Ngaba uvakalelwa kukuba amalungu akho aphelelwa ngamandla kancinci kancinci? Oku kusenokuba sisifo seMotor Neuron Disease (MND)!

Ngaba wakha waziva ngathi imilenze yakho iyaphelelwa ngamandla xa unyuka izinyuko, amazwi akho ayawa xa uthetha, okanye into ethile iwa ngequbuliso esandleni sakho? Usenokucinga ukuba ezi zizinto eziqhelekileyo, kodwa ukuba ezi mpawu ziyaqhubeka, licebo elihle ukuba ukhathazeke kancinci. Kuba ezi zinokuba ziimpawu zokuqala zesifo esibizwa ngokuba yiMotor Neuron Disease (MND) . Ngoko ke, masithethe ngale nto ngeenkcukacha ezithe vetshe namhlanje.

Yintoni isifo se-motor neurone (MND)?

Ngamafutshane, isifo se-motor neurone (MND) liqela lezifo ezichaphazela inkqubo yethu yemithambo-luvo. Sibangela ukuba ii-motor neurons zethu zife kancinci kancinci. Ngoku usenokuba uzibuza ukuba zeziphi ezi motor neurons. Ezi ziiseli zemithambo-luvo ezilawula izihlunu zethu. Ezi motor neurons zibalulekile kuyo yonke imisebenzi yethu, ukusuka ekuphefumleni, ekuthetheni, ekuginyeni nasekuhambeni.

Khawucinge nje, imiyalezo esuka kwingqondo yethu (esiyibiza ngokuba zii-upper motor neurons ) ifika kwi-spinal cord yethu. Ukusuka apho, loo miyalezo idlula kwii-low motor neurons ukuya kwimisipha emzimbeni wethu. Ezi neurons ziphezulu zezona zixelela ii-low motor neurons, "Kulungile, ngoku hambisa esi sihlunu ngolu hlobo."

Ngoku, kwenzeka ntoni xa le mithambo-luvo isezantsi ye-motor ingakwazi ukuthumela imiyalezo kwimisipha? Imisipha iqala ukuba buthathaka kwaye inciphe kancinci kancinci (i-muscle atrophy) . Kwakhona, xa imithambo-luvo ephezulu ye-motor ingakwazi ukuthumela imiqondiso kwimithambo-luvo esezantsi ye-motor, imisipha iyaqina kwaye isebenze kakhulu ( i-hyperreflexia ). Oku kwenza kube nzima kuthi ukwenza iintshukumo zokuzithandela, kwaye zenzeka kancinci kakhulu. Ekuhambeni kwexesha, sinokulahlekelwa nakukwazi ukuhamba nokulawula ezinye iintshukumo.

Into ebalulekileyo kukuba okwangoku akukho nyango lwesifo se-motor neurone. Ezi zizifo eziqhubekekayo eziya ziba mandundu ngokuhamba kwexesha. Nangona kunjalo, kukho unyango olunokunceda ukunciphisa impembelelo yale meko.

Zeziphi iintlobo ze-MND ezikhoyo?

Oogqirha bahlula i-MND ngokwendlela echaphazela ngayo imithambo-luvo ephezulu ye-motor, imithambo-luvo esezantsi ye-motor, okanye zombini. Kukho iintlobo ezininzi eziphambili ze-MND:

`I-Amyotrophic lateral sclerosis (ALS)`

Olu lolona hlobo luqhelekileyo lwe-MND. Abanye abantu balubiza ngokuba sisifo sikaLou Gehrig . I-ALS ichaphazela zombini imithambo-luvo ephezulu nesezantsi. Oku kunokubangela ukulahlekelwa ngokukhawuleza kolawulo lwemisipha, ekugqibeleni kukhokelela ekukhubazekeni . Oogqirha abaninzi ngamanye amaxesha basebenzisa amagama athi ALS kunye nesifo se-motor neurone ngokutshintshana.

`I-Progressive bulbar palsy (PBP)`

Yile nto isihlaselayo.Imithambo-luvo esezantsi yentshukumo edibanisa ne-brainstem (ummandla we-bulbar). I-brainstem yethu ilawula izihlunu ezifunekayo kwizinto ezifana nokuthetha, ukuhlafuna, nokuginya. Elinye igama le-PBP yi-"progressive bulbar atrophy".

`Isifo sokuqaqamba kwemisipha esisecaleni (i-PLS)`

Oku kuchaphazela kuphela imithambo-luvo ephezulu yentshukumo. Ngokuqhelekileyo, esi sifo siqala sichaphazele imilenze. Emva koko sichaphazela iingalo, izandla, kunye nomzimba. Ekugqibeleni, sinokuchaphazela izihlunu ezisetyenziselwa ukuthetha, ukuginya, kunye nokuhlafuna ukutya.

`Ukuwohloka kwemisipha okuqhubekekayo (PMA)`

Ichaphazela kuphela imithambo-luvo esezantsi yentshukumo. Le meko ixhaphake kakhulu kumadoda, kwaye idla ngokukhula ebancinci kunezinye ii-MNDs eziqala kubantu abadala. Iimpawu zokuqala bubuthathaka kwiingalo, ezithi emva koko zisasazeke ziye emzimbeni ongezantsi. Ingachaphazela nomzimba kunye nokuphefumla.

Ukuwohloka kwemisipha yomgogodla (SMA)

Le yimeko yelifa echaphazela imithambo-luvo esezantsi yentshukumo. Ikwathathwa njengeyona nto iphambili kwimfuza ebangela ukufa kweentsana . Utshintsho kwi-gene ye-`SMN1` lubangela ukulahleka kweproteni ye-`SMN` . Oku kutshabalalisa kancinci kancinci imithambo-luvo esezantsi yentshukumo, okubangela ukuphelelwa ngamandla kwemisipha kunye nobuthathaka, kwaye ekugqibeleni kufe.

Isifo sikaKennedy

Inxulunyaniswa nejini ekwi-chromosome X emadodeni. Ibangelwa kukuguquka kwejini ye -androgen receptor . Ekuhambeni kwexesha, ubuthathaka buyakhula ezingalweni nasemilenzeni, ngokuqhelekileyo kuqala kwiindawo ze-pelvic okanye zamagxa. Intlungu kunye nokungaziva mnandi ezingalweni nasemilenzeni nako kunokubakho.

I-Post-polio syndrome (PPS)

Iyenzeka kubantu abaye baphila kwi-polio. Inokwenzeka emva kweminyaka engamashumi amane emva kokugula kwabo kokuqala. I-polio inokubangela umonakalo omkhulu kwimithambo-luvo yemithambo-luvo. Iimpawu ze-PPS ziquka ubuthathaka bemisipha namalungu, ukudinwa, iintlungu ezikhula ngokuhamba kwexesha, ukuxhuzula nokuphelelwa ngamandla kwemisipha, kunye nokungakwazi ukunyamezela umkhuhlane.

Zithini iimpawu ze-MND?

Iimpawu ze-MND aziveli ngequbuliso, kodwa zibonakala kancinci kancinci. Kwinqanaba lokuqala, zisenokungabonakali kangako.

Iimpawu zokuqala

• Ubunzima bokunyuka izinyuko okanye ukukhubeka rhoqo ngenxa yobuthathaka emilenzeni okanye emaqatheni.
• Intetho engacacanga (dysarthria) .
• Ubunzima bokuginya ukutya .
• Ukuba buthathaka kwendlela yokubamba into oyiphetheyo. Umzekelo, kunokuba nzima ukuqhobosha ihempe, ukuvula ibhotile, okanye izinto ezisezandleni zakho zingawela phantsi rhoqo.
• Ukuxhuzula kwemisipha kunye nokuqaqamba .
• Ukuncipha kobunzima ngenxa yeengalo nemilenze ebhityileyo.
• Ukungakwazi ukuyeka ukuhleka okanye ukukhala ngamaxesha angafanelekanga (impembelelo ye-pseudobulbar) . Oku kufana nokuhleka xa uziva ulusizi, okanye uzive ulusizi xa uziva wonwabile.

Ezinye iimpawu

Ukongeza kwezi mpawu zokuqala, ezinye iimpawu zinokuvela:

• Ubunzima bokuphefumla (ukungakwazi ukuphefumla) .
• Ubunzima bokuphefumla xa ulele ebhedini.
• Usulelo oluqhelekileyo esifubeni .
• Iingxaki zokulala (ukuphazamiseka kokulala) .
• Ukuphazamiseka kwengqwalasela kunye nenkumbulo .
• Ukudideka .
• Intloko ebuhlungu yasekuseni .
• Ukudinwa .

Yintoni ebangela i-MND?

Njengoko besikhe sathetha ngaphambili, i-MND ibangelwa ziingxaki zee-motor neurons zethu. Ezi seli ziyeka ukusebenza kakuhle kancinci kancinci ngokuhamba kwexesha. Nangona kunjalo, abaphandi abakayazi ukuba kutheni oku kusenzeka .

I-MND ifuzwa njengelifa

Ezinye izifo ze-MND zidluliselwa kwilifa, oko kuthetha ukuba zidluliselwa kumalungu osapho ngee-genes. Kwezi meko, esi sifo sibangelwa kukutshintsha kwe-gene enye. Ezi zinokudluliselwa kwilifa ngeendlela ezininzi eziphambili:

• ``Autosomal dominant``: Kwimeko enjalo, nokuba ufumana ikopi enye yejini eguquliweyo komnye wabazali abachaphazelekayo, kusekho umngcipheko wokuba nesi sifo.
• ``I-Autosomal recessive``: Kwimeko enjalo, ukuze uphuhlise esi sifo, kufuneka ufumane iikopi ezimbini zejini eguquliweyo ezivela kubazali bobabini.
• `Ilifa elidityaniswe ne-X`: Kule meko, umama uthwala esi jini kwi-chromosome ye-X aze adlulisele esi sifo kubantwana bakhe abangamakhwenkwe.

Ezinye izizathu

Iimeko ze-MND ezingezizo ezelifa zinokubangelwa zizinto ezahlukeneyo. Ezi zinokubandakanya iintsholongwane, iityhefu, i-genetics, kunye nezinto ezisingqongileyo .

Ngubani osengozini enkulu yokuhlaselwa yi-MND?

I-MND idla ngokuchaphazela abantu abaphakathi kweminyaka engama-60 ukuya kwengama-70. Nangona kunjalo, inokuchaphazela abantwana kunye nabantu abadala bayo nayiphi na iminyaka. Ukuba isalamane sakho esisondeleyo sine-MND, okanye imeko efanayo ebizwa ngokuba yi-frontotemporal dementia , usemngciphekweni omkhulu wokuba ne-MND.

Oogqirha bayixilonga njani i-MND?

Kunokuba nzima ukuyifumanisa kwasekuqaleni i-MND, kuba akukho vavanyo lucacileyo lwayo. Ukuba ubuthathaka bemisipha kancinci kancinci ngaphandle kwentlungu okanye ukuphelelwa zimvakalelo, ugqirha wakho unokurhanela i-MND.

Imibuzo ebuzwe ngugqirha

Ungazibuza imibuzo efana nale:

• Zeziphi iindawo zomzimba ezichaphazelekileyo ?
• Ziqale nini ezi mpawu?
• Iimpawu zokuqalaIntoni?
• Ngaba iimpawu zitshintshile ekuhambeni kwexesha?

Uvavanyo olwenziweyo

Kunokwenziwa iimvavanyo ezininzi ukunceda ukufumanisa isifo:

• I-Electromyography (EMG): Le irekhoda umsebenzi wombane wezihlunu zakho. Oku kunokunceda ekuqinisekiseni ukuba ingxaki ikwizihlunu, kwiimithambo-luvo, okanye kwi-neuromuscular junction.
• Izifundo zokuqhuba imithambo-luvo: Oku kulinganisa indlela imithambo-luvo ethumela ngayo imiqondiso ngokukhawuleza.
• Iskeni seMagnetic resonance imaging (MRI): I-MRI yengqondo, kwaye ngamanye amaxesha i-MRI yomqolo, yenziwa ukujonga ezinye iingxaki ezinokubangela iimpawu ezifanayo.

Ukuze kuthintelwe ezinye iimeko zonyango, ugqirha angacela olunye uvavanyo:

• Uvavanyo lwegazi
• Uvavanyo lomchamo
• Impompi yomqolo (ukubhoboza i-lumbar)
• Uvavanyo lwe-genetic

Ekuhambeni kwexesha, iimpawu ze-MND ziyacaca kangangokuba kukho amaxesha apho isifo sinokufunyaniswa ngaphandle kovavanyo.

Ziziphi iindlela zonyango ze-MND?

Akukho nyango okanye unyango oluthile lwesifo se-motor neurone. Nangona kunjalo, abaphandi bayaqhubeka nokufunda iindlela ezikhuselekileyo nezisebenzayo zokunyanga esi sifo.

Kuya kufuneka usebenzisane neqela loogqirha ukuze bakuncede ukulawula iimpawu zakho. Unyango lwe-MND lungabandakanya:

• Unyango lomzimba: Oku kunceda ukugcina izihlunu zakho ziqinile kwaye kugcina amalungu akho eguquguquka.
• Ukuba unengxaki yokuginya , unganikwa ukutya okunesondlo ngetyhubhu (ityhubhu ye-gastrostomy) efakwe ngodonga lwesisu iye esiswini.

Amayeza

Amanye amayeza anceda abantu abaninzi abane-MND ukuba bafumane isiqabu kwiimpawu zabo:

• I-Baclofen: Yehlisa ukuqina kwemisipha kwaye yehlisa ukuqaqamba kwemisipha.
• I-Phenytoin okanye i-quinine: Yehlisa ukuqaqamba kwemisipha.
• I-Glycopyrrolate: Yehlisa ukuvuza kwamathe.
• Amayeza okudakumba: Ukunceda ekuxinezelekeni.
• IiBenzodiazepines: Zeentlungu ezenzeka njengoko isifo siqhubeka.

Kubantu abane-ALS, kukho amayeza amabini anokunceda ukwandisa ubomi kunye nokulawula ukwehla kokusebenza: i-riluzole kunye ne-edaravone .

Abantu abaninzi bathatha inxaxheba kwiimvavanyo zeklinikhi .

Ndifanele ndimbone nini ugqirha?

Ukuba unento efana nobuthathaka bemisipha, enokuba luphawu lokuqala lwe-MND, ngokuqinisekileyo kuya kufuneka ubone ugqirha. Usenokungabi nayo i-MND, kodwa ukufumana uxilongo oluchanekileyo ngokukhawuleza kubaluleke kakhulu ukuze ufumane unyango kunye nenkxaso oyifunayo.

Kwakhona, ukuba unesalamane esisondeleyo esine-MND okanye i-frontotemporal dementia kwaye ukhathazekile ukuba nawe unokuba nesi sifo, kulungile ukubona ugqirha. Ugqirha wakho angakuthumela kumcebisi wezemfuza ukuze axoxe ngomngcipheko wakho.

Yintoni onokuyilindela xa uphila nale meko?

Isifo se-motor neurone sisifo esiqhubekekayo, ngoko ke kubalulekile ukufumana ugqirha oyiqondayo imeko yakho kwaye onokukunceda ufumane unyango olufanelekileyo lokulawula imeko yakho.

Kubalulekile nokuba nenkqubo yenkxaso . Njengoko imeko yakho iqhubeka, unokufuna uncedo olungakumbi kwabanye ukuze wenze izinto ezinzima kuwe. Thetha nabahlobo kunye nosapho, okanye ubuze ugqirha wakho ngeendlela zokufumana inkxaso kuluntu lwakho. Khumbula, awuwedwa.

Ingakanani iminyaka yobomi bomntu one-MND?

Ngelishwa, isifo se-motor neurone sinokunciphisa kakhulu ixesha lokuphila kwakho . Ezi zizifo eziqhubeka ngokuhamba kwexesha kwaye ekugqibeleni zikhokelela ekufeni. Nangona kunjalo, ixesha elithathayo ukufikelela kuloo nqanaba liyahluka kumntu nomntu. Abanye abantu baphila iminyaka, ngamanye amaxesha amashumi eminyaka, ngaphambi kokuba bafe ngenxa yeziphumo zezi zifo. Ugqirha wakho angakunika ukuqonda okungcono ngemeko yakho yokuqikelela/indlela ocinga ngayo.

Eyona nto ibalulekileyo - into omawuyithethe

Ukufumanisa ukuba unesifo se-motor neurone disease (MND) kunokuba ngamava oyikisayo nanzima . Khumbula, asikuko ityala lakho . Awenzanga nto imbi. Kwaye ayitshintshi ukuba ungubani - kodwa inokwenza ubomi bube nomahluko kancinci. Nangona unokujongana nemingeni njengoko isifo siqhubeka, kukho unyango olunokukunceda ulawule iziphumo. Thembela kubahlobo bakho kunye nosapho lwakho ukuze bakuxhase. Ukuba unengxaki yokumelana okanye ufuna inkxaso engaphezulu, thetha nogqirha wakho. Banokukunceda ufumane izixhobo ozifunayo, kwaye baqinisekise ukuba ufumana uncedo oludingayo ngokuhamba kwexesha.

Isifo se - Motor neurone, i-MND, isifo se-neurological, ubuthathaka bemisipha, i-ALS, iiseli zemithambo-luvo, ukuphelelwa ngamandla kwemisipha