Picture description: Me standing on a pebble beach at sunset.
Known to my close friends as B – ambiguous, Gossip Girl vibes, I know, xoxo - I started experiencing vision loss last year, shortly after finishing my Master's degree in Global Media and Culture at the age of 23. Before we hit the heavy stuff, I should probably tell you a bit about myself; the person in the mysterious beach picture. I'm 24, British, a bit of a nerd (this may be an understatement) and very sarcastic/sassy (keep reading and you'll soon understand; dark humour is my unhealthy coping mechanism of choice). I love singing, writing, sunning myself and spending time with my family. Oh, and also apparently baking; thank you lockdown for giving me the time to learn how to make unhealthy snacks rather than buying them. I'm still all of these things post-diagnosis, but just with an extra helping of anxiety (who knew that was possible), and a double dose of sarcasm. Oh, and fatter, definitely fatter. But I can get away with blaming my treatment on steroids for that; the unhealthy bakes I’ve been making have played no role in this. None at all. Anyway, moving on... To the heavy stuff!
My vision loss journey started in October 2019. It began when I noticed a large spot just off my central vision in my left eye, which 'flashed' green when I blinked.
I knew this wasn't ‘normal’, and after turning to Doctor Google, I became terrified of the possibility that I was experiencing retinal detachment. So I made a request to see my optician urgently. The best way I could describe this change to my vision was by explaining how it resembled the temporary flash you are left with after a camera goes off in your face, or if you glance at the sun for a moment. Except, unlike those types of ‘flashes’ you get, this seemed to be permanent. Based on that alone, it's fair to say the optician didn't have much to go off. After lots of bright lights and my first experience of dilating drops (the absolute lie of a product 'No Tears' shampoo suddenly seemed like a luxury in comparison - if you know, you know), I was diagnosed with 'floaters'. Floaters are just fibres of the vitreous liquid that have broken away and 'float' (strange, that) about your eye. I was told they were harmless and could either go away on their own, or I'd get used to their presence and stop noticing them. I was also told that they don't 'interrupt' your vision, as you can see through them. Phew! I was relieved to hear that basically, I'd been a panicky drama queen for nothing. I was, however, warned to come back if I experienced any flashing lights or a shadow coming in over my vision as this could indicate retinal detachment. So whilst Doctor Google didn't offer a completely mismatched diagnosis given my symptoms, for now, I was okay.
Within a week I started getting a glittering light in the spot I had complained to my optician about; I call it my strobe, although it’s really not the party it sounds like. I also developed a streaking bright white light that travelled upwards from the bottom left hand corner of the affected eye. So back to the opticians I went, two days before my 24th birthday. More dilating drops, joy. More unbearable bright lights, joy. And this time photographs of the back of my eye, too. This appointment brought a bleaker prognosis. I was told that I had ‘lost pigment' from the back of my left eye, and sure enough, on the pictures you could see little white speckles where it appeared my retina was deteriorating. Confused, I asked what this meant and was told that I would eventually lose my central vision but that there was nothing that could be done. At most, I could try increasing my intake of anti-oxidant foods which may help slow down the process. Central vision is an integral part of eyesight; it's what you need to see fine detail, faces, and to read. I remember feeling overwhelmed, gutted and completely separated from real life.
After lots more visits to Doctor Google and scaring myself silly, two days later, on my 24th birthday (happy birthday to me, lol), I went to my actual GP to ask for a referral. Just in case there was something that could be done. As my optician notes hadn't been sent to my GP, he couldn't refer me. So after another phone call to my opticians (how I hadn't been blocked as a nuisance caller by this point, I do not know), they agreed to refer me. They did advise, however, that my case was not urgent as the condition was not treatable, and it could therefore take months to get an appointment. I was told that when I did eventually get an appointment, the hospital would simply tell me that nothing could be done.
After a few more weeks of flashing lights - and just to digress, let me tell you, this was confusing as hell. Christmas decoration euphoria was well upon us by this point and I honestly spent so many seconds glancing round thinking there was an exciting light show going on to my left, but nope, it was just my eye. When I wasn’t playing ‘festivities or eye disease’, I was freaking out that I was about to get hit side on by a car/bike/tram with its headlights on, but again, it was just my eyes. My brain was constantly rattled, and I frequently wonder what my heart rate spikes were during that time 😂 Anyway, the flashing was clearly worsening, as was my strobing spot. Luckily, my referral went through surprisingly quickly, and I was seen by my local eye clinic at the end of November. I had my first OCT scan (no complaints with this scan! Oh wait... actually... there was more dilating drops - YOU REALLY DO KNOW IF YOU KNOW) and a consultation the following day.
I was told that from my scan, my eyes looked 'normal'. I was so relieved. The opticians had gotten it wrong! I was going to be fine. That was until I was told they were "as normal as can be for someone of your prescription". For reference, at this point I was wearing a lens of a -6.5 prescription in my affected eye, and -7.00 in my 'good' eye. Given I had a pretty high prescription, I was told that the spots/flashing/strobing in my vision were to be expected, and were part of a process called 'pathological myopia'. Pathological myopia, to summarise, is where your eyesight is so bad it causes changes to the shape and health of your eye and can lead to vision loss. So yes, more good news. I was also diagnosed with PVD (changes to the gel in your eye which causes floaters) and astigmatism (where your eye is shaped like a rugby ball rather than a football; a strange analogy, but it's the go-to one). When I asked if I had macular degeneration - because that was the diagnosis that both Doctor Google and the optician had suggested - I was told they were the same thing.
All those feelings of relief quickly sunk away and devastation hit me like a tsunami. When I asked how long it would take to lose my central vision, I was told it could take anywhere between 6 - 40 years. I was gutted. This meant I could be without central vision by the time I turned 30. I couldn't imagine not being able to see my niece and nephew's faces, read books, study and drive. Would I be able to do my job? Would I still be able to live independently? Could I travel? How difficult was life going to become? I suddenly felt like my whole world was upside down, and I had no way of flipping it back round again. "Why are you upset?" the ophthalmologist asked. "It could take years". I really think he thought it would. But yet it didn't.
What should have taken six years minimum took six weeks. Every day my vision seemed to worsen. I developed more 'flashing' spots, and I couldn't see through them. Colour and detail was replaced with grey splodges of nothingness. They grew bigger and grouped together, like a string of fairy lights, but not as pretty, and rather than brightening the place, they made the world a lot darker. Gradually lines became wavy. Although I’d been told that the floaters wouldn't block my vision, suddenly, before I knew it, I couldn't read using that eye. In the weeks that came, I felt more and more depressed.
I went back to the opticians again after lines became wavy. My optician was concerned that when looking at an Amsler grid, there was both missing and distorted sections. He tried to test my vision, but I could barely make out the letters that had once been easy (with a corrected prescription). No amount of magnification could give me the vision I needed; there was just blank gaps instead of letters. I tried using my peripheral vision, but it was incredibly challenging.
I cried my eyes out the whole appointment. Bless that optician, he must have felt very uncomfortable whilst I sat there balling and sniffling, but he managed to stay professional; kudos to him.
Thankfully, it was the concern of this optician that got me on the right tracks for the correct diagnosis. Due to the waviness of the Amsler grid, he made the decision to refer me again, but this time for wet macular degeneration. Wet macular degeneration is treatable (in some cases). It differs from other forms of macular degeneration as the vision loss is caused by new blood vessel growth, so injections are required to treat it and preserve as much eyesight as possible.
The optician referred me to the ophthalmology department of my local hospital, rather than the local eye clinic where I had been sent previously. Frustratingly (but understandably), as it was just before Christmas, the appointment took four weeks rather than the usual two week wait for wet macular degeneration. During this period, I did attempt two more vision tests, just to see if I also needed stronger contact lenses/glasses to help. My prescription was updated and helped my vision somewhat, but naturally, it couldn't fill in the missing blanks.
Fast forward to mid-January, and I was prepping for my appointment at my local hospital. After everything, I was confident that they would tell me there was nothing that could be done. To say I felt depressed and unhopeful is a generous understatement. To my surprise, the ophthalmologist prescribed me high dose steroids to start immediately, informing me that they were necessary as my eye was inflamed. Despite the progress and proposed treatment plan, I felt annoyed and frustrated. I had been told something different again, so I felt like no one really knew what was going on. So yep, cue more tears, but this time over hot chocolate in the hospital café and to my dad, not some poor, unsuspecting optician. It’s possible a few of the hospital’s café - goers felt uncomfortable by my outburst, but what with the tears and snot, I can’t say I noticed.
Although I initially felt discouraged, I quickly changed my tune when the steroids started working and I regained some vision 😂 Within a week, I could read again using that eye. On the way back from my masters graduation, I told my dad how I could see the number plate on the car in front of us. I felt like I gained two achievements that day; the latter felt like the bigger triumph. My vision still had progress to make, but the fact that there had been a big central blind spot that cleared up so quickly and enabled me to read again, completely shocked me. I felt incredibly grateful to the ophthalmology team at my local hospital; they had given me back pieces of vision when I was so certain it was gone forever. I could feel little oozes of happiness seeping in.
It wasn't until my second appointment two weeks later that I asked my ophthalmologist to write down my diagnosis; Punctate Inner Choroidopathy (PIC), an inflammatory/autoimmune disease. The inflammation, untreated for so long, had caused scars on my retina which resulted in blind spots. To try and reduce the scarring, I was treated with steroids until mid April. In all honesty, results slowed down after first few weeks. My blind spots still changed; they continued to shrink and flatten, but the vision regain seemed less significant as each week passed. But, improvements were still being made, and for that I was grateful.
By the end of treatment I was left with six blind spots, spots which are likely to be a permanent smattering on my vision. I have four going diagonally across my central vision, and two big ones just to the left. Luckily, as the steroids worked really well (despite the permanent damage, they really did get me so much vision back), the way that the diagonal central spots sit leaves enough space to see letters between the scars. It means I can read using that eye. Well, both eyes 😂 Individually and together! Post treatment I have also been left with a difference in colour perception between my two eyes. My 'bad' eye tints everything white or cream with a pink hue. This won't be good if I get sunburnt; I guess I won't know what shade of lobster I really am. (Also side note - pls wear SPF to avoid becoming said lobster).
But of course, the drama doesn't end there though, because why would a tale of an autoimmune disease come to such a quick, happy ending? Naturally, we need more plot twists. And cue...
When I finally came off the steroids in April, I believe I had a flare up in three days. In 72 hours, my large blind spot to the left of my vision had darkened from grey to black and seemed bigger. So back to the hospital I went. Yet the scans showed no changes at all. I was baffled; was it all in my head? Was it just anxiety? I was certain it was worse. So, I came away with nothing more than a personal mental note to just keep monitoring it.
Four weeks later I went back to the hospital as my vision was constantly blurry, the waviness had increased, and I was absolutely certain that my left blind spot was bigger and darker still. This time, my scans showed that the scar I was referring to had flared, and had done so quite significantly. The ophthalmologist described my scar as 'fluffy', which may sound cute, but in PIC, it's a real pain. We want fluffy animals, not fluffy PIC! So, in the midst of the pandemic, despite the risks, we decided that high dose steroids would be the best course of action to prevent risking vision loss, and I was given strict instructions to shield.
However, I heard on the grapevine (thank you PIC community) that it was possible to have a steroid implant in my eye rather than taking daily tablets. As it turned out, my local hospital does not do the procedure, but as I was waiting on a consultation at another hospital, one which specialised in PIC, I contacted them and asked if there was any way we could fast-track my appointment and see whether I would be suitable for the implant. I also told them how I worried I had become for my 'good eye', because I was absolutely certain that it was developing PIC too. My local hospital felt that this was not a concern as it wasn't showing on the scans, but my fear was only intensified knowing that it has taken a month for the flare up in my bad eye to show on the OCT. I knew that I could see distortions even though it wasn't showing on the scan.
Amazingly, the specialist hospital agreed to see me within a week as they felt my case was urgent. After lots of scans, bright lights and incredibly, no dilating drops (I already like this hospital!!), the team felt that my scans were showing a significant flare, despite being on high dose steroids for over a week. The word ‘fluffy’ was used to describe my PIC again, but the nurse also showed me pictures of his new puppy later on in the appointment, so I suppose I kind of got the fluffy animals I wished for?
The specialist team, who have been incredible, presented a new treatment plan on top of my oral steroids. This consists of long-term immunosuppressant medication to prevent future flares and protect my good eye, an Ozurdex (steroid) implant in my bad eye, and a three month course of four-weekly Eyelea injections in my PIC eye as well, to treat the new blood vessel growth they found (choroidal neovascular membrane/CNV).
The injections should – hopefully - reduce the waviness of my vision. Importantly, whilst they agreed with my local hospital that my good eye wasn't showing anything concerning on the scans, upon physical examination, they did say that something was 'not quite right', although it was difficult to determine for certain. They felt that I may well be noticing a very, very early flare up, hence the decision to start immunosuppressants and stay on oral steroids too. Honestly, I was speechless. It's a lot of medication and treatment, but if it helps, I'll gladly take it.
So that brings us to the end of the drama and my journey so far... For now anyway... let's not underestimate the unpredictability of autoimmune diseases 😂 At present, I still have blind spots in my left eye and a mild distortion in my 'good eye', I'm still in the process of tapering off my oral steroids, slowly but surely, and my immunosuppressant medication should soon be at a high enough level that it starts to kick in. My implant will at its peak function in the next week or so, so pressure checks are due soon, as is another routine blood test and my next injection to fight the unwanted blood vessel growth.
I don’t know what the future may hold for me or how much vision loss I may experience, but in the meantime, I wanted to make this blog to raise awareness of this rare disease. I believe that if more people know about it (both medical and non-medical persons), then diagnosis may be quicker and vision loss may still be reversible, or at least perhaps less severe. Although I joke about my experiences, I have really struggled through the effects of this condition and I wouldn’t wish it on anyone. Vision loss is hard. On the days I find myself able to get off the couch, I try my best to make light of the situation, and sometimes that means laughing at my own experiences. I’m just trying to see the Big PICture... Yes, that was a shameless pun...
On a more serious, final note, PIC should not be underestimated as a disease. It needs treating immediately but that doesn’t happen for everyone. It is so rare that patients are often diagnosed with other eye diseases or conditions. For that reason, I do not have any resentment for the misdiagnoses I received, and a lot of eye diseases have very similar symptoms. I am very grateful to the ophthalmologists who have/are treating me, and for restoring a lot of vision. I am, though, really keen to help others with PIC get a faster diagnosis and I believe that awareness is a key step in this process. I really hope I can achieve this from the work I have planned.
On here and on my Instagram, I’ll be sharing my day to day life with PIC - the good, bad *and* the ugly (and yes, this will no doubt include more awkward crying scenarios, you lucky readers) - all in the hope that speaking up may help make a difference to someone else. Be this someone who already has PIC or vision loss from another disease, or someone who could lose vision in the future.
If you have any questions about my journey so far, feel able to share your journey with me or have a preference for the things you'd like to see on my account, please let me know in the comments below 💕
I look forward to sharing my journey with you and raising awareness together,
B x