ʻAʻole anei ʻoe e pilikia i ka hoʻōki ʻana i ke kahe ʻana o ke koko mai kahi ʻoki liʻiliʻi? E kamaʻilio kākou e pili ana i ka maʻi von Willebrand!

Loaʻa iā ʻoe kekahi ʻoki liʻiliʻi ʻaʻole e pau ke kahe koko no ka manawa lōʻihi? A i ʻole he pinepine kou kahe koko ihu? Loaʻa paha i kekahi o kou ʻohana nā pilikia like? Inā pēlā, he mea nui paha kēia iā ʻoe. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi kakaʻikahi akā maʻamau loa i kapa ʻia ʻo ka maʻi von Willebrand .

He aha ka maʻi von Willebrand?

I ka ʻōlelo maʻalahi, ʻo ka maʻi von Willebrand kahi maʻi i hoʻopili pono ʻole ai ko mākou koko, ʻo ia hoʻi, ua nāwaliwali ke kaʻina hana e kōkua ai i ka hoʻōki ʻana i ke koko. He mea hoʻoilina pinepine kēia, ʻo ia hoʻi, hiki i nā mākua ke hoʻoili i ka maʻi i kā lākou mau keiki. Akā, mai hopohopo, aia kahi lāʻau no kēia. Hiki i nā kauka ke hāʻawi iā ʻoe i nā lāʻau lapaʻau e kōkua ai i kēia kaʻina hana hoʻopili koko.

He aha ka mea e hiki mai ana i kahi kanaka me ka maʻi von Willebrand?

Hiki i ke kanaka me kēia maʻi ke kahe koko ma mua o ka maʻamau. E noʻonoʻo wale, ʻoiai he ʻoki liʻiliʻi a he unuhi niho paha e lōʻihi ka manawa e hoʻōki ai i ke kahe koko. Loaʻa i kekahi poʻe ke kahe pinepine i ka ihu. No nā wahine, hiki iā lākou ke kahe nui i ka wā o ka menstruation, a i ʻole hiki iā lākou ke kahe nui ma hope o ka hānau ʻana.

I nā hihia koʻikoʻi loa o ka maʻi, hiki ke kahe ke koko i kekahi manawa me ke kumu ʻole i loko o nā hono a i ʻole nā ​​​​ʻiʻo palupalu. Hiki i kēia ke hoʻoulu i ka pehu a me ka ʻeha nui. No kekahi poʻe, hiki iā ia ke alakaʻi i ka anemia , ʻo ia hoʻi ka nele o ke koko .

Pehea ka maʻamau o kēia ʻano?

ʻO kēia ka maʻi kahe koko maʻamau loa ma ka honua. Manaʻo kekahi mau hōʻike e pili ana ia ma kahi o 1% o ka heluna kanaka ʻAmelika. Ma ka honua holoʻokoʻa, ua manaʻo ʻia ma waena o 23 a me 110 no ka miliona mau kānaka e loaʻa paha ke ʻano. Eia nō naʻe, hiki ke ʻokoʻa kēia mau helu, no ka mea, he mau pilikia kahe koko paha kekahi poʻe akā ʻaʻole i ʻike ʻia me ka maʻi von Willebrand . Ua loaʻa paha i kekahi poʻe kēia mau pilikia no nā makahiki, akā ʻaʻole i ʻike ʻia a hiki i ka wā lohi.

He like anei ka maʻi von Willebrand a me ka hemophilia?

ʻAe, ʻoiai ʻo nā mea ʻelua he mau pilikia koko koko, ʻo ka maʻi von Willebrand kahi maʻi i emi pinepine nā hōʻailona a ʻoi aku ka liʻiliʻi o ka ʻino ma mua o ka hemophilia . Akā, ʻaʻole ia he manaʻo he mea ia e hoʻowahāwahā ʻia.

He aha nā hōʻailona o ka maʻi von Willebrand?

He nui ka poʻe me ka maʻi ʻaʻohe o lākou hōʻailona iki , a i ʻole he mau hōʻailona iki wale nō. Eia nō naʻe, hiki i ka poʻe me ka maʻi ʻoi aku ka koʻikoʻi ke ʻike i nā hōʻailona e like me:

• Kahe koko ihu : ʻAʻole kēia he kahe koko maʻamau. Hiki ke kahe koko no nā minuke he 10 a ʻoi, a i ʻole ke kahe koko mai ka ihu ma mua o ʻelima mau manawa i ka makahiki.
• Ke kahe ʻana o ke koko mai kahi ʻeha: Ke kahe ʻana o ke koko mai kahi ʻoki liʻiliʻi a i ʻole kekahi ʻeha ʻē aʻe no nā minuke he 10 a ʻoi.
• Nā palapū: Loaʻa maʻalahi i ka poʻe me kēia maʻi nā palapū. ʻAʻole wale kēia mau palapū e pālahalaha ma ka ʻili, akā hiki ke pehu iki a hāpai ʻia e like me kahi puʻupuʻu. Hiki i kēia mau palapū ke ʻoi aku ka nui ma mua o ke kālā ʻelima rupee.
• Ka nele i ka haoʻAneemia nele i ka hao: ʻO ka anemia kahi kūlana i loaʻa ʻole ai i ke kino nā hunaola koko ʻulaʻula lawa. Hana ʻia ka anemia nele i ka hao ke loaʻa ʻole i ko mākou kino ka hao lawa e hana ai i ka hemoglobin . Kōkua kēia mea, i kapa ʻia ʻo hemoglobin, i nā hunaola koko ʻulaʻula e lawe i ka oxygen. No laila, ke nalowale nui ke koko, hiki iā mākou ke nalowale i ka hao a loaʻa i kēia kūlana.
• Ke kahe koko ma hope o ke ʻoki ʻana: Hiki i ka poʻe me kēia maʻi ke kahe nui ke koko, ʻoiai ma hope o ke ʻoki liʻiliʻi, e like me ka unuhi ʻana o ka niho.
• Ka menstruation kaumaha: No kekahi mau wahine, inā kaumaha loa ke kahe ʻana o ke koko o ko lākou menstruation a pono lākou e hoʻololi i kā lākou pad a tampon paha i kēlā me kēia hola, a inā ʻoi aku ka lōʻihi o ko lākou menstruation ma mua o ʻehiku mau lā, he hōʻailona paha ia o kēia ʻano.
• Ke kahe koko nui ma hope o ka hānau keiki ʻana a i ʻole ka hāʻule ʻana o kahi keiki.
• Koko i loko o ka ʻōpala: Inā he koko kou i loko o kou ʻōpala a i ʻole ke koko ma hope o kou hele ʻana i ka ʻōpala, he hōʻailona paha ia o kekahi maʻi ʻē aʻe. No laila , pono ʻoe e ʻike i ke kauka .
• Koko i loko o ka mimi (hematuria): Inā ʻike ʻoe i ke koko i kou mimi ʻana, ʻoiai inā hele pū ke koko me ka makemake koke e mimi, e haʻi koke aku i ke kauka .

ʻO ka mea nui loa, inā loaʻa iā ʻoe kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​mai hoʻowahāwahā iā lākou a e ʻimi i ke kauka no ka ʻōlelo aʻo.

He aha ke kumu o ka maʻi von Willebrand?

He maʻi hoʻoilina kēia. ʻO ke ʻano kēia, ua hana ʻia kēia maʻi e kahi loli (mutation) i loko o kā mākou mau genes. Ma muli o kēia mau loli hoʻoilina, ʻaʻole hiki i ko mākou kino ke hana pono i kahi protein i kapa ʻia ʻo von Willebrand factor . ʻO kēia mau mea he mau protein e kōkua i ko mākou koko.

Loaʻa kēia von Willebrand factor i loko o ka plasma (ka ʻāpana wai o ko kākou koko), nā platelets (nā pūnaewele e kōkua i ke kāpili ʻana o ke koko), a me nā paia o nā kīʻaha koko . ʻO ka maʻamau, ke hōʻino ʻia kahi kīʻaha koko, pili nā platelets i ka wahi i hōʻino ʻia, e hana ana i kahi ʻūpā koko a hoʻōki i ke kahe ʻana o ke koko.ʻO ka Von Willebrand factor ka mea e kōkua ai i kēia mau platelets e hoʻopili pono. No laila, ke nalowale ʻoe i ka lawa o kēia factor, a inā nalowale loa ʻoe, ʻaʻole hiki i nā platelets ke hoʻopili pono. A laila lōʻihi ka manawa e hana ai i kahi clot koko.

Loaʻa i ka hapa nui o ka poʻe ka maʻi ma ka hoʻoilina ʻana i kahi gene mutated mai kekahi o ko lākou mau mākua . Ua kapa ʻia kēia he autosomal dominant inheritance . Hiki i kekahi poʻe ke hoʻoilina i ka gene mutated mai nā mākua ʻelua . Ua kapa ʻia kēia he autosomal recessive inheritance , a ʻo ia ke ʻano koʻikoʻi loa o ka maʻi. ʻO ke kanaka nāna e lawe i kēia gene mutated he 50% ka manawa e hoʻoili i ka mutation i kā lākou mau keiki.

Eia kekahi, i kekahi manawa hiki ke loaʻa ka maʻi von Willebrand ma ke ʻano he pilikia o nā maʻi ʻē aʻe, e like me ke kanesa , nā maʻi autoimmune , nā maʻi puʻuwai, a me nā maʻi kīʻaha koko.

Pehea e ʻike ai nā kauka i ka maʻi von Willebrand?

Ke ʻike ʻoe i ke kauka, e nīnau mua lākou iā ʻoe e pili ana i kāu mau hōʻailona. Hiki iā lākou ke nīnau inā ua loaʻa i kekahi o kou ʻohana nā hōʻailona like a i ʻole nā ​​​​maʻi koko. A laila hiki iā lākou ke hana i kekahi mau hoʻokolohua, e like me:

• Helu koko piha (CBC): Ke ana kēia i ka helu o nā hunaola koko ʻulaʻula, nā ʻano hunaola koko keʻokeʻo like ʻole, a me nā platelets i loko o kou koko. Ke ana pū nei hoʻi ia i ka nui o ka hemoglobin i loko o kou mau hunaola koko ʻulaʻula. ʻO ka hapa nui o ka poʻe me ka maʻi von Willebrand he CBC maʻamau ko lākou. Eia nō naʻe, ʻo ka poʻe e kahe nui ana ke koko, he haʻahaʻa paha ko lākou hemoglobin a he haʻahaʻa hoʻi ka helu o nā hunaola koko ʻulaʻula.
• Nā hoʻāʻo hōʻuluʻulu platelet: Hoʻāʻo kēia i ka maikaʻi o ka hoʻopili ʻana o kāu mau platelets e kōkua i ke hana ʻana i nā ʻūpā koko.
• Hoʻāʻo manawa thromboplastin hapa i hoʻāla ʻia (APTT): Hoʻāʻo kēia no ka von Willebrand factor a me nā mea hana clotting ʻē aʻe e kōkua i ke kāpili ʻana o ke koko. Inā haʻahaʻa nā pae o kēia mau mea ma mua o ka maʻamau, ʻoi aku ka lōʻihi o ke kāpili ʻana o ke koko.
• Hoʻāʻo manawa Prothrombin (PT): Ke ana pū nei kēia i nā mea hana ʻē aʻe o ke koko.
• Hoʻāʻo Fibrinogen:ʻO Fibrinogen kekahi protein ʻē aʻe e kōkua ai i ke kāpili ʻana o ke koko.
• Hoʻāʻo antigen Von Willebrand: Ke ana kēia i ka nui o ka protein von Willebrand factor i loko o kou koko.
• Hoʻāʻo cofactor Ristocetin: Loiloi kēia i ka hana o ka von Willebrand factor.
• Hoʻāʻo multimers kumu Von Willebrand: Nānā kēia i ke ʻano o ka kumu.

Pono paha kāu kauka e hana i kekahi mau hoʻokolohua koko e hōʻoia i ka maʻi, no ka mea, hiki i nā mea e like me nā pae hormone ke hoʻololi i ke kiʻekiʻe o ka von Willebrand factor i loko o kou koko.

Aia kekahi mau ʻano o ka maʻi von Willebrand. E hana nā kauka i nā hoʻokolohua hou aʻe e ʻike pono i ke ʻano āu i loaʻa ai. ʻO kēia mau ʻano:

• ʻAno 1: ʻO kēia ke ʻano maʻamau. Ma waena o 60% a me 80% o nā maʻi i loaʻa i kēia ʻano. He haʻahaʻa nā pae o ka von Willebrand factor i loko o ko lākou koko o kēia poʻe. ʻAʻole paha lākou e loaʻa nā hōʻailona. Inā loaʻa, he akahai loa lākou.
• ʻAno 2: Ma kēia ʻano, ʻaʻole hana pono ka von Willebrand factor. Hiki i kēia poʻe ke loaʻa i ke kahe koko iki a kaulike. Ma waena o 15% a me 30% o nā maʻi i loaʻa i kēia ʻano.
• ʻAno 3: ʻO kēia ke ʻano koʻikoʻi loa. He mea kākaʻikahi loa hoʻi. Hoʻopilikia ia ma waena o 5% a me 10% o nā maʻi. He haʻahaʻa loa nā pae o ka von Willebrand factor o kēia poʻe i ko lākou koko, a ʻaʻohe paha. Hiki i kēia ke hana i nā pilikia kahe koko koʻikoʻi.

He aha nā lāʻau lapaʻau no ka maʻi von Willebrand?

Hoʻoponopono nā kauka i kēia maʻi me nā ʻano lāʻau like ʻole:

• (Desmopressin): He hormone kēia. Hoʻonui ia i ka pae o ka von Willebrand factor i loko o ke koko. ʻO kēia ka lāʻau lapaʻau maʻamau no kēia maʻi.
• Nā hoʻokomo ʻana o Von Willebrand factor: Hāʻawi ʻia kekahi poʻe i kēia factor ma o kahi aʻa koko e hoʻōki i ke kahe ʻana o ke koko. Hiki ke hāʻawi ʻia kēia lāʻau lapaʻau ma mua o ke ʻoki ʻana. Pono paha kekahi poʻe me ka maʻi koʻikoʻi e loaʻa i kēia lāʻau lapaʻau i nā manawa a pau e mālama i nā pae factor i loko o ko lākou koko i paʻa.
• (Nā lāʻau antifibrinolytic):Hana kēia mau lāʻau lapaʻau ma ka pale ʻana i ke kūkulu ʻia ʻana o nā ʻūpā koko. Hiki i kāu kauka ke kuhikuhi i kēia mau lāʻau lapaʻau inā e hana ʻia ana ʻoe i ke ʻoki niho a inā paha e kahe nui ana ke koko o ka menstrual.
• Nā lāʻau hoʻomalu hānau: Kōkua kēia i ka poʻe i loaʻa ke kahe koko nui i ka menstrual. Hoʻonui ka hormone estrogen i loko o kēia mau lāʻau i ka pae o ka von Willebrand factor i loko o ke koko.

Hiki iaʻu ke hōʻemi i ka pilikia o ka loaʻa ʻana o kēia maʻi?

He maʻi hoʻoilina pinepine kēia. Inā loaʻa i kou mau mākua kēia maʻi, hiki iā ʻoe ke hoʻoilina mai kekahi a i ʻole mai lāua ʻelua. No laila, ʻaʻohe mea hiki iā mākou ke hana e pale aku ai i kona ulu ʻana.

Inā loaʻa iaʻu kēia maʻi, he aha kaʻu e manaʻo ai?

Hiki i nā kauka ke mālama i ka maʻi von Willebrand, akā ʻaʻole hiki iā lākou ke ho'ōla loa . Loaʻa i ka hapa nui o ka poʻe ke ʻano 1 a i ʻole ke ʻano 2. Pono lākou i ka mālama ʻana inā loaʻa iā lākou kahi ʻeha a pono paha ke ʻoki ʻana. Pono paha ka poʻe me ke ʻano 3 i ka mālama lapaʻau e hoʻomau ʻia e kāohi i ke kahe ʻana o ke koko.

Loaʻa iaʻu ka maʻi von Willebrand. Pehea lā wau e mālama ai iaʻu iho?

Ma muli o ka hapa nui o ka poʻe he mau hōʻailona ʻoluʻolu a kaulike paha, ʻo ka noho ʻana me kēia maʻi ke ʻano o ka noʻonoʻo ʻana i kekahi mau mea:

• E hōʻalo i nā hana e hiki ai ke hōʻeha: ʻOi aku ka maikaʻi o ka noho mamao ʻana mai nā haʻuki hopena kiʻekiʻe, e like me ka pôpeku, ka rugby, a me ka hockey.
• E haʻi aku i kāu mau kauka a pau, me kāu kauka niho, ua loaʻa iā ʻoe kēia maʻi: a laila hiki iā lākou ke hoʻolālā pehea e kāohi ai i ke kahe ʻana o ke koko ma hope o ke ʻoki ʻana.
• Mai hoʻohana i ka aspirin a i ʻole nā ​​​​lāʻau lapaʻau i loaʻa ka aspirin.
• Mai hoʻohana i nā lāʻau anti-inflammatory nonsteroidal (NSAIDs) , e like me ka ibuprofen, ke ʻole i kauoha ʻia e ke kauka nāna i ʻike he maʻi von Willebrand kou.
• E hōʻalo i ka lawe ʻana i nā mea hoʻopihapiha meaʻai e loaʻa ana ka huaora E, ka ʻaila iʻa, a me ka turmeric.
• E noʻonoʻo i kahi palapala hōʻoia lapaʻau: ʻO ke kau ʻana i kahi kūpeʻe lima e like me kēia a i ʻole ka lawe ʻana i kahi kāleka hōʻoia e hiki ke kōkua iā ʻoe e loaʻa ka mālama lapaʻau kūpono i ka wā pilikia.

Āhea wau e hele wikiwiki ai i ka haukapila?

E hele koke i ka haukapila i kēlā me kēia manawa ke kahe nei ke koko me ka hiki ʻole ke kāohi . ʻOiai inā he ʻeha liʻiliʻi ia, inā ʻaʻole e pau ke kahe ʻana o ke koko, e ʻimi pono i ke kōkua lapaʻau.

He aha nā nīnau e pono iaʻu e nīnau aku i ke kauka?

Inā loaʻa iā ʻoe ka maʻi von Willebrand, loaʻa paha iā ʻoe nā nīnau e pili ana i ka hopena o ka maʻi i kou ola. Eia kekahi mau nīnau hiki iā ʻoe ke nīnau i kāu kauka:

• No ke aha i loaʻa ai iaʻu kēia maʻi?
• Hiki i kaʻu mau keiki ke hoʻoilina i kēia maʻi?
• E lilo anei kēia i mea ʻino aʻe?
• He aha nā lāʻau lapaʻau no kēia?
• He aha nā hopena ʻaoʻao o ka lāʻau lapaʻau?
• ʻAʻole anei e hiki iaʻu ke hana i kekahi mau mea, e like me ka huakaʻi a i ʻole ka pāʻani haʻuki, ma muli o kēia maʻi?

I ka hōʻuluʻulu manaʻo, ʻo nā mea e pono ai mākou e hoʻomanaʻo

He maʻi maʻamau loa ka maʻi Von Willebrand , he maʻi hoʻoilina koko. Loaʻa i ka hapa nui o ka poʻe nā hōʻailona iki a waena. Loaʻa paha iā lākou ke kahe pinepine ʻana o ka ihu, a i ʻole nā ​​ʻoki liʻiliʻi e lōʻihi ka manawa e hoʻōki ai i ke kahe ʻana o ke koko. Loaʻa i kekahi poʻe nā hōʻailona koʻikoʻi. No ka laʻana, loaʻa paha ke kahe ʻana o ke koko i loko o ko lākou mau hono a me ka ʻeha o nā hono.

ʻAʻole paha e ʻike kekahi poʻe he maʻi ko lākou no nā makahiki. Inā loaʻa iā ʻoe, he mea hōʻoluʻolu paha ka ʻike ʻana i ke kumu o kou pilikia koko. E hopohopo paha ʻoe e loaʻa pū ana i kāu mau keiki ke maʻi. ʻOiai ʻaʻole hiki i nā kauka ke ho'ōla i ke maʻi, hiki iā lākou ke mālama iā ia . Hiki iā lākou ke pane i kāu mau nīnau e pili ana i ka hoʻoilina ʻana o kāu mau keiki. Hiki iā lākou ke hāʻawi iā ʻoe i ka ʻike e pono ai ʻoe e ola me ka maʻi von Willebrand i ʻole e pale iā ʻoe mai ka noho ʻana i kahi ola maʻamau a ikaika. No laila, mai hopohopo, inā maopopo iā ʻoe a hoʻokele pono iā ia, hiki iā ʻoe ke ola maikaʻi me ia.