Phocomelia
From Wikipedia, the free encyclopedia
 |
It has been suggested that phocomelia syndrome be merged into this article or section. (Discuss) |
| Phocomelia Classification and external resources |
|
| Alison Lapper Pregnant, by Marc Quinn | |
| ICD-10 | Q73.1 |
| ICD-9 | 755.2-755.4 |
| DiseasesDB | 10020 |
Phocomelia (from Greek φώκη = "seal" plus μέλος (plural μέλεα) = "limb") is a congenital disorder involving the limbs (dysmelia). An individual exhibiting phocomelia may be referred to as a phocomelus.
Contents |
[edit] Presentation
It presents at birth very short or absent long bones and flipper-like appearance of hands and sometimes feet. Amphibian deformities often take the form of phocomelia, in which the limbs are shortened due to lack or malformation of long bones, as opposed to other forms of dysmelia such as amelia, which is characterized by the complete lack of a limb, or polymelia, the presence of extra limbs, which are often fused together.
[edit] Causes
The condition may be inherited or occurs sporadically. It is also connected with prenatal exposure to the anti-nausea drug thalidomide. In Holt-Oram syndrome, the most severe forms manifest with phocomelia with rudimentary limbs.
[edit] Notable cases
Famous phocomelic people include Stanley Berent, also known as "Sealo The Seal Boy" (who made his living from performing / being exhibited in "freak shows") and, more currently, actor Mat Fraser, opera singer Thomas Quasthoff, guitarist Rick Renstrom and artist Alison Lapper. Marc Quinn's 15-foot-high nude sculpture, Alison Lapper Pregnant, is shown on the formerly-vacant fourth plinth in Trafalgar Square. Hee Ah Lee is a renowned pianist with only two fingers on each hand.
[edit] Phocomelus Characters in the Work of Philip K. Dick
In the science fiction of Philip K. Dick, three different phocomelus characters appear in three of his novels. The first is peripheral while the other two are central characters. In Chapter 2 of Martian Time-Slip (1964) there is a brief mention of Norbert Steiner's Wife's brother who was born without arms and makes use of artificial ones. The owner of the Red Fox Restaurant proposes that "those babies with seal flippers" should be euthanized. Steiner meekly agrees without mentioning his phocomelic Brother-in-law.
Dr. Bloodmoney, or How We Got Along After the Bomb (1965) depicts Hoppy Harrington, born phocomelus with minimally functional hands, due to thalidomide, and a brilliant handyman (referred to as a "handy" in the novel) due to his telekinetic powers. After the nuclear war that devastates Marin County as well as most of the world, Harrington becomes a darker character, with increasingly more powerful telepathic and telekinetic abilities.
In his later Deus Irae (1976), Dick and Roger Zelazny featured Tibor McMasters, an artist entrusted with the sacred responsibility of reproducing the image of Carleton Lufteufel, the avatar of the "God of Wrath" responsible for nuclear war, and worshipped by the "Servants of Wrath" cult. Ultimately, McMasters can only find a substitute for Lufteufel, but the Servants of Wrath accept the authenticity of the icon, and McMasters is canonised as a saint of the church in question after his own death for his services to religious art.
