Ese ni iki? Amyotrophic Lateral Sclerosis (ALS)? Ibimenyetso, Impamvu, n'Ubuvuzi

Amyotrophic lateral sclerosis, izwi cyane nka ALS cyangwa indwara ya Lou Gehrig, ni indwara y'ubwonko itera imbere ihungabanya uturemangingo tw'ubwonko dugenga imitsi ikora ku bushake. Uturemangingo twawe twa moteri, dukoresha uburyo nk'ubw'amashanyarazi butuma amakuru ava mu bwonko agera ku mitsi, buhoro buhoro birakonja bikareka gukora neza. Ibi bituma imitsi igucika intege, bigatangirira ahantu hamwe bigakwirakwira mu mubiri wose uko iminsi igenda ishira.

Ese ni iki ALS?

ALS igaragara cyane ku turemangingo twa moteri mu bwonko no mu mugongo. Aya turemangingo twihariye ni nka ba batumwa, batuma amakuru ava mu bwonko agera ku mitsi uyobora ku bushake, nka za mitsi zo mu maboko, amaguru n'isura.

Iyo utwo turemangingo twa moteri dukonje, isano iri hagati y'ubwonko n'imitsi irakonja. Imitsi yawe buhoro buhoro itakibasha kubona amakuru ahagije, bigatuma igucika intege, ukagira ibibazo byo guhindagurika, kandi amaherezo imitsi ikarangirika rwose mu bice byangiritse.

Ijambo "amyotrophic" risobanura "idafite amavuta yo kugaburira imitsi," naho "lateral sclerosis" risobanura gukomera kw'uturemangingo tw'ubwonko mu bice bimwe na bimwe by'umugongo. Nubwo izina ryayo rigoye, ALS isobanura ko umubiri wawe utakibasha kuyobora imitsi ikora ku bushake uko iminsi igenda ishira.

Ni ibihe bimenyetso bya ALS?

Ibimenyetso bya ALS bisanzwe bigaragara buhoro buhoro kandi bishobora gutandukana cyane ukurikije umuntu ku wundi. Ibimenyetso bya mbere bikunze gutangirira ahantu hamwe mbere yo gukwirakwira mu bindi bice by'umubiri.

Ibimenyetso bya mbere bikunze kugaragara ni ibi bikurikira:

• Igucika intege ry'imitsi mu maboko, mu biganza, cyangwa mu maguru bigatuma imirimo ya buri munsi ikugora
• Guhindagurika kw'imitsi cyangwa kubabara, cyane cyane mu maboko, mu bitugu, cyangwa mu rurimi
• Kugorana gukora imirimo isaba ubuhanga buto nko gufungura imyenda cyangwa kwandika
• Kugwa cyangwa kugwa kenshi ugereranyije n'ibisanzwe
• Kuvuga bidasobanutse cyangwa guhinduka kw'ijwi ryawe
• Kugorana kw'umunwa cyangwa kuruma ibiryo

Uko iyi ndwara itera imbere, ushobora kubona ko imitsi igucika intege cyane bigatuma utabasha kugenda, gukoresha amaboko, cyangwa kuvuga neza. Ibimenyetso muri rusange birakomeza gukomera mu mezi cyangwa imyaka, nubwo umuvuduko utandukana cyane hagati y'abantu.

Ni ingenzi kumenya ko ALS isanzwe idahungabanya ubwenge bwawe, kwibuka, cyangwa ibyiyumvo. Ubwenge bwawe busanzwe buguma bukomeye nubwo ibimenyetso by'umubiri bigaragara, ibyo bishobora gutera ihumure n'ibibazo kuri benshi.

Ni iyihe mityo ya ALS?

ALS muri rusange igabanywamo ubwoko bubiri bushingiye ku buryo itera imbere n'icyo iterwa na cyo. Gusobanukirwa ibi bice bishobora kugufasha gusobanukirwa neza uko ibintu byawe bihagaze.

Sporadic ALS igize hafi 90-95% by'abantu bose barwaye. Ubu bwoko butangira gitunguranye nta mateka y'umuryango cyangwa impamvu zisobanuka. Abantu benshi bapimwe bafite ubu bwoko bwa sporadic, kandi abashakashatsi bagikora ubushakashatsi kugira ngo bamenye icyo bubitera.

Familial ALS igira ingaruka kuri hafi 5-10% by'abantu barwaye kandi ikomoka mu muryango. Ubu bwoko buterwa n'impinduka z'impeshyi zirakomoka ku babyeyi bajya ku bana. Niba ufite familial ALS, hariho uburyo iyi ndwara igaragara mu bantu benshi bo mu muryango mu bihe bitandukanye.

Muri ibyo bice by'ingenzi, abaganga rimwe na rimwe basobanura ALS bushingiye aho ibimenyetso bitangirira. Limb-onset ALS itangira n'igucicika intege ry'imitsi mu maboko cyangwa mu maguru, naho bulbar-onset ALS itangira n'ibibazo byo kuvuga cyangwa kurya.

Ni iki giterwa na ALS?

Impamvu nyamukuru ya ALS ntirasobanutse neza, ibyo bishobora gutera agahinda iyo ushaka ibisobanuro. Abashakashatsi bizeye ko bishobora guterwa n'ivanguranyamirongo ry'impamvu z'impeshyi, iz'ibidukikije, n'imibereho.

Ku bijyanye na familial ALS, impinduka z'impeshyi ni zo mpamvu nyamukuru. Imigeni ikunze kugaragara harimo C9orf72, SOD1, TARDBP, na FUS. Izi mpinduka z'impeshyi zigira ingaruka ku mikorere n'ubuzima bw'uturemangingo twa moteri, bigatuma buhoro buhoro birakonja.

Muri sporadic ALS, impamvu ntizisobanuka neza. Abahanga mu bya siyansi baracyiga ibintu byinshi bishobora gutera iyi ndwara:

• Uburozi bw'ibidukikije cyangwa ibintu bishobora kwangiza uturemangingo twa moteri
• Indwara ziterwa na virusi zishobora gutera ubudahangarwa bw'umubiri
• Ibibazo byo gutunganya poroteyine mu turemangingo tw'ubwonko
• Oxidative stress yangiza ibice by'uturemangingo
• Kubabara mu mikorere y'ubwonko

Bimwe mu bimenyetso bito bishobora kuba bifitanye isano n'ibintu bimwe na bimwe nk'uko gukora igisirikare, imyitozo ikomeye, cyangwa kwibasirwa na bimwe mu bintu. Ariko rero, abantu benshi bafite ibyo bintu ntibarwara ALS, bigaragaza ko ibintu byinshi bigomba guhurirana kugira ngo iyi ndwara igaragare.

Ni ryari ukwiye kubona muganga kubimenyetso bya ALS?

Ugomba kujya kwa muganga niba ubona ugucika intege kw'imitsi, guhindagurika, cyangwa guhinduka mu kuvuga cyangwa kurya bikomeza ibyumweru birenga bike. Gupimwa hakiri kare ni ingenzi kuko indwara nyinshi zishobora kugaragara nk'ibimenyetso bya ALS, kandi gupimwa hakiri kare bifasha guha ubuvuzi bukwiye.

Shaka ubuvuzi vuba niba ugira ibibazo mu mirimo ya buri munsi nko kwandika, kugenda, cyangwa kuvuga neza. Nubwo ibyo bimenyetso bishobora kuba bifite impamvu nyinshi, gupimwa neza bishobora gufasha kumenya icyaba gikubayeho no gukuraho izindi ndwara zishobora kuvurwa.

Ntugatege amatwi niba ugira ibibazo byo kurya cyangwa guhumeka, kuko ibyo bimenyetso bishobora gusaba ubuvuzi bwihuse. Muganga wawe ashobora gusuzuma niba ibyo bihinduka bikeneye ubuvuzi bwihuse cyangwa bishobora gufashwa no gukurikirana ubuvuzi busanzwe.

Ni ibihe bintu byongera ibyago bya ALS?

Ibintu bimwe na bimwe bishobora kongera ibyago byo kurwara ALS, nubwo ufite ibyago ntibisobanura ko uzayirwara. Gusobanukirwa ibyo bintu bishobora kugufasha gusobanukirwa uko ibintu byawe bihagaze.

Imyaka ni yo ntandaro ikomeye y'ibyago, abantu benshi barwara ALS hagati y'imyaka 40 na 70. Igihe cyo gutangira cyane cyane kiba kiri hafi y'imyaka 55, nubwo iyi ndwara rimwe na rimwe ishobora kwibasira abantu bakiri bato cyangwa abakuze.

Igitsina kigira uruhare, abagabo bafite ibyago byinshi byo kurwara ALS kurusha abagore, cyane cyane mbere y'imyaka 65. Nyuma y'imyaka 70, uwo gutandukana birakomeza kugabanuka, kandi ibyago bihinduka bingana hagati y'abagabo n'abagore.

Ibindi bintu bishobora kongera ibyago birimo:

• Amateka y'umuryango wa ALS cyangwa izindi ndwara z'ubwonko
• Gukora igisirikare, cyane cyane mu bihugu byo hanze
• Imikino ikomeye cyangwa imikino yo ku rwego rwo hejuru
• Kwibasirwa na bimwe mu bintu, amabuye y'agaciro, cyangwa imiti yica udukoko
• Kunywesha itabi, bishobora kongera ibyago gato
• Gukomeretsa umutwe cyangwa gukomeretsa ubwonko kenshi

Abantu benshi bafite ibyo bintu ntibarwara ALS, kandi abantu benshi barwaye ALS nta bintu byongera ibyago bafite. Kuba ufite ibyago bisobanura ko hari amahirwe make, atari ukuri kw'ibintu.

Ni ibihe bintu bishobora guterwa na ALS?

Ibibazo bya ALS bitera imbere uko ugucika intege kw'imitsi bikomeza, bigira ingaruka ku bice bitandukanye by'umubiri uko iminsi igenda ishira. Gusobanukirwa ibibazo bishoboka bigufasha wowe n'itsinda ryawe ry'abaganga gutegura ubuvuzi burambuye.

Ibibazo byo guhumeka ni kimwe mu bibazo bikomeye. Uko imitsi yo guhumeka igucika intege, ushobora kugira ikibazo cyo guhumeka, cyane cyane iyo uri kuryama, indwara z'ubuhumekero, cyangwa umunaniro uterwa n'imbaraga nyinshi ukeneye guhumeka.

Ibibazo byo kurya, bizwi nka dysphagia, bishobora gutera ibibazo byinshi:

• Gukama ibiryo mu gihe cyo kurya cyangwa kunywa
• Pneumonia iterwa no kwinjira kw'ibiryo cyangwa amazi mu bihaha
• Imvura n'igucicika intege kubera kugorana kurya
• Kuma kubera kugabanuka kw'amazi

Ibibazo byo gutumanaho bishobora kuza uko imitsi igenga kuvuga igucika intege. Ibi bishobora gutera ukwigunga n'agahinda, nubwo ubuhanga butandukanye bwo gufasha bushobora gufasha kugumana ubushobozi bwawe bwo gutumanaho neza.

Ibibazo byo kugenda birimo kongera ibyago byo kugwa, ibikomere byo mu mitsi kubera kugenda gake, no gukomera kw'ingingo cyangwa gukomera kw'imitsi. Byongeye kandi, bamwe mu bantu bagira ibibazo by'amarangamutima, harimo agahinda cyangwa impagarara, ibyo ni ibintu bisanzwe bibaho kubera ibibazo byo kubana na ALS.

Nubwo ibyo bibazo byumvikana nkibyinshi, byinshi muri byo bishobora gufashwa neza n'ubuvuzi bukwiye, ibikoresho byo gufasha, n'ubufasha bw'abaganga bamenyereye kuvura ALS.

ALS ipima ite?

Gupima ALS bishingira ahanini ku kureba ibimenyetso no gukuraho izindi ndwara, kuko nta buryo bumwe bwo gupima buhari. Uyu murimo urasaba amezi menshi kandi ugomba gukorwa mu buryo butandukanye.

Muganga wawe azatangira akumaze kumenya amateka yawe n'isuzuma ry'umubiri, areba ibimenyetso by'igucicika intege ry'imitsi n'ibindi bimenyetso by'ubwonko. Azapima uburyo imitsi yawe ikora, imbaraga z'imitsi, n'uburyo ugendera kugira ngo amenye ibibazo.

Ibizamini byinshi byo gupima bifasha gushyigikira ibizamini:

• Electromyography (EMG) ipimira ingufu z'amashanyarazi mu mitsi kandi ishobora kugaragaza kwangirika kw'imitsi
• Isuzuma ry'uburyo amashanyarazi acengera mu mitsi
• Isuzuma rya MRI ry'ubwonko n'umugongo bikuraho izindi ndwara z'ubwonko
• Ibizamini by'amaraso bisuzumira indwara zandura, indwara ziterwa n'ubudahangarwa bw'umubiri, cyangwa ibibazo byo mu mubiri
• Isuzuma ry'impeshyi niba hari amateka y'umuryango wa ALS

Gupima bikunze gusaba igihe n'uburabyo, kuko abaganga bagomba kureba neza uko ibimenyetso bikomeza no gukuraho indwara nk'indwara nyinshi z'imitsi, udukoko mu mugongo, cyangwa indwara z'imitsi ziterwa n'ibibazo by'imitsi bigira ibimenyetso bisa.

Itsinda ryawe ry'abaganga rishobora kuba ririmo abaganga b'inzobere mu kuvura ALS, kuko ubuhanga bwabo bufasha guha ubuvuzi bukwiye kuva mu ntangiriro.

Ni iki kivura ALS?

Nubwo nta muti uravura ALS ubu, hari ubuvuzi bushobora gufasha kugabanya umuvuduko w'iyi ndwara, kuvura ibimenyetso, no kubungabunga ubuzima bwawe. Uburyo bwo kuvura bushingiye ku buvuzi burambuye bufasha mu bice byinshi by'iyi ndwara.

Imiti ibiri yemewe na FDA ishobora gufasha kugabanya umuvuduko wa ALS. Riluzole ishobora kongera igihe cyo kubaho mu mezi make gusa igabanya urwego rwa glutamate mu bwonko. Edaravone, itangwa mu buryo bwa IV, ishobora kugabanya igabanuka ry'imikorere ya buri munsi kuri bamwe mu barwaye ALS.

Kuvura ibimenyetso bikorwa mu buryo bwa buhanga:

• Ubuvuzi bw'umubiri kugira ngo ubungabunge imbaraga n'ubushobozi bwo kugenda
• Ubuvuzi bw'imirimo kugira ngo ufashe mu mirimo ya buri munsi n'ibikoresho byo gufasha
• Ubuvuzi bw'amagambo kugira ngo ufashe mu kuvuga no kurya
• Ubuvuzi bw'ubuhumekero kugira ngo ufashe mu guhumeka
• Inama ku biribwa kugira ngo ubungabunge imirire ikwiye

Uko iyi ndwara itera imbere, ibikoresho byo gufasha birakomeza kuba ingenzi. Ibyo bishobora kuba ibikoresho byo kugenda nka za walkers cyangwa amagare y'abamugaye, ibikoresho byo gutumanaho kubafite ibibazo byo kuvuga, kandi amaherezo ibikoresho byo gufasha mu guhumeka.

Uburyo bwo kuvura buhinduka ukurikije umuntu ku wundi, itsinda ryawe ry'abaganga rihinduranya ibitekerezo hashingiwe ku bimenyetso byawe, umuvuduko w'iyi ndwara, n'ibyo ukunda mu buvuzi.

Uburyo bwo kuvura ALS murugo?

Kuvura ALS murugo bishingiye ku kubungabunga ubuzima, umutekano, n'ubwigenge igihe kirekire bishoboka. Ihinduka n'ingamba byoroshye bishobora koroshya cyane ubuzima bwa buri munsi.

Kurema ahantu heza murugo ni ingenzi uko ubushobozi bwo kugenda buhinduka. Kuraho ibintu bishobora gutera kugwa nka za tapi zidashikamye, shyira ibikoresho byo gufata mu bwiherero, komeza umucyo mwiza mu rugo rwawe, kandi utekereze ku byubatswe niba intambwe zigoye.

Ubufasha mu biribwa burakomeza kuba ingenzi uko ibibazo byo kurya bikomeza:

• Hitamo ibiryo byoroshye kuruma no kurya
• Kurya ibiryo bike, ariko kenshi kugira ngo ugabanye umunaniro
• Gama imyanya ihagaze igihe cyo kurya no mu minota 30 nyuma yaho
• Koranira n'umuhanga mu mirire kugira ngo ubone imirire ikwiye
• Tegereza ibikoresho by'imirire niba byasabwe

Kubungabunga ingufu bigufasha kugumana ibikorwa igihe kirekire. Plan imirimo yawe ikomeye igihe wumva ufite imbaraga, ufate ibiruhuko, kandi ntutinye gusaba ubufasha mu mirimo ikomeye.

Gupanga gutumanaho ni ingenzi uko kuvuga bihinduka. Menya ibikoresho byo gufasha gutumanaho hakiri kare, menya kubikoresha igihe uvuga neza, kandi ushyireho uburyo bwo gutumanaho n'abantu bo mu muryango.

Wategura ute uruzinduko rwawe kwa muganga?

Gutegura neza ibijyanye no kuvura ALS bifasha guha umusaruro igihe cyawe n'abaganga. Gutegura neza bigatuma habaho ibiganiro byiza kandi kuvura neza.

Komeza inyandiko y'ibimenyetso byawe hagati y'ibindi bisuzumwa, ugaragaze impinduka mu mitsi, ibimenyetso bishya, cyangwa ibibazo mu mirimo ya buri munsi. Fata ingero z'imirimo ibaye ikugora n'igihe wabimenye bwa mbere.

Tegura ibibazo byawe mbere kandi ushyire imbere iby'ingenzi:

• Ni iyihe mpinduka nategereza?
• Hariho ubuvuzi bushya cyangwa ubushakashatsi nabona?
• Ni ibihe bikoresho byo gufasha bishobora kumbafasha ubu?
• Nshobora kubungabunga ubwigenge bwanjye igihe kirekire bite?
• Ni ayahe mahirwe aboneka ku muryango wanjye?

Zana urutonde rwuzuye rw'imiti, ibikoresho byo gufasha, n'ibikoresho byo gufasha ukoresha ubu. Fata amakuru yerekeye ibyakubereye byiza n'ibidakora neza.

Tegereza kuzana umuntu wo mu muryango cyangwa inshuti mu bisuzumwa. Bashobora gufasha kwibuka amakuru y'ingenzi, kubaza ibibazo ushobora kwibagirwa, no gutanga ibitekerezo by'impinduka babonye.

Ni iki cy'ingenzi cyerekeye ALS?

ALS ni indwara ikomeye y'ubwonko itera imbere, ariko kuyisobanukirwa biguha ubushobozi bwo gukorana neza n'itsinda ryawe ry'abaganga no kubungabunga ubuzima bwawe. Nubwo gupimwa bishobora gutera agahinda, abantu benshi barwaye ALS bakomeza kubona icyishimo, ibyishimo, n'isano mu buzima bwabo.

Ipfundo ryo kubaho neza na ALS riri mu buvuzi burambuye, gutegura hakiri kare, n'ubufasha bukomeye. Ubuhanga bugezweho bwo gufasha, uburyo bwo kuvura ibimenyetso, n'amakipe y'abaganga b'inzobere bashobora gufasha guhangana n'ibibazo byinshi bigaragara.

Wibuke ko ALS igira ingaruka kuri buri wese mu buryo butandukanye, hamwe n'umuvuduko utandukanye w'iyi ndwara n'ibimenyetso. Uburambe bwawe buzaba bwihariye, kandi ubuvuzi bugomba guhuzwa n'ibyo ukeneye n'ibyo ukunda.

Ubushakashatsi kuri ALS burakomeje, ubuvuzi bushya n'uburyo bushya biracyiga. Kugumana isano n'itsinda ryawe ry'abaganga n'imiryango ya ALS bishobora kugufasha kumenya amahitamo n'ibikoresho bishya.

Ibibazo bikunze kubaho kuri ALS

Ese ALS ihora yica?

Ubu ALS ifatwa nk'indwara yica, ariko igihe cyo kubaho gitandukana cyane hagati y'abantu. Bamwe mu bantu babaho imyaka myinshi nyuma yo gupimwa, abandi bagira umuvuduko wihuse. Hafi 20% by'abantu barwaye ALS babaho imyaka itanu cyangwa irenga nyuma yo gupimwa, kandi umubare muto babaho igihe kirekire. Ubuvuzi bushya n'ubuvuzi bwo gufasha birakomeza kunoza ibyavuye mu buvuzi n'ubuzima.

Ese ALS ishobora kwirindwa?

Ubu, nta buryo bwo kwirinda ALS buzwi, cyane cyane ubu bwoko bwa sporadic bugira ingaruka ku bantu benshi. Kuri familial ALS, inama z'impeshyi zishobora gufasha imiryango gusobanukirwa ibyago byabo, ariko kwirinda ntibishoboka. Ariko rero, kubungabunga ubuzima bwiza binyuze mu mikino, kwirinda kunywesha itabi, no kugabanya kwibasirwa n'ibintu byangiza ibidukikije bishobora gufasha ubuzima bw'ubwonko muri rusange.

Ese ALS ihungabanya ubwenge n' kwibuka?

Abantu benshi barwaye ALS bagumana ubwenge n'ubumenyi busanzwe mu gihe cy'indwara yabo. Ariko rero, hafi 15% by'abantu barwaye ALS barwara frontotemporal dementia, ishobora kugira ingaruka ku bwenge, imyitwarire, n'imico. Nubwo impinduka zo mu bwenge zigaragara, zisanzwe zigaragara mu buryo butandukanye n'ibimenyetso by'imitsi kandi zishobora kuba zoroheje mu ntangiriro.

Ese abantu barwaye ALS bashobora gukomeza gukora?

Abantu benshi barwaye ALS bakomeza gukora igihe runaka nyuma yo gupimwa, cyane cyane mu mirimo idasaba imbaraga nyinshi. Ibikoresho nka gahunda zihinduka, ubuhanga bwo gufasha, cyangwa imirimo ihinduwe bishobora gufasha kongera ubushobozi bwo gukora. Icyemezo gishingira ku mimerere y'umuntu, umuvuduko w'iyi ndwara, n'ibyo umuntu akunda mu bijyanye no gukomeza akazi.

Ni ayahe mahirwe aboneka ku miryango ifite abantu barwaye ALS?

Hariho ubufasha burambuye kuri bombi abantu barwaye ALS n'imiryango yabo. Ibyo birimo amashami ya ALS Association atanga inguzanyo z'ibikoresho, amatsinda y'ubufasha, n'ibikoresho by'uburezi. Akarere kenshi gatanga ubufasha, serivisi zo kugisha inama, na gahunda zo gufasha mu by'amafaranga. Amakipe y'abaganga akenshi aba afite abakozi b'imibereho bashobora guhuza imiryango n'ibikoresho bikwiye byo mu karere no mu gihugu.