Wakambova nekucheka kudiki kwakatora nguva yakareba kupfuura zvaitarisirwa kubuda ropa here? Kana kuti wakambova nemhino dzakabuda ropa kana mavanga mumuviri wako wese here? Ungave uchifunga kuti, "Ah... ndizvo zvandiri." Asi panogona kunge paine chikonzero chekurapa chiri shure kwezvinhu izvi chausingazive. Nhasi tichataura nezvechirwere chinokanganisa maitiro ekugwamba kweropa, icho vanhu vazhinji vasati vatombonzwa nezvacho, asi chisiri chinowanzoitika. Ndiwo chirwere chevon Willebrand.
Zvichitaurwa zviri nyore, chii chinonzi chirwere chevon Willebrand?
Chirwere chevon Willebrand chirwere cheropa chinokonzerwa nemajini chinokanganisa kugona kweropa redu kugomara zvakanaka.
Fungidzira kuti kana tikava neronda mumuviri wedu, pane boka diki revashandi vanomisa kubuda kweropa. Vashandi ava vanoshanda pamwe chete uye vanobatsira kuvhara ronda, seplaster. "Manager" iyoyo ipuroteni inonzi von Willebrand factor (VWF) . Iyi puroteni yeVWF ndiyo inobatsira maplatelet muropa kunamirana pamwe chete uye kugadzira ropa rakagwamba rinovhara ronda.
Zvino, mumunhu ane chirwere chevon Willebrand, muviri haugadzi puroteni iyi yeVWF yakakwana. Kana kuti puroteni inogadzirwa haishande nemazvo. Saka nekuda kwekuti "maneja" iyeye haapo, zvinotora nguva yakareba kuti maplatelet anamatire pamwe chete oumba ropa rakagwamba. Ndosaka kunyange ronda diki richiramba richibuda ropa.
Kana chirwere ichi chikanyanya, kubuda ropa kunogona kuitika mukati memajoini kana munyama dzakapfava, zvichikonzera kurwadziwa kwakanyanya uye kuzvimba. Mune vamwe vanhu, izvi zvinogonawo kukonzera anemia , chirwere chinoita kuti huwandu hweropa mumuviri huderere.
Ichi chirwere hachirapiki zvachose. Asi musazvidya mwoyo! Chiremba wenyu, kunyanya nyanzvi yeropa, anogona kukubatsirai kubata chirwere ichi zvakanaka uye kurarama hupenyu hwakajairika.
Pane mhando huru dzechirwere ichi here?
Ehe, chirwere chevon Willebrand hachina kufanana kune wese munhu. Kune mhando nhatu huru dzacho. Kuoma kwezviratidzo kunosiyana zvichienderana nerudzi rwacho.
| Rudzi rwechirwere | Tsananguro |
|---|---|
| Rudzi 1 | Urwu ndirwo rudzi rwakajairika . Vanhu vatatu pavanhu vana vane chirwere ichi vane rudzi urwu. Panyaya iyi, huwandu hweVWF hwakaderera, asi zviratidzo zvacho hazvina kunyanya kuipa. Dzimwe nguva panogona kunge pasina zviratidzo zvachose. |
| Rudzi rwechipiri | Murudzi urwu, muviri unogadzira puroteni yeVWF, asi haishande nemazvo. Kureva kuti, kunyangwe iine "maneja," haizive kuti yoita sei basa rayo. Izvi zvinogona kukonzera kubuda ropa zvishoma kusvika pakati nepakati. |
| Rudzi rwechitatu | Urwu ndirwo rudzi rusingawanzoonekwi uye rwakanyanya kuoma . Panyaya iyi, huwandu hweVWF protein mumuviri hunenge hwakaderera zvikuru, dzimwe nguva husingatoonekwi. Izvi zvinokuisa panjodzi yekubuda ropa zvakanyanya. |
Ndezvipi zviratidzo zvechirwere chevon Willebrand?
Kune vanhu vazhinji, kunyanya avo vane Type 1, zviratidzo hazvina kunyanya kuipa. Vanogona kurarama vasina kana kuziva kuti vane chirwere ichi. Zvisinei, muzviitiko zvakakomba, zviratidzo zvinotevera zvinogona kuitika:
- Kubuda ropa mumhino nguva dzose: Kana mhino yako ichibuda ropa kubva kuchinhu chidiki diki uye zvakaoma kumira.
- Kubuda ropa kwenguva refu kubva paronda diki: Kana ronda rikabuda ropa kwenguva inopfuura maminetsi gumi.
- Kukuvara zviri nyore: Kana ukava nemavanga makuru, akazvimba kunyangwe mushure mekubuda bundu diki.
- Kubuda ropa rakawanda panguva yekuenda kumwedzi kuvakadzi: Kana ukabuda ropa rakawanda kupfuura zvakajairika panguva yekuenda kumwedzi. Semuenzaniso, kana uchifanira kuchinja mapedhi ako usati wapera awa imwe kana maviri, kana ukabvisa ropa rakawanda, kana kana ukabuda ropa kwemazuva anopfuura manomwe, unofanira kunetseka.
- Kubuda ropa rakawanda mushure mekuvhiyiwa: Kana ukabuda ropa rakawanda kupfuura zvakajairika, kunyangwe mushure mechinhu chakareruka sekubviswa kwezino.
- Kubuda ropa rakawanda mushure mekusununguka kana kuti pamuviri pakabva.
- Ropa mutsvina kana weti.
Sei chirwere ichi chichiitika? Chinogarwa nhaka sei?
Chirwere chevon Willebrand chirwere chemajini . Zvichitaurwa zviri nyore, chinokonzerwa nekusakwana kuri mujini kunodzora kugadzirwa kweprotein yeVWF. Tinogara nhaka jini iri risina kunaka kubva kuvabereki vedu.
Kune nzira mbiri dzekugara nhaka iyi:
1. Kugara nhaka inotonga muviri: Izvi zvinobva kuna amai kana baba.Kunyangwe munhu mumwe chete akagara nhaka jini rakakanganisika, chirwere ichi chinogona kukura. Rudzi rwekutanga nerudzi rwechipiri zvinowanzogara nhaka nenzira iyi.
2. Kugara nhaka yeAutosomal recessive: Muchiitiko ichi, kuti chirwere ichi chibudirire, vabereki vese vanofanira kugara nhaka yejini rakakanganisika. Rudzi rwechitatu, rudzi rwakanyanya kuoma, runowanzogara nhaka nenzira iyi.
Kashoma kuti mamwe magomarara, zvirwere zve autoimmune, uye chirwere chemwoyo zvinogona kukonzera chirwere ichi gare gare muhupenyu. Chinonzi acquired von Willebrand syndrome .
Chiremba anoongorora sei chirwere ichi?
Kana ukaudza chiremba wako nezvezviratidzo zvako, iye achatanga akuongorora uye otarisa zviratidzo zvemavanga kana zvimwe zviratidzo zvekubuda ropa. Ipapo vachabvunza kana paine munhu mumhuri mako akambova nedambudziko reropa rakadai.
Kuongororwa ropa kwakawanda kunodiwa kuti chirwere chiongororwe nemazvo.
- Kuverengwa kweropa kwakazara (CBC): Izvi zvinoongorora huwandu hwemasero matsvuku, masero machena, uye maplatelet muropa rako. Kana huwandu hwemaplatelet ako hwakaderera, kana hemoglobin yako yakaderera nekuda kweanemia, chirwere ichi chingafungidzirwa.
- Kuongororwa kweropa: Iyi iongororo inoyera kuti zvinotora nguva yakareba sei kuti ropa rako rigwambe.
- bvunzo dzevon Willebrand factor (VWF): ongororo idzi dzakagadzirwa dzinoongorora huwandu hweprotein yeVWF muropa rako uye kana iri kushanda nemazvo.
- Kuongororwa kweFactor VIII: Puroteni yeVWF inodzivirira Factor VIII, imwe puroteni inokosha inobatsira kuumbwa kweropa. Saka kana VWF yakaderera, mazinga eFactor VIII anogonawo kuva akaderera. Kuongororwa uku kunotsvaga izvozvo.
Ndezvipi zvinorapwa izvi?
Kurapwa kunoenderana nerudzi rwechirwere chaunacho uye kuoma kwezviratidzo zvako. Vanhu vazhinji vanongoda kurapwa vasati vavhiyiwa kana mushure mekukuvara.
Kune nzira dzinoverengeka huru dzekurapa:
- Desmopressin (DDAVP): Uyu ndiwo mushonga unonyanya kushandiswa. Ihormone. Inopihwa semushonga wekupfapfaidza mumhino kana jekiseni. Inoshanda nekukurumidzisa kuburitswa kwemapuroteni eVWF akachengetwa mumiviri yedu muropa.
- Kuiswa kweVWF neVon Willebrand: Muzviitiko zvakakomba, mhando yeVWF yakawandisa inopiwa mumuviri kuburikidza nejekiseni. Izvi zvakafanana nekupa muviri puroteni yeVWF yapera.
- Mishonga inorwisa fibrinolytics: Mishonga iyi inoshanda nekumisa ropa kuti rirege kunyungudika nekukurumidza. Inopihwa kudzora kubuda ropa mumamiriro ezvinhu akadai sekubviswa kwezino.
- Mapiritsi ekudzivirira pamuviri: Aya anobatsira vakadzi vane ropa rakawanda rinobuda panguva yekuenda kumwedzi. Homoni yeestrogen iri mumapiritsi aya inobatsira kuwedzera huwandu hweVWF.
Ndinofanira kuona chiremba rini?
Kana uchibuda ropa zviri nyore, uchikuruma zviri nyore, kana kuti uchiita mwedzi yakawanda, iva nechokwadi chekuona chiremba wemhuri yako. Panogona kunge paine zvimwe zvinokonzera kubuda ropa zvakanyanya, saka zvakakosha kuti uwane chirwere chakakodzera.
Chinonyanya kukosha: Chero nguva yausingakwanise kudzora kubuda kweropa, enda kuEmergency Treatment Unit (ETU) yechipatara nekukasika.
Ndingaitei ndichirarama nechirwere ichi?
Kuva nechirwere chevon Willebrand zvinoreva kuti unofanira kungwarira zvakanyanya kudzivirira kubuda ropa. Chiremba wako achakupa mazano panyaya iyi. Kazhinji, zvakanaka kudzivisa zvinotevera:
- Dzivisa mitambo inogona kukonzera kukuvara: Mitambo inosanganisira kubata-bata zvakanyanya, senge rugby nebhokisi, haina kukodzera.
- Dzivisa kutora mishonga inobvisa ropa rakawanda: Usatore mishonga inoderedza marwadzo yakaita seaspirin kana NSAIDs (semuenzaniso, Ibuprofen, Diclofenac) usina mvumo yachiremba.
- Dzivisa kutora zvimwe zvinovaka muviri: Zvinhu zvakaita sevhitamini E nemafuta ehove zvinogona kuderedza ropa. Bvunza chiremba wako usati washandisa chero chinhu.
Udza vanachiremba vako vese (kusanganisira chiremba wemazino) kuti une dambudziko iri kuitira kuti vatore matanho ekudzora kubuda ropa vasati vaita chero chinhu, senge kuvhiyiwa. Zvakanakawo kupfeka bracelet yekuzivisa chiremba kana paine emergency.
Mharidzo Yekuenda Nayo Kumba
- Chirwere cheVon Willebrand chirwere chinotapukira kubva pakugayiwa kweropa chinokonzerwa nekushaikwa kana kusashanda zvakanaka kweprotein inonzi von Willebrand factor (VWF).
- Rudzi rwakajairika (Rudzi 1) rune zviratidzo zvishoma uye runogona kusaratidza zviratidzo zvachose.
- Kubuda ropa mumhino nguva nenguva, mavanga ari nyore, kuenda kumwedzi zvakanyanya, uye kubuda ropa kwenguva refu kubva mumaronda ndizvo zviratidzo zvikuru.
- Kunyangwe isingarapike zvachose, inogona kurapwa zvakanaka nekurapwa kwakadai seDesmopressin, VWF infusions, uye shanduko dzemararamiro.
- Kana uine zviratidzo izvi, usazeza kuona chiremba wako woongororwa. Kuongorora chirwere nemazvo uye kutarisirwa zvakanaka ndizvo zvinhu zvinonyanya kukosha.
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