Porfiri ye mun ye? Kana siran, an ka kuma o kan cogo nɔgɔman na.

Yala i delila ka jeninida walima kurukuru dɔ sɔrɔ i fari la min tɛ se ka muɲu ni i bɔra tile la wa? Walima yala i delila ka kɔnɔdimi jugumanba sɔrɔ ni kɔnɔboli ni fɔɔnɔ ye kun ɲɛnama tɛ min na wa? o taamasiɲɛw ni bana minnu bɛ sɔrɔ tuma caman na , olu tɛ kelen ye . Laala o kɔfɛ, bana dɔ bɛ yen min man ca kosɛbɛ, mɔgɔ caman yɛrɛ ma min mɛn. o ye o bana sugu dɔ ye min bɛ wele ko Porphyria . Aw kana siran ni aw ye nin tɔgɔ in mɛn. Bi, an bɛna kuma o kan cogo nɔgɔman na ani cogo la min bɛ se ka faamuya.

N’an y’a fɔ cogo nɔgɔman na, Porphyria ye mun ye?

Walasa k’o faamu, an ka fɔlɔ ka miiri izini fitinin dɔ la an farikolo la. Protéine nafamaba dɔ bɛ yen min bɛ an ka joli kuru bilenmanw ɲɛ bilen ani ka oksizɛni ta ka bɔ fogonfogon la ka taa farikolo tɔ la. A bɛ Weele ko ‘heme’. O hɛmɛ in bɛ Bɔ an farikolo la taamaʃyɛnw na, i n’a fɔ fɛn min bɛ ɲɔgɔn Lajɛ. I n’a fɔ ka mobili lajɛ cogo min na. O sen kelen-kelen bɛɛ la, farikolojɔlifɛn kɛrɛnkɛrɛnnenw bɛ baara kɛ minnu bɛ wele ko enzymes .

Sisan, i k’a filɛ, mun bɛ kɛ ni sen kelen min bɛ nin fɛn dilanni cakɛda in na, o sirilen don, walima ni baarakɛla kelen (enzyme) ma baara kɛ ka ɲɛ? Fɛn nata tɛ se ka kɛ, wa fɛnɲɛnamafagalanw bɛ ɲɔgɔn dalajɛ, ɔnhɔn? O cogo kelen na, ni fiɲɛ dɔ kɛra hɛmɛ dilanni senfɛ, kemikɛli minnu bɔra sen tɛmɛnen na, n’u bɛ wele ko ‘porfiriniw’ ani ‘porofirini ɲɛbilaw’, olu bɛ daminɛ ka dalajɛ farikolo la. Nin fura ninnu dalajɛli farikolo la, o de bɛ kɛ sababu ye ka pɔrfiriki taamasiɲɛw jira. Olu bɛ se ka farikoloɲɛnajɛ walima fari tiɲɛ.

Porfiri suguya jɔnjɔnw ye jumɛnw ye?

Porfiri bɛ se ka tila kuluba fila ye, ka da a taamasiɲɛw jiracogo kan. Ni aw ye nin danfara in faamuya, o bɛna aw dɛmɛ ka bana in faamuya ka ɲɛ.

Sugu fitinin damadɔ wɛrɛw bɛ o suguba fla in 'kɔnɔ. Misali la, suguya min bɛ wele ko `Acute Intermittent Porphyria (AIP)`, o bɛ bin porphyria acute jukɔrɔ, ani suguya min bɛ wele ko `Porphyria Cutanea Tarda (PCT)`, o bɛ bin farikolo porphyria kɔrɔ, olu man ca.

Fɛn jumɛnw de bɛ kɛ sababu ye ka bana taamasiɲɛw jira?

Hali ni porfiriya ye jamu ye, a taamasiɲɛw tɛ ye tuma bɛɛ. Fɛn dɔw bɛ yen minnu bɛ se ka taamasiɲɛw bila mɔgɔ la, walima "binkanni". Olu bɛ Weele ko ‘triggers’. Ka aw yɛrɛ tanga ninnu ma, o de ye bana in kunbɛncogo ɲuman ye.

• Fura dɔw: Dimimadafura dɔw , bangekɔlɔsi furakisɛ dɔw, anestezifura, ani kirinnifura dɔw bɛ se ka o sababu kɛ. Ni porfiriya bɛ aw la, aw ka kan ka kuma aw ka dɔgɔtɔrɔ fɛ sisan sani aw ka fura suguya o suguya ta.
• Dɔlɔ ni sigarɛtimin : Dɔlɔmin ni sigarɛtimin, kɛrɛnkɛrɛnnenya la waati kelen na, olu ye fɛnba ye minnu bɛ a bila mɔgɔ la.
• Dumuni dunni ni sunɔgɔ: Fɛn minnu bɛ i n’a fɔ dumuni minnu tɛ witaminiw dɔgɔya , dumuni minnu bɛ mɔgɔ farikolo girinya dɔgɔya, ani sunɔgɔ, olu bɛ se ka bana taamasiɲɛw bila mɔgɔ la.
• Hakili ni farikolo degun: Fɛn minnu bɛ i n’a fɔ baara caya kojugu, hakili degun ani opereli, olu bɛ se ka nɔ bila a la.
• Banakisɛw : A taamasiɲɛw bɛ se ka sɔrɔ degun fɛ min bɛ mɔgɔ bila farikolo la bana walima banakisɛ wɛrɛ fɛ.
• Tile yeelen : Tile yeelen de ye farikolojidɛsɛbanakisɛ bɛ mɔgɔ minnu na, olu sababuba ye.

Aw ka kan ka taa dɔgɔtɔrɔso la tuma jumɛn? Sɛgɛsɛgɛli bɛ kɛ cogo di?

Ikomi nin bana in man ca, wa a taamasiɲɛw ni bana caman wɛrɛw bɔlen don ɲɔgɔn fɛ, a bɛ se ka waati dɔɔni ta tuma dɔw la ka sɔrɔ ka a sɛgɛsɛgɛ. A nafa ka bon ka taa dɔgɔtɔrɔso la ni nin taamasiɲɛ ninnu dɔ bɛ aw la, kɛrɛnkɛrɛnnenya la ni u bɛ to ka kɛ.

Porfirii jugumanba la

Ni kɔnɔdimi jugumanba bɛ aw la ka fara a taamasiɲɛw kan i n’a fɔ kɔnɔboli, fɔɔnɔ, kɔnɔboli, dusukun tantanni caya, tansiyɔn jiginni, farikolo fanga dɔgɔyali, hakili ɲagami, walima kirinni, a nafa ka bon kosɛbɛ ka taa dɔgɔtɔrɔso la joona, kɛrɛnkɛrɛnnenya la furakɛli kɔrɔtɔlen (ETU), barisa nin bana in bɛ se ka mɔgɔ ka ɲɛnamaya bila farati la tuma dɔw la.

Ni Porfirie Cutanée ye

Ni kurukuruw, kurukuruw, funu, bilenni walima kurukuruw bɛ aw fari la nɔgɔya la tile kɛlen kɔfɛ, aw bɛ taa farikolodɔgɔtɔrɔ dɔ fɛ.

Sɛgɛsɛgɛli kɛcogo:

1. Dɔgɔtɔrɔso sɛgɛsɛgɛli: Fɔlɔ, dɔgɔtɔrɔ bɛna aw sɛgɛsɛgɛ, ka aw ɲininka kosɛbɛ aw ka bana taamasiɲɛw ni aw ka denbaya ka furakɛli tariku la.

2. Laboratowari sɛgɛsɛgɛliw : Ni siga bɛ a la ko porfirini bɛ a la, aw ka sugunɛ, joli, ani a bɛ se ka kɛ banakɔtaa lajɛw bɛna sɛgɛsɛgɛ walasa ka porfirini hakɛ dɔn. Ni bana juguman don, sugunɛ bɛ se ka kɛ bilen dibiman walima bulama ye.

3. Jɛnkulu sɛgɛsɛgɛliw : Ni bana in sɛgɛsɛgɛra laboratuwari sɛgɛsɛgɛliw fɛ, jamu sɛgɛsɛgɛliw bɛ se ka kɛ walasa ka jamu sɛgɛsɛgɛli kɛrɛnkɛrɛnnen dɔn min kɛra sababu ye ka bana in bila mɔgɔ la.

Furakɛli suguya jumɛnw bɛ yen?

Porfiriya furakɛcogo tɛ kelen ye ka kɛɲɛ ni bana suguya ni a taamasiɲɛw juguya ye. Hali ni fura tɛ yen, a taamasiɲɛw bɛ se ka kunbɛn ani ka ɲɛnamaya kɛcogo ɲuman kɛ.

Porfirii jugumanba furakɛli

Ni bana taamasiɲɛw juguyara (banakisɛ jugumanba), a ka kan ka don dɔgɔtɔrɔso la ani ka furakɛli kɛ.

• Kɔgɔji (ji IV) dili : Aw bɛ farikolo kɔgɔ ni ji hakɛ bɛn ɲɔgɔn ma.
• Hɛmɛ pikiri : Hɛmi dɛsɛ kunbɛn farikolo la ani ka dɔ bɔ porfirini hakɛ la min bɛ dalajɛ.
• Sukaro dili : Sukaro i n’a fɔ dekstrose dili jolisira fɛ walima da la, o bɛ taamasiɲɛ kɛ sugunɛ na walasa ka dɔ bɔ pɔrfirini bɔli la.
• Furakɛcogo wɛrɛw: Dimimadafura minnu bɛ dimi jugumanba la, ani kɔnɔboli ni fɔɔnɔ furaw.
• Fura kuraw i n’a fɔ Givosiran (Givlaari): Mɔgɔ minnu ka bana taamasiɲɛw ka ca, fura in bɛ di kalo o kalo walasa ka dɔ bɔ u caya ni u juguya la.

Furakɛli min bɛ kɛ ni farikolojidɛsɛbana ye

• Tile lakanani:O de ye ko ye min nafa ka bon kosɛbɛ. Bolo janw donli, finiw donli, ani tilekunfura kɛli ni a tangacogo ka bon, i n’a fɔ SPF 50+, o nafa ka bon.
• Flebotomie : O ye ka joli dɔɔni (unit kelen ɲɔgɔn) bɔ farikolo la dɔgɔkun damadɔ o dɔgɔkun damadɔ. O bɛ dɛmɛ ka nɛgɛ wɛrɛ hakɛ dɔgɔya farikolo la ani ka dɛmɛ don ka porfiriniw bɔ minnu dalajɛlen don sugunɛ na.
• Fura minnu bɛ kɛ : Fura minnu hakɛ ka dɔgɔn i n’a fɔ kulorokini walima kulorokini (hydroxychloroquine) olu bɛ se ka pɔrfiriniw bɔli teliya sugunɛ na.