Kuyeza ndi Kupewa Kulimbana Ndi Mliri wa Matenda a Sickle Anemia

Kusinthasintha ndi chinthu chomwe anthu ambiri amawagwirizanitsa ndi achibale awo okalamba, kotero zimakhala zochititsa mantha zomwe ana angakhale nawo. Kukwapula kumachitika kwa ana obadwa ndi ana, koma mwachisangalalo, chiopsezo chiri chochepa (osachepera 1 peresenti ya ana). Matenda a mtima (cardiac (heart)) komanso matenda a mtima wodwalayo (hemoglobin SS kapena sickle beta zero thalassemia) ndizo zimayambitsa matenda ambiri mu ubwana.

Popanda kuyang'anitsitsa bwino, ana khumi ndi anayi alionse omwe ali ndi matenda a sickle cell anemia adzadwala sitiroko ali ndi zaka 20. Pafupifupi mmodzi mwa odwala anayi adzadwala matenda a stroke ali ndi zaka 45. Kumva nkhani zotere zokhudza mwana wanuyo kungakhale kochititsa mantha, koma poyang'ana bwino, ngoziyi ingachepe kwambiri.

N'chifukwa Chiyani Anthu Amene Ali Ndi Matenda Achipatala Ali Pangozi Yowopsa Kwambiri?

Ambiri mwa ana omwe ali ndi kachilombo koyambitsa matenda a anthia omwe ali ndi matenda a ischemic , kutanthauza kuti kuyenda kwa magazi sikungathe kufika kumalo amodzi a ubongo. Maselo a tizilombo amawononga mitsempha ya mitsempha yayikulu (mitsempha ya magazi yomwe imanyamula oksijeni kumatumbo) a ubongo, kuwapangitsa kukhala opapatiza kwambiri. Mitsempha yaing'ono yamagazi imakhala yotsekedwa ndi khungu la maselo. Izi zikachitika, kuthamanga kwa magazi kumatsekedwa ndipo mpweya sungaperekedwe ku malo ena a ubongo, kuwononga.

Zizindikiro

Kukwapula kwa ana omwe ali ndi matenda a sickle cell akuwoneka mofanana ndi zikwapu kwa akulu akulu.

Ana akhoza kuwona:

Kodi Ndiyenera Kuchitanji Ngati Ndikuganiza Kuti Wina Ali ndi Stroke?

Itanani 911. Kukwapulidwa, mosasamala kanthu komwe, ndizochitika mwamsanga zamankhwala.

Kuchiza koyambirira ndizofunikira kuti tipewe mavuto a nthawi yaitali.

Zowopsa

Chiopsezo cha sitiroko ya ischemic ndi yaikulu kwa ana osakwana zaka khumi. Kuopsa kumeneku kumachepa mpaka kumapeto kwa zaka 30, ndiye kuti chiopsezo chikuwonjezanso. Kuopsa kwa zilonda zam'mimba (matenda omwe amayamba chifukwa cha magazi) ndi achinyamata akuluakulu komanso achinyamata.

Kuopsa kwa kupweteka kwapakati kumawopsa kwambiri kwa odwala omwe ali ndi hemoglobin SS ndi sickle beta zero thalassemia. Kuopsa kwa odwala matenda a hemoglobin SC ndi sickle beta kuphatikizapo thalassemia (makamaka kwa ana aang'ono) ndizochepa kwambiri, kotero kuyesera sikungakonzedwe mwa odwalawa pokhapokha pali zodandaula zina.

Kupewa

Kumapeto kwa zaka za m'ma 1980 ndi kumayambiriro kwa zaka za m'ma 1990, akatswiri ofufuza anapeza kuti dcpleral doppler (TCD) ingagwiritsidwe ntchito poyang'anira odwala ndi matenda a anthia omwe akudwala kuti awononge chiopsezo cha ischemic. TCD ndi ultrasound yosasokonezeka yogwiritsidwa ntchito poyeza kutaya kwa magazi kudzera m'mitsempha yayikuru ya ubongo. Pochita izi, kafukufuku wa ultrasound amaikidwa pa akachisi omwe fupa la fupa limakhala lochepa kwambiri, kulola wogwira ntchito kuyeza liwiro la magazi. Malingana ndi mfundo zimenezi, TCD zingatchulidwe ngati zachilendo, zovomerezeka, ndi zachilendo. Ngakhale ana omwe ali ndi makhalidwe abwino a TCD ali pangozi yapamwamba yokhala ndi stroke, awo omwe ali ndi TCD osazolowereka ali pangozi yaikulu ndipo ayenera kuikidwa pa njira yothandizira.

Ngakhale kuti TCD ikuwoneka ngati yosavuta, sikumveka mosavuta. Pali zifukwa zambiri zomwe zingakhudze miyeso ya TCD. Chiwindi ndi matenda zimakweza kakhalidwe ka TCD. Kuphatikizana, kuikidwa magazi kumachepetsa pang'ono makhalidwe a TCD. Mwachidule, mwana wanu ayenera kukhala pa umoyo wawo woyamba pamene TCD ikuchitika.

Kugona kumakhudzanso magazi kupita ku ubongo kuti sedation (kupereka mankhwala kuti athandize wodwalayo kuti asatope / kugona panthawiyi) kapena kugona panthawi yoyezetsa sikunakonzedwe. Ana ang'ono angapeze zovuta kuti agwirizane ndi kukhala chete, koma makolo angathe kuthandiza pogwiritsa ntchito kanema kapena kuwerenga mabuku panthawiyi.

Kodi Ndibwino Bwanji TCD pa Kuzindikira Odwala Amene Ali Pangozi Chifukwa cha Sitiroko?

Kuzindikiritsa za TCD yodalirika komanso kuyambitsidwa mwamsanga kumachepetsa chiopsezo cha kupweteka kwa ana omwe ali ndi sickle cell anemia kuyambira 11% mpaka 1 peresenti. Sikuti ana onse omwe ali ndi TCD osadziwika amatha kupweteka popanda kuchiza, koma chifukwa chakuti sitiroko ikhoza kukhala ndi zotsatira za nthawi yaitali, odwala onse amachitidwa chimodzimodzi.

Kodi Strokes Zimapewa Bwanji Ngati TCD Ndi Yachilendo?

Ngati mwana wanu ali ndi sickle cell anemia ali ndi TCD yodabwitsa, ndi bwino kuti TCD ibwererenso pakatha masabata awiri kapena awiri. Ngati kachiwiri TCD ndi yachilendo, ndibwino kuti ayambe pulogalamu yoika magazi.

Phunziro lachipatala la STOP-1 linasonyeza kuti kukhazikitsidwa kwa pulogalamu yopatsirana yoperewera kwambiri kunachepetsa chiopsezo cha kupwetekedwa. Mankhwala opatsirana odwala nthawi zonse amakhala ndi kuikidwa magazi milungu itatu kapena inayi iliyonse. Cholinga cha kuikidwa magazi ndi kubweretsa chiwerengero cha hemoglobin S kuchoka pa 95 peresenti kufikira osachepera 30 peresenti, kuti achepetse chiopsezo cha maselo okhwima omwe amaletsa kutuluka kwa magazi m'mitsempha ya ubongo.

Kodi Mwana Wanga Adzafunika Kukhala Wosasunthika Nthawi Zonse?

Mwinamwake ayi. M'mayesero amodzi omwe apangidwa ndi TWiTCH, odwala enieni (omwe amachokera ku zinthu monga hemoglobin S, kujambula kwa ubongo, zikhulupiliro za TCD zomwe zinabwereranso mwachibadwa) zinatha kusintha kuchokera kuchipatala chonyansa kupita kuchipatala cha hydroxyurea . Odwalawa anasiya kuyamwa pang'onopang'ono pamene mankhwala a hydroxyurea amakula pang'onopang'ono.

Odwala omwe ali ndi kusintha kwakukulu m'mitsempha ya m'mitsempha mu ubongo angafunike chithandizo chamankhwala chosatha nthawi yaitali, mofanana ndi odwala omwe ali ndi stroke.

Zotsatira:

George A. Kupewa kupwetekedwa (koyamba kapena kobwerezabwereza) mu matenda a ngodya. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Idapezeka pa May 11, 2016.)