Cystic Fibrosis Chii? Zviratidzo, Zvinokonzera, & Kurapa

Cystic fibrosis chirwere chinogara chakagadzika chinoita kuti muviri wako ugadzire mvura yakasimba uye mvura inobuda. Pane kugadzira mvura yakapfava, inoshanda inodzivirira nhengo dzako, vanhu vane cystic fibrosis vanogadzira mvura yakasimba, inonamira inogona kudzima nzira dzakakosha mumapapu nemuviri wemudumbu.

Ichi chirwere chinhu chaunozvarwa nacho, chinopfuudzwa mumhuri kuburikidza nemajini kubva kuvabereki vese. Kunyange zvazvo iri chirwere chakakomba chehupenyu hwese, kunzwisisa zvakanaka kunogona kukubatsira iwe kana vanhu vaunoda kuti murikurwisa zvakanaka uye murarame hupenyu hwakazara.

Cystic Fibrosis Chii?

Cystic fibrosis inoitika kana ma kopi maviri ejeni chaiyo inonzi CFTR isingashande zvakanaka. Iyi jeni kazhinji inobatsira kudzora kuenzana kwemunyu nemvura mumasero ako. Kana ikasakwana, muviri wako unogadzira zvinhu zvakasimba, zvinonamira.

Funga nezvemura yakajairika sekuvhara kunodzivirira kunoshanduka nyore. Mu cystic fibrosis, iyi mvura inoita sepasta yakasimba. Izvi zvinokanganisa kunyanya mapapu ako nemuviri wemudumbu, kunyange zvimwe zvikamu zvemukati zvinogona kukanganiswawo.

Chirwere ichi chinotapura vanhu vanosvika 1 mu 2,500 kusvika ku 3,500 vana vachangoberekwa, zvichiita kuti ive imwe yezvirwere zvakakomba zvinowanzoitika zvemajini. Zvinotapura vanhu vemarudzi ese, kunyange zvazvo zvichinyanya kuonekwa kune avo vekuNorth Europe.

Zviratidzo zveCystic Fibrosis Ndeapi?

Zviratidzo zve cystic fibrosis zvinogona kusiyana zvikuru kubva kune munhu kune mumwe, uye kazhinji zvinoenderana nezvikamu zvemukati zvakanyanya kukanganiswa. Vamwe vanhu vane zviratidzo zvishoma zvinobudirira zvishoma nezvishoma, nepo vamwe vachiwana matambudziko akaoneka pakutanga kweupenyu.

Aya ndiwo zviratidzo zvikuru zvaunogona kuona mune respiratory system:

• Kukosora kunogara kwenguva refu kunoburitsa mvura yakasimba, inonamira
• Kunetseka kwemapapu kwakawanda senge pneumonia kana bronchitis
• Kufema kana kushaya mweya panguva yemabasa ezuva nezuva
• Mhino yakazvimba kana kunetseka kwe sinus kazhinji
• Nasal polyps (kukura duku mukati memhino)

Zviratidzo zve digestive system zvinogona kukoshawo kuziva:

• Zvinhu zvakasviba, zvakakura zvakaoma kubvisa
• Kusakurira uremu kunyange uine chido chakanaka
• Kunetseka kwakanyanya, kunyanya muvana vachangoberekwa
• Kurwadziwa kwemudumbu kana kuzvimba mushure mekudya
• Ganda rinonaka munyu kana ukasveta kana kubata munhu wacho

Vamwe vanhu vanonzwawo zviratidzo zvisingawanzoitika zvinogona kuitika nekufamba kwenguva. Izvi zvinogona kusanganisira chirwere cheshuga (sezvo pancreas inogona kukanganiswa), matambudziko ehuro, kana matambudziko ekubereka mukukura. Chinhu chikuru chekurangarira ndechekuti kuziva nekurapa pakutanga kunoita musiyano mukuru mukutonga izvi zviratidzo zvakanaka.

Marudzi eCystic Fibrosis Ndeapi?

Cystic fibrosis haina chaizvoizvo "marudzi" munzira yakajairwa, asi vanachiremba vanoiisa mapoka zvichienderana nezviratidzo zvakanyanya kuoneka. Izvi zvinovabatsira kugadzira chirongwa chekurapa chakanaka chemunhu wese.

Kuisa mapoka kunowanzoitika kunotarisa pane zvikamu zvikuru zvemukati zvakakanganiswa. Vamwe vanhu vane zviratidzo zvinosanganisira mapapu, nekunetseka kwemapapu uye kuoma kufema sezvavanosangana nazvo. Vamwe vanogona kuva nematambudziko akawanda emudumbu, nekuoma kunwa zvinovaka muviri uye kuchengetedza uremu hwakanaka.

Vanhu vakawanda vanonzwa kusanganiswa kwezviratidzo zvemapapu nemuviri wemudumbu. Kunewo fomu isingawanzoitika umo vanhu vane zviratidzo zvishoma izvo zvisingagoni kuzivikanwa kusvika vakura. Vanhu ava kazhinji vane basa rakanaka remapapu asi vanogona zvakare kuda kutarisirwa uye kutarisirwa nguva dzose.

Chii Chinokonzera Cystic Fibrosis?

Cystic fibrosis inokonzerwa nekuchinja (mutations) mujeni rinonzi CFTR, rinomiririra Cystic Fibrosis Transmembrane Conductance Regulator. Iyi jeni kazhinji inobatsira kudzora kuti munyu nemvura zvinofamba sei mukati nekunze kwemasero ako.

Kuti munhu ave ne cystic fibrosis, anofanira kutora kopi yakashata yeiyi jeni kubva kuvabereki vese. Kana ukatora kopi imwe chete yakashata, unonzi "carrier" asi kazhinji hauzonzwi zviratidzo iwe pachako. Nekudaro, unogona kupfuudza iyo jeni yakashata kuvana vako.

Kune kupfuura 1,700 mutations dzakasiyana dzinotapura jeni reCFTR, kunyange zvimwe zvichinyanya kuwanda kupfuura zvimwe. Mutation inowanzoitika, inonzi F508del, inosanganisira anenge 70% yezviitiko pasi rese. Mutation imwe neimwe inogona kukanganisa kuti chirwere ichi chinokanganisa sei hutano hwemunhu.

Izvi zviri zvemajini chete, zvinoreva kuti hazvikonzerwe nechinhu chipi zvacho chakaitwa kana chisina kuitwa nevabereki panguva yekuzvitakura. Hazvina kuzarawo, saka haugone kuchibata kubva kune mumwe munhu kana kuchipfuudza kune vamwe.

Rini Unoona Chiremba weCystic Fibrosis?

Unofanira kubata chiremba kana ukaona zviratidzo zvemapapu kana mudumbu zvinogara kwenguva refu, kunyanya muvana. Kukosora kunogara kwenguva yakareba kupfuura mavhiki mashoma, kunoburitsa mvura yakasimba, kana kuri kuramba kuchidzoka kunoda kutarisirwa nachiremba.

Tarisai zviratidzo zvakakosha zvemudumbu senge zvinhu zvakasviba, zvinofamba mumvura, kana zvine kunhuhwirira kwakasimba. Kusakurira uremu muvana kunyange vachidya zvakanaka chimwe chiratidzo chakakosha chisingafanirwe kuregererwa.

Kana uine nhoroondo yemhuri ye cystic fibrosis, genetic counseling inogona kukubatsira kunzwisisa njodzi dzako usati waronga pamuviri. Vakawanda vakaroorana vanowana ruzivo urwu rwunobatsira pakusarudza zvinhu zvekuita zvekuita nezvekuita kwemhuri yavo.

Kune avo vakatoongororwa, kuongororwa nguva dzose kunokosha kunyange uchinzwa zvakanaka. Kurapa pakutanga kwezvinetso kazhinji kunodzivirira matambudziko makuru munguva yemberi.

Zvinokonzera Njodzi dzeCystic Fibrosis Ndeapi?

Chinhu chikuru chinokonzera njodzi ye cystic fibrosis ndechekuva nevabereki vanotakura jeni reCFTR rakashata. Sezvo iri chirwere chemajini chinoramba chichiitika, vabereki vese vanofanira kupfuudza kopi yejeni yakachinja kuti mwana wavo awane chirwere ichi.

Rudzi rwevanhu rinotamba basa mumazinga enjodzi, kunyange cystic fibrosis inogona kutapura vanhu vemarudzi ese:

• Vanhu vachena vekuNorth Europe vane njodzi yakakura (anenge 1 mu 25 vanhu vanotakura)
• Hispanic Americans vane huwandu hwevatakuri hweanenge 1 mu 46
• African Americans vane huwandu hwevatakuri hweanenge 1 mu 65
• Asian Americans vane huwandu hwakaderera hwevatakuri hweanenge 1 mu 90

Kuva nemhuri ine cystic fibrosis kunowedzera mukana wako wekuva mutariri. Kana uri kuronga mhuri uye une hanya nezvenjodzi dzemajini, kutaura ne genetic counselor kunogona kupa ruzivo rwakakosha nerunyararo rwepfungwa.

Matambudziko Anogona Kuitika eCystic Fibrosis Ndeapi?

Paunofunga nezvematambudziko anogona kuitika, kunzwisisa kunokubatsira kushanda nechikwata chako chehutano kudzivirira kana kutonga zvakanaka. Matambudziko akawanda anobudirira zvishoma nezvishoma uye anogona kurapwa kana akaonekwa pakutanga.

Matambudziko emapapu kazhinji ndiwo anotyisa zvikuru:

• Kunetseka kwemapapu kwenguva refu kunogona kukuvadza tishu remapapu nekufamba kwenguva
• Mapapu akadonha (pneumothorax) kubva kumhepo ichibuda mukamuri yechipfuva
• Kufema kusakwana mumamiriro ezvinhu akakomba
• Sinusitis inogara kwenguva refu uye nasal polyps

Matambudziko emudumbu anogona kukanganisa zvikuru zvinovaka muviri uye hupenyu hwakanaka:

• Kushomeka kwe pancreatic, zvichiita kuti zviome kudya mafuta nezvinhu zvakakosha
• Chirwere cheshuga kana pancreas isingagoni kugadzira insulin yakakwana
• Chirwere chechiropa kubva kune bile ducts dzakavharwa
• Kuumbwa kwematumbu, kunyanya muvana vachangoberekwa
• Kushomeka kwakanyanya kwezvinovaka muviri kunyange uchidya zvakanaka

Matambudziko asingawanzoitika asi akakosha anosanganisira matambudziko emuviri (osteoporosis), matambudziko ekubereka muvarume nevakadzi, uye kuoma kwemvura panguva yekunaya kana chirwere. Mashoko akanaka ndeawo ekurapa kwakanaka, vanhu vakawanda vane cystic fibrosis vanorarama zvakanaka kusvika vakura uye vanorarama hupenyu hunoshanda, hunogutsa.

Cystic Fibrosis Inogona Kudzivirirwa Sei?

Sezvo cystic fibrosis iri chirwere chemajini, haigone kudzivirirwa munzira yakajairwa. Nekudaro, genetic counseling uye kuongororwa kunogona kubatsira vabereki vanokwanisa kunzwisisa njodzi dzavo uye kuita sarudzo dzakachenjera pamusoro pekuronga mhuri.

Kana uri kuronga pamuviri uye une nhoroondo yemhuri ye cystic fibrosis, carrier screening inogona kuona kana iwe nemumwe wako munotakura jeni racho. Iyi bvunzo iri nyore yeropa inogona kupa ruzivo rwakakosha pakusarudza zvinhu zvekuita zvekuita nezvekuita kwemhuri yavo.

Kune vakaroorana umo vese vakaroorana vanotakura, sarudzo dzinosanganisira kuongororwa kwepamuviri panguva yekuzvitakura, preimplantation genetic diagnosis ne in vitro fertilization, kana kushandisa mazai kana sperm emunhu. Genetic counselor inogona kukubatsira kunzwisisa izvi zvisarudzo pasina kumanikidzwa kana kutongwa.

Nyika dzakawanda zvino dzinosanganisira cystic fibrosis mune zvirongwa zvekuongorora vana vachangoberekwa, zvinoreva kuti kuonekwa nekurapa pakutanga kunogona kutanga nekukurumidza kana mwana aberekwa ane chirwere ichi.

Cystic Fibrosis Inooongororwa Sei?

Kuongorora cystic fibrosis kazhinji kunosanganisira bvunzo dzakawanda dzinoshanda pamwe chete kupa vanachiremba mufananidzo wakajeka. Bvunzo yemvura inonzi gold standard uye inoyera huwandu hwemunyu mumvura yako inobuda.

Panguva yebvunzo yemvura, nzvimbo diki yeganda (kazhinji paruoko) inokurudzirwa kugadzira mvura uchishandisa simba diki remagetsi. Mvura yacho inobva yaunganidzwa uye yoongororwa kuti ione munyu. Vanhu vane cystic fibrosis vane munyu wakawanda mumvura yavo kupfuura zvakajairwa.

Genetic testing inogona kuziva mutations chaiyo mujeni reCFTR. Izvi zvinobatsira zvikuru kana mhinduro dzebvunzo yemvura dzisingaoneki kana paine nhoroondo yemhuri ine chirwere ichi. Bvunzo yeropa inogona kuona kuchinja kwakajairika kwemajini kunosanganiswa ne cystic fibrosis.

Bvunzo dzimwe dzinosanganisira bvunzo dzebasa remapapu kuona kuti mapapu ako anoshanda sei, X-rays yechipfuva kutarisa kukuvara kwemapapu, uye zvikamu zvematumbo kuongorora matambudziko emudumbu. Chiremba wako anogona zvakare kuraira bvunzo dzekuongorora basa re pancreas uye hutano hwese hwezvinovaka muviri.

Kurapa kweCystic Fibrosis Chii?

Kurapa kwe cystic fibrosis kunotarisa pakutonga zviratidzo, kudzivirira matambudziko, uye kuchengetedza hupenyu hwakanaka. Kunyange zvazvo pasina mushonga, kurapa kwakavandudzika zvikuru uye kuri kuramba kuchivandudzwa.

Airway clearance ndiyo inotanga kurapa kwemapapu. Izvi zvinosanganisira matekinoroji nezvishandiso zvinobatsira kusunungura uye kubvisa mvura yakasimba kubva mapapu. Chikwata chako chehutano chichakudzidzisa maekisesaizi chaiwo ekufemera uye chinogona kukurudzira zvinhu senge vibrating vests kana handheld oscillators.

Mishonga inotamba basa rinokosha mukutonga chirwere ichi:

• Mucus thinners inobatsira kuita kuti zvinhu zvive nyore kukosora
• Bronchodilators inovhura nzira dzekufema kuti zvive nyore kufema
• Mishonga inorwisa kuzvimba inoderedza kuzvimba kwemapapu
• Antibiotics inorapira uye inodzivirira kunetseka kwemapapu
• CFTR modulators (mishonga mitsva) inobatsira puroteni yakashata kushanda zvakanaka

Kurapa kwemudumbu kazhinji kunosanganisira zvinowedzerwa zve pancreatic enzyme zvinotorwa nechikafu kubatsira kudya chikafu zvakanaka. Mavhitamini anovaka mafuta (A, D, E, uye K) kazhinji anoda sezvo muviri une matambudziko ekunwa zvakanaka.

Rutsigiro rwezvinovaka muviri runokosha, kazhinji richida kudya kwakakwira-calorie, kwakakwira-mafuta kuchengetedza uremu hwakanaka. Kushanda ne registered dietitian anoziva cystic fibrosis kunogona kuita musiyano mukuru mukutonga zvinovaka muviri zvakanaka.

Maitiro Ekutora Kurapa Kwekumba Panguva yeCystic Fibrosis?

Kutonga cystic fibrosis kumba kunoda kugadzira tsika dzezuva nezuva dzinoita senge kugeza mazino. Chinhu chikuru ndechekuenderera mberi pane kukwana, uye zvishoma zvinokosha zvezuva nezuva zvinoita shanduko huru nekufamba kwenguva.

Airway clearance inofanira kuitwa kanenge kaviri pazuva, kunyange chiremba wako anogona kukurudzira zvikamu zvakawanda panguva yechirwere. Tsvaga matekinoroji anoshanda nehupenyu hwako, kungave kuri kushandisa vibrating vest uchitarisa TV kana kuita maekisesaizi ekufemera muchiwi.

Kuramba wakanyorova kunokosha, kunyanya panguva yekunaya kana uri kurwara. Muviri wako unorasikirwa nemunyu wakawanda kupfuura zvakajairwa, saka unogona kuda kuwedzera munyu wakawanda pakudya kwako kana kushandisa oral rehydration solutions panguva yechirwere kana kupererwa nemvura kwakanyanya.

Kuita maekisesaizi kunobatsira zvikuru kune basa remapapu uye hutano hwese. Kushambira, kufamba, kukwira bhasikoro, kana chero basa raunofarira rinogona kubatsira kusunungura mvura uye kusimbisa tsandanyama dzako dzekufema. Tanga zvishoma nezvishoma uye uvandudze zvishoma nezvishoma nerutsigiro rwechiremba wako.

Chengeta dhayari yezviratidzo kuti uteedzere maitiro ehutano hwako. Cherechedza kuchinja mukukosora, mazinga emagetsi, kana chido. Ruzivo urwu runobatsira chikwata chako chehutano kugadzirisa kurapa uye kubata matambudziko pakutanga.

Unofanira Kugadzirira Sei Kune Chiremba Wako?

Kugadzirira kuenda kuchiremba kunokubatsira kuti utore nguva yako nechikwata chako chehutano zvakanaka. Tanga nekugadzira zviratidzo zvawaona kubvira pakushanya kwako rekupedzisira, kusanganisira pavakatanga uye zvinhu zvinoita kuti zvive nani kana zvakanyanya kuipa.

Uye uunze runyora rwemishonga yese, zvinowedzerwa, uye kurapa kuri kushandiswa ikozvino. Sanganisira kuti unovatora kakawanda sei uye chero zvinokanganisa zvawakonzera. Usakanganwa kutaura nezve mishonga inowanikwa pasina kunyorwa nemuchiremba uye zvinowedzerwa zvemakwenziwo.

Gadzira mibvunzo usati watanga kuti usakanganwa zvinhu zvakakosha panguva yekushanya. Mibvunzo yakajairwa inogona kusanganisira kubvunza nezvekurapa kwakakosha, kukurukura zvipingamupinyi zvemabasa, kana kujeka mirairo yemishonga.

Kana zvichikwanisika, uunze nhengo yemhuri kana shamwari kuti ikubatsire kurangarira ruzivo rwakakurukurwa panguva yekushanya. Kuenda kuchiremba kunogona kuita kuti unzwe uchinetseka, uye kuva nerutsigiro kunogona kukubatsira kugadzirisa zvese zvakanaka.

Unganidza chero mhinduro dzebvunzo kana rekodhi dzehutano kubva kune vamwe vanopa kubvira pakushanya kwako rekupedzisira. Izvi zvinopa chiremba wako mufananidzo wakakwana wehutano hwako hwakakwana uye chero kuchinja kwakaitika.

Chinhu Chikuru Chekuyeuka Nezve Cystic Fibrosis Chii?

Cystic fibrosis chirwere chakakomba chemajini, asi hachisi chinhu chinokanganisa kurarama hupenyu hunoremekedzwa, hunoshanda. Nekurapa kwakanaka, tsika dzekutonga zuva nezuva, uye masisitimu akasimba ekutsigira, vanhu vakawanda vane cystic fibrosis vanoita zvidzidzo, mabasa, hukama, uye mabasa avanofarira.

Chinhu chakakosha chekurangarira ndechekuti kuongororwa pakutanga nekurapa kwenguva dzose kunoita musiyano mukuru mumagumo emangwana. Kana uchifungidzira cystic fibrosis iwe pachako kana munhu waunoda, kutsvaga kuongororwa kwemishonga nekukurumidza kunogona kugadzirira nzira yekutonga hutano hwakanaka.

Kutsvagisa kuri kuramba kuchizounza tariro itsva, nekurapa kuchivandudzwa nguva dzose uye mishonga mitsva iri kuwanikwa. Cystic fibrosis community yakasimba uye inotsigira, ich kupa zviwanikwa nehukama zvinogona kuita rwendo rusina kuparadzana.

Rangarira kuti kutonga cystic fibrosis ibasa rechikwata rinosanganisira iwe, mhuri yako, uye vanopa hutano hwako. Kutaurirana kwakavhurika, kutarisirwa kwenguva dzose, uye kuramba uchiudzwa nezve chirwere chako ndiwo maturusi ako akanakisa ekurarama zvakanaka ne cystic fibrosis.

Mibvunzo InowanzoBvunzwa Nezve Cystic Fibrosis

Vakuru Vanogona Kuwana Cystic Fibrosis Gare Gare Muupenyu Here?

Kwete, cystic fibrosis chirwere chemajini chaunozvarwa nacho. Nekudaro, vamwe vanhu vane mafomu asina simba asingazivikanwi kusvika vakura. Vanhu ava vangave vane zviratidzo zvishoma kwemakore akawanda izvo zvakataurwa kune zvimwe zvirwere senge asthma kana kunetseka kwemapapu kwenguva refu.

Cystic fibrosis inotapurirana here?

Cystic fibrosis pachayo haina kuzara sezvo iri chirwere chemajini. Nekudaro, vanhu vane cystic fibrosis vanonyanya kukanganiswa nezvirwere zvebhakitiriya, uye mabhakitiriya aya anogona dzimwe nguva kupararira pakati pevanhu vane cystic fibrosis. Ndosaka matanho ekudzora hutachiona akakosha munzvimbo dzehutano uye nharaunda dzeCF.

Vanhu vane cystic fibrosis vanogona kuva nevana here?

Vanhu vakawanda vane cystic fibrosis vanogona kuva nevana, kunyange zvazvo kubereka kunogona kukanganiswa. Anenge 95% yevarume vane cystic fibrosis vane matambudziko ekubereka nekuda kwekuvhara kwematumbo esperm, asi matekinoroji ekubatsira kubereka anogona kazhinji kubatsira. Vakadzi vane cystic fibrosis vanogona kunge vane kubereka kwakaderera asi vanogona kazhinji kubata pamuviri zvakasikwa nekutarisirwa kwakanaka kwehutano.

Upenyu hwekutarisirwa kune munhu ane cystic fibrosis ndihupi?

Upenyu hwekutarisirwa hwakavandudzika zvikuru mumakore mashoma apfuura. Parizvino, avhareji yekutarisirwa kwemakore iri pakati pemakore makumi mana nemana uye iri kuramba ichikwira sezvo kurapwa kuchivandudzwa. Zvinhu zvakawanda zvinokanganisa mhinduro dzega, kusanganisira kuti kurapa kwakatanga sei pakutanga, kuwana kutarisirwa kwakakosha, uye kutarisirwa kwese kwehutano.

Vanhu vane cystic fibrosis vanofanira kudzivisa mabasa chaiwo here?

Vanhu vakawanda vane cystic fibrosis vanokurudzirwa kuramba vachishanda, sezvo kuita maekisesaizi kunobatsira kune basa remapapu. Nekudaro, mabasa anofanira kuchinjwa zvichienderana nebasa remapapu reumwe neumwe uye hutano hwese. Kushambira kazhinji kuita maekisesaizi akanaka, nepo mabasa munzvimbo dzine guruva kana dzine tsvina anogona kuderera. Chikwata chako chehutano chinogona kupa mazano ekushanda akakodzera.