Uh, the strict techno-mancer that is Vinci V. Let us get to the technique and learn it Vinci V style. Hello everybody and welcome to another hobby-cheating video. And today, we begin with a story. A little while back, I was watching this man, the honest Wargamer Rob and Owen, and they introduced me to this wonderful thing called SmashBat. They mentioned this new FEC flesh-eater quartz list and this incredible Vargulf that could destroy entire enemy armies. And then I went and looked at the figure. Oh Lord. And I stopped thinking about that army. And then, they released this beautiful model for Cursed City. And I thought, okay, maybe we've got something here. So I converted everything up and it's time to paint an army. I'm going to take you through the entire process. And we begin here with the priming, actually. I started with my standard black primer, but instead of doing a normal zenithal, I'm shooting some rock-bron red primer from below, but any deep red would work. And as we're watching these steps, I want to talk about the concept here. Now we're moving into a standard zenithal. I'm using a light ivory color from above. When you're picking your army to do this process, to try to paint an army fast, there's a couple important things. One, stay around a regular army size, 2,000 points, something like that. Don't get too crazy. My next step here is just applying some Raikkon Flesh Shade over everything. Pick simple figures. The right army equals success. For example, here I'm able to drybrush this guy efficiently, the whole thing, because he's basically all flesh. Miniatures with huge amounts of detail, with lots of different types of textures, they're going to slow you down. Something like Flesh Eaters or whatever, make for a perfect army for this, because look how fast I can just zip through all of these guys. Dry brushing everything very quickly. Each individual step as I'm prepping them with all of this under shading is so fast, because they're mostly uniform. Now let's not say they have some different stuff going on. There's fur, there's wings and so on. Now if you wanted flesh colored by the way, you could kind of stop here, but we're going to go farther. And this leads us to the simple color palette. When you're working on an army fast, pick a nice simple but striking color palette. If I wanted to stay flesh shaded, I could have stopped there, but I didn't.  So I took some plague bearer flesh contrast paint and I'm airbrushing it over. Now it's not going to act like contrast paint, but it will tint them this awful hideous dead yellow color. Then I'm taking some green ink and shooting it from below. Now why did I use the red and the green? Because this is basically a rediverse verdaccio. Verdaccio, you use green and you put red fleshes over top to make it seem a little more neutral. Here, I'm using reds underneath and shooting green over the top to neutral out the green. So there's that hint of death, but it's not overwhelming. Next up, I take my airbrush and I shoot just a little bit of now kind of a whiter color. This is again going back to that flesh ivory just on the highest spots. Not over the whole thing. Top of the shoulders, top of the head, just creating that volumetric light. So that simple color palette with simple figures have set me up for success because I can execute on this amount of figures. It's 77 models. So every time you see this sort of time state of this fast process here, this is going through 77 different models, but I was able to do all of this very quick. And I planned all these steps out in advance. That's the other big thing. When it's a big project like this, plan it all out. Sick to simple steps. Set yourself up for success. All right. So it's about two something in the morning. I've been at it for about two and a half hours. And as you can see, I've got pain all over my face because we're working so fast. That's fine. I don't see an issue. And we're finally done with, you know, this air brushing stage. Now, the bright part of the bright side of this sorry, that's very dark at this angle, but the bright side of this is that the skin is mostly done. These guys look pretty good as far as skin goes for where I want them to be. So now we get to just move on to some details and from there, we're going to start picking some stuff out. Now it's time to get into really fun techniques. One of the best parts about picking an army like this and this is why I say it's so important to choose your force. As you know, when you're going to do a speed painting project, pick something that you can speed paint certain models, cancer models, can't as I mentioned. And the best part of this one is when you get done with the skin, you've got done with 80% of the model. So there you go. So now let's go back to the table and it's time to get out the brush and do some fun stuff. So I've set myself for success with a simple force, a simple scheme and the right amount of figures. 77 models is doable. And with the airbrush, I was able to complete the flesh, the major part of the figure all by itself, but we've got to get the detail and for this stuff, we're going to rely on contrast paints. Very heavily used contrast in this because I have so much other work and zenithal activities. It actually is really easy to just use contrast and have it look tabletop ready. So I took some black Templar covered about half of the the areas I wanted with that. Then I took some flesh tears red and you can see I'm just working over the top and I'm working wet on wet so that the two blend together again work fast with a brush and then the two wet contrast paints they seep into each other and I don't need to worry too much about blending because it's doing it for me. I did everything I could with the airbrush used all that undershading to set myself up to provide an interesting flesh tone. Now it's about picking out the rest of those details and making them interesting. So I'm doing here is going around on the model with things like the bones dropping a little Wildwood into the base and just mixing in the same ivory color I was using for my highlight but now it'll be much more strong when it's applied by brush and I'm just mixing that in with that wet Wildwood paint hidden the little teeth the eyes. By the way this was one brush I use for everything on here this is a size three and I do every part of every miniature with this size three. I don't swap around again everything I can do to increase the speed of the thing right here I'm getting into that pure ivory and really just making sure those bones stand out and you see now that that dark line of that Wildwood has been mostly subsumed into the layers I put on top but because there's a nice light dark light dark line everything stands out now for a little fun touch I took some thinned down black Templar contrast and I'm running that over their hands and feet and you notice how it just kind of seeps down in there and coats and everything this is thinned down about 50-50 just with water and it gives this wonderful staining effect to the hands and now I'm going to take some of the red and do the same thing again but over the edge of the claws so we get all these wonderful transitions of of hue between the edge of the feet where it goes red then to black then to the green and so on and that just creates more visual interest sometimes you're working fast to make mistakes like I did here all you've got to do is take a little bit of that ivory color and neutral tone touch it over the top nuts very complicated skin tone but small areas doesn't matter just touch a little bit on there because it's very opaque paint covers over the spot I let it dry and then I go right into that plate bear flesh and I just work a little bit over that to tint it back to the same color alright so we're six hours and one minute into it there and we're making pretty good progress you'll see a picture right up here one of these sides will be a picture and kind of show you where we're at I have about six five six hours of cool work left at this point the ghouls anytime you got 60 of a model boy oh boy does it take a long time because it's just a lot of repetitive steps so the key is when you hit this point and trying to get a project done like this and trying to do this kind of a paint the key is you just got a power through find some music to listen to put on a good book find a funny video that you can have on the side is listen to and laugh whatever it is find something to keep you working flip your phone over so you're not looking at it because that will just distract you and becomes an easy way out just focus in on taking those steps hit that flow state getting that Zen space and and keep going so this point I'm feeling pretty good we'll see how we feel next update so part of what you have to do when you're working like this is get everything down to a process I was able to do each one of these ghouls in about eight minutes which sounds insanely fast but the reality is when you have 60 of them to do and you multiply that out it's a lot of time right because what that means is every eight is a little more than an hour and I've got a lot more than eight to do so I actually thought I would put this whole sort of process and I recorded one whole ghoul you can see how I worked each of the steps in turn like every time I touch a color it's everything there I did all black and all the red over top of it mixing them in use a little bit of the wildwood to blend it together hit all of the necessary parts hit all of the ivory to get all that 10 the eyes red go right when you're working on these kinds of big projects we've got a lot of the same thing the key is to establish a process and you do that early that's why you plan your steps out why you paint a test model and why you know what you're doing you don't want to be discovering while you're working so look at the different models you have maybe some of them have some different elements that they don't all have figure out how you're going to tackle those deviations from the norm and that will help you move through everything quickly efficiently and get that army painted so here we are it is many hours later just shy of 11 hours as you can see there and all the ghouls are done so that's great 60 ghouls done and finished feeling good about where we are in the progress so now it's time to turn to some characters and this is sort of a piece of advice I have whether you're trying to paint your army in 24 hours or just trying to paint your army period oftentimes after a big push like that where I was really just grinding grinding grinding on the same base troops it's good to change pace go do a single character get that finished that's going to feel good feel rewarding you've got a big chunk they're finished sort of the points they represent and then you can move back to maybe another troop type and then back to a character by breaking up your work like that you sort of you know refresh your palette keep yourself going and make it so you can get through the whole project so let's get back to it and keep painting so I wanted to talk about how I tackled some of the special cases for example these crypt flair stand-ins which are the little viercos wolves or whatever that I or vampires that I added wings to I had to create a new technique for them because it was different but again it's the same principles lay down the black then lay down some new black work wet on wet with the red right keeping with these simple techniques is it refined is it perfect no we're not going for perfect time is our master here so we're just working wet and rough still pushing for that contrast so notice how I'm working a lot of wet over wet paint and just roughly stippling and blending it all together I do want to take a moment to talk about characters characters even in a speed paint are still important so with my smash bats the big characters of this army I go in and I put in some extra time like adding texture to the wing flaps putting some extra detail and picking out individual hairs picking out their claws I was so fast with the other ones this bought me time to do things like add texture to the wings put an extra little texture on their skin their faces clean up mistakes when it comes to rank and file masked infantry the reality is a big horde of them looks impressive if they're all painted but with the state you lose the detail but with the standalone characters it's worth spending the extra time again true if you're speed painting a whole army or just painting your army normal put the love into the characters all right so we're just shy of 15 hours into this whole mess and basically everything the primary stuff is done so we're way ahead on here we've got all our boys here ready to go all their primary colors done what we're going to do now is the bases and a little bit of the metallics and thankfully since the metallics are going to be heavily rusted we can actually do that all at the same time so everybody's getting a quick coat of varnish just to lock everything in flatten everything out and then bases metallics and we're going to call call this project closed now normally I like complicated bases but here we're keeping it simple in line with everything else we laid down some mud we're dry brushing it with the same ivory as everything else with the metallics we just coated them in a base simple metal then we're going to go in with browns and oranges and stipple those colors in more visual interest more break up to those elements adding that tonal variation into them because now there's little items to discover little rust spots a little texture that brings everything together and makes it interesting so here we go all done 77 figures took me 17 and a half hours total but we got there and one more army down this is a lot of fun I really hope you enjoy this video I'm going to end this with some shots of the whole army so you can see everything up close and and how we got on but this was really great and now can smash bat smash that why yes he can thanks for watching everybody I really hope you enjoy this give it a like if you did subscribe for more hobby cheating in the future if you've got questions drop them down below but as always thank you for watching this and we'll see you next time


Good afternoon, everyone. This is Melissa with thestockswish.com and welcome. Welcome to BBY. This was my watch for yesterday on Thursday when the stock actually got. And it was a follow-through watch for today. This move today is outstanding. Outstanding. The weakness in this BBY today, look at this. From the moment it opened, it tried to get over $27.00. It couldn't do it. Right out of the gate at 9.30 in the morning and never look back. The stock never looked back. This is actually a 15-minute chart. It is, you can't even barely see the green in this chart.  And this would have kept going. I mean, this will keep going. This will keep going in money. This could have kept going today. 24.33 actually was the dream target and it just ran out of day. And if it wouldn't have been 4 o'clock, this would have kept going here. This is a beautiful, beautiful, beautiful, beautiful gap. And I'm going to go over what I did with it yesterday, too. But the main thing about this chart and what I love about BBY is the overall understanding of the trend here. So I had a long, long, long discussion of this on Thursday in the room because it didn't work right as a short on the day of the gap. Even though the gap was an amazing gap, a beautiful gap, and I watched it short and I did short it, but it didn't work. And I saw right away then that it was a failure, so I never retook it. And again, this is part of experience and maturity and why it's good to have a mentor from someone that has experienced like me because I can see when something is a failure, so then if it doesn't work, I just don't keep going after it. The chart of BBY was in an uptrend here. Was it an uptrend here? Broke, broke, broke, broke. Fixed itself very quickly here and then followed through for a brief period of time, broke again, gaped again. This is back in 2011 and then fell off a cliff. Tried to recover here and couldn't do it. This is all on a downtrend still, downtrend, downtrend, downtrend. Finally, at some point in here, you got to go back and look and find it. The stock had a bullish gap and it was back up in an uptrend. It did not last long. It was very short lived. This is back in 2013 and the stock gap down and actually went neutral for a while. Strange, unusual stocks usually do not stay in a neutral trend position for long if they do happen to be neutral. It usually doesn't last and it's a pattern that when it sets up, will do one of two things. Either in the neutral position, it will go back in the original direction it was just in or it will actually change then. So that's what happened here this day, this gap yesterday. The problem was that there was so much volume in this into the gap in the pre-market morning at 7 o'clock when it got. There was 14 million shares I think or something before the open before 9.30. All the sell-off happened into the gap in pre-market. So then there really wasn't any more sell-off action that happened on the day but I knew. I knew this would follow through. I just knew it. This is exactly why the idea of people thinking that gap fills as a strategy is ridiculous, does not work and it's not a way to trade. If you thought you were going to buy this yesterday or today or anywhere and get this back anywhere up here in the 30s, 30, 31, 32, 33, anywhere in here, it's not going to do it. It is not going to do it. This is a real, real, real, real bearish gap that happened here yesterday and a follow-through today is amazing. And the target in this is 20, 22, 21. 22 is the first target in this for a short swing trade and the core target in this is even lower, basically 19, 18, 15. Really beautiful trade, beautiful, beautiful gap in BBY and why it's so important to understand how to read price. How do I know all the things that I know about gaps? It's because I understand how to reprice correctly and that tells me what the trend is in a chart and that tells me what direction to trade it, long or short and of course there would, you would never go long something like this. That is a real, real serious gap that happened in BBY yesterday and the follow-through today was amazing. Amazing. Just killer call I made in this here today. Let's come back this morning. Although you could have been in it really out of the gate. Sometimes you just know when something's going to go and fall off a cliff. I call this here 99 by 20605. 99 by 20605. I call this in the room. I said it's absolutely not a shot of a duck, $125. It is going to $25. I now have a 100% conviction it's going to $25 and not only did it not go to $25, it went to almost a dream target which was 2433 and like I said it just ran out of day 2441. The thing about understanding what you do as a trader and this is the talk that I had yesterday in reference to the conviction is when you understand what you do and you have conviction in your strategy, you have conviction in your analysis. It's the analysis that I have conviction in with what I do with reading trends and reading gaps and reading price and my understanding of technical analysis. When you have conviction in what you do, it is amazing how well you can read things and treat. It's one of these things where I talked about it yesterday in the lecture. You can't have too much conviction and when I see BBY today doing what I thought it was going to do yesterday but knew the gap yesterday even though it fell in the day was amazing, unbelievable, incredible, bearish gap. I can't tell you what this does from my conviction to see this year today. The system that I have to rate gaps is incredible. I am so grateful that I understand it, that I understand the market, that I understand gaps, that I understand how to read price and charts and trends and all of it. I consider myself so grateful that I know how to do it and for those people that are finding me to come learn from me, they're very special people because this stuff here that I am doing, that I'm seeing before it happens is by golly, it is special to be able to see that something's going to do like this today. This was not easy to see. It was not easy to read or even keep the conviction for the follow-through here today and that call that I made today, that six cents call I made today was incredible. Stock ran $1.50 from the call that I made with a six cent stock. So this is lower and on the day that this was on Thursday, I said hold on over night. Again, I don't do over nights, but so if anybody wants to do an overnight, hold it overnight. And if you held this overnight, you really got paid today. More sell-off happens today. Who's in control of the stock? Who's in control of the trend? The trend is down. Me, B, Y is in a down trend now. Boom, that's it. This is the confirmation. This is it. It's in a down trend. It's over for this thing now. It's over. I read something some analysts downgrade today that said the downgrade target was $33. That's above the price here. It's never going to get there. Never going to get there. This stock has been capitulated. This is crushed. This is over. This is done. That gap that happened yesterday was real. This move today is amazing. The follow-through will continue on this on Monday. Mark my words. And it's because I know how to read trends and I understand how to do this and gaps in everything. This is so crucial, so critical to having to be able to trade. I mean, how can you take signs in anything, whether it's a six cent stock or a 60 cent stock, it really doesn't matter. It doesn't matter. Seeing the entry and seeing the price and seeing what it's doing before it breaks, before it goes, before the momentum hits, that's what is so key. That's what's so special. That's where the skill is involved. To know. To know who's going to happen. I was one day early. One day early. This is what I wanted to do yesterday. So big whooped you do. I took the trade yesterday. I took a stop in it. Boom! Look at the thing that it did today. You got to understand what you do. And this is why people flip from thing to thing to thing to thing to thing. They'll flip and do 25 different strategies.  You have to know what you're doing. You can't be going short, long, the same stock at the same time. And not only that, you have to have conviction in your strategy. I read that gap yesterday. Spot on! It didn't work yesterday. Big whooped you do. It worked today and was a huge, huge, huge trade. And it's the lower. So you have to understand what you're doing. You have to have conviction in your strategy. You have to have a conviction in your targets and your entries and your sizing and everything. And that's how you make money. It's just so great. It's so great that I can see these things before they happen. I love what I do. I love teaching. I love trading. I love every time I do a class, it helps my conviction. Every time I see something important happens and it plays out that way, it helps my conviction. You can't have too much conviction. You can't make too much money trading. And you can't love what you do for a living enough. And I really, really, really love trading. Love trading. Love teaching how to trade. Love showing people that want to learn from me the right things to do what this is because it is very, very special. This is special to be able to see this here like this is a skill. This is not luck. This is 100% skill, 1000% skill. So amazing move on BBY today. Beautiful, beautiful, beautiful gap. The chart is lower. Next target is $22. And I don't see any reason why it doesn't get there next week. And then after that 1850, broke the number today, broke the $25 mark and couldn't get a breath of air to save its life. No rally, no rally, no rally in this today and all none, zero zip. Okay. So this is Melissa with the stockswish.com. If you're interested in more information on how to read trends so you know the right direction to take a trade. You know when to go long, you know when to go short. You want to learn how to do that. I'm doing the trends class next week, Wednesday and Thursday, 1pm to 5pm Eastern time. It's a great class on trends and explains a lot of information about understanding long term trends. This helps you as a day trader, helps you as a swing trader, helps you as a court trader. You know, you can't also learn enough from someone that can see because these are the types of things where it took me a long time to figure this out just trading and studying and really having no life for a long, long time just reading charts. You can do that. You can just take a class and learn from me and be in the realm. So great call today. This was a super, super, super duper. Some people actually think the gap feels work. Crazy. Doesn't work. This is a bearish gap. It's the charts broken here. BBY this is done now. Done. I can't tell you how bearish this gap is. I talked about it all morning yesterday. I talked about it all morning. I also talked about the fact there was too many people in the morning. And there was a chance it wasn't going to work right in the day. And that's why I didn't go crazy after. Look, this is gapping down here post market. Look, it's still moving down. Market's closed here. It's after four. It's still getting sold off. It's gapped out on Monday. It's gapping down right now from where it closed. Because it closed to 2440. It's gapped out on Monday morning. Look at the volume in this today. Actually, I think it's more than it was yesterday. No knock way. Still a lot. Alright, this is Melissa with thestockswitch.com. If you'd like more information or if you want to do the Trends class, it's next week. Email me at melissa at thestockswitch.com. If you'd like to sign up for the Trends class, what a great move and BBY today. Have a fabulous, fantastic weekend, everybody.


Right, ladies and gentlemen, Professor Zone, ladies and gentlemen, thank you for coming for the Class 6 Grand Rounds. When we organize this specialist Grand Rounds, the lads and I, we've given it some considerable thought as to the topic we choose, why we discuss it. We go to Grand Rounds after Grand Rounds, we discuss cases, procedures, how to do things. And we sort of wanted to introduce a little bit of a discussion as to what we do, why we do, what can we do differently, and is there a way to improve some things. I think one of the most rubbish books that I was made to read, not so long ago, was Pugul by Cheese. It's about 70 pages long.  And more people in the world have read it than I think the Bible, because it was the craze until about 2004. And basically it says change is good. It's done over 70 or 80 pages. And certainly change is good, as long as you focus in on the appropriate types of changes that not simply keep on changing.  So good things and bad things in life that come up in these meetings and books and things that we read. There are two sides to today's presentation. The first one is a subject that I think we need to make some changes in our management as an institution, as a division, as a department, as a group. The second one is a more philosophical one. When King Solomon was having his good days, he once had a sad day, so he got his wise men together and he said, I want you to do something that will make me feel happy when I'm sad. And so they came up with a ring and on the ring's inscription is that phrase that has now been used over the over the centuries, which the popes recite when they when they get inaugurated, which is this two shall pass. And so the point was made to him that you just look at your ring which says this two shall pass in Latin, and the sad days will pass. So he got the wise men together one day and he said, you made my life miserable because I'm more happy than I'm sad. And every time I look at the ring I realize that my happy days will pass too. So you can never feel them today. So this business about these two shall pass applies to our second presentation today. We forget that we are. We all know the sayings, the momento mori, the sick gloria mundi, all the phrases over the years that we've used to remind us of mortality and how life passes and that we're not invincible. And yet I was in the changing room a few weeks ago and a fellow plastic surgeon was changing and I heard the rattle of a bottle and he took out about, it must be 60 or 70 pills and he swallowed them. It was 7.30 in the morning. And I said, what are you taking? I can't imagine you're that ill. He says, oh, vitamin this, vitamin that, blah, blah, blah. You're supposed to anti-age and reduce your age in all this sort of business. I think we forget the fact that we are after all mortals and that the multiplication of our cells has an infinite number of times that we can split before our cells die. So that comes into play in our second discussion. As far as the first discussion goes, when we talk about diseases, we mustn't simply talk about what's the best treatment. There's an organization called NICE, which is present in the UK. There are similar organizations in Europe, National Institute of Clinical Excellence.  It's high time we had one. And what NICE does is it looks at those treatments that give you the most cost-effective results in a disease management process. So if something is excessively expensive and it only helps a very few patients for a very short time, they do not allow you to use it. I think this is not a matter of death panels. It is a very sensible approach. The converse to that is if you have a treatment that really works very well and in Charles' company, I can't say to you, you can't use this because the NICE organization basically tells you this is the best treatment for it. There's published proof that works. So before I let the first presentation be given, I want you to just listen to this little summary from the paper that was published just three or four years ago. This was forwarded to us by Professor Zohn. This control study had six patients who were in the study group and six patients who were in the control group. It was easy to choose the patients that fell into the control group because these were the patients who were refused the immunosuppressive treatment that we requested from the Charles' companies. So they automatically fell into the control group. All six of these patients were blind in both eyes. As it happens, the study group went on to show, or was suspected from other case reports, that by the way, been around for about 12 years, that you can save vision in at least one if not both eyes to a very significant extent and leave people independent. This is something we're dealing with in 2016. We still have this problem of treatment, controls, who can we treat, who can't we treat? So the first disease we're discussing is one that we all put our minds to as to what do we do, what do we change, how do we treat? And I hope most of you got a chance to see one of the two patients we're presenting upstairs. The juice is going to present the first one and stack the second one. Thank you. Thanks. Good morning everyone. So for those of you who didn't get a chance to see the patient upstairs, that's case number one that I'm going to go over briefly. There's a lot of different dates and details about the case, but I'll just try to summarize them as concisely as possible. So case one, she's a 57-year-old woman with history of diabetes. She presented back in February of 2015 to her optometrist, complaining of ocular irritation and sensitivity over the past three months. She'd actually been taping her lower eyelids to her cheeks because she'd found that that had improved her irritation. At that time, I just want to... This is important just to see where she started. Her vision was 2070 and 2040 with correction. She had some corneal staining that day and bilateral oral intropion. She was referred to oculoplastic service here at Moran. About a month, you know, six weeks later, she arrived at her oculoplastic consultation. She said both her lids were bothering her, but she had denied any previous problem with her lids. At that time, without correction, so this may not be as bad as it actually is, 2150 and 2100 in right and left eyes. She was diagnosed with bilateral lower lid secretricial intropion. And about two weeks later, she underwent a bilateral lower lid intropion repair and actually had a conjunctival biopsy of both lower eyelids at that time. This was repaired using a lateral tarsal strip technique and a reinsertion of lower lid retractors. This is a picture right before surgery, so you can see she's taped her lower lids here. A lot of conjunctival hyperemian injection and bilateral lower lid intropiants are with lashes touching the eye there. So the biopsy results came back a few days later from dermatopathology. The right conjunctival biopsy was completely normal. It showed some connective tissue fibers here, but in terms of the immunoglobulins here, they were all negative. So this really shows the utility of taking two biopsies in a patient, which was done here very wisely, and it shows the predominance of IgG IgG4 in the basement membrane zone and also IgA. So the interpretation from the dermatology department was that this was a predominantly IgA, but also a linear IgG, making a diagnosis of Pemphagoid and specifically linear IgA disease. So after the positive biopsy, this patient was called several times to the office to discuss the results. Unfortunately, she didn't show up several times and we actually made a formal appointment after the biopsy was confirmed to discuss this with her and she didn't show up a third time. So the patient obviously lost the follow-up, which will show why she progressed here. So she finally made it to the office a couple months after the biopsy and she's reporting that she cannot look up at that time. You can see her vision dropped to counting fingers in her right eye and her left eye as well. We requested an urgent referral to dermatology with Dr. Zone, who's present here today. In the meantime, we decided to have her follow-up with us and she canceled her appointment to Mr. Dermatology appointment with Professor Zone. So you can see the pattern here of poor follow-up. Urgently she called in a month and a half later saying that she was having problems, no specifics to that, but she'd actually developed bilateral corneal ulcers and upper lidentropian with chakrasis and here's a picture of how she looked when she called in that day. These are the quote problems that she was having. It's quite obvious what's going on. Bilateral ulcers, a ton of ton of inflammation here in the conjectiva with early fibrosis and you can see the symblephron formation and shortening the inferior fornix. So we urgently sent her to the, excuse me, the cornea service. At that time she was just light, clear light perception in both eyes. The left eye was soft, however the anterior chamber was formed, but it was shallow. They didn't see an active leak, but they suspected that there was kind of intermittent slow leak from that left eye. At that point cultures were taken, which grew staph aureus, or excuse me, just staph, I don't remember exactly what bug, but staph. She started on fortified vancomycin drops, also doxycycline and vitamin C to help with the corneal ulcers. The next day she underwent an upper lidentropian repair by this modified Trabu procedure and also the tracheitis was treated with the almond unit to both lower lids. She got in to see Professor Zohn later that week. At that time the history revealed that she'd been suffering from severe dysphagia, blistering of her throat and mouth and I actually had been reporting badinal symptoms for years, but those are unclear if that's a blistering in the vagina. She started on dapsone 25 mg daily and they submitted an insurance request here for IVIG and Rituximab infusions. We'll go over, that's the article Dr. Patel was alluding to before and we'll go over the benefit of those treatments. Her labs, after she saw Dr. Zohn, I'll go over this in more detail too, but there's an ELISA serum test that you can use to detect adenobodies and hers were obviously in the abnormal range here in red. She also had positive titers of both IGA and IgG in the basement membrane zone and the interpretation to this is similar to the conductive obiopsy, a mixed pattern of IgG and IgA and this was interpreted by Dr. Zohn to say maybe this has implications for her disease severity and possibly treatment monitoring. So in the meantime, next couple weeks she was following up with cornea molecule plastics and she had progressive thinning, new vascularization, congenitalization, some blepharone and I'll show some pictures of that. She started an oral prednisone to try to prevent further scarring and then you can see the timeline here up until, I think she saw Dr. Zohn here on the 14th a couple weeks later she finally started Rituximab, no IVIG but with solumendrol. Here's a picture of how she looked the next month and you can see this persistent staining of her corneas. This is after the upper lid procedure you see a nice eversion of the upper lids with the lashes away from the corneas that's really important. You can actually see some recurrence of the entropion on the lower lids here as well. So just some following of her labs we increased her dapsone further and followed this BP-180 lab that I referred to before and it's on a trend downwards however, because it was not completely normalized they considered starting IVIG which was not started. Overall she received 8 infusions of Rituximab as we followed her labs. Here's how she looks just last month in December. You can see this basically end stage disease here with conditivalization and scarring of the corneas, keratinization severe some blepharone and you can see that's how she looks today as well. Here's just from a couple weeks ago and this is how she looks today. You can see this basically 360 degrees of some blepharone and shortening of the fornex here just complete obliteration of the fornex. So very kind of end stage picture here. So the future plans for her there's nothing definite but Dr. Patel is planning some more lid surgery for her next month with a fornex reconstruction and a lower lid reconstruction as well. In the meantime she's been kept on dapsone lubrication with the rithromycin ointment and also a low-dose prednisone. We continue to epilator lash just to try to protect the cornea and then she's visited with cornea and the possible options cornea can comment on this later on but there's possibly a keratolimbal autographed with a transplant or possibly a keratoprosthesis and we'll discuss a little bit about that later. I just want to talk briefly about case number two who's not here today but a little bit different case given his long history but he's a 75-year-old man with history of diabetes. He was diagnosed with mucus membrane pemphagoid 12 years ago based on a pharyngeal biopsy. This was formally diagnosed as linear IgA bolus dermatosis. He also has a history of glaucoma in the right eye with steroid response. He's also had catarx surgery in the right eye. He was previously treated with cyclophosphamide and prednisone. He later went off of those immunosuppressants and then was restarted on celsept a couple of years later. I think the last time he had been on immunosuppression was about 2009. His visual acuity after his first bad, bad flare of pemphagoid he ended up blind basically blind in the left eye counting fingers. His right eye did not suffer any bad scarring of disease so he's fortunate to keep keep his one eye. In 2010 he had some blepharone surgery but he basically represented over Christmas in 2014 with new blistering in his nose, mouth and irritation of his eyes. Over almost about a month later he received a rituximab infusion and the same lab was drawn here. Nine is kind of the cutoff of normal but you can see this is elevated because below nine is normal and then he also elevated BP230 and another rituximab infusion. These are the only two infusions he received as far as I can tell from the records. His right eye dropped from 2030-2040 all the way to hand motion so now he's effectively blind in both eyes, legally blind in both eyes. He was seen in the dermatology clinic and this is I'll point out this gentleman's from St. George he had difficulty coming up to Salt Lake City and follow up so that was one kind of impedance to treatment here. He was started on dapsone 50 milligrams a day in prednisone. Over the course of three months he received three courses of solimedrol as well as some courses of IVIG that I'll show later and then we followed his labs. His BP-180 dropped down to the normal range so the BP230 he was kept on dapsone. At that point because of his antibodies were negative there was no more rituximab was recommended. His ocular surface was protected and tried to be treated with prednisone, restasis and tears and we also kept him on his alfagand to try to limit the damage due to his glaucoma. He received a total a total of 17 IVIG treatments I don't have the exact dates of those in the records though however. Back in September he had an ocular surface reconstruction with Dr. Patel. The plan for him was to try to blunt the inflammatory response by using prednisone a couple of days prior and up to two weeks after in a taper however the patient was confused about these instructions and did not do it unfortunately. The procedure Dr. Patel performed was amicus membrane graft. He resected some scarring from his cornea and resected some scar tissue. Next he was approved to 2050 from this count fingers hand motions type vision he had so a good improvement but as you can see what happens three weeks postoperatively you can see some chemosis and edema here where the reconstruction took place but his cornea is fairly clear you don't see that new vascularization his lashes look like they're in pretty good position away from the cornea and then about seven weeks postop you can see this progressive sub epithelial fibrosis shortening of his fornix he's got some irregular epithelium here and this beginning of this unfortunately it sounds like the procedure did not particularly take well and he's starting to progress despite immunomodulation so he was on the DAPS zone but he developed anemia he developed some heart problems as well he was taken off the DAPS zone and we saw him over Christmas time and at that time professor's own planned for further rituximab infusions and IVIG every month for six months and I don't know if this has been approved he's getting it now so that's a good news and then from the cornea standpoint what to do with this gentleman kind of the same plan possibly limbo autograph versus a K-PRO so that's where we stand with him so any comments from the audience so far just on these two cases maybe Dr. Patel or professor's own have any comments of anything I missed I have a lot of comments go ahead so I'm just going to review so mucous membrane pimphagoid is a group of chronic inflammatory and blistering diseases they affect the mouth, pharynx, larynx genital and anal mucosa it's characterized by these linear deposits of IgG, IgA or complement 3 in the basement membrane zone of the epithelium OCP is a type of mucous membrane pimphagoid that presents as a chronic sigatizing conjunctivitis the epidemiology of this it's relatively rare disease incidences quoted between 1 in 8000 and 1 in 46000 in ophthalmic patients there's differing numbers on there but they usually range in that in those numbers snow geographic or racial predilection average age of diagnosis is more elderly patients 60 to 70 years old however this disease may smolder along and present as kind of just a chronic low grade conjunctivitis for years and years before patients actually declare themselves or undergo a biopsy patients that are younger may present with more severe or rapidly progressive disease there's a slight female to male increase in the ratio there and it's actually rare in children there's only less than fewer than 20 patients reported in the pediatric literature pathogenesis of this is a systemic autoimmune disorder you can see of all the other sites that are involved there is an aberrant production of antibodies against antigens in the mucosal epithelial basement membrane zone there's several targets that have been identified this is a beta 4 of alpha 6 beta 4 integrin is one that's commonly cited in the literature and then this is the other one that I was referring to that people used to detect in the serum for possibly to follow the disease to see the response to treatment if it is positive so there's a couple of theories of why this occurs basically there's these autoantibodies bind to these antigens in the basement membrane zone this stimulates an inflammatory cascade release of pro-inflammatory cytokines and pro-fibrotic cytokines which lead to scarring there's a couple of proposed theories but nothing's particularly been proven there is some HLA haplitides that have been associated with it and there's also some links to topical ophthalmic and systemic medications that they term these as pseudopemphagoid and a lot of the glaucoma medications that I'll point out later have been shown to be associated with this the presentation the ophthalmic findings they can be unilateral as in the second case the gentlemen seem to have unilateral presentation but bilateral involvement is usually within two to four years the eye is the first side of involvement in one third of these patients they typically present with the chronic relapsing conjunctivitis redness, tearing, burning decrease in vision, mucus drainage form body sensation, etc the extraocular findings are present in the oral cavity in about 42% of patients with erosive gingivitis in erosions of the buccal mucosa etc can also present in the nose, pharynx, larynx and other areas in the body non-mucosal skin sites can be involved in 16% and then you have to remember this is actually a potentially lethal disease patients can asphyxiate from esophageal or tracheal stricture so it's something to keep in mind that this is just not localized to the eye can be deadly these are just a couple pictures that you may have the patient open their mouth and see this inflammatory response here of the gingiva and this is Frank blistering an ulceration of the gingiva here this is a kind of a confusing diagram I put together to confuse you all but so it basically starts with the inflammatory process and leading to sub-epithelial fibrosis and then there's three little three areas here that you can go down but they are all usually involved there's these lid changes that cause malposition of the lids and lashes there's the conjectival changes that lead to shrinkage, some blepharone and possibly ankle blepharone and then there's also destruction of the tear film in all three aspects of the tear film, the goblet cells, mybomine glands and the echorine glands so what does this lead to? Leads to dry-eye state leads to malposition of the lids, abnormal blinking and mechanical trauma from the lashes and exposure to keratopathy and then eventually the end stage is this corneal keratinization, neovascularization conjectivalization scarring and eventual blindness so the staging of this disease is proposed by Dr. Foster in the 80s stage one, this has been also been modified however stage one is a chronic inflammatory state with mucus discharge used as a rosemongal as a staining and shows sub-epithelial fibrosis I'll show some pictures of this next stage two is foreshortening of the interferophonics and this can be graded A through D depending on a percentage of how much it is shortened some blepharone is the next stage and also graded as a percentage A through D and stage four is the end stage and I'll show a picture of that and I think that's the category you'd put our patient upstairs so here's a picture of stage one disease, you see this white band underneath the conjectiva here, this is some beginnings of sub-epithelial fibrosis so this is kind of chronic conjectival inflammation here stage two you see some foreshortening of the interferophonics here, it's not as deep as it should be interferophonics is usually involved first as it was in our patients this is stage three, you can see the sub-blepharone formation here not all the way across but you can see a focal area of sub-blepharone and then stage four, just horrible, horrible disease keratinization of the cornea scarring, conjectitilization neovascularization, opacity of the cornea ankle of blepharone, just complete obliteration here of the foreshortening of this, just very, very sad end stage picture here in terms of diagnosis a biopsy is typical of what people do, a lot of people actually go ahead and biopsy an extra-ocular site if it's involved if this is negative there is high suspicion for the disease then the bulbaric conjectiva is frequently used I believe, I don't know where the biopsy is taken from in your case of our patient upstairs Dr Patel in fear of conjectiva they recommend taking a perilegional biopsy to avoid false negatives you don't want to biopsy the super, super inflamed conjectiva that may just show chronic inflammation and scarring and not give you the picture you're looking for negative biopsy however does not exclude the diagnosis of OCP direct immunofluorescence is done on all specimens at least at this institution it is 52% sensitive however if it's negative then they suggest using immunoperoxidase which increases the sensitivity to 83% and then positive immunohistochemistry does not rule out other diagnoses such as epidermolysis bullis aquisata or linear IgA bullis dermatosis so here's a picture of what you'd see on the direct immunofluorescence this is standing positively for IgG in the basement membrane zone here you can also see some other areas that look positive however this is normally inflamed conjunctivitis IgG is found in the conic taba there's also IgG found in the substantia propa here in normal specimens but this is what you're looking for here at the basement membrane zone so this is the test I was referring to earlier the BP 180 and BP 230 this is an ELISA test serum levels of this IgG basically BP 180 and BP 230 are these molecular structures found in the basement membrane zone that serve as antigens for the IgG to bind to they're in the negative range in normal individuals these antibodies are present in about 80% of both Pemphagoid patients and 20% of mucous membrane Pemphagoid patients it is highly sensitive and specific for Pemphagoid so this is just a long list of the differential diagnosis of a chronic secretarizing conjunctivitis the things that we as ophthalmologists would probably see more often would be maybe an atopic carotid conjunctivitis, ocular rosacea and there's actually a lot of association with Pseudo-Pemphagoid and topical glaucoma medication a lot of patients have intolerance of their glaucoma medications you try switching medications they still have conical time inflammation you may want to put this Pseudo-Pemphagoid type picture on your differential when you have a patient like that Pseudo-Pemphagoid the biopsy is negative for the linear stain that we showed however the topical glaucoma medications and systemic practology is a beta blocker we don't really use anymore has been associated with this and this is just a paper that showed the proportion of patients with drug induced Pseudo-Pemphagoid and what drops they're on it's kind of small here but the top of the list here was the beta blockers next was alpha agonist then goes down with pilocarpin, myotics and then latinoprost and the carbonic anhydrase are the lowest Pseudo-Pemphagoid picture so the goals of treatment in Pemphagoid number one suppress and control inflammation is a systemic disease this is your first and most important goal of treatment second is to promote healing number three prevent secretarization we cannot reverse scarring but we can hopefully prevent further scarring 75% of these patients may require systemic immunosuppression other tenants of treatment like I said is a systemic disease first and foremost immunosuppressive therapy should be administered only by those with experience not many of us ophthalmologists have experience with these immunomodulatory therapies however there are other medical subspecialists that we frequently work with that are very experienced in this make the appropriate referrals these patients need to see a corneal specialist someone that's very skilled in the anterior segment reconstruction perhaps down the line ocular plastics needs to be very very involved and also at this institution we frequently work with dermatology or basically always work with dermatology because of the systemic medications needed and it is a systemic disease these patients do need to commit to frequent follow-up that was a big problem in our patient here both the physician and the patient need to commit to having frequent follow-ups here so the treatment there's some stepwise treatments that have been proposed in talking with Professor Zohn in his articles that he's written the mild to moderate ocular disease you provide ocular care patients are typically started on DAPS zone between 50 and 200 milligrams a day for about 12 weeks there is less data on methotrexate celsaptase, ethylprin, but these have also been used with success in these patients severe refractor disease traditionally they've been used with psychophosphamine psychophosphamide does take a while to work there for your patients are covered with prednisone the role of steroids in this disease both of our patients were on oral prednisone a lot of that is to try to kind of cover them while they're getting heavier systemic immunosuppression but it can also control scarring but it's not enough to control this disease activity and basically the side effect profile of prednisone is not something that you would keep patients on long term I'll discuss IVIG and rituximab here but the basics of it is it can shorten the duration of conventional therapy in a study it reduced the duration of therapy from 8 to 4 months rituximab can provide long lasting improvement in 3 quarters of the patients over 24 months and then there's a combination of IVG and rituximab so that's what our second patient received here's the paper by Dr. Foster's group that Dr. Patel alluded to before 6 of 6 of patients in the control in the study group had no progression of their Pemphagoid disease and had no changes in their best corrective visual acuity that's really great numbers even though it's a small sample size and then 6 of 6 of the control patients those patients were put on infliximab cyclophosphamide and IVIG I believe these patients 6 of 6 became bilaterally blind there's no adverse effects noted from the rituximab group patients in terms of ophthalmic care when you see a patient like this aggressive lubrication is very very important some authors propose use of protecting the ocular surface with the bandage contact lens to prevent mechanical irritation from the lashes punctal occlusion is an option to provide more lubrication to the ocular surface topical steroids cyclosporine, tachrylimus there's kind of a limited role to those however they won't control the disease effectively but they can possibly limit the amount of scarring in the eye also it's important to treat patients for blepharitis to try to optimize their tear secretion management of trichiasis and distichiasis can be done in the office by epilating lashes however this is something that needs to be done monthly and there's also an ophthalmologist there's a high association of ocp and glaucoma some papers say up to 25% of patients actually have glaucoma that have this disease as well there's a little confusion is this glaucoma medication induced picture of ocp or is there just actually an association between the two diseases it's not really totally understood surgical considerations for these patients you want to make sure they're completely completely quiet before doing any type of surgery on them and these prior some studies you'll see that patients have been on disease modifying therapy for over a year to try to control their disease before any surgeries tried perioperative oral steroids as we suggested in our second patient lid procedures are typically addressed first if there's a stage procedure to be done with ocular surface reconstruction and then some authors recommend continued immunosuppression postoperatively for at least six months the nidus of inflammation from creeping out there eyelid surgery there's several techniques recommended for intropion repair which we won't go over tracheosis several techniques used to relieve that some blepharons Dr. Foster recommends just a simple license of some blepharon with the some blepharon ring placement and there's also techniques for foreignage reconstruction and several things available for use here mucus membrane grafting brain transplantation is quite popular anchoring sutures to try to deepen the forenecks and then some people will have to use mydomycin C to try to prevent the scarring effect that is of course not without complications with scleral melting and persistent epithelial defects so that needs to be used very cautiously this is a paper from a couple years back I think they had seven patients that they did 11 eyes and repaired the intropion and just this there's a lot of text here but basically the highlights of this is that the pre-operative immunosuppression was so so important these patients had a mean pre-operative immunosuppressions of over 15 months so it just goes to show these patients need to be so so well controllable for any surgeries tried they also really pushed for ocular surface protection pre-operatively with bandage contact lenses local entomical therapy and oral prednisone to try to prevent scarring then these patients underwent lid construction pre-operatively they had they used immunosuppression for at least six months so this just goes through the things I just mentioned on the last slide and all their patients had surgical success they had no disease progression control of inflammation and I believe their follow-up was over two years so in terms of cornea options I'm not a cornea specialist but there are several papers out there on what to do in these patients ocular surface reconstruction with amniotic membrane stem cell transplantation has been moderately successful there's a study of 11 patients where six of them were successful penetrating keratoplasty and lamella keratoplasty pretty poor success in the literature and then there's the option of keratoprosthesis type 1 has a very very poor retention rate at three years and not many patients better than 2200 type 2 keratoprosthesis where it actually goes through the lid has a much higher retention rate at three years all and so there's also some reports of using osteodontal keratoprosthesis however in these chronic psychotrizing diseases ocular psychotritional panphagoid is kind of towards the bottom in terms of success with osteodontal keratoprosthesis so what about cataract surgery some of these patients are going to go on to recover and be quiet for a while and then they may develop cataracts from being on chronic steroids etc and just in the age group they're in so I think it's important just to keep technique in mind clear corneal incisions are very very important here you want to try to manipulate the contractiva as minimally as possible there's some case series that document that the surgery can be very successful in ocp patients but they do recommend periopter steroids before after for at least ten days these patients also should be on there if they're on chemotherapeutic agent at least six months post-operatively judicious use of topical steroids with probably slow taper the prognosis for these patients it is a slowly progressive disease over ten to thirty years in most cases you would like to try to attempt to cease therapy after two years of patients being quiet there are long periods of remission off therapy achievable and about a third of patients however the relapse rate is unpredictable and these patients may flare just out of the blue and some studies say that up to 30% of patients with advanced contractival fibrosis do eventually become blind and like I said it's kind of unpredictable but despite optimum immunosuppression some of these patients still progress our patients both received retuximab and adequate immunosuppression however both of our patients are unfortunately effectively legally blind at this point some of the problems that I encountered through going through these cases of our two patients so this is a very complex disease there's multiple specialists involved different doctors different clinics sometimes it's hard to know who is it appropriate to refer to when do we have to refer these patients how soon do they need to be seen do they have to see a certain doctor can they see somebody else there are insurance issues and delays that I think definitely played a role in these cases and unfortunately the patients pay the price for that there's also access to care issues with our patient being from St. George and maybe not being able to come up here as often our other patient was maybe a little bit confused about the importance of follow up and no showed several appointments these patients just need to make sure that they're aware that this is a lifelong disease that they need very very regular follow up for some of my conclusions so like I said this is a systemic autoimmune condition patients need prompt delivery of immunomodulatory therapy and I think overall the university needs a better system as a whole to care for these patients some institutions have what's called a corneoplastic unit or corneoplastic team I think maybe a better collaboration between the cornea team and the plastics team to make sure these patients are seen maybe on the same day or maybe in conjunction with one another in disgust and what the treatment plan would better serve these patients obviously we work very closely with dermatology and professor zone I think maybe understanding the referral process and how to get these patients better seen more efficiently more quickly would also better serve these patients are there other physicians besides professor zone that if professor zones unavailable for some reason that we can see and get this patient in quicker I think that would also help the patients and then of course we all have problems with insurance but professor zone may be able to comment on this because he's probably intimately familiar with the insurance process of getting rituximab and IVIG approved but making sure starting the insurance process very early and if there's a system that we could use to try to expedite that for these patients of course rituximab is approved for lymphoma and rheumatoid arthritis and lupus it's not approved for this disease by the FDA so insurance companies aren't necessarily too excited to approve this for an off-label type use so that's obviously a kind of a barrier to treatment as well so I'd open any comments here that's the end of my presentation Dr. Lin I just want to make two comments I'm glad that you emphasized the importance of preoperative systemic human suppression because even with clear corneal cataract surgery if someone has OCP and is not even suppressed it can cause complete blood and titillization and the numbers that they were giving as far as success rates of Boston OCP was even more dismal than what you have presented so they're saying that the type 1 K-Pro which is the more common type has a 100% chance of failure in OCP and the type 2 K-Pro which is OCP has an 80% chance of failure even worse I don't know where those numbers come from but that was the general those are just the exposure rates I believe and not in us I guess that's a failure but those are over 3 years I don't know how long their study was Dr. Badi I have a few comments so I second Amy's in terms of the K-Pro Dr. Dolman told me I have a few comments first you do have a IVIG subconjunctival injection we have an option for IVIG we can try a subconjunctival IVIG it would be free to the patient second topical hydroxyprogesterone topical vitamin E ointment can be useful for these patients to help reduce the characterization and help improve the epithelialization third I believe Genentech does have a patient assistance program which would be eligible for that but Jim Rosenbaum in Oregon has an overview lastly just to give you some part of our good side to enjoy doing that Rutaksen I just checked the next website Rutaksen the UK is approved by NICE for as Zach mentioned similar diseases for which an anchor vasculitis is not approved for OCP and so like other biologics are often not available in that clinical trial in Oregon patients would have to travel to that site I know it's a multi-center okay I've dealt with this a lot for the past 40 years so I have a lot of comments 5 minutes please to start out with the diagnosis in the first case he showed something very very bothersome to us we get about 1,800 specimens from around the country a year and I would say probably 10% of those are conjunctival biopsies maybe 5% but we get a lot of them we do the reference laboratory, serology and direct amino fluorescence for ERUP what he showed was that when I was negative and one was positive that's very very bothersome because making the diagnosis is very important only about 20% of the people are positive on serum I would say 80% are negative so the first thing is making the diagnosis and biopsying conjunctival that is not so inflamed is very important as he mentioned because the inflammation if there's intense inflammation it will destroy the reactants that we're trying to find on direct amino fluorescence and that becomes a false negative or some people actually give us a piece of this for example those are almost universally negative scarring is a bad place to biopsy if you can get relatively quiet conjunctival I know a lot of these people don't really have quite conjunctival but stay away from the scar and stay away from the bright red stuff we have a much higher chance of positivity and we can increase that by 20% we don't really know I've had a lot of conversations with him in the article you were talking about in Up to Date he contributed that we've had conversations about it and he's done everything to try to increase his positivity by those immunoperoxidase things that you mentioned up to 70% so just because it's negative doesn't mean they don't have it and the second biopsy may be in order depending upon what your clinical suspicion is because of the diagnosis of cyclotrizing conjunctivitis obviously those people who have pseudo-pempegoid for the most part are going to be negative and are always a concern of ours because especially the ones we get in the mail we have no idea what things are on or anything like that so first point was diagnosis the second thing is 30 to 40% of the people have mucosal disease besides the mouth, the gingiva I do it all the time people have a little scar on their mouth or their eyes are pink I'll biopsy their mouth so if they have even a little bit of mouth disease we reported 8 people with esophageal stenosis back in the 90s and we biopsy esophagus anything to get a piece to make certain of the diagnosis especially those people who have IgA it turns out are more sensitive to dafts than people who have IgG so diagnosis, diagnosis, diagnosis really helps us like in this case when you can see the antibody levels going down in response to your treatment that kind of warms your heart from an immunotherapy standpoint so my entire clinical career has been devoted to immunosuppressing people and I would say probably 60% of the people I see are immunosuppressed I've treated probably a couple hundred people with rituxin so I don't really know I see a lot of Pemphagos and a lot of Pemphagoi so I would emphasize eukosmembrane involvement if they have just look in their mouth ask them do they have probably swallowing do they have sores in their mouth because if they do we can get a biopsy and make a diagnosis the next thing is treatment as was alluded to that the reflex is always treated with a lot of steroids that will cut down on the inflammation of the disease and it doesn't get rid of the antibody so it boils down to immunosuppression back in the 90s the only thing we had that worked was cyclophosphamide and foster and the people at Hopkins would always say if you don't give them cyclophosphamide that's bad I now have three of those patients that I'm following with bladder cancer so it's not without its complications and right now I don't think anyone would give cyclophosphamide when he was saying that things aren't approved because this is an orphan disease it's going to be very very unlikely that it's going to get approved so steroids, immunosuppresses I don't use much cyclophosphamide anymore I'm a great believer in rituxin and IVIG and as you alluded to that's a crisis with the insurance so I love that paper that Foster wrote on this topic six people got immunosuppression they're blind, six people got IVIG plus rituxin they can see you're responsible for this you make the choice I send that paper with usually a very nasty note I try to be very nasty to these people on the telephone I have to sometimes gather it up but it is in my nature so because I think if you don't fight for it first the first patient was obviously negligent we just see this in April when her first appointment was we might have been able to IVIG this way and rituxin but she just didn't show up and she I don't know, she had a bizarre opinion of what she had I personally believe that the best treatment is IVIG and rituxin if this institution that runs about $25,000 to $30,000 a month for that dosing $11,000 for the IVIG $11,000 for the rituxin and it's about $1,000 for the infusion so we're not talking small amounts it's usually I figure $30,000 a month for six months is the cost of this so it's very important that clinical trials be done to see whether or not it works we have a clinical trial on a B-cell poison for pemphagous right now for pemphagois the other thing that he mentioned that as a real disaster in this area is people who live a distance away I take care of these people and I have a junior associate Dr. Hull who I've worked with for a number of years and I'm encouraging him to see more and more of these people and he is and we see patients together on Thursday afternoons so there is hope to treat them but I always ask the question when I talk about this topic nationally and internationally actually and I always ask the question what would I do if I had this disease that's the essence of severe immunosuppression and aggressive therapy because this is aggressive therapy I've produced two microbial hemics who are on IVIG the rest of their life I've had some nasty infections I've never had anybody die on rituximab and the number of people I've treated IVIG I've had thrombi I've thrombosed the superior vena cava these things are not without problems so it's aggressive therapy but the question still becomes in the title of the talk I give what would I do if I had pephagoid not just skin but esophageal oral and if I had pephagoid and if I had pephagoid in my eye I would get rituximab and IVIG I would just take it for sixth grade months and neither of these people the one lady, the insurance company agreed to the rituximab and not to the IVIG and in the second one they agreed to one course of rituximab and IVIG and then I sent them the letter sent them the email or the the article and started arguing with them you know unfortunately in our system insurance companies are making these choices the medical person who is making them is usually a general practitioner or a family practitioner of some sort so that's a real crisis but I agree with your idea that we should have a more uniform approach when Maureen Lundergum is here she and I used to meet regularly to discuss these people but if we could have more of a group approach I think we'd be better off on managing them because the things that you were saying about and lip repair I know virtually nothing about but I can immunosuppress just about any case thank you I don't want to leave this hanging I think we should come to some sort of conclusion I go to too many meetings where we shake our heads and say insurance company this, insurance company that I'm beginning to see 30-year-olds and 40-year-olds with positive confirmed results for the past at least about 40-50 years of life and we need a team approach where we're courting as interested and that immunosuppression in some places it comes from rheumatology in other places we're lucky enough to have people like your team that needs to be established we had this problem with Maladoma we based a solid plan board and I and Antpaka got together a few years ago we set up a website-based approach where we have everything available for insurance companies to go to but we suggest to them that somebody needs a sentinel load biopsy we still have the same problem with getting approval and so on and we managed to overcome that by setting up a sort of a unit where we work together and it's very successful now and we rarely have problems with getting approval for biopsy sentinel load biopsy is lived under sanctions the major facial reconstruction that I do and so on and I think we need to set this up if Hal is your man who knows gender and interest in this and I accept it establish a team where people can reverse those patients too I'm all for it I'll do whatever I can to make that happen from our side I think that people need to be seen regularly the other thing he said is I don't think these people realize it's a very bizarre disease and I try to and then they get oh you're just trying to scare me no not really and so what do we what do we need to do as the next step to set that up do you think we'll be well first of all we're presenting this here because for the last three or four years I've been frustrated a little bit I've managed to get him sorted out this lady I've got three young patients now who are positive in the beginning to show signs of information coming on some of them you recognize plastics is almost an orphan child here we're dealing with the end stage this is the entropion this is a systemic disease it's inflammation that has to be controlled so whereas at the end of your segment the corneoplastic type of thing we're going to try to have a cornea that needs to be actively involved here there's some controversy about topical subcontinent travel as Mala was saying we need to be actively involved here so I think what we'll do to induce an eye is contact you and Hal and then we'll identify amongst our corneal corneal team who might be interested I used to do a lot of this with Hosefa and as you know between you and I Hosefa we still handle this pretty well so we just need somebody actively involved where we can say they should get seen on time every month by all three of us in an ideal way we'd have a clinic on the same day we can work towards that some time so look out for we'll send you an email we can play a web page and have some of this basic information available when you argue with an insurance company all the time some of these papers like Foster's paper there are a few others we didn't discuss which actually show there are more case reports than the study he did 6 and 6 we can have all that data available so in the next month we'll get this website paid setup hopefully we'll identify corneal that's interesting we'll set those numbers as a service that's provided when somebody gets a positive pharmacy or suspicion of Pemphloin because it's a stomach disease we'll get it seen by you all straight away Dr. Vitale? Yes, but thanks for this great presentation I couldn't emphasize more the importance of pre-operative in the expression of operative in the first patient reasons why I couldn't answer rapidly but in terms of the collaboration this is a disease it's like horrendous UDI outside of the office similar approach at least in UDI service we have a seamless liaison with rheumatology and with pediatric rheumatology so everybody is on board and it's been a forge over years of necessity because we have a very large number of patients as opposed to two patients with OCP that might come but it's been meet on a fairly regular basis and there's cross-fertilization in terms of educating one another about the drugs and the diseases so it's a seamless kind of referral that we have with pediatric rheumatology with all of the members of that department and in adult rheumatology it has been a little bit more difficult but we have identified individuals that are willing to take on some of these patients so part of the problem that you encounter is you have to suppress the patient with heavy duty immunosuppression for indications that they're not labeled and it requires a lot of work and so we work together to try to do these things and pre-office and manage them together communication is really, really important it can work I have a whole stack of doctors and insurance companies and I'm just like I have instances in which they just refuse it's a really bad situation John, the video upstairs is still active did you get a chance to see her today? No I didn't So she's still active we've been controlling the drugs so it's just with imagination I don't want to do big experiment drugs and more aggressive invasion so we'll make sure you get a chance to do it The insurance company improved 8 treatments with Rituximab they wouldn't improve the IVIG I'm sure the B-cell count is still 0 probably should treat her with IVIG intra sub-conjunctival IVIG is a fascinating idea there's actually papers of intra-oral Rituximab which makes almost no sense but intra-oral Rituximab working sub-mucosally and I wonder I have a sneaky feeling that intra-ocular sub-conjunctival Rituximab would work for a whole bunch of reasons that would take a long time to explain so I won't She's had a lot of Rituximab I think if we could get IVIG approved for her her insurance company under the exchange would broke that didn't help us she was insured by Arches which is one of the groups that wasn't supposed to be backed up by the federal government on the exchange and they weren't so somebody's got to come up with 20,000 a month if we're going to treat her with IVIG like we do with the guy in Boise who you know I go up to Boise and see Pentecost and Pentevoid people because they were being lost to follow-up up there every three months and see them all this guy's been on IVIG forever it's a struggle what is all of this stuff real expensive do you guys do what do you treat with is it real expensive do you do IVIG and Rituximab or anything like that biologics for UVIGs absolutely sure there are only a handful of patients that need treatments but you know what's mad it's always a problem it's not so much a problem in obtaining approval for those patients that have an indication so for example through the JIA there are people that fail to mention on immobulations that require biologics therapy and there you have a problem it's all insurance dependent and how aggressive in terms of most of the time we are able to as far as Rituximab is concerned we have the most common indication for Rituximab is actually intravitrile Rituximab for patients with intraocular lymphoma so we don't have a problem with that we just formulated here and the IRV is approved for that there are maybe two patients who are on Rituximab for Rituximab and one patient with autoimmune and one patient it's hard to get to use a lot of Rituximab okay in this bill Trump and ISIS refugees, Kardashians stags presentation is absolutely excellent it demands our presidents to discuss this in great detail it actually affects all of them how we practice this position I don't want to dilute it by having it presented for two minutes I'm going to organize another special day and I guess it's just as important a topic that we can present as this first one which I think will actually make a difference to our patients care now that we've had professors over here we've got an idea about how to at least put this together that was a superb prayer thanks thank you sorry about that


All aboard for the transcribe premiere production the crews of the Paul Parrot that exciting tale of wailing days In our last adventure we found our friends in all kinds of trouble on the strange island off the coast of brazil Where the good ship Paul Parrot has been wrecked by a great storm Mistow the fugitive magician and his black leopard controls a tribe of Indians on the island And he's taken captain Dalton prisoner when captain Karsh the privateer captain who was also cast away on the island with the Paul Parrot goes with his first mate briney to make a bargain with mistow a quarrel results in which mistow and briney joined forces But Karsh is trust up as a prisoner and thrown in the same hut with captain Dalton Meanwhile Johnny and Sue along with old Dickon and Paul Parrot himself have gone out on their own to search for captain Dalton Unknown to mr. Grange the ship's owner Annabelle Wilson and the rest of the Paul Parrot's crew back on the beach Johnny and Sue discover the Indians village and finally find the hut where Karsh and Captain Dalton are Prisoned but as they're debating where the captain Dalton is really held in the hut as well as Karsh Whose face they can see through the window Dickon suddenly shouts a warning to them and turning They see the black leopard slinking up on them as our story continues They stand frozen in their tracks almost afraid to move don't move Sue if you don't run he may not attack us Stay where you are you young ins I'll attract his attention over here.  Listen. It's Karsh inside the hut Oh, he's got some free. He's coming out of the hut and the leopard hears him. It's turning toward the hut Yeah And now Dalton you can stay in there a rot for all I can if you'd have talked to me and thrown in with me I'd have freed you but now you can see What are you two doing here and That's blue and black leopard stay away from me keep away He's after me keep away Johnny the leopard's gonna get Captain Karsh. Oh, it's horrible. We can't do a thing You know that leopard hates Karsh anyway. Don't run. Mr. Karsh. It'll only make the leopard spring on you Yeah, you brought him here you two Wanted this bloomin beast to get me well he has to catch me first. Oh, he's turning and running into the jungle We're safe for a minute here comes Deccan Blow me down and I've seen it all it looks like the leopard got the bloomin pirate in the jungle You two young ins of blinking lucky it turned out this way. Yeah, but Deccan the black leopard may come back Let's go in the hut away Karsh talk when he came out Captain Dalton must be tied up in there, too Betten down the edge. You're right, lad They're in the shadow Captain Dalton. It's us Johnny and Sue and I'm with him captain.  How did you get here? We've been hanging around the Indians kept trying to find where you were held captive and finally we heard Karsh yelling and We got her just as he broke loose. Hey the private here swabs been begging me to talk to him But I'll have no trade with the likes of him. I wouldn't answer and it fared drove him crazy Well, sir, we'll hear no more from him. We won't Johnny Let me help you untie those ropes from around Captain Dawson bless you Sue and you Johnny It seems whenever I'm in deepest danger you come sweeping in on the wind to help me I don't know what I do without the three of you, but Dickon What did you mean just now when you said we'll hear no more from Karsh? Well, sir, they'd bloom and black leopard that belongs to the white man who leaves these savages Came up just as the young ins here were looking in the earth When Karsh came out it trailed him into the jungle. Well, sir from the yelling we all heard There's been an enter Bruno Karsh and you may later that sir what the leopard attack Karsh It sounded like it captain just inside the jungle of assed.  It may not be too late to help. I'm going to look for Karsh, but captain It's good riddance. It is Dickon I can't see any man no matter how much a blacker he is suffer such a fate. Follow me. Oh there's captain You've got too big a heart. You can't keep out of trouble because of it. Dickon Let's look here in the underbrush. Maybe we can find Karsh's trail Look captain Dalton the underbrush is all flying down around here. It looks like a struggle went on here Betting down the edge. I'm afraid that kind of a struggle could have only one kind of an ending and here The bushes are crushed down all along as if as if something heavy had been dragged into the jungle. Oh, Johnny Johnny that means I'm afraid less. It means it's too late to do anything about it We might as well head back for our camp on the beach while the Indian village is still deserted as it seems now I'm still thinking captain. It may be all for the best We ought to be getting back to camp all right, you know, so we didn't tell mr. Grange where we were going and I'll bet he's worried about us That's right Johnny as we probably have some of the men searching for us. No, we'd better hurry and let him know that It's just one problem after another it wasn't bad enough to have Karsh and his band of cutthroats on this same island with us Then we find hostile Indians all around us captain Dalton disappears Obviously captured by these Indians and now there's no trace of sewer Johnny. I'm worried I can't help feeling that the children are all right, mr. Grange You said before that old Dickon was gone too. He's most likely with them in the woods I'd say jungle rather than woods miss Wilson and surrounded by enemies as we are it's hardly a safe place to go Besides we have no idea what's happened to captain Dalton mr. Grange Even though I've only been with your party for a short time I have never seen a man in whom I could put as much confidence as captain Dalton Somehow I feel that wherever he is. He'll be safe. I'm inclined to share your confidence miss Wilson I know of no man. I'd rather have on my side in an emergency George it's getting late. The Sun's almost down and still no sign of sewer Johnny I'm going out and have a look around. I believe I'll join you Mr. Grange the camp looks empty. Where are all the men? Well, most of them are still out searching for captain Dalton, you know And I sent mr. Jowd out with some of the men when Sue and Johnny disappeared Mr. Nicholson and one or two of the others are still over at the Paul parrot trying to patch up a few of the smaller damages Oh, it doesn't matter. It's so peaceful here The Sun is sinking behind the island and all the sea is red All the trees of the forest are in purple shadows. I'm not so sure that it's such a beautiful scene Heaven knows what threatening thing lurks in those purple shadows in the woods Perhaps right now we're being watched by some band of rogues wish to do us harm So that's the camp a brandy. Hey, mr. Matey. That's the camp of the blinkin Paul parrot crew Certainly looks as though we'd picked a good time There's not a man stirring Blimey scotch me rigging if I don't think they're all out out and for that bloomin captain Dalton You've got stowed in your up back at camp perhaps You think you can find the diamonds in the ship over there on the beach if my Indians take care of everybody at the camp Blimey mate I can find a bloomin minnow in the Pacific if you tell me which one you want Give me ten minutes. Don't worry my friend. No matter how large your force is here My Indians can keep them quite busy for considerably more than ten minutes I still think mr. Old Noggin It would have been a blinkin good idea to call some of my men from kasha's camp to help us on this job Oh, look briny my boy You admitted that it was wiser to put your not too beloved captain kasha way It means more for both of us. Hey, but his men would have stuck by me. I remind you again It's better to let no one in this little game who doesn't have to be My Indians will be quite enough. I assure you Hold on There are two people walking along the beach. Oh them. That's only as a grange the bloomin landlubber owns the pall parrot It didn't be frightened of the likes of him, but the other it's a woman. She's bad luck She is but hadn't been for her. We wouldn't have been shipwrecked. I says we when aboard ship is bad luck So help me but who is she? Annabella Wilson. She is our captain kasha's promised bride She was silent with us to South Africa to be married to kasha and she come aboard with him on the pall parrot just before that Storm that wrecked us when she found kasha was a privateer She left him and joined up with this bloomin crowd of whalers She's been staying with the little sister of that swab grange over there We search for diamonds and I find the pearl of great price hold on now mighty I tell you women is bad luck You raid the camp for provisions and I'll get the diamonds, but the woman's a Jonah silence You shall have your share of the diamonds, that's our agreement, but I shall manage my own affairs I'll be off of the ship.   I'll admit, but you'll grow to like it. No Hi spirits a but go with me you shall what miss don't want If only there had been more of the crew of the Paul parrot on the beach this might not have happened What have the Indians done to as regrange and what's going to happen to Annabelle? Will misto the magician really kidnap her will Johnny and Sue with Captain Dalton and Dickon return safely What about Karsh in the black leopard be sure to listen to the next transcribed adventure in the crews of the Paul parrot Until then this is your Paul parrot announcer Dave Ward saying goodbye