I-Sickle cell anemia ibizwa nangokuthi i-sickle cell anemia, i-sickle cell anemia, i-sickle cell anemia, i-hemoglobin S noma, ngesiNgisi, isifo se-sickle cell. Lolu hlobo lwe-anemia engapheli kanye nofuzo lubonakala, phakathi kwezinye izinto, ngokuhlaselwa okubuhlungu kakhulu. Njengoba isabalele, ihlasela ikakhulukazi abantu abanombala omnyama: ukutholakala kwayo ku-0% kuya ku-40% e-Afrika kanye no-10% kubantu base-Afrika baseMelika. Njengamanje e-United States, ingane eyodwa kwezingama-1 zase-Afrika yaseMelika inesifo se-sickle cell; ukwanda koku-500 koku-1 kuye kwayi-1 kubantwana base-Hispanic. Abantu baseWest Indies naseNingizimu Melika nabo basengozini enkulu.

Lesi sifo siwufuzo: sihlotshaniswa nokuba khona kwezakhi zofuzo ze-hemoglobin engavamile ezikhiqiza iphrotheni ye-hemoglobin engasebenzi, ebizwa ngokuthi i-hemoglobin S. Lokhu kuhlanekezela amangqamuzana abomvu egazi futhi kuwenze abukeke njengocwecwe noma ucezu. i-scythe (ngakho igama layo elimise okwesikela), ngaphezu kokubangela ukuba bafe ngaphambi kwesikhathi. Lamangqamuzana abomvu egazi akhubazekile abizwa nangokuthi ama-sickle cell. Lokhu kuguqulwa kwenza amangqamuzana egazi abomvu abe ntekenteke. Lezi zizibhubhisa ngokushesha. Ngaphezu kwalokho, ukuma kwabo okungajwayelekile kwenza ukudlula kwabo emithanjeni yegazi emincane kube nzima kakhulu. Ngezinye izikhathi zivimba igazi ezithweni ezithile futhi zidale izingozi zokujikeleza kwegazi.

Ukubhujiswa okusheshayo kwamangqamuzana abomvu egazi ekugcineni kuthuthukela ku-anemia ye-hemolytic - okungukuthi, i-anemia ebangelwa ukubhujiswa okusheshayo okungajwayelekile kwamangqamuzana abomvu egazi. Ngaphezu kwalokho, ukuma okungavamile kwalokhu kungadala izithiyo kuma-capillaries futhi kubangele izinkinga ezihlukahlukene ezihlobene nokungahambi kahle kwegazi. Ngenhlanhla, iziguli ze-sickle cell - abantu abanalesi sifo - banganqanda izinkinga kanye nokuquleka ngezinga elithile. Baphinde baphile isikhathi eside kunakuqala (Indlela yesifo).

Izimbangela

Ukuba khona kwe-hemoglobin S kuchazwa ukukhubazeka okuhlobene nesakhi sofuzo esikhiqiza i-hemoglobin. Eminyakeni eyizinkulungwane ezimbalwa edlule, ngesikhathi umalaleveva ubulala abantu abaningi, abantu abanalesi sici sofuzo babenethuba elingcono lokusinda ngoba i- hemoglobin S ivimbela igciwane likamalaleveva ukuba lingangeni emangqamuzaneni abomvu egazi. Njengoba lesi sici sofuzo sasiyinzuzo ekusindeni kwezinhlobo zezilwane, ngakho-ke sagcinwa. Namuhla, sekuphenduke ukukhubazeka manje njengoba umalaleveva welashwe kahle.

Ukuze ingane ibe ne-sickle cell anemia, bobabili abazali kumelwe badlulisele kubo isakhi sofuzo se-hemoglobin S. Uma umzali oyedwa kuphela edlulisela isakhi sofuzo kubo, ingane nayo izophatha isakhi sofuzo esinephutha. , kodwa ngeke aphathwe yilesi sifo. Ngakolunye uhlangothi, wayengadlulisela isakhi sofuzo ngokushintshana.

Inkambo yesifo

Lesi sifo sivela phakathi nezinyanga eziyisithupha futhi sizibonakalisa ngendlela ehlukile ukusuka kwesinye isiguli kuya kwesinye. Abanye banezimpawu ezithambile kuphela futhi banokuhlaselwa okungaphansi kokukodwa ngonyaka, lapho izimpawu ziba zimbi kakhulu. Esikhathini esidlule, lesi sifo sasivame ukubulala izingane ezineminyaka engaphansi kwemihlanu. Nakuba izinga lokufa lihlala liphezulu kuleli qembu lobudala, ukwelashwa manje kuvumela iziguli ukuthi ziphile okungenani zibe abantu abadala.

Izinkinga

Baningi. Phakathi kweziyinhloko, sithola lokhu:

• Ukuba sengozini kwezifo. Ukutheleleka ngamagciwane kuyimbangela enkulu yezinkinga ezinganeni ezine-sickle cell anemia. Yingakho ukwelashwa ngama-antibiotic ngokuvamile benikezwa. Ama-sickle cell alimaza ubende, odlala indima ebalulekile ekulawuleni amagciwane. Ikakhulukazi, izifo ze-pneumococcal, ezivame kakhulu futhi eziyingozi, kufanele zesatshwe. Intsha kanye nabantu abadala nabo kufanele bazivikele ezifweni.
• Ukukhula nokuthomba kubambezelekile, umthethosisekelo ubuthakathaka kubantu abadala. Lesi simo sibangelwa ukuntuleka kwamangqamuzana egazi abomvu.
• Izinkinga ezibuhlungu. Ngokuvamile zivela ezithweni, esiswini, emhlane noma esifubeni, futhi ngezinye izikhathi emathanjeni. Axhumene neqiniso lokuthi ama-sickle cell avimba ukugeleza kwegazi kuma-capillaries. Kuye ngecala, zingahlala kusukela emahoreni ambalwa kuya emavikini ambalwa.
• Iziphazamiso ezibonakalayo. Lapho igazi lijikeleza kabi emithanjeni emincane ezungeza amehlo, lilimaza i-retina futhi ngenxa yalokho lingabangela ubumpumputhe.
• Amatshe enyongo. Ukubhujiswa okusheshayo kwama-sikela cell kukhipha into ehambisana ne-jaundice, i-bilirubin. Kodwa-ke, uma izinga le-bilirubin likhuphuka kakhulu, amatshe enyongo angakheka. Ngaphezu kwalokho, i-jaundice ingenye yezimpawu ezihambisana nalolu hlobo lwe-anemia.
• I-Edema yezandla nezinyawo noma i-hand-foot syndrome. Nalapha futhi, lokhu kuwumphumela wokuvinjelwa kokujikeleza kwegazi okubangelwa amangqamuzana abomvu egazi angavamile. Ngokuvamile kuwuphawu lokuqala lokugula ezinsaneni futhi ezimweni eziningi kuhlotshaniswa nokuhlasela kwemfiva nobuhlungu.
• Izilonda emilenzeni. Njengoba igazi lijikeleza kabi esikhumbeni, isikhumba asikwazi ukuthola izakhi ezidingekayo. Omunye ngemva komunye, amangqamuzana esikhumba ayafa bese kuvela amanxeba avulekile.
• I-Priapism. Lokhu kuwukuqhanyelwa okubuhlungu nokuthatha isikhathi eside okuchazwa ngokuthi igazi liqoqana epipini lingakwazi ukuphindela emuva ngenxa yama-sikela cell. Lokhu kuqhanyelwa isikhathi eside kugcina kulimaze izicubu zomthondo kuholele ekutheni uphelelwe amandla.
• I-Acute chest syndrome (i-acute chest syndrome). Ukubonakaliswa kwayo kungokulandelayo: umkhuhlane, ukukhwehlela, i-expectoration, ubuhlungu besifuba, ubunzima bokuphefumula (i-dyspnea), ukuntuleka komoya-mpilo (hypoxemia). Lesi sifo sibangelwa ukutheleleka kwamaphaphu noma amaseli e-sickle cell avaleleke emaphashini. Kubeka impilo yesiguli engozini kakhulu futhi kufanele belashwe ngokushesha.
• Izilonda eziphilayo. Ukuntuleka komoyampilo okungapheli kulimaza izinzwa kanye nezitho ezinjengezinso, isibindi noma ubende. Lolu hlobo lwenkinga kwesinye isikhathi ludala ukufa.
• Isibungu. Ngokuvimba ukujikeleza kwegazi ebuchosheni, ama-sickle cell angabangela isifo sohlangothi. Cishe u-10% wezingane ezinalesi sifo zike zaphathwa yiso.