Te Rahunga o te Huango Wheua: Me kōrero taipitopito tātou mō tēnei!

Ko te hinu wheua te pokapū hanga toto matua i roto i tō tinana. He rite ki tētahi wheketere. Ka hangaia e tēnei wheketere ngā mea pēnei i ngā toto whero, ngā toto mā, me ngā pereti toto hei āwhina i ō tātou tinana kia totoka. Whakaarohia te mea ka tupu mēnā ka mutu ohorere te mahi a tēnei wheketere, mēnā rānei kāore e mahi tika? Koinei te ngoikore o te hinu wheua. Me kōrero tātou mō tēnei me ētahi atu taipitopito .

He aha te ngoikore o te hinu wheua?

Ki te kī ngāwari, ko te ngoikore o te hinu wheua ko te kore e taea e tō hinu wheua te whakaputa i ngā toto whero hauora, ngā toto mā, ngā pereti toto rānei e ranea ana mō te tinana. He mahi motuhake tā ia momo pūtau i roto i tō tātou tinana.

• Ko ngā pūtau toto whero ngā mea e kawe ana i te hāora puta noa i te tinana. Mēnā he iti, ka pāngia pea koe e te ngenge me te mā.
• He rite ngā pūtau toto mā ki te ope taua i roto i tō tātou tinana. Ka whawhai rātou ki ngā mate. Ki te iti, he nui te tūponotanga ka pāngia koe e te mate.
• Ka āwhina ngā pereti toto ki te totoka, arā, ka mutu te toto ina whara koe. Mēnā ka heke te maha o ngā pereti toto, ahakoa he patunga iti noa iho, ka nui te toto ka puta.

He maha ngā wā ka puta te ngoikore o te hinu wheua hei raruraru o tētahi atu mate hauora. Engari i ētahi wā ka puta mai, kāore he take e kitea ana. Ka taea e ngā tākuta te whakahaere i ngā tohu mā te whakamahi i ngā rongoā me ngā momo maimoatanga. Heoi, ko te maimoatanga roa anake mō tēnei mate ko te whakawhitinga pūtau take allogeneic .

He aha ngā momo matua o tēnei āhuatanga?

E rua ngā momo matua o te ngoikore o te hinu wheua:

1. I whiwhi: Kāore ngā tohunga i te tino mōhio he aha te take o tēnei āhuatanga. Engari kua whakaatuhia e ngā rangahau ka taea te whakaoho e ētahi mate, te pānga ki ētahi matū, te whakamahinga rānei o ētahi rongoā. Ka whanake haere tēnei momo i roto i te wā.

2. Tuku iho: Ka puta tēnei mēnā ka riro mai i a koe ngā huringa ira (ngā whakarerekētanga ira) mai i tētahi, i ō mātua tokorua rānei. Ka kiia tēnei e ngā tākuta ko te Bone Marrow Failure Syndrome .

He pēhea te noa o tēnei āhuatanga?

Ehara tēnei i te mate tino noa. Hei tauira, tata ki te 65 o ia miriona pēpi i whānau i te United States i ia tau he mate tuku iho mō te ngoikore o te hinu wheua. Ko tētahi atu tauira ko te mate tuku iho e kiia nei ko te "Fanconi Anemia." Ka pā ki te kotahi i roto i te rima o ia miriona. Nō reira, ka kite koe, he ahua onge tēnei.

He aha pea ngā tohu?

Ka rerekē pea ngā tohu i runga i te pūtake o te ngoikore o te hinu wheua. Hei tauira, ko te tangata he ngoikore o te hinu wheua tuku iho ka tīmata pea te whakaatu i ngā tohu i te rua tau te pakeke. Heoi, e whakaatu ana ngā rangahau ka tīmata pea te whakaatu a te tangata he momo mate kua riro i a ia te tohu i waenganui i te 20 me te 25 tau te pakeke, i te 65 tau rānei te pakeke. Ahakoa te wā ka tīmata, ko ngā tohu tino kitea ko:

• Te mamae o ngā wheua
• Te toto nui rawa (hei tauira, te toto o te ihu, te toto o ngā kapia, te toto nui i te wā paheketanga)
• Ngenge nui me te ngenge rawa
• Te kirika auau
• Ngā mate pāwera huakita maha
• Ānini
• Te uaua ki te manawa (Dyspnea)
• Ka mā ake te tae o te kiri i te mea noa
• Ngā ira whero iti i raro i te kiri (Petechiae)
• He marutanga i te tinana, kāore he take e mōhiotia ana

Mena kei te mau tonu tētahi, ētahi rānei o ēnei tohu ki a koe, me toro atu ki te tākuta.

He aha i ngoikore ai tēnei hinu wheua? He aha ngā take?

Ka taea e ēnei mea te whakanui ake i tō tupono ki te whakawhanake i te ngoikore o te hinu wheua:

• Kei a koe te Bone Marrow Failure Syndrome (he mea tuku iho).
• Kei a koe te mate pukupuku toto, tētahi atu mate toto, ētahi mate toto, mate pukupuku, mate pāwera rānei (ka puta ake ēnei i muri mai, arā, "Kua Whiwhi").
• Te pānga ki ētahi matū me ngā rongoā (kei roto hoki tēnei i te wāhanga `(I whiwhi)`).

I ētahi wā, ka puta tēnei āhuatanga kāore he take e kitea ana. Ka kiia hoki e ngā tākuta ko te " idiopathic bone hunger failure." E whakapono ana ngā kairangahau he hononga kei waenganui i te autoimmunity me te bone hunger failure. Ko te autoimmunity ko te whakaekenga hē a te pūnaha ārai mate o tō tinana i tō wheua.

Ngā Mate Pukupuku Iwi Tuku Iho

Anei ētahi āhuatanga tuku iho, e pā ana, e rite ana rānei ngā tohu:

• Te Agranulocytosis Whānau (Kostmann Syndrome): Ka pā tēnei ki tētahi momo toto mā e kiia nei ko ngā neutrophils.
• Te Thrombocytopenia Amegakaryocytic Congenital (CAMT): I roto i tēnei āhuatanga, ka heke te maha o ngā pūtau e kiia nei ko ngā megakaryocytes, e āwhina ana ki te hanga i ngā pereti toto, i roto i tō hinu wheua.
• Te Mate Kore o te Taimana-Blackfan (Diamond-Blackfan Anemia): He mate mau tonu tēnei e pā ana ki te hanga o ngā toto whero.
• Dyskeratosis Congenita (Dyskeratosis Congenita - `(Dyskeratosis Congenita)`):Ko ngā tohu o tēnei mate ko ngā panoni kiri, he tohu tōmua pea tēnei o te ngoikore o te hinu wheua.
• Te Mate Kore o te Huka o Fanconi (Fanconi Anemia): Koinei te mate ngoikore o te hinu wheua e tino kitea ana.
• Te Mate Pūnaha Reticular (Reticular Dysgenesis): He momo mate pūnaha ārai mate whakakotahi nui tēnei (SCID). I tēnei āhuatanga, kāore e ranea te hanga o ngā pūtau-T pakari e tō tinana.
• Neutropenia Hānau Nui (Neutropenia Hānau Nui): I tēnei āhuatanga, ko ō neutrophils, he momo toto ma, he iti iho i te mea noa.
• Te Mate Shwachman-Diamond (Te Mate Shwachman-Diamond): Ka pāngia tō repeure, tō hinu wheua, me ngā wheua e tēnei mate.

Mena kei tētahi o ō mātua te mate me ngā tohu (e kiia ana tēnei ko te "Autosomal Dominant"), e 50% te tūponotanga ka pāngia koe e te mate me te whanaketanga o te mate ngoikore o te hinu wheua.

Heoi, ki te mea he whakarerekētanga ira kei ō mātua e rua e pā ana ki te momo kotahi o te mate ngoikore o te hinu wheua, engari kāore tetahi o rāua e pāngia e ngā tohu (e kiia ana tēnei ko te "Autosomal Recessive"), ka whakaitihia tō tupono ki te whakawhanake i te mate ki te 25%.

Ngā Matepukupuku Toto

He nui ake tō tūponotanga ki te ngoikore o te hinu wheua mēnā kei a koe tētahi o ēnei momo mate pukupuku e whai ake nei:

• Lymphoma
• Myeloma maha
• Te Mate Myelodysplastic

Ētahi atu Mate Toto

Ka whānau mai ētahi tāngata me ēnei āhuatanga. Ko ētahi ka pāngia e ēnei āhuatanga i roto i te wā. Ko ētahi o ēnei ko:

• Te Mate Kore Āhua o te Huka (Aplastic Anemia): He mate toto onge tēnei. Ka pakaru tō hinu wheua, ā, ka ārai i te hanga toto hou me ngā pereti toto. Ka whakamahia pea e ngā tākuta te kupu "aplastic anemia" ina kōrero ana rātou mō te ngoikore o te hinu wheua.
• Cytopenias (Cytopenias): Kei roto i tēnei ko ngā āhuatanga pēnei i te neutropenia autoimmune, te neutropenia idiopathic , me te leukemia lymphocyte granular nui.
• Hemoglobinuria Pō Pākarekare (Paroxysmal Nocturnal Hemoglobinuria): I tēnei āhua, ka whakangaromia e tō pūnaha ārai mate ngā toto whero.
• Te Aplasia o ngā Pūtau Whero Parakore (Te Aplasia o ngā Pūtau Whero Parakore): He āhuatanga tuku iho anō hoki tēnei e pā ana ki te hanga o ngā pūtau toto whero.

Ngā Mate Huaketo

Ka taea hoki e ngā mate huaketo e whai ake nei te whakanui ake i tō mōrearea:

• Cytomegalovirus `(Cytomegalovirus)`
• Te Huaketo Epstein-Barr
• Huaketo Ārai Mate Tangata (HIV)
• Parvovirus B19 `(Parvovirus B19)`
• Hepatitis huaketo

Ka taea hoki e te rongoā matū me te rongoā irahiko mō te mate pukupuku te whakanui ake i tō tupono ki te ngoikore o te hinu wheua. Waihoki, ko te pānga ki ngā matū me ngā whakarewa e whakamahia ana i roto i ētahi patu pepeke me ngā patu pepeke ka whakanui ake i tō tupono.

He aha ngā raruraru o tēnei āhuatanga?

He mate kino tēnei , ā, ka mate pea . Ahakoa i muri i te maimoatanga, ka puta pea ngā raruraru e whai ake nei:

• Ngā mate kitakita, ngā mate huaketo rānei
• Te toto
• Te mate pukupuku toto, hei tauira, te mate pukupuku myeloid acute, te myelodysplasia rānei
• Ētahi atu pukupuku matepukupuku, pērā i te osteosarcoma me te rhabdomyosarcoma
• Matepukupuku Pūtau Squamous

Me pēhea te tautuhi i tēnei?

Ka pātai tuatahi te tākuta ki a koe mō ō tohumate, tō hītori hauora, me te hītori hauora o tō whānau. Ka tirotirohia hoki e rātou te tinana. Tērā pea ka tonoa e rātou he whakamātautau toto me ngā whakamātautau whakaahua.

Ngā Whakamātautau Toto

Kei roto pea i ngā whakamātautau toto:

• Whakamātautau Ceruloplasmin
• Tatau Toto Katoa (CBC)
• Whakamātautau Ferritin
• Whakamātautau mō te Kore o te Folate
• Whakamātautau mō te ngoikore o te huaora B12

Ngā Whakamātautau Whakaahua

Ko ngā whakamātautau whakaahua pea ko:

• Whakaahua Āhua Aukume (MRI - matawai `(Whakaahua Āhua Aukume)`)
• Te Tomography Tukunga Positron (PET - `(Positron Emission Tomography)` matawai)
• Whakamātautau Ultrasound `(Ultrasound)`

I runga i ngā hua o ngā whakamātautau toto me ngā whakamātautau whakaahua, ka tūtohu pea tō tākuta i tētahi koiora wheua . Ka tūtohu pea hoki ia i tētahi whakamātautau ira hei tautuhi i ngā whakarerekētanga ira e puta ai te ngoikore o te wheua.

Tērā pea ka tonoa e tō tākuta he whakamātautau hei tātaritanga i ētahi mate, hei whakakore rānei i ētahi atu mate. Mā rātou koe e whakamārama he aha rātou e mahi ana i ia whakamātautau, me ngā mea ka taea e ngā whakamātautau te whakaatu.

He aha ngā maimoatanga?

Ka whakawhirinaki te maimoatanga ka whiwhi koe ki ētahi āhuatanga, tae atu ki:

• Te momo ngoikore o te hinu wheua (arā, ahakoa he mea tuku iho, he mea whiwhi rānei).
• Te kaha o tō āhua.
• Tō tau me tō hauora whānui.
• Ō tohumate.

Ko ngā kōwhiringa maimoatanga pea ko:

• Ngā rongoā paturopi , ngā rongoā harore, me ngā rongoā patu huaketo hei whawhai i ngā mate pāwera.
• Te whakawhiti toto hei whakanui ake i te maha o ngā toto whero me te whakaiti i ngā tohu pēnei i te toto me te ngenge.
• Ka āwhina ngā mea whakaohooho hinu wheua i tō hinu wheua kia nui ake ngā pūtau toto e hanga ana.
• Ka āwhina ngā rongoā ārai mate ki te aukati i tō pūnaha ārai mate ki te whakaeke i tō hinu wheua.
• Te Whakatō Pūtau Take Allogeneic (Ngārara Wheua) .

He pānga taha, he raruraru rānei kei te rongoā? (Ngā raruraru o te rongoā)

He rerekē ngā raruraru me ngā pānga taha i runga i te maimoatanga, engari ko ngā whakawhitinga pūtau take (hinu wheua) ngā mea tino pea ka puta he raruraru nui. Kei roto i ēnei ko te mate graft-versus-host (GVHD) me ngā mate pāwera.

Ka pēhea te āhua o te oranga me tēnei mate? (Te matapae)

Ko te mea ka taea e koe te tumanako i muri i te maimoatanga (te matapae) ka whakawhirinaki ki ētahi āhuatanga, tae atu ki te momo mate, tō tau me tō hauora whānui, me te urupare a tō tinana ki te maimoatanga.

I te nuinga o te wā, me whiwhi tonu te hunga e ngoikore ana te hinu wheua i te tiaki hauora me te tautoko . Engari kāore i te rite te katoa. Kia mōhio koe ki tō āhua, ka pātai atu ki tō tākuta he aha te mea ka tupu i muri i tō maimoatanga tuatahi.

He mate whakamate te ngoikore o te hinu wheua? (He mate whakamate?)

He mate tino kino tēnei, he mate morearea hoki . Ko te nuinga o te hunga e ngoikore ana te hinu wheua ka rongoatia e ngā tohunga mohio pēnei i ngā tohunga pukupuku me ngā tohunga toto.

He aha ngā kōrero mō te roanga o te oranga o ēnei tūroro? (Te roanga o te oranga)

Ko te roanga o te oranga he whakatau tata mō te roa o te oranga o te tangata i muri i te maimoatanga mō tētahi mate. Ko te roanga o te oranga o te hunga e ngoikore ana te hinu wheua ka rerekē mai i ētahi marama ki te roanga o te oranga.

Engari, he motuhake koe, ā, he rerekē pea ō wheako i ō te katoa. Pātai atu ki tō tākuta mō ngā mea ka taea e koe te tumanako, me te roa o tō oranga. Ko rātou te puna mōhiohio pai rawa atu māu, nā te mea e mōhio ana rātou ki a koe me tō āhua.

Ka taea te whakaiti i te mōrearea? (Te Whakaiti i te Mōrearea)

Engari, kāore he huarahi hei whakaiti i te mōrearea o te ngoikore o te hinu wheua tuku iho. Heoi, mā te karo i ngā matū e mōhiotia ana ka whakaiti i te mahi a te hinu wheua ka taea te whakaiti i te mōrearea o ētahi ngoikore o te hinu wheua kua riro mai. Waihoki, mā te maimoatanga wawe ka taea te whakaora i ō tohu me te whakapai ake i te kounga o tō oranga.

Me pēhea taku tiaki i ahau anō? (Manaaki i ahau anō)

Ko te mea pai rawa atu ko te tiaki i tō hauora whānui . Hei tauira, kaua e whakamahi i ngā hua tupeka, kaua e whakawhāiti, kaua rānei e mutu te inu waipiro. Anei ētahi atu whakaaro:

• Tirohia tō tākuta i ia wā, i ia wā. Ka noho tonu pea te ngoikore o te hinu wheua hei mate mō te wā roa. Tērā pea me haere tonu te maimoatanga. Ka puta mai hoki he raruraru hauora hou. Nō reira, he mea nui kia tirotirohia koe i ia wā. Ka aroturukihia tō hauora whānui e tō tākuta, ā, ka rongoāhia hoki ngā raruraru hou.
• Kai i tētahi kai hauora. Tērā pea ka uaua ki a koe te kai, ka ngaro rānei tō hiahia kai i ō tohu me ngā pānga taha o tō maimoatanga. Mēnā kāore koe i te tino mōhio kei te whiwhi koe i te kai e hiahiatia ana e koe, kōrero ki tētahi tohunga kai. Ka taea e rātou te tuku tohutohu tika ki a koe.
• Whakakorikori tinana. Ka taea e te pāngia e te mate hauora kino pēnei i te ngoikore o te hinu wheua te whakararuraru. He huarahi pai te whakakorikori tinana hei whakamamae i te ahotea. Me kōrero ki tō tākuta i mua i te tīmatanga o tētahi mahi whakakorikori tinana hou.

Āhea koe me haere ki te Wāhanga Maimoatanga Ohotata (ETU) ?

Mena kei te ngoikore tō hinu wheua, he nui ake tō tūponotanga ki te pangia e ngā mate me ngā raruraru toto. Me haere koe ki te rūma whawhati tata mēnā ka pāngia koe e tētahi o ēnei e whai ake nei:

• He kirika kāore e heke ahakoa kua tangohia he rongoā mamae noa
• Te mātao me te wiri (Chills)
• Te toto e kore e taea te aukati

He aha ngā pātai me pātai atu e au ki te tākuta?

Tērā pea ka ngoikore tō hinu wheua nā ētahi mate tuku iho. Engari i ētahi wā ka pā mai tēnei āhuatanga me te koretake o te take. Ka taea e koe te pātai atu ki tō tākuta i ēnei pātai:

• E mōhio ana koe he aha i pā mai ai tēnei ngoikore o te hinu wheua ki ahau?
• He aha ngā kōwhiringa maimoatanga kei a au?
• Me tango maimoatanga tonu ahau?
• Ka taea te rongoā i tēnei?

Mena kei te ngoikore tō wheua wheua, tera pea kei te pāngia koe e tētahi raruraru hauora nui, pērā i te mate tuku iho, te mate toto, te mate pukupuku rānei. Tērā pea kei te puta ake he maha ngā pātai me ngā āwangawanga hou mai i tēnei mate hou, ā, ka tino taumaha rawa atu ēnei. He mea noa te manukanuka nā te mea he mate morearea tēnei. Kōrero ki tō tīma hauora mō ngā mea hei tumanako atu me ngā mea hei mahi i muri mai. Ka koa rātou ki te whakautu i ō pātai.

Kia maumahara hei whakarāpopototanga (Karere hei tango ki te kāinga)

He mate kino, he mate uaua hoki te ngoikore o te hinu wheua. Engari kia maumahara, he maha ngā take pea, ā, he maha ngā momo maimoatanga me ngā rautaki whakahaere. Ko te mea nui ko te kimi wawe i te tātaritanga, te rapu maimoatanga mai i ngā tohunga tika, me te whai tonu i aua tohutohu.Mena he pātai, he āwangawanga rānei āu mō tēnei mea, kaua e wehi ki te kōrero ki tō tākuta. Kei reira rātou hei āwhina i a koe.

Te ngoikore o te hinu wheua , Ngā pūtau toto whero, Ngā pūtau toto mā, Ngā pereti toto, Te whakawhitinga pūtau take, Ngā mate hinu wheua