Dupuytren disease (pronounced DOO-pah-tren) is the most common disorder crippling hands that most people have never heard of. It is a genetic condition of the fascia beneath the skin of the palms, and it stubbornly resists treatment. An expert and a patient discuss the disorder.
- Tom Knapp, Dupuytren patient and board member, Dupuytren Foundation
- Dr. Charles Eaton, hand surgeon and Executive Director, Dupuytren Foundation
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Dupuytren Disease and Disabled Hands
Nancy Benson: Tom Knapp is and insurance executive in South Florida who loves to golf. About 25 years ago he started having problem with his left hand; he couldn’t straighten his fingers completely and it kept getting worse and worse.
Tom Knapp: My fingers started bending when I was 46 and the middle and pinky finger on my left hand started to bend towards the palm and when I got to be 50 years old I couldn’t put a golf glove on. I had a hard time putting golf gloves on and off because the fingers were bent so much and that’s when I had to have my first surgery.
Nancy: Then it started in his right hand.
Tom: Fingers started bending, I started getting cords in the palm of my hand. No pain with the disease, I was one of the fortunate ones, it just the fingers just kept bending more and more and more.
Nancy: Knapp has Duputren disease a genetic condition that afflicts an estimated 16 million Americans and at least to some degree.
Dr. Charles Eaton: Duputren disease is the most common crippling hand disease that most people have never heard about.
Nancy: That’s Dr. Charles Eaton, a hand surgeon in Palm beach county Florida, whose Executive Director of the Dupuytren Foundation.
Dr. Eaton: It is primarily an inherited condition that causes gradual changes of tissues underneath the skin of the palm. These changes are like scar tissue and as scar tissue matures it shrinks and this change that occurs under the skin of the palm also shrinks lengthwise. The end result is that it tethers the fingers into a progressively more and more bent positions, so the fingers can’t be straightened out all the way and that causes a characteristic deformity that typically looks as though the hand is just sitting in a resting posture with the fingers bent. So its something that doesn’t always catch someones eye unless the person is trying to straighten out their fingers.
Tom: A lot of people who are affected or have it, it doesn’t even bother them. I’ve got ten brothers and sisters and one of my brothers who’s 50 years old has a little cord in the palm of his hand – doesn’t bother him at all, but yet he has a disease and then my sister I understand now, she’s maybe 51-52, she’s got a little bit of bend in her finger but it doesn’t bother them. I mean she hasn’t even been to a doctor, and for a good majority of people, it doesn’t bother them at all. Then you have people who have the very advanced case like I have, where my hands basically are crippled.
Nancy: Knapp who’s now on the board of the Dupuytren Foundation says the disease is often mistaken for tendonitis or arthritis, especially at first. So someone who’s afflicted may see multiple doctors before they’re diagnosed correctly. Eaton says a hand surgeon is probably the best place to go for answer – they’re very familiar with the characteristic bending of the fingers. Doctors believe Dupuytren disease is the result of an overactive repair mechanism in the layer of material just beneath the skin of the hands.
Dr. Eaton: For most of the body you can slide the skin around side to side if you reach and push on your cheek, you can slide that skin on your cheek up towards your eye and down towards your chin, but if you straighten out your fingers all the way and try to do the same thing with the skin on your palm – it just doesn’t move. And the reason for that is that there’s a special reinforcing layers that’s just underneath the skin which is like a sheath of heavy canvas and there are thousands of little threads that anchor the under surface of the skin into the palm down to this sheet that’s made of a tissue that’s called ‘fascia’ that’s a common kind of tissue in the body. And then the undersurface of that reinforcing layer has extensions that anchor it down to the bones that are in the back of your hands and your finger bones. And this is critical for the use of the hand if you’re going to grab something and you don’t want it to slide around in your hand, you don’t have to worry about that ‘cause the skin is anchored tightly.
Nancy: But when this layer of fascia thinks it has to repair itself, it shrinks, tightly pulling the fingers down with it. Surgery can cut the cords and free the fingers but recovery takes months and Eaton says it doesn’t always go without a hitch.
Dr. Eaton: Because the biology has something to do with the wound healing process, the healing that takes place after surgery make also be abnormal. So people have the traditional open surgery for this condition may have more inflammation, more swelling, stiffness, and pain than you’d expect and the complication rate of doing open surgery for Dupuytren contracture is overall higher than you would expect compared to other hand operations. The chance of having a serious complication is about 4x that you’d expect for a other common hand operation such as treating carpal tunnel syndrome or trigger finger or even replacing an arthritic thumb joint. Because of those issues there’s been a trend to look to minimally invasive approaches.
Tom: What they can do with needles now, pretty good sized needles, is that they just go in and they punch holes in the fascia and pull the finger until it pops back straight again and they basically break the fascia with the XIAFLEX treatment same thing the bacteria eating flesh goes in there and eats at that fascia and the next morning they just pop the finger back into place. It’s a very expensive, it’s $3,000 a vial and they can only usually do one or two joints at a time. If you got 3 joints in your fingers that are all affected, they can usually only do the one joint at a time, that’s a $9,000 treatment if you do all 3 fingers, plus you gotta get shots every time and the shots – they put a needle in your hand and that doesn’t feel very good.
Nancy: All of those procedures can straighten out the fingers, but in severe cases only for a while.
Dr. Eaton: The problem is none of these are cures, so there’s a high return of the contracture or recurrence after any of these procedures. For people with aggressive Dupuytren disease they may have an operation and have it come back within a year or a few years, have another operation and each time you have a re-operation, it increases the changes of complications. So the natural course in people who have aggressive Dupuytren disease is that they wind up with many operations over their lifetime – complications and they still wind up with progressive crippling of their hand despite the best that surgeons can currently offer.
Tom: Over the last 20 years, I’ve had five, six, seven general surgeries and three other treatments. I haven’t been able to shake hands since my last surgery which was 2.5 years ago, ‘Cause if somebody were to grab my hand I would go right to my knees – I have no cushion left in my right, my thumb and my two fingers, on my right hand. All the fascia’s been taken out ‘cause it was all damaged, it was all diseased, so I don’t have any cushion in there all I have is nerves. So if I grab my money clip or if I grab anything on an edge, it could be a pack of matches or it could be anything that has an edge to it, it hurts my hand. So, round things I can grip and it’s not so bad but its crippling to the effect that I just can’t shake hands, I just give everybody a fist-bump with my left hand; that’s how I shake today.
Nancy: Those kinds of affects are bad enough but people with severe case of Dupuytren disease also have a higher risk for other disorders including heart disease and some cancers. So the Dupuytren Foundation is anxious to find funding for research into the genetic flaw behind Dupuytren disease in hopes they can identify how it works and stop its crippling affects. You can find out more about Dupuytren disease on the foundation website, 4dup.com. You can find out more about all our guests on our website, RadioHealthJournal.net and also find archives of our programs as well as on iTunes and Stitcher. Our production director is Sean Waldron. I’m Nancy Benson.