Ngaba ngamanye amaxesha uziva ngathi unengxaki yokuphefumla, ngathi isifuba sakho siqinile? Ngaba uziva udiniwe nasemva kokuhamba umgama omfutshane? Sihlala sizilibala ezi zinto, sicinga ukuba kukudinwa nje okuqhelekileyo. Nangona kunjalo, namhlanje siza kuthetha ngemeko yezonyango enzulu enokubangela ezi mpawu, nangona zingaqhelekanga. Oko yiPulmonary Alveolar Proteinosis, okanye iPAP ngamafutshane.
Ngamafutshane, yintoni i-Pulmonary Alveolar Proteinosis (PAP)?
Khawuthelekelele ukuba imiphunga yethu izaliswe zizigidi zeengxowa zomoya ezincinci. Ezi ngxowa zomoya zincinci sizibiza ngokuba yi-alveoli . Xa siphefumla, sifumana ioksijini ngezi ngxowa zomoya.
Ngoku, kwi-PAP, okwenzekayo kukuba iiproteni, amafutha, nezinye izinto ziqokelelana ngaphakathi kwezi ngxowa zomoya zincinci, njengenkunkuma. Isizathu soku kukuba iiseli ezicoca into efana neoyile ebizwa ngokuba yi- surfactant, ekhoyo ngokwendalo eludongeni lwezi ngxowa zomoya, azisebenzi kakuhle. Emva koko le surfactant iyaqokelelana ize ivale iingxowa zomoya. Ngoko ke, umoya awunakungena ngokufanelekileyo, ngoko ke igazi alifumani umlinganiselo weoksijini eliwufunayo. Oku kubangela ubunzima ekuphefumleni.
Esi sisifo esibi kakhulu semiphunga. Ngoko ke, xa usiva eli gama, ungaziva usoyika. Kodwa ungakhathazeki, ngonyango olufanelekileyo, esi sifo kunye neempawu zaso zinokulawulwa kakuhle. Eyona nto ibalulekileyo kukubona ugqirha weyeza lemiphunga uze ufumane unyango.
Ngaba kukho iintlobo eziphambili ze-PAP?
Ewe, i-PAP yahlulwe kakhulu yaba ziintlobo ezintathu. Unobangela wohlobo ngalunye wahlukile.
| Uhlobo lwe-PAP | Ingcaciso elula |
|---|---|
| I-Autoimmune PAP | Olu lolona hlobo luxhaphakileyo. Luchaphazela ama-90% ezigulane ze-PAP. Okwenzekayo apha kukuba inkqubo yethu yokuzikhusela komzimba ihlasela iiseli ezinceda ukugcina iindlela zomoya zicocekile. |
| I-PAP yesibini | Oku kubangelwa yenye imeko yezonyango okanye ukuchatshazelwa ziityhefu ezifana nothuli kunye neekhemikhali, ezichaphazela ukusebenza kweeseli kwindlela yomoya. |
| I-Congenital PAP | Le yimeko yokuzalwa, yemfuza. Ingadluliselwa kubazali ukuya ebantwaneni. Ngamanye amaxesha iimpawu ziyabonakala xa umntwana ezalwa, ngelixa ngamanye amaxesha zinokuvela kamva. |
Ngubani osengozini enkulu yokuhlaselwa sesi sifo?
I-Autoimmune kunye ne-PAP yesibini zihlala zichaphazela abantu abaphakathi kweminyaka engama-30 ukuya kwengama-60. Ezi ntlobo zimbini zixhaphake kakhulu kumadoda . Abantu abatshayayo kunye nabo bachanabeke eluthulini okanye kwiikhemikhali basengozini enkulu.
I-Congenital PAP ixhaphake kakhulu kubantwana abangaphantsi kweminyaka eli-10 ubudala, kodwa inokwenzeka nakweyiphi na iminyaka.
Zithini iimpawu zesifo se-PAP?
Uphawu oluphambili noluqhelekileyo lwesi sifo kukuphefumla kancinci (dyspnea) . Uninzi lwabantu lufumana ukuphefumla kancinci ngexesha lokuzilolonga okanye emsebenzini. Nangona kunjalo, abanye abantu banokuhlangabezana nolu bunzima nangona bemi nje.
Ukongeza, unokubona ezi mpawu:
- Iintlungu zesifuba
- Ukukhwehlela (mhlawumbi kunye ne-phlegm okanye igazi)
- Ukutshintsha kombala oluhlaza okwesibhakabhaka kolusu kunye neenzipho (i-cyanosis) - Oku kwenzeka xa kungekho oxygen yaneleyo egazini.
- Ukudinwa rhoqo
- Ifiva
- Usulelo oluqhelekileyo lwemiphunga
- Iminwe eneeklabhu
- Ukunciphisa umzimba ngaphandle kwesizathu
Ugqirha uxilonga njani esi sifo?
Ukuba ukrokrela ukuba unesifo se-PAP, ugqirha wakho uza kuqala akuhlole aze amamele imiphunga yakho nge-stethoscope. Emva koko uza kukubuza imibuzo malunga nembali yezonyango yosapho lwakho kunye nendlela ophila ngayo. Ukuba uyatshaya, ujongene nothuli okanye iikhemikhali, ngokuqinisekileyo kufuneka umxelele ugqirha wakho .
Emva koko, kunokwenziwa iimvavanyo ezininzi ukuqinisekisa isifo:
- Uvavanyo lwegazi: Jonga inqanaba leoksijini egazini. Kwakhona, uvavanyo oluthile olufana ne -GM-CSF autoantibody lunokwenziwa ukuze kufunyanwe unobangela wesifo se-PAP.
- Uvavanyo lokusebenza kwephaphu: Lulinganisa indlela esebenza kakuhle ngayo imiphunga yakho.
- I-X-ray yesifuba okanye i-CT scan: Ukuze ubone kakuhle imeko yemiphunga.
- I-Bronchoscopy:Inkqubo apho kufakwa ityhubhu encinci enekhamera ngomlomo phantsi kwe-anesthesia ukuze kuhlolwe ingaphakathi lendlela yomoya kunye nemiphunga.
- I-biopsy yemiphunga: Iqhekeza elincinci lesicubu lithathwa emphungeni ngexesha le-bronchoscopy okanye ngexesha lotyando ukuze lihlolwe.
Ziziphi iindlela zonyango lwe-PAP?
Okwangoku akukho nyango lwe-PAP, kodwa kukho unyango olusebenzayo kakhulu olunokukunceda ulawule iimpawu zakho kwaye uphile ubomi obungcono.
Unyango oluphambili nolusetyenziswa kakhulu kwi-PAP yi- "Whole-Lung Lavage" (WLL) .
Yintoni le nto yokuhlamba imiphunga?
Okwenzekayo koku kukuba ugqirha usebenzisa i-bronchoscope (ityhubhu yekhamera) ukuhlamba imiphunga nganye nganye nge-saline (amanzi anetyuwa ahlanjululweyo) ukususa iiproteni kunye namafutha avalelekileyo. Emva koku, kuba lula kakhulu ukuphefumla.
Xa usenza olu nyango:
- Uza kupholiswa ngokupheleleyo yintlungu, ngoko awuyi kuva nto.
- Ugqirha ufaka i-bronchoscope ngomlomo aze ayibhekise kumphunga omnye.
- Ioksijini inikwa omnye umphunga ngumatshini.
- Ngamanye amaxesha unxiba ivesti engcangcazelayo esifubeni sakho. Oku kungcangcazela kukhulula izinto ezibambeke kwiingxowa zomoya zakho.
- Emva koko isisombululo se-saline sifakwa emiphungeni, kwaye xa sikhutshwa, siphuma nezinto ebezibambekile.
- Mhlawumbi nomnye umphunga nawo uyahlanjwa ngaxeshanye, okanye emva kweentsuku ezimbalwa.
Ngokuxhomekeke kubukhulu besifo, olu nyango lunokufuneka lwenziwe rhoqo emva kweenyanga ezimbalwa okanye kube kanye ngonyaka. Nangona kunjalo, abanye abantu basenokungaludingi olu nyango. Oko kuya kugqitywa ngugqirha.
Ezinye iindlela zonyango
- IiBronchodilators: La mayeza aphumza izihlunu ezijikeleze indlela yomoya, nto leyo eyenza kube lula ukuphefumla.
- Unyango lokutshintshwa kwe-GM-CSF: Olu hlobo lonyango luphefumlwa nge-nebulizer okanye lunikwe njengenaliti. Luphucula ukusebenza kweeseli ezicoca iindlela zomoya.
- Ukufakelwa imiphunga: Abantu abanesifo esibi kakhulu kwaye imiphunga yabo yonakele banokufuna ukufakelwa imiphunga esempilweni njengesisombululo sokugqibela.
- I-Plasmapheresis kunye nokutshintshiselana nge-plasma: Oku kuquka ukususa inxalenye yolwelo egazini (i-plasma) kunye nokuyitshintsha nge-plasma evela kumntu ophilileyo. Kwi-autoimmune PAP, oku kunceda ekuthinteleni inkqubo yomzimba yokuzikhusela ekuhlaseleni iiseli eziphilileyo.
- Ioksijini eyongezelelweyo: Ioksijini inikwa ngemaski okanye ngetyhubhu encinci efakwe empumlweni ukuze kube lula ukuphefumla.
Ndingenza ntoni njengomntu one-PAP?
Ukuba une-PAP, kubaluleke kakhulu ukulandela unyango oluyalelwe ngugqirha wakho kwaye unakekele nendlela ophila ngayo.
- Kuphephe ukutshaya ngokupheleleyo kwaye kuphephe ukuba kwiindawo apho abanye abantu batshaya khona.
- Fumana imijovo ye-influenza (flu) kunye ne-pneumonia njengoko ugqirha wakho ekucebisile.
- Hlala kude nabantu abagulayo.
- Yitya ukutya okunesondlo kwaye ulawule ubunzima bakho.
- Hlamba izandla zakho kakuhle emva kokusebenzisa indlu yangasese nangaphambi kokutya nasemva kokutya.
- Thetha nogqirha wakho uze wenze isicwangciso sokuzilolonga esikhuselekileyo esikufaneleyo.
Umyalezo Wokuya Ekhaya
- I-Pulmonary Alveolar Proteinosis (PAP) yimeko engaqhelekanga apho iiproteni kunye namafutha ziqokelelana kwiingxowa zomoya (ii-alveoli) zemiphunga.
- Uphawu oluphambili kubunzima bokuphefumla. Ukongeza, ukukhwehlela, iintlungu esifubeni, kunye nokudinwa kunokubakho.
- Nangona oku kuyinto yokoyika, unyango olusebenzayo kakhulu olufana ne-"Whole-Lung Lavage" lunokunceda ukulawula iimpawu kwaye lukuvumele ukuba uphile ubomi obuqhelekileyo.
- Ukuba unale mpawu, ngokuqinisekileyo kuya kufuneka ubone ingcali yemiphunga. Kubaluleke kakhulu ukufumanisa isifo kwaye uqale unyango ngokukhawuleza.
- Ukuphepha ukutshaya nokuzikhusela eluthulini nakwimiphunga yeekhemikhali kunokunceda ekuzikhuseleni kwesi sifo.
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