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