So wo ba no ho yɛ fitaa na ɔbrɛ bere nyinaa? So ebetumi aba sɛ ɛyɛ Thalassemia?

So ɛte sɛ nea wo ba kumaa no brɛ bere nyinaa? So ontu mmirika nkɔdi agoru te sɛ mmofra afoforo? So ɔwɔ ɔpɛ a emu yɛ den sɛ obedidi? So wate nka pɛn sɛ ne ho ayɛ kusuu? Ɛyɛ ade a ɛfata sɛ ɛna anaa agya biara bɛte ehu nka kɛse bere a ohu nneɛma a ɛte sɛɛ no. Bere a mpɛn pii no, tebea horow a ɛfata na ɛde saa sɛnkyerɛnne ahorow yi ba no, ɛtɔ mmere bi a ebia aduruyɛ mu tebea bi wɔ eyi akyi te sɛ Thalassemia. Enti ɛnnɛ, momma yɛnka nea Thalassemia yɛ, sɛnea ɛka yɛn nipadua, ne ayaresa a wɔde sa no ho asɛm.

Dɛn ankasa ne Thalassemia?

Sɛ yɛbɛka no tiawa a, thalassemia yɛ awosu mu yare a ɛka yɛn mogya . Ɛnyɛ yare a wɔde kɔ nnipa mu. Ɛno kyerɛ sɛ ɛntrɛw mfi onipa biako so nkɔ foforo mu te sɛ atiridii. Ɛyɛ biribi a yenya fi yɛn awofo hɔ denam yɛn awosu mu.

Sɛ yɛbɛte eyi ase yiye kakra a, ma yenni kan nka mogya mu nkwammoaa kɔkɔɔ a ɛwɔ yɛn mogya mu no ho asɛm kakra. Susuw mogya mu nkwammoaa kɔkɔɔ yi ho sɛ ɛyɛ adwuma a ɛde mframa pa kɔ yɛn nipadua mu nyinaa. Wɔfrɛ kar a wɔde saa nneɛma a wɔde kɔma nkurɔfo yi, kyerɛ sɛ, teaseɛnam a ɛde mframa pa kɔ no, hemoglobin . Hemoglobin yɛ protein titiriw bi a ɛwɔ mogya mu nkwammoaa kɔkɔɔ mu. Eyi ne nea ɛde mframa pa fi yɛn ahurututu mu na ɛkyekyɛ kɔ nipadua no mu nkwammoaa biara mu.

Obi a ɔwɔ thalassemia ntumi nnya protein a wɔfrɛ no hemoglobin yi nnɔɔso. Te sɛ bere a wɔreyɛ kar no, sɛ nneɛma a ɛho hia no bi nni hɔ anaasɛ wɔde afã horow a ɛba fam di dwuma a, kar no rentumi nyɛ adwuma yiye. Eyi ma mogya mu nkwammoaa kɔkɔɔ a ɛte apɔw a ɛwɔ nipadua no mu no so tew. Yɛfrɛ tebea yi a mogya mu nkwammoaa kɔkɔɔ a ɛba fam no mogya a ɛtɔ kɔ nipadua mu no .

Enti sɛ nkwammoaa a ɛwɔ nipadua no mu no nnya mframa pa dodow a ɛfata a, wontumi nnya ahoɔden a wohia no. Ɛno nti na thalassemia ayarefo nya sɛnkyerɛnne ahorow no.

Hena na ɔwɔ asiane kɛse sɛ obenya saa yare yi?

Wosusuw sɛ awosu mu nsakrae a ɛde eyi ba no dii kan bae wɔ nnipa mu sɛ ɔkwan a wɔfa so bɔ wɔn ho ban fi asramma ho. Enti, nnipa a wɔwɔ awosu mu abusuabɔ ne mmeae a asramma abu so te sɛ Afrika, Europa kesee fam, ne aman a ɛwɔ Asia Atɔe, Kesee Fam, ne Apuei fam no wɔ asiane kɛse mu sɛ wobenya thalassemia. Esiane sɛ Sri Lanka nso yɛ Asia Kesee Fam man nti, wɔn a wɔde thalassemia kɔ ne ayarefo wɔ yɛn manfo no mu.

Dɛn na ɛde thalassemia ba? Momma yɛnhwehwɛ mu yiye.

Saa "oxygen teaseɛnam" a wɔfrɛ no hemoglobin a madi kan aka ho asɛm no yɛ protein nkɔnsɔnkɔnsɔn anan. Wɔfrɛ eyinom mu abien alpha globin nkɔnsɔnkɔnsɔn, na wɔfrɛ abien a aka no beta globin nkɔnsɔnkɔnsɔn.

Yenya "akwankyerɛ" anaa "code" a yɛde bɛyɛ saa nkɔnsɔnkɔnsɔn yi fi awosu mu nkwaadɔm a yenya fi yɛn awofo hɔ no mu. Susuw saa awosu mu nkwaadɔm yi ho sɛ aduannoa a wɔde yɛ aduan. Sɛ sintɔ anaa mfomso bi wɔ aduannoa no mu a, wontumi nsiesie aduan no yiye. Saa ara na sɛ sintɔ anaa sintɔ bi wɔ saa awosu mu nkwaadɔm yi mu a, alpha anaa beta globin nkɔnsɔnkɔnsɔn no renhyehyɛ yiye. Ɛno ne bere a tebea bi a wɔfrɛ no thalassemia ba.

• Alpha globin nkɔnsɔnkɔnsɔn: Eyinom hia awosu mu nkwaadɔm 4 na ama wɔatumi ayɛ. Mmienu fi ɛna no hɔ na abien fi agya no hɔ.
• Beta globin nkɔnsɔnkɔnsɔn: Eyinom hia awosu mu nkwaadɔm abien na ama wɔatumi ayɛ. Baako fi ɛna no hɔ na biako nso fi agya no hɔ.

Thalassemia ko a wubenya no gyina saa alpha anaa beta nkɔnsɔnkɔnsɔn yi mu nea ɛwɔ awosu mu nkwaadɔm mu sintɔ so. Wogyina awosu mu nkwaadɔm a asɛe dodow so na ɛkyerɛ sɛnea yare no mu yɛ den.

Dɛn ne thalassemia ahorow atitiriw?

Wɔakyekyɛ thalassemia mu ayɛ no dodow atitiriw pii a egyina sɛnea yare no mu yɛ den so. Ɛne sɛ, thalassemia su, thalassemia ketewaa, thalassemia intermedia, ne thalassemia kɛse . Wɔ carrier tebea mu no, ebia worennya sɛnkyerɛnne biara. Anaasɛ ebia mogya a ɛtɔ kɔ nipadua mu a emu nyɛ den koraa nkutoo na wowɔ. Thalassemia major ne tebea a emu yɛ den sen biara a ɛsɛ sɛ wɔsa.

Ahorow atitiriw abien na ɛwɔ hɔ, a egyina sɛ ebia awosu mu sintɔ no wɔ alpha nkɔnsɔnkɔnsɔn no mu anaa beta nkɔnsɔnkɔnsɔn no mu so.

1. Alpha Thalassemia a ɛwɔ nipadua no mu

Saa tebea yi ba bere a sintɔ bi wɔ awosu mu nkwaadɔm 4 a ɛyɛ alpha globin nkɔnsɔnkɔnsɔn no mu biako anaa nea ɛboro saa mu no. Sɛnea sɛnkyerɛnne ahorow no mu yɛ den no gyina awosu mu nkwaadɔm a asɛe dodow so. Momma yɛnhwɛ eyi wɔ ɔkwan a ɛnyɛ den so na yɛate ase.

2. Beta Thalassemia a ɛwɔ nipadua no mu

Saa tebea yi ba bere a sintɔ bi wɔ awosu mu nkwaadɔm abien a ɛyɛ beta globin nkɔnsɔnkɔnsɔn no mu biako anaa abien no nyinaa mu (baako fi ɛna no hɔ, biako fi agya no hɔ).

• Awosu mu abɔde biako a asɛe: Wɔfrɛ tebea yi beta thalassemia minor anaasɛ carrier state. Nsɛnkyerɛnne no nyɛ den koraa.
• Awosu mu nkwaadɔm abien a asɛe: Nsɛnkyerɛnne no betumi afi nea ɛkɔ fam kosi nea emu yɛ den so.
• Wɔfrɛ tebea a ɛwɔ mfinimfini no Thalassemia intermedia .
• Wɔfrɛ nea emu yɛ den sen biara no beta thalassemia major anaa Cooley mogya a ɛtɔ kɔ nipadua mu . Saa nkurɔfo yi hia ayaresa bere nyinaa.

Dɛn ne thalassemia ho sɛnkyerɛnne ahorow?

Nsɛnkyerɛnne a wubehu no gyina thalassemia ko a wowɔ ne sɛnea emu yɛ den so koraa.

Nsɛnkyerɛnne biara nni hɔ anaasɛ sɛnkyerɛnne a emu nyɛ den koraa

Sɛ woyera alpha awosu mu nkwaadɔm anan no mu biako pɛ a, ebia worennya yare no ho sɛnkyerɛnne biara. Sɛ alpha awosu mu nkwaadɔm abien anaa beta awosu mu nkwaadɔm biako asɛe a, ebia worennya yare no ho sɛnkyerɛnne biara koraa. Anaasɛ ebia mogya a ɛtɔ kɔ nipadua no mu ho sɛnkyerɛnne a emu nyɛ den nkutoo, te sɛ ɔbrɛ a wobɛte nka bere nyinaa.

Nsɛnkyerɛnne a ɛwɔ mfinimfini (Thalsemia Intermedia) .

Saa nkurɔfo yi betumi anya sɛnkyerɛnne afoforo de aka mogya a ɛtɔ kɔ nipadua mu kakraa bi ho.

• Nkɔso a entumi nyɛ yiye: Abofra tenten ne ne mu duru nkɔ soro sɛnea ne mfe te.
• Mmabunbere a ɛkyɛ.
• Nnompe a ɛnyɛ ne kwan so:Tebea horow te sɛ nnompe mu yare.
• Akisikuru a ayɛ kɛse: Akisikuru yɛ akwaa bi a ɛwɔ yɛn yafunu benkum so a ɛboa ma wɔko tia nyarewa.

Nsɛnkyerɛnne a emu yɛ den (Thalassemia Major) .

Nsɛnkyerɛnne a emu yɛ den ba wɔ tebea horow te sɛ alpha gene mu sintɔ abiɛsa (Hemoglobin H yare) ne beta thalassemia major (Cooley mogya a ɛtɔ kɔ nipadua mu) mu. Mpɛn pii no saa sɛnkyerɛnne ahorow yi fi ase da adi ansa na abofra no adi mfe abien .

Wɔ atifi hɔ sɛnkyerɛnne ahorow no akyi no, ebia wubehu nea edidi so yi:

• Aduan no nyɛ dɛ.
• Wora a ɛyɛ mmerɛw anaasɛ ɛyɛ kɔkɔɔ (jaundice).
• Nsu a ɛyɛ tuntum (a ɛyɛ tii-kɔla).
• Anim nnompe a ɛnteɛ (esiane ti nhwi nnompe a enyin dodo nti).

Ɔkwan bɛn so na wobetumi ahu yare yi?

Wɔtaa hu thalassemia a ɛkɔ fam na emu yɛ den wɔ mmofraase, efisɛ sɛnkyerɛnne ahorow no fi ase da adi wɔ abofra nkwa mfe abien a edi kan no mu. Ebia wo duruyɛfo bɛhyɛ sɛ wɔnyɛ mogya mu nhwehwɛmu ahorow mfa nkyerɛ sɛ woahu yare no.

• Mogya Dodow a Edi Mu (CBC): Eyi kyerɛ hemoglobin dodow a ɛwɔ mogya no mu, mogya mu nkwammoaa kɔkɔɔ dodow, ne ne kɛse. Nnipa a wɔwɔ thalassemia no mogya mu nkwammoaa kɔkɔɔ a ɛte apɔw no sua na hemoglobin nso sua. Ebia mogya mu nkwammoaa kɔkɔɔ no nso sua sen sɛnea ɛte daa.
• Reticulocyte dodow: Eyi kyerɛ mogya mu nkwammoaa kɔkɔɔ dodow a wɔayɛ no foforo. Eyi betumi ama woanya adwene sɛ ebia wo nnompe mu ntini no reyɛ mogya mu nkwammoaa kɔkɔɔ a ɛdɔɔso anaa.
• Dade ho nhwehwɛmu: Saa nhwehwɛmu yi boa ma wohu pɛpɛɛpɛ sɛ ebia dade a enni wo mogya mu anaasɛ thalassemia na ɛde wo mogya a ɛtɔ kɔ nipadua no mu no ba.
• Hemoglobin electrophoresis: Eyi yɛ sɔhwɛ a ɛho hia titiriw a wɔde hu beta thalassemia.
• Awosu mu nhwehwɛmu: Wɔde nhwehwɛmu yi di dwuma de hu alpha thalassemia.

Dɛn ne ayaresa ahorow a wɔde sa thalassemia?

Ayaresa a wɔtaa de sa tebea horow a emu yɛ den te sɛ thalassemia major ne mogya a wɔde ma ne aduru a wɔde yi dade fi mu.

• Mogya a Wɔde Ma: Sɛ yɛbɛka no tiawa a, eyi yɛ mogya a wɔde ma wɔ abɔnten. Wɔde mogya a mogya mu nkwammoaa kɔkɔɔ a ɛte apɔw wom no fa ntini bi mu kɔ nipadua no mu. Eyi ma mogya mu nkwammoaa kɔkɔɔ a ɛte apɔw ne hemoglobin dodow san ba. Wɔ thalassemia tebea horow a emu yɛ den mu (sɛ nhwɛso no, beta thalassemia major) no, ebia ɛho behia sɛ wɔma mogya daa, te sɛ adapɛn 2-4 biara .
• Dade Chelation Ayaresa:Nneɛma bɔne a efi mogya a wɔtaa de ma mu ba no biako ne dade dodow a ɛkɔ soro a ɛho nhia wɔ nipadua no mu. Yɛfrɛ eyi dade a ɛboro so . Saa dade a ɛboro so yi betumi ayɛ kɛse wɔ akwaa a ɛho hia te sɛ yɛn koma ne yɛn mmerɛbo mu na asɛe wɔn. Enti wɔma nnipa a wɔtaa nya mogya no nnuru (aduru anaa nnuru a wɔde twitwiw wɔn) a eyi dade a ɛboro so yi fi nipadua no mu.
• Folic Acid Supplements: Wɔde folic acid ma de boa nipadua no ma ɛyɛ mogya nkwammoaa a ɛte apɔw.
• Nnompe mu Ntini ne Ntini mu Nkwammoaa a Wɔde Ma: Mprempren eyi nkutoo ne ayaresa a ebetumi asa thalassemia koraa . Nanso, eyi nti, ɔyarefo no hia obi a ɔfata koraa a ɔwɔ apɔwmuden a ɔde ma. Mpɛn pii no, ɛyɛ onua anaa onuawa. Eyi yɛ ayaresa a asiane wom kɛse na ɛyɛ den.
• Luspatercept: Eyi yɛ aduru a wɔde bɔ nkurɔfo ho ban adapɛn abiɛsa biara. Ɛyɛ adwuma denam boa a ɛboa nipadua no ma ɛma mogya mu nkwammoaa kɔkɔɔ pii no so.

Asetra te dɛn wɔ yare yi ho? So wobetumi asiw ano?

Sɛ wowɔ thalassemia minor a, wubetumi ahwɛ kwan sɛ wobɛtra ase sɛnea ɛsɛ. Sɛ́ ebia wo tebea no yɛ nea ɛkɔ fam anaasɛ emu yɛ den no, sɛ wudi ayaresa nhyehyɛe a wɔakyerɛw ama wo (mogya a wɔde ma ne aduru a wɔde yi dade fi mu) akyi pɛpɛɛpɛ a, wowɔ hokwan pa sɛ wobɛtra ase akyɛ.

Saa yare yi fi awosu mu, enti yɛrentumi nsiw ano. Nanso, awosu mu nhwehwɛmu betumi aboa wo ma woahu sɛ ebia wo anaa wo hokafo wɔ thalassemia awosu mu nkwaadɔm no anaa. Saa nsɛm yi a wobehu no ho hia paa ma awarefo a wɔreyɛ nhyehyɛe sɛ wɔbɛwo ba. Wo ne wo duruyɛfo nkasa na woanya afotu foforo wɔ eyi ho.

Awiei koraa no, thalassemia yɛ yare a wotumi di ho dwuma. Ayaresa ko a wuhia ne mpɛn dodow a wuhia no begyina sɛnea wo tebea no mu yɛ den so. Ɛho hia sɛ wo ne wo duruyɛfo bɔ wo tebea no ne wo bere tenten hwɛ nhyehyɛe ho nkɔmmɔ pefee.