Family Stories

Content of the Family Stories and Blogs is the personal views and experiences of the authors and should not be read as action or advice from the Norrie Disease Foundation.

Cameron’s Smile – Progressive Hearing Loss

Cameron playing outsideAs my son was born totally blind, it quickly became ‘our normal’. But despite being told all the possibilities of additional needs Norrie Disease brings, one thing we never mentally prepared ourselves for was progressive hearing loss. Over the months we noticed a change in his behaviour. Going out in noisy crowded places became a huge problem and would quickly send him into a sensory overload meltdown.

It was difficult to manage his frustrations and his severely delayed communication skills were showing no progress. After years of recurrent ear infections and traumatising pain, I began to notice certain sounds were somewhat painful to his ears.

After being closely monitored by audiology every 6 months, we decided to insert grommets – where it was then discovered he had full hearing loss in one ear at the age of 3.

When people learn about his blindness, the first thing that everybody says without fail, is how his ‘super senses’ will compensate for his vision, especially his hearing. But for Cameron the most important sense is touch. Unfortunately having dual sensory loss can prove difficult at times, he is still unable to form communication and language skills and we have to adapt our surroundings and environment to help him reach his full potential.

Every day we live in fear that the progressive hearing loss will affect his other ear before he develops the skills to talk, something I so long to hear. It is a ticking time bomb in the corner of the room just waiting to happen.

The grommets were completely life changing, helping to manage the pain and help keep his one hearing ear clear of fluid and congestion.

My son has to work ten times harder to allocate sounds and process them. He can not see what is making the noise and his hearing is severely affected when there is just too much background noise. Having a multi sensory impairment has caused my son to have very complex additional needs, so much more than just blindness or hearing loss alone.

When one loss accompanies the other, we feel the disability is far greater and much more difficult to overcome.

The Norrie Disease Foundation is developing a patient registry to support pioneering research into the Norrie disease hearing loss.

Samuel Wilkins – Using technology in daily life

Samuel and his dog PilotSamuel lives in Gillingham in Kent. Norrie disease means that Samuel is blind, partially deaf, and on the autistic spectrum. Samuel volunteers as a telephone befriender for carers. Samuel says ‘I find daily life fairly easy to handle, and I do my best to concentrate on the things I can do, rather than what I cannot do’. True, there are times when I get frustrated, and sometimes I feel I need to let off steam, but for the most part, I don’t let it get to me.  Moreover, my Christian faith is very important to me, and helps me get through having disabilities’.

Samuel says ‘It’s been very important for me to know how to use technology and to fix any bugs I find, and to keep on top of what is available. Technology is very important for blind and partially sighted people because without screen readers on computers and phones, Braille tablets, adapted domestic equipment and accessible toys, (to name but a few examples) we would not have so many advantages as fully sighted people.  However, it is also a good idea to find equipment that can easily be adapted by you personally. I have marked the knobs on an oven with a tactile marker, which is less expensive and easier to maintain than buying an expensive piece of specialist equipment’.

One of the top pieces of technology I have used is a screen reader on the PC.  I used the Window-Eyes screen reader from 2003, up until it ceased development in 2017.  Since then, I have been using the NVDA screen reader, but I also have experience of Jaws.

Regardless of the screen reader used, they are invaluable tools for anyone on a PC or Macintosh computer, as they can give access to all sorts of features, such as editing text, reading emails, working with the internet and scanning documents and converting Kindle books.

I also learned to touch type, which is a very important skill to have, as it makes writing documents a lot quicker.  Here blind people have an advantage over their sighted peers, many of whom cannot touch type or do not touch type well.

Another piece of technology I use a lot is the BrailleSense Braille tablet.  This is an Android based tablet that has many functions, including word processor, schedule manager, email, web browser, the ability to connect to phones and computers, and the ability to download apps from the Google Play store. This device, and other similar products, such as the BrailleNote, can be invaluable in many situations e.g. reading books, working in a classroom, office work, and writing notes.  In fact, these technologies can make learning Braille easier for both blind and sighted people, as the Braille is very clear, and since there is a screen as well, a sighted person can compare what a print symbol looks like with the same symbol in Braille.  I would even say that despite what some people have said, Braille is not dead, and if you have the ability to learn it, then it will be invaluable to you’. 

My IPhone is something I never leave home without. Every iPhone has a screen reader function (voiceover) built in, and don’t worry if you have an android phone, a screen reader can be installed.  Even with a touch screen, it is possible to learn how to use these phones in a relatively short time, and if using a BrailleSense or BrailleNote, all functions can be controlled by the Tablet, so all private information is read in Braille while the iPhone stays safely in its case. Not only does this make your information more private, but it also means you can text, or email with the speech turned off, thus avoiding annoying fellow train passengers or office colleagues with a constantly chatting phone.

There are countless apps that can be downloaded onto accessible smartphones, however it is often trial and error as to whether they are accessible. I often use the Moovit app (a public transport information app) because I travel around Kent regularly, and need to plan my journeys. I also use GPS on the phone, however I would advise caution when using any form of navigational aid. GPS systems cannot warn you that someone is coming up to you, or stop you from bumping into a lamppost. They also can’t tell you if it is safe to cross the road, or if this particular road has four way traffic.  Also remember that the map data may not always be up to date.  GPS systems won’t replace your cane or guide dog either. “