dyslimbic
03-04-2007, 04:55 PM
I am writing on behalf of my sister whose youngest daughter has Arnold Chiari.
The following is in my sister's words.
C's history is that at 3 and a half she was noted as having a mild scoliosis, which
we duly watched carefully.
As she grew this progressively got worse- every time she had a growth spurt it got
markedly more curved, to the point where we wanted her referred as she was now
leaning over to the left and began to have what looked like a hunched back on the
right (basically her spine was moving under her right shoulder blade). Her sleep
pattern began to regress around now with her having awful nightmares, frequent night
waking (say 5 times plus a night) and her general demeanour/character starting to
change from a sunny happy kid to a tired grumpy and very 'dark', moody kid.
Upon inital referral (Homerton then Royal London) they noted that she had a curve
of some 35 degrees (they usually do scoliosis surgery at 35-40 degrees when kids are
13/14- so this was extreme for a 6 yr old). At about this time C started to have a
lot of stumbles and falls- if you watched her walking you could see her right leg
dragging and she would also frequently trip over her own right foot and fall badly.
We were finally referred to the Royal National Othopaeodic (sp??) hospital and they
immediately noted that C had no feeling on her right side, i.e that the scoliosis
had a neurological root.
The first MRI's were done and Mr Casey (her surgeon to be) explained that these v
clearly showed she had Chiari as well as Syringomyelia- with a massive syrinx in her
spine (from about just above mid spine to skull, so it was long and wide in
cicumference) hence all the nerve damage on her right side and the severity of the
scoliosis as her body tried to accommodate the syrinx. The end result was that this
was serious enough to warrant surgery asap as the nerve damage may now rapidly
progress to the point that C may end up in a wheelchair -or worse.
The surgery was Foramen magnum decompression surgery with a trimming of the
cerebellar tonsils and her top vertebrae (C1& C2 I think they are called).
In the neary 2 years since her surgery the scoliosis has almost disappeared, but the
nerve damage remains on the right side. Her right leg does not drag as much (but
when she is tired/running you can see the difference) and she still has no feeling
on the right side. We hope this may improve as the syrinx goes but we were warned it
may not. On the last MRI we saw that the syrinx is indeed slowly but surely going.
Her last MRi was on your birthday so we see Mr Casey again in April to see what's
what. In normal cases peple don't get followed up for more than a year or two but
because her case was so severe they are keen to track her for a few more years-
which I'm happier with too.
Since then themain issue with C is that she easily tips into 'rage'- mostly I see
this as borne of major frustration, eg either wth her sister teasing/winidng her up, or
an inability to express what she is actually feeling. Her condition undoubtedly
causes her severe pain at times, another factor it's hard to get a handle on in
terms of behavioural impact.
She also does not listen/blanks certain things particulary if she finds them
unpalatable (eg being told off!) but thats fairly normal with most kids really. C
takes everything to heart though as well and her reactions can be extreme to even
the smallest things. Her self confidence is also very low (as teachers and others
have noted) which is a bit odd for someone who can be so extreme and adamant that
she is right!
Her sleep is still all over the place- the last 3 nights have been good but the
record got set at 9 wakings in the new year....
We struggle on but do find it very difficult to know what to do with her at times,
particulary when she is on one of her rages at the world.
She is certainly a bright child, and knows that she can be totally over the top, but
sometimes cannot accept the consequences of her actions or see the impact she has on
others. She can feel empathy for others (but often not until its all explained why
someone is so upset with her etc) and is certainly loving and cuddly.
She can also take instant dislikes and be quite hurtful to adults and children alike
- to the point of real rudeness- and we don't know where that comes from either!
Anyway, that's certainly a fair bit of background for you!!.
Essentially I think her condition is the primary cause of the behavioural changes we
have seen since she was around 6, but have no idea if we are stuck with this or if it may change/improve.
I would welcome some feedback on behalf of my sister.
Thanks Tim
The following is in my sister's words.
C's history is that at 3 and a half she was noted as having a mild scoliosis, which
we duly watched carefully.
As she grew this progressively got worse- every time she had a growth spurt it got
markedly more curved, to the point where we wanted her referred as she was now
leaning over to the left and began to have what looked like a hunched back on the
right (basically her spine was moving under her right shoulder blade). Her sleep
pattern began to regress around now with her having awful nightmares, frequent night
waking (say 5 times plus a night) and her general demeanour/character starting to
change from a sunny happy kid to a tired grumpy and very 'dark', moody kid.
Upon inital referral (Homerton then Royal London) they noted that she had a curve
of some 35 degrees (they usually do scoliosis surgery at 35-40 degrees when kids are
13/14- so this was extreme for a 6 yr old). At about this time C started to have a
lot of stumbles and falls- if you watched her walking you could see her right leg
dragging and she would also frequently trip over her own right foot and fall badly.
We were finally referred to the Royal National Othopaeodic (sp??) hospital and they
immediately noted that C had no feeling on her right side, i.e that the scoliosis
had a neurological root.
The first MRI's were done and Mr Casey (her surgeon to be) explained that these v
clearly showed she had Chiari as well as Syringomyelia- with a massive syrinx in her
spine (from about just above mid spine to skull, so it was long and wide in
cicumference) hence all the nerve damage on her right side and the severity of the
scoliosis as her body tried to accommodate the syrinx. The end result was that this
was serious enough to warrant surgery asap as the nerve damage may now rapidly
progress to the point that C may end up in a wheelchair -or worse.
The surgery was Foramen magnum decompression surgery with a trimming of the
cerebellar tonsils and her top vertebrae (C1& C2 I think they are called).
In the neary 2 years since her surgery the scoliosis has almost disappeared, but the
nerve damage remains on the right side. Her right leg does not drag as much (but
when she is tired/running you can see the difference) and she still has no feeling
on the right side. We hope this may improve as the syrinx goes but we were warned it
may not. On the last MRI we saw that the syrinx is indeed slowly but surely going.
Her last MRi was on your birthday so we see Mr Casey again in April to see what's
what. In normal cases peple don't get followed up for more than a year or two but
because her case was so severe they are keen to track her for a few more years-
which I'm happier with too.
Since then themain issue with C is that she easily tips into 'rage'- mostly I see
this as borne of major frustration, eg either wth her sister teasing/winidng her up, or
an inability to express what she is actually feeling. Her condition undoubtedly
causes her severe pain at times, another factor it's hard to get a handle on in
terms of behavioural impact.
She also does not listen/blanks certain things particulary if she finds them
unpalatable (eg being told off!) but thats fairly normal with most kids really. C
takes everything to heart though as well and her reactions can be extreme to even
the smallest things. Her self confidence is also very low (as teachers and others
have noted) which is a bit odd for someone who can be so extreme and adamant that
she is right!
Her sleep is still all over the place- the last 3 nights have been good but the
record got set at 9 wakings in the new year....
We struggle on but do find it very difficult to know what to do with her at times,
particulary when she is on one of her rages at the world.
She is certainly a bright child, and knows that she can be totally over the top, but
sometimes cannot accept the consequences of her actions or see the impact she has on
others. She can feel empathy for others (but often not until its all explained why
someone is so upset with her etc) and is certainly loving and cuddly.
She can also take instant dislikes and be quite hurtful to adults and children alike
- to the point of real rudeness- and we don't know where that comes from either!
Anyway, that's certainly a fair bit of background for you!!.
Essentially I think her condition is the primary cause of the behavioural changes we
have seen since she was around 6, but have no idea if we are stuck with this or if it may change/improve.
I would welcome some feedback on behalf of my sister.
Thanks Tim