Kodi Ndizithandizo Zotani Zomwe Zilipo pa Spinal Muscular Atrophy?

Zamkati

• Chisamaliro cha multidisciplinary
• Njira zochiritsira
• Spinraza
• Zolgensma
• Mankhwala oyesera
• Njira zothandizira
• Thanzi lopuma
• Thanzi komanso kugaya chakudya
• Thanzi komanso thanzi
• Thandizo pamtima
• Kutenga

Spinal muscular atrophy (SMA) ndichikhalidwe chosowa kwambiri chomwe chimapangitsa minofu kufooka komanso kuwonda. Mitundu yambiri ya SMA imapezeka mwa makanda kapena ana aang'ono.

SMA imatha kuyambitsa zovuta zolumikizana, kudyetsa zovuta, komanso mavuto omwe angakhale pachiwopsezo cha kupuma. Ana ndi akulu omwe ali ndi SMA atha kukhala ndi vuto kukhala, kuyimirira, kuyenda, kapena kumaliza ntchito zina popanda thandizo.

Pakadali pano palibe mankhwala odziwika a SMA. Komabe, njira zatsopano zothandizira zitha kuthandizira kukonza malingaliro a ana ndi akulu omwe ali ndi SMA. Chithandizo chothandizira chimapezekanso kuti chithandizire kuthana ndi zovuta komanso zovuta zomwe zingakhalepo.

Tengani kamphindi kuti mudziwe zambiri za njira zamankhwala zothandizira SMA.

Chisamaliro cha multidisciplinary

SMA imatha kukhudza thupi la mwana wanu m'njira zosiyanasiyana. Pofuna kusamalira zosowa zawo zosiyanasiyana, ndikofunikira kuti pakhale gulu lazachipatala.

Kupimidwa pafupipafupi kumathandiza gulu lazachipatala la mwana wanu kuti lizitha kuwunika momwe aliri komanso kuwunika momwe mapulani ake amathandizira.

Angakulimbikitseni kuti musinthe dongosolo la chithandizo cha mwana wanu ngati mwana wanu wayamba kukhala ndi zizindikilo zatsopano kapena zoyipa. Angathenso kulimbikitsa kusintha ngati mankhwala atsopano atha kupezeka.

Njira zochiritsira

Pofuna kuthana ndi zomwe zimayambitsa SMA, Food and Drug Administration (FDA) yalengeza posachedwapa njira ziwiri zochiritsira:

• nusinersen (Spinraza), yomwe imavomerezedwa kuchiza SMA mwa ana ndi akulu
• onasemnogene abeparvovec-xioi (Zolgensma), yomwe imavomerezedwa kuchiza SMA mwa ana ochepera zaka ziwiri

Mankhwalawa ndi achilendo, motero akatswiri sakudziwa kuti zotsatira zogwiritsa ntchito mankhwalawa zitha bwanji. Kafukufuku akuwonetsa kuti atha kuchepetsa kapena kuchepetsa kukula kwa SMA.

Spinraza

Spinraza ndi mtundu wa mankhwala omwe amapangidwira kuti apange kupanga puloteni yofunikira, yotchedwa sensor motor neuron (SMN) protein. Anthu omwe ali ndi SMA samadzipangira okha mapuloteni okwanira.

Chithandizo chovomerezedwa potengera maphunziro azachipatala omwe akuwonetsa kuti makanda ndi ana omwe amalandila chithandizo atha kusintha magwiridwe antchito, monga kukwawa, kukhala, kugudubuka, kuyimirira, kapena kuyenda.

Ngati dokotala wa mwana wanu akupatsani Spinraza, amubaya mankhwalawo mumadzimadzi ozungulira msana wa mwana wanu. Ayamba powapatsa mankhwala anayi pa miyezi iwiri yoyambirira yothandizidwa. Pambuyo pake, adzapereka mlingo umodzi pakatha miyezi inayi iliyonse.

Zotsatira zoyipa za mankhwalawa ndi monga:

• chiopsezo chowonjezeka cha matenda opuma
• chiopsezo chowonjezeka chotuluka magazi
• kuwonongeka kwa impso
• kudzimbidwa
• kusanza
• mutu
• kupweteka kwa msana
• malungo

Ngakhale zoyipa ndizotheka, kumbukirani kuti wothandizira zaumoyo wa mwana wanu angakulimbikitseni mankhwalawa ngati akukhulupirira kuti mapindulowo amaposa chiopsezo cha zotsatirapo zake.

Zolgensma

Zolgensma ndi mtundu wa chithandizo cha majini, momwe kachilombo kosinthidwa kamagwiritsira ntchito ntchito Zamgululi jini kumaselo amitsempha. Anthu omwe ali ndi SMA alibe jini yogwira ntchitoyi.

Anavomereza mankhwalawa potengera mayesero azachipatala omwe ali ndi ana okhaokha omwe ali ndi SMA ochepera zaka ziwiri. Ophunzira nawo mayeserowa adawonetsa kusintha kwakukulu pachitukuko, monga kuwongolera mutu komanso kutha kukhala opanda chithandizo, poyerekeza ndi zomwe zingayembekezeredwe kwa odwala omwe sanalandire chithandizo.

Zolgensma ndi mankhwala amodzi omwe amaperekedwa kudzera mu kulowetsedwa kwa intravenous (IV).

Zotsatira zoyipa ndizo:

• kusanza
• kuchuluka michere ya chiwindi
• kuwonongeka kwakukulu kwa chiwindi
• kuwonjezeka kwa zizindikiro za kuwonongeka kwa minofu ya mtima

Ngati dokotala wa mwana wanu akupatsani Zolgensma, adzafunika kuyitanitsa mayeso kuti awunikire thanzi la chiwindi cha mwana wanu asanalandire chithandizo, nthawi, komanso pambuyo pake. Akhozanso kupereka zidziwitso zambiri zakubwino ndi zoopsa za mankhwalawa.

Mankhwala oyesera

Asayansi akuphunzira mankhwala ena angapo omwe angapezeke ku SMA, kuphatikiza:

• alireza
• nthambi
• omakuma
• Zamgululi

A FDA sanalandirebe chithandizo choyeserachi. Komabe, ndizotheka kuti bungweli lingavomereze imodzi kapena zingapo zamankhwala mtsogolomo.

Ngati mukufuna kudziwa zambiri zamayeso oyesera, lankhulani ndi dokotala wa mwana wanu zamayeso azachipatala. Gulu lanu lazachipatala litha kukupatsirani zambiri za ngati mwana wanu atha kutenga nawo mbali pazoyeserera zamankhwala, komanso zabwino ndi zoopsa zake.

Njira zothandizira

Kuphatikiza pa chithandizo chamankhwala chothandizira SMA, adotolo a mwana wanu angakulimbikitseni chithandizo china kuti muthane ndi zizindikilo kapena zovuta zomwe zingakhalepo.

Thanzi lopuma

Ana omwe ali ndi SMA amakhala ndi minofu yopuma, yomwe imapangitsa kuti zikhale zovuta kupuma. Ambiri amapanganso nthiti, zomwe zimawonjezera mavuto kupuma.

Ngati mwana wanu akuvutika kupuma kwambiri kapena kutsokomola, zimawayika pachiwopsezo cha chibayo. Ichi ndi chiwopsezo chowopsa cha m'mapapo.

Pofuna kuthandizira njira zopumira za mwana wanu ndikuthandizira kupuma kwawo, gulu lawo laumoyo lingapereke malangizo awa:

• Buku chifuwa physiotherapy. Wothandizira zaumoyo amagogoda pachifuwa cha mwana wanu ndipo amagwiritsa ntchito njira zina kumasula ndi kuchotsa mamina panjira zawo.
• Kukoka kwa Oronasal. Chubu chapadera kapena syringe imayikidwa mu mphuno kapena mkamwa mwa mwana wanu ndipo amagwiritsira ntchito kuchotsa ntchofu m'mpweya wawo.
• Makina osakwanira / kutulutsa ndalama. Mwana wanu amalumikizidwa ndi makina apadera omwe amafanana ndi chifuwa kuti atulutse mamina panjira zawo.
• Mawotchi mpweya. Chigoba chopumira kapena chubu cha tracheostomy chimagwiritsidwa ntchito kulumikiza mwana wanu ndi makina apadera omwe amawathandiza kupuma.

Ndikofunikanso kutsatira ndondomeko yolandira katemera wa mwana wanu kuti muchepetse chiopsezo chotenga matenda, kuphatikizapo fuluwenza ndi chibayo.

Thanzi komanso kugaya chakudya

SMA imapangitsa kuti zikhale zovuta kuti ana ayamwe ndi kumeza, zomwe zimachepetsa kuthekera kwawo kudyetsa. Izi zitha kubweretsa kukula kosauka.

Ana ndi akulu omwe ali ndi SMA amathanso kukumana ndi zovuta zam'mimba, monga kudzimbidwa kosalekeza, gastroesophageal reflux, kapena kuchedwa kutaya kwa m'mimba.

Pofuna kuthandiza mwana wanu kukhala ndi thanzi labwino komanso limagaya chakudya, gulu lawo lazaumoyo lingalimbikitse:

• kusintha kwa zakudya zawo
• mavitamini kapena mchere wothandizira
• kudyetsa kwa enteric, momwe chubu lodyetsera limagwiritsidwa ntchito kuperekera madzi ndi chakudya m'mimba mwawo
• mankhwala ochizira kudzimbidwa, gastroesophageal Reflux, kapena mavuto ena am'mimba

Ana ndi ana ang'ono omwe ali ndi SMA ali pachiwopsezo chochepa thupi. Kumbali inayi, ana okalamba ndi achikulire omwe ali ndi SMA ali pachiwopsezo chonenepa kwambiri kapena kukhala onenepa kwambiri chifukwa chazinthu zochepa zolimbitsa thupi.

Ngati mwana wanu ali wonenepa kwambiri, gulu lawo lothandizira zaumoyo lingalimbikitse kusintha kwa zakudya zawo kapena zochita zawo zolimbitsa thupi.

Thanzi komanso thanzi

Ana ndi akulu omwe ali ndi SMA ali ndi minofu yofooka. Izi zitha kuchepetsa kuyenda kwawo ndikuwayika pachiwopsezo chazovuta zamagulu, monga:

• mtundu wopunduka palimodzi wotchedwa contractures
• kupindika kwachilendo kwa msana, kotchedwa scoliosis
• kusokonekera kwa nthiti
• kusunthika m'chiuno
• kuphwanya mafupa

Pofuna kuthandizira ndikutambasula minofu ndi mafupa awo, gulu lazachipatala la mwana wanu limatha kupereka:

• masewera olimbitsa thupi
• ziboda, zolimba, kapena ziwalo zina
• zipangizo zina zothandizira kumbuyo

Ngati mwana wanu ali ndi zovuta zolumikizana kapena zopindika, angafunike kuchitidwa opaleshoni.

Mwana wanu akamakula, angafunike njinga ya olumala kapena chida china chowathandiza kuti ziziyenda.

Thandizo pamtima

Kukhala ndi thanzi labwino kungakhale kovuta kwa ana, komanso makolo awo ndi ena omwe amawasamalira.

Ngati inu kapena mwana wanu muli ndi nkhawa, kukhumudwa, kapena zovuta zina zamaganizidwe, auzeni dokotala wanu.

Atha kukutumizirani kwa katswiri wazamisala kuti mukalandire upangiri kapena chithandizo china. Akhozanso kukulimbikitsani kuti mulumikizane ndi gulu lothandizira anthu omwe amakhala ndi SMA.

Kutenga

Ngakhale pakadali pano palibe mankhwala a SMA, pali mankhwala omwe angathandize kuchepetsa kukula kwa matendawa, kuchepetsa zizindikilo, ndikuwongolera zovuta zomwe zingakhalepo.

Ndondomeko ya chithandizo cha mwana wanu idzadalira zizindikiro zawo komanso zosowa zawo. Kuti mudziwe zambiri zamankhwala omwe alipo, lankhulani ndi gulu lawo lazachipatala.

Chithandizo cham'mbuyomu ndikofunikira polimbikitsa zotsatira zabwino kwambiri kwa anthu omwe ali ndi SMA.