Stargardt's Disease

Story available at www.billingsgazette.net/articles/2007/01/17/sports/local/25-bowler.txt

Published on Wednesday, January 17, 2007.
Last modified on 1/17/2007 at 12:09 am

Legally blind bowler achieves 300 game
By BILL BIGHAUS
Of The Gazette Staff

Jerry Black can't drive because he's legally blind, so to get to his bowling league one Thursday night in late October he did what he usually does: Hop a ride on a couple of city buses and walk the remaining four blocks to Sunset Bowl.

Shortly after arriving, though, the 49-year-old Billings man did something truly extraordinary - he rolled a perfect 300 game.

"I didn't really think it would ever happen," said Black, whose 300 was 121 pins over his average. "It's like something you don't really expect."

Especially when you consider that Black's vision is impaired to the point he can't see anything when he looks straight ahead.

He was diagnosed with Stargardt's disease, a degenerative eye condition, in his early 20s. That extremely rare disorder caused the rapid loss of the central vision in both of his eyes.

"It's just like a giant blind spot," he said.

He has overcome his disability so well, though, it's hard to tell by watching him bowl that the pins are just a white blur in the distance to him.

And those pins just keep crashing despite the fact Black's partial blindness prevents him from reading his own score on the overhead scoreboard. He also can't see the faces of his teammates from more than three feet away in the dimly-lit bowling center, but he can tell them apart by their build and the way they walk and talk.

"I've got decent peripheral vision but I've got no center vision," he said. "So everything I do is with my peripheral vision."

Black, a right-hander and captain of The Pretenders team, certainly made excellent use of the vision he does have that Thursday night at Sunset while bowling on lanes 11 and 12 in the Federal league.

"I can't really see my spots up there (on the lane), but I know the lane is only so wide," he said. "I can see the gutters, of course. It's kind of like I'm throwing toward a certain area. You just know that a certain area is there for a certain pin, so that's kind of what I throw at."

After opening with a 165 game, the 6-foot-1, 205-pound Black, armed with his 15-pound Brunswick Zone ball, strung 12 strikes together to really distinguish himself in his second game.

"I started feeling good and just found my shot," he recalled. "I relaxed and (the pins) started falling. I didn't get really nervous until probably the eighth frame."

Black, who was taught to bowl by his mother Bille and has been enjoying the sport for 44 years, said he found a reliable mark on the lanes - between the fifth and 10th board - and just consistently hit it. He has a smooth approach, with moderate hook on his ball.

He said he can't see clearly more than 10 feet down the lanes. Sometimes he'll have trouble distinguishing what pins are still standing on his second ball, so he'll ask his teammates for assistance so he can adjust his aim. They'll also tell him if one pin is lined up directly behind another.

But Black, whose previous high game was a 279, didn't need any help en route to his 300.

"My shot was there that night, at least for that period of time," he said. "It's not that often I can throw that many balls in the pocket. They were all in the pocket until the last one. I went Brooklyn (and hit high in the pocket)."

As the strikes mounted during his remarkable game, so did the attention and Black's level of nervousness.

"My hands were sweating so bad I couldn't dry my hand off. I was just praying the ball wouldn't go in the gutter," he said. "My friends quit talking to me after about the seventh frame. It was all quiet down on that end of the lanes. I guess I didn't really notice that everybody was watching."

When all of the pins toppled for the 12th time "all of my friends were up at the approach jumping up and down," said Black. "People were yelling stuff around me."

As for his own reaction: "It was a huge relief," he said. "My hands were shaking."

His longtime teammate, Ping Stiffarm, was out of town that week and missed Black's monumental 300.

"I didn't see it. He didn't either," said Stiffarm, while poking a little fun at his teammate. "I was really surprised he didn't call me. That's kind of the way he is."

Stargardt's disease, which is usually inherited, is a type of macular degeneration.

"It hits when you're younger and it runs its course fairly quickly," said Black, who wears glasses to enhance his peripheral view. "I went from 20-20 corrected to 20-200 in a matter of about six months."

His vision is now 20-300, and the retina damage he has suffered is irreversible.

"There's only like 25,000 cases in the United States," he said of Stargardt's. "Basically it's not cost-effective for them to work on a cure because there are not enough cases."

It's the competition that keeps Black coming to the bowling center.

"There are not many other things you can do when you've got bad eyes," he said. "I bowl and I golf."

Black, whose bowling average has climbed to 185 since the 300 game and also plays to an 18 handicap in golf, works as a contracting officer for Indian Health Service in Billings. The South Dakota native, who was born in Pine Ridge and is a member of the Oglala Sioux tribe, has lived and bowled here since 1996.

"I don't consider it a real handicap anymore," he said of dealing with his blindness on the lanes. "It's more of an inconvenience than anything else."

At work, his computer has magnifying software to enlarge the words on his screen. His office also has a closed-circuit TV system with a magnifying camera to help him read printed material.

Away from the office and bowling center, Black uses binoculars to watch his 16-year-old daughter Alex, a sophomore, play varsity basketball for Billings West.

Ryan Macdonald, 26, works and bowls with Black - and was there jumping, yelling and celebrating the night of the 300.

"When you see Jerry bowl and golf, you forget that he's (nearly) blind," said Macdonald. "I'm reminded every day at work because he has a lot of equipment that helps him read a document. It hits home that what he did (in rolling a 300) is really incredible."

Black received $300 from Sunset Bowl for his perfect game and a special ring from the United States Bowling Congress. There are over 3,000 league bowlers in Billings this season - and just six have 300s.

"It's just like a dream," Black said of his. "If you bowl or do any sport you always want to have perfection for even one time. Basically what happened was for one night and one game I was perfect.

"I was kind of scared to go to sleep that night because I felt like I was going to wake up and it was all going to be a dream and it didn't really happen."


Copyright © The Billings Gazette, a division of Lee Enterprises.

The Herald - Everett, Wash. - www.HeraldNet.com
Published: Thursday, March 1, 2007

To Snohomish girls' eyes, it's a miracle


By Melissa Slager
Herald Writer



SNOHOMISH - Three girls with rare vision disorders are seeing their classrooms like never before.

They're able to read more than one word at a time in textbooks.

They can see their teachers' faces.

The best part?

"The freedom to do what I want without having to worry about it," said Ali Steenis, 12.

A school principal, a father and three Lions Club organizations teamed to help Ali and two other Snohomish School District students: sisters Brianna Leavens, 15, and Mackenzie Leavens, 12.

Each girl has severe limitations on her sight. Ali's eyes cannot handle bright light, while the Leavens girls have only peripheral vision.

Thanks to $16,000 in donations, each girl now has a set of cameras and laptops that lets them zoom in on features they otherwise would see as little more than smudges.

Paul Leavens, the sisters' father, said it started with dissatisfaction over what currently exists to help students with low vision.

Until now, the girls have relied on magnifying glasses, good listening skills and notes taken on carbon paper by helpful classmates.

"There seemed to be a big gap between what was possible and what was being used," said Leavens, a Microsoft engineer. "That's what set us on using technology to close that gap."

Leavens came up with the methods.

Then Ginny Schilaty, principal of Machias Elementary School, where the Leavens girls used to attend, pursued the funding.

They found help with the Lions Club.

The Snohomish Lions Club donated $500, the Snohomish County Sight and Hearing Foundation chipped in $5,500, and the Northwest Lions Foundation for Sight and Hearing in Seattle topped it off with $10,000.

Outgrowth of mission

Lions Clubs are perhaps best known for recycling used eyeglasses for those in need.

"This project takes that a little farther," said Morrie Sachsenmaier, a member of the Snohomish and county groups.

Each of the girls has a special camera that hooks up to a laptop, displaying live images on a 17-inch screen.

The set-up allows the girls to turn the camera and zoom in on their teachers, whiteboards or anything else they need to see.

"It was weird to see the board for the first time in a few years. It was pretty cool," said Mackenzie, a Centennial Middle School seventh-grader who started losing her sight when she was in about third grade.

As the girls get more savvy, they can hit the "pause" button and take things a bit further with the equipment using special software.

Brianna, a Snohomish High School sophomore, is already doing so.

In chemistry class, for example, she will train her camera on the hydrogen compound notations her teacher writes on the board, hit "pause," and then copy and paste the image into her notes file instead of typing it all out by hand.

At home, the girls have a special docking station that elevates the camera and lets them slide a textbook underneath, projecting the page onto an even larger monitor.

Before, each of the girls had to hold books within an inch or two of their noses to see words - even if it was in large type - or use magnifying glasses.

So far, Mackenzie said she reads at about the same pace. "But my neck doesn't hurt."

Adapting to new tools

Although the limits on their vision are similar, the Leavens sisters have a different vision disorder than Ali.

Brianna and Mackenzie both have Stargardt's disease, which means they retain their peripheral vision but have very little central vision.

The girls can't make out faces, for example. They primarily know people by such features as hair color, body shape and voice.

"I can see there's two blobs, but I can't see what color your eyes are or where you're looking," Brianna said.

Ken and Cyndie Steenis say doctors have no specific diagnosis for their daughter Ali's vision disorder.

Something is wrong with the rods and cones in the retinas of her eyes. The photoreceptors help a person see in the dark and perceive color.

Ali's eyes are highly sensitive to light. She sees better in the dark, so she wears sunglasses to Totem Falls Elementary School, where she is in sixth grade.

Ali is getting used to the equipment. But her parents say they already see a difference.

"Her self-esteem has gone through the roof," Ken Steenis said.

Paul and Amy Leavens say their daughters adapted early on to their vision loss - getting used to seeing is the harder lesson now.

The new equipment likely will be of greatest benefit when the girls go to college, where the pace of learning accelerates and more independence is needed, Paul Leavens said.

The equipment belongs to the school district, although it's possible it could follow each of the girls beyond high school if there are no other students with similar needs behind them, Schilaty said.

Schilaty and Leavens said their primary goal is to get the word out to other schools about what's possible for helping students with low vision.

"Technology is a good equalizer," Leavens said. "I look at this as an incubator for proving it works."

Reporter Melissa Slager: 425-339-3465 or mslager@heraldnet.com.




Source Link: The Daily Herald Co. www.heraldnet.com/stories/07/03/01/100loc_a1vision001.cfm

Video: poconorecord.com/storemultimedia/Video/marathon/marathon.wmv

Blind runner qualifies for Olympic Trials at the Run for the Red
Beth Brelje
Pocono Record Writer
May 07, 2007
Source Link: Pocono Record www.poconorecord.com/apps/pbcs.dll/article?AID=/20070507/NEWS/705070320/-1/News

"I think I'm dreaming." Wrapped in a foil blanket Susan Graham-Gray's eyes brimmed with tears of accomplishment. The top women's finisher in the Run for the Red Marathon was surrounded by microphones and photographers.

She got laughs when asked about toughest part of the race, answering "Anything after 17 miles." That is about the time when the run gets mental and your legs get tired, she said.

She made mention of having "vision problems," but downplayed it and focused on having achieved her life-long dream of qualifying for the Olympic trials. To qualify, runners must finish in less than 2 hours 47 minutes on a certified course.

Graham-Gray of Greencastle, finished in 2 hours and 45 minutes.

Having missed the Olympics in 2004, Graham-Gray called this her last chance.

Graham-Gray, 38, is blind. She suffers from Stargardt's disease, a progressive macular degeneration that starts in the center of the field of vision. She was diagnosed at age 30, now has limited peripheral vision and really no depth perception, making divots, bumps and rocks unrecognizable. Something like a train track could be difficult to maneuver.

To navigate the marathon, Graham-Gray used two guides.

Runner Tim Skolzen, 27, ran with her and called out any road imperfections. As a back up, coach Mike Spinnler peddled a bike behind the two, ready to jump in if needed.

But guides are not always available. A problem she works around by training 80 to 90 miles a week running on a treadmill. A typical week also includes two to three miles of swimming and two or three intense weight sessions, according to Coach Spinnler.

Beyond Graham-Gray's grueling Olympic training commitments, she is the mother of three children, a 7-year-old and 5-year-old twins.

A lot of people with her diagnosis would have stopped running, Spinnler said, but, he said, "She is one of the toughest athletes I know. Remarkable."

Now that she qualifies, Graham-Gray will be able to run in the Olympic trials in Boston, April 2008.

Understanding Stargardt's Disease
by
Richard L. Windsor, O.D.
Laura K. Windsor, O.D.
The Low Vision Centers of Indiana

Stargardt's Disease is a form of macular dystrophy that begins early in life. Dr. Stargardt first described this condition in 1909 giving it its name. It is one of the most common forms of a juvenile macular degeneration. Stargardt's disease may occur in one of every 20,000 children over the age of 6 and is usually diagnosed before the age of 20. Boys and girls are equally affected by this condition. Over 25,000 Americans have Stargardt's disease.

Stargardt's is usually a recessive inherited condition requiring the person to receive a gene from each parent to cause the disease. However, there have been a number of cases identified as dominant inheritance, requiring only one gene from either parent. Recently researchers have identified the gene, ABCR, now called abca4, which causes Stargardt's Disease. This brings hope that a treatment will eventually be available.

With Stargardt's Disease, the macula and surrounding retina are affected. The macula is the very center of our retina. The images we see are focused on the retina like the film in the camera. Unlike camera film where every part of the film is equally sensitive, our retina concentrates the most sensitive vision in the very center. Additionally, our best color vision resides in the macula. Thus, damage to the macula results in loss of visual acuity or sharpness of vision, decreased color vision and small blind spots.

Diagnosis:

Early in the disease, the macula may appear normal which may slow the initial diagnosis of Stargardt's. Children may be misdiagnosed with a psychological vision loss given as the diagnosis. In time, characteristic changes occur in the retinas that help facilitate diagnosis. Fluorescein angiography is a test in which a dye is injected into the arm and the flow of this dye as it enters the eye is studied. The damaged retina sits above a layer called the choroids which is rich in blood vessels that supply nutrients to the retina. During angiography in a patient with Stargardt's, the damage to the retina blocks the flow of light from the choroids causing a "dark choroid" and this may be used to help diagnosis. This test alone is not considered to be completely diagnostic of the disease.

In later stages of Stargardt's Disease, the classic appearance of the retina allows a much easier diagnosis. The appearance of a "beaten metal" macula combined with small yellowish-white flecks (fundus flavimaculatus) in the peripheral retina is typical of Stargardt's disease.

Emotional Impact:

Adolescent years are difficult for every child, but imagine suddenly learning that your vision is failing. The sudden awareness to the child and parents that the child is losing vision can be devastating. The family and the child need to learn immediately about low vision care. Knowledge of the options to help the child can help the family and child put the problem in perspective. Counseling may be needed to help the child through their fears. Support groups or interaction between the parents of other Stargardt's patients can be beneficial.

Decrease of Visual Acuity:

Stargardt's disease may first be detected by a mild loss of visual acuity or sharpness of vision. In the early stages, however, the vision may be near normal. Visual acuity measurements may also vary due to the effects of light exposure and one should not be alarmed if your visual acuity varies on each test. Most Stargardt's patients have visual acuities from 20/100 to 20/400. One study of Stargardt's patients found that all persons tested had decreased visual acuities of at least 20/200 within approximately nine years after onset.

Come and Go Vision:

As Stargardt's disease progresses, patients may experience small areas of vision loss or blind spots. As images fall upon the damaged areas of the retina, objects may disappear and reappear causing a come and go effect.

Eccentric Viewing:

Stargardt's disease creates central blindspots that increase in size as the disease progresses. Patients learn to turn their eyes in a specific direction to see around the blindspots. They must place the image on an area adjacent the macula. Family members and teachers need to understand that this is an adaptive step used to maximize their vision.

Photostress and Dark Adaptation:

Children with Stargardt's disease often complain of difficulty adapting to the dark after sunlight exposure. Light striking our retina causes chemical reactions to occur in the rods and the cones. Our retina must continuously create new photo-reactive chemicals and remove the waste products of these chemical reactions. When a Stargardt's patient is exposed to bright sunlight, the retina may become bleached by the light and the sharpness of vision may decrease and blind spots may become denser. These are temporary conditions, but can be prevented or lessened by use of sun filters and hats.

From laboratory studies in mice, it has been suggested that sun filters may lessen the formation of lipofuscin, which is the waste products of the rods and cones. Lipofuscin, if allowed to accumulate, may damage the retina. Mice, without the ABCR gene like patients with Stargardt's, do not accumulate these waste products when raised in darkness. Additionally, younger patients, under 26 years old, transmit more ultraviolet light to the retina due to the clarity of the crystalline lens which in theory may lead to more retinal damage in Stargardt's patients. More research is needed.

Photophobia:

The damage to the retina also leads to greater internal reflection of light often causing an increase in light sensitivity.

Color Vision:

Though new theories indicate the damage may begin in rod cells, our black and white vision, surrounding the macula, the condition eventually damages the macular area where cone or color vision cells are. Color vision declines as the disease progresses, but patients usually maintain a significant amount of color vision.

The Paradox of Peripheral Vision Sensitivity:

Patient may miss or see poorly objects that fall in their central vision, but the far peripheral vision remains intact with Stargardt's. It is not uncommon to not be able to see a face, but notice a piece of lint on the shoulder. Family members often mistake this ability as an indication that the patient can see better than he or she claims.

Phantom Vision / Charles Bonnet Syndrome:

In more severe stages of vision loss, patients may experience Phantom vision or visual hallucinations. These episodes are not usually related to underlying psychiatric problems, but rather are normal attempt by the brain to make sense of impaired sensory information. The brain may embellish the image making it very real just as it does in our dreams.

Depth Perception:

Depth perception is dependent on two good eyes. Anything that decreases vision in one or both eyes will cause an immediate drop in our depth perception.

The Good News:

Stargardt's never causes total vision loss. Peripheral vision is left intact. Central vision is usually in the range of 20/100 to 20/400 with younger patients usually showing less loss. Low vision care can help Stargardt's patients lead very normal lives. Following diagnosis every Stargardt's patient should have a low vision examination by a doctor skilled in low vision rehabilitation.

Low Vision Care:

Stargardt's patients respond well to magnification. Simple bifocals may be used in the early stages. In later stages, CCTV systems are helpful. It is important to maintain good cosmetic appearance for young patients. Mobility is usually minimally affected. Some Stargardt's patients can become bioptic drivers, but it may be for a limited time.

School Age Issues

In children and teens with Stargardt's disease, it is very important to have adequate low vision care. These students may have light and glare control problems in their classrooms. Adaptations including sitting away from the window or shutting the curtains may be necessary. Also, these students should be allowed to wear special sun filters when needed to decrease glare and light sensitivity.

Large print materials may be indicated for these children. This may include textbooks, worksheets, and tests. Due to their vision loss and difficulty reading, some students may require extended time on tests and quizzes.

Because the vision loss may progress over time, each student should have a Teacher of the Visually Impaired. A VI teacher will assess the classroom and educational plan for the child. With the low vision specialist's recommendations, the VI teacher makes adaptations including low vision devices, large print materials and other special services the child will need each year in school.

Because of the vision loss, there are also safety issues to look at in young active students. Eyewear with polycarbonate lenses should be worn for protection of the eyes from unexpected injury. Counseling should include safety issues in physical education. Sports like swimming and track are good options. Sports with fast moving projectiles may put young Stargardt's patients at risk. Protective face shields are essential if the patient is to play in such a sport. With the proper safety devices, patients have played hockey successfully.

Good Advise from a Stargardt's Patient:

"Don't listen to negative influences. Believe in yourself, and show others what you can do. Only "you" can find your potential."

Marla Runyan, US Olympian and Stargardt's patient and the first legally blind athlete to compete in the Olympics


--------------------------------------------------------------------------------
About the Authors:

Drs. Richard and Laura Windsor are a father and daughter team of low vision specialists at the Low Vision Centers of Indiana. Dr. Laura Windsor was recently honored with the 2001 National Essilor Award. Her father is past recipient of the American Optometric Association's National Optometrist of the Year 1999. They have recently completed a new movie on bioptic driving. Their work and patients have been featured on Breakthroughs in Science and the CBS Evening News: Eye on America. You may reach them at:

Central Office:
Low Vision Centers of Indiana
P.O. Box 166
315 Huggins Drive
Hartford City, IN 47348
Tel: 765.348.2020
Website: www.eyeassociates.com
Other Offices:
Indianapolis: 844.0919 - 9002 N Meridian - Suite 208
Marion: 765.664.9637 - 711-A River Dr 46952
Ft. Wayne: 800.393.5555 or 260.432.0575 - 6708-B Constitution Dr 46804

Dr. Laura Windsor drlaura@eyeassociates.com
Dr. Richard Windsor richw@eyeassociates.com

Article Source Link: www.visionww.org/drswindsor-stargardt.htm