Just wanted to welcome all the new people

[Allison]

Hi,
I am Allison and have been a member of this group for a long time
now though when I got my new computer I couldn't get the email program
to recognize this account and have been terrible about remembering to
check it. Engelmann's has run in our family for many generations. My
grandfather, my dad as well as his brother and sister and my twin
sister Liz (fraternal twins) and I all have it. I am the only one in
the family that has as extreme involvement in the skull. My entire
skull is involved and was over five times thicker then normal. I have
undergone two cranial expansion surgeries. The first was my first
skull/brain surgery and they did it the old fashion way and the second
was actually my fifth and last skull/brain surgery and my surgeon that
did it was a different one then the first and he actually invented a
new way to do it which was much less painful and I recovered much
quicker then the first. He actually sent me photos of my surgery and
told me they went through over 50 saw blades to thin down the front
half of my skull. During my hospital stay (at Columbia Presbyterian
Medical Center in NYC) I was seen by a gentic researcher and she did a
full set of x-rays to confirm my diagnosis and sent the bone to
Washington DC to try to grow it. I was initially misdiagnosed though
at the age of 20 when I began having terrible morning headaches that
would subside once I was upright. After a few weeks of this I went to
a neurologist figuring they would diagnose me with migraines. They
discovered papilledema though and I was told that I probably had an
advanced brain tumor and I was sent for an MRI right away. It showed
no tumor but did sho that my skull was extremely thick and that I had
a mild Arnold Chiari Malformation. During the first year I was
treated medically with diaretics and frequent spinal taps but when one
of my eye exams showed changes they sent me to a neurosurgeon right
away for the placement of a lumbar shunt and that was something I wish
I had never agreed to. This was 19 years ago though and information
on such rare conditions was not readily available so I believed my
doctors knew what was best. After a year of frequent hospital stays
and spinal taps as well as a few revisions I was sent to NYU and had
my first expansion surgeries. Six months later I underwent emergency
surgery after developing a postop infection that went undiagnosed. I
lost a three inch section of skull and was on IV antibiotics for two
months. During the next year I lost my job and had no insurance and
gradually lost a lot of the feeling and strength throughout my left
side. When my husband and I got married and I was picked up on his
health insurance I went to a new neurosurgeon figuring my problems
were stemming from the shunt but they were caused from a brain stem
herniation. I was scheduled to see a craniofacial neurosurgeon within
two days but the morning of the appointment my husband had a tough
time waking me up and when he could I was confused and he ended up
rushing me to the hospital where I was stabilized and underwent
surgery to decompress the herniation. The brain stem herniation was
caused from the lumbar shunt and two months later they had to tie off
the lumbar shunt and I underwent my fourth brain/skull surgery to
place a shunt in my brain. That one was fun because I had to be awake
for the first half of the surgery. A year later I had my last skull
surgery which was my second cranial expansion surgery and that surgery
took away my daily headaches. I was also able to safely have
children. We now have two and they are both healthy and do not have
Engellman's.
I read that a few of the new people also have skull involvement
and would love to chat with them. Unfortunately I have never been
able to return to work (well expect being a full time stay at home mom
which is a full time job and harder at times then any other job I have
had and more fullfilling). The spinal shunt and all the spinal taps
and surgeries involving my neck have left me with spinal nerve pain
from my neck down to my lumbar spine. Something I will live with for
the rest of my life. I have great pain doctors though and am looking
into possibly having a spinal stimulator placed this summer if I am a
canidate. I miss working. I had wanted to be a nurse since I was
little and graduated nursing school when I was 20 and only got to
practice for three years before becoming disabled. I still have hope
that one day I will return.
Now that I have chatted everyones ears off I will sign off.
Again welcome to all you new folks. The people here are some of the
nicest people I have met. My twin sister is even a member!!
Hope you are all having a great week,
Allison