Uma unenxeba elincane emzimbeni wakho, noma uma ukhishwe izinyo, ingabe ukopha kuyaqhubeka ngaphandle kokuma? Noma ngezinye izikhathi uba nemivimbo eluhlaza okwesibhakabhaka emzimbeni wakho ngaphandle kokushaywa ndawo? Ubona imivimbo emzimbeni wakho wonke, ikakhulukazi lapho ingane yakho iqala ukukhasa noma ukuhamba? Uma izinto ezinjengalezi zenzeka, imbangela ingaba yisimo esibizwa ngokuthi i-hemophilia . Ungesabi uma uzwa leli gama. Ngokwelashwa okufanele , ungaphila impilo evamile, egcwele. Ake sixoxe ngalokhu ngokucacile nangokulula namuhla.
Kalula nje, iyini i-Hemophilia A?
Kalula nje, i-Hemophilia A yisimo lapho igazi lethu lingavali kahle. Ngokuvamile, lapho sithola ukulimala, igazi lethu liyavali ukuze kumiswe ukuphuma kwegazi. Lokhu kusizwa amaprotheni akhethekile egazini lethu. Lokhu sikubiza ngokuthi "izici zokuvalisisa."
Umuntu one-hemophilia A akakhiqizi ngokwanele i-clotting factor Factor VIII . Ubunzima besifo bunqunywa izinga le phrotheni ye-Factor VIII encishiswa ngalo. Ngakho-ke, ihlukaniswe izingxenye ezintathu:
- I-hemophilia encane: Amazinga e-Factor VIII akhona ngezinga elithile.
- I-hemophilia ephakathi nendawo: Amazinga e-Factor VIII aphansi kakhulu.
- I-hemophilia enzima: Amazinga e-Factor VIII aphansi kakhulu noma awekho.
Esikhathini esiningi, lesi yisifo sofuzo esitholakala emindenini. Kodwa-ke, cishe engxenyeni yesithathu yamacala, umuntu omusha angahlakulela lesi sifo ngaphandle kokuba noma ubani emndenini abe naso.
Yini ebangela i-hemophilia?
Lokhu kuyinto esiyizuza njengefa ngama-gene. Cabanga ngakho, zonke izici zomzimba wethu zinqunywa ama-gene. Owesifazane unama -chromosome amabili angu-X (XX), kanti owesilisa une-chromosome eyodwa engu-X kanye ne-chromosome eyodwa engu-Y (XY).
Iphutha lezakhi zofuzo elihlobene ne-hemophilia litholakala ku-chromosome X.
Ngakho-ke, uma umama "engumthwali" walesi sakhi sofuzo esingasebenzi kahle, ngokuvamile akabonisi zimpawu. Ngoba enye i-chromosome yakhe ye-X enempilo yenza iphrotheni ye-factor VIII edingekayo. Kodwa uma indodana yakhe izuza le chromosome ye-X engasebenzi kahle, leyo ndodana izoba ne-hemophilia. Ngenxa yokuthi umfana une-chromosome eyodwa ye-X kuphela, lowo mfana uzoba ne-hemophilia. Ngenxa yalesi sizathu, i-hemophilia ivame kakhulu kubafana kunamantombazane.
Akuvamile ukuthi abantu abadala abaneminyaka ephakathi kuka-60 no-80 bangaba ne-hemophilia. Kwenzeka lapho amasosha omzimba ehlasela izinto ezisiza ukujiya kwegazi ezinempilo. Kungenzeka futhi ngesikhathi sokukhulelwa, ngomdlavuza , kanye nezinye izimo zokuzivikela komzimba .
Ziyini izimpawu ze-hemophilia?
Izimpawu zincike ekutheni isimo sakho sincane, simaphakathi, noma sibi kakhulu.
| Izinga lesifo | Izici ezibonakalayo |
|---|---|
| I-hemophilia encane | Ngokuvamile, ukopha okukhulu kwenzeka kuphela ngemva kokuhlinzwa, ukukhishwa kwezinyo, ukubeletha, noma ingozi enkulu. Abanye abantu abazi nokuthi banayo kuze kube yilapho sebekhulile. |
| I-hemophilia ephakathi nendawo | - Uma ulimele, kuba nokopha ngokweqile. - Ngezinye izikhathi ukuphuma kwegazi kwenzeka ngaphandle kwesizathu. - Umzimba uyaphuka kalula futhi uphenduke ube luhlaza okwesibhakabhaka. - Uma uthola umjovo, kuthatha isikhathi eside ukopha. |
| I-hemophilia enzima | Ukopha kuvame ukwenzeka ngisho noma kungekho ukulimala. Ukopha kungavela ikakhulukazi emalungeni nasemisipheni . Lokhu kubuhlungu kakhulu. |
Izimpawu eziyisixwayiso zokuphuma kwegazi ebuchosheni
Uma umuntu one-hemophilia enzima ethola ngisho nokushaywa okuncane ekhanda, ukuphuma kwegazi ebuchosheni kungenzeka. Lesi yisimo esiphuthumayo esibucayi. Uma ulimele ekhanda nanoma yiziphi izimpawu ezilandelayo, shayela udokotela wakho ngokushesha noma uye emnyangweni wezimo eziphuthumayo oseduze (i-ETU).
- Ikhanda elibuhlungu elingapheli
- Ukuhlanza
- Ukozela njalo noma ukukhathala ngokweqile
- Ubuthakathaka obungazelelwe noma ubunzima bokuhamba
- Umbono ophindwe kabili
- Ukuquleka
Ungasithola kanjani lesi sifo?
Uma othile emndenini wakho ene-hemophilia, ungahlola ingane yakho ukuthi ayinaso yini lesi sifo ngesikhathi ukhulelwe. Kodwa-ke, kunezingozi ezithile ezihilelekile kulezi zivivinyo, ngakho-ke kufanele uxoxe ngazo nodokotela wakho.
Amacala amakhulu e-hemophilia ezinganeni ezincane avame ukutholakala phakathi nonyaka wokuqala wokuphila. Uma ingane yakho iqala ukugoba futhi iqala ukukhasa futhi iqala ukubona imivimbo ephakanyisiwe emzimbeni wayo, khuluma nodokotela wakho ngakho.
Udokotela angase akubuze imibuzo efana nale:
- Kwenzeka kanjani lokhu kuphuka nokopha kwegazi?
- Sekuyisikhathi esingakanani ukuphuma kwegazi kuqhubeka?
- Ingabe ikhona imithi okufanele ithathwe yizingane?
- Ingabe ukhona emndenini wakho onezinkinga zokujiya kwegazi?
Ngemuva kwalokho, kuzokwenziwa ukuhlolwa kwegazi okuningana ukuqinisekisa ukuxilongwa. Ungathunyelwa futhi kudokotela wegazi.
| Igama Lokuhlolwa | Ubonani kulokhu? |
|---|---|
| Inani Eliphelele Legazi (i-CBC) | Izinhlobo zamaseli egazini kanye nezinga le-hemoglobin kuyahlolwa. Lokhu kungasiza ekunqumeni ukuthi kukhona yini i-anemia ngenxa yokopha. |
| Ukuhlolwa kwe-PT kanye ne-aPTT | Ilinganisa ukuthi kuthatha isikhathi esingakanani ukuthi igazi lijiye. Inani le-aPTT livame ukuphakama ku-hemophilia. |
| Ukuhlolwa kwe-Factor VIII kanye ne-Factor IX | Zilinganisa kahle ukuthi ingakanani le phrotheni esegazini. Uma i-factor VIII iphansi, yi-hemophilia A. |
| Ukuhlolwa Kwezakhi Zofuzo | Ukuguquka kwezakhi zofuzo okubangela lesi sifo kungabonakala. Uma kwenzeka kowesifazane, kungatholakala nokuthi ungumthwali walesi sifo. |
Iphathwa kanjani?
Indlela yokwelapha incike ebunzimeni besifo, iminyaka yakho, kanye nezidingo zakho zomuntu siqu. Umgomo oyinhloko wokwelashwa ukufaka esikhundleni seprotheni ye-factor VIII engekho emzimbeni. Lokhu kubizwa ngokuthi ukwelashwa kokufaka esikhundleni se-factor . Nakuba lokhu kungelaphi ngokuphelele lesi sifo, kungasiza ekulawuleni ukopha futhi kukuvumele ukuthi uphile impilo evamile.
Kunezinhlobo ezimbili zokwelapha:
- Ukwelashwa Kokuvimbela: Ukujova njalo kwe-factor VIII ohlelweni oluhleliwe ukuze kuvinjelwe ukuphuma kwegazi, kunokulinda ukuthi kuvele ukopha . Lokhu kubaluleke kakhulu kubantu abane-hemophilia enzima.
- Ukwelashwa Okudingeka Kakhulu: Ukuthola ukwelashwa kuphela uma kuvela ukopha .
Kunezinhlobo ezimbili zemithi yokugomela i-factor VIII:
1. I-Recombinant Factor VIII: I-Factor ekhiqizwa elabhorethri kusetshenziswa ubunjiniyela bezakhi zofuzo. Lena isetshenziswa kakhulu namuhla.
2. I-Factor VIII etholakala ku-Plasma: I-Factor etholakala ku-plasma yegazi lomuntu.
Abantu abane-hemophilia A emnene kuya kwephakathi bangasebenzisa nomuthi obizwa ngokuthi i-Desmopressin (DDAVP) . Lokhu kusebenza ngokukhipha i-factor VIII egciniwe egazini.
Ngaphezu kwalokho, imithi yomlomo efana neTranexamic Acid, evimbela ukuqhekeka kwegazi, isetshenziswa nasezimweni ezifana nokukhishwa kwamazinyo.
Okubalulekile: Njengoba umuntu one-hemophilia engase adinge ukumpontshelwa igazi njalo, kubaluleke kakhulu ukugonyelwa i-Hepatitis A kanye ne-B. Buza udokotela wakho ngalokhu.
Uphatha kanjani impilo yansuku zonke?
Uma uphila ne-hemophilia, kufanele ucabange kabanzi ngokuphepha.
- Yiba matasa: Ukuzivocavoca kuqinisa imisipha. Imisipha eqinile inikeza isivikelo esihle samalunga. Kodwa-ke, gwema imidlalo yokuthintana njenge-rugby kanye ne-boxing. Ukubhukuda, ukuhamba, kanye nokugibela ibhayisikili (unesigqoko) kuyizinketho ezinhle. Khuluma nodokotela wakho mayelana nokuthi yimiphi imisebenzi ekufanele.
- Vikela izingane: Uma unengane encane, gqoka ama-knee pad, ama-elbow pad, kanye nesigqoko sokuzivikela uma udlala. Zivikele efenisha ebukhali ekhaya lakho.
- Impilo yamazinyo:Gcina inhlanzeko enhle yamazinyo. Hlanza amazinyo akho nsuku zonke. Lokhu kungasiza ekunciphiseni isidingo sokukhishwa kwamazinyo kanye nokwelashwa okukhulu kwamazinyo. Ngaphambi kokuba uye kudokotela wamazinyo, qiniseka ukuthi umtshela ukuthi une-hemophilia.
- Imithi okungafanele isetshenziswe: Iziqeda-buhlungu ezifana ne-aspirin kanye ne-NSAID (isib. i-ibuprofen, i-diclofenac) zingavimbela kakhulu ukujiya kwegazi. Ungathathi noma yimuphi umuthi ngaphandle kokubonisana nodokotela wakho.
Umlayezo Wokuya Nawe Ekhaya
- I-Hemophilia A yisifo sofuzo esibangelwa ukuntuleka kweprotheni i-Factor VIII, edingekayo ukuze igazi lijiye.
- Lokhu kuvame ukubonakala kubafana, kodwa abesifazane bangaba ngabathwali balesi sifo.
- Izimpawu eziyinhloko ukopha ngokweqile ngenxa yokulimala okuncane kanye nokulunywa okungadingekile.
- Uma ulimele ekhanda futhi unezimpawu ezifana nekhanda elibuhlungu, ukuhlanza, noma ukozela, kuyisimo esiphuthumayo. Funa iseluleko sezokwelapha ngokushesha.
- Ngokwelashwa okufanele (Factor Replacement Therapy) kanye nendlela yokuphila ephephile, abantu abane-hemophilia bangaphila impilo egcwele futhi esebenzayo.
- Xoxa njalo ngayo yonke imithi oyithathayo nodokotela wakho ngaphambi kokuhlinzwa noma ukwelashwa kwamazinyo.
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