Uzizwa sengathi izitho zakho zomzimba ziya ngokuya zilahlekelwa amandla azo? Lokhu kungaba yisifo se-Motor Neuron (MND)!

Wake wazizwa sengathi imilenze yakho iyaphela amandla lapho ukhuphuka izitebhisi, amazwi akho ayaphela lapho ukhuluma, noma into ethile iwa ngokuzumayo esandleni sakho? Ungase ucabange ukuthi lokhu kuyinto evamile, kodwa uma lezi zimpawu ziqhubeka, kuwumqondo omuhle ukukhathazeka kancane. Ngoba lezi kungaba izimpawu zokuqala zesimo esibizwa ngokuthi i-Motor Neuron Disease (MND) . Ngakho-ke, ake sixoxe ngalokhu ngemininingwane ethe xaxa namuhla.

Kuyini isifo se-motor neurone (MND)?

Kalula nje, isifo se-motor neurone (MND) yiqembu lezifo ezithinta uhlelo lwethu lwezinzwa. Sibangela ukuthi ama-motor neuron ethu afe kancane kancane. Manje ungase uzibuze ukuthi ayini la ma-motor neuron. Lawa amaseli ezinzwa alawula imisipha yethu. Lawa ma-motor neuron abalulekile kuyo yonke imisebenzi yethu, kusukela ekuphefumuleni, ekukhulumeni, ekugwinyeni nasekuhambeni.

Cabanga nje, imiyalezo evela ebuchosheni bethu (esiyibiza ngokuthi ama-upper motor neurons ) ifika emgogodleni wethu. Ukusuka lapho, leyo miyalezo idlula kuma -neuron aphansi emisipha yomzimba wethu. La ma-neuron aphezulu emisipha yiwo atshela ama-neuron aphansi ukuthi, "Kulungile, manje hambisa lo msipha kanje."

Manje, kwenzekani uma lezi zinzwa ezingezansi zemisipha zingakwazi ukuthumela imiyalezo emisipheni? Imisipha iqala kancane kancane ukuba buthaka futhi inciphe (ukuwohloka kwemisipha) . Futhi, lapho izinzwa ezingenhla zemisipha zingakwazi ukuthumela izimpawu emisipheni engezansi yemisipha, imisipha iba lukhuni futhi isebenze ngokweqile ( hyperreflexia ). Lokhu kwenza kube nzima ngathi ukwenza ukunyakaza ngokuzithandela, futhi kwenzeka kancane kakhulu. Ngokuhamba kwesikhathi, singase silahlekelwe ngisho nekhono lokuhamba nokulawula ezinye ukunyakaza.

Into ebalulekile ukuthi okwamanje ayikho ikhambi lesifo sezinzwa zomzimba. Lezi yizifo eziqhubekayo eziba zimbi ngokuhamba kwesikhathi. Kodwa-ke, kunezindlela zokwelapha ezingasiza ekunciphiseni umthelela walesi simo.

Yiziphi izinhlobo ze-MND ezikhona?

Odokotela bahlukanisa i-MND ngokuthi ithinta yini imithambo yegazi engenhla, imithambo yegazi engezansi, noma kokubili. Kunezinhlobo eziningana eziyinhloko ze-MND:

`I-Amyotrophic lateral sclerosis (ALS)`

Lolu uhlobo oluvame kakhulu lwe-MND. Abanye abantu balubiza nangokuthi isifo sikaLou Gehrig . I-ALS ithinta kokubili izinzwa zomzimba eziphezulu nezingezansi. Lokhu kungabangela ukulahlekelwa okusheshayo kokulawulwa kwemisipha, ekugcineni kuholele ekukhubazekeni . Odokotela abaningi ngezinye izikhathi basebenzisa amagama athi ALS kanye nesifo se-motor neurone ngokushintshana.

`I-Progressive bulbar palsy (PBP)`

Yilokhu okusihlaselayo.Izinzwa eziphansi zemisipha ezixhumanisa ne-brainstem (indawo ye-bulbar). I-brainstem yethu ilawula imisipha edingekayo ezintweni ezifana nokukhuluma, ukuhlafuna, nokugwinya. Elinye igama le-PBP yi-"progressive bulbar atrophy".

`Isifo sokuqaqamba kwemisipha esiyinhloko (i-PLS)`

Lokhu kuthinta kuphela imithambo yegazi engenhla. Ngokuvamile, lesi sifo siqala sithinte imilenze. Bese sithinta izingalo, izandla, nomzimba. Ekugcineni, singathinta imisipha esetshenziselwa ukukhuluma, ukugwinya, nokuhlafuna ukudla.

`Ukuwohloka kwemisipha okuqhubekayo (PMA)`

Kuthinta kuphela izinzwa ezingezansi zomzimba. Lesi simo sivame kakhulu kwabesilisa, futhi sivame ukuvela besebancane kunezinye izifo ze-MND eziqala kubantu abadala. Izimpawu zokuqala ubuthakathaka ezingalweni, bese zisakazekela emzimbeni ongezansi. Kungathinta nomzimba kanye nokuphefumula.

Ukuqina kwemisipha yomgogodla (SMA)

Lesi yisimo esizuzwa njengefa esithinta izinzwa ezingaphansi kwemisipha. Sibhekwa futhi njengembangela ehamba phambili yezakhi zofuzo yokufa kwezinsana . Ukuguquka kwezakhi zofuzo ku-'SMN1' kuholela ekulahlekelweni kwephrotheni 'SMN' . Lokhu kubhubhisa kancane kancane izinzwa ezingaphansi kwemisipha, okubangela ukuncipha kwemisipha nobuthakathaka, futhi ekugcineni kufe.

Isifo sikaKennedy

Kuhlotshaniswa nesakhi sofuzo esiku-chromosome X emadodeni. Kubangelwa ukuguquka kwezakhi zofuzo ze-androgen receptor . Ngokuhamba kwesikhathi, ubuthakathaka buyakhula ezingalweni nasemilenzeni, okuvame ukuqala ezindaweni ze-pelvic noma zamahlombe. Ubuhlungu kanye nokungazweli ezingalweni nasemilenzeni nakho kungase kwenzeke.

I-Post-polio syndrome (PPS)

Kwenzeka kubantu asebeluleme ku-polio. Kungavela eminyakeni engamashumi amane ngemva kokugula kwabo kokuqala. I-polio ingabangela umonakalo omkhulu ezinzwa zomzimba. Izimpawu ze-PPS zifaka phakathi ubuthakathaka bemisipha namalunga, ukukhathala, ubuhlungu obukhula ngokuhamba kwesikhathi, ukudikiza nokuncipha kwemisipha, kanye nokungakwazi ukubekezelela umkhuhlane.

Ziyini izimpawu ze-MND?

Izimpawu ze-MND aziveli ngokuzumayo, kodwa kancane kancane. Ezigabeni zokuqala, zingase zingabi sobala kakhulu.

Izimpawu zokuqala

• Ubunzima bokukhuphuka izitebhisi noma ukukhubeka njalo ngenxa yobuthakathaka emilenzeni noma emaqakaleni.
• Inkulumo engacacile (i-dysarthria) .
• Ubunzima bokugwinya ukudla .
• Ubuthakathaka bokubamba into oyiphethe. Isibonelo, kungaba nzima ukubopha ihembe, ukuvula ibhodlela, noma izinto ezisezandleni zakho zingase ziwe phansi njalo.
• Ukuqhaqhazela kwemisipha kanye nokuqaqamba .
• Ukwehla kwesisindo ngenxa yezingalo nemilenze emincane.
• Ukungakwazi ukuyeka ukuhleka noma ukukhala ngezikhathi ezingafanele (ithonya le-pseudobulbar) . Lokhu kufana nokuhleka lapho uzizwa udangele, noma uzizwa udangele lapho uzizwa ujabule.

Ezinye izimpawu

Ngaphezu kwalezi zimpawu zokuqala, kungase kuvele ezinye izimpawu:

• Ubunzima bokuphefumula (ukuphelelwa umoya) .
• Ubunzima bokuphefumula ngenkathi ulele embhedeni.
• Ukutheleleka kwesifuba okuvamile.
• Ukuphazamiseka kokulala (ukuphazamiseka kokulala) .
• Ukukhubazeka kokunaka kanye nenkumbulo .
• Ukudideka .
• Ikhanda elibuhlungu ekuseni .
• Ukukhathala .

Yini ebangela i-MND?

Njengoba sesike sakhuluma ngakho ngaphambili, i-MND ibangelwa izinkinga ngama-motor neurons ethu. Lawa maseli ayayeka kancane kancane ukusebenza kahle ngokuhamba kwesikhathi. Kodwa-ke, abacwaningi abakazi kahle ukuthi kungani lokhu kwenzeka .

I-MND iwufuzo

Ezinye izimo ze-MND ziwufuzo, okusho ukuthi zidluliselwa ngamalungu omndeni ngezakhi zofuzo. Kulezi zimo, lesi sifo sibangelwa ushintsho ku-gene eyodwa. Lezi zingazuzwa ngezindlela eziningana eziyinhloko:

• ``I-Autosomal dominant``: Kulesi simo, noma ngabe uthola ikhophi eyodwa yesakhi sofuzo esishintshiwe komunye wabazali abathintekile, kusenengozi yokuthola lesi sifo.
• ``I-Autosomal recessive``: Kulesi simo, ukuze uthuthukise lesi sifo, kufanele uthole amakhophi amabili esakhi sofuzo esishintshiwe kubazali bobabili.
• `Ifa elixhumene ne-X`: Kulokhu, umama uthwala lesi sakhi sofuzo ku-chromosome ye-X bese edlulisela lesi sifo kubantwana bakhe besilisa.

Ezinye izizathu

Izimo ze-MND ezingezona ezefa zingabangelwa yizinto ezahlukahlukene. Lokhu kungafaka phakathi amagciwane, ubuthi, izakhi zofuzo, kanye nezici zemvelo .

Ubani osengozini enkulu yokuthola i-MND?

I-MND ivame ukuthinta abantu abaneminyaka ephakathi kuka-60 no-70. Kodwa-ke, ingathinta izingane kanye nabantu abadala banoma yibuphi ubudala. Uma isihlobo sakho esiseduze sine-MND, noma isimo esifanayo esibizwa ngokuthi i-frontotemporal dementia , usengozini enkulu yokuthola i-MND.

Odokotela bayixilonga kanjani i-MND?

Kungaba nzima ukuxilonga i-MND kusenesikhathi, njengoba kungekho ukuhlolwa okuqondile kwayo. Uma ubuthakathaka bemisipha kancane kancane ngaphandle kobuhlungu noma ukulahlekelwa umuzwa, udokotela wakho angase asole i-MND.

Imibuzo ebuzwe udokotela

Ungazibuza imibuzo efana nale:

• Yiziphi izingxenye zomzimba ezithintekile ?
• Izimpawu zaqala nini?
• Izimpawu zokuqalaIni?
• Ingabe izimpawu sezishintshile ngokuhamba kwesikhathi?

Ukuhlolwa okwenziwe

Kungenziwa izivivinyo eziningana ukusiza ekuxilongeni lesi sifo:

• I-Electromyography (EMG): Lokhu kurekhoda umsebenzi kagesi wemisipha yakho. Lokhu kungasiza ekunqumeni ukuthi inkinga isemisipheni, ezinzwa, noma ekuhlanganeni kwemisipha.
• Izifundo zokuqhuba imizwa: Lokhu kulinganisa ukuthi imizwa idlulisela ngokushesha kangakanani imizwa.
• Ukuskena kwe-magnetic resonance imaging (MRI): I-MRI yobuchopho, futhi ngezinye izikhathi i-MRI yomgogodla, yenziwa ukuze kubhekwe ezinye izinkinga ezingase zibangele izimpawu ezifanayo.

Ukuze kususwe ezinye izimo zezokwelapha, udokotela angase acele ukuhlolwa okwengeziwe:

• Ukuhlolwa kwegazi
• Ukuhlolwa komchamo
• Ukubhoboza umgogodla (ukubhoboza i-lumbar)
• Ukuhlolwa kofuzo

Ngokuhamba kwesikhathi, izimpawu ze-MND ziba sobala kangangokuthi kunezikhathi lapho lesi sifo singatholakala khona ngaphandle kokuhlolwa.

Yiziphi izindlela zokwelapha i-MND?

Akukho ukwelashwa okuqondile noma ukwelashwa kwesifo sezinzwa zomzimba. Kodwa-ke, abacwaningi bayaqhubeka nokutadisha izindlela eziphephile nezisebenzayo zokwelapha lesi simo.

Kuzodingeka usebenzisane nethimba lodokotela ukuze likusize ulawule izimpawu zakho. Ukwelashwa kwe-MND kungafaka:

• Ukwelashwa ngokomzimba: Lokhu kusiza ukugcina amandla emisipha yakho futhi kugcine amalunga akho eguquguquka.
• Uma unenkinga yokugwinya , unganikwa ukudla okunomsoco ngepayipi (ithubhu ye-gastrostomy) efakwe odongeni lwesisu iye esiswini.

Imithi

Eminye imithi isiza abantu abaningi abane-MND ukuthola impumuzo ezimpawini zabo:

• I-Baclofen: Yehlisa ukuqina kwemisipha futhi yehlise ukuqaqamba kwemisipha.
• I-Phenytoin noma i-quinine: Yehlisa ukuqaqamba kwemisipha.
• I-Glycopyrrolate: Yehlisa ukuqunjelwa kwamathe.
• Ama-antidepressant: Usizo ngokucindezeleka.
• Ama-Benzodiazepine: Ubuhlungu obuvela njengoba isifo siqhubeka.

Kubantu abane-ALS, kunemithi emibili engasiza ekwandiseni impilo nokulawula ukwehla kokusebenza: i-riluzole ne-edaravone .

Abantu abaningi bahlanganyela nasezivivinyweni zemitholampilo .

Kufanele ngimbone nini udokotela?

Uma unokuthile okufana nobuthakathaka bemisipha, okungaba uphawu lokuqala lwe-MND, kufanele nakanjani ubone udokotela. Ungase ungabi nayo i-MND, kodwa ukuthola ukuxilongwa okunembile ngokushesha okukhulu kubaluleke kakhulu ukuze uthole ukunakekelwa nokusekelwa okudingayo.

Futhi, uma unesihlobo esiseduze esine-MND noma i-frontotemporal dementia futhi ukhathazekile ukuthi ungase ube nalesi sifo, kuwumqondo omuhle ukubona udokotela. Udokotela wakho angakudlulisela kumeluleki wezakhi zofuzo ukuze axoxe ngengozi yakho.

Yini ongayilindela uma uphila nalesi simo?

Isifo se-motor neurone siyisifo esiqhubekayo, ngakho-ke kubalulekile ukuthola udokotela oqonda isimo sakho futhi ongakusiza ukuthola ukwelashwa okufanele ukuphatha isimo sakho.

Kubalulekile futhi ukuba nohlelo lokusekela . Njengoba isimo sakho siqhubeka, ungase udinge usizo olwengeziwe kwabanye ukuze wenze izinto ezinzima kuwe. Khuluma nabangani nomndeni, noma ubuze udokotela wakho ngezindlela zokuthola ukwesekwa emphakathini wakini. Khumbula, awuwedwa.

Ingakanani iminyaka yokuphila komuntu one-MND?

Ngeshwa, isifo sezinzwa zomzimba singanciphisa kakhulu isikhathi sakho sokuphila . Lezi yizifo eziqhubeka ngokuhamba kwesikhathi futhi ekugcineni ziholele ekufeni. Kodwa-ke, ukuthi kuthatha isikhathi esingakanani ukufinyelela kulelo qophelo kuyahlukahluka kumuntu nomuntu. Abanye abantu baphila iminyaka, ngezinye izikhathi ngisho namashumi eminyaka, ngaphambi kokuba bafe ngenxa yemiphumela yalezi zifo. Udokotela wakho angakunikeza ukuqonda okungcono ngesibikezelo/umbono wesimo sakho.

Into ebaluleke kakhulu - lokho okufanele ukusho

Ukuthola ukuthi unesifo se-motor neurone (MND) kungaba yinto eyesabekayo nekhungathekisayo . Khumbula, akulona iphutha lakho . Awenzanga lutho olubi. Futhi akushintshi ukuthi ungubani - kodwa kungenza impilo ibe ngehlukile kancane. Nakuba ungase ubhekane nezinselele njengoba lesi sifo siqhubeka, kunezindlela zokwelapha ezingakusiza ukuphatha imiphumela. Thembela kubangani bakho nomndeni wakho ukuze uthole ukwesekwa. Uma ubhekene nobunzima bokubhekana naso noma udinga ukwesekwa okwengeziwe, khuluma nodokotela wakho. Bangakusiza ukuthola izinsiza ozidingayo, futhi baqinisekise ukuthi uthola usizo oludingayo ngokuhamba kwesikhathi.

Isifo sezinzwa zomzimba, i-MND, isifo sezinzwa, ubuthakathaka bemisipha, i-ALS, amaseli ezinzwa, ukulahleka kwemisipha