Xa ufumana inxeba elincinci okanye ubetheka ndaweni ithile, ukopha kuyayeka emva kwexesha elithile, akunjalo? Oko kungenxa yenkqubo yendalo yokujiya kwegazi emizimbeni yethu. Kodwa cinga ngako, abanye abantu abayeki ukopha ngokulula. Nokuba inxeba elincinci, bopha ixesha elide. Umzimba ujika ube luhlaza okwesibhakabhaka kwaye ube nemivumbo. Unobangela wale meko unokuba sisifo esibizwa ngokuba yi-hemophilia. Nangona abantu abaninzi bengazi kakuhle ngale nto, le yinto ebalulekileyo ekufuneka siyazi. Masithethe ngale nto ngokulula, ngendlela onokuyiqonda.
Yintoni kanye kanye i-hemophilia?
Ngamafutshane, i-hemophilia yimeko engaqhelekanga apho igazi lethu lingajiki kakuhle. Igazi lethu lineeproteni ezikhethekileyo ezinceda ekumiseni ukopha xa liqala. Sizibiza ngokuba "zizinto ezijikiyo." Njengokuba i-mortar ifuneka ukuze kubanjwe izitena xa kusakhiwa udonga, ezi zinto zijikiyo zibalulekile ekumiseni ukopha.
Umntu one-hemophilia akavelisi ngokwaneleyo enye okanye ezingaphezulu kwezi zinto zokujiya kwamalungu omzimba. Oku kuthetha ukuba nokuba nenxeba elincinci linokubangela ukopha ixesha elide. Ngamanye amaxesha, ukopha kunokubakho ngaphakathi emzimbeni, ingakumbi kumalungu nakwimisipha. Oku kunokuba yimeko esongela ubomi.
Oogqirha bahlula ubukhali besi sifo zibe ziindidi ezintathu, kuxhomekeke kubungakanani bezinto ezibangela ukugabha kwegazi.
- I-hemophilia engephi: Ukopha kudla ngokuvela emva kokwenzakala okukhulu okanye utyando.
- I-hemophilia ephakathi: Nokuba ukwenzakala okuncinci kunokubangela ukopha kakhulu. Umzimba unokuba luhlaza okwesibhakabhaka.
- Ukopha kakhulu: Ukopha kuyenzeka ngaphakathi nangaphandle, nokuba akukho sizathu sicacileyo.
Nangona kungekho nyango lupheleleyo loku, ngonyango olutsha olufumanekayo, kunokwenzeka ukulawula ukopha kwegazi nokuphila ubomi obuqhelekileyo.
Ngaba kukho iintlobo eziphambili ze-hemophilia?
Ewe, kukho iintlobo ezintathu eziphambili zesi sifo. Zisekelwe kuhlobo lwe-clotting factor engekhoyo emzimbeni. Ukuze ukuqonde kakuhle oku, jonga itheyibhile engasezantsi.
| Uhlobo lwe-hemophilia | Inkcazo |
|---|---|
| I-Hemophilia A | Olu lolona hlobo luxhaphakileyo. Lwenzeka xa kungekho nto yaneleyo yokunqamla igazi i-factor 8, okanye i-Factor VIII . |
| I-Hemophilia B | Olu hlobo lwenzeka xa kukho ukunqongophala okwaneleyo kwe-blood clotting factor 9, okanye i-Factor IX . |
| I-Hemophilia C | Olu lolona hlobo lungaqhelekanga. Lubangelwa kukunqongophala kwe-blood clotting factor 11, okanye i-Factor XI . |
Zithini iimpawu zesi sifo?
Iimpawu eziphambili ze-hemophilia zizinto ezingaqhelekanga okanye ukopha kakhulu kunye nokukrweleka. Ubunzima bezi mpawu buxhomekeke ekubeni une-hemophilia engembi kangako, ephakathi, okanye enzima.
Ukopha kakhulu
Khawucinge nje, ngequbuliso uphuma igazi empumlweni ngaphandle kwesizathu. Okanye nokuba nenxeba elincinci emnweni wakho okanye emva kotyando, ukopha akupheli ixesha elide. Ukuba usana oluncinci lunesi sifo, lusenokubeka ithoyizi emlonyeni walo lize liphume igazi.
Ukugruzuka
Ulusu lwethu luba luhlaza okwesibhakabhaka xa igazi lihamba ngaphantsi kwalo. Abantu abane-hemophilia banokujika babe luhlaza okwesibhakabhaka baze bagruzuke lula, nokuba batshaywe yinto encinci. Ukuba usana oluncinci lubetha intloko yakhe ndaweni ithile, lunokukhula amaqhuma amakhulu abizwa ngokuba "ngamaqanda eehansi."
Intlungu Yamalungu Nokudumba
Le yeyona ngxaki inzima ye-hemophilia. Ukopha kunokubakho ngaphakathi emzimbeni, ingakumbi kumalungu afana namadolo, iingqiniba, namaqatha. Amalungu ayadumba, abe buhlungu, aze afudumale xa echukunyiswa. Xa oku kusenzeka kwiintsana ezincinci, zinokukhala rhoqo, zingavumi ukuguqa, okanye ukuhamba.
Kubaluleke kakhulu: Kunqabile kakhulu ukuba i-hemophilia enzima inokubangela ukopha engqondweni. Oku kunokuba yingozi ebomini. Ukuba uneempawu ezifana nentloko ebuhlungu engapheliyo, ukubona kabini, okanye ukudinwa kakhulu , ezi zinokuba ziimpawu zokopha ebuchotsheni. Ukuba une-hemophilia kwaye ufumana ezi mpawu, yiya kwigumbi likaxakeka lesibhedlele (ETU) ngokukhawuleza.
Yintoni ebangela i-hemophilia?
I-Hemophilia idla ngokuba sisifo semfuza esidluliselwa kwizizukulwana ngezizukulwana.Oko kuthetha ukuba kubangelwa lutshintsho kwiijini esizizuza njengelifa kumama okanye kutata wethu. Masiyiqonde le nto ngokulula.
Imizimba yethu iyalelwe ukuba yenze izinto ezibangela ukujiya kwegazi ngee-genes. Xa kukho utshintsho okanye isiphene kwezi genes, loo miyalelo ayisebenzi kakuhle. Isiphumo kukuba umzimba awuvelisi ubungakanani bezinto ezibangela ukujiya kwegazi ozifunayo.
Esi siphene semfuza sifumaneka kwi-chromosome X.
- Intombazana inee-chromosomes ezimbini ze-X (XX), enye ivela kunina kwaye enye ivela kuyise.
- Inkwenkwe ine-chromosome enye ye-X kunye ne-chromosome enye ye-Y (XY). I-X ivela kumama kwaye i-Y ivela kutata.
Ngoku jonga indlela oku kuqhubeka ngayo kwizizukulwana ngezizukulwana:
- Ukuba umama une-chromosome X enale gene inesiphene (siyibiza ngokuba 'ngumthwali'), uhlala engabonakalisi zimpawu kuba une-chromosome X ephilileyo.
- Ukuba unyana walo mama uzuze i-chromosome X enesiphene, uya kuba ne-hemophilia kuba akanayo enye i-chromosome X ephilileyo yokuyilawula.
- Ukuba intombi yalo mama izuza ilifa le-chromosome X enesiphene, nayo iya kuba yithwali. Isenokubonisa iimpawu ezibuthathaka, ezifana nokopha kakhulu exesheni.
Ngamanye amaxesha, i-hemophilia inokuvela ngokuzenzekelayo, ngaphandle kokufunyanwa njengelifa, ngenxa yotshintsho lwe-gene oluzenzekelayo ngexesha lobomi bomntwana ongekazalwa. Kwakhona, kunqabile kakhulu, ukuba kwenzeke xa inkqubo yomzimba yomntu ihlasela izinto ezibangela ukujiya kwegazi. Oku sikubiza ngokuba yi-'acquired hemophilia'.
Ugqirha uyixilonga njani le nto?
Ukuba wena okanye umntwana wakho ninezi mpawu, ugqirha wakho uza kuqala akuhlole aze akubuze ukuba kukho umntu kusapho lwakho okhe waba nezi ngxaki. Emva koko, baya kuyalela uvavanyo lwegazi ukuqinisekisa oku.
- Ubalo lweGazi olupheleleyo (CBC): Lulinganisa iintlobo kunye nobungakanani beeseli egazini lakho.
- Uvavanyo lwexesha le-Prothrombin (PT): Lujonga ukuba kuthatha ixesha elingakanani ukuba igazi lakho lijiye.
- Uvavanyo lwexesha le-Thromboplastin elingaphelelanga (PTT): Olu lolunye uvavanyo olulinganisa ixesha lokujiya kwegazi.
- Uvavanyo oluthile lweClotting Factor: Yile nto imisela ukuba yeyiphi i-clotting factor ongenayo kwaye iphantsi kangakanani.
Zithini iindlela zonyango zoku?
Unyango olufumanayo luya kuxhomekeka kuhlobo lwe-hemophilia onayo kunye nobunzima bemeko yakho. Unyango oluphambili lunyango lokubuyisela.
Ngamafutshane, oku kuquka ukunika umzimba wakho inaliti ye-clotting factor ephantsi egazini lakho. Ezi -clotting factors zinokwenziwa nge-plasma yegazi lomntu okanye zenziwe kwilebhu. Olu nyango lunokunceda ekuthinteleni ukopha kwaye lumise ngokukhawuleza ukuba kuyenzeka.
Ukongeza, ngoku kukho iindlela ezintsha zonyango:
- Amayeza afana neMarstacimab-hncq (Hympavzi®) kunye neConcizumab (Alhemo®) : La mayeza athintela iproteni ethintela ukujiya kwegazi kwaye avuselele i-enzyme enceda ukujiya kwegazi.
- I-Fitusiran (Qfitlia®): Eli yeza lithintela isibindi sethu ekwenzeni into (i-antithrombin) ethintela igazi ukuba lingaqini.
- Unyango lweZiza: Le yindlela yonyango ephucukileyo. Kule ndlela, kufakwa i-gene esempilweni emzimbeni ukuze ithathe indawo ye-gene enesiphene, nto leyo evumela umzimba ukuba uvelise izinto ezifunekayo zokujiya kwamalungu omzimba.
Ukuba une-hemophilia, kufuneka uphephe ukusebenzisa amayeza okudambisa iintlungu afana ne-aspirin kunye ne-ibuprofen, kunye ne-blood thinners ezifana ne-heparin okanye i-warfarin. Qiniseka ukuba uthetha nogqirha wakho ngale nto.
Ufanele ufune nini ingcebiso yezonyango ngokukhawuleza?
Ukuba ubona naluphi na utshintsho emzimbeni wakho, njengokwanda kokopha okanye ukukrweleka, xelela ugqirha wakho ngoko nangoko. Kwakhona, ukuba ufumana naziphi na iimpawu ezinzulu ezilandelayo, yiya kwiSebe lezeMpilo eziNgxamisekileyo (ETU) lesibhedlele ngaphandle kokulibazisa.
| Iimeko ezingxamisekileyo | |
|---|---|
| Intloko ebuhlungu kakhulu, umbono ongaboni kakuhle, okanye ukudinwa kakhulu | Oku kungaba luphawu lokopha engqondweni. Yiya kwi-ETU ngoko nangoko. |
| Ukudumba okukhulu kunye nentlungu enganyamezelekiyo kwilungu | Oku kukopha kakhulu kwilungu. Yiya kwi-ETU ngoko nangoko. |
Izinto ekufuneka uzazi xa uphila ne-hemophilia
I-Hemophilia sisifo esihlala ubomi bonke, okanye esingapheliyo. Ngoko ke uya kufuna unyango kunye nokubekwa esweni ubomi bakho bonke. Kodwa ungakhathazeki. Ngonyango olutsha, umntu one-hemophilia unokuphila ubomi obufana nobomntu ongenaso esi sifo.
Nazi ezinye izinto ezinokukunceda uzive ungcono ngelixa uphila nesi sifo:
- Lawula ubunzima bakho: Njengoko usiya utyeba, uxinzelelo kumalungu akho luyanda. Ukugcina ubunzima obusempilweni kubalulekile ukukhusela amalungu asele onakele ngenxa yokopha ngaphakathi kwamalungu.
- Lumka kakuhle amazinyo akho: Kubalulekile ukuxubha amazinyo akho kakuhle kwaye ukhathalele impilo yamazinyo akho. Ngenxa yokuba iinkqubo ezifana nokukhupha amazinyo kunye neengcambu zemijelo zinokubangela ukopha, kungcono ukuziphepha.
- Cinga ngempilo yengqondo: Ukuphila nesifo esinganyangekiyo kunokubangela ixhala kunye nokudakumba. Thetha nogqirha wakho ngale nto kwaye ufumane iingcebiso ngempilo yengqondo ukuba kuyimfuneko.
- Yiba nomdla ngokukhuselekileyo: Ukuzibandakanya kwimisebenzi enciphisa umngcipheko wokwenzakala kulungile emzimbeni nasengqondweni yakho. Buza ugqirha wakho ukuba zeziphi imithambo efanelekileyo kuwe.
Xa une-hemophilia, nokuba yingozi encinci abanye abangayiboniyo inokuba yingxaki enkulu kuwe. Ukudumba edolweni emlenzeni wetafile kunokubangela ukopha emalunga. Ukopha impumlo akunakunqandeka nokuba sele uthimle kakhulu. Ukuphila nale meko kunokuba nzima. Kodwa khumbula, awuwedwa. Iqela lakho lezonyango lihlala likhona ukukunceda.
Umyalezo Wokuya Ekhaya
- I-Hemophilia yimeko yelifa apho igazi lingajiyi kakuhle.
- Oku kungenxa yokuncipha kohlobo lweproteni olubizwa ngokuba yi-"clotting factors" egazini.
- Iimpawu eziphambili kukopha kakhulu ngenxa yokwenzakala okuncinci, umzimba ujika ube luhlaza okwesibhakabhaka, kunye nokudumba kwamalungu.
- Intloko ebuhlungu kakhulu okanye intlungu yamalungu enganyamezelekiyo yimeko engxamisekileyo efuna unyango olukhawulezileyo (ETU).
- Nangona kungekho ndlela ipheleleyo yokunyanga oku, unyango lwanamhlanje lunokulawula ukopha kwaye lukuvumele ukuba uphile ubomi obuqhelekileyo.
- Soloko uthetha nogqirha wakho ngemeko yakho kunye nonyango lwakho.
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