Not the First and Not the Last

Wow, this has been an incredibly busy month for us. Sophia has had several big appointments at Riley and things with therapy. We have seen everyone from eyes, ears, lungs, heart, and speech. As you know, at our last pulmonolgy appointment they took Sophie off oxygen during the day. This has been the biggest blessing EVER. She is doing beautifully. No DESATs or real episodes without it. She is doing great with the HME too. Most of the time, she doesn't even really need that. Its nice to keep it on though for protection. She has so many secretions anyway that humidity isn't an issue. The biggest blessing comes in the sense that I now get to carry my daughter freely about our house like any other baby. I can bring her room to room as I go. I can put her on my hip and leave the room without someone carrying a machine after me or dragging and adjusting hoses. It truly is liberating. We have even been on a few mommy and daughter walks. Really sweet times to cherish. Still longing for the day, I am free to drive alone with her. (this will not happen as long as she is trached). So for now, we will be showvered by daddy and Sophia and I will ride in back. With all these appointments this month, so many changes have happened and yet there are so many answered unanswered. We met with cardiologist last week. They performed and EKG on Sophia's heart. I thought they would do an ECHO but they just wanted to listen to the murmur this time. The murmur is caused by a bicuspid atortic valve. The bicupsid valve is a birth defect (heart disease) that is associated with her CHARGE syndrome. One does not have to have CHARGE to have this abnormality. Many are born with it daily and many live without knowing. In terms of the H in CHARGE, it stands for HEART. In terms of Sophia's condition she has a very mild heart condition on the CHARGE spectrum. Sophia still also has the PDA, which is a small hole in the heart. The cardiologist did an excellent job at explaining both of these to me. At the time these were discovered, it was not priority to treat due to her life-threatening airway issues. The doctor explained that one they like to give the PDA a year to close on its own. We have another appointment schedule for the end of Novemeber for an ECHO. After the year mark, they will continue to monitor it for changes. The only need to go in and surgerically repair the hole is if it would start to leak blood the wrong direction, reguritation. This could never happen or happen several years down the road. The biscupsid valve is on the main artery. Instead of having three laps to open and close the valve Sophia has two that are moderately fused together, making her atery open and close assymetrically. Again, the only need to go in and repair this valve is if the valve starts to leak and the flaps of the openning do not close properly or flip (curl inward). Again, with age and life condition this could be teens, to 20's to 30's in terms of years into her life before they need to have surgery on her heart. The idea of heart surgery still looms heavily within my own heart. No matter at what age in her life if the need should occur she will always be my baby girl and the idea of it will always leave an ache in mine as well. However, for TODAY, we are fine. Today we are OKAY. So for today we will focus. Also, last week we visited with ENT for our hearing screen. The tests they perform in office and for her age were inconclusive. The performed 3 separate types of tests on her ears. One for her ear drums, one for activity and one to show her response. Each test showed a need to further examination. They said they did not see as much activity as they had hoped in both ears. Therefore with her age and the results, they want to do a more finite test that will give us the exact results of her hearing. This test requires Sophia to be put to sleep in the OR. Due to her age and the timing and sensity of the test she needs to be put under anthesthetics for the best results. The doctor also wants to revisit her nose and look at her airway while she is under in the OR. It makes sense to do as much as possible while she is asleep so we do not have to frequent the OR three more times in the nearer future. The doctors are suppose to call me next week with a time and date for this procedure. This isn't going to be our FIRST time into the OR and it most likely will NOT be our LAST. The idea of her being put to sleep again and so soon after being home unsettles me. I know that it needs to be done and I know it is for the "best" but I think the time I start getting use to the OR is the time I loose my hope as mommy. The hearing exam they perform in the OR should tell us exactly how Sophia is hearing and tell us exactly how and what the doctors need to do to help with her earing loss. It is inevidible at this time to say she will not have ANY hearing loss. THE E in CHARGE stands for EARS and the most common symptom is hearing loss. The doctor did say the ear drums look good but he has a feeling the issues may lie within the inner ear. I know Sophia can hear some. She reacts to some sounds now and makes great eye contact and tracking. However, I am not naive to think she will come out of this test with perfect hearing. Sophia will suffer from earing loss. My prayer is that we are able to help her enough that she doesn't loss the melody and sweet sounds of the world. My prayer is that Sophia's hearing loss in minor and with proper medical assistance can be almost resolved. My prayers are many. My answers are few. The doctor isn't sure what he will need to do until he is in there and able to better evaluate her. This goes for her airway and nasal passages as well. If needed he will dilate her nose then and remove scar tissue. There should be any visible evidence of this, not like last time. The doctors will be calling me this week to set up the times and details. Next week we start another busy month of appointments with an overnight stay at Riley for a sleep study. Hopefully the results of this study will bring some wanted and much needed good news and changes. Some times I feel like there are so many things regarding Sophia's health thrown at me at once I barely have time to process it ALL. In hind sight, that is probaby for the best. If I sit and think about everything we have been through and will go through, it becomes too much, much too much. Its becoming expected. Heatlh issues with your child should never be expected. Never. What I long for now, is the unexpected good news, the unpredicted achievements. For now is the mind-numbing  "well she looks GOOD"s, the well at leasts its mild, or the  "she's ready" or the "what do the doctors know" or the "I know she will be fine"s and the "she will pull through"s. Sophia condition will never change. It is expected she is going to have issues all her life. I am accepting of these things. I am accepting these things as they come one after another after another. We will be okay. Hit after mind-numbing HIT we will be okay. We are aware of our situation and perpared to handle these things accordingly. One at a time. Like, I said with all of these appointments, we are expected news. some good and some bad. It won't be our first and it certaintly isn't our last.

Head and Shoulders and Knees and Toes

And EYES, HEART, and LUNGS too. We have had a busy month already. We have already had 3 appointments the first week alone. I am very thrilled to announce that after a long and disappointing appointment last week, we received some very much needed and amazing new changes in Sophia's care. Last week we had two appointments at Riley, one for her eyes and one for her lungs. Her eye appointment was as expected. The doctors confirmed she can see (how exactly will not be known until she is able to tell us herself). They said she had good tracking and she didn't see a need for glasses at this time. They dilated her eyes to see the colobomas again. She has a larger one of the back of her right eye in the outter (nowhere too important) space. On the left eye, she has a small coloboma the nerve and the nerve looks enlarged.  They question (fear) there might be a coloboma inside the nerve. Okay, not the best news, but we already new most of that. What I am choosing to hang onto is that fact she is SEEING! Doctor confirmed. Mommy already knew that. Also, I am taking it as a good sign she doesn't need glasses yet and that her eyes are  focusing and tracking as they should. I will take it! The most exciting news however came from our lung doctor. She walked in saying she wanted to change this and this medicine and change that setting on the vent and to schedule a sleep study for the month. I was like okay good good good. Then I asked her about Sophia's oxygen needs. She said to decrease as tolerated. I said I didn't think she needed it because we hadn't had any oxygen needs previously and she said take it all away as tolerated. I said ok. :) Then I asked her about the paci muir valve again. I told her I didn't understand how Sophia was suppose to learn to breathe a new way if we weren't able to try it at home.  She said, well I won't quote her for sake of well for the sake of others, that I more or less stated she thinks I am very INTELLIGENT and capable and she agreed with me 100%. She said I will right you orders to try the valve at home under monitors and to try it as Sophia tolerates. SCORE for the parent with the brains. haha. So when the supplies come in, I will be able to work with her on the paci muir valve. I am eager, but cautious. I know there are risks and the rewards may take time but I am just so thrilled to be able to let her try. You never know until you TRY. And, if you haven't caught on already. NO OXYGEN DURING THE DAY MEANS MORE FREEDOM. We now only have to put her on an HME ( a small piece over her trach ) when we travel about. We can use it at home too. Sometimes, we may hook her up to humidity through a trach collar as needed for flow. I can now carry her around my whole house as I want. We are no longer restricted by time for worry of the oxygen running out. I no longer have to carry a 15lb battery and vent and hoses or oxygen tubes with me at all time. Granted, I still have a suction machine, emergency O2, emergency kit, and monitor BUT I'll take it. Those things I don't foresee ever being without. What a VICTORY! Sophia you are a rock star. Mommy and Daddy knew you were ready for the next step and we are so happy to be walking this path with you. 

It's been...

It's been 6 crazy, amazing, heart-wrenching, hopefully miraculous emotionally splendid, beautiful months. When I look back to December and all the days we spent in the hospital, I can not for the life of me ever stop my eyes from swelling with tears, I can't stop the lump in my throat or the flutter in my heart. When I look back on where Sophia was at birth and where she was after each surgery and to where she is now, I can't stop the smile from spreading to each corner of my mouth and I cannot stop thanking the Lord for the miracle I get to see in her daily. This past 6 months has been a whirl wind of emotional ups and downs. The downs are low and the ups well nothing gets better than the UPS. It hard to think that for half of 2012 we have been in the hospital and in and out of doctors visits. However, the optimist in me is anxious to see what the second half of 2012 has in store for my family. 

It's been a few blurry, hurried, busy, exciting, longing couple of weeks. I feel like I haven't even had time to sit and breathe the last couple of weeks. I know my husband feels the same. He works his butt off for his girls. Since coming back from the hospital, we have been playing catch up with our time and days. We have had a few more doctors visits. People (professionals) dropping by the house to check on Sophia and her equipment. Reordering medical supplies and medicines. Catching up on the usual day to days. I was so afraid that being back in the hospital for a week was going to set Sophia back in her development. To my surprise, it has had the opposite affect of Sophie. I swear in the past week or two, she has learned to hold her head up, steady herself better and...wait for it...she can sit independently for a few minutes at a time now. This is HUGE people. What an accomplishment. I can't wait to see our physical therapist mouth hit the floor Tuesday when she comes back from vacation. She is going to be soooo HAPPY. We have been working hard:) GAH I just love her. I get so excited for her and clapping and dance and she just smiles at me. She knows she doing good to. I have even seen huge differences in her personality. She is a character. She is nonverbal but her facial expression speak volumes. The other night she was laughing and just giddy. She had the whole room rolling in laughter. She really is the best baby. 

It's been a expected, unwanted, disappointing, knowing, wondering, needing more couple of days. We had our appointment with Speech Thursday to discuss the paci muir valve. What a loong night that ended up being. We took the last appointment of the day so we could get in a few months earlier. I was eager for the appointment. Hopeful for the appointment. Not surprised by the appointment. Sometime you want something so bad and in the back of your mind you know your wanting too much. We were the lasts one in and the lady came in to discuss what the valve does and how it works. The way it works is it allows her to breathe in through her trach but NOT exhale through it. She then is forced to move air past her vocal cords and out her nose or mouth. This is a completely new way to breathe for her. Considering she has now learned to breathe solely through the trach. When we tried it the first time, she looked panicked like she couldn't breathe and the shot a bunch of snot out of her nose. We took the valve off and then suctioned her clean. Then to add to things, our suction machine broke. As you all may know, I suction Sophia all day everyday. Its all I get done. We could not do anything further until we had suction. Luckily, we were in a doctors office however we can not drive home without suction in the car. So that put a halt on our visit. I had to call the company to replace the machine but they couldn't get to us for 5-6 hours. So thankfully, my sister came to the rescue. Kelsey is always clutch. She drove to Riley to bring our backup suction from home. The company came to our house the next day to replace the broken machine. Once we had the suction sorted out, we attempted the valve again. This time she didn't seem as panicked and was releasing some air through her nose, but not enough. All of a sound she exhaled a deep breath and shot the valve straight off her trach and across the room. The speech therapist said at this time she doesn't feel Sophia is ready for the valve and we would discuss it more at our next pulmonary visit. Ugh. I had a feeling. I didn't except her to start making sound right away, but I was hopefully hopeful. It is a little disappointing and heartaching to know we have to continue to wait. I completely understand the need to timing and safety but I still have questions. I guess those will be answered at our next visit. Thank goodness that is next week. This month we officially, have our vision and hearing screens as well. We also will be visiting cardiology this month to follow up on her heart issues. 

(God, to hear that Laugh...)

It's been exhausting, It's been up-lifting, it's been surprising and its been expected. It's been wonderful, its been loving, its been joyous.  Its been a PROUD few days. I have to say all in all these past couple of weeks/days have been surprisingly great. A few steps forward..wait many steps forward and a few hold ups for the moment.