When it Rains...It Pours

...But at least I have these little angels with me.  I took this picture during a fabulous rainstorm this morning.  It has been a crazy summer of rain.  In this picture, we see them in a single dimension brimming with health, vitality and beauty.  We spend a lot of time looking at others in a single dimension.  Ironically, singularity emodies both truth and deception.  These children are brimming with health, vitality and beauty.  But three of these children have PCD.  A disease which ravishes their lungs.  A disease we spend hours a day treating in an effort to halt its progression.  Two of them have hospital stays, surgeries and IV therapies dangling over their heads in the near future.  But they look so healthy, right?  There can't be anything to worry about...or so the well-meaning tell me over and over.  The best way to support is to acknowledge grieving and offer a listening ear.

I've said it before, but I think there has to be some sort of law of compensation.  If you are given a nasty disease to deal with when you are born, and for the rest of your life,  you are given an added measure (a huge one) of sweetness and depth.  I know it is true with my children.  And the 3 of us born without it, that same nasty disease strips us of ambivalence and clothes us with keen awareness. We are better too because of it.

There are a lot of times, in the moment, when I question if taking on the education of my children at home is really what I want to do.  It often pushes me to emotional exhaustion...Invariably, I conclude yes.  Most days I spend 5 hours on the piano, directing school from the bench next to a practicing child.  But the vigorous study of music has equipped my children with an amazing ability to work through very difficult things, which are not fun in the moment, for a greater, delayed reward of mastery.  It's taught me that same lesson.  I never imagined the role music would play in my life and in my children's, but I am grateful for it.  It was a driving force in bringing Ellery back to life after a severe prolonged illness.   I feel music is poised again to pull our family through some rocky times ahead.

It's been a very long 6 weeks.  I'll be grateful for answers tomorrow.

Happy Ninth Ellery and a Perfectly Pink Party

Ellery on her new Beach Cruiser.

A Perfectly Pink Party.

The girls went on a wild scavenger hunt all over the neighborhood.  Tasks included things like "touching a dirty boy," and "forming the number 9 with your bodies."

We opted for a very layered, frosting free cake.  It was dyed pink with beet juice and smothered in fresh raspberries and strawberries.

A few little personal notes on Ellery.  She... 

...has the poise and confidence to match many adults

...is learning intellectual integrity and courage by being unafraid to act and think and speak differently than her peers

...plays the piano with intensity and reflection

....loves to write poetry, especially poetry she considers extremely deep, romantic and metaphorical

....memorizes amazingly

...likes to be alone a lot

....has an intensely emotional connection with her physical body, she wears her emotions in her health

....drives her mom to jealousy because she favors dad

....worries incessantly over safety, which translates into being very responsible and very strict with her siblings

....is known to let out piercing screams when she feels something or someone is in harms way

....loves to perform and be in the spotlight, but wouldn't be labeled as outgoing

....perseveres through scary doctors appointments, and adamantly adheres to treatments, but is also very self conscious of coughing in public

....devotes herself to gymnastics

....loves to sew clothes, flowers, headbands, and pillows for herself and others, but doesn't love following a pattern

....watches and eavesdrops on her parents like a hawk

...loves fashion, dancing, pop music, and takes all of it quite seriously

....begs to sing solos in church

....has an uncanny, tender gift of connection with the elderly


We love you Ellery.  We are better parents because of you.  You've taught us how to love so much deeper.  A first child opens your heart in a way it never has been before.  Each year you grow opens our heart to more pain with an equal balance of love.  We've wept over your pain and struggles from day one battling PCD.  But your talents floor us in kind.  It's a risky game to be a parent but one we gladly take on.

The Best Christmas Gift: A Lung Function Test

It's easy to lose sight of the best gifts.  

Sometimes I forget 3 of my beautiful 4 children are running around happy and glowing, as persistent bacterias slowly eat away at their lungs.

I forget to talk louder and even sometimes to clean out their ears.

I think the drainage stops, only to find their ears are blocked solid with hardened fluid.

I let Emmett skip his vest because it hurts his tummy.  Ellery gets days off on her vest on Sunday and Saturday.

I forget how little I know of what the future holds.

It's hard to go back and remember what I've shelved from that painful winter last year.

I guess it can be okay to bring everything back again and remind me of things even more important than practicing the piano and cooking without refined sugar and processed, gunk filled foods.

Waking Up Again

The day before Thanksgiving the children had appointments with their pulmonologist at Primary Children's Hospital in SLC.  We were happy and confident that the changes we implemented the last 6 months would show remarkable improvement in the children.  Everyone seemed relatively healthy.  Gymnastics and flute were building lung strength and capacity...

The doctor nodded along smiling, as we explained how well everyone was doing.  Yes we have had 3 courses of antibiotics in 3 months for those nasty exacerbations that just don't seem to go away, but...

Then came the PFT's (pulmonary function tests).  We were in a hurry to get out of the office and back to Mimi's for Cousin's Camp, so I pushed to nurses and respiratory therapists to hurry.  I took note of the all the red on Ellery's PFT's.  I knew it wasn't looking good.  I wasn't prepared for the phone call from the doctor 30 minutes later.

Ellery's lung function had dropped 35% in her large airways and over 70% in her small airways.  This was precipitous and unacceptable.  She would need to start a vigorous course of antibiotics, increased airway clearance therapy to 14x per week, and a new nebulized medication.  In 2 weeks if her PFT's were not at baseline, we could expect to be in the hospital for IV antibiotics.  You can't drop 35% too many times before you get to zero.

The Interim

You can probably imagine what the ensuing 2 -1/2 weeks held.  Raw emotions in large doses.  We tried to shield Ellery from this information, but one night she happened to be sitting just out of sight at the top of the stairs while Brent and I were expressing our fears.  With Ellery in on the news, our anxiety just kept growing.  Often times I would find her just crying.  Scared.  I think eventually we helped allay most of her fears and even got her a little peaked about the attention and excitement we would provide with a Christmas hospital stay...  We put on a lot of brave faces.

It was easy to think...no way.  This isn't real.  She is fine.  I was ready to fight the doctors.  But then...what if I am wrong.  We waited.  We all ate cloves of crushed garlic.  Missing vest treatments was no longer an option.

The Best Gift

And then came the best gift.  On December 13th Ellery passed her PFT's with flying colors.  No further permanent lung damage, no psuedomonis, no hospitalizations.

The only thing we wanted for Christmas this year...we got.