Mob hauv siab ntau zaus, tsis hnyav nce ... Qhov no puas yog Cystic Fibrosis (CF)? Cia peb paub txog qhov no.

Koj tus me nyuam puas ib txwm muaj qhov ntswg txhaws? Txawm lawv pub zaub mov ntau npaum li cas los xij, lawv kuj nyias nyias yam tsis paub txog nws? Qee zaum puas zoo li lawv ua tsis taus pa? Nws yog ib qho ib txwm rau cov niam txiv ntshai thaum lawv pom cov tsos mob no. Cov no yuav yog cov tsos mob ntawm tus mob hu ua Cystic Fibrosis (CF), uas peb yuav tsis tau hnov ​​​​dua, tab sis nws tseem ceeb heev uas yuav tsum paub. Cia peb tham txog qhov no yam tsis ntshai thiab yooj yim hnub no.

Mob cystic fibrosis (CF) yog dab tsi tiag?

Hais yooj yim xwb, qhov no yog ib yam kab mob caj ces uas tau txais los ntawm niam txiv . Muaj ib hom noob tshwj xeeb uas tswj kev txav ntawm ntsev (chloride) thiab cov kua dej los ntawm cov cell ntawm peb lub cev. Qhov no hu ua CFTR (cystic fibrosis transmembrane conductance regulator) noob . Yog li, kab mob CF tshwm sim thaum muaj qhov tsis zoo lossis kev hloov pauv hauv cov noob no.

Vim yog qhov teeb meem no, muaj dab tsi tshwm sim? Feem ntau, cov kua qaub hauv peb lub cev, xws li cov hnoos qeev thiab cov kua qaub ncaug , yog nyias thiab nplua. Tab sis hauv lub cev ntawm ib tus neeg uas muaj CF, cov kua qaub no dhau los ua tuab heev, tuab, thiab nplaum .

Xav txog seb yuav muaj li cas thaum cov hnoos qeev tuab no sib sau ua ke hauv lub ntsws. Nws ua rau nws nyuaj rau ua pa, cov kab mob yooj yim raug ntes , thiab kev kis kab mob ntau dua . Raws li lub sijhawm dhau mus, qhov no tuaj yeem ua rau lub ntsws puas tsuaj loj heev, ua tsis taus pa, thiab txawm tias tuag.

Tsis tas li ntawd xwb, cov kua qaub no ua rau cov hlab ntsha hauv lub pancreas tsis ua haujlwm. Tom qab ntawd cov enzymes zom zaub mov uas pab zom cov zaub mov uas peb noj tsis raug tso tawm . Yog li ntawd, kev tsis noj zaub mov zoo thiab kev loj hlob qeeb tshwm sim. Tsis tas li ntawd, kab mob siab, ntshav qab zib ('Cystic Fibrosis-Related Diabetes' - CFRD) thiab teeb meem kev xeeb tub kuj tseem tuaj yeem tshwm sim.

Tiamsis tsis txhob txhawj. Niaj hnub no, ua tsaug rau kev kho mob siab heev, lub neej ntev ntawm cov neeg mob CF tau nce ntxiv ntau xyoo lawm.

Cov tsos mob ntawm tus kab mob no yog dab tsi?

Cov tsos mob ntawm CF yuav txawv ntawm ib tus neeg mus rau ib tus neeg. Lawv kuj txawv nyob ntawm qhov hnyav ntawm tus kab mob. Cia peb saib cov tsos mob tseem ceeb.

Cov kab mob cystic fibrosis uas tsis zoo li qub

Qhov no yog ib hom mob CF uas tsis hnyav heev. Cov tsos mob yuav tshwm sim tom qab hauv lub neej. Qee zaum tsuas yog ib lub cev xwb thiaj raug cuam tshuam.

Dab tsi ua rau CF?

Raws li peb tau tham ua ntej lawm, qhov no yog tshwm sim los ntawm qhov tsis zoo hauv cov noob caj noob ces hu ua CFTR . Yuav kom ib tug me nyuam muaj tus kab mob no, tus me nyuam yuav tsum tau txais ib daim qauv ntawm cov noob caj noob ces tsis zoo los ntawm leej niam thiab leej txiv .

Yuav kuaj mob li cas?

Nws yog ib qho tseem ceeb heev uas yuav tsum kuaj mob no thaum ntxov, vim tias kev kho mob tuaj yeem pib sai thiab tej yam teeb meem uas yuav tshwm sim yav tom ntej tuaj yeem txo qis. Hauv Sri Lanka, qee lub tsev kho mob tam sim no tab tom kuaj cov menyuam yaus rau qhov no thaum yug los.

Cov kev kuaj mob tseem ceeb uas siv los kuaj mob yog:

• Kev kuaj hws: Qhov no yog qhov kev kuaj mob zoo tshaj plaws los kuaj mob CF.Qhov kev kuaj mob no ntsuas qhov ntau ntawm cov ntsev (chloride) hauv koj cov hws. Yog tias qhov ntau ntau dua li qub, nws yog ib qho cim ntawm CF.
• Kev kuaj ntshav: Qhov no kuaj seb muaj tshuaj hu ua 'Immunoreactive Trypsinogen (IRT)' npaum li cas hauv cov ntshav. Cov neeg mob CF no muaj cov tshuaj no ntau heev.
• Kev kuaj noob caj noob ces (kev kuaj DNA): Qhov no ncaj qha kuaj xyuas seb puas muaj qhov tsis zoo hauv cov noob 'CFTR'.

Ntxiv rau cov kev kuaj no, koj tus kws kho mob yuav pom zoo kom ua lwm yam kev kuaj los xyuas kom meej tias koj tau kuaj pom thiab nrhiav seb puas muaj teeb meem dab tsi. Piv txwv li, kev thaij duab X-ray hauv siab, kev kuaj kev ua haujlwm ntawm lub ntsws (PFTs), thiab kev kuaj cov hnoos qeev thiab cov quav.

Cov kev kho mob yog dab tsi?

Vim CF yog ib yam kab mob nyuaj heev, kev kho mob yog muab los ntawm pab pawg kws tshwj xeeb ntau yam, suav nrog tus kws kho mob ntsws, tus kws kho lub cev, thiab tus kws noj zaub mov. Lub hom phiaj tseem ceeb ntawm kev kho mob yog kom tshem tawm cov hnoos qeev hauv lub ntsws, tiv thaiv kev kis kab mob, thiab muab cov khoom noj khoom haus tsim nyog.

Cov tshuaj

Koj tus kws kho mob yuav sau ntawv kom koj noj cov tshuaj xws li:

• Cov Tshuaj Tua Kab Mob: Tiv thaiv thiab kho cov kab mob ntsws.
• Cov tshuaj ua kom cov hnoos qeev nyias: Cov no yog muab los ntawm lub tshuab ua pa lossis lub tshuab nebulizer. Lawv pab ua kom cov hnoos qeev tuab nyias thiab ua rau nws yooj yim dua rau hnoos tawm.
• Cov tshuaj bronchodilators: Lawv ua kom cov hlab cua dav dua thiab ua rau ua pa yooj yim dua.
• Cov tshuaj enzyme pancreatic: Cov no yuav tsum noj nrog txhua pluas noj thiab khoom noj txom ncauj. Lawv pab zom zaub mov thiab nqus cov as-ham.
• CFTR modulators: Cov no yog cov tshuaj tshiab thiab zoo tshaj plaws. Cov tshuaj no pab kho qhov ua haujlwm ntawm cov protein 'CFTR' uas tsis zoo uas ua rau muaj kab mob. Qhov no tuaj yeem ua rau cov hnoos qeev nyias nyias, txhim kho kev ua haujlwm ntawm lub ntsws, txo qhov hnoos, thiab txo qhov hnyav nce. '(Trikafta, Kalydeco, Orkambi)' yog qee hom tshuaj no.

Cov Txuj Ci Kev Tshem Tawm Pa (ACT)

Cov no yog tej yam uas yuav tsum tau ua txhua hnub, tsawg kawg yog ob zaug hauv ib hnub. Lawv pab ua kom cov hnoos qeev tuab uas nyob hauv lub ntsws xoob thiab tshem tawm.

• Kev Kho Mob Lub Hauv Siab: Cia ib tug neeg kov nraub qaum thiab hauv siab kom cov hnoos qeev tawm.
• Lub Tsho: Qhov no yog lub tsho txuas nrog lub tshuab tshwj xeeb. Thaum hnav, cov kev co siab yuav kov lub hauv siab, ua rau muaj kev ua thiab ua rau cov hnoos qeev tawm.
• Cov khoom siv PEP: Kev ua pa tawm los ntawm cov khoom siv me me xws li '(Flutter, Acapella)' tsim kev nyuaj siab hauv lub ntsws thiab pab tshem cov hnoos qeev.

Kev phais mob

Qee qhov teeb meem yuav xav tau kev phais.

• Kev hloov ntsws: Txoj kev xaiv kawg thaum tus kab mob hnyav heev thiab lub ntsws yuav luag ua haujlwm tsis tau zoo.
• Kev hloov siab: Yog tias lub siab puas tsuaj loj heev.
• Lwm yam: Tej yam xws li tshem tawm cov polyps hauv qhov ntswg, ntxig ib lub raj pub mov rau hauv lub plab kom muab zaub mov.

Tej yam teeb meem uas yuav tshwm sim ntawm CF

Yog tias CF tsis raug kho kom zoo, ntau yam teeb meem tuaj yeem tshwm sim.

• Lub ntsws: Txoj hlab pa dav zuj zus mus tas li ('Bronchiectasis'), lub ntsws tawg ('Pneumothorax'), kev kis kab mob mus ntev.
• Kab mob Pancreatitis: Kab mob ntshav qab zib uas cuam tshuam nrog CF ('CFRD').
• Siab: Mob siab cirrhosis ('Cirrhosis').
• Plab hnyuv: Kev thaiv plab hnyuv, kev pheej hmoo ntawm mob qog noj ntshav hauv plab hnyuv.
• Kev yug me nyuam: Txiv neej tsis muaj menyuam thiab poj niam yug menyuam qeeb.
• Pob Txha: Pob txha nyias nyias (Osteoporosis).

Cov Lus Nug Feem Ntau (FAQs)