Ke maʻi mau nei kāu keiki liʻiliʻi? Hiki paha iā ia ke SCID (Severe Combined Immunodeficiency)! E kamaʻilio kākou e pili ana i ia mea!

Ke maʻi mau nei kāu keiki liʻiliʻi? Ke hoʻomaikaʻi nei kekahi maʻi ma mua o kekahi? I kekahi manawa he mea maʻamau kēia, akā hiki ke hana ʻia e kahi maʻi kakaikahi loa akā koʻikoʻi loa. ʻO ia ke ʻano o SCID (Severe Combined Immunodeficiency) . E kamaʻilio kākou e pili ana i kēia me nā kikoʻī hou aku i kēia lā, ʻeā? Mai hopohopo, he mea nui loa ka makaʻala i kēia.

He aha ka SCID (Severe Combined Immunodeficiency)? E hoʻomaopopo maʻalahi kākou.

I ka ʻōlelo maʻalahi, he maʻi hoʻoilina laha ʻole ʻo SCID e pili ana i ka poʻe kakaikahi. Hoʻopilikia ia i ka ʻōnaehana pale o ko mākou kino, ka mea e hakakā nei i nā maʻi, i ka hana hewa. Hiki ke lilo i maʻi koʻikoʻi loa.

Kāhea mākou i kēia he 'primary immune deficiency'. Kāhea pū kekahi mau puke iā ia he 'inborn error of immunity'. ʻO ia hoʻi, he mea ia e kū nei mai ka lā hānau a hiki ke hoʻoili ʻia i nā hanauna. Hiki i nā loli genetic like ʻole ke hana i kēia maʻi SCID.

E noʻonoʻo wale, ʻoiai ma kahi ʻāina e like me ʻAmelika, inā hānau ʻia he 58,000 mau keiki i kēlā me kēia makahiki, hoʻokahi wale nō o lākou e loaʻa i kēia maʻi i kapa ʻia ʻo SCID (Severe Combined Immunodeficiency). Hiki iā ʻoe ke noʻonoʻo i ka laha ʻole o ia mea.

He aha ka mea e hana ʻia i loko o kahi pēpē me SCID?

I mea e maopopo ai i kēia, pono mua kākou e nānā i ke ʻano o ka hana ʻana o ka ʻōnaehana pale o ko kākou kino, ka pūʻali koa e hakakā nei i nā maʻi.

E noʻonoʻo, i ka wā e noho ana kahi pēpē i loko o ka ʻōpū o kona makuahine, hoʻomaka kahi pūʻali koa o nā pūnaewele e hakakā ana i nā maʻi e hana i loko o kona kino. ʻO ke keʻena nui o kēia pūʻali koa ka iwi iwi . Aia nā pūnaewele kūikawā ma laila, a mākou e kapa nei he ' nā pūnaewele kumu ' . Hiki i kēia mau pūnaewele kumu ke hana i nā ʻano nui ʻekolu o nā pūnaewele koko:

• Nā hunaola koko ʻulaʻula : ʻO kēia nā mea e lawe ai i ka oxygen ma ke kino holoʻokoʻa.
• Nā hunaola koko keʻokeʻo : ʻO kēia nā koa i loko o ko mākou kino e pale iā mākou mai nā maʻi.
• Nā Platelets : Kōkua kēia i ke kāpili ʻana o ke koko.

I kēia manawa, ma waena o kēia mau keʻokeʻo koko, aia kahi hui kūikawā i kapa ʻia ʻo 'lymphocytes' . ʻO kēia nā mea e hakakā pololei ana i nā maʻi. ʻElua ʻano nui o kēia mau lymphocytes: nā T-cell a me nā B-cell. He mea nui loa kēia mau keʻena ʻelua i ke kaua ʻana i nā maʻi.

• Hoʻomaopopo nā pūnaewele T i nā 'mea hoʻouka kaua' e hoʻoulu ai i ka maʻi - nā mea e like me ka bacteria a me nā virus - e komo i ke kino, hoʻouka iā lākou, a luku iā lākou.
• Hana nā pūnaewele B i nā 'antibodies' . Ua like kēia mau antibodies me nā hoʻomanaʻo. Ke maʻi ʻoe, hoʻomanaʻo ʻoe iā ia a mākaukau e hakakā inā hoʻi mai ia.

ʻO ka huaʻōlelo 'Combined' ma ka inoa ʻo SCID (Severe Combined Immunodeficiency) ʻo ia hoʻi, hoʻopilikia kēia maʻi i nā cell-T a me nā cell-B. ʻO ia ke kumu i kapa ʻia ai he hemahema 'combined'.

He kakaikahi loa kēia mau lymphocytes i loaʻa i ke keiki me SCID, a i ʻole ʻaʻole hana pono nā cell i loaʻa iā lākou. No laila, no ka mea ʻaʻole hana pono ka ʻōnaehana pale, he mea paʻakikī loa, i kekahi manawa hiki ʻole, ke hakakā i nā germs—nā virus, nā bacteria, a me nā fungi.

He aha ke kumu o SCID?

Aia kekahi mau ʻano like ʻole o SCID (Severe Combined Immunodeficiency).

ʻO ke ʻano maʻamau ka mea i hoʻokumu ʻia e kahi pilikia me kahi gene ma ka chromosome X. Hoʻopilikia wale kēia i nā keikikāne. No ka mea, ʻelua mau chromosome X o nā kaikamahine, ʻoiai inā he pilikia kekahi, hiki nō i kā lākou ʻōnaehana pale ke hana ma muli o ka chromosome X olakino ʻē aʻe. Akā hoʻokahi wale nō chromosome X o nā keikikāne. No laila, inā nāwaliwali kēlā gene, e ulu ka maʻi. Hiki i nā kaikamahine ke lilo i 'mea lawe' o ka maʻi. ʻO ia hoʻi, ʻoiai inā ʻaʻole lākou i loaʻa i ka maʻi, hiki i kā lākou mau keiki ke hoʻoili i ka gene.

Aia kekahi ʻano ʻē aʻe o SCID, i hoʻokumu ʻia e ka nele o kahi enzyme e pono ai no ka ulu ʻana o nā lymphocytes. Ma waho aʻe o kēia, hiki ke hoʻokumu ʻia ʻo SCID e nā kumu genetic like ʻole.

He aha nā hōʻailona o SCID? Pehea e ʻike ʻia ai?

ʻOiai paha e ʻike ʻia nā pēpē me SCID he olakino i ka wā hānau , e hoʻomaka paha nā pilikia e ulu aʻe i ka hala ʻana o ka manawa. Eia kekahi mau hōʻailona e makaʻala ai:

• ʻO ka holomua ʻole, ʻaʻole e piʻi mau ke kaumaha .
• Ka maʻi pākī mau .
• Nā maʻi hanu pinepine, i kekahi manawa koʻikoʻi . ʻO ke ʻano kēia o ka paʻa mau ʻana o ka umauma a me ke kuni ʻana.
• ʻO kahi maʻi fungal e hoʻoulu ai i nā ʻāpana keʻokeʻo ma ka waha a me ka ʻāʻī (oral thrush)Ke hele mai nei. Kāhea mākou i kēia ʻo 'Ullogam'.
• Eia kekahi, hiki ke loaʻa pinepine nā maʻi bacteria, viral, a fungal ʻē aʻe i paʻakikī ke mālama a hiki ke koʻikoʻi . Eia kekahi laʻana:
• Nā maʻi pepeiao (otitis media koʻikoʻi)
• Ka maʻi sinus (sinusitis)
• Nā ʻōhū ʻili
• Ka wela o ka lolo, ka maningitis
• Pneumonia

E noʻonoʻo i ka paʻakikī o nā mākua inā e maʻi mau ana kahi pēpē liʻiliʻi e like me kēia, a inā e hoʻomaikaʻi kekahi maʻi ma mua o ka hiki ʻana mai o kekahi. No laila, inā hoʻomau kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​​​pono ʻoe e ʻike koke i ke kauka.

Pehea e ʻike ʻia ai ka SCID (Severe Combined Immunodeficiency)?

ʻO ka mea pōmaikaʻi, ua hana ʻia kahi hoʻāʻo koko maʻalahi, i kapa ʻia ʻo 'newborn screening' , e nānā i kekahi mau maʻi i nā pēpē i ka wā e hānau ʻia ai . No ka laʻana, hiki ke ʻike ʻia ka 'sickle cell disease' a me ka 'cystic fibrosis' ma o kēia. Ke hana ʻia nei nā hoʻāʻo like ma Sri Lanka. ʻO ka nūhou maikaʻi, hiki ke ʻike ʻia ka SCID (Severe Combined Immunodeficiency) ma o kēia hoʻāʻo ʻana i nā pēpē hou ma kekahi mau ʻāina.

Ma kēia ʻano , ke ʻike mua ʻia ka maʻi, hiki ke hoʻomaka koke ka lāʻau lapaʻau. A laila ʻoi aku ka maikaʻi o nā hopena.

Inā hōʻike ka nānā ʻana i nā keiki hānau hou i ka SCID, kuhikuhi pinepine ʻia ke pēpē i kahi loea i nā hemahema o ka pale ʻana. E kauoha ke kauka i nā hoʻokolohua koko hou aʻe, a me ka hoʻāʻo ʻana paha i ka genetic .

Inā loaʻa i kekahi o ka ʻohana ka SCID, a inā he moʻolelo ʻohana no ka immunodeficiency, hiki i nā mākua ke loaʻa ka ʻōlelo aʻoaʻo genetic . Hiki iā lākou ke hana i nā hoʻāʻo koko i ka wā e hānau ai ka pēpē. ʻO ka wikiwiki o ka hana ʻana i ka hōʻailona, ​​ʻo ka wikiwiki o ka hoʻomaka ʻana o ka lāʻau a ʻoi aku ka maikaʻi o ka hopena.

I kekahi manawa, inā ʻike ʻia ka hoʻololi ʻana o ka gene e hoʻoulu ai i ka SCID i loko o kahi ʻohana, hiki ke hoʻāʻo i ka pēpē no ka maʻi i ka wā hānau . Eia nō naʻe, no nā pēpē me ka ʻole o ka mōʻaukala ʻohana a i ʻole ka poʻe i nānā ʻole ʻia ma o ka nānā ʻana i nā keiki hānau hou, hiki ke lohi a hiki i 6 mau mahina ma mua o ka ʻike ʻia ʻana o ka maʻi. I kēlā manawa, ua lohi loa.

He aha nā lāʻau lapaʻau no SCID?

He pilikia olakino pediatric ʻo SCID. ʻO ke ʻano kēia inā ʻaʻole mālama koke ʻia, ʻoi aku ka nui o ka make ʻana o kēia mau pēpē ma mua o ko lākou hiki ʻana i ko lākou lā hānau mua. No laila, he mea nui loa ka mālama koke ʻana.

ʻO ka lāʻau lapaʻau maʻamau ka 'stem cell transplant'. ʻO kēiaUa kapa ʻia hoʻi he 'bone marrow transplant', pili kēia i ka hāʻawi ʻana i nā cell kumu mai kahi kanaka olakino i kahi pēpē maʻi. ʻO ka manaʻolana e kūkulu hou kēia mau cell hou i ka ʻōnaehana pale o ka pēpē.

• Hiki ke hoʻokō ʻia ke kāpili ʻana o nā pūnaewele kumu holomua loa inā lawe ʻia nā pūnaewele mai kahi hoahānau i kūlike i ka genetics.
• I kekahi manawa, hiki ke kūlike nā pūnaewele o nā mākua.
• Inā ʻaʻohe mea o ka ʻohana e kūlike, hiki i nā kauka ke hoʻohana i nā cell kumu mai kahi mea hāʻawi pili ʻole.

Pono paha kekahi mau pēpē me SCID i ka chemotherapy ma mua o ka hoʻoili ʻana.

ʻO ka mea nui loa, ʻo ia ka hana ʻia ʻana o kēia hoʻoili ʻana o nā cell kumu i loko o nā mahina mua o ke ola o ka pēpē, ma mua o kona loaʻa ʻana o kekahi mau maʻi. ʻO ia ka manawa e kūleʻa loa ai nā hopena.

Ua hōʻike pū ka lāʻau lapaʻau gene i nā hopena hoʻohiki i nā hoʻokolohua lapaʻau no kekahi mau ʻano SCID. Eia nō naʻe, ʻaʻole i hoʻohana nui ʻia ma nā ʻāina he nui a puni ka honua. Ke hoʻomau nei ka noiʻi ʻana i ka lāʻau lapaʻau gene no SCID.

I ka mālama ʻana i kahi keiki me SCID, aia maʻamau kahi hui lapaʻau i hana ʻia e kekahi mau loea. Eia kekahi laʻana:

• Kauka pale ʻōnaehana keiki
• Kauka hoʻoili iwi iwi
• Loea maʻi lele o nā keiki

ʻOi aku ka nui o ka pilikia o nā pēpē me SCID i ka loaʻa ʻana o nā maʻi e hoʻoweliweli i ke ola. No laila, hoʻomaka nā kauka e hāʻawi iā lākou i nā lāʻau lapaʻau e kōkua i ka pale ʻana i nā maʻi. No ka laʻana, nā antibiotics, antivirals, antifungals , a me nā inikini antibody/immunoglobulin . ʻO kekahi mau pēpē, ma muli o ko lākou ʻano genetic, pono paha i nā infusions enzyme .

Nā mea hou aku e ʻike ai e pili ana iā SCID

Ma waho aʻe o ka lāʻau lapaʻau a me ka mālama ʻana, aia kekahi mau hana palekana ʻē aʻe āu e pono ai e hana e pale aku i ka maʻi. E hoʻomanaʻo i kēia mau mea i ka wā e mālama ai i kahi keiki me SCID:

• I mea e pale aku ai i ka laha ʻana o ka maʻi, pono ʻo ia e hoʻokaʻawale ʻia mai nā poʻe ʻē aʻe. ʻO ke ʻano kēia o ka hāʻawi ʻana iā ia i kahi lumi kaʻawale a me ka hōʻoia ʻana ua maʻemaʻe nā mea āna e hoʻopā ai.
• ʻAʻole kūpono ke hāʻawi ʻana i kekahi 'lāʻau pale ola'. ʻO kēia no ka mea he mea weliweli paha ke hāʻawi ʻana i kahi pēpē me SCID, ʻoiai he maʻi nāwaliwali, ma ke ʻano he lāʻau pale. Eia nā hiʻohiʻona o kēia:Lāʻau pale rotavirus, lāʻau pale moʻo moa/varicella, lāʻau pale measles-mumps-rubella (MMR), lāʻau pale polio waha, lāʻau pale BCG, a me nā lāʻau pale maʻi palū ola.
• ʻO ka mea nui loa, ʻaʻole pono i kekahi e noho like ana me ka pēpē ke loaʻa i kēia mau lāʻau pale ola, no ka mea, hiki iā lākou ke hoʻoili i ka maʻi i ka pēpē.
• Inā pono ka hoʻoili koko, pono e mālama kūikawā ʻia ke koko. Hana ʻia kēia e pale aku i nā pilikia e hiki mai ana mai nā hoʻoili koko.
• Pono paha ʻoe e hoʻōki i ka hānai waiū no kekahi manawa a hiki i ka hoʻāʻo ʻia ʻana o kou waiū umauma no nā maʻi e hiki ke hoʻoulu i nā maʻi. E hahai pono i nā ʻōlelo a kāu kauka.

Pehea lā e hiki ai iā ʻoukou ma ke ʻano he mau mākua ke kōkua?

ʻOiai e hoʻāʻo ʻia ana kāu pēpē no SCID, a i ka wā o ka mālama ʻana, nui nā mea hiki iā ʻoe ma ke ʻano he makua ke hana e hōʻemi i ka pilikia o ka maʻi. Eia kekahi o lākou:

• E kaupalena i ka nui o nā malihini i ka home. ʻOi aku ka maikaʻi o ka hoʻemi ʻana i ka poʻe e hele mai e ʻike i ke pēpē.
• Mai lawe i kāu pēpē i nā wahi lehulehu i piha i nā kānaka , e like me nā hale kūʻai kūʻai a me nā ʻahaʻaina.
• Mai lawe mai i kāu pēpē i kahi kokoke i kekahi mea i loaʻa i ke anu , ke kuni, a i ʻole ke kuni. Inā he kanaka e like me ia ma ka home, e mālama iā lākou mai kāu pēpē aku.
• Ma mua o ka hoʻopā ʻana i ka pēpē, e hōʻoia i ka holoi pono ʻana o nā mea a pau e mālama ana i ka pēpē i ko lākou mau lima. E holoi i kou mau lima me ke kopa a me ka wai e kahe ana no 20 kekona ma ka liʻiliʻi.

E noʻonoʻo kākou i ka wā e hiki mai ana.

Pono paha kahi pēpē me SCID e hana i nā kaʻina hana lapaʻau he nui a me ka noho pinepine ʻana i ka haukapila. Hiki i kēia ke lilo i mea hopohopo loa no kekahi ʻohana. Akā e hoʻomanaʻo ʻaʻole ʻoe hoʻokahi.

Aia ke kime lapaʻau ma laila e kōkua iā ʻoe a me kāu pēpē ma mua, i ka wā, a ma hope o ka mālama ʻana. Hiki iā ʻoe ke loaʻa ke kōkua a me ka hoʻoikaika mai nā hui kākoʻo, nā limahana hana kaiaulu, ka ʻohana, a me nā hoaaloha. Aia paha he mau hui ma Sri Lanka e kōkua ana i nā keiki me kēia maʻi, no laila e nānā iā lākou.

No ka ʻike hou aku a me ke kākoʻo ma ka pūnaewele, hiki iā ʻoe ke kipa i nā pūnaewele honua e like me kēia (aia kēia ma ka ʻōlelo Pelekania):

• Ka Papahana Hoʻomalu Palekana
• SCID, Nā ʻĀnela no ke Ola

ʻO nā mea nui loa hiki iā ʻoe ke lawe i ka home mai kēia ʻatikala

ʻAe, no laila, mai nā mea a mākou i kamaʻilio ai, eia nā mea nui loa e pono ai ʻoe e hoʻomanaʻo:

• He maʻi laha ʻole ka SCID (Severe Combined Immunodeficiency) akā he maʻi koʻikoʻi ia e hoʻopilikia ai i ka hana pono ʻana o ka ʻōnaehana pale o ke pēpē.
• Inā loaʻa iā ʻoe nā hōʻailona e like me nā maʻi koʻikoʻi pinepine, ka nele o ke kaumaha, a i ʻole ka maʻi palahēhē mau , e ʻimi koke i ke kōkua lapaʻau.
• Hiki i ka nānā ʻana i nā keiki hānau hou ke ʻike i ka SCID i kekahi manawa i ka wā mua.
• He mea nui loa ka ʻike mua ʻana a me ka mālama ʻana. ʻO ka lāʻau nui ka hoʻoili ʻana i nā cell kumu / ka hoʻoili ʻana i ka iwi.
• He mea nui loa ka pale ʻana i kahi pēpē me SCID mai nā maʻi, a pono e hana i nā hana palekana kūikawā.
• ʻAʻole ʻoe hoʻokahi. E kiʻi i ke kōkua mai nā kauka, ka ʻohana, a me nā hui kākoʻo.

Ke lana nei koʻu manaʻo he kōkua kēia ʻike iā ʻoe. Ke makemake nei au i ka wikiwiki o ka ho'ōla ʻana o kāu pēpē!

SCID , Palekana Koʻikoʻi i Hoʻohui ʻia, Palekana ʻole, Pediatrics, Nā Maʻi Hoʻoilina, Hoʻoili Iwi ʻIwi, Nānā ʻana i nā Keiki Hānau Hou