Ngaba uziva izinto ezingaqhelekanga emzimbeni wakho? Mhlawumbi kubalulekile ukwazi ngeMultiple System Atrophy (MSA)!

Ngaba ukhe uzive ngathi izinto emzimbeni wakho ziyatshintsha ngeendlela ongenakukwazi ukuzilawula? Ngamanye amaxesha kuba ngathi ulahlekelwa yibhalansi yakho xa uhamba, okanye uzive unesizungu xa usukuma ngequbuliso. Ezi zizinto ngamanye amaxesha esingazinaki kangako, kodwa zinokuba ziimpawu zezifo ezithile zemithambo-luvo ezingaqhelekanga. Namhlanje siza kuthetha ngenye imeko enjalo eyinkimbinkimbi kancinci, kodwa kubaluleke kakhulu ukuba uyiqaphele. Yiyo loo nto i-Multiple System Atrophy (MSA) .

Yintoni kanye kanye iMultiple System Atrophy (MSA)?

Ngamafutshane, iMultiple System Atrophy (MSA) sisifo esingaqhelekanga semithambo-luvo apho iindawo zengqondo yethu zibuthathaka kancinci kancinci, oko kukuthi, ziwohloka. Ngokuhamba kwexesha, imisebenzi kunye nobuchule bomzimba wethu obulawulwa zezi ndawo zengqondo buqala ukuwohloka. Esi sisifo esibi kakhulu, kuba kunzima ukuchacha ngokupheleleyo kuso.

Ngaphambili, i-MSA yayibizwa ngamagama amaninzi ahlukeneyo. Usenokuba ukhe weva ngamagama anje nge-`Shy-Drager syndrome`, `Sporadic olivopontocerebellar atrophy`, `Striatonigral degeneration`. Kusemva koko apho oogqirha baqonda ukuba zonke ezi meko zineempawu ezifanayo. Yiyo loo nto bazidibanisa zonke baza bazinika igama elitsha, `Multiple System Atrophy` (MSA). Iimpawu ziyahluka ngokuxhomekeke ekubeni leliphi icala lobuchopho elonakeleyo. Yiyo loo nto umntu ngamnye efumana indibaniselwano eyahlukileyo yeempawu.

Zimbini iintlobo ze-MSA, akunjalo? Zithini ezo?

Ewe, ukuze bachaze ngokucacileyo imeko ye-MSA, oogqirha bayihlukanise yaba ziintlobo ezimbini eziphambili, kuxhomekeke kwiimpawu ezibonakalayo:

1. I-MSA-C : U-'C' apha umele i-'cerebellar'. Olu hlobo luchaphazela kakhulu amandla akho okulungelelanisa iintshukumo zakho. Oko kukuthi, ulahlekelwa yibhalansi yakho , nto leyo oogqirha abayibiza ngokuba yi-'ataxia'. Olu luphawu oluphambili lolu hlobo. Kwangaxeshanye, unokubona iingxaki kwiinkqubo zokulawula ngokuzenzekelayo zomzimba ('ukungasebenzi kakuhle kwe-autonomic') kunye nokuwa rhoqo.

2. I-MSA-P : Igama elithi 'P' apha limele 'i-parkinsonism'. Kolu hlobo, iimpawu ezifana nesifo sikaParkinson ziyabonwa. Nangona ezi mpawu zikaParkinson zibonakala ngakumbi kwizigaba zokuqala, ngokuhamba kwexesha, iingxaki zenkqubo yemithambo-luvo ezimeleyo kunye neempawu ze-'ataxia' nazo zinokuvela.

Ngubani ochaphazeleka kakhulu yile meko ye-MSA? Ixhaphake kangakanani?

I-MSA idla ngokuchaphazela abantu abadala abaneminyaka engaphezu kwama-30. Iimpawu zibonakala kakhulu phakathi kweminyaka engama-50 nama-59. Ingachaphazela nabani na, nokuba ungubani na.

Kodwa i-MSA yimeko engaqhelekanga kakhulu . Iingcali zithi kukho amatyala amatsha angama-0.6 okanye angama-0.7 kuphela kubantu abayi-100,000 ngonyaka. Inani lilonke lamatyala liqikelelwa ukuba liphakathi kwe-3.4 kunye ne-4.9 kubantu abayi-100,000. Ngoko ungabona ukuba oku kunqabile kangakanani.

Kwenzeka ntoni emzimbeni xa une-MSA? Zeziphi iindawo zobuchopho ezichaphazelekileyo?

I-MSA sisifo esiqhubekayo esichaphazela iindawo ezahlukeneyo zobuchopho. Iimpawu zixhomekeke kwinxalenye yobuchopho echaphazelekileyo. Iindawo eziphambili zobuchopho ezichaphazelekileyo yi-MSA zezi:

• I-Basal ganglia : Ezi zifumaneka kanye embindini wobuchopho. Zidibanisa iindawo ezahlukeneyo zobuchopho kwaye zincede ukuba zisebenze kunye. Zifana negumbi lolawulo eliphambili lobuchopho .
• Ingqondo : Yile nto ilawula iinkqubo ezininzi ezizenzekelayo emzimbeni wethu. Oko kukuthi, izinto ezenzeka ngokuzenzekelayo ngaphandle kokuba sicinge ngazo, umzekelo, ukuphefumla, ukubetha kwentliziyo, kunye noxinzelelo lwegazi. Ezi zibalulekile ukuze siphile.
• I-Cerebellum : Le ikwindawo engasemva kwentloko, ezantsi. Inoxanduva olukhulu lokulawula intshukumo kunye nokugcina ibhalansi. Ikwasebenza nezinye iindawo zengqondo. Abaphandi basafunda ngokusebenza kwayo ngokupheleleyo, kodwa ubungqina obuthile bubonisa ukuba ikwabandakanyeka kwiimvakalelo zethu nakwizigqibo esizenzayo.

Ngoko ke, xa ezi ndawo zobuchopho zonakele, imisebenzi elawulwa zezo ndawo ayinakwenziwa ngokufanelekileyo. Umzekelo, ukuba i-'Brainstem' yonakele, iingxaki zezinto ezizenzekelayo ezifana noxinzelelo lwegazi zinokwenzeka.

Zithini iimpawu ze-MSA? Sizibona njani?

Ezinye iimpawu ze-MSA ziqhelekile kuzo zombini iintlobo, kwaye ezinye zichaphazela uhlobo ngalunye. Kodwa into enye eqhelekileyo kuzo zombini ezi ntlobo kukungasebenzi kakuhle kwe-autonomic. Ngamafutshane, iinkqubo zokulawula umzimba wakho ngokuzenzekelayo ziyaphela.

Iimpawu ezibangelwa ziingxaki zenkqubo ye-autonomic nervous:

• Uxinzelelo lwegazi oluphantsi : Oku kwenzeka xa uxinzelelo lwakho lwegazi luyehla ngequbuliso, okubangela isiyezi , xa utshintsha indawo, umzekelo, xa uphakama ngequbuliso uhleli phantsi.
• Ukungakwazi ukulawula umchamo (`ukungakwazi ukuzibamba`) kunye nokungakwazi ukulawula indle (`ukungakwazi ukuzibamba`).
• Ukungasebenzi kakuhle ngokwesondo, ingakumbi kumadoda, kubunzima bokufumana ukuqina (ukungasebenzi kakuhle kwe-erectile).
• Ingxaki yokuziphatha kokulala ngokukhawuleza (REM) : Njengesifo sikaParkinson, xa uphupha, wenza izinto ozenzileyo emaphupheni akho. Usenokukhwaza okanye uphakamise iingalo zakho.
• Ukuncipha kokubila (i-anhidrosis).
• Iingxaki zokubona.
• Umlomo owomileyo.
• Ukuphelelwa ngamandla xa ulele.
• Ukucotha kokugaya ukutya kunye nokuqhina kwesisu.

Cinga ukuba uninzi lwezi mpawu zokuzimela komzimba zinokuvela iinyanga, kwaneminyaka, ngaphambi kokuba kuvele iimpawu zokunyakaza komzimba. Oku kwenzeka phakathi kwama-20% nama-75% ezigulane ze-MSA.

Iimpawu zengqondo nezeemvakalelo

Malunga nesithathu sabantu abane-MSA bafumana iingxaki ngokukwazi kwabo ukucinga nokugxila. Banobunzima nokulawula iimvakalelo zabo. Oku kunokukhokelela kwiingxaki zempilo yengqondo ezifana nezi:

• Ixhala.
• Uxinzelelo.
• Ukungazinzi ngokweemvakalelo: Oko kukuthi, ukulila nokuhleka ngendlela engafanelekanga.
• Uhlaselo loloyiko.
• Iingcinga zokuzilimaza okanye ukuzibulala.

Iimpawu ezinxulumene nentshukumo

Iimpawu ze-MSA-C (uhlobo lwe-cerebellar):

Olu hlobo lubandakanya ikakhulu i-'ataxia' (ukulahlekelwa yibhalansi). I-'cerebellum' yinxalenye yengqondo edlala indima enkulu ekulawuleni iintshukumo zemisipha. Ngoko ke, xa olo lungelelwaniso lulahlekile, izinto ezinje zinokwenzeka:

• Iintshukumo ezingalawulekiyo nezingaqhelekanga zamalungu omzimba.
• Ukungcangcazela kwesenzo : Oku kuthetha ukuba xa uzama ukwenza into ethile, ukungcangcazela kwamalungu omzimba wakho kuyanda.
• Ukuhamba ngeenyawo ezibanzi ngokungaqhelekanga .
• Ukuxhuzula kwamehlo okungalawulekiyo (nystagmus).

Iimpawu ze-MSA-P (uhlobo lweParkinsonism):

Olu hlobo lwempawu ludla ngokuqala kwelinye icala lomzimba luze lusasazeke kumacala omabini. Ezi mpawu zihlala zibonakala ngolu hlobo:

• Ukuhamba kancinci (bradykinesia), ukuziva ngathi umzimba awunabomi .
• Ukuziva ukuqina nokuqina emzimbeni, nto leyo ebangela ukuma okubonakala ngathi kugobile phambili.
• Ukukhubeka rhoqo xa uhamba.
• Ukuthintitha xa uthetha, intetho engacacanga .

Kutheni le MSA isenzeka? Yintoni unobangela?

Isizathu esichanekileyo se-MSA asikaziwa okwangoku , kodwa iingcali zicinga ukuba sinxulumene neproteni ebizwa ngokuba yi-'alpha-synuclein', enokuqokelelana kwiindawo ezahlukeneyo zobuchopho bethu. Le proteni ikwakholelwa ukuba yeyona nto ibangela isifo sikaParkinson.

Iiproteni ziikhemikhali ezibalulekileyo ukuze imizimba yethu isebenze. Zinceda kwizinto ezininzi, ezinje ngokunxibelelana phakathi kweenkqubo ezahlukeneyo emzimbeni kunye nokuthutha iikhemikhali ezijikeleza. Nangona kunjalo, ukuba ezi proteni ziqokelelana kwiindawo ezingafanelekanga, zinokubangela umonakalo. Iingcali zikholelwa ukuba lo monakalo yiwo obangela ukuba izicubu zengqondo zife kancinci kancinci kwi-MSA.

Izazinzulu zisaphanda ukuba kutheni le protein ye-'alpha-synuclein' iqokelelana kwiindawo ezithile zobuchopho. Zikwacinga ukuba utshintsho oluthile lwezakhi zofuzo lunokutshintsha indlela ezinye iiseli ezisebenzisa ngayo le 'alpha-synuclein'. Kukwakho nobungqina bokuba uhlobo lwe-MSA-C lusebenza kwiintsapho. Nangona kunjalo, akukho khonkco lofuzo olunjalo olufunyenweyo lohlobo lwe-MSA-P.

Eyona nto ibalulekileyo kukuba i-MSA ayisosifo esosulelayo . Awunakuyifumana komnye umntu, kwaye awunakuyisasaza komnye umntu.

Ungazi njani ngokuqinisekileyo ukuba une-MSA? Zeziphi iimvavanyo ezenziwayo?

Ukuba kukho i-MSAEyona ndlela yokuqiniseka ngokupheleleyo kukuhlola izicwili zobuchopho emva kokuba umntu eswelekile, kuba akukho ndlela yokubona ngokuchanekileyo ukuba i-'alpha-synuclein' iqokelelene na kwiindawo zobuchopho ngelixa umntu esaphila.

Ngelixa umntu esaphila, oogqirha bayayikrokrela i-MSA ngokusekelwe kwiimpawu, imbali yezonyango, imbali yosapho, kunye nokuba bayasabela na kunyango oluthile okanye akunjalo. Rhoqo, ukuxilongwa kokuqala sisifo sikaParkinson okanye esinye isifo esifanayo sokuhamba, kwaye ukuxilongwa kutshintshwa kube yi-MSA kamva xa iimpawu ezintsha zivela okanye amayeza athile eyeka ukusebenza.

Kukho iimpawu ezibalulekileyo ezinokwahlula i-MSA kwisifo sikaParkinson:

• I-MSA ikhula ngokukhawuleza : Kuthatha iminyaka ukuba umntu onesifo sikaParkinson abe neengxaki kwinkqubo yemithambo-luvo ezimeleyo. Kodwa kwi-MSA, ezi ngxaki zinokuqala kungekapheli nonyaka.
• Ezinye iimpawu zibonakala ngokwahlukileyo : Iimpawu zenkqubo yemithambo-luvo ezizimeleyo, ngakumbi, zinzima kakhulu kwi-MSA. Nangona kunjalo, izinto ezifana nokungcangcazela zisenokungabi nzima kangako, okanye zingabikho kwaphela. Indlela iimpawu ezisasazeka ngayo emzimbeni wonke inokwahluka.
• Unyango alusebenzi : I-Levodopa yeyona iyeza liphambili lesifo sikaParkinson. Nangona kunjalo, i-levodopa ayisebenzi kakuhle kwi-MSA. Esi sisizathu esiphambili sokuba oogqirha bacinge ukuba isenokuba yi-MSA hayi i-Parkinson's.

Zeziphi iimvavanyo ezenziwayo koku?

Zimbalwa kakhulu iimvavanyo ezinceda ngokuthe ngqo ekuxilongeni i-MSA. Uninzi lwezilingo lwenziwa ukuze kuthintelwe ezinye izifo kunye nokuqokelela ubungqina obungakumbi bokuxhasa ukurhanelwa kokuba isenokuba yi-MSA. Ezinye zezi ziquka:

• Iskeni se-Magnetic resonance imaging (MRI) : Oku ngamanye amaxesha kunokubonisa iindawo zobuchopho ezonakeleyo. Oku kunokunceda ekwenzeni uxilongo oluchanekileyo. Kukwaluncedo ekuchongeni i-MSA-C, njengoko inokubonisa ipateni enemigca enqamlezileyo kwinxalenye yobuchopho ("uphawu lwe-hot cross bun"). Nangona kunjalo, olu phawu lunokubonwa nakwezinye izifo, ngoko ke akwanelanga ukufumanisa i-MSA yodwa.
• Uvavanyo lwemfuza : Oku kunokujonga utshintsho lwemfuza oluchaphazela indlela umzimba womntu osebenza ngayo i-alpha-synuclein. Olu vavanyo lunokuchonga utshintsho lwemfuza olunxulumene ne-MSA-C phakathi kwabantu baseJapan.
• I-biopsy yolusu : Ezinye iintlobo ze-biopsy yolusu zinokubona iimpawu zokuqokelelwa kwe-alpha-synuclein kwizicubu zemithambo-luvo. Nangona kunjalo, kufuneka uphando oluthe kratya ukuze kuqinisekiswe ukuba oku kuluncedo ngokwaneleyo ukuba yinxalenye eqhelekileyo yenkqubo yokuxilonga.

Ugqirha wakho uza kukuxelela ngezinye iimvavanyo ezifanelekileyo kunye nendlela ezinokukunceda ngayo. Baza kuqwalasela imbali yakho yempilo, imbali yosapho, kunye nezinye izinto ukuze benze isigqibo esifanelekileyo kuwe.

Ngaba ikhona indlela yonyango lwe-MSA? Ngaba inganyangeka?

Okwangoku, akukho nyango luqinisekileyo olufunyenweyo lwe-MSA.Ngoko ke, unyango lujolise kakhulu ekulawuleni iimpawu ixesha elide kangangoko kunokwenzeka. Unyango lweempawu ze-MSA luxhomekeke kwizinto ezininzi, kuquka iimpawu zesigulana kunye nobunzima bazo.

Ngawaphi amayeza okanye unyango olusetyenziswayo?

Kukho iintlobo ezininzi zamayeza anokunceda ukulawula iimpawu ze-MSA. Uhlobo lwamayeza owafumanayo luya kuxhomekeka kwiimpawu zakho kunye nezinye izinto. Ugqirha wakho ngumntu ofanelekileyo ukucebisa amayeza afanelekileyo kuwe, njengoko eya kuba ngumntu onolwazi kakhulu ngemeko yakho. Baza kukuchazela nangemiphumo emibi enokubakho yonyango.

Kubalulekile: I-MSA yimeko enokufunyanwa kuphela ngugqirha oqeqeshiweyo nofanelekileyo. Ngoko ke, musa ukuzama ukuxilonga okanye ukunyanga iimpawu ngokwakho ngaphandle kokuthetha nogqirha.

Ngaba ikho indlela yokuthintela i-MSA ekuphuhliseni?

Iingcali azikayazi ukuba yintoni ebangela i-MSA okanye zeziphi izinto ezibangela loo nto. Ke ngoko, okwangoku akukho ndlela yokuyithintela okanye yokunciphisa umngcipheko .

Luhlobo luni lwekamva umntu one-MSA anokulilindela?

Abantu abane-MSA badla ngokuba neempawu zokuqala ezinxulumene nokuhamba. Le meko iba mandundu kancinci kancinci ngokuhamba kwexesha. Malunga nesiqingatha sezigulane zifuna uncedo lokuhamba ngeli xesha. Oku kuthetha ukusebenzisa intonga okanye isixhobo sokuhamba. Malunga neminyaka emihlanu emva kokuqala kwe-MSA, malunga nama-60% ezigulane kufuneka zisebenzise isitulo esinamavili. Kwiminyaka emithandathu ukuya kwesibhozo, ubuncinane isiqingatha sezigulane sihlala ebhedini.

Njengoko isifo siqhubeka, kunokufuneka iinkqubo ezongezelelweyo okanye ukungenelela ukuze kugcinwe ukusebenza komzimba kunye nokuthintela iingxaki eziyingozi. Ezinye zezi ziquka:

• I-Tracheostomy yokugcina ukuphefumla.
• Ukondla ngetyhubhu (`ukondla ngetyhubhu` / `isondlo sangaphakathi`).
• Ii-catheters ezihlala ngaphakathi okanye i-urostomy yokungakwazi ukuzibamba komchamo.
• I-Colostomy yokujongana neengxaki zokuhamba kwamathumbu.

Ihlala ixesha elingakanani i-MSA?

I-MSA yimeko ehlala ihleli, ubomi obuqhelekileyo. Umyinge wobomi obulindelweyo kunye nesifo yiminyaka emithandathu ukuya kwelishumi . Kwiimeko ezingezizo eziqatha, sinokufikelela kwiminyaka eli-15. Nangona kunjalo, kwiimeko eziqatha ngakumbi, ubomi obulindelekileyo bunokuba bufutshane kakhulu. Ezi meko ziqatha zihlala ziquka ezi mpawu zilandelayo:

• Iimpawu zenkqubo yemithambo-luvo ezizimeleyo ziba nzima ngaphambi kokuba kuqale iimpawu ezinxulumene nokuhamba.
• Ukwaluphala xa kuchongwa isifo.
• Ukuba neempawu zokushukuma ezibangela ukuwa rhoqo.

Ithini imbono ngale meko?

Imeko ye-MSA ayintle kangako. Iimpawu zale meko ziya zisiba mandundu kancinci kancinci, ziphazamisana nemisebenzi yomzimba kwaye zikhokelela kwiingxaki ezibulalayo. Iingxaki ezinokukhokelela ekufeni ziquka:

• Ukukrala kwemiphunga.
• Usulelo lomchamo (UTIs) lunokubangela i-sepsis (ityhefu egazini).
• Ukufa ngequbuliso (ngokuqhelekileyo ebusuku, ngenxa yokuphazamiseka kwindlela ingqondo elawula ngayo ukuphefumla ngexesha lokulala).

Ndingazinyamekela njani mna/umntu endimthandayo? Yintoni endifanele ndiyixhalabele?

Abantu abane-MSA baneempawu eziqhubekekayo. Oku kuthetha ukuba xa iimpawu zifikelela kwinqanaba elithile, abasakwazi ukuphila ngokuzimela. Esi simo sinokuchaphazela nokukwazi kwakho ukucinga, ukuthetha, kunye nokwenza izigqibo ngokwakho. Ngenxa yazo zonke ezi zinto, kubalulekile ukuthetha nabantu obathandayo malunga neminqweno yakho yekamva kunye nokwenza isicwangciso sonyango lwakho ukuba awukwazi ukwenza izigqibo .

Ufanele uye nini kugqirha?

Uninzi lweempawu zokuqala ze-MSA zizinto ekufuneka uzixoxe nogqirha. Ezi ziquka:

• Ukuziphatha okubi ngokwesondo.
• Uxinzelelo lwe-Orthostatic (isiyezi esingapheliyo okanye ukuquleka ngaphandle kwesizathu esicacileyo).
• Iingxaki zokulala kunye ne-sleep apnea (ukufuthaniselana ngexesha lokulala).

Ukuba ugqirha ukuxilonga ukuba unesifo sokuhamba esifana nesifo sikaParkinson, kubalulekile ukuthetha naye malunga naluphi na utshintsho kwiimpawu zakho. Ugqirha wakho uza kucwangcisa utyelelo olulandelayo ukuze ajonge imeko yakho aze alungise amayeza akho. Ungathetha ngalo naluphi na utshintsho oluqaphelayo ngexesha lotyelelo. Kuqhelekile ukuba abantu abaxilongwe ukuba banesifo sikaParkinson batshintshe uxilongo lwabo kamva, nokuba xa kuvela iimpawu ezintsha okanye xa i-levodopa iyeka ukusebenza kakuhle.

Abantu abane-MSA badla ngokufumana ezinye iimpawu, ingakumbi iingxaki zempilo yengqondo. Kubalulekile ukufuna unyango lweengxaki zempilo yengqondo ezinxulumene ne-MSA, kunye neempawu zomzimba. Ugqirha wakho unokukucebisa unyango okanye akuthumele kwingcali yezempilo yengqondo.

Ngamafutshane (Umyalezo Wokuya Ekhaya)

I-Multiple System Atrophy (MSA) sisifo esibi kakhulu, ekugqibeleni esibulalayo. Ngelishwa, akukho nyango okanye unyango oluthe ngqo lwale meko okwangoku.

>

Nangona kunjalo, uninzi lweempawu zayo zinokunyangwa , kwaye kukho iindlela zokunciphisa iziphumo kunye neempawu. Ngonyango, abantu abaninzi banokugcina umgangatho wobomi babo iminyaka, bachithe ixesha elixabisekileyo nabantu ababathandayo, kwaye balisebenzise kakuhle ixesha labo. Ke ngoko, kubalulekile ukulawula iimpawu ngeengcebiso zonyango ezifanelekileyo, ngaphandle kokubangela ukukhungatheka.

"Ukuwohloka Kweenkqubo Ezininzi, i-MSA, Isifo Semithambo-luvo, Isifo Sobuchopho, Iimpawu ZikaParkinson, Iingxaki Zokuhamba, Iingxaki Zenkqubo Yemithambo-luvo Ezimeleyo