Bibbitty, Bobbitty, Boo, Up the Stairs With You: A Poem by Naomi

Naomi admitted tonight that the last time we watched Cinderella, she had snuck a pencil and paper up to her bed so she could sit up and write a poem. Ultimately, it was a hard secret to keep 'cause the poem was so good she just had to share it. She's very much like her mother.


Bibbitty, Bobbitty, Boo
Up the stairs with you
Daddy hugs and kisses us good-night
And says, "After this you shouldn't fight"
In the morning the sun is shining very bright
And we get dressed, make beds, and do right

We do a puzzle, read a book,
Do computer games, and help Mommy cook
After lunch we read books some more
Then we help Mommy do a chore

Then we sit down and eat our supper
We practice manners, like please pass the butter
Mommy says, "It's clean-up time," and Toby complains
Sitting down, crying, yelling, "It's not fair," and being a pain
But, the house soon gets clean, 'cause we clean up with grins
And then this whole long poem all over again begins

-Naomi Eby, age 9


She's spot-on with our daily routine, except maybe the part about cleaning up with grins, and though it sounds somewhat agonizingly cyclical, the feeling comes through that she's happy to be part of this family and she likes expressing herself on paper. And that makes Mama happy too.

I LOVE YOU: An Acrostic Poem by Hannah

I  t is good to love


L  ittle kids love their toys

O  ver the ocean there are other people that love

V  ery many people love

E  verywhere there are people that love


Y  ou should love

O  h, but there are lots of people who do not love

U  nder us is China: people love there too


--Hannah Eby, age 7

Ain't No Fwang

Toddler language development is such an amazing and joyful process. Elijah's linguistic capabilities have exploded since his ear tubes were placed three weeks ago. He talks non-stop now, straining to understand, repeating endlessly, proud to communicate his thoughts.

So far we've only hit one major hitch in his acquisition of the English language, and it's so fundamental I'm not sure how many months it's going to take to undo it, but for now, I'm actually getting more than my daily dose of laughs out of it.

You see, early this summer Elijah categorized swings, cars, trucks, trains, and other moving objects as, "WEEEEE!!!!" This was based on an entirely correct observation that adults often make that noise around moving objects. For months they were all "WEEEEE!!!!" which only solidified his understanding that they all shared a name.

Finally, I decided that my little boy should grow up and learn the proper names for moving objects. Taking him to the backyard, he announced "WEEEEE!!!!" and I corrected him, "No. SWING. It's called a SWING, Elijah. SWING." He stood silent a moment, considering the implications of this new information, then replied sweetly, "Fwang." I praised him for his great wisdom, and had no idea what I had started.

Later that night he picked up one of Toby's hot wheels cars and announced with authority, "FWANG!" I made the mistake of thinking this was cute, instead of immediately correcting it. Two months later no amount of correction will convince that child that cars, trucks, trains, bicycles, and airplanes are not to be called fwangs. They are now fwangs. End of story. Someone inform Mr. Webster.

The strange thing is, instead of getting tired of hearing this, my warped brain seems to find it funnier each time he says it.

When the school bus pulls up in front of our house each afternoon, Elijah announces, "Konk, Konk! FWANG!" (Watch out! Falling Fwangs!)

When his toy tractor tumbled down the entire length of our staircase he stood at the top and cried, "Uh oh! Fwang!" (I sure hate when I drop my fwang.)

When Grandma and Grandpa Johnson's visit ended he sadly watched them pull away. And daily he reminds me, "Bumpa ouside. Go bye-bye, fwang. Bye-bye, fwang." (Nothing worse than seeing the people you love disappear in a fwang.)

When Toby steals all the hot wheels cars for himself Elijah comes to me crying, "Toby! FWAAAAANG!" (To which I respond, "Toby, give your brother back his fwang. We don't steal fwangs in this house.)

When I carry him to his crib, he will reach desperately for any object with wheels anywhere in his sight and beg, "Fwang!" (Sure kid, have your fwang. Have any fwang you want in your crib. There's no rule against napping with fwangs.)

When Elijah wants to get out of the house he lures me with, "Come on, Mama. Fwang!" (I haven't had the heart to tell him that fwangs aren't really my thing.)

And then there was today when he asked for a turn on Toby's fwang (formerly known as a scooter) and I cracked myself up by correcting him with, "That ain't no fwang!"

Sometimes you need to stop fighting it. You need to laugh at it. A fwang is a fwang, and a fwang by any other name would never sound as sweet.

A Moment on the Pine-Wrapped Hill

One night on a pine-wrapped hill
Above the rocks, above the sea
You played what your heart overflowed
To say good-bye, you played for me

I held that tune a thousand miles
And loathed the distance, loathed the time
And wished to heaven it were now
When I could wrap your hand in mine

Now gazing back eleven years
I cannot see a sweeter place
Untouched by complicated tears
Unworn by this life's urgent pace

Tonight with dishes in the sink
With laundry strewn about the floor
With small feet sneaking for a drink
With clouds of duty on my soul

I almost didn't hear you play
I almost didn't hear you feel
'Till I saw your heart overflow
And watched the thoughts your fingers tell

Then saw the business fade to mist
And felt the notes shake through my bones
To call me out of all of this
Above the steady drone of chores

Above the rocks, above the sea
Onto a pine-wrapped hill
Where once you played to feel the pain
And now I see you play there still

One night in a pine-wrapped house
Below the moon, below our dreams
You played what your heart overflowed
Orange sun slipping through the trees

Notes that flowed with strength and hope
Contentment in the drudgery
A moment on the pine-wrapped hill
You play to feel, you play to be

Managing

Three months ago I received a phone call that completely reshaped our summer, and our lives. We had been waiting for Matt to receive an overdue promotion, waiting to be able to buy a home to live in, and waiting to feel settled somewhere where we could raise this family--the sort of feeling that doesn't come when you're in a rental home that is managed by people you don't trust. We pushed on doors that wouldn't open, and God kept us here, living in an old farm house in the center of our town's yuckiest mobile home park.

It wouldn't have been a half-bad place to live if the management had cared at all about keeping the place up: if the grass was mowed and the buildings were painted and the roads were repaired, and if I had a chance to get to know more of my neighbors. But the owners of the park lived three states away and were apparently unaware that the current management company was letting the park go to pot and laughing all the way to the bank. Our neighbors seemed nice enough, but spoke mostly spanish. My spanish was rusty at best, and I lacked the motivation to bake 40 batches of cookies in order to knock on my neighbor's doors and smile awkwardly at each other.

Our "backyard" was the common area for the whole trailer park and consisted of knee-high grass gone to seed covering litter of every dangerous variety possible: pop cans, food wrappers, and broken beer bottles so filled the yard that the lawn care company refused to mow--not that anyone cared. A twenty-year-old "swing set" sat in the middle of this jungle, just daring my children to wish for a normal childhood. On occasion I would let them put on some thick-soled shoes and venture out back, under my close supervision, but mostly we stayed inside, where I knew they were safe. Outside was out of my control, and so we waited to move. And we wondered why God kept us here.

I pushed harder on doors, determined to get us out of here. I thought maybe if I could get some extra income from home (if any actual work-at home opportunities existed that weren't total scams) we could get a loan for a half-way decent home. I toyed with the idea of working away from home one or two days a week and leaving the kids with my in-laws. We met with a counselor at a non-profit agency in town to see if their programs could help us get a house. And we were blocked at every turn. God kept us here.

On June 18th Matt informed me that some person with an out-of-state area code and a name he didn't recognize had tried calling twice, but he wasn't going to waste his time answering it. "You never know what they want unless you answer it," I reminded him, "it might be something important."

"What are the odds of that?" he retorted.

What are the odds?

That evening that same persistent person called again, and I opted to answer, against Matt's better judgement.

"Hi, is this Katherine?" he asked.

"Yes," I answered tentatively. (Not sure I really want to tell you that, strange man on the phone).

"And you live in the house at the Skyview Park, right?" he pressed.

"...Um...yes..." (Really not sure I should be giving that info out to you either, creepy man.)

"OK, well, I'm calling because I'm the owner of that park, and I'm actually wondering if you would like to manage the park for me."

Here is where my jaw hit the floor, and my brain malfunctioned from the impact:

"What in the world?!"
"Who calls people up that they've never ever met before and offers them a job out of the blue?"
"Wouldn't this be a great opportunity?"
"Can I handle all that?"
"But we were wanting to move..."
"But maybe we wouldn't have to move anymore."
"A real work-at-home opportunity?"
"No. There is no way I can handle any more stress right now..."
"But...maybe...maybe we could actually save some money. Maybe I could clean this place up and develop a good relationship with the owners and get to know my neighbors. Maybe I could like living here. Maybe this is why we're here."

And so, after my power of speech returned, we talked about it, and the more we talked, the more I realized God's unmistakeable leading in our lives, and the more excited I was at the possibility of being the new manager of Skyview. The owner revealed some of the details of the failings of the previous management and that he had offered me the job because I lived onsite, which he was realizing was essential to actually keeping an eye on the place, and because I had taken so much pleasure in fixing up this house when we moved in. He was hoping I would put some of that same energy into fixing up his park. I talked with Matt and with my parents, who all confirmed that this opportunity seemed well matched to my gifts and that it would alleviate enough stress on us to more than make up for the added stress of the position. Later that evening I called the owner back and accepted.

Why did I wait three months to blog about this? Well, first of all, I have been a whole new kind of busy for the past three months, and second, I wanted to wait a couple of months before happily announcing my miracle position just to make sure I could live through it. Three months after that phone call, I can still say that I am happy to be the manager of Skyview.

I spent the first several weeks getting bids and haggling with paving companies over fixing our pot-hole -laden roads, even before my official position began on August 1st, so that residents could sit on their front porches and watch with smiles on their faces as the pavers came through just nine days after I took over.

I put on gloves and picked up every scrap of trash covering the common area behind my house--bagfuls  of trash and glass. I dug the riding lawn mower out of the barn and from under a quarter-inch of dust, sent it off for a tune-up, and lined up a resident to mow once a week. I knocked on 40 front doors and introduced myself to the residents, in English and Spanish. I walked the park with the state health inspector, then spent a day handing out notices of health-code violations, and park rule violations. I organized a pool for residents to chip in to buy a dumpster and haul out the enormous amount of large trash items that had accumulated on most of the lots. I hauled wheel-barrow loads of yard debris and trash. I even climbed into the dumpster to make more room when it was full.

I bought bags of crushed lime and dumped them on a septic system leak, then called a crew out for repairs. I showed my neighbors what weeds were and how to pull them out. I poisoned poison ivy. I planted plants around my house. I talked the tree-trimmers in our neighborhood into giving us an entire truck-load of mulch for free. My brother and his family came one day and helped us put up a new swing set and distribute the mulch under the swing set and around my house. My dad came with his power saw and helped trim all the neglected and overgrown bushes. I got some of the teens who had been prone to vandalism involved in the clean-up work, and they learned that work can be fun and rewarding.

When the state inspector returned to check on my progress he walked the park grinning and repeating, "Wow! This is fantastic!" And I felt exhausted, but fulfilled.

I have been busy developing spreadsheets, collecting rent, documenting expenses, and communicating with the owners. I have had some unpleasant confrontations, lots of interruptions to my routine, and a few too many emergency calls, but overall, I have enjoyed the challenge.

I have enjoyed getting to know my neighbors, most of whom are a delight to be around, even if we're just smiling awkwardly at each other for lack of other intelligible communication. But most of all I have enjoyed turning my kids loose in a backyard that is a clean and safe place for them to exercise, make friends, and learn some serious soccer skills from the neighbors.

A local church donated soccer goals for us, and even Matt has enjoyed getting out for the nightly game of soccer with the neighbors that is available almost every evening now. Where else can you have the pleasure of an enormous block-party in your backyard every night?

One of my neighbors recently said to me, "You work hard. Very hard. Before no one was here. No one cared. But you fix the roads and clean the park. You do a good job."

Yes, I am tired. And some days I feel the stress. But there is enormous reward in this work. I have picked up homeschooling my kids at the same time now, and (knock on wood) I think I can do this all. Maybe I have been given supernatural help. It seems there was a reason he wanted us here.


"He leadeth me, O blessed thought!
O words with heav’nly comfort fraught!
Whate’er I do, where’er I be
Still ’tis God’s hand that leadeth me.

Lord, I would place my hand in Thine,
Nor ever murmur nor repine;
Content, whatever lot I see,
Since ’tis my God that leadeth me."
--Joseph H. Gilmore

Stalker

Having a child with a chronic, incurable, progressive disease is like having a child who has a monster stalking them everywhere they go. You know it will never leave, in fact you know with certainty that it will one day strike. Even now it lashes out on occasion and sinks its claws into your child. As she cries, all you can do is take her back to the doctor, who will measure the wounds and try his best to treat them, but everyone is powerless to stop the monster. One day he will pounce, and when he does, it will take every ounce of strength in you and your child to fight him. She may succeed in holding him off another 10 or 20 years, or she may not. But for now, the doctors say, he appears to be calm. For now, they say, "just relax and enjoy her."

Just relax and enjoy her, and try not to think about the monster.

Until it looks like he is about to fight. Then rally your strength, gather your support, bring to mind what you have learned, and let the adrenaline wash over you...this is it.

~~~~~~~

Yesterday I took Naomi for her next blood test since we first learned her kidneys had taken a decisive turn for the worst in late May. I had been reading up on the dietary changes we would need to be making soon: low sodium, low potassium, low phosphorus and so on. We seem to have something in our family genetics that predisposes our kids to dangerously high potassium levels since both Naomi and Toby had been hospitalized as infants for several days with that condition. So I was concerned that we'd hear Naomi would need medication to keep that level low. I had decided it was time to transfer Naomi's care completely to the children's hospital.  I was prepared for the worst.

When the nurse woke me with a phone call this morning I feared the worst. But, contrary to all reasonable expectation: all was well. Naomi's creatinine level, the marker of kidney function that had suddenly and dramatically risen in May and which the kidney doctor fully expected to continue rising had (miraculously?) come back down to the same level it was at last year to 0.92. Her phosphorus level was a tad on the high side, but the other electrolytes were all properly in line. Her tiny, atrophied kidneys are still chugging along, upheld I am sure by the finger of God.

I don't know what this means. I don't know which reading was the fluke. In the last year her creatinine readings have risen and dropped, then risen even higher and dropped again. This is not supposed to be the way the numbers behave. According to every doctor I've ever spoken with, the kidney decline is supposed to be gradual and predictable, not bouncing all over the place. We know for sure from her last ultrasound that the kidneys have atrophied considerably, and it is quite surprising how well they are functioning right now, given how they appear. How long they will keep going at this point is truly anyone's guess.

This is all great news, for now. But what makes it so hard to process is the "for now" part. It's difficult to know how far to let my guard down. But we are thankful to let it down, even a little bit.

~~~~~~~

Then just as you are sure he is about to pounce, he slinks back to the shadows. It appears as if the monster is being held at bay, for now. You try your best to calm you nerves once more, to relegate the monster in your child's shadow to the shadows of your own mind. For now he is calm. For now she is "healthy." Just keep your eye on him. Just relax and enjoy her...until the next time he flinches.

Photo Album: The Fair 2013

We took the kids to the 4H fair yesterday evening to look at animals, rides, tractors, sights, sounds, and horse competitions.

Grandma and Grandpa Eby gave my kids their annual treat of a ride on the carousel. Elijah was absolutely thrilled when we put him on the horse:

And he was absolutely devastated when the ride stopped. It was all we could do to pry his clinging little hands from the bar as he sobbed, "No! No!" long after the other kids had already left the ride.

Thank goodness there were plenty of amazing animals after that to distract him from his broken heart.

Here is one of the most amazing fair experiences we've ever had: Meet Ollie, the 1800lb, 4 year old, Belgian horse--a gentle giant, who only wanted to play.

Naomi was fascinated at the sight of him.

And delighted with his nuzzles. Now I know how to get Naomi to smile naturally for the camera.

A little kiss for Emma...

Ollie liked my girls so much he began kicking at his gate to get out and play.

Hannah really liked Ollie, but she wasn't so sure she wanted to nuzzle with him. I love how she's totally plowing over Elijah here, to put a little more space between that horse's mouth and herself.

And yesterday's winning photograph: Ollie licking Naomi's hair. He must have liked that strawberry scented shampoo. This smile is worth a million dollars.

There were many other amazing sights and experiences at the fair as well. We spent over an hour at the horse arena watching 4H kids compete in barrel racing and keyhole racing. Naomi and I were both spellbound--the others not so much, so Matt took them for a walk to see the lights and rides. We watched fast horses and slow horses, spooked horses, rearing horses, and one that slipped going around a barrel and went down with the rider, but no one was hurt. It was fascinating and inspiring.

Is it time for the 2014 4H Fair yet?

The Beach Through Elijah's Eyes

They took me to a place called "beach"
Another world entirely
Where floor is gritty, air is hot
Where wet is good and dry is not

A giant bathtub full of friends
With birds that flew over my head
The ceiling was too bright to see
A world where walls are made of trees

My mother sat me in the dirt
Then laughed and said it wouldn't hurt
When I decided that I'd munch
A fistful of the sand for lunch

She held me in the giant bath
And didn't scold me when I splashed
She didn't try to scrub my back
I never even took a nap

No crib or highchair in that place
No rag to wipe my messy face
I'd never had such room to run
I'd never known that kind of fun

I ran into the tub some more
And ran until there was no floor
And for a second wondered why
It hurt to breathe this kind of sky

Until I popped back up again
In Mommy's arms and saw her grin
And with the floor and ceiling back
We shared a coughing sort of laugh

I thought it would be funny then
To dunk and cough and cough again
But Mommy frowned and called me "dense"
I think it's her that makes no sense

I didn't want to leave that place
When Mommy carried me away
She tricked me with a sippy cup
So cold and sweet...
Then I woke up

And cried to find myself at home
The land of "beach" completely gone
My mommy tried to soothe my cries
To hush my sobs and wipe my eyes

I fought to find the word to say
To tell her how I missed that place
But as my sobs grew stronger yet
All I could think to say was, "Wet."

The Beach

I went to the beach
because I thought
my children needed
more practice swimming
Duty-bound
I gathered the means
And slathered the cream
And felt exhausted
Just beginning








But the cool waves' kiss
and sea gulls' greeting
and the wandering puffs
in the blue abyss
Loosed the choking chains
Of duty
Freed my child's spirit
to hear them laughing











I went to the beach
Not knowing all the while
It was I who needed practice
Remembering how to smile

Splash Park

A friend and I took our kids to a local splash park today. The kids had a wonderful time--once they warmed up to the idea of freezing cold water blasting the senses out of them.

They also had a blast running down a hill with their towels trailing behind them, because what kid hasn't loved trying to fly?

Ah, but it seems no day of fun can finish unmarred for Naomi. After the first race down the hill all the kids ran right back up to do it again, but Naomi stood at the top, holding her sides while all the other kids ran down, because any amount of exercise is painful for her now. Her liver is quite enlarged now, extending a full 3 inches below her rib cage (which is a lot of extra liver for a little girl to carry), and I'm sure it is heavy and sore when she runs. She stood sadly, watching all the other kids laughing and running. She wiped sneaky tears away when she told me, "It just hurts too much any time I run at all." And I didn't really know how to answer her.

This picture is zoomed and cropped from the picture above it, so you can see Naomi standing by herself

Every day there is a shadow over us now. I just hope she remembers the sunshine and not the shadow.

Groan

Today I felt creation groan

Burdened and calloused

Under the weight of decay

And from those inept words

Glibly passed over the phone

The angst held back nine years

From safe enclosure found its way

To this mother’s heart

Trembling to be that brave

We knew his work insidious

That specter from her birth

But clung to hope his gentle words

Could turn the demon friend

And prayed to heaven that the curse

Be eased before the end

Before her heart should meet the ache

All earth has mourned to bear

Since the day you barred the gate

And left your guardian there

Today I felt creation groan

But from fierce sorrow

Sprang a hope

Fiercer than I’d ever known

When this world seemed

As if a home

Now shaken free from that deceit

I hold her in my arms and wait

The day that you return to free us

Even so

Come Lord Jesus

Grieving

Grief is an irrational thing. It does not respond well to reasoning, to soothing, or to being ignored. It lives on its own timetable. It will take your thoughts and your emotions where you don't want to go, at exactly the moment you don't want to go there. Grief must be looked in the eyes and walked through, or it will not relent.

Raising Naomi, I have walked through the stages of grief again and again, because each new phase brings with it new losses, new fears, and new realities. And I have found that I more fully adjust when I allow myself to voice and grieve those losses, fears, and changes.

There was the initial grief of a questionable prenatal ultrasound. Then came the intense grief a having my newborn taken from my beside and admitted to the neonatal intensive care unit, not knowing if or how she would come home. This was followed by the grief of adjusting to the idea that my child would be "special needs," something I didn't have much precedent for among my family and friends. When my ultrasound with Emma again showed bright kidneys I grieved the realization that we were indeed dealing with a serious genetic defect.

When Naomi and Emma finally received the diagnosis of ARPKD/CHF I entered my most prolonged grieving period to that point--grieving the unescapable severity of the illness that plagued my two daughters. I had to come to terms with the reality that my girls would only grow more and more ill, could only be supported as they declined, and that even modern medicine's wonders of dialysis and organ transplantation were only buying us time--hopefully many more happy years, but limited time. My daughters' childhoods would be shadowed by specialist appointments, invasive tests, and terrifying complications. If my daughters reached adulthood they would likely not marry, not bear children, not live independently--at least according to the information available to me at the time. More than all this, I grieved the realization that (barring my early death or advancing medical miracles) I would one day bury my daughters--maybe in their childhood, or maybe, if we were very blessed, in their 50's, but one day it was coming.

My understanding of the disease and my hope for the future has grown since that time five years ago, as I have met more and more families, and even adults, living with ARPKD/CHF. There are exceptions out there, and I began to hope--as anyone would--that my daughters would be those exceptions who marry, have children, and live well into adulthood.

I have had a relatively long period with relatively little grieving since that time, with the exception of each specialist appointment or battery of tests that would renew the fear in my mind: is this the appointment? Is this the test where we find something severely wrong? The irony is that when I was most worried, the news was, for the most part, fine. I was not prepared to receive that phone call out of the clear blue sky ten days ago.

I was thinking about swimming lessons and room rearranging. I was worried about watering my flowers, and making sure I had pancake mixes stored away in my cupboard, when the phone rang. It was the nurse from the pediatric GI office at the children's hospital we'd visited a week ago. She was calling with Naomi and Emma's blood work results. "Dr. M wants you to know that everything looked really stable with the girls," she announced cheerily, "except that Naomi's creatinine looked a little more elevated."

A little more elevated? It rose more in the last six months than it has risen in her previous nine years of life. And with that cheerfully-delivered bit of news everything changes. All the previous worries about teaching Naomi to swim, or ride a bike this summer; all the thought about what this fall's homeschool curriculum should be is suddenly eclipsed by the monstrous fear that had been lying dormant these last five years: imminent kidney failure.

When the nephrologist confirmed my fears and began to discuss the transfer of Naomi's care to the children's hospital and the process of preparing for transplant I wasn't sure how to feel. On the one hand I knew this was coming someday, but on the other hand, I had begun to hope that Naomi would reach adulthood before her kidneys failed. Suddenly having talk of a transplant injected into the here and now of our lives was more of a shock to my system than I expected it to be.

Yesterday I took Naomi for an ultrasound of her kidneys, and the nephrologist called this morning to discuss the results. He opened with, "Well, to be honest, this report is so different from all the previous reports, I don't really know what to make of it." The report didn't mention anything about bright, cystic kidneys, instead talking about how atrophied and scarred both kidneys were. The doctor and I believe that what the radiologist is seeing is basically the death and collapse of the nephrons (filtering units) in Naomi's kidneys. The process is advanced: it is irreversible. Before at appointments we discussed possible medications and dietary changes to slow the process, but the nephrologist clearly believes we are now beyond that point: what will happen will happen. We are just watching it now and preparing for transplant. When I asked about timetables, given this report, he still didn't want to guess, but I am becoming more convinced that these little, scarred kidneys will not carry Naomi for more than a year or two.

My emotions in the last ten days have been at war within me. Instinctively, a part of me feels like I just want to hurry up and get this over with, like it would be better if Naomi's kidneys failed sooner and she had a transplant and we could all move on, without this dread over our heads. But this emotion is always quickly followed by the sad reality that there is no "moving on" after transplant--not in the sense that we can put it behind us, stop our worrying, and get on with our lives as normal. After transplant there will be lots of close follow-up care, and new worries of rejection, complications, or infections. Transplanted kidneys do not last forever either: 10-15 years is a typical lifetime of a transplanted kidney. If Naomi lives well into adulthood, she could potentially need two or three kidney transplants. There will also be continued liver fibrosis complications. Coming to terms with all of this only brings me full circle to feeling like I want transplant to be as far away as possible, like I want to milk the sweetness out of every day that Naomi has until that point. I want to spoil her and enjoy her and take pictures of her feeling well and healthy, because I know it is only downhill from here until transplant.

It is difficult to reconcile these warring emotions: thankfulness for the health Naomi has now, wanting to enjoy this time, and being filled with sorrow over what is ahead. When I see her grinning at swimming lessons and sitting tall on her horse at LoveWay I am at the same time so filled with joy and sorrow that my chest literally hurts. This is heartache. This is grief. This is life outside of the Garden of Eden. All humans feel this. Something has gone very wrong in this world. We were not created for this place. And this feeling, as heart-rending as it is, creates in us the most beautiful emotion of all: longing for home.

A few nights ago I laid in bed beside Naomi as I tucked the kids in, and I sang. I didn't sing a quick song so I could run out of the room and have some "me time," as I so often have done. I sang song after song about heaven, about our hope, about our comfort in this world, and I let myself feel the sorrow and the hope as I sang. I started with a favorite song I had learned at Cono:

There is a higher throne
Than all this world has known
Where faithful ones
From every tongue
Will one day come

Before the son we'll stand
Made faultless through the lamb
Believing hearts find promised grace
Salvation comes

Hear heaven's voices sing
Their thunderous anthem rings
Through emerald courts
And sapphire skies
Their praises rise

All glory, wisdom, power
Strength, thanks, and honor are
To Christ our king
Who reigns on high forevermore

And there we'll find our home
Our life before the throne
We'll honor him with perfect song
Where we belong

He'll wipe each tearstained eye
As thirst and hunger die
The lamb becomes our shepherd king
We'll reign with him

Which I followed by the chorus of a song my mom used to lead our church choir in singing when I was a little girl:

No more night
No more pain
No more tears
Never crying again
Praises to the Great I Am
We will live in the light of the risen lamb

Then the Chris Tomlin song:

There's a peace I've come to know
Though my flesh and heart may fail
There's an anchor for my soul
I can sing, "It is well."

Jesus has overcome
And the grave is overwhelmed
The victory is won
He is risen from the dead

And I will rise
When he calls my name
No more sorrow
No more pain

I will rise on eagle's wings
Before my God
Fall on my knees
And rise
I will rise

And last, the hymn, which I sung to the Rich Mullins' tune that I like much better:

All the way my Savior leads me;
What have I to ask beside?
Can I doubt His tender mercy,
Who through life has been my Guide?
Heav’nly peace, divinest comfort,
Here by faith in Him to dwell!
For I know, whate’er befall me,
Jesus doeth all things well.

All the way my Savior leads me,
Cheers each winding path I tread,
Gives me strength for every trial,
Feeds me with the living bread.
Though my weary steps may falter,
And my soul athirst may be,
Gushing from the Rock before me,
Lo! a spring of joy I see,

All the way my Savior leads me;
Oh, the fullness of His love!
Perfect rest to me is promised
In my Father’s house above.
When my spirit, clothed immortal,
Wings its flight to realms of day,
This my song through endless ages:
Jesus led me all the way,
This my song through endless ages:
Jesus led me all the way.

"Mama," Hannah said sweetly when I was done, "I always just think your voice is so nice to listen to. My favorite part is, 'He'll wipe each tear-stained eye.'"

"You know, Hannah," I answered softly, "that's my favorite part too."

Cascade

Today Matt got called in to work, so I started the day with packing a diaper bag, a medical binder bag, a lunch, water bottles, and coffee. Then I drove 5 kids 90 minutes to Naomi's nephrologist. He said the rise in Naomi's creatinine was too big to be due to discrepancies in laboratory equipment alone--in other words her kidney function has definitely declined, at least some, since last December when it was last checked. He felt it was probably the beginning of a more steep decline in function now. He explained it like this:

Every person has a certain number of glomeruli--tiny tufts of capillaries which carry blood within the kidneys, and which filter or remove toxins from the blood. Most people could lose half of these glomeruli, (one of their kidneys) and never notice the difference because we are born with way more than we will ever need. Naomi was born with kidneys that were filled with thousands or millions of microscopic cysts which have rendered many of her glomeruli useless. When she was four years old we were told she still had about 60% of her kidney function, meaning about 60% of her glomeruli were still functioning, which was plenty to carry her through the last few years. But ARPKD is a progressive disease. The cysts continue to form and they continue to knock out the glomeruli with them, which causes a progressive decline in kidney function. Children with ARPKD progress to end stage renal failure at vastly different rates--some need dialysis within days of birth, while others have made it into their 20s and even 30s before needing dialysis or transplant.

All this I knew before, but what came clearer to me today in his explanation was this: there is a sort of tipping point for many kids where they hold steady for many years with their 60% or so of kidney function, where the kidneys are chugging away at a normal rate and able to handle the demands on them normally, but when enough glomeruli have been destroyed that the remaining 40% or so have to work harder than normal to keep up with demands it can actually cause the demise of the remaining glomeruli to occur faster. It's like a man trying to work double-overtime for months on end--sure he can do it for awhile, but eventually he's going to burn out.

It appears that Naomi's kidneys have hit this tipping point, where the demand is greater than they are able to comfortably meet. This starts the cascade: the kidneys work harder to meet the demand and more and more glomeruli begin to burn out, which only further stresses the kidneys, which only causes their demise faster. How fast she progresses to end stage renal failure, and needs dialysis or a transplant is still a guessing game. Just as one man can work double-overtime for years on end, while another burns out in a matter of months, so it goes with kidneys. I think Naomi's nephrologist would guess that we have a few years--maybe two, maybe six--we really don't know, but I don't think anyone is expecting decades anymore. The hope of Naomi reaching her 20's or 30's without transplant is pretty much out of the question now.

Right now he wants an ultrasound of Naomi's kidneys, since it's been over two years since her last one, just to make sure we don't see anything terribly abnormal (well, besides what we already know is terribly abnormal about them). Then we will repeat blood work in two months. We will not only be watching Naomi more frequently now, but also more thoroughly. Her kidneys are now at the point where their poor function can cause other problems to arise, such as anemia, parathyroid dysfunction, and electrolyte imbalances. I will probably be taking Naomi to the nephrologist every three months or so for the near future.

For the first time today we discussed what will happen when Naomi gets closer to transplant. Our current nephrologist does not work at a hospital that can handle pediatric transplants. He is comfortable following Naomi until either her creatinine reaches 4.0, or her symptoms become complicated by anemia, fatigue, or other renal failure issues. At that point we will have to transfer all of Naomi's care to the children's hospital that is 3 1/2 hours from our house, and we will have to get used to living on the road. This is the hospital where Naomi's GI doctor is, and it may benefit her to have all of her care coordinated at the same place even sooner, but that is something that is really up to me to decide.

Yet another decision we will have to make as this all unfolds is whether Naomi would benefit from a combined kidney/liver transplant or whether to transplant just the kidney and hope that her liver holds out without major complications. We know she currently has portal hypertension. We know this is causing her white blood cell counts to drop, and puts her at risk of episodes of internal bleeding or life-threatening infections. The question will be, as we get closer to kidney transplant time: is her liver going to be in need of transplant as well in a short amount of time and would it be better to just do them both at once? I know of at least one boy who had only a kidney transplanted, and has spent the last nine months since transplant battling one liver-related complication after another and is now looking at liver transplant less than a year after kidney. These are very difficult decisions to make, but the two best experts on ARPKD/CHF have recently released a paper recommending that children with significant liver involvement should receive combined transplants. The question will be, is Naomi's significant enough to warrant that?

There will be other questions to face as well: should we use a living donor or deceased donor? Who should be tested for donor compatibility? Should we get used to commuting or look for temporary housing nearer to the children's hospital? And the list goes on... But those questions are for another day. We could possibly still have five or six years (maybe more?) before we need to face them.

Today Naomi feels great. She's playing "fairies" in our backyard with some neighbor kids as I type. She's excited to be in swimming lessons. She's looking forward to going back to LoveWay for horse riding next week. She's a normal kid, in so many ways, and I have to remember to be thankful for the last nine years of "health" that she has had. They have been a blessing.

I'm not sure how to feel about all of this quite yet. It is sad to let go of the hope that Naomi would reach adulthood before this all happened. It seems very overwhelming to think about, yet could be so many years away that I can't get all bent out of shape over it just yet. The next round of blood work in August will tell us more about how steeply the decline is happening. If the creatinine stays the same, we may be able to breathe easier on another plateau for awhile. If it is steadily rising, we will have to buckle up and hold on. Time will tell.

The Inevitable Decline

Since we pulled food dyes from Naomi's diet in May 2011 she has enjoyed an amazingly long period of relative health. She does have a cyst in the pineal gland in her brain, which warranted five MRIs at the children's hospital to watch for growth, and a spinal tap that landed her in the hospital for a week in September of 2011, but other than that, things have been relatively calm.

When Matt and I stood over her bassinet in the Neonatal Intensive Care Unit nine years ago and listened to the nephrologist inform us that out tiny new daughter would one day need a kidney transplant, we pushed him for times. He didn't want to make predictions, but his best guess would be around the age of puberty for Naomi because her kidneys had only a limited amount of function in them, and as children tend to add more body mass with puberty, they often outgrow their kidney function at that time. Nine years ago that seemed like the wonderfully distant future.

We have waited and watched, and been constantly thankful each time Naomi's kidney function is checked and shows that it is holding steady at around 60% of normal. Her ultrasounds and CT scans always remark at how abnormal her kidneys look. They are full of cysts and scar tissue, and are far too small for her size. A CT scan report from 2011 reads, "The kidneys are diffusely abnormal. They are malrotated. There are multiple right renal lesions. There are multiple areas of bilateral renal cortical thinning, suggestive of prior episodes of infection/infarction." Her last ultrasound report summarized the findings as, "Atrophic, hyperechoic bilateral kidneys," (which means they have atrophied and are filled with microscopic cysts that make them appear bright on the ultrasound image). And yet her little, browbeaten kidneys have chugged on. We have always known renal failure, dialysis, and transplant were coming, but in some ways, we'd almost forgotten.

Last fall Naomi suddenly began to gain weight. We searched for answers--looking at possible fluid retention, checking her thyroid gland, and even locking up the snack foods at our house so she would have less temptation to overeat--yet she continued to gain. Last Tuesday I took the three girls to the children's hospital for their six-month check-ups on liver disease and celiac disease. There the GI doctor confirmed what I had begun to suspect--Naomi is showing some possible signs of early maturation. (...Insert heavy sigh here...) This could be "normal" as many girls are maturing at an earlier age now, but she only just turned nine, and I have a hard time seeing that as anything close to normal. It is also possible that that cyst in the pineal gland has grown or is causing the secretion of hormones too early, so the GI doctor wants me to check with the neurosurgeon who had followed the cyst and also to send Naomi to see an endocrinologist.

This is bad news all on its own, but grew worse yesterday when the nurse called me with last week's lab results. Naomi's creatinine (a marker of kidney function) has suddenly risen over the last six months, which means that (unless we're dealing with a lab error of some sort) her kidney function has declined quite a bit. When she was a baby her creatinine sat around 0.7 (somewhat higher than normal, but pretty good for her condition). As she's grown it has slowly risen over the past nine years to 0.9 (still not too bad). Last week her creatinine came back at 1.24, which is a sudden 30% increase and could (trying not to jump to conclusions here too quickly) put her kidney function down from her "normal" of 60% to more like 40%. End stage renal failure is between 15 and 20%, where the creatinine level would likely be around 3 or 4.

I am afraid that as Naomi has gained weight (and possibly even entered puberty) that first nephrologist's predictions are coming true--she may finally be outgrowing her limited kidney function. How fast she will outgrow the remaining roughly 40% of function she has is still a guessing game. I have heard that it can happen in a matter of months or over several years. Today I am waiting for a call back from her nephrologist to see if he got these lab results from the GI doctor's office yet or not, and to see what he wants to do now. I am guessing he will want to see Naomi in his office and repeat the blood work, since different lab equipment can sometimes give slightly different results, and that he will need to watch her more closely now.

Today I am re-imagining what going through a kidney transplant would look like for Naomi. She is still so young for that. I am praying that it was a lab error, or that we can slow her weight gain, or that there is a medication they can prescribe to slow down the deterioration--praying for a few more years. I am praying for some sort of good news. And I am waiting.

Healing Naomi

When we moved in with Matt's parents in the summer of 2009, our family's diet went downhill right along with my morale. There were enough stresses with losing our jobs, losing our house, finishing a dissertation, and moving in with the in-laws, that cooking healthy meals for the family was honestly the last of my worries. When we lived at Cono, we ate two (relatively healthy) meals a day in the dining hall.  But starting the summer that we moved our diet became loaded with boxed mac and cheese, frozen pizzas, hamburger helper, chicken nuggets, and the like--if it was quick and cheap we ate it for over a year. What I didn't correlate at the time was how Naomi's health declined in perfect parallel with our diet.

Her social skills (which had always been a struggle) took a nose dive, she had a sudden spike in horrific temper tantrums, she had trouble at school and needed constant discipline at home. She had a lot of stomach aches. Nine months later she began to be plagued by joint pain and headaches. In September of 2010 Naomi was diagnosed with Celiac disease and we pulled all gluten from her diet. We also pulled dairy in the hopes that it would help as well. Naomi improved in many ways after these changes: the stomach aches lessened and her energy rebounded. But in many ways she grew worse.

The tantrums grew so severe that dealing with the hour-long fits of rage multiple times a day nearly consumed us. She would scream with such force that she burst tiny blood vessels in her face, she would spit and foam at the mouth, she would break items--all this even though we had never, ever, given into a tantrum in her life. We consistently and firmly disciplined the behavior, but it did no good. Her first grade teacher was at a loss. Naomi was brilliantly smart and enrolled in their high-achievment classes, but she refused to participate. She caused disruptions in the classroom by constantly kicking her legs into desks while she worked, not cooperating with transitions between subjects, and even physically fighting with other kids. Matt and I were told that we might have to sign a release for Naomi to be physically restrained if she posed a danger to herself or others.

I felt like we were losing her. She had two completely opposite sides to her: the very sweet, very bright, very eager to please side--which we were seeing less and less of; and the other scary girl who would suddenly take over her body and seemingly cause behaviors beyond her control. When the scary child would finally fade away I would find myself staring into the face of a six year old girl with dark circles under her eyes, bright reds specks from burst blood vessels all over her face, and eyes full of weariness and remorse. She never could remember much of what had happened, only that she hated it as much as we did, but she was powerless to stop it.

In January of 2011 when we moved into our own house, we pulled Naomi from public school and began to homeschool her. This was an incredible relief to Naomi and brought much behavior improvement, but the joint pains and headaches became nearly debilitating. She cried daily in pain, avoided the stairs, avoided active play, and buried herself in books. In May 2011 her formerly benign heart murmur worsened and her mitral valve began leaking. What I didn't know then was that Naomi showed many signs of chronic inflammation. But I did finally draw the connection between birthday parties and an intense worsening of all of Naomi's symptoms. The cakes were already gluten and dairy free, so I pinpointed the culprit as the huge amounts of food dyes in the frosting. When we pulled all food dyes from Naomi's diet (strawberry Nesquick and "fruit" snacks were two big sources) the headaches and joint pain disappeared almost completely within two days. It felt like a miracle. But even more miraculously the temper tantrums vanished as well.

Within two days of pulling food dyes our daughter returned to us: attentive, affectionate, eager to please. Two years later I can still affirm: if we keep her diet free of gluten, dairy, food dyes (and other foods with high phenol content), and most preservatives, she remains a "healthy" eight-year-old girl. And if she accidentally gets a dose of any of these things, we can tell almost immediately.

Now here is more wonderful news for Naomi, just in from the cardiologist this morning: remember that heart valve that had begun leaking? It's not leaking anymore! He can't say why it started leaking. "The leaflets of the valves look normal," he had told me. And he can't say why it stopped leaking, just that it's good news and we don't need to see him anymore. I think I can tell you why though: chronic inflammation can cause heart valve damage, and resolving that inflammation can allow the valve to heal. Here's a link to a Wall Street Journal article that is a great read on this subject.

Why do I blog about this? Because I want you to celebrate with me. Naomi is healing! She will not heal from her kidney and liver disease, which is caused by a genetic mutation, but she is healing from all the assaults of her environment that have added insult to injury in her young body. But I also want you to share this information: there are many, many children who, for whatever reason are very sensitive to the foods they eat.

Do you know other parents who are struggling with children who have chronic pain and wild tantrums? Tell them that diet changes can help. Many parents of children with ADHD, Autism, and other psychiatric diagnoses swear that diet changes resolve symptoms. Lastly, if you see a child throwing wild, out of control tantrums in public, who is far beyond the normal age for tantrums, please don't assume you are witnessing the results of bad parenting. Sadly, it is more and more common now for children to have physiological, neurological breakdowns due to food toxicity, and it is safest for you to assume you are witnessing this kind of meltdown. Be informed, be part of the solution. Kids are what they eat. Some just show us in a more obvious way.

Today I feel encouraged: it's worth it. All the months of special diet shopping, and special diet planning, and special diet cooking are worth it. Naomi is pain free, she is pleasant, and beautiful, and her heart doesn't leak anymore. It's worth it.

Fresh Air

The fatigue had crept over me so subtly I hadn't realized how bad it had become, only that I longed to sit at my computer, and that it was taking more and more coffee just to get me through each day's basic chores. Sometimes when you have been tired for so long you forget what tired feels like, you forget how to shut down, relax, take a nap, or smell a flower, but you don't realize that you've forgotten. I began coaching myself on, bribing myself, "Come on, Kathy! Get up and do one load of laundry and then you can sit down again with a cup of coffee." I was secretly happy that the winter weather hung on so long, because I felt exhausted just thinking about spring time. How in the world could I handle taking the kids swimming and biking and to all the other outdoor activities that warm weather required when I was barely keeping it together sitting in the house day after day? Then there came a point where the coaching and the coaxing couldn't make this body go any longer, when I was just done.

I haven't blogged in a month because I've been so busy, but it has been a very different kind of busy than the previous year--I have been busy healing. This month I have been busy having surgery to remove an inflamed gallbladder with gallstones, and healing from the surgery. But even more, I have been busy reassessing my life--my daily habits, my priorities, my goals. I have been busy letting other people take care of me, remembering how to relax and renew my spirit, remembering the joys and the goals and the passions I had before I had five children, and trying to find ways to incorporate all these into my life now. Thanks to the generosity of my parents, my husband, my neighbors, and my friends, I have been taking naps, visiting coffee shops, reading good books, shopping for new clothes (yay for Goodwill!), going for long walks outside by myself, and even going on a few dates with Matt.

People have been asking how I've been doing. The first week after surgery was really rough. The pain was not so much a problem as the nausea and the burden of wondering if I had done the right thing. But slowly I began to feel better, the nausea left and I told people, "At least I'm not any worse than before surgery."

Two weeks after surgery I had a few days where I felt like I might have some extra energy, but they were always followed by more tired days. The chest pains and nausea finally stopped completely. I dared to hope then that I was on the right path.

Now, just over three weeks from surgery I can announce (knock on wood here) that I am feeling much better! If I get nine consecutive hours of sleep per night (and it has to be nine, eight doesn't cut it yet) I can actually wake up feeling well rested--a feeling I had almost forgotten entirely. I feel ambitious, like I want to paint the ugly back door, and clean the minivan, and scrub the shower, and cut Toby's hair all before lunch. Of course, making breakfast, getting a shower, and keeping Elijah from killing himself is usually more than enough to fill the morning, but at least I finally feel energetic doing those things. I have actually had mornings where I got all those things done and then realized I hadn't even thought about drinking coffee.

Two days ago, when the warm weather swept in. I spent the morning removing storm windows and putting up screens. I took the window insert out of our back storm door and fastened the ghetto screen insert in place. I duct taped the loose edges and the holes to move it from the "ghetto" category to "redneck"quality and felt pretty good about my achievements as the warm breeze swept in through the few remaining porous surfaces. But yesterday I got even more ambitious. Matt offered to clean up from dinner and put the kids to bed! (!!!) So I ripped that screen door back off the frame, and I got out my hammer, pliers, screwdrivers, wallet, and car keys. Two trips to Menards later, I had all the supplies I needed to improve that screen door ("fix"would probably too generous a word for what I did). I spent three hours plying all the tack strips off the edges of the frame, ripping up the old screen, pulling out at least fifty tiny rusty nails, stretching the new screen, and tacking everything back in place, but by 10:30pm our screen door had been promoted from "redneck" to "country-bumpkin," a category with which I am much more comfortable. And it felt good. It felt good to be outside, to be productive, and to use my mind and my hands for more than the daily grind of keeping everyone alive.


Today I feel bright and ambitious again. I made a huge pot of baked beans, put a roast and potatoes in the crock-pot, cleaned the kitchen, and kept Elijah off the kitchen table for an entire morning (with the last of those being by far the most challenging). I have big plans to cut the boys' hair, mow the lawn, finish switching out the fall to spring wardrobes and maybe even paint that country-bumpkin back door.

I have plans for this summer now too. I felt more excitement than dread when I went this week to sign the girls up for swimming lessons. I picked up a brochure at the Park and Rec office about local nature trails and took the kids for three long nature walks. We studied the signs of spring that we saw and wrote a poem about them, and I can't wait to go back again--after hair-cuts and lawn-mowing and painting, of course.

It feels wonderful to feel much more like myself again. I can't say for sure how much of my recovery is due to gallbladder surgery and how much is due to the generosity of people around me who have helped to shoulder the burden for awhile and allow me to rest and re-prioritize. Whatever the case, I have a really long list of things I look forward to getting done today, and I can't wait to get out from behind this computer and get going. If you've prayed for me, thought of me, or helped me out this past month, thank you! Maybe I'll eventually have the energy to return the favor.