So woanya saa ɔhaw yi wɔ w’ahurututu ho? (Pulmonary Alveolar Proteinosis - PAP) Momma yɛnka eyi ho asɛm!

So wote nka sɛ wo home yɛ wo yaw kakra? Anaasɛ woabrɛ kɛkɛ? Ɛtɔ mmere bi a wobu saa nneɛma yi sɛ ɛyɛ nea ɛfata, nanso wɔ tebea horow a ɛntaa nsi mu no, ebetumi ayɛ ahurututu mu yare ho sɛnkyerɛnne. Ɛnnɛ yɛrebɛka ahurututu mu yareɛ bi a ɛntaa nsi nanso ɛho hia a ɛho hia sɛ yɛhunu ho asɛm. Wɔfrɛ eyi `(Ahurututu mu Ntini mu Proteinosis)` anaa `(PAP)`. Ɛwom sɛ ebia edin no bɛyɛ te sɛ nea ɛyɛ den kakra de, nanso momma yɛnkyerɛkyerɛ mu tiawa.

Dɛn ne `(Ahurututu mu Ntini mu Proteinosis - PAP)`? Sɛ yɛbɛka no tiawa a...

Okay, afei momma yɛnhwɛ nea saa tebea a wɔfrɛ no `(PAP)` yi yɛ. Sɛ yɛbɛka no tiawa a, ɛyɛ yare a ɛntaa nsi a ɛba bere a protein, srade, ne nneɛma afoforo bi boaboa ano wɔ mframa kotoku nketenkete (alveoli) a ɛwɔ yɛn ahurututu mu no mu na ɛma esiw ano no. Wo de susuw ho hwɛ, mframa kotoku nketenkete ɔpepem pii na ɛka bom yɛ yɛn ahurututu. Saa mframa kotoku nketenkete yi ne nea yɛfrɛ no `alveoli` . Sɛ yɛhome a, mframa pa fa mframa nkotoku yi mu kɔ mogya no mu.

Enti, wɔ `(PAP)` fam no, mframa kotoku yi bɛyɛ nea nneɛma a ɛho nhia ahyɛ mu ma, te sɛ nsu a ɛkɔ mogya no mu a asiw, na esiw kwan ma oxygen bɛkɔ mogya no mu. Dɛn na ɛba saa bere no? Yɛn mogya nnya mframa pa a ɛdɔɔso. Eyi betumi ama ahome ayɛ den (`dyspnea`) .

Eyi yɛ ahurututu mu yare a emu yɛ den. Enti ɛyɛ ade a ɛfata sɛ wobɛte ehu nka kakra bere a wɔahu sɛ wowɔ yare no te sɛ eyi no. Nanso mma ɛnhaw wo. Sɛ wosa yare no yiye a, wubetumi adi yare no ne ne sɛnkyerɛnne ahorow so. Sɛ wowɔ `(PAP)` a, ɛho hia yiye sɛ wobɛkɔ ahurututu mu aduruyɛ ho oduruyɛfo nkyɛn akɔhwɛ wo. Ɔbɛtu wo fo nso wɔ asetena mu nsakraeɛ a ɛyɛ papa a wobɛtumi ayɛ ho. Eyinom betumi ama w’asetra atu mpɔn.

So saa `(PAP)` yareɛ yi ahodoɔ wɔ hɔ?

Yiw, `(PAP)` yareɛ ahodoɔ titire mmiɛnsa na ɛwɔ hɔ. Ma yɛnhwɛ nea wɔyɛ:

1. Autoimmune PAP: Eyi ne PAP a ɛtaa ba. Mpanyimfo bɛyɛ 90% nya saa ɔkwan yi. Nea ɛkɔ so wɔ ha ne sɛ yɛn nipadua no ankasa nipadua mu tumi a ɛko tia nyarewa no di mfomso tow hyɛ nkwammoaa a ɛboa ma mframa kwan no ho tew no so. Fa no sɛ yɛn ankasa ahobammɔ a ɛko tia yɛn ankasa nkwammoaa.

2. PAP a ɛto so abien: Saa ɔkwan yi ba bere a nkwammoaa a ɛwɔ yɛn mframa kwan mu no dwumadi nya nkɛntɛnso, esiane yare foforo anaasɛ awuduru bi a yɛde di dwuma (e.g. mfutuma, nnuru) nti. Ɛne sɛ, biribi foforo nti na ɛba.

3. Congenital PAP: Eyi yɛ tebea a ɛwɔ hɔ bere a wɔawo no. Ɛtɔ mmere bi a, yare no ho sɛnkyerɛnne betumi ada adi wɔ awo mu, anaasɛ ebetumi ada adi wɔ mfe ahorow mu. Eyi fi nsakrae a ɛba awosu mu nkwaadɔm bi a ɛhwɛ nkwammoaa a ɛwɔ yɛn ahurututu mu no dwumadi so. Yebetumi anya eyi afi awofo hɔ ama mmofra.

Hena na ɛda adi sɛ obenya saa `(PAP)` yare yi?

Mpɛn pii no, ahorow abien a ɛtaa ka no ne ``Autoimmune PAP'' ne ``Secondary PAP''.Wɔ nnipa a wɔadi fi mfe 30 kosi 60. Afei nso, saa ahorow abien yi abu so kɛse wɔ mmarima mu . Nnipa a wɔnom sigaret ne wɔn a wɔde wɔn ho hyɛ mfutuma anaa nnuru ahorow bi mu no wɔ asiane kɛse mu sɛ wobenya saa yare yi.

Ɔkwan a wɔfrɛ no `(Congenital PAP)` no, wɔtaa hu wɔ mmofra a wonnii mfe 10. Nanso, sɛnea yɛadi kan aka no, ebetumi aba wɔ mfe biara mu.

PAP yare no abu so dɛn?

Nokwarem no, `(PAP)` yɛ yareɛ a ɛntaa nsi koraa. Wɔ wiase nyinaa no, saa yare yi ka nnipa ɔpepem abiɛsa biara mu bɛyɛ aduanan. Eyi kyerɛ sɛ ebia nnipa kakraa bi pɛ na wɔwɔ saa yare yi wɔ Sri Lanka.

Adɛn nti na saa `(PAP)` yareɛ yi ba? Dɛn na ɛde ba?

Sɛnea yɛadi kan aka ho asɛm no, yɛn mframa kotoku (alveoli) no afasu yɛ teateaa yiye. Ɛyɛ saa afasu a ɛyɛ tratraa yi mu na mframa pa fi ahurututu no mu kɔ mogya no mu. Mframa kotoku yi afasu fi awosu mu wɔ ngo bi a wɔfrɛ no surfactants . Saa nneɛma a ɛma mframa no yɛ adwuma yi boa ma mframa kotoku no bue a ɛnsiw ano. Eyi ma mframa pa tumi fa mogya no mu ntɛm.

Afei, wɔ yɛn ahurututu mu no, nkwammoaa soronko bi wɔ hɔ a wɔfrɛ no `alveolar macrophages` . Saa nkwammoaa yi te sɛ adwumayɛfo a wosiesie nneɛma. Nea wɔyɛ ne sɛ bere ne bere mu no woyi saa `(surfactants)` yi fi mframa kotoku no mu, na wɔmma ɛnsiw.

Nanso, nea ɛba obi a ɔwɔ PAP so ne sɛ saa nkwammoaa a wɔfrɛ no macrophages yi nnya sɛnkyerɛnne a ɛbɛma wɔahohoro mframa kotoku no afasu ho yiye. Dɛn na ɛba saa bere no? Saa nneɛma a ɛma mframa no yɛ adwuma no boaboa ano na esiw mframa kotoku no ano. Te sɛ nsu a ɛkɔ sink mu a asiw no ara pɛ. Afei ɔkwan a mframa pa fa so kɔ mogya no mu no siw.

Sɛ yɛbɛka no tiawa a, saa tebea yi fi mmerɛwyɛ bi a ɛwɔ "nwura hohoro nhyehyɛe" a ɛwɔ ahurututu no mu.

Dɛn ne `(PAP)` yareɛ ho sɛnkyerɛnne?

PAP ho sɛnkyerɛnne titiriw ne nea ɛtaa ba ne ahome a ɛyɛ tiaa (ahome a ɛyɛ den) . Bere a nnipa dodow no ara home yɛ tiaa bere a wɔreyɛ apɔw-mu-teɛteɛ no, ebia nnipa binom behyia ɔhaw yi bere a wogyina hɔ ara kwa mpo no.

Nea ɛka ho no, sɛnkyerɛnne afoforo pii wɔ hɔ:

• Ɛyaw a wɔte nka wɔ koko mu.
• Ɛwa. Ɛtɔ da bi a, ebia mogya kakra bɛpue ne nsuo a ɛwɔ mu no.
• Wora ne nnadewa no kɔla a ɛsakra bruu (cyanosis) (eyi ba bere a oxygen a ɛdɔɔso nni mogya no mu).
• Ɔbrɛ a wɔte nka bere nyinaa (ɔbrɛ).
• Huraeɛ.
• Ahurututu mu yare a wɔtaa nya.
• Nsateaa no ano yɛ kɛse na ɛyɛ te sɛ sanku (`klubbed fingers`).
• Mu duru a ɛso tew a ntease biara nnim.

Sɛ wowɔ saa sɛnkyerɛnne ahorow yi mu biako anaa nea ɛboro saa a, ɛho hia yiye sɛ wohwehwɛ aduruyɛfo afotu.

So PAP yare betumi akum obi?

Wɔ tebea horow a emu yɛ den mu no, PAP betumi ama ɔhome a entumi nyɛ adwuma yiye a ɛde ne nkwa to asiane mu.Eyi kyerɛ sɛ ahurututu no ntumi nyɛ n’adwuma yiye. Nanso, sɛnea yare yi te gu ahorow wɔ nnipa biara mu. Nnipa binom tumi nya ahoɔden fi yare no mu a wonnya ayaresa biara (`spontaneous remission`). Wɔ afoforo fam no, yare no da so ara wɔ dodow koro no ara mu. Wɔ tebea horow a emu yɛ den kɛse mu no, owu betumi aba esiane ɔhome a entumi nyɛ adwuma yiye anaa nyarewa afoforo nti. Ɛno nti na ɛho hia sɛ wohu yare no ntɛm na wonya ayaresa.

Ɔkwan bɛn so na wohu PAP? Sɔhwɛ ahorow bɛn na wɔyɛ?

Sɛ oduruyɛfo bi susuw sɛ wowɔ PAP a, obedi kan ahwɛ wo na watie w’ahurututu. Ɔbɛbisa wo nsɛm nso afa w’ankasa ne w’abusua aduruyɛ ho abakɔsɛm ne w’asetena ho. Sɛ wonom sigaret anaasɛ wunya mfutuma anaa nnuru a, hwɛ hu sɛ wobɛka akyerɛ wo duruyɛfo.

Sɔhwɛ ahorow a wɔde besi PAP so dua no betumi ayɛ:

• Mogya mu nhwehwɛmu: Hwɛ oxygen dodow a ɛwɔ wo mogya mu.
• Mogya mu nhwehwɛmu titiriw bi: Hwehwɛ ade a ɛkyerɛ biribi a wɔfrɛ no `(granulocyte-macrophage colony-stimulating factor - GM-CSF) autoantibody`. Eyi boa ma wohu nea ɛde `(PAP)` yare no ba.
• Ahurututu mu dwumadi ho sɔhwɛ: Susuw sɛnea w’ahurututu reyɛ adwuma yiye.
• Mfoniniyɛ mu nhwehwɛmu: Eyinom bi ne koko mu X-ray anaa CT scan. Eyinom betumi ama ahurututu no tebea ho mfonini a emu da hɔ.
• Bronchoscopy: Wɔ eyi mu no, wɔde afiri a ɛyɛ tratraa (a mfoninitwa afiri wom) fa ano no mu kɔ mframa kwan no mu na wɔhwɛ ahurututu no mu.
• Ahurututu mu nhwehwɛmu: Woyi ntini ketewaa bi fi ahurututu no mu ma wɔyɛ mu nhwehwɛmu, bere a wɔreyɛ ahurututu mu nhwehwɛmu anaa bere a wɔreyɛ oprehyɛn no.

Saa nhwehwɛmu ahorow yi ne nea ɛma nnuruyɛfo tumi hu PAP pɛpɛɛpɛ.

So aduru a edi mũ bi wɔ hɔ a wɔde sa yare `(PAP)`?

Ɛde besi ha no, wonni aduru biara a wɔde bɛsa PAP. Nanso mma ɛnhaw wo. Ayaresa pii wɔ hɔ a ebetumi aboa wo ma woadi wo yare no ho sɛnkyerɛnne so na woabɔ bra pa.

Ɔkwan bɛn so na wɔsa PAP yare no?

Ayaresa titiriw a wɔtaa de sa PAP ne Whole-Lung Lavage (WLL) . Wɔsan frɛ eyi sɛ "ahurututu mu ahohoro." Fa no sɛ ɛte sɛ nea worehohoro w’ahurututu mu.

Oduruyɛfo de ahurututu mu afiri (afiri a ɛyɛ tratraa a mfoninitwa afiri wom) ne nkyene aduru (nkyene nsu a ɛnyɛ yare) di dwuma de yi ahurututu biako mu bere koro mu. Eyi bɛma ayɛ mmerɛw ama wo sɛ wobɛhome. Saa adeyɛ yi betumi agye bɛyɛ nnɔnhwerew anan kosi anum. Ɛdenam sɛnea wo sɛnkyerɛnne no mu yɛ den so no, ebia ebehia sɛ woyɛ WLL asram kakraa biara anaa pɛnkoro afe biara. Nanso, ebia PAP ayarefo binom renhia WLL. Eyi gyina sɛnea wo tebea no mu yɛ den so.

Dɛn na ɛba bere a woyɛ `(Whole-Lung Lavage - WLL)`?

• Wɔbɛma wo nnuru a wɔde kum nipadua no nyinaa anaa nnuru a ɛma obi dwo.
• Oduruyɛfo no de ahurututu mu afiri bi fa w’anom hyɛ wo mframa kwan mu na ɔde kyerɛ w’ahurututu no biako mu.
• Afiri bi de mframa pa kɔ ahurututu foforo no mu.
• Wohyɛ vest a ɛwosow wɔ wo moma so. Saa wosow yi ma nneɛma a ɛma mframa kotoku no mu no yɛ mmerɛw.
• Afei oduruyɛfo no de nkyene aduru no gu ahurututu no mu na ɔtwe san fi mu (suctions) ka nneɛma a ɛma ahurututu no ho no ho.
• Ɛtɔ da bi a, wotumi hohoro ahurututu foforo no bere koro mu, anaasɛ wotumi hohoro no nna kakraa bi.

Wɔ saa `(WLL)` ayaresa yi akyi no, akwan foforo wɔ hɔ a wɔfa so sa yare:

• Nnuru a ɛma ahurututu mu trɛw: Nnuru yi ma ntini a atwa wo mframa kwan no ho ahyia no dwo, na ɛma ɛyɛ mmerɛw ma wo sɛ wobɛhome.
• Granulocyte-macrophage colony-stimulating factor (GM-CSF) replacement therapy: Wɔde eyi ma denam nebulizer (afiri a ɛdan nnuru a ɛyɛ nsu ma ɛdan nsuonwini) so anaasɛ wɔde twitwiw nipadua mu. Saa ayaresa yi yɛ adwuma denam nkwammoaa a ɛtew mframa kwan (macrophages) no dwumadi a ɛma ɛyɛ yiye no so.
• Ahurututu a wɔde si ananmu: Ɛtɔ mmere bi a, ebia nnipa a wɔwɔ PAP yare a emu yɛ den na wɔn ahurututu asɛe no behia sɛ wɔde ahurututu foforo ma. Wɔ saa adeyɛ yi mu no, woyi w’ahurututu a yare no biako anaa abien no nyinaa na wɔde ahurututu a efi obi a ɔwɔ apɔwmuden a ɔde mae hɔ si ananmu.
• Plasmapheresis ne plasma exchange: Nea ɛka eyi ho ne sɛ wubeyi wo mogya no fã a ɛyɛ nsu a wɔfrɛ no plasma, na wode mogya a efi obi a ɔwɔ apɔwmuden a ɔde ma no mu asi ananmu. Wɔ nnipa a wɔwɔ autoimmune PAP fam no, saa ayaresa yi boa ma nipadua no mu tumi a ɛko tia nyarewa no ntumi ntow nhyɛ nkwammoaa a ɛwɔ apɔwmuden so.
• Oxygen a wɔde ka ho: Oxygen ayaresa betumi aboa wo ma woahome ntɛmntɛm. Wobetumi de akataso a ɛwɔ w’anim anaasɛ afiri ketewaa bi a wɔde ahyɛ wo hwene mu so ama.
• Ayaresa mu sɔhwɛ ahorow: Ebia wubenya hokwan nso de wo ho ahyɛ nhwehwɛmu a wɔyɛ wɔ ayaresa foforo ho mu. Wo ne wo duruyɛfo nka eyi ho asɛm.

So wobetumi asiw PAP yare ano?

Sɛnea yɛadi kan aka ho asɛm no, PAP ahorow ahorow wɔ hɔ. Enti, wontumi nsiw Autoimmune PAP anaa Congenital PAP ano efisɛ ɛne yɛn nipadua mu nneɛma a ɛkɔ so wɔ yɛn nipadua mu no wɔ abusuabɔ.

Nanso, ɛyɛ yie sɛ wɔbɛsi `(Secondary PAP)` ano akodu baabi. Wobɛyɛ dɛn ayɛ saa? Ɛsɛ sɛ wotew nneɛma a awuduru wom a wode wo ho bɛhyɛ mu no so sɛnea wubetumi . Sɛ ɛno ntumi nyɛ yiye a, ɛsɛ sɛ wode akataso pa (`respirator` anaa anim akataso a ɛfata) a ɛkata w’ano ne wo hwene so yiye di dwuma.

Dɛn ne obi a ɔwɔ PAP no anidaso?

Ayaresa a wɔyɛ no daa te sɛ `(Whole-Lung Lavage - WLL)` betumi aboa wo ma woahome mmerɛw na ama sɛnkyerɛnne afoforo afi hɔ. Nnipa kakraa bi na wɔn ho tɔ wɔn wɔ `(PAP)` mu a wonnya ayaresa biara. Nanso, sɛ wɔansa a, `(PAP)` tebea horow a emu yɛ den betumi ama ɔhome a entumi nyɛ adwuma yiye anaasɛ owu. Enti, ɛho hia paa sɛ wudi wo duruyɛfo akwankyerɛ akyi.

Sɛ mewɔ PAP a, dɛn na metumi ayɛ ama me ho?

Sɛ wowɔ PAP a, nneɛma pii wɔ hɔ a wubetumi ayɛ de akura w’akwahosan mu:

• Bisa wo duruyɛfo no nnuruyɛ mu sɔhwɛ ahorow a wɔde sa PAP ayaresa ho asɛm.
• Mma nnom sigaret. Afei nso, nhome wusiw a wɔde adi dwuma bere a nnipa afoforo nom sigaret no .
• Di wo duruyɛfo akwankyerɛ a ɛfa atiridii ne ahurututu mu yare ho nnuru a wɔde ma ho akyi.
• Twe wo ho fi nnipa a wɔyare ho.
• Di aduan a ahoɔden wom. Di wo nipadua mu duru so.
• Hohoro wo nsa yiye bere a wode tiafi adi dwuma awie, ansa na woadi ne bere a woadi awie no.
• Wo ne wo duruyɛfo nyɛ adwuma na yɛ apɔw-mu-teɛteɛ nhyehyɛe a ahobammɔ wom a ɛfata wo.

Kae sɛ Pulmonary Alveolar Proteinosis (PAP) yɛ ahurututu mu yare a ɛntaa nsi a ebetumi ama ayɛ den sɛ ɔbɛhome ne sɛnkyerɛnne afoforo denam mframa kotoku a ɛwɔ ahurututu no mu a ɛbɛma asiw so. Nanso, ayaresa ahorow pii wɔ hɔ a etu mpɔn a ebetumi aboa ma wɔadi PAP nkɛntɛnso so. Sɛ wowɔ saa tebea yi a, susuw ho sɛ wo ne kuw bi a wɔyɛ adwuma wɔ mmeae ahorow pii bɛyɛ adwuma. Sɛ wɔka bom a, wobetumi aboa ma w’asetra atu mpɔn.

Take-Home Message (Ade a ɛho hia sen biara a ɛsɛ sɛ wokae) .

Okay, enti yɛaka `(Pulmonary Alveolar Proteinosis - PAP)` ho asɛm pii. Bere a eyi betumi ayɛ te sɛ nea ɛyɛ den kakra no, ade titiriw a ɛsɛ sɛ yɛkae ne sɛ ɛwom sɛ eyi yɛ yare a ɛntaa nsi de, nanso ɛho hia sɛ wommɔ hu na wunya aduruyɛ mu afotu ne ayaresa a ɛfata.

• Sɛ wowɔ sɛnkyerɛnne ahorow te sɛ ahome a ɛyɛ den, ɔfe a ɛkɔ so daa, anaa ɔbrɛ a ɛtaa ba a, kɔ oduruyɛfo nkyɛn.
• Sɛ wohu sɛ wowɔ `(PAP)` a, hwehwɛ ayaresa wɔ ahurututu ho oduruyɛfo hwɛ ase .
• Ayaresa ahorow wɔ hɔ, enti mma anidaso a wowɔ. Ayaresa te sɛ `(Whole-Lung Lavage - WLL)` betumi ama ahotɔ kɛse.
• Aduruyɛ mu afotu a wobedi akyi pɛpɛɛpɛ na woatra ase yiye no boa kɛse ma wodi yare no so.

Sɛ wowɔ nsɛmmisa foforo bi fa eyi ho a, ntwentwɛn wo nan ase sɛ wo ne wo duruyɛfo bɛkasa. Kɔ so nya apɔwmuden!

` Ahurututu mu Alveolar Proteinosis, PAP, Ahurututu mu Yare, Ɔhome a Ɛyɛ Den, Ahurututu, Nneɛma a Ɛma Ahurututu So, Ahurututu Mu Nyinaa Lavage