He mau kiko ʻulaʻula nui ko kāu keiki liʻiliʻi ma kona kino? E aʻo kākou e pili ana i ka PHACE Syndrome.

E nā mākuahine a me nā mākua kāne, e hopohopo paha ʻoukou a makaʻu hoʻi ke ʻike ʻoukou i nā kiko ʻulaʻula nui a i ʻole nā ​​puʻupuʻu liʻiliʻi ma ke kino o kāu keiki liʻiliʻi, i kekahi manawa ma ka maka a me ka ʻāʻī. I kekahi manawa he mau mea wale nō kēia e ho'ōla ʻia, akā i kekahi manawa, hiki iā lākou ke lilo i hōʻailona o kahi maʻi kakaikahi a paʻakikī hoʻi e like me ka PHACE Syndrome . No laila, me ka ʻole o ka hoʻopaneʻe hou ʻana, e kamaʻilio kikoʻī kākou e pili ana i ke ʻano o ka PHACE Syndrome, nā mea e hana ai, a me nā mea a mākou e hana ai inā he mea like ia.

He aha ka maʻi PHACE?

ʻAe, e nānā mua kākou i ke ʻano o ka maʻi PHACE. I ka ʻōlelo maʻalahi, he maʻi kakaikahi loa ia. ʻO ia hoʻi, ʻaʻole ia e hiki i nā kānaka a pau. He maʻi hānau ia i loaʻa i ka wā hānau. ʻO ia hoʻi, hiki mai ia i ka wā e noho ana ke pēpē i loko o ka ʻōpū. Hiki i kēia maʻi ke hoʻopilikia i nā ʻāpana like ʻole o ke kino o ke pēpē. Hoʻopilikia nui ia i ka ʻili, ka lolo, ka puʻuwai, nā aʻa nui, a me nā maka .

ʻO ka hapa nui o ka manawa, he kakaikahi wale nō nā hemahema ma kēlā me kēia wahi. Hiki i kekahi mau keiki ke hōʻike wale i kekahi mau hōʻailona i helu ʻia ma lalo nei. ʻO nā kūlana maʻamau i ʻike ʻia i nā keiki me ka maʻi PHACE he hemangiomas , nā hemahema cerebrovascular , a me nā hemahema puʻuwai . Hiki ke ʻike ʻia kēia mau mea i ka wā hāpai, i ka wā hānau, a i ʻole i ka wā kamaliʻi.

ʻO PHACE kahi acronym no kekahi mau maʻi like ʻole. Hōʻike ia i nā ʻano ulu nui e pili ana i kēia syndrome. I kekahi manawa ua kapa ʻia ʻo ia ʻo PHACES syndrome. ʻO ke ʻano o ka 'S' keu no ka sternal clefting . ʻO ia hoʻi, ʻaʻole i hana pono ʻia ka iwi ma waenakonu o ka umauma, ʻo ka sternum, a i ʻole he kīnā i loko.

He aha nā hōʻailona o ka maʻi PHACE?

No laila, he aha nā hōʻailona o kahi keiki me ka maʻi PHACE? Hiki ke ʻokoʻa kēia mai kekahi keiki a i kekahi keiki. ʻOi aku ka liʻiliʻi o nā hōʻailona o kekahi mau keiki, ʻoi aku ka nui o kekahi. A ʻokoʻa hoʻi ke koʻikoʻi o nā hōʻailona mai kekahi keiki a i kekahi. E nānā kākou i nā hōʻailona nui:

• Hemangiomas: He mau puʻupuʻu ʻike ʻia kēia, ʻeleʻele ma ka ʻili. Hoʻokumu ʻia lākou e ka ulu ʻana o nā kīʻaha koko keu . ʻIke pinepine ʻia lākou ma ke poʻo, ka maka, ka ʻāʻī, ke kino, a me nā lima. He nui loa lākou a hiki ke pālahalaha. Eia nō naʻe, i kekahi manawa hiki i kēia mau hemangiomas ke hoʻopilikia i nā ʻōpū o loko o ke keiki. Inā hana ʻia kēia, hiki ke loaʻa nā pilikia me ka ʻike a me ka lohe.

• Nā ʻano ʻē o ka lolo:ʻO ka posterior fossa kahi o ka lolo e kaohi ana i nā hana koʻikoʻi e like me ke kaulike, ka hoʻonohonoho ʻana, a me ka hanu ʻana. Inā he ʻano ʻē ma kēia wahi, hiki i ke keiki ke loaʻa nā neʻe ʻana me ka makemake ʻole a me nā kīnā noʻonoʻo . Hiki nō hoʻi ke loaʻa nā loli i ke ʻano o ka lolo ponoʻī.

• Nā ʻano ʻē o ka puʻuwai: Hiki i kekahi mau kīnā o ka puʻuwai ke hana i nā hōʻailona e like me ka pōkole o ka hanu , ka nāwaliwali o nā ʻiʻo , ke koko kiʻekiʻe , ka hopohopo pinepine, a me ka hou ʻana. I kekahi manawa, ʻaʻole i hoʻomohala piha ʻia ka puʻuwai o kahi pēpē, a aia paha he lua ma waena o nā keʻena o ka puʻuwai (ventricular septal defect) . A i ʻole, aia paha he haiki o ka aorta (coarctation o ka aorta).

• Nā ʻano ʻē o ke aʻa: Inā ua haiki nā aʻa e lawe ana i ke koko i ka lolo a me ka ʻāʻī a i ʻole he mau ʻano ʻē aʻe, ʻaʻole paha e lawa ke koko i ka lolo. Hiki i kēia ke alakaʻi i nā kūlana weliweli e like me nā aneurysms , nā hopu ʻana , nā hahau ʻana , a i ʻole ka nalowale o ka ʻike ma kekahi ʻaoʻao o ke kino.

• Nā ʻano ʻē o ka maka: ʻAʻole paha i ulu pono nā maka o ka pēpē, nā aʻalolo optic, a i ʻole nā ​​​​kīʻaha koko i loko o nā maka. Hiki i kēia ke hana i ka ʻike maikaʻi ʻole, ka nalowale ʻana o ka ʻike , nā maka liʻiliʻi maʻamau , nā neʻe ʻana o ka maka i kāohi ʻole ʻia, a i ʻole nā ​​​​maka e kūlou ana .

• Nā ʻano ʻē o ka iwi umauma: E like me ka mea i ʻōlelo ʻia ma mua, ʻo ka iwi umauma (sternum) ma waenakonu o ka umauma o ka pēpē e nalowale ana paha a ʻaʻole paha e ulu pono. Hiki i kēia ke hana i nā māwae, nā puʻupuʻu, a me nā lua e ʻike ʻia ma ia wahi.

Nā pilikia kūpono o ka maʻi PHACE

ʻOi aku ka nui o ka hiki i nā keiki me ka maʻi PHACE ke loaʻa i kekahi mau pilikia ma muli o kēia mau ʻano ʻē i ko lākou ʻano kino a me nā kīʻaha koko. Eia lākou:

• Nā pilikia kaulike
• ʻO nā pilikia niho, no ka laʻana , ka hypoplasia enamel a me nā ʻano ʻino o ke aʻa niho.
• Paʻakikī ke ale ʻana
• Nā ʻano ʻino o ka ʻōnaehana endocrine . Eia kekahi laʻana, ua emi ka hana thyroid a ua emi ka hana ʻana o ka hormone.
• ʻO ka ʻeha poʻo pinepine, i kekahi manawa migraines .
• ulu maikaʻi ʻole
• Nā lohi o ka ʻōlelo a me ka ʻōlelo
• Hoʻonui ʻia ka pilikia o ka hahau ʻana .
• Nā ʻōpū hānau i hoʻomohala ʻole ʻia

He aha ke kumu o ka maʻi PHACE?

ʻAʻole maopopo i nā kānaka noiʻi ke kumu e loaʻa ai i kekahi mau pēpē ka maʻi PHACE. Hōʻike nā haʻawina he kumu paha ia e nā kumu genetic a me ke kaiapuni. Eia nō naʻe, ʻaʻole maopopo loa ka ʻokoʻa genetic e hoʻokumu ai. Manaʻo kekahi poʻe loea ua hoʻokumu ʻia ia e kahi loli gene random (de novo) e hana ʻia i ka wā o ka hāpai ʻana. ʻO ke ʻano kēia, ʻaʻole ia i hoʻoilina pinepine ʻia mai nā mākua.

Pehea e ʻike ʻia ai ka maʻi PHACE?

ʻAe, pehea lā e ʻike ai nā kauka i ka maʻi PHACE? ʻO ka maʻamau, e hana ke kauka i kahi hoʻokolokolo kino piha a hana i kekahi mau hoʻāʻo . Loaʻa iā lākou nā pae hoʻohālikelike kikoʻī, ʻo ia hoʻi nā alakaʻi. Pēlā lākou e hoʻoholo ai inā loaʻa i kahi keiki ka maʻi PHACE.

I kekahi manawa, hiki i nā kauka ke loaʻa kahi manaʻo o ke kūlana mai nā scan āu i loaʻa ai i kou wā hāpai. Akā i nā manawa ʻē aʻe, ʻaʻole paha e maopopo a hiki i ka hānau ʻana o ka pēpē, a ma hope paha o ka wā kamaliʻi.

I ka manawa pinepine, e nānā mua ke kauka i kahi hemangioma nui ma ke kino o ke keiki, ʻoi aku hoʻi ma ka maka, ka ʻāʻī, a i ʻole ke poʻo. ʻO ke alo o hoʻokahi hemangioma a me ka liʻiliʻi o hoʻokahi hiʻohiʻona koʻikoʻi e pili ana i ka PHACE e lawa paha no ka hōʻoia ʻana.

Eia kekahi, hiki ke hana ʻia kekahi mau hoʻokolohua e nānā pono aku i ka lolo, ka puʻuwai, nā aʻa koko, a me nā maka o ka pēpē. Eia nā hoʻokolohua i hana pinepine ʻia:

• Nā scan CT
• Echocardiograms - Nānā kēia i ke ʻano a me ka hana o ka puʻuwai.
• Nā hoʻokolohua maka - e ke kauka maka.
• Nā hoʻokolohua hoʻolohe
• MRI scan - E kiʻi i nā kiʻi kikoʻī, ʻoiai hoʻi o ka lolo a me nā kīʻaha koko.
• ʻO ke kiʻi ʻana o ka ultrasound

Pehea e mālama ʻia ai ka maʻi PHACE?

I ka mālama ʻana i ka maʻi PHACE, ʻo ke kumu nui ka pale ʻana i nā pilikia hiki ke hiki mai a me ke kōkua ʻana i ke keiki e ola maʻamau me kā lākou mau hōʻailona. ʻOkoʻa nā ʻano lapaʻau ma muli o nā ʻāpana a i ʻole nā ​​​​​​ʻāpana o ke kino e hoʻopilikia ʻia e ke kūlana.ʻO ia hoʻi, ʻaʻole i loaʻa i kēlā me kēia keiki ka mālama like.

Hana pinepine ʻia kēia ʻano lapaʻau:

• ʻO ka lāʻau lapaʻau laser no nā hemangiomas ma ka ʻili, a i ʻole ka hāʻawi ʻana iā lākou i nā lāʻau lapaʻau (e like me nā beta-blockers e like me propranolol) e hoʻēmi iā lākou.
• Ke hāʻawi nei i ka hoʻonaʻauao kūikawā , ka hoʻoponopono ʻōlelo, a me ka physiotherapy e kōkua i ke aʻo ʻana ma ke kula.
• I kekahi manawa pono ke ʻoki ʻana e hoʻoponopono i nā kīnā i ka puʻuwai, nā kīʻaha koko, a i ʻole ka lolo.
• Ke lawelawe ʻana i nā lāʻau lapaʻau like ʻole e kāohi i ka maʻi epilepsy, nā poʻo hina, a me nā hormonal imbalances.
• Ke hoʻolako nei i nā mea kōkua no ka nalowale o ka lohe a me nā aniani no ka nalowale o ka ʻike.

He nui nā ʻohana e kamaʻilio me kahi kākāʻōlelo genetic e aʻo hou e pili ana i nā koho lapaʻau no kā lākou keiki, a me pehea e kōkua ai iā lākou i ko lākou ulu ʻana. Hiki i kēia ke kōkua nui.

Hiki ke pale ʻia kēia?

ʻO ka mea pōʻino, ʻaʻohe ala e pale aku ai i ka maʻi PHACE. No ka mea, ʻaʻole ʻike ʻia ke kumu pololei, ʻaʻohe mea hiki iā ʻoe ke hana e hōʻemi i ka pilikia o ka loaʻa ʻana o kahi keiki me kēia maʻi.

Akā inā makemake ʻoe e ʻike hou aʻe e pili ana i kou loaʻa ʻana o kahi keiki me kahi maʻi genetic, hiki iā ʻoe ke kamaʻilio me kāu kauka e pili ana i ka loaʻa ʻana o ka hoʻāʻo genetic .

He aha ka mea e hana ai inā loaʻa i kaʻu keiki ka maʻi PHACE? He aha ka mea e manaʻo ai?

Inā loaʻa i kāu keiki kēia maʻi, pono lākou e nānā mau me kā lākou kauka mālama mua a me nā loea i nā ʻano like ʻole (e.g., cardiologist, neurologist, ophthalmologist) e hōʻoia ʻaʻole e hoʻopilikia kā lākou mau hōʻailona i ko lākou ola maikaʻi. He ʻano kēia e pono ai ka mālama lōʻihi.

ʻAʻole hiki i kēia maʻi ke hoʻopilikia wale i ke keiki, akā, iā ʻoe a me ke koena o ka ʻohana. No laila, he mea nui loa ia e noʻonoʻo e pili ana i kou olakino noʻonoʻo. Loaʻa i kekahi poʻe ka hōʻoluʻolu nui a me ka ikaika mai ke kamaʻilio ʻana me kahi loea olakino noʻonoʻo .

No laila he aha ke ola o kekahi me kēia maʻi? Aia maoli ia i ke koʻikoʻi o nā hōʻailona o ke keiki a me nā ʻōpū i hoʻopilikia ʻia e ka maʻi.

ʻOi aku ka pōkole o ke ola o nā keiki me ka hōʻino nui o ka lolo a me ka puʻuwai. Eia nō naʻe, inā he mau hōʻailona iki ko ke keiki a mālama pono ʻia, hiki iā lākou ke ola i kahi ola maʻamau.

Āhea ʻoe e ʻike ai i ke kauka?

Inā loaʻa i kāu keiki kekahi o kēia mau mea, e ʻike pono i ke kauka:

• Inā pilikia ʻoe i ka ʻai ʻana a i ʻole ka inu ʻana
• Inā ʻaʻole ke keiki e hoʻokō i nā pae hoʻomohala kūpono no ko lākou mau makahiki, ʻo ia hoʻi, inā e ulu lohi ana lākou (e.g., ʻaʻole e paʻa i ko lākou ʻāʻī i ka manawa kūpono, ʻaʻole hele wāwae, ʻaʻole kamaʻilio).
• Inā ʻaʻole ʻoe e pane i nā mea me ka ʻike maka, ʻo ia hoʻi, inā ʻike ʻoe i kahi mea ʻē i kou mau maka.
• Inā ʻike ʻoe i kahi kiko nui, pālahalaha, ʻulaʻula ma kou kino (e like me ka hemangioma).

Āhea ʻoe e lawe koke ai i kāu keiki i ka haukapila?

Inā ʻike ʻoe i kahi mea e like me kēia, e lawe koke i kāu keiki i ka lumi pilikia kokoke loa, a i ʻole e kelepona iā 1990:

• Inā ʻoe e hōʻike ana i nā hōʻailona o ka hahau ʻana (e like me ke kūlou koke ʻana o kekahi ʻaoʻao o ka maka, ka nalowale ʻana o ka hana lima, ka hiki ʻole ke ʻōlelo).
• Inā loaʻa iā ʻoe kahi ʻōʻō.
• Inā pilikia ʻoe i ka hanu ʻana, inā manaʻo ʻoe e like me ʻoe e ʻūlū ana.
• Inā ʻaʻole kūpono ka puʻuwai o ka naʻau, a inā polū paha ka pēpē.

Nā nīnau e nīnau aku ai i ke kauka/mrs.

Hiki iā ʻoe ke nīnau i ke kauka a i ʻole ke kahu maʻi o kāu keiki e like me kēia. E kōkua kēia iā ʻoe e hoʻomaopopo i ke kūlana:

• Loaʻa maoli anei i kaʻu keiki ka maʻi PHACE? Pono anei au e hana i kekahi mau hoʻokolohua hou aʻe e hōʻoia i ia mea?
• Pono anei kaʻu keiki i ke ʻoki kino ? Inā pēlā, no ke aha a i ka manawa hea?
• Aia kekahi lāʻau e hoʻopau ai i ka hemangioma o kaʻu keiki? E ho'ōla loa ʻia anei?
• Aia kekahi mau hopena ʻaoʻao i kēia mau lāʻau lapaʻau? He aha lākou?
• He aha nā mea e pono ai kākou e makaʻala nui ai no ke keiki?
• Pehea lā ke ola o kaʻu keiki?
• E hiki anei i ke keiki ke ola i ke ola maʻamau me kēia maʻi?

ʻO ka mea hope loa, he mea e hoʻomanaʻo ai

Ke ʻike ʻoe he maʻi laha ʻole kāu keiki a ʻaʻole ʻoe i ʻike nui e pili ana i ia mea, hiki iā ʻoe ke manaʻo i ka haʻalulu nui, ke kaumaha, a me ka makaʻu. He mea paʻakikī loa ke manaʻoʻiʻo. Hiki i kēia ke loaʻa ka hopena nui o ka naʻau iā ʻoe a me kou ʻohana.

Ola maikaʻi kekahi mau keiki a hiki i ka wā makua me kēia maʻi. Eia nō naʻe, no kekahi poʻe, hiki ke hoʻopōkole ʻia ke ola ma muli o nā hōʻailona koʻikoʻi. ʻO ke komo ʻana i kahi hui kākoʻo, a i ʻole ke kamaʻilio ʻana me kahi kākāʻōlelo genetic a i ʻole ka loea olakino noʻonoʻo , hiki ke lilo i kumu hōʻoluʻolu a me ka ikaika no ʻoe a me kou ʻohana i kēia manawa. No laila, mai kānalua e kiʻi i kēlā kōkua.

E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. Aia nā kauka, nā kahu maʻi, a me nā mea ʻē aʻe he nui e hiki ke kōkua iā ʻoe e hoʻokele i kēia mau pilikia, e hoʻolohe i kāu mau nīnau, a hāʻawi iā ʻoe i ka ʻike āu e pono ai. No laila, e noho ikaika a e hoʻāʻo e hāʻawi i kāu keiki i ka mea maikaʻi loa a lākou e pono ai.

ʻO ka maʻi PHACE, hemangioma, nā maʻi hānau ʻia, nā ʻano ʻē o ka lolo, nā kīnā o ka puʻuwai, nā kīnā o ke aʻa, nā ʻano ʻē o ka maka, nā maʻi hoʻoilina, nā maʻi laha ʻole, ke olakino o ke keiki