Thank you to the parents and doctors who attended our first-ever SJIA Family Day in Cincinnati, earlier this summer. You each played a major part in the event’s success. Everyone came together to learn, share knowledge and connect. For those who were unable to attend, we have gone through our panel transcripts and put together an informative Q&A that is presented below. If you want the full conference experience, or have questions of your own, head on over to YouTube to watch all the panel videos from the event, it’s possible a question you may have was answered during the event.
Each question below was asked by a parent either during the Expert Panel session or in the Q&A session of the physician talks. For each question that we present below, we are also linking directly to that section of the Q&A on YouTube. You’ll find a list of the doctors and their practice area at the end of this post.
Q 1: Can food help bring SJIA under control?
Dr. Grom: It’s unlikely that you’re going to change the disease as far as we know. However, if your child is treated with steroids, for instance, it is important to try to maintain a healthy weight. That can be furthered by using a healthy diet. My concern, for example, is that if you decide to go with a gluten free diet, it will limit the number of products that you can buy in a supermarket and use in your cooking. As a result, you may lose important sources of important vitamins. A gluten-free diet could eliminate whole grain bread, it’s a very important source of vitamin B1-B6 in our language. When you start playing with diets you may hopefully get some benefits, but you may also put your child at risk to develop some other deficiencies for vitamins, for certain micro elements. To me, I think the diversity of food – fruits, vegetables – is more important than a particular diet. Watch the response to this question and find out what other doctor’s added.
Q 2: What is the best way to help with communication between the different specialists your child is seeing?
Dr. Lovell: I think the gold standard is a care conference. This is where you actually get the people physically together in the same space with you. You have a discussion focused on your child’s problems and come up with a consensus about how you’re going to move forward. So that one doctor knows what his assignment is and another doctor knows what her assignment is, you know what your assignment is, and you move together as a team. I think that’s the ideal. There are other ways you can get at it in the same way. You can have virtual conferences with Facetime or WebEx if you have doctors in different institutions. And then if, by chance, your doctors and you utilize one of the patient portals like Mychart that can be the mechanism by which you can send questions to multiple physicians and everyone can see the responses from other people. Watch this response and see other Q&As.
Q 3: How do you handle the transition of care when patients enter young adulthood? We are concerned that doctors are now calling her disease Still’s Disease, when the only thing that has changed is her age.
Dr. Brunner: Even though your daughter is now 19, it doesn’t mean that she has a different disease. She still has systemic JIA. That is important for treatment purposes. For instance, Tocilizumab is approved for systemic JIA. And if she turns 19, she still has access to that drug because she still has systemic JIA. What happens sometimes when you go to the adult side, it’s easier for them to prove prior authorization if they have certain kinds of diseases that they’re used to. Sometimes they change the nomenclature a little bit, and it’s very possible that your daughter will be referred to an adult-onset Still’s disease for that. But in our formal scientific point of view, your daughter still has systemic JIA.
Stepping back to the transition, transition of care is a process. What it requires is that the patient is ready to advocate for themselves independently in another setting. Watch other physicians on the panel also answer this question.
Q 4: We know that Still’s disease and SJIA are the same disease. But when you say that it’s even more rare in adulthood – the Still’s disease – does that tell us something about the disease? Is it possible that as you get older that your immune system becomes more sophisticated in some way?
Dr. Grom: Recently, I’ve seen a couple of publications describing the incidence of systemic JIA and Still’s disease over time. If you look at the figure, you will see that there is clearly the highest instance somewhere around the age one, two, maybe three years old. And then it kind of starts tapering off and it sort of gradually keeps going down all the way until 15-17 years of age. In terms of incidence, it’s really a very small curve. We see it more often in kids who are young and whose immune system is still developing. Then somehow, with the maturation of the immune system, I think the risk for this disease tends to go down. So you are absolutely right, there is something about the immaturity of the immune system. Watch other physicians on the panel also answer this question.
Dr. Grom: Although we believe that only 10-15 percent of patients develop what they call full-blown MAS, mild or subclinical MAS may be occurring in as many as one third of patients with active systemic disease. As pediatric rheumatologists — as physicians — we need to recognize this phenomenon very early. Because if we recognize it early, just a slight increase in the dose of steroids may actually prevent the development of full-blown MAS which would require a much more aggressive and much more toxic treatment. Again when we suspect – when we worry – about the possibility of MAS, particularly when we suspect subclinical MAS, the typical laboratory tests that should be sufficient for monitoring include cell blood counts. Remember, we are looking for decreasing cell blood counts. Watch the response and see further information about MAS.
Dr. Huggins: We normally have conductors of our immune systems, the cytokines, like IL-1 or IL-6 and they can interact and direct things in different ways. So the cytokine like IL-1 would bind to its receptor on the cell’s surface and then conduct things downstream that will tell those cells what to do. So we have ways of blocking those conductors if they’re overactive.
We can either create a biological molecule that looks like the receptor, only if it’s in their bloodstream and binds that protein that way. Or we can make a protein against that cytokine before it ever gets to its receptor and prevent it from doing its work. And some of the examples of this is things like Etanercept, or some of the TNF agents. Another way that we can interfere with this normal interaction is we can have a receptor antagonist like Anakinra. “RA” at the end of that receptor stands for “receptor antagonist.” That we keep the cytokine from doing its normal function of binding. Or we can also make this protein a monoclonal antibody against the receptor like Actemra does. Watch the rest of this answer on YouTube.
Q 7: If you talk to other SJIA parents, a lot of them say their doctors told them their child is an atypical case of SJIA. What is a typical case of SJIA?
Dr. Huggins: I think it’s an excellent question. Every day is a humbling day and the minute that you think and you don’t look at all the things that are outside what you’re expecting to see, you’re going to miss some very important things. When we say that as physicians, it frequently means that we feel like we’re earning our pay that day because there’s something happening where we don’t quite know what we should do.
In rheumatology, especially if we stick to just what we know, we’re going to miss some very important things. It isn’t the easiest thing to teach, believe it or not, but it is something that we work hard at teaching, which is: each visit is a re-evaluation. For instance, do I have the right diagnosis? Do they still need these medications? Do I have the right medications? Am I missing something? Are they growing? Do they have the vaccines that they need? Are we paying attention to their cholesterol? All those kinds of things. So, it is a very good question. Watch the rest of this answer and others on YouTube.
For your reference, here’s a full list of the doctor’s who answered questions above:
Gary Hembry says
Hi, our daughter was 1.5 years old when she first displayed her SJIA symptoms – slightly stiff neck, rashes and extremely high fevers. She has been on 20ml daily Kineret since this, which seemed to completely solve all her symptoms. 5 months later, our doctor recommended we start to ween her off the Kineret since she had normal blood results (elevated ESR 21 and low creatinine 0.38) . Since then, her neck condition became a lot worse (from c1 inflammation) and now our doctor recommends changing to Actemra. What would your advice be?