Retinitis Pigmentosa

It’s such a weird concept for me when it comes to my condition.
I grew up low vision due congenital cataracts, even having made surgery at 3yo. My first experience in the visually impaired world wasn’t known for me. I never realised I saw so little compared to others, I never had something to take as reference so for me: it was just normal. I was just clumsy. It’s normal for people with myopia to have to wait the bus to be like 2 metres from you to signalise for it to stop. That’s how I grew up until an eye doctor tell me: “you must not lie and guess the letters. If you lie and guess them I won’t be able to properly diagnose, so if you can’t see them, just tell me.”
And that was my first time hearing the term “low vision”, at 18yo, the same year my retinal dystrophy decided to manifest, basically about 6 months before I became legally blind.

What makes it even weirder for me is taking years to know I have a retinal dystrophy, and to now be self aware to know the symptoms it displays. Not a self diagnostic but also not an opinion of a doctor but a guess I have.

My condition seems to be Retinitis Pigmentosa Sine Pigmento. A form of RP that’s really hard to catch because it doesn’t show itself as most forms of RP do.

And it’s a hard thing to grab because I didn’t know I was night blind until recently, I thought I couldn’t see at night because I have photophobia and the street lights and cars messed with my vision. I had to discover my night blindness in a very out of the box way. I knew I had tunnel vision but that’s one of the aspects that made realising the night blindness even harder.

For those with congenital aumarosis (100% blind by birth with no other condition in play), what they see, is absolutely nothing, and humans usually can’t grasp the idea of absolutely nothing. There are some tests you can do to have a partial idea of what it is, but it’s not black, it’s absolutely nothing. And for some reason, that’s a concept I understand. So when it comes to my tunnel vision there’s no blackness outside of my visual field, and I don’t have enough visual memory to even know if this lack of field of view was once lower, I have no idea what’s outside of my field of view, and it’s not blackness, it’s just nothing at all, and I even had to try to explain to blind people about nothingness because they acquired blindness and they see full black or full white, or just gray.

So I never even noticed the night blindness, because it portrays as street lights and vehicles headlights, and just a deep darkness that makes everything basically black, and I just didn’t understand that besides the lights, the blackness meant I couldn’t see. Until the day I went into a darker place with sunglasses and I couldn’t see, then it came, late again, but came, what night blindness looks like.

So basically: tunnel vision, check; night blindness, check; getting darker becomes something similar to static on a tv, check. I will go for a diagnosis to know if it’s RP or if it’s another retinal dystrophy that has similar symptoms, I plan on going after it this year, I just need to have some time to breathe since I haven’t stopped since September and I’m basically burned out.

Have you ever expressed you’re a level 2 support autistic person and people would invalidate you because “you came to this appointment by yourself”?

People think of autistic people as two sides of a coin, like it’s black and white, but the “scientific” name already says ‘spectrum’. And that spectrum isn’t only on some classic functional tasks, not only that, but autism usually comes with other comorbidities, like me who has a personality disorder, probably undiagnosed ADHD, and many body structural differences that might be related, such as dispraxia. And my luck is just right for me to also be born with congenital cataracts, an eye disease that also loves to come with something else, such as glaucoma (not my case) or in my case, retinal dystrophy, that might be retinitis pigmentosa, I still don’t know which type of dystrophy I have.

So even trying to put autism into three categories, you still have a whole spectrum on each, the only way I see that makes this classification possible is the wording:
1. Support Needs
2. Substantial Support Needs
3, Constant Substantial Support Needs

This wording makes it vague enough to make a classification possible, and due to people viewing autism as black and white, the support needs level 2 ends up being the one people often forget, invalidate and misunderstand.

I’m a type of person who’ve been through forced independence, I started catching busses by myself at 10 when I started going to school by bus after having gone through very aggressive bullying on the school van that would get me to school. Autism wasn’t so spoken about, much less autism on females assigned people, so I had to learn a lot of social cues, communication, non-verbal communication skills (tho I’m still terrible at them), social norms, expressiveness and knowing how to pick my words when talking. It’s exhausting, I suffer from autistic burnout for years, and one of the causes is the constant task of knowing how to articulate my needs, or bend myself to be flexible, despite having cognitive rigidity, because others wouldn’t do it, even being neurotypicals. So at first glance people can’t realise I’m on the level 2 category, because my adapting skills are very sharp and I adapt fast to new situations. That’s why I pointed this to blindness, because I got blind, and I adapted. I had no one to teach me how to use a cane so I taught myself. I’m still waiting for the daily activities rehab part from the blind foundation but I just can’t stop my life to sit and wait. I want to learn how to make my own make up, and I basically started making my own make up already, I need to learn the techniques for that, but while I wait, I just do my best to be able to do something.

I might not show so much support needs on socialising, learning communication skills and articulation, because I adapted. But if I get to a meltdown, there’s nothing that will make myself snap out of it, I need intervention from people to help me and avoid that I put myself at risk. Usually after a meltdown I have a shutdown too, so I just fall asleep without much control over it.
To catch the tube by myself I need to listen to music so I keep calm, and nowadays there’s a musical I listen to absolutely every time I need to go out, that helps me self regulate. I stim a lot and often get censored by people close to me, and I have to remind them that I need to make constant and repetitive movements when I’m overwhelmed. But this is not where it ends.
When it comes to my body, I don’t know when I need to pee unless I’m urgently needing it, so UTIs are basically my daily companions. I can’t feel satisfied after eating, so sometimes I need someone to give me a hint that I’m not eating in a healthy way. I don’t like most of the stuff that can make you gain weight but I’m obese because other factors. I have insanely high tolerance to pain, I might break a bone, and it happened before, and not know it, because well it might hurt but it’s too little to actually give me some concern. At the same time I am extremely sensible to my internal organs, so I know EXACTLY where I feel what I feel. If I feel chest pain I won’t jump to conclusions that I might be having a heart attack because I know if it’s a pain on the boobs, the lungs, the heart, the esophagus, etc. it even got me confused lately because I’m feeling a pain on a place I don’t know which organ could even be there to ache, other than my intestines, but the pain doesn’t give the vibe of intestinal cramps. So all that already show how much substantial support I need just for functioning a bit. But I also need support when it comes to daily tasks.

I hashed depression in here too because of this. Me getting depressed makes me not take care of my house and myself, and I do have very severe depression, that can be very crippling sometimes. And also have to deal with PDA. So trying to humiliate me, or repeat many times to me that my house needs cleaning actually makes me not clean it. My mum had to find the right words and empathy to make me start reacting and taking care of myself and everything. But I still need this support. Sometimes if I try giving myself rules, I don’t really follow them, I just feel frustrated when I do something out of the pattern I usually follow and then the repercussion is that I don’t know where I left something. And this is why I hashed blindness too. Because being blind means I have to be even more strict with organising routines so I know where everything is, and can find it later. But without clear instructions of what I have to do… I just don’t know how to. Yes I adapt, yes I try my best, yes I do without waiting, but I’m still not able to find directions without someone to direct me. It might sound silly, but my support needs actually makes me need direction from a third person. So I want so bad to get to the daily activities habilitation so I can learn how to organise stuff properly.

This is a bit frustrating for me to express, because I don’t want to use my depression, autism or blindness as crutches to justify a messy house. But I know that I need someone to direct me on some tasks, or I just can’t do it myself. And I wish people could understand how that is a need, and not me talking BS. Even if it’s not a classic need from a person with substantial support needs, it still is my need, just like there are people with ASD that can’t cross a street by themselves. It’s a need, and instead of invalidation and ableism, the world would be better if only everyone’s needs would be respected.

What Is Usher Syndrome?
Usher Syndrome is a genetic disorder that causes progressive hearing loss, vision loss or retinitis pigmentosa. Not to mention balance problems. Last but not least, even though there is no cure available, there are treatments available that focus on managing symptoms through hearing aids and cochlear implants, vision aids, rehabilitation, and early intervention, while research explores gene therapies and drugs to slow degeneration.

When I was 18 years old, I started to lose by vision. As an athlete, the signs, at first, were small – missed passes from my teammates on the basketball court. Losing a fly ball in the air at my baseball games. Not following my golf ball’s trajectory on the course. I went to see a doctor thinking that it was something perhaps with my brain and was shocked to eventually learn I had Retinitis pigmentosa (RP) – a rare, degenerative eye disease that makes cells in the eye breakdown over time – eventually causing vision loss. I have one of the most severe cases of RP that one can have – and by age 30 I was completely blind.

For years, as I worked through a range of emotions, I always knew one thing - I was determined to not let RP define who I am or what success I find in my life. Up until the point of my diagnosis, I had planned on being a doctor – and while that course was, and remains, unavailable, higher education and career fulfillment is absolutely not. Through a lot of tenacity, problem-solving, failure and entrepreneurship, two years ago I found myself starting a new job at NSITE just outside Washington, DC. Here, I have found my deepest purpose – helping blind and low vision individuals find jobs, while also helping companies and products make their tools more accessible to everyone. In my spare time – I’m entering my fourth year as a goalie for the U.S. National Blind Hockey Team because being blind has never dimmed my love of sports!

The journey to this role and this life was not a straight line. I hope as part of this community I can share a bit about my path in the hopes it shows other blind and low vision individuals how they can find fulfilling personal and professional lives, even in the wake of the unexpected.

I’m always happy to talk to anyone who has lost vision at any stage of life, to help them understand what steps they can take to come to terms with their diagnosis, and when ready, start to think about what is next.

I look forward to learning more from you all, too.

#Blindness #RP #Blind

#VisionLoss

The moments I remember are always filled with frustration, anxiety and #Depression. But it’s the time that I can still see the world. What am I going to do when I turn into #Blindness at last? Guess nothing needed to be focused specially anymore because all days will be filled with the same thing- sadness.

I really want to figure out a reasonable explanation about why god chooses me to suffer this. If I were talented, fine, then I would be the “selected” one who would be destined to go through difficulties first and at last make difference. However, I am just an ordinary, average-looking person, and only unusual in terms of the really fragile and emotional personality. I cannot imagine how to face the outcome with full consciousness. I cannot imagine how I can find another reliable partner except for my parents. I also cannot think out a way living independently by myself anymore, although independence is the most important quality which I truly admire.

I had been living without knowing my #RetinitisPigmentosa for 20 years. After then, I am struggled to deal with it. Sometimes I have a feeling that I am strong enough to overcome every challenge in the dark, while other time I am too terrified and desperate to do anything. I know it’s common for everyone, and I should accept the repetition.

There is no sadder thing to compare myself to others, and after being aware of that, I tried to keep my mind under surveillance and control. However, still, I am jealous of people who have healthy eyes now and then. Maybe it’s a warning to remind me of valuing the current life? Cause I am always the person who lives by plans and hopes. It gives me endless motivation, as well as #Anxiety. Be as it may, If I am healthy, I would rather choose motivation and anxiety. Well, guess now I have to choose current life or I don’t need to make decisions anymore.

You know what, I was once proud of my philosophy thinking way. Now, I am not going to read any articles about someone’s life stories and reflection. It leads to sadness, weakness and emptiness. It is not applied to the real and secular life at all. Instead, I prefer to read some practical psychological articles. And the best chance of me for now is to become a psychologist in counselling field. It can be seen that I am still paradoxical: for one thing I want to escape from life when I cannot see things anymore. for another thing, I still want to find a solution for the unseen future. I really hope that the day will never come, or come after I am 80 years old. Is it too long and a little bit greedy? Then maybe 50 I guess? Or, at least after getting my phd and obtaining a decent position?

Life is struggle. What is in your mind?