Ngaba wakha waba nokusikwa okuncinci okwathatha ixesha elide kunokuba bekulindelwe ukopha? Okanye wakha waba nokuphuma kwegazi empumlweni okanye imivumbo emzimbeni wakho wonke? Usenokuba ucinga, "Owu... ndinjalo kanye." Kodwa kusenokuba kukho isizathu sezonyango emva kwezi zinto ongazaziyo. Namhlanje siza kuthetha ngemeko echaphazela inkqubo yokujiya kwegazi, into abantu abaninzi abangazange bayive, kodwa ayiqhelekanga kangako. Yiyo loo nto i-von Willebrand Disease.
Ngamafutshane, yintoni isifo sikaVon Willebrand?
Isifo sikaVon Willebrand sisifo segazi esibangelwa yimfuza esichaphazela amandla egazi lethu okujiya ngokufanelekileyo.
Khawuthelekelele ukuba xa sifumana inxeba emzimbeni wethu, kukho iqela elincinci labasebenzi eliyekisa ukopha. Aba basebenzi banomphathi osebenzisanayo kwaye onceda ukuvala inxeba, njengeplasta. Loo "mphathi" yiproteni ebizwa ngokuba yi -von Willebrand factor (VWF) . Le protein ye-VWF yiyo enceda iiplatelets egazini ukuba zinamathele kunye kwaye zenze ihlwili legazi elivala inxeba.
Ngoku, kumntu onesifo se-von Willebrand, umzimba awuvelisi ngokwaneleyo le protein ye-VWF. Okanye iproteni eveliswayo ayisebenzi kakuhle. Ngenxa yokuba loo "mphathi" engekho, kuthatha ixesha elide ukuba iiplatelets zinamathelene kwaye zenze ihlwili legazi. Yiyo loo nto nenxeba elincinci lihlala liphuma igazi.
Ukuba le meko iba mandundu, ukopha kungavela ngaphakathi kwamalungu okanye kwizicubu ezithambileyo, nto leyo ebangela iintlungu ezinkulu kunye nokudumba. Kwabanye abantu, oku kunokukhokelela kwi- anemia , imeko apho kukho ukwehla komthamo wegazi emzimbeni.
Esi asisosifo esinokunyangeka ngokupheleleyo. Kodwa ungakhathazeki! Ugqirha wakho, ingakumbi ingcali ye-hematologist, angakunceda ulawule le meko kakuhle kwaye uphile ubomi obuqhelekileyo.
Ngaba kukho iintlobo eziphambili zesi sifo?
Ewe, isifo sikaVon Willebrand asifani kubo bonke abantu. Kukho iintlobo ezintathu eziphambili zaso. Ubukhali beempawu buyahluka ngokuxhomekeke kuhlobo lwaso.
| Uhlobo lwesifo | Inkcazo |
|---|---|
| Uhlobo 1 | Olu lolona hlobo luxhaphakileyo . Malunga nabantu abathathu kwabane abanesi sifo banalo olu hlobo. Kwimeko enjalo, amanqanaba e-VWF aphantsi, kodwa iimpawu zincinci kakhulu. Ngamanye amaxesha kusenokungabikho zimpawu kwaphela. |
| Uhlobo 2 | Kolu hlobo, umzimba uvelisa iproteni ye-VWF, kodwa ayisebenzi kakuhle. Oko kukuthi, nangona inomphathi, ayazi ukuba ingenza njani umsebenzi wayo. Oku kunokubangela ukopha okuncinci ukuya kokuphakathi. |
| Uhlobo 3 | Olu lolona hlobo lunqabileyo nolona lunzima . Kwimeko enjalo, inqanaba leprotheyini ye-VWF emzimbeni liphantsi kakhulu, ngamanye amaxesha alikho. Oku kukubeka emngciphekweni wokopha kakhulu. |
Zithini iimpawu zesifo sikaVon Willebrand?
Kwabaninzi abantu, ingakumbi abo bane-Type 1, iimpawu azikho mbi kangako. Basenokuphila bengazi nokuba banesi sifo. Nangona kunjalo, kwiimeko ezinzima, ezi mpawu zilandelayo zinokwenzeka:
- Ukopha rhoqo kwempumlo: Ukuba impumlo yakho iyopha nokuba yinto encinci kwaye kunzima ukuyiyeka.
- Ukopha ixesha elide kwinxeba elincinci: Ukuba inxeba okanye ukukrweleka kopha ngaphezulu kwemizuzu eli-10.
- Ukugruzuka lula: Ukuba ufumana imivumbo emikhulu, evuvukileyo nokuba emva kokudumba okuncinci.
- Ukopha kakhulu exesheni kwabasetyhini: Ukuba wopha kakhulu kunesiqhelo ngexesha lokuya esikhathini. Umzekelo, ukuba kufuneka utshintshe ii-pad zakho ngaphambi kokuphela kweyure okanye ezimbini, ukuba uphuma amahlwili egazi amakhulu, okanye ukuba wopha ngaphezu kweentsuku ezisi-7, kufuneka uxhalabe.
- Ukopha kakhulu emva kotyando: Ukuba wopha kakhulu kunesiqhelo, nokuba emva kwento elula njengokukhupha izinyo.
- Ukopha kakhulu emva kokubeleka okanye ukuphuma kwesisu.
- Igazi elikwindle okanye emchameni.
Kutheni esi sifo sisenzeka? Sifunyanwa njani njengelifa?
Isifo sikaVon Willebrand sisifo semfuza . Ngamafutshane, sibangelwa sisiphene kwijini esilawula ukuveliswa kweproteni yeVWF. Esi siphene sifunyanwa ngabazali bethu njengelifa.
Kukho iindlela ezimbini zokufumana oku njengelifa:
1. Ilifa elilawulayo le-Autosomal: Oku kwenzeka kumama okanye kutata.Nokuba ngumntu omnye kuphela ofumana ilifa le-gene enesiphene, esi sifo sinokukhula. Uhlobo 1 kunye nohlobo 2 zihlala zizuzwa ngale ndlela.
2. Ilifa le-Autosomal recessive: Kwimeko enjalo, ukuze baphuhlise esi sifo, bobabini abazali kufuneka bazuze ilifa le-gene enesiphene. Uhlobo lwe-3, uhlobo olubi kakhulu, ludla ngokuzuza ilifa ngale ndlela.
Amaxesha amaninzi, ezinye iintlobo zomhlaza, izifo ezibangelwa kukuzikhusela komzimba, kunye nesifo sentliziyo nazo zinokubangela le meko kamva ebomini. Ibizwa ngokuba yi -acquired von Willebrand syndrome .
Ugqirha uxilonga njani esi sifo?
Xa uxelela ugqirha wakho ngeempawu zakho, uza kuqala akuhlole aze akhangele iimpawu zokukrweleka okanye ezinye iimpawu zokopha. Emva koko baza kubuza ukuba kukho nabani na kusapho lwakho okhe waba nale ngxaki yokopha.
Kufuneka uvavanyo lwegazi oluninzi ukuze kufunyaniswe isifo ngokuchanekileyo.
- Ukubalwa kwegazi okupheleleyo (CBC): Oku kujonga inani leeseli ezibomvu, iiseli ezimhlophe, kunye neeplatelets egazini lakho. Ukuba inani leeplatelets zakho liphantsi, okanye ukuba i-hemoglobin yakho iphantsi ngenxa ye-anemia, le meko inokukrokrelwa.
- Uvavanyo lokuqunjelwa: Olu luvavanyo olulinganisa ixesha elithathayo ukuba igazi lakho liqunje.
- Uvavanyo lwe-von Willebrand factor (VWF): Olu vavanyo lukhethekileyo lujonga inqanaba leproteni ye-VWF egazini lakho kunye nokuba isebenza kakuhle na.
- Uvavanyo lweFactor VIII: Iproteni yeVWF ikhusela iFactor VIII, enye iproteni ebalulekileyo enceda ukujiya kwegazi. Ngoko ke xa iVWF iphantsi, amanqanaba eFactor VIII nawo anokuba phantsi. Olu vavanyo lukhangela loo nto.
Zithini iindlela zonyango zoku?
Unyango luxhomekeke kuhlobo lwesifo onaso kunye nobunzima beempawu zakho. Uninzi lwabantu lufuna unyango ngaphambi kotyando okanye emva kokwenzakala.
Kukho iindlela ezininzi eziphambili zonyango:
- I-Desmopressin (DDAVP): Olu lolona nyango lusetyenziswa kakhulu. Yihomoni. Inikwa njenge-spray yempumlo okanye njenge-injection. Isebenza ngokukhawulezisa ukukhutshwa kweeproteni ze-VWF ezigcinwe emizimbeni yethu egazini.
- Ukufakwa kwe-von Willebrand factor: Kwiimeko ezinzima, uhlobo oluxineneyo lwe-VWF lunikwa umzimba ngenaliti. Oku kufana nokunika umzimba iproteni ye-VWF ephelileyo.
- Amayeza okulwa nefibrinolytics: La mayeza asebenza ngokuthintela ukuqhekeka kwegazi ekunyibilikeni ngokukhawuleza. Anikwa ukulawula ukopha kwiimeko ezifana nokukhupha amazinyo.
- Iipilisi zokulawula ukukhulelwa: Ezi ziluncedo kubafazi abopha kakhulu ukuya exesheni. Ihomoni ye-estrogen kwezi pilisi inceda ukonyusa amanqanaba e-VWF.
Ndifanele ndimbone nini ugqirha?
Ukuba wopha lula, ukruquka lula, okanye unexesha elininzi lokuya exesheni, qiniseka ukuba ubonana nogqirha wakho wosapho. Kunokubakho ezinye izizathu zokopha kakhulu, ngoko ke kubalulekile ukuba ufumane uxilongo olufanelekileyo.
Eyona nto ibalulekileyo: Nanini na xa ungakwazi ukulawula ukopha, yiya kwiYunithi yoNyango oluNgxamisekileyo (ETU) yesibhedlele ngoko nangoko.
Ndingenza ntoni xa ndiphila nesi sifo?
Ukuba nesifo sikaVon Willebrand kuthetha ukuba kufuneka ulumke ngakumbi ukuze uthintele ukopha. Ugqirha wakho uza kukucebisa ngale nto. Ngokubanzi, kungcono ukuphepha oku kulandelayo:
- Ziphephe imidlalo enokubangela ukwenzakala: Imidlalo ebandakanya ukudibana kakhulu, efana neragbi kunye nebhokisi, ayifanelekanga.
- Kuphephe ukusebenzisa amayeza okunciphisa igazi: Musa ukusebenzisa amayeza okudambisa iintlungu afana ne -aspirin okanye ii-NSAID (umz., i-Ibuprofen, i-Diclofenac) ngaphandle kwemvume kagqirha wakho.
- Kuphephe ukuthatha izongezo ezithile: Izinto ezifana ne-vitamin E kunye ne-oyile yeentlanzi zinokunciphisa igazi. Buza ugqirha wakho ngaphambi kokuba usebenzise nantoni na.
Xelela bonke oogqirha bakho (kuquka nogqirha wamazinyo) ukuba unale meko ukuze bakwazi ukuthatha amanyathelo okulawula ukopha kwegazi ngaphambi kokuba benze nantoni na, njengotyando. Kukwangumbono olungileyo ukunxiba isongo sesilumkiso sezonyango ukuze uxelele abasebenzi bezonyango xa kukho imeko engxamisekileyo.
Umyalezo Wokuya Ekhaya
- Isifo sikaVon Willebrand sisifo esibangelwa kukungaqini kwegazi esibangelwa kukungabikho okanye ukungasebenzi kakuhle kweproteni ebizwa ngokuba yi-von Willebrand factor (VWF).
- Uhlobo oluqhelekileyo (Uhlobo 1) luneempawu ezincinci kakhulu kwaye lusenokungabonakalisi zimpawu konke konke.
- Ukopha rhoqo impumlo, ukudumba okulula, ukuya exesheni kakhulu, kunye nokopha ixesha elide ngenxa yamanxeba zezona mpawu ziphambili.
- Nangona ingenakunyangwa ngokupheleleyo, inokulawulwa kakuhle ngonyango olufana neDesmopressin, i-VWF infusions, kunye notshintsho kwindlela yokuphila.
- Ukuba unale mpawu, ungathandabuzi ukubona ugqirha wakho uze wenze uvavanyo olufunekayo. Ukuxilongwa ngokuchanekileyo kunye nolawulo zezona zinto zibalulekileyo.
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