Melanie Cole (Host): Arthritis and other inflammatory joint and autoimmune conditions are very common in children contrary to what many people think. My guest today is Dr. Linda Wagner-Weinger. She’s a pediatric rheumatologist at Shriners Hospital for Children – Chicago. Welcome to the show. What causes arthritis in children? Are there different types people don’t even realize that children can get arthritis?

Dr. Linda Wagner-Weinger, M.D., MS (Guest): We wish we knew what caused arthritis and that’s why the official term for child arthritis is JIA, which stands for juvenile idiopathic arthritis – idiopathic meaning that we do not know the cause. There are, depending on how one counts it, about eight different subtypes of child arthritis and some tend to be more severe and some may be less severe. Some may be associated with a positive family history of arthritis while others pop up just out of nowhere. They present at different ages. The joint distribution, the presence of other symptoms, and the family history help to guide us as to the diagnosis of the type of juvenile idiopathic arthritis, which we’ll call JIA from now on. The laboratory studies can help to support the diagnosis or lead us in other directions, but they do not make a diagnosis of JIA. In fact, there are children that have JIA that have perfectly normal laboratory profiles.

Melanie: If you're a parent, children having growing pains, different things hurt in children all the time. What are some of the symptoms that would signal a red flag that would say this is not just normal growing pain and this could be something more?

Dr. Wagner-Weinger: That’s an excellent question and that’s when the pediatricians or family practitioners refer children to us. To have a diagnosis of JIA, one must have symptoms of joint pain, inflammation would include swelling, heat and pain present for at least six weeks, they have to be a juvenile – they have to be less than 16 years of age – and we have to make sure that we’re not missing another cause of juvenile arthritis. For example, there are children that could have lupus or other rheumatologic diseases that look like they have JIA, but really have a different rheumatology process that would be considered autoimmune disease or a different type of inflammatory disease process.

There are approximately 300,000 children in the United States with juvenile arthritis and probably more than half of them, or even more than that, have juvenile idiopathic arthritis or JIA, but there are several other types of diseases that do give juvenile arthritis such as lupus, scleroderma, fibromyalgia is considered under that category, so is dermatomyositis and various types of inflammation of blood vessels called vasculitis and those related diseases. There are many other diagnoses besides JIA that can be associated with joint pain and inflammation. You do need a duration of pain at least six weeks to make a diagnosis because sometimes children can have viral infections and have joint pain for several weeks, but then it goes away or they could have an injury which resolves. One of the things we look for is morning stiffness. On an exam, we look for evidence of inflammation like swelling or increased heat of various joints.

Melanie: What treatments are available and are there complications if it’s not treated proactively? Of course, arthritis can build on itself, so if this isn't something that gets treated right away, are there certain complications? Speak about some of the treatment options. What's your first line of defense?

Dr. Wagner-Weinger: The issue of treating early, and that means getting diagnosed early, is very important and we’ve learned so much over the past five decades when juvenile arthritis in the whole field of pediatric rheumatology was developing. We have learned in research studies both in adults and now in children that the sooner you diagnose JIA or adult rheumatoid arthritis and the sooner that you treat proactively with medications, which we’ll get into in a moment, the less injury you have to the joints and the better outcome we have long-term. When we start to realize a child does have a chronic joint process that has inflammation like JIA, we want to jump in immediately. The type of treatment that we choose does depend on what subtype children have of JIA. For example, only about 5% or 10% of our children with JIA have the adult form of rheumatoid arthritis, which is associated with the positive rheumatoid factor that’s more aggressive joint disease and it has more damage complications. We’re going to be very aggressive in those children. On the other hand, we have children that have just one joint involved and we can be a little bit less aggressive, yet we want to make sure that we are appropriate providing the right amount of treatment, not too little treatment so there's ongoing inflammation, but not too much treatment because as you mentioned there can be side effects to every medication that we use.

The treatment approach to juvenile idiopathic arthritis has changed tremendously over the last 40 to 50 years and especially over the last two decades. When we started out in the 1940s and 1950s, we had aspirins, and after that, we developed variously related medications, which were easier to take and have a longer half-life than aspirin, such as naproxen or ibuprofen and many other related nonsteroidal medications. I should mention here that as opposed to all the medications in this class of medications called nonsteroidal that are approved for adults, there's only a few that are approved for children, so that can be a problem and only two that come in liquid form, which is a big problem for children that don't swallow pills yet. In the late 70s and early 80s, we started to use a medication called methotrexate. Many of the children who did not go into remission on nonsteroidal medications did enter remission with methotrexate, yet we still had a good 20%+ of children who continue to have unchecked inflammation of their joints.

We were so excited at the end of the 1990s when the biologics were introduced and these are the ones that people see advertised all the time on television or in other market forums that include anti-tumor necrosis factor agents. I won't use any brand names, but for example, Etanercept, Adalimumab, Infliximab and several others that totally change the world of rheumatology. We used to see children that have arthritis using forearm crutches, in wheelchairs, getting joint replacements and so many of our lectures back then were how to do pre- and post-therapy after joint replacements. We don't do that anymore since the availability of biologics. I think the first one available to children was around 2000. We've seen a marked decreased in the morbidity of the joint disease in our children and we’re so excited about it so that when we have our annual juvenile arthritis meeting, you cannot tell sometimes who is a patient and who is the sibling of the patient in our family meetings because the joint disease can be controlled. I don’t want to be overly rosy and if there are still some that have ongoing inflammation despite the many different biologics that are coming on the market now, but the prognosis has improved dramatically. Side effects with every medicine that we use whether it be something simple like aspirin or the nonsteroidal medications like ibuprofen or methotrexate or the biologics, there's always a potential of side effects such as allergic reactions or suppression of blood counts or irritation of liver, etc.

I think the main questions people have about the biologics, which by the way, these are targeted therapies that attack a particular place in the inflammation cascade. They don’t suppress the whole immune system. They attack one little area and that can be successful in quieting down the joint disease. In altering the person’s immune defense system, which these medications can do, and people are worried about whether there's an increase in malignancy, complications down the road, and there have been large demographic studies done, and at this point, we do not think that there's any statistical evidence to indicate that malignancy is increased in this population that are on these medications. We'll keep doing research studies, but it still is a black box warning for these medications. The other concern with these medications is that we alter the immune defense system so we’re always looking for infection, we always pretest to make sure there's no tuberculosis exposure, and if a child does get a fever or some evidence of infection, we hold the medications until they're back to their usual state of health.

Melanie: What about the psychosocial aspects of arthritis in children? As you're talking and giving hope and talking about how sometimes it's really a great situation and the siblings and all of that, speak about dealing with school and their medicines, as you say, some can't even swallow pills at this particular age and taking them and where exercise fits into this picture. Give us a little bit of the psychosocial aspects of arthritis in children.

Dr. Wagner-Weinger: When we treat our children with a diagnosis of chronic illness whether it be JIA or lupus or related inflammatory or rheumatologic conditions, we treat the whole patient. There's no question that it can take a village and I think that’s where I would want to bring in some of the wonderful services that I've enjoyed working at Shriners Hospital. With regard to school, that’s their job. Just like adults can't go to works sometimes because their joints hurt, if our kids can't go to school, they're not getting the education they need. They're also missing out of the social events, and we know how important sports are at school, that’s a big social medium in which to meet people and feel like you're part of the group.

We try to have our children live a normal life as possible. We give information to the school, to the teachers, to the principals, to let them know that the child does have this particular special condition and need. It may or may not interfere with the children’s education. If we feel that it does interfere, then we make sure that we have available when a child’s condition will interfere with full participation in school. We utilize the services in the school district such as physical and occupational therapy. We make sure that they get the home or extra tutoring that they may need for missed days. We sometimes try to give them computers instead of having them write long assignments, give them extra time to complete assignments and examinations, and also maybe to use the elevator at school. We write notes so that they can be excused for gym or not be penalized if they don’t participate in activities that may cause them pain. We are always asking how they are doing in school, are they missing school, are they able to complete everything that they need to do at school both academically and socially.

Melanie: Tell us about the close connection with orthopedic services at Shriners Hospital for Children – Chicago. Is an ortho consult sometimes possible during the same visit if kids have related ortho issues from their arthritis? Tell us about some of the other providers that are accessible to children with arthritis that you work with at Shriners – PT, OT – who else do they work with?

Dr. Wagner-Weinger: When we have children that have significant disease, it does take a full team to optimize their treatment and their outcome. The orthopedic service has been invaluable in so many different ways. It’s a two-way street. We may see patients who may need ortho consultation on and sometimes it’s to provide intra-articular injections into the joint with corticosteroids to quiet down inflammation. Sometimes it’s to access a joint that mechanically seems that it’s causing a problem more than the inflammatory component and often the orthopedic surgeons will see patients that come in with complaints of pain and they feel that it is more than likely an inflammatory condition. The orthopedic surgeons at Shriners are more exposed than many other pediatric orthopedic surgeons to inflammatory diseases, so they're very good at picking it up at referring us patients as quickly as possible and we value their services. What surprised me the most when I started working at Shriners was the ability to have rehabilitation services evaluate my patients the same day. That has never happened in any other place that I've worked. If I'm concerned about low extremities or gross motor skills and other functions, physical therapy can see the child that same day. If the child is having problems with writing or activities of daily living, they may see the occupational therapist that same day. Even patients that have involvement of their jaw joints called TMJ, they can sometimes see the speech therapist the same day. There is even an intensive day therapy program for those children that need multiple services to get them back on their feet and functional in their everyday life. We’re able to refer to children for evaluation for orthotics for their shoes, which is phenomenal, and they’ll see them the same day.

As we talked about earlier, it's not just the musculoskeletal issues, but it's the psychosocial issues. Many of the families have concerns about finances or insurance or how this changes their lives of the complexion of their family with the other siblings and they can meet with the social worker and psychology the same day. If there are any problems with scheduling, they can schedule that in the very near future. Other services that we have are dietary. Some of our children are underweight and some are overweight, so dietary guidance is key to our children’s full health and potential. There is also with new biologics. Many of them are given IV, there are nurse practitioners at Shriners that are able to write orders and supervise expertly the IV infusions of these newer biologic medications and this is so important to many of our patients that do require this. We have a nurse specialist who works with our clinic who is able to get approval for the medications that are necessary because these medications are very expensive. These biologics can range from $20,000 to $100,000 a year so you can imagine insurance companies are not happy with us and our wonderful nurse specialists can get the approvals that we need. There are other subspecialty physicians there such as neurology that have been very helpful in assessing our children with musculoskeletal disorders.

Melanie: Wrap it up for us with your best advice and points to remember about juvenile arthritis, including JIA, what you would like the listeners to know and the parents listening to know about what's going on out there and what you're doing at Shiners Hospital for Children – Chicago?

Dr. Wagner-Weinger: At Shriners, there's a unique opportunity to have a full evaluation, presence of all the services that we need for rehab, ortho, podiatry, psychology, social work, and dietary that you really can't get anywhere as often when a child has a chronic illness. There are so many services that a child needs that the parents have to often take them to different centers and it’s a very important aspect of their care to get all their necessary services under one roof and it’s unusual to be able to do that and also in a timely and friendly way. I have really never worked in a hospital that provides all these services so efficiently and effectively in such a timely manner as I have at Shriners.

Melanie: Thank you so much for being with us today. This is Pediatric Specialty Care Spotlight with Shriners Hospital for Children – Chicago. For more information, please visit shrinerschicago.org. That’s shrinerschicago.org. This is Melanie Cole. Thanks so much for listening.