Article by peter zdepski

DECENTRALIZED

Peter Zdepski

“How bad will it be?”

“Well, you have a retina disorder, we know that, but we don’t know the severity, but you will probably lose a couple of lines on the eye chart.”

At this moment Jane Zdepski’s whole life collapsed in her mind.

She had been a successful engineer for the last eight years and was about give birth to her first child. She had gone her whole life not knowing she had this disorder and now, knowing she had it, she had to face the reality that it was going to alter her life completely.

Zdepski, a New Jersey native residing in Mountain View since 1995, is a proud mother of four children and lives with the disease known as Stargardt’s, a type of macular dystrophy. At the time of this doctor’s visit, however, there was no way for her to know what exactly she had. As she kept prodding the doctor for more information, his answers became increasingly vague (later, she found out he was shielding the truth from her due to her pregnancy). In fact, her vision would progressively deteriorate until her central vision was all but gone, and within the year alone, her vision would drop from around 20/50 to 20/100. Her vision is currently between 20/400 and 20/800.

Jane grew up in Colonia, New Jersey, about 20 miles south of New York City. She grew up being a quiet type, passionately 2

staying focused on sports and academics, excelling in both.

Due to this drive she was able to get into the College of Engineering at Rutgers University, where she got her Bachelor’s degree in Environmental Engineering. She got married soon after college and began to work as a civil engineer, a profession she would take very seriously and would be very successful in.

Everything was going well in her life, and just as she thought she was going to be a comfortable working mother. But then, as she started her family, she was hit with the news. At first she just sat there in the doctor’s office and thought that her life was going to change drastically, yet as she looks back on it she realizes she was mostly in shock about her future.

It wasn’t until her meeting with a second doctor until she would be told the extent of her disease. The specialist told her that she did indeed have Stargardt’s, and that it was a disease that caused her to have juvenile macular degeneration. In other words, the macula near her optic nerve was basically dying and would continue to die until her central vision was lost.

Many people live with Stargardt’s disease (fundus flavimaculatus), which is “one of the most frequent causes of macular degeneration in childhood and accounts for 7% of all retinal dystrophies” (Nature.com). At the time, however, not much was known about

she asked her doctor.

it because not much extensive research had been done yet. Due to this lack of knowledge the doctor also believed that Jane’s ability to see color would be greatly affected. Fortunately, her ability to see color is much better than many others with Stargardt’s; the only colors she can sometimes get mixed up are blue, green, and grey.

At the time of her diagnosis she was on maternity leave, and notified her work that she would not be able to come back. She had already realized her job was affected in the months prior, and she had repeated troubles while doing math, and had in fact even filed her taxes incorrectly the last three years in a row due to her vision. She still had some time before her vision would become its worse, as instead of affecting the victim since birth, Stargardt’s is a “progressive dysfunction due to macular degeneration” (Amir Rattner & Jeremy Nathans). She would eventually have her license revoked but could still drive seven years after her diagnosis due to using specially fitted telescopic glasses that enabled her to easily see traffic lights; consequently, she was restricted to day driving.

Another large part of her life that she could never truly get back after her vision loss was her love for sports. She would play tennis almost weekly, had played a sport every season during highschool, and played intramural sports like

volleyball constantly in college.

Due to her vision loss, after a while she was not able to see the fine movements of the ball during sports and eventually had to give them up.

Stargardt’s also brought with it some problems she did not expect to run into. The largest of these problems being that she could not notice facial expressions while in conversation, and could not make eye contact anymore which would make many people uncomfortable during conversation. While she was adjusting she was also

constantly knocking things over and at first would trip and fall badly at least two or three times a month. The anxieties of these new challenges also greatly intertwined with the anxieties of being a new mother. She was constantly afraid she would forget her first child Larisa’s face after her vision was lost and would carefully study it when she was a baby. This was an realistic concern, however; as with her younger children that she has had since she was blind, she cannot and has never seen their faces in full detail, although

she obviously has a good idea.

Due to her clumsiness from her vision as well she was in constant worry of dropping her babies whenever she held them, and actually got close once or twice.

Having diminished vision altered the way Jane had to tackle her daily life. She became much more familiar with her town due to her increased amount of walking. Her children became accustomed to walking and biking to and from school, and many times walking to the grocery store in order to get food, since 3

her husband Joel would not return from work until night. Even though she had her vision problems, she retained her ability to bike, which has helped her get around town, though currently she has given this up due to safety concerns. Her children also did fewer after school activities, and the ones they did were within walking distance from the house, like AYSO soccer at the nearby park.

One of the most important observations Jane has made about being disabled is how, in her words, “It simplifies your life”. The amount of choices you have in any given situation deplete, like where you are going to work or where you are going to live. You don’t have to be overwhelmed with choices, since you only need to focus on things that you know are well within their ability. These feelings are mutual between many people in the disabled community.

In Jane’s case, she saw the simplification of her life as a good thing, she decided her time would be better spent just raising her kids, instead of balancing a career in engineering along with it. She believes that because of her disability her kids also have more empathy than other children. With her extra time she has also been able to become involved in programs such as the PTA and AYSO soccer. Jane has chosen to do things within the community that are not intensely related with paperwork or screens. Her time has been put mostly into volunteer work for AYSO Soccer, where she has coached more than 20 teams of all ages and served as the regional commissioner from 2006-2007.

It always puts a smile on her face seeing the little kids playing soccer in the park because she knows she had a major part in making this region one of the premiere youth sports organizations in Northern California. Two of the things she specifically introduced to the program was added a spring select program, and starting the process that hands out scholarships to kids playing soccer in the program who need financial help, which increased the amount of kids that could play.

Jane’s outlook on the whole situation has changed greatly since the time of her diagnosis. She may have thought initially that the life she once knew was over, but she quickly found out that while she may have lost most of her vision, she hadn’t lost her desire to push on. “You can still have a life.” Her vision is better than she would have ever hoped in relation to what she had first been told. The Stargardt’s support group she used to be a part of used to even refer 4

her number to parents of children who were recently diagnosed. When she would tell them about her life, her accomplishments, and her family, the kids could find hope in the future knowing that others have succeeded in similar situations. It may be an inconvenience at time, but that’s really what it is as heart, an inconvenience. In the world of disabilities, she is grateful that the one that affects her does not impede her motor or brain function at all, and that she still has part of her sense of vision intact. Most people do not even know that Jane is legally blind unless she tells them or they observe her looking at her phone. She even can see the silver lining of her illness sometimes, like her ability to get better seating at her favorite concerts or cut in line at certain amusement parks. She also values the amount of friends she has that are willing to help her out and drive her places, and in certain ways her disability has allowed her to meet more people than if she had not had it. Reflecting back on it Jane can see that “It’s made me a lot more patient, your life starts being focused on when other people can help you out, not necessarily when you want to do things yourself.”

While of course she is not happy to have Stargardt’s, she cannot imagine a life without it. In fact, she has enjoyed how her life has panned out so far and is hopeful for the future, as technology to help people with vision impairments is steadily improving.

Such technologies and research have the potential to alter how people with Stargardt’s live their lives in the future.

Stargardt’s research has been becoming more and more helpful to patients over the years and new and hopeful treatments are being developed. One of the treatments which has the most potential is stem cell research. Recently, stem cells have been the focus of many scientific research since “embryonic stem cells have been proposed as a source of replacement cells in regenerative medicine” (The Lancet). Since Stargardt’s deteriorates your retina, doctors aim at using stem cells in order to repair the damage that has been done and restore the tissue. Through its implementation already it has produced varied results, with the most promising groups in the study having vision improvements of up to 20 points. Recently, however, a better alternative in many people’s eyes has emerged – different kinds of visual aids. Instead of vision improvements having to come from medi5

cal procedures, many companies’ software or devices can aid your ability to see or locate different objects. One of the most exciting pieces of this technology are the E Sight 3’s. The device works by allowing the user to “see full color video images clearly, with no lag time, and can zoom in. He or she can also capture photos and video with the device” (Jolly). People with Stargardt’s can benefit greatly from this device, as they can use zoom into anything they need to an appropriate amount where they it is within their peripheral vision. Technologies like this were not even imaginable when my mother was diagnosed, and right 6

now is an insanely hopeful time for anyone with sight-related disabilities.

The scariest thing about Stargardt’s is its ability to be present while the individual doesn’t even know they have a disease. Stargardt’s is a recessive trait, and that means that both my grandparents had it in their lineage, and that means there was a chance that Jane’s siblings could have it too. Jane has two siblings, Charles and Aime, and each child has a 25% chance of having it. When Charles learned about this, he decided to not even go to the eye doctor anymore in order to keep himself negligent to the

fact that he could have it, and he would rather live his live without such a burden. Aime was 28 at the time that Jane was diagnosed with Stargardt’s, and in school was studying to be a computeraided drafter. She wanted to know so she went to the eye doctors, but at that time there was no damage to her retina and due to a blood test for stargardt’s not being invented yet, she thought she did not have it. Unfortunately, symptoms started to arise when she was about 36, and was diagnosed with stargardt’s at 37. The only reason she even got checked was that she had told Jane that the night prior she had to actu-

ally get out of her car in order to read a street sign, since she could not when she was driving. This caused a sinking feeling in Jane’s stomach, she could relate to these vision problems all too well, and urged Aime to go get checked for Stargardt’s. At that time there was significant visible damage to her retina. She took the news very hard because she knew exactly what was going to happen to her after watching her older sister lose her eyesight. Usually the fear of the unknown can tend to be scarier, but knowing exactly how bad her vision would be caused her to have less optimism than Jane did when getting to terms with her diagnosis. The main cause her plight however was the fact that she had gone to school to work in such a visually demanding field. It’s one thing to be a blind musician like Ray Charles, but having devoted so much time into a workfield that you would have to leave anyways due to unfortunate circumstances was hard to grip with. She hid it from her employer for about six months that she was losing her eyesight since she still wanted to work in it, but it soon became apparent that she would have to leave this field. Due to her know earning a lower income, she had to give up on renting a house and instead move into a small one bedroom apartment. She had been a bartender before she went to school for computer aided drafting, and decided to return to that profession. She went back to work in the same banquet hall and kept her vision problems a complete secret from her employ-

ers, since each time she would set up the bar she would set it up in the same exact orientation so that she could still easily navigate it. With both Jane and Aime, one of the hardest parts of becoming disabled was the fact that it affects your opinions to make a living because you can’t physically do everything. Such things as read-

ing efficiently, drawing, and math were not completely inaccessible.

Currently Aime has found work through a program through the state of New Jersey that finds jobs for people with disabilities, and has been working at old navy for the last four years. She is able to use magnification devices in order to read things such as prices and barcodes. She can only work on the floor due to her inability to work the register due to her

eyesight, and is working hard to try to become a floor manager for the store in the future. She is confident in her future however, since she even says herself, “I can find anything in the store for a customer better than anyone that can see.”

With the help of aids seventeen hours a week through a state program, Aime now has more driving, shopping, cleaning and cooking help. She has able to hire some of her Old Navy coworkers for this task and it helps them as well since the pay is higher and hours are more flexible. She has been able to have more freedom than ever before as technology gets better and better, and her quality of life has improved over the years. Almost everything she needs is within walking distance and her one bedroom apartment is in a great location on the main street.

Stargardt’s may alter and change the lives drastically of those who have it, but it in no way means the end of a happy life. Jane and Anime are both living proof that people with this disease can continue to lead successful and meaningful lives. Moreover, unlike most disabilities, Stargardt’s may become more and more easy to live with, as we are on the brink of widespread use of technologies such as vision-enhancing goggles. Jane and Aime have both found happiness in their own rights and should be an inspiration to anyone who is going through a monumental change in their life.

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Works Cited Jolly, Jennifer. “High-Tech Glasses Are Helping Blind People See.” USA Today, Gannett Satellite Information Network, 15 Feb. 2017, www.usatoday. com/story/tech/columnist/2017/02/15/high-tech-glasses-helping-blindpeople-see/97960470/. Accessed 25 May 2017.

Schwartz, Steven D, et al. “Human Embryonic Stem Cell-Derived Retinal Pigment Epithelium in Patients with Age-Related Macular Degeneration and Stargardt’s Macular Dystrophy: Follow-up of Two Open-Label Phase 1/2 Studies.”

The Lancet, 15 Oct. 2014, www.thelancet.com/journals/lancet/article/PIIS01406736(14)61376-3/abstract?cc=y=. Accessed 25 May 2017.

Kaplan, Josseline, et al. “A Gene for Stargardt’s Disease (Fundus Flavimaculatus) Maps to the Short Arm of Chromosome 1.” Nature News, Nature Publishing Group, 1 Nov. 1993, www.nature.com/ng/journal/v5/n3/abs/ ng1193-308.html. Accessed 25 May 2017.

Rattner, Amir, and Jeremy Nathans. “Macular Degeneration: Recent Advances and Therapeutic Opportunities.” Nature News, Nature Publishing Group, 11 Oct. 2006, www.nature.com/nrn/journal/v7/n11/abs/ nrn2007.html. Accessed 25 May 2017.

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Peter Zdepski is a 16 year old student attending freestyle academy where he takes Digital Media, English, and Design. He has lived in California is whole life but his family is originally from New Jersey. His mother Jane has been legally blind since before he was born. He has lived adjusted to her illness her whole life and wants to show the disability from different perspective. His interest in the disability was sparked through observing his mother and due to the fact that he is a carrier of the Stargardt’s gene.

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