Who’s The Patient?

It’s odd making the transition to observer instead of active-participant in a growing child’s doctor appointments.  I’m accustomed to being the one to provide information when I’m in an exam room with my children.  Kids grow up, though, and things change.

My daughter’s rheumatologist talks to her, not so much to us.  When my daughter shrugs her shoulders and says, “I dunno,” the doctor doesn’t turn to me or my husband for our input; she rephrases the question and pushes my daughter for an answer.

As we sat on the sidelines and eavesdropped while the rheumatologist talked with our daughter a few weeks ago, I reflected back over the years.  Our other daughter was only three when I took her to an allergist.  I remember interrupting him as he was explaining the scratch-test to me, and asking if he would please explain it to my daughter instead of me, since she was the patient.  He looked quite startled and turned his head to look at her, but only got two sentences out before his head gravitated back to me.  He just couldn’t do it.

My daughter’s rheumatologist is definitely different.  I love how she treats my daughter like an intelligent person capable of explaining what’s going on in her body, but it’s weird to not be included in the dialog.

It was interesting, this new way of doing things, when the doctor asked if our daughter thought her prescription was helping.  My husband and I have both seen a difference:  less pain, better appetite, more activity, happier kid.  It hasn’t brought complete pain relief, but it’s definitely an improvement.  We weren’t asked, though.  Our daughter – the person who was asked – wasn’t sure if the medication was helping.

The rheumatologist suggested that she stop taking it.  Give it two weeks.  If there was no change, no need to continue a med that wasn’t making a difference.  If she did notice a difference, then after two weeks a different NSAID might be better.

That seemed like a sensible plan.  My daughter (who hates taking pills) was delighted to have the doctor tell her not to take her prescription and looked forward to her two-week reprieve.  The day after her appointment, she loved not taking a pill with her breakfast.  The following day, she came downstairs and announced dejectedly, “The medicine was helping more than I realized.”

My husband and I recommended that she take her medicine, but she’d heard that she should wait two weeks before making a decision and didn’t want to go against what the doctor had said.  It was Saturday, so we couldn’t call.  Monday morning, first thing, I phoned and left a message.  When the nurse called back, she told me of course your daughter doesn’t need to suffer for two weeks!

The kid even got to pick whether she stuck with the existing prescription or switched.  Of course she stuck with a once-a-day pill instead of going to twice-a-day, but the particular med she’s taking isn’t that important.  The point is that she chose.  She’s the patient, and she’s learning to make her own decisions regarding her health.

Given the awful day she had earlier this week, my daughter came to me and asked if I thought it might help to try the other prescription.  Although I’m tempted to phone my daughter’s rheumatologist, I don’t think it’s time to do that yet.  It was a one-day problem, not a definite worsening of symptoms.  Trying a different medication seems like a reasonable first step.

A few months ago, my dear daughter announced that we could make her go to the doctor now, but once she turns eighteen she’s never going again.  I suspect that was an expression of frustration with the cards she’s been dealt, not her true intention, but I need to realize that it’s not too long until she’ll be autonomous.  She could choose to never see another doctor.  Instead of imposing my opinions and decisions on her, I’m trying to guide her in making good choices (healthcare and otherwise) so that when she has the legal freedom to make her own decisions, she chooses wisely.

JRA

What is JRA?  People sometimes think that Juvenile Rheumatoid Arthritis is the diagnosis when kids get Rheumatoid Arthritis, but that isn’t accurate. Some children with JRA have a disease like adult RA, but most JRA is different than the adult version of rheumatoid arthritis.

The term “JRA” was used in North America in the past (in Europe “JCA” for juvenile chronic arthritis was used), but current terminology is JIA.

There are three main diseases categorized as JRA:

About one-fifth of kids with JRA have systemic onset juvenile rheumatoid arthritis, which in the past has been called Still’s Disease and is now called systemic-onset juvenile idiopathic arthritis. In addition to joint pain and swelling, this type of JRA is noted for high fevers and a salmon-colored rash. The liver, spleen, heart, and digestive tract can also be affected by this form of JRA.

About half of kids with JRA have fewer than five joints affected. Formerly called pauciarticular juvenile rheumatoid arthritis, this is now called oligoarthritis. It tends to affect the larger joints such as knees, hips, shoulders, and elbows. The eyes can also be affected, so referral to an ophtholmologist is important.

Three-tenths of children with JRA have five or more joints affected. This type of JRA is categorized as polyarticular juvenile rheumatoid arthritis. Polyarticular is the form of JRA that most closely resembles adult-onset RA; in some cases it can be identical. The new JIA classification system distinguishes between RF+ and RF- (those whose laboratory tests show a positive rheumatoid factor, vs those whose lab results are negative for rheumatoid factor).

There are other types of juvenile arthritis that are classified at JIA, but didn’t qualify under the JRA criteria.

For more information about the different types of JRA and how they are treated, see:

Still’s Disease: SoJIA

Some eponymous diseases are named after a patient (think Lou Gerig’s Disease), while others derive their name from the physician who identified the condition.  Still’s Disease was first described in 1896 by an English pediatrician, Sir George Fredric Still.

I recall the first time I heard someone say that he had Stills Disease.  Nobody present at the time knew what Stills was, and so the person explained that it was adult-onset JRA.  I thought, “Wait!  Adult-onset of Juvenile RA would be RA.”  It’s not.  The out-dated term JRA didn’t simply mean RA diagnosed in children; it’s actually a different disease (technically, at least three different diseases).  Adult-onset Stills is similar to one type of juvenile arthritis, but it is not the same.

“Still’s Disease” is Obsolete

George Still’s doctoral thesis was published in 1897, and described a childhood arthritis characterized by joint pain, fevers, and a salmon-colored rash.  This was later renamed systemic onset juvenile rheumatoid arthritis (so as not to be confused with the other types of JRA).

JRA, however, is considered by many to be misnamed, and the word “rheumatoid” is no longer used.  What was originally considered Still’s Disease and was later known as systemic-onset JRA, is now classified as Systemic-Onset Juvenile Idiopathic Arthritis.

Adult-Onset Still’s Disease

In 1971, a British rheumatologist, Eric George Lapthorne Bywaters, noted that some adults had a disease similar to Still’s (systemic onset JIA), but that their disease didn’t meet the criteria for a diagnosis of rheumatoid arthritis.  The disease is relatively rare and hard enough to recognize that it was featured in Dr. Lisa Sanders’ book, Every Patient Tells a Story.

When discussing Still’s, it’s important to distinguish the age when the patient was first afflicted.  The cut-off is age sixteen.  Older, it’s adult-onset Still’s; younger, it’s systemic-onset JIA.

Adult Stills tends to follow three patterns.  Some people’s disease lasts less than one year (monophasic).  Others experience cycles of flares/remission (intermittent).  Unfortunately, others experience on-going disease activity that can lead to joint destruction (chronic).

Treatment

There is no cure for either the juvenile or adult versions of this disease.  There are treatments, though.

Historically, aspirin or NSAIDs were the only treatment available.  In time it was discovered that corticosteroids (such as prednisone) can be effective in reducing the inflammation of this disease, but should not be used indefinitely since long-term steroid use can be bad.

In 1988, methotrexate was FDA approved as an RA treatment, and over time it has been used to treat other autoimmune diseases, too.  Other DMARDs that have proven effective in treating other forms of autoimmune arthritis can also be effective in treating people with adult Still’s/SoJIA.

Additionally, there are stronger medications (known as biologics) that can be very effective if the older DMARDs don’t provide enough relief.

Further Reading
In case you’d like to know more about this disease than “joint inflammation, fevers, and rash,” following are two of the many articles I read in preparing this post.

Adult Still’s Disease
Juvenile Rheumatoid Arthritis

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My reading in preparation for my daughter’s follow-up appointment with the pediatric rheumatologist has followed a bit of a rabbit trail.  I’ll be doing a few posts on a variety of different autoimmune diseases.