up and down from 3 am but at least we got four hours of sleep?! He seemed to be teething, he pulled off and cried during nursing and was touching the back of his head. He wasn't rubbing his eyes and when anything else is wrong with him he wants to nurse continuously hence why we think it's teeth but we're keeping a watch over him today.

He's behaving now more like we thought he would yesterday - mostly grumpy, very tired and napping pretty much continuously. He's woken up to play a few times (including right now) but he's definitely more subdued and seems to want to rest. I think that's reasonable given he's just had surgery? I'm trying not to think the worst (the pressure's back up, his eyes are hurting him, etc, etc). Haven't seen much of his eyes today but they were still red this morning when I checked them. The doctor said that would last for a few days. Fingers crossed for continued recovery and that the squealing and crowing returns soon!

The first night and the day after the 360 degree trabeculectomy

We'd been told Aaron would have anaesthetic still in his system following his 360 degree trabeculectomy for congenital glaucoma and so would sleep a lot Friday night and Saturday. Instead he was up and down all night wanting milk and actually got up and played for an hour around 2 am! Saturday morning he was grumpy and then all of a sudden he seemed to wake up, cooing and screeching, taking everything in and talking and grinning away. We were amazed, he was like a different kid, so happy and wide eyed vs fussy and hiding his eyes like we'd been seeing lately. He had a little nap in the afternoon but otherwise played until 9 pm, even showing off a bit for some friends who'd popped round to see him. We were so proud of our little man and felt so lucky, but also a little sad that he'd clearly not been able to see very well for so long.

In terms of his eyes themselves, we hadn't had to use the shields as he seemed to understand that rubbing was a bad idea! He was a good sport about all the drops we had to give him (antibiotics 4x per day, pressure reducer 2x per day - he yells but doesn't cry) and didn't seem to mind that the whites of his eyes were blood red. The blue irises seem less cloudy but the contrast against the red might be skewing our perception...

playing with Daddy

Surgery day - we made it through the trabeculectomy

We were super lucky in that the onsite hostel at Moorfields, the Ronald McDonald House, had space for us to stay. It was so helpful as we were completely wiped after Thursday's appointments and it would have been stressful for Aaron to make the long journey back that night only to have to repeat it again the next morning.

The volunteers at RMH were very kind and had a travel cot set up for Aaron so that he would be comfortable. Nice beds for mummy and daddy too. Aaron ate like a piggy overnight  so hopefully he got his strength up.

We tried our best to keep things 'normal' for Aaron in the morning, playing for a few hours and being silly until it was time for his last feed before surgery (no milk after 9:30 am). The last feed worked well with nap time so Adam and I took him for a walk to J+A Cafe in Clerkenwell before heading into the day center for noon.

Aaron was starving by the time we went in and not in the best mood. Stripping and weighing him did not please him either so he had a full on meltdown with plenty of raspberry blowing, poor kid. I'm sure he was wondering why we wouldn't feed him! Rocking and singing finally knocked him out (I hope everyone else on the floor enjoyed Rockabye Baby and Wind the Bobbin Up 20 times. I've been told in the past I have a singing voice like the seagull from Little Mermaid).

Meanwhile we had a visit from the surgeon to explain the procedure, which would kick off with an eye exam under anaesthesia to find out if there were any other issues. All being well the 360 degree trabeculectomy could proceed from there.

About 1:45 PM the nurse came in to give him a gentle sedative so that they could get the IV in and he went into the theatre about 2 pm. Adam went with him to hold him while they gave him the IV and he fell asleep.

No matter how good you know the surgeon is, the moment you hand over your baby is heart wrenching so we headed to the canteen for distraction.

We had to laugh at the radio station that was on in the cafe as it kept playing 'romance gone wrong' songs. Haha. We didn't linger long, one can only handle so much Michael Bolton even on a good day.

We didn't need to wait long once back upstairs and the surgeon came out for an update. Poor Adam had gone to the loo so I took copious notes accompanied with drawings (I'm sure the surgeon thinks I'm bonkers). Everything with the surgery went well! Hurrah! He was able to get all the way through the Schlemm's canals in both eyes, so a full 360 degree trabeculectomy, which isn't always easy as babies Aaron's age don't always have the canals properly formed. We are probably lucky he didn't get referred any earlier I suppose. All the prayers and good wishes worked as we couldn't hope for better news at this stage. 

The pressure in Aaron's eyes was already dropping and hopefully the corneas will clear enough in a few weeks that we can get a look at the optic nerve to assess its condition, although we've been told damage is still reversible at this age.

I got to go in and get Aaron once he woke up and bring him back to his room for a feed. He just wanted cuddles and a nap at first but soon had a marathon milk fest - that's our boy!

The surgeon came back in for a check before discharge and was happy with the condition of Aaron's eyes. The bandages and eye shields came off before we even left and could stay off as long as Aaron didn't rub. 

Even though it was late Friday afternoon/early evening, the nurses never rushed us and let us take as long as we needed to collect our things. The nurse who did our discharge even fed us, gave us juice and helped us out to the taxi with all our bags! Such wonderful people at the Richard Desmond Children's Eye Centre at Moorfields, we couldn't have asked for a more caring, professional team to look after our sweet baby.

All in all a good day and the best we could hope for this first critical leg in the journey. 

Exam day at Moorfields Richard Desmond Children's Eye Centre

Typing from the iPhone but wanted to share that we had a productive day of eye exams, meeting with an orthoptist, the surgeon, a nurse and having an eye ultrasound as well. Very happy with our experience at Moorfields so far and we've met some lovely, very supportive folks.

Aaron was a champ for all of it and only got a little fussy when it was nap time (oh and he ate twice over the course of the 3.5 hour visit).

I will post more later once I'm back near a computer but we are definitely on for surgery tomorrow and are due to arrive at noon. The surgeon will perform a 360 degree trabeculotomy, which essentially is opening the entire Schlemm's canal vs just part of it as you would in a standard trabeculotomy. Success rates are 80% so fingers crossed.

We are keeping our positive thoughts flowing tonight! To kick off our happy thoughts we went out for pizza and beer with Aaron to Homeslice on Old Street and had a half chorizo/corn/coriander half mushroom/pumpkin/ricotta pizza and two pints of Camden Town lager. Heaven.

Off to bed now (yes it's 19:30 but I feel like it should be next Tuesday we've had such a crazy day)...

Happy holding pattern

Not much to report at the minute as we are waiting for the appointment at Moorfields on Thursday to find out next steps regarding Aaron's congenital glaucoma treatment.

On the one hand it's hard to wait - we want to know what the future holds for our little man. On the other hand, we are enjoying these few days with Aaron as we're anticipating at the very least he may be kind of uncomfortable in a week from now. We don't know exactly what to expect after Thursday's appointment, only that we've been asked to plan to stay overnight as surgery on Friday is very likely.

We don't know what the surgery would be but from the bit of research I've done it could be a goniotomy or a trabeculectomy. We received a hard copy of Aaron's referral letter from the PRUH to Moorfields and it has his corneal diameters measuring at 11mm (left) and 12mm (right) so based on that alone (and bearing in mind I am not an opthamologist and one week ago had never even heard of congenital glaucoma) it looks probable that it would be a goniotomy.

But who knows! Given the surprise of this diagnosis (as Clark Griswold once said, if I woke up tomorrow with my head sewn to the carpet I wouldn't be more surprised than I am right now) anything goes I suppose. We are going with the flow for now, ignorance is bliss and we'll enjoy our happy holding pattern.

This is how you give an infant eye drops

Aaron is on two types of drops to control his congenital glaucoma before surgery and we had tried a variety of methods to get them into his eyes including:

• Holding him like a newborn and leaning his head back (he pinched his eyes so tight we'd have needed a vice to get in there)
• Putting him over the shoulder and bending over (see above)
• Putting him in the jumperoo, tipping the jumperoo upside down and one of us distracted him with the toys while the other squirted drops (this was mildly effective but hit and miss)

It's tricky enough trying to get him the drops spaced 12 hours apart as his sleep is so erratic right now. Couple that with the fact that Adam can't always get home from work before Aaron's bedtime (and none of the above methods can be attempted solo, at least not in our house) and we were desperate.

I did a bit of searching and found the most helpful video from Moorfields for giving eye drops to infants or young children:

https://www.youtube.com/watch?v=d3wtEWX7HxU

I went with the method of putting him between my legs, sitting on his arms, squeezing the drops into the corner of his eye and then gently working his eyelids until the drops go in. Pleasant it's not but there's less screaming each time and it's been 100% effective. Fingers crossed that these drops help!

Follow up visit at the PRUH: the eye clinic

Adam had taken the day off work and we turned up absurdly early for the eye clinic at the PRUH. We were seen promptly and the specialist very gently did a series of tests. He quickly diagnosed that little Aaron indeed had high pressure in his eyes and clouded corneas, at which point I said 'glaucoma' and he said 'yes'.  Hopefully I'm paraphrasing correctly but he compared Aaron's congenital glaucoma to a tap and drain, basically the tap is running and the water isn't draining so his cornea is waterlogged - hence the cloudiness.

No one in our immediate family has glaucoma or has had any childhood eye issues that we know of. The odds of having congenital glaucoma are 1 in 10,000! So rare that even though it might have been present at birth and even though I'd been badgering health visitors about Aaron's squint for months, no one would have been looking for it. Rather unfortunate because the pressure on the optic nerve causes damage that can't be undone.

The specialist was quick to point out that there were treatment options, more so for babies with glaucoma than for an adult because a baby's eyes are still stretchy. He was incredibly kind with all of our questions:

• yes, surgery was probably the best option but this would be left to the doctor we'd be referred to at Moorfields
• no, Aaron isn't blind and if we treat this now probably won't lose all his vision
• no, it shouldn't affect his brain functions 
• yes, he may need really, really thick glasses 
• no, we don't know yet how independently he'll be able to function - in some cases the surgery can fix things almost completely, it all depends on how much damage there already is
• no, the congenital glaucoma doesn't hurt so to speak but it probably makes Aaron restless and exhausted

The next step was the referral to Moorfields Eye Hospital and we were given a prescription for drops whilst we were still at the PRUH so that we could start to reduce the pressure in Aaron's eyes before our appointment at Moorfields. We left the PRUH grateful that we could start treating Aaron right away but wondering how on earth to get eye drops in the eyes of a busy, strong-willed five month old?!

The first screening

Adam, Aaron and I had just spent two beautiful weeks visiting family and friends in the US and so Aaron and I were a bit jet lagged when we went to the PRUH for what was meant to be the screening to check out his squint.

The poor kid also had a cold from travelling and was sound asleep to boot when we arrived at the PRUH, so it took a while for the orthoptist to get a look at his eyes. I told her about noticing the squint in photos and that a few of my cousins have had squints. The orthoptist told me how misleading photos can be in diagnosing a squint and that it's really only immediate family members having a squint - not twice or three times removed cousins - that would indicate Aaron was genetically predisposed. At that point, I thought maybe I had overreacted by bringing him in.

However, once Aaron opened his eyes and engaged with the tests, the orthoptist immediately said 'This isn't what I'd expect for his age' (5.5 months). She did a few more tests and wanted to bring in a specialist for a second opinion. The specialist ended up being gone for the day but another doctor, one from Great Ormond Street and so very experienced with children, was available and she joined us in the exam room. The two doctors looked at Aaron's eyes and spoke back in forth in medical terms but I was able to pick up a few things: Aaron was very sensitive to light and his cornea looked cloudy - things Adam and I had noticed but hadn't realised were symptoms of possible serious problems.

They released us with a promise to get Aaron back in immediately to see the specialist they'd been trying to find earlier. The NHS isn't big on preventative maintenance so the sense of urgency was an instant red flag.

Naturally I couldn't resist having a look on Google once we left. I know, I know searching online usually leads to descriptions of rare diseases featured on web sites you wouldn't trust to diagnose dandruff let alone vision problems but the Type A personality I am needed to get at least a sense of what we might be dealing with.

However, on this occasion the search was useful. I learned that sensitivity to light is caused by pressure in the eyes and this eventually led me to congenital glaucoma. The results were all from sites like www.glaucoma.org and congenital glaucoma was pretty much the only thing coming up that could be the problem. The disease sounded scary, rare (1 in 10,000 babies are affected) and treatable mostly by surgery.

Meanwhile, within 10 minutes of leaving the PRUH (!) I got the call that we needed to come back the next day for an eye clinic with the specialist. Friends and family advised me to stay positive and avoid assuming my Dr. Google diagnosis was accurate.

Dinner at our house that night was subdued but we thought there must surely be some other reason for Aaron's symptoms - the rarity of congenital glaucoma made it seem too implausible. All we could do was wait for the clinic.

Why start this blog?

My husband Adam and I are 37-year old first-time parents to one adorable infant son called Aaron. Despite being born via emergency c-section this past May, Aaron has been reasonably healthy the first five months of his life.

When Aaron was about two months old I noticed he had a squint (lazy eye) in some of his photos and upon closer attention, could see it in person on occasion. Sometimes if something was particularly exciting, both eyes would roll around as if his mind was blown with amazement.

I raised it with the health visitor in July who promised to refer us to an orthoptist. It took three months and multiple chases to finally get the screening appointment scheduled and along the way Adam and I noticed some other things about Aaron's vision that were cast off as arbitrary, things like:

• Cloudy eye (diagnosed over the phone to the GP as conjunctivitis as Aaron poked himself in the eye)
• Occasional watery, red eyes (they look like allergies, the poor kid gets a rash if he spends too much time outside in a light breeze)
• Eye rubbing/eyes frequently closed even during activities like baths, changing, etc. (we assumed he was tired - most days we're lucky to get him to take three 30 minute naps)
• Dislike of bright lights/sunlight (I myself want sunglasses even if it's cloudy, so...)

I knew squint if untreated could cause some vision problems and though we weren't taking the list above as serious symptoms, I was eager to get Aaron seen by professionals.

Fast forward to the screening appointment in October, where I walked in with Aaron expecting recommendations for patching, glasses and exercises. Instead I walked out with more questions than answers, a rising suspicion that Aaron's condition was the incredibly rare congenital glaucoma and another appointment to take place the next day.

To answer the question 'why start this blog'...

It's for our friends and family who want to share in the journey as we work to help our baby boy live with congenital glaucoma.

It's for all other parents, who hopefully after reading this will believe even more firmly in their instincts as moms and dads and not hesitate to raise things or push the issues with health professionals, even if they feel silly. I wish now I had pushed even harder to get Aaron's appointment and maybe if I'd mentioned any of the above we'd have been seen in August vs. October.

And it's for other parents of children with congenital glaucoma, I hope they will take comfort that they are not alone in dealing with this horrible but treatable disease. We've struggled to find much online to read or many support groups given how rare congenital glaucoma seems to be.

Our journey has only just begun. I have no idea where it will lead or how much damage has been done but Adam, Aaron and I are taking it one day at a time and keeping the most positive outlook we can until it doesn't make sense to do so.

Thanks for reading.