macular degeneration, macular, diagnosis photoreceptors – My Macular Degeneration Journey/Journal

Eye Poop

Today was a “get your ‘stuff’ together” day. Every once in a while it gets so that if I don’t stop and take care of the business at hand, I will be having a screaming fit.

Like last evening for example! However, I have now gotten a haircut, gone grocery shopping and cleaned the living room, as well as having completed a few other tasks, like taking the recycling. Feeling a little more in control – I probably should not say that too loudly – and ready to tackle a page Lin suggested.

That page will be on – drum roll, please – eye poop! Okay, so that is not what they are really called. Most of the world call them drusen.

In a Harvard Health Publishing article that asks the questions: what are drusen and why do I have them, the author describes drusen as “deposits of extracellular waste”. You got it, eye poop.

In younger people, the sanitation department in eyes generally takes care of the eye poop. That ‘sanitation department’ is the retinal pigment epithelial cells aka RPEs. Yes, your RPEs are supposed to ingest eye poop. But you know what? Your RPEs are into recycling, too! They are discovering your eye is its own, little ecosystem. James Hurley at the University of Washington at Seattle and his team have discovered RPEs and the retinal cells are in this close relationship in which the wastes and byproducts of metabolism in one type of cell are the food another type of cell needs. Mess up in one part of the system and everything goes to Hades.

Why would the RPEs stop doing their recycling thing? No answers, just theories but one thing is for sure, age has something to do with it. Most people over 60 have at least a few piles of eye poop hanging around.

You know how it goes. Things don’t seem to work as well when we are older. Some messes pile up.

http://patient.info/health/age-related-macular-degeneration-leaflet

Eye poop becomes a problem when it starts wiggling in between the RPEs and the Bruch’s membrane. Bruch’s membrane is where the RPEs get the nutrients they need to feed the photoreceptors.

Think of it as a huge landslide standing between you and the grocery store. If you cannot get to the store, you go hungry and may die. If you die, those you are responsible for die too. Same with RPEs and photoreceptors.

Again, no one is exactly sure why some people get away with just a few, stray piles of eye poop and others have dozens. There is an underlying error or errors that have yet to be proven. The researchers are working on it.

The Harvard paper points out drusen aka eye poop do not cause AMD. They are just manifestations of the disease process.

Hope that was a help. Hope you understand things a little more thoroughly. Night!

Written March 18th, 2018


Next: Another Cautionary Tale

Home

Progress Daily

I really cannot win with this transportation business. Now they are on a string of late pick-ups and I am waltzing in 45 minutes after everyone else. Grrrrrrrrrr!

Oh well, can’t fix it. Time to start a page…..and as soon as I am into it, they will show up. Same concept as going to the bathroom in the restaurant to ‘make’ your meal arrive faster.  Aha! Recognition! You have done that, too!

PRELUDE, the study, is NCT02659098. I checked and this is the same study I put my name in for last year. I just shot off a message to my research contact and asked her to make sure my ‘registration’ is still good. I am nothing if I am not persistent. Sad to say it is one of my better traits (oh no!)

There are actually two, main measurable outcomes they are interested in. There are the efficacy of the delivery system and best corrected acuity after administration of the stem cells. In the clinicaltrials.gov post they refer to the stem cells as CNTO 2476. In other literature they named the stem cells Palucorcel.  I guess it is better than George (with apologies to the royal family. I have never liked the name George, although the little guy is a cutie!) Of course, Palucorcel does not exactly fall trippingly off the tongue.

Anyway, according to a one page write-up by Jessica Lynch, previous attempts to circumvent the vitreous and go in subretinally caused too many problems. They are, as I had been led to believe previously, trying to go around to the macula using the suprachoroidal space as their passage. (Anyone ever see Fantastic Voyage? I keep thinking how incredible it would be to jump in my microscopic submarine and motor through the suprachoroidal space!) After preclinical trials with mini pigs were successful, they launched into prime time with a phase 1 trial with people. As I said, they are now recruiting for phase 2. [Sue wrote about subretinal and suprachoroidal are in the previous page: Secret Passages in the Eyeball

Looking at the additional data on clinical trials.gov I discovered there are secondary outcomes for the study. They will be looking at quality of life and reading speed as well as whether the stem cell transplants slow or even stop the growth of the geographic atrophy. They are also looking at how many people convert to wet AMD. It sounds as if this study would be a long term commitment for the ‘lab rats’ chosen.

Going back to the Medscape article about phase 1, I discovered they had pretty good success threading through the space and the transplanted cells grew and started to function.

Cell placement was important. They used the microperimetry to figure out what retinal areas the subjects were using for eccentric viewing. Too close and that could be messed up. Cell placement other places was better.

Results? The subjects had some improvement in vision. That was SOME. Before you get too excited, remember this is RPE replacement. RPEs do not see. They support your photoreceptors. Some of the photoreceptors that are at death’s door may come back but the dead ones stay dead.

I did run off the journal write-up on phase 1 and I promise to tackle it and see if there were any other cool findings. Later. Right now I have laundry to sort. Maybe listen to an NCIS episode. It is now playing all the way through on my tablet!  What can I say? It really is the little things.

Progress daily, guys. Progress daily. We will get there.

written October 17th, 2017 Continue reading “Progress Daily”

Eye Poop Reduction

Looking for information on therapies that slow the vision cycle. Got all sorts of things on slowing traffic for safer cycling.

While I am all for fewer car/bicycle accidents, that is not exactly what I had in mind!

Back to Wikipedia, I discovered the visual cycle is the process through which light is transformed into electrical signals. If you have a penchant for chemistry, I refer you to the Wikipedia article. Social scientist here! As far as I am concerned…then there is magic!

Part of this magic includes having three different types of cone cells that respond to three, different wavelengths of light. By taking stock of the strength and blending of the stimuli from these three, types of cone cells, we are able to see color! Cool! Although my guess would have been the primary colors, the colors they detect are actually red, blue and green. Why? Dunno. Magic.

But back on track, visual cycle…

Since the job of RPEs is not only to feed the photoreceptors but also to clean up after them, RPEs have to be able to tolerate a lot of…uh, poop. (Gee, maybe I am just a big RPE! I feel like I deal with that stuff all the time!) When there is too much poop for them to handle, we get, among other problems, drusen.

Janet Sparrow in Therapy for Macular Degeneration: Insight from Acne (catchy title?) said “it is the responsibility of the RPE to internalize the membranous debris discharged daily by the photoreceptor cell.” In other words, they eat eye poop. Unfortunately, some of the molecules in the poop are toxic (as if eating eye poop was not bad enough) and not at all good for the RPEs or surrounding cells.

The theory goes something like this: Less eye poop would make life easier for the RPEs. While we cannot get rid of all the eye poop – after all it is a byproduct of what we want: sight – maybe we can reduce the volume of how much poop we actually have to deal with. If we slow down the chemical processes involved in sight maybe we can produce less poop and thus see for a longer period of time.

They are checking out that theory right now. Foundation Fighting Blindness (FFB) advertised for subjects for a phase 2 (proof of concept) clinical trial of ACU-4429, a “visual cycle modulator”. For our purposes, read “eye poop reduction strategy”.

FFB also published a one page blurb about Fenretinide. Fenretinide has successfully completed phase 2 clinical trials and is on the way to phase 3. They are hopeful it will slow down the visual cycle in those with dry AMD. The slowing should lead to fewer lesions in dry AMD and fewer cases of wet AMD.

Oh, and that chemistry I referred to earlier? I might actually have to understand some of it. Oy. In the visual cycle there is a pigment-y sort of thing called 11-cis. Helping the light signal along its way to become sight causes a chemical change in the 11-cis. In order to get changed back to its original form so it can do its job again and not contribute to the eye poop problem, 11-cis needs help from several molecules, one of which is REP65. REP65. Remember that name. It may be an up-and comer.

written October 14th, 2017 Continue reading “Eye Poop Reduction”

Important Cells

You would think that after saying I was running out of ideas, I would have the good grace to actually run out of ideas and shut up. You should be so lucky!? [Lin/Linda: =I= should be so lucky! ::grin::]

A comment was made suggesting we may have fostered some misinformation. We have stressed the idea of retinal pigment epithelial (RPEs) cells supporting the photoreceptors in the macula so much we may have given some people the impression the RPEs are only under the macula. This is not true. RPEs are under all of the photoreceptors and support all vision, not just central vision.

Once again my very erudite source, Wikipedia (I did print out another article but it is long and involved and I can probably get three or four pages out of it. Do you really want that?) reports the RPE layer was first discovered back in the late 1700s, early 1800s. It was noted to be black in color in many animals but brown in humans. This is because this single layer of hexagonal shaped cells is chock full of – all together now! – pigment. The RPE layer wraps around the back of the eye and ends practically at the iris.

We have talked about a couple of the functions of the RPEs. They are there to feed and clean up after the prima donna photoreceptors – both central cones and more peripheral rods – that apparently cannot do things for themselves.

Something I had not heard of before but makes sense is RPEs, as the conduit from the bloodstream to the interior of the eye, are also the gatekeepers. RPEs are at least partially responsible for the immune privilege of the eye. Remember we talked about how the eye is such a great place to do stem cell experiments because the immune response is so weak? Part of that weakness is due to the great jobs the RPEs usually do. They block bad things entering our eyes from the rest of our system.

RPEs gather up scattered light to make images sharper. That also keeps the light from causing extra oxidative stress.

Simply put the visual cycle is the amount of time as well as all the steps it takes for pigment in the photoreceptors to be depleted and then build back up again. The RPEs do much to control this.

Lastly, the RPEs produce signalling molecules that ‘talk’ to different parts of the system. Lots of very important functions for a one-cell thick layer of cells.

Age-related macular degeneration is not the only condition that causes vision loss due to malfunctions involving the RPEs. A more common one you may heard of is retinitis pigmentosa (RP). Their losses start in the periphery and progress inwards. Those with RP go blind. That’s BLIND. Maybe we ARE the lucky ones.

Do I know why our deterioration generally stops at the macula? Nope, but I have it on good authority it usually does. Usually does not mean 100% guarantee. Just usually. It is the best that I can do.

written October 10th, 2017

Continue reading “Important Cells”

Reading Modern Retina

Never thought I would be skimming back issues of Modern Retina, but here I am! Let us get back to some of the science stuff.

Amyloid beta is a major component of plague found in the brains of those with Alzheimer’s. There has been some suspicion AMD and Alzheimer’s are related at a genetic level. A recent study completed by Cheryl Guttman Krader failed to show any positive effects of injecting an antibody that targets amyloid beta into the eyes of those of us with geographic atrophy.

For the time being this means this line of inquiry will be abandoned or re-worked. Proof of concept did not occur and these researchers might go on to investigate something else.

Why are negative findings good news? One less blind alley to investigate! Since we don’t know which ideas may bear fruit, they all have to be investigated. Eventually we get to only the ones that have the most promise. Scientific method.

And another reason I think this finding is good news? It sort of suggests the Alzheimer’s and AMD connection may not be so cut and dry. Phew!

Here is another failure in proof of concept. Aflibercept is called Eylea when it is used as an inhibitor of vascular endothelial growth factor (VEGF – read “one of the things that makes the extra veins grow in AMD”). Michelle Dalton tried implanting stem cells in the eyes of patients who had been getting Aflibercept. She hoped the stem cell would produce the natural vascular endothelial growth factor and make the shots unneeded.

Unfortunately, many more patients than she had hoped required rescue doses of the drug. However, she also had people who kept the stem cells alive and these imported new stem cells did produce some of the Anti-VEGF molecule. Quantities were just too far below a therapeutic dose.

While this may be a failed experiment on the face of things, it is not all bad. Knowing there was some production of the desired molecules means this procedure may be very helpful once they figure out why it worked the little bit it did. Magnifying that effect may lead to fewer injections.

Last one, David S. Boyer wrote a review on multiple strategies being investigated for treating dry AMD. While many protective strategies for our photoreceptors and RPEs have failed, one they are still looking at with interest is brimonidine, brand name Allergen. Allergen is once again an intravitreally administered drug. (That is needle in the eye. We appear to be destined to join our wet AMD friends in that fate!) Coming out of phase 1 trials, brimonidine looks good. Next for it is phase 2, proof of concept. Will it perform as hoped?

Glatiramer acetate is looking good for reducing drusen. Glatiramer is used to treat multiple sclerosis, a disease in which the immune system wears away at the covering on the nerves. The theory is that glatiramer acts as a decoy to mitigate the autoimmune reaction. This treatment is based on the idea AMD really is an autoimmune disease.

There has been some evidence glatiramer reduces drusen, but Dr. Boyer warned us drusen can become fewer on their own. Drusen regression.

And that is a topic for another page.

written June 26th, 2017

Continue reading “Reading Modern Retina”

Yesterday’s News

Good morning! Lin just shared a video clip from something that looked like a local TV, health program. The clip was on geographic atrophy. That is GA to those in the know.

I have no problem with information being shared with the public. In fact, I think it is a good thing. The more exposure we get and the more noise we make I am hoping two things will happen. One would be law makers (read the deep pockets of government) will be more aware and sympathetic to our plight. (They might also come to realize it is going to cost BIG bucks to care for us!) The other will be people who have AMD will become more knowledgeable and go for help and support.

There are some drawbacks to these little TV presentations, though. For one, they are a bit behind the curve when it comes to breaking new news. The show talked about a fantastic, recent development that would help people with GA.

Fantastic? OK. Helpful? Yep. Recent? Only if you consider research published in 2013 to be recent.   So shoot me. I am an information snob. That information was just too yesterday’s news for me.

I also think they present half information. If you listen to the clip you will hear the expert talk about a ‘subset’ of patients who cannot be helped with current treatments. Not to put too fine a point on this – and look out because I can feel myself getting ready to rant! – but, honey, the group that can be helped with current treatments is the subset! 15% of AMD patients ‘go wet’. The 85% of us who are left are not the subset! (Told you I was going to rant!)

In the clip there is the implication that replacing RPEs will restore sight. We have talked about this a dozen times before. In GA the photoreceptors are dead. There is no sight without photoreceptors. The RPEs are support cells for the photoreceptors. They do not do any of the ‘seeing’.

But my big complaint about this clip? The expert says your world ‘ends’ when you develop GA!!! (Now I am really revving up. Head for the storm cellar!)

With every significant loss, there is a time of dismay and distress. That does not mean the end of your world! Everyone of us here is made of tougher stuff than you could ever have believed. Maybe you have never been tested before, but the steel is there.

Today I taught my class. I attended a staff meeting and saw two clients. Then I came home, walked the dog and made a meal. I am now writing this page. After that I have a psych report to write. Then maybe some down time ‘reading’ a BARD book.

Tomorrow I work, walk with a friend and go to my yoga class. I am making plans to go into New York City with a co-worker next month. The list goes on.

In short, if my world ended a year and a half ago, nobody bothered to tell me about it! I am still going pretty much full tilt!

So, bottom line? I guess it would be listen to the stuff in the media but remember it might not be accurate or current. Once again, caveat emptor. Best sources still remain published research. If you cannot read it or cannot understand it, ask Lin or me to look at it and we can tell you we don’t understand it either!

And about that end of the world business? Don’t believe everything you hear! GA is not a walk in the park. However, if you want to, you can still do that and dozens of other things as well.

Continue reading “Yesterday’s News”

The Blind Spot – Part 2

Lin found about the best article on scotomata that I have seen thus far. It has some basic information. Stuff that I had inferred from other articles but had never been defined.

Scotomata are areas of vision loss surrounded by intact vision. Scotoma, as I said, is Greek for ‘darkness’. Again, not a happy thought.

A scotoma can be in one eye or two. It can be physiological. Everyone has a natural blind spot where your optic nerve is connected to the retina. We don’t realize it because our brain just fills in. No need to worry about physiological scotomata.

Scotomata can also be pathological. Because these are the result of a disease process, these are the ones we get to worry about.

Relative scotomata are the kind you can ‘see through’. You no longer have a full complement of cones but enough remain to sort of get the job done. I have relative scotomata in my eyes. Unfortunately, one of them probably just had a massive die off because it has gotten several shades darker.

When the scotomata go black you have something called absolute scotomata. Those are the areas in which the photoreceptors – in our case, cone cells – have pretty much all died.

A positive scotoma is one that is obvious to the owner of the eye. I KNOW – I am in fact positive! – I have blurry spots and I am aware one of them just darkened.

We had a comment from a reader who has a negative scotoma (maybe two). She wrote she quit driving when cars on the road would disappear and reappear. Her brain was ‘filling in’ the blank spot with a vision of an empty road.

Aren’t brains just amazing? Scary, but amazing. After all, that little trick could kill both the brain and its owner! (Or would that be its servant? Hmmmm….)

And that, my dears, is what I know about scotomata. Not much considering I am the ‘proud’ owner of two of them! Will they all progress to black? Dunno. I keep looking and asking and continuing to feel like a mushroom. You remember: keep me in the dark and feed me bullshit.

What I was told was it was not a conversion to wet. Reassuring but I never thought it was. I was told there was no obvious difference between my last OCT scan and this one. I guess that means the die off was not severely massive, only mildly massive (but I can still see the difference!!!).

I was also praised for being proactive with my vision care. Important for us all.

So, darkening of your scotomata apparently may occur. It probably means things are dying in there. That is my interpretation, though. I was told it was progression of the disease, but if you have a disease in which cells die, would not progressing be cells dying? Stands to reason; yes?

If you perceive a significant change in the density of your scotomata, call your doctor and go in for an OCT just to be on the safe side. Not much can be done for the progression of the dry, but on the off chance you are converting to wet, you need to catch it quickly.

Thus we end another ‘adventure’ in AMD. Anyone else having these problems? Sigh.

Continue reading “The Blind Spot – Part 2”

SOS

J.R.R. Tolkien said once that “false hopes are more dangerous than fears.”

Every once in a while Lin and I hear someone make a claim about AMD that we know is not true. Then someone picks up on the claim, believes a foolproof treatment or cure has been found and they have been saved from blindness. This is only to have their hopes dashed a day or two later when the truth comes out. This can be heartbreaking to watch, let alone experience.

Right now, to our best knowledge, there is no cure or available treatment for dry AMD. There are a number of treatments being tested. Even the treatments most advanced in the process are several years away from being available to the general public.

The SOS has been sent. The cavalry is coming but they are still a very long ways away. It is up to us to hold out until then.

When I talk about medical treatments, I am talking only about drugs that will slow the deterioration process. To my knowledge there are no drugs that will regrow RPEs and/or photoreceptors. The drugs will not restore sight. If you have lost your photoreceptors, at this point in time you have lost your sight.

Stem cell replacement at present is for RPEs only. Remember RPEs are only servant cells. They take care of the photoreceptors. The pizza delivery guy may help to keep the neurosurgeon alive but we would not expect him to do brain surgery. Same concept. The RPEs will feed the photoreceptors but they won’t turn light into sight. The best we can hope for is for some of the photoreceptors that are at death’s door to be revived and start working again.

Dead photoreceptors are just that, dead photoreceptors. I have geographic atrophy. Photoreceptors that should be in that area are dead and gone. There is currently no way to get them back. The last I heard they were able to grow photoreceptors but they had been unsuccessful in having them connect with the rest of the nervous system. You have a cell phone in a deadzone. Your phone works great but the signal goes nowhere.

Am I saying have no hope? Hell, no. I live on hope. Just have realistic hope. Treatment is coming. Cures are coming. Just don’t expect them next Tuesday!

Why do people get so hung up by these crazy claims? First of all, they are desperate. Any port in a storm. Beyond that I am not sure. I wasn’t able to find a lot on the mechanisms behind false hope. Maybe it is better to feel better for a little while even if you are destroyed later. Sort of like substance abuse. It felt good at the time.

We try not to deal in rumors, just facts. If we say something you do not think is true, challenge us. If you read or hear something different from what you hear here, alert us to it and we will research it. Better yet, anybody capable of web research might volunteer to help us. There is a lot of information out there.

In the meantime, keep the faith but don’t go crazy. We are not there yet.

Continue reading “SOS”

I Am Not a Doctor

Commentary: Lin just sent me a post from someone in the Facebook group. She asked for my reactions. The person is claiming he completely reversed neovascular (wet) AMD with nutritional treatments. Here goes.

First the disclaimer. I am not a doctor. I am not a nutritionist. I am a woman with dry AMD who has tried to educate herself about her disorder. Therefore I do not, by any stretch of the imagination, have all of the answers. End of disclaimer.

That said, let me congratulate him on his greatly improved vision! I am glad he are doing well.

I know some of what he says is true. In general the diets of those in the developed world are atrocious. We should be eating many more fruits and vegetables, especially our leafy greens, than we do. The reason taking the AREDS/AREDS2 supplement works to slow the progression of the disease is probably our poor diets. If we ate well, the supplements would not be so needed. [Lin/Linda: I have to mention that there is some risk taking the AREDS or AREDS2 with 80mg zinc.  It can cause problems in the genitourinary tract but there is evidence that for people with certain genes, that high dose of zinc can cause their AMD to progress faster. Since not everyone has easy access to the genetic tests, there are supplements with no zinc or less zinc.  Check out this post for more information.]

Angiogenesis is the growth of new blood vessels. This is a hallmark of wet AMD. There is some evidence angiogenesis is part of the healing process and may be triggered by inflammation (Reiner O. Schlingemann in Role of Growth Factors and the Wound Healing Response in Age-Related Macular Degeneration). There is also evidence that retinal hypoxia (in English? Your retina is gasping for oxygen) is a trigger for angiogenesis and neovascular (wet) AMD. (Citation same guy. It is so nice not to have to follow APA format? [Lin/Linda: APA is the American Psychological Association and when you write something for them, you need to follow a very strict format for references to articles.]

That said, theoretically it is possible he hit upon a combination of nutrients that would reduce inflammation and increase oxygenation to his retina, thus somehow stopping the angiogenesis. Did this happen? No clue. I am just sort of a slightly-too-smart-for-my-own-good, visually impaired lady. (Gets me in a lot of trouble.) Is it possible? Sure. “There are more things in heaven and earth, Horatio, than are dreamt of in your philosophies.” (That is the bard, of course).

Now, it would be my supposition – again totally unfounded – he was not in advanced AMD and had not experienced much if any photoreceptor death i.e. geographic atrophy. Unfortunately from what I have been told dead is dead with those. They would not have come back.

That is pretty much my take on it. Again when it comes down to it, I know nothing but I have a helluva lot of opinions. Don’t believe me. Offer your opinions. What do you folks think? Continue reading “I Am Not a Doctor”

News: July 13, 2016

A Perfect Storm

January 31, 2016 was a breathtakingly beautiful Winter day. I know it was January 31 because I still have the ski tag on my jacket.

That day I drove to Wilkes-Barre and had lunch with a friend. I was able to read the menu. I was able to navigate the roads.

I left lunch and went to a local ski area where I had a glorious, three-hour session of skiing. The sky was bright blue with not a cloud to be seen. The snow was a glittering white. The air was mild but not mild enough to make ‘spring conditions’ also known as slop.

I wore my sunglasses constantly. Of course I wore my sunglasses. I knew I had macular degeneration and that’s part of the drill. My sunglasses were red and matched my jacket. Even we old ladies with eye problems like to look good on the slopes.

Of course I wore my sunglasses. I knew I had macular degeneration and that’s part of the drill.

Later, my husband insisted it was photostress that destroyed my macula. He believed the day on the slopes and the reflection on the snow was just too much for my eyes to handle. My optometrist and ophthalmologist did not agree but there is that lingering doubt.

A quick detour here to talk about photostress. Photostress is, exactly as the name suggests, the stress that light puts on your eyes. When the photoreceptors make ‘light into sight’, they deplete a chemical, basically a pigment, that has been fed to them by the RPEs. Remember those Servant cells that take care of the photoreceptors? To my understanding, photostress happens when the light is eating up so much pigment that the RPEs cannot keep up replacing it.

If you have ever gone ‘snowblind’ you understand what I’m talking about (although snowblindness is caused by something different).

In the past I have had trouble with photostress. I walked from the car to the building housing a yoga festival. I had forgotten my sunglasses but did not think much of it. I stood in the lobby for five minutes–blind.

There is a photostress test in which they purposely make you go blind and see how long it takes you to recover. It would appear that recovery time is the key variable here.

Recovery time is the key to knowing how serious the photostress is.

If you’re reading this website just because you have nothing better to do or out of curiosity, if you do not yet have a diagnosis of retinal disease and you become blind after coming in from bright light with long periods of recovery, get thyself to a reputable eye doctor. It is very possible you have the beginnings of a retinal disease. That’s the public service announcement.

If you become blind after coming in from bright light with long periods of recovery, get thyself to a reputable doctor.

Written February 2016. Reviewed September 2018.

Continue reading “A Perfect Storm”

The Man Behind The Curtain: The Wizard of Wills

For a rock star of retinas, Regillo, was not all that imposing. I finally got to see the man after multiple tests by multiple technicians. Towards the end of the gauntlet, I asked the girl if everyone went through so many tests. She confided in me that many people do not get as far as I got. That was certainly encouraging. My inner voice had been telling me I was on the right track. The stars seem to be aligned. However, this was the first outside confirmation that I had chosen a good path.

I had chosen a good path. The stars seem to be aligned.

When the good doctor came into the room, I was studying the scans of my left eye–not quite sure why, but he appeared to be amused. Perhaps this was highly unusual behavior in a patient with Age-Related Macular Degeneration.

Regillo proceeded to challenge me to tell him what I saw. So I told him. The lower area was Bruch’s membrane. This membrane is the connection to the proverbial greater world. It brings nutrients to the RPEs and takes away the garbage. The level above that contained the RPEs. It also contained yellowish piles of eye poop more appropriately known as drusen. Level above that were the photoreceptors. The divot in the top was geographic atrophy and the reason I was there.I might have impressed him. After all, I am more than just an impaired eyeball.

I am more than just an impaired eyeball.

Strange, staccato, conversation followed. I told him I wanted to read the article on the phase 1 results when it came out. It was out and he gave me the citation. I told him I had every intention of being in phase 2. The conversation was a bit of a tennis match. At times we are even finishing each other sentences. I felt as if we were definitely on the same page.

Also, talking to the doctor I felt as if we were in a cat & mouse game and I was the mouse. He seemed overly interested. No, not that way, I felt like I was prey but in a professional, scientific way. It was like he had found a live one. After reading the phase 1 results that were published in Lancet last winter, I figured out why. The mean age of the cohort for the phase 1 study was 77 years of age. The team was planning on doing a 15 year longitudinal follow-up on the study. There was now no confusion in my head as to why Regillo was looking at me as if I were a live one. Hell, in 15 years, with subjects like that, I was going to be the only live one!

The mean age in the phase 1 study was 77 years of age. That means in the 15 years of the study, I was going to be the only live one!

Written February 2016. Reviewed September 2018.

Continue reading “The Man Behind The Curtain: The Wizard of Wills”

Resources

June 2023 There’s an announcement that since Sue has not written any new journal pages for some time, the site has been archived until we can decide if the work necessary to make sure all information is accurate and up-to-date can be made. In the meantime, you’ll get some pages ‘not found’ or ‘private’ until that decision has been made. The emphasis for several years has been on the Facebook group.

2/14/2022 Because of the rapid and constant growth of our Facebook group, I cannot keep this list updated.  I have a large amount of information available in the Facebook group in Guides which are like chapters in a book or lessons in a course. Plus, in 3 years, the amount of information in the posts and comments is quite substantial. I recommend that you join us there where you can get the information and the support to help you in your journey.  Thanks for understanding. Hope to see you there! Lin/Linda…
I’ve added some pages from that group that might be of interest to you.

Frequently Asked Questions

Click here for the list of Frequently Asked Questions from our Facebook group.


AREDS2-based Supplements

There are several pages on the site that explain what AREDS2 means and who the AREDS2-based products are for. Click here to go to a list of articles.

AREDS2-based Supplements With 0 or 25mg of Zinc

Click here for the list.


Navigating

There are a lot of links here.  I’ve set up this page so that when you click on a link (words that are underlined & in blue or green), a NEW tab will open in your browser and this page STAYS WHERE IT IS.  When you are done with the new page you opened, just close it.  You do NOT need to use the back option.  If you click on a link and the new page replaces this one, I’VE MADE A MISTAKE so please let me know by sending me an email at light2sight5153@gmail.com.  Let me know exactly which link or links do not open a new tab or window.

Errors: If you click on a link and you get a ‘page not found’ error, please let me know by sending me an email at light2sight5153@gmail.com.  Let me know exactly which link or links do not open a new tab or window.

Additions: If you have a link you’d like to add, please email at light2sight5153@gmail.com.


Topics-click below to move to a topic

Links We Like

  • Click here for a GREAT resource where you answer some simple questions and you get a customized guide based on your responses
  • Click here for a great glossary
  • Click here for Low Vision Resources: A List of Lists (such as 8 ways to slow AMD, 15 tips for family and friends, etc)
  • Videos
    • Click here for several videos
    • Click here for the UK Macular Society’s Say Hello to Mac
    • Click here for one that uses illustrations and animation (explains how wet AMD progresses and how the injections work)
  • Click here for a description of dry vs. wet AMD (we are not recommending any products in this article)
  • Click here for an article about depression after diagnosis
  • Click here for a very comprehensive page about wet AMD
  • Click here for a very comprehensive page about dry AMD
  • Click here for a FAQ (Frequently Asked Questions) that answers a long list of questions such as ‘will resting help my eyes?’, ‘Can I see for myself if my retina or macula shows any signs of damage before I have symptoms?’, ‘why don’t new eye glasses help?’, ‘what is meant by degeneration?’, ‘is a macular hole the same as macular degeneration’, ‘I have had dry MD for years. Does this mean I’m going to get wet MD too?’, ‘No one else in my family has MD. Why did I get it?’, ‘can drusen be treated?’, ‘I have changes on the Amsler Grid, does this mean I have MD’, ‘I have Wet MD but my Doctor says there is nothing he can do or no treatment available. Why is this?’
  • Click here for a short introduction to stems cells, what they are and how they can be used.

See what vision is like at the various stages of AMD

Click here to find ways to see simulations of what vision loss due to AMD is like at various stages.


Glossary

Go to the Top


Websites devoted to AMD and Other Forms of Macular Degeneration

listed in no particular order

Go to the Top


Websites containing information about AMD and Other Forms of Macular Degeneration

listed in no particular order

Go to the Top


Support

I’ve not been able to verify if these are kept up to date. Let me know if you find that they are not or if you have one  you’d like to add.

Message Boards including ones from
By postal mail

I don’t know if these are still accurate.

  • Association for Macular Diseases
    210 E. 64th Street
    New York, NY 10021
    (212) 605-3719
    – Offers education and information on macular disease through seminars, newsletters, and a hotline. Offers counseling to patients and their families.
  • Macular Degeneration International
    is now a part of Foundation Fighting Blindness
    Toll Free Helpline 1-800-683-5555
    EMail: MDInfo@blindness.org
    – Provides support for people affected by inherited macular degeneration including Stargardt’s disease.
Start Your Own
  • Vision Support Group-download video presentations  This group provides free information and support through presentations to groups of senior adults affected by macular degeneration and related retinal diseases.  You can join & get access to their materials so you can use them in your own group.
On the phone/telesupport

Go to the Top


Where to find services

  • In the US: click here to find a low vision center, retina specialist, state agency, ophthalmologist
  • In the UK: click here to support services (listed on the right side of the page) such as skills for seeing, counseling, access to treatment…and more
  • In the US: click here to search for a wide variety of services (more than the link above)
  • In Australia: click here to find an ophthalmologist and optometrist
  • Worldwide: click here for resources worldwide

Resources for Students

Go to the Top


Books and reading materials

Specific Titles

Sources of Books

Formats: Braille, large print, e-book and audiobooks

Go to the Top


Videos

Go to the Top


Personal stories of living with AMD

Go to the Top


Online newsletters

Go to the Top


What is AMD?

Wet Form
Dry Form
How fast does AMD progress?
  • A good article about how difficult this is to answer
  • Great video that explains why early detection is important especially when detecting the change from dry AMD to wet

Go to the Top


What is Stargardt’s Disease?

Also called Stargardt’s Disease (SD) or Stargardt Macular Dystrophy (SMD) or Juvenile Macular Degeneration (JMD), it’s an inherited, juvenile macular degeneration. The progressive vision loss associated with Stargardt disease is caused by the death of photoreceptor cells in the central portion of the retina called the macula.

Go to the Top


The Science Stuff

Role of RPEs

Geographic Atrophy

Go to the Top


Symptoms

Charles Bonnet Syndrome/Visual hallucinations

Other problems with vision & AMD

  • problems with visual acuity, photostress, blindspots, color vision, sensitivity to light, depth perception
  • eye problems that have similar symptoms as AMD:

Go to the Top


Risk factors

Age

  • Age is a large factor but can start earlier
  • Much less common are several hereditary forms of macular degeneration, which usually affect children or teenagers. Collectively, they are called Juvenile Macular Degeneration. They include Best’s Disease, Stargardt’s Disease, Sorsby’s Disease and some others.  See Stargard’s Disease section above.

Diet/nutrition (working on this section)

  • diet low in various nutrients & high in others have been linked to AMD.
  • See Nutrition and Vitamins/Supplements under Self-care/self-maintenance below.

Race

Gender

  • AMD more common in women perhaps because women live longer than men

Uncontrolled high blood pressure

Uncontrolled high cholesterol

Smoking

Blue Light

Eye Color

Aspirin & other medications

Other possible causes

  • Biological Process in Wet AMD – some evidence that the photoreceptors are starved by the lack of food (oxygen & nutrients in the blood) and the growth of blood vessels is to compensate for that.

Connection between AMD and Alzheimer’s Disease

Go to the Top


Treatments

  • FDA approved options in the US, injections, implantable telescopes, laser treatment (also outside the US)
Injections for Wet AMD
Telescopic implants
Are there new treatments in the pipeline?
Vitamins (see Self Maintenance/Self Care section below)

Go to the Top


Research/Clinical trials

 

How can I become a part of a clinical trial?

  • A list of sources of information about clinical trials and how to find out for you to participate in.
  • You can search for clinical trials from the links above
  • There are registries where you sign up and enter information about the status of your eyes.  Researchers will use this information to find people that match their research and contact you.  Click here for more information about these registries in the US and elsewhere

Gene Therapy

Bionic Eye/Retinal Implants

  • What is a bionic eye?  It’s also called retinal implant or retinal prosthesis.   Implant is put in retina, camera worn by person sends image to implant which stimulates optic nerve
  • Click here for overview of retinal implants including videos of how it works & interviews with people who have them.
  • March 21, 2016 UK Bionic eye being tested
  • Here’s an article about one being developed at Carnegie Mellon institute in Pittsburgh, PA.

Nutritional Supplements

  • See Vitamins/Supplements section below.

Stem Cells

Go to the Top


Coping with low vision

Low Vision Aids

Wearable Technology

  • coming soon!

Suppliers of low vision aids

Financial Help

Sunglasses

Lamps

Transportation

  • A website for the US where you enter your zip code and transportation options for your area will be shown.

Bioptic Driving

Depression

Checking vision

Amsler Grid

Go to the Top


Self maintenance/self care

Low vision rehabilitation

Vitamins/Supplements

Nutrition

Exercise/Activity

 


More to come, you can check out these posts now

Video: Overview of Assistive Technology for People with Low Vision

Highlight: How do I use Zoom for Apple products?

Highlight: What about Apple’s accessibility features?

News: Top 10 Low Vision Aids for AMD

 


Go to the Top

Home

Research

Not being one to be told there’s nothing I can do about something, I went back to my research. There seem to be a couple of different avenues of research. They were working on lasers to blast the drusen, aka piles of eye-poop but it looked to me like a hoarder intervention. Somebody comes in and cleans up the mess one time. Problem solved for now but not later. They would have a clean place to live but would eventually start to become messy again. The second thing I found looked more like the Merry Maids that were cleaning up regularly. However, it did not solve the problem of who is going to feed the Master photoreceptors? The third option was to essentially put the RPE Servants that were left on steroids. The live ones would work harder but would that not mean they wear out more quickly?

There’s research focused on cleaning up the ‘eye-poop’ called drusen.

There was one I liked, OCATA, originally known as Advanced Cell Therapy (ACT) , that was trying to replace RPEs. They were actually giving the little guys some help in order to save the Master photoreceptors. The way they were doing this was with stem cells.

There’s research using stem cells to replace the RPEs.

Although some people see stem cell research as cutting up dead babies, this is not the case. There are several lines of stem cells that have been derived from fertilized eggs that were never implanted. Some of these lines of stem cells are 20 years old. They have been massaged and manipulated so that there would never be the possibility that they could become functioning human beings. If they were not being used for research they would be flushed down the proverbial toilet.

Stem cells can be harvested from old fertilized eggs not dead babies.

The research that interested me–and still interests me–involves stem cells that have been developed specifically to become RPE cells. The theory is that replacing RPE cells with new ones and giving the little Servant guys some help will allow more photoreceptors to live and turn light into sight.

So where, pray tell, does one find someone to do this procedure? The problem is that this is very new research. It has worked on rats and other traditional lab animals (and you college psychology students, I am not speaking of sophomores). However, work on human subjects is just beginning. At the time of this writing, hospitals in Florida, Massachusetts, California and Pennsylvania as well as in foreign locales such as London and China have only completed phase 1 research. Phase 1 of any clinical study is the safety and tolerability portion. [Lin/Linda: it’s 2018 & some of these studies have advanced to the next phase or phases. The links before will give you current information]

Warning: there are doctors and clinics in the US that are offering costly stem cell treatments that have NOT been proven safe or effective through research.  Before you enter into any stem cell treatment, please do your homework!  Click here for an excellent article called Nine Things to Know About Stem Cell Treatments.

Click here for current research using stem cells for Macular Degeneration

Phase 1 results have been extremely promising. For those who are capable of using the web, there is a Lancet article by Schwartz and Regillo that summarizes the study. Essentially, they found the stem cells did not do anything strange or different when implanted in eyes. Preliminary data suggested that it was safe and tolerable. Even more exciting, they found positive therapeutic effects. A great number of the people who had volunteered and participated in the study showed cessation of deterioration and even improvement.

Phase 1 trials using stem cells is VERY promising.

So why not replace the photoreceptors as well as the RPEs? After all, when the RPEs die, the photoreceptors die. Would it not be reasonable to replace them both?

Unfortunately, medical science is not to this point as of yet. They have been successful in growing photoreceptors in the lab. They have been successful in implanting photoreceptors in the eyes of rats. The only problem is that these will not connect into the neural net. It’s sort of like having invented a cell phone without having a tower for it to work through. You can talk on your phone all day but the message goes nowhere.

They can grow photoreceptors in the lab, implanting them in rats but they won’t connect to the neural net.

That said, they are still working on it very diligently. Some of the literature suggests that it will be quite awhile. However, it will be coming.

If you are interested in seeing some of the studies that are being done on eyes and other medical research, I would invite you to go to the clinical trials website. It is a government website that lists all sorts of fascinating things. Many of them are looking for clients.

You will discover that there are dozens, if not more, of studies that are related to eyes. There are multiple studies related to Age-Related Macular Degeneration. So why would that be?

Someone, I am not remembering who at the moment, has launched the Audacious Goal Project. The Audacious Goal Project is aiming to eradicate blindness in the lifetime of some of you younger folks.

Click here to learn more about the Audacious Goal Challenge in Vision Research and Blindness.

Like the name says it is an audacious goal!

Why now?

And questioning again, why now? What is happening that vision is such a hot topic that we need a national program to deal with blindness?

The truth of the matter is, the pig through the population python is getting towards the end. We baby boomers from the 50s and the 60s have always presented challenges. We have always been very popular and our hot topics have been the topics of the nation. When I was a little girl, they were building elementary schools left and right. Then everything was sweet 16 and on through my lifespan. Right now, everything is security call buttons and retirement accounts. We drive the economy.

We baby boomers from the 50s and 60s have always presented challenges.

Because there are so many of us, our concerns are essential. One of our big concerns is vision. According to my research, AMD is the leading cause of blindness in the developed world. In the United States alone there are as many as 11 million people who have some form of AMD.  They are predicting there will be 22 million by 2050! 

This is going to be a massive drain on the country. When somebody suddenly realized what the numbers were going to look like, they decided they had better do something to ameliorate the problem. Thus, all the research.

Click here for more facts & figures from 2016

As many as 11 million people in the United States have some form of age-related macular degeneration. This number is expected to double to nearly 22 million by 2050.

Written in February 2016. Reviewed September 2018.

Continue reading “Research”

The Science Stuff

Now comes the science stuff. Your eye has an area that takes care of the background and an area that takes care of seeing the stuff that you really want to look at. The part that does the seeing of what you really want to look at is called the macula. It is part of the retina which is the inside back layer of the eyeball. The retina converts light and images into electrical signals that are sent to the brain—light into sight.

Click on the image to get a detailed explanation of the diagram

 

The macula is made up of the photoreceptors, rods and cones, and the retinal pigmentation epithelials, RPEs for short. There are other parts of the eye, but this is Age-Related Macular Degeneration for Dummies.

 

 

I am not a physician nor a scientist.  There is quite a lot of information on the Internet if you’d like more details.

According to what I have read, the photoreceptors have the important job of turning ‘light into sight’. However, they are somewhat prima donnas and not very capable of taking care of themselves. I refer to them as the Masters. The RPEs are the Servants. Their job is to do everything for the Masters that the Masters cannot do for themselves. The Servants (RPEs) go to the store (the blood vessels in the choroid) and bring home nutrients for their Masters (the photoreceptors). They cook up a concoction of pigments and feed their Masters. They also clean up after their Masters.

The photoreceptors are the Masters, the RPEs are the Servants who feed and clean up after the Masters.

The trouble comes when the Servants/RPEs are not doing their job anymore. One of the first signs of AMD is the presence of something called drusen.  My reading very nicely indicated that these are fatty, metabolic byproducts of the photoreceptors’ job of turning light into sight. Basically, it seems to me that they are piles of poop. These piles of eye-poop suggest that the RPEs are not functioning as they should.

When the RPE Servants don’t clean up the eye-poop, it piles up & creates all kinds of problems!

As the eye-poop builds up, the environment becomes more toxic to the Masters, the photoreceptors. The Servants, the RPEs, also are not doing an extremely efficient job of feeding their Masters. As a result, both the RPEs and photoreceptors start to die. This causes the vision loss.

The Masters and the Servants die.  That’s what causes vision loss in dry MD.

Written in February 2016. Updated September 2018.

Continue reading “The Science Stuff”