Anigye a wunya bere a wohwɛ wo ba a wɔawo no foforo no yɛ nea wontumi nkyerɛkyerɛ mu, ɛnte saa? Nanso afei, sɛ wote nhwehwɛmu ahorow bi a wɔyɛ wɔ ayaresabea bere a wɔawo akokoaa no akyi a, wote ehu nka kakra na wopɛ sɛ wuhu. Sɔhwɛ a ɛte saa no biako ne PKU sɔhwɛ. Ebia woante din yi ho asɛm. Nanso eyi yɛ sɔhwɛ a ɛho hia paa. Ɛnnɛ yɛreka tebea a ɛntaa nsi a wɔfrɛ no PKU yi ho asɛm, nanso sɛ wohu ntɛm a, wobetumi adi so koraa na akokoaa no atumi atra ase yiye.
Sɛ yɛbɛka no tiawa a, dɛn ne PKU (Phenylketonuria)?
PKU, anaa Phenylketonuria, yɛ awosu mu yare a ɛntaa nsi . Wonya fi awofo hɔ. Akokoaa a ɔwɔ saa tebea yi ntumi nwe amino acid phenylalanine a ɛwɔ protein nnuan a yedi mu no yiye, anaasɛ ntumi nsɛe no.
Fa yɛn nipadua sɛ adwumayɛbea kɛse. Aduan a yedi no ne nneɛma a yɛde yɛ aduan a yɛde gu mu. Adwumayɛfo nkumaa a wɔfrɛ wɔn enzymes na wɔde saa nneɛma a wɔde yɛ nneɛma yi yɛ nneɛma a yɛn nipadua hia no. Akokoaa a ɔwɔ PKU no ntumi nyɛ enzyme titiriw bi a wɔfrɛ no phenylalanine hydroxylase (PAH), a ɛpaapae phenylalanine no pii anaasɛ onnya bi koraa.
Enti dɛn na ɛba? Phenylalanine a wonya fi nufusu mu na akyiri yi wonya fi nnuan a akokoaa no di mu no fi ase boaboa ano wɔ mogya no mu sen sɛ wobeyi afi nipadua no mu. Sɛ phenylalanine dodow a ɛboaboa ano wɔ saa kwan yi so no kɔ soro a, ɛbɛyɛ awuduru ma akokoaa no amemene . Sɛ wɔansa eyi a, eyi betumi asɛe amemene no na ɛde nsɛnnennen a emu yɛ den te sɛ adwene mu dɛmdi ne nkɔso a ɛkyɛ aba.
Nanso ade a eye sen biara wɔ ha ne sɛ wɔ aman pii mu, a Sri Lanka ka ho no, ayaresabea ahorow yɛ saa PKU nhwehwɛmu yi wɔ akokoaa biara a wɔwo no so. Enti, wobetumi ahu yare no ntɛm. Esiane sɛ wobetumi afi ayaresa ase bere a wɔahu no ara pɛ nti, akokoaa no nya hokwan nyin sɛnea ɛsɛ na ɔte apɔw, na ɛmma yare no ho sɛnkyerɛnne a emu yɛ den ntumi mma.
Dɛn ne PKU ho sɛnkyerɛnne ahorow?
Akokoaa a ɔwɔ PKU no hwɛ sɛ ɔwɔ apɔwmuden koraa wɔ awo mu. Enti, mfiase no, ebia nsakrae biara a ɛbɛda adi. Sɛ wɔansa a, yare no ho sɛnkyerɛnne no befi ase ada adi wɔ asram abiɛsa kosi asia ntam. Saa bere yi mu no, ebia wubehu sɛ wo ba no nyin akyɛ kakra.
Edu bere a akokoaa adi bɛyɛ afe biako no, sɛ wɔansa no yare a, sɛnkyerɛnne ahorow a edidi so yi betumi ada adi.
| Nsɛnkyerɛnne a ɛkyerɛ | Nkyerɛmu |
|---|---|
| Hua a ɛyɛ nwonwa | Hua a ɛyɛ nwonwa a ɛyɛ nwunu a efi akokoaa no home, fifiri, anaa ne nsu mu ba. |
| Kɔla sesa | Sɛ wɔde akokoaa no honam ani, ne ti nhwi, ne n’ani kɔla yɛ hare sɛ wɔde toto abusua no mufo a aka ho a. |
| Honam ani nyarewa | Honam ani nyarewa te sɛ eczema. |
| Onyin a ɛkɔ akyiri | Mu duru ne ne tenten a ɛnkɔ soro sɛnea mfe a obi adi (Growth delay). |
| Nneɛma afoforo a ɛwom | Nsɛnkyerɛnne te sɛ ɔfe, akisikuru, ne ahopopo. |
| Nsɛnkyerɛnne a emu yɛ den a sɛ wɔansa a, ebetumi aba | |
| Suban ho haw ahorow | Mpɛn pii a wɔyɛ basaa, nneyɛe a wɔmfa nyɛ hwee, ne mmɔden a wɔbɔ sɛ wobepira wɔn ho. |
| Nneɛma a wɔyɛ no boro so | Sɛ́ wobɛyɛ nnam dodo sɛ wobɛtra baabi biako. |
| Akisikuru a ɛma obi ho kyere no | Seizure -te sɛ tebea horow. |
Ɔkwan bɛn so na nyinsɛn ka ɛna a ɔwɔ PKU?
Eyi yɛ asɛm a ɛho hia paa. Sɛ ɔbea a ɔwɔ PKU nyinsɛn na wɔantumi anhyɛ ne PKU tebea no so wɔ saa bere no mu a, ebetumi aka akokoaa a ɔwɔ awotwaa no mu no. Phenylalanine dodow a ɛkɔ soro wɔ ɛna no mogya mu no betumi apira akokoaa no.
Eyi ma asiane a ɛwɔ hɔ sɛ nyinsɛn betu no yɛ kɛse na ebetumi nso de ɔhaw ahorow aba akokoaa a ɔhyɛ yafunu mu no so.
- Koma mu yare a wɔde awo obi
- Nsakrae bi a ɛba anim nsusuwii mu
- Awo mu duru a ɛba fam
- Ti ketewaa bi (microcephaly) .
Nanso mma nsuro. Sɛ wowɔ PKU, wo ne wo duruyɛfo kasa na wudi aduan titiriw bi akyi wɔ wo nyinsɛn no nyinaa mu a, wo nso wubetumi awo akokoaa a ɔwɔ apɔwmuden koraa .
Dɛn nti na PKU nya nkɔso? Dɛn na ɛde ba?
Sɛnea yɛadi kan aka ho asɛm no, PKU yɛ awosu mu yare. Ɛnam mutation a ɛba wɔ gene bi a wɔfrɛ no `PAH` mu wɔ yɛn nipadua mu. Saa `PAH` gene yi kyerɛ yɛn nipadua sɛ yɛnyɛ enzyme a ɛpaapae phenylalanine no. Sɛ nkwaadɔm mu nsakrae bi ba awosu mu nkwaadɔm no mu a, saa enzyme no ntumi nyɛ, anaasɛ sintɔ bi wɔ ne yɛ mu.
Wɔde yare yi nya fi awo mu wɔ autosomal recessive nhyehyɛe mu. Sɛ yɛbɛka no tiawa a, ɛkɔ so sɛ:
Sɛ akokoaa betumi anya yare no a, ɛsɛ sɛ wonya awosu mu abɔde a wɔasesa no biako fi wɔn maame ne wɔn papa nyinaa hɔ . Sɛ wonya awosu mu abɔde no fi ɔwofo biako pɛ hɔ a, akokoaa no rennya yare no. Nanso, ebia saa ɔwofo no yɛ obi a ɔsoa nneɛma. Eyi kyerɛ sɛ ebia wɔrennya yare no ho sɛnkyerɛnne, nanso wobetumi de awosu mu abɔde no akɔma wɔn mma daakye.
Ɔkwan bɛn so na nnuruyɛfo hu PKU?
PKU a wobehu no abɛyɛ mmerɛw kɛse mprempren esiane mmofra a wɔawo wɔn foforo nhwehwɛmu nti.
Wɔ wo ba no awo akyi nnɔnhwerew 24 kosi 72 no, oduruyɛfo anaa ɔyarehwɛfo bi a ɔwɔ ayaresabea no de ade ketewaa bi bɛbɔ wo ba no nan ase na waboaboa mogya kakraa bi ano wɔ krataa titiriw bi so. Wɔfrɛ eyi ‘heel prick sɔhwɛ.’ Wɔde saa mogya yi hwɛ phenylalanine dodow a ɛwɔ wo ba no mogya mu.
Sɛ phenylalanine dodow a ɛwɔ mogya mu no dɔɔso a, wɔyɛ mogya ne nsu mu nhwehwɛmu foforo de si so dua sɛ wɔahu yare no. Ɛtɔ mmere bi a, wobetumi ayɛ awosu mu nhwehwɛmu de ahu awosu mu nsakrae pɔtee a ɛde yare no ba.
Dɛn ne ayaresa ahorow a wɔde sa PKU?
Aduru biara nni hɔ a wɔde sa PKU. Nanso, wobetumi adi tebea no ho dwuma yiye wɔ asetra nyinaa mu . Sɛ wogyae ayaresa no a, yare no ho sɛnkyerɛnne betumi asan aba bio. Ayaresa titiriw ne aduan titiriw ne ɛtɔ mmere bi a nnuru.
1. Aduan titiriw a phenylalanine nnim
Eyi ne ade titiriw ne ade titiriw a ɛwɔ PKU sohwɛ mu.Ɛsɛ sɛ obi a ɔwɔ PKU di aduan a phenylalanine nnim akyi ne nkwa nna nyinaa.
Esiane sɛ phenylalanine yɛ protein fã nti, ɛsɛ sɛ wɔtew nnuan a protein pii wom so .
- Nam, mpataa, akokɔ
- Nkwaa a wɔde yɛ nkesua
- Nufusu ne nufusu mu nneɛma (yogurt, cheese) .
- Nnuadewa te sɛ atoko ne chickpeas
- Soya nneɛma a wɔde yɛ nneɛma
- Nnuadewa
Nanso, esiane sɛ nipadua no hia protein dodow bi na ama anyin nti, ɛsɛ sɛ wo ne aduan ho ɔbenfo kasa na ama woahu protein dodow a wubetumi adi. Wɔbɛyɛ aduane nhyehyɛeɛ a ahoɔden wom a ɛfata abofra/onipa a ɔwɔ PKU no.
Nea ɛho hia yiye: Ɛsɛ sɛ wɔkwati aduru a wɔde yɛ aduan dɛdɛ ‘aspartame’ no koraa. Aspartame mu paapae wɔ nipadua no mu ma ɛyɛ phenylalanine. Ebia anonne, nnuan, anom a wɔwe, vitamin ahorow bi, ne ɔfe aduru a wɔakyerɛw so sɛ ‘aduan’ anaa ‘asikre nnim’ pii na ɛwɔ mu. Enti, ɛho hia paa sɛ wobɛkenkan nea wɔakyerɛw so no yiye ansa na woadi anaa woanom biribiara.
Ɛsɛ sɛ wofi ase de nkokoaa a wɔawo wɔn foforo a wɔwɔ PKU no ma wɔn aduan titiriw bi a phenylalanine nnim ntɛm ara.
2. Nnuru a wɔde di dwuma
Wɔ PKU ayarefo binom fam no, nnuru betumi aboa de aka aduan ho. Ɛsɛ sɛ wɔde eyinom di dwuma bere a oduruyɛfo akamfo akyerɛ nkutoo.
- Sapropterin dihydrochloride (Kuvan®): Saa aduru yi boa ayarefo binom ma wɔpaapae phenylalanine a ɛwɔ nipadua no mu. Nanso, ɛsɛ sɛ wokɔ so di aduan bi akyi bere a worenom aduru yi.
- Pegvaliase (Palynziq®): Eyi yɛ aduru a wɔkamfo kyerɛ ma ayarefo a wɔanyinyin. Ɛma nipadua no nya enzyme bi a ɛpaapae phenylalanine mu.
So ebetumi aba sɛ wobɛtra ase sɛnea ɛsɛ wɔ PKU ho?
Yiw, akyinnye biara nni ho sɛ ɛte saa! Ɛwom sɛ PKU yɛ tebea a ɛkɔ so wɔ nkwa nna nyinaa mu de, nanso sɛ wohu ntɛm na wɔhwɛ so yiye a, wobetumi atew nkɛntɛnso a enya wɔ akwahosan so no so.
Ɛtɔ mmere bi a, aduan a protein nnim a wubedi akyi wɔ wo nkwa nna nyinaa mu no betumi ayɛ den. Nanso ɛnyɛ wo nko ara. Wo duruyɛfo ne aduannuru ho ɔbenfo wɔ hɔ bere nyinaa sɛ wɔbɛboa wo. Afei nso, sɛ wo ne nnipa afoforo a wɔwɔ PKU bɛka mmoa akuw ho a, ebetumi aboa wo ma woakyɛ osuahu ahorow na woanya ahoɔden.
Ebia woahu ‘aduan’ bi a wɔnom nkotoku ne chewing gum packets a kɔkɔbɔ ka sɛ "Phenylketonurics: Contains phenylalanine." Eyi yɛ nea wɔde bɛma nnipa a wɔwɔ PKU ahu sɛ aspartame a phenylalanine wom wɔ aduru no mu.
Sɛ wo anaa w’abusua te adwennwen nka wɔ wo PKU tebea no ho a, ntwentwɛn wo nan ase sɛ wo ne adwenemyare ho ɔfotufo bi bɛkasa . Adwene mu akwahosan ho hia te sɛ nipadua akwahosan ara pɛ.
Nkrasɛm a Wɔde Kɔ Fie
- PKU yɛ awosu mu yare a wotumi sa na wotumi di so. Nsuro no a ɛho nhia.
- Sɛ wohu yare no ntɛm denam mmofra a wɔawo wɔn foforo mu nhwehwɛmu so a, ɛno ne ade titiriw a ɛbɛma wɔatumi asa yare no yiye.
- Ayaresa titiriw ne sɛ wobedi aduan titiriw bi a phenylalanine nnim akyi nkwa nna nyinaa.
- Sɛ woretɔ nnuan ne anonne a wɔakyerɛw so sɛ ‘Diet’ anaa ‘Sugar-free’ a, bere nyinaa hwɛ sɛ aspartame wom anaa.
- Di akwankyerɛ a wo duruyɛfo ne aduannuru ho ɔbenfo de ama no akyi pɛpɛɛpɛ. Wo ne wɔn nni nkitaho daa.
- Sɛ wɔhwɛ abofra anaa obi a ɔwɔ PKU yiye a, obetumi atra ase a ahoɔden wom koraa, sɛnea ɛte daa, na anigye wom.
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