Living with Retinitis Pigmentosa
Fair warning: this post will probably have been edited/revised multiple times before you read/see this.
I have retinitis pigmentosa. A scientific definition of this is- Retinitis pigmentosa (RP) is a group of rare, genetic disorders that involve a breakdown and loss of cells in the retina — which is the light sensitive tissue that lines the back of the eye. Common symptoms include difficulty seeing at night and a loss of side (peripheral) vision.
The effects of RP can be difficult when explain to someone not affected by it. When you mention loss of vision to anyone, their first though goes to a potential issue with the lense of the eye, meaning glasses or something similar. Responding with the fact I have 20/20 vision in one eye, and 20/15 in the other usually only adds to the disconnect. The best way to explain it is to equate the eye to a traditional camera as whole. Our eyes, much like any camera, is made up of a lense and sensor. The lense being our actual eye itself, and the part of the eye responsible for most visual deficiencies, and the sensor being our retina, and the various cells/receptors it contains. With RP the issue doesn’t lie in the lense, but in the sensor.
The simplest way to explain it is as a gradual increase of one’s tunnel vision, where the periphery and night vision is the first noticeable loss. After that it can either remain dormant, or progress at a rate unique to every individual affected with it. Currently there are also no cures, and any cure in current development/testing is specific enough where it only will potentially help RP inherited on a specific gene. Mine was inherited on my 23rd gene, so any treatment developed for RP on another gene will not be useful for me.
As someone who was diagnosed with this disability during their second year of art school, I was affected deeply by it, and I can’t currently think of a better quote than one from Beethoven when he was confronting his incurring deafness, written shortly after the completion of his second symphony.
“Oh! ye who think or declare me to be hostile, morose, and misanthropical, how unjust you are, and how little you know the secret cause of what appears thus to you! My heart and mind were ever from childhood prone to the most tender feelings of affection, and I was always disposed to accomplish something great. But you must remember that six years ago I was attacked by an incurable malady, aggravated by unskilful physicians, deluded from year to year, too, by the hope of relief, and at length forced to the conviction of a lasting affliction (the cure of which may go on for years, and perhaps after all prove impracticable).
Born with a passionate and excitable temperament, keenly susceptible to the pleasures of society, I was yet obliged early in life to isolate myself, and to pass my existence in solitude. If I at any time resolved to surmount all this, oh! how cruelly was I again repelled by the experience, sadder than ever, of my defective hearing! — and yet I found it impossible to say to others: Speak louder; shout! for I am deaf! Alas! how could I proclaim the deficiency of a sense which ought to have been more perfect with me than with other men,–a sense which I once possessed in the highest perfection, to an extent, indeed, that few of my profession ever enjoyed! Alas, I cannot do this! Forgive me therefore when you see me withdraw from you with whom I would so gladly mingle. My misfortune is doubly severe from causing me to be misunderstood. No longer can I enjoy recreation in social intercourse, refined conversation, or mutual outpourings of thought. Completely isolated, I only enter society when compelled to do so. I must live like an exile. In company I am assailed by the most painful apprehensions, from the dread of being exposed to the risk of my condition being observed… What humiliation when any one beside me heard a flute in the far distance, while I heard nothing, or when others heard a shepherd singing, and I still heard nothing! Such things brought me to the verge of desperation, and wellnigh caused me to put an end to my life. Art! art alone, deterred me. Ah! how could I possibly quit the world before bringing forth all that I felt it was my vocation to produce?”
Having just begun my journey with therapy and medication for my recently diagnosed general anxiety disorder, and major depressive disorder, I have begun taking time to sit with the feelings I have been repressing for years with my disability. Having just come out of one of the darkest holes in my life, I have a desire to talk about my mental health and this disability, while at times can completely isolate me, I am not the only one affected by it. And these blog posts, alongside my posts centered on what I am learning through coding, are only being written in the hope that someone who is dealing with this, can come across them and realize that while it might feel that way, there are not alone in this. And others have been there.