Ke pilikia nei ʻoe a i ʻole kāu pēpē i ke kahe ʻana o ke koko mai kahi ʻoki liʻiliʻi? E kamaʻilio kākou e pili ana iā Hemophilia A

Ke loaʻa iā ʻoe kahi ʻeha liʻiliʻi ma kou kino, a i ʻole ke huki ʻia kou niho, ke hoʻomau nei ke kahe ʻana o ke koko me ka ʻole o ka pau ʻana? A i ʻole i kekahi manawa he mau palapū polū kou ma kou kino me ka ʻole o ka pā ʻana i kahi? Ke ʻike nei ʻoe i nā palapū ma kou kino holoʻokoʻa, ʻoiai ke hoʻomaka nei kāu keiki liʻiliʻi e kolo a hele paha? Inā hana ʻia nā mea e like me kēia, ʻo ke kumu paha he maʻi i kapa ʻia ʻo hemophilia . Mai makaʻu ke lohe ʻoe i kēia inoa. Me ka mālama kūpono , hiki iā ʻoe ke ola i kahi ola maʻamau a piha. E kamaʻilio kākou e pili ana i kēia me ka maopopo a me ka maʻalahi i kēia lā.

I ka ʻōlelo maʻalahi, he aha ka Hemophilia A?

I ka ʻōlelo maʻalahi, ʻo Hemophilia A kahi kūlana i ʻole e paʻa pono ai ko mākou koko. ʻO ka maʻamau, ke loaʻa iā mākou kahi ʻeha, paʻa ko mākou koko e hoʻōki i ke kahe ʻana. Kōkua ʻia kēia e nā protein kūikawā i loko o ko mākou koko. Kāhea mākou i kēia mau "nā mea paʻa koko."

ʻAʻole lawa ka hana a ke kanaka me ka hemophilia A i ka nui o ka clotting factor Factor VIII . Hoʻoholo ʻia ke koʻikoʻi o ka maʻi e ke kekelē o ka hoʻemi ʻia ʻana o kēia protein Factor VIII. No laila, ua māhele ʻia i ʻekolu ʻāpana:

• ʻO ka hemophilia ʻoluʻolu: Loaʻa nā pae Factor VIII i kekahi ʻano.
• Hemophilia waena: Haʻahaʻa loa nā pae Factor VIII.
• ʻO ka hemophilia koʻikoʻi: Haʻahaʻa loa a ʻaʻohe paha nā pae Factor VIII.

ʻO ka hapa nui o ka manawa, he maʻi hoʻoilina kēia e holo ana i loko o nā ʻohana. Eia nō naʻe, ma kahi o hoʻokahi hapakolu o nā hihia, hiki i kahi kanaka hou ke loaʻa i ka maʻi me ka ʻole o kekahi o ka ʻohana e loaʻa ana.

He aha ke kumu o ka hemophilia?

He mea kēia a kākou e hoʻoilina ai ma o nā genes. E noʻonoʻo e pili ana i ia mea, ua hoʻoholo ʻia kēlā me kēia ʻano o ko kākou kino e nā genes. Loaʻa i ka wahine ʻelua mau chromosome X (XX), aʻo ke kāne hoʻokahi chromosome X a hoʻokahi chromosome Y (XY).

Aia ke kīnā o ka gene e pili ana me ka hemophilia ma ka chromosome X.

No laila, inā he "mea lawe" ka makuahine o kēia gene kīnā, ʻaʻole ʻo ia e hōʻike pinepine i nā hōʻailona. No ka mea, ʻo kāna chromosome X olakino ʻē aʻe e hana i ka protein factor VIII e pono ai. Akā inā hoʻoilina kāna keiki kāne i kēia chromosome X kīnā, e loaʻa i kēlā keiki kāne ka hemophilia. No ka mea, hoʻokahi wale nō chromosome X o ke keikikāne. No kēia kumu, ʻoi aku ka maʻamau o ka hemophilia i nā keikikāne ma mua o nā kaikamahine.

He aha nā hōʻailona o ka hemophilia?

Pili nā hōʻailona i ke ʻano o kou kūlana inā he akahai, kaulike, a koʻikoʻi paha.

Nā hōʻailona hōʻike o ke kahe koko o ka lolo

Inā loaʻa i ke kanaka me ka hemophilia koʻikoʻi kahi hahau liʻiliʻi i ke poʻo, hiki ke kahe ke koko i loko o ka lolo. He pilikia koʻikoʻi kēia. Inā loaʻa iā ʻoe kahi ʻeha poʻo a me kekahi o nā hōʻailona ma lalo nei, e kelepona koke i kāu kauka a i ʻole e hele i ka keʻena pilikia kokoke loa (ETU).

• ʻO ke poʻo ʻeha mau loa
• Ka luaʻi ʻana
• Hiamoe pinepine a luhi nui paha
• Ka nāwaliwali koke a i ʻole ka paʻakikī i ka hele wāwae
• ʻIke pālua
• Nā hopu ʻana

Pehea e ʻike ai i ka maʻi?

Inā loaʻa i kekahi o kou ʻohana ka hemophilia, hiki iā ʻoe ke hoʻāʻo i kāu pēpē no ka maʻi i kou wā hāpai. Eia nō naʻe, aia kekahi mau pilikia e pili ana i kēia mau hoʻāʻo, no laila pono ʻoe e kūkākūkā me kāu kauka.

ʻIke pinepine ʻia nā hihia koʻikoʻi o ka hemophilia i nā keiki liʻiliʻi i loko o ka makahiki mua o ke ola. Inā hoʻomaka kāu keiki e ʻōwili a hoʻomaka e kolo a hoʻomaka e ʻike i nā palapū i hāpai ʻia ma ko lākou kino, e kamaʻilio me kāu kauka e pili ana i ia mea.

Hiki i ke kauka ke nīnau iā ʻoe i nā nīnau e like me kēia:

• Pehea i hiki mai ai kēia mau palapū a me ke kahe ʻana o ke koko?
• ʻEhia ka lōʻihi o ke kahe ʻana o ke koko?
• Aia kekahi mau lāʻau lapaʻau no nā keiki e lawe?
• Aia kekahi o kou ʻohana i loaʻa nā pilikia koko?

A laila, e hana ʻia kekahi mau hoʻāʻo koko e hōʻoia i ka hōʻailona. Hiki ke kuhikuhi ʻia ʻoe i kahi hematologist.

Pehea e mālama ʻia ai?

ʻO ke ʻano o ka mālama ʻana ma muli o ke koʻikoʻi o ka maʻi, kou mau makahiki, a me kou mau pono pilikino. ʻO ka pahuhopu nui o ka mālama ʻana, ʻo ia ke pani i ka protein factor VIII i nalowale i loko o ke kino. Ua kapa ʻia kēia ʻo ka factor replacement therapy . ʻOiai ʻaʻole kēia e ho'ōla loa i ka maʻi, hiki iā ia ke kōkua i ka kaohi ʻana i ke kahe koko a hiki iā ʻoe ke ola i kahi ola maʻamau.

ʻElua ʻano lapaʻau:

• Lapaʻau Palekana: Nā inikini mau o ka factor VIII ma kahi papa manawa i hoʻonohonoho ʻia e pale aku i ke kahe ʻana o ke koko, ma mua o ke kali ʻana i ke kahe ʻana o ke koko . He mea nui loa kēia no ka poʻe me ka hemophilia koʻikoʻi.
• Lapaʻau Inā Pono: Loaʻa ka lāʻau lapaʻau ke kahe wale nō ke koko .

Aia ʻelua ʻano o nā lāʻau pale o ka factor VIII:

1. Recombinant Factor VIII: Factor i hana ʻia ma ka hale hana me ka hoʻohana ʻana i ka ʻenekinia genetic. ʻO kēia ka mea i hoʻohana nui ʻia i kēia lā.

2. Factor VIII i loaʻa mai ka plasma: Factor i loaʻa mai ka plasma koko kanaka.

Hiki i nā poʻe me ka hemophilia A ʻoluʻolu a kaulike ke hoʻohana i kahi lāʻau lapaʻau i kapa ʻia ʻo Desmopressin (DDAVP) . Hana kēia ma ka hoʻokuʻu ʻana i ka factor VIII i mālama ʻia i loko o ke koko.

Eia kekahi, hoʻohana ʻia nā lāʻau lapaʻau waha e like me Tranexamic Acid, ka mea e pale ai i nā ʻūpā koko mai ka haki ʻana, i nā kūlana e like me ka unuhi ʻana o nā niho.

Pehea ʻoe e mālama ai i ke ola o kēlā me kēia lā?

I ke noho ʻana me ka hemophilia, pono ʻoe e noʻonoʻo hou aʻe e pili ana i ka palekana.

• E ʻeleu: Hoʻoikaika ka hoʻoikaika kino i nā ʻiʻo. Hāʻawi nā ʻiʻo ikaika i ka pale maikaʻi no nā hono. Eia nō naʻe, e pale i nā haʻuki pili e like me ka rugby a me ka mokomoko. ʻO ka ʻauʻau ʻana, ka hele wāwae ʻana, a me ke kaʻa paikikala (me ka mahiole) he mau koho maikaʻi. E kamaʻilio me kāu kauka e pili ana i nā hana kūpono iā ʻoe.
• E pale i nā keiki: Inā he keiki liʻiliʻi kāu, e ʻaʻahu i nā pale kuli, nā pale kuʻekuʻe, a me ka mahiole i ka wā e pāʻani ana. E pale iā ʻoe iho mai nā lako hale ʻoiʻoi i loko o kou home.
• Ola kino niho:E mālama i ka maʻemaʻe o kou niho. E palaki i kou mau niho i kēlā me kēia lā. Hiki i kēia ke kōkua i ka hōʻemi ʻana i ka pono no ka unuhi ʻana i nā niho a me nā mālama niho nui. Ma mua o kou hele ʻana i ke kauka niho, e hōʻoia e haʻi iā ia he hemophilia kou.
• Nā lāʻau lapaʻau ʻaʻole e lawe: Hiki i nā lāʻau ʻeha e like me ka aspirin a me nā NSAID (e.g., ibuprofen, diclofenac) ke kāohi hou aku i ke kāpili ʻana o ke koko. Mai lawe i kekahi lāʻau lapaʻau me ka ʻole o ke kūkākūkā ʻana me kāu kauka.