Brooklyn’s Journey with SJIA (MAS)
Brooklyn Harper Rex was diagnosed with Systemic Juvenile Idiopathic Arthritis (SJIA) complicated by a mysterious and rare condition called Macrophage Activation Syndrome (MAS), at the tender age of one. Little is known about MAS except that it can kill within hours and that it tends to strike about 10 percent of children who have Systemic Juvenile Idiopathic Arthritis.
At 6 months old, Brooklyn started getting an urticaria like rash that would come and go, usually appearing in the afternoons and gone by the following morning. Then 3 weeks after her 1st birthday she began to get fevers around 40’C which continued for 40 days.
During this time the Rex family met with more than half a dozen specialists but it was still unknown as to what was causing the fevers and rash. She was tested for diseases such as Lymphoma, Leukaemia, tumours, Lupus, as well as being treated for Kawasaki disease. Once she did not respond to treatment it was eventually suggested that Brooklyn be tested for HLH (Hemophagocytic Lymphohistiocytosis) through a bone marrow aspiration and lymph node biopsy, which came back positive for macrophage involvement. However, it didn’t explain the pain and inability to walk that Brooklyn was experiencing most days.
With researching further, there is a combination of HLH with juvenile arthritis called Macrophage Activation Syndrome, which is a rare life-threatening disease that has symptoms just like that of HLH. So we made a decision to fly to Cape Town to meet with a paediatric rheumatologist who had experience with this auto-inflammatory disease. After looking through Brooklyn’s medical history and examining her, he confirmed that she had sJIA and the worst that he had seen in a child her age.
The recommended treatment was to begin using chemotherapy, along with the already prescribed use of steroids and Ciclosporin daily. Within 3 weeks Brooklyn’s blood results plummeted and she was taken off the chemo. The next option was to use a new generation treatment called biologics. Biologics are generally difficult to get and often times not covered by insurance because they range from a few thousand to hundreds of thousands in value each and every month, however they can become effective enough for someone to begin to live a fairly normal life.
Brooklyn’s current (IL)-1 inhibitor biologic costs in the region of $4000 per month. She previously had a massive allergic reaction to another biologic that was an (IL)-6 inhibitor. In the future, she may need to move onto another (IL)-1 inhibitor biologic that currently costs in the region of $16000 per month. We are truly blessed because Brooklyn is now in a research program and her biologic is now being covered by an assistance program.
We hope that our story helps other parents out there to be brave and persistent when their little ones become ill. It’s never an easy road but always know there are others out there going through their own challenges, and sometimes even worse.
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