Ke ʻike pinepine nei ʻoe i nā ʻeha kino ʻē? E aʻo hou kākou e pili ana i kēia (Systemic Mastocytosis)!

Loaʻa iā ʻoe i kekahi manawa nā kiko ʻulaʻula a me ka maneʻo ma kou ʻili? A i ʻole he ʻeha ʻōpū a me nā poʻo ʻeha kou e hele wale mai a hele aku? Inā hana pinepine ʻia kēia mau mea, he maʻi laha ʻole paha ia i kapa ʻia ʻo "Systemic Mastocytosis" a mākou e kamaʻilio nei i kēia lā. Mai hopohopo, ʻaʻole kēia he maʻi e hoʻopilikia i nā poʻe he nui. Akā he mea nui e makaʻala i kēia.

No laila, he aha kēia "Systemic Mastocytosis"?

I ka ʻōlelo maʻalahi, ʻo ka `(Systemic Mastocytosis)` he maʻi laha ʻole e pili ana i ko kākou koko. ʻO ka mea e hana ʻia ma kēia, ʻo ia ka ulu ʻana o kahi ʻano cell kūikawā i kapa ʻia ʻo `(Mast cells)` i loko o ko kākou kino me ke ʻano ʻē. E noʻonoʻo, ua like kēia mau cell mast me nā koa liʻiliʻi i loko o ko kākou kino. ʻO lākou ka poʻe, ma ke ʻano he ʻāpana o kā kākou ʻōnaehana pale, e ʻike a hakakā me nā mea komo hewa mai nā ʻāina ʻē (e like me nā germs, allergens, etc.). Ke hiki mai kēlā mea komo hewa mai nā ʻāina ʻē, hoʻokuʻu kēia mau cell mast i nā kemika e like me `(Histamine)`. ʻO kēia `(Histamine)` ke kumu o kēlā mau hōʻailona allergy (e like me ka ʻulaʻula, ka maneʻo, ka pehu). ʻO ka maʻamau, hoʻōki nā cell mast i ka hoʻokuʻu ʻana i kēia `(Histamine)` ke pau ka hana. Eia naʻe, i loko o ke kino o ke kanaka me `(Systemic Mastocytosis)`, ulu nui kēia mau cell mast maʻamau a hoʻokuʻu mau i `(Histamine)`. Ua like ia me kekahi i waiho i kahi piula wai a laila poina e pani. ʻO ia ke kumu e loaʻa mau ai iā mākou nā hōʻailona allergy. Hiki iā ia ke hoʻopilikia ʻaʻole wale i ko mākou ʻili, akā i ko mākou mau kino kūloko e like me ko mākou ake, spleen, a me nā ʻōpū. ʻO kēia kūlana, i kapa ʻia ʻo "Systemic Mastocytosis," ua ʻōlelo ʻia e hoʻopilikia wale i 13 o kēlā me kēia 100,000 poʻe ma ka honua. ʻO ia hoʻi, he mea kakaikahi loa ia. Eia nō naʻe, ʻoi aku ka nui o ka pilikia o ka poʻe me kēia kūlana i ka anaphylaxis ( kahi hopena allergies koʻikoʻi, koke, a hoʻoweliweli i ke ola). He mea kakaikahi loa, hiki i ka "Systemic Mastocytosis" ke lilo i maʻi kanesa. ʻAʻole hiki i nā kauka ke ho'ōla piha i kēia kūlana. Eia nō naʻe, aia nā lāʻau lapaʻau e hiki ke kōkua iā ʻoe e kāohi i kāu mau hōʻailona a ola i ke ola maʻamau.

He aha nā ʻano nui o ka `(Systemic Mastocytosis)`?

ʻEono ʻano nui o ka "Systemic Mastocytosis". Hoʻopilikia lākou iā mākou ma nā ʻano like ʻole. Ma keʻano laulā, ʻo ka nui o nā cell mast ʻano ʻē i loko o kou kino, ʻo ka nui o ka hiki ke loaʻa iā ʻoe nā pilikia. E nānā kākou i kēlā mau ʻano: 1. ʻAno ulu lohi (ʻo ka "Indolent systemic mastocytosis"):

• ʻO kēia ke ʻano maʻamau. E like me ka lāʻau e ulu mālie ana, hiki ke hala nā makahiki no ka ʻike ʻia ʻana o nā hōʻailona. Hoʻonui ka nui o nā pūnaewele mast maʻamau ʻole i ka hala ʻana o ka manawa. Hiki i kēia ke hana i nā hōʻailona ma ka ʻili, ke akepaʻa , ka spleen , a me ka ʻōnaehana gastrointestinal (ʻo ia hoʻi, nā ʻōpū).

2. ʻAno holomua iki aʻe (`Systemic smoldering mastocytosis`):

• I kēia ʻano, hōʻiliʻili nui nā pūnaewele mast maʻamau ʻole i loko o ke akepaʻa a me ka spleen. I ka hala ʻana o ka manawa, hiki i kēia ke hoʻoulu i ke akepaʻa a i ʻole ka spleen e pehu. E noʻonoʻo e like me ka mea e hana ʻia ke piha loa kahi hale liʻiliʻi.

3. ʻAno e hele mai me nā maʻi koko ʻē aʻe (`Systemic mastocytosis with associated hematologic neoplasm`):

• Hiki i nā kānaka me kēia ʻano ke loaʻa i nā maʻi pili koko ʻē aʻe, e like me nā myeloproliferative neoplasms a me ka myelodysplastic syndrome . Hoʻopilikia kēia ʻano i kahi o hoʻokahi i loko o ʻelima mau kānaka me Systemic Mastocytosis.

4. ʻAno koʻikoʻi (`Mastocytosis ʻōnaehana hoʻouka kaua`):

• He ʻoi aku ka koʻikoʻi o kēia no ka mea hiki ke hoʻopilikia i ka iwi iwi (kahi i hana ʻia ai nā hunaola koko) a me nā iwi. Hiki i nā hunaola mast maʻamau ke ulu i loko o nā iwi, e hoʻonāwaliwali ana iā lākou a e haki maʻalahi ai.

5. Ke ʻano e lilo ai i leukemia (`Mast cell leukemia`):

• ʻAʻole pinepine loa, hiki i ka Systemic Mastocytosis ke ulu i kahi kūlana i kapa ʻia ʻo Mast Cell Leukemia . He ʻano kēia o ka Acute Myeloid Leukemia (AML) .

6. ʻAno o ka puʻupuʻu (`Mast cell sarcoma`):

• ʻO kēia ka wā e hōʻino ai nā puʻupuʻu (nā puʻupuʻu) i hoʻokumu ʻia mai nā pūnaewele mast maʻamau ʻole i nā ʻiʻo like ʻole o ke kino. He helu liʻiliʻi loa o ka poʻe me ka "Systemic Mastocytosis" e loaʻa ai kēia kūlana i kapa ʻia ʻo "Mast cell sarcoma".

He aha nā hōʻailona o kēia maʻi (Systemic Mastocytosis)?

Hiki ke ʻokoʻa nā hōʻailona mai kēlā kanaka a kēia kanaka. Aia ia i kahi o ke kino i nui loa ai nā pūnaewele mast ʻano ʻē. No ka laʻana, inā he nui nā pūnaewele mast i loko o kou ʻōpū, hiki iā ʻoe ke loaʻa nā ʻōpū a me ka ʻeha o ka ʻōpū. Eia kekahi, inā hōʻiliʻili nā pūnaewele mast i loko o kou iwi iwi, hiki iā ia ke hoʻopilikia i ka hana ʻana o nā pūnaewele koko. Eia kekahi mau hōʻailona maʻamau:

• Anemia ( ka nele o ke koko )
• ʻEha iwi
• Ke kahe nui ʻana o ke koko (e.g., nui ke koko mai kahi ʻeha liʻiliʻi)
• Luhi mau ka manaʻo (` Fatigue` )
• ʻIli ʻulaʻula
• Nā palpitations o ka puʻuwai
• ʻUʻuku (ʻili ʻālohilohi, piʻi aʻe nā puʻupuʻu ma ka ʻili)
• ʻIli ʻāʻī
• Nā loli o ke ʻano a i ʻole ke kaumahakaumaha
• Nā kiko ʻeleʻele a ʻānai hoʻi ma ka ʻili (`Urticaria pigmentosa`)

I kekahi manawa, hiki i ka poʻe me ka Systemic Mastocytosis ke ʻike i kekahi o kēia mau hōʻailona i ka manawa hoʻokahi. Ua kapa nā kauka i kēia he "mastocytosis attack" a i ʻole he "flare."

He mea nui: ʻAʻole nā ​​​​kānaka a pau i loaʻa i kēia mau hōʻailona he `(Systemic Mastocytosis).` Eia nō naʻe, inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona i ka manawa hoʻokahi, a hoʻomau lākou, pono ʻoe e ʻike i ke kauka no ka ʻōlelo aʻo.

No ke aha e hana ʻia ai kēia `(Systemic Mastocytosis)`?

Ua ʻike ʻia ma kahi o 80% o ka poʻe me ka `(Systemic Mastocytosis)` he `ʻano gene KIT` . Kōkua kēia gene `KIT` i kekahi mau ʻano cell i loko o ko mākou kino, ʻoiai nā cell koko a me nā cell mast, e ulu. Loaʻa kēia ʻano gene ma hope o ko mākou hāpai ʻia ʻana i loko o ka ʻōpū. ʻO ke ʻano kēia, ʻaʻole ia he mea hoʻoilina.

He aha nā mea e hoʻonui ai a hoʻonāukiuki paha i ka maʻi ('Triggers')?

Hiki i nā mea he nui ke hoʻoulu i nā hōʻailona o ka "Systemic Mastocytosis." ʻAʻole like nā mea a pau i ka hopena. Eia nō naʻe, aia kekahi mau mea maʻamau:

• ʻAila
• Nā meaʻai ʻala
• ʻO kekahi mau lāʻau lapaʻau: No ka laʻana , NSAID ( nā lāʻau ʻeha), nā mea hoʻomaha ʻiʻo, a me ka anesthesia.
• Hoʻoikaika kino a me ka hana kino ( no kekahi wale nō)
• Nā nahu pepeke (ʻoi aku hoʻi nā ʻanuʻu, nā nalo meli, a me nā pi)
• Ke kaumaha kino a i ʻole ka naʻau
• Ka hamo ʻana a i ʻole ka ʻūlū ʻana ma kou ʻili
• Nā loli koke o ka mahana (e.g., lele koke i loko o ka wai anuanu)

Inā loaʻa iā ʻoe kēia maʻi, he mea nui loa e nīnau i kāu kauka i kou mau nāwaliwali a me nā mea āu e pale aku ai.

Pehea ʻoe e ʻike pololei ai i kēia maʻi? (Diagnosis)

Ke hele ʻoe e ʻike i ke kauka, ʻo ka mea mua a lākou e hana ai, ʻo ia ke hāʻawi iā ʻoe i kahi hoʻokolokolo kino. E nīnau lākou e pili ana i kāu mau hōʻailona a me nā maʻi āu i loaʻa ai ma mua. A laila, hiki iā lākou ke hana i hoʻokahi a ʻoi aku paha o nā hoʻokolohua aʻe e hoʻoholo ai inā he Systemic Mastocytosis kou:

• Nā hoʻāʻo koko: Nānā kēia i ka nui o ka tryptase i loko o kou koko. He enzyme kūikawā ka tryptase a nā pūnaewele mast e hoʻokuʻu ai ke pane lākou i nā mea ʻē.
• Nā scan iwi: E nānā no ka pohō o ka iwi.
• Biopsy iwi ʻiwi: Pili kēia i ka lawe ʻana i kahi hāpana liʻiliʻi o kou iwi ʻiwi a hoʻāʻo iā ia e ʻike inā he mau cell mast ʻē aʻe i loko, a ehia.
• Nā hoʻāʻo hoʻoilina: E nānā no kahi mutation i loko o ka gene `KIT` i ʻōlelo mua ʻia.

Pehea e mālama ʻia ai?

Hoʻōla nā kauka i ka Systemic Mastocytosis ma o ka kaohi ʻana i nā hōʻailona a me nā pilikia. No ka laʻana, inā he kiʻekiʻe kou waikawa ʻōpū, hiki iā lākou ke kuhikuhi i nā mea pale H2 (kahi ʻano antacid). Inā he hopena ka anemia o ka maʻi, e mālama ʻia. Eia kekahi o nā lāʻau lapaʻau nui:

• Nā Antihistamine: E hōʻemi i nā hōʻailona ʻili e like me ka maneʻo a me ka ʻulaʻula.
• Corticosteroids: Hoʻēmi i ka pehu a me ka mumū i loko o ke kino.
• Nā Bisphosphonates: Kōkua lākou i ka hoʻoikaika ʻana i nā iwi inā nāwaliwali lākou.
• ʻO ka lāʻau lapaʻau i hoʻolālā ʻia: Pili kēia i ka hoʻolālā ʻana i kahi protein kikoʻī i loaʻa i loko o nā pūnaewele mast maʻamau.
• Chemotherapy: Hoʻohana wale ʻia kēia inā ua lilo ka Systemic Mastocytosis i ka maʻi kanesa.
• Splenectomy: Inā pehu nui ka spleen, hiki ke wehe ʻia ma ke ʻoki ʻana.
• Hoʻoili iwi iwi: Hana ʻia kēia no ka poʻe me ka maʻi koʻikoʻi loa, a i ʻole ka poʻe i nā pae hope loa.

E hoʻomanaʻo: Inā loaʻa iā ʻoe ka Systemic Mastocytosis, pono ʻoe e lawe mau i kahi EpiPen . ʻO ka EpiPen kahi lāʻau lapaʻau hiki ke hoʻohana koke ʻia inā loaʻa kahi hopena alerika koʻikoʻi, e like me ka Anaphylaxis i ʻōlelo ʻia ma luna.

ʻAʻole hiki ke pale ʻia kēia "Systemic Mastocytosis"?

E like me kā mākou i kūkākūkā ai ma mua, ʻo ka "Systemic Mastocytosis" ke kumu o kahi hoʻololi genetic. No laila, ʻaʻole hiki ke pale aku i kona ulu ʻana. Eia nō naʻe, hiki iā ʻoe ke kāohi i ka maʻi ma ka pale ʻana i nā "mea hoʻoulu" e hoʻonui ai i nā hōʻailona.

He aha ke ʻano o ka wā e hiki mai ana no kekahi me ka `(Systemic Mastocytosis)`?

Aia maoli kēia i ke ʻano o ka `(Systemic Mastocytosis)` āu e loaʻa ai. Hiki i ka poʻe me ke ʻano ulu lohi (`Indolent systemic mastocytosis`) ke kāohi maikaʻi i ka maʻi me ka lāʻau lapaʻau. Hiki iā lākou ke ola i kahi ola maʻamau. Eia nō naʻe, ʻoi aku ka pōkole o ke ola o ka poʻe me nā ʻano koʻikoʻi ʻē aʻe. Eia nō naʻe, hiki ke ho'ōla loa ʻia ka `(Systemic Mastocytosis)` me kahi lāʻau i kapa ʻia ʻo ``Bone marrow transplant``. Eia naʻe, hāʻawi wale nā ​​​​kauka i kēlā lāʻau lapaʻau i ka poʻe i nā pae koʻikoʻi loa o ka maʻi.

Pehea wau e mālama ai iaʻu iho i koʻu noho ʻana me ka `(Systemic Mastocytosis)`?

ʻO ka ʻike i nā mea e hoʻoulu ai i kāu mau hōʻailona a me ka pale ʻana iā lākou ka mea nui loa hiki iā ʻoe ke hana i ka wā e noho ana me ka Systemic Mastocytosis. Inā loaʻa iā ʻoe kēia maʻi, e makaʻala i kēia mau mea:

• E lawe mau i kahi EpiPen me ʻoe. E mālama koke i kahi maʻi alerika koʻikoʻi.
• E ʻike pono i kāu mau mea hoʻonāukiuki a e pale iā lākou i ka hiki.
• E hoʻokele i ke kaumaha. E hoʻomaʻamaʻa i ka noʻonoʻo ʻana a i ʻole nā ​​​​ʻano hana noʻonoʻo ʻē aʻe.
• E komo i kahi kūpeʻe lima makaʻala lapaʻau e papa inoa ana i nā lāʻau lapaʻau hiki ʻole iā ʻoe ke lawe. He mea nui loa kēia no ka mea kōkua ia i nā kauka e ʻike i kou kūlana inā ʻaʻole hiki iā ʻoe ke ʻōlelo i kahi pilikia.

Āhea wau e ʻike ai i ke kauka?

Inā loaʻa iā ʻoe ka Systemic Mastocytosis, pono ʻoe e ʻike i kāu kauka no nā nānā maʻamau. Eia kekahi, e hōʻoia e haʻi i kāu kauka inā loaʻa iā ʻoe kekahi mau hōʻailona hou, a inā paha e ʻoi aku ka ʻino o kekahi o kāu mau hōʻailona.

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

No ka mea, he maʻi laha ʻole ka Systemic Mastocytosis, nui paha kāu mau nīnau. Eia kekahi mau nīnau e nīnau aku ai i kāu kauka:

• He aha ke ʻano o ka "Systemic Mastocytosis" i loaʻa iaʻu?
• He aha nā hōʻailona ʻē aʻe e hōʻike ana e ʻoi aku ka ʻino o koʻu kūlana?
• He aha nā lāʻau lapaʻau i loaʻa no koʻu ʻano?
• E hoʻopau loa anei ka lāʻau lapaʻau i koʻu mau hōʻailona?
• Aia kekahi mau hoʻokolohua lapaʻau e hiki iaʻu ke komo?

ʻO ka ʻike ʻia ʻana me ka Systemic Mastocytosis he ʻike hoʻololi ola. Pono ʻoe e ʻike pinepine i kāu kauka, e hana i nā hoʻāʻo koko, a e aʻo e ʻike a pale i nā mea hoʻonāukiuki. ʻO ka noho ʻana me kēia maʻi he hoʻololi nui ia. No laila, e mālama i ka pilina maikaʻi me kāu kauka. Aia lākou ma laila e kōkua iā ʻoe. E hāʻawi lākou iā ʻoe i ka ʻike a me nā hui kākoʻo e hiki ke kōkua iā ʻoe. E hoʻoikaika kēia iā ʻoe e hoʻoholo i kou olakino.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)

ʻAe, ua kamaʻilio nui kākou e pili ana i ka `(Systemic Mastocytosis)`, ʻeā? E hoʻomanaʻo, ʻoiai he maʻi laha ʻole kēia, he mea nui loa ia e makaʻala.

ʻO ka mea nui loa, ʻo ia ke ʻike ʻana i ke kauka inā loaʻa iā ʻoe kēia mau hōʻailona. Inā ʻike mua ʻia ka maʻi, hiki ke hoʻomaka ʻia ka lāʻau lapaʻau a hiki ke kaohi ʻia nā hōʻailona.

ʻAʻole ʻoe hoʻokahi. Aia kāu mau kauka, ʻohana, a me nā hoaaloha e kōkua iā ʻoe. Ma ka ʻike ʻana i ka ʻike kūpono a me ka hahai ʻana i nā ʻōlelo aʻoaʻo a kāu kauka, hiki iā ʻoe ke ola maikaʻi me kēia maʻi. E noho mālie a noʻonoʻo maikaʻi!