I spent a lot of time trying to figure out how to write this post. The
challenges I have faced, whilst not huge when compared to the challenges
faced by those suffering a more terminal condition at times felt
insurmountable to me. They are also so numerous this will not be the only
post on the subject. In attempting to avoid a dark and hopeless tone to
this post I have tried to add all the ways I have found to adapt to each
challenge. I am still learning, I have made many mistakes whilst
attempting to come to terms with my condition.
I have never been a fan of night clubs which is fortunate as every time I
have set foot inside one I have been as good as blind, the darkness with
intermittent flashes of light do just enough to prevent my eyes
compensating even in a small way. Add this to the gaps on my peripheral
vision and a large number of people in a relatively small space and its a
recipe for disaster. things like being able to see what money I have in my
wallet, finding my friends and not spilling my drink everywhere took a
long time to find adaptative strategies for even in bars and pubs. I have
grown up with a close circle of friends all of whom know my condition and
are aware I have significant limitations when out and about at night, They
keep and eye on me and several of them knew how to alert to me a situation
without making me feel embarrassed. Lamp posts and bollards caused me a
lot of grief, walking into these wasn't just painful it was embarrassing,
have a friend with a keen eye to alert you when he feels you haven't seen
an obstacle is extremely useful. I carry a torch at all times however
walking down Southamptons high street late on a Friday night with a torch
can result in some undesired attention.
I always prefer to arrive at a bar with friends or before them. If I am
there first then they have to find me, it makes things far less stressful
just being a few minutes early and you don't get a phone call from a
friend after youve been walking around the pub for twenty minutes saying
"you've just walked past us twice". In regards to know what money is in my
wallet I have found coins are easy enough to feel and identify(however
every now and then some foreign currency will find its way into my wallet
which will confused me. Any coppers I received I removed from the main
part of my wallet and saved at home, this kept the coin part of my wallet
light enough for me to be able to find a correct value fairly quickly.
Notes proved a problem, some pubs and bars have just enough lighting to
allow me to make out what note I had, however there are many that don't. I
have gotten into the habit of folding my five pound notes horizontally, my
tens vertically and my twenties diagonally, this allows me to feel and
identify. when i collect a new note at the bar I tend to stuff it all in
my pocket and work on identification and folding discretely at the table.
There are several bars I frequent which make it extremely difficult for me
to find my way too and from the bar without knocking over several people.
I usually as a friend to come with me to that bar, I find it much easier
to let someone go ahead of me and I can follow their back, very much like
walking through a minefield. At the bar I pay it being my round and
usually ask my friend to wait at the bar for the drinks whilst I use the
toilet (the bars in question have toilets at the end of the bar). Toilets
I have found are almost universally well lit. I step back out, grab a
couple of the drinks and follow my friends back, back to the table.
This largely seems like babysitting not friendship and I resisted it for a
long time, again feeling embarrassed. However many of my friends are
insistent on helping, seem genuinely not to mind and I make sure I always
express my deep appreciation. A large part of trying to have a normal
social life with this condition seems to be knowing who to trust.
I tend to have a problem with bouncers a lot, my pupils don't dilate quite
normally, it could be my first bar of the night, I could be stone cold
sober however a bouncer seeing me approach after tripping over the
pavement and then seeing my well dilated eyes....well its perfectly
understandable he thinks I am drunk and refuses me entry. This largely
goes away when I talk. If I was drunk enough to have fully dilated pupils
and severe balances problems I shouldn't be articulating like I am sober.
Generally explaining that I have an eye problem in conjunction with clear
speech causes the bouncer to reconsider and let me in. Its a shame there
isn't some sort of medical id system for bar entry.
The final trial at the end of a night out(at which point I could be drunk
or sober, alcohol isn't always required on my nights out) is getting off
the bus at the right stop, I am entirely unable it seems to see through
bus windows when it is dark outside and the buses interior lights are on.
Asking a bus drink to notify you of the stop is largely unreliable,
through no fault of their own(its simply not their job). Fortunately my
phone has a pretty sound GPS system on it that allows me to ensure I am at
very least on the right road for my stop. Even if I do get off a stop or
two early once I am back in my village there are fewer security issues
with me using my torch.
As far as final words of advice go - Don't drink excessively on nights
out, you are vulnerable and getting absolutely wasted will only limit you
eyesight further. Remember to be security conscious, like most when I am
in the dark my hearing is exceptional, don't walk around with headphones
in or anything like that, you already have once sense down don't make it
two. More than a few times I have not seen someone, walked into them and
spilled their drink, they often assume I am drunk and say things like
"watch yourself mate", just shrug it off, offer and apology and a
replacement drink, don't dwell on it.
I am sure I shall be writing a second part to this post however I feel
this has probably covered the basic problems I encounter, I am not sure it
covers much in the way of solutions.
Thursday, 6 December 2012
The best place to start would probably be explaining and describing my exact condition.
Retinitis Pigmentosa(RP) is a group of genetic degenerative conditions of the eye. The present in much the same way as macular degeneration because ultimately that what they are. The key to defining RP is the mechanism.
Everybody who sat GCSE science is familiar with the image of the double helix. The spiral ladder structure that represents our DNA. That structure contains all the information required to build you. It is effectively your blueprint. A huge proportion of the data held in your DNA is "junk" it doesn't code for any specific genes as far as we know, in some cases it's just redundant coding left from our evolutionary past. The rungs on the ladder are bonds that join two objects called nucleotides. In humans there are roughly three billion of these rungs joining six million nucleotides.
Every time two people combine their DNA and make a child a number of coding errors occur. These are called mutations and they come in two forms. Deletions are where a nucleotide doesn't get copied leaving a gap. Or substations where a nucleotide is out in the wrong place. Mutations are normal and happen every generation most of the time they occur in areas of our DNA that don't code for anything and there are no physical symptoms as a result.
Here's the bit that blows my mind. At some point back in my family tree a deletion mutation occurred in one of the few areas of my DNA that codes for something. Of six billion nucleotides I am missing one. As a result my body is unable to produce sufficient quantities of an enzyme that would normally protect the retina of my eye. This condition is "X-Linked" which means I carry it in my X Chromosome. As I only have one X Chromosome(the other is a Y chromosome, XY is male, XX is female) all the data that codes me from that chromosome is used regardless of weather or not there are mutations. In women there is a certain degree of error checking, when their cells are dividing they identify that there is a mutation on the broken X chromosome so they use their other X chromosome. As a result women carry my variation of RP but don't develop any symptoms of it. If they have a child their is a fifty per cent chance they will pass on the mutation as each parent only passes one chromosome to their offspring. If they do pass on the mutated X and the child is a girl she will be a carrier, if the child is a boy he will be symptomatic with RP.
That's probably enough of the genetics you want to know what Retinitis Pigmentosa actually is. The retina of the eye is a disc covered with two types of photo sensitive cell. Light gets passed onto this disc through your eye and these cells act as your visual field, some control colour and fine detailed central vision, others control monochromatic night vision and peripheral vision. Due to the lack of the enzyme I mentioned previously my retina are being constantly damaged, the more vulnerable areas go first, the areas that effect my night vision and peripheral fields. As a result I have next to no vision in dim light and there are substantial gaps on my peripheral visual fields, and now one or two gaps in my central field.
The human brain is an incredible thing, I am largely unaware of exactly where these gaps are because my brain fills them in with what it thinks should be there. If I am looking at a floor and there is a wet floor sign in my visual field gap my brain fills in the gap with empty floor because that's what's on every side of the gap, as a result I won't see the luminous yellow wet floor sign. The same principle applies to bollards, chairs and almost anything else at a worrying height to walk into.
I have also started noticing a steady deterioration in my colour vision. Add all this together and you can see why I had to leave clinical practice as a nurse. I think my medical background has helped me though. I genuinely find this condition fascinating. For example. Every time the parents procreate there is only a fifty per cent chance the mutation will carry(assuming only one parent has it) and I have traced progressive blindness back in my family tree to the mid 1600s. It probably goes back further but records get a little more ambiguous past that point. Every generation, every man and woman in that long chain leading up to me has passed on the mutation.
I am sorry I could not relate the above information without going into so much scientific detail I know that will limit the audience who are interested in reading this considerably however I felt it was important when explaining what RP is to start with the foundations. I did simplify a large amount of the above information however I do not think that it has compromised the accuracy of the information.
Should you desire more information I can recommend these sites. I will add to this list as I find sites I feel appropriate, should you know of any please feel free to comment.
Wednesday, 5 December 2012
When I was eleven I noticed significent visual disturbances, I was already short sighted however my optician could not account for a blurring visual field. He referred me to an opthalmologist. My mother was concerned and unsure how else to react she insisted I see a private opthamologist, whilst this is a normal motherly powerless reaction, as an adult I now know that consultants you see in private hospitals have day jobs in NHS hospitals. I was promptly diagnosed with "probable retinitis pigmentosa" at that time there was no measurable way to be absolutely sure, the human genome hadnt been cracked and my retina hadnt deteriorated to the point where it was easily obvious using an opthalmoscope.Years later, with a confirmed diagnosis I became a nurse. A foolish choice you may think. My eyes are going to deteriorate into blindness or something so close it wont make a difference. I spent two years working general wards and a high dependency unit. I have to admit working on HDU despite the fact it was little over a year was a high point for me, years of training and experience brought to a point where i could function autonomously and I feel I earned a great deal of respect from the patients, doctors and nurses I worked with.However this is where my story finds me now. I recently made the move from HDU to an NHS led phone triage service(which i can not name for legal and confidentiality reasons, there are a number of these services around). With the noticable deterioration in my eyesight, despite occupational health suggesting I was still safe to practice I know it was only a matter of time before I made a mistake, and in all likelihood in that environment it would be a big one.I do not intend this blog to be a self mourning last will and testiment. I want to document here some of the things I have and will struggle with, ways I have found to adapt and any other information I have which could be used to help others in a similar situation to me. Weather this will attract any readership at all I have no idea however I feel its important for the imformation to be out there. I will occasionally slip into some science, that is something I cant help, I am a trained critical care nurse and as such I have a very scientific approach to presenting information and quite frankly my condition facinates me.So let us begin.....