macular degeneration, macular, diagnosis geographic atrophy – My Macular Degeneration Journey/Journal

Questions and Answers – FDA Approved Treatment for Advanced Dry AMD/Geographic Atrophy/GA

Questions and Answers – FDA Approved Treatment for Advanced Dry AMD/Geographic Atrophy/GA

You may find a lot of the answers to your questions in this article ‘SYFOVRE for geographic atrophy in macular degeneration’ https://clearsightcorner.com/articles/syfovre-first-treatment-approved-geographic-atrophy-amd

Many of your questions will have to be answered by your eye specialist. A retinal specialist, a specially trained ophthalmologist, will administer the treatments.

Syfovre is pronounced “si-FOV-ree.” Where DO they get these names?

Patient Brochure

This is the patient brochure with some of the answers to your questions. https://syfovre.com/wordpress/wp-content/themes/apellis/pdf/SYFOVRE-Patient-Brochure.pdf

What is ‘geographic atrophy’ that I’m hearing so much about now?

A recent CBS News article and interview has been widely shared alerting many to this new treatment (approved on Feb. 17) so we’re getting a lot of questions. Here is the article and video. You may have heard me talk about my friend Sue who just happens to be the person interviewed in this because she’s been in the phase 3 clinical trial and is currently in the long-term follow-up study.  https://www.cbsnews.com/philadelphia/news/first-and-only-fda-approved-drug-to-treat-advanced-macular-degeneration/

AMD has 3 basic stages: early dry, intermediate dry, and advanced or late AMD. There are 2 types of advanced AMD: wet AMD and geographic atrophy which is the advanced stage of the dry form. There are 2 basic types of GA: outside the fovea (the center of the macula) and inside the fovea. For more information, here’s a good site. https://eyesonga.com/what-is-ga

How do I know if I have it?

It can only be diagnosed by testing done by an eye professional. Ask your eye specialist to tell you whether it is inside or outside of the fovea. In the early stages of GA, you might not be able to see any changes especially if it is outside the fovea.

Who is it NOT for?

It’s not for those with dry AMD at the early or intermediate stages. It’s not for those with other forms of macular degeneration such as Stargardt’s Disease or Myopic Macular Degeneration.

What if I have wet and GA?

In the clinical trials, they did not use the treatment for anyone with both GA and wet AMD in the eye they treated. For that reason, retinal specialists are discussing its use in this case and will make decisions for each individual.

What if I have GA and glaucoma?

If you have glaucoma and are considering Syfovre eye injections, it’s important to let your retinal specialist know about your condition. While it’s possible for people with glaucoma to receive these injections, they need to be monitored closely or have pre-treatment eye drops before their injections.

Eye injections can sometimes cause a temporary increase in eye pressure, which can be harmful to people with advanced glaucoma. This is why it’s important to take steps to prevent any increases in eye pressure before giving the injections. Your doctor may use anti-glaucoma eye drops to lower the pressure in your eyes before giving you the injection to help minimize any risk. Your condition will also be monitored more closely.

Is it only available in the US?

As of February 17, 2023, Syfovre received approval in the United States, according to the company’s press release. The European Medicines Agency is currently reviewing a marketing authorization application for Syfovre, with a decision expected in early 2024. Additionally, a marketing application for Syfovre has been submitted to Health Canada. If you live outside of the United States, Europe, or Canada, please check with your doctor to find out if Syfovre is available in your local market.https://investors.apellis.com/news-releases/news-release-details/fda-approves-syfovretm-pegcetacoplan-injection-first-and-only

What does it do? What does it not do?

It slows the progression of the disease which is measured by the size and rate of growth of what’s called a lesion which is an area of damage of the retina. The word ‘geographic’ comes from how the macula appears when an eye doctor looks at it: it’s like looking at a map where there are islands of damaged retinal cells – the lesion – in a sea of healthy ones.

The lesion starts outside the fovea – the center of the macula. The lesion causes you to have blind or blurry spots outside the very center of your vision. The lesions can progress to inside the fovea where you you will have one or more blind or blurry spots sometimes called scotomas.

It does not improve vision or restore any lost vision.

How well does it work?

Syfovre has been shown in studies to reduce the growth of geographic atrophy lesions more effectively than a sham injection. The treatment’s effects also increased over time.

A sham injection is a procedure used in clinical trials where everyone receives an injection, but some do not receive the drug being tested. The growth rate of the lesion in the treatment group is then compared to that in the sham group to evaluate the effectiveness of the drug.

For the actual results in numbers, you can find them here. You can ask your retinal specialist to help you understand them. https://syfovre.com/about-syfovre/what-is-syfovre/ and https://syfovreecp.com/oaks-and-derby-efficacy

How is it administered?

It is injected into the eye much like the treatments for wet AMD. The eye is disinfected, then a numbing agent is administered, then the injection is given with a very small needle. The eye is rinsed out, and you’d be given instructions about taking care of the eye at home.

It can be given every 25 to 60 days depending on the advice of your retinal specialist. The research showed that monthly treatments worked better than every two months. Also, the longer the treatment, the better the results.

To get the best results, you have to keep getting the treatments as your retinal specialist advises.

What are the possible side effects or risks?

You can find them all at https://syfovre.com/faqs/ and the patient brochure https://syfovre.com/wordpress/wp-content/themes/apellis/pdf/SYFOVRE-Patient-Brochure.pdf

Most common: floaters, eye discomfort, infection (can be treated), blood in the white of the eye (resolves over a period of time), wet AMD (can be treated)

Other possible side effects: eye infection, retinal detachment, temporary increased eye pressure after the injection

I keep hearing that it may cause my GA to become wet AMD. What’s the chance of that and what would happen if it did convert to wet AMD?

First, it’s your retinal specialist who can tell you more about the chance of this for you. Everyone is different so everyone’s risk of this will be different. For example, if you have wet in one eye but not the other, that non-wet eye has a higher risk of becoming wet than if you didn’t have wet AMD at all – even if you don’t have this treatment. In that case, your retinal specialist may not want to treat an eye with GA if you have wet in the other one. Everyone will be monitored closely for signs of this change and treatment for wet AMD will be started.

For the actual numbers, my friend Sue who was in the phase 3 clinical trial, is in the long-term follow-up study, and who was in the CBS News piece, wrote about that in this article ‘Pegcetacoplan Side Effect Hunting.’ https://maculardegeneration.net/living/pegcetacoplan-study and https://syfovreecp.com/safety/

She talks about the risk for those who have a higher risk of developing wet to begin with (above) vs those who do not. Remember, it’s your retinal specialist who is the only one who can evaluate YOUR risk. She talks about how she made her decision to get the treatment by evaluating the risks vs the benefits to her.

How do I decide if it’s worth it for me?

There are risks with anything. It comes down to evaluating the benefits vs the risks for you. It’s your retinal specialist who can best help you with this. Sue wrote ‘What Does Syfovre Mean For You?’ https://maculardegeneration.net/living/thoughts-on-syfovre

What will it cost? Will my insurance pay for it?

Please don’t freak out if you see the ‘cost’ of Syfovre as over $2000. That is the cash price and we don’t think anyone is going to pay that! But for some people, getting the cost covered or getting financial help will take time. This is how the process goes with all new drugs such as the newest treatment for wet AMD Vabysmo. All the cash prices for the wet AMD treatments are about this, except Avastin, but no one that we know of pays it because of insurance and financial programs.

Medicare has been the first insurance to pay with Medicare Advantage plans requiring pre-approval. Commercial insurances are working on it, but it takes time, just as it did with the new treatments for wet AMD. You can appeal to your insurance company which might speed things up.

What if my insurance does NOT pay for it, or I don’t have insurance?

There are several sources of financial help. Your retinal specialist should have someone to help you navigate this.

Apellis Pharmaceuticals, the company who makes it, has a program called Apellis Assist. They’ll help you work with your insurance company if necessary and if eligible, provide financial assistance. This might be the best first source.  https://syfovre.com/resources-for-you/apellisassist/

There’s another program called Good Days which has helped with costs for wet AMD treatments and is expected to help with this. You can check it our here. https://www.mygooddays.org

So this is the only treatment for geographic atrophy – my only option?

It is currently, but there’s a second treatment that completed the 3 phases of clinical trials and is waiting for FDA approval from Iveric Bio called Zimura. Approval is expected in August 2023. You can find out more about that here. https://investors.ivericbio.com/news-releases/news-release-details/iveric-bio-announces-fda-accepts-new-drug-application-and-grants

How do these 2 treatments (Syfovre and Zimura) compare? Should I wait?

You can find that here. You and  your retinal specialist can discuss whether it might be best for you to wait for this treatment.  https://clearsightcorner.com/articles/syfovre-first-treatment-approved-geographic-atrophy-amd

There are MORE treatments at various stages in the pipeline. This recent article (Feb 14 2023) is titled ‘2023: The year of geographic atrophy: A comprehensive look at 87 clinical programs for investigative treatments in retina.’ https://www.retina-specialist.com/article/2023-the-year-of-geographic-atrophy

Why is my retinal specialist not using it?

Some retinal specialists are conservative and are waiting for more information from the long-term study going on and the ‘real world’ data which means how well is working now with patients that it’s been approved.  Sometimes treatments go through clinical trials and are approved, but when they used with a winder population, problems can arise.

That’s a great question to ask your retinal specialist. You might find this article written by an authority in the field interesting as Charles Wykoff, MD, PhD, Director of Clinical Research, Retina Consultants of Texas, talks about how he is approaching it with his patients. https://www.hcplive.com/view/charles-wykoff-md-significance-pegcetacoplan-approval-geographic-atrophy?

Why aren’t researchers trying to stop the disease before it gets to an advanced stage?

That’s a great question especially since -85-90% of those with AMD have early or intermediate AMD. AMD is a complicated disease with many factors and no one cause. Therefore, researchers are working in many areas including the prevention of it and stopping or slowing it at the early and intermediate stages. In many complicated diseases, we see research in the advanced stages because that’s where there can be the most damage. That’s why we have had so many treatments for wet AMD for 20 years: most of the damage to central vision is from untreated wet AMD. It’s the same with advanced dry AMD: the disease process needs to be slowed to prevent central vision loss.

Here’s an article with 87 research studies for various stages and types of retinal disease. https://www.retina-specialist.com/article/2023-the-year-of-geographic-atrophy

Many of your questions will have to be answered by your eye specialist. It is a retinal specialist, a specially trained ophthalmologist, who would be giving the treatments.

This is the patient brochure with some of the answers to your questions. https://syfovre.com/wordpress/wp-content/themes/apellis/pdf/SYFOVRE-Patient-Brochure.pdf

Created April 20th, 2023 Linda Chernek Moore, light2sight5153@gmail.com

 

 

Catching Up – December 2020

Hi. It has been absolutely months since I wrote a page for this website. With COVID-19, my workload as a therapist has expanded. In addition to my day job, I was asked to do a side gig writing for a “health community.” Add to that my “doom scrolling” of online news outlets to check on the pandemic and election, and I have managed to let a few things slide.

As some of you know, Lin, my friend, editor, webmaster and purveyor of many good things, has had some serious health issues that are hopefully mostly behind her now. Her reasons for being m.i.a. from the website are much better than mine. [Lin/Linda here: thanks for the kind words, but we both have perfectly good reasons for not being here – they’re just different. ::smile::]

All by way of saying, sorry and making a pledge to do somewhat better. Not that we are going to present the quantity of material that we used to. Come the end of January, 2021, I will have been legally blind for FIVE years! How time flies when you are having “fun.” After that amount of time, I have adjusted. In some ways, being visually impaired has become old hat. I don’t have the angst I once had and my life is pretty routine. In other words, I can be really boring! And, yes, being visually impaired can be boring, too.

That does not mean, however, that everything is boring about my geographic atrophy. A few of you may know of my quest to some day regain my sight. Yep. Cockeyed optimist that I am, I have every intention of someday no longer being legally blind. The first step in my diabolical, master plan was to get into a clinical trial. That goal was reached about 18 months ago.

Since that time I have been going monthly for injections of a trial drug. The earlier results of the study suggested this drug, APL-2, slows down the rate of degeneration by about 30 to 40%. If things continue to progress at the rate they are now, it is not inconceivable that the first, clinically proven treatment for geographic atrophy will be on the market by 2022.

And it might not even be “my” drug that wins that race to be the first treatment for GA. The concept behind “my” drug – interfering with the complement cascade – is also the underlying concept behind other treatments in the pipeline. Those drug trials are doing very well also.

The second step of my diabolical plan was to get into a long term study that would hold me over until step three was ready. I am supposed to achieve inclusion in a long term study in the spring. The study is to determine how years of use of the drug affects my eyes. Will I develop side effects? Will the drug continue to work as well? Worse? Better?

I am willing and anxious to get into that study because it is a stepping stone to my next objective. That objective is a study that currently only exists in my feverish, little brain…and maybe the brains of a few researchers. That study will see how well transplanting RPE stem cells into eyes treated with the drug works.

After that? By that time I am speculating they will be ready to transplant photoreceptor cells and get them to connect to the optic nerve. Endgame. We see again. [Check out the Audacious Goals Initiative of the NIH NEI (National Institute of Health, National Eye Institute). That’s what they’re working toward.]

At least that is my diabolical plan. Step one will be completed and step two will start in the spring. I love it when a plan comes together.

If you are ready and able to join me and thousands of others as “lab rats”, if you are ready to become part of the solution, please volunteer for clinical trials research. Remember, this really is the best time in history to be going blind.

Written December 1st, 2020.

Bad News, Good News

OK, so I like listening to myself talk. I decided to write another page. Lin is going to love this but I kinda miss writing sometimes.

Anyway, if I am going to write more, you might want to give me some assignments. It keeps me from rambling on. Sort of a self-defense move on your part, and I am still a half decent researcher. You might get some good information in return.

Enuf of that.

This page is going to be bad news – good news. Bad news first. According to the August 1, 2019 Healio ophthalmology post, yellow glasses do nothing to improve night driving. In a study by Hwang et al, people wearing yellow lenses ran down just as many simulated pedestrians as the folks not wearing the glasses. Pooh.

The bottom line for this study was leave your money in your pocket and do not invest in yellow-lenses driving glasses.

The commercials target older drivers because we have a lot more trouble with night vision and night driving. We are also more affected by oncoming headlights. Yellow lenses did nothing for that problem either. Spend your money another way.

Bad news number next is this: Eylea injected in the non-wet eye does not help to prevent it from going wet. Again in Healio, this time July 27 of this year, it was reported that in a two year study trying to use Eylea prophylactically, it didn’t work. In other words, the percentage of the group who converted to neovascular AMD was just about the same whether they received the Eylea injections or not. It appears that using Eylea to try to keep an eye from converting to wet has no positive effect.

And one more time about that word “converting”. Remember all AMD starts out as “dry”. Geographic atrophy and “wet” or neovascular AMD are both just different end stages of the same disease. Therefore, LumiThera products and complement inhibitors like APL-2 may conceivably help slow the progression of the disease so that people won’t ever have to worry about converting. Cool.

Now in the interest of fair and balanced reporting, let’s look at two, positive reports. First of all, Healio ophthalmology reported in their July 28, 2019 post that they are making progress with the so called “patch”. An ultra thin piece of probably polymer with stem cell derived retinal pigment epithelial cells was slid through the subretinal space and positioned within the geographic atrophy lesion.

The big news here is the delivery method. Using the subretinal space means no vitrectomy and no opening up the center of the eye to possible infection. It is a procedure that could eventually be done on an outpatient basis. Also, the study proved they were able to put the patch where it was needed with very respectable accuracy. Good going.

And another quick review: RPEs do no seeing. RPEs are the “servant cells” for the photoreceptors that do do the seeing. Replacing RPEs is a great step because it puts the support system back on the job. Somehow you have to replace the photoreceptors.

Won’t they just grow back? Nope. Skin heals. Bones heal. Neurons do not heal and photoreceptors are neurons.

Never fear, however, because our superheroes, the researchers, are on the job. Last year, September, 2018 the Review of Ophthalmology reported the first regrowth of rod cells in mammals.

Exciting, yes, but don’t throw the party quite yet. The mammals were mice and they regrew rod cells. We primarily lack cone cells. Just the same, the researchers at Mt. Sinai in NYC are expanding the frontier and getting us ever closer to real cures for blindness.

How do you like that for good news?

Written August 5th, 2019

Next: WANNA SEE MY BOO BOO?

What is geographic atrophy?

Question: What is Geographic Atrophy?

Answer: There are stages of AMD: early dry (everyone starts at that stage even if not diagnosed them), intermediate AMD, and advanced AMD which is either advanced dry which is geographic atrophy (GA) and/or wet AMD. A few things to clarify:

  • Although it is rare, a person can have both wet and GA in the same eye.
  • Not everyone progresses to an advanced stage. 85-90% of all people with AMD have the early or intermediate dry form.
  • Although they all have the word ‘dry’ in them, progression is not necessarily from early to intermediate to advanced dry AMD. The progression CAN be from early dry to intermediate dry. Not everyone progresses beyond that.
  • It’s called ‘geographic’ because when the eye doctor looks at the retina, there are often what looks like ‘islands’ of damaged macular cells.

The Science of AMD

One of the best websites I’ve found is called The Science of AMD. 

If you scroll/move down the page, you’ll find a section about Geographic Atrophy. This site gives you the ability to hear the text (convert text to speech) if you select the speaker icon in the upper right of any section.

I recommend that you take the time to explore this site.

Other Resources

‘What is Geographic Atrophy?’

‘Geographic Atrophy’

Living With GA

Living With GA

My friend of 40+ years Sue has had geographic atrophy for over 6 years. That means she has a blind spot in each eye and is legally blind. She has always led an active life and that hasn’t changed any. At 69, she works as a psychologist, attends multiple exercise classes, kayaks, skis, rides her bike, walks her 2 boisterous dogs…you name it, she does it. The only thing that she does not do that she did ‘before’ is drive. That hasn’t stopped her from going places and doing things.

I’ll let her tell you about living with Geographic Atrophy: “I am not a victim. I have had geographic atrophy (dry age-related macular degeneration) for over 6 years but it has not destroyed my life. It has not destroyed me. Nothing is in ruins and I am not suffering.”

Her story continues at ‘Living With Geographic Atrophy.’

She was enrolled in the phase 3 clinical trial for a drug then called APL-2, now called Pegcetacoplan. At the end of that trial, she was enrolled in the long-term study of the drug which will show the safety and effectiveness of it over a 3 year period. You can read about that and also about her plan to find a stem cell clinical trial! ‘My Diabolical Plan: Stem Cell Transplant for Dry AMD.’

You can read about the early days of her journey on our site ‘My Macular Degeneration Journey/Journal.’ In 2019, she started to write for the site maculardegeneration.net where you can read her articles.


GO BACK TO FREQUENTLY ASKED QUESTIONS

Happy Anniversary to Us!

Hi! Happy Anniversary to us! It was February 2016 we started this crazy journey. For those of you who have been around since nearly the beginning, thank you for sticking around and putting up with us! For you who are newcomers, welcome! [Lin/Linda: To read Sue’s very first page, it’s In the Beginning.  At the bottom of the page, there’s a link to In the Beginning: Revisited where you can read about how her life has changed.]

I had a déjà vu experience at the beginning of the week. It was January 31, 2016, when I went skiing and came home to a drastic vision loss. It was January 29, 2019, I went back to the same ski slope, had the same sort of glorious day on the slopes and…came home to the same vision loss I had when I went. That was a bit of a relief! [To read about that day of skiing, it’s The Perfect Storm.]

I cannot tell you I am above engaging in a bit of superstitious thinking about such things. I was relieved when nothing happened.

Anyway. What does my vision look like now ? I have a fuzzy section pretty much in the middle of things. Anything I want to focus on is not all there. I recognize people across a room by body type or other clues. I can see faces well enough to identify them from about two feet.

If I am running the dogs at the dog park, they can disappear into my blind spot at maybe 20 yards or so. That is a guesstimate. I am definitely not able to drive. There were men working on the road the other day. I did not see them until we were maybe 30 yards away. Not enough distance to stop if I had been driving at the same speed.

Reading is done but is greatly curtailed as compared to my pre-AMD rates. I can read a couple of pages of standard font using eccentric viewing. I have a terrible time reading handwriting, especially handwriting of those I do not know. Heck, sometimes I have trouble reading my own handwriting! [To read Sue’s page describing how she uses eccentric viewing, it’s How She Sees What She Sees.]

I am typing this with a standard, 12 point font. I read it back to myself and find typos at a fair, not excellent, level. That is on my iPad. If I am writing reports on the standard laptop I use 28 point font. After the office staff puts it in standard form, I proof it using my CCTV.

I am able to do most of my life skills just fine…or as fine as I ever did. I have never been a housekeeper or a cook. If I don’t want to resort to using my toys, I sometimes have trouble reading directions. I have trouble plugging plugs into outlets and, at the beginning of the cold weather, zipping my jacket can be an event.

In other words, it is not awful. A few toys, a few tricks and a little help from my friends and I am making it. This is three years into being legally blind.

Again, a reminder: I have geographic atrophy. That is “the other” advanced age-related macular degeneration. Even though we dry folks make up something like 80% of the AMD population, we get short shrift.

Why? Well, we do not have the dramatic changes in vision or the severe damage the wet people can have. We also do not have any treatments. If any one knows the term AMD and he finds out I have it, he will almost always ask about “eye shots”. Afraid not, buddy. No treatment. No cure. This girl is dry.

I gotta go, but I refer you to some pages Lin is putting on the FB page. Not everyone gets wet AMD. Most of us actually have dry.

Do I think I will get wet AMD? Nope. None of my doctors think I will either. GA is my way.

Thanks again for being with us!

[By the way, February is also AMD and Low Vision Awareness Month. What are YOU doing to make more people aware of these two important topics?]

Written February 2nd, 2019

Next: READING AND WRITING

Sue on Assignment – Special Topics

When Sue announced that she was going to take a break from writing journal pages, she asked if anyone had any topics that they’d like her to research. It didn’t take long for ME to find several projects for her.  I’ve also gotten requests from readers.  If you have a topic, please post it in the comments or send it to me at light2sight5153@gmail.com. I can’t guarantee that she’ll take them all but we can try!

AREDS2 Study & Geographic Atrophy (2 pages)

Money for Assistive Technology (2 pages)

Non-genetic Causes of Macular Degeneration (2 pages)

Got Milk? Research on Calcified Eye Spots

How to Conduct an Experiment for Yourself

How She Sees What She Sees

Altitude and AMD (2 pages)

Be My Eyes

Coping Fatigue (3 pages; Coping Fatigue, It’s Not Your Fault, and Exhausted by Life?)

Mitochondria – Part 1 (2 pages)

Photobiomodulation

Why Read My Pages? My Answer

Independence

Independence

Getting Food to Come to You

Supplements

Resveratrol: Efficacy Not Yet Proven for AMD

CBD Oil: Safety and Efficacy Not Yet Proven for AMD

Bilberry: Safety & Efficacy of Supplement Form Not Supported by Research for AMD

Astaxanthin: Has Potential But Not Backed by Scientific Evidence for AMD

Linda on Assignment

Me and My Cocoons – 2 pages

Electronic Glasses for Low Vision – SeeBOOST

Headworn Low Vision Glasses and Goggles – 2 pages

 

More to come!

Have an idea for a page for Sue? Let me know at light2sight5153@gmail.com

Sue on Assignment: AREDS2 Study & Geographic Atrophy – Page 2

Hi! Back with trying to understand the last, three pages of the article “Progression In Geographic Atrophy in Age-related Macular Degeneration”.

It would appear they are having trouble figuring out exactly how these suspect genes are causing the problems they seem to be causing. Like watching a magic show: you know the magician has something to do with what is happening but you cannot seem to figure out how he is doing it!

They have come up with theories, of course. Some people think ARM2 encodes for a mitochondrial protein. Remember the mitochondria are the powerhouses of the cells. Others believe the ARM2 gene has something to do with our old friendly nemesis, the complement immune system.

While in some cases they believe it makes more sense to believe one mechanism is responsible for several lesion characteristics, in other cases they believe there are separate mechanisms at work. And to make matters even more complex, they are thinking the whole thing may work on the “Goldilocks principle.” In other words, certain genes may not necessarily be bad or good. Like the chairs and the porridge, they may have times they are “just right” and highly beneficial for the cell. Other times? Not so much.

In short? Damned if I know and, more importantly, damned if the researchers know either. However, they are hot on the trail of…something.

Right now at this stage of the game, they are collecting knowledge. It may be quite a while until they connect the dots with all of this stuff, but connect the dots, they will. Then we shall see what the picture is.

So, what did I get out of this article? A lot of questions. Like good investigators, right now the researchers are gathering data. The AREDS2 study has made available to researchers thousands of people with AMD. These people are being poked, prodded and scanned in the name of trying to understand the nature of the beast. No clue what they may find but the hypotheses being generated are intriguing. Which one do you think may prove to be right?

Written September 20th, 2018

Next: coming soon!

Go back to the list of “On Assignment” pages

Sue on Assignment: AREDS2 Study & Geographic Atrophy – Page 1

I am back on “special assignment”. Lin asked me to try to make heads or tails out of a very scientific article entitled “Progression of Geographic Atrophy in Age-Related Macular Degeneration.” It is report number 16 in the AREDS series.

Me thinks she has more confidence in me than I have, but let’s give it a shot. Just remember, social scientist here. My interpretations are always subject to errors.

The article starts with a description of Geographic Atrophy (GA). As many of us know, GA is defined as discrete areas of cell death (atrophy) that eventually grow and come together to form “continents” of damage in the macula. Part of the definition requires the damage to be such that someone examining the eye can see the blood vessels in the next layer (the choroid).

GA usually starts in the macula but generally does not immediately affect the fovea. The fovea is the “prime” area of the macula where the best seeing is actually done. However, as the disease progresses, the fovea is often also involved.

GA involves scotomata, Greek for darkness. Scotomata are those dark islands in our visual fields and result from the death of the cells in those locations.

I am going to skip the methods sections of this article and go right to results….believe me, it is better for everyone that way?.

Okay…because there is no treatment and no cure for GA, science is presently focusing on trying to slow this train down. Worry about actually stopping it once we buy some time. In order to slow it down, we need to know something about it.

The study first calculated how many of their early AMD subjects either went on to develop wet AMD or went on to develop GA. This gave them some idea what the progression of the disease looks like when nothing is done. Basically, they were learning about the natural history of the disease as well as establishing a baseline.

They did make some interesting, incidental discoveries. For example, noncentral lesions grow faster than central ones. Enlargement of lesions occurred more rapidly if the subject had GA in both eyes as opposed to one eye.

Those of us with certain genotypes may be in worse trouble than others. Those who have risk alleles (half a gene pair) on ARMS2, for example, are potentially destined for faster lesion growth. One CFI at-risk allele is another example. Unfortunately, there are several more.

So, on to discussion! What did they actually conclude? Vision loss from GA was steady over the five-year life of the study. Of those in whom there was no central involvement when they were first assessed, 60% developed central involvement within five years. Up to 30% of the eyes with GA developed neovascular AMD within those five years. This was especially true if the companion eye was “wet.” GA lesions may grow more slowly when they are small and when they are large and more rapidly when they are midsized.

There are some genotypes – once again the infamous ARMS2 – that seem to predict both the formation of lesions and their faster growth. If you don’t have those genes, take up smoking. Smoking continues to be found to be a risk factor for both GA presence and growth.

There are three, more pages to go on this thing but I am above my self-imposed word limit here. Also, it is just about nap time. Take this up again tomorrow!

Written September 20th, 2018

Next: Sue on Assignment: AREDS2 Study & Geographic Atrophy – Page 2

Go back to the list of “On Assignment” pages

Taking Some Time Off

This is the new normal. I ride transportation. I set up my portable CCTV. Fire up my computer with ZoomText and I go to work.

New clients get a mini-course in vision loss. “This is my CCTV. It is nothing more than a magnifier….I do not see faces. If I come into the waiting area and look confused, I cannot identify you. Tell me you are here.”

At the end of the day, I get a ride home. If I have a class, I get more rides. “My” people are incredible. Friends who give me regular rides have now started finding their own substitutes! I am cared for.

Bottom line here is this: the angst is gone. I have settled into my new normal. It is pretty much like the old normal in many ways. One of those ways? It is boring.

Because it has become the same old, same old, I have less to write about. Or should I say I have less of substance to write about. I am also no longer servicing those who are supposed to be my target audience.

Could I have imagined coming out the other side (or at least sort of. This ride is not over yet) and having this all become humdrum when I first lost my vision? Most probably not. I needed someone to understand where I was then. Being told it was not so bad was…shall we say irritating?

Which brings me to the point of this missive. At this time in my disease, things are ridiculously stable. Thank you, God! Thank you! Thank you! I am losing visual functioning at an “average” rate and that is either very slowly, slowly enough I can keep adapting easily or a combination. For now, I am pretty much plateaued. No angst and no new skills to learn. I am hoping this state stays forever…or at least until there is a cure and I can stop dealing with this mess.

The downside of things being stable, of course, is boring, chatty posts. Not relevant to where most of you are at all.

What I am proposing is a hiatus on regular posts. That may be two weeks, two months or two days! I really like to write! Lin will give me “assignments” on occasion. We’ll be revisiting early pages with updates.

Why don’t you give me assignments, too? Some of you have said you envy my still having enough vision to be able to do research. Take advantage of my having enough vision while I still have it. What would you like me to look at and pick apart?

Of course, many of you know I cannot shut up for long periods of time so I am practically guaranteed to pop up from time to time just because. I plan on being particularly active when (not if. I try to always be positive even though this quest is not going well right now) I get into a clinical trial….or if I have a significant loss in vision. Bite my tongue!

So, the question is: anything special you want me to write about? If not, I will try to take some time off.

I also have a request: who among you – especially those not far along this road – would like to write a few pages about his/her challenges and successes? Any takers? There have to be some frustrated authors among you. This is your chance.


Lin/Linda: this is the first of the “revisited” series: In the Beginning – Revisited.

Written September 9th, 2018

Next: SALVATION: VISION REHABILITATION REVISITED – 2018

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Lookout

This has been day 2 of the partial retirement adventure. I don’t count the weekend. I took the puppygirls to the dog park today. They had not been since they were baby puppies.

Even though there was only one other dog there, they had a great time. Smellfest-2018! They are anxious to go back soon

I got my loaner CCTV set up. The machine itself is not only large but the lowest magnification is HUGE. Better than nothing, though.

I also contacted Eschenbach about repairs. They are really tightening up. Not only no more loaner machines but also you need a credit card on file to get service. No shipping and handling costs paid either.

I got the puppygirls out to the pool this afternoon. Took a look at the pool deck and thought it would be a perfect way to describe geographic atrophy.

What am I talking about? Well, a few years back my husband painted the concrete around the pool. Apparently, his family has something against naked concrete. Anyway, a back injury and my complaining the pool deck looked like it had the mange made him decide not to try to paint again. Being fanciful, I see all sorts of ‘maps’ in the peeling paint. The way the paint is peeling is similar to how your macula is degenerating with geographic atrophy. Patches here and there.

Just a thought. A weird one, but a thought.

And thoughts lead to intelligence which leads to artificial intelligence which leads to a new topic. Tada!

Watch out Microsoft! Google is nipping at your heels!

The Macular Society ran a post about how Google is launching a new Android app. This app, called Lookout, hopes to give Microsoft’s Seeing AI a run for its money. Lookout is reported to be able to do for Android users what Seeing AI does for Apple users. Only they hope to do it better, of course.

Lookout is reported to give auditory cues to what the VIP may meet in his environment. It is designed to wear on a lanyard around the neck or in a shirt pocket.

Lookout is supposed to process things in the environment and share what it believes to be relevant. Sounds like a pretty big order to me!

Supposedly Lookout is programmed to learn what your tastes are and point out to you things that match those tastes. Great as long as you don’t want to experience anything new. It sort of sounds like if the Italian restaurant is next door to the Indian restaurant, Lookout will always steer you in the direction you usually go. In my case, I would be eating a lot of Italian even if I were interested in a culinary adventure. I might not even know the Indian restaurant is there.

Not sure that is the way it is going to work but that could be a problem.

Lookout is again said to have four modes: home, work, and play, scan and experimental. Scan is supposed to have a text to speech feature. Thus, it can read to you.

So, there you have it. Blow by blow of my second ‘work day’ off the job. But don’t worry, you’re going to get a reprieve. I work at the counseling center tomorrow!

Hi ho, hi ho, it’s off to work I go!

Written June 12th, 2018

Next: My New Luggage

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Different Paths

Good morning! I promised myself I would be productive today. Bedding stripped and in the washer, dishes in the dishwasher, a good pancake breakfast in my tummy, now if this webinar would load I might be able to DO something!

Speaking of pancakes, I was taught to flip the pancake when the bubbles are coming up in the middle. Visual cue. I burnt a couple today because I did not catch the little bubbles.

Short of singeing my nose on the griddle, how do I know when my pancake is ready to turn? Any low vision cooking suggestions? Inquiring minds want to know.

Moving right along, I happened upon a 2015 article in Drug Discovery and Development (DD&D) magazine. The article is a bit dated which actually goes to prove the point: research is advancing incredibly fast. Besides remarking that a Nobel laureate, Sir John Gurdon, called RPE replacement one of the most successful stem cell treatments in existence at that time, the article also talked about how regulatory agencies such as the Food and Drug Administration in the States and the Medicine and Health Care Products Regulatory Agency (MHRA) in the UK needed to get up to speed in the area of stem cells.

The article tells a cautionary tale about how the company Geron tried to get the first ES-cell clinical trial approved back in 1998. Their request and justification document ended up being 22,500 pages long and the approval came in – ready for this? – 2009! Good grief. The point is not that the FDA is an obstructionist organization. The point is stem cell science was an ostrich and they were used to dealing with elephants. Nothing fit their paradigms and they had no knowledge about it all.

Pete Coffey of the London Project to Cure Blindness told his own, mini horror story. He had two regulatory agencies to deal with. His wait was one year. Once again his ‘ostrich’ did not fit a system set up to deal with elephants. But kudos to the MHRA. They were not so hidebound that they could not adapt and modify.

The recent, controversial study published by Coffey, et al., was also discussed a bit in the DD&D article. After battling with the regulatory agencies and having his sponsor bail because of no fault of his own, Coffey expressed optimism that, essentially, the worst was over and his research would be moving on. In fact, there are rumors coming out of the UK that they may already be preparing for the next leg of this research. (Remember Lin has spies and secret agents everywhere.)

The article goes on to compare some of the different paths that stem cell treatment of AMD is following. Coffey used stem cells in people with wet AMD early in their disease progression. Work in the States is using stem cells in patients with geographic atrophy. In other words here we are treating after major damage has been done. Coffey and several others have started to be critical of the approach being taken in the US. Are we not at a point now we can pretty much trust the stem cell treatment? Must we wait until so late in disease progression? [Lin/Linda: click here for an article that talks about both the UK stem cell and the US stem cell trials results.]

The other comparison Coffey made was between simple injection of stem cells into the appropriate place and the use of a membrane to give structure to the cells. Coffey supports the latter approach….but that is the topic of an article Lin just sent me. Later!

Written April 7th, 2018

Next: The Patch

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Another $64,000 Question

Last time I discussed why we believe clinical trials and experimental evidence are so much better than anecdotal evidence. A reason that I did not include for truly appreciating scientifically derived data is a little thing called peer review.

Wikipedia describes peer review as the evaluation of work by one or more people of similar competence to the producers of the work. It is a form of self-regulation by the members of a professional or scientific community.

In order for peer review to occur, research has to have a certain level of transparency. Experimental procedures and results must be put out there for scrutiny. And that is where this page comes in.

Seekingalpha.com publishes stock market insights and financial analysis. They had a source at the 2018 Macular Society meeting where Apellis presented their findings on APL-2. Gleaning information from the PowerPoint slides, an author for seeingalpha.com developed the opinion APL-2 may not be all that Apellis has been cracking it up to be. [Lin/Linda: the full research results have not yet been published.  The slides from this meeting is the best source we have of the preliminary results.]

Why? Well, for one thing he said the rate of conversion from dry to wet AMD was 21%. Ouch. That is just about 1 in every 5 subjects. The average number of dry AMD patients who convert to wet is between 10 and 15% (although in the trial, there was only 1 person or 1% of those in the sham group who converted to wet; that’s slide 14). Rounding it off, that is about one patient in 8. There may be something in APL-2 causing an increased conversion rate.  [If you look at slide 16, they have some theories on why this happened.]

Another issue the author of the article had had to do with what should be the primary outcome measure. Apellis measured the rate of growth of the lesion. That slowed down. There was no one debating that. What was being questioned was why visual acuity was not used as the primary outcome measure. After all, if you can see, who cares how big the hole in your retina actually is?

Speaking as me, Sue, I would have assumed the size of the lesion is related to how well you see. Slower growth would mean better retention of vision, right ? Not necessarily. While Apellis reported a modest, positive difference between monthly treatment and sham at 12 months into the project, this positive difference did not persist. While at 12 months monthly treatment had lost 3.3 letters on the chart and sham had lost 4.4, by month 18 monthly treatment had lost 7.7 and sham had lost 6.4. What the hell????? The data suggested a greater loss of acuity with the treatment than without it.

Bottom line for the seekingalpha.com writer was this: don’t sink a lot of money into Apellis stock. What is the bottom line for us? How about a few questions answered? Or at least a few, clever theories? I would like to know why saved macula does not equal saved acuity. That seems counterintuitive.

Another question would be how did they get through phase 1, safety and tolerability, if subjects were coming out in worse shape (if they were; I don’t know.) ? Are the acuity data for phase 2 all that different?

And the $64,000 question: should anyone volunteer for their phase 3 trials?….that one you answer for yourself.

Lin/Linda here: I was curious as to what the exact difference is between a placebo group and a sham group.  Here it is: “Placebo and sham treatment are methods used in medical trials to help researchers determine the effectiveness of a drug or treatment. Placebos are inactive substances used to compare results with active substances. And in sham treatments, the doctor goes through the motions without actually performing the treatment.”

Written March 3rd, 2018 Continue reading “Another $64,000 Question”

Always a Silver Lining

by Vickie Hoecherl

I started noticing that highway overpasses had nonexistent “crimps” in them back in 2009, on a cross-country a road trip.

I already knew I had drusen, but the crimps were something new. I made an appointment with an eye doctor, who diagnosed me with macular degeneration. I was 56 years old and otherwise in good health.

Since then both of my eyes have progressed to advanced dry macular degeneration, also known as geographic atrophy. The crimped distortions have morphed into quarter-mile stretches of highway that simply disappear. Somehow, my brain fills in the blank spot with sky and empty road. (Disconcerting, to say the least.)

Because my geographic atrophy is central, it started messing with my vision fairly quickly. There’s a silver lining though. I became highly motivated to research any lifestyle change that could help me.

Along the way, I’ve discovered some things about myself.

I love throwing whole-foods together in new, creative ways. I take pictures of my creations before I eat them — compiling photos and recipes for a future website.

I’ve also learned that I actually CAN walk a 5K with running intervals every day. Now, on days I don’t run, I know that something is missing.

I’ve discovered that good and bad things tend to happen simultaneously. I lost my driver’s license, but now I cherish my walks outdoors even more. Some relationships have faltered, others are deeply strengthened. I have a deeper empathy for all handicapped people – my soul is larger now.

I never dreamed I would start to lose my vision. I was always the one who stopped to look at an exquisite flower or beautiful sunset. I still drink in beauty wherever I see it. But now I know that, sometimes, it’s a smile that lights up a room.


Vickie Hoecherl is 65 and lives in northern Michigan, where she managed a branch of her county’s library, part-time, for 15 years prior to her retirement two years ago.  Before moving to her current town, she  traveled with her husband in his Air Force career.  She also has worked six years as a journalist, writing and editing newspaper copy.  She loves the outdoors and she walks every day through whatever weather northern Michigan can bring. Each summer she travels even farther north for hikes along Lake Superior and for time at a north woods cabin.  She takes photographs (quite often of her lunch before she eats it) and is planning a website to share the nutrient-dense recipes she has developed.


Our Guest Authors: Their Stories

My New Career?

Not to belabor a point, but today did start as a rather poopy day. We had our first snow overnight. Just a couple of inches but it was the puppygirls’ first snow. My theory is this: when they went out and squatted to poop, they got cold, wet bottoms. Go inside and poop? No cold, wet bottom. Problem solved!

Of course, since my husband was first on scene and got to clean it up, he was NOT a happy camper. Not a good start to the day.

Then there was the snow itself. Getting to my eye appointment is a 90 mile trip one way. My husband does not like city driving and he does not like driving in the snow. Once more he was not a happy camper.

It is hard to explain to some people why we have been dutifully driving 90 miles one way, every six months just to be told my eyes are worse. “Please pay your copay.” For those kind of results, I could go five miles down the road!

However, it is all part of a master plan. I have real problems with defeat. Real problem being told there is no answer. If you cannot supply me with an answer, I will find someone who can!

So here I am, running to see Regillo every six months for the past two years….and I think I am getting closer!

First the good news/bad news. Or, in this case, bad news/good news. Although I find it hard to believe, I have fallen down below 20/400 acuity. Bizarre because I don’t feel blind and don’t think I function as a blind woman.. After all, I got the “you don’t look blind!” routine just last month.

However, the good news on that is my vision is now so bad, I can satisfy the truly awful vision requirement for the Astellas’ study that is supposed to launch in March! Regillo referred me again. Is this time four or time five? I have sincerely lost count. [Lin/Linda: I put the details to that study in Sue’s page The Waiting Game.]

He has also put me on the list for APL-2 which is supposed to go into phase 3 clinicals sometime in 2018. He started to offer me “something else” when we were talking about lamp stuff (which is apparently very dead in the water) and I surprised him by knowing exactly where he was going. Working on being memorable. I want my name at the top of the list. [Lin/Linda: Sue wrote about APL-2 in her page My Friend in Manila?]

Also thinking I may be getting closer because I got a new test today. They ran me on the autofluorescence test. This test uses a very bright light. When the image is examined, the areas of the macula that are already dead are black and the areas that are in distress shine. My eye probably lit up like a Christmas tree. If that gets me into a study and gets this stuff stopped? Good.

So, that is where we are. I got a new test. I chose to be hopeful it means they want more information for my new career as a lab rat! Regillo generally seemed positive. Maybe I will be going to Philadelphia.

Written Dec. 14th, 2017 Continue reading “My New Career?”

Sue’s Best Pages – Part 1

If you are new to our website, you might have looked at the LONG list of Sue’s pages and felt overwhelmed.   I hope this series of “Sue’s Best Pages” will help you to navigate through some of them.  We hope you will eventually read them all.

Spoiler Alert – why should you read these pages?

After a year of learning how to deal with her visual impairment both physically and emotionally, Sue has a rather ‘normal for her’ life: At age 64 and with advanced AMD geographic atrophy, she works several jobs, attends regular exercise classes, rides her bike safely, travels, walks her dog, kayaks, attends social events with her friends.   We are not suggesting that reading her journal will ensure you the same results but we hope that Sue’s Journal of Her Journey will be educational and inspirational.

For the newly diagnosed
  • You need to start In The Beginning.  Follow the sequence of pages with the links that are at the bottom of each page.  The first 13 were written in the early days of this journal.
  • Page 13 “A Human Doing” is where Sue starts to talk about her experiences with Pennsylvania’s Office of Vocational Rehabilitation’s Bureau of Blindness and Visual Services.  Because she wanted to continue to work, they were instrumental in getting her the assistive devices and training she needed to do that.
  • Of course, we hope you continue to read from there.  If not, please continue with the next section.
Pages highly recommended by our readers

We ask readers to rate the pages.  I’ve taken the ratings and comments to select these pages.

How She Does What She Does

Sue was 62 when her vision deteriorated so quickly that she had to stop working and driving. She could have started early retirement but she is not the ‘retiring’ type. ::smile:: She contacted Pennsylvania’s Office of Vocational Rehabilitation’s (OVR) Bureau of Blindness and Visual Services (BBVS).  The services she received included low vision, technology, orientation & mobility and rehabilitation.   Counselors for each of these services came to her home and workplace to deliver assistive technology, software and training. There was a one-time co-pay based on income. Some people pay nothing. Sue paid a small fraction of the true cost of the services, software and devices.

166.  A Day in the Life which covers the time she is not working.

288. A Day in the Life: Work Day

Next:  Part 2 Dealing with the Emotional Reaction to Vision Loss

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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”

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”

Slogging Through Again

I am waiting for my ride to go hot air ballooning and working on deciphering an article Lin sent me. Once again the question is how much loss can we expect from dry AMD, especially geographic atrophy? Regillo told me 60 degrees of arc loss would be extreme but my local retinologist said some people in their 90s can have GA encompassing the entire retina. Ouch.  [Lin/Linda: Sue wrote about ‘degrees of arc’ in her page Love Wikipedia.]

So, here be me again, slogging through another article I about half understand. Want to slog along? I would appreciate the company!

The article is entitled Clinical Endpoints for the Study of Geographic Atrophy Secondary to Age-Related Macular Degeneration published October, 2016. You there in the home audience feel free to download it and play along!

First of all, I latched on the statement (paraphrasing) “drusen may not result in actual visual acuity loss but the effects of having drusen can be seen in functional deficits very early in the disease process”. What functional deficits?

A 2008 paper by Feng Qiu and Susan Leat found people with very early AMD have loss of “low spatial frequency static contrast sensitivity”. Yippee. Once more down the rabbit hole. It appears – according to the appendix of Emergent Techniques for Assessment of Visual Performance – spatial contrast sensitivity has to do with lighting, the place on the retina where the image is falling and something called field size as well as time factors and the orientation of the image.

Boiled down it has something to do with how sensitive we are to variations in the data our eyes are gathering. I think. Don’t hold me to it. Just know that 20/20 vision with drusen might not be as perfect as we might think.

We talked about reduced dark adaptation before and this is also a problem in early AMD. Apparently there are several effects early drusen have that have nothing to do with acuity.

The next thing I had to look up – in the same paragraph, mind you! – was information that might help me understand a statement suggesting advancement to GA from early AMD may in part depend upon the presence of “reticular pseudodrusen”. So now we have drusen impersonators????

According to Association of Pseudodrusen and Early Onset Drusen by De Bats, Wolff et al (doesn’t that team sound perfect for the Halloween season?) pseudodrusen form on top of the RPEs and not below them as do ‘real’ drusen. There seems to be a connection between having ‘eye poop’ aka drusen on top of the RPEs and early and rapid develop of advanced AMD.

And the above was all in one paragraph! I may be a very long time in deciphering this baby.

So what I have discovered so far is this: visual acuity does not tell the whole story about functional vision loss when it comes to early AMD. If you have drusen be aware your contrast sensitivity and dark adaptation are probably already compromised. Secondly, pseudodrusen, which is eye poop on top of the RPEs, can predict a more rapid and earlier progression to GA.

Have I found a thing about GA outside of the macula? Not yet, but I am still reading! Talk at ya later!

written October 7th, 2o17 Continue reading “Slogging Through Again”

My Friend in Manila?

Happy Tuesday! Waiting for the van to go to school. Yesterday I was picked up at 6:56 to ride 9 miles and be there by 8:30. Dare I say I was not pleased? I just keep turning my mind towards acceptance (DBT alert!).

This is the way it is in my life now and I need to accept such nonsense if I am going to get where I need to go.

Today is day 61 of “your dog is dying.” We took a nearly 40 minute walk yesterday. Pretty active ‘dead’ dog. One day at a time.

I continue to monitor for information on lampalizumab. As of yesterday, September 11, all the news was still financial, but not quite as doom and gloom-ish for Hoffman-La Roche. Just sit tight on that one.

Apellis is drumming up excitement for their geographic atrophy treatment, APL-2. We talked about this before. APL-2 decreased the rate of atrophy growth 29% as compared to sham when injected monthly and 20% when injected every other month. In the second 6 months of the trial the reduction was 47% in the monthly injection group. APL-2 now appears to be the ‘show’ to watch as they go into phase 3 clinicals.

Philip Rosenfeld wrote a short blurb for healio.com. His disclosure statement said he has investments in Apellis. Either he is talking up the product, is truly sincere, or putting his money where his mouth is. In any case, Rosenfeld remarked APL-2 worked across a genetically diverse population sample. There were no stars and no non-responders.

Unfortunately, Rosenfeld also remarked that there is more of a chance of dry AMD developing into wet AMD when APL-2 is used. His opinion was it would have happened anyway in the eyes that became wet, but that will require more research.

We will keep an eye on APL-2.

10 hours later: The van came at 8:01 and had two people going to the local hospital already on it. Late for work? You could say that. Once more the shortcomings of transportation here are giving me fits.

Next, this has nothing to do with eyes but it happened to me today and I do want to mention it. I got an email from a ‘friend’ asking for a ‘favor’. Since my friend lives in Florida I was thinking it was hurricane related.

Turns out it was a scammer ‘phishing’ for money. My ‘friend’ was stuck in Manila and needed $2000. Yeah, right. I asked a couple of questions, obscure stuff that only members of the group I hung with in my 20s and 30s would know, and that was the end of the communication. Maybe my real friend was not stuck in Manila at all!

It appears many people in the world think of Americans as rich and gullible. I am definitely not the first and I try hard not to be the second. I assume it is the same for you.

If your ‘grandson’ emails or calls for ‘bail money’ from Tijuana, be sure to ask a few, HARD, identifying questions. Something that never got online. If the ‘IRS’ or the phone company or gas company or whatever calls and gives you a phone number to call with your credit card number, have someone look up the number independently and call that number to inquire.

Just another public service announcement.

Will check in again later!

written September 11th, 2017 Continue reading “My Friend in Manila?”

Another Potential Treatment

The more I look into this, the more overwhelming it seems! I am glad I never tried to be a geneticist!

What am I talking about? The latest information on treatment for geographic atrophy suggests there is another gene being targeted. The gene is C3.

Remember how I have been saying Age-Related Macular Degeneration is looking not like one disease but like a family of diseases? When I followed up with some background research on complement factor C3, I found a list of – get this – over 3 dozen different SNPs that preliminary evidence suggests have a role in causing AMD. Remember SNPs or ‘snips’ are genetic coding errors. Some are beneficial. Some are neutral and some can really screw thing up.

Anyway, it appears there are literally dozens – if not more – of ways we can be ‘wrong’ to get AMD. Right now that means they are working on finding dozens of ways to intervene. May be a panacea sometime in the future, but right now they are nibbling at the problem a piece at a time.

And the piece they are nibbling on now is C3. According to a short article by FierceBiotech, Apellis has finished phase 2 – the proof of concept phase – trials with intravitreal injections of a drug they are calling APL-2. Later it will get a trendy brand name but for now look for APL-2.

BusinessWire identifies Apellis as a company “developing a platform of novel therapeutic compounds for the treatment of autoimmune diseases” so I guess people are coming around to see AMD as an autoimmune disease. APL-2 is described as a complement factor C inhibitor. It “binds specifically to C3 and C3b, effectively blocking all three pathways of complement activation (classic, lectin and alternative).” That sort of sounds like it is suppressing ‘friendly fire’ sooner in the process and may be closer to the ‘one treatment fits all’ that we would like to see. Not anywhere near there, but closer.

The results were very promising. At 12 months they showed a 29% reduction in growth as compared to sham.

But the weird – and great! – thing that happened was this: during the second six months of the study, the reduction rate was 47%! For some reason, the effects of the treatment appeared to be cumulative. Pretty cool.

Now I am not sure what type of genotype you have to have to profit from treatment with APL-2. The researchers are not sure at this point either. They decided to do some searching for genetic markers. Being the suspicious sort, I am wondering if they had star responders and non-responders just like they did with lampalizumab. Would make sense. Why do genetic testing on an ‘n’ of 246 people if you don’t have to? It’s expensive.

And speaking about money, there is a lot of money to be made with this drug. Apellis wants to get APL-2 to market quickly so it can compete with eculizumab, a treatment for PNH, a blood disease. (Apparently PNH is also related to complement factor C). Their competition, Soliris, was predicted to bring in more than $3 billion in 2017. Sometimes a little greed is a good thing! $3 billion can really motivated people.

So, there you go. It seems they have found one more way to save some of the sight of some of the people some of the time. Number two potential treatment for our ‘untreatable’ disease. The wall is coming down a brick at a time. There is hope.


Here’s another article about APL-2 that says “APL-2, a complement C3 inhibitor, has met its primary endpoint in its phase 2 clinical trial, reducing the rate of geographic atrophy (GA) associated with age-related macular degeneration (AMD).”

written August 27th, 2017 Continue reading “Another Potential Treatment”

Geez, It’s Dark in Here!

Back again. I don’t want to scrub the floor or score a test, soooo….a page!?

Still checking out short blurbs from Modern Retina. Rosenfeld reported that low-luminance visual acuity deficits are predictive of the rate of geographic atrophy (GA) progression. Low-luminance visual acuity is basically night vision.

Following up on this I discovered that back in 2008 Janet Sunness found GA patients who reported poor night vision were much more likely to go legally blind than their GA peers who could see better at night. These people made up the quarter of their GA patients (visual acuity of 20/50 or better) who became legally blind within four years.

I believe them but still have a couple of questions. Recovery time from being ‘blinded’ by bright light is forever for me. Leave me there and come back in an hour.

Night vision is not bad. I prefer to walk without a flashlight because I see better to navigate. How can that be considering I am one of those who became legally blind?

The study measured night vision by seeing how much could be read in low light conditions. Reading in low light, I am not so good. Maybe that is the difference.

Anyway, if you cannot afford a lot of fancy testing, seeing how much you can read at dusk may give you some idea of how bad things are going to get. Just what we want to know; right? How bad things can really be.

And in other news, inflammation remains a target for the AMD researchers. Lampalizumab, aka ‘lamp stuff’, blocks complementary factor D to help control the alternative complement pathway (that thing again!) and reduce retinal inflammation. ‘Lamp stuff’ is said to work with carriers of the complement factor I at risk allele. Considering​ Regillo wants to start poking needles in my eyes come 2018, I cannot help but wonder if I actually have that gene. I would hate to be poked in the eye every month to no good end.

Maybe I would rather use POT. ?That’s POT 4. POT4, aka APL 2, blocks all three pathways of complement action at the same time. They are looking to develop an intravitreous shot that would be very long-term. None of this four to six weeks business.

And talking about shots, I just lost the article somewhere in this mess (not domestic goddess material; remember?) but I also read a short article taking about a new, medication delivery system they are working on in the UK. This team has been working on developing a little, bitty molecule that can permeate the layers of the eye and deliver medication to the retina through daily eye drops and not monthly shots. Not only will the people getting the shots approve, but the NHS (National Health Service) will approve because it will cut the number of office visits way down. Save money. Ka ching! [Lin/Linda: never fear, I found the article, click here.]

So there you have a review of some of the articles I pulled off of Modern Retina. They have lots in the works. Some of it is promising and some proves not to be, but they are zeroing in on treatments (plural because with a condition caused by multiple genes I believe there will be multiple avenues of attack). We are getting closer to answers. There is hope. Continue reading “Geez, It’s Dark in Here!”

Uneventful Trip

Just came back from Walmart. When I checked the early days page Lin had published for today it was my first trip to Walmart as a visually impaired person. I would say what a coincidence but this girl has been known to ‘live’ at Walmart so it really was not.

Anyway today the trip to Walmart was….totally uneventful. Fine. No issues. I tell you this because in my cockeyed optImist (yes, there’s an upper case “I” in the middle), Pollyanna way I want to reinforce the concept there is hope. Yes, I have geographic atrophy with no scarring – just ‘no’ macular; my ‘divot’ just keeps getting bigger. And yes, I have no clue what it is like to be you in your situation.

However, for the great majority of us things can be OK with adaptations and the learning of skills.

I cannot drive myself to the store. My husband now parks near a cart corral. He makes sure I know we are down the line from the garden center or bank sign or whatever and then he turns me loose. I generally find my way back without incident. Do I wander around lost sometimes? Sure do. It is a matter of my not paying attention in the first place. I did that when I was fully sighted.

Absent mindedness is not a side effect of vision loss!

In the store I am using eccentric viewing…a lot. Although I carry my toys just in case, I seldom get them out. I have learned to use my peripheral vision and I am pretty good at finding things I need…and things I don’t need but really want. Got (another) cute pair of yoga pants and (another) cute scarf today.

There are times I have to be more careful and really LOOK. For example, I almost picked up hot sausage instead of mild today. If there are several varieties of something and the packaging is very similar you need to double-check. When you don’t drive often things don’t get returned. I have a chili potpie in the freezer that I could have sworn was beef. Been there for weeks. (Perhaps this is an opportunity to expand my horizons?)

I use a lot of habit learning. The credit card machine is now easy. That is habit training. I pretty much know what comes up next. Press the same buttons all of the time.

And if I don’t know or cannot see it, I ask. Sometimes I admit I am visually impaired. Those are generally the times when I know a full sighted person would have been able to figure it out and I don’t want to look like an idiot. Other times I just don’t bother to ask.

Nobody thinks the less of you if you cannot find something like the honey. Fully sighted people ask questions like that, too!

So there you go. One more page about my uneventful life. Stay tuned. Next I might write about watching paint dry!

Written May 28th, 2017

Continue reading “Uneventful Trip”

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”

Hodge Podge

This may end up as another chatty, hodge podge affair. There is really nothing major happening and in the world of progressive eye disease nothing major happening is a good thing!

So, actually, I guess that is my first offering here. Those of you who have recently received your diagnosis or have had a crisis and are really distressed – it is not all drama and disease focus for the rest of your life.

You adjust and other things take center stage. That is not only normal but it is a good thing.

Second offering is something I picked up last month at the support group. When I said dry AMD is the base disease, they looked at me as if I had three heads. What I meant – and what they had not gleaned. Why won’t people do their research! Or minimally ask questions? – is that even though the shots have stopped the neovascularization, the growth of new blood vessel that lead to a bleed, you still have the underlying cause of the problem. The cause is regular, old, dry AMD.

This is why, even though you think the stuff we publish on dry AMD does not relate to you, it does.

Wet AMD is one type of end stage AMD and geographic atrophy is the other. Stopping the bleeding does not eliminate the underlying disease. It just eliminates the symptom.

Which brought me to another thought. I have never seen anything that says if an eye prevented from going wet will go to geographic atrophy. Hmmmmm…..

Nuts! More to worry about. Kaszubski et al in Geographic Atrophy and Choroidal Neovascularization in the Same Eye: A Review stated there are people who can have both forms at the same time. Geographic Atrophy generally happens first. (That part is bad news for me although I am under the impression that for me there is very little left to ‘save’ by building new blood vessels.)

To follow the question posed above, though, they also say there is some evidence anti-VEGF shots can increase the chances of GA development.

While that is bad news for you getting the shots it does NOT mean to stop your shots. No shot and you will bleed. Bleeds lead to scarring and certain vision loss now. GA is slow and lead to vision loss later. Given a choice, battle the bleeds and worry about the atrophy later.

End of lecture.

Other than that, in real time Memorial Day approaches and I am thinking summer. Although I know there is ‘no rushing city hall’ (to paraphrase another old chestnut), I started looking up Astellas and Robert Lanza again. Just to see what the dear boy is up to. I have been hoping to get to Philly and the clinical trials this summer. It would be perfect timing for me but I am not sure about the Astellas Institute of Regenerative Medicine (AIRM). They will need to give Wills the go ahead to start one of ‘my’ clinical trials before anything happens for me.

Astellas is gearing up for something, though. Something big. A couple of years back they bought OCATA for $379 million. Now they are on a hiring binge and are looking for a bigger location in or near Marlborough, Mass.

In the business articles I read Lanza purposely hyped the work they are doing on AMD. I am assuming that is still their big thrust. (That is even though AIRM is in a variety of areas of regenerative medicine and Lanza himself is intellectually all over the place, including developing a theory of the Universe!)

Anyway, seeing this big a build-up with lots of business chatter tells me something is going to happen. Just hope it is in the trial I have volunteered for. My eyes and I are not getting any younger! Continue reading “Hodge Podge”

Hindsight is 20/20

Good evening! How are you all?

Lin has noticed I seem to have written soooo many pages they are overwhelming and confusing some people. She feels this is particularly true for some of the newbies who probably feel like they have walked in on the (boring and confusing) middle of a movie. [Lin/Linda: to be clear, those are Sue’s words! ::grin::]

Understood. Some of you are back in the shock and doom phrase and I am talking about getting newspapers on your phones and other trivial matters. Who wants to hear about that sort of thing while your world is unraveling?

In the interest of pointing you towards something that might actually be helpful, Lin is republishing some earlier pages for your attention and discussion. And I – always helpful – am going to add to the confusion by writing another page!?

This page will have a catchy title thanks to Lin, but right now I am going to call it “What I know now that I wish I had known a year and a half ago”.

First, you are not going everything black and dark blind.

It is not good but neither is it quite that bad. You are losing central vision. Things will not be good for anywhere from about 15 to 60 degrees of arc. Since normal visual fields are 170 or so degrees of arc, you have the potential to lose about a third of your vision. Not anything to cheer about but better than 100%.

You may not be doomed to progress to end stage AMD.

About 15% of patients become ‘wet’. About 15% progress to geographic atrophy. That means you – starting out with drusen and a diagnosis of early AMD – have a 85% chance of dodging the proverbial bullet for end stage AMD. You may very well not get as bad as I am and a year and a half after my second eye went to hell, I am still functional. [Lin/Linda: a person can have both wet AMD and geographic atrophy in the same eye.  I don’t what that does to the %, if anything.]

You did not cause this.

Yes, AMD is caused but it was not caused by anything you did or did not do. The causes are in your genes. This is a heritable disease. There are dozens if not hundreds of genes that are being investigated to try to figure out how AMD is created. It appears AMD may just be the result of a genetic ‘perfect storm’ and there is no one to blame.

There may come a time you are seeing things.

I saw some odd stuff when my brain was working overtime to assign meaning to the faulty images my eyes were sending it. You are not psychotic (I hope you are not psychotic). This is Charles Bonnet Syndrome. When your brain gives up trying to assign meaning to false signals you will stop seeing weird ‘stuff’. In the meantime, enjoy the fantasy.

Point number last: There is an amazing amount of hope for treatment and eventually a cure for AMD.

Research is going on everyday. New discoveries are announced with regularity. The medical community is hot on the trail of something that will arrest the progression and may even reverse this disease. All we have to do is hold on.

OK. Those were my biggie when I first lost my second eye. What are you worried about? Please share and we can discuss it. Continue reading “Hindsight is 20/20”