Monday, December 24, 2012

Our Little Christmas Miracle...

Just so excited to share this story of God revealing himself not only to us, but also to the staff here at Hopkins and hopefully to some of you that read this blog. 
There has been mass confusion taking place regarding whether or not Emri did, in fact, have a pneumothorax...or if it was actually just a hyperinflated top lung lobe. The doctors came in to explain to us that because there was so much uncertainty, and because the location of the puzzling pocket was hard to see in an x-ray, they wanted to do a CT scan. Now I know many of you have gone through CT scans...and I know that they now tend to be much safer than they used to be...but it was none the less making me uncomfortable. Emri is so small, and has been under the x-ray machine more times than I can count in the past 2.5 years. I really don't enjoy the idea of exposing her to the amount of radiation it takes to take a picture by CT (several hundred x-rays at once). Because of my uneasiness with getting a CT, they sent the senior radiologist down to Emri's room to talk with us. I thought he would relay some of my fears, but really, he just confirmed that, yes, even though they have gotten better over the years, it is still "last resort" when the choice is made to order a CT. They do not enjoy exposing small kids to that much radiation and know that in Emri's case, she does tend to get more x-rays and scans than the average person in the first place-and will continue to need many more of them in the future. He did make the point, however, that  the risks of taking a guess with her lung condition outweighed the risks associated with the radiation exposure in a CT. After he left, I asked the doctors on the floor if they would feel comfortable just letting us pray for a bit, getting one last x-ray, and then if the x-ray showed the same ambiguity, resorting to a CT. The doctors repeatedly voiced their doubt and skepticism that taking another x-ray would show anything more than the last one they took not but a few hours ago---but they felt comfortable with giving us a few minutes and one last x-ray. They kept saying that while they "knew" a CT would be necessary, they would get this x-ray for our own peace of minds as Emri's parents. Brett and I stepped outside to pray with Brett's mom, dad and baby Abe and then continued on to Emri's room to pray with her. We finished as the x-ray technician showed up to the room.
My thoughts while the x-ray took place were, "God, use this as an opportunity to show yourself here, in this place, to these people. Make yourself known here." 
20 minutes later, the same floor doctors came in and let us know that the senior radiologist was very happy with the pictures we captured by x-ray and that he was able to mark the outline of both lungs. There was, in fact, no pnuemothorax---just fluid surrounding the lining of the lung... Thank you God. We are now fully confident that we need to be treating for a lung collapse and can stop all pneumothorax treatment. 
I know this may seem small...but I am feeling overwhelmed with thankfulness and can't help but stand in awe of our Savior's powerful presence here. If you have been praying in earnest for our little girl's health...please stop to thank God wholeheartedly for his amazing work here, in PICU room 14 at Johns Hopkins on Christmas Eve.
We would like to wish everyone a very Merry Christmas! Brett put our thoughts on Christmas perfectly in the following words...
"Remembering at this time of year, how our Father in Heaven loved us SO much, that he sent his Son Jesus to become a baby, human flesh, and endure the torturous wrath of mankind that we may have everlasting life, praising him forever in Heaven someday! Merry Christmas and may we all dwell on the incredible sacrifice that Christ has made for us, and in return, offer our lives as living sacrifices to his work and the Gospel!"
Here are the facebook statuses of the last few days for those following the blog-sorry I have been slightly neglectful of the blog!

Friday, 8AM---"Finally got to hold my sweet girl last night for about 45 minutes. It went better than the first time Brett held her, but she was also on stronger pain meds. I sang some of her favorite songs to her, which she loved---her eyes were closed, but when I got done with one song she would strain them open to make sure I was going to sing another one:)"

Friday, 2PM---"Today was only slightly discouraging. Emri's lung x-ray this morning showed her left lung looking slightly better than yesterday, but the lower lobe of her right lung has now collapsed as well. She is on an even higher flow of oxygen now and we are trying to move her around a little more in hopes that the lungs will open back up. Her stomach is still very distended-just hard as a rock. Her nurse/doctor team here is trying to figure out how to solve this. We got a visit from Dietz today which was wonderful. He was pleased with how she looked-but a little concerned with the stomach issues. We are still chugging along---but it's hard to see our sweet girl so very, very uncomfortable and desperate to feel better. It's next to impossible to explain to a 2 year old why they are needing to go through so much pain. Praying for quick healing for my little hero-and that we may see her sweet smile light up the room again soon."

Friday, 5PM---"We are getting another chest x-ray soon. Emri's breathing has sped up and her O2 levels are slightly down. The doc is saying that if a higher O2 flow doesn't work, and if she won't tolerate a mask, we are going to have to talk about a ventilator. Please pray that this doesn't have to happen. I would really just love her lungs to inflate...right now."

Saturday, 12PM---"Emri just got finished with some chest PT-they used a pneumatic tamper to beat the left side of her chest in hopes to help open up that lung...and she LOVED it. She cried every time they stopped the machine. She is also on high flow (20) O2 and has been all night. The chest x-ray this morning showed no change in lung status. She is currently getting blood because her hemoglobin was super low and they are hoping it helps her get stronger. We are going to try to move her around a lot today in order to get her to breathe deeper for those lungs. We need her x-rays to start looking better so there won't be a need to put her on the ventilator. Thanks for all the prayers and please keep them rolling..."

Saturday, 6PM---"In shock right now---we just got a package in the mail from a 4th grade class of Sydney, a girl who is also affected by severe marfan syndrome. In the package were 17 of the sweetest cards from the kids and an Ipad. What a gift! Can't tell you how completely spoiled we feel right now! Emri is going to have so much fun playing games on it and talking to her Grandma, Grandpa, Aunts, Uncles and cousins on it's camera!!! THANK YOU! Slightly speechless- and that doesn't happen very often. "

Sunday, 3PM---"This morning was encouraging...but then about 3 hours ago things went downhill once again. Emri's lung x-ray this morning looked great-the left lung looked just about normal again and the right lung was completely clear. The x-ray this afternoon, however showed a pnuemothorax on the left lung. I have always been under the impression that a pnuemothorax meant collapsed lung-but it is actually an air pocket outside of the lung that can oftentimes cause a collapsed lung because of the added pressure to the outside of the lung. Emri now has to have a mask on with full oxygen for the next 6 hours until we x-ray the chest again. I have no idea how oxygen helps this situation...but they tell me it somehow does. Emri seems frustrated and discouraged---she doesn't seem like a 2 year old, she seems like a 12 year old---like she understands everything the doctors say and know this is another major setback. I feel for my sweetheart and just desire some relief for her, even if it means one more line being taken out, or a break from having something on her face. She is terrified every time a new person comes in the room-she just knows it means more pain and discomfort. Right now, all she wants is to hold our hands and watch Berenstein Bears, so that is what we will be doing for the next 6 hours. Please pray with us for some relief for our sweet girl.

Here she is with her brand new mask, watching her favorite family of bears."

Sunday, 9PM---"So...God is hearing the prayers across the nation...over the seas! We just took this picture of our sweetheart. Don't know if it is a short little break she is getting form her pain and discomfort...but it's a break none the less! She is singing songs, smiling and giggling with daddy for the first time since before surgery! We are waiting on x-ray results, but I can't imagine with how she's acting that they aren't going to look any better! Thank you, thank you, thank you for keeping our precious girl in your prayers! I can't tell you what a relief it is to see that big beautiful smile! I will keep posting updates...I am hoping we are turning the corner!!"

Monday, 1PM---"It's been a one step forward two steps back type of morning. We just had the floor doctors come on rounds and tell us that they are not so sure if Emri does in fact have a pneumothorax. It could still be a pneumothorax, but it could also be hyperinflation of the upper left lobe. These two problematic situations are treated differently and treating a hyperinflated lung aggressively can cause a pnemothorax to worsen...which can be dangerous and require a chest tube for immediate treatment. Since they can't tell what it is on x-ray they are thinking about a CT scan. I am not a huge fan of exposing Emri to more radiation, so I did ask if there was another way around this and the docs will be talking to radiation to see if there is something we can try other than a CT... I am feeling frustrated and emotionally drained---especially after last nights excitement-I really thought this morning would show better results. Please help us pray through this situation. Emri is once again, very frustrated with everything going on.
The picture below just makes me want to cry. Dr. Dietz came to wish us a Merry Christmas today with a giant basket of fruit, chocolate and stuffed animals. When the doctors came in to talk to us, he left and I saw him in the background checking out Emri's chest x-rays on a computer in the nurses station. He is just a wonderful, wonderful man-and I can't express how much it means to us that he truly, truly cares about Emri and desires the absolute best care for her. Just in case it wasn't enough that he discovered her life saving medication, he had to bring us a fruit basket too."

Thursday, December 20, 2012

Here are today's facebook updates. 

1AM---"Emri's body is trying to understand what just happened! She is pretty uncomfortable and just can't find a good position. She is still drifting in and out of sleep and every time she wakes up she seems to get kinda freaked out with how different her body feels. The whole middle section of her body is a completely different shape which we are thankful for-but we know it will be a difficult adjustment for Emri. God has blessed us with an incredible, tough little girl."

10AM---"Tried to let Brett hold her this morning-because she wanted it so bad-but it was too painful yet. Poor girl just wants to be cuddled---hopefully soon. She is doing well-remained stable all night! Got a visit from Sponseller already this morning and he is very happy with how she looks and how her stats are holding up. Continuing to thank our Lord for blessing us with a successful surgery!"

12PM---"Emri's respiratory and heart rate have been running high and she is still oxygen dependent. They did a chest x-ray and found that her left lung is partially collapsed. We are starting respiratory therapy now and were told this would lengthen her stay in the PICU."

2PM---"While the respiratory therapist was attempting to get Emri's mask on, Emri pushed it away and said, "I need a break.""

So as you can see, we had a little setback today with the partial lung collapse. Emri has been on high flow oxygen the whole second half of the day and they plan on taking a repeat x-ray some time tonight to see if it has helped that lung re-inflate. We are in the PICU until her lungs are back to normal and until her stomach is less distended. Emri seems to be experiencing minimal pain thanks to her continual morphine and tylenol drip along with regular doses of Valium. This surgery comes with the understandable reputation of being very painful and I am glad Emri's pain is being managed tolerably for her. Due to the fun mix of meds we have been hearing a variety slurry, nonsensical sentences come from Emri's mouth. She wakes up a few minutes at a time to catch a little Berenstein  Bears or Veggie Tales and usually wants a hand to hold and an apple juice soaked sponge to suck on. I am praying that God makes this painful time for Emri as short as possible and allows us to perhaps even enjoy a minimally painful Christmas together as a family. 
We are so thankful to have Brett's parents here to care for Baby Abe. I have no idea how we could be doing this without them. It is so nice for Brett and I to be able to be here together focusing our energy on Emri and her recovery. Once again, I can't express my thankfulness to have our sweet girl with us and surgery day behind us. Thank you to all who have been praying for our sweet girl and Brett and I. We feel so blessed to have so much support. Seeing how many people follow Emri and her progress on facebook just astounds me and allows me to feel the love of so many of you that can't be here in person. We feel so supported by family and friends and even a few people I hardly know! Emri is one special little girl---God has given us such a gift in her. Updates will continue.

Wednesday, December 19, 2012

So I wanted to update the blog by posting the facebook statuses of today for those of you who read the blog but are not on facebook. So here they are starting from early this morning to the very latest one written a few minutes ago.

8AM---"Brett and I both got to bring Emri back into the surgery room and be with her until she fell asleep. The most emotionally stressful moment of my life yet. Jesus take care of my sweet girl."

11AM---"Breathing a tiny sigh of relief-they called the front desk to say they were still working on her but it was going well! I just want that girl back in my arms! Thank you Lord for keeping Emri safe so far."

12PM---"Closing her up-waiting for Sponseller to come talk to us. She will be moved to the PICU in the next 45 minutes."

1PM---"Can't help but smile right now. Emri was just moved to the PICU-a nurse came out to get her binky because she is requesting it along with her mommy and daddy. Apparently she keeps telling the nurses, "NO new back, NO new back!" She has her wits about her already! THANK YOU GOD FOR KEEPING EMRI IN YOUR HAND THROUGH THIS ALL!!!! I am SO full of thanks right now...can't wait to go see my sweetheart! I will update more when we are more settled."

6PM---"We are settled in the PICU with Emri. She has been drifting in and out of sleep-and on a lot of pain meds. She wants to be held and drink water and can't really do either of these things at the moment which is causing a little frustration on her part. Surgery went well, besides a few dips in her blood pressure which was treatable with medication and fluids. We are PRAISING GOD that she is remaining stable in recovery so far and that her pain is being managed well. I can't tell you the flood of relief I am feeling just seeing her heart beating and chest rising and falling! Holding her little hand has never felt so good."

This was our day in a nutshell. It was long, it was draining, but is turning out to be so wonderful. We realize we are not homefree yet-and know that these first 48 hours are always the most vital...but we feel God holding us close and we know prayer is surrounding our little sweetheart. Please continue to help us pray Emri right through these first few days and the rest of her recovery. God has been truly blessing us.

Tuesday, December 18, 2012

So I wanted to write a quick blog tonight before we head off to surgery tomorrow morning at 5:30am.

Last week we had two appointments to discuss Emri's pectus (caved in chest wall). We went in to these doctors thinking that they would be wanting us to schedule Emri's pectus surgery next. However, when discussing Emri with them, and having them look at her and listen to her, they both said NO to a pectus surgery! We were thrilled to hear was slightly stress inducing-- thinking about scheduling another surgery before we were even started with her spine. The pectus repair doctor that we saw did say that Emri's pectus was quite severe-one of the worst he has seen. But the good thing about that is that her pectus can't get much worse than it already is. He said that he anticipates her lungs to still be able to grow with her and function enough to keep her oxygenated as she gets bigger. Emri's breathing has progressively sped up in the last 2 years, but that is because the pectus has also been progressing with her growth. SO-if the pectus is as bad as it is going to get, we are hoping that her breathing will also not get any worse. I have to mention that in th emiddle of this appointment Emri kept saying "daddy, daddy, daddy!" until he finally acknowledged her. When he did, Emri took his hands, put them together and said, "I want to pray daddy." Talk about a tear jerking moment. So- while I was asking the last of my questions to the Doctor, Emri and Brett were quietly praying in the background. Sweet, sweet girl we have.
The second doctor we saw was a pulmonologist that Dr. Dietz actually trained under...which was pleasing to us of course:) She agreed with the pectus repair doc that we should leave the pectus alone until it starts actually causing enough problems to get concerned. She also listened to Emri and heard her left lung inflate a few times. She ordered a chest x-ray and was pleased to see two lungs with air in pockets of collapsed lung-which reassured her sending Emri into surgery. We were so happy to see those big beautiful lungs with air in both of them!
Yesterday we had pre-op. We met with Emri's wonderful surgeon, Dr. Sponseller-head of pediatric orthopedics here at Hopkins. He explained the surgery to us and told us he would take good care of Emri. He did mention at one point that he had done a few other kids as young as Emri, which made me happy. He discussed the risks with us, which is never fun to listen to...but I kept my head steady-focusing on the fact that God is in control and HE has our precious girl in HIS hand. We met with Anesthesia after Sponseller. They made sure to let us know that Emri is indeed a very unique case and that they were making sure to cover all of their bases with her. We discussed her sudden drop in blood pressure during her last g-tube surgery which was, of course, concerning to them. They did emphasize that they have seen many many marfan cases and even some infantile cases. But we know that every case is unique and takes a specialized plan from the team of doctors, surgeons, and anesthesiologists involved in the case.
Today was "Dietz day"-I am working on getting it coined as a national holiday. Ha! We had Emri's echo first and then met with Dietz afterwards. Emri's echo looked great in the sense that it had not changed at all from the last echo! We were SO relieved and happy to hear this! I know Dietz loves to see good results like this too-- knowing the medication and the dosage he is giving Emri appears to be working! He was once again, so so sweet with Emri and even stopped to chat with us in the waiting room, along with his wonderful assistants, Gretchen and Libby. After Dietz walked away from talking with us in the waiting room, Emri piped up and said, "He is a good man" out of nowhere! Tell me that isn't hilarious??!? What a clown.

Throughout all of these appointments our sweet "baby Abee" has been an amazing, amazing baby. What a little blessing he is to us. He is currently spending the night with Grandpa and Grandma Van Andel in order to let Brett, Emri and I have a good night of sleep before surgery. We will see if I can actually shut my mind off enough to fall asleep!

We have given Emri her bath, sanitized her surgery site, and put her to bed. It is so hard knowing what she is going to have to wake up and face in the morning. Emri has, once again, been such a trooper this whole trip. She continues to charm every doctor, nurse, and fellow Ronald McDonald housemate she meets. I am certainly nervous to send her into that surgery room tomorrow, but delight in the fact that God is in complete control of the situation. I dread the pain my sweet girl will have to endure from this...but pray that God makes recovery quick and as relatively painless as it can be. To be completely honest-my prayer the last few days has just been for God to allow Emri to make it through this all-to feel her warm bear hugs again, and to hear that sweet little voice sing "I Yove you Yord".  This would all make me one happy and thankful mommy. Praying that the Lord will guide the surgeons hand tomorrow and keep our hearts at peace. Thank you to everyone who is praying right along with us.

This is the picture that I will be focusing on tomorrow while I wait the 4-6 hours during Emri's surgery. May be a bit "idealistic" but I can't help but find comfort in it. 

"Is anyone among you suffering? Let him pray. Is anyone cheerful? Let him sing praise. Is anyone among you sick? Let him call for the elders of the church, and let them pray over him, anointing him with oil in the name of the Lord. And the prayer of faith will save the one who is sick, and the Lord will raise him up. And if he has committed sins, he will be forgiven. Therefore, confess your sins to one another and pray for one another, that you may be healed. The prayer of a righteous person has great power as it is working."

Sunday, November 25, 2012

Well, here he is...

Our handsome, chubby, sweet baby Abe. Abe Douglas Robert Van Andel arrived on October 29, at 12:30 pm, weighing in at 8 lbs, 3 oz, 20 inches long...happy and healthy! Brett and I went in to Labor and Delivery on Monday, the 29th, to have my water broken for a number of important reasons. We arrived at the hospital at 7:00 AM, had my water broken by 7:30, and Abe entered the world exactly 5 hours later. Everything about Abe's birth was different from Emri's-which was a relief. My labor went so fast that by the time I even had a thought about pain medication, it was too late. Although the pain was profound to say in the least, the birth was beautiful and the recovery much quicker than with my first medicated birth. I cannot describe to you the overwhelming relief that came with hearing those first few cries coming from my sweet baby was such a beautiful sound. Abe's apgar (health) scores at birth were 8 and 9 out of 10. When Emri was born, she scored a meager 1 and 2 out of 10. Needless to say, the experiences of meeting my two precious kids for the first time, were entirely different. Brett and I were overcome with thankfulness and disbelief to have Abe come to us healthy. Along with feelings of gratitude and relief Brett and I felt with Abe's arrival, came some difficult memories of Emri's birth. Seeing our rosy-cheeked, round-bellied, perfectly healthy baby Abe made us remember how earnestly we desired that for Emri shortly after we learned she was not any of those aforementioned things. We are SO thankful for our healthy son, but know that sitting him next to his big sister and acknowledging their differences may be extremely hard at times. We know that Emri will inevitably get to a point where she is able to begin making comparisons, and will realize that Abe doesn't need to visit the doctor near as many times as she does. She has already come to recognize that Abe does not have a g-tube or glasses...and that is hard for me, as a mom, to watch. Through all of this, though, I have to remember that God gave us Emri and Abe, and we trust, wholeheartedly, that He knows what is best for our little family.
So far, Emri adores her little brother for the most part. She refers to him, endearingly  as "baby Abee" which  makes me smile every time I hear her say it. Emri loves to test her limits by pushing on him, touching his face, and making plenty of noise while he sleeps--but she also loves to hold him, help give him baths, and "watch" Barney with him. She gets slightly distressed every time he cries and insists on someone helping him until he is once again calm. It has been a stressful but sweet, draining yet thrilling, demanding but beautiful, last three weeks acquainting ourselves with having our two amazing children.

In other news, our little family of four will be departing Seattle and arriving in Baltimore on December 11th. We have a few big appointments for Emri at Johns Hopkins before her spinal rod surgery, scheduled to take place on the 19th. This surgery has been looming before us since it was scheduled this summer and lately is becoming very, very real. We leave in a little over two weeks and the thought is somewhat terrifying. Knowing that we will be watching  Emri suffer through more pain and constant discomfort is beyond distressing...but knowing that, as with any surgery, that something could go wrong and she could be taken from truly, truly unbearable. Please pray that God brings our sweetheart through this surgery safely and that we can see her smile and experience her bear hugs soon afterwards. My love for this girl is just...unbelievable.
Emri has been pretty consistently struggling with keeping all of her feeds down. Just about every morning and after many of her naps, Emri loses much of the milk that she has been given overnight. It has been terrible watching her go through this, and it is so hard to turn her g-tube pump on, knowing it may be causing her discomfort in the end. But what really gets me is that every time Emri does get sick, she perks up afterward and says "Emri feels better!" She is so good at turning every difficult instance around and pulling the good from the bad. God has given us an amazing little girl.
So that is the latest from us. We are hoping to be able to keep updates going on this blog and facebook during Emri's upcoming surgery. PLEASE keep our family in your prayers. We are praying for peace, perseverance and HEALTH in the coming weeks.

Thursday, October 11, 2012

"I am just going to be honest and say that yes, I am in tears writing this blog tonight. The pain of seeing Emri go though what she has over the past two days, over the past two's just getting to feel unbearable. This is our second night at Children's hospital and we are still attempting to figure out the cause behind Emri's rapid breathing, rising heart rate, reoccurring fever and now we have added dropping o2 levels and low blood pressure. She's being wheeled out now with Brett for a lung x-ray to rule out pneumothorax (lung collapse)... among other things.
I do in fact believe that our God is holding her in His hands and that there is a plan set in place for her life. Right now, watching this plan unfold just hurts. I know that I would love Emri even if she had the most rotten disposition- simply because she is my little girl...but I believe it has to hurt more seeing her sweet, endearing little personality dwindle into medicated sleep. The way that she looks at me when I am pinning down her arms while she gets poked time and time again just pierces my heart."

This ^ is what I wrote while staying at Children's-I just never posted it. It was a long, painful weekend and I am so thankful and happy we are home and that Emri is finally coming around to being Emri again. The stay at Children's was nothing less than proved to me once again that we, as Emri's parents, know her the best, and that we are just going to have to be those pushy "annoying" parents sometimes. Let me just say this; I wish that doctors would just admit that they know nothing about neonatal marfan syndrome....  when they know nothing about neonatal marfan syndrome. Emri is the only known case of this rare disease that they have treated at Children's---therefore, none of the doctors can say, "yes we have dealt with cases such as this one." We asked every day, multiple times, from the very beginning of our stay at Children's to have a cardiologist involved while Emri was at the hospital. When the resident doctor would call and ask for a cardio consult we would get the response, "cardio doesn't think this is necessary." REALLY??!?!? I have a daughter with a severely dilated aortic root, mitral valve prolapse, and a heart rate of 170...but it's PROBABLY nothing to worry about. When cardio DID finally come in to see Emri on the last day of our stay at Chilren's, when she no longer had any of the symptoms we were worried about in the first place, I let them know how we felt about the situation. I explained to them that we, as Emri's parents, are trying our best to play defense, so to speak, in Emri's situation. We are attempting to prevent a bedside emergency, and we need them on our team and well aware of the serious condition of Emri's heart. We got a sincere apology from the one cardiologist that came to see Emri, but I don't know if that is enough to change things in the future.
Also, we found our that the night Emri got poked and prodded 6 different times in an attempt to insert an IV was completely unnecessary. The pulmonologists on Emri's case were astounded to find out that this happened without their permission. They said that they never would have given their consent to put her under that much stress and that there were other ways of managing Emri's fluid levels.
And last, but certainly not least...the pharmacist that was filling Emri's prescriptions for her stay in the hospital decided, by his own judgement, that Emri's irbesartan dose was dangerously high and lowered it without asking any questions. I found out after actually looking at her dose the last day we were in the hospital, that they had been giving her a lower dose her entire stay at Children's. Uhg. Just so frustrating.
This all goes to show how unique and un-explored Emri's specific disease is. Brett and I have been feeling intimidated by the fact that we may very well know more about how Emri should be cared for than the doctors at Children's. THIS is why we are so relieved to be getting Emri's spine surgery in Baltimore, at a place where they know Emri's specific needs inside and out.
I am not writing all of these frustrations to slam Children's Hospital...we have had many good and helpful experiences there. More than anything, I write these things to give you a sense of how scary it can be...being a parent of a child with a rare condition...and also to shed even more light on our decision to get most of Emri's treatment and care all the way across the country. Not going to lie, Brett and I have often half seriously joked about a move to Baltimore in our future.
As of now, Emri continues to recover-still a little sleepy and pale-but happy to be home completely mezmorized by that dreaded purple dinosaur once again. She continues to be hooked up to her pump 20 hours a day, and her feeds are still going up every few days.
We are patiently awaiting the arrival of baby brother and can't wait to finally meet him. Emri seems to be getting sick of all the talk about her baby brother---I think she wants the tangible proof that he indeed exists:) Hopefully the next blog will be happy news about the new baby and his healthy, proud big sister. Thank you for all who followed our updates this last weekend....for the thoughts, prayers, and encouragement. We are continually praying God's blessings upon our little girl-but continue to acknowledge his perfect will in every situation.

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?  

Thursday, July 12, 2012

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 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 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 

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.