Help for this difficult, disfiguring hand condition

If you are a person of northern European ancestry, you might be at risk for developing Dupuytren’s disease. Since many people on Cape Cod do, in fact, have that ancestry and Dupuytren’s is more common in older people, it is not an unusual problem in this area.

Dupuytren’s is a disease of the palm of the hand that is caused by a hardening and thickening of the tendons due to a metabolic process. These patients have a genetic code that signals the body to make too much collagen, which deposits in the palms of the hand. It can lead to one or more fingers becoming tethered into a permanently bent position. It can also occur in the soles of the feet.

Hand surgeon Julian D’Achille, MD, explained that the genetic component isn’t quite as simple as hair or eye color. Instead, it is a type of genetic disorder that has “variable penetrance,” which means the disease is only expressed in parts of the population.

“What that means is people with the genetics for Dupuytren’s disease get affected differently from person to person,” he said. “So, even though they may have the genes, how they are expressed in each individual person can be very, very variable.”

Some people develop nodules or pits in their palm, but it never progresses beyond that. Other people progress to having contractions in their fingers caused by an excess build-up of collagen that can be quite disfiguring and painful.

Fortunately, there are several treatments available. Which treatment, and even which doctor, is best depends on the stage of the disease when diagnosed.

Treatment for Early Dupuytren’s

Radiation oncologists at Cape Cod Hospital’s Davenport-Mugar Cancer Center and Falmouth Hospital’s Clark Cancer Center can treat patients who are in the early stages of the disease with superficial radiation.

“Radiation therapy can be used for benign conditions such as Dupuytren’s” said Jeffrey Martin, MD, a radiation oncologist at both Cape centers. “In the early stages of the disease, when it’s just painful nodules or progressive nodules in the palm, we can use radiation to try to halt that progression. Nodules can stabilize and, in some cases, soften or flatten, with the goal of preventing further progression which can lead to flexion deformity of the finger and require surgery.”

Treatment of non-cancerous conditions is part of the services offered at Davenport-Mugar and Clark. Treatment for early-stage Dupuytren’s is based on experience in Germany, where they use radiation for benign conditions more than in the U.S., he said. Many other cancer centers in this country are also offering such treatment, he said.

The radiation treatment for Dupuytren’s is considered superficial and penetrates the first centimeter of the skin, only, where the tendons are, Dr. Martin explained. Patients are referred by their primary care provider, specialists or sometimes come in on their own, he said.

“If they’re already having contracture in their hand, it’s unlikely radiation is going to reverse that,” said Dr. Martin. But, the progression for Dupuytren's is slow for many people; they can be stable for many years. When it starts to progress, or the nodules start to become bothersome, say, when gripping a golf club or working in the garden, “then radiation could soften it up and help improve function,” he said.

Treatment consists of the patient placing their palm up on the treatment table and the machine is lined up exactly to the area to be treated. Treatments take a minute or two and the patient does not feel any sensation, Dr. Martin said. Typically, for treatment of Dupuytren’s, there are five treatments, then an eight-week break, and then five more.

Side effects are minimal and may include redness and/or itching of the palm area that was treated, he said. Rarely, the skin peels, which can be uncomfortable while it’s healing. There has never been a report in the medical literature of any cancer related to radiation exposure during treatment for Dupuytren’s, he added.

Treatment for More Advanced Dupuytren’s

For more advanced cases of Dupuytren’s, Dr. Martin and other radiation oncologists refer patients to hand surgeons like Dr. D’Achille. Because of its obvious physical appearance, most cases of advanced Dupuytren’s can be diagnosed with a clinical exam, Dr. D’Achille said. X-rays are usually not necessary. The one exception is people who have just a singular nodule, which is known as a palmar fibroma. In those cases, it is hard to distinguish between a palmar fibroma and a cyst, so he often sends those patients for an ultrasound.

If people do have contractures (joints fixed at a specific angle), there are three possible treatments. Dr. D’Achille has been trained to do the two most successful ones. They are:

Surgery - “The surgery entails making some incisions in the skin and then physically removing all of the cord that you can see and feel,” he said. “The surgery works really well. It is almost always covered by insurance, so for some folks that becomes the default option because it’s covered.”

The recovery time is about two months and patients have to wear a splint 24 hours a day, seven days a week for the first month. Dr. D’Achille will also refer these patients to a hand therapist.

Injection with an enzyme called Xiaflex - This medication is specifically designed to break down the collagen that is within the cords that are typical of Dupuytren’s. Dr. D’Achille injects the Xiaflex at regular intervals along the cord. The injections are done without anesthesia because the nerves in our fingers run pretty close to the cords, so he wants to make sure he’s not putting the needle in a nerve because Xiaflex can actually eat away at the nerve as well.

He then allows the medication to sit for about 48 hours. After two days, the patient comes back, and he numbs their hand and manipulates the fingers back and forth to straighten them out and break up the cord at the spots where the enzyme was dissolving the cord.

“If you think of a rope that you made a couple of cuts into to fray it a little bit and then you really try to stretch out the rope, it will obviously break at a couple of spots,” he said. “That’s the idea with the Xiaflex. You’re eating away at some spots to weaken it and then you break it apart.”

The risks are fairly low. There is a small risk of bleeding and infection or injuring surrounding nerves. Even though it has never happened to him, Dr. D’Achille said there is small risk of rupturing the tendons that bend your finger. The most common side effects are bruising and swelling and occasionally a tear to the skin if the cord is really tethered to it.

Afterwards patients are also sent to a hand therapist, but they don’t have to wear the splint all the time. Dr. D’Achille tells them to wear it as often as possible for one month and only at night for the second month.

When Surgery is Recommended

There are several reasons Dr. D’Achille might recommend surgery over Xiaflex:

• It’s faster to do surgery if multiple fingers and joints are involved. Multiple fingers and joints require multiple injections of Xiaflex.
• Surgery is more effective for patients with severe contractures and for those who have had the contractures for many years.
• Patients who don’t like needles tend to prefer surgery because they’re asleep for the surgery.
• If the cord cannot easily be felt, surgery is much better because there is no way to know if the Xiaflex is getting into the right spot.
• Xiaflex is expensive and even though most insurance companies cover it, Medicare only covers up to 80 percent of the roughly $5,000 cost. Without secondary insurance, those patients would be responsible for 20 percent of the cost of treatment.

Unfortunately, Dupuytren’s is a progressive disease. Without treatment it will continue to get worse and even with treatment it can recur, Dr. D’Achille said. The recurrence rate with surgery is about 30 percent at 10 years. With Xiaflex it is about 50 percent at five years.