Yala aw bɛ nin bana in dɔn min man teli ka sɔrɔ jamu fɛ wa? (Transthyrétine amiloïdose héritage - hATTR) .

Yala tuma dɔw la, i farikolo yɔrɔw bɛ sɔgɔsɔgɔ wa? Yala kɔnɔdimi walima ɲɛnamini bɛ kɛ aw la ni aw jɔlen don wa? Hali ni ninnu bɛ se ka kɛ i n’a fɔ fɛn gansanw, tuma dɔw la, fɛn min dogolen bɛ o kɔfɛ, o bɛ se ka kɛ bana ye min man ca, mɔgɔ caman yɛrɛ ma deli ka min mɛn, wa a bɛ tɛmɛ mɔgɔw fɛ mɔgɔw fɛ. Bi an bɛna kuma o bana sugu dɔ kan, n’o ye hATTR amiloyizi ye . Hali n’a y’a sɔrɔ nin ye barokun ye min ka gɛlɛn dɔɔnin, an k’a faamuya nɔgɔya la.

hATTR Amiloyidozi ye mun ye?

N’an y’a fɔ cogo nɔgɔman na, hATTR (Hereditary Transthyretin Amyloidosis) ye bana ye min man teli ka sɔrɔ, a bɛ bɔ mɔgɔw la ka taa mɔgɔw la, wa a bɛ juguya dɔɔni dɔɔni waati tɛmɛnen kɔfɛ. O sababu ye fɛn dɔ yeli ye an ka jɛnɛya la, o min bɛ wele ko fɛn falen-falen.

Miiri k’a filɛ ko fɛn o fɛn bɛ an farikolo la, o bɛɛ bɛ mara jeninida fɛ. Jɛnkulu dɔ bɛ an ka sugunɛ na min bɛ wele ko TTR . Ni fɛn dɔ jiginna o jamu in na, farikolojɔlifɛn min bɛ wele ko ‘transthyretin’ min bɛ sɔrɔ a fɛ, o fana bɛ kɛ cogo la min tɛ cogo la, min man ɲi.

O farikolojɔlifɛn minnu cogoya ma ɲɛ, olu bɛ ɲɔgɔn dalajɛ ka fɛnw kɛ minnu bɛ wele ko ‘ amyloïde ’ an farikolo nɛrɛmugumaw ni farikolo yɔrɔ suguya caman na. I n’a fɔ nɔgɔ ni nɔgɔ bɛ jibolisira datugu cogo min na, o amiloyiw bɛ o farikolo yɔrɔw tiɲɛ.

Nin bana in bɛ kɛ sababu ye ka joli-sira-funu (polyneuropathy) bila mɔgɔ la, o min ye tiɲɛni ye farikolo yɔrɔw la minnu bɛ bɔ kunsɛmɛ na ka taa farikolo yɔrɔ wɛrɛw la. Hali ni nin ye bana jugumanba ye, ni a furakɛra ka ɲɛ, a taamasiɲɛw bɛ se ka kunbɛn ani ka ɲɛnamaya kɛ ka taa a fɛ.

hATTR bana taamasiɲɛw ye mun ye?

Ikomi hATTR bana bɛ farikolo yɔrɔ caman minɛ, a taamasiɲɛw ka ca kosɛbɛ. Tuma dɔw la, o taamasiɲɛw ni bana caman wɛrɛw ta bɔlen don ɲɔgɔn fɛ, o la a bɛ se ka gɛlɛya ka bana in dɔn.

Aw ye sɛbɛn ɲɛ jukɔrɔla lajɛ walasa ka o taamasiɲɛw dɔn.

hATTR bana bɛ sɔrɔ cogo di?

Ikomi nin bana in man ca, wa a taamasiɲɛw ni bana tɔw bɔlen don ɲɔgɔn fɛ, a dɔnni bɛ se ka gɛlɛya dɔɔni. Nka dɔgɔtɔrɔ b’a sinsin fɛn damadɔw kan.

• Denbaya ka furakɛli tariku : Aw bɛ a ɲininka ni dusukun tantanni, dusukunnabana, walima hakiliɲagami wɛrɛw kɛra aw ka denbaya kɔnɔ.
• Ɲininkali kɛli bana taamasiɲɛw kan: Ka ɲininkali kɛ ni taamasiɲɛ minnu fɔra sanfɛ, olu bɛ aw la.
• Jɛnkulu sɛgɛsɛgɛli : Joli walima farilajidɛsɛ sɛgɛsɛgɛli bɛ kɛ walasa ka a dɔn ni fɛn dɔ b’a la ka TTR jamu sɛgɛsɛgɛ. Nin ye bana in dafacogo dafalen ye.
• Tissue Biopsy : Tumu dɔɔni min bɛ bɔ kɔnɔbara fari jukɔrɔ, o bɛ ta ni pikirijikɛlan fitinin ye ka a lajɛ ni mikroskɔpu ye walasa ka a dɔn ni amiloyi dɔw bɛ yen. Aw kana aw hakili ɲagami, nin tɛ opereliba ye, wa a bɛ se ka kɛ joona.
• Kɔrɔbɔli wɛrɛw:Joli ni sugunɛ sɛgɛsɛgɛli, farikolojidɛsɛ sɛgɛsɛgɛli, ani sɛgɛsɛgɛli minnu bɛ kɛ i n’a fɔ ECG ani Echo walasa ka dusukun baara lajɛ, olu fana bɛ se ka kɛ.

hATTR furakɛcogo jumɛnw ye?

Laɲiniba fila bɛ hATTR bana furakɛli la. Kelen ye ka bana taamasiɲɛw kunbɛn, tɔ kelen ye ka farikolojɔli dumuni nafama bɔli ni a bilali dɔgɔya walima k’a jɔ.

Taamasiɲɛw furakɛli

• fura bɛ di dusukun gɛlɛyaw ni ji caman sɔrɔli ma farikolo la .
• Fura bɛ di dumunikɛcogo gɛlɛyaw ma i n’a fɔ kɔnɔboli ni kɔnɔboli.
• Fura minnu bɛ kɛ ka farikolojidɛsɛ kunbɛn.

Furakɛli min ɲɛsinnen bɛ bana in sababu ma

Olu de ye furakɛli kunbabaw ye minnu bɛ se ka bana in taabolo yɛlɛma.

• Fura minnu bɛ jeninida bali: O furaw bɛ baara kɛ ni TTR jamu min bɛ sugunɛ na, o bali ka farikolojɔli dumuni juguw dilan. `Inotersen`, `Patisiran`, `Vutrisiran` ye fura ninnu dɔw ye. O dɔw bɛ sɔrɔ dɔgɔkun o dɔgɔkun pikiri ye, dɔw bɛ kɛ jolisira fɛ dɔgɔkun 3 o dɔgɔkun 3.
• Fura minnu bɛ jeninida sabati: O furaw bɛ baara kɛ ni TTR furakisɛ min ma datugu cogo jugu la, o bali ka fara ɲɔgɔn kan ani ka amiloyi (amiloïde) bila. `Tafamidis` ni `Diflunisal` ye furaw ye nin kalasi in na.
• Sugunɛ cili : Ikomi farikolojɔli dumuni nafama bɛ bɔ sugunɛ na, sugunɛ cili bɛ se ka bana jɛnsɛnni kunbɛn kosɛbɛ. Nka, o ye opereliba ye. Wa a bɛ ɲɛ sɔrɔ bana daminɛ na dɔrɔn.

Dɔgɔtɔrɔso jɛkulu min bɛ aw dɛmɛ

Ikomi nin bana in bɛ farikolo yɔrɔ caman minɛ, aw mago bɛna kɛ dɔgɔtɔrɔ kɛrɛnkɛrɛnnen caman ka dɛmɛ na.