Emri's toenails are currently pink and sparkly, just the way she likes them.
Emri still depends on her braces to walk any distance exceeding 30ish feet. She likes to show off and walk without her braces every now and then, but it's difficult and looks painful. She has NO calf muscles and doctors say that braces will always be a thing for her, because there is not much that can be done for her feet. Emri has been experiencing more and more pain walking with her braces on...most likely because as she gets bigger, and because she walks with her knees so bent, more and more pressure is being put on her chins and feet. Emri has been resorting to using her wheelchair when we venture out, and knee walks most often around the house, which, as you can probably imagine, is hard on her tough little knees. Part of the surgery being done as I type is a tendon release behind her knee, and guided growth plates along side of her knees. The tendon release is to make sure that tendon behind her knee is not a limiting factor in straightening her legs, and the guided growth plates are being put in to help her bones grow correctly as she continues to grow, so that hopefully, one day, Emri can walk with more ease and less strain on her braces. We are attempting to give her as much opportunity for independence and wheel-chair free roaming as possible....but we do know that there are other factors that could affect her need for the chair as well.
Emri still has her trusty old g-tube and receives 3 feeds a day through it following each "meal". I use quotations because Emri RARELY eats enough food by mouth to consider it a full meal. We like to describe her as a recreational eater...she dabbles in the art of eating. About a month before we came here for surgery we began following a weight gaining protocol that other marfan kids have had success with and hope to see her put on some additional weight soon. At 9 years old and 38 lbs., we have a ways to go for her to even be on the chart. We will make our first goal to gain enough weight to be chart-worthy! How beautiful that little dot on the chart would be.
Spine. We arrived at Hopkins last Thursday thinking that Dr. Sponseller would be tightening the loose screw on Emri's upper left rod. After x-rays on Monday morning, Sponseller noted that her rods were most likely running out of room to be lengthened and said that if we did not replace them in this visit, we would be back within a years time to have them replaced. It was SO HARD deciding to replace the rods now...but ultimately Brett and I felt like putting her through two separate surgeries would be less healthy for everyone and Sponseller felt comfortable doing all the procedures within the same surgery...so we went for it. When we told Emri that the surgery would be bigger than we originally thought, she was sad and there were tears shed. It's heart-wrenching to see her more fully comprehend these things and see the disappointment and fear in her face. For the past week I have just been in constant prayer, asking God to strengthen her and comfort her....to give her special peace and calm. There is just nowhere else to go but to the feet of the one who gives that peace that truly passes all understanding.
Lungs. Emri will always have restrictive lung capacity because her lungs are compromised by her chest wall (pectus), but our beloved pulmonologist, Dr. Neptune, was so encouraged with how Emri looked when she saw her this past Friday. She reviewed Emri's last sleep study with us and it was decided that we would move forward with getting Emri on a c-pap at night to help keep her airway and lungs open as the sleep study revealed that she is still experiencing moderate obstructive sleep apnea. Emri and masks are not friends...but we are praying we can slowly transition her from a nasal cannula to a mask without too many tears.
HEART. Always a GIANT sigh of relief to hear that Emri's heart looks stable!! And to hear it from our dearest Dr. Dietz, the man responsible for the research helping Emri's heart remain stable for so long in the first place, is just magical. Can't stress our adoration for this Dr. enough. Emri is very smitten by her Dr. Dietz and was giddy with anticipation to see him along with our genetic counselor, Gretchen. Before he came in the room, Emri HAD to take her hair out of her braids and make sure it was perfect for Dr. Dietz. It was really quite adorable. When he asked Emri if she had any questions for him, she asked him what his favorite colors were...so that the next picture she draws for him can include them all. My sweet girl.
Ears. You didn't know marfans involves the ears??!? Well, it doesn't. However, as some of you may recall, back when Emri was 4, she tripped and fell on our driveway and fractured her temporal bone, puncturing her inner ear. Emri has experienced moderate hearing loss in her left ear as a result of the fall and we have contemplated having surgery to remove scar tissue/repair whatever is blocking her from having her full hearing back as this corrective surgery was suggested by Seattle Children's a while back. We decided to have Emri seen here, at Hopkins, for a hearing test and evaluation by Dr. Tunkel in order to be able to perform a future surgery here, perhaps alongside some other surgery she may be needing. The hearing test was consistent with the findings in Seattle, but the advice was to leave it alone...which was cordially received by all three of us. If Emri were to be in an actual classroom, Dr. Tunkel suggested she get a hearing aid or have a surgery done...but her awesome homeschool teacher agreed make the necessary accommodations to help Emri learn well without need for interventions :) That's me...I'm the teacher.
Eyes. Emri's eyes are COMPLICATED. We are SO THANKFUL that there is a new eye doc, here, at Hopkins, that has specifically trained under Dr. Dietz to see kids with connective tissue disorders. Any friend of Dietz is a friend of ours. Emri was happy to meet Dr. Doyle and we were all very delighted with his English accent...an added surprise. Emri loves accents and always speaks in an English accent when drinking tea or dressing up. Dr. Doyle assessed Emri, and gave us the lowdown---I will try my very hardest to translate what we heard into human speak. Emri has balled up lenses that are obstructing her line of vision and greatly distorting how she sees the world, even despite her -21 prescription. We have been waiting to see if these lenses will move out of her line of vision on their own, and they do indeed appear to be going in that direction. Dr. Doyle said that the lense is right at the very edge of her line of vision in her left eye, and less out of the line of vision in her right. Apparently her vision will get more and more distorted until these lenses are completely removed from her line of vision. It is always an option to remove these lenses, but removing them comes with a much higher risk of retinal detachment, which isn't our favorite option. We are hoping and praying that these lenses will pack their bags and move out ASAP, but for now, we will keep her prescription the same and wait, wait, wait. If/when these lenses move out, Emri's vision would be greatly improved and she would probably be moving up to a +8ish prescription instead of -21...it's all pretty nutso. Despite all the goings-on in her eyes, Emri functions amazingly well. Dr. Doyle mentioned that these astounding marfan kids continue to be the educators, constantly kicking those silly "odds" in the pants. After Emri's visit, we warmly welcomed Dr. Doyle to Emri's Hopkins team and look forward to seeing him at our annual visits.
Brain. Emri is super smart. She's extremely artistic, loves math, loves listening to audiobooks since reading is super hard with globular lenses in the way, loves to play Lego with her bothers, and is absolutely smitten with her baby sister.
I think that about covers it. We shall call these blogs, "body blogs".
It's so hard. Being Emri's mom, my mind is SO wrapped up in all things Emri at the moment, which I feel like it needs to be...but I am experiencing so much guilt for missing my biggest boys' birthday and leaving my 7 month old babe behind. The boys have just been troopers through this all. There were many tears shed the night before we left and the morning of us leaving, but you could tell they were trying hard to be tough as they knew what had to be done for the sake of their sister, whom they both truly adore. We got to video message with Abe as he opened his presents and blew out his candles on the cake his grandma so sweetly made for him. My parents drove to Lynden to take the boys out for dinner and have cake afterwards. I am SO thankful for Brett's parents taking on the monumental task of watching all three kids and for the love and attention the kids are receiving from both sets of parents while we are away. I am missing those boys and my sweet smiley Eiley so, so much. It feels like we've been gone for months already. I almost asked a woman sitting with her baby this morning if I could just snuggle her baby for a minute, just to feel that warm little person lean against me and smell that sweet baby smell again....but then I realized that my request would perhaps come off as disturbing...so I resisted the urge. Anyway, the moral of the story is...THIS IS REALLY HARD. I miss my babies. But I have to fully be here for Emri. But I miss my babies. But I have to....yeah, you get it.
Well, I'm finished for now. For those of you that actually read through this all, congratulations and thank you. My sweet girl is still in surgery. I will try to update on facebook and this blog as we know things. PLEASE surround us in prayer like you always do so well!!!
