Yu dɔn gɛt dis prɔblɛm wit yu lɔng? (Pulmonary Alveolar Proteinosis - PAP) Lɛ wi tɔk bɔt dis!

Yu de fil se yu nɔ ebul fɔ blo smɔl? Ɔ yu jɔs taya? Sɔntɛnde, dɛn kin tek dɛn tin ya as nɔmal tin, bɔt nɔ kin apin so ɔltɛm, dɛn kin bi sayn dɛm fɔ wan sik na di lɔng. Tide wi go tɔk bɔt wan sik we nɔ kin bɔku bɔt we impɔtant fɔ no bɔt. dis dεn kכl am `(Pulmonary Alveolar Proteinosis)` כ `(PAP)`. Pan ɔl we di nem kin tan lɛk se i kɔmplikt smɔl, lɛ wi ɛksplen am simpul wan.

Wetin na `(Pulmonary Alveolar Proteinosis - PAP)`? Fɔ tɔk am simpul wan...

Okay, naw mek wi si wetin dis kondishon we dem kol `(PAP)` bi. Fɔ tɔk am simpul wan, na sik we nɔ kin apin so ɔltɛm we di prɔtin, fat, ɛn sɔm ɔda tin dɛn kin gɛda na di smɔl smɔl ay sak dɛn (alveoli) we de insay wi lɔng ɛn mek dɛn lɔk. Imajin, wi lɔng dɛn gɛt bɔku bɔku smɔl smɔl ɛya sak dɛn. Dɛn smɔl smɔl ay sak ya na wetin wi kɔl `alveoli` . We wi de blo, dɛn kin tek ɔksijɛn ɛn kɔmɔt insay di blɔd tru dɛn ay sak dɛn ya.

so, insay di kes fכ `(PAP)`, dεn ay sak dεm ya kin fulכp wit tin dεm we nכ nid, lεk drein we klos, we de blכk di we fכ כksijεn fכ rich di bכdi. Wetin kin apin da tɛm de? Wi blɔd nɔ kin gɛt bɛtɛ ɔksijɛn. Dis kin mek i nɔ izi fɔ blo (`dyspnea`) .

Dis na siriɔs lɔng sik. So na nɔmal tin fɔ fil smɔl frayd we yu gɛt diagnosis lɛk dis. Bɔt nɔ wɔri. If yu gɛt di rayt tritmɛnt, yu go ebul fɔ kɔntrol di sik ɛn di sayn dɛm we i kin gɛt. If yu gɛt `(PAP)`, i rili impɔtant fɔ go to pulmonary mɛrɛsin dɔktɔ fɔ tritmɛnt. I go advays yu bak bɔt wɛl bɔdi layf chenj dɛm we yu kin mek. Dɛn tin ya kin mek yu layf bɛtɛ.

Difrɛn kayn dis `(PAP)` sik de?

Yes, tri men kayn `(PAP)` sik de. Lɛ wi si wetin dɛn bi:

1. Awtoimyun PAP: Dis na di kayn PAP we kכmכn pas כl. Na lɛk 90% pan di big pipul dɛn kin gɛt dis kayn. Wetin kin apin ya na we wi bɔdi in yon imyun sistɛm kin mistek atak di sɛl dɛn we de ɛp fɔ mek di say dɛn we di briz de blo klin. Tink bɔt am as wi yon difens dɛn we de fɛt wi yon sɛl dɛn.

. Dat min se, i kin apin fɔ ɔda rizin.

3. PAP we dεn bכn pikin: Dis na kכndyushכn we de de we dεn bכn am. Sɔntɛnde, di sayn dɛm kin de we dɛn bɔn am, ɔr kin sho pan difrɛn ej dɛm. dis kin bi fכ chenj dεm na sכm jin dεm we de kכntro di wok we di sεl dεm na wi lכng dεm de du. Dis kin bi frɔm mama ɛn papa to pikin dɛn.

Udat kin gɛt dis `(PAP)` sik mɔ?

jεnarali, di tu tכp dεm we dεn kin afekt mכst na ``Autoimmune PAP'' εn ``Secondary PAP''.Fɔ pipul dɛn we ol bitwin 30 ɛn 60. Dɔn bak, dɛn tu kayn ya kin bɔku pan man dɛn . Pipul dɛn we de smok ɛn di wan dɛn we gɛt sɔm kayn dɔst ɔ kemikal dɛn kin gɛt dis sik.

di kayn we dεn kכl `(Congenital PAP)` dεn kin si am mכst pan pikin dεm we nכ rich 10. כltu, lεk aw wi bin dכn tכk, i kin apin pan εni ej.

Aw kɔmɔn tin na PAP sik?

Infakt, `(PAP)` na sik we nɔ kin bɔku. Ɔlsay na di wɔl, dis sik kin ambɔg lɛk fɔti pan tri milyɔn pipul dɛn. Dis min se na smɔl pipul dɛn we gɛt dis sik kin de na Sri Lanka.

Wetin mek dis `(PAP)` sik kin apin? Wetin na di kɔz?

As wi bin don tok bifo, di wol dεm na wi ay sak dεm (alveoli) dεn rili tכn. na tru dεn tin wכl dεm ya we כksijεn de pas frכm di lכng dεm go insay di bכdi. Di wɔl dɛn na dɛn ay sak dɛn ya kin gɛt wan layt ɔyl we dɛn kɔl sɔfaktant . Dɛn sɔfaynt dɛn ya kin ɛp fɔ mek di ay sak dɛn opin ɛn nɔ mek dɛn klos. Na da tɛm de ɔksijɛn kin pas izi wan insay di blɔd.

naw, insay wi lכng dεm, wan spεshal tכp sεl de we dεn kכl `alveolar macrophages` . Dɛn sɛl dɛn ya tan lɛk pipul dɛn we de wok fɔ klin. wetin dεn de du na fכ pul dεn `(surfactants)` dεm ya wan wan tεm frכm di ay sak dεm, fכ mek dεn nכ kכlכg.

כltu, wetin kin apin pan pכsin we gεt PAP na dat dεn sεl dεm ya we dεn kכl makrofag dεm nכ de gεt di signal fכ klin di wכl dεm na di ay sak dεm fayn fayn wan. Wetin kin apin da tɛm de? Dɛn sɔfaktant dɛn de kin bil ɛn blok di ay sak dɛn. Jɔs lɛk dreyn we dɔn klos na sink. Dɔn di we fɔ mek ɔksijɛn go na di blɔd kin blok.

fכ simpul wan, dis kכndyushכn de kכz fכ wik we di "trash cleaning system" fכ di lכng dεm.

Wetin na di sayn dɛm fɔ `(PAP)` sik?

Di men ɛn kɔmɔn sayn fɔ PAP na we yu nɔ de blo fayn (dyspnea) . Pan ɔl we bɔku pipul dɛn kin gɛt shɔt briz we dɛn de du ɛksɛsayz, sɔm pipul dɛn kin gɛt dis prɔblɛm ivin we dɛn jɔs tinap.

Apat frɔm dat, sɔm ɔda sayn dɛn de we de sho se yu gɛt dis sik:

• Fɔ fil pen na yu chɛst.
• Kɔf. Sɔntɛnde, smɔl blɔd kin kɔmɔt wit di mucus.
• di blu kכla na di skin εn di nel dεm (cyanosis) (dis kin apin we כksijεn nכ de na di bכdi).
• Fɔ fil taya ɔltɛm (taya).
• Fiva.
• Infεkshכn dεm na di lכng bכku tεm.
• di tip dεm fכ di finga dεm de big εn de tek wan shep we lεk dכm (`klub finga dεm`).
• We yu de lɔs yu wet we yu nɔ gɛt rizin.

If yu gɛt wan ɔr mɔr pan dɛn sik ya, i rili impɔtant fɔ go to dɔktɔ.

Yu tink se PAP sik kin kil pɔsin?

If i siriɔs, PAP kin mek yu nɔ ebul fɔ blo fayn we go mek yu layf de pan denja.Dis min se di lכng dεm nכ kin du dεn wok fayn fayn wan. Bɔt aw dis sik kin difrɛn frɔm wan pɔsin to ɔda pɔsin. Sɔm pipul kin wɛl frɔm di sik we dɛn nɔ gɛt ɛni tritmɛnt (`spontaneous remission`). Fɔ ɔda pipul dɛn, di sik kin stil de na di sem lɛvɛl. We i rili bad, day kin apin bikɔs i nɔ ebul fɔ blo fayn ɔ ɔda infɛkshɔn. Na dat mek i impɔtant fɔ no di sik kwik kwik wan ɛn gɛt tritmɛnt.

Aw dɛn kin no se pɔsin gɛt PAP? Us tɛst dɛn dɛn kin du?

If dɔktɔ saspek se yu gɛt PAP, i go fɔs chɛk yu ɛn lisin to yu lɔng. I go aks yu kwɛstyɔn dɛn bak bɔt yu pasɔnal ɛn famili mɛdikal istri ɛn yu layf. If yu de smok ɔ yu gɛt dɔst ɔ kemikal, mek shɔ se yu tɛl yu dɔktɔ.

Test fɔ kɔnfɔm PAP kin inklud:

• Blɔd tɛst: Chɛk di ɔksijɛn lɛvɛl na yu blɔd.
• wan spεshal bכdi tεst: Luk fכ wan indikεtכ we dεn kכl `(granulocyte-macrophage colony-stimulating factor - GM-CSF) כtoantibodi`. Dis kin ɛp fɔ fɛn di kɔz fɔ `(PAP)` sik.
• Pulmonary function testing: Mek yu no aw yu lכng dεm de wok fayn fayn wan.
• Imej ɛgzam: Dɛn tin ya na fɔ tek ɛkstrem rayt na in chɛst ɔ CT skan. Dɛn tin ya kin mek wi no klia wan aw di lɔng dɛn de.
• brכnkoskopi: insay dis, dεn de put wan tכn tכb (wit kεmεra) tru di mכt insay di aywe dεm εn dεn de egzamin di insay pat pan di lכng dεm.
• di lכng bayopsi: dεn kin tek sכm sכm tisu frכm di lכng fכ egzamin, we dεn de du bכnkoskopi כ we dεn de du כpεrayshכn.

Dɛn tɛst ya na dɛn tin ya we de mek dɔktɔ dɛn ebul fɔ no di PAP kɔrɛkt wan.

Yu tink se kɔmplit mɛrɛsin de fɔ di sik `(PAP)`?

So fa, no mɛrɛsin nɔ de fɔ PAP. Bɔt nɔ wɔri. Bɔrku tritmɛnt de wae kin ɛp yu fɔ kɔntrol yu sik ɛn liv bɛtɛ layf.

Aw dɛn kin trit PAP sik?

Di men ɛn di tritmɛnt we dɛn kin yuz mɔ fɔ PAP na Whole-Lung Lavage (WLL) . Dɛn kin kɔl dis bak "lung lavage." Tink bɔt am lɛk we yu de was yu lɔng.

Dɔktɔ kin yuz brɔnkoskɔp (di tin tiub we gɛt kamɛra) ɛn salin sɔlshɔn (stɛriyl sɔl wata) fɔ klin wan lɔng wan wan tɛm. Dis go mek am izi fɔ yu fɔ blo. Dis prɔses kin tek lɛk 4 to 5 awa so. Dipen pan aw yu sik kin tranga, yu kin nid fɔ gɛt WLL ɛvri sɔm mɔnt ɔ wan tɛm insay di ia. Bɔt sɔm PAP pasɛnt dɛn nɔ kin nid WLL. Dis kin dipen pan aw yu sik kin tranga.

Wetin kin apin we yu du wan `(Whole-Lung Lavage - WLL)`?

• Dɛn go gi yu jenɛral anestezi ɔ mɛrɛsin fɔ mek yu fil fayn.
• Di dɔktɔ kin put brɔnkoskɔp tru yu mɔt insay yu aywe ɛn pɔynt am insay wan pan yu lɔng dɛn.
• Wan mashin de gi ɔksijɛn to di ɔda lɔng.
• Yu wɛr vest we de shek shek rawnd yu chɛst. Dis vaybreshɔn de mek di sɔfaynt dɛn we dɔn trɔp na di ay sak dɛn, lɔs.
• Dɔn di dɔktɔ kin injɛkt di salin sɔlvushɔn insay di lɔng dɛn ɛn pul am bak kɔmɔt (suk) wit di sɔfaktant dɛn.
• Sɔntɛnde, dɛn kin was di ɔda lɔng di sem tɛm, ɔ dɛn kin was am fɔ sɔm dez.

Apat frɔm dis `(WLL)` tritmɛnt, ɔda tritmɛnt dɛn de:

• Bronchodilators: Dɛn mɛrɛsin ya kin mek di mɔsul dɛn we de rawnd yu aywe rilaks, ɛn dis kin mek i izi fɔ yu fɔ blo.
• Granulocyte-macrophage colony-stimulating factor (GM-CSF) riplesmεnt tεrapi: dεn kin gi dis bay we dεn inhal tru nebulizer (divays we de tכn likwid mεdisin to vapour) כ as injεkshכn. dis tritmεnt de wok bay we i de mek di sεl dεm we de klin di aywe dεm (macrophages) de wok fayn.
• Lɔng transplant: Pipul dɛn we gɛt siriɔs PAP sik ɛn we gɛt lɔng damej kin nid fɔ gɛt lɔng transplant sɔntɛnde. Insay dis prɔsidyu, dɛn kin pul wan ɔ ɔl tu pan yu lɔng dɛn we gɛt sik ɛn put wan lɔng we kɔmɔt frɔm pɔsin we gɛt wɛlbɔdi.
• Plasmapheresis ɛn plasma exchange: Dis min se yu fɔ pul di wata we de na yu blɔd we dɛn kɔl plasma, ɛn put plasma we pɔsin we gɛt wɛlbɔdi gɛt fɔ gi yu blɔd. Fɔ pipul dɛm we gɛt ɔtoimyun PAP, dis tritmɛnt de ɛp fɔ mek di imyun sistɛm nɔ atak di wɛlbɔdi sɛl dɛm.
• Sɔplɛnt ɔksijɛn: Ɔksijɛn tritmɛnt kin ɛp yu fɔ blo izi wan. Dɛn kin gi am tru mask na yu fes ɔ smɔl tiub we dɛn put na yu nos.
• Klinik trial: Yu kin gɛt di chans bak fɔ tek pat pan risach bɔt nyu tritmɛnt dɛn. Tɔk to yu dɔktɔ bɔt dis.

Yu tink se dɛn kin ebul fɔ avɔyd PAP sik?

As wi bin dɔn tɔk, difrɛn kayn PAP dɛn de. So, dεn nכ kin ebul fכ sכpכt כtoimyun PAP כ Congenital PAP biכs dεn de riliyt to intanεt prכsεs dεm na wi bכdi.

Bɔt i pɔsibul fɔ mek dɛn nɔ du `(Sɛkɛnd PAP)` te to sɔm mak. Aw yu go du dat? Yu fɔ ridyus di we aw yu de yuz pɔyzin tin dɛn as yu ebul . If dat nɔ pɔsibul, yu fɔ yuz gud mask (`respirator` ɔ fes mask we fit fayn fayn wan) we de kɔba yu mɔt ɛn nos fayn fayn wan.

Wetin na di lukin-grɔn fɔ pɔsin we gɛt PAP?

Tritmɛnt ɔltɛm lɛk `(Whole-Lung Lavage - WLL)` kin ɛp yu fɔ blo izi ɛn pul ɔda sayn dɛm. Smɔl nɔmba pan pipul dɛn kin wɛl frɔm `(PAP)` we dɛn nɔ gɛt ɛni tritmɛnt. Bɔt if dɛn nɔ trit am, siriɔs `(PAP)` kɔndishɔn kin mek yu nɔ ebul fɔ blo fayn ɔ day. So, i rili impɔtant fɔ fala wetin yu dɔktɔ tɛl yu fɔ du.

If a gɛt PAP, wetin a go du fɔ misɛf?

If yu gɛt PAP, sɔm tin dɛn de we yu kin du fɔ mek yu gɛt wɛlbɔdi:

• Aks yu dɔktɔ bɔt klinik trial fɔ PAP tritmɛnt.
• Nɔ smok. Dɔn bak, nɔ blo sɛkɔnhand smok we ɔda pipul dɛn de smok .
• Fɔ fala yu dɔktɔ in instrɔkshɔn bɔt aw fɔ tek flu ɛn nyumonia shot.
• Stay away frɔm pipul dɛn we sik.
• It tin dɛn we gɛt fayn fayn tin dɛn fɔ it. Kɔntrol yu bɔdi wet.
• Was yu an fayn fayn wan afta yu dɔn yuz tɔylɛt, bifo ɛn afta yu it.
• Wok wit yu dɔktɔ fɔ mek wan sef ɛksɛsayz plan we go fayn fɔ yu.

Mɛmba se, Pulmonary Alveolar Proteinosis (PAP) na wan sik we nɔ kin bɔku na di lɔng we kin mek i nɔ izi fɔ blo ɛn ɔda sayn dɛn bay we i kin mek di ay sak dɛn na di lɔng dɛn blok. Bɔt sɔm fayn tritmɛnt dɛn de we kin ɛp fɔ kɔntrol di tin dɛn we PAP kin du. If yu gɛt dis sik, tink bɔt fɔ wok wit wan tim we gɛt bɔku spɛshal pipul dɛn. Tugɛda, dɛn kin ɛp fɔ mek yu layf bɛtɛ.

Take-Home Message (Di tin we impɔtant pas ɔl fɔ mɛmba)

Okay, so wi dɔn tɔk bɔku bɔt `(Pulmonary Alveolar Proteinosis - PAP)`. Pan ɔl we dis kin tan lɛk se i kɔmplikt smɔl, di men tin fɔ mɛmba na dat pan ɔl we dis na sik we nɔ kin apin so ɔltɛm, i impɔtant fɔ mek yu nɔ fred ɛn gɛt di rayt advays ɛn tritmɛnt.

• If yu gɛt sɔm sayn dɛn lɛk aw i nɔ kin izi fɔ blo, yu kin kɔf ɔltɛm, ɔ yu kin taya ɔltɛm, go to dɔktɔ.
• If dɛn no se yu gɛt `(PAP)`, go to tritmɛnt ɔnda di sɔpɔtishɔn fɔ pulmonɔlɔjis .
• Tritmɛnt dɛn de, so nɔ giv ɔp fɔ op. Tritmεnt lεk `(Whole-Lung Lavage - WLL)` kin gi bכku rilif.
• If yu fala di advays we dɔktɔ gi yu ɛn liv fayn layf, dat kin ɛp yu fɔ kɔntrol di sik.

If yu gɛt ɛni ɔda kwɛstyɔn bɔt dis, nɔ shek fɔ tɔk to yu dɔktɔ. Stay wit wɛlbɔdi!

` Pulmonary Alveolar Proteinosis, PAP, Lכng Sik, Brith Difikulti, Alveoli, Surfaktant, Wכl-Lכng Lavage