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!