Thursday, August 30, 2012

New Lung info.

We had a special lung appointment on Tuesday. We found out a lot of things that we did not know about Emri. First, she is primarily breathing with her right lung-her left lung only inflates with big deep breaths. The reason for her left lung insufficiency is that, with the severity of her pectus, there is not enough room for the left lung to function properly. The doctor we saw specializes in pectus deformities and scoliosis and how they interfere with the lungs. He highly suggested an early pectus repair which is very rare in young children since they are still growing---if you fix the pectus, and they keep growing, it is not going to stay fixed. He also warned us that with the way Emri is put together, and with the small space inside her chest cavity, we need to be prepared for the worst if she gets sick. There is a high possibility of her lungs collapsing if they are at all compromised by a sickness, especially her left one. This is why it is so important that Emri remains healthy, and that when she does get sick, she is taken to her pediatrician right away. This is a lot to swallow-especially since lungs have not yet been an issue for Emri. It's a whole new system to be aware of and keep up with. Pray for peace and patience for us as we search for the right people to take on this new pectus repair-they are a rare commodity, since kids usually don't need this type of surgery so young. We are also attempting to ready ourselves for Emri's G-tube and spine surgery all coming up very quickly. We have been spending and will continue to spend much of our time seeing specialists at Children's throughout these next few months. It's a lot to handle, and we are continually reminding ourselves that we are not alone...sometimes it just feels like too much.

Saturday, August 18, 2012

The newest.

Today is the day that I finally received adequate motivation to write this blog. I am bursting at the seams with new information on Emri, but have just feeling a little too overwhelmed to share it all. Let's start with the fun stuff.
At the end of July we had a magnificent weekend in Ocean Shores with Brett's parents, brothers, sister-in-laws, and all of the kids. It was a relaxing, beautiful and fulfilling weekend spent catching up and watching the  cousins interact with each other. The kids all had so much fun on the beach and even thoroughly enjoyed the frigid waters of the Washington coast. 

We returned home on Sunday, July 29th, and left for the Chicago Marfan Conference on Wednesday, August 1st. In between our trips Emri decided it would be a great idea to pick up a double ear infection to prepare herself for the flight to Chicago. The poor girl had a lot of ear pain on the landings, but did spectacular on the actual flights. On the second takeoff, Brett decided to pretend we were on a roller coaster and told Emri to put her hands in the air as we started speeding down the runway. Emri quite gleefully obliged and turned to me to make sure I was going to join in on the fun. I can't help but laugh when I think about how ridiculous we must have looked, hands straight up in the air and cheesy smiles spread across our faces...:)  A good memory though.
We landed in Chicago at 2am and took a taxi to our hotel. After a series of unfortunate events, we ended up switching hotels at 4 in the morning. We slept in the next morning and headed off to get Emri's echocardiogram in the afternoon. Emri slept through the echo, which is always ideal, and woke up to tour around the city with us for a bit. That night we had the unique privilege of meeting a few families that we have only ever corresponded online with. It was a special few hours of watching Emri meet and interact with kids that have endured many of the same trials that she has. These are kids that hopefully in the coming years, Emri will be able to correspond with and get to know closely through conferences. 

Friday was the day of appointments. We first met with Dr. Lacro, a well-known pediatric cardiologist that practices in Boston. It was a privilege to meet with him and have him read Emri's echo, as we have heard so many good things about him. We learned through him that Emri's mitral valve leakage has moved from mild to moderate and that he aortic root did indeed grow from the last echo. This was news I didn't necessarily expect to hear, but wasn't astonished by either. Next, we waited for Dr. Dietz to come in and tell us his thoughts on the situation. Dr. Dietz was once again, wonderful and gracious to us. We went through our list of questions and he took the time to thoroughly answer all of them. It was slightly humorous because while we were deep in discussion, Emri had taken on the project of covering as much surface area of her legs with pen as she could, and Dr. Dietz was overly concerned about it. I remember asking him a question about Emri's aorta and his response being "well, at the moment, I am more concerned about Emri and her pen..." I thought it was so funny how pre-occupied he was with Emri and her choice of activity. It lightened the mood a bit despite the heaviness of the conversation. In the end, Dr. Dietz is definitely concerned about the fact that despite the serious dose of irbesartan Emri is on, her heart is still growing larger and leaking more. He made it apparent that instead of seeing him again in 9 months, like we have been doing, it is necessary that we come back within 6 months. 

We saw Emri's orthopedic surgeon, Dr. Sponseller after Dietz. He looked at Emri's curve and took a peek at her previous x-rays and decided that Emri really needs a growing rod as soon as possible. We asked him, since we are due with #2 in November, if December would be too late, and he said that he felt comfortable waiting till then. Dr. Sponseller's main concern with Emri's spine surgery is her weight. Emri has been floating right around 18-19 lbs now for way too long. SO because of all this, the choice has been made to surgically insert a g-tube into Emri's stomach to prepare her for this big spine surgery. We now have a g-tube surgery scheduled at Seattle Children's for September 20th, a Spine surgery scheduled at Johns Hopkins for December 19th...oh yeah-and a baby due November 4th. Should be a note-worthy few months for us. 
The rest of the conference consisted of gleaning lots of new research and information, building valuable relationships, and experiencing the city of Chicago. 

All in all the conference was a helpful, supportive and challenging experience. It has been difficult for us at both conferences, however, seeing Emri fit into the category of "most severe." Although oblivious to the fact, Emri has a lot on her plate as a 2-year-old. It's hard for me, as a mother, thinking about the day Emri begins realizing her differences and asking questions. My love for her is beyond deep-and although I know it is not the correct solution, I continually get the overwhelming urge to protect her from everyone and everything. 

I have been reflecting frequently on what it means to have a child with a "disability" and the implications it has on our life as parents with a "disabled" child. Emri is "disabled" because she is legally blind, has far below normal muscle tone and severe kyphoscoliosis which interfere with her gross moter development and cause a need for bracing and assistance from a walker. She takes life-saving medication daily and will soon rely on a g-tube for adequate caloric intake. Emri is anything but average. Emri loves reading books. She will sit and listen to book after book after book. She is starting to be able to recite sections of her favorite books which to me as her mother is beyond amazing. Emri is counting to 10, quite consistently skipping number 7...why? Nobody knows. Makes me laugh every time. She is already quite a little artist and loves to color. I have never seen a child crawl quite as fast as Emri. If she wants to get somewhere, she does so as quickly as possible on her calloused little knees. Emri has such an endearing, sweet personality and melts my heart daily. She is truly one of a kind. Because of all the blessings Emri brings to my life, it is difficult to dame her in my mind as disabled, or what some would call "less than". 
It is interesting, also, to see societies reaction to my "disabled" child. Emri is viewed by society in all sorts of different ways. As her mom, I get questions about Emri from anyone and everyone ranging from "how old is she?" to "what's wrong with her?"  I have received various inquiries about why she needs glasses, why her back looks so bad, why she can't walk yet, why she looks like an old person, why her fingers are abnormally long, and the list could go on. At children's hospital Emri is a unique case, and somewhat of an experiment
As her mother, I have been overtaken with a life unexpected to say in the least. While pregnant with Emri, the types of thoughts that ran through my head revolved around birthday parties, school, sports, and what kind of a mom I wanted to be to my children. I could have never imagined that my mind would actually be racing with thoughts of keeping my daughter alive, getting proper treatment for her ailments, and how in the world I was going to take my child's refrigerated medication everywhere I went. Processing this change in expectations and desires has been nothing less than painful. I have heard the phrase "death of a dream" used to explain the types of feelings parents sort through when God gives them a child with more than just the basic needs. It's true, it is the death of a dream...a dream that the world has made every parent believe is "ideal", and "the best." When your dream of dad, mom, and three glowing, healthy children becomes anything but that, how do you react?  
Sometimes the easiest way to cope with all of this is to shut out everyone and everything around me and become slightly calloused. Yes, like I have said many times before, I truly believe that this is all in God's plan, that he has had Emri's days numbered from before she came into being...and I have to accept and welcome that. Although I believe this, I have come to recognize that throughout this journey with Emri, my view of God has become relatively dry. I have been focusing so much on HIS will being done in Emri's life and my acceptance of it, that I forget that, as a believer, God is FOR me, and that he is working for my GOOD. 
I attended a Chris Tomlin concert last Thursday night at the Lynden fair. Right after I was thinking it, Chris started talking about how music has a way of breaking down walls in our hearts, and softening us. I felt that process taking place as I soaked in the words sang that night. It was a night of focusing on God's goodness, His love for His believers, His mercy and so much more. Sometimes it is important to remember that while going through tough situations, God doesn't expect us to accept it all without tears, and without falling into his arms for comfort. His arms are a "fortress for the weak." I need to be willing to be weak...willing to admit I am struggling and fighting for a breath. 
This is all the process of putting that initial "dream" to rest. Realizing that God has a different dream for our family, and believing that it is all working for our good as His believers. We are in the process of welcoming a new dream...a just-as-brilliant but different-than-we-thought dream. It will never be easy seeing Emri suffer, and struggle through things that we can't possibly understand...but God is good, and merciful, and gracious, and he loves Emri. He has a plan for her, for us as her parents, for her future brothers and sisters...It's our new dream for our that we can expect to be filled with pain, unpredictability, blessings, lessons... and so much different than we ever expected. 
If our family has found it's way into your prayers, thank you so much. God is continually working in our lives,  revealing himself to us in all sorts of different ways. We are a blessed family, to have been called by such a merciful, sovereign be His children. What would life be without His promises for us?