So wo nsu mu yɛ tumm? So woabrɛ bere nyinaa? Ebetumi ayɛ PNH (Paroxysmal Nocturnal Hemoglobinuria)!

So wo nsu kɔla nso sesa bere a wosɔre anɔpa, na ɛdan kɔkɔɔ tuntum, bruu, anaa ɛtɔ mmere bi a tuntum? Anaasɛ wote nka sɛ woabrɛ paa na woabrɛ a ntease biara nnim da mũ no nyinaa? Ebia eyinom bɛyɛ te sɛ nneɛma a ɛfata a yɛmfa yɛn adwene nsi so kɛse wɔ yɛn da biara da asetra mu. Nanso, ɛtɔ mmere bi a, ɛsɛ sɛ wɔde wɔn adwene si sɛnkyerɛnne nketenkete yi so kakra, efisɛ ebia ɛde akwahosan tebea bi a wɔntaa nhu nanso emu yɛ den asie. Ɛnnɛ yɛrebɛka tebea biako a ɛte saa a ɛfa mogya ho, PNH, anaa `(Paroxysmal Nocturnal Hemoglobinuria)` ho asɛm. Ɛwom sɛ ebia edin no bɛyɛ te sɛ nea ɛyɛ den kakra de, nanso momma yɛnte ase kɛkɛ.

Dɛn ne PNH? Momma yɛnte ase tiawa

Enti, dɛn ne ``Paroxysmal Nocturnal Hemoglobinuria'' anaa PNH? Nokwarem no, ɛyɛ mogya mu yare a ɛntaa nsi koraa. Sɛ wohwɛ nea edin no kyerɛ a:

• "Paroxysmal" kyerɛ sɛ mpofirim, ɛba mpofirim.
• "Anadwo" kyerɛ sɛ ɛfa anadwo ho.
• "Hemoglobinuria" yɛ mogya mu protein bi a wɔfrɛ no ``Hemoglobin`` a ɛwɔ nsuo mu. Saa ``Hemoglobin`` yi na ɛma yɛn mogya yɛ kɔkɔɔ. Enti, sɛ eyi ne nsu no fi mu a, nsu no kɔla yɛ tuntum.

Sɛ yɛbɛka no tiawa a, PNH yɛ tebea a ɛma wo nipadua mu tumi a ɛko tia nyarewa no tow hyɛ w’ankasa mogya mu nkwammoaa kɔkɔɔ so na ɛsɛe . Nnuruyɛfo frɛ eyi ``Hemolysis`` anaasɛ mogya mu nkwammoaa kɔkɔɔ a ɛpaapae. Eyi ho sɛnkyerɛnne titiriw biako ne nsu a ɛyɛ tuntum, kɔkɔɔ/biribiri bere a woretow nsu anadwo anaa anɔpatutuutu no.

Nanso, saa nsu a ɛyɛ tumm yi yɛ PNH ho sɛnkyerɛnne biako pɛ. Sɛ wɔansa tebea yi yiye a, ebetumi de tebea horow a emu yɛ den te sɛ mogya a ɛtɔ kɔ nipadua mu , asaabo mu yare a enni sabea , anaa mogya a ɛbɛtɔ mogya ntini mu aba . Nea eye ne sɛ, mprempren nnuru a etu mpɔn wɔ hɔ a ɛmma PNH nsɛe mogya mu nkwammoaa.

Hena na obetumi anya eyi?

PNH yɛ yare a ɛntaa nsi koraa. Sɛnea akontaabu kyerɛ no, nnipa ɔpepem 1 biara mu bɛyɛ 6 na wohu yare yi foforo afe biara. Mpɛn pii no, wohu tebea yi wɔ mmarima ne mmea a wɔadi fi mfe 30 kosi 40. Nanso, wɔsan ka sɛ mmea betumi anya PNH kakra sen mmarima.

Ade foforo ne sɛ ɛtɔ da bi a nnipa a wɔwɔ nnompe mu yare afoforo, sɛ nhwɛso no, tebea horow te sɛ `(Aplastic Anemia)` anaa `(Myelodysplastic Syndrome)`, nso betumi anya PNH akyiri yi.

Nsonsonoe bɛn na ɛda Hemoglobinuria ne Hematuria ntam ?

Wote nka sɛ w’adwene tu fra kakra bere a wote saa nsɛmfua abien yi ( confusion) .- adwene mu naayɛ, wei yɛ Tamil, ɛsɛ sɛ mekwati. Ma mensan nkyerɛkyerɛ mu bio: Ebia w’adwene betu afra kakra bere a wote saa nsɛmfua abien yi.) Ebia w’adwene betu afra kakra bere a wote saa nsɛmfua abien yi. Efisɛ wɔ nsɛm abien no nyinaa mu no, nea ɛba ne sɛ ɛte sɛ mogya wɔ nsu no mu.

• `(Hematuria)` ‍ .
• Hemoglobinuria (eyi ne nea yehu wɔ yɛn PNH mu) yɛ protein bi a wɔfrɛ no hemoglobin a ɛwɔ nsu mu. Sɛ mogya mu nkwammoaa kɔkɔɔ no sɛe a, hemoglobin yi fi adi na ɛde ka nsu no ho.

Sɛ yɛbɛka no tiawa a, biako kɔ nkwammoaa no nyinaa mu, na biako nso kɔ ade kɔkɔɔ a efi mu ba bere a nkwammoaa no asɛe no mu.

Dɛn na ɛde PNH ba? Dɛn nti na eyi ba?

Nea ɛde PNH ba titiriw ne sintɔ bi a ɛwɔ awosu mu abɔde bi mu wɔ yɛn nipadua mu. Saa awosu mu nkwaadɔm mu sintɔ yi ka mogya mu nkwammoaa ahorow abien, mogya mu nkwammoaa kɔkɔɔ ne mogya mu nkwammoaa a ɛma mogya tu no dwumadi. Eyi fi nsɛm a ɛtoatoa so a ɛde ɔhaw ahorow ba nnidiso nnidiso, na ɛtɔ mmere bi mpo a ɛde tebea horow a ɛde nkwa to asiane mu ba.

Ne nyinaa fi ase wɔ yɛn nnompe mu ntini mu . Wunim, nnompe mu ntini te sɛ mogya adwumayɛbea a ɛwɔ yɛn nipadua mu no. Ɛha na wɔyɛ ntini mu nkwammoaa. Akyiri yi saa ntini mu nkwammoaa yi nyin bɛyɛ mogya mu nkwammoaa kɔkɔɔ, mogya mu nkwammoaa fitaa, ne mogya mu nkwammoaa kɔkɔɔ.

Wɔ PNH mu no, nkwaadɔm mu nsakrae anaa nsakrae a ɛba awosu mu abɔde bi a wɔfrɛ no ``PIGA`` mu ba wɔ ntini mu nkwammoaa biako mu. Saa ntini mu nkwammoaa a wɔayɛ no foforo yi mu paapae na ɛma ntini mu nkwammoaa a ɛtɔ sin saa ara. Afei saa ntini mu nkwammoaa a asɛe yi ma mogya mu nkwammoaa kɔkɔɔ ne mogya mu nkwammoaa a asɛe.

Ɔkwan bɛn so na awosu mu nsakrae yi ka mogya mu nkwammoaa kɔkɔɔ?

Susuw ho saa kwan yi so: yɛn nipadua mu tumi a ɛko tia nyarewa no te sɛ yɛn man no ahobammɔ asraafo. Wɔfrɛ ne tumi titiriw no mu biako `Complement System` . Wɔn adwuma ne sɛ wɔbɛko atia mmoawa ne nyarewa a ɛhyɛn nipadua no mu. Nanso, ɛtɔ da bi a protein ahorow a ɛwɔ saa `(Complement System)` yi mu no fi ase tow hyɛ yɛn ankasa mogya mu nkwammoaa kɔkɔɔ a ɛwɔ apɔwmuden no so wɔ mfomso mu.

Mogya mu nkwammoaa kɔkɔɔ a ɛteɛ na ɛte apɔw wɔ protein a ɛte sɛ kyɛm a ɛbɔ ho ban a ɛbɔ ho ban fi ntua a ɛtete saa ho. `PIGA` awosu mu abɔde a yɛadi kan aka ho asɛm no boa ma wɔyɛ saa ahobammɔ kyɛm yi. Enti, sɛ wɔyɛ `PIGA` gene no mu nsakrae a, entumi nyɛ saa ahobammɔ kyɛm yi yiye. Afei, nea ɛba ni:

1. Ntua ne Ɛpaapae: Sɛ wo ``Complement System`` tow hyɛ mogya mu nkwammoaa kɔkɔɔ a enni ahobammɔ yi so a, ɛnyɛ den sɛ saa nkwammoaa no bɛsɛe na abubu. ``Hemoglobin`` a ɛwɔ mogya mu nkwammoaa kɔkɔɔ no mu nyinaa – kyerɛ sɛ, protein kɔkɔɔ a ɛde oxygen kɔ – no kɔ mogya no mu. Nnuruyɛfo frɛ eyi ``Free Hemoglobin`` .

2. Ahotew nhyehyɛe no huammɔdi:Mpɛn pii no, yɛn nipadua wɔ ade bi a wɔfrɛ no ``Haptoglobin'' a ɛkyere na eyi hemoglobin a enni mu yi fi hɔ. Nanso wɔ PNH mu no, mogya mu nkwammoaa kɔkɔɔ dodow a ɛpaapae no dɔɔso araa ma saa kuw a wosiesie hɔ yi ntumi nkɔ so.

3. Nitric Oxide a Ɛho Nni: Nipadua no fa ɔkwan foforo so di hemoglobin a ɛboro so yi ho dwuma, na ɛno ne nnuru bi a wɔfrɛ no nitric oxide. Nanso eyi nso sae ntɛmntɛm. Sɛ wo nipadua no nni nitric oxide a ɛdɔɔso a, wubetumi anya akisikuru a emu yɛ den a ɛyɛ yaw mpofirim wɔ wo yafunu mu ntini, wo esophagus (afiri a efi wo menewam kɔ ​​wo yafunu mu), ne w’akyi ntini mu.

4. Mogya a ɛtɔ mogya mu: Ɔkwan biako so no, sɛ mogya mu nkwammoaa kɔkɔɔ sɛe a, ɔkwan foforo so no, nhyɛso kɛse ba nnompe mu ntini no so ma ɛyɛ mogya mu nkwammoaa kɔkɔɔ foforo. Nanso, sɛ wontumi nyɛ no ntɛmntɛm sɛnea wɔsɛe no no ara pɛ a, mogya mu nkwammoaa kɔkɔɔ dodow a ɛwɔ nipadua no mu no so tew. Eyi ne nea yɛfrɛ no mogya a ɛtɔ kɔ nipadua mu .

5. Asaabo a ɛsɛe: Saa hemoglobin a ɛboro so yi nso sɛe asaabo no. Wɔkyerɛ sɛ nnipa a wɔwɔ PNH no wɔ asiane a ɛkɔ soro mpɛn asia sɛ wobenya asaabo mu yare a enni sabea .

6. Nsu a ɛyɛ sum: Awiei koraa no, saa hemoglobin a enni mu yi nso ka nsu no. Ebia nnipa pii a wɔwɔ PNH behu sɛ nsu a ɛyɛ tumm bere a wɔtow nsu anadwo anaa anɔpatutuutu no. Nea enti a ɛte saa ne sɛ hemoglobin dodow a ɛwɔ nsu mu no kɔ soro, na ɛma nsu no yɛ tuntum.

Sɛ yɛbɛka no tiawa a, esiane sintɔ a ɛwɔ `PIGA` awosu mu abɔde mu nti, mogya mu nkwammoaa kɔkɔɔ kyɛm a ɛbɔ ho ban no yera, na nipadua no mu tumi a ɛko tia nyarewa no ankasa sɛe, na ɛde ɔhaw ahorow a ɛtoatoa so ba.

Ɔkwan bɛn so na awosu mu nsakrae yi ka mogya nkwammoaa a ɛma mogya tu?

Nkwammoaa a ɛma mogya tu yɛ nkwammoaa foforo a ɛho hia wɔ yɛn mogya mu. Wɔboa ma mogya tu no gyae denam mogya a ɛma mogya tu no so. Nsakrae a ɛba PIGA awosu mu nkwaadɔm mu no ka ntini mu nkwammoaa a ɛyɛ mogya mu nkwammoaa kɔkɔɔ ne ntini mu nkwammoaa a ɛyɛ mogya nkwammoaa no nso.

Eyi ma mogya nkwammoaa a asɛe no yɛ mogya a ɛtɔ kɛse sen sɛnea ɛte daa . Eyi kyerɛ sɛ mogya a ɛyɛ den betumi ayɛ wɔ mmeae a ɛho nhia, wɔ mogya ntini mu. Wɔfrɛ eyi `(Thrombosis)` (thrombosis). Eyi yɛ nea asiane wom kɛse, efisɛ sɛ mogya a ɛtwetwe yi kɔ amemene, koma, anaa ahurututu mu a, ebetumi de nkwa ato asiane mu.

Dɛn ne PNH ho sɛnkyerɛnne ahorow?

Ɛwom sɛ wonya edin PNH no fii sɛnkyerɛnne biako mu (nsu a ɛyɛ sum) de, nanso nnipa pii a wɔwɔ PNH di kan kɔ oduruyɛfo nkyɛn esiane ɔbrɛ a ɛtra so a ɛkɔ so daa a ɛma ɛyɛ den ma wɔn sɛ wɔbɛyɛ wɔn da biara da dwumadi ahorow nti . Eyi ne sɛnkyerɛnne a ɛtaa ba.

Nsɛnkyerɛnne afoforo betumi ayɛ:

• Ɔhome a ɛyɛ den (`(Dyspnea)`) .
• Asaabo mu haw (sɛ nhwɛso no, nsu a ɛtɔ so tew, nan a ɛho ahonhon) .
• Aduan a ɛyɛ den sɛ wobɛmene (Dysphagia) .
• Esophageal Spasms a Ɛyɛ Fɛ
• Yafunu mu yaw
• Akyi yaw
• Mmarima nna mu ɔhaw (`(Erectile Dysfunction)`) .

Sɛ wowɔ saa sɛnkyerɛnne ahorow yi mu biako anaa nea ɛboro saa, titiriw ne nsakrae a ɛba wo nsu mu kɔla mu a, eye sɛ akyinnye biara nni ho sɛ wobɛhwehwɛ aduruyɛfo afotu.

Ɔkwan bɛn so na nnuruyɛfo hu PNH? (Nyare a Wɔahu) .

Sɛ wokɔ oduruyɛfo nkyɛn a, wobebisa wo wo yare no ho sɛnkyerɛnne na wɔahwɛ wo. Afei, sɛ wosusuw sɛ wowɔ PNH a, ebia wɔbɛyɛ nhwehwɛmu ahorow bi de asi so dua.

Eyinom mu atitiriw ne:

• Mogya Dodow a Edi Mu (CBC w/diff): Eyi hwɛ sɛ mogya mu haw afoforo te sɛ mogya a ɛtɔ kɔ nipadua mu ne mogya nkwammoaa dodow a ɛba fam (thrombocytopenia).
• Basic Metabolic Panel (BMP): Eyi hwɛ asaabo dwumadi ne asaabo mu yare a enni sabea ho sɛnkyerɛnne ahorow.
• Nsu mu nhwehwɛmu: Eyi tumi hu nneɛma te sɛ hemoglobin a ɛwɔ nsu mu (Hemoglobinuria) ne dade a ɛboro so a ɛwɔ nsu mu (Hemosiderosis).
• Reticulocyte Dodow: Reticulocytes yɛ mogya mu nkwammoaa kɔkɔɔ a ennyinii. Wɔkan eyinom de hwɛ sɛ nnompe mu ntini no reyɛ mogya mu nkwammoaa kɔkɔɔ a ɛte apɔw anaa.
• Haptoglobin Sɔhwɛ: Saa protein yi na ɛyi nneɛma fĩ fi mogya mu nkwammoaa kɔkɔɔ a abubu mu. Sɛ mogya mu nkwammoaa kɔkɔɔ no sua a, ebetumi akyerɛ sɛ mogya mu nkwammoaa kɔkɔɔ no asɛe dodo.
• Lactate Dehydrogenase (LDH) sɔhwɛ: LDH yɛ enzyme bi a wohu wɔ mogya mu nkwammoaa kɔkɔɔ mu. Eyi dodow a ɛkɔ soro wɔ mogya mu no kyerɛ sɛ mogya mu nkwammoaa kɔkɔɔ no resɛe.
• Mmɛn Dwumadi Ho Nhwehwɛmu: Saa nhwehwɛmu yi kyerɛ bilirubin dodow bere a mogya mu nkwammoaa kɔkɔɔ no mu apaapae no.

Gyina nea efii nhwehwɛmu ahorow yi mu bae so no, sɛ oduruyɛfo no nya adwenem naayɛ foforo wɔ PNH ho a, obetumi ahyɛ sɛ wɔnyɛ mogya mu nhwehwɛmu titiriw bi a wɔfrɛ no ``Flow Cytometry.'' Eyi ne nea ebetumi ahu mogya mu nkwammoaa kɔkɔɔ a ɛwɔ PNH no pɛpɛɛpɛ.

Dɛn ne ayaresa ahorow a wɔde sa PNH?

Bere bi a atwam no, na wobu PNH sɛ ɛyɛ yare a ɛyɛ hu yiye na ɛyɛ den sɛ wɔbɛsa. Saa bere no, na ayaresa titiriw ne mogya a wɔde ma daa de sa mogya a ɛtɔ kɔ nipadua mu. Nanso, mpɛn pii no, nnipa a wɔwɔ PNH no tra ase mfe 10 kosi 22 bere a wɔahu sɛ wɔanya bi no. Ɔkwan biako pɛ a na wobetumi afa so asa PNH koraa ne sɛ wɔbɛyɛ allogeneic stem cell transplant . Na ɛno nyɛ biribi a obiara betumi ayɛ.

Nanso, tebea no yɛ soronko koraa mprempren!

Nea eye ne sɛ, mprempren ayaresa ahorow a etu mpɔn yiye, a wɔde wɔn ani asi so wɔ hɔ a wɔde sa PNH. Wɔfrɛ eyinom sɛ complement inhibitors.Nea saa nnuru yi yɛ ne sɛ esiw ``Copplement System`` no kwan sɛ ɛbɛtow ahyɛ mogya mu nkwammoaa kɔkɔɔ so na asɛe no.

Nhwehwɛmu ahorow ada no adi sɛ PNH ayarefo a wonya ayaresa foforo yi betumi atra ase sɛnea ɛsɛ, te sɛ obi a onni PNH no ara pɛ. Eyi yɛ ade kɛse a wɔatumi ayɛ ampa!

So ayaresa no mu nsunsuanso bɔne bi wɔ hɔ?

Yiw, saa `(Complement Inhibitors)` nnuru yi betumi anya nsunsuanso bɔne bi. Sɛ nhwɛso no, ebia nnipa binom behu nneɛma te sɛ tiyɛ ne akisikuru. Nanso, asɛmpa no ne sɛ saa nsunsuanso bɔne yi mu dodow no ara brɛ ase wɔ nna kakraa bi akyi bere a wɔafi ayaresa ase no. Wo duruyɛfo bɛka eyi ho nsɛm pii akyerɛ wo.

So wobetumi asiw PNH ano? So wobetumi asa no yare?

Esiane sɛ awosu mu nsakrae na ɛde PNH ba nti, ɔkwan biara nni hɔ a wɔbɛfa so asiw ano.

Wɔ ayaresa ho no, nnuru foforo ``Complement Inhibitor`` betumi agyae mogya mu nkwammoaa kɔkɔɔ ne mogya nkwammoaa a ɛsɛe a PNH de ba no. Eyi kyerɛ sɛ wobetumi adi yare no so. Ebia PNH ayarefo pii behia ayaresa foforo ama tebea horow te sɛ mogya a ɛtɔ kɔ nipadua mu. Sɛnea yɛadi kan aka no, ɛwom sɛ ``Stem Cell Transplant`` betumi aba de, nanso ɛyɛ ayaresa a ɛyɛ den yiye. Sɛ wɔde nnuru a ɛwɔ hɔ seesei di dwuma a, wobetumi adi yare no ho dwuma yiye na nkurɔfo atumi atra ase sɛnea ɛsɛ.

Wobɛyɛ dɛn ahwɛ wo ho sɛ obi a ɔwɔ PNH?

Sɛ wowɔ ayaresa mpo a, ɛho hia paa sɛ woyɛ nneɛma bi de siw mogya a ɛbɛtɔ wo ano sɛ wowɔ PNH a. Nhwɛsoɔ:

• Sɛ ɛsɛ sɛ wɔyɛ obi a ɔwɔ PNH oprehyɛn , asiane wɔ hɔ sɛ mogya bɛtɔ na mogya atɔ no dodo bere a wɔreyɛ no oprehyɛn no. Enti, nnuruyɛfo hwɛ yiye titiriw wɔ eyi ho.
• Nyinsɛn de akwahosan ho asiane ba mmea a wɔwɔ PNH so, ma wɔn ankasa ne wɔn ba a wɔhyɛ yafunu mu nyinaa. Enti, ɛho hia sɛ wobɛtra oduruyɛfo titiriw bi hwɛ ase ansa na woanyinsɛn ne bere nyinaa.

Wo duruyɛfoɔ bɛtu wo fo wɔ nsakraeɛ a ɛsɛ sɛ woyɛ wɔ w’asetena mu, nneɛma a ɛsɛ sɛ wode w’adwene si so ( attention , this is Sanskrit/Malayalam, should I avoid it. Ma mensan nkyerɛkyerɛ mu bio: nsakraeɛ a ɛsɛ sɛ woyɛ wɔ w’asetena mu, nneɛma a ɛsɛ sɛ wode w’adwene si so.) nsakraeɛ a ɛsɛ sɛ woyɛ wɔ w’asetena mu, nneɛma a ɛsɛ sɛ wode w’adwene si so. Ɛho hia paa sɛ yedi saa afotu no akyi.

Awiei koraa no, ade a ɛho hia sen biara (Take-Home Message) .

PNH, anaa ``Paroxysmal Nocturnal Hemoglobinuria'', yɛ mogya mu yare a ɛntaa nsi nanso ebetumi ayɛ kɛse. Bɛyɛ mfe 20 a atwam ni no, na wɔn a wɔyare no nni ayaresa a wobetumi apaw, na na daakye a wontumi nsi pi.

Nanso ɛnnɛ, tebea no yɛ soronko koraa! Esiane nnɛyi ayaresa ahorow nti, nnipa a wɔwɔ PNH no betumi akora wɔn mogya mu nkwammoaa so, akwati nsɛnnennen a emu yɛ den, na wɔatra ase a edi mũ na apɔwmuden wom.

Sɛ wohu sɛ wowɔ PNH a, mma wo ho nnyɛ wo hu. Wo duruyɛfo bɛkyerɛkyerɛ ayaresa a eye ma wo mu. Ɛwom sɛ ayaresa ntumi nsa PNH koraa de, nanso ebetumi aboa ma woasiw akwahosan ho haw a emu yɛ den a yare no de ba no ano na aboa wo ma woanya asetra pa. Ade a ɛho hia sen biara ne sɛ wobɛhwehwɛ aduruyɛfo afotu ntɛm ara sɛ wowɔ sɛnkyerɛnne ahorow a, na wudi ayaresa no akyi sɛnea wɔakyerɛ wo no pɛpɛɛpɛ.

` PNH, Paroxysmal Nocturnal Hemoglobinuria, Mogya mu Nyarewa, Hemoglobinuria, Mogya a Wɔyare, Mogya a Ɛtwetwe, Asaabo mu Nyarewa