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Sickle - Wikipedia
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1History of sickle
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1.1Pre-Neolithic
1.2Neolithic
1.3Bronze Age
1.4Iron Age
1.5Americas
2Nepal
3Serrated ''Simple'' or "toothed" sickles
4Use
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4.1As a weapon
4.2Other uses
5Bagging hook
6See also
7References
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Sickle
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From Wikipedia, the free encyclopedia
Single-handed agricultural tool
For other uses, see Sickle (disambiguation).
SickleNepalese sickle from PanchkhalOther namesBagging hook, reaping-hookClassificationCuttingRelatedScythe
One of 12 roundels depicting the "Labours of the Months" (1450-1475)
A sickle, bagging hook, reaping-hook or grasshook is a single-handed agricultural tool designed with variously curved blades and typically used for harvesting or reaping grain crops, or cutting succulent forage chiefly for feeding livestock. Falx was a synonym but was later used to mean any of a number of tools that had a curved blade that was sharp on the inside edge such as a scythe.
Since the beginning of the Iron Age hundreds of region-specific variants of the sickle have evolved, initially of iron and later steel. This great diversity of sickle types across many cultures can be divided into smooth or serrated blades, both of which can be used for cutting either green grass or mature cereals using slightly different techniques. The serrated blade that originated in prehistoric sickles still dominates in the reaping of grain and is even found in modern grain-harvesting machines and in some kitchen knives.[citation needed]
History of sickle[edit]
Pre-Neolithic[edit]
A very early sickle, c. 7000 BC, flint and resin, Tahunian culture, Nahal Hemar cave, now in the Israel Museum.
The development of the sickle in Mesopotamia can be traced back to times that pre-date the Neolithic Era. Large quantities of sickle blades have been excavated in sites surrounding Israel that have been dated to the Epipaleolithic era (18000-8000 BC).[1] Formal digs in Wadi Ziqlab, Jordan have unearthed various forms of early sickle blades. The artifacts recovered ranged from 10–20 cm (3.9–7.9 in) in length and possessed a jagged edge.
This intricate ‘tooth-like’ design showed a greater degree of design and manufacturing credence than most of the other artifacts that were discovered. Sickle blades found during this time were made of flint, straight and used in more of a sawing motion than with the more modern curved design. Flints from these sickles have been discovered near Mt. Carmel, which suggest the harvesting of grains from the area about 10,000 years ago.[2]
Neolithic[edit]
Neolithic sickle
The sickle had a profound impact on the Agricultural Revolution by assisting in the transition to farming and crop based lifestyle. It is now accepted that the use of sickles led directly to the domestication of Near Eastern Wild grasses.[1] Research on domestication rates of wild cereals under primitive cultivation found that the use of the sickle in harvesting was critical to the people of early Mesopotamia. The relatively narrow growing season in the area and the critical role of grain in the late Neolithic Era promoted a larger investment in the design and manufacture of sickle over other tools. Standardization to an extent was done on the measurements of the sickle so that replacement or repair could be more immediate. It was important that the grain be harvested at the appropriate time at one elevation so that the next elevation could be reaped at the proper time.[2] The sickle provided a more efficient option in collecting the grain and significantly sped up the developments of early agriculture.[3]
Bronze Age[edit]
The sickle remained common in the Bronze Age, both in the Ancient Near East and in Europe. Numerous sickles have been found deposited in hoards in the context of the European Urnfield culture (e.g. Frankleben hoard), suggesting a symbolic or religious significance attached to the artifact.
In archaeological terminology, Bronze Age sickles are classified by the method of attaching the handle. E.g. the knob-sickle (German Knopfsichel) is so called because of a protruding knob at the base of the blade which apparently served to stabilize the attachment of the blade to the handle.[4]
Iron Age[edit]
Ancient Greek iron sickle, Kerameikos Archaeological Museum, Athens.
The sickle played a prominent role in the Druids' Ritual of oak and mistletoe as described from a single passage in Pliny the Elder's Natural History:
A priest arrayed in white vestments climbs the tree and, with a golden sickle, cuts down the mistletoe, which is caught in a white cloak. Then finally they kill the victims, praying to a god to render his gift propitious to those on whom he has bestowed it. They believe that mistletoe given in drink will impart fertility to any animal that is barren and that it is an antidote to all poisons.[5]
Due to this passage, despite the fact that Pliny does not indicate the source on which he based this account, some branches of modern Druidry (Neodruids) have adopted the sickle as a ritual tool.[citation needed]
Americas[edit]
Congolese sickle, or Trumbash, (left) and replica throwing knife (right) at Manchester Museum
Indigenous sickles have been discovered in southwest North America with unique design. There is evidence that Kodiak islanders had for cutting grass "sickles made of a sharpened animal shoulder blade".[6] The artifacts found in present-day Arizona and New Mexico resemble curved tools that were made from the horns of mountain sheep. A similar site discovered sickles made from other material such as the Caddo Sickle, which was made from a deer mandible. Scripture from early natives document the use of these sickles in the cutting of grass. The instruments ranged 13–16 in (330–410 mm) from tip to haft. Several other digs in eastern Arizona uncovered wooden sickles that were shaped in a similar fashion. The handles of the tools help describe how the tool was held in such a way so that the inner portion that contained the cutting surface could also serve as a gathering surface for the grain. Sickles were sharpened by scraping a shape beveled edge with a coarse tool. This action has left marks on artifacts that have been found. The sharpening process was necessary to keep the cutting edge from being dulled after extended use. The edge is seen to be quite highly polished, which in part proves that the instrument was used to cut grass. After collection, the grass was used as material to create matting and bedding.[6] The sickle in general provided the convenience of cutting the grass as well as gathering in one step. In modern times, the sickle is being used in South America as tool to harvest rice. Rice clusters are harvested using the instrument and left to dry in the sun.[7]
Nepal[edit]
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Called Hasiya (or Aasi), a sickle is very common in Nepal as the most important tool for cutting used in the kitchen and in the fields.[8] Hasiya is used in the kitchen in many villages of Nepal where its used to cut vegetables during food prep. The handle of Hasiya (made of wood) is held pressed by the toe of one's foot and the curve inverted so vegetables can be cut with two hands while rocking the vegetable. Outside of home, Hasiya is used for harvesting.
Hasiya have traditionally been made by local blacksmiths in their charcoal foundries that use leather bellows to blow air. Sharpening of the Hasiya is done by rubbing the edges against a smooth rock or taken back to the blacksmith. Sharpening of the Hasiya is generally done during the beginning of the harvesting season.
Bigger Hasiya is called Khurpa (or Khoorpa) where the curve is less pronounced, is much heavier and is used to sever branches of trees with leaves (for animal feed), chop meat etc. The famous Nepali Khukuri is also a type of sickle where the curve becomes least visible.
Carrying around a sharp and naked Hasiya or Khurpa is unsafe. So Nepalis have traditionally built a cover/holder for it called "Khurpeto" (meaning Khurpa holder in Nepali). It could be a simple piece of wood with a hole big enough to slide the blade of Hasiya inside or could be an intricately carved piece of round wood slung around one's waist with a string made of plants (called "hatteuri"). Nowadays though many use cotton, jute or even cloth strings as a replacement of hatteuri which is not easy to find.
A Village lady holding 'Hasiya' (Sickle) in Saptari, Nepal
Sumerian harvesting sickle, circa 3000BC
Serrated ''Simple'' or "toothed" sickles[edit]
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The genealogy of sickles with serrated edge reaches back to the Stone Age, when individual pieces of flint were first attached to a "blade body" of wood or bone. (The majority among the well-documented specimens made later of bronze are smooth-edged.) Nevertheless, teeth have been cut with hand-held chisels into iron, and later steel-bladed sickles for a long time. In many countries on the African continent, Central and South America as well as the Near, Middle and Far East this is still the case in the regions within these large geographies where the traditional village blacksmith remains alive and well.
England appears to have been the first to develop the industrial process of serration-making. Then, by 1897, the Redtenbacher Company of Scharnstein, in Austria—at that time the largest scythe maker in the world—designed its own machine for the job, becoming the only Austrian source of serrated sickles. In 1942, its recently acquired sister company Krenhof also began to produce these. In 1970, a year before the sickle production branch of Redtenbacher was sold to Ethiopia, they were still making 1.5 million of the serrated sickles per year, predominately for market in Africa and Latin America. There were other enterprises in Austria, of course, who produced the smoothed-edged sickles for centuries. The last of the classical "round" versions were forged until the mid-1980s and machined until 2002.
While in Central Europe the smooth-edged sickle—either forged or machined (alternately referred to as "stamped") - has been the only one used (and in many regions the only one known), the Iberian Peninsula, Sicily and Greece long had fans of both camps. The many small family-owned enterprises in what is now Italy, Portugal and Spain produced sickles in both versions, with the teeth on the serrated models being hand-cut, one at a time, until the mid-20th century.
The Falci Co. of Italy (established in 1921 as a union of several formerly independent forges) developed its own unique method of industrial scale serrated sickle production in 1965. Their innovations, which included tapered blade cross section (thicker at the back - for strength - gradually thinning towards the edge - for ease of penetration) were later adopted by Europe's largest sickle producer in Spain as well as, more recently, a company in India.
Nepali sickle (hasiya) with its carrier (khurpeto)
Use[edit]
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Modern harvesting sickle
The inside of the blade's curve is sharp, so that the user can either draw or swing it against the base of the crop, catching the stems in the curve and slicing them at the same time. The material to be cut may be held in a bunch in the other hand (for example when reaping), held in place by a wooden stick, or left free. When held in a bunch, the sickle action is typically towards the user (left to right for a right-handed user), but when used free the sickle is usually swung the opposite way. Other colloquial/regional names for principally the same tool are: grasshook, swap hook, rip-hook, slash-hook, reaping hook, brishing hook or bagging hook.
A serrated sickle was used for harvesting wheat, the ears being held bunched up in the free hand as described above. After this the straw was cut with a scythe. Oats and barley on the other hand were simply scythed. The reason for this is that wheat straw, unlike that of oats or barley, whose softer straw was suitable only for bedding or fodder, was a valuable crop, used for thatching, and subjecting it to the battering of a flail would have rendered it useless for this purpose.
The blades of sickle models intended primarily for the cutting of grass are sometimes "cranked", meaning they are off-set downwards from the handle, which makes it easier to keep the blade closer to the ground. Sickles used for reaping do not benefit by this feature because cereals are usually not cut as close to the ground surface. Instead, what distinguishes this latter group is their often (though not always) serrated edges.
A blade which is used regularly to cut the silica-rich stems of cereal crops acquires a characteristic sickle-gloss, or wear pattern.
As a weapon[edit]
Like other farming tools, the sickle can be used as an improvised bladed weapon. Examples include the Japanese kusarigama and kama, the Chinese chicken sickles, and the makraka of the Zande people of north central Africa. Paulus Hector Mair, the author of a German Renaissance combat manual also has a chapter about fighting with sickles. It is particularly prevalent in the martial arts of Malaysia, Indonesia and the Philippines. In Indonesia, the native sickle known as celurit or clurit is commonly associated with the Madurese people, used for both fighting and as a domestic tool.
Other uses[edit]
The hammer and sickle is a symbol representing proletarian solidarity, a union between the peasantry and the urban working class. It was first adapted during the Russian Revolution, the hammer representing the industrial workers, and the sickle representing the farmers/peasants.
The emblem of the Grim Reaper, who is sometimes portrayed as carrying a sickle rather than the more traditional scythe.
Tacitus reports that golden sickles were used in Druidic rituals.
Paulus Hector Mair's Manuscript Dresd. C 93 includes a section regarding the martial application of the sickle.
Three (or two) entwined sickles were the heraldic badge of the medieval Hungerford family. See also Hungerford knot.
Bagging hook[edit]
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A bagging hook, badging hook, fagging hook, reap hook or rip hook, is a large sickle usually with an offset handle so that the user's knuckles do not make contact with the ground. The Oxford dictionary gives the definition of the word to bag, or badge, as the cutting of grain by hand. The blade is heavier than that of a normal sickle and always without serrated blades. It is usually about 40 mm (1.6 in) wide with an open crescent shaped blade approx 45 cm (18 in) across. It developed from the sickle in most parts of Britain during the mid to late 19th century, and was in turn replaced by the scythe, later by the reaping machine and subsequently the swather. It was still used when the corn was bent over or flattened and the mechanical reaper was unable to cut without causing the grain to fall from the ears and wasting the crop.
It was also used in lieu of the bean hook or pea hook for cutting field beans and other leguminous crops that were used for fodder and bedding for livestock.
Sometimes confused with the heavier and straighter billhook used for cutting wood or laying hedges. While the scythe or bagging hook blade was heavy enough to remove young growth instead of, say, shears for clipping a hedge, it was not strong enough to cut woody material for which the stronger, similarly shaped, but longer handled, staff hook was used. Many variations in blade shape were used in different parts of England and known under a variety of names. Its close relations in shape and usage are the grass hook and the reap hook.
See also[edit]
A rooster holding sickle in the coat of arms of Laitila
Aruval, an Indian instrument similar to the billhook
Billhook, a version of the sickle used for cutting woody stems
Brush hook
Kaiser blade or sling blade
Kama
Machete
Scythe
Hammer and sickle, a symbol in communism
Harpe, a Greek or Roman long sickle or scythe
Khopesh, an Egyptian long sickle or scythe as a weapon
References[edit]
^ a b Unger-Hamilton, Romana (July 1985). "Microscopic Striations on Flint Sickle-Blades as an Indication of Plant Cultivation: Preliminary Results". World Archaeology. 17 (1): 121–6. doi:10.1080/00438243.1985.9979955.
^ a b Banning, E.B. (1998). "The Neolithic Period: Triumphs of Architecture, Agriculture, and Art". Near Eastern Archaeology. 61 (4): 188–237. doi:10.2307/3210656. JSTOR 3210656. S2CID 164006022.
^ Unger-Hamilton, Romana (1989). "The Epi-Palaeolithic Southern Levant and the Origins of Cultivation". Current Anthropology. 30 (1): 88–103. doi:10.1086/203718. S2CID 143186819.
^ Christoph Sommerfeld: Gerätegeld Sichel. Studien zur monetären Struktur bronzezeitlicher Horte im nördlichen Mitteleuropa (Vorgeschichtliche Forschungen Bd. 19), Berlin/New York 1994 ISBN 3-11-012928-0, p. 157.
^ Pliny the Elder. Natural History XVI, 95.
^ a b Heizer, Robert F. (1951). "The Sickle in Aboriginal Western North America". American Antiquity. 16 (3): 247–252. doi:10.2307/276785. JSTOR 276785. S2CID 161405622.
^ Works, Martha A. (1987). "Aguaruna Agriculture in Eastern Peru". Geographical Review. 77 (3): 343–358. doi:10.2307/214125. JSTOR 214125.
^ Bhattarai, Devendra; Singh, Suman Bahadur; Baral, Dharanidhar; Sah, Ram Bilakshan; Budhathoki, Shyam Sundar; Pokharel, Paras K. (2016-10-26). "Work-related injuries among farmers: a cross-sectional study from rural Nepal". Journal of Occupational Medicine and Toxicology. 11 (1): 48. doi:10.1186/s12995-016-0137-2. ISSN 1745-6673. PMC 5080695. PMID 27800010.
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Sickle Definition & Meaning - Merriam-Webster
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Est. 1828
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sickle
1 of 3
noun
sick·le
ˈsi-kəl
1
: an agricultural implement consisting of a curved metal blade with a short handle fitted on a tang
2
: the cutting mechanism (as of a reaper, combine, or mower) consisting of a bar with a series of cutting elements
Illustration of sickle
sickle 1
sickle
2 of 3
adjective
: having the form of a sickle blade : having a curve similar to that of a sickle blade
the sickle moon
sickle
3 of 3
verb
sickled; sickling
ˈsi-k(ə-)liŋ
transitive verb
1
: to mow or reap with a sickle
2
: to change (a red blood cell) into a sickle cell
intransitive verb
: to change into a sickle cell
the ability of red blood cells to sickle
Examples of sickle in a Sentence
Recent Examples on the WebNoun
Abnormal hemoglobin alters the shape of red blood cells, turning them from discs to sickles.
—Emily Mullin, WIRED, 19 Dec. 2023
Padlocks, sickles and other objects made of iron, a metal said to possess anti-demonic powers, were stashed in the grave as preventives.
—Franz Lidz, New York Times, 5 Sep. 2023
Popular weeding tools include claw weeders, sickles, hoes, rakes, and digging forks.
—Andrea Wurzburger, Better Homes & Gardens, 1 Sep. 2023
On shelves were jugs of oil and vinegar, slabs of pecorino, a bag of nails, a small hand sickle, and a pot of honey.
—Stanley Stewart, Condé Nast Traveler, 18 July 2023
While there have been reports of people placing scythes or sickles near a grave as an offering to prevent demons from entering the body, the placement of this sickle was different.
—Jennifer Ouellette, Ars Technica, 6 Sep. 2022
Kasparov refused to compete under the Soviet flag — the hammer and sickle.
—Jay Nordlinger, National Review, 22 June 2023
Cala Saladeta, Ibiza, Spain On the west coast of the White Isle lies Cala Salada, a little cove backed by thick shrubbery, with a sickle of sand and rock slabs cantilevered over the water to moor boats against or sunbathe on.
—Julia Buckley, Travel + Leisure, 12 Apr. 2023
And last year, archaeologists uncovered an unusual example of people using these tips in a 17th-century Polish cemetery near Bydgoszcz: a female skeleton buried with a sickle placed across her neck, as well as a padlock on the big toe of her left foot.
—Jennifer Ouellette, Ars Technica, 17 Mar. 2023
Adjective
The announcement is a landmark in the treatment of sickle cell disease, a devastating condition in which red blood cells deform into a sickle shape and clog up blood vessels.
—Beth Mole, Ars Technica, 8 Dec. 2023
Those misshapen hemoglobin proteins stick together in people with sickle cell, distorting the shape of red blood cells from a round shape to a sickle one that cannot flow through our blood vessels as easily.
—Janice Blanchard, Scientific American, 18 Dec. 2023
In other words, sickle probes (those pointy metal things used to, yep, probe your mouth for cavities) and syringes shouldn’t just be lying out on the counter or metal tray.
—Jenna Ryu, SELF, 19 Sep. 2023
The illness causes the body’s red blood cells, usually disk-shaped, to take on a crescent or sickle shape.
—Berkeley Lovelace Jr., NBC News, 31 Oct. 2023
Thousands of rural Nepalis joined their ranks, vowing to kill millions and hoist the hammer and sickle atop Chomolungma.
—Sean Williams, Harper's Magazine, 11 Sep. 2023
The faulty gene causes blood cells to become misshapen into a characteristic crescent or sickle shape that can slow or halt blood flow, damaging tissues and leaving patients in extreme pain.
—Angelica Peebles, BostonGlobe.com, 29 Mar. 2023
In India, which has the second-highest rate of sickle-cell prevalence in the world, hydroxyurea is often difficult to access, said Gautam Dongre, secretary of India’s first national sickle cell patient advocacy group, whose two children suffer from the disease.
—Megan Molteni, STAT, 7 Mar. 2023
In the trailer, Law sports a long, frizzy gray wig and a furry mustache while wielding the villain’s signature sickle.
—Los Angeles Times, 2 Mar. 2023
Verb
In 1948, pediatrician Janet Watson noticed that children with sickle cell disease had normal blood cells as infants, but that the cells became sickled around six months.
—Emily Mullin, WIRED, 19 Dec. 2023
Exertional sickling deaths occur in athletes carrying the sickle cell trait.
—Jennifer Borresen, courier-journal.com, 18 Apr. 2023
The result is hemoglobin S—a misshapen version that causes red blood cells to sickle.
—Dhruv Khullar, The New Yorker, 22 Mar. 2022
Blood cells filled with fetal hemoglobin do not sickle.
—New York Times, 11 Jan. 2020
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These examples are programmatically compiled from various online sources to illustrate current usage of the word 'sickle.' Any opinions expressed in the examples do not represent those of Merriam-Webster or its editors. Send us feedback about these examples.
Word History
Etymology
Noun
Middle English sikel, from Old English sicol, from Latin secula sickle, from secare to cut — more at saw
First Known Use
Noun
before the 12th century, in the meaning defined at sense 1 Adjective
1688, in the meaning defined above Verb
1922, in the meaning defined at transitive sense 1
Time Traveler
The first known use of sickle was
before the 12th century
See more words from the same century
Phrases Containing sickle
sickle-cell trait
sickle cell
sickle cell anemia
hammer and sickle
Dictionary Entries Near sickle
sickish
sickle
sickle alfalfa
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Merriam-Webster
“Sickle.” Merriam-Webster.com Dictionary, Merriam-Webster, https://www.merriam-webster.com/dictionary/sickle. Accessed 12 Mar. 2024.
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Kids Definition
sickle
1 of 2
noun
sick·le
ˈsik-əl
: a tool with a sharp curved metal blade and a short handle used to cut grass
sickle
2 of 2
verb
sickled; sickling
ˈsik-(ə-)liŋ
: to change into a sickle cell
the ability of red blood cells to sickle
Medical Definition
sickle
1 of 3
noun
sick·le
ˈsik-əl
: a dental scaler with a curved 3-sided point
sickle
2 of 3
adjective
: of, relating to, or characteristic of sickle cell anemia or sickle-cell trait
sickle hemoglobin
sickle
3 of 3
verb
sickled; sickling
ˈsik-(ə-)liŋ
transitive verb
: to change (a red blood cell) into a sickle cell
intransitive verb
: to undergo change into a sickle cell
the ability of red blood cells to sickle
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Britannica.com: Encyclopedia article about sickle
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Sickle cell anemia - Symptoms & causes - Mayo Clinic
le cell anemia - Symptoms & causes - Mayo ClinicSkip to contentCare at
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Red blood cells are usually round and flexible. In sickle cell anemia, some red blood cells look like sickles used to cut wheat. These unusually shaped cells give the disease its name.
Products & Services A Book: Mayo Clinic Family Health Book, 5th Edition Newsletter: Mayo Clinic Health Letter — Digital EditionSymptomsSymptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over time. Symptoms can include:
Anemia. Sickle cells break apart easily and die. Typical red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells. This is known as anemia. Without enough red blood cells, the body can't get enough oxygen. This causes fatigue.
Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints. The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year. A severe pain crisis requires a hospital stay. Some people with sickle cell anemia also have chronic pain from bone and joint damage, ulcers, and other causes.
Swelling of hands and feet. Sickle-shaped red blood cells block blood circulation in the hands and feet, which can cause them to swell.
Frequent infections. The spleen is important for protecting against infections. Sickle cells can damage the spleen, raising the risk of developing infections. Babies and children with sickle cell anemia commonly receive vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.
Delayed growth or puberty. Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in babies and children and delay puberty in teenagers.
Vision problems. Tiny blood vessels that supply blood to the eyes can become plugged with sickle cells. This can damage the portion of the eye that processes visual images, called the retina, and lead to vision problems.
When to see a doctorSee your healthcare professional right away if you or your child has symptoms of sickle cell anemia, including fever or stroke. Infections often start with a fever and can be life-threatening. Because children with sickle cell anemia are prone to infections, seek prompt medical attention for a fever greater than 101.5 degrees Fahrenheit (38.5 degrees Celsius).Seek emergency care for symptoms of stroke, which include:
One-sided paralysis or weakness in the face, arms or legs.
Confusion.
Difficulty walking or talking.
Sudden vision changes.
Unexplained numbness.
Severe headache.
Request an appointmentFrom Mayo Clinic to your inboxSign up for free and stay up to date on research advancements, health tips, current health topics, and expertise on managing health. Click here for an email preview. Subscribe!Learn more about Mayo Clinic’s use of data.To provide you with the most relevant and helpful information, and understand which information is beneficial, we may combine your email and website usage information with other information we have about you. If you are a Mayo Clinic patient, this could include protected health information. If we combine this information with your protected health information, we will treat all of that information as protected health information and will only use or disclose that information as set forth in our notice of privacy practices. You may opt-out of email communications at any time by clicking on the unsubscribe link in the e-mail.CausesSickle cell anemia is caused by a change in the gene that tells the body to make hemoglobin. Hemoglobin is the iron-rich compound in red blood cells that allows these cells to carry oxygen from the lungs to the rest of the body. The hemoglobin associated with sickle cell anemia causes red blood cells to become rigid, sticky and misshapen.For a child to have sickle cell anemia, both parents must carry one copy of the sickle cell gene and pass both copies to the child.If only one parent passes the sickle cell gene to the child, that child will have the sickle cell trait. With one typical hemoglobin gene and one sickle cell gene, people with the sickle cell trait make both typical hemoglobin and sickle cell hemoglobin.Their blood might contain some sickle cells, but they generally don't have symptoms. They're carriers of the disease. That means they can pass the gene to their children.Risk factorsFor a baby to have sickle cell anemia, both parents must carry a sickle cell gene. In the United States, sickle cell anemia most commonly affects people of African, Mediterranean and Middle Eastern descent.ComplicationsSickle cell anemia can lead to a host of complications, including:
Stroke. Sickle cells can block blood flow to the brain. Signs of stroke include seizures, weakness or numbness of the arms and legs, sudden speech difficulties, and loss of consciousness. If your child has any of these signs or symptoms, seek medical treatment right away. A stroke can be fatal.
Acute chest syndrome. A lung infection or sickle cells blocking blood vessels in the lungs can cause this life-threatening complication. Symptoms include chest pain, fever and difficulty breathing. Acute chest syndrome might need emergency medical treatment.
Avascular necrosis. Sickle cells can block the blood vessels that supply blood to the bones. When the bones don't get enough blood, joints may narrow and bones can die. This can happen anywhere but most often happens in the hip.
Pulmonary hypertension. People with sickle cell anemia can develop high blood pressure in their lungs. This complication usually affects adults. Shortness of breath and fatigue are common symptoms of this condition, which can be fatal.
Organ damage. Sickle cells that block blood flow to organs deprive the affected organs of blood and oxygen. In sickle cell anemia, blood also is low in oxygen. This lack of oxygen-rich blood can damage nerves and organs, including the kidneys, liver and spleen, and can be fatal.
Splenic sequestration. Sickle cells can get trapped in the spleen, causing it to enlarge. This may cause abdominal pain on the left side of the body and can be life-threatening. Parents of children with sickle cell anemia can learn how to locate and feel their child's spleen for enlargement.
Blindness. Sickle cells can block tiny blood vessels that supply blood to the eyes. Over time, this can lead to blindness.
Leg ulcers. Sickle cell anemia can cause painful open sores on the legs.
Gallstones. The breakdown of red blood cells produces a substance called bilirubin. A high level of bilirubin in the body can lead to gallstones.
Priapism. Sickle cell anemia can cause painful, long-lasting erections, known as priapism. Sickle cells can block the blood vessels in the penis, which can lead to impotence over time.
Deep vein thrombosis. Sickled red blood cells can cause blood clots, increasing the risk of a clot lodging in a deep vein, known as deep vein thrombosis. It also increases the risk of a blood clot lodging in a lung, known as pulmonary embolism. Either can cause serious illness or even death.
Pregnancy complications. Sickle cell anemia can increase the risk of high blood pressure and blood clots during pregnancy. It also can increase the risk of miscarriage, premature birth and low birth weight babies.
PreventionIf you carry the sickle cell trait, it can help to see a genetic counselor before you get pregnant. A counselor can help you understand your risk of having a child with sickle cell anemia. You also can learn about possible treatments, preventive measures and reproductive options. Request an appointment By Mayo Clinic StaffDec 22, 2023
PrintShow ReferencesSickle cell disease. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease. Accessed Aug. 4, 2023. Field JJ, et al. Overview of the management and prognosis of sickle cell disease. https://www.uptodate.com/contents/search. Accessed Aug. 4, 2023. AskMayoExpert. Sickle cell disease. Mayo Clinic; 2022.Sickle cell disease (SCD). Centers for Disease Control and Prevention. https://www.cdc.gov/ncbddd/sicklecell/index.html. Accessed Aug. 4, 2023. Hoffman R, et al. Pain Management and Antiemetic Therapy in Hematologic Disorders. In: Hematology: Basic Principles and Practice. 8th ed. Elsevier; 2023. https://www.clinicalkey.com. Accessed Aug. 4, 2023.Ferri FF. Sickle cell disease. In: Ferri's Clinical Advisor 2024. Elsevier; 2024. https://www.clinicalkey.com. Accessed Aug. 4, 2023.Lyfgenia (prescribing information). Bluebird Bio; 2023. https://www.fda.gov/vaccines-blood-biologics/lyfgenia. Accessed Dec. 11, 2023.Casgevy (prescribing information). Vertex Pharmaceuticals; 2023. https://www.fda.gov/vaccines-blood-biologics/casgevy. Accessed Dec. 11, 2023. Diagnosis & treatmentDiseases & ConditionsSickle cell anemia symptoms & causesNews from Mayo Clinic Ask the Mayo Mom: Managing sickle cell disease in children and teens Mayo Clinic Minute: Sickle cell disease explainedMore Information Sickle cell anemiaAssociated Procedures Blood transfusion Bone marrow transplantProducts & Services A Book: Mayo Clinic Family Health Book, 5th Edition Newsletter: Mayo Clinic Health Letter — Digital EditionCON-XXXXXXXXSkip AdvertisementAdvertisementMayo Clinic does not endorse companies or products. Advertising revenue supports our not-for-profit mission.
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Sickle Cell Disease (SCD)
What is Sickle Cell Disease?
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Sickle cell disease (SCD) is a group of inherited red blood cell disorders. Red blood cells contain hemoglobin, a protein that carries oxygen. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, the hemoglobin is abnormal, which causes the red blood cells to become hard and sticky and look like a C-shaped farm tool called a “sickle.” The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious complications (health problems) such as infection, acute chest syndrome and stroke.
Types of SCD
There are several types of SCD. The specific type of SCD a person has depends on the genes they inherited from their parents. People with SCD inherit genes that contain instructions, or code, for abnormal hemoglobin.
Below are the most common types of SCD:
HbSS
People who have this form of SCD inherit two genes, one from each parent, that code for hemoglobin “S.” Hemoglobin S is an abnormal form of hemoglobin that causes the red cells to become rigid, and sickle shaped. This is commonly called sickle cell anemia and is usually the most severe form of the disease.
HbSC
People who have this form of SCD inherit a hemoglobin “S” gene from one parent and a gene for a different type of abnormal hemoglobin called “C” from the other parent. This is usually a milder form of SCD.
Infographic: 5 Facts You Should Know About Sickle Cell Disease
Did you know SCD affects people from many parts of the world?
HbS beta thalassemia
People who have this form of SCD inherit a hemoglobin “S” gene from one parent and a gene for beta thalassemia, another type of hemoglobin abnormality, from the other parent. There are two types of beta thalassemia: “zero” (HbS beta0) and “plus” (HbS beta+). Those with HbS beta0-thalassemia usually have a severe form of SCD. People with HbS beta+-thalassemia tend to have a milder form of SCD.
There also are a few rare types of SCD, such as the following:
HbSD, HbSE, and HbSO
People who have these forms of SCD inherit one hemoglobin “S” gene and one gene that codes for another abnormal type of hemoglobin (“D”, “E”, or “O”). The severity of these rarer types of SCD varies.
Sickle Cell Trait (SCT)
HbAS
People who have sickle cell trait (SCT) inherit a hemoglobin “S” gene from one parent and a normal gene (one that codes for hemoglobin “A”) from the other parent. People with SCT usually do not have any of the signs of the disease. However, in rare cases, a person with SCT may develop health problems; this occurs most often when there are other stresses on the body, such as when a person becomes dehydrated or exercises strenuously. Additionally, people who have SCT can pass the abnormal hemoglobin “S” gene on to their children.
Learn more about sickle cell trait »
Cause of SCD
SCD is a genetic condition that is present at birth. It is inherited when a child receives two genes—one from each parent—that code for abnormal hemoglobin.
Diagnosis
SCD is diagnosed with a simple blood test. In children born in the United States, it most often is found at birth during routine newborn screening tests at the hospital. In addition, SCD can be diagnosed while the baby is in the womb. Diagnostic tests before the baby is born, such as chorionic villus sampling and amniocentesis, can check for chromosomal or genetic abnormalities in the baby. Chorionic villus sampling tests a tiny piece of the placenta, called chorionic villus. Amniocentesis tests a small sample of amniotic fluid surrounding the baby.
Because children with SCD are at an increased risk of infection and other health problems, early diagnosis and treatment are important.
Talk to your doctor to find out how to get tested and to explain the results after testing.
SCD Fact Sheet
View and print
Complications
People with SCD may start to have signs of the disease during the first year of life, usually around 5 months of age. Symptoms and complications of SCD are different for each person and can range from mild to severe. Learn about the complications.
Prevention and Treatment of SCD Complications
General Prevention Strategies
Management of SCD is focused on preventing and treating pain episodes and other complications. Prevention strategies include lifestyle behaviors as well as medical screening and interventions to prevent SCD complications.
Lifestyle Behaviors
There are simple steps that people with SCD can take to help prevent and reduce the occurrence of pain crises, including the following:
Drink plenty of water.
Try not to get too hot or too cold.
Try to avoid places or situations that cause exposure to high altitudes (for example, flying, mountain climbing, or cities with a high altitude).
Try to avoid places or situations with exposure to low oxygen levels (for example, mountain climbing or exercising extremely hard, such as in military boot camp or when training for an athletic competition).
Simple steps to prevent harmful infections include the following:
Wash your hands often. Washing hands with soap and clean water many times each day is one of the best ways people with SCD, their family members, and other caregivers can help prevent an infection.
Prepare food safely. Bacteria can be especially harmful to children with SCD.
Medical Screenings & Interventions to Prevent SCD Complications
Prevention of Infections
Vaccines can protect against harmful infections. It is important that children with SCD get all regular childhood vaccines. Similarly, it is important for children and adults to get the flu vaccine every year, as well as the pneumococcal vaccine and any others recommended by a doctor.
Penicillin greatly reduces the risk of infections in people with HbSS and has been shown to be even more effective when it is started earlier. To decrease the risk of infection, it’s important that young children with HbSS take penicillin (or other antibiotic prescribed by a doctor) every day until at least 5 years of age. Penicillin on a daily basis is usually not prescribed for children with other types of SCD unless the severity of the disease is similar to that of HbSS, such as HbS beta0-thalassemia.
Prevention of Vision Loss
Yearly visits to an eye doctor to look for damage to the retina (the part of your eye that senses light and sends images to your brain) are important for people with SCD to avoid vision loss. If possible, it’s best to see an eye doctor who specializes in diseases of the retina.
If the retina is damaged by excessive blood vessel growth, laser treatment often can prevent further vision loss.
Prevention of Stroke
Children who are at risk for stroke can be identified using a special type of exam called transcranial Doppler ultrasound (TCD). If the child is found to have an abnormal TCD, a doctor might recommend frequent blood transfusions (a procedure in which new blood is put into a person’s body through a small plastic tube inserted into a person’s blood vessels) to help prevent a stroke.
People who have frequent blood transfusions are usually watched closely because there can be serious side effects. For example, because blood contains iron, transfusions can lead to a condition called iron overload, in which too much iron builds up in the body. Iron overload can cause life-threatening damage to the liver, heart, and other organs. Therefore, it is important for people with SCD receiving regular blood transfusions to also receive treatment to reduce excess iron in the body. This type of treatment is known as iron chelation therapy.
Prevention of Severe Anemia
Blood transfusions may be used to treat severe anemia. A sudden worsening of anemia resulting from infection or enlargement of the spleen (an organ in the upper left side of the abdomen) is a common reason for a transfusion.
As with preventing stroke, frequent blood transfusions can cause iron overload, and iron chelation therapy may be needed to reduce excess iron in the body.
Management of Pain Crises
When pain crises do occur, clinical management may include the following:
Intravenous fluids (giving fluids directly into a person’s vein)
Pain-reducing medicine
Hospitalization for severe pain crises
Specific Treatments to Prevent SCD Complications
SCD is a disease that worsens over time. Treatments are available that can prevent complications and lengthen the lives of those who have this condition. These treatment options and their effects can be different for each person, depending on the symptoms and severity of their disease. It is important to understand the benefits and risks of each treatment option. Currently, the FDA has approved four treatments for SCD[1].
Hydroxyurea (pronounced “hi-DROK-see-yoo-REE-uh”) may help people with SCD ages 2 years and older. More information about hydroxyurea can be found here.
L-glutamine (pronounced “L-gloo-ta-meen,”), or ENDARI® may help people with SCD ages 5 years and older. More information about L-glutamine can be found on page 2 here.
Voxelotor (pronounced “vox-EL-o-tor”), or OXBRYTA® may help people with SCD ages 4 years and older. More information about Voxelotor can be found on the FDA website here.
Crizanlizumab (pronounced “criz-an-liz-u-mab”), or ADAKVEO® may help people with SCD ages 16 years and older. More information about Crizanlizumab can be found on page 1 here.
Several other treatments and therapies for SCD have recently been developed that are still undergoing clinical trials and thus have not yet been approved by the FDA.
Cure
The only therapy approved by the FDA that may be able to cure SCD is a bone marrow or stem cell transplant.
Bone marrow is a soft, fatty tissue inside the center of the bones, where blood cells are made. A bone marrow or stem cell transplant is a procedure that takes healthy cells that form blood from one person—the donor—and puts them into someone whose bone marrow is not working properly.
Bone marrow or stem cell transplants are very risky and can have serious side effects, including death. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister. Bone marrow or stem cell transplants are most common in cases of severe SCD for children who have minimal organ damage from the disease.
Find clinical treatment guidelines for providers as well as patient and family resources derived from clinical treatment guidelines:
For Healthcare Providers
Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014, National Heart, Lung, and Blood Institute
Clinical Practice Guidelines on Sickle Cell Disease, American Society of Hematology
For People with SCD and their Families
Steps to Better Health for People with Sickle Cell Disease Toolkit
[1] CDC will periodically review and update this treatment list when new treatments are approved by the FDA.
Last Reviewed: July 6, 2023 Source: National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention
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Sickle Cell Anemia: Types, Symptoms, and Treatment
Sickle Cell Anemia: Types, Symptoms, and Treatment
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A Quiz for TeensAre You a Workaholic?How Well Do You Sleep?Tools & ResourcesHealth NewsFind a DietFind Healthy SnacksDrugs A-ZHealth A-ZConnectFind Your Bezzy CommunityBreast CancerInflammatory Bowel DiseasePsoriatic ArthritisMigraineMultiple SclerosisPsoriasisFollow us on social mediaHealthlineHealth ConditionsDiscoverPlanConnectSubscribeNutritionSpecial DietsHealthy EatingFood FreedomConditionsFeel Good FoodProductsVitamins & SupplementsSustainabilityWeight ManagementSickle Cell AnemiaMedically reviewed by Graham Rogers, M.D. — By The Healthline Editorial Team — Updated on June 11, 2019SymptomsTypesRisk factorsComplicationsDiagnosisTreatmentOutlook What is sickle cell anemia?Sickle cell anemia, or sickle cell disease (SCD), is a genetic disease of the red blood cells (RBCs). Normally, RBCs are shaped like discs, which gives them the flexibility to travel through even the smallest blood vessels. However, with this disease, the RBCs have an abnormal crescent shape resembling a sickle. This makes them sticky and rigid and prone to getting trapped in small vessels, which blocks blood from reaching different parts of the body. This can cause pain and tissue damage.SCD is an autosomal recessive condition. You need two copies of the gene to have the disease. If you have only one copy of the gene, you are said to have sickle cell trait. What are the symptoms of sickle cell anemia?Symptoms of sickle cell anemia usually show up at a young age. They may appear in babies as early as 4 months old, but generally occur around the 6-month mark. While there are multiple types of SCD, they all have similar symptoms, which vary in severity. These include:excessive fatigue or irritability, from anemiafussiness, in babiesbedwetting, from associated kidney problemsjaundice, which is yellowing of the eyes and skinswelling and pain in hands and feetfrequent infectionspain in the chest, back, arms, or legs What are the types of sickle cell disease?Hemoglobin is the protein in red blood cells that carries oxygen. It normally has two alpha chains and two beta chains. The four main types of sickle cell anemia are caused by different mutations in these genes.Hemoglobin SS diseaseHemoglobin SS disease is the most common type of sickle cell disease. It occurs when you inherit copies of the hemoglobin S gene from both parents. This forms hemoglobin known as Hb SS. As the most severe form of SCD, individuals with this form also experience the worst symptoms at a higher rate.Hemoglobin SC diseaseHemoglobin SC disease is the second most common type of sickle cell disease. It occurs when you inherit the Hb C gene from one parent and the Hb S gene from the other. Individuals with Hb SC have similar symptoms to individuals with Hb SS. However, the anemia is less severe.Hemoglobin SB+ (beta) thalassemiaHemoglobin SB+ (beta) thalassemia affects beta globin gene production. The size of the red blood cell is reduced because less beta protein is made. If inherited with the Hb S gene, you will have hemoglobin S beta thalassemia. Symptoms are not as severe. Hemoglobin SB 0 (Beta-zero) thalassemiaSickle beta-zero thalassemia is the fourth type of sickle cell disease. It also involves the beta globin gene. It has similar symptoms to Hb SS anemia. However, sometimes the symptoms of beta zero thalassemia are more severe. It is associated with a poorer prognosis.Hemoglobin SD, hemoglobin SE, and hemoglobin SO These types of sickle cell disease are more rare and usually don’t have severe symptoms.Sickle cell traitPeople who only inherit a mutated gene (hemoglobin S) from one parent are said to have sickle cell trait. They may have no symptoms or reduced symptoms. Who is at risk for sickle cell anemia?Children are only at risk for sickle cell disease if both parents carry sickle cell trait. A blood test called a hemoglobin electrophoresis can also determine which type you might carry.People from regions that have endemic malaria are more likely to be carriers. This includes people from:Africa Indiathe Mediterranean Saudi Arabia What complications can arise from sickle cell anemia?SCD can cause severe complications, which appear when the sickle cells block vessels in different areas of the body. Painful or damaging blockages are called sickle cell crises. They can be caused by a variety of circumstances, including:illnesschanges in temperaturestresspoor hydrationaltitudeThe following are types of complications that can result from sickle cell anemia.Severe anemiaAnemia is a shortage of RBCs. Sickle cells are easily broken. This breaking apart of RBCs is called chronic hemolysis. RBCs generally live for about 120 days. Sickle cells live for a maximum of 10 to 20 days.Hand-foot syndromeHand-foot syndrome occurs when sickle-shaped RBCs block blood vessels in the hands or feet. This causes the hands and feet to swell. It can also cause leg ulcers. Swollen hands and feet are often the first sign of sickle cell anemia in babies.Splenic sequestrationSplenic sequestration is a blockage of the splenic vessels by sickle cells. It causes a sudden, painful enlargement of the spleen. The spleen may have to be removed due to complications of sickle cell disease in an operation known as a splenectomy. Some sickle cell patients will sustain enough damage to their spleen that it becomes shrunken and ceases to function at all. This is called autosplenectomy. Patients without a spleen are at higher risk for infections from bacteria such as Streptococcus, Haemophilus, and Salmonella species.Delayed growthDelayed growth often occurs in people with SCD. Children are generally shorter but regain their height by adulthood. Sexual maturation may also be delayed. This happens because sickle cell RBCs can’t supply enough oxygen and nutrients.Neurological complications Seizures, strokes, or even coma can result from sickle cell disease. They are caused by brain blockages. Immediate treatment should be sought.Eye problemsBlindness is caused by blockages in the vessels supplying the eyes. This can damage the retina.Skin ulcersSkin ulcers in the legs can occur if small vessels there are blocked.Heart disease and chest syndromeSince SCD interferes with blood oxygen supply, it can also cause heart problems which can lead to heart attacks, heart failure, and abnormal heart rhythms.Lung diseaseDamage to the lungs over time related to decreased blood flow can result in high blood pressure in the lungs (pulmonary hypertension) and scarring of the lungs (pulmonary fibrosis). These problems can occur sooner in patients who have sickle chest syndrome. Lung damage makes it more difficult for the lungs to transfer oxygen into the blood, which can result in more frequent sickle cell crises.PriapismPriapism is a lingering, painful erection that can be seen in some men with sickle cell disease. This happens when the blood vessels in the penis are blocked. It can lead to impotence if left untreated.GallstonesGallstones are one complication not caused by a vessel blockage. Instead, they are caused by the breakdown of RBCs. A byproduct of this breakdown is bilirubin. High levels of bilirubin can lead to gallstones. These are also called pigment stones.Sickle chest syndromeSickle chest syndrome is a severe type of sickle cell crisis. It causes severe chest pain and is associated with symptoms such as cough, fever, sputum production, shortness of breath, and low blood oxygen levels. Abnormalities observed on chest X-rays can represent either pneumonia or death of lung tissue (pulmonary infarction). The long-term prognosis for patients who have had sickle chest syndrome is worse than for those who have not had it. How is sickle cell anemia diagnosed?All newborns in the United States are screened for sickle cell disease. Prebirth testing looks for the sickle cell gene in your amniotic fluid.In children and adults, one or more of the following procedures may also be used to diagnose sickle cell disease.Detailed patient historyThis condition often first appears as acute pain in the hands and feet. Patients may also have:severe pain in the bonesanemiapainful enlargement of the spleengrowth problemsrespiratory infectionsulcers of the legsheart problemsYour doctor may want to test you for sickle cell anemia if you have any of the symptoms mentioned above.Blood testsSeveral blood tests can be used to look for SCD:Blood counts can reveal an abnormal Hb level in the range of 6 to 8 grams per deciliter.Blood films may show RBCs that appear as irregularly contracted cells.Sickle solubility tests look for the presence of Hb S.Hb electrophoresisHb electrophoresis is always needed to confirm the diagnosis of sickle cell disease. It measures the different types of hemoglobin in the blood. How is sickle cell anemia treated?A number of different treatments are available for SCD:Rehydration with intravenous fluids helps red blood cells return to a normal state. The red blood cells are more likely to deform and assume the sickle shape if you’re dehydration. Treating underlying or associated infections is an important part of managing the crisis, as the stress of an infection can result in a sickle cell crisis. An infection may also result as a complication of a crisis. Blood transfusions improve transport of oxygen and nutrients as needed. Packed red cells are removed from donated blood and given to patients. Supplemental oxygen is given through a mask. It makes breathing easier and improves oxygen levels in the blood.Pain medication is used to relieve the pain during a sickle crisis. You may need over-the-counter drugs or strong prescription pain medication like morphine.(Droxia, Hydrea) helps to increase production of fetal hemoglobin. It may reduce the number of blood transfusions.Immunizations can help prevent infections. Patients tend to have lower immunity.Bone marrow transplant has been used to treat sickle cell anemia. Children younger than 16 years of age who have severe complications and have a matching donor are the best candidates.Home careThere are things you can do at home to help your sickle cell symptoms:Use heating pads for pain relief.Take folic acid supplements, as recommended by your doctor.Eat an adequate amount of fruits, vegetables, and whole-wheat grains. Doing so can help your body make more RBCs.Drink more water to reduce the chances of sickle cell crises.Exercise regularly and reduce stress to reduce crises, too.Contact your doctor immediately if you think you have any type of infection. Early treatment of an infection may prevent a full-blown crisis.Support groups can also help you deal with this condition. What is the long-term outlook for sickle cell disease?The prognosis of the disease varies. Some patients have frequent and painful sickle cell crises. Others only rarely have attacks. Sickle cell anemia is an inherited disease. Talk to a genetic counselor if you’re worried that you might be a carrier. This can help you understand possible treatments, preventive measures, and reproductive options.Facts about sickle cell disease. (2016, November 17). Retrieved from http://www.cdc.gov/ncbddd/sicklecell/facts.htmlLópez, C., Saravia, C., Gomez, A., Hoebeke, J., & Patarroyo, M. A. (2010, November 1) Mechanisms of genetically-based resistance to malaria. Gene, 467(1-2), 1-12 Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/20655368Mayo Clinic Staff. (2016, December 29). Sickle cell anemia. Retrieved from http://www.mayoclinic.com/health/sickle-cell-anemia/DS00324Sickle cell anemia. (2016, February 1). Retrieved from http://www.umm.edu/ency/article/000527.htm Article sources What are the signs and symptoms of sickle cell disease? (2016, August 2). Retrieved from http://www.nhlbi.nih.gov/health/health-topics/topics/sca/signs Last medically reviewed on March 29, 2017How we reviewed this article:HistoryOur experts continually monitor the health and wellness space, and we update our articles when new information becomes available.Current VersionJun 11, 2019Written ByThe Healthline Editorial TeamMar 29, 2017Medically Reviewed ByGraham Rogers, MDShare this articleMedically reviewed by Graham Rogers, M.D. — By The Healthline Editorial Team — Updated on June 11, 2019Read this nextSickle Cell TestMedically reviewed by Karen Gill, M.D.A sickle cell test is a blood test used to determine if you have sickle cell disease (SCD) or sickle cell trait. People with SCD have abnormally…READ MOREHow Is Sickle Cell Anemia Inherited?Medically reviewed by Karen Gill, M.D.Sickle cell anemia is an inherited condition in which a person’s red blood cells are shaped like a crescent or sickle. Learn what genes each parent…READ MOREChronic AnemiaMedically reviewed by Alana Biggers, M.D., MPHIf you have anemia, your level of red blood cells is lower than normal. Chronic anemia results from long-term health conditions that affect your…READ MOREWhat Is Anemia?Medically reviewed by Debra Sullivan, Ph.D., MSN, R.N., CNE, COIAnemia happens when the number of healthy red blood cells in your body is too low. We’ll show you what causes this condition and what symptoms to…READ MOREWhat Is Anisocytosis?Medically reviewed by Suzanne Falck, MDUnderstand anisocytosis, its relation to anemia, and how it is typically diagnosed and treated.READ MOREBlood Diseases: White and Red Blood Cells, Platelets and PlasmaMedically reviewed by Shuvani Sanyal, MDBlood cell disorders impair the formation and function of red blood cells, white blood cells, or platelets.READ MORERed Blood Cell (RBC) CountMedically reviewed by Avi Varma, MD, MPH, AAHIVS, FAAFPAn RBC count is used to find out how many red blood cells you have. Learn why your doctor might order one, how it’s performed, and what results mean.READ MORECan Sickle Cell Anemia Be Cured?A stem cell transplant may cure sickle cell anemia, but the risks often outweigh the benefits. New therapies like gene therapy may have promise.READ MOREBlack People and Sickle Cell Anemia: Your Questions AnsweredMedically reviewed by Alana Biggers, M.D., MPHLearn why sickle cell anemia affects African Americans at significantly higher rates than other groups.READ MOREAbout UsContact UsPrivacy PolicyPrivacy SettingsAdvertising PolicyHealth TopicsMedical AffairsContent IntegrityNewsletters© 2024 Healthline Media LLC. All rights reserved. Our website services, content, and products are for informational purposes only. Healthline Media does not provide medical advice, diagnosis, or treatment. See additional information. See additional information.© 2024 Healthline Media LLC. All rights reserved. Our website services, content, and products are for informational purposes only. Healthline Media does not provide medical advice, diagnosis, or treatment. See additional information. 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Sickle cell disease is a group of inherited red blood cell disorders that affect hemoglobin, the protein that carries oxygen through the body. Normally, red blood cells are disc-shaped and flexible enough to move easily through the blood vessels. In sickle cell disease, red blood cells become crescent- or “sickle”-shaped due to a genetic mutation. These sickled red blood cells do not bend or move easily and can block blood flow to the rest of the body.The blocked blood flow through the body can lead to serious problems, including stroke, eye problems, infections, and episodes of pain called pain crises.Sickle cell disease is a lifelong illness. A bone marrow transplant is currently the only cure for sickle cell disease. Gene therapy is also being explored as another potential cure, but there are other effective treatments that can reduce symptoms and prolong life. If you have sickle cell disease, your healthcare team will work with you on a treatment plan to reduce your symptoms and manage the condition.The condition affects more than 100,000 people in the United States and 20 million people worldwide. In the United States, most people who have sickle cell disease are of African ancestry or identify themselves as Black.About 1 in 13 Black or African American babies are born with sickle cell trait.About 1 in every 365 Black or African American babies are born with sickle cell disease.Many people who come from Hispanic, Southern European, Middle Eastern, or Asian Indian backgrounds also have sickle cell disease.NHLBI is leading and supporting research and clinical trials to find a cure for sickle cell disease.
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sickle, one of the most ancient of harvesting tools, consisting of a metal blade, usually curved, attached to a short wooden handle. The short handle forces the user to harvest in a stooped or squatting position. The longer-handled scythe, the user of which remains upright, evolved from the sickle. Harvesting with a sickle is very slow, but because of its simplicity and low cost, it is still widely used over the world, especially to reap cereals such as wheat and rice and also as a gardening tool.
What Is Sickle Cell Anemia? Everything You Should Know
Is Sickle Cell Anemia? Everything You Should KnowHealth CategoriesDiscoverHealth ToolsCommunitiesSee All CategoriesAll In FocusSee All In FocusKalusugan Ng KababaihanSexual WellnessPain, Pain, Go Away!June is Nat'l Kidney MonthDiscoverHealth CategoriesCancerWomen's HealthHeart HealthInfectious DiseasesSexual WellnessSkin HealthSee All CategoriesAll In FocusSee All In FocusKalusugan Ng KababaihanSexual WellnessPain, Pain, Go Away!June is Nat'l Kidney MonthHealth ToolsHealth ToolsKidney Disease Symptoms Screener - Hello DoctorKidney Disease Risk ScreenerChild Growth ChartNeuropathic Pain Assessment ToolSevere Acne Assessment Online - Hello DoctorAcne Scarring Assessment Online - Hello DoctorSee All Health ToolsFavorite ToolsBMI CalculatorCalculate your Body Mass Index (BMI) by using this quick and easy tool.See MoreSigns of Pneumonia Risk Screener - Hello DoctorSee MoreCervical Cancer Symptoms Assessment Online - Hello DoctorSee MoreCommunitiesFind your communitiesDiabetesParentingSkin HealthSee all communitiesHighlight PostsSee moreDrewPregnancy•3 yearsIngat mga moms. If you think you are experiencing depression,...HelloDoctorDiabetes•2 yearsPagkain para sa Gestational Diabetes: Heto ang Dapat mong KaininLanie SeneraParenting•2 yearsLahat ba ng buntis ay dapat magpa BPS ultrasound? HelloDoctorDiabetes•2 yearsMaagang Sintomas Ng Diabetes Na Dapat Mong MalamanENENFILBlood DisordersAnemiaDiscoverHealth ScreeningAsk DoctorSaveCommentsShare your thoughtsBe the first to let Hello Doctor know your thoughts!Join Hello DoctorStart a new postJoin Us
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to join the discussionWhat Is Sickle Cell Anemia?Medically reviewed by Jobelle Ann Dela Cruz Bigalbal, MD · General PractitionerWritten by Kip Soliva · Updated Jun 02, 2021The human body is composed of intricate systems that all have some specific purpose so the body functions as it should. Aside from the organs, the blood also plays a vital role. Blood is mainly responsible for transporting nutrients and oxygen to the different organs. It also collects waste that the body will dispose of. Sickle cell anemia mainly affects the natural functioning of the blood. Over time, sickle cell anemia can cause damage to the different organs such as the lungs or the heart. Learn more about this condition’s causes, signs and symptoms, and treatment. Sickle Cell Anemia: the BasicsSickle cell anemia, also known as sickle cell disease, sickling disorder caused by hemoglobin S, or hemoglobin S or SS disease, is a red blood cell disorder mainly affecting the hemoglobin in blood which is responsible for transporting oxygen and collecting carbon dioxide. A healthy person possesses red blood cells with hemoglobin that are smooth and round. This enables red blood cells to easily glide through the blood vessels. However, a person with sickle cell anemia will have red blood cells containing hemoglobin that are rod-shaped. This causes red blood cells to be shaped like crescents or sickles. Everything You Need to Know About AnemiaThe sickle-shaped red blood cells are inflexible and sticky, causing them to get stuck or burst when they’re travelling in small blood vessels. Sickle cells also have a shorter lifespan of only 10 to 20 days which means that the body may not be able to replace the red blood cells lost. A shortage of red blood cells is called anemia, which is why this disease is called “sickle cell anemia’.Anemia vs. Sickle Cell Anemia: What’s the Difference? Confusion may arise between anemia and sickle cell anemia. In general, anemia is a term that refers to disorders affecting the red blood cells. Anemia occurs when there is a lack of or a malfunction of the red blood cells. Sickle cell anemia is categorized as a type of anemia. However, there are some kinds of anemia that can have different causes. Types of Anemia Other varieties of anemia include: Iron-deficiency anemia: The body’s inability to properly absorb iron causes this type of anemia, which reduces the amount of red blood cells. Vitamin-deficiency anemia: This is a type of anemia that may result from a poor diet severely lacking in vitamin B12 or folic acid. Aplastic anemia: This happens when the bone marrow fails to produce any cells, including red blood cells. Hemolytic anemia: The destruction of red blood cells in the spleen or blood vessels characterizes this type of anemia. Disease-related anemia: Anemia can develop from conditions affecting the kidney. Chemotherapy can also affect the body’s ability to create more red blood cells. Sickle cell anemia is different because this type of anemia is inherited, which means that it can be passed through genes. Who Is at Risk? Anyone can get sickle cell anemia. This disorder is passed on from generation to generation. However, data shows that people with ancestry located in the following parts of the world are more at risk: Africa The Arabian Peninsula The Mediterranean (in particular, Greece, Turkey, and Italy) South America and Central America Some regions of the Caribbean However, it’s important to note that this condition can happen to anyone regardless of their nationality. Signs and Symptoms Since sickle cell anemia is inherited, symptoms can start showing as early as five months of age. Signs and symptoms include:Anemia A shortage of red blood cells in the body is called anemia. A person with sickle cell anemia has red blood cells that don’t live as long as healthy ones, which will cause a lack of oxygen in the body. This can lead to fatigue. Pain crises Acute pain crisis can occur without warning when sickle cells block blood flow and decrease oxygen delivery. A lowered amount of red blood cells, coupled with the immobility of sickle cells, will cause some parts of the body to not receive enough blood. This can cause episodes of pain often referred to as “pain crises“. These episodes can affect any part of the body, such as the chest, joints, legs, arms, or back. The duration of these pain crises can range from a few hours to weeks. Sometimes, it can cause severe pain that requires hospitalization. Acute pain crisis can occur without warning when sickle cells block blood flow and decrease oxygen delivery. Vision problems Sickle cells can block proper blood flow to the small blood vessels in the eyes, damaging or impairing the retina. This can lead to vision problems. Swelling in the hands and feet Sickle cells, due to their irregular shape, may decrease blood flow to the hands and feet, which can cause swelling.Growth irregularities A shortage of red blood cells can mean that the body won’t be able to receive enough oxygen and nourishment to grow at the right pace. Children with sickle cell anemia might grow slower compared to other kids. Infections Sickle cell disease can cause damage to the spleen, which can result in the body having less protection against certain infections like salmonella or chlamydia. Prevention and Treatment Unfortunately, since sickle cell anemia is an inherited disease, there’s not much to do to prevent it. If a person has sickle cell anemia, then they will have had it from birth. Currently, treatment for sickle cell disease is a blood and bone marrow transplant. However, this treatment is only effective for younger patients as the procedure is too risky for older people. Conclusion Sickle cell anemia results from an abnormality in the shape of the hemoglobin in the blood, caused by crescent or sickle-shaped red blood cells. This can result in a shortage of red blood cells in the body, or anemia. This disease is inherited, meaning it’s passed on from the genes of parents to their child. Learn more about Anemia here. DisclaimerHello Health Group does not provide medical advice, diagnosis or treatment.SourcesSickle Cell Disease, https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease, Accessed Aug. 4, 2020
Sickle Cell Disease, https://medlineplus.gov/sicklecelldisease.html, Accessed Aug. 4, 2020
Anemia, https://www.hematology.org/education/patients/anemia, Accessed Aug. 4, 2020
Sickle Cell Crisis (Pain Crisis), https://kidshealth.org/en/teens/sickle-crisis.html, Accessed Aug. 4, 2020
Sickle Cell Anemia, https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876, Accessed Aug. 4, 2020
HistoryCurrent Version06/02/2021Written by Kip SolivaMedically reviewed by Jobelle Ann Dela Cruz Bigalbal, MDUpdated by: Vincent SalesPeople Are Also Reading ThisChildhood Anemia: What Parents Need To KnowHow to Prevent Anemia NaturallyMedically reviewed byJobelle Ann Dela Cruz Bigalbal, MDGeneral PractitionerWritten by Kip Soliva · Updated Jun 02, 2021advertisementWas this article helpful?advertisementadvertisement LoadingHello Doctor wants to be your most trusted ally to make more informed decisions and to live healthier and happier lives.Sign Up NowFollow usDiscoverHealth ToolsInformationTerms Of UsePrivacy policyEditorial & Corrections PolicyAdvertising & Sponsor PolicyCommunity GuidelinesHello HealthAbout UsExecutive biosRecruitmentContact UsVisit our other Hello Health sites©2023 Hello Health Group Pte. Ltd. All Rights Reserved. Hello Health Group does not provide medical advice, diagnosis or treatment.
What is Sickle Cell Disease? | CDC
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Sickle Cell Disease (SCD)
What is Sickle Cell Disease?
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Sickle cell disease causes “sickle” shaped red blood cells.
Sickle cell disease (SCD) is an inherited blood disorder that causes “sickle” shaped red blood cells that can stick together, blocking blood flow and oxygen from reaching all parts of the body. People with SCD can experience pain, anemia, infection, and other serious health problems (also known as complications) that may require care by a healthcare provider. Below are a variety of resources people with SCD and their caregivers may find helpful.
Resources for Patients and Caregivers
SCD materials webpage has fact sheets and infographics on living well with SCD. SCD resources are also available in Spanish at our SCD Spanish webpage.
Sickle Cell Trait Toolkit is a collection of fact sheets covering various health problems affecting people with sickle cell trait.
Stepping Up: A 2-part video series for teenagers and young adults with SCD. The videos share the stories of Kevin and Calvanay, two young adults with SCD, and their transition to adult care, how it has affected them, and how they’ve overcome challenges. Spanish transcripts of the videos are available online.
SCDC webinars, hosted by the SCDC program in California, are free and open to the public. Watch previous recordings or sign up to join the email list for announcements about upcoming webinars.
Steps to Better Health Toolkit is a collection of health guidance materials for people with SCD based on the ASH Clinical Practice Guidelines on Sickle Cell Disease. These materials include information about the common complications of SCD and how patients can manage their health.
Real Stories of People Living with SCD is a collection of personal stories shared by people who have SCD to increase awareness of the disease, show support, and offer tips for others recently diagnosed with the condition.
5 Facts You Should Know About SCD Infographics (information graphics) are graphic pictures of data, information or knowledge that tell a story. Check out this infographic to learn important facts about SCD.
Sickle Cell Has Many Faces video for a general audience. Watch people with SCD of diverse backgrounds share that SCD can affect anyone, no matter what you look like or where your family comes from.
A Message for Healthcare Providers: Sickle Cell Has Many Faces video targets healthcare providers, community workers, policymakers, and other SCD supporters to increase their awareness of the fact that SCD affects diverse groups so that all people with SCD can be properly diagnosed and treated.
Last Reviewed: July 6, 2023 Source: National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention
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Overview of Anemia
Anemia Due to Excessive Bleeding
Anemia of Chronic Disease
Aplastic Anemia
Autoimmune Hemolytic Anemia
Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
Hemoglobin C, S-C, and E Diseases
Iron Deficiency Anemia
Sickle Cell Disease
Thalassemias
Vitamin Deficiency Anemia
Sickle Cell Disease
(Hemoglobin S Disease)
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Evan M. Braunstein
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Reviewed/Revised Jul 2022 | Modified Sep 2022
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Sickle Cell Disease
Sickle cell disease is an inherited genetic abnormality of hemoglobin (the oxygen-carrying protein found in red blood cells) characterized by sickle (crescent)-shaped red blood cells and chronic anemia caused by excessive destruction of the abnormal red blood cells. People always have anemia and sometimes jaundice. Worsening anemia, fever, and shortness of breath with pain in the long bones, abdomen, and chest can indicate sickle cell crisis. A special blood test called electrophoresis can be used to determine whether people have sickle cell disease. Avoiding activities that may cause crises and treating infections and other disorders quickly can help prevent crises. (See also Overview of Anemia Overview of Anemia Anemia is a condition in which the number of red blood cells is low. Red blood cells contain hemoglobin, a protein that enables them to carry oxygen from the lungs and deliver it to all parts... read more .) Sickle cell disease affects people with African or Black American ancestry almost exclusively. About 10% of people with such ancestry in the United States have one copy of the gene for sickle cell disease (that is, they have sickle cell trait). People who have sickle cell trait do not develop sickle cell disease, but they do have increased risks of some complications such as blood in their urine. About 0.3% of people with African or Black American ancestry have two copies of the gene. These people develop the disease. Red Blood Cell Shapes Normal red blood cells are flexible and disk-shaped, thicker at the edges than in the middle. In several hereditary disorders, red blood cells become spherical (in hereditary spherocytosis), oval (in hereditary elliptocytosis), or sickle-shaped (in sickle cell disease). In sickle cell disease, the red blood cells contain an abnormal form of hemoglobin (the protein that carries oxygen). The abnormal form of hemoglobin is called hemoglobin S. When red blood cells contain a large amount of hemoglobin S, they can become deformed into a sickle shape and less flexible. Not every red blood cell is sickle-shaped. The sickle-shaped cells become more numerous when people have infections or low levels of oxygen in the blood. The sickle cells are fragile and break apart easily. Because the sickle cells are stiff, they have difficulty traveling through the smallest blood vessels (capillaries), blocking blood flow and reducing oxygen supply to tissues in areas where capillaries are blocked. The blockage of blood flow can cause pain and, over time, cause damage to the spleen, kidneys, brain, bones, and other organs. Kidney failure Chronic Kidney Disease Chronic kidney disease is a slowly progressive (months to years) decline in the kidneys’ ability to filter metabolic waste products from the blood. Major causes are diabetes and high blood pressure... read more and heart failure Heart Failure (HF) Heart failure is a disorder in which the heart is unable to keep up with the demands of the body, leading to reduced blood flow, back-up (congestion) of blood in the veins and lungs, and/or... read more may occur. Sickle Cell Disease VIDEO Symptoms of Sickle Cell Disease People who have sickle cell disease always have some degree of anemia (often causing fatigue, weakness, and paleness) and may have mild jaundice Jaundice in Adults In jaundice, the skin and whites of the eyes look yellow. Jaundice occurs when there is too much bilirubin (a yellow pigment) in the blood—a condition called hyperbilirubinemia. (See also Overview... read more (yellowing of the skin and whites of the eyes). Some people have few other symptoms. Others have severe, recurring symptoms that cause enormous disability and early death. Sickle cell trait In people with sickle cell trait, red blood cells are not fragile and do not break easily. Sickle cell trait does not cause painful crises, but rarely, people die suddenly while undergoing very strenuous exercise that causes severe dehydration, such as during military or athletic training. People with sickle cell trait are at increased risk of chronic kidney disease Chronic Kidney Disease Chronic kidney disease is a slowly progressive (months to years) decline in the kidneys’ ability to filter metabolic waste products from the blood. Major causes are diabetes and high blood pressure... read more and pulmonary embolism Pulmonary Embolism (PE) Pulmonary embolism is the blocking of an artery of the lung (pulmonary artery) by a collection of solid material brought through the bloodstream (embolus)—usually a blood clot (thrombus) or... read more . Rarely, they may notice blood in their urine. People with sickle cell trait are also at risk for an extremely rare form of kidney cancer. Sickle cell crisis Anything that reduces the amount of oxygen in the blood, such as vigorous exercise, mountain climbing, flying at high altitudes without sufficient oxygen, or an illness, may bring on a sickle cell crisis (also called an exacerbation). A sickle cell pain (vaso-occlusive) crisis is an episode of increased symptoms and can consist of a sudden worsening of anemia, pain (often in the abdomen or long bones of the arms and legs), fever, and sometimes shortness of breath. Abdominal pain may be severe, and vomiting may occur. Sometimes, additional complications occur with a pain crisis, including Aplastic crisis: Production of red blood cells in the bone marrow stops during infection with some viruses Acute chest syndrome: Caused by blockage of capillaries in the lungs Acute splenic or hepatic (liver) sequestration (a large accumulation of cells in an organ): Rapid enlargement of the spleen or liver Acute chest syndrome can occur in people of all ages, but it is most common among children. It is usually characterized by severe pain and difficulty breathing. Acute chest syndrome can be fatal. In children, acute sequestration of sickled cells in the spleen (sequestration crisis) may occur causing an enlarged spleen and worsening anemia. Acute hepatic (liver) sequestration is less common and can occur at any age. Complications Most people who have sickle cell disease develop an enlarged spleen Enlarged Spleen An enlarged spleen is not a disease in itself but the result of an underlying disorder. Many disorders can make the spleen enlarge. Many disorders, including infections, anemias, and cancers... read more during childhood because sickled cells become trapped in the spleen. By the time the person reaches adolescence, the spleen is often so badly injured that it shrinks and no longer functions. Because the spleen helps fight infection, people with sickle cell disease are more likely to develop pneumococcal pneumonia Pneumococcal Infections Pneumococcal infections are caused by the gram-positive, sphere-shaped (coccal) bacteria (see figure How Bacteria Shape Up) Streptococcus pneumoniae (pneumococci). These bacteria commonly... read more and other infections. Viral infections, in particular, can decrease red blood cell production, so anemia becomes more severe. The liver can become progressively larger throughout life (causing upper abdominal fullness), and gallstones Gallstones Gallstones are collections of solid material (predominantly crystals of cholesterol) in the gallbladder. The liver can secrete too much cholesterol, which is carried with bile to the gallbladder... read more often form from the pigment of broken-apart red blood cells. The heart usually enlarges, and an enlarged heart is less effective in pumping blood to the body, possibly leading to heart failure Heart Failure (HF) Heart failure is a disorder in which the heart is unable to keep up with the demands of the body, leading to reduced blood flow, back-up (congestion) of blood in the veins and lungs, and/or... read more . Heart murmurs are common. Children who have sickle cell disease often have a relatively short torso but long arms, legs, fingers, and toes. Changes in the bones and bone marrow may cause bone pain, especially in the hands and feet. Episodes of joint pain with fever may occur, and the hip joint may become so damaged that it eventually needs to be replaced. Poor circulation to the skin may cause sores on the legs, especially at the ankles. Young men may develop persistent, often painful erections (priapism Persistent Erection Persistent erection (priapism) is a painful, persistent, abnormal erection unaccompanied by sexual desire or excitation. It is most common in boys aged 5 to 10 years and in men aged 20 to 50... read more ). Episodes of priapism may permanently damage the penis so that the man can no longer have erections. Blocked blood vessels may cause strokes that damage the nervous system. In older people, lung and kidney function may deteriorate. Diagnosis of Sickle Cell Disease Blood tests Hemoglobin electrophoresis Prenatal testing Doctors recognize anemia, stomach and bone pain, and nausea in a young person with African or Black American ancestry as possible signs of a sickle cell crisis. When doctors suspect sickle cell disease, they do blood tests. Sickle-shaped red blood cells and fragments of destroyed red blood cells can be seen in a blood sample examined under a microscope. Hemoglobin electrophoresis, another blood test, is also done. In electrophoresis, an electrical current is used to separate the different types of hemoglobin and thus detect abnormal hemoglobin. Further testing may be needed, depending on the specific symptoms the person experiences during the crisis. For example, if the person has difficulty breathing or a fever, a chest x-ray may be done. Screening Blood tests are done on relatives of people with the disorder because they also may have sickle cell disease or trait. Discovering the trait in people may be important for family planning, to determine their risk of having a child with sickle cell disease. In the United States, newborns are routinely screened with a blood test. Tests can be done during early pregnancy to screen the fetus and allow prenatal counseling Prenatal Testing for Genetic Disorders and Birth Defects Prenatal testing for genetic disorders and birth defects involves testing a pregnant woman or fetus before birth (prenatally) to determine whether the fetus has certain abnormalities, including... read more for couples who are at risk of having a child with sickle cell disease. Fetal cells obtained through amniocentesis Amniocentesis Prenatal testing for genetic disorders and birth defects involves testing a pregnant woman or fetus before birth (prenatally) to determine whether the fetus has certain abnormalities, including... read more or chorionic villus sampling Chorionic Villus Sampling Prenatal testing for genetic disorders and birth defects involves testing a pregnant woman or fetus before birth (prenatally) to determine whether the fetus has certain abnormalities, including... read more are tested for the presence of the sickle cell gene. Treatment of Sickle Cell Disease Treatments aimed at preventing crises Treatment of crises and conditions that cause them Treatment is aimed at Preventing crises Controlling the anemia Relieving symptoms Stem cell transplantation Stem Cell Transplantation Stem cell transplantation is the removal of stem cells (undifferentiated cells) from a healthy person and their injection into someone who has a serious blood disorder. (See also Overview of... read more may cure sickle cell disease. Bone marrow or stem cells from a family member or other donor who does not have the sickle cell gene may be transplanted in a person with the disease. Although such transplantation may be curative, it is risky and so it is not often done. Recipients must take drugs that suppress the immune system for the rest of their life. Gene therapy, a technique in which normal genes are implanted in precursor cells (cells that produce blood cells), is being studied. Preventing sickle cell crisis People who have sickle cell disease should try to avoid activities that reduce the amount of oxygen in their blood and should seek prompt medical attention for even minor illnesses, such as viral infections. Because people are at increased risk of infection, they should receive vaccines for pneumococcal Pneumococcal Vaccine Pneumococcal vaccines help protect against bacterial infections caused by Streptococcus pneumoniae (pneumococci). Pneumococcal infections include ear infections, sinusitis, pneumonia... read more , meningococcal Meningococcal Vaccine The meningococcal vaccine protects against infections caused by the bacteria Neisseria meningitidis (meningococci). Meningococcal infections can lead to meningitis (an infection of tissue... read more , influenza Influenza (Flu) Influenza (flu) is a viral infection of the lungs and airways with one of the influenza viruses. It causes a fever, runny nose, sore throat, cough, headache, muscle aches (myalgias), and a general... read more , and Haemophilus influenzae type b Haemophilus influenzae Type b Vaccine The Haemophilus influenzae type b (Hib) vaccine helps protect against bacterial infections due to Hib, such as pneumonia and meningitis. These infections may be serious in children. Use... read more infections. Children typically take penicillin by mouth from age 4 months until age 6 years. Drugs can help control sickle cell disease. For example, hydroxyurea increases the production of a form of hemoglobin found predominantly in fetuses, which decreases the number of red blood cells becoming sickle-shaped. Therefore, it reduces the frequency of sickle cell crises and acute chest syndrome. New drugs that help control the symptoms and complications of sickle cell disease are L-glutamine, crizanlizumab, and voxelotor. Controlling anemia People are given folic acid, a vitamin that helps the body make new red blood cells. Blood transfusions Overview of Blood Transfusion A blood transfusion is the transfer of blood or a blood component from one healthy person (a donor) to a sick person (a recipient). Transfusions are given to increase the blood's ability to... read more may be given to correct the anemia. Treating sickle cell crisis Sickle cell crisis may require hospitalization. People are given oxygen, fluid by vein (intravenously), and drugs to relieve pain. Blood transfusions Overview of Blood Transfusion A blood transfusion is the transfer of blood or a blood component from one healthy person (a donor) to a sick person (a recipient). Transfusions are given to increase the blood's ability to... read more and oxygen are given for anemia that is severe enough to pose a risk of stroke, heart attack, or lung damage. Conditions that may have caused the crisis, such as an infection, are treated. More Information The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource. Sickle Cell Disease Association of America: provides comprehensive education and support, including peer mentoring, to people with sickle cell disease and their caregivers
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