Sometimes reading can be a positive...Methotrexate, Mortality & Pain

Three of the latest articles I'm reading...you can find the links to all the articles I am reading at the side of the blog in: Articles I'm Reading
Click on the title of the article to read it in it's entirety. I'm pulling out pieces of note to me, but they are not the full article.
Kids in Remission May Be Able to Stop Meds Sooner
Deaths Due to Childhood Arthritis Drop
The Importance of Pain Management
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Bean's Journiversary...

I have been remembering last year's 22 days of sheer panic. The anniversary of this journey, to take a conjunction from a dear friend, our journiversary.

Each day that passes without a fever... I am thankful,
each day that passes without complaints of pain... I am thankful,
each day that passes with less complaints of stiffness... I am thankful.

I am thankful that we started this journey a year ago and not this year...
we have gotten two blizzards in the past week, the first 30 inches, and the second 2o inches...with 50 inches of snow on the ground this week I can't imagine what we would have done last year...spending those days in the hospital and not able to switch off with J, having one of us snowed in with the other 3 children.

Did I mention H1N1...last year no one knew of such a thing...this year our three other children would not have been allowed to visit Bean in the hospital...I don't know how any of us would have handled that one either.

See for your self below...

Children's hospital didn't have any Internet access and cell reception wasn't great either. So our time there was spent updating friends and relatives through facebook postings from my blackberry and a photo journal of pictures taken on that same blackberry. Some were posted to facebook...but most weren't "post worthy." I am now rethinking that and want to make sure I remember.

Bean's first night in the hospital...she did smile and she did have a rash.

View from of our room

"Dr. Will" - Bean's resident - playing Zingo with Bean - God bless Dr. Will wherever he is now, he truely made a difference during our stay

Bean's Room and set up - signs from friends at school, art supplies from the volunteers at the hospital, and a "ducky attention diverter" from B and family!

Grammy came and took some much needed naps together (especially so J and I could go home to the 3 other kids one night together).

One of two pairs of PJ's I will always associate with her stay...a picture of her knees that didn't "work"...a picture of my 5 year old who couldn't walk....in a stroller

Bean willed herself to sleep during her bone scan so she wouldn't need another "needle!"

The fantastic social worker that helped Bean and her fellow patient deal with all the procedures they were undergoing...and all the blood draws!

Playroom right next door to Bean's room where we spent lots of time.

Even when Bean had a fever and was too week to participate Belle, Bob and Puppy got to be close by - but loved the day the dogs came!

The playroom that Bean, when she was up to it, was able to have a regular playdate with her brothers and sister!

Bean's eye on her stay :
(including the other PJ's that I will never forget)

Bean and the family right after she had her bone marrow test to make sure she did not have leukemia...waiting for results....and then 2 hours later...

The sun set outside Bean's room as we packed up to leave....together, as a family.

Ironic - the sign as we were leaving....

I'm thankful that this journey started last year and not this. I hope the next anniversary is one that brings us closer to a cure.

Making it real.

After all the research and googling I have been doing a friend's mom C mentioned the Arthritis Foundation. She mentioned a walk, information, research...I kept hearing her talk, but my mind was going...

The first thought that popped into my head?

Arthritis Foundation...that isn't my thing...

See, for the past 7 years our family has focused our time and fundraising "walks" on the Race for Hope J's dad died October 9, 2002 from a brain tumor.

Bean was named for him.
She was born on my in-laws' anniversary.
A bright light after the sad loss of a 57 year old gentle and kind man.
Brain cancer is our cause...Arthritis...

If I make the call, does that make the journey real? Do I make that call?
I don't know if I'm ready for that.

Well, I didn't have to be ready, with my permission, my friend's mom - C - the wife of a Rheumatologist, made the connection for me and had the Arthritis Foundation contact me.

They asked if I wanted to get involved...if I wanted a welcome package. I heard the words come out of my mouth "yes."

But it wasn't really what I wanted to say. I wanted to say, no - I don't want a welcome package, I don't want to get involved...I don't want my daughter to have arthritis...I mean, if I do say yes it means this is real, that my baby has arthritis...that she is on a chemo drug...that she has pain...that she has to deal with this and not just get better while we wait it out.

But, I said yes.

Today my package came - Thank you C for helping me take this first step:

When reading is not a good idea...

My daily Google alert led me to this article. Do I understand it? Maybe. What does it mean? I have no idea.

But it is education, and a direction to research farther. (SOJIA stands for Systemic onset juvenile idiopathic arthritis)

The section below is in plainish English and might be helpful for understanding the disease. Bolding the parts that got my eye.

SOJIA represents about 10% of all the cases of JIA. The course and prognosis of SOJIA is heterogeneous, as fifty per cent of patients have a monophasic course with resolution of the symptoms, while the remaining fifty per cent develop a chronicrelapsing and remitting course and a very severe form of polyarticular chronic arthritis. Patients with SOJIA also display an increased risk of developing hemophagocytic syndrome, a potentially fatal complication (Cassidy, J T and Ross, E. 2001. Textbook of Pediatric Rheumatology, 4th ed, Books on Demand Publishers, Visby, Sweden; p. 218-321).

Children with SOJIA present with severe systemic symptoms (fever and rash) that usually precede the development of arthritis for weeks to years. The high spiking fever, which is the hallmark of this disease, usually follows a quotidian patternwith 1-2 spikes/day. Patients look characteristically well when the fever is not present, but become quite ill with the spikes. In many patients, the fever is accompanied by a salmon-pink rash that becomes more apparent with the fever. Additionally,children with SOJIA may have hepatosplenomegaly, lymphadenopathy, pericarditis and other manifestations of serositis. These systemic manifestations may last from weeks to months and eventually tend to subside to be followed by the development of chronicarthritis. About 50% of patients will present oligoarticular involvement and will eventually recover. The other half will evolve into a polyarticular pattern, the prognosis of which correlates with the number of joints involved six months into thedisease course. Up to 48% of children with SOJIA will have active arthritis ten years after the diagnosis is made (Cassidy, J T and Ross, E. 2001. Textbook of Pediatric Rheumatology, 4th ed, Books on Demand Publishers, Visby, Sweden; p. 218-321; andLomater C, et al., 2000. J Rheumatol 27:491-496).

There are no available specific tests to establish the diagnosis of SOJIA, nor are there known prognostic indicators to ascertain its clinical course. Fever, anemia, leukocytosis and elevated erythrocyte sedimentation rate (ESR) are the maininitial features of the disease, sometimes lasting several months before the diagnosis can be established. As these symptoms are nonspecific and can mimic infections, malignancies, and other diseases, patients undergo a series of very costly diagnostictests and prolonged hospitalizations.


One of the most serious complications in patients with SOJIA is the development of hemophagocytic syndrome, also known as macrophage activation syndrome (MAS) (Cassidy, J T and Ross, E. 2001. Textbook of Pediatric Rheumatology, 4th ed, Books onDemand Publishers, Visby, Sweden; p. 218-321). The hemophagocytic syndrome, which can occur as well in the context of infectious and neoplastic diseases, is associated with serious morbidity and/or death. Its etiology, especially in the context ofSOJIA, is unknown. Familial cases of MAS occur as the result of defective viral killing due to mutations in genes like perforin (involved in the release of granzyme by cytotoxic T cells/natural killer cells to target cells) or Rab27 (involved in thecontrol of granzyme vesicle degranulation).

Therefore, SOJIA remains a chronic inflammatory disease of unknown etiology for which a specific treatment has not been determined. Despite extensive study, multi-drug treatment of patients with SOJIA is similar to that of oligoarthritis andpolyarthritis, which depends on the phase (systemic phase versus arthritic phase) of the disease and on the extent of involvement. While a minority of patients does well with non-steroidal anti-inflammatory drugs (NSAIDs), most children require the useof oral and/or parenteral steroids as well as methotrexate to control the symptoms. Intravenous immunoglobulin (IVIG) has been used in recalcitrant cases. Most recently, anti-TNF therapy (e.g., etanercept and infliximab) is being added to theseregimens. Nevertheless, there are some limitations and risks associated with these drugs. For example, long term treatment of these patients with immunosuppressive drugs such as methotrexate and steroids have been reportedly associated with an impairedability of the patients to eliminate viral infections. Intravenous immunoglobulin treatment is accompanied by the risks associated with transfusions of blood-derived products. The present inventors have found that SOJIA patients do not respond as welland require higher doses of anti-TNF agents to control the symptoms than any other type of JIA. Therefore, there is a continuing need to identify effective drug treatments for SOJIA disease.

So far so good Sunday and a little bit of research.

It is Sunday night. Don't want to give a kinahurra....but it is almost 10pm Sunday night and Bean didn't have a ferver. Just taking note.

Google search alert came up with a report today. Dr. C. told me not to do my own research...but I feel like I need to know and need to learn...all these reports talk about the mortality rates of kids with SJIA - it isn't something we have brought up with the doctors yet, or have they brought up with us. But it is on my radar.

Here is the latest report.

One month out...

One month ago today Bean was diagnosed.

I keep praying that this illness is like labor, the pain keeps coming - you don't know when - but at somepont it ends and you couldn't be happier.

I keep praying that the medicine she is on will be all she needs and it won't come back.

I keep praying that she is in the 1/3 of children who outgrow this.

I keep praying for the strength to be her support and not for her to see me crumble.

I keep praying for knowledge and strength so that J and I can make the best decisions we know how.

I keep praying. May god watch over and protect my baby.

Superficial

I gave Bean a shower tonight...the effects of the steroids are evident.

Her belly…the one that I have always been amazed is so flat – was distended… firm – looked like it belonged on a different body.

I washed her beautiful dark brown curly locks…and my tears started to roll down my cheeks…the thought of her thick beautiful hair, the hair that at four years old she donated 11 inches of to locks of love….the thought of her thick beautiful hair thinning…falling out…is overwhelming to me.

It is so superficial all of it – I feel so guilty. We are so lucky that what she has is life altering and not life ending….I keep telling myself that. I keep telling others around me, in the hopes that at some point my heart will believe the words coming out of my mouth.

My wish for her – may she not suffer pain anymore, may she be one of the 1/3 of children who outgrow SJIR… may her self esteem prove as strong willed as she is….

Education

It is time for me to educate myself about Systemic Juvenile Idopathic Arthritis...What do I know, what can I handle, how much I don't know or where to look.

A quote from a pharmaceutical sponsored website:
Children get JIA, but families live with it

JIA is not just a child's disease. For every kid with JIA, there's a family living right alongside—helping that child manage the pain and stiffness. Being the parent of a child with JIA can be an overwhelming responsibility. You have to make decisions that affect your child's life. A better understanding of JIA and its symptoms will help bring you up-to-date on your child's treatment options.

Right now - all I would like to do is be sad, I'm not ready to mobilize yet. I see/read of all these families the mobilize around their sick child and pull it together and do the extraordinary...BUT I HAVE FOUR CHILDREN! It isn't fair...but as Scar said..."life's not fair"

How it started...

Bean has been sick...should I go back to December 2008. A co-worker mentioned that my kids don't get sick...that I never seem to take a sick day because of my kids...talk about a kinahurra. First was Bean on December 30th - 102.5 fever and croup! By New Years Eve we had sick children...4 sick children!









So they recovered...in time for Bean's birthday - she turned 5 on January 5th!

And MLK weekend happened...it was supposed to have her birthday party the morning of the 18th. We had spent a lovely Shabbat at the M&M R's and a lovely lunch at L&M R's - Sunday we got up to make a cake for the party. 30 minutes into it Bean started vomiting - she perched herself on the couch - and we had to postpone her party...we decided to wait two weeks, and re-schedule it for February 1st. Bob got the bug on the 19th - and was able to stay home at watch the inauguration on the 20th...Puppy got it on Tuesday the 20th - Bean was back to school that day...and on the 21st Belle got it!!

On the 22nd - Bean got 103.5 fever. She said everything hurts. We thought she got the flu! It was the first time in my children's lives that I missed getting them their flu shot. We took her to the ped - wasn't strep. In-laws were here - we tried to do the normal stuff - motrin made a trip to the Silver Diner and Air and Space museum doable.

January 28th we took Bean back to the ped - for more blood work. My best friend had her baby on the 31st and we planned to go to NJ for the bris. February 3rd we were back at ped for more bloodwork...she STILL had a fever!

On February 4th - Bob's birthday I took Bean with me to get candles for Bob's birthday at school - walking out of the store Bean fell to the ground, her legs fell out from underneath her...she couldn't walk.

That afternoon we got the call to have her admitted to Children's Hospital (during a terrible meeting at work - different story line). We chose to go to Silver Diner with the kids for Bob's birthday, Bean was doing ok - and it seemed unfair to Bob's to not celebrate after we went to have her admitted... Mom came down to help.

I spent the first night with Bean in the hospital. Early morning blood draw - beeping machine of the baby next to us. On February 5th Bean had a chest xray, and two separate parts of a bone scan. The bone scan without sedation...first kid in the hospital to do so!!

We were hoping it was "just pneumonia!"

I spent the second night with her at the hospital. I got no sleep that night, but she did. Grammy came to visit at the hospital, and I tried to get a shower...they were broken!!

J brought the kids down to see Bean before Shabbat. They got to spend some time together before mom took them home. I stayed for the optomatrist visit. And then went home to sleep while J stayed with her. We missed Baby B's bris...sad and overwhelming on many levels.

It was Saturday, February 7th and Bean walked for the first time in days!! I stayed with her that night.

Grammy came down to be with her on Sunday. And slept over. It was the first time in a while J and I got to be with the other kids together.

Monday the 8th Bean had an MRI....and Grammy had to go back to NY.

Tuesday, February 9th Infectious Disease says they are done with her - on to Reumatology!

Wednesday, February 10th J brought all the kids for dinner at the hosptal. Bean was feeling a bit better. It was nice to be together!!

By Ferbruary 11th we were giving been 5 doses of oral liquid meds a day. She was hurling thing and going crazy. Not the easiest of patients.

And then we had to cancel our trip to California for cousin R's bat mitzvah. I wrote that day: "overwhelmed by the irony missing last year's pres weekend flight for snow- and this year for fever (thought it is 65)...this weekend doesn't have good luck!"

That day Bean said she was done watching tv! Ever heard of such a thing?!?

Belle lost her first tooth....Bob was her tooth fairy! And I missed the kids Cubs Club performances.

On February 12th I took Bean for her last invasive test. The bone marrow test. We needed to rule out the worst. The kids all came straight from school and played with Bean in the playroom. And then the news came....it was NOT Leukemia!! We were all going home. No diagnosis...but a plan.

February 26th we took Bean for a follow up at Children's with Dr. J. We were able to start tapering off her prednisone.

May 7th J took Bean back to Children's with Dr. J. He reported back: "Best...Dr.Appt...EVER!! I posted on Facebook:

It looks like we might be ARTHRITIS FREE!! She is off meds and we don't need to go back unless symptoms come back!! "Best Dr. Appt EVER is right!"

June 4th Bean complained of her knees "not working."

June 16th Bean "graduated" from pre-school. I posted on facebook: "got teary eyed at Bean's graduation...watching her move and shake while dancing...4 months ago I wasn't sure she would move like this again...thank god for miracles!"

August 4th Bean had 104 fever - I took her into Dr. C - he said we were looking for

"good news like a UTI or ear infection!"

Neither were true, no other symptoms - looked like a repeat fever with unknown origin. It left as fast as it came.

September 21st 1:30am - we returned from a trip to NY, Bean could not climb into her bed. She had a 102 fever, couldn't walk so well. She stayed home the next day with a swollen left knee, & fever. We took her in for blood work on the 22nd, we put her on Naproxen.

September 24th Bean returned to school. The school agreed to take her back even with her fever, knowing that she wasn't contagious. But not only did she have a fever, and a swollen knee - her fingers hurt and 6 of them were swollen. They didn't even look like they belonged on her hands.

October 5th Bean continued to have a fever of 100.2 she was down 4lbs, fingers & knees are hurting...sed rate is up to 80... we were STILL praying the naproxen to starts to work & we could avoid steroids.

October 6th Bean dressed herself and skipped out the door on her own, for the first time in 3 weeks. I posted on facebook:

"There may be a light towards the end of this tunnel...hope the next tunnel is FAR FAR AWAY!"

October 8th I took Bean back to Children's for more blood work and to be seen by the new rheumatologist, Dr. H.

October 9th Dr. H called to report back on the findings, give us a diagnosis and outline for me the next steps. I posted on facebook:

We got a diagnosis. Systemic juvenile idiopathic arthritis. We pray that Bean is one of the 1/3 of children who will outgrow this debilitating disease....and for the strength to make the best decisions as far as medicine to treat her condition...and for the strength to tolerate her behavior on steroids!!