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11 Facts You Should Know About LHON
11 Facts You Should Know About LHON
No one can prepare themselves for a life altering disability. The best you can try to do is gather an understanding of what is going on with your body. We’re glad to share this information and to help anyone going through the condition, with these 11 facts about Leber Hereditary Optic Neuropathy.Onset effects Young Adults
When Dreamscape founder learned of his disability, he was only 16 years old. Often, we listen to similar stories of those effected by LHON. People usually develop LHON mid-late teens, and is described as loss of sharpness and a fading of color vision. The vision loss mainly affects central vision, which is needed for tasks such as reading, driving and recognizing faces. In a small percentage of cases, the central vision loss can improve; but in most cases loss of vision is permanent. It’s only after a year or so living with the disability, is when people fully adapt to their new surroundings. With the help of accessible technology, and charities like Dreamscape Foundation, you too can find the freedom you are looking for!LHON is Painless but Quick Acting
You can rest a bit easier to learn that LHON’s onset is a painless one. The experience on the other hand, can throw quite a curve ball. It only takes a few moments for the central vision loss to kick in, leaving only peripheral view. We often hear stories of rapid blindness, usually after rubbing ones eye. In some cases, one eye will start to be effected before the other. The other eye’s condition soon worsens, at an average of 8 weeks after the initial onset. LHON is still has a cloudy reputation, as most people have never heard of the hereditary disability. It’s a good reminder to know that most of the visually disabled are not 100% blind! This can be a common misunderstanding, due to outdated stereotypes of the blind.LHON is Transmitted by the Mother
LHON is passed down to children primarily due to mutations in the mitochondrial (rather than the male nuclear) genome, and only the egg contributes mitochondria to the embryo. Mitochondria constantly convert energy locked in our food into energy that the cell can use, and are small sub-units that reside within the cell. LHON follows a mitochondrial pattern of inheritance, also known as maternal inheritance. Only egg cells contribute mitochondria to a developing embryo, therefore only females can pass mitochondrial conditions to their children. Fathers affected by LHON or carrying LHON mutations do not pass the condition to their children.Discovery of Disability
This disease was first written and described by the German ophthalmologist Theodor Leber in 1871, only 31 years old at the time. In said paper, Leber described 4 families in which a number of young men suffered abrupt loss of vision in both eyes either simultaneously or sequentially. This disease was initially thought to be nuclear linked but was subsequently shown to be mitochondrial.Other Useful LHON Facts
- LHON affects both males and females, but more commonly found in men.
- The birth prevalence of LHON is approximately 1 in 50,000 people.
- More than 50% of men and more than 85% of women with a mitochondrial mutation will never experience vision loss.
- Without a known family history of LHON the diagnosis usually requires a neuro-ophthalmological evaluation and blood testing for mitochondrial DNA assessment.
- “LHON Plus” is a name given to a rare variant of the disorder with eye disease together with other conditions. (Many cases of LHON plus have been comparable to multiple sclerosis because of the lack of muscular control.)
- In 1988 Wallace et al. identified the nature of the first known causative mutation (50-70% of all LHON cases).
- The other two mutations known to cause LHON were identified in 1991 (8-25% of all LHON cases) and 1992 (10-15% of all LHON cases).
Justin Holland: A Blind Guy with a Vision
Justin Holland: A Blind Guy with a Vision
Eighteen is a pivotal age for anyone. As we graduate high school and begin our first steps into the adult world through work and higher education, our life begins to change in a drastic way. Yet for Justin Holland, “drastic” was an understatement. In 2016, just as preparing to go to college to study engineering, something unexpected happened. He began to notice a spot in his eyesight that within two months would leave him legally blind.A Lifechanging Diagnosis
After months without answers, Justin discovered he had a rare genetic disease called Leber’s Hereditary Optic Neuropathy, or LHON. This degenerative disease is irreversible, robbing him of his sight and leaving him with only light perception to guide him. “It happened so fast,” Justin admitted. “It was really weird. Even the doctor didn’t believe it.” Yet the reality was coming on fast, as his vision faded at a rapid rate. So quickly, in fact, that it was hard for him to grasp what was happening. “You could say I was being positive, but now, looking back on it, it was me denying what was happening,” Justin explained. “I didn’t know for sure it was going to keep getting worse, but I didn’t want to accept that it was. I even went and got new glasses to see if it would do anything, and, of course, it didn’t.” Suddenly the future of going to college and becoming an engineer slid to the backburner. Instead, he stayed home with his parents as he fought to adapt to life without sight. He had to relearn everything, from how to navigate inside and out of his home to how to do everyday tasks like pick out clothes, get dressed, and prepare food. “Dealing with the situation, it was difficult, and the shock of it is what hit me the most,” Justin said. “I tried to stay as busy as possible and that’s what helped me. Staying busy, going to the gym, and having my family.” That family became his cornerstone, supporting him as he began to reorient himself with the world through assistive technology, a guide dog, a support cane, and the unyielding encouragement of his family and fiancé. “Being able to hang out at home and get a ride to the gym and get a ride to work, it was really awesome to have my family there,” he explained. “My mom was and still is a really big help in contacting people with services that I needed.” As difficult as his diagnosis was, Justin didn’t let it be an anchor on his life. Instead, he chose to keep moving forward.Justin Sets His Sights on Bodybuilding and YouTube
As Justin began finding ways to still be active and embrace life despite his new disability, he wanted a platform where he could share his journey. Familiar with filming, he turned to YouTube. After seeing what others like him were doing online, he found himself considering a different angle on the “blind life documentary” genre. “When I started going blind, I was still watching YouTube and I found Tommy Edison and a handful of other YouTubers, but none of them are really doing the fitness thing,” Justin said. Justin’s passion for fitness has been going strong since he was fourteen, and he wasn’t about to let LHON stop him from keeping that outlet. In fact, he even competed in a bodybuilding competition after his disability set in. “I’m a blind guy with a vision to show people that even though I’m blind, I’m still going to do the things I love,” he said. “I find a way to work around the blindness and go on adventures. A lot of the things I enjoy are active.” You’ll find many videos on Justin’s YouTube channel demonstrating his zeal for strength training, physical activity, and the great outdoors.Life Without Sight Can Still Have a Clear Vision
This isn’t to say that Justin hasn’t seen his share of struggles. Since LHON began to impact his life 2 years ago, there have still been many moments where he had to face the reality of this huge life change. At times, it was really difficult as he felt the pain of his loss and the frustration of his limitations. But at the end of the day, he chooses not to be paralyzed by them. Rather, he perseveres and embraces the things he can do. “I was always constantly trying to become stronger and become better,” he said. “That, for me, is what it’s all about. The hardest part was the mental battle. It’s a challenge like no other but challenging myself is what I’m all about and fitness really does it for me.” As for those who may be able to relate to his story, Justin has a word of advice. “Find something you enjoy doing that keeps you busy,” he said. “There’s all kinds of things that blind people can do. No matter what the disability is, you can still find things to do and live life.” « PreviousNext »Living with LHON: An Interview
Living with LHON: An Interview
Leber Hereditary Optic Neuropathy. Commonly referred to as LHON, the disease is a mouthful to pronounce. On average, only 100 United States citizens are diagnosed per year, yet those 100 lives are drastically changed the moment symptoms start to unfold. Dreamscape Foundation Founder Joe Sehwani is one of the few to inherit it unexpectedly. His experience was a core influence in the creation of our non-profit. Yet he isn’t the only one who had to learn how to live life anew thanks to this rare disease. Kenny and Justin Jongsma are two young examples. For the last 7 years, they’ve been adapting to the limitations brought on by LHON from their home in New Jersey. The disease impacts one’s central vision to a degree that is often irreversible. Below is their story, told from their own perspective. From the moment of diagnosis to how they adapted to living with their disability, the 28-year-old twins share their insight on what life has been like with LHON. Facing a Sudden Diagnosis
The onset of symptoms came without notice. Justin was the first to experience them. He was 21 and already used to wearing glasses and contacts, so when the symptoms first began he simply thought it was time for a new prescription. “I went to the eye doctor and she said, ‘That’s not good’,” he recounted. “Of course, that’s not something you want to hear.” He booked additional appointments, but it wasn’t until he woke up one day and couldn’t see his television that he realized something was truly wrong. From that point on, his vision began to decline more rapidly. “I was very scared and very depressed for a while,” Justin confessed. “To wake up one day and not be able to do everything you love to do and are used to doing… I took it pretty rough.” Within a month or two, Kenny’s own symptoms followed. Together, they began searching for answers when they discovered that it was LHON, a hereditary disease carried by their mother. Their uncle had it, but neither of them had ever fathomed the disease would be passed on to them. “I started getting nervous,” Kenny said. “I didn’t know much about it. I had a lot of questions. How bad are my eyes going to get? When will it stop? Is it going to stop?” Their diagnosis did little to stave those fears, as the disease came with more questions than answers. Yet they had to press on and learn how to live with their newfound challenges, one step at a time.Living with LHON
Life changed drastically after Kenny and Justin lost their central vision. In many ways, they had to relearn how to navigate the world they had come to know so well. Their limited sight brought much of their lifestyle to a screeching halt as they tried to figure out what they could and could not do. “It pretty much changed every aspect of how we live,” Kenny explained. “I was working a different job back then where I was doing a little of paperwork and computer work. When I could no longer see what I was doing, I was forced to go on disability and quit my job. “It got to the point where I couldn’t drive anymore and do things like reading, video games, or watching TV. For a while, I was afraid to walk down the street because I might bump into somebody or run into a sign.” “Everything was definitely affected,” Justin agreed, “But I didn’t let [LHON] take control of everything. I refused to let it do that.” Determined, the twins took a step back and found ways to adapt. From identifying their limitations to relearning how to do the things they loved most, they carved a new way to live, work, and have fun along the way. “Our friends are understanding,” Kenny said. “They have no problem picking us up and dropping us off. Same goes for our coworkers. We get rides to work. As far as hobbies, I’m very into video games and that’s something that really changed. “But it was one of those things that I refused to get up. I found a way around it. It took some saving, but I bought myself a large screen TV. When I sit closer to it, I can see more of the details. I’m not as good as I used to be, but with practice, I can still enjoy it.” Both Justin and Kenny now work together at a local supermarket where they handle overnight stocking. Their boss, mindful of their strengths and limitations, provides them with responsibilities they can tackle with confidence. There are also many things made possible thanks to the accessibility provided through everyday technology. “In the beginning, I had this magnifier that helped a little bit, but it was only for reading so it was very limited,” Justin recalled. “Eventually, it got to the point that it didn’t help me anymore so I just use my phone.” Their iPhones offer multiple features, including zoom, voice-to-text, and Siri, to make everyday activities not only easier but possible. “As far as doing anything online, the iPad really helps with that,” Kenny elaborated. “I use the iPad Pro – the big one – and it’s really helpful. It has contrast options or inverts the colors if you need to. For me, the contrast really helps to see things a little better. We also use the magnifying option on our phones at work if we need to read something written in small print.” The brothers are also exploring a new piece of technology on the market called eSight, which are electronic glasses designed to make seeing easier for the legally blind. “It must have been hard for people dealing with this condition 20, 30 years ago when we didn’t have these things,” Kenny admitted, to which Justin quickly agreed. But technology wasn’t the only aspect that made life with LHON possible. The twins attribute much of their positive attitude, tenacity, and ability to embrace life to the unyielding support of their friends and family. “Everyone is willing to help us,” Kenny said. “They don’t show any sign of an issue. If we need or want to go somewhere, our parents are more than happy to take us. As far as friends, they’re more than willing to pick us up and drop us off or take us to wherever we’re hanging out. There’s definitely a ton of support there.” “Absolutely,” Justin agreed. “Almost everybody we ever talked to about it has been supportive of us. Even our boss helps us out.”A Word of Hope for Others
Living with LHON is a challenge. One that both Justin and Kenny will admit isn’t always easy. Although they endure and overcome its challenges together, they’ve experienced both highs and lows along the way. In these moments, the twins find their optimism and inspiration in others. “Ever since I started going through this, I have had a humongous respect for anybody that has a handicap or disorder they have to go through in life,” Justin said. “Everything you go through takes a toll. To be strong enough to go on and deal with anything like that is very courageous. Seeing other people deal with these problems gave me courage and made me very positive. “Every day is a constant reminder that I can’t see anything, but I never let it bring me down anymore. I had that time in my life and it’s over.” Justin’s words, spoken with conviction, were shared by his brother. When asked what advice they would give to others in similar circumstances, they didn’t hold back. “Whether it’s this or any disability or negative thing you’re dealing with in life, don’t spiral down into depression. Don’t let it stop you,” Kenny stressed. “There may be things that your situation makes difficult. There may be things you may have to give up in certain situations. But you can’t spend the rest of your life wishing it could be different. If there’s nothing you can do about it, you have to find a way to push through it. “That’s what we’re doing. We’re doing the best we can with the hand that we were dealt and we’re not letting it bring us down.” “It’s a lot easier said than done,” Justin confessed. “So do your best to find support. Even if it’s just from one person, like a family, friend, or loved one, support is everything. That’s what got me and Kenny through a lot of stuff. If you don’t have anybody there, it makes it a lot darker and lonely. Support is what helped us.” Whatever your disability may be, Dreamscape Foundation encourages others to become rooted in both hope and support. For more information on support resources or how you can get involved in making life more accessible for those with disabilities, reach out to us. « PreviousNext »10 Different Ways to Prevent Blindness
10 Different Ways to Prevent Blindness
Most people take the ability of sight for granted, never thinking that they could become blind. Glaucoma, cataracts, diabetes and macular degeneration are just a few of the ways one can lose their eyesight. Below, we have broken down the multiple ways one can prevent eye damage and vision loss.