[Ohio-Talk] Great work.

[Richard Payne]

Christopher , this was great work and it was my pleasure to read knowing
that you were a member of our affiliate.

Optic Nerve Hypoplasia: Unraveling the Mysteries
by Christopher Sabine


>From the Editor: In the National Federation of the Blind, we seldom focus on
specific conditions that lead to blindness. What truly matters is the fact
that a person's vision is low enough to require nonvisual methods for
carrying out tasks that typically are performed with the help of sight. In
some instances, however, a particular diagnosis may involve a constellation
of disabilities in addition to blindness. Such disabilities can pose some
unique challenges. In this article Christopher Sabine looks at one of the
most prevalent causes of blindness in children in the U.S., optic nerve
hypoplasia, or ONH. Chris has been a member of the NFB of Ohio since 2014,
and he serves as secretary of the Cincinnati Chapter.
As a blind adult and a member of the National Federation of the Blind, I am
amazed by the diversity within our organization. We represent a wide variety
of cultures, and we come from all walks of life. We have a tremendous
capacity to absorb new ideas and perspectives.  
The Federation promotes such skills of blindness as Braille, assistive
technology, and independent travel with the long white cane. In the
Federation we also encourage a set of daily living skills and social skills
necessary for blind children to become successful, productive adults. We
recognize that a confident, positive attitude, willingness to take risks,
and the ability to adapt to change are all essential for a blind child to
grow into a successful, competent blind adult. 
Blind children are as unique, wonderful, and complicated as any other
children. However, I believe that some blind children experience
developmental challenges that are not related to their blindness per se, but
are a part of the underlying condition of which blindness is a
characteristic. Some children who receive (or do not receive) blindness
instruction in the educational system also have autism, behavioral
disorders, chronic health conditions, or developmental disabilities. These
additional challenges can have a huge impact on the ability of these
children to learn the skills of blindness and thrive in the general
educational environment. I believe it is vital that we do all that is
possible to enable these children to learn the values and skills they need
in order to live the lives they want. As an example, I would like to share a
bit about my own life. 
I was born with optic nerve hypoplasia (ONH), which is the underdevelopment
of one or both optic nerves during pregnancy. Once considered a rare
disorder, ONH is now one of the leading causes of childhood blindness in the
United States and some other Western countries. Optic nerve hypoplasia is
typically bilateral, affecting both eyes, and this is true in my case. The
condition can also occur in one eye, known as unilateral ONH. 
The underlying process that leads to the birth of a child with ONH also can
affect adjacent structures of the midline brain. Therefore, children with
ONH may experience a range of medical complications, developmental delays,
and behavioral characteristics. According to a leading article published by
the Keck School of Medicine at the University of Southern California,
between 60 and 80 percent of children with ONH have deficiencies in their
ability to produce one or more vital hormones. These include growth hormone
and thyroid hormone, which are both essential for growth and metabolism, and
cortisol, which enables the body to respond properly to physical or
emotional stress or illness. Children and adults who are unable to produce
these and other hormones must be seen regularly by an endocrinologist, and
most require daily hormone replacement. 
According to the same article, 71 percent of children with ONH exhibit
developmental delays. Global developmental delays, sensory processing
disorders, sleep disturbances, attention difficulties, and seizure disorders
are common in children with ONH. However, these conditions are seen much
less frequently in adults with this diagnosis.
Sensory processing disorder (SPD) is a catch-all term used by occupational
therapists and other health-care professionals to describe difficulties
processing input through the senses. In my case, sensory processing issues
made learning Braille an arduous task. Furthermore, I did not master such
fine-motor skills as tying shoes and buttoning shirts until I was in my
teens. Food aversions are common in people with SPD, often related to the
inability to tolerate certain food textures. SPD may also involve delays in
the development of oral-motor movements such as the ability to swallow solid
foods. For further information about sensory processing disorders, I
recommend Carol Stock Kranowitz's comprehensive and easily accessible book,
The Out-of-Sync Child.
Recent studies as well as anecdotal reports from families and professionals
also point to an increased incidence of autism spectrum disorders and
autism-like behaviors in children with ONH. However, the assertion that
blind children with ONH are prone to autism is problematic. No clinical
screening tool is available for diagnosing autism spectrum disorders in
blind children. Nearly all screening instruments used to test for autism
spectrum disorders were developed based on a population of typically sighted
children. Furthermore, a high percentage of children on the autism spectrum
are considered to be primarily visual learners, based on diagnostic and
testing criteria established by sighted, typically developed clinicians and
evaluators. Our understanding of how blind children process information
using brain structures typically associated with visual processing is in its
infancy.
When a blind child rocks, engages in constant, rhythmic hand-flapping, or
repeats words or phrases in a sing-song voice, we cannot say with clinical
certainty that the child's behavior indicates autism. The symptoms may be
related to factors that are specific to the experiences of a blind child.
For this reason, some clinicians are reluctant to diagnose autism spectrum
disorder in a child who is blind. Nevertheless, studies suggest that 46
percent of children with ONH display significant autistic behaviors or could
qualify for a clinical diagnosis of an autism spectrum disorder, independent
of visual acuity. This finding suggests a neurological basis for the
presence of a clinically significant autism spectrum disorder in many
children with ONH. 
Children with ONH often need intensive services from disciplines such as
occupational and speech therapy as well as the more typical instruction in
Braille, cane travel, and assistive technology, to alleviate developmental
delays or behaviors associated with the autism spectrum. These services are
insufficient or nonexistent in most school systems.  
When I was born my family had no knowledge of the autism spectrum or of
sensory processing difficulties. When I was about six months old, I began
looking directly into the sun, and my parents suspected that I was blind. My
mother took me to a local ophthalmologist, who diagnosed me with ONH. While
I understood what my mother was saying at ten months, I did not speak until
I was three years old. My mother remembers that my speech delay was due to
my difficulty coordinating the muscles of my mouth to produce intelligible
sounds. Such speech delays are not uncommon in children with ONH. When I
finally did speak, I spoke in complete sentences. 
For as far back as I can remember, I was obsessed with certain sounds and
with topics related to sound and music. My primary interest was the sound of
telephone switches from different exchanges. (This was back in the days of
rotary dial and touch-tone land lines.) I learned every area code in North
America, and I could tell where a person was calling from by the sounds of
the local and long-distance switching equipment. I spent hour upon hour
dialing numbers and listening to the sounds of calls as they traversed the
network. I know of a few blind people who gained some notoriety for this
area of expertise, but obsessing on telephone exchanges was not a way for me
to make many friends. This didn't matter much to me, though, as I had no
real interest in interacting with kids my own age. 
I also loved beeping electronic games, and I spent hours playing with my
electronic toys. One of my favorites was the Speak and Spell, a handheld
educational toy designed to help children learn spelling. A synthetic male
voice would speak a word, and you would spell it on a keyboard. Add-on
modules contained more challenging vocabulary and other games. In short, I
craved anything that gave me immediate auditory feedback-especially if it
was musical or electronic. Indeed, my favorite word throughout much of my
childhood was "beep."
I had perfect musical pitch and learned to play the piano when I was four.
However, I lost my musical skill after I started attending first grade at my
local elementary school. I also had an exceptional autobiographical memory,
and I could compute difficult math problems in my head. 
Braille and fine motor skills were another matter entirely. I attended a
preschool and kindergarten program for blind students at a public elementary
school, and I had one of the best TVIs (teachers of the visually impaired)
in our state. Nevertheless, my first attempt to learn Braille was a
nightmare. The letters on the Braille page felt rough and abrasive, and
remembering how dots were supposed to form letters was laborious. Processing
the Braille code was a tedious, frustrating ordeal, and I physically
resisted my teachers' efforts to run my hands over the Braille page. This
reaction is sometimes referred to as tactile defensiveness. It is common in
children with ONH due to delayed information and sensory processing. I
remember one incident where I became so frustrated trying to read the word
horse that I had a crying meltdown and refused to cooperate with my teacher.

Like many children with ONH, I also dealt with behavioral challenges and
difficulties with self-regulation, some of which I still face to this day.
My behaviors included rocking, hand-flapping, picking at scabs, and crying
when I was frustrated, anxious, or overstimulated. Although I have outgrown
many of these behaviors, impulse control and behavior management still take
conscious effort. 
My family and I decided to abandon Braille about midway through my
first-grade year in favor of a closed-circuit television and recorded books.
Through high school I attended regular education classes in my local school
district, where I was the only blind student. I was the school's human
calculator, but reading print for any length of time was frustrating and
physically exhausting for me. While I had a few friends in grade school, I
had trouble relating to peers-particularly if the play was not on my own
terms. 
I received occupational and speech therapy as well as orientation and
mobility instruction until I was in eighth grade. I also attended a summer
youth program for three years at the Clovernook Center for the Blind and
Visually Impaired. There I learned most of the daily living, communication,
and travel skills I use to this day-including dressing, tying my shoes, and
using a long white cane. I learned to use our local bus system, and I was
introduced to screen readers and other assistive technology.  
During my freshman year of high school, after I had finished my third year
in the summer youth program, something curious happened. I noticed that many
fine motor skills, such as plugging in appliances, were suddenly much easier
for me to perform than they had been in the past. It was as if I had
downloaded external software into my brain that enabled me to perform these
tasks. I finally learned Braille in much the same way. I sought out Braille
instruction the summer after my freshman year of high school from a local
agency for the blind as part of a state program for blind youth
transitioning to employment. I found that I could read the first ten letters
of the Braille alphabet with ease after only one day, and I learned the rest
of the alphabet within a month. Memories from my attempts to learn Braille
in kindergarten and first grade came flooding back, as if my brain finally
had become able to process my earlier experiences. With the support of a
blind colleague who is now a teacher of the visually impaired and a leader
in our state NFB affiliate, I began reading literary Braille and finished my
first Braille novel, J.D. Salinger's The Catcher in the Rye.
While I still deal with aspects of ONH every day, I live as active a life as
I can. I graduated cum laude from college and earned a master's in social
work. I worked for three-and-a-half years as a service coordinator, helping
youth with chronic health conditions and disabilities transition out of high
school and develop plans for their futures. I now operate a consulting firm
specializing in educational support and community referrals to families of
children with ONH. In my business I apply the knowledge and skills I learned
from my professional training and my personal experience as a blind adult
with ONH.  
Most children with ONH have the potential to lead successful and fulfilling
lives, despite a fairly complex set of challenges. I am shocked and saddened
at how many children with ONH are placed in segregated classrooms for
children with more significant developmental disabilities. These placements
are wholly inappropriate for the majority of children with ONH, who have the
capacity to achieve far beyond the expectations of many of the professionals
who work with them. 
I believe that children with ONH and other etiologies of blindness can
benefit from many of the educational supports geared toward children on the
autism spectrum. These supports emphasize social skills, reading and
reacting appropriately to nonverbal cues, and managing or eliminating
socially unacceptable behaviors. For me, learning to conduct myself in a
socially acceptable manner has taken an ongoing, conscious, and concerted
effort.  
How can the National Federation of the Blind promote equality, security, and
opportunity for children and adults with ONH, or indeed, for all blind
people who have disabilities in addition to blindness? We have amassed a
comprehensive body of knowledge of best practice in teaching and mentoring
blind children and adults. We have found ways to encourage tactile fluency
and Braille literacy, and we have developed and advocated for equal access
to information. We have championed the use of structured discovery for
travel in a wide variety of unfamiliar and complex environments. All of this
knowledge has been developed and disseminated by the blind speaking for
ourselves. However, though the Federation has a division for people who are
deaf-blind, knowledge about how to raise, educate, and foster the
development of blind children with other disabilities is still very limited.

I believe we need to educate ourselves about the most effective strategies
to help people with a variety of medical, behavior, and learning
characteristics. We must find ways to help them develop the skills and
attitudes they need to achieve their dreams. We have made monumental strides
to develop and advocate for the supports necessary for blind people, but
don't let us forget the blind child who operates on the autism spectrum or
the child who needs extra support to learn Braille. Let us help the blind
child who has trouble interacting with blind or sighted peers. 
Blind people with other disabilities can teach us a great deal about
alternative means of living our everyday lives, and about blindness itself.
I believe it is important that we collaborate with professional and consumer
organizations that serve other disability groups. From children and adults
with disabilities such as autism and complex medical conditions we can learn
a great deal about how people with diverse experiences develop their
capabilities.  
Finally-and most importantly-I believe that we need to promote high
expectations for all children and adults who are blind, including those who
have other disabilities or alternative learning characteristics. One of the
great guiding philosophies of the National Federation of the Blind is that
blindness is not the characteristic that holds a person back. We live this
philosophy every day of our lives as Federationists, and we instill it into
all the work we do. We view blind people as inherently capable of benefiting
from education, engaging in fulfilling careers, raising families, and
enjoying all that life has to offer. Similarly, the emerging neurodiversity
movement is predicated on the right of those with autism spectrum disorders
and other neurological differences to speak for themselves, achieve
equality, and live meaningful and productive lives. 
REFERENCES
Fink, C. and Borchert, M. (2011) "Optic Nerve Hypoplasia and Autism: Common
Features of Spectrum Diseases." Journal of Blindness & Visual Impairment.
105 (6), 334-338.
Kranowitz, C.S. (2005) The Out-of-Sync Child. New York: TarcherPerigee.
Ryabets-Lienhard, A., Stewart, C., Borchert, M. et al. (2016) "The Optic
Nerve Hypoplasia Spectrum: Review of the Literature and Clinical
Guidelines." Advances in Pediatrics, 63, 127-146.


Richard Payne,  President
National Federation of the Blind of Ohio
937-396-5573 or 937/829/3368
Rchpay7 at gmail.com
The National Federation of the Blind knows that blindness is not the
characteristic that defines you or your future. Every day we raise the
expectations of blind people, because low expectations create obstacles
between blind people and our dreams. You can live the life you want;
blindness is not what holds you back


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