The Speed of Sound

Image representing the sound wave of an audio file.

On the recommendation of blog reader Margaret, I’ve been reading through Norman Doidge’s book, The Brain that Changes Itself. The book investigates neuroplasticity, the process by which the brain rewires itself and changes its physical structure in response to specific demands. There are a lot of interesting insights in there for people with sensory loss, and I’m already planning to write a longer post about it once I finish. 

In the meantime, I just wanted to share a short passage that caught my attention, and a few thoughts about it. Doidge writes:

I was at a dinner party with a friend, whom I shall call Emma; her writer husband, Theodore; and several other writers.

Emma is now in her forties. When she was twenty-three, a spontaneous genetic mutation led to an illness called retinitis pigmentosa that caused her retinal cells to die. Five years ago she became totally blind and began using a seeing-eye dog, Matty, a Labrador. Emma’s blindness has reorganized her brain and her life. A number of us who were at the dinner are interested in literature, but since she has gone blind, Emma has done more reading than any of us. A computer program from Kurzweil Educational Systems reads books aloud to her in a monotone that pauses for commas, stops for periods, and rises in pitch for questions. This computer voice is so rapid, I cannot make out a single word. But Emma has gradually learned to listen at a faster and faster pace, so she is now reading at about 340 words a minute and is marching through all the great classics. “I get into an author, and I read everything he has ever written, and then I move on to another.” She has read Dostoyevsky (her favorite), Gogol, Tolstoy, Turgenev, Dickens, Chesterton, Balzac, Hugo, Zola, Flaubert, Proust, Stendhal, and many others. Recently she read three Trollope novels in one day. She asked me how it might be possible for her to read so much more quickly than before she went blind. I theorized that her massive visual cortex, no longer processing sight, had been taken over for auditory processing.

Naturally this caught my eye, since I have RP and—like Emma—have taken to consuming most of my books in audible form. I too have increased the speed incrementally, and now listen at between 400 and 450 words per minute.

Anyone who has heard blind people use their phones knows this is pretty common. The advent of smartphone technology in the decade since this book came out has improved text-to-speech synthesis by leaps and bounds over the old Kurzweil machines. It is cheaper and more widely available, and the voices are more pleasant and natural-sounding, clearer and easier to understand at higher speeds.

Now that I’m used to it, I actually love using my phone to listen to books. For one thing, the high rate of speed keeps me engaged and focused. I know exactly how long it will take to read a book, because my reading app tells me, down to the second. And It makes note-taking easy—I just pause, highlight the text I want, and export all of my highlights once I’m done with the book. 

But there’s good news for those of you whose eyes still rule your brains: this is not some kind of blind superpower, and you can learn it too. I know, because my wife Kristin does it, and her vision is completely intact. She found herself getting impatient with the pace of audiobooks and podcasts and, since she knew I listened fast, she started increasing the speed. Now she listens to everything sped up anywhere from 1.5x to 2x normal speed, depending on what it is and what else she is doing at the time.

You can always learn new skills, even if large tracts of your cortical real estate haven’t been recently vacated. Much of the rest of Doidge’s book discusses how regions of the brain can be recruited for novel tasks, at the same time as they continue to do their old jobs. So it seems hiss explanation here is overly simplistic. Blindness had certainly prompted changes in Emma’s brain, but some—like fast-listening—could have happened even if she was sighted.

Brain function is not a zero-sum game, where gains in one domain can only happen at the expense of another. We haven’t found—or even gotten close to— the limits of the human potential to learn. We don’t know how many skills a person can master, or to what depth. The brain is glorious in its flexibility and ability to change, at any age and in any circumstances..

I’m excited to share more from this book and a few others I’ve read recently in the same vein, they all tend to show the benefits of that hard work I wrote about last week. The grueling work of learning to live again without sight not only allows us to lead happy and fulfilling lives. but could protect the health and longevity of our very brains.

Stay tuned!

The Hardest Part

There’s a saying among people with Retinitis Pigmentosa: “Being blind isn’t hard. Going blind is hard.” I’m not sure that being blind is easy, exactly, but I do believe that this season, the transition between sight and blindness, will probably be one of the hardest of my life.

Part of this is the basic sense of loss, and loss of control. Something I once had is disappearing, and there is nothing I can do to stop it. I don’t know—can’t know—what I will be able to see next year this time, or five years down the line. It’s hard to literally watch my vision spark and fizzle away. 

But that sense of loss isn’t really the hardest part. The hardest part is just how much work it is to go blind. Because I’m not just losing my sight; I’m losing all of my habits, routines, and processes that rely on it. 

Every time I use my eyes to locate something, track something, or assess something, to organize or to remind myself of something. Every task that relies on even the tiniest bit of visual information-gathering. All of them have to be reconsidered and relearned.

Because of course there are different ways to do most of these things. I can replace my lost abilities, can replace the visual information with information from my other senses or my pieces of assistive technology. But it means changing the way I’m accustomed to doing almost everything I do.

It means adaptation, and adaptation is hard.

Learning—at every turn, in every part of life, every day—is exhausting.

And demoralizing. All of these things I’m learning? They are things you’re supposed to learn as a child, not in your thirties. Actually, they’re things I did learn as a child. I learned them at two months, when I smiled back at faces that smiled at me, at three months when I grabbed a toy and shook it for the first time. I learned them  when I sat up, crawled, took my first steps. When I learned to read the menus on our first computer, and went from there to reading chapter books, novels, and a list of languages stretching back to the dawn of history.

I learned them over the course of a lifetime, one skill building on another. Skills so basic and so foundational we barely think of them as skills at all. Skills that need to be mastered to the point of effortlessness so we can focus our energy and attention on the next, greater height. Now much of that knowledge and skill is becoming useless. 

Many days, I feel like I’m spending all of my energy digging myself out of a hole, or bailing out a leaky boat. Like I’m running as hard as I can just to get to the starting line—never mind the finish line. 

I miss the feeling of progress, the feeling that the work I’m doing is moving me forward, instead of just keeping me from falling behind, or falling apart. 

And when will it end? RP is a long and slow disease. I’ll be living in this process for years, for decades even, weaning myself off of vision in bits and pieces, one task, one process at a time.

It will be hard, grueling at times, but the alternative would be much, much worse. Refusing to face the difficulty head-on and avoiding the hard work would lead somewhere worse than daily exhaustion. It would lead to stagnation and despair, and that’s something I refuse to tolerate.

So I’ll keep working, facing the days when simple tasks become suddenly arduous and stressful, finding new ways to do them, and then not giving up until they are mastered again. And hopefully, with each new adaptation and each new skill, it will get a little easier, until someday I reach that place where I can say “Going blind was hard, but being blind isn’t.”

My Vision in Experiential Terms

(This is part two of a series on my vision at the start of this blog. To read the first part, see My Vision in Medical Terms.)

Image of an old TV set. It is displaying fireworks. The color is muted and the picture is grainy. A metaphor for my vision.

In the last post, I described how my photoreceptor cells are dying one by one and in turn taking with them what remains of my vision. I wrote that photoreceptors are like the “eye’s pixels,” and some of you may have imagined that a retina full of dead photoreceptors looks like an iPhone screen full of dead pixels. It would be nice if that were the case, but the truth is much more bizarre.

The Low-Res Retina

The pixel analogy holds in one important respect: fewer cones and rods mean less detail, just as fewer pixels on a screen do. In a sense, I was born with lower resolution vision than most. Think of an old, first-generation color TV from the 1950s. Broadcast resolution was low, and the images that showed up on these sets were blurry and indistinct. In this analogy, people with normal vision are like the latest generation of iPad, which have about nine times as many pixels as a broadcast TV. The difference is stark.

Comparing retinal resolution is the concept behind the common method of expressing visual acuity as “20/xx.” This method compares the distances at which two people can see the same object. The first “20” represents the patient, or more precisely, text the patient can read from 20 feet away. The second number is the distance from which a normally-sighted person can read the same text. Since people with a higher resolution can see something clearly from further away, the second number is usually higher for RP patients.

The last time I had my eyes checked, about a year ago, my right eye (which is my better eye) had an acuity of 20/250, meaning I can read at twenty feet what a normally-sighted person can see at 250 feet. To put this in more concrete terms, if there was a sign on the goal line of an American Football field, and I could read it from the 10 yard line, a normally-sighted person could still read it while sitting in the stands behind the opposite goal post. Keep in mind that this is with glasses.

Mind the Gaps

Poor resolution isn’t the only problem with my vision. There are also the gaps left by groups of dead photoreceptors. On a phone screen, dead pixels don’t give up their spot. They leave little black squares, constant reminders that there is a small part of the image you cannot see. The brain, on the other hand, doesn’t much like admitting there are gaps in your vision. It tries to fill in these gaps as best it can to create a cohesive picture of the world.

Have you ever tried that experiment where you use your finger to find your blind spot? You close your left eye and stare at a dot or letter or something on a piece of paper. Then you put a finger on the dot and slowly move it to the right, while keeping your eye on the dot. When your finger gets an inch or two away from the dot, it disappears!

This happens because everyone’s retinas have a barren spot with no photoreceptors where the optic nerve leaves the back of the eye. Your brain doesn’t like to let on that it’s missing anything, though, so usually it fills in the hole with the image from your other eye. Even when one eye is closed, you don’t see a big blank space, Your vision seems continuous; your finger is just missing.

Now imagine that, instead of one blind spot in each eye, you had a few dozen spread out across your visual field. Some are big, some are small, but your brain tries to hide them all.

I have a lot of gaps in my vision. In fact, my last visual fields exam said I had “central vision with peripheral islands,” meaning it might be more gap than vision. Most of these, my brain tries to hide from me. When I try to read long words with my eyes, some letters will often just not be there. I’ll have to look again or move my eyes slightly to see what I’m missing. The gaps across the bottom of my visual field explain my antagonistic relationship with “Wet Floor” signs, which have cause me much more grief than wet floors themselves ever have.

One hole has finally developed that my brain cannot hide. It is located near the middle of my vision in my left eye, probably spreading out from around the optic nerve. It looks like a smudge of something  on my glasses, with blurry edges that fade into clearer vision. It is always there, but I think my brain still tries to hide it away. In the daytime it is white or light grey, but at night it goes dark. It’s not very good camouflage, but it still tries.

The Fireworks

A screen with dying pixels fades into darkness, and many people think of blindness the same way, as vision fading into darkness. The Bard himself described “looking on darkness which the blind do see.”

Sorry Shakespeare, but going blind from RP is a constant chaos of color and light,

I mentioned some alarming visual phenomena in the last post, which I started to notice when I was working at an archaeological site in Israel. After a full day of digging in the blazing sun, we would go back to the air-conditioned hotel to wash up and eat. In the dim, cool room I would notice intricate, tightly-packed patterns that flashed and twinkled in my left eye. Sometimes it would look like the pattern of bumps on a basketball, sometimes like raindrops on glass. Other times it looked like a repeating tile mosaic, In every form, the pattern would flicker and flash brightly and gradually fade.

To this day, my left eye provides a near-constant psychedelic light show. The right has joined in, just a bit tamer. The patterns are still there, joined by other flashes, sparks, and twinkles. Electric whips crack across my vision. Fireworks blaze and pop, sometimes so large they blot out everything else. My entire field of vision strobes very quickly from bright red to bright blue, like I’m staring at flashing police lights from about six inches away. It must be a real party in there, because someone even turned on the smoke machine. Mist covers everything some days, like a thick morning fog.

The colors are brilliant, even beautiful, but of course it’s not a party. It is the anguished thrashing of half-dead photoreceptors, the aimless firing of bored and bereft neurons, the brain trying to synthesize an image when the signal is lost or senseless. It changes every day, every hour, every minute. I don’t know today what the world will look like tomorrow, this morning what it will look like in the afternoon, at 10 AM what it will look like at 10:05.

What I see—what I can and can’t see—is impossible to predict, difficult to communicate, and risky to trust. Vision is still useful, and many days I am grateful for what I have left, but it is not as useful as it once was, and far less dependable. I can no longer rely on my old ways of doing things. I have to change and adapt to keep moving forward through the mist and the fireworks..

Appendix A: Videos!

As I was writing this, Kristin reminded me of a video I had showed her that she found really helpful in understanding what RP looks like.

Aaron Morse made this video called “How I See the World with my RP Eyes” (bonus: cute baby!). His tunnel vision is a more classic pattern of RP than I have, but it gives a good general idea. Another YouTuber simulated some of the lights he sees in “My RP Visual Flashes.” I found it interesting that he too sees tightly-packed repeating patterns. Unlike mine, his are very angular.

(Photo credit: King-of-Herrings)

My Vision in Medical Terms

(This is Part One in a series of posts about the state of my vision at the start of this blog. The next post will deal with my personal experience of my vision. There’s a lot of technical medical information in this post, but I’ll try not to make it too boring.)

I have had very poor eyesight since birth—poor enough that I was deemed “low vision.” I got my first pair of glasses before I was two. No one could identify exactly what was wrong with my eyes, though, and not for lack of trying. My parents carted me around to practically every expert in the state of Colorado, and none of them could provide an answer. To all appearances, my eyes were perfect; I just couldn’t see well. The closest approximation of the truth was probably given by Dr. Alexander, my favorite childhood optometrist, who said “You may just have been born with fewer cones and rods [retinal photoreceptors] than other people.”

For the first thirty years of my life, this was about all that could be said about it. My vision was poor, but it seemed at least to be stable. In 2012, though, while I was working at an archaeological excavation in Israel, I started to notice strange visual phenomena that prompted me to visit an ophthalmologist when I returned home to Massachusetts. This ophthalmologist gave me a preliminary diagnosis of Retinitis Pigmentosa and referred me to the Massachusetts Eye and Ear Infirmary for confirmation.

The doctors at MEEI took high-res photographs of my retinas and subjected me to a battery of tests, including color discrimination, visual fields, dark adaptation, and the notorious ERG. The ERG, or Electroretinograph, measures the electrical activity of your retina (like an EKG for your eyeball. You put on a giant contact lens with electrodes implanted in it, and stare at a strobe light for what feels like an hour (probably five minutes). People with healthy eyes produce a graph with a nice wave of ups and downs. Mine? Flatline.

A flat ERG response is the hallmark of advanced Retinitis Pigmentosa. There’s no measurable electrical activity going on in the retina, but most of us can still see to some extent. As the doctor told me, “it’s like there are people who don’t register a pulse, but they’re still up and walking around.”

So what is this strange form of retinal zombiism known as Retinitis Pigmentosa?? There’s a boilerplate description that appears with a little variation across the internet, and goes a little something like this:

Retinitis Pigmentosa (RP) refers to a group of inherited diseases causing retinal degeneration. The cell-rich retina lines the back inside wall of the eye. It is responsible for capturing images from the visual field. People with RP experience a gradual decline in their vision because photoreceptor cells (rods and cones) die.

(I took this version from the article at http://www.blindness.org/retinitis-pigmentosa — the rest of the article is quite good also, and the Foundation Fighting Blindness is an excellent resource for learning about retinal disorders and new research)

Photoreceptor death explains the diminished ERG response. Fewer cells means less electrical activity, and a weaker signal traveling from the eyes to the brain. You can imagine these photoreceptor cells as the eyes’ “pixels”—the fewer there are the less detail there is in the image. Healthy eyes contain over 120 million photoreceptors—way too many for the optic nerve and brain to handle at once, so much of the signal is just discarded. For people with RP, vision loss only begins once the number of photoreceptors falls below the maximum number the brain can interpret at one time.

A hand-drawn graph showing the loss of photoreceptors over time in RP, as it relates to the capacity of the optic nerve and brain. There is a horizontal line representing the amount of input the optic nerve and brain can handle at one time. A second line starts in the upper left and slopes downward. It crosses the horizonatl line about halfway and ends in the lower right. This line represents the number of photoreceptors that someone with RP has, which decrease across their lifespan.

Graph of photoreceptor loss in RP

This explanation is a bit simplistic, since degradation does not occur evenly across the visual field. Most people with RP develop dead spots in some areas of their vision, while other areas remain relatively clear for much longer. In the majority of patients, degeneration happens from the outside of the retina in. They lose their peripheral vision and night vision first, and only lose their central vision in the final stage of the disease.

Since my vision has always been poor, I was probably born with a reduced number of photoreceptors across the board, as Dr. Alexander surmised so many years ago. That means my graph might look more like this:

A second hand-drawn graph representing my personal case. The same horizontal line is there representing the brain/optic nerve capacity, but this time the line representing the number of photoreceptors I have starts below the line on the left and slopes gradually downward toward the bottom right.

Graph of how I imagine my photoreceptor loss

Now more of my photoreceptors are dying, but not in the usual pattern. I am losing cells from the center of my retinas outward. My detailed central vision is going first, and the periphery will follow.

It may seem strange that I stray so far from the norm, but this leads to an important point: RP is not one disease, but many.

When I first started seeing that standard description of RP four years ago, it did not mention a “group” of inherited diseases, but in the past several years it has become very clear just how many forms RP can take. It can strike at any age, progress at any speed, and carry with it a number of other symptoms. Some of the nastier varieties, like Usher’s Syndrome, cause deafness as well as blindness. They all culminate in the death of photoreceptors, but can differ substantially in how long this takes and the path they take to get there.

The variety in RP patterns probably results from the variety of its causes. It is commonly considered an inherited genetic disorder, but the details get pretty complicated. Genetic testing of RP patients has linked the disease to mutations in more than 250 genes at this time, and that number keeps growing. Certain mutations are quite common and well-understood, while others are rare and more tenuously linked to disease symptoms.

With this many genetic links identified, it is a startling fact that the genetic cause of RP cannot be determined in about 45% of patients. Almost half of patients’ RP is caused by either an unidentified genetic factor or something else entirely. This is my lot—despite the cutting-edge genetic testing I received from the Ocular Genomics Institute in Massachusetts, a genetic cause for my RP has not been found. 

It is possible that an as-yet-unidentified gene mutation or combination of gene mutations is causing my vision loss, but it could also be something completely unrelated to genes. No one else in my extended family has suffered from any similar malady, which points away from an inherited cause. It could have been some accident of development in the womb, a vascular event that starved my retinas of oxygen or nutrients at a critical moment. At this point, who knows?

So have I learned anything from being diagnosed with RP? I have confirmed the suspicion that I don’t have enough photoreceptors, and have learned that more are dying every day. I’ve learned that this condition is progressive, and my vision will continue to deteriorate. I have passed the boundary into legal blindness, and without medical treatment I will end up totally blind at some point in the future. This won’t happen right away—my last photoreceptor may not blink out for another twenty or thirty years— but with the current state of technology, it is inevitable.

So it’s a good thing technology changes. This diagnosis has also clued me in to a world of exciting research on retinal deterioration and rejuvenation. There is nothing on the market right now that can halt or stop what is happening in my eyes, but research teams are working on treatments and cures using gene therapy and stem cells. All of these are still at least 5 to 10 years away from widespread deployment, but clinical trials are going strong. Hope may not be right around the corner, but it is on its way. 

 

(Note: The information in this post comes from my research on the topic of RP and from personal conversations with retinal specialists, I am not an expert in the field, so there may be a few inaccuracies. I alone am responsible for all of them and I welcome corrections!)

C[ong]ratul[ation]s!

Eric reading Braille

“Congratulations, you have completed the study of contracted Braille!” said the dots to my fingers earlier this week. Or rather, “C[ong]ratul[ation]s, [you] [have] [com]plet[ed] [the] [st]udy [of] [con]tract[ed] brl!”

I’ve been studying Braille for a year. I learned Grade One quickly and easily enough — it’s what most people think of when they think of Braille, where each cell represents one letter or one punctuation mark. Grade Two, or contracted Braille, is another story. Various contractions are used to shorten common words or series of letters, so one or two cells can represent two, three, four or more letters. In the quote above, everything within brackets is contracted. There are dozens of these contractions, and many of the signs play multiple roles, depending on context. So Grade Two Braille took a little longer, due to its complexity and, well, life getting in the way.

I feel proud of this little milestone (and relieved that there are no more lists of brain-breaking contractions left to learn), but I also know I’ve got a lot of milestones left ahead of me.

I am slowly going blind, and slowly learning to be blind and work as a blind scholar. I am not at the very beginning, but neither am I anywhere near the end. I have a long path ahead of me as I gain the skills I need to conduct my research, finish my dissertation, and teach what I have learned.

I know all the contractions now, but I also know I need to speed up. I timed myself to see my current pace: sixteen minutes and twenty-five seconds for one page—just shy of fourteen words per minute. It’s not bad for a beginner, but I feel like a six-year-old. I want to fly through academic prose; instead, I’m struggling through the simple stories in my Braille primer.

So now I am shifting to work on speed and technique. “Elite” Braille readers usually read around 130–150 words per minute, and I’ve heard rumors that some have reached 400 words per minute. They use three fingers on each hand, reading with both hands. I have a lot of work ahead to master Braille, and that is just English Braille. I will probably end up using it for German, French, and Hebrew as well.

The state of my Braille is much like the state of my journey into blindness as a whole. I’ve made progress, but there is still a lot of work and learning to do. I’m starting this blog in the middle, not the beginning. I hope to make it a space to share the process— not only with Braille but with all the other strange adventures of blind scholarship: exploration, experimentation, collaboration, frustration, and hopefully a few moments of exhilaration. I’ll get into the nitty gritty of multi-lingual Braille reading, my quest for the perfect word processor, adventures with assistive technologies, and much, much more. I’ll also use the space to share more general thoughts on life, blindness, my research, and everything else besides.

Please read along and tell me what you think. Whether a lifelong friend, another blind person on a similar path, or just a curious stranger, I look forward to hearing from you!

(Addendum: As of January 1, 2016, Level One and Level Two Braille are outdated terms. The new Unified English Braille standard is now the most prevalent form of Braille, as it combines and streamlines literary and computer Braille codes. The primer I used to learn Braille used the old system. Those in the know may have noticed the [ation] abbreviation, which no longer exists in UEB.)