Koj puas paub txog Sickle Cell Anemia? Cia peb tham txog nws!

Koj puas tau hnov ​​txog lub npe 'Sickle Cell Anemia'? Tej zaum lub npe no suab txawv me ntsis, lossis txawm tias ntshai me ntsis. Tab sis nws yog ib qho mob caj ces uas cuam tshuam nrog peb cov ntshav. Hais yooj yim, qhov tshwm sim hauv qhov no yog tias peb cov qe ntshav liab, uas yog, cov qe ntshav liab hauv cov ntshav, hloov pauv hauv cov duab. Zoo li ib tug sickle (yog vim li cas nws thiaj hu ua 'sickle'). Qhov kev hloov pauv no tuaj yeem ua rau muaj ntau yam teeb meem kev noj qab haus huv. Yog li, peb puas yuav tham txog qhov no kom ntxaws me ntsis hnub no?

Sickle Cell Anemia yog dab tsi? Muaj dab tsi tshwm sim tiag?

Kab mob ntshav liab hu ua sickle cell anemia yog hom kab mob hnyav tshaj plaws ntawm pawg loj hu ua 'Sickle Cell Disease'. Qhov no yog kab mob ntshav uas muaj txij thaum yug los, uas yog, kab mob ntshav uas los ntawm noob caj noob ces . Qhov laj thawj tseem ceeb rau qhov no yog kev hloov pauv ntawm caj ces. Vim yog qhov kev hloov pauv ntawm caj ces no, cov qe ntshav liab uas tsim tawm hauv peb lub cev feem ntau tsis yog puag ncig, tab sis lawv khoov thiab ncab zoo li ib tug sickle (hauv cov duab ntawm tsab ntawv C). Cov kws kho mob kuj hu cov no ua "sickle cells".

Tam sim no saib seb, peb cov qe ntshav liab ib txwm muaj puag ncig thiab yoog tau, yog li lawv tuaj yeem yooj yim mus los ntawm peb cov hlab ntsha mos mos thiab nqa cov pa oxygen mus rau txhua qhov ntawm lub cev, txhua lub cev, thiab txhua cov nqaij. Tab sis cov qe ntshav liab zoo li daim hlau no tawv thiab nplaum . Yog li lawv tsis yooj yim mus los ntawm cov hlab ntsha, tab sis feem ntau daig rau hauv qhov chaw. Zoo li kev sib tsoo ntawm txoj kev. Lwm yam yog, cov qe ntshav 'daim hlau' no tawg thiab tuag sai dua li cov qe ntshav liab ib txwm muaj. Vim yog txhua yam no, tus lej ntawm cov qe ntshav liab noj qab haus huv hauv koj lub cev txo qis, ua rau muaj ntshav tsis txaus. Ntawd yog, tsis muaj ntshav.

Yav dhau los, cov menyuam mos uas yug los muaj tus kab mob no muaj feem tsawg heev uas yuav muaj sia nyob mus txog thaum laus. Txawm li cas los xij, nrog kev nce qib hauv kev kho mob, kev kuaj pom thaum ntxov, thiab kev kho mob tshiab , qhov no tau hloov pauv ntau heev. Muaj coob tus neeg tam sim no nyob tau 50 xyoo lossis ntau dua.

Kab mob no muaj ntau npaum li cas hauv Sri Lanka? Leej twg yuav muaj feem ntau dua?

Nws yeej nyuaj nrhiav cov ntaub ntawv txog tus kab mob no hauv Sri Lanka. Tab sis thaum koj saib lub ntiaj teb, tus kab mob no feem ntau tshwm sim rau cov neeg African, Southern European, Middle Eastern lossis Asian Indian. Qhov ntawd txhais tau tias muaj qee qhov kev sib txuas ntawm caj ces.

Cov tsos mob ntawm tus kab mob sickle cell anemia yog dab tsi?

Cov tsos mob ntawm tus kab mob no yuav txawv ntawm ib tus neeg mus rau ib tus neeg, tab sis muaj qee cov tsos mob uas pom ntau:

• Qaug zog los ntawm kev tsis muaj ntshav txaus: Thaum tus mob no tshwm sim rau cov menyuam yaus, lawv yuav quaj ntau zaus, xav tias tsis zoo, thiab tsis kam pub niam mis.
• Cov kab mob sib kis ntau zaus:Kab mob no ua rau peb lub qhov ntswg puas tsuaj. Lub qhov ntswg yog ib feem tseem ceeb ntawm peb lub cev tiv thaiv kab mob. Yog li ntawd, thaum nws tsis muaj zog, muaj feem ntau dua los ntawm kev muaj kab mob thiab kev kis kab mob. Txawm li cas los xij, ua tsaug rau cov tshuaj tua kab mob thiab cov tshuaj tiv thaiv kab mob uas muaj tam sim no, qhov kev pheej hmoo no tau raug txo qis heev.
• Mob: Qhov no yog ib qho ntawm cov tsos mob feem ntau thiab tsis zoo ntawm tus kab mob no. Cov nqaij ntawm lub cev tsis tau txais oxygen txaus, uas ua rau cov nqaij puas tsuaj. Qhov kev puas tsuaj no yog qhov ua rau mob. Cov chaw mob feem ntau yog caj npab, ceg, hauv siab, thiab nraub qaum. Qee zaum nws tuaj yeem pib ua qhov mob me ntsis thiab maj mam nce ntxiv, lossis nws tuaj yeem ua rau mob tsis tuaj yeem ua tau.
• Mob o ntawm tes thiab taw: Qhov no yog ib qho ntawm thawj cov cim qhia ntawm tus menyuam mos uas muaj kab mob ntshav liab hu ua sickle cell anemia. Qhov o no tshwm sim vim tias cov qe ntshav liab zoo li sickle cell tau daig rau hauv cov hlab ntsha me me hauv tus menyuam txhais tes thiab taw, ua rau cov ntshav tsis ntws.
• Qhov muag thiab daim tawv nqaij daj vim yog mob jaundice: Peb lub siab yog lub luag haujlwm lim cov qe ntshav liab. Hauv cov qe ntshav dawb sickle cell anemia, ib yam khoom hu ua bilirubin, uas raug tso tawm los ntawm cov qe ntshav dawb sickle uas tuag sai sai, sib sau ua ke hauv lub cev, ua rau mob jaundice.

Cov tsos mob no feem ntau pib tshwm sim thaum tus menyuam muaj li ntawm 6 txog 9 lub hlis . Raws li lub sijhawm dhau mus, thaum cov hlwb tsis zoo (malignant) hauv lub cev nce ntxiv, cov tsos mob yuav hloov pauv thiab hnyav dua.

Vim li cas thiaj muaj tus kab mob ntshav liab me me hu ua sickle cell anemia? Dab tsi ua rau nws tshwm sim?

Kab mob ntshav liab hu ua sickle cell anemia yog tshwm sim los ntawm kev hloov pauv ntawm cov noob caj noob ces hauv peb cov noob caj noob ces. Tshwj xeeb, muaj ib lub noob hu ua HBB gene. Lub noob no qhia peb kom ua ib qho protein hu ua beta-globin, uas yog ib feem ntawm peb cov hemoglobin. Hemoglobin, raws li koj paub, yog qhov tseem ceeb tshaj plaws uas pab peb cov qe ntshav liab nqa oxygen.

Yog li ntawd, kom koj thiaj li tau txais cov kab mob sickle cell anemia, koj yuav tsum tau txais cov noob caj noob ces uas tau hloov pauv los ntawm koj niam thiab koj txiv . Qee zaum, yog tias koj tau txais ob lub noob caj noob ces uas tau hloov pauv los ntawm ob leeg niam txiv, koj tuaj yeem tsim lwm yam kab mob sickle cell, tej zaum yuav tsis hnyav heev. Cov kws kho mob hu cov no ua 'variant genetic mutations'.

Vim muaj tus mob no, yuav muaj teeb meem dab tsi tshwm sim?

Kab mob ntshav liab sickle cell anemia tuaj yeem ua rau muaj teeb meem loj, qee zaum yuav ua rau tuag taus . Piv txwv li, cov neeg uas muaj tus kab mob no feem ntau xav tau kev kho mob ceev thiab mus pw hauv tsev kho mob, tshwj xeeb tshaj yog rau cov mob hu ua acute chest syndrome (ACS) thiab vaso-occlusive crisis (VOC).

Muaj lwm yam teeb meem:

• Kab mob raum ntev: Qhov mob no tuaj yeem tshwm sim thaum lub raum tsis tau txais oxygen txaus, ua rau cov nqaij puas tsuaj.
• Cov retinas cais tawm: Qhov no tuaj yeem tshwm sim yog tias cov qe ntshav liab sickle thaiv cov hlab ntsha hauv lub qhov muag.
• Priapism (mob erection): Ib qho mob uas tshwm sim los ntawm kev thaiv cov hlab ntsha hauv qhov chaw mos.
• Kev cais cov qe ntshav dawb hauv lub qhov zis: Yog tias cov qe ntshav dawb raug kaw hauv lub qhov zis thiab thaiv cov ntshav ntws, qhov no tuaj yeem ua rau muaj ntshav tsis txaus tam sim ntawd.
• Mob stroke: Txhua tus neeg uas muaj mob sickle cell anemia, txawm tias cov menyuam yaus ( mob stroke rau menyuam yaus), muaj feem yuav muaj mob stroke.

Mob Hlwb Hauv Siab Acute Chest Syndrome (ACS) yog dab tsi?

Qhov no yog qhov teeb meem feem ntau ntawm cov kab mob sickle cell anemia. Nws kuj yog qhov ua rau tuag thiab qhov thib ob ua rau pw hauv tsev kho mob. Qhov no tshwm sim thaum cov kab mob sickle cell sib sau ua ke thiab thaiv cov hlab ntsha hauv lub ntsws. Cov tsos mob suav nrog:

• Mob hauv siab tam sim ntawd.
• Hnoos.
• Ua npaws.
• Ua pa nyuaj.

Vaso-occlusive Crisis (VOC) yog dab tsi?

Qhov no yog thaum cov qe ntshav dawb thaiv cov hlab ntsha. Cov kws kho mob kuj hu qhov no ua "kev mob hnyav heev." Hauv VOC, qhov mob uas tsis tuaj yeem ua rau mob hauv caj npab, ceg, nraub qaum qis, thiab plab.

Qee zaum cov kws kho mob hu cov VOCs ua "mob uas tsis pom." Vim tias, feem ntau, tsuas muaj mob xwb. Tsis muaj cim qhia tias raug mob lossis lwm yam mob. Nws tsuas yog mob xwb.

Kev kho mob tseem ceeb rau qhov mob hnyav no yog tshuaj opioid painkillers. Txawm li cas los xij, qee qhov kev tshawb fawb tau qhia tias qhov kev xav tau opioid no tuaj yeem ua rau muaj kev sib cais hauv zej zog rau cov neeg uas muaj kab mob sickle cell anemia, uas tuaj yeem ua rau muaj kev nyuaj siab thiab ntxhov siab. Qhov no yog lwm qhov kev cov nyom uas yuav tsum tau ntsib thaum nyob nrog tus kab mob no.

Yuav kuaj mob li cas? (Kev kuaj mob)

Cov kws kho mob kuaj pom tias muaj tus kab mob sickle cell anemia los ntawm kev kuaj koj ua ntej. Lawv yuav kov koj lub qhov ntswg lossis siab. Lawv yuav nug txog koj cov tsos mob, tshwj xeeb tshaj yog mob hauv koj txhais tes thiab ceg, thiab txog koj lub plab. Lawv kuj yuav nug txog koj cov kab mob yav dhau los thiab seb koj puas tau muaj kab mob. Tom qab ntawd lawv yuav ua cov kev kuaj xws li:

• Kev suav cov ntshav tag nrho (CBC): Qhov no suav nrog ntau qhov kev kuaj uas saib koj cov qe ntshav liab.
• Hemoglobin electrophoresis: Kuj hu ua high-performance liquid chromatography, qhov kev kuaj no tshuaj xyuas koj cov hemoglobin kom pom thiab ntsuas hom hemoglobin tsis zoo uas ua rau muaj ntshav liab sickle cell.
• Kev kuaj mob caj ces: Koj tus kws kho mob yuav xaj cov kev kuaj no kom pom tias koj puas muaj kev hloov pauv caj ces uas ua rau muaj ntshav liab ntau.

Yuav kho qhov no li cas? Puas kho tau?

Kev kho mob rau tus kab mob sickle cell anemia nyob ntawm koj cov tsos mob thiab koj txoj kev noj qab haus huv tag nrho. Piv txwv li, yog tias koj muaj teeb meem loj xws li mob hauv siab, mob hnyav heev, lossis yog tias koj muaj mob xws li tuag tes tuag taw, koj tus kws kho mob yuav pom zoo kom hloov hlwb hlwb allogeneic stem cell. Qhov no yog tib txoj kev los kho tus kab mob sickle cell anemia kom zoo .

Lwm cov kev kho mob yog:

• Kev hloov ntshav.
• Cov tshuaj tua kab mob rau kev kis kab mob.
• Cov tshuaj uas pab txo cov tsos mob tshwj xeeb. Ib txhia ntawm lawv yog:
• Hydroxyurea: Qhov no yog tshuaj tiv thaiv kab mob qog noj ntshav. Nws siv rau cov menyuam mos hnub nyoog ntawm 6-9 lub hlis, cov menyuam yaus, thiab cov neeg laus. Nws tuaj yeem txo qhov tshwm sim ntawm cov teeb meem loj thiab txo cov tsos mob ntawm cov ntshav tsis txaus.
• Voxelotor: Qhov no tiv thaiv cov qe ntshav liab kom tsis txhob hloov mus ua cov qe ntshav liab me me. Nws yog muab rau cov menyuam yaus hnub nyoog tshaj 4 xyoos.
• Kev kho mob L-glutamine: Cov tshuaj no, muab rau cov menyuam yaus hnub nyoog tshaj 5 xyoos thiab cov neeg laus, tiv thaiv cov hlwb sickle kom tsis txhob loj hlob tsis zoo.
• Crizanlizumab-tmca: Qhov no txo ​​qhov zaus ntawm VOC/mob hnyav heev. Nws yog muab rau cov neeg hnub nyoog tshaj 16 xyoo.

Yog tias kuv muaj mob ntshav liab ntshav liab (sickle cell anemia) ces kuv lub neej yuav zoo li cas?

Rau ntau tus neeg, tus kab mob ntshav liab sickle cell anemia yog ib yam kab mob uas mob ntev mus tas li . Qhov no txhais tau tias koj yuav xav tau kev kho mob thiab kev txhawb nqa thoob plaws koj lub neej. Cov tsos mob yuav tsis hnyav rau qee tus, nruab nrab rau lwm tus, thiab hnyav txaus kom ua rau lwm tus muaj kev phom sij rau lub neej. Txhua tus neeg qhov xwm txheej txawv. Yog li ntawd, nrog koj tus kws kho mob tham txog yam yuav tshwm sim. Nws paub koj txoj kev noj qab haus huv zoo tshaj plaws thiab tus kab mob yuav cuam tshuam li cas rau koj.

Qhov mob no puas zuj zus tuaj thaum koj laus zuj zus?

Yog, thaum peb laus zuj zus, lwm yam teeb meem kev noj qab haus huv uas yuav loj dua tuaj yeem tshwm sim vim tsis muaj pa oxygen txaus rau cov kabmob thiab cov nqaij. Cov neeg uas muaj kab mob sickle cell anemia muaj feem yuav tuag tes tuag taw, mob ntsws, mob raum, mob po, thiab mob siab.

Lub neej nruab nrab ntawm ib tus neeg uas muaj tus kab mob no yog dab tsi?

Tam sim no tej yam zoo dua li yav tas los lawm. Yog tias kuaj pom thiab kho thaum ntxov kom txo tau cov teeb meem , cov neeg uas muaj kab mob ntshav liab qis tuaj yeem nyob tau txog li 50 xyoo. Qee tus neeg tseem nyob tau ntev dua. Yog tias koj muaj lus nug txog qhov no, nrog koj tus kws kho mob tham. Nws tuaj yeem piav qhia rau koj.

Puas muaj ib txoj kev los tiv thaiv kom tus kab mob no tsis txhob loj hlob tuaj?

Kab mob ntshav liab (sickle cell anemia) yog ib yam kab mob uas los ntawm noob caj noob ces, yog li ntawd nws tsis tuaj yeem tiv thaiv tau.Txawm li cas los xij, koj tuaj yeem kuaj ntshav kom paub seb koj puas muaj kev hloov pauv caj ces uas ua rau muaj ntshav liab ntau. Hauv ntau lub tebchaws uas muaj kev vam meej, txhua tus menyuam mos raug kuaj mob thaum yug los. Qhov tseem ceeb tshaj plaws yog kuaj mob thaum ntxov thiab pib kho .

Kuv yuav ua li cas thiaj nyob tau zoo nrog tus kab mob no? (Kev saib xyuas tus kheej)

Cov kev kho mob tshiab tau tso cai rau cov neeg uas muaj tus kab mob sickle cell anemia kom muaj lub neej zoo dua. Yog tias koj muaj tus kab mob no, xav txog cov no:

• Nrhiav kev kho mob tshwj xeeb: Nws yog ib qho tseem ceeb uas yuav tsum nrhiav kev kho mob los ntawm pab pawg kws kho mob ntshav.
• Mus kuaj mob tsis tu ncua: Kev sib raug zoo nrog koj tus kws kho mob hauv tsev neeg thiab mus kuaj mob tsis tu ncua tuaj yeem pab tiv thaiv cov teeb meem loj.
• Xav txog kev tswj qhov mob: Qhov mob yog ib feem ntawm tus kab mob no. Nrhiav kev qhia los ntawm tus kws tshwj xeeb tswj qhov mob.
• Nrhiav kev pab txhawb nqa kev noj qab haus huv ntawm lub hlwb: Qee zaum tus kab mob no tuaj yeem ua rau muaj kev xav tias koj nyob ib leeg, nyuaj siab, thiab ntxhov siab. Yog tias koj xav li no, nrog kws kho mob tham.
• Tiv thaiv koj tus kheej kom tsis txhob muaj kab mob: Tham nrog koj tus kws kho mob txog kev txhaj tshuaj tiv thaiv kab mob. Ua cov kauj ruam los tiv thaiv koj tus kheej kom tsis txhob muaj kab mob.
• Ua tib zoo xav txog koj ib puag ncig: Nws yog ib qho tseem ceeb heev rau cov neeg uas muaj tus kab mob sickle cell anemia kom tswj tau qhov kub ntawm lub cev kom sib npaug. Nyob deb ntawm qhov chaw kub thiab txias heev.
• Saib seb puas muaj kev sim tshuaj kho mob: Cov kws kho mob thiab cov kws tshawb fawb yeej ib txwm sim cov kev kho mob tshiab. Tej zaum koj yuav muaj lub sijhawm koom nrog ib qho.

Kuv yuav tsum mus ntsib kws kho mob thaum twg?

Yog koj xav tias koj cov tsos mob hnyav zuj zus tuaj, lossis yog koj muaj cov tsos mob tshiab, mus ntsib kws kho mob tam sim ntawd .

Thaum twg koj yuav tsum mus rau Chav Xwm Ceev?

Kab mob ntshav liab (sickle cell anemia) tuaj yeem ua rau muaj mob hnyav. Mus rau chav kho mob ceev tam sim ntawd yog tias koj muaj ib qho ntawm cov tsos mob no:

• Cov tsos mob ntawm tus kab mob anemia (qaug zog heev, ua pa luv luv, lub plawv dhia tsis xwm yeem).
• Kub taub hau siab tshaj 38.5 degrees Celsius (101.3 Fahrenheit).
• Mob hauv siab.
• Hnoos.

Qhov mob no kuj ua rau muaj kev pheej hmoo ntawm kev mob stroke. Mob stroke yog ib qho xwm txheej ceev hauv kev kho mob . Yog tias koj muaj cov tsos mob hauv qab no, mus rau tsev kho mob tam sim ntawd:

• Teeb meem nrog kev sib npaug ntawm lub cev lossis kev sib koom tes.
• Pom kev tsis meej los yog pom ob sab.
• Kev tsis meej pem.
• Tsis muaj zog los yog loog ntawm ib sab ntawm lub cev.
• Nyuaj hais lus (hais lus tsis meej).

Thaum kawg, ob peb yam uas yuav tsum nco ntsoov

Kab mob ntshav liab ntshav liab yog ib qho mob uas nyuaj heev. Xyoo dhau los, cov menyuam mos uas yug los nrog tus kab mob no muaj kev cia siab me ntsis rau kev ciaj sia. Tiam sis,Kev tshawb fawb txog kev kho mob tau ua tiav zoo heev hauv kaum xyoo dhau los lossis ntau dua, yog li ntawd ntau tus neeg tam sim no muaj peev xwm tswj hwm tus kab mob ntev no thiab ua neej nyob zoo.

Yog tias koj lossis koj tus menyuam muaj tus kab mob no, thaum koj zoo siab txog qhov kev vam meej uas koj tau ua yav dhau los, cia li cia siab rau yav tom ntej. Cov kws tshawb fawb yeej ib txwm sim nrhiav kev kho mob tshiab rau tus kab mob no. Tham nrog koj tus kws kho mob thiab paub txog cov kev sim tshuaj tshiab. Tsis txhob tso tseg kev cia siab . Nws yog ib qho tseem ceeb heev uas yuav tsum ua raws li cov lus qhia kho mob kom zoo thiab muaj lub siab zoo.

` Kab mob ntshav liab ntshav liab, ntshav liab, cov kab mob uas los ntawm noob caj ces, cov kab mob ntshav, kev hloov pauv ntawm cov noob caj ces, hemoglobin, cov lus qhia txog kev noj qab haus huv