18 months with MAGEC

In honor of Scoliosis Awareness month (although we’ve just missed the end of it), we want to update Miriam’s story to share how well she is doing with her MAGEC rod. It has now been over 18 months since the surgery where her doctor switched her hardware from two VEPTRs to one MAGEC rod. Miriam has had six expansions of the rod so far. The expansions take place during a visit to her orthopedic surgeon’s office, where a remote control containing a magnet is placed directly over the rod in her back. The magnet makes the magnet inside her MAGEC rod rotate, which very slowly expands the rod. Miriam always feels a little uncomfortable during the procedure and is sometimes sore for a day or two afterwards, but it’s a lot easier than recovering from surgery! Here is a video of her first expansion if you’d like to see how quickly it happens.

Miriam goes for expansions every three months. She started out getting three millimeters with each of her first few expansions, but the last three have all gotten less than 2. There is a safety on the remote that prevents it from pushing past a safe level of tension, and that has been stopping the expansion around 1.7 mm. It seems like that is the maximum her body can tolerate, and since her growth curve is on the small end her doctor isn’t concerned. As long as the rod can keep expanding, it is doing its job. And on the positive side, slower expansions mean this rod can last longer in her body, further delaying any need for future surgery.

Speaking of future surgery, Miriam’s likely next step in 18 – 24 months will be spinal fusion. Sometimes MAGEC patients swap out their rods for larger ones when they reach maximum expansion, so there is a small chance that could happen if it seems she still has a lot of growth potential left. However, her doctor has let us know that most spinal growth for girls is done by age 12 (the rest is mostly in the legs). If she can make it there on this rod it probably won’t benefit her much to keep trying to expand. The spinal fusion will be a major surgery. We haven’t gone over it in detail with her doctor yet, but given the complexity of her vertebral anomalies we expect her entire thoracic spine will require fusion. We’ll share more when we know more details, but feel free to start your prayers now!

There have been many achievements in Miriam’s life over the last 18 months. She has continued to excel in all of her subjects in school, including reading and writing in Spanish in her dual language classes. She recently completed the fourth grade and performed in her fifth ballet recital this spring. We just returned from a wonderful family vacation to upstate NY and NYC, where Miriam swam almost every day and fell in love with archery. We’ve now had two summers and a Christmas without sweaty bandages or healing incisions that need to be kept dry, and Miriam has enjoyed the freedom! Thanks as always for your thoughts and prayers. You support her and us through the hard times and the joyful ones, and she knows she never faces her challenges alone!





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THANK YOU from 10 year old miso

All of you have prayed for me, loved me, and cared for me so much that everything I do will not be enough! Thank you! ;) <3 Miso





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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!





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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!





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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

 

 





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Miriam’s Thoughts

I am collecting clean stuffed animals for cancer patients until the beginning of December (my next expansion).
Happy Thanksgiving,
Miriam





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Ready for recovery

2017-06-02 09.07.50

Miriam was released at 10:30 this morning. She woke up chatty and cheerful, and other than an unpleasant bandage change kept in good spirits. We took a quick trip to the playroom, and were able to attend a children’s prayer service in the chapel before heading home. She’s glad to be home with her sisters and grandparents and her own bed. Recovery will probably take a little longer than usual due to the size of her incisions, but the swelling on her shoulder has already disappeared.

2017-06-02 09.11.12





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Expansion and Cradle Exchange

Miriam was in a good mood as usual for our early arrival at the hospital this morning.

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After we got her undressed, we took a look at the spot where the end of her rod was protruding, and it was significantly more prominent than even a week ago. It is the oval-shaped lump on her right side, halfway between her spine and shoulder blade. (The smaller, bonier lumps on her lower back are also hardware attachments, but they always look like that and are secure.) We brought it up with Dr. Williams, who promised to take a thorough look at it in the OR.

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On the left, here is the x-ray from her pre-op visit to his office last week, with last year’s x-ray on the right for a comparison. You can see how much less bone is inside the top of the long rod in the newer image, and how much closer it is to the surface of the skin.

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After the operation, Dr. Williams let us know that her hardware had not come loose, but it had deformed the rib so much that the attachment cradle was aggravating the overlying skin. The swelling was a bursa (a fluid-filled sac) that her body was forming over it in an attempt to protect itself. He knew this wasn’t a sustainable situation, so he removed the cradle and attached a new one to the next rib down. This required some searching for a suitable spot, as the ribs in that area have largely fused together due to the stress of the hardware. Because the distance between attachment points was shortened, he was able to retract the rod a few notches. This means that instead of replacing the entire rod, we have actually gotten back more time with this one and we can wait longer before eventually replacing it. He was also able to expand her rib-to-rib device as usual.

Because of the need to remove and replace the cradle (and to find a new attachment point), Dr. Williams had to make a large incision around her shoulder blade. This was the biggest incision since her initial implant. Knowing it would be very painful, he attached an On-Q Pain Pump to the area. It bathes the incision area with anesthetic to reduce the nerve activity so less pain medicine is required. He let us know it would be attached for at least 24 hours, so we were in for an overnight stay.

I took the opportunity to thank Dr. Williams for listening to our concerns and taking the time to follow up with them in the OR. We have heard several horror stories recently of other kids similar to Miriam who have developed complications which could have been minimized if caught early, but which have turned into catastrophic events because doctors ignored parents’ concerns. After I thanked him, Dr. Williams answered, “I always listen to moms!” As any parent can tell you, especially of kids with special needs, we really do know our kids better than anyone else, no matter what their expertise, and we really appreciate having a doctor who trusts us.

Unsurprisingly, the recovery room wasn’t easy for Miriam today. She had a hard time getting comfortable, and the medication was giving her double vision for a while. Sprite and a grape popsicle made things a little better.

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Once we got up to a room at 2, she was ready for lunch. She ate normally, enjoying pasta and meatballs and Coke for a special treat. Soon we had visitors: her wonderful Godmother, than her amazing grandparents (visiting from New York) and her sisters. And of course, balloons and presents.

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2017-06-01 15.23.08This has definitely been a harder day one than most of her other procedures. She has struggled more to position herself, and despite being very tired didn’t manage to get to sleep until after 11. Thankfully she had lots of new activities and Mary Poppins on the DVD player to pass the time and distract her. The plan is to go home tomorrow after the Q-ball has run its course, as long as everything still looks good. I know she’ll sleep better in her own bed, and her sisters will keep her mind off the pain much better than a TV. She is hoping to make it over to the playroom here before we go, because otherwise our stay wouldn’t be complete.

Please pray for her total healing, and for her comfort over the next few days. We are so blessed to have her grandparents here to help, and her other pair of grandparents coming on Saturday. No matter what trials she endures, Miriam knows she is loved and in God’s hands. Thank you.

 





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Expansion #9

Miriam's First Communion

Miriam will have another expansion tomorrow morning. This one may be routine, which would mean a short surgery and an outpatient stay. However, her long rod has started eroding the rib where it is attached near her shoulder. The attachment point has become more prominent under her skin, but the rod does not feel loose and she isn’t in pain. Her orthopedic surgeon could not tell based on the pre-op x-ray how much it has moved, so he will be investigating during surgery tomorrow. If the attachment seems loose or in danger of eroding through the bone before the next surgery, he will go ahead and exchange the rod for a larger one and re-seat the attachment point on a different rib. In that case, she will be admitted for observation and stay overnight. If the attachment still seems secure, he will only expand the existing rod, and we will plan to exchange and re-seat the rod next time since she has nearly outgrown it.

Miriam is missing the last day of second grade for surgery, but she is ready to move on to third grade. She brought home literal piles of work from school over the last few days, and we are always amazed by her creativity and her maturity in writing. We’ve had lots of big events in the family in recent months: Miriam and her cousin Kate both received their first communions, we traveled to New York to witness the ordination of her grandfather as a deacon in the Catholic Church, and we attended the baptism of her newest baby cousin. God has been good to bring many joys to our family, and we pray Miriam will bounce back from this procedure quickly so she can embrace all the new adventures of summer. Thank you as always for your prayers and support.





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Adenoidectomy

Miriam’s last expansion in November went perfectly. She healed well and was back to herself in time for the holidays. Her health has been good so far this winter, except that she has begun to make a lot of noise in her breathing while she is asleep. Sometimes it has sounded like typical snoring; other times, it has sounded raspy and there have been strange clicks and pops. We are hyper-aware of any strange noises from her airway because of the fibrous granuloma that grew in her larynx after her first VEPTR surgery which nearly choked her to death. Thankfully it was removed and has not been a problem for the last three years, but as the strange noises continued we decided to take her back to the ENT for an evaluation. Based on some recordings of her night breathing, he decided she probably has enlarged adenoids and ordered an x-ray to check. When the nurse called with the results, she said that according to the doctor’s notes Miriam’s adenoids were not only enlarged, but “enormous.” So tomorrow morning she is scheduled for adenoid removal, as well as a scope of her throat to make sure there is no change in the area where she previously had that growth excised. Please pray that the procedure goes smoothly and her breathing and her sleep will be improved because of it.

In happier news, Miriam’s amazing imagination led her to win first place in the entire district at the second grade UIL creative writing competition. She never ceases to awe us with her brilliance and creativity, not to mention her fantastic attitude despite all of her procedures.

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