Laternenacht

I slid the door of the mini-van closed, and as I turned around, one of the lanterns slipped a bit in my hand. It was Laternenacht at Grace's school, which is a politically correct version of Martinstag, a German holiday celebrating Saint Martin and his good deeds.

I readjusted the lanterns in my left hand, and I took Meghan's mittened hand in my right hand. Grace grabbed my left elbow, and we shuffled along toward the school. As we walked along, children swung their lanterns back and forth as their parents reminded them to be careful.

"Mommy, I want my lantern on!" Grace protested.

"Sorry Grace, we'll turn it on when we get inside the gym. Hurry with me. We're a little late, and the program is starting."

We walked through the glass-paned front doors of Grace's school. The main hallway was teeming with parents and children. Some parents were still trying to buy their children lanterns to use in the procession around the park later. I kept hearing, "I want that one!" from various little helium voices. Laternenacht was a big deal for these grade schoolers. They had been preparing with their teachers for weeks by making lanterns and practicing the traditional German songs such as Laterne, Laterne.

Last year, we had missed Laternenacht because Gracie was incredibly sick with a stomach virus. I wasn't sure what to expect. We finally squeezed past the table where the lanterns were being sold, and quickly proceeded to the gym.

"Stay close girls. I don't want you to get lost in here."

The lights were down in the gym except for the backlighting on the stage. A "high tech" overhead projector shone onto the screen. A blurry song form appeared. I couldn't make out any of the letters. Four parents were standing on the stage, and each held various instruments readied for the sing-along.

"Mommy, I want to turn my lantern on." Grace said again.

"Go ahead Grace. Let's take your hat and mittens off."

The folded accordian-style paper of her lantern light glowed. It was a traditional style German lantern in the shape of a ball. Two round metal wires held the ball together.



Families were packed in like sardines in the gymnasium. We sat down on the floor.

Just as we were sitting down, a young boy bumped into Meghan. She fell directly onto her paper lantern. It ripped in half. Grace promptly began to cry because she thought it was her lantern.

"Gracie, this is not your lantern."

"Oh." She turned around, wiped the easily formed tears from her eyes, and turned her lantern off and on several times.

"Mommy! My lantern is smooshed," Meghan whined.

I wispered, "I know Megs. Mommy is going to fix it for you." I dug around in the pocket of my slacks, and found a small black binder clip that I brought along just in case the mechanism on which the lantern was perched failed. I felt rather MacGyverish because it worked perfectly to keep Meghan's lantern together. I thought, "Yay me!"

The sing-along began, and Grace's prowess of her Laternenacht songs came shouting out of her. She was in heaven. The songs flowed easily out of her memory, and her voice matched the tune perfectly. A mom sitting in front of her turned around smiling. Gracie was clearly shouting songs in her ears, but that mom didn't seem to mind.

Ich gehe mit meiner Laterne, Laterne Laterne, Der Herbst ist da all were sung by the children. Some of the parents are native Germans, and it was clear they were reminicing about their childhood Martinstag memories. Lots of smiling faces were reflected in the glow of lanterns.

The program was entirely auf Deutche, and only lasted for 10 minutes. I was glad because the gymnasium was beginning to swelter. Meghan attempted to sing along, but didn't know the songs yet and as a result, became quite bored. She shifted and poked her lantern. A couple of times, she smashed her lantern into my face. My patience was slowly eroding. Thank goodness for short programs, though.

With what seemed like an abrupt halt, all of the families streamed into the main hallway of the school again. We proceeded East toward the playground exit. As I stopped to take a picture of the girls with their lanterns, I heard, "Gracie!"

I turned around, and there was Grace's best pal, Zoe. Her pink, piggy lantern glowed in her hand. Zoe's brother, Sam, was on his dad's shoulders. His Winnie der Pooh lantern glowed in his hand.

"Mommy, I want to go with Zoe," Grace whined.

"Alright Grace. We'll catch up to them. Now smile please." I snapped the picture, and we walked out the playground area doors.





As I inhaled, I started to cough since there several moms who had lit their cigarettes just outside the school doors.

Out of my mouth came, "Oh gross! Who is smoking?"

A response came out of the darkness, "I'm so sick of people telling me I can't smoke. C'mon. I am outside!"

I thought, "Yeah, just outside the doors of a school, where you know people are bringing their children to join the Laternenact procession. You can't wait another half an hour to get your fix, huh?"

I kept walking with the girls, but said loudly, "I have 2 children, who have a genetic disorder that doesn't allow them to be exposed to cigarettes. It is a huge deal when someone smokes around them even if it is outside. A little bit of their lung function just got damaged from your addiction."

No reply came forth, and we ran ahead to get away from the smoke. My inner evil side thought, "What a hag!"

"Why are we running Mommy?" Meghan huffed.

"To catch up with Zoe and Sam and to get away from the cigarette smoke."

Grace found Zoe, and they held hands. As they walked along, their lanterns glowed and bounced in cadence with their steps. They began singing, "Ich gehe mit meiner Laterne..."

It was a crisp fall evening, and leaves crunched under our feet. I held Meghan's hand, and she would periodically shout toward Sam, "Hi Sammy!"

It was our first Laternenacht but will be one of many we'll attend in the coming years. Time is speeding up as my girls go full speed into their school years. I soaked in the memories as I'm sure I'll eventually reflect on nights like Laternenacht as some of the best of my life.

We reached the end of the procession, grabbed our cookies, and piled back into the mini-van as it was now close to bedtime. Ah to be a kid again...

Party Bugs Update

Grace is definitely allergic to Bactrim so no more of that ever again.

We kept her off any antibiotic for 48 hours, and the pedi started her on a powerful ointment to be applied to her wound twice per day. He wants us to call on Monday to report on the progress. We'll see what happens.

When It Rains...

It pours...

Gulp. My child is one of the children that most people in my city would fear. She has a staph infection that may be MRSA, but hasn't been cultured. She is taking antibiotics for it, and we keep it covered. We wash our hands, and we encourage her to wash hers often. Even with all of that prevention, she still got a huge staph infection. I don't know why. She just did.

Last night, Grace said, "Mommy, it feels like there are bugs biting me on the inside." She pointed to her back near her right kidney. I didn't think much of it at first since Grace is often in sensory overload, but I did tell her that if the "itchies" got worse to come find me in the night.

"Okay Mommy." Her sleepiness must have overruled the "itchies."

In the morning, I gave her 2 teaspoons of Bactrim as prescribed without thinking much about those "itchies." When she came home from school, the tops of her hands were flaming red sort of like a sun burn. I knew the drug made her photosensitive, but she claimed that she was wearing her mittens on the playground.

At dinner, Grace said, "Mommy, I'm really itchy." She scratched the sides of her torso up and down and said emphatically again, "I'm itchy!"

A light bulb turned on inside of me.

"Crap, she must be having a reaction to her Bactrim. Crap!"

I turned to my husband and said, "Great. Now, we have to take her to the doctor for a third time in two weeks." Don't misunderstand me though. I have no problem taking her to the doctor, but I feel like Gracie can't catch a break here. It really can't be fun to take antibiotics all the time.

MRSA, hmmmm?

Party Bugs

When Grace was two years old, she developed a pretty high fever which scared me quite a bit. It was the first time I had seen her get so incredibly ill from a virus. She was beginning to really understand what was happening to her then, and that day, I began, what I think will be, a life long journey of helping Grace to understand the world around her.

I don't recall the exact conversation anymore, except for two words that sprung forth from my explanation. At that time, my inexperience as a parent was giving me much to ponder. I was literally stumbling over my words with her, all while Grace was suffering from a 104 fever.

Grace kept asking me why she didn't feel well. She wanted to know why, to get it, to understand. Gracie's fever made her more adament than ever. She was determined to know what the heck was making her feel so bad.

I struggled to come up with something to appease a two-year old. I hemmed and hawed and even thought that Grace might be delirious.

The final result was that I explained to Grace that there were "party bugs" having a dance party inside of her, and she needed to take her medicine to kick the bugs out.

This explanation has transcended her current state of knowledge. We mention party bugs frequently in our house now.

Recently, some nasty party bugs took up residence inside of Grace. I'm worried about it, but hoping that things turn out for the best.

I took Gracie back into the pediatrician today because the "boil" on her collar bone had become angry looking, had grown larger, and a tell-tale halo of infection was beginning to appear again. The pedi thinks it may be MRSA, Methicillin-resistant Staphylococcus aureus. He didn't culture the infection though. He wants to see if the bacteria responds to a drug in the sulfa family first.

She began taking Bactrim tonight, and after her shower, her wound opened up. The party bugs erupted from their house inside of her, and hopefully, this is the beginning of her healing.

"Mom, are the party bugs getting kicked out by my new medicine?"

Isn't she so smart?

Diva Ballerina

Ever have one of those parenting days that just sets off the most ridiculous scenarios? I know I had one last year at Christmas time when Norovirus made its way through our household.

Michelle at In The Life of a Child wrote this laugh out loud mama post recently. Enjoy her Diva Ballerina!

http://inlifeofachild.blogspot.com/2007/10/groceries-diva-ballerinas-and-things.html

Curve Ball

As I walked down the long hallway of the school, Grace plopped her hand into mine and started swinging our arms back and forth. She was skipping, and I was shuffling along side her.

"Let's see if Tante is in her classroom today," I said.

I stuck my head inside the door of her classroom, but Tante wasn't there. Grace hopped up and down beside me as she usually does before school. As I backed up to re-enter the hallway, I noticed two figures toward the end of the hallway. I recognized their profiles: Melissa's tall thin figure and Tante's belly was in its telltale form. There was no mistaking her pregnant figure...all out in front. I smiled as Gracie said, "There's Tante!"

Tante responded, "There's my Gracie!"

Melissa said, "Good morning Grace." as she turned around and walked down the hallway toward her classroom.

"I need my hug, Gracie!" Tante added.

As Grace wrapped her arms across Shanna's belly I said, "Big day today."

"Yep. Today's the day."

Later that day, Tante would have her 20 weeks ultrasound, where they would find out the sex of the twins.

"Well Gracie, we better get you into the gym (for her morning before school care)." I instructed. Grace ran off toward the gym, and we exchanged our morning hugs and kisses before I went off to work.

When I left the gym, Tante had gone somewhere else. I was excited for her, but began to focus on getting to work on time.

*****************************************************************

I had very little sleep the night before, and later that day, left work early. I was home before my girls and Charlie were. I was taking advantage of the quiet and had snuggled into my bed to grab a nap. I had just drifted off when I heard a loud buzzing sound. It startled me awake.

I finally determined it was my cell phone vibrating on top of my jewlery box. I grabbed it, and said "Hello."

"Hi Jen. Where are you? Work?" Tante asked.

"I'm at home. I came home early."

"Oh. Can I come over? I'm only about a block away right now."

"Sure." I responded sleepily and then I hung up the phone. "Gee, she sounded happy. I wonder if the twins are boys or girls."

I came down the stairs to the first floor of my house, and surveyed the gigantic mess which had accumulated over one weekend of me needing to work extra hours. I laughed and thought, "Oh well. She knows us very well."

I peeked out the window of my front door, and saw her silver car pull up. Tante got out and waddled up the front walk. She had a serious look on her face, and it made me nervous instantly. Having been on the receiving end of pregnancy complications twice, my "oh crap" radar went off inside my head. I was hoping I was wrong.

We sat down at the kitchen table, and I immediately asked, "What's up? What's going on?"

Tante sighed deeply, "I have twin-to-twin transfusion syndrome."

There it was...the "oh crap" moment I was hoping wouldn't happen. My dear friend had wanted to be a mother ever since we had shared an apartment as bachelorettes in 1996. She had some odds stacked against her in getting pregnant, and miraculously, it hadn't been an issue for her and her hubby. She was so excited, but very "morning" sick very quickly in the pregnancy. In fact, when she had her first ultrasound, and it revealed two babies, Tante being very sick made a lot of sense. Twins bring on lots and lots of HCG, which amplifies the morning sickness.

"What did Dr. C say? Tell me what he told you."

"My babies could die. I going to a specialist to find out for sure, but Dr. C thinks I'll need surgery to fix this, and then bedrest."

"Oh Tante! I'm so sorry this is happening to you..."

We kept on talking, but from my perspective, Tante was just going through the motions. She was in shock, and nearing tears. I fumbled through my words. I attempted to guide her in taking each day one at a time from that point on. I'm pretty sure she didn't hear much of what I said.

When I was diagnosed with severe preeclampisa, all that I heard was my life was in danger and so was my baby's. It was as if my ability to take in information was dammed off by those words. Nothing else could get in. Nothing. No emotions. No information. Just pure panic. I don't know for sure, but I imagine Tante was replaying "my babies could die" over and over in her head.

As Tante walked out my front door and toward her car, tears pooled in my eyes. I didn't want this for her. Why in the world do bad things happen to good people? Why? I kept wondering and worrying about her and the babies.

Tante has had a whirlwind of a week. She had the appointment with the specialist, who told her that he would need to perform surgery on her placenta. The surgery went well, but it was now a wait to see what happens time frame. So far, both babies are hanging in there. Tante is now on complete bed rest, and those babies, hopefully, will stay inside of her for a long time. God willing.

Most of you don't know Tante, but I ask that you pray for her and her beautiful, still growing baby girls.

Tante was an amazing amount of support and love for our family during both of my pregnancies and NICU stays. She helped keep Charlie sane when my life and my daughters' lives were in danger. Tante's ability to just listen to me during those scary, dark times was like a beacon of light. I only hope I can help her through what may be challenges in the upcoming weeks and months. Our family was dealt several curve balls in the last 5 years. I only hope we can help Tante and her husband to hit this curve ball right out of the park!

Please pray.

Happy Birthday to me...

Trick Or Treat

I switched off NPR as I turned into our alley. A young girl about 8, ran past the front of the van. Her witch hat bounced with each step, and the fake green hair of her wig wriggled as she ran. A few other neighborhood kids scurried past my van on their way to the H's house for the hot dog party.

I smiled as I figured my girls would be waiting for me at the back door. Their faces would be eager, and their bodies would be jumping around to the tune of "Mommy is home! Mommy is home!"

My assumption lived up to its expectation. :)

We immediately ascended the stairs to the girls' room, and found the princess costumes which would adorn my beauties. Meghan could barely contain her enthusiasm as Halloween was perhaps the most exciting thing ever in her entire life. She bounced, skipped, and clung closely to me as I gathered all the necessary components of her "most-beautiful" Tinkerbell costume. I kept picturing that little puppy in the Loony Tunes cartoon that would jump over Spike the bulldog as he marched down the street. "Is it time yet? Is it time yet? I want my costume on! I want my costume on! Is it weddy (ready) yet Mommy?"

I didn't answer, but Grace felt the need to fill my silence. "Meghan, Mommy is almost done. I'll go get your wand." I grabbed Grace's costume too.

"Meghan, come here. Your costume is ready."

"Yaaaaaaaaaaaaaaaaaaaaaaaaaaaaay!"

She wiggled and danced as I pulled the white turtleneck shirt over her head. Next, she put her hands on my shoulders as she stepped into the Tinkerbell green dress. She slipped her arms into her Tinkerbell wings, and finally wriggled on some "leg socks" to complete the ensemble. I straightened out her shirt underneath the bodice of her dress, and Meghan danced and twirled and jigged around the room. "I'm a beautafull Tinka Bell! I'm a beautafull Tinka Bell!"

She really did look like Tinkerbell. She is pixie-like in most ways physically, and her yellowy blond hair accentuated the whole look. I thought, "Wow, she is too frickin cute in this."



"Mooommmmmmy! I want my dress on," Grace emphatically shouted as she realized that Meghan had gotten dressed first.

"Hold on Gracie, I'm looking for your tiara. Where did it go?"

"I don't know. Meghan must have taken it."

"Well, it is gone Grace. I can't find it here in your pigsty of a bedroom Grace."

"Meghan did it!"

"Grace, no she didn't. It is here somewhere, though. How about we put this barrette in your hair instead?"

"Okay, Mommy. I like that one."

I thought, "Wow, that was so easy...almost too easy. It must be the allure of the candy that is keeping the tantrum at bay today. Wow!"

Earlier, Grace had not seemed as excited as Meghan, but all of a sudden, she could no longer stand still either. Her enthusiasm came in the form of jumping up and down, as I attempted to catch her limbs in rhythm with the jumps.

Grace slipped on her white long sleeved shirt, and I found the Barbie, Princess Rosella, Island Princess dress of low quality material, but exceptionally sparkly and glittery fabric. The turquoise "chiffon" clung to her arms, and I pulled her long blond locks out of the back of her shirt. She also wriggled into her white tights, and then spun around in a few twirls. Her costume shimmered as she spun.

"Mommy, we forgot my feathers."

"Okay Grace. Let's put them on now."

I fastened the pseudo peacock feathers to her dress, and wondered to myself just why exactly would a girl princess have boy peacock feathers. Her long blond hair helped her to look like Barbie, too. She was beautiful. She is getting so big so fast. Where did my baby Grace go?



The transformation was complete. Princess Grace and Pixie Meghan were ready for the fun. They both raced downstairs to gulp down some dinner before heading out for their candy rewards.

After dinner, we opened the glow stick packages. Their cousins Chloe, Molly, and Trey had arrived, and were coming along in their most beautiful princess ensembles too. Chloe wore a hot pink Jasmine costume, and Molly was a Cinderella bride. Molly wore her Aunt Laurie's veil, which made me smile. Her Aunt went to Heaven in 2004 shortly after Meghan was born, and I know that Laurie would have loved seeing Molly wearing it with pride. Their baby brother, Trey, was in a darling little monkey costume.



I cracked and snapped the sticks, and a soft purple hue glowed brightly in my hands. I put a glow stick in each of their pumpkin buckets, and we headed for the front hall closet. All the girls clamored for their coats. Simply put, they were "itching" to get out the door, and it made me giggle a bit.

As we walked down the front path, the leaves rustled under our feet. Pumpkins glowed orange. Ghosts, gobblins, witches, princesses, and parents moved about gathering the booty.






Grace enjoyed holding Molly's hand as they paired up down the sidewalk. Meghan clutched my index finger, and pulled me up each path to the houses. Chloe wondered aloud to herself why nobody seemed to know who she was. Afterall, it was easy to see that she was Jasmine from the movie Aladdin.

A few times, Meghan's enthusiam could not be contained, and she would let go of my hand as she bolted to catch up with the big girls. Her orange pumpkin bucket jiggled and her Tinkerbell wings bounced up and down in cadence with her steps. "Hey guys, wait foooorrrr meeeeeeee!"

Grace and Molly continued to walk hand-in-hand with their pumpkin buckets in their free hands, and then I noticed that I had forgotten to take Grace's peacock feathers off. They were now sticking straight up out the back of her jacket near her neck.

"Do you want me to take your feathers off Grace?"

"Nut uh Mommy. I like them. They're kinda like a turtle neck."

My sisters and my brother's girlfriend walked with us. It was a warm evening compared to some of our past Halloween's here in the frozen tundra.

It made me remember running around as a girl with my sisters and brothers. It seems so long ago now. Life seems to be speeding at a faster pace as I get older now.

I want to savor each moment of tonight. Meghan's Tinkerbell wings bouncing up and down in the shadows. Grace's hand firmly placed into Molly's. Chloe's independence. Trey shuffling down the sidewalk with his Monkey ears at attention. Molly's light up shoes. Charlie and his Mom proudly handing out candy while a fake skull shouted out spooky sayings. Kristen and Bob enjoying their little man. Lauren and Sarah guiding the girls and Lia. Our neighbors gathered around. Children laughing, running, and getting spooked. Children with sugar highs. Children being children. Adults yearning to be children again. Adults soaking in the traditions. People living life.

Owie, It Hurts

When Gracie was a baby in the NICU, she had several leads on her chest to monitor various vital signs and such. I remember the circular stickers that attached the leads to Grace covered nearly all of her chest. I have a vivid memory of watching her tiny 3 pound body breathe in and out. Her retractions made me very nervous. I kept willing her to calm the rapidity of her breaths. My telepathic notions didn't work, but some time and patience did.

Preemie skin is often frail, and the NICU used something they called "second skin" to help protect Grace's skin from sores while in their care. Grace only ended up with one tiny scar on her collar bone. It is pea-sized and slightly raised. Recently, Grace asked me what the bump was on her chest. I told her how it got there, and she said, "I like to touch it."

Grace often gets obsessed with the textures of things, which I think is tied into her sensory issues. From the time she was an infant, she was obsessed with touching both of her ears. Whe she was a baby, she'd even grab her ears while we were feeding her. Grace's need to touch and hold and carress things is sometimes very profound.

A few days ago, I was helping her put a shirt on before school, and I noticed the scar was red and angry looking. I instructed her to stop touching the bump, and she seemed to understand. I even thought that maybe she had received a bug bite or something like that.

Today, when she woke up the pea-sized bump had morphed into a 50-cent piece sized infection. The color of it was now redish purple, and a halo of inflamation was surrounding the bump.

So, today we went to the pediatrician. He isn't sure what the bump is except that he thinks it is some sort of boil. By touching the scar so much she may have accidentally introduced a staph or strep infection into the area. I also asked the doctor if it was possibly something called panniculitis, which can afflict people who have Alpha-1 Antitrypsin Deficiency. He said he wasn't sure but that he'd check into it. My gut feeling is that she has an old fashioned boil.

We're trying a broad spectrum antibiotic to see if that will help. I'm also supposed to apply warm compresses, and put Neosporin on the bump. Grace acts like I'm electrocuting her when I get near the bump though. It must really hurt her.

I hope the medication helps and that Grace's former scar doesn't become larger. Isn't it interesting how her early beginning in life keeps coming back to haunt her?

Thank You Mama

Whoever said people don't need validation of our feelings is/was just plain lying.

I want to profoundly thank "Mama" who commented on my Flashbacks post. She said, "Thanks for making me feel normal today. You aren't supposed to "get over" it. You are supposed to learn to live with your "new normal" life, and in that life OB/GYN visits and seeing pregnant women are hard things for you. It seems like you are doing that."

Sometimes it takes a complete stranger to help me. So many of my friends and family, who love me dearly, just don't quite know what to say to me after all this time. They don't understand why preeclampsia was such a profound life changing experience.

I assume they feel sorry for me. Or maybe they don't want to bring up a bad subject? I assume they just don't understand. I assume mostly because few have truly acknowledged what happened to our family. (Thank you to those of you who have acknowledged it.) In typical cursory fashion, most say "Wow, but it's over now." Nope, not really. Or, "why do you keep talking about this?" Or, "Why can't you get over it?"

It will never really be over for me. My life was altered by my experiences. I am forever changed physically and emotionally by preeclampsia.

All that I can do now is accept and embrace my new normal: the Jen who survived severe preeclampsia twice, the Jen who was a NICU mom twice, the Jen who now worries about what might seem to be the most mundane of things when it comes to her kiddos.

Thank you "Mama" for your acknowledgement of my experience. Thank you!

Alpha-1 Carriers and Anxiety/Bipolar Disorders???

Wow, I guess I would have never put these two together. Interesting, but I'm not sure I completely understand it...

Art, alpha-1-antitrypsin polymorphisms and intense creative energy: Blessing or curse?

NeuroToxicology
Volume 28, Issue 5, September 2007, Pages 899-914
Twenty-Third International Neurotoxicology Conference: ”Neurotoxicity in Development and Aging”

Donald Everett Schmechel, Department of Medicine, Duke University Medical Center, Medical Director, The Falls Neurology and Memory Center, 4355 Hickory Boulevard (US 321), Granite Falls, NC 28630, United States
Received 1 February 2007; accepted 21 May 2007.

Abstract

Persons heterozygous for Z, S and rare alpha-1-antitrypsin (AAT, SERPIN1A) polymorphisms (ca. 9% of population) are often considered ‘silent’ carriers with increased vulnerability to environmentally modulated liver and lung disease. They may have significantly more anxiety and bipolar spectrum disorders, nutritional compromise, and white matter disease [Schmechel DE, Browndyke J, Ghio A. Strategies for the dissection of genetic–environmental interactions in neurodegenerative disorders. Neurotoxicology 2006;27:637–57]. Given association of art and mood disorders, we examined occupation and artistic vocation from this same series. One thousand five hundred and thirty-seven consecutive persons aged 16–90 years old received comprehensive work-up including testing for AAT ‘phenotype’ and level, nutritional factors, and inflammatory, iron and copper indices. Occupations were grouped by Bureau of Labor Standards classification and information gathered on artistic activities. Proportion of reactive airway disease, obstructive pulmonary disease, and pre-existing anxiety disorder or bipolar disorder were significantly increased in persons carrying AAT non-M polymorphisms compared to normal MM genotype (respectively, 10, 20, 21, and 33% compared to 8, 12, 11, and 9%; contingency table, pulmonary: χ2 =37, p = 0.0001; affective disorder: χ2 = 171, p = 0.0001). In persons with artistic avocation (n = 189) or occupation (n = 57), AAT non-M polymorphisms are significantly increased (respectively, proportions of 44 and 40% compared to background rate of 9%; contingency table, avocation: χ2 = 172, p = 0.0001; occupation: χ2 = 57, p = 0.0007). Artistic ability and ‘anxiety/bipolar spectrum’ mood disorders may represent phenotypic attributes that had selective advantage during recent human evolution, an ‘intensive creative energy’ (ICE) behavioral phenotype. Background proportion of ICE of 7% consists of 49 of 1312 persons with AAT MM genotype (4%), and 58 of 225 persons with non-MM genotypes (26%) (contingency table, χ2 = 222, p = 0.0001). Penetrance of ICE increases in genotypes with lower AAT levels: PiMS, 18%; PiMZ, 44%; PiSS and PiZZ, 100% (five cases). At all ages, persons with non-MM genotype had significantly higher proportion of thiamine deficiency (50% in PiMZ), reactive hypoglycemia (20% in PiMZ), and possibly fatty liver (thiamine: χ2 = 28, p = 0.0001; hypoglycemia: χ2 = 92, p = 0.0001). In older persons, PiMZ genotype had significantly increased proportion (46%) of brain MRI T2 white matter abnormalities (χ2 = 49, p = 0.003).

Persons with ICE and MM genotype showed increased prevalence of pulmonary disorders and same signature as S and Z carriers and homozygotes (see above). Z polymorphism was associated with delayed age of onset (average 7 years) for persons with toxic environmental or occupational exposures (log rank, p = 0.0001) and more stable cognitive change in persons with neurodegenerative illness (p < 0.05). At all ages, ICE phenotype and Z polymorphism were associated with altered copper homeostasis with low or absent non-ceruloplasmin bound copper (p < 0.05). AAT polymorphisms which affect iron, lipid and copper metabolism may affect early events in nervous system development, function and response to environmental exposures. AAT may also be a ‘switch’ for copper metabolism and low ‘free’ copper would be theorized to provide protection for lipid oxidation and favorably affect beta-amyloid and other aggregation, but possibly alter early ‘critical’ period of CNS development. AAT polymorphisms may define an important and treatable subset of persons presenting with CNS disorders. This new proposed phenotype for AAT transcends classic pattern of strictly liver and lung disease, and should be considered for proper evaluation and management of patients presenting with classic AAT-related disorders, affective disorders, persons with ICE, white matter disease or multisystem disorders of memory.

Fall Color

Mama So Proud

Today, Meghan had her first trip to the pumpkin farm. I've missed virtually all of Meghan's "firsts" throughout her life for various reasons, but this one, I could control. I could be there.

While carting two of Meghan's preschool friends and her teacher, Miss Amy, I struck up a conversation with Miss Amy. I was interested in hearing how she thought Meghan was doing in K3. I've always wondered if Meghan would need to be "held back" an extra year before starting K4. With her micropreemie start in life, I wondered if she'd need extra time to mature and learn before competing with her peers in school. I very vividly recall a conversation I had with her neonatologist while she was still incubating. He had indicated Meghan would be high risk for learning problems and attention deficit disorder.

I'm happy to report that Miss Amy doesn't see any lagging or delays in Meghan's abilities when compared with her peers. This makes my heart warm today! :)

The pumpkin farm was great too, but somehow this spur of the moment conversation helped me breathe a large sigh of relief for now. Meghan is still at risk for learning problems based on her micropreemie birth, but for now, she seems to be beating some of those odds. Who knows? Maybe K4 isn't as far away at it seemed yesterday?

*********************************************************************

Yesterday, Grace's class visited an apple orchard and pumpkin farm, too. I chaperoned and rode the bus with her there. She had fun sitting next to Dylan and pretty much ignored me all the way there. It is good to see her social skills are exploding now. Whew!

Tonight, we had Grace's parent/teacher conference. Last year, Grace did well, but this year she is going great according to her teacher. Teacher said "Grace is in the top of the class." Wow! Wahoo! Yeehaw!

It was a "mama so proud" day! I'm sure it was a "daddy so proud day" too.

Research into the Impact of Testing Children for Alpha-1

I think I may investigate this opportunity to contribute to the Alpha-1 community.

Hey all you Alpha parents out there, please consider participating in this
important study within our community. To be clear, this is for the parents not
the kids. Or, if you were diagnosed as a child, this study is for you, too.

Alpha-1 Genetic Testing of at Risk Children

The objective of this study is to investigate the ethical, legal, and social
impact of testing at risk children for Alpha-1. The results will inform the
development of recommendations for health care professionals on what
constitutes disclosure of risks and benefits in informed consent or assent by
children who are considering testing for Alpha-1.

As a pre-study, we will conduct 2 focus groups: one in Denver, Colorado on
November 10, 2007 and one in Orlando, Florida on December 1, 2007. To
participate in a focus group, you must be a parent who has made a decision
to test or not to test your at risk child for Alpha-1 or an adult who was tested
for Alpha-1 as a child. To enroll in the focus group or for more information
please email marilyn.coors@uchsc.edu or call 1-303-315-0203. To participate
in the study survey, please enroll in the Alpha-1 Research Registry. The study
is funded by the Alpha-1 Foundation.

To enroll in the Alpha-1 Research Registry at the Medical University of South
Carolina, read the following information:

The Alpha-1 Research Registry is a confidential database of individuals
diagnosed with Alpha-1 Antitrypsin Deficiency (Alpha-1) and persons identified
as Alpha-1 carriers. It serves as a resource for investigators seeking
individuals with Alpha-1 to participate in clinical trials, surveys, and other
scientific and medical data collection activities. The Registry’s Family Linkage
Program facilitates genetic research and other studies requiring family member
participation while protecting the privacy and autonomy of each family
member. The Registry is also a vital component to other Alpha-1 research
endeavors such as the Alpha-1 Coded Testing (ACT) Study and Genetic
Modifiers Study. The Registry is conducted under the direction of Charlie
Strange, M.D. at the Medical University of South Carolina. For more
information about the Registry Program, you may visit the website at
www.alphaoneregistry.org, email at alphaone@musc.edu or call toll free at 1-
877-886-2383.

I Love Photoshop Elements

I've embossed my girls. haha

Comedic Interactions

...between Meghan and Grace, and Meghan and Charlie.

Recently, we were on our way to my in-laws house for a visit. Grace and Meghan were fastened into their car seats appropriately. About half way there, Meghan began to make some vocalizing sounds sort of like when she was baby and had just discovered that she had a voice. Her squeals morphed into an exhaling song of some kind, which she was making up as we drove.

It was beginning to grate on me and Grace.

"Meghan, please stop!" I asked. "You're getting too loud. Use your inside voice."

Songs continued to come out of her mouth in an increasing tone and volume.

"Meghan!" I said again.

"Meghan! I don't want to hear another peep out of you!" Grace exclaimed.

To which, Meghan placed her index finger on her thumb to form the equivalent of a chick's beak and began to taunt Grace with:

"Peep! Peep! Peeeeeep! Peeeeeeeeeeeeeeeeeeeeeep!"

We all began laughing our heads off.

I was stunned at her ability to judge her sister's frustration with her, and keep pressing Grace's "buttons" further.



***************************************************************

On Thursday, Charlie, Meghan, and I were gathered around the kitchen table. Meghan was picking at her food, and Charlie and I were coaxing her to eat just a few more bites of this or a few more bites of that.

Eventually, the dinner began to whind down, and Charlie began to tell Meghan how they would be going to the library after dinner. He began setting the stage of going to the library in his best parental tone.

Meghan blankly stared at him and responded with this: "Blah! Blah! Blah! Blaaaaaaaah!"

Charlie and I couldn't help but laugh. I envisioned a Peanuts-like "wa wa wa. wa wa."

I can't help but wonder if we have a budding commedian in our midst. LOL

Mommy William Tell Overture

This is just so unbelievably funny to me. For all the mothers I know, please watch!

Flashbacks

As I walked across the parking lot, I noticed that there was still salt in the cracks of the asphalt. It had been there since spring when the snow finally melted. A misty wind hit my face, and I squinted to spot my minivan in the lot. October was here, and I was on my way to my annual exam with my OBGYN, Dr. C. As I hopped into the drivers seat, the rain began to pour down.


Photo: www.microsoft.com

I switched on my wipers, and turned on my headlights. The sky was a slate color and fog was coming in waves in front of me. I made the drive with ease down the highway, though.

My van glided off the exit ramp, and I made the turns to arrive at Dr. C's office.

As I turned left into the drive way of the office building, I began to have flashbacks of being there before. I wasn't prepared for it. I wasn't ready to "relive" it in my mind today. A lump formed in my throat, and I stuffed down the urge to cry. I know I'm supposed to be over this by now, but it just isn't going away. I really don't think it ever will.

I scolded myself saying, "No Jen! Not now!"

Every year with my annual exam, I face the past. It is an internal struggle for me. I was supposed to go for my visit in June. They had already sent me 2 reminders, but I just couldn't bring myself to make the appointment until last week. What finally made me call was the story of a woman who died of cancer. That finally clicked with me, and upon calling, they fit me in since I was overdue. (Upon rereading this, I laughed at the prospect of saying I was overdue, that never applied to me when I was pregnant.)

As I left my van, I clutched my umbrella and a white piece of paper I wanted to share with Dr. C. I walked quickly through the parking lot, and rain water was rushing past my feet down an incline toward a drain.

As I opened the door, I inhaled deeply to prepare myself for seeing pregnant women in the waiting room. I noticed a sign that said bone density screenings were on the second floor. Fortunately, this was enough of a distraction for me. My mind wandered considering just what exactly a bone density test entailed. I found myself at the desk, and checked in.

As I turned around to sit down for the wait, I noticed a woman who appeared to be close to 9 months pregnant. Her face seemed puffy, and I noticed her ankles were swollen. I wondered if preeclampsia was in her destiny. She was quickly called back to her exam room, and this was a relief for me. I hate feeling jealous of pregnant women at the same time I'm scared to death for them. To me, pregnancy is an illness and with it comes horrible consequences. It isn't a time for joy and anticipation.

It starts with unbelievable nausea and vomitting, which lasts until to the mid-way point. Then, the headaches start. Shiny silvery flashing lights appear in my vision, and then the "fireballs" appear before my eyes. The incredible swelling comes, which results in stretching, painful skin. My central nervous system gets irritated, and every thing results in insults to my senses. Eventually, my blood pressure creeps up to scary levels, and then my kidneys start to malfunction. The baby stops moving around in a scary silent kind of way, and I start to obsess about counting kicks or movements. I poke and prod my belly to make sure baby is alive in there. I stuff down the fears and focus on the kicks. I'm so swollen that my teeth are tight inside my mouth, and it irritates me that I can feel my pulse in my gums surrounding my molars.

All of this is flashing through my brain. All because I'm facing my past. Twice I suffered from severe preeclampsia. Twice I had premature children. Twice it didn't go well for me. Twice I felt like a failure. Twice I prayed every moment my child was fighting in the isolette. Twice I cursed me and my failure of a body.





There I was in the waiting room hoping like hell that my name would be called soon. I just wanted to get out of there. I wanted it over. Reliving it again was too much for me.

To distract myself, I looked around the room to check the door where the nurse would appear to call me back to my exam room. There she was. It was Erin. I hadn't seen her in a few years.

I thought, "Not you too."

Erin was about seven months pregnant, too.

"Jen, come on back."

Dry At Night

When Grace was nearing age 4, I began to question when exactly she would sleep through the night without the assistance of a “princess panty” otherwise known as a pull-up. As is typical of me, I began asking other mommies when their children had accomplished the milestone. I received several answers, all of which made me feel a little inadequate in the parenting department since the ages included in the answers were younger than Grace’s age at the time.

“Hmm,” I thought.

I didn’t want to make a big deal out of it, but we began to implement restrictions on amounts of fluids after 7:00 p.m. Even with the restriction, the morning pull-up was flooded.

I thought again, “Hmm. Wonder what we’re doing wrong?”

So, I went to the source: The Internet. It seemed that we weren’t doing anything wrong. She just had an immature bladder and was a very deep sleeper for 12-13 hours per night.

“Great!” I thought. “We’ll just wait this out. It was no deal to purchase the pull-ups anyway.”

Eventually, Gracie began to ask me things like:

“When will I stay dry at night Mom?”

“Why can’t I stay dry at night Mom?”

I just kept telling her that it was okay, and that her body would learn how to stay dry at night. She seemed pretty okay with that explanation, but then she must have asked some of her friends if they stay dry at night. Their responses must have been to the contrary of Grace’s experiences. Her inquiries resulted in repeat conversations between us about how her body would eventually learn how to stay dry at night. Occasionally, she would cry and worry that the inevitable would never happen.

At her 5 year old check-up in May, Charlie had asked the pediatrician whether we should be concerned or not. At that time, Dr. M said that he wouldn’t worry until Grace was age 6. So, again, I figured we’d wait and see what happens, but in all honesty, I had begun to worry that her younger sister, Meghan, might figure it out before Gracie did.

Gracie and I even had a conversation a couple months ago about how she could decide if she would want to tell the other kids at school that she couldn’t stay dry at night. She opted for leaving that part out, which I said was probably for the best seeing as kids will be kids.

Well, almost 2 ½ years have passed since I felt like I was bumbling my way through motherhood and potty training. With my fingers crossed, I can now report that Grace has stayed dry at night for almost 30 days in a row. WOOHOO!

She is so quirky though. :) I hadn’t been paying too much attention to her pull-up results since she dresses herself in the morning. Last week, I asked her if she was staying dry at night. Grinning from ear-to-ear, she responded by opening a cabinet door on her desk. Inside the cabinet were at least 25 used, dry pull-ups piled high with pride. I couldn’t believe my eyes, and I immediately started to laugh and hugged her close.

Way to go Gracie!



We’ll be finishing up the package of pull-ups, and then Grace will be in the big kid world of underwear at night. Wow, and she did it before Meghan. Yes! We safely navigated away from that potential childhood tragedy.

Beautiful Alpha-1 Organ Donation Story

This is an amazingly beautiful story of a woman, just about 1 year younger than me, who received a liver transplant due to ZZ Alpha-1 in 1984. She lost her older brother to Alpha-1, but her story is so beautiful. Enjoy the reminder of life's blessings.

http://www.azcentral.com/arizonarepublic/news/articles/0923transplant0923.html

Perspective

Today, I arrived home earlier than usual because Charlie needed to be at his school for an open house. After he sped off on his motorcycle, I decided to take the girls to our local park to play for awhile. We all got in the van, and quickly arrived at the park.

"Can I go Mommy?" Meghan squealed as I lifted her out of the carseat.

"Go ahead."

Grace jumped out of the side of our minivan, and I clicked the button to lock it up. As I turned around, Meghan's petite legs were trotting up a slight grassy incline. Her tiny rear end was hidden underneath her capri pants, and her long yellow locks bounced with each step. Grace raced behind her and passed her by to beat Meghan to the playset.

It had been a while since I'd been to the park with the girls so I was pleased to see that Meghan could now climb most every part of the playset with ease. Honestly, I still have my moments of doubting her abilities. I know I shouldn't do this, but I can't help but noticing how other kids seem to take their profound agility for granted somehow. I secretly yearn to have kiddos who can "leap a tall building in a single bound." I know that is screwed up, but having been Mommy to two former preemies, it is hard not to think that way. It is hard not to remember that if I hadn't gotten sick, my babies would have been full term agility prone gals, at least that is what I would have given them...a full long ride inside my womb with little to no stress and a peaceful low blood pressure experience. Alas, it was not to be.

All-in-all, today's trip to the park provided me with much perspective. Thank you cosmic energy. Thank you God.

"Watch me Mom!" Meghan squealed as she climbed up the slide in the wrong direction.

"Meghan, you know the rules. We go up ladders and down slides."

A different voice appeared from the top of a platform on the playset. It came from a bulky little boy named Rico who also said, "Watch me Mom! I told you I could do it."

His mom shouted, "Watch out Rico. There is a little girl at the bottom of the pole. Watch out for her." Meghan was standing at the bottom of the equivalent of a fire pole so I guided her out of the way as he jumped on the pole and rode down.

I turned around to find his mom sitting at a picnic table.

"She is so cute. How old is she? Two? Three?"

I responded, "She is 3, but tiny for her age."

"I keep trying to teach him to be careful around girls and that if he is nice that he'll have some girl friends. I mean girl friends not girlfriends."

By this time, Rico had climbed up the platform and was ready to jump down the pole again. "Meghan, please get away from the bottom of the pole. He wants to come down."

"Is that her name? Meghan?"

"Yes."

"That is a beautiful name."

"Thank you. She is tiny because she was born very early. She weighed 1 pound, 9 ounces when she was born. We took her home 79 days after she was born."

"Wow, what happened?"

"I got very sick with preeclampsia, which causes very high blood pressure."

"Oh. She looks great now. I had preemie twins, who were 4 and 5 pounds when they were born."

"Aha, so you know how it goes then," I believed.

"Sort of. I gave them up for adoption because I couldn't take care of three kids. It was so hard to give them up...the hardest thing I ever done. I had them in Utah and the mom and dad were there in the NICU every day with my daughter and son. I was only there until they made me go home after my c-section."

"Wow. You were so brave to make that incredibly generous decision. It sounds like you made the right decision for you."

"I don't know why I'm telling you this. I don't usually tell anyone, but you seem so nice."

"Thank you for sharing it with me. I bet your twins' parents thank God every day that you blessed them with your twins," I meekly responded.

"Yeah. They are a white family, and my twins were biracial. My son had green eyes at birth, and my daughter had brown eyes." I wondered why she felt the need to share that detail with me besides the fact that I have the fairest of fair skin, and she was looking for a way to find some commonality between us.

"Mommy, I want to go swing! Mommy!"

"Hold on Grace! Just a minute! I'm talking to this lady. Hold on."

"It's okay. Let her swing."

Grace raced to a "big kid" swing, and Rico followed her to the other one. Meghan wimpered a bit as I placed her in a baby swing. "I'm not a baby!"

"I know Meghan, but the other swings are being used. You'll have fun on this one."

"Noooo!"

"Yes, Meghan. In just a bit, you can have a turn on the big kid swing." Rico's mom joined us and began pushing him on his swing too.

"My son had jaundice and had to go under those lights to get better."

I pointed to Grace, and said, "She had that too. Those lights work great."

"Their mom sends me pictures on all the holidays, and she sends a video tape on their birthday each year."

"So, you get to see how well they are doing?"

"Yes, but it is so hard. They are so beautiful, and I miss them so much."

"I suppose you'll miss them forever since they are a part of you, but I hope you know that you did a really great thing. You gave your twins two parents who love them, which is a beautiful gift."

"I know. It is just so hard sometimes. I still don't know why I'm tellin' you this."

Grace and Rico had gotten bored swinging and had run off to the playset again.

"Mommy! I want to stop swinging now. Mommy, I'm all done now," Meghan shouted.

I was so immersed in Rico's mom's story that I wasn't hearing Meghan.

"Mom!"

"Oh, alright Meghan. Go slide."

Rico's mom and I continued to exchange mommy small talk until it was time to go. I'll probably never see her again, but I learned a lot from her tonight.

I'm an incredibly lucky person. I have my children with me. I never had to make a decision like Rico's mom did. I have a spouse who loves me, a nice home, my health, and my beautiful children. I have so much for which to be thankful. I don't think I'll ever forget Rico's mom even though I never got her name.

Thank you cosmic energy. Thank you God.

12 years

I was in Oklahoma City this week on a business trip. Having never been there, I wasn't sure what to expect. I was pleasantly surprised to see a beautifully maintained downtown environment. The streets were clean, plants were trimmed, flowers were blooming, and community assistants made sure tourists were finding their ways around appropriately. I'd love to have the mayor of my city tour Oklahoma City. My city saddens me lately in that it is so incredibly depressed.

Anyway, I left my hotel room and walked 4 blocks to the Oklahoma City Memorial. I had wondered how close I would be to the former site of the bombing on April 19, 1995. As I walked along the streets toward the memorial. I kept thinking it was strange to know that such an unbelievable act of violence had taken place on such quiet, well maintained streets where the street signs actually say "Drive Friendly."

I felt slightly queasy upon climbing the stairs.

I wasn't sure what to expect, but I still have vivid memories of the TV footage of a fireman carrying the lifeless body of a small little girl with blond hair out of the site.

On a sprawling green lawn, carefully manicured, were chairs that symbolized all of the victims of the bombing. Upon spying smaller versions of the chairs, I felt the tears well up in my eyes. Those poor children, who were just spending their day in the building's day care center. They must have been so scared. I can't imagine.

I also thought about the individuals who were just sitting at their desks, in meetings, or were just walking by on the street. After reflecting on the reflecting pool, I walked along a chain-link fence which was still full of mementos of the victims. Many of the items were faded, but many of the items were carefully maintained. I guessed that their families come periodically to make sure no one forgets.

Even 12 years afterward, it seems so recent. When the bombing happened, it was the first time I realized, as an adult, that some people have no respect for human lives.

So many more acts of violence have happened since then. My trip to the OKC memorial reminded me that I need to enjoy life in the moment.

But I Want to Cut the Grass When I Grow Up

Grace's understanding of Alpha-1 is exploding now. Her naiveness is fading, and we'll she is one smart cookie. I hope that I can help her believe that she is special because she is an Alpha, but that it doesn't limit her (within reason). This is the hard part of parenting a child with a medical condition...explaining things with kid logic and just enough without giving too much. It certainly is a balancing act. Here is another example of our conversations lately.

While driving down our alley in the minivan, Grace noticed our neighbor cutting his lawn.

"Look Mom! Emma's Daddy is cuttin' the grass. Mom, when can we make the Alpha go away?"

"Honey, we can't make it go away. It isn't like a germ. It is part of who you are, but we are taking good care of you by protecting your lungs from bad stuff."

"Why can't I go outside when Daddy is cuttin' the grass?"

"Well, the lawn mower is kind of stinky, and cutting the grass makes a lot of dust fly around in the air. That stuff isn't good for your lungs Grace."

"But I want to cut the grass when I grow up!"

"Honey, you will be able to cut the grass, but you may wear something called a respirator over your mouth and nose to keep the yucky stuff out of your lungs."

"Oh. How come Daddy can cut the grass? He has Alpha?"

"Grace, remember how I told you that Daddy and I have a different kind of Alpha-1 (we're carriers) than you."

"Uh huh. Well, Daddy can cut the grass because his Alpha-1 is different than yours. Do you know who has Alpha-1 just like you?"

"Uh huh...the Alpha friends (our local support group members)."

"Yes, and your sister, Meghan, has the same kind of Alpha-1 as you do. You can both be special Alpha Girls together. Remember that you can do all the same stuff as the rest of your friends. You just need to keep yucky stuff out of your lungs like nasty cigarette smoke and cutting-the-grass stuff."

"Oh, but I still want to cut the grass when I grow up."

Disenchanted

As I approached the front door of the home, I paused and composed myself. I guessed that I'd have a hard time getting through the meeting. After all, I had decided I needed to tell them I could no longer serve.

I gulped some air, and entered. I walked in the house behind another woman. The individuals in the living room quieted. A few minutes later, we gathered around a large oak table for the meeting. Several agenda items were to be completed. All that I wanted was for the last item to arrive. I wanted to say what I had to say, and get out of there. With much anticipation, I shifted in my chair and managed to gulp down some food. My nerves were on end, and I'm pretty sure my blood pressure was rising.

Eventually and finally, we arrived at the agenda item I anticipated. I informed the group that I'd be stepping down because of some accusations that had been made in regard to me. None of the accusations were true.

Most of the individuals stated that what I was told and believed was simply not true. I couldn't believe what was unfolding in front of me. Even upon confrontation, the truth would not come out. It was disappointing human behavior. Tears kept welling up inside me, and they eventually flowed.

I left, and amazingly, I felt relieved. I had said what I wanted to say...whether they actually heard and believed me, I'll probably never know.

************************************************
Some days, it is simply amazing how human behavior has allowed our species to progress as far as we've come.

As you may know, I've been immersed in the Alpha-1 community since Grace was diagnosed in 2002. I've always believed that this was a good thing to do. Considering my need to understand, gather all the facts, and form an educated opinion of how Alpha-1 can affect my family's life, I initially felt like I had found a new "home" where other community members would understand and welcome me. These precious few would understand, and hold me up in tough times.

Much to my dismay, and possibly because I'm quite naive, I've lately experienced a pure opposite to my former naive beliefs.

Why is it that minority groups feel the need to splinter? Why do we need to align ourselves with one augmentation company over another? Why do we "split hairs" over who is liver, lung, or skin affected by Alpha-1? Why is it that I've supposedly not been in the community long enough to supposedly understand? As if, I'm some young pup to be mentored.

It is an amazing bunch of bull if you ask me.

Why is it that matters? Aren't we all members of the Alpha-1 community? I don't give a flying fig what your phenotype is? I don't care how long you've been diagnosed? You are a member of my community and deserve to be among us.

Instead of banding together into a group with strength and unity, we instead splinter and fracture into he-said, she-said. Gossip becomes "truth" without basis in fact, and emotions rule what would be normally calmer heads.

It doesn't matter what is fact or fiction. We believe what we're told without a frame of reference and embrace the "false" truth. It is a sorry state of affairs.

If this keeps up, our community will crumble under the weight of half understood issues and lies. All that I know is that I'm sick of being accused of things I haven't done. I'm sick of individuals who lie directly to my face. I'm sick of the gossiping. I'm sick of the individuals who only see what they want to see. They'd rather see others unhappy and sour to the idea of one united Alpha-1 community.

Volunteering in my community is becoming less fulfilling. I'm disenchanted and hoping that I can keep my daughters far, far away from this ill will. One thing is for sure. I will do everything in my power to raise helpful, kind, considerate, and respectful future members of the Alpha-1 community.

Those who choose to believe that I have less than pure intentions are wrong...plain and simple.

Another Cousin Gone

Gosh, I'm pretty depressed since I recently found out my cousin, Butch, passed away on June 27th at age 44. I'm assuming his Alpha-1 liver disease is what finally released his soul. How is it that we just found out you're gone???

I hadn't seen him in many years, but it just feels strange knowing he is gone. As a little girl, I remember him hanging out with my uncle David and Butch's older brother, Tony. They would have been teenagers at the time, and they seemed so old to me then. But now I think that Butch was simply too young to die. There are too many Alpha angels in heaven. It just makes me so so sad to know he is gone. How is it that your brother, Tony, is the only one left of your siblings now? How is it that 4/5 siblings are gone from Alpha-1? Sometimes, I wonder...

Butch, I know you joined your mom, Beth, Amy, and Pam. Fly with the angels.

Wow

Grace and I were sitting next to one another on an airplane trip to Los Angeles, CA. I was leafing through one of those "mind candy" gossipy magazines, and I happened upon a picture of Christina Agulera, who is currently pregnant. She was looking quite trendy.

Grace glanced at my magazine, and inquired, "Mommy, how come God sometimes gives teenagers babies?"

I thought, "Yikes! What? Already? She is maturing too fast for my liking."

Since I didn't want to have that conversation on an airplane in close proximity to too many perfect strangers, I said, "I'm not sure Grace. What do you think?"

Grace shrugged and moved onto her next thought.

I dodged a bullet, but for how long?

First Day of School

Tuesday, 9/4, was the first day of preschool for Meghan. She was so proud. "I'm in K3! I'm in K3!"



That day was also Grace's first day of 5 year old kindergarten (K5). It was soooooooooo much easier this year to take her to school since she was already familiar with the routine and surroundings. :) Last year was quite a challenge.



I took the day off of work, and nursed my "babies are growing up too fast" wounds a bit. But, aren't they adorable?

Ode to Clean Air

Well, I'm just jokin' about the ode part...

Charlie and I just got back from sin city, aka Las Vegas. We attended our dear friend Shanna's wedding to Steve. It was 107 that day, and well, I've never worn sun screen to a wedding before. :)



In any case, it was fun to get some one-on-one time with my husband. It was down right decadent at times what with us being able to lie down on the bed, and lazily flip channels without a child whining something to the effect of "I wanna watch Dora!" I missed the girls, and they were frequently our topic of conversation.

What I didn't like about Las Vegas was the amount of cigarette and cigar smoke I encountered on what seemed to happen in 30-second intervals. Blech! Now that I've been educated about how exposure to one cigarette completely wipes out the available Alpha-1 hanging out in a person's lungs, I'm a whole lot neurotic about avoiding the stuff.

At one point, Charlie and I were walking to our elevator to go up to our room, and there were 4 young men (approximately 21 years old) carrying pints of beer, and smoking swisher sweets. Eeewwww! Immediately I tried to avoid them. Well upon entering the area outside of the elevators, the men came strolling in past humongous signs that read "It is against the law in Nevada to smoke in this location." Pas de fumer!!!!! A-holes!

I pointed to the sign, and said "You can't smoke those in here." One kid acknowledged what I said, and promptly extinguished his cigar in that sandy stuff...don't really know what it is called. Anyway, the others blew me off, and of course, the elevator comes and they decide to get on still smoking. So...

I got off the elevator, and said, "I'm not riding if you're getting on!" A-holes!

They then decided to leave the elevator because I've supposedly made them feel uncomfortable. Yeah right. One kid says, "We're really nice boys maam. We're from Canada." Meanwhile I've crossed my arms in anger, and the elevator doors close. As the elevator went up, I heard, "Bitch!" Charlie got mad and yelled back "F you!"

I know that youth makes people feel invulnerable, but it just makes me cringe to see those men smoking. I mean there are so many Alphas who'd love to have their healthy lungs, and there they go wasting them. Sometimes, I just don't get it.

An Alpha Realization

As I was tucking Meghan into bed tonight, I told her that she would be going to the eye doctor tomorrow.

"Why'm goin' ta the eye docta Momma?" Meghan inquired.

"To get your eyes checked out Meggie so that they stay healthy. Sometimes, kids who were born too early need to get glasses like the ones I'm wearing Meggie. Daddy will take you to see if your eyes need glasses."

Meghan responded, "Okay, Mommy."

Grace joined in, "Mommy were you born too early?"

"No honey, I wasn't born too early. I was born one week late."

"Did your mommy get sick when you were in her tummy?"

"No honey, but my mommy's mommy got sick from those nasty "party bugs" called preeclampsia like me.

"Mom, when can we make the Alpha go away?" Grace pondered.

Ah ha, there it is again. Gracie is growing up so quickly now, and her self-realization is expanding.

"Alpha-1 is not party bugs Gracie. Honey, we can't make the Alpha go away. It is part of who you are. All that we can do is take care of ourselves by staying healthy. It is in your genes, and I'm not talking about the jeans that you wear on your legs."

"How come Havalah got a new liver Mommy?"

"Her liver got very sick, and she almost had to go to heaven. Thankfully, they put a new liver inside Havalah, and she is better now. She didn't have Alpha honey. She had something else wrong with her liver."

"Oh."

"Honey, if you have questions about Alpha-1, always ask Mommy. We can figure out your questions together. Okay?"

"Uh huh."

"Now, you get some sleep."

Rage

I'm not sure where to begin except that I was on the receiving end of someone's rage today. I'm confused.

When I was about 5, I visited my grandparents' home. My sister and I had been playing in the side yard of the house near the garden. We were running and jumping and doing what little girls do best. At some point, I decided to go inside to use the bathroom. I scrambled up the steps, and opened the screen door. An old-fashioned black and white tile kitchen floor appeared beneath my feet. On my right, I could see the thick pedastal of the oak kitchen table. What shocked me into paying attention was the arguing and yelling and swearing. I didn't know what it was about, but the argument was quickly escalating. My grandfather grabbed my grandmother's wrist in a rapid wrenching motion. Grandma swore at Grandpa. I must have gasped because the next thing I heard was my grandfather shouting at me, "You! You get outta here." It freaked me out, and I quickly retreated. I ran as fast as I could, and grabbed my little sister. We ran into the garage, and crouched down low. My sister was confused, but I didn't want to say anything. I just stared at the cement block walls of the garage, and collected myself. I recovered in a few minutes. When my mom arrived, I told her what had happened. I don't recall what she said, but I will never forget seeing unbottled rage for the first time. Ever since, I have dreamt of what I saw.

To be honest, today's run-in with rage scared the hell out of me again. It only lasted about 30 seconds, but it was enough to make me question a whole lot of the decisions I've made in my adult life. I wasn't prepared for it, and it was petrifying.

I spoke a few words before they were cut off abruptly. A fist came crashing down on the table close to me. Arms went flinging up and a foot slammed a chair backward. The chair fell over. Another person was nearly injured by the chair falling. As I sat in the chair, a pointed index finger was within a few milimeters of my nose. It was meant to intimidate me, and the person succeeded.

Although I did initially tremble with fear, my next reaction was one of preservation. To put it bluntly, I was not going to be subjected to that rage, and I removed myself from the situation. I had to protect myself.

When I was a teenager, I dated someone who had a problem with rage. At one point that person punched the slate top of a pool table in anger. His rage was never taken out on me, but I've always wondered to myself. If I had stayed with that person, would that rage ever have been redirected into the form of hurting me? I suppose I'll never know, but I do know this:

When the fuse was lit, there was no way I could calm the person down today. It literally was like TNT was about to blow, and well, all of us around the enraged person knew it was going to happen. It was glaringly obvious in demeanor, expression, and in a bright red face. One ran away to try to hide. Another shook and burst into tears. All I wanted to do was diffuse the situation. I wanted it to stop. It was terrifying me and those around me. It was not warranted. It was not deserved. It was not appropriate. It was not fair. I will never forget it.

Why can't we all just get along? Why does the stupidest thing set someone off? Why am I shaking as I write this? Why?

Connecting the dots...

Is is possible for a 5-year old to begin philosophical thinking? You know. The kind of person who thinks of how we came to be. I'm reminded of that character on Northern Exposure who was the disk jockey. Chris was his name. In one episode, Chris wonders if we are a flea on the tick's back or something to that effect. Tonight, Grace seemed to be spiraling into a philosophical debate with herself and me.

"Mom, Zoe says that when we go to Heaven that our whole body goes in the ground...like our skin and everything. I told her it doesn't."

"Grace, everyone has a different idea of what happens when we go to Heaven. Next time Zoe talks about that, tell her she needs to ask her mommy and daddy about it. Okay?

Are you talking about how we believe that our soul goes to Heaven when we die? You know the part inside of us that thinks and feels and knows we are Gracie or Mommy or Meggie."

"Uh huh. Zoe says it doesn't. I don't ever want to grow up and get old."

"Grace, it takes a long time to grow up. You don't need to worry about getting old and becoming a lady."

"I don't ever want to have a baby and be a lady."

"Gracie, you don't have to do anything you don't want to do when you are an adult, but honey, we all grow up. We can't make our growth stop."

"Who goes to Heaven, Mommy? Does everybody?"

"Not everyone goes to Heaven Grace."

"Why not?"

"You have to be a good person to go to Heaven."

"Am I good Mommy?"

"Yes, Grace, you are very good. You'll go to Heaven when you are an old lady."

Meanwhile, I'm thinking, "Where is this coming from? Why is Gracie so obsessed with death, dying, and Heaven. More importantly, why are we talking about this at nine o'clock at night?"

"Mom, where is Heaven?"

"Uh. It is a place very, very high in the sky up past the clouds where the angels live...where unicorns and pegasus horses live. It is a beautiful place."

"Why do unicorns and pegusus live there? You said they aren't real."

"You're right Grace. They aren't real. Mom is trying to tell you that Heaven is a beautiful place where you'll go when you are a very old lady. (Fingers crossed.) I've never been there so I don't know for sure. We live on a big ball called Earth."

"Mom, IT IS NOT A BALL! It is called Earth and it is a planet."

"Alright Grace! It is shaped like a ball."

"It is a very large. Mom, who told you about Heaven?"

"My mommy and daddy told me about it."

"Who told them?"

"I suppose we all learned it from Jesus."

"How does Jesus know?" Questions were coming out in rapid fire sequence...bam, stuttering mom, bam, more stuttering mom, bam, bam, more bumbling stuttering mom, bam, bam, bam...

"Where is Jesus? Who is he? How do we know him?"

"Grace, you learned about Jesus at your day care, right?"

"Uh huh."

"You tell me about Jesus."

"I don't wanna Mommy."

"Okay."

"Where did we come from Mommy? Who lived before us?"

"The dinosaurs."

"No, Mommy, not the dinosaurs. Which people lived before you and me?"

"Well, let's see. Your Grandma Kathy's mommy is Grandma Lorraine, right?"

"Uh huh."

"Grandma Lorraine's mommy and daddy lived before us, right?"

"Oh."

"Grace, it is time to go to sleep honey."

"Mommy, I want to talk. I want to talk to you."

"Grace, we can talk and figure stuff out together anytime honey. You are yawning, and well, Mommy, needs to get some rest too. I'm turning out the lights now."

"Okay Mommy. Sing me Cici and Chickory-Chick."

Smiling Faces

Visit to GI Clinic

Well, today was a big day for the girls. We went in for the annual liver check-ups. Last year had been a very difficult visit since Grace was quite distressed by the requisite lab work. She attempted to bite the hand of the lab tech last year. I suppose remembering that fact heightened my anxiety too.

I had spent most of the morning preparing the girls for what was about to happen. I described how Dr. B would talk about what had happened in the last year. We also went over and pretended to have Dr. B check our livers. We tap, tap, tapped on each others tummies and pressed down gently to figure out if we could feel our spleens. It was something I had to do. My kids need a perfect amount of prep time, but I have trouble determining what that amount of time is.

Before the kids woke up that morning, I had spent a few minutes trying to figure out how to approach them about the visit. "Should I tell them about the labs? How do I tell them about the labs? Gosh, I don't want Grace to go balistic again. I wonder how Meghan will do this year."

I know that these are my issues, and I must be careful not to project my fears onto my girls. Besides that lingering fear of what will happen to my girls, I find the constant questioning of my approach to Alpha-1 tiring. Most of my friends will tell me I'm a good mom because I question myself, but this affirmation doesn't make those questions stop inside my head. Maybe it is all just normal "mommy" worries, but I feel like the Alpha-1 adds yet another layer of complexity to my life.

The actual visit went quite well. Grace bravely jumped up on the exam table without coaxing. Dr. B examined her, and exclaimed, "I don't feel her spleen, and her liver is not enlarged." I breathed a sigh of relief. It was Meghan's turn next.

In a perfectly opposite reaction to what I had envisioned, Meghan promptly started to cry and refused to cooperate. She squirmed, twisted, whined, and cried through the entire exam which took place on part of my lap as well as Dr. B's lap. Again, Dr. B said that all was well. A weight seemed to be lifted off my shoulders. Or should I say my lap? ha ha

So, the girls' Alpha-1 is so far staying pretty dormant in their livers. That is great news, and I pray this trend continues. Grace is now 3 feet, 9 inches tall, and weighs 46.8 pounds. Meghan is just shy of 3 feet tall, and has made some small gains again. She is up to 26 pounds, 8 ounces. She falls between the 5-10th percentiles for height and weight on the female growth charts.

Both of them had liver labs drawn today. Grace had an extra set of tests run though. We reported that Grace has bloody noses here and there, but has always been a kid who bruises easily. So, Dr B. ordered a clotting time study, which just meant an extra couple of tubes of blood. All of this went right over Grace's head so she is none the wiser. Whew!

I'm pretty confident that each of my girls will have mild elevations in ALT/AST, and Grace's clotting time will come out normal as it did when the test was run when she was 2. Okay. I should probably say that I'm crossing my fingers for those results.

As you can see, I struggle with denial versus reality versus maintaining a normal childhood experience for my girls. Both of them identify themselves as Alphas, but I still don't think they quite "get" what Alpha-1 is yet. I wonder how much longer I can help maintain that blissful ignorance. I wonder if my girls will appreciate how difficult the one-at-a-time decisions were when they get older. Only time will tell, but I wonder. Who wouldn't?

Reality Setting In

Tomorrow, my girls go for their liver checks at the GI clinic.

Why does it always send me into an inner panic mode? Well, I can answer that question, but I guess this just an Alpha mom voicing her inner most fears. Please God, please let everything be fine.

Oh and God, please help Gracie with her anxiety over blood draws. Last time, she tried to bite the lab tech, and well, we need some bravery Lord. Please?

A humble Alpha mom,

Jen

Talecris Sues Baxter

The Delware IP Law Blog is reporting "Baxter Loses Bid To Have Patent Declared Invalid for Indefiniteness."

http://www.delawareiplaw.com/index.php?itemid=149

Augmentation therapy is big pharmaceutical business in the Alpha-1 community. I suppose this goes on more than most of us know. As a parent of Alphas, I hope this kind of drama stays far, far away from my girls as they get older.