boardwalk-edited

WHERE THE BOARDWALK ENDS
A Journey Through My Eyes
by
Matthew Lipsey

With notes by
Mindy Lipsey

Matthew Lipsey, the oldest son of Mindy Lipsey, is eleven years old and attends middle school in Baltimore, Maryland. He is enrolled in the gifted and talented program and wants to be a nuclear engineer when he grows up.

Mindy has two other children: Josh, age 8, and Emily, age 5. She has a Bachelor of Science degree from Towson University and an M.B.A. from Loyola College. Mindy and her three children reside in Owings Mills, Maryland, where she is employed in the advertising field.

Introduction

Growing up with low vision isn’t easy. People call me “special” or “different,” but I’m no more special or different than any other eleven year old boy. I just can’t see in the middle of my vision. I have a scotoma, or blind spot, taking up 30 degrees of a 160 degree visual field, so I go through life missing details.

Older people with low vision say I’m lucky I got Stargardt disease so young. They think it’s easier to adapt to low vision when you’re young. I was actually born with it, but my vision began to fail when I was six. I don’t remember what normal vision is, so maybe they’re right. I really don’t know what I’m missing.

Now, at age 11, I’m pretty good at reading Braille, and I can use low vision aids better than most old folks. And I’ll never have my license taken away, because I’ll never drive in the first place. So it’s going to be okay for me, but I think it’s hard on Mom.

I rate Stargardt disease sixth on a scale of one to ten (ten being really bad). Mom always uses that scale when I’m sick or hurt, so she can try to judge my level of pain. There are so many worse things out there: fatal diseases, losing a limb, brain damage, living in a wheelchair. So I try to play the cards I was dealt.

I have an “orphan disease,” which means very few kids have it. Mom looked online when I was diagnosed and found lots of support for herself, but she had trouble finding anything for me. That’s when we decided we should write my story, so other kids won’t feel so alone.

Diagnosis

I was diagnosed on June 25, 2003. Mom was eight months pregnant with my little sister, Emily, and she says that’s a day she’ll never forget. She took me in for an examination, because, from the way I held my books touching my nose, she thought I needed glasses. The pediatric ophthalmologist had me read the eye chart. I read the first line: “E.” He said, “Read the next line,” and I said I couldn’t see anything else. He told Mom, “There’s something else going on here.”

The nurse gave me some eyedrops that opened my pupils all the way. Then the doctor looked inside my eyes with a lighted magnifier. After a little while, he asked me to leave the room. I stood outside with my ear pressed against the door, trying to hear what they were saying. Mom calls me “Big Ears”, but not because I have big ears. They’re actually kind of small, like the rest of me. But I can always hear everything she says, even when she’s whispering.

The doctor told Mom a bunch of things she couldn’t understand. She started to cry. She asked the doctor to write down what he thought was wrong with me so she could look it up online when she got home. Then she took my hand, and we left. I really don’t remember much about the rest of the day, except “Mommom” Rita and “Pop” David coming over. I’m sure I thought my grandparents were just visiting, like they did at least once a week.

The next few days, Mom spent a lot of time on her computer. She learned that Stargardt disease is a juvenile form of macular degeneration that affects central vision in people under age twenty. What the doctor saw in my eyes were yellowish-whitish spots in all kinds of shapes. He knew those spots were going to eventually take away my central vision, and I wouldn’t be able to see details and maybe not colors, either. Reading and recognizing faces would become very hard or impossible for me.

Some people with Stargardt disease lose their central vision faster than others, with about half of us becoming “legally blind” by the time we’re fifty. That means things we can barely see at twenty feet, people with normal vision can see from two hundred. Eventually, almost all of us will become legally blind. And glasses won’t help.

Scientists know that Stargardt disease is caused by a mutant gene called ABCR-4.¹ They’re working on treatments now, but so far, there is no cure. Until there is, we need to learn how to use low vision devices, and we have to learn how to use other senses in place of our eyesight.

Coping

Mom believes helping a son or daughter live with an eye disease is like dealing with death. Before she could accept my condition, she had to go through things like denial, guilt, fear and sadness, just like part of me was dying. Mom had to watch me lose a little vision every day, and she couldn’t do anything to stop it.

Growing up and going to school is tough enough for any kid, but it’s a lot harder for those of us with low vision. We have to face things like getting through crowded hallways, not being able to get a driver’s license, and not being able to see normal print, faces or Friday night football games. But all these things are easier to take when our families and friends are there for us.

Here are some things my family does that helps me:

1. They admit that I’m visually impaired.
2. They’re honest with me.
3. They talk to me on my level.
4. They teach me what I need to know when I need to know it.
5. They give me confidence enough to stand up for myself.
6. They don’t feel sorry for me.
7. They concentrate on what I can do, not what I can’t.
8. They remind me I’m more than my eyes.

photo of Matthew's glasses

What Do I See?

After I was diagnosed, Mom asked me all the time, “What do you see?” It’s kind of hard to explain. People with Stargardt disease see at different levels. And those visual acuity numbers from the eye chart are just numbers. They don’t really describe how well we can get around or do things. People with normal vision have a hard time understanding what we see.

Actually, it’s more about what we don’t see than what we do see. I tell my friends to try wearing glasses with a finger-tip size spot of Vaseline in the middle of each lens. I tell them to walk around and try to read without looking away from the spot, and that will give them a pretty good idea. ²

photo of Matthew

School

When I was in first grade, Mom asked for a meeting at my school. I was an early reader, so I was already reading above grade level. I didn’t need too much in terms of low vision devices at the time, but Mom wanted the school to be aware of my disease. She wanted them to understand how it would affect me as I got older. They started working with me early, and that helped a lot.

All school districts are supposed to have teachers trained to work with kids like me. It’s the law. When Mom asked for the meeting, they had thirty days to get back to her about it. Once they decided I was eligible for low vision services, they set up an individualized education plan (IEP) for me. This was put together by a whole team of people: my parents, teachers, a teacher trained to work with visually impaired kids, my school counselor, the assistant principal, and the school nurse. Anyone else could have attended, too, as long as Mom let the school office know.³

Mom distributes information to my teachers to help them learn how Stargardt disease affects me at school. It’s called “Helping Teachers Understand Students With Stargardt Disease,” and there’s a copy of it at the end of my story.

Questions

Stargardt disease is progressive, so Mom takes me to the retinal specialist every six months. I don’t look forward to all the tests, but they really aren’t so bad. Since we can’t think of everything we want to know when we’re at the clinic, Mom and I write down questions ahead of time. This is a sample of some we have asked:

Are you sure this diagnosis is absolutely correct?
Should I have a blood test to identify the gene and confirm the disease?
Should my siblings and parents have their blood tested?
How often should I be examined?
When will my next appointment be, and with whom?
Will vitamin supplements help?
Do you know of any promising treatment studies?
Where can I get sunglasses that will protect my eyes?
Where can I get help understanding what is happening to my eyesight?
Where can I get help with daily living?
Where can I get educational assistance?
Where are support groups specifically for parents or children dealing with Stargardt disease?
Would you be willing to give my phone number to other patients’ families?
Can you recommend a foundation my family can donate to that will use our money specifically for fighting juvenile macular degeneration?
When should I see a rehabilitation specialist?
When should I start using low vision devices?
When should my school be told to begin the IEP process?
Will you provide a copy of your evaluation and diagnosis to my school?

These are all fair questions the doctor won’t mind answering. Unfair questions are those that expect him to tell the future. No one can tell me how slow or fast my condition will progress, or how much I’ll be able to see ten years from now.

It’s also unfair to expect the doctor to teach me all there is to know, but he should be able to tell me where I can learn. I can find out an awful lot from trusted websites, books, and other people who are going through this. Mom has found some great organizations and support groups that have really helped us. Here are our favorites:

Foundation Fighting Blindness
www.blindness.org

Macular Degeneration Support
www.mdsupport.org

National Federation of the Blind
www.nfb.org

There is also an Internet site where families like mine can create websites for sharing with others. It’s called CaringBridge, and our site is at www.caringbridge.org/md/matthewandjosh.

Low Vision Devices

Since there isn’t a treatment or cure for Stargardt disease, low vision assistive devices are the best tools I have.The first things we got were a large wall clock, a talking clock, a talking watch, and a phone with large numbers. I also have a scanner/reader so I can listen to books, a dome magnifier, an electronic magnifier, a monocular, special television glasses, and magnification software for my computer.⁴

I got a lot of these things through our state’s rehabilitation service and my school. I was glad they let me try them out first to decide which ones were right for me. People who write to Internet message boards and email discussion groups have also helped us decide which devices to buy.⁵
These devices are really helpful, and even fun to use. I can’t wait to see what they’re going to come up with next!

Fighting Back

Fundraising is a great way to fight back against this disease, and it’s something our family has fun doing together. Over the past few years, we have raised over $50,000 for non-profit organizations like the Foundation Fighting Blindness, Wilmer Eye Institute, U.S. Space and Rocket Center, and Hoover Low Vision Rehabilitation Center. We always make sure our money goes right where we want it to. Most charities will let you do that.

Volunteering for clinical trials⁶ is another way to fight back. Right now, no trials are being done for Stargardt disease, but the Foundation Fighting Blindness has me on a waiting list for when the time comes. My cure and treatment could come from gene research, stem cell research, retinal implantation, a new drug or supplement, or maybe all of these things. I’m ready to help whenever they need me.

In This Together

I don’t like to end this part of my story with bad news, but in September 2004, my younger brother, Josh, was diagnosed with Stargardt disease. Now he’s showing signs of vision loss like I did at his age. Our little sister, Emily didn’t get those genes, and that makes us very happy.

photo of Emily

I’m happy and sad at the same time about Josh. I’m glad I have someone to share this with now, but I’m sad it had to be him. Still, we have a lot going for us. Scientists are working hard on cures for eye diseases, lots of devices and computer programs are out there for us, rehabilitation therapists are always ready to help, and, of course, we have our family, friends and teachers all doing their best to help us grow up strong and confident.

Josh and I are still kids, so this story isn’t over. We’ve got a long way to go, and we know our next chapter is only going to be as good as we decide to make it. With everyone’s help, I know we’ll be just fine.

photo of Matthew and Josh

Epilogue: The End of the Boardwalk

When I was eight, my family vacationed at the beach in Ocean City, Maryland. One day, we walked along the boardwalk to 27th street. I couldn’t see it very well, but Mom said that was where the boardwalk ended. She told me it was a beautiful sight where the dunes began and the beach widened to the ocean.

I asked her if we could keep going, and she started to say something about how much easier it would be to stay where the walking was easier. But then she took my hand, like she did that day in the doctor’s office, and we ran off into the sand together.

“Blind is an adjective to describe yourself.
Be careful not to let it become the noun.”
--Matthew Lipsey

Letters From Mom

2004

Dear Matthew,

Last night you asked me if I was upset when I learned that you had Stargardt disease. When I told you “yes”, you reminded me that “it’s no big deal to have it,” and that “you were just born with it.” You also asked me if your eyes will get better on their own and I told you “no.”

I promised that I would always tell you the truth when you asked me questions about your eyes. I shared with you that your help would come in terms of low vision aids like magnifiers and monoculars, and that we would continue to raise money to give to the doctors to help them come up with a cure.

Your sense of humor, positive attitude, bright aptitude and loving heart will get you through this. I wish I could give you great words of wisdom and advice, but I’m going to have to let you handle some of this on your own as you grow older, wiser and more mature.

As a child growing up with Stargardt disease, you have already begun to realize your limitations. You may one day think of yourself as being a little different in a way you did not choose, and that can be tough. But with any limitation, you either overcome it, or it overcomes you.

Others have told me without hesitation that being the mother of a child who has vision problems is much harder than being the child. I know you may find that difficult to understand until you are a parent yourself one day. As your mom, it was only when I was willing to let go of my grief that I was able to help you. It's ok to grieve, but sooner or later we all have to move past it.

No matter how old you are, you will always be my baby. And I’ve told you many times that it’s my job as a mom to always worry about you. Another explanation for why I am sometimes sad is because I don’t have the power to make your eyes better, and I cannot always be there to protect you. You will find your own way, and I will have to sit by and watch.

Most importantly, in the end you will have your achievements and your character to show for the challenges you will endure. My love for you may sometimes appear in different ways. Just remember that I will always love you, and know in the end that some things I do are designed to make you a stronger person.

I Love You,
Mom
__________________________

2007

Dear Friends, Family, Neighbors and Co-workers,

My name is Mindy Lipsey and I’m the mother of three children – Matthew, age 9, Josh-age 6, and Emily-age 3. Like all parents, we breathe a sigh of relief when our children are born healthy. As they grow and thrive, we begin to take their health for granted. We revel in their developing talents and dream about their futures.

But for some of us, the joy of parenting is interrupted by unimaginable tragedy. In our case, it started three years ago with a visit to the ophthalmologist, because Matthew was sitting very close to the television, and we thought he needed glasses. The doctor said he couldn’t correct Matthew’s sight and that our son needed to see a retinal specialist. The specialist told us that Matthew had a genetic, hereditary eye disease called Stargardt disease, and that our other children would need to be examined.

I’ll never forget the day the doctor confirmed that our middle child, Josh, who was 3 at the time, also had Stargardt disease. The sadness and dread is one that I cannot easily describe in words. Never in our darkest fears would we have thought that blindness would be an issue with our boys. I feel so helpless, knowing that each year things get a bit worse.

There is no cure for this gradual vision degeneration and no treatment available to slow the progression. The effect of Stargardt disease is a continuous loss of the central vision that is responsible for visual acuity and color perception. Matthew is currently legally blind, and Josh has recently begun to show signs of vision loss, as well.

It breaks my heart when the boys talk about driving a car, or that Matthew wants to be a surgeon and that one day Josh will have to stop playing baseball. The doctors feel that Josh’s vision will deteriorate as quickly as Matthew’s did. We want to prolong his childhood and avoid confronting him with a life altering experience, and so we have not yet told Josh of his diagnosis. But soon we’ll have to, and that’s a day we dread.

Every night, we pray that God will answer those who are looking for a miracle. Miracles can occur in so many ways, and more and more, I have come to understand that research will make miracles happen. And so, over the past three years, Alan and I started to raise money to find cures for retinal degenerative diseases. To date, we’ve raised over $30,000 through various fundraisers. That may not sound like much, but there are many more families like ours who are also working with various foundations to make miracles happen.

The research needed to cure this devastating disease is extremely expensive, but with your help, a cure is in sight. We are grateful for your continued charitable support.

Warmest Regards,
Mindy Lipsey

Helping Teachers Understand Students
With Stargardt Disease

Eccentric Viewing

Stargardt disease creates central blind spots that increase in size as time progresses. Children with the disease learn to turn their eyes in a specific direction to see around the blind spots on their damaged macula. It may appear as if they are not making eye contact, even though they are. As a result, they do not see objects that appear in their central vision even though the far peripheral vision remains intact.

Photostress and Dark Adaptation

Children with Stargardt disease have difficulty adapting to the dark after sunlight exposure. When a child with low vision is exposed to bright sunlight, the sharpness of vision decreases, and blind spots become denser. Continual damage to the retina also leads to an increase in light sensitivity.

Depth Perception

A child, like anyone else, needs two healthy eyes to have good depth perception. Anything that decreases vision in one or both eyes will cause an immediate drop in depth perception.

School Age Issues

In children with Stargardt disease, it is imperative to have low vision rehabilitation. For example, large print materials are needed, including textbooks, worksheets, and standardized exams. Children must sit up front and close to the blackboard. Due to their vision loss and difficulty reading, some students require extended time on tests in resource rooms.

Safety issues usually include eyewear with polycarbonate lenses for protection of the eyes in physical education. Sports with fast moving projectiles put young Stargardt patients at risk.

Individualized Education Plan

Below is a sample outline of ideas a parent can take to an Individualized Education Plan (IEP) meeting with their school administration. Don’t expect the school officials to tell you the best ideas to implement for your child. As a parent, no one knows your child better than you. You are their best advocate. This outline is not an IEP, rather it just provides ideas.

Implementation of the “Buddy System”

Preferential seating

Optimum seating to view teacher and chalk board / front row seating

Seating in cafeteria with friends

Gym class adaptations

Close monitoring involving catching balls

Depth perception

Awareness of limited distant viewing

Computer room adjustments

Monitor placed close to edge of table

Large print keyboard stickers

Larger monitor for improved viewing

Enlarged font and software – Zoom Text

Learning needed for alternative communication skills per expanded core curriculum

Braille instruction

Keyboard instruction

Handwriting Without Tears program

Cursive handwriting assistance if needed

Do not require cursive handwriting

Raised lined paper or contrast paper

Feedback regarding reading speed

Access to assistive technology devices

Monocular

Optical magnification

Flipper by Enhanced Vision

Victor Stream Reader, Bookshare.org

Lighting considerations

Avoid bright lights and glare from window

Wear sunglasses during outside activities

Classroom

Use slant board to avoid awkward posture by bending over to see papers
Contrast – dark black ink on white paper, white chalk on blackboard
Enlarged papers needed if small font size
Provide all standardized tests and classroom exams in large print
Allow extra time if needed for all tests
Allow scribe for exams if needed
If using overheads, provide desktop copy
Desktop copies of board work as needed
Roaming privileges – Ability to walk toward the chalk board or other training aids without asking
Teachers to make parents aware if child falls behind
Provide dictionary, resource books, textbooks in large print
Opportunity for breaks for eye fatigue
Sensitivity of teachers
Monitor child and disclose any perceived changes in visual performance
Miscellaneous
Parents to attend field trips
Orientation and Mobility Assessments
Low Vision Assessments
Functional Vision Assessments

Mom’s Footnotes
(Use back button to return to text)

1. Stargardt disease is usually autosomal recessive. Unaffected parents who are carriers have one abnormal gene paired with one normal gene. Each of their children then has a 25% chance (one chance in four) of inheriting the two diseased genes (one from each parent) needed to cause the disorder. Carriers are unaffected because they have only one copy of the gene.

For people with retinal degenerative diseases and their family members, genetic testing can reveal valuable information about the disease, including: the likelihood of passing it on to children, the potential severity of long-term vision loss, eligibility for forthcoming clinical trials, and the benefits of emerging treatments. Genetic testing can help people make informed decisions about their future, especially in the areas of family and career planning.

When handled with appropriate counseling and education, the identification of disease-causing genes can empower patients to deal more effectively with often-formidable retinal conditions.

Though genetic testing is becoming more widely available, it is still a process that requires a fair amount of education and effort for both the patient and physician. The Foundation Fighting Blindness offers a genetic testing information packet to help patients and physicians better understand and navigate the process.

2. Lighthouse International, based in New York, 212-821-9200, sells simulated vision glasses with a blurry spot in the central vision.

3. To learn more about the IEP process, a wonderful website may be found at www.wrightslaw.com. I highly recommend that parents learn what their child’s rights are, and what assistance they are eligible to receive. If the school system doesn’t respond, parents have to advocate for their children at a local level. The National Association of Parents with Visually Impaired Children (www.spedex.com/napvi) is an excellent advocacy resource.
Prior to the beginning of the new school year, it is best to educate your child’s teachers regarding their vision disability and what adaptations will need to be made. It is not enough to assume your child’s teacher will review their IEP in detail. Request that your child’s teacher of the visually impaired do a presentation for the teachers as a whole, so they better understand how your son or daughter’s vision loss impacts their learning.

4. Links to nearly all low vision assistive devices on the market can be found on the MD Support site at www.mdsupport.org/resources.html.

5. Very active message boards and email discussion groups may be found at www.mdsupport.org and www.blindness.org.

6. Clinical trials are the final clinical research phase necessary for the Food and Drug Administration (FDA) to approve a treatment for use in humans. The clinical testing of experimental drugs is normally done in three phases, each successive phase involving a larger number of people.