Ngaba ukhe waba nale ngxaki ngemiphunga yakho? (Pulmonary Alveolar Proteinosis - PAP) Masithethe ngale nto!

Ngaba uziva uphelelwa ngumphefumlo? Okanye udiniwe nje? Ngamanye amaxesha ezi zinto zithathwa njengesiqhelo, kodwa kwiimeko ezingaqhelekanga, zinokuba ziimpawu zesifo semiphunga. Namhlanje siza kuthetha ngesifo semiphunga esingaqhelekanga kodwa esibalulekileyo ekufuneka sisazi. Oku kubizwa ngokuba yi-`(Pulmonary Alveolar Proteinosis)` okanye `(PAP)`. Nangona igama lisenokuvakala liyinkimbinkimbi kancinci, masilichaze ngokulula.

Yintoni i-`(Pulmonary Alveolar Proteinosis - PAP)`? Ngamafutshane...

Kulungile, ngoku masibone ukuba le meko ibizwa ngokuba yi-`(PAP)` yintoni. Ngamafutshane, sisifo esingaqhelekanga esenzeka xa iiproteni, amafutha, kunye nezinye izinto ziqokelelana kwiingxowa zomoya ezincinci (i-alveoli) ngaphakathi kwemiphunga yethu zize zibangele ukuba zivaleke. Khawucinge nje, imiphunga yethu yenziwe zizigidi zeengxowa zomoya ezincinci. Ezi ngxowa zomoya zincinci zezo sizibiza ngokuba yi-`alveoli` . Xa siphefumla, ioksijini ithathwa ize ikhutshwe egazini ngezi ngxowa zomoya.

Ngoko ke, kwimeko ye-`(PAP)`, ezi ngxowa zomoya zizaliswa zizinto ezingafunekiyo, njengomsele ovalekileyo, ovala indlela yokuba ioksijini ifikelele egazini. Kwenzeka ntoni ke ngoko? Igazi lethu alifumani ioksijini eyaneleyo. Oku kunokubangela ubunzima bokuphefumla (`dyspnea`) .

Esi sisifo esibi kakhulu semiphunga. Ngoko ke kuqhelekile ukuba uzive usoyika kancinci xa ufumana isifo esifana nesi. Kodwa ungakhathazeki. Ngonyango olufanelekileyo, ungalawula esi sifo kunye neempawu zaso. Ukuba une-`(PAP)`, kubaluleke kakhulu ukubona ugqirha wezonyango zemiphunga ukuze ufumane unyango. Uya kukucebisa nangotshintsho olusempilweni olunokulwenza kwindlela ophila ngayo. Oku kunokuphucula umgangatho wobomi bakho.

Ngaba kukho iintlobo ezahlukeneyo zesi sifo `(PAP)`?

Ewe, kukho iintlobo ezintathu eziphambili zesifo `(PAP)`. Masibone ukuba zeziphi:

1. I-Autoimmune PAP: Olu lolona hlobo luqhelekileyo lwe-PAP. Malunga nama-90% abantu abadala bakhula ngolu hlobo. Okwenzekayo apha kukuba inkqubo yomzimba wethu yokuzikhusela ihlasela ngempazamo iiseli ezinceda ukugcina iindlela zomoya zicocekile. Cinga ngayo njengokhuselo lwethu olulwa neeseli zethu.

2. I-PAP yesibini: Olu hlobo lwenzeka xa umsebenzi weeseli kwimigudu yethu yomoya uchaphazeleka, nokuba kungenxa yesifo esithile okanye ukuvezwa yinto enobuthi (umz. uthuli, iikhemikhali). Oko kukuthi, kwenzeka ngesizathu esinye.

3. I-Congenital PAP: Le yimeko ekhoyo xa umntwana ezalwa. Ngamanye amaxesha iimpawu zinokubakho xa umntwana ezalwa, okanye zinokuvela kwiminyaka eyahlukeneyo. Oku kungenxa yotshintsho kwiijini ezithile ezilawula ukusebenza kweeseli kwimiphunga yethu. Oku kunokuzuzwa kubazali ukuya ebantwaneni.

Ngubani onokuba nethuba elingcono lokufumana esi sifo `(PAP)`?

Ngokubanzi, iintlobo ezimbini ezichaphazeleka kakhulu yi-"Autoimmune PAP" kunye ne-"Secondary PAP".Kubantu abaphakathi kweminyaka engama-30 ukuya kuma-60 ubudala. Kwakhona, ezi ntlobo zimbini zixhaphake kakhulu kumadoda . Abantu abatshayayo kunye nabo bachatshazelwa ziintlobo ezithile zothuli okanye iikhemikhali basengozini enkulu yokuhlaselwa sesi sifo.

Uhlobo olubizwa ngokuba yi-`(Congenital PAP)` lubonakala kakhulu kubantwana abangaphantsi kweminyaka eli-10 ubudala. Nangona kunjalo, njengoko bekutshiwo ngaphambili, lunokwenzeka nakweyiphi na iminyaka.

Ixhaphake kangakanani isifo se-PAP?

Enyanisweni, `(PAP)` sisifo esingaqhelekanga. Kwihlabathi liphela, esi sifo sichaphazela abantu abamalunga namashumi amane kwizigidi ezintathu. Oku kuthetha ukuba kusenokwenzeka ukuba kukho inani elincinci kakhulu labantu abanale sifo eSri Lanka.

Kutheni esi sifo `(PAP)` sisenzeka? Yintoni unobangela?

Njengoko besikhe sathetha ngaphambili, iindonga zeengxowa zethu zomoya (i-alveoli) zincinci kakhulu. Kuphela kwezi ndonga zincinci apho ioksijini idlula khona isuka emiphungeni iye egazini. Iindonga zezi ngxowa zomoya ngokwendalo zinomaleko weoyile ebizwa ngokuba zii-surfactants . Ezi ngxowa zinceda ukugcina iingxowa zomoya zivulekile ngaphandle kokuzivala. Kulapho ke ioksijini inokudlula khona ngokulula egazini.

Ngoku, ngaphakathi emiphungeni yethu, kukho uhlobo oluthile lweeseli ezibizwa ngokuba yi -'alveolar macrophages' . Ezi seli zifana nabasebenzi bokucoca. Into abayenzayo kukususa ezi '(surfactants)' rhoqo kwiingxowa zomoya, ukuze zingavaleki.

Nangona kunjalo, okwenzekayo kumntu one-PAP kukuba ezi seli zibizwa ngokuba zii-macrophages azifumani signal yokucoca iindonga zeengxowa zomoya ngokufanelekileyo. Kwenzeka ntoni ke ngoko? Ezo zixhobo zokucoca umoya ziyakha kwaye zivale iingxowa zomoya. Njengedreyini evalekileyo kwisinki. Emva koko indlela yokuba ioksijini ifike egazini iyavalwa.

Ngamafutshane, le meko ibangelwa bubuthathaka "kwinkqubo yokucoca inkunkuma" yemiphunga.

Zithini iimpawu zesifo `(PAP)`?

Uphawu oluphambili noluqhelekileyo lwe-PAP kukuphefumla kancinci (ukuphelelwa ngamandla) . Nangona uninzi lwabantu lufumana ukuphefumla kancinci ngexesha lokuzilolonga, abanye abantu banokuhlangabezana nolu bunzima nokuba bame nje.

Ukongeza, kukho nezinye iimpawu ezininzi:

• Ukuva iintlungu esifubeni.
• Ukukhohlela. Ngamanye amaxesha kunokubakho igazi elincinci eliphuma kunye nomkrwelo.
• Ukutshintsha kombala oluhlaza okwesibhakabhaka kolusu kunye neenzipho (i-cyanosis) (oku kwenzeka xa kungekho oxygen yaneleyo egazini).
• Ukuziva udiniwe ngalo lonke ixesha (ukudinwa).
• Ifiva.
• Usulelo oluqhelekileyo lwemiphunga.
• Iincam zeminwe ziyakhula zize zibe ngathi yidrama ('iminwe ebambeneyo').
• Ukunciphisa umzimba ngaphandle kwesizathu.

Ukuba unenye okanye ngaphezulu kwezi mpawu, kubaluleke kakhulu ukufuna ingcebiso kugqirha.

Ngaba isifo se-PAP singabulala?

Kwiimeko ezinzima kakhulu, i-PAP inokubangela ukungaphumeleli kokuphefumla okusongela ubomi.Oku kuthetha ukuba imiphunga ayikwazi ukwenza umsebenzi wayo ngokufanelekileyo. Nangona kunjalo, uhlobo lwesi sifo luyahluka kumntu nomntu. Abanye abantu banokuphila kwesi sifo ngaphandle konyango (`ukukhululwa okuzenzekelayo`). Kwabanye, esi sifo sihlala sikwinqanaba elifanayo. Kwiimeko ezinzima ngakumbi, ukufa kunokubangelwa kukusilela kokuphefumla okanye ezinye izifo. Yiyo loo nto kubalulekile ukufumanisa isifo kwangethuba kwaye ufumane unyango.

Ifunyaniswa njani i-PAP? Zeziphi iimvavanyo ezenziwayo?

Ukuba ugqirha ukrokrela ukuba une-PAP, uza kuqala akuhlole aze amamele imiphunga yakho. Uza kukubuza nemibuzo malunga nembali yakho yezonyango kunye neyosapho kunye nendlela ophila ngayo. Ukuba utshaya okanye ujongene nothuli okanye iikhemikhali, qiniseka ukuba uxelele ugqirha wakho.

Uvavanyo lokuqinisekisa i-PAP lungabandakanya:

• Uvavanyo lwegazi: Jonga amanqanaba eoksijini egazini lakho.
• Uvavanyo lwegazi olukhethekileyo: Khangela isalathisi esibizwa ngokuba yi-`(granulocyte-macrophage colony-stimulating factor - GM-CSF) autoantibody`. Oku kunceda ekufumaneni unobangela wesifo se-`(PAP)`.
• Uvavanyo lokusebenza kwephaphu: Linganisa ukuba imiphunga yakho isebenza kakuhle kangakanani.
• Uvavanyo lwemifanekiso: Olu luquka i-X-ray yesifuba okanye i-CT scan. Olu vavanyo lunokubonelela ngomfanekiso ocacileyo wemeko yemiphunga.
• I-Bronchoscopy: Kule nto, kufakwa ityhubhu encinci (enekhamera) ngomlomo iye kwimibhobho yomoya kwaye ingaphakathi lemiphunga liyahlolwa.
• I-biopsy yemiphunga: Kuthathwa iqhekeza elincinci lesicubu esiphuma emphungeni ukuze sihlolwe, nokuba ngexesha le-bronchoscopy okanye ngexesha lotyando.

Olu vavanyo luvumela oogqirha ukuba baxilonge i-PAP ngokuchanekileyo.

Ngaba ikho indlela yokunyanga esi sifo ngokupheleleyo `(PAP)`?

Okwangoku, akukho nyango lwe-PAP. Kodwa ungakhathazeki. Kukho unyango oluninzi olunokukunceda ulawule iimpawu zakho kwaye uphile ubomi obungcono.

Isifo se-PAP sinyangwa njani ?

Unyango oluphambili nolusetyenziswa kakhulu kwi-PAP yi -Whole-Lung Lavage (WLL) . Oku kukwabizwa ngokuba yi-"lung lavage." Cinga ngayo njengokuhlamba imiphunga yakho.

Ugqirha usebenzisa i-bronchoscope (ityhubhu encinci enekhamera) kunye nesisombululo se-saline (amanzi anetyuwa angeyiyo intsholongwane) ukucoca umphunga omnye ngexesha. Oku kuya kwenza kube lula kuwe ukuphefumla. Le nkqubo ingathatha malunga neeyure ezine ukuya kwezintlanu. Ngokuxhomekeke kubukhali beempawu zakho, unokufuna ukwenza i-WLL rhoqo emva kweenyanga ezimbalwa okanye kube kanye ngonyaka. Nangona kunjalo, abanye abaguli be-PAP basenokungayidingi i-WLL. Oku kuxhomekeke kubukhali bemeko yakho.

Kwenzeka ntoni xa usenza i-`(Whole-Lung Lavage - WLL)`?

• Uza kunikwa amayeza okuthomalalisa okanye amayeza okuthomalalisa umzimba.
• Ugqirha ufaka i-bronchoscope ngomlomo wakho kwi-airway yakho aze ayikhombe kwenye yemiphunga yakho.
• Umatshini unika ioksijini komnye umphunga.
• Unxiba ivesti engcangcazelayo esifubeni sakho. Oku kungcangcazela kukhulula izinto ezifakwe kwi-air bag.
• Emva koko ugqirha ufaka isisombululo se-saline emiphungeni aze asikhuphe kwakhona (ii-suction) kunye ne-surfactants.
• Ngamanye amaxesha omnye umphunga unokuhlanjwa ngaxeshanye, okanye ungahlanjwa iintsuku ezimbalwa.

Ukongeza kolu nyango lwe-`(WLL)`, kukho ezinye iindlela zonyango:

• Iibronchodilators: La mayeza aphumza izihlunu ezijikeleze indlela yakho yomoya, nto leyo eyenza kube lula kuwe ukuphefumla.
• Unyango lokubuyisela i-Granulocyte-macrophage colony-stimulating factor (GM-CSF): Olu nyango lunikezelwa nokuba ngokuphefumla nge-nebulizer (isixhobo esiguqula amayeza olwelo abe ngumphunga) okanye njengenaliti. Olu nyango lusebenza ngokuphucula ukusebenza kweeseli ezicoca iindlela zomoya (ii-macrophages).
• Ukufakelwa imiphunga: Abantu abanesifo se-PAP esibi kunye nomonakalo wemiphunga ngamanye amaxesha banokufuna ukufakelwa imiphunga. Kule nkqubo, omnye okanye yomibini imiphunga yakho enesifo iyasuswa kwaye ithathelwe indawo yimiphunga evela kumntu ophilileyo.
• I-Plasmapheresis kunye nokutshintshiselana nge-plasma: Oku kuquka ukususa inxalenye yolwelo egazini lakho, ebizwa ngokuba yi-plasma, uze uyifake endaweni yayo i-plasma evela kumntu ophilileyo. Kubantu abane-autoimmune PAP, olu nyango lunceda ekuthinteleni inkqubo yomzimba yokuzikhusela ekuhlaseleni iiseli eziphilileyo.
• Ioksijini eyongezelelweyo: Unyango lweoksijini lunokukunceda uphefumle lula. Lunokunikwa ngemaski ebusweni bakho okanye ngetyhubhu encinci efakwe empumlweni yakho.
• Uvavanyo lwezonyango: Unokufumana ithuba lokuthatha inxaxheba kuphando malunga nonyango olutsha. Thetha nogqirha wakho ngale nto.

Ngaba isifo se-PAP singathintelwa?

Njengoko besitshilo ngaphambili, kukho iintlobo ezahlukeneyo ze-PAP. Ngoko ke, akukho PAP ye-Autoimmune okanye i-Congenital PAP ezinokuthintelwa kuba zinxulumene neenkqubo zangaphakathi emzimbeni wethu.

Nangona kunjalo, kunokwenzeka ukuthintela `(i-Secondary PAP)` ukuya kuthi ga kwinqanaba elithile. Ungakwenza njani oko? Kufuneka unciphise ukuvezwa kwakho zizinto ezinobuthi kangangoko kunokwenzeka . Ukuba oko akunakwenzeka, kufuneka usebenzise imaski elungileyo (`i-respirator` okanye i-facemask efakwe kakuhle) egquma umlomo nempumlo yakho ngokufanelekileyo.

Injani imbono yomntu one-PAP?

Unyango oluqhelekileyo olufana ne-`(Whole-Lung Lavage - WLL)` lunokukunceda uphefumle lula kwaye lunciphise nezinye iimpawu. Inani elincinci labantu liyaphila kwi-`(PAP)` ngaphandle konyango. Nangona kunjalo, ukuba alunyangwa, iimeko ezinzima ze-`(PAP)` zinokukhokelela ekusileleni kokuphefumla okanye ekufeni. Ke ngoko, kubaluleke kakhulu ukulandela imiyalelo kagqirha wakho.

Ukuba ndine-PAP, ndingenza ntoni mna?

Ukuba une-PAP, kukho izinto ezininzi onokuzenza ukuze ugcine impilo yakho isempilweni:

• Buza ugqirha wakho malunga novavanyo lweklinikhi lonyango lwe-PAP.
• Musa ukutshaya. Kwakhona, musa ukuphefumla umsi osetyenziswa ngabanye abantu xa abanye abantu betshaya .
• Landela imiyalelo kagqirha wakho malunga nokufumana imijovo yomkhuhlane kunye ne-pneumonia.
• Hlala kude nabantu abagulayo.
• Yitya ukutya okunesondlo. Lawula ubunzima bomzimba wakho.
• Hlamba izandla zakho kakuhle emva kokusebenzisa indlu yangasese, ngaphambi nasemva kokutya.
• Sebenzisana nogqirha wakho ukuze uyile isicwangciso sokuzilolonga esikhuselekileyo esikulungeleyo.

Khumbula, i-Pulmonary Alveolar Proteinosis (PAP) sisifo esingaqhelekanga semiphunga esinokubangela ubunzima bokuphefumla kunye nezinye iimpawu ngokubangela ukuba iingxowa zomoya ezikwimiphunga zivaleke. Nangona kunjalo, kukho unyango oluninzi olusebenzayo olunokunceda ukulawula iziphumo ze-PAP. Ukuba unale meko, cinga ngokusebenza neqela leengcali ezininzi. Xa bebonke, banokukunceda ukuphucula umgangatho wobomi bakho.

Umyalezo Omele Uwuthathe Uhambe Nawe Ekhaya (Eyona nto ibalulekileyo ekufuneka uyikhumbule)

Kulungile, ngoko ke sithethe kakhulu nge-`(Pulmonary Alveolar Proteinosis - PAP)`. Nangona oku kungabonakala kunzima kancinci, into ephambili ekufuneka uyikhumbule kukuba nangona esi sisifo esingaqhelekanga, kubalulekile ukuba ungoyiki kwaye ufumane iingcebiso zonyango ezifanelekileyo kunye nonyango.

• Ukuba uneempawu ezifana nobunzima bokuphefumla, ukukhohlela okungapheliyo, okanye ukudinwa rhoqo, bona ugqirha.
• Ukuba ufunyaniswe une-`(PAP)`, funa unyango phantsi kweliso lengcali yemiphunga .
• Kukho unyango olufumanekayo, ngoko ke musa ukuphelelwa lithemba. Unyango olufana no `(Whole-Lung Lavage - WLL)` lunokubonelela ngoncedo olukhulu.
• Ukulandela iingcebiso zonyango ngokuchanekileyo nokuphila ubomi obusempilweni kunceda kakhulu ekulawuleni esi sifo.

Ukuba unemibuzo engaphezulu malunga noku, ungathandabuzi ukuthetha nogqirha wakho. Hlala usempilweni!

I- Pulmonary Alveolar Proteinosis, i-PAP, isifo semiphunga, ubunzima bokuphefumla, i-Alveoli, ii-Surfactants, i-Whole-Lung Lavage