Ngaba ufuna ukufunda nge-ALS (Amyotrophic Lateral Sclerosis), isifo esichaphazela inkqubo yakho yemithambo-luvo?

Ngaba ngamanye amaxesha uziva ngathi uyaphambana, amalungu akho aphelelwe ngamandla, okanye amazwi akho ayabhideka xa uthetha? Ezi zizinto eziqhelekileyo. Nangona kunjalo, azifane zibe luphawu lwento enzulu ngakumbi. Namhlanje siza kuthetha ngemeko enzima kancinci, kodwa kubaluleke kakhulu ukuba uyiqaphele. Yiyo loo nto i-ALS, okanye i-Amyotrophic Lateral Sclerosis .

Yintoni i-ALS (Amyotrophic Lateral Sclerosis)? Masiyiqonde ngokulula!

Cinga ngengqondo yakho kunye nomqolo (intambo yemithambo-luvo engaphakathi komqolo wakho) njengeyunithi yokulawula ephambili yekhompyutha. Ezi zezona zilawula yonke into emzimbeni wakho. Kule nkqubo, kukho iintlobo ezikhethekileyo zeeseli ezibizwa ngokuba yi -'neurons' . Zisebenza njengezithunywa ezincinci. Ezi seli zemithambo-luvo zithwala imiyalezo esuka engqondweni iye kwimilenze nakwimisipha yethu, zisithi mazikwenze oku.

Ngoku, kwesi sifo sibizwa ngokuba yi-ALS, okwenzekayo kukuba iiseli zemithambo-luvo (`ii-neurons') ozikhankanyileyo zonakele . Ngokukodwa, iiseli zemithambo-luvo ezilawula iintshukumo zomzimba wethu zezona zinto ziphambili apha. Kungathi abo bathunywa abakwazi ukwenza umsebenzi wabo. Kwenzeka ntoni ke ngoko? Imiyalezo evela engqondweni ayihambi kakuhle kwimisipha. Kancinci kancinci, le meko iba mandundu .

Ekuqaleni, ungaziva ubuthathaka kancinci kwimilenze yakho, ngokungathi izihlunu zakho ziyashukuma ('ukushukuma kwemisipha') . Unokufumana kunzima ukuhamba wedwa, ukolula isandla sakho ukuze uthabathe into ethile, ukuhlafuna ukutya, okanye ukuthetha ngokucacileyo. Ekuhambeni kwexesha, izihlunu, ngenxa yokungasetyenziswa, ziqala ukushukuma ('ukushukuma') . Kufana nomatshini ongasetyenziswayo kwaye ugqwala. Ekugqibeleni, le meko inokuchaphazela ukuphefumla kwakho. Ngamanye amaxesha inokuba yingozi ebomini.

Mhlawumbi ukhe weva ngesifo sikaLou Gehrig . Yiyo loo nto i-ALS ithetha ngayo. ULou Gehrig wayengumdlali webhola ekhatywayo odumileyo ngeminyaka yoo-1920 noo-1930. Wayenaso nesi sifo.

Kwanakwilizwe elifana neMelika, izibalo zibonisa ukuba malunga nabantu abangama-5,000 bafunyanwa benesifo se-ALS rhoqo ngonyaka. Ngoko ke, nangona oku kungaqhelekanga, kufanele kuthathwe njengesimo esinokukhula nakubani na.

Akukho nyango loku okwangoku . Kodwa ungakhathazeki. Iindlela zonyango ziyaphucuka imihla ngemihla. Ngokudityaniswa konyango olufanelekileyo, kunokwenzeka ukulawula ukusasazeka ngokukhawuleza kwesi sifo nokuphucula umgangatho wobomi ngokubanzi.

Kukho iintlobo ezimbini eziphambili ze-ALS.

I-ALS inokwahlulwa ibe ziintlobo ezimbini eziphambili ngokusekelwe kwindlela ekhula ngayo:

1. I-ALS engaqhelekanga: Olu lolona hlobo luqhelekileyo.Malunga nama-90% ezigulane ze-ALS zikolu didi. Oku kwenzeka ngokungacwangciswanga. Oko kuthetha ukuba akuyonto yemfuza. Kunzima ukutsho kanye isizathu sokuba kwenzeke.

2. I-ALS yosapho: Oku akuvamile kangako, kubalelwa malunga ne-10% yamatyala. Kubangelwa lutshintsho lwemfuza . Oku kuthetha ukuba le meko ibangelwa sisiphene kwiimfuza ezizuzwe njengelifa kumama okanye kutata.

Zithini iimpawu ze-ALS? Makhe sijonge.

Iimpawu ze-ALS zinokwahluka kancinci kumntu nomntu, kwaye ziyatshintsha ngokuhamba kwexesha. Ekuqaleni, ezinye iimpawu zinokuba zingabonakali kangako kangangokuba azibonakali.

• Ubuthathaka bemisipha ezingalweni, emilenzeni nasentanyeni: Ukuziva ngathi awusaphili. Kunzima ukuphakamisa okanye ukubamba into ethile.
• Ukuqaqamba kwemisipha: Ukuqina kwemisipha ngequbuliso, okubuhlungu.
• Ukuziva ushukuma kwizihlunu zeengalo, imilenze, amagxa kunye/okanye ulwimi: ngokungathi kukho ukushukuma okuncinci okuvela ngaphakathi.
• Ukuqina kwemisipha ('spasticity'): Ukuziva ukuqina, njengelungu eliqinileyo, eliyiplanga, ngokungathi kunzima ukuligoba okanye ukulilungisa.
• Ubunzima bokuthetha: Intetho engacacanga, ubunzima bokubiza amagama ngokuchanekileyo, kunye notshintsho kwilizwi.
• Ubunzima bokuginya okanye ukuvuza amathe rhoqo.
• Ukuziva udiniwe ngalo lonke ixesha (ukudinwa).
• Ubunzima bokuginya ukutya okanye amanzi (dysphagia): Ukuziva ngathi ukutya kubambekile okanye kunzima ukuginya.
• Ukuphazamiseka ngequbuliso kweemvakalelo, njengokuhleka okanye ukukhala, okungalawulekiyo: Ungalila ungenalusizi, okanye ungahleka ngaphandle kwesizathu. Oku kukwabizwa ngokuba yi-'Pseudobulbar affect' ngokwezonyango.

Kwinqanaba lokuqala, ezona mpawu zixhaphakileyo bubuthathaka kunye nokuqina kweengalo nemilenze, ubunzima bokuthetha, kunye nobunzima bokuginya. Oku kunokwenza kube nzima ukwenza imisebenzi yemihla ngemihla efana nokubhala nokutya. Ekuhambeni kwexesha, ezi mpawu zihlala zisasazeka emzimbeni wonke. Nangona kunjalo, ukuba ezi mpawu zikhawuleza kangakanani ukukhula ziyahluka kumntu nomntu.

Njengoko iimpawu zisiba mandundu , kunokuba nzima ukuphefumla, ukuma, okanye ukuhamba. Usenokulahlekelwa bubunzima ngequbuliso. Ukuba unale mpawu kwaye zibonakala zisiba mandundu, qiniseka ukuba ubona ugqirha . Ukuba unengxaki yokuphefumla , yingxamiseko kwaye kufuneka uye esibhedlele ngoko nangoko.

Kutheni esi sifo sibizwa ngokuba yi-ALS senzeka? Yintoni unobangela?

Enyanisweni, abaphandi abakafumani ukuba yintoni kanye kanye ebangela i-ALS, kodwa bakholelwa ukuba isenokuba yindibaniselwano yezinto.

• I-genetics:Sikhe sathetha nge-ALS yelifa ngaphambili. Ngoko ke, kukho utshintsho oluthile lwezakhi zofuzo olubandakanyekayo. Olu tshintsho lwezakhi zofuzo lubonwa malunga nama-70% ezigulana ze-ALS zelifa kunye naphakathi kwe-5% kunye ne-10% yezigulana ze-ALS ezihlala zihleli. Ngokukodwa, utshintsho kwizakhi zofuzo `C9orf72`, `SOD1`, `TARDBP` kunye ne-`FUS` zezona zixelwayo kakhulu. Kufunyenwe ukuba zingaphezulu kwama-40 iizakhi zofuzo ezinxulunyaniswa noku.
• Izinto ezisingqongileyo: Ngamanye amaxesha iityhefu (ezifana nelothe okanye i-mercury ), iintsholongwane ezithile, okanye ukwenzakala emzimbeni kucingelwa ukuba zidlala indima, kodwa ezi zinto zisephantsi kophando.

Oko abaphandi bakwazile ukukuqinisekisa kukuba esi sifo siyonakalisa ii-motor neurons zethu. Ezi neurons ziiseli zemithambo-luvo ezilawula izinto esizenzayo ngabom, njengokuthetha, ukuhlafuna ukutya, ukushukumisa amalungu ethu, nokuphefumla.

Khawuthelekelele ukuba ingqondo yakho kunye nemisipha zinonxibelelwano olufana nomgca wefowuni. Iiseli zemithambo-luvo zithumela imiyalezo kwimisipha ngalo mgca, zisithi mazenze oku, zenze okuya. Kwi-ALS, isignali kulo mgca wefowuni iyaphambuka . Kufana nokulahlekelwa kukwamkelwa kwifowuni. Imiyalezo esuka ebuchotsheni eya kwimisipha iyaqhekeka, ayiveli ngokucacileyo. Ekugqibeleni, olu nxibelelwano luyalahleka ngokupheleleyo, njengokuba umnxeba unqanyulwe. Emva koko, ezo seli zemithambo-luvo azikwazi ukuthumela imiyalezo emitsha. Yiyo loo nto ufumana ezo mpawu ndizikhankanyileyo ngaphambili.

Ngaba i-ALS sisifo esizuzwe njengelifa?

Ezinye iintlobo ze-ALS ziyimfuza, oko kuthetha ukuba zinokudluliselwa kwizizukulwana ngezizukulwana. Usenokuzuza utshintsho lwemfuza olubangela i-ALS kubazali bakho. Nangona kunjalo, olu hlobo lwe-ALS (`i-ALS ezuzwe njengelifa`) aluxhaphakanga kakhulu . Uninzi lwexesha, utshintsho lwemfuza lwenzeka ngokungacwangciswanga, oko kuthetha ukuba lunokwenzeka nokuba akukho mntu kusapho lwakho okhe waba nale meko ngaphambili.

Ngubani osengozini enkulu yokuba ne-ALS?

Nangona nabani na enokuba ne-ALS, kufunyenwe ukuba abanye abantu basengozini enkulu kancinci kunabanye.

• Ubudala: Abantu abaphakathi kweminyaka engama-55 ukuya kwengama-75 banamathuba amaninzi okufumana iimpawu.
• Uhlanga kunye nohlanga: Idatha ibonisa ukuba abantu abamhlophe (abangengabo amaHispanic) banamathuba amaninzi okufumana i-ALS.
• Isini: Kubantu abangaphantsi kweminyaka engama-55 ubudala, amadoda anamathuba amaninzi okufumana esi sifo kunabafazi.
• Amajoni asele emfazweni: Ezinye izifundo zibonisa ukuba amalungu eenkonzo zasemkhosini anokuba nomngcipheko ophezulu kancinci. Abaphandi bakholelwa ukuba oku kusenokuba kungenxa yokuchatshazelwa zizinto ezingqongileyo (ezifana neetyhefu okanye izibulali-zinambuzane).) okanye iingozi ezenzekayo ngexesha lomsebenzi.

Ziziphi iingxaki ezinokubakho ze-ALS?

Njengoko iimpawu zesi sifo zisiba mandundu, ngelishwa, ixesha lokuphila linokuncipha . Ukufunda ngesi sifo nokujongana naso imihla ngemihla kunokuba nzima kakhulu engqondweni. Ungaziva uxinezelekile, ulahlekile, ungenathemba, kwaye uxinezelekile. Ngenxa yoko, abantu abaninzi abafunyaniswe bene-ALS nabo bafumana iingxaki zempilo yengqondo ezifana nokudakumba kunye nokuxhalaba .

Kukho oogqirha abaninzi kunye nabahlengikazi abanokukunceda ukhathalele impilo yakho yomzimba. Kodwa kubalulekile nokunyamekela impilo yakho yengqondo . Ukuba ufuna uncedo, thetha neqela lakho lezonyango okanye umcebisi wezempilo yengqondo .

Oogqirha bayixilonga njani ngokuchanekileyo i-ALS?

Ugqirha uza kuqala akuhlole ngokwasemzimbeni (`uvavanyo lomzimba`) , aze emva koko enze` uvavanyo lwemithambo-luvo` . Ukongeza, kwenziwa ezinye iimvavanyo ezininzi ukuze kuqinisekiswe ukuba unayo na i-ALS.

Awunakwazi ukubona kwangoko ukuba une-ALS. Kusenokufuneka ubone ugqirha wakho amaxesha amaninzi, okanye kusenokufuneka ubone iingcali ezininzi. Ugqirha wakho uya kuyalela iimvavanyo ezahlukeneyo ukuze ahlolisise ngakumbi iimpawu zakho aze abone indlela ezichaphazela ngayo umzimba wakho. Zininzi ezinye izifo ezineempawu ezifana neze-ALS. Ke ngoko, kubalulekile ukwenza zonke ezi vavanyo zahlukeneyo ukuze ufumane uxilongo oluchanekileyo .

Ziziphi iimvavanyo ezisetyenziselwa ukuxilonga i-ALS?

Ukuqinisekisa ukuxilongwa, kunokufuneka wenze iimvavanyo ezininzi, ezifana nezi:

• Uvavanyo lwegazi: Ukuqinisekisa ukuba akukho ezinye iimeko zempilo.
• Uvavanyo lomchamo: Olu vavanyo lwenziwa ukuze kuthintelwe ezinye izizathu.
• I-Electromyogram (EMG): Le isetyenziselwa ukujonga ukusebenza kwezihlunu. Ijonga ukuba imiyalezo ithunyelwa ngokufanelekileyo na ukusuka kwimithambo-luvo ukuya kwizihlunu.
• Uphononongo lokuqhuba imithambo-luvo: Olu vavanyo luvavanya ukuba imithambo-luvo ingazithumela kakuhle kangakanani na imiqondiso.
• I-Magnetic Resonance Imaging (MRI): Le mifanekiso ithatha ingqondo yakho okanye umqolo ukuze ibone ukuba akukho monakalo na. Ingajonga nokuba ezinye iimeko (ezifana nomhlaza) zibangela iimpawu.

Ziziphi iindlela zonyango lwe-ALS? Nangona kungekho nyango, ngaba ikho into encedayo?

Ngelishwa, akukho nyango okwangoku elinokubuyisela umva umonakalo kwiiseli zemithambo-luvo ezibangelwa yi-ALS. Kodwa ungakhathazeki. Unyango lunokucothisa ukuqhubela phambili kweempawu kwaye lukuncede uhlale ukhululekile kangangoko kunokwenzeka.

Iqela lakho lezonyango linokucebisa unyango olufana nolu:

• Amayeza
• Iindlela ezahlukeneyo zonyango okanye iinkqubo zokubuyisela kwimeko yesiqhelo
• Inkxaso yesondlo
• Inkxaso yobunzima bokuphefumla

Njengoko isifo siqhubeka, ungadinga iintlobo ezahlukeneyo zonyango okanye unyango oluninzi. Ukongeza, ungafumana uncedo oluza kukunceda uphile ngokuzimela nangokukhululekileyo kangangoko kunokwenzeka ixesha elide kangangoko kunokwenzeka.

Amayeza anikwa i-ALS

Kukho iintlobo ezine zamayeza avunyiweyo yi-United States Food and Drug Administration (FDA) kunyango lwe-ALS:

• I-Riluzole: Kukholelwa ukuba oku kunciphisa umonakalo kwii-motor neurons. Kunokunceda nokwandisa ixesha lokuphila ngeenyanga eziliqela.
• I-Edaravone: Eli yeza linokucothisa izinga lokulahlekelwa yimisipha.
• I-Sodium phenylbutyrate/taursodiol: Oku kunokulawula izinga lokuqhubela phambili kweempawu.
• I-Tofersen: Eli yeza linokunciphisa umonakalo kwiiseli zemithambo-luvo. Oku kunokuba luncedo ukuba ugqirha wakho ufumene utshintsho kwi-gene ebizwa ngokuba yi-SOD1.

Ukongeza kula mayeza aphambili, kukho amanye amayeza anokukunceda ukulawula iimpawu zakho. Umzekelo, ungasebenzisa amayeza okuqaqamba kwemisipha, ukuqina, ukuvuza amathe kakhulu, iintlungu, kunye neengxaki zempilo yengqondo.

Iindlela ezahlukeneyo zonyango (`Unyango`)

Ugqirha wakho unokucebisa iindlela ezahlukeneyo zonyango okanye iinkonzo zokubuyisela kwimeko yesiqhelo, ezinje:

• Unyango lomzimba: Oku kukunceda uhlale uzimele kwaye ukhuselekile ixesha elide kangangoko unako. Kukufundisa imithambo elula eqinisa izihlunu kwaye ikhuthaza impilo iyonke.
• Unyango lomsebenzi: Oku kufundisa amaqhinga okwenza imisebenzi yemihla ngemihla ngaphandle kokudinwa, usebenzisa izixhobo zokuncedisa ezifana nezitulo ezinamavili okanye izixhasi.
• Unyango lokuthetha: Oku kukunceda ukuginya nokunxibelelana ngokukhuselekileyo. Kukufundisa neendlela zokunxibelelana ngaphandle kokuthetha (umz., iimpawu, ukubhala) ukukunceda uthethe ixesha elide kangangoko unako kwaye wonge amandla.

Inkxaso yesondlo

Kumntu one-ALS, ngamanye amaxesha kunokuba nzima ukufumana izondlo azidingayo. Ubunzima bokuginya bunokubangela ukwehla kobunzima kwaye kwenze kube nzima ukufumana iivithamini kunye neeminerali azidingayo.

Ingcali yeZondloUkukunceda uphephe ukutya okunzima ukukuginya kwaye wenze isicwangciso sokutya esibonelela ngobungakanani obufanelekileyo beekhalori, ifayibha, kunye nolwelo. Ingcebiso ngesondlo inokukunceda ugcine ukutya okunempilo. Njengoko ukuginya kuba nzima ngakumbi, ingcali yesondlo ingacebisa nezinye iindlela zokutya ezifanelekileyo.

Ukuba kuyimfuneko, kungasetyenziswa ityhubhu yokondla . Oku kunokunciphisa umngcipheko wokungena kokutya okanye ulwelo emiphungeni nto leyo ebangela iimeko ezifana nokukrwitshwa okanye inyumoniya .

Inkxaso yokuphefumla

Njengoko i-ALS iqhubeka, ukuphefumla kunokuba nzima. Kukho indlela ebizwa ngokuba yi -noninvasive ventilation (NIV) . Oku kuquka ukusebenzisa imaski egubungela impumlo nomlomo ukukunceda uphefumle. Ingasetyenziswa ebusuku kuphela ekuqaleni, kodwa kamva inokufuneka isetyenziswe imini yonke.

Ekuhambeni kwexesha, ungadinga umoya ongeniswa ngomatshini, umatshini wokuphefumla obizwa ngokuba yi-respirator, ukukunceda uphefumle ngaphakathi nangaphandle kwemiphunga yakho. Ukuba unengxaki yokuphefumla, ingakumbi xa ulele phantsi okanye usenza nantoni na, xelela iqela lakho lezempilo. Bangathetha nawe ngeendlela zokukunceda uphefumle lula.

Ufanele uye nini kugqirha? Ziqaphele ezi zinto

Ukuba ufumana naziphi na kwezi mpawu, bona ugqirha:

• Ukuba uziva ngathi unengxaki yokwenza imisebenzi yakho yemihla ngemihla .
• Ukuba iimpawu zibonakala zisiba mandundu .
• Ukuba ukwimeko apho ungakwazi ukuhamba wedwa.
• Ukuba unyango lubangela iziphumo ebezingalindelekanga .

Sele sithethe ngendlela i-ALS enokwenza ngayo kube nzima ukuphefumla. Nazi ezinye zeempawu zobunzima bokuphefumla eziya kukubangela ukuba ubone ugqirha:

• Ukuba uziva uphelelwa ngumphefumlo , nokuba uphumle.
• Ukuba ukukhwehlela kubonakala kubuthathaka .
• Ukuba umphimbo kunye nemiphunga azikwazi ukucocwa lula .
• Ukuba amathe amaninzi aqokelelana.
• Ukuba awukwazi ukulala ebhedini (kuba uziva ufuthanisekile).
• Ukuba ufumana usulelo lwesifuba rhoqo (i-pneumonia) .

Ezi mpawu zingakhokelela kwimeko ebizwa ngokuba kukusilela kokuphefumla . Oku kuthetha ukuba awukwazi ukuphefumla ioksijini eyaneleyo ukuze ufumane ioksijini efunekayo emzimbeni wakho. Oku kusongela ubomi . Ngoko ke, ukuba unengxaki yokuphefumla, yiya kwigumbi likaxakeka ngoko nangoko .

Ngaba ikho indlela yokuthintela i-ALS?

Enyanisweni, i-ALS inokuthintelwa.Akukho nyango luqinisekisiweyo okwangoku. Uphando luyaqhubeka ukuze kuqondwe ngakumbi izizathu kunye nezinto ezibangela oku, kunye nokuphuhlisa iindlela zokuthintela kwixesha elizayo.

Ungaphila ixesha elingakanani une-ALS? Injani imeko yakho?

Umyinge wobomi obulindelekileyo be-ALS yiminyaka emithathu ukuya kwemihlanu emva kokufunyaniswa kwayo. Nangona kunjalo, kuqikelelwa ukuba malunga nama-30% abantu baphila iminyaka emihlanu nangaphezulu, kwaye phakathi kwe-10% kunye ne-20% baphila ubuncinane iminyaka elishumi. Imeko yakho inokwahluka kwezi zibalo . Ngoko ke, thetha nogqirha wakho ukuze ufunde okungakumbi ngemeko yakho.

Ingqikelelo ye-ALS ayilunganga kangako, ngenxa yendlela echaphazela ngayo ukusebenza kwee-motor neurons. Imbono yakho ixhomekeke ekubeni umonakalo owenzeka ngokukhawuleza kangakanani kwiiseli zemithambo-luvo. Nangona kungekho nyango lunokubuyisela lo monakalo, ugqirha wakho unokukunika iindlela zokucothisa ukuqhubela phambili kweempawu.

Okwangoku akukho nyango lwe-ALS.

Ukuba une-ALS, unokuba nomdla wokuthatha inxaxheba kuvavanyo lweklinikhi . Ezi zifundo zinceda abaphandi ukuba baphuhlise unyango olutsha kwaye basiqonde ngcono esi sifo.

Okokugqibela, eyona nto ibalulekileyo ekufuneka uyikhumbule!

Xa ufumanisa ukuba une-amyotrophic lateral sclerosis, okanye i-ALS, unokuba nemibuzo neemvakalelo ezininzi.

Usenokuzibuza, ‘Kutheni oku kwenzeke kum? Yintoni ebangele oku?’ Usenokuziva ukhathazekile, ulusizi, kwaye uxinezelekile xa ungasakwazi ukwenza izinto ebezisoloko zifika kuwe, ezinje ngokukama iinwele zakho, ukutya isidlo esimnandi, okanye ukuthetha nabantu obathandayo. Oku kunokukhokelela ekudandathekeni nasekuxhalabeni, ngakumbi njengoko iimpawu zakho zisiba mandundu.

Nokuba uphi na okanye uziva njani, iqela lakho lezonyango lilapha ukukunceda . Unyango lwe-ALS luyaphucuka yonke imihla, kwaye unyango olutsha luyaphandwa kwaye luvavanywa. Nangona kungekho nyango okwangoku, unyango olufumanekayo lunokucothisa ukuqhubela phambili kweempawu kwaye lukuvumele ukuba uchithe ixesha elingakumbi nabantu obathandayo. Ungaze uzive unesizungu. Cela uncedo kunye nenkxaso. Amandla akho yeyona nto ibalulekileyo kuwe.

I- ALS, i-Amyotrophic Lateral Sclerosis, i-Neuropathy, i-Muscular Dystrophy, i-Lou Gehrig's, ii-Neurons, ii-Motor Neurons, i-Respiratory Distress