Ènya nu tso domenyiŋusẽfianu ƒe dɔléle sia si gblẽa nu le lãkusi kple aklã siaa ŋua? (Alpha-1 Antitrypsin ƒe Agbɔsɔsɔme) .

Ðe kɔdzidɔ kple gbɔgbɔ sesẽna hã le ŋuwò si le edzi yim ɣeyiɣi didi aɖea? Ðewohĩ àsusu be esia sɔ. Gake ɣeaɖewoɣi la, susu vevi aɖe ate ŋu anɔ megbe na dzesi siawo si ŋu míese nu geɖe tsoe o. Egbea míele nu ƒom tso nɔnɔme sia tɔgbe ŋu. Emae nye Alpha-1 Antitrypsin ƒe Gbɔdzɔgbɔdzɔ, alo `(Alpha-1 Antitrypsin ƒe Gbɔdzɔgbɔdzɔ)`. Ame aɖewo hã yɔnɛ be "Alpha-1" kpuie.

Kpuie ko la, nukae nye Alpha-1?

Alpha-1 nye domenyiŋusẽfianu ƒe dɔléle si ƒe dome míenyi tso mía dzilawo gbɔ. Nusi dzɔna le esia me enye be míaƒe ŋutilã mete ŋu wɔa protein si woyɔna be alpha-one antitrypsin `(AAT)`, si nye protein si kpɔa míaƒe lãkusiwo ta, si sɔ gbɔ o. Bu `AAT` protein sia be enye dzɔla na míaƒe lãkusiwo. Ne dzɔla sia bu la, anɔ eme be míaƒe lãkusiwo ate ŋu agblẽ.

Eyata amesi ŋu Alpha-1 ƒe nɔnɔme le la ate ŋu axɔ lãkusidɔ geɖe wu, vevietɔ emphysema (ya kotoku siwo le lãkusi me ƒe gbegblẽ), cirrhosis (aklã ƒe abi), kple panniculitis, si nye ŋutigbalẽ ƒe dɔléle si mebɔ o. Ɣeaɖewoɣi la, nɔnɔme siawo ate ŋu ade ame ƒe agbe afɔku me.

Le susu sia ta la, ame aɖewo hã yɔa dɔléle sia be "domenyiŋusẽfianu ƒe COPD".

Aleke nɔnɔme sia dzɔnae? Amekae le afɔku me wu?

Be Alpha-1 nadze ame dzi la, ele be míanyi domenyiŋusẽfianu si mesɔ o la ƒe dome tso mía dzila evea siaa gbɔ. Kpuie ko la, domenyiŋusẽfianuwoe nye mɔfiame siwo fiaa alesi míaƒe ŋutilã wɔa dɔe. Ne tɔtrɔ vi aɖe va le mɔfiame siawo me la, ŋutilãa ƒe dɔwɔwɔ ate ŋu atrɔ.

Le Alpha-1 me la, domenyiŋusẽfianu ƒe tɔtrɔ si woyɔna be `SERPINA1` hea kuxiwo vɛ le `AAT` protein ƒe wɔwɔ me. Esia wɔe be womewɔa `AAT` protein la le agbɔsɔsɔ si sɔ me o, alo protein si wowɔ la xɔa nɔnɔme si mesɔ o. Ne exɔ nɔnɔme si mesɔ o la, protein ma mate ŋu ado le aklãa me ato ʋua me ayi aɖuɖɔtoewo me o. Emegbe protein ma si ƒe nɔnɔme mesɔ o la gena ɖe aklãa me, si gblẽa nu le aklãa ŋu. Azɔ hã, esi protein aɖeke meli si ayi ɖe lãkusi me o ta la, lãkusiwo hã te ŋu gblẽa nu le wo ŋu bɔbɔe.

Gake ame aɖewo nyia domenyiŋusẽfianu sia si gblẽ la ƒe dome tso dzila ɖeka ko gbɔ (wo dada alo wo fofo). Míeyɔa wo be "ametsɔlawo." Togbɔ be zi geɖe la, woƒe ŋutilã wɔa `AAT` protein si sɔ be wòakpɔ woƒe lãkusiwo ta hã la, wogakpɔtɔ le afɔku me be woƒe lãkusiwo nagblẽ. Afɔku sia lolo ŋutɔ ne wono atama.

Aleke esia kpɔa ŋusẽ ɖe lãkusi kple aklã dzii?

Be míase esia gɔme la, mina míawɔ kpɔɖeŋu sue aɖe.

Wowɔa míaƒe `AAT` protein le aklã me. Emegbe ezɔa mɔ to ʋua me yia lãkusiwo me. Enzyme aɖe le míaƒe lãkusiwo me si woyɔna be `Neutrophil Elastase` si kpena ɖe wo ŋu wowɔa avu kple dɔlékuiwo. Bu enzyme sia be enye asrafo sesẽ aɖe si bi ŋutɔ. Egblẽa dɔlékuiwo dome. Gake ne wowɔe vɔ la, ele be ame aɖe natɔ te ewɔwɔ. Ne menye nenema o la, edzea míaƒe lãkusi me lãmenugbagbevi siwo le lãmesẽ me hã dzi dzedze gɔme.

Nu ma "tɔtrɔ", "tsi switch" dɔwɔwɔ ma, nye nusi míaƒe `AAT` protein wɔna. Ele abe aʋafia la gblɔ na asrafoa be, "Enyo, esia sɔ gbɔ, dzudzɔ."

Fifia, le ame si ŋu Alpha-1 le me la, `AAT` dɔwɔla sia mehiã le ŋutilã me o. Ekema ame aɖeke meli si axe mɔ na asrafo sesẽ ma (`Neutrophil Elastase`). Eyi edzi dzea lãkusiwo dzi. Esia nana be protein `Elastin` si le lãkusi me la gblẽna. Elastin siae naa yakotokuwo (alveoli) siwo le lãkusiwo me te ŋu nɔa te sesĩe eye ŋusẽ nɔa wo ŋu abe rababa ene. Ne ebu la, yakotokuawo gbɔdzɔna eye woɖiɖina abe ʋuƒo si me ya le ene. Esiae nye nusi míeyɔna be `Emphysema` . Esia wɔnɛ be gbɔgbɔ kple oxygen xɔxɔ sesẽna.

Nusi dzɔna ɖe aklã dzi la to vovo vie. Le domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔ aɖewo ta la, wowɔa `AAT` protein la le nɔnɔme si mesɔ o me. Abe pepa si woxatsa ɖe mɔ gbegblẽ nu ene. Le nɔnɔme sia si mesɔ o ta la, protein la mete ŋu doa go le aklãa me o eye wòtsia aklãa me. Ne protein si sɔ gbɔ akpa tsi alea la, aklãa gblẽna eye wòdzea abi xɔxɔ gɔme. Woyɔa ema be ʋumenugbagbeviɣidɔ .

Nukae nye Alpha-1 dɔlélea ƒe dzesiwo?

Dzesi siwo Alpha-1 hena vɛ la toa vovo le ŋutinu si le ŋutilã si ŋu wògblẽ nu le nu. Zi geɖe la, esiawo ɖia COPD (Chronic Obstructive Pulmonary Disease) ƒe dzesiwo.

Aleke woawɔ anya dɔléle sia?

Mɔ vevitɔ si dzi woato anya Alpha-1 enye ʋudodokpɔ . Esi wònye be dzesiawo sɔ kple dɔléle bubuwo ta la, ate ŋu axɔ ɣeyiɣi aɖe ɣeaɖewoɣi hafi woate ŋu anya dɔlélea nyuie. Ne aklã ƒe dzesiwo le ŋuwò, alo ne wokpɔ COPD le ŋuwò eye wò dzesiwo mele nyonyom ɖe edzi o togbɔ be wowɔ atike na wò hã la, wò ɖɔkta ate ŋu atso nya me be yeado wò kpɔ be Alpha-1 le ŋuwò hã.

Dodokpɔ siwo wowɔna zi geɖee nye:

• Ʋudodokpɔ: Woado wò ʋu kpɔ be protein `AAT` ƒe agbɔsɔsɔme le eme hã. Ne agbɔsɔsɔa le sue la, woawɔ domenyiŋusẽfianu dodokpɔ akpɔe ɖa be domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔ aɖe si do ƒome kple Alpha-1 le ŋuwò hã.
• Nɔnɔmetatawo dodokpɔ: Akɔta ƒe X-ray alo CT scan ate ŋu anya afisi lãkusia gblẽ le kple afisi wòle.
• Aklã ƒe dɔwɔwɔ dodokpɔ: Togbɔ be esiawo mate ŋu ade dzesi Alpha-1 tẽ o hã la, woate ŋu ana wò ɖɔkta nakpɔ alesi wò aklã le dɔ wɔm nyuie.
• Aklã ƒe Ultrasound alo FibroScan®: Ne wosusu be aklãa gblẽ la, wowɔa foto siawo tsɔ léa ŋku ɖe abi le aklãa ŋu hã.
• Aklãdɔ: Ne aklãa ƒe gbegblẽ nu sẽ la, woɖea lãkusi sue aɖe ŋutɔ tso aklãa me hedoa wo kpɔna be woanya afisi tututu nusi gblẽ le eŋu.

Nukawoe nye atike siwo wozãna na Alpha-1?

Togbɔ be womekpɔ atike aɖeke koŋ na Alpha-1 ƒe dɔlélea o hã la, atikewɔmɔnu geɖe li siwo ate ŋu aɖu dzesiawo dzi, aɖe ŋutinuwo ƒe gbegblẽ dzi akpɔtɔ, eye woana agbenɔnɔ nanyo ɖe edzi.

Atikewɔwɔ ƒe mɔnuwo toa vovo le ŋutinu si ŋu wògblẽ nu le nu:

Atikewɔwɔ na lãkusiwo

• Augmentation Therapy: Esia nye atike tɔxɛ aɖe na Alpha-1. Ebia be woakɔ `AAT` protein si woxɔ tso ʋudola siwo le lãmesẽ me gbɔ eye woatsɔe ana ŋutilã to ʋuka aɖe me, si sɔ kple saline. Togbɔ be esia mate ŋu atrɔ nusiwo gblẽ xoxo o hã la, ate ŋu atɔ te nusiwo agblẽ le lãkusiwo ŋu le etsɔme alo akpɔ wo dzi le akpa gãtɔ me.
• Atikewo: Wonaa atikewo abe atike siwo wogbɔna , abe atike siwo kekea aɖuɖɔtoewo ene, dɔnɔ siwo ŋu COPD le be gbɔgbɔ nanɔ bɔbɔe.
• Oxygen-dɔdamɔnu: Ne oxygen ƒe agbɔsɔsɔ si le ʋua me mesɔ gbɔ o la, wotoa mɔ̃ sue aɖe si wotsɔ dea ŋɔti me alo nutsyɔnu si wotsɔna tsyɔa nu me dzi naa oxygen kpeɖeŋutɔ.
• Aklã ƒe ɖɔɖɔɖo ƒe ɖoɖowo: Wonaa hehe be woana gbɔgbɔ nanɔ bɔbɔe to gbɔgbɔ ƒe kamedede kple kamedede bubuwo me.
• Aɖuɖɔtoe dodo: Ne lãkusia gblẽ vevie la, ate ŋu ahiã be woado lãkusi si le lãmesẽ me abe mɔnu mamlɛtɔ ene.

Atikewɔwɔ na aklã

Wodaa aklã ƒe gbegblẽ le dzesiwo nu. Gake mɔ ɖeka kolia si dzi woato ada Alpha-1 aklãdɔ keŋkeŋ enye aklã dodo na ame . Ne wodo aklã si le lãmesẽ me la, aklãa dzea `AAT` protein si sɔ la wɔwɔ gɔme.

Nukae mate ŋu awɔ be manɔ agbe le lãmesẽ me kple Alpha-1?

Ne wokpɔe be Alpha-1 le ŋuwò hã la, menye wò agbe ƒe nuwuwue wònye o. Nu geɖe li nàte ŋu awɔ atsɔ aɖe nusiwo agblẽ le wò ŋutinuwo ŋu dzi akpɔtɔ.

Le ema ɖeɖeko megbe la, nya nu tso nusiawo hã ŋu:

• Te ɖa le teƒe siwo amewo noa atama le. (Ƒo asa na dzudzɔ si wozãna tsã)
• Ƒo asa na nusiwo gblẽa nu le wò lãkusi ŋu, abe ke kple atikewo ene, gbɔgbɔ. Ne èdo go nu mawo tɔgbe le dɔwɔƒe la, do ametakpɔnu.
• Dzudzɔ ahanono keŋkeŋ alo nàɖo seɖoƒe nɛ alesi nàte ŋui. Ahanono ate ŋu ana aklã ƒe gbegblẽ nadzi ɖe edzi.
• Ƒo asa na vevesesenutsitikewo (e.g., paracetamol nono fũ akpa) alo gbetike bubu siwo ate ŋu agblẽ nu le aklã ŋu ne mèbia wò ɖɔkta o.
• Na atikewɔmɔnu siwo katã hiã. Ele vevie ŋutɔ be woado atike na dɔlélewo abe aklãdɔ, dzamezã (dzamezã), COVID-19, kple aklãdɔléle A kple B, siwo gblẽa nu le aklã ŋu.
• Klɔ asi enuenu, nɔ dzadzɛ, eye nàkpɔ ɖokuiwò ta tso dɔlékuiwo me.
• Ne Alpha-1 le wò ƒomea me la, anyo be wò ŋutɔ nàdoe kpɔ. Ƒo nu tso eŋu kple wò ɖɔkta.
• Ne Alpha-1 le ŋuwò alo nènye amesi tsɔa vi la, ele vevie ŋutɔ be nàƒo nu kple domenyiŋusẽfianu ŋuti aɖaŋuɖola ne èle ɖoɖo wɔm be yeadzi vi.

Ɣekaɣie wòle be nàkpɔ ɖɔkta?

• Ne lãkusi alo aklã ƒe dzesi siwo ŋu míeƒo nu tsoe va yi la le ŋuwò.
• Ne wokpɔ Alpha-1 le wò ƒomea me.
• Ne wokpɔ COPD alo asrãdɔ le ŋuwò hedae nɛ, gake wò dzesiwo mele nyonyom ɖe edzi o la, ƒo nu kple wò ɖɔkta tso Alpha-1 dodokpɔ wɔwɔ ŋu.
• Ne Alpha-1 le ŋuwò xoxo la, yi ɖɔkta gbɔ enumake ne dzesi yeyewo do alo dzesi siwo li xoxo la gagblẽ ɖe edzi.

Alpha-1 ƒe agbenɔnɔ ate ŋu asesẽ. Gake ne sidzedze, atikewɔwɔ, kple agbenɔnɔ nyuitɔ le asiwò la, wò hã àte ŋu anɔ agbe si me lãmesẽ le, si me dzo le. Mɔ nyuitɔ si dzi nàto awɔ esiae nye be nàƒo nu kple wò ɖɔkta gaglãa eye nàto ɖoɖo aɖe si awɔ dɔ na wò vɛ.