So wowɔ adamfo bi a ɔware paa, ne nsa ne ne nan atenten, na ne ho yɛ hare? Sɛ yehu obi a ɔte saa a, yesusuw sɛ ɔbɛyɛ kɛse ama agumadi te sɛ basketball. Nanso ɛnnɛ yɛrebɛka akwahosan tebea bi a ɛka honam fam su ahorow yi ho asɛm, a yɛnka ho asɛm pii, nanso ebetumi ayɛ nea ɛho hia yiye. Ɛno ne Marfan Syndrome. Ebetumi aka koma no titiriw, enti ɛho hia paa sɛ wohu eyi.
Sɛ yɛbɛka no tiawa a, dɛn ne Marfan Syndrome?
Marfan syndrome yɛ awosu mu tebea bi a ɛba yɛn awosu mu. Eyi fi ‘ntini a ɛka bom’ a ɛwɔ yɛn nipadua mu a ennyin yiye no. Susuw yɛn nipadua ho sɛ ɔdan. Wɔde semɛnte kyekyere biribiara a ɛwɔ ɔdan yi mu, te sɛ birikisi, afasu, ne ɔdan atifi no bom na ɛma ɛyɛ den. Saa ara nso na ntini a ɛka bom yɛ ‘glue’ a ɛboa ma biribiara a ɛwɔ yɛn nipadua mu te sɛ akwaa, nnompe, ne mogya ntini no bom na ɛma ɛyɛ den.
Wɔ obi a ɔwɔ Marfan syndrome mu no, ‘gum’ anaa ntini a ɛka bom, a ɛwɔ nipadua no mu no yɛ mmerɛw. Ɛte sɛ nea wode semɛnte a ne bo nyɛ den resi ofie. Eyi betumi aka mmeae pii, a aniwa, ahurututu, ne nnompe nhyehyɛe ka ho. Nanso nkɛntɛnso a emu yɛ den sen biara betumi aba yɛn koma ne yɛn mogya ntini atitiriw so.
Dɛn nti na eyi ka koma no kɛse? Ɔhaw atitiriw abien!
Ntini a ɛka bom a ɛyɛ mmerɛw betumi asɛe koma no afã atitiriw abien.
1. Aorta: Eyi ne mogya ntini titiriw a ɛsõ sen biara a ɛde mogya fi yɛn koma mu kɔ nipadua no nyinaa mu. Te sɛ paipu nhyehyɛe titiriw a ɛde nsu fi nsukorabea a ɛwɔ yɛn fie no mu kɔ baabiara no ara pɛ.
2. Koma Valves: Eyinom te sɛ apon nketewa a ɛwɔ koma no mu. Saa ntini ahorow yi hwɛ ma mogya no kɔ ɔkwan biako pɛ so.
Afei ma yɛnhwɛ nkɛntɛnso a ɛwɔ afã abien yi so.
1. Dɛn na ɛba aorta no so?
Obi a ɔwɔ Marfan syndrome no wɔ ntini a ɛka bom yɛ mmerɛw wɔ wɔn aorta afasu mu. Eyi betumi de ɔhaw atitiriw abien aba:
- Aortic Dilation: Mpɛn pii no, mogya ntini bi yɛ nea ɛyɛ mmerɛw. Nanso, esiane mmerɛwyɛ yi nti, aorta no fi ase trɛw nkakrankakra na ɛyɛ kɛse, na entumi nnyina nhyɛso a koma bɔ biara de ba no ano.
- Aortic Aneurysm: Ɛtɔ mmere bi a, saa ntrɛwmu yi nnyae kɛkɛ. Ntini no fã a ɛyɛ mmerɛw sen biara no fi ase bɔ te sɛ balloon. Eyi ne nea yɛfrɛ no ``Aortic Aneurysm''. Eyi ne ɔhaw a ɛyɛ hu sen biara a ɛde Marfan yare no ba. Efisɛ saa ahurututu a ɛte sɛ balloon yi betumi apae (apaapae) anaasɛ atetew (apaapae) bere biara. Ɛyɛ asɛm a egye ntɛmpɛ a ɛde nkwa to asiane mu.
2. Dɛn na ɛba koma no mu ntini ahorow no so?
Ntini a ɛka bom a ɛyɛ mmerɛw nso betumi ama koma no mu ntini ahorow no anyɛ adwuma yiye. Wobetumi ayɛ mmerɛw na wɔrenbɛn wɔn ho yiye. Eyi nti na:
- Valve Regurgitation: Sɛ valve bi anto mu yiye a, mogya betumi asan akɔ koma no mu. Eyi ma koma no yɛ adwumaden de mogya kɔ nipadua no mu. Bere kɔ so no, eyi betumi ama obi koma atu. Valve ahorow a ɛtaa ka no ne Aortic Valve ne Mitral Valve.
- Mitral Valve Prolapse: Wɔ tebea yi mu no, mitral valve a ɛwɔ koma no benkum so no yɛ mmerɛw na ɛnto mu yiye, na mmom ɛbobɔw n’akyi.
Ade a ɛho hia ne sɛ nnipa du biara mu bɛyɛ akron a wɔwɔ Marfan syndrome benya koma anaa aortic haw, enti ɛho hia sɛ wohu eyi.
Dɛn ne komayare ho sɛnkyerɛnne ahorow yi? Yɛyɛ dɛn hu wɔn?
Mpɛn pii no, ebia sɛnkyerɛnne biara nni hɔ wɔ mfiase no mu. Nanso, saa sɛnkyerɛnne ahorow yi betumi ada adi bere a aorta no trɛw anaasɛ valve no mu haw ahorow no mu yɛ den no. Sɛ wowɔ eyinom mu biako anaa nea ɛboro saa a, ɛho hia paa sɛ wobɛkɔ oduruyɛfo nkyɛn.
| Nsɛnkyerɛnne a ɛkyerɛ | Nkyerɛkyerɛmu a ɛnyɛ den |
|---|---|
| Ɛyaw a ɛba wo koko anaa akyi atifi | Ɛyɛ asiane titiriw sɛ ɛyaw no ba mpofirim na emu yɛ den a. |
| Ɔhome a ɛyɛ tiaa anaasɛ ɛyɛ den sɛ ɔbɛhome | Ɔbrɛ a ɛba bere a obi nantew kakra anaa adwuma a ɔyɛ no. |
| Palpitations (te nka sɛ wo koma rebɔ ntɛmntɛm) . | Sɛ wote nka sɛ wo koma bɔ resakra anaasɛ wo moma so rebɔ. |
| Sɛ wote nka sɛ wo ho ayɛ wo yaw anaasɛ wo ti ayɛ hare | Sɛ wote nka sɛ wo ho yɛ wo yaw bere a wugyina hɔ anaasɛ wosɔre mpofirim no. |
| Ɔbrɛ ne mmerɛwyɛ a ɛyɛ soronko | Ɔbrɛ a wɔte nka bere nyinaa a ntease biara nnim. |
| Nan ne anan a ɛho ahonhon | Ebetumi ayɛ sɛnkyerɛnne a ɛkyerɛ sɛ koma no dwumadi so atew. |
Ɔkwan bɛn so na oduruyɛfo no hu yare yi pɛpɛɛpɛ?
Marfan yare no a wobehu no betumi ayɛ anifere kakra efisɛ ɛnyɛ obiara na ɔwɔ yare no ho sɛnkyerɛnne koro, enti oduruyɛfo bɛhwɛ nneɛma pii.
- Nipadua mu nhwehwɛmu: Oduruyɛfo no bɛhwɛ wo tenten, wo nsa ne wo nan tenten, wo nsateaa tenten, wo koko nsusuwii, w’ani, ne w’akyi berɛmo.
- Abusua aduruyɛ ho abakɔsɛm: Esiane sɛ eyi yɛ awosu mu yare nti, wobebisa wo sɛ ebia obi a ɔwɔ w’abusua mu anya saa sɛnkyerɛnne ahorow yi anaasɛ wawu esiane komayare mpofirim nti anaa.
- Sɔhwɛ titiriw: Wɔyɛ nhwehwɛmu pii de hu tebea pɔtee a koma no te.
- Echocardiogram: Eyi te sɛ koma no mu nhwehwɛmu. Etumi hu nneɛma te sɛ aorta no kɛse ne sɛnea ntini ahorow no yɛ adwuma pefee.
- Electrocardiogram (EKG): Eyi boa ma wɔhwɛ sɛnea koma no mu anyinam ahoɔden yɛ adwuma, kyerɛ sɛ, sɛ ebia ɔhaw bi wɔ koma no bɔ a ɛyɛ den no mu anaa.
- Awosu mu Nhwehwɛmu: Mogya mu nhwehwɛmu a wɔde hu sɛ ebia wowɔ awosu mu nsakrae a ɛde Marfan yare no ba anaa.
- Dwumadi ahorow a ɛde ɔhaw kɛse ba koma no so a wobɛkwati. Sɛ nhwɛso no, agumadi a enya nkɛntɛnso kɛse te sɛ adesoa a wɔma so, rugby, ne bɔɔlbɔ mfata.
- Sɛ aorta no trɛw ma ɛyɛ hu a, wɔyɛ oprehyɛn na amma antetew (dissection). Ɛyɛ papa sɛ wobɛyɛ eyi ho nhyehyɛe ansa na abɛyɛ nea egye ntɛmpɛ.
- Oduruyɛfo no betumi akyerɛw nnuru te sɛ ``Beta-blockers'' anaa ``ARBs''. Eyinom yɛ adwuma denam mogya mmoroso a wɔhwɛ so na ɛtew nhyɛso a ɛwɔ aorta no so no so. Eyi ma sɛnea ntini no trɛw ntɛmntɛm no brɛ ase.
- Aorta no fã a asɛe no a wobeyi afi hɔ na wɔde afiri a wɔde yɛ afiri (graft) asiesie.
- Koma mu ntini a entumi nyɛ adwuma yiye a wɔde besiesie anaasɛ wɔde afiri a wɔde ayɛ adwuma besi ananmu.
- Ɛho hia sɛ wɔyɛ scan te sɛ echocardiogram anyɛ yiye koraa no pɛnkoro afe biara de hwɛ sɛnea aorta no kɛse te. Eyi betumi aboa ma wɔahu ɔhaw biara ntɛm sɛ ɛba a.
- Sɛ aorta no ntwemu boro sɛntimita 5 a.
- Sɛ ntini no trɛw ntɛmntɛm yiye wɔ afe biako mu a.
- Sɛ obi wɔ w’abusua mu wɔ abakɔsɛm a ɛfa aortic dissection ho a.
Sɛ obi wɔ w’abusua mu wɔ saa yare yi anaasɛ ɔwɔ yare no ho sɛnkyerɛnne a ɛte saa ara a, ho asɛm a wobɛka akyerɛ wo duruyɛfo no boa kɛse ma wohu yare no.
Okay, afei dɛn ne ayaresa ahorow a wɔde yɛ eyi?
Wontumi nsa Marfan yare no koraa. Efisɛ ɛyɛ biribi a ɛwɔ yɛn awosu mu. Nanso, ayaresa a eye paa wɔ hɔ a wɔde siw ɔsɛe a ɛde ba koma no so, siw tebea horow a emu yɛ den ano, na ɛma nkurɔfo tra ase sɛnea ɛsɛ. Wobetumi akyekyɛ ayaresa no mu ayɛ no afã atitiriw abien.
| Ayaresa a Ɛnyɛ Oprehyɛn | Oprehyɛn a wɔde sa yare |
|---|---|
Nsakrae a ɛba asetra mu: | Dɛn nti na oprehyɛn ho hia?: |
Nnuru a wɔde yɛ: | Oprehyɛn ahorow: |
Aduruyɛ mu nhwehwɛmu a wɔyɛ no daa: | Wɔkamfo kyerɛ sɛ wɔnyɛ oprehyɛn ma: |
Tebea horow a egye ntɛmpɛ a ɛhwehwɛ sɛ wɔde obi kɔ ayaresabea ntɛm ara! (Kɔkɔbɔ Nsɛnkyerɛnne) .
Saa sɛnkyerɛnne ahorow yi betumi ayɛ sɛnkyerɛnne a ɛkyerɛ sɛ aortic no mu apaapae anaasɛ wɔatwa. Sɛ eyinom mu biara ba a, kɔ Emergency Department (ETU) a ɛbɛn wo no nkyɛn a ɛnntwentwɛn wo nan ase.
| Sɛ wowɔ saa sɛnkyerɛnne ahorow yi a, kɔ ETU ntɛm ara! | |
|---|---|
| - a ewo mpofirim, a wontumi nnyina ano wo koko, akyi, anaa yafunu mu . | - a ɛyɛ den sɛ ɔbɛhome. |
| - a woyera wo nhumu. | - a ɛyɛ mmerɛw anaasɛ ɛyɛ mmerɛw wɔ nipadua no fã bi. |
| - a ɛyɛ fifiri dodo, honam ani a ɛyɛ nwini. | - a ɛyɛ akisikuru ne akisikuru. |
So ebetumi aba sɛ wobɛtra ase yiye wɔ Marfan yare no ho?
Yiw koraa! Mfe bi a atwam no, na nnipa a wɔwɔ saa tebea yi nkwa nna yɛ tiaa. Nanso ɛnnɛ, esiane aduruyɛ a ɛkɔ akyiri, nhwehwɛmu a wɔyɛ no daa, ne oprehyɛn nti, obi a ɔwɔ Marfan yare no betumi atra ase yiye, akosi ne mfe 70 anaa 80 mu mpo, te sɛ onipa a ɔte ase no ara pɛ.
Ade a ɛho hia sen biara ne sɛ wo ne wo duruyɛfo bɛkɔ so adi nkitaho daa, adi n’akwankyerɛ so pɛpɛɛpɛ, ayɛ nhwehwɛmu wɔ bere ano, na wode wo nnuru adi dwuma yiye.
Titiriw no, sɛ ɔbea bi a ɔwɔ Marfan yare no reyɛ nhyehyɛe sɛ obenyinsɛn a, akyinnye biara nni ho sɛ ɛsɛ sɛ ɔkɔ komayare ho oduruyɛfo ne mmea ho oduruyɛfo nkyɛn ansa na wanyinsɛn. Esiane sɛ nhyɛso a ɛba koma no so no dɔɔso wɔ nyinsɛn mu nti, ɛsɛ sɛ wɔhwɛ yiye titiriw.
Ɛdenam tebea yi a wobɛte ase, anammɔn a ɛho hia a wobɛtu, na wode adwene atra ase so no, wubetumi abɔ wo koma ho ban na woatra ase apɔwmuden, akyɛ.
Nkrasɛm a Wɔde Kɔ Fie
- Marfan syndrome yɛ awosu mu yare a ɛma nipadua no ntini a ɛka bom no yɛ mmerɛw.
- Eyi betumi aka koma no ne mogya ntini titiriw, aorta no so nkɛntɛnso kɛse.
- Aortic dilation ne aneurysm ne nsɛnnennen a asiane wom sen biara.
- Ɛho hia sɛ wokɔ oduruyɛfo nkyɛn wɔ bere a wɔahyɛ no mu na wɔyɛ wɔn mfonini te sɛ echocardiogram.
- Kwati dwumadi ahorow a ɛde ɔhaw ba koma no so, te sɛ nneɛma a emu yɛ duru a wɔma so.
- Hwɛ yiye wɔ kɔkɔbɔ nsɛnkyerɛnne te sɛ koko mu yaw a emu yɛ den mpofirim.
- Sɛ wohwɛ wo so yiye na wosa wo yare a, wubetumi atra ase a ɛfata koraa, na woatra ase akyɛ.
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