Today's events...

Today was quite the productive day for Miss Sophie. We got up early for our morning weigh ins and care. We are up to 9lbs..woot woot! Normally, I lady never likes to admit weight gain but in Sophia's case its about time we start packing on the LBS:). She also had PT and OT work with her this morning. They will be coming around twice a week to work with her development. Then Sophie and Daddy got some quality and much needed cuddle time in. We also had some visitors come by and love on our sweet girl as well:) So naturally, it was then nap time for the little one. While she napped, mommy and daddy snuck out for a quick bit to eat. After nap time, John and I got to do our first trach change all by ourselves with Sophia today. It really wasn't as horrible as I had thought. It is definitely a lot easier and less painful on all three of us than changing the dang gauze and straps. After the trach change, daddy got to learn more care and earned lots of checks today. As part of the requirements to go home, John and I have to be checked off on different areas of her care in order to make sure we are capable and understanding the levels of her needs and care before they send us out the door. Good thing too, this is definitely helping to ease the anxiety and transitioning as we work towards home. We still have several weeks to get her care routine down pat. John did an awesome job today by the way. I hate to admit it but I think Sophie liked bath time with daddy more than mommy! Good for them though, I loved watching them together. John is such an amazing and caring daddy. He is very meticulous with her care and has the best way to make sure Sophie knows daddy's there and will never let anything bad happen to her. LOVE, so in love with my family! Finally, today's BIG EVENT was that they put Sophie on the home vent machine today. She is still under the weight limit but because she wasn't requiring a rate they gave it a try. She was doing wonderful for like 6 hrs, but weren't quite pleased with her breathing so they added in a low rate. Hmm...here is the kicker with the rate her breathing became irregular or out of rhythm. So they are going to watch it closely and may end up putting her on the hospital vent again. I questioned them because the point of adding a rate was suppose to help her and give her more comfort in her breathing, so why did it make it worse, Doc?? I swear before I leave this place I am going to have my medical degree or at least I am going to become the patient that doctors hate dealing with lol:) Overall today's events were on the up and up...a couple of steps forward and a couple of steps back...the daily tango of dealing with Sophia's health.

HEAR yeh HEAR yeh....

We got some really great news the other night...Sophie had her initial hearing screen done the last night in the NICU and she passed in the left ear and referred in the right ear!! She hears !! She hears !! What an amazing gift. Mommy already knew she could hear me because she response to our voices but to have it in writing!! Ahh words can't express how beyond happy I am to know she has hearing! Hey one ear in better than none! We already knew she was going to need a A further hearing exam so we are just tickled that she passed with "normal" earring in the left ear. So I am ok with knowing she may still have hearing complications. We won't know the extent of her earring in the right ear until later. but what a joy to know my baby can hear the world's sweet music, laughter, and mommy and daddy's voice! This little girl keeps giving me small gifts I will treasure all the days of my life! Even though this isn't perfect news it is definitely GREAT news for us. Brace for the worst and hope for the best..to us this is beyond wonderful news! Also, we are officially all moved and settled into the infant ICU. I am really liking our new digs! Semi-private space, tv, more visitors, food, room, and Sophie has a big girl bed that makes her look like she shrunk;) I think we will like it here. More play time and more focus on getting home... It will stil be a long road there but none the less we are making BABY steps!

She makes bad days good, and good days BETTER

Mixed Emotions and New Focuses

We've come to a fork in the road... As many of you know, we have been working on lowering Sophia's need for the vent. The ventilator is assisting Sophia in her breathing. Last week we were able to get Sophia on the lowest vent setting allowing her to be doing most of the breathing work on her own. Tuesday and Wednesday this week, they tried taking the vent pressure support away. That would keep her on CPAP only. Tuesday she only lasted 25 minutes off of the support. Wednesday, under better circumstances, she lasted about two hours off of support. Once I got ready to do her care, she started to DSTAT a little on me and then could not settle back into a good breathing rhythm so they added back the pressure support. Yesterday we sat down with all the doctors in a Care Conference to discuss Sophia's status, goals, needs, etc. I began the meeting by asking if the trach was suppose to fix her airway issues, then why is she continuing to need support?? Their response, was one of a few things, of course there is no definite answers ever in Sophia illness because her condition is so rare and TIME is the best answer they have. They mentioned that given her cranial nerve damage, swallowing issues, and her heart condition may be adding the continual breathing complications. There suggestion was that at this time, is it still important to keep pushing her and weening her off the vent or is it time to help her with her breathing and start focusing on her development. GOOD POINT DOC. After her surgery, it has been my growing concern, that now if the breathing is "fixed" then it is time to start allowing Sophie to be a baby! The doctors also made a good point in saying we can continue to work on vent rates, but Sophia can not lay in a bed all her life. She has to be able to sustain her breathing and be a baby. So, we have come to the decision that we are going to be making a move. We are going to continue to support Sophia on the vent allowing her to be comfortable in her breathing until she tells us it is time to make a change in that again. We are also switching the focus now on her development. She is already two months behind from being in the hospital and having 5 surgeries. She is also going to continue to be delayed due to her condition. Again. CHARGE is one of the most complicated syndromes today and it is the only condition that causes so many complex medical conditions. CHARGE babies (people) are the only group of people that have so many and complex sensory losses and disorders as well. Sophia will also struggle in her development because she is/ may be partially blind/deaf. DEEP BREATH and breathe. It is still so hard for me to just vocalize her conditions because I know the level of care and needs she is going to have daily. So we are switching our focus on her development. We are going to start NOW at getting her involved with different therapies and specialists in order to give her the best chance possible and to stimulate and meet her growing needs. First and for most, she is a baby and needs to be that. Second, it is important that we involve these therapies given we do not know the extent of her needs yet but know there is a need. Lastly, we have to let Sophia be Sophia without limitations and we have to wait and she what is truly capable of doing.

So we are making the move, literally... Today we are planning to move Sophie to the Infant ICU at Riley. At the ICU, the focus on development and letting a baby be a baby. They also focus a lot on parent care and getting us ready for home. Here is the kicker, a limited stay at the Infant ICU is 6-8 weeks. Oh it gets better... also because Sophie will continue to be on a ventilator (until she grows and strengthens her airway on her own) once we come home she cannot be switched to the home vent system until she is 10lbs in weight. Ok, Sophia is just now 8lbs 7 oz. She was born 6 lbs 12 oz. It took her 2 whole months to gain just under 2lbs. So given the minimum stay in the ICU and the weight requirements are stay at Riley has just been extended for months....months. :/  Another perk about the ICU, is that is allows 24 visitations for John and I. We can also have one parent sleep by her bed at nights when we need. We are allowed FOOD in the room and there is a TV. Oh the little things in life I tell you what!!! So we are at a catch 22, a stand still if you will. We have decided to control her breathing comforts and allow Sophie to grow and strengthen her airway on her own while allowing her to grow and develop in other ways as a baby should. The benefits are great...Sophia gets to be a baby! The downfall, we have a baby on a ventilator (life-support) its just heartbreaking. Its saving her life, but its heartbreaking at the same time.

On a side note, John and I have been taking the classes and learning her care. Today that care has been made a little more intense by remaining on a vent. Sophia will come home on a vent and require 24 hr care and monitoring. IF, big IF, John and I need to travel with Sophie I will be required to carry a vent machine, a suction machine, an emergency resuscitator, an emergency bag with trachs, tubes, gauze etc, and oh yeah I have to carry a BABY!!!! Let just say John and I will never be going anywhere again, or at least never again fast. It still so difficult to wrap our minds around our new lives and new normal...and really it hasn't even began yet. Staying in the hospital for months on end is the easy part, really! Home is going to be scary...at home we are on our own. Good thing we have several more months to prepare! But for today, we will focus on our move and our new goals of getting Sophia everything she needs to be a happy, healthy baby girl!

Special Gifts and Special People

John and I have been receiving some very special gifts lately on Sophia's behalf. What a wonderful thing is it to be a part of such a giving, loving, and supportive community. Small towns ROCK! Above is a blanket a very wonderful young lady made for us. Honestly, one of the best gifts I have ever received and I will treasure it always... :)

Also, some really special people I graduated from high school with had custom bracelets made for Sophia and are selling them for donations in order to raise money for her medical expenses and needs. They have also set up a donation account at Old National Banks. Really words cannot express how humbling is it to know people truly do care about one another. The best gift ever is to feel loved and the people I have in my life and the people of my great little town truly have made me feel very loved.  Words really can not even begin to express how much this mean to my family. PEOPLE ARE GOOD. I am so honored and can not wait to share these BLESSINGS with Sophia, and by blessing I mean with each and everyone of you! I have a special place in my heart for all of you. 

8 weeks

Sophia Updates: Today makes our stay at Riley 8 weeks and we have yet to be told when could be heading home or what steps it will take to get closer. I have a Care Conference arranged for us to discuss the goals for Sophia, progress, expectations, follow-ups and future plans for Thursday afternoon. Of course, all of the questions I have dying to have answered will only come with time, but I will take what they can give me for now. Like I have said time and time before, the only thing in my control at this point in Sophia's life is making sure I do everything in my power to see to it see gets the best care, specialists, educations and resources she needs. As far as things go with Sophia lately, she has been doing wonderful. Except she has her days and nights a little messed up since being a sleep for several days after the surgery and then getting a fever. She was quite the night owl over the weekend. Monday was a good day. John and I took the first course in the Trach Care classes and then we got to practice on the real thing, Sophia:) Let's just say the doll was A LOT easier. We were able to change her trach ties and clean her site and change the gauze. It all sounds simple enough, but had in a very unhappy, swarming baby, a butt-load of secretions pouring out from every whole possible and two very anxious parents. It was enough to make all three of us want to curl up and surrender. Oh of course with time and practice it will become like second nature BUT do not underestimate the seriousness and stress level this trach business has on the three of us. In time we will (I HOPE) be able to do this in our sleep and in time Sophia will be able to sleep right through it?!?!? UGH!

Yesterday, I think I would have titled this blog "Fallacy and Frustration". Reason: I guess I had the false impressions that when and if Sophia comes off the vent we will be hose free...FALSE. I was told that she would need a Trach Collar to go home on, well to me COLLAR sounds just what it is a collar. I had no idea a collar meant it was a collar then attached to a hose for oxygen and humidity. Seriously! I just want to get rid of the hoses and wires. For crying out loud, I would like to pick my baby up free of HOSES AND WIRES!!! Is that so much to ask??? But to my luck and misperceptions, we will remain on hoses and wires and monitors once we come home. I am guessing you can see where the FRUSTRATION part is coming into play. Also, given the amount of equipment and level of care she is going to require around the clock, that term mobile is growing hazier and hazier. Today, the tried to ween her off of the pressure support for a few hours and that didn't go so well. She was quite fussy and once she got worked up she didn't settle into her breathing as they had hoped so they put her back on CPAP and PSV. We are going to try again tomorrow. This time I told them to wait until I was present so I could console her. She likes mommy to hold her better than the nurses:) So fingers crossed that is successful. They will keep her off for a few hours and then add it back and rotate every 4 hours or so until she has been successfully weened off the pressure all together. I hope this works!

Today, I came into the hospital with every intention of demanding ways to hold my child the way I want...well I got it (with little demands on my part)! Sort of... the nurses worked with me today with tips and tricks for being able to pick up Sophie, hoses and wires and all, by myself. At home, I will not have nurses to help me get her when I need and hold hoses in place while I move her about. So we worked on rigging up ways to fasten hoses to her and wrapping these wires there and grabbing her and everything at once and AAAGH. It works but I am not going anywhere fast again. I was like a spider. One hand on her, one on the vent hose, one on the feeding tube, one pouring in her formula, and my nose holding in her paci!! I will never underestimate the saying about mothers needing an extra hand. I literally could use about three more to handle all of the things I need to do with just moving, feeding, changing, and rocking my baby. Hmm... those simple things many consider the easy stuff. I also got to help with the Trach change today. I removed the trach while the nurse put in the new one. This is undoubtedly a two-three person job, literally. Lucky for John and I that only has to be done once a week, unless for emergency reasons. It was also the first time I was able to see the incision site completely. It was exactly as I had imagined seeing a hole in my child's neck:/ I am not quite sure that I will ever fully get use to that site. Some things are easier that others....The suctioning part is easy. Cleaning isn't too terrible. Changing the gauze makes us both sweat. And adjusting the trach ties while holding the trach in place will require a cocktail afterwards.... (kidding people). So today, I was able to do all of her care by myself with the nurses assistance. From here on out John and I will be doing more and more so that when we do get home and our completely on our own we won't freak out too much (that is the hope anyway). OT came to visit with Sophie today as well. I got some good tips on things to do with her to keep her moving forward so she can start reaching her milestones. Again, being in the hospital for two months and having 5 surgeries has put her a little behind; however, with the right help we will continue to work with her to meet her needs and goals.

ALL in all we have had a few more good days. It is so wonderful to see her smiling and comfortable. She is starting to get more and more personality and facial expressions. It about time she is able to act like the baby she is meant to be. So as I end, I continue to ask for prayers that we continue moving in the right directions and comfort and support along our long journey.

I stumbled upon..

“I am just as deaf as I am blind. The problems of deafness are deeper and more complex, if not more important than those of blindness. Deafness is a much worse misfortune. For it means the loss of the most vital stimulus– the sound of the voice that brings language, sets thoughts astir, and keeps us in the intellectual company of man.

"Blindness separates us from things but deafness separates us from people" - Helen Keller

Tonight, I stumbled upon a blog about a boy named Mathew, a two year old boy with CHARGE. Mathew's father started the blog about his son and his journey with CHARGE syndrome. I immediately was intrigued and couldn't stop reading, crying, and smiling. Many of the things he describes in the daily life of Mathew and their family are very difficult to read... Difficult to read because I cannot help but to think is this a glimpse at what our future may hold with Sophia...for Sophia's future. As difficult as some of the reading may have been, it was also enlighting, hopeful, joyous....and proud. It is not only a blog about a boy but a blog about a family and their true testament of faith and family. It was inspiring to read how a man was making a difference in his world and the world of others through his gift... his son. I hope too be as strong and courageous for Sophia and my family. I hope to see the same achievements and gifts Sophia will bring to our lives. I hope I can make a difference in Sophia's life. I hope to inspire and educate people about charge. With this same hopes I also carry fears.  I  still fear for her hardships and the challenges her syndrome may bring to her and our family.  I pray that as fast as these fears overcome me they are  quickly layed to rest by HOPE. The unknown is scary. CHARGE is a very rare syndrome that was only recently discovered in 1979 and named in 1981 and linked to the chromosome mutation in 2004. There are so many unknown variables in involved in this young syndrome. Many of these answers will not come until Sophia grows or until modern medicine evolves again and again. CHARGE is a disorder that affects one in 12,000 children a year. Statistics say you have better odds of getting struck by lighting in your lifetime. Who knew John and I were so lucky. I guess it is time to start playing the POWERBALL, right? So as we continue on this journey, I hope to find answers but mostly I hope to find joy, strength, pride and wisdom. I hope that Sophia can inspire and change the world as Mathew's story touched and opened my heart tonight. As I end my blog for the night, I will leave you with a quote that Mathew's dad listed in his blog...

“Character cannot be developed in ease and quiet. Only through experiences of trial and suffering can the soul be strengthened, vision cleared, ambition inspired and success achieved.”  Helen Keller

John and I's character and faith is being tested and shaken to the core. Our hearts have been broken and are aching for the things we cannot control. Our minds swirl with questions and fears. BUT our SOULS will withstand these trials and tribulations. Our SOULS will conquer and our spirits will sour. Our hearts will be full again. And our love for Sophia will never waver.... And when we forget how strong we are...please remind us. 

Our Little Love Bug

Today in Sophia World

Sophia has been sucessfully taken off the morphine drip and has shown to be very comfortable and content with pain management without extra support. She has also been switched to manual settings on the vent(CPAP). Both IVs are gone. She is up to full feeds. We are giving them slower though because she did have a Lil tummy upset. They have also made arrangements for Sophia to start working with speech and occupational therapy. They even have music therapy coming in to work with her. These specialists are going to be great for Sophia. She needs all the stimulation she can get now. Being in the hospital for nearly two months isn't good for anyone. So this is wonderful proactive, progress. They are going to continue to ween her off the vent. So hopefully in a few days we can ditch a few more hoses and wires. All and all today was a pretty good day for the Lil miss. We rocked and then she puked on me. Then we took a bath and ate and rocked some more( no puke that round). Then we had some wonderful visitors! We did notice the trach was bleeding a little today. They said it was due to rubbing so let's hope that doesn't lead to anything more. So all and all a rather good day. And if I may say so, no more funny business Sophia...mommys heart can't handle it.

On the up and up!!

Well, Little Miss Sophia had a wonderful day today. I got an update from the NNP this morning and she said she was so happy to see Sophia with so much personality today. Oh and she truly is beginning to develop her own personality. It is wonderful to see her start acting like herself, like a happy little baby. She has remained fever free and her blood pressure is a steady normal. They have also reduced her morphine drip and if she continue to show no signs of pain they are removing it at midnight. They have removed the isolation precautions and her antibiotics as well. YAY!! In the next few days, they are going to ween her off of the ventilator as well. They cut her rate in half today to 14 and are going to move her to CPAP tomorrow morning. Fingers crossed she comes off the vent successfully! One less tube means mommy can pick her up as I please...finally! The doctors use the term mobile, but I like to call it cuddle-able.
Enlight of all the downfalls we have faced lately, I received a very special gift tonight. Sophia smiled ear to ear for me tonight. She smiled, a smile I thought I had lost forever. Tears of joy literally filled my eyes. I will never forget that moment and I will carry it with me always. I hope and pray that precious smile is here to stay, but if tonight was all I get I will be happy with that. I know there will be smiles in the future, but everyone knows what I am saying without saying it.

Sophia was so fun to watch tonight. We rocked and cuddled and smiled:) She is starting to feel well enough to start acting like a healthy baby should. Sophie is getting good at telling us when she is hungry or needs changed. She is even developing the cutest yet saddest little pout face ever. Man, daddy is in a world of trouble when he sees that face. Sophia will have him wrapped so tightly around her little finger when that bottom lip gets stuck out. Sophia Kay mommy and daddy love you deeply and cannot wait to get you home. So, today's update is short and oh oh so sweet.... Here is to staying on the upside of things.

My valentine...

Poor baby girl was too sick to wear her valentine outfit, but we still had to wear our bow for a quick photo:)

Very humbled...

I know it has been a few days... The past couple of nights Sophie has been fighting an up and down fever. Her blood pressure has also been very low at times. The doctors have started her on three strong antibiotics and ordered labs. They put Sophie in isolation for the time being which means gowns and gloves when we visit. It also gave us our own space for a little while. They nurses hung pictures and decorated our new digs for us...love them. So far all of the labs have came back negative. Which is great news. Today, she has been fever free and has had a regular temperature since early this morning. They are going to keep close watch for 24 hrs to make sure she is in the clear until they remove the isolation precautions. She seemed to be doing better today so we will keep our fingers crossed we our on the upward move. Sophie has also had her first trach change. Doc said it is healing nicely. She has also had all of her stitches removed. I was finally able to hold her today and give her a bath. Mommy and Sophie both enjoyed that very, very much.

 You know through all of this, John and I have been very, very blessed and humbled by all of the support and love we have been receiving from everyone. I started this blog in order to share our story with loved ones and to keep them updated. I have also found comfort in being able to speak openly about what is going on in our lives. It is so refreshing to hear the encouraging words from our supporters. It seems like any time we are at a low, I get such amazing and inspiring advice that lifts me right back up. John and I want to let you all know we without a doubt thank you all for showing us such amazing support and love.  We are humbled by your thoughts, prayers, and actions. You are not going without notice! We can not wait for Sophia to hear, see and know all of this same love and support. It is so wonderful to know people are good. There always seems to be a pattern of bad things happening to good people but it often goes unnoticed that those same good people always pull together for each other. Amazing! We are blessed beyond blessed by the people we know in our lives and we take great joy knowing we get to share that blessing with Sophia. And in return we our happy to share our blessing with you.

Good Days

Today was a good good day. Sophia is still in recovery and being kept mostly sedated in order to help her heal. It was very nice to see her so comfortable today and breathing easy. It was reassuring that we made the right decision with the trach. I have a feeling in time I will hardly even notice the foreign tubes sticking out of my sweet girl. Today in fact, I could hardly keep my eyes off of her cute little face. It has been several weeks that I have been able to see her face unobstructed by tape and tubes. She is just so perfect and I love kissing those little lippies and cheekies! I am also so excited to be able to hold her soon. I still can not hold her for several more days while she is healing. So that is hard but we love holding hands. This has been the most difficult 7 weeks of my life (our lives) but I can not imagine another day without Sophia Kay. No matter how much John and I hurt or how angry we get with things with cannot change to make her life easier, when we look into her eyes...we melt and OUR whole world makes sense to us. If being a mother means always knowing your heart is walking around on the outside of your body...then I am glad to have placed it with Sophia. I am glad that our two hearts have formed such an amazing little lady. Sophia is John and I's true testament of our love for each other and for her.

Taking CHARGE for Sophia

This post is one I have been struggling to find the words to write for some time now. Mostly, for some time now John and I have been struggling to understand the many unanswered questions going on with Sophia's health and the uncertainties that would mean for our family's future. Only a few days ago, we got those answers. So we took some time to figure out between ourselves what these answers mean before we decided there is no time like today to share these unanswered questions with those who truly love and support our sweet girl.

After a few days in the hospital, Sophia's health issues started to grow. It seemed like each few days another symptom would emerge from no where. It was a constant battle of up and down between thinking one thing was being cured and another symptom creeping in to cause more problems. I am sure those of you who have been following our story have noticed this trend. Because of the number of symptoms and concerns that kept arising with Sophia, the doctors ordered a geneticist to start following our daughter more closely. The genetic doctor began ordering tests, labs, and several different exams to try and put the pieces together as to why Sophia had so many life threatening health condition right from birth. As you remember, this all started with her Cohanal Atersia, then the heart echo showed some minor defects in the heart. However, many of you weren't aware (as neither were we until after the fact) that this began in the womb when they discover Sophia had small reflux on her kidneys or what the call, Hydronophrosis.We were told by doctors then that this was something that could be cleared up with a round of antibiotics. Little did we know, it was leading to something much much more. So within, the first week Sophia was presenting three related symptoms and from then they just kept seeming to pile up. The genetic doctor began to speculate that Sophia had a very rare condition called CHARGE SYNDROME. He then, pursued a chromosome work up and gene analysis with several more screening. These labs were to take several weeks for results. These were the results we were dreading to receive but were not at all surprised by the findings when we received word a few days ago....

The day of Sophia's first surgery, the doctors had wondered and worried given her current symptoms if there were more hidden symptoms that could complicate her procedure. So right before her surgery, the doctors ordered a heart echo stat to check for complications. They also at this time ordered an eye exam. So right before my one week old daughters first surgery, literally right as the wheeled her into the OR I was told the results of these two exams. One being that my daughter has two small black spots/holes in her eyes which are called Colobomas. I was then told that colobomas can not be fixed and may result in mild to severe vision loss. Then there is the kicker, as they had wheeled her into OR, the anesthesiology told us that Sophia had two small holes in her heart. Literally at that moment my heart and myself hit the floor. John had to pick me up both physically and emotionally of the floor. I was devastated. Inconsolable! John at that moment never shined more as my hero and I know he was just as broken on the inside. After we gained composure....which felt like several days later. John and I started to research and educate ourselves on what CHARGE syndrome was and how it could be affecting our daughter and her future. It was very scary reading. So as days progressed, we were on the look out for new symptoms. We found comfort and relief as we could mark some off the list and then disappointed and yet quite consciously aware when another symptom would appear. Perhaps, one of the most devastating symptoms that appeared  for me to handle was partial facial palsy. Days before the discovery, we had been having several really good days with Sophia. She was a happy, content for the moment little baby girl. We had even got her to smile. Sophia gave John and I a very special gift and smiled ear to ear one day while we were holding and singing to her. We were even able to capture the precious moment on video. So much to my surprise and heartbreak, I was torn, anger, and simply felt robbed when the palsy appeared. I felt like this was the icing on the cake. I felt attacked and betrayed that one of a mother most precious treasures was being taken from me right before my eyes, my daughter perfect, sweet smile. Stolen from me forever. I was so angry and at a loss of faith. I felt like I was being punished. It simply was not fair. The palsy too was an evil evil symptom of CHARGE. As a result of the palsy, we had an MRI done to look for answers. There were non to be found because part of the mystery with CHARGE is that is causes cranial nerve damage and the nerves still look completely fine on an MRI. The next couple days, we also discovered on the MRI that Sophia had minor under-developments of the inner ear which could lead to partial or profound hearing loss. Really, what else can this disease be robbing my daughter of... My daughter could miss some of the worlds most precious little gifts. Absolutely heartbreaking. It is still to this day hard for John and I to talk about and to be optimistic about. We want nothing more than our daughter to be able to take part and enjoy life in all of its richest and we hurt because we know she will have many hardships. No one wants your child to have to struggle and deal with hardships out of your control. This is the hardest thing for John and I to understand and there is so much we do not understand. So as days continued to progress, we arrived at surgeries two and three and then more symptoms arise which led us to the current surgeries 4 and 5. Everything is related to CHARGE syndrome.

As days progressed, it became quite clear to John and I as well as the doctors that Sophia had CHARGE. These speculations and fears were confirmed a few days ago. We have had 6 weeks to process this information, this fear, this uneasiness, this worry and well I think we will continue to process these things for much of our lives. However, we have also realized many things along the way and have grown and conquered many of these fears and will continue to battle as a family unit. We are grieving the loss of our "perfect newborn" and our going through all of those stages of loss. We waver between anger and sadness and doubt and fear and acceptance. It is hard to not be jealous and envious of our friends as we watch them bring their "perfect babies" home and yet we kick ourselves for thinking that way. We would never wish anyone to have to go through these hardships with their child. We struggle, we cry, we yell, and we move on. We have accepted our challenges and know we will conquer this together. We have found comfort in knowing that Sophia will never know the difference between our normal and hers. We are slowly growing and getting adapted to our new normal. We have many things to learn and many things to overcome. We will overcome.

So what is CHARGE syndrome...C.H.A.R.G.E. is an acronym that stands for Colobomas, Heart, Atersia, Retardation of Development, Genital/Urinary, and EARS. It is a very very rare syndrome that causes many severe and profound medical issues from birth. Often life-threatening condition like Atersia and heart defects. It can also causes many learning disabilities due to cranial nerve damages and children being hospitalized for so long and often. It can also cause physical changes to ones appearance like small ears or palsy. Charge is a rare syndrome that affect 1 and 10,000 families a year. It is very rarely heard of and many doctors are still unclear of what all the symptoms entail. It is not genetic or passed down from parent to child. It is a random change in  one very small chromosome when a baby is being form at conception. It could happen to anyone, but no one ever think it can happen to them.

John and I have been very apprehensive to share this knowledge about Sophia with the world because we ourselves have so many unanswered questions and we fear being bombarded with questions and worry and pity. The doctors often respond that only time will tell what Sophia will be capable of or how  these symptoms will affect her health in the future. It is so hard to know that these things exist but not know how they will affect your child until she grows and develops further. Its hard knowing and yet not knowing anything at the same time.  Our greatest fear is that Sophia will not be accepted as normal or will be treated differently. We have many fears and worries about her health and future. We are greatly hurt because we can not stop or change the hardships she may face. We have also found comfort and confidence in knowing we will never stop doing and giving Sophia everything she needs to make her life easier. We have found great comfort in knowing she will always be LOVED and she has an amazing support system within us and her family. So for now, our goals and struggles are to get Sophia through these current health issues so we can get her healing and home. We will be proactive in doing what we can to provide for Sophia in all of medical, educational, and emotional needs the best we know how. Sophia may have a long journey ahead of her but it is John and I's goal to make her life the very best we can and fill that life with love and support and comfort. She is our greatest treasure. She is nothing to be ashamed of and she is worth sharing with the world. It may not be the life we had planned but it is our life, she is our life, our love and our strength. She is a blessing beyond blessings and we hope the world will grow to love her as much as we do.

Time to Heal

There is no turning back now. Today has been one of the biggest days for us and Sophia since we arrived at Riley. Today, Sophia underwent surgeries 4 and 5, the tracheotomy and the g-tube. Like most days at Riley, we spent a lot of time talking to doctors, nurses, waiting, preparing and waiting for Sophie's surgeries. Surgery ended up starting around 2 today and once we got the phone call that it was go time, I instantly got that pit in my stomach. John being the awesome daddy that he is, drove Sophia down to the OR in her travel bed because of course no one can drive like daddy and of course no one "handles with care" like daddy. Once in the OR, John and I got to say our "I love yous" as we sent her off in the hands of the surgeons. Today was always the biggest day for Sophia in terms of the nature of her surgeries. Knowing how invasive and how lasting these surgeries were going to be on Sophia really weighed heavy on John and I as we left the OR. John and I both were very overcome with emotions just knowing what our baby girl was about to go through in the OR, and now what we are doing to be going through as a family in terms of the level of care she will continue to need. It just simply overwhelming. Knowing that your baby girl is going into the OR to have holes put in her is hard to digest and knowing these holes aren't going away anytime soon only makes it more difficult. As I have said time and time before, John and I are not naive and we of course know this is for the absolute best and will give Sophia the comfort, support, and care she needs to get healthy and home. It is just simply hard to manage the process, the appearance, and then channel and deal with the management and care. So we broke down and then we helped each other get right back u!!! John and I have found so much strength in each other throughout this journey. I can never express how completely grateful I am to have such an amazing partner and friend in him and I can only hope I am half of what he is to me for him.

 It was very long 4 hour wait. We are very thankful that we were able to have our close family there to support us today and to really just love us and help ease our minds. Once we got news Sophia was in recovery and the surgeries had been successful and small weight was lifted off our hearts but I still had a lingering pit in my stomach. I was so anxious and scared to see her. I was nervous for her new appearance and feared to see her hurting from the pains of surgery. Throughout this journey, I have surprisingly managed to keep my composure when needed, but today was just too much. I almost think I had an anxiety attack just trying to process the "new" and trying not to hurt when I saw Sophia wincing in pain. All and all once the initial shock of it all wore off, I was able to step back, reflect, and say you know what I am so happy that I am now able to see and kiss on her sweet little face without being obstructed by tubes and tape. The new holes and tubes will take some getting use to, but in time I think I will begin to notice them less and less. And the greatest thing of all today, was our nurse allowed us to listen to her breathe through the stethoscope and her breathing was finally sounding free and clear and her baby chest was able to relax. That you couldn't help but to smile at, and be overjoyed with the fact that now she can finally be on the track to healing. Knowing your child now has the comfort she needs to grow is such a blessing that no one can overlook. It gives us peace knowing we made the right decisions for her. And in time, the fears and worries about the trache will too vanish figuratively and literally (someday)!

So now, little miss Sophia is going to be resting and healing for the next week. She will be kept relatively sedated and calm until the trache is changed for the first time and the wounds have been able to firm and grow around the tubes. At the 5-7 day mark the doctor will come around and change the trache, at that time we will finally be able to hold her again. Not being able to hold her will be hard, but worth it in the long run because now I will finally be able to get my hands on her and lover her and hold her like I want too. (well in time). Tonight, the should be able to start feeding her with the g-tube and monitor how that is functioning and healing. Once she is healing and stable enough, John and I will be taught how to properly care for her trache and g-tube and then begin to start the process of heading HOME!!. We can not wait to reach HOME.

GO SOPHIA GO

All At Once, The World Can Overwhelm You...

Today was a big day for TEAM RHODES, Sophia had her breathing tube taken out at 8:30 this morning to see how affect the previous surgery was in opening her airway. Once the vent tube was out, Sophia had a tough time settling into her breathing. In order to give her the best fighting chance, they gave her a quick breathing treatment and vapotherm. All in all her oxygen stats looked good and her CO2 wasn't too high, but within the hour you could tell she was just working too hard and her baby chest was contracting so hard to keep up. It was not worth the risk to make her keep struggling like that so we decided it was best for her needs and comfort to put the vent tube back. I will say one thing, Sophia gave it her very best try and mommy was proud of her for that. Mommy also knows if she can make it easier for Sophia to breathe then maybe mommy could breathe easier too. So now we our on a new path to getting home and that path begins with a tracheotomy and a g-tube. Much to my surprise, the doctors are ready to move on this quickly and have scheduled her surgery for tomorrow afternoon. She will be put under again and will be having two operations at once in order to save her from having to go under a 5th time. Tomorrow will make surgeries 4 and 5 in her 6 weeks of life. John and I are very nervous for tomorrow and for what lays ahead for our family. Having the trache is going to mean many different things and changes for our family. Luckily for Sophia, she will never know any different. I do want to say they are very certain the trache or g-tube will not be permanent things but like many of their answers, only time will tell as she grows and gets stronger. I just can not believe we have gotten to this point so quickly. Perhaps, this in a way with the doctors promptness is a good thing because it keeps me from sitting and worrying about it for too long. On the contrary, I am going to be worrying about it for much longer once it is in my child. I am so apprehensive for the trache because it will mean we are going to be going home on machines and monitors not just with diaper bags and balloons. This also means that we are no longer just mommy and daddy, but we our now Sophia nurses and medical experts. We will have to be trained on her care and what to do in case of emergencies. I DO NOT doubt that John and I can handle this. We are handling this and doing what we need to do for the care of our daughter. We are just so overwhelmed by the nature of it all and how it got from driving to the hospital to have Sophia to still longing for that care ride back home. So for now, we and Sophia are yet again preparing for a very big day, the biggest day yet in terms of her surgeries, and from there we will prepare for the rest of our lives or at least one day at a time or perhaps even one hour at a time...

The surgery is scheduled for tomorrow at noon. We again would appreciate as many good vibes, positive thoughts, encouraging cheers, and powerful PRAYERS for our baby girl tomorrow.

With all the love in the world,
John, Amanda and Sweet Sophia

Hope for the best and brace for the worst!

Well,  Miss Sophia had her third surgery today. Our ENT doctor took her into the OR around 1pm today to take a closer look at her throat with a scope and while he was at it he looked at her nose again. In the OR, they took Sophia off of the ventilator and for a while she was breathing just fine. It is when she is stressed or worked up she panics and tries to breathe in so hard her baby airway completely closes off on her. COMPLETELY!! He was able to capture a picture of it he shared with us. This also explains why she was turning every shade of purple I did NOT want to see on my child. So now, they are saying the laryngomalacia is severe. Dr. Morganstein didn't sound to convinced that the superglytoplasty surgery was too successful but it was still worth giving her the chance to let it work. We are going to give her until Wednesday to heal and then they are going to take the vent out and give her a chance to breathe freely on her own. IF for some awful chance, she cannot successfully and efficiently breathe on her own then they will have to perform a tracheotomy. We will know by weeks end if that will be the next step and the trache will be performed beginning of the following week along with the surgery to put in the g-tube. At this point, we can still only prayer for positive outcomes, but John and I are very aware of what the next steps will be and our preparing ourselves both mentally and emotionally. With the trache there will be set backs but nothing out weighs the risk of not having our sweet baby girl with us.  We know a trache will save her life and we will (no matter what the circumstances) always vouch to give our daughter everything she needs and a fighting chance to get healthy and home is always top priority.Realy, when it is all said and done, a trache will be a very small price to pay for a lifetime of sweet memories with our baby girl. AND in the spirit of being positive, it will not be permenant...once she is older and stronger and ready, we can have it taken out!!  A trache will get us home. And you know what they say, you can not rush perfection and our little sugar butt is absolutely perfect to us.  So for now, sweet Sophia is sound asleep in her little crib in the NICU dreaming of mommy and daddy while mommy and daddy are heading to bed to dream of our sweet Sophia.

Waiting for Surgery

Sophia is just waiting for her surgery on Monday. They are trying to keep her sedated until then because the ventilator makes her gag and she hates it. I guess I would hate it too. It's so heartbreaking seeing her gag over and over until her face turns beat red. Monday they will perform a superglytoplasty (check spelling). This is where they will go in and trim tissue above her voice box in hopes of allowing air to flow freely. She is still so stinking cute and we love her bunches. Again, if this superglytoplasty does not work they will have to perform the trache, and that sucks for us. Amanda and I are doing good and appreciate everybodys kind thoughts and words.

Going Through the Steps

Sophia has been on steroids for the last 48 hours to help reduce the effects of the laryngomalacia. We spoke with Sophie's specialist today and were informed that on Monday he was going to take out her ventilator and see if the laryngomalacia has been reduced from the steroids. If Sophie does okay after the ventilator is taken out then he will take her back to the NICU and monitor to see how she does. However, if the steroids did not seem to help then he is going to perform whats called a superglytoplysty (check spelling). This is where he will take a laser and trim some of the tissue around the air way to open it up a little more so she can breath on her own.
Moreover, if neither of these options work than they will have to perform a trachea. We are hoping for the best but are prepared for the worst. Thanks for reading!