Wednesday, December 28, 2011

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. 

Tuesday, December 13, 2011

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 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..



Jonah S.



Klaire and her twin sister Kylah
Our angel, Klaire
Our angel, Jonah

Thursday, October 27, 2011

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. 

Wednesday, August 24, 2011

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~

Monday, July 18, 2011

We are now Marfan Experts:)

Wow. Brett, Emri and I just returned late last night from the 30th annual National Marfan Foundation conference in Portland, Oregon. We are overloaded with information on Marfan's and overwhelmed with thankfulness for the people we met and connected with at the conference. Let me start from the beginning. We headed down to Portland Wednesday night and made it safe and sound to my Aunt's house (THANKS Aunt Hilly:) in Beaverton, OR. Thursday morning was Emri's Echo at Shriner's Hospital for Children. We had to wait till Friday morning to find out that Emri's echo is once again STABLE! Her average aortic measurement ranges from 21-24mm which the cardiologist at Shriner's informed us is the standard size of an adult aorta. Her aorta is big, but as long as it remains stable, no action is required. "Stable" has become my new favorite word! We learned at the conference that surgery becomes a reality when the aorta of a child or adult reaches 45-50mm. She still has mild mitral valve regurgitation, but it has not progressed enough to enlarge her heart which is when it becomes an issue. Dr. Cameron, the heart surgeon from Johns Hopkins, spoke at the conference all about aortic root replacement and valve sparing/replacement operations and we now understand more about the options we have if surgery ever becomes a necessity. We had the opportunity to speak face to face with many of the worlds leading doctor's and surgeon's in the realm of Marfan syndrome.

Meeting and connecting with other individuals with marfan's or those parenting a child with marfan's was probably the highlight of the entire conference. It was so refreshing for me, as a mother of a child with marfan's, to speak with other mothers that have gone through so many of the same emotions and frustrations that I have trying to attain information and find the best care for our children. Brett and I had the opportunity of connecting with parents that could let us in on the struggles of older children with marfan's and the frustrations they have with other kids and siblings because of marfan's. We became especially close to one family in particular that have Ethan, a 9-year-old boy with marfan, and Christopher, a 7-year old without it. They too had never heard of marfan's before Ethan was born. Emri and Ethan are both in the more severe catagory of marfan's which is why I feel our families connected easily. The Johns' were an amazing family and Brett and I were both inspired by their desire to make things the best they possibly can in their situation. Ethan and Christopher were very sweet brothers and some of the stories their mother told me about their love for each other melted my heart! We will be staying in contact with them and can't with to see them at next year's conference in Chicago! I also FINALLY got to meet with a person that has been a mentor for me throughout this past year-Maya. I was so excited to finally meet Maya, Mark and their handsome boys! We ran into many other people that I have been connected with throughout this past year including Alix and Traci; moms of a precious little girls that remind us so much of our little Emri. Alix organized a huge run this past year, raising over $40,000 for the NMF! It was such a blessing to finally get to hang out with and talk with these wonderful, inspiring families and individuals!

Emri made a new friend named Ashley at the conference. Ashley is a teen with marfan's and actually just two months out of heart surgery! Throughout the conference Emri and Ashley became pretty good buds. It was very fun to see them interact and know that in the future Ashley could potentially be a mentor for Emri as she gets older and has to deal with the realities of growing up with marfan's.

All in all, the conference was an absolutely amazing and eye-opening experience. I was reminded again that marfan's is nothing to take lightly. I struggled a little with seeing a lot of kids and teens that didn't necessarily have a severe form of marfan's like Emri. I have learned that there aren't many individuals with marfan's that exhibited all the signs of the disease at birth. Emri deals with so many issues already as a baby, and that is sometimes hard to swollow. Many people at the conference had a hard time even achieving an actual diagnosis because they didn't exhibit enough signs of marfan's to be officially diagnosed. Emri has it all from the long fingers and crooked back to the mitral valve prolapse and severely enlarged aortic root. As Emri's mom, I know that the road ahead will be long and bumpy and it a lot of times is painful just to think about the things Emri may be faced with as she grows older. Thank goodness we have a God who promises us that it is all for the good of those who love him. We will struggle here, in this life, but can always look forward to eternal life with HIM in heaven, where we are promised life without pain and an eternity with not one tear.

"The LORD is the everlasting God, 
   the Creator of the ends of the earth. He will not grow tired or weary, and his understanding no one can fathom. 29 He gives strength to the weary and increases the power of the weak. 30 Even youths grow tired and weary, and young men stumble and fall; 31 but those who hope in the LORD will renew their strength. They will soar on wings like eagles; they will run and not grow weary, they will walk and not be faint." Isaiah 40. 28-31

Wednesday, July 6, 2011

She's one!

Well folks, we officially have a one-year-old. She is now, according to, a full blown toddler. A TODDLER! Crazy times, these are. Looking back to a year ago June 8, words cannot begin to describe the emotions that take over. Our precious Emri Simone has brought us an overwhelming amount of joy this past year. Yes, a hefty load of stress, tears, and pain accomplanied the joy, but it only makes us that much more grateful for the beautiful gift God has granted us in Emri. Throughout the year, Emri has slowly been revealing her silly, joyful and stubborn personality to those around her. She is quite the little comedian and tremendiously social...don't know where she gets that from! We had the privilage of celebrating Emri's one year of life with friends and family in our new backyard! We are not officially moved into the house yet, but the backyard is open and ready for business! It was a fantastically sunny beautiful afternoon and we had a great time celebrating. Emri seemed to sense that the day was all about her and had no trouble at all enjoying every second of it.
We have been working vigorously trying to get the house ready to move in. Of course there was more work than previously anticipated and as a result the new tentative move in date will optomistically come at the end of July. I can't wait until I can post before/after shots of the house-it has changed pretty drastically already! Brett drives directly from his long day of work to work at the house-he has been dubbed as the energizer bunny. We are both a little overly excited to finally get into a home of our own!!
It has been a while since I have last blogged which I think can be taken as a good thing. Emri's appointments have been fewer and farther between which is a welcome relief from the previous appointment packed few months. She DID have a repeat sleep study and the results came back much better than the first test! She no longer has to be on Oxygen while she sleeps (I am not going to lie, she hasn't been on it very much do you keep a nasal canula on a one-year-old?!?!?!!?). Emri's next echo will be at the National Marfan Conference that we are attending in Portland next weekend. We are hoping and praying that the results come back as positively as last time! We are excited to attend the conference and eager to absorb as much knowledge as possible about marfan syndrome and all it accompanies. As for future appointments, Emri has a pulmonary checkup, spine traction x-ray and Orthopedic followup scheduled for August 12, and an eye appointment for August 25.
Friday, July 1st, the Lord decided to call Brett's Grandma Van Andel home. Brett's dad got to be there when she passed, which was a blessing. She will be greatly missed, but we can rejoice knowing she gave her life to Jesus Christ and is now being rewarded in Heaven!
We had a wonderful fourth of July celebrating at Fairhaven park in Bellingham. Excellent food, games and sunshine was follwed by a magnificant firework show! We wondered how Emri would react to the fireworks and she absolutely LOVED them! It was precious the way she snuggled up with Steph and I and marveled at the bright lights in the sky. I know it sounds silly, but watching Emri sitting in awe of the firworks stirred a little emotion from me. I am so very blessed and am in awe myself of the beautful life God has given me. Our sweet little girl; the one doctors once informed us had a 50% chance of making it past one; is thriving, smiling and loving life. I am so grateful and so in love.

Wednesday, April 27, 2011

21 & 301- our two favorite numbers!!

We have some very exciting things to report! Let me start with the most exciting news, which I believe most of you have already heard. Emri had her first echo last Wednesday since getting on losartan. The echo showed that Emri's aorta had not grown at all since the last echo which is just absolutely amazing. Every echo before this one has shown aortic root growth. Emri has grown 3 lbs since the last echo and her heart has remained at 21mm which is a good sign that Emri's little body is responding positively to the losartan. Therefore 21 is one of our new favorite numbers:) We couldn't be happier about this and continue to praise our God for his work in our lives and his healing power. We have had SO many ups and downs in the past year-but God has been holding us in his hand and granting us his peace through it all. We did also find out on Wednesday that Emri will have to be put on Oxygen during the night due to some sleep apnea she is struggling with. This will continue until the next sleep study in 3 or 4 months...We are praying that this is something Emri grows out of quickly! I am not so sure how this Oxygen thing is going to go over with Emri-not so sure she will tolerate having something up her nose all night... Just another adventure!  
Brett and I have a little more exciting news to report! NO-Emri is not getting a brother or sister...yet. We bought a house!! Brett and I had JUST decided that we were going to stop our house search and save for a down payment when this house popped up online. It was an online auction home owned by HUD and we just decided to make an offer because the starting price was so low that it was actually something we could afford. We were told a week later that someone else had won the bid and went back to the original "saving plan." However, two weeks later we found out that the first, and perhaps even second bid had fallen through and that our bid had been accepted!! We were beyond thrilled and began the VERY ARDUOUS process of submitting all of the nit-picky detailed information that the government needed to move forward with our bid. We began to understand why the first and second bid may have fallen through because it is a very complicated process to go about buying a HUD home. We are now just waiting on the notes(conditions) from the appraisal and then we move on to closing on May 23! We will be moving in with my parents for a few weeks in order to make a few fixes on our fixer-upper-it needs a little work;) We are VERY excited to finally be moving into a house of our own and can't wait to start having people over to barbecue and play volleyball in our backyard!!  Our new address will be 301 N. Section St.-hence why 301 is another one of our new favorite numbers!
Well-I just wanted to share the latest will everyone. I hope things are going well with all of you and keep up the prayers for our little Emri-God is SO good.

Friday, March 25, 2011

Spring brings....more appointments!

Yes, I am happy to have spring here and winter left in the dust...or mud for us Washingtonians. The flowers are blooming the sun is occasionally shining and the birds are singing-it's a beautiful thing! With spring has sprung a flurry of activity for our family. My Beppe (grandma) had a hip replacement and is recovering well. God willing she will be home this coming Monday! Because of the surgery my Aunt Hilly and Stephanie came home for their Spring breaks! It was fun to see Steph for a bit and my Aunt Hilly is here for another week to take care of my Beppe. Emri and I have been making frequent stops by the nursing home to check up on Beppe and keep Aunt Hilly company.
Besides all of the surgery excitement, we have been busy with appointment after appointment with Emri. Emri had her FIRST little cold in her 9 months of life a few weeks ago and came out of it great. We took her to the doctor just to make sure her lungs were clear and they were. Besides a few rough nights of sleep and losing a little weight Emri handled the cold marvelously. With Emri's latest rash and first cold I have had frequent phone conversations with nurse Kim and we have seen Dr. Bochsler more than a few times! I can't even tell you how blessed we feel to have them taking care of Emri. Kim puts up with my frequent and frantic phone calls and Dr. Bochsler has made time to see Emri any time anything comes up. As Emri's mom, it's a relief to have a Christian nurse and doctor that care so much about my little girl.
We had 3 appointments at Children's last week Tuesday. Emri had a repeat swallow study done to once again check for aspiration, and then saw her OT and Pulmonologist. Stephanie was able to go to Seattle with us, which was SO nice! I don't know if I could go through one of these Seattle Children's days alone!
For the swallow study, they attempted to make Emri swallow barium in order to be able to see where the liquid goes in her throat. Emri was not too intrigued with this whole barium gig and only gave them three swallows to work with. They did not find any aspiration which made everyone very happy.
Next we saw Emri's Occupational therapist, Karen, whom we LOVE and adore. Karen sat in on the swallow study and carried Emri from radiology on the 4th floor to the OT clinic on the 5th floor. Emri was exhausted from fighting the barium at her swallow study and was laying on Karen's s shoulder the whole time. It was very sweet and Karen, a lover of babies, was in her heaven. Emri did great in her OT session and Karen was impressed with her improvement in gross moter skills. She gave us a long list of things to practice with Emri in order to strengthen her muscles and get her closer to sitting up and crawling.
After OT, we ate a quick lunch and headed to Pulmonary. It was a quick appointment and the Pulmonologist was happy with how Emri looked and sounded. At the end of the appointment she fit us in with a nutricianalist  who re-evaluated Emri's calorie intake and made necessary adjustments to get her weight up. Emri is now a whopping 13lbs, 13.5oz....what a fatty.
A week after our day at Children's we had the dreaded SLEEP STUDY...yuck. We headed up to Children's at 7:00 Monday night and checked into our "hotel room" in the hospital. The pulmonologist wanted this study done to get a baseline read of Emri's sleeping stats for the future. Marfan kids usually have narrower breathing airways and I guess it becomes a concern as they get older. When Emri was all pooped out and ready for bed, the nurse came in with the wires....all 26 of them. Honestly, it was the most pathetic, sad state I have ever seen Emri in. I thought the NICU was bad, but this was a whole different ball game. there were about 17 wires attatched to Emri's head, and the rest were spread out over her body. She had two belts on to hold down the wires and one large monitor belted to her stomach. When the wires on her head were in place, the nurse wrapped Emri's head with pre-wrap and put a "hat" over that. The final touch was the oxygen monitor in her nose. It was AWFUL and heart wrenching to watch this being done. The nurse kept saying she couldn't believe how well Emri was reacting to all of the wires, because most babies go hysterical. There was one point where Emri looked up at me, her face turning red, and her eyes tearing, but she never made a peep. She just sat there and let the nurse do her thing. When I picked Emri up for a picture at the end, she SMILED! I couldn't believe it. I can tell you what I wouldn't be doing if this were happening to me....smiling.
The night was long with little sleep for Brett, no sleep for me, and interrupted, frustrating sleep for Emri. In the morning the nurse said that they got the information they needed and we were free to go. We were ready to get out of there.
Thursday Emri had a very uneventful eye appointment. The doc said her prescription looked good and that she should come back in 4 months.
Later today, I will get Emri's blood drawn to make sure her body is responding well to the final losartan dose increase. Fun fun. Oh! And I think the first tooth is coming through!!
SO-there you have the latest. Thank you to all those who continue to pray for Emri and our little family.  Emri's a special little girl and very loved. We are so blessed. I will update again soon, but for now, I think a nap is in order. Goodnight.