Idiopathic pulmonary fibrosis (i- IPF ) ayilulapheki, kodwa iyaphathwa. Ngokujabulisayo, imithi emisha ivunyiwe kusukela ngo-2014 okwenza umehluko ezinkomba , izinga lokuphila , nokuthuthukiswa kwabantu abaphila nalesi sifo. Ngokuphambene nalokho, imishanguzo esetshenzisiwe kuze kube yilapho ithathwa njengobangela ukulimala kunokuhle kwabanye abantu abane-IPF.
Uma uthola ukuthi unesifo, qiniseka ukuthi ungadangali ngolwazi oludala.
Izinhloso zokwelapha ze-Idiopathic Pulmonary Fibrosis
Umonakalo okwenzekile ku-IPF ngencazelo ayikwenzeki; i-fibrosis (i-scarring) eyenziwe ayikwazi ukuphulukiswa. Ngakho-ke, imigomo yokwelashwa yilezi:
- Nciphisa umonakalo owengeziwe emaphashini. Njengoba imbangela eyinhloko ye-IPF yingozi futhi ilandelwa ukuphulukiswa okungavamile, ukwelashwa kuqondiswa kulezi zindlela.
- Thuthukisa ubunzima bokuphefumula.
- Khulisa umsebenzi kanye nekhwalithi yokuphila.
Njengoba i-IPF yisifo esingavamile, kuyasiza uma iziguli zifuna ukunakekelwa esikhungweni sezokwelapha okugxile kwi-idiopathic pulmonary fibrosis kanye nezifo zamaphaphu emaphakathi. Kungenzeka ukuthi ochwepheshe unolwazi lwamuva kakhulu lwezinketho zokwelashwa olutholakalayo futhi angakusiza ukhethe okusemandleni akho esimweni sakho ngasinye.
Imithi ye-Idiopathic Pulmonary Fibrosis
I-Tyrosine Kinase Inhibitors
Ngo-Okthoba ka-2014, imithi emibili yaba yizidakamizwa zokuqala ezamukelwa yi-FDA ngokukhethekile ekwelapheni i-idiopathic pulmonary fibrosis. Le mithi, i-pirfenidone ne-nintedanib, ihlose ama-enzyme okuthiwa i-tyrosine kinase futhi isebenze ngokunciphisa i-fibrosis (i-antifibrotics).
Ngokulula kakhulu, ama-enzyme okuthiwa i-tyrosine kinase asebenze izimbangela zokukhula ezibangelwa i-fibrosis, ngakho-ke le mithi ivimbela ama-enzyme ngakho-ke izimbangela zokukhula ezingabangela i-fibrosis eqhubekayo.
Le mithi itholakala ukuthi inezinzuzo eziningana:
- Banciphisa ukuqhuma kwesifo ngesigamu phakathi nonyaka iziguli zithathe umuthi (manje usufundela isikhathi eside).
- Zinciphisa ukusebenza komsebenzi wamaphaphu (ukwehla ku-FVC kungaphansi) ngesigamu.
- Zibangela ukucindezeleka okuncane kwesifo.
- Iziguli ezisebenzisa lezi zidakamizwa zinezinga elingcono lezempilo elihlobene nempilo.
Lezi imithi ngokuvamile zibekezeleleke kahle, okubaluleke kakhulu kwisifo esiqhubekayo ngaphandle kokwelapha; uphawu oluvame kakhulu ukuhuda.
N-Acetylcysteine
Esikhathini esidlule i-n-acetylcysteine yayivame ukusetshenziselwa ukuphatha i-IPF, kodwa izifundo zakamuva azitholanga ukuthi lokhu kuphumelele. Uma kuphukile kubonakala sengathi abantu abanezinhlobo ezithile zezakhi zofuzo bangathuthukisa imithi, kuyilapho labo abanezinye izinhlobo zofuzo (enye i-allele) empeleni belimazwa yizidakamizwa.
I-Proton Pump Inhibitors
Okuthakazelisayo yisifundo esisodwa esenziwe ngokusebenzisa i-esomeprazole, i- proton pump inhibitor , kumaseli wamaphaphu ebhokisini nasemagatsheni. Lo muthi, ovame ukusetshenziselwa ukwelapha isifo se-reflux ye-gastroesophageal, kwaholela ekusindeni okwenyuka kwamangqamuzana amaphaphu nasemanzini. Njengoba i-GERD iyisandulela esivamile ku-IPF, kucatshangwa ukuthi i-asidi esuka esiswini efisa emaphashini ingaba yingxenye ye-etiology ye-IPF.
Nakuba lokhu kungakaze kuhlolwe kubantu, ngokuqinisekile ukwelashwa kwe-GERD engapheli kubantu abane-IPF kufanele kucatshangelwe.
Ukwelashwa Kwama-Lung
Ukusetshenziselwa ukufakelwa kwamaphaphu angamazwe amabili noma okulodwa njengoba ukwelashwa kwe-IPF kuye kwanda kancane eminyakeni engu-15 edlule futhi kubonisa iqembu elikhulu kunazo zonke labantu abalinde ukuguqulwa kwamaphaphu e-United States. Iphethe ingozi enkulu kodwa yodwa ukwelashwa okuyaziwa ngalesi sikhathi ukukhulisa ngokucacile isikhathi sokuphila.
Njengamanje, ukusinda komphakathi (isikhathi esilandelayo isigamu sabantu esesifile kanye nesigamu sisaphila) sekuyiminyaka engu-4.5 ngokufakelwa, nakuba kungenzeka ukuthi ukusinda kuye kwathuthuka ngaleso sikhathi ngenkathi umuthi uba ngcono kakhulu.
Izinga lokusinda liphakeme kakhulu kunezingxenye ezimbili zomhlaba ngaphandle kokufakelwa kwamaphaphu owodwa, kodwa kucatshangwa ukuthi lokhu kuhlangene kakhulu nezinye izinto ngaphandle kokufakelwa, njengezici zabantu abaphethe amaphaphu amabili noma amabili.
Ukwelashwa okusekelayo
Njengoba i-IPF yisifo esiqhubekayo, ukwelashwa okusekelayo ukuqinisekisa ukuthi izinga eliphezulu lokuphila kungenzeka kunesibaluleke kakhulu. Ezinye zalezi zinyathelo zihlanganisa:
- Ukuphathwa kwezinkinga ezifanayo.
- Ukwelashwa kwezimpawu.
- Ukuqhuma kwesibhamu kanye ne-pneumonia ukudubula ukusiza ukuvimbela izifo.
- Ukuvuselelwa kwamapulmonary .
- Ukwelashwa kwe-oksijeni - Abanye abantu bayanqikaza ukusebenzisa i-oksijini ngenxa yenhlamba, kodwa kungasiza kakhulu kwabanye abantu abane-IPF. Ngokuqinisekile, kwenza kube lula ukuphefumula futhi kuvumela abantu abanesifo ukuba babe nekhwalithi engcono yokuphila, kodwa futhi kunciphisa izinkinga ezihlobene ne-oksijini ephansi egazini futhi kunciphise ukucindezeleka kwegazi (ukucindezeleka okukhulu kwegazi emithanjeni ehamba phakathi kwesokudla inhliziyo nemaphaphu).
Imibandela Ekhona Nezinkinga
Izinkinga eziningana zivamile kubantu abahlala ne-IPF. Lokhu kufaka:
- I-apnea yokulala
- Isifo se-reflux se-gastroesophageal (GERD)
- Umfutho wegazi ophezulu we-pulmonary - Umfutho wegazi ophezulu emithanjeni yemaphaphu kwenza kube nzima ukushaya igazi emithanjeni yegazi ehamba emaphashini, ngakho-ke, ngakwesokudla kwenhliziyo (ventricle kwesokudla nangesobunxele) kufanele usebenze kanzima.
- Ukucindezeleka
- Umdlavuza we-Lung - Amaphesenti angama-10 abantu abane-IPF bathola umdlavuza wamaphaphu
Uma usuvele uthola i- IPF , kufanele uxoxe ngokungenzeka ngalezi zinkinga nodokotela wakho bese uhlela uhlelo lokuthi ungayiphatha kanjani noma ubavimbele kangcono.
Amaqembu asekela ku-inthanethi kanye nemiphakathi
Akukho okufana nokukhuluma nomunye umuntu olwa nesifo esifana nawe. Noma kunjalo, kusukela i-IPF engavamile, kungenzeka ukuthi ayikho iqembu lokusekela emphakathini wakho. Uma uthola ukwelashwa esikhungweni esisezingeni elikhethekile le-IPF, kungenzeka kube namaqembu okusekela ngaphakathi komuntu atholakalayo esikhungweni sakho sezokwelapha.
Kulabo abangenayo iqembu lokusekela elifana nalokhu-okusho ukuthi abantu abaningi abanamaqembu asekela i-IPF-inthanethi kanye nemiphakathi kuyindlela enhle. Ngaphezu kwalokho, lena yimiphakathi ongayifuna izinsuku eziyisikhombisa ngesonto, amahora angu-24 ngosuku lapho udinga ngempela ukuthinta isisekelo nomunye umuntu.
Amaqembu asekelayo ayasiza ekubolekiseni abantu abaningi ukusekelwa ngokomzwelo futhi kuyindlela yokugcina izinto ezitholakalayo zakamuva kanye nokwelashwa kwalesi sifo. Izibonelo zalabo ongajoyina zihlanganisa:
- I-Pulmonary Fibrosis Foundation
- Umphakathi ophefumulayo we-Pulmonary Fibrosis
- IziguliLomphakathi wePulmonary Fibrosis
Izwi elivela
Ukuchazwa kwesifo se-idiopathic pulmonary pulsary fibrosis kuyahlukahluka kakhulu, nabanye abantu abanezifo eziqhubekayo ngokushesha, nabanye abahlala bezinzile iminyaka eminingi. Kunzima ukubikezela ukuthi inkambo izoba yimuphi isiguli esisodwa. Isilinganiso sokusinda esiphakathi kwaba ngu-3.3 eminyakeni ka-2007 ngokuqhathaniswa neminyaka engu-3.8 ngo-2011. Olunye ucwaningo lwathola ukuthi abantu abaneminyaka engama-65 nangaphezulu baphila isikhathi eside nge-IPF ngo-2011 kunokuba babekho ngo-2001.
Ngisho ngaphandle kwemithi esanda kuvunyelwa, ukunakekelwa kubonakala kuthuthuka. Ungathembeli kolwazi oludala olutholayo, okungenzeka ukuthi alukho usuku. Khuluma nodokotela wakho mayelana nezinketho ezichazwe lapha futhi okukuhle kuwe.
Imithombo:
Antoniou, K., Tomassetti, S., Tsitoura, E., noCancheri. Umdlavuza we-Idiopathic pulmonary ne-lung lung: isibuyekezo somtholampilo nesipathogenesis. Ama-Opinions amanje ku-Pulmonary Medicine . 2015 Sep 18. (Epub ngaphambi kokuphrinta).
de Boer, K., noJee Lee. Ukuncintisana okungaziwa ngaphansi kwe-fibrosis ye-idiopathic pulmonary: ukubuyekezwa. I-Respirology . 2015 Sep 13. (Epub ngaphambi kokuphrinta).
O'Riordan, T., Smith, V., noG. Raghu. Ukuthuthukiswa kwama-Novel Agents for Idiopathic Pulmonary Fibrosis: ukuqhubekela phambili ekukhethweni kwelitshe kanye nokuklanywa kwesilingo somtholampilo. Isifuba . 2015 Meyi 8. (Epub ngaphambi kokuphrinta).
Raghu, G. et al. Ukuhlolisisa i-idiopathic pulmonary pulmonary fibrosis nge-CT-high-resolution CT ezigulini ezinobumbalwa obuncane noma obungenabuzwayo obuhle bokuthi u-honeycombing: ukuhlaziywa kwesibili kokulingwa okungahleliwe nokulawulwa. I-Lancet Imithi Yokuphefumula . 2014. 2 (4): 277-84.
IsiSpanishi, uP., Maher, T., noL. Richeldi. I-fibrosis ye-Idiopathic pulmonary: Ukuthuthukiswa kwakamuva kwindlela yokwelashwa kwemithi. I-Pharmacology & Therapeutics . 2015. 152: 18-27.