He aha ka maʻi MRKH (Mayer-Rokitansky-Küster-Hauser Syndrome)? E kamaʻilio kākou e pili ana i kēia?

ʻAʻole anei i hoʻomaka ka wā o kāu kaikamahine, a i ʻole kahi keiki liʻiliʻi āu i ʻike ai, i kona wā maʻamau i ka manawa kūpono i ka makahiki? A i ʻole, ke manaʻo nei ʻo ia i kekahi manawa i ka ʻeha a i ʻole ka ʻoluʻolu ʻole i ka wā e hoʻāʻo ai e moe? I kēia lā e kamaʻilio mākou e pili ana i kahi kūlana olakino kūikawā e hiki ai ke hana i kēia mau mea, akā ʻaʻole maʻamau loa a ʻaʻole nui ka poʻe i lohe. Ua kapa ʻia kēia ʻo Meyer-Rokitansky-Küster-Hauser syndrome , a i ʻole "MRKH syndrome" no ka pōkole. ʻOiai ke ʻano lōʻihi iki ka inoa, he kūlana genetic kakaikahi kēia e hoʻopilikia i nā wahine. E kamaʻilio maʻalahi kākou e pili ana iā ia, ma ke ʻano hiki iā ʻoe ke hoʻomaopopo.

He aha maoli ka maʻi MRKH?

I ka ʻōlelo maʻalahi, ʻo ka maʻi MRKH kahi kūlana kahi i ulu pono ʻole ai ka vagina a me ka uterus o ka wahine a ʻaʻole paha i hoʻokumu piha ʻia i ka wā hānau. E noʻonoʻo e like me kahi hale e kūkulu ʻia ana me kekahi mau lumi i hoʻokumu pono ʻole ʻia. Eia nō naʻe, i ka hapa nui o nā hihia, hana maʻamau nā ovaries a me nā fallopian tubes o ka poʻe me kēia maʻi . Eia kekahi, ʻo nā genitalia waho, ʻo ia hoʻi, ka ʻaoʻao haʻahaʻa o ka vagina, ka puka vaginal, ka labia, ka clitoris, a me ka ulu ʻana o ka lauoho a puni lākou, he mea maʻamau. ʻAʻole pilikia ka urethra, no laila ʻaʻohe pilikia o ka mimi ʻana.

Eia nō naʻe, i kekahi mau ʻano o ka maʻi MRKH, aia paha kekahi mau pilikia ulu i nā ʻōpū ʻē aʻe, e like me nā puʻupaʻa a me ka iwi kuamoʻo.

ʻO ka hapa pinepine, ʻike ʻia kēia maʻi i ka wā ʻōpiopio, ʻo ia hoʻi, i ka makahiki 15-16, i ka wā ʻaʻole hoʻomaka ka menstruation. ʻO kēia no ka mea ʻaʻole i ulu piha ka ʻōpū, no laila ʻaʻole hiki mai ka menstruation. I kekahi manawa, i ka wā e hoʻāʻo ai e moe kolohe, hiki i ka vagina ke ʻeha a paʻakikī paha no ka mea he pōkole a haiki.

No ka mea, ʻaʻole i piha ka ʻōpū a ʻaʻole paha ma laila, ʻaʻole hiki ke hāpai a hāpai i kahi keiki me ke kōkua lapaʻau ʻole ma kēia kūlana. Eia nō naʻe, inā hana pono nā ovaries a ke hana ʻia nei nā hua manu , hiki ke noʻonoʻo ʻia nā koho e like me ka "IVF - in vitro fertilization" a me ka "surrogacy". Inā ʻoe e manaʻolana nei e loaʻa nā keiki, he mea nui loa ke kamaʻilio me kāu kauka e pili ana i ia mea. A laila hiki iā lākou ke aʻo iā ʻoe i nā koho kūpono iā ʻoe.

Ua hoʻohana ʻia kekahi mau inoa ʻē aʻe no ka `(MRKH syndrome)`:

• Ka nele o ka ʻōpū a me ka vagina (CAUV) i ka wā hānau
• ʻO ka agenesis vaginal
• ʻO ke agenesis Müllerian
• ʻAplasia Mullerian (MA)
• ʻO ka maʻi pepeiao renal genital (GRES)

Aia kekahi mau ʻano o ka maʻi MRKH?

ʻAe, aia ʻelua ʻano nui o ka `(MRKH syndrome)`:

• ʻAno 1:I kēia ʻano, hana maʻamau nā ovaries a me nā fallopian tubes, akā ua ālai ʻia a ʻaʻole paha i kahi o ka ʻaoʻao o luna o ka vagina, cervix, a me ka uterus. Eia naʻe, ʻaʻole kēia e hoʻopilikia i nā ʻāpana ʻē aʻe o ke kino.

• ʻAno 2: Ma kēia ʻano, me ka ʻaoʻao o luna o ka vagina, cervix, a me ka uterus e ālai ʻia ana a i ʻole ʻaʻole i kahi, aia paha kekahi mau pilikia me nā fallopian tubes, ovaries, spine, puʻupaʻa, a i ʻole nā ​​​​​​ʻōpū ʻē aʻe.

Pehea ka maʻamau o kēia kūlana?

He maʻi laha ʻole ka maʻi MRKH. Hoʻopilikia wale ia i hoʻokahi i loko o 4,500 mau kaikamahine. No laila, ʻaʻole ia he mea kupanaha ʻaʻole mākou i lohe nui e pili ana i ia mea.

He aha nā hōʻailona o MRKH?

Hiki ke ʻokoʻa nā hōʻailona o ka maʻi MRKH ma muli o ke ʻano āu e loaʻa ai.

I ka hapanui o nā hihia, ʻo ka hōʻailona nui ka nele o ka menstruation (amenorrhea) i ka piha ʻana o ka makahiki 16. He hōʻailona kēia ʻaʻole i ulu pono ka uterus a i ʻole ka vagina. Eia nō naʻe, no ka mea e hana maʻamau ana nā ovaries, hiki ke loaʻa nā hōʻailona ʻē aʻe e pili ana i ka menstruation, e like me ka pehu ʻana a me nā loli o ke ʻano, ʻoiai me ke kahe ʻole o ke koko.

No ka mea, he mau chromosome wahine a me nā hormones kou, ʻike ʻoe i ka ulu ʻana o ka moekolohe maʻamau, e like me ka ulu ʻana o ka umauma a me ka ulu ʻana o ka lauoho ma nā armpits a me ka ʻāpana genital. Eia nō naʻe, hiki iā ʻoe ke ʻeha a ʻoluʻolu ʻole paha ke hoʻāʻo mua ʻoe e moe kolohe. ʻO kēia no ka mea, ʻoi aku ka lahilahi o kou vagina, ka haiki, a me ka pōkole ma mua o ka maʻamau.

Inā loaʻa iā ʻoe ka Type 2 MRKH, hiki iā ʻoe ke ʻike i nā hōʻailona ʻē aʻe e like me:

• Nā pilikia o nā puʻupaʻa, a i ʻole ka hāʻule ʻana o kekahi a i ʻole nā ​​​​puʻupaʻa ʻelua.
• Nā pilikia me nā vertebrae o ka iwi kuamoʻo a i ʻole nā ​​​​​​deformities ʻē aʻe o ka ʻōnaehana iwi.
• Ka hemahema o ka lohe.
• ʻO kekahi mau pilikia o ka puʻuwai.

He aha ke kumu o MRKH?

ʻAʻole ʻike nā kānaka noiʻi i ke kumu pololei o ka maʻi MRKH. Eia nō naʻe, ua ʻike lākou e pili ana ia i kekahi mau pilikia me nā genes a me nā chromosomes. Eia naʻe, ʻaʻole hiki iā lākou ke hoʻopaʻa iā ia i hoʻokahi gene kikoʻī. ʻO ka mea nui , ʻaʻole ia he mea a ka makuahine i hana ai a i ʻole i hana ʻole ai i ka wā hāpai . No laila, mai hopohopo no ia mea.

Hoʻomaka ka ʻōnaehana hānau o ka wahine e ulu i nā pule mua o ka hāpai ʻana. Hoʻokumu ʻia nā ʻōmole fallopian, ka uterus, ka cervix, a me ka ʻaoʻao o luna o ka vagina mai kahi mea i kapa ʻia ʻo Müllerian ducts . I ka poʻe me ka maʻi MRKH, ʻaʻole i ulu piha kēia mau ʻōmole Müllerian. ʻAʻole maopopo i ke kumu e hana ai kēia i kekahi poʻe. Eia nō naʻe, no ka mea, ua ulu kaʻawale nā ​​ovaries mai ke koena o ka ʻōnaehana hānau, ʻaʻole i hoʻopilikia pinepine ʻia nā ovaries.

Pehea ʻoe e ʻike pono ai inā loaʻa iā ʻoe ʻo MRKH?

Hoʻomaopopo pinepine nā kauka i ka maʻi MRKH ke loaʻa ʻole i kahi kaikamahine ʻōpio kona menstruation mua.

ʻO ka hana mua i ka ʻike ʻana i kēia kūlana he hoʻokolohua kino . E hoʻokomo kāu kauka i kahi manamana lima i loko o kou vagina a ana i kona hohonu a me ka laulā. Hiki i kēia hoʻāʻo ke ʻike iā MRKH no ka mea ua pōkole ka vagina. A laila, e noi ʻia paha ʻoe e hana i nā hoʻokolohua kiʻi, e like me ka ultrasound scan a i ʻole ka MRI (magnetic resonance imaging) scan, e ʻike inā ua hoʻopilikia ʻia kou uterus, nā fallopian tubes, nā puʻupaʻa, a i ʻole nā ​​​​​​ʻōpū ʻē aʻe. I kekahi manawa, hiki ke hana ʻia nā hoʻokolohua koko e nānā i kāu mau pae hormone.

He aha nā lāʻau lapaʻau no MRKH?

ʻOkoʻa ka mālama ʻana no ka maʻi MRKH ma muli o kāu mau pahuhopu a me nā hōʻailona. Aia nā lāʻau lapaʻau ʻoki kino a me nā lāʻau lapaʻau ʻoki ʻole. ʻO kekahi o kēia mau mea e komo pū ana me ka vaginoplasty, ka dilation vaginal, a me ka hoʻoili ʻana o ka uterine.

Hoʻonui ʻia ka vaginal

ʻO kekahi ala a nā kauka e hoʻohana ai e hoʻolōʻihi i ka vagina me nā mea i kapa ʻia ʻo vaginal dilators . Hana ʻia kēia mau mea i ka plastic a silicone paha a loaʻa i nā lōʻihi a me nā laulā like ʻole. Kōkua kēia mau mea hana e like me ka paipu e hoʻonui a hoʻolōʻihi i ka vagina mai loko mai. E wehewehe kāu kauka iā ʻoe i ke ala maikaʻi loa e hoʻohana ai i kahi dilator vaginal no kou kūlana.

ʻO Vaginoplasty (hana hou ʻana o ka vagina)

He hana ʻoki kino ka Vaginoplasty e hana ai i kahi vagina . He nui nā ala a nā kauka lapaʻau e hana ai i kēia kaʻina hana. ʻO ka hapa pinepine, hana lākou i lua a uhi iā ia me nā ʻiʻo i lawe ʻia mai kahi ʻāpana ʻē aʻe o kou kino. ʻAe kēia iā ʻoe e moe kolohe maʻamau.

Hoʻoili ʻōpū

Hiki i ka hoʻoili ʻōpū ke hāʻawi i kahi wahine me ka maʻi MRKH i ka manawa kūpono e hāpai i kahi keiki. He hana nui kēia. Pili ia i ka hoʻoili ʻana i kahi ʻōpū mai kahi mea hāʻawi i loko o kahi wahine ʻaʻohe ona. Hāʻawi kēia i nā poʻe me MRKH i ka manawa kūpono e hāpai a hānau i kā lākou keiki ponoʻī. ʻAʻole i hana nui ʻia nā hoʻoili ʻōpū ma nā wahi āpau o ka honua, akā hiki iā lākou ke lilo i lāʻau lapaʻau holomua loa i ka wā e hiki mai ana.

Hiki ke pale ʻia kēia kūlana?

ʻAʻole, ʻaʻohe ala e pale aku ai i ka maʻi MRKH . Hiki ke loaʻa i nā poʻe i loaʻa ʻole ma mua, a i ʻole he genetic. E like me ka mea i ʻōlelo ʻia ma mua, ʻaʻole ia i hoʻokumu ʻia e kahi gene kikoʻī hoʻokahi.

Hiki i ka poʻe me MRKH ke hānau keiki?

ʻAe, hiki i ka poʻe me ka `(MRKH syndrome)` ke hānau keiki!Inā hana ana kāu mau ovaries, hiki ke hoʻohui ʻia kāu mau hua me ka sperm a hoʻomomona ʻia me ka hoʻohana ʻana i ka fertilization in vitro (IVF). A laila hoʻoili ʻia ka embryo i kahi mea lawe hāpai/makuahine pani . ʻO ia hoʻi, e loaʻa iā ʻoe kahi keiki me kāu mau genes ponoʻī, e ulu ana i loko o ka ʻōpū o kekahi wahine olakino ʻē aʻe.

He aha nā pilikia olakino ʻē aʻe e hiki ke hana ʻia e MRKH?

Ua kūkākūkā mua mākou e hiki i ka poʻe me ka "Type 2" MRKH ke loaʻa nā pilikia me nā ʻōpū ʻē aʻe, e like me nā puʻupaʻa, ka iwi kuamoʻo, a i ʻole ka puʻuwai. Eia kekahi, ʻo nā pilikia olakino ʻē aʻe i maʻamau i ka poʻe me ka "Type 2" MRKH:

• Nā pilikia e pili ana i nā vertebrae o ka iwi kuamoʻo.
• ʻO ke skoliosis.
• Nā maʻi ʻino o nā puʻupaʻa, me ka hui ʻana o nā puʻupaʻa a i ʻole ka nele o kekahi a i ʻole nā ​​​​puʻupaʻa ʻelua (renal agenesis).
• ʻO ka hoʻonui ʻia o ka pilikia o nā pōhaku puʻupaʻa, nā maʻi ʻaʻai mimi, a me ka pale ʻana o ke ala mimi.
• Ka hemahema o ka lohe.
• Maʻi puʻuwai.

He mea maʻamau nā pilikia o ka ʻōnaehana hānau i nā poʻe me MRKH. Ma waho aʻe o ka hiki ʻole ke hāpai a loaʻa paha i kā lākou keiki ponoʻī, hiki i ka poʻe me ka ʻōpū i ulu ʻole ke loaʻa i nā maʻi e like me ka endometriosis.

Me kēia mau mea a pau, he mea maʻamau ke manaʻo i kekahi kaumaha o ka naʻau . Hiki i kāu kauka ke kuhikuhi iā ʻoe e hui pū me kahi hui kākoʻo a i ʻole e ʻimi i kahi lawelawe aʻoaʻo noʻonoʻo, e like me ka cognitive-behavioral therapy (CBT), e kōkua iā ʻoe e hoʻoponopono i ke kūlana. Hiki i kēia mau mea ke kōkua nui.

ʻAʻole ia he mea kupanaha ka ʻike ʻana ua loaʻa iā ʻoe ka maʻi Mayer-Rokitansky-Küster-Hauser (MRKH) hiki ke lilo i mea hoʻohoka nui. Inā hānau ʻia ʻoe me kahi ʻōpū a i ʻole ka vagina i ulu ʻole, ʻaʻole paha ʻoe e ʻike a hiki i kou wā ʻōpiopio a hoʻomaka wale ka menstruation. ʻOiai ʻaʻole ia he maʻi e hoʻoweliweli i ke ola, hiki iā ia ke hoʻoulu i ke kaumaha nui, hoʻopilikia i ka hiki i ka wahine ke hāpai a moe. I kekahi mau hihia, hiki iā ia ke hoʻopilikia i nā kino ʻē aʻe a hoʻonui i ka pilikia o kekahi mau maʻi olakino.

Nā mea nui e hoʻomanaʻo (Leka Lawe-Home)

ʻAe, i kēia manawa ua ʻoi aku kou maopopo i ka mea a mākou i kamaʻilio ai, ʻo ia hoʻi ka "MRKH syndrome." ʻO ka mea nui e hoʻomanaʻo ai , ʻaʻole ʻoe hoʻokahi . Aia kekahi poʻe ʻē aʻe ma ka honua e noho nei me kēia mau kūlana.

• Inā loaʻa iā ʻoe nā hōʻailona e like me kēia, he mea nui loa ia e ʻike i ke kauka maikaʻi a loaʻa i kahi hōʻailona pololei .
• Aia nā lāʻau lapaʻau i loaʻa. E kamaʻilio me kāu kauka a koho i ka lāʻau lapaʻau kūpono loa iā ʻoe.
• Mai hoʻopau i ka manaʻolana no nā keiki. Me nā ʻenehana e like me `(IVF)` a me `(surrogacy)`, aia nā manawa kūpono e hoʻokō ai i kēlā moeʻuhane.
• E mālama pū i kou olakino noʻonoʻo.Inā pono, e ʻimi i ke aʻo ʻana a hui pū me nā poʻe i loaʻa nā ʻike like. E hāʻawi kēia iā ʻoe i ka ikaika nui.
• ʻAʻole kēia kou hewa. He kūlana kūlohelohe kēia e hiki ke hana i kekahi.

E hāʻawi kāu kauka, a me kahi hui o nā loea paha, iā ʻoe i ke kākoʻo a me ke alakaʻi e pono ai ʻoe e neʻe i mua me kēia `(MRKH syndrome)`. E hilinaʻi iā lākou. Inā he mau nīnau hou aku kāu e pili ana i kēia, mai makaʻu e nīnau iā lākou. E olakino!

ʻO ka maʻi MRKH, ʻO ka maʻi Mayer-Rokitansky-Küster-Hauser, agenesis vaginal, agenesis uterine, olakino wahine, olakino hānau hou, amenorrhea