Wake waba nokusikeka okuncane okwathatha isikhathi eside kunokulindelekile ukuze kuphume igazi? Noma wake waba nokuphuma kwegazi ekhaleni noma imihuzuko emzimbeni wakho wonke? Ungase ucabange, "O... yilokho kanye engiyikho." Kodwa kungase kube nesizathu sezokwelapha ngemuva kwalezi zinto ongasazi. Namuhla sizokhuluma ngesimo esithinta inqubo yokujiya kwegazi, abantu abaningi abangakaze bezwe ngaso, kodwa esingavamile kangako. Leso yisifo sika-von Willebrand.
Kalula nje, siyini isifo sika-von Willebrand?
Isifo sika-von Willebrand siyisifo segazi esibangelwa izakhi zofuzo esithinta ikhono legazi lethu lokujiya kahle.
Cabanga ukuthi uma sithola inxeba emzimbeni wethu, kunethimba elincane labasebenzi elivimba ukopha. Laba basebenzi basebenza ndawonye futhi basiza ukuvala inxeba, njengeplasta. Lowo "mphathi" uyiprotheni ebizwa ngokuthi i-von Willebrand factor (VWF) . Le phrotheni ye-VWF yiyona esiza ama-platelet egazini ukuba anamathele ndawonye futhi akhe i-blood clot evala inxeba.
Manje, kumuntu onesifo se-von Willebrand, umzimba awukhiqizi ngokwanele le phrotheni ye-VWF. Noma iphrotheni ekhiqizwayo ayisebenzi kahle. Ngakho-ke ngenxa yokuthi lowo "mphathi" akekho, kuthatha isikhathi eside ukuthi ama-platelet anamathele ndawonye futhi akhe ihlule legazi. Yingakho ngisho nesilonda esincane siqhubeka nokopha.
Uma lesi simo siba sibi kakhulu, ukuphuma kwegazi kungavela ngaphakathi kwamalunga noma ezicutshini ezithambile, okubangela ubuhlungu obukhulu nokuvuvukala. Kwabanye abantu, lokhu kungaholela nasekuntuleni igazi , isimo lapho kunokwehla khona kwenani legazi emzimbeni.
Lesi akusona isifo esingelapheki ngokuphelele. Kodwa ungakhathazeki! Udokotela wakho, ikakhulukazi udokotela wegazi, angakusiza ukuthi uphathe lesi simo kahle futhi uphile impilo ejwayelekile.
Ingabe zikhona izinhlobo eziyinhloko zalesi sifo?
Yebo, isifo sika-von Willebrand asifani kuwo wonke umuntu. Kunezinhlobo ezintathu eziyinhloko zaso. Ubukhali bezimpawu buyahlukahluka kuye ngohlobo lwaso.
| Uhlobo lwesifo | Incazelo |
|---|---|
| Uhlobo 1 | Lolu uhlobo oluvame kakhulu . Cishe abantu abathathu kwabane abanalesi sifo banalolu hlobo. Kulesi simo, amazinga e-VWF aphansi, kodwa izimpawu zincane kakhulu. Ngezinye izikhathi kungase kungabi nazimpawu nhlobo. |
| Uhlobo 2 | Kulolu hlobo, umzimba ukhiqiza iphrotheni ye-VWF, kodwa ayisebenzi kahle. Okusho ukuthi, yize inomphathi, ayazi ukuthi ingayenza kanjani umsebenzi wayo. Lokhu kungabangela ukopha okuncane kuya kokuphakathi. |
| Uhlobo 3 | Lolu uhlobo olungavamile nolunzima kakhulu . Kulesi simo, izinga le-VWF protein emzimbeni liphansi kakhulu, ngezinye izikhathi alikho nhlobo. Lokhu kukubeka engcupheni yokopha okukhulu. |
Ziyini izimpawu zesifo sikaVon Willebrand?
Kwabaningi, ikakhulukazi labo abanesifo sohlobo 1, izimpawu azizimbi kangako. Bangase baphile bengazi nokuthi banaso lesi sifo. Kodwa-ke, ezimweni ezimbi kakhulu, izimpawu ezilandelayo zingase zivele:
- Ukopha impumulo njalo: Uma ikhala lakho liphuma igazi ngisho noma kuyinto encane futhi kunzima ukuyeka.
- Ukopha isikhathi eside okuvela enxebeni elincane: Uma ukusikeka noma ukuklwebheka kopha imizuzu engaphezu kweyi-10.
- Ukuphuka kalula: Uma uthola imihuzuko emikhulu, evuvukele ngisho nangemva kokuqhuma okuncane.
- Ukopha kakhulu kokuya esikhathini kwabesifazane: Uma wopha kakhulu kunokujwayelekile ngesikhathi usesikhathini. Isibonelo, uma kufanele ushintshe ama-pad akho ngaphambi kokuphela kwehora noma amabili, uma uphuma amahlule amakhulu egazi, noma uma wopha izinsuku ezingaphezu kweziyi-7, kufanele ukhathazeke.
- Ukopha ngokweqile ngemva kokuhlinzwa: Uma wopha kakhulu kunokujwayelekile, ngisho nangemva kokuthile okulula njengokukhipha izinyo.
- Ukopha okukhulu ngemva kokubeletha noma ukuphuphuma kwesisu.
- Igazi endle noma emchameni.
Kungani lesi sifo sivela? Sizuzwa kanjani njengefa?
Isifo sika-von Willebrand siyisifo sofuzo . Kalula nje, sibangelwa yiphutha esakhiweni esilawula ukukhiqizwa kweprotheni ye-VWF. Sizuza lesi sakhi sofuzo esingalungile kubazali bethu.
Kunezindlela ezimbili zokuthola lokhu njengefa:
1. Ifa elibusayo le-Autosomal: Lokhu kwenzeka kumama noma kuyise.Ngisho noma umuntu oyedwa kuphela ozuza ifa lofuzo olunephutha, lesi sifo singakhula. Uhlobo 1 nohlobo 2 luvame ukuzuza ngale ndlela.
2. Ifa le-Autosomal recessive: Kulesi simo, ukuze bahlakulele lesi sifo, bobabili abazali kumele bazuze ifa lesakhi sofuzo esinephutha. Uhlobo 3, uhlobo olubi kakhulu, luvame ukuzuza ngale ndlela.
Akuvamile ukuthi eminye imidlavuza, izifo ezibangelwa amasosha omzimba, kanye nesifo senhliziyo nakho kungadala lesi simo kamuva ekuphileni. Sibizwa ngokuthi i-acquired von Willebrand syndrome .
Udokotela usithola kanjani lesi sifo?
Uma utshela udokotela wakho ngezimpawu zakho, uzoqala akuhlole bese ebheka izimpawu zokuphuka noma ezinye izimpawu zokopha. Bese bebuza ukuthi kukhona yini emndenini wakho oke waba nalolu hlobo lwenkinga yokopha.
Kudingeka ukuhlolwa kwegazi okuningana ukuze kutholakale ngokunembile lesi sifo.
- Ukubalwa kwegazi okuphelele (i-CBC): Lokhu kuhlola inani lamaseli abomvu, amaseli amhlophe, nama-platelet egazini lakho. Uma inani lama-platelet akho liphansi, noma uma i-hemoglobin yakho iphansi ngenxa ye-anemia, lesi simo singasolwa.
- Ukuhlolwa kokujiya kwegazi: Lokhu ukuhlolwa okukala ukuthi kuthatha isikhathi esingakanani ukuthi igazi lakho lijiye.
- Ukuhlolwa kwe-von Willebrand factor (VWF): Lokhu kuhlolwa okukhethekile kuhlola izinga leprotheyini ye-VWF egazini lakho nokuthi isebenza kahle yini.
- Ukuhlolwa kwe-Factor VIII: Iphrotheni ye-VWF ivikela i-Factor VIII, enye iphrotheni ebalulekile esiza ukujiya kwegazi. Ngakho-ke uma i-VWF iphansi, amazinga e-Factor VIII nawo angaba phansi. Lokhu kuhlolwa kubheka lokho.
Yiziphi izindlela zokwelapha lokhu?
Ukwelashwa kuncike ohlotsheni lwesifo onaso kanye nobukhulu bezimpawu zakho. Iningi labantu lidinga ukwelashwa ngaphambi kokuhlinzwa noma ngemva kokulimala.
Kunezindlela eziningana zokwelapha eziyinhloko:
- I-Desmopressin (i-DDAVP): Lena yindlela yokwelapha evame ukusetshenziswa kakhulu. Iyi-hormone. Inikezwa njengesifutho samakhala noma njengomjovo. Isebenza ngokusheshisa ukukhishwa kwamaprotheni e-VWF agcinwe emizimbeni yethu egazini.
- Ukufakwa kwe-von Willebrand factor: Ezimweni ezimbi kakhulu, uhlobo oluhlanganisiwe lwe-VWF lunikezwa umzimba ngomjovo. Lokhu kufana nokunikeza umzimba iphrotheni ye-VWF enciphile.
- Imithi yokubulala amagciwane: Le mithi isebenza ngokuvimbela ukuqhekeka kwegazi ekuncibilikeni ngokushesha. Inikezwa ukulawula ukopha ezimweni ezifana nokukhishwa kwamazinyo.
- Amaphilisi okuvimbela inzalo: Lawa awusizo kwabesifazane abaphuma igazi eliningi ngesikhathi sokuya esikhathini. I-hormone ye-estrogen kula maphilisi isiza ekwandiseni amazinga e-VWF.
Kufanele ngimbone nini udokotela?
Uma wopha kalula, uphuka kalula, noma unesikhathi esiningi, qiniseka ukuthi ubona udokotela womndeni wakho. Kungaba nezinye izimbangela zokopha kakhulu, ngakho-ke kubalulekile ukuthola ukuxilongwa okufanele.
Okubaluleke kakhulu: Noma nini lapho ungakwazi ukulawula ukuphuma kwegazi, iya e-Emergency Treatment Unit (ETU) yesibhedlela ngokushesha.
Yini engingayenza ngenkathi ngiphila nalesi sifo?
Ukuba nesifo se-von Willebrand kusho ukuthi udinga ukuqaphela kakhudlwana ukuze uvimbele ukuphuma kwegazi. Udokotela wakho uzokweluleka ngalokhu. Ngokuvamile, kungcono ukugwema okulandelayo:
- Gwema imidlalo engabangela ukulimala: Imidlalo ehilela ukuthintana kakhulu, njenge-rugby kanye ne-boxing, ayifaneleki.
- Gwema ukuthatha imithi yokunciphisa igazi: Ungaphuzi imithi yokunciphisa ubuhlungu njenge -aspirin noma ama-NSAID (isib., i-Ibuprofen, i-Diclofenac) ngaphandle kwemvume kadokotela wakho.
- Gwema ukuthatha izithasiselo ezithile: Izinto ezifana ne-vitamin E namafutha enhlanzi zinganciphisa igazi. Buza udokotela wakho ngaphambi kokusebenzisa noma yini.
Tshela bonke odokotela bakho (kufaka phakathi udokotela wamazinyo) ukuthi unalesi simo ukuze bakwazi ukuthatha izinyathelo zokulawula ukopha ngaphambi kokwenza noma yini, njengokuhlinzwa. Kungumqondo omuhle futhi ukugqoka isongo sesaziso sezokwelapha ukuze uxwayise abasebenzi bezokwelapha uma kwenzeka isimo esiphuthumayo.
Umlayezo Wokuya Nawe Ekhaya
- Isifo sikaVon Willebrand siwukugula kwegazi okubangelwa ukuntuleka noma ukungasebenzi kahle kweprotheni ebizwa ngokuthi i-von Willebrand factor (VWF).
- Uhlobo oluvame kakhulu (Uhlobo 1) lunezimpawu ezincane kakhulu futhi lungase lungabonakali nhlobo izimpawu.
- Ukopha impumulo njalo, ukuphuka kalula, ukuya esikhathini okunzima, kanye nokopha isikhathi eside ngenxa yamanxeba kuyizimpawu eziyinhloko.
- Nakuba ingelapheki ngokuphelele, ingaphathwa kahle kakhulu ngokwelashwa okufana ne-Desmopressin, i-VWF infusions, kanye nokushintsha indlela yokuphila.
- Uma unalezi zimpawu, ungangabazi ukubona udokotela wakho bese wenza izivivinyo ezidingekayo. Ukuxilongwa nokuphathwa ngendlela efanele yizinto ezibaluleke kakhulu.
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