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.

Over the river and through the woods to Children's we go...

We went back to Children's. Dr. H had a baby this week and he was out - so we met with Dr. J. I am so glad we made the decision to switch to Dr. H. His approach, his interaction with us and Bean....we made the right decision to switch. Don't get me wrong, Dr. J is a good Dr., he is patient...just nothing like Dr. H.

The plan. We increase Methotrexate by 50% to 15mg x1 per week. We went down on the prednisone to 15mg 1x per day. The rest stays the same.

We will see how it goes.

The memorable part of today's visit. Bean usually gets stickers after getting her blood draw in the lab. Because we went to Dr. C last week to draw blood, they didn't need to this week.

As we were leaving Bean asked to go to the lab - I almost laughed out loud...I had to explain to the nurses who looked at her, then looked at me, then looked at her again...that there was more behind the ask.

I said Bean, you don't have to have a blood draw to get a Hannah Montana sticker. Would you just like to ask Nurse Rachel for one? The smile slowly grew to include her entire face!!

My child was willing to get a blood draw, just to get a sticker. Talk about conditioning....talk about love for Hannah....talk about the ability to make my child happy with such a simple thing, I love her!

Dear Dr H - what is it about Sundays....

Happy Sunday Dr. H, I hope this note finds you well.

I'm not sure what it is about Sundays...but Bean had a fever again this evening - and was complaining about hip pain and had trouble walking. Though she walked a mile each way to and from synagogue yesterday - and played outside with a friend today.

This is the third Sunday in a row that she had a fever and pain. I'm not sure what it is about.

As long as it is like the other Sundays and nothing more comes of it - we will see you as planned on Thursday at 10:40 am. I just wanted you to know what was happening here.

All my best,
L

Dose 4

Bean's 4th dose came and went with little fan fair, just 4 extra pills on Friday night.

Dear Dr. H - Relapse Correspondence

Sunday - November 8th - 10am

Dear Mrs. S,

Does Bean have any additional symptoms other than the fever and joint pains? Runny nose, congestion, cough, vomiting, diarrhea, stomach pain? When you said she did not have her medicine before she fell asleep, are you referring to naproxen or prednisone? I am not sure about the origin of the back pain, is this a persistent pain?

I am concerned that this might represent early recurrence of the systemic JIA, particularly if there are no other symptoms suggesting an alternative, intercurrent illness, such as an upper respiratory infection or gastroenteritis. If the back pain is persistent, we have to investigate this further.

I would like to obtain lab tests, if possible, tomorrow (Monday) this will include a CBC with differential count, ESR, CRP, comprehensive metabolic profile, CK and ferritin. Please let me know where is the best place for you to get them drawn, either at CNMC or at an outside lab. I will be able to order those labs first thing in the morning.

In addition, I would like to re-evaluate Bean in the clinic this week, unless symptoms quickly and completely resolve. We will schedule an urgent follow-up appointment for you (M, can we schedule this tentatively? The earlier the better during the week). No change in medications at this time.

Best regards,
C H
----------------------------

Sunday - November 8th - 11:30am

Thank you for getting back to me, and so quickly!

Last night she had congestion - she blew her nose a few times and she felt better. She had no other symptoms. And this morning she is running around as if nothing is wrong.

The medicine she didn't have before bed was her (we gave it to her at 3am):
20mg of prednisone
15 mg of prevacid
1mg Folic Acid
250mg naproxen

Her back pain is not persistant. She said "it comes when I get tired - like when my legs hurt." Interperating a 5 year old is difficult - but what I take that to mean is when she is lethargic - her word is tired. She becomes lethargic and her back and legs hurt.

I can take her into our pediatrician first thing tomorrow (Monday) and ask the doctor to run the tests that you asked for. I will bring the e-mail - but his (Dr. C) phone is: and his fax is:

We can bring Bean in Tuesday or Thursday morning - just let us know what you have available - my cell number is:

Thanks so much,

L
-------------------------------

Monday - November 9th - 8:00am

OK, that sounds good I will contact Dr. C as well. I would suggest that Dr. C also takes a look at her to determine there is no obvious source of infection (ear infection, pneumonia etc.). Let’s just be on the careful side while we taper the steroids. The fever might have occurred because she missed a dose of prednisone. Actually, I was going suggest to you to have her take the prednisone in the morning rather than in the evening since this mimics the natural circadian rhythm of steroid secretion, and, also, it might cause less side effects on sleep and emotional lability.

Thanks!
CH

-------------------------------

Monday - November 9th - 12:00pm

Dr. H - we saw Dr. C this morning. Bean seemed fine. She hasn't had a fever since that one early sunday morning. She doesn't have an ear infection, no soar throat, we are waiting to get a urine sample. Dr. C did all the blood work that you asked for.

She went off to school.

Should we move her meds to the morning?

Thanks for your support,
L

-------------------------------

Monday - November 9th - 4:40pm

Mrs. S,

I was trying to call Dr. C’s office but could not get through (it does not give me the option to leave a message either). Is there another way for you to contact them and have the lab report faxed to: ?

Thanks!
CH

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.

And the fever returns...and the pain.

Did I speak to soon? At 3am Bean walked into our room...with 103.2 fever, back pain and knee pain. Is it related - is it something different?

Here is my e-mail to Dr. H.

Dear Dr. H,

Thank you for the e-mail and the follow up. The week went well. She started the naproxen on Monday - 2x per day. And here week went well.

Friday she had her 3rd dose of methotrexate and we dropped her dose of prednisone down to 20mg 1x day - from 25mg 1x day (as it had been 2 weeks).

10mg of methotrexate x1 per week
20mg of prednisone x1 per day
15 mg of prevacid x1 per day
1mg Folic Acid - x1 per day
250mg naproxen - x2 per day

Early this morning (Sunday morning) - 3am - Adina came into our room - saying her back hurt, and knees hurt. I picked her up as she couldn't climb into the bed and I realized she was quite hot. She had 103.2 fever. We realized that for the first time, she hadn't had her medicine before she fell asleep - we gave her the medicine and she went back to sleep.

I'm not sure if it was a coincidence that she got the fever and hadn't had her medicine - one dose.
If this is an unrelated illness...
What this back pain is about - lower back pain.

You had asked about the rash in your last e-mail. I'm not really sure if it has come back. It is SO hard to tell as her cheeks are often red from the steroids. And the phantom rash comes and goes so quickly. There have been a few times I think I have seen it, but I can't be 100% sure.

What are your thoughts?

Thanks so much,
L

Dose 3

Friday night Bean got her third dose of methotrexate. Again she didn't have any rection...I have to say handing my daughter 12 pills brings me to tears...it is too much.

But she didn't have any reaction. Life is good.

The Dr's Response...

Dear Mrs. S,

Thank you for the update.

Her numbers from the last bloodwork looked good (sorry, I thought you were called about them).

Specifically, her hemoglobin has improved from 9.9 to 12.0 (anemia has resolved), plt decreased from 580 to 436 (good), ESR went from 103 (extremely elevated) to 20 (normal), CRP went from 9.9 (very high) to 0.06 (very normal, normal < 1.0). The ESR and CRP are the best markers for inflammation, and they are normal now, this is very positive. Kidney, liver and muscle-related enzymes are all normal. Tests for Epstein-Barr and Cytomegalovirus were negative. These test results corroborate the impression from the last visit that her disease process was entirely controlled.

The symptoms you are describing (pain in legs, back and head) are unlikely to be symptoms of any of the medications in particular. Lethargy/feeling unwell is sometimes seen after methotrexate but I would expect it to occur immediately but not with a delay of 24-48 hrs.

Why do you think she has trouble sleeping? Is it because of pain? Or is it because of agitation? Sleep problems can occur with corticosteroids but I was hoping for this to improve with once daily dosing in the morning.

I agree with continuing the naproxen twice daily for now while she has these symptoms. Did she have any recurrence of fevers or rash? While we taper corticosteroids, a recurrence of her s-JIA is also always a consideration but I would think this is somewhat unlikely given that we have not tapered very much. I would suggest for the time being to continue with the current treatment plan. If these symptoms should persist or worsen, or if fevers/rash should recur, I would like to see her back in clinic earlier than the planned four weeks. I would also consider to get repeat blood tests earlier rather than in four weeks, so we can pick up any early signs of inflammation.

C H

Dear Dr. H - Dose 2

I composed this e-mail to Dr. H at Children's today.

I wanted to update you on how Bean is doing at see what her results were from her bloodwork from the last visit - what did her numbers look like?

We saw you on Thursday, October 22nd - up until that day she was taking:
25mg of prednisone x1 per day
250 mg of naproxen x2 per day
15 mg of prevacid x1 per day

Bean had her first dose of methotrexate on Friday, October 23rd - she had no reactions that we could tell.
we stopped the naproxen as a trial.
For the past week she has been on -
10mg of methotrexate x1 per week
25mg of prednisone x1 per day
15 mg of prevacid x1 per day
1mg Folic Acid - x1 per day

Bean had her second does eof methotrexate on Friday, October 30th - she had no reactions that we could tell.

However - today, November 1st, she was quite lethargic and tired, she complained of pain in her legs then back and head. Do you think these are these side effects of any of the medication she is on?
I think tomorrow we will start back with the naproxen x2 per day and see how she does. But I was concerned with the pain symptoms she is having.

Also- she has been having trouble sleeping. I think she has only slept through the night 3 times in the past two weeks. Could that be a side effect of the medication?

Thanks for all you help and support,

L & J

Dose one

Bean got her first Dose of Methotrexate last night. We noticed NO side effects today. She had a friend come over and play, which was a great distraction...but really, I don't think she noticed anything bothering her. I am hopeful that it stays this way - though I have no expectations. The medicine builds on itself.

First dose - went off without a hitch....so far.

A plan.

We took a trip to Children's today with Bean.

Bean has been on Naproxen(Alieve):

Nonsteroidal anti-inflammatory drugs (NSAIDs) — These medicines provide pain relief and reduce swelling. Some are available over the counter and others require a prescription. Examples include ibuprofen and naproxen. These medicines can cause nausea and stomach upset in some people and need to be taken with food.

She has also - for the past two weeks been on oral Prednisone:

Corticosteroids (steroids) — In patients with oligoarthritis or in patients with very painful/swollen joints with other types of JIA, these medications are very effective when given as an injection (shot) into the affected joint. In younger children or if several joints are injected, sedation is often used. In patients with more severe widespread disease, these medications occasionally need to be given by mouth as a pill. These medicines when given by mouth are effective, but can have serious side effects—including weakened bones —especially when used for long periods. Doctors generally try to avoid using steroids in children because they can interfere with a child’s normal growth.

Obviously this was a stop gap measure - we needed a plan.

Dr. H laid out three options - this is the information as I remember it....with some internet based research in between...

1. Methotrexate - a disease modifying drug - has 20 years of research.
20 % of children get side effects - stomach/abdominal pain 12-24 hours after taking the medicine, mouth ulcers -- some say hair thinning/loss.
Suppresses production of blood cells.
Need to take folic acid.
Long term possible liver injury.
Need Serial blood testing to check on liver - first every month...hopefully every 2 months after that.
2 ways to administer - oral (pill) or injectable - once a week.
Effective in 30-40% of SJIR kids

Disease-modifying anti-rheumatic drugs (DMARDs) — These medications work by changing, or modifying, the actual disease process in arthritis. The aim of DMARDs is to prevent bone and joint destruction by suppressing the immune system’s attack on the joints. Methotrexate is the DMARD most often used to treat JIA. Other medications used include sulfasalazine and leflunomide.

2. Enbrel - biological modifying drug - has 10 years of research.
It works by blocking the protein molecule TNF and its function (a marker in SJIA patients)
Side effects include decrease in immune system - especially fighting tuberculosis
Administered by injection once a week
Effective in 50% of SJIR kids

3. Kineret - a newer biological modifying drug - it is newer.
It works on blocking the protein molecule IL1
Side effects include decreased in immune system -- especially for severe infections like bacterial infection, pneumonia, sinusitis
Administered by injection daily...though it burns at the injection site
Effective in 80-90% of SJIR kids

Biological modifying agents — Biological agents are medications that directly target molecules or proteins in the immune system responsible for causing the inflammation. They are used to treat children with more severe arthritis that is not responsive to other medications and are given by injection or by infusion. Etanercept (Enbrel), infliximab (Remicade), adalimumab (Humira) and anakinra (Kineret) are examples of this type of medication.

Also - one we know but didn't talk about yet:

Physical and occupational therapy - Exercise, physical and occupational therapy can help reduce pain, maintain muscle tone, improve mobility (ability to move) and prevent permanent handicaps. In some cases, splints or braces also may be used to help protect the joints as the child grows. Special accommodations with schools may be needed to adjust for children with limitations from their arthritis. The Americans with Disabilities Act (“504” plan) can help facilitate these issues.

After much discussion....we chose the first. Methotrexate.
It is the one that is least invasive and the most tested...when there are no good decision...ya have to pick the lesser of the evils.

Bean gets her first dose tomorrow night.

FOOD!!!

The steroids are doing crazy things to my little girl. Food, the quantity of food is amazing! Between the 4:15 pm, when she arrived home from school, and 6:35 pm when I put her to bed she consumed:

Tortilla Chips
Craisins

Break in time....
12 chicken nuggets
Peppers

Break in time....
Pineapple
Sunflower butter sandwich

Break in time....
Quesadia

How can you tell a child who is truly hungry they can't eat? I just slow her down....we talk about it being part of her medicine...but still. Looking forward to a day when she is off the steroids!

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!!