Wednesday, August 11, 2010

What is JRA & why do you make such a big deal about it?

It occurs to me that many people don't understand how serious arthritis can be.  Several times this week I've heard "Just be happy that what your daughter has isn't serious!"  One day, my child will be told that she can't be sick because she looks ok.  People will minimize her pain.  They will not understand.  Why?  Because it's invisible, and people don't understand that this isn't "nothing".  First of all, arthritis is the #1 cause of disability in men, women & children!  There is no cure.  It can be treated, but damage cannot be reversed.  What these kids have is NOT like what your grandmother eventually got when she was 80 or even 60.   Juvenile arthritis is all rheumatic in nature, an auto-immune disease.  Back when my parents were children, rheumatic fever was common.  Many of those kids developed temporary arthritis.  Other rheumatic diseases include Lupus, Scleroderma & Raynaud's.  Overlap Syndrome (Mixed Connective Tissue Disease) is also common, affecting 10-15% of the JA population.

There are many forms of Juvenile Arthritis.  Since there are so many, they get lumped together.  JA is an umbrella term for many different forms.  There is a form that only affects the back and neck.  There are children that have 4 or fewer joints affected.  Then there are the more severe forms. 

The "pauciarticular", now referred to as "Oligoarticular" is the type that affects 4 or fewer joints, BUT the kids that test positive for the Autonuclear Antibodies (ANA) have a much higher chance of arthritis of the eye.  There is uveitis.    "Uveitis is a rare medical illness that affects vision. Uveitis is the third leading cause of preventable blindness in the developed world.  Uveitis affects children, adults, and the elderly and is found worldwide in every race."

There is "polyarticular", which is what my daughter suffers from.  Some children are only affected by five joints, but others just get taken over.  Many of these children have had hips replaced, numerous jaw surgeries, & knee surgeries.   Before the newer biologic drugs came out many of these children became deformed over time.  I wish that I could find the quote, but what I remember went like this:  (from a FB post) "I remember when I went to arthritis camp as a child that many kids were in wheelchairs and / or deformed.  Nowadays, since Enbrel came out you can barely tell that they kids have JA."   (Thanks, Ms. Rabbit!)   I have no doubt in my mind that without Enbrel my daughter would be permanently in a wheelchair.  What is Enbrel?  Enbrel & Humira are TNF blockers.  They block inflammation before it can start.  Emily has 2 injections per week of Enbrel.  When her body behaves, she also has an injection of Methotrexate.  They're not exactly sure how MTX works... they just know that it does.  MTX is actually a chemo drug, but then it is used in much larger doses.  When her body allows, she has one MTX injection per week.  We are blessed that she only needs 2-3 shots a week to move.   Right now she is sad that she cannot take her MTX.  She can walk without it, but she's been very stiff, & her fingers are extremely puffy, as well as her knees & elbows.    Her hands are also affected by her Raynaud's & scleroderma.  They are going to teach her typing for her testing at school because writing is so hard for her.  And she's 8.  How will that be when she's 48?  And very soon her doctor is supposed to put her on a newer medicine that is only given via hospital IV, Orencia.  Yeah, that's not serious.  Did I mention that these are also immune suppressors, so these children are more susceptible to every bug that comes along?    Oh, and Emily has labwork done every 6 weeks to make sure that her white counts don't drop too low, and because one of her meds may possibly cause liver damage.

Side effects of long term JA can include joint contractures.  A contracture happens when a child alters the way that they walk in an attempt to lessen their pain.  After a long enough time of moving the wrong way the body reacts.  The joints and tendons ball up so often that they can't go back to normal.  This can cause one leg or arm to be shorter than the other.  It can also cause hip damage.  I guess that it's lucky that Emily has this in both knees. She's less likely to have hip damage because her legs are both equally messed up.

The next type that I will address tonight scares the heck out of me.  Systemic JA is like an internal arthritis.  Just think... I'll bet that you think of arthritis as a normal wear of the lining of the joints.  It doesn't always have to be joints.  Arthritis can affect the linings around the internal organs as well.  Systemic is usually found after someone notices that the child has unexplainable fevers daily.  They have to spike and fall at least once a day to be true systemic.  Often they are admitted in to the hospital and have every test known to man done on them.  At the end of 2 weeks the internists call in the rheumy's.  Usually the rheumy's can take the FUO "fever of unknown origin" and give them a name.  Systemic can really terrorize the body.   If you can, check out this note:!/note.php?note_id=387313903894, or visit the Sloan blog.  Systemic can kill our children if not properly treated.

There are other problems that my girl has.  Apparently, the turning purple is rare.  (Good to know!!!)  That one really freaks me out.  Every year it gets worse.  We have a drill in place at school with rice socks and hand warmers.  (Yes, we have hand warmers in Florida.)  Raynaud's is the overreaction of the blood vessels to cold.  Pretty much, they go into overdrive when she gets a tad bit cold.  Raynaud's can be a primary condition or a secondary condition.  Oddly enough, the secondary condition can be much more severe than the primary.  Raynaud's can affect hands, fingers, toes, ears, noses, and the tongue.  (How do ya fix that???)  It is more often associated with Scleroderma than JRA, but nothing about rheumatics is typical.  With the Raynaud's, Emily is ALWAYS cold.  She used to be able to wear dresses to school but now she can't even wear shorts in the summer without an episode or 12.  A true episode will start with the affected area turning white as the blood stops flowing, then purple as the blood starts to move again, then bright red as it all rushes back.  Eventually it goes back to normal.  If it doesn't turn white & purple it isn't true Raynaud's.   My little one sometimes gets these big, painful lumps.  They are so painful that if they are on her fingers she can't hold a pencil, and if they are on her feet she can't walk.  They can be bright red, or sometimes purplish.  We've been told that it's caused from the Raynaud's, but at the conference the rheumy that we spoke to said that it's Lupus Pernio.  We need to revisit that with her rheumy.  He hasn't actually seen them.  I've shown him a pic, but it's different than seeing it. 

Next is Scleroderma.  Scleroderma isn't an arthritis, but arthritis can be a side effect or an associated disease.  With Emily it's part of the overlap condition, MCTD.  Scleroderma can also be systemic, or it can be linear, affecting the skin.  Some have both.  Systemic, from what I have read, is similar to the systemic form of arthritis.  Emily has linear.  It is a tightening of the skin, making it tougher.  "Sausage fingers" are common in this type- very puffy, swollen looking fingers.  I couldn't tell on any other area for the longest time, but her doctor showed me that if you pinch the fatty part of an arm that is not affected you can pinch a big portion.  If you pinch an arm affected by scleroderma you don't get much of a handful.  Some days I can't get her needle in.  In some cases the tightening is severe and can actually make areas look deformed.  IE... a wrist affected by scleroderma can tighten enough to make a finger stick straight out.  A knee affected won't allow the leg to straighten.  The most common medicine for this is the Methotrexate that my little one can only sporadically take.  She has labwork every 6 weeks like clockwork because they worry about liver issues with MTX use, and often when she's on it her white cells crash.  Apparently, that doesn't normally happen either.  

And so, thinking about the short-term and the long-term pictures,  I think it would have been better for her if she had leukemia.  MOST types of leukemia have a 98% cure rate.  It invades, it's treated, it goes away.  Most of the time it stays that way.  Arthritis is forever, breaking down joints, bone & cartilage  with every flare.  Making these kids suffer in ways that most adults don't have to.  Most of the time they don't complain, they just carry on.  Even when they hurt, they keep going as long as they can. 

No comments:

Post a Comment