So woatwitwa ketewaa bi a egyee bere tenten sen sɛnea na wohwɛ kwan sɛ mogya tu wo pɛn? Anaasɛ wo hwene mu mogya anaa wo nipadua nyinaa akuru pɛn? Ebia wobɛdwene sɛ, "Oh... saa ara na mete." Nanso ebia aduruyɛ mu ntease bi wɔ saa nneɛma yi akyi a wunnim ho hwee. Ɛnnɛ yɛbɛka tebea bi a ɛka mogya a ɛtwetwe no ho asɛm, nea nnipa pii nte ho asɛm mpo, nanso ɛntaa nsi saa. Ɛno ne von Willebrand Yare no.
Sɛ yɛbɛka no tiawa a, dɛn ne von Willebrand yare?
von Willebrand yare yɛ awosu mu mogya mu yare a ɛka tumi a yɛn mogya tumi tu yiye.
Fa no sɛ, sɛ yenya kuru wɔ yɛn nipadua mu a, adwumayɛfo kuw ketewaa bi wɔ hɔ a wogyae mogya no. Saa adwumayɛfo yi bom yɛ adwuma na wɔboa ma wɔto kuru no mu, te sɛ plaster. Saa "ɔhwɛfo" no yɛ protein bi a wɔfrɛ no von Willebrand factor (VWF) . Saa VWF protein yi na ɛboa ma mogya nkwammoaa a ɛwɔ mogya no mu no bata ho na ɛyɛ mogya a ɛtɔ a ɛkata kuru no so.
Mprempren, wɔ obi a ɔwɔ von Willebrand yare mu no, nipadua no ntumi nyɛ saa VWF protein yi sɛnea ɛsɛ. Anaasɛ protein a wɔyɛ no ntumi nyɛ adwuma yiye. Enti esiane sɛ saa "adwuma sohwɛfo" no nni hɔ nti, egye bere tenten ansa na mogya nkwammoaa a ɛwɔ nipadua no mu no abata ho na ayɛ mogya a ɛtɔ. Ɛno nti na kuru ketewaa mpo kɔ so tu mogya no.
Sɛ tebea yi mu yɛ den a, mogya betumi atu wɔ nkwaa anaa ntini a ɛyɛ mmerɛw no mu, na ɛde ɛyaw a emu yɛ den ne ɔfe aba. Wɔ nnipa binom fam no, eyi nso betumi ama mogya a ɛtɔ kɔ nipadua no mu , tebea a ɛma mogya dodow a ɛwɔ nipadua no mu no so tew.
Eyi nyɛ yare a wotumi sa koraa. Nanso mma ɛnhaw wo! Wo duruyɛfo, titiriw mogya ho ɔbenfo betumi aboa wo ma woadi tebea yi ho dwuma yiye na woabɔ bra sɛnea ɛsɛ.
So yare yi ahorow atitiriw bi wɔ hɔ?
Yiw, von Willebrand yare no nyɛ ade koro mma obiara. Ɛwɔ ahorow atitiriw abiɛsa. Ɛsono sɛnea sɛnkyerɛnne ahorow no mu yɛ den no gyina ɔkwan a wɔfa so yɛ no so.
| Yare no su | Nkyerɛmu |
|---|---|
| Ɔkwan a wɔfa so yɛ 1 | Eyi ne ɔkwan a ɛtaa ba . Nnipa baanan biara a wɔwɔ yare no mu bɛyɛ baasa na wɔwɔ saa yare yi. Wɔ eyi mu no, VWF dodow no sua, nanso yare no ho sɛnkyerɛnne no nyɛ den koraa. Ɛtɔ mmere bi a, ebia yare no ho sɛnkyerɛnne biara nni hɔ koraa. |
| Ɔkwan a wɔfa so yɛ 2 | Wɔ saa su yi mu no, nipadua no na ɛyɛ VWF protein no, nanso ɛnyɛ adwuma yiye. Ɛne sɛ, ɛwom sɛ ɛwɔ "adwuma sohwɛfo" de, nanso onnim sɛnea ɛbɛyɛ n'adwuma. Eyi betumi ama mogya atɔ no kakra kosi nea ɛkɔ fam. |
| Ɔkwan a wɔfa so yɛ 3 | Eyi ne nea ɛntaa nsi na emu yɛ den sen biara . Wɔ eyi mu no, VWF protein dodow a ɛwɔ nipadua no mu no sua koraa, ɛtɔ mmere bi mpo a enni hɔ. Eyi de wo to asiane mu sɛ mogya bɛtɔ wo kɛse. |
Dɛn ne von Willebrand yare no ho sɛnkyerɛnne?
Wɔ nnipa pii fam, titiriw wɔn a wɔwɔ Type 1 no, yare no ho sɛnkyerɛnne no mu nyɛ den saa. Ebia wɔbɛtra ase a wonnim mpo sɛ wɔwɔ yare no. Nanso, wɔ tebea horow a emu yɛ den mu no, sɛnkyerɛnne ahorow a edidi so yi betumi aba:
- Mogya a ɛtaa tu fi hwene mu: Sɛ wo hwene mu mogya fi ade ketewaa bi mpo mu na ɛyɛ den sɛ wubegyae a.
- Mogya a ɛtɔ bere tenten fi kuru ketewaa bi mu: Sɛ nea wɔatwitwa anaa wɔatwitwa no mogya tu bɛboro simma 10 a.
- Akuru a ɛnyɛ den: Sɛ wunya akuru akɛse a ɛho ahonhon bere a woabɔ akuturuku ketewaa bi mpo akyi a.
- Mogya a ɛtɔ kɛse wɔ mmea mu wɔ brayɛ mu: Sɛ mogya tu wo sen sɛnea ɛte daa wɔ wo brayɛ mu a. Sɛ nhwɛso no, sɛ ɛsɛ sɛ wosesa wo pads ansa na dɔnhwerew biako anaa abien aba awiei, sɛ wotwam mogya akɛse, anaasɛ mogya tu wo bɛboro nnafua 7 a, ɛsɛ sɛ ɛhaw wo.
- Mogya a ɛtɔ dodo wɔ oprehyɛn akyi: Sɛ mogya tu wo sen sɛnea ɛte daa a, wɔ biribi a ɛnyɛ den te sɛ wo sẽ a woyi fi mu akyi mpo.
- Mogya a ɛtɔ kɛse wɔ awo akyi anaasɛ nyinsɛn a atu agu.
- Mogya a ɛwɔ nsu mu anaa nsu mu.
Dɛn nti na saa yare yi ba? Ɔkwan bɛn so na wɔde fa awo ntoatoaso ahorow mu?
von Willebrand yare no yɛ awosu mu yare . Sɛ yɛbɛka no tiawa a, efi sintɔ bi a ɛwɔ awosu mu abɔde a ɛhwɛ VWF protein no a wɔyɛ so no mu. Yenya saa awosu mu abɔde a asɛe yi fi yɛn awofo hɔ.
Akwan abien na ɛwɔ hɔ a wobɛfa so anya eyi agyapade:
1. Autosomal dominant inheritance: Eyi fi ɛna anaa agya no hɔ.Sɛ onipa biako pɛ na onya awosu mu nkwaadɔm a asɛe no mpo a, yare no betumi aba. Wɔtaa nya Type 1 ne Type 2 fi awo mu wɔ saa kwan yi so.
2. Autosomal recessive inheritance: Wɔ eyi mu no, sɛ awofo baanu no nyinaa betumi anya yare no a, ɛsɛ sɛ wonya awosu mu abɔde a asɛe no. Ɔkwan yi so na wɔtaa nya Type 3, ɔkwan a emu yɛ den sen biara no fi awo mu.
Ɛntaa mma sɛ kokoram ahorow bi, nipadua no mu tumi a ɛko tia nyarewa, ne komayare nso betumi de tebea yi aba akyiri yi wɔ nkwa nna mu. Wɔfrɛ no von Willebrand yare a wonya .
Ɔkwan bɛn so na oduruyɛfo hu yare yi?
Sɛ woka wo yare no ho sɛnkyerɛnne kyerɛ wo duruyɛfo a, obedi kan ahwɛ wo na wahwehwɛ sɛnkyerɛnne ahorow a ɛkyerɛ sɛ woapirapira anaa sɛnkyerɛnne afoforo a ɛkyerɛ sɛ mogya retu wo. Afei wobebisa sɛ ebia obi wɔ w’abusua mu a wanya mogyatɔw ho haw a ɛte sɛɛ anaa.
Ɛsɛ sɛ wɔyɛ mogya mu nhwehwɛmu pii na ama wɔahu yare no pɛpɛɛpɛ.
- Mogya dodow a edi mũ (CBC): Eyi hwɛ sɛnea nkwammoaa kɔkɔɔ, nkwammoaa fitaa, ne mogya mu nkwammoaa a ɛwɔ wo mogya mu no dodow te. Sɛ wo mogya nkwammoaa dodow sua, anaasɛ wo hemoglobin sua esiane mogya a ɛtɔ kɔ wo mogya mu nti a, wobetumi asusuw sɛ tebea yi wɔ hɔ.
- Clotting tests: Eyinom yɛ nhwehwɛmu a ɛkyerɛ bere tenten a egye ansa na wo mogya atɔ.
- von Willebrand factor (VWF) nhwehwɛmu: Saa nhwehwɛmu titiriw yi hwɛ VWF protein dodow a ɛwɔ wo mogya mu ne sɛ ebia ɛreyɛ adwuma yiye anaa.
- Factor VIII nhwehwɛmu: VWF protein no bɔ Factor VIII, protein foforo a ɛho hia a ɛboa ma mogya tu no ho ban. Enti sɛ VWF sua a, Factor VIII dodow nso betumi ayɛ nea ɛba fam. Saa sɔhwɛ yi hwehwɛ saa.
Dɛn ne ayaresa ahorow a wɔde sa eyi?
Ayaresa no gyina yare ko a wowɔ ne sɛnea wo sɛnkyerɛnne ahorow no mu yɛ den so. Nnipa dodow no ara hia ayaresa ansa na wɔayɛ wɔn oprehyɛn anaasɛ wɔapirapira akyi nkutoo.
Akwan atitiriw pii wɔ hɔ a wɔfa so sa yare:
- Desmopressin (DDAVP): Eyi ne aduru a wɔtaa de di dwuma. Ɛyɛ hormone a ɛma nipadua no yɛ adwuma. Wɔde ma sɛ aduru a wɔde petepete hwene mu anaasɛ wɔde gu hwene mu. Ɛyɛ adwuma denam VWF protein ahorow a wɔde asie yɛn nipadua mu a ɛma ɛkɔ mogya no mu ntɛmntɛm no so.
- von Willebrand factor infusions: Wɔ tebea horow a emu yɛ den mu no, wɔde VWF a wɔahyɛ no den ma nipadua no denam aduru a wɔde twitwiw nipadua no so. Eyi te sɛ nea wɔde VWF protein a ayɛ mmerɛw no ama nipadua no.
- Nnuru a ɛko tia fibrinolytics: Nnuru yi yɛ adwuma denam mogya a ɛretu no a esiw ano sɛ ɛnsɛe ntɛmntɛm dodo no so. Wɔde ma de siw mogya a ɛba wɔ tebea horow te sɛ sẽ a woyi mu no so.
- Nnuru a wɔde siw awo ano: Eyinom ho wɔ mfaso ma mmea a mogya tu wɔn kɛse wɔ brayɛ mu. Estrogen hormone a ɛwɔ saa nnuru yi mu no boa ma VWF dodow kɔ soro.
Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn?
Sɛ mogya tu wo ntɛmntɛm, wo ho yɛ wo yaw ntɛmntɛm, anaasɛ wo nna mu yɛ den a, hwɛ hu sɛ wobɛkɔ w’abusua oduruyɛfo nkyɛn. Nneɛma afoforo betumi ama mogya a ɛtɔ kɛse aba, enti ɛho hia sɛ wuhu sɛ obi anya yare no yiye.
Nea ɛho hia sen biara: Bere biara a wuntumi nni mogya a ɛretu no so no, kɔ ayaresabea hɔ Emergency Treatment Unit (ETU) ntɛm ara.
Dɛn na metumi ayɛ bere a me ne yare yi te ase no?
Sɛ wowɔ von Willebrand yare a, ɛkyerɛ sɛ ɛsɛ sɛ wohwɛ yiye kakra na amma mogya antɔ wo. Wo duruyɛfo betu wo fo wɔ eyi ho. Mpɛn pii no, eye sɛ wobɛkwati nea edidi so yi:
- Kwati agumadi a ebetumi ama obi apirapira: Agumadi a ɛfa nkitahodi pii ho, te sɛ rugby ne akuturukubɔ, mfata.
- Kwati sɛ wobɛnom nnuru a ɛma mogya yɛ mmerɛw: Nnom nnuru a wɔde kum ɛyaw te sɛ aspirin anaa NSAID (e.g., Ibuprofen, Diclofenac) a wunnyaa wo duruyɛfo no kwan.
- Kwati sɛ wobɛnom nnuru bi a wɔde ka aduan ho: Nneɛma te sɛ vitamin E ne mpataa mu ngo betumi ama mogya no ayɛ mmerɛw. Bisa wo duruyɛfo ansa na wode biribiara adi dwuma.
Ka kyerɛ wo nnuruyɛfo nyinaa (a wo sẽ ho oduruyɛfo ka ho) sɛ wowɔ saa tebea yi sɛnea ɛbɛyɛ a wobetumi ayɛ nneɛma bi de asiw mogya a ɛretu no so ansa na wɔayɛ biribiara, te sɛ oprehyɛn. Ɛyɛ adwene pa nso sɛ wobɛhyɛ aduruyɛ ho kɔkɔbɔ abɔso de abɔ aduruyɛ adwumayɛfo kɔkɔ sɛ biribi si ntɛm a.
Nkrasɛm a Wɔde Kɔ Fie
- Von Willebrand yare yɛ mogya a ɛma mogya tu a wonya fi awo mu a protein bi a wɔfrɛ no von Willebrand factor (VWF) a enni hɔ anaasɛ ɛnyɛ adwuma yiye na ɛde ba.
- Ɔkwan a ɛtaa ba (Type 1) no wɔ sɛnkyerɛnne a emu nyɛ den koraa na ebia ɛrenna sɛnkyerɛnne biara adi koraa.
- Mogya a ɛtaa tu fi hwene mu, akisikuru a ɛnyɛ den, brayɛ a emu yɛ duru, ne mogya a ɛkyɛ fi akuru mu ne sɛnkyerɛnne atitiriw.
- Ɛwom sɛ wontumi nsa koraa de, nanso wobetumi de ayaresa te sɛ Desmopressin, VWF a wɔde hyɛ nipadua mu, ne asetra mu nsakrae adi ho dwuma yiye.
- Sɛ wowɔ saa sɛnkyerɛnne ahorow yi a, ntwentwɛn wo nan ase sɛ wobɛkɔ wo duruyɛfo nkyɛn na woayɛ nhwehwɛmu a ɛho hia. Nhwehwɛmu a wɔyɛ no yiye ne sɛnea wɔhwɛ so no ne nneɛma a ɛho hia sen biara.











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