Final NephCure Kidney Network Data Snapshot August 2, 2018 by Kylie Karley Since 2014, NephCure Kidney International (informally called NephCure) enthusiastically collected your survey responses in a systematic and safe way to help researchers and doctors better understand Nephrotic Syndrome, FSGS, and related diseases. Over the past few years, we have released annual reports about the trends of diagnosis patterns, treatment options, and your opinions about research and clinical trials. In addition, through our participation in larger networks of data (thanks to our involvement in PCORnet), we learned about electronic health record access for Nephrotic Syndrome patients, sleep habits, and potential mobile app use. We are pleased to release the final Annual Report, which includes data collected over a 13-month period between 2017 and 2018. While the NephCure Kidney Network Patient Registry is no longer open to registrants, the data will be available for researchers to study for the next 3 years (should they be approved by a patient-centric committee for data access). If you have any questions about the NKN Patient Registry, please email registry@nephcure.org.
International Podocyte Conference in Montreal August 1, 2018 by Rebecca Cook NephCure hosted the 12th International Podocyte Conference in Montreal from May 31 to June 2, 2018. With over 309 attendees, including researchers, study coordinators, pharmaceutical representatives, physicians, and trainees, the conference was a huge success and one of the largest in the conference’s history. New this year was a deliberate focus on “bench to bedside” research, concentrating on applying research uncovered in the lab towards improving treatments for patients. The crowd of 309+ participants! The biannual conference – most recently also held in Germany, Miami, and Israel – focuses on a specific cell in the kidney called a podocyte. These cells have “foot processes” that look like tentacles, which wrap around capillaries to help the kidney filter blood. The tentacles and general shape of the podocyte resemble an octopus, which is also the conference’s mascot! Podocytes wrapping around a capillary. The banner for this year’s Podocyte Conference. NephCure CEO Josh Tarnoff introduced the conference on a positive note: While 20 years ago little was known about the function of the podocyte and how it related to chronic kidney disease, today, in part because of the connections made at past Podocyte Conferences, we have huge developments in treatments. There are now many pharmaceutical companies interested in Nephrotic Syndrome, and they are seeking to develop treatments as a result of discussions at these biannual conferences. NephCure CEO Josh Tarnoff introducing the conference. As in past years, NephCure hosted a patient education workshop for families in conjunction with the conference. The one-day program featured talks on treatment options, updates in research, and dietary recommendations, and brought together 25 patient families and more than 75 people impacted by Nephrotic Syndrome. Dr. Tomoko Takano of McGill University speaking on Cytoskeleton Regulators in Health and Disease. In Nephrotic Syndrome, the kidney’s podocytes are not functioning properly. This loss of function is one of reasons that protein leaks into the urine. It was once thought that when podocytes stop functioning, they could not be restored. However, Podocyte Conference Steering Committee Member Stuart Shankland discussed new research in regenerating these cells in animal models with the intention of developing treatments for human patients during his presentation, “Developmental origin of podocyte in injury.” Shankland has been researching how parietal epithelial cells may be able to repair or regenerate injured podocytes. Both parietal epithelial cells (PECs) and podocytes are a part of the Bowman’s capsule, a membrane that surrounds the glomerulus of each nephron in a kidney. The Bowman’s capsule, featuring the tubular pole (TP), the podocytes (Pod), the parietal epithelial cells (PEC), the vascular pole (VP), and the Bowman’s space (BS). Ryuichi Nishinakamura of Japan’s Kumamoto University spoke on “Dissecting podocyte development and disease in kidney organoids.” An organoid is an group of artificially-grown cells that resemble an organ. Researchers use organoids to learn more about how organs and organ cells like podocytes function. Anna Greka, a researcher and physician at Harvard’s Broad Institute, delivered a talk in the Advances in Therapeutics for Glomerular Nephropathies portion of the conference. Her lecture, “Mechanism-based precision therapies for progressive kidney disease,” in addition to many other presentations, emphasized the importance of personalized treatments for patients. Dr. Peter Mathieson of the University of Edinburgh at the question mic. The feedback we received on the conference was overwhelmingly positive, and many participants said it will impact their practice and research. One participant said, “More collaboration with basic scientists will lead to find[ing a] cure for podocytopathy,” meaning all diseases that affect podocytes. Other participants commented that the conference helped them in “Recognizing novel pathways of disease that will provide targets for ongoing and upcoming clinical trials.” The researchers were grateful for a space to share their developments and forward the field. Another idea common to the evaluations was the gift of hope for the future many of these talks provided. One responder explained that the conference “raises optimism for future care.” The multi-faceted concept of research, from basic science to clinical trials to cures, is ingrained in NephCure’s DNA. We are invigorated by the discussions at this year’s Podocyte Conference and look forward to hosting the next conference in Manchester, England in 2020. (Photography by Photos FERA – www.photosfera.ca)
Why I Do What I Do: Spotlight on Kara Jones, Patient Mom August 1, 2018 by Lauren Eva On October 6th, the first annual Silicon Valley Pig Jig will be rocking the day away at the San Jose Giants Muni Stadium in California. The Silicon Valley Pig Jig (or SVPJ) is an all-day live music festival, with great bands, delicious food, an amateur barbecue competition, beer garden, craft vendors, kids zone and games, all set in a fun tailgate environment. Number 1 hit country music singer Dylan Scott, with singles “My Girl” and “Hooked,” is headlining. Longtime NephCure supporter and patient mom, Kara Jones, is leading the efforts behind Silicon Valley Pig Jig, and she recently told us more about what to expect at the upcoming inaugural event. Cheech, nearly 5 years old, on his first day of T-ball. NephCure Kidney International: How did you first find out that your son, Christian (known affectionately as Cheech), was sick? Kara Jones: It started when he was 2 years old. We were in the process of potty-training him, so I didn’t realize how infrequently he was going to the bathroom. He had what I now realize are all the classic symptoms—puffy eyes, edema, and so on. One night he was running around in his diaper, and his belly was extremely distended. My mother-in-law felt his stomach and said, “This is not good.” We took him to the doctor right away. We got lucky, because our pediatrician had done rotations in the nephrology department and was immediately familiar with Nephrotic Syndrome. We were quickly introduced to our doctors at Lucille Packard, and Christian has had those same doctors now for 16 years. Cheech is steroid-dependent and a frequent relapser. He’s been on Prograf, Cytoxan, Cellcept, and Cyclosporine. He’s done three rounds of Rituximab. Now he’s on Myfortic, which is supposed to be less harsh on your GI system. I’ve lost track of how many relapses he’s had and how many times he’s had to go to the hospital because of them. His immune system is a wreck. I often wonder if he was born with a weak immune system, or if all the years of the immunosuppressants have wreaked havoc on him. Kara with Cheech today. He graduated from high school in May. One of his good friends has family in France and invited him to go over there for a month this summer—his first trip abroad! As fate would have it, three days before he left, he started to relapse. Prior to that, he had not relapsed for close to a year. For the first time ever, he is managing a relapse on his own. He just texted me to say that he’s finally trace as of this morning. NephCure: Throughout so much of Cheech’s journey, I know you’ve been deeply involved with NephCure. How did you first get involved? Kara: It was at a pivotal point for me. Cheech was in the fourth grade, and he had just had a bad relapse and had been in the hospital. I knew he wasn’t feeling well, but he wasn’t throwing up and didn’t have a fever. I’ve always tried to treat him just like any of our other kids, so I said, “Look, you’ve got to go to school.” I dropped him off, and he was so bloated and overweight and chubby-looking. His clothes didn’t fit right. He walked into that classroom all by himself with his head hanging so low. It was such a painful time, to watch him go through that. It was devastating. I drove home and thought, “I am so tired of this.” I was tired of taking him to doctors’ appointments and giving him medicine that we didn’t even know if it was working, and all the damage that I knew it was causing him. I said to myself, “I’m done just watching this happen. I have to be more involved.” Kara (center) and supporters at one of their Bay Area NephCure Walks. I went home and called someone from NephCure and said that I wanted to hold a walk. I got lucky, because a lot of my friends had watched Cheech suffer, and saw the pain I was going through too, so they jumped in to help. We had the first walk in 2010, and we did it every year since then. NephCure: Where did you get the idea for the Silicon Valley Pig Jig? Kara: That came from the Tampa Pig Jig. I was so impressed with what a bunch of young men could do that I figured a bunch of moms could do it, too! The walk was great and I enjoyed the process, but I got to the point where I was yearning for a bit more. We wanted to do something that we thought would get our early 20s and teenage kids more excited about going and being involved. NephCure: Can you describe the event—what will the sights and sounds be? Kara: It’s going to start early in the morning. There will be people laughing and having a great time, barbecue teams setting up, people having fun with their tailgates—we’re going to have an award for the best tailgate environment, so people will be decking those out! Everyone will be having a lot of fun, there’ll be great music playing, and there’ll be lots of good smells, smoky meats cooking in the air. All of this will be taking place in one of my favorite places in San Jose—the San Jose Giants Muni Stadium! My husband and I started going to games there before we were even married, and I feel like our kids spent their summers watching baseball games there. So I picture this all happening at a spot that’s personal to me, surrounded by 20 to 25 of my closest friends who have put their heart and soul into this great event. We have amazing support from the Silicon Valley and San Jose community and so many great businesses. Everyone is making us proud to follow in the footsteps of Tampa Pig Jig! Dylan Scott, hit country music singer with singles “My Girl” and “Hooked,” is headlining. NephCure: Can you describe the barbecue competition? Kara: Businesses or individual teams can join the barbecue competition. We’ll have about twenty teams, and they’ll be judged on tri-tip, ribs, wild card, and best overall. We’ll also have an award for best tailgate setup. We’re lucky to have the California Barbecue Association coming out to help us with the judging. Even though this is a non-sanctioned event, they’re going to judge the entries as if it were, so the barbecue teams will be able to get professional feedback on the foods that they turn in. San Jose Giants Muni Stadium, home to the inaugural Silicon Valley Pig Jig to be held Oct. 6. NephCure: Is there going to be a kids’ area? Kara: Yes, we’ll have a whole kids’ zone with inflatables, face paint, crazy hair, games and something we always did at the walks—cards for kids in the hospital. We’re carrying on that tradition, so there’ll be a card table set up with all the supplies if you want to make a card for children who are in the hospital. Kara and family have been dedicated members of the NephCure community for many years. NephCure: Will there be something for patient families at SVPJ? Kara: Yes! Our goal is to have at least 20 patient families at the Pig Jig. They’ll be treated as VIPs and will receive an All-Access Pass. We would love for patient families from near and far to come and meet and mingle with others. We’ll have a gathering for everyone at 2 pm in the sponsor lounge. I’m hopeful that some of the health professionals from Lucille Packard Children’s Hospital will be there, too. NephCure: I’m so impressed with how many sponsors you’ve gathered. I know many people are daunted by the idea of asking friends and business owners for money. How did you learn to do that? Kara: You have to find the right people to help you. I have been a stay-at-home mom or teacher for most of my life, so I have not been hanging out with CEOs or decision-makers of companies. But I am lucky that my husband does and has friends who do as well. If you personally don’t have those connections, find a friend or someone you trust who is well-connected and invite them to be on your sponsorship committee. Ask them to help you by making those introductions. Our friends have really gone to bat for us. One gentleman who’s done a lot recently said to me, “Kara, you have the passion, and you can tell the story, and that’s all that people really need to hear. How can they not jump on board?” Our friends have made so many introductions and gotten us in front of a lot of business owners. They’ve really taken on this cause with the same passion that a parent of a child who was sick would. There’s a group of 4-6 of us who have been meeting every other week since February. We go over our list, manage our contacts, figure out if there’s any overlap with businesses or who knows who. Even though we’re all volunteers, it’s not really a “volunteer gig”—we’re all taking it very seriously! I hate that I’m in this situation, but who better than me, as a parent, to make the ask. Because it is so personal—I have to find a cure. Cheech at our 2015 Countdown to a Cure Gala. He and his family were our featured Fund a Cure Champions that year. NephCure: Is there anything else we should share? Kara: Yes! Partnering with KRTY and the San Jose Giants has been vital for us. Being able to say to potential sponsors that we’ve got KRTY behind us and we’re doing this at the San Jose Giants Muni Stadium has given us immediate credibility. KRTY have been so supportive in getting good music for this event. I cannot emphasize the importance of those two parts of this “puzzle” enough. The volunteers inspire me every single day. On Sunday, the two women who are spearheading volunteers were working all day on this together. On Saturday, the two women who are doing merchandise were putting their spreadsheets together and working. And they all work full-time! I am just amazed at how much of their own personal time they’re giving up. My friend Teri Molinaro is a full-time lawyer with three kids, but she is dedicating above and beyond. It’s amazing, what they’re all doing and how much they’ve backed up Pig Jig and NephCure. Throughout all of this, my many years with NephCure and spreading awareness and fundraising, my main goal is that one day, everyone knows about NephCure and knows about Nephrotic Syndrome. Learn more about the Silicon Valley Pig Jig at their website: siliconvalleypigjig.com If you’re interested in learning about sponsorship opportunities, please contact Kara: siliconvalleypigjig@gmail.com If you’re interested in attending the Silicon Valley Pig Jig as a patient family, please contact Allison to register: akostiuk@nephcure.org