Ke maʻi mau nei ʻoe? Hiki ke kumu o ka Primary Immunodeficiency!

Ke maʻi pinepine nei ʻoe a i ʻole kekahi o kou ʻohana? Loaʻa iā ʻoe i kekahi manawa ke anu a i ʻole ka maʻi palū, akā lōʻihi ka manawa e hoʻōla ai? A i ʻole loaʻa hou iā ʻoe ka maʻi like? I kekahi manawa ʻo ke kumu o kēia he nāwaliwali o ka ʻōnaehana pale o ko mākou kino, ka mea e hakakā nei i nā maʻi. I kēia lā e kamaʻilio mākou e pili ana i ia ʻano maʻi, ʻo ia hoʻi ka nele i ka pale kino mua, a i ʻole e like me ka mea i kapa ʻia ma ka ʻōlelo Pelekania ʻo `(Primary Immunodeficiency)`.

He aha ka Immunodeficiency mua?

I ka ʻōlelo maʻalahi, ʻo ka hemahema pale mua he hui o nā kūlana like ʻole he 400 a ʻoi aku kahi i hana pono ʻole ai kā mākou ʻōnaehana pale . E noʻonoʻo i kēia ʻano: ua like ko mākou kino me kahi pā kaua. Aia nā koa e pale aku i kēia pā kaua. ʻO kēlā mau koa ko mākou ʻōnaehana pale. No laila, inā nāwaliwali kēia mau koa, a inā ʻaʻole lākou e hana pono ana, hiki i nā ʻenemi o waho, ʻo ia hoʻi, nā huakini lele (bacteria, viruses, a pēlā aku), ke komo maʻalahi i ke kino a hoʻoulu i nā maʻi.

Ua hoʻohana ʻia kekahi mau inoa ʻē aʻe no kēia ʻano:

• ʻO ka maʻi pale mua.
• Ka maʻi pale ʻole mua (PIDD).
• Nā Kuhihewa Hānau o ka Palekana (IEI).

Hoʻokumu pinepine ʻia kēia mau kūlana e nā loli i loko o kā mākou mau genes, i kapa ʻia ʻo "genetic mutations." Hoʻoili pinepine ʻia kēia mau mea mai kēlā hanauna a i kēia hanauna, ʻo ia hoʻi, ua hoʻoilina ʻia lākou . Akā i kekahi manawa hiki i ke kanaka ke hoʻomohala piha i ke kūlana me ka ʻole o ka mōʻaukala ʻohana. Hoʻōla nā kauka iā ia ma ka pale ʻana a me ka kaohi ʻana i nā maʻi, a ma ka hoʻololi ʻana i nā ʻāpana o ka ʻōnaehana pale i nele.

He aha nā hiʻohiʻona o ka immunodeficiency mua?

E like me kaʻu i ʻōlelo ai ma mua, aia ma mua o 400 mau ʻano o ka immunodeficiency mua. He ʻokoʻa kēlā me kēia ʻano, a ʻokoʻa ko lākou koʻikoʻi . ʻO ia ke kumu e ʻokoʻa ai ka makahiki e ʻike ʻia ai kēia mau kūlana. Hoʻopilikia kekahi mau ʻano i ka wā e hānau ai kahi pēpē, i ka wā kamaliʻi. No laila hiki ke ʻike mua ʻia. Akā aia kekahi mau ʻano ʻē aʻe ʻaʻole i koʻikoʻi. No laila, ʻaʻole paha ʻoe e ʻike he loaʻa iā ʻoe kēia maʻi a hiki i kou wā makua.

Eia kekahi mau laʻana o kēia kūlana:

• ʻO ka hemahema o ka pale ʻana i ka loli maʻamau (CVID)
• ʻAtaxia-telangiectasia
• Ka Maʻi Granulomatous Mau Loa (CGD)
• ʻO ka maʻi DiGeorge
• ʻO ka lymphohistiocytosis hemophagocytic `(ʻO ka lymphohistiocytosis hemophagocytic)`
• Ka hemahema IgA koho
• ʻO ka agammaglobulinemia i hoʻopili ʻia me X

I kēia manawa, mai huikau ʻoe i kēia mau inoa. He mau huaʻōlelo lapaʻau wale nō kēia. ʻO ka mea nui loa, ʻo ia ke ʻimi i ka ʻōlelo aʻoaʻo lapaʻau inā he kānalua kou inā loaʻa iā ʻoe kēia maʻi.

He aha nā hōʻailona o ka immunodeficiency mua?

No nā kānaka he nui, ʻo ka hōʻailona mua o ka immunodeficiency mua he mau maʻi pinepine, mau, a i ʻole nā ​​maʻi ʻē aʻe i paʻakikī ke mālama . Hiki i kēia mau maʻi ke koʻikoʻi loa , a i ʻole he mau pilikia like paha nā lālā ʻē aʻe o ka ʻohana.

ʻO nā hōʻailona ʻē aʻe e ʻike ʻia paha:

• Pono e lawe i nā antibiotics i nā manawa he nui e ho'ōla i ka ma'i.
• Nā pilikia e kū mai ana ma hope o ka loaʻa ʻana o kahi lāʻau pale ola.
• ʻO ka spleen i hoʻonui ʻia (ʻO ka spleen kahi ʻāpana aia ma ka ʻaoʻao hema o luna o ko mākou ʻōpū e kōkua ana i ka ʻōnaehana pale).
• Nā kīʻaha lymph pehu (nā mea e like me nā puʻupuʻu ma ka lima).
• ʻO ka pohō kaumaha a i ʻole ka ulu ʻole ʻana (ʻoi aku hoʻi i nā keiki ʻōpio).
• Nā pilikia hoʻoheheʻe pinepine e like me ka diarrhea.
• Nā maʻi Autoimmune ( nā kūlana kahi e hoʻouka ai ka ʻōnaehana pale o ko mākou kino i kā mākou mau cell ponoʻī).

E noʻonoʻo, loaʻa i kāu keiki ke kuni a me ka ʻūlū i hoʻokahi a ʻelua paha o ka mahina a pono e hāʻawi ʻia i nā lāʻau antibiotic. A i ʻole, hoʻomohala mau lākou i nā ʻulaʻula koʻikoʻi a me nā pehu ma ko lākou ʻili, kahi e lōʻihi ai ka manawa e ho'ōla ai. Inā hoʻomau kēia mau mea, pono ʻoe e hopohopo no ia mea.

He aha ke kumu o kēia?

E like me kaʻu i ʻōlelo ai ma mua, ʻo ka hemahema mua o ka pale ʻana i ka maʻi maʻi ke kumu o nā hoʻololi genetic e hoʻopilikia i nā ʻāpana o kā mākou ʻōnaehana pale, e like me nā cell a me nā protein . Hiki i kēia mau hoʻololi genetic ke hana i kekahi mau ʻāpana o kā mākou ʻōnaehana pale:

• Malia paha ʻaʻole ʻoi aku ka hana ma mua o ka maʻamau.
• He hemahema paha ia.
• Hiki ke nalo loa aku paha .

Ma waena o 50% a me 60% o kēia mau kūlana i hana ʻia e nā hemahema i loko o nā cell i kapa ʻia ʻo B lymphocytes (nā cell B) . ʻO kēia mau cell B kahi ʻano cell kūikawā i loko o kā mākou ʻōnaehana pale. ʻO lākou nā mea e hana i nā antibodies , nā protein kūikawā e kōkua i ka luku ʻana i nā pathogens e like me nā bacteria a me nā virus e komo i loko o ko mākou kino. No laila e noʻonoʻo, inā ʻaʻole hana pono kēia mau cell B, inā ʻaʻole lākou e hana i nā antibodies, ʻoi aku ka maʻalahi o ko mākou kino i ka maʻi, ʻeā?

ʻO wai ka mea i ʻoi aku ka pilikia o ka loaʻa ʻana o kēia maʻi?

Hiki i kekahi ke loaʻa i ka PIDD. Akā inā loaʻa i kekahi o kou ʻohana maoli, ʻoi aku ka nui o kou loaʻa ʻana . ʻO ka hapa nui o ka manawa, ʻike ʻia kēia mau hemahema pale mua ma mua o ka piha ʻana o 20 makahiki. Eia kekahi, no nā kāneUa ʻōlelo ʻia hoʻi he mea maʻamau kēia ʻano.

He aha nā pilikia e hiki ai i kēia ke hana?

Hiki i ka immunodeficiency mua ke hoʻonui i ka pilikia o nā pilikia ma hope o ke ola. Hiki i kēia ke komo pū me nā maʻi autoimmune a i ʻole kekahi mau maʻi kanesa . Inā ʻaʻole mālama ʻia, hiki i ka PIDD ke alakaʻi i nā maʻi ʻino loa .

Pehea e ʻike ai nā kauka i kēia?

E hoʻoholo kāu kauka inā he PIDD kāu ma ka nānā ʻana i kāu mōʻaukala olakino pilikino a me kou ʻohana, kahi hoʻokolohua kino, a me ka hana ʻana i kekahi mau hoʻokolohua hoʻokolohua .

No ka hōʻoia ʻana i ka maʻi, hiki i kāu kauka ke kauoha i nā hoʻokolohua e like me:

• Nā hoʻāʻo koko: Hiki i kēia ke ʻike i nā ʻano ʻē aʻe i loko o ka ʻōnaehana pale (no ka laʻana, nā pae antibody haʻahaʻa a i ʻole ka helu haʻahaʻa o nā cell immune).
• Nā hoʻāʻo hoʻoilina: No ka ʻike ʻana i nā hemahema i loko o nā genes, a i ʻole nā ​​​​mutation.
• ʻO ka cytometry kahe: He ʻano kēia no ka nānā ʻana i nā laʻana o nā pūnaewele ʻōnaehana pale me ka hoʻohana ʻana i nā kukuna laser kūikawā.

Eia kekahi, ma kekahi mau ʻāina, e like me ʻAmelika Hui Pū ʻIa, hana ʻia ka nānā ʻana i nā keiki hānau hou ma aneane kēlā me kēia keiki hānau hou e nānā no kahi ʻano o ka immunodeficiency i hui pū ʻia (SCID). ʻAe kēia i ka ʻike mua ʻana a me ka mālama ʻana i nā pēpē me kēia maʻi koʻikoʻi.

He aha nā lāʻau lapaʻau no kēia?

Inā ua ʻike ʻia ʻoe me ka immunodeficiency mua, ʻo nā pahuhopu nui o ka mālama ʻana, ʻo ia ke kāohi ʻana i nā maʻi o kēia manawa a pale i nā maʻi e hiki mai ana. ʻO ka lāʻau lapaʻau pololei āu e loaʻa ai e pili ana i ke ʻano o ka maʻi āu e loaʻa ai a me ke ʻano o PIDD.

Hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau e like me:

• Nā lāʻau lapaʻau antibiotic: Kāohi a mālama paha i nā maʻi bacteria.
• Nā lāʻau antiviral: Kōkua i ka ho'ōla ʻana mai nā maʻi i hoʻokumu ʻia e nā virus.
• ʻO ka globulin pale: Hiki ke hāʻawi ʻia kēia mau mea ma ke ʻano intravenous (IV) a i ʻole ma ke ʻano subcutaneous. Hana lākou ma ka hoʻololi ʻana i kekahi o nā ʻāpana o ka ʻōnaehana pale i nalowale (e like me nā antibodies i haʻahaʻa loa).

I kekahi manawa, pono paha ke ʻoki ʻana e kāohi i nā pilikia i hoʻokumu ʻia e ka maʻi. No ka laʻana, inā he abscess (kahi hōʻiliʻili o ka pus i loko o ke kino), hiki ke wehe ʻia ma ke ʻoki ʻana e hoʻokahe i ka pus. Hiki i kēia ke kōkua i ka hōʻemi ʻana i ka ʻeha a me ka wikiwiki o ka ho'ōla ʻana.

I nā hihia koʻikoʻi aʻe, hiki i ke kauka ke hana i kahi hoʻoili cell stem.Hiki ke ʻōlelo ʻia. Pili kēia i ka hoʻololi ʻana i nā pūnaewele ʻōnaehana pale kīnā a nalowale paha me nā pūnaewele hou. Ma kēia, ua hoʻokomo ʻia nā pūnaewele kumu (hiki ke ulu i nā ʻano pūnaewele ʻē aʻe) i lawe ʻia mai kahi mea hāʻawi i loko o kou kino. I ka hala ʻana o ka manawa, ulu kēia mau pūnaewele kumu i nā pūnaewele ʻōnaehana pale maʻamau.

Ua lilo ka Gene therapy i mea lapaʻau kūleʻa no kekahi mau ʻano PIDD.

Hiki ke pale ʻia ka immunodeficiency mua?

ʻOiai ʻo ka hapa nui o nā PIDD i hoʻokumu ʻia e nā hoʻololi genetic , ʻaʻohe ala e pale aku ai iā lākou. Eia nō naʻe, inā loaʻa i kekahi o kou ʻohana kahi immunodeficiency mua, makemake paha ʻoe e noʻonoʻo i ke aʻoaʻo genetic . Hiki i kēia ke hāʻawi iā ʻoe i ka ʻike hou aku a me ke alakaʻi.

He aha ka mea e hiki mai ana no ka poʻe me kēia maʻi? (Wānana)

Me ka mālama kūpono, hiki i ka hapa nui o ka poʻe me PIDD ke ola olakino. I kekahi mau hihia, pono paha ʻoe e lawe i ka lāʻau lapaʻau no ke koena o kou ola. Pono ʻoe e hana i kāu mea maikaʻi loa e pale aku i nā maʻi . Eia kekahi mau ʻōlelo aʻoaʻo e kōkua:

• E hoʻomaʻamaʻa i ka holoi maikaʻi ʻana i kou mau lima - holoi pono i kou mau lima me ke kopa a me ka wai. E holoi pono i kou mau lima ma mua a ma hope o ka ʻai ʻana, ma hope o ka hoʻohana ʻana i ka lua, ma hope o ka hoʻopā ʻana i nā holoholona, ​​a ma hope o ka hoʻopā ʻana i kekahi mea lepo.
• E noho mamao aku mai nā wahi i piha ai ka poʻe a me ka poʻe maʻi .
• E hahai pono i nā ʻōlelo a kāu kauka e pili ana i ka loaʻa ʻana o ka lāʻau pale .
• E hoʻomaha pono .
• E hahai i kahi papaʻai olakino a me ka hoʻolālā hoʻoikaika kino e kūpono iā ʻoe.

Inā loaʻa iaʻu, a kānalua paha he pilikia pale mua koʻu, i ka manawa hea e ʻike ai au i ke kauka?

Inā loaʻa iā ʻoe kahi maʻi i hala ʻole, he koʻikoʻi loa, a i ʻole e hoʻi hou mai ana, e ʻike i ke kauka e nānā i ka PIDD. Inā loaʻa iā ʻoe ka PIDD, e haʻi koke i kāu kauka inā loaʻa iā ʻoe ke kuni a i ʻole kekahi mau hōʻailona o ka maʻi . He mea nui kēia e pale aku i nā pilikia.

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

Inā kānalua ʻoe i kēia kūlana, a inā ʻike ʻoe ua loaʻa iā ʻoe, hiki iā ʻoe ke nīnau i kāu kauka e like me kēia:

• He aha ke ʻano o ka immunodeficiency mua i loaʻa iaʻu?
• Hiki ke hoʻoilina ʻia kēia e kaʻu mau keiki?
• He aha ke ʻano o ka lāʻau lapaʻau āu e paipai nei?
• He aha nā hopena ʻaoʻao e hiki mai ana o ka lāʻau lapaʻau? Pehea lā wau e hopohopo ai no lākou?
• He aha nā pilikia e hiki ke hana ma muli o kēia ʻano?

Hiki ke paʻakikī ka noho ʻana me nā kūlana e pili ana i ka hemahema o ka pale kino mua. ʻO nā maʻi pinepine, mau, a paʻakikī ke mālama ʻia hiki ke hoʻonāwaliwali maoli, a hiki ke loaʻa ka hopena nui i kou olakino noʻonoʻo.

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Akā, ʻaʻole pono ʻoe e hele wale ma kēia huakaʻi. E noi i kāu kauka e kōkua iā ʻoe e ʻimi i kahi hui kākoʻo kahi e hiki ai iā ʻoe ke kamaʻilio a kaʻana like i nā manaʻo me nā poʻe ʻē aʻe i hele i nā ʻike like. Hiki i kēia ke lilo i kumu ikaika nui.

ʻO ka mea hope loa, e hoʻomanaʻo i kēia (Leka Lawe-Home)

ʻAe, eia kekahi mau mea e hoʻomanaʻo ai i ka hōʻuluʻulu manaʻo e pili ana i ka Primary Immunodeficiency a mākou i kamaʻilio ai i kēia lā:

• He maʻi kēia i hoʻokumu ʻia e ka nāwaliwali o ko kākou ʻōnaehana pale kino , pinepine ʻia ma muli o nā kumu hoʻoilina .
• ʻO kekahi o nā hōʻailona nui , ʻo ia nā maʻi pinepine a koʻikoʻi e lōʻihi ai ka manawa e ho'ōla ai .
• Nui nā ʻano o kēia, no laila hiki ke ʻokoʻa nā hōʻailona a me ke koʻikoʻi mai kēlā kanaka a kēia kanaka.
• He mea nui loa ka ʻike mua ʻana a loaʻa ka lāʻau kūpono .
• Me ka lāʻau lapaʻau, hiki ke kaohi ʻia ka maʻi a hiki i nā poʻe he nui ke ola maʻamau .
• He mea nui e hana i nā hana e pale aku ai iā ʻoe iho mai nā maʻi .

Inā loaʻa iā ʻoe a i ʻole kekahi āu i ʻike ai kekahi o kēia mau hōʻailona, ​​mai kānalua e ʻimi i ke kōkua lapaʻau. ʻO ka wikiwiki o kou ʻike ʻia ʻana, ʻo ka nui o ka holomua o ka lāʻau lapaʻau. Aloha nui i nā mea a pau!

ʻO ka hemahema mua o ka pale ʻana i ka maʻi, ka ʻōnaehana pale, nā maʻi, nā loli hoʻoilina, PIDD, ka lāʻau lapaʻau, nā hōʻailona