Ufuna ukufunda nge-ALS (Amyotrophic Lateral Sclerosis), isifo esithinta isimiso sakho sezinzwa?

Ingabe ngezinye izikhathi uzizwa sengathi uyahlanya, izitho zakho zomzimba ziyathuthumela, noma amazwi akho ayathuthumela uma ukhuluma? Lezi kungaba izinto ezivamile. Kodwa-ke, akuvamile, ukuthi zibe uphawu lokuthile okungathi sína kakhulu. Namuhla sizokhuluma ngesimo esiyinkimbinkimbi kancane, kodwa kubaluleke kakhulu ukuqaphela. Leso yi -ALS, noma i-Amyotrophic Lateral Sclerosis .

Kuyini i-ALS (Amyotrophic Lateral Sclerosis)? Masiyiqonde kalula!

Cabanga ngobuchopho bakho kanye nomgogodla (intambo yemizwa engaphakathi komgogodla wakho) njengeyunithi yokulawula eyinhloko yekhompyutha. Lawa yiwo alawula konke emzimbeni wakho. Kulesi simiso, kunezinhlobo ezikhethekile zamaseli abizwa ngokuthi 'ama-neurons' . Asebenza njengezithunywa ezincane. Lawa maseli emizwa athwala imiyalezo evela ebuchosheni eya emilenzeni nasemisipheni yethu, ewatshela ukuthi enze lokhu.

Manje, kulesi sifo esibizwa ngokuthi i-ALS, okwenzekayo ukuthi amangqamuzana ezinzwa (`ama-neurons') okukhulunywe ngawo alimele . Ikakhulukazi, amangqamuzana ezinzwa alawula ukunyakaza kwemizimba yethu yiwona ahloselwe kakhulu lapha. Kunjengokungathi lawo manxusa awakwazi ukwenza umsebenzi wawo. Kwenzekani-ke? Imiyalezo evela ebuchosheni ayihambi kahle emisipheni. Kancane kancane, lesi simo siba sibi kakhulu .

Ekuqaleni, ungase uzizwe ubuthakathaka kancane ezithweni zakho, njengokungathi imisipha yakho iyanyakaza ('ukunyakaza kwemisipha') . Ungase ukuthole kunzima ukuhamba wedwa, ukwelula isandla sakho ukuze uthathe okuthile, ukuhlafuna ukudla, noma ukukhuluma ngokucacile. Ngokuhamba kwesikhathi, imisipha, ngenxa yokungasetshenziswa, iqala ukunyakaza ('ukunyakaza') . Kufana nomshini ongasetshenziswa futhi ogqwala. Ekugcineni, lesi simo singathinta ukuphefumula kwakho. Ngezinye izikhathi kungaba yingozi empilweni.

Cishe uke wezwa ngesifo sikaLou Gehrig . Yilokho okushiwo yi-ALS. ULou Gehrig wayengumdlali we-baseball odumile kakhulu ngawo-1920 nawo-1930. Wayenalesi sifo futhi.

Ngisho nasezweni elifana neMelika, izibalo zibonisa ukuthi abantu abangaba ngu-5,000 batholakala benesifo se-ALS minyaka yonke. Ngakho-ke, nakuba lokhu kungavamile kakhulu, kufanele kubhekwe njengesimo esingakhula kunoma ubani.

Akukho ukwelashwa kwalokhu okwamanje . Kodwa ungakhathazeki. Izindlela zokwelapha ziyathuthuka usuku nosuku. Ngokuhlanganisa kahle ukwelashwa, kungenzeka ukulawula ukusabalala kwesifo ngokushesha futhi kuthuthukiswe ikhwalithi yokuphila ngezinga elikhulu.

Kunezinhlobo ezimbili eziyinhloko ze-ALS.

I-ALS ingahlukaniswa ngezinhlobo ezimbili eziyinhloko ngokusekelwe endleleni ekhula ngayo:

1. I-ALS engavamile: Lolu uhlobo oluvame kakhulu.Cishe ama-90% eziguli ezine-ALS angaphansi kwalesi sigaba. Lokhu kumane kuyisenzakalo esingahleliwe. Lokho kusho ukuthi akulona ufuzo. Kunzima ukusho ukuthi kungani kwenzeka.

2. I-ALS Yomndeni: Lokhu akuvamile kangako, kubalelwa cishe ku-10% wamacala. Kubangelwa izinguquko zofuzo . Lokhu kusho ukuthi lesi simo sibangelwa ukukhubazeka kwezakhi zofuzo ezizuzwe kumama noma kubaba.

Ziyini izimpawu ze-ALS? Ake sibheke.

Izimpawu ze-ALS zingahluka kancane kumuntu nomuntu, futhi ziyashintsha ngokuhamba kwesikhathi. Ekuqaleni, ezinye izimpawu zingase zibe zicashile kangangokuthi cishe azibonakali.

• Ubuthakathaka bemisipha ezingalweni, emilenzeni nasentanyeni: Ukuzizwa sengathi awusaphili. Kunzima ukuphakamisa noma ukubamba into ethile.
• Ukuqaqamba kwemisipha: Ukuqina kwemisipha ngokuzumayo, okubuhlungu.
• Ukuzwa ukudikiza emisipheni yezingalo, imilenze, amahlombe kanye/noma ulimi: njengokungathi kukhona ukudikiza okuncane okuvela ngaphakathi.
• Ukuqina kwemisipha ('ukuqaqamba'): Umuzwa wokuqina, njengomlenze oqinile, wokhuni, njengokungathi kunzima ukuwugoba noma ukuwuqondisa.
• Ubunzima bokukhuluma: Inkulumo engacacile, ubunzima bokuphimisa amagama ngendlela efanele, kanye nokushintsha kwezwi.
• Ubunzima bokugwinya noma ukuvuza amathe njalo.
• Ukuzizwa ukhathele ngaso sonke isikhathi (ukukhathala).
• Ubunzima bokugwinya ukudla noma amanzi (i-dysphagia): Umuzwa wokuthi ukudla kubambekile noma ukuthi kunzima ukugwinya.
• Ukuqhuma kwemizwa okungazelelwe, njengokuhleka noma ukukhala, okungalawuleki: Ungakhala ungazizwa udangele, noma ungahleka ngaphandle kwesizathu. Lokhu kubizwa nangokuthi 'i-Pseudobulbar affect' ngokwezokwelapha.

Ezigabeni zokuqala, izimpawu ezivame kakhulu ubuthakathaka nokuqina kwezingalo nemilenze, ubunzima bokukhuluma, kanye nobunzima bokugwinya. Lokhu kungenza kube nzima ukwenza imisebenzi yansuku zonke njengokubhala nokudla. Ngokuhamba kwesikhathi, lezi zimpawu zivame ukusabalala emzimbeni wonke. Kodwa-ke, ukuthi lezi zimpawu zithuthuka ngokushesha kangakanani kuyahlukahluka kuye ngomuntu nomuntu.

Njengoba izimpawu ziba zimbi kakhulu , kungaba nzima ukuphefumula, ukuma, noma ukuhamba. Ungase futhi wehle isisindo ngokuzumayo. Uma unalezi zimpawu futhi zibonakala zimbi kakhulu, qiniseka ukuthi ubona udokotela . Uma unenkinga yokuphefumula , kuyisimo esiphuthumayo futhi kufanele uye esibhedlela ngokushesha.

Kungani lesi sifo esibizwa ngokuthi i-ALS senzeka? Iyini imbangela?

Eqinisweni, abacwaningi abakakatholi ukuthi yini ngempela ebangela i-ALS, kodwa bakholelwa ukuthi kungaba yinhlanganisela yezici.

• Izakhi zofuzo:Sesike sakhuluma nge-ALS ezuzwe njengefa ngaphambili. Ngakho-ke, kunezinguquko ezithile zezakhi zofuzo ezihilelekile. Lezi zinguquko zezakhi zofuzo zibonakala cishe kuma-70% eziguli ze-ALS ezizuzwe njengefa kanye naphakathi kwama-5% nama-10% eziguli ze-ALS ezingavamile. Ikakhulukazi, izinguquko zezakhi zofuzo `C9orf72`, `SOD1`, `TARDBP` kanye ne-`FUS` yizona ezibikwa kakhulu. Kutholakale ukuthi izakhi zofuzo ezingaphezu kwama-40 zihlotshaniswa nalokhu.
• Izici zemvelo: Ngezinye izikhathi ubuthi ( njengomthofu noma i-mercury ), amagciwane athile, noma ukulimala emzimbeni kucatshangwa ukuthi kudlala indima, kodwa lokhu kusacwaningwa.

Lokho abacwaningi abakwazile ukukuqinisekisa ukuthi lesi sifo silimaza ama-motor neuron ethu. Lawa ma-motor neuron angamaseli ezinzwa alawula izinto esizenzayo ngamabomu, njengokukhuluma, ukuhlafuna ukudla, ukuhambisa izitho zethu kanye nokuphefumula.

Cabanga ukuthi ubuchopho bakho nemisipha yakho kunokuxhumana okufana nocingo. Amaseli ezinzwa athumela imiyalezo emisipheni ngalo mugqa, ebatshela ukuthi benze lokhu, benze lokho. Ku-ALS, isignali kulo mugqa wocingo iyaphambuka . Kufana nokulahlekelwa ukwamukelwa ocingweni. Imiyalezo evela ebuchosheni eya emisipheni iyaqhekeka, ayiveli ngokucacile. Ekugcineni, lokhu kuxhumana kuyalahleka ngokuphelele, njengokungathi ucingo lunqanyuliwe. Ngemuva kwalokho, lawo maseli ezinzwa awakwazi ukuthumela imiyalezo emisha. Yingakho uthola lezo zimpawu engizikhulumile ekuqaleni.

Ingabe i-ALS iyisifo esizuzwe njengefa?

Ezinye izinhlobo ze-ALS ziwufuzo, okusho ukuthi zingadluliselwa esizukulwaneni kuya kwesinye. Ungase uthole izinguquko zofuzo ezibangela i-ALS kubazali bakho. Kodwa-ke, lolu hlobo lwe-ALS (`i-ALS ezuzwe njengefa`) aluvamile kakhulu . Esikhathini esiningi, izinguquko zofuzo zenzeka ngokungahleliwe, okusho ukuthi zingenzeka ngisho noma kungekho muntu emndenini wakho oke waba nalesi simo ngaphambili.

Ubani osengozini enkulu yokuthola i-ALS?

Nakuba noma ubani angaba ne-ALS, kutholakale ukuthi abanye abantu basengozini enkulu kancane kunabanye.

• Ubudala: Abantu abaneminyaka ephakathi kuka-55 no-75 banamathuba amaningi okuba nezimpawu.
• Uhlanga kanye nobuzwe: Idatha ikhombisa ukuthi abantu abamhlophe (abangewona amaHispanic) banamathuba amaningi okuthola i-ALS.
• Ubulili: Eqenjini leminyaka elingaphansi kweminyaka engu-55, amadoda asengozini enkulu yokuthola lesi sifo kunabesifazane.
• Amasosha Asempini: Ezinye izifundo zisikisela ukuthi amalungu enkonzo yezempi angase abe nengozi ephezulu kancane. Abacwaningi bakholelwa ukuthi lokhu kungenzeka kungenxa yokuchayeka ezintweni zemvelo (njengobuthi noma izibulala-zinambuzane).) noma izingozi zomzimba ezenzeka ngesikhathi somsebenzi.

Yiziphi izinkinga ezingaba khona ze-ALS?

Njengoba izimpawu zalesi sifo ziba zimbi kakhulu, ngeshwa, isikhathi sokuphila singafinyezwa . Ukufunda ngalesi sifo nokubhekana naso nsuku zonke kungaba nzima kakhulu engqondweni. Ungase uzizwe ukhungathekile, ulahlekile, ungenathemba, futhi ucindezelekile. Ngenxa yalokho, abantu abaningi abatholakale benesifo se-ALS nabo babhekana nezinkinga zempilo yengqondo njengokucindezeleka nokukhathazeka .

Kukhona odokotela nabahlengikazi abaningi abangakusiza ukuthi unakekele impilo yakho engokomzimba. Kodwa kubalulekile futhi ukunakekela impilo yakho yengqondo . Uma udinga usizo, khuluma nethimba lakho lezokwelapha noma umeluleki wezempilo yengqondo .

Odokotela bayixilonga kanjani ngokunembile i-ALS?

Udokotela uzokuhlola ngokomzimba kuqala (`ukuhlolwa ngokomzimba`) , bese enza 'ukuhlolwa kwemizwa' . Ngaphezu kwalokho, kwenziwa ezinye izivivinyo eziningana ukuze kutholakale ukuthi une-ALS yini.

Awukwazi ukubona ngokushesha ukuthi une-ALS. Kungase kudingeke ubonane nodokotela wakho izikhathi eziningana, noma kungase kudingeke ubonane nochwepheshe abaningana. Udokotela wakho uzoyala ukuhlolwa okuhlukahlukene ukuze ahlole izimpawu zakho futhi abone ukuthi zithinta kanjani umzimba wakho. Kunezinye izifo eziningi ezinezimpawu ezifana ne-ALS. Ngakho-ke, kubalulekile ukwenza zonke lezi zivivinyo ezahlukene ukuze uthole ukuxilongwa okunembile .

Yiziphi izivivinyo ezisetshenziswa ukuxilonga i-ALS?

Ukuze uqinisekise ukuxilongwa, kungadingeka wenze izivivinyo eziningana, njenge:

• Ukuhlolwa kwegazi: Ukuqinisekisa ukuthi azikho ezinye izimo zezokwelapha.
• Ukuhlolwa komchamo: Lokhu kwenziwa futhi ukuze kutholakale ezinye izimbangela.
• I-Electromyogram (EMG): Lokhu kusetshenziselwa ukuhlola ukusebenza kwemisipha. Kuhlola ukuthi imiyalezo ithunyelwa kahle yini isuka ezinzwa iye emisipheni.
• Ucwaningo lokuqhutshwa kwemizwa: Lokhu kuhlola ukuthi imizwa ingathumela kahle kangakanani izimpawu.
• I-Magnetic Resonance Imaging (MRI): Lokhu kuthatha izithombe zobuchopho bakho noma zomgogodla ukuze kubonakale ukuthi kukhona yini umonakalo. Kungabuye kuhlole ukuthi ezinye izimo (njengesimila) zibangela izimpawu.

Yiziphi izindlela zokwelapha i-ALS? Nakuba kungekho ikhambi, ingabe ikhona impumuzo?

Ngeshwa, alikho ikhambi okwamanje elingalungisa umonakalo kumaseli ezinzwa obangelwa yi-ALS. Kodwa ungakhathazeki. Ukwelashwa kungalibazisa ukuqhubeka kwezimpawu futhi kukusize uhlale ukhululekile ngangokunokwenzeka.

Ithimba lakho lezokwelapha lingancoma izindlela zokwelapha ezifana nalezi:

• Imithi
• Izindlela zokwelapha ezahlukahlukene noma izinhlelo zokuvuselela
• Ukusekelwa kokudla okunempilo
• Ukusekelwa kobunzima bokuphefumula

Njengoba isifo siqhubeka, ungadinga izinhlobo ezahlukene zokwelashwa noma ukwelashwa okwengeziwe. Ngaphezu kwalokho, ungathola ukunakekelwa okusekelayo okuzokusiza ukuthi uphile ngokuzimela nangokunethezekile ngangokunokwenzeka isikhathi eside ngangokunokwenzeka.

Imithi enikezwa i-ALS

Kunezinhlobo ezine zemithi evunyelwe yi-United States Food and Drug Administration (FDA) yokwelapha i-ALS:

• I-Riluzole: Lokhu kukholakala ukuthi kunciphisa umonakalo kuma-motor neuron. Kungasiza futhi ekwandiseni ukusinda ngezinyanga ezimbalwa.
• I-Edaravone: Lo muthi unganciphisa izinga lokulahlekelwa yimisipha.
• I-Sodium phenylbutyrate/taursodiol: Lokhu kungalawula izinga lokuqhubeka kwezimpawu.
• I-Tofersen: Lo muthi unganciphisa umonakalo othile kumaseli ezinzwa. Lokhu kungaba usizo uma udokotela wakho ethole ushintsho ku-gene ebizwa ngokuthi i-SOD1.

Ngaphezu kwale mithi eyinhloko, kuneminye imithi engasiza ukulawula izimpawu zakho. Isibonelo, ungasebenzisa imithi yokucindezeleka kwemisipha, ukuqina, ukuqunjelwa ngokweqile, ubuhlungu, kanye nezinkinga zempilo yengqondo.

Izindlela zokwelapha ezahlukahlukene (`Ukwelashwa`)

Udokotela wakho angase ancome izindlela ezahlukene zokwelapha noma izinsizakalo zokuvuselela, njenge:

• Ukwelashwa ngokomzimba: Lokhu kukusiza ukuthi uhlale uzimele futhi uphephile isikhathi eside ngangokunokwenzeka. Kukufundisa ukuzivocavoca okulula okuqinisa imisipha futhi kukhuthaze impilo yonke.
• Ukwelashwa Komsebenzi: Lokhu kufundisa amasu okwenza imisebenzi yansuku zonke ngaphandle kokuzikhandla, usebenzisa amadivayisi okusiza njengezihlalo zabakhubazekile noma ama-braces.
• Ukwelapha ngenkulumo: Lokhu kukusiza ukuthi ugwinye futhi ukhulume ngokuphephile. Kukufundisa nezindlela zokuxhumana ngaphandle kwamazwi (isib. izimpawu, ukubhala) ukuze kukusize ukhulume isikhathi eside ngangokunokwenzeka futhi wonge amandla.

Ukusekelwa kokudla okunempilo

Kumuntu one-ALS, ngezinye izikhathi kungaba nzima ukuthola ukudla okunomsoco akudingayo. Ubunzima bokugwinya bungabangela ukwehla kwesisindo futhi kwenze kube nzima ukuthola amavithamini namaminerali akudingayo.

Uchwepheshe wezokudlaKuzokusiza ugweme ukudla okunzima ukukugwinya futhi udale uhlelo lokudla olunikeza inani elifanele lama-calories, i-fiber, kanye noketshezi. Ukwelulekwa ngokudla okunempilo kungakusiza ukuthi ugcine ukudla okunempilo. Njengoba ukugwinya kuba nzima, isazi sokudla okunempilo singaphakamisa nezindlela zokudla ezihlukile ezifanele.

Uma kudingeka, kungasetshenziswa ipayipi lokuncelisa . Lokhu kunganciphisa ingozi yokuthi ukudla noma uketshezi kungene emaphashini bese kubangela izimo ezinjengokuklinywa noma i-pneumonia .

Ukusekelwa kokuphefumula

Njengoba i-ALS iqhubeka, ukuphefumula kungaba nzima. Kukhona indlela ebizwa ngokuthi i-noninvasive ventilation (NIV) . Lokhu kuhilela ukusebenzisa imaski emboza impumulo nomlomo ukuze ikusize uphefumule. Ingasetshenziswa kuphela ebusuku ekuqaleni, kodwa kamuva ingadinga ukusetshenziswa usuku lonke.

Ngokuhamba kwesikhathi, ungadinga ukuphefumula ngomshini, umshini wokuphefumula obizwa ngokuthi i-respirator, ukukusiza ukuthi uphefumulele ngaphakathi nangaphandle kwamaphaphu akho. Uma unenkinga yokuphefumula, ikakhulukazi lapho ulele phansi noma wenza noma yini, tshela ithimba lakho lezempilo. Bangakhuluma nawe ngezinketho zokukusiza ukuthi uphefumule kalula.

Kufanele ubonane nini nodokotela? Qaphela lezi zinto

Uma uhlangabezana nanoma yiziphi zalezi zimpawu, bheka udokotela:

• Uma uzizwa sengathi unenkinga yokwenza imisebenzi yakho yansuku zonke .
• Uma izimpawu zibonakala ziba zimbi kakhulu .
• Uma usesimweni lapho ungakwazi khona ukuhamba wedwa.
• Uma ukwelashwa kubangela imiphumela emibi .

Sesikhulume kakade ngendlela i-ALS engakwenza kube nzima ngayo ukuphefumula. Nazi ezinye izimpawu zobunzima bokuphefumula okufanele zikushukumisele ukubona udokotela:

• Uma uzizwa uphelelwa umoya , ngisho nalapho uphumule.
• Uma ukukhwehlela kubonakala kubuthakathaka .
• Uma umphimbo namaphaphu kungenakuhlanzwa kalula .
• Uma amathe amaningi eqoqana.
• Uma ungakwazi ukulala embhedeni (ngoba uzizwa ucindezekile).
• Uma uthola izifo zesifuba njalo (i-pneumonia) .

Lezi zimpawu zingaholela esimweni esibizwa ngokuthi ukwehluleka kokuphefumula . Lokhu kusho ukuthi awukwazi ukuphefumula umoya-mpilo owanele ukuze uthole umoya-mpilo odingwa ngumzimba wakho. Lokhu kusongela impilo . Ngakho-ke, uma unenkinga yokuphefumula, iya ekamelweni lezimo eziphuthumayo ngokushesha .

Ingabe ikhona indlela yokuvimbela i-ALS?

Eqinisweni, i-ALS ingavinjelwa.Akukho ikhambi eliqinisekisiwe okwamanje. Ucwaningo luyaqhubeka ukuze kuqondwe kabanzi izimbangela kanye nezici eziyingozi zalokhu, kanye nokuthuthukisa izindlela zokuvimbela esikhathini esizayo.

Ungaphila isikhathi esingakanani ne-ALS? Uyini umbono?

Isilinganiso sokuphila kwe-ALS siyiminyaka emithathu kuya kwemihlanu ngemva kokuxilongwa. Kodwa-ke, kulinganiselwa ukuthi cishe abantu abangu-30% baphila iminyaka emihlanu noma ngaphezulu, kanti phakathi kuka-10% no-20% baphila okungenani iminyaka eyishumi. Isimo sakho singase sihluke kulezi zibalo . Ngakho-ke, khuluma nodokotela wakho ukuze ufunde kabanzi ngesimo sakho.

Isibikezelo se-ALS asisihle kangako, ngenxa yendlela esithinta ngayo ukusebenza kwama-motor neuron. Umbono wakho uncike ekutheni umonakalo kuma-neuron uvela ngokushesha kangakanani. Nakuba kungekho ukwelashwa okungabuyisela emuva lo monakalo, udokotela wakho angakunikeza izinketho zokunciphisa ijubane lokuqhubeka kwezimpawu.

Okwamanje akukho ukwelashwa kwe-ALS.

Uma une-ALS, ungase ube nesifiso sokubamba iqhaza ocwaningweni lwezokwelapha . Lezi zifundo zisiza abacwaningi ukuthi bathuthukise izindlela zokwelapha ezintsha futhi baqonde kangcono lesi sifo.

Ekugcineni, into ebaluleke kakhulu okufanele uyikhumbule!

Uma uthola ukuthi une-amyotrophic lateral sclerosis, noma i-ALS, ungase ube nemibuzo nemizwa eminingi.

Ungase uzibuze, 'Kungani lokhu kwenzeke kimi? Yini ebangele lokhu?' Ungase uzizwe ukhungathekile, udabuke, futhi ukhungathekile uma ungasakwazi ukwenza izinto ezazivame ukuza kuwe, njengokukama izinwele zakho, ukudla ukudla okumnandi, noma ukukhuluma nabantu obathandayo. Lokhu kungaholela ekucindezelekeni nasekukhathazekeni, ikakhulukazi njengoba izimpawu zakho ziba zimbi kakhulu.

Kungakhathaliseki ukuthi ukuphi noma uzizwa kanjani, ithimba lakho lezokwelapha lilapha ukuzokusiza . Ukwelashwa kwe-ALS kuyathuthuka nsuku zonke, futhi ukwelashwa okusha kuyacwaningwa futhi kuhlolwa. Nakuba okwamanje kungekho ikhambi, ukwelashwa okutholakalayo kunganciphisa ukuqhubeka kwezimpawu futhi kukuvumele ukuthi uchithe isikhathi esiningi nabathandekayo bakho. Ungalokothi uzizwe unesizungu. Cela usizo nokusekelwa. Amandla akho ayimpahla yakho enkulu.

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