Momma yɛnka mogya mu yare a ɛntaa nsi a wɔfrɛ no PNH (Paroxysmal Nocturnal Hemoglobinuria) yi ho asɛm .

Nyarewa bi wɔ hɔ a abu so kɛse wɔ yɛn mu, ne nyarewa a ɛho yɛ na yiye, kyerɛ sɛ, ɛho yɛ na yiye nso wɔ hɔ. Ebia wontee yare a ɛte saa ho asɛm mpo. Ɛnnɛ yɛrebɛka yareɛ a ɛntaa nsi, nanso ɛho hia a ɛfa mogya ho a ɛho hia sɛ wɔdi ho dwuma yie no ho asɛm. Ne din ne Paroxysmal Nocturnal Hemoglobinuria, a yɛfrɛ no PNH tiawa.

Sɛ yɛbɛka no tiawa a, dɛn ne PNH?

PNH nyɛ awosu mu yare, na mmom ɛyɛ yare a wonya fi awofo hɔ anaasɛ wonya fi awofo mu kɔ mmofra mu. Ebetumi anya nkɔso wɔ mfe biara mu. Sɛ yɛbɛka no tiawa a, obi a ɔwɔ saa yare yi wɔ yare bi a nipadua no mu tumi a ɛko tia nyarewa , nhyehyɛe a ɛbɔ yɛn ho ban fi nyarewa ho no di mfomso tow hyɛ wɔn ankasa mogya mu nkwammoaa kɔkɔɔ so na wɔsɛe no. Fa no sɛ ɛte sɛ kyɛm a wɔde protein soronko a ɛbɔ yɛn ho ban fi nyarewa ho ayɛ. Mogya mu nkwammoaa kɔkɔɔ bi a ɛwɔ PNH ayarefo nipadua mu nni saa ahobammɔ kyɛm yi, kyerɛ sɛ, saa protein titiriw no. Enti nipadua no mu tumi a ɛko tia nyarewa no hu saa nkwammoaa yi a enni kyɛm, esusuw sɛ ɛyɛ ntua a epira nipadua no na ɛsɛe wɔn. Ɛwom sɛ tebea yi betumi de wo nkwa ato asiane mu de, nanso sɛ wonya ayaresa a ɛfata a, wubetumi adi sɛnkyerɛnne ahorow no so, atew nsɛnnennen so na woabɔ bra pa. Saa yare yi nka obiara wɔ ɔkwan koro so. Nnipa binom wɔ ɔhaw nketenkete nkutoo. Afoforo nso betumi aka wɔn kɛse. Asiane titiriw ne mogya a ɛbɛtɔ. PNH ayarefo bɛyɛ 30% wɔ asiane mu sɛ anyɛ yiye koraa no wobenya mogya a ɛbɛtɔ pɛnkoro wɔ wɔn asetra mu.

Dɛn nti na ɛte saa?

Nea ɛde yare yi ba ne awosu mu nkwaadɔm mu nsakrae. Nanso eyi nyɛ biribi a wonya fi awo mu. Ɛno kyerɛ sɛ ɛnyɛ biribi a wunya fi w’awofo hɔ, na saa ara nso na ɛnyɛ biribi a wode ma wo mma. Eyi yɛ awosu mu nsakrae a ɛba kwa wɔ nkwa mu. Esiane saa awosu mu nkwaadɔm mu nsakrae yi nti, wo nnompe mu ntini no fi ase yɛ mogya mu nkwammoaa kɔkɔɔ a ɛnyɛ ne kwan so a enni saa protein ahorow a ɛbɔ ho ban no. Enti esiane sɛ nipadua no mu tumi a ɛko tia nyarewa no mmɔ saa nkwammoaa yi ho ban nti, ɛnyɛ den sɛ ebebubu. Wɔ nnuruyɛ mu no, yɛfrɛ eyi a ɛma mogya mu nkwammoaa kɔkɔɔ a ɛpaapae no hemolysis . Nnuruyɛfo binom gye di sɛ PNH ne nnompe mu ntini mu mmerɛwyɛ wɔ abusuabɔ. Titiriw no, nnipa a wɔwɔ tebea bi a wɔfrɛ no aplastic anemia no taa nya PNH. Afei nso, nnipa a wɔwɔ PNH no betumi anya aplastic anemia akyiri yi. Wɔ tebea a wɔfrɛ no aplastic anemia yi mu no, nnompe mu ntini no gyae mogya mu nkwammoaa foforo a ɛyɛ.

Dɛn ne PNH ho sɛnkyerɛnne ahorow?

Wɔde ne sɛnkyerɛnne atitiriw no biako din ato yare no so. Paroxysmal kyerɛ sɛ "ɛba mpofirim", Nocturnal kyerɛ "anadwo", na Hemoglobinuria kyerɛ sɛ "hemoglobin a wɔyi fi nsuo mu". Hemoglobin ne protein a ɛma mogya kɔla kɔkɔɔ. Enti, sɛ mogya mu nkwammoaa kɔkɔɔ no sɛe a, saa hemoglobin yi pue kɔ nsu no mu, na nsu a wɔayi afi mu anadwo anaa anɔpa no betumi ayɛ tuntum (te sɛ cola) anaa kɔkɔɔ . Nnipa a wɔwɔ PNH no mu bɛyɛ 50% nya saa sɛnkyerɛnne yi. Nneɛma abiɛsa titiriw na ɛde sɛnkyerɛnne ahorow no ba:

• Mogya mu nkwammoaa kɔkɔɔ a ɛpaapae.
• Mogya mu nkwammoaa kɔkɔɔ dodow a ɛso tew (a ɛde mogya a ɛtɔ kɔ nipadua mu ba).
• Mogya a ɛtwetwe mogya ntini mu .

Dodow a mogya mu nkwammoaa a asɛe pii wɔ wo nipadua mu no, dodow no ara na ebia wubehu yare no ho sɛnkyerɛnne pii. Sɛ wowɔ adwenem naayɛ ketewaa bi sɛ mogya atɔ wo te sɛ eyi a, .Ka kyerɛ wo duruyɛfo ntɛm ara. Kɔ ayaresabea Emergency Department (ETU) a ɛbɛn wo a wonntwentwɛn wo nan ase, titiriw sɛ ɛyɛ den sɛ wobɛhome, ɛyɛ den sɛ wobɛkasa/ahu ade, wo ti yɛ wo yaw kɛse mpofirim, wo koko yɛ wo yaw, anaasɛ wo ho yɛ wo yaw a.

Ɔkwan bɛn so na wobetumi ahu yare no pɛpɛɛpɛ?

Sɛ wokɔ wo duruyɛfo nkyɛn a, obebisa wo yare no ho sɛnkyerɛnne ne w’ayaresa ho abakɔsɛm. Afei ɔbɛhwehwɛ wo mu na wahyɛ sɛ wɔnyɛ wo mogya mu nhwehwɛmu bi. Nhwehwɛmu titiriw a wɔyɛ ma eyi ne mogya mu nhwehwɛmu titiriw bi a wɔfrɛ no flow cytometry . Eyi na ɛma wo mogya mu nkwammoaa kɔkɔɔ no wɔ protein ahorow a ɛbɔ wo ho ban yi. Nea ɛka ho no, wɔhwɛ wo mogya mu nkwammoaa dodow (mogya dodow a edi mũ) ne dade dodow nso. Ɛtɔ mmere bi a, ebia ɛho behia nso sɛ wɔyɛ nnompe mu ntini a wɔde yɛ nhwehwɛmu.

Dɛn ne ayaresa ahorow a wɔde sa PNH?

Botae atitiriw abien na ɛwɔ PNH ayaresa mu. Biako ne sɛ wɔbɛma yare no ho sɛnkyerɛnne ahorow no afi hɔ, na biako nso ne sɛ wobesiw ɔhaw ahorow a ɛyɛ hu ano (titiriw mogya a ɛbɛtɔ). Ayaresa a wo nsa bɛka no begyina sɛnea wo tebea no mu yɛ den so.

• Aduannuru foforo: Wɔde folic acid ne iron a wɔde ka ho ma de siw mogya a ɛtɔ kɔ nipadua mu ano.
• Nnuru pɔtee: Nnuru bi wɔ hɔ te sɛ Eculizumab (Soliris) ne Ravulizumab (Ultomiris) a esiw mogya mu nkwammoaa kɔkɔɔ a ɛpaapae ano. Wɔde eyinom fa ntini mu ma. Wɔwɔ mfaso a ɛne sɛ wotumi siw mogya a ɛtɔ kɔ nipadua mu no so, ɛmma ɛho nhia sɛ wɔde mogya a wɔde ma wɔ abɔnten so no so tew, na ɛtew asiane a ɛwɔ hɔ sɛ mogya a ɛbɛtɔ no so. Pegcetacoplan (Empaveli) yɛ aduru foforo a wɔde di dwuma de yɛ eyi.
• Mogya a Wɔde Ma: Sɛ mogya a ɛtɔ kɔ nipadua mu no mu yɛ den a, wobetumi anya ahotɔ bere tiaa bi denam mogya a wɔde bɛma afi obi a ɔwɔ apɔwmuden hɔ so.
• Nnuru a ɛma mogya tu: Wɔde nnuru a ɛma mogya tu ma nnipa a asiane kɛse wom sɛ mogya bɛtɔ.
• Bone Marrow Stem Cell Transplant : Eyi nkutoo ne ɔkwan a wɔfa so sa PNH koraa . Nea ɛka eyi ho ne sɛ wobeyi nnompe mu ntini a yare no afi hɔ na wɔde ntini mu nkwammoaa afi obi a ɔwɔ apɔwmuden (a mpɛn pii no ɔyɛ onua) nsam. Nanso, esiane sɛ eyi yɛ adeyɛ a asiane wom kɛse na ɛyɛ den nti, mpɛn pii no wɔkamfo kyerɛ mmabun a wɔwɔ yare a emu yɛ den yiye nkutoo.

Wo duruyɛfo na ɔbɛkyerɛ ayaresa kwan a ɛfata wo kɛse.

Ɛho hia paa nso sɛ wobɛhwɛ wo ho.

Sɛ wo ne PNH te a, ɛho hia sen bere biara sɛ wobɛhwɛ wo ho.

• Aduan pa: Di nnuan a ahoɔden wom. Sɛ wodi nnuan a iron pii wom (spinach, nam) ka biribi a vitamin C pii wom (akutu, tangerines) ho a, ɛboa ma nipadua no twetwe iron yiye.
• Apɔw-mu-teɛteɛ: Ɔbrɛ betumi ama ayɛ den sɛ wobɛteɛteɛ w’apɔw mu. Bisa wo duruyɛfo apɔw-mu-teɛteɛ a ɛfata wo.
• Bɔ wo ho ban fi nyarewa ho : Hohoro wo nsa mpɛn pii. Kwati mmeae a nnipa ahyɛ hɔ ma ne mmeae a ayarefo wɔ. Sɛ wunya atiridii a, ka kyerɛ wo duruyɛfo ntɛm ara.
• Adwene mu akwahosan: Ɛyɛ ade a ɛfata sɛ wubedi awerɛhow, abufuw, ne dadwen bere a wuhu sɛ wowɔ yare bi a ɛntaa nsi te sɛ eyi no. Sɛ wo ne obi a wogye no di anaa ɔfotufo bi bɛka saa nkate horow yi ho asɛm a, ebetumi ama woanya ahotɔ kɛse.
• Nyinsɛn: Sɛ woyɛ ɔbea a woreyɛ nhyehyɛe sɛ wubenyinsɛn a, hwɛ hu sɛ wobɛkɔ wo duruyɛfo nkyɛn ansa na woanyinsɛn. PNH betumi de asiane aba wo ne wo ba no so bere a wonyinsɛn no.

Nkrasɛm a Wɔde Kɔ Fie

• PNH yɛ mogya mu yare a ɛntaa nsi a awosu mu nsakrae na ɛde ba, nanso ɛnyɛ awo mu de.
• Ɔhaw titiriw a ɛyɛ hu sen biara a ɛde yare yi ba ne mogya a ɛtɔ. Hu ne kɔkɔbɔ nsɛnkyerɛnne (tiyɛ a emu yɛ den, koko mu yaw, ahome a ɛyɛ den) bere nyinaa.
• Ɛwom sɛ eyi betumi de nkwa ato asiane mu de, nanso ayaresa a etu mpɔn yiye wɔ hɔ nnɛ a wɔde siw yare no ho sɛnkyerɛnne ano na ama asetra atu mpɔn.
• Ɛwom sɛ nnompe mu ntini a wɔde si ananmu nkutoo ne ayaresa a ɛsa PNH koraa de, nanso asiane kɛse wom na ɛfata ayarefo a wɔapaw wɔn nkutoo.
• Ɛho hia yiye sɛ wo ne wo duruyɛfo di nkitaho daa na wudi n’akwankyerɛ akyi.

Paroxysmal Nocturnal Hemoglobinuria, PNH, mogya mu nyarewa a ɛntaa nsi, mogya mu nkwammoaa kɔkɔɔ, hemoglobinuria, mogya a ɛtɔ kɔ nipadua mu, mogya a ɛtwetwe, PNH sɛnkyerɛnne, PNH ayaresa, nnompe mu ntini a wɔde si ananmu