Khawuthelekelele, ngequbuliso uba nobunzima bokuhamba, ulahlekelwe yibhalansi yakho, uze uzive unesizungu kwaye uwe xa umi kwindawo ohleli kuyo. Kuqhelekile ukuba uzive usoyika kakhulu xa ezi zinto ziqala ukwenzeka ngequbuliso. Nangona ngamanye amaxesha singazinaki kakhulu ezi zinto, zinokuba ziimpawu zokuqala zezifo ezingaqhelekanga. Namhlanje siza kuthetha ngesifo esingaqhelekanga, kodwa esibi kakhulu se-neurological. Esi sisifo se-Multiple System Atrophy , isifo esibizwa ngoogqirha kunye nezigulane (MSA) ngamafutshane. Nangona esi sisihloko sinzima kancinci, siza kuthetha ngaso ngokulula, ngendlela onokuyiqonda.
Kalula nje, yintoni le MSA?
I-MSA sisifo esingaqhelekanga semithambo-luvo apho iindawo zengqondo yethu ziba buthathaka kancinci kancinci zize zife. Ekuhambeni kwexesha, izakhono zomzimba kunye nemisebenzi elawulwa zezi ndawo zobuchopho zonakeleyo ziyanyamalala kancinci kancinci. Ukuthetha inyani, le yimeko ebuhlungu kakhulu, kuba esi sifo ekugqibeleni siphela ngokufa.
Ngaphambili, oogqirha babebiza olu luhlu lweempawu ngamagama amathathu. Yayizi:
- Isifo seShy-Drager
- Ukuwohloka kwe-olivopontocerebellar okungacwangciswanga
- Ukuwohloka kweStriatonigral
Kodwa kamva, abaphandi baqonda ukuba ezi zifo zintathu zineempawu ezininzi ezifanayo. Ngoko ke, bazidibanisa zonke baza bazibiza ngokuba yi-"Multiple System Atrophy" (MSA). Igama elithi "Multiple System" lithetha "iinkqubo ezininzi." Oku kuthetha ukuba esi sifo sichaphazela iinkqubo ezahlukeneyo emzimbeni wethu. Iimpawu zixhomekeke ekubeni zeziphi iindawo zobuchopho ezonakeleyo. Yiyo loo nto umntu ngamnye efumana indibaniselwano eyahlukileyo yeempawu.
Kamva, esi sifo sahlulwahlulwa ngakumbi, ngokusekwe kwiimpawu esizibonakalisayo. Ngoko ke, kukho iintlobo ezimbini ze-MSA.
| Uhlobo lwe-MSA | Inkcazo kunye neempawu eziphambili |
|---|---|
| I-MSA-C | U-'C' apha umele 'iCerebellar'. I-cerebellum yinxalenye yobuchopho elawula iintshukumo zethu. Iimpawu eziphambili zolu hlobo kukulahlekelwa yibhalansi (ataxia) . Oku kuthetha ukuba asinakulawula amalungu ethu ngokufanelekileyo, njengaxa sihamba. Ukongeza, kukho iingxaki ngemisebenzi yomzimba ezenzekelayo (ukungasebenzi kakuhle kwe-autonomic) kunye nokuwa rhoqo. |
| I-MSA-P | U-'P' apha umele 'iParkinsonism'. Olu hlobo lwe-MSA-C luneempawu ezifana kakhulu nesifo sikaParkinson. Umzekelo, ukungcangcazela, ukuhamba kancinci, kunye nokuqina. Nangona ezi mpawu zikaParkinson zezona ziphambili kwizigaba zokuqala, ngokuhamba kwexesha, iimpawu ezifana nezezinye i-MSA-C (ukulahlekelwa yibhalansi, iingxaki ngemisebenzi ezenzekelayo) nazo zinokuvela. |
Ngubani osengozini enkulu yokufumana i-MSA?
I-MSA idla ngokuchaphazela abantu abadala, ingakumbi emva kweminyaka engama-30. Iimpawu zibonakala kakhulu phakathi kweminyaka engama-50 nengama-59. Esi sifo sinokuchaphazela nabani na, nokuba ungubani na.
Esi sisifo esingaqhelekanga kakhulu. Ngokwezibalo, kukho amatyala amatsha angama-0.6 ukuya kuma-0.7 kuphela kubantu abayi-100,000 ngonyaka. Oku kuthetha ukuba esi asisifo siqhelekileyo kuluntu lwethu.
Isifo se-MSA siwuchaphazela njani umzimba wethu?
I-MSA ibangela ukuba iiseli zife kwiindawo ezahlukeneyo zobuchopho. Iimpawu zixhomekeke ekubeni zeziphi iindawo zobuchopho ezonakeleyo. Iindawo eziphambili zobuchopho ezichaphazelekileyo zezi:
- I-Basal Ganglia: Ezi zifumaneka kanye embindini wobuchopho. Zifana nehabhu edibanisa iindawo ezahlukeneyo zobuchopho. Kukule nxalenye apho iindawo ezahlukeneyo zobuchopho zidibana khona kwaye zisebenze kunye.
- Ingqondo: Kulapho iinkqubo ezizenzekelayo emzimbeni wethu ezibalulekileyo ukuze siphile zilawulwa khona. Umzekelo, izinto ezinje ngokuphefumla, ukubetha kwentliziyo, kunye noxinzelelo lwegazi. Ezi zinto zenzeka ngokuzenzekelayo ngaphandle kokuba sicinge ngazo, akunjalo? Eli liziko lolawulo.
- I-Cerebellum: Le ikwindawo engasemva kwentloko, kufutshane nesiseko. Le yinxalenye ephambili yengqondo yethu eququzelela iintshukumo zomzimba wethu kwaye igcina ibhalansi yethu. Ukongeza, uphando ngoku lufumanise ukuba le nxalenye ikwabandakanyeka kwiimvakalelo zethu kunye nokwenza izigqibo.
Ngoko ke, xa ezi ndawo zobuchopho zonakele, zonke iinkqubo ezilawulwa zezo ndawo ziqala ukuba mdaka. Umzekelo, xa `(i-brainstem)` yonakele, kubakho iingxaki ezinkulu kwiinkqubo ezizenzekelayo ezifana noxinzelelo lwegazi.
Ziziphi iimpawu eziphambili ze-MSA?
Kwi-MSA, ezinye iimpawu ziqhelekile kuzo zombini ezi ntlobo. Ukongeza, kukho iimpawu ezithile kuhlobo ngalunye. Uphawu oluphambili oluqhelekile kuzo zombini ezi ntlobo kukungasebenzi kakuhle kwe-autonomic . Ngamafutshane, iinkqubo ezenzeka ngokuzenzekelayo emzimbeni wethu azisebenzi kakuhle.
Iimpawu zokungasebenzi kakuhle kwe-Autonomic
- Uxinzelelo lwegazi oluhambelana ne-Orthostatic Hypotension: Olu luphawu lokuqala abantu abaninzi abalubonayo. Xa usukuma ngequbuliso uhleli okanye ulele , uxinzelelo lwakho lwegazi luyehla ngequbuliso . Oku kunokubangela isiyezi, ukubona okufipheleyo, kwanokuphelelwa ngamandla.
- Ukungakwazi ukulawula umchamo kunye nendle: Ukungakwazi ukuzibamba komchamo kunye nokungakwazi ukuzibamba kwindle kunokwenzeka xa ungalindelanga.
- Ukungasebenzi kakuhle ngokwesondo: Iingxaki ezinje ngokungasebenzi kakuhle kwe-erectile, ingakumbi kumadoda, zenzeka ngexesha lokwabelana ngesondo.
- Iingxaki zokulala: Ngokukodwa, imeko ebizwa ngokuba yi-"(REM) sleep behavior disorder" inokubonwa. Kule meko, xa uphupha, ushukumisa umzimba wakho, ukhwaze, kwaye uphakamise amalungu akho ngenxa yamaphupha akho.
- Ukuncipha kokubila (i-Anhidrosis): Amandla omzimba okubila ancipha kakhulu.
- Iimpawu ezifana neengxaki zokubona, umlomo owomileyo, i-sleep apnea, kunye nokuqhina nazo ziqhelekile.
Okubalulekileyo kukuba, ezi ngxaki zokuzimela komzimba zinokwenzeka kwiinyanga, okanye iminyaka, ngaphambi kokuba kuqale iimpawu zokunyakaza (ezifana nokuphazamiseka kwendlela yokuhamba kunye nokungcangcazela). Oku kwenzeka phakathi kwama-20% nama-75% ezigulane ze-MSA.
Iimpawu zengqondo nezeemvakalelo
Malunga nesithathu kwisithathu sabantu abane-MSA bafumana iingxaki ngokukwazi kwabo ukucinga nokugxila. Banobunzima bokulawula iimvakalelo zabo, nto leyo enokubangela iingxaki ezahlukeneyo zempilo yengqondo.
- Ixhala
- Uxinzelelo
- Ukulila okanye ukuhleka ngendlela engafanelekanga
- Uhlaselo loloyiko
- Iingcinga zokuzilimaza okanye ukuzibulala
Iimpawu ezinxulumene nokuhamba
Ezi mpawu ziyahluka ngokuxhomekeke kwiintlobo ezimbini ze-MSA esizixubushileyo ngaphambili.
| Iimpawu ze-MSA-C (Cerebellar) | Iimpawu ze-MSA-P (Parkinsonism) |
|---|---|
| Uphawu oluphambili yi -ataxia , nto leyo ethetha ukungasebenzi kakuhle kwemisipha. | Iimpawu eziphambili yiParkinsonism , oko kuthetha ukuba iimpawu ezifana nesifo sikaParkinson. |
| - Iintshukumo ezingalawulekiyo nezingaqhelekanga zamalungu omzimba. | - Iintshukumo zihamba kancinci kakhulu (bradykinesia) . |
| - Ukungcangcazela kwamalungu omzimba xa uzama ukwenza into ethile (Ukungcangcazela kwesenzo). | - Ukuziva uxinene kwaye uqinile emzimbeni, kunye nesimo somzimba esibonakala ngathi sigobile phambili. |
| - Ukuhamba ngeenyawo ezikude ngendlela engaqhelekanga, njengokuhamba okushukumayo. | - Ukuwa rhoqo xa uhamba. |
| - Ukuqhaqhazela kwamehlo okungalawulekiyo kunye nokunyakaza (nystagmus) . | - Ukungaqondi kakuhle nokungaqondi kakuhle amagama xa uthetha. |
Yintoni ebangela i-MSA?
Isizathu esichanekileyo se-MSA asikaziwa . Nangona kunjalo, izazinzulu zicinga ukuba sibangelwa yiproteni ebizwa ngokuba yi -alpha-synuclein . Le proteni kucingelwa ukuba iqokelelana ngokungaqhelekanga kwiindawo ezahlukeneyo zobuchopho, nto leyo ebangela umonakalo kwiiseli zobuchopho. Okumangalisayo kukuba, le proteni ikwacingelwa ukuba yimbangela yesifo sikaParkinson.
Iiproteni zibalulekile ekusebenzeni kwemizimba yethu. Nangona kunjalo, xa ezi proteni zihlangana kwiindawo ezingalunganga, ngendlela engalunganga, zingonakalisa iiseli. Yiloo nto eyenzekayo kwi-MSA.
Uphando lusaqhubeka malunga nokuba kutheni le protein ye-alpha-synuclein iqokelelana engqondweni. Kucingelwa ukuba isenokuba ibangelwa kukuguquka kwezakhi zofuzo. Ngokukodwa, kukho ubungqina bokuba uhlobo lwe-MSA-C lunokudluliselwa kwisizukulwana ukuya kwesinye ukuya kwinqanaba elithile. Nangona kunjalo, akukho khonkco lofuzo elifunyenweyo lohlobo lwe-MSA-P.
Kubalulekile: I-MSA ayisosifo esosulelayo. Ayidluliselwa ukusuka komnye umntu ukuya komnye nangayiphi na indlela.
Ifunyaniswa njani i-MSA?
Le yeyona nxalenye inzima kakhulu yesi sifo. Yindlela ekuphela kwayo yokuqinisekisa i-MSA ngokuqiniseka okupheleleyo.kukuhlola izicwili zobuchopho emva kokuba umntu eswelekile. Isizathu soku kukuba okwangoku akukho teknoloji yokubona ukuba iproteni ye-alpha-synuclein ibekwe engqondweni yomntu ophilayo.
Nangona kunjalo, ngelixa isigulana sisaphila, oogqirha banokukrokrela ukuba sisifo. Basebenzisa izinto ezifana neempawu, imbali yezonyango yesigulana, imbali yosapho, kunye nempendulo kunyango oluthile. Rhoqo, oogqirha banokuqala baxilonge isifo sikaParkinson. Nangona kunjalo, njengoko ezinye iimpawu zikhula ngokuhamba kwexesha, okanye xa amayeza kaParkinson eyeka ukusebenza, uxilongo lunokufuneka lutshintshwe lube yi-MSA.
Umahluko ophambili phakathi kwe-MSA kunye nesifo sikaParkinson
| Uphawu | Ukuwohloka Kweenkqubo Ezininzi (i-MSA) | Isifo sikaParkinson |
|---|---|---|
| Isantya sokusasazeka kwesi sifo | Iimpawu ziya zisiba mbi ngokukhawuleza . | Esi sifo siqhubeka kancinci kancinci . |
| Ubuthathaka obuzenzekelayo | Iingxaki ezinkulu (umz., iingxaki zoxinzelelo lwegazi) zihlala zibonakala kunyaka wokuqala wokugula. | Kungathatha iminyaka ukuba ezi mpawu zibonakale. |
| Uloyiko | Ukungcangcazela kuncinci, mhlawumbi akukho kwaphela . | Ukungcangcazela okwenzeka xa uphumle luphawu olukhulu . |
| Impendulo kwiLevodopa | Bambalwa kakhulu abasabelayo kweli yeza.Okanye akunjalo konke konke. | Iphendula kakuhle kakhulu kweli yeza. |
Uvavanyo lokuxilonga
Zimbalwa kakhulu iimvavanyo ezinokuxilonga ngqo i-MSA. Ngokuqhelekileyo, kwenziwa uthotho lweemvavanyo ukuze kuthintelwe ezinye iimeko kunye nokuqokelela ubungqina bokuxhasa ukurhanelwa kokuba i-MSA isenokuba yimbangela.
- Iskeni ye-MRI (Magnetic Resonance Imaging): Oku ngamanye amaxesha kunokubonisa umonakalo kwiindawo ezithile zobuchopho. Kwi-MSA-C, ngokukodwa, ipatheni ebizwa ngokuba yi-"hot cross bun" inokubonwa kwinxalenye yengqondo. Ifana ne-hot cross bun. Nangona kunjalo, olu phawu lunokubonwa nakwezinye izifo, ngoko ke aluyonto yokuxilonga i-MSA.
- Uvavanyo lwemfuza: Oku kunokujonga utshintsho lwemfuza olunxulumene neproteni ye-alpha-synuclein.
- I-biopsy yolusu: Uphando olutsha lubonisa ukuba olu vavanyo lunokunceda ekuqinisekiseni ukuba i-alpha-synuclein ikhona na kwizicubu zemithambo-luvo yolusu. Nangona kunjalo, oku kusesekwinqanaba lophando.
Ugqirha wakho uza kukuchazela ukuba zeziphi iimvavanyo acinga ukuba ziyimfuneko, ngokusekelwe kwimeko yakho, kunye noko ukulindeleyo kuzo.
Ngaba kukho unyango lwe-MSA?
Ngelishwa, okwangoku akukho nyango lwe-MSA . Ke ngoko, injongo ephambili yonyango kukulawula iimpawu nokugcina umgangatho wobomi olungileyo kangangoko kunokwenzeka.
Unyango olunikwayo luxhomekeke kwiimpawu zesigulana kunye nobunzima bazo. Umzekelo, amayeza oxinzelelo lwegazi anikwa unyango lwe-orthostatic hypotension, amayeza okukhulula imisipha yokuqina, kunye namanye amayeza okulawula umchamo. Kwakhona, izinto ezifana ne-physiotherapy kunye nonyango lokuthetha zibaluleke kakhulu.
Ukuba wena okanye umntu omaziyo unale meko, musa ukuzama ukuzinyanga okanye ukulawula iimpawu. Soloko ubona ugqirha ofanelekileyo kwaye ulandele icebiso lakhe.
Luhlobo luni lwekamva umntu onesifo anokulilindela?
I-MSA sisifo esiqhubekekayo. Malunga nesiqingatha sabo bachaphazelekileyo baya kufuna uncedo lokuhamba (njengomsimelelo okanye isixhobo sokuhamba) kwiminyaka emi-5 emva kokuqala kweempawu. Malunga nama-60% baya kufuna isihlalo sabakhubazekileyo kwiminyaka emi-5. Kwiminyaka emi-6 ukuya kweyesi-8, uninzi lwezigulane luba sebhedini.
Njengoko isifo siqhubeka, kufuneka iinkqubo ezahlukeneyo zonyango ukuze kugcinwe imisebenzi yomzimba.
- I-Tracheostomy yinkqubo apho kwenziwa umngxuma omncinci emqaleni kwaye kufakwa ityhubhu ukunceda ubunzima bokuphefumla.
- Ukondla ngetyhubhu kukunika ukutya ngetyhubhu xa isigulane singakwazi ukuginya.
- Utyando lokulawula umchamo kunye nendle.
Ngokwesiqhelo, umyinge wobomi bomguli one-MSA ukususela ngexesha lokufunyaniswa kwesi sifo uphakathi kweminyaka emi-6 ukuya kweli-10. Kwezinye iimeko ezingezizo ezinzulu kangako, kunokwenzeka ukuba umntu aphile iminyaka eli-15. Nangona kunjalo, ukuba isifo sibi kakhulu, ubomi obulindelekileyo bunokuba bufutshane kakhulu.
Ukufa kubangelwa ikakhulu ziingxaki zesifo. Umzekelo:
- Ukukrala kwemiphunga.
- I-Sepsis (ityhefu egazini) ngenxa yosulelo lomchamo.
- Ukufa ngequbuliso okubangelwa ziingxaki zokulawula ukuphefumla kwengqondo ngexesha lokulala.
Sibanyamekela njani abantu esibathandayo?
Njengoko iimpawu zomntu one-MSA zisiba mandundu ngokuhamba kwexesha, ekugqibeleni usenokungakwazi ukuphila ngokuzimela. Basenokulahlekelwa nakukwazi ukucinga, ukuthetha, nokuzenzela izigqibo.
Kungoko ke, kubaluleke kakhulu ukuthetha ngekamva nabantu obathandayo kwangethuba kwesi sifo, ngelixa isigulana sisesezingqondweni. Ukuba ukwimeko apho ungakwazi ukwenza izigqibo, kulula kakhulu kuye wonke umntu ukuba ungaxoxa kwangaphambili ngezigqibo ekufuneka zenziwe malunga nonyango lwakho kwaye ulungiselele amaxwebhu asemthethweni.
Ufanele uye nini kugqirha?
Uninzi lweempawu zokuqala ze-MSA zizinto ekufuneka uzixoxe nogqirha.
- Ukuziphatha okubi ngokwesondo.
- Ukudinwa rhoqo kunye nokulahlekelwa zingqondo xa umi ngenyawo.
- Iingxaki zokulala, ingakumbi i-sleep apnea.
Ukuba ugqirha wakho ukuxelele ukuba unesifo sokuhamba esifana nesikaParkinson, kubalulekile ukuthetha ngazo naziphi na iinguqu kwiimpawu zakho. Ingakumbi ukuba iyeza (levodopa) leParkinson's alisebenzi, loo nto ibonisa ukuba usenokuba une-MSA.
I-MSA sisifo esibi nesibulalayo. Liyinyaniso elokuba akukho nyango lwaso. Nangona kunjalo, iimpawu ezininzi zinokunyangwa. Ngolo nyango, umgangatho wobomi besigulana unokugcinwa kwinqanaba elifanelekileyo kangangeminyaka emininzi. Elo xesha lithuba elixabisekileyo lokuchitha ubomi ngolonwabo nabantu ababathandayo kwaye bonwabele ubomi ngokupheleleyo.
Umyalezo Wokuya Ekhaya
- I-MSA sisifo semithambo-luvo esingaqhelekanga nesibulalayo esitshabalalisa kancinci kancinci iindawo zobuchopho.
- Kukho iintlobo ezimbini eziphambili: i-MSA-C (ukulahleka kwebhalansi kuphambili) kunye ne-MSA-P (iimpawu zeParkinson ziphambili).
- Ukungasebenzi kakuhle kwe-autonomic, okufana nokwehla koxinzelelo lwegazi xa umile kunye nokulahlekelwa lulawulo lomchamo, kubonakala kwasekuqaleni.
- I-MSA ikhula ngokukhawuleza kunesifo sikaParkinson kwaye ayiphenduli kakhulu kumayeza kaParkinson.
- Nangona kungekho nyango lwesifo, kukho unyango lokulawula iimpawu nokuphucula umgangatho wobomi.
- Kubaluleke kakhulu ukuqaphela iimpawu kwangethuba, ukufuna ingcebiso kagqirha, kunye nokuceba ikamva.
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