Awunankinga yini nokuvimba ukopha ngisho noma unesilonda esincane? Ake sixoxe ngesifo sika-von Willebrand!

Ingabe ngezinye izikhathi uba nokusikeka okuncane okungayeki ukuphuma kwegazi isikhathi eside? Noma ingabe uvame ukuphuma igazi ekhaleni? Mhlawumbe othile emndenini wakho unezinkinga ezifanayo? Uma kunjalo, lokhu kuyinto engaba yinto ebalulekile kuwe. Namuhla sizokhuluma ngesimo esingavamile kodwa esivame kakhulu esibizwa ngokuthi i -von Willebrand Disease .

Kuyini isifo sikaVon Willebrand?

Kalula nje, i-von Willebrand Disease yisifo lapho igazi lethu lingavimbi kahle, okusho ukuthi inqubo esiza ekumiseni ukopha iyancipha. Lokhu kuvame ukwenziwa njengefa , okusho ukuthi abazali bangadlulisela lesi sifo ezinganeni zabo. Kodwa ungakhathazeki, kukhona ukwelashwa kwalokhu. Odokotela bangakunikeza imithi esiza kule nqubo yokuvimbisisa igazi.

Kwenzekani kumuntu onesifo sikaVon Willebrand?

Umuntu onalesi sifo angase ophe kakhulu kunokujwayelekile. Cabanga nje, ngisho nokusikwa okuncane noma ukukhishwa kwezinyo kungathatha isikhathi eside ukuyeka ukopha. Abanye abantu bavame ukuphuma igazi ekhaleni. Kwabesifazane, bangase bophe igazi kakhulu ngesikhathi sokuya esikhathini, noma bangase bophe igazi kakhulu ngemva kokubeletha.

Ezimweni ezimbi kakhulu zesifo, ukuphuma kwegazi ngezinye izikhathi kungenzeka ngaphandle kwesizathu esibonakalayo emalungeni noma ezicutshini ezithambile. Lokhu kungabangela ukuvuvukala nobuhlungu obukhulu. Kwabanye, kungaholela ngisho nasekulahlekelweni kwegazi , okuwukuntuleka kwegazi .

Sivame kangakanani lesi simo?

Empeleni lesi yisifo sokuphuma kwegazi esivame kakhulu emhlabeni. Eminye imibiko iphakamisa ukuthi sithinta cishe u-1% wabantu baseMelika. Emhlabeni wonke, kulinganiselwa ukuthi abantu abaphakathi kuka-23 no-110 esigidini bangaba nalesi simo. Kodwa-ke, lezi zinombolo zingahluka, njengoba abanye abantu bengase babe nezinkinga zokuphuma kwegazi kodwa bangatholakali benesifo se-von Willebrand . Abanye abantu kungenzeka ukuthi bebe nalezi zinkinga iminyaka eminingi, kodwa abatholakali kuze kube sekwephuzile.

Ingabe isifo sikaVon Willebrand kanye ne-hemophilia ziyafana?

Yebo, nakuba zombili ziyizinkinga zokujiya kwegazi, i-von Willebrand Disease yisimo esivame ukuba nezimpawu ezimbalwa futhi esingesibi kakhulu njenge -hemophilia . Kodwa lokho akusho ukuthi kuyinto okufanele ungayinaki.

Ziyini izimpawu zesifo sikaVon Willebrand?

Abantu abaningi abanalesi sifo bangase bangabi nazimpawu nhlobo , noma babe nezimpawu ezincane kakhulu. Kodwa-ke, labo abanesifo esibi kakhulu bangase babe nezimpawu ezifana nalezi:

• Ukopha ekhaleni : Lokhu akuyona nje ukopha okuvamile. Kungaba ukopha isikhathi esingaphezu kwemizuzu eyi-10, noma ukopha ekhaleni izikhathi ezingaphezu kwezihlanu ngonyaka.
• Ukopha okuvela esilondeni: Ukopha okuvela esilondeni esincane noma esinye inxeba isikhathi esingaphezu kwemizuzu eyi-10.
• Imivimbo: Abantu abanalesi sifo bathola imivimbo kalula. Imivimbo ayigcini nje ngokuba yisicaba esikhumbeni, kodwa ingavuvukala kancane futhi iphakanyiswe njengeqhubu. Imivimbo ingaba nkulu kunemali yama-rupee amahlanu.
• Ukuntuleka kwensimbiI-anemia yokuntuleka kwe-iron: I-anemia yisimo lapho umzimba ungenawo amangqamuzana abomvu egazi anele. I-anemia yokuntuleka kwe-iron yenzeka lapho umzimba wethu ungenayo i-iron eyanele yokwenza i-hemoglobin . Le nto, ebizwa ngokuthi i-hemoglobin, isiza amangqamuzana abomvu egazi ukuthwala umoya-mpilo. Ngakho-ke, uma silahlekelwa igazi eliningi kakhulu, singalahlekelwa yi-iron futhi sibe nalesi simo.
• Ukopha ngemva kokuhlinzwa: Abantu abanalesi sifo bangopha kakhulu, ngisho nangemva kokuhlinzwa okuncane, njengokukhishwa kwezinyo.
• Ukuya esikhathini okukhulu: Kwabanye besifazane, uma ukuphuma kwabo esikhathini kunzima kangangokuthi kufanele bashintshe iphedi noma i-tampon njalo ngehora, noma uma ukuya kwabo esikhathini kuqhubeka izinsuku ezingaphezu kweziyisikhombisa, kungaba uphawu lwalesi simo.
• Ukopha okukhulu ngemva kokubeletha noma ukuphuphuma kwesisu.
• Igazi esitulweni: Kungakhathaliseki ukuthi unegazi esitulweni sakho noma igazi ngemva kokudlula esitulweni, kungaba uphawu lwesinye isimo sezokwelapha. Ngakho-ke , kufanele nakanjani ubone udokotela .
• Igazi emchameni (hematuria): Uma ubona igazi lapho uchama, ikakhulukazi uma igazi lihambisana nesifiso sokuchama ngokuzumayo, tshela udokotela ngokushesha .

Into ebaluleke kakhulu ukuthi uma unesinye noma ngaphezulu salezi zimpawu, ungazinaki bese ubona udokotela ukuze akunike iseluleko.

Yini ebangela isifo sikaVon Willebrand?

Lesi yisifo esibangelwa izakhi zofuzo . Lokhu kusho ukuthi lesi sifo sibangelwa ushintsho (ukuguquka) kwezakhi zofuzo zethu. Ngenxa yalezi zinguquko zezakhi zofuzo, imizimba yethu ayikwazi ukukhiqiza kahle iphrotheni ebizwa ngokuthi i-von Willebrand factor . Lezi zinto zingamaprotheni asiza ukujiya kwegazi lethu.

Lesi sici se-von Willebrand sitholakala ku -plasma (ingxenye ewuketshezi yegazi lethu), ama-platelet (amaseli asiza ukujiya kwegazi), kanye nezindonga zemithambo yegazi . Ngokuvamile, lapho umthambo wegazi wonakele, ama-platelet anamathela endaweni eyonakele, akhe i-blood clot futhi amise ukuphuma kwegazi.I-Von Willebrand factor yiyona esiza lawa ma-platelet ukuthi anamathele kahle. Ngakho-ke, uma ulahlekelwa ngokwanele yilesi sici, noma uma usilahlekelwa ngokuphelele, ama-platelet awakwazi ukunamathela kahle. Bese kuthatha isikhathi eside ukuthi kwakheke ihlule legazi.

Iningi labantu lithola lesi sifo ngokuthola ifa lofuzo eliguquliwe komunye wabazali babo . Lokhu kubizwa ngokuthi i-autosomal dominant inheritance . Abanye bangathola ifa lofuzo eliguquliwe kubo bobabili abazali . Lokhu kubizwa ngokuthi i-autosomal recessive inheritance , futhi kuyindlela embi kakhulu yalesi sifo. Umuntu ophethe lesi sakhi sofuzo esiguquliwe unethuba elingu-50% lokudlulisela lesi sakhi sofuzo ezinganeni zakhe.

Futhi, ngezinye izikhathi isifo se-von Willebrand singaba yinkinga yezinye izimo zezokwelapha, njengomdlavuza , izifo zokuzivikela komzimba , isifo senhliziyo, kanye nezifo zemithambo yegazi.

Odokotela bahlonza kanjani isifo sikaVon Willebrand?

Uma ubona udokotela, bazoqala bakubuze ngezimpawu zakho. Bangase futhi bakubuze ukuthi kukhona yini emndenini wakho oke waba nezimpawu ezifanayo noma izinkinga zokopha. Bese benza ezinye izivivinyo, ezifana nalezi:

• Ukubalwa kwegazi okuphelele (i-CBC): Lokhu kulinganisa inani lamaseli abomvu egazi, izinhlobo ezahlukene zamaseli amhlophe egazi, nama-platelet egazini lakho. Kulinganisa nenani le-hemoglobin kumaseli akho abomvu egazi. Iningi labantu abanesifo se-von Willebrand bane-CBC evamile. Kodwa-ke, abantu abopha kakhulu bangase babe ne-hemoglobin ephansi kanye nenani lamaseli abomvu egazi eliphansi.
• Ukuhlolwa kokuhlanganiswa kwama-platelet: Lokhu kuhlola ukuthi ama-platelet akho anamathelana kahle kangakanani ukusiza ekwakheni ama-blood clots.
• Ukuhlolwa kwesikhathi se-thromboplastin okungaphelele (APTT) okusebenzayo: Lokhu kuhlola i-von Willebrand factor kanye nezinye izinto eziqinisa igazi ezisiza ekuqhekekeni kwegazi. Uma amazinga alezi zinto ephansi kunokuvamile, kuthatha isikhathi eside ukuthi igazi liqine.
• Ukuhlolwa kwesikhathi se-Prothrombin (PT): Lokhu kulinganisa nezinye izici zokujiya kwegazi.
• Ukuhlolwa kwe-Fibrinogen:I-Fibrinogen ingenye iphrotheni esiza ukujiya kwegazi.
• Ukuhlolwa kwe-antigen ye-Von Willebrand factor: Lokhu kulinganisa inani leprotheyini ye-von Willebrand factor egazini lakho.
• Ukuhlolwa kwe-ristocetin cofactor: Lokhu kuhlola umsebenzi we-von Willebrand factor.
• Ukuhlolwa kwe-Von Willebrand factor multimers: Lokhu kubheka isakhiwo se-factor.

Udokotela wakho kungadingeka enze ukuhlolwa kwegazi okuningana ukuqinisekisa ukuxilongwa, ngoba izinto ezifana namazinga ama-hormone zingashintsha izinga le-von Willebrand factor egazini lakho.

Kunezinhlobo eziningana zesifo se-von Willebrand. Odokotela bazokwenza izivivinyo ezengeziwe ukuthola ukuthi hlobo luni olunalo. Lezi zinhlobo yilezi:

• Uhlobo 1: Lolu uhlobo oluvame kakhulu. Phakathi kuka-60% no-80% weziguli zinalolu hlobo. Laba bantu banamazinga aphansi e-von Willebrand factor egazini labo. Kungenzeka bangabi nazimpawu. Uma benazo, ziba zimbi kakhulu.
• Uhlobo 2: Kulolu hlobo, i-von Willebrand factor ayisebenzi kahle. Laba bantu bangase bazwe ukopha okuncane kuya kokuphakathi. Phakathi kuka-15% no-30% weziguli banalolu hlobo.
• Uhlobo 3: Lolu uhlobo olubi kakhulu. Luphinde lungavamile kakhulu. Luthinta phakathi kuka-5% no-10% weziguli. Laba bantu banamazinga aphansi kakhulu e-von Willebrand factor egazini labo, noma abanawo nhlobo. Lokhu kungabangela izinkinga ezinkulu zokopha.

Yiziphi izindlela zokwelapha isifo sikaVon Willebrand?

Odokotela baphatha lesi sifo ngezinhlobo ezahlukene zemithi:

• (Desmopressin): Lena yi-hormone. Ikhulisa izinga le-von Willebrand factor egazini. Lena yindlela yokwelapha evame kakhulu yalesi sifo.
• Ukufakwa kwe-Von Willebrand factor: Abanye abantu banikezwa le factor ngemithambo yegazi ukuze bamise ukopha. Lokhu kwelashwa kunganikezwa ngaphambi kokuhlinzwa. Abanye abantu abanesifo esibi kakhulu kungadingeka bathole lokhu kwelashwa njalo ukuze amazinga e-factor egazini labo azinzile.
• (Imithi yokulwa ne-fibrinolytics):Le mithi isebenza ngokuvimbela ukwakheka kwamahlule egazi. Udokotela wakho angakunikeza le mithi uma uhlinzwa amazinyo noma uma uzwa ukopha okukhulu kokuya esikhathini.
• Amaphilisi okuvimbela inzalo: Lawa asiza labo abaphuma igazi eliningi ngesikhathi sokuya esikhathini. I-hormone ye-estrogen kula maphilisi inyusa izinga le-von Willebrand factor egazini.

Nginganciphisa yini ingozi yokuthola lesi sifo?

Lokhu kuvame ukwenziwa ngofuzo . Uma abazali bakho benalesi sifo, ungasithola njengefa komunye wabo noma kubo bobabili. Ngakho-ke akukho esingakwenza ukuvimbela ukuthi singabi khona.

Uma nginalesi simo, yini okufanele ngiyilindele?

Odokotela bangayilapha isifo se-von Willebrand, kodwa abakwazi ukuselapha ngokuphelele . Iningi labantu linesifo sohlobo 1 noma sohlobo 2. Badinga ukwelashwa kuphela uma belimele noma bedinga ukuhlinzwa. Abantu abanesifo sohlobo 3 bangadinga ukwelashwa okuqhubekayo ukuze balawule ukopha.

Nginesifo sikaVon Willebrand. Kufanele ngizinakekele kanjani ?

Njengoba abantu abaningi benezimpawu ezincane kuya kweziphakathi, ukuphila nalesi sifo kusho ukucabangela izinto ezimbalwa:

• Gwema imisebenzi engabangela ukulimala: Kungcono ukugwema imidlalo enomthelela omkhulu, isibonelo, ibhola, i-rugby, kanye ne-hockey.
• Tshela bonke odokotela bakho, kuhlanganise nodokotela wamazinyo, ukuthi unalesi simo: bangabe sebehlela ukuthi bangakulawula kanjani ukopha ngemva kokuhlinzwa.
• Ungasebenzisi i-aspirin noma imithi equkethe i-aspirin.
• Ungasebenzisi imithi elwa nokuvuvukala engeyona eye-steroidal (ama-NSAID) , njenge-ibuprofen, ngaphandle kokuthi unikezwe udokotela owaziyo ukuthi unesifo se-von Willebrand.
• Gwema ukuthatha izithasiselo zokudla okunempilo eziqukethe i-vitamin E, uwoyela wezinhlanzi kanye ne-turmeric.
• Cabanga ngokuhlonza isaziso sezokwelapha: Ukugqoka isongo esinjengalesi noma ukuphatha ikhadi lokuhlonza kungakusiza ukuthi uthole ukunakekelwa okufanele kwezokwelapha esimweni esiphuthumayo.

Kufanele ngiye nini esibhedlela ngokushesha?

Yiya esibhedlela ngokushesha noma nini lapho uphuma igazi ngendlela engalawuleki . Ngisho noma kuyinxeba elincane, uma ukuphuma kwegazi kungayeki, qiniseka ukuthi ucela iseluleko sezokwelapha.

Yimiphi imibuzo okufanele ngiyibuze udokotela?

Uma unesifo se-von Willebrand, ungase ube nemibuzo mayelana nokuthi lesi sifo sizoyithinta kanjani impilo yakho. Nazi ezinye zemibuzo ongayibuza udokotela wakho:

• Kungani ngithole lesi sifo?
• Ingabe izingane zami zingayithola ifa lesi sifo?
• Ingabe lokhu kuzoqhubeka nokuba kubi nakakhulu?
• Yiziphi izindlela zokwelapha lokhu?
• Iyini imiphumela emibi yokwelashwa?
• Ngeke ngikwazi yini ukwenza izinto ezithile, njengokuhamba noma ukudlala imidlalo, ngenxa yalesi sifo?

Ngamafuphi, izinto okudingeka sizikhumbule yilezi:

Isifo sikaVon Willebrand siyinkinga evamile kakhulu, ebangelwa ufuzo yokujiya kwegazi. Iningi labantu linezimpawu ezincane kuya kweziphakathi. Bangase babe nokopha kaningi ekhaleni, noma ngisho nokusikeka okuncane kungathatha isikhathi eside ukuyeka ukopha. Abanye banezimpawu ezinkulu. Isibonelo, bangase babe nokopha emalungeni abo kanye nobuhlungu emalungeni.

Abanye abantu bangase bangazi ukuthi banalesi simo iminyaka eminingi. Uma unaso, kungaba impumuzo ukwazi ukuthi kungani unezinkinga zokopha. Ungase futhi ukhathazeke ngokuthi nezingane zakho nazo zizokhula nalesi simo. Nakuba odokotela bengenakusilapha lesi simo, bangaselapha . Bangaphendula nemibuzo yakho mayelana nokuthi izingane zakho zizosizuza yini njengefa. Bangakunikeza nolwazi oludingayo ukuze uphile nesifo sika-von Willebrand ukuze singakuvimbeli ekuphileni impilo esebenzayo nejwayelekile. Ngakho-ke, ungakhathazeki, uma usiqonda futhi usiphatha kahle, ungaphila kahle naso.