So wunnya ɔhaw nso sɛ wubegyae mogya a efi wotwitwa ketewaa bi mpo mu? Momma yɛnka von Willebrand Yare ho asɛm!

So ɛtɔ mmere bi a wotwa kakra a mogya nnyae bere tenten? Anaasɛ wo hwene mu mogya taa tu? Ebia obi wɔ w’abusua mu wɔ ɔhaw a ɛte saa ara? Sɛ saa a, eyi yɛ biribi a ebia ɛho hia wo. Ɛnnɛ yɛbɛka tebea bi a ɛntaa nsi nanso ɛtaa ba kɛse a wɔfrɛ no von Willebrand Yare ho asɛm .

Dɛn ne von Willebrand Yare?

Sɛ yɛbɛka no tiawa a, von Willebrand Yare yɛ yare a yɛn mogya ntumi nkɔ yiye, a ɛkyerɛ sɛ adeyɛ a ɛboa ma mogya tu gyae no yɛ mmerɛw. Eyi taa fi awosu mu , a ɛkyerɛ sɛ awofo betumi de yare no akɔ wɔn mma so. Nanso mma ɛnhaw wo, aduru bi wɔ hɔ a wɔde sa eyi. Nnuruyɛfo betumi ama wo nnuru a ɛboa ma mogya a ɛtwetwe yi.

Dɛn na ɛto obi a ɔwɔ von Willebrand Yare no?

Obi a ɔwɔ saa yare yi betumi mogya atɔ no asen sɛnea ɛte daa. Susuw ho hwɛ, sɛ obi twa ne sẽ ketewaa bi mpo a, ebetumi agye bere tenten ansa na mogya atɔ no. Nnipa binom hwene mu mogya taa tu. Wɔ mmea fam no, mogya betumi atu wɔn kɛse bere a wɔrekɔ brayɛ mu, anaasɛ mogya betumi atu wɔn kɛse wɔ awo akyi.

Wɔ yare no a emu yɛ den sen biara mu no , ɛtɔ mmere bi a mogya tumi tu kɔ nkwaa anaa ntini a ɛyɛ mmerɛw mu a ntease biara nnim. Eyi betumi ama obi ho ahonhon na wate ɛyaw a emu yɛ den. Wɔ afoforo fam no, ebetumi ama wɔanya mogya a ɛtɔ kɔ nipadua mu mpo , a ɛyɛ mogya a wonnya .

Saa tebea yi abu so dɛn?

Nokwarem no, eyi ne mogyatɔw a abu so sen biara wɔ wiase. Amanneɛbɔ ahorow bi kyerɛ sɛ ɛka Amerikafo dodow bɛyɛ 1%. Wɔ wiase nyinaa no, wobu akontaa sɛ nnipa ɔpepem biara mu 23 kosi 110 betumi anya yare no. Nanso, dodow yi betumi ayɛ soronko, efisɛ ebia nnipa binom wɔ mogyatɔw ho haw nanso wonhu sɛ wɔwɔ von Willebrand Yare . Ebia nnipa binom anya saa ɔhaw ahorow yi mfe pii, nanso wonhu kosi sɛ ɛbɛkyɛ dodo.

So von Willebrand yare ne hemophilia yɛ ade koro?

Yiw, bere a abien no nyinaa yɛ mogya a ɛtwetwe ho haw no, von Willebrand Yare yɛ tebea a mpɛn pii no enni sɛnkyerɛnne kakraa bi na emu nyɛ den te sɛ hemophilia . Nanso ɛno nkyerɛ sɛ ɛyɛ biribi a ɛsɛ sɛ wobu ani gu so.

Dɛn ne von Willebrand Yare no ho sɛnkyerɛnne?

Ebia nnipa pii a wɔwɔ yare no nni sɛnkyerɛnne biara koraa , anaasɛ sɛnkyerɛnne a emu nyɛ den koraa nkutoo. Nanso, wɔn a wɔwɔ yare a emu yɛ den kɛse no betumi anya yare no ho sɛnkyerɛnne te sɛ:

• Nosebleed : Eyi nyɛ mogya a ɛba daa ara kwa. Ebetumi ayɛ mogya a ɛretu bɛboro simma 10, anaasɛ mogya a efi hwene mu bɛboro mpɛn anum afe biara.
• Mogya a ɛtɔ fi kuru mu: Mogya a efi akuru ketewaa bi a wɔatwa anaa kuru foforo mu bɛboro simma 10.
• Akuru: Nnipa a wɔwɔ saa yare yi nya akuru ntɛmntɛm. Saa akuru yi nyɛ petee wɔ honam ani kɛkɛ, na mmom ebetumi ahonhon kakra na akɔ soro te sɛ akuru. Saa akuru yi nso betumi ayɛ kɛse asen sika a ɛyɛ rupee anum.
• Iron a enni nipadua mumogya a ɛtɔ kɔ nipadua mu a dade nnim : Mogya a ɛtɔ kɔ nipadua no mu yɛ tebea a nipadua no nni mogya mu nkwammoaa kɔkɔɔ a ɛdɔɔso . Dade a enni mogya mu ba bere a yɛn nipadua nni dade a ɛdɔɔso a ɛbɛma ayɛ hemoglobin no . Saa ade a wɔfrɛ no hemoglobin yi boa mogya mu nkwammoaa kɔkɔɔ ma wɔde mframa pa kɔ. Enti, sɛ yɛhwere mogya pii a, yebetumi ahwere dade nso na yɛanya saa tebea yi.
• Mogya a ɛtɔ wɔ oprehyɛn akyi: Nnipa a wɔwɔ saa yare yi tumi mogya pii, wɔ oprehyɛn nketenkete te sɛ sẽ a wɔayiyi mpo akyi.
• Brayɛ a emu yɛ duru : Wɔ mmea binom fam no, sɛ mogya tu wɔn pii wɔ wɔn nna mu araa ma ɛsɛ sɛ wɔsesa wɔn pad anaa tampon dɔnhwerew biara, anaasɛ sɛ wɔn bra kyɛ bɛboro nnanson a, ebetumi ayɛ tebea yi ho sɛnkyerɛnne.
• Mogya a ɛtɔ kɛse wɔ awo akyi anaasɛ nyinsɛn a atu agu.
• Mogya a ɛwɔ wo nsu mu: Sɛ́ ebia mogya wɔ wo nsu mu anaasɛ mogya wɔ wo nsu mu bere a woafa nsu mu akyi no, ebetumi ayɛ aduruyɛ mu tebea foforo ho sɛnkyerɛnne. Enti akyinnye biara nni ho sɛ ɛsɛ sɛ wokɔ oduruyɛfo nkyɛn .
• Mogya a ɛwɔ nsu mu (hematuria): Sɛ wuhu mogya bere a woretow, titiriw sɛ mogya no de ɔpɛ a ɛba mpofirim sɛ wobɛtow nsu ka ho a, ka kyerɛ oduruyɛfo ntɛm ara .

Nea ɛho hia paa ne sɛ, sɛ wowɔ saa sɛnkyerɛnne yi mu biako anaa nea ɛboro saa a, mmu w’ani ngu so na kɔ oduruyɛfo nkyɛn kɔhwehwɛ afotu.

Dɛn na ɛde von Willebrand Yare no ba?

Eyi yɛ awosu mu yare . Eyi kyerɛ sɛ nsakrae (mutation) a ɛba yɛn awosu mu nkwaadɔm mu na ɛde saa yare yi ba. Esiane awosu mu nsakrae yi nti, yɛn nipadua no ntumi nyɛ protein bi a wɔfrɛ no von Willebrand factor yiye. Saa nneɛma yi yɛ protein ahorow a ɛboa ma yɛn mogya tu.

Wohu von Willebrand ade yi wɔ plasma (yɛn mogya no fã a ɛyɛ nsu), mogya nkwammoaa (nkwaboaa a ɛboa ma mogya tu), ne mogya ntini afasu mu . Mpɛn pii no, sɛ mogya ntini bi sɛe a, mogya nkwammoaa no bata baabi a asɛe no, na ɛma mogya tu na ɛma mogya no gyae.Von Willebrand factor ne nea ɛboa ma mogya nkwammoaa yi bata ho yiye. Enti, sɛ wohwere saa ade yi sɛnea ɛsɛ, anaasɛ wohwere koraa a, mogya nkwammoaa no ntumi nkata ho yiye. Afei egye bere tenten ansa na mogya a ɛretu.

Nnipa dodow no ara nya yare no denam awosu mu abɔde a wɔayɛ no foforo a wonya fi wɔn awofo no mu biako hɔ so . Wɔfrɛ eyi sɛ autosomal dominant inheritance . Afoforo nso betumi anya awosu mu nkwaadɔm a wɔayɛ no foforo no afi awofo baanu no nyinaa hɔ . Wɔfrɛ eyi autosomal recessive inheritance , na ɛno ne yare no a emu yɛ den sen biara. Obi a okura saa awosu mu abɔde a wɔayɛ no foforo yi wɔ hokwan 50% sɛ ɔde mutation no bɛma wɔn mma.

Afei nso, ɛtɔ mmere bi a von Willebrand yare betumi aba sɛ aduruyɛ mu tebea afoforo te sɛ kokoram , nipadua no mu tumi a ɛko tia nyarewa , komayare, ne mogya ntini mu nyarewa a ɛde ba.

Ɔkwan bɛn so na nnuruyɛfo hu von Willebrand Yare no?

Sɛ wokɔ oduruyɛfo nkyɛn a, wobedi kan abisa wo wo yare no ho sɛnkyerɛnne. Ebia wobebisa nso sɛ ebia obi a ɔwɔ w’abusua mu anya yare no ho sɛnkyerɛnne anaa mogyatɔw a ɛte saa ara anaa. Afei ebia wɔbɛyɛ sɔhwɛ ahorow bi te sɛ:

• Mogya dodow a edi mũ (CBC): Eyi kyerɛ mogya mu nkwammoaa kɔkɔɔ, mogya mu nkwammoaa fitaa ahorow, ne mogya mu nkwammoaa a ɛwɔ wo mogya mu dodow. Ɛsan nso susuw hemoglobin dodow a ɛwɔ wo mogya mu nkwammoaa kɔkɔɔ mu. Nnipa dodow no ara a wɔwɔ von Willebrand yare no wɔ CBC a ɛfata. Nanso, ebia nnipa a mogya tu wɔn pii no hemoglobin a ɛba fam ne mogya mu nkwammoaa kɔkɔɔ a ɛba fam.
• Nkwammoaa mu nkwammoaa a ɛboaboa ano ho nhwehwɛmu: Eyi sɔ sɛnea wo mogya nkwammoaa no bata ho yiye ma ɛboa ma mogya tu no hwɛ.
• Activated partial thromboplastin time test (APTT): Eyi hwehwɛ von Willebrand factor ne nneɛma afoforo a ɛma mogya tu a ɛboa ma mogya tu no mu. Sɛ saa nneɛma yi dodow sua sen sɛnea ɛte daa a, egye bere tenten ansa na mogya no atɔ.
• Prothrombin bere (PT) nhwehwɛmu: Eyi nso susuw nneɛma afoforo a ɛma mogya tu.
• Fibrinogen nhwehwɛmu:Fibrinogen yɛ protein foforo a ɛboa ma mogya tu.
• Von Willebrand factor antigen test: Eyi kyerɛ von Willebrand factor protein dodow a ɛwɔ wo mogya mu.
• Ristocetin cofactor sɔhwɛ: Eyi hwɛ sɛnea von Willebrand factor no yɛ adwuma.
• Von Willebrand factor multimers test: Eyi hwɛ sɛnea factor no nhyehyɛe.

Ebia ɛho behia sɛ wo duruyɛfo yɛ mogya mu nhwehwɛmu pii de si so dua sɛ woahu, efisɛ nneɛma te sɛ hormone dodow betumi asesa von Willebrand factor dodow a ɛwɔ wo mogya mu.

Von Willebrand yare ahorow pii wɔ hɔ. Nnuruyɛfo bɛyɛ nhwehwɛmu pii de ahu ɔkwan pɔtee a wowɔ. Saa ahorow yi ne:

• Ɔkwan 1: Eyi ne ɔkwan a wɔtaa de di dwuma. Ayarefoɔ 60% kɔsi 80% na wɔwɔ saa saa. Saa nkurɔfo yi wɔ von Willebrand factor a ɛba fam wɔ wɔn mogya mu. Ebia wonni yare no ho sɛnkyerɛnne biara. Sɛ wɔyɛ saa a, wɔyɛ odwo paa.
• Ɔkwan a ɛto so 2: Wɔ saa ɔkwan yi mu no, von Willebrand factor no nyɛ adwuma yiye. Ebia saa nkurɔfo yi bɛte mogya a ɛtɔ kakra kosi nea ɛkɔ fam. Ayarefoɔ 15% kɔsi 30% na wɔwɔ saa saa.
• Ɔkwan a ɛto so 3: Eyi ne ɔkwan a emu yɛ den sen biara. Ɛho yɛ na nso. Ɛka ayarefoɔ 5% kɔsi 10%. Saa nkurɔfo yi wɔ von Willebrand factor a ɛba fam koraa wɔ wɔn mogya mu, anaasɛ wonni bi koraa. Eyi betumi de mogyatɔw ho haw ahorow a emu yɛ den aba.

Dɛn ne von Willebrand Yare no ayaresa?

Nnuruyɛfo de nnuru ahorow sa yare yi:

• (Desmopressin): Eyi yɛ hormone. Ɛma von Willebrand factor dodow a ɛwɔ mogya no mu no kɔ soro. Eyi ne ayaresa a wɔtaa de sa yare yi.
• Von Willebrand factor infusions: Wɔnam ntini bi so ma nnipa binom saa factor yi na ama mogya atɔ no agyae. Wobetumi de saa ayaresa yi ama ansa na wɔayɛ oprehyɛn. Ebia ɛho behia sɛ nnipa binom a wɔwɔ yare a emu yɛ den nya saa ayaresa yi daa na ama factor dodow a ɛwɔ wɔn mogya mu no akɔ so ayɛ nea ɛyɛ den.
• (Nnuru a wɔde ko tia fibrinolyly): .Saa nnuru yi yɛ adwuma denam mogya a ɛtwetwe no a ɛmma ɛnhyew no so. Ebia wo duruyɛfo bɛkyerɛw nnuru yi ama wo sɛ woreyɛ wo sẽ oprehyɛn anaasɛ mogya retu wo kɛse wɔ brayɛ mu a.
• Nnuru a wɔde siw awo ano: Eyinom boa wɔn a mogya tu wɔn kɛse wɔ brayɛ mu no. Estrogen hormone a ɛwɔ saa nnuru yi mu no ma von Willebrand factor dodow a ɛwɔ mogya no mu no yɛ kɛse.

So metumi atew asiane a ɛwɔ hɔ sɛ menya yare yi so?

Eyi taa yɛ awosu mu . Sɛ w’awofo wɔ saa yare yi a, wubetumi anya bi afi wɔn mu biako anaa wɔn baanu nyinaa hɔ. Enti biribiara nni hɔ a yebetumi ayɛ de asiw ano.

Sɛ mewɔ saa tebea yi a, dɛn na ɛsɛ sɛ mehwɛ kwan?

Nnuruyɛfo betumi asa von Willebrand yare no, nanso wontumi nsa no koraa . Nnipa dodow no ara wɔ type 1 anaa type 2. Sɛ wɔapira anaasɛ wohia oprehyɛn nkutoo a, na wohia ayaresa. Ebia nnipa a wɔwɔ type 3 no behia ayaresa a ɛkɔ so na ama wɔatumi asiw mogya a ɛretu no ano.

Mewɔ von Willebrand Yare. Ɔkwan bɛn so na ɛsɛ sɛ mehwɛ me ho?

Esiane sɛ nnipa dodow no ara wɔ yare no ho sɛnkyerɛnne a emu nyɛ den kosi nea ɛkɔ fam nti, yare yi a wɔbɛtra ase no kyerɛ sɛ wubesusuw nneɛma kakraa bi ho:

• Kwati dwumadi ahorow a ebetumi ama obi apirapira: Ɛyɛ papa sɛ wobɛtwe wo ho afi agumadi a enya nkɛntɛnso kɛse ho, sɛ nhwɛso no, bɔɔlbɔ, rugby, ne hockey.
• Ka kyerɛ wo nnuruyɛfo nyinaa, a wo sẽ ho oduruyɛfo ka ho, sɛ wowɔ saa tebea yi: afei wobetumi ayɛ nhyehyɛe a ɛbɛma wɔadi mogya a ɛbɛtɔ so bere a wɔayɛ oprehyɛn awie no.
• Mfa aspirin anaa nnuru a aspirin wom nni dwuma.
• Mfa nnuru a ɛnyɛ steroid a ɛko tia ɔyare mmoawa (NSAIDs) , te sɛ ibuprofen, nni dwuma, gye sɛ oduruyɛfo bi a onim sɛ wowɔ von Willebrand yare no akyerɛw ama wo.
• Kwati sɛ wobɛnom aduannuru a vitamin E, mpataa mu ngo, ne turmeric wom.
• Susuw aduruyɛ mu kɔkɔbɔ krataa a ɛkyerɛ sɛ obi hu obi ho hwɛ: Sɛ wohyɛ abɔso te sɛ eyi anaasɛ wukura krataa a ɛkyerɛ sɛ obi hu obi a, ebetumi aboa wo ma woanya ayaresa a ɛfata wɔ tebea a egye ntɛmpɛ mu.

Bere bɛn na ɛsɛ sɛ mekɔ ayaresabea ntɛm?

Kɔ ayaresabea ntɛm ara bere biara a mogya retu wo a wuntumi nni so . Sɛ ɛyɛ opira ketewaa bi mpo a, sɛ mogya no annyae a, hwɛ hu sɛ wobɛhwehwɛ aduruyɛfo afotu.

Nsɛm bɛn na ɛsɛ sɛ mibisa oduruyɛfo no?

Sɛ wowɔ von Willebrand yareɛ a, ebia wowɔ nsɛmmisa fa sɛdeɛ yareɛ no bɛka w’asetena ho. Nsɛmmisa bi a wubetumi abisa wo duruyɛfo ni:

• Dɛn nti na minyaa saa yare yi?
• So me mma betumi anya yare yi fi awo mu?
• So eyi bɛkɔ so asɛe?
• Dɛn ne ayaresa ahorow a wɔde sa eyi?
• Dɛn ne ayaresa no mu nsunsuanso bɔne?
• So merentumi nyɛ nneɛma bi, te sɛ akwantu anaa agumadi, esiane yare yi nti?

Sɛ yɛbɛbɔ no mua a, nneɛma a ɛsɛ sɛ yɛkae ne

Von Willebrand yare yɛ ɔyare a abu so yiye, a efi awosu mu mogya a ɛtwetwe. Nnipa dodow no ara wɔ sɛnkyerɛnne ahorow a emu nyɛ den kosi nea ɛkɔ fam. Ebia mogya taa tu wɔn hwene mu, anaasɛ ntwitwiridii nketenkete mpo betumi agye bere tenten ansa na mogya atɔ wɔn. Afoforo nso wɔ sɛnkyerɛnne ahorow a emu yɛ den. Sɛ nhwɛso no, ebia mogya bɛtɔ wɔn nkwaa mu na wɔate wɔn nkwaa mu yaw.

Ebia nnipa binom nnim sɛ wɔwɔ yare no mfe pii. Sɛ wowɔ bi a, ebia ɛbɛyɛ wo ahotɔ sɛ wubehu nea enti a mogya tu ho haw. Ebia ɛhaw wo nso sɛ wo mma nso benya tebea no. Ɛwom sɛ nnuruyɛfo ntumi nsa yare no de, nanso wobetumi asa . Wobetumi nso abua wo nsemmisa a ɛfa sɛ ebia wo mma benya bi agyapa mu. Wobetumi nso ama wo nsɛm a wuhia na ama woanya von Willebrand yare no sɛnea ɛbɛyɛ a ɛrensiw wo kwan sɛ wobɛtra ase a ɛyɛ nnam na ɛyɛ daa. Enti, mma ɛnhaw wo, sɛ wote ase na wohwɛ so yiye a, wubetumi ne no atra ase yiye.