Do You See What I See?

by Maurie Hill on August 15, 2012

What Maurie sees on the eye chart

This represents approximately what I see with my left eye while looking straight in the middle of the eye chart from one meter away.

Before anyone can understand any functional visual improvements that may occur from my recent participation in a stem cell clinical trial, an explanation of my disease and its progression over the years is in order.  Without that, it may be tough to explain what I may or may not notice in functional visual improvement moving forward.  In a normal eye, a high concentration of healthy cone photoreceptor cells are centrally located in the macula, providing superior visual acuity, resolution and color definition when you look directly at an object.  In simple terms, these cone cells act like the auto-focus of a camera.  Just like the camera, your eye starts its auto-focus function when you look directly at the subject.  With Stargardt Disease, instead of providing photoreceptor protection, unhealthy cells in the RPE layer are causing degeneration of those amazing cone cells.  Therefore, their ability to focus with any reasonable resolution while looking directly at something is reduced dramatically depending on the exact stage of degeneration.

I’ve lived with Stargardt Disease for a very long time, moving from perfect vision to very poor central vision over a period of 30-40 years.  But it can be tough to mark specific points on a degenerative continuum; it’s more like a grayscale with an infinite number of points of interest.  It complicates it further since so many of those markers are functionally insignificant; when you can’t recognize someone’s face, it doesn’t really matter if it’s because you can’t see enough detail or if you do not see any features at all.  For me personally, when I use both of my less than perfect eyes, I can see facial features at close range but they lack fine detail; I may not be able to distinguish this face from three other similar faces that I know.

And since I don’t typically walk around with one eye closed, it is not often that I pay attention to the visual ability of one eye alone.  However, when I learned that I was going to participate in this trial and get my functionally worse (left) eye treated, I began paying closer attention to exactly what I could see just using that eye.   While in Minneapolis at the Visions 2012 conference, I remember sitting under a shady tree on the steamy streets while doing a little people watching covering my best (right) eye.  Depending on the distance away from me, I observed walkers completely disappear as they passed through the void of my central vision.  It’s like they were actually swallowed up by their surrounding scenery.  It’s not a black spot or white spot – it’s just that it looks like you’re seeing right through them, their edges indistinct, perhaps like a Monet painting without the luxury of subtle color differentiation.  At a closer distance, it’s kind of fun when you can just see their head and their walking legs and feet clearly but their body is simply missing as they pass right by.

More scientifically, the eye chart at four meters does not exist for my left eye’s central vision.  I might as well be staring at a blank wall.  When I look in the center of a big lighted ETDRS visual acuity chart and it’s slowly pulled towards me to one meter away, I can see that there are letters on the top line, down the right hand side, and toward the bottom; all of this sight lies on the outskirts of the big circle of central vision blankness, where there is not even a hint of any text.  But normal peripheral vision has a visual acuity no better than 20/200 so it’s odd to try to distinguish letters without looking straight at them.  These letters are still hard or impossible to read depending on their size and location.

And I stepped up my observation of the Ansler grid before surgery as well.  With my left eye, not only is the middle dot invisible, but an entire circle of grid lines are missing.  While looking straight into the middle, I can see grid lines at the four corners of the square with my peripheral vision. But those grid lines are narrow and too light, leaving me wanting more information.  With my trusty thick black Sharpie, which I always keep close at hand, I drew a heavy black circle around that little black dot in the center, forming a circle two inches in diameter.  Again with the grid about 12 inches from my nose, that bold circle I’ve drawn is wiped off the face of the earth.   You might say that my central vision loss in this eye is at the “end stage”; there are no good spots.

On the other hand, when I look straight on at the middle black dot on this Ansler grid with my “better” right eye, only the bottom half of that thick circle I’ve drawn is missing.  This “sweet spot” in the top half of my central vision functionally serves me very well.  Though to a retina specialist, my retinal photographs appear to be equally worse in both eyes, the usefulness of my right eye is drastically different.  This is somewhat apparent in the visual field tests I’ve taken but I don’t think one could argue against the fact that it is the combination of test results and personal observation that gives the complete picture as to my actual functioning vision.  But more tests will be coming in the future, giving the doctors solid points of comparison before and after surgery.

There was just one more thing on my list before getting my eye injected with good RPE cells that could potentially change things forever.  I remember even at 18 having trouble with those color vision tests required to get a driver’s license.  This would have been the 1976-1978 time-frame.  Remember those numbers buried in different colored dots?  Evidently I passed enough to get a license but it was always a struggle for me.  So the day before surgery, I went online and found such a color vision test.  I failed miserably with my left eye – 0 percent.  I also took a color vision test at Mass Eye and Ear around 1995.  There were about 10 circular wooden chips with varying degrees of two opposing colors.  I was supposed to put them in order of intensity from one color to the other.  I was only able to identify the two endpoints, the two solid colors; all the various shades of color looked identical to me  I imagine it will be a while before I’ll even attempt a color test again since my injected RPE cells would have to make up for 15-30 years of degeneration.

But post- surgery, you can bet that I have repeated some of these little unscientific tests over and over again.  My daughter says “stop doing that!” when I stare at her face with my good eye closed. Like that grayscale, it’s really hard to pinpoint a difference between gray and gray, so I’ve added a new test:  while lying in bed, looking up at the white ceiling, I move my hand across my face at arm’s length.  Because of the white ceiling, it appears that my hand disappears behind a very thick cloud.  I can’t tell that my fingers are wiggling or even that it is a hand.  I am realistic – there’s a lot of fog for those new RPE cells to munch up.

I spent my first ten years at Ai Squared working in a technical support position, helping people use ZoomText.  It was common for me to talk with 20 to 30 low vision ZoomText users per day.  We often would compare notes on our eye conditions and what visual activities we had trouble with.  As far as the retinal degenerative diseases go, there were a lot of similarities like bright light sensitivity, a need for contrast, and an inability to perform functions that required detail (reading text or doing any crafts or handyman work).  But the degeneration is a continuum and though two people may test with the same visual acuity, actual functioning sight depends on many factors:  the location, size, and degree of the visual atrophy as well as one’s ability to adapt.

And no two eyes are alike even in the same person.  For example, when I hold my hand at arm’s length and move it across my “blind” spot using just my left eye, it completely disappears into the background for a span of about three hand widths both horizontally and vertically.  But in my “better” untreated eye, this blind spot is only about two hand widths, leaving some remaining functional vision that I rely on heavily.

While walking around a familiar city, I’m lucky enough to be able to walk as fast and as confidently as anybody.  Knowing where the curbs are likely to be helps me see them.  Just don’t ask me to find a particular store or read the street signs.  I also can thoroughly enjoy a scenic landscape.  Just don’t ask me to look at a particular tree or animal – that’s the job for the central vision.

There has been a lot of talk about what the timeline and degree of improvement were for the first two patients in this trial.  I ask you to refer back to the original Lancet report for that plus take a second look at the trial’s outcome measures, which don’t include visual improvement.  No two people are alike so I suspect that timelines and actual functional improvement will vary.  Will those missing letters on the eye chart start appearing in gray?  Or will a visual “sweet spot” grow in my central vision and if so, at what point does that become large enough to be functionally useful?  There are no guarantees and only time will tell.  In the meantime, I will look over my doctor’s shoulder with eagerness as he intently observes my retinal photographs and test results.  All I feel comfortable saying at this point is that there is definitely no bad news.  September and October are the milestones I’ll be looking for.  And with good advice from my sister, I’m going to relax a bit and stop driving my daughter crazy being a one-eyed pirate and just hope those new RPE cells are extremely ambitious workers.

Disclosure: Maurie Hill is a patient in this Stargardt clinical trial and is also an Advanced Cell Technology (ACT) stockholder.  ACT is the trial’s sponsor.

  • Irv Arons

    Maurie, what a wonderful writeup, to explain in so much detail what you have and are going through. As you know, you have a whole group of new friends pulling for those RPE cells to do their job and improve your vision. We all hope that will be the outcome.

    Best wishes,

    Irv

  • Selluwud

    Maurie, thank you for the update. It is nice to know that you are not experiencing any safety issues and that you are so diligently looking for signs of visual improvement. I see that you have joined iCell and many of us (members) have responded to your post there. Does it help you if we use larger and bold text in our post or does your software make that unnecessary? Thanks again for you ground breaking undertaking.

    Selluwud

  • msemporda

    Very good background summary Maurie – so much there to compare future developments to. Thank you for the effort to
    educate us all and look forward to your next installment. Have a great final few weeks of summer. Best regards, Msemporda

  • Kim

    Great blog, Maurie. You have an attitude and outlook on life and challenges that is inspiring. Thank you for sharing your journey.
    Warm Regards,
    Kim

  • http://twitter.com/StemCellsCURE StemCellsCure

    Thank you Maurie for a fascinating write-up. You have a gift to write with clarity that not everyone enjoys. I pray in coming days your visual acuity will improve and look forward with great anticipation to your updates. God bless and wishing only the best to you.

  • Danny_NJ

    Forgive us out here for being just as “eager” to hear some news from you. When you describe your vision, it sounds so similar to mine. I’ve been a bit impatient to hear if you’ve had any results and I would be equally impatient/eager if I were the RPE recipient. Perhaps the RPE cells are working in your eyes like folks who set up sandbags, one has to pass to another and another until the job gets done. Maybe there’s more going on than is visible to you. The RPE cells closest to your retina are getting the info they need and passing it to the next cell and so on. With 100K of them in there, that’s a lot of cell training going on. THANK YOU SO MUCH for letting us know. My left eye is better than my right and I’ve done the one eyed pirate thing too!!!! LOL. I will keep you in my thoughts and hopes. It’s only been a month and before you know it, more time will have gone by and who knows what may happen. You aren’t driving your daughter crazy, your daughter will be the first one to cry with you when you can see her in your pirate eye for the first time in years. Best of luck to you Maurie.

  • Phyl

    Maurie
    Thank you for writing with such clarity and sharing your journey with us. Phyl

  • Elrod

    Terrific write-up, Maurie. Thank you, and best of luck ! We’re with ya !

  • Maurie Hill

    Thanks for asking – my ZoomText software makes it unnecessary. If I didn’t need magnification for the other parts of the screen, it would be helpful, but I have to magnify the whole screen anyways.

  • Maurie Hill

    Love your sandbag analogy. I also wonder how suddenly a change becomes noticeable and what it takes internally to get there.

  • http://www.facebook.com/estreett Elizabeth Licht

    Thank you Maurie,
    For someone who struggles so much sight, you offer great perspective, insight and vision about SMD and the hopes and challenges that come with it.
    Elizabeth L

  • Ur_blog_reader

    Hi Maurie

    Best wishes and best of luck. You are paving the way for many people for years to come.
    You are also an ACT share holder, what you blog may impact your stock price both negative and positive ways.
    I am sure there might be a binding contract to which you can and can’t say. Please, save the best for last
    Regards.

  • http://www.facebook.com/jerry.dotto Jerry Dotto

    i really hope this therapy works and you can regain at least some of the lost sight back.
    your explanation of how it is to live with such a disability is informative to say the least.
    at church we have a large congregation of elderly 70-90’s and one woman in particular has AMD
    as i was watching her view the video screen it seemed she was looking at me it makes more sense now that i have a perspective to work from.
    may the Lord bless you with highly energetic RPE cells

  • Barbara

    Maurie,
    Love, love, love the detail! Good luck with the trial! I’m not in a trial but have found it’s helpful to assess visual changes by keeping a journal. You could use it to describe subjectively or objectively how you see an item. I found change was most noticeable if I hadn’t seen the object for quite some time.

  • Danny_NJ

    Hey, HI Maurie. I’m always speculating on lots of things, sometimes I’m wrong, often I’m right. In my mind, you having 100K cells means initially there is more of a “cloud” of cells that need to transform. The first patients with 50K may have had earlier results with a smaller “cloud” of cells to train. I rely on instinct more and more as I get older. I’m seeing your vision improve, one day soon you’ll be wondering what is going on as your brain realizes your bad eye wants to see again. For me, my brain compensates for my vision loss in many ways but if I’m tired and my brain is tired it doesn’t want to do those things and I feel more blind than usual. After all these years of your one eye being the worst, your brain has shut down a bit in favor of your good eye. As that eye wakes up as the cells organize, your brain will pay attention and you’ll know it. I’m excited for you. This sounds dumb, but my predictions often sound dumb, but you are going to start seeing with that eye, foggy at first, but it’s going to wake up. I literally see it in my instincts. If I were you I’d be checking every day just like you probably do, and that can be a bit of a downer as things don’t seem to change. BUT change they will. !!!!! Hang in there Maurie. I know that this can be a rollercoaster of emotions for you, but rollercoasters can be incredibly fun too. We are all pulling for you.

  • Julien

    Dear Maurie,

    I am a PhD working on RPR differentiation from human pluripotent stem cells. Since I am mostly doing research, I have very few opportunities to discuss with patients suffering from vision loss: thanks a lot for sharing your experience. I really do hope the RPE injection will bring some improvements to your vision.

    Best wishes,
    Julien

  • Julien

    Sorry I meant RPE differentiation…

  • jon

    ty

  • Maurie Hill

    Hi Julien,
    Thanks so much for writing. Your work gives so many people potential for hope. And we’re all fascinated by the science as well. We know the research takes a lot of dedication, creative thinking, and college debt! Feel free to educate us here.
    Maurie

  • Maurie Hill

    Thanks Danny for your fun and thought provoking insights. Your interpretation of what my brain needs to do makes sense to me. And yes, it is a roller coaster which I’m really learning to appreciate and enjoy.
    Maurie

  • Maurie Hill

    There is no binding contract, that I am aware of, with anyone telling me what I can or can not say. I am sharing my trial experience for the purpose of sharing my trial experience. Though I understand that this trial is very early and every data point is important, what one patient says at one point in time should not trump the collective data over time. If it does, than that is out of my control. I take my responsibility as a clinical trial patient very serously as well as the responsibility for relaying information that may help others in the future.

  • Danny_NJ

    Maurie, thanks for saying that. Besides being a brave soul, your a very smart one. When you explain your vision, it’s better than any explanation any of the 5 doctors I’ve seen have ever explained mine to me. Most of the time I’m trying to convince THEM of what is going on. Along with my central vision loss, I have what I call an Octopus in each eye. It’s part of the retinal tissue that died and the blood vessels that grew to it to sequester it. Once my immune system did it’s job, the piece of retina and vessels broke free and now they swim around in my peripheral vision. I see them often but sometimes I just see motion that I confuse for something else. When I told one specialist about this and asked him if it was what I just told you it was, he said “possibly” with no further explanation. Some docs are simply outstanding though so I won’t throw them all into the same barrel. My point is that your explanations are actually amazing. You sure have a gift in that department. You’ve combined science and sentiment in a way that anyone reading can really grasp. You should write a book. I’ve found your blog comforting and I’m not feeling as alone with my disease as I once was. Thanks Maurie. Hey, is Maurie short for Maureen, or is it simply Maurie? Just curious, I’ve never seen that name before. Take care, be well. It’s going to be a beautiful Autumn. Danny

  • always alert

    it is a very nice and cool site.

  • always alert

    i like it

  • always alert

    what a wonderful writeup, to explain in so much detail what you have and
    are going through. As you know, you have a whole group of new friends
    pulling for those RPE cells to do their job and improve your vision. We
    all hope that will be the outcome.

  • http://www.facebook.com/ryanplume Ryan Plume

    Hi Maurie!

    I have to say that I sort of agree with the original poster of this comment although I feel a patient blog is a GREAT idea and I support it. I’d much rather read results from a patient perspective than that of a corporate press release – it’s much more meaningful to me. However, I also feel that more eyes are on you than you probably think and that your potential impact on the company is incredibly substantial. With 100k being the suspected dose for commercialization and you being the most “vocal” patient, right now a misconstrued sentence or an ill-timed blog post when ACTC isn’t prepared could have a big impact on both the stock and the company since you’re essentially the center of attention at this point. It’s obviously entirely up to you and within your rights to do as you wish, but I personally feel it would benefit the science, the company, and the trials if a patient blogger worked intimately with the company in regards to any results, data, or blogs released to the public. This is a critical time in ACT’s trial process and history, and you are a critical patient with not only stock holders, but also future patients, potential partners or licensees, and even big pharma all likely reading into every post that you make.

  • Rocky

    Ryan, I forgot to mention….

    While Maurie has responsibilities regarding the information she shares.

    We also have a responsibility to use it wisely and to keep in perspective she is one patient.
    Facts are facts, using them to illuminate the big picture is the collective responsibility of all.

    Thanks again for your interest.

  • Sarah

    Hi. So have you noticed any change in your eyesight lately?I’m really hoping this will be a cure some day because I’m dieing to drive but my vision is to bad. I think these stem cells have lots of promise for the future for all of us.

  • Sam

    Hi Maurie,

    It’s been a while after your treatment for stargartd. I am hoping and wising your vision has improved dramatically. I didn’t notice any updates to this thread or any where else.

    Please share us your experience so far. It is very much appreciated.

    -Sam

  • Maurie Hill

    Hi Sam,

    I don’t notice a change in vision. I will be posting a one year overview and update next week. Meanwhile, here are all the articles I’ve written so far that mention my stem cell clinical trial participation:

    http://www.aisquared.com/blog/?s=stem+cell

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