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Living with Ushers II is like?

Being a professional robberee! (the one being robbed!!) On an all too regular basis, emotionally and often practically it is like being robbed (or sometimes having the whole house burned down!!) I often say, your body is the biggest thief you’ll ever meet!

Being born with a ‘stable’ disability, or getting disabled at a very young age is one thing. It’s unfair, but from then on you learn the tricks and skills you need to function to the best of your ability.

You start from ‘point zero’ and work your way up from there. As was the case with Helen Keller, who fortunately had a supportive understanding family and more importantly, Ann Sullivan.

When you are totally blind, you do not try to see but find other ways of coping with it. The same is true for when you are totally deaf, you will not try to hear; you communicate in a different way. When you are totally deafblind, the challenges are that much greater, even ‘gynormous’, but it is still ‘point zero starting point’.

But when you are born with a progressive disability (like Usher syndrome) no matter what you do, you are moving TOWARDS point zero.

For those of us who try to see and hear and function in a hearing-sighted world, the energy level needed is growing exponentially with the increasing disability; it often is exhausting. What takes maybe 5% of a seeing/hearing person’s energy and brain power, will take up most of ours (75-95%)! Consequently we appear to have a very short fuse, are intolerant etc while in truth often this is as much stress as we can handle and anything else on top may be the last straw.

People generally do NOT understand the impact hearing loss has; after all you can do just about anything other than use the phone, or so they seem to think. They do NOT understand the alienation and isolation that follows, because communication has broken down. They do not understand that it is not just a matter of loss of volume but even more the loss of an ability to discriminate/distinguish different sounds.

“Alo when you a li-ning you a conanly illing in all the mi you ave no hur”
… … … also, when you are listening, you are constantly filling in all the bits you have not heard.

Remember, this is how we hear ALL THE TIME!!!!  So constantly the brain has to work out all the misheard and not heard syllables and letters. Never mind losing all the other pleasures, of music, birds singing, the different sounds water can make, etc.

As we are a society that uses vision as the major sense people respond to its loss with a lot more sympathy, as it is the disability ‘easiest to understand’. Luckily, Australians are accepting that a lot of ‘blind’ people can still see something and use their limited vision without judging one as a fake, at least in my experience, (there will always be exceptions of course).

But, the two together as one condition, in its many possible variations, is rarely, rarely understood by those who do not have to grapple with it and sometimes even by those who do, including the person suffering from the condition. I moved into a retirement village at 52 and I find that the only people who GET it are 85 or over for they have the same problems that I do!! (I have also had hip replacements and other back and joint problems, so I fit in well!!).

What often happens, when discussing my challenges with people, they will offer ideas and advice relating to my blindness, and I will have to remind them “But I am deaf!”, and when discussing my challenges regarding deafness, I will have to remind them “But I am blind!”

The challenges:

A: Different perception

Retinitis Pigmentosa (RP) causes tunnel vision, so we are told. But to us it doesn’t LOOK like a tunnel at all!!! Technically, that is how it FUNCTIONS, but it’s not how we see, or rather how we THINK we see.

Have you ever driven along a tree-lined road and the sun is low behind the trees? There is this light/shadow/light/shadow effect on the road that makes it hard to see and you have to slow right down? Now imagine instead of nice straight trees, the pattern is cast by random shapes (like tartan or irregular spots and stripes everywhere). The ‘tunnel’ we have consists of this kind of light/shadow pattern. But again, we are unaware of this. I asked another Usher person if he saw this; he said: NO. I turned the lights off so he would sit in the dark and made him think about what he was seeing. So often we, the people suffering the condition are unaware of this light/dark pattern. As a rule we are too busy trying to function to think about it and observe the actuality; life goes on.

Everybody has ONE blind spot, and they are totally unaware of that. With RP you have hundreds of them and you are unaware of most of them most of the time. On the other hand, when driving a car changing lanes, all of a sudden people are made aware of something they have missed before and nearly have an accident. This unawareness of the blind spot(s) until you are confronted with something unexpected, is typical in the case with RP too!!

B: Lying eyes/brain

To complicate things the human brain is a marvellous compensator and fills in all the blind spots, not accurately mind you, but it gives you a full picture. I find that as the condition worsens, the bigger something is (i.e the car closest to me) the more likely my brain will dismiss it; it is too big to make sense of and gets dismissed and ‘wiped’ from the picture (hearing aids are no help at all for me here). So the eyes/brain LIE like a trooper, about what you can see, sometimes that is dangerous; often it is CONFUSING. You sit down and you can see ‘fine’!

Your brain is putting the whole ‘puzzle’ of what is around you together very quickly (it also recognizes faces by putting  the puzzle together; with those closest to us we become very familiar with individual puzzle pieces and the process works at lightning speed). Then you get up and move (not necessarily to another area!!) and all of a sudden there is this: ‘shit, I can’t see enough to move’ moment happening. This happens sometimes umpteen times in a day, depending on what you are doing and where you are.

And eyes/brain tell lies in the opposite direction too!!! Seeing things that are NOT THERE!! Talk about confusion!! You may see ants crawling where there aren’t any, see prints on a blank wall or something way bigger even!! (my sister met someone who saw a horse in the kitchen!?) For example, when you are walking, out of the “corner’ (or what passes for the corner in RP) of your eye, you may glimpse something and come to a dead stop, thinking you are going to walk into, or stumble over something. There is nothing there, but you need to stop and reassure yourself you are not going to end up hurting yourself. This may be very confusing if not downright frustrating for someone guiding you, but it is almost impossible to trust them when you ‘see things’ while they do not, unless and until you are attuned to one another, and have built up this level of trust.

All this makes guiding/being guided a challenge for both and requires REAL skill on the part of the guide. On the one hand you want to keep your independence to some degree, on the other hand there are times you will hold on rather tightly, because you KNOW you can’t see enough to function without help, and this can change from moment to moment.

Even when you HAVE the confidence to move independently it may be misplaced, but you can’t ‘see’ (both meanings intended here), and off you go!! The person guiding you (not wanting to steal your independence) then has a problem because they CAN see and will want to warn: there are/is a …. (fill in the blank) in your path x paces ahead. But since you have a hearing problem too, you are now on a collision course. You have to give them the opportunity to give you that information too. Oh boy, that is really hard, there goes a bit more of your independence!

So it is a real challenge to find a balance in enabling the other to help you and not totally relinquishing independence, all the while not putting yourself in too much danger; because let’s be real, we can’t live totally without risk and still do something!

C: The moving goalposts

Moreover, with a progressive disability, what worked for a while, doesn’t work anymore and you have to invent new ways and keep the people involved with you abreast of the changes. In your twenties and thirties you still have enough energy to do so. As you get older it does get ‘real old’. What keeps you going for the longest time is being in denial, and there is something to say for it. It may become dangerous, but it also keeps you going and getting on with things. Without that stage you would give up very quickly. Of course it has the disadvantage that you are not really aware of the severity of your loss and cannot inform others of the same. They will often have a better idea than you have of how bad the situation is, albeit incomplete, but are stymied in their options to help, as you are a free agent, sometimes dangerously so!

Then you get to the stage of part-admission: ‘as long as it doesn’t get any worse I can live with this’ and of course it DOES get worse and you say the same thing about the next stage and the next. But there comes a day when these coping mechanisms no longer work, hopefully BEFORE a serious accident forces you to admit it. Then you are faced with the stark reality, THE GOALPOSTS KEEP MOVING and as you get older it seems to happen faster and faster. You face loss after loss; loss of hobbies, loss of employment, loss of being able to do some of the simplest things, sometimes loss of a partner who doesn’t want to deal with it all.

Loss requires a grieving process; Elisabeth Kübler-Ross describes the 5 stages in that process:

1/ Denial – 2/ Anger – 3/ Bargaining – 4/ Depression – 5/ Acceptance

Now a grieving process takes time; in the case of Usher Syndrome, too much time. By the time you have gone through it and you can say; well I can live with this, it’s too late!! It has deteriorated further and the adjustment and grieving processes start all over again. So the 5 stages of grieving become a constant companion, all of them at once because you grieve different stages of loss at one time. So you can be in denial on one level, angry about another, depressed about another level of loss etc, whatever you have accepted has long been and gone, so you are confused on top of it all.

The problem is: you can’t deal with the grieving process of loss ahead of the loss to come, and get it all done and over with. It does not work that way. Also, when other parts of the body start to deteriorate too as happens with ageing, (in my case it began to happen at age 24!!) life becomes an incessant battle of trying to overcome/compensate and trying to live with it. You can‘t adjust to ‘moving goalposts’. That’s an oxymoron. You adjust to changes, constant changes demand constant adjustments. As stated before, your brain compensates so well that you are actually ‘behind’ the real level of loss, (causing both confusion and real danger), and so your constant adjustment is ‘behind’. Even when you ‘know’ this, the problem remains. You can’t say: ’let’s see what I can really see’. You can’t switch your brain off; not for any length of time and still DO anything.

The whole process feels like a robbery and a betrayal. Your body/eyes are betraying you all the time; your brain is betraying you all the time. The robbery and betrayal can only build resentment and/or self-pity. So there is the battle against that as well.

And then of course, the BIG ISSUE is, feeling useless: WHY AM I HERE, how can I possibly contribute in any way anymore?

Everybody needs meaning in their life; these ongoing losses can rob you of it. From what I have observed, having children/grandchildren seems to give tremendous meaning, as you can be there for them in some way.

Having a supportive family and/or friends can be a help, but it doesn’t necessarily give meaning, you feel you are a burden to everyone instead of being useful in some way. If you have to deal with it largely on your own, as I do, friends and family notwithstanding, it can become an increasing battle to find that meaning in your life.

Ambitions can keep you going, but when the moment arrives (and it will) where you know you no longer can fulfil ‘whatever that ambition was’ you have to deal with that loss too. Hobbies are fun, and very necessary, but they don’t give real meaning to your life. So now, if you don’t believe in anything, life becomes pretty pointless. Sometimes the things you DID believe in, no longer are meaningful.  In truth, it now becomes a battle… for your ‘soul’ (for want of a better word). For those of you who don’t think so, it’s not your body that is satisfied with being a parent, although a functioning body certainly helps; it’s your self /soul that is satisfied with the meaning of parenthood; or for that matter, any service you provide for others. Everybody needs that ‘meaning’, whatever form it may take. So for better or worse, it now becomes a spiritual battle of sorts.

So then…what now?

A few years ago, David Brown gave a lecture; he was talking about his work with deafblind children and what HE had learnt from THEM! He had a list of 10 things. I can’t remember the exact list he used, but when I heard him go through the list of things he had learnt I, for the first time since I was a child, broke down and sobbed and sobbed. Because the things he mentioned were the things the whole world needs to embrace and practice (those closest to me, when I was a child, did not). We are the teaching tools, as well as having to embrace and practice these characteristics ourselves of course, which is really what life, the why-we-are-here, is all about.

The list:
Compassion, understanding, courage, generosity, patience, acceptance, kindness, love, humility, empathy, gratitude, trust, responsibility, determination, joy, walking in another’s shoes, I AM my brother’s Keeper.

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