Wednesday, August 20, 2008

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.

Friday, August 15, 2008

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.

Monday, August 11, 2008

Please Keep Leah in Your Thoughts

Leah turned 3 years old today and finds herself celebrating in a Children's Hospital in Des Moines. She was sent there today after a suspected reherniation of her intestines. Her mom said she had been complaining of her tummy hurting but would have moments of being fine. Her motherly instinct took over and she took her to the doctor to be checked out.

Leah had her initial repair surgery here in Omaha but they live closer to Des Moines now and they have another child getting ready to start school so they needed to be closer to home.

Please keep Leah in your thoughts and prayers. I will keep you posted.

Wednesday, August 6, 2008


Little Miss has two teeth poking through on her bottom gum. She spends a lot of her day trying to find things to shove in her mouth and chew. I will *try* to get a picture of these new little pearly additions but I can't make any promises.

Liviana and I have a lot of conversations. She seems to have so much to say, and sometimes with such seriousness. She also expresses her displeasure quite well. She adores her brother and sister but sometimes they crowd her a little too much and she is quite clear about wanting her space. It is actually funny to hear her and see these outbursts of displeasure...It try not to laugh.

We had a few visitors a couple of weeks ago. My mom was here for a few days for her birthday and Brad's sister and nephew were here for almost a week also. We loved the company and they all of course saw the sweetness that is Liviana. It has been SO hot and humid here and we have not been outside as much as we would like. We met friends at the Splash Park last week and Aria and Miles had a blast. I took Liviana over to get sprinkled a little but that was all of splashing for her. She did look awful cute in her swimsuit.

Liviana is making moves to crawl but most of the time she goes backward instead of forward. She has this awkward side crawl, kind like a breath stroke in swimming. She lays on her shoulder and puts her little tush in the air and pushes forward. I have not doubt she will get the legs and arms working together soon though and be all over the place.

Enjoy the pictures. We seem to like polka dots around here. She is showing off a couple of her cloth diapers and covers. She likes cloth on her tushy :). The picture in the polka dotted dress if from my mom's birthay (Nonna).