Uke waba nale nkinga ngamaphaphu akho? (Pulmonary Alveolar Proteinosis - PAP) Ake sixoxe ngalokhu!

Ingabe uzizwa uphelelwa umoya? Noma ukhathele nje? Ngezinye izikhathi lezi zinto zibhekwa njengezivamile, kodwa ezimweni ezingavamile, zingaba izimpawu zesifo samaphaphu. Namuhla sizokhuluma ngesifo samaphaphu esingavamile kodwa esibalulekile okubalulekile ukwazi ngaso. Lokhu kubizwa ngokuthi `(Pulmonary Alveolar Proteinosis)` noma `(PAP)`. Nakuba igama lingase lizwakale liyinkimbinkimbi kancane, ake silichaze kalula.

Kuyini `(Pulmonary Alveolar Proteinosis - PAP)`? Kalula nje...

Kulungile, manje ake sibone ukuthi lesi simo sibizwa ngokuthi `(PAP)`. Kalula nje, yisifo esingavamile esenzeka lapho amaprotheni, amafutha, nezinye izinto ziqoqana emasakeni amancane omoya (ama-alveoli) ngaphakathi kwamaphaphu ethu futhi zibangele ukuthi avaleke. Cabanga nje, amaphaphu ethu akhiwe izigidi zamasakeni amancane omoya. Lawa masakeni amancane omoya yilokho esikubiza ngokuthi `ama-alveoli` . Uma siphefumula, umoya-mpilo uyangeniswa bese ukhishwa egazini ngalezi zikhwama zomoya.

Ngakho-ke, uma kwenzeka i-`(PAP)`, lawa masaka omoya agcwala izinto ezingadingekile, njengomsele ovalekile, ovimba indlela yokuthi umoya-mpilo ufike egazini. Kwenzekani-ke? Igazi lethu alitholi umoya-mpilo owanele. Lokhu kungabangela ubunzima bokuphefumula (`dyspnea`) .

Lesi yisifo esibi kakhulu samaphaphu. Ngakho-ke kujwayelekile ukuzizwa wesaba kancane uma uthola ukuxilongwa okunje. Kodwa ungakhathazeki. Ngokwelashwa okufanele, ungalawula lesi sifo kanye nezimpawu zaso. Uma une-`(PAP)`, kubaluleke kakhulu ukubona udokotela wezokwelapha amaphaphu ukuze uthole ukwelashwa. Uzokweluleka nangezinguquko zendlela yokuphila enempilo ongazenza. Lokhu kungathuthukisa ikhwalithi yempilo yakho.

Ingabe kunezinhlobo ezahlukene zalesi sifo `(PAP)`?

Yebo, kunezinhlobo ezintathu eziyinhloko zesifo `(PAP)`. Ake sibone ukuthi ziyini:

1. I-Autoimmune PAP: Lolu uhlobo oluvame kakhulu lwe-PAP. Cishe abantu abadala abangama-90% bahlakulela lolu hlobo. Okwenzekayo lapha ukuthi amasosha omzimba wethu ahlasela ngephutha amangqamuzana asiza ukugcina izindlela zokuphefumula zihlanzekile. Cabanga ngakho njengokuzivikela kwethu okulwa namangqamuzana ethu.

2. I-PAP yesibili: Lolu hlobo lwenzeka lapho umsebenzi wamaseli emigudwini yethu yomoya uthinteka, kungaba ngenxa yesinye isifo noma ukuchayeka entweni enobuthi (isib. uthuli, amakhemikhali). Okusho ukuthi, kwenzeka ngesinye isizathu.

3. I-Congenital PAP: Lesi yisimo esikhona lapho kuzalwa. Ngezinye izikhathi izimpawu zingase zibe khona lapho kuzalwa, noma zingase zivele eminyakeni ehlukene. Lokhu kungenxa yezinguquko kwezinye izakhi zofuzo ezilawula ukusebenza kwamaseli emaphashini ethu. Lokhu kungazuzwa kubazali kuya ezinganeni.

Ubani onamathuba amaningi okuthola lesi sifo `(PAP)`?

Ngokuvamile, izinhlobo ezimbili ezivame ukuthinteka kakhulu yi-"Autoimmune PAP" kanye ne-"Secondary PAP".Kubantu abaneminyaka ephakathi kuka-30 no-60. Futhi, lezi zinhlobo ezimbili zivame kakhulu kwabesilisa . Abantu ababhemayo kanye nalabo abachayeka ezinhlotsheni ezithile zothuli noma amakhemikhali basengozini enkulu yokuthola lesi sifo.

Uhlobo olubizwa ngokuthi `(Congenital PAP)` luvame ukubonakala ezinganeni ezingaphansi kweminyaka eyi-10. Kodwa-ke, njengoba kushiwo ngaphambili, lungenzeka kunoma yimuphi ubudala.

Sivame kangakanani isifo se-PAP?

Eqinisweni, `(PAP)` yisifo esingavamile kakhulu. Emhlabeni wonke, lesi sifo sithinta abantu abangaba yizigidi ezingamashumi amane kwabathathu. Lokhu kusho ukuthi kungenzeka ukuthi kunenani elincane kakhulu labantu abanalesi sifo eSri Lanka.

Kungani lesi sifo `(PAP)` sivela? Iyini imbangela?

Njengoba sesike sakhuluma ngakho ngaphambili, izindonga zama-air sac ethu (i-alveoli) zincane kakhulu. Kungalezi zindonga ezincane lapho umoya-mpilo udlula khona usuka emaphashini uye egazini. Izindonga zalezi zindawo zomoya ngokwemvelo zinengqimba yamafutha ebizwa ngokuthi ama-surfactants . Lawa ma-surfactants asiza ukugcina ama-air sac evulekile ngaphandle kokuwavala. Yilapho umoya-mpilo ungadlula kalula khona ungene egazini.

Manje, ngaphakathi kwamaphaphu ethu, kukhona uhlobo olukhethekile lweseli olubizwa ngokuthi `ama-alveolar macrophages` . Lawa maseli afana nabasebenzi bokuhlanza. Abakwenzayo ukususa lawa `(ama-surfactants)` ngezikhathi ezithile emasakeni omoya, ukuze angavaleki.

Kodwa-ke, okwenzekayo kumuntu one-PAP ukuthi la maseli abizwa ngokuthi ama-macrophage awatholi isignali yokuhlanza izindonga zama-air sac kahle. Kwenzekani-ke? Lawo ma-surfactants ayakha futhi avimbe ama-air sac. Njengepayipi elivalekile esinkini. Khona-ke indlela yomoya-mpilo eya egazini iyavalwa.

Kalula nje, lesi simo sibangelwa ubuthakathaka "ohlelweni lokuhlanza udoti" lwamaphaphu.

Ziyini izimpawu zesifo `(PAP)`?

Uphawu oluyinhloko noluvame kakhulu lwe-PAP ukuphelelwa umoya (ukuphelelwa umoya) . Nakuba abantu abaningi bephelelwa umoya ngesikhathi sokuzivocavoca, abanye abantu bangase babhekane nalobu bunzima ngisho noma bemi nje.

Ngaphezu kwalokho, kunezinye izimpawu eziningana:

• Ukuzwa ubuhlungu esifubeni.
• Ukukhwehlela. Ngezinye izikhathi kungase kube negazi elincane eliphuma ne-mucus.
• Ukushintsha kombala wesikhumba nezinzipho okuluhlaza okwesibhakabhaka (i-cyanosis) (lokhu kwenzeka uma kungekho oxygen eyanele egazini).
• Ukuzizwa ukhathele ngaso sonke isikhathi (ukukhathala).
• Imfiva.
• Ukutheleleka kwamaphaphu okuvamile.
• Izihloko zeminwe ziyakhula futhi zibe nesimo esifana nesigubhu ('iminwe eboshwe nge-clubbed').
• Ukwehlisa isisindo ngaphandle kwesizathu.

Uma unesinye noma ngaphezulu salezi zimpawu, kubaluleke kakhulu ukufuna iseluleko sezokwelapha.

Ingabe isifo se-PAP singaba yingozi?

Ezimweni ezimbi kakhulu, i-PAP ingabangela ukwehluleka kokuphefumula okusongela impilo.Lokhu kusho ukuthi amaphaphu awakwazi ukwenza umsebenzi wawo kahle. Kodwa-ke, uhlobo lwalesi sifo luyahlukahluka kuye ngomuntu nomuntu. Abanye abantu bangalulama kulesi sifo ngaphandle kokwelashwa (`ukukhululwa okuzenzakalelayo`). Kwabanye, lesi sifo sihlala sisezingeni elifanayo. Ezimweni ezimbi kakhulu, ukufa kungenzeka ngenxa yokwehluleka kokuphefumula noma ezinye izifo. Yingakho kubalulekile ukuxilonga lesi sifo kusenesikhathi bese uthola ukwelashwa.

Ihlolwa kanjani i-PAP? Yiziphi izivivinyo ezenziwayo?

Uma udokotela esola ukuthi une-PAP, uzoqala ngokukuhlola futhi alalele amaphaphu akho. Uzophinde akubuze imibuzo mayelana nomlando wakho wezokwelapha kanye nowomndeni kanye nendlela yakho yokuphila. Uma ubhema noma ubhekene nothuli noma amakhemikhali, qiniseka ukuthi uyamtshela udokotela wakho.

Ukuhlolwa kokuqinisekisa i-PAP kungafaka:

• Ukuhlolwa kwegazi: Hlola amazinga omoya-mpilo egazini lakho.
• Ukuhlolwa kwegazi okukhethekile: Funa inkomba ebizwa ngokuthi `(granulocyte-macrophage colony-stimulating factor - GM-CSF) autoantibody`. Lokhu kusiza ukuthola imbangela yesifo `(PAP)`.
• Ukuhlolwa kokusebenza kwamaphaphu: Kala ukuthi amaphaphu akho asebenza kahle kangakanani.
• Ukuhlolwa kwezithombe: Lokhu kufaka phakathi i-X-ray yesifuba noma i-CT scan. Lokhu kunganikeza isithombe esicacile sesimo samaphaphu.
• I-Bronchoscopy: Kulokhu, kufakwa ipayipi elincane (elinekhamera) ngomlomo liye emigudwini yomoya bese kuhlolwa ingaphakathi lamaphaphu.
• I-biopsy yamaphaphu: Kuthathwa ingxenye encane yezicubu emaphashini ukuze ihlolwe, kungaba ngesikhathi se-bronchoscopy noma ngesikhathi sokuhlinzwa.

Lezi zivivinyo yizo ezivumela odokotela ukuthi baxilonge i-PAP ngokunembile.

Ingabe ikhona ikhambi eliphelele lalesi sifo `(PAP)`?

Kuze kube manje, alikho ikhambi le-PAP. Kodwa ungakhathazeki. Kunezindlela eziningi zokwelapha ezingakusiza ukulawula izimpawu zakho futhi uphile impilo engcono.

Isifo se-PAP selashwa kanjani ?

Ukwelashwa okuyinhloko nokusetshenziswa kakhulu kwe-PAP yi -Whole-Lung Lavage (WLL) . Lokhu kubizwa nangokuthi "ukuhlanza amaphaphu." Cabanga ngakho njengokugeza amaphaphu akho.

Udokotela usebenzisa i-bronchoscope (ipayipi elincane elinekhamera) kanye nesisombululo sikasawoti (amanzi anosawoti ahlanzekile) ukuze akhiphe iphaphu elilodwa ngesikhathi. Lokhu kuzokwenza kube lula ngawe ukuphefumula. Le nqubo ingathatha cishe amahora amane kuya kwamahlanu. Kuye ngobukhulu bezimpawu zakho, kungadingeka ukuthi ube ne-WLL njalo ezinyangeni ezimbalwa noma kanye ngonyaka. Kodwa-ke, ezinye iziguli ze-PAP zingase zingayidingi i-WLL. Lokhu kuncike ebunzimeni besimo sakho.

Kwenzekani uma wenza i-`(Whole-Lung Lavage - WLL)`?

• Uzonikezwa imithi yokubulala izinzwa evamile noma imithi yokudambisa ubuhlungu.
• Udokotela ufaka i-bronchoscope ngomlomo wakho iye endaweni yakho yokuphefumula bese eyikhomba kwelinye lamaphaphu akho.
• Umshini unikeza umoya-mpilo kwelinye iphaphu.
• Ugqoka ijazi elidlidlizayo elizungeze isifuba sakho. Lokhu kudlidliza kukhulula ama-surfactant avaleleke ezikhwameni zomoya.
• Udokotela ube esefaka isixazululo se-saline emaphashini bese esikhipha (ama-suction) kanye nama-surfactants.
• Ngezinye izikhathi elinye iphaphu lingagezwa ngesikhathi esifanayo, noma lingagezwa izinsuku ezimbalwa.

Ngaphezu kwalokhu kwelashwa kwe-`(WLL)`, kunezinye izindlela zokwelapha:

• Ama-Bronchodilator: Le mithi ikhulula imisipha ezungeze imigudu yakho yokuphefumula, okwenza kube lula ngawe ukuphefumula.
• Ukwelashwa kokufaka esikhundleni se-granulocyte-macrophage colony-stimulating factor (GM-CSF): Lokhu kunikezwa ngokuhogela nge-nebulizer (idivayisi eguqula umuthi owuketshezi ube umhwamuko) noma njengomjovo. Lokhu kwelashwa kusebenza ngokuthuthukisa ukusebenza kwamaseli ahlanza izindlela zomoya (ama-macrophage).
• Ukufakelwa kwamaphaphu: Abantu abanesifo se-PAP esibi kanye nomonakalo wamaphaphu ngezinye izikhathi bangadinga ukufakelwa amaphaphu. Kule nqubo, elilodwa noma womabili amaphaphu akho anesifo ayasuswa bese kufakwa elinye elivela kumnikeli onempilo.
• I-Plasmapheresis kanye nokushintshana kwe-plasma: Lokhu kuhilela ukususa ingxenye ewuketshezi yegazi lakho, ebizwa ngokuthi i-plasma, bese uyifaka esikhundleni sayo nge-plasma evela kumnikeli onempilo. Kubantu abane-autoimmune PAP, lokhu kwelashwa kusiza ukuvimbela amasosha omzimba ekuhlaseleni amaseli anempilo.
• Umoya-mpilo owengeziwe: Ukwelashwa komoya-mpilo kungakusiza ukuthi uphefumule kalula. Kunganikezwa ngemaski ebusweni bakho noma ngepayipi elincane elifakwe ekhaleni lakho.
• Izivivinyo zemitholampilo: Ungase futhi ube nethuba lokubamba iqhaza ocwaningweni lwezokwelapha ezintsha. Khuluma nodokotela wakho ngalokhu.

Ingabe isifo se-PAP singavinjelwa?

Njengoba sixoxile ngaphambili, kunezinhlobo ezahlukene ze-PAP. Ngakho-ke, i-Autoimmune PAP noma i-Congenital PAP azinakuvinjelwa ngoba zihlobene nezinqubo zangaphakathi emzimbeni wethu.

Kodwa-ke, kungenzeka ukuvimbela `(i-Secondary PAP)` ngezinga elithile. Ungakwenza kanjani lokho? Kufanele unciphise ukuchayeka kwakho ezintweni ezinobuthi ngangokunokwenzeka . Uma lokho kungenzeki, kufanele usebenzise imaski enhle (`i-respirator` noma i-facemask efakwe kahle) emboza umlomo nekhala lakho kahle.

Uyini umbono womuntu one-PAP?

Ukwelashwa okuvamile okufana nokuthi `(Whole-Lung Lavage - WLL)` kungakusiza ukuthi uphefumule kalula futhi kunciphise ezinye izimpawu. Inani elincane labantu liyalulama ku-`(PAP)` ngaphandle kokwelashwa. Kodwa-ke, uma kungelashwa, izimo ezimbi ze-`(PAP)` zingaholela ekuhlulekeni kokuphefumula noma ekufeni. Ngakho-ke, kubaluleke kakhulu ukulandela imiyalelo kadokotela wakho.

Uma ngine-PAP, yini engingayenza mina?

Uma une-PAP, kunezinto eziningana ongazenza ukuze ulondoloze impilo yakho:

• Buza udokotela wakho mayelana nokuhlolwa kwezokwelapha kwe-PAP.
• Ungabhemi. Futhi, ungaphefumuli intuthu ebhemayo uma abanye abantu bebhema .
• Landela imiyalelo kadokotela wakho mayelana nokuthola imijovo yomkhuhlane kanye ne-pneumonia.
• Hlala kude nabantu abagulayo.
• Yidla ukudla okunomsoco. Lawula isisindo somzimba wakho.
• Geza izandla zakho kahle ngemva kokusebenzisa indlu yangasese, ngaphambi nangemva kokudla.
• Sebenzisana nodokotela wakho ukuze wakhe uhlelo lokuzivocavoca oluphephile olukufanele.

Khumbula, i-Pulmonary Alveolar Proteinosis (PAP) yisifo esingavamile samaphaphu esingabangela ubunzima bokuphefumula kanye nezinye izimpawu ngokubangela ukuthi amasaka omoya emaphashini avaleke. Kodwa-ke, kunezindlela eziningana zokwelapha ezisebenzayo ezingasiza ekulawuleni imiphumela ye-PAP. Uma unalesi simo, cabanga ngokusebenza nethimba elinolwazi oluningi. Ndawonye, ​​​​zingasiza ekuthuthukiseni ikhwalithi yokuphila kwakho.

Umyalezo Ofanele Uwuthathe Ekhaya (Into ebaluleke kakhulu okufanele uyikhumbule)

Kulungile, ngakho-ke sesikhulume kakhulu nge-`(Pulmonary Alveolar Proteinosis - PAP)`. Nakuba lokhu kungase kubonakale kuyinkimbinkimbi kancane, into eyinhloko okufanele uyikhumbule ukuthi yize lesi kuyisifo esingavamile, kubalulekile ukungesabi futhi uthole iseluleko sezokwelapha esifanele kanye nokwelashwa.

• Uma unezimpawu ezifana nobunzima bokuphefumula, ukukhwehlela okuqhubekayo, noma ukukhathala okuvamile, bona udokotela.
• Uma kutholakale ukuthi une-`(PAP)`, funa ukwelashwa ngaphansi kokuqondisa kukadokotela wamaphaphu .
• Kukhona ukwelashwa okutholakalayo, ngakho ungalilahli ithemba. Ukwelashwa okufana nokuthi `(Whole-Lung Lavage - WLL)` kunganikeza impumuzo enkulu.
• Ukulandela iseluleko sezokwelapha ngqo nokuphila ngendlela enempilo kusiza kakhulu ekulawuleni lesi sifo.

Uma uneminye imibuzo mayelana nalokhu, ungangabazi ukukhuluma nodokotela wakho. Hlala uphilile!

I- Pulmonary Alveolar Proteinosis, i-PAP, Isifo Samaphaphu, Ubunzima Bokuphefumula, i-Alveoli, Ama-Surfactants, Ukuhlanza Amaphaphu Aphelele