Thank you to Becky, for sharing her journey of Autoimmune Retinopathy and it’s complications with this blog. Becky does a fantastic job of raising awareness on her Instagram page. Please read her story and give her a follow: @visionwithoutsight.
Hello, I am Becky! I wear many hats including being an infectious disease pharmacist and yoga studio owner and instructor. A year ago, I was diagnosed with paraneoplastic autoimmune retinopathy (AIR). AIR is a rare autoimmune disease which destroys the retinal cells of the eye leading to progressive vision loss. There are different causes of AIR, no set diagnostic criteria, and no standard treatment which makes it a challenging disease to both diagnosis and manage.
How did your vision loss start?
In late 2019, I suddenly experienced visual changes and lost part of my left peripheral vision. Nothing seemed to cause it, nor did anything make it better. From there, my vision continued to worsen with progressive peripheral vision loss and increasing visual disturbances like floaters, flashes of lights and moving portions of my vision. I had more testing than you can imagine with many potential diagnoses which typically eventually got ruled out. I was referred two large tertiary centers with no definitive diagnosis despite progressive visual changes.
How were you diagnosed with AIR?
Over time, I had blood vessel leakage peripherally and on electroretinogram (ERG, which tests the function of the photoreceptors of the eye), I had substantial loss of retinal function in the left eye. All of this was pointing towards AIR rather than other autoimmune conditions of the eye. This prompted the retinologist to order retinal antibodies to confirm the diagnosis of AIR and to help point towards the underlying cause. Retinal antibodies are all immune mediated; however, they can come from three casual categories: autoimmune, cancer associated, or melanoma (skin cancer) associated. Six weeks later, I had zero retinal antibodies in the autoimmune, one which was cancer associated, and two in the melanoma associated category so I was officially diagnosed with presumed paraneoplastic (cancer related) AIR almost a year after symptoms started. Obviously, this is not what we expected nor wanted. Receiving the diagnosis that you are eventually going to be blind and that you likely have cancer was devastating.
How did they not know if you had cancer?
Cancer associated retinopathy (CAR; a subtype of AIR) and melanoma associated retinopathy (MAR; also, a subtype of AIR) cause damage to the eyes typically before the cancer is evident and the cancer sometimes is not evident up to 10 years after the vision loss starts. In CAR and MAR, the production of the antibodies slows the cancer growth substantially, but destroys the retina leading to the loss of vision function.
Did you have cancer?
I have no overt signs of melanoma; however in an extensive workup, I was found to have a tumor of the thymus (thymoma), which is an organ in the center of the chest connected to the heart, lungs and aorta. Ultimately, I had the thymoma surgically removed with no beneficial changes in my vision. Currently, thymomas are always considered cancerous; however that was not always the case since many are slow growing including the one I had so my doctors argue among themselves whether it was cancer or not.
What daily activities has your vision loss affected?
One challenge I experience daily is that I no longer drive. Driving allows you to have a freedom to go and do whatever you want or need to do. After more than 20 years of driving, it is a difficult thing to give up. With that said, the safety of others means more to me than my convenience. To do even the smallest errand, I need plan extensively and either have to walk, ride my bike in a non-bike friendly city, rely on someone else, or use Uber/Lyft.
Additionally, I no longer have night vision thus going anywhere when there is limited lighting is almost impossible unless I have someone with me who knows how to effectively be a guide. Most notice that I am able to do things during the day as my central vision is minimally affected so they assume that same function is there when other environmental conditions change like lighting.
Furthermore, since my condition is immune-mediated, I have been immunosuppressed nearly the entire pandemic. Couple this with working in infectious diseases and seeing vaccinated immunosuppressed get hospitalized, there is a constant fear of getting infected anytime I am outside of my home.
How has vision loss affected you emotionally?
Having a diagnosis is a relief but also, its own sort of a nightmare. I struggle with knowing how to prepare myself to be blind. Losing your vision is mentally taxing with fears of the unknown. To help reduce my fear of being blind, I look towards others who have lost their sight for both inspiration and guidance. Seeing others live normal but adapted lives makes the future seem brighter.
Another challenge is to constantly face the subtleties of ableism is our daily society. Vision loss is a spectrum and it can be difficult to explain what one actually sees. While my visual acuity is relatively intact, I have a reduced visual field, flashing lights, random black spots and floaters, fog rolling through my vision, and an area that always blinks/moves. With that, people will often comment that I function great and they could never tell something was wrong or that I don’t look blind or visually impaired. These well-meaning friends and family often say things that are intended to be supportive but imply pity. Yes, it is a hard situation but receiving pity isn’t going to improve how I feel or see. I do appreciate the support and love; however, I am gently trying to educate them on how their words are perceived. Luckily, I have an amazing support system and understanding friends and families who are learning alongside me.
What are your next steps?
My next chapter will likely look (yes, pun intended) different than it does today, but I know my connections in the visually impaired community as well as my family and friends will help me navigate the changes that vision loss brings. While I know there will be ups and down on this journey, I am tenacious in all things I do and will continue to do everything possible that I do with vision albeit adapted.
I am currently learning Braille and am looking into adaptive tools to allow me to stay in my current profession. Also, I am in the process of starting a non-profit to further support those with autoimmune eye conditions to find purpose and fulfillment in this new chapter. I will thrive blind.
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