Weakness or paralysis, numbness, tingling, and muscle pain are other signs of porphyria. Hereditary coproporphyria, which is characterized by a deficiency in coproporphyrinogen oxidase, coded for by the CPOX gene, may also present with both acute neurologic attacks and cutaneous lesions. Periodically drawing blood (phlebotomy) to reduce the iron in your body, which decreases porphyrins. 2. The links between porphyrias and mental illness have been noted for decades. The king Georges III, the lady with the tattoos, Sean Albright - American racecar driver has Variegate Porphyria (VP). [2] Genetic testing may be done to determine the specific mutation. Smoking. General acute porphyria is more common in women and cutaneous porphyria is more common in men. [31] In nearly all cases of acute porphyria syndromes, urinary PBG is markedly elevated except for the very rare ALA dehydratase deficiency or in patients with symptoms due to hereditary tyrosinemia type I. Depression is part and parcel of the Acute Porphyrias. Thanks for reading Scientific American. Finally, and most important, the fact that vampire reports were literally rampant in the 18th century, and that congenital erythropoietic porphyria is an extremely rare manifestation of a rare disease, makes it an unlikely explanation of the folkloric vampire.[58]. "It all began with a horrible pain in my right leg from the muscle downward," she remembered. In September 2019, she was appointed as the first female . Charlotte of Mecklenburg-Strelitz PreviousNext The fifteen children of George III, Copyright 2004 - 2022 www.englishmonarchs.co.uk All rights reserved All rights reserved Privacy Policy, Copyright 2004 - 2022 www.englishmonarchs.co.uk All rights reserved All rights reserved . [2], The precise prevalence of porphyria is unclear, but it is estimated to affect between 1 and 100 per 50,000 people. The most commonly seen painting of Vlad the Impaler is a copy that hangs in the gallery of horrors at Innsbrucks Ambras Castle. Maria I of Portugal in a c. 1790s portrait attributed to Giuseppe Troni or Thomas Hickey. Russell Brand is a British comedian, actor, and activist. These include hemochromatosis and hepatitis C. Treatment of iron overload may be required. Hot baths and showers may lessen nausea temporarily, though caution should be used to avoid burns or falls. People who have inherited porphyria from one or both parents have deficiencies in certain enzymes that help with normal hemoglobin functions. He was buried alive after an accident left him in a deep coma that doctors couldn't differentiate from death. Only 6 cases of ALAD-deficiency porphyria (ADP) are documented. Fewer than 300 cases have been reported in the medical literature of Congenital Eyrthropoeitic Porphyria. Porphyria cutanea tarda affects people around the world and isn't limited to a particular region or country. In 1889, Dr. B.J. Heme, a component of the oxygen transporter hemoglobin, is made in a sequence of eight steps, as in a factory assembly line. Erythropoietic protoporphyria (EPP), the most common kind of porphyria to occur in childhood, causes people's skin to become very sensitive to light. Porphyria is an inherited blood disorder that causes the body to produce less heme a critical component of hemoglobin, the protein in red blood cells that carries oxygen from the lungs to the. [2] Complications may include paralysis, low blood sodium levels, and seizures. He had since noticed that his skin was prone to a blistering rash, particularly on exposure to sunshine. She is a Professor of Medical Education in the University of Manchester and former Hospital Dean in Salford Royal. Erythropoietic . Heme is made of body chemicals called. Whoopi Goldberg treated for fear of flying using TFT on The View, part 1. Published by University Books, Inc., 1959. avg. Recent medical tests performed on the remains of Charlotte and Feodora, who committed suicide at the age of 66 in 1945, have revealed that both probably suffered from porphyria. Porphyria had tormented Mary Queen of Scots, who passed it on to her son, King James I of England. HISTORY reviews and updates its content regularly to ensure it is complete and accurate. 5 In people with protoporphyria, bile carries extra porphyrins from the liver to the gallbladder, which may lead to gallstones that are made of porphyrins. On exposure to sunlight the leaves blister, and eventually wither and die. Vampires' famed sun-aversion is likely connected to the symptoms of cutaneous porphyrias such as PCT. Nina Simone. These symptoms occur in 12 months or less after exposure. Sufferers shun bright light because it irritates their skin and hurts their eyes. Continue reading with a Scientific American subscription. At Christmas, 1819, George suffered another violent attack of porphyria, after talking incessantly for fifty-eight hours he sank into a coma. [citation needed], The theory has been rejected by a few folklorists and researchers as not accurately describing the characteristics of the original werewolf and vampire legends or the disease, and as potentially stigmatizing people with porphyria.[56][57]. These heme-like substances theoretically inhibit ALA synthase and hence the accumulation of toxic precursors. The blockage of the assembly line also means that the body cannot make enough heme to produce normal red blood cells. Porphyria is an inherited disease caused by an abnormal hemoglobin, the chemical that carries oxygen in red blood cells. It is actually believed that the legend of the vampire was created around this specific disorder. None of these are associated with acute attacks; their primary manifestation is with skin disease. First, they overcome the bodys shortage of heme, relieving anemia. 6 Acute porphyria, whether symptomatic or not, carries an increased risk of hypertension, hepatocellular carcinoma, and chronic renal failure. Oscar Wilde (June 1954) 3. However, oral contraceptives have also triggered photosensitivity and withdrawal of oral contraceptives has triggered attacks. She went into labour on 3rd November 1817, the birth proved to be difficult and protracted, Caroline's ordeal lasted for fifty hours. These are associated with accumulation of porphyrins in erythrocytes and are rare. [citation needed], There are eight enzymes in the heme biosynthetic pathway, four of whichthe first one and the last threeare in the mitochondria, while the other four are in the cytosol. Two types of subcutaneous porphyria erythropoietic protoporphyria and x-linked protoporphyriaare a bit different and do not cause blisters at all. Vlad Had The Habsburg Lip. 891,100 views made by Traveler. People with porphyria should, therefore, avoid medications as much as possible, including recreational drugs such as cannabis. Make a Gift. 1. 5. [2][4] If the skin is affected, blisters or itching may occur with sunlight exposure. Explore our digital archive back to 1845, including articles by more than 150 Nobel Prize winners. Erythropoietic Protoporphyria (EPP). [1] Porphyrias may also be classified by whether the liver or bone marrow is affected. Porphyria cutanea tarda (PCT) is a type of porphyria or blood disorder that affects the skin. [1] The disease was described as early as 370 BC by Hippocrates. Exposed to light, the porphyrins can turn caustic and destroy surrounding tissue. These porphyrins accumulate in the skin and other organs before being excreted in feces and urine (which may turn a port-wine color). Gender. Public Domain George III ruled Britain for more than half a century and presided over the Seven Years' War and the American. [2], Most types of porphyria are inherited from one or both of a person's parents and are due to a mutation in one of the genes that make heme. 3. After 2 months when my mother cut my nails, there was some water. See "New Light on Medicine," by Nick Lane; Scientific American, January 2003.). Porphyria is an incurable genetic disease, the symptoms of which align perfectly with those ascribed to vampires. Porphyrins are photoactive molecules, and exposure to light results in promotion of electrons to higher energy levels. However, even if a mutated gene is inherited, it doesn't guarantee that someone will experience symptoms (this is called latent porphyria). Some psychotropic drugs are porphyrinogenic, limiting the therapeutic scope. [47] A bone marrow transplant has also been successful in curing CEP in a few cases, although long-term results are not yet available. However, this can worsen cases of low blood sodium levels (hyponatraemia) and should be done with extreme caution as it can prove fatal. Porphyria is a group of rare diseases that affect the heme production in our blood. Porphyria cutanea tarda (PCT) is the most common type of porphyria and can be acquired, which means that other factors than the inherited gene can cause this, says the NIH. National Organization for Rare Disorders (NORD) 1900 Crown Colony Drive Suite 310 Quincy, MA 02169 Phone: 617-249-7300 Other Locations: Danbury, CT office 55 Kenosia Avenue Danbury, CT 06810 Phone: 203-263-9938 Fax: 203-263-9938 Washington, DC Office 1779 Massachusetts Avenue Suite 500 Washington, DC 20036 Phone: 202-588-5700 AHP is a hereditary disease, meaning that it . Download Porphyria stock photos. George III's recurring bouts of illness resulted in withdrawal from society to recuperate out of the public eye at Kew Palace, near Richmond. 55 Fruit Street. While the accumulation of porphyrins is usually caused by a genetic mutation, toxins (such as alcohol excess) and environmental contaminants can also cause the disease. Hodder Arnold. In some cases, paraplegia can be improved by extensive physical therapy and stem cell treatments. Some people might also experience chest, leg, or back pain. This accounts for the property of fluorescence typical of the porphyrins. This poem published in the year of 1836. William Blake, Friedrich Nietzsche, Ludwig Von Beethoven, Edgar Allen Poe, Vincent Van Gogh, and Ernest Hemingway all reported going through similar cycles of mania and depression. They are not curative drugs but can shorten attacks and reduce the intensity of an attack. This causes local skin damage. The exact prevalence of porphyria is unknown, but it likely ranges from 1 in 500 to 1 in 50,000 people worldwide. Thankfully, there was another man standing behind Churchill: his physician Lord Moran. [27], The body requires porphyrins to produce heme, which is used to carry oxygen in the blood among other things, but in the porphyrias there is a deficiency (inherited or acquired) of the enzymes that transform the various porphyrins into others, leading to abnormally high levels of one or more of these substances. [39], Patients who experience frequent attacks can develop chronic neuropathic pain in extremities as well as chronic pain in the abdomen. 2023 Scientific American, a Division of Springer Nature America, Inc. The abnormal porphyrins: deposit in skin, where they cause a terrible itchy rash on exposure to sunlight For example, all fox squirrels (Sciurus niger) have a gene defect that gives them a form of CEP, yet they do not suffer any adverse consequences, for unknown reasons. There are high incidence reports of AIP in areas of India and Scandinavia. According to one historian, James suffered from itchy skin, gout and abdominal pain. Luckily, most people with AIP have a latent form, and never develop any symptoms. This is a rare disorder that mainly affects the skin. It could be a decision that makes life a whole lot easier. The products of the earlier steps, porphyrin intermediates, may build up to toxic levels. Daytime hours are generally best. Interestingly, the heme pigment is robust enough to survive digestion, and is absorbed from the intestine (even though the protein parts of hemoglobin are broken down). Its sometimes referred to colloquially as vampire disease. When heme binds with. In 1816, the author of such beloved novels as Emma and Pride and Prejudice came down with a host of mysterious ailments including exhaustion, back pain, skin discoloration, bilious attacks and fever. People who are under extreme emotional or physical stress may also suffer from porphyria attacks. As a result of feedback, the decreased production of heme leads to increased production of precursors, PBG being one of the first substances in the porphyrin synthesis pathway. Murray ED, Buttner N, Price BH. Children have a 50% chance of inheriting any of the 8 different types of Porphyria if their parents have the disorder. The Great Emancipator managed to lead the country through one . Her daughter, Princess Feodora of Saxe-Meiningen (born 19 May 1879) is also claimed to have suffered from the disease. Estrogen use by women. Much speculation has been devoted to Jane Austens sickness in the years since, with the most common diagnosis being that she suffered from Addisons disease, a malfunction of the adrenal glands that wasnt known to medicine until several years after her death. Some drugs are absolutely contraindicated for patients with any form of porphyria. For other uses, see. Among other descendants of George III claimed to have suffered from porphyria were his granddaughter, Princess Charlotte Augusta of Wales, the only child of George IV and his first cousin, Caroline of Brunswick. AIP is caused by low levels of porphobilinogen deaminase (PBGD), an enzyme also often called hydroxymethylbilane synthase. The treatment apparently cures the disease over a period of years. [citation needed] Porphyrias are a group of rare inherited blood disorders. 2. Porphyrins and porphyrin precursors and related substances originate in excess amounts predominantly from the liver in the hepatic types and mostly . George III's 2 greats granddaughter, Princess Victoria, Queen Victoria's eldest daughter, and her daughter Princess Charlotte of Prussia, (born 24 July 1860), was further suspected of suffering from the condition. Pricing level. About 1 in 75,000 people have acute porphyria attacks. Bipolar disorder is also difficult for a manager to handle. It's thought Bram Stoker named Count Dracula after Vlad Dracul, also known as Vlad the Impaler. The Romans called the disease by various names including morbus caducus (the falling sickness), and it was widely believed at the time that its victims were touched by the gods. I am currently continuing at SunAgri as an R&D engineer. Martin A Crook.2006. Individuals with congenital erythropoietic porphyria do not crave blood. It seems likely that this disorder is the origin of the vampire myth. [2], Hormonal fluctuations that contribute to cyclical attacks in women have been treated with oral contraceptives and luteinizing hormones to shut down menstrual cycles. Famous British People. Episodes of . These eight celebrities and famous historical figures have all lived with bipolar disorder. Porphyria's Lover And My Last Duchess Comparison. The most notorious environmental episode happened in Turkey in the 1950s, when 4,000 people developed a form of porphyria after eating wheat seeds that had been sprayed with a fungicide, hexachlorobenzene. 15. Felicity helped organise the first BPA Awareness Day in Salford in 2005 and was made a patron in 2012. [2] Treatment of porphyria of the skin generally involves the avoidance of sunlight, while treatment for acute porphyria may involve giving intravenous heme or a glucose solution. Treatment options are generally to reduce symptoms. "New Light on Medicine," by Nick Lane; Scientific American, January 2003. [1] Diagnosis is typically made by blood, urine, and stool tests. "An Encyclopaedia of Obscure Medicine". The royal porphyria is porphyria variegata, which has an incidence in the general population of 1 in 100,000. The hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems, usually a light-sensitive blistering rash and increased hair growth.Variegate porphyria (also porphyria variegata or mixed porphyria), which results from a partial deficiency in PROTO oxidase, manifests itself with skin lesions similar to those of porphyria cutanea tarda combined with acute neurologic attacks. They may either have one of the acute porphyrias or they may have a mixed porphyria. It's also called the Vampire disease. Other future treatments for porphyria will depend on the results obtained from research with experimental animal--and even plant--models. Acute porphyrias The most common of these is acute intermittent porphyria. 12 April 2012. Drawing blood (phlebotomy) can also help, because this quickly removes porphyrin intermediates from the circulation. Porphyria is a disorder that occurs when there is a build-up of the chemicals in the body that produce a substance called porphyrin. 2. Porphyria cutanea tarda is the most common type of porphyria worldwide. [4] Attacks may be triggered by alcohol, smoking, hormonal changes, fasting, stress, or certain medications. Find out which celebrities, athletes or public figures have Porphyria. "Porphyria's Lover " is one of the famous poems by Browning that gains the attention of the critics and this has become critically issued. In principle, CEP can be cured by bone-marrow transplantation, which replaces the faulty stem cells with fully functional ones. Bone-marrow transplants have been carried out successfully in at least five children with CEP, usually within the first few years of life. He was reported to have had lucid moments when he agonized pitifully about what he had become. Population Estimate: Fewer than 1,000 people in the U.S. have this disease. Other typical risk factors for liver cancer need not be present. Porphyria has been suggested as an explanation for the origin of vampire and werewolf legends, based upon certain perceived similarities between the condition and the folklore. Graham Greene. When these return to the resting energy level or ground state, energy is released. Bradley WG, Daroff RB, Fenichel GM, Jankovic J (eds.) Androgens and fertility hormones have also triggered attacks. Fewer than 300 cases have been reported in the medical literature of Congenital Eyrthropoeitic Porphyria. This diagnosis is pervasive throughout the scientific and popular literature, and is often referred to as the 'Royal Malady.' . Some of these abnormal red cells rupture, leading to hemolytic anemia, while the spleen detects abnormalities in other red cells and breaks them down, making matters worse. In general, testing involves sending samples of blood, stool, and urine to a reference laboratory. (2012) Depression and Psychosis in Neurological Practice. [28], Deficiency in the enzymes of the porphyrin pathway leads to insufficient production of heme. This rare neurological condition often results in weakness of the facial muscle and temporary paralysis of the face. Some people may have porphyria, but have it be in remission until lifestyle circumstances change. 3 to 4 mg/kg of Panhematin given once daily for four days early in an attack produces a highly beneficial effect in most patients. It affects about 1 in 25,000 people in the UK. He made various sounds with his mouth, his friend and biographer James Boswell wrote, sometimes giving a half whistle, sometimes making his tongue play backwards, from the roof of his mouth, as if clucking like a hen. Johnson was also known to exhibit compulsive behaviors such as counting his steps and touching every light post he passed on the street. He was subjected to the appalling medical treatment of the day, bound and gagged and strapped into a chair for hours. Donate to the Mass General Porphyria Center and help our multidisciplinary team provide comprehensive care and the latest therapies to patients with all types of porphyria. He often . Meanwhile, a 2005 analysis of hair samples found the even if King George did suffer from porphyria, his symptoms were likely exacerbated by arsenic poisoning from his doctor-prescribed medicines. He experienced regular vomiting and temporary paralysis in addition to widespread nerve pain. As such he also descended from King George III. 10. Vlad Dracul was born in Transylvania, Romania. [1][2][4] The attacks usually last for days to weeks. I love to write and share science related Stuff Here on my Website. Pain should be treated as early as medically possible. In fact, in the majority of cases, people who test positively . Skin disease is encountered where excess porphyrins accumulate in the skin. William was later examined by haematologists at Addenbrookes hospital in Cambridge and also by Professor Ishihara in Tokyo, both of whom also concluded he was suffering from variegate porphyria, by then in remission. DNA tests carried out on Princess Charlotte's remains revealed a mutation that affected the gene that codes for protoporphyrinogen oxidase (which controls the transition from step 5 to step 6 in haem production). When experiencing a porphyria attack, it is not uncommon for their to be eyesight blurring or other changes that occur because of a dysfunction in the nervous system. My thesis aimed to study dynamic agrivoltaic systems, in my case in arboriculture. [1] The underlying mechanism results in a decrease in the amount of heme produced and a build-up of substances involved in making heme. What are the types of porphyria? For most of his life, the sixteenth president was plagued by a feeling of gloom and despair that he dubbed the hypo. He often spoke of suicide as a young man, and once told an acquaintance that he never carried a pocketknife out of fear of hurting himself. These mutations obstruct heme synthesis to varying degrees, giving a spectrum of severity. Bill Gates (1955 - ) American businessman, founder of Microsoft. Physiologically, porphyrias are classified as liver or erythropoietic based on the sites of accumulation of heme precursors, either in the liver or in the bone marrow and red blood cells. There are also cases of porphyria that have been known to be triggered by environmental factors. It is due to a deficiency in the enzyme protoporphyrinogen oxidase (PPOX). The principal problem in these deficiencies is the accumulation of porphyrins, the heme precursors, which are toxic to tissue in high concentrations. Generally they do not have symptoms, but may pass the gene onto offspring. [citation needed], The rarest is congenital erythropoietic porphyria (CEP), otherwise known as Gunther's disease. Symptoms include burning blisters and swelling of the skin when exposed to the sun, along with severe. Mother Teresa (1910 - 1997) Macedonian Catholic missionary nun. . Thisa reference to the characteristic, Descendants of George III. Signs and symptoms. Extended licenses. Michael Phelps-ADHD. A number of psychologists claim that Smith exhibited many of the symptoms of histrionic personality disorder. Many people with bipolar disorder find they do best in a quiet, relaxed workspace where they can easily concentrate. This is very useful for diagnosis. Jaundice, anemia, enlarged liver and spleen, often neonatal. Plants make chlorophyll via a pathway very similar to that for heme production in animals. James' physician kept detailed notes on his royal patient, which describe his urine as being 'purple as Alicante wine' - a sign of porphyria. How does civil disobedience relate to society today? 2. Later, in the Byzantine Empire, the term porphyrogenitos, or "born to the purple," literally meant that the imperial heir was born after the fathers accession to the throne, in a palace room draped in the color. Basic biochemical tests may assist in identifying liver disease, hepatocellular carcinoma, and other organ problems. They exhumed his body days after he was buried, and the doctors found that he was alive. Demi Lovato. She later developed a pain in her chest, before going into convulsions. People demanded those methods because extravagant cases like the one about Angelo Hays's gained fame. George Clooney, actor and star of movies like "Up in the Air" and the "Ocean's Eleven" series was diagnosed with Bell's palsy when he was a 14. Although some types can be cured, most people who receive a diagnosis of this disorder will be managing it for the rest of their lives. A careful medication review often will find the cause of pseudoporphyria. Studies of the animals could yield clues that would be useful in fighting CEP. Donald Trump (1946 - ) Businessman, US President. [citation needed], It is recommended that patients with a history of acute porphyria, and even genetic carriers, wear an alert bracelet or other identification at all times. Symptoms of acute porphyrias can develop over hours or days and last for days or weeks. Avoiding alcohol, smoking, and having excess iron are all beneficial. From Michelangelos arthritis to Abraham Lincolns depression, learn the stories behind seven historical figures who may have suffered from undiagnosed physical ailments. George III ruled Britain for more than half a century and presided over the Seven Years War and the American Revolution, but throughout his life he also suffered recurring bouts of insanity that left him incapacitated and occasionally confined to a straitjacket. There are seven different types of porphyria and in most cases, they are inherited. It's estimated that 1 out of 10,000 to 25,000 people has this condition. Graduated from ENSAT (national agronomic school of Toulouse) in plant sciences in 2018, I pursued a CIFRE doctorate under contract with SunAgri and INRAE in Avignon between 2019 and 2022. Alcohol consumption and cigarette smoking (nicotine use) should be limited. Porphyrin is necessary for hemoglobin to function properly. There are several different types of porphyria that are known and all vary in severity. These risk factors include: High alcohol consumption Smoking Viral infection (hepatitis C or HIV) Estrogen replacement Iron build up in the body Risk Factors of Porphyria [citation needed] Porphyria cutanea tarda is the disease that both Dabney Pratt and Brother Rush suffer from in Virginia Hamilton 's children's novel Sweet Whispers, Brother Rush. 4 However, more recent studies have suggested that acute crises are rarely fatal. Hippocrates is often cited as the first to recognize porphyria (which was then referred to as blood/liver disease) but the causal role of porphyrin pigments was only established in 1871 by the great German pioneer of biochemistry Felix Hoppe-Seyer. King George IIIPorphyria George III ruled Britain for more than half a century and presided over the Seven Years War and the American Revolution, but throughout his life he also suffered recurring bouts of insanity that left him incapacitated and occasionally confined to a straitjacket. [citation needed], Any sign of low blood sodium (hyponatremia) or weakness should be treated with the addition of hematin, heme arginate, or even tin mesoporphyrin, as these are signs of impending syndrome of inappropriate antidiuretic hormone (SIADH) or peripheral nervous system involvement that may be localized or severe, progressing to bulbar paresis and respiratory paralysis. But if you see something that doesn't look right, click here to contact us! 1. [35], Hematin (trade name Panhematin) and heme arginate (trade name NormoSang) are the drugs of choice in acute porphyria, in the United States and the United Kingdom, respectively. Acute attacks happen in all acute porphyrias. Frederick the Great of Prussia (1712 - 1786) a grandson of George I, is believed to have suffered from an acute form of porphyria, like his relative George III. It causes blisters in sun-exposed skin areas, excessive scarring, increased hair growth when the skin is exposed to sunlight, and even liver damage. Stokvis described the clinical syndrome as "porphyria," and from then on more and more forms of the syndrome were discovered. Pain treatment with long-acting opioids, such as morphine, is often indicated, and, in cases where seizure or neuropathy is present, gabapentin is known to improve outcome. The addition of hematin or heme arginate has been used during status epilepticus. 4 Adolf Hitler Adolf Hitler (April 20, 1889 - April 30, 1945) was a German politician who was the leader of the Nazi Party, Chancellor of Germany from 1933 to 1945, and Fhrer of Nazi Germany from 1934 to 1945. The ailment likely stemmed from an incident during her childhood on a Maryland plantation, when an overseer threw a lead weight at another slave and instead hit Tubman on the head, fracturing her skull. Thanks for reading Scientific American. Porphyrias are generally classified into two groups: the hepatic and erythropoietic types. She chaired the British and Irish Porphyria Network from 2008 to 2014. [43], Some liver diseases may cause porphyria even in the absence of genetic predisposition. Mutations in the gene for the final step in this pathway lead to a buildup of porphyrins in the leaves. ", The Drug Database for Acute Porphyria - comprehensive database on drug porphyrinogenicity, https://en.wikipedia.org/w/index.php?title=Porphyria&oldid=1140390972. Though Charlotte seemed at first to be recovering well from her horrendous ordeal, she complained that evening of severe stomach pains and began to vomit. Throughout his life, acquaintances noted that the writer exhibited the kinds of involuntary verbal and physical tics associated with the neurological disorder including repetitively shaking his head and rubbing his left knee with the palm of his hand.