Pane mumwe munhu mumhuri yako ane chirwere che "Sickle Cell Disease" here? Ngatizvionei zvechokwadi!

Nhasi tichataura nezvechirwere chitsva kuvanhu vazhinji, asi chinogona kunge chiri pedyo zvikuru nedzimwe mhuri. Chinonzi Sickle Cell Disease (SCD). Ungave wakambonzwa zita iri kare. Chaizvoizvo chirwere chine chekuita neropa redu, kureva kuti, chine chekuita neropa, uye chinogarwa nhaka. Ngationei kuti chii chaizvo, nei chichiitika, uye zvimwe zvingaitwa nezvazvo.

Chii chinonzi Sickle Cell Disease? Ngatizvinzwisise zviri nyore!

Zvichitaurwa zviri nyore, chirwere chesickle cell (SCD) chirwere chinokanganisa masero matsvuku eropa mumuviri wedu, anobva kuvabereki kuenda kuvana. Ichi chimwe chezvirwere zveropa zvakajairika pasi rose. Zvino tarisai, mukati memasero edu matsvuku eropa mune puroteni inonzi hemoglobin . Iyi hemoglobin inokosha zvikuru. Nekuti, ndiyo puroteni iyi inotakura okisijeni mumuviri wedu wese. Yakafanana ne oxygen taxi. Masero matsvuku eropa emunhu ane hutano anowanzo tenderera uye anochinjika. Semabhora madiki erabha. Nekuda kweizvi, anogona kukambaira nyore nyore nepakati petsinga diki dzeropa mumuviri (tinodzidaidza kuti capillaries) uye anopa okisijeni kunhengo dzedu dzese nematishu. Zvisinei, chinoitika kumunhu ane chirwere chesickle cell ndechekuti pane shanduko diki muhemoglobin iyi. Iyi hemoglobin isina kujairika inonzi hemoglobin S. Izvi zvinoita kuti masero matsvuku eropa ave akaita secrescent, kana kuti akaita sehafu yemwedzi, pachinzvimbo chekutenderera uye anochinjika. Kwete izvozvo chete, asi masero aya akaomarara, haakotame nyore, uye anonamatirana. Fungidzira, chii chinoitika kana masero aya akaita sesickle achifamba netsinga diki dzeropa? Anonamira! Ipapo kufamba kweropa kunokanganiswa. Izvi zvinoreva kuti, nhengo dzedu dzinokosha zvikuru hadziwani okisijeni yakakwana. Ndokusaka matambudziko akakomba akadai sekurwadziwa kwakanyanya, hutachiona hwakasiyana-siyana, kukuvara kwenhengo, uye kusashanda zvakanaka kwemuviri zvichigona kuitika. Chimwe chinhu ndechekuti masero aya akaita sejeko haararame kwenguva yakareba semasero matsvuku eropa akajairwa. Anofa nekukurumidza. Ipapo muviri unogara uine kushomeka kwemasero matsvuku eropa. Izvi ndizvo zvatinodaidza kuti anemia . Chirwere chejeko chirwere chehupenyu hwese. Asi usazvidya moyo, kune mishonga yeizvi. Nekurapa uku, unogona kuderedza zviratidzo uye kuwedzera hupenyu hwako.

Kune mhando dzakasiyana dzechirwere chesickle cell here?

Ehe, kune mhando dzakasiyana dzechirwere chesickle cell. Mhando idzi dzinotsanangurwa nemajini anowanikwa nemunhu kubva kuvabereki vake.

Hemoglobin SS (HbSS)

Ichi ndicho chirwere chesickle cell chakanyanya kuipa . Vanhu vanosvika 65% vane SCD vane rudzi urwu. Vanhu ava vanogara nhaka gene re hemoglobin S kubva kuvabereki vavo vese. Izvi zvinoreva kuti hemoglobin yavo yakawanda kana yese haina kujairika. Izvi zvinoita kuti vave neanemia isingaperi.

Hemoglobin SC (HbSC)

Izvi zviri pakati nepakati kana kuti zvishoma.Rudzi ruri padanho repamusoro. Vanhu vanosvika 25% vane SCD vane izvi. Vanhu ava vanogara nhaka jini re hemoglobin S kubva kune mumwe mubereki uye jini reimwe mhando ye hemoglobin isina kujairika inonzi hemoglobin C kubva kune mumwe mubereki.

Hemoglobin (HbS) Beta Thalassemia

Vanhu ava vanogara nhaka jini re hemoglobin S kubva kumubereki mumwe uye jini risingawanzoitiki rinonzi beta thalassemia kubva kune mumwe mubereki. Kunewo marudzi maviri eizvi:

• "Plus" (HbS beta +): Iyi imhando inobata vanhu vanosvika 8% vane SCD uye inowanzova isina kusimba.
• "Zero" (HbS beta 0): Urwu rudzi rwechirwere runobata vanhu vanosvika 2% vane SCD uye runogona kuva rwakanyanya sechirwere che hemoglobin SS (HbSS).

Mamwe marudzi asingawanzoonekwi

Pamusoro peizvi, kune dzimwe mhando dzisingawanzoonekwi dzakadai sehemoglobin SD (HbSD), hemoglobin SE (HbSE), uye hemoglobin SO (HbSO) . Vanhu ava vanogara nhaka imwe gene rehemoglobin S nerimwe gene risingawanzoonekwi rinonzi D, E, kana O.

Chii chiri musiyano uripo pakati peSickle Cell Anemia neSickle Cell Disease?

Apa ndipo panovhiringidzika vanhu vazhinji. Chirwere cheSickle Cell (SCD) izwi rinowanzo shandiswa pamhando dzakasiyana dzechirwere chesickle cell dzataurwa pamusoro apa. Zvakafanana neamburera. Mamwe marudzi ese anowira pasi peamburera iyoyo. Vanachiremba vanoshandisa izwi rekuti "Sickle Cell Anemia" chete pamhando dzakakomba dzeSCD dzinokonzera anemia . Kureva, mhando dzinonzi hemoglobin SS (HbSS) uye hemoglobin beta zero thalassemia. Wanzwisisa here?

Chii chakasiyana pakati peSickle Cell Trait neDisease?

Vamwe vanhu vane hunhu hwesickle cell chete. Izvi zvinoreva kuti vanogara nhaka yehemoglobin S gene kubva kumubereki mumwe chete . Mumwe mubereki anogara nhaka yejini rine hutano. Kazhinji, vanhu vane hunhu hwesickle cell havaratidzi zviratidzo zvechirwere chesickle cell. Zvisinei, vanogona kutapurira jini iri risingawanzoitiki kuvana vavo. Izvi zvinoreva kuti ndivo vanotakura jini iri. Zvisinei, kashoma, kunyange vanhu vane hunhu hwesickle cell vanogona kuva nematambudziko ehutano kana vakashaya mvura mumuviri zvakanyanya kana kuti vakaita maekisesaizi akaoma . Tsvagiridzo ichiri kuenderera mberi pane izvi.

Chirwere chesickle cell chakajairika zvakadii?

Vaongorori vanofungidzira kuti vanhu vanosvika zviuru zana muUnited States chete vane chirwere chesickle cell. Chinonyanya kuwanikwa pakati pevanhu vane dzinza reAfrica. Chinowanikwawo pakati pevanhu vekuHispanic America, nevanhu vanobva kuMediterranean, Middle East, Indian, neAsia. Kunewo vanhu vane chirwere ichi muSri Lanka.

Chii chinokonzera chirwere chesickle cell?

Chirwere cheSickle cell chinokonzerwa nekuchinja kwemajini mujini rinonzi HBB gene . Iri jini reHBB ndiro rinoita kuti pave nechikamu chimwe chete chehemoglobin. Vanhu vane SCD vanogara nhaka majini maviri eHBB akachinja anogadzira hemoglobin isina kujairika - rimwe kubva kumubereki mumwe nemumwe. Izvi zvinonzi autosomal recessive inheritance. Izvi zvinoreva kuti vabereki vese vemwana ane SCD vane kopi imwe chete yejini rakachinja (zvinoreva kuti vanogona kuva nechiratidzo chesickle cell). Zvisinei, vabereki ivavo havawanzo ratidza zviratidzo.

Ndiani ari panjodzi huru yekubatwa nechirwere chesickle cell (SCD)?

Mamwe mapoka evanhu ane mukana wakakura wekuwana chirwere ichi. Ndiwo:

• Vanhu vane dzinza reAfrica (semuenzaniso maAfrican American).
• MaHispanic Americans muSouth America neCentral America.
• Vanhu vanobva kuMediterranean, Middle East, Indian, uye Asia.

Ndezvipi zviratidzo zvechirwere chesickle cell?

Zviratidzo zvechirwere chesickle cell zvinowanzotanga kuonekwa mwana ava nemwedzi mishanu kusvika mitanhatu . Zviratidzo izvi zvinogona kusiyana kubva pamunhu kusvika pamunhu. Vamwe vanhu vane zviratidzo zvishoma, nepo vamwe vanogona kuva nematambudziko akakomba. Zviratidzo zvikuru zvinogona kuonekwa ndeizvi:

• Kurwadziwa kunowanzoitika.
• Anemia : Izvi zvinogona kukonzera kuneta kwakanyanya, kucheneruka, uye kushaya simba.
• Jaundice : Kutsvuka kweganda uye machena emaziso.
• Kuzvimba kunorwadza kwemaoko netsoka.

Ndezvipi zvinetso zvechirwere ichi?

Chirwere chesickle cell chinogona kukanganisa nhengo dzakawanda dzemuviri. Zvimwe zvinotanga kamwe kamwe (zvakanyanya), nepo zvimwe zvinogara kwenguva refu (zvisingaperi). Matambudziko aya anogona kutanga nekukurumidza uye kwenguva refu.

Marwadzo

Iri ndiro dambudziko rinowanzoitika rechirwere chesickle cell. Marwadzo anoitika kana masero akaita sesickle akabatwa mutsinga dzeropa uye avhara kuyerera kweropa. Ungangove nekurwadziwa kwakakomba kamwe kamwe. Izvi zvinonziwo kurwadziwa, sickle cell crisis, vaso-occlusive crisis (VOC), kana vaso-occlusive episode (VOE) . Marwadzo aya anogona kunge ari madiki kana kuti akaoma, uye anogona kutanga kamwe kamwe uye anogara kwenguva yakareba. Marwadzo anowanzova muchipfuva, kumusana, makumbo, nemaoko. Vamwe vanhu vanogona kunge vaine kurwadziwa kusingaperi , zvinoreva kurwadziwa kunogara kwenguva inopfuura mwedzi mitanhatu.

Kushaya ropa

Chirwere cheSickle cell chinokonzera anemia nekuti masero matsvuku eropa anofa nekukurumidza. Anemia kushaikwa kwemasero matsvuku eropa ane hutano anogona kutakura okisijeni mumuviri wese. NdosakaKuneta kwakanyanya, jaundice, kusagadzikana, dzungu, uye kusuruvara zvinogona kuitika.

Chirwere Chechipfuva Chakanyanya

Iyi injodzi yekurapa inotyisidzira hupenyu . Inogona kukuvadza mapapu, kukonzera kufema kuome, uye kuderedza kupihwa kweokisijeni kune dzimwe nhengo dzemuviri. Dambudziko iri rinoitika kana masero e sickle avhara kuyerera kweropa neokisijeni kuenda kumapapu.

Mavharo Eropa

Kushaya simba kwemasero eropa (sickle cell anemia) kunowedzera njodzi yekugwamba kweropa. Izvi zvinowedzera njodzi yekugwamba kweropa mutsinga yakadzika (deep vein thrombosis - DVT). DVT inogonawo kutyoka yogara mumapapu (pulmonary embolism - PE).

Kurohwa

Kana masero eropa akabatwa mutsinga yeropa inoenda kuuropi, kuyerera kweropa kuenda kuuropi kunovharika. Uropi hahuwani oxygen yakakwana kuti hushande. Izvi zvinogona kukonzera sitiroko . Vanhu vanosvika 10% vane SCD vachava nesitiroko. Vanhu vane sickle cell anemia vari panjodzi huru yesitiroko.

Matambudziko Ekuona

Masero emusana anogona kuvhara mitsipa yeropa mumaziso. Izvi zvinowanzoitika mu retina . Dzimwe nguva hapana zviratidzo uye kuona kunogona kunyangarika kamwe kamwe, uye zvinogona kutokonzera upofu husingaperi.

Kuzvikudza

Kuzvimba kwenhengo yemurume (Priapism) chirwere chinovhara masero akaita sejeko munhengo yemurume, zvichikonzera kusimba kwenhengo yemurume ( priapism ). Pamusoro pekurwadziwa, kuzvimba kwenhengo yemurume (priapism) kunogona kukonzera kukuvara kusingaperi uye kusashanda zvakanaka kwenhengo yemurume . Kuzvimba kwenhengo yemurume (Priapism) kunotora maawa anopfuura mana idambudziko rehutano.

Kukuvara kwenhengo uye kutadza kushanda

Vanhu vane chirwere chesickle cell vari panjodzi yekuva nematambudziko emwoyo, mapapu, itsvo, nedzimwe nhengo dzemuviri nekuti havawani ropa neoxygen yakakwana. SCD inogona kukonzera kutadza kushanda kwenhengo dzakawanda dzemuviri .

Ko hunhu kana chirwere chesickle cell chinokanganisa here pamuviri?

Vakadzi vazhinji vane chirwere chesickle cell vane pamuviri pakanaka. Asi njodzi dzacho dzakakura. SCD inogona kuwedzera njodzi yeBP yakakwira, kuvhara ropa, kubvisa pamuviri, vana vanozvarwa vaine huremu hwakaderera, uye kusununguka nguva isati yakwana . Saka zvakakosha kutevedzera zano rachiremba wako.

Chirwere chesickle cell chinoonekwa sei?

MuSri Lanka, semunyika dzakawanda pasi rose, mwana wese achangoberekwa anoongororwa chirwere chesickle cell sechikamu chekuongororwa kwemwana achangoberekwa . Izvi zvinosanganisira kutora ropa diki kubva pachitsitsinho chemwana uye kuriongorora. Izvi zvinotarisawo zvimwe zvirwere zvakawanda. Kuti asimbise kuongororwa, chiremba wemwana achaita bvunzo ye hemoglobin electrophoresis . Zvakare, chirwere chesickle cell chinogona kuonekwa mwana asati aberekwa kuburikidza nekuongororwa kwemwana asati aberekwa . Miedzo iyi inosanganisira:Izvi zvinosanganisira chorionic villus sampling uye amniocentesis .

Pane mushonga wakakwana wechirwere chesickle cell here?

Ehe, kuisirwa kwemapfupa emumaro , kunozivikanwawo se stem cell transplant, kunogona kurapa chirwere che sickle cell. Izvi zvinosanganisira kutora bone maronda ane hutano kubva kune munhu ane hutano, anoenderana nemajini (senge mukoma kana munun'una) woisa mumurwere. Zvisinei, vanhu vanosvika 18% chete vane SCD ndivo vanogona kuwana mukana wakadaro. Kunewo njodzi nematambudziko ekuisirwa uku. Chiremba wako achakurukura newe izvi.

Ndeipi mishonga yekurapa chirwere chesickle cell?

Kurapa chirwere chesickle cell kunosanganisira mishonga, kuwedzerwa ropa, kuisirwa mubofu, uye kurapwa nemajini . Kurapa kunogona kutanga nemishonga inorwisa mabhakitiriya . Vana vacheche vane SCD yakanyanya vanopiwa mishonga inorwisa mabhakitiriya kaviri pazuva kwemakore mashanu kudzivirira hutachiona.

Mimwe mishonga

Vanhu vazhinji vane SCD vanoshandisa mishonga yekuderedza kuoma kwechirwere chavo nekurapa zviratidzo. Zvimwe zvacho zvinosanganisira:

• Voxelotor: Izvi zvinogona kudzivirira masero matsvuku eropa kuti asave akaita sejeko uye kuti anamatire pamwe chete. Izvi zvinogona kuderedza kuparadzwa kwemamwe masero matsvuku eropa, kunatsiridza kufamba kweropa munhengo dzemuviri, uye kuderedza njodzi yekusakwana kweropa.
• Crizanlizumab: Mushonga uyu unobatsira kudzivirira masero matsvuku eropa akaita sesickle kuti asanamatira pamadziro etsinga dzeropa . Izvi zvinogona kuvandudza kuyerera kweropa uye kuderedza kuzvimba nekurwadziwa.
• Hydroxyurea : Izvi zvinogona kuderedza kana kudzivirira matambudziko akawanda eSCD, akadai sekurwadziwa kunowanzoitika, chirwere chechipfuva chakanyanya, uye kupererwa neropa kwakanyanya.
• L-glutamine: Iyi inoderedza marwadzo. Inogona kubatsira kuderedza huwandu hwemarwadzo aunosangana nawo. Mimwe mishonga inoderedza marwadzo inosanganisira mishonga isingashandisi masteroidal anti-inflammatory (NSAIDs) uye opiates .

Kuwedzerwa ropa

Chiremba wako anogona kukurudzira kuwedzerwa ropa kuti arape uye adzivirire matambudziko eSCD.

• Kuwedzerwa ropa zvakanyanya:Izvi zvinobatsira kurapa matambudziko anokonzera kupererwa kweropa kwakanyanya. Vanachiremba vanogona zvakare kuzvishandisa pamatambudziko akadai sestroke, acute chest syndrome, uye kutadza kushanda kwenhengo dzemuviri.
• Kuisirwa masero matsvuku eropa: Izvi zvinogona kuwedzera huwandu hwemasero matsvuku eropa mumuviri uye kupa masero matsvuku eropa akajairwa asina chimiro chejeko.

Kuisirwa kwemasero emuviri (kunonziwo kuisirwa kwemasero emapfupa)

SCD inogona kurapwa nekuisirwa stem cell. Izvi zvinoda mupi weropa akakodzera (senge mukoma kana munun'una). Tsvagiridzo iri kuitwawo kuti vabereki kana vanun'una vakwanise kuisirwa masero aya zvakanaka. Chiremba wako achakurukura nezvenjodzi nemabhenefiti ekurapwa uku zvichienderana nemamiriro ako chaiwo.

Kurapa Majini

Vaongorori vari kuongorora kurapwa kwemajini kurapa SCD. Izvi zvinosanganisira kugadzirisa jini rehemoglobin risingawanzoitiki kana kuisa jini rehemoglobin rine hutano mumasero emuviri wemunhu. Ruzivo rwekutanga pamusoro peizvi rune tariro huru. Tariro ndeyekuti kurapwa kwemajini rimwe zuva kuchava kurapwa kwakajairika kweSCD.

Izvi zvinogona kudzivirirwa here?

Chirwere cheSickle cell chirwere chinokonzerwa nemajini, saka hachigone kudzivirirwa . Kana uine pamuviri, zvakanaka kutaura nachiremba wako nezvekuongororwa kwemajini kana kupiwa mazano ezvemajini . Izvi zvichakubatsira iwe nemumwe wako kuziva kana uine jini iri uye kuti njodzi yako yekuripfuudza kuvana vako ndeyei.

Chii chingatarisirwa nemunhu ane chirwere chesickle cell?

Vanhu vane chirwere chesickle cell vanogona kurarama kwenguva pfupi pane vanhuwo zvavo. Zvisinei, mishonga mitsva yekurapa SCD yakavandudza zvikuru nguva yekurarama uye mhando yehupenyu . Kana chirwere ichi chikarapwa zvakanaka, vanhu vane chirwere chesickle cell vanogona kurarama kusvika pamakore ekuma50.

Ndingatarisira sei mwana wangu kana aine chirwere chesickle cell?

Kana mwana wako aine chirwere chesickle cell, pane zvinhu zvakawanda zvaunogona kuita kuti ugadzirise chirwere chake:

• Gara uchiendesa mwana wako kuna chiremba.
• Ipa mwana wako majekiseni ese anokurudzirwa panguva yakakodzera.
• Batsirai mwana wenyu kurovedza muviri nguva dzose uye kudya chikafu chine hutano .
• Kana paine dambudziko rekurwadziwa, ipai mwana wenyu mvura yakawanda uye mushonga unodzivirira kuzvimba usingashandisi steroidal (NSAID) . Kana marwadzo asingagone kudzorwa kumba, endai nemwana wenyu kuchipatara kuti anorapwa marwadzo ane simba.

Unofanira kuenda kuEmergency Treatment Unit (ETU) riini?

Chirwere cheSickle cell chinogona kukonzera matambudziko akasiyana-siyana anotyisidzira hupenyu. Kana iwe kana mwana wako mukasangana nechimwe chezviratidzo zvinotevera, fonerai 911 kana kuenda kuchipatara chiri pedyo nekukurumidzira (ER) nekukasika:

• Kurwadziwa kwakanyanya.
• Zviratidzo zvekushaya ropa zvakanyanya: kuneta kwakanyanya, dzungu, uye kufema zvishoma nezvishoma.
• Fivha inodarika 101.3 degrees Fahrenheit (38.5 degrees Celsius).
• Matambudziko ekuona.
• Kutadza kufema zvakanaka.
• Kumira kwenhengo yemurume kunotora maawa mana kana kupfuura (priapism).
• Zviratidzo zvechirwere chechipfuva chakanyanya: kurwadziwa kwechipfuva, kukosora, fivha.
• Zviratidzo zvestroke: Kushaya simba kamwe kamwe kune rumwe rutivi rwemuviri, kusanzwa, uye kurasikirwa nepfungwa.

Sei chirwere chesickle cell chichikonzera marwadzo?

Zvichitaurwa zviri nyore, masero matsvuku eropa akaita sejeko anotaridzika sebhii C, kana kuti mwedzi mudiki. Paanofamba nepakati petsinga dzeropa, anonamira uye anovhara kufamba kweropa. Ndipo panotanga kurwadziwa. Funga nezvazvo semota dziri mumugwagwa.

Ko sickle cell chirwere che autoimmune here?

Kwete. Kunyange hazvo chirwere chesickle cell chine zvimwe zviratidzo zvezvirwere zve autoimmune , vanachiremba havaoni SCD sechirwere che autoimmune. Vanoona SCD sechirwere chemajini .

Chirwere cheSickle cell chirwere chinogara kwenguva refu. Kuisirwa stem cell, izvo zvinogona kupa mushonga wakakwana, hazvigoneke nguva dzose, uye zvine njodzi. Zvisinei, kuongororwa nekukurumidza uye kurapwa kunogona kubatsira kuderedza zviratidzo uye kuderedza njodzi yematambudziko. Nekurapwa nguva dzose, unogona kurarama hupenyu hwakazara, hunoshanda.

Chekupedzisira, zvimwe zvinhu zvakakosha zvaunofanira kurangarira (Mharidzo Yekuenda Kumba)

Zvakanaka, saka tataura zvakawanda nezvechirwere chesickle cell. Heano mamwe mashoko akakosha ekurangarira:

• Chirwere cheSickle cell (SCD) chirwere cheropa chinogara nhaka umo chimiro chemasero matsvuku eropa chinochinja, zvichikonzera matambudziko ekufamba kweropa.
• Chikonzero chikuru cheizvi imhando isina kujairika yehemoglobin inonzi hemoglobin S.
• Marwadzo, kupererwa neropa, utachiona, uye kukuvara kwenhengo dzemuviri zvakajairika.
• Kuonekwa nekukurumidza uye kurapwa kwakakodzera kwakakosha zvikuru. Vana vacheche vanoongororwa izvi.
• Kunyange hazvo kuisirwa mapfupa emubofu kuchigona kuva mushonga, hazvina kukodzera munhu wese. Kune mamwe marapirwo, akadai semishonga uye kuwedzerwa ropa.
• Unogona kurarama zvakanaka nechirwere ichi nekuchinja mararamiro ako, kutevedzera mazano akakodzera ekurapwa, uye kuita chimwe chinhu nekukurumidza kana paine dambudziko .

Kana iwe kana mumwe munhu waunoziva ane mibvunzo nezvechirwere chesickle cell, iva nechokwadi chekuona chiremba kuti akupe mazano. Hapana chekutya kana kunyara nacho. Chinhu chakakosha ndechekuziviswa! Chirwere chesickle cell, hemoglobin, masero matsvuku eropa, anemia, zvirwere zvemajini, matambudziko ekurwadziwa, kurapwa kwesickle cell