The Front

If I smile, laugh and seem to be enjoying myself, it is a front. While I am human and completely capable of enjoyment despite what we are facing, I feel pain down deep into the core of me all the the time. I feel guilt when I enjoy myself, fear when I see Livi's unsteadiness and overwhelming sadness when I see clothes in her closet that I had been setting aside from Aria that she may never wear. Walking through TJMaxx and seeing clothes and thinking, "This would be cute for Liviana" and then realizing, "Oh...she may not even get to wear that size, or make it to that season" was more than I could handle yesterday.

I really feel like I am an empty shell going from room to room, task to task. I love my time with the kids, they still make me smile and happy but for the absolute love of all that is good...how can I possibly live for one minute of any day without thinking of losing my baby. I cry, when I am alone, at the fleeting thought of our days without Liviana. She is part of us. We love her, we laugh with her, we imitate her saying "alright" in her funny, sweet little voice.

It really is such a deep, deep pain. I don't know what we are facing either, which makes it worse. I can handle the physical deficits. I will be by her side for every feeding, walk across a room...anything she needs. I am overcome with fear however about mental decline. I don't know how I will handle Livi not being "all there" mentally. I want her to know love every minute of her day and I am fearful that she won't be able to feel that love as she mentally declines.

I want to tell everyone I see about her, despite me being a somewhat private person. I want everyone to know that amazing face, those big eyes and those long, dark lashes. I want everyone to hear her sweet voice, see her kindness and how she takes care of her little brother and watch her grow. How, how, how can we possibly live without her. I want to scream as loud as I can, "This isn't F------ fair". I am wasting away inside as I wait for what comes next and live this nightmare for Liviana. I would give anything and everything for her as long as I could still be a mother to my kids. I have actually thought that if I could give my legs and just be a half a person I would still be able to mother them, hug and love them and she would still be here.

Blah...dinner is waiting thanks to some amazing, wonderful people from our church. I don't know what we would do without all of the people in our lives, whether they have known us for years and years, are family, are long lost friends or our new family at church. No parent should have to go through this and I would give anything in the world for Liviana to not have to go through this.

Thank you from the bottom of my heart for all of you.

Another Journey, Another Fight

I always felt bad that I didn't keep Liviana's blog updated. She was doing great though and part of me was happy to not have to update on medical issues.

I am saddened beyond sadness however to make this newest update and begin a new journey and fight in Liviana's life.

At this time, I am not going to recap the slow, progressive decline that led us to this new diagnosis. I likely will throughout upcoming posts but I told the same story about 10 times in the hospital this week and don't have it in me to go through it again.

Liviana has been diagnosed with a rare genetic disorder called Leukodystrophy. There are 2 dozen types of LD and we are awaiting tests to determine what type she has. The hard part....LD is not treatable. It is a progressive, degenerative brain disease. She is basically losing the white matter on her brain. I am sure I will talk in more detail about LD later but that is the basics for now.

Livi has significant gross and now, fine motor skill deficits. She has difficulty walking, although she surprises us at the end of the day with a mini-rebound of sorts. She has balance and coordination issues and hand tremors. She has lost her gag reflex and is on thickened liquids to prevent further aspiration (which a swallow test revealed) into her lungs.

She is still so so beautiful, funny, witty and a ray of sunshine wherever she goes.
She is surrounded by love and we will do all we can possibly do for her.

This is short and sweet. Her blog will become active again so there will be more updates. I am overwhelmingly fearful, in denial that my daughter will not be here one day and hoping that the doctors are wrong, although that is 99.9% not possible at this point.

I welcome all of the research warriors out there to search out information on Leukodystrophy. She does not have ADL, which only effects boys. They believe she may have one of a few types...the two they mentioned were Metachromatic and Krabbe's Disease. At this point her EEG looked normal and she her optic nerve looks great. There is experimental gene therapy being done in Europe for Metachromatic LD.

That is all for now. When I have a chance I will update her photos on the blog. She is an amazing beauty, inside and out.

Thank you to everyone for your support and love. It keeps us going!

Bad Blog Mommy

I cannot believe it has been February since I last updated Liviana's blog. I should be ashamed of myself.



It seems like time gets away from me with the kids and my photography business and life. I feel terrible that I have done a horrible job checking in with the other CDH babes out there. I know there have been some heart breaking losses lately. I guess I try to avoid that pain in some childish hope that it will all stop...that we will stop losing little ones to CDH. I was contacted recently by a friend I met on Cafemom in 2007. She is a Kansas girl and we had many things in common. She recently contacted me on facebook and told me that a friend of hers had just received a CDH diagnosis. My heart just dropped when I read those words. It is still all so fresh and real. I HATE that any parent will go through those moments and here those words.



Liviana, Liviana....what a little spit fire she is. She spends her days terrorizing the house, taking off all of her clothes and diaper and running past me with a giant smile and the one arm she swings when she runs flapping next to her. She is an adorable, cuddly, lovey, silly, funny little hellion. We are all entertained by her antics. She currently likes climbing on top of anything she can reach and get herself on to. I was standing at the sink washing dishes the other day and suddenly found her standing on a stool beside me, smiling big and trying to see into the sink.

Liviana and Miles are quite the pair. When they get going together watch out. He gets so excited when she starts playing with him and more than once they have had some collisions as they run into each other from all of the excitement. Both Aria and Miles are still so excited when they hear her wake up from a nap or in the morning. They run in the room, smile, laugh, tickle her...so much love to go around.

Health wise Livi is doing great. We had a lovely stomach bug make the rounds in late March. Miles is the only one in the house to escape it but it only lasted 24 hours or less for each of us. It barely phased Livi to throw up. She seemed like it was more of a nuisance to her day than any big deal. We also had a nasty respiratory virus in the beginning of April that all of us had. High fevers, congestion and coughs all around. It seemed to hit Aria and Miles harder than Liviana. She is still breastfed so I'm sure some of those immunities helped her along.



She has another appointment in late August for her spine. I may call and see if we can move it up. I can see that her shoulders are not even and she has horrible posture. If I put her on a counter or other flat surface with her feet hanging off she cannot sit up straight. It really worries me but I am trying to stay positive about it all and know that she is so strong and will make it through anything. She can't turn her head in each direction completely but she has adapted well to that challenge as well.

Have I mentioned that Liviana has curly hair like Miles....too cute!

My business is going really well. You can visit my website at Amy Price Photography . I am loving photographing newborns the most. Take a peek and enjoy :).

Liviana, very unexpectedly, very not planned and despite prevention methods, is going to be a big sister. It is obvious that there is a plan out there much greater than us. We don't know much at all yet. It will probably be late December, early January when the little surprise will arrive. Aria will be 5, Miles will be 3 and Livi will be almost 2. I must be insane....all while taking care of my kids and running a business. Don't look in my laundry room...something has got to give ;). Of course I am terrified about birth defects and a genetic disorder since that issue is still unresolved with Livi. I will keep you all updated.

Gosh..I think that is all for now. I will try to update more often and certainly will after any appointments. To all of the CDH parents out there I think of you and your little ones often.

All my best....
Amy

Another Special Anniversary

A year ago today we got to bring our sweet, strong, beautiful girl home for the first time. I remember the day so clearly like it was yesterday but it strangely feels like it was someone else's life too. She is so "normal". Nobody would ever know what the first month of her life held. When I tell people about her (which is every chance I get) they look in such wonder and amazement. Not only have they never heard of a Congenital Diaphragmatic Hernia but they would expect someone who had major organs relocated, a partial left lung, a heart defect and multiple hemivertebrae to look less well, less normal, less vibrant. That's our Liviana though.

I apologize for the delay in posting. I know have three very active, bipedal children who keep things going around here. Livi has been walking for a few weeks now but not until this week had she decided to make it her main mode of transportation around the house. I don't think I saw her crawl at all today. It is so funny to see her walking. People have been startled to see her walking because she is so petite looking.

She has not had her 1 year appointment yet (It's next week) but I believe she is over 20lbs. On our scale she is right at 20 and feels every bit of that weight. She looks so petite but she is quite the lug...a cute, adorable lug though.

She is starting to vocalize words quite a bit. She says, "There she is" is in her own words which she picked up from us saying, "Where's Liviana"? She has the usual Dada and Mama and she also says "Uh, Oh" and has mimicked "camera", "Miles" and "Aria".

I wish everyone could be around her. She is a ray of sunshine. She smiles, laughs and tries to carry larger than her objects around. She loves to eat and is still getting mama's milk too. She adores her brother and sister and when they go to bed before her she tries to escape down the hall to wake them up. I love seeing the kids all play and laugh together and Miles and Livi are quite the troublesome pair when left to their own devices.

As you can see I have bombarded you with tons of photos. I am in the process of building into a professional photography business. I am very lucky to have willing, happy and beautiful subjects, *most* of the time. I will be adding yet another blog to my listing soon with my photography information (A. Price Photography). The blog did an odd crop on my photos. They all lost the side...I'll have to work on adjusting that.

Thanks everyone for continuing to follow Liviana's Journey. She will be having more appointments this year and another x-ray for her spine soon. She really has poor posture and I can see that the curvature I saw back in the summer seems even more exentuated. Let's hope it is mommy paranoia.

Enjoy the photos and keep all of the CDH babes, past, present and those yet to come in your thoughts and prayers please!

Much love....Amy

Happy 1st Birthday Liviana!

In honor of Liviana's 1st Birthday I have written her a letter and made a tribute video for her. Make sure you have your sound on. Enjoy!

My Dear Liviana,
I can't believe it has been a year already. I swear it was just yesterday that I was planning for your arrival with a combination of joy and fear. So small, yet so strong, describes you perfectly. You took CDH on head first and never looked back.

I prepared for every possibility with you. I researched anything and everything related to CDH so we would be prepared for the worse, yet hope for the best. I never anticipated you would kick CDH as hard and swift as you did. Not only did you beat CDH but you surprised us with heart and spinal issues that barely phased you and still don't today.

When I look in your eyes I see someone who already has learned an important life lesson...the lesson that life is too short, anything can happen, live each day as if it were your last. With your smile, your daily joy and your amazing interest in everything around you I feel as though you already know what you have been through. You already know that it could have easily been different and you could be an angel above with all the other CDH angels. I feel as though the CDH angels who have gone before and after you are with you, cheering you on each day.

I thank you for so much Liviana. I thank you for inspiring me. I thank you for challenging me. I thank you for making me smile in an instant. I thank you for bringing joy to your brother and sister's lives. I thank you for tucking your sweet hand under my chin each night as you sleep. I thank you for your loving gaze up at me as you nurse. I thank you for the peace you seem to exude. I thank you for teaching me about facing fears and being strong. I thank you for the hugs and cuddles. I thank you for teaching me about CDH and giving me hope that together we can fight it. I thank you for being such a fighter. I thank you for your spirit. I thank you for your sense of humor. I thank you for your feistiness. I thank you for being in our lives each and every day. I can't imagine a single moment without you.

Happy Birthday sweet girl. You made it here with strength, grace and perseverance. I look forward to what you will continue to teach me and bring to our family for all your years to come.

Make video montages at www.OneTrueMedia.com

Brad's Surgery Update

I decided to just post his update here for ease and simplicity.

Good news first...no apparent cancer in the initial frozen sample. They also ended up NOT doing an entire lobectomy and just removed the part that was infected. The doctor said he had several areas called "Blebs" in the upper left lobe. He also had an apparent unknown infection, inflammation and his lungs were stuck to his ribs. He had to separate his lungs from his ribs and removed the area that had all the issues. No wonder he has been in so much pain.

I did not get to see him before I had to leave. I left his bag for him and will probably get up there first thing in the morning with the kids. This is such good news. The doctor kept reiterating that he did not know if this would take away his pain. He said he is not about removing his pain. I have to think it will though, even if the doctor is not in the business of pain removal.

We will need to await toxicology to find out the unknown infection. It must be something obscure since tests already done have come back negative. I am betting on something fungal but we will see.

Ironically when I did a search on Blebs I found that it is sometimes linked to Congenital Diaphragmatic Hernias. They can cause a collapse of the lung. Brad and Liviana now share incisions from surgery in the same place. I always knew they had a special bond :).

He will probably be in the hospital for 2 days. They said he can go home once his chest tube comes out.

Thanks everyone for your support! It means the world to us! I am doing a Happy Dance tonight!

Sour Apple!

Do you see that ticker...the one above this post that says how old Liviana is? Is that right? It can't be right. I really cannot believe she is almost 1. I swear I was pregnant with her just yesterday, sitting by her isolette in the NICU just yesterday and bringing her home, just yesterday. This has been the fastest, most fearful and most joyous year of our lives.

Liviana amazes me every single day. She is getting so big and active. She is so smart and still such a keen observer. I can' really tell she is growing. On our scale at home she is 19.5lbs. She really doesn't look it at all. She is a petite little lady. When people see her walking in public they are going to freak out and think she is a 6 month old. About that walking...she takes steps here and there like a pro but she has not made the decision to go bipedal full time yet. I'm in no hurry, as it will add to my daily challenges but I'm sure she will be there before her 1st birthday.

I'm working on a video and post in honor of her 1st birthday. It is an emotional and rewarding endeavor and I hope you will all enjoy it upon completion.

A quick update on Brad's situation. He is having surgery this Friday, January 9th. They will be removing the upper left lobe of his lung. He is expected to be in the hospital for about 3 days. They said that once his chest tube comes out he can go home assuming all is well, which is usually 2-3 days. I am hoping the doctor's surgical skills are better than his bedside manner, which left something to be desired. I'm going into this positive even though I can't help but be worried about he process. This is almost 1 year from when Liviana had her repair surgery that directly impacted her lung function. Brad will also be having his done laproscopically just like Liviana, in the same place, with the same incisions. What irony! If he has too much scar tissue they will have to do an open surgery which I really hope does not happen.

Keep him in your thoughts and prayers please as well as the kids who have a hard time when Daddy is not around (especially little Miles).

I will likely move the update on surgery over to our other blog but I will post the notice and link here on Liviana's blog for all who want to follow.