Wake waba nokusikeka okuncane ongakwazi ukuyeka ukuphuma kwegazi isikhathi eside? Noma uthola imivimbo emikhulu eluhlaza okwesibhakabhaka ngisho nangemva kokuqhuma okuncane? Ngezinye izikhathi sicabanga ukuthi lezi yizinto ezivamile, kodwa zingaba uphawu lwenkinga encane egazini lethu. Namuhla, sizokhuluma ngesimo esingakhulunywa ngaso kakhulu kodwa esivame kakhulu esithinta ukujiya kwegazi. Leso yisifo sika-von Willebrand.
Kalula nje, siyini isifo se-von Willebrand (VWD)?
Isifo sika-von Willebrand (VWD) yisimo esenzeka lapho inqubo yethu yokujiya kwegazi ingasebenzi kahle. Siwubuthakathaka obuzuzwe njengefa emizimbeni yethu kunokuba yisifo.
Cabanga nje, lapho sinqunywa komunye wemithambo yethu yegazi, uhlobo oluncane lweseli (ama-platelet) emzimbeni wethu luyaye lumise ukuphuma kwegazi futhi luvale inxeba. Kufana nokugcwalisa imbobo odongeni ngezitini. Lawa ma-platelet anamathelana futhi anephrotheni ekhethekile efana "nensini" egazini lethu ewasiza ekwakheni i-clot enamandla. Yilokho esikubiza ngokuthi i-von Willebrand factor (VWF) .
Umuntu one-VWD angase angabi naleli phrotheni le-VWF elanele emzimbeni wakhe, noma iphrotheni anayo ingase ingasebenzi kahle. Ngemuva kwalokho, ngenxa yokuthi ihlule legazi alikhi kahle, kuthatha isikhathi eside ukuyeka ukuphuma kwegazi ngisho noma kubangelwa ukulimala okuncane.
Lesi yisifo sokuphuma kwegazi esivame kakhulu emhlabeni. Kulinganiselwa ukuthi cishe umuntu oyedwa kwabayi-100 angaba nalesi simo.
Yiziphi izinhlobo eziyinhloko ze-VWD?
Lesi simo sihlukaniswe kakhulu ngezinhlobo ezintathu. Kukhona nohlobo olungazalwa nalo kodwa oluvela kamuva.
| Uhlobo | Incazelo |
|---|---|
| Uhlobo 1 | Lolu uhlobo oluvame kakhulu (phakathi kuka-60% - 80%). Lubangelwa ukuthi umzimba une-VWF protein encane kakhulu. Izimpawu zivame ukuba zincane. |
| Uhlobo 2 | Uhlobo lwesibili oluvame kakhulu (phakathi kuka-15% - 30%). Nakuba iphrotheni ye-VWF ikhona lapha, umsebenzi wayo ubuthakathaka. Ifana ne-gum endala, ayisebenzi ngisho noma ikhona. Izimpawu zingaba zincane kuya kweziphakathi. |
| Uhlobo 3 | Lolu uhlobo olunzima kakhulu nolungavamile. Lapha, iphrotheni ye-VWF cishe ayikho nhlobo emzimbeni. Ngakho-ke, izimpawu zinzima kakhulu. |
| I-VWD etholiwe | Lokhu akuyona into ozalwa nayo. Kungakhula kamuva ngenxa yezifo ezithile zokuzivikela komzimba (isib. i-lupus), isifo senhliziyo, izinhlobo ezithile zomdlavuza, noma imithi oyiphuzayo. |
Ziyini izimpawu zalesi simo?
Izimpawu ziyahlukahluka kuye ngohlobo lwe-VWD onayo.
Izimpawu ezincane neziphakathi zezinhlobo 1 no-2
- Ngisho nokulimala okuncane kungabangela ukulimala okukhulu okuluhlaza okwesibhakabhaka: Uyaqaphela yini ukuthi umzimba wakho uphenduka ube luhlaza okwesibhakabhaka ezindaweni ezithile?
- Ukopha igazi ekhaleni njalo: Abanye abantu bavame ukopha igazi ekhaleni, futhi kunzima ukukuyeka.
- Ukopha ngokweqile ngemva kokuhlinzwa noma ukukhishwa kwezinyo: Ingabe ukopha akupheli amahora amaningi ngemva kokukhishwa kwezinyo?
- Abesifazane babhekana nokopha okukhulu ngesikhathi sokuya esikhathini: Ingabe ukopha kunzima kangangokuthi kufanele ushintshe iphedi yakho okungenani kanye ngehora? Ingabe kuza namahlule? Ngokuhamba kwesikhathi, lokhu kungaholela ekuntulekeni kwe-anemia yokuntuleka kwensimbi.
- Igazi endle noma emchameni.
Izimpawu ezinzima zohlobo 3
Kulolu hlobo, zonke izimpawu ezingenhla zingaba zimbi kakhulu. Ngaphezu kwalokho,
- Ukopha okungazelelwe, okukhulu ngaphandle kwesizathu.
- Igazi livuza emalungeni nasezicutshini ezithambile, okubangela ukuvuvukala nobuhlungu obukhulu.
Into ebalulekile ukuthi akuwona wonke umuntu onalezi zimpawu one-VWD. Kodwa uma unesinye noma ngaphezulu salezi zimpawu njalo, kungcono ukubona udokotela ukuze akunike iseluleko.
Ukubona kanjani lokhu?
Udokotela wakho uzoqala ngokubuza ngezimpawu zakho kanye nomlando wezokwelapha womndeni. Ngenxa yokuthi lesi yisimo esizuzwa njengefa, kubalulekile ukuthola ukuthi ngabe kukhona yini omunye emndenini wakho oke waba nalolu hlobo lwenkinga yokopha.
Ngemuva kwalokho, bazokwenza izivivinyo ezikhethekile ukuze babone ukuthi kuthatha isikhathi esingakanani ukuthi igazi lakho lijiye nokuthi lijiya kangakanani. Ngokuyinhloko,
- Ukulinganisa inani leprotheyini ye-VWF egazini (ukuhlolwa kwe-Antigen).
- Ukuhlola ukusebenza kwaleyo phrotheni.
Ngezinye izikhathi, izinto ezifana nokucindezeleka nokuzivocavoca umzimba kungashintsha amazinga e-VWF, ngakho-ke kungadingeka ukuthi wenze lezi zivivinyo izikhathi ezingaphezu kwesisodwa ukuze uthole imiphumela enembile kakhulu.
Yiziphi izindlela zokwelapha? Indlela yokuphatha?
Nakuba i-VWD ingelapheki, ingalawulwa futhi iphathwe ukuze iphile impilo evamile. Into ebaluleke kakhulu ukunciphisa ingozi yokopha.
1. Ukugwema imithi ethile
Kufanele ugweme ukuthatha imithi ethile enciphisa igazi. Udokotela wakho uzovame ukukutshela ukuthi ungayithathi i-aspirin nama -NSAID (njenge-ibuprofen ne-naproxen). I-Acetaminophen iyindlela ephephile yokwelapha ubuhlungu noma umkhuhlane. Kodwa qiniseka ukuthi ubuza udokotela wakho ngaphambi kokuthatha noma yimuphi umuthi.
2. Ukwelashwa okukhethekile
Ukwelashwa kuncike ebunzimeni kanye nohlobo lwezimpawu zakho.
- I-Desmopressin Acetate (DDAVP): Lena yindlela yokwelapha evame ukusetshenziswa kakhulu. Inganikezwa njengesifutho sekhala noma njengomjovo. Isebenza ngokuvuselela ukukhishwa kweprotheyini ye-VWF egcinwe emaseli ethu egazini. Lo muthi ungabangela ukugcinwa kwamanzi emzimbeni, ngakho udokotela wakho angancoma ukuthi unciphise uketshezi olusetshenziswayo.
- I-Clotting Factor Concentrates: I-concentrate equkethe iphrotheni ye-VWF enikezwa nge-intravenously (IV). Lokhu kuvame ukusetshenziswa ezimweni ezimbi kakhulu nangaphambi kokuhlinzwa.
- Eminye imithi: Ezimweni ezifana nokukhishwa kwamazinyo, amaphilisi noma imithi yoketshezi efana ne-aminocaproic acid noma i-tranexamic acid inganikezwa ukuze kumiswe ukuqhekeka kwegazi.
- Ukwelashwa okukhethekile kwabesifazane: Kwabesifazane abanesikhathi esiningi, amaphilisi okuvimbela inzalo angakhuphula amazinga e-VWF. Kukhona nezindlela ezifana nokugqoka i-loop equkethe ama-hormone (idivayisi ye-levonorgestrel intrauterine) noma ukususa ulwelwesi lwesibeletho (i-endometrial ablation).
Okokugcina, uma unesifo esibi njengohlobo lwesithathu, uma uzwa ukopha, kuyisimo esiphuthumayo. Kufanele uye eMnyangweni Wezimo Eziphuthumayo wesibhedlela (ETU) ngokushesha. Ukulibala kungaba yingozi empilweni.
Umlayezo Wokuya Nawe Ekhaya
- Isifo se-von Willebrand (VWD) siyisimo esivamile, esizuzwe njengefa esithinta inqubo yokujiya kwegazi.
- Uma unezimpawu ezifana nokulunywa njalo, ukopha ekhaleni, noma ukopha kakhulu kokuya esikhathini, khathazeka.
- Uma unalezi zimpawu, ungesabi ukubona udokotela ofanelekayo bese ucela iseluleko.
- Uma utholakale une-VWD, gwema ukuthatha i-aspirin noma imithi yokubulala izinhlungu ye-NSAID ngokuphelele ngaphandle kweseluleko sikadokotela.
- Ngokuphathwa nokwelashwa okufanele, ungaphila impilo enempilo nejwayelekile ngalesi simo.
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