Ingabe uzwa imizwa engavamile emzimbeni wakho? Mhlawumbe kubalulekile ukwazi nge-Multiple System Atrophy (MSA)!

Uke uzizwe sengathi izinto emzimbeni wakho ziyashintsha ngezindlela ongakwazi ukuzilawula? Ngezinye izikhathi kuba sengathi ulahlekelwa yibhalansi yakho lapho uhamba, noma uzizwa udidekile lapho usukuma ngokuzumayo. Lezi yizinto ngezinye izikhathi esingazinaki kakhulu, kodwa zingaba futhi izimpawu zezifo ezithile zemizwa ezingavamile. Namuhla sizokhuluma ngesimo esisodwa esinjalo esiyinkimbinkimbi kancane, kodwa kubaluleke kakhulu ukuqaphela. Leso yi- Multiple System Atrophy (MSA) .

Kuyini ngempela i-Multiple System Atrophy (MSA)?

Kalula nje, i-Multiple System Atrophy (MSA) yisifo esingavamile sezinzwa lapho izingxenye zobuchopho bethu ziba buthaka kancane kancane, okungukuthi, ziwohloka. Ngokuhamba kwesikhathi, imisebenzi namakhono omzimba wethu alawulwa yilezi zingxenye zobuchopho aqala ukuwohloka. Empeleni lesi yisimo esibucayi kakhulu, ngoba kunzima ukululama ngokuphelele kuso.

Esikhathini esidlule, i-MSA yayibizwa ngamagama amaningi ahlukene. Kungenzeka ukuthi wake wezwa ngamagama anjenge-`Shy-Drager syndrome`, `Sporadic olivopontocerebellar atrophy`, `Striatonigral degeneration`. Kungemva kwesikhathi lapho odokotela baqaphela khona ukuthi zonke lezi zimo zazinezici ezithile ezifanayo. Yingakho bazihlanganisa zonke bazinika igama elisha, `Multiple System Atrophy` (MSA). Izimpawu ziyahlukahluka kuye ngokuthi iyiphi ingxenye yobuchopho eyonakele. Yingakho umuntu ngamunye eba nenhlanganisela ehlukile yezimpawu.

Kunezinhlobo ezimbili ze-MSA, akunjalo? Ziyini?

Yebo, ukuze kubonakale ngokucacile isimo se-MSA, odokotela basihlukanise saba izinhlobo ezimbili eziyinhloko, kuye ngezimpawu ezivelayo:

1. I-MSA-C : I-'C' lapha imele i-'cerebellar'. Lolu hlobo luthinta kakhulu ikhono lakho lokuhlanganisa ukunyakaza kwakho. Okusho ukuthi, ulahlekelwa ibhalansi yakho , odokotela abayibiza nangokuthi 'i-ataxia'. Lolu uphawu oluyinhloko lwalolu hlobo. Ngesikhathi esifanayo, ungase ubone nezinkinga ngezinhlelo zokulawula ezizenzakalelayo zomzimba ('ukungasebenzi kahle kwe-autonomic') kanye nokuwa njalo.

2. I-MSA-P : Igama elithi 'P' lapha limele 'i-parkinsonism'. Kulolu hlobo, izimpawu ezifana nesifo sikaParkinson ziyabonakala. Nakuba lezi zimpawu zikaParkinson zibonakala kakhulu ezigabeni zokuqala, ngokuhamba kwesikhathi, izinkinga zesimiso sezinzwa esizimele kanye nezimpawu ze-'ataxia' nazo zingavela.

Ubani othinteka kakhulu yilesi simo se-MSA? Sivame kangakanani?

I-MSA ivame ukuthinta abantu abadala abaneminyaka engaphezu kwengu-30. Izimpawu zivame ukuvela phakathi kweminyaka engu-50 nengu-59. Ingathinta noma ubani, kungakhathaliseki ubulili.

Kodwa i-MSA iyisimo esingavamile kakhulu . Ochwepheshe bathi kunezimo ezintsha ezingu-0.6 noma ezingu-0.7 kuphela kubantu abayi-100,000 ngonyaka. Inani eliphelele lamacala lilinganiselwa phakathi kuka-3.4 no-4.9 kubantu abayi-100,000. Ngakho ungabona ukuthi lokhu kuyivelakancane kangakanani.

Kwenzekani emzimbeni nge-MSA? Yiziphi izingxenye zobuchopho ezithintekayo?

I-MSA yisifo esiqhubekayo esithinta izingxenye ezahlukene zobuchopho. Izimpawu zincike engxenyeni yobuchopho ethintekile. Izindawo eziyinhloko zobuchopho ezithintekile yi-MSA yilezi:

• I-Basal ganglia : Lezi zitholakala phakathi kobuchopho. Zixhumanisa izingxenye ezahlukene zobuchopho futhi zizisize ukuthi zisebenze ndawonye. Kufana negumbi lokulawula eliyinhloko lobuchopho .
• I-Brainstem : Yilokhu okulawula izinqubo eziningi ezizenzakalelayo emzimbeni wethu. Okusho ukuthi, izinto ezenzeka ngokuzenzakalelayo ngaphandle kokuthi sicabange ngazo, isibonelo, ukuphefumula, ukushaya kwenhliziyo, kanye nomfutho wegazi. Lokhu kubalulekile ukuze siphile.
• I-Cerebellum : Lokhu kutholakala ngemuva kwekhanda, phansi. Inesibopho esikhulu sokulawula ukunyakaza nokugcina ibhalansi. Isebenza nezinye izingxenye zobuchopho. Abacwaningi basafunda ngokusebenza kwayo okugcwele, kodwa ubufakazi obuthile bubonisa ukuthi ihilelekile emizweni yethu nasekuthatheni izinqumo.

Ngakho-ke, uma lezi zingxenye zobuchopho zonakele, imisebenzi elawulwa yilezo zingxenye ayikwazi ukwenziwa kahle. Isibonelo, uma i-'Brainstem' yonakele, izinkinga ngezinto ezizenzakalelayo njengomfutho wegazi zingavela.

Ziyini izimpawu ze-MSA? Sizibona kanjani?

Ezinye izimpawu ze-MSA zivamile kuzo zombili izinhlobo, kanti ezinye ziqondile kuhlobo ngalunye. Kodwa into eyodwa evamile kuzo zombili izinhlobo ukungasebenzi kahle kwe-autonomic. Kalula nje, izinhlelo zokulawula umzimba wakho ziyaphela.

Izimpawu ezibangelwa izinkinga ohlelweni lwezinzwa oluzimele:

• Ukwehla kwe-Orthostatic Hypotension : Lokhu kwenzeka lapho umfutho wegazi wakho wehla ngokuzumayo, okubangela isiyezi , lapho ushintsha isikhundla, isibonelo, lapho usukuma ngokuzumayo uhleli.
• Ukungakwazi ukulawula umchamo (`ukungakwazi ukuzibamba`) kanye nokungakwazi ukulawula indle (`ukungakwazi ukuzibamba`).
• Ukungasebenzi kahle kocansi, ikakhulukazi kwabesilisa, kuwubunzima bokuthola ukuqina (ukungasebenzi kahle kwe-erectile).
• Isifo sokuziphatha kokulala esihambisana nokunyakaza kwamehlo okusheshayo (REM) : Njengesifo sikaParkinson, uma uphupha, empeleni ulingisa izinto ozenzile emaphusheni akho. Ungase ukhale noma uphakamise izingalo zakho.
• Ukwehla kokujuluka (i-anhidrosis).
• Izinkinga zokubona.
• Umlomo owomile.
• Ukuphefumula komoya.
• Ukugaya ukudla kancane kanye nokuqunjelwa.

Cabanga ukuthi eziningi zalezi zimpawu ezizimele zingavela ezinyangeni, ngisho neminyaka, ngaphambi kokuba kuvele izimpawu zemisipha. Lokhu kwenzeka phakathi kuka-20% no-75% weziguli ze-MSA.

Izici zengqondo nezingokomzwelo

Cishe ingxenye yesithathu yabantu abane-MSA babhekana nezinkinga ngekhono labo lokucabanga nokugxila. Banobunzima futhi bokulawula imizwa yabo. Lokhu kungaholela ezinkingeni zempilo yengqondo ezifana nalezi:

• Ukukhathazeka.
• Ukucindezeleka.
• Ukungazinzi ngokomzwelo: Okusho ukuthi, ukukhala nokuhleka ngendlela engafanele.
• Ukuhlaselwa uvalo.
• Imicabango yokuzilimaza noma yokuzibulala.

Izici ezihlobene nokunyakaza

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

Lolu hlobo luhilela kakhulu i-'ataxia' (ukulahlekelwa yibhalansi). I-'cerebellum' iyingxenye yobuchopho edlala indima enkulu ekulawuleni ukunyakaza kwemisipha. Ngakho-ke, lapho lokho kuxhumana kulahleka, izinto ezinjengalezi zingenzeka:

• Ukunyakaza okungalawuleki nokungahlelekile kwamalungu.
• Ukuthuthumela kwesenzo : Lokhu kusho ukuthi uma uzama ukwenza okuthile, ukuthuthumela ezithweni zakho kuyanda.
• Ukuhamba ngezinyawo ezibanzi ngendlela engavamile .
• Ukuqhaqhazela kwamehlo okungalawuleki (i-nystagmus).

Izici ze-MSA-P (uhlobo lwe-Parkinsonism):

Lolu hlobo lwezimpawu luvame ukuqala ohlangothini olulodwa lomzimba bese lusakazekela ezinhlangothini zombili. Lezi zimpawu zivame ukubukeka kanje:

• Ukunyakaza okuhamba kancane (bradykinesia), ukuzizwa sengathi umzimba awuphili .
• Ukuzwa ukuqina nokuqina emzimbeni, okuholela ekumeni okubonakala sengathi kuncike phambili.
• Ukukhubeka njalo lapho uhamba.
• Ukungingiza lapho ukhuluma, inkulumo engacacile .

Kungani le MSA yenzeka? Iyini imbangela?

Imbangela eqondile ye-MSA ayaziwa okwamanje , kodwa ochwepheshe basola ukuthi ihlobene neprotheni ebizwa ngokuthi i-'alpha-synuclein', engaqongelela ezingxenyeni ezahlukene zobuchopho bethu. Le protheni efanayo nayo kusolwa ukuthi iyimbangela enkulu yesifo sikaParkinson.

Amaprotheni ayikhemikhali ebalulekile ukuze imizimba yethu isebenze. Asiza ezintweni eziningi, njengokuxhumana phakathi kwezinhlelo ezahlukene emzimbeni kanye nokuthutha amakhemikhali azungezile. Kodwa-ke, uma lawa maprotheni eqoqana ezindaweni ezingafanele, angabangela umonakalo. Ochwepheshe bakholelwa ukuthi lo monakalo yiwona obangela ukuba izicubu zobuchopho zife kancane kancane ku-MSA.

Ososayensi basacwaninga ukuthi kungani le phrotheni ethi 'alpha-synuclein' iqoqana ezingxenyeni ezithile zobuchopho. Baphinde basole ukuthi izinguquko ezithile zezakhi zofuzo zingashintsha indlela amanye amaseli asebenzisa ngayo le 'alpha-synuclein'. Kukhona nobufakazi bokuthi uhlobo lwe-MSA-C lusebenza ngemindeni. Kodwa-ke, akukho ukuxhumana okunjalo kwezakhi zofuzo okutholakale okwamanje kohlobo lwe-MSA-P.

Into ebaluleke kakhulu ukuthi i-MSA ayisona isifo esithathelwanayo . Awukwazi ukusithola komunye umuntu, futhi awukwazi ukusidlulisela komunye umuntu.

Wazi kanjani ukuthi une-MSA? Yiziphi izivivinyo ezenziwayo?

Ukuthi kukhona i-MSAUkuphela kwendlela yokuqiniseka nge-100% ukuhlola izicubu zobuchopho ngemva kokushona komuntu, ngoba ayikho indlela yokubona ngokunembile ukuthi i-'alpha-synuclein' iqongelele yini ezindaweni zobuchopho ngesikhathi umuntu esaphila.

Ngesikhathi umuntu esaphila, odokotela basola i-MSA ngokusekelwe ezimpawini, emlandweni wezokwelapha, emlandweni womndeni, nokuthi uyasabela yini ekwelashweni okuthile noma cha. Ngokuvamile, ukuxilongwa kokuqala yisifo sikaParkinson noma esinye isifo sokunyakaza esifanayo, futhi ukuxilongwa kushintshwa kube yi-MSA kamuva lapho kuvela izimpawu ezintsha noma imithi ethile iyeka ukusebenza.

Kunezici eziningana ezibalulekile ezingahlukanisa i-MSA nesifo sikaParkinson:

• I-MSA ithuthuka ngokushesha : Kuthatha iminyaka ukuthi umuntu onesifo sikaParkinson abe nezinkinga ohlelweni lwezinzwa oluzimele. Kodwa ku-MSA, lezi zinkinga zingaqala phakathi nonyaka.
• Ezinye izimpawu zivela ngendlela ehlukile : Izimpawu zesimiso sezinzwa esizimele, ikakhulukazi, zimbi kakhulu ku-MSA. Kodwa-ke, izinto ezifana nokuthuthumela zingase zingabi zimbi kangako, noma zingase zingabi khona nhlobo. Indlela izimpawu ezisakazeka ngayo emzimbeni wonke ingahluka.
• Ukwelashwa akusebenzi : I-Levodopa iwumuthi oyinhloko wesifo sikaParkinson. Kodwa-ke, i-levodopa ayisebenzi kahle ku-MSA. Lesi yisona sizathu esiyinhloko esenza odokotela bacabange ukuthi kungaba yi-MSA hhayi i-Parkinson's.

Yiziphi izivivinyo ezenziwayo ngalokhu?

Zimbalwa kakhulu izivivinyo ezisiza ngqo ekuxilongeni i-MSA. Izivivinyo eziningi zenziwa ukuze kukhishwe ezinye izifo nokuqoqa ubufakazi obengeziwe bokusekela izinsolo zokuthi kungenzeka ukuthi yi-MSA. Ezinye zalezi zifaka:

• Ukuskena kwe-magnetic resonance imaging (MRI) : Lokhu ngezinye izikhathi kungabonisa izindawo zobuchopho ezonakele. Lokhu kungasiza ekwenzeni ukuxilongwa okunembile. Kuwusizo futhi ekuboneni i-MSA-C, njengoba kungabonisa iphethini enemigqa eqondile engxenyeni yobuchopho ("isibonakaliso se-hot cross bun"). Kodwa-ke, lesi sibonakaliso singabonakala nakwezinye izifo, ngakho-ke akwanele ukuxilonga i-MSA ngokwakho.
• Ukuhlolwa kwezakhi zofuzo : Lokhu kungahlola ukuguquka kwezakhi zofuzo okuthinta indlela umzimba womuntu ocubungula ngayo i-alpha-synuclein. Lokhu kuhlolwa kungenzeka kakhulu ukuthi kutholakale ukuguquka kwezakhi zofuzo okuhlobene ne-MSA-C phakathi kwabantu baseJapan.
• I-biopsy yesikhumba : Ezinye izinhlobo ze-biopsy yesikhumba zingabona izimpawu zokuqongelela kwe-alpha-synuclein ezicutshini zemizwa. Kodwa-ke, kudingeka ucwaningo olwengeziwe ukuze kutholakale ukuthi lokhu kuwusizo ngokwanele yini ukuba yingxenye ejwayelekile yenqubo yokuxilonga.

Udokotela wakho uzokutshela ngezinye izivivinyo ezifanele nokuthi zingakusiza kanjani. Bazocabangela umlando wakho wezempilo, umlando womndeni, kanye nezinye izici ukuze benze isinqumo esingcono kakhulu kuwe.

Ingabe ikhona indlela yokwelapha i-MSA? Ingabe ingelapheka?

Kuze kube manje, akukho ikhambi eliqinisekile elitholakele le-MSA.Ngakho-ke, ukwelashwa kuhloswe kakhulu ukulawula izimpawu isikhathi eside ngangokunokwenzeka. Ukwelashwa kwezimpawu ze-MSA kuncike ezintweni eziningi, okuhlanganisa izimpawu zesiguli kanye nobunzima bazo.

Yimiphi imithi noma izindlela zokwelapha ezisetshenziswayo?

Kunezinhlobo eziningi zemithi engasiza ekulawuleni izimpawu ze-MSA. Uhlobo lwemithi oyitholayo luzoncika ezimpawini zakho nakwezinye izici. Udokotela wakho ungumuntu ongcono kakhulu wokuncoma imithi engcono kakhulu kuwe, njengoba ezoba nolwazi oluningi ngesimo sakho. Bazokuchazela nemiphumela emibi engaba khona yokwelashwa.

Okubalulekile: I-MSA yisimo esingahlolwa kuphela udokotela oqeqeshiwe noqeqeshiwe. Ngakho-ke, ungazami ukuxilonga noma ukwelapha izimpawu ngokwakho ngaphandle kokukhuluma nodokotela.

Ingabe ikhona indlela yokuvimbela i-MSA ukuthi ingakhuli?

Ochwepheshe abakazi ukuthi yini ebangela i-MSA noma ukuthi yiziphi izinto ezibangela lokhu. Ngakho-ke, okwamanje ayikho indlela yokuyivimbela noma yokunciphisa ingozi .

Hlobo luni lwekusasa umuntu one-MSA angalulindela?

Abantu abane-MSA bavame ukuqala babe nezimpawu ezihlobene nokunyakaza. Lesi simo siba sibi kancane kancane ngokuhamba kwesikhathi. Cishe ingxenye yeziguli idinga usizo lokuhamba phakathi nalesi sikhathi. Lokhu kusho ukusebenzisa induku noma i-walker. Cishe eminyakeni emihlanu ngemva kokuqala kwe-MSA, cishe ama-60% eziguli kudingeka asebenzise isihlalo sabakhubazekile. Phakathi neminyaka eyisithupha kuya kweyisishiyagalombili, okungenani ingxenye yeziguli isuke ilala embhedeni.

Njengoba isifo siqhubeka, kungase kudingeke izinqubo ezengeziwe noma ukungenelela ukuze kulondolozwe ukusebenza komzimba nokuvimbela izinkinga eziyingozi. Ezinye zalezi zifo zifaka:

• I-Tracheostomy ukuze kugcinwe ukuphefumula.
• Ukondla ngepayipi (`Ukondla ngepayipi` / `ukondla kwangaphakathi`).
• Ama-catheter angaphakathi noma i-urostomy yokungakwazi ukuzibamba komchamo.
• I-Colostomy yobunzima bokuhamba kwamathumbu.

I-MSA ihlala isikhathi esingakanani?

I-MSA iyisimo esihlala njalo, esihlala isikhathi eside. Isilinganiso sokuphila esihambisana nalesi sifo siyiminyaka eyisithupha kuya kweyishumi . Ezimweni ezingatheni kakhulu, singahlala iminyaka eyi-15. Kodwa-ke, ezimweni ezimbi kakhulu, isikhathi sokuphila singaba sifushane kakhulu. Lezi zimo ezinzima zivame ukufaka izimpawu ezilandelayo:

• Izimpawu zesimiso sezinzwa esizimele ziba zimbi kakhulu ngaphambi kokuqala kwezimpawu ezihlobene nokunyakaza.
• Ukuguga lapho kutholakala ukuthi une-HIV.
• Ukuba nezimpawu zokunyakaza ezibangela ukuwa njalo.

Uyini umbono ngalesi simo?

Ithemba le-MSA alilihle neze. Izimpawu zalesi simo ziya ngokuya ziba zimbi, zivame ukuphazamisa imisebenzi yomzimba futhi ziholele ezinkingeni ezibulalayo. Izinkinga ezingaholela ekufeni zifaka:

• Inyumoniya.
• Ukutheleleka komgudu womchamo (UTIs) kungabangela i-sepsis (ubuthi egazini).
• Ukufa ngokuzumayo (ngokuvamile ebusuku, ngenxa yokuphazamiseka endleleni ubuchopho obulawula ngayo ukuphefumula ngesikhathi sokulala).

Ngizinakekela kanjani mina/othandekayo wami? Yini okufanele ngikhathazeke ngayo?

Abantu abane-MSA banezimpawu eziqhubekayo. Lokhu kusho ukuthi uma izimpawu sezifinyelele ezingeni elithile, abasakwazi ukuphila ngokuzimela. Lesi simo singathinta nekhono lakho lokucabanga, ukukhuluma, nokwenza izinqumo. Ngenxa yazo zonke lezi zici, kubalulekile ukukhuluma nabathandekayo bakho ngezifiso zakho zesikhathi esizayo nokwenza uhlelo lokunakekelwa kwezokwelapha uma ungakwazi ukwenza izinqumo .

Kufanele ubonane nini nodokotela?

Izimpawu eziningi zokuqala ze-MSA yizinto okufanele uzixoxe nodokotela. Lezi zifaka:

• Ukuziphatha okubi ngokocansi.
• Ukwehla kwengcindezi yegazi okubangelwa yi-orthostatic (isiyezi esiqhubekayo noma ukuquleka ngaphandle kwesizathu esibonakalayo).
• Izinkinga zokulala kanye nokuphefumula okuphefumulayo (ukuminyana ngesikhathi sokulala).

Uma udokotela ekuxilonga ukuthi unesifo sokunyakaza njengesifo sikaParkinson, kubalulekile ukukhuluma naye nganoma yiziphi izinguquko ezimpawu zakho. Udokotela wakho uzohlela ukuvakasha okulandelayo ukuze aqaphe isimo sakho futhi alungise imithi yakho. Ungakhuluma nganoma yiziphi izinguquko oziqaphelayo phakathi nalokhu kuvakasha. Kuvamile ukuthi abantu abaxilongwe ukuthi banesifo sikaParkinson bashintshe ukuxilongwa kwabo kamuva, kungaba lapho kuvela izimpawu ezintsha noma lapho i-levodopa iyeka ukusebenza kahle.

Abantu abane-MSA bavame ukubhekana nezinye izimpawu, ikakhulukazi izinkinga zempilo yengqondo. Kubalulekile ukufuna ukwelashwa kwezinkinga zempilo yengqondo ezihlobene ne-MSA, kanye nezimpawu zomzimba. Udokotela wakho angakuncoma ukwelashwa noma akuthumele kuchwepheshe wezempilo yengqondo.

Ngamafuphi (Umyalezo Wokuya Ekhaya)

I-Multiple System Atrophy (MSA) iyisifo esibi kakhulu, ekugcineni esibulalayo. Ngeshwa, akukho ukwelashwa okuqondile kwalesi simo okwamanje.

>

Kodwa-ke, izimpawu zayo eziningi zingelashwa , futhi kunezindlela zokunciphisa imiphumela nezimpawu. Ngokwelashwa, abantu abaningi bangagcina impilo yabo isezingeni eliphezulu iminyaka, bachithe isikhathi esibalulekile nabathandekayo babo, futhi basisebenzise kahle isikhathi sabo. Ngakho-ke, kubalulekile ukuphatha izimpawu ngeseluleko esifanele sezokwelapha, ngaphandle kokubangela ukukhungatheka.

'Ukuwohloka Kwesistimu Eningi, i-MSA, Isifo Sezinzwa, Isifo Sobuchopho, Izimpawu ZikaParkinson, Izinkinga Zokunyakaza, Izinkinga Zesistimu Yezinzwa Ezimele