Archive for December 2017

Project for Awesome fundraiser

There are less than 24 hours remaining for voting in the annual Project for Awesome fundraiser on YouTube. Miriam and her sisters made a fundraising video for her hospital, Dell Children’s Medical Center of Central Texas. Please click below and vote for their video before Sunday at noon (less than 24 hours to go!). All of the money raised will be split between the most-voted charities. Please vote and share with your friends!

Click here for video!

Exchange of VEPTR for MAGEC, with an added surprise

Surgery yesterday began at 8 am and went until 1 pm. Dr. Williams started by checking on the rib-to-rib VEPTR, and discovered that the bottom anchor of that rod had also migrated through the bone. In other words, she hasn’t had either rod holding her up for a month or more. There were no good places to reconnect the rod, and obviously her ribs have not been collapsing without the rod’s support. He decided to just take it out. He left the upper anchor in for now, just in case her chest does begin to collapse and we need to put in a new rod.
He then removed the long rib-to-spine VEPTR, which came out easily.

Miriam’s VEPTRs after removal


They used an in-room CT to create an image of her lumbar vertebrae so he could find the best place to attach the 38-mm(!!)-long screws for her new anchor. He ended up using L3 & L4, which are lower than her previous attachments. He also added some bone graft material between the vertebrae to get them to eventually fuse together. This means her activity will be more limited than usual – no PE for four months, and at least six weeks of no ballet, with limited participation for a while if she’s able to go back. Her range of bending motion may also be more limited than in the past. However, the good news is that he was able to install the MAGEC rod, and her body tolerated all of the work really well. 


If everything holds in place, Miriam will go for in-office expansions with the magnetic remote every 2-3 months until the MAGEC rod is fully extended (28 additional mm). Dr. Williams expects this to take 2-3 years, by which point she may be grown enough to go through a final fusion. Because he removed the rib-to-rib VEPTR today, she shouldn’t have any need for surgery until then. The prospect of YEARS without surgery is thrilling, and we’re praying hard that everything holds so our sweet girl can finally get a break.

She was very uncomfortable and having some side effects from the meds for the first couple of hours, but after moving out of recovery to the IMC she began to feel better. She had a good night with a few long stretches of sleep. She had some tummy upset this morning from switching to oral pain meds, but otherwise she has done well. She moved out of the IMC unit to a regular floor, sat up in a wheelchair for over 20 minutes, walked to the playroom, and had a solid meal of mashed potatoes, strawberries, and chocolate chip cookie . She also got to pick a new baby from the toy cart! She was having some dizziness, so we decided not to rush going home. She’s currently asleep, and we plan to head home when she wakes up.

This is a very exciting moment – to have hope for the first time in five years that we won’t have to return to surgery next year. However, it’s kind of bittersweet. For most of Miriam’s life, she has been a VEPTR “rib kid.” Now she is a MAGEC kid (not that she wasn’t magical before), which is of course also very rare and special, but different. We pray everything goes well so that she doesn’t have to return to repeat surgeries in the future, but at the same time we’re so grateful to the amazing doctors who developed this technology that has allowed her to develop and grow like any other child. And we’re grateful to her surgeon, John Williams, who has helped her get this far (and who will continue to follow up with her MAGEC expansions), and to the amazing people at Dell Children’s who have always take such good care of her.

Speaking of Dell Children’s, Miriam and her sisters made a fundraising video for the hospital for the Project for Awesome happening right now! If you happen to read this within the first 48 hours of posting, please click here and vote for their video before Sunday at noon. All of the money raised will be split between the most-voted charities. Please vote and share with your friends!

It’s Never Simple


Miriam was originally scheduled for her regular six-month expansion today (Monday, Dec. 11). We went in for her pre-op visit last week expecting just to update health history and confirm a simple expansion of both rods.

You’d think we would know by now that it’s never simple.

This time, Miriam’s x-rays revealed that the lower anchor of her rib-to-spine rod (which is supposed to hook over one of her lumbar vertebrae) has become detached. And by detached, I mean that the tension in the rod has very slowly pulled the anchor through the bone until it has come free (in her doctor’s terms, it has “migrated” through the bone). It’s a surprise, but not a shock for a couple of reasons. First of all, it has happened before. The rod was originally hooked to the vertebra below this one, and after 18 months (Nov. 2014) it migrated through that attachment point. Second, the attachment point on her back has become really pronounced under the skin. It has always been noticeable, but it is really obvious now.

Because the hardware has pulled through two vertebrae in five years, her doctor wants to change anchors and replace it with one that screws into the bone of two vertebrae for reinforcement.This means the attachment point is going to be a little lower than it was before, which would mean extending the rod more than we normally would for just an expansion. This will shorten the useful life of this rod. Because it will need to be switched for a larger rod soon, her doctor is considering using this surgery as a chance to exchange her current VEPTR rod with a MAGEC rod. The great advantage of this would be that the MAGEC rod contains a rare-earth magnet mechanism that does not require surgery to expand. Instead, the doctor uses an external magnetic remote in his office to lengthen the rod in small increments. Miriam would need to visit the office for expansions four times a year rather than two, but this would mean far fewer hospital stays, less risk from anaesthesia, and much less risk for infection. She would still require surgery to expand her rib-to-rib rod as the MAGEC rod cannot be curved into the proper shape to replace that one, but her doctor is hopeful this would only need to be done once a year. The surgeries would only require one small incision, and that rod still has a lot of growth potential left.

We are currently waiting on the insurance to see if they approve the exchange to the MAGEC rod. It is costly, but the potential savings of avoiding even just one surgery will pay for the equipment. Whether or not her doctor does install the MAGEC rod, he will still be putting screws into her vertebrae for the first time. They have a piece of equipment at Dell Children’s called an O-arm, which is a highly localized CT that allows the surgeon to get images of his work area in the middle of surgery. It was unavailable today, so the surgery was pushed back to Thursday (Dec. 14) when he can use it to see exactly where to safely install the screws. She will need to stay at least overnight in the hospital because of the invasiveness of the procedure. If her surgeon also performs the exchange to MAGEC she may stay two nights.

Though this isn’t the news we wanted, we are continually grateful to Dr. Williams and all of those who worked to develop the VEPTRs and now the MAGEC rods. We can only imagine where Miriam’s health and activity would be right now if her lungs were still locked in the tiny cage created by her fused ribs. Miriam is able to live most of her life as a totally normal kid. She added jazz dance on top of ballet this year, and sang a solo in her school’s recent choir performance. She continues to love reading, writing, and doing all kinds of crafts and projects. Please join us in praying that she will be protected from further complication or infection through her procedure and recovery, and that she will bounce back in time for all of us to have a joyful Christmas together.

Miriam's choir solo