Appointment with Geneticist

We had Livi's follow up today with the Geneticist, Dr. O. who saw her in NICU. To review, Liviana had a Congenital Diaphragmatic Hernia, Multiple Hemivertebrae in the thoracic and cervical spine, a missing rib and a Ventricular Septal Defect. Because of the combination of anomalies they automatically check to see if there is an underlying genetic or chromosomal component.

The appointment was very thorough. We went through our family histories and any issues or concerns with us, Aria and Miles and our extended families. The issue of Brad's height and Mitral Valve Prolapse came up and Dr. O. did a very crude test (physical exam) to rule out Marfans. He got the all clear, which we were already pretty confident was not an issue.

Based on the very comprehensive testing with blood drawn in NICU she does not appear to have a "syndrome" that they have the ability to identify. She said initially, with her combination of issues they wanted to check her for Fryn's Syndrome and that has been completely ruled out. She said if Livi had Fryn's she would not be the normal looking, smiling child today. She said there is the label of VATER which is a combination of vertebrae and other anomalies which do not fall into any other condition or syndrome. Basically, at this point they don't know why or how these anomalies developed and at this point they cannot identify one syndrome or genetic disorder for all of it, based upon genetic testing and seeing her in person.

The relevance of all of this is whether we need be concerned about future issues showing up and whether this is was caused by a genetic or chromosomal component that will be relevant to other potential future children or grandchildren.

She wants to see her again in 2 years. She said if she ends up having other issues appear we would likely see them between now and then.

Liviana weighed 14lbs. 7oz. which is up from 13lbs. 10oz. one week ago. The difference in scale could account for such a large increase. She typically gains an ounce a day so that would be a HUGE gain in one week. Our ped's office uses a digital scale and they used a manual scale today. So, she is around the 50th percentile which is so awesome for her condition (she burns so many more calories with her breathing) and all she went through in the first month of life.

So, all in all the appointment was great. They think she is beautiful and amazing and we completely agree.

Much delayed update

Hello everyone! Let me first apologize for the delay in update. Liviana is doing well. She still has a tiny bit of fluid in the upper part of her left lung but it is much better. You would barely have known she was sick. She never stopped smiling and laughing. We have had two follow up x-rays and doc visits to stay on top of things. This last Friday I took her in because I was worried about how much she was moving her upper body just to breath. She fell asleep on me in the office and was breathing the same way, with a lot of upper body movement so we checked her oxygen saturations and it was 98. Honestly, she may have always had that movement with breathing and I am just so much more focused on it because of the pneumonia. She weighed 13lbs. 7oz. at her appt. last Friday and was 13lbs. 10oz. this Friday. She has been gaining 1oz. a day for a couple of months now but only had 3oz. for all of last week. The doctor said that they typically slow down a little right now but we will keep an eye on the weight just in case. We will be back at her Ped next week to xray and check her pneumonia again. She also has a follow up on Friday with the Geneticist from UNMC who saw her in the NICU. I honestly am not sure what this appointment will entail but I am looking forward to speaking with them further about her Hemivertebrae, CDH, VDS and missing rib combination.

We have had a bit of transition that she too (and Aria and Miles) has handled just fine. We ended up in between house closings and found ourselves with a 3 week period of time with no home. We got lucky and were able to rent a home for 3 weeks that was available for the College World Series and Olympic swim trials. The kids are enjoying the "vacation house" as we are calling it because it is right next to a park so they get lots of outdoor playtime everyday. Liviana loves tagging along in the sling or the stroller and watching Miles and Aria play. We were a little concerned about the age of the home (1857) and dampness in the basement as it relates to Liviana's breathing. The doc said it could bother her a little and to just give her breathing treatments as needed. So far, so good. We move into our new home on June 30th and we can't wait to get settled.

We have been lucky that the Suburb we are in right now is having their annual fair called Papillion Days right next to our home. The past two evenings we have taken the kids down to ride the merry-go-round, eat and people watch. Liviana has spent the time in the sling kicking her legs and smiling at those who pass by. Every single person who has said something to her or about her has called her "Fella", "Handsome", or "Little Guy". Hmmm, on the first night she had on a pink dress with cherries. I'm not quite sure how you could think she looks like a boy but she enjoyed the attention nonetheless.

I surprisingly found myself a little emotional this past couple of weeks with our move. I think it was a combination of moving from the home we brought Liviana home to and hearing from some new moms expecting their CDH babes soon. I honestly have never stopped and taken the time to think about how this entire experience has impacted me. I think that is good, in that I have been able to continually focus on the here and now but it can have the downside of catching me off guard with these emotions. As I packed up our room I remembered the nights laying in bed during my pregnancy thinking about her arrival, how would she do and whether she would overcome her CDH. I packed up her pictures from NICU and the little signs the nurses made for her. I folded blankets that were in her isolette and put her little butterfly ornament sent to me from a CDH mom in her keepsake box. Liviana will never remember these times and as time goes on the clarity of the memories will fade a little for me too. However, it takes such a small moment, a blanket, a message from another mother or a photo to make all of those emotions of fear, joy and the unknown come flooding back.

I have posted some new links to the side for some new CDH babes who will soon make their grand entrance into the world or already have. Kinley is in Kansas City and was born on May 25th. Carter (NC) and Baby Girl Reed (KC) are soon to grace us with their presence. Please visit their pages and send them your well wishes. They are going through a time mixed with joy and fear that I wish no mother or father had to endure. I welcome any CDH parents, past, present and future to contact me.

Enjoy the pictures!
Amy

The Honeymoon Is Over

I think we have had an amazing honeymoon period with Little Miss Livi. By all accounts she has been a completely "normal" baby. Now, she has pneumonia. Her little cough had been progressing and even though she was still all smiles and laughs I still thought I needed to have her checked out.

Dr W said it did sound like fluid on her lungs and she was wheezing. They checked her oxygen saturations and it was 92. That would not have gotten her released from the hospital. They did a breathing treatment and she went up to 93, hey, it's something. The x-ray confirmed a little pneumonia on her left lung (the "bad" one) She has breathing treatments at home and an antibiotic in case this is bacterial (which we doubt it is but we have to be safe). I requested an oximeter and Children's home health is delivering that today.

Dr. W. would admit her if her saturations fall below 90, she is not eating or begins running a fever (which has not occured). Let's hope and pray that does not happen and she gets over this easily and quickly.

I am 99.9% sure that this little "virus" was picked up by Aria in the doctor's office waiting room during Livi's 4 month check-up. She was playing with a couple of kids and 5 days later was sick, then Miles, then Livi. She got pneumonia from it though which is the difference.

On a bright note Livi broke the 13lb mark weighing in at 13lbs 01 ounces! She remains her happy self even though I know she doesn't feel well. She sounds like an 80 year old man at times but still smiles through it.

Today is my birthday and the only wish I have is for Livi to be well. I will certainly keep you posted.

Amy- Older and Wiser

Time Flies.......

The Whole Gang



Her First Stroller Ride with Miles


Awaiting Her 4-Month Checkup



Sittin' Pretty


Sweet Feet



I can't believe my little girl is 4 months old! She seems to be getting so big, so fast. Her 4 month appointment went wonderfully! She is 12lbs. 6oz and 24 1/4 inches long (although I think she was a little more on weight...the nurse was quick to pick a weight even though it was fluctuating as much as 6oz up with movement). She has moved up into the 40-50 percentile which is fabulous since she started out in the 5th at her 2 month appointment. Her check-up appointments amaze me because they are so "normal". She has no current issues, she is growing, eating, no reflux problems, rolling over, smiling, laughing, everything she should be doing. She is so awesome!

We also had a follow up surgeon appointment on the same day. She is doing great. They are always so thrilled to see her and her progress in their office. Dr. C. talks alot about her reflux and seems so surprised that she has not been on meds for so long. We will see him again in August on the same day she has a developmental evaluation from the NICU clinic. In August she also has her next orthopedic surgeon appointment to check her spine.

Little Livi (yes, I have changed her nickname spelling :)) is very eager to begin standing. When she sits on my lap she will suddenly stand up. I was shocked the first time she did it a few weeks ago. She completely holds her own weight and just holds onto my hands for balance. I think we are going to have our hands full with this little one. She has a Jumparoo and she has taken to jumping so hard that I'm afraid she is going to get whiplash. She is very active and thrilled about it.

Both Aria and Miles have been a little under the weather and I am hoping that Liviana stays healthy. She has a little cough but nothing I am too concerned with right now. I hope she stays healthy!

I have been reflecting so much lately on the last year. We learned we were pregnant with Liviana almost a year ago. She has made us all stronger and taught us so much. She was always meant to be part of our family and not a day goes by that I am not so thankful for what we have.

Please check out the links to the side. Little Ned recently went home and Sofia is heading home TOMORROW! Yea for them! It is so wonderful to see these babes doing so well.

More later......

Jumping Bean

Liviana acts like she is ready to jump up and start running around. This little girl is a mover and a shaker. Her little legs rarely stop moving. If she is sitting in her bouncy seat or swing she moves her feet non-stop with this look of eagerness on her face. When I change her diaper she pushes her feet off the floor and pushes up so she starts moving away from me. I am amazed by her energy and physical activity and strength at just under 4 months old. She already burns more calories because of her respiratory system, I can't imagine how many more she is burning with the frenzy of movement.

Our little miss is the sweetest. She is such an easy going little girl. She smiles and laughs all day long, mostly at her brother and sister. She is patient and content to just sit back and watch the activity if I am busy with something. She is loving her Bumbo seat, like such a big girl. She is completely enamored with her daddy. Mile and Aria loved their daddy but it is obvious that he is something extra special to her. She will just stare at him as soon as he walks in a room. At dinner last night I held her on my lap and she looked up at him the whole time with a huge smile on her face. I think it was their time together in the hospital all those nights that created a special bond.

I have not had any new concerns about her bowels. She also rarely has any issues with reflux. I was able to modify my diet enough to eliminate any triggers and she has been completely unmedicated since February. When I was first pregnant with Aria in 2004 I eliminated caffeine from my diet and since I have always been either pregnant, breastfeeding or both since then I am still caffeine free. I had started drinking decaf in the mornings but I noticed that it bothered Liviana so that too has gone by the way side. It is an easy sacrifice to make for my sweet girl though. Hot tea in the morning gives me the illusion of a warm cup of coffee.

She has her 4 month appointment on May 18th. I really have no idea how much she weighs. I would say 12lbs. some odd ounces. Your guess is as good as mine though. i will be sure to update after her appointment for everyone.

The pictures are what I wanted to share with you the most. Isn't she just a doll? Not a day goes by that my eyes don't well up with tears as I look at her perfect self in my arms. I remember my emotions of fear during our pregnancy and those frightening early days in NICU and here she is, healthy, happy and brightening our days.

Enjoy the pictures and I will update after her next appointment.

Update tomorow.....

Good News

Liviana does not have an intestinal blockage. After 3 hours and multiple x-rays they do not see any areas of concern in her small or large intestine. They did not see reflux either (as far as I know, the "official" report had not been reviewed yet when Dr. C called me). I am SO relieved.

The process was much longer than I expected. Liviana did great. When I saw the barium in a bottle I was worried she would not take it well since she has NEVER had a bottle before, not even in NICU. She was so hungry from not eating for 4 hours, she did not have any problem at all. After the first "flora" exam we went out and waited and repeated the process every 30 minutes until the barium had moved past her small bowel, which took much longer than expected. Everyone oood and ahhhd over how cute and sweet she was. We had people stopping by the little area we were waiting in to see her. She enjoyed the attention and always gave a big smile for her admirers.

I was really surprised at my negative emotional and physical reaction to the hospital. The smell really hit me when I walked in and got off on the NICU floor to go across the skywalk. It brought everything back to me in an instant. The worry, the fear, the pump room, the bells and alarms, the driving back and forth. I could not get out of there fast enough today.

Despite the worry of the day it was a blessing in disguise. Having three little ones I don't get to spend much time one on one with any of them very often. Yesterday it was just Liviana and I, hanging out together in our little corner. After each exam she would either snuggle into me and go to sleep or she would sit on my lap and chew on her hands, looking up at me. I really, really enjoyed the 3 hours holding her, cuddling her and loving her. I could tell by her calm demeanor that she enjoyed it also. I think however I will have to find a way to share that same one on one time with her outside of the hospital though!

Thank you everyone for your concern, thoughts and prayers. She is doing great and has no plans for surgery again any time soon.