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Billy's CRS/VACTERLS Journey - six to present

Billy's Spine Page
Billy's fusion surgery
Billy's Leg and Foot Page
Life with a fixator
Make a fixator cover
Gastroparesis and Billy's GI story
What is CRS/SA - Caudal Regression Syndrome/Sacral Agenesis?
Littlest Heroes Project photos
Make A Wish trip pictures
2011 VACTERLS conference
2009 VACTERLS Conference
2007 VACTERLS conference
CRS gathering January 09
Old updates
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From six years old to present - how we got here

June/July 2006 - Good news is that the MRI and bladder function testing done both look good, so no spinal cord surgery is needed now.  Testing for pulmonary function and cardiac followup to get ready for the surgery next month went well, and we were cleared for surgery.
August 2006 - Had preop appointment for surgery, found out that NS wanted to meet with us, as he was having second thoughts about Billy not needing to be detethered prior to the surgery.  Met with him, and he said that after talking with ortho, due to extent that spine would be moved and the neurological symptoms that Billy has shown during growth spurts, he felt that he DID need to go in and detether him prior to the spine surgery.   Had spine surgery and detethering on 8/18, all went well.  NS said that it was a good thing we detethered since Billy was very tightly retethered with scar tissue.  Ortho straightened spine nicely and stabilized pelvic junction, putting in 16 pedicle screws and two rods.  Billy was in the hospital 6 days, the first night in the PICU.  He had neuro pain down his right leg after the surgery from the detether/spine manipulation, and had to take neurontin for several weeks till that calmed down.  That put his walking off for a bit, but by about two weeks postop he was up and about again. 
September 2006 - 3 week postop check with ortho, all looks good.  Removed steri strips from back.  Xray shows bone healing nicely, everything in place.  NS postop check things look good also, can start to wean off neurontin.  6 week ortho postop check removed staples, things still look good.  Struggling with bowel management program, and the bowel nerves seem sluggish and are requiring a significant increase in laxative to work as well as usual.
October 2006 - Removed cast and was fitted for a Wilmington TLSO brace.  Brace fits well and is very comfortable.  Billy returned to school, although with strict physical activity restrictions.  I do notice that Billy's bowel and bladder nerves, or maybe it's just the pelvic floor nerves, are still in shock, and not functioning as well as pre surgery.  We see urology next week, and will bring that up.  Urology visit showed elevated blood pressure, and we decided that it's time to go in and do a cystoscopy to see if there is some kind of blockage in the urethra from the hypospadius repair that is causing the portion above to be dilated.  OR time has been booked so anything we find can be repaired right then and there.  Date will be 12/27.
November 2006 - Three month ortho check looks good, brace can be weaned off over the next month.  Billy will wear it at school and out at play till then, but have it off in the house.  We met with nephrology since Billy has had several blood pressure readings in 2006 that are higher than normal for his age.  For now, bloodwork was drawn and the school nurse will monitor his BP weekly to see if anxiety at the hospital clinics are to blame, as BP on nephro visit day was okay.  Bloodwork came out okay, so we are just monitoring the situation for now.
December 2006 to January 2007 - We had followups with neurosurgeon and ortho, things are looking good.  The bowel issues are stable, if unchanging, and bladder nerves seem to be settling down.  Uro went in after Christmas to check for stricturing but everything looks good in the urethra and bladder, so nothing was done.  We saw the LLD doc in Baltimore, and nothing has really changed there, the discrepancy is about 4.2cm.  No doc visits in January, so nice and calm, other than colds and strep.  BP is being monitored weekly at school, and while some numbers are just barely in the high BP range, I don't think they are that bad.  Some are lower, so not steadily in the high BP area.  We'll see next month what nephrology thinks.  Billy had a GI bug with vomiting, and his first ever bout of diarrhea (almost), and due to its odd pattern of vomiting only at night till morning, then being fine during the day for four days, we ended up getting a flat plate xray to check things out.  I was worried that he might be constipated, and we were seeing overflow stool instead of diarrhea.  The xray looked good, and since he wasn't eating well or drinking his milkshakes, there wasn't even any gas in there.  We decided to do a trial of lactose free milk to see if he is lactose intolerant and if that is the cause of his extreme gassiness.  We'll see  next month how the xray looks and what GI thinks.
February 2007 - We saw gastro, things look good, Billy's belly is nice and soft with the switch to Lactaid and he feels better and is eating better so well will stick with it.  Neurosurgeon is very happy with Billy's progress since the TC release.  Nephrology appointment also went well and she thinks that Billy's BP is okay for now, she ordered blood tests and will look at the kidney ultrasound this summer with uro, and work with him closely if any problems.  We don't have to see her again for a year :)  Ortho appointment xrays look good, fusion is coming along nicely.  Some of Billy's restrictions have been lifted for home while supervised, he can now ride his motorcycle and bike, do light running and play on playground equipment, but still no contact sports or jumping/bouncing.  After a month and a half he can do these things at school again .
March 2007 to  April 2007 - Strep again, go figure... Also, we got a letter stating that the urinalysis from our nephrology visit was positive for Ecoli infection, so Billy was taken off the Amox he was on for strep, and put on Keflex.  A repeat urinalysis done a week after stopping the antibiotics again showed Ecoli.  As did a third test.  We saw the uro in April and he thinks it is just "normal" for Billy and we wouldn't treat unless he showed symptoms of a UTI.  The kidney ultrasound looks good :) Also, by the end of March, his bladder issues resolved and function has been restored to normal with no more accidents or leaking! 
July 2007 to August 2007- We had an ortho followup in July, and after discussing endurance problems and Billy's tibial torsion and hip issues, we decided to have a gait analysis done for Billy.  Billy was fitted with a new brace and shoelift.  Due to his foot changes, they took 1cm off his shoelift and added it as a heel to his brace.  This allows his foot to remain flat without extending to a ninety degree angle since his achilles tendon has tightened up and won't allow that. See leg page for pics.  The gait analysis was done in August, and they had him walk in the lab with sensors on his body, and videotaped the procedure.  He also walked throughout the basement of the hospital with a mask over his face to measure his metabolic functions during walking exertion.  He was a trooper and did everything they needed.  We will get the results in about six weeks.  
October 2007 - We had our conference with ortho to discuss the gait analysis findings.  It seems that the function we have now is as good as it's gonna get.  Endurance issues will continue to be a problem, and we'll just have to find ways to work around this.  Good news is that the current orthotics we are using really help Billy's gait, and he is being protected from long term joint damage using them.   Because of problems walking long distances, especially in the heat and outdoors, we are ordering a lightweight portable wheelchair for Billy.  We saw the uro and we are going to do another urodynamics study (fluoroscopic) to check things out and address my concerns about urine refluxing into the seminal vessicles and possible damage long term. I had heard problems other children were having as they went into puberty with pain from this, and also problems with single kidneys going into kidney failure.  The ultrasound still looks good, so no immediate worries.
November 2007 to December 2007 - We had the fitting for the wheelchair, and put the order in, and also had the videourodynamics in November.  That looked very good, so no concerns to address at this point.  Went to see LLD clinic in Baltimore, all looks the same.
January 2008 to March 2008- Saw ortho, discussed leg pains Billy has been having along with reduced flexibility of legs.  Everything from the fusion standpoint looks great, and he sees no reason from ortho perspective for leg pains.  We also saw NS, and he thinks things look fine, but gave us a script for a PT evaluation since legs did seem a bit tight.  He wants to see us back in two months, after a new MRI.  We had the eval the end of January, and the PT felt things weren't too bad, but gave us stretches to do at home.  She felt it was probably due to a growth spurt, as she often sees reduced flexibility until tendons catch up with bone growth.   We saw gastro in February, all is well, Billy still eating better and on the Periactin, actually broke 50lb mark! We did some more PT and they released us, things looking better, can do home stretches when needed.  Saw NS again in March, the new MRI looks pretty much the same, and no more leg pains after those few times in January. 
2008-2010 were very quiet, stable years, with little other than checking in with the doctors.
2011- Billy's foot continued to turn in and his leg length discrepancy started to grow again.  In July we had surgery to correct his clubfoot deformity again, and after a longer than anticipated healing period, all looks good.  Tibial torsion is much more pronounced now that the foot is back in a plantarflexed position and will have to be addressed soon.  In December, our visit with the LLD clinic has determined we need to schedule lengthening surgery and they will rotate the tibia back into position while lengthening it.  Billy had the fixator placed on 12/27/11. 
Ortho-  the fixator lengthening achieved 6+cm (2.5 inches) of new bone growth.  We did LOTS of physical therapy and running back and forth to the doctors, had 2 pin site infections, had issues with knee contracture, but overall this has been a very successful surgery (scheduled for fixator removal on 6/5/12)  See Life with a Fixator page for all the details on this journey!
Endocrinology- the endo doc is a bit concerned over Billy's lack of growth and weight gain overall.  A lot most likely has to do with a combination of genetics and the 8 level spinal fusion he had at age 6, plus 2 surgeries this year, but they are keeping a close eye on things.  Endo wants to see him 6 months after surgeries are all done (hmm, does fixator removal count?) to see how things settle down.  One thing in our favor is that two of Billy's cousins were very small throughout childhood, but then suddenly spurted up in adolescence and are now over 6' tall!  Billy's brother seems to be following this growth track also.  We know with the spinal fusion we will not see this height, but it gives us hope that he might not be too severely affected.

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