Clenched fists in the sagas

The crippling, hereditary “Viking Disease” has new treatments, but still no cure

Photo: MikkTooming / Wikimedia Commons
Dupuytren’s Contracture causes connective tissue in the hands to stiffen, pulling fingers toward the palm. It’s a progressive disease with no cure.

Judith Gabriel Vinje
Los Angeles

My 22-year-old granddaughter has the early signs of Viking Disease—a hereditary affliction of the hands that can result in permanently clenched fingers. Tradition has it that the disease originated with the Norsemen, who spread it throughout Northern Europe and beyond.

Being Norwegian, Danish, and German, she certainly has the genes. Centuries ago, Viking raiders, traders, and settlers left behind a set of genes that would rise up to plague future generations. It is their descendants who are the recipient of genes for blond hair, blue eyes, and Viking Disease—Dupuytren’s Contracture.

Genetic disorder
Viking Disease is a genetic disorder that mainly affects older people. It is usually progressive. The hand develops a crippling condition medically known as Dupuytren’s Contracture, or Dupuytren’s Disease. It is named after Napoleon’s surgeon, Baron Dupuytren, who reported on it in 1834. [Dupuytren’s is pronounced Doo-pwe-trens.]

There is good reason it is technically called Dupuytren’s Contracture. The condition results in some of the fingers becoming permanently clenched—claw-like—due to the formation of stiff “cords” in the palm. (In Norway, it is known as krokfinger—crooked finger.) You can’t put your hand in your pocket, you can’t wear a glove, you can barely do anything with the clenched hand. There is no cure, although there are steps doctors can take to alleviate the condition.

It is unusual for someone as young as my granddaughter to manifest the disease. Her symptoms are minor. There is a raised area on the palm of one hand, and the beginning of swelling of the cords that lead to the fingers. She’s been advised to “monitor it.” It may be many years before her case gets any worse, and it might not change at all. She says that her hand is sometimes sensitive and achy, but her fingers are definitely not yet involved.

She’s seen a case that’s somewhat more developed than hers. Her 80-year-old German-American paternal grandfather has it. On his right palm, you’ll see a distinct “V” where the cords are slightly swollen. (The V is coincidental—it’s not code for Viking.) But he’s lucky; there is no bending of the fingers. We hope she will be lucky too, and that a cure will be found before she reaches her older years. But, as she notes, “I’m proud of my heritage—even if it’s painful.”

Krokfinger in Norway
The highest incidence of Dupuytren’s Disease is found where Vikings made their home—in Iceland, Norway, Denmark, Sweden, Germany, the UK, as well as Australia and other places Scandinavians migrated to. It is a progressive, incurable disease affecting millions worldwide. (There also are cases found in Spain and northern Japan. Viking destinations?)

Scandinavian and British immigrants brought the genes along with them when they came to the U.S., settling heavily in the northern and Midwestern states.

Photo courtesy of Judith Gabriel Vinje
A very early sign of Dupuytren’s, the raised area in Vinje’s granddaughter’s palm (on the left side) is sometimes achy but not yet causing a problem for her fingers.

As many as 25 percent of people over 40 in Western countries have it. But it has also been seen in a 12-year-old boy. In the U.S., more than 10 million Americans have bent fingers from the condition, with 20 million more in earlier stages with skin tightness or a lump in the palm, like my granddaughter.

Dupuytren’s runs through the blood, meaning it is a hereditary disease, although there are factors that may trigger it. There are at least nine genes involved. Some are inherited from the mother, and some can come from either parent. Other than heredity, medical professionals still don’t know what causes it.

The disease has baffled surgeons for centuries. The only recorded cures have been reputed miracles. In the Norse sagas, there is a reference to the skald Bjorn Krepphandi—whose name means “crippled hand.” The sagas also record four incidents of miracle cures set in Orkney and Iceland in the 12th and 13th century. The condition of the hands that are supposedly healed by priests bears a resemblance to Dupuytren’s—“fingers clenched in the hands.”

In The Longer Saga of Magnus, we read of a man named Sigurd from the north of Shetland, which was heavily settled by Vikings. He had “cramped hands,” so that all the fingers laid against the palm. “He sought the halidom of the Saint Earl Magnus and there he got his cure with straight and lissome fingers for all his needs.” The sagas also tell of a servant woman whose bent fingers were freed when someone kicked her in the hand.

Knots and frozen fingers
In Dupuytren’s Contracture, the connective tissue under the skin of the palm contracts, keeping fingers from straightening. Knots form under the skin, eventually forming a thick cord that can pull one or more fingers into a bent position.

The ring finger is most commonly affected, followed by the middle and little fingers; the thumb and index finger are only rarely affected. More than one finger can be affected. Dupuytren’s contracture progresses slowly and is often accompanied by some aching and itching.

It is a capricious disease. Sometimes, the nodules go away without any treatment. And not everyone with nodules will experience contracture. Others have crippling recurrences even after many operations, according to the Dupuytren’s Foundation, which is compiling an international data bank in its research efforts to find a cure through DNA sampling.

Expanding treatment options
In the past, Dupuytren’s disease was almost always treated with surgery. Surgeons now perform more than 70,000 procedures each year. In a very few extreme cases, amputation has been necessary.

Two new treatments have been developed that can be done in a doctor’s office. One involves a collagenase injection of a new product, Xiaflex, followed by manipulation of the fingers. Xiaflex is injected into the hand to dissolve the problematic collagen. A day later, the doctor tries to straighten the finger. Several injections, spaced over months, might be needed, especially if more than one finger is affected. Xiaflex is very expensive, but the manufacturer assists in paying.

Another new treatment involves making many small punctures of the diseased cords with a needle and then straightening the fingers. The recovery time for both procedures is minimal and the early results are promising.

Mild cases of the condition can be eased by stretching exercises, according to WebMD. Radiation therapy is also being used. Nonetheless, a real cure has not yet been found, although research is continuing, according to Dr. Charles Eaton of the Dupuytren’s Foundation, who has introduced a new treatment with the U.S.

The Viking Disease is a silent epidemic that usually isn’t noticed until it is a severe problem. Geneticists are trying to find the exact cause and are making some progress. For young patients with Dupuytren’s Contracture—like my Norwegian-Danish-German granddaughter, a true Viking princess—let’s hope they stay proud of their unique heritage and that an end to the disease will be found in the near future.

Minneapolis-born Judith Gabriel Vinje has been a journalist for nearly 50 years, including a stint as a war correspondent. Now a Los Angeles resident, she started writing for Norway Times in 1998, and has been with the paper through its merges and changes. An active member of Sons of Norway, Edvard Grieg Lodge, Glendale Calif., she is also a member of Odins of Raven, a Viking reenactment group on the West Coast, and writes frequently about Viking Age subjects for several publications.

This article originally appeared in the March 24, 2017, issue of The Norwegian American. To subscribe, visit SUBSCRIBE or call us at (206) 784-4617.