Category: conference

NextGen Therapies in SJIA, Still’s & MAS

Posted on by Grayson

The Systemic JIA Foundation recently held its NextGen Conference. The conference, which had over 350 participants, featured three days of presentations and discussions around a few core ideas:

  • There are cases of SJIA without arthritis and arthritis should not be required for diagnosis of SJIA.
  • Different phenotypes of refractory SJIA (with arthritis or lung/liver involvement).
  • The use of IL18 as a biomarker for disease & trials.
  • Planning for clinical trials for both refractory SJIA and MAS.

One of the sessions covered Bone Marrow Transplantation in refractory SJIA. The last session focused on SJIA-LD, with a talk about use of lung ultrasound to diagnose/track lung disease and a new hypothesis for SJIA-LD – the Cytokine Plasticity Hypothesis.

The participation of the FDA in many of the sessions, with comments and feedback, added a whole other dimension – enabling us as a community to go from discussion to planning of clinical trials.

Weren’t able to attend?

Rewatch or view all of the ground-breaking sessions from NextGen 2022 through the virtual conference platform. All of the content is now available to view until the end of August. Anyone can register free (though the conference is over) to gain free access to conference videos for the next 6 months – including patients & parents!

Register now

mas session

Macrophage Activation Syndrome Session #ACR17

Posted on by Grayson
This morning, I was lucky enough to attend a session all about one of my biggest fears – Macrophage Activation Syndrome (MAS).

 

The first thing I found interesting was that the physician who presented this session – Rayfel Schneider from the University of Toronto – believes that SJIA and AOSD are essentially a spectrum. A person may have juvenile or adult onset and present essentially the same. Newer research over the last few years from leading pediatric rheumatologists – like Dr. Alexei Grom and others – have proven through various pieces of research that SJIA more closely resembles periodic fever syndromes and is autoinflammatory rather than autoimmune.

 

What is MAS?

For those of you who aren’t familiar, MAS is one of the leading causes of death for people with Systemic Juvenile Idiopathic Arthritis (SJIA) or Adult Onset Still’s Disease (AOSD). This can also be a worry for people with SLE lupus or Kawasaki Disease. Otherwise, it’s rare to see MAS in conditions like other JIA subtypes, IBD, etc.

 

Macrophage Activation Syndrome isn’t well understood. Essentially, though, what MAS is a product of is a “highly stimulated but ineffective immune response.” This can be due to an infection or the underlying disease (SJIA, for example) itself.

 

Symptoms of MAS include increased fevers, higher ferritin levels, liver issues, cytopenia (low white and/or red blood cells), coagulopathy (difficulty to form blood clots), and hepatosplenomegaly (enlargement of both liver and spleen). Additionally, lymph nodes may swell and a patient’s mental state may change. They may also experience issues in a wide range of bodily systems, including neurological (from seizures to a coma), cardiac, pulmonary, and renal. Schneider explained MAS as a hyperinflammation and cytokine storm, leading to organ damage, multisystem failure, and, potentially, death. In fact, mortality in kids sits at 8-22%. For adults, that’s about 9%.

 

MAS is related to another condition – Hemophagocytic Lymphohistiocytosis (HLH). I won’t talk much about that for now, but MAS is thought to be one form of secondary HLH brought on as a part of another condition (again, SJIA). There are many types of secondary conditions like SJIA that could cause MAS, such as Epstein-Barr Virus and even some kinds of cancers. The thing I found most interesting about this was that one type of cancer Schneider pointed out was leukemia. In my personal journey, I was misdiagnosed with leukemia and given six weeks to live. It always seemed to me that my physicians had been grasping at straws to find a diagnosis, but this link makes much more sense now – especially when the majority of MAS cases present at SJIA onset. Research shows that 7-17% of SJIA patients will experience MAS.

 

Recognizing and treating MAS

From a lab standpoint, the following can be considered as potential situations for MAS development:
Decreasing
  • Platelets
  • WBC
  • Hemoglobin
  • ESR
  • Fibrinogen
  • Albumin
  • Sodium

 

Increasing
  • Ferritin
  • CRP
  • Transaminases
  • LDH
  • Triglycerides
  • D-dimers, INR, PTT
  • Bilirubin

 

Only recently has there been an effort to revamp the guidelines to diagnose MAS in SJIA patients. Unfortunately, while helpful, these guidelines may not be sensitive enough. Schneider says we should also look to the AOSD diagnostic criteria to make sure we’re paying closer attention to some of these lab values and symptoms.

 

Schneider played with some lab values, asking providers to think about how we can diagnose MAS earlier and get a head start on treating it. Possible treatments are an IL-1 inhibitor – Anakinra/Kineret has been shown to help a lot in higher doses during MAS episodes. Steroids may need to be given in high doses initially to help get the inflammation under control. A calcineurin inhibitor, such as cyclosporine, is a great tool to add as well.

 

It’s important to note that some medications – notably Ilaris and Actemra – can alter how MAS presents due to the effects of the drugs on the body. This must be kept in mind by the treating physicians in order to recognize the signs and treat MAS effectively.

 

New research is showing that interferon gamma (IFNγ) plays a pivotal role in MAS, most specifically CXCL9. Additionally, this can be linked to research that shows higher levels of IL-18 in the bloodstream of SJIA patients dealing with/who have gone through MAS. Understanding the links between CXCL9 and IL-18 – and how we can lower IL-18 – will be pivotal in treating MAS in the future.

 

Takeaways

The biggest takeaway for me is that MAS doesn’t show up as quickly as I’ve always personally feared. Watching friends and other SJIA patients struggle with MAS is scary, but knowing that we may be able to monitor and predict pre-MAS status is comforting. Furthermore, knowing that my rheumatologist will be open to this information and act upon it if/as needed really helps me feel less anxious. That’s part of why it’s important to snag a HCP that listens as well as mine does.

 

My secondary takeaway is to start monitoring my own labs more. When I get home, I’ll spend a while and plot my relevant labs into Excel and graph them. While it will help me keep tabs on things – especially concern over heading into MAS territory – maybe it’ll help me find more data around whatever weird neuro-ish thing I’ve been experiencing as well.

 

The world of rheumatic disease is one that’s ever-changing and improving. I think it will become incredibly important to focus on changing alongside it instead of staying stuck in the old ways like many providers and patients often do.

 

 

Opening Lecture #ACR17

Posted on by Grayson
The ACR/AHRP Annual Meeting kicked off last night with an opening lecture that had me in tears a few times.

 

Opening Remarks

Dr. Sharad Lakhanpal, current head of the ACR, started the opening lecture off by sharing more about what’s gone on in the last year for the ACR. One of the things that many may not know is that the ACR’s annual meeting is the largest of its kind in the world, with over a hundred countries in attendance. ACR has started a fellowship program as well, allowing newer physicians abroad the opportunity to come to ACR in addition to spending time in American clinics seeing patients. This exchange goes both ways, too, with American physicians heading to other countries. This exchange allows providers to develop a variety of problem-solving skills, learn about cultural importance, and more.

 

Lakhanpal then went on to talk about ACR/AHRP’s place in fighting against the repeal of the Affordable Care Act. Cue teary-eyed me in the audience. Dr. L echoed things I’ve always felt around the ACA – that we know it isn’t perfect and aspects do need to be fixed, but that scrapping it doesn’t fix anything. They truly have been fighting for their patients, reaching out just as we have. The fact that they have been using the same methods as patients to fight the same bills is comforting. We haven’t been the only ones using Resistbot, social media, and more to fight for our own rights.

 

One of their biggest focuses for the next five years is on technology. With the amount of technology in the world growing exponentially each day, it’s changed how we learn, teach, and grow as humans. Thankfully, the ACR is working to utilize technology for more. My hope is that patients will have access to some of these tools as well, making our learning process – and participation in research – even better.

 

Dr. L touched on the importance of viewing issues through a global lens as well. We’re all in this together. The more we fight for better access to care and lower costs in one place, the more we learn about how to fight it in other areas. As a “global community of learners” and rheumatology nerds, what we do matters. I say ‘we’ because he also highlighted the importance of utilizing patients to improve public awareness around RMDs.

 

Awards were handed out to a variety of impressive providers for their efforts on research, teaching, patient care, and more.

 

Emerging and Re-Emerging Infectious Diseases: From AIDS to Zika

Dr. Anthony Fauci is the director of the National Institute of Allergy and Infectious Diseases (NIAID), a role he’s been in since 1984. This man has testified more times before Congress than anyone else! He moved from doing more rheumatology-related work to focusing on IDs when the first cases of HIV/AIDS came about, back when they didn’t know what this even would be. Fauci could see that this would be something far bigger than anyone else could predict.

 

As a sex educator, Fauci’s talk hit me hard – in a good way. I was born at a time when we knew that HIV/AIDS was an infectious disease that could be transmitted a number of ways. Having a disease that wasn’t diagnosed right away meant that I faced a lot of stigma. Rumors went around that maybe I had something like HIV/AIDS. Even before I realized I was pansexual and genderfluid, I felt so much like a part of the LGBT+ community. It’s definitely not the same and I’m certainly not equating SJIA to HIV/AIDS, but there was a kinship there. It was my first exposure, too, to just how homomisic people are.

 

I could never understand how people could be happy about others dying due to a horrible undertreated disease – especially when it took quite a long time for many to realize this was a public health issue. I just remember so much about stigma and discrimination.

 

Dr. Fauci echoed those sentiments while discussing how our reactions to infectious diseases have changed massively since the early 1980s. He related some amazing stories around his interactions with past presidents. Perhaps the most surprising, to me, was George H. W. Bush. When he was Vice President, Bush seemed to share Fauci’s concerns around HIV/AIDS much more than Ronald Reagan had. One of the most impactful points in Fauci’s talk was him sharing how Bush asked to tour the NIAID’s facilities as he was working on running for President. He came with members of his staff (including George W. Bush!) and sat in a support group for patients Fauci helped facilitate. Since this was early on and before newer medications, every single patient in that group was dead within two years.

 

Cue me crying for a second time.

 

By the time Clinton came into office, he asked Fauci to start work on a vaccine for HIV. This helped push vaccine-related research around infectious diseases further. The center built for this purpose now works on vaccine and treatment development for many conditions, from HIV/AIDS to Zika.

 

George W. Bush helped push forward the notion that the US could do more to fight infectious diseases – especially HIV/AIDS – in other countries. Fauci traveled to several countries in Africa and observed the realities of living with HIV/AIDS there. Patients were eager for medications and focused on taking them daily (when possible), fighting some extremely racist notions around the ability of people of color to ‘adhere’ to medical treatments. The only real thing that holds many back is a lack of access to medications, including costs – and that’s a universal problem.

 

Bush and Fauci worked together, then, to develop PEPFAR – the President’s Emergency Plan for AIDS Relief. To date, over 11 MILLION people have received antiretroviral medications through this program. If I recall correctly, Fauci said that over 2 million children were protected from transmission during pregnancy.

 

There were so many lessons to take from Dr. Fauci’s talk and experience. The thing that hits me the most, though, is how far we’ve come – and how people work towards amazing feats while receiving very little press for it. Fauci touched upon the improvements in vaccine development. Zika vaccines in the works have been developed in a matter of a few months versus years. There’s a lot of work to be done on a variety of infectious diseases, but the ways technology has allowed us to improve our processes is fascinating.

 

With regards to rheumatology, the biggest takeaway for me is that technology and persistence continue to push us forward to a world with better treatments. The number of patients who have helped with research and clinical trials is not lost on me, either. It’s only through the sacrifices and work of our fellow patients, in addition to work from scientists and providers, that we have the treatments we rely on every single day.

 

I would be remiss to not mention the many animals who have been subject to testing as well. As I reflect on what fellow patients have given to the cause, I can’t even begin to quantify the number of animals who have been harmed through our scientific processes. Like, I’m sitting here in a comfortable bed and bawling because the weight of that harm is just so much. I quite literally owe my life to lab rats and mice and guinea pigs.

 

And people wonder why I have piggies.

 

four photos arranged in a square; top left is an illustration of Kirsten feeding piggies; top right is a photo of Oreo (black and white and grey piggie) with hay all over his head looking happily up at the camera; bottom left is a photo of Jaq (brown and white piggie) on the floor near a zebra striped blanket eating lettuce; bottom right is a picture of Gus (black piggie with white nose stripe) snuggling on my chest under a blue Tardis blanket

 

 

Off to Healthevoices 2017

Posted on by Grayson
I’m heading to Chicago for the weekend for HealtheVoices 2017!
Chicago is one of my favorite places, so I’m excited to spend some time there with some of my favorite health activists in the entire world. It will also be great to see many of my Joint Decisions friends for the first time since our October summit.
Since I’m at the end of my master’s capstone writing, I may not get a recap up as quickly as I did last year. Still, I’m excited to be able to share things I learn with everyone else via the interwebs.
PS: Janssen is paying my travel expenses for this conference. All thoughts and opinions expressed here or on social media are my own and do not reflect the thoughts or opinions of Janssen or Johnson & Johnson. 
 
And, yes, in case you were wondering, I will be binging on Do-Rite Donuts again.

 

Conference Catch-up

Posted on by Grayson
There is so much from the last few months to catch up on!
After visiting Toronto earlier this year, I made my way to Phoenix, Arizona, for the Juvenile Arthritis Conference (West).
Being able to be around some of my favorite advocates is something that I truly love about attending these conferences… but to present? That was a whole new level of awesome.
I was so blessed to be able to present with my pals Mariah Leach and Jeremy Forsyth as well. Their dedication to helping others, being informative, and being silly matched me so well. Jeremy, for example, thought we could loosen everyone up by screaming sex in ridiculous ways before our session started.
It worked and that laughter was so contagious.
We definitely tried to copy that at the east JA Conference in Philly in August, where Keegan Stephen was our male expert.
These two groups of college-aged peeps with various types of arthritis were so engaged, so interested, and so in tune with wanting to learn everything they could about what we were saying. So many thoughtful questions came out of both the west and the east conferences. Over at Chronic Sex, Mariah and I will be going through the question cards and providing some answers within the next few months.
All of this gives me so much hope for what Chronic Sex can and will be able to accomplish. I am SO excited.
In September, I traveled back to California to present Chronic Sex at Stanford Medicine X.
I got to spend time with some of my favorite people like Dawn Gibson and Heather Corini Aspell. I also got to room with my pal Danielle Edges who FINALLY got to meet my sister.
I’M NOT CRYING YOU’RE CRYING. Seriously, this picture alone was worth my travels. My sister and Danielle have become close friends and we’re almost like triplets with time in between our births. It’s just too awesome.
Dawn spent a few days near where my sister lives with me and got to spend time playing auntie. I spent a couple of extra days and then headed down to Los Angeles for the Women in Pain conference… BUT not before meeting one of my favorite people in the world, Kate McCombs!
We had dinner the night before the conference and then spent basically the whole next day together, from the conference to just hanging out.
There are so many people in my life that mean so much to me, but meeting Kate in person is something that I cannot even put into words. We are so similar and so in-tune with each other, bringing out the most compassionate parts of ourselves to deal not only with our own illnesses but helping others as well.
I stayed an extra night to spend time on the Queen Mary because I have a ghost hunting problem… though I was so tired, I didn’t try this time!
In October, I was lucky enough to attend the Joint Decisions Empowerment Summit in Philadelphia.
One of my favorite parts of the whole experience was spending time doing the Bioexperience tour at Janssen! That alone needs its own blog post because it was absolutely fascinating to see how much pharmaceutical companies care about us – how we’re going to administer treatments, costs, protective measures, and more.
Well, and then I got to spend time with some of my absolute favorite people in the world from Janssen, Tonic, and us Joint Decisions/Creaky Joints peeps!
I got a new nickname from Kenzie, too: whimsical unicorn warrior.
It’s my absolute favorite thing ever.
I already have written about my experience at the summit with the What I Be project, so make sure to check that out, too.
And then it was back to New York, this time for a project with Healthline. A pharma company wanted to hear more about the patient (and patient advocate/activist) experience. I was lucky to hang out with some amazing people like Emily Lemiska and MarlaJan Wexler:
I had been jokingly looking at Hamilton ticket prices because I am obsessed with the play as we all know.
Turns out that it was just slightly less than my stipend to go see it alone while I was in New York so guess what I did?
I FUCKING SAW HAMILTON ON BROADWAY BITCHES.
It was so amazing. I cannot even fully put the experience into words. It was well worth the post-show in-flight migraine.
I had a big week last week, conference-wise and seeing Hamilton and more!
When I got back, I was able to do some podcasting, work on homework, and, you know, just casually meet my favorite person in the world – Joe Biden.
This picture was a big fucking deal y’all. YES.
Later this week, I’m headed back to New York – this time for a family vacation with T’s mom and stepdad to visit his sister and her dude who live in Queens. I am so excited to go travel where I’m not necessarily tweeting and such but able to be silly.
More conferences are on the way, though thankfully not until 2017. I’m ready to expand the work that I do and help others as much as I can.
Disclaimer time: the Arthritis Foundation, Stanford Medicine X, and Women in Pain all paid for my travels to and/or from their conferences. Janssen Pharmaceuticals paid for my travels to the Joint Decisions Empowerment Summit and provided perks while traveling. Healthline paid for my travel as well as providing me a stipend for my time in New York. All opinions here, though, are mine and mine alone.

 

Off to Cali!

Posted on by Grayson
I am SO excited (and hella nervous) to head back to MedX this year.
See, I’m presenting on Chronic Sex for the Health Care Innovation day AKA talking to patients, healthcare providers, medical system reps, insurance reps, and venture capitalists… AKA I may vomit. This is a huge opportunity for me to get news about Chronic Sex out there in addition to potentially securing some funding.
After MedX, I get to visit my sister and the kiddos. I’m excited to spend time with them and play with them. I also get to visit some close friends that I haven’t met in person before, which is going to be amazing!
Then I’m off to Los Angeles for the For Grace: 9th Annual Women in Pain Conference on “Pain Takes a Vacation: Actions and Strategies that Get Us Away.”
You can live-stream both so make sue to check into it!
I’ll be away for the next two weeks ish. Take care of you!

 

Heading to Phoenix!

Posted on by Grayson
This time tomorrow, I’ll be in Phoenix, Arizona, frantically prepping to talk sex and arthritis with the young adults (18+) living with JA at the Juvenile Arthritis Conference West.
I’m super excited. Oddly, I feel like I should be much more nervous than I currently am? Like, running a session isn’t something I’ve ever done?
But I’ll have some amazing people up there with me including my pal Mariah.
I have to go finish resource sheets and frantically pack while singing Hamilton so I get cocky instead of too freaked out.

 

July is Juvenile Arthritis Awareness Month

Posted on by Grayson
July is one of my favorite months, and it’s not just because T’s birthday is the 15th.
It’s because it’s JA Awareness Month!
According to the Arthritis Foundation, there are six main types of Juvenile Idiopathic Arthritis (JIA): Oligoarthritis (4 or fewer joints affected), Polyarthritis (5 or more joint affected), Systemic, Enthesitis-Related, Juvenile Psoriatic Arthritis or Undifferentiated. There are also additional types of Juvenile Arthritis that also commonly occur in adults such as fibromyalgia, lupus, MCTD, and more.
We all know that illness can be hard to bear, but this is especially true in childhood. Often, we get upset that we cannot function as another child may, even resorting to thinking that everyone has this pain but we just super suck at handling it.
The elimination of self-esteem in illness is so real… which is definitely why, for adults, we have Chronic Sex now.
Speaking of: Are you heading to either of the Juvenile Arthritis Conferences put on by the Arthritis Foundation this year? I’ll be at both and would LOVE to see you! If you are in the young adult age range (18+ with arthritis), please note that I will be presenting along with some pals on relationships and sex in a closed-door session taking place during the final time slot on the Saturday of each conference. Come talk sex with us!

 

Recapping #Healthevoices16 in gifs

Posted on by Grayson
Chicago was SO MUCH FUN.
I cannot even begin to fully unpack everything – mentally, emotionally, and from my actual bags.
I got to hang out with some of my favorite people like Eduardo (RA Guy), Britt (Hurt Blogger), Leslie (Getting Closer to Myself), Kristin (Chronically Kristin), and Mariah (From This Point. Forward).
I also got to meet some of the wonderful people with Janssen that I will have the luck to work with more in the future as a part of Joint Decisions.
If all I did was meet them, the trip would have been worth it.
Thanks, Meme Crunch!
Our opening dinner was amazing. During which, I consumed two glasses of wine, sat next to Britt, and got to hear from the founder of Patients Like Me.
People came up and introduced themselves, excited about Chronic Sex or having been readers of mine for a long time.
HELLO TO YOU MY FRIENDS
On Saturday, I attended an amazing session with Trevis Gleason. Like my great grandmother Katie Mae, Trevis has MS and is a passionate advocate. He shared his journey, including my favorite part of the conference sessions themselves:
“Your illness did not make you better.”
It doesn’t stop there, but the following is a paraphrase: You had a Shawshank Redemption moment where you tore down bricks, made a tunnel, and “crawled through your own shit” to come out on the other side as a transformed person.
Thanks, Tumblr!
As if that alone wasn’t enough, he shared great ideas like working on publishing, inviting others through our apparel or accessories to discuss our illness, and then read to us from his book.
I’m in literary crushville.
The next session I hit up involved the legal aspects of blogging – defamation, trademarks, copyrights, protecting your information, etc – with Jimmy Nguyen.
With websites stealing information from us bloggers being a rampant issue lately, this was a much-needed session.
Many of us came together during the next session to discuss how to encourage a community feeling.
I hope that we will have visits, virtual convos, and tweet chats to help foster this.
.@Kirstie_Schultz shares her excitement at #HealtheVoices16 and how advocates can work together effectively! pic.twitter.com/Mf9NdxBau0

— HealtheVoices (@healthevoices) April 16, 2016

Ironically, I had to go have a rest during the Compassion Fatigue session I was hoping to attend with the amazing Rhonda Waters. The fact that she works often with the Joint Decisions group has me ecstatic, though.
Dinner Saturday night was tons of fun. I was with Britt and Leslie again, Molly (And Then You’re At Jax), some of the most amazing IBD/Crohn’s activists, and others.
You’ll have to forgive me on some of the deets because the amazing Deirdre from Tonic Communications snagged me a pitcher of red sangria… and I was the only one drinking it, save one cup.
Thanks, Giphy!
It was amazing.
KevinMD gave the Sunday keynote to a very… contentious room. He did not really alter his talk from speaking to HCPs to speaking to patient activists and advocates. This meant that he was asking us for help that we cannot give while downplaying the important things we do for free.
I was not thrilled with his talk, and he also didn’t stay very long for people to share more information with him afterward.
That was nothing compared to what happened next – all my new friends started to LEAVE!
Thanks, Mashable!
NOT OKAY, YOU GUYS.
Alas, I had to go home eventually as well.
Thanks, Tumblr!
I ended up getting motion sick and tossing my cilantro on the bus to the airport. The nice thing about that is the amazing patients I was with rubbed my back, got me bags, and took care of me until we hit the airport.
It was super sweet. Not unsurprisingly for those of you who know my background, my sister and my husband are the only ones who have ever really done those kinds of things for me.
That just secured it, though.
Thanks, Movie Pilot!
I CANNOT wait until next year.
No, seriously. Please let’s do things in the interim.
Growing up in an abusive home, a family of choice is something I’ve embraced wholeheartedly… I don’t communicate with my family of origin. Not everyone in my family of choice was at HealtheVoices16, but so many were: my sisters Britt, Kristin, and Leslie; my brother Eduardo; and our momma hens, Becky and Deirdre.
There is much I want to say, but right now I just cannot find the words.
Thanks, Goodreads!
PS: While Janssen paid for my travel to HealtheVoices16, all thoughts and opinions expressed here or on social media are my own… especially regarding how amazing people are.

 

I’m off to #Healthevoices!

Posted on by Grayson
This weekend, I get to attend the Healthevoices conference in Chicago! I’m actually leaving tonight after work because I love Chicago.
Not just because I get to snag a ton of gluten free Do-Rite Donuts in the morning then.
Chubbs the Adipose remembers
I am SUPER excited!
PS: While Janssen is paying for my travel expenses for the summit, all thoughts and opinions expressed here or on social media are my own… especially regarding donuts.