Volume XXI, No.2 December, 2000
New Center For Arthrogryposis
Treatment in New York
From: The Hospital for Joint Diseases,
New York City
"We are proud to announce the new Center for
Arthrogryposis Treatment at The Hospital for Joint Diseases. Directors
Harold van Bosse, M.D. and Joan Gold, M.D. coordinate a team approach
to these complex children. Our philosophy is that most children
with arthrogryposis generally carry a good prognosis for walking
and independent functioning, they should therefore be given the
full benefit of a multi disciplinary approach. Children at The Center
are evaluated and followed at every visit by a compliment of pediatric
specialists, including orthopaedics, physiatry, and physical and
occupational therapies. At their first visit, they are also examined
by a geneticist and a neurologist to help establish underlying conditions.
This is particularly helpful to young families, to help them establish
the risks of having future affected children.
Treatment plans are designed to take both the child's
medical/physical needs and their social/emotional needs into account.
Our social workers and child life specialists are integral in formulating
such treatment plans. Aggressive physical and occupational therapies
are supplemented by serial casting and surgery. Our experience with
the Ilizarov external fixator for treating arthrogrypotic joint
contractures has demonstrated its effectiveness, especially in getting
children with severe knee flexion contractures ambulatory. Children
requiring intensive inpatient therapy may be admitted to the Rusk
Institute for Rehabilitation, our sister facility.
The Center meets monthly. We hope we can be of
service to any of your East Coast members. For appointments, please
call (212) 598-6250. For further information, please do not hesitate
to contact Dr. Gold or myself directly."
Harold J.P. van Bosse, M.D.
Pediatric Orthopaedic Surgery
and Pediatric Spine Surgery
The Hospital for Joint Diseases
301 East 17th Street
New York, NY 10003
(212) 598-2311 Fax
Joan Gold, M.D.
Rusk Institute/NYU Hospital
34th Street and 2nd Avenue
New York, NY 10016
(212) 263-0603 Fax
Robert Wallerstein, M.D.
Ellen Moran, M.S., C.G.C.
The Hospital for Joint Diseases
301 East 17th Street
Linda Both, M.A., C.C.C.
The Hospital for Joint Diseases
301 East 17th Street
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Adults with Arthrogryposis
Chris Johnson, coordinator of the Mid-Atlantic
support group for Arthrogryposis, has started a new Internet Discussion
List aimed at issues faced by adults with arthrogryposis. To join
the list, point your Internet browser at: http://www.egroups.com
and follow the instructions from there. Chris Johnson can be contacted
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On Muscle Biopsies
From: Marnie King, OTR/L
As the occupational therapist at A.I. duPont Hospital
for Children's Arthrogryposis Clinic, I want to respond to comments
about the muscle biopsy. You need to know why there is reluctance
to do the biopsy with a child with arthrogryposis. First, the biopsy
protocol says to take the muscle sample from the quadriceps which
is ordinarily a big muscle. However, it is not necessarily a large
muscle with AMC. Since the biopsy may remove the very fibers that
give the child the chance to extend the knee enough to stand and
walk, our physicians do not order it unless there are other unknowns.
Clinical experience tells our doctors here whether the child has
clinical AMC and if the child needs the biopsy. Often it is done
during the foot surgery and muscles other than the quads could be
used. Since joint contractures (arthrogryposis) may come with other
diagnoses, the least invasive procedures are recommended by us.
Our orthopedic and therapy management would not be any different
with the information gained by the biopsy.
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Genetic Study of
Distal Arthrogryposis Makes Progress
Department of Pediatrics,
Eccles Institute of Human Genetics
15 N 2030 E. Rm 2100
University of Utah
Salt Lake City, Utah 84112
e -mail: email@example.com
Last year Avenues published a brief letter that
I wrote to someone inquiring about the genetics of arthrogryposis.
This letter began by explaining that one of the goals of my lab
is to identify genes causing multiple congenital contracture syndromes.
To this end we recently started working with two families with trismus-pseudocamptodactyly
TPC), a unique and uncommon form of distal arthrogryposis. We have
tentatively defined the location of the gene causing this disorder
on a specific human chromosome. However, we would like to test other
families to confirm or refute that this finding is correct. Identification
of the chromosomal location is one of the preliminary steps to identifying
the gene and further understanding the mechanisms during development
that produce congenital contractures. Is there a mechanism to ask
families who are members of Avenues with TPC, if they are interested
and/or willing to participate in this project? For example, could
I e-mail a "call for participants" to you to be distributed
to your members via e-mail? This is a common practice used by medical
journals to announce research studies to clinicians. Additionally,
one of the families with TPC with whom I am currently working would
like to speak to members of other families having children with
TPC. Thus, if TPC families in Avenues (not necessarily only those
who might participate in our project) were willing to speak to this
family, it would be appreciated.
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Arm Surgeries and Arthrogryposis
The question of how to deal with limitations in
arm movement caused by arthrogryposis is a difficult one for many
parents. The choices are generally between physical therapy only;
surgery to increase the passive range of motion in the elbow( an
elbow "release"); or an elbow release and surgery to transfer
muscles to increase the active range of motion. In this issue of
Avenues, we explore these options and hear from a number of parents
and adults who give their opinions on the best option.
In the book, Arthrogryposis: A Text Atlas
(Cambridge University Press, New York, 1998. 178pp. Edited by Lynn
Staheli, M.D., Judith G. Hall, M.D. Kenneth M. Jaffe, M.D., Diane
O. Paholke,B.S. ISBN 0-521-57106-5 List price: $85.00 ) the authors
"the stiffness seen in the arthrogrypotic
limb is highly variable and may be in flexion or extension . Passive
range of motion at birth may be limited to just a toggle. The
joint capsule, muscle, tendons, and skin are all affected…The
elbows are often in extension. Triceps function will be present,
but biceps and brachialis [other arm muscles] are non-functional
or extremely weak. When the elbow is flexed, biceps function will
be better but is limited by the stiffness of the elbow.
It is important to consider the lower extremity
function before planning treatment for a stiff elbow. The usual
goal for elbow treatment is to allow the hand to at least passively
reach the face. However, if crutches or other assistive devices
are needed for ambulation, an elbow release may not be wise or
may be deferred until the lower extremity function is improved.
…The majority of children with upper extremity
manifestions of arthrogryposis will not need surgical treatments.
At Children's Hospital and Medical Center in Seattle, 70% of the
children seen at the arthrogryposis clinic did not undergo surgery
on the upper extremity…
…Our goal has been to obtain flexion to
90 degrees in elbows that are initially extended. Thermoplastic
splints are applied within a few days of birth. The orthopedist
monitors progress every 4-6 months and continues treatment until
no improvement is seen. We have not usually made decisions on
the necessity of elbow release procedures until the child has
reached approximately 8 months of age. If both elbows are not
required to be in extension for ambulation, then a posterior elbow
release is considered to allow one hand to reach the face for
feeding and self-care. This is often done early.
During posterior capsulotomy of the elbow, the
triceps tendon is lengthened by a long oblique tenotomy, and the
posterior capsule [back of the elbow] is released. Even aggressive
releases rarely result in more than 100 degrees of passive flexion.
(Williams, 1973). Splinting continues for 8-12 weeks after surgery.
A few children will have bilateral [both arms]
flexion deformities of the elbow that may not respond to splinting.
However, anterior elbow release [front of the elbow] is indicated
only in severe contractures, and this is rare. Even with the elbow
flexed at 90 degrees, the functional level is high.
Restoration of active elbow flexion in the arthrogrypotic
child is often a consideration, since some active flexion power
will greatly improve feeding, facial care, and carrying. However,
all the muscle transfers available involve some cost. Available
donor muscles include the latissimus dorsi [lower back muscle],
pectoralis major [chest muscle], triceps [muscle that straightens
the arm], sternocleidomastoid [neck muscle] and the common forearm
The latissimus dorsi offers a large donor muscle
with little loss of function if it is transferred. Unfortunately,
in most cases of arthrogryposis, the latissimus muscle does not
develop to a point that transfer is possible.
The pectoralis major is most often a fairly strong
muscle. Transfer is possible, but the cosmetic appearance of the
donor site is often unacceptable. The patient will lose ability
to forward flex the shoulder in most cases.
Triceps transfer can be done simply but will
result in loss of active elbow extension, and this procedure should
only be performed unilaterally [on one arm]. Progressive flexion
contracture of the elbow has been noted after triceps transfer
but may be purely a natural progression of the arthrogryposis
and not due directly to the transfer.
Many children with amyoplasia will have little
strength of finger and wrist flexion and a Steindler flexorplasty
[using the forearm flexor muscles] cannot be considered. The Steindler
procedure ideally can be performed with posterior elbow release
and triceps lengthening. It can be performed with little extension
loss but gives active flexion to 40-50 degrees. The strength depends
on the muscle power available. Often, both the forearm extensor
and flexor muscles are advanced proximally.
Rarely, supracondylar [above the elbow] extension
or flexion osteotomy [removal of a section of bone] will be indicated
for the stiff elbow. This should be performed for specific functional
Comments Regarding Arm Surgery From the Archives
of the Avenues Discussion List, 1997 to present [Note: Names have
been deleted to protect the privacy of the persons who made the
Comment #1:…Both of my
arms were straight and my bone doctor bent the right arm. My surgery
was done about 23 years ago. I don't regret the surgery at all
because, with my right arm I can feed my self, type, brush my
hair and teeth. I use my left arm for writing and reaching for
Comment #2: My 13-year-old daughter
has straight arms—virtually no active range of motion in
her elbows, but her hands can reach her face if she pushes her
forearms against something. She has figured out how to do all
her personal care—brush her teeth, wash her hair, bathe,
etc. She also plays basketball and football with her 14-year-old
brother and the other boys in the neighborhood, does her own laundry,
does chores around the house, rides a bike, plays the piano, and
has taken tae-kwan-do with the arm movements adapted. Every case
is different, of course. But unless the elbows are locked, with
no (or very little) passive range of motion, kids can make all
sorts of adaptations.
Comment #3: My daughter, now
16, has very limited use of her arms. When she was diagnosed at
4 weeks of age, her arms were still so tight against her body,
that it was hard to get a wash cloth under her arms. We started
OT and PT five days a week with us doing range of motion exercises
4 to 5 times a day. We also splinted her wrists and fingers. The
doctor told us that day that her arms would be useless to her
and that all they were, were flippers. Well for flippers this
girl has learned to feed herself, do all of her toileting needs,
dress herself including hooking her bra, and putting on her socks.
She can brush her hair, brush her own teeth. She has also learned
to be pretty independent in the kitchen, doing light cooking,
scrambling eggs, making taco's. etc.
What we did was do the range of motion, exercises very regularly
until she was school age. At that time we started independent
living skills. It is her goal to go away to college and not have
to have an aide help her with anything.
Comment #4: My daughter Jessica
was born January 22, 2000. She is affected in her arms and lower
body. Her one elbow was fixed at birth but it can bend a little
now. Her wrists were both very bent and look better. The arm surgeon
that we saw in California wanted to do a tendon release in one
or both of her arms. We have opted to seek more therapy and use
that surgery as a last resort. We have seen improvement. The only
option that I have heard of, and she has seen two upper body doctors,
has been the tendon release of the elbow... I am going to Seattle
in November to get a third opinion since the other two opinions
were a little different.
My only reservations with doing the surgery is that Jessica can
bend her one arm a little. She can bend it enough to put a pacifier
in her mouth. Yes, she pulls back on her shoulder to do it, but
her arm is still bending. The other arm she can bend while lying
on her back and I think that when she gets older she can learn
how to position it to bend it. Her one arm was completely straight
when she was born but now it does move but mostly with the assistance
of gravity. I just don't want her to lose the control that she
Comment #5: My son Devan was
born with a flexion contracture on his left side and extension
contractures on the right. His right wrist was/is turned in completely
while the left was/is more flexible and the hand opens more. Babies
are always tighter at first. We are glad we did nothing early
on, except range of motion and splinting, as he uses the flexed
side for self care and eating and the right straight side (arm
that bends passively with turned in wrist) to write with and any
fine motor task a right-handed person would do…
Comment #6: I had two elbow
releases. One for each arm. Before my arms were completely straight!
I think it's a good thing to have done because now I can eat by
myself and I can write. I can do a lot of things!…
Comment #7: I just wanted to
pass along a great suggestion for working on passive range of
motion in babies' elbows which was given to us by our PT. She
told us to bend Eli's elbows inside the kind of baby carrier that
you wear on your chest (the one that we have is called a Baby
Bjorn, there's another brand called Snuggli). This has been great
because it sort of acts as a splinting device but he's very comfortable
and happy in there and can tolerate it as long as my back can
stand carrying him around. I hope this might be useful to someone.
Comment #8: Lisa no longer wears
the elbow brace which she wore following surgery. Actually, I
can't remember how long we had to use that. I can remember we
had to have her in flexion for part of the day and extension for
the other. It was a great splint. …I had a local O. T. make
it using Dr. Van Heest's instructions and it had to be remade
during surgery. Not a huge deal - just be sure Dr. Van Heest sees
the splint before hand and all the measurements are perfect. We
have had no changes since the surgery. She came out with full
passive flexion on her left elbow and still has it. The only "complication"
we had (which we don't consider a complication) is that Lisa is
now at about 20 degrees of flexion, unable to fully extend that
arm. She gained so much function in that arm from the surgery
that the flexion contracture doesn't bother us. I hope the surgery
has a positive outcome for Juliet. I'm always leery about recommending
surgery to anyone. Each child has such different limitations and
function. It would seem that with only 10-20 degrees of passive
flexion you can't go wrong. We're trying to get some information
on latissimus transfers. …to gain active elbow flexion.
Comment #9: I have some information
on Latissimus dorsi muscle transfers to be used for elbow flexion.
My daughter had that surgery on the right side when she was two
years old. Unfortunately, it was unsuccessful. I understand that
the reason it failed was because her latissimus dorsi was not
a normal muscle to begin with. It was just better than her biceps.
It did not end up to be strong enough to work as a substitute,
even with tons of therapy and electrical stimulation. It also
caused internal scarring which prevented subsequent surgery from
being successful. I truly believe it would have worked if her
latissimus dorsi was a normal muscle. So please, don't be scared
away by my story.
My daughter had a left sternocleidomastoid transfer and that worked
successfully as an elbow flexor. The same surgeon performed both
surgeries. He is highly experienced with AMC surgeries and I highly
recommend him despite the fact that we have been through some
disappointment. His name is Dr. Melvin Rosenwasser at 212-305-8036.
He is affiliated with Columbia Presbyterian Hospital in Manhattan.
We discovered the latissimus dorsi muscle was not normal during
the surgery. We had EMG studies prior to surgery but it was very
difficult to get fully accurate results because the sedation was
not enough to keep my daughter still. It was a very difficult
diagnostic test that I would never want to go through again. Once
we discovered this during surgery, we decided to give it a shot
since she was already on the OR table with the incision made.
The doctor felt it was possible for it to work but not definite.
The scarring that occurred internally was in the biceps area on
the right side. This interfered with a later attempt for a sternocleidomastoid
transfer to the right biceps. The sternocleidomastoid transfer
was successful on the left side. The left side had not been operated
Comment #10: Ana had her surgery
yesterday and it went great!!! After more than a year of therapy
Ana only had 10-15 degrees of passive range of motion in her right
elbow. She came out of surgery with her brace set at 90 - what
a sight to see. During surgery Dr. VanHeest got full passive range.
Ana has to wear her brace for the next 6 weeks set not to go beyond
90 and then we can start to work it up more. We are allowed to
do some range of motion exercises but we can't let her extend
her elbow on her own. She is not in major discomfort (she was
more bothered by the fact that she had an IV in her foot and couldn't
have her AFO on). Mary - Shriner's will have you come in a day
before Julliette's surgery and make the brace there. They will
have her wear it for an hour or so and then refit it and Dr. VanHeest
will recheck it before & after surgery. They are absolutely
wonderful there and we had a great stay. Ana is going to be discharged
Comment #11: Ana had what is
called an Elbow Release... Right now her elbow is set at 90 degrees
for six weeks, until her muscle heals. Then we will be able to
work her up to hopefully getting her hand to her mouth. Right
now the surgery appears to have gone very well. Before the surgery
Ana had only 15-20 degrees of passive range of motion in this
arm. All range of motion that we achieve from this surgery will
also be passive. Dr. Van Heest, who performed the surgery, told
me that they usually do it between the ages of 2 & 3 years.
There was really very little down side for us as Ana has no voluntary
shoulder or elbow movement and little hand use so there was really
nothing for her to lose. The idea of her being able to feed herself
is very exciting for us.
Comment #12:…When we did
Ana's elbow release, everyone agreed to it but Ana's OT, who was
concerned that Ana might lose the ability to straighten her arm
completely. This was a valid concern and turned out to be true
(it now only straightens to about 15 degrees which was all the
passive range of motion it had before surgery) but we felt very
strongly that Ana being able to get her hand to her mouth was
more important than being able to straighten it. We are somewhat
fortunate in that Ana can do so little we really don't have much
to lose with her surgeries…
Comment #13: I just read your
post regarding Ana's flexion contracture following her elbow release
and wanted to share what Lisa's elbow eventually ended up at.
Following surgery, we were able to straighten her elbow completely.
As she grew, however, we began to lose the ability to straighten
her elbow. She slowly went from a straight elbow to what is now
a 45 degree flexion contraction. We continuously work on straightening
it - we don't want it to get much beyond 45 degrees. The contracture
is not a problem for her Lisa. In fact, she has gained an incredible
amount of function with it in that position. We do have to watch
it though. Lisa is almost 5, had the surgery at age 21 months.
Comment #14: We are finding
that Ana's elbow is "pulling up" more as time goes on.
Like Lisa, Ana likes this as it is more useful to her. ( Her left
arm is still in extension). We brace every other night straight
but I think Dr. VanHeest [Shriner's Hospital, Minneapolis] told
us there could be changes as she gets older and her arms grow.
Since our main goal with the surgery was function, I am not too
upset by the effects but I am trying to help her keep as much
range of motion as possible. We haven't decided what plan we will
take with her left arm.
Comment #15: I have also never
been able to straighten my elbows and I am able to do many things,
almost everything. The doctors never wanted to touch them either.
I am now 39 yrs. old.
Comment #16: My daughter had
surgery on both elbows to bend her arms in front of her, The doctors
said, either way the elbows will be stiff, either straight or
bent. She has very little mobility.
Comment #17: I'm a 38 yr. old
with AMC and I have maybe 5% use of my arms. Both my elbows are
locked and my fingers are pretty much useless. I'm curious, how
involved are your daughter's arms. Are they really thin? Does
she grasp well? If they are like mine, hopefully she can use her
feet like I do. When I was very young, my mother purchased an
electric typewriter, which helped me figure out that I could do
many things, how to use my feet for many of the everyday things
others use their hands for. Whatever you and Texana create for
her to be as independent as possible will undoubtedly be fun and
inspiring for your family as I experienced when I was a child,
learning about myself and my disability.
Comment #19: My son had no movement
in his arms at birth. But every day he gets more and more. At
about 6 months he started flexing his fingers on one hand. Believe
me, at first I thought I was seeing things. My OT thought the
same way. It won't be the same as you and I, but they learn to
use what they have. My son can pick things up with his hands like
a claw. What he can't do with his hands he uses his feet for.
He has no active elbow movement but he has learned to bend down
to use his leg to bend his elbow to put it where he wants it as
well as using his teeth to bite his long sleeve to lift his arm.
I keep working with him but he seems to figure it out on his own
best. He is 14 months old now and what a difference. Don't give
up, there is always hope!
Comment #20: My son Char's both
upper arms were broken in delivery. He was such a sight, they
were not sure if he had spinal injury due to breach birth. Char
has very little arm muscle, both arms…straight, no bend
in the elbow, limited movement in each wrist, and both hands are
in a cupped or semi-closed contracture. When Char was 18 months,
Shriner's did surgery on the right to bend at the elbow, giving
him the ability to get at least one hand to his face. His arms
still have no muscle and no lifting ability. He will brace his
right hand against something to get things into his mouth, i.e.
carrot sticks, pretzels, chips, etc.
Char uses a pencil in his mouth to write, and uses a pointing
stick to type on the computer (when we don't watch, he uses his
tongue and/or chin) to type. He tried to write with his hands
but, since he has no muscle to press hard, he is only able to
use soft markers, etc.
Comment #21: Have you tried
any elbow splinting? We would use this for Lisa until she was
about 18 months old. We attached tabs to her wrist splints. We
made a vest for her with tabs at the shoulders. Rubber tubing
would attach at the shoulders and wrist tabs, flexing her elbows.
We eventually went from 20 degrees of flexion to 90.
Comment #22: Our daughter and
I have limited movements in our elbows, wrists, fingers, shoulders.
Also many other joints.…We both drive. (I taught my husband.
He is a police officer.) We enjoy drawing, sewing, writing. I
was a telephonist and Cheran is going to be a primary school teacher.
It is very difficult to look ahead and think of all that [your
daughter] will be able to do. As parents we worry about the future
for our children. Remember that "small" achievements
lead to big ones. You will feel up and down, but be sure that,
when you look back later on, you will treasure it all.
Comment #23: I was born with
both elbows in extension also, before I started school they were
surgically fused in a bent position, one at a lesser degree then
the other. (Note, this was 35 years ago).
Comment #24: Em (almost 15)
has stiff elbows—she can get her right hand to her head
by resting her forearm against something and bending her elbow
passively with her body weight, but her active range is pretty
limited. The only thing that I can think of that she can't do
is put the backs on her pierced-ear earrings! She's taking driver's
Comment #25: When Sam was born,
his elbows were in total extension, with almost no flexibility.
We did range of motion exercises and, at 15 months, I decided
to have bilateral triceps and tendon lengthening. It was the best
decision I've made, even though many professionals told me I was
crazy to do both sides at once. Now both his elbows have passive
flexion well over 100 degrees, and active flexion around 30. His
recovery was awkward at first, I think the casts weighed more
than he did, but quick. The first time he was able to put his
fingers in his mouth was very exciting for both of us.
From the editors: Our daughter,
Melissa, was born with both arms in extension with no active flexion.
With therapy and use, she eventually achieved about 90 degrees
of passive flexion in her right arm without surgery. (She was
scheduled to have an elbow release but the doctor called it off
when he found out how much passive flexion that she had). With
90 degrees of passive flexion and relatively good hand function,
Melissa can meet all of her self-care needs.
[back to top]
From Families Around the World
My name is Chris Hartwick. I am a person with
AMC. I am 31 years old. I work as an artist. During the last couple
months I have been experiencing some swelling in my left armpit
area and pectoral muscle. We have done CT's and MRI's but both have
come back negative. Do you know of any others who may have experienced
this problem? My doctor doesn't know much about this type of disease.
He knows as much as I know and I think I still know a little more
than he, which isn't much. We think it could be muscle related.
I'm just trying to find answers. I've seen your site a couple of
times so I thought I would give you a try.
I am very active. I teach CPR and First Aid for
the Red Cross in South Carolina. I am a martial artist studying
Chinese Kenpo. I am currently a blue belt (halfway point to the
black belt). As I said earlier I am an artist. I draw with my mouth.
I do computer work with my feet. I do type 30 words/min. with my
left ring finger. I'm a pretty good cook. I like to swim, bowl,
miniature golf, and volleyball.
I don't drive yet but would love to. Do you know
any organization who could help me drive a car? Financial assistance
in getting a car and equipment to drive it? That would be great!
Any info on either the muscles or car would be greatly appreciated.
Hi, I remember contacting you when you lived in
Washington. I can't believe so many years have passed. Your newsletter
has been so helpful. I used to read them over and over; they were
and still are very important to me. My daughter Blair was born with
AMC 11/82. She is now a senior in high school. I read your daughter
is attending Sonoma State. I went there for a year myself after
graduating from Santa Rosa JC.
We are in the very stressful process of filling
out college applications. I would love to hear from you regarding
how and why you chose Sonoma and what other schools you considered.
I am sure you did a very thorough search and would really appreciate
any help you could give me...I have been searching for scholarships
for physically challenged individuals and have had no luck. Do you
have any suggestions...This is such a big move in all our lives
and I am very nervous....I would love any advice you could send
From the editors: Hello, Martha.
It's always nice to hear from one of our longtime readers. After
being home-schooled through high-school, Melissa attended the local
Junior College (Columbia) for a year, then transferred to Santa
Rosa J.C.for a year. Her main interests are music and graphic design
and Santa Rosa seemed to have a strong program in both. She then
transferred to Sonoma State to continue her music studies while
at the same time continuing to take classes at Santa Rosa. Sonoma
offered the advantage of a fairly accessible campus, a small school,
a good music program, and a reasonable tuition. Since Melissa didn't
get any large scholarships coming out of Junior College, cost was
definitely an issue. Melissa looked long and hard for scholarships
for persons in her situation and didn't find much. It's a bit surprising
that there isn't more help available to assist the physically disabled
in attending college.
Hi, we are the Barbers. We have a son who is
1 year old. He has arthrogryposis. He has a dislocated left hip,
club feet, upper deformities on hands and wrist, problems chewing.
Gravity beats him. He cannot walk or sit up. He scoots or rolls
everywhere he has to go. When he is sitting we have to put him
in a sitting position and then he can't bring his hands up. Only
he is lying down he can do this. He has had three surgeries on
his feet. None of them worked. We have been to the Geisingner
in Danville, Pa., also the DuPont Inst. in Delaware. We don't know
if we should put him through any more surgeries or not. If you
please help us in any way, it will be greatly appreciated.
1130 13st South
Lethbridge, Alberta. T1K 1S7
Hi, My name is Corinne. I am a resident of Alberta,
Canada. I was born with arthrogryposis. I am 25 and currently live
in a house with 2 other girls. I have personal care workers come
into my home and care for me. My only income is AISH (Assured Income
For Severely Handicapped) which pays my main bills and groceries.
I am only receiving this assistance until I find
a full time paying job. I took Travel Tourism for 2 years at a local
college to become a Tour Director/Manager. But in order to find
and keep a job I need to be more independent and have my own transportation.
I got my learners when I was 19 and was tested
at the Glenrose Rehabilitation Hospital by Vivian Hyrinko in Edmonton
for driving. I drove a vehicle around a parking lot for an hour
and am able to drive
a vehicle adapted for me.
The question I need your help for is...where do
I get the money to do this? There has to be something out there
for us. Last week I met a young man with the same disability and
he is having the same problem.
Easter Seals here said they will fund me for the
special equipment I would need. But they would not give the money
until they see my valid drivers license and vehicle registration.
So this means I need to purchase the vehicle before they will give
I feel I am being punished for being born this
way. You can't deny the fact that if I was hurt on the job, Workers
Compensation would already have 3 vehicles fixed up for me to get
back on my feet and have a full time job. All I want is to be more
independent. Is that too much to ask for? Please let me know what
I can do about this...or whom I may speak to that would know more.
My name is Ron Campbell, I live in Melbourne Australia
and I just read your arthrogryposis page....I have just visited
your site, the reason being I was born with arthrogryposis in 1973
and had never really gone around to finding out anything much about
it. I had a club foot and both wrists were affected.. Looks like
I had a relatively mild case. I've had 3 operations on my foot,
3 on my left wrist and one on my right wrist. I also had one on
my otherwise "normal" right leg to remove the growth plate
when I was 15 or so, as my right leg was 2 inches longer than my
left. By the time I finished growing the difference was more like
1 inch... I still notice it but it generally doesn't slow me down
Over the years I have picked up habits of roller
skating and roller blading (I impress people who don't even realize
I had arthro) I snow ski, snorkel, walk a lot and run a little,
though my ankle doesn't appreciate the running much...I thought
I'd write to thank you for the information and I'd like to add my
story to your list if you have one. Might be of assistance to some
kiddies coming up in the ranks...
I don't use any special devices to cope with life...Like
your page says I have become very resourceful and work out my own
ways to do things...
My name is Bruce Mayer and I have a 10 year old
son (11 in July) who has arthrogryposis. He has been treated since
birth by Dr. Peter Pizzutillo in Philadelphia. He has responded
very well to his combined treatment of operations, casting, physical
and occupational therapy and can walk with the assistance of long
leg braces over short distances. He has been mainstreamed in school
and has adjusted pretty well inside and outside school. I am trying
to find a social/camp atmosphere where he can interact with children
his own age who have similar physical challenges. Do you have any
Several weeks ago I e-mailed you folks with a question
about our youngest daughter who had AMC and got a quick answer.
Brittany was born in 1975 and died seven years later. She was severely
retarded but a very cheerful child who faced life with a quick giggle
and a big smile while those around her (mostly her mother) fretted,
denied, bargained (with God) and then gradually accepted it. The
physical aspects I figured I could deal with but the acceptance
of the retardation was the biggest problem. Yet I am a far better
and entirely different person when she died than when she arrived.
At that time AMC was so rare that the pediatrician who first examined
her came up with chromosome damage. Then it took a geneticist to
realize the true diagnosis. This was in Omaha which I see now has
some great specialists!
Of course we didn't have the Internet back then
and there was simply was very little if anything to know or learn
about AMC condition, though of course it soon became evident that
since she'd never sit up straight or walk (due to the retardation)
then exercises, splints or surgery weren't really appropriate. How
different it is today! Since AMC is so rare, the Internet and your
web site in particular are an oasis of information in an otherwise
desert of ignorance about this condition! Just by scanning through
the chat room messages I see how much patients/and their parents
learn through exchanging information! From what I read I realize
now that Brittany's physical condition was rather minor compared
to what I see in other children - but if I'd had your site back
then perhaps I could have found someone else who's AMC child was
I published a book about Brittany several years
ago and this week I'll be reviewing it for a church group. And part
of your information about AMC will be an opening display of what
AMC is, its cause and treatment. I'll be able to demonstrate how
much more informed parents/patients are today than when Brittany
was born through sites like yours!. It's a wonderful instrument
and opportunity to provide a service to those who would seek and
possibly not find otherwise. [Editor's note: We asked Anne to send
more information about her book.]…I've always thought Brittany's
severe retardation was an unusual side effect of the AMC since from
what I understand most AMC kids are just physically affected. But
I've always been glad to talk about Brittany and the lessons she
taught us. My book is Brittany, Child of Joy and it came out from
Broadman Press (Southern Baptist Press) in 1986. It's out of print,
but I have a few copies and I've always wanted to reprint it if
I had the chance. It's Christian in emphasis but hopefully I don't
preach, just state what happened to me and let that speak for itself.
This is my first book (I have others in the works) and I've published
in Christian and secular publications during my 20 some years as
I'm a Methodist and I've been on the staff of a
large Methodist church here in Houston, Texas so you might use that
address and e-mail address since it'd probably be easier (above).
I had written you previously about my son, Bryce,
who was born with Arthrogryposis and just wanted to update you in
the hope that other mothers and families can take hope in our story.
Bryce's surgery to correct his feet was performed at Shriner's Hospital
in Greenville, SC in October, 1999. The surgery which lasted 12
long hours was successful and he is now out of his casts and walking
!!! with the aid of AFOs and a walker!!! We are thrilled. We anticipate
another surgery to correct a hip which is out of place within the
next 6 months if the surgeons decide he is positively a candidate.
They will let us know soon - we will keep you posted.
8145 NW 21st Street
Sunrise, FL 33322
My letter is in regards to information on arthrogryposis.
I have a fifteen-year-old son who was diagnosed with arthrogryposis.
We currently live in Sunrise, Florida. I'm interested in learning
more about this disorder. My son is one of the more severe cases,
I would say. He has deformities throughout his bodily joints. He
has severe spinal deformity, club hands and feet. He had spinal
surgery where three steel rods were placed in his spine, both hips
reset, and foot surgery. At the present time, Nolan has limited
function of his joints, but overall, her surprises me everyday.
He's been placed in a motorized wheelchair for the last four years.
He gets around well. When out of the chair and on the floor, his
mobility is great by rolling from room to room throughout the house.
I wanted to know if there are any organizations
in my area or some orthopedists in the area familiar with the disorder.
I want to learn as much as possible about this disorder so that
I can get my son Nolan the best help. In the past I've sought very
little and received very little assistance with Nolan's care. At
this time, the Elks hospital in Umatilla, Florida, has been the
most helpful to Nolan with all surgeries. Nolan has benefited a
lot from the surgeries.
2601 Camelback Lane #11
Silver Spring, NM
It has been many years since I have written about
my daughter Deborah, who is now 23 years old. My, how time flies…She
is one of my four daughters and is an identical twin. She was born
with severe paralysis and contractures to all limbs and joints,
clubbed hands and feet, etc. We've also battled persistent scoliosis.
There were no internal complications, thank goodness. She has had
13 major surgeries, including two full spinal fusions. The surgeries
were all somewhat successful - at least they halted any further
skeletal regression. We've done the whole nine yards with physical
and occupational therapy, of course, until she reached adulthood
when growth stopped and we decided that she had the body and skills
that she would have from then on.
Deborah basically does what a high-functioning
quad would do. She can type with a pencil stuck between her clubbed
fingers, and she pushes the hand controls of her motorized cart
with the back of her hand. She can eat finger foods by grabbing
the food in her left hand, hoisting that hand onto her right arm,
and leaning her mouth down to catch the food. There have been some
hilarious misses with food flying everywhere. She has a wonderful
sense of humor, which helps. She has to have assistance with every
life skill and all personal grooming. She wears one full-length
locked leg brace. She walked some with a helmet and walker when
she was little, but several horrible falls led to prolonged seizures
and five years on Dilantin.
Added growth and weight and lack of successful
scoliosis treatment has made it difficult for her to walk just for
short distances without full support with another person. It is
doubtful that she will ever be able to drive. Being a single parent,
I worked at home all these years so that I could take care of Deborah
and my other daughters. She graduated from high school in the Level
5 OH program and went to our local community college part-time for
two years. Our county has subsidized disabled transportation in
the form of mini-buses and cab service, although late or missed
pickups and deliveries were more common than we had hoped. She has
gone to an annual summer camp for disabled children/adults every
summer, which has given us a 10-day break each year.
Finding adult services has been a challenge. Our
state DDA office was less than helpful and often quite condescending.
Her state DDA assessment upon high school graduation noted that
she wouldn't be helped by any further education or training! Her
SSI counselor was lovely and helpful, however. We went to the DuPont
Institute in New Jersey for extensive training and evaluation. She
has a wonderful pediatrician who has continued to be her doctor
and her friend. She now likes to ride the regular Metro buses to
and from work, doctor, shopping trips, etc. even though they don't
always have a wheelchair lift at all or one that is functioning
at the moment. Our subway system is very good, however, and she
takes that to go into downtown D.C. or to see her father in suburban
Our success story - one that I hope will give parents
of severely-disabled AMC children some sense of the future - centers
around Deborah's independent life. I fought the state DDA for years
for services and finally got a small amount of aide service before
school each school day. I put her on numerous housing lists and
program lists. We both decided that she didn't need or like the
few adult day programs that were offered, since they mainly addressed
the needs of the elderly. Finally, in December of 1998, I had the
accessible 2-bedroom apartment in hand but no supporting services.
I had called all possible avenues, including political representatives,
when in desperation I called our governor's office and actually
got the secretary of health on the phone. Within two days, we had
a DDA permanent grant for aide service which covers morning, noon
and evening hours. We are still trying for fuller service to cover
nights and more weekend hours.
Our church gave Deborah a big new-home shower,
and she moved in. Her twin sister Elizabeth is her roommate - free
rent did the trick! She is very good about covering emergencies
and running errands and shopping for the two of them. In fact, they
had grown apart quite a bit during their teenage years, so this
was a pleasant surprise for me and for Deborah!
Deborah now works full-time at Home Depot. She
races around the huge expenses of the warehouse-like store. She
waters plants, collects trash, and leads the large forklifts through
the maze of aisles and customers. She is assertive and very vocal,
and every time I go to visit her she has a new nickname and is part
of the 20's-something crowd that works there. I nominated Home Depot
and Deborah for the Montgomery County disabled employee-employer
award of the year in 1999, and they won! I teased her boss that
he can't fire her now that he has this lovely glass/marble award
on his desk. She takes the bus to and from work and often buzzes
on major streets to go to McDonald's or some other culinary outlet.
When she needs help from a stranger, she just asks for it!
For the first time in 22 years, I am not a primary
caregiver! It still amazes me, and I never would have guessed that
we could ever reach this point for both of us. I am now working
outside the home and enjoying the sights and sounds and people (but
not the commute!) in downtown Washington, D.C. These changes did
not come without costs - I sold my big house when Deborah moved
out and my ex-husband stopped child support payments against the
existing court order. We all had to give up memories of how things
were and move on to new physical and emotional arrangements. We
also have had to deal with the results of years of stress and exhaustion
and family patterns.
Deborah keeps her own checking and savings accounts,
maintains her employment, schedules her aides, and lives her life
pretty much under her own steam. She even handles the emergencies
that are presented when her cart breaks down. I still do some shopping
and grooming and emergency runs for her, but we are basically free
women who each have a life of her own. It is simply miraculous.
Don't give up hope - there are lots of people who will help, and
when you find those who won't - you go around them or through them!
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My name is Lauren Faraino and I'm in 3rd grade
and 8 years old. I live in Birmingham, Alabama and I was born
arthrogryposis. When I was 18 months old I had foot surgery at
the DuPont Institute. I can walk with long-legged braces but I
to have someone very close to me because I can't catch myself if
I fall. I have broken my arm three times from falling. I can only
stay in my braces 90 minutes or my feet and knee will begin to
hurt. When I began Kindergarten, we bought a power wheelchair
controlled by my feet. In second grade we bought a beach wheelchair
that helps me around at the beach.
My favorite hobby is art but I use my feet instead
of my hands. I take art lessons once a week. Every week I get a
massage to loosen up my joints and I go swimming twice a week. I
already have one pen pal with arthrogryposis and would like to have
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New Information Resource
DIRLINE (Directory of Information Resources Online) is
the National Library of Medicine's online database containing location
and descriptive information about a wide variety of information
resources including organizations, research resources, projects,
and databases concerned with health and biomedicine. This information
may not be readily available in bibliographic databases. Each record
may contain information on the publications, holdings, and services
DIRLINE contains approximately 14,000 records and
focuses primarily on health and biomedicine, although it also provides
limited coverage of some other special interests. These information
resources fall into many categories including federal, state, and
local government agencies; information and referral centers; professional
societies; self-help groups and voluntary associations" academic
and research institutions and their programs; information systems
and research facilities. Topics include HIV/AIDS, maternal and child
health, most diseases and conditions including genetic and other
rare diseases, health services research and technology assessment.
DIRLINE is available through a simple to use interface
It is also available through Internet Grateful Med, a system developed
by NLM to offer interactive assisted searching for some of its databases.
DIRLINE can be searched using subject words (Such as disease or
condition) including Medical Subject Headings (MESHS) or for the
name or location of a resource.
Bike for Persons with Arthrogryposis
Eric On His
Step n' Go Bike
A bike that several people have recommended for
those with arthrogryposis is the Step n' Go manufactured in Vermont.
The rider can power the bike by shifting his weight from on foot
to the other. Adaptations can be made for braking and hand position.
On the company website (www.stepngo.com),
one of the featured stories describes a nine-year-old boy with arthrogryposis
who is using a Step n' Go:
"The challenge was to design a steering system
Eric could use that didn't require using a hand brake. Using high
rise handlebars upside down and backwards, we located the handgrips
near Eric's natural hand position. With the stepper pads only 4"
off the ground, Eric can easily mount his Step 'n Go and pedal by
doing a simple weight shift. For braking, we designed the unique
"Belly Brake", which Eric operates by leaning on the chest
The cost of the bike is substantial ($1299) but
it may be covered by your insurance. For further information, contact:
Treadle Power, Inc.
6 Linden Terrace
Burlington, VT 05401-4928
800-648-7335 or 802-862-2980
FAX: (802) 864-6156
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