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 things....cleaning, 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 winter...it 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. Dietz....so 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 lighter...like 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 non-refundable...so 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 to...so 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!
