LIVING WITH LHON

 
  1. 1. Emotions

  2. 2. Mobility

  3. 3. Assistive Technology, Reading & Films

  4. 4. Social Security

  5. 5. Rehabilitation

  6. 6. School/College

  7. 7. Careers

  8. 8. Activities/Sports

  9. 9. Explaining LHON

  10. 10.  Resources

  11. 11.  Genealogy

  12. 12.  Vision Tests

  13. 13.  Lifestyle Choices


1. Emotions


Everything about LHON is emotion-laden, both for the patient and for family members.  Speaking with a counselor can be helpful in talking through the grief, fear and anxiety the sudden loss of vision can produce.  The patient and family members can all benefit from finding a counselor with experience supporting people dealing with vision loss.  These specialists can often be found at non-profit organizations devoted to supporting people with blindness.


Adjusting to LHON vision loss can lead to lots of crying, and feelings of depression.  It’s normal, but it’s important to make sure it doesn’t get too intense.   Many other families with blindness have a very different sequence of emotional adjustment.  Often blindness occurs in babies, so the parents can deal with their shock before the child is aware.  With LHON, both the patient and their family members must deal with shock and grief all at the same time, while striving to stay positive for the others.  


One of the major challenges of LHON is coping with loss. Losing the ability to drive, which means losing some of one’s independence.  Losing the ability to see and recognize faces.  Losing the ability to read standard text.  Coping with loss involves grieving…mourning what’s been lost and adapting to what remains.


An aspect of LHON that can create additional emotional distress is the possibility of spontaneous recovery.  The odds of spontaneous recovery differ based upon the specific mutation you have. The good news is that there is always hope that you will be one of the lucky ones whose vision improves at some point in time.  The bad news is that this hope can make it difficult to fully adapt to the vision loss, hoping that you’ll return to “normal.”  If your vision does not improve after a year or more of waiting, then you may experience the depression and sadness of the initial vision loss all over again.  Striking a balance between acceptance of vision loss and hope for vision recovery is an ongoing challenge of LHON.


Keeping a positive attitude is one of the most important aspects of dealing with LHON.  It’s easy to give in to sadness from the vision loss, but it is quite possible to live a full and meaningful life with LHON vision.  That doesn’t mean it’s easy.  Some with LHON vision wake up each day and curse the fact that their vision isn’t normal, then they just get on with it, and make that day the best it can be.  Others eventually embrace their new lives as visually impaired, and enjoy the awareness that comes with recognizing that life’s obstacles can be overcome.


Since the LHON gene is passed maternally, some mothers feel a sense of responsibility for their child’s vision loss.  Yet like all genes there’s nothing that can be done about it so it’s helpful to focus on all the good genes that were passed to your children that will help them cope with this disorder, rather than to dwell on the negative.



Connecting with others who have lost vision and who are leading rich, productive lives can help with the adjustment to LHON vision.  Since LHON is rare it’s difficult to find others living nearby with the condition.  One approach is to click on the Register tab at the top of this page and ask to be connected with local LHON families.  Joining the LHON Facebook group offers lots of information and support.  Other possibliities are to connect with people with other vision disorders such as Retinitis Pigmentosis (RP) or Stargardt’s which are more common than LHON and are similar in that vision is lost at a relatively comparable age.  However these conditions are different in that they are progressive.  This gives the patient a longer timeframe to adapt to the vision loss, but creates a dimension of perpetual fear since those patients are always aware their vision will get worse, compared with LHON where the vision loss is rapid and profound.


The American Foundation for the Blind (AFB) has an excellent resource called FamilyConnect, for parents of children with visual impairments.  Several articles on the emotional impact of learning that your child is visually impaired are at: 

     www.familyconnect.org/parentsite.asp?SectionID=80&TopicID=370


The Hadley School for the Blind offers free correspondence courses for people with vision loss and their families.  You communicate with a teacher as needed toll-free or via email.  They have courses such as “Self-Esteem and Adjustment to Vision Loss.”

     www.hadley.edu



2.  Mobility


When vision is lost, it’s natural to wonder how you’ll get around.  Most with LHON vision retain their peripheral vision, which gives enough sight to walk around without seeming to be blind.  Since there’s no visible symbol of the vision loss, others may not react appropriately.  Some with LHON vision carry a white cane which helps make clear there’s vision loss; some use a guide dog.  Often, the local blind services organization or state rehabilitation program offers Orientation & Mobility (O&M) training which includes how to cross the street safely and how to access public transportation.


Since driving is not an option with LHON vision, it’s helpful to live in an area where services can be easily accessed without a car.  Locations can be assessed at this website:

  www.walkscore.com




3.  Assistive Technology, Reading & Films


There is an extensive variety of assistive technology available for people with low vision, and the options continue to increase.  With adequate magnification, such as with a CCTV, the remaining peripheral vision can allow those with LHON vision to read.  Talking cell phones, and computer software such as ZoomText, Kurzweil and JAWS, as well as the built-in accessibility features of Apple products, are used by many with LHON.


                                          


Most insurance companies do not cover such equipment.  Some Flexible Spending Accounts do cover such assistive technology.  If you’re an adult and you work with your state’s Dept. of Rehabilitation, they may purchase some of this equipment as part of the “rehabilitation” process.  It takes time to go through that process.  If the vision loss occurs under age 18 then the parents’ income is considered when determining need.  Some agencies such as Lighthouse or The Braille Institute will loan equipment while awaiting the Dept. of Rehabilitation.  Students in school may be provided access to the technology needed to succeed in school and for college students there might be a student disability center with the required equipment available for use.  Community groups such as the Lions Club may offer grants.  The National Federation of the Blind (NFB) offers a technology loan program with a low interest rate, and the Association of Blind Citizens (ABC) offers an assistive technology fund at:
     http://blindcitizens.org/assistive_tech.htm

Sometimes family and friends wish to donate money to help purchase needed equipment.  One way to do so online is via the program called GiveForward:

    www.GiveForward.com


Another way to read is to access books in a digital format.  Several services exist for free access to electronic books simply by filling out a form signed by a medical professional confirming that the applicant is legally blind.  Special MP3 players with DAISY format may be required to access these materials.  Sources include:

    www.LearningAlly.org

    www.bookshare.org

    www.braillelibrary.org

Note that LearningAlly was formerly called RFB&D.  Much of the material offered by the free sources noted above has been recorded by volunteers so the quality of the readings is varied.  Other options may be more expensive but may have professional readers for a higher performance quality.  These books may be downloaded to more common MP3 players such as an iPod.  Sources include:

    www.bn.com


Going to the movies and watching DVD’s and TV becomes a challenge, as much of the detail cannot be seen with LHON vision.  Descriptive video provides the information that a vision impaired person cannot gather when watching a film or TV.  To learn more about movie theaters that offer descriptive video and to watch an example, visit this site:

     ncam.wgbh.org

For a list of TV shows with audio description available:

     http://main.wgbh.org/wgbh/pages/mag/services/description/ontv/

On online resource to find descriptive videos has both closed captioning (CC) and descriptive video (DV), so ignore the CC listings and select descriptive video in the drop down menu.  Call the theater in advance to ensure it’s available for all theaters and all showings, and ask how early to arrive since the equipment is only rarely used so it may take a while to get it set up properly.  Be sure they don’t set the audio to hearing impaired.

     http://www.captionfish.com

For free mp3 downloads of audio described movies, check out:

     www.blindmicemart.com/assets/product_images/movies2.html

    

One way of keeping up with the news is through the newsline service from NFB.  This free service provides audio access to over 300 newspapers and magazines.

     www.nfb.org/nfb/Newspapers_by_Phone.asp


Disneyworld offers wireless headsets that provide recorded narratives throughout the park.



4.  Social Security


Once vision in both eyes is 20/200 or worse, it’s possible to apply for Social Security benefits depending upon income and assets, work history, etc.   To get information about how Social Security works for those who are legally blind, check out this site:

  www.ssa.gov/pubs/10052.html


Setting up an appointment as soon as you are legally blind protects your benefits from the time the appointment is made.  By reading the information and preparing information in advance, the process will go more quickly, though not all will qualify for benefits.



5.  Rehabilitation


If the person with LHON vision loss is an adult, it’s worthwhile to contact your state’s Department of Rehabilitation to learn what they can do to assist in the adjustment to vision loss.  A counselor specializing in blind clients will probably need all the same information provided to the Social Security Department—evidence of blindness, medical records, etc.  Their process requires forms and meetings.  It may be helpful to find a counselor who lost their vision as an adult, which gave us exposure to another adult with vision loss as well as to the rehab process.


The counselor may refer the patient to a low vision specialist (and pay for the visit).  That specialist will assess low vision needs and make recommendations for items required for success in rehabilitation such as binocular glasses or a monocular telescope.  There may be a separate appointment with a representative from a company that makes high tech aids such as a CCTV, etc.  Once all the paperwork is complete, the client and the counselor agree on a “plan” whereby the state will pay for items required to get the blind person to their goal of working.  This can include all the required assistive technology and, if appropriate, college tuition, books and supplies.

   http://discoveryeye.org/blog/low-vision-what-to-do-when-theres-nothing-more-that-can-be-done/




6.  School/College


Since the typical age of onset is around 15-25, a large percentage of those affected by LHON vision loss are in high school or college.  For those in high school, there is usually a vision specialist associated with the school or the school district who can support the student in the transition from fully-sighted to vision-impaired.  For those in college there is usually a Student Disabilities Center that can help with absence notification to professors at the time of vision loss and provide access to assistive technology, a notetaker, testing accommodations such as extra time and a reader, etc.  However, since most students with vision impairments have had them since they were children and have been trained in living with vision loss before arriving at college, these centers may not be structured to provide a simple path for the transition from fully sighted to significantly vision impaired.  These services may be found in the community at a Lighthouse, Center for the Blind and Vision Impaired, or similar organization.


To learn more about the government’s responsibility to educate children with disabilities consider this FAQ document:

     www.ed.gov/about/offices/list/ocr/504faq.html


For students heading off to college, here are some tips:

     www.pacer.org/parent/php/PHP-c170.pdf


There are many scholarships available to students who are visually impaired.  

     www.nfb.org/nfb/scholarship_program.asp

     www.collegescholarships.org/scholarships/blind.htm

      www.bellaonline.com/articles/art28386.asp

      www.jgb.org/guildscholar.asp

      www.freeeducationguide.com/?Campaign_ID=5220&PID=CD15695

      www.disabilityscholarships.us/disability-scholarships/unknown-blind-disability-christian-scholarship/289/

      www.afb.org/Section.asp?Documentid=2962

      www.nfbcal.org/cabs/resources/scholarships.php

      http://blindcitizens.org/abc_scholarship.htm

     https://www.learningally.org/about-learning-ally/awards/

     http://www.onlineschools.org/financial-aid/disabilities/



7.  Careers


Some affected by LHON are already in a career, and need to figure out how to overcome their vision loss to continue with their career.  Others wonder what career could they possibly do with LHON vision.  Some ideas about careers and blindness can be found on this site:

     www.afb.org/Section.asp?SectionID=7


Information about the rights of employees with a disability, including legal blindness, can be found at:

     www.visionaware.org/what_is_the_americans_with_disabilities_act



8.  Activities/Sports


It’s important to find sports and activities that can be done successfully with LHON vision.  The USABA supports vision impaired athletes:

     www.usaba.org/Pages/movie/usaba_video.html

Some sports to consider include:

     Tandem bike riding - back seat only!

        www.visionaware.org/how_can_i_cycle_if_i_cant_drive

         bicyclingblind.org/

         www.facebook.com/bicyclingblind

        www.blindstokersclub.org

     Skiing/snowboarding

        www.visionaware.org/id_like_to_continue_skiing_any_advice

        www.adacs.org

      Golf – the ball doesn’t move, which is good.

Find a “coach/caddy” who can assist with aim and finding the ball.

        www.blindgolf.com

    Swimming - here is a link to a very successful LHON swimmer: 

http://news14.com/content/sports/612380/despite-condition--local-swimmer-refuses-to-leave-pool/?RegionCookie=104

    Rowing - like tandem biking, the person in back doesn’t need good vision   

    Ping pong – can’t get hurt by the ball, and peripheral vision may suffice

    Tennis – find an opponent who hits the ball accurately and with consistent pace

    Basketball – bounce passes only, please! 

    Running and triathalons are also popular - often with a visual interpreter to help guide the route 

        www.facebook.com/groups/running.eyes

    Beep ball - a form of baseball, adapted for the visually impaired.

       www.nbba.org

And many others...people with LHON have successfully played football, soccer and other sports when their teammates and coaches have been willing to work with them.  For additional ideas check out:

        www.visionaware.org/sports_exercise

        http://www.seacoastnh.com/arts/book-of-the-week/encyclopedia-of-sports-and-recreation-for-the-blind/


To obtain equipment, training funds or funds to travel to competitions in these activities, consider contacting an organization such as Challenged Athletes Foundation and applying for a grant:

     www.challengedathletes.org/


Other activities include online games.  Resources include:

    www.audiogames.net

    www.allinplay.com


A travel pass provides discounted access to U.S. National Parks:

     http://store.usgs.gov/pass/access_pass_application.pdf





9.  Explaining LHON


There are many medical challenges people face that are immediately understood by the general public.  When the diagnosis is cancer or Alzheimer’s, for example, people generally have a sense of the situation.  LHON is different—most people you speak with will have no reason to know what LHON is.  They’ll never have heard of mitochondrial disease.  And most will have had no real contact with anyone who is legally blind.  Often blindness is perceived as an all-or-nothing condition, so those with LHON find themselves explaining again and again what they can and can’t see, and how they’ve lost central vision but can get around using peripheral vision.  Often times people know someone with macular degeneration, and while macular degeneration is a retinal disorder which tends to onset late in life unlike LHON which is an optic nerve disease which tends to onset in young adults, the impact on vision is similar in both cases.


People often ask if you can have an operation to “fix” the eyes, or to have an eye transplant.  It’s helpful to explain that there are 3 key aspects to vision:  the eyes, the brain, and the optic nerve.  With LHON the eyes and the brain work just fine.  What’s not working is the optic nerve which functions similarly to a wire that transmits information from the eye to the brain and back.  With LHON the cells in the optic nerve are overdoing it on apoptosis—preprogrammed cell death—too many cells are committing suicide, causing the optic nerve to atrophy.  It’s like a TV cord that’s been chewed by a mouse.  Since optic nerve cells can’t regenerate, the ‘cord’ can’t be fixed (though some with LHON are fortunate in that their optic nerve cells don’t die, they’re just sick, hence there can be spontaneous recovery in some cases).


Someone with LHON may not “look blind,” and if they don’t use a cane or a guide dog, people may not understand their problems.  When people walk by and the person with LHON vision “ignores them,” or doesn’t say hi back because they can’t see the person who said “hello”, it’s awkward for everyone.  Teaching friends to say “hi, this is so-and-so” upon approaching someone with LHON vision, and encouraging other friends ‘in the know’ to tell the LHON vision person when someone they know is approaching, can be a big help in overcoming this awkwardness.


Since those with LHON vision usually have some peripheral vision, they tend to use that peripheral vision to see.  They may look straight ahead so that others don’t find their gaze disconcerting, yet will look upward or to the side to really see.


  

         

Looking straight ahead

trying to appear fully sighted

 
                        

Truly looking straight ahead

using LHON vision

 

In social situations with strangers one cannot choose who to talk to, let alone make eye contact or ‘read’ another person’s facial expressions.  This can cause a sense of apprehension or unease in social situations and the more that friends can help out, the better.



10.  Resources


Most resources for the blind are for those who either became blind as children, or those who lose vision at a relatively advanced age.  The age at which LHON vision onsets is far different so the needs are different.  Those affected by LHON may feel that they don’t fit in at facilities for the blind since everyone else there are much younger or much older.  People who have gone through childhood without sight have a different sense of the world than those who were once fully sighted and lost vision as with LHON.   People who lose sight after completing school, college and careers are impacted differently than those losing vision as teens or young adults.


Parents with a child losing vision to LHON may find help from NAPVI’s message board:

    www.familyconnect.org/parentsitehome.asp

While most NAPVI parents have younger children, there is also some helpful information about teens.  Since the site is monitored and there is someone representing the organization who responds to questions, it can be a helpful resource.


Since LHON is a mitochondrial disorder, it can be helpful to learn more about mito disease at:

    www.umdf.org/

However, it’s important to keep in mind that LHON is very, very different from most mito diseases.  Most mito diseases onset in childhood.  Many affect several systems of the body at once, and generally not vision.  Some are life-shortening.  None of these descriptions match LHON, so those affected by LHON should be aware that their situation is quite different.  The information is helpful in that researchers studying mitochondrial disease are studying the same issues as for LHON,  but the same symptoms tend to be much, much different.



11.  Genealogy


LHON can be a good reason to delve into the family’s genealogy.  It can be interesting to trace the maternal line through all the women who carried and passed the LHON gene, and the men who carried but didn’t pass it on.

Here’s a video by a woman whose brother was diagnosed, and how his being affected by LHON impacted her life.



























 

12.  Vision Tests


One of the most important tests taken by those with LHON is a Visual Field Test.  It tests where your eye can see and where it can’t.  Those with LHON tend to have what’s called a central scotoma; an area in the center of the visual field where no information is transmitted from the eye to the brain.


Another important test is an OCT (Optical Coherence Tomography).  This test measures the thickness of the optic nerve.  Generally the optic nerve is unusually thick at onset of vision loss, as the fibers are swollen.  Later the nerve becomes unusually thin, as the fibers atrophy.



13.  Lifestyle Choices


For people with LHON vision loss and for those carrying the LHON gene but not affected, there is usually a concern about what choices to make to increase the odds of spontaneous recovery and/or to reduce the odds of losing vision due to LHON.  Living as healthy a life as possible is usually recommended by medical professionals who understand LHON.  Most importantly, there has been research clearly linking smoking and smoke to LHON vision loss, including the following article:

    http://brain.oxfordjournals.org/cgi/content/abstract/awp158



This website should be considered general information only and should not be considered medical guidance or professional advice.  Always direct any questions concerning your personal health to your doctor or another appropriate health care professional