Ngaba wakha waba nenxeba elincinci ongazange uliyeke ukopha ixesha elide? Okanye ufumana imivumbo emikhulu eluhlaza okwesibhakabhaka emva kokudumba kancinci? Ngamanye amaxesha sicinga ukuba ezi zizinto eziqhelekileyo, kodwa zinokuba luphawu lwengxaki encinci egazini lethu. Namhlanje, siza kuthetha ngemeko engathethwa kangako kodwa eqhelekileyo echaphazela ukujiya kwegazi. Leyo sisifo sika-von Willebrand.
Ngamafutshane, yintoni isifo se-von Willebrand (VWD)?
Isifo sikaVon Willebrand (VWD) sisifo esenzeka xa inkqubo yethu yokuqunjelwa kwegazi ingasebenzi kakuhle. Sibubuthathaka obuzuzwe njengelifa emizimbeni yethu kunokuba sisifo.
Khawuthelekelele, xa sinqunyulwe kwenye yemithambo yethu yegazi, uhlobo oluncinci lweeseli (iiplatelets) emzimbeni wethu luyeza ukunqanda ukopha kwaye luvale inxeba. Kufana nokugcwalisa umngxuma eludongeni ngezitena. Ezi platelets zinamathelana kwaye zineproteni ekhethekileyo efana "nentsini" egazini lethu enceda ukuba zenze ihlwili elinamandla. Yiloo nto siyibiza ngokuba yi -von Willebrand factor (VWF) .
Umntu one-VWD usenokungabi nala protein ye-VWF eyaneleyo emzimbeni wakhe, okanye iprotein anayo isenokungasebenzi kakuhle. Emva koko, ngenxa yokuba ihlwili legazi alikheki kakuhle, kuthatha ixesha elide ukuyeka ukopha nokuba kukwenzakala okuncinci.
Le yeyona ngxaki yokopha exhaphakileyo emhlabeni. Kuqikelelwa ukuba umntu omnye kwabayi-100 unokuba nale ngxaki.
Ziziphi iintlobo eziphambili ze-VWD?
Le meko yahlulwe kakhulu yaba ziintlobo ezintathu. Kukwakho nohlobo olungazalwa nalo kodwa oluvela kamva.
| Uhlobo | Inkcazo |
|---|---|
| Uhlobo 1 | Olu lolona hlobo luxhaphakileyo (phakathi kwama-60% - 80%). Lubangelwa kukuba umzimba uneproteni encinci kakhulu ye-VWF. Iimpawu zihlala zincinci. |
| Uhlobo 2 | Uhlobo lwesibini oluxhaphakileyo (phakathi kwe-15% - 30%). Nangona iprotheyini ye-VWF ikhona apha, umsebenzi wayo ubuthathaka. Ifana ne-old gum, ayisebenzi nokuba ikhona. Iimpawu zinokuba ncinci ukuya kwephakathi. |
| Uhlobo 3 | Olu lolona hlobo lunzima nolunqabileyo. Apha, iprotheyini ye-VWF phantse ayikho kwaphela emzimbeni. Ke ngoko, iimpawu zinzima kakhulu. |
| I-VWD efunyenweyo | Oku akuyonto ozalwa nayo. Ingakhula kamva ngenxa yezifo ezithile zokuzikhusela komzimba (umz. i-lupus), isifo sentliziyo, ezinye iintlobo zomhlaza, okanye amayeza owasebenzisayo. |
Zithini iimpawu zale meko?
Iimpawu ziyahluka ngokuxhomekeke kuhlobo lwe-VWD onayo.
Iimpawu ezibuthathaka neziphakathi zeentlobo 1 kunye ne-2
- Nokuba ukwenzakala okuncinci kunokubangela ukudumba okukhulu okuluhlaza okwesibhakabhaka: Ngaba uyaqaphela ukuba umzimba wakho ujika ube luhlaza okwesibhakabhaka kwiindawo ezithile?
- Ukopha rhoqo kwempumlo: Abanye abantu bakopha rhoqo kwempumlo, kwaye kunzima ukuyeka.
- Ukopha kakhulu emva kotyando okanye ukukhupha izinyo: Ngaba ukopha akupheli emva kweeyure emva kokukhupha izinyo?
- Abafazi bafumana ukopha kakhulu ngexesha lokuya exesheni: Ngaba ukopha kunzima kangangokuba kufuneka utshintshe iphedi yakho ubuncinane kanye ngeyure? Ngaba kuza namahlwili? Ekuhambeni kwexesha, oku kunokukhokelela kwi- anemia yokungabikho kwesinyithi.
- Igazi elikwindle okanye emchameni.
Iimpawu ezinzima zohlobo lwesithathu
Kolu hlobo, zonke ezi mpawu zingasentla zinokuba nzima kakhulu. Ukongeza,
- Ukopha ngequbuliso, okuqatha ngaphandle kwesizathu.
- Igazi livuza emalungeni nakwizicwili ezithambileyo, nto leyo ebangela ukudumba kunye nentlungu eqatha.
Into ebalulekileyo kukuba ayinguye wonke umntu onezi mpawu one-VWD. Kodwa ukuba unenye okanye ngaphezulu kwezi mpawu rhoqo, kungcono ukubona ugqirha ukuze akunike ingcebiso.
Uyibona njani le nto?
Ugqirha wakho uza kuqala akubuze ngeempawu zakho kunye nembali yezonyango zosapho lwakho. Ngenxa yokuba le yimeko yelifa, kubalulekile ukufumanisa ukuba kukho na omnye umntu kusapho lwakho okhe waba nale ngxaki yokopha.
Emva koko, baya kwenza uvavanyo olukhethekileyo ukuze babone ukuba kuthatha ixesha elingakanani ukuba igazi lakho lijiye kwaye lijiye kakuhle kangakanani.
- Ukulinganisa ubungakanani beprotheyini ye-VWF egazini (uvavanyo lwe-Antigen).
- Ukuvavanya umsebenzi waloo protein.
Ngamanye amaxesha, izinto ezinje ngoxinzelelo kunye nokuzilolonga zinokutshintsha amanqanaba e-VWF, ngoko ke kunokufuneka ukuba olu vavanyo lwenziwe izihlandlo ezingaphezu kwesinye ukuze ufumane iziphumo ezichanekileyo.
Zithini iindlela zonyango? Indlela yokulawula?
Nangona i-VWD ingenakunyangwa, inokulawulwa kwaye ilawulwe ukuze iphile ubomi obuqhelekileyo. Eyona nto ibalulekileyo kukunciphisa umngcipheko wokopha.
1. Ukuphepha amayeza athile
Kufuneka uphephe ukusebenzisa amayeza athile anciphisa igazi. Ugqirha wakho uya kukuxelela rhoqo ukuba ungayisebenzisi i-aspirin kunye ne -NSAIDs (ezifana ne-ibuprofen kunye ne-naproxen). I-Acetaminophen lukhetho olukhuselekileyo lwentlungu okanye umkhuhlane. Kodwa qiniseka ukuba ubuza ugqirha wakho ngaphambi kokuba usebenzise naliphi na iyeza.
2. Unyango oluthile
Unyango luxhomekeke kubukhali kunye nohlobo lweempawu zakho.
- I-Desmopressin Acetate (DDAVP): Olu lolona nyango lusetyenziswa kakhulu. Lunokunikwa njenge-spray yempumlo okanye njenge-injection. Lusebenza ngokuvuselela ukukhululwa kweprotheyini ye-VWF egcinwe kwiiseli zethu egazini. Eli yeza linokubangela ukugcinwa kwamanzi emzimbeni, ngoko ugqirha wakho unokucebisa ukuba unciphise ukungenisa kwakho ulwelo.
- I-Clotting Factor Concentrates: I-concentrate equlethe i-VWF protein enikwa nge-intravenously (IV). Le idla ngokusetyenziswa kwiimeko ezinzima nangaphambi kotyando.
- Amanye amayeza: Kwiimeko ezifana nokukhupha amazinyo, iipilisi okanye amayeza olwelo afana ne-aminocaproic acid okanye i-tranexamic acid anokunikwa ukuthintela ukuqhekeka kwegazi ekunyibilikeni.
- Unyango olukhethekileyo lwabasetyhini: Kwabasetyhini abanengxaki yokuya exesheni kakhulu, iipilisi zokulawula ukukhulelwa zinokunyusa amanqanaba e-VWF. Kukwakho neendlela ezifana nokunxiba iluphu equlethe iihomoni (isixhobo se-levonorgestrel intrauterine) okanye ukususa ulwelo lwesibeleko (i-endometrial ablation).
Okokugqibela, ukuba unemeko embi efana nohlobo lwesithathu, ukuba ufumana ukopha, yimeko engxamisekileyo. Kufuneka uye kwiSebe lezeMpilo eziNgxamisekileyo (ETU) esibhedlele ngoko nangoko. Ukulibaziseka kunokuba yingozi ebomini.
Umyalezo Wokuya Ekhaya
- Isifo sikaVon Willebrand (VWD) sisifo esiqhelekileyo, esizuzwe njengelifa esichaphazela inkqubo yokujiya kwegazi.
- Ukuba uneempawu ezifana nokugruzuka rhoqo, ukopha impumlo, okanye ukopha kakhulu exesheni, xhalaba.
- Ukuba unale mpawu, ungoyiki ukubona ugqirha ofanelekileyo kwaye ucele ingcebiso.
- Ukuba ufunyaniswe une-VWD, kuphephe ukuthatha i-aspirin okanye i-NSAID ngokupheleleyo ngaphandle kwengcebiso kagqirha.
- Ngolawulo olufanelekileyo nonyango, ungaphila ubomi obusempilweni nobuqhelekileyo xa unesi sifo.
💬 Comments (0)
Akukho zimvo zithunyelweyo okwangoku. Faka uluvo lwakho apha okokuqala.
Faka uluvo lwakho