Dwarfism

Dwarfism is a condition in which a person, animal or plant is much below the ordinary size of the species. When applied to people, it implies not just extreme shortness, but a degree of disproportion. Dwarfism is now rarely used as a medical term and is sometimes (but not always) considered impolite or pejorative. Today, the term little person tends to be preferred.

Of the hundreds of causes of dwarfism in humans, most are genetic, and most involve single gene variations that affect the structure or metabolism of bone, cartilage, or connective tissue. This class of disorder is referred to by physicians as skeletal dysplasias. Chondrodystrophies, chondrodysplasias, osteochondrodystrophies are also used (fairly interchangeably) in the medical literature to refer to most of these conditions. In many of these conditions, the bones are the primary affected body part, and the person is otherwise healthy. In many others, the genetic difference affects other body systems, causing unusual features or other major problems. Achondroplasia is one of the most common and perhaps the most easily recognized skeletal dysplasia. It affects approximately 1 in 40,000 children, both males and females as it is due to a mutation of an autosomal gene.

Problems faced by people with these conditions

• Social and employment opportunities are greatly reduced, regardless of anti-discrimination laws. Self-esteem and family relationships are often affected.
• Extreme shortness can interfere with ordinary activities of daily living, like driving or even using countertops built for taller people.
• Many can have problems produced by the abnormal bone structures. Early degenerative joint disease, exaggerated lordosis or scoliosis, and constriction of spinal cord or nerve roots can cause pain and disability. Reduced thoracic size can restrict lung growth and reduce pulmonary function.
• Many of the conditions are associated with disordered function of other organs, such as brain or liver. These problems can be more disabling than the abnormal bone growth.

Diagnosis

Shortness is usually the concern that brings the child to medical attention. Dwarfism becomes suspected because of obvious physical features, because of an obviously affected parent, or because body measurements (arm span, upper to lower segment ratio) indicate disproportion. Bone x-rays are often the key to recognition of specific skeletal dysplasia. Most children with suspected skeletal dysplasias will be referred to a genetics clinic for diagnostic confirmation and genetic counselling. See www.dwarfism.org for a list of American referral centers with special expertise in skeletal dysplasias. In the last decade, gene tests for some of the specific disorders have become available.

Medical treatment

For many forms of dwarfism, the best medical treatment that can be offered is accurate diagnosis, prognosis, and genetic counselling information.

Treatments to enhance height have major drawbacks. Growth hormone treatment has been shown to accelerate growth velocity over 1-2 years, but it may make a relatively small difference in adult height and involves years of expensive injections. The Ilizarov limb lengthening surgery was pioneered in Russia in the 1970s and is now performed in many places. Other newer devices such as the orthofix and intramedullary skeletal kinetic distractor (ISKD) are also used for limb lengthening. It can add over 6 inches to per bone (there are two in the leg), but is expensive, painful, and time-consuming. Some insurance companies may be willing to cover the cost. Each procedure lasts around 8-12 months. Typically two bones are done at a time (the same one on each side), but a patient can do more than that if they're willing to endure the extra pain and inconvenience.

Orthopedic or neurosurgical operations may be needed for specific problems associated with some of the conditions.

In the last decade, enzyme replacement treatments have been devised for a few of the inherited metabolic diseases, most of which involve more severe problems than short bones.

Terminology

Little person (as opposed to big person), and short-statured are currently preferred terms to refer to a person with extreme, disproportionate shortness. Dwarf is sometimes perceived as having negative connotations, although the term is often used by those affected. The plural is dwarfs — dwarves is used only for the imaginary creature. In the 19th century both dwarf and midget were ordinary medical terms referring to persons of disproportionate and proportionate shortness, respectively. Like many other older medical terms, they became primarily pejorative as they entered popular use. Midget is now considered offensive in all contexts to most, but not all, little people.

Dwarfism and extreme shortness in popular culture and the arts

The Dwarf Don Sebastián de Morra, by Velázquez. In his portraits of the dwarfs of Spain's royal court, the artist preferred a serious tone that emphasized their human dignity.

Dwarfism is such an obvious difference that it attracts the curiosity, humor, and imagination of most other people. "Normal" people of most cultures have paid just to look at dwarfs or to keep them around for amusement (e.g., dwarf tossing). In many times and places this may be the most attractive employment option available, as was the case for some jesters of medieval Europe.

When depicted in art, literature, or movies, dwarfs are rarely depicted as "regular people who are very short" but often as a species apart. Novelists, artists, and moviemakers attach special moral or aesthetic significance to the "apartness" or the misshapenness.

Artistic representations of dwarfism can be found on Greek vases and other ancient artifacts. Many European paintings (especially Spanish) of the 16th-19th centuries depict dwarfs by themselves or with others.

Several novels have treated dwarfism as a major theme, not necessarily realistically:

• The Tin Drum (Die Blechtrommel) by Günter Grass
• Stones of the River by Ursula Hegi
• The Dwarf by Pär Lagerkvist
• A Prayer for Owen Meany by John Irving

Leslie Fiedler's Freaks: Myths and Images of the Secret Self (1979) is considered an intelligent and sensitive exploration of the cultural significance and artistic treatments of differentness. Other readers may feel that he valued physically unusual people for their differentness, not for their personhood, when he laments medical treatment for reducing the number of picturesquely different people around.

Several twentieth century movies have addressed the topic or made much use of dwarfs:

• Freaks (1932)
• The Terror of Tiny Town (1938)
• The Wizard of Oz (1939)
• Even Dwarfs Started Small (Auch Zwerge haben klein angefangen) (1970)
• The Tin Drum (Die Blechtrommel) (1979)
• Time Bandits (1981)
• Willow (1988)
• Simon Birch (1998)
• The Station Agent (2003)

The 1960s television series The Wild Wild West featured a dwarf, Michael Dunn, as the recurring character Dr. Miguelito Loveless, the brilliant but insane arch-enemy of Secret Service agents James West and Artemus Ward.

In addition, the actor and stunt man Verne Troyer has become famous playing the character "Mini-Me" in two Austin Powers movies, Austin Powers: The Spy Who Shagged Me (1999) and Austin Powers in Goldmember (2002).

Famous people with dwarfism

• Jason Acuña, US actor
• Michael J. Anderson, US actor
• Kenny Baker, actor who portrayed R2-D2
• Billy Barty, US actor
• Bushwick Bill, US musician, founding member of The Geto Boys
• Sebastiano Biavati, 17th century curator of museum of curiosities
• Joseph Boruwlaski, Polish "count"
• Caroline Crachami, Sicilian dwarf
• Peter Dinklage, US actor
• Meredith Eaton, US actor
• The Doll Family, German-born siblings
• Josh Ryan Evans, US actor
• Nicholas Feny. French-born court dwarf
• Charles Flato, American writer and Soviet spy, he was also hunchbacked
• Phil Fondacaro, US Actor
• Jeffrey Hudson, English court dwarf and jester to Charles I
• Martin Klebba, US actor
• Wybrand Lolkes, Dutch dwarf
• George Washington Morrison Nutt, "Commodore Nutt"
• Alexander Pope, poet
• Meinhardt Raabe, oldest surviving munchkin from "The Wizard of Oz"
• David Rappaport, UK & US actor
• Judy-Lynn del Rey, US science fiction editor
• Timothy Roman, the son of US actress Susan Cabot
• Charles Proteus Steinmetz, scientist and engineer
• Charles Sherwood Stratton, "General Tom Thumb"
• Verne Troyer, US actor
• Herve Villechaize, French-born actor
• Lavinia Warren, US
• Chick Webb, big band drummer

External links

• www.dwarfism.org
• Little People of America (http://www.lpaonline.org/)
• International Center for Limb Lengthening (http://www.mcllr.com/)
• www.shortsupport.org for a variety of information related to short stature, and a somewhat cautionary view of limb lengthening surgery.

da:Dværg (menneske) es:Enanismo fr:Nanisme