Uma uthola inxeba elincane noma ushayeka ndawana thize, ukuphuma kwegazi kuyaphela ngemva kwesikhashana, akunjalo? Lokho kungenxa yenqubo yemvelo yokujiya kwegazi emizimbeni yethu. Kodwa cabanga ngakho, abanye abantu abayeki ukuphuma kwegazi kalula. Ngisho noma benenxeba elincane, baphuma igazi isikhathi eside. Umzimba uphenduka ube luhlaza okwesibhakabhaka futhi ube nemihuzuko. Imbangela yalesi simo ingaba yisifo esibizwa ngokuthi i-hemophilia. Nakuba abantu abaningi bengazi kahle ngalokhu, lokhu kuyinto ebalulekile okufanele siyazi. Ake sixoxe ngalokhu kalula, ngendlela ongayiqonda.
Kuyini ngempela i-hemophilia?
Kalula nje, i-hemophilia yisimo esingavamile lapho igazi lethu lingavali kahle. Igazi lethu liqukethe amaprotheni akhethekile asiza ekumiseni ukopha lapho liqala. Sibiza lezi ngokuthi "izici zokuvalela." Njengoba nje kudingeka udaka ukuze kuhlanganiswe izitini lapho kwakhiwe udonga, lezi zici zokuvalela zibalulekile ekumiseni ukopha.
Umuntu one-hemophilia akakhiqizi ngokwanele enye noma ngaphezulu yalezi zinto ezibangela ukujiya kwegazi. Lokhu kusho ukuthi ngisho nokusikeka okuncane kungabangela ukopha isikhathi eside. Ngezinye izikhathi, ukopha kungenzeka ngaphakathi emzimbeni, ikakhulukazi emalungeni nasemisipheni. Lokhu kungaba yingozi empilweni.
Odokotela bahlukanisa ubucayi balesi sifo ngezigaba ezintathu, kuye ngokuthi zingakanani izinto ezibangela ukujiya kwegazi.
- I-hemophilia encane: Ukopha kuvame ukwenzeka ngemva kokulimala okukhulu noma ukuhlinzwa.
- I-hemophilia ephakathi nendawo: Ngisho nokulimala okuncane kungabangela ukopha ngokweqile. Umzimba ungase uphenduke ube luhlaza okwesibhakabhaka.
- I-hemophilia enzima: Ukopha kungenzeka ngaphakathi nangaphandle, ngisho nangaphandle kwesizathu esibonakalayo.
Nakuba kungekho ikhambi eliphelele lalokhu, ngemithi emisha etholakalayo, kungenzeka ukulawula ukopha nokuphila impilo evamile.
Ingabe zikhona izinhlobo eziyinhloko ze-hemophilia?
Yebo, kunezinhlobo ezintathu eziyinhloko zalesi sifo. Zisekelwe ohlotsheni lwesici sokujiya kwegazi esingekho emzimbeni. Ukuze uqonde lokhu ngokucacile, bheka ithebula elingezansi.
| Uhlobo lwe-hemophilia | Incazelo |
|---|---|
| I-Hemophilia A | Lolu uhlobo oluvame kakhulu. Luvela lapho kungekho khona i-blood clotting factor 8 eyanele, noma i-Factor VIII . |
| I-Hemophilia B | Lolu hlobo lwenzeka uma kungekho i-blood clotting factor 9 eyanele, noma i-Factor IX . |
| I-Hemophilia C | Lolu uhlobo olungavamile kakhulu. Lubangelwa ukuntuleka kwe-blood clotting factor 11, noma i-Factor XI . |
Ziyini izimpawu zalesi sifo?
Izimpawu eziyinhloko ze-hemophilia ziwukuphuma kwegazi okungavamile noma ukuphuka ngokweqile kanye nokulunywa. Ubukhulu balezi zimpawu buxhomeke ekutheni une-hemophilia emnene, ephakathi nendawo, noma enzima.
Ukopha Okukhulu
Cabanga nje, ngokuzumayo uphuma igazi ekhaleni ngaphandle kwesizathu. Noma ngisho nokusikeka okuncane emunweni wakho noma ngemva kokuhlinzwa, ukuphuma kwegazi akupheli isikhathi eside. Uma ingane encane inalesi simo, ingase ifake ithoyizi emlonyeni wayo bese yopha igazi.
Ukuphuka
Isikhumba sethu siphenduka sibe luhlaza okwesibhakabhaka uma igazi ligeleza ngaphansi kwaso. Abantu abane-hemophilia bangashintsha babe luhlaza okwesibhakabhaka futhi bavuvuke kalula, noma ngabe bashaywe yinto encane. Uma ingane encane ishaya ikhanda layo ndawana thize, ingaba nezigaxa ezinkulu ezibizwa ngokuthi “amaqanda e-goose.”
Ubuhlungu Nokuvuvukala Kwamalunga
Lena inkinga enkulu kakhulu ye-hemophilia. Ukopha kungenzeka ngaphakathi emzimbeni, ikakhulukazi emalungeni afana namadolo, izindololwane, nasemaqakaleni. Amalungeni abe esevuvukala, abe buhlungu, futhi afudumale uma ethintwa. Uma lokhu kwenzeka ezinganeni ezincane, zingase zikhale njalo, zenqabe ukuguqa, noma ukuhamba.
Kubaluleke kakhulu: Akuvamile ukuthi i-hemophilia enzima ingabangela ukopha ebuchosheni. Lokhu kungaba yingozi empilweni. Uma unezimpawu ezifana nekhanda elibuhlungu eliqhubekayo, ukubona kabili, noma ukukhathala okukhulu , lezi kungaba izimpawu zokopha ebuchosheni. Uma une-hemophilia futhi uhlangabezana nalezi zimpawu, iya ekamelweni lezimo eziphuthumayo esibhedlela (ETU) ngokushesha.
Yini ebangela i-hemophilia?
I-Hemophilia ivame ukuba yisifo esibangelwa izakhi zofuzo esidluliselwa ezizukulwaneni ngezizukulwane.Lokho kusho ukuthi kubangelwa ushintsho ezizakhini zofuzo esizizuza kumama noma kubaba. Ake sikuqonde lokhu kalula.
Imizimba yethu iyalwa ukuba yenze izakhi zokujiya ngamajini. Uma kukhona ushintsho noma iphutha kula majini, leyo miyalelo ayisebenzi kahle. Umphumela uba ukuthi umzimba awukhiqizi inani lezizakhi zokujiya olidingayo.
Lesi sici sofuzo sitholakala ku-chromosome X.
- Intombazane inama-chromosome amabili e-X (XX), elilodwa elivela kunina kanye nelinye elivela kuyise.
- Umfana une-chromosome eyodwa engu-X kanye ne-chromosome eyodwa engu-Y (XY). I-X ivela kumama kanti i-Y ivela kuyise.
Manje bheka ukuthi lokhu kuhamba kanjani ezizukulwaneni ngezizukulwane:
- Uma umama ephethe i-chromosome X enalesi sakhi sofuzo esingasebenzi kahle (sisibiza ngokuthi 'isithwali'), ngokuvamile akabonisi zimpawu ngoba unenye i-chromosome X enempilo.
- Uma indodana yalo mama izuza i-chromosome X enephutha, izoba ne-hemophilia ngoba ayinayo enye i-chromosome X enempilo yokuyilawula.
- Uma indodakazi yalo mama izuza i-chromosome X enephutha, nayo izoba yimthwali. Ingase ibonise izimpawu ezincane, njengokuphuma kwegazi okukhulu kokuya esikhathini.
Ngezinye izikhathi, i-hemophilia ingakhula ngokuzenzakalelayo, ngaphandle kokutholwa njengefa, ngenxa yokushintsha kwezakhi zofuzo okuzenzakalelayo ngesikhathi sokuphila kombungu. Futhi, akuvamile kakhulu, kungenzeka lapho amasosha omzimba womuntu ehlasela izakhi zawo zokujiya. Lokhu sikubiza ngokuthi 'i-hemophilia etholakale'.
Udokotela uhlonza kanjani lokhu?
Uma wena noma ingane yakho ninalezi zimpawu, udokotela wakho uzoqala akuhlole bese ebuza ukuthi kukhona yini emndenini wakho oke waba nalezi zimo. Bese, bazoyala ukuhlolwa kwegazi ukuqinisekisa lokhu.
- Inani Eliphelele Legazi (CBC): Lilinganisa izinhlobo kanye nenani lamaseli egazini lakho.
- Ukuhlolwa Kwesikhathi Se-Prothrombin (PT): Kuhlola ukuthi kuthatha isikhathi esingakanani ukuthi igazi lakho lijiye.
- Ukuhlolwa Kwesikhathi Se-Thromboplastin Engaphelele (i-PTT): Lokhu okunye ukuhlolwa okulinganisa isikhathi sokujiya kwegazi.
- Ukuhlolwa Okuqondile Kwesici Sokugoqa: Yilokhu okunquma ngqo ukuthi iyiphi isici sokugoqa esintulayo nokuthi siphansi kangakanani.
Yiziphi izindlela zokwelapha lokhu?
Ukwelashwa okutholayo kuzoncika ohlotsheni lwe-hemophilia onayo kanye nobunzima besimo sakho. Ukwelashwa okuyinhloko ukwelashwa kokufaka esikhundleni.
Kalula nje, lokhu kuhilela ukunikeza umzimba wakho umjovo we-clotting factor ephansi egazini lakho. Lezi zi-clotting factor zingenziwa nge-plasma yegazi lomuntu noma zenziwe elabhorethri. Lokhu kwelashwa kungasiza ekuvimbeleni ukopha futhi kumise ngokushesha uma kwenzeka.
Ngaphezu kwalokho, manje kunezindlela ezintsha eziningana zokwelapha:
- Imithi efana ne -Marstacimab-hncq (Hympavzi®) kanye ne-Concizumab (Alhemo®) : Le mithi ivimba iphrotheni evimbela ukujiya kwegazi futhi ivuselele i-enzyme esiza ukujiya kwegazi.
- I-Fitusiran (Qfitlia®): Lo muthi uvimba isibindi sethu ekwenzeni into (i-antithrombin) evimbela igazi ukuthi lingaqini.
- Ukwelashwa Ngezakhi Zofuzo: Lena yindlela yokwelapha esezingeni eliphezulu kakhulu. Kulokhu, kufakwa isakhi sofuzo esinempilo emzimbeni ukuze sithathe indawo yesakhi sofuzo esinephutha, okuvumela umzimba ukuthi ukhiqize izinto ezidingekayo zokujiya kwamangqamuzana.
Uma une-hemophilia, kufanele ugweme ukuthatha imithi yokunciphisa ubuhlungu njenge-aspirin ne-ibuprofen, kanye nemithi yokunciphisa igazi njenge-heparin noma i-warfarin. Qiniseka ukuthi ukhuluma nodokotela wakho ngalokhu.
Kunini lapho kufanele ufune khona iseluleko sezokwelapha ngokushesha?
Uma ubona noma yiziphi izinguquko emzimbeni wakho, njengokwanda kwegazi noma ukuklwebheka, tshela udokotela wakho ngokushesha. Futhi, uma uhlangabezana nanoma yiziphi izimpawu ezilandelayo ezimbi, iya eMnyangweni Wezimo Eziphuthumayo (ETU) wesibhedlela ngokushesha.
| Izimo Eziphuthumayo | |
|---|---|
| Ubuhlungu obukhulu bekhanda, umbono ofiphele, noma ukukhathala okukhulu | Lokhu kungaba uphawu lokopha ebuchosheni. Yiya ku-ETU ngokushesha. |
| Ukuvuvukala okukhulu kanye nobuhlungu obungabekezeleleki emalungeni | Lokhu kuphuma kwegazi okukhulu emalungeni. Yiya ku-ETU ngokushesha. |
Izinto okufanele uzazi uma uphila ne-hemophilia
I-Hemophilia iyisifo esihlala isikhathi eside, noma esingamahlalakhona. Ngakho-ke uzodinga ukunakekelwa kwezokwelapha kanye nokuqapha impilo yakho yonke. Kodwa ungakhathazeki. Ngezindlela ezintsha zokwelapha, umuntu one-hemophilia angaphila impilo eseduze neyomuntu ongenaso lesi sifo.
Nazi ezinye izinto ezingakusiza uzizwe ungcono ngenkathi uphila nalesi sifo:
- Lawula isisindo sakho: Njengoba ukhuluphala, ingcindezi emalungeni akho iyanda. Ukugcina isisindo esinempilo kubalulekile ukuvikela amalunga awonakele kakade ngenxa yokopha ngaphakathi emalungeni.
- Nakekela amazinyo akho kahle: Kubalulekile ukuxubha amazinyo akho kahle futhi unakekele impilo yamazinyo akho. Ngenxa yokuthi izinqubo ezifana nokukhipha amazinyo kanye nezimpande zemisele zingabangela ukopha, kungcono ukuzigwema.
- Cabanga ngempilo yengqondo: Ukuphila nesifo esingamahlalakhona kungaholela ekukhathazekeni nasekucindezelekeni. Khuluma nodokotela wakho ngalokhu bese ucela ukwelulekwa ngempilo yengqondo uma kudingeka.
- Yiba matasa ngokuphephile: Ukuzibandakanya emisebenzini enciphisa ingozi yokulimala kuhle emzimbeni nasengqondweni yakho. Buza udokotela wakho ukuthi yikuphi ukuzivocavoca okufanele wena.
Uma une-hemophilia, ngisho nokulimala okuncane abanye abangase bangakuqapheli kungaba yinkinga enkulu kuwe. Ukugoba edolweni emlenzeni wetafula kungabangela ukopha emalungeni. Ukopha ekhaleni kungaba yinto enganqandeki ngisho nangemva kokuthimula kanzima. Ukuphila nalesi simo kungaba yinselele. Kodwa khumbula, awuwedwa. Ithimba lakho lezokwelapha lihlala likhona ukukusiza.
Umlayezo Wokuya Nawe Ekhaya
- I-Hemophilia yisimo esizuzwa njengefa lapho igazi lingajiyi kahle khona.
- Lokhu kungenxa yokwehla kohlobo lweprotheni olubizwa ngokuthi "izici zokugoba" egazini.
- Izimpawu eziyinhloko ukopha ngokweqile ngenxa yokulimala okuncane, umzimba uphenduka ube luhlaza okwesibhakabhaka, kanye nokuvuvukala kwamalunga.
- Ikhanda elibuhlungu noma ubuhlungu bamalunga obungabekezeleleki kuyisimo esiphuthumayo esidinga usizo lwezokwelapha ngokushesha (ETU).
- Nakuba kungekho ikhambi eliphelele lalokhu, izindlela zokwelapha zesimanje zingalawula ukopha futhi zikuvumele ukuthi uphile impilo evamile.
- Khuluma nodokotela wakho njalo ngesimo sakho kanye nokwelashwa kwakho.
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