Saturday, September 03, 2005


I have had two migraines this week. One on Sunday, all day, which totally sucked, of course. It hovered around, never really going away, until Thursday, when WHACK! WHAM! POW! I was flattened. Luke came home and I was a wreck. And worried, because I needed to hold up for Niko's SURGERY on Friday! We needed to go downtown to the hospital (not Childrens) so the doctor could pop open Niko's blocked tear ducts. Luke worked, so I took Niko by myself. Nothing to eat or drink before, so I was afraid Niko would be starving and emotional. So Marilyn and Jason came over and the men gave Niko a blessing, and me one, as well. It helped.

So anyway, we went, and Niko did amazingly. He played with the other kids in pre-op, and I put on a gown and a hairnet and went in with him while he was anesthetized. (He was in love with the anesthesiologist...he asked our general health questions with Niko sitting on his knee gazing with unabashed love into his eyes.) Niko screamed, which I told them to expect, so I sang to him and knew that every scream meant a big mouthful of gas. He was asleep pretty quickly. His tear ducts passageways were too small, so instead of using a blunt metal wire to pop open any membranes obstructing the passages, they ended up using little balloons to open the passageway. Pretty smooth...20 minutes in and out. The surgeon said to expect some bleeding from the eyes, nose, and mouth (because the tears run into the back of the nose and into the mouth), but it should be minor and pretty much one or two days only. So Niko woke up, was cranky, ate some, cuddled some, played some, and we were out of there. Five hours start to finish.

He did bleed out of his eyes, nose, and mouth, and even though I expected it, it was extremely disconcerting. But the bleeding has stopped and he's completely back to his old self again.

The surgeon himself called last night just to see how Niko was doing and to ask if I had any questions. I thought that was above and beyond the call of duty, and I'm sending him a thank you card for that!

Calcium and Early Katrina

Journal Entry August 18, 2005

Niko's walking a little more day by day, which is great. His muscle tone is really improving. Everybody misjudges his age by a year, guessing that he's one year old. Time for another blood test, which we only have to get quarterly now. I thought his calcium level would be sky high, since he hasn't had his infusion in a year, and he's eating more foods with calcium. Still not dairy, of course, but the occasional pancake. But the labs came back completely normal, and I was shocked. And very, very happy. It seems like his hypercalcemia is slowly going away, that he truly is growing out of it like we had hoped. I still monitor calcium like crazy. Watching the solids he eats (more and more every week, but he definitely has his favorites and sticks to them religiously) and checking out his baby food jars with a vengeance. He snacks on cereal now (10% calcium a serving) when that never would have happened before. It's

Niko has also turned into a walking fool, staggering hither and yon. Last night Luke held Niko's hand and together they toddled down the hall to bed. (Well, maybe the little one did slightly more toddling.) I know that almost every parent is entranced by what their child can do. But I also feel that Luke and I seem to appreciate Niko's accomplishments more than usual, because we had to wait so very long for them. We're still waiting for Niko's first word. A "mama" would bowl me over.

Hurricane Katrina took out New Orleans. It's absolutely devastated. How many entries like this am I going to end up writing?

First Steps

Journal Entry, July 24, 2005

We're down in Luke's hometown for family reunions. While visiting everybody, Niko took his first steps! He kind of hunkered down and took three quick steps before falling. The cabin erupted into applause. We were so proud! Tonight Luke's cousins were over, and Niko was in fine form. He spun, did downward dog, laughed, bounced, crawled, chased, stood, flirted, rolled balls, balanced, crawled in laps, tried to bite, walked on his knees, waved, did "You're A Winner", clapped his hands, ate name it. He was the life of the party, all eyes on him, and he totally worked the audience. It was one of the funniest things I had ever seen. He was so conscious of everybody paying attention to him. That night he collapsed in bed, weary from the show. What an entertainer.

Wednesday, August 31, 2005

Time Out For Me

Journal Entry May 23, 2005

I wrote a poem two days ago. I worked on the blog. I found my old "Scent of Gravity" notes and want to turn it into a short story, maybe possibly expand it later. I am on freakin' fire!

The adrenaline, the twitchiness, the crabbiness until I'm a caffeine rush at 3:00 AM. I'd flippin' forgot this feeling!

Luke and Nikolai are probably both snoozing away at Ensign Ranch. I, myself, had a fabulous night, despite being perturbed at being stranded without a car on my ONE NIGHT A YEAR!!! Grrrrr....sigh. Anyway, Marilyn picked me up and we watched "Hedwig and the Angry Inch". The she fell asleep on the couch and Jason dropped me off at home. I've been roaming around for hours now, and it is so very nice to be alone.

Second Birthday

Journal Entry, May 2, 2005

Niko's birthday was not too long ago...two years old! The best part was that Mom and Dad came up. They could only stay two days but they were so fun and so helpful. Mom swept the floor and Dad mopped, and they both changed and spoiled Niko, and thereby spoiled me.

The party was fun. All of our friends came. They brought Cookies (for the Cookie Monster party) and Niko, of course, reveled in the attention. A room full of people who genuinely love my son. Luke took a video and Jason held Niko and helped him blow out the candles on the cake...who's the parent here, anyway?! I thought it was pretty funny. Luke made the Cookie Monster cake, and the first thing Niko did was pull off and suck on one of the googly eyes. He sat in his high chair and ate fistfuls of cake. I just kind of stood back and watched him, remembering his birthday last year and how he choked immediately on the cake, throwing it up. Mom put her arm around me and looked at me, and I could tell that she remembered the same thing. After all, she helped bathe him and change him into clean clothes after the last cake "fiasco". We looked back at Niko and both started to laugh. Really, things can't get too much better than this.

In Need of Caffeine

Journal Entry, April 23, 2005

Deeeeeeeeear Self,

I'm sure it has happened, but I don't recall being all anxious for Niko to wake up before. Ah, nap, glorious nap! The best part of my day! But today...oh, the restlessness. I call people and I don't want to talk to them. My feet are twitchy. My head has pressure building up inside it...I need a drink I need a drink I need a drink! Caffeine withdrawal impending....Ooooh! Aaaah! Eeeee! As soon as The Bean awakes, I'll cuddle him in my arms and then slingshot him into the carseat. Off! Off we'll go into the wide blue yonder, in search of mental/physical substinance and party supplies for Niko's Cookie Monster birthday! How fun! Some of our favorite people all together, celebrating little Niko-freako!

Come on, WAKE UP, BABY, so Mama can get her Diet Coke fix!!!

The Stupid Question

Journal Entry February 17, 2005

Yesterday Niko had another heart echo. They really wanted to take a good look, so they sedated him with an oral anesthetic, and he was out for about half an hour. No change from his last echo in December, which could be considered good OR bad. Let's should I consider it? Let's try it this way. I choose GOOD!!! Since he is bigger (24 pounds 6 oz) the cardiologist upped his propranolol dose to 3ML three times a day instead of 2ML three times a day. Ferrous sulfate and zantac still the same. Last week he went two days without zantac, and started throwing up again. I guess it's good to know that the medicine is working. He's developing quite the personality, my little Niko-freak. He laughs all of the time now. He is also the fastest crawler known to mankind...complete strangers will remark on it. Come to think of it, complete strangers remark on him all of the time. Good things, bad things. How cute he is, how a child his age should be walking. How wonderful to see such a friendly little boy, how a child his age should not be drinking formula. "Yes, yes," I think. "I'll just give him some milk to satisfy your ego, you curious stranger. It will just KILL HIM." Usually, I am very polite. Sometimes, however, I am not.

The worst time was about a year or so ago. Niko and I were in line at Costco, and Niko was sitting in the cart smiling and shaking his head back and forth, probably to make his curls bounce. This woman standing next to me turns and says, "Is your child retarded?"


About a year ago, if you remember, Self, we were just coming out of kidney failure. Williams was pretty new to us. I was overwhelmed all of the time. I turned to that woman, and I just told her what she had wanted to know. I told her about having a new baby, who was born sick, and how long it was before I could hold him. We were living away from our family, and then we took our four month old and moved to Finland for a semester abroad. I told her how my husband spent all day in school and how I was in a concrete, unfurnished apartment sitting on a sleeping bag with a baby who screamed all day and all night, and clawed at his face until he bled because something was wrong and we didn't know what. I told her that as soon as we hit America we took him to a doctor where they did test after test after test, and finally it was determined that his severe heart problems, among other things, pointed toward a strange thing called WILLIAMS SYNDROME. And how after even more testing, it was determined that this unusual syndrome was, indeed what my son had. I mentioned that Williams had masses of things associated with it, like the famous "cocktail personality", the hypercalcemia, the facial features, the cognition, the musical and language aspect...and yes, I said, there is some retardation that usually occurs, of varying severity. So, yes, there is that possibilty. Thank you very much for bringing it up in such a sensitive manner in such an appropriate place. Also, I said, you're very lucky that you mentioned such a thing to me, because I am an unusually nice person. Anybody else would have gotten very, very offended and angry with such a stupid, stupid question. My voice was very calm, but I'm sure my eyes were on fire. I could have burned her to the ground with my eyes.


She didn't say anything, just moved her cart to a line a few lanes away. I stood there for a second, then smiled at Niko who was grinning and still shaking his curls.


Tuesday, August 30, 2005

Boot Casting and Wondertoonel

Journal Entry January 31, 2005

Today at Niko's school, he had his feet cast for his new orthopedic corrective boots. It scared him, of course. Being restrained always does, since I think he associates it with those blasted blood tests where they miss his veins and stab him again and again and again and again. Anyway, he was scared, but I brought Cookie Monster to comfort him, and freshly baked chocolate chip cookies to stuff in his mouth whenever he opened it to yell. All in all, we survived the half-hour relatively unscathed, although we cut classes short and ran screaming from the building.

Marilyn, Jason, Luke, Niko and I went to the museum the other day to see Mark Ryden's "Wondertoonel" exhibit. How totally wonderful and totally bizarre. Niko has sprouted a mawful of sharklike teeth and can now eat graham crackers...what a thrill! I wrote a poem sparked by a conversation that Marilyn and I had as we walked around Green Lake. It's called, "The note pinned to my son's back." I like it.

I'm crazy tired. I want to lie on the carpet and sleep. Niko, of course, is perky. Which I, of course, should be grateful for.

The Tsunami Part II

Journal Entry Wednesday, December 31, 2004 Later That Night

Death count: 76,000. Many places have stopped counting. I turned off the news. No more hugging couples being swept away while their friends reach out to them. I cleaned some, played with Niko some, assembled a few toys from Nikolai's successful haul from his grandparents. I cuddled him. I read him a story. I gave him a bottle, sang a lousy rendition of "Good night, sweet heart" and tucked him into bed with Tickle-Me-Cookie-Monster, his best friend and confidante. There's a very special relationship between boy and monster. I hope he goes right to sleep and stays there, my good little boy.

The Tsunami

Journal Entry Wednesday, December 30, 2004

There was a horrible tsunami that hit Sri Lanka and that area on...was it Sunday night? I believe it was. Right now the death toll is 63,000. I cannot fathom such a large number. Footage of the water coming, people being washed away...they show a hand limp under tons of broken concrete, bodies being pushed into a mass grave by a bulldozer. They think malaria and waterborne diseases will kill as many as the water did. Self, I am sick. My soul hurts. I have nightmares of running through the streets, Niko torn from my arms by water. I think about it and can barely function. And that happened to so many people! One woman lost her four children and her husband. She said she had her eight month old ripped out of her arms, and I almost couldn't bear it. They had a little boy, maybe two or so, found and taken to the hospital by two American tourists. They gave him a nebulizer with the exact same purple dinosaur facial mask that Niko had in the hospital two weeks ago, and that's what got Luke.

Monday, August 15, 2005


Journal Entry Monday, December 15, 2004

Yesterday, right after I wrote you in bitterness and stomped around some more, Marilyn came. She had taken the day off to study, but spent three hours with us instead, holding Niko. I said, "Where's your gown, mask, and gloves, girl? Aren't you afraid of the mystery disease that Niko has?" She just kissed him and laughed, and honestly, that's all it took to break down this armor of anger and fear that I had built up. After she left, I managed to talk to a doctor who actually knew what was going on...pneumonia, RSV AND bronchialitis. I guess one kind of sets the other off, or something. Later that night, Luke came after work, and Marilyn and Jason visited, pizza in hand. Luke took Niko by the hands and helped him walk around the hospital room, looking so proud of himself with his binky and his little diapered bum showing through the back of his hospital gown. They cheered my handmade Christmas cards (I had lots of time those past few days!) and we had pizza and Cokes and made a party of it, right there in that hated isolation room. Niko loved it.

Today we got sprung in the afternoon. I dressed Niko's Tickle Me Cookie Monster up in a diaper, some bandages, and Niko's purple dinosaur nebulizer and stuck him in Niko's hospital bed and took a picture. Then we went home. It's Luke's and my anniversary tonight, and thus far we've always done something spectacular. Tonight we had a great meal of homemade chicken soup brought over by my friend, Andrea, in our ward, and kicked back with a movie. Just being free seemed to be spectacular enough.

Angry In Isolation

Journal Entry Tuesday, December 14, 2004

I can't get a straight answer from anybody. So many nurses parade in and says that he has pneumonia. One says that he has RSV. One says that he has bronchialitis. What I DO know is that we have been in this hospital room since Sunday night, and that we have been put on isolation. No hospital staff may enter the room without masks, gloves, and gowns. Nobody stays long. Nobody tells me what's going on! One nurse tried to double dose Niko with medicine. I said, "Is that his propranolol?" She looked annoyed and said yes. I told her that he had been given propranolol by another nurse less than 15 minutes before. She whitened a little bit, and it did nothing to temper my anger at being left in the dark. I have been told not to socialize with other parents, please. Not to go into the lobby. Not to be around the food. When they bring Niko's dinners, they put the trays outside the door on the floor. THIS is a very bad feeling.


Journal Entry Sunday, December 12, 2004

Do you see this crazy scrawl? This is a sign of fatigue. It's 2:00 in the morning, and Niko and I are at Children's Hospital. We're in the emergency room for coughing and wheezing...what looks like pneumonia. Of course, with pneumonia, he has to work harder to breathe. Of course, working harder to breathe strains his heart. Or course, the medicine they give him so that he can breathe (and thereby not strain his heart) causes his heart to race, thereby straining his heart. So we're here, we've been here since 9:00, and, two treatments and some oxygen later, I find that we have another treatment to go and then he'll be admitted. So no going home tonight, we'll wait who knows how long while they get everything set up and then more treatments tomorrow. Honestly, Self, I'm exhausted. I was up all night with Niko last night, and of course all day today, and while he napped I ran to the pharmacy and got his medicine and then finally started lunch which is, of course, when poor Niko coughed himself awake. But now he lies here, still sick, but breathing quietly, and I watch his unsteady heartbeat on the monitor and notice his pulse throbbing in his neck, and I think that I've been here before. SO MANY TIMES BEFORE. But I know without a doubt that it's okay to complain sometimes, as long as you make the right decisions.

Niko's New Class

Journal Entry Wednesday, September 22

I'm resentful lately of doling out parcels of time to people that I don't want to see, friendships that I don't necessarily want to cultivate. I think of that Smiths song, "In my life, why do I give valuable time to people I'd much rather kick in the eye?" I value time alone, I treasure it, I guard it jealously, because it is in such short supply.

That said, Niko started his 18 month to 3 years classes at his new school. He really loves them: 2 1/2 hours of play time, gym time, song time, snack time, and more play time. While there, he also does two hours a week of physical therapy, half and hour of eating therapy, and half an hour of speech therapy. He's really doing well, and has his first two teeth in (on top) and is crawling now! He pulls himself to stand on furniture, and especially on me. I'm really proud of him, my little Niko-freak. His Classes are Mondays, Wednesdays, and Fridays, and I have to admit that it's hard for me to grit my teeth and go constantly. Sometimes I feel guilty that some women seem to enjoy staying home, love playdates and keeping a clean house, and I hate it so badly. But hate it or not, I go, because it's the right thing to do. Because it's helping my son. Because somehow I believe that it will make things better.

Sick As A Dog

Journal Entry Wednesday, September 15, 2004

I have been sick as a dog for the last two days. Did I say sick as a dog? I meant SICK AS A DOG. Saturday and Sunday I had strange spots all over my body, then they went away. Monday I woke up with this killer headache that only increased in intensity (how can this be?!) Nowhere on the pillow (or earth) was comfortable. Different positions caused my body to hurt in different ways. Unfortunately (because I felt so terribly) and fortunately (because I felt so terribly) Dad had come up and took Niko so I didn't have to worry. Yesterday was worse! Not the nausea, but the sheer physical pain- I felt like I had fallen from a seven story window and landed on my back. Everything felt broken...I got up a couple of times and each step completely jarred my back and shoulders. The worst part was not being able to pick up Niko. It's Wednesday and I still don't have the strength to pick him up. But I found happiness today as he lay beside me on the bed, staring wide-eyed at me with his fizzy hair tickling my nose. I have a new appreciation for how he must feel when he's not feeling well.

Brief Summary of Life's Truths

Journal Entry Sunday, August 30, 2004

I have a lot to tell you, Self, but Niko just went to sleep, so shall I. Just a brief summary:

I went in to surgery for the second tumor in the same spot. Also benign, but I'm very vigilant.

Pet and house-sitting for Marilyn. Their dog had a stroke but seemed to recover. ("Not on my watch!" I silently pleaded. Oliver must have been listening.)

I realize that I genuinely believe that there is no learning, fun, or productivity as an adult. You work hard at a bad job until you fall apart, sacrificing for your kids. Very disturbing.

Niko now goes to bed at 11:00! Hooray!

Saturday, May 21, 2005

High Alert

Journal Entry, July 21, 2004

I'm on bed. I'm not in bed, snuggled up like a cozy little Salvation-colored paisly, and I'm in no way, I'm perched atop my bed warily, a bundle of nerves. No, that's not right--more like I'm on high alert. I'm always on high alert, ready to F-L-I-N-G back my covers in a flurry and zip with all haste to the phone, the door, to start the dishwasher so the bottles will be done by the time Niko wakes up, or whatever.

So Niko really only had the IV for four hours, and then we were out of there. The dialysis floor is kind of an IV catch-all, and that's why we were there. The little boy getting actual dialysis next to us vomited over and over and over-- what a change in lifestyle, if Niko really did have Chronic Renal Insufficiency. How blessed we are that our wonderful little boy is A-OK!!!

Roasting on a Spit

Journal Entry, July 19, 2004

This week has been all kinds of busy! Something going on every single day, all day appointments, etc. etc. We took Niko to get his upper GI done, and it was pretty traumatic. They actually strapped him to a board with his arms strapped over his head, so they could get an unobstructed picture, and force fed him Barium. (I thought, "There goes all of his eating therapy!" and the sheer prissiness of that thought cheered me up some.) Then they slid the board into the machine and had to rotate him around to get shots of was completely like he was cooking on a spit. Poor kid. He came completely undone, which surprised none of us. He had a fever a couple of days ago, and lots of vomiting. But his calcium's up again and he's going in for it tomorrow. I guess they'll give it to him a different way, so it only takes one day, not two. I'm supposed to take him to the dialysis floor. I'm a little nervous, but most things don't seem to turn out quite as badly as I fear, so we'll see.

The Piano, Part II

Journal Entry, June 30, 2004

We had six guys show up to move our piano, and it was nestled in our living room about 15 minutes after they pulled it off of the truck. Wonderful! It's scratched and out of tune, but I love it dearly, and have already logged in hours of playing time. Niko seems to love it. It's fantastic!

I was thinking about how my life never seems like it accomplishes much. Everything I do needs to be redone, over and over. Wash the dishes AGAIN, clean the counter AGAIN, give Niko his medicine AGAIN. I need to get a project that actually gets accomplished and stays that way! Draw a line through it and cavort around! Done Done Done, ha ha ha! See, I haven't actually accomplished anything yet, but the excitement is infectious!

The Piano

Journal Entry, June 22, 2004

Today was a great day, a whirlwind of doctors, friends, and Enrichment, which went well, by the way. But the best part...we bought a piano! $150, a complete steal. I just knew that if I didn't jump at the chance, I'd really regret it. Imagine, a piano of our very own!


Journal Entry, June 21, 2004

Tomorrow our Enrichment is on Journaling, and guess who gets to teach a small segment on it? I'm excited. My focus is going to be on writing for yourself. How it's a free place to say whatever you want, no rules of etiquette apply. We're covering notebooks and doing some writing exercises. I can hardly wait, actually. I use writing to process and find peace. By going off on a writing tangent, I see what I'm really thinking, and allow myself to explore those thoughts. I remember hearing once that writer's write to find out what their current obsessions are, and I believe it.

Meanwhile, it's still very hot and we're all suffering. Marilyn and I took Niko with us on our walk yesterday, when it was finally cooler outside than it was in our house. He was the pantless wonder, and all of us were very jealous.

The greatest thing, these last two days, is that Niko has been cracking up. First on my lap in church playing with a piece of curled ribbon. "Huh huh HUH," He booms. It's shockingly hardy. For maybe five minutes, he just laughed and laughed, and everybody smiled and looked at him. We were laughing, too. Tonight Luke shook his little truck with the marbles inside of it, and we heard that "Heh Ha Huh!" that makes my head whip in his direction, searching for the cause of his distress. "I hope he doesn't always laugh like this!" Luke says, and we're giggling again, shaking that truck even harder. Utterly delightful.

Tomorrow Niko sees Gastroenderolgy for the first time, to hopefully get a clue about his vomiting. He's taking Zantac right now, which seems to be helping. (Also Propranolol, Nieflex and an ear infection antibiotic, but who's counting?) What a good, sweet, beautiful, boy. I'm, so proud of him.


Journal Entry, June 13, 2004

Last night my sweet Luke walked for his graduation. Robe, hat, and beautiful velvet hood signifying MBA! I'm so proud of him, and he's worked so hard. Both of our families are up, and how great is that? We've spent a lot of time walking and a lot of time eating. Oh, and I went back to blonde and feel like my old self again.

Thursday, May 19, 2005

Sounds of the City

Journal Entry, May 29, 2004

I always thought that my life had this huge, great purpose. I'm hoping that is still the case, that it isn't all refolding laundry and sweeping chocolate chips off the floor. I just gave Niko his Zantac and a bottle. I'm trained to wake up at every sound now, and the city has a lot of sounds! The flashing of police lights woke me the other night, and I watched the whole ticket-giving exchange through the dark blinds like some voyeur. All of this life going on around me, these men twenty feet away away that have no idea that somebody is up here, that there's drama that unfolds in this space. Sometimes I look at the cars go by and I'm overwhelmed by the fact that inside every car is a life, or a bunch of lives, and that every life goes on and on and on. And all I'll ever see of that life is this five seconds of somebody in a car. That's all they'll ever mean to me, when in fact, that's not who they are at all. If the driver or the cop were to look up and see me peering out, they'd just chalk it up to some nosy, crazy woman in the middle of the night, and that wouldn't be right, either.


Journal Entry, May 26, 2004

Niko is coughing in the other room. I expect him to sleep soon. I have a new love! On Mondays and Wednesdays I have kickboxing, and I love it as much, if not more, than I thought I would! It's definitely challenging, and takes me beyond the point of exhaustion, but I feel strong and powerful, and love the time to myself. I focus on kicking straight, or on solid punches, not on how many doses of medication I've given that day, or what tomorrow's blood tests will say. The war in Iraq doesn't even cross my mind. I don't even think about the new lump in my breast, to the side of my old pink scar from the last one. I noticed it maybe three months ago, but of course we don't have any health insurance, so I've been putting it off. Luke thinks I should go in anyway, and he's probably right. I just keep thinking that one day, POOF! there will be a magical, wonderful job with great insurance for the whole family. I told Luke tonight, "I know you're trying to find a job, but I'm waiting to go to the hospital, so maybe you could try a little bit harder?" and I started to tear up and covered my face with my hands until I was composed again. And Luke-- oh, his face. He looked positively stricken, and was tearing up as well, and I thought, "Great job, way to pile on the pressure. Let's see if we can break him now, why don't we." I am deeply shamed. Sometimes I'm very unimpressed with myself.

Tuesday, May 03, 2005


Journal Entry May 20, 2004

I took Niko to nephrology and held him out in my arms like a sacrifice. "Forget everything we were going to talk about," I said. "Look at him! He's lost a pound since last week! 18 pounds! Something is very wrong!" My concern was the constant throwing up and his refusal to take any food whatsoever. Another switch to the formula, a much more concentrated amount, and somehow he's doing better.

"Somehow" includes a lot of prayers...that were answered. We were scheduled to go into Admitting today for another two day hospitalization for the IV, and we did some labs first. His creatnine had dropped to 1.7. His calcium had dropped to .9. The doctor let us go. "Enjoy the sunshine," she said, and smiled, and Niko and I skipped out of the hospital. Considering his condition on Tuesday, truly a miracle.

A Painful Rock Bottom

Journal Entry May 18, 2004

Niko looks awful today. Thinning out, this skinny neck with the blue jugular pulsing-- He's pale, his eyes are red, his lips colorless. I genuinely don't think he smiled all day. Clingy, fragile, lethargic. He vomited twice, and it hurts him now, I can tell. Not like his usual daily vomiting. I can see his veins running under the skin of his face. He didn't want to play, just cuddle. Bless these doctors tomorrow with inspiration-- I will do anything to fix this.

Pulling It Together, Somehow

Journal Entry May 17, 2005

I got up to give Niko his bottle, and thought I'd write you. So many feelings, Self. Today I feel pulled together, which is heaven considering the disjointed feeling of my life lately. I'm calling Luke's mom today and asking her to come-- just having someone to help feed us and keep the counter clean-- heck, to take care of US as we take care of Niko-- the thought alone strengthens me. Last night I looked at today's calendar and my mouth gaped open. Nothing scheduled except for kickboxing! I hardly belive it! Of course, who knows what could come up, but maybe...nothing.

So on Saturday I went to the Stake Humanitarian event. I helped organize and prepare for it a bit. The Stake Relief Society got together and made knit and crochet hats for babies, sewed and tied quilts, and made dolls in hospital gowns to for the Children's Hospital. (The dolls are staying local. The rest is going world wide.) I tied quilts and stuffed dolls (I wonder how many of those I'll see around the hospital) and it felt great to climb out of my own sphere for once and leave myself behind for a few hours. Now I want to learn to sew, and re-learn to crochet. Will I feel more fulfilled, do you think?

Marilyn has been extra wonderful. Calling at just the right time, basically checking up every day, inviting us over to dinner on Mother's Day. We went for one of our famous weekly walks last night. I vented for maybe a mile and a half, and she listened, adding insightful comments and support, and then we both started laughing at something (typical) and it was comfortable and wonderful and we were truly inspired to become friends.

The news is filled with ugliness-- the abuse of prisoners, which sickens me, and the beheading of Nick Berg in possible retaliation of that abuse. Apparently you can go online and watch the beheading, and some DJ's were fired for playing audio of it on the air and making jokes. The depravity of the world astounds me. We had an interesting discussion with our friends about being informed of world events, but not being stained by them. Possible? How? I fear for my children. But then again, I think every generation does.

On Tuesday we have the nephrology clinic. We get to ask questions. I'm starting a list. Does he really have Chronic Renal insufficiency? It sounds like the goal is to keep the kidneys functioning as long as possible. What then? A transplant? Will that take care of the problem? When would that take place, if necessary? Will we raise our future children with the knowledge that one day they might get to give big brother Nikolai a kidney? What about dialysis? What's his life expectancy? John called yesterday and said, "What are your feelings? Do you think he'll make it?" I was totally taken back. I said, "John, yes, I'm sure he'll make it!" The doctors never mentioned any other possibility. In all of his blessings Luke says, "You'll grow up to do this and be this..." and I cling to that simple phrase...GROW UP! But his words make me wonder...IS THIS A POSSIBILITY I SHOULD BE PREPARED FOR?! Sometimes I get these brief flashes of clarity and insight, from a larger perspective. If we have to move from this place and from this wonderful hospital, we will survive. Yesterday I thought, I believe in this gospel. If something were to happen to my baby, I know he'd go to God. I truly believe that. So deep in my heart, I know that the worse case scenario has joy in it as well, yet the natural man persists, and I cry, as I do now, and my stomach twists, and I remember being on hands and knees and vomiting repeatedly last week, and knowing that would only be the beginning of the reaction I would feel if we lost our son.

As I said, so many feelings. Luke has been wonderful. We've somehow learned to pull together instead of apart, as we were. I think a lot of it has to do with my verbal appreciation for him. It'd not enough for me to know it....he needs to hear it, too.

Hopeful Hospitalization

Journal Entry Friday, May 14, 2004

Yesterday I got a call from the nephrologist. They found a medication to help reduce his calcium and fuse it into his bones where it belongs. It's an IV...could I be here in two hours? Yes! I could! So we packed up and came. Yesterday he had the IV for two hours, stayed overnight (I camped out on the pull-out couch) and today he had it for four hours. The 6:00 AM blood tests said his calcium was even higher, but the doctor said it wasn't much higher, and she expected it to take a day or two to work. So now we've been here 28 hours and we're just waiting for the dietician to run us a container of Calci-lo formula to last us through the weekend. We're still trying to find someplace to supply us with this formula longterm. Otherwise we're defeating the purpose, pumping MILK into my over-calcified little boy.

I got a great letter from my was wonderful. Mom and Dad really want to come up, and so does Luke's mom. We might take her up on it.

Wednesday, April 27, 2005

Blood Belongs In The Body

Journal Entry, May 12, 2004

A'ight, Self, here's the scoop. Aorta is too small-- surgery looks bad. They'll only do it if it reaches the point where there's really no choice. We give him six milligrams a day of Propranolol, a beta-blocker to keep his heart from working so hard. Hopefully it won't thicken any more.

Creatnine went down to 2.0, which is positive. We're discussing the possibility of dialysis, which, frankly, terrifies me. I remember visiting Grandma in the hospital one time when she was really, really sick, and the tubes running in and out frightened me. That comes to mind when I think of running Niko's blood out of his body. There's such a primitive fear when it comes to should never be outside of the body. Ever.

His phosphorus is too high, so we're trying to get this certain formula, and I just got a phone call five minutes ago saying his calcium is too high, as well. They're afraid of seizures, so I'm supposed to mix Similac 60:40 with Calci-lo. (When we actually can find some.) The Nephrology clinic sent me home with a big book on Chronic Renal insufficiency, and how people with CRI need certain diets, medications, and care. Next week I learn how to inject him daily with a growth hormone.

We started physical therapy at a school instead of the hospital, and he seems to like it there as well. We'd like to get him to a bi-weekly preschool there when he turns 18 months old. They do therapy and other things that will help him.

Meanwhile, no job. I remember how hard I searched and how frustrated I was when I was looking for a job. We want to stay here because of the care for Niko. We really love it here and have great friends...isn't that why Luke went to school? To find a great job?

A little overwhelmed. I'm near tears almost all of the time. I hope to get organized tomorrow.

Heart and Kidneys

Journal Entry, May 6, 2004
What an ordeal yesterday turned out to be! We arrived at 7:00 AM and left at 6:30 PM. A few things came to light. The MRI showed that the aorta to his heart is too small, which explains why his heart is pumping so hard. Our cardiologist doesn't know if we can treat it with medication, or if there's surgery to help. She'll call around and find out. So there's that. But his creatnine levels came back at 2.4. I guess for a baby they would be 0.3 (?) or so, and for an NFL football player, about 1.7, so it's super high. They put Niko on an IV and tried to figure things out. Today's blood draw shows that the creatnine went down a point, from 2.4 to 2.3, so we go in for a blood draw and an echo on the arteries leading to the kidneys tomorrow. I have questions to ask. I'm beat. See you tomorrow.

Birthdays and MRI

Journal Entry, May 5, 2004

We're spending the morning at Children's hospital. Niko is under sedation and going in for an MRI and a heart echo. Luke doesn't have class until later, so he's here with me. Babies with WS are usually more sensitive to anethesia, so that's a concern. Heart problems complicate it, blah blah blah. They put him in a little gown and he cried and fought the gas mask, but I smoothed his hair, looked him in the eyes and talked to him ( until they booted me out) and he'll wake up to Mommy and Daddy's smiling faces!

It was his first birthday on Saturday. We had a mini party with friends. Luke made a baseball and mitt cake, and we had balloons. Of course, Niko is unable to eat any sort of solid foods, and is still throwing up his formula several times a day. He tried a lick of cake and vomited dramatically in his high chair. But a quick whisk to the tub and bedroom and he was all clean and ready to be entertained within minutes. We opened all the windows and kept the balloons in the air, trying to bat them out of each other's open window. It was extremely juvenile, and extremely fun! The "Real" party will be in June when our families come up.

They just wheeled him in and will be doing the echo. His eyes are taped shut. We love him.


Journal Entry, April 25, 2004

An unusually high number of nightmares about Niko lately. I'm feeling pretty disturbed right now.

Challenge for Charity

Journal Entry, April 24, 2004

Niko is down for a nap (hooray!) and I willingly tuck myself into bed with you and a Cadbury Cream Egg. Easter was great. We talked to our families, and put golf balls in the Family Easter Basket for Luke, a pair of WONDERFUL high heels in for me, and the rest of the basket was candy and NIKO NIKO NIKO! How fun to pick out a little church outfit for him...and a pair or Batman pajamas with detatchable black cape. Who wanted the church outfit and who wanted the cape, I wonder? (Innocent look) Luke and I are backwards from stereotypical relationships. He wants salad, I play video games. He thinks church, I think superhero. We like it! I notice that I've been shopping like crazy lately. That's totally unlike me. And Luke's been lying around the house, which is unlike him. We are learning to cope with stress in better ways.

So last weekend we went to California to do a Challenge for Charity with Luke's school program. How wonderful to get a break from daily life. We stayed in a great hotel, and it was beautiful. I did the obstacle course and Tug-Of-War with the students while Luke held Niko. It was fun to break out of my shell and do something out of the norm for me...whoever would have thought that my "norm" would have become so boring???

The Day After

Journal Entry, April 9, 2004

I had a night to process this, looked it up on the internet so as to be prepared for today's conference, and am trying to assimilate it into future life. One one hand, I see my sweet Niko, and on the other I see this syndrome, and I'm certainly having difficulty laying one transparent picture over the other and seeing the finished product. Anyway, it's a beautiful day, and we're going for a walk this morning! Bye!

The Call

Journal Entry, April 8, 2004

What to say, where to start. Luke loved China. I loved New Orleans. We love being back. I love him and he loves me...we both love Niko. Niko loves us. So maybe everything else is secondary.

I received "The Call" about 20 minutes ago. "The Call" clarifies the elusive SOMETHING that has been dogging our steps the last eleven months. Well, first...last week Niko's sedated echo showed some blockages in the veins going to the lungs. So to compensate, his heart has to pump harder, and since it's a muscle and is working harder, it's thickening a little within the chamber. Since the chamber is smaller, there's not as much room for the blood to go, so it's having to pump harder-- vicious cycle, and all that. The Cardiologist says she thinks there is another obstruction in the aorta, but she couldn't tell from the last echo. In three weeks we do another one, and get a 24 hour heart monitor for him. And do a CAT scan, so they can see better. And probably a million other things that I don't know about yet. Honestly, though, I'm so glad that they are so eager and willing to help. I have confidence in them.

Anyway, "The Call" states that the preliminary report finds that he DOES have Williams Syndrome, although it will take a week or so for the final results to come in. That's consistent with some, though not all, of his heart symptoms and his developmental delay. Although I thought he was okay, he's 11 months and has the development of a six month old. We go to physical therapy with him once a week and I work with him at home every single day. We have a conference tomorrow with the geneticist to discuss it. I guess I'm not surprised. When they first mentioned Williams as an option months ago, something rang true to me. I mentioned that to Luke then, and we brought that up today. Overwhelmed, I guess, but not surprised.

Living At The Hospital

Journal Entry, March 5, 2004

So Niko and I practically live at Children's Hospital. Tests, tests, and more tests. Due to the folds of skin under his eyes, the heart murmur that never went away, the head tilt to one side... all signs point to...SOMETHING. Ah, that ever elusive something! When shall it be found, if ever? So far Cranialfacial has discovered...NOTHING. Next stops: Cardiology for a sedated echo, Physical Therapy for an evaluation of the head tilt, and a blood draw to test for possible William's Disease, having to do with the skin, connected to Chromosome Seven. that's all I know. I won't look it up, or worry, because we walk in and shoot down all their tests-- BLAM BLAM BLAM!!!-- and then blow the smoke from our barrels. I appreciate their thoroughness, and I appreciate their concern, but Niko is happy and is developmentally normal. I don't know what they expect to find.

Arrival of the Tsar

Journal Entry, September 2003

Niko is the most beautiful boy ever born. He's oohing and aahing over the wonders of this life. He's entranced by thing that we take for granted, like his own hands. They must be delicious.

He scared us to death when he was born-- bright red and very angry, always screaming and trembling his "fists of rage". Most babies cry-- he was absolutely livid! Turned out his hematocrit level was so high , 77, that when they drew blood out of his arm, it was thick like maple syrup and strung from his arm to the needle. With so many red blood cells, his immature liver couldn't break them down, and he developed severe jaundice. They had him in an incubator for phototherapy for FIVE days before we could leave the hospital, and then another four days at home. Nine days of not being able to hold my baby except during feedings. Except, of course, that one brief moment where I slipped him out of the lights and danced with him to David Bowie's "Magic Dance." Three forbidden minutes of smiling, laughing, and twirling, NO WIRES. NO LIGHTS. That will always be our song. Every day we thought would be the last day...a new bloodtest on my little one, both soles of his feet pricked raw, and a new heartbreak every time they said he'd lost more weight, or his bilirubin level had spiked again. It was awful. I actually began to tear up when they called with the bad news the last few days.

I don't remember if I told you that the tests showed that Babe was at a higher risk for Downs Syndrome. He had a chance of 120 out of 1100. Well, while in the hospital, the nurse noticed that Niko's hands had a completely horizontal crease in them, a symptom of Downs. That, couple with his heart murmur (common in infants) and the high HCT, gave them cause for concern, and the next thing I know I'm giving them the okay to count his chromosomes. He was beautiful, and looked normal, but there are different levels of severity. A month later, his tests come back completely normal. A series of coincidences. The crease is called a simian crease, and appears to be hereditary. Luke's brother has one. "Gosh, I'm so sorry!" He said, looking down at his hands. "I thought everybody's hands looked like these!"

Monday, April 25, 2005

Adventures in Williams Syndrome, Part One

Hey, gang, my name is Mercedes and this is my maiden blogging voyage. It's daunting to write down all of my thoughts and concerns about my little boy and then shoot it out into cyberspace, but I'm thinking that cyberspace is exactly where this information needs to be shot.

My two year old son Niko was diagnosed with Williams Syndrome about a year ago. My husband and I had never heard of it, and were quite astounded at the unusual characteristics that it tends to entail. I performed an exhaustive search on the internet for information, which only succeeded to depress and horrify me. There's really not a lot known about Williams Syndrome yet, and the severity can vary so much that it's almost impossible to get a guideline of what to expect.

So of course, the information that I found wasn't really helpful. Reports contradicted each other, and many were so dry and BLEAK that I finally couldn't read any more. This was my BABY, and to come upon such hazy "predictions" for his future was pretty frustrating. What I really needed was a blog. I needed to hear from somebody who either had Williams themselves or had a child who had WS. I needed to cut through the medical jargon and hear somebody's real words saying, "Yeah, you know, this is what to look out for." Foundations didn't really interest me at that point, and the Mother's Groups I attended (not for Williams specifically) were real downers. I am not a victim. My son is not a victim. I do not in any way feel that we have been cursed with some horrible thing, and that people owe us. I wanted to read somebody's words from afar, so I could contact them if I desired, but I didn't feel obligated to. I wanted to be a silent sponge that soaked up their knowledge so I could take what fit and use it in my own life.

I haven't found anything like that, so I'm starting my own. After this beginning intro, I'm typing in entries from my Journal just as I wrote them at the time. So you'll be able to follow along with us as we make our new discoveries, and believe me, there's something that we learn every single day. But before I begin, I want to say three very important things that I wish I had known up front. It would have saved a lot, and I mean, A LOT of pain!

Number One: definitely keep a journal. Not only will it clear your head and heart out, but it's really useful for looking up the dates that things happened. When did they start their meds? How much did they weigh at the time? It also lets you see how much progress really is being made. I read my journal last night, and found out that almost a year ago exactly, Niko was going into kidney failure. Right this very second, he is holding onto my knees, growling. He has come such a long way since then!

Number two: If you notice your child itching him/herself, check out their calcium level. Niko has Hypercalcemia, where his body can't really use the calcium, and that is what was sending him into kidney failure. It's not something that people really check out a lot. A chance comment from a nutrition student sitting by me in the bleachers alerted me to it. It can be corrected fairly easily with diet and IV infusions.

Number three: This only gets better. Kids get older, doctor's get smarter, you get stronger.