Ingabe unezimpawu ezifana nokukhathala okuqhubekayo, ukuphelelwa umoya uma uhamba kancane, noma umchamo onsundu omnyama ekuseni? Ngezinye izikhathi lezi zingaba izimpawu zesifo segazi esingavamile esibizwa ngokuthi i-Paroxysmal Nocturnal Hemoglobinuria, noma i-PNH njengoba thina odokotela sikubiza kanjalo. Ungesabi uma uzwa leli gama. Nakuba liyinkimbinkimbi kancane, ake sixoxe ngalesi sifo kanye nokwelashwa okusha kwaso okubizwa ngokuthi i-Fabhalta ngamagama alula futhi anemininingwane.
Iyini ngempela i-PNH?
Kalula nje, i-PNH iyisifo segazi esingavamile kakhulu esibangelwa yisici sofuzo. Amaseli ethu abomvu egazi (ama-RBC) ane-coating yeprotheni evikelayo ewazungezile. Kodwa-ke, ku-PNH, le coating evikelayo ayakheki.
Cabanga ngamaseli ethu abomvu egazi njengamasosha angenazo izikhali zempi. Ngaphandle kwalokhu kuvikeleka, amasosha omzimba wethu ayadideka bese eqala ukuhlasela nokubhubhisa la maseli abomvu egazi aphilile, ecabanga ukuthi ayizitha. Lokhu kuqhekeka kwamangqamuzana abomvu egazi ngaphambi kwesikhathi yilokho esikubiza ngokwezokwelapha ngokuthi i-hemolysis . Lokhu kubhujiswa kungenzeka ngaphakathi kwemithambo yegazi (ngaphakathi kwemithambo yegazi) nangaphandle kwemithambo yegazi (ngaphandle kwemithambo yegazi).
Uma amangqamuzana abomvu ebhujiswa ngale ndlela, izimpawu eziyinhloko zingaba ukuntuleka kwegazi kanye nokuqhekeka kwegazi. Lawa maqhubu egazi ngezinye izikhathi angaba yingozi empilweni. Umuntu one-PNH angase abe nezimpawu eziningi zalezi, noma angaba nezimbalwa nje.
| Izimpawu ezivamile zesifo se-PNH | |
|---|---|
| Ukukhathala noma ubuthakathaka obuhlala njalo | Ubunzima bokuphefumula |
| Ikhanda elibuhlungu | Ukushaya kwenhliziyo okungajwayelekile noma okusheshayo |
| Ubuhlungu besisu | Isikhumba esibuhlungu kalula |
| Umchamo omnyama (onsundu), ikakhulukazi ebusuku noma ekuseni | Ubomvu, ubuhlungu, nokuvuvukala endaweni ethile yesikhumba (kungaba uphawu lwegazi eliqhekekile) |
Ukwelashwa kwe-PNH kuhlanganisa ukumpontshelwa igazi, amaphilisi e-folic acid noma ensimbi e-anemia, ama-anticoagulant, kanye nemithi evikela amangqamuzana abomvu egazi ekuhlaselweni yisimiso somzimba sokuzivikela. Ukwelashwa okutholayo kuzoncika ekutheni unezimpawu ezingaki nokuthi zimbi kangakanani.
Usebenza kanjani umuthi iFabhalta?
Imithi efana ne-eculizumab (Soliris) noma i-ravulizumab (Ultomiris) evame ukunikezwa njengemijovo ye-PNH isebenza ngokuvimba ingxenye yesimiso sethu somzimba esibizwa ngokuthi uhlelo lokugcwalisa, olubizwa nge-C5. Nakuba lokhu kuvimbela i-intravascular hemolysis, abanye abantu bangase baqhubeke nokuba ne-anemia ngenxa ye-extravascular hemolysis.
Yilapho umuthi wokuqala ophuzwayo obizwa ngokuthi iFabhalta uba khona obalulekile.
Cabanga ngesimiso sethu somzimba sokuzivikela njengebutho elivikela izwe lethu. Ku-PNH, izingxenye zaleli butho ziyadideka bese zihlasela amangqamuzana ethu egazi abomvu aphilile. I-Fabhalta ivimba iphrotheni ebizwa ngokuthi i-factor B ebangela lokhu kudideka. Lokhu kuvimbela isimiso somzimba sokuzivikela ekuhlaseleni amangqamuzana egazi abomvu aphilile. Lokhu kwandisa inani lamangqamuzana egazi abomvu aphilile, kunciphisa izimpawu, futhi kunciphisa isidingo sokumpontshelwa igazi.
Ngingawusebenzisa kanjani lo muthi?
I-Fabhalta iyi-capsule egwinywa iphelele kabili ngosuku, noma ingadliwe. Ungayiphuli, ungayichobozi, noma uyihlafune. Uma weqa umthamo, yithathe ngokushesha nje lapho ukhumbula bese uthatha imithamo esele njengenjwayelo. Uma usuvele uthatha omunye umuthi we-PNH (njenge-eculizumab noma i-ravulizumab), udokotela wakho uzochaza indlela efanele yokushintshela ku-Fabhalta.
Kubaluleke kakhulu: Kubalulekile ukuthi uthole yonke imijovo yakho edingekayo okungenani amasonto amabili ngaphambi kokuqala lokhu kwelashwa. Lokhu kufaka phakathi umuthi wokugomela i-pneumococcal kanye nomuthi wokugomela i-meningitis. Uma ungakwazi ukuthola imijovo yakho futhi ungakwazi ukuqala i-Fabhalta, udokotela wakho uzokunikeza imijovo bese ekuqalisa inkambo yama-antibiotic ngesikhathi esifanayo.
Ngisho noma izimpawu zakho zithuthuka, ungayeki ukuphuza i-Fabhalta ngaphandle kweseluleko sikadokotela . Uma wenza kanjalo, amangqamuzana akho abomvu egazi angase aqale ukuwohloka ngokushesha futhi.
Ihlolwe kanjani imiphumela yalo muthi?
Kwenziwe izifundo ezimbili eziyinhloko ukuqinisekisa ukusebenza kahle nokuphepha kwe-Fabhalta. Zombili zazihlanganisa iziguli ezine-PNH kanye ne-anemia eyabangelwa yiyo.
1. Ucwaningo lwe-APPLY-PNH: Lokhu kwahilela abantu ababene-anemia naphezu kokuthatha eminye imithi ye-PNH (i-eculizumab noma i-ravulizumab) okungenani izinyanga eziyi-6. Bahlukaniswa baba amaqembu amabili, iqembu elilodwa lanikwa i-Fabhalta, kanti elinye iqembu laqhubeka nokuthatha imithi endala.
2. Ucwaningo lwe-APPOINT-PNH: Lokhu kwahilela iziguli ezazingakaze zithole eminye imithi eyengeziwe ye-PNH ngaphambilini. Zonke zanikwa i-Fabhalta.
Imiphumela yalezi zifundo, ngemva kwamasonto angama-24, iyakhuthaza kakhulu.
| Inzuzo etholiwe | Umphumela |
|---|---|
| Izinga le-hemoglobin elandayo | Iziguli ezingaphezu kuka-82% ezathatha i-Fabhalta zazinokwenyuka kwe-hemoglobin okungu-2 g/dL, uma kuqhathaniswa neyodwa yalezo ezathatha eminye imithi. |
| Ukugwema ukumpontshelwa igazi | Ama-95% eziguli ezathatha i-Fabhalta azizange zidinge ukumpontshelwa igazi ngaphandle ngesikhathi socwaningo, uma kuqhathaniswa nama-50% kuphela alabo abathatha eminye imithi. |
Khumbula, naphezu kwemiphumela yalezi zifundo, imiphumela yakho ingahluka kumuntu nomuntu. Kungcono ukuxoxa ngezinzuzo nezingozi zomuthi nodokotela wakho ngaphambi kokwenza isinqumo.
Wazi kanjani ukuthi umuthi uyasebenza?
Uma ukwelashwa kwe-Fabhalta kuphumelela, amazinga akho abomvu egazi kanye ne-hemoglobin azoqala ukwanda. Ungase ubhekane nezinto ezifana nalezi:
- Amandla akhuphukile kanye nokukhathala okunciphile
- Ukuncipha kombala omnyama womchamo
- Ukwanda kwesifiso sokudla kanye nokuncipha kobuhlungu besisu
- Ukunciphisa isidingo sokumpontshelwa igazi ngaphandle
- Ukukwazi ukwenza imisebenzi yansuku zonke kalula
Njengoba imiphumela yokwelashwa ihlukahluka kumuntu nomuntu, gcina irekhodi lezimpawu zakho bese uxoxa ngazo nodokotela wakho njalo uma umbona.
Imiphumela emibi nokuthi ungayiphatha kanjani?
Njenganoma yimuphi umuthi, i-Fabhalta ingadala imiphumela emibi. Evame kakhulu ikhanda elibuhlungu, isifo sohudo, isicanucanu, ubuhlungu besisu, kanye nokuqubuka kwesikhumba. Izimpawu zomkhuhlane ovamile kanye nezinye izifo ezibangelwa amagciwane noma amagciwane nazo zingase zivele.
Izifo ezingathi sína ezidinga ukunakekelwa!
Ngenxa yokuthi lo muthi ucindezela ingxenye yesimiso somzimba sokuzivikela, kunengozi yokutheleleka okukhulu okuvela ezinhlotsheni ezithile zamagciwane. Ikakhulukazi i-*Streptococcus pneumoniae*, i-*Neisseria meningitidis*, kanye ne-*Haemophilus influenzae type B*. Lokhu kutheleleka kungaba yingozi empilweni uma kungabonwa futhi kwelashwa ngokushesha.
Indlela engcono kakhulu yokuvimbela ukutheleleka ukuthola yonke imijovo enconywayo , ugeze izandla zakho kahle, futhi uhlale kude nabantu abagulayo. Uma uhlangabezana nanoma yiziphi izimpawu ezilandelayo, tshela udokotela wakho ngokushesha noma uye egumbini lezimo eziphuthumayo esibhedlela esiseduze (i-ETU).
| Izimpawu eziyisixwayiso zokutheleleka okukhulu | |
|---|---|
| Umkhuhlane one-phlegm noma ongenawo, ubuhlungu besifuba nokukhwehlela, ukushaya kwenhliziyo okusheshayo | Izimpawu ezifana nobuhlungu bomzimba kanye nomkhuhlane |
| Isikhumba esinama-clammy | Ikhanda elibuhlungu elihambisana nomkhuhlane, isicanucanu, nokuhlanza, noma ukuqina kwentamo/umhlane |
| Ukudideka | Izinguquko zombono |
Uma ukhathazekile nganoma yimiphi imiphumela emibi, khuluma nodokotela wakho ngokushesha. Uma usola ukuthi umuthi uqukethe ubuthi, ungashayela iNational Poisons Information Centre eColombo National Hospital .
Yikuphi ukuhlolwa kwegazi okudingekayo ngesikhathi sokwelashwa?
Udokotela wakho uzokwenza ukuhlolwa kwegazi okuningana ukuze aqaphe impilo yakho kanye nendlela osabela ngayo emithini.
- Amazinga amangqamuzana abomvu egazi (ama-RBC) kanye ne-hemoglobin:Lokhu kuhlolwa njalo ku-PNH. Lokhu kusiza ukubona ukuthi usabela kanjani ekwelashweni kanye nokunquma ukuthi udinga ukumpontshelwa igazi yini.
- Amazinga amafutha egazini: I-Fabhalta ingakhuphula amazinga akho e-cholesterol kanye ne-triglyceride. Ngakho-ke, udokotela wakho uzoncoma ukuhlolwa kwephrofayili yamafutha ngaphambi nangesikhathi sokwelashwa.
Ingabe kungaba nezinkinga ngeminye imithi?
Yebo, eminye imithi ingasebenzisana ne-Fabhalta. Eminye imithi, njenge-Rifampin, inganciphisa ukusebenza kwe-Fabhalta. Akukhuthazwa ukuthatha i-Fabhalta neminye imithi, njenge-Gemfibrozil.
Ngakho-ke, kubaluleke kakhulu ukwazisa udokotela wakho ngayo yonke imithi oyithathayo - okuhlanganisa imithi kadokotela, imithi ethengiswa ngaphandle kwemithi kadokotela, amavithamini, imithi yendabuko, ngisho nezithasiselo.
Umlayezo Wokuya Nawe Ekhaya
- I-PNH yisimo esingavamile lapho amasosha omzimba ebhubhisa khona amangqamuzana abomvu egazi ngoba antula ungqimba lwamaprotheni oluwavikelayo.
- I-Fabhalta umuthi omusha ophuzwayo we-PNH osebenza ngokuvimbela ukungasebenzi kahle kwesimiso somzimba sokuzivikela kanye nokuvikela amangqamuzana abomvu egazi.
- Ngaphambi kokuqala lo muthi, kubalulekile ukuthi ube usugonyelwe izifo ezifana ne-meningitis kanye ne-pneumonia.
- Uma uba nezimpawu zokutheleleka okukhulu ngesikhathi sokwelashwa, njengomkhuhlane, ikhanda elibuhlungu, noma ukudideka, funa iseluleko sezokwelapha ngokushesha.
- Ngisho noma izimpawu zakho ziba ngcono, ungalokothi uyeke ukuthatha imithi ngaphandle kweseluleko sikadokotela wakho.
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