Sickle cell disease

**Sickle cell disease** (**SCD**), dem sanso simply call **sickle cell**, be a group of inherited haemoglobin-related blood disorders. De most common type be known as **sickle cell anemia**. Sickle cell anemia dey result in an abnormality insyd de oxygen-carrying protein haemoglobin dem find insyd red blood cells. Dis dey lead to de red blood cells adopting an abnormal sickle-like shape under certain circumstances. Plus dis shape, dem be unable to deform as dem pass thru capillaries, wey dey cause blockages.

Problems insyd sickle cell disease typically dey begin around 5 to 6 months of age. Chaw health problems fi develop, such as attacks of pain (dem know as a **sickle cell crisis**) insyd joints, anemia, swelling insyd de hands den feet, bacterial infections, dizziness den stroke. De probability of severe symptoms, wey dey include long-term pain, dey increase plus age. Widout treatment, people plus sickle cell disease rarely dey reach adulthood, buh plus good healthcare, median life expectancy be between 58 den 66 years. All of de major organs be affected by sickle cell disease. De liver, heart, kidneys, lungs, gallbladder, eyes, bones, den joints fi be damaged from de abnormal functions of de sickle cells den dema inability to effectively flow thru de small blood vessels.

Sickle cell disease dey occur wen a person inherit two abnormal copies of de β-globin gene wey dey make haemoglobin, one from each parent. De abnormal gene dey generate haemoglobin S (HbS) wich dey change de properties of red blood cells. A sickle cell crisis dey occur wen red blood cells switch from de normal saucer-like shape to a sickle-like shape wich fi obstruct small blood vessels; an attack fi be set off by temperature changes, stress, dehydration, den high altitude. A person plus a single abnormal gene usually no dey get symptoms wey na dem say e get sickle cell trait, dese people sanso be referred to as carriers. Diagnosis be by a blood test, den sam countries dey test all babies at birth for de disease. Diagnosis of de unborn foetus sanso be possible during pregnancy.

De care of people plus sickle cell disease fi include infection prevention plus vaccination den antibiotics, high fluid intake, folic acid supplementation, den pain medication. Oda measures fi include blood transfusion den de medication hydroxycarbamide (hydroxyurea). Insyd 2023, na dem approve new gene therapies wey dey involve de genetic modification den replacement of blood forming stem cells insyd de bone marrow.

, sickle cell disease be estimated to affect about 7.7 million people worldwide, directly dey cause an estimated 34,000 annual deaths den a contributory factor to a further 376,000 deaths. About 80% of sickle cell disease cases be believed to occur insyd sub-Saharan Africa. E sanso dey occur to a lesser degree among people insyd parts of India, Southern Europe, West Asia, North Africa den among people of African origin (sub-Saharan) wey dey live insyd oda parts of de world. Na dem first describe de condition insyd de medical literature by American physician James B. Herrick insyd 1910. Insyd 1949, na ein genetic transmission be determined by E. A. Beet den J. V. Neel. Insyd 1954, na dem establish say carriers of de abnormal gene be protected to sam degree against malaria, wich dey account for ein persistence insyd populations dem threaten by malaria.

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