Ðe wò aɖuɖɔ do vivitia? Ðe ɖeɖi tea ŋuwò ɣesiaɣia? Ate ŋu anye PNH (Paroxysmal Nocturnal Hemoglobinuria)!

Ðe wò aɖuɖɔ ƒe amadede hã trɔna ne èfɔ le ŋdi me, eye wòtrɔna zua aŋutiɖiɖi, blɔ, alo yibɔ ɣeaɖewoɣia? Alo ɖe ɖeɖi tea ŋuwò ŋutɔ eye ɖeɖi tea ŋuwò le susu aɖeke manɔmee le ŋkekea mea? Esiawo ate ŋu adze abe nu dzɔdzɔe siwo ŋu míeléa ŋku ɖo boo le míaƒe gbesiagbegbenɔnɔ me o ene. Gake ɣeaɖewoɣi la, ehiãna be woalé ŋku ɖe dzesi sue siawo ŋu vie, elabena ɖewohĩ wole lãmesẽkuxi aɖe si mebɔ o gake nu sẽ ŋutɔ la ɣlam. Egbea míele nu ƒom tso dɔléle mawo dometɔ ɖeka si do ƒome kple ʋu, si nye PNH, alo `(Paroxysmal Nocturnal Hemoglobinuria)` ŋu. Togbɔ be ŋkɔa aɖi nya sesẽ vie hã la, mina míase egɔme bɔbɔe.

Nukae nye PNH? Mina míase egɔme bɔbɔe

Eyata nukae nye ``Paroxysmal Nocturnal Hemoglobinuria'' alo PNH? Le nyateƒe me la, enye ʋumenugbagbeviɣidɔ si mebɔ kura o. Ne èlé ŋku ɖe gɔmesese si le ŋkɔa ŋu ŋu la:

• "Paroxysmal" gɔmee nye kpata, si dzɔna zi ɖeka.
• "Zã me" gɔmee nye esi ku ɖe zã ŋu.
• "Hemoglobinuria" nye ʋu me protein si woyɔna be ``Hemoglobin`` ƒe anyinɔnɔ le aɖuɖɔ me. ``Hemoglobin`` siae nana míaƒe ʋu dzena dzĩ. Eyata ne woɖe esia do goe kple aɖuɖɔa la, aɖuɖɔa ƒe amadede doa viviti.

Kpuie ko la, PNH nye nɔnɔme si me wò dɔlélenutsiŋutete dzea wò ŋutɔ wò ʋumenugbagbevi dzĩwo dzi hegblẽa wo . Ðɔktawo yɔa esia be ``Hemolysis`` alo ʋumenugbagbevi dzĩwo ƒe gbagbã. Esia ƒe dzesi vevitɔwo dometɔ ɖekae nye aɖuɖɔ si do viviti, si biã/blɔ ne èle aɖuɖɔ tom le zã me alo ŋdi kanya.

Gake PNH ƒe dzesi ɖeka koe aɖuɖɔ doviviti sia nye. Ne womeda dɔléle sia nyuie o la, ate ŋu ahe nɔnɔme sesẽwo abe ʋusɔgbɔdɔ , ayikudɔ si nɔa anyi didi , alo ʋu ƒe sisi le ʋukawo me vɛ . Dzɔgbenyuietɔe la, atike nyuiwo li fifia siwo xea mɔ na PNH be wòagagblẽ nu le ʋumenugbagbeviwo ŋu o.

Amekae ate ŋu atu esia ɖo?

PNH nye dɔléle si mebɔ kura o. Akɔntabubuwo ɖee fia be ame siwo ade 6 le miliɔn 1 ɖesiaɖe mee wokpɔa dɔléle sia le ƒe sia ƒe. Zi geɖe la, wokpɔa nɔnɔme sia le ŋutsuwo kple nyɔnu siwo xɔ tso ƒe 30 va ɖo ƒe 40 siaa ŋu. Gake wogblɔna hã be nyɔnuwo ate ŋu axɔ PNH vie wu ŋutsuwo.

Nu bubu enye be ɣeaɖewoɣi la, amesiwo ŋu ƒumeŋuɖui ƒe dɔléle bubuwo le, le kpɔɖeŋu me, nɔnɔmewo abe `(Aplastic Anemia)` alo `(Myelodysplastic Syndrome)`, hã ateŋu axɔ PNH emegbe.

Vovototo kae le Hemoglobinuria kple Hematuria dome ?

Èsena le ɖokuiwò me be yetɔtɔ vie ne èse nya eve siawo ( tɔtɔ) .- tɔtɔ, esia nye Tamil, ele be maƒo asa nɛ. Mina magblɔe ake be: Àte ŋu atɔtɔ vie ne èse nya eve siawo.) Àte ŋu atɔtɔ vie ne èse nya eve siawo. Elabena le go eveawo siaa me la, nusi dzɔnae nye be wòdzena abe ʋu ene le aɖuɖɔa me.

• Ʋumenugbagbeviɣidɔ nye ʋumenugbagbevi dzĩwo ƒe anyinɔnɔ le wò aɖuɖɔ me.
• Hemoglobinuria (esiae nye nusi míekpɔna le míaƒe PNH me) nye protein si woyɔna be hemoglobin ƒe anyinɔnɔ le aɖuɖɔ me. Ne ʋumenugbagbevi dzĩwo gblẽ la, ʋumenugbagbevi dzĩ sia dona eye wòdea aɖuɖɔa me.

Kpuie ko la, ɖeka yia lãmenugbagbevi bliboa gbɔ, eye evelia yia nu dzĩ si dona ne lãmenugbagbeviawo me gblẽ la gbɔ.

Nukae hea PNH vɛ? Nukatae esia dzɔna ɖo?

Nusi koŋ gbɔ PNH tsonae nye domenyiŋusẽfianu aɖe ƒe gbɔdzɔgbɔdzɔ le míaƒe ŋutilã me. Domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔ sia gblẽa nu le ʋumenugbagbevi ƒomevi eve, ʋumenugbagbevi dzĩwo kple ʋumenugbagbevi dzĩwo ƒe dɔwɔwɔ ŋu. Esia dzea nudzɔdzɔ siwo kplɔ wo nɔewo ɖo siwo hea kuxiwo vɛ ɖe wo nɔewo yome, eye ɣeaɖewoɣi la, nɔnɔme siwo dea ame ƒe agbe afɔku me gɔ̃ hã dzea egɔme.

Nusianu dzea egɔme tso míaƒe ƒumeŋuɖui me . Ènyaa, ƒumeŋuɖui le abe ʋudɔwɔƒe si le míaƒe ŋutilã me ene. Afi siae wowɔa lãmenugbagbeviwo le. Emegbe lãmenugbagbevi siawo va zua ʋumenugbagbevi dzĩwo, ʋumenugbagbevi ɣiwo, kple ʋumenugbagbevi dzĩwo.

Le PNH me la, domenyiŋusẽfianu si woyɔna be ``PIGA`` ƒe tɔtrɔ alo tɔtrɔ dzɔna le lãmenugbagbevi ɖeka me. Lãmenugbagbevi sia si trɔna la ma eye wòwɔa lãmenugbagbevi siwo gblẽ nenema ke wu. Emegbe lãmenugbagbevi siawo siwo gblẽ la wɔa ʋumenugbagbevi dzĩwo kple ʋumenugbagbevi dzĩ siwo gblẽ.

Aleke domenyiŋusẽfianu ƒe tɔtrɔ sia kpɔa ŋusẽ ɖe ʋumenugbagbevi dzĩwo dzii?

Bu eŋu ale: míaƒe dɔlélenutsiŋutete le abe míaƒe dukɔa ƒe ametakpɔha ene. Woyɔa eƒe ŋusẽ tɔxɛwo dometɔ ɖeka be `Kpekpeɖeŋunaɖoɖo` . Woƒe dɔe nye be woawɔ avu kple dɔlékuiwo kple dɔlékui siwo gena ɖe ŋutilãa me. Ke hã, ɣeaɖewoɣi la, protein siwo le `(Complement System)` sia me dzea mía ŋutɔwo ƒe ʋumenugbagbevi dzĩ siwo le lãmesẽ me dzi dzedze gɔme vodadatɔe.

Protein si le abe akpoxɔnu ene si kpɔa eta la le ʋumenugbagbevi dzĩ si le lãmesẽ me si sɔ la ŋu be wòakpɔ eta tso amedzidzedze mawo me. `PIGA` domenyiŋusẽfianu si míeyɔ va yi kpena ɖe ame ŋu be woawɔ ametakpɔnu sia. Eyata, ne wotrɔ `PIGA` domenyiŋusẽfianu la, mate ŋu awɔ ametakpɔnu sia nyuie o. Emegbe, nusi dzɔnae nye esi:

1. Amedzidzedze kple Gbagbã: Ne wò ``Complement System`` dze ʋumenugbagbevi dzĩ siawo siwo ŋu ametakpɔkpɔ mele o dzi la, lãmenugbagbevi mawo gblẽna bɔbɔe eye wogbãna. ``Hemoglobin`` siwo katã le ʋumenugbagbevi dzĩwo me – si nye protein dzĩ si tsɔa oxygen – dona ɖe ʋua me. Ðɔktawo yɔa esia be ``Free Hemoglobin`` .

2. Dzadzɛnyenye ƒe ɖoɖoa ƒe kpododonu:Zi geɖe la, nusi woyɔna be ``Haptoglobin'' nɔa míaƒe ŋutilã me si léa ʋumenugbagbeviɣi sia si le ablɔɖe me eye wòɖenɛ ɖa. Gake le PNH me la, ʋumenugbagbevi dzĩ siwo me gblẽ ƒe xexlẽme sɔ gbɔ ale gbegbe be dzadzɛnyenye ƒe ƒuƒoƒo sia mete ŋu nɔa edzi yim o.

3. Nitric Oxide ƒe Agbɔsɔsɔ: Ŋutilãa zãa mɔnu bubu aɖe tsɔ kpɔa ʋumenugbagbeviɣi sia si sɔ gbɔ la gbɔ, eye emae nye atike si woyɔna be nitric oxide. Gake esia hã nu yina kabakaba. Ne nitric oxide mesɔ gbɔ ɖe wò ŋutilã me o la, kasia àte ŋu akpɔ ʋuʋudedi sesẽ si me vevesese le le wò dɔgbo ƒe lãmekawo, nuɖuɖutome (mɔ̃ si tso wò ŋɔti me yi wò dɔgbo me), kple akɔta ƒe lãmekawo me.

4. Ʋusɔgbɔdɔ: Le go ɖeka me la, ne ʋumenugbagbevi dzĩwo tsrɔ̃, le go bubu me la, nyaƒoɖeamenu geɖe nɔa ƒumeŋuɖui dzi be wòawɔ ʋumenugbagbevi dzĩ yeyewo. Gake ne womate ŋu awɔ wo le ablaɖeɖe si me wogblẽ wo me o la, ʋumenugbagbevi dzĩ siwo le ŋutilãa me dzi ɖena kpɔtɔna. Esiae nye nusi míeyɔna be ʋusɔgbɔdɔ .

5. Ayikuwo ƒe gbegblẽ: Hemoglobin sia si sɔ gbɔ la gblẽa nu le ayikuwo hã ŋu. Wogblɔ be amesiwo ŋu PNH le ƒe ayikudɔléle si nɔa anyi didi ƒe afɔku dzina ɖe edzi zi gbɔ zi ade .

6. Aɖuɖɔ si do viviti: Mlɔeba la, hemoglobin sia si me woɖea mɔ ɖo la kpɔa ŋusẽ ɖe aɖuɖɔa hã dzi. Ame geɖe siwo ŋu PNH le ate ŋu ade dzesi aɖuɖɔ doviviti ne wole aɖuɖɔ tom le zã me alo ŋdi kanya. Esia le alea elabena hemoglobin ƒe agbɔsɔsɔ si le aɖuɖɔ me dzina ɖe edzi, si wɔnɛ be aɖuɖɔa doa viviti.

Kpuie ko la, le `PIGA` domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔ ta la, ʋumenugbagbevi dzĩwo ƒe ametakpɔnu la bu, eye dɔlélenutsiŋutete ŋutɔ tsrɔ̃a wo, si hea kuxiwo ƒe kɔsɔkɔsɔ vɛ.

Aleke domenyiŋusẽfianu ƒe tɔtrɔ sia kpɔa ŋusẽ ɖe ʋumenugbagbevi dzĩwo dzii?

Ʋumenugbagbevi dzĩwo nye lãmenugbagbevi vevi bubu le míaƒe ʋu me. Wokpena ɖe ame ŋu wòdzudzɔa ʋudodo to ʋu ƒe sisi me. Dzɔdzɔmenugbagbevi siwo trɔna le PIGA domenyiŋusẽfianu me kpɔa ŋusẽ ɖe lãmenugbagbevi siwo wɔa ʋumenugbagbevi dzĩwo kpakple lãmenugbagbevi siwo wɔa ʋumenugbagbevi dzĩwo hã dzi.

Esia nana ʋumenugbagbevi dzĩ siwo gblẽ la ƒoa ʋu geɖe wu alesi wòle dzɔdzɔmee . Esia fia be ʋukawo ate ŋu adzɔ le teƒe siwo mehiã o, le ʋukawo me. Woyɔa esia be `(Thrombosis)` (ʋumenugbagbeviɣidɔ). Esia nye afɔku gã aɖe, elabena ne ʋuka siawo zɔ mɔ yi ahɔhɔ̃, dzi, alo lãkusi me la, woate ŋu ade ame ƒe agbe afɔku me.

Nukae nye PNH ƒe dzesiwo?

Togbɔ be woɖe ŋkɔ PNH tso dzesi ɖeka me (aɖuɖɔ doviviti) hã la, ame geɖe siwo ŋu PNH le la yia ɖɔkta gbɔ gbã le ɖeɖiteameŋu si nu sẽ, si nɔa anyi ɖaa si wɔnɛ be wòsesẽna na wo be woawɔ woƒe gbesiagbedɔwo ta . Esiae nye dzesi si dzena wu.

Dzesi bubuwo ate ŋu anye:

• Yagbɔgbɔ sesẽ (`(Dyspnea)`) .
• Ayikukuxiwo (e.g., aɖuɖɔtoe dzi ɖeɖe kpɔtɔ, afɔ siwo vuvu) .
• Nuɖuɖu mimi sesẽ (Dysphagia) .
• Nuɖuɖutome ƒe Ʋuʋudedi
• Dɔgbo ƒe vevesese
• Akɔtave
• Ŋutsu ƒe gbɔdɔdɔ ƒe dɔmawɔmawɔ nyuie (`(Erectile Dysfunction)`)

Ne dzesi siawo dometɔ ɖeka alo esi wu nenema le ŋuwò, vevietɔ tsɔ kpe ɖe aɖuɖɔ ƒe amadede ƒe tɔtrɔ ŋu la, anyo wu be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo godoo.

Aleke ɖɔktawo wɔa dea dzesi PNH? (Dɔléle ƒe dzesi) .

Ne èyi ɖɔkta gbɔ la, woabia wò dɔlélea ƒe dzesiwo eye woado wò kpɔ. Emegbe ne wosusu be PNH le ŋuwò la, woate ŋu awɔ dodokpɔ aɖewo atsɔ aɖo kpe edzi.

Wo dometɔ vevi siwo le esiawo domee nye:

• Ʋu Xexlẽme Blibo (CBC w/diff): Esia léa ŋku ɖe ʋukuxi bubuwo abe ʋusɔgbɔdɔ kple ʋumenugbagbevi dzĩwo ƒe xexlẽme si bɔbɔ (thrombocytopenia) ene ŋu.
• Basic Metabolic Panel (BMP): Esia léa ŋku ɖe ayiku ƒe dɔwɔwɔ kple ayiku ƒe dɔléle si nɔa anyi didi ƒe dzesiwo ŋu.
• Aɖuɖɔtoe me dzodzro: Esia ate ŋu ade dzesi nuwo abe ʋumenugbagbeviɣi ƒe anyinɔnɔ le aɖuɖɔ me (Hemoglobinuria) kple iron si sɔ gbɔ ɖe aɖuɖɔ me (Hemosiderosis).
• Reticulocyte Xexlẽme: Reticulocytes nye ʋumenugbagbevi dzĩ siwo metsi o. Woxlẽa esiawo be woakpɔe ɖa be ƒumeŋuɖui le ʋumenugbagbevi dzĩ siwo le lãmesẽ me wɔm hã.
• Haptoglobin Dodokpɔ: Protein siae ɖea gbeɖuɖɔ ɖa le ʋumenugbagbevi dzĩ siwo gbã me. Ne ʋumenugbagbevi dzĩwo mesɔ gbɔ o ate ŋu afia be ʋumenugbagbevi dzĩwo ƒe gbagbã fũ akpa.
• Lactate Dehydrogenase (LDH) dodokpɔ: LDH nye enzyme si le ʋumenugbagbevi dzĩwo me. Esia ƒe agbɔsɔsɔ fũ le ʋu me ɖenɛ fiana be ʋumenugbagbevi dzĩwo le tsɔtsrɔ̃m.
• Aklã ƒe Dɔwɔwɔ Dodokpɔ: Dodokpɔ sia dzidzea bilirubin ƒe agbɔsɔsɔ ne ʋumenugbagbevi dzĩwo me gblẽ.

Le dodokpɔ siawo me tsonu nu la, ne ɖikeke bubuwo gali ɖɔktaa le PNH ŋu la, ate ŋu adɔ ʋudodokpɔ tɔxɛ aɖe si woyɔna be ``Flow Cytometry.'' Esiae nye nusi ateŋu ade dzesi ʋumenugbagbevi dzĩ siwo ŋu PNH le pɛpɛpɛ.

Nukawoe nye atike siwo wozãna na PNH?

Tsã la, wobua PNH be enye dɔléle si me afɔku le ŋutɔ eye edada sesẽ. Ɣemaɣi la, atike vevitɔe nye ʋudodo edziedzi be woatsɔ ada ʋusɔgbɔdɔ. Gake zi geɖe la, amesiwo ŋu PNH le nɔa agbe ƒe 10 va ɖo 22 le dɔlélea kpɔkpɔ vɔ megbe. Mɔ ɖeka kolia si dzi woato ada PNH keŋkeŋ enye be woawɔ allogeneic stem cell transplant . Eye menye nusi ame sia ame ate ŋu awɔe nye ema o.

Gake, nɔnɔmea to vovo kura fifia!

Dzɔgbenyuietɔe la, atikewɔmɔnu siwo wɔa dɔ ŋutɔ, siwo woɖo ɖe PNH ŋu, li fifia. Woyɔa esiawo be complement inhibitors.Nusi atike siawo wɔna enye be woxea mɔ na ``Complement System`` be wòagadze ʋumenugbagbevi dzĩwo dzi ahagblẽ wo o.

Numekukuwo ɖee fia be PNH dɔnɔ siwo xɔa atike yeye siawo ate ŋu anɔ agbe si sɔ, abe amesi si PNH mele o ene. Esia nye dzidzedzekpɔkpɔ gã aɖe vavã!

Ðe atikewɔwɔa ƒe nugbegblẽ aɖewo lia?

Ẽ, atike siawo siwo nye `(Complement Inhibitors)` ate ŋu akpɔ nugbegblẽ aɖewo. Le kpɔɖeŋu me, ame aɖewo ate ŋu akpɔ nanewo abe taɖuame kple taɖuame ene. Gake nya nyuiae nye be nugbegblẽ le ame ŋu siawo ƒe akpa gãtɔ nu yina le ŋkeke ʋɛ aɖewo megbe tso esime wodze atikewɔwɔ gɔme. Wò ɖɔkta agblɔ nya geɖe tso esia ŋu na wò.

Ðe woate ŋu axe mɔ ɖe PNH nua? Ðe woate ŋu adaea?

Esi wònye be domenyiŋusẽfianu ƒe tɔtrɔ gbɔe PNH tsona ta la, mɔ aɖeke meli si dzi woato axe mɔ ɖe enu o.

Le atikewɔwɔ gome la, atike yeyewo ``Complement Inhibitor`` ateŋu atɔ te nusiwo PNH gblẽna le ʋumenugbagbevi dzĩwo kple ʋumenugbagbevi dzĩwo ŋu. Esia fia be woate ŋu aɖu dɔlélea dzi. PNH dɔnɔ geɖe ate ŋu ahiã atikewɔwɔ bubuwo na nɔnɔmewo abe ʋusɔgbɔdɔ ene. Abe alesi míegblɔe va yi ene la, togbɔ be ``Stem Cell Transplant`` ateŋu adzɔ hã la, enye atikewɔwɔ si sesẽ ŋutɔ. Ne wozã atike siwo li fifia la, woate ŋu akpɔ dɔlélea gbɔ nyuie eye amewo ate ŋu anɔ agbe si sɔ.

Aleke nèléa be na ɖokuiwò abe amesi ŋu PNH le ene?

Le atikewɔwɔ gɔ̃ hã me la, ele vevie ŋutɔ be nàɖe afɔ aɖewo atsɔ axe mɔ ɖe ʋu ƒe sisi nu ne PNH le ŋuwò. Le kpɔɖeŋu me:

• Ne ele be woawɔ dɔ na ame aɖe si ŋu PNH le la , afɔku li be ʋu nado eye ʋu nado le eme fũ le dɔwɔwɔ nɛ me. Eya ta ɖɔktawo léa ŋku ɖe esia ŋu vevietɔ.
• Fufɔfɔ dea lãmesẽ afɔku me na nyɔnu siwo ŋu PNH le, na woawo ŋutɔ kple wo vi si le dɔ me siaa. Eyata ele vevie be woanɔ eŋutinunyala bibi aɖe ƒe dzikpɔkpɔ te do ŋgɔ na fufɔfɔ kple le eƒe ɣeyiɣi katã me.

Wò ɖɔkta aɖo aɖaŋu na wò le tɔtrɔ siwo wòle be nàwɔ le wò agbenɔnɔ me, nusiwo ŋu wòle be nàlé ŋku ɖo ( attention , esia nye Sanskrit/Malayalam, ele be maƒo asa nɛ. Mina magblɔe ake: tɔtrɔ siwo wòle be nàwɔ le wò agbenɔnɔ me, nusiwo wòle be nàlé ŋku ɖe wo ŋu.) tɔtrɔ siwo wòle be nàwɔ le wò agbenɔnɔ me, nusiwo wòle be nàlé ŋku ɖe wo ŋu. Ele vevie ŋutɔ be míawɔ ɖe aɖaŋuɖoɖo ma dzi.

Mlɔeba la, nu vevitɔ kekeake (Take-Home Message) .

PNH, alo ``Paroxysmal Nocturnal Hemoglobinuria'', nye ʋumenugbagbeviɣidɔ si mebɔ o gake ate ŋu anye nu sesẽ. Anɔ abe ƒe 20 enye sia la, atikewɔmɔnu ʋɛ aɖewo koe nɔ dɔnɔ siwo ŋu dɔlélea le si eye kakaɖedzi menɔ wo si le etsɔme o.

Gake egbea la, nɔnɔmea to vovo kura! Le atikewɔwɔ yeye siwo li egbea ta la, amesiwo ŋu PNH le ate ŋu akpɔ woƒe ʋumenugbagbeviwo ta, aƒo asa na kuxi sesẽwo, eye woanɔ agbe si me dzidzeme le, le lãmesẽ me.

Ne wokpɔe be PNH le ŋuwò la, mègatsi dzodzodzoe o. Wò ɖɔkta aɖe atikewɔmɔnu siwo nyo na wò la me na wò. Togbɔ be atikewɔwɔ medaa PNH keŋkeŋ o hã la, ate ŋu akpe ɖe ŋuwò nàxe mɔ ɖe lãmesẽkuxi sesẽ siwo dɔlélea hena vɛ nu eye wòakpe ɖe ŋuwò nànɔ agbe le lãmesẽ me. Nu vevitɔe nye be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo kaba ne dzesiwo le ŋuwò eye nàwɔ ɖe atikewɔwɔa dzi pɛpɛpɛ abe alesi wogblɔe na wò ene.

` PNH, Paroxysmal Nocturnal Hemoglobinuria, Ʋudɔlélewo, Hemoglobinuria, Ʋusɔgbɔdɔ, Ʋu ƒe Ʋuʋu, Ayikudɔlélewo