So mogya taa tu wo hwene mu nso? Ebetumi ayɛ HHT (Hereditary Hemorrhagic Telangiectasia)!

So wo hwene mu mogya taa tu? Anaasɛ woahyɛ nsensanee kɔkɔɔ nketenkete bi nsow wɔ wo nipadua no afã horow bi, sɛ nhwɛso no, w’anim, w’ano, anaa wo nsateaa ano? Ɛtɔ mmere bi a yensusuw eyinom sɛ nneɛma a ɛfata, nanso ebia aduruyɛ mu tebea bi wɔ hɔ a ɛsɛ sɛ wɔhwɛ so kakra. Ɛno ne nea HHT yɛ, nanso nnipa pii nnim ho asɛm. Ɛnnɛ yɛreka saa HHT yi ho asɛm, anaasɛ wɔ aduruyɛ mu no, `(Hereditary Hemorrhagic Telangiectasia)`, awosu mu yare. Ebinom nso frɛ no `(Osler-Weber-Rendu yare)`.

Dɛn ankasa ne HHT?

Sɛ yɛbɛka no tiawa a, HHT yɛ awosu mu yare a ɛka mogya ntini a ɛwɔ yɛn nipadua mu no. Wunim, yɛwɔ saa nsukorade nketewa yi a ɛde mogya fa yɛn nipadua nyinaa mu, a yɛfrɛ no mogya ntini. Wɔ tebea yi mu no, nea ɛba ne sɛ mogya ntini yi, titiriw nea ɛyɛ fɛ yiye a wɔfrɛ no capillaries no nnyin yiye. Yɛfrɛ saa ntini nketenkete a ɛnyɛ ne kwan so yi ``telangiectasias''.

Susuw ho hwɛ, yɛwɔ ntini akɛse a ɛde mogya fi yɛn koma mu kɔ yɛn nipadua no fã a aka, ne ntini a ɛde saa mogya no san kɔ koma no mu. Nea wɔfrɛ no capillaries na ɛma abusuabɔ a ɛda abien yi ntam no ba. Wɔ HHT mu no, ɛtɔ mmere bi a saa ntini ne ntini ahorow yi betumi anya nkitahodi a ɛnteɛ tẽẽ, a ɛnfa ntini nketenkete no mu. Yɛfrɛ saa arteriovenous malformations yi, anaasɛ AVMs tiawa.

Saa mogya ntini a ɛnteɛ yi yɛ mmerɛw yiye na ɛyɛ mmerɛw. Ɛnyɛ den sɛ wobetumi apae anaasɛ wɔbɛpaapae, na ama mogya atu (mogya atɔ). Nsɛnkyerɛnne ne nsɛnnennen no gyina baabi a mogya no tu so.

Henanom na wobetumi anya HHT?

Saa tebea a wɔfrɛ no HHT yi betumi aka mmea, mmarima, ne mmofra nkumaa mpo . Abusuakuw, nyamesom, ne kɔla mfa eyi ho. Titiriw esiane sɛ ɛnam awosu mu nkwaadɔm so fi awo ntoatoaso so kɔ awo ntoatoaso so nti, sɛ obi wɔ abusua no mu a, afoforo nso wɔ hokwan sɛ wobenya bi.

Wobu eyi sɛ tebea a ɛntaa nsi koraa . Nanso, mpɛn pii no, wonhu no yiye. Eyi kyerɛ sɛ, ɛwom sɛ wobu akontaa sɛ nnipa bɛyɛ 5,000 biara mu biako wɔ wiase nyinaa de, nanso wɔn mu dodow no ara nnim sɛ wɔwɔ bi.

Dɛn na ɛde HHT ba?

Sɛnea madi kan aka no, HHT yɛ awosu mu tebea koraa . Eyi kyerɛ sɛ wonya fi awofo hɔ ma mmofra. Wobu no sɛ ``dominant disorder``. Sɛ yɛbɛka no tiawa a, abofra betumi anya yare no sɛ ɔwofo biako pɛ, ɛna anaa agya no, nya awosu mu abɔde a ɛnteɛ no mpo a .

Nyansahufo ahu nkwaadɔm mu nsakrae ahorow ɔhaha pii wɔ awosu mu nkwaadɔm asia a ɛne HHT wɔ abusuabɔ mu. Nanso, mprempren, nkwaadɔm mu nsakrae a ɛtaa ba no wɔ awosu mu nkwaadɔm abien a wɔfrɛ no ENG ne ACVRL1 mu. Nyansahufo da so ara reyɛ eyi ho nhwehwɛmu.

Dɛn ne HHT ho sɛnkyerɛnne ahorow?

HHT ho sɛnkyerɛnne betumi ayɛ soronko kɛse wɔ nnipa biara mu. Egyina baabi a mogya ntini a ɛnyɛ ne kwan so a mekaa ho asɛm no wɔ wɔ nipadua no mu. Ebia nnipa binom rennya yare no ho sɛnkyerɛnne titiriw biara. Nanso afoforo nso betumi anya yare no ho sɛnkyerɛnne a emu yɛ den yiye na emu yɛ den.

Nanso, sɛnkyerɛnne a ɛtaa ba wɔ nnipa a wɔwɔ HHT mu ne mogya a wɔtaa tu fi hwene mu (epistaxis) . Ebia nnipa binom hwene mu mogya bɛtɔ mpɛn pii da biara. Ebia na ɔhaw yi wɔ hɔ fi mmofraase.

Nea ɛka ho no, ebia nnipa binom a wɔwɔ HHT behu nsensanee kɔkɔɔ nketenkete (telangiectasias) wɔ wɔn nipadua so. Ebia eyinom kɔla asesa kakra ma wo. Wɔtaa hu eyinom wɔ:

• Wɔ anim
• Wɔ nsateaa anaa nsateaa ano
• Wɔ nsa mu
• Ano mu (wɔ mucous membrane no so) .
• Wɔ anofafa so
• Atwa hwene no ho ahyia

Eyinom akyi no, nnipa binom a wɔwɔ HHT nso betumi anya:

• Monemia : Eyi kyerɛ sɛ mogya mu nkwammoaa kɔkɔɔ nni nipadua no mu. Eyi betumi aba esiane mogya a ɛtaa tu nti.
• Mogya a ɛtɔ fi yafunu anaa dwensɔtwaa mu : Ebia eyi rentumi nhu, nanso ebia mogya wɔ nsu mu anaasɛ nsu a ɛyɛ tuntum.

Arteriovenous Malformations (AVMs) ne nea efi mu ba

Ɛtɔ mmere bi a nnipa a wɔwɔ HHT tumi nya ntini akɛse (AVMs) wɔ mogya ntini akɛse mu. Saa AVM ahorow yi betumi ayɛ kɛse wɔ akwaa ahorow te sɛ ahurututu, amemene, akyi berɛmo, ne mmerɛbo mu titiriw. Afei wobetumi de sɛnkyerɛnne ahorow a ɛfa akwaa biara ho aba.

• Sɛ wowɔ AVM ahorow wɔ w’ahurututu mu a:
• Honam ani kɔla a ɛyɛ bruu (titiriw anofafa, nsateaa nnadewa) .
• Mogya a wɔfe (hemoptysis) .
• Ɔbrɛ a wɔte nka ntɛmntɛm
• Ɔhome a ɛyɛ den (dyspnea) .
• Sɛ wowɔ `(AVMs)` wɔ amemene no mu a: Eyinom ne nea ɛyɛ hu kakra. Efisɛ sɛ saa `(AVM)` yi mu biako pae wɔ amemene no mu a, ɛyɛ aniberesɛm yiye.
• Adwene a ɛyɛ basaa
• Anisoadehu abien
• Akisikuru a ɛma obi ho kyere no
• Tebea horow a ɛte sɛ ɔyare a ɛma obi hwe ase betumi aba.
• Sɛ wowɔ mmerɛbo mu AVM ahorow a:
• Koma no tumi kɔ tebea a ɛyɛ ``Koma a entumi nyɛ adwuma yiye'' mu efisɛ sɛ mogya de ahopere fa ``(AVM)'' a ɛwɔ mmerɛbo no mu no mu a, ɛsɛ sɛ koma no yɛ adwumaden kɛse de mogya kɔ nipadua no nyinaa mu.
• Sɛ wowɔ akyi berɛmo mu AVM ahorow a:
• Akyi yaw
• Nsa anaa anan betumi ayɛ mmerɛw anaasɛ atenka a ɛyera.

Nea ɛho hia: Sɛ saa ``(AVMs)`` yi mu paapae na mogya tu a, ɛyɛ ntɛmpɛ a anibere wom yiye. Enti, sɛ wowɔ sɛnkyerɛnne ahorow a yɛaka ho asɛm wɔ atifi hɔ no bi a, ɛho hia yiye sɛ wohwehwɛ aduruyɛfo afotu ntɛm ara.

So me mma nso betumi anya HHT?

Yiw, sɛ wowɔ HHT a, wo mma no mu biara wɔ hokwan 50% sɛ obenya yare no afi awo mu.Aane. Ɛno kyerɛ sɛ bere biara a wɔbɛwo abofra no, ɛyɛ 50% sɛ abofra no benya HHT na 50% nso wɔ hɔ sɛ ɛrennya bi. Ɛte sɛ nea worebɔ sika na woanya ti anaa dua.

Ɔkwan bɛn so na wohu HHT?

Wobetumi ayɛ awosu mu nhwehwɛmu de asi HHT so dua. Nanso mpɛn pii no, nnuruyɛfo gyina ayaresabea ho nsɛm te sɛ yare no ho sɛnkyerɛnne ne abusua abakɔsɛm so hu. Sɛ wokɔ oduruyɛfo nkyɛn a, mpɛn pii no wɔbɛyɛ nea edidi so yi:

• Wɔbɛbisa wo nsɛm a ɛkɔ akyiri afa w’ankasa w’ayaresa ho abakɔsɛm ho (e.g. bere tenten a mogya atɔ wo hwene mu, mpɛn dodow a woatu, ne ɔhaw afoforo a woahyia).
• Bisa w’abusua a wɔbɛn wo paa (awofo, anuanom, mmofra) aduruyɛ ho abakɔsɛm efisɛ eyi kɔ so wɔ mmusua mu.
• Wɔbɛhwɛ wo nipadua mu (ahwɛ sɛ nsensanee kɔkɔɔ bi wɔ hɔ anaa nea ɛtete saa anaa).
• Sɛ ɛho hia a, hwɛ sɔhwɛ ahorow (e.g. `(scan)` ahorow, `(Endoscopy)`) na hwɛ sɛnea nipadua no mu akwaa ahorow te .

Oduruyɛfo betumi asusuw anaasɛ obehu sɛ wowɔ HHT sɛ anyɛ yiye koraa no wowɔ sɛnkyerɛnne ahorow a edidi so yi mu abiɛsa a:

• Mogya a ɛtaa tu fi hwene mu.
• Telangiectasias pii a ɛwɔ mmeae a wɔtaa hu wɔ honam ani (te sɛ anim, anofafa, nsateaa).
• ``Telangiectasias'' anaa ``AVMs'' a ɛwɔ akwaa a ɛwɔ nipadua no mu (e.g. ahurututu, amemene, yafunu, dwensɔtwaa) (eyinom nam nhwehwɛmu nkutoo so na wohu).
• Abusua muni bi a ɔbɛn no a ɔwɔ HHT.

Dɛn ne ayaresa ahorow a wɔde sa HHT?

Nea ɛyɛ awerɛhow no, wonni HHT aduru biara. Nanso, ayaresa ahorow bi wɔ hɔ a ebetumi aboa ma wɔadi yare no ho sɛnkyerɛnne ahorow so, atew yare no ho sɛnkyerɛnne so, na atew asiane a ɛwɔ hɔ sɛ ɔhaw ahorow a emu yɛ den betumi aba no so. Nyansahufo da so ara reyɛ adwuma de ahwehwɛ ayaresa a wɔde bɛsa HHT, titiriw bere a wɔayɛ nnuru a wɔde ko tia ntini a ɛma mogya tu no.

Bere a wo duruyɛfo no resa yare no ho sɛnkyerɛnne a ɛwɔ hɔ dedaw no, ɔbɛhwɛ nso sɛ ebia ɔhaw biara a ɛfa HHT ho a ebia ennya nhuu sɛnkyerɛnne biara anaa.

Ebia nea edidi so yi ka ayaresa no ho:

• Laser ayaresa (`(Ablation)`): Oprehyɛn ketewaa bi a wɔde laser hann di dwuma de gyae mmeae a mogya tu (telangiectasias) a mogya taa tu no.
• Embolization: Oprehyɛn ketewaa bi a esiw mogya ntini bi a ɛkɔ baabi a mogya tu anaa AVM a mogya betumi atu no kwan.
• Ayaresa a wɔde sa mogya a ɛtɔ kɔ nipadua mu: Dade aduru a wɔde ma, ne mogya (mogya a wɔde ma) sɛ ɛho hia a.
• Sɛnea ɛbɛyɛ a wubesiw hwene mu mogya ano: Fa nneɛma te sɛ srade ne nkyene a wɔde petepete so di dwuma na ama hwene no mu ayɛ nwini.
• Sɛnea wobeyi AVM ahorow afi hɔ: Wobetumi de mframa a wɔde sa yare anaa oprehyɛn ayi AVM ahorow bi afi hɔ.
• Aduruyɛ mu ayaresa a ɛko tia ntini a ɛma mogya tu: Wobetumi de eyinom ama sɛ nnuru a wɔde twitwiw nipadua no mu anaasɛ sɛ nnuru a wɔde sa yare.

Ebia wobɛkɔ akɔhwɛ animdefo nso ma wɔasa nipadua nhyehyɛe pɔtee bi te sɛ mmerɛbo, ahurututu, yafunu mu nhyehyɛe, ne amemene.

So wobetumi asiw HHT ano?

Esiane sɛ ɛyɛ awosu mu yare nti, ɔkwan biara nni hɔ a wobɛfa so asiw HHT ano anaasɛ wobɛtew asiane a ɛwɔ hɔ sɛ wubenya bi no so. Nanso, sɛ w’awofo, wo nuanom, anaa wo mma wɔ HHT a, ɛho hia sɛ woka kyerɛ wo duruyɛfo. Eyi betumi aboa wo ma woahu sɛ ebia wowɔ tebea no ntɛm, afi ase sa yare no ntɛm, na asiw nsɛnnennen ano.

Dɛn ne adwene a ɛwɔ hɔ ma nnipa a wɔwɔ HHT?

Nnipa a wɔwɔ HHT betumi atra ase a ɛkame ayɛ sɛ ɛte sɛ nea ɛfata. Nanso, AVM ne mogya a ɛkɔ so wɔ ahurututu ne amemene mu no yɛ aniberesɛm na ehia sɛ wɔsa. Sɛ wɔhwɛ so yiye a, nnipa dodow no ara betumi atra ase sɛnea ɛsɛ.

Dɛn na ɛsɛ sɛ wɔyɛ ma mogya a ɛba hwene mu esiane HHT nti?

HHT mu mogya a ɛba hwene mu abu so kɛse, enti nneɛma kakraa bi wɔ hɔ a wubetumi ayɛ de aboa ma woasiw ano:

• Kwati sɛ wode nnuru bi bedi dwuma, titiriw nnuru a wɔde kum ɛyaw te sɛ aspirin ne NSAIDs (e.g. ibuprofen, diclofenac). Eyinom betumi atew mogya a ɛtwetwe no so na ama mogya atu no ayɛ kɛse. Bisa wo duruyɛfo ansa na woanom aduru biara.
• Fa da biara da nsɛmma nhoma sie. Kyerɛw nnuan a wudi ne dwumadi ahorow a woyɛ a ɛma mogya tu wo hwene mu. Afei wubetumi akwati saa nneɛma no.
• Ma wo hwene mu nyɛ nwini na srade nhyɛ mu. Wobetumi ayɛ eyi denam ade a ɛma nsu yɛ hyew a wode bedi dwuma, srade a wo duruyɛfo kamfo akyerɛ a wode bedi dwuma, anaasɛ nkyene a wɔde petepete hwene mu a wode bedi dwuma so. Ɛda adi sɛ mogya bɛtu hwene a ayow.

Dɛn bio na metumi abisa me duruyɛfo afa HHT ho?

Sɛ wohu sɛ wowɔ HHT a, ɛbɛyɛ papa sɛ wubebisa wo duruyɛfo nsɛm te sɛ eyinom:

• So ɛsɛ sɛ wɔsɔ m’abusua mufo a aka no hwɛ sɛ ebia eyi te anaa?
• So metumi adi agumadi? Agumadi ahorow bɛn na eye na dɛn na ɛnyɛ papa?
• So dwumadi pɔtee bi wɔ hɔ a ɛsɛ sɛ mekwati?
• So nsa, nnuan titiriw, anaa nnuru foforo bi wɔ hɔ a ɛsɛ sɛ mekwati?
• Dɛn na metumi ayɛ na amma hwene mu mogya antɔ anaasɛ asiw ano ntɛm?
• So asiane biara nni ho sɛ menyinsɛn? So nneɛma titiriw bi wɔ hɔ a ɛsɛ sɛ mehwɛ yiye?
• Sɛ mogya tu me a, bere bɛn na ɛsɛ sɛ mekɔ ayaresabea ntɛm ara?

Awiei koraa no, nea ɛsɛ sɛ yɛkae

HHT yɛ awosu mu yare a mpɛn pii no wonhu. Nsɛnkyerɛnne no betumi afi mogya a ɛtaa tu fi hwene mu so kosi nsɛnnennen a emu yɛ den a ɛka nipadua nhyehyɛe ahorow pii so. Sɛ wususuw sɛ wo anaa w’abusua mu obi wɔ HHT a, yɛsrɛ wo ne oduruyɛfo nkasa.Sɛ wɔsa yare ntɛm a, ebetumi aboa ma wɔasiw nsɛnnennen ano. Awosu mu nhwehwɛmu nso betumi aboa ma wɔahu sɛ ebia tebea no aka abusua no mufo afoforo anaa. Sɛ́ wobɛkɔ so ahu nneɛma pii no ne ade a ɛho hia sen biara wɔ tebea a ɛte sɛɛ mu.

` HHT, Hereditary Hemorrhagic Telangiectasia, Osler-Weber-Rendu syndrome, hwene mu mogya, mogya ntini, awosu mu nyarewa, AVM, telangiectasia