Yu dɔn ɛva notis se sɔm pipul dɛn kin blɔd bɔku bɔku wan ivin we dɛn skrach smɔl ɔ we dɛn gɛt simpul brus, ɔ we kin brus izi wan ɛn tek lɔng tɛm fɔ wɛl? Sɔmtɛm dis kin bi bikɔs ɔf wan kɔndishɔn wae wi nɔr dɔn yɛri bɔrku bɔt, bɔt i rili impɔtant fɔ no bɔt. Wan pan dɛn kayn tin ya na Bernard-Soulier Syndrome, ɔ ``(BSS)`` fɔ shɔt. Lɛ wi tɔk bɔt dis smɔl mɔ tide.
Wetin na di sik we dɛn kɔl Bernard-Soulier Syndrome (BSS)?
Fɔ tɔk am simpul wan, Bernard-Soulier Syndrome `(BSS)` na wan sik we nɔ kin apin so ɔltɛm. Wetin kin apin pan dis na dat di prɔses we blɔd kin klɔt na wi bɔdi, we min se di blɔd kin stɔp we wi wund, kin ambɔg. Imajin, yu gɛt smɔl wund na yu an. Afta sɔm tɛm, di blɔd kin stɔp, nɔto so? Wɛl, wetin de ɛp fɔ du dat na di pletlɛt, we na wan kayn smɔl sɛl na wi blɔd, ɛn plasma, we na di wata we de na di blɔd.
Pɔsin we gɛt di sik we dɛn kɔl Bernard-Soulier Syndrome (BSS) nɔ kin gɛt bɛtɛ pletlɛt pas aw i kin gɛt. Dɔktɔ dɛn kɔl dis kɔndishɔn trombocytopenia. Dɔn bak, di pletlɛt dɛn we de de big pas di nɔmal pletlɛt dɛn. So, fɔ dɛn rizin ya, pipul dɛn we gɛt BSS kin blɔd ɛn brus izi wan.
Aw dis sik nɔ kin apin so ɔltɛm?
Dis na rili wan sik we nɔ kin apin so ɔltɛm. Masta sabi bukman dɛn we sabi bɔt mɛrɛsin tɔk se dis sik de ambɔg wan pan ɛvri wan milyɔn pipul dɛn ɔlsay na di wɔl. Dis min se ivin na Sri Lanka, na smɔl pipul dɛn nɔmɔ de sɔfa wit dis sik.
Wetin na di sayn dɛm wae de sho se pɔrsin gɛt Bernard-Soulier Syndrome (BSS)?
Bɔku tɛm, pikin dɛn we gɛt BSS kin bigin fɔ sho dɛn sik ya we dɛn dɔn bigin fɔ liv. Chek fɔ si if yu ɔ pɔsin we yu sabi gɛt ɛni wan pan dɛn sik ya:
- Yu kin brus izi wan, ɛn i kin tek lɔng tɛm fɔ mek i wɛl: Ivin wan simpul bɔmp na chia kin lɛf big brus. I kin tek sɔm wiks fɔ mek da brus de wɛl.
- Blɔd bɔku ivin frɔm smɔl smɔl kɔt ɛn wund dɛn: Ilɛksɛf yu kɔt smɔl we yu de sheb ɔ kɔt yu an wit nɛf we yu de kɔt vɛjitebul, i nɔ kin izi fɔ stɔp di blɔd.
- Nɔs blɔd kin kɔmɔt ɔltɛm: Sɔm pipul dɛn kin kɔl dis `(epistaxis)`. If yu kin gɛt blɔd na yu nos ɔltɛm fɔ natin, dat na sayn bak.
- Blɔd we de kɔmɔt ɔnda di skin: Dɛn tin ya kin tan lɛk smɔl smɔl rɛd ɔ pepul dɔt dɛn na di skin. Dɔktɔ dɛn kin kɔl dɛn tin ya ``petechiae''.
- Ebi blɔd we de kɔmɔt we uman dɛn de gɛt mɔnt: If di blɔd de te fɔ sɔm dez pas aw i kin bi, ɔ if i ebi (menorrhagia), dat bak na sɔntin we fɔ wɔri bɔt.
Nɔr frayd BSS jɔs bikɔs yu gɛt wan ɔ tu pan dɛn sik ya. Bɔt if dɛn sik ya kɔntinyu, i go fayn fɔ mek yu go to dɔktɔ.
Wetin kin mek pɔsin gɛt di sik we dɛn kɔl Bernard-Soulier Syndrome (BSS)?
Dis na di pɔynt we impɔtant pas ɔl. `(BSS)` na wan jenɛtik disɔda.. dat min se, wan kכndyushכn we de apin we dεn bכn wi biכs fכ chenj (mכtεshכn) na wi jin dεm. Dis na tin we pɔsin kin gɛt frɔm in mama ɛn papa , we min se pikin dɛn kin gɛt dis chenj we dɛn de chenj dɛn jɛnɛtiks frɔm dɛn mama ɛn papa.
dis kכndishכn (BSS) de kכz fכ mכtεshכn dεm na di jin dεm `GP1BA`, `GP1BB` כ `GP9` patikyular. Ɔl dɛn jin ya rili impɔtant fɔ mek blɔd klɔt na wi bɔdi. So we prɔblɛm de wit dɛn jin ya, blɔd nɔ kin klɔt fayn fayn wan.
Aw yu go no if yu gɛt Bernard-Soulier Syndrome (BSS)?
If yu gɛt dɛn sik ya, di fɔs tin we dɔktɔ go du we yu si dɛn na fɔ aks yu ditayli kwɛstyɔn. Dɔn, dɛn go ɔda fɔ mek dɛn du bɔku blɔd tɛst fɔ no if pɔsin gɛt di sik.
- Plɛtlɛt kɔnt tɛst: Dis kin chɛk di nɔmba fɔ di pletlɛt dɛn we de na yu blɔd ɛn if dɛn smɔl.
- jin mכtεshכn tεst: dis kin no if mכtεshכn de insay di `GP1BA`, `GP1BB`, `GP9` jin dεm we wi bin dכn tכk bכt we de mek `(BSS)`.
- Maykroskɔp fɔ chɛk di pletlɛt dɛn: Dɛn kin tek wan sɛmpul pan yu blɔd ɛn chɛk am ɔnda maykroskɔp fɔ si if di pletlɛt dɛn nɔ big.
- di pletlet fכ fכlכ saytכmetri: pan כl we i gεt komplikεt nem, dis tεst de mכsu di amoun fכ di glycoprotein dεm we dεn kכl `GP1BA`, `GP1BB`, `GP9` na di sεf fכ di pletlet dεm. If dɛn tin ya smɔl, na sayn fɔ `(BSS)`.
Na di rizɔlt fɔ dɛn tɛst ya di dɔktɔ go tɛl yu klia wan if yu gɛt BSS ɔ yu nɔ gɛt am.
Wetin na di tritmɛnt fɔ di Bernard-Soulier Syndrome (BSS)?
Naw, no mɛrɛsin nɔ de fɔ BSS, bikɔs na jenɛtik kɔndishɔn. Bɔt bɔku tin dɛn de we yu kin du fɔ mek yu nɔ gɛt bɔku blɔd ɛn fɔ mek yu nɔ gɛt ɔda prɔblɛm dɛn we kin apin. Di tritmɛnt kin pe atɛnshɔn mɔ pan dat.
Tin dɛn we yu kin du fɔ mek yu nɔ gɛt bɔku blɔd:
Dɛn tin ya simpul, bɔt dɛn rili impɔtant we yu kin ad pan yu layf ɛvride.
- Nɔ tek mɛrɛsin dɛn we de mek yu blɔd bɔku: Sɔm mɛrɛsin dɛn we de mek yu blɔd, lɛk nonsteroidal anti-inflammatory drugs (NSAID), aspirin, ɛn sɔm antihistamines kin mek yu gɛt mɔ blɔd. So, nɔ tek dɛn mɛrɛsin ya we yu nɔ go to dɔktɔ.
- Yuz tut brɔsh we gɛt sɔft brist we yu de brus yu tit: Dis kin mek yu gɔm nɔ gɛt blɔd.
- Lan di kɔrɛkt we fɔ stɔp blɔd na yu nos: aks yu dɔktɔ bɔt aw fɔ ol yu nos ɛn aw fɔ ol yu ed.
- Limit sɔm it ɛn drink dɛn: Fɔ it we gɛt bɔku rɔm ɛn rifin shuga kin afɛkt yu pletlɛt kɔnt, so i bɛtɛ fɔ lɛ yu stɔp dɛn as yu ebul.
- Rɛjista na wan sɛnta we de gi yu 24 awa imejensi kia: Dis go ɛp yu fɔ gɛt tritmɛnt kwik kwik wan pan imejensi, lɛk we yu gɛt big blɔd.
- Tek ayɔn sɔpɔtmɛnt if yu nɔ gɛt ayɔn: If yu blɔd bɔku tɛm, dat kin mek yu nɔ gɛt ayɔn. Tek ayɛn tablɛt dɛn lɛk aw yu dɔktɔ tɛl yu.
- Wear mɛdikal aydentifikeshɔn breslɛt ɔ tag: I rili impɔtant we yu gɛt imejensi fɔ wɛr breslɛt ɔ tag we rayt klia wan se yu gɛt `(BSS)`.
Di tritmɛnt we dɛn kin gi fɔ mɛn pipul dɛn:
Sɔntɛnde, mɔ we blɔd kɔmɔt wantɛm wantɛm ɔ we dɛn de rɛdi fɔ ɔpreshɔn, dɔktɔ dɛn kin gi dɛn tritmɛnt lɛk:
- Plɛtlɛt transfyushɔn: Bɔku tɛm, dis na di fɔs tritmɛnt fɔ blɔd we pɔsin kin gɛt kwik kwik wan. di pletlet dεm frכm wan dכna de gi yu insay yu bכdi tru wan IV layn. Dɛn pletlɛt ya kin ɛp fɔ mek yu blɔd klɔt.
- Antifibrinolytic therapy: Dis na mɛrɛsin wae de ɛp fɔ mek blɔd klɔt. fכ egzampl, tranexamic acid, we yu kin yeri bכt, de bak כnda di brand nem ``Cyklokapron®''. Dis kin ɛp fɔ kɔntrol di blɔd we de kɔmɔt.
Yu tink se dɛn kin ebul fɔ avɔyd di sik we dɛn kɔl Bernard-Soulier Syndrome (BSS)?
כnכfכs, no we nכ de fכ mek BSS nכ de divεlכp biכs na jεnεtik kכndishכn.
Bɔt if pɔsin na yu famili gɛt BSS, ɔ if yu tink se yu go gɛt dis jin muteshon, yu kin tink bɔt fɔ tɛst yu jɛnɛtiks . I impɔtant bak fɔ go fɛn advays bɔt yu jɛnɛtiks . Wan pɔsin we de advays yu bɔt di jɛnɛtiks kin tɛl yu mɔ bɔt di sik ɛn ɛksplen di prɔblɛm we kin apin we yu pas am to yu pikin dɛn.
Wetin na sɔm tin dɛm wae yu fɔ no bɔt wae yu de liv wit Bernard-Soulier Syndrome (BSS)?
Bɔrku pipul dɛm wae gɛt BSS kin liv nɔrmal layf. Bɔt dɛn nid fɔ tek tɛm smɔl fɔ mek dɛn nɔ gɛt bɔku bad bad aksidɛnt.
Fɔ ɛgzampul, i bɛtɛ fɔ mek yu nɔ de nia kɔntakt spɔt, bikɔs dɛn kin gɛt mɔ risk fɔ injuri ɛn fɔ mek yu blɔd bɔku.
I impɔtant bak fɔ go to yu praymari kia ɔltɛm fɔ mek yu gɛt ɔl yu wɛlbɔdi. If dɛn du yu ɔpreshɔn, i go nid fɔ mek yu du am bifo tɛm ɛn sɔntɛm dɛn go gi yu pletlɛt bifo dɛn du di ɔpreshɔn.
If yu gɛt Bernard-Soulier Syndrome (BSS), us kwɛstyɔn dɛn yu fɔ aks yu dɔktɔ?
If yu tink se yu gɛt (BSS) ɔ dɛn dɔn no se yu gɛt am, i go fayn fɔ aks yu dɔktɔ kwɛstyɔn dɛn lɛk dɛn wan ya:
- Wetin na di fɔs sayn dɛm wae de sho se yu gɛt Bernard-Soulier Syndrome?
- Us tɛst dɛn a fɔ du fɔ no if a gɛt di sik we dɛn kɔl Bernard-Soulier Syndrome?
- Wetin na de tritmɛnt opshɔn fɔ Bernard-Soulier Syndrome?
- If a gɛt Bernard-Soulier Syndrome, wetin a go du fɔ mek a nɔ blɔd pasmak?
- Us tin dɛn we a nɔ fɔ du bikɔs a gɛt di sik we dɛn kɔl Bernard-Soulier Syndrome?
- Wetin na di chans fɔ mek mi pikin dɛn gɛt Bernard-Soulier Syndrome frɔm mi?
Apat frɔm dɛn kwɛstyɔn ya, nɔ fred fɔ tɔk to di dɔktɔ bɔt ɛnitin we kam na yu maynd.
Yu tink se we yu gɛt Bɛnard-Sɔli Sindrom (BSS) we yu gɛt bɛlɛ, dat kin mek di pikin gɛt prɔblɛm?
If yu gɛt bɛlɛ ɛn yu gɛt BSS, yu go nid spɛshal mɛdikal kia (high-risk obstetric and hematology care) . Yu go nid fɔ de wach yu pletlɛt kɔnt ɛn ɔl yu wɛlbɔdi ɔltɛm. We yu de bɔn pikin, yu nɔ go ebul fɔ gɛt sɔm kayn anestezi we go mek yu blɔd bɔku.
Ivin afta we dɛn bɔn di pikin, dɛn fɔ de chɛk di pikin in pletlɛt ɔltɛm. biכs chans de fכ mek yu pikin gεt di jεnεtik mכtεshכn we de mek ``(BSS)'' frכm yu. So, i impɔtant fɔ tɔk to yu dɔktɔ bɔt dis ɛn tek di step dɛn we yu nid.
Wetin na di men prɔblɛm wae de kam wit Bernard-Soulier Syndrome (BSS)?
in shot, di men prכblεm we ``BSS'' de kכz na di inability fכ di bכdi fכ kכlכt . Dis na di rizin we mek we pɔsin de blɔd pasmak ɛn we i izi fɔ brus na di sayn dɛn we kin apin mɔ.
Us ɔda tin dɛn kin mek yu gɛt sayn dɛn we fiba di Bernard-Soulier Syndrome (BSS)?
Bɔku ɔda tin dɛn de we kin mek pɔsin gɛt prɔblɛm wit blɔd ɛn simptom dɛn we fiba BSS. I fayn fɔ no dɛn tin ya bay dɛn nem nɔmɔ:
- `Ɛmofilia`
- `May-Hegglin anomaly`
- `Stɔrej pul sik`
- `Glanzmann trombastɛnia`
- `Von Willebrand sik`
Bay di sayn dɛm we yu gɛt, na dɔktɔ nɔmɔ go ebul fɔ no uswan pan dɛn sik ya.
Wi las wɔd dɛn
Bernard-Soulier Syndrome `(BSS)` na wan sik wae nɔr kin bɔrku wae yu mama ɛn papa kin gɛt. If yu gɛt dis sik, yu kin gɛt sɔm prɔblɛm wit blɔd we de klɔt. Bɔt, i nɔ min se yu nɔ go ebul fɔ liv nɔmal layf. Di tin we impɔtant pas ɔl na fɔ avɔyd tin dɛn we go mek yu wund ɛn fala yu dɔktɔ in advays ɔltɛm.
Si yu famili dɔktɔ ɔltɛm. I kin ansa yu kwɛstyɔn dɛn, ɛp yu fɔ mɛn dis sik, ɛn wok wit yu fɔ mek yu gɛt wɛlbɔdi. Nɔr frayd, aw fɔ no ɛn kia na wi big tin wae wi kin gɛt wae wi de liv wit dɛn kayn tin ya.
` Bernard-Soulier Syndrome, BSS, blɔd we de klɔt, pletlɛt, blɔd we de kɔmɔt pasmak, jɛnɛtik sik, brus











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