dwarfism

Dwarfism is typically a pathological condition in which the physical size of a person, animal, or plant is well below normal. This article primarily discusses dwarfism in human beings.

Causes

Dwarfism is usually caused by genetic disorders. The most common cause is achondroplasia, in which individuals grow up with disproportional limbs. It accounts for 70% of dwarfism cases. Other causes include spondyloepiphyseal dysplasia congenita (SED), diastrophic dysplasia, pseudoachondroplasia, hypochondroplasia, and osteogenesis imperfecta (OI). Proportional dwarfism is usually caused by a hormonal imbalance. ^[1]

Symptoms

The distinguishing symptom of dwarfism is abnormally and significantly shorter stature with respect to other persons in the same age, and especially in adults who have reached their final heights. Many conditions that cause dwarfism also have other distinctive symptoms. Additionally, spinal stenosis can also be a problem, especially among adults^[1].

Diagnosis

Unusually short stature for a child's age is usually what brings the child to medical attention. Skeletal dysplasia ("dwarfism") is usually suspected because of obvious physical features (e.g., unusual configuration of face or shape of skull), 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 diagnosis of a specific skeletal dysplasia, but they are not the key diagnosis. Most children with suspected skeletal dysplasias will be referred to a genetics clinic for diagnostic confirmation and genetic counselling. (See External links, below, for a list of American referral centers with special expertise in skeletal dysplasias.) In the last decade, genetic tests for some of the specific disorders have become available.

During the initial medical evaluation for shortness, the absence of disproportion and the other clues above usually indicates other causes than bone dysplasias. Extreme shortness with completely normal proportions sometimes indicates growth hormone deficiency (pituitary dwarfism).

Short stature alone, in the absence of any other abnormalities, may simply be genetic, particularly if a person is born into a family of people who are relatively short.

Treatment

Treatment of most dwarfism arising from genetic defects is limited to palliation of symptoms and side effects; there is no cure.

Pituitary dwarfism can be effectively treated through administration of human growth hormone, if treatment begins early enough (before skeletal development is complete). The patient may still end up slightly shorter than average, but the inconveniences of extremely short stature can usually be avoided.

Short stature arising from normal genetic inheritance requires no treatment. The use of growth hormone to increase adult height in children who are short but otherwise normal is sometimes attempted for aesthetic or practical reasons, but remains controversial and risky. This controversy arises from the fact that there is nothing inherently bad about being extremely short statured.

Occasionally, limb-lengthening surgery is employed, but some older methods are painful and controversial. ^[1]

Insular dwarfism

Insular dwarfism is the reduction of size of large animals, especially mammals, when they are isolated over a small area. Since smaller animals need less energy, they are more successful in their small environment. ^[2]. Pygmy mammoths, dwarf elephants, and the sauropod Europasaurus are examples of insular dwarfism.

Problems faced by dwarfs

• Social and employment opportunities are greatly reduced, regardless of anti-discrimination laws. Self-esteem and family relationships are often affected, although not necessarily if the person(s) involved are emotionally healthy.
• Extreme shortness (in the low 2–3 foot [60–90 cm] range) can interfere with ordinary activities of daily living, like driving or even using countertops built for taller people.
• Many problems can arise from other abnormalities associated with pathological dwarfism, such as abnormal bone structure. 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 modern causes of dwarfism are associated on rare occasions with disordered function of other organs, such as the brain or liver. These problems can be more disabling than the abnormal bone growth.

Terminology

When applied to people, the term dwarfism usually implies not only extremely short stature, but also a pathological cause for the short stature. Persons who are simply very short in the absence of pathology (short people born into a short family, for example) are not usually referred to as dwarfs. According to the Little People of America, the human definition of dwarfism is "a medical or genetic condition that usually results in an adult height of 4'10" (147 cm) or shorter for men and 4' 7" (139 cm) or shorter for women, although in some cases a person with a dwarfing condition may be slightly taller than that."

Little person (as opposed to big person), and short-statured are common 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 as in "Peter Dinklage and Wee Man are famous dwarfs." The plural term 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, and can be taken as an insult, in all contexts to most little people, because of its connotation with little people being gawked at in circus/sideshows. Additionally, since proportionate short stature is usually due to pituitary dwarfism—a condition that is readily treatable—there are few people today who would qualify as midgets in developed countries.

In popular culture and the arts

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, including ancient Egyptian art. Documentation of dwarfs can also be found on European paintings and many pictures. 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, although not necessarily realistically:

• The Tin Drum (Die Blechtrommel) by Günter Grass
• Stones from the River by Ursula Hegi
• The Dwarf by Pär Lagerkvist
• A Prayer for Owen Meany by John Irving
• A Son of the Circus by John Irving
• Hop-Frog, or The Eight Chained Ourang-Outangs by Edgar Allan Poe
• Maybe the Moon by Armistead Maupin
• Swords and Crowns and Rings by Ruth Park
• Tale of the Wind by Kay Nolte Smith
• Memoir of a Dwarf in the Sun King's Court by Paul Weidner
• The Eye of Night by Pauline Alama

Leslie Fiedler's Freaks: Myths and Images of the Secret Self (1979) explored the value of differentness of "freaks" to "normal" people, lamenting medical treatment for reducing the number of picturesquely different people around.

Several 20th and 21st century movies & TV shows have addressed the topic or made much use of dwarfs:

• Freaks (1932)
• The Wizard of Oz (1939)
• Even Dwarfs Started Small (Auch Zwerge haben klein angefangen) (1970)
• Willy Wonka & the Chocolate Factory (1971)
• Time Bandits (1981)
• Willow (1988)
• Austin Powers: The Spy Who Shagged Me (1999)
• Austin Powers in Goldmember (2002)
• Monster Garage (2002-2006)
• "The Station Agent" (2003)
• Little People, Big World (2006) – Reality TV series following the daily lives of a family with two dwarf parents and one dwarf child (as well as three other children of average height)

The actor and stunt man Verne Troyer has become famous playing the character "Mini-Me" in two Austin Powers movies, as has fellow stuntman, and Jackass castmember, Wee-Man .

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 Gordon.

In the mid-1970's, Sid and Marty Krofft built an indoor theme park in Atlanta, Georgia called The World of Sid and Marty Krofft. This had a live stage production that was at that time the largest gathering of "little people" since the filming of The Wizard of Oz in 1937-38 as well as being the largest indoor theme park built to that time. The facility that was built to house this theme park is today the studios of CNN, the Cable News Network, and CNN Headline News.

In the 1990s, the immensely popular series Seinfeld featured a dwarf character, Mickey Abbott, in seven episodes; Mickey was played by actor Danny Woodburn. Notably, few references were made to his height, indicating an improved acceptance of little people in entertainment.

From 1999 until 2003, the popular television series The Man Show featured dwarfs in many of their segments.

In Monster Garage, Chris "Body Drop" Artiaga made his debut as a contestant in episode 'Ramp Rage', but later became parts runner for the series. In addition, there are 2 episodes featuring all-dwarf build teams.

In George R.R. Martin's A Song of Ice and Fire series, one of the main characters, Tyrion Lannister, is a dwarf. Though a brilliant and well-read man (some would say genius), he struggles with acceptance by "normal" people, who pejoratively refer to him as "the Imp," or "half-man". This is especially true of his father, Lord Tywin Lannister, who views Tyrion as a failure, especailly when compared to Tyrion's handsome, talented older brother Jaime, and Jaime's equally beautiful and talented twin sister, Cersei. Tyrion often wonders if any woman could ever truly love him in spite of his condition.