Ka Hāʻule ʻana o ka Iwi Moa: E kamaʻilio kākou e pili ana i kēia me nā kikoʻī!

ʻO ka iwi iwi ke kikowaena hana koko nui i loko o kou kino. Ua like ia me kahi hale hana. Hana kēia hale hana i nā mea e like me nā hunaola koko ʻulaʻula, nā hunaola koko keʻokeʻo, a me nā platelets e kōkua i ko mākou kino e hoʻopaʻa. E noʻonoʻo i ka mea e hana ʻia inā e pau koke ka hana a kēia hale hana, a inā ʻaʻole ia e hana pono? ʻO ia ke ʻano o ka hāʻule ʻana o ka iwi iwi. E kamaʻilio kākou e pili ana i kēia me nā kikoʻī hou aku.

He aha ka hemahema o ka iwi iwi?

I ka ʻōlelo maʻalahi, ʻo ka hāʻule ʻana o ka iwi iwi ke hiki ʻole i kou iwi iwi ke hana i nā hunaola koko ʻulaʻula olakino, nā hunaola koko keʻokeʻo, a i ʻole nā ​​platelets no ke kino. Loaʻa i kēlā me kēia ʻano pūnaewele kahi kuleana kūikawā i loko o ko mākou kino.

• ʻO nā hunaola koko ʻulaʻula ka mea e lawe i ka oxygen ma ke kino holoʻokoʻa. Inā haʻahaʻa lākou, e ʻike paha ʻoe i ka luhi a me ka mae.
• Ua like nā hunaola koko keʻokeʻo me ka pūʻali koa i loko o ko kākou kino. Ke kaua nei lākou i nā maʻi. Inā haʻahaʻa lākou, kiʻekiʻe ka manawa e maʻi ai.
• Kōkua nā platelets i ke kāpili ʻana o ke koko, ʻo ia hoʻi ke hoʻōki nei lākou i ke kahe ʻana o ke koko ke ʻeha ʻoe. Inā hāʻule kāu helu platelet, hiki i kahi ʻeha liʻiliʻi ke hoʻoulu i ka nui o ke kahe ʻana o ke koko.

ʻO ka hāʻule ʻana o ka iwi iwi he pilikia pinepine ia o kekahi maʻi olakino ʻē aʻe. Akā i kekahi manawa hiki ke hana me ke kumu ʻole. Hiki i nā kauka ke hoʻokele i nā hōʻailona me nā lāʻau lapaʻau a me nā ʻano lāʻau like ʻole. Eia nō naʻe, ʻo ka lāʻau lōʻihi wale nō no kēia maʻi he allogeneic stem cell transplant .

He aha nā ʻano nui o kēia maʻi?

ʻElua ʻano nui o ka hāʻule ʻana o ka iwi iwi:

1. Loaʻa: ʻAʻole maopopo i nā loea ke kumu o kēia ʻano. Akā ua hōʻike ʻia e ka noiʻi hiki ke hoʻoulu ʻia e kekahi mau maʻi, ka ʻike ʻana i kekahi mau kemika, a i ʻole ka hoʻohana ʻana i kekahi mau lāʻau lapaʻau. Hoʻomohala mālie kēia ʻano i ka hala ʻana o ka manawa.

2. Hoʻoilina: Hana ʻia kēia inā hoʻoilina ʻoe i nā loli genetic (mutations) mai kekahi a i ʻole ʻelua o kou mau mākua. Kāhea nā kauka i kēia he Bone Marrow Failure Syndrome .

Pehea ka maʻamau o kēia ʻano?

ʻAʻole kēia he maʻi maʻamau loa. No ka laʻana, ma kahi o 65 o kēlā me kēia miliona pēpē i hānau ʻia ma ʻAmelika Hui Pū ʻIa i kēlā me kēia makahiki he maʻi palupalu iwi hoʻoilina. ʻO kekahi laʻana he maʻi hoʻoilina i kapa ʻia ʻo "Fanconi Anemia." Hoʻopilikia ia i hoʻokahi o kēlā me kēia miliona. No laila hiki iā ʻoe ke ʻike, he mea kakaikahi kēia.

He aha nā hōʻailona?

Hiki ke ʻokoʻa nā hōʻailona ma muli o ke kumu o ka hāʻule ʻana o ka iwi. No ka laʻana, hiki i kekahi me ka hāʻule ʻana o ka iwi hoʻoilina ke hoʻomaka e hōʻike i nā hōʻailona i ka wā ʻelua o kona mau makahiki. Eia nō naʻe, hōʻike ka noiʻi e hiki i kekahi me ke ʻano i loaʻa o ke ʻano ke hoʻomaka e hōʻike i nā hōʻailona i ka wā ma waena o nā makahiki 20 a me 25, a i ʻole a hiki i ka 65. Me ka nānā ʻole i ka wā e hoʻomaka ai, ʻo nā hōʻailona maʻamau:

• ʻEha i nā iwi
• Ke kahe koko nui (e like me ke kahe koko o ka ihu, nā ʻāʻī koko, ke kahe koko nui o ka menstruation)
• Luhi nui a me ka paupauaho
• Kuni pinepine
• Nā maʻi bacteria pinepine
• ʻEha poʻo
• Ka paʻakikī o ka hanu ʻana (Dyspnea)
• Ke lilo nei ke kala o ka ʻili i ʻoi aku ka mae ma mua o ka maʻamau
• Nā kiko ʻulaʻula liʻiliʻi ma lalo o ka ʻili (Petechiae)
• ʻO ka palapū ma ke kino me ke kumu ʻike ʻole ʻia

Inā hoʻomau ʻoe i kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​e ʻike pono i ke kauka.

No ke aha i hāʻule ai kēia iwi iwi? He aha nā kumu?

Hiki i nā mea aʻe ke kōkua i kou pilikia o ka hoʻomohala ʻana i ka hāʻule ʻana o ka iwi iwi:

• Loaʻa iā ʻoe ka Bone Marrow Failure Syndrome (he maʻi hoʻoilina ia).
• Loaʻa iā ʻoe ke kanesa koko, kekahi maʻi koko ʻē aʻe, a i ʻole kekahi mau maʻi koko, nā kanesa, a i ʻole nā ​​maʻi (ʻo kēia mau mea ma hope aku, ʻo ia hoʻi, no ke ʻano `(Loaʻa)`).
• Ka ʻike ʻia ʻana i kekahi mau kemika a me nā lāʻau lapaʻau (hāʻule pū kēia ma lalo o ka māhele `(Loaʻa)`).

I kekahi manawa, hiki ke kū mai kēia ʻano me ke kumu ʻole. Ua kapa pū nā kauka iā ia he " idiopathic bone marrow failure." Manaʻo nā kānaka noiʻi aia kahi pilina ma waena o ka autoimmunity a me ka iwi marrow failure. ʻO ka Autoimmunity ka wā e hoʻouka hewa ai ka ʻōnaehana pale ponoʻī o kou kino i kou iwi.

Nā Syndrome Hoʻoilina o ka Iwi Moa

Eia kekahi mau kūlana i hoʻoilina ʻia, pili, a i ʻole he mau hōʻailona like:

• ʻO ka Agranulocytosis Congenital (Kostmann Syndrome): Hoʻopilikia kēia i kahi ʻano koko keʻokeʻo i kapa ʻia ʻo neutrophils.
• ʻO ka Congenital Amegakaryocytic Thrombocytopenia (CAMT): Ma kēia ʻano, aia ka emi ʻana o ka helu o nā pūnaewele i kapa ʻia he megakaryocytes, e kōkua ana i ka hana ʻana i nā platelets, i loko o kou iwi iwi.
• ʻO Diamond-Blackfan Anemia (Diamond-Blackfan Anemia): He maʻi mau kēia e hoʻopilikia ai i ka hana ʻana o nā hunaola koko ʻulaʻula.
• Dyskeratosis Congenita (Dyskeratosis Congenita - `(Dyskeratosis Congenita)`):ʻO nā hōʻailona o kēia ʻano e pili ana i nā loli o ka ʻili, hiki ke lilo i hōʻailona mua o ka hāʻule ʻana o ka iwi iwi.
• ʻO Fanconi Anemia (Fanconi Anemia): ʻO kēia ka maʻi hāʻule ʻana o ka iwi iwi maʻamau.
• ʻO Reticular Dysgenesis (Reticular Dysgenesis): He ʻano kēia o ka Severe Combined Immunodeficiency (SCID). Ma kēia kūlana, ʻaʻole hana kou kino i nā cell T makua lawa.
• ʻO ka Neutropenia Hānau Koʻikoʻi (Severe Congenital Neutropenia): Ma kēia kūlana, ʻoi aku ka haʻahaʻa o kāu mau neutrophils, kahi ʻano koko keʻokeʻo, ma mua o ka maʻamau.
• ʻO Shwachman-Diamond Syndrome (Shwachman-Diamond Syndrome): Hoʻopilikia kēia maʻi i kou pancreas, iwi ʻiʻo, a me nā iwi.

Inā loaʻa i kekahi o kou mau mākua ke ʻano a me nā hōʻailona (ua kapa ʻia kēia he "Autosomal Dominant"), he 50% kou manawa e loaʻa ai ke ʻano a me ka hoʻomohala ʻana i ka bone marrow failure syndrome.

Eia nō naʻe, inā loaʻa i kou mau mākua ʻelua kahi hoʻololi gene e pili ana i ke ʻano like o ka bone marrow failure syndrome, akā ʻaʻohe o lāua i loaʻa nā hōʻailona (ua kapa ʻia kēia ʻo "Autosomal Recessive"), ua emi kou pilikia o ka loaʻa ʻana o ke ʻano i 25%.

Nā maʻi ʻaʻai koko

Aia ʻoe i ka pilikia kiʻekiʻe o ka hāʻule ʻana o ka iwi iwi inā loaʻa iā ʻoe kekahi o kēia mau ʻano maʻi kanesa:

• ʻO ka maʻi ʻōpū
• ʻO ka Myeloma lehulehu
• ʻO ka maʻi Myelodysplastic

Nā maʻi koko ʻē aʻe

Ua hānau ʻia kekahi poʻe me kēia mau kūlana. Loaʻa i kekahi poʻe i ka hala ʻana o ka manawa. ʻO kēia mau mea:

• ʻAnemia Aplastic (Aplastic Anemia): He maʻi koko laha ʻole kēia. Hoʻopōʻino ia i kou iwi iwi a pale iā ia mai ka hana ʻana i nā hunaola koko hou a me nā platelets. Hoʻohana paha nā kauka i ka huaʻōlelo "anemia aplastic" ke kamaʻilio lākou e pili ana i ka hāʻule ʻana o ka iwi iwi.
• Cytopenias (Cytopenias): ʻO kēia nā kūlana e like me ka neutropenia autoimmune, neutropenia idiopathic , a me ka leukemia lymphocyte granular nui.
• Paroxysmal Nocturnal Hemoglobinuria (Paroxysmal Nocturnal Hemoglobinuria): Ma kēia ʻano, hoʻopau kāu ʻōnaehana pale i nā hunaola koko ʻulaʻula.
• ʻAplasia Kelepona ʻUlaʻula Maʻemaʻe (Aplasia Kelepona ʻUlaʻula Maʻemaʻe): He ʻano hoʻoilina nō hoʻi kēia e hoʻopilikia ai i ka hana ʻana o nā kele koko ʻulaʻula.

Nā maʻi viral

Hiki i nā maʻi viral ma lalo nei ke hoʻonui i kou pilikia:

• ʻO ka maʻi Cytomegalovirus (Cytomegalovirus)
• ʻO ka maʻi Epstein-Barr
• Ka maʻi pale ʻole kanaka (HIV)
• ʻO ka maʻi Parvovirus B19 `(Parvovirus B19)`
• Hepatitis viral

Hiki i ka chemotherapy a me/a i ʻole ka radiation therapy no ka maʻi ʻaʻai ke hoʻonui i kou pilikia o ka hāʻule ʻana o ka iwi iwi. Eia kekahi, ʻo ka ʻike ʻana i nā kemika a me nā mea hoʻoheheʻe i hoʻohana ʻia i loko o kekahi mau insecticides a me nā pesticides hiki ke hoʻonui i kou pilikia.

He aha nā pilikia o kēia maʻi?

He kūlana koʻikoʻi kēia e hiki ke hoʻoweliweli i ke ola . ʻOiai ma hope o ka mālama ʻana, hiki ke loaʻa nā pilikia penei:

• Nā maʻi bacteria a viral paha
• Ke kahe ʻana o ke koko
• ʻO ke kanesa koko, no ka laʻana, ka leukemia myeloid acute a i ʻole ka myelodysplasia
• ʻO nā maʻi ʻaʻai ʻē aʻe, e like me ka osteosarcoma a me ka rhabdomyosarcoma
• ʻO ke kanesa o ke kelepona Squamous

Pehea ʻoe e ʻike ai i kēia?

E nīnau mua ke kauka iā ʻoe e pili ana i kāu mau hōʻailona, ​​​​kou mōʻaukala olakino, a me ka mōʻaukala olakino o kou ʻohana. E hana pū lākou i kahi hoʻokolokolo kino. Hiki iā lākou ke kauoha i nā hoʻokolohua koko a me nā hoʻokolohua kiʻi.

Nā hoʻāʻo koko

Hiki i nā hoʻokolohua koko ke komo pū me:

• Hoʻāʻo Ceruloplasmin
• Helu Koko Piha (CBC)
• Hoʻāʻo Ferritin
• Hoʻāʻo no ka nele o ka Folate
• Hoʻāʻo no ka nele o ka wikamina B12

Nā Hoʻāʻo Kiʻi

Hiki i nā hoʻokolohua kiʻi ke komo pū me:

• Ke kiʻi ʻana i ka Magnetic Resonance Imaging (MRI - `(Magnetic Resonance Imaging)` scan)
• ʻO ka Tomography Hoʻokuʻu Positron (PET - scan `(Positron Emission Tomography)`)
• Hoʻokolokolo Ultrasound `(Ultrasound)`

Ma muli o nā hopena o nā hoʻāʻo koko a me nā hoʻāʻo kiʻi, hiki i kāu kauka ke ʻōlelo aku i kahi biopsy iwi . Hiki iā ia ke ʻōlelo aku i ka hoʻāʻo genetic e ʻike i nā hoʻololi genetic e hoʻoulu ai i ka hāʻule ʻana o ka iwi.

Hiki i kāu kauka ke kauoha i nā hoʻokolohua e ʻike i kekahi mau maʻi a i ʻole e kāpae i nā kūlana ʻē aʻe. E wehewehe lākou iā ʻoe i ke kumu e hana ai lākou i kēlā me kēia hoʻokolohua a me nā mea e hiki i nā hoʻokolohua ke hōʻike.

He aha nā lāʻau lapaʻau?

ʻO ka lāʻau āu e loaʻa ai e hilinaʻi ia ma luna o kekahi mau kumu, me:

• ʻO ke ʻano o ka hāʻule ʻana o ka iwi iwi (ʻo ia hoʻi inā he hoʻoilina a loaʻa paha).
• Ke koʻikoʻi o kou kūlana.
• Kou makahiki a me kou olakino holoʻokoʻa.
• Kou mau hōʻailona.

Hiki i nā koho lapaʻau ke komo pū me:

• Nā lāʻau antibiotic , antifungals, a me nā lāʻau antiviral e hakakā ai i nā maʻi.
• Hoʻoili koko e hoʻonui i ka helu o nā hunaola koko ʻulaʻula a hōʻemi i nā hōʻailona e like me ke kahe ʻana o ke koko a me ka luhi.
• Kōkua nā mea hoʻouluulu iwi i kou iwi e hana i nā hunaola koko hou aku.
• Kōkua nā Immunosuppressants i ka pale ʻana i kāu ʻōnaehana pale mai ka hoʻouka ʻana i kou iwi iwi.
• Hoʻoili Kelepona Kumu Allogeneic (Iwi Maloko) .

Aia kekahi mau hopena ʻaoʻao a i ʻole nā ​​​​pilikia o ka lāʻau lapaʻau? (Nā pilikia o ka lāʻau lapaʻau)

ʻOkoʻa nā pilikia a me nā hopena ʻaoʻao ma muli o ka lāʻau lapaʻau, akā ʻo nā hoʻoili ʻana o nā cell stem (iwi iwi) ka mea e hoʻoulu ai i nā pilikia koʻikoʻi. ʻO kēia mau mea e komo pū me ka maʻi graft-versus-host (GVHD) a me nā maʻi.

Pehea lā ke ʻano o ke ola me kēia maʻi? (Wānana)

ʻO ka mea hiki iā ʻoe ke manaʻo ma hope o ka mālama ʻana (prognosis) e pili ana i kekahi mau kumu, me ke ʻano o ka maʻi, kou makahiki a me ke olakino holoʻokoʻa, a pehea e pane ai kou kino i ka mālama ʻana.

ʻO ka maʻamau, pono ka poʻe me ka hāʻule ʻana o ka iwi iwi i ka mālama olakino a me ke kākoʻo . Akā, ʻaʻole like nā kānaka a pau. E ʻike pono i kou kūlana, a e nīnau i kāu kauka i ka mea e hana ʻia ma hope o kāu mālama mua ʻana.

He maʻi make anei ka hāʻule ʻana o ka iwi iwi? (He maʻi make anei?)

He kūlana koʻikoʻi loa kēia a hoʻoweliweli i ke ola . ʻO ka hapa nui o ka poʻe me ka hāʻule ʻana o ka iwi iwi e mālama ʻia e nā loea akamai e like me nā oncologists a me nā hematologists.

He aha ka mea hiki ke ʻōlelo ʻia e pili ana i ke ola o kēia mau maʻi? (Ke ola o ka poʻe)

ʻO ke anamanaʻo ola he kuhi ia o ka lōʻihi o ke ola o kekahi ma hope o ka mālama ʻia ʻana no kekahi ʻano maʻi. Hiki ke loli ke anamanaʻo ola o ka poʻe me ka hāʻule ʻana o ka iwi mai kekahi mau mahina a i ke ola holoʻokoʻa.

Akā, he kūikawā ʻoe, a ʻokoʻa paha kou ʻike mai nā mea ʻē aʻe. E nīnau i kāu kauka e pili ana i nā mea hiki iā ʻoe ke manaʻo, a me ka lōʻihi o kou ola ʻana. ʻO lākou kou kumu ʻike maikaʻi loa, no ka mea, ua ʻike lākou iā ʻoe a me kou kūlana.

Hiki ke hoʻemi ʻia ka pilikia? (Hoʻemi i ka pilikia)

ʻO ka mea pōʻino, ʻaʻohe ala e hōʻemi ai i ka pilikia o ka hāʻule ʻana o ka iwi iwi i hoʻoilina ʻia. Eia nō naʻe, ʻo ka pale ʻana i nā kemika i ʻike ʻia e hōʻemi i ka hana o ka iwi iwi hiki ke hōʻemi i ka pilikia o kekahi mau hāʻule ʻana o ka iwi iwi i loaʻa. Eia kekahi, hiki i ka mālama mua ke kōkua i ka hōʻoluʻolu ʻana i kāu mau hōʻailona a hoʻomaikaʻi i kou ʻano ola.

Pehea e mālama ai iaʻu iho? (Mālama ponoʻī)

ʻO ka mea maikaʻi loa e hana ai, ʻo ia ka mālama ʻana i kou olakino holoʻokoʻa . Eia kekahi laʻana, mai hoʻohana i nā huahana paka, mai kaupalena a hoʻōki paha i ka inu ʻana i nā mea inu ʻona. Eia kekahi mau manaʻo ʻē aʻe:

• E ʻike pinepine i kāu kauka. Hiki i ka hāʻule ʻana o ka iwi iwi ke lilo i maʻi no ke ola holoʻokoʻa. Pono paha ʻoe i ka mālama mau ʻana. Hiki iā ia ke hana i nā pilikia olakino hou. No laila he mea nui e hana i nā nānā mau. E nānā kāu kauka i kou olakino holoʻokoʻa a mālama i nā pilikia hou.
• E ʻai i ka meaʻai olakino. ʻO kāu mau hōʻailona a me nā hopena ʻaoʻao mai kāu lāʻau lapaʻau e paʻakikī ai iā ʻoe ke ʻai a nalowale paha kou makemake. Inā ʻaʻole ʻoe maopopo inā loaʻa iā ʻoe ka meaʻai āu e pono ai, e kamaʻilio me kahi meaʻai meaʻai. Hiki iā lākou ke hāʻawi iā ʻoe i nā ʻōlelo aʻoaʻo kūpono.
• E hoʻoikaika kino. ʻO ka loaʻa ʻana o kahi maʻi olakino koʻikoʻi e like me ka hāʻule ʻana o ka iwi iwi hiki ke lilo i mea kaumaha. He ala maikaʻi loa ka hoʻoikaika kino e hōʻoluʻolu ai i ke kaumaha. E kamaʻilio me kāu kauka ma mua o ka hoʻomaka ʻana i kahi hana hoʻoikaika kino hou.

I ka manawa hea ʻoe e pono ai e hele i ka Emergency Treatment Unit (ETU) ?

Inā loaʻa iā ʻoe ka hemahema o ka iwi iwi, ʻoi aku kou pilikia o ka loaʻa ʻana o nā maʻi a me nā pilikia koko. Pono ʻoe e hele i ka lumi pilikia inā loaʻa iā ʻoe kekahi o kēia mau mea:

• Loaʻa i ke kuni ʻaʻole e iho i lalo ma hope o ka lawe ʻana i kahi lāʻau ʻeha maʻamau
• Ke anu nei a me ka haʻalulu (Chills)
• Ke kahe koko ʻole e hiki ke hoʻōki

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

Loaʻa paha iā ʻoe ka hemahema o ka iwi ma muli o kekahi mau maʻi hoʻoilina. Akā i kekahi manawa hiki ke hana ʻia me ke kumu ʻole. Hiki iā ʻoe ke nīnau i kāu kauka i nā nīnau e like me kēia:

• Ua ʻike anei ʻoe i ke kumu o kēia hemahema o ka iwi?
• He aha nā koho lapaʻau e loaʻa iaʻu?
• Pono anei au e lawe i ka lāʻau lapaʻau no ka wā pau ʻole?
• Hiki ke ho'ōla ʻia kēia?

Inā loaʻa iā ʻoe ka hemahema o ka iwi iwi, aia paha ʻoe i kahi pilikia olakino koʻikoʻi, e like me kahi maʻi hoʻoilina, kahi maʻi koko, a i ʻole ke kanesa. Hiki i kēia hōʻailona hou ke kumu iā ʻoe i nā nīnau a me nā hopohopo hou he nui, hiki ke hoʻoluhi. He mea maʻamau ka hopohopo no ka mea hiki i kēia ke lilo i mea hoʻoweliweli i ke ola. E kamaʻilio me kāu hui lapaʻau e pili ana i ka mea e manaʻo ai a me ka mea e hana ai ma hope. E hauʻoli lākou e pane i kāu mau nīnau.

E hoʻomanaʻo ma ke ʻano he hōʻuluʻulu manaʻo (Leka Lawe-Home)

He kūlana koʻikoʻi a paʻakikī ka hāʻule ʻana o ka iwi iwi. Akā e hoʻomanaʻo, he nui nā kumu hiki ke loaʻa, a he nui nā ʻano lapaʻau like ʻole a me nā hoʻolālā hoʻokele. ʻO ka mea nui loa, ʻo ia ka loaʻa ʻana o kahi hōʻailona ma mua, e ʻimi i ka lāʻau lapaʻau mai nā loea kūpono, a e hoʻomau i ka hahai ʻana i kēlā mau kuhikuhi.Inā he mau nīnau a hopohopo paha kāu e pili ana i kēia, mai makaʻu e kamaʻilio me kāu kauka. Aia lākou ma laila e kōkua iā ʻoe.

ʻO ka hāʻule ʻana o ka iwi iwi, nā hunaola koko ʻulaʻula, nā hunaola koko keʻokeʻo, nā platelets, ka hoʻoili ʻana o nā hunaola kumu, nā maʻi iwi iwi