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Usher Syndrome: Living (& Loving) Life With NOT One But Two Disabilities



Six years ago, I wouldn’t have included the words “& loving” in the title above. In May 2018, my optometrist told me I couldn’t drive myself home after my appointment, let alone ever sit behind a car steering wheel again!


My vision loss was below the legal driving requirements and even worse, she could 99% confirm that I had retinitis pigmentosa (RP).

 

The bad news didn’t end there – she advised me to get tested for Usher syndrome because I was born hard of hearing as well. I went into complete denial when she told me to contact a guide dog association to start training on how to use THE white cane.


That day was one of the worst days of my life.

 

Late last year, I was telling someone at a friend’s party what a life coach does. I also shared that I wrote blogs for my website – she asked if I wrote about my disabilities and that I should create more awareness about Usher syndrome.


This prompted me to write today’s post.  

 

My Journey With NOT One But Two Disabilities

I first heard the words “retinitis pigmentosa” in 2006 when an ophthalmologist hinted that I may have this condition. I chose to ignore what he told me and went on living life. But, when I heard those daunting words again in 2018, I had to pay attention, especially as I had to hang my car keys up for good.

 

I wrote snippets of my journey with Usher syndrome in 2022 when I blogged about not quitting when life gets you down and does your story serve you. However, this blog is about creating awareness around a condition that’s quite rare and not known or understood by many people.

 

What is Usher Syndrome? (The Simple Version)

It’s estimated that up to 400,000 people worldwide have Usher syndrome. But, what is this condition? I promise to keep it as simple as possible but you can read more about it by Googling it.


According to the National Eye Institute, Usher syndrome “is a rare genetic condition that affects both hearing and vision.”

 

There are three types of this condition:

  • Type 1: Profound hearing loss or deafness at birth with loss of night vision by the age of 10. Severe vision loss sets in around midlife and people with this type also battle with balance problems.

  • Type 2: Moderate to severe hearing loss at birth or in early childhood with loss of night vision by your teenage years. Sufferers have normal balance but can expect to have severe vision loss by midlife.

  • Type 3: Normal hearing at birth but onset of deafness in childhood with similar vision loss and balance to that of Type 2.

 

Deafness or hard of hearing in Usher syndrome is caused by the abnormal development of hair cells in the inner ear. RP symptoms are caused by the progressive degeneration of cells in the retina of the eye.


Besides losing night vision, RP sufferers also battle with progressive loss of peripheral vision, leaving them with tunnel vision only.

 

Usher syndrome is an inherited condition and both parents need to be carriers of the same mutated Usher syndrome gene for a child to potentially end up with the disease. In this case, there’s a one in four chance of a child inheriting Ushers syndrome.


I come from a family of four children – guess who hit the jackpot prize?



 

My Usher Syndrome Diagnosis

Miracles do happen in strange ways and I was fortunate to have a friend who had been suffering similar hearing and vision loss all her life. She received the same doomsday instructions from the optometrist just months before I did (which is why I ended up having an eye check-up a few months later!)

 

After my appointment with the optometrist, Sue and I spent a morning at a coffee shop. Admittedly, we wallowed in misery over a delicious slice of chocolate cake bemoaning the fact that we were now living with TWO disabilities with no cure in sight or hearing distance!


However, we quickly realized that we had a choice – we could become victims of our situation or we could be proactive. Thankfully, we chose the second option.

 

We immediately contacted S A Guide Dogs, expecting to be handed cute bundles of four-legged guides. Instead, much to our horror, we were introduced to a (wonderful) two-legged orientation and mobility trainer who promptly gave us white canes.


How we hated that first time (and many times thereafter) wearing our disability badge so blatantly obvious. But with caring and firm guidance from our O & M trainer, we soon discovered that our white cane was our BFF!

 

The other piece of proactive advice we took was to undergo genetic testing which would:

  • Confirm if we had Ushers syndrome and,

  • Determine which type we had.


We met with a genetic counselor who explained the process, took saliva swabs, and sent them off to Estonia. In anticipation, we waited for our results.


In February 2019, I was called into my genetic counselor’s office – a) yes, I had Usher syndrome and b) I was Type 2. Now, what was I meant to do with this information?

 

Learning to Live and Love Life with Usher Syndrome

The first thing I had to accept was that in 2019 there was no known cure for Usher syndrome. That meant I had to face the possibility of eventually going completely deaf and blind.


This was a HUGE ask from me at that time and while I got used to and was grateful for my white cane (and hearing aids), I was angry that I had to live with two debilitating disabilities.

 

I began to isolate myself and to be honest, I was grateful when the COVID-19 pandemic forced a lockdown in South Africa. Now, I could pretend I wasn’t blind or deaf in the comfort of my own home without being forced to display my disabilities by walking in public with a white cane.

 

Yes, I was scared that if I caught the virus it would speed up my hearing or vision loss but I ignored that possibility as much as I could. Another benefit of the pandemic (for me) was choosing to quit my full-time job and go self-employed with my life coaching business online. That was the best move I have made in a very long time!

 

How Life Coaching and Shamanic Principles Empowered Me

My partner’s love and support were invaluable during the initial diagnosis stages. However, it was my personal growth and development journey that I began in 2014 that empowered me to accept my condition. I eventually learned to embrace life to its fullest, despite the challenges of losing my peripheral vision.

 

Through training as a life coach, I was equipped with powerful self-empowerment tools which I turned to over and over again during the first few years after my Usher syndrome diagnosis.


My shamanic training also supported me in more ways than one – I continued to do inner work with my tarot cards, meditated every day, and talked non-stop with my spirit guides!

 

I can gratefully say today, that my personal growth and development journey was the best thing I ever did for myself. Not only did I discover my true SELF but I was given incredible tools that opened my mindset, changed my perspectives on life (and my disabilities), and allowed me to come to a place of acceptance.



 

The Way Forward

When lockdown was finally lifted, I slowly rejoined life with my white cane firmly in hand. I no longer felt ashamed of it and was open to discussing my condition with curious bystanders who wondered if I was really blind!

 

Sue decided to go for a guide dog while I chose to stick with my cane. We both continued with our hearing aids but Sue took the big brave step of having a cochlear implant in January 2023. I will be following in her footsteps later this year – watch the space!


We’re both grateful that this option is available to us – if (IF in capital letters) we ever do lose our eyesight completely at least we can hear.

 

Great strides are taking place concerning improving sight for people battling RP. I recently discovered glasses similar to space-age gear that can improve your peripheral vision with the use of a camera. Technology is amazing!

 

Gene therapy is a potential treatment for those of us living with RP and I truly believe that people like myself and Sue have hope of seeing in the future. For now, I’m extremely grateful that I have better central vision than most people and that I only need to wear reading glasses.

 

My white cane gives me the freedom to move around on my own although I do enjoy using my partner as my long-suffering and patient human guide. If not, why not, I say! I have chosen to live AND love life fully now and I do it all in the present. I don’t think about tomorrow but instead, choose to embrace every moment as they come.

 

Final Thoughts

Embracing two disabilities wasn’t easy but over time, I began to realize that they came with many wonderful benefits. Most of the time, I’m met with much kindness from others and I’ve been introduced to some special souls along the way because of my condition.

 

I get to go to the front of the queue at the bank, Home Affairs, and other places that often entail standing for hours waiting for a service! The treatment I have received in these places has been phenomenal.


I’m fully assisted whenever I fly with an airline – I don’t need to stress about trying to navigate busy airports on my own anymore. I am always well taken care of!

 

Going online with my coaching business has opened up a whole new world for me and I now coach amazing people worldwide. I “see and hear” life differently now and as a result, I’m more in tune with my intuition and inner wisdom. I am truly blessed.

 

Would I have it any other way now? No. Usher syndrome has taught me to be grateful, to live in the NOW, and to love life to its fullest. I don’t take anything for granted anymore – every day is another opportunity to marvel at the sunrises and sunsets, the moon and the trees.


I choose to be hopeful and to celebrate life and love. It took not ONE but two disabilities to show me the way.

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