Bɔrku tɛm we yu chɛst kin kɔnjɛshɔn, we yu nɔ kin gɛt bɔku bɔku bɔdi... Dis na Sistik Faybrosis (CF)? Lɛ wi no bɔt dis.

Yu smɔl pikin kin gɛt in nos we kin stɔp ɔltɛm? Ilɛk ɔmɔs it dɛn de gi, dɛn kin tan lɛk tin we dɛn nɔ ivin no? Sɔntɛnde, i kin fil lɛk se dɛn gɛt prɔblɛm fɔ blo? Na nɔmal tin fɔ mek mama ɛn papa dɛn fred we dɛn si dɛn sik ya. Dis kin bi sayn dɛm fɔ wan sik wae dɛn kɔl Cystic Fibrosis (CF), wae wi nɔr kin yɛri bɔt bɔrku, bɔt i rili impɔtant fɔ no. Lɛ wi tɔk bɔt dis witout fred ɛn jɔs tide.

Wetin rili na sistik fibrosis (CF)?

Fɔ tɔk am simpul wan, dis na sik we pɔsin kin gɛt frɔm in mama ɛn papa . Wan spɛshal jin de we de kɔntrol aw sɔl (klɔrayd) ɛn wata de muv tru di sɛl dɛn na wi bɔdi. dis dεn kכl dis di CFTR (cystic fibrosis transmembrane conductance regulator) jin . So, CF sik kin apin we difεkt כ mכtεshכn de na dis jin.

Wetin kin apin bikɔs ɔf dis bad tin? Nɔmal wan, di tin dɛn we de kɔmɔt na wi bɔdi lɛk mɔkus ɛn pus , kin tan ɛn slipul. Bɔt na pɔsin we gɛt CF in bɔdi, dɛn tin ya kin rili tik, tik, ɛn kin stika .

Imajin wetin kin apin we dis tik tik mכkus kin bכku na di lכng. I kin mek i nɔ izi fɔ blo, i kin izi fɔ mek di jem dɛn trɔp , ɛn di sik kin kam bɔku . As tɛm de go, dis kin mek di lɔng dɛn pwɛl bad bad wan, i nɔ kin ebul fɔ blo fayn, ɛn i kin ivin day.

כlso, dεn tik tik sekreshכn dεm ya de blכk di dakt dεm na di pankrias. Dɔn di dijestiv ɛnzaym dɛn we de ɛp fɔ dayjɛst di it we wi de it nɔ de kɔmɔt . Dis kin mek pipul dɛn nɔ de it fayn ɛn dɛn nɔ kin gro fayn. pan tap dat, liva sik, dayabitis (`Cystic Fibrosis-Related Diabetes` - CFRD) εn fεtiliti prכblεm kin apin bak.

Bɔt nɔ wɔri. Tide, bikɔs ɔf di advans tritmɛnt dɛm, di layf we di wan dɛn we gɛt CF de liv dɔn go ɔp fɔ bɔku bɔku ia.

Wetin na di sayn dɛm fɔ dis sik?

Di sayn dɛm fɔ CF kin difrɛn frɔm wan pɔrsin to ɔda pɔrsin. Dɛn kin difrɛn bak dipen pan aw di sik kin tranga. Lɛ wi tek wan luk pan di men sayn dɛm.

Atypical sistik fibrosis we nɔ de apin

Dis na wan we we nɔ gɛt bɛtɛ CF. Di sayn dɛm kin apin leta na layf. Sɔntɛnde, na wan ɔgan nɔmɔ kin afɛkt.

Wetin kin mek pɔsin gɛt CF?

As wi bin dɔn tɔk, dis kin apin bikɔs ɔf wan prɔblɛm we de na di jin we dɛn kɔl CFTR . Fɔ mek pikin gɛt dis sik, di pikin fɔ gɛt kɔpi fɔ di jin we nɔ fayn frɔm di mama ɛn di papa .

Aw dɛn kin no di sik?

I rili impɔtant fɔ no dis sik kwik, bikɔs afta dat dɛn kin bigin fɔ trit am kwik ɛn dɛn kin stɔp di prɔblɛm dɛn we kin apin tumara bambay. Na Sri Lanka, sɔm ɔspitul dɛn de tɛst pikin dɛn naw fɔ dis we dɛn bɔn dɛn.

Di men tɛst dɛm wae dɛn kin yuse fɔ no di sik na:

• Swet tɛst: Dis na di tɛst we pɔsin kin abop pan pas ɔl fɔ no if pɔsin gɛt CF.Dis tɛst we nɔ de mek yu fil pen, de sho aw di sɔl (klɔrayd) de na yu swet. If di amount bכku pas di nכmal, na sayn fכ CF.
• bכdi tεst: Dis de tεst di lεvεl fכ wan kεmikכl we dεn kכl `Immunoreactive Trypsinogen (IRT)` insay di bכdi. Dis lεvεl de hכy pan CF pasεnshכn dεm.
• jεnεtik tεst (DNA tεst): dis de chεk dεn wan dεm fכ difεkt dεm na di `CFTR` jin.

Apat frɔm dɛn tɛst ya, yu dɔktɔ kin tɛl yu fɔ du ɔda tɛst fɔ no if yu gɛt di sik ɛn fɔ luk fɔ prɔblɛm dɛn. Fɔ ɛgzampul, dɛn kin du ɛkstrem rayt na di chɛst, dɛn kin du tɛst fɔ no aw pɔsin de wok (PFT), ɛn tɛst di sputum ɛn stɔl.

Wetin na di tritmɛnt dɛn?

Bikɔs CF na kɔmpleks sik, na wan tim we gɛt spɛshal pipul dɛn we gɛt difrɛn difrɛn wok dɛn, we gɛt wan dɔktɔ we de mɛn di pulmonɔlɔjis, wan dɔktɔ we de mɛn di bɔdi, ɛn pɔsin we sabi bɔt it, de gi di tritmɛnt. Di men gol dɛm fɔ tritmɛnt na fɔ klin di lɔng dɛm fɔ di mכkus, fɔ mek i nɔ gɛt infεkshɔn, ɛn fɔ gi di it we i nid.

Di mɛrɛsin dɛn we dɛn kin yuz

Yu dɔktɔ kin gi yu mɛrɛsin dɛn lɛk:

• Antibayɔtiks: Fɔ mek yu nɔ gɛt ɛn trit yu lɔng infɛkshɔn.
• Mεdikeshכn dεm we de mek mכkus tכn: dεn kin gi dεm tru inhala כ nebulayz. Dɛn kin ɛp fɔ mek tik tik mucus tan ɛn mek am izi fɔ kɔf.
• Bronchodilators: Dɛn de mek di say dɛn we di briz de blo big ɛn mek i izi fɔ blo.
• Pankrias ɛnzaym sɔpɔtmɛnt: Dɛn fɔ tek dɛn tin ya wit ɛvri it ɛn snek. Dɛn kin ɛp fɔ digest ɛn fɔ tek di nyutriɛnt.
• CFTR modulators: Dis na di nyu ɛn ifektiv klas fɔ drɔgs. dis dכg dεm de εp fכ rεstכr di fכnshכn fכ di difεktiv `CFTR` protin we de kכz di sik dεn wan dεm. Dis kin mek di mכkus tan, i kin mek di lכng wok fayn, i kin mek i nɔ kɔf, εn ivin i kin mek i nɔ gɛt bɔku bɔku wet. `(Trikafta, Kalydeco, Orkambi)` na sɔm pan dɛn kayn drɔgs ya.

Tɛknik fɔ Kliarens di Aywe (ACT) .

Dis na tin dɛn we dɛn fɔ du ɛvride, at le tu tɛm insay di de. Dɛn kin ɛp fɔ lus ɛn pul di tik tik mucus we dɔn stɔp na di lɔng dɛn.

• Chɛst fizik tɛrapi: Fɔ mek pɔsin tap di bak ɛn chɛst wit ritm fɔ mek di mכkus lɔs.
• Di Vɛst: Dis na vest we dɛn kɔnɛkt to spɛshal mashin. We dɛn wɛr am, di ay frikyuɛnsi vaybreshɔn dɛn kin tap di chɛst, we kin mek wan akshɔn apin ɛn mek di mכkus lɔs.
• PEP divays dεm: we yu blo tru sכm sכm divays dεm lεk `(Flutter, Acapella)` de mek prεshכn na di lכng dεm εn i de εp fכ kכl mכkus.

Di ɔpreshɔn dɛn we dɛn kin du

Sɔm prɔblɛm dɛn kin nid fɔ gɛt ɔpreshɔn.

• Lɔng transplant: Na las tin we di sik kin rili bad ɛn di lɔng dɛn nɔ kin wok fayn igen.
• Liva transplant: If di liva dɔn pwɛl bad bad wan.
• כ da tin dεm lεk fכ pul di nos polip dεm, fכ put fכ it tyub insay di bεlε fכ gi yu it.

Posisibul komplikashɔn dɛn we CF kin gɛt

If dɛn nɔ trit CF fayn, difrɛn prɔblɛm dɛn kin apin.

• di lכng dεm: di aywe dεm de wayd fכ כltεm (`Bronchiectasis`), di lכng kכllaps (`Pneumothorax`), infεkshכn dεm we de te.
• Pankrɛayt: CF-rilayt dayabitis (`CFRD`).
• Liva: Liva skata (`Sirosis`).
• Intestinal: Intestinal obstruction, inkris risk fɔ gɛt kɔlon kansa.
• Riprodaktiv sistεm: Man infεtiliti εn delay fכ bכn pikin na uman dεm.
• Bon dεm: Di bon dεm de tכn (Osteoporosis).

Kwɛstyɔn dɛn we dɛn kin Aks Bɔku tɛm (FAQ) .