So mogya pii nso tu fi wo kuru ketewaa bi mu? Momma yɛnka Bernard-Soulier Syndrome (BSS) ho asɛm!

So woahyɛ no nsow pɛn sɛ mogya tu nnipa binom pii fi akuru ketewaa bi anaa akuru ketewaa bi mpo mu, anaasɛ akuru a ɛyɛ mmerɛw na egye bere tenten ansa na ayɛ yiye? Ɛtɔ da bi a eyi betumi afi tebea bi a yɛntee ho asɛm pii, nanso ɛho hia yiye sɛ yehu ho asɛm. Tebea a ɛte saa no biako ne Bernard-Soulier Syndrome, anaa ``(BSS)`` tiawa. Momma yɛnka eyi ho asɛm nkɔ akyiri kakra nnɛ.

Dɛn ne Bernard-Soulier Yare (BSS)?

Sɛ yɛbɛka no tiawa a, Bernard-Soulier Syndrome `(BSS)` yɛ tebea a ɛntaa nsi koraa. Nea ɛkɔ so wɔ eyi mu ne sɛ, ɔkwan a mogya tu wɔ yɛn nipadua mu a ɛkyerɛ sɛ mogya no gyae bere a yɛapira no, sɛe. Fa no sɛ, wowɔ kuru ketewaa bi wɔ wo nsa so. Bere bi akyi no, mogya no gyae, ɛnte saa? Wiɛ, nea ɛboa ma ɛno ne mogya nkwammoaa a ɛyɛ nkwammoaa ketewaa bi a ɛwɔ yɛn mogya mu, ne plasma a ɛyɛ mogya no fã a ɛyɛ nsu no.

Obi a ɔwɔ Bernard-Soulier Syndrome (BSS) no mogya mu nkwammoaa a ɛma mogya tu no sua sen sɛnea ɛte daa. Nnuruyɛfo frɛ tebea yi thrombocytopenia. Afei nso, mogya nkwammoaa a ɛwɔ hɔ no sõ sen mogya nkwammoaa a ɛteɛ. Enti, esiane saa nneɛma yi nti, nnipa a wɔwɔ BSS no mogya tu na ɛnyɛ den sɛ wɔbɛpirapira.

Tebea yi ho yɛ na dɛn?

Eyi yɛ tebea a ɛntaa nsi koraa, yiye ankasa. Sɛnea aduruyɛ ho abenfo kyerɛ no, saa tebea yi ka nnipa ɔpepem biako biara mu biako pɛ wɔ wiase nyinaa. Eyi kyerɛ sɛ wɔ Sri Lanka mpo no, nnipa kakraa bi pɛ na wɔyare saa yare yi.

Dɛn ne Bernard-Soulier Syndrome (BSS) ho sɛnkyerɛnne ahorow?

Mpɛn pii no, nkokoaa a wɔwɔ BSS no fi ase da saa sɛnkyerɛnne ahorow yi adi wɔ wɔn nkwa nna mu. Hwɛ sɛ wo anaa obi a wunim no wɔ saa sɛnkyerɛnne yi bi anaa:

• Ɛnyɛ den sɛ wobɛpirapira, na egye bere tenten ansa na ayɛ yiye: Sɛ wobɔ nkongua so kɛkɛ a, ebetumi ama woanya akuru kɛse. Ebetumi agye adapɛn pii ansa na saa akuru no asa.
• Mogya a ɛtɔ kɛse mpo esiane ntwitwiridii nketenkete ne apirakuru nti: Sɛ́ ebia wutwa wo nsa ketewaa bi bere a worebɔ wo ti nhwi anaasɛ wode sekan twitwa wo nsa bere a woretwitwa nhabannuru no, ɛyɛ den sɛ wubegyae mogya no.
• Mogya a ɛtaa tu fi hwene mu: Nnipa binom frɛ eyi `(epistaxis)`. Sɛ mogya taa tu wo hwene mu a ntease biara nnim a, ɛno nso yɛ sɛnkyerɛnne.
• Mogya a ɛtɔ wɔ honam ani: Eyinom da adi sɛ nsensanee nketenkete kɔkɔɔ anaa kɔkɔɔ wɔ honam ani. Nnuruyɛfo frɛ eyinom ``petechiae''.
• Mogya a ɛtɔ kɛse bere a mmea rekɔ brayɛ mu: Sɛ mogya tu no kyɛ nna pii sen sɛnea ɛtaa ba, anaasɛ ɛyɛ kɛse (menorrhagia) a, ɛno nso yɛ asɛm a ɛhaw adwene.

Nsuro BSS esiane sɛ wowɔ saa sɛnkyerɛnne ahorow yi mu biako anaa abien nti. Nanso sɛ saa sɛnkyerɛnne ahorow yi kɔ so a, ɛbɛyɛ papa sɛ wobɛhwehwɛ aduruyɛfo afotu.

Dɛn na ɛde Bernard-Soulier Syndrome (BSS) ba?

Eyi ne asɛm a ɛho hia sen biara. `(BSS)` yɛ awosu mu yare.. Ɛne sɛ, tebea bi a ɛba bere a wɔawo yɛn esiane nsakrae (mutation) a ɛba yɛn awosu mu nkwaadɔm mu nti. Eyi yɛ awosu , a ɛkyerɛ sɛ mmofra betumi anya awosu mu nsakrae yi afi wɔn awofo hɔ.

Saa tebea yi (BSS) fi nkwaadɔm mu nsakrae a ɛba awosu mu nkwaadɔm `GP1BA`, `GP1BB` anaa `GP9` titiriw mu. Saa awosu mu nkwaadɔm yi nyinaa ho hia yiye ma mogya a ɛtwetwe wɔ yɛn nipadua mu no. Enti sɛ ɔhaw bi wɔ saa awosu mu nkwaadɔm yi ho a, mogya a ɛtwetwe no nkɔ yiye.

Wobɛyɛ dɛn ahu sɛ wowɔ Bernard-Soulier Syndrome (BSS) anaa?

Sɛ wowɔ saa sɛnkyerɛnne ahorow yi a, ade a oduruyɛfo bɛyɛ bere a wuhu no ne sɛ obebisa wo asɛm bi a ɛkɔ akyiri. Afei, wɔbɛhyɛ sɛ wɔnyɛ mogya mu nhwehwɛmu mpɛn pii na ama wɔahu tebea no.

• Mprɛte dodow ho nhwehwɛmu: Eyi hwɛ mogya mu nkwammoaa dodow a ɛwɔ wo mogya mu ne sɛ ebia ɛsua anaa.
• Awosu mu nkwaadɔm mu nsakrae sɔhwɛ: Eyi betumi ahu sɛ ebia nkwaadɔm mu nsakrae bi wɔ `GP1BA`, `GP1BB`, `GP9` awosu mu nkwaadɔm a yɛadi kan aka ho asɛm no mu a ɛde `(BSS)` ba.
• Nkwammoaa mu nkwammoaa nketenkete mu nhwehwɛmu: Wɔfa wo mogya no bi na wɔde afiri ketewaa bi hwehwɛ mu hwɛ sɛ mogya nkwammoaa no sõ wɔ ɔkwan a ɛnteɛ so anaa.
• Platelet flow cytometry: Ɛwom sɛ ɛwɔ din a ɛyɛ den de, nanso saa sɔhwɛ yi susuw glycoproteins dodow a wɔfrɛ no `GP1BA`, `GP1BB`, `GP9` wɔ mogya nkwammoaa ani. Sɛ eyinom sua a, ɛyɛ `(BSS)` ho sɛnkyerɛnne.

Egyina nea efi nhwehwɛmu ahorow yi mu ba so na oduruyɛfo no bɛkyerɛ wo pɛpɛɛpɛ sɛ ebia wowɔ BSS anaasɛ wunni bi.

Dɛn ne ayaresa a wɔde sa Bernard-Soulier Syndrome (BSS)?

Mprempren wonni aduru biara a wɔde sa BSS, efisɛ ɛyɛ awosu mu tebea. Nanso, nneɛma pii wɔ hɔ a wubetumi ayɛ de asiw mogya mmoroso ano na woama nsɛnnennen afoforo a ebetumi aba no so atew. Ayaresa no twe adwene si ɛno so titiriw.

Nneɛma a wubetumi ayɛ na ama mogya a ɛbɛtɔ wo no so atew:

Eyinom yɛ nneɛma a ɛnyɛ den, nanso ɛho hia yiye a wubetumi de aka wo da biara da asetra ho.

• Kwati nnuru a ɛma mogya tu no yɛ kɛse: Nnuru bi a wɔde kum ɛyaw te sɛ nnuru a ekum ɔyare mmoawa a ɛnyɛ steroid (NSAIDs), aspirin, ne nnuru bi a ekum akisikuru betumi ama mogya a ɛbɛtɔ obi no ayɛ kɛse. Enti, nnom nnuru yi a wonkɔ oduruyɛfo nkyɛn.
• Fa brɔs a ɛyɛ mmerɛw di dwuma bere a worehohoro wo sẽ no: Eyi betumi atew mogya a ɛretu wo sẽ so.
• Sua ɔkwan pa a wobɛfa so asiw mogya a ɛba wo hwene mu ano: bisa wo duruyɛfo sɛnea wobɛkura wo hwene mu ne sɛnea wobɛkura wo ti mu.
• To nnuan ne anonne bi ano hye: Nnuan a nsa ne asikre a wɔayam pii wom betumi aka wo mogya nkwammoaa dodow, enti ɛbɛyɛ papa sɛ wobɛto ano hye sɛnea wubetumi biara.
• Kyerɛw wo din wɔ beae bi a wɔde hwɛ a egye ntɛmpɛ ma nnɔnhwerew 24: Eyi bɛboa wo ma woanya ayaresa ntɛm wɔ tebea a egye ntɛmpɛ te sɛ mogya a ɛtɔ kɛse mu.
• Fa iron a wɔde ma sɛ wo ho nni iron a: Mogya a wotaa tu no betumi ama woanya iron a enni wo mu. Fa dade aduru a wɔde hyɛ nipadua mu sɛnea wo duruyɛfo kyerɛ no.
• Hyɛ aduruyɛ mu abɔsoɔ anaa tag: Ɛho hia paa wɔ ntɛmpɛ mu sɛ wobɛhyɛ abɔsoɔ anaa tag a ɛkyerɛ pefee sɛ wowɔ `(BSS)`.

Aduruyɛ mu ayaresa:

Wɔ tebea horow bi mu, titiriw wɔ mogya tu mpofirim anaasɛ bere a wɔresiesie wɔn ho ama oprehyɛn no, nnuruyɛfo betumi de ayaresa te sɛ:

• Nkwammoaa mu nkwammoaa a wɔde ma: Mpɛn pii no, eyi ne ayaresa a edi kan a wɔde sa mogya a egye ntɛmpɛ. Wɔnam IV nhama so de mogya nkwammoaa a efi obi a ɔde ma no mu kɔ wo nipadua mu. Saa mogya nkwammoaa yi boa ma wo mogya tu.
• Antifibrinolytic therapy: Eyinom yɛ nnuru a ɛboa ma mogya tu. Sɛ nhwɛsoɔ no, tranexamic acid, a ebia woate ho asɛm no nso wɔ hɔ wɔ din ``Cyklokapron®'' ase. Eyi boa ma wodi mogya a ɛba no so.

So wobetumi asiw Bernard-Soulier Syndrome (BSS) ano?

Nea ɛyɛ awerɛhow no, ɔkwan biara nni hɔ a wɔbɛfa so asiw BSS ano efisɛ ɛyɛ awosu mu tebea.

Nanso, sɛ obi wɔ w’abusua mu wɔ BSS, anaasɛ wususuw sɛ ebia wowɔ saa awosu mu nkwaadɔm mu nsakrae yi a, ebia wobɛpɛ sɛ wususuw awosu mu nhwehwɛmu ho . Ɛho hia nso sɛ wohwehwɛ awosu mu afotu . Awosu ho ɔfotufo betumi aka tebea no ho asɛm pii akyerɛ wo na wakyerɛkyerɛ asiane a ɛwɔ hɔ sɛ wode bɛma wo mma no mu.

Nneɛma bɛn na ɛsɛ sɛ wohwɛ yiye bere a wo ne Bernard-Soulier Syndrome (BSS) te no?

Nnipa dodow no ara a wɔwɔ BSS no betumi atra ase sɛnea ɛsɛ. Nanso, ɛsɛ sɛ wɔhwɛ yiye kakra na ama asiane a ɛwɔ hɔ sɛ wobepirapira no so atew.

Sɛ nhwɛso no, eye sɛ wobɛtwe wo ho afi agumadi a wɔde di nkitaho ho, efisɛ ɛde asiane kɛse ba sɛ wubepira na mogya atɔ wo kɛse.

Ɛho hia nso sɛ wobɛkɔ wo ɔyarehwɛfo titiriw nkyɛn daa na ama w’akwahosan nyinaa akɔ so atra hɔ. Sɛ wɔyɛ wo oprehyɛn a, ebia ɛho behia sɛ wudi kan yɛ ho nhyehyɛe na ebia wɔma wo mogya mu nkwammoaa kɔkɔɔ ansa na wɔayɛ oprehyɛn no.

Sɛ wowɔ Bernard-Soulier Syndrome (BSS) a, nsɛm bɛn na ɛsɛ sɛ wubisa wo duruyɛfo?

Sɛ wususuw sɛ woanya (BSS), anaasɛ wɔahu sɛ wowɔ bi a, ɛbɛyɛ mfaso kɛse sɛ wubebisa wo duruyɛfo nsɛm te sɛ eyinom:

• Dɛn ne Bernard-Soulier Syndrome ho sɛnkyerɛnne a edi kan?
• Nhwehwɛmu bɛn na ɛsɛ sɛ meyɛ na ama mahu sɛ ebia mewɔ Bernard-Soulier Syndrome anaa?
• Dɛn ne ayaresa a wobetumi apaw ama Bernard-Soulier Syndrome?
• Sɛ mewɔ Bernard-Soulier Syndrome a, dɛn na metumi ayɛ na amma mogya mmoroso?
• Dwumadi ahorow bɛn na ɛnsɛ sɛ meyɛ esiane sɛ mewɔ Bernard-Soulier Syndrome nti?
• Hokwan bɛn na ɛwɔ hɔ sɛ me mma benya Bernard-Soulier Syndrome afi me hɔ?

Wɔ nsɛmmisa yi akyi no, nsuro sɛ wo ne oduruyɛfo no bɛkasa afa biribiara a ɛba w’adwenem ho.

So Bernard-Soulier Syndrome (BSS) a obi benya bere a ɔyem no yɛ asiane ma akokoaa no?

Sɛ woanyinsɛn na wowɔ BSS a, wubehia ayaresa titiriw (awo ne mogya ho ayaresa a asiane kɛse wom) . Ɛho behia sɛ wɔhwɛ wo mogya nkwammoaa dodow ne w’akwahosan nyinaa so daa. Bere a worewo no, ebia worentumi mfa nnuru ahorow bi a ɛma mogya tu no yɛ kɛse.

Bere a wɔawo akokoaa no akyi mpo no, ɛsɛ sɛ wɔhwɛ sɛnea akokoaa no mogya mu nkwammoaa kɔkɔɔ dodow te. Efisɛ hokwan wɔ hɔ sɛ wo ba no betumi anya awosu mu nsakrae a ɛde ``(BSS)'' ba no afi wo hɔ. Enti, ɛho hia sɛ wo ne wo duruyɛfo kasa fa eyi ho na woyɛ nneɛma a ɛho hia.

Dɛn ne ɔhaw titiriw a Bernard-Soulier Syndrome (BSS) de ba?

Ne tiawa mu no, ɔhaw titiriw a ``BSS'' de ba ne mogya a entumi nkɔ mu . Eyi nti na mogya a ɛtɔ dodo ne akisikuru a ɛnyɛ den ne sɛnkyerɛnne a ɛtaa ba no.

Tebea afoforo bɛn na ɛde sɛnkyerɛnne a ɛte sɛ Bernard-Soulier Syndrome (BSS) ba?

Tebea afoforo pii wɔ hɔ a ɛde mogyatɔw ho haw ne sɛnkyerɛnne ahorow a ɛte sɛ BSS ba. Ɛyɛ papa sɛ wubehu eyinom din nkutoo:

• `Hemophilia`
• `May-Hegglin asɛm a ɛnteɛ`
• `Storage pool yareɛ`
• `Glanzmann ntini a ɛma mogya tu`
• `Von Willebrand yareɛ`

Oduruyɛfo nkutoo na obetumi agyina wo yare no ho sɛnkyerɛnne so ahu nyarewa yi mu nea ɛyɛ pɛpɛɛpɛ.

Yɛn nsɛm a etwa to

Bernard-Soulier Syndrome `(BSS)` yɛ tebea a ɛntaa nsi koraa a wonya fi w’awofo hɔ. Sɛ wowɔ saa tebea yi a, ebia wubehyia ɔhaw ahorow bi wɔ mogya a ɛbɛtɔ ho. Nanso, ɛnkyerɛ sɛ wuntumi ntra ase sɛnea ɛsɛ. Ade a ɛho hia sen biara ne sɛ wobɛkwati tebea horow a asiane wom a ebetumi ama woapirapira na wudi wo duruyɛfo afotu akyi bere nyinaa.

Kɔ w’abusua oduruyɛfo nkyɛn daa. Obetumi abua wo nsɛmmisa, aboa wo ma woadi tebea yi ho dwuma, na ɔne wo ayɛ adwuma de akura w’akwahosan nyinaa mu. Mma nsuro, nhumu ne ɔhwɛ yɛ yɛn ahoɔden kɛse bere a yɛne saa tebea horow yi te ase no.

` Bernard-Soulier Syndrome, BSS, mogya a ɛtwetwe, mogya nkwammoaa a ɛma mogya tu, mogya a ɛtɔ dodo, awosu mu nyarewa, akuru