ICystic fibrosis

ICystic fibrosis yisifo esidala ukuthi amafinyila aqine, anamathele akhele emaphashini, emgodini wokugaya ukudla nakwezinye izindawo zomzimba. Ingesinye sezifo ezivame ukwedlula zonke zamaphaphu ezinganeni nasebancane. Kuyisifo esisongela impilo.

ICystic fibrosis (CF) yisifo esidluliselwa ngemindeni. Idalwa yisakhi sofuzo esingalungile esenza ukuthi umzimba ukhiqize uketshezi olujiyile olunamathele ngokungavamile, olubizwa ngokuthi amafinyila. Lo mfinyila wakhela ezindaweni zokuphefumula zamaphaphu nasemanyikwe.

Ukwakheka kwamafinyila kubangela ukutheleleka kwamaphaphu okusongela ukuphila nezinkinga ezinkulu zokugaya ukudla. Lesi sifo singaphinde sithinte izindlala zomjuluko kanye nohlelo lokuzala lomuntu.

Abantu abaningi baphatha isakhi sofuzo se-CF, kodwa abanazo izimpawu. Lokhu kungenxa yokuthi umuntu one-CF kufanele azuze izakhi zofuzo ezinesici ezimbili, 1 kumzali ngamunye. Abanye baseMelika banesakhi sofuzo seCF. Kuvame kakhulu kulabo abavela enyakatho noma enkabeni yeYurophu.

Iningi lezingane ezine-CF zitholwa ngeminyaka engu-2, ikakhulukazi njengoba ukuhlolwa kwezinsana kwenziwa e-United States. Ngenombolo encane, lesi sifo asitholakali kuze kube yiminyaka engu-18 noma ngaphezulu. Lezi zingane zivame ukuba nesimo sesifo esibi.

Izimpawu ezinganeni ezisanda kuzalwa zingabandakanya:

• Ukukhula okubambezelekile
• Ukwehluleka ukuthola isisindo ngokujwayelekile ngesikhathi sobuntwana
• Akukho ukunyakaza kwamathumbu emahoreni okuqala angama-24 kuye kwangama-48 empilo
• Isikhumba esinambitha usawoti

Izimpawu ezihlobene nokusebenza kwamathumbu zingafaka:

• Ubuhlungu besisu obuvela ekuqunjelweni okukhulu
• Ukwenyuka kwegesi, ukuqunjelwa, noma isisu esivela ukuvuvukala (okuhlukanisiwe)
• I-nausea nokulahlekelwa isifiso sokudla
• Izindle ezinombala okhanyayo noma onobumba, iphunga elibi, zinamafinyila, noma ezintantayo
• Ukwehla kwesisindo

Izimpawu ezihlobene namaphaphu nezono zingafaka:

• Ukukhwehlela noma ukukhulisa amafinyila ezonweni noma emaphashini
• Ukukhathala
• Ukucinana kwamakhala okubangelwa ama-polyp acinene
• Iziqephu eziphindaphindiwe ze-pneumonia (izimpawu ze-pneumonia kumuntu one-cystic fibrosis zifaka imfiva, ukukhwehlela okwandayo nokuphefumula okuncane, ukukhuphuka kwe-mucus, nokulahlekelwa isifiso
• Ubuhlungu besono noma ingcindezi ebangelwa ukutheleleka noma ama-polyps

Izimpawu ezingaqashelwa kamuva empilweni:

• Ukungazali (emadodeni)
• Ukuvuvukala okuphindiwe kwamanyikwe (i-pancreatitis)
• Izimpawu zokuphefumula
• Iminwe ehlutshiwe

Ukuhlolwa kwegazi kwenziwa ukusiza ukuthola i-CF. Ukuhlolwa kubheka ushintsho kuhlobo lweCF. Ezinye izivivinyo ezisetshenziselwa ukuxilonga i-CF zifaka:

• Isivivinyo se-Immunoreactive trypsinogen (IRT) isivivinyo esivamile sokuhlolwa kwe-CF. Izinga eliphakeme le-IRT liphakamisa ukuthi kungenzeka i-CF futhi idinga ukuhlolwa okwengeziwe.
• Ukuhlolwa kwe-Sweat chloride ukuhlolwa okujwayelekile kokuxilongwa kwe-CF. Izinga likasawoti eliphakeme emjulukweni womuntu kuwuphawu lwesifo.

Ezinye izivivinyo ezikhomba izinkinga ezingahlobana ne-CF zifaka:

• I-x-ray yesifuba noma i-CT scan
• Ukuhlolwa kwamafutha endle
• Ukuhlolwa kwemisebenzi yamaphaphu
• Ukulinganiswa komsebenzi we-pancreatic (isitokisi se-pancreatic elastase)
• Isivivinyo sokukhuthaza i-Secretin
• I-Trypsin ne-chymotrypsin esitulweni
• I-Upper GI nochungechunge lwamathumbu amancane
• Amasiko emiphunga (atholakala ngesikhwehlela, i-bronchoscopy noma i-throat swab)

Ukuxilongwa kusenesikhathi kwe-CF necebo lokwelashwa kungathuthukisa ukusinda kanye nekhwalithi yempilo. Ukulandelela nokuqapha kubaluleke kakhulu. Uma kunokwenzeka, ukunakekelwa kufanele kutholwe emtholampilo okhethekile we-cystic fibrosis. Lapho izingane zifinyelela ebudaleni, kufanele zidlulisele esikhungweni esikhethekile se-cystic fibrosis sabantu abadala.

Ukwelashwa kwezinkinga zamaphaphu kufaka phakathi:

• Ama-antibiotic ukuvikela nokwelapha ukutheleleka kwamaphaphu nesinus. Zingathathwa ngomlomo, noma zinikezwe emithanjeni noma ngezindlela zokuphefumula. Abantu abane-CF bangathatha ama-antibiotic kuphela uma kudingeka, noma ngaso sonke isikhathi. Ukweqiwa kuvame ukuphakama kunokujwayelekile.
• Imithi ehogelwayo yokusiza ukuvula imigwaqo yomoya.
• Eminye imishanguzo enikezwa ukwelashwa kokuphefumula kumafinyila amancane nokwenza kube lula ukukhwehlela yi-DNAse enzyme therapy kanye nezixazululo zikasawoti ezigxile kakhulu (i-hypertonic saline).
• Umuthi wokugomela umkhuhlane kanye nomuthi wokugoma we-pneumococcal polysaccharide (PPV) minyaka yonke (buza umhlinzeki wakho wezokunakekelwa kwempilo).
• Ukufakelwa kwamaphaphu kuyindlela kwezinye izikhathi.
• Ukwelashwa kwe-oxygen kungadingeka njengoba isifo samaphaphu siba sibi kakhulu.

Izinkinga zamaphaphu zibuye ziphathwe ngezindlela zokwelapha ukuze kunciphise amafinyila. Lokhu kwenza kube lula ukukhwehlela amafinyila ngaphandle kwamaphaphu.

Lezi zindlela zifaka:

• Umsebenzi noma ukuzivocavoca umzimba okubangela ukuthi uphefumule kakhulu
• Amadivayisi asetshenziswa phakathi nosuku ukusiza ukusula imigwaqo yomoya amafinyila amaningi
• Ukushaywa kwesifuba ngesandla (noma i-physiotherapy yesifuba), lapho ilungu lomndeni noma umelaphi eshaya kancane isifuba somuntu, emuva nendawo yakhe ngaphansi kwezingalo

Ukwelashwa kwezinkinga zamathumbu nezomsoco kungafaka:

• Ukudla okukhethekile okunamaprotheni amaningi namakhalori ezingane ezindadlana nabantu abadala
• Ama-enzyme epancreatic okusiza ukumunca amafutha namaprotheni, athathwa njalo ngesidlo
• Izithako zevithamini, ikakhulukazi amavithamini A, D, E, noK
• Umhlinzeki wakho angakweluleka ngezinye izindlela zokwelapha uma unezihlalo ezinzima kakhulu

I-Ivacaftor, i-lumacaftor, i-tezacaftor, ne-elexacaftor yimithi elapha izinhlobo ezithile ze-CF.

• Zithuthukisa ukusebenza kwesinye sezakhi zofuzo esingalungile esidala i-CF.
• Kuze kube ngama-90% eziguli ezine-CF futhi ezifanelekela ukuthola eyodwa noma ngaphezulu yale mithi iyodwa noma ihlanganisiwe.
• Ngenxa yalokhu, kunokwakheka okuncane kwenqwaba yamaphaphu aminyene emaphashini. Ezinye izimpawu ze-CF ziyathuthukiswa futhi.

Ukunakekelwa nokuqapha ekhaya kufanele kufaka:

• Ukugwema intuthu, uthuli, ukungcola, intuthu, amakhemikhali asendlini, intuthu yomlilo, nesikhunta noma isikhutha.
• Ukunikeza uketshezi oluningi, ikakhulukazi ezinganeni nasezinganeni lapho kushisa, lapho kunesifo sohudo noma indle engasebenzi, noma ngesikhathi sokuzivocavoca umzimba okwengeziwe.
• Ukuzivocavoca izikhathi ezi-2 noma ezi-3 isonto ngalinye. Ukubhukuda, ukugijima nokuhamba ngebhayisikili yizindlela ezinhle ongakhetha kuzo.
• Ukususa noma ukukhuphula amafinyila noma uketshezi oluvela ezindleleni zomoya. Lokhu kufanele kwenziwe izikhathi ezi-1 kuye kwezingu-4 usuku ngalunye. Iziguli, imindeni, kanye nabanakekeli kumele bafunde ngokwenza ukushaywa kwesifuba kanye nemisele yamanzi yangemuva ukusiza ukugcina imigwaqo yomoya icacile.
• Akukho ukuxhumana nabanye abantu abane-CF okunconywayo njengoba bekwazi ukushintshana ngezifo (akusebenzi kumalungu omndeni).

Unganciphisa ukucindezela kokugula ngokujoyina iqembu le-cystic fibrosis support. Ukwabelana nabanye abanokuhlangenwe nakho okuvamile nezinkinga kungasiza umndeni wakho ukuthi ungazizwa uwedwa.

Iningi lezingane ezine-CF zihlala zinempilo enhle zize zifinyelele ebudaleni. Bayakwazi ukubamba iqhaza emisebenzini eminingi futhi baye esikoleni. Abantu abasha abaningi abane-CF baqeda ikolishi noma bathole imisebenzi.

Isifo samaphaphu sigcina sesiba sibi kuze kube sezingeni lokuthi umuntu akhubazeke. Namuhla, isilinganiso sobude besikhathi sokuphila kubantu abane-CF abaphila kuze kube badala singaba yiminyaka engama-44.

Ukufa kuvame ukubangelwa izinkinga zamaphaphu.

Inkinga ejwayelekile kunazo zonke ukutheleleka okungapheli kokuphefumula.

Ezinye izinkinga zifaka:

• Izinkinga zamathumbu, njengama-gallstones, ukuvinjelwa kwamathumbu, kanye ne-rectal prolapse
• Akhwehlele igazi
• Ukwehluleka kokuphefumula okungapheli
• Isifo sikashukela
• Ukungabi nenzalo
• Isifo sesibindi noma ukwehluleka kwesibindi, i-pancreatitis, i-cirrhosis ye-biliary
• Ukungondleki
• Ama-polyp nasal kanye ne-sinusitis
• I-osteoporosis ne-arthritis
• I-pneumonia elokhu ibuya njalo
• Pneumothorax
• Ukwehluleka kwenhliziyo emaceleni angakwesokudla (cor pulmonale)
• Umdlavuza we-Colorectal

Shayela umhlinzeki wakho uma usana noma ingane inezimpawu ze-CF, kanye nokuhlangenwe nakho:

• Umkhuhlane, ukukhwehlela okwandayo, ushintsho esikhwehleleni noma egazini esikhwehlelweni, ukungathandi ukudla, noma ezinye izimpawu zenyumoniya
• Ukwanda kwesisindo
• Ukuhamba kwamathumbu kaningi noma indle enephunga elibi noma enamafinyila amaningi
• Isisu esivuvukile noma ukuqunjelwa okwandayo

Shayela umhlinzeki wakho uma umuntu one-CF eba nezimpawu ezintsha noma uma izimpawu ziba zimbi kakhulu, ikakhulukazi ukuphefumula kanzima noma ukukhwehlela igazi.

I-CF ayikwazi ukuvinjelwa. Ukuhlola labo abanomlando womndeni wesifo kungathola isakhi sofuzo se-CF kubathwali abaningi.

CF

• Ukudla okunomsoco - izinkinga zokuphatha izingane
• Ishubhu yokudla ye-Gastrostomy - bolus
• Ungaphefumula kanjani uma ushoda ngokuphefumula
• Ishubhu yokudla yeJejunostomy
• I-postural drainage

• Ukubhuqa
• I-postural drainage
• Iminwe ehlutshiwe
• ICystic fibrosis

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UFarrell PM, i-White TB, uRen CL, et al. Ukuxilongwa kwe-cystic fibrosis: imihlahlandlela yokuvumelana evela kuCystic Fibrosis Foundation. J Pediatr. 2017; 181S: S4-S15.e1. I-PMID: 28129811 pubmed.ncbi.nlm.nih.gov/28129811/.

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UGrasemann H. ICystic fibrosis. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 83.

URowe SM, uHoover W, uSolomon GM, uSorscher EJ. ICystic fibrosis. Ku: Broaddus VC, Mason RJ, Ernst JD, et al, abakwa-eds. Incwadi kaMurray neNadel Yemithi Yokuphefumula. Umhlaka 6. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 47.

UTaylor-Cousar JL, uMunck A, uMcKone EF, et al. I-Tezacaftor-ivacaftor ezigulini ezine-cystic fibrosis homozygous ye-phe508del. N Engl J Med. I-2017; 377 (21): 2013-2023 PMID: 29099344 kushicilelwe.ncbi.nlm.nih.gov/29099344/.