When we first met, you were, to put it kindly, not interested in
science or health or, for that matter, history or politics. I would
describe you then as something of a literary bohemian, very knowledgable
about contemporary poetry and music and art, and generally aware
of recent social and cultural events. But I'd have to say your disinterest
in the world of science and technology bordered on disdain. That's
all changed. I assume your interest in scientific ideas and discoveries
is connected to your need to learn about what's been happening to
Okay, so I was an artsy semi-ignoramus. Two factors, besides aging,
inspired new intellectual enthusiasm: becoming legally blind; and
achieving Internet and Web access to broader, deeper knowledge.
I discovered an online forum, the RP List, where science professionals
like Dr. Gislin Dagnelle of Johns Hopkins and Foundation Fighting
Blindness information specialist Tom Hoglund contribute details
about retinal degeneration and the efforts to analyze and cure it.
My interest soon extended to the mechanics and neurology of vision,
cellular biology, stem cell and gene therapy research, and more,
abetted by another subscriber who generously compiles each Tuesday's
New York Times Science News as a text file and distributes it free.
But the lure of the Web is strong, and one thing leads to another,
given enough solitude and time, which circumstances have afforded
me in spades. Thus, a general erudition, however modest, beyond
my youthful imaginings, in many areas.
So, your need to know about what was going wrong inside your
eyes led you to scientific explanations, which in turn led to more
and broader interest in science. Is that kind of knowledge--the understanding
of how things work--of much practical value to you? Or is it mostly
the pleasure of learning and understanding?
"Practical value?" You mean some way in which my interest in the causes
and mechanics of retinal degeneration, and about research, could lead
me to a cure or at least uncover palliative measures to slow down
the progression of my RP and lessen its effects? It's not that I didn't
have such hopes at first. But it wasn't long before I understood that
the work toward finding a cure was advancing haltingly, due at least
as much to funding shortfalls as to the complexity of the scientific
challenge. And treatments such as vitamin therapy and nutritional
supplementation turn out to be of questionable value. Regardless,
becoming aware of developments in such areas as cellular biology,
genetics and neurology has added to the store of knowledge that increases
my understanding not only of my particular situation but of all living
things. A large profit, cure or no cure.
That's part of what I meant. But to be honest, without much conscious
thought I've been harboring a glib little psychological theory that
goes something like this: Losing your vision must make you feel a
general loss of control over your life; understanding the physiology
of how that came about might help restore some sense of control. I
don't mean to reduce all of your fascination with the beauty and elegance
and complexity of nature to pathological compensation, but, well,
is there anything to it?
That's not at all glib. Being struck with the statistically rare genetic
defect or defects that cause retinal degeneration, and losing my visual
grasp of the world, is a continual, frightening confrontation with
chaos. My interest about the particular pathology certainly sprang
from a need to feel some kind of psychological mastery over the chaos,
even if medical control was impossible. And my appreciation of the
general biological world opened out from there: the clinical stories
of neurologist Oliver Sacks, the Human Genome Project, the discovery
of deep-water marine creatures whose entire body surfaces are their
eyes, the versatile potentialities of stem cells, the myriad pathways
of evolutionary development. None of which might have grabbed my attention
before, and all of which now provides for me no less than a new window
on life. Of course, I could have paid attention before, but didn't.
So it goes.
What is your understanding of the current state of research on retinal
degeneration? Do they know what genes are involved? Is there much
promise of a medical cure, or of a mechanical device that would replace
a defective retina, in our lifetime?
The active research is running in several complementary directions.
The genetic research has so far identified only the first few of the
many genes that are thought to cause the numerous forms of retinal
degeneration collectively known as RP. The hope, of course, is early
diagnosis and prevention, or cure by way of gene therapy techniques
already in use. Surgical transplantation of retinas seems to hold
little promise, as hooking up the retina's millions of photoreceptor
cells to the optic nerve is like trying to hotwire a car with an ignition
system as complex as the Universe. On the prosthetic side, there are
implanted microchips with mini camera image feeds under development,
as well as other ideas for artificially stimulating or approximating
vision. Much of this is promising, but I wouldn't dare to predict
outcomes. Too many variables.
But from what you said earlier, it sounds like you're not optimistic
that a remedy will come along in time to restore a significant part
of your vision.
Sure, something might turn up, at any time. With scientific breakthroughs,
you never know. And, as long as I take good care of myself and don't
get exterminated by some random twist of fate in this increasingly
hazardous world, I'm likely to see some kind of remedy available,
before I die. What I'd prefer, of course, would be an organic solution.
Genetic therapy, stem cell photoreceptor replacement, or even surgery
involving tissue. Rather than the high-tech ocular equivalent of a
wooden leg. But I'm far from confident about anything coming along
while I'm still in my vigorous middle age, instead of in my frail
dotage. The research work itself is difficult enough under the best
conditions, and the fact that retinal degeneration is given such a
lower priority in terms of funding than less rare, more lethal diseases,
is a major drag on progress.
Do you find that others with vision disabilities share your interest
in science in general and vision research in particular?
I've had only a passing social acquaintance with others who have RP,
and my impressions come primarily from observing my fellow subscribers
to an Internet mailing list forum. I suppose these people constitute
a self-selected sampling of an afflicted population with a high average
level of interest in the science of retinal problems. That said, there
are differences of degree and differences of kind. All want to understand
something about why their retinas are dying and what, if anything,
can be done about it. But some are better educated than others, generally,
and accordingly the level of interest ranges from rudimentary to highly
sophisticated in terms of the details. Then there is the split, with
some overlap, between those who place their trust more in alternative
medicinečexpensive Russian and Cuban "treatments," nutritional supplements,
etc.čand those who look primarily to mainstream science for explanations
Have you ever tried any of those alternative treatments?
I haven't tried the Russian or Cuban treatments, and don't intend
to. Neither has been validated by published data based on scientific
research. The Russian approach, done only in Moscow, involved (I'm
not sure it's still offered) enhancing blood supply to the retina,
using surgery, ozone therapy to "clean out" the capillaries, and ENKAD,
an enzyme to improve certain chemical conversions required for light
reception. The Cuban method, offered at a Havana clinic, stresses
ozone and involves different procedures. Both cost thousands of dollars,
involve travel, and require repetition. Then there are claims that
a daily megadose of Vitamin A may retard the advance of RP (one published
study), that a dietary supplement called Lutein may save the macula
(central retina), and that others such as DHA and Omega-3 fatty acids
may also be of benefit. I take the Vitamin A and the Lutein, albeit
skeptically, just for insurance.