Uyazi nge-Sickle Cell Anemia? Ake sixoxe ngayo!

Wake wezwa ngegama elithi 'Sickle Cell Anemia'? Mhlawumbe leli gama lizwakala lingavamile, noma liyesabeka kancane. Kodwa kuyisimo sofuzo esihlobene negazi lethu. Kalula nje, okwenzekayo kulokhu ukuthi amangqamuzana ethu abomvu egazi, okungukuthi, amangqamuzana abomvu egazini, ashintsha ngesimo. Njengesikela (yingakho libizwa ngokuthi 'isikela'). Lolu shintsho lungabangela izinkinga ezahlukene zempilo. Ngakho-ke, ingabe sizoxoxa ngalokhu ngemininingwane eyengeziwe namuhla?

Kuyini i-Sickle Cell Anemia? Kwenzekani ngempela?

I-sickle cell anemia yilokho odokotela abakubiza ngokuthi uhlobo olubi kakhulu lweqembu elikhulu elibizwa ngokuthi 'iSickle Cell Disease'. Lesi yisifo esizalwa naso, okungukuthi, isifo segazi esizuzwe njengefa . Isizathu esiyinhloko salokhu ukuguquka kwezakhi zofuzo. Ngenxa yalokhu kuguquka kwezakhi zofuzo, amangqamuzana abomvu egazi akhiqizwa emzimbeni wethu ngokuvamile awayindilinga, kodwa agobile futhi anwetshwe njengesikela (ngesimo sohlamvu C). Odokotela babiza futhi lawa ngokuthi "amangqamuzana esikela".

Manje bheka, amangqamuzana ethu abomvu ajwayelekile ayindilinga futhi aguquguquka, ngakho angahamba kalula emithanjeni yethu yegazi ethambile futhi athwale umoya-mpilo kuwo wonke amalungu omzimba, kuzo zonke izitho, nakuwo wonke amathishu. Kodwa lawa maseli abomvu egazi anjengomgibelo aqinile futhi anamathela . Ngakho awahambi kalula emithanjeni yegazi, kodwa avame ukubhajwa ezindaweni ezithile. Njengokuxinana kwezimoto emgwaqweni. Enye into ukuthi, lawa maseli 'esikela' ayaphuka futhi afe ngokushesha kunamaseli abomvu egazi avamile. Ngenxa yakho konke lokhu, inani lamangqamuzana abomvu egazi aphilile emzimbeni wakho liyancipha, okubangela i-anemia enkulu. Okusho ukuthi, ukuntuleka kwegazi.

Esikhathini esidlule, izingane ezizalwa nalesi sifo zazinethuba eliphansi kakhulu lokusinda zize zibe ngabantu abadala. Kodwa-ke, ngentuthuko kwezokwelapha, ukutholwa kusenesikhathi, kanye nokwelashwa okusha , lokhu kushintshe kakhulu. Abantu abaningi manje baphila iminyaka engama-50 noma ngaphezulu.

Sivame kangakanani lesi sifo eSri Lanka? Ngubani onamathuba amaningi okusithola?

Empeleni kunzima ukuthola izibalo ngalesi sifo eSri Lanka. Kodwa uma ubheka umhlaba, lesi sifo sivame kakhulu kubantu base-Afrika, eNingizimu Yurophu, eMpumalanga Ephakathi noma eNdiya yase-Asia. Lokho kusho ukuthi kukhona ukuxhumana kwezakhi zofuzo.

Ziyini izimpawu ze-sickle cell anemia?

Izimpawu zalesi sifo zingahluka kuye ngomuntu, kodwa kunezinye izimpawu ezivamile:

• Ukukhathala okubangelwa yi-anemia: Uma lesi simo senzeka ezinganeni ezincane, zingase zikhale njalo, zizizwe zingaphilile, futhi zenqabe ukuncelisa.
• Ukutheleleka okuvamile:Lesi sifo silimaza udengezi lwethu. Udengezi luyingxenye ebalulekile yesimiso sethu somzimba sokuzivikela. Ngakho-ke uma lubuthakathaka, kunethuba elikhulu lokuthola izifo kanye nokutheleleka. Kodwa-ke, ngenxa yama-antibiotic kanye nemijovo yokugoma etholakalayo manje, le ngozi incishisiwe kakhulu.
• Ubuhlungu: Lesi ngesinye sezimpawu ezivame kakhulu nezimbi zalesi sifo. Izicubu zomzimba azitholi umoya-mpilo owanele, okubangela umonakalo ezicutshini. Lo monakalo yiwona obangela ubuhlungu. Izindawo ezivame kakhulu zobuhlungu yizingalo, imilenze, isifuba kanye nomhlane. Ngezinye izikhathi kungaqala njengobuhlungu obuncane bese kukhula kancane kancane, noma kungaba ubuhlungu obungabekezeleleki ngokuzumayo.
• Ukuvuvukala okubuhlungu ezandleni nasezinyaweni: Lokhu kungenye yezimpawu zokuqala ze-sickle cell anemia enganeni. Ukuvuvukala kwenzeka ngoba amangqamuzana abomvu egazi amise okwesickle anamathela emithanjeni yegazi ethambile ezandleni nasezinyaweni zomntwana, okuvimbela ukugeleza kwegazi.
• Amehlo nesikhumba esiphuzi esibangelwa yi-jaundice: Isibindi sethu sinesibopho sokuhlunga amangqamuzana abomvu egazi. Ku-sickle cell anemia, into ebizwa ngokuthi i-bilirubin, ekhishwa kumangqamuzana esickle afa ngokushesha, iqoqana emzimbeni, okubangela i-jaundice.

Lezi zimpawu zivame ukuqala ukuvela lapho umntwana esenezinyanga ezi-6 kuya kweziyi-9 ubudala . Ngokuhamba kwesikhathi, njengoba inani lamaseli angavamile (ayingozi) emzimbeni landa, izimpawu zingase zishintshe futhi zibe zimbi kakhulu.

Kungani kuvela i-sickle cell anemia? Yini ebangela lokhu?

I-sickle cell anemia ibangelwa ukushintshashintsha kwezakhi zofuzo ezizakhini zethu zofuzo. Ngokukhethekile, kukhona isakhi sofuzo esibizwa ngokuthi i-HBB gene. Lesi sakhi sofuzo sisitshela ukuthi senze iphrotheni ebizwa ngokuthi i-beta-globin, eyingxenye ye-hemoglobin yethu. Njengoba wazi, i-hemoglobin iyinto ebaluleke kakhulu esiza amangqamuzana ethu abomvu egazi ukuthi athwale umoya-mpilo.

Ngakho-ke, ukuze uthole i-sickle cell anemia, kufanele uthole ifa elifanayo elishintshiwe kumama wakho nakubaba wakho . Ngezinye izikhathi, uma uthola ifa lezakhi zofuzo ezimbili ezishintshiwe kubazali bobabili, ungahlakulela ezinye izinhlobo zesifo se-sickle cell, okungenzeka ukuthi azinzima kangako. Odokotela babiza lokhu ngokuthi 'ukuguquguquka kwezakhi zofuzo okuguquguqukayo'.

Yiziphi izinkinga ezingaba khona ngenxa yalesi simo?

I-anemia ye-sickle cell ingabangela izinkinga ezinkulu, ngezinye izikhathi ezisongela ukuphila . Isibonelo, abantu abanalesi sifo bavame ukudinga ukunakekelwa kwezokwelapha okuphuthumayo kanye nokulaliswa esibhedlela, ikakhulukazi ezimweni ezibizwa ngokuthi i-acute chest syndrome (ACS) kanye ne-vaso-occlusive crisis (VOC).

Kunezinye izinkinga:

• Isifo sezinso esingamahlalakhona: Lesi simo singase senzeke lapho izinso zingatholi umoya-mpilo owanele, okubangela ukulimala kwezicubu.
• Ama-retina ahlukene: Lokhu kungenzeka uma amaseli esikela evimba imithambo yegazi esweni.
• I-Priapism (ukuma okubuhlungu): Isimo esibangelwa ukuvaleka kwemithambo yegazi epenis.
• Ukususwa kwe-splenic: Uma amaseli esikele ebhajwa e-spleen futhi evimba ukugeleza kwegazi, lokhu kungabangela i-anemia engazelelwe, enzima.
• Isifo sohlangothi: Noma ubani one-sickle cell anemia, ngisho nezingane ezincane ( isifo sohlangothi sabantwana), usengozini.

Iyini i-Acute Chest Syndrome (ACS)?

Lena inkinga evame kakhulu ye-sickle cell anemia. Futhi iyimbangela ehamba phambili yokufa kanye nembangela yesibili ehamba phambili yokulaliswa esibhedlela. Lokhu kwenzeka lapho ama-sickle cell ehlangana futhi evimba imithambo yegazi emaphashini. Izimpawu zifaka:

• Ubuhlungu besifuba obungazelelwe.
• Ukukhwehlela.
• Imfiva.
• Ubunzima bokuphefumula.

Iyini i-Vaso-occlusive Crisis (VOC)?

Yilapho amaseli esikela evimba khona imithambo yegazi. Odokotela babiza lokhu nangokuthi "inkinga yobuhlungu obukhulu." Ku-VOC, ubuhlungu obungabekezeleleki bungenzeka ezingalweni, emilenzeni, emhlane ongezansi, nasesiswini.

Ngezinye izikhathi odokotela babiza ama-VOC ngokuthi “ukugula okungabonakali.” Ngoba, ezimweni eziningi, uphawu olulodwa kuphela ubuhlungu. Akukho sibonakaliso sokulimala noma esinye isifo. Kubuhlungu nje.

Ukwelashwa okuyinhloko kwalolu buhlungu obukhulu yi-opioid painkillers. Kodwa-ke, ezinye izifundo zikhombisile ukuthi le mfuneko ye-opioid ingaholela ekuhlukanisweni nabantu kubantu abane-sickle cell anemia, okungaholela ekucindezelekeni nasekukhathazekeni. Lena enye inselele okufanele ubhekane nayo uma uphila nalesi sifo.

Lesi sifo sitholakala kanjani? (Ukuxilongwa)

Odokotela baxilonga i-sickle cell anemia ngokukuhlola kuqala. Bangase bakuzwe ubende noma isibindi sakho. Bazokubuza ngezimpawu zakho, ikakhulukazi ubuhlungu ezingalweni nasemilenzeni yakho, kanye nangesisu sakho. Bazokubuza nangezifo zakho zangaphambilini nokuthi ngabe uke waba nazo yini izifo. Bese benza izivivinyo ezifana nalezi:

• Ukubalwa Kwegazi Okuphelele (i-CBC): Lokhu kuhilela ukuhlolwa okuningana okubheka ngqo amangqamuzana akho abomvu egazi.
• I-Hemoglobin electrophoresis: Ebizwa nangokuthi i-high-performance liquid chromatography, lokhu kuhlolwa kuhlaziya i-hemoglobin yakho ukuthola nokulinganisa uhlobo olungavamile lwe-hemoglobin olubangela i-sickle cell anemia.
• Ukuhlolwa kwezakhi zofuzo: Udokotela wakho angase akucele lokhu kuhlolwa ukuze abone ukuthi unezinguquko zezakhi zofuzo ezibangela i-sickle cell anemia.

Yiziphi izindlela zokwelapha lokhu? Ingabe kungelapheka?

Ukwelashwa kwe-sickle cell anemia kuncike ezimpawini zakho kanye nempilo yakho iyonke. Isibonelo, uma unezinkinga ezinkulu njenge-acute chest syndrome, izigameko ezivame ukuba nobuhlungu obukhulu, noma uma unesimo esifana nokukhubazeka, udokotela wakho angancoma ukufakelwa i-allogeneic stem cell. Lena yindlela kuphela yokwelapha ngokuphelele i-sickle cell anemia .

Ezinye izindlela zokwelapha yilezi:

• Ukumpontshelwa igazi.
• Ama-antibiotic okutheleleka.
• Imithi eqeda izimpawu ezithile. Eminye yayo yile:
• I-Hydroxyurea: Lona umuthi wokulwa nomdlavuza. Usetshenziswa ezinganeni eziphakathi kwezinyanga eziyi-6-9, izingane kanye nabantu abadala. Unganciphisa ukwanda kwezinkinga ezinkulu futhi unciphise izimpawu ze-anemia.
• I-Voxeletor: Lokhu kuvimbela amangqamuzana abomvu egazi ukuthi angaguquki abe amangqamuzana esikela. Inikezwa izingane ezingaphezu kweminyaka engu-4 ubudala.
• Ukwelashwa nge-L-glutamine: Lo muthi, onikezwa izingane ezingaphezu kweminyaka emi-5 ubudala kanye nabantu abadala, uvimba amaseli esikele ekubeni angajwayelekile.
• I-Crizanlizumab-tmca: Lokhu kunciphisa ukuvama kwe-VOC/izinhlungu ezibukhali. Inikezwa abantu abangaphezu kweminyaka eyi-16 ubudala.

Impilo yami izoba njani uma ngine-sickle cell anemia?

Kubantu abaningi, i-sickle cell anemia iyisifo esingamahlalakhona . Lokhu kusho ukuthi uzodinga ukunakekelwa kwezokwelapha kanye nokusekelwa kukho konke ukuphila kwakho. Izimpawu zingaba zincane kwabanye, zibe ziphakathi kwabanye, futhi zibe zimbi ngokwanele ukuba zibe yingozi empilweni yabanye. Isimo sabo bonke sihlukile. Ngakho-ke khuluma nodokotela wakho ngalokho ongakulindela. Uyazi impilo yakho kangcono nokuthi lesi sifo sizokuthinta kanjani.

Ingabe lesi simo siba sibi njengoba ukhula?

Yebo, njengoba sikhula, ezinye izinkinga zempilo ezingaba zimbi kakhulu zingavela ngenxa yokungafiki kahle komoya-mpilo ezithweni nasezicutshini. Abantu abane-sickle cell anemia basengozini enkulu yokukhubazeka, ukulimala kwamaphaphu, izinso, udende kanye nesibindi.

Ingakanani isilinganiso sokuphila komuntu onalesi sifo?

Izinto sezingcono kakhulu manje kunangaphambili. Ngokutholakala kusenesikhathi kanye nokwelashwa ukunciphisa izinkinga , abantu abane-sickle cell anemia bangaphila baze bafike eminyakeni engu-50. Abanye abantu baphila isikhathi eside nakakhulu. Uma unemibuzo ngalokhu, khuluma nodokotela wakho. Angakuchazela.

Ingabe ikhona indlela yokuvimbela lesi sifo ukuthi singakhuli?

I-sickle cell anemia iyisifo esidluliselwa ngofuzo, ngakho-ke asinakuvinjelwa.Kodwa-ke, ungathola ukuhlolwa kwegazi ukuze ubone ukuthi une-genetic mutation ebangela i-sickle cell anemia. Emazweni amaningi athuthukile, zonke izingane ziyahlolwa lesi sifo lapho zizalwa. Into ebaluleke kakhulu ukuxilonga lesi sifo kusenesikhathi bese uqala ukwelashwa .

Ngingaphila kanjani kahle nalesi sifo? (Ukuzinakekela)

Izindlela zokwelapha ezintsha zivumele abantu abane-sickle cell anemia ukuba baphile impilo engcono kakhulu. Uma unesifo, cabanga ngalezi zinto:

• Funa usizo lwezokwelapha olukhethekile: Kubalulekile ukufuna ukwelashwa eqenjini lodokotela begazi.
• Thola ukuhlolwa kwezokwelapha njalo: Ukugcina ubuhlobo obuhle nodokotela womndeni wakho kanye nokuya kokuhlolwa njalo kungasiza ekuvimbeleni izinkinga ezinkulu.
• Cabanga ngokuphathwa kobuhlungu: Ubuhlungu buyingxenye evamile yalesi sifo. Funa iseluleko kuchwepheshe wokuphathwa kobuhlungu.
• Thola ukwesekwa kwempilo yengqondo: Ngezinye izikhathi lokhu kugula kungabangela imizwa yokuzihlukanisa, ukucindezeleka, kanye nokukhathazeka. Uma uzizwa ngale ndlela, khuluma nodokotela.
• Zivikele ezifweni: Khuluma nodokotela wakho mayelana nokugonywa. Thatha izinyathelo zokuzivikela ezifweni.
• Qaphela indawo okuyo: Kubaluleke kakhulu kubantu abane-sickle cell anemia ukugcina izinga lokushisa lomzimba lilinganisela. Gwema ukushisa okukhulu kanye nezindawo ezibandayo.
• Bheka izivivinyo zemitholampilo: Odokotela nabacwaningi bahlala behlola izindlela ezintsha zokwelapha. Ungase ube nethuba lokuzibandakanya kolunye.

Kufanele ngimbone nini udokotela?

Uma uzizwa sengathi izimpawu zakho ziya ziba zimbi kakhulu, noma uma uba nezimpawu ezintsha, bona udokotela ngokushesha .

Kunini lapho kudingeka uye eGumbini Lezimo Eziphuthumayo?

I-sickle cell anemia ingabangela izimo zezempilo ezimbi kakhulu. Yiya ekamelweni eliphuthumayo ngokushesha uma unezinye zalezi zimpawu:

• Izimpawu ze-anemia (ukukhathala okukhulu, ukuphelelwa umoya, ukushaya kwenhliziyo okungajwayelekile).
• Umkhuhlane uphakeme ngaphezu kwama-degree Celsius angu-38.5 (101.3 Fahrenheit).
• Ubuhlungu besifuba.
• Ukukhwehlela.

Lesi simo sandisa nengozi yokushaywa yisifo sohlangothi. Isifo sohlangothi siyisimo esiphuthumayo sezokwelapha . Uma unezimpawu ezilandelayo, hamba esibhedlela ngokushesha:

• Izinkinga ngokulinganisela komzimba noma ukuhlangana kwawo.
• Umbono ofiphele noma umbono ophindwe kabili.
• Ukudideka.
• Ubuthakathaka noma ukuba ndikindiki kolunye uhlangothi lomzimba.
• Ubunzima bokukhuluma (ukungingiza).

Okokugcina, izinto ezimbalwa okufanele uzikhumbule

I-sickle cell anemia iyisimo esiyinselele ngempela. Eminyakeni edlule, izingane ezizalwa nalesi sifo zazinethemba elincane kakhulu lokusinda. Kodwa,Ucwaningo lwezokwelapha luye lwenza intuthuko enkulu eminyakeni eyishumi edlule noma ngaphezulu, ngakho abantu abaningi manje sebeyakwazi ukuphatha lesi sifo esingamahlalakhona futhi baphile impilo enhle.

Uma wena noma ingane yakho inalesi sifo, ngenkathi ujabule ngentuthuko oyenzile esikhathini esidlule, yiba nethemba ngekusasa. Abacwaningi bahlala bezama ukuthola izindlela ezintsha zokwelapha lesi sifo. Khuluma nodokotela wakho futhi uqaphele izivivinyo ezintsha zezokwelapha. Ungalokothi ulahle ithemba . Kubaluleke kakhulu ukulandela iseluleko sezokwelapha esifanele futhi ube nesimo sengqondo esihle.

I- Sickle cell anemia, i-anemia, izifo ezizuzwe njengefa, izifo zegazi, ukuguquka kwezakhi zofuzo, i-hemoglobin, amathiphu ezempilo