Norovirus

On December 23rd, I was wrapping gifts in our rec room, and heard "Jen, Meghan just puked." I walked up the basement stairs to hear whimpering from Meghan, the puker. She was covered in it and so was my mom (the pukee) who had tried to help her. Upon entering the living room, I smelled THAT smell. You know. That horrible, awful virus puke smell. I'm sure any day care provider could identify it as the "smell smelled round the world." haha

In any case, this began Megs nonstop episode of consistent vomitting for nearly 8 hours into the wee hours of Christmas Eve. I spent much time cleaning up Meghan in baths, scrubbing soaked clothes, cleaning the carpet, and washing my hands over and over again. I was hoping beyond hope that I wouldn't catch the ick, but in the back of my mind, I kept remembering that I was still on prednisone for my bronchitis. Right on the package, it said something to the effect of this medicine can lower your resistance to infection.

Upon the puking ending, Meghan then started with the worst smelling BMs. She put pig farm smells to shame. Even the flys wouldn't come around her. Poor baby...In any case, our Christmas experience in the B family household was a bit lacking. We were too busy being covered in puke to get into the spirit of the holiday, but we still went through the motions.

Ultimately, we made it to Christmas morning. Charlie and I were a bit blurry eyed due to the normal parent experiences during the wee hours of Christmas morning. About 1/2 hour before the girls woke up, I came down with a nasty migraine headache. Merry frickin Christmas! Sorry if that offends you but this holiday is going down in the number one spot on my list of worsts. Ooh la la! How wonderful! A blinding, raging headache to go with your Christmas cheer.

After the headache had been dulled a bit, we opened our gifts. The girls were quite pleased with what Santa brought, but Meghan's pallor was ghastly to say it nicely. Plus, she gifted us with some of those lovely scents of ransid, rotting flesh in her diaper.

In any case, Meghan seemed to be past the worst of norovirus, and I was glad that she was a bit better. Well much to my chagrin, Meghan was sitting nicely in my lap helping me to open one of my gifts and suddenly hurled into the very convenient gift bag I was "opening." Thus, commenced round 2 of puke fest 2006. Eeeeeeewwwwwwww!

So, is anyone wondering if I acquired this ick yet?

DA DA DA DA DAAA DAAA DAAAA (say that to the theme of STAR WARS)

I awoke on 12/26 to a fun filled festival of pukiness too. 12/27 was a day of visits to the potty for me too.

So there...there was my holidays wrapped up in a nutshell.

Wasn't this the most pleasant posting I've ever written?

Yours in norovirus recovery,

Jen

PS: Somehow, Charlie and Grace have evaded the fun. I hope that doesn't change. I've already "boiled" myself and everything around me to minimize germs. :)

Boston Marathon

Today, I found out that a runner in the Boston Marathon will be running in honor of Grace & Meghan. Her name is Jen, too.

In any case, I'm so excited about this. It just means so much to me that someone else is thinking of my girls. We are so blessed for their current state of good health, and I'm honored that the girls were matched up with Jen.

She is part of a team made up of about 230 runners, who last year raised over $1.1 million for the American Liver Foundation. The team goal this year is $1.2 million. Over the past 3 years, Jen has personally have raised over $20,000. Her goal this year is $8,500.

Isn't that awesome!

The marathon is on Grace's 5th birthday, and we'll be routing for Jen all the way...from Wisconsin. :)

Ho, ho, ho…I’m sick

Last week, I caught one of the nastiest colds I’ve had in a very long time. My nose was rather faucet-like. ha ha I think I caught the bug that Meghan had to fend off with antiobiotics recently.

Today, I took myself to the doctor because my left lung hurt. I’ve never had lungs hurt before. I also have a very nasty “chunky” cough with lots of Christmas green color “things” to use a technical term. I guess you could say that my Alpha-1 radar was up.

Being that I’m a gene carrier (MZ) for Alpha-1, I know that I have less Alpha-1 circulating in my blood stream to fend off this particular nasty infection. I do admit that I say that with a little hesitation, since I don’t want to state my case as being horrible when compared to the many individuals who live with ZZ Alpha-1 on a daily basis. ZZ Alphas certainly deal with much greater consequences of repeat lung infections. In any case, I thought it wise to protect my lungs by telling the doc about my recent illness.

Well, my dear old doc informed me that I’m wheezing pretty badly especially on my left side. So, guess what? I get to take prednisone, a steroid to reduce the inflammation in my lungs, along with an antibiotic for 10 days.

Fun, fun, fun

Cough, cough, cough

Ho, ho, ho

I hope to be better by Christmas.

NICU Reunion

Cross posting an entry I wrote on Preeclampsia Survivors:

http://preeclampsiasurvivors.blogspot.com/2006/12/nicu-reunion.html

Jen

Always See the Bright Side

Today while I was dropping Meghan off at her day care, Grace looked at the back of our filthy mini-van and said, "Mommy, why is there a rainbow on our car?"

I paused for a moment and thought, "Huh? Rainbow?"

As I examined the back of our van, I noticed the perfect shape of a rainbow on our back window. It was the only clean spot on the back of the van...exactly where I had turned on the window wiper.

This made me laugh since I was being the jaded, scruntinizing adult that I am. Gracie simply saw something pretty in the middle of something so dirty. All I saw was the filthy, gray "guck" on the back of my van.

I need to act more like a 4-year old from now on.

Jenni Richters

My sister reminded me of how my baby brother, Timmy, used to call one of my high school friends Jenny Richters. Her name was then Jenni Richter. It made me laugh since I accessed a very archived memory. I had almost forgotten that.

Jen, are you out there? Tim is now almost 22. Come out, come out whereever you are.

"What? Drop your face?"

"Look at all the snow men!"

Missing you my friend...

Jen :)

Bronchitis

Well, I took Meghan into the doctor today. She has had a cough that got gradually worse, and thus worried Alpha mom Jen. Meghan's cough reminded me of someone who had been a smoker for years. Ick! She is now on Zithromax and hopefully, it should do the trick.

So what I just described probably sounds like normal "mommy" stuff, right? You are right except...

Every time Meghan gets a lung infection, it means she loses healthy lung tissue, too. Since she is a ZZ Alpha, her body is not able to produce enough Alpha-1 in her liver to be sent to the lungs to turn off neutrophil elastase. Those neutrophils are like minature pac-men who come in and eat up the bad stuff hanging out in Meghan's lungs. Unfortunately, Meggie doesn't have enough Ms. Pac-Man (aka Alpha-1 proteins) to nag, nag, nag, nag the heck out of Mr. Pac-Man (aka neutrophils) until he finally backs down and stops destroying healthy lung tissue.

Fortunately, my mommy instincts kicked in about Meg's cough today, and we caught her bronchitis in an early stage. I'm grateful for that, but I can't quite turn off that obsessive thought of how much lung tissue will be destroyed with this infection. These are my inner most thoughts, and well, are now shared with you all in cyberspace. Getting my thoughts out into a narrative is what helps me to feel better.

In any case, Megs was so cute at the doctor. She actually cooperated since I was able to tell her what the doctor would do before he came into the room. When he walked into the room, she told him, "Just listen, right?" Funny...

"I has medcines. Make me fee betta. I go docta's room."

She is growing up so fast now. I can't believe how much she is talking and figuring things out now.

Juxtaposition

Today, I was late getting the girls up to get ready for school and was still at home about 20 minutes after I should have been. While ironing a very wrinkled set of pants for myself around five after eight, I heard a very loud "metalic" crashing sound almost as if something had fallen over.

I thought, "What the heck was that?"

I peaked out my window to look into our alley, but didn't see anything. I figured it must have been someone getting one of those huge metal dumpsters delivered, but then I thought, hey everyone has snow on their roofs so it can't be a roofing dumpster...strange. In any case, I quickly put the strange loud noise out of my mind.

Then, I proceeded to go about scurrying the kids off to school. When I arrived at work, my hubby had left me a message telling me to get online to see the huge explosion that had happened about 8 miles from my house.

The Falk Corporation complex here in Milwaukee which does metal forging, etc...literally blew up today. Two full city blocks are gone in the valley near Lake Michigan...when I saw the coverage on CNN.com, it looked like 9/11 again...they think it was just an accident with a gigantic propane tank. Thankfully, it sounds like an evacuation was in progress because of the smell of gas. It could have been much worse, but horribly, three people died and another 50 or so people were injured some critically.

It was upsetting to me that as I was chastizing my kids for being pokey, people were dying or literally running for their lives. I so very much feel for the families of those men who died...they all had young children and wives. Certainly, this makes me feel grateful for all of the blessings in my life, and I'll be praying for all of the victims of this tragedy.

It was very strange juxaposition at least in how I experienced it.

Please pray for the affected employees and families.

All I Want for Christmas...

As I've gotten older, I've noticed that I'm less inclined to "want" for physical items. Our family is already so blessed with a home, cars, food to eat, clothes to wear, toys to play with, and other extravagences. So, this year for Christmas, I'd rather have my family and friends donate to some very worthy causes, which are near and dear to our hearts, rather than receive gifts.

The Alpha-1 Foundation is dedicated to providing the leadership and resources that will result in increased research, improved health, worldwide detection, and a cure for Alpha-1 Antitrypsin Deficiency. Since both Grace & Meghan have ZZ Alpha-1, we'd love it if you could help fund research for Alpha-1. 100% of what you donate goes to research. The Alpha-1 Foundation has other ways of generating the money to run the business side of the foundation.

Donate at: http://www.alphaone.org/help/commemorative-giving

The Make a Wish Foundation grants the wishes of children with life-threatening medical conditions to enrich the human experience with hope, strength and joy. Several of our liver friends have been granted wishes and their experiences were invaluable. Please share in helping critically ill children find some joy in their lives.

Donate at: http://www.wish.org/help/donate

The Curative Foundation, which runs our Birth to Three Program provides pediatric outpatient therapy services and special education to children ages birth to 3 years in Milwaukee County who have a developmental delay. Our little peanut girl, Meggie, has received speech, occupational, and physical therapy from this organization for the last 2 years. She is thriving because they've shown us how to help Meghan on a day-to-day basis so that she can overcome her former micropreemie beginning.

Donate at: https://www.curative.org/form/gift_form.htm

The March of Dimes' mission is to improve the health of babies by preventing birth defects, premature birth, and infant mortality. Both of our daughters were born premature due to my preeclampsia. Both of them suffer the side effects of being born preemie. Yes, they are doing well, but they both wear the "badge" of prematurity still. Please give generously.

Donate at: https://www.marchofdimes.com/howtohelp/donation_in_honor.asp

Have a very magical holiday season! Thank you for considering these organizations.

Jen

I'm Sad...

Today, I dropped Grace off at her before school day care program. When I was walking down the hallway out of the school, Shanna stopped me. She said she wanted to tell me something that Grace said to her the day before when they were having some bonding time.

Me: "What did Grace say?"
Shanna": "I'm sad I was born with Alpha-1."
(Hmmmm...but I remained calm. Guess mom Jen was having a good day emotionally.)
Me: "What were you talking about?
Shanna: "I think it was about painting finger nails."

Tonight before I put Grace to bed, I asked her about why she was sad she had Alpha-1.

Grace: "Because I can't paint my finger nails."
Me: "Grace, you can have painted finger nails sometimes. We just have to be careful because of fumes. We can paint your nails in the back yard where the wind can take the fumes away."
Grace: "What are fumes?"
Me: "They are like stinky smells that hurt our lungs."
Grace: "Oh."
Me: "What color nail polish do you want Gracie?"
Grace: "Blue with glitter like Aisha from school."
Me: "How about pink? Or purple?"
Grace: "I like red and green."
Me: "Just because you have Alpha-1 doesn't mean that you can't have painted nails Gracie. We just can't do it too much. It is more important not to smoke cigarettes or drink alcohol."
Grace: "Ok. What is alcohol?"
(Oh boy...)
Me: "A drink that makes people act silly but it hurts their livers. We don't drink alcohol because we are Alphas, right?"
Grace: "Right."
Me: "We'll paint our nails some pretty colors. It will be fun."

Grace gave me a hug. Her Alpha-1 realizations are coming fast and strong now, but I'm proud she talks about it with people she loves. Way to grow Gracie!

Angiotensinogen Thr235 Mutation

Have always been interested in why the heck I got preeclampsia, but when I developed chronic hypertension after the birth of Meghan, things started to make sense. Hypertension runs in my family, and the medication that I take is an ACE inhibitor, which is an antiangiotensingen controlling enzyme (ACE). This study abtract makes me wonder...I never had a placental abruption though. I suppose my mom and Aunt Susan might like to read this. Let me know if you do Mom or Susan.

1: Placenta. 2006 Nov 17; [Epub ahead of print]

Placental Abruption Is More Frequent in Women with the Angiotensinogen Thr235 Mutation.

Zhang XQ, Craven C, Nelson L, Varner MW, Ward KJ.
Department of Obstetrics and Gynecology and Reproductive Genetics, University of Utah, School of Medicine, 50 N Medical Drive, Wintrobe Building Room 657, Salt Lake City, UT 84132, USA.

OBJECTIVE: Obstetrical complications such as preeclampsia, fetal growth restriction, and placental abruption are associated with inadequate placental perfusion. Previous studies have shown that the angiotensinogen (AGT) Thr235 mutation is associated with abnormal remodeling of the uterine spiral arteries and occurs at higher frequencies in preeclampsia. This study was done to evaluate whether the AGT Thr235 mutation increases the risk of placental abruption. MATERIALS AND METHODS: We compared 62 placentas from women who had placental abruption with 240 control patients of similar age and ethnicity. DNA was extracted from paraffin blocks from placentas. AGT Met235Thr mutation status was determined by single fluoresceine labeled probe real-time PCR using a LightCycler system. RESULT: AGT genotypes were divided into three groups: MM (homozygous wild), TT (homozygous mutant), and MT (heterozygous). The constituent ratio of AGT genotype in abrupted placentas (MM 14.5%, MT 43.5%, TT 41.9%) was significantly different from in control group (MM42.5%, MT 39.6%, TT 17.9%) (p<0.001). AGT mutant allele frequency in placental abruption (0.637) was significantly higher than in the control group (0.377) (p<0.001). CONCLUSION: The AGT Thr235 mutation was observed more frequently in placental abruption. AGT Thr235 mutation may be considered a risk factor for placental abruption.

PMID: 17116328 [PubMed - as supplied by publisher]

Promising Alpha-1 Gene Therapy

I heard about this research last February at the Alpha-1 Foundation's Research Update. While this is very promising because it did no harm to study subjects, there is much to be done before this can actually be used as an effective therapy. I still applaud this research, and encourage you to support the Alpha-1 Foundation (http://www.alphaone.org) in its quest to fund a cure for Alpha-1. WOOHOO! :) Jen

Gene therapy shows promise against hereditary lung disease

Tuesday, November 21, 2006. GAINESVILLE, Fla. — An experimental gene therapy to combat alpha-1 antitrypsin deficiency, a common hereditary disorder that causes lung and liver disease, has caused no harmful effects in patients and shows signs of being effective, University of Florida researchers say.

In a clinical trial, researchers evaluated the safety of using a so-called gene vector — in this case an adeno-associated virus — to deliver a corrective gene to 12 patients who are unable to produce a protein essential for health called alpha-1 antitrypsin.

“The primary end point in the trial was to see whether it was safe to give patients this gene transfer vector and then to try to begin to see if we could get the dose into a range where we would begin to replace the missing protein in the blood,” said Dr. Terence Flotte, a pediatrician, geneticist and microbiologist with UF’s College of Medicine and a member of the Powell Gene Therapy Center and the UF Genetics Institute. “We found that we can use this agent safely and we also saw evidence in the patients’ blood that the higher doses successfully introduced the vector DNA. In one patient we saw evidence for a very brief period that some of the alpha-1 protein was being produced, but not at a high enough level to be beneficial.”

The findings appeared online today (Nov. 21) in the journal Human Gene Therapy.

Physicians injected doses of the virus containing copies of the gene for alpha-1 antitrypsin into the patients’ upper arms. Essentially, the virus is intended to “infect” patients’ cells with replacement genes that will do the necessary work to produce alpha-1 protein. UF scientists have successfully developed the technique in animal models.

The next step is to test the therapy with a different version of the adeno-associated virus; about 200 variations of the virus exist in nature.

“We have another version of the virus that appears in animal studies to be close to a thousandfold more potent at making protein,” Flotte said. “That’s very encouraging to us. So the next trial, which has already begun, is to use the new version of the virus and take patients through a similar range of doses, in a very similar scheme, and see if we can maintain the safety while pumping up the efficiency of the protein production.”

In most people, alpha-1 antitrypsin is made in the liver and protects the lungs by counteracting inflammatory products that destroy lung tissue. But about 100,000 Americans have alpha-1 antitrypsin deficiency, according to the Miami-based Alpha-1 Foundation, a national not-for-profit organization devoted to finding a cure. In addition, medical authorities suspect less than 5 percent of affected individuals are diagnosed, often not until they are in their mid- to late-30s, after extensive lung damage occurs. Shortness of breath, wheezing, chronic cough and recurring chest colds are signs of the disease.

It is important that alpha-1 patients avoid cigarette smoke, said Dr. Mark Brantly, a professor of medicine and molecular genetics and microbiology at UF’s College of Medicine who develops clinical research programs aimed at developing therapies for alpha-1 patients. Alpha-1 deficiency can in some patients lead to emphysema and cirrhosis, both progressive diseases that can be fatal.

Alpha-1 patients with symptoms of emphysema can be treated through weekly intravenous injections of alpha-1 protein derived from human plasma. The injections must continue throughout a patient’s life, according to the American Lung Association. It does not cure, but it does appear to slow the progression of this disease.

Patients in the clinical trial – 10 men and two women who ranged from 42 to 69 — were asked to discontinue their replacement therapy 28 days before receiving the gene therapy.

One volunteer who had not been on protein replacement therapy exhibited low-level expression of alpha-1 antitrypsin, which was detectable 30 days after receiving an injection. However, residual levels of alpha-1 antitrypsin from the replacement therapy in the other patients obscured whether the alpha-1 gene had begun to express protein.

“As the authors conclude, the results set up the more interesting approach of using other AAV serotypes more suited for muscle delivery as an alternative with the same transgene in the next trial,” said Richard J. Samulski, a professor of pharmacology and director of the University of North Carolina’s Gene Therapy Center. “These studies are important milestones that allow the potential for gene correction of AAT to advance, as well as the (gene therapy) field in general. They also represent the step-by-step process established by the FDA and research community to ensure that safe and good clinical studies are employed in these early days, and I applaud Terry Flotte and his group for being cautious and thorough in their clinical design.”

The trial is funded by a National Institutes of Health grant, and the Alpha-1 Foundation played a crucial role in helping to build the infrastructure to support the research, Flotte said. UF holds an equity interest in Applied Genetic Technologies Corp., a company formed by UF researchers to develop gene therapies.

Miracles Aren't Free

Cross posting my entry on Preeclampsia Survivors:

http://preeclampsiasurvivors.blogspot.com/2006/11/miracles-arent-free.html

Long Live the Alphas

Yesterday, I attended another Alpha-1 education day in Chicago. I always look forward to attending the days. I think that is because I keep hoping that one of these events will reveal that latest advance, the "ah ha" moment for some doctor who might be there, or possibly that glimer of hope for my beautiful Alpha daughters.

As is usually the case, I was sorely disappointed.

I know that I'm really quite lucky to have Alpha girls who are doing well. They are growing and learning about all the subtle nuances in life. I get to love, cherish, and hold them every day, and I always try to remember that there are parents of children with Alpha-1 who can't do that. Their precious children earned angel wings.

So some people might say that I'm torturing myself by attending these events. Some people might say that I'm wasting my time. Sometimes I wonder the same thing, but what those nay sayers don't know is what keeps me coming back.

Everyone in that room understands how important it is to find a cure for Alpha-1. Everyone knows how it feels. Everyone has felt the guilt of Alpha-1...the guilt of placing a burden on loved ones...the guilt of passing on a life threatening gene...the guilt of being less than perfect.

What makes these education days perfect is that we are all less than perfect in the room. We are all part of the Alpha-1 community. We all know how it feels. We all know that a cure must be found. My babies lives depend on it.

Long live the Alphas! Nay sayers be damned!

November is Prematurity Awareness Month

A little more than 4 years ago, I learned that my baby would be born too soon at 34 weeks gestation. My precious cargo, Grace Ann, had to be born early because of my preeclampsia. She was my biggest baby weighing in at 3 pounds, 14.5 ounces, and 17 1/2 inches long. She had difficulty breathing, eating, and growing. To this day, Grace still deals with issues related to her prematurity, but nonetheless is doing well overall in kindergarten. Can she really be 4 already? Wow! I'm eternally grateful for Grace and the wonderful neonatal care she received.

A little more than 2 years ago, I learned that my second baby would be born extremely too early at 27 weeks gestation. My tiny peanut, Meghan Rose, was born because I developed preeclampisa yet again. She was unbelievably small to me weighing in at just 725 grams, aka 1 pound, 9.5 ounces, and 13 inches long. She was called a micropreemie, and proved herself to be a fighter every scary step of the way. Today, she still contends with issues related to her prematurity, too. We're happy she is growing, albeit ever so slowly, but she is happy and quite the character. :)

Every day 1 in 8 babies born in the U.S. arrives too soon. Premature birth can happen to any pregnant woman. It is a serious, common, and costly problem. The March of Dimes is leading the campaign to reduce premature birth by supporting research and by educating the public and health care providers.

In tribute to the miracle baby survivors of preeclampsia, please consider donating to the March of Dimes. I would be so proud if you could. Grace & Meghan thank you too.

Jen, mom of Grace & Meghan

Already?

When I came home from work today, Gracie bolted up our basement stairs from the play room and squeezed her arms around my waist. I hugged her back, and said, "Did you have a good day?" She stuck her bottom lip out and said sighing, "No." Meghan then interupted by saying with delight, "Mommy home!" Grace disappeared behind me.

As I took my coat off, I walked around calling for Grace. She answered me with "I'm in here in the living room."

"Where?"
"In here!"
"Where?"
"In the corner by the table!"
"Why?"
"I just like it here, Mom."
"Why? What are you doing?"
"Nothing. I just like it here."

(I'm now thinking...what is up with her? In fact, I thought that she might be pickin' her nose or something, but as is usually the case for me, I was distracted by many things...the mail to be opened, my hubby telling me various things, wondering about the election results, noticing that it looks like a bomb went off in my house, and remembering that Charie and I had German class tonight...dang it, I forgot!)

A little while later, I went up to the girls' room to pick out their clothes for the next day. Grace followed me upstairs. Then, I remembered I should check the school calendar to see if Grace needed to wear her gym shoes tomorrow so I went back downstairs for a quick minute. When I came back into the girls' room, I found quite a mess on the floor that hadn't been there just the minute before. There were books everywhere, a dolly stroller overturned, stuffed animals strewn about, and dirty clothes taken out of the hamper and scattered on the floor.

WTF?

"Grace, what happened?"
(no answer)

"Grace, what happened in here?"
(long pause)..."Meghan did it!"

"No, Meghan is downstairs with Daddy. What happpened in here?"
(no answer again)

"What is going on Grace?"
(Grace covers her face.)

"Are you angry?"
(no answer)

"Grace, if you don't answer, you'll get a timeout! Are you mad?"
(I suddenly see the ring of chocolate residue around her mouth and remember her hiding in the corner...my aha moment comes regarding the hiding, but I decide to talk to her about stealing from her candy stash without permission later.)

"You can tell me anything Grace. I won't yell."

"Yes, you will. You yell at me a lot." (Ut oh, busted!)

"No, I don't, but honey, what is wrong?"

"The girls were mean to me at school. I'm sad."

(I'm thinking...oh crap! I have no idea how to handle this yet. C'mon! Why do little girls have to be so dang mean to one another? What should I say to her? How can I let her know that it is okay to be sad about it, but not okay to throw things around because of it? Also, is this why she stole chocolate? Have I already shown her the errors of my ways in drowning my sorrows in chocolate? Crap! Where is Charlie when I need him? Ah, he's a man. He won't get this. He's never been a girl. Nothing against my hubby, cause I love him a lot, but he doesn't always understand girl "stuff.")

"They didn't want to play with me."
"Who didn't?"
"I don't know their names yet."
"Zoe?" (Zoe is Grace's best gal pal since they were about 4 months old.)
"Uh huh and some other girls."
"Gracie, remember how I told you that kids are still learning how to be good people and adults help children to learn how to be good people?"
"Uh huh."
"Maybe the girls still need to learn how to be nice? Or, maybe they were having a bad day? Sometimes, we have bad days, right?"
"Mom, Zoe said she wasn't having a bad day and told me to go away." (Crushing painful blow to my heart at this point. Why are girls so dang mean?)
"Gracie, you know that you are a good girl, and that sometimes, other people can hurt our feelings or make us sad, but that doesn't mean you aren't a good girl and nice to be around."
"Mom, they wouldn't play with me."
"I'm sorry that made you sad honey." (I gave her big hug.)
"Gracie, when Daddy picks you up from school and you've had a bad day, tell Daddy that you need a hug. Hugs can make you feel better, okay?"
(no answer)
"Now Grace, there are 3 things I need you to know. Number 1, you are a very special girl, and you should always be proud of yourself. Number 2, it is not okay to throw things when you are sad or mad. You can always talk to Mommy or Daddy...always. Number 3, if you feel like you want to eat something, it is not okay to take food without asking. Having chocolate before dinner is not okay with me. Chocolate is a sometimes food. Okay?"
(no answer)
"Let's pick up your mess and go downstairs to eat some dinner."

Gracie's face said it all. Her pain was real, and there was nothing I could do besides kiss her and give her hugs. I couldn't take her pain away though. She had hurt feelings, and damnit, I couldn't make her feel better. She sulked for most of the night about it even when my sister came over to watch the girls, and Gracie loves Lauren. Forgive my language please, but damnit, she is only 4!

Already??????

Damn it!

Cross posting one of my entries on Preeclampsia Survivors:

http://preeclampsiasurvivors.blogspot.com/2006/11/met-some-other-survivors-in-person.html

Jen

A Bright Idea

Today, I helped launch a new blog called Preeclampsia Survivors.

http://preeclampsiasurvivors.blogspot.com

I'm pretty proud of it since I helped conceive the concept and then worked it through to completion. I hope that other preeclampsia survivors will benefit from the blog.

It will grow in time, but I'm happy to help other PE moms feel less alone with their guilt over having had preeclampsia.

Maybe this time next year, we'll have regular bloggers contributing content. We'll see what lies ahead, but it was fun putting together.

Jen

Scientists Use Umbilical Cord Stem Cells to Grow Livers

Scientists Use Stem Cells to Grow Tiny Livers in Lab

British scientists used umbilical-cord stem cells to grow small artificial livers in the laboratory, a breakthrough that could be the first step in being able to grow full-sized livers for human transplants.

These tiny livers -- smaller than a penny -- can already be used for drug testing, reducing the need to conduct such tests on humans and animals, The Times of London reported.

The Newcastle University scientists placed the umbilical-cord stem cells in a bioreactor, a device developed by NASA to mimic the effects of weightlessness. In this gravity-free environment, the stem cells were able to multiple more quickly than normal. The scientists added hormones and chemicals to instruct the stem cells to turn into liver tissue.

"We cannot build a full-sized [liver] yet -- that will take about 10 years -- but this is the first important step," researcher Nico Forraz told The Times. "And we have excellent facilities... to be able to make this happen. We expect this to really take off within the next 18 months or so."

Source: www.healthday.com

A Beautiful Soul

An extremely tiny, beautiful soul entered Heaven on Friday.

Only his mother and father truly knew him. They knew his potential, his unending love, and what could have been his future. Even though I never met him, I feel as if I knew him inside and out. I'm not sure that makes logical sense, but I'll try explaining.

I simply knew of him as Jack.

He was growing inside my friend, Jack's mom. His mommy was so proud to be creating him that she told me when she was only 5 weeks pregnant. It was a secret, but I was absolutely honored to know my friend would become a mother. I was elated for her, and glad to have a new mommy to welcome to the world of parenting.

Even through all day sickness, Jack's mom was a fighter. Her strong and bright spirit helped her to keep her eye on the prize. Having also experienced all day sickness, I could sympathize immensely.

From the moment I first spoke with Jack's mom when I was 17, I saw her spirit was strong and bright. Even though Jack's mom didn't always know she was strong, I knew it. She proved it everyday that she would stay up late sharing her thoughts or just being silly with me. She never judged me or my circumstances. We had the best of times together. These times meant and still mean the world to me.

Unfortunately for Jack's parents, horrible circumstances unfolded. Jack's strong and bright spirit was held in a less than perfect body. He had to leave this world too soon, and leave his parents behind.

I've been praying for Jack and his parents. Even though they don't have him to hug and hold anymore, they will never forget their first born son, who made them parents. Jack's mom will forever be a proud mommy. Welcome to the club, Ames. I only wish you could have had more time with your dear sweet, little boy. I love you!

Oh and one more thing, hey Jack, up there with the angels, you need to know one very important thing about your mommy. She is so very proud of you little man, and she'll miss you forever.

Love,
Jenny-O

Cha, Cha, Cha, Changes...

I just got back from airport hell. Atlanta shut down due to a very bad rain storm yesterday afternoon, and as a result, all of the flights in the entire airport were either cancelled or delayed. Ick! I definitely could have thought of a better way to spend the time.

I was so looking forward to sleeping in my own bed after the awful hotel bed. Well, dear hubby decided to give me a brand new matress for my birthday. First change, upon reentry, that I perceived.

He also decided to put Meghan into her first official "big girl bed" while I was away. Another change that I missed.

Meghan went trick or treating for the very first time tonight while I was in airport hell...another change that I missed.

Some of my oldest friends called me while I was away. They were inquiring about our shared friend who went through the worst experience of her life while I was away. Another change that I missed.

I hate change right now. There is simply too much for me to handle right now. Is it too much to ask that if I go away for 2 days that when I return things are mostly the same? Guess it is, at least today.

Happy Birthday to me. I'm 34 today, and my only plan is to check in with aforementioned old friend to see how she is doing. Please pray for her. Thanks!

Jen

I'm Missing Halloween!

Tomorrow is our neighborhood association's night time trick or treat event. I'm currently in a hotel room in Miami on business, and won't be home until after the girls have hit the candy parade. I'm sad and frustrated about that, but had little choice in the matter. I told Charlie he had better take pictures or else. LOL

On Saturday, I turn 34. I'm not quite sure what that means to me. Since I've been so busy with a crazy project at work, I haven't really had time to think about it. The other day, I realized that I'd be alone for most of the day since Charlie and the girls will be attending a band concert for Kesa in Madison. About a month ago, I chose not to go to the concert because of my birthday, but I didn't realize that I'd be alone. In response to this realization, I cried. Before any of my friends and family fire off an email to me or call me about it, please don't. I'm not looking for an invitation to being distracted from the fact that I won't be with the most important people in my world on my birthday (for part of the day). I just wanted to let out how I feel here in my journal. I find that feelings fester if I don't communicate them, and well folks, there really are much WORSE things than getting some alone time on my birthday. I've gained perspective on the matter, and am most concerned for one of my oldest friends.

She is going through the worst possible events right now, and I can't share what they are. All that I can ask is for you all to pray for my pal. She needs every single one she can get to help her get through grief.

Jen in Miami, FL

Clueless Newbie Blogger Here

Today, I added a site meter to my blog and was feeling pretty proud of myself. That is until I went to republish my blog and accidentally stumbled upon unpublished comments that my visitors have been leaving for me. To those of you who left me comments, thank you so much! You made my day.

Gratuitous family picture:

I'm Tallin'!

This morning, Meghan was standing on our bed and having a good time with Char. Out of the blue, she says: "I'm tallin'!"

At first, I wondered what she meant, but then came my "ah ha" moment.

I said, "Oh! You are standing."

Gotta love the cute things kids say when they are learning to talk.

Pioneering Immunosuppression...woohoo!

http://www.post-gazette.com/pg/06205/708203-114.stm

Pittsburgh, Pa.
Monday, July 24, 2006

Less drug better for transplant survival
Immune system can accept donor tissue
Monday, July 24, 2006

By Anita Srikameswaran, Pittsburgh Post-Gazette

A protocol that allows children who get intestinal transplants to minimize the drugs needed to prevent rejection appears also to improve their survival chances.

The regimen avoids steroids and encourages the immune system to welcome the donor tissue, explained Dr. Rakesh Sindhi, co-director of pediatric transplantation at Children's Hospital.

"There's no question, as we look back at our data, that using less drug has been better for these patients," he said. "Today we'll often send patients home with barely about three to four medicines. It used to be there would be about 10 to 15 of them."

Dr. Sindhi will present findings today and tomorrow from four studies at the World Transplant Congress in Boston.

One of them reviewed 75 children who between 2002 and 2006 had transplants of small bowel either alone or with a liver at Children's. Three years after their operations, 84 percent were still alive.

Before transplant physicians implemented the new protocol, two-year survival rates were about 70 percent, comparable to international figures, Dr. Sindhi noted.

The new approach, which is now routinely used at Children's, is definitely better because patients live longer and need fewer drugs, he said.

It's based on a premise proposed by transplant pioneer Dr. Thomas Starzl, of the University of Pittsburgh, and others that creating the right balance between patient cells and donor cells will favor the development of immunological tolerance.

Near the time of transplantation, patients get a drug called thymoglobulin, which depletes the number of immune system T-cells that could attack the donor organ.

No steroids are given after the surgery, Dr. Sindhi said. "That's our way of starting the drug reduction process right up front."

Steroids can slow growth in children and lead to diabetes and hypertension, among other problems.

A standard dose of an anti-rejection drug called Prograf is administered initially, but after about three months, it is rapidly reduced. In general, the intestinal transplant patients require about half the amount of the drug than had been the norm, and no steroids, the doctor said.

The same approach is being used in the transplantation of other organs at Children's.

Liver transplant recipients might get to the point where they don't need the medicine anymore, or only need it every other day in minimal doses. The liver has a greater capacity to regenerate, so it can recover more easily than the intestine or kidney from mild rejection episodes, Dr. Sindhi said.

"If rejection occurs, we treat it the same as we always have," with steroids and more medicine, he said. But "using this approach, we've noticed that rejections are not any worse than before."

Renee Rosner's daughter Adelynn, now 4, got a liver transplant when she was 9 months old and weighed 13 pounds. The North Huntingdon girl had biliary atresia, in which the ducts that drain bile from the liver are obstructed.

Adelynn had the thymoglobulin protocol during her transplant, but did need steroids because of mild rejection.

"She was the classic steroid baby," her mother said. "She was chubby with the big moon face, which for us, believe it or not, was almost a relief because she had been so skinny."

But Ms. Rosner was glad when the steroids were tapered, and Adelynn no longer needs them. The child used to take a total of 7 milligrams of Prograf each day, and now takes 2 milligrams once a day. That will soon be reduced again, Ms. Rosner said.

"The goal would be the need for no immunosuppressor [drug] at all," she said. " Quite honestly, I would be very happy with every other day."

As the Prograf dose has come down, Adelynn is sleeping better, her mother said. And if she no longer needs the antirejection drug, she should also be able to stop taking other medications that counteract its side effects.

(Anita Srikameswaran can be reached at anitas@post-gazette.com or 412-263-3858. )

Asthma common in alpha-1 antitrypsin deficiency patients

Asthma common in alpha-1 antitrypsin deficiency patients
Aug 02, 2006 - Asthma is common in patients with alpha-1 antitrypsin deficiency (AATD) and is usually associated with chronic obstructive pulmonary disease (COPD), suggesting that patients with the deficiency should undergo allergy evaluation, say US scientists.

AATD contains a wide range of AAT phenotypes that confer varying susceptibility to obstructive lung disease and emphysema. The most common variant for homozygous severe deficiency is protease inhibitor (Pi) Z, giving the PiZZ phenotype.

Seeking to identify the prevalence of asthma and atopy in AATD individuals, Edward Eden, from Columbia University in New York, and colleagues sent a survey to 2418 individuals in the Alpha-1 Foundation Research Registry.

The 757 responses revealed that 44.6% had been diagnosed with asthma, while wheezing was reported by 76%. Common triggers were respiratory infections, activity and exposure to dusts, fumes, and allergens.

Dividing the patients into those with the PiZZ phenotype, those with the less severe PiMZ phenotype, and other phenotypes showed that the prevalence of asthma ranged from 20–25%, with wheezing significantly less common in the PiMZ group than in the others.

Of those with asthma, 83% of PiZZ individuals also had a diagnosis of COPD, compared with just 48% of those in the PiMZ group, the team writes in the journal Respiratory Medicine. Asthma was three times more prevalent as the only reported diagnosis in the PiMZ group than in the PiZZ group.

“Asthma is difficult, if not impossible, to distinguish clinically from COPD in those with established airflow obstruction,” the researchers write.

“Given the overlap between asthma and COPD in this population and the potential impact of asthma on forced expiratory volume in 1 second loss, evaluation of the wheezy patient with AATD should include allergy testing and aggressive treatment to reduce bronchial hyperresponsiveness.”

Respir Med 2006; 100: 1384–1391

Go Badgers!

Laura Needs Prayers Again...Ugh!

From Laura's caringbridge page:

Thursday, September 21, 2006 11:58 AM CDT

Laura's liver is not doing well. Her liver enzymes are very high (ALT and AST in the thousands), her kidneys aren't working as efficiently as we would like. Kidney doc's are working reviewing this and can hopefully stimulate her urine flow.

For reasons we don't know her liver does not appear to be functioning at this time. But also her doc's are hoping this tren will correct itself soon. For the time being her Dr.'s are keeping a very close eye on her and are waiting.

She is comfortable and sedated.

Please once again, say a prayer for the little red-head.

Hooray for Laura!

Laura got her second call for her gift of life this morning. She is currently in surgery receiving her new liver. I'll be praying for the medical staff, Laura, her family, and the donor's family.

http://www.caringbridge.org/mn/laurasliver

Mom Jen

23 Weeker's Blog

Oh my goodness, take a look at this peanut baby. I know I'll be saying prayers for Teeny Tiny Truman.

http://www.tinytruman.blogspot.com/

Jen

GI Visit Today

First of all, happy 6th birthday to my beautiful goddaughter, Chloe! :)

Both my girls visited their GI today. At first, I hesitated to post this since our community seems to be on a roller coaster this week, but as a mom, I'm happy with our visit today. I do want add my prayers and love for all of our liver families.

Even though, we waited nearly 1 1/2 hours, Grace was the most cooperative she has ever been with our GI before. She climbed up on the exam table herself, and giggled her way through the exam of her liver and spleen. She even answered the doctor's questions herself. I was a pretty proud mama and filled in a few details as needed. She really is growing up so quickly.

The GI even had an intern along to learn about our girls since the intern had never seen Alpha-1 patients before. It was quite refreshing to have a newly minted doc do a complete patient history on my kiddos. So, both Grace and Meghan received their exams twice today.

We did have one episode of 4-year old "ness" though. Or, should I say Gracieness? Grace is severely frightened of blood tests and immunizations. She was due for labs so we headed down to the lab. They called her name, and she bolted for the door to the hallway. I had to catch up to her, and pick her up. When got into the room, and she kicked up a fuss. I put her on my lap so that I could help calm her down. Well, 5 minutes later she had whipped up her "meltdown magic" and the blood draw didn't look like it was going to happen. I let her go, and told her we needed a "do-over." I took her to the bathroom, and washed her face. She calmed down a bit so I took her back for the draw. This time, she cried, but was able to sit for the test. She even watched the blood flow into the container. Whew! I was mentally exhausted when it was done, but we got those labs. Hooray!

Meghan had her exam too, and was pretty cooperative as much as a 2 year old can be. No labs for her though. She has been visiting our GI quite a bit due to weight issues. Which leads me to our really great news...

Meghan is now up to 23 pounds, 7 ounces. Our GI actually clapped with those numbers. Grace weighed 40 lbs, 2 ounces. So all-in-all, it was a good visit. Livers appear to be good based on physical assesment alone, but I don't have any lab results yet. I'm expecting the status quo of mild elevations on AST and ALT.

Prayers Needed for Laura

Laura, our Alpha friend from MN, received a new liver at the end of August. Well, the liver is failing, and she has been relisted status 1, which means top priority.

Please pray for her and her family! She needs all the positive energy she can get right now.

Darn Alpha-1!

Just look at this smiling face. Doesn't she have the most beautiful red curly hair?

Thanks for praying!

A Glimer of Realization

A Glimer of Realization

(Authors Note: Alpha-1 Antitrypsin Deficiency is a genetic disorder of the liver, which over time can cause damage to the lungs. Each patient with Alpha-1 takes his/her own journey with Alpha-1, but severe emphysema is often an outcome in early adulthood.)

This morning, I shuffled Grace and Meghan out of the bathroom as I was about to use some hair spary on my perpetually unruly moptop of red and gray hair. She complied, but then turned around and said, "How come you can be around hair spray and me and Meghan can't?"

I paused for a second and thought about what to say but before I tell you what I said, I'd like to try to explain my inner panic. You see Grace has always believed that our entire family has Alpha-1 except for her big sister, Kesa. She knows that Kesa has a different mommy, but until today, she thought we all were Alphas...her mom, her dad, and her sister, Meghan.

If someone might have observed our conversation, he/she might not have understood the gravity of our conversation. We used our typical matter-of-fact tones and silly inclinations to talk about it, but there IT was again: Alpha-1. One of my worst fears coming alive...Grace coming to a realization about what Alpha-1 means.

To her, Alpha-1 is just like her lunch box, or her really cool Dora backpack. It is just a part of her life like putting on her socks before her shoes. Don't misunderstand me though. In Grace's short life, she has already experienced two of our Alpha friends who moved onto Heaven. It was confusing to her that Mr. Gary and Ms. Pat weren't going to be at our "Alpha friends" support group meetings anymore. We talked about how we believe that we'll see them again in Heaven when we are very old. (Hey, maybe if I say it enough? It can come true. Peter Pan is coming to mind right now.)

Since Grace is beginning to put 2 and 2 together now, I guess I'm going to have to explain things further. She seems like she is ready, but I just don't want to be the one who has to end her naivety. She is blissfully unaware, and sometimes, I need and want her to stay that way. Heck, I'd love to still be blissfully unaware. Ain't denial grand?

We finished our one minute conversation this way:

Mommy: "Do you remember how I told you that we are all Alphas in our family?"
Grace: "Uh huh, 'cept Kesa."
Mommy: "We'll honey, Mommy doesn't have the same kind of Alpha-1 that you have. Neither does Daddy. That is why I can be around hair spray, but just a little bit. I still need to take care of my lungs too. Meghan and you can't be around it since we need to be really careful with your lungs. Don't worry though. We'll take care of you."
Grace: "Okay, mommy. Meghan lets get away from the "icky" hair spray!"

I closed the bathroom door and opened the window for some fresh air. Having not taken a deep breath during our chat, I needed the air.

Mom Jen

Ah, I don't feel so alone anymore...

Moreena

Since Wednesday's meltdown, Grace has had so many more that I can't count them. A really big one happened at a birthday party yesterday. Of course, Grace decided that she was "queen" of the party and that the actual birthday girl wasn't even a runner up. I really wanted to just curl up and die right then and there. I took her outside for a half an hour to watch her spit, kick, and whirl up her meltdown "magic." Yuck!

Jen

An Alpha's First Day of School

Tuesday, September 5th was Grace's first day of kindergarten at our local German immersion school. It was mostly an orientation day where she could explore the classroom and the parents could ask questions. I attended with her. She did pretty well, but became bored quickly since the kids were not allowed to play with the toys that day. She stuck very close to me, but did seem to like talking to her new teacher, Frau Savignac.

The next day, 9/6, was her first official day in kindergarten. When Grace woke up at 5 AM that day, I thought it might be hard on her. As the morning went on, she kept telling me that she didn't want to go to school. She didn't want to "be a big girl."

Anyway, I took her to the before school day care program called Learning Links. Coincidentally, the day care is run by Kristen, who we met in the NICU more than 2 years ago when Meghan was born. Kristen's son, Tanner, was born at 27 weeks (like Meghan) due to preeclampsia (like Meghan again). I feel really good about Grace being in Kristen's day care. When you're in NICU, you really learn a lot about the other parents there. I know Kristen is a caring parent.

I signed Grace into the day care, and we ran into Tante Shanna right away. Grace raced over to see her, and give a hug. Then, Kristen and I walked Grace down to the kindergarten area of the day care, which is in the gym. Gracie immediately withdrew from me upon entering the gym, and tried to leave. All the while, she is sticking her index finger in her mouth with quite a worried look on her face. (This, of course, immediately created a very large lump in my throat, and I had to remind myself not to cry, too.) I took her into the hall for a do-over, told her to get a drink of water, and informed her that this was not acceptable behavior since she was a big girl and I knew she could do it. I eventually coaxed her inside, and then was able to show her around a bit.

I introduced her to the teachers, Miss Rachel and Miss Shawna, who both seemed quite nice and willing to help. Grace began clinging to my leg so I suggested we sit down at the table and look at a book. We sat, and then we met another little K4 kiddo named Alexandria, who was quite shy.

Anyway, I stayed about 15 minutes total, but when I told Grace that I had to leave for work, it started...the scream heard 'round the world. Well, maybe I'm exagerating a bit, but I can testify that the entire school could hear her. "No, no, no, no, no, noooooooooooooooooooooooooo!!!!" She then got up, clung to my leg, and actual tears began to fall from the corners of MY eyes. The teachers came over to help, and I told them that Grace is very strong and fast...as in, they would need to hold her, but I never said the words.

I quickly turned around, and left while trying to remain composed. I met Shanna in the hallway again. She knew I was about to lose it, and as ever, Shanna, my very good friend, helped me to be distracted somewhat.

I wasn't crying because she was in kindergarten. It upsets me to the core that Grace has NEVER been able to soothe herself in any situation. From the time we brought her home from NICU, Grace's emotions have ruled her. She really has no control over them at all. I makes me sad to know that Grace was so upset. It was really a traumatic experience for me to see her pushed beyond her limits. I cried all the way to work, and it was of no comfort when most people I saw that day thought it was about her growing up and going to kindergarten. Nope, I know Grace is ready for a learning challenge. I'm afraid that my precious daughter is never going to grow past her emotional reactions. She will have a difficult existence if this doesn't change. I don't know how to help her any more than I already do. I let her talk it out, and give lots of hugs, but really, I'm not doing much more than that. I suppose that is all I can do for her.

Tomorrow, I'm taking her back to Learning Links in the morning. We've been talking about how crying is okay, but screaming like "we're being hurt" is not okay at school. I'm saying a prayer that tomorrow will be a better day.

Really My Life???

Ever have one of those days where you feel like you are looking "in" at your life. I picked up our local Alpha-1 support group's new 2007 calendar, and was admiring an absolutely gorgeous picture of Grace. I was looking at her with the eyes that only a mother has. To me, it seems she has a glow around her. It is like her life force is bigger than the boundaries of her body.

http://www.spiderspun.net/wi2007cal.htm

All of a sudden, it hit me.

Grace is in the calendar because she has Alpha-1. She has a life threatening genetic disorder, and no matter how much I love her and take care of her needs, I can't take Alpha-1 away. It hurts me to the bone sometimes. I'm sure you are wondering where the hell I've been.

I've been with Grace all along. I don't see Alpha-1 in her. She is my daughter first. Her ability to make my heart swell with love is immense. I think it is so immense that I can actually disconnect myself from her Alpha-1. Yes, my brain knows it is reality, but my heart, a mother's heart, doesn't accept it. It can be summed up in one word: denial.

In case you are wondering, I can do this with my Meghan, too. Her former micropreemie status distracts me enough to keep the nasty A word at bay.

So, I guess I got a face full of reality again. I suppose I'll go back to rebuilding that denial again. It is the only thing that gets me through most days.

Tomorrow is Grace's first day of kindergarten. I get to describe Grace's Alpha-1 to yet another adult who is about to influence Grace's life. I remember my kindergarten teacher like it was yesterday.

All Pink and Beautiful

DeAnna is doing well post liver transplant. She is so beautiful with such beautiful white skin and eyes. What a beauty. Her mom and dad are just beaming right now.

Still Sick and Tired

Well, I did get my test results back from the doctor about a 1 1/2 weeks ago. He said I don't have pnuemonia, but I do have 68% reactive lymphocytes on my blood work. This indicates I have an infection of some kind. My 2nd mono test came back negative again.

Doc asked if I was still having fevers. Yes! "Are you still taking Advil?" Yes! "Oh." Finally, he told me that he "thinks" I caught Charlie's virus which gave him mono, but for some reason, I'm not converting to mono, which is some sort of atypical cell in the blood work. The doc said that he would call this "viral syndrome," whatever that is. So I asked him what the plan of action would be. He told me that I should come back in 2 weeks if I'm not feeling better.

Still not feeling better! I don't have the fevers anymore, but I still have aches and pains all over with a large headache. I'm still taking 6 advil a day, and well, my stomach does not thank me for that right now. Eeewww...

Tomorrow the 2 week time frame is up, and well, there is no way I can get into to see him before we leave on our annual expedition to visit Charlie's grandma and my grandma in North Dakota. Advil does make me physically feel better, but I'm still so very tired. I'm booked all day long in meetings at work, and well, there is no getting out of them.

A new symptom I have now is that my lymph glands are all swollen and hard and actually hurt to touch. Maybe this time I would test postive for mono? Oh, who am I kidding? That would be too easy.

On a positive note, I'm so very excited to see my dear Grandma Eve who is living in her own home at 87 and still drives her car (only in town). I hope this won't be my last visit with her though. I guess I've always got that in the back of my mind. I still miss Grandpa Neil so much when we go there. It is so strange to walk in and not have him greet me with "Well, hello there honey! Come and give us a kiss! There's my Jen-eh. (in his Kentucky drawl) And of course, I couldn't leave out my dear Grandpa Gene on my mom's side. He'd always greet me by saying "Hi ya darlin'!"

I'm going to be so careful with not getting my Grandma sick. I don't want my virus to send her to the hospital. That would be horrible.

Happy Birthday to Lauren

My the resemblance of my siblings is uncanny...at least that is what other people say to us all the time.

L to R: Tim, Jen, Matt, Kristen, and Lauren

Sick and Tired of Feeling Sick and Tired

Warning: rant ahead!

I've had a fever for 3 straight weeks. It started out as a super high 103.5 fever and then came down to 99.5-100 range. I had the high fever for 4 days, and then another 3 days of low grade. Off to the doctor I went, and he tells me it probably a virus and did bloodwork to make sure that I didn't catch my hubby's Mono. Turned out I didn't have mono. We assumed I'd just get better. That was the first week.

Then the second week, I developed a viral rash all over my body, which looked bad but didn't itch and wasn't raised. Still had the low grade fever with body aches, headaches, and general malaise. Called the doctor's office and his stupid MA tells me that "We don't do rashes. You'll need to see a dermatologist." All the while, I'm thinking that this rash is part of my illness, and I even tell her that. Still she sends me to the derm, and won't let me talk to my doctor. I'm pretty sure she thought I had prickly heat since we've been above 95 degrees for more than a week. I told her that I didn't have prickly heat, but she wanted to be a pain in the you know what. So, I go to the stupid derm, and the derm freaks out like a 14 year old high schooler at seeing how many moles/freckles I have on me. (I'm a red head!) After I put her in her place, I reminded her that I was there for the rash! UGH
She tells me that the rash is viral exanthem most likely caused by Fifths Disease. We end the 3 minute conversation, and she tells me it will go away on its own when the virus finally leaves my system. Meanwhile, I'm frustrated and still sick with a fever and now a rash and no def. diagnosis.

Well, here we are at week 3 of still having a fever and feeling like crap. Rash is now gone. I went to the doctor again yesterday, and well, now he is stumped. He isn't sure what is going on with me now. He ordered a chest x-ray and additional bloodwork, and I'm still waiting on those results. Doc thanked me for coming back. He was clueless that the MA had sent me to the derm for the rash. I guess the derm hadn't sent her report to him yet. So now the doctor is wondering if I have viral pneumonia, hyperthyroidism, or possibly a virus attacking my heart. While in the office, my resting heart rate was 120!!!!! WTF??????

All I know is that I've been eating 6 Advil a day for 3 straight weeks so I don't I feel like crap 100% of my days. I'm feeling very frustrated, and tired of what feels like the run around. All I want are some answers.

The one good thing is that DH took the kids up to our family cabin on the lake for the week. I'm alone, but at least I can be sick in peace. I'm longing for a good hug from my hubby, though.

DeAnna Got the Transplant Call

In March, I was contacted by a man on my caringbridge website for my daughters. He was a grandpa to a little girl named DeAnna who had Alpha-1 liver disease. Well, eventually, DeAnna's mom contacted me, and we were able to meet at our local Alpha-1 support group meeting.

DeAnna was so tiny and so very jaundiced. It made my heart feel heavy when I'd look at her since her color reminded me of the gravity of the situation. She was still a baby though...happy, fussy, giggling, and alert...very cute. Overall, she was a doll!

Anyway, today, DeAnna's mom, Sarah, called to tell us that DeAnna has been called for her liver transplant. They are at the hospital doing presurgical tests as I write this. I called Sarah and heard DeAnna making baby noises in the background.

I hope and pray that I get to hear those baby noises again. I'm sad that another family's devastating loss is possibly creating immense joy for DeAnna's family.

Be an organ donor! It saves lives.

I'll be praying that DeAnna's post transplant journey goes smoothly.

Jen

Meghan Home 2 Years

On Sunday, we will have had Meghan at home with us for 2 full years. Wow, the time has flown by, but we are still dealing with former micropreemie issues such as PT, weight gain, and sensory issues. Anyway, I'm so proud of my peanut girl and how far she really has come. Here is my journal entry from 2 years ago. Just thought I'd share... JEN

On Friday July 23rd, Meghan was down 10 grams to reach 2090, which is 4 pounds, 9.75 ounces. They also measured her height, which was 17 3/4 inches long. Those were her official discharge statistics. Yay! Meghan is home with us, but before I cover that subject, I'll cover a little of the discharge process...

Charlie and I raced over to the hospital after lunch to meet with the monitor company. It was pretty much just a review for us since it was the same monitor that Grace used just 2 short years ago. After that, Charlie and I hung out with Meg's daytime primary nurse, Debbie Cieslak. She filled us in on specific recommendations that Doctor Ragatz had made. After that, we attended infant CPR class. It was just Charlie and me in the class so that was nice. Finally, after CPR, we met with the dietician who had been monitoring Meg's food intake since birth. She sent Meghan home on breast milk fortified with 22-cal Neosure formula. We learned how to mix the milk and powdered formula.

After that, it was past 3:30pm so Meg's other primary nurse, Kate Kirchner, joined us to do the rest of the discharge. Both Charlie and I hugged nurse Debbie goodbye when her shift ended. Doctor Ragatz came by shortly after that, and hugged me goodbye, too. That meant a lot. Nurse Kate looked Meghan over one more time, before handing her to me. We put her in the car seat and bundled rolled up blankets all around her. It was a proud moment for all of us. I can't tell you how long we've waited for this...but really I can, it was 79 days! Anyway, we quietly left the NICU with nurse Kate escorting us to the parking lot. She wished us well, and we took our peanut girl home. Grandma Kathy and Grandpa John watched Gracie while we were at the hospital. Gracie met Meghan on the sidewalk and promptly asked to hold her. It was so cute!

We all settled in at home, and prepared for a restless night. Meghan was a bit disoriented by her new non-hospital surroundings, but did well overall. She woke us 2 times that night. Boy! I forgot what it feels like to be "that" tired. She is up every 3-4 hours to eat, and she lets you know when she is hungry.

To wrap this all up...

Meghan came a long way from 715 grams at her lowest point, shortly after birth, to reach 2090 grams. She fought a long, hard, uphill battle, and we are so thankful to have her in our lives. Not all babies make it out of NICU, and I thank God every time I look at her. She truly is a living, breathing miracle, and I will never forget the staff at St. J*****'s for helping our family through the process. We are blessed to have such a well-reknowned and honored NICU just 5 minutes from our home.

Finally, I must thank each and every one of you who prayed for us, helped us, or just plain cared about what was happening to our family. Words cannot express our gratitude and forever loyalty to you all. We only hope that we can someday, somehow repay you all. It meant the world to us. When tragedy strikes, you really find out how many people care for and love you. Your collective voices and prayers worked to help Meghan fight her battle to live a long and healthy life.

So with all of that said, I want you to know that I will periodically update this web site to keep you all informed of her continued progress. Eventually, I'll be printing this journal out and commemorating it for Meghan to read when she is older.

Thanks again for all of your caring and support.

Proud Mom Jen, Dad Charlie, Big Sis-Sis Kesa, Little Miss Gracie, and the Peanut Girl Meghan Rose!

The Kissing Disease

Ok, my dear husband, has mono. It was diagnosed shortly after we came home from San Diego for the national Alpha-1 conference. He and I had a get away without the kids and needless to say we "got to know each other" again. So, I've been exposed to the Epstein Barr Virus (EBV) or otherwise known as Mono.

Charlie has a pretty bad case of it with an enlarged spleen and hepatitis of the liver. Today, I'm running a fever and have aches and pains everywhere. Advil takes care of it for 6 hours, but then it all returns. I'm hoping beyong hope (denial?) that this does not turn out to be mono. A couple of days ago, I had a nasty GI bug. Maybe that illness lowered my immune system's ability to fend off mono? In any case, it doesn't matter. What will be...will be, but can I just say this sucks? I've never done being sick well.

In My Daughter's Eyes

Artist: Martina McBride
Album: Martina (2003)
Song: In My Daughter's Eyes

Lyrics:
In my daughter's eyes I am a hero
I am strong and wise and I know no fear
But the truth is plain to see
She was sent to rescue me
I see who I wanna be
In my daughter's eyes

In my daughter's eyes everyone is equal
Darkness turns to light and the
world is at peace
This miracle God gave to me gives me
strength when I am weak
I find reason to believe
In my daughter's eyes

And when she wraps her hand
around my finger
Oh it puts a smile in my heart
Everything becomes a little clearer
I realize what life is all about

It's hangin' on when your heart has had enough
It's giving more when you feel like giving up
I've seen the light
It's in my daugter's eyes

In my daughter's eyes I can see the future
A reflection of who I am and what will be
Though she'll grow and someday leave
Maybe raise a family
When I'm gone I hope you see how happy she made me
For I'll be there
In my daughter's eyes

In Memory of Pat

We lost a precious member of our Alpha Pack support group yesterday, July 9th. When Meghan was born (on Pat's birthday), Pat began sending us cards in the mail, and leaving notes on Meghan's caringbridge web page: http://www.caringbridge.org/wi/meghanrose. Pat shared that she had lost a daughter at 20 weeks. She could describe her daughter perfectly to me, and now, I'm glad that they've been reunited, but I'm also sad that another Alpha had to go to heaven. God speed Pat!

San Diego Pictures

Liver Walk Photos

Tribute to Dr. Giles

When I was pregnant with my oldest daughter, Grace, I developed severe preeclampsia. A c-section later and a few days post partum, I was still being treated with a life saving drug called Magnesium Sulfate and a narcotic for pain relief.

I'm not certain which medication gave me halucinations, but I had them. Visions of baby faces, foot prints, and bugs...the creepy, crawling version. Needless to say, I became quite distraught at what was appearing before my eyes. It was 2:00 AM, and I was not sleeping. My husband, Charlie, was stretched out on the equilavent of bamboo mat passed out. I started freaking out, and he didn't wake up. I called the nurse and explained the creepy, crawlies and baby faces/foot prints. All the while, I'm thinking: "Am I insane?"

Soon after, Dr. Giles, a second year resident, walked into my room. I asked him if I was going insane. He said, "I don't know you very well so I can't answer that question." Dr. Giles then turned to the sleeping hump also known as my husband and said, "Hey, is your wife insane?" In his sleepy stupor, Charlie grumbled, "Yes, very." To which, I became a crying ball of post partum emotions, high blood pressure, and tears. The doctor didn't seem too impressed by my description. Dr. Giles discontinued the magnesium sulfate and my PCA for pain relief. By morning, I became a normal emotional post partum mommy, but I didn't see any more visions. Hooray!

The next morning, I remember thinking that doctor was a jerk. He seemed annoyed by being awaken at 2:00 AM and dealing with me, an "emotional" mommy. )Looking back on it, I can see how he was tired, and had probably been on rotation for more than 24 hours.) For quite some time after, I replayed the scene in my head. It was a low point for me in my preeclampsia and NICU journey.

A little more than two years later, I was inpatient in the hospital again pregnant with my second daughter, Meghan. I was almost 27 weeks along with soaring blood pressure, a baby that was too small (IUGR), about 40 pounds of swelling, and the worst headache of my life. Preeclampsia was back with a vengence.

It was day 8 of bedrest, and I noticed that my baby had not been moving around as much. It was a Sunday, and there were several mommies on my antepartum floor whose pregnancies were giving the nurses and doctors much to do. Well, it took what seemed like forever for my nurse to come. She finally came, and hooked me up to a fetal heart monitor. Within two minutes, nurse Yulia was watching and listening to the monitor with a concerned look on her face. She quickly left the room, which gave me some anxiety. When she returned, she walked into the room with Dr. Giles, who was now the chief OB resident.

Needless to say, I was none too happy to see Dr. Giles. I'm not sure what experiences Dr. Giles had been through in the prior two years, but he was a completely different man. His dedication to his chosen profession, desire to answer my questions, and general bed side manner were exceptional. He explained that my baby was experiencing heart decelerations, which meant fetal distress. I would need to be sent to Labor and Delivery to start the dreaded Mag. This also meant that my baby would be coming 13 weeks too soon. 13 weeks! Crap!

I had been trying to prepare myself for this reality all the while on bedrest, but it hadn't quite sunken in yet. I had been a NICU mom with Gracie, but she was only 6 weeks early...small, but mostly healthy. This baby would be around 1 1/2 pounds and need immediate ventilation support. Her chances of survival within the first 4 days would only be 75%. There were so many risks to my dear sweet baby still growing (albeit quite slowly) inside of me...too many to comprehend completely.

For twenty minutes, Dr. Giles sat down in a chair while carefully, quietly, calmly, and lovingly walking me and my husband through what was about to unfold. He also explained that I'd probably have preeclampsia with any future pregnancies. Before he left the room, he walked over to my bed. While touching my hand, he said, "I hope this turns out okay for you Jennifer. Good luck."

How Dr. Giles morphed from "that jerk doctor" into a genuine caring human being I'll never know. But I do know that I'll always remember his kindness and concern. So, Dr. Giles, I pay tribute to your growth and how in the end, all became right in my world. I delivered Meghan Rose the next day weighing 1 pound, 9.5 ounces. She was cared for in the NICU for 79 days, and then came home to our loving arms. Meghan was our living, breathing miracle.

Gracie's Original Diagnosis was SZ

Ann Hum Genet. 1994 Jan;58 ( Pt 1):11-7.

'SZ like' alpha 1-antitrypsin phenotypes in PI ZZ children with liver
disease.

MRC Human Biochemical Genetics Unit, Galton Laboratory, London.

Using high resolution isoelectric focusing, alpha 1-antitrypsin phenotypes were studied in 106 individuals of the PI ZZ genotype including 71 with liver disease, 22 with chest disease and 13 healthy subjects. The resulting Z patterns were found to be highly variable. In the majority of cases (89/106) the maximum staining intensity was either in the most basic isoform or shared equally between two basic isoforms of the Z phenotype. However, in 17 cases there was a marked intensification of the more acidic isoforms resulting in a pattern which closely resembled the SZ phenotype. This 'SZ like' pattern occurred more frequently in the liver group (16/71) than the
chest group (0/22) or healthy (1/13) controls. One possible consequence of the 'SZ like' pattern is confusion with the genuine SZ phenotype leading to misclassification. If this were so, there could be an erroneous exaggeration of the actual incidence of childhood liver disease associated with PI SZ.

Source -Pubmed

Alpha-1 Carrier State a Genetic Modifier?

Presentation Title: ALPHA-1-ANTITRYPSIN - A MODIFIER GENE IN PEDIATRIC LIVER DISEASE?
Presentation Time: 10/31/2004 12:30:00 PM
Author Block: Kathleen M. Campbell, Gajra Arya, Frederick C. Ryckman, Maria Alonso, Gregory Tiao, William F. Balistreri, Jorge A. Bezerra, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Purpose: Homozygous alpha-1-antitrypsin (A1AT) deficiency is the most common genetic cause of liver disease in children; however, the role of polymorphic heterogeneity in A1AT as a modifier of other forms of pediatric liver disease has not been explored. Therefore, we designed a study to determine A1AT allele frequencies in a population of pediatric patients with chronic liver disease, and compare these findings with published population-based data. We hypothesized that non-M alleles are more common in patients with chronic liver disease than in the general population.Methods: In this retrospective, single-center study, A1AT phenotypes were obtained by chart review of patients with chronic liver disease followed at the Cincinnati Children’s Hospital Pediatric Liver Care Center. Chi square analysis was used to compare allele frequencies in the liver disease population to published epidemiologic survey data including more than 27,000 individuals in the United States, and to compare allele frequencies among disease subgroups.Results: A1AT phenotypes were available on 264 children with chronic liver disease. Of these, 23 patients with A1AT deficiency (ZZ phenotype) were excluded from analysis. The distribution of A1AT alleles in the remaining 241 patients was significantly different than published population-based data (p<0.001). The differences in distribution were due to an increased frequency of “Z” and “other” alleles in the chronic liver disease population. When this population was divided into subsets, “biliary atresia” (N=67) and “other liver disease” (N=174), the distribution of A1AT alleles remained significantly different than published data for each subset (p<0.001). Among children with “other liver disease”, allele frequencies did not vary between those with long-term survival with native liver and those requiring liver transplantation. In contrast, among biliary atresia patients referred for liver transplant evaluation, the presence of a non-M allele was associated with a lower mean age at transplant listing than the MM phenotype (235 vs. 779 days, p=0.036), and more frequent loss of native liver by 24 months of age (90% vs. 65%, p=0.04).Conclusion: A1AT non-M alleles are more frequent in children with chronic liver disease than in the general population. Among those with biliary atresia, the presence of non-M alleles is highly prevalent in the subgroup with more rapidly progressive disease. We speculate that these non-M alleles may act as genetic modifiers in pediatric liver disease in general, and modulate disease progression in children with biliary atresia in particular.

A Diagnosis

In April 2002, our Gracie entered the world screaming as loud as she possibly could. She’d just been “extracted” from her warm, soothing water world by the unexpected hands of a surgeon. It was as if she was saying, “Put me back in there.” But, I remember feeling so relieved when she cried and wailed loudly.

In the previous two weeks, I had been on the severe preeclampsia roller coaster ride with sudden onset swelling/edema (40+ pounds), dangerous high blood pressure, vision disturbances, and kidney failure issues, the worst headache of my life, and lots and lots of bed rest on my left side. I was only 34 weeks pregnant. It didn’t seem like life could be any scarier.

Well, the moment I saw my baby Gracie, I forgot about what I was experiencing. All that I could see, hear, or think about was Gracie, our gift from God. My husband, Charlie, was all grins, too.

Grace was born small for gestation because of my preeclampsia, weighing in at 3 pounds, 14.5 ounces. She had some difficulty breathing because she was a bit premature so she went straight to NICU.

About two weeks into Grace’s stay in the NICU, her neonatologist mentioned that something might be wrong with her gallbladder, and he wanted an ultrasound with some blood work. He was so casual about it that we didn’t think much of it since Gracie was being put through the gamut of NICU experiences anyway.

In NICU with Gracie, we passed some of the time by reading her chart from top to bottom. Most of the time, we didn’t know what we were looking at but did see abbreviations in the chart that had abnormal values. I can still picture that piece of paper in my mind. The letters AST, ALT, GGT, and Alk Phos had abnormal results. We both wondered what that meant.

We asked the doctor about the ultrasound results. He said they were normal. I also asked about her blood work. He said that premature babies often have abnormal blood work, but that he was calling in a specialist from our local Children’s Hospital to check her out just in case since her direct bilirubin was still elevated.

I can’t remember the exact day that the gastroenterologist found us in the NICU, but I do remember Charlie and I were caught off guard and quite confused. The doctor kept asking us if any members of our families had ever had emphysema or liver issues. I didn’t know of any and neither did Charlie. I finally asked her, “Why?” Then came the words that changed our lives forever…Alpha-1 Antitrypsin Deficiency. I thought, “What did she just say? Did she just say genetic disorder? Alpha what? Possible liver transplant? Huh? How do you spell that? We carry what gene?”
Well, Grace went home with us 23 days after she was born. She struggled to gain weight, took a battery of fat soluble vitamins, threw up quite a bit, and was generally a fussy baby. She eventually started to gain weight when she was about 6 months old. Her liver labs started to go in the right direction, and life just kept going on and on.

Now, Grace is a healthy girl. Looking at her, you would never know that her liver doesn’t work properly and that her lungs need vigilant protection from cigarette smoke, pollution, and other irritants. Gracie doesn’t understand that she has a genetic disorder. All that she knows is that playing with toys, reading books, spending time with Mommy and Daddy and chasing our cat and her sisters is what life is all about. Only time will tell if Alpha-1 will ravage Grace’s liver or lungs. Meanwhile, Charlie and I treat her like the precious gift that she is, remembering that Grace’s liver allows her to LIVE.

Pictures

Dear Meghan

Dear Meghan,

When your sister was about 15 months old I was having so much fun being a mommy, I began to have what we, mommies, call baby lust. Your big sister, Gracie, was at a very fun age filled with exploration and inquisitiveness, but she had Alpha-1.

Your daddy and I eventually decided to add to our family, but that was not without reservations. We discussed having another baby from a risk benefit perspective. After all, I had a high risk pregnancy and birth experience with Gracie because preeclampsia reared its ugly head. My life and Gracie’s life had been threatened by my high blood pressure, seizure risk, and kidney dysfunction. In addition to preeclampsia, we also carried the Alpha-1 genes. It was a confusing and stressful time in our lives. We weren’t sure we wanted to inflict that possibility on another child by choice.

In looking at it in terms of benefits, we were normal adults who wanted to grow our family and cultivate the love we had for one another in our children. It wasn’t fair that preeclampsia happened to me. It wasn’t fair that we both carried the Alpha-1 Z gene. It wasn’t fair that we had no control over preeclampsia or our genes. I knew in my head that I didn’t ask for preeclampsia or Alpha-1, but somewhere deep down inside, I felt like I failed as a parent. I wallowed in the guilt, but somehow, somewhere I eventually found the strength to pull myself out of the darkness and be there for your daddy and Gracie. I’m so happy that God helped me because that led me to you, my baby girl, Meggie.

Unfortunately, I developed preeclampsia with you too, and this time at 25 weeks gestation. You were born a peanut weighing an unbelievable 1 pound, 9.5 ounces, and stretching out to be 13 inches long at 27 weeks. You were in the fight for your life. We weren’t certain that you’d be okay. Your lungs were quite immature, and your liver was not working as it should. I kept wondering what that meant if you were an Alpha like Gracie. What would it mean if you had Alpha-1 and you were born too early? What would that mean for your future lung and liver health?

After about 2 ½ months in NICU, you had finally stabilized enough to have your Alpha-1 tests. I remember getting a call from your daddy, who was visiting you in the NICU. He was there when they delivered the test results. You were an Alpha, too. My heart sank. I could hear the sadness in his voice. Tears streamed down my cheeks. It was a hard day, but I soon remembered how much of a miracle you truly were. You were not a guaranteed child. You were a gift from God.

So Meghan, I write this letter so you understand that your mommy and daddy didn’t want to take a chance with your life. We just knew deep down inside that we needed to be parents again. You were meant to be. We’re sorry that Alpha-1 is a part of your life, but we want you to know that it should not define you. You are our sweet, inquisitive, joyful daughter who also happens to have Alpha-1. Your mom and dad love you, and, yes, we will probably always struggle with guilt, but we get to experience the miracle of you. That overrides the guilt.

Love,
Mom

My Introspection

Reading a post by Neonatal Doc made me think about how I can help improve the health care system as a consumer. I've certainly not paid enough into my insurance for what I've gotten out of it. I've been severely preeclamptic twice, and have two beautiful former preemie daughters. It feels like day in and day out I review insurance paper work to identify errors...some to my advantage and some not. As a consumer of health care, I need to do a better job of understanding the costs of what my family consumes. I think we Americans have become lazy. We rely on somebody else (aka insurance cos.) to do our jobs as consumers. I know that I'll be doing my small part to find the best quality health care while paying reasonable prices for it. I will not go blindly into the night anymore.

Geeked Up

Call me strange! Call me weird! Call me whatever. I find it very interesting that I've become addicted to reading blogs on the net. Today, I was reading one of my favorites by Moreena called the Wait and the Wonder. She referenced a new blog that was her favorite called Pediatric Grand Rounds. I checked it out, and since I'm all geeked up for medical topics, I'm hooked. I especially like reading a blogger called Neonatal Doc. He shared a story of a woman who was given a toxic dosage of magnesium sulfate while on bedrest 7 months pregnant. This hit home for me, the former 2 time severe preeclamptic. It reminded me how easily I could have met the same fate. Thank goodness for proper medical care. Of course, I can say that now since preeclampsia is becoming somewhat of a distant memory. I do have some rather vivid memories of cursing my doctors when my lymph glands swelled up and I looked like a horrible swollen Michellin man, also known as a postpartum preeclamptic. Ugh! I guess those memories aren't all that distant. My former 27 weeker is now 2 and only 21 pounds, but has become quite the girly toddler. She has become quite the diva in regard to specific Sesame Street characters who must or must not appear on her diapers.