So wunim awosu mu yare yi a ɛka ahurututu ne mmerɛbo nyinaa? (Alpha-1 Antitrypsin a Ɛnyɛ Den) .

So wo nso wowɔ ɔfe na ɛyɛ den sɛ wobɛhome a akɔ so akyɛ? Ebia wubesusuw sɛ eyi yɛ ade a ɛfata. Nanso ɛtɔ da bi a, ebia ntease a ɛho hia paa wɔ saa sɛnkyerɛnne ahorow yi akyi a yɛntee ho asɛm pii. Ɛnnɛ yɛrebɛka tebea a ɛte saa ho asɛm. Ɛno ne Alpha-1 Antitrypsin a Ɛnyɛ Den, anaa `(Alpha-1 Antitrypsin a Ɛnyɛ Den)`. Nnipa binom nso frɛ no "Alpha-1" tiawa.

Sɛ yɛbɛka no tiawa a, dɛn ne Alpha-1?

Alpha-1 yɛ awosu mu yare a yenya fi yɛn awofo hɔ. Nea ɛba wɔ eyi mu ne sɛ yɛn nipadua no ntumi nyɛ protein a wɔfrɛ no alpha-one antitrypsin `(AAT)`, a ɛyɛ protein a ɛbɔ yɛn ahurututu ho ban a ɛdɔɔso. Susuw saa `AAT` protein yi ho sɛ ɔwɛmfo ma yɛn ahurututu. Sɛ ɔwɛmfo yi yera a, ɛda adi kɛse sɛ yɛn ahurututu bɛsɛe.

Enti, obi a ɔwɔ Alpha-1 tebea no wɔ asiane kɛse mu sɛ obenya ahurututu mu nyarewa, titiriw emphysema (mframa kotoku a ɛwɔ ahurututu no mu a ɛsɛe), cirrhosis (mrɛbo mu akuru), ne panniculitis, honam ani yare a ɛntaa nsi. Ɛtɔ mmere bi a, saa tebea horow yi betumi de nkwa ato asiane mu.

Esiane eyi nti, nnipa binom nso frɛ saa yare yi "genetic COPD".

Ɔkwan bɛn so na tebea yi ba? Hena na ɔwɔ asiane mu kɛse?

Sɛ yebenya Alpha-1 a, ɛsɛ sɛ yenya awosu mu abɔde a ɛnteɛ no fi yɛn awofo baanu no nyinaa hɔ. Sɛ yɛbɛka no tiawa a, awosu mu nkwaadɔm ne akwankyerɛ a ɛkyerɛ sɛnea yɛn nipadua yɛ adwuma. Sɛ nsakrae kakra ba akwankyerɛ ahorow yi mu a, nipadua no dwumadi betumi asesa.

Wɔ Alpha-1 mu no, nkwaadɔm mu nsakrae a ɛba awosu mu abɔde a wɔfrɛ no `SERPINA1` mu no de ɔhaw ahorow ba `AAT` protein no a wɔyɛ mu. Nea ɛde ba ne sɛ, wɔnyɛ `AAT` protein no dodow a ɛdɔɔso, anaasɛ protein a wɔyɛ no nya nsusuwii a ɛnteɛ. Sɛ ɛyɛ nea ɛnteɛ a, saa protein no ntumi mfi mmerɛbo no mu nkɔfa mogya no mu nkɔ ahurututu no mu. Afei saa protein a ɛnteɛ no kɔhyɛ mmerɛbo no mu, na ɛsɛe mmerɛbo no. Afei nso, esiane sɛ protein biara nni hɔ a ɛbɛkɔ ahurututu mu nti, ahurututu no nso yɛ mmerɛw.

Nanso, nnipa binom nya saa awosu mu abɔde a asɛe yi fi ɔwofo biako pɛ (wɔn maame anaa wɔn papa) hɔ. Yɛfrɛ wɔn "asoafo." Ɛwom sɛ wɔn nipadua taa yɛ `AAT` protein a ɛdɔɔso a ɛbɛbɔ wɔn ahurututu ho ban de, nanso wɔda so ara wɔ asiane mu sɛ ahurututu bɛsɛe. Asiane yi yɛ kɛse titiriw sɛ wɔnom sigaret a.

Ɔkwan bɛn so na eyi ka ahurututu ne mmerɛbo?

Sɛ yɛbɛte eyi ase a, momma yɛmfa nhwɛso ketewaa bi.

Yɛn `AAT` protein no yɛ nea wɔyɛ wɔ mmerɛbo mu. Afei ɛfa mogya no mu kɔ ahurututu no mu. Yɛn ahurututu wɔ enzyme bi a wɔfrɛ no `Neutrophil Elastase` a ɛboa ma wɔko tia nyarewa. Fa saa enzyme yi sɛ ɔsraani a ne ho yɛ den yiye na ne ho akokwaw. Ɛsɛe ɔyare mmoawa. Nanso sɛ wɔyɛ wie a, ɛsɛ sɛ obi siw ano. Sɛ ɛnte saa a, efi ase tow hyɛ yɛn ahurututu mu nkwammoaa a ɛte apɔw nso so.

Saa "gyina" ade no, saa "off switch" adwuma no, yɛn `AAT` protein no na ɛyɛ. Ɛte sɛ nea ɔsahene no ka kyerɛ ɔsraani no sɛ, "Okay, ɛno ara dɔɔso, gyae."

Afei, wɔ obi a ɔwɔ Alpha-1 mu no, saa `AAT` ɔsraani panyin yi nni nipadua no mu. Afei obiara nni hɔ a obesiw saa ɔsraani a ne ho yɛ den (`Neutrophil Elastase`) no kwan. Ɛkɔ so tow hyɛ ahurututu no so. Eyi ma protein `Elastin` a ɛwɔ ahurututu mu no sɛe. Saa elastin yi na ɛma mframa kotoku (alveoli) a ɛwɔ ahurututu no mu no tumi yɛ den na ɛyɛ den te sɛ rɔba. Sɛ ɛyera a, mframa kotoku no yɛ mmerɛw na ɛhwe ase te sɛ afiri a mframa fi mu. Eyi ne nea yɛfrɛ no `Emphysema` . Eyi ma ɛyɛ den sɛ ɔbɛhome na wanya mframa pa.

Nea ɛto mmerɛbo no yɛ soronko kakra. Esiane awosu mu sintɔ ahorow bi nti, wɔyɛ `AAT` protein no wɔ ɔkwan a ɛnteɛ so. Te sɛ krataa bi a wɔabobɔw wɔ ɔkwan a ɛnteɛ so. Esiane sɛnea protein no nyɛ papa yi nti, entumi mfi mmerɛbo no mu na ɛkɔhyɛ mmerɛbo no mu. Sɛ protein pii kyekyere saa a, mmerɛbo no sɛe na efi ase yɛ akuru. Wɔfrɛ ɛno sɛ akisikuru .

Dɛn ne Alpha-1 yare no ho sɛnkyerɛnne?

Nsɛnkyerɛnne a Alpha-1 de ba no gu ahorow gyina akwaa a ɛwɔ nipadua no mu a ɛka no so. Eyinom taa te sɛ COPD (Chronic Obstructive Pulmonary Disease) ho sɛnkyerɛnne ahorow.

Ɔkwan bɛn so na wobetumi ahu yare yi?

Ɔkwan titiriw a wɔfa so hu Alpha-1 ne mogya mu nhwehwɛmu . Esiane sɛ yare no ho sɛnkyerɛnne te sɛ nyarewa afoforo nti, ɛtɔ mmere bi a ebetumi agye bere kakra ansa na wɔahu yare no pɛpɛɛpɛ. Sɛ wowɔ mmerɛbo mu sɛnkyerɛnne, anaasɛ wɔahu sɛ wowɔ COPD na wo sɛnkyerɛnne no nkɔ yiye ɛmfa ho ayaresa a, ebia wo duruyɛfo besi gyinae sɛ ɔbɛsɔ wo ahwɛ sɛ wowɔ Alpha-1 anaa.

Sɔhwɛ ahorow a wɔtaa yɛ ne:

• Mogya mu nhwehwɛmu: Wɔbɛsɔ wo mogya mu nhwehwɛmu ahwɛ sɛ protein `AAT` dodow te. Sɛ dodow no sua a, wɔbɛyɛ awosu mu nhwehwɛmu de ahwɛ sɛ wowɔ awosu mu sintɔ bi a ɛfa Alpha-1 ho anaa.
• Mfoniniyɛ mu nhwehwɛmu: Koko mu X-ray anaa CT scan betumi ahu baabi a ahurututu asɛe no kodu ne baabi a asɛe.
• Ahurututu mu dwumadi ho nhwehwɛmu: Ɛwom sɛ eyinom ntumi nhu Alpha-1 tẽẽ de, nanso ebetumi ama wo duruyɛfo anya sɛnea w’ahurututu no reyɛ adwuma yiye no ho adwene.
• Liver Ultrasound anaa FibroScan®: Sɛ wosusuw sɛ mmerɛbo no asɛe a, wɔyɛ saa scan ahorow yi de hwɛ sɛ akuru bi wɔ mmerɛbo no mu anaa.
• Berɛbo mu Nhwehwɛmu: Sɛ mmerɛbo no sɛe kɛse a, wɔfa ntini ketewaa bi fi mmerɛbo no mu na wɔhwehwɛ mu hwɛ sɛnea asɛe no kodu ankasa.

Dɛn ne ayaresa ahorow a wɔde sa Alpha-1?

Ɛwom sɛ wonni aduru pɔtee biara a wɔde sa Alpha-1 yare no de, nanso ayaresa pii wɔ hɔ a ebetumi adi sɛnkyerɛnne ahorow no so, atew akwaa a ɛsɛe so, na ama asetra atu mpɔn.

Ɛsono akwan a wɔfa so sa yare no gyina akwaa a ɛka no so:

Ayaresa a wɔde sa ahurututu no

• Augmentation Therapy: Eyi yɛ aduru pɔtee a wɔde sa Alpha-1. Nea ɛka ho ne sɛ wɔbɛtew `AAT` protein a wogye fi mogya a wɔde ma a wɔwɔ apɔwmuden hɔ no ho na wɔde ma nipadua no denam ntini bi a ɛte sɛ nkyene so. Bere a eyi ntumi nsan nsɛe ɔsɛe a aba dedaw no, ebetumi asiw ahurututu no a ɛbɛsɛe daakye no ano kɛse anaasɛ ebetumi adi so.
• Nnuru: Wɔde nnuru te sɛ nea wɔde home , te sɛ nea ɛma ahurututu mu trɛw ma ayarefo a wɔwɔ COPD na ama ahome ayɛ mmerɛw.
• Oxygen ayaresa: Sɛ oxygen dodow a ɛwɔ mogya no mu no sua a, wɔde oxygen foforo ma denam afiri ketewaa bi a wɔde gu hwene mu anaa ano akataso so.
• Ahurututu mu apɔw-mu-teɛteɛ nhyehyɛe ahorow: Wɔde ntetee ma na ama ahome ayɛ mmerɛw denam ahomegye apɔw-mu-teɛteɛ ne apɔw-mu-teɛteɛ afoforo so.
• Ahurututu a wɔde si ananmu: Sɛ ahurututu no asɛe kɛse a, ebia ɛho behia sɛ wɔde ahurututu a ɛfata ma obi foforo sɛ ɔkwan a etwa to.

Ayaresa a wɔde sa mmerɛbo no

Wɔde sɛnkyerɛnne kwan so sa mmerɛbo a asɛe no. Nanso, ɔkwan biako pɛ a wobetumi afa so asa Alpha-1 mmerɛbo mu yare koraa ne mmerɛbo a wɔde bɛma obi foforo . Sɛ wɔde mmerɛbo a ɛte apɔw si ananmu a, mmerɛbo no fi ase yɛ `AAT` protein a ɛfata.

Dɛn na metumi ayɛ na ama matra ase apɔwmuden wɔ Alpha-1 ho?

Sɛ wohu sɛ wowɔ Alpha-1 mpo a, ɛnyɛ w’asetra awiei. Nneɛma pii wɔ hɔ a wubetumi ayɛ na ama nneɛma a ɛsɛe w’akwaa no ayɛ ketewaa bi.

Sɛ ɛno da nkyɛn a, hwɛ nneɛma yi nso:

• Twe wo ho fi mmeae a nkurɔfo nom sigaret. (Kwati wusiw a wɔde di dwuma) .
• Kwati sɛ wobɛhome nneɛma a epira w’ahurututu te sɛ mfutuma ne nnuru. Sɛ wuhyia nneɛma a ɛtete saa wɔ adwumam a, hyɛ akataso a ɛbɔ wo ho ban.
• Gyae nsa nom koraa anaasɛ to ano hye sɛnea wubetumi biara. Nsã betumi ama mmerɛbo a ɛsɛe no ayɛ kɛse.
• Kwati nnuru a wɔde kum ɛyaw (sɛ nhwɛso no, paracetamol a wobɛnom dodo) anaa nhabannuru afoforo a ebetumi aka mmerɛbo no a wunkɔ wo duruyɛfo nkyɛn.
• Nya vaccinations a ɛho hia nyinaa. Ɛho hia titiriw sɛ wonya nnuru a wɔde bɔ wo ho ban fi nyarewa te sɛ ahurututu mu yare, atiridii (atiridii), COVID-19, ne hepatitis A ne B a ɛka mmerɛbo no ho.
• Hohoro wo nsa mpɛn pii, tew wo ho, na bɔ wo ho ban fi nyarewa ho.
• Sɛ obi wɔ w’abusua mu wɔ Alpha-1 a, ɛyɛ papa sɛ w’ankasa wobɛyɛ sɔhwɛ. Wo ne wo duruyɛfo nka ho asɛm.
• Sɛ wowɔ Alpha-1 anaasɛ woyɛ obi a ɔwɔ ɔyare mmoawa a, ɛho hia yiye sɛ wo ne awosu ho ɔfotufo kasa sɛ woreyɛ nhyehyɛe sɛ wobɛwo mma a.

Bere bɛn na ɛsɛ sɛ wokɔ oduruyɛfo nkyɛn?

• Sɛ wowɔ ahurututu anaa mmerɛbo mu sɛnkyerɛnne a yɛadi kan aka ho asɛm no a.
• Sɛ wɔahu sɛ obi a ɔwɔ w’abusua mu anya Alpha-1 a.
• Sɛ wɔahu sɛ wowɔ COPD anaa ayamtu na wɔasa wo yare, nanso wo sɛnkyerɛnne no nkɔ yiye a, wo ne wo duruyɛfo nkasa mfa Alpha-1 nhwehwɛmu a wobɛyɛ ho.
• Sɛ wowɔ Alpha-1 dedaw a, kɔ wo duruyɛfo nkyɛn ntɛm ara sɛ sɛnkyerɛnne foforo ba anaasɛ sɛnkyerɛnne a ɛwɔ hɔ dedaw no mu yɛ den a.

Alpha-1 a wobɛtra ase no betumi ayɛ den. Nanso sɛ wunya nimdeɛ, ayaresa, ne asetra kwan a ɛfata a, wo nso wubetumi atra ase a ahoɔden wom na ɛyɛ nnam. Ɔkwan a eye sen biara a wobɛfa so ayɛ saa ne sɛ wo ne wo duruyɛfo bɛkasa pefee na woayɛ nhyehyɛe bi a ɛbɛyɛ adwuma ama wo.