Yu dɔn ɛva gɛt smɔl kɔt we tek lɔng tɛm pas aw yu bin de tink fɔ mek blɔd kɔmɔt? Ɔ yu dɔn ɛva gɛt blɔd na yu nos ɔ brus ɔlsay na yu bɔdi? Yu kin de tink se, "Oh... na so a bi." Bɔt sɔntɛm wan mɛrɛsin rizin de biɛn dɛn tin ya we yu nɔ no bɔt. Tide wi go tɔk bɔt wan sik we de afɛkt di we aw blɔd de klɔt, wan we bɔku pipul dɛn nɔ ivin yɛri bɔt, bɔt nɔto so rare. Dat na von Willebrand Sik.
Fɔ tɔk am simpul wan, wetin na di sik we dɛn kɔl von Willebrand?
von Willebrand sik na wan jenɛtik blɔd disɔda we de afɛkt di ebul we wi blɔd ebul fɔ klɔt fayn fayn wan.
Imajin se we wi gɛt wund na wi bɔdi, na wan smɔl tim we de wok de we de stɔp di blɔd. Dɛn wokman dɛn ya kin wok togɛda ɛn ɛp fɔ lɔk di wund, lɛk plasta. dat "manija" na wan protin we dεn kכl von Willebrand fכktכ (VWF) . dis VWF protin na in de εp di pletlεt dεm na di bכdi fכ stik tכgeda εn fכ mek bכdi kכlכt we de sial di wund.
Naw, pan pɔsin we gɛt von Willebrand sik, di bɔdi nɔ de prodyuz inof pan dis VWF protin. Ɔ di prɔtin we dɛn mek nɔ de wok fayn. so biכs da "manija" de nכ de, i kin tek lכng tεm fכ di pletlεt dεm fכ stik togeda εn fכm bכdi kכlכt. Na dat mek ivin smɔl wund kin kɔntinyu fɔ blɔd.
If dis kכndyushכn kam tranga, bכdi kin kכmכt insay di joyn dεm כ di sכft tisu dεm, we kin mek yu fil bad bad wan εn swel. Na sɔm pipul dɛn, dis kin mek bak dɛn gɛt anemia , we na wan sik we de mek di blɔd we de na di bɔdi nɔ bɔku.
Dis nɔto sik we pɔsin kin mɛn ɔltogɛda. Bɔt nɔ wɔri! Yu dɔktɔ, mɔ di dɔktɔ we de mɛn yu blɔd, kin ɛp yu fɔ kɔntrol dis sik fayn fayn wan ɛn liv nɔmal layf.
Yu tink se men kayn dis sik de?
Yɛs, von Willebrand sik nɔto di sem fɔ ɔlman. Tri men kayn we de fɔ am. Di kayn we aw di sik kin tranga kin difrɛn difrɛn wan fɔ di kayn sik.
| Di kayn sik we pɔsin kin gɛt | Tɔk bɔt |
|---|---|
| Tayp 1 | Dis na di kayn we we dɛn kin yuz mɔ . Na lɛk tri pan ɛvri 4 pipul dɛm wae gɛt dis sik kin gɛt dis kayn. Insay dis kayn we, di VWF lɛvɛl nɔ kin bɔku, bɔt di sayn dɛn kin rili smɔl. Sɔntɛnde, nɔr kin gɛt ɛni sayn fɔ de atɔl. |
| Tayp 2 we de na di wɔl | insay dis kayn we, di bכdi de mek di VWF protin, bכt i nכ de wok fayn. Dat min se, pan ɔl we i gɛt "manija," i nɔ no aw fɔ du in wok. Dis kin mek yu blɔd kɔmɔt smɔl to smɔl. |
| Tayp 3 we yu de du | Dis na di kayn we we nɔ kin bɔku ɛn we kin rili bad . insay dis kes, di lεvεl fכ VWF protin na di bכdi de rili lכw, sכmtεm i ivin nכ de. Dis kin put yu pan denja fɔ mek yu gɛt siriɔs blɔd. |
Wetin na di sayn dɛm fɔ di sik we dɛn kɔl von Willebrand?
Fɔ bɔrku pipul dɛm, mɔr di wan dɛm wae gɛt Tayp 1, de sik nɔr kin so bad. Dɛn kin liv ɛn nɔr ivin no se dɛn gɛt dis sik. Bɔt if i siriɔs, dɛn sayn ya kin apin:
- Blɔd we de kɔmɔt na yu nos ɔltɛm: If yu nos de blɔd frɔm ivin smɔl tin ɛn i at fɔ stɔp.
- Blɔd we de kɔmɔt fɔ lɔng tɛm frɔm smɔl wund: If yu kɔt ɔ skrap blɔd fɔ pas 10 minit.
- Brus izi: If yu gɛt big big brus we swel ivin afta yu dɔn gɛt smɔl bɔmp.
- Ebi blɔd we de kɔmɔt na uman dɛn we yu de gɛt mɔnt: If yu blɔd pas aw i fɔ blɔd we yu de gɛt mɔnt. Fɔ ɛgzampul, if yu fɔ chenj yu pad bifo wan ɔ tu awa dɔn, if yu pas big big blɔd, ɔ if yu blɔd fɔ pas 7 dez, yu fɔ wɔri.
- Blɔd we de kɔmɔt pasmak afta dɛn dɔn du di ɔpreshɔn: If yu blɔd pas aw i fɔ blɔd, ivin afta sɔntin we simpul lɛk fɔ pul yu tut.
- Bɔku blɔd we de kɔmɔt afta yu bɔn pikin ɔ we di bɛlɛ dɔn pwɛl.
- Blɔd we de na di stɔl ɔ di urine.
Wetin mek dis sik kin apin? Aw dɛn kin pas am fɔ lɔng lɔng tɛm?
von Willebrand sik na wan sik we de mek pɔsin gɛt jɛnɛtiks . Fɔ tɔk am simpul wan, i kin kɔmɔt frɔm wan difrɛns na di jin we de kɔntrol di prodakshɔn fɔ di VWF protin. Wi kin gɛt dis jin we nɔ fayn frɔm wi mama ɛn papa.
Tu we dɛn de fɔ gɛt dis:
1. Autosomal dominant inheritance: dis kin apin fr כm di mama כ di papa.Ivin if na wan pɔsin nɔmɔ gɛt di jin we nɔ fayn, di sik kin kam. Bɔku tɛm, dɛn kin gɛt tayp 1 ɛn Tayp 2 dis we.
2. Autosomal recessive inheritance: insay dis kes, fכ mek di sik go divεlכp, dεn tu mama εn papa fכ gεt di jin we nכ de wok fayn. Tayp 3, di kayn we we kin rili bad, na in dɛn kin gɛt dis we.
Nɔr kin ɔltɛm, sɔm kansa, ɔtoimyun disɔda, ɛn at sik kin kam bak wit dis sik leta na layf. Dɛn kɔl am akwyayz von Willebrand sindrom .
Aw dɔktɔ kin no bɔt dis sik?
We yu tɛl yu dɔktɔ bɔt yu sik, i go fɔs chɛk yu ɛn luk fɔ sayn dɛn we de sho se yu gɛt brus ɔ ɔda sayn dɛn we de sho se yu de blɔd. Dɔn dɛn go aks if ɛnibɔdi na yu famili dɔn gɛt dis kayn prɔblɛm wit blɔd.
Dɛn nid fɔ du bɔku blɔd tɛst fɔ no di sik kɔrɛkt wan.
- Kɔmplit blɔd kɔnt (CBC): Dis kin chɛk di nɔmba fɔ di rɛd sɛl dɛn, wayt sɛl dɛn, ɛn di pletlɛt dɛn we de na yu blɔd. If yu pletlɛt nɔ bɔku, ɔ if yu ɛmoglobin nɔ bɔku bikɔs yu nɔ gɛt blɔd, yu kin tink se yu gɛt dis sik.
- Test fɔ mek yu blɔd klɔt: Dis na tɛst dɛn we de sho aw lɔng i tek fɔ mek yu blɔd klɔt.
- von Willebrand factor (VWF) test: Dɛn spɛshal tɛst ya de chɛk di lɛvɛl we di VWF protin de na yu blɔd ɛn if i de wok fayn fayn wan.
- Fכktכ VIII tεst dεm: di VWF protin de protεkt Factor VIII, כda imכtant protin we de εp fכ blכd kכlכt. So we VWF smɔl, Factor VIII lɛvɛl dɛn kin smɔl bak. Dis tɛst de luk fɔ dat.
Wetin na di tritmɛnt dɛm fɔ dis?
Di tritmɛnt de dipen pan di kayn sik we yu gɛt ɛn aw yu de fil bad. Bɔku pipul dɛn kin jɔs nid tritmɛnt bifo dɛn du dɛn ɔpreshɔn ɔ afta dɛn dɔn wund.
Bɔku men we dɛn de fɔ trit pɔsin:
- Desmopressin (DDAVP): Dis na di tritmɛnt we dɛn kin yuz mɔ. Na wan ɔmon. Dɛn kin gi am as sprɛy na in nos ɔ as injɛkshɔn. i de wok bay we i de mek di VWF protin dεm we dεn stכr na wi bכdi kכmכt kwik kwik wan insay di bכdi.
- von Willebrand factor infusions: insay siriכs kes dεm, dεn de gi wan kכnsantreyt fכm fכ VWF to di bכdi tru injεkshכn. dis tan lεk fכ gi di bכdi di VWF protin we dεn dכn dכn.
- Antifibrinolytics: Dɛn mɛrɛsin ya kin wok bay we dɛn kin stɔp wan blɔd we de klɔt fɔ mek i nɔ sɔlv kwik kwik wan. Dɛn kin gi dɛn fɔ kɔntrol di blɔd we de kɔmɔt pan tin dɛn lɛk fɔ pul di tut.
- Pils fɔ kɔntrol bɔn pikin: Dɛn tin ya kin fayn fɔ uman dɛn we gɛt ebi ebi blɔd we dɛn kin gɛt we dɛn de gɛt mɔnt. di εstrojen כmon we de insay dεn pils ya de εp fכ inkrεs di VWF lεvεl.
Ustɛm a fɔ go to dɔktɔ?
If yu blɔd izi, brus izi, ɔ yu gɛt ebi ebi prɛgnɛshɔn, mek shɔ se yu go to yu famili dɔktɔ. Ɔda tin kin de we kin mek yu blɔd bɔku, so i impɔtant fɔ mek yu no di rayt tin fɔ no if yu gɛt di sik.
Di tin we impɔtant pas ɔl: Ɛni tɛm we yu nɔ ebul fɔ kɔntrol di blɔd we de kɔmɔt, go na di ɔspitul in Imejɛnsi Tritmɛnt Yunit (ETU) wantɛm wantɛm.
Wetin a kin du we a de liv wit dis sik?
If yu gɛt von Willebrand sik, dat min se yu nid fɔ tek tɛm smɔl fɔ mek yu nɔ gɛt blɔd. Yu dɔktɔ go advays yu bɔt dis. Jɛnɛral wan, i bɛtɛ fɔ avɔyd dɛn tin ya:
- Nɔ du spɔt dɛn we kin mek pɔsin wund: Spɔt dɛn we gɛt fɔ du wit bɔku kɔntakt, lɛk rɔgbi ɛn bɔksin, nɔ fayn.
- Nɔ tek tin dɛn we de mek yu blɔd tan: Nɔ tek tin dɛn we de mek yu fil pen lɛk aspirin ɔ NSAID (e.g., Ibuprofen, Diclofenac) if yu dɔktɔ nɔ gri fɔ yu.
- Nɔ tek sɔm sɔpɔtmɛnt dɛn: Tin dɛn lɛk vaytamɛn E ɛn fish ɔyl kin mek di blɔd tan. Aks yu dɔktɔ bifo yu yuz ɛnitin.
Tɛl ɔl yu dɔktɔ dɛm (inklud yu dɛntist) se yu gɛt dis sik so dat dɛn go tek step fɔ kɔntrol blɔd bifo dɛn du ɛnitin, lɛk ɔpreshɔn. I fayn bak fɔ wɛr mɛdikal alɛt breslɛt fɔ wɔn di mɛdikal wokman dɛn if ɛnitin apin.
Mɛsej we dɛn kin kɛr go na os
- Von Willebrand sik na wan sik we pɔsin kin gɛt we i gɛt blɔd we de klɔt we pɔsin kin gɛt we i nɔ de wok fayn ɔ we wan prɔtin we dɛn kɔl von Willebrand factor (VWF) nɔ de wok fayn.
- Di kayn we we kɔmɔn pas ɔl (Tayp 1) gɛt siriɔs sik dɛn we nɔ kin rili at ɛn i nɔ kin sho ɛni sayn atɔl.
- blɔd we de kɔmɔt na yu nos ɔltɛm, we i izi fɔ brus, we yu kin gɛt bɔku bɔku mɔnt, ɛn we yu kin gɛt blɔd fɔ lɔng tɛm bikɔs yu gɛt wund na di men sayn dɛm.
- Pan ɔl we dɛn nɔ kin ebul fɔ mɛn am ɔl, dɛn kin ebul fɔ mɛn am fayn fayn wan wit tritmɛnt dɛn lɛk Desmopressin, VWF infusions, ɛn chenj dɛn layf.
- If yu gɛt dɛn sik ya, nɔ shem fɔ go to yu dɔktɔ ɛn du di tɛst dɛn we yu nid. Kɔrɛkt diagnosis ɛn manejmɛnt na di tin dɛn we impɔtant pas ɔl.
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