Jigiya kura Fabry bana bɛ mɔgɔ minnu na: An ka kuma fura Galafold kan!

Ni Fabry bana bɛ aw walima aw dɔnbaga dɔ la , n’a sɔrɔ aw ye fura dɔ mɛn min tɔgɔ ye ko Galafold. O ye Fabry bana fura fɔlɔ ye min bɛ ta da la. Fabry bana furakɛcogo wɛrɛw, i n’a fɔ anzimu caman cili furakɛli, olu bɛ kɛ ni pikiri ye. Galafold ye "chaperone" furakɛli sugu ye min bɛ baara kɛ cogo wɛrɛ la pewu. A tɛ baara kɛ Fabry bana suguya bɛɛ la. Nka, a bɛ se ka dɛmɛba lase mɔgɔ ma, fɛn dɔw b’u la, fɛn dɔw b’u la minnu bɛ bana in bila mɔgɔ la.

Fabry ka bana ye mun ye? Galafold bɛ baara kɛ cogo di?

N’an y’a fɔ cogo nɔgɔman na, Fabry ka bana ye bana ye min man teli ka sɔrɔ jamu fɛ , wa a bɛ bɔ bangebaga fɛ ka taa den wɛrɛ la. Fabry bana bɛ mɔgɔ minnu na, olu farikolo la, anzimu dɔ bɛ u fari la min bɛ wele ko alpha-galactosidase A (alpha-gal) . Nka nin anzimu in tɛ baara kɛ ka ɲɛ. O bɛ Kɛ bari fɛn dɔ bɛ 'yɔrɔ la min bɛ 'yɔrɔ la min b'a Fɔ a ye ko a ka nin anzimu in Kɛ.

Alpha-gal ye farikolojɔlifɛn nafamaba ye min bɛ sɔrɔ farikolokisɛ caman na. A baara kunba ye ka tulumafɛn dɔ tiɲɛ min bɛ wele ko GL-3 . O la, ni nin anzimu in ma baara kɛ ka ɲɛ, fɛn min bɛ wele ko GL-3, o bɛ daminɛ ka dalajɛ farikolo yɔrɔw kɔnɔ. Waati tɛmɛnen kɔfɛ, o dalajɛli bɛ se ka tiɲɛniba lase farikolo yɔrɔ nafamaw ma, i n’a fɔ sugunɛ , dusukun , fari, kunsɛmɛ ani farikoloɲɛnajɛ .

Fabry bana suguya fila de bɛ yen.

Ni Fabry bana bɛ mɔgɔ minnu na, olu bɛ kɔrɔbaya, GL-3 bɛ to ka dalajɛ u ka farikolokisɛw la. O bɛ se ka kɛ sababu ye ka kɛnɛyako gɛlɛyabaw lase mɔgɔ ma waati tɛmɛnen kɔfɛ. Mɔgɔ caman bɛ sugunɛ gɛlɛyaw sɔrɔ, o bɛ se ka laban ka kɛ sababu ye ka sugunɛbaralabana kɛ . Bana in bɛ se ka dusukun ni joli siraw fana tiɲɛ , ka dɔ fara dusukun tantanni walima kirinni farati kan.

Mɔgɔ caman bɛ furakɛli kɛ ni Enzyme Replacement Therapy (ERT) ye o kama. O ye ka anzimu dɔ pikiri kɛ min bɛ baara kɛ ka ɲɛ, min bɛ bɔ kɛnɛma ka don farikolo la. O kɔfɛ, farikolo bɛ se ka baara kɛ n’o anzimu ye walasa ka GL-3 tiɲɛ.

Nka, Galafold bɛ baara kɛ cogo wɛrɛ la pewu. Galafold kɔnɔfɛn min bɛ baara kɛ, o ye fura ye min bɛ wele ko `migalastat`. A bɛ min kɛ, o ye, a bɛ nɔrɔ `alpha-gal` anzimu la min bɛ banabagatɔ yɛrɛ farikolo la, nka a tɛ sabati. Miiri k’a filɛ ko `alpha-gal` anzimu min bɛ i fari la, o ‘funulen don’ dɔɔni, ‘a tɛ sabati’. O la, a tɛ se ka baara kɛ ka ɲɛ. Fura min bɛ wele ko Galafold, o bɛ min kɛ, i n’a fɔ a terikɛ, a bɛ nɔrɔ nin anzimu ‘funulen’ in na, k’a ‘sigi’. O kɔ, o anzimu bɛ se k’a ka baara kɛ ka ɲɛ. An bɛ min wele ko "Chaperone Therapy" o ye nin ye .

Yala bɛɛ bɛ se ka baara kɛ ni Galafold ye wa?

Ayi Galafold bɛ se ka kɛ mɔgɔw dɔrɔn de la minnu ka fɛn falen suguya kɛrɛnkɛrɛnnen dɔ bɛ u la min bɛ `alpha-gal` enzyme dilan. `alpha-gal` enzyme min bɛ sɔrɔ o fɛn falen in fɛ, o bɛ se ka baara kɛ hali bi, nka a tɛ sabati kojugu fo a tɛ se ka baara kɛ ka ɲɛ. Galafold bɛ nɔrɔ o anzimu la, k’a sabati, k’a kɛ baara ye.

Fabry banabagatɔ dɔw tɛ `alpha-gal` anzimu dilan fewu, walima anzimu min bɛ bɔ, o tɛ baara kɛ fewu. Galafold tɛna nafa foyi lase o mɔgɔw ma. Sabula anzimu si tɛ farikolo la min bɛ se k’a sabati. Enzyme replacement therapy (ERT) bɛnnen don u ma kosɛbɛ.

O la, sani dɔgɔtɔrɔ ka Galafold sɛbɛn di aw ma, a bɛna sɛgɛsɛgɛli kɛrɛnkɛrɛnnen dɔ kɛ aw ka jamu sɛgɛsɛgɛli la walasa ka a dɔn tigitigi jamu jiginni suguya min bɛ aw la. O de bɛna a jira ni nin fura in nafa bɛna kɛ aw la walima ni nafa tɛ aw la.

Aw bɛ fura in ta cogo di?

Nin yɔrɔ in nafa ka bon kosɛbɛ barisa walasa ka fura in nafa bɛɛ sɔrɔ, aw ka kan ka a kɛ ka ɲɛ.

• A tacogo: Galafold ye furakisɛ ye. A bɛ ta tile fila o tile fila , o kɔrɔ ye ko tile fila o tile fila.
• Tile waati: A nafa ka bon kosɛbɛ ka a ta waati kelen na don o don. Misali la, ni aw y’a ta tile fɔlɔ sɔgɔma 8 waati, aw ka kan k’a ta o dugujɛ fana sɔgɔma 8 waati.
• Min nafa ka bon kosɛbɛ: Nin fura in ka kan ka ta kɔnɔbara lankolon na . O kɔrɔ ye ko aw man kan ka foyi dun lɛri 2 kɔnɔ ka kɔn fura tali ɲɛ walima lɛri 2 fura tali kɔfɛ.
• Fɛn wɛrɛ min nafa ka bon: Kafeyin bɛ fɛn o fɛn na fana (i n’a fɔ te, kafe, minfɛn gazima dɔw) aw kana o min lɛri 2 kɔnɔ ka kɔn furakisɛ tali ɲɛ ani lɛri 2 kɔfɛ. O waati kɔnɔ, aw bɛ se ka ji min, jiriden minfɛn minnu tɛ jolisegindumuni ye, walima minfɛn gazima minnu tɛ kafeyin sɔrɔ.

Walasa ka aw dɛmɛ aw kana aw ka donw ɲagami, fura in bɛ sɔrɔ foroko kɛrɛnkɛrɛnnen dɔ kɔnɔ. A bɛ fɔ a ma ko `blister card'. Fɛɛrɛ dɔ b’a la ka donw taamaʃyɛn Kɛ. Aw bɛ fura ta don min na, aw bɛ blister karti bɔ, aw ma fura ta don min na, aw bɛ sɛrɛkili lankolon bɔ a nɔ na. O cogo la, aw bɛ se ka taamasiyɛn kɛ karti kan don o don, aw mago bɛ fura tali don min na, aw kana tɛmɛ o kan.

Yala kɔlɔlɔw ni gɛlɛyaw bɛ fura wɛrɛw la wa?

Mɔgɔ dɔw bɛ Galafold ta, ninakilidegun (farigan, dawolodimi) ani sugunɛbana bɛ se ka caya dɔɔni. Banakisɛw kunbɛncogo ɲuman ye ka aw tɛgɛ ko tuma caman, ka aw yɔrɔ janya banabagatɔw la, ka ji caman min.

Ka fara o kan, i n’a fɔ an y’a ɲɛfɔ cogo min na ka tɛmɛ, kafeminkutuO la, Galafold bɛ se ka kɛ a tɛ baara kɛ kosɛbɛ. O la, aw bɛ aw janto fɛnw na kafeyin bɛ minnu na. A nafa ka bon kosɛbɛ fana ka dɔgɔtɔrɔ ladɔnniya fura wɛrɛw, witaminiw walima tubabufura wɛrɛw la aw bɛ minnu ta.