So many ways...

This disease has changed Emily's life in so many ways.  This morning my husband made the mistake of using the word skinny and Emily began feeling bad that she was not skinny, like she used to be.  She means she doesn't look the same anymore; the steroids cause puffiness which gives Emily the appearance of being overweight.  In fact when she take her anywhere and she rides in the stroller, like the zoo yesterday, people will often stare at her and I feel like they are probably thinking something like "Why is the child in a stroller, she should be walking" or perhaps the term "childhood obesity" goes through their mind.

Even with the weight gain Emily is only in the 30% for height and weight so she is not overweight at all and she is very active, but until we shake the steroids completely we won't be able to shake the puffiness.  Even once we do get off steroids we won't be able to shake this nasty disease.  It's here to stay and there are so many things it impacts.  Going somewhere like Disney Land, that would be a monumental task because steroids + exhaustion + stiffness and pain from an all day adventure = super tantrum or at the very least extreme whininess. 

We have to be careful what we say; we don't talk about weight (or try not to) instead we talk about getting plenty of exercise and eating healthy.  We don't talk about Emily having children, instead we are sure she understands that adoption is a great way to become a mother too and we encourage her to learn and say maybe you could be a doctor or a scientist someday.  Someday we will have to explain to her that these new medicines could make is hard for her to have children.  I imagine when she marries that will be a scary point for her, when her husband and her decide to try she will wonder...

Dentist visits are another thing we learned yesterday.  Cavities are common with this children because they are immune suppressed and cavities are caused by bacteria.  The medicines can also reduce saliva in the mouth and make her teeth and enamel more susceptible to decay.  Now she has a cavity to fill and I don't want to put her through that, but we have to because cavities are because by bacteria and we need to get rid of the bacteria.

We talk about finding the good out of the bad.  I'm afraid I have yet to find the good out of this nasty disease, but I still have my sweet girl and if this had been five or ten years ago I would not and so I can at least say treatments have made progress.  I wish more people knew what this did to these sweet children.  I read a post in a Facebook group about a SOJIA child who tried to commit suicide.  These children are in pain all the time.  It breaks my heart to think what that mother is going through right now.  We've just got to keep showing Emily all the good in life to help the puffy badness of SOJIA maybe not seem quite so bad.  I hope we can do that as she gets older.

This is our story in a nutshell.

I'm a member of three great groups where we talk about SOJIA and JIA, mostly other parents, but these support groups have been my lifesaver.  No one can understand what it's like unless you live through it or watch your baby live through it.  So when one group asked me re-introduce ourselves I wrote this below and thought I would share it.  This is our story in a nutshell.

Hi friends. My daughter was diagnosed almost one year ago with systemic JIA, March 7th, 2012 to be exact. Her onset was atypical, no joint involvement just fever, rash, pericardial and pleural infusions and a bad case of Macrophage Activation Syndrome (MAS). She spent 57 days in two different hospitals getting this nasty disease under control and came down with it while I was on deployment (I'm in the Navy) so that last time I saw her pre-SOJIA was over a year ago. I mention that because this seems surreal to me. We make a family photo calendar and the photos from last December where those we took pre-SOJIA and it seems unreal to me that this has all happened. The doctors originally thought it could be leukemia and then she got very bad very quickly so they flew me home and I spent every day with her in the hospital except for the first week. Since her onset we've seen lots of joint involvement, although the inflammation comes and goes quickly. She has constant flares that last between a day to a week or more, and although the fevers are gone the rash is her constant companion. Emily hates the steroid puffiness and her infusions, but she's a trooper and no longer even cries when she gets her IV. She's on bi-weekly Actemra infusions, cyclosporine, prednisone and prevacid. We've finally got her down to only 3ml of prednisone once a day, but her Rheumatologist thinks she may be on steroids for a long time. She was off them for two weeks about three months ago and had a flare and MAS return so we are taking things very slow now. I hate this disease and have made it a life goal to inform everyone I can how nasty it is and to spread the truth about it; it's not just arthritis and it's not the same at RA or osteoarthritis and it has and can kill children. Oh and my pet peeves are anyone who suggests this can be cured with probiotics or tells me their grandma had the same thing in her old age.

I say it's a nasty disease, but today Emily is happy and active and six months ago she was depressed and could barely walk so there is lots of hope for a great future.

The morning after

Since we have talked about infusions a lot and I try to let everyone know when infusion Monday is I thought it would be important to mention the day after, more importantly the morning after.

Infusions have become routine.  Emily only says "ow" during the IV stick and she rarely cries.  So I can say without a doubt she is a tough cookie.  It's the morning after that causes us grief.  All these pokes and pricks and medicines can take an emotional toll on everyone, but especially Emily.  She is not herself a lot of the time thanks to Prednisone and infusions can really drain her physically and emotionally.  The morning after an infusion is where we see it.  She doesn't want to get up, she doesn't want to get dressed, eat, take her medicine or go to school.  She just wants to lie in bed.  I can't say I blame her.  We are suppressing her immune system, pulling her out of school early, imprisoning her in a hospital bed for hours and then have the nerve to make her go to bed a couple hours after we get home. 

I will blame this all on adjustment.  It's not an easy thing to adjust to, her body needs the infusions because by Friday night she'll have a rash again and soon after joints will hurt and it will be hard to get up in the morning because she is stiff and feeling yucky.  The problem is we get used to getting better after going to the doctor.  We take some antibiotics or other form of medicine for two weeks, sometimes go in for a follow up and then we recover and get on with our lives.

I think this is when not having a cure really hits home.  Emily wants to know when she will be done with infusions and medicine and I have no answer for her.  I wish I could say there were only two more, but it's a steep climb for her.  Some children respond immediately and are off steroids and doing fewer infusions and some, like Emily, still have elevated white blood cell counts and rashes and joint pain and there doesn't seem to be a light showing an end to the SOJIA tunnel. 

Tomorrow morning things will be back to normal and we'll be assuring Emily she doesn't have another infusion for another week.  Then infusion Monday arrives and we start all over again.

These children deal with a lot.  We are blessed though; Emily is in school (most infusion center children are not able to go to school because they are on lots of chemotherapy and dealing with cancer), Emily is happy and relatively healthy.  It could be better, but it could be a lot worse.  For now I am happy to keep the status quo because I fear if I start longing for better I may actually get the worse and four hours every other Monday is no problem when compared to being a patient in the hospital or having an empty bed with no six year old to wake up grumpy in. 

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. 

Life Definitions

Health

This morning on the way to work I heard a commercial about staying healthy and I found myself wondering if anyone really wants to be unhealthy.  I mean health is defined by merriam-webster as "the condition of being sound in body, mind, or spirit; especially: freedom from physical disease or pain" so when I hear the children shows on TV telling kids what to do to be healthy I feel a little pain.  By the definition above our Emily is not healthy.  She is happy and active and alive, but not free from disease and pain. Emily did not choose this nasty disease.  Somehow it found us through genes or virus or a combination of the two or perhaps something entirely unknown (idiopathic means of unknown cause) so we don't talk about being healthy so much as we talk about doing good things for our body and helping us feel good even with arthritis.  We have a new job as parents; we have to teach Emily how to live with this her whole life. 

Motherhood

Someday she will need to understand the medicine she is taking to control her arthritis may keep her from having children of her own, she will need to understand and make peace with that. So being a mother will have a different meaning for her.

Life

Life does have a different meaning know.  Little things don't bother me so much because life means we still have our sweet daughter to hold.  This disease has killed before and the possibility is always present in our minds; so life has a new meaning.

So many new meanings; so many ways to say something and look at life.

Happiness

After Emily was discharged from the hospital she was sad.  She couldn't do things she could before she was sick.  She was so weak walking was difficult and she couldn't jump or run at all.  I was so worried she would hate life.

I remember overhearing someone say they would commit suicide if they got fat and lost their hair due to another similar disease and all I could think about was what if that is how Emily feels.

I am very happy to report she has been her happy self since I got home from my last work trip.  She is running, smiling, laughing, jumping and playing like she used to.  She might be a little slower and not as strong as before, but she doesn't seem to even notice it any more. 

As a parent I decided if I could teach my children to love life I would have done a good job.  It's still my hope that in spite of infusions and doctor visits and steroids and arthritis Emily will love life and find joy.