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.