If the war seems difficult for the majority of us, it can be overwhelming for the youngest of us who suffer from juvenile idiopathic arthritis (JIA). The American College of Rheumatology estimates there are about 300,000 children in the United States who suffer from JIA. According to the American College of Rheumatology, there are four subvariants of the disease: oligoarticular JIA (most common), polyarticular JIA (most like RA); and enthesitis-related JIA. Recently a former subvariant of this condition; systemic onset JIA was recently reclassified as an Autoinflammatory disease.
The front on this part of the war on RA can be both hopeful and devastating, depending on where I visited. According to aboutkidshealth, oligoarticular JIA (oJIA) patients are mostly girls, and they are divided into two groups: those who develop the condition before 4 years of age, who are termed oligoarticular-persistent arthritis, and those diagnosed after 4, who are diagnosed with oligoarticular-extended arthritis. Both of these types of JIA impact up to five separate joints — usually knee, ankle, wrist and/or elbow joints.
Polyarticular JIA (pJIA) is the second most common form of JIA. According to aboutkidshealth, it also affects more girls than boys. It impacts small joints of the hands and feet, and large joints like the knees, hips and ankles. Like RA, pJIA is often associated with fatigue and may also cause low red blood cell count, rheumatoid nodules and a low grade fever. You can imagine how difficult this is for young children and teenagers who want more than anything just to fit in with others. It sickens me to think of children enduring such pain when they should be playing and enjoying life.
According to the American College of Rheumatology, Enthesitis-related JIA is a term used to define inflammation of the connective tissue sometimes at the base of the spine or in other joints. Children with this condition often experience painful joints and significant back pain.
The least common but most devastating form of JIA is systemic onset JIA (sJIA). To get a full measure of it I contacted Emma Brown, the dedicated mom of Reagan, who suffers the unimaginable impact of sJIA. Reagan is one of the children who fight every day in our war. Reagan was diagnosed in 2012 at the age of 9 months. At that time she suffered a terrible fever that the doctors thought might be an ear infection. After a visit to the local ER and a referral to a large children’s hospital, Reagan was initially misdiagnosed with Kawasaki’s disease. In Reagan’s case it paid that her mom stuck with it and continued to insist that this once active child who was starting to crawl was now reduced to laying in her mother’s arms and crying whenever she was shifted.
The financial strain that is placed on her parents is awful. As they anticipate a life of treatments, it is sometimes difficult for them to see how they will be able to afford these lifesaving medications. Like many of us, cost is one of the major complications of having sJIA and for Reagan’s family, it is a constant worry. CreakyJoints has a resource to help find financial assistance for medications.
Another complication that Reagan has experienced is macrophage activation syndrome (MAS). MAS occurs more frequently in sJIA patients. The website Rheumatologist describes MAS as “a condition caused by excessive activation and expansion of T lymphocytes and macrophagic histiocytes that exhibit hemophagocytic activity.” Emma describes MAS as “one of wickedest things Reagan has ever had to endure.” Again, it was through persistence that Reagan survived her first bout with MAS. Doctors were unable to immediately diagnose this syndrome, and it was a struggle for Emma to help them pinpoint Reagan’s life threatening complication.
It took research by Emma, Reagan’s local doctors and a faraway medical team to finally come to the correct diagnosis and treatment regime. Even then it was close. And that highlights the most difficult part of the fight against JRA. There are simply not enough trained pediatric rheumatologists in the world to cover these complex cases. JRA is different than RA — it is often more aggressive, the outcome is often life changing and the stakes are at the highest level even for seemingly minor issues. A sore throat or the flu can quickly escalate into a full blown fight of life and death.
Emma Brown puts it this way: “This disease is a silent killer. You may think it’s just arthritis, like the kind our grandparents have, which is painful enough, but the name is misleading. In fact, the name prevents people from understanding the terrible things she has to go through daily.” One can only imagine how relatable these words are for most of the families who deal with JRA.
It is true that those who suffer from JRA are our most vulnerable warriors. Even as we adults fight on our own front line, we must also fight the war on RA up and down the line. It is our obligation for all the warriors who fight alongside us, even those who cannot yet speak for themselves.