by John M. Williams
Lenyard, Conn. - Enthusiastic. Determined. Ambitious. Independent. Visionary. These words describe 10-year-old Sofia Priebe.
As with most children her age, Sofia is challenged academically, physically and socially. Academically, she is a top student. Physically, she can keep up with her peers on the playground, and socially she has friends inside and outside of school.
A fourth-grader at Ledyard Center School, Sofia is challenged daily by an obstacle that all but one of her friends confronts. With a visual acuity of 20/500, she is legally blind. When asked how long she's been blind, she replies, "I think my whole life, but we first knew it when I was three or four."
Sofia has Leber's congenital amaurosis, a degenerative condition that leads to complete blindness. After years of medical visits and research she was recently given a genetic diagnosis, identifying the gene causing her blindness. Sofia can see small things within three to six inches, and she can distinguish details, and sometimes colors. Although her color vision is deteriorating, contrast (not color) is increasingly important. Beyond that point, the best is she sees shapes and shadows. Sofia is extremely sensitive to light and will sometimes have her eyes almost closed. She usually wears sunglasses, even indoors. Shortly after she was diagnosed as being legally blind, she started learning braille. By 5, she was reading braille. She says she reads contracted braille and adds, "I learned un-contracted braille first, and now I know all of the contractions. I read and write in contracted braille now. I don't use un-contracted braille anymore unless I have to."
Braille is not a language. Rather, it is a code by which languages such as English or Spanish may be written and read. Any combination of one to six dots may be raised within each cell and the number, and position of the raised dots within a cell conveys to the reader the letter, word, number, or symbol the cell represents. There are 64 possible combinations of raised dots within a single cell. Due to the varying needs of braille readers, there are three different grades of braille. In braille grade 1, each possible arrangement of dots within a cell represents only one letter, number, punctuation sign, or special braille composition sign. It is a one-to-one conversion. Because of this grade's inability to shorten words, books and other documents produced in grade 1 braille are bulkier and larger than normally printed text. Grade 1 braille is typically used only by those who are new to learning braille, but as of the early 2000s a new movement was in place among elementary school teachers of braille to introduce children with sight difficulties to grade 2 braille immediately after teaching the basics of grade 1 braille.
Grade 3 braille is essentially a system of braille shorthand. Because it has not been standardized, it is not used in publications. Instead, it is typically used by individuals for their own personal convenience. It contains over 300 word contractions and makes great use of vowel omission. In addition, the amount of spacing between words and paragraphs is decreased in order to shorten the length of the final document. Sometimes it substitutes combinations of punctuation symbols for words.
Sofia uses a variety of assistive technology products at school and home for school work and entertainment. The products include JAWS, CCTV, magnifier, BrailleNote, and the iPad at school. JAWS is a screen reader developed for computer users whose vision loss prevents them from seeing screen content. It reads aloud what's on the PC screen. A BrailleNote is a computer made for people with visual impairments. It has a braille keyboard, speech synthesizer, and a refreshable braille display. Sofia also has access to the Internet so she can download books. She uses technology to take notes, for writing assignments in school and then to send the assignments wirelessly to an embosser to print in braille and send to the teacher's personal computer for printing and correction.
CCTV magnifiers provide low-vision aid for a full range of visual needs, specializing in assisting individuals with macular degeneration, glaucoma, cataracts, retinitis pigmentosa, diabetic retinopathy, and other eye conditions that cause low vision. The benefits of a CCTV magnifier are many, as the versatility provided allows for independence through visual magnification and technology. Depending on the video magnifier, duties such as reading the mail, books, writing, enjoying a crossword puzzle, connectivity to a computer and much more can be accomplished. Sofia uses the CCTV for reading, assembling puzzles and playing with Legos.
The iPad's magnification feature helps Sofia see the board. "I use all of my technology. I use the iPad so I can see the board in school," she said. She can synch the iPad's content to the Promethean board or Smart Board. Smart Boards meld high-tech functionality and tradition by acting as a computer monitor and a chalkboard at the same time. Smart Boards instructors can show videos, write equations and check homework all on the same board in the classroom.
A Promethean board is an interactive learning whiteboard that connects to a computer. This technology allows Sofia to have hands-on practice and personal involvement with learning. "I usually use the CCTV to read or see the white board, which we don't use as much," says Sofia. She mainly uses the BrailleNote to write documents or stories for the Writers Club, or something for school. She also uses it for fun. "I can read and play games with it," she adds. She also uses BrailleNote to download books from Bookshare. Her life's goal is to be a writer. Sometimes she writes her stories in journals because, she says, "I have a lot of stories to write."
Sofia loves audiobooks. She has listened to all of the Harry Potter books. They are her favorite books. She receives orientation and mobility (O&M) training and has for years. She believes that O&M builds her self-confidence and increases her independence. She often walks the neighborhood by herself. To visit friends, she crosses the street by herself. When she is older, she wants to travel sometimes with others and maybe by herself. She is planning a solo trip to visit her aunts in a few months.
There are a variety of skills and techniques that facilitate safe travel. An O&M instructor teaches the student to focus on and accurately interpret sensory information available in the environment. Orientation is the ability to know where one is, where one wants to go and how to get there. To do this, a person learns to create and maintain a "mental map" that changes as he or she moves through space, using landmarks and environmental clues to supplement whatever vision the person has. Depending on the student's goals, teaching can include skills in traveling through complex urban environments and the use of public transportation. Priebe will receive O&M training as long as she needs it. Connecticut's Bureau of Education and Services for the Blind (BESB) pays for the service.
Sofia is precocious. She is determined to be a writer. She is not sure what college she will attend. She knows she has years ahead of her before she decides. She has a second and third condition for the college she picks. Proud of her achievements in O&M, she says, "I want it to be easy to get around so I don't have to drive. … I want to go to a college that teaches you about Greek mythology and stuff like that."
Sofia is well adjusted. While she wishes she was not blind, she knows she can't change her situation. She has a strong family. Her parents, Chuck and Laura, and her brothers, Luca (youngest) and Dante (oldest) love her and she loves them. She loves dancing and swimming, and during the summer, she spends a week at camp with other blind children. She has made friends at camp. She sees them at other activities and workshops for the blind organize by BESB. Recently she met some of her blind friends at the Northeast Regional Braille Challenge at the Carroll Center for the Blind, Newton, Mass.
Sofia doesn't set herself apart because of her vision or dwell on it too much at this point in her life either. She's a typical 10-year-old girl. While many things are harder, and there are a lot of things she does differently from her friends, she has a positive attitude about life. Laura says, "She's extremely driven and competitive, not to mention fiercely independent. Her independence has served her well so far; I have no doubt it will always serve her well. When she loses her vision or we bump up against things she can't do, we try to be creative about and just deal."
This isn't to say things are always rosy. Her parents say it's hard to watch when she's playing with friends and, as kids do, they'll run off playing. There's Sofia spinning in place or talking to air because she didn't see her friends walk off, let alone which direction. She also stumbles and falls and has run into more than one tree, door, wall, person, off steps, you name it. Her parents try to make sure she uses her cane, but things still happen. "I'm always worried about safety issues, friendships, school services, etc. But at the end of the day, I worry about exactly these same things for her brothers, and they are sighted," says Chuck. "With sight, they've managed to break more bones, secure more bruises and get more stitches." (The chipped tooth count is currently tied, 2-2.) If there is one thing that Laura does differently for Sofia because of her vision, perhaps it's that she is very conscious of teaching her independence and self-advocacy. "If it weren't for her visual impairment I can't say I would think about this much, especially from such a young age," says Laura.
Like every mother, Laura worries a lot. However, Sofia doesn't sit around worrying or feeling sorry for herself. So Laura takes a deep breath, steps back from the situation, and as objectively as possible tries to assess if it's one of those times she needs to intervene, or just look away and let Sofia figure things out. "Hovering is not exactly teaching independence," Laura says. "It's not easy to hold back, but when she needs me, she'll ask and I'll be there. She's a pretty cool kid. I'm very proud of her."
For Sofia, ability counts, and she has lots of it.