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Parents often ask me when I will be able to tell if their baby can see normally. That's hard. But vision screening is something that we do at well-child visits if your child does not already see an eye doctor. Today, I'll discuss the basics of vision screening in kids.
Vision Screening for Children Under Age 3

For children under age 3, any vision concerns need to be referred to a pediatric ophthalmologist, who is a medical doctor who treats eye issues. Some optometrists, who are doctors of optometry, or ODs, will see kids as young as 6 months old, but not usually.

Now, as pediatricians, we look at the eyes of kids starting at birth. We look to see if they have congenital cataracts, if their red reflexes are good. You know, when you take a picture of your kid and they have really bright red pupils, that's actually a good thing. We'll see if they have lazy eye or any other eye concern for which we need to have them see a specialist. But it's hard to check in the office to see if they need glasses at that age.

The American Academy of Pediatrics last updated their vision screening guidelines in 2021. We start doing vision exams at well-child visits starting at the age of 3. I usually tell kids at their 2.5-year well-child visit that when they come back to see me at 3, we'll have them play a picture game with my medical assistants.

It's actually the screening for visual acuity, which is to evaluate to see if they need to see an eye doctor for possible glasses. But a picture game sounds a whole lot more fun.
Vision Screening for School-age Children

Kids up until age 4 normally have 20/40 vision, and by age 5, they will have 20/30 vision. After that, their vision should be 20/25 or better. 20/20 is what most people know as normal vision, and that's what they should ideally have by age 6.

Often, the schools will want a child's vision screened before kindergarten. They will also do vision screening in the schools periodically. The parents will be notified if their child fails their vision screen, and we can repeat the vision exam here to make sure of the results, especially if the screener at school did not document on the letter what the child's visual acuity was.

Then we can help the family find an optometrist that can do a more in-depth vision evaluation and see what kind of glasses the child may need.

The forms that the schools send home usually require a signature from an actual optometrist to prove that the child saw an eye doctor. As pediatricians, we are not qualified to complete those forms and they will be returned to the parents if we fill them out. So if your child gets one of those forms, they do need to see an actual eye doctor.
How Often Should Your Child Have Their Vision Screened?

We recommend that a child has their vision screened at least once a year. That's why we do them at the well-child visits, which also happen once a year.

If your child wears glasses or contacts, the optometrist will let them know how often they need to be seen. Normally, it's still every year to make sure their prescription has not changed.

If you have any questions about your child's vision, talk to your child's pediatrician and we will evaluate what we can and let you know if your child needs to see an optometrist or an ophthalmologist for further testing.

Interviewer: Are you working out from home with exercise bands? Well, you might want to watch out for this injury.

Dr. Troy Madsen is an Emergency Room physician at University of Utah Health. And a lot of us are trying to get in some exercise at home, and we might reach for those exercise bands. But, Dr. Madsen, I understand that there could be some risk working out with those exercise bands. Tell me more about that.

Dr. Madsen: You know, Scot, I have used exercise bands, and this is a risk I've never really considered, but apparently there is an increase in risk and injury to the eye that has been something that's been noted since the pandemic started. So what we're seeing, I think, more and more people are not going to the gym, they're working out from home, and a great tool is a resistance band. If you've ever used this, it's like a giant elastic band. You know, these things are huge. You put it around your foot, and then maybe you're leaning back or doing something with your leg, all kinds of different things, stretching, strengthening.

Well, at the University of Miami, they actually published their experience with seeing multiple patients come to the Emergency Department with injuries to their eyes from these resistance bands. So the title of this article is "Ocular Trauma Secondary to Exercise Resistance Bands During the COVID-19 Pandemic," published in the "American Journal of Emergency Medicine."

And you can imagine how this can happen. I don't know if this has ever happened to you, but let's say you wrap it around your foot, and you're stretching your leg out, and that thing is really tight. And then, maybe you've got socks on or something, and it slips off your foot and flips back and hits you in the eye.

Interviewer: Oh. Ow. Oh.

Dr. Madsen: Yeah, sounds miserable. Sounds absolutely miserable.

So they reported their experience in the "American Journal of Emergency Medicine," and they talked about 11 patients they had seen, and these were not minor injuries to the eye. So they said 11 patients, 14 eyes, so that means several of these patients had both eyes injured. Eighty-two percent of these patients had a hyphema.

So a hyphema is a pretty big deal. That's where you get blood behind the cornea. And, you know, if you ever look in the mirror, you see the cornea, you see your iris, the colored part of your eye. The cornea is the clear part over the top of that. And if you ever see blood there, it just looks like just this red line that's filling up behind there, that's a pretty big deal. That's a serious injury.

And then, vitreous hemorrhage in 36% of these patients. That's blood back behind the iris, back in kind of the main part of the eye. That can really affect your vision. Potentially, if it causes enough damage, potentially have long-term effects. Same thing with a hyphema if it's not treated.

So these are not minor injuries, but they saw a number of these, and just given the number they've seen, they reported on it in the "American Journal of Emergency Medicine" to make people aware that things are happening with resistance bands.

Interviewer: All right. So not happening to, necessarily, a large number of people that we know of, but is in the realm of possibility of happening apparently.

Dr. Madsen: Exactly. And I think the reason they published this and their conclusion was, if you're using a resistance band, wear glasses or consider wearing goggles. I mean, it may seem like overkill. It is something that emergency departments are seeing. This is one emergency department's experience. I'm sure it's happening elsewhere. I have to be honest. I have not seen this in the ER yet, but if we talk to some of our ophthalmologists, my guess is that they probably have. So it's out there, it's happening. You know, takeaway, be aware of it and consider wearing some glasses or goggles if you're using a resistance band.

Interviewer: Yeah, or consider just making sure that you're looking at how you're using it, and "If it was to slip right now, would it slip back and snap me in the eye?" And is there an adjustment you can do in your form that would, you know, prevent that from happening?

Dr. Madsen: Yeah, exactly.

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Interviewer: All right, today's patient question is from a gentleman named Ken. He says that his dad is starting to lose his eyesight, and they're concerned that his driving isn't as good as it needs to be.

So how do you have that conversation to take away the keys? It can be a scary thing to do. And to help answer this question we have Lisa Ord. She's a licensed clinical social worker. She also has a PhD in social work and is the Patient Support Program Director at the Moran Eye Center.

So how do you have that conversation?

Lisa: Having that conversation with one of your parents is probably one of the toughest conversations you're going to have, especially here in the West where we rely so much on driving and how much of our individual freedom is attached to be able to get in the car and go where you want when you want to. But the problem is that when someone isn't seeing as well as they need to be seeing, it gets frightening.

And it's not only frightening for them, but it's frightening for anybody else that's on the road or walking on the side of the road. So being able to have the conversation with your parents is very important. And to start with saying, "You know, dad, you've just had your eye checkup, and I see that it's not getting better. They can't correct it. Have you thought about other ways of getting to where you need to go other than driving yourself?"

Interviewer: And then you just pause and wait for that answer?

Lisa: And you pause and wait. Exactly. And it may be met with anger and just being able to be with that and say, "I know that this is a tough conversation, not something you even want to talk to me about." But the tougher conversation is the conversation after you've hit somebody because you didn't see them.

Interviewer: So this is a conversation that if you feel that somebody that you love is not seeing well, you need to have a . . . you outlaid a very direct approach. Are there other ways, if you don't think the direct approach is going to work with your particular loved one?

Lisa: I've had patients use different approaches. One patient said, excuse me, one daughter of one of my patients said that she finally said, "My children will not ride with you in the car. We either need to have someone else drive, or you're not going to be able to take them where you want to take them." So it was kind of like putting it in terms of, "I don't feel safe enough to be in the car with you. I don't feel safe enough for you to take my children in the car with you."

Interviewer: And that really kind of brought the reality to bear?

Lisa: To bear, yeah.

Interviewer: Is there somebody else you could involve, like a health care professional perhaps, because maybe they would be more willing to take it from a third party?

Lisa: I have a lot of patients that say, "But my license doesn't expire for another two years, so I don't have to worry about it." And that's not the case. You're still going to be very much liable whether your license is expired or not. If you're not safe to be driving, your eye care professional will have to fill out a medical form stating what your visual acuity is, and your visual field if you are not being able to be corrected to 20/40 or better. So that is something that they're going to have to do. Having the health provider have that conversation with your parent is sometimes easier because it does kind of put that onus on the healthcare professional.

The other thing is that if you really can't have the conversation, you really are concerned about your parents' driving or anyone's driving, you can make a report to the DMV, and they will take it upon themselves to have that person do a driving test.

Interviewer: Okay. All right. Probably not the way that most people would want to handle it. But I guess as a last resort . . .

Lisa: As a last resort. And I have had some family members who say their parents are so adamant that they're, you know, having extra keys made every time the keys are taken away from them, things like this. And so you're left with no other resort, except for to call in the people whose ultimate responsibility is to take away the license, and that is the driver's license division.

Interviewer: Ultimately, though, if somebody is faced with a vision impairment that could endanger them or others while driving, whatever you do, you would recommend something has to be done.

Lisa: Yes.

Interviewer: Don't leave it to chance.

Lisa: Don't leave it to chance. Not a good plan.

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Interviewer: When first faced with the reality of vision loss, life can seem overwhelming. Denial, anger, fear, grief, hurt, rejection, abandonment, those are just some of the common psychological reactions that somebody could have when faced with the reality of life without or just even with limited sight. And without proper guidance, in many cases there's a great potential for isolation, depression, and dependence. That's why it is important to intervene early if somebody's losing their vision to help avoid some of these things happening.

Lisa Ord's a licensed clinical social worker. She also has a PhD in social work and is the Patient Support Program Director at the Moran Eye Center. It sounds like that vision loss is a lot more than just a physical issue, which a lot of people obviously know. There's a lot of psychological and mental stuff going on that we might not be aware of.

Dr. Ord: It really does affect every aspect of your life. It affects how you see yourself. It affects your roles with other people. You have to grieve it.

Interviewer: Yeah. And it affects your family. It affects the individual. So I'm somebody that has a loved one that's going through vision loss. And now, because of this first little bit here, I realize that there's a lot more going on than just the physical loss of sight. So how could I better support a loved one going through this?

Dr. Ord: I think the first thing you need to do is acknowledge with them that, you know, Mom, Dad, I know that you don't see as well as you used to, and I know that that can also affect other things that you've got going on in your life, and I want to know how I can best help you if you do want help, or help you to learn how to do what you want to do without as much sight as you've had.

Interviewer: Got you. So just open that dialogue.

Dr. Ord: Open the dialogue.

Interviewer: Do you find that most times that people are pretty responsive with that, or are they like my parents? They'd be like, "Oh, I'm fine."

Dr. Ord: It's the generation. Isn't it? You know, some are, some aren't really. But even if they, you know, "I'm fine," to you, they've heard you, and they know that you're aware and that you're there and that you want to help.

Interviewer: How much should you push the conversation at that point?

Dr. Ord: I would never push too hard. I have patients who say, you know, "My family, they won't leave me alone."

Interviewer: Oh, yeah? And that's all they want, is just leave me alone.

Dr. Ord: And there's others, they're just like, you know, "I really need some help, and there's nowhere to be found." So there's a happy medium.

Interviewer: That's right. Okay. So do you have any tips on how to approach the conversation? I think you gave us one already, just open up with, "I understand that this can be an issue that can lead to other issues. How are you doing? How are you feeling? Can I help you?" What other tips?

Dr. Ord: Exactly. I think, you know, acknowledging that maybe what you've been seeing in the anxiety of, "Am I going totally blind, or am I just losing some of my vision? What's going to happen? How am I going to manage?" Those kind of conversations I think are super important to have and just opening up to them and saying, you know, "I've been reading. I've heard that things can be very difficult. And how are you doing? How are you feeling about this?" Allow them and encourage them to continue doing the things that they want to do. And there is a way of doing everything if you know how to do it without sight. Sometimes they just need some tips and tricks on how to do something without being able to see it as well as they have in the past.

Interviewer: So tell me about some of the programs that you have at the Moran Eye Center.

Dr. Ord: So we have support groups. It's very important they get a lot from being able to talk with other people going through very similar things that the individual is going through at the time. We also have psychotherapy because it is a loss. You need to grieve it. There's a lot of anxiety. There's a lot of depression, so we do counseling. We also do skills, some basic vision loss skills and how to do things. We have an orientation to vision loss once a month on a Saturday that we talk about magnification, lighting, contrast, using your other senses, giving some tips on how to do things in a different way.

Interviewer: Yeah, my dad had macular degeneration, and I didn't really realize any of these things existed, and it would have been great to have had access to that sort of thing. We're very lucky here in Salt Lake City to have access to, you know, those resources at Moran Eye Center. Somebody who's not in Salt Lake City, how can they find resources?

Dr. Ord: There is a great website. It's the American Foundation for the Blind or afb.org, and there is a directory that you can search by state to find services in your area. Every state will have some kind of services for the visually impaired and blind. Sometimes it goes through the Office of Rehabilitation, sometimes it goes through the Office of Education, but every state will have some services.

Interviewer: And it sounds like people that use these resources find them very beneficial.

Dr. Ord: Oh, very beneficial.

Interviewer: It might be difficult to get somebody there at first but well worth it.

Dr. Ord: Absolutely. If you're losing your vision, it's okay to really have some strong emotions about it. It's okay. Then it comes to a point where it's like, "Okay, what am I going to do about it?" So then you take the next steps in learning how to live life fully, even with your vision loss.

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Interviewer: Broken blood vessel in the eye. Dr. Troy Madsen, ER or not?

Dr. Madsen: Well, a broken blood vessel in the eye is one of those things that's just absolutely obvious to everyone you see. Everyone is going to ask you about it, say, "What happened to your eye?" It's essentially just a bruise on the eye. If it happened on your hand and you had a bruise there, no one would ask about it, but the blood vessels in the eye are so small that, sometimes, maybe while you're sleeping, you just turn wrong and bump something, and it causes a little bruise there. You don't need to go to the ER for it.

Now, if you've had significant trauma to the eye, you've been hit in the eye, if you notice that there's blood behind the cornea, so in front of the colored part of your eye, that's much more concerning. But if you just wake up one day, you've got some red on your eye, it's going to heal up after a few days. No need to rush to the ER.

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Interviewer: When you think of vision correction surgery, most people think of Lasik. It's not the only option. We'll discuss what the other treatments might be next on The Scope.

Announcer: Medical news and research from University of Utah physicians and specialists you can use for a happier and healthier life. You're listening to The Scope.

Interviewer: We are in the office of Dr. Amy Lin today. She's an ophthalmologist at the Moran Eye Center at the University of Utah. Dr. Lin, first of all, tell me why someone would even need to go get Lasik.

Dr. Lin: People get Lasik to get out of their glasses or contact lenses. They want to correct their nearsightedness or their farsightedness or their astigmatism. That's why someone would want to have Lasik.

Interviewer: That's not the only option, I'm hearing. There are other surgery options besides Lasik.

Dr. Lin: That's correct. The most common alternative to Lasik is something called PRK. PRK was actually the precursor to Lasik, but we still do a lot of PRK nowadays because there are certain advantages with PRK. And it does the same thing as Lasik, corrects nearsightedness and farsightedness and astigmatism. Instead of having a flap in the cornea like there is with Lasik. With PRK, there is no flap in the cornea, but your eye has to heal over naturally.

Interviewer: When a patient comes to the office and they ask you for suggestions of what treatments and what surgery they should do, how do you decide Lasik is better for you or PRK?

Dr. Lin: We do a whole variety of measurements in the office. We measure the steepness and the shape of the cornea. We measure the thickness. We measure the prescription in the eyes. And based off of that data, we decide is the cornea thick enough for Lasik and PRK because you do need a thicker cornea for Lasik. Is the prescription too high for Lasik and maybe still ok for PRK? That's kind of one objective measure that we have for choosing one or the other.

There are other parameters that we look at. We actually look at the patient and if they have a lot of dryness in their eyes, like they can't wear the contact lenses for a long time because their eyes become too dry, with Lasik, we know that you get a lot of dry eye afterwards than with PRK. If you have dry eye existing, it may be a better option to go with PRK rather than Lasik so you don't worsen your dry eye.

Interviewer: When your doctor tells you that they recommend PRK as your treatment, does that mean that you are not a candidate for Lasik? Can you not do Lasik if you are recommended PRK?

Dr. Lin: Usually, people are either candidates for both or candidates for just PRK. If a doctor recommends to you that they recommend PRK, usually, it means there's something that usually bothers them in Lasik and they think it might be too risky to do Lasik, but it would be safe to do PRK.

Interviewer: Now, are the outcomes of both of the surgeries the same?

Dr. Lin: Yes, the outcomes are the same. When they do the studies that compare PRK versus Lasik, the visual outcomes are the same. PRK takes a lot longer to heal whereas Lasik is a lot faster. Lasik people are saying well after a day or so. With PRK, it takes several weeks. That's not to say that you're blind for several weeks. It's just not to be quite as crisp and clear for several weeks, but the vision does get there. Interviewer: With gradual outcome.

Dr. Lin: Exactly.

Interviewer: With Lasik, from what I understand, there is a laser involved that corrects your eye vision for you. Tell me about PRK. Is that the same thing? Is there a laser involved or is it some totally different procedure?

Dr. Lin: Both Lasik and PRK have a laser involved. With Lasik, there are actually two lasers involved. There is one laser that cuts a flap and the cornea and then, there is a second laser that corrects for the vision. And with PRK, we just use the laser that corrects for the vision. After the laser procedure, with PRK, a bandage contact lens is actually put on the eye and that contact lens is kept in the eye for several days so that your eye can heal. Whereas with Lasik, there isn't any extra material put on your eye. Your eye kind of . . . it's fast and your eye is almost kind of healed at that point. There's really nothing to cover up.

Interviewer: Is there one that you would prefer over the other, in terms of their kind of better outcome long-term.

Dr. Lin: PRK could be a little bit safer and the reason is that with Lasik, there's kind of a long life risk of having additional damage to your eye if your eye gets hit really hard. We're talking hard injury like a car accident, baseball to the eye, a big fall, something like that because the cornea isn't that 100% strength. There could be additional injuries to the eye with whatever injury hits you in the eye, but if you have PRK and you get hit in your eye later on, any eye injury you would have wouldn't be any different than getting hit in your eye right now.

Interviewer: PRK is not a surgery that a patient could come into your office and say, "I want this surgery." It's something that you need to evaluate and it's a doctor-prescribed treatment?

Dr. Lin: Exactly. PRK is an elective surgery, but we still need to see if you are a candidate for it. But some people are not candidates for Lasik and some people are not candidates for Lasik or PRK. I think they're both great procedures and the only way to for you to determine that is to see a doctor, get all the testing to see if you're a candidate.

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Dr. Miller: Do you know the difference between the cornea and the lens of your eyes? We're going to talk about that next on Scope Radio.

Announcer: Access to our experts with in-depth information about the biggest health issues facing you today. The Specialists, with Dr. Tom Miller, is on The Scope.

Dr. Miller: Hi, I'm Dr. Tom Miller and I'm here with Bala Ambati, and he is a Professor of Ophthalmology here at the University of Utah. Bala, tell us a little bit about the difference between the lens of the eye and the cornea of the eye for those who maybe don't know much about that.

Dr. Ambati: No one in the listening audience probably remembers a film camera, but the camera has many parts to it. Just like a camera, the eye has a focusing part in the front of the eye, and the film of the camera, in the case of the eye, is the retina.

Dr. Miller: The back of the eye.

Dr. Ambati: The back of the eye, exactly. So if you look just at the front part of the eye, there are two main structures that focus light: the cornea, which is the front clear window of the eye. And that's the part that sparkles when you're sitting across somebody that you care about in a romantic restaurant.

Dr. Miller: That hasn't happened for a while, but I like the idea of it.

Dr. Ambati: And then behind that . . .

Dr. Miller: Not true, not true. For my wife, if she's listening.

Dr. Ambati: Behind the cornea is the colored part of the eye, the iris, and behind that is the lens. And the cornea actually provides protection for the eye. It's the clear window of the eye. It provides two-thirds of the focusing power of the eye.

Dr. Miller: The cornea does?

Dr. Ambati: It does, more so than the lens. The lens provides the last third, but the lens provides what's adjustable. The lens is what helps you focus from distance to up close and then back out again. And so the lens provides the swing, in terms of accommodation and in terms of changing focus, and the cornea is providing structural protection and most of the focusing power.

Dr. Miller: So it sounds like the cornea is the anterior-most portion of the eye . . .

Dr. Ambati: Indeed.

Dr. Miller: Or the portion that is exposed to the environment . . .

Dr. Ambati: Absolutely.

Dr. Miller: So this is the part that can become dry or irritated. There can be problems with allergies. It's the front-facing piece, is that correct?

Dr. Ambati: Absolutely. The cornea is the window of the eye and it has to protect the rest of the eye from anything that hits it, whether it's speeding steel or allergy or infections.

Dr. Miller: So also, the cornea is exposed to tears and tears, I guess, lubricate the cornea and the eyelids. Is that . . .

Dr. Ambati: Indeed. The eye works because you have all of these intricate structures working together. The eyelids provide a windshield wiper as well as producing tears, which lubricate the cornea and keep it smooth.

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Announcer: Examining the latest research and telling you about the latest breakthroughs, the science and research show is on the Scope.

Interviewer: My guest Dr. Wolfgang Baehr says you have to be fearless to make advances in science. He researches the genetic causes of blindness and has just been given a career award from ARVO, the Association for Research in Vision and Ophthalmology. You've been in the same field for about 30 years..

Dr. Wolfgang Baehr: Almost 40.

Interviewer: Almost 40..

Dr. Wolfgang Baehr: 1976.

Interviewer: Oh, right. 1976, yeah. What has been the most exciting moment of your career?

Dr. Wolfgang Baehr: Well, it's kind of hard, I have published about 200 papers, each one was a major, not each one but most of them were hard work. And, in the last 5 years I discovered it is very difficult to publish, even, a good paper. One of my best papers in Nature Neuroscience about two years ago, we worked five years on this project. And tried eight different [terms], we started with a top major in science, and it was very frustrating. But, eventually, we got it published in Nature Neuroscience. It was a wonderful paper and that was very satisfying. So persistence is something which is really my characteristic. We don't give up.

Interviewer: Do you think that you approach science any differently than some of your colleagues?

Dr. Wolfgang Baehr: Well, I think I do because I'm trained as an organic chemist, with basic science education and when I finished my PhD I basically decided to drop chemistry and learn something else, and I got into biophysics, fast kinetics, Stopped-flow kinetics and eventually into biochemistry. And then I came to the United States as a post doc and got into retinal research, so that's really when our research started back in 1976. During that time we started to work on phototransduction, which is the mechanism that starts to generate an electrical impulse in the retina, which is transmitted through the optic nerve to the brain, which is initially [meant] for vision.

Interviewer: What are some of the most striking ways that your field has changed over the last 30 years?

Dr. Wolfgang Baehr: At that time phototranduction was completely unknown, and at that time biochemistry was the thing to do to identify [inaudible 00:02:23] to identify G-proteins like transducing and eventually the main components we identified and then molecular biology started, DNA sequencing became possible in the 1970s, so I was one of the first starting sequencing trials, in [inaudible 02:46] for example. And then in 1990 the first mutation linked to autosomal dominant retinitis pigmentosa was identified in rhodopsin. And we decided to take this mutation, make the same mutation in mouse, and the mouse would [generate] the molecule internally to transgenic mouse, which was really not easy in those times.

Interviewer: One impression that I have is that you're not afraid to jump in with both feet.

Dr. Wolfgang Baehr: You have to be fearless. And just try and go and see this new gene published and new mutations. So we are very quick to make a knockout because it's relatively easy in mice.

Interviewer: Well, right, and this is something that seems to make you stand out from others..

Dr. Wolfgang Baehr: Yes.

Interviewer: Is that you do make these mouse models for genetic retinal diseases.

Dr. Wolfgang Baehr: We've probably knocked out 20 different genes in the last 10 years, and many of those models were first and unique. Nobody had ever produced any of those mice.

Interviewer: Can you give one example of a mouse model and what defects it had and how you were able to reverse some of those?

Dr. Wolfgang Baehr: Well, the best mouse model would be the dominant disease caused by a mutation in a calcium binding protein, which is called guanylate cyclase activating protein, called GCAP for short.

Interviewer: So, basically, if there's a mutation in this GCAP it causes a dominant disease and the protein is overactive, correct?

Dr. Wolfgang Baehr: Protein is overly active, but using a molecule cGMP, which is damaging photoreceptors. We depend on cone vision for color vision, acuity, reading newspapers is all dependent on cones, and this disease destroys first, cones. So, you basically become photophobic because light intensity is very damaging. And you start having problems with reading newspapers, begin central vision problems and you wear sunglasses. So it's very difficult for patients to very slowly lose cone vision and visual acuity and then slowly becoming blind. And mice being inoculated with AV virus that knocks down the mRNA...

Interviewer: When you're knocking down the mRNA, what happens is that there's less protein being made, right?

Dr. Wolfgang Baehr: It's less damaging protein being made and the [inaudible 05:22] of the retina is not damaged.

Interviewer: Right. So, you're taking away the problem in the first place.

Dr. Wolfgang Baehr: We can slow down the damage and have a very positive effect after one year, in terms of visual acuity and cone photo function is way improved. So in a mouse model it works beautifully, and the additional advantage that we have with this construct is that it is applicable to any mutation in GCAP, there are about 2,000 families worldwide, which have been identified so far, with 100 to 150 patients. All of those patients could use the same [inaudible 06:01] virus with our knock-down construct and could have beneficial health experiences and slow down the disease dramatically. The point where we are right now is, is it worthwhile to do clinical trials, there's a very small patient collection...

Interviewer: Right.

Dr. Wolfgang Baehr: In different parts of the country or the world. And, as you know, will there be a company that supports us, and that's the question I don't know.

Interviewer: Another aspect, of course, of your work is that you're understanding the whole process of phototransduction and getting new insights into how that works..

Dr. Wolfgang Baehr: Yes, how that works. In the beginning we know all details, and my interest really, is to understand mechanisms leading to disease, and I think we might essentially, each gene is a new area, [of possibilities]

Interviewer: What keeps you fascinated in this work?

Dr. Wolfgang Baehr: Because there's always something new. We've focused, of course, at genes very specific for photoreceptors, but eventually we got into [Centronics dystrophy] like effecting multiple tissues. You know, cut a gene and some of those genes would cause embryonic lethality, mice would never be born; or would die early. And, of course, that's much more complicated, you cannot do just retinal research, you have to look into kidneys, for example. And that is, of course, not my field, so I need collaborators.

Interviewer: Yeah. Yeah.

Dr. Wolfgang Baehr: But that makes it more interesting because those diseases are very, very devastating. But it's also expanding research and making it more interesting, you know. Getting into new areas, being [fielders] and being persistent I think that's the characteristic in which we can demonstrate.

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Man: Medical news and research from the University of Utah physicians and specialists you can use for a happier and healthier life. You're listening to The Scope.

Interviewer: Dr. Paul Bernstein at the Moran Eye Center. Let's talk about some of the trials and some of the work and research that have been done into AMD and what you've discovered.

Dr. Paul Bernstein: Okay. We at the Moran Eye Center have been very involved in many of the key clinical studies for age-related macular degeneration. One of our primary treatments is a drug called Lucentis, which involves injections once a month of a medicine into the eye every month for age-related macular degeneration for the wet form.

Interviewer: And that's where the veins are starting to come into the eye?

Dr. Paul Bernstein: That's correct.

Interviewer: What does this medication do? Does it just stop that?

Dr. Paul Bernstein: The Lucentis and similar drugs, when injected into the eye, combat the growth of blood vessels. They actually interact with the signal to grow blood vessels, a compound called VEGF or vascular endothelial growth factor.

Interviewer: We learned in our previous podcast that what's going on there is that those cells are sending those signals to grow those blood vessels, and this tells it to stop.

Dr. Paul Bernstein: Yes.

Interviewer: Okay.

Dr. Paul Bernstein: It just blocks the signal right there.

Interviewer: Gotcha.

Dr. Paul Bernstein: It binds them, and the signal can no longer interact with the receptor to grow the blood vessels, and the blood vessels that are abnormal begin to get smaller and kind of wither away. After the drug wears off in a month or so, we have to give another injection into the eye.
So, we're trying to develop longer acting versions of the drug, and we're involved in some early stage clinical trial planning of either reservoirs that can be implanted into the eye that will slowly release a compound that would block vascular endothelial growth factor. We're also trying to look at different ways to get the medicine across the sclera, across the white part of the eye instead of using a needle, trying to use electrical impulses to get the drug to cross the eye.

Interviewer: Interesting.

Dr. Paul Bernstein: We would love to have a drug that could be just given as an eye drop. As you know, we have many medicines we give as eye drops. The challenge is that the retina is the back of the eye, and trying to get a drug to go all the way back and interact with the retina is difficult. Drug companies are very actively trying to improve that process.

Interviewer: So I suppose a pill, an orally taken thing, is just completely out of the question?

Dr. Paul Bernstein: People are looking at pills for macular degeneration that could combat the growth of blood vessels. The process is the macula and the eye is a very small part of the body.

Interviewer: Yeah.

Dr. Paul Bernstein: You have to worry about side effects and other reactions.

Interviewer: Sure.

Dr. Paul Bernstein: We certainly like the idea of local delivery, of targeted delivery to the eye.

Interviewer: Yeah. That's interesting. And what other kind of research of trials are you involved in or do you have going on?

Dr. Paul Bernstein: Well, we were one of the centers for the Age-Related Eye Disease Study, too, the AREDS 2 Study. We're really trying to understand the role of nutrition and nutritional supplements in age-related macular degeneration. This was a very large trial. There were nearly 100 centers across the country. Over 5,000 patients were in the study. It lasted five years. They had to come in every few months for eye examinations and to be given their supplements or placebos. It was definitely a placebo-controlled trial to really understand how these supplements work.

Interviewer: In a situation like that, I think you gave me a hint. How would you even know where to start and what to look for as far as nutrition that might be helpful? It sounds like you said these two things are high concentration in the eye, and that's where you started.

Dr. Paul Bernstein: Yes. So, the lutein and zeaxanthin are particularly interesting because the macula, which we all hear about for macular degeneration, is technically the macula lutea, which means "yellow spot" in Latin. And the macula uniquely in humans and fellow primates concentrates yellow compounds from our diet, the lutein and zeaxanthin. So I was always fascinated as to why nature went out of its way to put these two compounds that are antioxidants and light screening compounds directly into the back of the eye.

Interviewer: So somebody would take this supplement, and you would look to see if the concentration increased in their eye. Is that what you were looking for?

Dr. Paul Bernstein: That was in part. We were a sub-study in the trial to do measurements of the macular pigment levels, but ultimately for a national eye institute sponsored study, we're looking to see if it is effective with whether we can decrease the rate of progression to advanced age-related macular degeneration. That was the ultimate endpoint in the study. We have to see that we are getting an effect.

Interviewer: Yeah. Because if you increase those compounds, who cares if it's not solving the problem?

Dr. Paul Bernstein: That's exactly right.

Interviewer: Yeah. Interesting. What else do you have going on? You mentioned something on the horizon. Can you talk about that a little bit?

Dr. Paul Bernstein: We are looking at some other trials trying to interact with the immune system and the complement system. There is some exciting work as some of my colleagues here in the Genetics have discovered that the inflammatory system in the eye may be part of macular degeneration. So drug companies are developing new compounds that could interact with the complement system. Those are very early stage trials, and we're very hopeful that we can get involved in preventing macular degeneration earlier.
Other studies are to improve the compounds that we use to treat wet macular degeneration by doing combinations. Just like in cancer and chemotherapy, we know that there are multiple pathways that need to be attacked, and we are about to be a part of a new study that will be involved in even a second injection in the eye of a different compound. So it's a large commitment for patients to be in studies such as that.

Interviewer: So what's the theory there with the inflammatory system that's causing the problem? Is that, like, inflammation in other parts of my body, but it's happening in my eye? What would cause that?

Dr. Paul Bernstein: Inflammation as part of macular degeneration relates, we think, to the formation of drusen, the yellowish deposits we see underneath the retina. That is a very important sign of early macular degeneration. This causes damage to the cells and also contributes to the stimulus of new blood vessel growth underneath the retina.

Interviewer: So the thought that this is happening in the immune system that's trying to fight it is actually causing more harm than good. Is that what I'm hearing?

Dr. Paul Bernstein: We think that there may be a problem, and modifying that, that's what we need to test in these trials.

Interviewer: Any final thoughts for our listeners about the future of research trials?

Dr. Paul Bernstein: We always are looking to improve treatments. We've been very gratified by the improvements that have occurred in the last two decades for macular degeneration. We especially appreciate the commitment that the patients who join these trials have committed to this to trying to help both themselves and to others.

Interviewer: Could somebody listening be part of one of these trials? How would they make that happen?

Dr. Paul Bernstein: They need to talk with their doctor.

Interviewer: Okay.

Dr. Paul Bernstein: If you have macular degeneration, the best thing is to ask your doctor. Ask what trials are available, and how they could be improved on the standard therapy that we have now.

Man: We're your daily dose of science, conversation, medicine. This is The Scope, University of Utah Health Sciences Radio.

Interviewer: If you've had a relative with macular degeneration you know how heartbreaking it can be to watch them lose their sight; and a little terrifying for you, too, because you've got to wonder if you're next. We're doing to discuss that next on The Scope.

Recording: Medical news and research from University Utah Physicians and Specialists you can use for a happier healthier life. You're listening to The Scope.

Interviewer: Coming up today on The Scope we're going to talk about macular degeneration. We're going to cover what it is, what are the risk factors that could help you get it, what you can do to prevent it, and some possible treatments. We're with Dr. Paul Bernstein of the Moran Eye Center.
First of all macular degeneration, or also called AMD, what's going on? There are two different types if I understand correctly.

Dr. Paul Bernstein: Yes, age related macular degeneration is the leading cause of blindness in the developing world. And there are two types and it affects the central part of vision called the macula. The macula is the area of the eye that is responsible for reading, driving, recognizing faces, and in age related macular degeneration there is a dry form, which is a slow form that progresses slowly over years if not even decades, it can eventually lead to central blindness in the eye.
And there is the wet form which involves growth of new blood vessels under the retina and this can have a much more rapid course and lead to loss of vision in a matter of days or weeks.

Interviewer: So the dry form, what exactly is happening in the eye?

Dr. Paul Bernstein: In the dry form initially it starts with deposits forming underneath the retina; something that we call drusen that are yellowish spots under the retina. The patient may have completely normal vision, and not have any symptoms. But eventually there is more malfunction of the cells of the retina and some of them begin to die in the center of the macula and that is why the center of the retina no longer senses light properly, and that can lead to blindness.

Interviewer: So it's like a camera not being able to sense light?

Dr. Paul Bernstein: That's correct; it's a problem of a camera with bad film.

Interviewer: And in the wet you're actually getting some growth back there and that's blocking your vision?

Dr. Paul Bernstein: That is disturbing the vision.

Interviewer: Okay.

Dr. Paul Bernstein: It's interrupting the retina from having contact with its supporting cells, and eventually this can damage both types of cells as these blood vessels get in between these two layers and they can bleed. They can leak fluid, and eventually they can form scars and that's what leads to loss of vision there.

Interviewer: What's going on? Why are those starting to grow there?

Dr. Paul Bernstein: We don't know completely, but we understand that with age there is some malfunction of the cells and they for reasons that we don't completely understand start sending out signals and that seems to be an important factor for causing these blood vessels to start to grow underneath the retina. So it's kind of a malfunction; it's the wrong signals being sent out, that's correct.

Interviewer: All right, so who is at risk for age related macular degeneration? I think the name kind of gives you some sort of an indication?

Dr. Paul Bernstein: Yes, there are many risk factors, and certainly age is one of the most important ones. We know that age related macular degeneration is not very common in the people in the 50 to 60 year old range. Only a couple percent of people show the signs, but as we age it rises almost exponentially so that over age 75 at least 30% to 35% of people show at least some signs of age related macular degeneration.

Interviewer: So it's hereditary, so if you get old enough you're just going to get it?

Dr. Paul Bernstein: Well, there are a number of important risk factors for age related macular degeneration. And these genes are risk factors and we know of two very important ones; one on chromosome 1 related to complement and inflammation. And another one on chromosome 10 that we still don't quite understand, but if you have the wrong combination of genes that you've inherited from your parents it can put you at much, much higher risk. It can raise your risk three, five, even tenfold higher than the average population.
Other things that we also know as risk factors include being Caucasian; it is much more common in light skinned populations to have age related macular degeneration. And we also know in terms of modifiable risk factors that smoking is the number one modifiable risk factor. If you are a smoker, it increases your risk of developing the wet form of macular degeneration many fold and we certainly encourage our patients not to smoke.
And then the other area that I'm quite interested in is nutrition. We've learned that nutrition is very important in helping to prevent age related macular degeneration; specifically dark green leafy vegetables, orange and yellow fruits and vegetables, and cold water fish are certainly things that we recommend even in children at risk for age related macular degeneration because their parents have macular degeneration. And in the patients with macular degeneration we often recommend that they take supplements because it's often hard to change diet late in life.

Interviewer: How much do you know about the direct causation of diet?

Dr. Paul Bernstein: We know that diet is very important. And there is accumulating research that modifying diet can change the levels of the lutein and zeaxanthin which are from the dark green leafy vegetables that are actually accumulated in the eye. So we know that diet can make a difference, but as I said, when people are older changing their diet is difficult and that's why through the Areds 2 Study...

Interviewer: And what does AREDS stand for?

Dr. Paul Bernstein: AREDS stands for Age Related Eye Disease Study.

Interviewer: Okay.

Dr. Paul Bernstein: And that most recently specifically looked at adding lutein and zeaxanthin, and omega 3 supplements to the vitamins that we already knew were effective against age related macular degeneration.

Interviewer: So what are some other things that you can do then? So you have these supplements, and do you really feel they are effective from what you've seen?

Dr. Paul Bernstein: I think they are, and I certainly . . . it's a big part of my practice, encouraging patients to take these supplements, because often my patients present they've lost vision in one eye, and they are very concerned that it's going to affect their other eye, because you can get by with good vision as long as you have good vision in one eye. But if you have age related macular degeneration that's significant in both eyes, it can be devastating to these otherwise well-functioning patients. We encourage them not to smoke. We also think that there is a negative role for excessive sunlight, so we encourage patients to wear sunglasses on bright sunny days. That's an easy thing to do.

Interviewer: And it sounds like all of these things that you're currently recommending are really pretty low impact? It's not like anything too serious. What's the risk of taking a few vitamins?

Dr. Paul Bernstein: We think that lutein, zeaxanthin, and the basic AREDS vitamins are very safe and few patients complain of side effects.
The other thing that I encourage my patients is to monitor their vision and to learn the early signs of age related macular degeneration, especially the conversion from the dry form to the wet form. With the wet form of macular degeneration we have developed very effective treatments now for this once almost universally blinding form of macular degeneration.

Interviewer: The wet form is actually treatable; dry form once it's started, not so much. Is that correct?

Dr. Paul Bernstein: That's correct. We are certainly looking at new treatments for the dry form, but currently the standard of care is just antioxidant vitamins and good diet.

Interviewer: Does the wet form always come before the dry form, or the dry form before the wet form?

Dr. Paul Bernstein: The dry form comes before the wet form. Okay, so and they almost always start with the dry form of macular degeneration, the slow form. The wet form can then rear its ugly head and we have to treat it and sometimes we get it back under control and still the dry form slowly progresses over time. Macular degeneration is a manageable disease; we'd like to make it a curable and a preventable disease, and that's what we're working on here.

Interviewer: If I have a family history of it can I get a genetic test and can you actually see if I have the gene mutations you were talking about?

Dr. Paul Bernstein: As a research tool, yes. We can and there are models that can predict that you have an increased risk. But it is I would caution a risk assessment, so not everyone even with the relatively high risk develops macular degeneration and other people develop macular degeneration for reasons that we don't completely understand. We are trying to figure out the role of the test still in clinical practice and in helping counsel people and that's part of further research that we will be conducting.

Recording: We're your daily dose of science, conversation, medicine; this is The Scope, University of Utah Health Sciences Radio.

Interviewer: What is a physician trying to find when they look into your eyes? That's next on The Scope.

Announcer: Medical news and research from University of Utah physicians and specialists you can use for a happier and healthier life. You're listening to The Scope.

Interviewer: A lot of times on TV you'll see, or if you've ever even gone to the doctor yourself, they got the little flashlight and they start looking in your eyes and I've always wondered, what are they looking for? We're with Dr. Troy Madsen, emergency medicine at the University of Utah Hospital. In your particular situation, in the emergency room, if you get out the light and are looking into somebody's eyes, what are you trying to figure out?

Dr. Madsen:It's going to vary depending on who I'm looking at. But it's just part of a standard physical exam that when I see a patient I will document something that says on the chart, PERRL. What that stands for is the pupils are equal and reactive to light. And the pupil is the black part of your eye so I'm looking at that. I'm looking at are they the same size, and when I shine a light into it does it close? Does it react to that light and constrict like you'd expect?

And the relevance of that kind of varies from person to person. I mean, in the average person, it's not really a big issue. I can just kind of look at you and look at your eyes and say, "Oh, yeah, they look fine." But in different situations I'm looking for different things.

So if someone comes in after a head injury and they've been in a trauma, I really want to get a good look at those eyes to make sure the pupils are equal, because if they're not, that can be the sign of potentially something very serious in the brain that is affecting the brain's ability to send that message to the eye to have that pupil squeeze down and constrict. That can be a sign of some kind of bleeding in the brain, which is the more serious thing I'm really looking for there. So that's kind of the number one thing I'm looking when I do that.

The other thing I'm looking for often times, and this is a tough thing to do sometimes in the E.R., but sometimes I'll try and get a look at the back of the eye at what's called the fundus of the eye, called a fundoscopic exam, where I'm looking at optic nerve, so where the nerve inserts into the back of the eye. And if a person has a lot of pressure in their brain from bleeding in the brain or something like that, I can actually see swelling on that nerve. So that for me says this person potentially has something that's raising the pressure in their brain, like bleeding, a tumor, something like that. So that's kind of the other big thing I'm looking for when I do that.

Interviewer: All right. So two reasons you would look into somebody's eyes, none of them related to the eyes. Are there things you're looking in somebody's eyes for if they have an eye issue?

Dr. Madsen: Oh, certainly. Yep. And that's one of these things where if someone . . . and usually there I need to have something that's going to push me toward that, someone saying I'm having a lot of pain in my eye or I feel like just something is scratching my eye. And there, I'm going to do an even more detailed exam. I'll kind of flip their eyelid out, kind of like kids do to gross people out. So I'm doing that to look for some kind of piece of dirt or a splinter or something like that in the eyelid itself that's scratching the eye.

Interviewer: And that actually happens?

Dr. Madsen: It does.

Interviewer: That's gross.

Dr. Madsen: Oh, it does, yeah. And then I'm looking at the cornea, so the front part of the eye and sometimes you'll look at that, you'll see little pieces of metal that are stuck on there, say, from a welder or someone who is working with metal. I can see that. Sometimes I'll see a rust ring there. You can actually see rust on the eye itself from a piece of metal that may have been there and then came off.

And then I'll do a very detailed exam, something called a slit lamp exam. It's basically a microscope where I'm sitting down kind of with this microscope that focuses right on the person's eye. I'm looking in the front part of the eye for any, what we call just any cells, any inflammation there that would suggest a lot of irritation in the eye itself. And then I actually put a little thing on the eye that's kind of like a dye that will light up to look for any scratches.

Interviewer: Okay.

Dr. Madsen: Which is what's called a corneal abrasion.

Interviewer: Sure.

Dr. Madsen: So lots of different things you're looking for there on the eye.

Interviewer: So any of these tricks that people can try at home? For example, taking the flashlight and if a person's pupils aren't dilating properly, knowing that you might potentially have an issue?

Dr. Madsen: Yeah, and that's something you can do. If you've had a head injury and you feel comfortable looking at that, you can even look at your own eyes in a mirror and just say, "Do my pupils look like they're the same size?" If you have a family member who's had a head injury, you can shine a light in their eye, just watch, does that pupil squeeze down? And at the same time that one squeezes down does the other one do the same thing? And if it's not, those are concerning things.

Interviewer: Is time of the essence for any sort of eye injuries, generally?

Dr. Madsen: It is, yeah. So time is really of the essence for eye injuries if you actually have something that cuts the eye open. So if we have what's called an open globe injury, so the globe being the eye, the big eyeball, if something actually gets in there and cuts that where there's fluid coming out, time is absolutely of the essence. You need to get to the emergency department. We call our ophthalmologist and they'll oftentimes get you to the operating room to repair that emergently.

Interviewer: All right. Any final thoughts on the eyes?

Dr. Madsen: Final thoughts on the eyes. Obviously, a lot of these things are things we are going to need to do in the E.R. but, like you said, you can kind of take a look at the eyes at home. And certainly if anything comes up, make sure you come in so we can evaluate you further.

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updated: October 31, 2018
originally published: March 19, 2014