Loaʻa i kāu pēpē kekahi o kēia mau pilikia i ka manawa like? E kamaʻilio kākou e pili ana i ka VATER Syndrome / VACTERL Association!

E hopohopo nui paha ʻoe ke haʻi aku nā kauka iā ʻoe ua loaʻa koke i kāu pēpē hou kekahi mau loli a kīnā paha ma kekahi mau ʻāpana o kona kino. Malia paha ua hāʻawi nā scan i hana ʻia ma mua o ka hānau ʻana o ke pēpē iā ʻoe i kahi kuhi e pili ana i kahi mea e like me kēia. ʻO kēia ke kūlana a mākou e kamaʻilio ai i kēia lā, ʻo VATER Syndrome, a i ʻole VACTERL Association. Mai hopohopo, e hoʻomaopopo maʻalahi kākou i kēia.

He aha ka VATER Syndrome / VACTERL Association?

I ka ʻōlelo maʻalahi, ʻo ka maʻi VATER kahi hui o nā kīnā hānau e kū mai ana i ka wā mua o ka ulu ʻana o kahi pēpē. ʻO ke kikoʻī, kū kēia mau pilikia i nā pule mua o ka ulu ʻana o ka embryo o kahi pēpē. Hiki ke ʻokoʻa ke ʻano mai kekahi pēpē a i kekahi. ʻAʻole e loaʻa i kēlā me kēia pēpē nā hōʻailona like. Manaʻo nā kauka i ke ʻano ke loaʻa kahi pilikia me ka liʻiliʻi o ʻekolu o nā ʻāpana kino i hōʻike pinepine ʻia e nā leka VATER.

He aha ke ʻano o kēia mau leka VATER / VACTERL?

He huaʻōlelo pōkole kēia. ʻO ia hoʻi, he huaʻōlelo i hoʻokumu ʻia ma ka hoʻohui ʻana i nā leka mua o nā inoa Pelekane o nā ʻāpana kino i hoʻopilikia ʻia e kēia maʻi. Ua kapa mua ʻia ʻo VATER:

• V - Vertebrae : ʻO ia hoʻi nā iwi liʻiliʻi (vertebrae) i loko o ka iwi kuamoʻo .
• A - Anus : ʻO ia hoʻi ka anus .
• T - Trachea : ʻO ia hoʻi ka ʻōpū hanu .
• E - Esophagus : ʻO ia hoʻi ka paipu meaʻai.
• R - Renal : ʻO ia hoʻi nā pilikia o ka puʻupaʻa .

Ma hope mai, i ka ʻike ʻia ʻana o nā pilikia ma nā ʻāpana kino ʻelua me kēia kūlana, ua hoʻonui iki ʻia ka acronym i VACTERL:

• C - Cardiac : ʻO ia hoʻi nā pilikia e pili ana i ka puʻuwai .
• L - Limbs : Pili kēia i nā pilikia e pili ana i nā lālā (ʻoi aku ka iwi radius ma ka ʻaoʻao o ka manamana wāwae nui).

Maopopo iā ʻoe ke ʻano o VACTERL? ʻO kēia ke kūlana i ka wā e loaʻa ai kekahi mau hemahema i kekahi mau ʻāpana i ka manawa like.

ʻO wai ka mea hiki ke hoʻopilikia ʻia e kēia kūlana VACTERL?

Hiki i ka VACTERL ke hoʻopilikia i kekahi. ʻAʻole ia he hoʻoilina. ʻO ke ʻano kēia, hoʻokahi wale nō lālā o ka ʻohana e hoʻopilikia ai. ʻOiai ʻaʻole i ʻike ʻia ke kumu pololei, ua manaʻo ʻia ua hoʻokumu ʻia e ka hui pū ʻana o nā mana genetic a me nā kumu kaiapuni i ka wā hāpai.

Ma ke ʻano o ka maʻamau o kēia maʻi, hoʻopilikia ia ma kahi o hoʻokahi i loko o 10,000 a 40,000 mau pēpē hānau hou. Eia nō naʻe, ʻoi aku paha kēia helu no ka mea he mau hōʻailona palupalu loa kekahi mau keiki, a he kakaikahi wale nō paha nā hōʻailona.

He aha nā hōʻailona o ka maʻi VACTERL?

ʻIke nui ʻia nā hōʻailona ma nā ʻāpana o ke kino i hōʻike ʻia e nā leka VACTERL a mākou i kamaʻilio ai ma mua. ʻIke ʻia kekahi o kēia mau hōʻailona i ka wā hānau (congenital) , aʻo kekahi e ʻike ʻia ma hope. Hiki ke ʻokoʻa loa kēia mau hōʻailona mai kekahi keiki a i kekahi. E nānā kākou i kēlā me kēia ʻāpana e pili ana i kēlā me kēia ʻāpana.

Nā pilikia o ka iwi kuamoʻo (Vertebrae)

He ʻāpana koʻikoʻi loa ka iwi kuamoʻo o ko kākou kino. Ma VACTERL, aia paha kekahi mau loli i ke ʻano o ka ulu ʻana o kēia iwi kuamoʻo.

• Ke kūlou ʻana o ke kuamoʻo i kekahi ʻaoʻao ( scoliosis ).
• Ka hewa o ke ʻano o nā iwi kuamoʻo (spine) (e.g., hemivertebrae , nā iwi kuamoʻo i hoʻohui ʻia ).
• Ka nalowale ʻana o ka iwi huelo.
• Nā ʻano ʻē o nā iwi ʻaoʻao (nā iwi ʻaoʻao i nalowale, loaʻa kahi iwi ʻaoʻao keu, nā iwi ʻaoʻao i paʻa pū ʻia a māhele ʻia paha).

E noʻonoʻo pehea e hoʻopilikia ai kekahi mea e like me kēia i ke kūlana a me ka neʻe ʻana o ke keiki i ka wā e pili ana i ka iwi kuamoʻo.

Nā pilikia Anal (Anus)

ʻAʻole paha i piha ka anus o kekahi mau keiki me kēia maʻi. ʻO ke ʻano kēia, he liʻiliʻi loa paha ka puka e puka mai ai ka ʻōpala a i ʻole ma kahi kūpono ʻole. Ua kapa ʻia kēia he anal atresia . He mea paʻakikī kēia no ke keiki ke neʻe i ka ʻōpū.

Nā pilikia puʻuwai

Hiki i nā pilikia puʻuwai ke koʻikoʻi loa. Ma VACTERL, hiki ke loaʻa nā pilikia like ʻole me ke ʻano o ka hana ʻana o ka puʻuwai a pehea e hana ai.

• Ua neʻe ʻia nā kīʻaha koko nui (aorta a me ke aʻa pulmonary).
• Nā lua ma waena o ka atria o ka puʻuwai ( atrial septal defects (ASD) ).
• Ka wikiwiki o ka puʻuwai ( tachycardia ).
• Ka hāʻule ʻana o ka puʻuwai .
• Ka ulu ʻole ʻana o ke keʻena puʻuwai hema ( hypoplastic left heart syndrome ).
• Ka hanu wikiwiki a me/a i ʻole ka paʻa ʻana o ka umauma.
• Nā maʻi puʻuwai paʻakikī e like me Tetralogy o Fallot .
• Nā lua ma waena o nā keʻena o ka puʻuwai ( ventricular septal defects (VSD) ).

Hiki i kekahi o kēia mau maʻi puʻuwai ke hoʻoweliweli i ke ola. Eia nō naʻe, hiki i kekahi mau lua, e like me ASD a me VSD, ke pani ma o lākou iho i ka hala ʻana o ka manawa.

Nā pilikia Trachea a me Esophagus

ʻO kekahi o nā kūlana maʻamau i ʻike ʻia ma VACTERL ʻo ia ka pili ʻole o ka trachea a me ka esophagus ma kahi e pono ai lākou (ʻO ka fistula tracheoesophageal. A i ʻole, ʻaʻole i pili pono ka esophagus i ka ʻōpū ( esophageal atresia ). ʻO kēia no ka mea:

• Paʻakikī i ka inu ʻana i ka waiū a me ka ʻai ʻana.
• Nā maʻi ʻōpū hanu (e like me ka niumonia ).
• Paʻakikī ke ale ʻana.
• Hiki ke pilikia ka hanu ʻana.

E noʻonoʻo wale, inā he kūlike ʻole ma waena o ka paipu meaʻai a me ka paipu hanu, hiki i ka meaʻai a me ka mea inu ke komo i loko o nā māmā, ʻeā? He mea weliweli kēlā.

Nā pilikia o ka puʻupaʻa

Hiki ke ʻike ʻia nā pilikia o nā puʻupaʻa a me ka ʻōnaehana mimi ma kēia ʻano.

• Kahi ʻē o ka wehe ʻana o ka urethral (ʻoi aku hoʻi i nā keikikāne - Hypospadias ).
• ʻAʻole ulu pono nā puʻupaʻa ( renal dysplasia ).
• ʻO nā puʻupaʻa ma kahi kūpono ʻole ( renal ectopia ).
• Ka mimi e kahe ana mai ka ʻōpū mimi a i nā puʻupaʻa ( vesicoureteral reflux ).
• Ka hōʻiliʻili ʻana o ka mimi i loko o nā puʻupaʻa ( Hydronephrosis ).
• Nā maʻi pinepine o ke ala mimi.

Nā pilikia o nā lālā a me ke radius

Ma VACTERL, hiki ke ʻike ʻia nā pilikia ma nā lālā, ʻoiai ma ka iwi radius ma ka ʻaoʻao manamana nui o ka lima.

• Ka nalowale piha ʻana o ka iwi radius ( radial aplasia ).
• ʻO ka hypoplasia radial ( ka hoʻomohala ʻole ʻia ʻana o ka iwi radius).
• Ka nalowale ʻana o ka manamana wāwae nui.
• Loaʻa kahi iwi keu ma ka manamana wāwae nui (Triphalangeal thumb ).

Eia kekahi, hiki ke ʻike ʻia nā pilikia ʻē aʻe ma nā lālā ʻē aʻe (me ka iwi radius):

• He ʻano ʻē o ke kuʻekuʻe wāwae ( clubfoot ).
• ʻAʻole hiki ke hoʻohana pono i kekahi mau manamana lima, ʻoiai he liʻiliʻi ( hypoplasia ).
• Loaʻa nā manamana lima keu ( polydactyly ).
• Ka hui ʻana o nā iwi ʻelua o ka lima poʻohiwi ( radioulnar synostosis ).
• Paʻa pū nā manamana lima (e like me ka membrane - syndactyly ).

ʻO nā hōʻailona ʻē aʻe e ʻike ʻia ma waho aʻe o ka inoa ʻo VACTERL

Ma waho aʻe o nā ʻāpana i haʻi ʻia me nā leka VACTERL, hiki ke ʻike ʻia kekahi mau ʻano ʻē aʻe i kēia mau keiki.

• Ke ʻano ʻē o nā pepeiao.
• ʻAsymmetry o nā ʻaoʻao ʻelua o ka maka.
• Ka paʻakikī o ka hanu ʻana ma ka ihu ( choanal atresia ).
• Ua paʻa ke kaula iwi kuamoʻo ma kahi hoʻokahi (tethered spinal cord ).
• ʻOi aku ka lohi o ka ulu ʻana ma mua o ka maʻamau.
• ʻO ka omphalocele (ka puka ʻana o nā ʻōpū ma waho o ke kino).
• Ka hāiki ʻana o ka pahu leo/ larynx .

He mea nui: ʻAʻole i loaʻa kēia mau ʻano a pau i nā keiki a pau. Loaʻa paha i kekahi mau keiki kekahi mau ʻano liʻiliʻi wale nō. Eia kekahi, ʻokoʻa paha ke ʻano like i kekahi keiki ma mua o kekahi.

He aha ke kumu o ka hoʻokumu ʻia ʻana o ka hōʻiliʻili VACTERL?

ʻO kaʻoiaʻiʻo, ʻaʻole i loaʻa ke kumu kikoʻī o ka maʻi VACTERL. Ke noiʻi ʻia nei kēia. Eia nō naʻe, ʻo ka manaʻo o kēia manawa, hiki ke hana ʻia kēia ʻano ma muli o ka hui pū ʻana o kekahi mau mea hoʻoilina a me ke kaiapuni e hoʻopilikia ana i ka ulu ʻana o ka ʻōpū i nā wā mua o ka hāpai ʻana. ʻO ia hoʻi, ʻaʻole paha he kumu hoʻokahi, akā he mau kumu i hui pū ʻia.

Pehea e ʻike ai i ka hōʻiliʻili VACTERL?

ʻIke pinepine ʻia ka maʻi VACTERL i ka wā o nā hoʻokolohua prenatal a i ʻole i ka wā ʻōpiopio o ke ola. Eia nō naʻe, i kekahi manawa ʻaʻole maopopo loa nā hōʻailona, ​​​​no laila hiki ke ʻike ʻia i ka wā kamaliʻi a i ʻole ka wā ʻōpio.

ʻO ka maʻi VACTERL kahi "diagnosis of exclusion." ʻO ke ʻano kēia, e kāpae mua nā kauka i nā maʻi a me nā kūlana ʻē aʻe e hiki ke hana i nā hōʻailona like. Ke loaʻa ʻole kekahi o kēia mau kūlana, manaʻo lākou he kumu kūpono ʻo VACTERL.

E hana mua ke kauka i kahi hoʻokolokolo kino piha. E nānā kēia i nā kīnā hānau ma ka liʻiliʻi o hoʻokahi o nā ʻāpana kino i pela ʻia me nā leka VACTERL a mākou i kūkākūkā ai ma mua. ʻO ka pinepine, e loaʻa i kahi keiki me VACTERL kēia mau pilikia ma ka liʻiliʻi o ʻekolu mau ʻāpana kino .

Ma hope o ka hoʻokolokolo kino, e kauoha ke kauka i nā hoʻokolohua hou aʻe (e like me nā X-ray , ultrasounds , nā hoʻokolohua koko). Hana ʻia kēia mau mea e ʻike i nā pilikia e hoʻoweliweli ana i ke ola, ʻoiai hoʻi nā pilikia o ka puʻuwai a me nā puʻupaʻa, i hiki ke mālama koke ʻia.

He aha nā lāʻau lapaʻau no ka maʻi VACTERL?

I ka mālama ʻana i ka maʻi VACTERL, ʻo ke kumu nui ka hōʻemi ʻana i nā hōʻailona i hoʻokumu ʻia e kēia mau malformations i loko o ke kino i ka wā embryonic. ʻOkoʻa nā ʻano lapaʻau mai kekahi keiki a i kekahi. ʻO kēia no ka mea ʻaʻole like nā pilikia o nā keiki āpau. ʻO nā lāʻau lapaʻau nui:

• ʻOki kino: Pono ke ʻoki kino e hoʻoponopono i kēia mau kīnā. I kekahi manawa, hana ʻia kēia ʻoki kino i loko o kekahi mau lā o ka hānau ʻana o ka pēpē, ma mua o ka hiki ʻana mai o nā pilikia nui. I ka ulu ʻana o ka pēpē, pono paha nā ʻoki kino hou aku.
• Hoʻōla kino a i ʻole ka hoʻōla hana: Kōkua kēia mau lāʻau i ka hoʻoikaika ʻana i nā ʻiʻo a hoʻomaikaʻi i ka neʻe ʻana o nā hono.
• Lāʻau lapaʻau: Hiki ke hāʻawi ʻia nā ʻano lāʻau like ʻole e kāohi a hōʻemi i nā hōʻailona.

Pehea ʻoe e mālama ai i kahi keiki me ka VACTERL syndrome?

He mea paʻakikī paha ka ʻike ʻana ua loaʻa i kāu keiki ka maʻi VACTERL. Akā, e hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. Pono kāu keiki i ka mālama olakino a me ka nānā ʻana e hoʻokele i nā hōʻailona i ko lākou ola a pau.

Ma ke ʻano he mea mālama i ke keiki, he kuleana nui kou.

• E makaʻala mau i ke olakino a me ka ulu ʻana o kāu keiki. E nānā e ʻike inā ua hiki kāu keiki i nā pae hoʻomohala kūpono no ko lākou mau makahiki (e.g., ke paʻa ʻana i ke poʻo, ka ʻōwili ʻana, ka noho ʻana, ka hele wāwae ʻana).
• E hoʻomau i ka launa pū me nā kauka e mālama nei i kāu keiki. ʻO ka maʻamau, pono ke keiki me VACTERL i ke kōkua o kekahi mau loea (e laʻa, pediatrician, cardiologist, surgeon, nephrologist). E mālama i ka pilina maikaʻi me lākou āpau.
• He mea kōkua nui ke aʻo ʻana i nā ʻano hoʻoilina no kekahi mau ʻohana. Hiki iā ia ke kōkua iā lākou e aʻo hou e pili ana i ke kūlana o kā lākou keiki, e kūkākūkā i nā koho lapaʻau, a loaʻa ka ikaika noʻonoʻo e pono ai lākou e kū i kēia pilikia.

Hiki ke pale ʻia ka maʻi VACTERL?

ʻOiai ʻaʻole i ʻike ʻia ke kumu kikoʻī o ka maʻi VACTERL, ʻaʻohe ala e pale aku ai. Ke hoʻomau nei ka noiʻi ʻana e hoʻoholo i ke kumu o ke kūlana a me nā mea pilikia i loaʻa i nā mākua hou.

He aha ke ʻano o nā manaʻolana e hiki i kekahi me ka VACTERL syndrome ke loaʻa?

I ka wā hāpai, e kamaʻilio me kāu kauka e pili ana i ke ʻano e hiki ai iā ʻoe a me kāu pēpē ke noho olakino. Hiki i ka hoʻāʻo genetic a me ka mālama prenatal ke ʻike i kekahi mau ʻano VACTERL ma mua o ka hānau ʻana o kāu pēpē. A laila hiki i nā kauka ke hoʻomākaukau e hoʻomaka i ka lāʻau lapaʻau i ka wā e hānau ai kāu pēpē.

Ma hope o ka hānau ʻana o ke pēpē, nānā mua nā kauka e ʻike inā ua lawa ke olakino o ke pēpē e mālama ai i nā hōʻailona hoʻoweliweli i ke ola. Pono paha ka lāʻau lapaʻau i ke ola holoʻokoʻa, no ka mea, e ʻike ʻia paha nā hōʻailona hou a i ʻole e loli paha nā hōʻailona kahiko i ka wā kamaliʻi a me ka wā ʻōpio.

ʻAʻohe lāʻau lapaʻau no ka maʻi VACTERL. Eia nō naʻe, me ka mālama pono ʻana a me ka hoʻokele ʻana, hiki ke kōkua ʻia ke keiki e ola i kahi ola maikaʻi.

Āhea ʻoe e ʻike ai i ke kauka?

Inā loaʻa i kāu keiki nā hōʻailona o VACTERL a ke hoʻopilikia nei lākou i kā lākou hana o kēlā me kēia lā a i ʻole ka ulu ʻana, e ʻike pono i ke kauka.

I ka manawa hea e hele ai i ka lumi pilikia:

• Inā ʻaʻole kūpono ka puʻuwai o ka pēpē.
• Inā ʻaʻole ʻoe e ʻai i ka meaʻai a inu waiū paha.
• Inā pilikia ʻoe i ka hanu ʻana.
• Inā ʻaʻole hiki iā ʻoe ke kīkēkē a mimi paha.

I ke kūlana e like me kēia, mai hoʻokaʻulua iki.

He aha nā nīnau āu e nīnau ai i kāu kauka?

He mea maʻamau ka loaʻa ʻana o nā nīnau he nui ke lohe ʻoe e pili ana i ke kūlana o kāu keiki. Mai makaʻu e nīnau i kāu kauka i nā mea āpau āu e hiki ai. Eia kekahi mau laʻana:

• Pono anei kaʻu keiki i ke ʻoki kino?
• He aha nā hopena ʻaoʻao o nā lāʻau lapaʻau āu e ʻōlelo nei?
• He aha kaʻu e hana ai inā ʻaʻole i hiki kaʻu keiki i nā pae hoʻomohala?
• Pehea e mālama ai i kahi keiki hānau hou ma hope o ke ʻoki kino?

Ma waho aʻe o kēia mau nīnau, e nīnau i nā mea āu e noʻonoʻo nei. Eia nā kauka e kōkua iā ʻoe.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

He nui nā kīnā hānau o nā pēpē hānau hou, no laila he mea maʻamau ke kaumaha ke ʻike ʻoe ʻaʻole hiki iā ʻoe ke lawe koke i kāu pēpē i ka home. Akā e hoʻomanaʻo, e hana kahi hui o nā kauka loea i kā lākou mea maikaʻi loa e hāʻawi i kāu pēpē i ka lāʻau e pono ai a hoʻopau i nā hōʻailona e hoʻoweliweli i ke ola.

He nui nā mākua a me nā mea mālama e loaʻa ka ʻoluʻolu nui a me ka ʻike i nā lawelawe e like me ke aʻoaʻo genetic ma hope o ka ʻike ʻia ʻana o ka maʻi VACTERL. No laila, mai kānalua e ʻimi i ke kōkua mai ia ʻano lawelawe. Me ka mālama lapaʻau kūpono, ke aloha, a me ka mālama, hiki iā ʻoe ke kōkua i kāu keiki e ola i ke ola maikaʻi loa.

ʻO ka maʻi VATER, ka hui ʻana o VACTERL, nā ʻano ʻē o ka hānau ʻana, nā kīnā hānau, ke olakino o ka pēpē, ke olakino o ke keiki, ka ulu ʻana o ka embryonic, nā kīnā o ka vertebrae, anal atresia, nā kīnā o ka puʻuwai, ka fistula tracheoesophageal, nā kīnā o ka renal, nā kīnā o nā lālā, Sri Lanka