tag:theconversation.com,2011:/ca/topics/unusual-conditions-30865/articlesUnusual conditions – The Conversation2017-06-13T03:17:26Ztag:theconversation.com,2011:article/765232017-06-13T03:17:26Z2017-06-13T03:17:26ZAn extra organ or body part is more common than you think<figure><img src="https://images.theconversation.com/files/172590/original/file-20170607-3710-vrt3zk.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=496&fit=clip" /><figcaption><span class="caption">Society has long treated people with extra limbs as anatomical oddities. But having an extra body part or organ is surprisingly common and many people don't know they have them.</span> <span class="attribution"><a class="source" href="https://upload.wikimedia.org/wikipedia/commons/1/1d/The_Peerless_Prodigies_of_Physical_Phenomena.jpg">Ddicksson/Wikimedia Commons</a>, <a class="license" href="http://creativecommons.org/licenses/by-nc-sa/4.0/">CC BY-NC-SA</a></span></figcaption></figure><p>Doctors thought they were operating on a malignant tumour when they set about removing an unusual oval lump on the right side of a 40-year-old woman’s body. What they recovered instead was a perfectly normal and fully functioning <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4580026/">extra spleen</a>.</p>
<p>Most of us only have one spleen, an organ involved in immune function and blood filtering. But accessory or extra spleens are quite common, appearing in more than <a href="https://www.ncbi.nlm.nih.gov/pubmed/22764656">one in ten</a> people.</p>
<p>It is not unusual for people with extra organs to be completely unaware of their existence. Often they are discovered accidentally during diagnostic scans for unrelated conditions. While many of these extra organs are rare, others are far more common than many of us believe. Some need to be surgically removed and others can be left alone.</p>
<p>The extra spleen, described above, is an example of what doctors call supernumeracy, when the body has an extra organ, part or structure. </p>
<h2>Supernumeracy in history</h2>
<p>Supernumeracy has long fascinated us, with many obvious and peculiar examples throughout history.</p>
<p>Witch hunters in the 16th and 17th centuries often identified supposed witches by their <a href="https://www.ncbi.nlm.nih.gov/pubmed/17521013">third nipple</a>, although these extra nipples were often mistaken for moles or birthmarks.</p>
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<a href="https://images.theconversation.com/files/172596/original/file-20170607-3698-1b6re3v.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=1000&fit=clip"><img alt="" src="https://images.theconversation.com/files/172596/original/file-20170607-3698-1b6re3v.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=237&fit=clip" srcset="https://images.theconversation.com/files/172596/original/file-20170607-3698-1b6re3v.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=600&h=891&fit=crop&dpr=1 600w, https://images.theconversation.com/files/172596/original/file-20170607-3698-1b6re3v.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=600&h=891&fit=crop&dpr=2 1200w, https://images.theconversation.com/files/172596/original/file-20170607-3698-1b6re3v.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=600&h=891&fit=crop&dpr=3 1800w, https://images.theconversation.com/files/172596/original/file-20170607-3698-1b6re3v.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=754&h=1120&fit=crop&dpr=1 754w, https://images.theconversation.com/files/172596/original/file-20170607-3698-1b6re3v.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=754&h=1120&fit=crop&dpr=2 1508w, https://images.theconversation.com/files/172596/original/file-20170607-3698-1b6re3v.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=754&h=1120&fit=crop&dpr=3 2262w" sizes="(min-width: 1466px) 754px, (max-width: 599px) 100vw, (min-width: 600px) 600px, 237px"></a>
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<span class="caption">Myrtle Corbin, with her four legs, was a side-show star. But most examples of supernumeracy are more subtle or hidden.</span>
<span class="attribution"><a class="source" href="https://commons.wikimedia.org/wiki/Category:Myrtle_Corbin#/media/File:Myrtle_Corbin.jpg">Wikimedia Commons</a></span>
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<p>Then there are the famous cases in the era of the <a href="https://www.sheffield.ac.uk/nfca/researchandarticles/freakshows">Barnum and Bailey freak shows</a>, which displayed truly extraordinary examples of supernumeracy. These included the sideshow stars Frank Lentini, the three-legged man, and Myrtle Corbin, the four-legged woman.</p>
<p>Their conditions were the result of being attached to partially formed parasitic twins (also known as an asymmetrical or unequal conjoined twins) that had not fully separated during development. Both went on to marry other people and have normal children.</p>
<p>More recently was the internationally celebrated case of the eight-limbed Indian girl Lakshmi Tatma, born in 2005, who had four arms and four legs. Some considered her to be a reincarnation of a Hindu goddess. A 72-hour operation eventually separated her from her parasitic twin.</p>
<h2>What causes supernumeracy?</h2>
<p>Supernumeracy is caused by errors in how the embryo develops. While some of these conditions can be genetic, most occur spontaneously and have no known cause.</p>
<p>To understand how this happens it helps to think of the development of an embryo into a human as being like a finely tuned orchestra following the directions of a strict conductor.</p>
<p>Every player in the orchestra needs to know when to start playing and when to stop, how fast the pace should be and what instrument needs to dominate in every part of the symphony. If percussion plays too fast or strings come in too soon, it can end in a disaster.</p>
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<figcaption><span class="caption">Errors in embryonic development led to Indian girl Lakshmi Tatma being born with eight limbs.</span></figcaption>
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<p>Likewise, when the embryo develops, structures that will eventually make up a human baby need to fold, move, fuse and disappear at exactly the right time. If one structure persists too long or appears too early, it may block the way for another structure migrating to a new position. If a structure duplicates or fails to fuse with its other half, it can end up forming an extra organ.</p>
<p>What is most remarkable about the process of embryonic development is that in the great majority of cases we produce perfectly formed children.</p>
<figure class="align-left zoomable">
<a href="https://images.theconversation.com/files/172592/original/file-20170607-3662-11urvjg.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=1000&fit=clip"><img alt="" src="https://images.theconversation.com/files/172592/original/file-20170607-3662-11urvjg.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=237&fit=clip" srcset="https://images.theconversation.com/files/172592/original/file-20170607-3662-11urvjg.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=600&h=388&fit=crop&dpr=1 600w, https://images.theconversation.com/files/172592/original/file-20170607-3662-11urvjg.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=600&h=388&fit=crop&dpr=2 1200w, https://images.theconversation.com/files/172592/original/file-20170607-3662-11urvjg.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=600&h=388&fit=crop&dpr=3 1800w, https://images.theconversation.com/files/172592/original/file-20170607-3662-11urvjg.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=754&h=488&fit=crop&dpr=1 754w, https://images.theconversation.com/files/172592/original/file-20170607-3662-11urvjg.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=754&h=488&fit=crop&dpr=2 1508w, https://images.theconversation.com/files/172592/original/file-20170607-3662-11urvjg.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=754&h=488&fit=crop&dpr=3 2262w" sizes="(min-width: 1466px) 754px, (max-width: 599px) 100vw, (min-width: 600px) 600px, 237px"></a>
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<span class="caption">This child developed extra toes as a result of exposure to the drug thalidomide in the womb.</span>
<span class="attribution"><a class="source" href="https://www.flickr.com/photos/22719239@N04/2241322031/">Otis Historical Archives, National Museum of Health and Medicine/flickr</a>, <a class="license" href="http://creativecommons.org/licenses/by/4.0/">CC BY</a></span>
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<p>In many cases of supernumeracy we don’t know what disrupted the development of the embryo, although in some cases a mother’s exposure to certain drugs or chemicals during pregnancy may be the cause.</p>
<p>One of the best known examples is <a href="https://theconversation.com/remind-me-again-what-is-thalidomide-and-how-did-it-cause-so-much-harm-46847">thalidomide</a>, a drug prescribed to pregnant women in the 1950s and 1960s to treat morning sickness but caused some 10,000 children worldwide to be born with significant birth defects.</p>
<p>Although absent or short limbs were among the most common birth defects reported, <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4737249/">some babies had extra toes</a>.</p>
<p>The drug was commonly taken in the first trimester of pregnancy when morning sickness is more common and when, coincidentally, the embryo develops most rapidly.</p>
<h2>Why supernumeracy matters today</h2>
<p>The historical cases highlighted earlier are all examples of extreme supernumeracy. But most cases of supernumeracy are so inconspicuous they are found by chance and have little impact on people’s lives.</p>
<figure class="align-right zoomable">
<a href="https://images.theconversation.com/files/172593/original/file-20170607-3677-blegdr.JPG?ixlib=rb-1.1.0&q=45&auto=format&w=1000&fit=clip"><img alt="" src="https://images.theconversation.com/files/172593/original/file-20170607-3677-blegdr.JPG?ixlib=rb-1.1.0&q=45&auto=format&w=237&fit=clip" srcset="https://images.theconversation.com/files/172593/original/file-20170607-3677-blegdr.JPG?ixlib=rb-1.1.0&q=45&auto=format&w=600&h=600&fit=crop&dpr=1 600w, https://images.theconversation.com/files/172593/original/file-20170607-3677-blegdr.JPG?ixlib=rb-1.1.0&q=30&auto=format&w=600&h=600&fit=crop&dpr=2 1200w, https://images.theconversation.com/files/172593/original/file-20170607-3677-blegdr.JPG?ixlib=rb-1.1.0&q=15&auto=format&w=600&h=600&fit=crop&dpr=3 1800w, https://images.theconversation.com/files/172593/original/file-20170607-3677-blegdr.JPG?ixlib=rb-1.1.0&q=45&auto=format&w=754&h=754&fit=crop&dpr=1 754w, https://images.theconversation.com/files/172593/original/file-20170607-3677-blegdr.JPG?ixlib=rb-1.1.0&q=30&auto=format&w=754&h=754&fit=crop&dpr=2 1508w, https://images.theconversation.com/files/172593/original/file-20170607-3677-blegdr.JPG?ixlib=rb-1.1.0&q=15&auto=format&w=754&h=754&fit=crop&dpr=3 2262w" sizes="(min-width: 1466px) 754px, (max-width: 599px) 100vw, (min-width: 600px) 600px, 237px"></a>
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<span class="caption">Many people have extra teeth, like this 25-year-old man.</span>
<span class="attribution"><a class="source" href="https://radiopaedia.org/cases/supernumerary-tooth-2">Dr Dalia Ibrahim/Radiopaedia.org, rID: 46513</a></span>
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<p>In fact, most of the supernumerary organs we see in cadavers in the anatomy laboratory belong to body donors who were unaware of them during their lifetime.</p>
<p>Some supernumerary structures are very rare, including additional <a href="https://www.ncbi.nlm.nih.gov/pubmed/11450783">kidneys</a>, <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3446048/">penises</a> or <a href="https://www.ncbi.nlm.nih.gov/pubmed/17465296">vaginas</a>. Others are relatively common, including extra <a href="https://www.ncbi.nlm.nih.gov/pubmed/9809822">nipples</a> and <a href="https://www.hindawi.com/archive/2014/764050/">teeth</a>. </p>
<p>Understanding these less obvious cases can be very important when diagnosing and treating patients. That’s why medical students learn about them.</p>
<p>One of the supernumerate structures taught in medical school is the cervical rib, an extra rib at the base of the neck above the normal rib cage, which occurs in about one in 200 people. </p>
<p>While not apparent without diagnostic imaging, it can compress nerves and blood vessels that pass between the neck and shoulder, leading to <a href="https://www.ncbi.nlm.nih.gov/pubmed/24316293">numbness and pain</a> in the arms and fingers. So, every medical student is taught about this anatomical variation.</p>
<p>Being aware of supernumerate structures can help doctors make a correct diagnosis.
For example, about one in 2,000 of us have an extra ureter – a muscular tube that carries urine from the kidneys to the bladder, where it is stored until ready to be excreted through the urethra. While an extra ureter does not necessarily cause problems, in some people the ureter connects the kidney <a href="http://www.sciencedirect.com/science/article/pii/S0140673662919736?via%3Dihub">with the wrong structure</a>, for example with the vagina or urethra.</p>
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<a href="https://images.theconversation.com/files/172594/original/file-20170607-3710-y5aw5n.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=1000&fit=clip"><img alt="" src="https://images.theconversation.com/files/172594/original/file-20170607-3710-y5aw5n.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=237&fit=clip" srcset="https://images.theconversation.com/files/172594/original/file-20170607-3710-y5aw5n.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=600&h=657&fit=crop&dpr=1 600w, https://images.theconversation.com/files/172594/original/file-20170607-3710-y5aw5n.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=600&h=657&fit=crop&dpr=2 1200w, https://images.theconversation.com/files/172594/original/file-20170607-3710-y5aw5n.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=600&h=657&fit=crop&dpr=3 1800w, https://images.theconversation.com/files/172594/original/file-20170607-3710-y5aw5n.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=754&h=826&fit=crop&dpr=1 754w, https://images.theconversation.com/files/172594/original/file-20170607-3710-y5aw5n.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=754&h=826&fit=crop&dpr=2 1508w, https://images.theconversation.com/files/172594/original/file-20170607-3710-y5aw5n.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=754&h=826&fit=crop&dpr=3 2262w" sizes="(min-width: 1466px) 754px, (max-width: 599px) 100vw, (min-width: 600px) 600px, 237px"></a>
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<span class="caption">Extra nipples are another common example of supernumeracy.</span>
<span class="attribution"><a class="source" href="https://commons.wikimedia.org/wiki/File:Trzeci_sutek.jpg">Zureks/Wikimedia Commons</a>, <a class="license" href="http://creativecommons.org/licenses/by-sa/4.0/">CC BY-SA</a></span>
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<p>This means the ureter bypasses the normal mechanism that stops urine from leaking out of the bladder. This anomaly is usually noticed in childhood, as patients who have this type of ureter often have continuous dripping of urine which needs to be surgically corrected.</p>
<p>Doctors also need to be aware that for patients with supernumerary organs, these organs need to be included in cancer screening. </p>
<p>For example, if a patient has supernumerary breasts, these breasts need to be included when screening for lumps or in mammograms. </p>
<p>Even the smallest supernumerary structure may alter some body functions, as it did for a 29-year-old optometry student who in a practical class accidentally discovered she had an <a href="https://www.ncbi.nlm.nih.gov/pubmed/25943682">extra opening in her eyelid</a> known as a punctum. </p>
<p>The punctum helps drain away excessive tears. Usually, we only have one in each eyelid but this student had two in the same eyelid.</p>
<p>This explained why anaesthetic eye drops used for optometry procedures were far less effective in that eye, as the drops drained away twice as fast.</p>
<p>Knowing that she had a supernumerary punctum, doctors told her to press down on it to close off the opening when she needed to use eye drops.</p>
<p>So, while many of us may be aware of supernumerary structures because of the extraordinary examples usually related to parasitic twins, the more subtle supernumerary structures, which are far more common, have an important place in the study of anatomy and medical practice.</p><img src="https://counter.theconversation.com/content/76523/count.gif" alt="The Conversation" width="1" height="1" />
<p class="fine-print"><em><span>Michelle Moscova does not work for, consult, own shares in or receive funding from any company or organisation that would benefit from this article, and has disclosed no relevant affiliations beyond their academic appointment.</span></em></p>Most people don’t know if they have a hidden extra organ. But they’re surprisingly common and often harmless.Michelle Moscova, Senior Lecturer in Anatomy, UNSW SydneyLicensed as Creative Commons – attribution, no derivatives.tag:theconversation.com,2011:article/705302017-02-09T01:06:03Z2017-02-09T01:06:03ZUnusual conditions: delusional infestation with insects or spiders<figure><img src="https://images.theconversation.com/files/153185/original/image-20170118-21148-1id1qv9.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=496&fit=clip" /><figcaption><span class="caption">What causes some people to become convinced their skin or bodies are infested with crawling critters?</span> <span class="attribution"><a class="source" href="https://www.shutterstock.com/download/confirm/545509525?size=medium_jpg">from www.shutterstock.com</a></span></figcaption></figure><p>Insects often scare or disgust us. But a small proportion of people don’t just experience normal fear. They live with the terrifying and unshakeable belief that insects have invaded their body despite medical evidence suggesting otherwise. This is called a delusion of infestation, or delusional infestation.</p>
<p>Delusions of infestation feature in popular films like <a href="http://www.imdb.com/title/tt0470705/">Bug</a> and <a href="http://www.imdb.com/title/tt0405296/">A Scanner Darkly</a>. These detail the extreme emotional distress, preoccupation and conviction with which the characters believe insects have infested them.</p>
<figure>
<iframe width="440" height="260" src="https://www.youtube.com/embed/ZZbG6RUoHu4?wmode=transparent&start=0" frameborder="0" allowfullscreen=""></iframe>
<figcaption><span class="caption">The 2006 horror movie Bug depicts insects that “live in your blood and feed on your brain”</span></figcaption>
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<p>Although delusional infestations of insects are most commonly reported, some people report infestations of parasites, larvae, worms, fibres and even small animals.</p>
<p>Many people believe these symptoms are a side-effect of substance use, where they are known as “coke bugs” or “meth mites”. Government <a href="https://www.youtube.com/watch?v=qSXrTrhx6Us">public health messages</a> about the dangers of “ice” promote this view.</p>
<p>But delusions of infestation can occur in the absence of other conditions (known as the primary form and given the name delusional disorder, somatic subtype) or secondary to a range of other conditions such as schizophrenia, mood disorders, dementia and medical ailments. </p>
<p>People with the primary disorder do not have other delusional ideas or thought disorder like people with schizophrenia. If they experience hallucinations (seeing, hearing or feeling things others can’t) then these are <a href="http://psycnet.apa.org/psycinfo/1996-23895-001">only related</a> to their belief of infestation, for instance seeing bugs on their skin.</p>
<p>There’s little research into delusional infestation, so it is difficult to estimate how common it is. Also, the limited detail provided in published case studies means we are not confident the diagnoses <a href="http://journals.sagepub.com/doi/abs/10.1177/1039856216641307">are always correct</a>. </p>
<h2>Why do people develop these delusions?</h2>
<p>Prominent entomologist Jeffrey Lockwood <a href="https://global.oup.com/academic/product/the-infested-mind-9780199930197?cc=au&lang=en&">says</a> a normal disgust or fear of insects has an evolutionary basis. This is because insects can harm our health and spread disease; so, being frightened of insects is to our advantage and helps us survive. </p>
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<a href="https://images.theconversation.com/files/152978/original/image-20170117-9052-sj95f3.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=1000&fit=clip"><img alt="" src="https://images.theconversation.com/files/152978/original/image-20170117-9052-sj95f3.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=237&fit=clip" srcset="https://images.theconversation.com/files/152978/original/image-20170117-9052-sj95f3.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=600&h=912&fit=crop&dpr=1 600w, https://images.theconversation.com/files/152978/original/image-20170117-9052-sj95f3.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=600&h=912&fit=crop&dpr=2 1200w, https://images.theconversation.com/files/152978/original/image-20170117-9052-sj95f3.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=600&h=912&fit=crop&dpr=3 1800w, https://images.theconversation.com/files/152978/original/image-20170117-9052-sj95f3.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=754&h=1146&fit=crop&dpr=1 754w, https://images.theconversation.com/files/152978/original/image-20170117-9052-sj95f3.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=754&h=1146&fit=crop&dpr=2 1508w, https://images.theconversation.com/files/152978/original/image-20170117-9052-sj95f3.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=754&h=1146&fit=crop&dpr=3 2262w" sizes="(min-width: 1466px) 754px, (max-width: 599px) 100vw, (min-width: 600px) 600px, 237px"></a>
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<span class="caption">The Infested Mind describes our complicated relationship with insects.</span>
<span class="attribution"><a class="source" href="https://global.oup.com/academic/product/the-infested-mind-9780199930197?cc=au&lang=en&">Oxford University Press</a></span>
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<p>Lockwood has also argued we find insects threatening because they have minds of their own, reproduce quickly, move unpredictably and can live on and in us.</p>
<p>But with delusional infestations, additional factors are at play. Researchers <a href="https://www.ncbi.nlm.nih.gov/pubmed/1555406">suggest</a> the <a href="https://www.ncbi.nlm.nih.gov/pubmed/17671278">involvement of</a> dopamine activity (a neurochemical released by the brain with a range of functions), pre-existing skin conditions or skin sensitivity, specific brain regions and psychological factors.</p>
<p>One <a href="https://www.ncbi.nlm.nih.gov/pubmed/17671278">psychological approach</a> proposes a “two hit” model. This model suggests an itch or tactile hallucination is followed by the belief insects caused it. </p>
<p>This belief is caused by cognitive biases such as “<a href="https://www.ncbi.nlm.nih.gov/pubmed/11838027">jumping to conclusions</a>” and occurs when people form a belief with little evidence. This cognitive bias is also common in the development of delusions in other psychotic disorders. </p>
<h2>Where do people go for help?</h2>
<p>People with delusions of infestation rarely seek help from mental health services. Instead, they visit pharmacists, general practitioners, dermatologists, emergency departments and <a href="http://bjp.rcpsych.org/content/early/2014/11/30/bjp.bp.114.144469">vets</a>. They sometimes also approach non-medical professionals, like <a href="https://www.techletter.com/Archive/ER%20articles/DPclassicdop.html">pest exterminators</a> and removalists.</p>
<p>People will often <a href="https://www.ncbi.nlm.nih.gov/pubmed/26475644">bring evidence</a> of their infestation for testing. These are called “matchbox signs” or “specimen signs” and are usually particles of dust or skin rather than insects.</p>
<p>People often have a series of investigations and procedures to test for infestation. When no evidence is found, they may then be referred to psychiatrists or psychologists. But they often refuse this referral.</p>
<p>Some people take matters <a href="https://www.ncbi.nlm.nih.gov/pubmed/26475644">into their own hands</a> to “remove” or “scratch out” insects, often needing medical attention.</p>
<h2>How is it treated?</h2>
<p>Psychiatrists tend to prescribe antipsychotic medications to treat the condition. Antipsychotic medications are used to treat a variety of psychotic disorders, including schizophrenia, and aim to reduce the strength of the delusion and associated distress. For a time, doctors recommended the antipsychotic <a href="http://jamanetwork.com/journals/jamadermatology/article-abstract/538819">pimozide</a> but this came with significant <a href="http://bjp.rcpsych.org/content/191/3/198.long">side effects and safety issues</a>. </p>
<p>Now, doctors prescribe a range of different antipsychotics, depending on how acute the symptoms are, the individual person and other medical difficulties.</p>
<p>While <a href="https://www.ncbi.nlm.nih.gov/pubmed/17766758">some research</a> finds antipsychotic medications improve or stop symptoms in 60-100% of cases, some report less success. As there have been no randomised-controlled trials, which would provide stronger evidence, we still don’t know how effective these drugs really are.</p>
<p>Of the case studies that have been published, researchers don’t always report how long patients stayed on their medications, if they had side-effects, and if they remained symptom free over time. Very few also said whether people’s quality of life improved or if they returned to their previous level of functioning after taking medication.</p>
<h2>What about psychological treatments?</h2>
<p>Published research rarely refers to psychological treatments for delusional infestations. This could be due to the nature of the disorder, as patients often refuse to accept the problem may be psychological, so refuse psychological treatment.</p>
<p>But interventions such as <a href="https://theconversation.com/explainer-what-is-cognitive-behaviour-therapy-37351">cognitive behavioural therapy</a> plus medication has been a popular and effective treatment for other <a href="http://au.wiley.com/WileyCDA/WileyTitle/productCd-047195618X.html">psychotic disorders</a>.</p>
<p>Cognitive behavioural therapy addresses thoughts and behaviours. The aim is to reduce the level of conviction people have about their delusions, their preoccupation with the delusion and the distress and change to functioning that can result from the delusion. This may prove a promising area for future research and intervention with delusions of infestation.</p><img src="https://counter.theconversation.com/content/70530/count.gif" alt="The Conversation" width="1" height="1" />
<p class="fine-print"><em><span>Jessica O'Connell works for Orygen Youth Health. This is a service for young people experiencing mental health difficulties including psychotic disorders. </span></em></p><p class="fine-print"><em><span>Henry James Jackson currently receives research funding from NH&MRC. In the past he has received research funding from NH&MRC, ARC, Ian Potter Foundation, Australian Rotary Health Foundation, Victorian Health Promotion Foundation, Beyond Blue, University of Melbourne and Janssen-Cilag. In the past he has received book royalties from Cambridge University Press and McGraw-Hill. </span></em></p>Some people have terrifying delusions they are infested with insects, spiders or even small animals. But help is available.Jessica O'Connell, Psychologist, The University of MelbourneHenry James Jackson, Professor Emeritus of Clinical Psychology, The University of MelbourneLicensed as Creative Commons – attribution, no derivatives.tag:theconversation.com,2011:article/667612016-10-18T19:10:08Z2016-10-18T19:10:08ZUnusual conditions: what is Rapunzel syndrome and why do some people eat hair?<figure><img src="https://images.theconversation.com/files/141955/original/image-20161017-4757-qcn2ue.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=496&fit=clip" /><figcaption><span class="caption">Most patients with Rapunzel syndrome present to doctors with abdominal pain, nausea and vomiting.</span> <span class="attribution"><a class="source" href="https://www.flickr.com/photos/9012854@N05/4658793643/in/photolist-86Fx3k-GHg689-GHg6QS-pzJaZ9-GHg6hN-GHg5Xj-bTd65x-9wLeVG-9xcZM3-opqtVV-qQvtmM-ca3b9U-rtLgiY-bujjLv-pMrJnF-jBWL-9xd163-bBASAJ-9xd1hj-bR7DZ6-pNUFx7-qtubsv-47JPr-qtsGAx-p85K5s-qtmkvE-bDGTgf-qtsGpk-ndQHAq-pBuCwD-8UCPKB-cQzQyL-bTd5XK-pFYjDx-7KnVkv-onJLyB-4sd33S-bQvBKF-9bFcvp-ci1PtA-bN2DTt-nTCqbE-MCMB9-pQ28Gq-eagbQE-6aeLAN-c1M8qb-bDZpmf-c1M2dm-bYXbiy">ihave3kids/Flickr</a>, <a class="license" href="http://creativecommons.org/licenses/by/4.0/">CC BY</a></span></figcaption></figure><p>In the <a href="http://germanstories.vcu.edu/grimm/rapunzel_e.html">Brothers Grimm fairy tale</a>, the trapped Rapunzel lets down her long hair through a tower window so a prince can climb up and rescue her. </p>
<p>Named after this tale, Rapunzel syndrome is an extremely rare medical condition where hairs the person has eaten become tangled and trapped in their stomach. This causes a trichobezoar (hair ball) to form, which has a long tail extending into the small intestine. </p>
<p>Recently, a <a href="http://www.livescience.com/56403-rapunzel-syndrome.html">38-year-old woman</a> had a 15 x 10 cm hair ball surgically removed from her stomach and a 4 x 3 cm hair ball removed from the top of her small intestine. This case, published in the journal BMJ Case Reports, marks the 89th published instance of Rapunzel syndrome in medical literature.</p>
<p>Like 85 to 95% of <a href="https://www.researchgate.net/publication/51723178_Telling_the_tale_of_Rapunzel_syndrome">patients with Rapunzel syndrome</a>, the woman presented to doctors with abdominal pain, nausea and vomiting. Other symptoms of Rapunzel syndrome include a bloated stomach, reduced appetite, weight loss and constipation or diarrhoea. In some cases the bowel is punctured, which can lead to sepsis (blood infection). Death <a href="https://www.researchgate.net/publication/51723178_Telling_the_tale_of_Rapunzel_syndrome">has occurred</a> in 4% of cases. </p>
<figure class="align-right zoomable">
<a href="https://images.theconversation.com/files/141957/original/image-20161017-4757-1lkkcpn.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=1000&fit=clip"><img alt="" src="https://images.theconversation.com/files/141957/original/image-20161017-4757-1lkkcpn.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=237&fit=clip" srcset="https://images.theconversation.com/files/141957/original/image-20161017-4757-1lkkcpn.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=600&h=800&fit=crop&dpr=1 600w, https://images.theconversation.com/files/141957/original/image-20161017-4757-1lkkcpn.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=600&h=800&fit=crop&dpr=2 1200w, https://images.theconversation.com/files/141957/original/image-20161017-4757-1lkkcpn.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=600&h=800&fit=crop&dpr=3 1800w, https://images.theconversation.com/files/141957/original/image-20161017-4757-1lkkcpn.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=754&h=1005&fit=crop&dpr=1 754w, https://images.theconversation.com/files/141957/original/image-20161017-4757-1lkkcpn.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=754&h=1005&fit=crop&dpr=2 1508w, https://images.theconversation.com/files/141957/original/image-20161017-4757-1lkkcpn.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=754&h=1005&fit=crop&dpr=3 2262w" sizes="(min-width: 1466px) 754px, (max-width: 599px) 100vw, (min-width: 600px) 600px, 237px"></a>
<figcaption>
<span class="caption">In Rapunzel syndrome, a trichobezoar (hair ball) forms in the stomach.</span>
<span class="attribution"><a class="source" href="https://www.flickr.com/photos/nonox/167247299/in/photolist-fMbN8-4gvJf2-9deb77-iJcFNw-dZH3Rm-9j12nz-aEeDVh-f4atdm-Nh7mM-5JY9i8-r6a189-9W6AuM-9W9qkG-aBKXMY-9W6CGZ-aAmY1P-7MSmF2-e2vD1A-9Zcv2L-9iKgAi-dKMumL-81r3qc-oY3Yng-5nXVAm-7EwDre-9W9sHL-9xAt7P-pUMm9M-81uess-81r5d2-9W9sqh-na55vf-bMZD1D-5pB7PU-572yk5-9W9sfJ-56Xoav-4eUL6U-qn1KNR-dpoW54-7v4Df6-7zxqos-7tgswC-9W9rSQ-9W6Atg-9W6Ayt-54z1C6-9W9rvf-8sGbwd-7EwDjg">Noemie/Flickr</a>, <a class="license" href="http://creativecommons.org/licenses/by/4.0/">CC BY</a></span>
</figcaption>
</figure>
<p>Fortunately, this woman made a successful recovery. But it’s unknown why she had been eating her own (or possibly <a href="https://www.medpagetoday.com/upload/2007/12/28/242.pdf">other people’s</a>) hair in the first place, or for how long. It can take six months for a hair ball to develop and <a href="https://www.ncbi.nlm.nih.gov/pubmed/23583142">there are reports</a> of people coping with the dangerous symptoms of Rapunzel syndrome for 12 months before seeking treatment. </p>
<p>The authors of the <a href="http://casereports.bmj.com/content/2016/bcr-2016-216600.abstract">BMJ Case Reports</a> review found nearly 70% of patients with Rapunzel syndrome were females aged younger than 20. The youngest reported patients have been <a href="http://www.jcpsp.pk/archive/2014/SS_Nov2014/43.pdf">toddlers</a> while the oldest patient was a <a href="https://casesjournal.biomedcentral.com/articles/10.1186/1757-1626-1-286">55-year-old man</a>. </p>
<p>It’s thought more females than males develop Rapunzel syndrome because their hair strands are typically longer, and long hairs are more likely to get stuck in the stomach’s layers of mucous membranes. As more hair is consumed and is unable to be digested, the hair ball grows bigger. </p>
<h2>Why do people eat hair?</h2>
<p>Some people with intellectual disabilities and certain psychiatric disorders eat their own hair – a behaviour called trichophagia. These groups are thought to be at heightened risk for developing Rapunzel syndrome.</p>
<p>There are two particular psychiatric disorders that people who eat their hair are likely to have: trichotillomania and pica.</p>
<p>People with <a href="https://theconversation.com/pulling-out-your-hair-in-frustration-what-you-need-to-know-about-trichotillomania-45228">trichotillomania</a> feel compelled to pull out their hair, often to the point of visible hair loss. It’s very common for people to then play with the removed hair strands. For example, nibbling the root of the hair or mouthing the hair along the lips can feel relaxing. </p>
<p>One <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2605948/">study found 20% of people</a> with trichotillomania engaged in these behaviours on a daily basis, including actually swallowing the hair. Another <a href="https://www.ncbi.nlm.nih.gov/pubmed/1748781">study found</a> of 24 people with trichotillomania, 25% had developed a hair ball in the stomach because of eating the hair. </p>
<p>Pica comes from the Latin word for “magpie”, because of the bird’s unusual eating habits. The disorder <a href="http://www.eatingdisorders.org.au/eating-disorders/what-is-an-eating-disorder/classifying-eating-disorders/dsm-5#pica">involves craving and eating</a> non-nutritious, non-food substances such as clay, dirt, paper, soap, cloth, wool, pebbles and hair. </p>
<figure class="align-left ">
<img alt="" src="https://images.theconversation.com/files/142085/original/image-20161017-12443-1xq4bd4.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=237&fit=clip" srcset="https://images.theconversation.com/files/142085/original/image-20161017-12443-1xq4bd4.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=600&h=601&fit=crop&dpr=1 600w, https://images.theconversation.com/files/142085/original/image-20161017-12443-1xq4bd4.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=600&h=601&fit=crop&dpr=2 1200w, https://images.theconversation.com/files/142085/original/image-20161017-12443-1xq4bd4.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=600&h=601&fit=crop&dpr=3 1800w, https://images.theconversation.com/files/142085/original/image-20161017-12443-1xq4bd4.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=754&h=755&fit=crop&dpr=1 754w, https://images.theconversation.com/files/142085/original/image-20161017-12443-1xq4bd4.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=754&h=755&fit=crop&dpr=2 1508w, https://images.theconversation.com/files/142085/original/image-20161017-12443-1xq4bd4.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=754&h=755&fit=crop&dpr=3 2262w" sizes="(min-width: 1466px) 754px, (max-width: 599px) 100vw, (min-width: 600px) 600px, 237px">
<figcaption>
<span class="caption">It’s thought more females than males develop Rapunzel syndrome because their hair strands are typically longer.</span>
<span class="attribution"><a class="source" href="https://www.flickr.com/photos/25697729@N08/14311388534/in/photolist-nNDBdb-2uztBV-fQHCrh-6dU8f-duQWzr-6AhsQz-4mKe7w-4mRbaD-6AmAD3-4mRaBk-9gq9bG-8sjKPv-5Sf6fY-2uzoPt-4QdtdX-2uzjDi-2uzyjt-5iYKMD-2jtnNR-78fYWe-fZtP23-72AnKs-goWGYX-51X82p-4mVcFG-6Amz6d-baUTar-nRrXPM-wrU8e-dupA9h-oye7FJ-fyWRk-fheekT-2uzrcX-2jtjtT-4FWQjt-hHepBd-crtY8C-2jxJ2A-nzW5Sp-8KiPkF-aqttBG-7hMTu2-c7JbtE-m4t2Xj-8KmRPN-a7iPeJ-e2GVBY-oye3GG-cKrpr1">hairfreaky long hair/Flickr</a>, <a class="license" href="http://creativecommons.org/licenses/by/4.0/">CC BY</a></span>
</figcaption>
</figure>
<p>Pica generally isn’t diagnosed in infants or toddlers because mouthing (and accidentally ingesting) non-food substances is considered pretty normal at this age. It is most common in children, <a href="https://www.ncbi.nlm.nih.gov/pubmed/26892693">pregnant women</a>, and in people with <a href="http://www.sciencedirect.com/science/article/pii/S1750946711000390">intellectual disabilities</a> such as autism spectrum disorder. </p>
<p>There have been <a href="http://www.annualreviews.org/doi/full/10.1146/annurev.nutr.012809.104713_">many theories to explain</a> trichophagia and pica, such as hunger during famine or childhood neglect, as a way of coping with stress, and a part of <a href="http://medind.nic.in/daa/t14/i1/daat14i1p144.pdf">cultural practices</a>. For instance, in some regions of India, Africa and the <a href="https://pdfs.semanticscholar.org/f4dc/52c08b4ae33112475989f733b9321b20165e.pdf">United States</a>, eating clay is considered to have positive health or spiritual benefits. </p>
<p>Both trichophagia and pica have been found to occur in people with iron deficiency. In some case reports of Rapunzel syndrome, hair pulling and hair eating stopped after the person was treated for <a href="http://www.jpeds.com/article/S0022-3476(80)80429-X/abstract">iron deficiency</a> or <a href="https://www.ncbi.nlm.nih.gov/pubmed/24574811">coeliac disease</a>. </p>
<p>Coeliac disease causes damage to the small intestine, which leads to poor nutrient absorption. Hair does contain trace elements of iron and other minerals, but it’s still unclear if this promotes some kind of biological drive to eat hair. <a href="http://www.sciedupress.com/journal/index.php/crim/article/view/8808/5519">Other case studies</a> have found the blockage caused by the hair ball was actually the root cause of the iron deficiency. </p>
<h2>What are the treatments?</h2>
<p>In most cases, surgery is required to remove the hair ball in one piece. It’s also possible to dissolve the hair ball with chemicals, break it up into smaller pieces with a laser or remove it via a tube fed through the mouth and into the stomach, called an <a href="https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/endoscopy">endoscopy</a>. However, these methods are generally less successful than surgery.</p>
<p>Psychological treatment is recommended to prevent future compulsive hair eating. This is especially important for patients with trichotillomania or stress-related pica because they may be at risk of <a href="http://www.sciencedirect.com/science/article/pii/S0163834313000303">developing Rapunzel syndrome again</a>.</p>
<p>Involving parents and spouses in psychological treatment is important so they can learn to support their loved one to stop the behaviour, and also because the impact of Rapunzel syndrome can be upsetting for them too.</p>
<p><em>Information and support for trichotillomania and trichophagia can be found through the <a href="http://www.bfrb.org">TLC Foundation for Body-Focused Repetitive Behaviors</a>.</em></p><img src="https://counter.theconversation.com/content/66761/count.gif" alt="The Conversation" width="1" height="1" />
<p class="fine-print"><em><span>Imogen Rehm does not work for, consult, own shares in or receive funding from any company or organisation that would benefit from this article, and has disclosed no relevant affiliations beyond their academic appointment.</span></em></p>Rapunzel syndrome is an extremely rare medical condition where hairs the person has eaten become tangled and trapped in their stomach.Imogen Rehm, Research Psychologist, Swinburne University of TechnologyLicensed as Creative Commons – attribution, no derivatives.tag:theconversation.com,2011:article/640002016-09-16T02:55:22Z2016-09-16T02:55:22ZUnusual conditions: what are gigantism and acromegaly?<figure><img src="https://images.theconversation.com/files/136683/original/image-20160906-21919-11k2sb7.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=496&fit=clip" /><figcaption><span class="caption">Robert Wadlow is known as the tallest man who ever lived.</span> <span class="attribution"><a class="source" href="https://www.flickr.com/photos/paille-fr/24559019804">Paille/Flickr</a>, <a class="license" href="http://creativecommons.org/licenses/by/4.0/">CC BY</a></span></figcaption></figure><p>Gigantism is embedded in the folklore of many civilisations – think of the giant Goliath who fought with the legendary King David in the Bible, for instance.</p>
<p>But it’s not all the stuff of legends. Present-day famous “giants” include André René Roussimoff, known as Andre the Giant (224cm), and Richard Kiel (218cm), otherwise known as Jaws in two James Bond movies. </p>
<p>Another real-life giant was Robert Wadlow, also known as the Alton giant. He was <a href="http://www.sciencealert.com/it-s-the-birthday-of-the-tallest-man-who-ever-lived">the tallest man ever</a>, standing at just over 272cm. Robert died in 1940 at the age of 22 after his foot became infected.</p>
<figure>
<iframe width="440" height="260" src="https://www.youtube.com/embed/TLNLXJSdaj0?wmode=transparent&start=0" frameborder="0" allowfullscreen=""></iframe>
<figcaption><span class="caption">Actor Richard Kiel played ‘Jaws’ in two James Bond films.</span></figcaption>
</figure>
<p>Gigantism and its counterpart, acromegaly, <a href="http://www.mayoclinic.org/diseases-conditions/acromegaly/home/ovc-20177622">are rare conditions</a> caused by excessive production of growth hormone (GH), which stimulates the growth of the skeleton and all the tissues in the body.</p>
<p>Gigantism develops when a child experiences accelerated growth due to excessive GH production, which eventually leads to their tall stature. Acromegaly is what the condition is called when excessive GH production develops in an adult after he or she has reached their final height. It comes from the Greek words akron (extremity) and megas (big). </p>
<p>Continued stimulation of tissue growth causes large hands and feet, nose, jaw and forehead, which are the most noticeable features. Acromegaly affects <a href="http://www.ncbi.nlm.nih.gov/pubmed/11081170">around 60 people per million</a>. There are <a href="https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/acromegaly">an estimated 1,200 people</a> living with the condition in Australia.</p>
<h2>What causes it?</h2>
<p>Growth hormone is produced by the pituitary gland, at the base of the brain. The gland produces several hormones that control growth, metabolism, development, and reproduction.</p>
<figure class="align-right zoomable">
<a href="https://images.theconversation.com/files/136391/original/image-20160902-20213-mi7mkd.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=1000&fit=clip"><img alt="" src="https://images.theconversation.com/files/136391/original/image-20160902-20213-mi7mkd.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=237&fit=clip" srcset="https://images.theconversation.com/files/136391/original/image-20160902-20213-mi7mkd.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=600&h=720&fit=crop&dpr=1 600w, https://images.theconversation.com/files/136391/original/image-20160902-20213-mi7mkd.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=600&h=720&fit=crop&dpr=2 1200w, https://images.theconversation.com/files/136391/original/image-20160902-20213-mi7mkd.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=600&h=720&fit=crop&dpr=3 1800w, https://images.theconversation.com/files/136391/original/image-20160902-20213-mi7mkd.jpg?ixlib=rb-1.1.0&q=45&auto=format&w=754&h=905&fit=crop&dpr=1 754w, https://images.theconversation.com/files/136391/original/image-20160902-20213-mi7mkd.jpg?ixlib=rb-1.1.0&q=30&auto=format&w=754&h=905&fit=crop&dpr=2 1508w, https://images.theconversation.com/files/136391/original/image-20160902-20213-mi7mkd.jpg?ixlib=rb-1.1.0&q=15&auto=format&w=754&h=905&fit=crop&dpr=3 2262w" sizes="(min-width: 1466px) 754px, (max-width: 599px) 100vw, (min-width: 600px) 600px, 237px"></a>
<figcaption>
<span class="caption">The pituitary gland is located at the base of the brain.</span>
<span class="attribution"><span class="source">from shutterstock.com</span></span>
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<p>A <a href="http://www.mayoclinic.org/diseases-conditions/pituitary-tumors/symptoms-causes/dxc-20157631">tumour in the pituitary gland</a> can secrete uncontrolled amounts of any of its hormones. Pituitary tumours are benign, can vary in size and develop at any time through life. </p>
<p>Their effects on health <a href="http://www.mayoclinic.org/diseases-conditions/pituitary-tumors/symptoms-causes/dxc-20157631">depend on the hormone type and amount secreted</a>. In the case of acromegaly, too much GH is produced. If the tumour is large, it may cause headaches and can compress the nerves to the eyes, impairing vision.</p>
<p>The cause of tumour formation is not well understood, <a href="http://www.ncbi.nlm.nih.gov/pubmed/25470569">but evidence points</a> to gene mutations that control cell growth and hormone secretion.</p>
<h2>Symptoms</h2>
<p>In contrast to the marked tall stature that characterises the childhood onset of the disease, manifestations in adults are different.</p>
<p>Symptoms of acromegaly develop gradually from slow growth of soft tissue, organs and bones. It can take more than ten years from when the first symptoms develop to diagnosis. Patients may be unaware of the progressive enlargement of their hands and feet, <a href="http://www.sciencedirect.com/science/book/9780123809261">although specific questioning usually elicits</a> a history of ring- and shoe-size increase.</p>
<p>Daytime sleeping, fatigue and poor concentration are common from sleep disruption caused by an enlarged tongue that blocks breathing during sleep. The <a href="http://www.sciencedirect.com/science/book/9780123809261">enlarged tongue</a> also causes heavy snoring, drooling, and difficulties with speech and chewing. </p>
<p>Carpal tunnel syndrome can also develop due to compression of a major nerve tissue growing at the base of the hand. The tumour may also be large enough to cause visual field loss from pressure on the nerves to the eyes.</p>
<p>People with gigantism and acromegaly are <a href="http://www.ncbi.nlm.nih.gov/pubmed/14764779">two to three times more at risk</a> of premature death than the general population. But life expectancy is restored when the disease is adequately treated.</p>
<h2>Diagnosis and treatment</h2>
<p>Acromegaly and gigantism are diagnosed with a blood test that measures concentration of GH and another insulin-like growth hormone called IGF-I. It is produced mainly by the liver due to the stimulation of GH. </p>
<figure class="align-center ">
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<figcaption>
<span class="caption">Andre the Giant had gigantism as a child which developed into acromegaly in adulthood.</span>
<span class="attribution"><a class="source" href="https://www.flickr.com/photos/noticeofmeowery/86288815/in/photolist-8CfEc-6uxxfF-fdtVSz-93hZ24-9BsNNa-drM7md-co7y65-6Sd7kJ-qUSNep-qUSNwt-qCqyGg-abAQGb-e2P89d-pY6nvZ-aLp8W6-9JutQm-qCqztr-aqqsX3-7QVjSS-5pKHQb-ihL6p-7GyC8Y-nQu7pA-fQRN5E-7apFBv-atpSgn-9Xcpdc-7ASEhe-ravhLB-cEgQay-e2PnVs-6f3rE3-9V56hx-4x2Ttk-e2P82W-azv7nC-qCin1d-avHxGs-bcZDHk-bqh3Pd-nNvouJ-bA3ZpQ-4RfpSP-5re9Qi-qSzQk5-4x72Gw-2wL27-4x72v7-4x72Vy-nQfP8K">John McKeon/Flickr</a>, <a class="license" href="http://creativecommons.org/licenses/by/4.0/">CC BY</a></span>
</figcaption>
</figure>
<p>Both conditions <a href="http://www.ncbi.nlm.nih.gov/pubmed/19208732">can be treated</a> by surgery, radiotherapy or medication. No single treatment is effective in every patient. </p>
<p>Surgically removing the tumour is done by approaching the pituitary gland through the nose. </p>
<p>Radiotherapy is used where it’s impossible to remove the tumour completely. The radiation kills off the remaining tumour but this occurs slowly and can take up to ten years. The radiation can also harm the neighbouring normal pituitary gland and lead to progressive loss of pituitary function. </p>
<p>Medications are used for those for whom surgery has been unsuccessful or not suitable, or as an interim treatment while waiting for radiotherapy to take effect.</p>
<p>Drugs called somatostatin analogues (SSAs), such as octreotide, lanreotide and <a href="http://www.ncbi.nlm.nih.gov/pubmed/24423324">pasireotide</a>, <a href="http://www.ncbi.nlm.nih.gov/pubmed/2404445">have an established place</a> in the treatment of acromegaly. They work by inhibiting the secretion of GH. </p>
<p>Drugs developed for rare diseases are not cheap. The cost of pasireotide, which is dosage-dependent, is <a href="http://www.health.gov.au/internet/ministers/publishing.nsf/Content/health-mediarel-yr2016-ley1408.htm">up to A$50,000 per year</a>. But as of the beginning of September, the government has listed Signifor on the Pharmaceutical Benefits Scheme.</p>
<p>Acromegaly has negative physical and psychological effects, including appearance changes. These are <a href="http://www.ncbi.nlm.nih.gov/pubmed/17555502">preventable and reversible</a> as long as the condition is diagnosed early and patients have access to effective treatments.</p><img src="https://counter.theconversation.com/content/64000/count.gif" alt="The Conversation" width="1" height="1" />
<p class="fine-print"><em><span>Ken Ho consults for Pfizer, manufacturer of pegvisomant, and receives speaker fees from Novartis, manufacturer of Signifor. </span></em></p>Gigantism and acromegaly are rare conditions where a benign tumour causes excessive production of growth hormone (GH), which stimulates the growth of the skeleton and all tissues in the body.Ken Ho, Professor, The University of QueenslandLicensed as Creative Commons – attribution, no derivatives.