What Tomorrow Holds

Little Baby,

Tomorrow the world will decide
Whether you are worthy of life
They will use their best instruments
To painstakingly measure
Each tiny feature of your forming body

They will compare what they find
With the millions of others they've studied
They will make their best educated predictions
As to how long and in what way
You would live on this earth

And they will base the value they assign to you
On the numbers they have gathered
They will advise me to keep you alive
If they predict that you will live a long life,
Feel little pain, and cause me little discomfort

They will advise me to end your life as soon as possible
If they predict that you would not live to a full life expectancy,
That you may have to endure suffering,
Or that it may difficult for me to walk beside you
As you struggle through your life

Little Baby,
Tomorrow I will love you
As I have loved you today
Tomorrow I will protect you
As I have protected you
Since the day you were sent to me

Perhaps I will breathe a sigh of relief
When the medical experts predict
A long healthy life for you
And an easy, predictable road for me

But if they deem you unworthy of life
If they advise me that you would prefer not to live
And that the world would benefit
From not having to meet the challenges
Your unique life would present it
I will fight for you

I will fight for you
Because life's value is more than an equation
Than can be computed from the days of life
One is predicted to live
And the degree of hardship they are predicted to face

I will fight for you
Because my love for you is not founded
On what you can offer to me
Because love does not recoil at the prospect of pain
Or abandon when another suffers

I will fight for you
Because ending your life early
Would rob you of the chance
To see the sun's rays peeking
Between the ominous clouds
To learn the blessing of enduring
When everyone tells you to give up
To know the peace of resting in warm arms
That would not leave you in your darkest hour

I will fight for you
Because the world may not know that it needs you
But I know

They have forgotten that life without trials
Brings laziness, discontentment, and self-centered greed
They have forgotten that in giving ourselves
For the helpless, the hopeless, the defenseless and innocent
We learn patience, endurance, thankfulness, and selfless love
In laying down our own hopes, and sacrificing ourselves
We find greater joy than our own dreams
Could ever have brought us
I learned this when I fought for your sisters' lives
And I pray that I can teach them as I fight for yours

Little Baby,
Tomorrow we only learn their best guess
As to what sort of life we will share
But I promise our ways will not part
Until the hands that placed you in my safe arms
Reach down to lift you back to His

Low White Cell Counts: Follow-Up

I just spoke with a nurse who finally released Naomi's labs to me. The white cell count rose slightly to 4.2, well below the minimum 5.2, but increased enough that it is not dangerous. We will just continue watching, probably with another count in a month. We do not know for sure what is causing the low counts, but it isn't low enough to warrant lots of investigation. I guess we just pray they continue to climb without intervention. Thanks for your prayers.

Low White Cell Counts: Naomi's Next Challenge

I'm spending another afternoon waiting for test results today. Naomi's white blood cell counts have been progressively dropping over the last five months. White blood cells counts are supposed to be between 5.2 and 14.8 K/mm3. In February Naomi's levels were 4.7, in May 3.6, and in June 3.0. In June Naomi had a terrible time getting over a stomach virus that went through our family. She had awful diarrhea for a week and lost four pounds, then was excessively tired and listless with low appetite for another two weeks. This is almost certainly due to her body's lowered ability to fight infection without the proper number of white cells.

We had another Complete Blood Count drawn yesterday, and I am anxiously awaiting word now. It's difficult not to speculate about what all this means. I have a detective's mind that is endlessly trying to draw connections to help understand my daughters better. Sometimes this gets me in trouble, but sometimes I am right and my thoughts prove valuable to helping the doctors treat my children. As writing seems to be my best therapy, I'm writing out my thoughts today.

I was told that if the counts dropped below 3.0 at all it would be considered dangerously low and we'd be referred to a hematologist for follow-up. The most likely explanation for the low count is that Naomi's liver fibrosis has progressed to where the blood in the portal vein cannot easily flow though the liver, causing portal hypertension (high blood pressure in the portal vein). This is an extremely common result of congenital hepatic fibrosis. The pressure in the portal vein causes the spleen to enlarge and small blood vessels in the esophagus may burst, causing life-threatening bleeding. The spleen under pressure may begin to sequester or trap platelets and/or white blood cells. Typically an enlarged spleen with low platelet counts are the first signs, but a slightly enlarged spleen with only low white counts can be the first sign as well.

Obviously, there are other explanations for low white cell counts including immune-system disorders where white cells are destroyed in large amounts (since Naomi has one auto-immune disease already this is not entirely out of the question), and bone-marrow disorders where white cells are not produced in sufficient quantities. However, I think the theory of the spleen trapping the white cells because of portal hypertension is most likely.

If this proves to be the case we are probably looking at four options:

1) Shots of a medicine that forces the bone marrow to produce more white cells, though if the spleen is just going to trap these again this seems an unlikely solution.

2) Surgery to place a shunt from the portal vein to another major vein. This allows some of the blood in the portal vein to be rerouted off of the backed-up portal "highway" to a less-crowded side road that leads to the body's main "interstate highway." This is an effective way to relieve pressure on the spleen and the vessels in the esophagus. It is proven effective at restoring blood cell counts and preventing esophageal bleeds. The problem is that the blood in the portal vein was bound for the liver for a reason: it is full of toxins that need to be filtered out by the liver before being released to the rest of the body. When a shunt sends portal vein blood to the main vascular system again these toxins can reach the brain and cause slowed brain waves (hepatic encephalopathy) in 1/3 of shunt patients. Naomi has enough issues with clear thinking already; I am not anxious to add encephalopathy to her troubles.

3) Removal of the spleen. This would restore normal blood counts, but does not lower pressure in the portal vein and so does not prevent the life-threatening esophageal bleeds. When the spleen is otherwise healthy and normal sized this is often not the option of choice.

4) Liver transplantation. Now that organ transplantation is becoming more common and successful, and now that it is possible to transplant one lobe of liver from a living donor this is fast becoming the treatment of choice. The fibrosis does not recur in the donor liver, portal vein pressure is restored to normal, the spleen can remain intact, blood counts return to normal, and there is no longer a risk of esophageal bleeds or the liver infections that often plague kids with hepatic fibrosis. Of course, there are the draw-backs of major surgery for both donor and recipient, life-long immune suppression therapy for the recipient, and the possibility that the liver may be rejected or may need to be retransplanted later in life.

As I wait for the phone to ring, none of these options sound appealing to me. I would like to hear that Naomi's white cell counts have inexplicably returned to normal levels and that no further follow-up is needed, but that probably isn't what I'm going to hear. I will post again when I have more information.

Toby Wins the Word Battle

Yesterday Toby was shadowing my every move as I cooked dinner, as he always does. I turned my back for a minute, and he cautiously picked up a knife from the table. Emma spied this from across the room and yelled, "No! Goby! No ay!" (No Toby, no knife). I turned to Toby and scowled at him with a "put that down right now" look.

Toby was disgusted with Emma and fired angrily back at her, "Go away, Emma. Why don't you go play with toys!"

I stood a moment processing what he had just said, but it didn't take me long to figure out where he'd learned that language. I think I've used those same words to him when he was driving me nuts before. His amazing ability to remember phrases and idioms and use them correctly with appropriate intonation at the age of 2 years, 4 months leaves me astounded every time. It might be time for me to start watching what I say to him.

Tonight Toby dragged a bag of toys into my office and announced, "I've got myself and my bag!" Yourself is all you're ever going to need, Little Buddy.

A Letter to a Mother Considering Terminating a Pregnancy for ARPKD

I joined the yahoo group for ARPKD/CHF (the genetic kidney/liver condition that Naomi and Emma live with) just a few days ago in order to post a question about Naomi's recent low white cell counts. Now I have found myself in a world with hundreds of others affected by this disease. Most are asking questions like mine, or sharing support and comfort, but this morning a mother posted that she had just received the news that her 13 week gestation unborn baby has ARPKD/CHF (definitive genetic testing had been done). She is considering terminating the pregnancy in order to avoid the otherwise inevitable suffering of her child. Many on the message board have also chosen that option, so I chose my words carefully, but I just could not remain silent. The following is my response. Please pray for Emma as she makes this most heart wrenching decision.

Emma,

I am so sorry that you received this news. Please know there are hundreds around you who have been in this same or a very similar position. We know the pain that facing this decision brings you. Many others before you have followed the advice of doctors, family, and friends to terminate such a pregnancy. I understand that the decision they make is almost always out of the highest love for their child and a desire to prevent suffering. I want to be very sensitive to that, but to also encourage you to look from a different point of view.

It seems to be a foregone conclusion in our culture that preventing suffering is the highest goal, but I think we lose sight of the fact that sometimes in our lives the greatest blessings come to us after we have gone through the greatest suffering. I was advised to terminate with two of my ARPKD daughters after their 20 week ultrasounds. The following weeks, months, and years have been difficult and even terrifying, but I am so glad that I did not follow my doctors’ advice. Yes, my daughters have suffered to some degree (though I know not as much as many other ARPKD kids do), but their pain and tears have grown them into strong little girls who do not take life or health for granted, and who know how to be thankful for the little things in life. They are more mature, more wise, more grateful, more loving, than so many other children their age who have always had “perfect” lives.

Children with special needs have a way of blessing and inspiring those around them too, in a way that healthy children never could. I know greater suffering probably lies ahead for our girls as we face esophageal bleeds and organ transplantation, but we have talked these things through with our oldest, and if my seven year old daughter can face these things with courage, then perhaps she doesn’t need to be shielded from the suffering, but only equipped to walk through it. Someday my girls will take the faith and the strength that they learned from their sufferings and use it to inspire and bless all those around them. It would have been great loss for all who know them to have ended their lives early.

I know that this is one of the most sensitive and personal topics. I pray that I do not sound judgmental in any way. I only mean to offer hope.

With love,

Katherine Eby

Fireworks!

Naomi and Hannah were anticipating the Fourth of July celebration ever since they remembered that July is the month that follows June. "We're going to go see fireworks!" Hannah informed Toby. "You'll like fireworks! They make a loud BOOM!" And after that cat slipped out of the bag Toby wouldn't stop asking if today was the day we were going to see fireworks.

When I laid him down for a nap on July 2nd he cried, "I not going nigh-night! I going to see fireworks! Makes loud noise!" I stretched the limits of his two-year-old brain by assuring him we would see fireworks together "the day after tomorrow." Thankfully, he fell asleep trying to figure that one out.

We tucked the kids in bed by 8:30pm on Sunday the 3rd and talked about seeing fireworks the next day. Then, with a phone call from my mother-in-law, I realized I had wrongly assumed that the show we'd been planning on seeing as a family was on the 4th. It was actually set for 10:15 that night, in less than two hours. Frantically, I gathered diapers, wipes, snacks, sippy cups, blankets, sweatshirts, bug spray, a double stroller, and a wagon. I scoped out the best place to park and walk online, then Matt and I loaded four very excited children into the van, still in their pajamas.

We arrived a little after 9:30pm, but ended up having to park 3/4 of a mile away, a bit farther than I'd planned. With Toby and Emma in the stroller, and our enormous pile of supplies in the wagon we began the speed-walk race to the show with Naomi and Hannah skipping ahead. "Oooohhhh!" Hannah squealed, each time someone in the neighborhood lit off one of their own fireworks. "This really is the Fourth of July! It really is, because those are fireworks. I know because they make that loud boom, and they look like real fire! Real fire, Emma, see? So I know it's the Fourth of July! It really is! And we're going to see more fireworks, even bigger ones, and....Ooooohhh! There's another one! Did you see that Naomi? Mommy, did you see that? That was a real firework! It looks just like real fire!"

I wasn't sure whether my legs or my ears were more tired by the time we found our place among the throngs and settled down on our blankets. Fortunately, the crowds of moving, chatting people decked out in glowing necklaces entranced my children, and even Hannah found herself speechless. Even better, every mosquito in the city seemed to have already drunk its fill by the time we arrived and I was spared the ordeal of bug spraying the kids. I distributed baggies of apple slices and we quietly munched as the sunset disappeared.

Naomi squinted and covered her eyes as the first few flares lit up the sky. "It's too bright," she complained, backing off our blanket a few feet, as if that extra yard would protect her eyes from the light. Toby quickly scrambled into the safety of my lap, then sat happily mesmerized with the show. Hannah and Emma's faces glowed as they smiled quietly at the colorful sky. One of the first loud "Booms" set the tiny baby in my tummy kicking and squirming. I had just read about how hearing and reflexes were intact by this point in my pregnancy, and I've no doubt that little boy was startled by the sound. I snuggled Toby in close over my tummy to muffle the noise for his little brother.

"Look at Toby," I whispered, nudging Matt beside me. Toby had suddenly reached both hands high into the sky above him and silently held them there. We chuckled a little, then Toby gasped, straining his little voice, "I can't reach them!"

It was a spectacular show, but, of course, over too soon. We packed up and quietly made our way through the crowds. It wasn't until we were walking again through the dark, less crowded neighborhood that Hannah found her voice. "Those sure were fireworks!" she sighed. "Maybe we'll still see some more. Maybe more people aren't done with them yet. Oooohhhh! There's one! See? I told you there would be more fireworks still. The Fourth of July isn't over yet, because it isn't really even the Fourth of July yet. It's only the third today. So the fireworks aren't done. I'm glad, because I like the Fourth of July. Right, Emma?" But Emma was already sound asleep in the stroller. "Well, right, Toby?"

"Makes loud noise!" Toby agreed.

"We'll probably see some more in Grandma Eby's neighborhood tomorrow," I reassured Hannah. "And we'll have a cookout, and you can go swimming."

"I just can't wait!" Hannah squealed. She was not disappointed, and now we have fuel for the imagination for a whole nother year.

Today, if you visited my house you would hear little girls asking each other, "Who wants to pretend seeing fireworks? How about Narnian fireworks?! I bet they have fireworks in Narnia!"

Into the Wardrobe, with No Hope of Return

Matt has been reading through C.S. Lewis' Chronicles of Narnia with our girls at bedtime for several months now. The story has filled their minds and our lives for weeks on end. Each "Little People" doll in our house is now designated as a character from the story. Paper towel rolls have been transformed into swords. Two egg cartons, cereal boxes, and yarn have been intricately designed into an exact replica of the Dawn Treader. Finger-paint swirls are a Narnian landscape at sunset. Play time now holds epic battles against the White Witch or Miraz's army. And I have lost the privilege of calling my children the names I chose for them.

The girl I used to call Naomi now answers to "Susan." Hannah fires an angry response my way each time I forget that she is really "Edmund." Emma doesn't mind being called Emma, but the others are quick to remind me that her name is "Lucy." "Peter" is always present, I just can't see or hear him. Toby announces with a grin, "I Caspian! Prince Cas-Pi-An!" "Reapacheep" also haunts our house along with a female counterpart mouse named "Dally" that Hannah imagined to keep him company.

My children have all acquired something of a British accent as well, no doubt from hearing how the characters talk in the movies. If I question my children about spilled cereal I am likely to hear the response, "Eet wahs prohbably Reapacheep. He's ahlways geetting into trohble. Reap-a-CHEEP! Geet in thah ahnd clean up yah mess!" Or a slightly more realistic, "Go ahsk Prince Caspian."

It's hard for Susan, Edmund, and Lucy to understand that their cousins and church playmates don't understand or enjoy living in Narnia the way they do. No matter how they try to explain to their cousin, whom they've dubbed "Eustace," that the boat in the swimming pool is actually the Dawn Treader in a vast Narnian ocean, all he wants to do is sink it. This infuriates Susan, and she sometimes has to be removed to a Narnian time-out to be reminded that not everyone realizes they are in Narnia yet.

I have been assigned multiple roles in this drama, probably based on my children's disposition toward me at the time. While overseeing the evening toy clean-up I have heard all of the following from my children: "The professor wants us to clean up our toys, he's tired of stepping on them all the time," "Yes, you have to clean up, Edmund. Aslan says so, and you have to do what Aslan says," and my favorite, "The White Witch just told us to clean. Don't do it, Susan. Don't do what the Witch says!"

I probably ought to scold my children for this disrespect, but I get better results if I play the part and offer them some Turkish Delight if they clean, or threaten to turn them all into stone when they don't. Somehow, imagination suddenly transforms toy clean-up into a race to save their lives from the evil witch. We soon find ourselves laughing, in the clean castle at Cair Paravel and enjoying "Narnian Popsicles," which, according to Edmund, taste "much better" than ordinary Popsicles. Yes, of course, everything tastes better with a little imagination added.