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Docs/Blood Basics.txt ADDED
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+ Blood Basics
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+ Blood is a specialized body fluid. It has four main components: plasma, red blood cells, white blood cells, and platelets. Blood has many different functions, including:
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+ * transporting oxygen and nutrients to the lungs and tissues
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+ * forming blood clots to prevent excess blood loss
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+ * carrying cells and antibodies that fight infection
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+ * bringing waste products to the kidneys and liver, which filter and clean the blood
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+ * regulating body temperature
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+ The blood that runs through the veins, arteries, and capillaries is known as whole blood, a mixture of about 55 percent plasma and 45 percent blood cells. About 7 to 8 percent of your total body weight is blood. An average-sized man has about 12 pints of blood in his body, and an average-sized woman has about nine pints.
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+ The Components of Blood and Their Importance
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+ Many people have undergone blood tests or donated blood, but hematology - the study of blood - encompasses much more than this. Doctors who specialize in hematology (hematologists) are leading the many advances being made in the treatment and prevention of blood diseases.
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+ If you or someone you care about is diagnosed with a blood disorder, your primary care physician may refer you to a hematologist for further testing and treatment.
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+ Plasma
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+ The liquid component of blood is called plasma, a mixture of water, sugar, fat, protein, and salts. The main job of the plasma is to transport blood cells throughout your body along with nutrients, waste products, antibodies, clotting proteins, chemical messengers such as hormones, and proteins that help maintain the body's fluid balance.
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+ Red Blood Cells (also called erythrocytes or RBCs)
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+ Known for their bright red color, red cells are the most abundant cell in the blood, accounting for about 40 to 45 percent of its volume. The shape of a red blood cell is a biconcave disk with a flattened center - in other words, both faces of the disc have shallow bowl-like indentations (a red blood cell looks like a donut).
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+ Production of red blood cells is controlled by erythropoietin, a hormone produced primarily by the kidneys. Red blood cells start as immature cells in the bone marrow and after approximately seven days of maturation are released into the bloodstream. Unlike many other cells, red blood cells have no nucleus and can easily change shape, helping them fit through the various blood vessels in your body. However, while the lack of a nucleus makes a red blood cell more flexible, it also limits the life of the cell as it travels through the smallest blood vessels, damaging the cell's membranes and depleting its energy supplies. The red blood cell survives on average only 120 days.
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+ Red cells contain a special protein called hemoglobin, which helps carry oxygen from the lungs to the rest of the body and then returns carbon dioxide from the body to the lungs so it can be exhaled. Blood appears red because of the large number of red blood cells, which get their color from the hemoglobin. The percentage of whole blood volume that is made up of red blood cells is called the hematocrit and is a common measure of red blood cell levels.
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+ White Blood Cells (also called leukocytes)
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+ White blood cells protect the body from infection. They are much fewer in number than red blood cells, accounting for about 1 percent of your blood.
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+ The most common type of white blood cell is the neutrophil, which is the "immediate response" cell and accounts for 55 to 70 percent of the total white blood cell count. Each neutrophil lives less than a day, so your bone marrow must constantly make new neutrophils to maintain protection against infection. Transfusion of neutrophils is generally not effective since they do not remain in the body for very long.
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+ The other major type of white blood cell is a lymphocyte. There are two main populations of these cells. T lymphocytes help regulate the function of other immune cells and directly attack various infected cells and tumors. B lymphocytes make antibodies, which are proteins that specifically target bacteria, viruses, and other foreign materials.
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+ Platelets (also called thrombocytes)
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+ Unlike red and white blood cells, platelets are not actually cells but rather small fragments of cells. Platelets help the blood clotting process (or coagulation) by gathering at the site of an injury, sticking to the lining of the injured blood vessel, and forming a platform on which blood coagulation can occur. This results in the formation of a fibrin clot, which covers the wound and prevents blood from leaking out. Fibrin also forms the initial scaffolding upon which new tissue forms, thus promoting healing.
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+ A higher than normal number of platelets can cause unnecessary clotting, which can lead to strokes and heart attacks; however, thanks to advances made in antiplatelet therapies, there are treatments available to help prevent these potentially fatal events. Conversely, lower than normal counts can lead to extensive bleeding.
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+ Complete Blood Count (CBC)
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+ A complete blood count (CBC) test gives your doctor important information about the types and numbers of cells in your blood, especially the red blood cells and their percentage (hematocrit) or protein content (hemoglobin), white blood cells, and platelets. The results of a CBC may diagnose conditions like anemia, infection, and other disorders. The platelet count and plasma clotting tests (prothombin time, partial thromboplastin time, and thrombin time) may be used to evaluate bleeding and clotting disorders.
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+ Your doctor may also perform a blood smear, which is a way of looking at your blood cells under the microscope. In a normal blood smear, red blood cells will appear as regular, round cells with a pale center. Variations in the size or shape of these cells may suggest a blood disorder.
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+ Where Do Blood Cells Come From?
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+ Blood cells develop from hematopoietic stem cells and are formed in the bone marrow through the highly regulated process of hematopoiesis. Hematopoietic stem cells are capable of transforming into red blood cells, white blood cells, and platelets. These stem cells can be found circulating in the blood and bone marrow in people of all ages, as well as in the umbilical cords of newborn babies. Stem cells from all three sources may be used to treat a variety of diseases, including leukemia, lymphoma, bone marrow failure, and various immune disorders.
Docs/Prenatal Diagonosis.txt ADDED
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+ WHAT IS PRENATAL DIAGNOSIS? Prenatal diagnosis (PND) is a diagnosis made in an unborn child (foetus or fetus) still in the mother’s womb. PND is usually requested by a couple who have a risk of bearing children with a particular disorder, usually an inherited one, such as sickle cell anaemia. WHY CHOOSE PND OF SICKLE CELL DISORDER? It is worth noting that most couples who are at risk of bearing children affected with sickle cell anaemia (Hb SS) are themselves healthy carriers of the sickle cell gene (i.e. both would have Hb AS) and thus have 3 out of 4 chances (a 75% chance) in each pregnancy, of bearing an unaffected child (i.e. with Hb AA or AS). Many such couples cherish the opportunity to know the child’s diagnosis before birth, in order to relieve anxiety that the child is not affected or to prepare their minds for the birth, if affected. Depending on circumstances, such as whether or not they already have affected children and their perception of the quality of care that would be available to an affected child, the couple may decide to continue with the pregnancy and prepare their minds for the birth of the baby or choose to terminate the pregnancy bearing an affected child and try again for an unaffected child. Now that better health care of affected children is available and is increasing their life expectancies and reducing their frequency of illness, more and more couples are choosing to continue with affected pregnancies. THE DIFFERENT PND TESTS AVAILABLE The 3 possible procedures are (i) chorionic villus sampling, (ii) amniocentesis and (iii) fetal blood sampling. The recommended procedure for inherited haemoglobin disorders is chorionic villus sampling (CVS) as it can be carried out early in pregnancy and with a high degree of safety in experienced hands. Fetal blood sampling is only possible quite late in pregnancy and requires such highly specialised equipment and expertise that it is hardly used anywhere now for PND of sickle cell disorder. Amniocentesis is less difficult but cannot be done in the first three months of pregnancy or diagnosis obtained as rapidly as with CVS and so it is less favoured. The modern method for diagnosing the sample from the baby is by DNA analysis of the haemoglobin genes. CHOOSING PRENATAL DIAGNOSIS Once you have become pregnant, we ask you to call our Centre as soon as possible for an appointment to see us. If possible, it is important for both partners to come for this first visit. The first visit is necessary for the following reasons. 1. To counsel you and fully discuss the advantages and risks of the procedure, answer your questions, and provide all the information you would require in making your decision. 2. To take a blood sample from you and your partner, if you decide to go ahead. 3. To prepare your medical records. If you choose to have prenatal diagnosis, you make a separately appointed visit for the procedure. You come to the Centre alone or with your partner. We do an ultrasound scan to see exactly where the baby and the placenta are. During the procedure to obtain the sample for diagnosis, we use the ultrasound scan throughout so that we can see exactly what is happening. You are not put to sleep and you can go home later the same day. CHORIONIC VILLUS SAMPLING CVS can be safely done early in pregnancy from about 9½ weeks after your last menstrual period. The best timing is between 10 and 14 weeks. A small amount of material is taken from the developing placenta. The placenta is where the baby is attached to the mother. It develops from the tissues of the baby, not of the mother, so it has the same genes as the baby. It is made of up of chorionic villi. Villi is plural for villus. Two routes may be taken to obtain a sample of chorionic villi; either through the abdomen (tummy) or through the cervix of the uterus via the vagina. The abdominal route is mostly taken except when the position of the placenta dictates otherwise. Most samples are therefore obtained through the abdomen and this route leads to a much lower chance of a miscarriage of the pregnancy than going through the vagina and cervix. The procedure is carried out by a specially trained and experienced obstetrician. We use an ultrasound scan all the time so that we can see what we are doing. We reach the placenta by putting a long needle through the abdomen and into the womb. We inject a local anaesthetic into the skin in order to prevent pain before we insert the needle. The local anaesthetic stings a little and there is a brief feeling of pressure when the needle is put in, but you should feel very little pain. When the ultrasound picture shows that the needle is in the right place, the obstetrician fixes a syringe to it and gently removes some chorionic villi. Alternately, the obstetrician puts a very thin forceps through the vagina and then into the womb. It is so thin that most women hardly feel it. It does not touch the baby, or the little bag of water surrounding the baby. Then the forceps are opened and closed to take a very small sample of chorionic villi from the placenta. Alternatively, the obstetrician may pass a very thin tube by the same route to the placenta. Then a syringe is attached to the outer end of the tube and a very sample of the chorionic villi is sucked out. The picture shows the route. Once the sample has been obtained, we immediately examine it under a microscope to check that it is the right sample from the placenta. If it is, we stop. If it is not the correct sample, we manipulate the tip of the tube or needle slightly to obtain the right sample. The test usually takes 10 t0 20 minutes. After the test, we invite you to lie down for about 30 minutes before you go home. It is wise to take things easy for a day or two. This means you can go about as usual, but should avoid long journeys for a few days and sexual intercourse for about 10 days after the test. If the test has been done through the vagina you may observe some blood spots for a few days. This is usually from the vagina and not the womb and is usually harmless. However, if there is a lot of blood or you have pain or fever, you should contact us immediately. HOW THE CVS IS TESTED The genes responsible for all the characteristics inherited from parents, including haemoglobin, are made of a material called DNA. All the tissues of our body contain our whole DNA pattern. In the fetus this includes the placenta. We study DNA from the chorionic villi to see if the baby’s genes for haemoglobin are normal, or if an alteration has been passed on from the parents. It takes a few days to analyse DNA, so we usually have the result in about one week, or longer, when we have to send it abroad for analysis. IS DNA TEST ACCURATE? DNA analysis is the best method for diagnosing inherited disorders. However, in every medical test there is a small possibility of an error sometimes, arising from human error but sometimes due to nature ‘playing a trick’. The chance of a mistake is very small: less than 1 in 200 (0.5%). IS CVS SAFE? There is practically no risk to the mother. The main risk is that the test could lead to a miscarriage. We do the test as expertly as possible but anything that interferes with a pregnancy can cause a miscarriage. At the moment, the risk of miscarriage
Docs/Sickle Cell Anaemia Yoruba.txt ADDED
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+ KÍ NI OHUN TÍ ÀWỌN ÈTÒ TÓ DÁ LÓRÍ ÀRÙN SẸ̀JẸ̀DỌ̀LẸ LE ṢE LÁTI DOJÚKỌ ÀRÙN NÁÀ? 1. A lè ràn wọ́ n lọ́ wọ́ láti jẹ kí wọ́ n ní ẹ̀ mí-gígùn, a sì tún le dín iyé àrùn àti ìṣòrò tí wọ́ n le máa ní kù nípa ṣíṣe àyẹ̀ wò àrùn Sẹ̀ jẹ̀ dọ̀ lẹ fún gbogbo àwọn ọmọ tuntun ní kété tí wọ́ n bá ti bí wọn sílẹ̀ , tí ó bá sì jẹ́ pé a rí àrùn yìí lára wọn, kí a máa gba àwọn òbí wọn nímọ̀ ràn nípa àwọn ìtọ́ jú tó yẹ fún àwọn ọmọ wọ̀ nyìí láti ìbẹ̀ rẹ̀ pẹ́ pẹ̀ . 2. A lè dín ìrora àwọn alárùn sẹ̀ jẹ̀ dọ̀ lẹ àti ti àwọn òbí wọn kù nípa gbígbà wọ́ n nímọ̀ ràn tó kájúà lórí àwọn àrùn àjogúnbá àti pípèsè ìtọ́ jú tó kúnjú ìwọ̀ n fún gbogbo mùtúmùwà. 3. A lé járá mọ́ ìwádìí láti le wá àwọn oògùn tó le dènà tàbí mú kí àárẹ̀ tó máa ń ṣe àwọn alárùn ṣẹ̀ jẹ̀ dọ̀ lẹ dín kù 4. Ìdàsílẹ̀ àwọn ibùdó àrùn sẹ̀ jẹ̀ dọ̀ lẹ fún ìgbélárugẹ àti ìṣètò ìgbaninímọ̀ ràn, àyẹ̀ wò, ìtọ́ jú tó péye, ẹ̀ kọ́ ètò ìlera, ìwádìí àti ìkọ́ ni lórí àrùn yìí ṣe pàtàkì fún àṣeyọrí àwọn èròǹgbà (1) àti (3) tí a kọ sókè. 5. A lé rí i dájú pé ìfidípò ọ̀ pá-igi hóró ẹ̀ jẹ́ wà ní àrọ́ wọ́ tó tí owó rẹ̀ kò sì ní gara fún àwọn tó bá nífẹ̀ ẹ́ sí i. KÍ NI DÍẸ̀ LÁRA ÀWỌN ÈTÒ TÍ Ó WÀ NÍLẸ̀ FÚN ALÁRÙN ṢẸ̀JẸ̀DỌ̀LẸ? Ní agbègbè Èkó, àwọn Ilé-ìtọ́ jú Àrùn Sẹ̀ jẹ̀ dọ̀ lẹ wà ní: ‰ Ilé-ìwòsàn Ìkọ́ ni Fásitì Ìjọba Apapọ̀ tó wà ní Èkó (LUTH), ‰ Ilé-ìwòsàn Ìkọ́ ni Fásitì Ìjọba Ìpínlẹ̀ Èkó (LASUTH) ‰ Àwọn Ilé-ìwòsàn Ijọba Ìpínlẹ̀ ní Èkó àti Gbàgádà ‰ Ilé-ìwòsàn àwọn Ọmọdé ní Òpópónà Massey Ní ìlú mìíràn yàtọ̀ sí Èkó, kàn sí ilé-ìwòsàn ìkọ́ ni tàbí ilé-ìwòsàn alákànṣe ti ìjọba ìpínlẹ̀ . Àwọn ètò yìí ṣì ń gbèèrú síi ni. Fún àlàyé kíkún, kàn sí Àjọ tó ń rí sí Ìtọ́ jú Àrùn Sẹ̀ jẹ̀ dọ̀ lẹ ti Orílẹ̀ -èdè Nàìjíríà (Sickle Cell Foundation Nigeria) tàbí Àwọn Ẹgbẹ́ tó ń rí sí Àrùn Ṣẹ̀ jẹ̀ dọ̀ lẹ̀ ní àdúgbò rẹ. KÍ NI ÀWỌN ÈTÒ TÓ WÀ NÍLẸ̀ NÍ ÀJỌ TÓ Ń RÍ SÍ ÌTỌ́JÚ ÀRÙN SẸ̀JẸ̀DỌ̀LẸ TI ORÍLẸ̀-ÈDÈ NÀÌJÍRÍÀ (SICKLE CELL FOUNDATION NIGERIA)? 1. Ìmọ̀ ràn tó dá lórí ìjogún àrùn fún àwọn tí ọ̀ rọ̀ kàn àti àwọn olùtọ́ jú aláìsàn, àti ìmọ̀ ràn ìṣáájú-ìgbéyàwó fún àwọn alárùn sẹ̀ jẹ̀ dọ̀ lẹ. 2. Yàrá àyẹ̀ wò hóró ẹ̀ jẹ̀ 3. Ètò ìpààrọ̀ ẹ̀ jẹ̀ alájàáàbalẹ̀ . 4. Ètò àyẹ̀ wò fún àwọn olóyún tó jẹ́ alárùn sẹ̀ jẹ̀ dọ̀ lẹ. 5. Ìtọ́ jú ọgbẹ́ ẹsẹ̀ . 6. Ètò ìdènà àrùn rọpárọsẹ̀ fún àwọn ọmọdé tó ní àrùn sẹ̀ jẹ̀ dọ̀ lẹ (ọdún méjì sí ọdún mẹ́ rìndínlógún) KÍ NI ÀWỌN OHUN TÍ O LÈ ṢE LÁTI RAN ẸNI TÓ NÍ ÀRÙN SẸ̀JẸ̀DỌ̀LẸ LỌ́WỌ́ NÍ ÀGBÈGBÈ RẸ? 1. Pèsè owó fún ìwòsàn, ìtọ́ jú àti ìgbáyégbádùn ẹni tó ní àrùn yìí. 2. Dara pọ̀ mọ́ ẹgbẹ́ tó ń rí sí ìtọ́ jú àwọn alárùn sẹ̀ jẹ̀ dọ̀ lẹ ní agbègbè rẹ kí ọ̀ rọ̀ ẹgbẹ́ náà sì múmú láyà rẹ dáadáa. 3. Ràn wá lọ́ wọ́ láti dá Ẹgbẹ́ tó ń rí sí ìtọ́ jú àwọn alárùn sẹ̀ jẹ̀ dọ̀ lẹ ní agbègbè tàbí ní ilé-ẹ̀ kọ́ rẹ sílẹ̀ .
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+ KÍ NI ÀRÙN SẸ̀JẸ̀DỌ̀LẸ? Àrun sẹ̀ jẹ̀ dọ̀ le jẹ́ àmodi àjogúnbá inú ẹ̀ jẹ̀ tí kò ní ìwòsàn tí ó sì máa ń dín agbára hóró ẹ̀ jẹ̀ kù, ó máa ń fa ara-ríro lẹ́ ẹ́ kọ̀ ọ̀ kan àti àwọn àrun-burúkú tó máa ń ránni nígbà èwè. Kí èèyàn tó le ní àrùn yìí, ẹni náà yóò ti jogún abàmì hóró ẹ̀ jẹ̀ tó dà bíi ọ̀ kọ̀ tí wọ́ n fi ǹ ká èsò orí-igi láti ọ̀ dọ̀ àwọn òbí méjéèjì. Ní èdè kúkúrú, abàmì hóró-ẹ̀ j���̀ yìí ni a mọ̀ sí Hb S, lẹ́ yìn tí ọmọ bá jogún rẹ̀ tán, ọmọ náà yóò padà wá ní ohun tí a mọ̀ sí HbSS. Hóró ẹ̀ jẹ̀ tí ó dá pé ni a mọ̀ sí Hb A. KÍ NI HÓRÓ Ẹ̀JẸ̀? Hóró ẹ̀ jẹ̀ tàbí Hb, ní èdè kúkúrú, ni ohun-èlò aláwọ̀ pupa tí ó wà nínú ẹjẹ̀ . Iṣẹ́ rẹ̀ ni láti máa gbé afẹ́ fẹ́ àmísínú láti inú ẹ̀ dọ̀ -fóró lọ sí oríkèéríkèé àgọ́ ara wa. KÍ NI ÀÌTÓ-Ẹ̀JẸ̀? Àìtó-ẹ̀ jẹ̀ túmọ̀ sí kí hóró ẹ̀ jẹ̀ má tó bó ṣe yẹ kó tó nínú ara. Nípa báyìí, ẹ̀ jẹ̀ kò ní ní agbára láti le gbé afẹ́ fẹ́ àmísínú tó yẹ kó gbé. Ìyárakọṣẹ́ sílẹ̀ Hb S ni ó máa ń ṣokùnfa àìtó-ẹ̀ jẹ̀ fún àwọn tí ó ní àrùn sẹ̀ jẹ̀ dọ̀ lẹ. Okùnfà rẹ̀ kì í ṣe nítorí àìtó èròjà áyọ́ ọ̀ nù, kò sí nílò ìtọ́ jú pẹ̀ lú oògùn eléròjà áyóònù tàbí oògùn ẹ̀ jẹ̀ . Èyí le pa aláìsàn bẹ́ ẹ̀ lára. KÍ NI AFARASIN ÀRÙN SẸ̀JẸ̀DỌ̀LẸ? Afarasin àrùn sẹ̀ jẹ̀ dọ̀ lẹ máa ń wáyé lára ẹni tó jogún abala ẹ̀ yọ́ - ìran tí kò dá pé kan (tàbí èso) ti Hb S láti ara ọ̀ kan nínú àwọn òbí, tí ó sì tún jogún Hb A láti ara òbí kejì. Irú ẹni báyìí máa wá ní ohun tí a mọ̀ sí Hb AS. Àmì àrùn sẹ̀ jẹ̀ dọ̀ lẹ kò ní fojúhàn nínú irú ẹni bẹ́ ẹ̀ ṣùgbọ́ n ó jẹ́ ẹni tó le kó Hb S ran àwọn ọmọ rẹ̀ . Ìfarahàn Hb A tó dá pé nínú ẹ̀ jẹ̀ ẹni yìí máa bo agbára àti àtúbọ̀ tán Hb S mọ́ lẹ̀ , nípa báyìí, kò ní ní àrùn sẹ̀ jẹ̀ dọ̀ lẹ. Irú ẹni yìí (ẹni tí àmí àrùn sẹ̀ jẹ̀ dọ̀ lẹ farasin lára rẹ̀ ṣùgbọ́ n tó le fún ẹlòmíràn ní Hb S) máa ń ní ìlera pípé, wọn kò nílò ìtọ́ jú kankan, wọn a sì máa gbé ìgbé-ayé àlàáfíà. Ọ̀kan nínú ọmọ Orílẹ̀ -èdè Nàìjíríà mẹ́ rin (ìdá mẹ́ ẹ̀ ẹ́ dọ́ gbọ̀ n nínú ìdá ọgọ́ rùn-ún) ló ní Afarasin àrùn sẹ̀ jẹ̀ dọ̀ lẹ– Hb AS. Láti mọ̀ bóyá ènìyàn ní àrùn sẹ̀ jẹ̀ dọ̀ lẹ (SS), tàbí bóyá ẹ̀ nìyàn kò ní (AA), ó ṣe pàtàkì láti ṣe àyẹ̀ wò ẹ̀ jẹ̀ ní yàrá àyẹ̀ wo. Ṅ̀JẸ́ ÀWỌN ABÀMÌ TÀBÍ IRÚFẸ́ HÓRÓ Ẹ̀JẸ̀ MÌÍRÀN WÀ BÍ? Bẹ́ ẹ̀ ni, wọ́ n pọ̀ pẹ̀ lú, ṣùgbọ́ n Hb S ni ó gbajúmọ̀ káàkiri àgbáyé. Hóró ẹ̀ jẹ̀ mìíràn tó tún gbajúmọ̀ tẹ̀ lé Hb S ní ilẹ̀ Áfíŕkà ni a mọ̀ sí Hb C. Apá Aríwá Orílẹ̀ -èdè Ghana àti Orílẹ̀ -èdè Burkina Faso ni ó ti kọ́ kọ́ fojúhàn. Ènìyàn kan nínú àwọn ènìyàn márùn-ùn (ìdá ogún nínú ìdá ọgọ́ rùn-ún) láàrin àwọn ara ìlú wọ̀ nyí ni ó ní ẹ̀ yọ́ -ìran C (èyí nnì ìfarasin ti hóró C). Èyí túmọ̀ sí pé wọ́ n ní hóró ẹ̀ jẹ̀ Hb AC. A tún le rí Hb C yìí ní Orílẹ̀ -èdè Nàìjíríà pàápàá júlọ̀ ní agbègbè Ìwọ̀ -oòrùn Nàìjíríà àti ní ààrín àwọn aláwọ̀ dúdú Ilẹ̀ America, Europe àti ní Caribbean. Àwọn ènìyàn kọ̀ ọ̀ kan ní àwọn agbègbè tí a ti mẹ́ nubà wọ̀ nyí máa ń ní abàmì hóró ẹ̀ jẹ̀ Hb SC nípa pé wọ́ n jogún Hb S láti ara òbí kìíní tí wọ́ n sì jogún Hb C láti ara òbí kejì. Àwọn ìfarahàn àmì hóró Hb SC nínú ẹ̀ jẹ̀ jọ ti Hb SS ṣùgbọ́ n kì í jà lóòrèkóòrè, kò sì burú bíi ti Hb SS. Àrùn ṣẹ̀ jẹ̀ dọ̀ lẹ náà ni orúkọ tí à ń lò fún ẹni tó ní yàlá Hb SS, Hb SC tàbí Hb S–betathalassaemia (SBthal). BÁWO NI ÀRÙN SẸ̀JẸ̀DỌ̀LẸ ṢE GBILẸ̀ TÓ NÍ ORÍLẸ̀-ÈDÈ NÀÌJÍRÍÀ? Àrùn yìí pọ̀ gidi. Nǹkan bíi ìdá méjì nínú ìdá ọmọ ọgọ́ rùn-ún tí àwọn obí Orílẹ̀ -èdè Nàìjíríà bí ni ó le ní Hb SS. Pípọ̀ tí a pọ̀ ní orílẹ̀ -èdè yìí ni ó fà á tí ó fi jẹ́ pé àwa ni a ní àwọn tó ní àrùn yìí jùlọ ní gbogbo àgbáńlá ayé. KÍ NI ÀWỌN ÀMÌ TÍ Ó MÁA Ń FI HÀN PÉ ÈNÌYÀN NÍ ÀRÙN SẸ̀JẸ̀DỌ̀LẸ? Ní ọpọ̀ ìgbà, àmì kankan kì í fojú hàn ní àárín oṣù mẹ́ fà tí ọmọ bá kọ́ kọ́ délé ayé. Lẹ́ yìn ìgbà yìí, ojú yóò bẹ̀ rẹ̀ sí ní pọ́ n rẹ́ súrẹ́ sú (Ibà apọ́ njú). Egungun-ríro, inú-rírun tàbi inú-wíwú. Ọwọ́ -wíwú, ọwọ́ -ríro àti ẹsẹ̀ -ríro láàrin àwọn ọmọdé. Ibà, àyà-dídùn àti mímí gbúlegbúle. Àwọn ìṣẹ̀ lẹ̀ ìrora a máa wọ́ pọ̀ nígbà òjò tàbí nígbà otútù. Bí ọjọ́ -orí ọmọ náà ṣe ń dàgbà, ó le kéré sí ọjọ́ -orí rẹ̀ , àwọn àmì tí ó ń fi hàn pé ọmọ ti bàlágà kò ní tètè fojúhàn, fún ọmọbìnrin, ó le má tètè bẹ̀ rẹ̀ nǹkan-oṣù. Lẹ́ yìnọ̀ rẹyìn, ìdàgbàsókè pípé nínú àgọ́ -ara máa wáyé nígbà tí wọ́ n bá ń gbẹ́ nu lé ogún ọdún. Oúnjẹ aṣaralóore máa túbọ̀ ran ìdàgbàsókẹ̀ lọ́ wọ́ . Láàrin àwọn ọ̀ dọ́ tí wọn kò tíì pé ogún ọdún ṣùgbọ́ n tí wọ́ n ti kọjá ọdún méjìlá, àti láàrin àwọn àgbàlagbà, ìrọra kì í sábà wọ́ pọ̀ ṣùgbọ́ n ọgbẹ́ ẹsẹ̀ ní àyíká kókósẹ̀ le máa wáyé. Àwọn aláìsàn sẹ̀ jẹ̀ dọ̀ lẹ wọ̀ nyí kì í le kópa nínú àwọn eré ìdárayá tó nílò ipá àti agbára nítorí àìtó-ẹ̀ jẹ̀ tó ń bá wọn fínra. Ẹ dákun, ó yẹ kí ẹ mọ̀ pé kì í ṣe gbogbo àwọn alárùn yìí ni wọ́ n máa ń ní àwọn àmì tí a kà sókè wọ̀ nyí. Àwọn àmì àrùn yìí kì í tiẹ̀ fojúhàn lára àwọn mìíràn títí dìgbà tí wọ́ n fi máa gbẹ́ nu lé ogún ọdún tàbí títí ìgbà tí irú ẹni bẹ́ ẹ̀ bá lọ ṣe àyẹ̀ wò ẹ̀ jẹ̀ . Ara àwọn mìíràn a máa ya gágá fún ìgbà pípẹ́ wọn kì í sí nífẹ́ ẹ̀ sí kí àwọn ènìyàn máa káàánú àwọn. Wọ́ n máa n fi hàn pé àwọn dá pé àti pé àwọn ní ọpọlọ-pípé bí àwọn tí kò ní àrùn yìí. BÁWO NI ÀWỌN ALÁÌSÀN SẸ̀JẸ̀DỌ̀LẸ ṢE RÍ AYÉ SÍ? Ìwòsàn pípé àti ìtọ́ jú àyíká, ìmọ́ tótó ara, ìdènà àrùn-kíkó, jíjẹ àwọn oúnjẹ́ aṣaralóore àti wíwá ìwòsàn fún àwọn àrùn ní kíákíá, tí mú kó ṣe é ṣe fún ọ̀ pọ̀ àwọn ọmọ tó ní àrùn sẹ̀ jẹ̀ dọ̀ lẹ láti ye àrùn náà pẹ̀ lú ẹ̀ mi-gígùn tí wọ́ n sì ń gbé ìgbé-ayé ìdùnnú. Nígbà tí ọmọ bá wà ní ilé-ẹ̀ kọ́ , igbé-ayé tí kò ní ìṣòro ni yóó máa gbé. Ọpọlọ rẹ̀ máa jí pépé, yóó sì máa ta yọ nínú ẹ̀ kọ́ rẹ̀ . Ní ọ̀ pọ̀ ìgbà, wọ́ n máa ń yege nínú iṣẹ́ wọ́ n ní ọ̀ nà tí wọ́ n bá yàn láàyò àti ní àwọn ọ̀ nà mìíran. Ó máa jẹ́ ohun ọlọ́ gbọn láti yàgò fún àwọn iṣẹ́ tó nílò ipá àti agbára ní ṣíṣe. Wọ́ n le ní àfojúsùn ìgbéyàwọ́ -ṣíṣe àti ọmọ-bíbí. Ohun tó dára jù ni fún àwọn tó bá jẹ́ obìnrin láti dín ewu tó rọ̀ mọ́ ọmọ-bíbí kù nípa pé kí wọ́ n má bí ju ọmọ méjì lọ. Kí wọ́ n kàn sí dókítà wọn tàbi Àjọ tó ń rí sí Ìtọ́ jú Àrùn Sẹ̀ jẹ̀ dọ̀ lẹ ti Orílẹ̀ -èdè Nàìjíríà (Sickle Cell Foundation Nigeria) fún àlàyé nípa níní àǹfàní sí àwọn ètò ìfètò-sọ́ mọ-bíbí. KÍ NI ÀWỌN ÌṢÒRO KỌ̀Ọ̀KAN TÓ LE TI ÌDÍ ÀRÙN YÌÍ JÁDE? Bí ó ti lẹ̀ jẹ́ pé wọ́ n le gbé ìgbé-ayé gidi pẹ̀ lú ẹ̀ mí-gígun, àwọn ìṣòro bíi egungun-ríro, àrùn rọpárọsẹ̀ , kíkó-àrùn láti ibòmíràn, àìtó-ẹ̀ jẹ̀ pàápàá jùlọ lára ọmọ-ọwọ́ tàbí lára àwọn èwe, le já sí ikú àìtọ́ jọ́ tí wọn ò bá dènà tàbí kí wọ́ n tètè wá ìwọ̀ sàn to yẹ sí wọn. KÍ NI ÀWỌN OHUN TÍ A LE ṢE LÁTI DÈNÀ ÀWỌN ÀÌSÀN TÓ MÁA Ń ṢENI NÍPA ÀRÙN ṢẸ̀JẸ̀DỌ̀LẸ? 1. Rí i dájú pé àyíká rẹ́ mọ́ tónítóní nígbà gbogbo 2. Rí i dájú pé o ní ìmọ́ tótó pípé fún ara rẹ 3. Dáàbò bo ara rẹ lọ́ wọ́ ẹ̀ fọn 4. Máa lo àwọn oògùn tó máa ń dènà àrùn ibà gẹ́ gẹ́ bí dókíta bá ṣe ṣo pé kí o máa lò wọ́ n. 5. Dáàbò bo ara rẹ lọ́ wọ́ àwọn àìsàn tí a lè kó láti ibòmíràn
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+ WHAT CAN WE DO TO PREVENT CRISES? 1. Maintain a clean environment always 2. Maintain good personal hygiene 3. Protect yourself from mosquito bites 4. Take a malaria prevention medicine as prescribed by the doctor 5. Protect yourself from infections 6. Avoid extreme cold and extreme heat and from exposure to cold weather and rain. 7. Avoid strenuous physical exertion 8. Follow your doctor’s advice 9. Seek prompt treatment whenever you are sick 10. Drink plenty of water/fluids 11. Take one folic acid tablet daily 12. Attend the nearest sickle cell clinic for proper care 13. Join a Sickle Cell Club and attend meetings regularly WHAT CAN SICKLE CELL PROGRAMMES DO TO CONTROL THE DISORDER? 1. We can help prolong their lives and drastically reduce the number of their illnesses and crises by testing all new born children for sickle cell anaemia and if positive, counselling the parents about proper care right from the start. 2. We can reduce the suffering of affected people and their parents by making professional genetic counselling and good medical care available to all. 3. We can intensify research to find drugs that will prevent or make crises less severe. 4. The establishment of sickle cell centres to promote and co-ordinate counselling, diagnosis, proper care, health education, training and research into the disorder is essential for achieving objectives (1) to (3) above. 5. We can ensure that Stem cell transplantation is accessible and affordable to interested persons. WHAT ARE SOME OF THE SERVICES AVAILABLE TO AFFECTED PEOPLE? In Lagos area Sickle Cell Clinics are available at: ‰ Lagos University Teaching Hospital (LUTH), ‰ Lagos State University Teaching Hospital (LASUTH) ‰ General Hospitals Lagos and Gbagada ‰ Massey Street Children’s Hospital Outside Lagos, get in touch with the nearest teaching or State specialist hospital. These services are expanding. For more information, contact Sickle Cell Foundation Nigeria or Sickle Cell Clubs in your area. WHAT ARE THE SERVICES AVAILABLE AT THE SICKLE CELL FOUNDATION NIGERIA? 1. Genetic counseling for the affected persons and caregivers and pre-marital counseling for sickle cell carriers. 2. Haemoglobin laboratory for blood tests 3. Automated Exchange Blood Transfusion service 4. Prenatal diagnosis for sickle cell disorder 5. Leg Ulcer treatment 6. Stroke prevention programme for children with Sickle cell disorder (2 to 16 years old) WHAT CONTRIBUTION CAN YOU MAKE TO HELP PERSONS WITH SICKLE CELL DISORDER IN YOUR COMMUNITY? 1. Make donations for the treatment, care and welfare of affected persons 2. Join your local Sickle Cell Club and become an active member 3. Help start a Sickle Cell Club in your locality or in your school
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+ WHAT IS SICKLE CELL ANAEMIA? Sickle cell anaemia is a lifelong hereditary disorder of the blood which results in anaemia, occasional body pains and some serious infections in childhood. To have it, one has to inherit the unusual variant sickle haemoglobin, known for short, as Hb S, from each parent and thus end up having HbSS. The usual or normal haemoglobin (Hb) is known as Hb A. WHAT IS HAEMOGLOBIN? Haemoglobin or Hb for short, is the red substance in the blood. It is responsible for carrying oxygen from our lungs to different parts of our bodies. WHAT IS ANAEMIA? Anaemia means a shortage of haemoglobin. The blood is therefore not able to carry as much oxygen as it should. The anaemia of sickle cell is due to the rapid breakdown of Hb S. It is not due to lack of iron and does not require treatment with iron-containing drugs or blood tonics. These can be harmful to the patient. WHAT IS SICKLE CELL TRAIT? Sickle cell trait is said to occur in a person who inherits only a single dose of the sickling gene (or seed) for Hb S from one parent and for Hb A from the other parent. This individual thus possesses the Hb AS. He does not have the symptoms of sickle cell anaemia but he is a carrier who can transmit the Hb S to his or her children. The presence of the normal Hb A suppresses the adverse effects of Hb S and thus protects such persons from suffering from the symptoms of sickle cell anaemia. These carriers of the sickle cell gene ie persons with sickle cell trait), are healthy, require no treatment and live a normal life span. One in every 4 Nigerians (25%) has the sickle cell trait -Hb AS. In order to find out whether one has sickle cell anaemia (SS), the trait (AS), or is free (AA), a laboratory blood test is necessary. ARE THERE OTHER UNUSUAL OR VARIANT HAEMOGLOBINS? Yes, very many, but Hb S is the most common of these all over the world. The second commonest variant haemoglobin in Africa is termed Hb C. It originated from Northern Ghana and Burkina Faso where about 1 in 5 of the population (20%) are carriers of the C gene (ie C trait); i.e. they have Hb AC. Hb C is also found in Nigeria especially in Western Nigeria and among the black populations of America, Europe and the Caribbean. Some persons in these areas do therefore have HbSC disorder from inheriting Hb S from one parent and Hb C from the other. The symptoms of Hb SC are similar but much less frequent and much less severe than those of Hb SS. The term Sickle Cell Disorder is generally used to refer to a person with Hb SS or Hb SC or Hb S – betathalassaemia (SBthal). HOW COMMON IS SICKLE CELL ANAEMIA IN NIGERIA? Very common. About two of every hundred children born to Nigerian parents would have Hb SS. Our large population ensures that we have the largest number in the whole world. WHAT ARE THE SIGNS OF SICKLE CELL ANAEMIA? Usually, none in the first 6 months of life. After that, pale skin or eyes due to anaemia. Yellow eyes (Jaundice). Bone or abdominal pains or swelling. Swelling and pain of the hands and feet in young children.Fever, chest infections and rapid breathing. Pain crises are commoner in the rainy season or in cold weather. As the child approaches the age of puberty, he may remain small for his age and signs of puberty and menstruation may be delayed for some years. Eventually, normal development occurs by the time they reach the end of their teenage years. Good nutrition will promote faster growth. In teenagers and adults, the pain crises occur less frequently but leg ulcers around the ankles may occur. They (SS) cannot cope well with strenuous competitive sports because of their anaemia. Please note that not everyone develops all the above symptoms. Some are not even suspected of having the condition until they have reached their late teens or until a blood test is performed. Many remain well for very long periods and hate to be treated as objects of pity. They are as intelligent and capable as anyone else. WHAT IS THE OUTLOOK FOR PEOPLE WITH SICKLE CELL ANAEMIA? Good medical care and environmental sanitation, personal cleanliness, prevention of infection, balanced nutrition and prompt treatment of infections have made it possible for many children to survive and lead long and happy lives. At school age the child begins a less dangerous period of life. He is of normal intelligence and can perform very well at school. They often succeed with careers in the professions and other areas. It is wise to avoid careers requiring strenuous physical activities. They can look forward to marriage and child-bearing. It is best for females to reduce the risk of child-bearing by limiting their children to no more than two. They should consult their doctors or contact Sickle Cell Foundation Nigeria for information about access to birth control. WHAT ARE SOME OF THE COMPLICATIONS ARISING FROM THE DISORDER? Though they can live long useful lives into old age, some complications such as bone pains, stroke, infection, severe anaemia especially in infancy and childhood often arise and can lead to premature death if not prevented or promptly and correctly treated.
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+ ABOUT SICKLE CELL DISEASE
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+ Thousands of years ago, sickle cell emerged as a genetic mutation to protect populations in tropical regions from severe forms of malaria. While inheriting this genetic trait from one parent offers some evolutionary protection against malaria, inheriting the genetic trait from both parents risks disease. Sickle cell disease (SCD) is an inherited, lifelong blood disorder that causes individuals to produce abnormal hemoglobin, causing their red blood cells to become rigid and sickle-shaped. These sickled cells have a shortened lifespan, resulting in a constant shortage of red blood cells. When these sickled cells travel through the blood, they often get stuck in the smaller blood vessels, blocking other oxygen-rich red blood cells from freely flowing throughout the body. This leads to complications ranging in severity, including severe pain, acute chest syndrome (a condition that lowers the level of oxygen in the blood), stroke, organ damage, and even premature death.
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+ Sickle cell disorder is by far the commonest inherited disorder in the world and three quarters of cases occur in Africa. In Nigeria, where it affects two out of every hundred children born, it causes suffering for innumerable patients and their families. However, despite its importance, until now there has been no dedicated sickle cell center in Africa. This is partly because the very scale of the problem makes it difficult to see how to start.
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+ Sickle cell disorder is an inherited haemoglobin disorder comprising sickle cell anaemia (Hb SS) and some less prevalent but related conditions such as sickle haemoglobin C disorder (Hb SC) and sickle beta thalassaemia (Hb SBthal). The genes for these conditions arose by mutation but proliferated in areas where there is or there was a high incidence of falciparium malaria, the most lethal strain of malaria known to man.
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+ The individual with the sickle cell trait (Hb AS) is protected from sickle cell symptoms and complications by the presence as well of Hb A. He is largely protected from death caused by malaria, especially in early childhood, by the presence of Hb S.
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+ Thus he enjoys the best of both worlds and is naturally better adapted to the malaria infested environment than the individual with the normal Hb AA who is more liable to childhood death from malaria, or the sickle cell affected one with Hb SS in whom malaria easily induces a lethal degree of anaemia.
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+ Hence, more persons with Hb AS survived to reproduce and pass the Hb S gene on to their progeny. Thus, the prevalence of sickle trait (Hb AS) increased in each successive generation. Falciparum malaria therefore encouraged the expansion of the gene within the population by conferring on healthy carriers a selective survival advantage. Conversely, when populations emigrated to a non-endemic malaria zone the incidence of the Hb S gradually waned over many generations owing to the absence of the selective survival and reproductive advantage. This phenomenon is demonstrated in South Africa, in the West Indies and in the United States of America.
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+ While the Bantus in African countries north of the Zambesi River have a significant incidence of sickle cell disorder, the Bantus in temperate malaria-free South Africa have no significant incidence of SCD.
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+ They are reputed to have migrated to South Africa about 2,000 years ago and the prevalence of the sickle trait is less than 0.5% in their population. In African-Americans and African-West Indians ‘Who migrated relatively recently, only 300-400 years ago, the frequency of the sickle trait among them is 8% and 10% respectively.