Miriam’s first VEPTR expansion will be performed this Monday morning (June 24) by Dr. Williams. We apologize for neglecting to update the schedule here for those keeping her in your prayers (thank you!). The procedure was moved up because Miriam has been having some discomfort and irritation from one of her devices near the spot where her skin broke down in March. At implantation Dr. Williams intentionally left the VEPTR that anchors into her spine in her lower back longer than usual. (When she gets tall enough it will need to be exchanged for a larger size rod, but this extra length should help delay that larger surgery.) However, this left a tail end of the rod that protrudes noticeably under her skin. It has continued to irritate her from the inside, even after Dr. Shapiro shortened it in March. Over the last month it has developed a bursa – a fluid filled sac – and Dr. Williams decided to go ahead with her expansion to remove the irritation. He will move the extra length of rod up rather than lengthening the device, so the end should be flush with her spine and no longer protrude.

Dr. Nowlin is also scheduled to be there for the procedure, to scope Miriam’s throat and make sure there is no trace of the fibroma present. She has thankfully had no obvious symptoms, but we are glad he will be there to make sure her throat is healthy before they begin. He will also have a good baseline for comparison if (God forbid) the brief intubation required for this procedure causes anything to grow in her throat again.

Dr. Williams has told us that Miriam will likely go home Monday night, barring any unusual complications from the surgery. She should be back to herself in just a few days. Please pray for a straight-forward surgery, an easy recovery, and protection over her throat. Thank you for walking this road with us.

Miriam’s ENT performed a third endoscopy on her throat early this morning, with mostly positive results. There was some growth on her throat in the same spot as he has previously removed fibromas twice before, but it was much smaller (even after seven weeks between excisions). It was clearly a lump running vertically along the wall of her throat, whereas before the masses were growing out into her throat and blocking her airway. Dr. Nowlin said this type of growth is much more typical of an “irritation fibroma,” caused by an over-reaction to a stress to the throat – in Miriam’s case, the breathing tube from her VEPTR implantation surgery. He is not ruling out the possibility of the mass growing back once again, but overall it sounds like this is much closer to the expected progression of similar cases and that is reassuring going forward.

Dr Nowlin does not anticipate needing to see us again before Miriam’s expansion surgery in August. He will be in attendance then so that he can scope her throat while she is already asleep for the expansion. If anything is present, he will be able to remove it at that time. It will also give us a good baseline for the health of her throat before it is subjected to another breathing tube. Thankfully, this surgery will only require a breathing tube for a couple of hours at most, rather than over two days like the implantation. Her risk for injury or irritation should hopefully be much lower this time around.

We are greatly relieved that the growth seems to be slowing, and hoping that it will heal itself completely this time. Thank you as always for your prayers.

Miriam went in today to scope and, if necessary, and excision of any cyst which had formed on her vocal folds. In the 18 hours prior to this morning her voice had begun to make extra noises again. And her breathing was much more noisy than the previous nights since the last excision, but it was not nearly to the level of the weekend before the last one.

We started 2 hours late, but Miriam was once again in very good spirits. She is really used to all of these routines, is very cooperative, and is very smart / aware of what is going on. She was very tired though.

Her anesthesia doctor geeked out over the VEPTR a bit. :)

Dr Nowlin was not sure what to expect going in. The initial cyst was unusual: this type of cyst is generally caused by irritation from long intubations, much longer than she has had. He did not expect any regrowth.

After the procedure, he reported that the cyst was returning but about 20% the size of the first time. He is extremely puzzled – he’s never seen a case like this and he’s been doing this a long time.

Miriam has a prescription for an oral steroid (basically flonase in an inhaler) to help control inflammation to hopefully reduce any potential irritation. Using the inhaler should prevent any issues with recovering from the VEPTR-related procedures and keep her immune system up.

We will take her back in if she has any of the same symptoms again. Otherwise, her ENT will be able to scope again during her VEPTR adjustments. This will avoid extra anesthesia and will allow them to have a reference point if something else comes up after the next procedure.

St Blase, pray for us.

Miriam is feeling pretty good and recovering well:

Before Miriam goes in for her throat scope and (excision maybe?), here’s a happy update. We posted pictures of Miso’s back before the installation procedure. Here are the same postures afterwards.

You can see the major improvements in her posture.

The bumps in her spine are gone (note the lack of the shadow to the left of her spine). There are a few new bumps from the device (under her right shoulder blade, and in the lumbar area in particular). Her abdomen is also noticeably longer, though this is hidden by the higher-waisted pull-up she is wearing in the after picture.

You can also see in the lowest part of the picture the more fresh wound/scar where they shortened/adjusted the rod after her skin breakdown. The actual opening where the skin breakdown occurred was to the right (a faint horizontal line). Hope that’s not too gruesome for the internet…

Miriam had a follow-up today with Dr Williams to remove the bandages and the staples from her lower back after last week’s unexpected surgery to re-close the extra wound caused by the rod pressing against her skin.

Everything looks very healthy, and Miriam has been complaining much less about her back hurting. She has to wait until next week to go back to her ballet class and two weeks before she can take a bath again, but other than that, she is good to go for a while.

Also, whereas she cried at her first x-rays, she was very concerned today she would leave the office without having one done. Luckily for her, they wanted to double check that everything was where it should be, and it was.

Miso just drove her little car back into the surgery area. No separation anxiety and not worried by the environment or all the nurses. Anesthesiologist is actually the same doctor as on Monday, which is good since he is familiar with the issues around her breathing/vocal folds and can make sure to work around that.

Everything went really well with the procedure. They shortened the extra length on the rod to help protect this area in the future and stapled her up.

Also said her throat looked good, with no obvious evidence of Monday’s procedure. She’s not complaining of any soreness now.

We’re with her in recovery now, finishing off some apple juice.

For the second procedure in three days.

Miriam woke up with blood on her sheets this morning. The skin over the prominent lower end of the rod attached to her spine has broken down. It was probably a spot where a knot in the stitches dissolved and left a blister or abscess which was irritated by the rod. It is not directly on the scar from the original incision, which is actually good.

Her doctor is unfortunately out of town this week, but we took her in to see his partner who also does VEPTR procedures. He was relieved to see that it was not as severe as it could be / often is. We were able to get her in early since we live in town. It looks very clean and the skin around the new wound is thick and healthy.

At 5:30 she will go under and the doctor will clean up the wound and make sure it looks healthy inside. Then they will re-close the new wound. Because of the procedure on Monday and the infection risk, she will have a 23 hour monitoring period. that means we will be overnight in the hospital.

So now we are back in the PANDA at Dell Children’s. And this time she gets to ride in one of the cars she was so excited about after her first tour.

She is in very high spirits, and she is very excited about the white smoke and ringing bell videos.

St Francis, pray for us.

UPDATE: And then Miss Emily just happened to walk by and saw Miso, so now Miriam is jumping up and down very excited!

Miriam did really well with her procedure this morning. She was very brave and went back for the procedure with the nurses by herself, happily.

The cyst turned out to be much larger than when they scoped it a few weeks ago. It was not yet blocking her breathing, but probably would soon.

To give you an idea of the difference it made already, here is the girl herself.

BEFORE:

AFTER:

She was able to swallow and talk right away in the step down room.

Later in the day she excitedly proclaimed: “My throat is its self again!”

Thank you everyone for your prayers. We really hope this will be the last issue before her next adjustment.