The story of Beth
My sight loss journey started in October 2019, when I was twenty-three years old. It began with a large spot just off my central vision in my left eye, which ‘flashed’ green when I blinked. The best way to describe this change to my vision is to compare it to the temporary flash you are left with after a camera goes off, or if you glance at the sun for a moment. Except, unlike those types of ‘flashes’ you get, this seemed to be permanent.
I knew this wasn’t ‘normal’, and a quick Google search suggested that it could be a sign of retinal detachment – a medical emergency. So I went to the opticians as a matter of urgency.
The optician dilated my pupils, as this helps them to see more of the retina (the back of the eye), but there was nothing untoward to be seen, and no evidence that I was experiencing a retinal detachment.
For my symptoms, I was diagnosed with ‘floaters’. Floaters are just fibres of the vitreous liquid (a natural part of the eye structure) that have broken away and drift about your eye. It is a common condition – harmless – and one that the brain often adapts to so that the affected person stops noticing the fibres. Importantly, floaters don’t ‘interrupt’ vision; you can see through them.
I was, however, warned to come back if I experienced any flashing lights or a shadow coming in over my vision, as these are serious signs that indicate retinal detachment. For the moment however, I was okay.
Within a week I started getting a shimmering in the spot I had complained to my optician about. I called it my ‘strobe’, as the frequent, quick-paced flashing simulated that of a strobe light. On other occasions this spot would glitter, and sometimes, it wasn’t noticeable.
Soon after the strobe appeared, I 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 were used, as were more unbearable bright lights, but this time, photographs of the back of my eye were taken too.
This appointment brought a bleaker prognosis. I was told that I had ‘lost pigment’ from the back of my left eye, which presented on the photographs as small white speckles.
Confused, I asked the optician what this meant and I was told that the condition of my retina was deteriorating, so I would eventually lose my central vision. I was informed that there was nothing that could be done to prevent it. At most, I could try increasing my intake of antioxidant foods to attempt to slow down the process, but this wasn’t guaranteed. I was devastated. Central vision is an integral part of eyesight; it’s what you need to see fine detail, faces, and to read. I couldn’t imagine what it would be like to lose that vision.
Two days later, on my 24th birthday, I went to my GP to ask for a referral to the hospital, just in case the specialists could find something to help me. Frustratingly, my optician notes hadn’t been sent to my GP, so he couldn’t refer me. Desperate, I called my optician again and they agreed to refer me to the hospital themselves. However, the optician advised that my case was not urgent due to the untreatable nature of my condition. It could therefore take months to get an Ophthalmology appointment. I was then told that when I did eventually get an appointment, the hospital would simply tell me that nothing could be done.
Over the next few weeks my symptoms worsened, but thankfully, my referral appointment came through surprisingly quick.
At the end of November, I had my first OCT – an ‘Optical Coherence Tomography’ scan. This technology uses light to view the retina in micro detail. I returned to the hospital the following day for my results.
At consultation, 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. Until I was told they were “as normal as can be for someone of your prescription”…
I have a high prescription (known as high myopia), which means I cannot see things at distance without correction from contact lenses or glasses. Any prescription greater than -6.00 is classed as ‘high’ myopia and increases the risk of eye complications in the affected individual.
Given my strong prescription, I was told that the spots/flashing/strobing in my vision were to be expected as part of a deterioration process called ‘pathological myopia’. Pathological myopia, in short, is where your eyesight is so severe that natural changes occur to the shape and health of your eye which can eventually lead to sight loss. I was also diagnosed with PVD – a change to the gel in the eye, resulting in floaters – and astigmatism, where your eye is shaped like a rugby ball rather than a football. This stretched shape causes visual blurring which needs correcting with glasses/contacts.
The feeling 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 six and forty years. I was gutted. This meant I could be without central vision by the time I turned thirty. I couldn’t imagine not being able to see my niece and nephew’s faces, read books, study, 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 it didn’t.
What should have taken six years minimum took only six weeks. Every day the quality of my vision worsened. I developed more ‘flashing’ spots, and I couldn’t see through them. Colour and detail were 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. 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. The optician was concerned that I was experiencing both missing and distorted sections to my visual field; to experience both of these symptoms together is unusual. He tried to test my vision, but I could barely make out the large letters; letters that had once been easy to read. No amount of magnification could give me the vision I needed; there were simply blank gaps where letters should be. I tried to use my peripheral vision, but it was incredibly challenging. I cried my eyes out the whole appointment.
Thankfully, it was the concern of this optician that got me on the right track for the correct diagnosis. Due to the distortions I was seeing, 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, rather than pigment deterioration. Injections are used 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 felt certain that nothing 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. I felt like no one really knew what was going on with my sight.
Although I initially felt discouraged, I quickly came around when the steroids started working and I regained some vision. Within a week, I could read again using my left eye. On the way back from my Master’s graduation, I told my dad how I could see the number plate on the car in front of us with my left eye. I experienced two achievements that day, but the latter felt like a bigger triumph. My vision still had progress to make, but the fact that a large central blind spot had cleared up so quickly and enabled me to read again filled me with hope. The ophthalmology team at my local hospital had given me back pieces of sight when I was so sure it was gone forever.
It wasn’t until my second appointment two weeks later that I asked my ophthalmologist to write down my diagnosis; Punctate Inner Choroidopathy or ‘PIC’, a rare, inflammatory autoimmune disease. My immune system had attacked my eye and caused inflammation. This inflammation, untreated for so long, had caused scars on my retina which resulted in blind spots. This was the ‘damaged pigment’ that the ophthalmologist’s predecessors had seen on my scans. To try and reduce the scarring, I was treated with steroids until mid April.
The visual improvement slowed down after the first few weeks. My blind spots still changed; they continued to shrink and flatten, but the visual changes seemed less significant as each week passed. But, improvements were still being made, and for that I was, and still am, very grateful.
By the end of treatment I was left with six blind spots; a permanent smattering on my vision. Four sat diagonally across my central vision, with two larger ones just to the left of my sight line. Luckily, the steroids worked so well that the diagonal central scars had reduced down enough that there was space between each scar that I could look through to read. The disease also left a slight difference in colour perception between my two eyes; the ‘bad’ eye tints everything, often with a pink or blue hue.
When I came off steroids in April 2020, 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 larger. I went back to the hospital urgently, but the scans did not show any changes – my eye looked stable. 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 mental note to simply monitor things.
Four weeks later I went back to the hospital for a second time as I was experiencing constant and worsening blurring. The waviness had also increased, and I was certain that my left blind spot had grown larger still. This time, my scans showed that the scar I was referring to had flared, and quite significantly. So, in the midst of the pandemic, we decided that high dose steroids would be the best course of action to prevent risking vision loss. Covid was rampant though, and steroid tablets suppress the immune system, so with the prescription of Prednisone came strict instructions to shield.
However, I heard from the PIC community that as an alternative to oral medication, it was possible to have a steroid implant injected into my eye to deliver the anti-inflammatories locally. It would mean I wouldn’t need to shield. Unfortunately, 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 to see if I would be suitable for the implant.
Amazingly, the specialist hospital agreed to see me within a week as they felt my case was urgent. After lots more scans and bright lights, the team confirmed that I was still in a significant flare, despite being on high dose steroids for over a week.
The specialist team, who have been incredible, presented a new treatment plan which was to be implemented in addition to oral steroids. This did mean that I had to continue shielding, but the new treatment plan was much more thorough than what I had previously been offered. It consisted of an Ozurdex (steroid) implant as well as a three month course of four-weekly Eyelea injections in my PIC eye to treat the new blood vessel growth they found (choroidal neovascular membrane/CNV), or ‘bleeds’ as it is also known. The vessel growth explained the distortions I was seeing. Importantly, the team wanted to look at what could be done long-term, so they introduced to my treatment regime an oral immunosuppressant medication that calms down overactive immune systems. The aim was to prevent my body from attacking my eye with future flares and to ultimately protect my good eye from developing PIC.
The other treatments I had were short-term, as Ozurdex implants dissolve after around three months, and eye injections only being needed as a course of three. From September 2020 until February 2021, my eye stayed calm; the treatment had, and was, working. I still had blind spots and distortions – a permanent reminder that this disease tried to take my sight – but, I could see, and I could see very well.
In February 2021 (just in time for Rare Disease Day!), I was diagnosed with my third flare. The immunosuppressant medication was not enough on its own to keep my eye stable. My sight had deteriorated centrally again as the inflammation came back to attack my retina with a vengeance, bringing with it not one, but two bleeds. We went through the treatment plan again: an Ozurdex implant, a course of anti-VEGF injections and a continued prescription of immunosuppressant drugs.
In June 2021, the Ozurdex had dissolved once more and I had managed to taper my oral steroids down to a low dosage. My body wasn’t happy on such a reduced dose of steroids, and so my eye reacted with a fourth flare – more inflammation and another bleed. This time, to treat it, we needed to do more, otherwise this would keep happening.
My care team and I opted against another steroid implant; I was worried that I would only flare up again once the implant dissolved. So instead, the decision was made to increase the dosage of the oral immunosuppressant, and add a second immunosuppressant; one that I inject into my stomach every two weeks.
So far, this has been the perfect combination. My eye has been stable since last September, and my good eye remains PIC free. The treatment comes with its own risks and complications; I’m at higher risk of infections and complications from illnesses – which isn’t ideal in a pandemic! – and we’ve had to change my immunosuppressant tablets to an alternative drug as the original medication caused me to develop colitis. Despite these hurdles, my eye is doing well. If you had told me in December 2019 that I would still be reading over two years on, I wouldn’t have believed you.
I don’t know what the future may hold for me or whether I will lose more sight in the future, but in the meantime, I’m working to raise awareness of this condition. I’ve created a blog and social media pages to help with this, because I truly believe that if more people know about PIC (both medical and non-medical persons), then diagnosis may be quicker. This could result in reversible, or less severe sight loss. The awareness has also helped to connect a community of rare people with one another. Although often virtually due to the pandemic and country borders, I have found people to lean on during my hard times, and I hope others feel the same way and know that they can turn to me when they need. I am hoping to develop my efforts of raising awareness further and I have big aspirations for this year, which I am hoping will make others with PIC feel less alone. Every day, I am working to see, and to help other people to see The Big PICture…
From this journey, I have also learnt that sight loss isn’t the sentence I once felt it was. I’ve learnt that this is also the case for those with bilateral, advanced and permanent sight loss. My feelings at the beginning of my PIC experience were natural and are experienced by many in the sight loss community. But, it’s 2022, and there is so much in the way of accessible technology and equipment out there that helps people with sight loss – including those who have no sight at all – live fulfilling and independent lives.
In June 2021, I began working for my local sight loss charity as an Independent Living and Welfare Rights Advisor. Within this role I demonate accessible equipment to help people with reading, cooking, moving; I help with grants to get equipment, I run a social group, I complete benefit and concession applications and I provide general advice around the topic of sight loss. It’s truly my passion to help others with sight loss, in both my personal and professional life. My PIC journey has not been easy, but I feel that I have found a way to channel the experience into something purposeful. If my efforts make a difference to just one other person, then it’s worth sharing my story.
If you would like to support my awareness efforts and learn more about sight loss, please follow my blog on www.thebigpictureblog.co.uk, as well as my social media pages on Instagram and Facebook.