Ingabe ngezinye izikhathi uzizwa sengathi unenkinga yokuphefumula, sengathi isifuba sakho siqinile? Ingabe uzizwa ukhathele ngisho nangemva kokuhamba ibanga elifushane? Sivame ukukhohlwa ngalezi zinto, sicabanga ukuthi ziwukukhathala okuvamile. Kodwa-ke, namuhla sizokhuluma ngesimo esibucayi sezokwelapha esingaba yisisusa salezi zimpawu, nakuba singavamile kakhulu. Lokho yi-Pulmonary Alveolar Proteinosis, noma i-PAP ngamafuphi.
Kalula nje, iyini i-Pulmonary Alveolar Proteinosis (PAP)?
Ake sithi amaphaphu ethu agcwele izigidi zezikhwama zomoya ezincane. Lezi zikhwama zomoya ezincane sizibiza ngokuthi ama-alveoli . Uma siphefumula, yilapho sithola khona umoya-mpilo ngalezi zikhwama zomoya.
Manje, ku-PAP, okwenzekayo ukuthi amaprotheni, amafutha, nezinye izinto ziqoqana ngaphakathi kwalezi zikhwama zomoya ezincane, njengodoti. Isizathu salokhu ukuthi amangqamuzana ahlanza into efana namafutha ebizwa ngokuthi i-surfactant, ekhona ngokwemvelo ezindongeni zalezi zikhwama zomoya, awasebenzi kahle. Khona-ke le surfactant iyaqoqana futhi ivimbe izikhwama zomoya. Ngakho-ke, umoya awukwazi ukungena kahle, ngakho igazi alitholi inani lomoya eliwudingayo. Lokhu kubangela ubunzima ekuphefumuleni.
Lesi yisifo esibi kakhulu samaphaphu. Ngakho-ke, uma uzwa leli gama, ungase uzizwe wesaba. Kodwa ungakhathazeki, ngokwelashwa okufanele, lesi sifo kanye nezimpawu zaso kungalawulwa kahle. Into ebaluleke kakhulu ukubona udokotela wezokwelapha zamaphaphu bese uthola ukwelashwa.
Ingabe zikhona izinhlobo eziyinhloko ze-PAP?
Yebo, i-PAP ihlukaniswe kakhulu ngezinhlobo ezintathu. Imbangela yohlobo ngalunye ihlukile.
| Uhlobo lwe-PAP | Incazelo elula |
|---|---|
| I-Autoimmune PAP | Lolu uhlobo oluvame kakhulu. Luthinta ama-90% eziguli ze-PAP. Okwenzekayo lapha ukuthi amasosha ethu omzimba ahlasela ngephutha amaseli asiza ukugcina izindlela zokuphefumula zihlanzekile. |
| I-PAP yesibili | Lokhu kubangelwa esinye isimo sezokwelapha noma ukuchayeka ebuthi obufana nothuli namakhemikhali, okuthinta ukusebenza kwamaseli emigwaqweni yomoya. |
| I-Congenital PAP | Lesi yisimo sokuzalwa, esihambisana nofuzo. Singadluliselwa kusuka kubazali kuya ezinganeni. Ngezinye izikhathi izimpawu ziba khona lapho kuzalwa, kanti ngezinye izikhathi zingase zivele kamuva. |
Ubani osengozini enkulu yokuthola lesi sifo?
I-Autoimmune kanye ne-PAP yesibili ngokuvamile zithinta abantu abaneminyaka ephakathi kuka-30 no-60. Lezi zinhlobo ezimbili zivame kakhulu kwabesilisa . Abantu ababhemayo kanye nalabo abachayeka othulini noma kumakhemikhali basengozini enkulu.
I-Congenital PAP ivame kakhulu ezinganeni ezingaphansi kweminyaka eyi-10 ubudala, kodwa ingenzeka kunoma yimuphi ubudala.
Ziyini izimpawu zesifo se-PAP?
Uphawu oluyinhloko noluvame kakhulu lwalesi sifo ukuphelelwa umoya (ukuphelelwa umoya) . Iningi labantu liba nokuphelelwa umoya ngesikhathi sokuzivocavoca noma emsebenzini. Kodwa-ke, abanye abantu bangase babhekane nalobu bunzima ngisho noma bemi nje.
Ngaphezu kwalokho, ungabona lezi zimpawu:
- Ubuhlungu besifuba
- Ukukhwehlela (mhlawumbe nge-phlegm noma igazi)
- Ukushintsha kombala wesikhumba nezinzipho okuluhlaza okwesibhakabhaka (i-cyanosis) - Lokhu kwenzeka uma kungekho oxygen eyanele egazini.
- Ukukhathala okuqhubekayo
- Imfiva
- Ukutheleleka kwamaphaphu okuvamile
- Iminwe eboshwe nge-clubbed
- Ukwehlisa isisindo ngaphandle kwesizathu
Udokotela usithola kanjani lesi sifo?
Uma usola ukuthi unesifo se-PAP, udokotela wakho uzoqala akuhlole futhi alalele amaphaphu akho nge-stethoscope. Ngemuva kwalokho uzokubuza imibuzo mayelana nomlando wezokwelapha womndeni wakho kanye nendlela yakho yokuphila. Uma ubhema, ubhekene nothuli noma amakhemikhali, kufanele nakanjani umtshele udokotela wakho .
Ngemuva kwalokho, kungenziwa izivivinyo eziningana ukuqinisekisa lesi sifo:
- Ukuhlolwa kwegazi: Hlola izinga lomoya-mpilo egazini. Futhi, ukuhlolwa okuthile okufana ne -GM-CSF autoantibody kungenziwa ukuthola imbangela yesifo se-PAP.
- Ukuhlolwa kokusebenza kwamaphaphu: Kukala ukuthi amaphaphu akho asebenza kahle kangakanani.
- I-X-ray yesifuba noma i-CT scan: Ukuze ubone kahle isimo samaphaphu.
- I-Bronchoscopy:Inqubo lapho kufakwa khona ipayipi elincane elinekhamera enamathiselwe ngomlomo ngaphansi kwe-anesthesia ukuze kuhlolwe ingaphakathi lendlela yokuphefumula namaphaphu.
- I-biopsy yamaphaphu: Kuthathwa ingxenye encane yezicubu emaphashini ngesikhathi se-bronchoscopy noma ngesikhathi sokuhlinzwa ukuze kuhlolwe.
Yiziphi izindlela zokwelapha i-PAP?
Okwamanje akukho ukwelashwa kwe-PAP, kodwa kunezindlela zokwelapha ezisebenza kahle kakhulu ezingakusiza ukuthi ulawule izimpawu zakho futhi uphile impilo engcono.
Ukwelashwa okuyinhloko nokusetshenziswa kakhulu kwe-PAP yi- "Whole-Lung Lavage" (WLL) .
Kuyini lokhu kuhlanza amaphaphu?
Okwenzekayo kulokhu ukuthi udokotela usebenzisa i-bronchoscope (ithubhu lekhamera) ukugeza amaphaphu ngalinye nge-saline (amanzi anosawoti ahlanziwe) ukuze asuse amaprotheni namafutha avalelekile. Ngemva kwalokhu, kuba lula kakhulu ukuphefumula.
Uma wenza lokhu kwelashwa:
- Uzophelelwa yi-anesthesia ngokuphelele, ngakho ngeke uzizwe lutho.
- Udokotela ufaka i-bronchoscope ngomlomo bese eyikhomba ephashini elilodwa.
- Umoya-mpilo unikezwa kwelinye iphaphu ngomshini.
- Ngezinye izikhathi ugqoka ijazi eliqhaqhazelayo esifubeni sakho. Lokhu kudlidliza kukhulula izinto ezinamathele ezikhwameni zakho zomoya.
- Ngemuva kwalokho isixazululo sikasawoti sifakwa emaphashini, futhi uma sikhishwa, siphuma nezinto ebezinamathele.
- Mhlawumbe elinye iphaphu nalo liyagezwa ngesikhathi esifanayo, noma ngemva kwezinsuku ezimbalwa.
Kuye ngokuthi lesi sifo sibucayi kangakanani, lokhu kwelashwa kungadinga ukwenziwa njalo ezinyangeni ezimbalwa noma kanye ngonyaka. Kodwa-ke, abanye abantu bangase bangadingi lokhu kwelashwa. Lokho kuzonqunywa udokotela.
Ezinye izindlela zokwelapha
- Ama-Bronchodilator: Le mithi ikhulula imisipha ezungeze imigudu yomoya, okwenza kube lula ukuphefumula.
- Ukwelashwa kokufaka esikhundleni se-GM-CSF: Lokhu kuhogela nge-nebulizer noma kunikezwa njengomjovo. Kuthuthukisa ukusebenza kwamaseli ahlanza imigudu yomoya.
- Ukufakelwa amaphaphu: Abantu abanesifo esibi kakhulu futhi amaphaphu abo alimele bangadinga ukufakelwa amaphaphu anempilo njengesixazululo sokugcina.
- I-Plasmapheresis kanye nokushintshana kwe-plasma: Lokhu kuhilela ukususa ingxenye ewuketshezi yegazi (i-plasma) bese kufakwa i-plasma kumuntu ophilile. Ku-autoimmune PAP, lokhu kusiza ukuvimba amasosha omzimba ekuhlaseleni amaseli anempilo.
- I-oxygen eyengeziwe: I-oxygen inikezwa ngemaski noma ngepayipi elincane elifakwa ekhaleni ukuze kube lula ukuphefumula.
Yini engingayenza njengomuntu one-PAP?
Uma une-PAP, kubaluleke kakhulu ukulandela ukwelashwa okuyalelwe udokotela wakho futhi unakekele nendlela yakho yokuphila.
- Gwema ukubhema ngokuphelele futhi ugweme izindawo lapho abanye abantu bebhema khona.
- Thola imijovo ye-influenza (flu) kanye ne-pneumonia njengoba kunconywe udokotela wakho.
- Hlala kude nabantu abagulayo.
- Yidla ukudla okunempilo futhi ulawule isisindo sakho.
- Geza izandla zakho kahle ngemva kokusebenzisa indlu yangasese nangaphambi nangemuva kokudla.
- Khuluma nodokotela wakho bese udala uhlelo lokuzivocavoca oluphephile olufanela wena.
Umlayezo Wokuya Nawe Ekhaya
- I-Pulmonary Alveolar Proteinosis (PAP) yisimo esingavamile lapho amaprotheni namafutha eqoqana khona emasakeni omoya (ama-alveoli) amaphaphu.
- Uphawu oluyinhloko ubunzima bokuphefumula. Ngaphezu kwalokho, ukukhwehlela, ubuhlungu besifuba, kanye nokukhathala kungase kube khona.
- Nakuba lokhu kuyinto okufanele uyesabe, ukwelashwa okuphumelelayo kakhulu njenge-"Whole-Lung Lavage" kungasiza ekulawuleni izimpawu futhi kukuvumele ukuthi uphile impilo evamile.
- Uma unalezi zimpawu, kufanele nakanjani ubonane nochwepheshe wamaphaphu. Kubaluleke kakhulu ukuthola lesi sifo bese uqala ukwelashwa ngokushesha okukhulu.
- Ukugwema ukubhema nokuzivikela othulini nasentuthwini yamakhemikhali kungasiza kakhulu ekuzivikeleni kulesi sifo.
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