Ingabe nawe unakho ukukhwehlela nobunzima bokuphefumula obekulokhu kuqhubeka isikhathi eside? Ungase ucabange ukuthi lokhu kuyinto evamile. Kodwa ngezinye izikhathi, kungase kube nesizathu esibaluleke kakhulu ngemuva kwalezi zimpawu esingakazwa kakhulu ngazo. Namuhla sizokhuluma ngesimo esinjalo. Leso yi-Alpha-1 Antitrypsin Deficiency, noma `(Alpha-1 Antitrypsin Deficiency)`. Abanye abantu basibiza nangokuthi "Alpha-1" ngamafuphi.
Kalula nje, iyini i-Alpha-1?
I-Alpha-1 isifo sofuzo esizuzwe njengefa esisizuza kubazali bethu. Okwenzekayo kulokhu ukuthi imizimba yethu ayikwazi ukukhiqiza iphrotheni eyanele ebizwa ngokuthi i-alpha-one antitrypsin `(AAT)`, okuyiphrotheni evikela amaphaphu ethu. Cabanga ngale phrotheni ethi 'AAT' njengesivikelo samaphaphu ethu. Uma lesi sivikelo silahlekile, amaphaphu ethu asengozini enkulu yokulimala.
Ngakho-ke, umuntu onesimo se-Alpha-1 usengozini enkulu yokuthola izifo zamaphaphu, ikakhulukazi i-emphysema (umonakalo wezikhwama zomoya emaphashini), i-cirrhosis (izibazi zesibindi), kanye ne -panniculitis, isimo sesikhumba esingavamile. Ngezinye izikhathi, lezi zimo zingaba yingozi empilweni.
Ngenxa yalesi sizathu, abanye abantu babiza lesi sifo ngokuthi "i-COPD yofuzo".
Lesi simo senzeka kanjani? Ubani osengozini enkulu?
Ukuze sithuthukise i-Alpha-1, sidinga ukuzuza ifa lofuzo olungavamile kubazali bethu bobabili. Kalula nje, izakhi zofuzo ziyimiyalelo enquma indlela imizimba yethu esebenza ngayo. Uma kukhona ushintsho oluncane kule miyalelo, umsebenzi womzimba ungashintsha.
Ku-Alpha-1, ukuguquka kwezakhi zofuzo okubizwa ngokuthi i-'SERPINA1' kubangela izinkinga ekukhiqizweni kweprotheyini ye-'AAT'. Ngenxa yalokho, iphrotheyini ye-'AAT' ayikhiqizwa ngobuningi obanele, noma iphrotheyini ekhiqizwayo ithatha isimo esingalungile. Uma ithatha isimo esingalungile, leyo phrotheyini ayikwazi ukushiya isibindi futhi ihambe ngegazi iye emaphashini. Ngemuva kwalokho leyo phrotheyini engaguquki inamathela esibindini, yonakalise isibindi. Futhi, njengoba kungekho phrotheyini eya emaphashini, amaphaphu nawo ayasengozini.
Kodwa-ke, abanye abantu bathola lesi sakhi sofuzo esingasebenzi kahle kumzali oyedwa kuphela (kungaba unina noma uyise). Sibabiza ngokuthi “abathwali.” Nakuba imizimba yabo ivame ukukhiqiza amaprotheni e-‘AAT’ anele ukuvikela amaphaphu abo, basesengozini yokulimala kwamaphaphu. Le ngozi iphezulu kakhulu uma bebhema.
Lokhu kuthinta kanjani amaphaphu nesibindi?
Ukuze siqonde lokhu, ake sithathe isibonelo esincane.
Iphrotheni yethu ethi 'AAT' yenziwa esibindini. Bese ihamba ngegazi iye emaphashini. Amaphaphu ethu ane-enzyme ebizwa ngokuthi 'i-Neutrophil Elastase' ewasiza ekulweni nezifo. Cabanga ngale enzyme njengesosha elinolaka kakhulu nelinekhono. Ibhubhisa izifo. Kodwa uma isiqediwe, othile kufanele ayimise. Ngaphandle kwalokho, iqala ukuhlasela namaseli ethu amaphaphu aphilile.
Leyo nto "yokumisa", lowo msebenzi "wokuvala i-switch", wenziwa yiprotheni yethu ethi 'AAT'. Kufana nomlawuli etshela isosha ukuthi, "Kulungile, sekwanele, yeka."
Manje, kumuntu one-Alpha-1, lesi sikhulu se-'AAT' sishoda emzimbeni. Khona-ke akekho ozomisa lelo sosha elinolaka ('Neutrophil Elastase'). Lihlala lihlasela amaphaphu. Lokhu kubangela ukuthi iphrotheni ethi 'Elastin' emaphashini ibhujiswe. Le elastin yiyona enikeza ama-air sac (alveoli) emaphashini ukuqina kwawo namandla njengebhande lerabha. Uma elahlekile, ama-air sac aba buthakathaka futhi awe njengebhaluni eliphumile. Yilokhu esikubiza ngokuthi 'i-Emphysema' . Lokhu kwenza kube nzima ukuphefumula nokuthola umoya-mpilo.
Okwenzeka esibindini kuhlukile kancane. Ngenxa yokukhubazeka okuthile kwezakhi zofuzo, iphrotheni ethi `AAT` yakhiwa ngesimo esingalungile. Njengephepha eligoqwe ngendlela engafanele. Ngenxa yalesi simo esingalungile, iphrotheni ayikwazi ukuphuma esibindini bese inamathela ngaphakathi kwesibindi. Uma iphrotheni eningi kakhulu inamathela kanje, isibindi siyalimala bese siqala ukuba nesibazi. Lokho kubizwa ngokuthi i-cirrhosis .
Ziyini izimpawu zesifo i-Alpha-1?
Izimpawu ezibangelwa yi-Alpha-1 ziyahlukahluka kuye ngesitho somzimba esithintekile. Lezi zivame ukufana nezimpawu ze-COPD (Isifo Esingapheli Sokuvimbela Amaphaphu).
| Isitho esithintekile | Izimpawu ezivamile |
|---|---|
| Amaphaphu (Ngokuvamile kuqala phakathi kweminyaka engama-30-50) |
|
| Isibindi (Cishe u-10% wezinsana kanye no-15% wabantu abadala) | |
| Isikhumba (Akuvamile kakhulu) |
Ungasithola kanjani lesi sifo?
Indlela eyinhloko yokuthola i-Alpha-1 iwukuhlolwa kwegazi . Ngenxa yokuthi izimpawu zifana nezinye izifo, ngezinye izikhathi kungathatha isikhathi ukuthola ukuxilongwa okunembile. Uma unezimpawu zesibindi, noma uma utholakale une-COPD futhi izimpawu zakho zingathuthuki naphezu kokwelashwa, udokotela wakho angase anqume ukukuhlola i-Alpha-1.
Ukuhlolwa okwenziwa ngokuvamile yilokhu:
- Ukuhlolwa kwegazi: Igazi lakho lizohlolwa izinga leprotheni ethi `AAT`. Uma izinga liphansi, kuzokwenziwa ukuhlolwa kofuzo ukuze kubonakale ukuthi unesici sofuzo esihlobene ne-Alpha-1.
- Ukuhlolwa kwezithombe: I -X-ray yesifuba noma i-CT scan inganquma ubukhulu nendawo yomonakalo wamaphaphu.
- Ukuhlolwa kokusebenza kwamaphaphu: Nakuba lokhu kungakwazi ukubona i-Alpha-1 ngqo, kunganikeza udokotela wakho umbono wokuthi amaphaphu akho asebenza kahle kangakanani.
- I-Liver Ultrasound noma i-FibroScan®: Uma kukhona ukusolakala komonakalo wesibindi, lokhu kuskena kwenziwa ukuze kuhlolwe ukuthi kukhona amanxeba esibindini.
- Ukuhlolwa Kwesibindi: Uma umonakalo wesibindi umkhulu, kuthathwa ingxenye encane kakhulu yezicubu ezivela esibindini bese ihlolwa ukuze kutholakale ukuthi umonakalo ungakanani ngempela.
Yiziphi izindlela zokwelapha i-Alpha-1?
Nakuba kungekho ikhambi eliqondile le-Alpha-1 syndrome, kunezindlela eziningi zokwelapha ezingalawula izimpawu, zinciphise ukulimala kwezitho zomzimba, futhi zithuthukise ikhwalithi yokuphila.
Into ebaluleke kakhulu ukuxilonga lesi sifo kusenesikhathi, wenze izinguquko ezidingekayo endleleni yokuphila, bese ulandela iseluleko sikadokotela.
Izindlela zokwelapha ziyahlukahluka kuye ngokuthi isitho sithintekile:
Ukwelashwa kwamaphaphu
- Ukwelashwa Kokwandisa: Lokhu kuyindlela ethile yokwelapha i-Alpha-1. Kuhilela ukuhlanza iphrotheni ethi 'AAT' ethathwe kubanikeli begazi abanempilo bese uyinika umzimba ngomthambo, ofana nosawoti. Nakuba lokhu kungenakukwazi ukubuyisela emuva umonakalo osuvele wenzeke, kungamisa noma kulawule kakhulu umonakalo wamaphaphu esikhathini esizayo.
- Imithi: Imithi efana ne -inhaler , njenge-bronchodilators, inikezwa iziguli ezine-COPD ukuze kube lula ukuphefumula.
- Ukwelashwa nge-oxygen: Uma izinga le-oxygen egazini liphansi, i-oxygen eyengeziwe inikezwa ngethubhu elincane elibekwe ekhaleni noma imaski yomlomo.
- Izinhlelo zokuvuselela amaphaphu: Ukuqeqeshwa kunikezwa ukuze kube lula ukuphefumula ngokuzivocavoca ukuphefumula kanye nokunye ukuvivinya umzimba.
- Ukufakelwa kwamaphaphu: Uma amaphaphu onakele kakhulu, ukufakelwa kwamaphaphu okunempilo kungadingeka njengesisombululo sokugcina.
Ukwelashwa kwesibindi
Ukulimala kwesibindi kwelashwa ngezimpawu. Kodwa-ke, okuwukuphela kwendlela yokwelapha ngokuphelele isifo sesibindi se-Alpha-1 ukufakelwa kwesibindi . Lapho isibindi esinempilo sitshalwa, isibindi siqala ukukhiqiza iphrotheni efanele ye-'AAT'.
Yini engingayenza ukuze ngiphile impilo enhle nge-Alpha-1?
Ngisho noma kutholakale ukuthi une-Alpha-1, akusikho ukuphela kwempilo yakho. Kunezinto eziningi ongazenza ukuze unciphise umonakalo ezithweni zakho zomzimba.
Into ebaluleke kakhulu neyokuqala ukugwema ngokuphelele ukubhema. Ukubhema kumuntu one-Alpha-1 kufana nokufaka uphethiloli emlilweni. Kukhulisa kakhulu izinga lokulimala kwamaphaphu.
Ngaphezu kwalokho, qaphela lezi zinto ezilandelayo:
- Hlala kude nezindawo lapho abantu bebhema khona. (Gwema intuthu ebhemayo)
- Gwema ukuhogela izinto eziyingozi emaphashini akho, njengothuli namakhemikhali. Uma ubhekene nezinto ezinjalo emsebenzini, gqoka imaski evikelayo.
- Yeka ukuphuza utshwala ngokuphelele noma unciphise ngangokunokwenzeka. Utshwala bungandisa ukulimala kwesibindi.
- Gwema ukusebenzisa imithi yokubulala izinhlungu (isib., ukuthatha i-paracetamol eningi) noma amanye amakhambi angathinta isibindi ngaphandle kokubonisana nodokotela wakho.
- Thola yonke imijovo edingekayo. Kubaluleke kakhulu ukugonyelwa izifo ezifana ne-pneumonia, i-influenza (flu), i-COVID-19, kanye ne-hepatitis A no-B, ezithinta isibindi.
- Geza izandla zakho njalo, uhlale uhlanzekile, futhi uzivikele ezifweni.
- Uma othile emndenini wakho ene-Alpha-1, kuwumqondo omuhle ukuzihlola wena. Khuluma nodokotela wakho ngakho.
- Uma une-Alpha-1 noma unesifo esithathelwanayo, kubaluleke kakhulu ukukhuluma nomeluleki wezakhi zofuzo uma uhlela ukuba nezingane.
Kufanele ubonane nini nodokotela?
- Uma unezimpawu zamaphaphu noma zesibindi esikhulume ngazo ngaphambili.
- Uma othile emndenini wakho etholakale ene-Alpha-1.
- Uma utholakale une-COPD noma i-asthma futhi welashwa, kodwa izimpawu zakho azithuthuki, khuluma nodokotela wakho mayelana nokuhlolwa kwe-Alpha-1.
- Uma usuvele unayo i-Alpha-1, bona udokotela wakho ngokushesha uma kuvela izimpawu ezintsha noma izimpawu ezikhona ziba zimbi kakhulu.
Ukuphila ne-Alpha-1 kungaba yinselele. Kodwa ngolwazi olufanele, ukwelashwa, nendlela yokuphila, nawe ungaphila impilo enempilo nesebenzayo. Indlela engcono kakhulu yokwenza lokho ukukhuluma ngokukhululekile nodokotela wakho bese uqhamuka necebo elikusebenzelayo.
Umlayezo Wokuya Nawe Ekhaya
- Ukuntuleka kwe-Alpha-1 Antitrypsin yisifo esidluliselwa kubazali. Kulokhu, umzimba awunayo iphrotheni ye-AAT, evikela amaphaphu nesibindi.
- Lokhu kwandisa ingozi yokulimala kwamaphaphu (i-Emphysema) kanye nesibindi (i-Cirrhosis) .
- Uma unezimpawu ezifana nokukhwehlela isikhathi eside, ubunzima bokuphefumula, noma ukuba phuzi kwamehlo, funa iseluleko sikadokotela.
- Into ebaluleke kakhulu umuntu onalesi sifo angayenza ukugwema ngokuphelele ukubhema.
- Ngokwelashwa okufanele kanye nokushintsha indlela yokuphila, ungalawula lesi sifo futhi uphile impilo enempilo.
💬 Comments (0)
Awekho amazwana athunyelwe okwamanje. Faka amazwana akho lapha okokuqala ngqa.
Engeza amazwana akho