Download The local town of Gibsonton, Florida is located about 45 minutes

The local town of Gibsonton, Florida is located about 45 minutes southwest of Lakeland. This
town was once a home for touring carnival side show performers. It was a place where they were
accepted and felt a sense of community. In the 1920’s and 30’s the carnival sideshow displays of human
deformities were popular attractions. The performers would travel from city to city for more than half
the year and would retreat back to Gibsonton in the winter. These residents viewed as monstrosities
and human oddities now represent models for genetic mutation.
Al Tomaini was one of the Gibsonton’s most famous residents. In town he served as both police
and fire chief for some time. He then opened The Giant’s Fishing Camp complete with restaurant and
trailer camp. This was so named after his role as the giant in the side show world. Al stood 8 feet and 4.5
inches tall, weighed 356 pounds, and wore a size 22 shoe. His great height was due to a disorder known
as gigantism or acromegaly. Acromegaly is the result of a tumor on the pituitary gland which releases
important regulatory hormones such as human growth hormone. When released human growth
hormone enters the blood and stimulate the production of insulin-like growth factor 1 (IGF-1) via the
liver. IGF-1 is responsible for promoting bone and tissue growth. This system is naturally regulated in a
feedback loop, but the tumor on the pituitary gland disrupts this loop and does not allow for the
suppression of human growth hormone secretion. The formation of these pituitary gland tumors has
been linked to deformities in the aryl hydrocarbon receptor interacting protein (AIP) gene. How exactly
this gene functions in tumorigenesi s is not known. Unfortunately the same genetic mutation that made
Al Tomaini famous was also responsible for his premature death at the age of 50. He passed away on
August 30, 1962 leaving behind his side show legacy. Today his fishing camp and restaurant remain
popular tourist attractions in the town.
Another equally famous resident of Gibsonton is Grady Stiles. While Al Tomaini served as police
and fire chief Grady lived life on the other side of the law. He was born into a family with a long history
of ectrodactyly, a condition caused by different genetic mutations. The most common is a mutation on
chromosome 7. This mutation occurs in a region that contains the two homeobox genes (genes that
encode for physical development) DLX5 and DLX6 near the split hand/foot malformation type 1 gene
(SHFM1). The genetic mutations cause the middle fingers and toes of the person to be missing and the
remaining fingers of each side fuse forming a claw like appearance. This formation in Grady Stiles’ hands
and feet lead to his career as the side show performer The Lobster Boy. Grady’s malformation was so
severe that he was not able to walk. He relied on his arms to assist in movement allowing him to
develop great upper body strength. This strength he used against those dearest to him. Accusations of
being a drunk and being an abusive father and husband were only the start of Grady’s trouble with the
law. After two failed marriages Grady was a father of four, two daughter and two sons. It was the night
before his oldest daughter’s wedding that Grady committed his most severe crime. Not approving of his
daughter’s fiancée Grady shot and killed him in cold blood. At the trial Grady admitted his guilt without
remorse, but due to the prison systems lack of ability to care for a person with Grady’s condition it was
found to be cruel and unusual punishment for him to be detained. He was instead given a sentence of
15 years probation. After the trial Grady and his first wife rekindled their romance and remarried, but
unfortunately the conviction had not changed his ways. He continued with his drunken abuse of his wife
and children until a final act of violence stopped it all. This act was not one committed by Stiles, but
against him. On November 29, 1993 Grady Stiles was shot and killed by another circus performer hired
by Grady’s wife and son. All three were found guilty and served varying lengths of time in prison.
While love was not Grady Stiles’ forte it did exist between two other sideshow performers who
were also Gibsonton residents. Priscilla and Emmitt Bejano met in the late 1930’s while both were
appearing in an exposition. Their romance blossomed and the two eloped in 1938. They returned to the
spotlight of sideshow performance together and were promoted as The World’s Strangest Married
Couple. Priscilla who once performed alongside a chimpanzee as The Mokey Girl now stood proudly
beside her husband Emmitt The Alligator Skinned Man. Priscilla had been born with the disorder
hypertrichosis that caused excessive hair growth in addition she had two rows of teeth. Her thick black
hair covered her entire body and she grew a long beard and mustache. Her disorder is a result of an
insertion into the region q27.1 of the X chromosome. Insertions of both chromosome 4 and 5 have been
found to cause the disruption at the q27.1 region. These insertions take place within a palindromic
sequence near the SOX3 gene involved in hair growth. The importance of the palindromic sequence is
not known, but it is theorized that the insertion in the sequence causes the SOX3 gene to become upregulated thus causing the excessive growth of hair seen in Priscilla. Her condition resulted in significant
cosmetic symptoms, but unlike Emmitt’s disorder it does not jeapordize her health or increase chances
of other complications. Emmitt’s genetic disorder is known as ichthyosis vulgaris. It is characterized by
thickening of the keratin of the skin resulting in the formation of a scaly reptile appearance. This build
up may occur in one of two ways: the first is the natural shedding of the skin is slowed or completely
inhibited and the second is the rate of new skin cell production is increased and the natural shedding
process is unable to keep up with the increased rate. This abnormality has been linked to a mutation in
the filaggrin gene (FLG) found on chromosome 1q21. The FLG encodes the protein profilaggrin that
breaks down to filaggrin a protein normally found in the outer most layer of the skin, but found to be
lacking in ichthyosis vulgaris individuals. This disorder is passed from generation to generation by way of
autosomal dominance indicating Emmitt inherited his condition from one of his parents that also
expressed the disorder. In addition to his outward appearance that landed him in the sideshow business
his condition also comes with significant health risks. Because of the thickening of his skin Emmitt was
unable to sweat and thus unable to cool himself. Between performances he would submerge his body in
tubs of ice water to lower his body temperature and prevent overheating. In addition with his disorder
infection was a great concern. The cracking of the skin created wounds easily accessible to infectious
microbes. Despite these risks Emmitt enjoyed a long life with his true love Priscilla passing away on April
17, 1995. In mourning Priscilla shaved her beard and remained shaved until her death in February 5,
2001. Her gesture signified the attraction and foundation of their love was not found in outward
appearance, but rooted much deeper in the persons they saw within each other.
While the residents of Gibsonton were observed as sideshow freaks and unnatural beings in the
carnival and exhibition hayday. Their exploitation made them famous and took its toll on each individual
in its own way. While their paths in their personal lives may differ they all shared a common link in their
unique individuality caused by their disorders. These disorders unexplained and misunderstood are
coming into a new light as research continues. As new genetic discoveries are made this town becomes
less a collection of freaks and more a wealth of genetic knowledge.
http://iml.jou.ufl.edu/projects/Fall07/Caramanica/
http://www.teddwebb.com/legends/al_tomaini.html
http://www.sciencedaily.com/releases/2011/01/110107083729.htm
http://thehumanmarvels.com/?p=66
http://www.iamunwell.com/pdf/Diseases-Alphabet-E/ectrodactyly.pdf
http://jcem.endojournals.org/content/93/6/2390.short
http://stochasticscientist.blogspot.com/2011/07/genetic-cause-of-hypertrichosis.html
http://www.mayoclinic.com/health/ichthyosis-vulgaris/DS00734/DSECTION=complications
http://dermnetnz.org/scaly/ichthyosis.html
http://www.niams.nih.gov/News_and_Events/Spotlight_on_Research/2007/iv_filaggrin.asp