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 family...one 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 savior...to be His children. What would life be without His promises for us?  

Today's appointments among other things...

Well lets start with today. Today was...draining. We arrived at Children's for what we were told (by a scheduler) was an appointment to insert an NG tube. We weighed Emri...she lost weight...big surprise. We waited for a good 20 minutes before a GI counselor came in to simply "council" us on our feeding tube options. We learned quite quickly that we were not in fact receiving the actual tube and that the process itself includes an all night stay in the hospital. SO, after working myself up for a good two weeks thinking this would be the day that we shoved a tube up Emri's nose and down her throat...nothing happened. Instead of Emri leaving the GI clinic screaming and thrashing about as I imagined, she left grinning sheepishly, stickers in hand. You might think that this would make Brett and I happy, which in some ways, it did...but we both feel like this process has dragged on long enough. We have dealt with an arrogant, assuming, non-kid-friendly GI doctor, scheduling mishaps, and way too many mis-communications between doctors and coordinators and we are ready to move on and get this all over with. The GI counselor very appropriately used the phrase "too many cooks in the kitchen". After learning more about the processes of "tube installation" and recalling the discussions with other marf moms that I have talked to about their experiences, Brett and I made the decision to skip the whole NG tube trial, and go straight to the G tube. If you do not know the difference, an NG tube goes up the nose and down the throat, and a G tube is inserted directly into the stomach. In the next few weeks we will be spending 1-3 nights at Children's getting this whole process over with. The hope with putting in this tube is that Emri is able to gain an appropriate amount of weight before her spine surgery coming up in the next while.


With that, lets move to Emri's spine x-rays.After waiting over an hour for the pediatric orthopedist to grace us with his presence, we finally learned that Emri's scoliosis curve remained nearly the same as 3 months ago. Unexpected? Yes. Good? Yes. Confusing? Slightly. We will be going over this all with Emri's orthopedic surgeon at the Marfan conference in August and will be scheduling the surgery based on that consultation. We have already informed him of the fact that we are due for baby #2 in November and he said he would most definitely work around that...so kind:) We have no idea what Emri's kyphosis (Bump sticking out on her back) curve is looking like as they "forgot" to get an x-ray of it today...so we will not know for sure if that curve has gotten any worse until the next set of x-rays.


Later this month Emri will be completing a lung function test in which she is sedated and put in a box to read all of her "lung levels". This is to get a base line read of how her lungs are functioning before surgery. Then after surgery we will need to repeat the test and see if there is any difference in function. Our pulmonologist thought that Emri's breathing has sped up a bit as of late, which could potentially be a sign of lung restriction due to her pectus and/or kyphoscoliosis. Got a call from a nurse the other day warning me that the drugs they use to sedate these kids tends to make them irritable, grouchy and off-balance for a few DAYS...should be fun!


That's most of the medical news for now. I apologize, only mostly sincerely, for my perhaps less than sunny disposition tonight, but I must admit that having the "correct" attitude has been nothing less than a struggle for me lately. I have thought more times than once about how much I would love to just whisk Emri away for while and not let anyone touch her. No poking, no evaluating, no bracing, no therapy, nothing...just for a while. A break from it all. I just want her to get to be a kid---laugh, play, be free... you know??? But when I do the whole "step back and look" thing, I know that all of these people poking, prodding, and consistently evaluating Emri are doing it for her good...and without them, Emri may not even be here. I need to focus on that. I need to remember that this is God's will for her life and that she is a strong, brave little girl, always smiling, always loving life and always inspiring others...including me. These are the things I must think about. We are blessed beyond anything I could ever imagine to be Emri's parents...but just like any parent who loves their child with everything they have...I just can't stand to see her suffer.



I know another mom that knows more about watching her child suffer than I do! Danny, our brave little Marfan friend in the UK recently survived major heart surgery! Not only did he survive it, but a week after the surgery he was home and thriving! What a trooper! This kid and his mom are a constant inspiration to me. We are SO happy that Danny is doing exceptionally well, proving so many doctors wrong! We are very much looking forward to finally meeting him and his mom at the conference in a few short weeks!!



I am now 23 weeks pregnant with baby number two and if you haven't already heard it is a BOY!!! We were thrilled to find out a few weeks ago that we are in fact expecting a baby brother for Emri. I am feeling awesome compared to my last pregnancy and can't wait to meet this munchkin in October or November. I think Emri may understand more than we know. Every time I pull up my shirt and tell her to kiss her little brother, she politely says "NO!!!" and pulls my shirt back down as quickly as she can manage. It will for sure be an interesting experience to see Emri adjust to her big sister role:)






The last bit of news I wanted to share, most of you already know. On June 8, our sweet little Emri turned 2. This was not just any birthday...this was the birthday we were told she had a 5% chance of making it to!!! It was a tad bit surreal thinking about the journey we have been on with Emri in the past two years. Remembering the day Emri was born, and all of the events in between then and now...seeing how God has shaped and molded us as a family. Nothing could have prepared Brett and I for the experiences Emri would bring us, but God was and continues to be with us every step of the way, always reminding us that HIS will be done. I made a little birthday video that if you have facebook, you most likely have already seen. But for anyone who missed it, here is the link: http://www.youtube.com/watch?v=qkJaWTrez68&feature=youtube_gdata 





I am guessing the next blog will be after our much anticipated Marfan conference in Chicago. We leave August 1 and return August 7. Prayers for safety for us and all of those traveling to get to this conference would be so appreciated! Thank you to all who continue to write messages of encouragement, and to those who have always kept us in their prayers. It means more than you know. Love from here to there, Erin. 

So many things...

Ok folks, we have a lot to cover in one blog! Let's start with the Marfan Awareness event that successfully occurred on March 10th. God not only allowed us to reach our monetary goal of $3,500 for Danny, he allowed us to surpass it! We made a grand total of $5,700 from donations and auction items the night of the event and the number just keeps growing. There have been individuals who heard about the event that have been donating after the fact, and other marfan moms who have been raising funds for Danny and his mom Sarah as well. Sarah has thanked us over and over for everything that has gone on, but it is really all of you, the "donatees", if you will, that we have to thank! THANK YOU! Thank you for coming to the event and/or donating money, time and prayers for this event. It has meant more than you know seeing so many people showing so much support for something very important to us, as parents of a child with Marfan Syndrome. We are SO excited that we will be able to meet Sarah and Danny IN PERSON this summer at the Marfan conference in Chicago. Danny will get to meet and be thoroughly examined by all the "big" marfan doctors and his mom will be able to leave knowing she has seen and received care and advice from the very best! I know that this means the world to Sarah and we could not have done it without all of the support we have received throughout the past few weeks.

Brett and I had the opportunity at the event to share Emri's story so far. It felt good to be able to tell it and have it well-received by many. It was intimidating indeed to stand in front of 150+ people and share many intimate details of our life and journey with Emri, but we pray that God used it and continues to use it for his good purpose. There have been many individuals that have responded to Emri's story saying things such as, "wow, I had no idea it was that serious," or "I can't believe all the things you have been through with Emri!" To be quite honest, I felt a little relief being able to let the world know what it takes to be the parent of a child like Emri, but I also want to respond to these comments with how I really feel being Emri's mommy. I can't imagine having a more precious child. I feel more blessed than I ever have in my life to be the mother of a child so unique, so sweet, smart, and incredibly funny. That girl puts more smiles on my face in a day than I can count and I truly, honestly wouldn't want it any other way. Yes, there is a lot of responsibility involved and tough, tough days that we go through together, but I wouldn't trade a second of it. I know that everything Emri has already been through in her 21 months is shaping her into the beautiful person God intended her to be. I feel like kids that go through trials early on in their life already, learn even faster that life is a precious gift, not to be taken for granted. Emri has already been called a "wise old soul" by many, some she only just met. She is so special, such a blessing from God.
Before I move on to the next subject there is an apology I must make to everyone who drank coffee at the marfan event... Those of you who thought they were drinking decaff...you weren't. Those of you who wanted your kick of caffine...sorry- whether or not you thought you got it, you didn't. Yes, it's true-the signs were switched. Shortly after the event, while cleaning up, the gruesome discovery was made. I am not going to lie, we all shared a good laugh thinking about all the poor people that would be laying awake all night thinking they were just "over-inspired" by the whole event...I do apologize.


The next subject of matter is Emri's spine. We had a spine appointment for Emri on Thursday to check in on her kyphoscoliosis (scoliosis and kyphosis) measurements. As it turns out, they are in surgery range already. Emri's lower scoliosis curve is now beyond 50% without traction (stretching Emri for the x-ray) and 48% with traction. He kyphosis degree is still beyond 60. These numbers have grown big enough to talk surgery dates. Surgery will be in Baltimore by Dr. Sponseller. We have seen Sponseller a number of times already and have known that he would be the one surgically repairing Emri's spine when the time came. Well, the time has come. I can't say I'm trilled about it, but I know it has to be done. The big concern that lies before surgery is Emri's weight. She is still under 20 lbs at 18.7. Her appetite has been increasing as of late, so we are hoping we can completely avoid a g-tube (tube inserted directly into the stomach for feeding) before surgery. We want Emri as big and fat as possible before undergoing a serious surgery. We also want to grow Emri up as much as possible before surgery because following the initial rod implantation, Emri will need a lengthening surgery every 9 months to a year in order to have the rod grow with her spine. Anesthesia given that often isn't great for kids and we want as little of it as possible. So all that being said, we are anticipating a surgery in the next 6-9 months...although it could be sooner if we decide to combine the conference and surgery in one trip. Please pray for this.


Emri has had a heart appointment since last blog as well, and we are grateful to announce that the aorta and mitral valve prolapse has remained quite stable. This is something that we are extremely thankful for...we would really like to focus on one surgery at a time!!!




The last order of business is that...........

EMRI IS GOING TO BE A BIG SISTER!!! Yes, it's true! We found out we were expecting on Feb. 22nd and we are beyond thrilled to be able to make this announcement:) Emri doesn't quite get it yet but is correlating the word "baby" with the idea of mommy being sick. I am, once again, very sick with "morning" (haha) sickness. It lasts all day and doesn't allow room for much fun in Emri's world. Emri has watched a few more movies than I would like to admit to, but it is truly the only way to get through the day for me sometimes...poor kid! We are due November 4th, which puts me at about 7 weeks along. I would like to mention that although I was already pregnant at the event, I was NOT sick. God spared me until the very next day! THANK YOU GOD! We are very much looking forward to meeting our little sweet potato next fall and will keep you updated with lots of ultra sound pictures! We even get a 3D ultra sound this time!


So, there you have it-all the news you can handle:) Thank you once again to all those who helped with the event, supported it financially, and to those who prayed for it's success. We felt God's blessing on the event throughout the entire process. If you have already been prayerfully supporting our family...thank you. If you are interested in beginning to pray for our family, thank you also. God takes such good care of us and we are so thankful to feel so supported by so many. I hope this blog finds you all well...until next time...~Erin~

So, since the last blog, we have joined the ranks of hospital dwellers. I am sure there are a lot of you that don't know exactly what is going on with Emri, and to be quite honest, neither do we. We do have a few answers as to what is NOT going on however. 
About 3 and 1/2 weeks ago on Sunday afternoon Emri began her vomiting marathon. Within the last 3 and 1/2 weeks, there were maybe 5 days where vomit remained unseen. We visited her pediatrician 3 times before he heard the "rubbing" sound in her heart and sent us to Childrens for an emergency echocardiogram. Thankfully, the echo showed no new mitral valve regurgitation and an aortic measurement consistent with what we had seen on her last echo. We were sent home relieved and determined to get her feeling better. Unfortunately the vomiting continued and the consistent lethargy became more evident. After 2 or 3more trips to the pediatrician we were still left with no answers as to the cause behind Emri's apparent sickness. Yesterday morning Emri couldn't keep anything in her poor little stomach and I took her in immediately. Emri was sent to Saint Josephs Hospital for fluids, monitoring and blood work in an attempt to locate the vomit monster. All day yesterday Emri did not lift her head from my shoulder---no sly grins, giggles, or mischievous moves all day long. It was very troubling for me to see her this way. Eventually they took blood, got an IV in her foot and started fluids. So far, there have been no giant red flags that have come back in her bloodwork signalling something dangerous or concerning. All the high/low counts have most likely been due to severe dehydration. Today, the doctor wanted to get an upper GI tract study. The study was to see if there was what's called an "eventration hernia" making her vomit. This is a common occurrence in the marfan world and would need to be fixed with a surgical procedure. This was the worst experience in my/Emri's life yet. She had to have a feeding tube put in in order to drip the barium into her stomach so that the doctor could see her stomach and intestines. Needless to say the feeding tube was "slightly" disturbing for Emri. She screamed for a good half hour waiting to be taken to the x-ray room. We made it to the x-ray room where Emri proceeded to choke on her own vomit and scream hysterically while the "people in blue" got he x-rays they needed. It was absolutely heartbreaking. It is the most helpless feeling in the world looking at Emri, knowing that she is just longing for me to save her from the pain she is experiencing. My poor sweet little girl has had far too many similar experiences for being alive a meager 1.5 years. Thankfully, at 18 months, Emri quickly moved on from her horrifying experience while I sit here, still struggling to hold back tears and frustration. The doctor came in about 45 minutes ago to confirm that there is, indeed, no hernia or gastrointestinal swelling. We are grateful for this news, but still anxiously awaiting answers. The next step was going to be a CT scan to make sure the brain was ok, but since Emri is acting more like herself today (teasing grandpa, kissing tinkerbell stickers, and smiling ceaselessly) the doctor dubbed it as unnecessary. As of right now, the plan is to start Emri on some reflux meds, keep her on fluids through the night, take more blood in the morning, and if everything checks out normal and Emri seems herself, we will be heading back home tomorrow sometime. We would really appreciate prayers for our sweet, brave little Emri. 
I am sitting here in the hospital knowing that we are not the only ones experiencing hurt due to marfans. There are many close marfan friends in hospitals all over the place that are going through more pain than we know. We are especially thinking of Ethan as he is on day 20 in the hospital with complicated lung issues and a tracheotomy done yesterday. The hurt still flows deep with the strong moms that have lost their precious kids to marfans. I truly can't imagine. 
It is so hard to see Emri struggle, harder than I could have ever pictured it to be. It's a struggle not to question God in times such as these; seeing someone I love so much go through so much pain. As I typed that last sentence I was struck with the reminder that God himself, watched his Son suffer and die...for sinners...for me. I am praying that despite my natural sinful tendencies I am able to count this all joy, and trust in the Lord with all my heart, not leaning on my own understanding. Through these tears of pain, I am remembering to thank God for giving me the joy of being Emri's mother, despite the hard times. There is not one other object or individual in this world that can make me smile as big, or laugh as much as my little girl. I have never struggled so much as now, but I also have never been happier. She lights up my life in an unspeakable way. Thank you God for blessing me with such a beautiful child and for loving your sinners enough to watch your own son die on a cross. 

Life....throws a few curves, don't it? I'm angry. I'm heartbroken, devastated, anxious, and to be quite honest-just hate marfans.  Klaire, a precious little 9 month old, passed away Saturday night from complications due to marfans. She left her dad, mom and sweet twin sister behind, who, I just found out, was admitted into the hospital yesterday because of marfans. Ethan, an intelligent, brave 9-year old with marfans that we met at July's conference, is in the hospital with a collapsed lung, not breathing on his own, and is now dealing with many painful complications from it...all because of marfans. This is only 1 out of many, many hospitalizations that Ethan has already experienced in his 9 years of life. Because of marfans, little Brody bear is recovering from his first open heart surgery at the fragile age of 1. Danny, our other handsome little one year old from the UK has spent his share of time in the hospital as well because of...you guessed it---marfans. 
I desire with all of my heart to have just one day where I don't have to think of or hear the word "marfans". But that's not going to happen...ever again. I am going to continue hearing it ring loud and clear at doctors appointments, in phone conversations, in emails, on facebook, and in my own head. I will continue to see this destructive disease hurt, crush, and devastate people I love and care about. And I will see it manifest itself day after day in my sweet Emri's fragile little body. I really hate marfans. It's tough to keep a sunny outlook on life and maintain a good attitude while all of this is taking place. There are so many individuals so close to my heart that are all in pain; physical and emotional; because of this ugly disease. As of late, trusting in God's goodness has been a struggle to say in the least. I am overcome by grief, fear, and bitterness.
I often find myself tearing up when looking at a healthy, glowing child, just wishing and yearning that Emri could have that. I struggle seeing pictures of a newborn baby being cuddled close by his/her mom and just can't help but remember the horrifying events that took place after Emri was born. I didn't get that "perfect" birth experience. Not even close. Emri didn't cry when she was born because she wasn't breathing. Brett didn't get to cut the cord because they were so rushed to resuscitate Emri...because of marfans. It feels unfair. It feels cruel. But it's life. It's in HIS plan. 
As a mother with a child with marfans, I am struggling. I am hurting with all the rest of my special moms. I am praying that God gives us a break and lets us breathe. That He grants us His peace and gives us grace. That despite all of the horrifying events taking place, we can all lean on him and put our trust in His plan for our lives.  
Please pray for these precious kids and their brave mommies..

Cassie

Danny

Ethan

Jonah S.

Brody

Jordyn

Julian

Klaire and her twin sister Kylah

Our angel, Klaire

Our angel, Jonah

     

Baltimore...round 2

Yesterday, around 2pm, Brett, Emri and I arrived back, safe and sound in our new home. My wonderful parents took Emri to play at their house while Brett and I slept for a good 6 or 7 hours. THANK goodness. I don't think I have felt that tired since the week Emri was born. 
Baltimore was....lets just say "different" this time around. Emri is of course older and declaring her independence...loudly. The plane ride to Baltimore proved to be somewhat trying this time. Emri doesn't exactly sit still anymore and vocalized her discontent in a rather intolerable fashion. By the time we arrived in Baltimore, Brett and I were exhausted and ready for bed. Emri, however, was filled with joy to be off the plane, putting a halt to our dreams of crawling into bed and drifting off into a beautiful night of much-needed sleep. 
We did eventually get sleep and woke up to enjoy our Saturday. We walked around Baltimore, enjoying the 40 degree difference in weather since the last time we visited. We saw Baltimore's terrific aquarium and enjoyed the first 45 minutes of it until our little munchkin decided she had seen enough. 
Sunday we gave ourselves plenty of rest and spent time exploring the city some more. We did a lot of walking  both days-I think enough walking to burn off the 3 or 4 pumpkin spice lattes I consumed within them:) 
Monday was Em's spine x-ray and consultation with Dr. Sponseller. The x-ray was disappointing yet not unexpected. Emri's spine is getting worse very fast. Fast enough that she will soon be a very young recipient of a spinal rod. We were really hoping that we could grow Emri up a lot more before she had spinal surgery, but her scoliosis is telling us otherwise. Emri's scoliosis has gone from 13 degrees last January, to 26 degrees in July, and is now at 36 degrees 3 months later. That's only speaking of her scoliosis. Her Kyphosis measurement (the bump on her back where her spine bows out) is now at 63 degrees. 
We were less than thrilled with the news that Emri will most likely need a spinal rod around 3 years old, or perhaps even before, depending on how her spine progresses. Consequently, our next trip to Baltimore will be for her surgery. The surgery includes attaching a rod along the entire spine with screws and bolts and lengthening it yearly as she grows. This means Emri will be for sure having a once-a-year surgery until she stops growing. 

Tuesday was the echo-cardiogram and metting with Dr. Dietz. The echo showed that Emri's aortic root had expanded a couple millimeters. This, also, was not the news we we hoping to hear. We are thankful that it wasn't a large growth, but we were really hoping that the losartan would completely stop the growth of the aorta. The good news is that the mitral valve prolapse is still minimal and we hope it stays that way! 
We met with Doctor Dietz to discuss all of our and his concerns and it was, of course, very informative. Because of the aortic growth, Dietz changed Emri's losartan to irbesartan. This is a medication in the same class as losartan but can be given in higher doses. Dietz has given it to many marfan patients where losartan hasn't quite done the trick. We will be switching to irbesartan soon and taking it along with atenolol (blood pressure lowering medication) in hopes that Emri's heart will stabilize and completely stop growing! 
There's the news folks. It was a tough trip. Not only because we didn't get great news, but also because there is so much going on in our marfan world right now. Emri's friend Brody was in the hospital with pneumonia and is now in heart failure. Brody and his parents are flying to Hopkins tomorrow and Brody will have emergency valve repair surgery on Monday. Brody is a couple months younger than Emri, so heart surgery sounds a little intimidating to his mom and dad. 
Klaire and Kylah, a pair of twins less than a year old with marfan syndrome, are also in heart failure and will need surgery very soon. 
Danny, our little friend from the UK, just underwent glaucoma surgery and is dealing with a plethora of health concerns due to his infantile marfans. 
Ashley Hood, a mom I am friends with on facebook, just last week, lost her 7-month-old son who also had marfans. I can't even imagine. 
Needless to say, there is a lot of stress, sadness, and heartache that I read about daily in my conversations with my wonderful group of marfan moms. 

It has been difficult to keep my head up lately. The only way I can is when Christ gives me the strength. Satan has been working hard lately to destroy any feelings of peace or contentment I may have. I have been feeling a little angry, utterly defeated, and completely overwhelmed by everything going on. I am praying for peace, energy and help in trusting that God is working for our good. I have been thinking on a verse I read right before our trip to Baltimore. It is Hebrews 4:14-16. 


"Since then we have a great high priest who has passed through the heavens, Jesus, the Son of God, let us hold fast our confession. For we do not have a high priest who is unable to sympathize with our weaknesses, but one who in every respect has been tempted as we are, yet without sin. Let us then with confidence draw near to the throne of grace, that we may receive mercy and find grace to help in time of need." 


Beautiful, yes? 
Jesus went through it all. God GAVE up his only Son. He gets it. 


Here's another, more familiar verse that I've been dwelling on. James 1:2-4


"Count it all joy, my brothers, when you meet trials of various kinds, for you know that testing of your faith produces steadfastness. And let steadfastness have it's full effect, that you may be perfect and complete, lacking nothing."


I can't, on my own, choose to be joyful in these kind of situations, but I can pray that God grants me His grace and gives me the ability to be joyful through it all, knowing that in the end, steadfastness will come. 


I love my little Emri more than I can possibly explain. She brings an immeasurable amount of joy to my life daily. It hurts so bad to know how much she will have to overcome and defeat, even in the first few years of her little life. But I know what to cling to, and what to pray for in order to be content and receive joy through it all. You can bet that this is what I will be teaching Emri as she grows and questions the trials she is facing. I thank God for his promise to grant us mercy in our time of need...if only we are willing to draw near to the throne of grace. 

Spine update

I know I said a long time ago on facebook that I would update you all on Emri's lung and spine appointments and I am sorry it has taken this long! This whole "fixing the house" thing is taking a lot out of us! We are, however, getting close to move in day! Hopefully by the end of this month or beginning of next we will finally be IN OUR HOUSE! We are very excited for this and I can't wait to post before/after pics:)

As for Emri's appointments-they were a little bitter-sweet this time, perhaps a little more bitter for me. We had a Pulmonologist appointment that went smoothly. Since Emri's sleep apnea has cleared up we only need a precautionary lung check up every 6 months. Emri's lungs sounded healthy and strong and we are so thankful for this. There are a lot of individuals with marfans that deal with lung issues, but so far, Emri's have remained quite healthy. Emri's spine however did not look near as promising. I, as her mom, had a feeling that it had gotten worse, but I wasn't expecting it to have gotten as bad as it has. Emri's scoliosis went from 13 degrees 6 months ago to 26 degrees now. That is a big, scary jump. We know that a spinal rod is in Emri's future, but we were/are hoping that we can put off surgery until her lungs are more fully developed because we do NOT want to interfere with lung growth. The orthopedic surgeon in Seattle does not want to interfere with Emri's physical development by having her wear a brace. There isn't a lot of proof that a brace really works anyway. We are still thinking about weather or not to try it out and we are waiting until our next trip to Baltimore (October 21-26) to make a decision.

Other than all of that, we are doing well-Emri is happy and developing QUITE the sense of humor:) She is army crawling all over the place and trying so hard to get up on her hands and knees. She has also been trying to pull herself up to a standing position-but the chicken legs give out pretty fast. She is working so hard to gain strength and doing a great job. We have an eye appointment tomorrow, so we are hoping and praying that goes well. She may need a larger prescription, but we will just have to see. Thank you for those who continue to pray for our family. God is taking very good care of us. ~erin~