TSBVI Coffee Hour
Usher Syndrome - What's New?
01/11/2021

>> Kaycee: Hello everybody. Welcome. We will wait just a couple of minutes as everybody comes in to join us today. While you're waiting, if you want to set your chat to be ready, you can change it from all panelists to all panelists and attendees. That way everyone can see your questions and comments. Again, you can set your chat from all panelists to all panelists and attendees. Thank you, Christine. The handout for today was just put in the chat. Welcome welcome. (Greeting attendees) While you're waiting, we've got one more minute before we get started, if you want to set your chat from all panelists to all panelists and attendees, that will allow everyone to see your questions and All right. We'll go ahead and get started. I've got a few announcements before I turn it over. If you have a question or comment during the time our presenter is speaking please post that in the chat box and make sure it says all panelists and attendees. Your microphones and cameras are automatically muted so you don't need to worry about that. The handout for today's session is shared in the chat for immediate viewing and will also be posted for later viewing along with a recording of this and past Coffee Hour sessions in our link to the Coffee Hour page. Once you're on the page, scroll down under the list of sessions where it lists visit the new TSBVI Coffee Hour archives. That's a link that will take you to the recordings, handouts and chat information. To obtain your CEUs respond to the e-mail that's sent to you. You'll enter the code given at the end of today's session and the CEU certificate will automatically generate upon completion of that evaluation. There's no opening code, only a coffeeing code and it will be given at the end. We'll stop the presentation at 3:55 to give you your code and announcements. I'm happy to introduce today's presenter Nancy O'Donnell. 

>> Nancy: Thank you, Kaycee. Thank you for inviting me today. I'm thrilled. This is my fourth time speaking to people from all over I see from the chat box, but Texas has been very generous in including the Usher Syndrome Coalition in Webinars this year and last year. So thank you to y'all. I have put together, I've adapted a presentation today for all of you and if I have any information that's not clear, please be sure to put it into the chat box. I don't want to leave anyone out or le So let's get started and chat about Usher syndrome. I have my cup of tea here and I hope you have yours as well. My name is Nancy O'Donnell, as I mentioned, and I am with the Usher Syndrome Coalition. I have been with the coalition since 2015. I worked actually from the Helen Keller national center for a good 37, 38 years in various capacities, and in 2015 I felt so lucky to be taken on board with the coalition. I also work for Ava's voice, a small nonprofit, that focuses on children and families with Usher syndrome. And for four years I was an adjunct professor at Molloy College teaching to others in the helping profession. I have worked with the Usher community and learned -- working at Helen Keller as a national vocational and rehabilitation training center, I've met literally hundreds, maybe thousands of individuals who are deaf-blind from all causes. It's my pleasure to share what I've learned from these experts. The Usher Syndrome Coalition, for those of you who aren't familiar wit, was established in 2008 by some parents who had children who were recently diagnosed with Usher syndrome and they were looking for researchers and other families, and there basically was no central place for everyone to get together and share information. Because, as we know, the Usher community is part of the deaf-blind community at large. So they decided that let's kind of separate out and make a separate organization that's just focused on Usher syndrome. Now, as many parents are interested in treatments and cures, especially for the vision and hearing loss VOES indicated with Usher syndrome, one of the main goals of the coalition is to identify researchers around the world and to connect researchers and the Usher community. We raise awareness. We don't pay for or sponsor or give financial contributions to individual researchers. We are here to build the community. Because researchers have told us that finding the community, identifying them and building them, is the most important thing that we can do to help them as they work on their goals. We provide information and support to the Usher community worldwide. We have a big mission. We're a small organization with 3 part time employees. That's myself, Krista Vasi and Julia Dunning who organizes all the conferences and is our program coordinator. So that's a little bit about us. This I call my pet peeve slide. In my years of working in the community I've done a lot of editing so I always want to bring consistency into all of our discussions and writings about various topics. So for Usher syndrome, just to clarify, it is Usher, not Usher's with an apositively trophy S. And unless it's used in a title or the beginning of a sentence, syndrome is always lower case. Just as it is with Down's syndrome. You'll hear me or researchers refer to it as USH 1 A or USH 2 B. That's because the JEENs causing Usher syndrome -- I want to make sure we're all using the same language and you'll also see Usher type 2 with a Roman Numeral or a new America number. So there. We got that out of the way. So there are 5 areas I'd like to touch on today. Just to give you an FYI. If at any point we get diverted or sidetracked or we just run out of time, I have hyperlinks aplenty throughout the presentation and I encourage you to follow those hyperlinks and learn more on your own. I've tried to pick some fun and diverse hyperlinks you may not have seen because my career has been working with adults and in the past five years working more with the younger kids, so I pull a lot of -- oh, yeah. My own pet peeve, I misspelled syndrome. There are a lot of resources here from the adult world from whom we can learn a lot. So let's start out what is Usher syndrome? I have a graphic up here that shows that it is a -- it impacts children, it impacts adults, it impacts people from all ethnicities, tall, short, black, white, Asian, etcetera. So it is a syndrome that we've known about for more than 100 years which kind of blows my mind. It was first written about in 1858 by a German ophthalmologist who noticed -- it's the ophthalmologists who are typically the ones who have identified Usher syndrome because as you can imagine they have individuals coming in who are experiencing vision issues of some sort and then they notice, oh, this person with restricted visual fields is also deaf. And, oh, they have a sibling who's also deaf. So it took quite a few years for the syndrome to be recognized as a unique syndrome. And of course back in the 1800's and early 1900's specific Jean s hadn't yet been identified, but through family trees and mapping out relationships, they were able to say, yes, this is a genetic syndrome. In 1914 it was Charles Usher a Scott issue ophthalmologist that got to put his name on this syndrome that we all know. There are several features. A syndrome just means features or symptoms or signs or indications that are all together all the time. And for Usher syndrome, it means that there is congenital bilateral hearing loss, whether it's profound or mild, moderate. It is a leading genetic cause of deafness. So within schools for the deaf or in deaf populations, there are a range of numbers, but up to 10 percent of children who are deaf, it's attributed to Usher syndrome. The vision loss is caused by retinitis pigmentosa, or RP, signed by the eye and cheek, and that is a progressive vision loss and it's also connected with difficulties seeing in dimly lit areas and it's commonly called night blindness. In certain sub types of Usher syndrome there are vestibular issues meaning the inner ear and balance mechanism is off. So in types 1 and 3 you'll see individuals who are having difficulty dealing with the balance issues and the impact of that on their daily living and children's development. I want to make clear that there is no associated intellectual disability with Usher syndrome. There used to be back in the 80's I believe a thought that there was Usher type 4 and that was thought to be Usher syndrome plus an intellectual disability, and that was later dismissed. So there are three major types, no associated intellectual disability with the genetic component. So the three types, 1, 2 and 3, have 10 sub types, meaning the researchers, once they were able to identify GENEs, were able to go in and locate 10 different genes. The hyperlink here will bring you to a website hereditary hearing loss which updates information and if you are scientific and like that type of stuff, you can look a little bit more at the actual genes that cause Usher 1, 2 and 3. So for Usher type 1, I'm going to breeze through these for a review just so we're all on the same page, Usher type 1 has the severe hearing loss associated with it. There are 5 sub types that are recognized. You'll notice there's no type 1 A in the list. Apparently researchers thought they had identified a type 1 A and later it was found not to be unique to Usher syndrome, it was found to be something else. So they renamed all of the others -- instead of renaming all of the others that came after it, they eliminated 1 A. Usher 1 B is the most common cause of Usher syndrome hitting about 40 to 45 percent of that community. There is earlier on set RP in Usher 1 community compared to the others. This is the sub type where vestibular issues are generally included. The three areas for Usher 1, profound hearing loss, early on set RP, including the night blindness, and vestibular issues. I've given you two hyperlinks here, two personal stories by and about people who have Usher type 1 so you can become familiar with and comfortable with what someone with type 1 might look like and how they might communicate. You'll notice type 1 C has French, Canadian SDRIBD described before it and that is the origin of it. Whereas the last one says Ashkenazi Jews which is European Jews and that sub type is found more commonly in Ashkenazi Jewish community. So the impact of type 1, there's a huge community in Lafayette, Louisiana with usual type 1 C. I just became aware there's another community in Baton Rouge with Usher syndrome as well, but there are multiple generations in families of Usher syndrome in the Lafayette area. So for type 1, the impact would be and parents have told me this is how they have kind of diagnosed their own child as having Usher syndrome as opposed to just being deaf, they're late in sitting up and walking. Their muscle tone is floppy, so they are late sitters, late walkers. And as you get to an adult with Usher type 1, you'll notice that they may walk as if they are drunk. In fact, we've had some adults report that they were I don't want to say pulled over because they weren't driving at this point but someone did approach them like a police officer or someone in the community to see if they were drunk and obviously the communication issues that come up when someone doesn't use speech to communicate and sign language is their primary method of XHUNTHing, Communicating and if there are visual issues, there can be a lot of room for miscommunication. But just be aware that a lot of the younger generation who are having cochlear implants also have access to physical activities such as horse back riding and swimming and parents have recorded this has tremendously helped with the vestibular issues. These brothers in New Jersey are well known swimmers in the paragraph are an olympic world. For the older generation with Usher type 1 those would typically be those who went to a school for the deaf for whom sign language is the preferred or native method of communication. And now we see a trend in the little ones and even the younger adults because we have a group that gets together through Facebook and zoom calls of young adults with 1 B and children with Usher type 1 getting cochlear implants. So it's totally changed the profile of what it is to have Usher type 1. Not everyone is getting cochlear implants and the coalition doesn't take a stand on what someone should or shouldn't do. Our goal is to provide information in as many modalities as possible so that parents can make an informed decision that works for them and their child. So what we have heard from parents and we have heard from those young adults who have cochlear implants is to remember when the implants come off, and this also applies to those with 2 and 3, when the hearing aids come off, they are deaf. So at that point it raises issues to discuss about alternate methods of communication. Also in darkened environments, my friend who is the president of Ava's voice, her daughter Ava has Usher syndrome type 1 B and in darkened environments Ava relies on tactile signing and finger spelling in order to communicate with her mom. And if a CI malfunctions as happened up at camp when we ran a camp last year for kids with Usher syndrome in upstate New York, one child arrived and his processor had totally died. So we always want to think of what are the backup methods of communication in case the technology just doesn't work. Driving is an issue for Usher type 1 community. I remember working at Helen Keller in the early days and a mom might come to get training, a mom with Usher syndrome. And, oh, she has two kids, she has three kids. Through discussion you find out that she's still driving even though her vision is extremely narrow. Lots of states, as you probably know, don't test for visual field acuity, they just test for acuity and if you can read the eye chart, you're okay. So that's a big hurdle. I don't tell someone when to or when not to because that's not my thing. Again, we provide information for people so that they can make an informed decision. And it's a little sticky because as a person who is sighted and hearing, I don't have anyone in my family with Usher syndrome, you can see the struggle that teenagers are going through because that's a sign of such independence when you get your license. On the flip side when someone is in their 20s or 30s or 40s to give up their license is also a big hurdle. So just to bring that up as a topic, that's important to talk about. Usher type 2 has three sub types, A, C and D. Usher type 2 A is the most common type of Usher syndrome and it has moderate to severe non-progressive hearing loss, although a recent discussion with a person who has two adult children with Usher 2 A revealed, she mirrored what my experience has been that a lot of people in their 50s and 60s with Usher type 2 go for cochlear implants because they feel they have a loss, a progressive loss. What I don't know and what this colleague didn't know either is if this additional hearing loss is associated with Usher type 2, we still need more research in this area. But according to the scientists we follow it is a non-progressive hearing loss. There are no balance problems associated with Usher type 2 and there's a video link there to meet BREN Dan back when he was at the Helen Keller center back in 2017 just to get to know someone with type 2. This part of the community are basically raised oral/aural. Their preferred language is usually spoken language. They are often mainstreamed in schools with support and they can be quite invisible because many of them are doing great in school and not even aware that the vision loss is happening or that it is happening in darkened situations. Again, if that's all you know, then you think that everyone else is experiencing the same type of vision as you are. So I've spoken with lots of adults who don't get diagnosed until their 20s, 30s, 40s. We had someone in our conference in 2019 who was only diagnosed in his 50s. Now genetic testing we're getting kids in our registry at months old. There are parents are finding out at 5, 6 months because genetic testing is become more common, also prenatal testing. If someone has Ashkenazi Jews in their background, they may be tested and discover that their child has Usher syndrome in utero prior to the birth. Genetics is really changing the whole landscape. Who determines which type genetic testing which type and sub type? That's right now the only way of definitively diagnosing what type of person has it. Prior to genetic testing I can tell you someone would come into my office, my classroom when I was teaching at Helen Keller and I always tried to meet every adult student who came there and it would be clinically or as they say -- not genetically. Hold on. The word is coming to me. It will come back. Through observation of the symptoms they have or how they present. Their educational history. Fee no typically. The the only way is to have genetic testing. So again type 2 might be invisible in a school setting. And for many of the adults with whom I've spoken, they don't really feel the impact until maybe their 20s when they're going to college or starting on a career or maybe even when the career hits critical mass, when the vision loss hits a period where they can no longer function visually in their job. And then type 3 is the most rare type of Usher syndrome. They say about maybe 5 percent of individuals with Usher have type 3. You'll see 3 A and 3 B. 3 A is the more common and there's a link there to Rebecca Alexander who is a well known person with Usher type 3. She's a SKOL therapist practicing here in New York City. I am here in New York on long island. In fact, it was Rebecca's family genetics that helped to identify the JEEN that causes 3 A. 3 B you have a family in Texas actually that I got to meet a couple years ago. Up until that time, 3 B was associated with the older aMISH order and had been found in Canada and in a group of aMISH individuals in Pennsylvania. This person with two children with 3 B is of Indian heritage. So that was quite unusual. And then we've since discovered another young woman who has 3 B of Indian heritage. So this is one of the exciting, if you will, parts of working with the community, learning about it, and doing outreach. Because India, China, Europe, Asia are untouched lands for us. So we are increasing our outreach efforts to countries outside of the United States because we're sure that there are hundreds of thousands of people that we have not yet identified and maybe there are other sub types out there. Do people with Usher syndrome loose color abilities with with -- that's a great question. The acuity usually stays. It's one of the last things to go. But individuals with Usher syndrome are also more prone to cataracts and to mack you LAR edema. So those are two other things thrown in and can affect the central vision and the ability to discriminate information visually. So the impact of those with type 3 is they function orally similarly to Usher type 2. They're mainstreamed. They do well. They are invisible for many. And again their career, when it becomes impacted, then they go to an eye doctor and the doctor says hold on, hold on, I see something on your retina, and that's when the vision loss is With Usher 3 versus 2, 3 is a progression of both vision and hearing loss. So a person is born maybe with a mild hearing loss, but it's -- distinguishing features are that the vision and hearing loss are both progressive, whereas in Usher type 2 A or 2, we know that the hearing loss is supposed to be stable. And then there is always that one that doesn't want to fit in with the others, it's atypical Usher syndrome and this is where someone tests their genes, they're found to have the Usher Jean, but either they just have the hearing loss or they just have the vision loss, even though the Usher GENE is there. There may be a modifier GENE down the row, the symptoms could be totally absent and researchers are very curious about this and need to do more work in that area. We also want to remind you that deaf plus RP does not always equal Usher syndrome. We know that hearing individuals can have RP. So a person can be born with RP and acquire deafness from another cause, etiology. So back to our point of genetic testing being the only definitive way to diagnose someone. Researchers also caution us to say they feel they have identified the genes for about 80 percent of the population, there are still genes to be identified. Someone may fall into, hmm, we haven't found that GENE yet but it feels like this person has Usher syndrome, but they don't get a definitive diagnosis. But they know that they have 1 or 2 of the identified genes so far. Is mack you LAR degeneration ever miss diagnosed with USHer 3? Not that I'm aware of because macular did he JEEN -- we're still learning. Still learning. So the population estimates. Basically what I want to say here is that the population estimates are way undercounted. So what we expect from different researchers working different parts of the Usher community that there are between 20,000 and 50,000 people with Usher syndrome in the United States. That's based on a prevalence of between 1 in 17,000 and 1 in 6,000. And if you look at the EHDI statistics, the early detection, 3 to 10 percent of children with congenital bilateral sense so neural hearing loss, between 3200 to 11,000 kids birth to 21 according to the EHDI numbers. And then if you look at 20 to 50,000 you say how many are born with Usher syndrome every year? If you divide that by life expectancy, we might say there are between 250 and 650 Usher syndrome born every year. If you look at the national center on deaf-blindness, they just published the 2019 child count where there are out of 10,000 plus deaf-blind children across the country, 383 were listed as having Usher syndrome. Out of those, interestingly, 58 percent of the kids who have cochlear implants have Usher syndrome as compared to the overall deaf-blind community where the non-Usher kids 11 percent. So again this just illustrates the point that cochlear implants are completely changing the demographic, if you will, of kids with Usher type 1. And Usher syndrome now compared to back when it was named in 1914, this is not your father's Buick. There's a dramatic change in the kids who are getting implants, the kids who have low vision tests or who are aware of it, who are being diagnosed at months or young toddler years because they have a body of information to plan their communication, to plan their life strategy, to know to not feel diminished, to know why they may feel clumsy or different, etcetera. That it's not all in their head, that it is a syndrome and there is a huge community out there where they can join in and belong. The Usher Syndrome Coalition maintains an usher only international registry. It's estimated TLSH over 400,000 individuals with Usher syndrome. We actually don't have a handle on the exact number, but that's the number that researchers are comfortable with. In the United States we have 1100 registered out of the 20 to 50,000 that we anticipate. So the numbers are very, very low. In Texas there are 79 on our registry and according to the population of the great state of Texas, there should be 1700 to 4800. Whether there are 1 in 6,000 or 1 in 17,000 as the prevalence. We definitely know that this is an underrecorded group. Where is everyone? Why is it underrecorded? The first may be professionals are unaware of this disease or rare syndrome. It is categorized as one of 7,000 rare diseases, so you can't really expect doctors to know all of the 7,000 in a robust type of way. So medical professionals, which would be the entry into services and awareness and diagnosis, we really want to work with them to get more information out there about it. Children with Usher are mainstreamed more so they may be the only child in their school or school district with Usher, which doesn't give a lot of knowledge base for comparison. Also, since vision loss is late on set, kids with Usher type 1 might be identified earlier but those with Usher 2 and 3 go through until their late teens, early 20s, etcetera, so they may not be diagnosed unless they're getting genetic testing at some point. The vision is progressive with RP for type 1, 2 and 3. The hearing is stable for type 1 because they're already born with profound hearing box. I'm just looking at the chat box every once in a while here. Adults with Usher syndrome who have come to the coalition say I thought I was the only one. I didn't even know there was anyone else out there. And interestingly the more we build our community the more we find people within states and sometimes even within towns or counties that are next door to each other. So that's an exciting aspect to doing outreach. And as we know some cultures are very protective of people with disabilities. They hide them. They take care of their own as they say and that's a cultural value. For example, some of the more conservative Jewish communities are sometimes reluctant to talk with us as an outsider. I've been to Lafayette, Louisiana. It's a very large community of people with Usher syndrome, but they take care of themselves and that's great. That's fine. It's just one of the challenges of trying to get the numbers, if that's important to you. Also, children are very, very clever at adapting to their vision loss because it's progressive. It's slow. We don't have a time line. There are national history studies that have taken place and are taking place to try to map the projection of the vision loss and the hearing loss, but of various types, because if you don't know the national progression, then you won't know if an intervention is doing anything positive or negative. So these natural history studies show even within families that have 2 or 3 siblings with the same type of Usher syndrome, there's quite a bit of variety in the rate of vision loss that occurs. So there's still a lot of unknowns. And this is not unusual for parents to know that their child has Usher syndrome and to not reveal it because many say I don't want my child to have that burden of knowing that they have it. I don't know when to tell him or her. I don't know how to tell him or her. It's something that we as a community and we as a field haven't really looked into in order to provide some guidance to parents who are struggling with that. So lots of issues about finding everyone. Students with Usher syndrome. As I mentioned before, they can be invisible. There's no intellectual disability. Many are visual community tors. They are different from the congenitally deaf-blind that is served -- the -- test test test test Many kids with Usher syndrome are awesome and thriving, so it can be difficult to identify the kids. USHer means constant change. I have a couple of friends who are mentors or counselorers to people in the Usher syndrome community and that constant change is the hardest thing to deal with. Because as a person adjusts to their vision or hearing loss, they go along for a few years and something happens. It can also fluctuate from day to day, week to week, based on the weather, how someone is feeling. So all of this change has an impact on how the child might function in an educational setting So something that worked yesterday may not work today. Where the interpreter is sitting. The lighting in the classroom. Whether they use regular print, large print, and then do we switch over to Braille and if we do, when? Speech reading may be affected in darkened situations where the lighting is poor or as the vision loss continues, adults have said, oh, I've lost more hearing but they've actually lost the ability to speech read. So all of these constant changes make for challenges in day to day life. Communication is diverse. We have individuals who use sign language, spoken language, tactile sign language, large print, regular print, Braille. And then when you get into the website and web access IBLTS with captions and image descriptions and descriptive information, there are a lot of pieces to put together in order to make sure that the Usher community is fully included. So educational considerations. The impact on the communication and the seating in a classroom is quite important. All of these are recommendations that I've gotten directly from teens, young adults with whom I've interacted over the past five years or from their parents. So preferential seating, assistive listening devices. The lack of glare. I'm wearing black even who I'm not signing this presentation and I have a black back drop behind me so that I reduce the visual noise. I reduce glare as much as possible. Do we use large print? Do we have at some point a loss of color difference YAGS that we want to check with? Situational blindness? Certain areas. For example if someone is in a gym locker room as compared to a gym or cafeteria and is the lighting adequate so someone can see. Speech reading, if they're signing, etcetera. In the adult world, about I'd say seven or eight years ago I noticed that deaf-blind adults would start to take a hands down break where they take 3 to 5 minutes at a conference or workshop, they take a break where they can rest their shoulders, hands, eyes, because the mental and visual fatigue of communicating even with cochlear implants, my friends with CI say they're exhausted by the end of the day. Parents say their kids, and we know from years ago just with hearing aids, Hearing aids would end up in the top drawer of the desk at home. I don't want to hear anything more. I want silence. So the sensory breaks can be important in a classroom if that's possible so that there's not this exhaustion from just participating in daily life. And then we've talked about in some of the groups I've worked with, I've spoken with state deaf-blind project leaders and we know that intervenors are used with a lot of deaf-blind children who do not have Usher syndrome. So what exactly is the role of an intervenor for kids who are following the general academic curriculum? Do they need intervenors? What would their role be as opposed to a deaf-blind intervenor for a child who is congenitally deaf-blind or may have other issues. Yes, sensory breaks are very important. The vestibular issues, there's a comment in the chat box about the importance of paying attention to them. Educational considerations. Again pulling on my experience from Helen Keller, when I was teaching you'd see a person with Usher syndrome arrive at the door with or without their cane, stand there from the change in lighting from the hallway to the classroom, wait for their eyes to adjust. While they're there, do some visual scanning to get a sense of where they were walking into, who was where, where are the desks. Once that adaptation had occurred, then they would walk in, always scanning. So this is an important thing to know about in a classroom setting, in a high school setting. Then, as I mentioned before, that's one of the first questions that parents might ask once their child is born, do I have to get a hearing aid right away? Do we have a get a cochlear implant right away? Should I start teaching them Braille right away? Do we get a cane for them? There is not a lot of great guidance on that. These are just some of the conversations that we really need to have with parents and with the adult Usher community, with the teens and the young adults, to see how were you raised? When were you introduced to Braille or a cane and how did that feel? Because there are so many emotional overlays that go into those decisions. And then haptics. Haptics is a system of touch signals on neutral parts of the body. When I worked at Helen Keller we brought over the originators, actually they were the stewards of the original development of the haptics from Norway and they taught our staff haptics and basically they are, for example, PT uses this as well, pro tactile. There are touch cues on different parts of the body that can give information to an adult or a student kind of benignly or in the background. If someone is laughing, they can use the open 5 land shake claw on the shoulder and repeat that hand shake opening and closing on the shoulder to show someone is laughing. I had a friend, we were in a diner, she reached over with her index finger on my elbow and brushed it up to my shoulder meaning I'm leaving for a second. When she came back she touched the top of my shoulder down to my elbow and that means I'm back. And then she said, okay, why don't you do that to me if you have to leave for a minute. So there are all these ways of communicating which I refer to as PARA linguistic methods of communication that can be helpful for your students. It's important to balance play and work because the kids put in so much work there managing their technology, setting their technology up, breaking it down, changing classes, going to locker rooms, trouble shooting devices that don't work. And that mental, visual, auditory fatigue with managing all of their stuff. They are still kids and need to have fun. Okay. 10 minute warning. The fourth one is visual or tactile markers. I want to emphasize that the X on the back is an exit now marker that has been taught for years. If there is an emergency situation, you put that on the back and then the person, teach the person to just grab your arm or to exit with you no questions asked until you reach a safe space. And, yes, great point. Be careful when looking at preferential seating because not all of those with RP benefit from front row seating. So if you all put up your hands as if you have the inside of a paper towel roll and you look closely at the screen up close where preferential seating might be considered and then you move back, you'll see that you're seeing more of the screen, more of the situation. So they may actually want to sit a little bit farther back and, thank you, that's a great point. And then there is a webinar on the website with a hyperlink here. It was about an hour and a half, two parents going over all of the accommodations that their children who are students with Usher syndrome received in their school district. It's very comprehensive. It was really amazing. So COVID, I threw in this educational considerations during COVID because, again, in mining information from various sources, these are some of the topics and areas that we really have to pay attention to in terms of visual accessible TI, is the Q and A or chat box, I'm looking at that, but with my glasses I have 20/20 vision. So it's just a list of things for you all to take a look at and discuss among yourselves and with your students as to whether or not the accessibility is optimal. There's no perfect solution and we're still struggling to figure out the best way to provide equity in distance learning. And then I always suggest to look at the experts and for me the experts are the Usher community. So what I have here is a picture that's well known for representing retinitis pigmentosa, on the left two boys smiling with balls and on the right it's a tiny picture of two boys' faces and that's supposedly all you can see when you have end stage RP or getting towards the end. I'll go back to that in a second. So we want to learn from families and this slide talks about Ava's cheat sheet, Ava being the 14 year old daughter of my friend CarlI who created Ava's voice and they provide resources for families and for kids. Lots of hyperlinks for you to look at later. We have our USH connections conference, this year it will be online. Check our website for that. Ava's voice also made a tremendous video called the diagnosis where the kids talk about what it was like when they received their diagnosis. As teenagers they're talking now retrospectively and their parents are cut into the video to say how they're dealing with it, what their reactions was. Ava's voice has organized a discuss USH family weekend. We can get together in a camp in upstate New York and be other families with kids with Usher. Learn from youth. We have a youth group on Facebook and a website that has a dedicated blog to youth. They're saying ask us what we need and there are various resources here to connect you especially through the Ava's voice summer camp which we ran in 2019 and was tremendous. More information at all these hyperlinks. So back to the picture of RP as we have always seen it with the two little boys with the ball. One of the experts is Tim CHAMers, an adult who is a master painter. He does portraits, land escapes. He has Usher type 2 A. This last summer for our conference he created a series of 4 paintings that you see in front of you, 4 versions of a church steep PEL under different lighting. He said it's not black black black and then I see a little hole of vision. The colors and details are muted under different lighting conditions. This totally blew my interpretation of what it was to have RP. And there's a website where he goes into more detail. Basically the rest of the presentation is that we go back to the community itself where the experts are living with Usher syndrome. Parents. Adults. Children. Teens. Learn from the community. And then go to the Usher Syndrome Coalition website to find our registry, information about our conference, our blue book, our many, many presentations through our blog and our USH talks. We have done a first ever series in ASL about Usher syndrome. We have ambassadors throughout the country, we coordinate with other national organizations. The third Saturday of September is Usher syndrome awareness day. So see if you want to organize something around that. Social media and our e-mail addresses. How did I do? 

>> Kaycee: Perfect! I love having you come present. I learn new things every single time I hear you speak. 

>> Nancy: Thank you, Kaycee. 

>> Kaycee: The details and information you share are so great and so important in where we're thinking about eligibility for our students who are deaf-blind and Usher syndrome absolutely falls into that. In Texas there are 4 ways to qualify and it's that progressive piece. So when you were talking about now we're getting students who are identified at 4 months and 6 months old. Even though they may be type 1 and have totally normal vision at that point, they can qualify because we have diagnosis of a progressive condition. All of that is so important to think about and how we can get them ready for their future and help their families through that time of diagnosis. So it's just wonderful stuff. Great information. 

>> Nancy: Good. Yeah. There's a lot to learn. People like to label other people as experts. I am not an expert. I am just an information hound. I hoard it. I have an office full of it. And I love to share it with all of you. So thank you for being such a great audience. I love seeing the comments. I'm sorry if I missed anything. As I went through I see one about camp. You can go to Ava's voice.org for more information about camp. We are hoping to run it this summer, but we will keep you posted through that. And Canada, we have an ambassador for Canada. If you want to get in touch with me, I'll put you in touch with Pam mason, the mother of the two boys who are olympic swimmers. 

>> Kaycee: Join us on Thursday for accessible interactive map with our guest speakers. We will not have a session on the 18th for MLK day, but we will be back on the 21st when using the TSBVI talking graphic calculator with our special guest. Check our website TSBVI.EDU/Coffee Hour for more information. Respond to the e-mail today from our website ESC works. You'll get that e-mail this evening. Enter the code and your certificate will automatically generate upon completion of that evaluation. Also the handouts and recordings from this and past sessions are available through our link on our Coffee Hour page at TSBVI.EDU/Coffee Hour. Once you're on the page scroll down to the list of sessions where it says visit the new TSBVI Coffee Hour archives. That will have the recordings, handouts and chat information. On the evaluation you receive, there are two boxes, 10 and 11, that say additional comments you would like to share with the presenter and the event planning committee. Please let us know in those boxes if there are days for Coffee Hour that work for you or if you have suggestions. We love to hear your ideas on topics for future coffee hours. We have the mental health symposium for deaf and deaf-blind upcoming April 22nd and 23rd. I will drop that information in the chat right now so you can get registered. And then I'll also drop into the chat some -- a survey link which is to two quick survey questions. If you wouldn't mind filling those out for an upcoming Coffee Hour session that we have. That is being dropped now. Thank you so much for coming and thank you so much to Nancy. 

>> Nancy: Thank you thank you thank you guys. Take care and be in touch. Please feel free to get in touch with me if you have any additional questions.