So wo duruyɛfo akyerɛw wo anaa obi a ɔwɔ w’abusua mu aduru bi a wɔfrɛ no Glycerol Phenylbutyrate? Ɛyɛ aduru a ɛwɔ din soronko bi, enti ebia wowɔ nsɛmmisa pii wɔ ho. Mma ɛnhaw wo, ɛnnɛ yɛbɛka saa aduru yi ho asɛm kɛkɛ, nea ɛyɛ, sɛnea wɔde di dwuma, ne nneɛma a ɛsɛ sɛ yɛhwɛ yiye wɔ ho.
Sɛ yɛbɛka no tiawa a, dɛn ne aduru yi?
Glycerol Phenylbutyrate (a wɔsan frɛ no edin RAVICTI) yɛ aduru a wɔde siw ammonia dodow a ɛwɔ nnipa a wɔwɔ urea kyinhyia mu yare no mogya mu ano .
Susuw ho hwɛ, sɛ yɛn nipadua de protein di dwuma a, ɛma ammonia yɛ ade a wɔsɛe no. Wɔ obi a ɔwɔ apɔwmuden mu no, urea kyinhyia no dan saa ammonia yi ma ɛbɛyɛ urea a asiane biara nni ho na eyi fi mu wɔ nsu mu. Nanso wɔ obi a ɔwɔ urea cycle disorder mu no, nipadua no ntumi nyɛ adwuma yi yiye. Afei, ammonia betumi aboaboa ano wɔ nipadua no mu na asɛe amemene ne ntini ahorow no.
Saa aduru yi boa ma woyi ammonia a ɛboro so no fi nipadua no mu. Nanso ade biako na ɛsɛ sɛ yɛma ɛtra yɛn adwenem. Eyi nyɛ aduru a ɛsa yare no koraa. Eyi yɛ aduru a ɛboa ma wotumi si yare no so nkutoo.
Mpɛn pii no, sɛ wɔma wo saa aduru yi a, ebehia sɛ wudi aduan titiriw bi a protein nnim a wo duruyɛfo akyerɛ wo no akyi, na ebia wobɛnom aduan foforo a wɔde ka aduan ho.
Nneɛma a ɛsɛ sɛ woka kyerɛ wo duruyɛfo ansa na woanom nnuru
Ansa na wobɛhyɛ saa aduru yi ase no, ɛho hia sɛ woka nsɛm a edidi so yi kyerɛ wo duruyɛfo. Mfa saa nneɛma yi nsie, efisɛ ɛho hia paa ma w’ahobammɔ.
| Tebea a ɛsɛ sɛ wobɔ ho amanneɛ | Dɛn nti na ɛno ho hia? |
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| Ɔhaw ahorow a ɛba dwensɔtwaa mu | Ebia aduru no a wɔtwe kɔ nipadua no mu no bɛka nipadua no. |
| Asaabo, mmerɛbo anaa dwensɔtwaa mu yare | Esiane sɛ wɔnam saa akwaa yi so yi aduru no fi nipadua no mu nti, sɛ aduruyɛ mu tebea horow bi wɔ hɔ a, ebia ebehia sɛ wɔyɛ nsakrae wɔ dodow a wɔde ma no mu. |
| Nneɛma a ɛma obi ho yɛ no hyew | Hwɛ hu sɛ wobɛka akyerɛ wo duruyɛfo sɛ wowɔ aduru yi, nnuru afoforo, nnuan, nneɛma a wɔde siesie nneɛma, anaa kɔla ahorow bi ho adwenemhaw bi a. |
| Sɛ woanyinsɛn anaasɛ worebɔ mmɔden sɛ wubenyinsɛn a | Ɛsɛ sɛ wo ne wo duruyɛfo kasa fa sɛ ebia aduru no bɛka akokoaa a ɔwɔ awotwaa mu no anaa. |
| Sɛ wɔma akokoaa no nufu a | Hwɛ wo duruyɛfo sɛ ebia aduru no nam nufusu so kɔ akokoaa no nkyɛn anaa. |
Ɔkwan bɛn so ankasa na mede saa aduru yi di dwuma?
Saa aduru yi ba wɔ aduru a wɔnom mu, enti hwɛ nneɛma yi yiye.
- Fa w’ano nom: Eyi yɛ aduru a wɔnom no ano.
- Susuw yiye: Fa nsenia, kuruwa, anaa syringe soronko a ɛka aduru no ho no susuw . Mfa teaspoons a wɔde di dwuma daa nni dwuma wɔ fie, efisɛ ɛnyɛ pɛpɛɛpɛ koraa.
- Fa ka aduan ho: Ɛyɛ papa sɛ wode saa aduru yi bɛka aduan ho anaasɛ, wɔ nkokoaa fam no, fa aduan a wɔde ma ho .
- Fa no bere ano: Nom aduru no wɔ bere a wɔakyerɛw ama wo no mu, wɔ bere a wɔahyɛ no mu, sɛnea wo duruyɛfo akyerɛ wo no. Mfa dodow a wɔakyerɛw ato hɔ no nnwe.
- Nnyae: Nnyae aduru no nom mpofirim a wunni aduruyɛ mu afotu.
- Sɛ wɔde ma mmofra a: Ɛtɔ mmere bi a wɔkyerɛw saa aduru yi ma nkokoaa a wɔawo wɔn foforo mpo. Nanso, ahwɛyiye soronko bi wɔ hɔ a ɛsɛ sɛ wodi akyi. Enti, wo ne wo mmofra oduruyɛfo nkasa wɔ eyi ho yiye na nya afotu.
Na sɛ wɔma aduru no dodow kɔ soro (wɔnom boro so) nso ɛ?
Sɛ wususuw sɛ woanom nnuru pii a, ade a edi kan a ɛsɛ sɛ woyɛ ne sɛ wobɛfrɛ Ɔman Awuduru Ho Amanneɛbɔbea a ɛwɔ Colombo Ɔman Ayaresabea no . Anaasɛ kɔ ayaresabea a ɛbɛn wo no Emergency Treatment Unit (ETU) . Mma eyi nntwentwɛn wo nan ase.
Sɛ wopa nnuru dodow bi a, dɛn na ɛsɛ sɛ woyɛ?
Sɛ wo werɛ fi sɛ wobɛnom dose bi a, fa no bere a woakae ara pɛ. Nanso, sɛ ɛkame ayɛ sɛ bere aso sɛ wobɛma wo dodow a edi hɔ no a, twa dodow a woayera no so na fa wo dodow a edi hɔ no wɔ bere a woahyɛ da ayɛ no mu. Mfa nnuru abien bere koro mu esiane biribiara nti.
Nneɛma afoforo a ebia ɛne aduru yi bedi nkitaho
Nnuru afoforo a worenom betumi ne saa aduru yi adi nkitaho, enti ka nnuru a worenom nyinaa (a vitamin, nhabannuru, ne nnuru a wɔde ka ho) kyerɛ wo duruyɛfo.
| Nnuru ahorow a ebetumi de ntawntawdi aba (nhwɛso ahorow) . |
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| Hyɛ no nsow: Eyi nyɛ nsɛm a wɔahyehyɛ a edi mũ. Afei nso, sɛ wonom sigaret, wonom nsa, anaa wode nnubɔne foforo di dwuma a, ka kyerɛ wo duruyɛfo. |
Nsunsuanso bɔne a ebetumi afi aduru no a wɔnom mu aba
Te sɛ aduru biara no, eyi betumi de ɔhaw ahorow bi aba. Ebi yɛ aniberesɛm, ebi nso abu so.
| Nsunsuanso bɔne a ɛsɛ sɛ wobɔ wo duruyɛfo amanneɛ ntɛm ara |
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| Nneɛma bɔne a ɛsɛ sɛ wɔbɔ ho amanneɛ kyerɛ oduruyɛfo sɛ ɛkɔ so anaasɛ ɛhaw adwene a |
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Sɛ wunya sɛnkyerɛnne foforo biara a ɛyɛ soronko a wɔanka ho asɛm wɔ saa nsɛm yi mu a, wo ne wo duruyɛfo nkasa mfa ho nso.
Ɔkwan bɛn so na ɛsɛ sɛ mede aduru no sie?
- Twe wo ho fi mmofra ho.
- Fa sie wɔ dan mu hyew mu, a ɛyɛ digrii Celsius 20 kosi 25 (68-77 Fahrenheit).
- Tow nnuru a ne bere atwam no gu.
Nkrasɛm a Wɔde Kɔ Fie
- Glycerol Phenylbutyrate nyɛ aduru a ɛsa urea kyinhyia mu haw, ɛyɛ aduru a ɛboa ma ammonia dodow a ɛwɔ mogya mu no so.
- Fa saa aduru yi ka aduan ho bere nyinaa, na fa susudua kuruwa soronko a ɛka ho no di dwuma.
- Di aduan a protein kakraa bi wom a wo duruyɛfo akyerɛ wo no akyi pɛpɛɛpɛ.
- Nnyae nnuru no nom a wunnyaa wo duruyɛfo afotu.
- Sɛ wonom nnuru pii anaasɛ wunya ɔhaw ahorow a emu yɛ den a, hwehwɛ aduruyɛfo afotu ntɛm ara.
- Ka nnuru afoforo a worenom nyinaa (a vitamin ne nnuru a wɔde di dwuma wɔ amanne kwan so ka ho) kyerɛ wo duruyɛfo.
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