Monday, December 1, 2014

Vitamin A and Stargardt's - Part 1

The only advice I was given when diagnosed with SD was that vitamin A was causing my vision loss and to not take supplements with Vitamin A. This totally contradicts the widely known fact that Vitamin A is essential for eyes. It's not that this fact is incorrect, it's that it doesn't wholly apply to SD. 

Since I love my science and research, I took it upon myself to spend my spare time reading every published article on SD and vitamin A to find out more about the mechanisms at play in the eye causing problems and what evidence exists to support these theories. The earliest article I found was published in 2008 and all used mouse models for investigations.

A paper published in Cell Death and Disease this year gives a good overview of the mechanism leading to vision loss and how Vitamin A is implicated. Essentially, SD is caused by an accumulation in the RPE of Vitamin A dimers (dimers are two molecules joined chemically), which are known as A2E (shortened version of the chemical name). Using an in vivo model, they found that increased A2E led to greater cell death. A word that always comes up when talking about SD is lipofuscin which is the yellow-brownish pigment deposits seen on a retinal scan used to diagnose the condition. Tying it all together, A2E derived from vitamin A is a retinal lipofuscin which is causing the damage and subsequent vision loss. These findings support earlier studies in mice such as articles n the Journal of Biological Chemistry and Investigative Ophthalmology and Visual Science which both looked at the effects of vitamin A, SD and mice demonstrating that higher doses of vitamin A led to increased lipofuscin development. 

Another paper in the Journal of Biological Chemistry also used a mouse model to investigate the effects of A2E and  also oxidative stress. It was found that A2E increased inflammation in the RPE, which is an important finding for prospective treatment solutions.
There are no studies performed in humans on the effects of vitamin A probably because it is not exactly ethical to get a group of SD patients and give them high doses of vitamin A and see whether they deteriorate! Despite this, the research is available to support that vitamin A is indeed causing damage in the eye and leading to vision loss. The continuing research has led to greater understanding on the different components at play and, no doubt, will help in the future to develop therapies. 

So now we know vitamin A is not our best friend, where do we go from here? Part 2 will discuss diet and vitamin A and how we can ensure not to overload vitamin A and contribute to the SD process.

Wednesday, November 19, 2014

Second chance for Mr Cane

I have been walking a lot more since unwillingly giving up driving. I have grown in confidence with walking alone but I still find road crossing difficult. I'd given the cane the flick because we just weren't getting along. I haven't used it in some months now although there has been a little voice in the back of my mind telling me to pull it out again and give it another go. 

Yesterday was the big day when Mr. Cane got his second chance to win me over. I must admit, he did a damn good job at it! I went for a walk with a friend who hadn't encountered anyone using a cane before so it was interesting for her to see how it was used and the reactions of others. 

One of the big issues I've been having is that when I'm walking, others have no idea I'm vision impaired and if I make a mistake crossing they will think I'm an idiot. I like the fact the cane is a way of saying "Hey, I don't see good, look out for me". This was evident when we crossed a main road, without traffic lights, but with an island in the middle (so we could go half the road at a time). This particular spot had been an issue for me because the road is so busy I can't simply rely on my hearing and limited sight. In a way the cane was like my indicator signal of a car. People knew to be mindful of me. We had a great experience, each time we crossed (we did it a few times!), when we were on the island, a person would stop the traffic to let us cross. It really was a testament to how kind people can be. These small gestures increased my confidence with crossing roads and also gave me more trust in those around me. 

We also noticed people's reactions when walking toward us. Each person we passed, even someone with a pram, moved off the footpath out of the way to let me pass. I don't know what I expected, that people would walk into me or make comments, but experiencing it first hand made me feel more at ease with the whole idea of using the cane and reduced the stigma to using it.

Mr Cane and I have gone on another date today, with similar success, and we are on the road to a happy and long partnership. Up until this point, I wasn't ready to accept the fact I may need extra help getting around and there is nothing wrong with that. Now is the right time for me and I will embrace it and continue to gain more confidence and independence. 

Monday, October 27, 2014

Update on Stem Cell Treatment

If you haven't already heard, results from the phase I trial using human embryonic stem cells for patients with Stargardt's Disease and Macular Degeneration have been published in the Lancet, and the results are very promising!

Nine patients were treated from each disease which involved surgical implantation of the stem cells into the retina of one affected eye. The main aim of phase I trials is to test the safety of the intervention, so whether there were an adverse events or complications from having the stem cells implanted, and in this case whether the stem cells 'stuck' to the eye. The investigators also looked at whether there was any improvement in vision.

Excitingly, the results were very promising - there was no major adverse events (although some patients with Stargardt's developed cataracts but this was treated surgically and didn't affect vision after treatment), no rejection of the implanted cells, no abnormal tumour growth and it looked as though the cells remained functioning at follow-up (which was up to two years after initial implantation). To top it off, there was also objectively reported improvement in vision in the treated eye!

To put it in perspective, this is still early days and we won't be running out to get stem cells injected tomorrow. Although it is definitely looking like a very possible treatment in the future and in our lifetime! The next step is to proceed to a phase II trial which will include a larger number of patients and find the best dose (number of cells to be injected) to gain the best results. Further evidence of the effectiveness will be developed to show how much vision can be restored, if it is permanent, whether the implantation needs to be done in the early stages of the disease and many other questions.

This is extremely exciting and shows how amazing modern medical science can be. It can give us all a little more hope that potentially in the future we may be able to see better, and if not us, the generations to come who will unluckily have to experience the hurdles we have faced. 

For more information here are the links to the results and news reports:

Wall Street Journal

The Lancet

Wednesday, September 3, 2014

Selling My Independence

This week was a big milestone in my journey of acceptance. I am actually quite surprised that I did it and finally let go of something I had been holding on to so tightly.

Probably slightly melodramatic, but I sold my independence this week, my beloved car. I had bought my car brand new almost five years ago to the day. After driving around in hand me down cars (including a red, seven-seater Mercedes Benz station wagon from before I was born), I had finally splurged on a shiny, fresh new car. I was very attached to my car, it had travelled with me through so many life events and it was like my solace where I could feel calm and even have a cry. Ok it does sound like I was in a relationship with my car but since I'd driven for 10 years I had really gotten used to having it.

I should have stopped driving a few years ago although I didn't stop (and I in no way recommend doing this). I continued to drive locally to and from work. As my sight deteriorated I realised I had to let go, I had to give it up. In March this year I made the decision that I was going to let go, I wasn't going to drive again and I was going to literally start from the bottom and learn to be independent in a new way. This caused me great anxiety and stress and by no means has it been easy or am I close to being entirely independent. I grew to rely on driving, I never caught public transport, so I'm literally learning how to do everything again with poor vision. 

I tried to find a reason to keep my car - someone in my family would drive it, I could give it to my brother, we could have a spare car, but deep down I knew it was pointless. I put it up for sale a month ago, grudgingly. 

As the weeks past I still tried to find reasons to keep it and more people came to look at it. Once I found a buyer, the few days before they picked the car up I started to think to myself I should just keep it and I didn't want to give it up. The morning they came to pick it up, I paced around the yard and my anxiety levels grew. I knew I had to do it and I knew that once I did I would feel stronger.

It was actually easier than I thought (possibly helped my a massive envelope of cash). It was even empowering. I was proud of myself. I had let go. I had let go of something I had been holding onto for many years. I knew I could do it, that I could face this new chapter. 

And here I am. Car-less, less independent perhaps but with a greater level of determination. Of the years I have been battling Stargardt's, this week definitely was a big step for me. Onwards and upwards!

Tuesday, August 12, 2014

Increased Senses

A common question I get asked is whether since losing my vision my other senses have increased. Generally I would answer no, but lately I've noticed that my senses have been changing and adapting. This is probably due to being more aware of what is happening around me and being more accepting of the need to use my other senses more in every day life.

I would not describe myself as having good hearing, quite the opposite especially since I had ear problems as a child which have continued into adulthood. Since learning how to use a cane to navigate, I have had to tune into my hearing more to help me cross roads. I think in the past I just didn't pay attention to what was going on around me on an aural level. The first time I tried to listen to determine whether it was safe to cross I felt overwhelmed and had no idea whether I should wait or go. I was also not accepting that I needed to be using a cane and my ears, and this also played a role in having so much difficulty tapping into the sense.

Another big help to me learning to tune into my other senses is practising mindfulness meditation everyday. I started learning about mindfulness to help with anxiety and I was instantly hooked on it. I was always interested in meditation, using it infrequently, but mindfulness really resonated with me. It teaches you about being in the present moment and to be aware of your body and your surroundings. 

My favourite way to practice mindfulness is to listen to the sounds around me. I was shocked when I first tuned in and heard at least five different birds, all in the middle of a big city! I had totally forgot the existence of the wildlife around me! I also can hear the traffic (which is surprisingly relaxing), the trains which are about a five minute drive away and the bats squabbling constantly. This probably sounds lame and like I have no life but I actually enjoy just listening! I've always been a person constantly on-the-go and always engaged in an activity but I've actually learnt to stop and just be, and it has helped with accepting my diagnosis and also in aiding my adaptation.

It truly is incredible what goes on around us and within us, and tuning into it has been a rewarding experience. I'm learning to embrace just listening, not having to rely on my eyes, to experience the world in a different way. You don't need to see every detail, you can combine what you see, hear, feel and smell and that is just as satisfying as being fully sighted. 

Wednesday, August 6, 2014


Living with an incurable, degenerative disease which leads to permanent disablement and blindness is one of the hardest challenges a person can face. Doubled with the fact that the process usually begins in early adulthood when a person has discovered who they are and figured out their life goals and aspirations can make the situation even more confronting and distressing.

Hope can be a strong and powerful feeling that helps anyone going through this situation, and other situations, get through the day. But is there such thing as 'unhealthy hope'?

My main concern is when hope for a cure dominates everyday thinking and finding a cure is the only way someone can accept their diagnosis or find the will to continue on. 

The one thing for those who have Stargardt's, and any other inherited retinal disease, is that we may be losing our sight, becoming 'disabled' and 'legally blind' but we still have amazing, fulfilling, satisfying and successful lives. Our lives may be different from what we expected them to be, but I guarantee we will look back when we are older and think we wouldn't change a thing.

In saying that it won't be a walk in the park, but in life what ever is? I know myself struggling through another uni degree determined to complete my third degree as a 'legally blind' person and then ultimately completing my PhD will be one of the most satisfying moments in my life. People always ask me how I manage, and to be honest, I don't even know! It is literally pain and tears to get me to where I am and I still have a long way to go. 

I really want everyone to remember that a cure isn't the only answer for us, while it would be fantastic, our lives are still worthwhile and filled with incredible opportunities. 

Always stay positive and remember, no matter what the goal is, you can always get there, you may just have to take a different path to achieve it. 

Friday, July 25, 2014

Audible Book Giveaway

I'm very excited to share that Audible has given free audio books to give away to five readers.

For those of you that haven't tried Audible, it has thousands of audio books, current and past, and you can access them on your phone or tablet through their easy to use app. I have been using Audible for some time now and without it I wouldn't be able to have continued 'reading' the books I love.

All you need to do is become a 'follower' of this blog (right hand side), and comment below with your name, age, condition and a book you'd love to have as an audio book.

You have until midnight Sunday 10 August to enter.

Good luck :)

Wednesday, July 16, 2014

The Finger Reader

Now this is one invention I am super excited about. Since I came across a news article about it a few weeks ago, I've been thinking of all the ways I could use it and how beneficial it would be to the vision impaired.

The FingerReader is a prototype developed by MIT and is a ring which reads printed text. It really is incredible. Personally, I despise using any magnifiers in public, so I struggle to read things or ask for help. My mind has come up with countless uses for the FingerReader:

  • I could actually read the menu for once, instead of making it up!
  • I could actually read the price on tags when shopping, especially clothes, so no excuses for spending too much money!
  • When I go to sign various forms, I could read through the document myself instead of relying on a dodgy person who really just wants my signature!
  • I could tell who the mail is for, instead of opening everyone's mail!
  • And the list goes on....
Personally, I don't find magnifiers useful for the above situations. I find that they are usually large, draw attention and time consuming as it takes time to focus on what I'm trying to read. 

I look forward to the progress of the FIngerReader and really hope it come to fruition in the near future.

Check out the MIT website.

Monday, June 23, 2014

Online Support Groups

I know I have been very slack with my blog and it's time I made up for it! I have been very busy having a love hate relationship with my cane, giving up driving and leaving my job. You could say I'm having a mid life crisis (at 27). 

Something I have been meaning to do for a while is organising online support groups where we can talk in real time no matter where we are in the world. This will happen through Skype and I hope to organise it on a monthly basis. 

I will mediate the groups so we can get the most out of it and we can discuss different topics/experiences at each meeting.

If you would be interested in participating, please send me an email with your name, diagnosis and location to or alternatively comment with the details below but I will need your email to contact you.

Let's get this going and connect to help eahother!

Friday, February 7, 2014

My cane adventures

I've had a cane for over a year now but haven't been brave enough to use it. My vision is further deteriorating and I decided it was time to accept the cane. I have an amazing mobility instructor from Guide Dogs who has been helping me learn how to get around using the cane.

A big issue for me is not feeling like I'm in control stemming from the fact I can't see what's in front of me and what's coming toward me. I know I'm a control freak so not being able to see where I am or what's coming up terrifies me and cause me anxiety. I've also realised than to cross roads I simply hope for the best which is not something I'd advise! 

Working with somebody is so beneficial, it is giving me the confidence to explore and feel comfortable using the cane. I've learnt how to cross a street, safe places to cross and to trust my ears more. I've also learnt how to use the cane for stairs which hopefully will prevent some of the bruises I have from constantly stacking it! Going up stairs isn't to hard but down is definitely a challenge with the cane and more practice is needed.

In my last session I walked down a main road near my house. I felt as though everybody was watching, of course I couldn't see them, but you get the feeling that people are staring. I was also thinking that someone who knows me could see me and I felt uneasy at the thought. Not so much that I'm embarrassed of my condition but I'm still getting my head around it. It's definitely a foreign feeling that's hard to describe unless you have experienced it. In a way the moment was like me coming out and saying 'hey I'm vision impaired'. I also made a comment about the impression I'd make, walking down the street in high heels with my Louis Vuitton handbag.. Definitely a sight to see!

I highly recommend talking to your local vision impairment organisation and getting involved in a mobility program. Guide Dogs NSW/ACT has been the most positive experience I've had on this journey. And most importantly, don't be afraid to accept help!

Saturday, January 18, 2014

The things you don't get told!

Over the past few years I've learnt so much more about what is actually involved in being vision impaired. When I was diagnosed, I was not told much except I will progressively lose my central vision. I was not warned of any of the other aspects I might experience. Here's a list of things I've learnt and what I do to help. Please share any other tips!

1. Eye pain: Typically I experience eye pain after a day at work or a solid studying session (usually after about one hour). I'm assuming it is related to the strain I put on my eyes to try and see everything as best I can! I experience this daily and it gets VERY frustrating.
What helps: Before reaching for my Panadols (paracentamol) I first try and rest my eyes. Since I always have to be doing something I will either close my eyes and meditate or have a nap. Obviously I can't just lay on the floor for a nap at work (I have tried!) so in worst case scenarios I go for pain killers. I also have found an eye drop that has helped with the burning and heat (Systane Ultra) and you can use this as much as needed. I usually use this after being on the computer. 

2. Headaches/Migraines: This is something I've struggled with for a few years. Generally I get a migraine on my left side which is the weaker eye. Migraines can be extremely debilitating as not only is the pain intense it can cause nausea and further sensitivity to light. I've learnt my triggers for an attack, including sun glare and overworked eyes, so I try to intervene as soon as I feel it starting to take over. Sometimes the attacks are so severe I have to take time off work. At the moment I get about one a month which has reduced. 
What helps: If I am having an attack, I sit (or sleep!) in a dark room and usually wrap a scarf around my head (the pressure helps the pain). Generally I don't find over the counter pain killers effective and I avoid taking anything stronger. My migraines have improved since changing other medications I was on so it's best to talk to your doctor too. Someone also told me to put a drop of lavender oil on each temple and I find this relaxing. To prevent an attack, I'll avoid spending too much time outdoors on a day with high glare and always wear sunglasses outside, which is a given for Stargardt's. And drink loads of water!

3. Sensitivity to light: Some days are ridiculously hard to be outdoors and even inside with the curtains open. Not only does it lead to headaches and migraines, I find it harder to focus and see. I'm guessing this is because the peripheral vision uses light perception to form vision, and since that is what I rely on to see, I am extra sensitive to it. 
What helps: If outdoors always sunglasses! Otherwise unfortunately it's a day where instead of being outside I put on a movie and stay inside!

4. Neck/back pain: I'm surprised my neck is still attached to my body after all the bending it does to read! I'm constantly leaning closer to the computer screen, iPad or whatever is I want to see! This is another constant pain. 
What helps: Making sure that the desk you are working off is set up well, with an arm for the screen. Also any adaptive technology to make the font bigger to eliminate the need to lean. I've also found that getting a remedial massage once a month to be a great help (and I enjoy massages). Massage also helps with the migraines/headaches. 

5. Anxiety/Depression: This is a HUGE part of diagnosis that is not addressed. It is understandable that when you are told that you are losing your vision, feelings of anxiety and/or depression develop. It is a hard thing to deal with. The most important thing to remember is you are not alone in this!
What helps: Talking about how you feel and what you are going through can help a huge deal, if it means talking to friends or family or speaking to a therapist. There's lots of strategies to deal with anxiety and depression and it's not something to be ashamed of. I for one battle anxiety a great deal. As I've mentioned in a previous post the grieving process ties in with these feelings. Also try mindfulness meditation, I have found it really helpful and it's also a time where I can rest my eyes. 

Our experiences are similar but also unique. The best thing we can do is talk about them and help one another. Sometimes simply knowing somebody else is experiencing the same pain or emotions can make you feel a little better. 

Friday, January 10, 2014

It's the little things

One thing I have come to realise is that the littlest tasks can cause me frustration. As my eyesight has deteriorated I've noticed that tasks I normally wouldn't think about while doing are becoming more difficult. At first I get surprised that I'm having difficulty, then determined to do it, then just plain frustrated. The world really was not created with vision impaired and blind people in mind. 

I now find it difficult to decipher between coins when paying for things. Here in Australia, our 1 dollar and 2 dollar coins are gold (the 1 dollar is larger for some reason) and the rest silver. I can't tell the difference between the two gold coins even though they are different sizes unless I have the two in my fingers. Same with the silvers, I can't tell the difference between a 5 cent and 10 cent coins. Luckily I can still differentiated between the colours of the notes. 

Another thing I have difficulties with is using the ATM. I find myself with my nose almost touching the screen or withdrawing a surprise amount of cash. Or even better still, I press the wrong button and it cancels my transaction and I need to start again. I've taken to using the same ATM machines because they are predictable, unlike most of life, and I have learnt which buttons I need to press.

And then there is my number 1 addiction made harder - using my credit card to shop online. What smart person thought it would be clever to put the numbers so they are almost invisible even to well sighted people?! By the time I've correctly entered my card number I've usually given up, which is probably a good thing for my addiction. 

As much as I don't want to rely on others, I need to accept that I may well need to and that it is ok to do so. I could always carry around my magnifier and whip it out when I need it although I think I'd prefer to ask for help! It's intriguing how the littlest things can cause so much continual frustration. The sooner I realise that it's not worth wasting energy on, I can find a way to improve life. Giant credit cards anybody?!