Juvenile Arthritis: A Cautionary Diagnostic Tale of Rheumatology

Mini-Mumps,” he declared. 

This doctor was not our first choice, but we lived in a one red light town. The preferred doctor was near retirement and did not accept new patients. So we saw Dr. Second Best and he declared that seven year old B, presenting with sudden sore throat, high fever, and severe headache, had Mini-Mumps. This malady was apparently his own construct. When I questioned that it seemed like Strep to which our family is prone, he dismissed my concerns. Instead, he tested her for Mononucleosis. As I type this today, I am floored all over again by how ludicrous the notion. I was a young mom at the time, and I could have never foreseen how pivotal and life-changing this illness and proper diagnosis would become.
 
Of course the Monospot test was negative, and Dr. Second Best prescribed antibiotics. Still, she continued to languish with throat pain, fever, and the swelling on her neck grew to a 2” diameter lump. She lost five pounds, developed sores at the corners of her mouth, and for the first time in her life was having spontaneous nosebleeds during the night. When she did not respond to the antibiotics, we took her to Dr. Next Town. He took one look at her and thought it was strep and clearly more serious. He contacted an ENT in Austin, 45 miles away and arranged an immediate appointment.
 
The ENT was very concerned about the significant lump on the side of her neck and did a fine needle aspiration and some scans. The lump was not a tumor, so we were not dealing with childhood cancer. We were grateful beyond words. After a one-week trial of Augmentin, the ENT admitted B for intravenous antibiotics. Though still tired, she did well in the hospital, as the heavy doses of antibiotics calmed the illness. However, the lump on her neck did not respond. So it was time for surgery. As it turned out, she had an abscessed lymph node. He had to drain the abscess, and B was sent home with a soft rubber tube protruding from the surgical wound to allow the final drainage. David and I had to wear our Perfectly Calm Parent Faces as we gently tugged at the rubber tube and cleaned the area as instructed.
Second Grade
 
B started Second Grade with the tube still in place, and wore a large gauze bandage around her neck. She answered the glaring questions, but was excited to see friends and to use that box of 48 crayons. She continued to take antibiotics until the tube was removed and the surgery declared a success. However, a short time later, strep returned. This time, we saw only Dr. Next Town who never hesitated to test for strep and treat her appropriately. She would complete a 10-day course, and two days later the strep would return. It became necessary to take a prophylactic approach and keep her on daily Amoxicillin.
 
One September morn at 2 a.m., B crawled to our room in terrible pain. Her ankles and wrists were locked. This was beyond our experience and we did our best to comfort her. We did not quite know what to think, and wondered — a reaction to surgery, another infection? Dr. Next Town saw her immediately, and once again arranged an immediate appointment with a very kind pediatric orthopedic surgeon.
 
The orthopedist ran labs and did a physical exam, noting a pinpoint red rash on her thighs, and the fact that her surgical wound was slow to heal. His immediate assessment was that this was a reactionary arthritis from the illness. Even after weeks of treatment and intravenous antibiotics, she still registered an elevated ASO titer (indicates recent Strep infection). Based on this and the collection of unusual symptoms, he declared that she had Rheumatic Fever. He referred B to a Pediatric Rheumatologist and prescribed liquid Advil in a fairly strong dose for a seven year old.
 
It brings tears to my eyes to think of the pain and stiffness B had in those early days, yet how usual and tough she remained. She immediately seemed to accept that “what is” had changed, far more easily than her parents. Kids are resilient like that. We parents have to remember to embrace that good cheer, and just quietly wipe away our tears. She would become stiff after sitting for periods of time, and her nightly bath seemed to escalate rather than relieve symptoms.  After she bathed, I would help lift her out each night and wrap her in a thick, cozy towel. I donʼt believe she ever cried.
 
Sitting at her desk or cross-legged on the floor at story time was causing stiffness. So, I spoke with her teacher to explain what was going on. She was a tall, gray haired wife of a preacher, but failed the lesson on compassion. She suggested that B had enjoyed the attention of her hospital stay and surgery, and that she was trying to push my buttons. I am not making this up. This episode could comprise a blog post, but I’ll spare you the vitriol.
 
We waited four months to see a pediatric rheumatologist in Austin. During this time, B developed more pain areas including her hips and her rib joints. Her ribs would lock up and her hips would pop. As long as she remained on Advil, the symptoms were fairly controlled. Each time weʼd try to reduce the dosage, her pain and stiffness would increase. She would have good days and not so good days.
 
She saw Dr. Red Suit on a good day in January. This young pediatric rheumatologist wore stiletto heels and a bright red skirt and jacket to greet a seven year old. Not exactly warm and fuzzy. And it foretold the outcome of the appointment.
 

Dr. Red Suit examined B and peppered her with questions. When B had forgotten that her rib joints had been painfully locking up, the doctor lifted an eyebrow toward me. Her clinical notes would state that “the child could not recall rib pain.” I related to her that  the orthopedist believed B had Rheumatic Fever, and she haughtily told me that she did not meet the Jones Criteria. This was 1992, and I would find out years later that the Jones Criteria were amended that very year. She did in fact meet the new criteria.

Revised Jones Criteria, 1992
Nor did Dr. Red Suit believe that B had any form of juvenile arthritis, and declared that she was simply hypermobile. She suggested we discontinue the Advil, though I explained the cause and effect each time weʼd try to lower the dosage. Dr. Red Suit did not order labs, nor did she find a follow-up appointment necessary. We were on our own.
 
As the years went by B would still have intermittent pains, stiffness, popping joints, and was prone to ankle sprains. We would refer to it as her “arthritis or whatever it is,” and she recalls finding it uncomfortable to sit at her desk for a full day of school. When she was ten I tried again, this time with an adult rheumatologist. I explained her history, the presumed Rheumatic Fever, and her intermittent but ongoing joint pain. I emphasized that we just wanted to know why this was still occurring. After the briefest of exams, Dr. Dismissal chastened me with, “You donʼt want your child to have arthritis.”  No shit.
 
It would be fourteen years and several doctors later that B was diagnosed with Psoriatic Arthritis. This diagnosis was later amended to Undifferentiated Spondyloarthropathy, Rheumatoid Arthritis, and Sjogrenʼs Syndrome.
 
I still donʼt want my child to have arthritis. 

An Advocate for One

Back in the mid-nineties everyone was watching Seinfeld.  We caught up years later when it went to syndication.  Hubby and I were too busy raising our three little kids and restoring a 1921 Bungalow (well, just keeping the doorknobs on most days).

Our world changed when our seven year old daughter, B, awoke in the middle of the night with her wrists and ankles locked.   She’d had uncontrollable strep throat, followed by presumed Rheumatic Fever (specialists were in disagreement), and suddenly we were confronted with Juvenile Arthritis.  It was presumed to be temporary; when it proved not to be, I was suddenly that mother who was issued eye rolls by rheumatologists.   You don’t “want” your child to be diagnosed with arthritis one doctor told me.  To be fair, he was right.  I didn’t want her to have it.  Twenty-two years later, I still don’t want her to have arthritis.

For years, we contended with “normal” labs and disbelieving rheumatologists, all the while teaching our daughter to be strong. Come up with a good back story for that surgical scar!  Ride the bike, climb trees, play basketball — despite ankles that roll and rib joints that lock up and make breathing painful.  We taught her to keep dreaming.


I became an advocate for one, seeking answers.   We did not have the luxury of a second car, so while B was in school I walked my baby boys to the public library in their double stroller.  An outing in the dappled sunlight of tree-lined streets for them, and information for me.  I lifted heavy, dusty volumes off the shelf to find only side-note mentions of Juvenile Arthritis.  


This was the sole means of information for parents of children with arthritis in the early days.  We relied entirely on existing publications found in the library or browsed for newer research in book stores.  Years later in Alaska, I booked passage on the World Wide Web.  I spent long winter nights with my beige Mac Performa 6320.  The same old dusty volumes were now available electronically, and I sought information as to why her joint pain persisted and what form it might be.

 

Entrance to our neighborhood, adorned in ice fog
 
B finally saw a visiting pediatric rheumatologist from Seattle who shared my suspicion of Spondyloarthropathy.  Yet when the HLA-B27 test proved negative, this diagnosis was discounted by our local rheumatologist.   It was not until she was twenty-one that finally, finally a rheumatologist believed that she had Psoriatic Arthritis. That diagnosis was ultimately amended to Undifferentiated Spondyloarthropathy and Rheumatoid Arthritis.


The world has changed so much in the twenty-two years since B was stricken.  Information is now at our fingertips, yet diagnoses are sometimes still just as elusive.  


Despite the easy availability of information today, it is still just as important for parents to advocate.  First for your child’s medical needs, for awareness that Kids Get Arthritis Too, and to help your child find new ways to dream. Keep adventuring, even if the path takes a new curve.  You never know what you will find around the bend.


Autumn in the Chugach Mountains, Alaska
(our favorite hiking area & after-dinner drive)
 
When did Chronic Life visit you & how have you helped your child keep dreaming?