Ngaba uneempawu ezifana nokudinwa rhoqo, ukuphelelwa ngumoya xa uhamba kancinci, okanye umchamo omnyama kusasa? Ngamanye amaxesha ezi zinokuba ziimpawu zesifo segazi esingaqhelekanga esibizwa ngokuba yiParoxysmal Nocturnal Hemoglobinuria, okanye iPNH njengoko thina oogqirha siyibiza njalo. Musa ukoyika xa usiva eli gama. Nangona liyinkimbinkimbi kancinci, masithethe ngesi sifo kunye nonyango olutsha lwaso olubizwa ngokuba yiFabhalta ngokulula nangokuneenkcukacha.
Yintoni kanye kanye i-PNH?
Ngamafutshane, i-PNH sisifo segazi esingaqhelekanga esibangelwa sisiphene semfuza. Iiseli zethu ezibomvu zegazi (ii-RBC) zine-protective protein coating ezijikelezileyo. Nangona kunjalo, kwi-PNH, le-protective coating ayidalwanga.
Cinga ngeeseli zethu ezibomvu zegazi njengamajoni angenazo izixhobo zokulwa. Ngaphandle kolu khuseleko, inkqubo yomzimba wethu yokuzikhusela iyadideka ize iqale ukuhlasela nokutshabalalisa ezi seli zibomvu zegazi ziphilileyo, icinga ukuba ziintshaba. Oku kuqhekeka kwangaphambi kwexesha kweeseli ezibomvu zegazi yinto esiyibiza ngokuba yi-hemolysis ngokwezonyango. Oku kutshabalaliswa kunokwenzeka ngaphakathi kwemithambo yegazi (intravascular) nangaphandle kwemithambo yegazi (extravascular).
Xa iiseli ezibomvu zegazi zitshatyalaliswa ngale ndlela, iimpawu eziphambili zinokuba yi-anemia kunye nokuqhekeka kwegazi. La mahlwili egazi ngamanye amaxesha anokuba yingozi ebomini. Umntu one-PNH unokufumana uninzi lwezi mpawu, okanye anokuba nazo ezimbalwa.
| Iimpawu eziqhelekileyo zesifo se-PNH | |
|---|---|
| Ukudinwa okanye ubuthathaka obuhlala buhleli | Ubunzima bokuphefumla |
| Intloko ebuhlungu | Ukubetha kwentliziyo okungaqhelekanga okanye okukhawulezayo |
| Intlungu yesisu | Ulusu olukrwelwe lula |
| Umchamo omnyama (omdaka), ingakumbi ebusuku okanye kusasa | Ubomvu, iintlungu, kunye nokudumba kwindawo ethile yolusu (kusenokuba luphawu lokuqhekeka kwegazi) |
Unyango lwe-PNH lubandakanya utofelo-gazi, iipilisi ze-folic acid okanye i-iron ze-anemia, amayeza okuthintela ukugabha kwegazi, kunye namayeza akhusela iiseli ezibomvu zegazi ekuhlaselweni yinkqubo yomzimba yokuzikhusela. Unyango olufumanayo luya kuxhomekeka ekubeni zingaphi iimpawu onazo kunye nokuba zinzima kangakanani.
Isebenza njani iyeza iFabhalta?
Amayeza afana ne-eculizumab (Soliris) okanye i-ravulizumab (Ultomiris) adla ngokunikezelwa njengeenaliti ze-PNH asebenza ngokuthintela inxalenye yenkqubo yethu yomzimba yokuzikhusela ebizwa ngokuba yi- complement system, ebizwa ngokuba yi-C5. Nangona la mayeza ethintela i-intravascular hemolysis, abanye abantu banokuqhubeka nokuba ne-anemia ngenxa ye-extravascular hemolysis.
Kulapho ke iyeza lokuqala elisetyenziswa ngomlomo elibizwa ngokuba yiFabhalta libaluleke khona.
Cinga ngenkqubo yethu yomzimba yokuzikhusela njengomkhosi okhusela ilizwe lethu. Kwi-PNH, iinxalenye zalo mkhosi ziyadideka zize zihlasele iiseli zethu ezibomvu zegazi ezisempilweni. I-Fabhalta ivimba iproteni ebizwa ngokuba yi-factor B ebangela olu bhideko. Oku kuyawuthintela umbutho wokuzikhusela ekuhlaseleni iiseli ezibomvu zegazi. Oku kwandisa inani leeseli ezibomvu zegazi ezisempilweni, kunciphisa iimpawu, kwaye kunciphisa isidingo sotofelo-gazi.
Ndingayisebenzisa njani le yeza?
I-Fabhalta yikhaphu eginywa yonke kabini ngemini, nokuba kutyiwe okanye akutyiwa. Musa ukuyiqhekeza, ukuyityumza, okanye ukuyihlafuna. Ukuba uphoswe yidosi, yithathe kwangoko xa uyikhumbula uze uthathe idosi eziseleyo njengesiqhelo. Ukuba sele uthatha elinye iyeza le-PNH (elifana ne-eculizumab okanye i-ravulizumab), ugqirha wakho uza kuchaza indlela echanekileyo yokutshintshela kwi-Fabhalta.
Kubaluleke kakhulu: Kubalulekile ukuba ufumane zonke iigonyo ozifunayo ubuncinane kwiiveki ezimbini ngaphambi kokuba uqale olu nyango. Oku kuquka ugonyo lwe-pneumococcal kunye nogonyo lwe-meningitis. Ukuba awukwazi ukufumana iigonyo zakho kwaye awukwazi ukuqala iFabhalta, ugqirha wakho uza kukunika iigonyo aze akuqalise ngekhosi yee-antibiotics ngaxeshanye.
Nokuba iimpawu zakho ziyaphucuka, ungayeki ukusebenzisa iFabhalta ngaphandle kwengcebiso kagqirha . Ukuba wenza njalo, iiseli zakho ezibomvu zegazi zisenokuqala ukuwohloka ngokukhawuleza kwakhona.
Imiphumo yale mithi iye yafundwa njani?
Izifundo ezimbini eziphambili zenziwe ukuqinisekisa ukusebenza kakuhle kunye nokhuseleko lweFabhalta. Zombini ziquka izigulane ezine-PNH kunye ne-anemia ebangelwe yiyo.
1. Uphononongo lwe-APPLY-PNH: Olu phando lubandakanye abantu ababene-anemia nangona bethathe amanye amayeza e-PNH (i-eculizumab okanye i-ravulizumab) ubuncinane iinyanga ezi-6. Bahlulwahlulwa baba ngamaqela amabini, elinye iqela lanikwa i-Fabhalta, kwaye elinye iqela laqhubeka nokuthatha amayeza amadala.
2. Uphononongo lwe-APPOINT-PNH: Olu lubandakanya izigulane ezazingazange zifumane naluphi na olunye unyango oluncedisayo lwe-PNH ngaphambili. Zonke zanikwa i-Fabhalta.
Iziphumo zezi zifundo, emva kweeveki ezingama-24, ziyakhuthaza kakhulu.
| Inzuzo efunyenweyo | Isiphumo |
|---|---|
| Ukwanda kwenqanaba le-hemoglobin | Ngaphezulu kwama-82% ezigulana ezithathe iFabhalta zazinokunyuka kwe-hemoglobin ye-2 g/dL, xa kuthelekiswa nanye kwezo zithathe ezinye iziyobisi. |
| Ukuphepha utofelo-gazi | Ama-95% ezigulana ezithathe iFabhalta azizange zifune utofelo-gazi lwangaphandle ngexesha lophando, xa kuthelekiswa nama-50% kuphela abo bathathe amanye amayeza. |
Khumbula, nangona iziphumo zezi zifundo ziphumile, iziphumo zakho zinokwahluka kumntu nomntu. Kungcono ukuxoxa ngeenzuzo kunye neengozi zamayeza nogqirha wakho ngaphambi kokuba wenze isigqibo.
Uyazi njani ukuba iyeza liyasebenza?
Ukuba unyango lweFabhalta luphumelele, amanqanaba eeseli zakho ezibomvu zegazi kunye ne-hemoglobin aya kuqala ukunyuka. Usenokuhlangabezana nezinto ezifana nezi:
- Amandla anyukileyo kunye nokudinwa okunciphileyo
- Ukuncipha kombala omnyama womchamo
- Ukwanda kokutya kunye nokuncipha kwentlungu yesisu
- Ukunciphisa isidingo sotofelo-gazi lwangaphandle
- Ukukwazi ukwenza imisebenzi yemihla ngemihla ngokulula
Ekubeni iziphumo zonyango zahlukile kumntu nomntu, gcina ingxelo yeempawu zakho kwaye uxoxe ngazo nogqirha wakho ngalo lonke ixesha xa umbona.
Iziphumo ebezingalindelekanga kunye nendlela yokuzilawula?
Njengayo nayiphi na iyeza, iFabhalta inokubangela iziphumo ebezingalindelekanga. Ezona zixhaphakileyo ziintloko ezibuhlungu, urhudo, isicaphucaphu, iintlungu zesisu, kunye nokurhawuzelela kolusu. Iimpawu zomkhuhlane oqhelekileyo kunye nezinye izifo zebhaktiriya okanye zentsholongwane nazo zinokuvela.
Iintsholongwane ezinzulu ezifuna ingqalelo!
Ngenxa yokuba eli yeza lithintela inxalenye yenkqubo yomzimba yokuzikhusela, kukho umngcipheko wosulelo olukhulu oluvela kwiintlobo ezithile zeebhaktheriya. Ingakumbi *Streptococcus pneumoniae*, *Neisseria meningitidis*, kunye *Haemophilus influenzae type B*. Ezi sulelo zinokuba yingozi ebomini ukuba aziqatshelwa kwaye zinyangwe ngokukhawuleza.
Eyona ndlela ilungileyo yokuthintela usulelo kukufumana zonke iigonyo ezicetyiswayo , uhlambe izandla zakho kakuhle, kwaye uhlale kude nabantu abagulayo. Ukuba ufumana naziphi na ezi mpawu zilandelayo, xelela ugqirha wakho ngoko nangoko okanye uye kwigumbi likaxakeka lesibhedlele elikufutshane (ETU).
| Iimpawu ezilumkisayo zosulelo olunzima | |
|---|---|
| Umkhuhlane kunye okanye ngaphandle kwe-phlegm, iintlungu zesifuba kunye nokukhwehlela, ukubetha kwentliziyo ngokukhawuleza | Iimpawu ezifana neentlungu zomzimba kunye nomkhuhlane |
| Ulusu oluxineneyo | Intloko ebuhlungu kakhulu enomkhuhlane, isicaphucaphu, nokuhlanza, okanye ukuqina kwentamo/umqolo |
| Ukudideka | Utshintsho lombono |
Ukuba ukhathazekile malunga nemiphumo emibi, thetha nogqirha wakho ngokukhawuleza. Ukuba ukrokrela ukuba kukho ityhefu evela kumayeza, ungatsalela umnxeba iZiko loLwazi lweTyhefu leSizwe kwiSibhedlele sikaZwelonke saseColombo .
Zeziphi iimvavanyo zegazi ezifunekayo ngexesha lonyango?
Ugqirha wakho uza kwenza uvavanyo lwegazi oluninzi ukujonga impilo yakho kunye nendlela osabela ngayo kumayeza.
- Amanqanaba eeseli ezibomvu zegazi (ii-RBC) kunye ne-hemoglobin:Ezi zijongwa rhoqo kwi-PNH. Oku kunceda ukubona indlela osabela ngayo kunyango kunye nokufumanisa ukuba ufuna utofelo-gazi na.
- Amanqanaba amafutha egazini: I-Fabhalta inokunyusa amanqanaba akho e-cholesterol kunye ne-triglyceride. Ke ngoko, ugqirha wakho uya kucebisa uvavanyo lweprofayili ye-lipid yokuzila ngaphambi nangexesha lonyango.
Ngaba kukho iingxaki ngamanye amayeza?
Ewe, amanye amayeza anokusebenzisana neFabhalta. Amanye amayeza, afana neRifampin, anokunciphisa ukusebenza kweFabhalta. Akukhuthazwa ukuthatha iFabhalta kunye namanye amayeza, afana neGemfibrozil.
Kungoko ke, kubaluleke kakhulu ukwazisa ugqirha wakho ngazo zonke iindlela ozisebenzisayo – kuquka amayeza abhalwe ngugqirha, amayeza athengiswa ngaphandle kwemvume kagqirha, iivithamini, amayeza esintu, kwanezongezo.
Umyalezo Wokuya Ekhaya
- I-PNH yimeko engaqhelekanga apho amajoni omzimba atshabalalisa iiseli ezibomvu zegazi kuba zingenalo umaleko weproteni ozikhuselayo.
- I-Fabhalta liyeza elitsha elithathwa ngomlomo le-PNH elisebenza ngokuthintela ukungasebenzi kakuhle kwenkqubo yomzimba yokuzikhusela kunye nokukhusela iiseli ezibomvu zegazi.
- Ngaphambi kokuba uqale eli yeza, kunyanzelekile ukuba ube sele ugonyelwe izifo ezifana ne-meningitis kunye ne-pneumonia.
- Ukuba ufumanisa iimpawu zosulelo olukhulu ngexesha lonyango, ezinje ngomkhuhlane, intloko ebuhlungu, okanye ukudideka, funa ingcebiso kagqirha ngoko nangoko.
- Nokuba iimpawu zakho ziyaphucuka, ungaze uyeke ukuthatha amayeza ngaphandle kwengcebiso kagqirha wakho.
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