Retinitis Pigmentosa is a degenerative eye disease that affects the light receptors in the eye. The light receptors are in the retina and are referred to as the rods, which are located around the edges of the retina and are responsible for peripheral and low-light vision, and cones located centrally in the retina and are responsible for central vision.
Since RP usually begins as rod degeneration a person would first notice problems with night blindness. You might also experience “clumsiness” which is really the peripheral vision fading. Eventually, vision fades until you experience tunnel vision. A wonderful series of videos showing how RP patients see were created by Rikard Andre, the links are below.
Retinitis Pigmentosa is really a group of hereditary progressive retinal degenerations or dystrophies. There are a lot of variations within the disease, but all the forms of RP have several things in common. The rods are usually affected first and this leads to peripheral vision loss and the inability to see in darker settings. Adjusting to changes in light, for example entering a softly lit room from a bright sunny room can take several seconds to a full minute for eyes to adjust. Cone degeneration occurs later and affects central or reading vision and the ability to perceive colors.
Below are some images that explain a little about the visual fields. This is the entire scope of where a person can see. What surprised me when I was diagnosed was when they said peripheral I thought only of side vision, not up and down. I had been tripping over things and banging my head on things for years before I was diagnosed with Retinitis Pigmentosa which makes me think that my up and down periphery was affected more significantly first. A person is considered legally blind in most countries when their visual fields are below twenty degrees. You may also experience blind spots in your central vision which will also put you into the legally blind category.
The experiences that you have with RP will be different than other people with this same disease and there are many reasons for this. Each of us is unique and this disease is unique in how it expresses itself. Faulty genes are the cause of the disease but it isn’t just one gene, there have been over 100 different effected genes that cause RP. Some, usually males experience deafness in conjunction with their diminishing vision. Some people experience floaters, flashing lights or light shows as they are often called, some have headaches, lethargy, dry and irritated eyes. There are many symptoms that doctors don’t always think of as part of the RP experience which is why I am trying to collect them all here. This should be your resource so that you don’t have to search for reasons why you are more tired than usual, no it’s not just because your getting older, or why you tend to get sick with colds and flue a little easier than you used to. For these last two examples I ask you to consider that you have a chronic disease. When an organ, in this case your eyes, are not healthy your body can fight very hard to compensate for its failing parts, so it stands to reason that you would suffer a bought of occasional tiredness and that your immune system might not be firing on all cylinders. The point is that you should try to be kind to yourself, give yourself the extra care that you need so that you can minimize these occurrences. I have added yet another video link to a young woman named Molly Burke whose video about her RP helped me to understand some of these issues. You might recognize Molly from her Dove soap commercials on Canadian TV.
I am only touching the surface here but if you want more in-depth details into RP the best online resource I have found is from the Foundation Fighting Blindness. A link to their booklet on RP is found at the bottom of this post. I have also put together further information in the blog about genes and I pull it all together in the book; RP Tunnel of Sight: Information for RP sufferers and those who care for them (due out December 2017). I will be adding to this site regularly so that I can build a real resource for you. There will be information on everything from the disease itself to tips and tricks to living with Retinitis Pigmentosa. I am hoping to put together a podcast in the new year and there are some videos on YouTube talking about the disease and showing you some of the tech and tips for dealing with RP. If you are finding this information useful to you and would like to help keep the information rolling out you can support the work through Patreon, just $1 per month would be a great help in paying for web-hosting, technical support, and equipment to make the site better and better. Any help that is offered is greatly appreciated and I also encourage you to get involved in the community https://www.facebook.com/pg/rptunnelofsight
One thing I would like to add at this juncture is that although there currently is no cure or treatment, there is hope. The scientific community is busy working on an amazing variety of cures and treatments that make me believe a cure will be found in my lifetime. In the meantime, there are many things that RP sufferers can do. This disease is slow moving and can be quite frustrating to cope with, but with help and support, we can live rich and rewarding lives.