Kei roto i tō whānau tetahi o te "Mate Pūtau Hāmana"? Kia tino mōhio tātou!

I tēnei rā ka kōrero tātou mō tētahi mate hou ki te nuinga o te tangata, engari he tino tata ki ētahi whānau. Ko te ingoa o tēnei mate ko te Mate Pūtau Hāri (SCD). Kua rongo pea koe i tēnei ingoa i mua. He mate e pā ana ki ō tātou toto, arā, e pā ana ki te toto, ā, he mate tuku iho. Me titiro tātou he aha tonu tēnei mate, he aha i puta ai, me ētahi atu mea ka taea te mahi mōna.

He aha te mate pūtau hāripiri? Kia mārama tātou!

Ki te kī ngāwari, ko te mate toto toto hāripiri (SCD) he mate e pā ana ki ngā toto toto whero i roto i tō tātou tinana, he mea tuku iho mai i ngā mātua ki ngā tamariki. Ko tētahi tēnei o ngā mate toto tuku iho tino noa o te ao. Inaianei tirohia, kei roto i ō tātou toto toto whero he pūmua e kiia nei ko te hemoglobin . He mea tino nui tēnei hemoglobin. Nā te mea, ko tēnei pūmua te mea e kawe ana i te hāora puta noa i tō tātou tinana. He rite ki te taxi hāora. Ko ngā toto toto whero o te tangata hauora he porowhita, he ngāwari hoki. He rite ki ngā pōro rapa iti. Nā tēnei, ka taea e rātou te ngokingoki ngāwari i roto i ngā oko toto iti rawa o te tinana (e kiia ana e mātou he capillaries) ka hoatu te hāora ki ō tātou whekau me ngā kiko katoa. Heoi, ko te mea e pā ana ki te tangata he mate toto toto hāripiri ko te iti o te huringa o tēnei hemoglobin. Ko tēnei hemoglobin rerekē e kiia ana ko te hemoglobin S. Mā tēnei ka huri ngā toto toto whero hei āhua marama, hei āhua haurua marama rānei, kaua hei porowhita, hei ngāwari hoki . Ehara i te mea koinā anake, engari he tino pakari ēnei pūtau, kāore e piko ngāwari, ā, ka piri tahi. Whakaarohia, he aha te mea ka tupu ina haere ēnei toto toto āhua hāripiri i roto i aua oko toto iti? Ka mau rātou! Kātahi ka whakararuraruhia te rere o te toto. Arā, kāore e nui te hāora e whiwhihia ana e ō tātou whekau me ngā kiko tino nui. Koia te take ka puta ai ngā raruraru nui pēnei i te mamae nui, ngā mate maha, te kino o ngā whekau, me te kore mahi tika . Ko tētahi atu mea, kāore ēnei pūtau āhua-toru e ora rite tonu ki ngā toto whero noa. Ka tere te mate. Kātahi ka kore tonu ngā toto whero i roto i te tinana. Koinei te mea e kiia nei e tātou ko te anemia . He mate mō te wā katoa te mate pūtau-toru. Engari kaua e manukanuka, he maimoatanga kei reira mō tēnei. Mā ēnei maimoatanga, ka taea e koe te whakaiti i ngā tohu me te whakaroa i tō oranga.

He rerekē ngā momo mate pūtau hāripiri?

Ae, he maha ngā momo mate pūtau maripi. Ko ēnei momo ka whakatauhia e ngā ira ka riro mai i te tangata i ōna mātua.

Hemoglobin SS (HbSS)

Koinei te momo tino kino o te mate pūtau maripi. Tata ki te 65% o te hunga whai SCD he momo tēnei. Ka riro i ēnei tāngata te ira hemoglobin S mai i ngā mātua e rua. Ko te tikanga he rerekē te nuinga, te katoa rānei o ō rātou hemoglobin. Nā tēnei ka pāngia rātou e te anemia mau tonu.

Te Hemoglobin SC (HbSC)

He ngawari, he taurite rānei tēnei.He momo kei te taumata teitei. Tata ki te 25% o te hunga whai SCD kei a rātou tēnei. Ka riro i ēnei tāngata te ira hemoglobin S mai i tētahi matua, me te ira mō tētahi atu momo hemoglobin rerekē e kiia nei ko te hemoglobin C mai i tētahi atu matua.

Te Hemoglobin (HbS) Beta Thalassemia

Ka riro i ēnei tāngata tētahi ira hemoglobin S mai i tētahi matua, me tētahi ira rerekē e kiia nei ko te beta thalassemia mai i tētahi atu matua. E rua anō ngā momo momo o tēnei:

• "Tāpiri" (HbS beta +): He momo tēnei e pā ana ki te 8% o te hunga whai SCD , ā, he ngawari noa iho.
• "Kore" (HbS beta 0): He momo tēnei e pā ana ki te 2% o te hunga whai SCD, ā, ka rite te kino ki te mate hemoglobin SS (HbSS).

Ētahi atu momo onge

Haunga tēnei, kei reira anō ētahi atu momo onge pērā i te hemoglobin SD (HbSD), te hemoglobin SE (HbSE), me te hemoglobin SO (HbSO) . Ka riro i ēnei tāngata tētahi ira hemoglobin S me tētahi atu ira rerekē e kiia nei ko te D, E, O rānei.

He aha te rerekētanga i waenga i te mate toto iti me te mate pūtau iti?

Koinei te wāhi e pōhēhē ai te tini o te tangata. Ko te Mate Pūtau Hīnati (SCD) he kupu whānui mō ngā momo mate pūtau hīnati katoa kua whakahuatia i runga ake nei. He rite ki te maru. Kei raro i taua maru ngā momo katoa. Ka whakamahia noa e ngā tākuta te kupu "Anemia Pūtau Hīnati" mō ngā momo SCD tino kino e puta ai te anemia . Arā, ko ngā momo e kiia nei ko te hemoglobin SS (HbSS) me te hemoglobin beta zero thalassemia. Kei te mārama koe?

He aha te rerekētanga i waenga i te Āhuatanga o te Pūtau Hārire me te Mate?

Ko te āhuatanga pūtau maripi anake kei ētahi tāngata. Ko te tikanga o tēnei ka riro i a rātou te ira hemoglobin S mai i tētahi mātua anake . Ka riro i tētahi atu mātua tētahi ira hauora. I te nuinga o te wā, kāore ngā tāngata whai āhuatanga pūtau maripi e whakaatu i ngā tohu o te mate pūtau maripi. Heoi, ka taea e rātou te tuku i tēnei ira rerekē ki ā rātou tamariki. Ko te tikanga he kawe rātou i tēnei ira. Heoi, he tino onge, ka pāngia te hunga whai āhuatanga pūtau maripi e ngā raruraru hauora mena ka tino matewai rātou , ka uru rānei rātou ki te whakakori tinana kaha . Kei te haere tonu ngā rangahau mō tēnei.

He pēhea te noa o te mate pūtau hāripiri?

E ai ki ngā whakatau tata a ngā kairangahau, tata ki te 100,000 ngā tāngata i te Hononga o Amerika anake e pāngia ana e te mate pūtau maripi. He mea tino noa tēnei i waenga i ngā tāngata o te uri Āwherika. Ka kitea anō hoki i waenga i ngā Amerikana Hispanic, me ngā tāngata o te uri Mediterranean, Middle East, Indian, me Āhia. Kei reira anō hoki ngā tāngata e pāngia ana e tēnei mate i Sri Lanka.

He aha te take o te mate pūtau hāripiri?

Ko te mate pūtau maripi he ira whakarerekē i roto i te ira e kiia nei ko te ira HBB . Ko tēnei ira HBB te kawenga mō te hanga i tētahi wāhanga o te hemoglobin. Ko te hunga whai SCD ka whiwhi i ngā ira HBB e rua kua whakarerekētia e hanga ana i te hemoglobin rerekē - tētahi mai i ia mātua. Ka kiia tēnei he tukunga iho autosomal recessive . Ko te tikanga o tēnei ko ngā mātua e rua o te tamaiti whai SCD he kape kotahi o te ira kua whakarerekētia (ko te tikanga ka taea e rātou te whai i te āhuatanga pūtau maripi). Heoi, ko aua mātua kāore e whakaatu i ngā tohu.

Ko wai kei te nui ake te mōrearea ki te whakawhanake i te mate pūtau maripi (SCD)?

Ko ētahi rōpū tāngata ka nui ake te tūponotanga ka pāngia e tēnei mate. Ko ēnei:

• Ngā tāngata nō te iwi Āwherika (hei tauira, ko ngā Āwherika-Amerika).
• Ngā Amerikana Hispanika i Amerika ki te Tonga me Amerika Waenganui.
• Ngā tāngata o te Moana-nui-a-Kiwa, te Waenganui Rawhiti, ngā Īnia, me ngā Āhia.

He aha ngā tohu o te mate pūtau hāripiri?

Ko ngā tohu o te mate pūtau maripi ka tīmata te puta mai i te wā e 5 ki te 6 marama te pakeke o te tamaiti. Ka rerekē ēnei tohu i ia tangata, i ia tangata. He ngawari ngā tohu o ētahi tāngata, ko ētahi ka pāngia e ngā raruraru nui. Ko ngā tohu matua ka kitea ko:

• Ngā wā mamae maha.
• Te mate toto iti : Ka taea e tēnei te whakangā nui, te kiri mā, me te ngoikore.
• Te kiri māwhero : Te kōwhai o te kiri me te mā o ngā kanohi.
• Te pupuhi mamae o ngā ringa me ngā waewae.

He aha ngā raruraru o tēnei āhuatanga?

Ka pāngia e te mate pūtau maripi te maha o ngā wāhanga o te tinana. Ka tīmata ohorere ētahi pānga (kaha), ko ētahi ka roa te wā (pūmau). Ka tīmata wawe ēnei raruraru, ā, ka roa mō te wā katoa.

Mamae

Koinei te raruraru tino noa o te mate pūtau maripi. Ka puta te mamae ina piri ngā pūtau āhua maripi ki roto i ngā oko toto, ā, ka aukati i te rere o te toto. Tērā pea ka pāngia koe e te mamae ohorere, nui. Ka kiia hoki tēnei he raruraru mamae, he raruraru pūtau maripi, he raruraru vaso-occlusive (VOC), he wāhanga vaso-occlusive rānei (VOE) . Ka taea e ēnei raruraru mamae te ngawari, te kino rānei, ā, ka tīmata ohorere, ka roa mō tētahi wā. Ko te nuinga o te wā ka pāngia te uma, te tuara, ngā waewae, me ngā ringa. Tērā pea ka pāngia ētahi tāngata e te mamae mau tonu , arā, ko te mamae e roa ake ana i te ono marama.

Te mate toto iti

Ka puta te anemia i te mate pūtau toto whero nā te mea ka tere rawa te mate o ngā pūtau toto whero. Ko te anemia te korenga o ngā pūtau toto whero hauora e taea ai te kawe hāora puta noa i te tinana. Koia te takeTērā pea ka pā mai te ngenge nui, te kiriuhi waikura, te manukanuka, te whanoke, me te māuiui.

Te Mate Uma Whakapeka

He āhuatanga ohorere hauora tēnei e morearea ana ki te ora . Ka taea e ia te kino i ngā pukahukahu, te whakararu i te manawa, me te whakaiti i te tuku hāora ki ētahi atu wāhanga o te tinana. Ka puta tēnei raruraru ina aukatia e ngā pūtau maripi te rere o te toto me te hāora ki ngā pukahukahu.

Ngā Putunga Toto

Ka whakanui ake te mate toto toto i te anemia pūtau maripi. Ka whakanui ake tēnei i te mōrearea o te whakawhanake i te toto toto i roto i te uaua hohonu (thrombosis uaua hohonu - DVT). Ka taea hoki e tēnei DVT te whati ka noho ki roto i ngā pukahukahu (embolism pūkahukahu - PE).

Whiu

Ki te mau ngā pūtau toto marino ki roto i tētahi oko toto e anga atu ana ki te roro, ka āraia te rere o te toto ki te roro. Kātahi ka kore e nui te hāora e whiwhi i te roro hei mahi. Ka taea e tēnei te pāngia e te whiu . Tata ki te 10% o te hunga whai SCD ka pāngia e te whiu haumanu. Ko te hunga whai anemia pūtau marino he nui ake te mōrearea ki te whiu.

Ngā raruraru tirohanga

Ka taea e ngā pūtau hāripiri te aukati i ngā oko toto i roto i ngā kanohi. Ko te nuinga o te wā ka puta tēnei i roto i te retina . I ētahi wā kāore he tohu, kātahi ka ngaro ohorere te tirohanga, ā, ka arahi pea ki te matapo tuturu.

Priapism

Ko te Priapism he āhuatanga e aukatia ai ngā pūtau āhua-toru i roto i te ure o te tāne, ka puta he ure mamae, he pumau ( priapism ). Haunga te mamae, ka taea e te priapism te whakararuraru tuturu me te kore e taea te ure . Ko te priapism e roa ana neke atu i te whā hāora he āhuatanga ohorere hauora.

Te pakaru me te ngoikore o te okana

Ko te hunga whai mate pūtau maripi kei te mōrearea ki ngā raruraru o ō rātou ngākau, o ō rātou pukahukahu, o ō rātou whatukuhu, me ētahi atu okana nā te mea kāore e nui te toto me te hāora e whiwhi ana rātou. Ka taea e te SCD te arahi ki te ngoikore o ngā okana maha .

Ka pāngia te hāputanga e te āhuatanga, te mate rānei o te pūtau hāripiri?

He maha ngā wāhine e pāngia ana e te mate pūtau maripi he hāpu hauora. Engari he nui ngā mōrearea. Ka taea e te SCD te whakanui ake i te mōrearea o te toto toto tiketike, te toto totoka, te materoto, ngā pēpi iti te taumaha whānau, me ngā whānautanga o mua . Nō reira he mea nui kia whai i ngā tohutohu a tō tākuta.

Me pēhea te tautuhi i te mate pūtau maripi?

I Sri Lanka, pērā i te maha atu o ngā whenua o te ao, ka tirotirohia ia pēpi hou mō te mate pūtau maripi hei wāhanga o ngā tirotiro pēpi hou . Ko te tikanga o tēnei ko te tango i tētahi tauira toto iti mai i te rekereke o te pēpi ka whakamātautau. Ka tirohia hoki tēnei mō ētahi atu mate. Hei whakaū i te mate, ka whakahaerehia e te tākuta o te tamaiti he whakamātautau hiko hemoglobin . Waihoki, ka taea te kite i te mate pūtau maripi i mua i te whānautanga o te pēpi mā te whakamātautau i mua i te whānautanga . Kei roto i ēnei whakamātautau:Kei roto i ēnei ko te tango tauira chorionic villus me te amniocentesis .

He rongoā katoa kei te wātea mō te mate pūtau hāripiri?

Ae, ka taea e te whakawhitinga wheua wheua , e mōhiotia ana ko te whakawhitinga pūtau take, te rongoā i te mate pūtau maripi. Ko te tikanga o tēnei ko te tango i te wheua wheua hauora mai i tētahi kaituku hauora, he hototahi ki ngā ira (pērā i te tuakana/teina) ka whakawhitinga ki te tūroro. Heoi, tata ki te 18% noa iho o te hunga whai SCD ka kitea he hoa ōrite. He tūponotanga me ngā raruraru anō hoki kei roto i tēnei whakawhitinga. Ka kōrerohia tēnei e tō tākuta ki a koe.

He aha ngā maimoatanga mō te mate pūtau hāripiri?

Ko te maimoatanga mō te mate pūtau maripi he rongoā, he whakawhiti toto, he whakawhitinga wheua wheua, me te rongoā ira . Tērā pea ka tīmata te maimoatanga ki ngā paturopi . Ka hoatu he paturopi ki ngā pēpi hou e pangia ana e te SCD kino e rua ngā wā i te rā, tae atu ki te 5 tau hei ārai i te mate.

Ētahi atu rongoā

He maha ngā tāngata whai SCD e whakamahi ana i ngā rongoā hei whakaiti i te kaha o tō rātou mate, hei rongoā hoki i ngā tohumate. Ko ētahi o ēnei ko:

• Voxelorator: Ka ārai tēnei i te hurihanga o ngā toto whero hei āhua maripi, me te piri tahi. Ka whakaitihia te whakangaromanga o ētahi toto whero, ka whakapai ake i te rere o te toto ki ngā whekau, ā, ka whakaitihia te tūponotanga o te mate toto kore toto.
• Crizanlizumab: Ka āwhina tēnei rongoā ki te ārai i ngā toto whero āhua-herehere kei piri ki ngā pakitara o ngā oko toto . Ka taea e tēnei te whakapai ake i te rere o te toto me te whakaiti i te mumura me te mamae.
• Hydroxyurea : Ka taea e tēnei te whakaiti, te ārai rānei i ētahi raruraru o te SCD, pērā i te mamae nui, te mate uma whakapeka, me te anemia kino.
• L-glutamine: He rongoā mamae tēnei. Ka taea e ia te āwhina ki te whakaiti i te maha o ngā whakaekenga mamae e pā ana ki a koe. Ko ētahi atu rongoā mamae ko ngā rongoā anti-inflammatory kore-steroidal (NSAID) me ngā opiates .

Ngā whakawhiti toto

Ka tūtohu pea tō tākuta i ētahi whakawhiti toto hei rongoā me te ārai i ngā raruraru o te SCD.

• Ngā whakawhiti toto ohorere:Ka āwhina ēnei ki te rongoā i ngā raruraru e puta ai te anemia nui. Ka taea hoki e ngā tākuta te whakamahi i ēnei mō ngā āhuatanga ohorere pēnei i te whiu, te mate uma ohorere, me te ngoikore o ngā okana.
• Ngā whakawhiti toto whero: Ka taea e ēnei te whakanui ake i te maha o ngā toto whero i roto i te tinana, ā, ka puta mai ngā toto whero noa kāore i te āhua rite ki te toronaihi.

Te whakawhitinga pūtau take (e kiia ana hoki ko te whakawhitinga wheua)

Ka taea te rongoā i te SCD mā te whakawhitinga pūtau take. Me whai kaituku pūtau e ōrite ana (pēnei i te tuakana/teina). Kei te haere tonu ngā rangahau hei whakapai ake i ngā whakawhitinga mai i ngā mātua, i ngā tuakana/teina rānei kāore i ōrite. Ka matapakihia e tō tākuta ngā mōrearea me ngā painga o tēnei maimoatanga i runga i tō āhuatanga motuhake.

Te Whakaora Ira

Kei te rangahau ngā kairangahau i te rongoā ira hei rongoā i te SCD. Ko te tikanga o tēnei ko te whakatika i te ira hemoglobin rerekē, te whakauru rānei i tētahi ira hemoglobin hauora ki roto i ngā pūtau take o te tangata. He tino whai hua ngā raraunga tōmua mō tēnei. Ko te tumanako ka noho te rongoā ira hei rongoā noa mō te SCD i tētahi rā.

Ka taea te ārai i tēnei?

He mate ira te mate pūtau hāripiri, nō reira kāore e taea te ārai . Mena kei te hapu koe, he mea pai kia kōrero koe ki tō tākuta mō ngā whakamātautau ira , mō te tohutohu ira rānei. Mā tēnei ka āwhina i a koe me tō hoa rangatira ki te mōhio mēnā kei a koe te ira me te mōrearea o te tuku atu ki ā kōrua tamariki.

He aha ngā mea ka taea e te tangata whai mate pūtau maripi te tumanako?

Tērā pea he poto ake te roanga o te oranga o te hunga whai mate pūtau maripi i te taupori whānui. Heoi, kua tino pai ake te roanga o te oranga me te kounga o te oranga nā ngā maimoatanga hou mō te SCD. Mēnā ka pai te whakahaere i te mate, ka taea e te hunga whai mate pūtau maripi te ora tae noa ki te 50 tau.

Me pēhea taku tiaki i taku tamaiti mēnā he mate pūtau marino tōna?

Mena he mate pūtau marino tō tāu tamaiti, he maha ngā mea ka taea e koe te mahi hei whakahaere i tōna āhua:

• Me kawe tonu tāu tamaiti ki te tākuta.
• Hoatu ki tāu tamaiti ngā kano ārai mate katoa kua tūtohutia i te wā tika.
• Āwhina i tāu tamaiti kia whakakorikori tinana i ngā wā katoa, kia kai hoki i ngā kai hauora mō te ngākau .
• Ina pā mai he mamae nui, hoatu ki tāu tamaiti he nui ngā wai me te rongoā anti-inflammatory kore-steroidal (NSAID) . Ki te kore e taea te whakahaere i te mamae i te kāinga, haria tāu tamaiti ki te hōhipera kia whiwhi ai i ngā rongoā whakamamae mamae kaha ake.

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

Ka taea e te mate pūtau maripi te whakararu i te ora o te tangata. Mena ka pāngia koe, tō tamaiti rānei e ētahi o ngā tohu e whai ake nei, waea atu ki te 911, haere rānei ki te rūma ohorere (ER) tata tonu:

• Mamae nui.
• Ngā tohu o te toto toto nui: te ngenge nui, te whanoke, me te poto o te manawa.
• Te kirika neke atu i te 101.3 nekehanga Fahrenheit (38.5 nekehanga Celsius).
• Ngā raruraru tirohanga.
• Te uaua ki te manawa.
• Te whakatūnga o te ure e whā hāora, neke atu rānei te roa (priapism).
• Ngā tohu o te mate pūkahukahu acute: mamae o te uma, mare, kirika.
• Ngā tohu o te whiu: Te ngoikore ohorere o te taha kotahi o te tinana, te ngoikore o te tinana, me te ngaro o te māramatanga.

He aha te take e mamae ai te mate pūtau hāripiri?

He māmā noa iho, he rite te āhua o ngā toto toto whero he rite ki te reta C, ki te marama kōwhai rānei. I a rātou e haere ana i roto i ō uaua toto, ka mau, ka aukati i te rere o te toto. Koinā te wā e puta ai te mamae. Whakaarohia he rite ki te waka i runga i te rori.

He mate pūnaha ārai mate te pūtau hāripiri?

Kāo. Ahakoa he āhuatanga tō te mate pūtau hāripiri ki ngā mate pūnaha ārai mate , kāore ngā tākuta e whakaaro he mate pūnaha ārai mate te SCD. E whakaaro ana rātou he mate ira te SCD.

He mate mō te wā roa te mate pūtau maripi. Kāore e taea te whakamahi i ngā whakawhitinga pūtau take, e taea ai te whakaora katoa, ā, he morearea hoki. Heoi, mā te tātaritanga me te maimoatanga wawe ka āwhina i te whakaiti i ngā tohu me te mōrearea o ngā raruraru. Mā te tiaki hauora auau, ka taea e koe te ora i tētahi oranga ora, ora tonu.

Hei whakamutunga, ētahi mea nui hei maumahara māu (Karere hei tango ki te kāinga)

Āe, kua maha ā tātou kōrero mō te mate pūtau maripi. Anei ētahi mea nui hei maumahara:

• Ko te mate toto hāripiri (SCD) he mate toto tuku iho e rerekē ai te āhua o ngā toto toto whero, ka puta ai ngā raruraru ki te rere o te toto.
• Ko te take matua mō tēnei he momo hemoglobin rerekē e kiia nei ko te hemoglobin S.
• He mea noa te mamae, te kore toto, ngā mate pāwera, me te kino o ngā whekau .
• He mea tino nui te kitenga wawe me te maimoatanga tika. Ka tirotirohia ngā pēpi whānau hou mō tēnei.
• Ahakoa he rongoā te whakawhitinga wheua, kāore e pai mō te katoa. Tērā atu anō ngā maimoatanga, pērā i ngā rongoā me ngā whakawhiti toto.
• Ka taea e koe te ora pai me tēnei mate mā te huri i te āhua noho, te whai i ngā tohutohu hauora tika, me te mahi tere i roto i te āhuatanga ohorere .

Mena he pātai āu, he pātai rānei tā tētahi e mōhio ana koe mō te mate pūtau maripi, kia kite i te tākuta mō ētahi tohutohu. Kāore he mea hei wehi, hei whakama rānei. Ko te mea nui ko te mōhio! Te mate pūtau maripi, te hemoglobin, ngā toto whero, te anemia, ngā mate ira, ngā mamae ohorere, te maimoatanga pūtau maripi