Monday, November 13, 2017

Master Kirsten Has to Rest Sometimes

obligatory feet in ocean photo with white text "Master Kirsten Has to Rest Sometimes" and "Not Standing Still's Disease"

I was hoping to report more from the ACR. 

Unfortunately, after being so excited to attend, I just didn't think about how my body would cope with things. I was unable to attend the last day I was there (Tuesday) and had to go home earlier most other days as well.

I spent a lot more time in bed watching Netflix than I had anticipated. 

I was originally really upset with this when I had to head home early on Sunday. However, after getting back to my Airbnb, I realized part of why I was feeling so crummy was that my mystery WTF was hitting. There wasn't really a choice for me - I had to be in a space where I could have a lie-down and, honestly, a convention center really isn't that.

After a few hours of resting on Sunday - and a phone call from T (and the piggies!) - I wound up having the energy to go to the beach and get some food. After all, I needed to eat dinner and it was 30 degrees at home. Laying down outside would still be laying down.

glossy photo of Ocean Beach with the water reflecting the partly cloudy and sunny sky

While I stayed all day Monday, I wound up having to sit out the afternoon sessions because I was just not feeling well at all. After a quick meetup with some friends at the conference, I headed out to have dinner with a few disability justice friends.

I felt like it was the push I needed to get through the next day. However that helped emotionally, my physical body just screamed hell no Tuesday morning. It was a Netflix and rest day, rounded out well by visiting the windy and cold beach before snagging dinner with a dear friend in the area.

As much as I wished I could've attended some of the intense sessions on Tuesday - especially the one on Still's and other autoinflammatory diseases - I know that I needed to rest. It's incredibly hard to let myself do that, but it's getting more commonplace.

It also occurred to me while writing this that I never shared - I got my degree! My Masters is now hanging proudly on the wall in our short hallway. Things feel a lot lighter with that finished. I'm also grateful to have snagged two part-time jobs that will let me still work at home and around my schedule.

So, if you're looking for more updates about sessions from the ACR, I, unfortunately, don't have them. I hope to write stuff up about some of the posters and research I saw, though! It just might take a bit before those make their way up here.

I hope that you're resting as much as your mind and body need.

Monday, November 6, 2017

Macrophage Activation Syndrome Session #ACR17

square photo; background design has a blue background with beehive shapes in a tealish color; in the middle is a white circle with black text "Macrophage Activation Syndrome Session" "Not Standing Still's Disease" and "#ACR17"

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:
  • Platelets 
  • WBC 
  • Hemoglobin 
  • ESR 
  • Fibrinogen 
  • Albumin 
  • Sodium

  • 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 (IFN╬│) 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.


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.

Sunday, November 5, 2017

Opening Lecture #ACR17

white crinkly paper background; grey oval with black outline and teal text "Opening Lecture #ACR17"; teal text "Not Standing Still's Disease"

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

Friday, November 3, 2017

Heading to ACR

This afternoon, I board the first of two flights on my way to San Diego for the American College of Rheumatology annual meeting!

I'm incredibly excited to be attending this year. It'll be my first year there. I'm unsure what exactly to expect. I'll be covering sessions I attend here (where relevant). There are a number of SJIA/Still's/MAS related sessions, and I'm excited to be able to bring you news from those.

If you're in San Diego, hit me up! I leave Wednesday morning.