Dr. Greene, my 14-year-old daughter Jaymie has been diagnosed with a connective tissue disorder. The disease has two names -- Dupuytren's contracture in the hands, and Plantar fibromatosis in her feet. We have been told that before she graduates from high school she will be in a wheelchair for the rest of her life.
The disease is a genetic type of disease and it comes from my side of the family. They have told us that there is nothing they can or will do for her because she is so young. She has, however, been accepted to a wonderful hospital in Portland, Oregon (Shriner's Hospital for Crippled Children). What do you know about this disorder, and is there a cure for it? What is the prognosis for her life? Is there any good news that you can give me about a possible future? If not, what can we expect for her? The progress of the symptoms are getting worse fast and she has had some major difficulties lately.

Robi, most parents hold the deep, unquestioned belief that over the next several years we will watch our children grow ever more independent and more mobile as they walk out of our homes into the world beyond. This poignant, long departure that begins at the moment of birth provides a bittersweet backdrop to the many wonderful moments when our children are young.

How jarring, how deeply wrong, it must feel for you to be told that your teenage daughter is going to become progressively less mobile, will soon be wheelchair bound for life, and -- at the time she is becoming an adult woman -- needs to be cared for at a hospital for crippled children! You are in a discouraging and bewildering situation. Let me tell you what I know of her condition. I'm sorry that you both have to face this, but there is hope with the treatment options now available.

In the palms of our hands and the soles of our feet, we each have a tough fibrous layer called fascia (the palmar fascia and plantar fascia, respectively). In Dupuytren's contracture (pronounced du-pwe-trahns), one or both of these fibrous layers begins to grow awry. In palmar fibromatosis ("classic" Dupuytren's contracture) the palmar fascia slowly begins to thicken, and then shorten. The fingers are relentlessly drawn inward into a rigid, misbegotten fist. As flexibility slips away, so does the useful functioning of the hand. In plantar fibromatosis, this same relentless shortening happens in the soles of the feet, drawing the toes downward, folding the feet into a frozen fist, and making it impossible to walk. The foot version is much less common. Either way, untreated Dupuytren's contracture can be a crippling disease.

Dupuytren's contracture was first described by Baron Guillaume Dupuytren, a celebrated French surgeon of the early 1800's who was apparently successful with the surgical treatment of this condition. By carefully cutting the involved fascia he was able to achieve good results -- for a while. Whatever had caused the fascia to grow incorrectly before, caused the regrowing fascia to eventually shorten and thicken as well. Thus, for over 100 years the condition was thought to be relentlessly progressive. We now know that it can follow many courses, from quite mild to very severe. There are also more effective treatment options than ever before, and there is real reason to have hope for Jaymie.

Dupuytren's contracture is a genetic condition that is passed as a dominant trait with "variable penetrance." This means that, if it runs on your side of the family, it is present in someone in every generation, although it may be so mild as to go unnoticed. Either you or her father must have it, Robi, and one of your parents must have it as well -- although neither of you may ever have any symptoms from it at all. Whoever carries this gene will pass it along to about half of his or her offspring.

The real problem in Dupuytren's contracture is with the DNA in the cells of the fascia. DNA provides the blueprint that instructs the fascia how to grow. Fascial cells with abnormal DNA will eventually produce abnormal fibrous tissue time and time again. Really, then, Dupuytren's contracture may be classified as a benign tumor of the fascia.