Created at:1/13/2025
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I-Alpha-1-proteinase inhibitor iyindlela yokwelapha yokufaka esikhundleni samaprotheni enikezwa nge-IV ukusiza abantu abanesimo esingavamile sezakhi zofuzo esibizwa ngokuthi i-alpha-1 antitrypsin deficiency. Lo muthi usebenza ngokufaka esikhundleni samaprotheni angekho noma anezinkinga okujwayele ukuthi avikele amaphaphu akho ekulimaleni.
Uma wena noma othile omkhathalelayo etholiwe ukuthi unokuntuleka kwe-alpha-1 antitrypsin, ungase uzizwe ukhungathekile ngayo yonke imininingwane yezokwelapha. Ake sihambe ngalokho okuhilelekile kulokhu kwelashwa nokuthi kungasiza kanjani ukuvikela impilo yamaphaphu akho ngokuhamba kwesikhathi.
I-Alpha-1-proteinase inhibitor ngumuthi wamaprotheni ahlanzekile owenziwe ngegazi lomuntu elinikelwe. Iqukethe amaprotheni afanayo okuvikela abantu abanempilo abawenza ngokwemvelo esibindini sabo, okubizwa nge-alpha-1 antitrypsin.
Abantu abanokuntuleka kwe-alpha-1 antitrypsin abakwazi ukwenza lawa maprotheni ngokwanele noma bakhiqize inguqulo enezinkinga engasebenzi kahle. Ngaphandle kwamaprotheni asebenzayo anele, ama-enzyme emaphashini akho angalimaza izikhwama zomoya ezibucayi ngokuhamba kwesikhathi, okuholela ezinkingeni zokuphefumula.
Lo muthi usebenza njengendlovu yamaphaphu akho. Uma unikezwa njalo nge-IV, usiza ukubuyisela amazinga amaprotheni okuvikela egazini lakho nasemaphashini, wehlisa ukuqhubeka kokulimala kwamaphaphu.
Lo muthi wenzelwe ngqo ukwelapha ukuntuleka kwe-alpha-1 antitrypsin, isimo sezakhi zofuzo esithinta cishe umuntu oyedwa kwabangu-2,500. Isetshenziswa uma ukuhlolwa kwegazi kukhombisa ukuthi unamazinga aphansi kakhulu amaprotheni e-alpha-1 antitrypsin.
Umgomo oyinhloko ukwehlisa ukulimala kwamaphaphu nokulondoloza umsebenzi wakho wokuphefumula. Ngenkathi ingakwazi ukuguqula umonakalo osuvele ukhona, ingasiza ekuvikeleni noma ukubambezela ukwanda kokonakala kwesicubu sakho samaphaphu.
Udktela wakho ngokuvamile uzoncoma lokhu kwelashwa uma une-genetic deficiency kanye nezimpawu zesifo samaphaphu, njenge-emphysema noma i-chronic obstructive pulmonary disease (COPD). Akusetshenziselwa ezinye izinhlobo zezinkinga zamaphaphu ezingahlobene ne-alpha-1 antitrypsin deficiency.
Lo muthi usebenza ngokufaka esikhundleni sephrothini evikelayo elahlekile emzimbeni wakho. Cabanga nge-alpha-1 antitrypsin njengomqaphi wamaphaphu akho - ngokuvamile ugcina ama-enzyme ayingozi engahlaseli izicubu zamaphaphu ezinempilo.
Lapho wamukela i-IV infusion, umuthi uhamba ngegazi lakho futhi ufinyelele emaphashini akho. Lapho, ubopha futhi unqamule ama-enzyme abizwa ngokuthi i-neutrophil elastase, engabhidliza izindonga zama-air sacs akho uma ingahloliwe.
Lokhu kuthathwa njengokwelashwa okunamandla amaphakathi okudinga ukusetshenziswa okuqhubekayo. Umuthi awulaphi isimo se-genetic esingaphansi, kodwa unikeza ukuvikelwa okuqhubekayo uma unikezwa njalo. Abantu abaningi badinga ukufakwa kwamasonto onke ukuze bagcine amazinga e-protein evikelayo ohlelweni lwabo.
Lo muthi ngaso sonke isikhathi unikezwa njenge-intravenous (IV) infusion endaweni yokunakekelwa kwezempilo. Ngokuvamile uzoyithola kanye ngesonto, ngakunye ukufakwa kuthatha imizuzu engaba ngu-15 kuya kwengama-30 ukuqedela.
Ngaphambi kokufakwa kwakho, akudingeki ukuthi ugweme ukudla noma ukuphuza. Eqinisweni, ukuba nokudla okulula ngaphambi kwesikhathi kungakusiza ukuthi uzizwe ukhululekile ngesikhathi sokwelashwa. Qiniseka ukuthi uhlala unamanzi ngokwanele ngokuphuza amanzi amaningi usuku lonke.
Ukufakwa kuzonikezwa kancane ukuze kuncishiswe noma yiziphi izindlela zokuphendula. Ithimba lakho lokunakekelwa kwezempilo lizokuqapha eduze ngesikhathi nangemva kokwelashwa. Abanye abantu bakuthola kuwusizo ukuletha incwadi noma ithebhulethi ukuze bahlale bematasa ngesikhathi sokufakwa.
I-Alpha-1-proteinase inhibitor ngokuvamile yindlela yokwelapha yokuphila konke. Njengoba ukuntuleka kwe-alpha-1 antitrypsin kuyisimo semvelo, umzimba wakho uyohlale unzima ukukhiqiza amaprotheni anele okuvikela ngokwawo.
Umgomo uwukugcina ukuvikeleka okungaguquki emaphashini akho iminyaka eminingi. Ukuyeka ukwelashwa kungavumela ama-enzyme ayingozi ukuthi aqhubeke nokulimaza izicubu zamaphaphu akho, okungenzeka kuholele ekuqhubekeleni phambili ngokushesha kwe-emphysema noma ezinye izinkinga zamaphaphu.
Udokotela wakho uzohlola njalo ukusebenza kwamaphaphu akho kanye namazinga amaprotheni ngokusebenzisa izivivinyo zegazi nezivivinyo zokuphefumula. Lokhu kusiza ukunquma ukuthi ukwelashwa kusebenza ngempumelelo yini nokuthi ngabe kukhona yini ukulungiswa okudingekayo ohlelweni lwakho lokunakekelwa.
Abantu abaningi bayakubekezelela kahle lokhu kwelashwa, kodwa njenganoma yikuphi ukwelashwa, kungabangela imiphumela emibi. Ukuqonda okulindelekile kungakusiza ukuthi uzizwe ulungiseleleke kakhulu futhi wazi ukuthi ungaxhumana nini nethimba lakho lokunakekelwa kwempilo.
Imiphumela emibi ejwayelekile ethinta abantu abangaphezu koyedwa kwabayishumi ihlanganisa ukusabela okuncane endaweni yokufakwa, njengobuhlungu obuncane, ububomvu, noma ukuvuvukala lapho inaliti ye-IV yayifakwe khona. Ungase futhi uhlangabezane nekhanda elibuhlungu elincane, isiyezi, noma ukukhathala ngemuva kokufakwa kwakho.
Le miphumela emibi ejwayelekile ivamise ukuthuthuka kungakapheli amahora ambalwa ngemuva kokwelashwa futhi akuvamile ukuthi idinge ukuyeka umuthi.
Imiphumela emibi engavamile kodwa ebucayi ingenzeka kubantu abangaba munye kwabayikhulu. Lokhu kufaka phakathi ukusabela komzimba, okungase kuvele njengokuqubuka kwesikhumba, ukuluma, ubunzima bokuphefumula, noma ukuvuvukala kobuso bakho, izindebe, noma umphimbo.
Uma uhlangabezana nanoma yiziphi zalezi zimpawu ezingathi sína, yazisa ithimba lakho lezempilo ngokushesha. Bangashintsha ukwelashwa kwakho noma banikeze ukwesekwa okwengeziwe ukusiza ukuphatha lezi zimpembelelo.
Ngokuvamile, abanye abantu bangase bathuthukise amahlule egazi noma bahlangabezane nokuphendula komzimba okukhulu okubizwa ngokuthi i-anaphylaxis. Lezi zinkinga ezingathi sína zenzeka kubantu abangaphansi kuka-1 kwabangu-1,000, kodwa ithimba lakho lezokwelapha lizohlala lilungele ukuzisingatha uma zenzeka.
Lo muthi awufaneleki kuwo wonke umuntu. Udokotela wakho uzobuyekeza ngokucophelela umlando wakho wezokwelapha ukuze aqiniseke ukuthi kuphephile ukuthi uthole lokhu kwelashwa.
Akufanele uthole i-alpha-1-proteinase inhibitor uma uke waba nokuphendula komzimba okukhulu kulo muthi noma yiziphi izithako zawo esikhathini esidlule. Abantu abanezifo ezithile zegazi noma labo abathatha imithi ethile yokunciphisa igazi bangase badinge ezinye izindlela zokwelapha.
Udokotela wakho uzofuna ukwazi nganoma yimuphi umlando wamahule egazi, izinkinga zenhliziyo, noma isifo sezinso. Ngenkathi lezi zimo zingakhiphi ngokuzenzakalelayo ukwelashwa, zingase zidinge ukuqapha okwengeziwe noma ukulungiswa komthamo ukuze zikugcine uphephile.
Uma ukhulelwe noma uncelisa, qiniseka ukuthi uxoxa ngalokhu nethimba lakho lezempilo. Ngenkathi kukhona ucwaningo olulinganiselwe ngokuphepha ngesikhathi sokukhulelwa, udokotela wakho angasiza ukulinganisa izinzuzo nezingozi zesimo sakho esithile.
Izinkampani eziningana zemithi zenza imithi ye-alpha-1-proteinase inhibitor, futhi zitholakala ngaphansi kwamagama ahlukene omkhiqizo. Izinhlobo ezivame kakhulu ukunqunywa zihlanganisa i-Prolastin-C, i-Glassia, ne-Zemaira.
Noma le mikhiqizo iqukethe isithako esifanayo esisebenzayo, ingase yehluke kancane ngendlela ecutshungulwa ngayo noma igxilwe ngayo. Udokotela wakho uzokhetha inketho efanele kakhulu ngokusekelwe ezidingweni zakho ezithile kanye nomlando wakho wezokwelapha.
Yonke le mithi isebenza ngendlela efanayo futhi inokuqina okufanayo. Uma udinga ukushintsha phakathi kwemikhiqizo nganoma yisiphi isizathu, ithimba lakho lokunakekelwa kwezempilo lizokuqondisa ngokuphepha ekushintsheni.
Njengamanje, i-IV alpha-1-proteinase inhibitor iyindlela yokwelapha eyinhloko yokuntuleka kwe-alpha-1 antitrypsin. Kodwa-ke, abacwaningi basebenza ekuthuthukiseni ezinye izindlela zokuletha ezingase zibe lula esikhathini esizayo.
Ifomu eliphefumulwayo lomuthi lifundwa ezivivinyweni zomtholampilo. Lokhu kungakwazi ukukuvumela ukuthi uthathe ukwelashwa ekhaya kunokuvakashela isikhungo sokunakekelwa kwezempilo masonto onke. Kodwa-ke, le nketho ayitholakali okwamanje ekusetshenzisweni okuvamile.
Kwabanye abantu, ukwelashwa okusekelayo njengama-bronchodilators, ukwelashwa nge-oxygen, noma ukuvuselelwa kwamaphaphu kungase kugcwalise ukwelashwa kwe-alpha-1-proteinase inhibitor. Lokhu akuthathi indawo yesidingo sokushintshwa kwamaprotheni kodwa kungasiza ekuphatheni izimpawu futhi kuthuthukise izinga lempilo.
I-Alpha-1-proteinase inhibitor njengamanje iyona kuphela indlela yokwelapha eqondisa ngqo ukuntuleka kwamaprotheni okuyisisekelo ekuntulekeni kwe-alpha-1 antitrypsin. Eminye imithi ingasiza ekuphatheni izimpawu kodwa ayinikezi izinzuzo ezifanayo zokuvikela emaphashini akho.
Uma kuqhathaniswa nokusebenzisa ama-bronchodilators noma eminye imithi ye-COPD yodwa, ukwelashwa ngokushintshwa kwamaprotheni kuye kwabonakala kunciphisa ukuqhubeka kokulimala kwamaphaphu ngempumelelo enkulu. Ucwaningo luphakamisa ukuthi lungasiza ekulondolozeni ukusebenza kwamaphaphu ngokuhamba kwesikhathi, ikakhulukazi uma kuqaliswa ngaphambi kokuthi kulimale kakhulu.
Inzuzo enkulu ukuthi iqondisa imbangela eyinhloko yenkinga kunokumane iphathe izimpawu. Ngenkathi ezinye izindlela zokwelapha zibalulekile ekuphatheni izinkinga zokuphefumula zansuku zonke, i-alpha-1-proteinase inhibitor inikeza ukuvikelwa okuyingqayizivele kwesikhathi eside okungenakunikwa ezinye izinketho.
I-Alpha-1-proteinase inhibitor ngokuvamile ingasetshenziswa ngokuphepha kubantu abanezifo zenhliziyo, kodwa ukuqapha okwengeziwe kubalulekile. Umuthi unikezwa kancane ukunciphisa ingozi yokulayishwa kwamanzi noma ukucindezeleka kwenhliziyo nemithambo yegazi.
Udokotela wakho uzobuyekeza isimo senhliziyo yakho ngokucophelela ngaphambi kokuqala ukwelashwa. Bangase balungise izinga lokufakwa noma bakuqaphe eduze ngesikhathi sokwelashwa ukuqinisekisa ukuthi inhliziyo yakho iphatha kahle uketshezi olwengeziwe.
Njengoba lo muthi unikezwa ochwepheshe bezempilo ezindaweni ezilawulwayo, ukweqisa ngengozi akuvamile. Ukulinganisa kucatshangelwa ngokucophelela ngokuya ngesisindo sakho futhi kuqashwe ngesikhathi senqubo yokufakwa.
Uma ukhathazekile ngomthamo wakho noma uba nezimpawu ezingavamile ngesikhathi noma ngemva kokwelashwa, khuluma nethimba lakho lezempilo ngokushesha. Bangahlola ukuthi ngabe ukuqapha okwengeziwe noma ukwelashwa kuyadingeka yini.
Uma uphuthelwa ukufakwa kwakho okuhlelelwe masonto onke, xhumana nomhlinzeki wakho wezempilo ngokushesha ngangokunokwenzeka ukuze uhlele kabusha. Kubalulekile ukugcina amazinga amaprotheni aqhubekayo ohlelweni lwakho ukuze kuvikelwe kahle amaphaphu.
Zama ukungahambi ngaphezu kwezinsuku eziyi-7-10 phakathi kokwelashwa uma kungenzeka. Ithimba lakho lezokwelapha lingakusiza ukuthi ubuyele emgqeni ngeshejuli yakho ejwayelekile futhi lingaqapha amazinga akho amaprotheni eduze ngemva komthamo ophuthelwe.
I-Alpha-1-proteinase inhibitor ngokuvamile iyaqhubeka unomphela uma nje uphile ngokwanele ukuyithola futhi inikeza izinzuzo. Isimo se-genetic asinyamalali, ngakho ukuvikelwa okuqhubekayo kuvamile ukuthi kudingeke.
Udokotela wakho uzohlola njalo ukuthi ukwelashwa kusafanele yini kuwe. Izinto ezinjengempilo yonke, ukusebenza kwamaphaphu, kanye nekhwalithi yempilo konke kunendima ekunqumeni ukuthi ngabe uzoqhubeka yini nokwelashwa.
Yebo, ungahamba ngenkathi uthola lokhu kwelashwa, kodwa kudinga ukuhlela okuthile. Kuzodingeka ukuthi uhlele nezikhungo zokunakekelwa kwezempilo lapho uya khona ukuze uhlele ukufakwa, noma ulungise uhlelo lwakho lokuhamba ngosuku lwakho lokwelashwa.
Izikhungo eziningi zokwelashwa zingasiza ekuhleleni ukunakekelwa nezikhungo kwamanye amadolobha. Kungcono ukuqala ukuhlela emasontweni ambalwa ngaphambi kohambo lwakho ukuze kuqinisekiswe ukuqhubeka kokunakekelwa ngenkathi ungekho.