Ua ʻike anei ʻoe i ka Hemophilia C? E kamaʻilio kākou e pili ana i kēia maʻi laha ʻole!

I kekahi manawa, ʻoiai he ʻoki liʻiliʻi, pono ka manawa e hoʻōki ai i ke kahe ʻana o ke koko, ʻeā? A i ʻole ua lohe paha ʻoe ua loaʻa i kekahi o kou ʻohana kahi maʻi e pili ana i ke koko? I kēia lā, e kamaʻilio mākou e pili ana i kahi maʻi koko i kakaʻikahi iki, akā he mea nui loa ia e ʻike. Ua kapa ʻia kēia ʻo Hemophilia C.

He aha ka Hemophilia C?

I ka ʻōlelo maʻalahi, he ʻano hemophilia kakaikahi loa ka hemophilia C. ʻO ka hemophilia kahi kūlana i ʻole e paʻa pono ai kou koko, ʻo ia hoʻi, ke kahe ke koko ʻoe, e pau mālie a ʻaʻole paha e pau. Loaʻa i ka poʻe me ka hemophilia C kahi protein koko kūikawā i kapa ʻia he clotting factor , i kapa ʻia ʻo Factor XI , e kōkua ana i ke kāpili ʻana o ke koko. Ua kapa ʻia kēia ʻo Factor XI Deficiency a i kekahi manawa ua kapa ʻia ʻo Rosenthal syndrome .

Eia nō naʻe, ʻoi aku ka maʻalahi o nā hōʻailona o ka hemophilia C ma mua o nā ʻano hemophilia ʻē aʻe (A a me B). Eia nō naʻe, hiki i ka poʻe me ka hemophilia C ke kahe koko ma mua o ka maʻamau i ka wā o ke ʻoki kino a i ʻole nā ​​​​​​hana niho. Hoʻōla nā kauka i kēia me ka plasma hau hou, i hana ʻia mai ke koko i hāʻawi ʻia. Kōkua kēia plasma i ke kāpili ʻana o ke koko.

He maʻi maʻamau anei ka hemophilia C?

ʻAʻole, he maʻi laha ʻole kēia. E noʻonoʻo, ma kahi ʻāina e like me ʻAmelika, ma kahi o hoʻokahi i kēlā me kēia 100,000 kāne a me nā wahine e loaʻa i ka hemophilia C. Inā mākou e hoʻohālikelike iā ia me nā ʻano hemophilia ʻē aʻe, hoʻopilikia ka hemophilia A ma kahi o 12 i kēlā me kēia 100,000 kāne, a hoʻopilikia ka hemophilia B ma kahi o 4 i kēlā me kēia 100,000 kāne. ʻAʻole maopopo i nā mea noiʻi i ka pinepine o ka hemophilia A a i ʻole B e hoʻopilikia ai i nā wahine.

He aha nā ʻokoʻa ma waena o ka hemophilia A, B, a me C?

ʻO nā ʻano hemophilia ʻekolu he mau maʻi koko hoʻoilina . ʻO ia hoʻi, hoʻopilikia ka hoʻololi ʻana o ka genetic i ke kaʻina hana koko. Akā aia kekahi mau ʻokoʻa liʻiliʻi ma waena o kēia mau ʻano ʻekolu. E nānā kākou i ke ʻano o lākou:

• Loaʻa ka Hemophilia A a me ka Hemophilia B i ka wā e hoʻoilina ai ke kanaka i kahi gene i hoʻololi ʻia mai kekahi o ko lākou mau mākua maoli. Eia nō naʻe, i ka hemophilia C, hiki ke hoʻoilina ʻia ka gene ʻano ʻē mai nā mākua ʻelua .
• ʻAʻole e like me ka poʻe me ka hemophilia A a i ʻole B, ʻaʻole pinepine ka poʻe me ka hemophilia C i nā pilikia kahe koko e hoʻopilikia ai i nā hono a i ʻole nā ​​​​ʻiʻo . He ʻokoʻa koʻikoʻi kēia.
• ʻO ka maʻamau, ʻo nā hōʻailona o ka poʻe me ka hemophilia A a i ʻole B ua hoʻoholo ʻia e nā pae o nā protein clotting, a i ʻole 'clotting factors', i loko o ko lākou koko. No ka laʻana, ʻo ka mea me ka hemophilia A koʻikoʻi e loaʻa nā pae haʻahaʻa loa o kēlā factor. Akā, ʻo ka mea kupanaha, ʻo ka mea me ka hemophilia C he haʻahaʻa loa paha nā pae o kēlā factor, akā he akahai loa paha kā lākou mau hōʻailona .
• Hiki ke loaʻa ka Hemophilia A a me B i nā kānaka o nā lāhui a me nā ʻano lāhui āpau. Eia nō naʻe, ʻoi aku ka maʻamau o ka hemophilia C i nā kānaka o ka hui Iudaio Ashkenazi . ʻAʻole kēia manaʻo ʻaʻohe mea ʻē aʻe e loaʻa iā ia, akā ʻoi aku ka maʻamau i waena o lākou.
• ʻOi aku ka maʻamau o ka Hemophilia A a me B i nā kāne ma mua o nā wahine, akā hoʻopilikia like ka hemophilia C i nā kāne a me nā wahine .

He aha nā hōʻailona o ka hemophilia C?

Hiki i ka poʻe me ka hemophilia C ke loaʻa i ke kahe koko mau loa a hiki ʻole ke hoʻōki ma hope o ke ʻoki kino nui, kahi ʻeha koʻikoʻi, a i ʻole ka hānau keiki ʻana. Eia naʻe, ʻaʻole lākou e ʻike i ke kahe koko koke , ʻo ia ka manawa e hoʻomaka ai ke kahe o ke koko me ke kumu ʻole.

Hoʻokahe pinepine ka poʻe me ka hemophilia C i ke koko ma hope o ke ʻoki niho, ka unuhi ʻana o ka niho, a i ʻole ka tonsillectomy.

ʻO nā hōʻailona ʻē aʻe paha:

• Ka kahe pinepine ʻana o ke koko ihu .
• Nā palapū ma ke kino mai kahi mea liʻiliʻi loa.
• Koko i loko o ka mimi .
• Ke kahe nui ʻana o ke koko ma hope o ke ʻoki poepoe ʻana.
• Nā palapū ʻeha a pehu ma hope o ke ʻoki ʻana.
• No nā wahine, hiki i ka menstruation ke mau no kekahi mau lā, kahi lōʻihi loa.

He aha nā kumu o ka hemophilia C?

He maʻi koko hoʻoilina ʻo Hemophilia C. Hana ʻia ia ke nele ʻoe i kahi protein koko i kapa ʻia ʻo Factor XI , kekahi o nā kumu clotting 13 e kōkua ana e hoʻolohi a hoʻōki paha i ke kahe ʻana o ke koko. ʻO kēia no ka mea ʻaʻohe ou gene F11 kūpono.

ʻO ka maʻamau, loaʻa i kēia gene F11 nā kuhikuhi no ka hana ʻana i ka Factor XI. Inā ua loli kēia gene, ʻo ia hoʻi, inā lilo ia i mea ʻē, ulu ka hemophilia C. ʻAʻole paha e hana kēia gene ʻē i ka Factor XI lawa, a i ʻole ʻaʻole paha e hana iki.

Loaʻa i nā kāne a me nā wahine ka hemophilia C inā hoʻoili nā mākua olaola ʻelua i ka gene mutated i kā lāua keiki . Inā hoʻoili kekahi i hoʻokahi gene F11 maʻamau a me hoʻokahi gene ʻē, he mea lawe ia kanaka o ka hemophilia C, akā ʻaʻole lākou e hōʻike i nā hōʻailona.

I kēia manawa, e nānā i ka mea e hiki ke hana ke loaʻa nā keiki i nā mākua me kēia gene ʻokoʻa:

• ʻO nā keiki i hānau ʻia i nā mākua me kēia hoʻololi ʻana o ka gene F11 he 25% ka manawa e loaʻa ai ka hemophilia C.
• ʻO nā keiki i hānau ʻia e kēlā mau mākua he 50% ka manawa e lilo i mea lawe i ka maʻi, ʻo ia hoʻi, lawe wale lākou i ka gene me ka ʻole o ka maʻi.
• He 25% ka manawa kūpono o nā keiki a kēlā mau mākua e hoʻoilina i ka gene F11 maʻamau.

Pehea e ʻike ai nā kauka i ka hemophilia C?

Ua ʻike anei ʻoe pehea e ʻike ai nā kauka i ka hemophilia C? E nānā mua lākou iā ʻoe ma ke kino . A laila e nīnau lākou e pili ana i kāu mau hōʻailona, ​​​​e like me ke kahe ʻana o ka ihu a i ʻole ke kahe ʻana o ke koko ma hope o ka hana niho. E nīnau pū lākou e pili ana i kou mōʻaukala ʻohana a inā he hemophilia a i ʻole nā ​​​​maʻi koko ʻē aʻe kekahi o kou ʻohana . He mea nui loa kēia ʻike i ka ʻike ʻana i ka maʻi.

He aha nā hoʻokolohua nui i hoʻohana ʻia e hōʻoia i ka maʻi?

E hoʻohana kāu kauka i ʻelua mau hoʻokolohua nui e hōʻoia i kāu hōʻoia:

• Hoʻāʻo manawa thromboplastin hapa i hoʻāla ʻia (APTT): He hoʻāʻo kēia i hoʻohana ʻia no ka ʻike ʻana i ka hemophilia. Hiki i kāu kauka ke ʻike i ka lōʻihi o ka manawa e hoʻopili ai kou koko .
• Hoʻāʻo kumu clotting: Hōʻike kēia hoʻāʻo koko i ke ʻano o ka hemophilia a me kona koʻikoʻi .

I kekahi mau hihia, hiki i kāu kauka ke noi aku i kekahi mau hoʻokolohua hou aʻe:

• Helu koko piha (CBC): Ke ana a me ke aʻo ʻana o kēia hoʻokolohua i nā hunaola koko.
• Hoʻāʻo manawa Prothrombin (PT): Nānā kēia i ka wikiwiki o ke kāpili ʻana o kou koko.
• Hoʻāʻo Fibrinogen: Ke ana kēia hoʻāʻo koko i ka nui o kahi protein koko i kapa ʻia ʻo fibrinogen, ka mea e kōkua ai i ke kāpili ʻana o ke koko.

He mea nui e hoʻomaopopo he paʻakikī ke ʻike i ka hemophilia C i kekahi manawa. No ke aha mai? ʻAʻole paha e pili nā hopena o ka hoʻāʻo koko, ʻoiai nā hopena o ka hoʻāʻo clotting factor, me nā hōʻailona o ka mea maʻi. No ka laʻana, hiki i kahi hoʻāʻo clotting factor ke hōʻike he haʻahaʻa loa kou pae factor, akā ʻaʻole paha ʻoe e loaʻa i kekahi mau hōʻailona. Eia kekahi, loaʻa paha iā ʻoe nā hōʻailona o ka hemophilia C ʻoiai inā maʻamau kou pae factor. No laila e noʻonoʻo ke kauka i kēia mau mea a pau a hiki i kahi hopena.

Pehea e mālama ʻia ai ka hemophilia C?

ʻAʻole paha e pono ka poʻe me ka hemophilia C i ka lāʻau lapaʻau a hiki i ka wā e hana ʻia ai ke ʻoki kino a i ʻole kekahi hana lapaʻau ʻē aʻe. Inā pono kēlā, hoʻohana nā kauka i ka 'plasma hau hou', i hana ʻia mai ke koko i hāʻawi ʻia.a me ka hui pū ʻana o nā lāʻau lapaʻau e hoʻōki i ka haki ʻana o nā 'clotting factors' i hāʻawi ʻia ma ke ʻano he pani. Inā loaʻa i nā wahine nā wā kaumaha loa, e mau ana i kekahi mau lā, hiki i nā kauka ke kuhikuhi i nā lāʻau hoʻomalu hānau .

Hiki ke pale ʻia ka hemophilia C?

ʻAʻole, he maʻi koko hoʻoilina ka hemophilia C, no laila ʻaʻole hiki ke pale ʻia . Eia nō naʻe, inā loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana ke ʻano, hiki i ke aʻo ʻana i nā ʻano genetic ke kōkua iā ʻoe e hoʻomaopopo i ka pilikia o ka hoʻoili ʻana i ia mea i nā hanauna e hiki mai ana.

He aha ka mea e hiki ai i kekahi me ka hemophilia C ke manaʻo?

Loaʻa i ka hapa nui o ka poʻe me ka hemophilia C nā hōʻailona iki . Kākaʻikahi lākou e hoʻoulu i nā pilikia olakino koʻikoʻi. ʻAʻole loaʻa i ka hapa nui o ka poʻe nā hōʻailona a hiki i ko lākou wā makua, a ʻo kēlā mau hōʻailona he ʻoluʻolu loa ia.

Eia nō naʻe, hiki iā ʻoe ke loaʻa nā pilikia kahe koko ma hope o ke ʻoki ʻana a i ʻole ka mālama ʻana i nā niho. Inā loaʻa iā ʻoe ka hemophilia C, he mea nui e nīnau i kāu kauka e pili ana i nā hana palekana āu e pono ai e lawe . Eia kekahi laʻana, e hoʻomaopopo i kāu kauka ma mua o ke ʻoki ʻana a me ka pale ʻana i kekahi mau lāʻau lapaʻau e hoʻonui ai i ke kahe koko (e like me ka aspirin).

ʻO ka mea hope loa, aia kekahi mau mea nui e pono ai ʻoe e hoʻomanaʻo

ʻAe, no laila, eia kekahi mau mea e pono ai ʻoe e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai:

• He maʻi koko laha ʻole ka Hemophilia C.
• ʻO ka maʻamau, he akahai loa kona mau hōʻailona a ʻaʻole pinepine ia e hoʻoulu i nā pilikia olakino nui.
• Hōʻike wale nā ​​​​kānaka he nui i nā hōʻailona i ka wā makua.
• Eia nō naʻe, hiki ke kahe ke koko ma mua o nā poʻe ʻē aʻe i ka wā o ke ʻoki ʻana a i ʻole ka mālama niho , no laila he mea nui e hoʻomaopopo i kāu kauka i ia mau hihia.
• Inā ua ʻike ʻia ʻoe me ka hemophilia C, mai hopohopo. E kamaʻilio me kāu kauka e hōʻoia ua maopopo iā ʻoe ka mea e pono ai ʻoe e ʻike a me nā hana palekana e pono ai ʻoe e hana .

E hoʻomanaʻo, ʻo ka loaʻa ʻana o ka ʻike kūpono i kekahi kūlana olakino ke kaʻina hana maikaʻi loa e hoʻoponopono ai iā ia! ʻAʻole ʻoe hoʻokahi, a loaʻa mau iā ʻoe ke aʻo a me ke kākoʻo lapaʻau.

ʻO Hemophilia C, ke koko ʻana, Factor XI, maʻi hoʻoilina, ke kahe ʻana o ke koko, nā hōʻailona, ​​ka lāʻau lapaʻau