Mommy, my fingers hurt.

The words I have been dreading to hear for years.

"Mommy, my fingers hurt."

Not sure if they hurt for a reason, or hurt because it was cold or because SJIA is around the bend.

I cheerfully said grab a pair of gloves and it might help. I didn't say anything to J or anyone...but I did take a picture. Those joints on her left hand look a bit big to me on those beautiful slender and long fingers.

Maybe if I don't say it out loud it will be nothing...

Procrastination...

I sat down to write this post on January 14th, but I was afraid to put into words what I was feeling. On that Thursday, Bean and I took what had become our monthly trip to Children's, we are SO lucky that it is less than an hour away. See, we started off going in once every two weeks, then for 4 months it was every four weeks.

Bean has started really liking our trips to Children's. For one - we have taken away the need for the blood-draws there - as we have been doing them at Dr. C's and 2nd - the ART ROOM! A magnificent place for children who are staying in the hospital or in for outpatient clinics to spend time and not focus on the scary - but to focus on what the beauty of creating a masterpiece.

Each time we are at Children's and wait for our turn to see Dr. H - Bean gets to create - to be a part of something so beautiful. It is during these visits that we get the opportunity to meet other patients and their parents.

Bean and I talk about medicine side effects, hair loss, vomiting, loss of limb, looking different...acknowledge people's differences and similarities - but it is also a real opportunity for us to be reminded what we have to be thankful for.

This particular day another girl, about 15 was there with her mom and brother - they had come in from a ways away. We spent about an hour on and off with them in the art room. They're a lot of parts to our routine there.

1. Sign in at main desk, write down list of all medications child is on - and why
2. Go to art room and wait....this could be 10 minutes...could be an hour
3. Called back by tech to take vitals - weight, height, temperature & blood pressure
4. Go to the art room and wait ...this could be 10 minutes...could be an hour
5. Called back to room to wait for Dr. H...could be 10 minutes, could be 45 minutes
6. Physical exam - conversation and plan
7. Go to the art room and wait - for either blood draw, or prescriptions...could be 5 minutes, could be 30
8. Set up appointment to come back

Bean and I were on step 4 when this young ladies mom walked in, mouth hung open...though I could see the joy (not fear) behind the shock..."we don't have to come back for a year!" She didn't know what to do or how to respond...with the information. We spoke about it for a minute, the fear of what would happen in that time. Would she able to spot any signs and symptoms that would require her to come back sooner? A year...I thought to myself, and even wondered out-loud what - what would it be like to be told to come back in a year?

The fantastic mom...who has been through hell and back looked at me and said - you'll get told "come back in a year" too - she was comforting me.

It was at that moment that that a flood of emotions came through me...I worry about my little girl - I even panic about her...but her prognosis is no where near as dire as these other children we meet in the hemotology oncology department.

At that moment in time, I had the ridiculous guilt of my child not being as sick as these other children...

There, I said it out loud. How ridiculous is that of me? Guilt that my child is not as sick as these other children...that this mom took the time to support me.

We moved on to steps 5 - 7....and then it was time for step 8.

Dr. H met us in the art room - handing us our prescriptions and the check out form...written clear as day - return in 6 weeks. While it wasn't the 1 year mark, it was 1.5 times as long as we had been coming up till then. That fantastic mom...who was still on step 8 even an hour later...looked at me and said, you'll get to your one year...one step at a time.

I shook Dr. H's reassuring hand....The fantastic mom gave me a hug...and Bean and I were on our way back to school.

I was afraid to get excited. I was afraid to be thrilled with the news...we have been down the "good news" path before...and burned. I decided to keep the good news to myself, still waiting for the other shoe to drop.
_____________________

Bean and I went back 6 weeks later - Thursday, February 25th - we went through the motions. We followed our steps 1-8. Stopping to talk about hair loss and what would happen if she lost her hair, the picc line inserted a girls arm, or the pump connected to another girls upper chest.

Between steps 7 and 8 Dr. H came in - giving us the news that there was no visible sign of swelling or active SIJA...her blood work looked good - the methotrexate seems to be working. We were to continue on the same treatment path...and come back in 8 weeks!

This time I allowed myself to post the good news on my facebook status - it read:

Great visit with Dr. H...no symptoms visible...we don't have to go back for 8 weeks! That is AFTER Passover!

I still have the pit in the bottom of my stomach - the one that is waiting for the other shoe to drop....at the same time feeling guilty that we get 8 weeks to not have to come back, while others we get to know at Children's will not have even been released during that time.

For now I worry. But I am grateful and thankful that we haven't found the other shoe.

Paranoia, when does it go away? Does it go away?

I look at Bean every day.

Well, to be fair I look at ALL my children every day. I give them a once over, checking on parts, boo boos, hairs, nails, toes, noses...it is a two fold mission...I want to make sure they are ok, safe and healthy.

Really though, I try to make sure I soak them in. I want to remember their parts, know each "beauty mark," each freckle, each crevice, each spot.

Bob's two birthmarks - one that I noticed on the day that he was born and knew no one could ever mix him up...and the other the memory of his NICU stay...

Belle's gentle curve to her collar bone with beauty mark that seems to have been placed on her ivory skin by the model gods (a la Cindy Crawford),

Bean's long skinny fingers with beautiful long nail beds...a hand model as it were...with not a freckle on any of them, her beauty mark of prominence is just off center of her midline, right by her hairline....

Puppy's fantastically edible earlobes with an adorable beauty mark right in the middle of his right ear...

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.

Too good to be true.

Bean took her 14th dose of Methotrexate last night. She didn't want to eat dinner it just isn't like my little beauti. She said her mouth hurt so I took a look and there it was, her first side effect - a mouth sore.

For 14 weeks she had no side effects...I guess it was just too good to be true.

We had some medicine to put on it - bad tasting medicine, bad smelling medicine...medicine that needs to dry before you can close your mouth our eat/drink anything. Bean is one of the worst medicine takers I know...

But our amazing friend B was over for dinner with her family, this is the woman who (in adition to J) helped me deliver all four of our children. If she could get me through that - she surely get Bean through this. Well she did! A bit if distraction, a bit of fun and we did it - She counted "black cats" and with that we were able to distract Bean enough to put medicne on and sooth her pain.

I will write Dr. H an e-mail to see if we increase the Folic Acid or if there is something else we can do. But for now - we are counting "black cats!"

1 black cat
2 black cats
3 black cats...

One year tomorrow...

One year tomorrow....I need to say that again. One year tomorrow. This journey began one year ago tomorrow. I watched my Bean dance today...with every joint of in her body - her hips, her knees, her elbows, her wrists, her ankles, her sholders, her neck...
One year ago tomorrow this journey began. But today, she danced!

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

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….