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Chii chinonzi sickle cell chirwere (uye inorapwa sei)?

Chii chinonzi sickle cell chirwere (uye inorapwa sei)?Dzidzo yehutano

Sickle cell chirwere (SCD) iboka reropa rakagara nhaka zvinokanganisa masero matsvuku eropa, kunyanya mamorekuru ari mumasero aya anonzi hemoglobin inoendesa oxygen kumaseru mumuviri wese. Zvinoenderana ne Nzvimbo dzekudzivirira nekudzivirira hosha (CDC), mamiriro acho anokanganisa mamirioni avanhu kutenderera pasirese, kusanganisira vanofungidzirwa vanhu zviuru zana muUnited States.

Unowana sei chirwere chesero?

Kuti munhu ave nejeko cell chirwere, vese vabereki vanofanirwa kupfuudza chakakanganisika chimiro chehemoglobin gene kumwana wavo. Kana chete mubereki mumwe chete akapfuudza jeni, mwana anogara nhaka yejazi masero maitiro. Izvi zvinoreva kuti ropa ravo rinogona kunge riine mamwe masero ejeko, asi havawanzorwara.



Varwere veSickle cell vakanganisa mamorekuru ehemoglobin anotambanudza masero matsvuku eropa kuita jeko, kana chimiro. Aya maseru akaomarara, uye membrane yavo inokuvara uye inoparara zviri nyore. Nepo masero matsvuku eropa achigara mukutenderera kwemuviri kwemazuva zana nemakumi maviri, masero ejeko anorarama mazuva anosvika gumi nemasere kana gumi nemapfumbamwe. Masero anorwara anovhara tsinga dzeropa diki (capillaries), iyo inononoka kuyerera kweropa kunhengo dzakasiyana uye inogona kukonzera kukuvara kwenguva refu kwemitezo.



Kunyangwe hazvo kusarongeka kwekutanga kuri mukati meiyo tsvuku tsvuku, chirwere chemajeko masero chinova chirwere chemultisystem, anodaro Abdullah Kutlar, MD , mukuru hematologist uye director weSickle Cell Center kuAugusta University. Matambudziko emakiriniki anokanganisa nhengo dzese dzemuviri kubva kumusoro kusvika kutsoka. Iyo inosangana nesitiroko, inokanganisa maziso, mapapu, spleen, uye mapfupa.

Sickle cell chirwere chinonyanya kubata vanhu vane madzitateguru aibva kuSub-Saharan Africa, nzvimbo dzinotaura chiSpanish kuWestern Hemisphere (South America, Caribbean, neCentral America), India, nedzimwe nyika dzeMiddle East neMediterranean. Chirwere chemasero chinowanzoitika kazhinji muvanhu vanobva kunzvimbo dzepasi uko marariya yakajairika-kungave nhasi kana munguva yakapfuura. Vanhu vanotakura jeko masero maitiro vane mukana uri nani wekupona vachirwara nemalaria.



Sickle cell chirwere chinoitika mune mumwe pavane 365 vokuzvarwa vemuAmerica vokuAmerica; vakakomberedza 1 mu13 vacheche vacheche muU.S. vanozvarwa vaine jeko masero maitiro.

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Mhando dzechirwere chemasero chirwere

Sickle cell chirwere izwi rakapamhamha rinosanganisira akasiyana mafomu eiyo mamiriro. Aya ndiwo marudzi akajairika eSCD.



  • Sickle cell anemia (HbSS):Vanhu vane fomu iyi yeSDD vanowana nhaka imwe yemagenera kubva kumubereki mumwe nemumwe. Sickle cell anemia inowanzo kuve yakakomba fomu yechirwere chesero.
  • Sickle hemoglobin C chirwere (HbSC):Nerudzi urwu rweSCD, imwe siketi yesero geni inotorwa kubva kumubereki mumwe, uye kubva kune imwe, geni rehemoglobin isina kujairika inonzi C. Inowanzo kuve fomu yakapfava yeCDD.
  • Sickle beta thalassemia (HbS beta thalassemia):Vanhu vane fomu iyi yeSDD vanowana nhaka imwe yeresa geni kubva kune mumwe mubereki uye rimwe geni re beta thalassemia ( mhando yekushaya ropa ) kubva kune mumwe mubereki. Kune mhando mbiri dze beta thalassemia: 0 uye +. Hbs beta 0-thalassemia ndiyo yakanyanya mhando yechirwere chesero, nepo Hbs beta + iri yakaoma.

Zviratidzo zvechirwere chemasero echirwere

Vanhu vane chirwere chejeresa masero kazhinji vanotanga kuratidza zviratidzo zvechimiro kana vari vadiki, vangangoita mwedzi mishanu. Vacheche vadiki pane zera iri havaratidze zviratidzo nekuti fetus hemoglobin inodzivirira masero matsvuku eropa kubva kuhuro. Sezvo pavanokura, hemoglobin yejere inotsiva mhando yemucheche.

Zviratidzo uye kunetsekana kweSCD zvakasiyana kune munhu wega wega uye zvinogona kusiyana kubva pakunyoro kusvika pakuoma. Dambudziko rakajairika rechirwere cheseroye marwadzo akakomba, anonzi episodes ekurwadziwa kana matambudziko ekurwadziwa, ayo anogona kubva kubva pakapfava kusvika pakakomba. Zvinoitika kana masero ejeko achipfuura nemumidziyo midiki yeropa, akanamira, uye achivhara kuyerera kweropa. Izvi zvinonzi sickle cell dambudziko uye ndicho chinonyanya kukonzera kugamuchirwa kuchipatara pakati pevarwere veSCD. Zvimwe zvinetso zvinosanganisira:

  • Ruoko-tsoka tsoka: Uku kuzvimba mumaoko nemakumbo kazhinji chiratidzo chekutanga cheSCD.
  • Anemia:Kushaikwa kwemasero matsvuku eropa kutakura okisijeni kunokonzeresa kushomeka kweropa uko vanhu vane SCD vanonzwa kuneta, kutenderera kana musoro wakare, uye kunetseka kufema mumatambudziko akakomba.
  • Utachiona :Vanhu vane SCD, kunyanya vana vadiki, vari panjodzi yekuwedzera hutachiona nekuda kwekukuvara kwepleen.
  • Acute chest syndrome :Aya mamiriro anotaridzika uye anonzwa akafanana nemabayo nekurwadziwa pachipfuva, kukosora, kunetseka kufema, uye fivha. Inogona kutyisidzira hupenyu.
  • Splenic kudzora :Aya mamiriro ekutyisidzira hupenyu anoitika kana huwandu hwakawanda hwemasero ejeko abatwa mupepa uye oita kuti iwedzere.
  • Maronda emakumbo :Kushungurudzika, hutachiona, kuzvimba, uye kutenderera kwakashata mumidziyo yeropa kunokonzera maronda muzasi pegumbo.

Chirwere chesero chemasero chinowanikwa sei?

Kuongororwa ropa ndiyo chete nzira yekuongorora chirwere chemajeresa kana kuona kana mumwe munhu akatakura jeko remajeresa raanogona kupfuudza kumwana.



Anopa hutano anotora ropa kubva kuruoko mutsinga muvakuru, uye chigunwe kana chitsitsinho muvacheche nevana vadiki. MuU.S., vana vachangozvarwa vanogara vachiongororwa chirwere chesero. Mushure mekudhonzwa kweropa, lab inovhenekesa muyenzaniso weiyo hemoglobin isina kukwana.

Nguva zhinji, vanhu vanowanikwa vakatakura jeko masero ejeni vanobva vaendeswa kunachipangamazano anobatsira varwere nemhuri dzavo kuti vanzwisise ruzivo rwemajini uye kuti zvinokanganisa sei kurapwa kwavo kana kubata mamiriro avo.

Sero rekere rinogona kurapwa here?

Kurarama nejeko remasero chirwere kunogona kunetsa, asi zvichiri kugona kuva nehupenyu huzere uye hune hutano. Kwemakore makumi maviri kusvika makumi matatu apfuura, kutsvagisa kwekurapa, kuchengetedza, uye dzidzo nezvechirwere chesero masero zvakabatsira kusimudzira maonero evanhu vane chirwere ichi.



Huwandu hwekufa kweSCD muvechidiki vanhu vane akadonha zvakanyanya kwemakumi emakore apfuura, kubva pa9.3% muna 1975 kusvika 2.6% muna 1989 . TO ongororo ichangoburwa inoratidza huwandu hwevakafa muvana vemuAmerica vekuAmerica vachidonha neanosvika 68% panguva yemakore gumi nematanhatu. Vatsvagiridzi vanoti kuderera kune mushonga wepneumococcal wakaburitswa muna 2000. Vanhu vane SCD havana kudzivirirwa zvakanyanya kubva kuzvirwere zvakakomba uye zvinouraya zvakaita semabayo.

Kunyangwe hazvo vana vane chirwere chekaradhi masero vari kurarama kwenguva yakareba, mamiriro acho achiri kubatana ne hupfupi hupenyu hwevanhu vakuru . Kutsvagurudza kunoratidza huwandu hwepakati pekutarisira hupenyu makore makumi mana nemaviri evakadzi uye makumi matatu nemasere evarume.

Nepo kuchengetwa kwevana kuchikurumidza nenzvimbo nyowani dzekurapa uye varapi vanoshanda, varwere vakuru vachiri kusangana nemipengo mune yehutano system.



Mushure mekunge munhu ane jeko cell asvitsa makore gumi nemasere kana gumi nemapfumbamwe, anofanirwa kusiya nzvimbo yevana, uye zvinosuwisa kuti ndiyo nzvimbo apo vamwe vanhu vanowira padenga, anodaro Barbara Harrison, chipangamazano wemazamu uye director weCommunity Outreach uye Dzidzo ye Nzvimbo yeSickle Cell Zvirwere paHoward University Hospital. Iko hakuna kukwana kwakakwana, vanoziva mahematologist avo vakadzidziswa mune sickle cell chirwere uye vanoziva nematambudziko muvakuru.

Rimwe dambudziko: yekutsvagisa mari. Sickle cell chirwere ndechimwe cheanowanzo tyisidzira hupenyu hutachiona hutachiona muUS Zvakadaro, inogamuchira zvakanyanya kushomeka kwekutsvagisa mari Kupfuura zvimwe zvinokonzerwa nemageneti secystic fibrosis, mutsauko imwe mhosva pahurongwa hwesaruro mune hutano. Iyo mamorekuru uye genesis hwaro hwechirwere chemasero echirwere yaive imwe yekutanga kuwanikwa muzvirwere zvevanhu, zvakadaro, isu tiri kumashure kumashure kusvika pakurapa uye nhanho dzakatorwa kubatsira kurapa mamiriro, Harrison anodaro.

Pari zvino, stem cell transplant (inonziwo bone marrow transplantation) ndiyo chete mishonga yechirwere chesero. Munguva iyi maitiro, vanachiremba vanopa maseru ane hutano (masero maseru) mumuviri kutsiva yakanganisa kana inorwara mongo wemapfupa. Muviri unoita masero eropa mumongo wemapfupa pakati pefupa. Kazhinji kazhinji, vanachiremba vari kushandisa ropa uye mapfupa emuchina kubva kune vanopa vane hutano kurapa SCD, asi varwere vanoda mupi kubva kune mumwe munhu akafanana nemongo wemapfupa. Kuisazve zvinogona dzimwe nguva kukonzera zvakakomba mhedzisiro uye kunyangwe rufu, uye hazvisi kuwanikwa zvakanyanya kunze kweUS neEurope.

Imwe nzira inogona kurapa padhuze ndeye kurapa kwemajini . Inosanganisira kubvisa masero emadzitateguru ayo anoumba ropa uye masero emunodzivirira kubva mumwongo wemapfupa kana ropa uye nekuwedzera geni rinokanganisa muvanhu vane chirwere chemajeko. Vanachiremba vanobva vadzorera maseru kumurwere, izvo zvinotungamira kumuviri kuburitsa anti-sickling hemoglobin. Vatsvagiri vachiri kuitisa kiriniki miedzo, asi mhedzisiro yekutanga inoita seinovimbisa.

Parizvino, mushonga unoshanda wakakosha. Tinoda mamwe marapirwo patiri kushanda pahigh-tech, epamusoro-zviwanikwa marapirwo senge STEM cell transplant uye gene kurapa, Dr. Kutlar anodaro.

Sickle masero ekurapa

Nhanho yekutanga yekurarama hupenyu hwakareba neSCD iri kutsvaga kurapwa kwakanaka kubva kune nyanzvi mune mamiriro. Ipapo, iyo mishonga iri chaiyo kune ako chaiwo zviratidzo.

Hydroxyurea

Iyo yakajairwa kurapwa kwechirwere chesero masero iri hydroxyurea , mushonga uyo vatapi vezvehutano vakatanga kushandisa kurapa cancer. Vanhu vane SCD vanotora diki diki, uye inowedzera fetus hemoglobin nepo ichivharira matambudziko mazhinji eiyo mamiriro- kudzikisa masero esero, kuenda kuchipatara, zviitiko zvemabayo, uye kudiwa kwekuisirwa ropa.

unogona here kutora mushonga weprobiotic nemishonga inorwisa mabhakitiriya panguva imwe chete

Mumakore achangopfuura, makambani emishonga ave nehanya zvakanyanya mukuvandudza marapirwo matsva echirwere chesero. Izvi zvinokodzera, muchidimbu, nekushomeka kwemarapirwo, uye nekuti chirwere chesero rechirwere muenzaniso wekurapa mamwe mamiriro sekuzvimba nezvirwere zvemitsipa yeropa.

Endari (L-glutamine muromo upfu)

Kubva 2017, iyo US Chikafu uye Dhivhosi Kutonga (FDA) yakabvumidza kurapwa kutsva kwechirwere chesero, yekutanga mumakumi emakore. Endari ( L-glutamine poda yemuromo ndeyevanhu vane SCD vane makore mashanu nekure. Mushonga, hupfu hunotorwa nemuromo, hunoshanda se antioxidant kugadzirisa zvinetso zvakakomba zvakabatana nemamiriro acho. Vatsvakurudzi vakaona kuti vanhu vanotora Endari vane mashoma masero esero uye nzendo dzinoenda kuchipatara, pamwe nekugara kwenguva pfupi muchipatara.

Oxbreaker (voxelotor)

Mu2019, iyo FDA inokurumidza-kuteedzera mvumo ye Oxbryta (voxelotor), wekutanga mushonga kurapa zvinokonzeresa sickle cell anemia pane kungoita zviratidzo zvayo. NaOxbryta, masero ejeko haashoma kusunga pamwe nekuumba chimiro chejeko, izvo zvinogona kukonzeresa huwandu hwehemoglobin nekuda kwekuparadzwa kwemasero eropa, Richard Pazdur, MD, director weDFA's Oncology Center of Excellence (OCE) akadaro mune kuburitswa kwenhau kuzivisa kurapwa . Iyi kurapa inopa nzira nyowani yekurapa kune varwere vane chakakomba uye chinotyisa hupenyu mamiriro. Oxbryta ndeye vana nevakuru makore gumi nemaviri zvichikwira.

Adakveo (crizanlizumab-tmca)

Iyo FDA yakabvumidza mumwe mushonga mukupera kwa2019 , Adakveo (crizanlizumab-tmca), iyo inoderedza huwandu hwematambudziko esero mune vanhu vakuru uye vana vanopfuura makore gumi nematanhatu. Mushonga uyu jekiseni, iro vanhu vane SCD vanogashira nguva nenguva, vhiki mbiri dzese pakutanga uyezve voenda kamwe chete pamwedzi.

Sickle Cell Center yeAugusta University yaive chikamu chemuedzo wepasirese wekurapwa uku, uye Dr. Kutlar vanoti maitiro kubva kuvarwere aive akanaka kwazvo. Mushure mekunge kudzidza kwapera, varwere vashoma vakauya kwatiri ndokuti, 'Ndanga ndichinzwa zvirinani zvakanyanya pandakanga ndichiwana izvo infusions. Pane imwe nzira yaungandidzorera nayo pane izvi? ’Saka, muchidimbu, icho chaive chiitiko chakanakisa kubva pakuona kwemurwere.

Sickle cell chirwere kurapwa
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Vanhu vane chirwere chemajeresa vanotorawo-the-counter kana mishonga yemishonga yekurwadziwa kusingaperi uye ingangoda kuwedzerwa ropa kurapa matambudziko senge anemia kana hutachiona.

Sickle cell zviwanikwa

United States Congress yakadoma Nyamavhuvhu seNyika Sickle Cell Yekuzivisa Mwedzi kubatsira kuunza ruzivo kutsvagisa uye kurapwa kwechirwere chesero. Kuti udzidze zvakawanda nezve ichi chiitiko, shanya: