macular degeneration, macular, diagnosis Bruch’s membrane – 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

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Down the Rabbit Hole

Hey, back to the article Lin sent, Clinical Endpoints, etc. I now have another question: what scale is my retinologist using to classify me as ‘advanced’? Turns out there are two rating systems in use. One is the Wisconsin Age-Related Maculopathy Grading System and the other is the Beckman Initiative for Macular Research scale. If you are rated on the first one you would be advanced if you had foveal involvement. In the second one any type of GA, foveal involvement or no, you are considered advanced.

I have foveal involvement at least in my left eye so for me, it is actually a moot point. If you have been told you have advanced AMD, you might want to find out which scale is being used. It would appear – and again this is me surmising, not someone who knows something – a pronouncement based on the Beckman scale might not be as bad. You might still have a functional fovea.

Moving right along, lunch!? Be back in about 20!

We are still getting vine-ripened tomatoes out of the garden. In October! Weird…but yummy. Remember to stop and smell the roses, or in this case, taste the tomatoes, on your Journey. Neither one requires decent eye sight!

Back to work. We have talked about optical coherence tomography (OCT) before. I did not know it was not invented until 1996 and not improved to be of any ‘real’ benefit until 2003. One more reason this is not your parent’s AMD. At least the doctors and technicians can see what they are looking at much better now!

I think I said this before, too, but OCT screening can give views in 2 and 3 dimensions. You get depth as well as width and height measures of your lesions, or in my terms, divots. In GA OCT (and an ABC…XYZ to you, too!) you can see sharply delineated regions of degradation in both RPEs and photoreceptors and your choroid poses – gasp! – naked for the camera.

The article also mentions choriocapillaris and thinning and apposition of the overlying nuclear layer. Hold a moment for the translation for the rest of that. What did I EVER do without my iPad?

The capillary lamina is also called choriocapillaris. It is a layer of itsy bitsy blood vessels immediately adjacent to Bruch’s membrane. My assumption is it is below the retina but above Bruch’s membrane The drawings in Wikipedia are too small for me. [Lin/Linda: I can’t find a much bigger picture but this one shows that it is below Bruch’s membrane.]

Apposition means next to. The inner nuclear layer is, again according to Wikipedia, my highly professional source, a layer of the retina made up of closely packed cells of the three following types: bipolar cells (I wonder if they have mood swings? Ouch. Bad psychology joke), horizontal cells and amacrine cells.

Amacrine cells? No clue. That will just have me falling down another layer in the rabbit hole. I am quitting before I meet the Queen of Hearts. Off with their heads!

I will try to get through at least part of the rest of this article later. Maybe impossible but, hey…..

“Sometimes I’ve believed as many as six impossible things before breakfast.”

Lewis Carroll, 1865

Written October 8th, 2017 Continue reading “Down the Rabbit Hole”

Always Learning More and More

Moving right along with the article I am reading (in Webvision’s Age-Related Macular Degeneration), I am finding a lot of new vocabulary and abbreviations. Have you heard of PEDs, for example? PEDs are not nylon footies. They are pigment epithelial detachments. They happen when a bunch of drusen join forces and push up the RPE layer of your eye. Since the RPEs are under the retina and need to be in contact with Bruch’s membrane in order to take care of the photoreceptors, having them jacked up is not a good thing.

There is more and more information suggesting Bruch’s membrane is not totally blameless in this whole debacle. I am not going to pretend to understand it but there is evidence structural and biochemical differences in Bruch’s membrane occur in those with AMD but not in people who do not have the disorder. It may not be all the fault of the RPEs.

Recently I have been seeing the terms classic, predominantly classic and occult to describe different forms of wet AMD. They are mentioned in the article I am reading but not well defined.

According to the American Macular Degeneration Foundation the terms classic, predominantly classic and occult describe the choroidal neovascularization (read “formation of new blood vessels in an inner layer of your eyeball”) that happens in wet AMD. Classic choroidal neovasculazation is characterized by well-defined boundaries. Average visual acuity is between 20/ 250 and 20/400.

Occult CNV sounds like it should be scary but it is actually the more benign. Occult lesions are not as well-defined as classic ones. They tend to leak less and average visual acuity is between 20/80 and 20/200. If given a choice, I would take this one!

Predominantly classic is, as it sounds, a mixed type. The other designation for this type is minimal classic.

According to Joachim Wachtin in Classical Choroid Neovascularization CNVs can also be classified by where they can be found in relation to the fovea. Some of them are directly under the fovea and are called subfoveal. Those that are extremely close to the fovea are called juxtafoveal and the ones that are farther away are called extrafoveal.

Like I said, lots of new vocabulary coming our way! But I do believe that, when in a strange land, you should always learn a few basic phrases. These are some basic phrases in the land of wet AMD.

My article takes a serious detour into science babble and I truly don’t understand much. Glaze over time! That means I am going to stop sharing info from it.

Hope these scraps of information fit into your ‘puzzle’ somehow. One of these days we will have gathered enough pieces of knowledge to actually figure out what the picture is!

In the meantime, keep on learning. In the famous words of Schoolhouse Rock, “….because knowledge is power!” Gather knowledge. Be powerful. Continue reading “Always Learning More and More”

Always Learning More

Hey, there! I think I have found a good article on macular degeneration, our favorite but somewhat distasteful topic. The article is in Webvision and is entitled Age-Related Macular Degeneration. Another catchy title. The main author is Hageman.

Did you know the name up until around 1990 was ‘senile macular degeneration’? Makes it sound like our eyes have lost some of their mental faculties. Glad that was changed!

Also discovered the fovea is the center of the macula. It contains the highest concentration of cone photoreceptors and is the only region of the retina that can attain 20/20 vision.

I think when my optometrist said I had such an abrupt vision loss because the deterioration had reached the center of my macula she was talking about the loss of my fovea. That means 20/20 vision is no longer possible for me. Even if I use prisms or eventually get that eye max mono thingee, things will not be ‘perfect’. [Lin/Linda: she means the EyeMax Mono lens implant.]

This article says macular vision is 10% of vision! Estimates of degrees of arc of potential loss seem to be getting better, but don’t get too excited. Remember we are talking my interpretation of things I read. It is guess-work. I know nothing.

Although I used to think hard drusen sound more ominous than soft ones, it is actually the other way around. Hard drusen are smaller and soft ones are larger. If they are looking in your eyes and mention soft drusen, you have more of a problem than if they see hard drusen.

I thought that all dry AMD would progress to GA (geographic atrophy) if the person lived that long. This article says only 10 to 15% of dry AMD patients progress rapidly enough to ‘achieve’ GA. Interesting.

That means my visual state is something many of you will not have to experience. That is a good thing! And FYI? I am functional so you can remain functional as well.

For you ‘wet’ folks, the article once again cautions you to stay on top of things and get your shots. Left to its own devices wet AMD progresses to a cicatrical stage. Cicatrix is a fancy word related to scars and scarring. Disciform scars occur when fibrous tissues develop in Bruch’s membrane between the RPEs and the retina. Scarring is, needless to say, not good and can result in severe vision loss. Bottom line for this paragraph is: do not allow bleeds to happen to you!

Closing in on my 500 words and I still have pages to read in this article. I think I will close this page, read some more and start another.

And FYI, I emailed by doctor. And – while he also believes the increased density/opacity of my blind spot is related to expected disease progression – I am going in for a vision screen in two days. Perceivable changes in your vision? I expect you to call, too. Check it out.

written April 25th, 2017

Continue reading “Always Learning More”

In a Pig’s Eye

About three hours later and the ‘tapering’ snowstorm is not tapering. Anyone ever read ‘Ghost Story’ by Peter Straub? It scared my socks off on a summer day. On a day like today I would probably be quivering under the covers! [Lin/Linda: this was written in March of 2017.  In ‘real time’ it’s July 2017 when many of us in the US are having record high temperatures. Thinking of snow is ‘refreshing’!]

In ‘Ghost Story’ it starts to snow. They cannot keep the roads open and it snows. The electricity goes out and it snows. The phones go out and it snows. Eventually some smart soul figures out there is a malevolent force at work in this small, New York town. Yipes!

‘Ghost Story’ is on page 3 of the Gs in BARD. It is available as an audiobook on Amazon for $17.95. If you are still able to read print, you can get it used for about ⅕ of that price.

Another way to scare your socks off? ‘Turn of the Screw’ by Henry James. This one is a classic and free on Kindle. It is on page 15 of the Ts in BARD.

I will vouch for them both as excellent reads. Anyone else have any recommendations they would like to share? Just because we cannot see so well, doesn’t mean we cannot enjoy a good book. Since we like to think of our group as a cut above, try to avoid recommending trashy novels.  Although for a whole series of semi-trashy novels I would recommend the ‘….in Death’ series by J.D. Robb, also available on BARD.? [Lin/Linda: one of my favorites, too, but I don’t think I’d call it ‘trashy’ but definitely R-rated.   Click here for the list in order of publication date.  They’re available at amazon.com, too.  Click here for the first one ‘Naked In Death’.]

Anyway, that was NOT the way I was going to start this page. Not the topic either. I just looked outside and found it all a bit surreal. We are approaching an accumulation of two feet. Not much for some other places but impressive for Pennsylvania.

What I was going to do was tell you about “in a pig’s eye” and how the phrase now has a new meaning. For our international friends, “in a pig’s eye” is an old American expression that implies disbelief. It is the antiquated version of “No way!”

Now, they are finding a way to study drusen in a pig’s eye. Well, actually in a culture medium in which they have placed retinal ‘pig’-ment epithelium cells. (Alright, so it was corny, but I couldn’t resist.) They have found out that pig RPEs are similar in many ways to human RPEs. They have discovered the RPEs in early AMD are actually still functioning and the Bruch’s membrane may have more of a part in the process than previously believed.

This should just be the first of many good discoveries to come out of the pig’s eye experiments. Because they are now able to do a lot of manipulations of pig RPEs being grown in cultures, research can go faster. A lot faster than it would go trying to get people to have all these manipulations done on THEIR eyes.

So there is ever increasing hope here. When you tell people there is a bright future for AMD folks and they say “in a pig’s eye!”, your response can now be “Exactly!”

Keep on keeping on. There is hope.

Now could somebody stop this snow? Enough is enough already! Continue reading “In a Pig’s Eye”

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”

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”