Saturday, May 26, 2018


Starting blogs isn't my thing-I feel like need to begin with a different, creative and elaborate welcoming every time, and I'm just not feeling it. There...was that creative enough??😎😁
ANYWAY...We left for our Baltimore road trip on May 11th. We had planned on taking our little Toyota, Sienna minivan all the way across the country like we did last year, but we received a call from a family member a week before we were planning on leaving saying they would happily rent us a bigger/newer van for a month so that we did not need to travel with the concern about our old minivan. WHAT A HUGE BLESSING. I can't even wrap my mind around how huge of a blessing this van has been for us on this trip. Emri's wheelchair, oxygen machine, and medical supplies take up the space of 3 extra people, and space has become an issue traveling in a minivan. Plus, we added a 6th person to our trip this time....ENTER MICHELLE.

HUMUNGO BLESSING #2. Michelle came to us through a crazy set of life circumstances and decided to dive right into our crazy set of life circumstances. Basically, she had just been through a life trial of mass proportions, and I was struggling just making "it" in life with all the, laundry, medical appointments, grocery shopping, meal making, toenail clippings, showers..."the things". Brett also just so happened to be looking for someone to hire a few hours a week for his architecture business. Michelle and I met for coffee one day in December, and by April we were folding laundry together in my living room and she seems to be ok with sticking around for a while! She has agreed to help Brett with some secretarial work, as well as helping me out with life and being a support in times where we need extra help with the boys during Emri's hospital stays/medical appointments. It has been a TREMENDOUS blessing having her help, friendship, and willingness to tag along on our crazy adventures, such as this very road trip across the country. The "plan" (plans are funny little things) is to turn the water tower in our backyard into a spectacular three-level tiny house for Michelle to live in by mid-summer. SO, now ya'll know....Michelle is here! And we are LOVIN' it.

EMRI. Emri is doing so well right now. We are FINALLY through sick season (I mean, right??!?) and boy howdy, it was a rough one. I AM SO THANKFUL to be through the winter and heading right on into summer. The sun is just so glorious. It really felt like ANYWHERE we went this last winter, we would catch some horrific bug, and our whole family would be down for the count for several weeks following. Brett and I have literally discussed a hibernation plan for next would involve our family staying inside the house always, visitors being sanitized and temperature screened at the door, ordering groceries on Amazon prime, face timing those brave, germy souls in the outside world, loads of vitamin C, D E, F and G and movies. If you want to join us, starting date is when I hear the first cough at church.
Got sidetracked...sorry....Emri.
Eyes: We saw an ophthalmologist at Hopkins. He pretty much re-iterated what we have heard from our guy in Seattle. Emri's eyes are super complicated. Her lenses are balled up, partially blocking her vision. She is constantly looking through, or above (when she really needs to see something) a globby lense ball. We have been waiting for her lenses to drop naturally, but they really aren't moving. Doc at Hopkins and at SCH are both suggesting beginning to think about lense removal. We would have jumped on removing those silly lenses years ago if it weren't for the fact that removing them dramatically increases the risk of retinal detachment. Emri's pressures in her eyes read normal, which is encouraging, because of how many marfan patients deal with glaucoma. So all in all, a good visit with nothing surprising/new. In July, there is a new ophthalmologist joining the team at Hopkins that is specializing in connective tissue disorders. He has worked with Dr. Dietz and comes highly recommended by Dr. obviously we won't be thinking twice about seeing him the next time we are at Hopkins.

Spine: Emri's spinal rods are just a whole complicated, slightly frustrating, utterly confusing issue. I will not go into specific detail on this blog, but basically we have not had much success in magnetically lengthening Emri's rods at SCH. In fact, I believe Emri's rods have been unintentionally shortened more times than they have actually been lengthened. This has caused Emri a great deal of unnecessary pain and consequentially frustration for us as her parents. The orthopedic department at SCH told us her rods would no longer lengthen and needed to be replaced. When we arrived at JHH, Emri's orthopedic doctor/surgeon here, Dr. Sponseller, easily and effortlessly lengthened her rods and informed us that surgery would not, in fact, be needed. Although we were pleasantly surprised, this also leads us to think that we may need to be seen either at JHH for all of Emri's spinal lengthening's, or find a team somewhere closer to WA more familiar with the MAGEC rods. We also discussed Emri's loose screw in her upper back, and her CSF leak in her lower spine with Sponseller. Both of these issues we were told at SCH would need to be operated on quickly, and Sponseller assured us that as long as Emri is in no pain, she is fine to be left alone. We, of course, like the idea of Emri being left alone. It can be emotionally/mentally tolling, getting such opposite/conflicting direction from two big medical establishments...but if any of you, my lovely readers, were to accompany us to appointments at the two different hospitals, you would quickly understand why we take one opinion much more seriously than the other.

Heart: Dr. Dietz is the best....have I mentioned that before?? Every time we see him, every time he evaluates Emri, every time we attempt to even remotely dissect/unpack the new and exciting medical information he casually throws our way in conversation, every time he smiles so genuinely for his yearly picture with our precious daughter....every time....we just feel like we can fly. We feel all the heavy questions/medical concerns we have about Emri are just lifted for a bit---knowing that he is pouring all the effort he is able into understanding this disease and then finding ways to destroy it. Emri's heart remains stable. Her aorta is within its normal range, and her valves are all still leaking the same amount. We are so thankful.

Lungs: We had the wonderful opportunity to have Emri seen by our favorite pulmonologist, Dr. Neptune. We just love this amazing woman and all she does for the marfan community. At the moment, she is working on the ability to provide specialized masks for people with unique facial shapes--like those with the long, narrow marfan face. Emri is in need of a mask for her sleep apnea issues, but finding the right mask is no simple task. Dr. Neptune was concerned with how often Emri was sick this past winter, but when I mentioned it was ALL of us, she felt a little better about it. Dr. Neptune also observed some crackling in the lower portions of Emri's lungs, but thinks it's most likely just chronic lower lobe collapse. This is not uncommon for kids that deal with long, skinny lungs and persistent pneumonias, but it is something that definitely puts Emri at higher risk when dealing with any respiratory virus. Hibernating is just sounding better and better.

There you have it...Emri's medical update. Because of the trouble we have been having with SCH with Emri's spine lengthenings and a few other incidences, Dr. Dietz mentioned that being seen elsewhere may be something to think about. Of course, we would love to be seen at Hopkins for every appointment, but that is a tough travel schedule to commit to, especially when flying with Emri is so challenging and hard on her body. Dr. Dietz mentioned that he would feel very comfortable assisting us in assembling a team at Stanford in northern CA. This is a situation that we would love prayer over as we pursue what we feel to be good and trustworthy medical care for Emri. It has been a little bit of a rollercoaster over the years, and we would love a little more consistency (in opinion and level of infantile marfan knowledge) in Emri's care. Pray with us as we contemplate these next steps in Emri's care.

If you have been following on facebook at all, you probably saw that Emri made a very special little friend during our time in Baltimore. Emri had the unique privilege of spending some very sweet, quality time with her friend Ellie. Ellie also has infantile marfan syndrome and is staying in Baltimore for a series of leg surgeries. Emri cried and cried and cried the first night we left after seeing Ellie. Emri was just an emotional little basket case leaving her new friend. Emri shared with us how great it felt to spend time with someone who faces so many of the same struggles and challenges that she does. She really spilled a lot of thoughts and feelings that meeting Ellie must have allowed her to more fully process. It was quite a night for us as Emri's parents hearing all of these big feelings come out of our little girl. Thankfully we were able to cancel some plans for the next day and the girls got to spend more time together. Emri and Ellie talked pills, g-tubes, spinal rods and wheelchairs, as well as shared interests in art, animals, math i-pads and fingernail polish. It was pure bliss as a mom, watching Emri feel so special as she realized how similar her and Ellie really were. I can't help but think this may be the beginning of a very special little friendship.💕

So, since Emri's spine surgery was cancelled, we attempted to change the course/duration of our trip...but alas, our VRBO in Myrtle Beach, SC was we were FORCED to go on vacation! Bummer, right??!? We were planning on enjoying some time in Myrtle Beach in between all of Emri's appointments at Hopkins and her surgery which was scheduled for the 29th...but now we have NO SURGERY to look forward we are literally just relaxing here. It's really, really great. The kids have plenty of fun to choose from between the ocean, the lazy river, the hot tub, the waterslide, or the pool. The boys are doing their boy thing, where they expend vast amounts of stored up energy and boy-ness swimming viciously ALL. DAY. LONG. and then not-always-so-gracefully melting into a puddle of sleep-deprived despair around dinner time. But seriously though, these boys have just really blown me away. They have been TROOPERS throughout the many hours on the road and all of the medical appointments. I am so happy they are getting some time to just be crazy little men and let it all out over here. We are savoring these times, watching our kids create memories together and soaking in the time to just BE. It's so nice. Praise God for this unexpected dread-free time.

We have yet to plan our exact route home...I guess we will see where the winds take us...which, judging by the east coast forecast, could be a little nutso. I am just bursting with thankfulness to our gracious Father for His never-ending provisions in our lives. Life isn't always so beach-y. Sometimes it's really kind of dark, stormy and rainy...but those storms sure do make the sun feel that much more sunny.

If you actually read this all, thank you for persevering. I hope to update this blog about our trip adventures when our trip is actually through. Hopefully that silly wind eventually gets us back home!

Friday, November 10, 2017

Emri Update

SO. I've decided that I'm not feeling particularly eloquent, smooth, wise, or wordy today...but I'm going to write anyway. My apologies in advance for a potentially rough/jumbled read...but perhaps it will give you a clearer picture of what my brain actually looks like right now....blah.

Emri and I have made ourselves nice and cozy at Seattle Children's Hospital AGAIN. Guys, we've been in and out of this place like every other weekend since July. We've been seen for a spinal fluid leak, (which included a sedated contrast CT scan), a loose spinal rod screw, (I DON'T KNOW HOW) potential spinal rod infection, strep throat (but we came in to be evaluated for a potential bacterial meningitis), pneumonia, and yesterday we came in to see if the pneumonia was back. The pneumonia does not seem to be back, but we are now inpatient because of an overwhelming amount of concern (from me, the doctors and also the random, semi-annoying, overly verbal child at the park that told Emri she was too skinny).
This is what I know. Emri has infantile marfan syndrome. Kids with infantile marfan syndrome are really skinny. Kids with Infantile marfan syndrome don't gain weight easily or quickly...but they do gain weight. Emri hasn't really gained weight in like a year. This is concerning. Emri weighs about 32 pounds and is 7 years old. The average weight of a THREE year old girl is 30-36 pounds. These are all the facts.
Emri literally has not caught a break from sickness since July. Our family has had all of the viruses. As of late, when Emri gets a virus, it lingers right on into the next virus... and just seems to wreak havoc on her frail little body. You would *think* that Emri can't possibly lose weight, but she does somehow. It's so scary to see.
What I have learned over the past month about Emri while researching and asking questions is that although Emri is not psychologically anorexic, she is physically. Her little body is starving itself. She has no appetite, no fat, and nothing stored up to fight viruses with. She is malnourished. This is not because we don't feed her. She eats food by mouth and gets 3 tube feedings daily. It's just apparently not enough.
Are these words as painstaking for you to read as they are for me to write?!?? Anorexia, malnourished, starving. Anorexia, malnourished, starving. I am Emri's mother. I feed this kid. I work hard to get all three tube feedings in her tummy by the time she goes to bed...and she's still "starving". I'm her mom and I see her body on a daily basis. It's hard for me to look at. I can't imagine how shocked the world must be when they see her.

This is really hard. Just like it was hard to hear that Emri has marfan syndrome, a "HUGE aorta", leaky heart valves, severe kyphoscoliosis, -20 vision, restrictive lung disease, may be wheel chair bound...etc. Unlike many issues that have arisen in the past, there are no clear answers/solutions to this problem. There is, however, hope seen through the recent weight gain of some of Emri's more severely affected marfan friends. After communicating with another marfan mom and reading her story on how she worked with a gastroenterologist at Hopkins and a nutritionist in her area to have her daughter actually put on weight, we are going to attempt the same protocol in an attempt to have Emri also gain some weight. We are hoping and praying that Emri can tolerate the amount of formula she needs through her tube and that she will not entirely lose interest in eating by mouth.
Obviously this program is going to require more of my time, attention, energy, and perhaps even some math. Counting calories, more tube time, and consistent weigh-ins will all be added into our already chaotic schedule. Obviously this dear, sweet girl of mine is worth every new contraption and calorie counting protocol thrown at us, but you can all still pray for us as we navigate yet another obstacle in this challenging marfan life.

Here is a hospital pic collage for your viewing pleasure...because when you are in the hospital, you have time to do these silly things. 

Also, here is a link to Emri's current favorite song...because she likes to pick songs that make her mom cry. And because...truth.  Enjoy.

Monday, December 12, 2016

The year 2016 in review!

Wow It's swallowed me whole and left me blogless for like a whole year now! I do apologize to anyone who actually depends on this blog alone to keep up with our family, because as of right now, you still only know about 2 out of 3 of our children, and are still left with our latest blog update written in January. I WANT to blog-I ENJOY blogging...but I have THREE kids, a house, a husband, and I don't even shower every day-time is precious.
I just received our Christmas cards and in order to get out of writing a letter updating everyone on our family every year, I always include our blog on the back of the card. So now before I actually send out the cards, I must update. Where do I even begin??!?

January 2016:
-I turned 28. I discovered I have grey hair-like a lot if it. I'm just going to embrace them. The more grey, the more wisdom, they say. ;)
-Brett began his new job at Elements! He is working in Lynden, commuting daily while continuing to design some houses for his own company, ANC Design on the side. A packed schedule for him, but we are grateful for what God has provided work-wise!
February 2016:
-Marfan Awareness month! If you haven't looked up the it please. SO many dangerous undiagnosed cases.
-We lost another little life in the marfan world. Logan was Emri's age and had already overcome so many adversities in his little life. He walks, brace-free, in glory now.
March 2016:
-We received amazing news from Hopkins that Emri's heart looked stable, despite Seattle Children's seeing growth on her echo done in January. Tears of joy!
-Emri got her MAGEC rods lengthened and experienced severe pain afterwards. Valium is magical.
-We got the flu...but made it through without a trip to the hospital! Praise GOD!
-We got a new little fluff-ball dog named Harley that we continue to thoroughly enjoy. She is a stress-draining ball of love.
April 2016:
-April 5, 2016 at approximately 12:41pm: Abe and Emri napped AT THE SAME TIME. I was too excited about them napping at the same time, that I couldn't even nap with them.
-Emri weighed in at the doctor at 30 POUNDS! Big day for us.
May 2016:
-Emri received another STABLE echo report. The word "stable" will never, ever, ever get old for me. PRAISE THE LORD!
-While we received the amazing report mentioned above, another little marfan love went to be with Jesus. Constant reminders that we are NOT in control and that life must be cherished as long as we are gifted it.
June 2016:
-Our big girl turned SIX. WHAT??!? God has done great things for us-and we are FILLED with JOY! Our sovereign God has given us 6, going on 7 years with our sweetheart and we could not be more grateful.
-We had a blast at our church's VBS.
July 2016:
-We went on a family road trip to Venice Beach, CA. Brett had to work, measuring things at a Hotel there. We had a great time seeing the redwoods, visiting the beach, hanging at our hotel, visiting a wild animal park in which we received an elephant "car wash", and the kids did amazing in the car!
-Brett survived the RAGNAR race. I don't remember how many miles his team ran, but it was an indecent amount. They did well, apparently experienced moments of FUN (I will never understand) and Brett lived, which was great.
August 2016:
-We received our long-awaited court date for September 9 in Korea! SO exciting. This meant that we would be meeting Jaemin for the first time, and appear before court in South Korea to make sure the judge thought we were fit to be parents...because the 450,000 pounds of paper, 2 ten page autobiographies, multiple doctors visits, psychiatric approval, and home study weren't quite enough to prove it! ;) The frantic planning began!
-Brett and I celebrated our 8 year anniversary on August 8! What a full, action-packed 8 years it has been!
September 2016:
-Another STABLE heart report for our girl!
-We flew across the world to meet our SON and get approved by the Korean court! We stayed in one of Eastern Social Welfare Societies' guest rooms which was amazing because we received the unique opportunity of spending many hours with the babies located just one floor down in the babies nursery. This is where Jaemin spent the first 7 months of his life. It was humbling to see a very full nursery of babies and a very small staff involved in their care. Bottles propped up by towels, crying babies unable to be comforted because of the small number of caregivers available, nameless, ageless babies...overwhelming doesn't cut it.

-We met our words to describe the feeling. I will share some facebook posts below to update anyone who hasn't seen them on how our 1st trip to Korea, meetings, and court date went.

"So we've been up since 2:30am Korea time! We waited until Starbucks opened at was rough. Finally got our triple shots and delicious breakfast. My stomach is in knots, my hands are sweating... We meet our boy in 45 minutes. 45 minutes. After waiting 2+ years for this moment, excited doesn't even begin to describe how we feel. I know we are in for another roller coaster of an adventure with the decision to adopt a child that does not even know our language, but God has opened so many doors for this to happen and it is obviously HIS will that we are here, experiencing all of this. I praise our Heavenly Father for all of this and give HIM the glory. 
Now, to meet our son."

"We finally met our boy. The social worker deemed our first meeting a "success"! Jaemins foster mom has been showing him our picture and calling us "daddy" and "mommy" and when he saw us, he knew who we were and was definitely on guard! He refused toys from us at first, but warmed up very gradually! He colored with us, played play dough, gave some high fives and even let Brett zoom him around the room like an airplane at the end. He heard a baby crying in the hall and immediately showed his concern thinking it was his little foster brother or his foster moms grandson-it was very sweet. At the end when we asked him if he wanted to come back he answered yes!
The meeting went well and of course was such an amazing and completely surreal experience for Brett and I. Any time Jaemin brushed hands with me, let me put my hand on his back, or allowed me interact with was just an immensely emotional experience! But my heart does hurt...because I see so clearly the love and safety he feels from his foster mom. I know that someday soon Brett and I have to be the "bad guys" and take him away from everything he has ever known and bring him into a strange, completely foreign land across the ocean. And although I know that this is all inside of God's perfectly orchestrated plan, it does not mean it will not be an intensely heartbreaking experience for all involved. We get to spend another hour with Jaemin on Thursday, have our court date on Friday---and then we leave him for 4-6 weeks until we gain permanent custody. So many emotions. Pray for us. Pray for our darling Jaemin. Pray for that sweet peace that passes all understanding. Praise God, from whom ALL blessings flow!!"

And then our second meeting...

"No words. Just these pictures."

And then our court date...
"The only verse on my mind all day..."The Lord has done great things for us, and we are filled with JOY!!!" Psalm 126:3. Our adoption must be processed by the court for the next 4-6 weeks and then we will receive final approval. We are having a hard time expressing in words how absolutely amazing this experience has been. Our next trip here will be short and will revolve around gaining custody, signing papers at the embassy, and traveling home with our precious new son. We are so overwhelmed and in awe of how our GREAT, MIGHTY, and SOVEREIGN God has provided for us and showered us with blessings beyond what we could have ever imagined. Thank you to everyone who has showered us with support and especially to those who are praying for and with us!! Please continue to pray and know we are immensely thankful!!"

October 2016:
-After a little over 2 YEARS of waiting, Jaemin Paul (family name and Paul in the Bible) DaOn (his Korean name) Van Andel officially became our son on October 14, 2016. WHAT an experience. October 14 has always been meaningful for Brett and I-it is the day we decided to begin our relationship as silly little youngsters at Dordt College. Now it has quite a bit more significance as it marks the day Jaemin officially became a Van Andel. Brett and I flew to South Korea on October 12, and gained custody of our boy on October 14. Here are some facebook posts of that monumental day, and the few days following, as we spent time getting to know our sweet boy.

"2.5 hours left and counting. I got 10 hours of sleep(!!!), and we are now drinking our triple shots. We are going to eat some breakfast, spend some time with our amazing God who made this all possible and then head to Eastern to "collect" our precious boy. Jaemin, daddy and mommy are ready for you buddy!!!"

"Brett and I feel immensely honored and overwhelmingly grateful to FINALLY introduce our SON, Jaemin Paul DaOn Van Andel to you all! What a crazy, intense, beautiful, heartbreaking, emotional 24 hours it has been. Jaemin has been amazingly brave and mature as he mourns the loss of his foster family and especially his beloved "Eomma" (mom). He has clung to Brett and I for comfort through his big, brave tears and we are very grateful that he has so willingly been allowing us to comfort him. He slept mostly through the night, only waking up to cry for his Eomma once. We are grateful for every prayer being said on our and Jaemins' behalf and ask for them to continue!! Please add Jaemin's foster family to your prayers as well-they were heartbroken to let him go and we saw very plainly the immense love and adoration they have for this little boy. We are off to breakfast and coffee and another full day of getting to know our new son!"

'"For you created my inmost being; you knit me together in my mother’s womb. I praise you because I am fearfully and wonderfully made; your works are wonderful, I know that full well. My frame was not hidden from you when I was made in the secret place, when I was woven together in the depths of the earth. Your eyes saw my unformed body; all the days ordained for me were written in your book before one of them came to be."

God knew from before you were born, Jaemin, that He was going to gift us with you. We are so thankful. You are exactly who God wanted you to be and exactly where He wants you. Praise HIM from whom all blessings flow."'

"This kid is making astounding progress with us. Although he did not want anything to do with non-pajama pants today, (who can blame him??) he allowed us to put on a shirt that we brought along, which he hasn't wanted the past two days. He's ok with diaper changes now, which is a big difference from day 1. He didn't want ANYTHING to do with the pool the day we brought him here, but today he jumped right in! Praising God for all of these seemingly small steps in the right direction, which I know are NOT small steps for Jaemin. Keep the prayers coming!! Emri and Abee, daddy and mommy miss you SO much!! Can't wait to be home and all together!"

And then, just like that, we were home...

It has been a journey to bring our little buddy home, but a journey well worth traveling. God has provided every step of the way and we are so grateful for His mercy and provision throughout this last year! Jaemin is doing SO well adjusting to a whole new life, with a whole new family, in a whole new environment. So many blessings, challenges and lessons have already come out of the last two months of this new adventure with Jaemin. Praise God!

Continuation of October, 2016:
-Abe turned 4!! Our buddy is growing, growing, growing! Not only is Abe growing in size (he's a big kid!) but he is also showing that he is growing in maturity and knowledge of God! We love our not-so "baby Abee"!

November 2016:
-Brett turned 32, and Jaemin turned 3!! They share a birthday of November 19. Jaemin LOVED his firetruck birthday party:)
-We took another family road trip to Colorado to celebrate "thanks-Christmas" with Brett's family! Our trip there went great and the kids were SO good in the car. When we arrived, we had a few days of fun before Emri ended up in the hospital for 6 days with pneumonia and a partially collapsed lung. It was another week full of unknowns, scary fevers, a very sick, miserable Emri, and confused doctors...but it was also a week of blessings surrounded by family love and support. It was strange feeling being back at the same hospital Emri was born in. The last time we were at U of CO hospital, our baby Emri was 4 lbs 11 oz. and we didn't know anything...anything about what was really going on inside of her little body, anything about marfans, anything about being parents...It is just astounding to look back and see where we have been and how far we have come with our sweet girl.
We got out of the hospital just in time to celebrate Christmas with Brett's family and left the next evening for home. We bought three oxygen tanks to take on our road trip, since Emri's lungs were still compromised and we were going to be hitting some thin air over passes. Emri's oxygen level (with a little help from the tanks) stayed above 90 for most of the trip and we were grateful that we were blessed with a fairly uneventful ride home.

December 2016:

Well, here we are December 2016. Reflecting on 2016 while writing this blog has included so many emotions on my part. I have quickly listed the events of our 2016, but I haven't even touched on the heart changes, life lessons, and immense spiritual growth that God has led us to and through this year. God has been refining us in so many ways this year...healing and growth in our marriage, major realizations and repentance of sin still present in our hearts, shifts in parenting perspectives (still learning every day...obviously), and now learning how to navigate life with 3 kids-which is no joke, by the way. Brett and I have ended this year with the realization that we have too many commitments and too little time together as a family. We are slowly filtering through all of our commitments and attempting to eliminate what isn't necessary in an attempt to savor more time together as a family, and pour in the time and energy into prayerfully teaching our children and leading them by example. We pray earnestly that the Lord calls each of them to be His own, and that He uses us as vessels to point our kids to Christ and His saving work on the cross, which we realize means that we need to be available to be used-and not too busy and distracted to be the parents we feel God is calling us to be.

I hope and pray that this Christmas season brings everyone back to the only things that matter...back to the humble realization that we are here for the sole purpose of bringing glory to our Father above, who sent His ONE and ONLY son to this earth, in the most humble form to live among men and to fulfill His purpose of taking our sin upon him as he bled and died on the cross, and then rising again on the third day! Christ fulfilled God's wrath upon men, so that we can now call upon God to save us and accept the gift of eternal life in heaven with Him. THAT is our ONLY hope! Without this hope we are lost and destined for eternity without God. I hope and pray that all of you reading this have, or will find this HOPE! The joy that follows is inexpressible. Merry, merry Christmas to all of you and congratulations on making it through this 3 mile long blog! I hope to write more often so that these don't have to happen any more! Until next time, Erin.

Tuesday, January 12, 2016

Updates in twenty 16

Some days are just more happy than others. Today was a two-appointment day at Seattle Children’s. We started with a lung function test…Emri’s first LFT. We are taking the results with a grain of salt, because of the fact that this was her first time, and she didn’t necessarily blow as hard as she could-but from what we gathered Emri has low lung volume. Expected??? Yes. A bummer?? Of course. There is always a little sparkle of hope in me that maybe, just maybe, one test could come back and be surprising in a pleasant sort of way---but alas, her lung volume is indeed quite a bit less than the “average” 5 year old. The lady completing Emri’s tests chalked it up to her pectus interfering with lung growth, but we will have to see what our Baltimore pulmonologist thinks of it all.

Next was the echo. This echo came 5 months after the last one, which, because of my poor scheduling abilities, is the longest stretch of time that we have had in between echos. I was watching the screen intently waiting for the aortic measurement to take place, and I saw it…3.2. According to the latest in mathematics, 3.2 is larger than 3.0. 3.0 is the last measurement I remembered Emri getting. I immediately felt the good ol’ kicked-in-the-stomach type nausea overtake me and attempted to restrain my emotions until we could hear the results from the doc. Throughout the echo, Emri continued to ask me questions about how her heart looked. She looked at the echo screen and at my face a lot. It killed me. She knows stuff now. She has been asking a lot more questions lately. What happens if her heart doesn’t look good?? Does she need “shurgery”?? What will they have to do to her if she has a “bad” heart? These kind of questions, and the concern in her face during the echo are emotionally more than I feel equipped to handle. She’s FIVE and she gets it. When she was much younger, I often imagined her much, much older asking questions and figuring “it all” out. As much I know that this is all a great opportunity to share the good news of God’s sovereignty and how we don’t need to worry about tomorrow, it is still so, so hard. I am teaching my daughter concepts that I didn’t even remotely grasp until I was 22. Because of the life Emri has been given, she will be faced with the tough stuff from an age that most kids receive the privilege of dwelling solely on what flavor and shape of birthday cake they want each year (which she also dwells on often enough).
When the doctor came in he asked all the “normal” questions. Where is Emri’s energy level at?? I always answer, “above average”. She IS the energizer bunny. Any new meds? Nope. After a few more questions, I just asked about the aorta.

“I saw a 3.2.”

“Yeah. Her aorta is big. It’s the biggest I’ve seen in a kid that age…well, I’ve seen one other kid with one that big. It’s really big.”

 Aaaaaaaaand, that folks, is why we are seen at Hopkins.
I am very, very much aware of the fact that her aorta is, in fact, big. Not only is it big, but it’s growing. We are, of course, sending the echo to Dietz to get it read by him, but we won’t get it there for another 2 weeks because we have to send it by CD. I am hoping that he doesn’t see as much change and we saw today….but it could happen. We will prayerfully await an answer.
SO that was today. I got in the car and let the tears flow. Sometimes this is SO overwhelming. But as the drive went on Brett encouraged me, as he always does, to think on the good. Emri’s new rods…AH-MAZING. Emri’s alive, energetic, and full of songs. We have put off heart “shurgery” for 5 years so far…which is great! And the best part…GOD IS SOVEREIGN…and he works for the GOOD for us that love and serve him.

I really, really try to remain focused on these things. But sometimes sadness just takes over….and I don’t think it’s necessarily a BAD sadness. It’s sadness over Emri finally beginning to understand that this life isn’t so easy. It’s a sadness over suffering. I shed tears knowing that Emri’s body is frail and doesn’t work the way it should. When the sadness becomes wrong is when I begin feeling sad for things that haven’t even occurred yet…which is actually fear. This is when I am reminded, quite often, of Philippians 4:8,

Finally, brothers, whatever is true, whatever is honorable, whatever is just, whatever is pure, whatever is lovely, whatever is commendable, if there is any excellence, if there is anything worthy of praise, think about these things.

I cannot be worrying about things that aren’t truth. Right now Emri doesn’t need heart surgery, so I don’t need to dwell on how horrifying heart surgery would be. It has been my largest challenge, by far, on this journey to not dwell on the unknown of the future. But dwelling on things that are not truth is not honorable to God. So tonight I will dwell on his promise, once again, of that day when there will be no more suffering. That’ll be the day.

As for other life happenings…oh my goodness…the drama never ends. Brett has a new job. ANC design, the business we opened last year did GREAT for the first year it was opened. THEN came the dry spell. NO WORK for weeks. In a very round-about, unmistakably God-orchestrated way, Brett got hired in Lynden at “Elements” as “Director of Architecture and Interior Design”. A GREAT job and a steady paycheck. God just continually provides and we have no reason to ever doubt that he WILL provide in our times of need. Brett is commuting to Lynden and back daily. Does this mean we are moving AGAIN??!?!?!? I DON’T KNOW. And frankly, I don’t really want to talk about it.

Homeschooling is hard. I only have one student. Her name is Emri and she is stubborn and doesn’t love sitting in one place for longer than 4 seconds. We are making progress…ever so slowly we are making progress. Emri is not missing any crayons in the box (if the "box" were to signify the "brain", and "crayons" were to play the role of "smarts"), and she gets bored rather easily. So it has been my job to balance making sure she isn’t just being lazy, not wanting to do work, and making things more fun and less monotonous. She is a great student, when motivated, and we shall see how the remainder of the year goes.

Abe is just a cool, cool kid. He also does not lack energy and Brett is anxious to get him in a soccer program come spring. Once in a while I just make him do laps around the house in an attempt to get out some of the excess energy and spare the rest of us from getting jumped on and trampled by his bursts of fun. He is FULL of amazing quotes and regardless of what it may look like at times, he LOVES his sister dearly. Emri and Abe are playing well together for longer periods of time, which is wonderful for me getting a few things done around the house…like playing with my niece (what dishes??)…which brings me to my next point of discussion.

Daniel and Steph have inhabited our living room because of a horrible mold problem they had in their quaint little cottage behind our house. Daniel is planning on addressing the mold soon, but for now I thoroughly enjoy the company of my sister and Felicity in our home. Felicity gets cuter, stronger, and more stubborn every day. She is so much fun, and the terrific part is that when she gets cranky I get to hand her off to my sister;) Ahhh, the perks of being an auntie. But really though, having them in our home has been a delight and I will miss the constant companionship and baby giggles when they move back across the driveway.

We heard from our adoption agency that South Korea has FINALLY requested our paperwork, but don’t get TOO excited-there is still anywhere from 6-11 months to wait for our court date. Our littlest man turned 2 on November 19th, has seen pictures of us and is doing well. I can’t help but think that him and Abe look like brothers. I know, I know, it’s weird…but I haven’t been the only one to notice. In addition to information on his daily bowel habits (all good by the way), his last report discussed a little about his “stubborn nature” to which I replied, “yep, he’ll fit right in!” He is just meant to be our little boy and I cannot wait to bring him home and shower you all with an irritating number of pictures. He is so darn cute.

Well, my brain is begging to cease thinking for the evening and the kids are now finished watching “Bolt” so I must now give kisses, hugs, snuggles and sippy cups to my littles. Thank you all for taking time to work through this novel and for the ongoing support you give our family. Oh! And if you didn’t get a Christmas card this year it’s because we DIDN’T DO ONE. But we still hope you had a Merry Christmas, even without our card. I refuse to not do a family picture and card this year, so you might just be getting a “Happy Valentines day” card or a “Welcome to spring” card…perhaps a “Happy summer” card….or maybe just two Christmas cards next year. But mark my words, we will get a family photo taken before 2017.

Love to you all.


Wednesday, August 12, 2015

It's "Magec"!

Brett and I are PRAISING THE LORD for a much more peaceful experience putting our dear girl to sleep this morning. We had talked to Emri about getting an IV to go to sleep instead of the dreaded mask, and she agreed to try it out. Emri already had three little bruises on her arms from her blood draw yesterday (her veins are nearly impossible), and she was SO brave getting her IV in this morning. One of the nurses came in and was shocked to hear that Emri had chosen the IV...she said that they never have five-year-olds willingly get poked in order to avoid the mask...but Emri just hates it that much. Emri was given Versed shortly after her IV placement. She was sitting on my lap, and became more and more limp as the meds kicked in and helped her relax. By the time we were ready to wheel her back to the OR, she could barely lift her little eyelids, and was working hard to put simple sentences together. We walked into the OR and for the first time, she did not cry, and she didn't look scared. WHAT a different experience than the last 5 times we have done this! I promptly asked the doctor why parents weren't offered this miracle drug while we sit and anxiously wait for surgery to be over! SO here we sit, once again, waiting for our updates from the OR, and praying,,,always praying.
We came to Baltimore planning on another Spinal rod lengthening surgery. We weren't planning on upgrading Emri to magnetic rods because last year when we asked about them, we were told she was still too small to get them. When we got here and had our pre-op appointment, we learned that Dr. Sponseller is now switching the majority of his young patients to magnetic (magec) rods. After a lot of questions and conversations we decided that magnetic rods are really the best path at this point for Emri. These rods really are "magec" because they are able to be lengthened WITHOUT SURGERY! That means that Emri will no longer need surgery yearly to lengthen her rods! We will need to be seen in Seattle 3-4 times are year for external, magnetic rod adjustments. We will still need to be seen yearly at Hopkins by Dietz (heart), Sponseller (back), and Neptune (lungs). But we are hoping that maybe we can be seen at the Marfan conferences and make that our visit if all three doctors are present. 
The reason we were contemplating NOT going through with the magec rods was because with magnetic rods, Emri will be unable to get MRI's. If she were to need an MRI, we would have to resort to a CT scan. We foresee Emri eventually needing cardiac MRI's if her heart worsens, but after speaking with Dietz yesterday, he does not foresee the need for cardiac MRI's in the very near future. We are hoping and praying that Emri's heart remains stable until she is fully grown, at which point the megec rods can be removed and she can officially receive a definitive spinal fusion (and would be able to get MRI's again). If her heart does progressively get worse while she is still growing, we would need to resort to cardiac CT's, which doesn't thrill us due to the radiation exposure...but we all agreed that the benefits of less surgery far outweigh the risks associated with yearly surgery. 
While I sit here waiting for news, I thought I would take the opportunity to update you on the latest with Emri. I will start from the top of her head and move down! 
I think most of you remember Emri's temporal skull fracture that occurred last fall when she fell on our driveway. After seeing ENT at Children's twice, it has been concluded that because of scar tissue build up in her ear, Emri has lost more than 50 % of her hearing in her right ear. This will need to be surgically repaired some time in the near future, or she will need a hearing aid. 
We saw the eye doctor at Children's about a month ago and he gave us good and bad news. The lenses that are balled up and sitting in her line of vision are slipping down covering the bottom 3/4ths of her pupil. She was given a prescription of -21 glasses since her vision is so severely distorted. The good news is that the eye doc was able to see that in the top 1/4th of Emri's pupil, Emri's refraction is +11! Consequently, if Emri's lenses slip all the way down, she will have a much more manageable +11 prescription! The other good news is that since Emri's lenses are slipping down, the doctor is not nearly as worried about pressure build up behind the eye, AKA glaucoma. Sigh of relief! 
Emri's lungs have been doing quite well since her bout with RSV and pneumonia this winter. We saw Dr. Neptune yesterday here at Hopkins and she was pleased with how Emri looks. Emri will be undergoing a lung function test and sleep study in the near future to get more information on how her lungs are functioning. Emri continues to get 02 at night and we will revisit her sleep apnea issues after her next sleep study. We need to keep a close eye (and ear) on Emri's lungs to make sure that her lungs are able to function properly despite her chest wall deformities and the rigidity of her spine.
The wonderful Dr. Neptune!

We received our annual Dr. Dietz picture and hug yesterday and also celebrated a STABLE heart report. Emri's aortic root size increased by an impeccably tiny amount, which Dietz was unconcerned with. Her valves are all leaking, but her heart function still looks good. It was a refreshing appointment! I have to say that I actually know what to look for on the screen while Emri gets her I already knew the aortic measurements before we got the report. Proud moment for this non-medically minded mother (who used to faint just walking into a hospital)! God's funny, isn't He???
"Dr. Dieeeeeetzzzz...are you out there???"


Funny side note...we had our pre-op anesthesia appointment yesterday, and while reviewing the medication list, the anesthesiologist says, "So I know this can't be right...what dose of irbesartan is your daughter on?? The dose in the computer is way too high!" Nope. Right dose. Our 27 pound Emri is on quite a heavy dose of irbesartan and it seems to be doing it's job! 
The lower half of Emri's body seems to be much less complicated than the top half! Emri is walking like a pro-and even RAN, like literally RAN on the treadmill the other night. She frequently shows off her brace-free walking and asks, "mom, aren't you so proud of me??!?" I AM SO PROUD EMRI!!! So proud. And SO thankful for the strength, mental and physical, that the Lord has provided for our girl! 

We left the three day marfan conference on Sunday afternoon and once again, are so thankful to have received the opportunity to go! Such a whirlwind of a weekend. We got to meet friends from the UK that I have only ever known on facebook...such a wonderful experience! We made some new friends, and met many fellow believers at this conference. We also attempted to absorb all the new information available and get questions answered that we have been waiting to ask all year. It was truly a blessed time.
Ready for the welcoming reception at the conference.

Isaiah Austin!

Our buddy Donovan!

Austin Carlile (Of Mice & Men)

Our Friends Soo and Toby from the UK!

Emri up on the screen. Still amazes me to see this!

Isaiah Austin's mom

Stuffing "Jenna" with new friends!



Catherine's mom-first time meeting each other after years of facebook communication!

victory is...

ipad watching with Toby!

New friend, Hope!

We've seen Peter at every conference:)

Marfan moms!

New friend Katie!

Donovan again:)


This morning as Brett and I walked through the hospital hall to the surgical unit, we were greeted by a mother that I have known on facebook for years. Her sweet, brave little girl, Ellie, has broken her spinal rods multiple times, and is in the hospital for 21 days for an infection after her recent rod replacements. She was waiting for us because she knew we had surgery scheduled for this morning! We were greeted with hugs and prayers...what an amazing family we have found in the marfan community...our "marfamily". Truly a community that loves, cares, and even prays for each other. 

I know this journey is a difficult one...the worst form of torture is watching your child suffer over and over again. Emri has been through more pain and nightmarish ordeals than I like to recall...but we are so blessed. Emri has a mature soul and loves Jesus and Heaven more than anyone I know. She has met so many kids that suffer with her, and shows empathy to these kids. Emri just met a little girl named Samantha in pre-op yesterday. This little sweetheart was non-verbal, blind. She had a trach, and a g-tube, and was pre-op for spinal rods. Not only did Samantha's mom offer to pray for Emri, but she also gave Emri a bracelet from Samantha. Emri wasn't afraid of this girl...she felt for her. She was concerned about what this little girl was going through and wanted to make her smile. It's a hard life, but a beautiful one! I have said this many times before, but Emri is a constant reminder of what is really important in life. We are here to glorify our Savior! God promises to work in the lives of His sheep for their good, and He tells us that suffering refines us and brings us closer to Him! Emri knows pain, but she knows JOY even more. I learn from that girl every day. She knows suffering, but when she isn't suffering, she is the happiest. We are so blessed. This is our life, and we give God the glory. 

Thank you for reading this update, and stay tuned for more news as we get it! Keep the prayers rolling!