Although health disparities are prevalent in many chronic diseases, including rheumatic diseases like rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), they remain poorly understood in pediatric rheumatology.
Two analyses conducted by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry have highlighted important disparities among children with polyarticular juvenile idiopathic arthritis (pJIA).
The following is a press release from the ACR dated today:
The American College of Rheumatology (ACR) released two updated guideline papers for the treatment and management of Juvenile Idiopathic Arthritis (JIA). These two guidelines are companions to previously updated JIA guidelines released by the ACR and Arthritis Foundation in 2019 covering the treatment of polyarthritis, sacroiliitis, uveitis and enthesitis. One paper provides updates on the pharmacologic management of JIA, focusing on treatment of oligoarthritis, temporomandibular (TMJ) arthritis and systemic JIA (sJIA), with and without macrophage activation syndrome (MAS). The other focuses on non-pharmacologic therapies, medication monitoring, immunizations and imaging, irrespective of JIA phenotype.
The original JIA guidelines were published in 2011 and 2013, and this update reflects the ever-changing rheumatology field with new criteria on how to define disease and new medications to treat those diseases.
“As rheumatologists, our patients and caregivers expect us to review the literature and weigh the evidence so that we can suggest the best treatments, while also considering their preferences,” said Karen Onel, MD, Chief of the Pediatric Rheumatology Division at the Hospital for Special Surgery in New York and the lead investigator of the guidelines. “The field has changed tremendously since the 2011 and 2013 efforts, so we needed to adapt our guidance to the times in order to offer our patients the most nimble and state-of-the-art care possible.”
One example of where the guideline team has adapted is in their recommendations for using disease-modifying antirheumatic drugs (DMARDs). The guideline on the pharmacologic management of JIA emphasizes early use of conventional synthetic and biologic disease-modifying antirheumatic drugs. This is a much different treatment approach than what was previously recommended.
“For many years, treatment of JIA consisted of corticosteroids, non-steroidal anti-inflammatory drugs (NSAIDs), physical therapy, bracing and surgery. There were no DMARDs and even if there were, they were not tested or used in children,” said Dr. Onel. “These guidelines stress the early use of conventional synthetic and biologic DMARDs and the avoidance of glucocorticoids and NSAIDs. In fact, for systemic JIA the guidelines suggest using biologic DMARDs as a first line. We have turned the pyramid upside down.”
Two areas of importance for the non-pharmacologic paper were guidance on how to monitor drug toxicities for children with JIA and the importance of immunization. Laboratory test monitoring recommendations for medications such as NSAIDs, methotrexate, and hydroxychloroquine can be found on pages 6-9. There was strong support for the use of immunizations in children with JIA and specific guidance for children with JIA receiving immunosuppression, not on immunosuppression, and children who are under-immunized or unimmunized can be found on pages 10-11. Additionally, the guideline recommends the use of physical therapy and occupational therapy interventions and a healthy, well-balanced, age-appropriate diet.
Though the scope of the two guidelines differs, one thing they have in common: the importance of shared decision-making with the patient/caregiver.
“Not every decision will be appropriate for every patient, which is why it was so instrumental to receive input from both patients and caregivers when creating these recommendations,” said Dr. Onel.
While the guideline was being developed, the COVID-19 pandemic began, and COVID-19 immunization became possible. As none of the currently available vaccines against COVID-19 are live vaccines, recommendations for use in JIA should be similar to those stated for inactivated vaccines. While specific guidance on immunizing children with rheumatic diseases against COVID-19 is still lacking, the ACR has published guidance on COVID-19 vaccines for adults with rheumatic and musculoskeletal diseases.
At the time the manuscript was approved for publication, the Pfizer-BioNTech COVID-19 vaccine was approved for emergency use in children 5-15 years of age in the U.S and FDA approved for adolescents 16-18. In addition, two new medications were also approved while the guidelines were developed. These will be considered for future updates.
“There were areas that we didn’t consider at the start that now belong. The guidelines will have to be updated again. But that is a sign of a growing and changing field,” said Dr. Onel.
Like many other ACR guidelines, the updated guidelines for JIA were developed using Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, which creates rigorous standards for judging the quality of the literature available and assigns strengths to the recommendations. The papers containing the full list of recommendations and supporting evidence are available at on the ACR website.
PS: Hi, I helped with this!! Check out the other teams involved (pdf).
Hey guys.
How are things?
I know I don’t always address y’all, and that isn’t cool. If you’ve read a lot on my site, you’ll know that I don’t really relate well with the whole parenting thing. I’d love to, you know, not feel like an orphan. Where’s a tardis to turn back time when you need one?
In my recent life as a guinea pig mom, I’ve started learning a lot more about mothering and just how amazingly difficult it can be to handle a sick kid. I know guinea pigs aren’t kids, but gosh, they really are my baby boys. They’ve each had issues that T and I freaked out over, made vet appointments, and loved on those boys a lot more than normal.
I’m not stranger to worrying about and helping with kids, having rushed to California earlier this year due to my then-newborn nephew’s congenital heart defect and resulting open heart surgery. He’s doing well now, but still. And his big sister is so smart. I think of them every day.
I can’t imagine what it’s like to deal with this all as a parent, from the practical matters of working or income with doctor’s appointments to the lack of pediatric rheumatologists forcing you to have long breaks in between visits you have to travel hours for to watching your baby be sick and yet so amazingly them.
So here’s a post for you, to recognize all the things you do and all the emotional crud you go through while you hide it to fight for your kids.
You freaking rock.
It seems like every conference or gathering I go to brings me new ideas and thoughts to raise more awareness of arthritis. The JA conference was no different.
Check in at the hotel was just a little bit busy, but that’s bound to happen when you have 1700+ people! 1100 of us were first-timers which is a big part of why they have the conference set up this way for next year…
How exciting is that?!
My first order of business was to run down and snag my swag bag before heading over to the young adult opening session.
The opening night dinner was the next event, and the last that I went to for the night even though there was a young adult networking session.
The opening dinner was great with plenty of gluten free foods. We heard from AF CEO Ann Palmer, JA conference chair for the year and SJIA mom Rochelle Lentini, and others. Probably my most favorite part of the night was when Ana Villafañe talked and performed for us.
One of the things that I loved about the whole conference was how much more of a focus on SJIA there seemed to be compared to past events. SJIA is rare, so I get that many may not feel it as prudent to discuss, but it was great. I say this because Ana has SJIA. Despite her illness issues, she’s debuting on BROADWAY in November in On Your Feet, a musical about Gloria Estefan and her family.
Instead of hitting the YA networking session, T and I hit the pool – something that surprisingly we hadn’t done all trip yet! It was a wonderful way to rest.
The next day started at nine with a panel of amazing people – Ana, Todd Peck (NASCAR driver), and juvenile/adult rheumatologist Dr. Sandra Pagnussat.
All three of these amazing people have fought hard to become who they are today. They each went through difficult periods in their lives and how they made it through.
One of the nice things about being in the YA category is that I could attend the sessions meant for parents and caregivers… even though they couldn’t attend our sessions. For the next few sessions of the day, I was able to pop out and enjoy some SJIA and very science-driven sessions.
The first session I attended was on diseases like SJIA that are autoinflammatory in nature instead of autoimmune. Perhaps the biggest thing I learned in this session was that adolescent boys are more likely to have one SJIA flare and be done with it. If you have five years or more of active disease, it’s likely you will deal with it the rest of your life. The good thing, though, is that less than 50% of cases have severe or life-threatening complications… how sad is that what slightly less than half is good news?
Dr. Elder also talked a bit about Macrophage Activation Syndrome, or MAS, and how it often isn’t recognized right away due to the many diseases it mimics like hepatitis. It’s estimated that about 10% of SJIA patients will have overt MAS but that 30-40% will have symptoms of MAS without it being full blown. She explained the difference between autoinflammatory and autoimmune, which I hope to cover in a later post.
Interleukin (IL) 1 Beta, which Ilaris and Kineret treat, is responsible for many of the systemic features including rash. It’s also the major cytokine responsible for septic shock, which is what Kineret was first developed to treat but it failed to do so. IL-6 is responsible for maintaining many of the arthritic features including later osteoporosis and growth retardation. IL-18 is responsible for MAS and can possibly be used as a biomarker for SJIA in the future.
Something interesting that I found was that, in theory, you’re not supposed to consider a diagnosis of SJIA if the patient or his/her immediate family members have psoriasis. This is supposed to be consider Psoriatic Arthritis. I was a little shocked honestly, as I have psoriasis and SJIA, but Dr. Elder explained that there are outliers and both can exist in one patient. It’s just more rare because of the autoinflammatory and autoimmune differences. You don’t often see a person who has issues in both their innate and adaptive immune systems.
Novartis, the company that makes Ilaris, was kind enough to host a SJIA lunch Friday. I ended up at a table with the Sloan family and the Burgos family… whose little guy kept flirting with me throughout the rest of the conference.
I’ve developed baby fever Rafael!
I also was able to meet Leah Bush and Amanda Hendrix, two SJIA moms that I just LOVE. Honestly they’re a big part of why I blog and it was so nice to be able to give them some hugs and say hey.
The lunch itself was great. They displayed some amazing pictures from the Picture Your Best Day with SJIA project and talked about the new Know SJIA website as a joint venture between Novartis and the Arthritis Foundation. The website has some great resources including this handy SJIA symptom tracker.
The next parental session I crashed was Genetics 101 with Dr. Troy Torgerson from Seattle. It was more focused on the autoimmune side than the autoinflammatory side, but was very interesting. One of my favorite quotes from this presentation was “we’re all mutants.” There was something so comforting in knowing all of our DNA is messed up in different ways, and to have a pediatric rheumatologist who is also an immunologist kind of say that this doesn’t make us that different.
With me being a big of a science nerd, I LOVED this presentation. It was nice that I knew about some of it beforehand because it helped me to grasp some of the other issues he discussed.
The next session was led by Shelly Baer, Robert Hernandez, and Kevin Purcell (founder of Arthritis Introspective) on self-esteem and body image – “I’m sexy and YOU know it!” It, like many of the other YA sessions, was really a forum to bring up issues we had. It was interesting sitting in there and really realizing how far I have come in the last two years on self love and self care. I had multiple things I liked about myself and others were struggling to find just one.
It both made me proud of myself and show compassion towards others. It’s so hard to live with this disease as a young person, dealing with societal ideals of beauty when we can’t meet them.
That night the young adults hung out by the pool and took some selfies!
It’s been a while.
The next morning started off with an awards session, followed by a conversation on the new partnership between the AF and CARRA (the childhood arthritis and rheumatology research alliance).
I was starting to feel a little beat so I had to take up a lot of room.
The cool thing about CARRA is that it started out as a group of pediatric rheumatologists and researchers coming together to see what they could do to help these sick kiddos. We have all these great drugs, but more are coming down the pipeline. The registry that CARRA has developed can help to track information better and utilize patient information, deidentified of course, to bring more change for the better.
It’s an amazing way to promote patient engagement in research, which is something we all should be interested in.
The most moving part of the morning opener was when Vincent Del Gaizo got up to speak about his involvement with CARRA as a parent of a SJIA patient. He discussed his son’s difficult case and the changes in how well he’s doing now.
The next awesome presentation was about relationships. We heard from Dr. P (from the opening YA panel), Jeremy and Renee Forsyth, and George and Joy Ross. We heard about their stories and then it, again, turned into a very open panel. We discussed topics like disclosure, where I brought up telling T on my first date and how he’s always seen it as a part of me, communication, and recommitting to each other daily.
During our lunch break, I stopped by the Novartis table to ask about Ilaris and the different patient assistance programs available. Apparently they will even help you fight to get the drug put on your insurance company’s formulary! How cool is that?
T and I enjoyed some food truck goodies and I headed off to the next session on transition.
I was a little disappointed in that I heard there were supposed to be several people on this panel, but at the end we only had two – Catherine Miney and Janet Hess, PhD, MPHS, CHES. Janet helped to standardize the transition to adulthood across the state of Florida, which I’m sure wasn’t easy to do!
It was a very open discussion, and I wound up contributing to a lot of the answers with new ideas on how to handle what some of these amazing people were dealing with.
After sharing information on some of the apps I enjoy like Arthritis Power, I headed up to my room. There was another session to go but I was just wiped out and I needed to rest. Theron and I took a short nap and then went out for some late night barbecue before working on packing up.
In the morning we stopped by the walk & jungle bell run expo, where we ran into some of my favorite people like Tory who runs Mariah’s Movers. Then I ran into AF CEO Ann Palmer…
I bugged her for a minute to thank her for the change she’s brought to the organization because, honestly, this was a lot better than I thought it would be. Apparently she’s heard of me? Yikes!
We headed over to the hope tree next where we saw some of the cutest hopes, and I put up my own that no one else would have to lose a loved one to JA.
And then we heard from Joy Ross at the general closing.
They announced my friend Colleen Ryan as the next conference host and that we’d have TWO to look forward to!
The conference itself was so amazing. There were so many different sessions to chose from. The hotel was perfect, though sometimes the signage could’ve been better. The food was great and gluten-free friendly. The people I met were amazing and the changes on the way will make the AF into what I’ve felt I wanted it to be, at least for the young adults – a way to shape us into advocacy leaders, a way to get us more involved, and a way to shine despite our illnesses.
My time in Florida was already amazing, from meeting my friend Emily to finally going to Harry Potter world to enjoyed feeding giraffes at Busch Gardens to sitting on the beach and relaxing. I didn’t have time to hit up Disney, but that just means I’ll be back Florida. You better be ready for me!
There are SO many more recaps out there, including the official AF ones. You can also snag presentations and materials from the conference here.
Wanna see me live-tweet at a conference? You can! Join me on my MedX journey Sept 24-27! I’ll be live tweeting the whole time so hit me up@kirstie_schultz, catch the live streams at the Medicine X website, and join in the conversations using the tag #MedX.
One of the perks of my new job is being able to attend meetings geared towards those in pediatrics for improvement. Last week, I was able to attend a meeting on transitioning children to adult care – a subject that gets very little focus here in the US. Studies have been done recently, but most of them are based in the UK or elsewhere abroad. As a country, we have to get a system down.
Many peds patients don’t realize they can’t just keep the same doctor. True, some can if they’re seeing someone in family or internal medicine. However, if you’re seeing a pediatrician, you’ll have to leave that practice by the time you’re 25 (depending on your condition). For a lot of families, this comes as a surprise. The biggest first step is making sure that patients get that information – and that docs can help ease you into that transition.
Often, these changes mean that your insurance could be changing as well, which is an important topic to go over with young adults. The biggest thing of course is encouraging self care and self advocacy. As a provider, you want your patient to feel comfortable with who they’re going to but also to speak up when things aren’t right.
For those without a transition plan in place, there are increased hospitalizations, worsened medicine adherence (I HATE THIS WORD THOUGH), and possible lapses in insurance coverage. Kids need to know how to handle themselves to prevent these issues.
On the other hand, patients with a transition plan in place experience improved quality of life (from knowledge & education they receive), reduced readmissions, and there is often a lower cost involved for MD transition teams. This saves the patients and the overall healthcare system time and money.
So how do we get a good transition plan in place?
For patients, we need to have educational programs in addition to skills programs and support. Staff also needs to receive education, possibly with transition coordinators and joint care clinics to help with this process. The UK has already experimented a bit with joint care/transition clinics, and that’s helped tremendously. There also needs to be enhanced service delivery. We don’t often think of docs and other healthcare providers as having service type jobs, which I think we tend to associate with jobs akin to garbage collecting, etc. However, HCPs provide services for which they are billed. Our satisfaction as customers comes up more than one would think, but not as often as it should. Young adult/joint care clinics will help with this, as will enhanced follow up and phone support. I would also add support via other means of technology – social media, email, chat, etc. My generation hates talking on the phone, so PLEASE make it easier for us to reach out when we need to. I don’t want to have to work myself up to calling someone if I’m already concerned.
The gal who spoke to us was from the Center for Youth & Adults with Conditions of Childhood (CYAC, pronounced kayak). There is all sorts of great information on their website about how they handle the transition process including a workbook. The clinic takes care of people ages 11-22… which is flexible. Their oldest patient is in her 60s, but there just aren’t enough resources for her to get help elsewhere due to location. Damn the rural midwest!
They set up visits at predictable or personal milestones. Did you get your driver’s license? Let’s have a visit! Did you get accepted into a couple of colleges? Let’s have a visit and talk about what accommodations if any you may need, etc. They do a comprehensive assessment with transition plans starting early and care coordination across the board. If you have juvenile arthritis, the clinic would take over your primary care while coordinating with your pediatric rheumatologist. Once you decide on a college, the first step would be to get set up with a rheumy out there. The last step would be to set up a meeting with the new primary care doc. This helps gives the clinic more coordination, but also the patient more time to adjust to some of the other changes while keeping some things stable.
Their team consists of social workers, nurses, family advocates, and of course docs. They also have graduations! In order to graduate from clinic, you have to be engaged with your new primary care doc, have sustainable insurance, either be self-managing or have an assigned decision maker, have an engaged support system, and have any special care requirements met. Now that last piece doesn’t require you to be out of peds completely. If your ped rheumy will continue to see you longer and you’re on board with that, great!
There is a website called Got Transition – a partnership between the Maternal and Child Health Bureau & the National Alliance to Advance Adolescent Health. It’s great and you should definitely check it out! There are resources there for HCPs, which gives you a clue as to how you’ll be tracked and measured. There is also a section for youth and families.
In Washington DC, they’re moving to a new model of handling transition which seems to be going well. You meet with the peds doc, then s/he talks with the adult doc, then you meet with the adult doc. Once the full transition is complete, THEN docs get paid. Docs weren’t necessarily pleased at the idea, but hey what can you do?
As a part of the transition planning, docs need to be way more engaged in these patients’ lives. They have to find the new docs, give them info on the patients, and see if the docs will take on these cases.
A downside for patients is that they get MORE HOMEWORK. Ugh! But this is meaningful homework 😉 It may start out with you needing to learn the names of your meds to dosages to docs to setting up and attending appts on your own, etc.
One thing that struck a grumpy chord with me was the case studies section. Often, these patients were not sticking to taking their meds in order to fit in better and then having adverse events. It is true that this happens, but instead of placing blame on the patients, I think we need to look at our society as a whole and what we can do to improve how we treat ill people in general.
All in all this was a very informative hour. There need to be some changes made in how this runs I think to get it stable and running in more areas. However, we are SO far behind Europe on transitioning. It’s awful. Something is better than nothing, and changes can be made later on.
Do you find these ideas helpful? If you’re an adult, what would’ve helped you transition to adult care?
It’s not that I hate my old doc, but it’s very obvious that the office is too busy to really dedicate time to so many patients. It also shows in their lack of attention to detail (refill issues, lab results taking a week to get back to me instead of the three days, etc). They’re very sweet people and I love that the rheumy teaches, but the teaching is getting in the way of my care at this point. I gotta do what’s best for me and peace out.
I see my new rheumy Thursday morning which means it’s time for me to start obsessing as I usually do before appointments, about what I’m going to say in the short time I see the doc. This time, I’m scheduled for a whole hour, which will be longer than I’ve sat with one of my docs in a long time.
I obviously am going to have to bring up the fact that I don’t have any records from my childhood, since I didn’t get to see doctors. I want to bring up frustrations with my current rheumy team and the fact that they’re not treating me for SJIA/Still’s. I want to start talking about other meds. Cimzia isn’t awful, but I’ve also not done my shot for like a month and not felt any real difference other than not feeling quite as flu-like (I’ll do it tonight).
I’m sure that part of the issue with that too though is the torn labrum. I would think it is distracting my body more, even with the pain lesser than it was a month ago when this all started. It still is limiting my activity and causing pain. My quality of life is still lesser than it should be. Perhaps I can discuss surgery and thoughts about that with the new doc. Even though it’s feeling better, the fact that it’s still causing problems AND has always been an awful joint for me makes me feel like surgery would be best.
I also am going to start working on getting my records so that I can go through and correct them… and so I can have my x-rays and scans for fun decorations.
I have problems.
But seriously though, how cool is it to have pictures essentially of your insides, of what makes you move and function?
Anyway.
What are some things that you’ve brought up to new specialists? What is the most important to bring up in your short time? Is listing your expectations a good idea, or too bossy?