He mau cysts kou i loko o kou mau puʻupaʻa? E kamaʻilio kākou e pili ana i ka ADPKD (Autosomal Dominant Polycystic Kidney Disease)

Malia paha he maʻi puʻupaʻa ko kekahi o kou ʻohana. A i ʻole ua ʻike ʻia ʻoe me ke koko kiʻekiʻe i kou mau makahiki 30 a 40 paha a ke ʻimi nei ʻoe i ke kumu. I kēia lā, ke kamaʻilio nei mākou e pili ana i kahi maʻi hoʻoilina ʻokoʻa iki, akā maʻamau loa, e hoʻopilikia ana i nā puʻupaʻa. Ua kapa ʻia kēia ma ke ʻano lapaʻau ʻo Autosomal Dominant Polycystic Kidney Disease. No ka mea, ua lōʻihi loa ia no mākou, e kapa mākou iā ia ʻo ADPKD no ka pōkole.

I ka ʻōlelo maʻalahi, he aha ka ADPKD?

I ka ʻōlelo maʻalahi, hoʻoulu kēia maʻi i ka nui o nā cysts i piha i ka wai e hana i loko o kou mau puʻupaʻa. E noʻonoʻo i nā puʻupaʻa e like me nā kānana i loko o ko mākou kino. I ka ulu ʻana o kēia mau cysts, ʻaʻole hiki i nā puʻupaʻa ke hana pono. Hiki i kēia ke alakaʻi i nā pilikia olakino e like me ke koko kiʻekiʻe, nā maʻi ʻōpū mimi, a me nā pōhaku puʻupaʻa. ʻOiai ʻaʻole loaʻa i nā kānaka a pau kēia maʻi, hiki i kekahi poʻe ke hele a hiki i ka hāʻule ʻana o ka puʻupaʻa.

ʻO kekahi o nā hiʻohiʻona kūikawā o kēia maʻi, ʻo ia ka hiki ʻole iā ʻoe ke loaʻa kekahi mau hōʻailona no nā makahiki ʻoiai inā loaʻa iā ʻoe. Hoʻomaka pinepine nā hōʻailona e ʻike ʻia ma waena o nā makahiki 30 a me 40. ʻO ia ke kumu i kapa ʻia ai ʻo "adult PKD."

He aha nā hōʻailona o ADPKD?

ʻAʻole nā ​​kānaka a pau me ADPKD e loaʻa nā hōʻailona. No kekahi poʻe, hiki ke hala nā makahiki e ʻike ʻia ai nā hōʻailona. E nānā kākou i kekahi o nā hōʻailona maʻamau e pili ana i kēia maʻi. E hoʻokaʻawale wau i kēia i ʻelua ʻāpana no ʻoe e hoʻomaopopo ai.

No ke aha i loaʻa ai ʻo ADPKD? He mea hoʻoilina anei ia?

ʻAe, he maʻi hoʻoilina kēia. ʻO ke kumu nui o kēia he kīnā i ʻelua mau genes i loko o kā mākou DNA, ʻo ia hoʻi ʻo `PKD1` a me `PKD2`. ʻO kēia mau genes nā mea e hāʻawi i nā hōʻailona i nā pūnaewele puʻupaʻa "i ka wā e ulu ai". Ke loaʻa kahi kīnā i kekahi o kēia mau genes, ulu nā pūnaewele puʻupaʻa me ke kāohi ʻole a hana i kēlā mau cysts a mākou i ʻōlelo ai.

E noʻonoʻo e pili ana i ia mea, ʻo ka hapa nui o nā maʻi hoʻoilina e pono ai kahi gene kīnā mai nā mākua ʻelua. Akā ma ADPKD, pono wale nō ke gene kīnā e hele mai mai hoʻokahi makua . ʻO ia ke kumu i kapa ʻia ai ʻo "autosomal dominant."

ʻO ke ʻano kēia, inā loaʻa i kekahi o kou mau mākua ka maʻi, he 50% kou manawa e loaʻa ai . Eia nō naʻe, he mea kakaikahi loa, hiki ke loaʻa pū ka maʻi ma muli o kahi loli maʻamau i ka gene o ke kanaka, me ka ʻole o ka loaʻa ʻana o ka maʻi i nā mākua ʻelua.

Pehea ʻoe e ʻike ai inā loaʻa iā ʻoe kēia maʻi?

Inā kānalua kāu kauka i kahi pilikia puʻupaʻa ma muli o kāu mau hōʻailona, ​​​​hiki iā ia ke kuhikuhi iā ʻoe i kahi loea puʻupaʻa (nephrologist). E nīnau ke kauka iā ʻoe i nā nīnau e like me:

• He aha kou mau hōʻailona a i ka manawa hea i hoʻomaka ai?
• Ua ʻike anei ʻoe i kou koko?
• Aia ka ʻeha ma kahi? Inā pēlā, ma hea?
• Ua loaʻa anei iā ʻoe nā pōhaku puʻupaʻa ma mua?
• Aia kekahi o ka ʻohana i loaʻa i ka maʻi puʻupaʻa?

A laila e kauoha ke kauka i kekahi mau hoʻokolohua e nānā i kou mau puʻupaʻa.

• ʻO ke kiʻi ʻana i ka ultrasound: Hoʻohana kēia i nā nalu kani e kiʻi i nā kiʻi o loko o ke kino. ʻO kēia ka hoʻāʻo mua i hana ʻia.
• MRI scan: He scan ikaika e hoʻohana ana i nā magnet a me nā nalu lekiō e ʻike i nā ʻōpū liʻiliʻi loa e ʻike ʻia me ka ultrasound.
• CT Scan: ʻO kekahi ʻano hana scan ikaika e hoʻohana ana i nā kukuna X e kiʻi i nā kiʻi kikoʻī o ke kino.

Eia kekahi, hiki ke hoʻāʻo ʻia kāu DNA e ʻike inā loaʻa iā ʻoe ka gene kīnā ʻole ʻo `PKD1` a i ʻole `PKD2`. Eia nō naʻe, ʻoiai hiki i kēia hoʻāʻo genetic ke hōʻike inā loaʻa iā ʻoe ka gene kīnā ʻole, ʻaʻole hiki iā ia ke wānana i ka wā e ulu ai ka maʻi a i ʻole pehea kona koʻikoʻi .

ʻO ka mālama ʻana a me nā mea hiki iā ʻoe ke hana ma ka home

ʻAʻohe lāʻau lapaʻau no ADPKD i kēia manawa. Akā, nui nā mea hiki iā kākou ke hana e mālama ai i nā pilikia olakino i hoʻokumu ʻia e ka maʻi a pale i ka hāʻule ʻana o nā puʻupaʻa.

Lapaʻau lapaʻau

• Nā lāʻau lapaʻau e hoʻolohi i ka hāʻule ʻana o ka puʻupaʻa: No nā pākeke i pilikia i ka holomua wikiwiki o ka maʻi, hāʻawi ʻia nā lāʻau lapaʻau e like me `Tolvaptan (Jynarque)` e hoʻolohi i ka emi ʻana o ka hana o ka puʻupaʻa.
• Nā lāʻau lapaʻau e kaohi ai i ke koko kiʻekiʻe.
• Nā lāʻau antibiotic no nā maʻi ʻōpū mimi.
• Nā lāʻau hoʻopau ʻeha.

Inā hāʻule kou mau puʻupaʻa, pono paha ʻoe e hana i kahi lāʻau i kapa ʻia ʻo dialysis . Pili kēia i ka hoʻohana ʻana i kahi mīkini e kānana i kou koko, e wehe ana i nā huahana ʻōpala, ka paʻakai, a me ka wai keu mai kou kino. ʻO kekahi koho ʻē aʻe he hoʻoili puʻupaʻa. E kamaʻilio me kāu kauka e pili ana i kēia e aʻo hou aku.

Nā mea hiki iā ʻoe ke hana ma ka home

I mea e pale ai i kou mau puʻupaʻa a mālama iā lākou e hana pono no ka lōʻihi loa, he mea nui e noho olakino e like me ka hiki.

• ʻAi kūpono: E ʻai i ka ʻai kaulike haʻahaʻa i ka paʻakai. Hiki i ka paʻakai ke hoʻonui i ke koko.
• E noho hana: Hiki i ka hoʻoikaika kino ke kōkua i ka kaohi ʻana i ke kaumaha a me ke koko, akā e pale i nā haʻuki pili, hiki ke hōʻino i kou mau puʻupaʻa.
• Hōʻalo i ka puhipaka: Hoʻopōʻino ka puhipaka i nā kīʻaha koko i loko o nā puʻupaʻa a hiki ke hoʻonui i ka pilikia o ka hoʻokumu ʻia ʻana o nā cysts.
• E inu i ka wai he nui: Hiki i ka maloʻo ke hana pinepine i nā cysts.

Nā pilikia ʻē aʻe e hiki ke kū mai ma muli o ADPKD

Hiki i ka ADPKD ke hoʻonui i ka pilikia o nā pilikia olakino ʻē aʻe, a he mea nui e ʻike i kēia mau mea.

ʻO ka wikiwiki o ka holomua ʻana o ka maʻi e pili ana i ka loaʻa ʻana o kahi kīnā i loko o ka gene `PKD1` a i ʻole `PKD2`. Ma keʻano laulā, ʻoi aku ka pilikia o ka poʻe me kahi kīnā i loko o ka gene `PKD1` i ka loaʻa ʻana o ka hāʻule ʻana o ka puʻupaʻa ma mua o ka poʻe me kahi kīnā `PKD2`. No laila, ʻoi aku ka maikaʻi o ke kamaʻilio ʻana me kāu kauka e ʻike pono i kou kūlana.