Loaʻa i kāu keiki liʻiliʻi kēia mau hōʻailona? E kamaʻilio kākou e pili ana i ka Alagille Syndrome!

Ua manaʻo paha ʻoe i kekahi manawa he paʻakikī iki kekahi o nā maʻi i loaʻa i kā mākou poʻe liʻiliʻi? ʻĀ, ʻo kekahi ʻano maʻi e pono ai ka mālama iki, akā hiki ke mālama ʻia inā loaʻa iā ʻoe ka ʻike kūpono , ua kapa ʻia ʻo Alagille Syndrome. Ua kapa ʻia kēia ʻo Alagille-Watson syndrome. He ʻano maʻi genetic kēia, ʻo ia hoʻi he mea ia a mākou e hoʻoilina ai mai ko mākou mau mākua. Hoʻopilikia nui ia i ko mākou akepaʻa a me ka puʻuwai. Eia naʻe, hiki ke hoʻopilikia ʻia nā ʻāpana ʻē aʻe o ke kino. E kamaʻilio kākou e pili ana i kēia me nā kikoʻī hou aku, ʻeā?

He aha maoli ka Alagille Syndrome?

I ka ʻōlelo maʻalahi, he maʻi hoʻoilina ka Alagille Syndrome. Hiki i kēia ke hoʻopilikia i ka hana o ko kākou akepaʻa, a me ke kumu o kekahi mau loli a me nā nāwaliwali i ke ʻano o ka puʻuwai, ʻo ia hoʻi, ke ʻano o ka hana ʻia ʻana o ka puʻuwai. Loaʻa i kekahi mau keiki i hānau ʻia me kēia maʻi kekahi mau hiʻohiʻona kikoʻī i ko lākou helehelena. ʻO kekahi o nā pilikia e kū mai ana mai kēia hiki ke lilo i mea weliweli loa a hiki i ka hoʻoweliweli i ke ola. ʻO ia ke kumu he mea nui loa ka makaʻala i kēia.

He aha nā ʻāpana o ke kino e hoʻopilikia ai ka Alagille Syndrome?

Ua ʻōlelo mua mākou e pili nui ana kēia i ke akepaʻa a me ka puʻuwai. Akā, ʻaʻole ia wale nō. Hiki iā ia ke hoʻopilikia i ka ulu ʻana o kekahi mau ʻāpana koʻikoʻi ʻē aʻe i loko o kou kino. Eia kēia mau mea:

• Puʻuwai: Hiki ke loaʻa nā pilikia me nā valves puʻuwai a me nā kīʻaha koko.
• Nā puʻupaʻa: Nā mea e like me ka emi ʻana o ka puʻupaʻa a me ka hoʻokumu ʻia ʻana o ka cyst.
• ʻAkeka: Hiki ke hoʻopilikia ʻia ka hana o ka ʻakeka, ka mea e kōkua ai i ke kaʻina hana hoʻoheheʻe.
• Nā maka: Kekahi pilikia o ka ʻike.
• Iwi iwi: Kekahi mau loli i nā iwi, e like me ka iwi kuamoʻo.
• Nā kīʻaha koko: Hiki ke loaʻa nā pilikia i loko o nā kīʻaha koko o ka lolo.

ʻO wai ka mea hiki ke loaʻa i kēia maʻi? Pehea ka maʻamau?

He maʻi hoʻoilina ka Alagille Syndrome. ʻO ia hoʻi, inā hoʻoilina ke keiki i ka gene mai ka makuahine a i ʻole ka makuakāne, hiki i ke keiki ke loaʻa i kēia mau hōʻailona. Ua kapa nā kauka i kēia he ʻano hoʻoilina "autosomal dominant". ʻO ia hoʻi, inā loaʻa i kekahi o nā mākua ka gene, hiki nō i ke keiki ke loaʻa i ke ʻano. ʻO ka maʻamau, ma waena o 30% a me 50% o nā hihia e hoʻoili ʻia mai ka ʻohana a i ka ʻohana ma kēia ʻano.

Eia nō naʻe, i kekahi manawa hiki ke ulu aʻe i loko o kahi kanaka hou, ʻoiai inā ʻaʻohe mea o ka ʻohana i loaʻa i ke ʻano ma mua. ʻO ia hoʻi, hiki ke ʻike ʻia me ka ʻole o ka mōʻaukala ʻohana.

Ma ke ʻano o ka maʻamau o kēia, ua manaʻo ʻia e pili ana kēia maʻi i hoʻokahi i loko o 30,000 a 45,000 mau kānaka.Akā, ʻaʻole kēia ka nui kūpono, no ka mea, i kekahi manawa hiki ke hele ʻole ʻia, a i ʻole hiki ke kuhihewa ʻia he maʻi ʻē aʻe no ka mea ʻo nā hōʻailona koʻikoʻi wale nō i mālama ʻia.

Pehea ka hopena o ka Alagille Syndrome i koʻu kino?

ʻOkoʻa nā hōʻailona o ka Alagille Syndrome mai kēlā kanaka a kēia kanaka. ʻO ke ʻano kēia, ʻaʻole e loaʻa i nā kānaka a pau me kēia maʻi nā hōʻailona like. ʻO ka mea e hana ʻia, ʻaʻole i ulu pono nā ʻauwai bile i loko o ko kākou ake. He pōkole loa lākou, he haiki, a he kakaikahi paha nā ʻauwai bile. ʻO kēia mau ʻauwai bile ka mea e lawe ai i ka bile i hana ʻia i loko o ke ake - kahi wai e kōkua ai i ka hoʻoheheʻe ʻana i nā momona i loko o ka meaʻai a kākou e ʻai ai - i ka gallbladder a me ka ʻōpū liʻiliʻi. No laila, ke pau ka hana pono ʻana o kēia mau ʻauwai bile, ua paʻa ka bile i loko o ke ake. A laila hoʻomaka ka ʻeha o ke ake .

Pēlā nō, hoʻopilikia pū kēia i ka puʻuwai. E like me ka lilo ʻana o nā ʻauwai bile i haiki, hiki i nā kiwikā puʻuwai a me nā kīʻaha koko ke haiki a hiki ke hana i nā loli. Hiki i kēia ke hoʻopilikia i ke kahe koko mai ka puʻuwai a i ke koena o ke kino, a ʻaʻole paha e hāʻawi pono ʻia ka oxygen.

He aha nā hōʻailona o ka Alagille Syndrome?

ʻIke pinepine ʻia kēia mau hōʻailona i ka wā kamaliʻi a i ʻole ka wā kamaliʻi . Eia naʻe, ʻaʻole paha lākou e ʻike ʻia a hiki i ka wā makua. Hiki ke ʻokoʻa ke koʻikoʻi o nā hōʻailona i loko o ka ʻohana hoʻokahi.

Nā hōʻailona e pili ana i ke akepaʻa

Ua haʻi ʻia mai iā mākou e hiki i ka Alagille Syndrome ke hōʻino i ke akepaʻa. Ke hōʻino ʻia ke akepaʻa, e ʻike paha ʻoe i nā hōʻailona e like me kēia:

• Melemele o ka ʻili a me nā maka - kapa mākou i kēia he jaundice (a i ʻole icterus).
• ʻAneʻane nui ka ʻili.
• Nā waihona momona melemele (e like me nā puʻupuʻu) ma ka ʻili (xanthomas).
• Mimi ʻeleʻele.

Hiki mai kēia hōʻino ʻana o ke akepaʻa ma muli o ka nele, ka haiki, a i ʻole ke ʻano hewa o nā ʻauwai bile. Lawe kēia mau ʻauwai bile i ka bile, kahi wai e kōkua ai i ka hoʻoheheʻe ʻana i nā momona, mai ke akepaʻa a i ka gallbladder a me ka ʻōpū liʻiliʻi. Ke hana pono ʻole kēia mau ʻauwai, hōʻiliʻili ka bile i loko o ke akepaʻa. ʻAʻole hiki i ke akepaʻa ke hana pono i kāna hana, ʻo ia hoʻi ka wehe ʻana i nā huahana ʻōpala mai ke koko.

No ka mea, ʻaʻole hiki i ke kino ke omo pono i nā momona a me kekahi mau huaora (ʻoi aku hoʻi ʻo A, D, E, a me K), hiki ke ʻike ʻia nā hōʻailona hou aʻe:

• Ka nāwaliwali o ka iwi.
• Hoʻemi ʻia ka ulu ʻana, ʻaʻohe piʻi ʻana o ke kiʻekiʻe.
• Nā pilikia ʻike (e pili ana i ka cornea a me ka retina).
• Nā pilikia me ke kaulike o ke kino a me nā neʻe ʻana.
• Ke ʻano maʻamau i ke koko.
• Nā lohi o ka hoʻomohala ʻana.
• ʻO ka ʻeha o ke akepaʻa (cirrhosis).

Ma kahi o 15% o ka poʻe me ka Alagille Syndrome e pilikia ana i ka loaʻa ʻana o nā maʻi hoʻoweliweli i ke ola e like me ka maʻi akepaʻa koʻikoʻi a me ka hāʻule ʻana o ke akepaʻa.

Nā hōʻailona e pili ana i ka puʻuwai

Hiki i ka Alagille Syndrome ke hoʻopilikia i ka puʻuwai a me kāna hana. ʻO ke ʻano kēia:

• ʻO ka stenosis o ke aʻa pulmonary ka haiki ʻana o ke kīʻaha koko e lawe ana i ke koko mai ka puʻuwai a i nā māmā.
• Nā loli i ke ʻano o ka puʻuwai. No ka laʻana, he lua ma waena o nā keʻena haʻahaʻa ʻelua o ka puʻuwai (ventricular septal defect), a i ʻole he ʻūmiʻi o ka ʻōmole e lawe ana i ke koko i nā māmā, he lua ma waena o nā keʻena o ka puʻuwai, kahi loli i ke kūlana o ke kīʻaha koko nui (aorta), a me kahi ventricle ʻākau i hoʻonui ʻia (tetralogy o Fallot).
• Nā leo puʻuwai ʻē aʻe (nā murmurs puʻuwai).
• ʻO ke kala polū o ka ʻili (cyanosis) ma muli o ka haʻahaʻa o ka oxygen i loko o ke koko.

Nā hiʻohiʻona i ʻike ʻia ma ka helehelena a me ke kino

He nui nā hiʻohiʻona helehelena o nā keiki i hānau ʻia me ka Alagille Syndrome:

• He ākea ka mamao ma waena o nā maka, ua hohonu nā maka.
• ʻAuwae kiko, ʻike nui ʻia.
• Lae nui.

ʻO nā hōʻailona ʻē aʻe e ʻike ʻia ma ke kino:

• Nā ʻano ʻino o ka iwi. Eia kekahi laʻana, nā iwi kuamoʻo o ka pulelehua.
• Ka pilikia o ke kahe ʻana o ke koko a me ka hahau ʻana i loko o ka lolo ma muli o nā ʻano ʻē i loko o nā kīʻaha koko i loko o ka lolo.
• Ka hāiki mālie ʻana o kahi aʻa nui i loko o ka lolo (Moyamoya syndrome).
• Nā pilikia o nā puʻupaʻa (ka liʻiliʻi ʻana, nā cysts, ka hana palena).
• ʻAʻole hiki i ka pancreas ke hoʻokaʻawale pono a hoʻopili i nā meaʻai mai ka meaʻai.

Ke hoʻopilikia nei anei ka Alagille Syndrome i nā mākau noʻonoʻo?

Ma kahi o 2% o nā keiki me ka Alagille Syndrome he kīnā noʻonoʻo ko lākou . Eia nō naʻe, hiki i kekahi mau keiki (ma kahi o 16%) ke lohi iki i ka hoʻomohala ʻana i nā pae holomua o ka neʻe ʻana, e like me ka hele wāwae. Eia naʻe, ʻaʻole kēia he kīnā noʻonoʻo.

He aha ke kumu o ka Alagille Syndrome?

I ka hapanui o nā hihia, ʻoi aku ma mua o 90% o nā hihia o ka Alagille Syndrome i hoʻokumu ʻia e kahi pilikia me ka gene JAG1. Hiki i kēia ke lilo i mutation i loko o ka gene a i ʻole kahi holoi ʻia. ʻO kekahi 2% a 3% he mutation i loko o ka gene NOTCH2. Eia nō naʻe, no kahi o 3% o nā hihia, ʻaʻole ʻike ʻia ke kumu genetic pololei.

ʻO kēia mau genes, i kapa ʻia ʻo JAG1 a me NOTCH2, ke kuhikuhi nei i kā mākou mau cell e hana i nā protein e pono ai ke kūkulu ʻana i nā ʻāpana like ʻole o ke kino i ka wā embryonic. Inā he mutation i loko o kēia mau genes ʻelua, a inā he nalowale o nā mea genetic ma ka ʻāpana o ka chromosome 20 kahi i loaʻa ai ka gene JAG1, ʻaʻole loaʻa pono i nā cell kēlā mau kuhikuhi. ʻO kēia ka manawa e ʻike ʻia ai nā hōʻailona e like me nā kīnā kūkulu i loko o ka puʻuwai, ka haiki ʻana o nā ʻauwai bile, a me nā ʻano ʻē o ka iwi.

Pehea e ʻike ʻia ai ka maʻi ʻAlagille?

Hiki ke paʻakikī ke ʻike i ka Alagille Syndrome no ka mea ʻokoʻa nā hōʻailona mai kēlā kanaka a kēia kanaka. Hoʻomaka ka ʻike ʻana me ka mōʻaukala olakino piha a me ka nānā pono ʻana i kāu mau hōʻailona. Hiki i ke kauka ke kānalua i ka Alagille Syndrome inā loaʻa iā ʻoe ma ka liʻiliʻi he ʻekolu o kēia mau hōʻailona:

• Nā ʻano ʻē o nā ʻāpana bile.
• Maʻi akepaʻa a i ʻole ke ālai ʻana o ke kahe ʻana o ka bile (cholestasis).
• Maʻi puʻuwai.
• Nā ʻano ʻino o ka iwi.
• Nā pilikia ʻike.
• Nā hiʻohiʻona helehelena ʻokoʻa.

Hana ʻia kekahi mau hoʻokolohua e hōʻoia i ka maʻi:

• Ke lawe ʻana i kahi ʻāpana liʻiliʻi o ke akepaʻa no ka hoʻokolokolo ʻana (biopsy akepaʻa).
• Nā hoʻāʻo koko.
• He hoʻokolokolo maka.
• He kiʻi X-ray o ke kuamoʻo.
• ʻO ka ultrasound o ka ʻōpū a me/a i ʻole ka puʻuwai.
• He hoʻāʻo hoʻoilina.
• Nā hoʻāʻo hana puʻupaʻa.
• Nā hoʻāʻo hana pancreas.

Pehea e ʻike ai inā loaʻa i kaʻu pēpē ʻo Alagille Syndrome a i ʻole Biliary Atresia?

Loaʻa i ka Alagille Syndrome a me Biliary Atresia nā hōʻailona like. I ka Alagille Syndrome, ua kaupalena ʻia ke kahe ʻana o ka bile ma o nā ʻauwai bile i loko o ka ʻōpū liʻiliʻi, a hōʻiliʻili ka bile i loko o ke ake. Hoʻokumu ʻia ʻo Biliary Atresia e ka hōʻino ʻia a i ʻole ka ʻeha o nā ʻauwai bile, hiki ke hana i nā hōʻailona like. Hiki i nā pēpē hānau hou ke ʻike i nā hōʻailona like ma nā kūlana ʻelua:

• Mimi ʻeleʻele.
• Nā noho waihoʻoluʻu māmā.
• Pehu ʻōpū.
• Hoʻomau ka jaundice no kekahi mau pule ma hope o ka hānau ʻana.

Hiki i nā pēpē me ka biliary atresia ke loaʻa i nā kīnā hānau ʻē aʻe, e like me ka Alagille Syndrome, a me nā ʻano ʻē o ka puʻuwai, nā puʻupaʻa, a me ka spleen. Ua hōʻike kekahi mau noiʻi ʻo ka gene like i kapa ʻia ʻo JAG1 e hoʻoulu ai i ka Alagille Syndrome e pili ana paha i ka biliary atresia.

No ka mea, ʻoi aku ka maʻamau o ka biliary atresia ma mua o ka Alagille syndrome, a no ka mea, ʻaʻole ʻike ʻia nā hopena āpau o ka Alagille syndrome i ka wā kamaliʻi, hiki i nā kauka ke manaʻo mua i ka biliary atresia. Eia nō naʻe, ua like like ka mālama ʻana no nā kūlana ʻelua . Inā loaʻa i kāu keiki ma hope nā hōʻailona ʻē aʻe o ka Alagille syndrome, hiki ke ʻike ʻia i kēlā manawa.

Pehea e mālama ʻia ai ka Alagille Syndrome?

ʻAʻohe lāʻau lapaʻau kūikawā no kēia maʻi i kēia manawa.No laila, ʻo ka manaʻo o ka lāʻau lapaʻau ke kāohi ʻana i nā hōʻailona e kū mai ana. ʻO ia hoʻi, ua hoʻoholo ʻia ka lāʻau lapaʻau e nā hōʻailona. Hiki ke hana ʻia kēia mau mea ma ke ʻano he lāʻau lapaʻau:

• Ka hāʻawi ʻana i nā wikamina A, D, E a me K.
• ʻO ka hāʻawi ʻana i nā pēpē i ka waiū waiū i loaʻa nā "medium-chain triglycerides" hiki ke omo maikaʻi i nā meaola.
• Ka hānai ʻana ma o kahi ʻōmole gastrostomy a i ʻole kahi ʻōmole nasogastric e hāʻawi pololei i ka meaʻai i loko o ka ʻōnaehana hoʻoheheʻe.
• Ke hoʻolako nei i nā lāʻau lapaʻau (ursodeoxycholic acid) e mālama ai i nā maʻi akepaʻa.
• Ka hoʻohana ʻana i nā lāʻau lapaʻau e mālama ai i ka maneʻo (e like me antihistamines, cholestyramine, naltrexone, rifampin) a me nā moisturizers a i ʻole lotions e hoʻomaʻū i ka ʻili.
• ʻO ka hoʻohuli ʻana o ka bile hapa kahi hana ʻoki e hoʻololi i ke ala o ka bile ma waena o ke akepaʻa a me ke ala ʻōpū.
• ʻO ke ʻoki kino no nā kūlana e pili ana i ka puʻuwai, nā kīʻaha koko, a me nā puʻupaʻa.

Inā hoʻoulu ka Alagille Syndrome i ka hōʻino nui ʻana o ke akepaʻa a me ka hāʻule ʻana o ke akepaʻa, pono paha kahi hoʻoili ake.

Pehea wau e noho ai a mālama i kaʻu mau hōʻailona?

Ma muli o ke ʻano ʻokoʻa o nā hōʻailona no kēlā me kēia kanaka me kēia maʻi, na kāu kauka e hoʻoholo i ka lāʻau kūpono iā ʻoe. He mea nui e loaʻa nā hoʻokolohua maʻamau e nānā i ke olakino o kou puʻuwai a me kou akepaʻa. Inā hoʻomaka kāu kauka iā ʻoe i kahi lāʻau hou, pono ʻoe e ʻike hou i kāu kauka i loko o kekahi mau pule e ʻike i ke ʻano o ka hana a me nā hopena ʻaoʻao. Hana ka hapa nui o nā lāʻau lapaʻau ma ka hōʻemi ʻana i nā hōʻailona, ​​​​e kōkua iā ʻoe e ʻoluʻolu, a me ka pale ʻana i nā pilikia koʻikoʻi e hoʻoweliweli i ke ola.

Hiki ke pale ʻia ka Alagille Syndrome?

ʻOiai he kūlana kēia i hoʻokumu ʻia e nā loli genetic, ʻaʻohe ala e pale aku ai . Inā loaʻa i kekahi o kou ʻohana ka Alagille Syndrome, a inā paha ʻoe e hāpai ana i kahi keiki a makemake ʻoe e ʻike i kou pilikia o ka hoʻoili ʻana i ke kūlana genetic i kāu keiki, e kamaʻilio me kāu kauka e pili ana i ka hoʻāʻo genetic.

He aha ke ʻano o ka wā e hiki mai ana e hiki i kekahi me ka Alagille Syndrome ke manaʻo?

ʻAʻohe lāʻau lapaʻau kūikawā no ka Alagille Syndrome, no ka mea, he maʻi ia no ke ola holoʻokoʻa . ʻO ke kumu o ka lāʻau lapaʻau ʻo ia ke kāohi ʻana i nā hōʻailona, ​​​​​​e hāʻawi ana i ka hōʻoluʻolu, a me ka pale ʻana i nā pilikia e hoʻoweliweli ana i ke ola.

He mea nui loa ka ʻike ʻana i kēia maʻi i ka wā mua a hoʻomaka i ka lāʻau lapaʻau. Hiki i kēia ke hōʻemi i nā hopena.

ʻO ka poʻe me nā maʻi akepaʻa koʻikoʻi a me nā maʻi puʻuwai, he pōkole paha ko lākou ola. Eia nō naʻe, i kēia manawa, he nui nā lāʻau lapaʻau e hiki ke kōkua i ka hoʻohuli ʻana i kēia kūlana.

Pono nā kānaka me ka Alagille Syndrome e ʻike pinepine i kā lākou mau kauka no nā nānā mau ʻana . Hiki i kēia ke kōkua i ka hoʻoholo ʻana inā ke kumu nei ke kūlana i nā hōʻailona hoʻoweliweli i ke ola a pehea ka maikaʻi o ka lāʻau lapaʻau. Hiki i nā nānā mau ke komo pū me:

• ʻO ka hoʻokolokolo ʻana o ka puʻuwai me ka ultrasound (echocardiogram).
• ʻO ka ultrasound o ka ʻōpū (ake a me nā puʻupaʻa).
• Ka hoʻokolokolo maka makahiki.
• ʻO ke kiʻi MRI o nā kīʻaha koko o ka lolo.

He aha ke kūlana o ke ola o kekahi kanaka me ka Alagille Syndrome?

Hiki i ka hapa nui o ka poʻe me nā hōʻailona iki o ka Alagille Syndrome ke ola i kahi ola maʻamau . Eia nō naʻe, ʻo ka poʻe me nā hōʻailona koʻikoʻi e emi paha ka manaʻolana ola.

E kamaʻilio me kāu kauka e pili ana i kāu mau hōʻailona. E kauoha pinepine ʻo ia i nā hoʻokolohua e nānā i ke ʻano o ka hana ʻana o kou mau kino i loko. ʻO ka pahuhopu nui o ka mālama ʻana, ʻo ia ka pale ʻana i nā hōʻailona e hoʻoweliweli ana i ke ola.

Āhea wau e ʻike ai i kaʻu kauka?

Inā ua ʻike ʻia ʻoe me ka Alagille Syndrome a ʻike ʻoe i kekahi o kēia mau hōʻailona o ka hōʻino ʻana o ke akepaʻa, e ʻike koke i ke kauka:

• Inā melemele kou ʻili a/a i ʻole kou mau maka.
• Inā ʻānai nui ka ʻili no ke kumu ʻole.
• Inā ʻike ʻia nā waihona momona (nā puʻupuʻu melemele) ma ka ʻili o ka ʻili.
• Inā ʻoi aku ka ʻeleʻele o kou mimi ma mua o ka maʻamau.

Inā ʻoe e ʻike nei i kekahi mau hōʻailona o ka Alagille Syndrome e paʻakikī ai iā ʻoe ke hoʻokō i kāu mau hana o kēlā me kēia lā, e ʻike koke i kāu kauka. He mea nui hoʻi e hoʻomaopopo i kāu kauka inā hōʻike kāu keiki me Alagille Syndrome i kekahi mau hanana ulu i ka wā kamaliʻi a i ʻole ka wā kamaliʻi.

Āhea wau e hele ai i ka Emergency Treatment Unit (ETU) ?

Hiki i ka Alagille Syndrome ke hoʻoulu i nā hōʻailona puʻuwai e hoʻoweliweli i ke ola . Inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona, ​​​​e hele koke i ka lumi pilikia:

• Inā kū ʻole ka puʻuwai o kou naʻau.
• Inā pilikia ʻoe i ka hanu ʻana.
• Inā ʻike ʻia kou ʻili, lehelehe, a i ʻole nā ​​​​kui he polū.

Aia kekahi mau hihia o ka Alagille Syndrome i ka nui o ka pilikia o ka hahau ʻana. Inā ʻike ʻoe i kekahi o kēia mau hōʻailona o ka hahau ʻana, e kelepona koke i ka 911 (a i ʻole e hele i kou lumi pilikia kokoke loa):

• Inā ʻoe e manaʻo ana he ʻano maeʻa ma kekahi ʻaoʻao o kou kino.
• Inā pilikia ʻoe i ka ʻōlelo ʻana, a inā paha ua paʻa kāu mau ʻōlelo.
• Inā he loli koke o ka ʻike maka.
• Pōʻino, nalowale ke kaulike, nā pilikia hoʻonohonoho.
• He ʻeha poʻo weliweli.

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

Ke ʻike ʻoe i kāu kauka, hiki iā ʻoe ke nīnau i nā nīnau e like me kēia:

• Pehea ka koʻikoʻi o koʻu mau hōʻailona?
• E pono anei iaʻu ke ʻoki kino?
• Āhea wau e hoʻi mai ai e ʻike i ka holomua o ka lāʻau lapaʻau?
• He aha nā hopena ʻaoʻao o nā lāʻau lapaʻau āu i kuhikuhi ai?

E hoʻomanaʻo, ʻaʻole like ka hopena o ka Alagille Syndrome i nā kānaka a pau. Loaʻa paha i kekahi poʻe nā hōʻailona liʻiliʻi loa, aʻo kekahi poʻe he mau hōʻailona koʻikoʻi e pono ai ka mālama ʻana a i ʻole ke ʻoki ʻana. No laila e hana pū me kāu kauka e loaʻa ai ka mālama āu e pono ai. He mea nui e hoʻomau i kāu mau hoʻokolohua maʻamau e hoʻokele i nā hopena ʻaoʻao a me nā hōʻailona e hoʻoweliweli ana i ke ola.

Leka Lawe-Home

He maʻi hoʻoilina ka Alagille Syndrome. Hoʻopilikia nui ia i ke akepaʻa a me ka puʻuwai. Hiki ke ʻokoʻa nā hōʻailona mai kēlā kanaka a kēia kanaka. ʻOiai ʻaʻohe lāʻau lapaʻau, aia nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona a pale i nā pilikia e hoʻoweliweli ana i ke ola. He mea nui ka ʻike mua a me ka mālama ʻana, a pono ʻoe e hahai i nā ʻōlelo aʻo a kāu kauka. Inā loaʻa iā ʻoe a i ʻole kāu keiki kekahi o kēia mau hōʻailona, ​​​​mai makaʻu e ʻike i ke kauka a loaʻa ke kōkua. E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi, a aia nā kauka a me nā mea aloha e hiki ke kōkua iā ʻoe ma kēia huakaʻi.

ʻO ka Alagille Syndrome, nā maʻi genetic, nā maʻi ate, nā maʻi puʻuwai, nā maʻi pediatric, jaundice