CNTF Study


I hope everybody is enjoying this blog so far, even though it just got off the ground. 🙂 I wanted to tell you all about a study that I have briefly referenced in a previous post.

At the Retina Foundation, 3 days after I turned 18 years old, I was implanted with a CNTF capsule in one of my eyes. It was a double-blind study so only the Doctor, Dr. Spencer, and Kirsten, the technician/clinical study coordinator were supposed to know which eye it was in. This didn’t ultimately end up being the case due to a funny story that I’ll tell you in a short bit, but for the most part it was kept quite secret.

Now, this may gross you out a bit. A man who was driving a motorcycle was an organ donor and passed away within the past few years surrounding my 18th birthday and the start of the study. He was found to have extra cells of this particular variety that could potentially help individuals with Retinitis Pigmentosa stabilize their vision so that it doesn’t progress. I decided that I definitely wanted to try it even though I had only been going to the Retina Foundation a few months. I have several other unrelated medical issues, so hospitals and surgeries were not very scary to me, although I still do like to avoid them when possible. 🙂

This is a picture of my eye, taken with special retinal photography, with the capsule in it. It will either look really cool to you or weird/gross you out completely. I’ve heard both quite often when showing this picture.

Taken with special retinal photography instruments at the Retina Foundation

I can’t seem to find a picture of myself right after implantation surgery but I found a picture of myself after explanation which I will further explain in a minute.

The eye had to stay covered for 24 hours after surgery in order to let it heal and start the repair process

Okay, so now let me explain more.

What is it?:
As mentioned above, it’s an implant that has cells from a fellow who passed away several years ago. This capsule inside of my eye releases the cells in intervals. The goal was to slow or halt the progression of Retinitis Pigmentosa. Currently, there is no cure for it however.
Implantation:
I had to be 18, as mentioned above, to get the implant. They wanted to already have everybody implanted by this time, however they fought to get me an extension. Three days after my 18th birthday, I was implanted with it into one of my eyes. I didn’t have a definite confirmation which eye it was in until two years later. The surgery really wasn’t bad, as Kirsten was there to help and talk me through the whole thing. I was also passed out for all the actual surgery. When I woke up, both eyes were completely covered. I would not be able to see for 24 hours. I also had a stitch in both eyes. This was to ensure that I truly didn’t know which eye it was in. The protection was to make sure that my eyes had the chance to start healing. The picture above is just of one eye, after explanation, however I had both eyes covered after implantation. I went home and just really relaxed for 24 hours. My boyfriend came over to hang out with me, help me check my email, read texts, etc and so forth. It wasn’t that bad, but by the end of the 24 hours I was really ready to see again. It was painful seeing again however because I had stitches in both eyes, they were really dry and itchy yet I couldn’t scratch them, and very sensitive to light. It was a little rough, and I remember it like it was yesterday. I went in to the doctor, 1 day, 1 week, 1 month, and then at various timed intervals after that to check up on how I was doing, as well as take research results.  The labs that they had to run drove me nuts and after 2 years of them, I really began to hate them. I think I’ll dedicate a whole post to those darn things. 🙂
Explanation:
2.5 years after I was implanted, the implant was removed. At the beginning of the study, they told us that it was mandatory the implant was removed after the two years, even if they discovered it was beneficial. However, when it came time for me to take the implant out, they were giving the clients an option. I chose to take it out, because I didn’t visibly see much difference and with it in I would be disqualified for all future studies pretty much until I got it out. The study was also completely free to me, including surgeries, so if I were to get it out later I would have to pay for the surgery myself. (Or my family would, rather). I got it out, and this time after the surgery I only had to get one eye patched. I was happy to know that it was indeed in my left eye after questioning it, and sort of knowing although I wasn’t supposed to, for 2 years.  I did the same with this, I visited the doctor 1 day, 1 week, 1 month up until 6 months after. I also chose to enroll in an exit study so that they could monitor how one does after the implant is removed. Really, I’m just about ready to try anything because even though I know it is likely coming and it will always be at the back of my mind, I really don’t want to become blind later. The implant was removed after 2.5 years instead of 2 years because I was away at college and I really couldn’t take the time off. They filed for another extension and it was granted. I got it out during my Spring Break. (Lovely vacations/celebrations huh?)

Pain Level:
The implantation and a complication a few days after were the most painful parts. After 24 hours, my eyes were itchy, painful, red, and light-sensitive. I also had to put these drops in them every 3 hours and I hate hate hate drops. This was an unpleasant experience because I made my Mom do it but I never can manage to hold open my eyes long enough for the medicine to get in there. By the end of the drops, my face is practically soaked. A few days after the surgery, my eyes were really bugging me. They were hurting and I really couldn’t figure out what was going on. We scheduled an emergency appointment at Texas Retina and they discovered that part of the stitch had come loose. They numbed (with drops) and then I see this big giant tool thing coming towards my eye. It was just a little bit intimidating. I had to continue to put drops in my eye, but after that I wanted to…. for like a day. 🙂 That sentiment didn’t last long.

Rules:
We of course had to be 18 years of age, agree to use contraception if sexually active, agree to all the other terms and conditions and make the clinical coordinators immediately aware of any change in medical condition. The reason we were required to use contraception is that they didn’t know if it would have any affect on the fetus and they didn’t want to risk it.

Funny Story:
So at the beginning, I really truly did not know which eye the implant was in. However, as it healed my left eye really hurt more than my right. I started to think that was a clue, oooh lala. Then one day I was having an appointment and a “Fellow” came in to evaluate my eyes and take a look before the doctor came in. He walked in and said,  “So we’re taking a look at the implant in your left eye, correct?” I didn’t want to make him feel bad so I confirmed it. I am not sure if he ever was made aware that he slipped that. I tried however to do all the tests accurately despite me knowing which eye it was in. There was always the chance that it really wasn’t in that eye.

Measuring Progress/Success?:
Along the way, the tested our vision with several devices. My most favorite was the electro test, or the contrast test. During the electro test, we had a contact hooked up to a sensor that went into our eye like a normal contact. Except protruding from this contact were little wood sticks that kept one’s eye open. I also had a wired patch on my forehead. Really bright fast lights were flashed in my eyes and they measured what my eye and brain saw and didn’t see. It was very interesting. During the contrast test, we are giving about 20+ different circles and each of them vary slightly in color. We had to rearrange these circles so that they were in order according to their color. It was a challenge for a few of them but apparently I did better than most. This was exciting, however I never really feared about losing my ability to differentiate various contrasts. My least favorite tool was the Visual Field test. I will show and tell you more in another blog but basically you had to sit up straight with your forehead against this bar and you were surrounded by this half circle. You had to stare right ahead at the center and press a button when you saw a light flash in your periphery. Each test was 20 minutes long and they had to run two on each eye. It was a long frustrating day of testing and now I hate to take those tests. Ultimately, it was determined that my RP and eye condition didn’t deteriorate or improve while the implant was in my eye. We don’t think it’s related, however it very well could be.

So that’s a lot of information to soak in but I just wanted to give you a little background on it. Please let me know if you want to ask anything. I promise it really won’t offend me.
Always,
Jess

Resources


One of the most valuable tools for me to cope with and adjust to the diagnosis of Retinitis Pigmentosa and the years to come were various resources, both online and in person.

The Retina Foundation
Locally, The Retina Foundation of the Southwest is amazing. Even if you aren’t local, but need help or advice I would strongly suggest to contact them. They can likely guide you where to turn for something locally. They also conduct clinical trials, one of which I have participated in, and are a very good knowledge base as well as support system.
http://www.retinafoundation.org/ At the Retina Foundation I personally see Dr. Birch and Kirsten, the assistant.

Texas Retina Associates
Texas Retina Associates is another great resources for the medical side of RP. They have amazing Dr’s. Dr. Spencer is with Texas Retina Associates and he is actually the doctor that performed the surgery on my eye for the clinical study which I will explain later.
http://www.texasretina.com/default.aspx

The Retinitis Pigmentosa Community on Facebook
I’m an avid Facebook user, so having an online support group and interactive forum very easy to access has been wonderful. On the Retinitis Pigmentosa Community FB page, there are people from all walks of life who just want to vent, share their experiences, ask questions about experiences or others, or just know that there are others out there that are experiencing similar issues. For me, it’s reassuring to see some people who are middle-aged who do still have their vision, as I hope to still have it by that time as well. I love technology so there are lots of posts about Ipads, and other products and how visually impaired users use them. Its a very fascinating site and I would encourage you to check it out if you have a Facebook.
http://www.facebook.com/TheRetinitisPigmentosaCommunity

Foundation Fighting Blindness
This is a wonderful organization which will provide you tons of information on RP itself as well as living with blindness. They can help you find resources and are just another great resource out there, free of charge.

Vocational Rehabilitation Program or Department of Assistive and Rehabilitation Services
The name of the program depends on what state you are in. In Texas, it’s called DARS (Department of Assistive and Rehabilitation Services). This is a program which helps individuals either get through elementary-high school or get gainfully employed. Since many careers require a degree nowadays, this often involves helping get the consumer through college. I will go into this in more detail in another post as well.

Again, I would avoid a lot of the sites that just give purely medical knowledge. Most of what they say is correct however each person who lives with Retinitis Pigmentosa should not be lumped into a statistic about how many RP individuals lose their sight by such and such age. Everybody progresses at their own rate and a lot of times these statistics do more harm than good, I’ve found.

There’s so much information I want to tell you all about, and I think it will be nice for others to have one place to get a lot of this information. I just have to break it into posts and unfortunately my schedule hasn’t allowed me to get online very often and sit down to write a post.

If you have any other resources that you have found helpful and would like to share, I highly encourage you to do so. At the same time, if you have any additional questions or comments, please feel free to comment and I’ll get back to you as soon as possible.

Have a great weekend!
-Jess

What is Retinitis Pigmentosa?


Dear Reader,

I’m very excited to start this blog. If you haven’t done so already, please click “About the Author” in the upper right hand corner of the screen to learn more about me, and my purpose for this blog.

If you’re unfamiliar with what Retinitis Pigmentosa is, especially if you have a loved one that was diagnosed with it, don’t immediately go to Wikipedia. The first thing it will tell you is it “leads to incurable blindness,” which isn’t always the case. Let me tell you what it really is, from someone who experiences it.

It is an eye disorder or disease which is also progressive. An individual who has RP can lose their vision at a slow rate or a fast rate, or somewhere in between. It is really different for every person. Some never become legally blind, whereas some become blind in their childhood. I have been fortunate to still have my sight, and I am about to finish my undergraduate degree.

In medical terminology, it is a “progressive retinal dystrophy,” and is an inherited disorder. There are abnormalities in your photoreceptors (aka the rods and cones in your retina) which cause the progressive visual loss. I love photography and love representing what is happening not only through text, but also through pictures. The picture below is a picture of what my retina looks like. This is only one part of my eye. The black dots you see around the border are the abnormalities mentioned above. They are “pigments” hence the latter part of the name “pigmentosa.” Basically, where the dots are, I can’t see. As the disease progresses the dots increase in number and density, which leads to the visual loss.

This is one part of my eye, where you can see the abnormalities.

There are two main ways RP affects ones vision. Usually first to occur, is night blindess. The term is pretty self-explanatory. You pretty much can’t see anything in the dark, except for very well lit areas. This makes travel, by any means, difficult in any dimly lit area, regardless of day or night.

Next to go is your peripheral vision. For me personally, this is what has made adjustment and coping with RP a difficult journey. I will explain more in future blogs. As I mentioned above, as the pigments become more dense and greater in number, your vision decreases. These dots close in around the border of your eye, making a tunnel.. (“tunnel vision.”) This can also lead to total blindness if the disease progresses to that point.

My central vision, with glasses, is 20/25. It is actually quite remarkable, and I am very glad for that. I use my great central vision to help make up for the lack of peripheral or night vision.

Now that you know a little more about Retinitis Pigmentosa, I hope you enjoy my future blog posts. If you have any questions, comments, concerns.. or would like me to address a specific topic, please let me know! You can leave an anonymous comment below.

Yours,
Jess