Awunangxaki na nawe yokuyeka ukopha nokuba kukusikeka okuncinci? Masithethe ngesifo sikaVon Willebrand!

Ngaba ngamanye amaxesha unesilonda esincinci esingayeki ukopha ixesha elide? Okanye usoloko uphuma igazi empumlweni? Mhlawumbi kukho umntu kusapho lwakho oneengxaki ezifanayo? Ukuba kunjalo, le yinto enokuba yinto ebalulekileyo kuwe. Namhlanje siza kuthetha ngesimo esingaqhelekanga kodwa esixhaphakileyo esibizwa ngokuba yi -von Willebrand Disease .

Yintoni isifo sikaVon Willebrand?

Ngamafutshane, i-von Willebrand Disease sisifo apho igazi lethu lingajiki kakuhle, oko kuthetha ukuba inkqubo enceda ekumiseni ukopha iyabuthathaka. Oku kudla ngokuba yimfuza , oko kuthetha ukuba abazali banokudlulisela esi sifo kubantwana babo. Kodwa ungakhathazeki, kukho unyango loku. Oogqirha banokukunika amayeza anceda kule nkqubo yokujiya kwegazi.

Kwenzeka ntoni kumntu onesifo sikaVon Willebrand?

Umntu onesi sifo ungopha kakhulu kunesiqhelo. Khawucinge nje, nokuba kunqunyulwe izinyo okanye kukhutshwe izinyo kungathatha ixesha elide ukuyeka ukopha. Abanye abantu baphuma igazi rhoqo empumlweni. Kwabasetyhini, baphuma igazi elininzi ngexesha lokuya exesheni, okanye baphuma igazi elininzi emva kokubeleka.

Kwiimeko ezinzima kakhulu zesifo, ukopha ngamanye amaxesha kunokwenzeka ngaphandle kwesizathu esicacileyo kumalungu okanye kwizicubu ezithambileyo. Oku kunokubangela ukudumba kunye nentlungu eqatha. Kwabanye, kunokukhokelela kwi- anemia , okubangelwa kukungabikho kwegazi .

Ixhaphake kangakanani le meko?

Esi sesona sifo sixhaphakileyo sokuphuma kwegazi ehlabathini. Ezinye iingxelo zibonisa ukuba sichaphazela malunga ne-1% yabemi baseMelika. Ehlabathini lonke, kuqikelelwa ukuba phakathi kwabantu abangama-23 kunye ne-110 kwisigidi banokuba nale meko. Nangona kunjalo, la manani anokwahluka, njengoko abanye abantu banokuba neengxaki zokuphuma kwegazi kodwa bangafunyaniswa ukuba bane- von Willebrand Disease . Abanye abantu basenokuba nezi ngxaki kangangeminyaka, kodwa abafunyaniswa de kube kade.

Ngaba isifo sikaVon Willebrand kunye neHemophilia ziyafana?

Ewe, nangona zombini ziyingxaki yokujiya kwegazi, isifo sikaVon Willebrand sisifo esidla ngokuba neempawu ezimbalwa kwaye esingebi kakhulu njenge -hemophilia . Kodwa oko akuthethi ukuba yinto ekufuneka ingananzwa.

Zithini iimpawu zesifo sikaVon Willebrand?

Abantu abaninzi abanesifo basenokungabi nazimpawu konke konke , okanye babe neempawu ezincinci kakhulu. Nangona kunjalo, abo banesifo esibi kakhulu banokufumana iimpawu ezifana nezi:

• Ukopha empumlweni : Oku akusiyo nje ukopha okuqhelekileyo. Kunokuba kukopha ngaphezu kwemizuzu eli-10, okanye ukopha empumlweni ngaphezu kwezihlandlo ezihlanu ngonyaka.
• Ukopha igazi elivela kwinxeba: Ukopha igazi elivela kwinxeba elincinci okanye elinye inxeba ixesha elingaphezulu kwemizuzu eli-10.
• Imivumbo: Abantu abanesi sifo bafumana imivumbo ngokulula. Le mivumbo ayipheleli nje eluswini, kodwa inokudumba kancinci ize iphakame njengeqhubu. Le mivumbo inokuba nkulu kuneengqekembe zeerandi ezintlanu.
• Ukunqongophala kwesinyithiI-anemia yokunqongophala kwe-iron: I-anemia yimeko apho umzimba ungenazo iiseli ezibomvu zegazi ezaneleyo. I-anemia yokunqongophala kwe-iron yenzeka xa umzimba wethu ungenayo i-iron eyaneleyo yokwenza i-hemoglobin . Le nto, ebizwa ngokuba yi-hemoglobin, inceda iiseli ezibomvu zegazi zithwale i-oksijini. Ngoko ke, xa silahlekelwa ligazi elininzi kakhulu, sinokulahlekelwa yi-iron size siphuhlise le meko.
• Ukopha emva kotyando: Abantu abanesi sifo bangopha kakhulu, nokuba emva kotyando oluncinci, olufana nokukhupha amazinyo.
• Ukuya exesheni kakhulu: Kwabanye abafazi, ukuba ukuphuma kwegazi kwabo exesheni kunzima kangangokuba kufuneka batshintshe i-pad okanye i-tampon yabo rhoqo ngeyure, okanye ukuba ukuya exesheni kwabo kuhlala ngaphezulu kweentsuku ezisixhenxe, oko kusenokuba luphawu lwesi sifo.
• Ukopha kakhulu emva kokubeleka okanye ukuphuma kwesisu.
• Igazi elikwisitulo: Nokuba unegazi kwisitulo sakho okanye igazi emva kokuba uphumile, oko kungaba luphawu lwenye imeko yempilo. Ngoko ke , ngokuqinisekileyo kufuneka ubone ugqirha .
• Igazi kumchamo (hematuria): Ukuba ubona igazi xa uchama, ingakumbi ukuba igazi lihamba nomnqweno wokuchama ngequbuliso, xelela ugqirha ngoko nangoko .

Eyona nto ibalulekileyo kukuba ukuba unenye okanye ngaphezulu kwezi mpawu, ungazityesheli kwaye ubone ugqirha ukuze akunike ingcebiso.

Yintoni ebangela isifo sikaVon Willebrand?

Esi sisifo semfuza . Oku kuthetha ukuba esi sifo sibangelwa lutshintsho (ukuguqulwa) kwimfuza zethu. Ngenxa yolu tshintsho lwemfuza, imizimba yethu ayinakuvelisa ngokufanelekileyo iproteni ebizwa ngokuba yi -von Willebrand factor . Ezi zinto ziiproteni ezinceda ukuqhekeka kwegazi.

Le von Willebrand factor ifumaneka kwi -plasma (inxalenye engamanzi egazini lethu), kwiiplatelets (iiseli ezinceda ekuqhekekeni kwegazi), nakwiindonga zemithambo yegazi . Ngokwesiqhelo, xa umthambo wegazi wonakele, iiplatelets zinamathela kwindawo eyonakeleyo, zenze i-blood clot kwaye ziyeke ukopha.I-Von Willebrand factor yiyo enceda ezi platelets zinamathelene kakuhle. Ngoko ke, xa ulahlekelwa ngokwaneleyo yile factor, okanye ukuba uyilahleka ngokupheleleyo, iiplatelets azinakubambana kakuhle. Emva koko kuthatha ixesha elide ukuba ihlwili legazi lenzeke.

Uninzi lwabantu lufumana esi sifo ngokufumana i-gene eguquliweyo komnye wabazali babo . Oku kubizwa ngokuba yi -autosomal dominant inheritance . Abanye banokuyifumana i-gene eguquliweyo kubazali bobabini . Oku kubizwa ngokuba yi -autosomal recessive inheritance , kwaye lolona hlobo lubi kakhulu lwesifo. Umntu ophethe le gene eguquliweyo unethuba le-50% lokudlulisela i-mutation kubantwana bakhe.

Kwakhona, ngamanye amaxesha isifo se-von Willebrand sinokubakho njengengxaki yezinye iimeko zonyango, ezinje ngomhlaza , iingxaki zokuzikhusela komzimba , isifo sentliziyo, kunye nezifo zemithambo yegazi.

Oogqirha bayixilonga njani i-von Willebrand Disease?

Xa ubona ugqirha, baza kuqala bakubuze ngeempawu zakho. Basenokubuza nokuba kukho umntu kusapho lwakho oye waba neempawu ezifanayo okanye iingxaki zokopha. Emva koko banokwenza uvavanyo oluthile, olufana nolu:

• Ukubalwa kwegazi okupheleleyo (CBC): Oku kulinganisa inani leeseli ezibomvu zegazi, iintlobo ezahlukeneyo zeeseli ezimhlophe zegazi, kunye neeplatelets egazini lakho. Kukwalinganisa ubungakanani be-hemoglobin kwiiseli zakho ezibomvu zegazi. Uninzi lwabantu abanesifo se-von Willebrand bane-CBC eqhelekileyo. Nangona kunjalo, abantu abopha kakhulu banokuba ne-hemoglobin ephantsi kunye nokubalwa kweeseli ezibomvu zegazi okuphantsi.
• Uvavanyo lokuhlanganiswa kweeplatelet: Olu vavanyo luvavanya indlela iiplatelet zakho ezinamathelana ngayo ukunceda ekwenzeni amahlwili egazi.
• Uvavanyo lwexesha le-thromboplastin olusebenzayo (APTT): Olu vavanyo lwe-von Willebrand factor kunye nezinye izinto ezibangela ukujiya kwegazi ezinceda ukujiya kwegazi. Ukuba amanqanaba ezi zinto aphantsi kunesiqhelo, kuthatha ixesha elide ukuba igazi lijiye.
• Uvavanyo lwexesha le-Prothrombin (PT): Oku kukwalinganisa nezinye izinto ezibangela ukujiya kwegazi.
• Uvavanyo lweFibrinogen:I-Fibrinogen yenye iproteni enceda ukujiya kwegazi.
• Uvavanyo lwe-Von Willebrand factor antigen: Olu vavanyo lulinganisa ubungakanani beprotheyini ye-von Willebrand factor egazini lakho.
• Uvavanyo lwe-ristocetin cofactor: Olu vavanyo luvavanya umsebenzi we-von Willebrand factor.
• Uvavanyo lwe-Von Willebrand factor multimers: Olu vavanyo lujonga ulwakhiwo lwe-factor.

Ugqirha wakho unokufuna ukwenza uvavanyo lwegazi oluninzi ukuqinisekisa ukuba unesifo, kuba izinto ezifana namanqanaba eehomoni zinokutshintsha inqanaba le-von Willebrand factor egazini lakho.

Kukho iintlobo ezahlukeneyo zesifo se-von Willebrand. Oogqirha baza kwenza uvavanyo oluthe kratya ukuze bafumane ukuba loluphi uhlobo oluchanekileyo onalo. Ezi ntlobo zezi:

• Uhlobo 1: Olu lolona hlobo luqhelekileyo. Phakathi kwama-60% nama-80% ezigulana zinalolu hlobo. Aba bantu banamanqanaba aphantsi e-von Willebrand factor egazini labo. Basenokungabi nazimpawu. Ukuba banazo, azibonakali kakhulu.
• Uhlobo 2: Kolu hlobo, i-von Willebrand factor ayisebenzi kakuhle. Aba bantu banokufumana ukopha okuncinci ukuya kokuphakathi. Phakathi kwe-15% kunye ne-30% yezigulana zinalolu hlobo.
• Uhlobo 3: Olu lolona hlobo lunzima kakhulu. Lukwanqabile kakhulu. Luchaphazela phakathi kwe-5% kunye ne-10% yezigulane. Aba bantu banamanqanaba aphantsi kakhulu e-von Willebrand factor egazini labo, okanye abanawo kwaphela. Oku kunokubangela iingxaki ezinkulu zokopha.

Ziziphi iindlela zonyango zesifo sikaVon Willebrand?

Oogqirha banyanga esi sifo ngeentlobo ezahlukeneyo zamayeza:

• (Desmopressin): Le yihormone. Yonyusa inqanaba le-von Willebrand factor egazini. Olu lolona nyango luqhelekileyo lwesi sifo.
• Ukufakwa kwe-Von Willebrand factor: Abanye abantu banikwa le factor ngemithambo yegazi ukuze bayeke ukopha. Olu nyango lunokunikwa ngaphambi kotyando. Abanye abantu abanesifo esinzima banokufuna ukufumana olu nyango rhoqo ukuze bagcine amanqanaba e-factor egazini labo ezinzile.
• (Ii-Antifibrinolytics):La mayeza asebenza ngokuthintela ukwakheka kwamahlwili egazi. Ugqirha wakho angakunika la mayeza ukuba wenza utyando lwamazinyo okanye ukuba unengxaki yokopha kakhulu xa usexesheni.
• Iipilisi zokulawula ukuzala: Ezi zinceda abo baphuma igazi elininzi ukuya exesheni. I-hormone ye-estrogen kwezi pilisi inyusa izinga le-von Willebrand factor egazini.

Ndingayinciphisa na ingozi yokuba nesi sifo?

Oku kudla ngokuba yimfuza . Ukuba abazali bakho banale sifo, ungasifumana komnye wabo okanye kubo bobabini. Ngoko ke akukho nto sinokuyenza ukuthintela ukuba singabikho.

Ukuba ndinayo le meko, ndimele ndilindele ntoni?

Oogqirha banokunyanga isifo se-von Willebrand, kodwa abanakusinyanga ngokupheleleyo . Uninzi lwabantu lunohlobo 1 okanye uhlobo 2. Bafuna unyango kuphela xa benomonzakalo okanye befuna utyando. Abantu abanohlobo 3 banokufuna unyango oluqhubekayo ukulawula ukopha.

Ndinesifo sikaVon Willebrand. Ndingazinyamekela njani ?

Ekubeni uninzi lwabantu luneempawu ezincinci ukuya kweziphakathi, ukuphila nesi sifo kuthetha ukuthathela ingqalelo izinto ezimbalwa:

• Ziphephe izinto ezinokubangela ukwenzakala: Kungcono ukuba ungabandakanyeki kwimidlalo echaphazela kakhulu, umzekelo, ibhola ekhatywayo, iragbi kunye nehoki.
• Xelela bonke oogqirha bakho, kuquka nogqirha wamazinyo, ukuba unale meko: emva koko banokuceba indlela yokulawula ukopha emva kotyando.
• Musa ukusebenzisa i-aspirin okanye amayeza ane-aspirin.
• Musa ukusebenzisa amayeza angengo-steroidal anti-inflammatory (NSAIDs) , afana ne-ibuprofen, ngaphandle kokuba unikwe ngugqirha owaziyo ukuba unesifo se-von Willebrand.
• Kuphephe ukuthatha izongezo zesondlo eziqulethe i-vitamin E, ioyile yentlanzi kunye ne-turmeric.
• Cinga ngesazisi sesilumkiso sezonyango: Ukunxiba isongo esifana nesi okanye ukuphatha ikhadi lesazisi kunokukunceda ufumane unyango olufanelekileyo xa kukho imeko engxamisekileyo.

Ndingaya nini esibhedlele ngokukhawuleza?

Yiya esibhedlele ngoko nangoko nanini na xa uphuma igazi ngendlela engalawulekiyo . Nokuba kukwenzakala okuncinci, ukuba ukopha akuyeki, qiniseka ukuba ucela ingcebiso kagqirha.

Ndingayibuza yiphi imibuzo ugqirha?

Ukuba unesifo se-von Willebrand, usenokuba nemibuzo malunga nendlela esi sifo esiza kuyichaphazela ngayo ubomi bakho. Nazi ezinye zemibuzo onokuzibuza ugqirha wakho:

• Kutheni ndifumene esi sifo?
• Ngaba abantwana bam banokufumana esi sifo njengelifa?
• Ngaba oku kuya kuba kubi ngakumbi?
• Zithini iindlela zonyango zoku?
• Zithini iziphumo ebezingalindelekanga zonyango?
• Ngaba andizukukwazi ukwenza izinto ezithile, ezinje ngokuhambahamba okanye ukudlala imidlalo, ngenxa yesi sifo?

Ngamafutshane, izinto ekufuneka sizikhumbule zezi:

Isifo sikaVon Willebrand sisifo esixhaphakileyo, esibangelwa lufuzo lokujiya kwegazi. Uninzi lwabantu luneempawu ezincinci ukuya kweziphakathi. Basenokuba nokopha rhoqo impumlo, okanye nokusikeka okuncinci kungathatha ixesha elide ukuyeka ukopha. Abanye baneempawu eziqatha. Umzekelo, banokuba nokopha emalungwini abo kunye nentlungu emalungwini.

Abanye abantu basenokungazi ukuba banale meko kangangeminyaka. Ukuba unayo, kusenokuba luncedo ukwazi ukuba kutheni uneengxaki zokopha. Usenokuba nexhala lokuba nabantwana bakho baza kuba nale meko. Nangona oogqirha bengenakuyinyanga le meko, banokuyinyanga . Banokukuphendula nemibuzo yakho malunga nokuba abantwana bakho baza kuyizuza na. Banokukunika nolwazi olufunekayo ukuze uphile nesifo sika-von Willebrand ukuze singakuthinteli ukuba uphile ubomi obuqhelekileyo. Ngoko ke, ungakhathazeki, ukuba uyayiqonda kwaye uyilawula kakuhle, ungaphila kakuhle nayo.