Hi, Miss Smarty Pants!

I must admit, I am very biased. I think my kids are beautiful, wonderful, smart little people. Liviana, of course is no exception. She is, by my mommy standards...a genius.

Little Miss has started waving and saying "Hi". It is the cutest thing ever. She says Mama and has many other jibber jabber sayings that I'm sure are quite profound and meaningful. I am so proud of my little sweet pea. I need to try to get her on video waving and saying "Hi". I will make that my new goal for the week.

I have always noticed how incredibly observant Liviana is. I wonder sometimes if her days laying in the isolette and crib at the hospital have made her skills of observation beyond those normally found in an infant. Maybe she learned how to maximize observation from because of being a passive observer in her previous environment. I realize how much she observes when I see her begin doing things she has watched Aria and Miles do. She sat for at least 10 minutes the other day trying her hardest to put Legos together. She was not just banging them on each other, she was turning them over, lining them up and trying to put them together. She also will put them, with success on the big Lego board, but has a harder time putting two blocks together.


Sunday, I heard her making her growling sounds and when I glanced at her, she had picked up the paw that goes with Miles tiger costume and was waving it around and growling at Miles. She has seen him with his costume on waving his hands, er paws, and growling and she was imitating him even though he had not had his costume on for several hours. So, stinking cute...and brilliant :).

She is pulling up everywhere and anywhere, beginning to cruise along furniture and this morning I caught her with one leg up on the bottom step trying her hardest to get the rest of the way up. She puts everything in her mouth and I will see her picking up something she shouldn't as it heads into her mouth I'll say, "Livianaaaa" and she will look at me, start chuckling and drop it. I wish everyone could see her adorable little face and smile.

Okay...enough of my bragging. I am just so proud of her. I am so struck each day by how "normal" she is. I never, at this time last year could have imagined more perfect, sweet, adorable, funny, precious, healthy little girl. We are so truly blessed.

She now has three teeth...the two on the bottom and one on top. She is still just exclusively breastfed. I have not started her on solids and she is doing great and growing well. I can tell she is getting taller. I think she is going to be a tall, skinny little girl, imagine that.

We have lots of snot going around the house right now. All of the kids have a lovely runny nose, a tell-tale sign that winter is approaching. Livi seems to have it the worst and I am hoping that she does not get pneumonia like the last time a little cold went around the house. She really did great last time so I'm optimistic we will get through this unscathed.

There is so much going on with Liviana's CDH pals. Carter made his appearance almost 2 weeks ago in Blue Devil/Tarheel land and is resting his lungs on ECMO and charming everyone with his rockstar hairstyle. Little Nayeli is expected to make her debut today or tomorrow and Sydney is not far behind. Little Ava Helmick reherniated and has had an extremely difficult time post-surgery. Please keep her mom Terri and Ava in your thoughts and prayers. I have added a link to their blog at the right. We also had lunch and an extended afternoon with Angle Elli's mom last week. It was wonderful to meet her and I can see what an amazing mother she is and how lucky Elli is to have her.

More to come soon.....

Amy

Changing of Seasons and Memories

Fall is in the air. Upon the first glimmer of fall about four weeks ago I found myself feeling very emotional. The change in the air, the sound of leaves, the smell of fall, all brought me straight back to this time of year last year. We are coming up on one year since we got Liviana's diagnosis (October 12) and I never would have thought it would have impacted me as it has.

It is amazing to me how something so simple as a season change can force so many emotions and memories to the surface. I could not escape it. I found myself right back in Wichita, first getting our CDH diagnosis, crying all the time, fearing for the life of our daughter at every minute of every day, not knowing where she would be cared for, not knowing how we were financially going to handle all of it, not knowing what the future held for our family that was to become 5. Would we still become 5 or remain 4 with only memories of Liviana? I remembered Rainbow House and our appointments in Omaha, trying to find a home, trying to stay positive for Miles and Aria despite my overwhelming fears and anxiety. I had a friend recently ask about Halloween and what the kids did last year. I had to stop and think for a minute before remembering that Halloween was spent in Rainbow House while we looked for a place to live.

It really took me several days to fight against the constant memory of last year. As the days have gone on and the season gets more and more evident I have been distracted from the negative emotions by Liviana's smile, her laugh, her keen observation of her environment, her crawling (that started this past Saturday) and her growing butterball belly. Liviana co-sleeps with us and I am replacing those memories from last year with the memory of her sweet little hand on my arm at night as she snuggles close. I am focused on this amazing little girl who has no idea the powerful impact her short life has had on us.

Next year at this time I may still be taken back to those challenging and emotional days, and that is fine. I know however that new memories will be created this year, of her crawling, her growing, her overwhelming desire to eat the grass outside when she is in the yard. I will never forget the challenging times but I am so amazingly grateful and blessed that we are able to create new memories that are so joyful each and every day.

We Interrupt This Dance Party...

I'm taking a break out from our family room dance party to share some more recent pictures before I forget.

I am debating about making "Liviana's Journey" transform into a Price Family Blog or add the Price Family Blog onto my other blog, "The Vacuum Chronicles". I only have one post on The VC but I could easily fill pages with the antics of our daily life. I really feel like I want to keep Liviana's Journey just for her so I will probably begin adding and updating on the family as a whole on The VC. Now that I have worked through the issue, while typing on the blog I will let you look at some new photos. Have a great weekend everyone!

So Much To Update

I apologize for the updates being far and few between. Each time I had something to update I would decide to wait since something else (an appt., a gathering, a visit) was coming up soon.

Liviana is doing really well. She crawls...backwards. She has not quite figured out the usefulness of the crawl for propelling her forwards. She goes from crawling or laying down to sitting and back again. She covers quite a bit of ground when given the opportunity. She has been saying "Hi" in a sweet sing songy voice after I prompt her with my sing songy "Hhhiiii" several times. Anytime I say "Boo" from anywhere in a room she will start laughing. She anticipates the angst that is soon to follow and can't contain herself. I have heard "mama" several times and they seemed very specifically to be directed towards me. She is indeed the cutest, sweetest, funniest little 8 month old around...I am of course a little biased.

Since my last update Liviana has had several appointments. First, for her weight. CDH babies are often watched for their weight gain. It can be difficult for them since they burn so many more calories with their respiratory system than normal. At Liviana's appointment yesterday she weighed.....drum roll....17 lbs. even! I am so proud of my little butterball. I didn't need doctors telling me she was gaining weight thought. I could easily tell when I was holding her that she has been packing on the pounds. She was also measured at 27 inches in length. Livi is still pretty much just breastfed. She has minimal bites here and there. She really is not interested in food. She likes the spoon and the bowl but the food, she is not so fond of. The faces she makes are quite laughable...they really must taste horrible to her. So far the only thing she seems to like is plain, organic, Greek yogurt. She makes quite the mess of it also. I'm not too worried about the eating. She is clearly getting all she needs from breastmilk given her growth. She'll eat more solids when she is ready. Miles did not eat until almost 8 months old and you can't keep food out of his mouth now.

She had her NICU follow up last month and that went really well. She had developmental assessments by the psychologist, PT's, OT's and doctors. It was a 2 hour appointment and we somehow managed it with all three kids in tow. I got teary eyed watching her during the psychological assessment. They had her in a high chair and tested several concepts like her ability to pick up two items at one time, her ability to take items out of a cup, looking for items that have dropped on the ground and stacking blocks. To sit and watch my little girl perform these tasks with ease was very emotional given the first month of her life. She ended up scoring right at her age and a little ahead in some areas on the assessment. So proud. She also has been sitting unassisted for quite a while and she demonstrated her rolling expertise also. She was right on for those skills also.

I brought the letter from Dr. Olney regarding the potential SCD diagnosis. Dr. N was unfamiliar with the condition, which did not surprise me considering there are only around 130 documented cases in literature. He said he would recommend getting the pricey genetic test done if for no other reason than peace of mind for me, whom he referred to as a "pain in the neck" in a good way :). He really thought Livi looked amazing and he wants to see her back when she is 16 months old.

Our next appt. was with Dr. C, her surgeon for a follow-up. This was short and to the point and immediately followed our already 2 hour NICU clinic appointment. He always checks her incision sites, says she looks great and that's that. He will see her again around her 1 year birthday with X-ray.

Yesterday, Liviana had her follow up with Dr. G., her Orthopedic surgeon. I sent the report from Dr. Olney ahead of time so he had a chance to look it up since most docs are unfamiliar with the condition, I don't think he did. He said that if Livi did have this condition it was a mild variant and not one that was going to produce the high mortality rates commonly found, YEA! He said she appeared to have some kyphosis in her neck but is only showing around a 15% curvature which is too low for them to rule out as being from position during x-ray. He also said he cannot get a good picture of her cervical spine (neck) on x-ray and only CT scan would show that in enough detail. He said he would not order a CT scan until he is at the point of considering surgery. She does have limited motion in her neck to the right and given the issues with her spine it has been decided among many docs that she will not get PT for the issue. He really thought she looked great but wants to follow her closely and will see her again in 6 months. I left the appointment relieved and thankful. We now will await genetic testing to be sent off to Wisconsin for the SCD.

Liviana is now a little sister to a big, 2-year old boy. Miles turned 2 on September 9th and we had a celebration on August 31st when family could be in town. He is such a sweet, loving boy and he loves his big and little sister so much. I am so blessed and proud of all of my kids.

I am being summoned by the little ones and I hear a "mamamama" coming from Miss Livi so I will run for now. I promise more updates soon.

~~Elliott Arcile Reed ~~August 19, 2008 - September 8, 2008

Heaven just gained one beautiful, spunky, amazing, strong little girl this morning. Little Elli passed away at 5:10am this morning in her mommy's arms. You will be so missed Elli, even by those who never were able to see your sweet face in person. You have touched so many.

You are in our thoughts and prayers Cassi and Brian.

Angel Wings (by Jean Rozon)

A precious angel slipped away, no one heard a cry.
No time for Dad and Mommy to sing me lullabies.
My time with you was much too short. I had to leave too soon,
But love had joined us as I grew inside my Mommy's womb.
It wove it's way within our hearts, in all our hopes and dreams,
Until the very purest love became my tiny wings.
Although I could not stay with you, I knew right from the start,
That once you felt your angel's love, you'd keep me in your hearts.
I'm just a little angel but my time was not in vain.
As dark clouds that surround you give way unto the sun,
My precious parents you will see that any heart will sing,
If only for a moment it is brushed by angel wings

William Ethan Morgan ~ 8-19-08 - 8-20-08

It is with heavy heart that I am sharing that beautiful, strong William Ethan Morgan born on August 19, 2008 has passed away. Please keep his family in your thoughts and prayers. Please visit their blog (listed on the right) and leave them a message in this difficult time.

Why must this devastating birth defect continue to take the lives of such strong, perfect babies? We must fight do all we can do to fight this.

Spondylocostal Dysostosis

I bet you are thinking, "What"? I was too.

I really thought we had our answers, or the best they could provide from the Geneticist appointment from June. I was really surprised to get a letter from them with two possible explanations for Liviana's "collection" of issues (Congenital Diaphragmatic Hernia, Multiple Hemivertebrae, Ventricular Septal Defect and one Missing Rib).

The first is a vascular disruption between her and I at some point in the first trimester. I did have a freakishly low blood pressure at 7.5 weeks (60/80) but I don't know if that would cause a vascular disruption. I consider this to be the "we really don't know what caused it so we will call it 'this' explanation."

The next is called Spondylocostal Dysostosis (I have also found it listed as Jarcho-Levin Syndrome). The report says, "...after an extensive literature review, Dr. Olney suspects that a condition called Spondylocostal Dysostosis (SCD) may be a diagnostic consideration for Liviana". Further, "...we would like to stress that it is unknown whether Liviana's birth differences are due to a vascular disruption or to SCD. Genetic testing is available for SCD, if you are interested in pursuing whether this diagnosis may be correct for Liviana. If testing concluded that Liviana does not have the genetic change for SCD, then vascular disruption may be the diagnosis by exclusion."

The letter goes onto to discuss SCD in scientific, genetic detail. I really should have paid better attention to science class. Basically, because Brad and I don't have SCD that means that we both have an SCD gene (there are 3) that are working properly. If Livi did have SCD that would mean that both Brad and I are carriers or that it was an occurrence in just Liviana based on an abnormality in the sperm or the egg. It is incredibly rare and oddly, much of the literature I have read are case studies where the mom and the dad are related...hmmm, does someone have something to tell us?:)

So, what does this mean for Liviana? I don't know. She is perfect. She looks perfect and she is an absolute wonder to me. I am EXTREMELY concerned about her spine and the hemivertebrae. I am already seen signs of issues on her back (I posted about it on our blog but not here). I am terrified that she has a future that involves spinal surgery, rods or even a halo. I want to snatch her up and protect her from that ever happening. The outcomes for much of the SCD or Jarcho-Levin are not good but I would assume if she does have it that she is a mild variant and her mortality is not going to be impacted (can't believe I even have to consider that).

I don't know, I don't know, I don't know. My head is spinning. Livi has a surgeon follow-up on Monday, as well as the NICU Clinic follow-up. She also had an orthopedic surgeon follow-up on September 18th which I am REALLY anxious for. I wasn't expecting to receive something like this at all. We have to decide if we want to have her genetically tested for SCD. She has been tested for hundreds of genetic disorders but this one is so rare that there is one place in Wisconsin that can perform the test. The cost would be around $1300. The only reason I would want to test her is for her future, what she may still face and for her future children. I also plan to wait for our Ortho Surgeon appointment, as I think he will be a good judge of whether he is seeing SCD in her.

She is going to be fine....She is going to be fine....She is going to be fine....

I just keep telling myself that.

On a better note....Leah went home just one day after her second hernia repair surgery. Can you say warrior? She is doing well and wanted to celebrate her 3 year hernia repair anniversary by having another one on the exact same day.

There are a couple of CDH babes expected to make their grand debut this coming week (looks like they all like Monday). I will keep you updated and you can always follow their progress on the links to the right.