by Maureen Duffy
Editor's note: One of the many benefits associated with an online information center and website, such as VisionAware, is the ability to track readers' search terms [i.e., information readers are seeking as they search online].
Of particular concern to many readers are issues related to the diagnosis and treatment of low vision, as evidenced by the following searches:
- I've been told I have low vision, but what does this mean?
- How is low vision different from blindness?
- Is there a cure for low vision?
An Answer from VisionAware: What "Low Vision" Means
As we age, our eyes change too. In most cases, regular eyeglasses or contact lenses can correct many of these vision changes. However, if your eye doctor tells you that your vision cannot be fully corrected with ordinary prescription glasses, medication, or surgery and you still have some usable vision, you have what is called "low vision."
Having low vision means that even with regular glasses, contact lenses, medication, or surgery, you may find it difficult to perform everyday tasks, such as reading your mail, shopping, preparing meals, and signing your name.
Signs and Symptoms of Low Vision
There are many signs and symptoms that can indicate low vision. For example, even with your regular eye glasses, do you have difficulty:
- Recognizing faces of your friends and relatives?
- Performing tasks that require you to see well up close, such as reading, crafting, making home repairs, or organizing and labeling your clothing?
- Performing tasks at work or home because lights now seem dimmer?
What Causes Low Vision?
Among older persons, low vision can result from specific eye conditions, such as macular degeneration, glaucoma, and diabetic retinopathy; from a stroke; or from a range of other eye conditions. Low vision can affect your ability to see people's faces, watch television, read, drive, and use a computer.
What You Should Know about Low Vision
Having "low vision" is not the same as being "blind." For example, your doctor may tell you that you have a blind or blank spot in the center of your vision that limits your ability to read or see people's faces; nevertheless, you can still get around using your side (or peripheral) vision:
A simulation of central visual field loss from macular degeneration
Source: Henry Ford Center for Vision Rehabilitation and Research
Or you may have problems seeing well with your peripheral (or side) vision, but still see clearly enough to read the newspaper using your central vision:
A living room viewed through a constricted visual field.
Source: Making Life More Livable. Used with permission.
There is Help for Low Vision
The important thing is to know that help is available for you. For example, eye doctors who are low vision specialists can provide you with a low vision exam as a first step in determining how you can use your remaining vision. Often, a low vision specialist can give you recommendations about low vision optical devices, non-optical devices, electronic magnifiers, and vision rehabilitation services that can help you maximize your remaining vision and learn new ways of doing everyday tasks.
Some examples of helpful devices that a low vision specialist can discuss with you include:
Reading with a lighted
- illuminated stand magnifiers or electronic aids for reading
- magnifying reading glasses or loupes for seeing the computer screen, sheet music, or for sewing and embroidery
- telescopic glasses or monoculars for seeing television, faces, signs, or other items at a distance
- glare shields or absorptive sunglasses for reducing glare and enhancing contrast
- adaptive daily living equipment to make everyday tasks easier, such as timepieces with larger numbers, writing guides, or black and white cutting boards.
Low Vision Services
Low vision services can include any or all of the following:
- training to use optical and electronic devices effectively
- training to help you use your remaining vision more effectively
- improving the lighting and enhancing contrast in each area of your home
- providing a link with a counselor or a support group to help you deal with your feelings related to your changing vision
- learning about other helpful training and rehabilitation resources in your community and state.
What Are Low Vision Devices?
Stand magnifier with a handle
Low vision devices can help you make the most of your vision so that you can perform everyday tasks more easily and with less frustration.
Some devices, such as optical and non-optical aids, offer very simple and relatively inexpensive solutions. Other devices, such as electronic and digital magnifiers, may be slightly more complex and costly.
However, both optical devices and electronic or digital devices require training to use them efficiently and effectively. Training is always one of the main keys to success with the use of low vision devices.
There are several different categories of low vision devices: optical devices, non-optical devices, and electronic magnifiers and magnifying systems. Low vision devices are task-specific, designed for close-up visual tasks or distance viewing. You may require several different devices to accomplish different tasks, depending upon your eye condition and your everyday living needs.
Low Vision Optical Devices
Low vision optical devices include a variety of helpful visual aids, including stand and hand-held magnifiers, strong magnifying reading glasses, loupes, and small telescopes. Because these devices can provide greatly increased magnification powers and prescription strengths, along with higher-quality optics (i.e., the way the lens bends or refracts light), they are different from regular glasses and magnifiers that you can buy in a local store or online. Most often they require training to help you use them effectively.
Low Vision Non-Optical Devices
Low vision non-optical devices can include adaptations such as reading stands, supplemental lighting, absorptive (or glare control) sunglasses, typoscopes, and tactile locator dots. They can be used in combination with low vision optical devices and can help with reading, organizing, labeling, and a variety of everyday tasks.
Electronic Magnifying Systems
Electronic magnifying systems come in many different varieties and sizes, depending upon the task or activity you want, or need, to do. Some have a camera system that displays a magnified image on a monitor, which can be helpful for reading mail, books, and magazines, while others are hand-held, portable, and can be taken to the supermarket to read labels and coupons, or to restaurants for reading menus.
How Can I Obtain a Low Vision Device?
They are often recommended as part of a low vision examination. A low vision exam by a low vision specialist — an ophthalmologist or optometrist with credentials or specialization in low vision testing, diagnosis, and treatment — is the best way to decide what type of device or devices are best for you, your eye condition, and your everyday living needs. At your low vision evaluation, you will have the opportunity to try a variety of devices in a variety of settings and learn first-hand how they can work for you.
For more information about low vision services and providers, see Meet Joseph Fontenot, MD, CVLT: Be Informed and Proactive About Low Vision Services, Protect Yourself, and Always "Buyer Beware" on the VisionAware blog.
How Can I Locate a Low Vision Specialist?
You can find a listing of low vision specialists in the Low Vision Services category in the AFB Directory of Services. In addition to the low vision providers in the Directory listings, you can find additional providers through the following directories:
- The American Academy of Ophthalmology directory. Use the subspecialty category "Low Vision Rehab."
- The American Optometric Association Doctor Locator database. Use the "Advanced Search" and look for members of the Vision Rehabilitation section.
- The American Occupational Therapy Association (AOTA) database of specialty certified low vision practitioners
by Maureen Duffy
Two questions asked most frequently by readers about age-related macular degeneration (AMD) involve (a) individual risk for the disorder and (b) the likelihood of eventual involvement of both eyes. In response, several recent studies have attempted to address these critically important questions:
- Data from the ongoing Blue Mountains Eye Study (BMES) and the Age-Related Eye Disease Study (AREDS) identified a number of demographic and eye-related factors that were used to develop a "scoring system" to predict the risk of developing AMD up to 10 years in the future. You can read more about this research at Is It Possible to Predict Risk for Developing Macular Degeneration? on the VisionAware blog.
- Data from the ongoing Beaver Dam Eye Study investigated the pattern and progression of wet (also called neovascular) AMD and determined that having wet AMD in one eye was associated with an increased incidence and progression of AMD in the other eye. You can read more about this research at Macular Degeneration in One Eye Associated with Increased Incidence and Progression in the Other Eye on the VisionAware blog.
Now, an international group of researchers from the three-continent AMD Consortium have combined data from the Blue Mountains Eye Study, the Beaver Dam Eye Study, and the Rotterdam Study (all explained below) and concluded that "one in four to one in five unilateral [i.e., occurring in one eye] any stage [i.e., early through late] of AMD cases, and up to one in two unilateral late AMD cases, progressed to bilateral [i.e., both eyes] in 5 years. Known AMD risk factors, including smoking, are significantly associated with the progression to bilateral involvement."
From the British Journal of Ophthalmology
This new macular degeneration research, titled Five-year progression of unilateral age-related macular degeneration to bilateral involvement: the Three Continent AMD Consortium report, has been published "online first" in the January 2017 edition of the British Journal of Ophthalmology (BJO). BJO is an international peer-reviewed journal for ophthalmologists and visual science specialists that publishes clinical investigations, clinical observations, and clinically relevant laboratory investigations related to ophthalmology.
The authors are members of the AMD Consortium (with all authors listed online) and represent the following countries and institutions: University of Sydney; University of Newcastle; John Hunter Hospital and Hunter Medical Research Institute, Australia; Erasmus Medical Center, Rotterdam; Royal Netherlands Academy of Arts and Sciences, Leiden; Netherlands Genomics Initiative, The Hague; Radboud University Nijmegen Medical Center, The Netherlands; Case Western Reserve University, Cleveland, Ohio; University of Wisconsin School of Medicine and Public Health, United States.
More about Age-Related Macular Degeneration
Looking at the world with AMD
Age-related macular degeneration (AMD) is a gradual, progressive, painless deterioration of the macula, the small sensitive area in the center of the retina that provides clear central vision. Damage to the macula impairs the central (or "detail") vision that helps with essential everyday activities, such as reading and writing, preparing meals, watching television, and personal self-care.
AMD is the leading cause of vision loss for people aged 60 and older in the United States. According to the American Academy of Ophthalmology, 10-15 million individuals have AMD and about 10% of people who are affected have the "wet" type of AMD.
Wet (Neovascular) Macular Degeneration
In wet, or exudative, macular degeneration (AMD), the choroid (a part of the eye containing blood vessels that nourish the retina) begins to sprout abnormal new blood vessels that develop into a cluster under the macula, called choroidal neovascularization (neo = new; vascular = blood vessels).
Because these new blood vessels are abnormal, they tend to break, bleed, and leak fluid under the macula, causing it to lift up and pull away from its base. This damages the fragile photoreceptor cells, which sense and receive light, resulting in a rapid and severe loss of central vision.
Dry Macular Degeneration
A retina with dry AMD and drusen
The dry (also called atrophic) type of AMD affects approximately 80-90% of individuals with AMD. Its cause is unknown, it tends to progress more slowly than the wet type, and there is not – as of yet – an approved treatment or cure.
"Atrophy" refers to the degeneration of cells in a portion of the body; in this case, the cell degeneration occurs in the retina.
In dry age-related macular degeneration, small white or yellowish deposits, called drusen, form on the retina, in the macula, causing it to deteriorate or degenerate over time.
Drusen are the hallmark of dry AMD. These small yellow deposits beneath the retina are a buildup of waste materials, composed of cholesterol, protein, and fats. Typically, when drusen first form, they do not cause vision loss. However, they are a risk factor for progressing to vision loss.
Risk Factors for Macular Degeneration
The primary risk factors for AMD include the following:
- Smoking: Current smokers have a 2-3 times higher risk for developing AMD than do people who never smoked. It's best to avoid second-hand smoke as well.
- Sunlight: Ultraviolet (UV) light is not visible to the human eye, but can damage the lens and retina. Blue light waves that make the sky, or any object, appear blue, are visible to the human eye and can also damage the lens and retina. Avoid UV light and blue/violet light as much as possible by wearing sunglasses with an amber, brown, or orange tint that blocks both blue and UV light.
- Uncontrolled hypertension: The National Eye Institute (NEI) reports that persons with hypertension were 1.5 times more likely to develop wet macular degeneration than persons without hypertension. It's important to keep your blood pressure controlled within normal limits.
- A diet high in packaged, processed food and low in fresh vegetables: NEI suggests that eating antioxidant-rich foods, such as fresh fruits and dark green leafy vegetables (kale, collard greens, and spinach) may delay the onset or reduce the severity of AMD. Eating at least one serving of fatty fish (salmon, tuna, or trout) per week may also delay the onset or reduce the severity of AMD.
- Race: According to NEI, Whites/Caucasians are more likely to have AMD than people of African descent.
- Family history: NEI reports that individuals with a parent or sibling with AMD have a 3-4 times higher risk of developing AMD.
You can read more about the full range of AMD risk factors at Risk Factors for Age-Related Macular Degeneration on the VisionAware website.
What is the Blue Mountains Eye Study?
The longitudinal Blue Mountains Eye Study (BMES), named after the Australian mountain range, is the first large population-based assessment of visual impairment and common eye diseases of a representative sample of older Australians. A longitudinal study follows, and gathers information about, the same individuals or group of people over an extended period of time – often many decades.
The ongoing BMES began in 1992-1993, with the recruitment of 3,654 people aged 50 and older. Follow-up exams were conducted in 1997-1999 (5-year), 2002 (10-year) and 2007-2009 (15-year). You can read more about the BMES at Research Data Australia.
What is the Beaver Dam Eye Study?
The longitudinal Beaver Dam Eye Study is funded by the National Eye Institute. The purpose of the Study is to collect information on the prevalence and incidence of age-related cataract, macular degeneration, and diabetic retinopathy. The study was designed to discover, or detect, causes of these conditions.
The study was funded initially in 1987. A private census conducted in the city and township of Beaver Dam, Wisconsin found that there were approximately 6,000 people aged 43 through 84 years. Approximately 5,000 of them participated in the baseline examination between 1988 and 1990.
Five-, 10-, 15-, and 20-year follow-up examinations have taken place and 3,700; 2,800; 2,100; 2,000; and 1,900 people participated in each of the respective examination phases. Thus far, there have been more than 300 publications describing the prevalence and incidence of visual loss, age-related macular degeneration, cataract, and associated risk factors as a result of this study. You can read more about the study at the Beaver Dam Eye Study website.
What is the Rotterdam Study?
The Rotterdam Elderly Study is a longitudinal study in the Ommoord district in Rotterdam, the Netherlands. Following the pilot study in 1989, recruitment began in January 1990. The main objectives of the Rotterdam Study are to investigate the risk factors of cardiovascular, neurological, ophthalmological, and endocrine diseases in older persons. The study has recruited approximately 15,000 subjects aged 45 years or older.
Participants were interviewed at home and received an extensive set of examinations, repeated every 3-4 years. They were followed for the most common diseases in older persons, including coronary heart disease, heart failure, and stroke; Parkinson's disease; Alzheimer's disease and other dementias; depression and anxiety disorders; macular degeneration; glaucoma; diabetes; and osteoporosis. You can read more about the study at the University Medical Center Rotterdam website.
What the Macular Degeneration Progression Study Found
Excerpted from the study abstract:
Purpose: To assess the 5-year progression from unilateral [i.e., in one eye] to bilateral [i.e., in both eyes] age-related macular degeneration (AMD) and associated risk factors.
Design: Pooled data analyses of three prospective population-based cohorts, the Blue Mountains Eye Study, Beaver Dam Eye Study and Rotterdam Study.
Methods: Retinal photography and interview with comprehensive questionnaires were conducted at each visit of three studies. AMD was assessed following the modified Wisconsin AMD grading protocol. Progression to bilateral any (early and late) or late AMD was assessed among participants with unilateral involvement only.
Results: In any 5-year duration, 19–28% of unilateral any [i.e., early through late] AMD cases became bilateral and 27–68% of unilateral late AMD became bilateral. Factors associated with the progression to bilateral involvement of any AMD were age, carrying risk forms of the complement factor H (CFH) and age-related maculopathy susceptibility 2 (ARMS2) genes, smoking, and the presence of large drusen area or retinal pigmentary abnormalities in the first eye.
[Editor's note: The CFH gene makes a protein called complement factor H (CFH), which regulates a part of the body's immune response known as the complement system. It destroys bacteria and viruses, triggers an inflammatory response, and removes debris from cells and tissues. CFH protects healthy cells by preventing the complement system from being activated when it is not needed. (Source: Genetics Home Reference.)]
[The ARMS2 gene provides instructions for making a protein whose function is unknown. Studies suggest that the ARMS2 protein is found primarily in the placenta and in the retina. However, it is unclear what role, if any, the protein plays in early development or normal vision. (Source: Genetics Home Reference.)]
Conclusion: One in four to one in five unilateral any [i.e., early through late] AMD cases, and up to one in two unilateral late AMD cases, progressed to bilateral in 5 years. Known AMD risk factors, including smoking, are significantly associated with the progression to bilateral involvement.
Additional Information About Macular Degeneration
New Glaucoma Research: Will Patients and Family Members Pay for a Glaucoma-Specific App? Not Likely, Results SayPosted on 1/30/2017
by Maureen Duffy
New – and highly relevant – research from the Wills Eye Glaucoma Research Center, in collaboration with Drexel University, sought to "evaluate the interest of glaucoma patients and their caregivers in a smartphone-based and tablet-based glaucoma application" (app) that contained a range of features (explained below) designed to (a) increase patients' level of knowledge about glaucoma and (b) improve their adherence to medication and follow-up appointment recommendations.
Their research results, which are applicable to doctors, patients, family members, rehabilitation professionals, and app developers, indicate that participants were more likely to download the App if it was free of charge, compared with a version that cost $3.00.
From the Journal of Glaucoma
This new glaucoma app research, titled The Wills Eye Glaucoma App: Interest of Patients and Their Caregivers in a Smartphone-based and Tablet-based Glaucoma Application has been published in the Journal of Glaucoma, the official journal of the American Glaucoma Society. The purpose of the Journal is to provide "a stimulating forum for discussion of clinical, scientific, and socioeconomic issues of greatest concern to clinicians who care for glaucoma patients."
The authors are Michael Waisbourd, MD; Hermandeep Dhami, BSc; Chen Zhou, BSc; Michael Hsieh, BSc; Pramod Abichandani, PhD; Michael J. Pro, MD; Marlene R. Moster, MD; L. Jay Katz, MD; Lisa A. Hark, PhD, RD; and Jonathan S. Myers, MD, from the Glaucoma Research Center, Wills Eye Hospital, and the Drexel University iSchool and College of Engineering, Philadelphia, PA.
About the Wills Eye Hospital Glaucoma App Research
Excerpted from Wills Eye Hospital study details why they built a glaucoma app and patient price sensitivity to medical apps, via iMedicalApps:
Wills Eye Hospital has a [new] glaucoma app for patients called Glaucoma from Wills Eye. They built their glaucoma app over a two-year period, in conjunction with Drexel University's iSchool and College of Engineering. Before releasing the glaucoma app, they did an internal study to see what key features patients would want.
Fifty participants were in the study and they were asked questions on key features they would want in the app. Some key findings from the study were the respondents' answers when it came to mobile technology. Almost 70 percent of patients already had a smartphone or tablet. Nearly half the patients used their smartphones at some point to look up health information.
Out of all the conclusions, the price sensitivity for downloading a health app was the most interesting. While more than 70% of the patients were interested in downloading the Wills Eye Glaucoma app, only 25% of the patients would actually download the app if it was priced at just $3.00. I call this the "Angry Birds" effect. The gaming section is filled with apps that are free or just $0.99. They can afford to do this since there are a significant number of individuals available to download these apps.
For medical specialty apps to succeed financially (be able to at least pay for development costs), they need to charge [a much higher price], since the number of individuals that have the potential to actually use the app is significantly smaller. Unfortunately — even though patients find these types of medical apps useful — the barrier of price, which is similar to a cup of coffee at Starbucks, prevents this from happening.
What Is Glaucoma?
Glaucoma is a group of eye diseases that can lead to blindness by damaging the optic nerve, which transmits information from the eye to the brain, where it is processed and interpreted. The eye continuously produces a fluid, called the aqueous, that must drain from the eye to maintain healthy eye pressure. Glaucoma is particularly dangerous to your vision because there are usually no noticeable initial symptoms or early warning signs.
The Different Types of Glaucoma
Primary Open Angle Glaucoma
The most common type of glaucoma is Primary Open Angle Glaucoma (POAG). In POAG, the eye's drainage canals become blocked, and the 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 with this type of glaucoma is usually gradual, and often there are no early warning signs. There is a strong genetic predisposition for this type of glaucoma.
Angle Closure Glaucoma
Angle Closure Glaucoma is much less common than POAG in the United States. In this type of glaucoma, the aqueous cannot drain properly because the entrance to the drainage canal is either too narrow or is closed completely. In this case, eye pressure can rise very quickly and can be triggered by pupil dilation.
Symptoms can include sudden eye pain, nausea, headaches, and blurred vision. If you experience these symptoms, you should seek immediate medical treatment.
Normal Tension Glaucoma
In this type of glaucoma, also called low-pressure glaucoma, there is damage to the optic nerve, even though the eye pressure is not elevated excessively. A family history of any type of glaucoma, cardiovascular disease, and Japanese ancestry are a few of the risk factors for this type of glaucoma.
This type of glaucoma is treated much like POAG, but the eye pressure needs to be kept even lower to prevent progression of vision loss.
The typical progression of vision loss from glaucoma
Source: National Eye Institute
National Glaucoma Awareness Month provides an excellent opportunity to learn more about glaucoma, a leading cause of vision loss that affects more than 3 million people in the United States. Glaucoma often is called "the sneak thief of sight" for good reason: Many people are unaware that glaucoma has few symptoms or warning signs in its early stages. Early treatment for glaucoma can usually (but not always) slow the progression of the disease. However, as of yet, there is no cure for glaucoma.
Because glaucoma has no obvious initial symptoms, a comprehensive dilated eye exam is critical to detect early glaucoma changes. People who are over 40 should have a dilated eye examination from an ophthalmologist or optometrist at least every two years. African Americans; people who are over 35 and have a family history of glaucoma; and everyone age 60 or older should schedule a comprehensive eye examination every year.
You can learn more about glaucoma detection and treatment at How Can I Detect Glaucoma if There Are No Initial Symptoms?, What Are the Different Treatments for Glaucoma?, and Tips for Taking Glaucoma (and Other) Eye Drops at VisionAware.
More about the Wills Eye Hospital Glaucoma App
Screen shot of the app
Excerpted (with screen shot) from the free Wills Eye Hospital Glaucoma App, available at the iTunes Store:
The free Wills Eye Hospital Glaucoma App was designed to educate patients and their caregivers about glaucoma and help them manage their eye disease by using an automated eye-drop reminder feature and a calendar for tracking eye exam appointments. The main features of the App include:
- Educational videos about glaucoma, how to use eye drops, benefits of glaucoma laser treatment, and surgery
- Tutorial on how to take a visual field examination
- Eye-drop reminder feature
- Appointment reminder feature
- Storage of medical information
- Tracking of eye pressure results
Compatibility: Requires iOS 8.0 or later. Compatible with iPhone, iPad, and iPod touch.
More about the Study from the Journal of Glaucoma
Excerpted from the study abstract:
Purpose: To evaluate the interest of glaucoma patients and their caregivers in a smartphone-based and tablet-based glaucoma application (App), developed by the Wills Eye Glaucoma Research Center in collaboration with Drexel University.
Materials and Methods: Cross-sectional survey of patients with glaucoma and their caregivers. Main outcome measures are answers to survey questions regarding how receptive participants are to using the Glaucoma App.
Results: Fifty subjects completed the survey. The mean age was 59.5 years. A total of 88.6% of the participants lived in a household with access to a smartphone or tablet. The majority (72.3%) of participants would consider downloading the Glaucoma App, and younger participants (less than 65 years) were more likely to do so compared with their older (more than, or equal to, 65 years) counterparts.
Participants were more likely to download the App if it was free of charge, compared with a version that costs $3. Although only about one third (37.8%) of participants used eye drop reminders, nearly 3 of 4 (72.9%) participants were receptive to using the automated reminder feature of the Glaucoma App.
Conclusions: Glaucoma patients and their caregivers were very interested in using a Glaucoma App; however, many were not willing to spend $3 for an App they seem to value. The free Wills Eye Glaucoma App currently available on the Apple store, includes educational videos, eye drop and appointment reminders, medical and ocular data storage, visual field tutorial, and intraocular pressure tracker. These features aim to increase patients' level of knowledge about glaucoma and improve their adherence to medication and follow-up appointment recommendations.
More Helpful (and Free) Apps from American Foundation for the Blind (AFB) and VisionAware
- The AFB VisionConnect™ App: Provides a searchable directory of services available in the US and Canada for children and adults who are blind or visually impaired.
- AccessWorld® App: The official app of the American Foundation for the Blind's online magazine AccessWorld®: Technology News for People Who Are Blind or Visually Impaired.
- AccessNote: the Official iOS Notetaker App: The first notetaker for the iOS platform designed particularly for VoiceOver users looking for a highly efficient, feature-rich note taking experience.
- The AFB CareerConnect App: An online resource center specific to career exploration, job seeking skills, transition from school to work, e-mentoring, and navigating the employment process as a person who is blind or visually impaired.
Additional Glaucoma Information
by Maureen Duffy
A retina with wet AMD
Many readers have been following closely the development of Squalamine Eye Drops for wet age-related macular degeneration, hoping that a self-administered at-home eye drop could reduce, or even eliminate, the need for monthly or as-needed eye injections. Unfortunately, a clinical trial designed to test this concept has produced disappointing results: Squalamine Eye Drops failed to reduce the average number of Lucentis injections required by the trial participants.
However, that clinical trial also revealed that squalamine produced positive changes in visual acuity – both overall in the Lucentis/squalamine treatment group, and, more significantly, in participants with a specific type of AMD lesion. As a result, a newer clinical trial, initiated in 2016 and continuing into 2019, will continue to study the effects of Squalamine Eye Drops, in combination with Lucentis injections, on gains in visual acuity in persons with wet macular degeneration.
What Is Squalamine?
Ohr Pharmaceutical, Inc. is dedicated to the clinical development of new drugs for underserved therapeutic needs. One of its lead drugs in development is Squalamine Eye Drops for the treatment of wet age-related macular degeneration (AMD).
Squalamine is a water-soluble molecule derived from the internal organs (primarily the liver) of the dogfish shark. It is believed to have great potential for treating some human viruses. Medically, squalamine has been shown to interrupt and reverse the process of angiogenesis.
About Angiogenesis and Anti-Angiogenic Drugs
Angiogenesis is a term that describes the growth of new blood vessels and plays a critical 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 AMD (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 for wet macular degeneration 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.
The squalamine researchers theorized that the ability to self-administer eye drops to treat wet AMD could be more effective (and less invasive) than the current standard of care, which involves regular injections of Lucentis, Avastin, or Eylea directly into the eye, via a very small needle. These injections are administered at the doctor's office.
A History of Squalamine Eye Drop Research
Initially, the Genaera Corporation developed the squalamine project and administered squalamine as an intravenous formulation for wet AMD in Phase 1 and Phase 2 clinical trials. However, in 2007, Genaera discontinued their clinical trials, due to financial and subject-recruitment issues. The squalamine project was then acquired by Ohr Pharmaceutical, which continued the development of squalamine in a topical eye drop formulation.
In 2012, Ohr was awarded "Fast Track" designation by the United States Food and Drug Administration (FDA) to further develop its Squalamine Eye Drop product for the potential treatment of wet AMD. Fast Track is a process designed to facilitate the development, and expedite the review, of drugs to treat serious diseases and fill an unmet medical need. Filling an unmet medical need is defined as providing a therapy where none exists or providing a therapy that may be potentially superior to existing therapy.
The Phase 2 Clinical Trial
In August 2012, the FDA commenced a Phase 2 clinical trial to study the safety and effectiveness of Squalamine Eye Drops in combination with Lucentis injections. The researchers were seeking to determine (a) if squalamine was safe (safety) and (b) if Squalamine Eye Drops could reduce the number and frequency of Lucentis injections required to control the neovascularization and retinal bleeding associated with wet AMD (effectiveness).
The initial enrollment in the clinical trial was 142 "treatment-naïve" participants [i.e., meaning that no participant had received prior treatments] at 23 clinical ophthalmology centers across the United States. 128 participants completed the study.
All participants in the nine-month study received an initial injection of Lucentis to begin and then were randomly assigned to receive either Squalamine Eye Drops [i.e., the treatment group] or placebo eye drops twice a day throughout the study. [Editor's note: A placebo is an inactive substance that has no therapeutic effect, used as a control in testing new drugs.]
All participants were seen monthly during the study period and received eye examinations, visual acuity testing, and optical coherence tomography (OCT) testing. Additional Lucentis injections were given on an as-needed basis to control the new blood vessel development and retinal bleeding associated with wet AMD. [Editor's note: Optical coherence tomography (OCT) is a type of medical imaging technology that produces high-resolution cross-sectional and three-dimensional images of the eye. It is used to gain a clearer picture of the retina and its supporting layers.]
You can learn more about the Phase 2 squalamine clinical trial, including criteria for enrollment and the locations of the clinical trial study centers, at Efficacy and Safety of Squalamine Eye Drops at ClinicalTrials.gov.
The Clinical Trial Results
Safety: Squalamine Eye Drops were well-tolerated. Only two participants in the treatment group discontinued the study, due to eye pain or swelling.
Effectiveness: Unfortunately, Squalamine Eye Drops failed to decrease the average number of Lucentis injections required by the study participants. This was the primary goal of the clinical trial and the result was disappointing, both to researchers and to people with wet AMD, who were hoping that Squalamine Eye Drops could possibly reduce, or even eliminate, the need for eye injections.
There Were Some Positive Results from the Phase 2 Clinical Trial
The Early Treatment of Diabetic Retinopathy Study (ETDRS) Chart
Despite not reaching the study's primary end goal, there were additional results from the Phase 2 clinical trial that may be encouraging. Although Squalamine Eye Drops did not reduce the need for, or the frequency of, eye injections, squalamine did produce positive changes in visual acuity, or clearness of vision – both overall in the Lucentis/squalamine treatment group, and more significantly, in participants with a specific type of AMD lesion.
31% of the study participants who received the combined Lucentis/squalamine treatment gained 3 or more letters on the Early Treatment of Diabetic Retinopathy Study (ETDRS) Eye Chart (pictured at left), versus 25% of participants who received Lucentis treatment alone (meaning the Lucentis/placebo treatment).
However, 37 participants with "classic" AMD lesions who received the combined Lucentis/squalamine treatment gained 11 letters on the ETDRS Eye Chart, versus 5 letters with Lucentis treatment alone. Classic AMD lesions have also shown benefit in other studies of combined treatment.
A Phase 3 Clinical Trial Is Now Underway
Due to these gains in vision from the study's combination therapy, Ohr has moved forward with a Phase 3 clinical trial. The two-year trial, which commenced in 2016, will enroll 650 participants and is estimated to be completed in mid-2019. It will continue to study the effects of Squalamine Eye Drops in combination with Lucentis injections, versus Lucentis injections alone, on best-corrected visual acuity.
Update: On February 15, 2107, Ohr Pharmaceuticals announced that it had temporarily suspended new enrollment in the Phase 3 clinical trial. While the 200 patients currently enrolled in the study will continue to receive the therapy, the trial will be closed for new patients.
"This approach is intended to provide prospective efficacy data before year end 2017 to enable us to potentially confirm the visual acuity benefits observed in the patient population we identified as the most likely to benefit from Squalamine combination therapy. Given the recent study readouts from other combination therapy agents and the reaction to these results, we feel that a change in our clinical development program is warranted," said Dr. Jason Slakter, Chief Executive Officer of Ohr.
Additional Information About Macular Degeneration
New Research: Ebola Survivors Have Ongoing Risk of Eye Disease, Even When the Initial Outbreak Has ConcludedPosted on 1/16/2017
by Maureen Duffy
Although worldwide attention was focused on the 2014-2016 Ebola outbreak in West Africa, considerably less attention – until now – has been paid to the eye and vision complications resulting from the disease. This month, a group of researchers from the United States, Liberia, and Uganda have published data describing the ocular findings, visual impairment, and associated complications of Ebola in a group of survivors in Monrovia, Liberia.
They conclude that "survivors of Ebola virus disease (EVD) are at risk for uveitis (explained below), which may lead to eye damage, visual impairment, and blindness. Eye care resources should be mobilized for EVD survivors throughout West Africa, who are at ongoing risk of uveitis and severe vision loss, although the acute EVD outbreak has concluded."
From the Journal Ophthalmology
This new Ebola/eye disease research, titled Ophthalmic Manifestations and Causes of Vision Impairment in Ebola Virus Disease Survivors in Monrovia, Liberia, has been published online ahead of print in the December 2016 edition of Ophthalmology, the official journal of the American Academy of Ophthalmology. Ophthalmology publishes original, peer-reviewed research in ophthalmology, including new diagnostic and surgical techniques, the latest drug findings, and results of clinical trials.
The authors are Jessica G. Shantha, MD; Ian Crozier, MD; Brent R. Hayek, MD; Beau B. Bruce, MD, MPH; Catherine Gargu; Jerry Brown, MD; John Fankhauser, MD; and Steven Yeh, MD, who represent the following organizations and institutions: Emory University School of Medicine and Emory University, Atlanta, Georgia; the Infectious Diseases Institute, Kampala, Uganda; the Ministry of Health and Sanitation, Monrovia, Liberia; and Eternal Love Winning Africa Hospital, Monrovia, Liberia.
About the Ebola and Uveitis Research
Edited and excerpted from Vision impairment and eye diseases continue to be concerns for Ebola survivors, via Medical Xpress:
The acute outbreak of Ebola virus disease (EVD) has subsided in West Africa, but the medical community continues to learn about long-term complications for survivors – including the potential for blinding eye disease. One particular condition of concern is uveitis.
Uveitis refers to inflammation of the eye, which can lead to problems ranging from mildly reduced vision to severe vision loss and blindness. Patients with uveitis can experience eye redness, blurred vision, eye pain, headache and/or sensitivity to light.
The [study] reports on nearly 100 EVD survivors in Monrovia, Liberia. Liberia, along with Sierra Leone and Guinea, were the three countries most affected by the 2014 EVD outbreak. More than 10,000 cases of EVD were reported in Liberia alone during the recent outbreak; over 28,000 cases were reported during the West African EVD outbreak between 2013 and 2016.
Of the 96 patients examined, 21 had developed an EVD-associated uveitis and three developed an EVD-associated optic neuropathy [i.e., damage to the optic nerve]. In addition, nearly 40 percent of the patients with uveitis were legally blind. Other findings include cataract and [floaters/flashes of light], which can both be complications of untreated uveitis.
"These findings have implications from medical and surgical perspectives, particularly given our prior finding of live Ebola virus in the ocular fluid of a recovered Ebola survivor," said [study co-author] Steven Yeh, MD.
The public health risk in these cases lies in knowing what kinds of precautions health care workers should take when treating Ebola survivors who develop cataracts.
What is Ebola Virus Disease?
From the World Health Organization (WHO) Ebola key facts:
- Ebola virus disease (EVD), formerly known as Ebola hemorrhagic [i.e., bleeding, both internally and externally] fever, is a severe, often fatal illness in humans.
- The virus is transmitted to people from wild animals and spreads in the human population through human-to-human transmission.
- The average EVD case fatality rate is around 50%. Case fatality rates have varied from 25% to 90% in past outbreaks.
- The first EVD outbreaks occurred in remote villages in Central Africa, near tropical rainforests, but the most recent outbreak in West Africa has involved major urban as well as rural areas.
- Community engagement is key to successfully controlling outbreaks. Good outbreak control relies on applying a package of interventions, namely case management, surveillance and contact tracing, a good laboratory service, safe burials, and social mobilization.
- Early supportive care with rehydration, and [treatment of symptoms] improves survival.
- There is as yet no licensed treatment proven to neutralize the virus, but a range of blood, immunological, and drug therapies are under development.
- There are currently no licensed Ebola vaccines but two potential candidates are undergoing evaluation.
You can read more about Ebola transmission, symptoms, diagnosis, treatment, prevention, and controlling infection in health-care settings at Ebola virus disease: Fact sheet from WHO.
What is Uveitis?
An eye with anterior uveitis
Uveitis (you-vee-EYE-tis) is an internal inflammation of the eye, involving the middle layers of the eye, also called the uveal tract. The uveal tract contains veins and arteries that transport blood to the parts of the eye that are critical for vision:
- Ciliary body: Contains the ciliary muscles, which change the lens shape and curvature, and the ciliary processes, which produce aqueous humor, a clear, watery fluid that provides nutrients to all parts of the eye.
- Iris: A tissue inside the eye that has a hole in the center, forming the pupil. The iris contains muscles that allow the pupil to become larger (open up or dilate) and smaller (close up or constrict). The iris regulates the amount of light that enters the eye by adjusting the size of the pupil opening. The iris also determines eye color.
- Choroid: A dark brown membrane that is rich with blood vessels. It supplies blood and nutrients to the retina and nourishes all other structures within the eye.
Uveitis has many potential causes, including inflammatory disease affecting other parts of the body; a viral infection; bacteria; a fungal infection; a parasite, or an injury to the eye. Symptoms of uveitis include eye redness and irritation; blurred vision; eye pain; and increased sensitivity to light.
Complications of uveitis can include glaucoma; cataracts; abnormal growth of blood vessels in the eyes that interfere with vision; fluid within the retina; and vision loss. Early diagnosis and treatment is critical. See New Research: Uveitis, an Inflammatory Eye Disease, May Signal the Onset of Multiple Sclerosis for other potential complications.
More about the Ebola Study from Ophthalmology
From the study summary and abstract:
Purpose: To describe the ocular findings, visual impairment, and association of structural complications of uveitis with visual impairment in a cohort of survivors of Ebola virus disease (EVD) in Monrovia, Liberia.
Participants: Survivors of EVD who were evaluated in an ophthalmology clinic at Eternal Love Winning Africa (ELWA) Hospital in Monrovia, Liberia.
Methods: A cohort of EVD survivors who underwent baseline ophthalmic evaluation at ELWA Hospital were retrospectively [i.e., examining data and records that were collected in the past] reviewed for demographic information, length of Ebola treatment unit (ETU) stay, visual acuity (VA), and ophthalmic examination findings. For patients with uveitis, disease activity (active vs. inactive) and grade of inflammation were recorded according to Standardization of Uveitis Nomenclature criteria.
The level of VA impairment was categorized according to World Health Organization classification as follows: normal/mild, VA 20/70 or better; moderate, VA 20/70–20/200; severe, VA 20/200–20/400; blindness, VA less than 20/400. Visual acuity, length of ETU stay, and structural complications were compared between EVD survivors with and without uveitis. Structural complications associated with moderate VA impairment or poorer were analyzed.
Results: A total of 96 survivors of EVD were examined. A total of 21 patients developed an EVD-associated uveitis, and 3 patients developed an EVD-associated optic neuropathy. Visual acuity was blind in 38.5% of eyes with uveitis. Examination findings associated with at least moderate visual impairment by World Health Organization criteria (VA less than 20/70) included keratic precipitates [i.e., inflammatory deposits on the cornea]; posterior synechiae [i.e., the iris adhering to the lens]; vitritis [i.e., inflammation of the vitreous]; and chorioretinal scars [i.e., scarring or inflammation of the choroid and retina].
Conclusions: Survivors of EVD are at risk for uveitis, which may lead to secondary structural complications, visual impairment, and blindness. Eye care resources should be mobilized for EVD survivors in West Africa because of the frequency of this spectrum of disease complication and its potential for severe VA impairment and blindness.
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