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Julie's Website

New! PT 2011
August 2008 Bethlem Myopathy/Ullrich CMD conference notes
October 2005 Bethlem Myopathy/Ullrich MD conference notes
Update 2006
Update 2005
Update 2004
Julie's Hypotonia Story
The Medical Puzzle
The Muscle Biopsy
The Diagnosis
Physical Therapy--updated in 2008!
Seating at school
Julie's Wheels
The Sleep Studies
Julie's flexible Daddy
Bethlem Myopathy--DNA and Muscles
Julie's Bethlem Myopathy Book

Keeping contractures at bay

Julie does her "stretchies" with her new knee immobilizers.

With Julie's condition, Bethlem Myopathy, there is a possibility of "contractures" or tightness in joints that limits the normal range of motion.  Over the years, the PTs have noticed that Julie's hamstrings are "a little tight" and one PT used to joke that it was all that was holding Julie upright! 


Summer of 2004, the PT at the neurologist measured her hamstrings as being tight a bit beyond the normal range of motion.  Her new PT in August read the report and felt it important to begin a nightly regimen of stretching Julie's hamstrings.  We were able to comply with the regimen pretty well for a year.


This summer, 2005, we had Julie's 6 month neuro visit and the PT measured her hamstrings as being significantly TIGHTER than a year ago!  We were shocked and upset since we had been pretty good about keeping up with "stretchies" (as Julie calls them).  The PT recommended changing our stretching routine to having Julie sit against a wall or a couch in knee immobilizers for at least 20-30 minutes a day.


Thanks to my wonderful pal Dave LaWall, the orthotist at orthotist Harry J. LaWall and Sons, we got Julie's knee immobilizers a few weeks later and started using them.  My husband got her a little bed tray at Ikea which is just the right height for Julie to sit on the floor and use it as a table while she is stretching.  She watches tv, colors, eats, plays on the laptop computer, and when I have time, we play cards and I make her lean forward to reach for a new card which creates an even bigger stretch.


Happily, this new routine has returned normal range of motion to Julie's hamstrings.  This past week, I was sick and didn't have Julie do her stretchies at all.  The PT noticed that she was a little tighter after only a week--definitely a wake up call that we need to be vigilant.


So we are left with the question of "why" or "why now?"  The leading theory from the PTs is that Julie had a growth spurt that caused the tightness.  They say that they see this a lot.  I think that is a partial explanation...the OT at school told me that she had to raise Julie's desk in June because she had grown!  I also think Julie's swimming at camp last summer contributed.  She kicks from the knee when she swims rather than with her whole leg and I think it's like when a weightlifter doesn't use full range of motion...they get stronger, but they can't unbend their arms anymore.  Finally, I have to consider the possibility that this tightness is the disease process--people with Bethlem Myopathy tend to get contractures over the years.  According to Dr. Bonnemann at CHOP who helped to diagnose Julie, you typically see contractures in the teen years.  The fact that we were able to stretch Julie and regain her normal range of motion suggests that we can battle against the disease for now and win.