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.
It’s hard to believe it’s that time again, but Miriam is headed in for her next expansion in the morning. She has done really well for the last six months. Her health has been great, she’s keeping up in growth with her younger sister, and she’s been active and energetic at home and at school. This surgery should be a routine expansion, with only two small incisions performed outpatient. Please pray to avoid any complications so she can bounce back in plenty of time for the holidays.
In absence of other news, I always like to share cute pictures to show what a joyful and peaceful life Miriam has despite the unique stresses of her life. Here she is receiving a Pride award from her school for her excellent behavior and attitude.
And here she is with her sisters, dressed up as Hermione Granger for Halloween.
Field Trip to the Thinkery
It’s hard to believe another six months have passed, but it’s time for Miriam’s next VEPTR expansion surgery. There has been very little medical news to share about her since her last expansion and exchange, because she has been feeling great! She has only complained once about soreness a week ago; this is so much better than some previous expansions where she has been aching enough to need tylenol for the weeks leading up to surgery. She has been feeling so good that she has been able to have a pretty normal seven-year-old life as she finishes first grade. She has been running laps around the track for her school’s running club and actively participating in PE. She performed in her third ballet recital, a version of Alice in Wonderland, where she was a white rose with her sister Hildi. (Cecilia was an excellent audience member.)
This surgery, scheduled for tomorrow morning (May 22), should be as simple and straightforward as possible. There will be a small incision over the middle of each VEPTR rod, the expanding device will crank each rod a few millimeters longer, and the surgeon will sew her up again. We should be home from the hospital just after lunchtime. Of course, prayers for her protection from complication and infection and minimal pain while the incisions heal are greatly appreciated. Thank you!
Miriam did great this morning throughout the check-in process. She was excited and happy to head to the hospital (she’s been looking forward to it for weeks), and even tolerated the “B’air” warming gown better than ever before. In surgery, her doctor expanded her spine-to-rib VEPTR a few millimeters as usual and exchanged her rib-to-rib device on her side for a new larger one. He had told us he was planning to open up her large incision (from the initial placement) in order to remove the old rod, but while in surgery he decided to try a less invasive technique. He created small incisions near the top and bottom of the rod, disconnected the middle of the rod from its rib connectors, and used a chest tube to surround the rod and pull it out. He then used the chest tube to place the new rod and secured it to the connectors. He told us to expect soreness from all the movement around and under the tissue, but we’re grateful she does not have a new huge incision to heal.
She had a rough time in the recovery room (which is normal for her; morphine makes her cranky and itchy), but once she got to her own room and got her lunch she started feeling much better. Since 1 p.m. she has eaten three large meals, gone for two short walks, and visited the play-room for some art time [well, mostly art-directing Mom doing her work for her]. She has had some complaints of pain but no worse than when she has had to have her connectors re-positioned in the past. She will see Dr. Williams one more time in the morning, and as long as everything is clear we should be home by tomorrow afternoon. She will probably stay home from school for the rest of the week until she feels good enough to get off narcotics. In the past she has switched to regular Tylenol (alternating with Ibuprofen) in just a few days, but her doctor has warned we will probably have a longer recovery time this time around.
We have not updated in a while, so we should add that Miriam is in first grade now, and will turn 7 in January. She qualified to enter into the Talented and Gifted program at her school, so starting in the new year she will get to choose an independent project to research and present. She loves to create things; as soon as she comes home from school she runs off to the playroom to start drawing or writing another idea. She has lots of friends at school, but her best friends are still her sisters and her cousins. She continues to learn ballet and started in the higher level class this year (though she will be missing the next several weeks while she heals). The last six months she has been blessedly healthy and care-free, and she has had a lot of fun. Here’s an example: her grandmother made all three girls Powerpuff Girl costumes for Halloween. We’re hoping she’ll get back to playing with her sisters like this soon. Thank you for all your prayers and support.
This is just a quick note to let everyone know that Miriam is having her sixth expansion surgery tomorrow morning (Monday, Nov. 30). There is a good chance she will also have her first exchange, where one device is removed and replaced with a larger size. The rib-to-rib device on her right side is nearly fully expanded and may not have enough room left to get the necessary growth this time, so Dr. Williams may have to take it out and replace it with the next size up. The attachment points on her ribs don’t have to be removed; they stay in place and only the rod between them is exchanged. This will be nowhere near as intense as her initial placement surgery (no separating ribs, no hole in the chest wall, no extended intubation), but if the exchange happens it will mean larger incisions and possibly incisions in new places. If she has the exchange we will likely stay overnight in the hospital. If her doctor just performs an expansion we should be home tomorrow afternoon. We’ll share a more extensive update after surgery. Thank you for your prayers and support!
Thankfully there is not much to report about Miriam’s surgery today. Dr. Williams expanded both veptrs about 1 cm and was done within 2 hours of Miriam heading back to surgery. We were able to bring her home before noon. She had a bit of a rough time coming out of anesthesia in the recovery room, but was feeling well enough to color and play gently with her sisters in the evening. She seems very happy to be sleeping in her own bed tonight.
She was excited to be at the hospital this morning, as you might be able to tell from this video of her waiting to go back:
The last six months have probably been the easiest and healthiest of Miriam’s life since before she started surgeries. She seems much more comfortable since Dr. Williams moved her long VEPTR up a vertebra at the last procedure. She has only complained of pain once or twice in the last month, which is a huge change from the lead-up to her previous surgeries. In the past, as we got close to time for another expansion, she has complained often of soreness around her devices and has acted worn out in the evening. This time, she still seems comfortable, and her energy level (even with the business of kindergarten) has been great.
Among her recent accomplishments, she danced in a ballet recital with her sister Cecilia. It was a production of Cinderella, and they danced as the snow angels that arrive with the Winter Fairy. We tried to take a picture of Miriam’s back to show off her scars in her costume, but they are hardly noticeable! We thank God for her body’s incredible ability to heal, and pray it will continue to recover quickly from each procedure.
Her next surgery is scheduled for Monday, May 11. This time her doctor anticipates a simple expansion on both devices. This is great news, as it means she will need only a small incision over the middle of both devices – just enough to fit the tool which is used to ratchet the devices out to expand the ribs. This surgery should be outpatient, and we should go home Monday afternoon if all goes well. We were able to do outpatient surgery just once before, and Miriam bounced back in only a few days. We pray it will go as easily this time, and as always that she will be protected from infection or other complication. She has shown almost no anxiety, though she is fully aware of the coming surgery and what it entails. We are so grateful for her peace of mind and heart, and we pray it will stay with her as she continues to grow. Thank you all for joining us in these prayers!
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!
Miriam was looking at all the cards and letters that you wonderful friends sent to her before and after her first surgery. She got an excited look in her eyes and said, “I have to write a letter!” After she finished, she said, “I don’t know who to mail this to!” I promised her I’d put it where everyone could see it. Thank you again to all who have supported her and prayed for her. We are so blessed to have you in our lives!
Here is the text in case you can’t read it:
“Dear all people that gave me cards at my 1st surgery
I will say a BIG thanks to evrey one of [continue] you. Espesley the ones that I know! <3 MIRIAM
P.S. I have another surgery on November 17th.”
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