Saturday, June 28, 2008

Alright then...


When my daughter Grace was born six weeks early and underweight, she was quickly placed in our hospital's neonatal intensive care unit (NICU). At that time, I was quite ill with the effects of severe preeclampsia, and couldn't be with Grace in the NICU.

My husband, Charlie, spent much of his time being with me or Gracie in the NICU. Honestly, I have just a few memories of the first weeks of Grace's life. A medication called magnesium sulfate was dripping into my body through an IV placed in my hand. I was tired, hot, swollen, and dealing with the beginnings of post traumatic stress. At some points, Gracie was the furthest from my mind. I was trying desperately not to have seizure, and focused on forgetting that she was fighting for her life in the NICU. Whenever I became stressed, my blood pressure would spike to extreme highs in the 220/180 range and my whole body would experience shuttering and shaking. Uncontrollable actions would randomly happen to me because I had cerebral swelling and high blood pressure. I had to forget the guilt I had about my baby, and focus on me. Today as I sit here writing this, tears pool as I think about what I had to endure to survive for my precious baby girl.

As I said earlier, I don't have very many memories of that time frame, but there are two instances that stand out. These memories revolve around Grace's neonatologist, John who ended all of his conversations with: "Alright then, any questions?"

Tiny blood vessels had burst within my retinas from the high BP, and it appeared as though I had spilled black coffee across my entire field of vision. I was afraid my vision would never return to normal, and honestly, I was having trouble getting the medical professionals to acknowledge this very rare complication of severe preeclampsia. I had just finished describing my symptoms to the nurse who was caring for me post c-section.

Suddenly, a blurry dark figure of a man appeared at the foot of my bed in light blue scrubs. He wore a blue scrub cap as well, and bright red and gray wiry hairs escaped from underneath his hat. Large gold rimmed glasses adorned his face, which made his eyes appear even larger with the magnification.

"Hi, I'm Dr. G. I'm caring for your baby Grace." His voice was very distinct, and it seemed that he enunciated all of his syllables very carefully. I wondered if he was British at first, but couldn't place his accent to anywhere specific.

I responded meekly, "Oh. Hello."

"She is stable right now, but I have a bit of news that most mommies don't like to hear."

My heart skipped a beat and my head throbbed even harder than before. I frantically searched my thoughts for a reasonable excuse for an introduction like that. Charlie interrupted and said, "Is she okay?"

He quickly understood our fear, and said, "Oh. I'm sorry. Um, she is doing well, but I had to give her a hair cut so I could place her central line in her scalp. I put it on the right side of her head. She has very tiny veins, and I could only find a good one in her head. I have something for your memory book though."

He held up a tiny plastic bag which held a lock of Grace's hair. Charlie received the bag, and quickly gave it to me. I remember feeling relieved that it was nothing more than a hair cut, but wondered how she would look with a shaved "sidewall" on the right side of her head. I put the clear plastic bag close to my right eye and tried to study the hair color.

"Oh, she has dark brown hair. I never saw her hair color in the OR because she had a hat on when I got my glimpse." I searched my thoughts and suddenly thought of a baby picture I'd seen of Charlie as a newborn. He had a head of dark brown hair. Plus, my mom was a brunette too. My thoughts were almost clinical. I approached the idea of Grace in factual terms and emotions didn't come.

Dr. John responded, "She is a beautiful baby, and you should be very proud. You get yourself better, and then come visit her when you feel better. Alright then, any questions for me?"

My memory begins to fade at that point but what I do remember is his compassion and genuine concern for me and my baby. This may sound trite, but I think I remember a twinkle in his eye, too. His demeanor was happy and comfortable. His nonchalant approach actually helped me to relax and know that it was okay to get better so I could care for Gracie later.

A little over two weeks later, Dr. John greeted us by Grace's bedside in the NICU. I had been discharged from the hospital, and I was feeling a bit more like myself again. The 40 pounds of swelling was fading, and my blood pressure had begun dropping. I still felt unsteady about my experience and was quite nervous with Gracie. To me, she was incredibly tiny, and at the time, I hadn't quite bonded with her. There were so many wires, tubes, and barriers to her. I secretly wondered if I'd feel like I was her mommy. You know that kind of mommy love that leaps from within in your heart and seeps out of every pore of your existence. It just wasn't there yet, and I had an overwhelming need to feel that. I couldn't figure out what was wrong with me.

"Hello. I wanted to talk with you about Grace. She seems to have a bit of a problem with her gall bladder. Her jaundice isn't improving in the way we'd like it to improve by this age. I called a specialist to come in and figure out what is going on. She is from Children's Hospital and she'll be here tomorrow to examine Grace."

"What kind of doctor is she?" I asked.

"She is a pediatric gastroenterologist. She takes care of livers and digestive tracts."

Charlie responded, "Oh. Okay. I suppose we'll have to wait and see what happens."

I remember studying Dr. John's face through my poor excuse for sight, but rather it was the concern in his voice that caught my attention. Charlie seemed to respond to his news with concern too, and then suddenly, it was like my emotions had broken through a protective levee. My thoughts were suddenly frantic: What's wrong with her? Will she be okay? This can't be good. I can't loose my baby. It was so much work to get her here. I'm afraid. I was suddenly filled with mommy love for her. The reason we named her Grace in the delivery room was that we thought God had delivered his grace to us in the form of our daughter. My squelched love leaped out of my heart and could no longer be contained. Somehow I felt as if God was speaking to me through Dr. John, and I will forever be indebited to him for reminding me to let my love for Gracie come to the surface.

As I reflect on those brief, memorable interludes with Dr. John, I can now see yet another miracle of his careful watch of Grace in the NICU. As a result of the GI specialist coming to examine Grace, we were quickly informed she had a genetic disorder of the liver called Alpha-1 Antitrypsin Deficiency. While this was a devastating blow to us as parents, it really was a miracle for us to know this fact about Grace. The majority of children with Alpha-1 are undiagnosed. A subtle symptom of an elevated direct bilirubin past the age of two weeks is what tipped off Dr. John there might be an issue with Grace's liver.

With the knowledge we have about Grace's genetic disorder, we were then able to arm ourselves with information about Alpha-1. Because the alpha-1 antitrypsin protein is not very effective and can't often times make its way out of her liver, it is not circulating in her bloodstream. Alpha-1 has a primary purpose of being an anti-inflammatory agent within the body. Its largest job is to enter the lungs to provide a counterbalance to neutrophil elastase, which eats up bad things which we breathe in on a daily basis. Because Grace does not have enough Alpha-1, her lungs are constantly being damaged by things she breathes in such as bacteria, viruses, particulates, fumes, perfumes, and dust.

Dr. John's curiosity set something wonderful into motion. We now have knowledge and can protect Grace from some of those harmful effects of Alpha-1. We keep her away from all cigarette smoke, candles, cleaners, ozone on Ozone Action Days, and swimming pool chlorine. It helps to know that these everyday little things can save small amounts of lung tissue over time. I know that only God will control Grace's Alpha-1 outcome, but it helps to know that we won't be inadvertently making bad choices for Grace along the way. We have little to no control over how her liver functions, but we can help her breathe freely along the way. The average Alpha lives to the age of 56, but I hope and pray that she'll live a long happy life.

So I pay tribute to Grace's neonatologist. He may not know how he influenced our lives, but I will forever be in his debt. Thank goodness for wonderful physicians like you, Dr. John. Your ability to notice subtle clues is what helped me to know I could get better after preeclampsia, to know that I did have love for Grace, and to know that she has Alpha-1. I will have reverence tinged with awe in regard to you for the rest of my life.

Thursday, June 26, 2008

A Soldier

Words are not coming to me tonight. Emotions prevail right now.

I waffle between fear and tears, but at the same time have an immense amount of pride.

My 23 year old baby brother, Tim, enlisted in the Army Infantry today. He will be in for a little over 4 years, and has qualified to learn airborn (i.e. jumping out of airplanes). From the day he was born when I was 12, he has always sought out the military. I've got a ton of pictures of him pretending to be John Rambo. I'm surprised he waited this long, but here we are today. He is leaving for Fort Benning in Georgia on 7/15.

As much as I'm proud of Tim, I also terrified for him. He is after all my baby brother, and I love him with a very large heart. I pray he will be safe, and I'm pretty teary eyed right now.

So if you could spare prayers and positive thoughts for him from now on, please keep him in your prayers. Tim is such a good man, and he wholeheartedly believes in this life changing decision.

I guess I'm not ready to be the sister of an enlisted man. I've had such a mother-like role with him and all I want to do is protect him. Of course, that won't be possible when he may be so far away.

Thanks for thinking of him. He won't be put into active duty until sometime in November after his training is completed.

I've got a soldier for a brother now.

Monday, June 23, 2008


Oxygen tubes were strewn across the top of their lips and clear tubing clutched in their hands. Occasionally, a hiss would erupt from an oxygen unit. Here and there, the pulse of oxygen being delivered could be heard as a pshshhhhhhhh kind of noise.

I had been there before. Their gulping breaths in and out, pursed lips, laugh lines in all the right places, and wide smiles greeted me. It was what I had waited for yet overwhelming at the same time.

I was alone but yet among relatives of the same family. My Alpha-1 family.

As I walked throughout the conference hall, I began to calm myself with subtle inhales and exhales as I encountered familiar faces as well as new faces. Some were babies and children. Some were young adults; some were middle-aged. All were in attendance to learn, grow, relate, and feel less alone.

I was there to learn more, but I wore a smile on my face like a mask. I really don't think anyone noticed though. All were too busy enjoying each other. I wondered if I wasn't the only one feeling like tears were going to down pour from within at any moment.

I was uneasy there. Denial keeps me safe inside of the world of being at arms length. I don't walk the earth in the body of an Alpha. My children do though, and that knowledge profoundly affects me when it bubbles like a volcano letting a subtle burp burst through the cool crust of lava. It is always there smoldering in my thoughts.

This annual education conference can be both an inspiration and realization. Speeches about treatments and cures inspire this mother's heart yet they grab reality and shove it squarely in my face. Here Jen! Here! See! Your children have a life threatening genetic disorder. Stop living like they are free from potential liver and lung disease! As I screamed on the inside, I smiled and clapped and cheered the idea of a cure. It will come someday, hopefully soon.

My conflicting emotions startle me. I hold Alphas in my heart. They are the bravest group of people I've ever encountered. They deal with failing lungs, sick livers, skin ailments, and bodies failing to keep up with their ferocious spirits. All of this in the prime of their lives. Lives often interupted by the need to take a break to catch their breath. Lives with yellow skin or bellys full of ascites fluid.

It is life though. My brain keeps reminding me of that, but my heart keeps wanting to run from the reality of my daughters' Alpha-1. I remain firmly half way between acceptance and denial.

A kind doctor invites my children into his one of a kind pediatric Alpha-1 practice. I want to hug him. Tears well up, but my defenses shield him from my happy yet sad tears. Conflicted, I know that Grace and Meghan will have access to leading research and potential treatments. At the very least, they along with their Alpha-1 will be entirely understood there with yet another life blessing in a kind, for-a-cure hepatologist and biochemist. A man, who has lost so much himself, but still there giving and giving to our small community of children with Alpha-1. He gathers Gracie and Meghan into his circle of hope. I embrace that hope like a life preserver.

I ride the elevator and head for my hotel room. Upon shutting the door, I break down. I'm simply overwhelmed with his compassion and understanding. I feel honored for my girls. They really do deserve the very best in life, and today, God's blessings erupt like lava and shower down on them. I want to shout the news so all will know, but I place my smile mask back on my face. I reemerge from my room and make my way to the memorial service for Alphas who we've lost, tissue in hand.

There in that room though, I can let it out. I listen to the brave stories of Alphas who now breathe free and live their eternal lives among the angels. I tell the story of my cousin, Butch, who left his Alpha life last year. I recount his dimples, bright eyes, and mile wide smile. I hope he hears me. I hope he knows that my daughters will have a different outcome. I know it deep down in my heart. Angels whisper it to me everyday. Some days I hear their guiding words. Other days I don't.

A lovely lady hugs me close, and tells me that she feels like she knows me a little better after what I've said. She tells me that my children will be fine and that I must believe. I say that I believe but it is tough listening to the stories of Alphas who had believing parents just like me.

Another couple with salt and pepper hair get up to speak. Their daughter has recently passed, and they've come to the conference to understand Alpha-1. Their two other adult children have it too. I think to myself that they are like me. Their parental love seeps from within them. They don't understand why she is gone and why their other children may leave this earth before them. It isn't right. I sob along with them. I am just like them still hoping and praying and desperate for a cure.

The weekend conference comes to a subtle end. I learned. I laughed. I cried. I smiled. I lived. I soaked in the love all around me all with the knowledge that we need a cure for all the Alphas. Long live the Alphas!

Saturday, June 14, 2008

Amusement Park Photos

An approaching storm hastened our departure from the amusement park.

Meghan napped on the way home, and Grace enjoyed being silly.

We closed out the day with a double rainbow. Grace and Meghan were ecstatic as this was only the second rainbow they've ever seen.


A bright blue sign that said Meteor graced us as we walked up the ramp toward the gate. I held her 4-year old hand in mine. Our hands were both sweating in response to the 80 degree, humid weather.

She skipped along in a clipity-clop cadence. With each skip, her size 8 feet landed on the gray planks of wood.

We approached the end of the line, and she began to dance impatiently in place. Her hand tugged on mine in an up and down motion. At one point, she nearly collided with the woman in front of her.

"Hold on, Meghan. It's almost our turn."

"I looove, loooove, looooove this one Mommy."

"I know honey. This one is fun, and I'm so glad to be going with you this time."

It was probably Meghan's sixteenth time, but we hadn't gone together. Suddenly, a tall linebacker sized man appeared before us, and he opened the black chain keeping us back. Meghan squealed with joy in anticipation, and tugged my hand in an attempt to run toward her latest obsession.

"Mommy! C'mon Mommy! It's our turn."

"Okay. Okay. Slow down though."

A cobalt blue roller coaster appeared before us. It was perched on top of a brand new wooden structure. The gray planks led us toward the coaster. Meghan broke into a run, and ran toward the first car. Her Dora crocks seemed to hover off the ground as she ran before me. Along the way, she sized up each seat. She selected the second row of seats, and jumped inside. I followed her into the blue seat.

"Sit down, Mommy. Sit down!"

"Alright. Alright."

A loud rachetlike sound popped, and a metal bar wrapped in a red cushion folded down on the top of our laps. The technician announced, "Keep your hands inside the coaster at all times." Meghan snuggled in next to my left side, and said, "I'm weady, Mama."

"Good because here we gooooooo."

The coaster lurched forward, and caught the bottom of our car on a large chain, which pulled the coaster up a large incline. The loud clicks and pops built the anticipation of the corresponding ride down the hill. Suddenly, Meghan put both of her arms in the air. In one confused thought, I was beaming with pride at her lack of fear, but also scared she'd slide right out of the coaster. I wrapped my left arm around her back and hip to hold her in.

We were now on the ride down the hill, and a scream erupted from within me. Meghan gleefully screeched, "Weeeeeeeeeeeeeeeeeeeeeeeeeeee! Weeeeeeeeeeeeeeeeeeeeeeeeeeeeeee!" Meghan's yellowy hair bounced up and down in response to the coaster's movements, but I never loosened my grip on her hip. Several twists and turns later, and we were back into the covered area of the Meteor. The technician pulled a large lever, and we picked up speed to go back around another time.

"We're goin' again Mama! We're goin' again." Two more times around the track, and the technician finally brought the coaster to a stand still. Meghan's smile seemed nearly a mile wide. I'm not sure this is possible, but my heart was a little bit fuller with love for her.

Monday, June 09, 2008

A Von Trapp style kindergarten graduation

Gracie graduated from kindergarten today. All of the children at her German immersion school performed in a very cute show. Several, all in German, songs were performed, and Grace was a little unsure how she would do in her "show." She did great, but along the way, we had a few laughs. I also teared up a little bit as she is growing up so fast. Enjoy!

Saturday, June 07, 2008

Super Cell Update

Well, there have been several funnel clouds, but nothing that did too much damage around here. The weatherman said that the system was as tall as 60,000-65,000 feet. Airplanes fly around 39,000 feet.

Now, they are talking about intense flash flooding in some areas. One town near us got 11 inches of rain in an hour. WOW!

Softball sized hail fell to the west of us.

So, we're safe. That is the good news. :)

Super Cell

We've got a super cell thunderstorm front flying through WI right now. We don't get super cells here...that is usually out on the plains of the midwest. Our weather man has visible fear on his face. Right now, we've got 1/2 hour before it hits our neighborhood. We're starting to hear the rumbles of thunder off in the distance and it is so dark our photo cell outside lights have come on.

My kids are squirrelly as I've brought them down into our basement. I hope everyone stays safe. Sheesh...

I'll report more later.


Friday, June 06, 2008

Happy Birthday Matt!

Happy Birthday little brother! Or, should I say "Uncle Pewy"?

Preschool Graduation

With every milestone achieved, this mommy feels tears of joy and pride. I can't believe how fast Meghan is growing up. My baby is growing up so quickly, and I feel like I'm missing it. I know that I'm really not missing it, but for some reason, Meghan's milestones are somewhat bittersweet. She is my last, and I tell her this all the time: "You will always be my baby." This picture was just about four years ago today. Look how small she was. Amazing.

Meghan graduated from preschool, K3, today. Tonight, we celebrated in the basement of the church. Her class sang three songs:
* Father I Adore You
* Devil is a Sly Old Fox
* We are the Dinosaurs
It was a very nice program. She was quite proud to sing in "the show" and spend time with her friends. I sat back, soaked it all in, and tried not to cry through the program. I know that most mommies don't cry, but she is a such a miracle in our lives. I can't help it. Enjoy these pictures of her K3 commencement:

The Devil is a Sly Old Fox...

Her class had a favorite prayer before snack time and lunch. It is called the shark prayer, and it made us giggle and giggle. She just loves it. Here is a video. I cut off the first couple of words, but you'll get the jist.


Someone, a not so nice someone, stole my beautiful petunia flower basket right off my porch. Our neighbors lost their planters as well. They were nice while they lasted, but I'm pretty mad right now.

Monday, June 02, 2008

Grace's Surgery Scheduled

Charlie just called me. The scheduler called him at work to arrange the date of Grace's surgery.

It will be on July 23rd at 1:00 p.m. To me, that is a funny coincidence of a date. That is the date we brought Meghan home from the hospital four years ago. It is also my aunt Joy's birthday.

So we'll hope for the best outcome afterward and enjoy a little bit of summer before Grace needs to have the surgery. Plus, she'll get to recover before returning to school in early September.