Sunday, May 27, 2012

Alkeus to start clincal trials for SD later this year or start of 2013

Another pharmaceutical company dedicated to finding a cure for Stargardt's Disease will start their clinical trials later this year or in 2013. Alkeus research is more focused on a preventive treatment, with an aim of developing compounds to  prevent formation of toxic A2E and lipofuscin pigments, and thus to slow down macular degeneration. Currently, they are finding patients for their clinical trials. You can add yourself to their registry to get future updates on trials.

Excerpt of a comprehensive article from their website:

Plant 1
The retina is the thin membrane located at the back of the eye. It contains millions of photoreceptors used for vision, and plays a similar role as the light sensitive film at the back of a camera. These specialized receptors cover the retina and are responsible for black and white (rods) and for colored vision (cones). Vitamin A, which can be found on the tip of the photoreceptors, is the key molecule to vision. Its exact role was explicited by George Wald and resulted in a Nobel Prize of 1967. In other words, vitamin A is the fuel of vision.
Vitamin A and its derivatives (such as beta-carotene), originate from certain types of food: for example, eggs, milk and other dairy products all contain vitamin A while vegetables, fruits, carrots, etc. contain beta-carotene. After vitamin A is transported into the retina, it is struck by light forcing it to change its molecular shape, and acting like an electrical switch which enables the delivery of an electric signal to the brain. This signals the presence of light to the brain.
After vitamin A has changed its shape, it becomes insensitive to light and needs to be reactivated by specialized cells, the retinal pigmented epithelium (RPE). The geometric switch of vitamin A as well as its recycling by the RPE is called the "visual cycle".
While we would wish vitamin A to be a clean burning fuel, unfortunately, the visual cycle is imperfect and some vitamin A molecules are able to escape the recycling system: these vitamin A can then bind to other vitamin A molecules and create toxic aggregates of vitamin A called vitamin A dimers (or A2E). A2E is then absorbed and stored in the RPE cells where they are considered to be responsible for the formation of other toxic granules named lipofuscin.
With age, accumulation of lipofuscin reduces the proper function of the RPE cells and is thought to be partly responsible for inducing macular degeneration.
Stargardt disease patients present a defective gene which prevents proper transport of vitamin A back into the RPE, which results in even faster formation and accumulation of A2E and lipofuscin. This is why Stargardt disease is also called "juvenile macular degeneration" as the clinical presentations may be somehow similar.
Visual cycle imperfections lead to A2E formation and to macular degeneration. Alkeus Pharmaceuticals is developing compounds that can help perfect this cycle, prevent the formation of toxic A2E and lipofuscin pigments and potentially slow down vision loss in dry-AMD and Stargardt disease."

Increasing subtitle font size to watch foreign films

Stargardt's disease has limited my access to all possible forms of visual arts. With more loss in central vision, my depth and colour perception has further decreased. I no longer visit art galleries and museums. I hesitate to take out my camera for street photography. Cinema and theatre are still accessible from the front row, but only in the languages I know. Few weeks back, an Afghan troupe performed Shakespeare at Rangashankara, a local theatre. I was in dilemma for a week whether to buy tickets or not.  (Will I be able to read subtitles? or even see subtitle panels? :P)

I don't have problems with subtitles while watching movies at home. There is a feature in VLC media player which allows to enlarge the subtitle font size:

1. Go to Menu Tools > Preferences

2. Select Subtitles & OSD

3. In Subtitle effects, set font size to large or larger and Save.

At larger font size, the screen appears like this. You can download VLC media player from this link

Tuesday, May 22, 2012

Moving to a new city

Sometimes disability is imposed. Like, there are accessible cities and there are inaccessible cities. Experience is relative still. 

Ever since I arrived in this city 4 years back, I've been uncomfortable. Running in circles and always finding myself back at the starting line. Several change of jobs and relationships. Permanence is perhaps a utopia.

I feel no connection with this culturally inaccessible city. It limits me. It disturbs me. It took me sometime to understand that I need to move to a new place. More than 2 years. And then when I thought I need to move, I found someone. It didn't last long. I live my life from one minute to another (Either it is SD or just me, difficult to disassociate) and this person came with a schedule where I had to fit in or where I couldn't fit in. I'm the kind of person who will skip the world to be with someone for even 10 minutes and he was the kind of person who was too entangled in several worlds. Yet there was an inexplicable connection between us and now we are unrelated. 

I'm free to move to a different city. A city that expands my possibilities. It did take me a while to work things out and finally I got relocation approved from my employer. 

Being visually impaired, I like being in big cities, where people may not be monitoring your every move. I can get lost without looking stupid. I can look up and someone will guide me home. (And not be embarassed to ask 20 people for directions, within just a mile!. With more than 20,000 people per square kilometer to ask for help, I do not need a map). I can walk in crowded streets incognito. Be led by the pace of the city and find several things to do. And when I get tired of the world, find a pretty spot to renew my morale and spirit. I can depend on the city. 

Even though it will take a month or two to actually move, I'm already excited and feel less disabled :o)

Sunday, May 20, 2012

Reading problems

Finished reading La carte et le mémoire  (Michel Houellebecq) and Cosmicomics (Italo Calvino) - around 600 pages over 3 weeks, in office shuttle, cafés. Had sore eyes as a result. This month was exceptionally hectic even at work with a huge volume of translations and tight deadlines.

I find I move between reading and non-reading phases all the time. As continuous reading always results in sore eyes and/or discomfort, i really avoid reading. And then when it has been too long (say a month or two), I pick up again a book and try to finish it.

I'm still not used to text-to-speech. It sounds too odd and kills any pleasure whatsoever of reading. Audio books are alright, but the experience is completely different. Perhaps reading is irreplaceable. Text-to-speech fails with fiction and complex non-fiction books.

As a possible solution, I'm thinking of buying a compatible refreshable braille display. My reading speed is better with braille and it would not involve any reading stress. But braille displays are very expensive currently, between $2500 - $8000. Several projects for developing an affordable refreshable Braille display are going on. Once in the market, the retail price will be around $300 for these displays (For e.g., Quixote, by Bristol Braille). These new displays are expected to be available by the end of 2012. I will wait for few months.

Do you use a refreshable Braille display for reading? Any suggestions?

Tuesday, May 15, 2012

Interpreting disappointments

Stargardt's has defined my choices in life. It has also defined other people's choices about me.  Every day I face a  prejudiced world, a world that only understands "normal", that often tries to weigh me down with its distorted perspectives and its coloured opinions.

Most of the days I can easily shrug them off, but sometimes they settle deep. Some I manage to remove over time, but some just linger like so many other things in life.

I could have finished my Ph.D in French literature some years back, had the doctors not told me to stop to read.

I would have not lost a relstionship, had they not disabled me in their imagination. We were not left any choice. I've still not been able to deal with that empty space.


There are other things that lie beneath. Things that we think we are comfortable with. But we forget the dimension of time. We forget that everything evolves, even the past.

Some people say they have become comfortable with Stargardt's. Am I comfortable with Stargardt's? I don't know. I'm not even sure what is the right answer. Acceptannce and being comfortable are two different things.

Discomfort can be positive. Like an exercise in reverse. It can thrust you forward to force you find a way. If people act as mirrors and if we are constantly trying to adjust our reflections to their mirrors, perhaps I constantly adjust my life to not be that reflection. Because their reflections are all "normal". And I'm a different person.

Thursday, May 3, 2012

All about Pens

I used to like sending postcards to my friends. Now it is almost impossible to decipher what I write. There was a time when writing was the only way to self-discovery.

These days I try to avoid writing. I take my laptop for meetings instead of a paper and a pen. I take notes on my phone or PC. Yet there are situations when the use of a pen really cannot be avoided. Then, I find I must avoid cursive writing if I want to read it. 

Recently, a fourth grade student designed a pen  for visually impaired children to better recognize shapes and letters. And just out of a small spool of yarn, paper clip and a paper cup attached to a hollowed out pen! 

I thought I'll do a post on two pen systems for low vision which might be useful for different people depending on their needs.

PenFriend - RNIB's voice labeling system, PenFriend,  has been there since some time now and still very useful for both low vision and the blind. You can record your voice onto dime sized self-adhesive labels that you can stick on any household objects. And then touch it again with the pen and listen to the label/note. Unlike the demo video, I can still distinguish between a can of tomatoes and a can of beans. I really liked the idea about voice labels on medicines. Especially I find it difficult to read the expiry dates and sometimes I don't know what I'm popping has long expired. It can be useful for prescriptions and labeling important documents and posts which are unreadable.  These stickers can also be used as tiny reminders or notes that I can stick on pretty much everything and anywhere.It comes with a 70 hour of recording time and you can also download mp3 and audiobooks on it from your PC. PenFriend is available over Amazon for $125 and comes with 125 labels. Extra labels would need to be purchased. A pack of 381 labels is for $30.

Livescribe Echo SmartPen - This is more of a recorder pen that lets you record everything you hear,say or write. Add voice notes to handwritten notes and convert these handwritten notes to digital by using an app. Just like the SmartPen labels, Livescribe also requires a Livescribe paper which has a pattern of dots that enables to recognize and record pen writing movements. A very useful device for those who do not use or carry around their iPad or iPhone, in a classroom or in a meeting or for maintaining an agenda or a diary. It is priced at $134 for a 4GB Echo SmartPen. Livescribe claims that the special compatible paper can be printed but most reviewers seem to contradict this point. Lot of people have also complained that the handwriting recognition app MyScript doesn't recognize correctly even the clearest of handwriting. And if you need to record or write a lot, the cost of paper will soon add up to be more than the cost of a smartphone or an accessible all purpose tablet.