Ngaba umzimba wakho uziva ungalawuleki? Masifunde ngale (Autoimmune Autonomic Ganglionopathy - AAG)!

Ngaba ngamanye amaxesha uziva unesizungu ngequbuliso? Okanye uziva ngathi kukho into engaqhelekanga esiswini sakho, umlomo wakho womile, okanye awukwazi ukulawula ukuphefumla kwakho? Nangona ezi zinto zingabonakala ngathi zizinto eziqhelekileyo, ngamanye amaxesha kunokubakho ibali eliyinkimbinkimbi ngakumbi emva kwazo. Le yenye yeemeko ezinqabileyo, kodwa ezifanelekileyo ukuzazi, esiza kuthetha ngazo namhlanje.

Yintoni le "Autoimmune Autonomic Ganglionopathy - AAG"? Masiyiqonde ngokulula, akunjalo?

Ngamafutshane, "i-Autoimmune Autonomic Ganglionopathy" okanye "(AAG)" yimeko apho inkqubo yokuzikhusela yomzimba wethu, oko kukuthi, inkqubo yokuzikhusela, ihlasela ngempazamo inkqubo yethu yemithambo-luvo ezimeleyo. Khawucinge nje, oku kufana nento ebenokwenzeka ukuba umkhosi omele ukhusele ilizwe lethu uhlasela ngempazamo iindawo zethu ezibalulekileyo.

Inkqubo yemithambo-luvo yomzimba wethu ezimeleyo ifana "nenkqubo yethu ezenzekelayo". Oko kukuthi, ilawula izinto ezininzi ezenzeka ngokuzenzekelayo, ngaphandle kokuba siqaphele. Umzekelo:

• Ukuphefumla kwakho
• Isantya sentliziyo (isantya sentliziyo)
• Ifuthe legazi
• Ukubila
• Ukugaya ukutya

Ezininzi zezi zinto asizizo izinto esizicingayo. Ziyenzeka ngokuzenzekelayo. Inkqubo yemithambo-luvo ezimeleyo yiyo enceda ukugcina loo misebenzi izenzekelayo isebenza kakuhle. Kwi-AAG, le nkqubo ibalulekileyo yonakele. Le yimeko ebizwa ngokuba yi-autonomic neuropathy, okanye i-dysautonomia.

Kutheni imizimba yethu isenza oku? Zithini izizathu?

Enyanisweni, noogqirha abakakwazi ukusho ngqo ukuba yintoni kanye kanye imbangela yale `(AAG)`. Kodwa kukho iinyani ezifunyenweyo.

• Amaxesha amaninzi, oko kukuthi , malunga nabantu abathathu kwabahlanu abafumana i-"AAG", le meko yenzeka emva kokuchacha kwesinye isifo, okanye emva kosulelo. Ifana nesiphumo esibi seso sifo.
• Abanye abantu banokuba nesifo esibizwa ngokuba yi-paraneoplastic AAG. Kulapho ezinye ii-antibodies ezenziwa yinkqubo yethu yokuzikhusela komzimba xa isabela kwimeko efana nomhlaza zihlasela inkqubo yemithambo-luvo ezimeleyo.

Oku kwenza ntoni emizimbeni yethu? Makhe sijonge ngokusondeleyo.

Inkqubo yethu yemithambo-luvo ezimeleyo ineeseli zemithambo-luvo ezibizwa ngokuba yi -autonomic ganglia . Ezi zifana nee-train junctions. Imiyalezo eyahlukeneyo ithunyelwa kwaye ifunyanwe ngazo. Olu tshintshiselwano lwemiyalezo lubalulekile ukuze izenzo zomzimba wethu zilawuleke ngokuzenzekelayo, oko kukuthi, izenzo esingazilawuliyo (reflexes).

Kwi-AAG, inkqubo yethu yomzimba yokuzikhusela ihlasela ii-receptor ezithile kwezi ganglia. Ii-receptors ziindawo apho imiyalezo ifunyanwa khona. Ngoko ke xa ezi receptors zonakele, loo miyalezo yemithambo-luvo ayihambi okanye ayidluli ngendlela efanelekileyo. Ngenxa yoko, izinto ezenzeka ngokuzenzekelayo, njengokulawula uxinzelelo lwegazi kunye nokubila, ziqala ukuba mdaka.

Khawucinge nje, ukuba 'uqhagamshelo' kwi 'main switch board' yekhaya lakho luyakhululeka, izibane azivulwa, ifeni ayijikelezi, njl. Kunjalo ke ngale nto.

Ixhaphake kangakanani le meko ibizwa ngokuba yi-`(AAG)`?

Esi sisifo esingaqhelekanga ngokwenene. Umzekelo, eMelika, ngabantu abamalunga ne-100 kuphela abafunyaniswa benesi sifo minyaka le. Ngoko ke asiqhelekanga eSri Lanka. Ngoko ke ungoyiki xa usiva ngale nto, oku akwenzeki kubantu abaninzi.

Zithini iimpawu ze-`(AAG)`? Sizibona njani?

Iimpawu zale meko zinokwahluka kumntu nomntu. Asinguye wonke umntu oza kuba neempawu ezifanayo. Iimpawu eziqhelekileyo zezi:

• Ukuqhinwa: Oku kuthetha ukuqhinwa kunye nobunzima bokuya kwindlu yangasese.
• Iimbombo ezivulelekileyo (iimbombo zika-Adie): Ngamanye amaxesha ngomnye umbombo weliso elinye onokuvuleka kwaye unokuba novakalelo ekukhanyeni.
• Umlomo owomileyo okanye amehlo omileyo: Ukuziva ngathi akukho mathe okanye iinyembezi emehlweni.
• Ukuquleka/Ukungakwazi ukunxibelelana: Usenokuquleka ngequbuliso ngaphandle kwesizathu esicacileyo.
• Uxinzelelo lwegazi olubangelwa yi-neurogenic orthostatic hypotension: Ukuziva unesiyezi kunye nokuphelelwa lithemba xa umi kwindawo yokuhlala okanye uphuma ebhedini. Oku kubangelwa kukwehla ngequbuliso koxinzelelo lwegazi .
• Ubunzima bokuchama / Ukugcina umchamo: Ukuziva ungakwazi ukuchama nangona ufuna ukuchama.

Malunga nabantu ababini kwabathathu abane-AAG bafunyenwe benamanqanaba aphezulu ee-antibodies ezibizwa ngokuba yi -ganglionic acetylcholine receptor antibodies (g-AChR antibodies) egazini labo. Abaphandi bakholelwa ukuba kukho unxibelelwano phakathi kwamanqanaba ezi antibodies ze-g-AChR kunye nobunzima beempawu.

Ugqirha usixilonga njani esi sifo `(AAG)`?

Ukuba uneempawu ezinje, into yokuqala ugqirha aza kuyenza kukubuza ngeempawu zakho aze akuhlole. Emva koko, banokwenza uvavanyo oluthile ukujonga ukusebenza kwenkqubo yakho yemithambo-luvo.

Nazi ezinye zazo:

• Uvavanyo lwenkqubo yokugaya ukutya: Umzekelo, uvavanyo "lokukhupha isisu". Olu vavanyo luvavanya ukuba ukutya kukhawuleza kangakanani ukuphuma esiswini sakho.
• `Uvavanyo lwe-axon reflex ye-quantitative sudomotor (QSART)`: Olu vavanyo lulinganisa indlela esebenza ngayo i-birth glands yakho.
• `Uvavanyo lokubila olusebenzisa i-thermoregulatory sweat`: Olu vavanyo lwenziwa ukuze kufunyaniswe ukuba ukubila komzimba kuye kwehla okanye kwanda, kwaye yintoni unobangela.
• `Uvavanyo lwetafile yokuthambeka`: Kolu vavanyo, wenziwa ukuba ulale ebhedini ekhethekileyo kwaye ibhedi ijikeleziswa kancinci ngokuthe nkqo, ujonge indlela isantya sentliziyo yakho kunye noxinzelelo lwegazi olutshintsha ngayo.
• Uhlalutyo lomchamo: Oku kunokukunika umbono wokuba isinyi sakho sisebenza kakuhle kangakanani.

Ukongeza, uvavanyo lwegazi lunokwenziwa ukujonga inqanaba le-`(g-AChR antibodies)` ekhankanyiweyo . Ukuba inqanaba le-antibody liphezulu, bubungqina obongezelelekileyo bokuba isenokuba yi-`(AAG)`. Nangona kunjalo, olu vavanyo lwegazi lwahlukile kuvavanyo lwe-`AChR antibody`, olusetyenziselwa ukujonga esinye isifo se-neurological esibizwa ngokuba yi-`(Myasthenia Gravis).`

Ngaba ikho indlela yokunyanga oku? Yintoni enokwenziwa?

Ngenxa yokuba i-AAG yimeko engaqhelekanga, akukho ndlela iqhelekileyo, "le yindlela yonyango". Uninzi lwexesha , ingqwalasela ikwindlela yokulawula iimpawu.

Oogqirha benza isicwangciso sonyango ngokudibanisa iindlela ezahlukeneyo zonyango. Ezinye zazo ziquka:

• Ukutshintshiselana ngeplasma/iplasmapheresis: Oku kuquka ukuthatha inxalenye yegazi lakho, ukususa izinto eziyingozi ezifana nee-antibodies kwiplasma, uze ubuyisele igazi elicociweyo emzimbeni wakho.
• Unyango lwe-Intravenous immunoglobulin (IVIG): Oku kuquka ukunika umzimba wakho i-immunoglobulin (inxalenye yeplasma elwa nesifo) ethathwe kubantu abaphilileyo. Oku kunceda ukuzola inkqubo yomzimba yokuzikhusela.
• Ii-corticosteroids ezifakwa kwimithambo: Iidosi eziphezulu zee-steroid ezilwa nokuvuvukala, ezifana ne-methylprednisolone (Solu-Medrol®) okanye i-hydrocortisone (Solu-Cortef®), zinikwa ngenaliti.
• Amayeza okunciphisa umzimba: La mayeza asebenza ngokunciphisa ukusebenza ngokugqithisileyo kwenkqubo yomzimba yokuzikhusela. Umzekelo, amayeza afana ne-rituximab (Rituxan®).

Ngaba ikho indlela yokuthintela le meko `(AAG)`?

Akukho ndlela iqinisekisiweyo yokuthintela oku, kuba kunqabile kakhulu. Nangona kunjalo, ezinye iimeko, ezifana nomhlaza, zinokunyusa kancinci umngcipheko wakho wokuba nakho. Ukuba ukhe waba nomhlaza, nazi ezinye izinto onokuzenza ukuze unyamekele impilo yakho iyonke:

• Ukuzilolonga rhoqo: ingakumbi ukuzilolonga nge-aerobic.
• Ukuba une-`(Dysautonomia)`, ukutya ukutya okunetyuwa eninzi kunye nolwelo kunokunceda ukulawula uxinzelelo lwakho lwegazi. (Le yinto omele uyixoxe nogqirha wakho.)
• Ukulawula uxinzelelo: Sebenzisa iindlela ezisempilweni ezifana nokuzilolonga, ukuthetha nomhlobo, nokubhala kwincwadi yemihla ngemihla.
• Lala kakuhle: Lala ubuncinane iiyure ezisi-7-8 ngosuku.
• Ukunxiba iimpahla zokucinezela: Ezi zinceda ukuphucula ukujikeleza kwegazi nokunciphisa ukudumba (ingakumbi emilenzeni).

Ngaba le meko `(AAG)` inganyangeka ngokupheleleyo? Kuza kwenzeka ntoni kwixesha elizayo?

Ndimele nditsho, nangona ndibuhlungu,I-AAG ayinyangeki. Nangona kunjalo, abantu abaninzi banokukwazi ukulawula iimpawu zabo kakuhle ixesha elide. Okumangalisayo kukuba, malunga nomntu omnye kwabathathu abane-AAG uya kuba ngcono kancinci kancinci ngaphandle konyango. Nangona kunjalo, uninzi lwabantu kuya kufuneka lujongane nezinye iimpawu ebomini babo.

Yintoni enye ofuna ukuyibuza ugqirha?

Ukuba ukrokrela ukuba unesifo esifana nesi, kubaluleke kakhulu ukubona ugqirha olungileyo uze ubuze ngale nto ilandelayo:

• Yintoni enokubangela iimpawu zam?
• Zeziphi iimvavanyo ekufuneka ndizenze ukuze ndifumanise ukuba `(AAG)` ndinayo na?
• Ziziphi iindlela zonyango ze-`(AAG)`?
• Ingaba kunokwenzeka ukuba iimpawu ze-`(AAG)` ziphele nokuba akukho nyango?
• Zeziphi iinguqu endimele ndizenze kwindlela yam yokuphila ukuze ndiphucule impilo yam iyonke?

Ziziphi ezinye izifo zomzimba ezinxulunyaniswa ne-autonomic neuropathy?

``(AAG)`` ikhethekile kuba, kwelinye icala, sisifo esinxulumene nenkqubo yomzimba yokuzikhusela, kwaye kwelinye icala, sisifo sokuzikhusela komzimba kwinkqubo yemithambo-luvo.

Ukongeza, kukho ezinye izifo ezinxulumene nenkqubo yomzimba yokuzikhusela ezinyusa umngcipheko wokuba ne-"dysautonomia" (iingxaki zenkqubo yemithambo-luvo ezimeleyo). Eminye imizekelo yile:

• Isifo seCeliac
• `(Isifo sikaGuillain-Barré)` (isifo sikaGuillain-Barré)
• `(Lupus / systemic lupus erythematosus)` (isifo seLupus)
• `(Isifo samathambo esibizwa ngokuba yi-rheumatoid arthritis)` (Isifo samathambo esibizwa ngokuba yi-rheumatoid arthritis)
• Sjögren's syndrome (isifo sikaSjögren)

Ngoko ke ungabona, ezi ziimeko ezinokudityaniswa.

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, ngoku uyazi ukuba `(Autoimmune Autonomic Ganglionopathy - AAG)` sisifo esingaqhelekanga apho inkqubo yethu yokuzikhusela ihlasela inkqubo yethu yemithambo-luvo ezimeleyo. Iimpawu zingabandakanya ukulahlekelwa yingqondo ngesiquphe, ukwehla 'koxinzelelo' xa umi, kunye neengxaki zesisu.

Musa ukoyika xa usiva oku. Oku kunqabile kakhulu. Kwakhona, amayeza okunciphisa amasosha omzimba kunye ne-'plasmapheresis' zisetyenziswa njengonyango lwale nto. Nangona ingenakunyangwa ngokupheleleyo, uninzi lwabantu lunokulawula iimpawu kakuhle kwaye luphile ubomi obuqhelekileyo.

Eyona nto ibalulekileyo kukubona ugqirha ofanelekileyo ngokukhawuleza ukuba unale mpawu. Ukuba uziqaphela kwangethuba, ungaqala unyango kwaye unciphise impembelelo ebomini bakho. Awuwedwa, kwaye kukho oogqirha abanokukunceda.

` I-Autoimmune Aneurysmal Ganglionopathy, i-AAG, Inkqubo yeNervous ye-Autoimmune, Inkqubo yoMzimba, Isifo seNeural, Iimpawu, Unyango