Miriam’s surgery went as smoothly as possible today. She was so happy and excited as we went through the pre-op process this morning. We thank you for your prayers and thoughts. We know it is the grace and strength coming from all of you that protects her from fear and keeps her heart joyful. And Dell Children’s Hospital is such a gift to us. Here she is modeling the new gowns they have today:

modeling the gown

Her surgeon was able to move the attachment point of her spinal rod up to the next vertebra so he did not need to exchange the device. Instead, he trimmed the existing rod a little and connected it to the new hook on the higher vertebra. This way he was able to get the usual expansion without having to adjust the length of the device. He mentioned that the other rod, connected rib-to-rib on her side, may need to be exchanged at her surgery in the spring, as it appears to be nearing the end of its expansion (although one more might be possible).

This afternoon she has had visits from her grandfather, her sweet Godmother, and her wonderful kindergarten teacher. She has a bag full of cards from her classmates, many coloring projects, a beautiful handmade bag full of crafts from her grandmother, numerous new toys, and six big balloons. No wonder this girl is always so brave for surgery – she knows the outpouring of love is always more than enough to cover the pain (and the drugs help, too).

We are spending the night to make sure pain is under control and then we should go home tomorrow. Right now she is sleeping peacefully so we seem to be on the right track for a morning discharge. As always, thanks for your love and support!

We have not updated Miriam’s page for a while, but as anyone with a health issue knows, no news is usually good news. Miriam’s last expansion in May went very well, with no complications. She had a fun and easy summer. The highlight was definitely when she and her sisters got to spend a week with their cousins at their grandparents’ house. They called it “cousin camp” and had a great time. Now she has started school! She is in kindergarten, enrolled in a dual language program where her classes alternate between instruction in English and in Spanish. It is challenging, but she enjoys it and loves her teacher and her new friends. Here is her school picture (we can’t believe how old she looks!):

Miriam had a CT scan in October to check the volume of her lungs. Her last CT was done just before her initial VEPTR surgery, so this gave us a chance to see how much separating her fused ribs and inserting the VEPTRs has allowed her lungs to grow. Her doctors were all thrilled to see that her lung volume has nearly doubled in the (almost) two years since her first surgery. The rest of her has only grown a few inches, so proportionately it is a huge change. It was a welcome affirmation of our decision to go ahead with surgery. While the corrections made to her spinal curve are a wonderful byproduct of the surgeries, it is the lung growth that should likely most improve her quality of life and possibly extend her life down the road.

Three weeks ago, we visited Dr. Williams for Miriam’s pre-op visit and got some disappointing news. Based on her x-ray, and the fact that rod bump on her back has gotten more prominent, he thinks that the hardware that attaches the long rod to her spine has worked its way through the bone and is no longer anchoring her VEPTR. This means that in addition to the planned expansion, he will need to move the hook which anchors the rod to another vertebra. We are hoping he can move the anchor up to the vertebra above and shorten her rod to match. If for some reason that is not possible, he will need to move the anchor to the vertebra below where it is now. He would then replace the entire long rod, as the one she has now would need to fully extend to reach the next vertebra and would need to be replaced at the next extension anyway. We are of course hoping that she can keep the existing rod as that surgery would be less invasive and have a quicker recovery time. We won’t find out what will work best until she is in surgery, so please pray with us that it is as easy as possible for her.

Surgery is scheduled for this Monday, Nov. 17. The girls are excited because their grandparents arrived today to help. Here are a couple of cute photos of Miriam and her sisters from Halloween. Thank you so much for keeping Miriam in your prayers!

Cecilia, Hildi, and Miriam

Halloween donuts

Miriam will be having her third expansion surgery this Thursday, May 8. She saw her orthopedist a few days ago, and he said everything looks like it has remained well in place. Miriam has been feeling good, with little complaint of pain or discomfort. She’s been actively playing like any other five year old, and is even asking to try some sports this summer once her recovery is over.

The surgery should be fairly quick and we should be able to go home the same afternoon. The first few days of recovery are always rough, so all prayers are greatly appreciated!

Here are some pictures of Miriam and her sisters just for fun: at Easter, and at the new Family Garden at the Lady Bird Johnson Wildflower Center.

Miriam saw Dr. Williams last week to check on her progress after surgery. He had lots of good news. The devices look secure and her incisions are healing nicely. Before surgery, the VEPTR on her right side had deformed the lower rib holding it in place. Without the device to prop her ribs apart, her right lung had shrunk significantly, and the curve in her back had increased to 24 degrees. Post-surgery, her lung volume has increased to match the left side, and the curve has decreased to 13 degrees. She is doing so well that he doesn’t expect to see her again until her next pre-op visit in May. You can see the expansion he achieved in the devices in the x-ray.

In other news, Miriam just turned 5! We had to postpone her official party because of illness passing around the family, but she still got to celebrate with her visiting Grandma and Grandpa. It is incredible to us that she has grown up so much so fast. Here are a few of her favorite gifts.

Her Uncle Joe gave her a knight costume for Christmas, and we have been calling her Miriam the Brave. Her other grandparents (Abeea and Papateaux) completed it by making leggings, a skirt, a banner, and a handcrafted sword. Now it doubles as a Joan of Arc costume!

She also got a new play kitchen. One of the benefits of her petite size is that it should be big enough for her to play with for a while!

Surgery went smoothly for Miriam this morning. Her throat surgeon checked her vocal cords and airway after she went under anesthesia, and to our great relief, everything looked clear and healthy. In the photo he showed us, there wasn’t even a sign of the redness caused by the reflux she was experiencing earlier this year.

After surgery, Dr. Williams told us that the rib-to-rib VEPTR on her side had definitely deformed the bottom rib where it was anchored, though he was not sure if the rib was actually cracked. This meant the device was no longer supporting her right side, and her chest had contracted significantly since her post-op check this summer. She was likely having a hard time getting enough oxygen in her lung in the collapsed space, which would explain her recent bouts of grumpiness and lethargy. Dr. Williams was planning on removing the long part of the VEPTR and replacing it with a smaller version, as it would need to attach to a higher and stronger rib this time. However, once he was in surgery he realized that the existing rod had already been extended, so he put it back to its original size and it was small enough to connect to a higher rib. This is great for two reasons: one, less new devices implanted mean less chance of accidental infection; and two, this device can be extended several times before it will need replacement. If Dr. Williams had needed to replace the device with a smaller model, it would likely have only been extended a few times before we would have needed to exchange it back to a bigger one (meaning another bigger surgery). After re-seating the device on the higher rib, he was able to expand it even beyond the capacity that Miriam had before the lower rib deformed. He also was able to extend the rib-to-spine device in the middle of her back by over a centimeter. Once Miriam recovers from the surgery, she should be full of energy again (because she’ll be full of oxygen)!

Dr. Williams tried a new dressing on Miriam’s wounds that places ice on the site and is supposed to help reduce the need for medication. This afternoon she has been uncomfortable, but with meds has at least been able to sleep. So far they are not allowing anything more than ice chips, and when she is awake she is hungry. She also loves the hospital’s system of letting the patient choose their meals off a menu and ordering over the phone, and she’s sad she can’t do it yet. :( Meanwhile, she’s already been given a treasure chest worth of toys and books from the various charities that take care of the kids here, and we’re looking forward to when she’s feeling well enough to play with them.

Thank you as always for your prayers and support. They mean so much to her and to us.

Miriam’s next VEPTR expansion is scheduled for Dec. 16. We were originally thinking it would wait until January, but she has been showing increasing discomfort and her devices started to appear more prominent under her skin in mid-November. This is in part because she has been growing (hooray!), and the devices may just be too small for her at their current setting. However, based on her x-rays her doctor is concerned that the VEPTR on her side may have eroded the bottom rib where it is attached. They won’t be able to see for sure until she is in surgery. If this is the case, they will need to attach the device to a different rib, which would likely be higher up in her chest. This would mean removing the current VEPTR (leaving the upper connector in place) and replacing it with a shorter one. This would mean a bigger incision and more recovery time (possibly two nights in the hospital). Please pray again for wisdom for her doctors and nurses, good pain management, and protection from infection or injury.

Just for fun, here are some recent pictures. She got a big hair cut just before Halloween. She was Little Red Riding Hood, and her sisters were Cinderella and Tinkerbell.

The folks over at the Room to Breathe Foundation and the Titanium Rib Foundation are celebrating “VEPTR Awareness Day” today across the country. They want to make sure all children who could benefit from this technology, as well as their parents and doctors, know that help is available. We will celebrate by sharing this video made just a few weeks ago to celebrate the 25th anniversary of the first rib implantation. The people speaking are some of the first to undergo the surgery, and their stories are heartfelt and hopeful.

Miriam is currently having a great time visiting her grandparents in Michigan. We’re grateful for this relatively healthy time, when she is able to play outside like any other kid.

This has been an extremely busy two months for our family, so we apologize for failing to post after Miriam’s first expansion in June. The surgery went beautifully. Miriam was really comfortable with the routine of waking early and checking in at the hospital. Her dear friend Miss Emily (the Child Life specialist) arrived in time to talk with her and escort her into surgery (with a dress-up game on her iPad, of course). Dr. Nowlin, Miriam’s ENT, began the procedure by scoping her throat to check on the polyp in her vocal cords. Though she had been having hardly any symptoms, there was a small lump in the same place as seen in the previous endoscopies, and Dr. Nowlin removed it. Then Dr. Williams took over and expanded Miriam’s two VEPTRs. He was able to get good expansion within the rib-to-rib device, and was able to move the spine-to-rib device up so that there is no longer any tail sticking out. This left her back much smoother, with hardly any protrusion of the end of the device. You can see the expanded devices and the improved position of the long rod in the x-rays below.

Miriam’s recovery was very fast. She went home from the hospital that afternoon and was back to herself after only a few days. It was a big help that Abeea and Papatoes, her grandparents, came to visit and take care of us. The adjustment of the spinal rod position has made a world of difference in her comfort level with the device. She used to complain of pain anytime she sat in a hard-backed chair or her car seat for more than a few minutes, and that no longer seems to bother her. Dr. Williams also got very good muscle tissue to close over the incision site so the scar is much more solid than last time.

We were very grateful for her quick recovery because shortly after surgery we made a huge life change for our family. We moved into our first real house! Until just a few weeks ago the five of us (and our dog) were living in a tiny two-bedroom apartment. All three girls were crammed into one bedroom, which was sometimes a challenge during Miriam’s recovery from her initial surgery. Now we have four bedrooms, with one currently in use as a playroom. If there are times in the future when Miriam needs privacy and quiet during a recovery, we can arrange it so she has her own room.

One of the best features of the house is the swing-set left by the previous owners. Miriam and her sisters love having a yard, especially with swings. Here they are playing with Papteaux just a week after the expansion.