To talk or not to talk

One thing we have struggled with is when to discuss different aspects of this nasty disease with Emily.  She's only five years old so it can be a lot to take in that young, but when you have to spend 57 days in the hospital and hear announcements like "code blue room xyz" topics like life and death tend to come up.

At one point Emily decided she would not take any more medicine.  We were still in the hospital and she just decided she was done.  It was at that point we had to sit down and explain the very nature of her arthritis and how the medicine was saving her life.  20 years ago MAS killed and doctors couldn't explain it.  30 years ago SOJIA was called Rheumatic fever and baby Aspirin was the only regimen.  Those children who didn't lose the battle then grew up in wheel chairs and I've watched as some of them have lost their battles as adults.

We found because this was happening to Emily she needed to talk about it.  She needed to understand it was a very serious matter and so it helped put things in perspective for her.  She still doesn't understand it all, but when she introduces herself to another child she will say, "I'm Emily and I have arthritis." Because it is a part of her life now and will be forever.  She is learning to live with it and live around it.

Because talking helps we look for ways to encourage that conversation.  Emily has a drawer filled with medical play items and she enjoys watching Doc McStuffins, a Disney show about a child playing doctor.  We've found it especially helps to talk about other children going through similar experiences.  We met a dog the other day with only three legs and so I made a point to talk to her about how brave the dog must have been to go into the dog hospital and how he had to get shots and IVs too.

Since we may have to re-introduce nightly injections the point of talking about it has come up once again in our lives.  Emily is not happy about the possibility, but she knows it may happen.  We talk about it once and then don't worry about it.  She knows and if it happens we will revisit the discussion and importance of them.  We will also have to explain why the infusions didn't work which will be harder, but we'll figure it out.

Lately Emily has been mimicking Doc McStuffins and even has a door sign to show when she "doc" is in or out.  I think Emily would make an amazing doctor someday, she would be able to relate to her patients and I even told her perhaps she could be the one to cure arthritis in children.

The "doc" is in:

The "doc" is out:

When the child is living the trauma talking is very important.

Doctor

There aren't enough Rheumatologists to treat the 300,000 children in the U.S. There are only about 220, according to the Arthritis Foundation.  Emily's case is pretty sever so we didn't experience the normal 6 month wait to confirm a diagnosis and we've always had priority at the office, but most of these children wait 6 months or more.  I couldn't imagine.  We first heard Still's Disease two weeks into her first hospitalization.  I believe that doctor saved Emily's life because by the time we got her into the children's hospital she was in heart failure and taking a turn for the worse quickly.

Whenever Emily goes to the Rheumatologist it is not a quick, easy visit.  The time with the doctor is quick and usually only takes about 20 minutes, but we wait and wait and wait.  This last time we waited 1.5 hours in the exam room (which is torture for small children) because her normal doctor is on maternity leave.  By the time we saw the doctor for our 10:30 a.m. appointment it was noon, we were all hungry and Emily had drawn all over the entire exam table paper twice.  Our two year old had finally slid his way over the entire room floor (picking up who knows what type of germs) and was considering drawing on the actual exam table with a pen (ok, I may have allow him to draw a line or two, but hey don't shut us in a 10x10 room for that long with a two year old).

We are lucky, some children have to drive hours to see a doctor and some have no choice but to see an adult Rheumatologist and that's difficult because the adult diseases are different from the children's.

We need more pediatric Rheumatologists. 

Rheumatologist

We had our bi-weekly visit with Emily's Rheumatologist and my suspicions were right.  She's been more tired then usual and I had a feeling as we were weaning down on the steroids the disease was becoming more active and labs confirmed this.

There are two different inflammation markers the doctor looks at; one tells if MAS is coming back and one tells us about SOJIA and how active the disease itself is. 

It's now I have to remind myself there is no cure only control through medication.  The medication she is on right now is Prednisone, Cyclosporine, and Kineret.  Kineret is the long term medicine that is supposed to control everything and eventual be the only medicine she takes.  Prednisone is like a band aid medicine, it's what she got in the hospital because she was so very sick.  Prednisone reduced the inflammation quickly and controls it very well, but the side effects are pretty nasty (think best case two hip replacements eventually) and can be life threatening if taken too long so we have to get her off it as quickly as possible. 

We found out yesterday her D-dimer, white blood cell count and platelet counts were all elevated which means SOJIA is still with her and becoming more active as we wean down on the steroids.  Good news was her feritin levels were low, not normal, but not 24,000.  Normal is around 60, Emily's now is a little above 100.  In the hospital with MAS they were 24,000.  So anyways MAS is under control; SOJIA not so much.

I'm worried it's not working as well as we wanted it to, but happy we can try a different medicine.  This one is given via IV in the hospital twice a month.  They can also do labs at the same time so if the IV goes in well that means just two pokes a month...hallelujah!  There is a huge emotional toll that is being paid and if we can reduce that cost just a bit I will take it.  Let's just hope this new medicine works.

Doctors

Before SoJIA a doctor appointment with a sick child was a relief.  I would leave with a prescription and knowledge about why my child felt ill.

Now we spend four times as long with the doctor every two weeks and I leave feeling hopeless.  Emily doesn't feel well, she's not herself and I am sure the medicine is partly to blame, but all the doctor can tell us is she doesn't know why she is feeling that way.  Her feritin is "jumping" around according to the doctor so while we may (if she gives me a call this afternoon) be able to slowly start dropping the steroid dosage Emily will always be taking Cyclosporene and Kineret.  I knew before about the Kineret, but to find out about the Cyclosporene was a bit depressing. 

I do hope we can start going down on the steroids today.  It will still be baby steps, reducing one milliliter every two weeks.  Sigh.  Emily takes two five milliliter doses a day, that's 20 weeks until we are off them completely.  I hate the steroids the most.  Sure they saved her life, but at the same time I feel like they are transforming her.  She rides in a stroller a lot and we are getting stares more and more because her face has ballooned out.  I can almost hear the judgmental thoughts behinds those stares.  I've got my new pamphlet printed out though and just need to remember to bring it along and hand it out.  After all, May is arthritis awareness month.