Celebrating Christmas

Here is a summary of our Christmas preparations and celebrations over the past few weeks.
(Warning...this post contains excessive amounts of pictures!)

Visiting Santa

My sister and I took our girls to see Santa together.  This is her daughter, Tatum's first Christmas, Audra's "second" and Maura's sixth!

 Maura is an old pro with Santa...she was so excited.  She gave him a story she had written for him and a big hug!

Tatum didn't seem to mind sitting on the jolly old man's lap.

Audra, though, would have NO part of it!

 As if to say...don't even try to make me do this:

Baking Christmas cookies

This was definitely a more desirable Christmas activity for Audra.

Maura did almost all of the decorating this year:  mommy frosted and she sprinkled.

Christmas portraits

We had portraits done with cousins Daniel and Charlotte.

the girl cousins...

the boy cousins...

my sweet kiddos...

my handsome boys...

my beautiful girls...

Christmas Eve...

We always spend Christmas Eve at my parents house...dinner and presents with my siblings and their families.

When we got home to kids were anxious to go to bed so Santa could come.  But first, Maura picked out the most perfect of the cookies she had made and left them out for Santa.  Then she sprinkled the magic reindeer food she had made at school on our lawn.

I love the beauty of the tree on Christmas Eve, with sparkling lights in the dark of the night, and colorfully wrapped presents below...waiting for the children who lay sleeping upstairs...dreaming of what tomorrow will bring.

Christmas morning

Yep...Santa came!

The older he gets...the smaller his presents get in size?! 

(but not cost!!)

Ready for church

The rest of the day was spent enjoying new toys and the company of  the many family members who stopped by throughout the day.  And since Christmas is also Daddy's birthday, the presents and celebrating continued...

Just in the {Saint} Nick of time

Christmas crept up on me this year.

Maybe it's because Thanksgiving was late this year, or maybe it was just me.  I found it hard to get myself into the holiday groove this year.  For whatever reason I wasn't "feeling it"...which is very unlike me!  And before I knew it time was running out.

On Saturday I found myself wishing for just a little more time...

I needed Sunday (my last day off before Christmas Eve) to be 48 hours long instead of only 24.  I really didn't know how I was going to manage to get everything done before the BIG day.

Sunday came and went, and I tried my best to be as productive as possible...

Shopping,
wrapping,
baking,
cleaning,
decorating.

And when the day was done I resigned myself to be satisfied with what I was able to accomplish in those 24 hours, even if I didn't get everything crossed of my Christmas to-do list.  Ready or not, Christmas would be here in just a few short days.  And those few days I would be spending at work.

Then Monday morning came and Aidan wasn't feeling well.  He stayed home from school...so I stayed home from work.  A blessing for me...not so much for Aidan.  (Thanks for taking one for the team, Aidan!)  I had a very productive day at home getting all those last minute things done that I thought just wouldn't happen this year.

Bring it on Christmas...I'm ready!

Infusion #3

3rd SCIg infusion...
2nd one at home...
1st time on our own!

...and Audra did even better this week than last week.  She still fought us getting the needle in (who wouldn't?!), but as soon as that part was done she was happy to sit in bed with us and watch TV.  Trouble was she fell asleep...and 90 minutes later when the infusion was done we had to wake her up to take the needle out :(

Not the way anyone wants to get woken up...especially when you are comfy, cozy...snuggling in bed with Mommy and Daddy.

Lucky for this Mama and Baba, Audra is quick to forgive and doesn't hold a grudge.

So, snuggle time resumed!

Audra seems to be having some headaches associated with the immunoglobulin infusions.  They are random, but significant.  He holds her forehead and says "ow", and she rarely if ever complains of pain.  One particularly bad day she was crying and inconsolable at school so we had to go pick her up.  For the 24 hours before each infusion I give her lots of extra fluid in her G-tube in hopes of minimizing the side effects.  I may need to start giving her Tylenol routinely too.

1st infusion at home

Tonight was Audra's first home infusion of SCIg.  A home care nurse arrived at 6:30pm.  First we had to complete some paperwork...consents, insurance forms and such.  Then the nurse examined Audra....the obligatory temperature, blood pressure, weight and height check.

When it was time to prepare the medication I pulled out all the supplies that had been delivered earlier in the week....

medication
syringes
tubing
needles
dressings
pump
alcohol preps
sharps container

I laid out everything we would need for tonight's infusion and the nurse looked at me as if to say "go ahead".
Alrighty...I can do this...I am a nurse...

The preparation of the medication is actually quite easy.  Except that it is quite thick, which makes it challenging to pull up into the syringe and remove air bubbles from.  And to think that something so viscous needs to be injected into Audra to keep her healthy?!

Then it was time to insert the needle.  Our delivery of supplies only included single needles, not double needles like they used at the hospital last week.  So we would have to inject the entire 10ml of thick antibody goodness into one area of my skinny girl.

Again...I got the "go ahead" look from the home care nurse.  Silly me, I thought we were going to ease our way into doing the infusions by ourselves.

once Audra was more comfortable with the process...
once her mama was more comfortable with the process!

Sure, I know HOW to insert a needle...but inserting a needle on my own little girlie is so.very.different!

To my surprise Audra did much better than I anticipated.  She still fought and we had to hold her down, but the minute the needle was in she was FINE.  She wanted to keep the site covered with her blanket and wasn't to happy when I checked on it every so often.  The infusion took about 1.5 hours and then the fun of getting the needle out started.  The home care nurse had left before the infusion was done, she had deemed us competent enough to manage on our own!  Lucky us.

I really think Audra was more bothered by pulling the dressing off her skin than the actual needle removal.  Once the needle was out and safely stowed in the sharps container (these needles don't have a safety lock to prevent accidental  pokes) we tucked her in for the night and she fell asleep fast and hard.

SCIg...Hizentra

Audra's treatment for CVID is weekly subcutaneous immunoglobulin replacement therapy (SCIg).  Her prescription is for Hizentra.  It contains antibodies from the plasma of thousands of blood donors.

Antibodies that her little body doesn't make to protect itself.

Audra's immunologist prefers subcutaneous administration vs. intravenous administration for Audra.  She gets her monthly dosage of immunoglobluin divided into four doses, given once a week.  This will allow her body to have more steady antibody levels.  When given intravenously, the antibody levels spike on the day of administration and then taper off as the month goes on.  This can result having less protection from illness in the 3rd and 4th weeks after receiving the IV immunoglobulin.  When given weekly, Audra should have a steady level of antibodies at all times to help protect her.

Here are the basics:
~ draw the medication up into a syringe
~ attach tubing to the syringe and prime tubing with the medication
~ put the syringe into the pump
~ clean the skin site with an alcohol wipe
~ pinch the skin up and insert the needle, cover with a dressing to keep the needle in place
~ start the pump, it runs by pressure so the amount of time it takes to infuse is somewhere around an hour

Next...try to distract a rambunctious little girl during the approximately one hour long infusion!!

When the syringe is empty, remove the needle from the skin and discard in a sharps container.

Then repeat 7 days later.

For SCIg the side effects are usually local swelling or irritation.  Some people experience headaches, body aches, and GI symptoms.  Generally, subcutaneous administrations has significantly less side effects than intravenous administration.  

CVID

The immune deficiency that Audra has recently been diagnosed with is:

Common Variable Immune Deficiency

Common Variable Immune Deficiency (CVID) is a frequently diagnosed immunodeficiency characterized by low levels of serum immunoglobulins and antibodies, which causes an increased susceptibility to infection.  People with CVID often have infections that are described as recurrent, persistent, unusual or severe.  While CVID is thought to be due to genetic defects, the exact cause of the disorder is unknown in the large majority of cases.  Compared to other human immune defects, CVID is a relatively frequent form of primary immunodeficiency, found in about 1 in 25,000 persons; this is the reason it is called “common.” The degree and type of deficiency of serum immunoglobulins, and the clinical course, varies from patient to patient, hence, the word “variable.”  Patients with CVID also have an increased incidence of autoimmune or inflammatory manifestations and an increased susceptibility to cancer when compared to the general population.  Both males and females may have CVID.  In the majority, the diagnosis is not made until the third or fourth decade of life. However, about 20% of patients have symptoms of the disease or are found to be immunodeficient in childhood.

Diagnostic Criteria:

• recurrent infections (especially involving the ears, sinuses and lungs)
• digestive system problems (malabsorption, diarrhea, poor growth)
• low immunoglobulin leves (IgG, IgA and sometimes IgM)
• low antibody responses to vaccines 

Treatment:

People with CVID receive intravenous (through the vein) immunoglobulin replacement therapy every three to four weeks or subcutaneous (just under the skin) immunoglobulin replacement therapy every week to restore normal immunoglobulin levels.  The immunoglobulin given by either of these methods provides antibodies from about 60,000 healthy donors each time it is given.  Many patients need preventive antibiotics as well.

Audra has:

• low IgG and IgA levels
• loss of antibody response to vaccines (diptheria, tetanus, measles, pneumococcal)
• digestive problems:  malabsorption, diarrhea, failure to thrive, villi blunting on endoscopy
• recurrent ear infections resulting in ruptured ear drum and hearing loss
• usually severe UTI's resulting in pyelonephritis and kidney abcesses, with a severe inflammatory response (very high CRP and ESR)
• unusual fungal pneumonia when she was an infant in China

Infusion Day #1

Audra did very well today with her first immune globulin infusion.  

(her super power antibody infusion as I told her)

I picked her up from school just before lunch time and she just knew it probably wasn't a good thing.  (Well, really, it IS a good thing...but there's probably no convincing her of that!)  She procrastinated a bit by taking some extra time saying her goodbyes and then reluctantly took my hand as we walked to the car.  

I had picked up a happy meal for her to eat on the way down to the hospital but she must have been really nervous because she wouldn't eat.  And Audra not wanting to eat is like...Stop the Press!!  Breaking News!!

The day medicine unit where the infusion was done is very nice.  She was in her own little cubicle with a recliner-type chair and a TV.  I brought plenty of activities and snacks to keep her entertained so we were set.  

Then it was time to begin...

Audra has been around hospitals enough during her 19 months home to pick up on the subtle clues pretty quickly...

the nurse puts gloves on

another nurse enters the room

they gather supplies from the cart

She was not happy and she let us know!

This sweet little girl needed 4 nurses to hold her still while two nurses put a needle in each of her thighs.  

Tiny but oh.so.mighty!  That's my girl :)

Once the needles were in we covered her legs with a blanket and it was like nothing ever happened...

~snacks in hand

~Doc McStuffins on TV

...all was well.

The infusion took one hour and she tolerated it beautifully.  I had pre-hydrated her last night and this morning with extra water in her g-tube to hopefully prevent some of the nasty side effects:  nausea/vomiting, headache, body aches, etc.  They said she may be more tired than usual, and I think she was.  Our "little tornado" was more like a gently breeze the rest of the day.  She is definitely sore at the infusion sites and won't let me go near them.  

We were "gifted" with many things on this first infusion day ...

~ books and a DVD from the Immune Deficiency Foundation for me (looks like I have some homework to do!)

~ a binder for organizing all of Audra's medical information

~a cute little stuffed monkey with play infusion supplies for Audra (from the company who make the immune globulin)

Infusion #1 was a success.

Infusion #2 at home next week.