good evening everyone welcome to this webinar hosted by the IG nephropathy Foundation titled help take control of a nephropathy with tarpo this webinar is sponsored by khas Therapeutics we will have a question answer session at the end please put your questions in the chat box and we will answer them at the end of the presentation today we are excited to introduce you to Dr sa narui Dr narzi is an assistant professor of medicine and clinical nephologist at Linda University Medical Center she completed her fellowship at Baylor College of Medicine in Houston Texas she is a founder and director of the glal disease clinics in lalinda she's also co-director of online glom con Glaria diseases fellowship program she has been selected as the educator of the year for three consecutive years and is a member of Alpha Omega Alpha Medical Honor Society she has a special interest in GL glal diseases and is involved in multiple research projects and clinical trials welcome Dr nari well thank you so much for the kind introduction I'm really happy to be here I'm really honored to be here as well um you know um we're today we're going to talk about one of the topics that is really really close to my heart I have a glomular disease clinic here on the east side of LA and I have a huge patient population with Ian so this is something that I deal with it on a daily basis basically I I do research projects I see patients with Ian I do clinical trials um I like prescribe new medications for them and this is something that is really really close to my heart again thank you so much for having me today and today this is the agenda for today we're going to talk about Ian we want to know actually understand why IG propop is happening in some people and that's one of the questions that most of my patients they ask me why me and why is it happening to me and then we're going to talk about the new medication that came out tpeo and how we can preserve your kidney function then we're going to talk about tpeo touch points and then we have one of our patients here Katherine who's going to share her story and her experience with a new medication tarpo and after that I'm going to take questions I'm going to answer your questions so um I think understanding IG nephropathy helps a lot helps Physicians helps patients because when you are a patient and something happens to you which is called an autoimmune disease it sounds scary and it's not like one of those common diseases that you hear about it all the time it's not high blood pressure that you've heard it like you know your family members they have it or like your friends they have it this is some kind of like an uncommon disease and you hear the words autoimmune your immune system is attacking your body so it sounds scary but I feel like having a better understanding of what's going on inside your body when you're getting diagnosed with again it's going to help a lot to navigate your treatment options and to have a better understanding of the disease State as well so what is IGN property it's an Progressive autoimmune disease and by autoimmune disease I mean that your immune system is attacking your body meaning that your immune system is got activated for some reason you got an infection that you didn't even think about it it was a simple infection like a cold like you were just sneezing for a couple of days you had a sore throat and you're now fine and all of a sudden your immune system is just not calming down it's activated and it's attacking your own body because your immune system it feels like there is something that your immune system wants to remove it but there is nothing there and that's the foundation of like whenever we say that it's autoimmune disease it means that your own immune system is attacking something that is not there and it's just attacking your own body how does it happen like how does your immune system is a affecting your body in the setting of I again is that in the setting of IG property you are producing a kind of antibodies and uh antigens that is called ig1 this ig1 is not normal and your body doesn't recognize this so your immune system gets super activated to remove this and in order to remove this ig1 you're going to produce a lot of antibodies so these antibodies and antigens they're going to fight with each other so your the antibodies are trying to remove iga1 and they're going to form something that is called an immune complex this immune complex is really like now a bigger are molecule and not your kidneys they have special receptors for this kind of immune complexes so they are circulating in your body and they sit on these special receptors on your kidneys and once you have these immune complexes sitting on your kidneys you're going to have more inflammation there because your immune system is going to go after this immune complexes and trying to remove those immune complexes from your kidneys so you're going to have more inflammation there and over time you're going to have irreversible damage because because of the fight between your immune system and your kidneys that they're trying to remove these immune complexes because of that fight over time you have some you have some um inflammation like chronic inflammation there that over time it's going to affect your kidney function and you're going to have kidney damage and your kidney function is going to drop over time how do we diagnose IGN property so the thing is that when I have patients that they have symptoms and signs of IG nephropathy and I'm suspecting IG neop I kind of like in my mind I feel like okay I I I'm suspecting IG neop but I cannot say it for sure until I get the kidney biopsy and the way that kidney biopsy is done is a procedure as an outpatient it usually takes 20 minutes or so they're going to take a sample of your kidney like a really small pieces piece of one of your kidneys just one and then we're going to take a look at that sample under the microscope and under the microscope we're going to see this ig1 that I'm talking about it's going to light up under the microscope and this is the way that we can diagnose Ian and this is the way that we can tell you that you definitely have IG nephropathy and um so for that reason in order to diagnose someone you're going to need to to do the kidney biopsy so um something that is really really interesting about IGN property is that we think that so this is a disease that is affecting my kidney function so everything should be in the kidneys but the research shows that there is a relationship between your gut and kidney and that's super interesting and this is something that is all these research projects all these new medications are focusing on the connection between on the connection between gut and kidney what is this connection that is everyone is paying attention to it these days and this is the most interesting fact that we know about Ian is that that when I mention that we have this production of ig1 from your immune system this iga1 is the main one of the main sources of the production of iga1 is your gut so it's coming from this piece of like it's a really specific part of your gut so your gut is producing ig1 and because you have a lot of ig1 your immune system is going to get activated trying to remove that you're going to produce antibodies and then you're going to make those immune complexes and then those immune complexes are going to sit on your kidneys so your gut is actually doing something in the setting of of IG nephropathy and there is this huge connection there is a strong connection in the setting of IG nephropathy so it would be interesting when we are looking at treatment options for IG in property to see that if we can stop this connection right if we can stop this process somewhere if we can make the gut not to make ig1 maybe we can stop the process and maybe we can stop the progression of the disease on the kidneys because if you're not producing a lot of ig1 maybe maybe we can solve the problem for IG in their property so what are the potential signs and symptoms of Ian and so when you are getting diagnosed with IG nephropathy and someone tells you that you have a specific disease on your kidneys that is called IG nephropathy it is an a autoimmune disease and you're producing a lot of iga1 so you feel like okay so how is it going to affect my life and then you think about your own symptoms a lot of my patients they mention that they've seen blood in their urine at some point but if you haven't seen blood in your urine it doesn't mean that you you do not have IG property because the type of blood that we see in the urine sometimes it's only being we can see it under the microscope so you're not able to see it like when you're trying to pee like you look at your urine and like it looks normal to me but then you go to your doctor you go to your nephologist and say like you have blood in your urine because the type of blood that we see in the setting of IG nephropathy sometimes is only the type that we can see it under the microscope and then your urine might be might be a bit foamy and the foam that we are talking about is that when you're trying to flush the foam is going to stay it's kind of sticky and it's it's not going to go away and that's the protein in your urine that is looking like bubbles like a sticky bubbles there you might have some swelling on your legs and you might have some lower back pain as well by the time that you are getting diagnosed by by IGN propery most of the times you have some kidney damage on your kidneys and this is unfortunately something that we see in the setting of in the United States because we do less biopsies when you compare that to Asian countries and a lot of my patients when they come to clinic and I tell them that okay we have to do the kidney biopsy we see that already they have already CKD stage two or three meaning that they have chronic kidney diseases stage two or three meaning that they already had some damage done to their kidneys at that time so how do I monitor my patients with IGN property when you get diagnosed with IGN property I'm sure that there are a lot of things that come comes to your mind how can I preserve my kidney function how can I treat this med this situation this I what are the medications out there and what's going to happen from now on every time that I come to my doctor like am I going to do blood Works am I going to repeat my kidney biopsies what's going to happen to me and those are a really good questions I always try to um you know have like a really long visit with my patients and tell them about everything that's going to happen from now on because I feel like it's going to come with a lot of anxiety when you you don't know what's going to happen at the next visit there are two things that I'm going to look at your um labs when you come back for the next visit I'm not going to repeat the kidney biopsy we usually diagnose a patient with IG nephropathy with only one time kidney biopsy and from Once you you're done with your kidney biopsy and you got diagnosed with IG nephropathy from now on you're going to do blood works and urine test we're going to do blood works and we're going to check something that is called estimated GFR and that GFR is going to show your kidney function so the higher GFR is is better number so we want to see high gfrs and the number ranges between 0 to 120 and for example if your number is more than 100 that's amazing that's normal if your number is dropping like your GFR every time that you come to the visit and your GFR is dropping from 100 to 90 then 80 it means that you're like having more more of a kidney disease and your chronic kidney disease is actually getting worse and then we check something on your urine sample it means it it says PCR and it means urine protein to creatin ratio meaning that that foam that those bubbles those sticky bubbles I want to measure them and see how much protein you have in your urine because the more protein that you have in your urine it means that you have more aggressive type of IG property and you need more treatment for it and you're at risk of losing your kidney function much faster so basically we do not want to see any any protein in the urine so basically we should see Zero protein it should be zero so any amount of protein in your urine is not normal and as long as this number is going up this upcr is going up it means that your igen is not under control and we have to think about something we have to add like a new medication to it we have to do something about it as long as you have your PCR a positive upcr and you have a lot of protein it means that your disease is Super Active but you know what one thing about kidney disease is that once the damage is done we're not able to reverse that so all the things that we do in the setting of kidney disease we actually want to prevent that kind of a damage so when you come to me I say like I want to start a treatment as soon as possible because once the damage is done I'm not able to reverse that but I want to prevent that so everything about uh kidney disease is to prevent the kidney damage not to reverse that so for example if you come to my clinic and you already have some degree of chronic kidney disease no one can fix that but if you come early and we diagnose you early we can prevent that damage and that's the key about H NE propy that we want to diagnose you we want to do the kidney biopsy as soon as possible we want to diagnose you as soon as possible so we can prevent a chronic kidney disease for you so but how can we preserve your kidney function there is I'm going to talk about a new medication today as we all know and the medication is called tarpo tarpo is the first and only FDA approved treatment that is proven to reduce the loss of kidney function impatient with IGN property and it's been studied in adult patients so we don't have data in children yet but what is tpeo tarpo is a capsule that you take it in the morning and it's um it's a prescription medication so you have to go to your nephologist and they're going to prescribe the medication for you and the way that tpeo works is that it's a targeted release capsule it doesn't get released until to it gets to that point on your gut the point that is making a lot of ig1 and sits there once it gets to the certain level like once it sits in the C on a certain receptors and gets to a certain pH this capsule is going to get opened up and it starts working there so it's like a really amazing technology um you know like as a physician I really get excited about new medications and when I hear that it's targeted release and it doesn't get released as soon as you swallow the the capsule um it sounds exciting to me because as a physician I want to use targeted Rel these medications for my patients a medication that doesn't get released as soon as you you take the medication because then the side effects is going to be much higher then you compare that to a medication that doesn't get opened and doesn't work until it gets to the point that it should work so that's like an interesting technology that we have with tarpo and these specific cells in your guts they are already super activated and they are already making a lot of abnormal ig1 so this capsule is going to go go go go until it gets to that c like certain level in your gut it's going to sit there it's going to get opened up and it's going to decrease the production of this kind of abnormal IGA one that you are making so uh from the pathophysiology standpoint it makes sense because you know as I said like in the setting of IG nephop you are making a lot of ig1 from your gut and this medication doesn't get opened until it gets to the gut it gets open there sits there and it's going to decrease the production of ig1 so hopefully it can stop the process of IG nephop because if you're not producing a lot of ig1 you're not going to make the immune complexes and then the immune complexes they're not going to sit on your kidneys so it makes sense that this medication should be able to stop the process of IG neop disease so but how do we know that tarpo works so the way that a medicine we all we we do research on new medications is that we do clinical trials and clinical trials it means that we have patients that they've been diagnosed with IG nephropathy uh we talk to them about this new medication that sounds promising and if they want to go into a clinical trial so we have patients that they sign up to go into a clinical trial and they receive the study medication for like nine months in this study and they agreed to get followed up for two years so for tarpe we had 364 patients entered to this study and these patients they get divided into two groups one group they receive tarpeia and the other group they only receive sugar pill like a placebo so because we want to compare like we want to compare the group of patients who are receiving tarpo to patients that they did not receive tarpo and see that if actually tarpo is going to work for IG propery so these patients they took the medication they took the capsules for nine months and none of the patients they they don't know if they are taking tarpo or they're taking the sugar pill or they're taking the placeable so they have no idea The Physician who's treating them they have no idea if they are receiving tarpo or not but in the background they receiving the blood pressure medication which is known to help IG property as well so all of them are taking the blood pressure medication but some of them are receiving tpeo for nine months so during this time we're going to check their egfr and we're going to check the amount of protein and they have in their urine and then we're going to see if the patients who are receiving tarpo if they're going to do better at the end so this is study they actually followed these patients for total of two years for 9 months they were taking the medications and then after that everyone stopped taking the medications but they continue to follow up the patients for 15 months because we wanted to see that if you finish the treatment with tarpo are you going to see the effects of tpeo after finishing the treatment for a couple of months after that or not is it going to preserve your kidney function for a longer time or not so that's the reason that they followed patients for two years to see if actually tpeo works in the setting of IG property um okay so here here are the results so basically they did the blood works and urine test on these patients every um depending on the research protocol that they had but over the two years they had many many data from these patients and they compared the kidney function from the group that they received tpeo compared that to the patients that they only received the blood pressure medications and as you see this is the egfr data and patients who received tarpo they had less reduction in their kidney function over two years so for example patients who did not receive tarpo they had a reduction about 11.2 points in their kidney function comparing to patients who received tarpo which was 5.3 and then they do the statistical analysis between these two numbers and it was significant obviously it shows that if you're taking tarpo it's going to preserve your kidney function at least over two years that we are we we're having the data for it so this is something that sounds promising as a physician you know I'm a nephologist I'm a kidney doctor when you come to my office in my mind I want to preserve your kidney function I want I don't want my patients to end up on dialysis I don't want that you know like to follow you up and say that oh by the way you need transplant I want to do everything that I can to preserve your kidney function so you do not end up on dialysis or you do not need transplant at the end and this data sounds promising to me because you know I see that patient patents who received tarpo they had less reduction so this is something that shows that actually patients who are taking tarpo they had less than 50 like you know you had more than 50% reduction decline in the kidney function versus um patients who received blood pressure medications alone so this is something huge you know more than 50% reduction if you're not taking tpeo um and then you know what they compared the upcr so upcr is the amount of protein that kind of like you know bubbles the sticky bubbles that you see in your urine on the protein that you see in your urine and they compared that between patients who were receiving tarpo uh and the patients who did not receive tarpo over to nine months and as you see the protein was reduced significantly in the group that they received tarpo 34% comparing to 4% so again as I said in the beginning if you have more protein in your urine it means that IGN property is going to affect your kidney function over time and if we can reduce the amount of protein in your urine with any medications out there it means success to us these are the numbers that I follow in the clinic when you come with IG nephropathy and if I see that the urine protein is going down it's actually such a good sign and it makes both of us happy I was like okay this is such a good sign that you it sounds like you're responding to the medication and you have less protein in your urine and it's just not about the upcr this is something that we can actually calculate that from your urine the less protein that you have in your urine you're going to have less edema you're going to feel better you're going to have more energy because your disease is less active and these are the things that you cannot measure in a clinical trial study this is something that comes from the patient side that I see that my patients are doing better because their numbers are getting better and sounds like it's controlling the disease so uh what did patient when did patient start to see results so this is something that happens in my clinic so whenever I start tarpo for my patient patient um we both are excited and so like okay when should we check your Labs next time to see that if you're responding to the medication so during the clinical trial we had um they followed up on the patients and basically we should be patient with the medication you're going to see the results um around like three to four months after starting the medication so I usually tell my patients um once I start the new medication tarp I'm going to see you back in two months just because I want to make sure that you're tolerating the medication well we're going to check your blood pressure we're going to talk about like your new medication I want to make sure that you don't have any issues but we're going to check your blood test and your urine test in four months because you know um I know that the medication takes time to work and I don't want to check numbers when I know that it's too early to check this um in this study in this clinical trial tarpo was a studied for a two-year clinical trial and it included nine months of tpeo treatment plus their prescribed blood pressure medications but it's and the the fact is really important that you need to stay on tarpo for at least nine months you know to complete the treatment course it's not like you can take it on and off or take it for five months and then stop it you want to take your medication for the full nine months to have the full benefits of the medication otherwise if you take it on and off then you might not see the full benefits is that it's the same as the same medications if you're not taking your blood pressure medications every day you're not going to have a good control on your blood pressure if you're not taking your diabetes medications every day you're not going to see the full benefits of the medications as well so the same thing once we start the medication we have to take it every day and then we're going to check your Labs first time in three to four months to see if you're responding what are the safety profile of the tarpo tarpo is generally well tolerated there are some side uh side effects that uh it was reported from the clinical trial uh some of them are one of the some of the most common side effects are swelling on the legs your blood pressure might go up during the treatment with tarpo but the um the amount of number of like the amount of high blood pressure that we're going to see like the amount of increase in your blood pressure is not going to be super significant and then you're going to have some muscle spasms you might see some acne headaches uh we always tell our patients your immune system might be a bit suppressed so you have to be careful about upper respiratory tract infections your weight might be increasing so the median increased weight in during the clinical traal was about 2.2 pounds so it's not a lot when you compare that to systemic cortical steroids um which is something that I try not to do for my patients as much as possible and usually at the end of the treatment you're going to be able to lose the weight first of all it's not that much of a weight it's about two pounds and then um usually like it goes back once you stop the treatment you might have some indigestion um you might have some inflammation inflammation of the skin some joint pain and then increase white blood cell counts but it doesn't mean like for example when we check your CBC we might see that your wbcs are increasing but it doesn't mean that you have an infection it just could be a side effect of the medication and we don't have to do anything about it we're just going to monitor those numbers for but you know for before taking any medications you have to go to the website for tarpo just check everything on there because I always believe in that I always tell my patients read about it we have brochures we have informations about tarpo I give it to my patients um I think it's really important as a patient to read up on the medication as well um this is a new medication you want to know everything about it before starting taking the medication so um you know 87% of the patients in the clinical trial study they completed the two-year study so it shows that you know usually in the clinical trials because it's like clinical trials they usually run for two years one to two years and it's really hard hard to keep track of all those those patients a lot of patients they stop treatment in between or they have some side effects of the medication but 87% is such a good number that it shows that a lot of these patients they were tolerating the medication really well and the side effects weren't that bad that they continued the study for two year and they came back for their blood test and your their urine test like every time so um this is a good number like I always like when I'm reading about clinical trials I look at this number because I want to see that how well tolerated is the medication in general because if they tell me like 40% of the patients completed the treatment I know that the medication is not getting like tolerated well but 87% is such a good number and then we mentioned about increased in blood pressure which was a small and I see it in my uh Clinic as well that blood pressure goes up but it's not something that makes you nervous about it it's not something that we say that it's not um manageable you know and my patients they don't have a lot of symptoms as well so the increase in the blood pressure is going to be mild and then A1C for people that they have diabetes the A1C was generally unchanged throughout the treatment so you do not have to worry about your blood sugars that much when you are taking tarpo and I think that's really important because you know diabetes is really common and a lot of patients are dealing with diabetes as well and having this fact that A1C was unchanged throughout the treatment that's something that gives us a peace of mind that we don't have to worry too much about diabetes and the weight gain as I mentioned the um increase in the body weight was minor it's not it wasn't more than 2.2 pounds and this is something that you should be able to return to your original weight within three months of ending the treatment so you should be able to lose that weight and um you know it's not that much um anyways and I usually tell my patients that it's a good time to address your lifestyle as well try to exercise more try to eat healthy as well because it's going to help your kid K as well and it's going to help you to maintain your weight when you are taking tarpo as well so unlike other products like for tarpo you do not have to do like you know check your liver enzymes you do not have to do pregnancy test during this um um taking this medication so it's not like you know a medication that you take and then you have to come back so often to do Labs um you get your prescription and then next time that you need to do your blood works and your urine test is going to be in three to four months so how do you take tarpo when I'm prescribing tarpo it's usually a nine months treatment course and um it's a capsule so you're going to take four capsules in the morning and we want you to take it before breakfast the first thing in the morning so you wake up you take your tpeo at least one hour before your breakfast because as we said this medication is going to go and sit on a special s of your gut and that's the reason that is related to the food and we don't want you to take tarpo with food so take it first thing in the morning at at least 1 hour before a mail and you do not Crush you do not chew or you do not want to open the capsule you just take the capsules and do not take grapefruit juice with it because there is an interaction between grapefruit juice and uh medication and then um you know take it as prescribed and once you're done with the treatment in order to taper down the medication we're going to go down to two capsules for two weeks and then you're done with the treatment so tapering down tarpo it's not that complicated as well so as I said the treatment course is usually 9 months it could be longer sometimes and then um once you're ready to taper down we're just going to go down to two capsules and then for two weeks and then you're done you can stop taking the medication so um one thing is that um it's really important take it before breakfast one hour before breakfast and you don't want if you forget taking it and then you want to take it next time like you know it's going to get mixed up but at least you have to make sure that when you are taking it it's at least 1 hour before meal so you don't want you don't want to take it with lunch or dinner so you know what the reason that we have this discussion today is that I want you to advocate for yourself so um I'm on the east side of LA and I get a lot of referrals from the community and a lot of my patients even though that they've been diagnosed with IGN propop they do not know about these new treatment options so they come to my clinic and feel like oh okay there is a disease that I have it's called IGA and there is no specific treatment for it there is a specific treatment and the more you know the more you can advocate for yourself and uh for example you can go to your primary care doctor and say that I've heard that there is a new treatment for IG nephropathy can I see a nephologist or do you think if you can have more information on it maybe you can help me to get tpeo you know like because um the more like you advocate for yourself it's just TR it's just a truth you're going to get more from the healthcare and you're going to get more the options you know because sometimes you're seeing the best physician and they are the best but they just not be updated on IGN neop which is such an uncommon disease and um you know your primary care doctor might not be updated on IG nephropathy and might take months to see a nephology so the more you know you have you can you know you can have more options in um maybe you can have access to these medications much faster than other people so I'm really happy that we are talking about this uh medication today because this is something that um you know can change the disease course and can help you in the future not to go on dialysis this is huge and this is something that you actually everyone should advocate for themselves this is something that we have a treatment out there and we should know about it and um I want every patient to have access to this medication so we can preserve your kidney function what are the things that you can start like what are the conversation starters that you can do that can we discuss that this is a specific treatment option like tpeo based on my re ENT lab results could tpeo be an option for me you know you can bring up tpeo and see what your doctor thinks and if they think that you are a good candidate for it or um what results might I expect once I'm on tarpo do you think that tarpo is going to change my disease course do you think that it can affect um you know my health in general do you think it can save my kidneys over time or not so how long will I need to take tarpo I've heard about this new medication and uh you know I want to know how long do I need to take take it what are the side effects how is it going to help me these are the things these are really good questions that U we can we all can ask our doctors in every situation you know there are things that it's not in my specialty as well and the more you know you can advocate for yourself and your family members and that's the reason that I think that it's really important to have such these conversations so uh tarpo touch points uh is um a team of support that can navigate uh your treatment needs they can navigate like how like prescription can be done this is something that I do in my clinic um there's a specific form that we fill it out it have we have to send it to a special Pharmacy so it's this not a medication that your physician can just type it in on the system you can actually send it to a special Pharmacy and then you order tarpo and um they have a group that they help us for example if the insurance company is not able to cover it they have a coverage plan that they actually provide the medication for my patients as a bridge until we can solve the issues with the insurance companies so this is something that um they can track your prescription shipment every month and these are the helps that like you know actual life there is one point about prescribing the medication and there is another thing about receiving the medication and touch points can help you to actually receive the medication and yeah as I said they have Financial programs which I think that it's important and I have a couple of patients that they use the bridge and um you know until we were able to solve the issues with the insurance company so you do not have to wait you know months to in order to receive the medication so these are the things that again are important to know on the patient side as well so you can advocate for yourself and receive the medication as soon as possible and this is how you can contact them yeah so um as we said the indication and important safety information for tarpo um tarpo is the only FDA approved medication for IG nephropathy um this medication was studied for adult patients in IG propop so we do not have the information for children at this time and I'm sure that we're going to have more clinical trials in the future um if you are allergic to budesonide which is the actual the actual substance in tpeo then you're not taking the medication it's like any other medications if you're allergic to it then you're not going to be a good candidate for it and always always review the important safety information on the website just to make sure that you know about all the side effects before starting uh taking a new medication and as we said these are the safety information and tell always always tell your doctors um tell your doctor about your anything that has been going on in your past medical history if you've had stomach ulcers in the past if you had osteoporosis in the past if you've had high blood pressure like these are if you have an active infection right now or had chicken pox or M meel uh or you've been near someone who had chicken pox recently these are the things that we need to know uh before starting tpeo um if you have like if you have traveled outside United States recently and you stayed there for a long time and you think that you might have TB tuberculosis that's something that you need to discuss it with your physici as well if you have history of diabetes as we mentioned tarpo doesn't affect your A1C that much but we want to know everything about your past medical history when we starting a new medication so always mention everything in your um medical history and medical background when you're seeing a new doctor I get a lot of referrals in my clinic because I have a Subs specialty clinic for igny and a lot of patients when they come to my clinic I don't have any information on them I just know that they have IGN propop so we always go through everything every medications that they are taking all the health issues just to make sure sure that we are offering the best treatment options for them yeah so these are how you take tarpo it's once a day as we mentioned it's going to be 1 hour before a meal and you want to take it first thing in the morning that's the best time to take the medication if you have any side effects if you feel like you're taking tarpo and something new happening always tell your P your physician right away and um if you miss a dose um just prescribe it at the next schedule dose so you do not want to take two doses at the same time so for example if you take like if you forget to take your tarpo um this morning you do not want to double the dose you know the next day because you miss the day you just like continue the continue like you know as the next schedule time um this is the way that like we don't want to cause toxicity as well so as we said uh there is like an interaction between grapefruit juice and tpeo so eating grapefruit or like drinking grapefruit juice can increase the level of tpeo in your blood and can cause toxicity so um this is something that we always tell our patients you have to be careful about that uh during the time that you are taking tarpo uh tarpo can have like if you get tarpo toxicity could be too much corticosteroids medicine in your blood meaning that like it's going to be like an stress hormone it can cause more acne for you it can cause more weight gain it can cause like some swelling and it can cause like you know some fat pads around your shoulders so these are the things that you might see if you to you take a lot of tarpo and tarpo like substance has some effects of cortico anyway so that's the reason that it's really important to make sure that you're not taking like grapefruit like grapefruit juice with the tarpo or you're not doubling your dose because we don't want to cause this kind of toxicity in your PL and then uh we talked about this possible side effects of tpeo but as we said because the substance is like close to cortical steroids it can cause some adrenal supress suppression and it can cause like imos suppression as well so you have to be careful like you know you're going to be at risk of infections and we always tell our patients that you might be more likely to get more infections avoid people that they have contagious diseases like for example if someone has chicken pox even if you're not on torpo you know you shouldn't be in contact with them all the time so but when you are taking tarap you do not want to spend a lot of time with them I always tell my patients this is something not on the label but I always tell my patients if you're going somewhere that's really crowded or if you feel like like people are going to have flu or like you know covid like symptoms wear your mask and try to protect yourself use a hand sanitizer all the time you're going to be at risk of like infections you have to be more careful and um because it's going to suppress your immune system and then if you have any symptoms of infection they when you are taking tpeo always always talk to your physician right away uh because you know sometimes if you are having like really high fever or like you're vomiting you have like severe nausea we want to know as soon as possible so we can make adjustment to your prescription and other side effects that we talked about it acne muscle spasms high blood pressure you can have some swelling on your legs you can have some swelling on your face as well your your weight might go up you can have some indigestion but all of the symptoms so far in my experience it's been like mild to moderate and you should be able to tolerate the side effects um as we said based on the clinical trial as well um 87% of patients they completed the treatment for two years so um it sounds like the side effects were like they were able to tolerate that as well if you have severe um symptoms like any of these symptoms are really severe for you call your doctor right away okay um because we want to know about this this is not something that we are expecting and then tarpo can help you to control your IG neop as we said that this is the first and only FDA approved medication that is that is proven to help your IGN prop and it's proven to um help to save your kidney function it's only ifda approved treatment designed to Target a source yeah like it's going to go to your gut it's going to decrease the production of ig1 so from the pathophysiology standpoint it makes sense and it's one of the medications that helps you to stop the process of IG nephropathy before it gets to your kidneys so it makes sense uh from the mechanism of action that should help with patients uh with I nephop and it's we have the proven results over two years and we have um other patients who took this medication for 9 months and they were followed up for total of two months it showed that it can preserve your kidney function and it can reduce the amount of protein in your urine which are two markers that we check when we are following up on our patients with igf property so you usually take the medication for nine months and it's four capsules once a day and you want to take it before breakfast first thing in the morning when you wake up and we talked about tarpo touch points that is going to offer many programs and it's going to help you to afford the medication so now we have an Katherine here she is a patient who took tarpa and she is willing to share her experience with us thank you so much for being here Katherine thank you so much um can you hear me okay good yes um so my name is Katherine I'm 37 years old uh I'm a deep space artist originally from the UK spent many years in Australia and now I'm living in Alabama um I was diagnosed with a theopathy when I was 25 and I actually discovered it kind of by accident because I was actually going for a Visa medical and they noticed my elevated protein and blood in my urine so I went and got a kidney biopsy and that was where I was officially diagnosed um but yeah I I remember being told I'd be on medication for the rest of my life and I felt kind of overwhelmed uh because I guess I'd never been prescribed a lifelong medication before and uh I was always been quite healthy up until this point so anyway and also finding out that even with that there could be a possibility of needing dialysis or maybe a transplant later on and that is honestly terrifying so my nephologist at the time started me on ACE inhibitors um and I was taking them for quite a while but we weren't really seeing much of an improvement I we maxed out the dose um I changed my diet I stopped eating quite a lot of stuff that I really liked um but even even with all of that I still had quite high protein and blood in my urine it always kind of stayed the same um but anyway so to years ago I I moved to the US and I needed a neologist obviously and honestly I feel really lucky because I found a a very highly regarded doctor and he was telling me about this grain breaking research paper on a new medication that had recently come out and that the medication was designed to treat the gut area of the body that was thought to be the source of Igan which is really funny because until that point I had like no idea that where Igan proteins were made and for 10 years I had no idea that I was the one I was the one making them so anyway that was when he was told me about tpeo um now I'm the kind of person that likes to be well read on these things so I went and looked up all the studies and reviewed them highly recommend everyone does it and um I thought it were really interesting and in particular I was very impressed with the long-term data um yeah I I guess this is the first time I've seen a medication that was specific to my condition and especially one that could potentially help longterm um so anyway started uh toeo 6 months ago obviously it is a 9mon course so I'm a little past halfway um I've already seen a a good uh reduction of protein and blood in my urine and my kidney function has remained really consistent so far which is awesome um and my doctor's very happy with my results and honestly I am stoked uh with the improvements we're not even finished yet so um I'm excited to see in the next set of r results um but yeah anyway this is the first time in a decade that I've I've taking medication that I've seen a a result like this which is very exciting um so as far as side effects uh I had like uh light cramps in my legs in the morning but that only really lasted for the first month or so um it kind of felt like uh I guess an adjustment phase um I don't know if everyone will get that um or be the same but it certainly was for me um lately I've noticed that my face is a little rounder but I still think I look fine um but you know your own face but fortunately it's just a temporary thing and I you know expect it to go back to how it was once I finished my course um yeah in a few months I'm going to be finishing my treatment and I'm really excited for the next stage in my life um yeah the chance to create a future that I've always wanted um and it's a possibility that isn't terrifying and I honestly couldn't ask for more um I'm yeah I'm really grateful get to share my experience with tarpo with you guys um obviously just remember that everyone is different have different experiences and um you've got to do the right thing for you but honestly I'm very happy with my results so far and I really hope everyone gets to experience that too that's that's amazing thanks so much for sharing your experience I think it's really important to like share these kind of experiences because IG is such an uncommon disease and have this kind of community to share experience it means a lot and I think it helps patients and you can choose you know like which one is going to suit you the best and what option is going to be the best for you because as you said every patient is just so different and we have a lot of options now and um it's important to know about the options so thank you so much um we have a couple of questions should I take the questions or we do have a couple of questions Dr nusi um patient wants to know um can you tell the difference or can you explain the difference between tarpo and the typical ceroid treatment yes of course um so you know a typical cortico steroid medication is that when you take the medication it's going to get absorbed as soon as you take the medication so you're going to have the systemic effects of corticosteroids with these medications you're going to have you're going to gain a lot of weight the round face that Katherine was mentioning with systemic cortical steroid is going to be more obvious you're going to gain some weight on your shoulders you're going to have increasing your blood pressure you're going to be at risk of infections much higher than tpeo because this medication is going to get absorbed as soon as you take the medication so it's not a targeted release medication and it gets absorbed systematically so you're going to have all of these side effects times like you know much much higher than like tpeo so tarpo is a targeted release so when you take the medication it doesn't get released until it gets to that point of your gut that is making a lot of ig1 so basically we are hoping that this medication is going to have less side effects and you compare that to systemic cortico steroids and it's not just hoping based on the clinical trial data they had less side effects for example if you're taking systemic corticosteroids you're going to have increased A1C like your blood sugar is going to get affected right away with tarpo we did not have that much of a change in A1C in our patients who took tarpo in the clinical trial increase in the blood pressure with systemic corticoid is usually like really obvious and you have to make a lot of adjustments to the medications uh we had a clinical trial with cortical steroids on IGN property that is called testing trial and the side effects that was reported on the testing trial was were so high that they had to stop the clinical trial because of the side effects so there were like severe infections that they couldn't complete the study because of that so it shows that with systemic cortico stories you're just going to have higher chances of uh having these side effects and you have to be much more careful so one good thing about tarpo is that we feel like it has less side effects and it's going to work on your kidneys so this is going to be a better option for the patients um do you recommend any specific diet to your patients when they're on tarpo so as I said I always I'm So Pro I'm in California so I'm So Pro like lifestyle modifications like eating healthy less salt these are the things that actually helps your blood pressure and it helps with the swelling on your legs so I always tell them my patients this is the perfect time to make a lot of good adjustments to your lifestyle like try to exercise as much as possible try to eat healthy there is no specific um um restrictions or other than the grapefruit juice that I mentioned because it has an interaction with the medication tpeo otherwise we just want you to eat healthy and we want you to minimize the amount of salt that you are taking the less salt it's going to affect like your blood pressure in a better better way and it's going to help your kidney function as well um but not just because of torpo you know just because of your kidney disease and tarpo the only restriction is grape food and do you recommend tarpo with an sg2 uh inhibitor as well yes of course so sglt2 Inhibitors is one of the background therapies now the same as the blood pressure medication that been mentioned Leno or lartan we do estal T2 inhibitors for all of our patients so most of my patients uh they are on sglt Inhibitors they are on a blood pressure medications that they like usually it's lioil or lortan here in the United States and then we do Fara or jardians both of them are sglt2 Inhibitors and you can pick one of them and take them once a day great and how long will we have a patient wants to know how long will I need to take tarpo So based on the clinical trial patients they took nine months of tpeo and all of the results that we were talking about it was about taking tarpo for 9 months and then we tapered down tarpo for two weeks so they were taking 16 milligrams of tar for 9 months went down to 8 Mig of tarpo for 2 weeks and then they were done in real life sometimes I have patients that they are responding to Taro really well for example we start with two grams of protein at nine months we are at 900 milligrams of protein or one gram of protein like it's it's going down and it's amazing but it's not there you know I just want to see better results so sometimes I continue it for a longer time so I have patients that they've taken it for more more than a year as well and once I see that their protein is close to zero at that time I'm going to stop it in the clinical trial you are following a protocol and you have to follow the protocol because you want to see like you know you are comparing patients that are taking tarpo and patients who are not taking that so you have to follow the protocol in real life patients are different sometimes you are responding to the medication a bit slower than other people and you might need a longer time and I think that's fine as long as you're responding to the medication I would continue that until I see the perfect results and then after at that time I'm going to tape her down and we have a patient who has been diagnosed with aggressive again and his nephologist is very hesitant about using tarpo um what kind of advice can you give our patients to let them know the type of conversations they need to have with their doctors about Taro oh I saw that comment and you know what um I know that this is a complicated situation because we always um kind of believe in our physicians um and um I can tell you that just to get a second opinion you know um you do not have to fight with the physician because um that's his opinion and I think that just get the second opinion this is an uncommon disease and it's not like diabetes or as I said high blood pressure that you say that oh okay this is something that my physician is seeing this IGN in property every day in the office you know this is an uncommon disease and you need to get a second opinion especially if you're not being seen at the glomar disease clinic I would make an appointment at a GN Clinic which is like a sub specialty clinic for IG property to get a second opinion because there are a lot of options we talked about tarpo today but for IG property you have a lot of options and cortico steroid is such an old medication now um so I really want you to talk to other doctors and see what they think um and you know what maybe based on your blood works and maybe based on your situation and um your health history and everything maybe cortico Street in some specific situation could be the choice but you just need to get more opinions about this just to make sure because we have a lot of more options with less side effects and I want to make sure that you are receiving the right treatment uh do you have any idea when tarpo may be available in Canada I don't know I think Dana should know about that I know that um it's getting more available like worldwide I don't know the timeline right and we have a patient who's currently taking tarpo two months into their treatment and they're not seeing a change in their protein do you think that TPO is working for them or yeah so that's the thing like on the clinical trial as I mentioned like we have to be really patient with tarpo if you are taking tarpo for two months it's it's too early to check your um your blood works and urine test we usually check it at four months and at that time we should see some improvement and after four months when we check it like at six month or seven months usually that's the point that I see a really good reduction in the amount of protein and I see like better results at that time so this this medication doesn't work overnight um you have to take it for a while and the reason is that this medication is working on decreasing the production of ig1 um so it takes time because you already have some ig1 on your body so you have to clear those and then we're going to decrease the production of the ig1 for the future right so it takes time to work and you have to be patient with tarpo um check your labs in two months and uh you should see some improvement hopefully we pray for you thank you Dr nzi for joining us and Katherine thank you as well and thanks to khas for sponsoring this webinar and for those of you who are still navigating your Igan Journey please visit www.i.org for some tips and on how to navigate that journey and everybody have a good night thank Youk you so much take care thanks for having me