Carival rides bring smiling eyes...

Friends & Family-

In an effort to remind everyone that in spite of these two nasty diseases that life does go on, we are posting some pictures of fun we had this past weekend.  Lucianna's physical limitations and rigorous medicine schedule are tough to work around in order to go out for the day, but we have fallen into our "new normal" and have learned how to make it work.

This is her second week on Methotrexate and she did great.  The relief from this drug could take up to 6 weeks to witness.   She wasn't grumpy on Saturday at all.  However, Bella decided to fill that 'academy award winning role'.  Argggg.  Can't we all just get along?

So this past weekend the girls visited their Grandma and Papa, (my parents), and we spent Saturday at a fun carnival/festival.  I mean seriously..toothless, country boys, herding our children onto a metal car that goes around in circles for 45 seconds...what's more fun than that?!?! 

Enjoy the photo's!
Love,
Lucianna's Proud Mommy

This picture speaks volumes to the resiliency of a child...

Update- Eye Dr appt moved to Friday, 9/28

FRIDAY, SEPTEMBER 28TH UPDATE:  The ophthalmologist has confirmed that her eyes are improving!  She is now listed as having "moderate" Uveitis, no longer "severe" - I can't wait to hear that it's, "mild".  (We are so exicted.)  So she is still responding to the drops, which is great news! Her dosage has schedule changed as well - she moves from hourly drops in each eye, to 2 drops, each eye, every 2 hours.  HOORAY!  We are very encouraged that she is continuing to improve! 

Thursday, September 27, 2012:

Dear Family & Friends-

Our eye doctors office called today to express their concern about us waiting until October 4th for our next appt. (We were waiting that long because there were no other appointments available.)  The main doctor in the practice feels it's too long to wait; our current doctor is out of the country. Well, we found out that indeed they do have open appointments, but we would just have to go to their other office further from our home - which we are willing to do.  So they requested we come in tomorrow, Friday, September 28 at 11:15am.  Daddy is taking her to the appointment and I will be at work biting my nails off.

Here is what I hope to hear:

• She is responding very well to the drops.
• She has no residual eye damage. 
• Her Uveitis is calming down and getting better.
• We can discontinue drops at "such time" or decrease the frequency.

I will post an update within this blogpost as soon as I know something.

My fingers are crossed...

Love, 

Lucianna's proud Mama

Oral Methotrexate Administration - Step by Step Pictures and Video (for other parents)

**Video's do not come through on the email alerts, you need to go to the website to view them.

Fellow JRA/Uveitis parents-

**I am not a doctor or a nurse, just a parent trying to share information.  Before administering any drug to anyone, you should consult with your physician.

Administering this drug was not self explanatory since there was no documentation from the pharmacy to explain to a parent how to do it.  I have personally never worked with syringes, needles, etc.  I want to share, frame by frame how to do this in case any of you need a little help like I did.  We chose, after consulting on a message board with some other parents to administer the drug at night on a Friday.  That way, if there is any side effects or fatigue, she is home with us to monitor her.  I also chose to give it right before bed so that if she suffers nausea, she sleeps through much of it.  (I hope.)

To administer Methotrexate, [MTX], orally, (which means you will REMOVE THE NEEDLE prior to squirting it into your child's mouth):

1. Your RX should include 3 parts:  MTX vial, alcohol swabs and needles with syringes attached. From what I understand, there are two types of MTX, with and without preservatives.  Ours is with a preservative so that we can get the maximum dosage from the bottle with no waste.  The other kind does not have a preservative in it, therefore after the first dose, the rest is discarded.  (Discuss with your own doctor though.)

 2.  SHAKE BOTTLE WELL.  After carefully removing the orange cap from the vial, wipe the top of the vial lid off with the alcohol swab.  This will ensure it's sterile.

3.  Remove the needle/syringe from the sealed package.  There is a cap on the end, remove the cap to expose the needle.  (Blech...I really hate needles.)

4.  Insert the needle directly into the "bull's eye" of the vial lid.  

5.  Turn the bottle upside down carefully balancing the needle & the bottle in one hand, while the other hand is pulling down on the plunger to fill the syringe. (Notice the iced coffee in the background...Mama plans to be up late into the night to monitor her and make sure she has zero side effects.  In fact, I plan to sleep in her room. Call me a worry wart!)

 

 6.  Make sure you don't have any air bubbles or gaps, so that you are certain that you have the exact dose required by your doctor.  For our daughter, the dose is .3 mls once a week.

7.   Replace the orange cap on the vial and put the remaining MTX back in the fridge - keep it in the original box so that it's not exposed to the light every time the fridge is opened.  Keep the syringe out on your counter and allow it to achieve room temperature which takes approximately, 60 to 90 minutes - this was our doctor's recommendation.

8.   REMOVE THE NEEDLE FROM THE SYRINGE - (you just twist it off) - I used a napkin and the needle came right off, leaving just a medicine filled syringe.  Keep it pointing up so you don't lose any!

9.   Our doctor recommended that we give our daughter no fluids 10 minutes before or 10 minutes after because the enzyme's could cause the MTX to begin to break down.  

10.  Our doctor advised that we squirt the medicine into her cheek, NOT in the back of the mouth because that feeling would cause her to gag/choke. **We are told it is tasteless...I am not sure that Lucianna agrees with that - but she did great. 

HERE IS OUR FIRST EVER MTX ORAL DOSE & OUR LITTLE TROOPER!
                     

 

Below is a picture of the empty syringe, the needle after it was removed, and our little munchkin at 9 months old.  Little did we know that just over 2 years later, we would be battling this disease.  She is strong though, and we are so proud of her.

We have read online from other parents that say hydration is very important on this drug - so lots of water from here on out!

Love,
Lucianna's Proud Mama

BELOW THIS LINE, I WILL UPDATE THIS BLOG POST 

IF WE RECOGNIZE ANY SIDE EFFECTS IN THE NEXT FEW DAYS.

STAY TUNED!

-----------------------------------------------------------------------------
Sunday, September 23rd:  - So far so good...
    As you read, MTX is administered on Friday nights in hopes that if she feels any nausea, she would perhaps sleep through it.  I monitored her throughout the night (until about 3am).  She had some fairly interrupted sleep (a lot of tossing and turning) but overall did well.  The next day however....she was VERY GRUMPY.  That said, her grandparents were here from NY and I think some of it was just the change in her daily norm but I also think some of it was drug/sleep related.  Otherwise, she did great and I am so thankful that "week 1" went so well.  **A special thanks to my in-laws who were so helpful in coming to visit this weekend.  My MIL cooked, cleaned, helped with the girls, brought food, helped with laundry...my FIL, played with the kids, took out trash, helped around the house and both are just the most amazing extra set of parents that I could ever hope for.  I don't know if they truly know how much it meant to me to have them spend the weekend and help so much. (Lucianna's daddy worked this weekend.)  I have always felt so lucky to have them as in-laws.  They are living proof that "family is always there for you". 
     If Lucianna has any other side effects or changes, I will post them within this blogpost for anyone reading who is worried about side effects for their child.  Whatever you do, don't read the accompanying documentation for the MTX.  It will terrify you.  Just leave it in the box and know the major and minor side effects in advance.
     Have a great week...next dose is Friday night. 

Float like a butterfly - sting like a bee!

THE WILMER EYE INSTITUTE AT JOHNS HOPKINS HOSPITAL:

Dear friends and family,

A short update on Lucianna's Uveitis progress.  Her normal pediatric ophthalmologist is on vacation this week, so we used this opportunity to get her an appointment with a Uveitis specialist at the Wilmer Eye Institute, (which is next door to Johns Hopkins Hospital), in Baltimore, MD.  After a grueling afternoon of waiting, waiting and more waiting, (her Daddy took her), we learned nothing new about her condition.  The doctor today just reiterated what we already knew:  her Uveitis is severe and we need to continue on hourly eye drop treatments and nightly dilation drops until further notice.  He agreed that we are on the right path with our current ophthalmologist, Dr. P., and for us to continue seeing her since she was much closer to our home. Daddy shared that Dr. P.  has equipment that is better suited for kids, newer and an appointment to see her certainly doesn't take a half day like our Hopkins visits normally do - so we are sticking with her for now. (She's pretty great by the way - another bonus.)  We are glad we took the time to go to TWEI though.  We never want to look back on this time 5 years from now and think that we should have gotten a second opinion.  Now we know that we are doing exactly what we need to be doing.

Currently, her treatment course is hourly steriod eye drops, one drop nightly that dilates the eyes, Naproxen - 3 times a day and Zantac - twice a day.  This Friday, (or Saturday), we will introduce Methotrexate.    Lucianna is the strongest kid I know.  I do not say that as her parent, but as an adult who has had a great deal of exposure to kids of all ages throughout my life - Lucianna is my Mohammad Ali.  She is a fighting machine.  She has the fiestiness of her Mama and the determination of her Daddy.  She amazes me everyday.

I learned some disheartening information about the other 299,999 children who suffer from this JRA, there are 10 states that DO NOT have a pediatric rheumatologist at all.  

Today, I am grateful that we live in an area where access to a pediatric rheumatologist is not an issue for us.  I can't even imagine the hardship for families that have to fly to their appointments or drive very long distances just to see a doctor.  See the chart below to fully understand the shortage.  (Note to self: "encourage up-and-coming college students to study pediatric rheumatology!!!)

Purple - 0 , Dark Green - 1, Light Green - 2, Orange - 3, White - 4 or more.

Ending on some of Mohammad Ali's great quotes:

"When you are as great as I am, it is hard to be humble."

"I'm so fast that last night I turned off the light switch in my hotel room and was in bed before the room was dark."

"It isn’t the mountains ahead to climb that wear you out; it’s the pebble in your shoe."
 

“If my mind can conceive it, and my heart can believe it - then I can achieve it.” 

Love,

Lucianna's proud Mama

Fast Walkin' - caught on video!

She always has a smile...or she sticks out her tongue.

--------Video's Attached, you may only be able to see it on the blog site though.------

Dear family and friends,

We have had to share bad news to over the last few months...I want to reserve this blog entry for something fun, uplifting and encouraging!

Despite the Uveitis diagnosis last Tuesday, this kid has been AMAZING!  She takes her hourly eye drops like a pro and never complains.   She stops what's she's doing and doesn't even give us a peep! We have weaned her off getting an hourly jelly bean with her drops and now she only gets a candy treat at night - Nonna....send more pez refills!!!  Anyway, the point of this blog entry is that Lucianna's once stiff, painful legs that had her on the couch for most of the month of July, and some of August, has been replaced with very capable "fast walker"...she's really showing off here (and I love it).

Her Rheumatologist did say the Uveitis would be our primary concern and the arthritis should get less and less bothersome. I wonder if this is exactly he means or this is a miracle coincidence?  *Her rheumatologist says that her long term issue would be the Uveitis and that we would see an increase in her mobility.  It basically means the illness has taken a different course.  I still like seeing her be able to dance, run and play.

Whatever the case may be, this weekend we were met by my dear friends Carrie, Skylar and Diana who brought us up casserole dishes galore for us to freeze and use on those tougher weeks.   Everything from french toast breakfast casseroles to dinner casseroles/lasgana's, Paula Deen soups, you name it.    They spent the night, helped me out...made me laugh...they were wonderful.  I can't thank them enough.

So let's end on all the smiles we had over the weekend:

Love, Lucianna

Her follow up eye appt was today...



This is one of my favorite pictures of her taken last Christmas...

All-

Let me start with the good news:  Her eyes are responding to the steroid drops, (there are kids with Uveitis who simply do not respond to any therapies/meds). This is the first big hurdle - and incredibly great news.   That said, she is NOT, by any stretch, "recovered" or out of the woods. 

The not-so-great news:  She has a severe case of Uveitis, which is shocking considering she was just checked two months ago and wasn't supposed to go back for a re-check until late October.  Thank goodness she had some symptoms.  Uveitis is typically a silent, symptom-free and painless disease.  You don't always know about it until it's too late.  Severe cases are more difficult to get under control and have more serious implications when it comes to vision complications.  Therefore, she will be undergoing treatment for the next SEVERAL months.  That's right, several months.  Currently, she needs hourly drops during her awake times and a nightly drop, which she hates, because it burns.  This disease is not convenient by any means.  To have to stop everything you are doing, every hour, is quite annoying....but necessary.  The drops are milky white, not a pretty thing to see coming from her eyes, but Lucianna handles it like a trooper!  Her teachers at her school rave at how amazing she is at taking the drops.  Her teachers have been amazing at dealing with this rigorous medicine schedule.  We are so lucky to have them.

The eye drops are a steroid - steroid drops cannot be given long-term because they can cause cataracts and glaucoma.  Yes, you read that right....we have to give her a medicine to keep her from eye damage and vision loss that could cause her -eye damage and vision loss.  Makes no sense, but it's our only option and we have to treat her and hope that she does well with the steroid.  Long term:  she will have to have routine eye appointments for the rest of her life to make sure this condition stays in check.  This condition doesn't just go away, (I wish it were that simple).

To end on good news:  her ophthalmologist does not think she has suffered any damage in these two short months - so we just need to focus on getting her better.  I was hoping it wouldn't take so long to treat her.  "Several months" is a lot of waiting, hoping and anxiety.  I am learning that I need to lower my expectations and just focus on the day-to-day of getting her well.   Anyone who knows me, knows that I don't do anything day-to-day...I am a planner and I need to know what's going to happen.  Unfortunately, with JRA and Uveitis, projecting her outcome is impossible.  There are too many variables involved and every child is different - so I am going to learn "patience" in ways I never knew before.  Sigh.  Next Friday, we move to Methotrexate - and my hope is that she does well with it.

Also, she is also doing great from a walking stand point.  She is getting around much more independently.  She looks like she has wooden legs, but we are hoping that just continues to walk better and better.  Lately she has been "fast walking", which is so cute and enjoyable to see!

She is due back to the eye doctor on Thursday, September 20th, and as long as she keeps responding well, we will likely go every week until further notice.

Matt and I have reached out to her rheumatologist about teaming with the experts at the Wilmer Eye Institute at Johns Hopkins. 

Keep the well wishes coming and thank you for keeping up with her condition.  I will update you when we have news to share.

Love,
Lucianna's Proud Mama

Uveitis, Pauciarticular JRA diagnosis and next steps...

Saturday, September 1st.

EYE FOLLOW UP APPT IS FRIDAY,

SEPTEMBER 14TH AT 1:30PM ET. 

STAY TUNED FOR AN UPDATE!

Friends and family,

I have noticed some changes in Lucianna's eyes over the past few days, she has had redness and just a cloudiness about them that didn't seem quite right. Knowing that Uveitis,{"U-Vee-I-TIS"}, a condition associated with JRA, whereby the inflammation attacks the eyes is a very real concern, I decided to get her an appt right away. Her Dad took her this morning. She has Uveitis in both eyes. The news has hit us like a freight train and we are currently trying to process what this means for us and her. Learn more: How Juvenile Arthritis Affects the Eyes?

I don't think I ever allowed myself to believe that she would get Uveitis, although I tried to keep it in the back of mind. Today, all that changed.

What we know so far:
The eye medication should get her inflammation under control. That said, the drops used to treat this condition are incredibly strong and do not come without their own set of side effects. There is just nothing okay about this eye disease and I can't sugar coat the serious nature of it.

The pediatric ophthalmologist has prescribed two types of eye drops, one that must be administered every hour, yes, every hour....until Friday. The other drop will be given every night before bed and we are told that particular drop will burn. I have no clue how I am going to get these drops in her eyes without her Dad's help on the nights that he works. It will be a learning experience that I will couple with M&M's & jelly beans to aid in my success. Luckily, Dad has taken off work tonight so that we can regroup, figure out what this means and just be together as a family. Today has been very hard emotionally for us.

So now she is officially diagnosed -(this diagnosis could change if she develops any other joint problems between now and December), she has Pauciarticular Juvenile Rheumatoid/Idiopathic Arthritis. (JIA/JRA acronyms are basically one in the same.) What does that mean? Her biggest burden will be her eyes and keeping them well. The arthritis will be her secondary problem and we may see it become less and less bothersome - however, her eyes will need more and more attention and careful monitoring.

Lucianna will go back to the eye doctor on Friday for a follow up to see how she is responding to the drops. If her eyes aren't under control by Friday, they will take the next steps in treatment and monitor her all next week. (This means several visits to the eye Dr's, possibly daily visits through next week, until it's under control.)

That brings us to Methotrexate, (MTX). JRA is a vicious disease that can wreak havoc on her joints causing a great deal of pain and deformity if left untreated. MTX is a powerful drug and comes with its share of side effects that would make any parent cringe to have to make this decision to put their child on it. Bottom line, the JRA needs stopped...the MTX is capable of doing that. We will begin this drug next week. It is considered a "2nd line of defense", (the Naproxen was the 1st line of defense). This drug is a "DMARD", disease modifying antirheumatic drug. It is described as a drug that knocks JRA over the head, over and over with a baseball bat until the JRA finally falls down to the ground.  This DOES NOT mean it will never come back again.  MTX will treat her arthritis and help her eyes. The benefits of MTX do outweigh the burdens - as scary of a drug as it is. Most kids have great success on it. We hope she does well with it and tolerates the "oral form" well so that we don't have to inject it. You can read about it here: Methotrexate for treatment of JRA. Normally we would have started the MTX today but she needed caught up on one vacinne (the MMR), and should get a flu shot before starting it. She was at the pediatrician's office this afternoon getting both those shots to prepare her for the MTX which can be started one week after the immunization. (Poor kid.) Once we start MTX, she will likely be on it for 6 months to a year. We also need to keep her relatively healthy so that her already compromised immune system can fight without anything else bringing her down. (Luckily she only has one other little girl in her class at school so not a ton of sickness exposure.) --small blessings--

So that's where we are. I am still in the "why us" phase but in day or so, I will get my game face on and keep up this fight. I will try to educate myself on Uveitis, MTX and Pauciarticular JRA. We will continue to do whatever means necessary to aid in Lucianna's comfort. And....we will not forget that Bella, our healthy child, needs love and attention too. Thank you for all your support and well wishes.

I will post another update as soon as I have something new to share.

Love,
Lucianna's Proud Mama