Kodi mukudziwa za matenda a Sickle Cell Anemia? Tiyeni tikambirane za izi!

Kodi mudamvapo za dzina lakuti 'Sickle Cell Anemia'? Mwina dzinali likumveka lachilendo pang'ono, kapena loopsa pang'ono. Koma ndi matenda a majini okhudzana ndi magazi athu. Mwachidule, chomwe chimachitika pamenepa ndichakuti maselo athu ofiira amagazi, omwe ndi maselo ofiira m'magazi, amasintha mawonekedwe. Monga chikwakwa (ndicho chifukwa chake chimatchedwa 'sickle'). Kusinthaku kungayambitse mavuto osiyanasiyana azaumoyo. Ndiye, kodi tikambirane izi mwatsatanetsatane lero?

Kodi matenda a Sickle Cell Anemia ndi chiyani? Kodi kwenikweni chimachitika ndi chiyani?

Matenda a sickle cell anemia ndi omwe madokotala amatcha mtundu woopsa kwambiri wa gulu lalikulu lotchedwa 'Sickle Cell Disease'. Uwu ndi matenda obadwa nawo, kutanthauza kuti, matenda obadwa nawo m'magazi . Chifukwa chachikulu cha izi ndi kusintha kwa majini. Chifukwa cha kusintha kwa majini kumeneku, maselo ofiira amagazi omwe amapangidwa m'thupi lathu nthawi zambiri samakhala ozungulira, koma amapindika ndi kutambasulidwa ngati chikwakwa (monga chilembo C). Madokotala amatchanso "maselo a sickle".

Tsopano taonani, maselo ofiira athu abwinobwino ndi ozungulira komanso osinthasintha, kotero amatha kuyenda mosavuta kudzera m'mitsempha yathu yofewa yamagazi ndikunyamula mpweya kupita ku gawo lililonse la thupi, chiwalo chilichonse, ndi minofu iliyonse. Koma maselo ofiira awa ooneka ngati chikwakwa ndi olimba komanso omata . Chifukwa chake sayenda mosavuta kudzera m'mitsempha yamagazi, koma amakonda kukodwa m'malo ena. Monga kuchuluka kwa magalimoto pamsewu. Chinanso n'chakuti, maselo 'okhala ndi chikwakwa' awa amawonongeka ndikufa mwachangu kuposa maselo ofiira amagazi abwinobwino. Chifukwa cha zonsezi, kuchuluka kwa maselo ofiira amagazi abwinobwino m'thupi lanu kumachepa, zomwe zimapangitsa kuchepa kwa magazi m'thupi. Ndiko kuti, kusowa kwa magazi.

Kale, makanda obadwa ndi matendawa anali ndi mwayi wochepa kwambiri wopulumuka mpaka kukula. Komabe, chifukwa cha kupita patsogolo kwa zamankhwala, kuzindikirika msanga, komanso njira zatsopano zochiritsira , izi zasintha kwambiri. Anthu ambiri tsopano amakhala ndi moyo wa zaka 50 kapena kuposerapo.

Kodi matendawa ndi ofala bwanji ku Sri Lanka? Ndani ali ndi mwayi waukulu woti adwale matendawa?

N'zovuta kupeza ziwerengero zokhudza matendawa ku Sri Lanka. Koma mukayang'ana padziko lonse lapansi, matendawa amapezeka kwambiri mwa anthu ochokera ku Africa, Southern Europe, Middle East kapena Asian Indian. Izi zikutanthauza kuti pali kulumikizana kwa majini.

Kodi zizindikiro za sickle cell anemia ndi ziti?

Zizindikiro za matendawa zimatha kusiyana malinga ndi munthu, koma pali zizindikiro zina zodziwika bwino:

• Kutopa chifukwa cha kuchepa kwa magazi m'thupi: Matendawa akachitika mwa ana aang'ono, amatha kulira pafupipafupi, kumva kudwala, komanso kukana kuyamwitsa.
• Matenda opatsirana pafupipafupi:Matendawa amawononga ndulu yathu. ndulu ndi gawo lofunika kwambiri la chitetezo chathu chamthupi. Choncho ikakhala yofooka, pamakhala mwayi waukulu wopeza matenda ndi matenda. Komabe, chifukwa cha maantibayotiki ndi katemera omwe alipo tsopano, chiopsezochi chachepa kwambiri.
• Ululu: Ichi ndi chimodzi mwa zizindikiro zofala komanso zosasangalatsa za matendawa. Minofu ya thupi siilandira mpweya wokwanira, zomwe zimayambitsa kuwonongeka kwa minofu. Kuwonongeka kumeneku ndi komwe kumayambitsa ululu. Malo ofala kwambiri a ululu ndi manja, miyendo, chifuwa, ndi msana. Nthawi zina ululu ungayambe pang'ono pang'onopang'ono ndikuwonjezeka pang'onopang'ono, kapena mwadzidzidzi ukhoza kukhala ululu wosapiririka.
• Kutupa kowawa m'manja ndi m'mapazi: Ichi ndi chimodzi mwa zizindikiro zoyambirira za matenda a sickle cell anemia mwa mwana. Kutupa kumeneku kumachitika chifukwa maselo ofiira a magazi ooneka ngati sickle amamatira m'mitsempha yamagazi yofewa m'manja ndi m'mapazi a mwana, zomwe zimalepheretsa kuyenda kwa magazi.
• Maso ndi khungu lachikasu lochokera ku jaundice: Chiwindi chathu chimayambitsa kusefa maselo ofiira a magazi. Mu sickle cell anemia, chinthu chotchedwa bilirubin, chomwe chimatulutsidwa kuchokera ku maselo ofiira omwe amafa msanga, chimasonkhana m'thupi, zomwe zimayambitsa jaundice.

Zizindikiro zimenezi nthawi zambiri zimayamba kuonekera mwana akafika miyezi 6 mpaka 9. Pakapita nthawi, pamene chiwerengero cha maselo osazolowereka (oopsa) m'thupi chikuwonjezeka, zizindikirozo zimatha kusintha ndikukhala zoopsa kwambiri.

N’chifukwa chiyani matenda a sickle cell anemia amayamba? N’chiyani chimayambitsa vutoli?

Kuchepa kwa magazi m'thupi chifukwa cha kusintha kwa majini m'majini athu. Makamaka, pali jini yotchedwa HBB gene. Jini iyi imatilangiza kupanga puloteni yotchedwa beta-globin, yomwe ndi gawo la hemoglobin yathu. Hemoglobin, monga mukudziwa, ndi chinthu chofunikira kwambiri chomwe chimathandiza maselo ofiira athu kunyamula mpweya.

Choncho, kuti mupeze matenda a sickle cell anemia, muyenera kulandira majini omwewo osinthidwa kuchokera kwa amayi ndi abambo anu . Nthawi zina, ngati mulandira majini awiri osinthidwa kuchokera kwa makolo onse awiri, mutha kukhala ndi mitundu ina, mwina yosakhala yovuta kwambiri, ya matenda a sickle cell. Madokotala amatcha izi 'variant genetic mutations'.

Ndi mavuto ati omwe angachitike chifukwa cha vutoli?

Kuchepa kwa magazi m'thupi (sickle cell anemia) kungayambitse mavuto aakulu, nthawi zina omwe amaika moyo pachiswe . Mwachitsanzo, anthu omwe ali ndi matendawa nthawi zambiri amafunika chithandizo chamankhwala chadzidzidzi komanso kugonekedwa m'chipatala, makamaka matenda otchedwa acute chest syndrome (ACS) ndi vaso-occlusive crisis (VOC).

Pali zovuta zina:

• Matenda a impso osatha: Matendawa amatha kuchitika impso zikapanda kulandira mpweya wokwanira, zomwe zimapangitsa kuti minofu iwonongeke.
• Ma retina olekanitsidwa: Izi zitha kuchitika ngati maselo otupa atseka mitsempha yamagazi m'diso.
• Kupweteka kwa mbolo (priapism): Matenda omwe amayamba chifukwa cha kutsekeka kwa mitsempha yamagazi mu mbolo.
• Kuchotsa ma splenic: Ngati ma sickle cells agwidwa mu ndulu ndikuletsa kuyenda kwa magazi, izi zingayambitse kuchepa kwa magazi mwadzidzidzi komanso kwakukulu.
• Stroke: Aliyense amene ali ndi vuto la kuchepa kwa magazi m'thupi, ngakhale ana aang'ono ( stroke ya ana), ali pachiwopsezo.

Kodi Acute Chest Syndrome (ACS) ndi chiyani?

Imeneyi ndi vuto lofala kwambiri la sickle cell anemia. Ndilonso vuto lalikulu la imfa komanso lachiwiri lalikulu la kugonekedwa m'chipatala. Izi zimachitika pamene sickle cells zimasonkhana pamodzi ndikutseka mitsempha yamagazi m'mapapo. Zizindikiro zake ndi izi:

• Kupweteka pachifuwa mwadzidzidzi.
• Chifuwa.
• Malungo.
• Kuvuta kupuma.

Kodi Vaso-occlusive Crisis (VOC) ndi chiyani?

Apa ndi pamene maselo otupa amatseka mitsempha yamagazi. Madokotala amatchanso izi kuti "mavuto aakulu a ululu." Mu VOC, ululu wosapiririka ukhoza kuchitika m'manja, miyendo, pansi pa msana, ndi m'mimba.

Nthawi zina madokotala amatcha ma VOC kuti "matenda osawoneka." Chifukwa, nthawi zambiri, chizindikiro chokhacho ndi ululu. Palibe chizindikiro cha kuvulala kapena matenda ena. Zimangopweteka.

Chithandizo chachikulu cha ululu waukuluwu ndi mankhwala ochepetsa ululu a opioid. Komabe, kafukufuku wina wasonyeza kuti kufunika kwa opioid kumeneku kungayambitse kudzipatula kwa anthu omwe ali ndi vuto la kuchepa kwa magazi m'thupi, zomwe zingayambitse kuvutika maganizo ndi nkhawa. Ili ndi vuto lina lomwe mungakumane nalo mukakhala ndi matendawa.

Kodi matendawa amapezeka bwanji? (Kuzindikira)

Madokotala amazindikira matenda a sickle cell anemia poyesa kaye. Angamve ndulu yanu kapena chiwindi chanu. Adzakufunsani za zizindikiro zanu, makamaka kupweteka m'manja ndi miyendo yanu, komanso za m'mimba mwanu. Adzakufunsaninso za matenda anu akale komanso ngati mudadwalapo matenda enaake. Kenako angachite mayeso monga:

• Kuchuluka kwa Magazi Okwanira (CBC): Izi zimaphatikizapo mayeso angapo omwe amayang'ana makamaka maselo ofiira a magazi anu.
• Hemoglobin electrophoresis: Yotchedwanso high-performance liquid chromatography, mayesowa amafufuza hemoglobin yanu kuti azindikire ndikuyesa mtundu wosadziwika wa hemoglobin womwe umayambitsa matenda a sickle cell anemia.
• Mayeso a majini: Dokotala wanu angakupatseni mayesowa kuti aone ngati muli ndi kusintha kwa majini komwe kumayambitsa matenda a sickle cell anemia.

Kodi mankhwala ake ndi otani? Kodi angachiritsidwe?

Chithandizo cha matenda a sickle cell anemia chimadalira zizindikiro zanu komanso thanzi lanu lonse. Mwachitsanzo, ngati muli ndi mavuto aakulu monga acute chest syndrome, nthawi zambiri mumakhala ndi ululu waukulu, kapena ngati muli ndi vuto monga kufooka kwa ziwalo, dokotala wanu angakulangizeni kuti mupatsidwe allogeneic stem cell. Iyi ndiyo njira yokhayo yochiritsira matenda a sickle cell anemia kwathunthu .

Mankhwala ena ndi awa:

• Kuikidwa magazi.
• Maantibayotiki oletsa matenda.
• Mankhwala omwe amachepetsa zizindikiro zinazake. Ena mwa iwo ndi awa:
• Hydroxyurea: Iyi ndi mankhwala oletsa khansa. Amagwiritsidwa ntchito kwa makanda a miyezi 6-9, ana, ndi akuluakulu. Angachepetse kuchuluka kwa mavuto akuluakulu ndikuchepetsa zizindikiro za kuchepa kwa magazi m'thupi.
• Voxelotor: Izi zimaletsa maselo ofiira a magazi kusanduka maselo ofiira. Zimaperekedwa kwa ana azaka zopitirira 4.
• Chithandizo cha L-glutamine: Mankhwalawa, omwe amaperekedwa kwa ana azaka zopitilira 5 komanso akuluakulu, amaletsa maselo otupa kuti asakule kwambiri.
• Crizanlizumab-tmca: Izi zimachepetsa kuchuluka kwa VOC/acute pain crisis. Zimaperekedwa kwa anthu azaka zopitilira 16.

Kodi moyo wanga udzakhala bwanji ngati ndili ndi vuto la kuchepa kwa magazi m'thupi (sickle cell anemia)?

Kwa anthu ambiri, matenda a sickle cell anemia ndi matenda osatha . Izi zikutanthauza kuti mudzafunika chithandizo chamankhwala ndi chithandizo m'moyo wanu wonse. Zizindikiro zimatha kukhala zochepa kwa ena, zochepa kwa ena, komanso zoopsa kwambiri moti zitha kupha ena. Mkhalidwe wa aliyense ndi wosiyana. Choncho lankhulani ndi dokotala wanu za zomwe mungayembekezere. Iye amadziwa bwino thanzi lanu komanso momwe matendawa angakukhudzireni.

Kodi vutoli limakula kwambiri mukamakula?

Inde, pamene tikukalamba, mavuto ena aakulu azaumoyo angabwere chifukwa cha kusapereka mpweya wokwanira m'ziwalo ndi minofu. Anthu omwe ali ndi vuto la kuchepa kwa magazi m'thupi (sickle cell anemia) ali pachiwopsezo chachikulu cha ziwalo zopuwala, kuwonongeka kwa mapapo, impso, ndulu, ndi chiwindi.

Kodi nthawi ya moyo wa munthu amene ali ndi matendawa ndi yotani?

Zinthu zili bwino kwambiri tsopano kuposa kale. Pozindikira msanga ndi kulandira chithandizo kuti achepetse mavuto , anthu omwe ali ndi matenda a sickle cell anemia amatha kukhala ndi moyo mpaka zaka 50. Anthu ena amakhala ndi moyo wautali. Ngati muli ndi mafunso okhudza izi, lankhulani ndi dokotala wanu. Iye akhoza kukufotokozerani.

Kodi pali njira yopewera matendawa kuti asakule?

Matenda a sickle cell anemia ndi matenda obadwa nawo, kotero sangathe kupewedwa.Komabe, mutha kuyezetsa magazi kuti muwone ngati muli ndi kusintha kwa majini komwe kumayambitsa matenda a sickle cell anemia. M'maiko ambiri otukuka, mwana aliyense amayezetsa matendawa akangobadwa. Chofunika kwambiri ndikupeza matendawa msanga ndikuyamba kulandira chithandizo .

Kodi ndingakhale bwanji bwino ndi matendawa? (Kudzisamalira)

Mankhwala atsopano athandiza anthu omwe ali ndi matenda a sickle cell anemia kukhala ndi moyo wabwino kwambiri. Ngati muli ndi matendawa, ganizirani izi:

• Funani chithandizo chamankhwala cha akatswiri: Ndikofunikira kufunafuna chithandizo kuchokera ku gulu la akatswiri a zamagazi.
• Pitani kuchipatala nthawi zonse: Kukhala ndi ubale wabwino ndi dokotala wa banja lanu komanso kupita kukayezetsa nthawi zonse kungathandize kupewa mavuto aakulu.
• Ganizirani za kuchepetsa ululu: Ululu ndi gawo lofala la matendawa. Funsani upangiri kwa katswiri wochepetsa ululu.
• Pezani chithandizo cha thanzi la maganizo: Nthawi zina matendawa angayambitse kudzipatula, kukhumudwa, komanso nkhawa. Ngati mukumva choncho, lankhulani ndi dokotala.
• Dzitetezeni ku matenda: Lankhulani ndi dokotala wanu za katemera. Chitanipo kanthu kuti mudziteteze ku matenda.
• Samalani ndi malo omwe muli: Ndikofunikira kwambiri kuti anthu omwe ali ndi vuto la kuchepa kwa magazi m'thupi azikhala ndi kutentha kwabwino m'thupi. Pewani malo otentha kwambiri komanso ozizira kwambiri.
• Yang'anani mayeso azachipatala: Madokotala ndi ofufuza nthawi zonse amayesa njira zatsopano zochizira matenda. Mungakhale ndi mwayi wochita nawo chimodzi.

Kodi ndiyenera kupita kwa dokotala liti?

Ngati mukuona kuti zizindikiro zanu zikuipiraipira, kapena ngati mukuyamba zizindikiro zatsopano, pitani kwa dokotala nthawi yomweyo .

Kodi ndi liti pamene muyenera kupita ku Chipinda cha Emergency?

Kuchepa kwa magazi m'thupi kungayambitse matenda aakulu. Pitani kuchipatala mwamsanga ngati muli ndi zizindikiro izi:

• Zizindikiro za kuchepa kwa magazi m'thupi (kutopa kwambiri, kupuma movutikira, kugunda kwa mtima kosasinthasintha).
• Malungo opitirira madigiri 38.5 Celsius (101.3 Fahrenheit).
• Kupweteka pachifuwa.
• Chifuwa.

Vutoli limawonjezeranso chiopsezo cha sitiroko. Siroko ndi vuto lachipatala . Ngati muli ndi zizindikiro zotsatirazi, pitani kuchipatala nthawi yomweyo:

• Mavuto okhudzana ndi kulinganiza bwino thupi kapena kugwirizana kwa thupi.
• Kuona zinthu molakwika kapena kuona zinthu ziwiri.
• Chisokonezo.
• Kufooka kapena dzanzi mbali imodzi ya thupi.
• Kuvuta kulankhula (kugwedezeka).

Pomaliza, zinthu zingapo zoti muzikumbukira

Matenda a sickle cell anemia ndi vuto lovuta kwambiri. Zaka zapitazo, makanda obadwa ndi matendawa anali ndi chiyembekezo chochepa kwambiri chopulumuka. Koma,Kafukufuku wazachipatala wapita patsogolo kwambiri m'zaka khumi zapitazi, kotero anthu ambiri tsopano akutha kuthana ndi matendawa osatha ndikukhala ndi moyo wabwino.

Ngati inu kapena mwana wanu muli ndi matendawa, pamene mukusangalala ndi kupita patsogolo komwe mwapanga kale, khalani ndi chiyembekezo chamtsogolo. Ofufuza nthawi zonse amayesetsa kupeza njira zatsopano zochizira matendawa. Lankhulani ndi dokotala wanu ndipo dziwani za mayesero atsopano azachipatala. Musataye mtima . Ndikofunikira kwambiri kutsatira upangiri woyenera wazachipatala ndikukhala ndi malingaliro abwino.

Kuchepa kwa magazi m'thupi, kuchepa kwa magazi m'thupi, matenda obadwa nawo, matenda a magazi, kusintha kwa majini, hemoglobin, malangizo azaumoyo