Ukuhlola nokukhusela iStroke kwi-Sickle Cell Anemia

Isibetho siyinto eninzi abantu abadibeneyo kunye neentsapho ezizaliseleyo, ngoko kusenokubakho ukutshatyalaliswa ukuba abantwana banako. Izibetho zivela kwiintsana kunye nabantwana, kodwa ngelanga, ngokubanzi, umngcipheko uphansi (ngaphantsi kwe-1 ekhulwini yabantwana). Isifo senhliziyo (i-heart) nesifo se-anemia yesigane (i-hemoglobin SS okanye i-beta ye-beta zero thalassemia) zizona zizathu eziqhelekileyo zokubethwa kwintlungu ebuntwaneni.

Ngaphandle kokuhlola okufanelekileyo, iipesenti ezilishumi elinanye zezingane ezine-anemia ye-sickle cell ziza kuhlaselwa yintlungu engama-20 ubudala. Phantse enye yezigulane ezine ziza kuba ne-stroke ngeminyaka engama-45. Ukuva olu hlobo lweendaba malunga nomntwana wakho omncinci kunokumangalisa, kodwa ngokuphonononga kakuhle, lo mngcipheko ungancitshiswa kakhulu.

Kutheni Abantu Abagulayo I-Anemia Esemngciphekweni?

Uninzi lwabantwana abane-anemia ye-anemia ye-anemia ye-anemia ye-anemia ye- acrochemism , oku kuthetha ukuba ukuhamba kwegazi akunakufikelela kwindawo enye yengqondo. Iiseli zesifo ziwonakalisa umbane weetriyiti ezinkulu (iimithambo zegazi ezithwala i-oksijini kwimizimba) yengqondo, ezibangela ukuba zibe nzima kakhulu. Le mijelo yamanzi emancinci inokuthi ivaliwe yi-clump yeesele zegale. Xa kwenzeka, ukuphuma kwegazi kuvaliwe kwaye i-oksijini ayikwazi ukuhanjiswa kwindawo ethile yengqondo, ebangela umonakalo.

Iimpawu

Izibetho kubantwana abane-anemia ye-sickle cell ekhangeleka njengento ebethayo kubantu abadala.

Abantwana banako ukuva:

Yintoni endiyenzayo ukuba ndiyenze ukuba Ndicinga Ngomntu Ofumana I-Stroke?

Ifowuni ye-911. Ukubetha, kungakhathaliseki ukuba yintoni isizathu, kuyingozi yonyango.

Uphulo lwangokokuqala luphambili lokuthintela iingxaki zexesha elide.

Izinto zobungozi

Umngcipheko wesifo sokunyuka kwe-ischemic uphezulu kunabantwana abangaphantsi kweminyaka elishumi ubudala. Lo mngcipheko wehla ude uneminyaka engama-30, kwaye ingozi iyanda kwakhona. Umngcipheko wezibetho ezinzima (izibetho ezibangelwa ukuphuma kwegazi) ziphezulu kakhulu kulutsha kunye nabantu abadala.

Umngcipheko wokuba uhlangothi luphezulu kakhulu kwizigulane ezine-hemoglobin SS kunye ne-beta ye-beta zero thalassemia. Umngcipheko kwizigulane ezine-hemoglobin SC kunye ne-sickle beta kunye ne-thalassemia (ngokukodwa kubantwana abancinci) yinto encinci kakhulu, ngoko ke ukucocwa akukhuthazwa kwezi zi gulane ngaphandle kokuba kukho ukukhathazeka okongeziweyo.

Thintelo

Ngasekupheleni kweminyaka yama-1980 kunye neye-1990s, abaphandi banqume ukuba i-transcranial doppler (TCD) ingasetyenziselwa ukukhenkca izigulane ezine-anemia yesalente yegaleki ukucaca umngcipheko wesifo soqhenqa. I-TCD yi-ultrasound engeyiyo ingavumelekanga esetyenziselwa ukulinganisa ukugeleza kwegazi ngeemibhobho emikhulu yengqondo. Ukwenza oku, iprojeysi ye-ultrasound ifakwa kwiitempileni apho ithambo lekrele lincinci, ukuvumela uchwepheshe ukuba alinganise isantya sokuhamba kwegazi. Ngokusekelwe kwezi zixabiso, i-TCD ingabhalwa njengento eqhelekileyo, enemiqathango, kwaye engaqhelekanga. Nangona abantwana abaneempawu ze-TCD ezinemeko basengozini encinci yokuba ne-stroke, abo abane-TCD engaqhelekanga basengozini enkulu kwaye kufuneka bafakwe kwisicwangciso sokhathalela unyango.

Nangona i-TCD ibonakala ngathi ivavanyo olulula, akulula nje njengoko kuzwakala. Kukho izinto ezininzi ezinokuchaphazela imilinganiselo ngexesha le-TCD. Ifiva kunye nezifo ziphakamisa okwakhayo ixabiso le-TCD. Ngokuchasene, ukumpontshelwa igazi okwexeshana kweTCD. Okubalulekileyo, umntwana wakho kufuneka abe sempilo yabo yesiseko xa i-TCD isenziwa.

Ukulala kuphazamisa ukuhamba kwegazi kwi-brain so sedation (ukunika amayeza ukunceda isigulane ukuphumla / ukulala ngexesha lwenkqubo) okanye ukulala ngexesha loviwo alucebisi. Abantwana abancinci banokufumana kunzima ukusebenzisana nokuhlala, kodwa abazali banokunceda ngokudlala i-movie okanye ukufunda iincwadi ngexesha le nkqubo.

Ilungile kangakanani i-TCD ekuboneni izigulane ezisengozini yokubethelwa kwesisu?

Ukuchongwa kwe-TCD engaqhelekanga elandelwa ukuqaliswa kwonyango ngokukhawuleza kunciphise umngcipheko wokushaya isifo kubantwana abane-anemia yesalente yesigane ukusuka kwi-11% ukuya kwi-1 ekhulwini. Akunabo bonke abantwana abanee-TCD ezingavamile abaya kuba ne-stroke ngaphandle kwonyango, kodwa ngenxa yokuba isifo sinokubangelwa yimiphumo emide yesikhathi eside, zonke izigulane ziphathwa ngokufanayo.

Ziya kuthinteka njani iiStrokes ukuba i-TCD ayimfuneko?

Ukuba umntwana wakho ene-anemia yesalente yesigulo aneTCD engavumelekanga, kucetyiswa ukuba iTCD iphindwe rhoqo kwiiveki ezimbini. Ukuba kwakhona i-TCD ayiqhelekanga, kuyacetyiswa ukuba iqaliswe kwiprogram engapheliyo yokumpontshelwa.

Ucwaningo lwe-STOP-1 lwezonyango lubonise ukuba ukuqaliswa kwenkqubo yokuxilongwa okungapheli kuncitshisi kakhulu umngcipheko wesifo. Unyango olungapheliyo lokumpontshelwa igazi luquka ukunyelwa igazi nganye kwiiveki ezintathu ukuya kwezine. Injongo yokumpontshelwa kukuzisa i-hemoglobin S iphesenti ukusuka kwiipesenti ezingama-95 ukuya ngaphantsi kwama-30 ekhulwini, ukunciphisa umngcipheko weeseli zamagalelo ezivimba ukuhamba kwegazi kwimibhobho yengqondo.

Ngaba Umntwana Wami Uya Kuhlala Efuna Ukutshintshiswa?

Mhlawumbi hayi. Kwimvavanyo ye-TWiTCH yakutshanje yamatyala amaninzi (esekelwe kwizinto ezinjengezinga le-hemoglobin S, ukucinga ngomqondo wengqondo, ixabiso le-TCD elibuyela kwinto evamile) bakwazi ukutshintshwa kwindlela yokwelashwa okungapheliyo kwi- hydroxyurea unyango. Ezi zi gulane zancinciwe ngokukhawuleza ukuxilongwa njengoko unyango lwe-hydroxyurea lwanyuka kancinci.

Izigulane ezineentsholongwane ezibalulekileyo kwimida yegazi kwingqondo zingadinga unyango olungapheliyo lwexesha lokugqibela, olufana nezigulane ezine-stroke.

Imithombo:

UGeorge A. Ukuthintela ukushaywa kwesifo (kuqala okanye ngokuphindaphindiweyo) kwisifo segciwane lesigane. Ku: U-UpToDate, i-Post TW (Ed), UpToDate, i-Waltham, MA. (Kufumaneka ngoMeyi 11, 2016.)