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What's New in iOS 8 Accessibility Part 1: Scott Davert, AppleVis Editorial Team

Scott Davert head shot

Guest blogger Scott Davert, M.A., VRT, is an AppleVis Editorial Team Member and a regional representative for the Region 8 Rocky Mountain area with the Helen Keller National Center for Deaf-Blind Youths and Adults.

Most recently, Scott compiled his personal picks for book-reading apps and iDevice apps that are user-friendly and accessible to braille, and also speech, users.

According to Scott, "As a power user of braille devices on iOS, it's very liberating to me, as a deaf-blind person, to be able to take full advantage of the technology we have in our society today. Just a decade ago, my access to resources was much more limited if braille was my only means of accessing the world. Today, with the help of technology, I can be just as well-informed about what's going on around me as my sighted and hearing counterparts."

This week, Scott is reviewing the new iOS 8 release from Apple, with an emphasis on accessibility features for individuals who are blind, deaf-blind, or have low vision. iOS is Apple's mobile operating system, or OS. Originally developed for the iPhone, it has since been extended to support other Apple devices, such as the iPod touch and iPad. In June 2010, Apple rebranded the iPhone OS as simply iOS.

About the iOS 8 Release

It's fall, which means that it's time for another iOS update for your iDevices – that is, if you are using an iPhone 4S or later, iPad 2 or later, or iPod Touch fifth generation or later. I, along with several AppleVis editorial team members, have been working with the beta versions of iOS 8 since its first build was released to developers in June.

This year, Apple is introducing many new mainstream features, including the ability to share purchased items with family members on joint accounts with the iTunes and App Store; further harmonization of iOS and OS X; interactive notifications; and WiFi calling. Since many mainstream sources will be covering these features in great detail, my review will focus on changes in accessibility.

Siri Updates

Hey, Siri!

If you've ever wanted to use Siri hands-free, you now have that ability in iOS 8. If you go into the settings for Siri (Settings > General > Siri), you will now find an option called Voice Activation. Turn this on, and you can talk to Siri without ever having to touch your phone. Since this feature is a tremendous battery drain, it is only active when your iDevice is plugged in to a power source; therefore, be sure to plug your phone in before you start talking.

Name That Song

Siri can now listen to a song that you're playing and tell you what it is. Just ask "What song is this?" and Siri will listen for a few seconds and then try to figure it out. If Siri can identify the song, it will tell you the name and artist – and then offer to let you buy the song on iTunes.

This is great for the times you're listening to a song on radio services or stations that don't provide accessible song information to listeners who are blind. It can also come in handy when you hear a song in a public place, such as a store or restaurant, that you can't identify.

VoiceOver Changes

I'll Take a New Voice, Alex

That's right – if you're running the iPhone 5S or newer (sorry, the 5C doesn't offer this), the iPad Air or newer, or the iPad mini with retina display or newer, you can now use Alex (from Mac OS X) as your default voice for VoiceOver.

This is a voice many users of the Mac have grown used to over the years, and many users will no doubt be happy to have it residing on their iDevices. This is also going to be a welcome addition for users who have both vision and hearing loss – with hearing loss in the higher frequencies, which makes understanding female voices more difficult.

You can find and download the Alex voice, which is not on by default, by going into Settings > General > Accessibility > VoiceOver > Speech > Dialect. Under the U.S. English heading, you will find the option to download and then use the Alex voice. Be sure you have plenty of space available, as this speech synthesizer weighs in at a hefty 900 megabytes. Also, it appears that after downloading the Alex voice for the first time, you must restart VoiceOver before using it.

And the Possibilities Keep Growing!

iOS 7 enabled you to download multiple enhanced-quality voices for both multi-language support and for access to other dialects or languages like English and Spanish. With iOS 8, you can now download enhanced-quality versions of voices on the fly [i.e., without reloading the application] if you are connected to WiFi.

Alex voice users can even add a second instance of U.S English as a dialect, which will give you the familiar Samantha voice which you can then switch to on the fly if Language is enabled in your rotor settings.

All of These Voices are Taking Up So Much Space – I Need a Disk Diet!

If you find that too many of the enhanced-quality voices are installed on your device, you can remove the ones you are not currently using. Once you have multiple voices added to your device, go to Settings > General > Accessibility > VoiceOver > Speech and you will find an edit button. There is a delete option next to each voice. Just double-tap, confirm your choice, and the voice will be removed, creating more space for other stuff.

Keep it Cranked!

A new rotor item, Audio Ducking, is available in iOS 8. No, activating this rotor option will not make VoiceOver sound like Donald Duck, but it will let you toggle Audio Ducking.

What is Audio Ducking, you ask? Audio Ducking is when iOS decreases the volume on whatever other audio is playing when VoiceOver is speaking. It has done this for quite some time, but now you can disable this feature if desired.

You can accomplish this by turning your rotor to Audio Ducking and flicking up or down with one finger. If Audio Ducking isn't in your rotor, head over to Settings> General > Accessibility > VoiceOver > Rotor, and then select the Audio Ducking option. Once selected, Audio Ducking will always be available in your rotor.

Is That the End?

Another minor – but welcome – change is that when you have reached the bottom of a page in, for example, Settings, VoiceOver will now announce "footer," quickly letting you know that you have reached the bottom of the current screen.

However, it is a bit sporadic, in the sense that VoiceOver now sometimes announces "footer" with certain blocks of text in Settings when there is more information beyond that point. This could be confusing to a new user, as the user may think he or she is at the end of a screen when they actually are not.

VoiceOver, No Longer Interrupted…Sort Of

Prior to iOS 8, when you were reading text (such as an e-mail or audiobook) using the Read All gesture, VoiceOver would interrupt whatever it was you were doing to let you know of notifications such as Twitter replies, Dice World rolls, and breaking news. This no longer happens, with the exception of text messages. So read away – just make sure no one texts you at the same time.

More E-Mail, More Options

In the Mail app, the Custom Actions option in the Rotor setting has been modified. Previously, when you were on a thread of messages in prior versions of iOS, Trash/Archive and More were your only options. Now, added to the list of custom actions and not requiring the selection of the More menu are Flag, Mark-as-Read/Unread, Archive, and – yes -- More.

The More menu now consists of Reply-All, Forward, Flag, Mark-as-Read/Unread, Move to Junk, Move Message, and Notify Me. The Notify Me option allows you to get push notifications when there is a reply to a specific message thread.


In addition to standard and touch typing for touchscreen input, there is now a new option called Direct Touch Typing. If you are in a text field and move to the Typing rotor option, you will still find the Standard Typing and touch Typing selections available.

Direct Touch Typing is similar to Standard Typing in that you can find a key with one finger, then tap another finger on the screen to enter the character. The difference with Direct Touch Typing is that if you touch a key and immediately lift your finger, that character is entered. If you're extremely confident in your ability to locate the key you want on your first try, Direct Touch Typing is for you.

You can also find the key you want by dragging a finger around the onscreen keyboard, then touching the same spot quickly to type that key. Typing feedback is, as with the other typing modes, based on the verbosity settings you have set in Settings > General > Accessibility > VoiceOver > Typing Feedback > Software Keyboards.

The Braille is Everywhere

The letter z represented in braille

Another new feature that has been added in to the rotor in iOS 8 is a built-in braille keyboard. This is similar to what users of the mBraille app have grown accustomed to, but without many of mBraille's advanced editing or shortcut features. However, this braille entry is now a universal keyboard option and works in any text field. You'll find it as a new rotor selection called Braille Screen Input.

Like the Handwriting mode that was introduced in iOS 7, you simply turn the rotor to the Braille option and begin typing in braille on the touchscreen. If you aren't finding this option in your rotor, you will need to enable it as described above in the Audio Ducking section.

Getting a Feel for Using the Braille Keyboard

  • To orient yourself to the braille dots, press and hold down a finger on the touchscreen until you hear two tones and the phrase "entering explore mode." Drag your finger around the screen to discover where the dots are. To exit this mode, simply lift your finger off the screen.
  • There are two keyboard layout modes: Tabletop and Screen Away. If you are not happy with the layout of your keyboard, try turning the screen in another direction to change the orientation. Note: If you have orientation locked, entering Braille Screen Input mode will disable that feature.
  • To change the input from or to contracted or uncontracted braille, flick three fingers to the right or left to change from one to the other. You can also find this feature under Settings > General > Accessibility > VoiceOver > Braille > Braille Screen Input.
  • After typing a word, flick right with one finger to enter a space. If you've made a mistake, flicking one finger to the left will perform a backspace.
  • If you flick up or down with one finger after inputting part or all of a word, you will be offered word suggestions based on common braille mistakes (such as "job" if you wrote "dob"). Flick up or down with one finger until you hear the word you want, then flick right with one finger to select that choice. (There's no need to double tap - that would just add two dots to your text.) Once you have chosen a word, you can continue inputting braille.
  • If you need to enter a new line, flick right with two fingers.
  • When you are done using braille as your input method, simply turn the rotor and all functionality of the touchscreen returns to normal – just as in Handwriting mode.
  • To search for an app on the Home Screen, when you type, iOS will pop up apps matching what you've typed so far; flick up and down to cycle through these, and two-finger flick right to open one.
  • Not a fan of entering your passcode with the touchscreen? No problem! You can also do this with braille gestures. Note: If the first character in your passcode is a number, you will need to first enter a number sign (dots 3-4-5-6) before typing your passcode. Once you are finished entering your passcode, the phone will unlock automatically.

It's important to note that 8-dot braille is only supported on the iPad, not the iPod or iPhone, due to the limited amount of touchscreen space available.

Also worthy of note is that performing a 6-dot gesture is a bit tricky, since Apple's touchscreen only permits five-finger gestures. As such, if you want to write a capitol Q in 8-dot braille, for example (dots 1-2-3-4-5-7), you need to lift a couple of fingers after pressing those dots, but not all, so that the system knows you are going to use other dots. The same scenario applies to writing a full cell in 6- or 8-dot mode.

What's New in iOS 8, Part 2

In Part 2 of his iOS 8 accessibility review, Scott will discuss additional braille changes and updates for users with low vision. For more information, you can contact Scott at

More Information

Assistive Technology
In the News

Meet Lorraine Keller, Ph.D. and My Mobile Light™ Low Vision Aid at Technical Vision, Inc.

Lorraine Keller, PhD

Lorraine Keller, Ph.D. is the CEO of Technical Vision, Inc., a medical equipment company specializing in the design and manufacture of quality personal assistive devices. Technical Vision's customers, many of whom are older adults, live every day with chronic, uncorrectable eye conditions. These conditions include macular degeneration, glaucoma, retinal disease, and age-related night blindness – conditions that can compromise personal safety and the ability to live independently.

Technical Vision's newest – and most innovative – product is My Mobile Light™ Low Vision Aid. My Mobile Light™ projects an intense, clear, ultra-bright LED light beam in a wide arc around the feet and in the immediate walking area. This additional lighting can help the user feel more secure, confident, and independent when walking from the bedroom to the bathroom at night or when traveling in unfamiliar locations outside the home. My Mobile Light™ also functions as a support cane to assist with gait and balance.

After a career of almost 20 years as a life sciences researcher at a large corporation, Dr. Keller "retired" and began a second career as an entrepreneur. Over the past 11 years, she co-founded four life sciences companies that develop products as diverse as gene therapies, immunotherapeutic cancer vaccines for humans, and a new oral drug for dogs and cats with cancer. Technical Vision, Inc. is Dr. Keller's first medical device company.

Maureen Duffy: Hello Dr. Keller. I am very excited to talk with you about the progress you've made in developing My Mobile Light™. To begin, can you tell us the history of this project? How did you initially envision the product and determine it was needed?

Lorraine Keller: Our goal was to create an effective, practical, and easy-to-use daily living aid for people with uncorrectable visual impairments such as age-related night blindness, glaucoma, retinitis pigmentosa, macular degeneration, and diabetic retinopathy, as well as other chronic conditions, like multiple sclerosis and Parkinson's disease.

Low vision impairs depth and color perception and the ability to judge distances, especially in poorly lighted environments. This can dramatically decrease a person's mobility. One of the greatest fears of people with vision loss is getting around safely at home and away without being injured by tripping or falling. Research has shown that the combination of low vision and low light doubles the risk falling and that fifty percent of falls occur at home, most often on level surfaces.

MD: Can you explain some of the features of My Mobile Light™? What makes it different from using a regular support cane and a flashlight, for example?

LK: My Mobile Light™ combines two different assistive concepts – bright lighting and mobility support – in a completely new way. My Mobile Light™ projects clear, even, bright light directly onto the ground around the user's feet and immediate walking area to illuminate obstacles on the floor or outdoor ground.

The support cane function assists with balance and gait, both of which are frequently impaired by vision loss and physical instability. My Mobile Light™ can help people overcome social and infrastructural barriers and enable them to more easily and independently carry out everyday activities that would otherwise be difficult, dangerous, or even impossible.

My Mobile Light graphic

Many people ask us "Why not just use a flashlight or headlamp with a cane?" There are several reasons. Flashlights require a separate hand for operation; a hand that's not available for carrying things, or holding a railing or grab bar. The light emitted by many flashlights is bluish, and not an optimal color for people with low vision. The light tends to be concentrated in a small, intense, and limited area only where the flashlight is pointed. The electronic circuitry in many flashlights causes the light intensity to decrease as the batteries are drained, so light gradually becomes dimmer and less useful.

As for headlamps, the light projects ahead of the traveler and not down around the feet where the immediate tripping hazards are. The wearer has to look down to aim the light, which increases the risk of losing his or her balance. Headlamp straps can cause headaches. And who wants to go out for an evening with family or friends sporting a light on the forehead?

MD: Can you tell us about the research you conducted as you field-tested My Mobile Light™?

LK: We designed and tested My Mobile Light™ under conditions that simulated the same night-time vision impairments and travel conditions that would be experienced by visually impaired users. The most profound experience for me was becoming completely sightless under low vision simulation in the dark – only then did I understand the degree of vision loss and disorientation in the absence of adequate lighting.

Those of us who are fully sighted can only appreciate the difficulties of navigating in low lighting with low vision by walking in the "other person's shoes," and those are the conditions we used to develop our product. We wore goggles that simulated 20/800 visual acuity and conducted our concept tests outdoors at night in suburban neighborhoods with cars, curbs, signposts, shrubbery, fallen tree limbs, and trash cans on and around the streets and sidewalks.

Our research identified the optimal light color, light intensity, and positioning of the light source most useful to the visually impaired user. This was a multidisciplinary collaboration: We worked with Dr. Audrey Smith, Professor and Dean of the Salus University College of Education and Rehabilitation and with professional engineers from IMET Corporation and MTS Ventures, bringing together expertise in low vision, LED lighting, electronics, and product design.

We began by researching how light and color perception are affected by different chronic eye conditions, and selected and tested several colors of LED lights from different color spectra to identify the optimal one for visually impaired eyes. IMET Corporation identified the LED and built special electronic circuitry to provide constant light output that does not gradually dim like a flashlight, and MTS Ventures created the final design that is practical and easy to use.

Shielding this tiny but intense light source is very important to direct the light to the walking area and to minimize glare to the user and people in the surrounding area.

My Mobile Light and descending stairs

Descending stairs with My Mobile Light™

My Mobile Light and ascending stairs

Climbing stairs with My Mobile Light™

MD: What is the difference between My Mobile Light™ and the long white cane that is part of a structured program of orientation and mobility instruction?

LK: My Mobile Light™ is an offset cane that offers gait and balance support. It is not a mobility cane, although we do have a prototype LED mobility cane waiting for a source of funds to develop. An offset cane handle is angled so that the hand grip falls directly over the cane tip or base. It offers better ergonomics and stability, since the person's full weight is centered over the cane shaft and base.

My Mobile Light™ is meant for use by individuals with mild to moderate low vision who also need gait and balance support – typically older adults.

MD: I understand you're in the midst of a major fundraising and manufacturing campaign right now. When will My Mobile Light™ be ready for distribution?

LK: We are still a very small company and have funded virtually all product development ourselves. I am delighted to say that we are in the process of manufacturing our first run of 200 My Mobile Light™ devices that will be ready for purchase online at Technical Vision, Inc in Fall 2014.

We are seeking "angel" investment to fund the next manufacturing run and to support company operations and product launch. We are currently running a crowdfunding campaign on, a new site specializing in fundraising for healthcare companies.

MD: Where can readers locate more information about My Mobile Light™?

LK: Readers can visit our website at, where there is much more information about the My Mobile Light™ product, including a video demonstrating how the product works in dark environments. I also encourage readers to visit our blog, where we post informative and educational articles about vision impairment, falls prevention, and independent living.

MD: Do you have any new research and development projects on the horizon?

My Mobile Light cane

LK: We are planning several enhancements to My Mobile Light™. Chip-based technology makes it easy to incorporate many different safety and convenience functions into a single device for navigation and personal protection.

We are exploring some new concepts to assist with nighttime navigation in the home, and we also plan to use the LED and electronics technology in the My Mobile Light™ device in other products to help people with vision impairment navigate in low lighting.

Right now My Mobile Light™ is only available in basic black; we plan to add more colors and designs to our product line.

We thank Dr. Lorraine Keller for her support of VisionAware and for her research on behalf of blind and visually persons worldwide. You can find additional information on lighting and aging in place at There's No Place Like Home: Planning to Age in Place and Task or Directed Lighting on the VisionAware website.

More Vision Research

My Mobile Light™ images provided by Technical Vision, Inc. Used with permission.

Low Vision
Orientation and Mobility
Getting Around

The True Ice Bucket Challenge: Never Saying "There's Nothing More that Can Be Done"

Artie Kraemer head shot

As the Amyotrophic Lateral Sclerosis (ALS) Association's Ice Bucket Challenge winds down, I can't help but regret that my friend Arthur (Artie) Kraemer (February 12, 1960 - June 12, 2012), who succumbed to ALS, didn't live to see the outpouring of support for the disease (a progressive, incurable neurodegenerative disorder) that claimed his life.

Artie was totally disabled from ALS, also known as Lou Gehrig's Disease, and was, after Stephen Hawking, the longest-lived person with ALS in the world. He wrote and spoke (via voice synthesizer) with the assistance of a specially-designed computer system that he controlled by twitching his right cheek.

He always found it interesting that I worked with people who were blind or had low vision. As he put it, "You work with people who are visually impaired, but have mostly working body parts. I'm the exact opposite: I can see, but I'm pretty much everything-else-impaired. Go figure."

Artie wrote the following story in response to a blog essay in which I roundly chastised a National Public Radio interview with Robert Mazzoli, a retired Colonel and former consultant in ophthalmology to the Army's Surgeon General. Colonel Mazzoli perpetuated (unwittingly, I hope) the harmful notion that "There's nothing more that can be done" as he discussed the vision rehabilitation options available to blind and visually impaired veterans.

Artie's story, about his own experience with a medical system that tried to tell him that "nothing more could be done," is, I believe, a universally applicable one. I hope you agree.

Artie's Story: The Diagnosis

I was diagnosed with ALS at age 21 after displaying the initial symptoms for about a year. I was a happy, healthy soldier serving in Korea and enjoying life. At a unit softball practice in 1980, I hurt my left thumb the same way twice, which was, unbeknownst to me then, the beginning of my ALS misadventure.

I went to the base hospital with a constantly twitching left thumb. Since it was a very small hospital (actually little more than a clinic), the staff there had no clue what was wrong with me, and gave me a cream to rub on my thumb. I returned to the States in February of the following year and was stationed at Fort Campbell in Kentucky.

The hospital there admitted me, knowing that something was seriously wrong with my body. They couldn't pin it down, so they sent me to Walter Reed Army Hospital in Washington, D.C. After a couple months and every neurological test known to mankind, they determined what it was by ruling out everything else.

My doctor was very young, and I definitely felt he didn't want to tell me I was going to die. I practically had to drag it out of him that ALS usually kills within 3-5 years. I have to tell you I never believed that, and never felt like my death was imminent.

How could I live my life thinking any moment could be my last? I couldn't. So I made a conscious decision to more or less ignore it. I've treated ALS as an inconvenience and lived my life around it.

Artie Kraemer in bed

If you do the math, you know I'm now fifty years old and have had this disease for thirty years. I've loved my life and still do, even though I'm pretty messed up: I can't move, can't talk, can't eat, and can't breathe, even. I'm upbeat and happy in spite of all that.

Trying to Breathe

Speaking of breathing, let me tell you why I decided to write this. A doctor gave me the serious "There's nothing more that can be done" speech earlier this year – when I spent nearly three months in the hospital with three separate cases of pneumonia; more likely, it was one case, but the bug simply wasn't killed completely.

The second hospitalization was when "the speech" happened. I was about to be released and the doctor wanted to wean me from the hospital ventilator back to my home ventilator. The process started at 6:00 AM on a Thursday morning. My ventilator had been brought from home so that I could begin to acclimate myself to it.

Under normal procedure, it usually goes easily. This time I felt something was wrong immediately. It felt like I had five claws digging into my chest, plus it was difficult to breathe. I told the therapist what I was feeling, and his response was, "You're fine, all your numbers are within limits, there's nothing to worry about."

After two hours, I finally convinced my nurse to take it off because of the discomfort I was feeling, even though I was trying hard to cooperate because I knew I had to go home with the thing. The next morning they did the same thing again – with the same result. This time I made it for six long hours.

I told the doctor, my home respiratory therapist, and the nurse how I was suffering with this machine, but they left the room. My aide ran after them, telling the doctor to take me off that ventilator right now because I was in agony. They took me off it immediately, but the nurse said that even though I had a break for now, we'd go at it again first thing tomorrow.

Oh, great (not).

Getting "the Speech"

That day, I decided I was never going through that again, even if it took death to accomplish it. This was on Friday afternoon. I told my aide to put the offending ventilator in his truck and take it back home so I couldn't be forced to try it again. The ventilator should have worked, but it just didn't. It was like my body simply rejected it.

So now Saturday morning rolled around. The nurse came into my room, looking for the offending ventilator, expecting to torture me further (and it was torture). She looked around with a puzzled expression, then walked back out without saying a word.

The doctor came in shortly after that. I wasn't hooked up to my computer yet, so my aide told the doctor he would hook me up if he wanted to discuss anything with me. The doctor had a fit! He slammed his clipboard down, ripped off his hospital gown and threw it away, and stormed out. I was so proud!!

Fifteen minutes later, he came back and this is how our conversation went:

Doctor: Why did you take the ventilator away?

Me: So you can't force me to use it again. I'm tired of fighting so hard for everything. There has to be an easier way.

Doctor: Are you ready to die, Arthur?

Me: Is that possible?

Doctor: Yes.

Me: How exactly is that done?

Doctor: We would overdose you with morphine and you'd die within fifteen minutes. You know, it might not be a bad time if you are considering this, because of the problems we're having accessing your venous blood supply. Also, your system is very slow when digesting your food.

Me: (becoming frightened) No, I just want to talk to talk to my Veterans' Administration (VA) case worker on Monday.

Moving to the Veterans' Administration Hospital: Success!

I wrote to my caseworker that day and explained what had happened. She sent an ambulance to take me to the VA hospital, and it arrived at 3:00 PM. My brother-in-law Ken began checking online and actually located a catalog that carried the same ventilator the hospitals use. He found the "baby brother" of the hospital ventilator I had been using, and the VA hospital began searching for it. There was never any death-speak at the new hospital, only how-can-we-make-your-life-better-speak.

They finally located it in Boston and it took about a week for the VA to acquire it. Someone who had a new ventilator was willing to let me borrow their spare for a trial run. You see, when someone uses a "baby brother" mini-ventilator at home, they're given two, a main and a spare, because if the main ventilator fails, you just plug in the spare, which saves your life.

Anyway, the ventilator arrived on Wednesday of the second week. Everyone was nervous, because what if this one didn't work, what if nothing worked? It would be a nursing home for me. Ughh.

So they set it all up, and it took a while, because it was a brand-new machine and no one was familiar with it. The entire setup took about an hour, while my stomach was in knots.

Then they put me on it, and ... instant relief! It felt the same as its big brother! I could tell immediately that this was the "bad boy" I needed. When I gave the thumbs up, the ten or so people who had gathered to witness the trial all clapped and cheered. Don't you know that felt wonderful? And after living with it for a couple months I like it even more, because it's so gentle, almost like breathing on my own.

The Moral of the Story: Never Accept that "Nothing More Can be Done"

The moral? Get more opinions and research, research, research on your own. Nobody cares as much about your vision or your life as much as you do, so definitely be your own best advocate, and don't ever worry about hurting anyone's feelings when it comes to your health.

Rest in peace, Artie – and thank you for never, ever accepting that "There's nothing more that can be done."

Additional Information

Assistive Technology
In the News
Personal Reflections

In Clinical Trials: A Potential Self-Administered Treatment for Diabetic Macular Edema

retina with diabetic retinopathy

Aerpio Therapeutics, Inc. is a new Cincinnati, Ohio-based biopharmaceutical company focused on developing new therapies for vascular [i.e., blood vessel] diseases, including diabetic macular edema (DME) and age-related macular degeneration (AMD).

This week, Aerpio announced positive clinical trial results for AKB-9778, which shows potential for self-administered subcutaneous [i.e., beneath-the-skin injection] treatment of DME, offering an alternative to the current treatment standard of EYLEA, Avastin, and Lucentis, administered via eye injection.

The as-yet-unnamed AKB-9778 demonstrated promise in initial trials with laboratory mice, as reported in the September, 2014 edition of The Journal of Clinical Investigation, and subsequently initiated two new human clinical trials: the TIME-1 DME study (completed) and the TIME-2 DME study (in progress).

More about AKB-9778 and Diabetic Macular Edema

From Aerpio Therapeutics Announces Publication of Preclinical Data on AKB-9778 for Common Eye Diseases at BusinessWire:

Aerpio Therapeutics, Inc., announced the publication of preclinical data demonstrating that lead candidate, AKB-9778, reduces abnormal blood vessel growth and leakage in mouse models of ophthalmic diseases, such as diabetic macular edema (DME) and age-related macular degeneration.

These data were published in collaboration with researchers at Johns Hopkins School of Medicine, Max Planck Institute, and Duke University. AKB-9778 is a small molecule activator of Tie2 currently in a Phase 2 clinical study for the treatment of DME.

[Editor's note: Tie2 plays a central role in blood vessel maturation and maintenance, which prevents the blood vessel leakage that is characteristic of retinal diseases, such as DME.]

"Importantly, the results support the benefit of AKB-9778 as either a mono [i.e., stand-alone] therapy or as an adjunct to anti-VEGF agents [explained below]. Taken together, the results strongly support our ongoing development program in patients with diabetic eye disease and the expansion of the program into other major retinal diseases such as wet age-related macular degeneration and retinal vein occlusion," said Kevin Peters, MD, Chief Scientific Officer of Aerpio.

"We also showed that AKB-9778 was effective in these models when administered either directly into the eye or when given systemically by subcutaneous [i.e., beneath the skin] injections. Given the high cost and inconvenience of current therapies for retinal disease, which are typically injected directly into a patient's eye at a physician's office, we sought to develop AKB-9778 as a potential self-administered subcutaneous alternative that could be used as a mono therapy or potentially [in combination with] anti-VEGF agents."

About Diabetic Macular Edema (DME)

Diabetic macular edema [edema = a swelling or accumulation of fluid] (DME) can occur in people with diabetes when retinal blood vessels begin to leak into the macula, the part of the eye responsible for detailed central vision. These leakages cause the macula to thicken and swell, which, in turn, creates a progressive distortion of central vision.

Although this swelling does not always lead to severe vision loss or blindness, it can cause a significant loss of central, or detail, vision, and is the primary cause of vision loss in people with diabetic retinopathy.

Anti-VEGF Treatments and Diabetic Macular Edema (DME)

Angiogenesis is a term used to describe the growth of new blood vessels and plays a crucial role in the normal development of body organs and tissue. Sometimes, however, excessive and abnormal blood vessel development can occur in diseases such as cancer (tumor growth) and retinal and macular bleeding.

Substances that stop the growth of these excessive blood vessels are called anti-angiogenic (anti=against; angio=vessel; genic=development), and anti-neovascular (anti=against; neo=new; vascular=blood vessels).

The focus of current anti-angiogenic drug treatments is to reduce the level of a particular protein (vascular endothelial growth factor, or VEGF) that stimulates abnormal blood vessel growth in the retina and macula; thus, these drugs are classified as anti-VEGF treatments.

At present, these drugs are administered by injection directly into the eye after the surface has been numbed. Current anti-VEGF drugs in use include EYLEA, Avastin, and Lucentis.

About Clinical Trials

Most clinical trials are designated as Phase 1, 2, or 3, based on the questions the study is seeking to answer:

  • In Phase 1 clinical trials, researchers test a new drug or treatment in a small group of people (20-80) for the first time to evaluate its safety, determine a safe and effective dosage range, and identify possible side effects.
  • In Phase 2a/2b clinical trials, the study drug or treatment is given to a larger group of people (100-300) to determine if it is effective and to further evaluate its safety.
  • In Phase 3 studies, the study drug or treatment is given to even larger groups of people (1,000-3,000) to confirm its effectiveness, monitor side effects, compare it to commonly used treatments, and collect information that will allow the drug or treatment to be used safely.
  • In Phase 4 studies, after the Food and Drug Administration (FDA) has approved the drug, continuing studies will determine additional information, such as the drug's risks, side effects, benefits, and optimal use.

The completed Phase 1b/2a TIME-1 DME Study, entitled Safety and Pilot Efficacy of AKB-9778 in Subjects with Diabetic Macular Edema, evaluated the safety and effectiveness of one month of twice-daily subcutaneous injections of AKB-9778 in 24 subjects with DME. The treatment was well-tolerated and improved visual acuity in some subjects.

The ongoing Phase 2 TIME-DME Study, entitled Safety and Efficacy of AKB-9778 as Monotherapy or Adjunctive to Ranibizumab Compared to Ranibizumab Monotherapy in Subjects with Diabetic Macular Edema, will evaluate the safety and effectiveness of AKB-9778 alone and in combination with ranibizumab [i.e., Lucentis] for three months in subjects with DME.

Additional Information

From Aerpio Therapeutics at the ARVO Annual Meeting, via an Aerpio news release:

"Alternative therapies are needed for treating patients with DME who have persistent macular edema and vision loss despite frequent anti-VEGF injections and also for patients who don't want or don't tolerate intravitreal injections," said Jeffrey Heier, MD, Ophthalmic Consultants of Boston.

"Based on these early clinical data, AKB-9778 may provide a patient self-administered alternative that could be helpful in the treatment of diabetic macular edema."

VisionAware will continue to report the results of this ongoing, yet still unproven, research as they become available.

Medical Updates
Clinical Trials
Diabetes and diabetic retinopathy
Macular Degeneration

Is Glaucoma a Genetic Disease? Three New Research Projects Pinpoint Six Specific Genes

photograph of retina with glaucoma

Three new research projects exploring the role of genes – and six genes in particular – as possible causes of glaucoma have been published simultaneously in the August 31, 2014 online edition of Nature Genetics.

Nature Publishing Group (NPG) is a publisher of scientific and medical information in print and online. NPG publishes a range of journals across the life, physical, chemical, and applied sciences and clinical medicine. Although research scientists are the primary audience, news summaries and articles make many of the most important papers understandable to scientists in other fields and the general public. Topics include current affairs, science funding, scientific ethics, and research breakthroughs.

About Genetics and Glaucoma Research

Excerpted from Six gene variations linked to glaucoma risk at ABC Science-Australia:

Scientists have identified six genetic variants associated with the eye condition glaucoma in people from around the world including Australia. The discovery, in three major studies, could help identify people at higher risk of the disease and lead to earlier screening and treatments.

All three studies, published … in Nature Genetics, identify gene sequence variations of the ABCA1 gene, which is involved in the regulation of cellular cholesterol and lipid metabolism, as playing a role in the eye disease.

Professor Jamie Craig, of the South Australian Health and Medical Research Institute and joint leader of the Australian project, says the finding is significant. "It's rock solid that this is an important result because it has been found in three different ways," says Craig, who is also from Flinders University's Centre for Ophthalmology and Eye Vision Research.

"All [three] papers were done in different populations with different strategies and all identified the same gene. It … tells us for sure it is contributing to glaucoma at least partly through intraocular [i.e, within the eye] pressure pathways."

Craig says the findings may in the future be used to develop risk profiles that will allow doctors to know whether a person has high-risk of their glaucoma being severe. "We are looking at ways to add up a genetic risk profile," says Craig. "So if you can say if you've got a larger load of these variant genes, your risk is high."

However, he cautions it will take several years of experiments before the exact role of the genes identified in these studies is known, and these steps need to be taken before new treatment strategies can be planned.

What Is Glaucoma?

The term "glaucoma" describes a group of eye diseases that can lead to blindness by damaging the optic nerve. It is one of the leading causes of vision loss and blindness. The human eye continuously produces a fluid, called the aqueous, that must drain from the eye to maintain healthy eye pressure.

Types of Glaucoma

  • In primary open-angle glaucoma (POAG), the most common type of glaucoma, the eye's drainage canals become blocked, and the resultant fluid accumulation causes pressure to build within the eye. This pressure can cause damage to the optic nerve, which transmits information from the eye to the brain. Vision loss is usually gradual and often there are no early warning signs.
  • In normal-tension glaucoma, also called low-tension or low-pressure glaucoma, persons with the disease experience optic nerve damage and subsequent vision loss, despite having normal intraocular [i.e., within the eye] pressure (IOP). This type of glaucoma is treated much like POAG, but eye pressure needs to be kept even lower to prevent progression of vision loss.
  • Secondary glaucomas develop as secondary to, or as complications of, other conditions, including cataracts, diabetes, eye trauma, eye surgery, or tumors. In many of these glaucomas, damage to the fluid drainage canal must be addressed with medication or surgery.

Eye Pressure and Glaucoma

Most eye care professionals define the range of normal within-the-eye pressure as between 10 and 21 mm Hg [i.e., millimeters of mercury, which is a pressure measurement]. Most persons with glaucoma have an IOP measurement of greater than 21 mm Hg; persons with normal-tension glaucoma, however, have an IOP measurement within the normal range.

Visual Field Loss from Glaucoma

Glaucoma results in peripheral (or side) vision loss initially, and the effect as this field loss progresses can be like looking through a tube or into a narrow tunnel. This "tunnel vision" effect makes it difficult to walk without bumping into objects that are off to the side, near the head, or at foot level:

A living room viewed through a constricted visual field

A living room viewed through a constricted visual field.
Source: Making Life More Livable. Used with permission.

Glaucoma is an especially dangerous eye condition because most people do not experience any symptoms or early warning signs at the onset. Glaucoma can be treated, but it is not curable. The damage to the optic nerve from glaucoma cannot be reversed.

More about the Research from Nature Genetics

The First Study: Australia and New Zealand

The first study, entitled Common variants near ABCA1, AFAP1 and GMDS confer risk of primary open-angle glaucoma, examined potential genes involved in primary open-angle glaucoma (POAG).

The study included 1,155 subjects from the Australian and New Zealand Registry of Advanced Glaucoma with severe POAG, and 1,992 control subjects, who did not have the disease. Genetic testing identified variants of three genes that significantly increased the risk for POAG in Australians and Americans of European descent. (Source: ABC Science)

From the Nature Genetics abstract:

Primary open-angle glaucoma is a major cause of irreversible blindness worldwide. We performed a genome-wide association study in an Australian discovery cohort comprising 1,155 cases with advanced POAG and 1,992 controls… [W]e show that these genes are expressed within the human retina, optic nerve, and trabecular meshwork, and that two of the genes are also expressed in retinal ganglion cells.

[Note: Aqueous fluid drains through a filtering meshwork of spongy tissue along the outer edge of the iris, called the "trabecular meshwork," where the iris and cornea meet, and into a series of tubes, called Schlemm's canal, that drain the fluid out of the eye.]

[Note: In the human eye, a layer of cells in the retina, called photoreceptor cells, detects light and a separate layer of cells, called "ganglion cells," relays that information to the brain.]

The Second Study: Multi-Ancestry

The second study, entitled Genome-wide analysis of multi-ancestry cohorts identifies new loci influencing intraocular pressure and susceptibility to glaucoma, examined potential genes associated with high intraocular pressure (IOP) and glaucoma.

This study involved genetic screening of 35,296 subjects from the International Glaucoma Genetics Consortium. The subjects included persons of Asian and European descent from seven countries.

Genetic testing identified four new genes associated with high IOP and glaucoma. One of the genes is the ABO gene, which determines blood group, and higher eye pressure appears to be linked to blood type B. (Source: ABC Science)

From the Nature Genetics abstract:

Elevated intraocular pressure (IOP) is an important risk factor in developing glaucoma and variability in IOP might herald glaucomatous development or progression. We report the results of a genome-wide association study meta-analysis of 18 population cohorts from the International Glaucoma Genetics Consortium, comprising 35,296 multi-ancestry participants for IOP. We confirm genetic association of known [locations] for IOP and POAG and identify four new IOP-associated [gene locations].

The Third Study: China and Singapore

The third study, entitled Common variants near ABCA1 and in PMM2 are associated with primary open-angle glaucoma, is the first large-scale study to examine potential genes involved in primary open-angle glaucoma (POAG) in an Asian population.

The study included 1,007 subjects with severe POAG, and 1,009 control subjects who did not have the disease, all from southern China. Genetic testing identified variants near two genes associated with glaucoma risk in people from China and Singapore. (Source: ABC Science)

From the Nature Genetics abstract:

We performed a genome-wide association study for primary open-angle glaucoma (POAG) in 1,007 cases with high-pressure glaucoma (HPG) and 1,009 controls from southern China. Both the ABCA1 and PMM2 [genes] are expressed in the trabecular meshwork, optic nerve and other ocular tissues. In addition, ABCA1 is highly expressed in the ganglion cell layer of the retina, a finding consistent with it having a role in the development of glaucoma.

Additional Information

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