Dyschondrosteosis and Madelung Wrist Deformity ( 2005 )
Personal stories concerning MWD / DCS submitted to this
site in year 2005
From M6 in the USA in e-mail contact with. [ CL ] MWD
Thank you for your work on this site. My name is M and I am from _ , New York. My daughter, who is 13 was recently diagnosed with this deformity and surgery has been recommended by a Pediatric Orthopedist in my area on her left wrist which seems to have a worse manifestation than her right one . She has had an X-Ray and MRI. This orthopedist says that there are no known reasons for Madelungs and that there are no other associated illnesses, which seems to conflict with what I've found in reading your site and other information on the internet. I was also told that it is very difficult to find surgeons with experience with this deformity, this particular surgeon cites "along with other surgeries of the wrist" eight instances in which he's operated for this. I am questioning the surgery but at the same time see that my daughter's pain is on a steady incline literally since her first mense in November. She turns 14 in March . Can you refer me to someone in the USA who has some experience with this deformity? I am relatively close to Manhattan and am willing to travel anywhere to get this right. I'm finding that doctors I've visited in my area are somewhat under-informed yet recommend surgery rather quickly.
As far as family history, my father says he remembers that his deceased sister complained of pain in her left wrist throughout her teenage years. My sister has had several problems with her wrist, but never been diagnosed with Madelungs, she had micro-surgery to correct an injury to soft tissue in her right wrist and broke her left wrist twice. She has no protrusion but has subluxation in both wrists. Thank you again for your time.
update and thank you
Nigel,
So many people have written with advice and accounts from your site, I wanted to
thank everyone altogether, it's been very helpful. As a result of the
information I received I've found a physician in NYC who is well respected
and has experience with WMD. He is the head of _ surgery at
_ and works out of the Hospital _ _ _.
We have an appointment for a consultation on February 14 and I'll let everyone
know what the next opinion is on _'s case. I am thankful I didn't go with
the opinion that surgery is required right away after reading the experiences
so many have offered from your site I've determined that I need the opinion
not just about surgery, but from someone who has experience in the progressiveness
of this deformity; the timing of the surgery is key. I will let you know what
this specialist says. Thank you so much for your valuable site.
From J8 in the USA in e-mail contact with. [ CL ] MWD
Hello. My name is J and I too suffer from mwd. I was diagnosed when I was about 12 or 13 and have lived with this disease only knowing the name of it. I was interested in knowing if more people lived with this disease so I started looking into it on the internet today. I found your site and I have to say that I was very surprised to learn that there are so many other people who have this disease. When i was diagnosed I was told that there where not very many people who have it so I have never really looked into it. There was so much information on your site about what exactly it is. Thank you!! It really helped me understand what I have and that I am not alone. If you could help me find some people that I could get in touch with in the u.s. it would be much appreciated.
From N in Israel in e-mail contact with. [ CL ] MWD
I'm a 55 female from Israel .
I was recently diagnosed as a MWD ( Madelung Wrist Deformity) carrier.
My left wrist hurt me for the last 30 years. and its getting worse.
I read your article: www.divdev.fsnet.co.uk/dysch.htm with great interest.
I wish to receive some information:
1) Which hospital is specialize in this field, with experience.
2) Can you recommend the Doctors who have experience with it.
Many Thanks for the good Work you are doing.
(from her son) I suggested to perform a genetic test on my grandmother, who has bad feet
with platfus (pes planus, flat foot ) , and one of my sisters who has a leg shorter then the other
(by 1.5 cm). have no idea if that related to MWD.
In short - at present we don't know of anyone else with MWD.
From _ _, Surveillance Specialist, Birth Defect Monitoring Division, _ Dept. of State Health Services, USA
I simply wanted to thank you for my good fortune in discovering your web
site today. I meant to get a better idea of the disorder in my head and
here was this exhaustive research with great pictures and engaging text. I'm
going to share it with my colleagues. Thanks so much!
From P in UK? in e-mail contact with. [ CL ] MWD
The 14yo daughter of a friend has just been diagnosed with Madelung's Disease after previous diagnoses of arthritis and TB. She has had treatment with Fenopron, which was discontinued when she started coughing up blood, although a connection was not made between them. She had an x-ray showing enlarged blood vessels in her left lung and scarring.
Her left hand is smaller than the right and her arm is 2.5 cm shorter. Her finger bones are fused and she suffers a certain amount of pain.
I am enquiring whether you are aware of anyone having any success with unconventional treatment. In particular with homeopathy, as I am a trained homeopath and of course would like to be able to help.
Thank you for your very informative site.
followup:-
I will give details of the group to the person affected, although the family
are not online. Can I join as an interested party though not a family
member?
From B2 in the USA in e-mail contact with. [ ? ] MWD
Replies, on 3 different accounts, to your email address (edited) ra....@comcast.net
bounced as "551 not our customer"
Hi, my name is B_ from New Mexico USA.
I was dianosied when I was 10 (after a sprain in my left wrist) which the doctors found out it is in both wrists but worse in my left (i'm left handed also). I was told I'm not a good candidate for surgery.60/40 % chance pain would be worse after sugery.I have lots of pain .I drop lots of things and my hands sometimes go numb after prolong use of hands . I wondered if anyone else had the same problems.Does anything make it better? I was a pet groomer and I had to stop working because the pain.I'm now on ssa disability . but I miss working and I have stomach problems from pain killers.Any help? Thank you for this site.
From D4 in the USA in e-mail contact with. [ ? ]
I've just finished reading your site, I came upon it in my search for a
diagnosis of my birth defect, a deformed right arm.
My parents didn't notice my birth defect until I was four years old, my dad
was tossing a ball back and forth with me. They discovered that I could not
turn my wrist palm up to throw under handed. Upon closer inspection, it
became evident that my arm hung by my side at an odd angle, my elbow
touching my side and my forearm facing upwards. I was taken to the doctor
for x-rays and the diagnosis was.a deformed arm!!
I have never known exactly what is wrong or if there is a name for this
condition, I am now 53 years old. My wrist has very limited range of motion,
palm will not turn upwards at all.Have you ever run across this condition?
Thank you,D
From B in the UK ( Scotland ) in e-mail contact with [ CL ] MWD
I have just been (original contact in mid 2004 ) diagnosed as bilateral madelung wrists after 24 years. I am a 39 year old female and 5ft 8 and have suffered this from the age of 15. I had xrays done from age 15 and they said that both my wrists were abnormal but nothing to worry about. It first came to my attention while doing typing at school. Over the years they have gradually increased in size and the pain has been unbearable at times. November 2002 I changed my job to a more office based job and began using the computer for the first time. Last August I was off sick for a month as I could not move my arm. Work carried out a work station assessment as they were concerned of rsv. My right wrist gives me the pain but apparently my left is more abnormal but I am right handed. I am making a conscious effort to use my left. My bone gets so large at times that the vein is so prominent that you would think it is going to explode.I have had anti inflamatories and pain killers but they are no use. I am worried about becoming addicted to Dihyracodine. I went to see a hand surgeon in _ who diagnosed this but he feels it is a huge decision to operate and it is my decision. He discussed 2 ways, shortening the bone and putting in a plate or removing the bone. Both carry high risks. If there was a guaranteed op I would be first on the list. I feel happier that I finally have a diagnosis and now that I have gone on the web I do not feel like an alien. All I wanted was to see even one person who had this. I really do not know what to do. Surgeon said if any op it should be removing the bone but there have been reports of people suffering from their wrist clicking which is more painful, but it may not happen to me. I have to give it some thought and get back to him when I am ready or I want more info. He has advised me not to discuss it with my gp as they will say Mr _ great surgeon, go ahead but they are not aware of the technicalities. I would welcome some support.
B
i contacted you in 2004, and you gave me the link to get into the support group but I lost it. My wrists are so painful at the moment I am considering surgery, I would like some support.
From T2 in Canada in e-mail contact with [ CL ] MWD
My 8 year old daughter has just been diagnosed with Madelung Deformity. I noticed she has had chubby wrists from birth, but I had never noticed anything else abnormal about them. A couple of weeks ago, she went to her cousin's birthday party and later that night I received a phone call from my sister-in-law. She had noticed that my daughter had weird shaped wrists and that her movement was a little odd. My sister-in-law had a friend whom had similiar shaped wrists, which was diagnosed as Madelung over twenty years ago, and she wanted me to meet her. I did a internet search that night to investigate this deformity and came across your website. The pictures I saw gave me a very uneasy feeling as I began to think my daughter could very well have this problem. I went to see my sister-in-law's friend the next day and I could not believe the similarities between this woman's wrists and my daughter's wrists. I was now convinced that my daughter had Madelung Deformity. I made a doctor's appointment right away, only to find out that my doctor had never heard of the deformity. I showed the doctor all the information I had found on the deformity, including the movement tests. She conducted both tests in the office, and my daughter failed both tests for both wrists, so the doctor decided to send my daughter to have x-rays. Thanks to the radiology pictures on your site, once I saw the x-rays I knew for sure that my daughter had Madelung Deformity, although the radiology techincian also had never heard of the deformity. Thankfully, the radiologist had heard of Madelung Deformity and my daughter was diagnosed with it. My main concern at this point is what to do from here. My daughter currently has no pain associated with her wrists, but according to the testimonies I have read on this site, it is only a matter of time. I am concerned that because of her age I will have to wait until pain occurs prior to having any options due to her immature skeletal system. I am also concerned about the lack of knowledge I have encountered thus far in the medical profession. Perhaps surgery may not be an option here in Canada due to a lack of expertise. I would appreciate any information I can get at this point. Particularly from people who may have been diagnosed at a young age that could give me an idea of the progression of the deformity between the 8-15 age period. Thanks
I would like to join the group, particularily for conversion for my daughter as she grows and learns more about the deformities, as well as a support group for myself as I deal with the road that lies ahead. As far as I can tell, there is no known relative with physical signs of the abnormalities (MWD or DCS) on either my parents side or her father's side. However, our family does have people who are relatively short (5' to 5'4"). In fact, I fall within that range and I have short arms and legs, but not excessively so. I also have large calves and have a stocky bone structure. The movement tests that were perfomred are those that you have mentioned N1L,N1R, P1 and P2, although they were not measured. My daughter has limited movement of both wrists vertically and horizontally, but the actual limitations have not been measured at this point. Any idea of the youngest diagnosed person thus far. I have been told that MWD is not normally diagnosed until pre-teen years or at the onset of pain, I consider myself lucky to have her diagnosed prior to the onset of pain.
Followup.
She has been other health issues that I was curious about-particularly whether there was any connection. Firstly, she has shortened achilles (not sure of spelling) tendons, which is scheduled to be corrected on Feb 16th, 2006. Secondly, she has extremely pigieon toed feet, which the specialist says should correct itself when she reaches puberty. And, lastly, she has dealth with chronic constipation with frequent overflows ("accidents in her underware"). My family doctor has told me that is not a huge concern as constipation is a common problem. After many attempts to have something done, she prescribed lactulose. I feel the problem is much more severe and the solution must be better than a gentle laxative. Are there any readers who have such bowel issues?? I am at my wits end, as is my daughter, with the embarassment of these occurences (its been six years).
From K8 in the USA in e-mail contact with [ CL ] MWD
Hello,
I have madelungs and I was curious to know if you would recommend
surgury. I am only 18 and the pain is tolerable. Will it get worse with
age?
I am in the US. I dont personally own a screename this is my friends that
she lets me use. I wouldnt mind joining the group. Most of the research
i've done points towards not getting the surgery. I just wish there was
more information on this....I feel left in the dark, ya know? Well
thanks for the quick response!
Bye,
K
From C7 in the USA in e-mail contact with [ CL ] MWD
I tried reading and understanding your article on DCSMWD but got lost
somewhere. I am a 57 year old female born in sweden, my mother had this
affliction but is deceased. My aunt in sweden has it severly in both wrists,
her hand being almost 1-1/2 inches below th wrist area. Her daughter is fine yet
her grand daughter has it but not yet diagnsoed. My youngest of three
children have it and has been diagnosed and had three surgeries on her arms,
helping lightly but she still remains in pain. She has also been in contact
with you as we were sent this website by my family in sweden wishing to know
more. Myself, i have it in my left wrist but it appears it has calcified
since i have limited movement in that wrist, i am also not diagnsoed with
it. My question here being, is it possible for this to affec the legs, i.e.
the ankles?...mine hurt beyond description and take pain meds. I also have
osteo arthritis and poly neuropathy in my legs and arms.
P.S. In my family, a total of 10 members, living and decesead have been
affected by this disorder, possibly more since this is just my recollection.
I thank you for your speedy reply, greatly appreciated. I currently live in the US in the state of Pennsylvania. I would very much like to join the group and be added, i also have no prolem with my name ro emailaddress being given out.Pleae furnish me with the link i need for the group. I am experiencing extreem pain and discomfort in my anles more so than the wrists but as you said, if it affext other areas as well, i need to make sure my doctor knows what to test me for, even at this stage in my life. As i indicated, way too many family members in my family have been affetected by this and it could possibly be even more than the 10 i am aware of ( 4 USA,6 Sweden) . This just being my immediate famly, not second or third cousins and such. It also seems that the new generation in my family is producing more with this affliction then ever before. I
am eagery awaiting your reply and link, again i thank you for your reply.
From L3 in the USA in e-mail contact with DCS
My 13 year old daughter was just dx with this condition. I was very happy to find your site. I was wondering if anyone has experience with the ilizarov technique?
From C3 in the UK in e-mail contact with [ CL ] DCS + MWD
Hi,
My name is C _ , I live in the UK and I have Leri Weill syndrome and as a part of my Leri Weill, madelungs deformity. I was treated with growth hormone treatment from the ages of 9-14 and I now measure 1m41.7, not exactly a giant but I was told the treatment would prevent any discomfort as I got older. I was fascinated to find your site and it's links to Leri Weill syndrome. The medical profession were fascinated when they found me and used me rather as a guinea pig, I'm in the _Journal ! I was wondering if you know of any other Leri Weill sufferers I could talk to (other than members of my family whom also have the condition). I'd absolutely love to discuss my condition! Hope to hear from you soon!
I would really like to talk to others that
have been treated as I can't help but think I was a guinea pig as depite the
treatment I get pains in my leg, and their reassurance was that although the
treatment may not make me much taller it would prevent any pain as I got
older. If I find that the treatment does not prevent pain in Leri Weill when
I have children, if they are sufferers I will not put them through it. I am
affected and it comes from my fathers side so my half sister is affected, my
grand mother was, my aunty and all her daughters are and the next generation
down also. Though Leri Weill is prevalent in female my father and brother
are also of short stature. It sounded so familiar reading about that first
Leri Weill case, especially about most of the family being normal height!!!
So yes I would love to hear from others!!!
Thanks so much.
It's difficult to say how many of us are affected, if I could get hold of my
medical notes that would be extremely useful as when they 'discovered' me
they chased up the entire family so that they could get an understanding of
how it acts genetically. Also we're a very big and widespread family so it's
hard to say! These are ones I know of, either diagnosed or showing to be
Leri Weill: Me, (Leri Weill comes through tmy fathers family),my sisters, my half
sister, her mother, my aunty, my grandmother, two or three of my female
cousins, two of my second cousins. On the male side it is more difficult to
tell as Leri Weill is shown more distinctly in the females but my father has
Leri Weill and madelungs, my brother shows some signs, but he may grow as he
is only 15, a couple of my cousins on that side also look like Leri Weill
sufferers. I will look into seeing my notes so I can confirm these things!
followup, 2006
I'm now twenty five week pregnant and have been through all the genetic
testing. We were told that it was 50/50 as to whether our child would have
the condition or not, what we were also told which I had not realised was
that there was no middle ground where Leri Weill and parental heights were
concerned. Thus it would appear I am not having a Leri Weill child..
this however brings other difficulties as my partner is 6ft2 our child is
on the 'high' part of the growth scale and I have been told I will most
likely be having an elective caesarian section. I would really like to know
how this other girl got on and how he was treated by the medical profession
as due to having been previously studied this pregnancy has been a source
of great interest to the medical profession...thus we have seen three
geneticists and two consultants and have had four scans...already!
From L3 in the UK in e-mail contact with [ CL ] MWD
Thak you so much for all the information on your web site. You've put in so
much work. My daughter, aged 13, has 'a madelung deformity with knobs on'
(orthapedic surgeon's words). She is left handed and the problem is, of
course, in that wrist.We've seen a man in the _ who has shown her x-rays
to a meeting of paediatric orthapedic surgeons who all say they have never seen
anything like it. Until about a year ago the were no problems but now it
seems that there is nothing but problems. She plays the violin and this is
becoming more dificult, fences and this is now more dificult and of course
writing is a big problem. The wrist is being scanned tomorrow as there is
anecdotal evidence of a similar problem in Australia which was helped by
removal of tissue?? Have you ever heard of this. I see from your page that
this is not an easily solved problem and I wonder if there is an expert in
London who deals with this.
It all seems so terribly hopeless that a 13 year old is being written off. I
would be grateful for any advice you can offer.
From H2 in the USA in e-mail contact with [ CL ] MWD
My name is H - I am 33 years old and have had problems with my wrist
for years. Yesterday I finally went to see an orthopedic surgeon. He
informed me that I had a rare deformity called Madelung's Deformity. I saw
my X-rays and was amazed at how much my wrist bones overlap on my hand bones
and seem to be completely separated from them on the side. The top of my
hand sits about an inch below my wrist. I was told that there isn't much
that can be done until I'm much older and in severe pain. How will this
affect my ability to work and will I be forced to go on disability just to
take care of my little girl? What are the chances that I will pass this
down to my little girl? I have an older sister who's arms are shorter than
mine. Will she experience the same problems?
I would love to be able to receive guidance from others that have my same deformity.
As I said, my doctor said that it was very rare. My older sister,
36, has an even shorter forearm and I am assuming that she also has this
deformity because her wrist is also starting to look like mine only not nearly
as defined. I hope that she doesn't have the same trouble that I do. I was told
by my mother that this deformity was in most of my grandfather's side of the
family, so I don't understand why websites that I have visited are saying that it isn't
inherited. I have had this condition for years without knowing I had it. I have
always worked very hard in factories and played sports such as vollyball,
basketball, and softball. The deformity shouldn't have any
effect on how you play a sport. I am 4' 11".
I have always been shorter than just about everyone over
10 years of age.lol. I'm not embarassed by something I can't control.
If I can, would you like x-rays or pics of my wrist?
I will try to send you a digital pic if you want one.
,
H,
Ohio
From A6 in the USA in e-mail contact with [ CL ] MWD , DCS?
My daughter E, now 15, was diagnosed with Madelung's Deformity of her left wrist
when she was 9 years old. At the time she had just started palying the violin
and it was the violin teacher who noticed that she was not able to extend her
harm fully with her palm up: It came as a shock to me. I immediately took her
to the Orthopaedist and after an X-ray they made the correct diagnosis.
They said it would be progressive and assured me it was not connected
with any other disease of the skeleton E has alweays been shorter than other
kids her age, although not pathologically shorter, plus her legs have toed in
since birth and the Orthopadist always told me she would outgrow it. Now at
15 sh eis still visibly shorter (5feet) and not showing signs of further growth.
Another doctor who saw her in Italy said her legs situation is also partly a
deformity. Nobody wants to intervene, only if she herslef will want to have
surgery for pure aesthetic reasons. E has never had pain so far. She often wears
bracelets to cover the bump and diminish the curve on her left forearm.
What should we do? We are afraid this will impact her teen life an it surely
does tocertain extent: she does not want to engage in any competitive sport
although her functionality is very good. What progress and where has been
made in trems of surgery and aestethic surgery. The situation has fully
stabilized and from now on she can have surgery whenevr she wants to.
My daughter has often gone into your site but never wanted to participate herself.
Nobody, to our knowledge has ever had Madelung appearance.
Two of the grandparents are short, but not pathologically.
We do not know further back than the Grandparents.
Thanks. A ( P. S. They never tested her for discondrosteosis.)
From P in the USA in e-mail contact with MWD
I came across your site while browsing for info recently. I like your site.
For your information and individual research, I wanted to let you know that
I have Madelung's. I'm from the U.S.. I have had corrective surgery on my
left arm in _ _ at _ Hospital. My sister (there are
five siblings total) also has it, albeit a lighter version.
I thought you might want the information for your site. If you have
questions, please email me.
Thanks
From S8 in the USA in e-mail contact with MWD + DCS
I first read your website a couple of years ago, and it was enlightening.
I am a 55 year old woman, with both Madelungs and dyschondrosteosis.
All four of my children have various skeletal deformities.
I am very interested in corresponding with others who know more about the complications of this.
I thought it was interesting that you mentioned people
not wish to discuss it. I think I understand that decision,
as to admit any type of flaw, be it our own fault or a genetic error, can be very difficult.
I suppose that I am in a sense "forced" to admit to having this condition because it has
become increasingly bothersome as I age.
From S3 in Australia in e-mail contact with MWD
Hi, my name is S _ from Sydney Australia. My 11 year old niece as just been diagnosed with madelung yeasterday. 30/11/2005. My family and I do not know much about this condition, but am wanting to find out more about this. A doctor she went to see yesterday at a hospital in Sydney call _ _ said, "She has the worst case with her left wrist that he would have ever seen or read about."
Since I have Started read alittle about this, I have self dianosed myself as haveing this condition myself. Due to the fact that when I was 11 years old I broke my wrist and damaged the growth plate in my right wrist, making it grow off 2 the left. When I was 15 years old I went and saw a specialist about my wrist as well due to pain, but nothing of this Madelung was even mentioned, he said I can operate, so I went on the waiting list where he said it would take 3 to 6 months before it would happen. Anyway 15 years later it's still not operated on, due to the fact that after 2 years on the waiting list he went private and said for about $25,000 he would operate, I said forget it. I never went to see another doctor about this unfortunatly because it has left me so I have virtually no use with my right wrist, I have very limited wrist movement, I cannot lift things over 2 kg's I find it hard to keep job because my wrist is constantly sore. So I want to help my neice with her wrist deformity, I was wandering if you know of anysupport groups for madelung in Australia.
I am 30 years old now and am thinking of once again getting my wrist attended to at the same time as my neice.
Thanks S
From S9 in the USA in e-mail contact with MWD
I'm a 34 female _ from the Boston area with MWD, diagnosed 6
years ago after years of bilateral wrist pain. I've found a surgeon in
Kentucky who's done extensive research on various genetic wrist deformities
and has created a prosthetic that mimics the DRUJ (distal radio ulnar
joint). I'm having the device implanted in late December (2005) by this KY
surgeon (and one of the 6 hand surgeons that I've seen over the years), at
_ in Boston. All the 70+ patients who've had this device
implanted have been able to get back to their normal activities.
http://www.aptismedical.com/
I'd be willing to give you some constructive critisism, if you're inclined
to listen.
Most of the people who find your web site are not geneticists and the information you provide may leave them more confused and like much medical jargon they've already encountered, disenchanted and overwhelmed. Please consider re-writing not to show your literary and genetic acumen, but your compassion at bringing the content to a level where most people can understand and feel empowered.
That been said, I want to sincerely thank you for your web site. I found it interesting.
From A7 in the USA in e-mail contact with [ CL ] MWD
I am a 25 year old female who has had Madelungs deformity since birth. It is
very hereditary.I got ot from my fathers side.My grandmother had it and
passed it on to a few of my aunts and uncles too.Two of my aunts have it and
my father.One of my aunts had a identical male twin who i assume had it
too.The other aunt passed it on to her oldest daughter and her daughter may
have passed it on to her youngest daughter(she has shorter forearms).I am 1
of 3 childern and my father is one of 8. I was warned that if i have
childern there is a 50/50 chance they will have it too. I have had 3
operations to try to correct the problem ,they have helped a little bit but
i still have problems.My family is on the shorter side most of us under 5'4
me being the shortest at 4'9''. If you see my fathers side of the family
walking down the street you can tell that we are family we all have the same
build(the ones with madelungs)Its a hard painful road and it hurts to know
that it will never go away and it can never be fixed. One of my operations i
had was to reset the bone giving it a straighter appearance and to make me
more confortable.I had a metal plate about 5 inches attached to my ulna but
a year later my body rejected the plate.My options ran out.I am a
hairdresser and work with the pain on a daily basis.I always hear comments
but just ignore them.I really have no clue how rare this disorder is but am
curious to the statistics.My family seems to be 50/50. I am the middle child
which means 50/50 because i am female.My cousin has 3 kids 2 girls 1 boy.The
boy was first (doesnt have it )Then her 2nd daughter-doesnt have it then the
third who shows some charecterists of having it. 50/50 with the girls.The
gene is dominate in females.