U-Ilya Kokarev wazalwa ngoJanuwari 4, 2021 futhi waba ngunyaka omusha wokuqala wesimangaliso se-vyacheslav ne-catherine. Ubaba wazakhela amacebo endodana elindelwe isikhathi eside ngaphambi kokuzalwa kwakhe kanye ne-griz, njengoba umfana edlala ngendabuko yakhe ngokuthi "Krasnodar" yakhe. U-Ilya, icebo elinjalo libuye lawa eshawini: Ingane kusukela ezinsukwini zokuqala ikhombisile le mpilo "isithupha", ukumamatheka okumamatheka kakhulu ekunqobeni nasemilenzeni yakhe emadolweni ...
Siza u-Ilya athole umjovo wempiloUSma watholakala enabazali baka-Ilya abaphansi kwezinyawo zakhe. Odokotela abavela ngaphandle kwamandla basakaza izandla zabo: izibikezelo azilungile kakhulu, umuthi ubiza kakhulu emhlabeni. Ngakho-ke i-ekaterina noVyacheslav Kokareva bafunda ukuthi kungakanani impilo yomuntu kungabiza malini: "Unembeza we-Cross-Nanembeza" uzobiza ngaphezu kwe Izigidi ezingama- $ 2.3 . Futhi abanazinsuku ezingaphezu kuka-600 ukucinga lesi sikhala.
"Kulesi sibalo, konke kufihliwe - ukwesaba, ukuphelelwa yithemba, ukungaqondi, kanjani ukuthola le nqwaba ye-ilseshka yethu kuphela! Kepha futhi kule mali wabeka into ebaluleke kakhulu - impilo! " (Catherine Koireva).
I-SMA: Izinhlobo nezimpawu
I-SMA (Decoding - Umgogodla we-Muscular Atrophy) yisifo sofuzo esingavamile. Ngenxa yokwephulwa kwemisebenzi yamangqamuzana ezinzwa zomgogodla, i-atrophy yemisipha kwenzeka, ukungasebenzi komuntu. Okokuqala, izicubu nemisipha yomlenze zingama-atrophy, khona-ke izicubu ezibhekele ukugwinya nokuphefumula. Kulokhu, isiguli sigcina ubuhlakani, futhi ubona umoya wokugcina ngaphambi kokuba umzimba ufe.
Siza u-Ilya athole umjovo wempiloIzinhlobo ze-spinalinal muscular atrophy:
- Smy I Type - Ifomu elinzima kunazo zonke, kwakungukuthi i-ilya yatholakala nje. Uhlobo lokuqala lunesihloko sezokwelapha - isifo seVerdnig-Hoffman, sizibonakalisa ezinganeni ezingaphansi kwezinyanga eziyisithupha. Ukuxilongwa komama enganeni efomini elinjalo kusho izimpawu ezibizwa kakhulu nesivinini sokuthuthuka. Izingane azikwazi ukugcina amakhanda zazo zihlale, zinobunzima bokuphefumula nokugwinya. Ngaphandle kokwelashwa, babulawa ekwehlulekeni okuphefumula ngaphansi kweminyaka emibili;
- Uhlobo lwe-SMI II , noma isifo sikaDubovits, sizibonakalisa ezinganeni kusuka ezinyangeni eziyi-7 kuye kwayi-1.5. Izimpawu zifana nohlobo lokuqala, noma kunjalo, ekusekelweni kokuphefumula kanye nosizo olufike ngesikhathi, izingane zingadla zihlale, kepha zingakwazi ukuhamba zodwa. Impilo yesikhathi sokuphila kwempilo yohlobo lwesibili ivame ukuncika ekuvikeleni izicubu zokuphefumula zishaye ngayo. Ngokulashwa okufanele, iziguli zingaphila iminyaka engaphezu kwengu-20;
- Uhlobo lwe-SMI III (noma i-atrophy ye-spices yomgogodla yentsha) - uhlobo oluncane lwezifo ezinganeni, luzibonakalisa ezigulini ezindala kunonyaka nesigamu. Izingane ezinokuxilongwa okunjalo zithola ubuthakathaka obuqinile, ukubikezela okungathandeki - ukukhubazeka nokulahlekelwa amandla okuma;
- Uhlobo lwe-smi iv - Ukwakheka kwabantu abadala, okuzibonakalisa ngemuva kweminyaka engama-35. Lolu hlobo lubonakala ngokukhubazeka kwesiguli okungaholeli ekunciphiseni okukhulu kokulindelwe empilweni.
Isibonelo, ummeleli walowo omele iRussia e-Eurovision-2018 uYulia Samolova, uHlalishi waseRussia uValery Spiridonov, osopolitiki basePoland kanye nesishoshovu seMarek Plow kanye ne-British TV eyethuli Kim Churkezia, bukhoma.
"UGen Gen": Izimbangela Eziyingozi
I-spinalinal muscular atrophy inyuka ngenxa ye- "creakage" ye-SMN1 gene. Inesibopho sokufaka amakhodi amaprotheni adingekayo ngomsebenzi obalulekile wezimoto ze-Neurons. Amagama alula, lezi ze-neurons "zixhumana" nomunye futhi ziphoqa izicubu ukuba zinciphise zombili ngesikhathi sezinqubo ezazi futhi phakathi nokungazi (ngokwesibonelo, ukuphefumula). Ukungatholi izithiyo, izicubu ziyafa.Ukuguqulwa kwenguquko ku-SMN1 gene kungenxa yephutha ngesikhathi sokwakhiwa kwe-DNA, lapho omunye wabazali bethola ukuba abathwali abaphilile. Lesi sifo yize siyivelakancane, kepha sisondele kakhulu kunokuba kubonakala ngathi: Yonke indawo engama-40 yomhlaba yomthwali onempilo yenethiwekhi "yofuzo ephukile". Amathuba okuzalwa kwesithwali sezingane ezithwaleni ezimbili kungu-50%, "emoticon" - 25%.
Siza u-Ilya athole umjovo wempiloAzikho izibalo zesifo ezinembile eRussia. Ngokusho kwe- "SM Family" (Inhlangano EyiyisiNgazo Eyisisekelo Esisiza Izingane Ngokuxilongwa Okunjalo), kuneziguli eziyizinkulungwane ezingama-5-7 ezine-gene gene gene ye-SMN1. Njalo ngonyaka, inani lezithonjana "zezithonjana" likhula cishe abantu abangama-300.
Ukwelashwa kokuhlaselwa komgogodla kwemisipha eRussia
Kuze kube ngu-2016, izingane ezine-sma zazenzelwe ukufa kobuhlungu. Iziguli zaqokwa kuphela ukunakekelwa kwe-palliative: "Izithonjana" zohlobo lokuqala kuma-85% amacala azizange ziphile iminyaka emi-2. Ngo-2021, sesivele sinezidakamizwa eziningana zokwelashwa kwalesi sifo, okuyinhloko yezingane - "Spinraz" kanye ne- "Zolgennma".
Umuthi wokuqala emhlabeni wokwelashwa kwe-spinalinal muscular atrophy wabonakala ngenxa yenkampani yaseMelika Biogen - "Spinraz". Lesi sidakamizwa sikhombise ukusebenza kahle, enye yezingqinamba zayo yintengo. "Spinraz" kubiza cishe Ama-ruble ayizigidi eziyi-8 Ukuze uthole i-ampoule, ngonyaka wokuqala wokwelashwa, kudingeka imijovo eyisithupha, kwalapha - amathathu ngonyaka impilo.
Siza u-Ilya athole umjovo wempiloKulula ukubala lokho eminyakeni engu-5 yokuqala, kuzodingeka ama-ruble ayi-168 ayizigidi eziyizigidi eziyishumi nambili., Ngemuva kweminyaka eyi-10, kuzohlolwa emabhulobane angaphezu kwezigidi ezingama-300. njll. Lesi sidakamizwa sibhalisiwe eRussia, sifakwa ohlwini lwe-GRICT futhi kufanele luthengwe ngokuchithwa kwesabelomali sesifunda. Kepha ekusebenzeni, kuyadingeka ukufezekisa ukwelashwa, imibundu ehluphayo yemikhumbi, abashushisi, ababusi. Maye, ezinye izingane aziphili umjovo wazo wokuqala.
Ngo-2019, izingane ezine-MIS zazilinde ukuphumelela kwangempela: Kwabanjwa isifundo semitholampilo e-United States (eZolgenma), esikwaziyo "ukulungisa" uhlobo lweSMN1 lube yisingeniso esisodwa emzimbeni. Esekuqaleni umuthi usetshenzisiwe, izingane eziningi zinamathuba okuthola ukuthuthukiswa kwezimoto okugcwele: "Izithonjana" ziqala ukugcina amakhanda, zihlale, zime zihambe. "I-Zolgensma" ikhonjiswa kuphela ezinganeni ezingaphansi kweminyaka engu-2. Ukwelashwa ngomuthi kuzobiza cishe Ama-ruble ayizigidi eziyi-170. Kepha ayibhalisiwe eRussia. Lokhu kusho ukuthi abazali baka-Ilya abakwazi ngisho nokubalwa kosizo lwesimo esinamandla ekuthengeni kwalo.
Siza u-Ilya athole umjovo wempiloKukhona futhi amalungiselelo athi "RobDiplam", "Barnaplam", "Releditive" nabanye abanobufakazi obuhlukile.
Ibhokisi lokungenayo lentengo yezemithi lichazwa ngezizathu zezomnotho: Ukuthuthukiswa kanye nocwaningo oludingekayo iminyaka engaphezu kwengu-10 nezigidigidi zamaRandi ngemali yangasese, umuthi awunakubiza okuncane. Intengo yenzelwe ohulumeni abazohlinzeka izakhamizi zabo ngomuthi, kepha hhayi ngemindeni ephakathi.
"I-Flashlight ye-Ilsishka"
UCatherine noVyacheslav bethule umkhankaso "Flashlight we-Ilsiaka" kunethiwekhi ukuqoqa imali yokwelashwa kweNdodana "Zolgensma". Ukubamba iqhaza kuyo kalula: Kuyadingeka ukudela noma yiziphi imali ezishisayo (noma ama-ruble angama-50) bese udlulisela "iLuchik of Light" kusuka ku-Ilya kumngane wakhe. Kubalulekile hhayi kakhulu inani lokunikela njengenombolo yabahlanganyeli abasebenzayo. Ukusiza, ngendlela, awukwazi ukweneka ngokwezimali kuphela: Extender imininingwane yokuqoqa ekuxhumaneni nomphakathi.Kungani kubaluleke kakhulu ukubamba iqhaza kuleli qoqo? Inkambu yeKrasnodar, lapho kwazalwa u-Ilya, - eyesithathu inani labantu abanesifunda saseSma saseRussia (ephansi kuphela eMoscow nakuMo). Ngamanye amagama, omunye wabanzima kakhulu. Ngo-Mashi 2021, izingane ezingama-61 zibhekwa nalokhu kuxilongwa, okungu-25 ngohlobo lwe-SM kuphela ngithayipha. Kepha akubona bonke abathola ukwelashwa okudingekayo: ngemuva kobhubhane, isidakamizwa sokuphila konke "iSportraz" sathola amayunithi, kanye ne-zolgensma "engathembisa kakhulu futhi iyatholakala nakancane ukuthola imali yangasese.
Siza u-Ilya athole umjovo wempiloIsimo ezweni lase-Ilya sichaze inhloko yesisekelo sosizo sisiza imindeni ngo-Alexander Kurdyyshkin engxoxweni ne-BBC. Ngakho-ke, ngesizinda sobhubhane, abantu abangu-19 baqala ukwenqaba kubo ngaphansi komthetho wamakhomishini ezokwelashwa nokwelashwa. Abazali bama- "Smileys" kufanele bashaye imishanguzo ngokusebenzisa izinkantolo nangamaphakethe, ukuqeda amandla emphakathini eMoscow. Kepha kulokhu, akuwona wonke umuntu othola usizo.
"Ngaphakathi esifundeni [emibonisweni - approx. Ed.] Konke akunandaba, "kusho u-Alexander Kurdyshkin.Iziphathimandla zasekhaya ziyayiqonda le nkinga. Ngakho-ke, ngemuva kokuqulwa kwecala, umshushisi uSergei Tagelsky wathi ezinye izingane azizange zithenge imishanguzo ngenxa ye- "Motokate ukuze uthole isabelomali" kanye "nokuxhaswa ngezimali". Nasezinhlanganweni zomusa, kwesinye isikhathi kungenzeka ukuzwa isinqumo: "Ungathathi izingane ezinezingane ezinkulu." Izimali eziningana sezivele zalile u-Ilya ecela usizo ngokubheka inani elikhulu lezikhalazo nomugqa. Lezo zimali ezimbalwa ezithathwa "izithonjana" ngokumane ngokwenyama azikwazi ukukhokha ukwelashwa kwawo wonke amawadi. NgoMashi 19, u-Ilya wathola umjovo wokuqala wezidakamizwa "Spinraz", kepha ukwelashwa kukhokhwe kuphela ngonyaka wokuqala.
Ngokusobala, izingane ezinalokhu kuxilongwa ngeke zilinde lapho ubhubhane sesiphelile. Asikho isikhathi sabo futhi silinde kuze kube yilapho abantu abadala bazoyithola nge- "fiber" yabo nezinye "zibi".
Usizo oluncane alwenzeki. I-ruble ngayinye inyusa ithuba lika-Ilya Kocareva ukuze liphile impilo ende, libe yindoda efanelekile bese liya emkhakheni ngesimo se- "Krasnodar". Ihhovisi lokuhlela 24CMI likholelwa emithini yesimanje kanye nekhono le-Intanethi, ngakho-ke sithumela imali yombhali wale ndatshana u-Ilya futhi sidlulise "iLuchik of the Light" kuwe. Imininingwane yokusiza ama-ilyos - kuleli khasi.
Ngingasiza kanjani u-Ilya?
1. Yiba yivolontiya. Manje asikho isandla esanele sokusebenza ekuqoqweni kwemali. Ivolontiya lingaba noma ngubani onemizuzu engu-10-15 yesikhathi samahhala ngosuku. Uma unethuba, bese ufaka amavolontiya engxoxo.
2. Sabalalisa imininingwane. Uma une-akhawunti ku- "Instagram", "VKOntakte", Facebook noma Odnoklassniki, ungabelana ngomlando we-Iltishi nabangane bakho kanye nabantu obajwayele. Uma okungenani umuntu oyedwa kusuka ezikuzungezile kuzohumusha noma yiliphi inani kumjovo, khona-ke lokhu kuyasiza. Uma ungazi ukuthi ungasabalalisa kanjani imininingwane ekuxhumaneni nomphakathi, ngena kwizisebenzi zokuzithandela zokuxoxa, uzosizwa ukuba uqonde.
3. Ngezinto ezibonakalayo. Ngisho nama-ruble ayi-100 asondela ku-ilya emjovo oyigugu. Uma abantu abayizigidi eziyi-1,7 bahumusha ngama-ruble ayi-100 kuphela ngama-ruble ayi-100 ngama-ruble ayi-100, inani lizoqoqwa ngokushesha. Kulezi zinsuku, ama-ruble ayi-100 akayona inkomishi yekhofi, kepha i-ilya leli nani izosindisa impilo. Imininingwane yosizo ingatholakala kusixhumanisi.
Isiphetho Sodokotela
