Ua loaʻa anei iā ʻoe kahi puʻupuʻu e like me kēia? E kamaʻilio kākou e pili ana i nā puʻupuʻu Desmoid!

Ua ʻike anei ʻoe i kahi puʻupuʻu ʻē a pehu paha ma kekahi wahi o kou kino a hopohopo? I kekahi manawa, ʻaʻole paha he mea weliweli kēia mau mea. Eia nō naʻe, i kēia lā e kamaʻilio mākou e pili ana i kahi ʻano puʻupuʻu i kūikawā iki a pono e nānā kūikawā ʻia. Ua kapa ʻia kēia mau mea he mau puʻupuʻu desmoid. E nānā kākou i ko lākou ʻano, no ke aha lākou e hana ai, pehea e ʻike ai iā lākou, a pehea e mālama ai iā lākou.

He aha nā ʻōpū Desmoid? E ʻike maʻalahi kākou!

I ka ʻōlelo maʻalahi, ʻaʻole he maʻi kanesa nā puʻupuʻu desmoid. Akā ua kapa ʻia lākou he fibromatosis aggressive. ʻO ia hoʻi, i kekahi manawa hiki iā lākou ke ulu wikiwiki a me ka ikaika. Hoʻokumu lākou i loko o nā ʻiʻo pili i loko o ko mākou kino. E noʻonoʻo e like me ke kāpili e hoʻopaʻa ana i nā ʻāpana like ʻole o ko mākou kino.

ʻOiai hiki i kēia mau puʻupuʻu ke ulu ma nā wahi āpau o ke kino, ʻike pinepine ʻia lākou ma ka ʻōpū, nā lima, nā wāwae, a i kekahi manawa ke poʻo a me ka ʻāʻī .

ʻO ka mea nui loa, ʻaʻole e laha kēia mau puʻupuʻu mai kekahi wahi o ke kino a i kekahi wahi (ʻaʻole lākou e metastasize) e like me ke kanesa.

Eia nō naʻe, aia kekahi pilikia. Ulu lohi pinepine kēia mau puʻupuʻu. Eia nō naʻe, hiki i kekahi ke ulu koke a pālahalaha i loko o nā ʻiʻo kokoke (locally infiltrative). I kēlā hihia, he mea paʻakikī loa ka wehe ʻana iā lākou me ke ʻoki ʻana. ʻO kekahi mea ʻē aʻe, ʻoi aku ka nui o ko lākou hoʻi hou ʻana (recur) ʻoiai ma hope o ka mālama ʻana. Akā, ʻaʻole like ke kūlana o kēlā me kēia kanaka, a he mea nui e hoʻomanaʻo i kēlā.

Aia kekahi ʻano o kēia mau nati?

ʻAe, hiki ke hoʻokaʻawale ʻia nā ʻōpū desmoid i kekahi mau ʻano nui, ma muli o kahi i hana ʻia ai lākou:

• Nā puʻupuʻu desmoid o ka paia ʻōpū: Hoʻomohala kēia i loko o ka paia ʻiʻo (paia ʻōpū) e hoʻopuni ana i nā ʻōpū i loko o ko mākou ʻōpū (e like me ka ʻōpū).
• Nā puʻupuʻu desmoid intra-abdominal: Hoʻomohala kēia i loko o ka uhi o nā ʻiʻo a puni nā ʻōpū (mesentery).
• Nā puʻupuʻu desmoid ma waho o ka ʻōpū: Hoʻomaka kēia ma waho o ka ʻōpū a me ka paia o ka ʻōpū. Hoʻomaka pinepine lākou ma nā poʻohiwi, nā lima o luna, ka paia o ka umauma, a me nā wāwae o luna. Hiki iā lākou ke ulu ma ke poʻo a me ka ʻāʻī.

He aha nā hōʻailona? Pehea mākou e ʻike ai inā loaʻa iā mākou?

ʻAʻole mau nā ʻōpū desmoid e hōʻike i nā hōʻailona nui. Eia nō naʻe, ʻike wale ʻia nā hōʻailona ke ulu nui ka ʻōpū e kaomi i nā aʻalolo a i ʻole nā ​​​​​​kino kokoke. Hiki i kēia mau mea ke komo pū me:

• He puʻupuʻu a pehu paha: ʻIke pinepine ʻia kēia ma nā lima, nā wāwae, a i ʻole ka ʻōpū.
• Ka luaʻi a me ka luaʻi: ​​Hiki ke loaʻa kēia mau hōʻailona inā he puʻupuʻu i loko o ka ʻōpū.
• ʻEha a ʻeha paha: Hiki ke ʻike ʻia kēia ke kaomi ka puʻupuʻu i nā aʻalolo, nā ʻōpū, a i ʻole nā ​​​​kīʻaha koko kokoke i ka puʻupuʻu.
• He paʻakikī ke hoʻoneʻe i ka lima a i ʻole ka wāwae.

He mea nui: ʻAʻole nā ​​puʻupuʻu a pau e kū mai ana ma ke kino he puʻupuʻu desmoid. Eia nō naʻe, inā he puʻupuʻu kou i ʻoi aku ma mua o ʻelua ʻīniha (ma kahi o 5 kenimika), e hoʻomau ana i ka ulu ʻana, a i ʻole he ʻeha , ʻoi aku ka maikaʻi o ke ʻike ʻana i ke kauka no ka ʻōlelo aʻo.

No ke aha i hoʻokumu ʻia ai kēia mau puʻupuʻu desmoid? He aha nā kumu?

Hoʻokumu ʻia kēia mau puʻupuʻu i ka wā e loli ai nā pūnaewele i loko o nā ʻiʻo pili a hoʻomaka e māhele wikiwiki a me ke kāohi ʻole ʻia. I ka hala ʻana o ka manawa, hōʻiliʻili kēia mau pūnaewele keu a hana i kahi puʻupuʻu.

ʻAʻole maopopo loa i nā kauka ke kumu e loli ai kēia mau cell ma kahi mua. Eia nō naʻe, ua ʻike ʻia kekahi mau hoʻololi gene e pili ana i nā puʻupuʻu desmoid. Hiki i kēia ʻike ke kōkua i ka hoʻomohala ʻana i nā lāʻau lapaʻau e kuhikuhi ana i kēlā mau hoʻololi a kāohi i ka ulu ʻana o nā puʻupuʻu.

ʻO wai ka mea i ʻoi aku ka paʻakikī o ka loaʻa ʻana o kēia mau mea? (Nā kumu pilikia)

ʻOi aku ka nui o ka hiki i kekahi poʻe ke loaʻa i nā ʻōpū desmoid. E nānā kākou iā lākou:

• ʻŌpio: ʻIke pinepine ʻia kēia maʻi i nā poʻe ma waena o 30 a me 40 mau makahiki.
• ʻOiai he wahine: ʻOi aku ka nui o ka hiki i nā wahine ke loaʻa i kēia mau puʻupuʻu, ʻoiai i ka wā hāpai a i ʻole ma hope koke iho o ka hānau ʻana. ʻIke pinepine ʻia lākou ma ka paia ʻōpū. Ma muli o ka piʻi ʻana o nā pae estrogen i ka wā hāpai, manaʻoʻiʻo kekahi poʻe loea aia kahi pilina ma waena o ka estrogen a me nā puʻupuʻu desmoid.
• ʻO ka loaʻa ʻana o kahi maʻi i kapa ʻia ʻo Familial Adenomatous Polyposis (FAP): He maʻi hoʻoilina kēia. Ma waena o 5% a me 10% o ka poʻe me FAP e loaʻa i nā ʻōpū desmoid. ʻO ka hapa pinepine, ulu kēia mau ʻōpū ma kahi i hana mua ʻia ai ke ʻoki ʻana e wehe i kahi ulu liʻiliʻi (colon polyp) i loko o ka colon.
• ʻEha: Ua hōʻike ʻia e ka noiʻi ʻana he ʻeha nui, a i ʻole ka ʻeha pinepine ʻana i ka wahi like (no ka laʻana, ke kuʻi pinepine ʻana i ka wahi like i ka wā e pāʻani ana i nā haʻuki), hoʻonui i ka pilikia o ka hoʻomohala ʻana i kahi puʻupuʻu desmoid.

Pehea e ʻike ai nā kauka i kēia? (Diagnosis)

Inā loaʻa iā ʻoe kēia mau hōʻailona, ​​e hana mua kāu kauka i kahi hoʻokolokolo kino a nīnau e pili ana i kāu mau hōʻailona. A laila, e hana paha lākou i kēia mau hoʻokolohua:

• Nā hoʻāʻo kiʻi: ʻO nā mea maʻamau ka CT scans a me MRIs. Hiki i kēia ke ʻike maopopo i ka puʻupuʻu i loko o ke kino.
• Biopsy: ʻO kēia ka hoʻāʻo koʻikoʻi loa e hana . Pili kēia i ka lawe ʻana i kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka puʻupuʻu a me ka nānā ʻana i nā ʻāpana ma lalo o kahi microscope. ʻO kēia wale nō ke ala e hoʻoholo pono ai i ke ʻano o ka puʻupuʻu.

Inā kānalua kāu kauka he FAP kou, hiki iā lākou ke hana i nā hoʻāʻo hou aku. ʻAʻole nā ​​​​kānaka āpau me nā cysts desmoid i loaʻa iā FAP. Eia nō naʻe, he mea nui e ʻike inā loaʻa iā ʻoe, no ka mea, ʻoi aku ka nui o ka pilikia o ka poʻe me FAP i ka loaʻa ʻana o ka maʻi ʻaʻai colon.

Pehea e mālama ai? (Lapaʻau)

Hoʻomaʻamaʻa pinepine ʻia nā puʻupuʻu desmoid ma ke ʻano like me nā puʻupuʻu kanesa. ʻO ke ʻano kēia e hana pū ʻoe me kahi hui o nā kauka mai nā ʻoihana kūikawā like ʻole, me kahi oncologist.

I ka wā ma mua, ʻo ke ʻoki kino ke ʻano nui o ka lāʻau lapaʻau no kēia mau puʻupuʻu. Eia nō naʻe, no ka mea ua ʻike ʻia he kiʻekiʻe ka manawa e hoʻi hou mai ai kēia mau puʻupuʻu ma hope o ke ʻoki kino, ua hoʻokumu nui ʻia nā lāʻau lapaʻau o kēia manawa ma nā lāʻau lapaʻau. ʻO nā lāʻau lapaʻau ʻē aʻe:

• Nānā ikaika (a i ʻole ke kali makaʻala): Inā e ulu mālie ana kāu puʻupuʻu a ʻaʻole e hōʻike ana i nā hōʻailona nui, e nānā mau kāu kauka iā ʻoe. E hana lākou i nā scan maʻamau. Inā loli kou kūlana, hiki ke hoʻomaka koke ʻia ka lāʻau lapaʻau.
• ʻOki kino: Pono paha ke ʻoki kino inā ulu wikiwiki loa ka puʻupuʻu a i ʻole ke kumu nei i nā hōʻailona koʻikoʻi. A i ʻole, inā pilikia ka puʻupuʻu i ka hoʻoulu ʻana i kahi pilikia koʻikoʻi, e like me ke ālai ʻana o ka ʻōpū, hiki ke hana ʻia ke ʻoki kino.
• Lapaʻau i kuhikuhi ʻia: Hana kēia mau lāʻau lapaʻau ma ka kuhikuhi ʻana i nā genes kikoʻī a i ʻole nā ​​​​protein e kōkua i ka ulu ʻana o nā ʻōpū desmoid. Hiki i kāu kauka ke ʻōlelo aku i kahi ʻano lāʻau i kapa ʻia ʻo Tyrosine Kinase Inhibitor (TKI).
• Cryoablation: He koho ʻē aʻe kēia i ke ʻoki kino. Hoʻohana ia i ke anu nui e luku ai i ka puʻupuʻu. Hiki ke hoʻohana ʻia kēia lāʻau lapaʻau no nā puʻupuʻu desmoid liʻiliʻi i loaʻa ma waho o ka ʻōpū (no ka laʻana, ma nā lima a me nā wāwae).
• Chemotherapy: Hoʻohana ʻia kēia mau lāʻau lapaʻau e hoʻēmi a hoʻopau loa paha i nā puʻupuʻu desmoid. Eia nō naʻe, aia nā manaʻo like ʻole e pili ana i ka holomua o kēia lāʻau.
• Hoʻōla Hormone: Hiki i nā lāʻau lapaʻau hormonal e like me Tamoxifen ke kōkua i ka hoʻōki ʻana i ka ulu ʻana o nā ʻōpū desmoid.
• Lapaʻau radiation: Hiki ke hoʻohana ʻia ka radiation e hoʻēmi a luku paha i ka puʻupuʻu. Hoʻohana wale ʻia a i ʻole i hui pū ʻia me nā lāʻau lapaʻau ʻē aʻe.

Pono anei au e ʻike pinepine i ke kauka? (Hope up)

ʻAe, ʻoiaʻiʻo nō. ʻOiai ma hope o ka mālama ʻana, pono ʻoe e ʻike pinepine i kāu kauka no ka mea he kiʻekiʻe ka manawa e hoʻi mai ai nā puʻupuʻu desmoid.ʻO ka maʻamau, pono ʻoe e hana i nā hoʻokolohua a me nā scan i kēlā me kēia ʻeono mahina no nā makahiki ʻekolu mua. Ma hope o kēlā, inā ʻaʻohe pilikia, pono ʻoe e hele no nā nānā ʻana i hoʻokahi manawa i ka makahiki a i ʻole i kēlā me kēia ʻelua makahiki. Na kāu kauka e hoʻoholo i ka pinepine o kou hele ʻana mai, ma muli o kou kūlana.

He aha kaʻu e manaʻo ai me kēia kūlana? (Prognosis)

ʻAʻole like nā puʻupuʻu desmoid no kēlā me kēia kanaka. Loaʻa i kekahi poʻe lākou akā ʻaʻole hōʻike i kekahi mau hōʻailona. Ma kahi o ʻelua o ka ʻumi mau kānaka i loaʻa iā lākou a nalowale lākou me ka ʻole o ka lāʻau. Eia naʻe, no kekahi poʻe, hiki iā lākou ke ulu a lilo i mea hoʻoweliweli i ke olakino.

Ma muli o ke ʻano o ka hoʻololi ʻana o ka genetic i loko o ka puʻupuʻu, hiki i nā kauka ke hana i kekahi mau kuhi e pili ana i ke ʻano o ka huhū o ka puʻupuʻu a pehea ka hiki ke hoʻi hou mai ma hope o ka mālama ʻana. Akā, ʻaʻohe mea hiki ke ʻōlelo maopopo ʻia.

ʻOiai ʻaʻole ia he maʻi kanesa, ʻaʻole ia manaʻo e kali wale ʻoe. He maʻi koʻikoʻi ka puʻupuʻu desmoid e pono e nānā ʻia e kāu kauka.

Pehea ka nui o ke ola? (Nā helu ola)

ʻAʻole hoʻoweliweli pinepine nā ʻōpū desmoid i ke ola, akā hiki iā lākou ke lilo i pilikia no ʻoe e hoʻomau i ka mālama ʻana.

ʻO ka mea nui loa ma ʻaneʻi, ʻo ia ka helu ola hou ʻole ('recurrence-free survival rate`) . Ma hope o ʻelima mau makahiki, hiki i kēia helu ke piʻi mai ka haʻahaʻa o 23% a hiki i ka 65%. Aia nui kēia i ke ʻano o ka hoʻololi ʻana o ka genetic i loko o ka puʻupuʻu.

E wehewehe kāu kauka iā ʻoe i ka mea hiki iā ʻoe ke manaʻo ma muli o kāu hōʻailona.

ʻO ka mea nui loa: ke kākoʻo loea

Hiki i nā puʻupuʻu desmoid ke paʻakikī loa ke wānana. Hiki iā lākou ke hōʻeha, kaupalena i ka neʻe ʻana, a hoʻopilikia paha i nā ʻōpū kokoke. Hiki i kēia kānalua ke lilo i mea kaumaha loa. Ulu mālie kekahi mau puʻupuʻu a ʻaʻole pono e mālama koke ʻia, akā pono nā mea ʻē aʻe e mālama pono loa.

No ka mea, he maʻi laha ʻole nā ​​cysts desmoid a hoʻopilikia like ʻole i kēlā me kēia kanaka, he mea nui e ʻimi i ka lāʻau lapaʻau mai kahi hui o nā loea i ʻike i ka hoʻokele ʻana i kēia mau kūlana paʻakikī. Hāʻawi kahi ala multidisciplinary i ka manawa kūpono loa e hoʻokele ai i nā hōʻailona a hoʻomaikaʻi i ka maikaʻi o ke ola.

Nā mea e pono ai kākou e aʻo mai kēia (Leka Lawe-Home)

ʻAe, no laila, mai nā mea a mākou i kamaʻilio ai, aia kekahi mau mea nui e pono ai ʻoe e hoʻomanaʻo:

• ʻOiai ʻaʻole he maʻi kanesa ka puʻupuʻu desmoid, ʻaʻole ia he mea e lawe māmā ʻia.
• Inā he puʻupuʻu a pehu paha kou ma kou kino, ʻoiai inā e hoʻomau ana ka ulu ʻana a ʻeha paha, mai hoʻonele i ka manawa a e ʻike koke i ke kauka.
• Hiki i kēia kūlana ke lilo i mea paʻakikī iki, no laila ʻo ka loaʻa ʻana o kahi hōʻailona pololei a me ke kākoʻo o kahi hui lapaʻau akamai e kōkua nui iā ʻoe e ola holomua me kēia kūlana.
• Mai hopohopo, akā e makaʻala. ʻO ka mea nui loa, ʻo ia ka hahai ʻana i nā ʻōlelo aʻoaʻo lapaʻau kūpono.

Inā he mau nīnau hou aku kāu e pili ana i kēia, e kamaʻilio me kāu kauka. E noho olakino!

ʻO nā puʻupuʻu desmoid, fibromatosis aggressive, nā puʻupuʻu pili i nā ʻiʻo pili, nā puʻupuʻu ʻaʻole maʻi kanesa, nā hōʻailona puʻupuʻu, ka mālama ʻana i ka puʻupuʻu, Sri Lanka Health